Poster IANCONAocn 2022

ID: 501

Dural AV Fistula Presenting as Rapidly Progressive Dementia, Stimulus Sensitive Myoclonus and Seizures

Naveen, Balakrishnan R

Neurology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India

E-mail: peenav007@gmail.com

Background and Aim: Dural arteriovenous fistulas (dAVFs) are abnormal connections between an artery and a vein in the tough covering over the brain or spinal cord (dura mater). In this rare condition, abnormal passageways between arteries and veins (arteriovenous fistulas) may occur in the brain, spinal cord or other areas of your body. Dural AVFs tend to occur later in life (50 to 60 years of age), and they’re not typically passed on genetically. Methodology: here we present a interesting case referred from outside hospital to EMD with history of memory disturbance, seizure, tremors. Result: Patient had multiple hospitalisation in the previous Hospital first the patient presented with seizure imaging was done suggestive of chronic Cvt .in the subsequent admission she admitted for ?encephalopathy imaging done suggestive of meningitis and treated with antibiotics, again in a few weeks time again admitted for tremors, myoclonus and urinary incontinence thy treated as autoimmune encephalitis her sensorium worsened she was intubated and extubated since there was no improvement she was referred here for further management on examination pt had persisting encephalopathy repeat imaging was done suggestive of Dural AV Fistula suggested DSA. Conclusion: DSA was done confirmed the same embolisation was attempted but the encephalopathy worsened had a cardiac arrest intubated and tracheostomy was done but sensorium there was no improvement hence she was discharged against medical advice

ID: 502

Harlequin Syndrome Secondary to Herpes Simplex Encephalitis

Varun, Abhishek Dixit, Abhishek Pathak, Rameshwar Nath Chaurasia, Anand Kumar, Vijaya Nath Mishra, Abhishek Dixit, Abhishek Pathak, Anand Kumar, Rameshwar Nath Chaurasia, Abhishek Dixit, Abhishek Pathak, Anand Kumar, Rameshwar Nath Chaurasia, Pratishtha Sengar

Neurology, IMS, BHU, Varanasi, UP, India

E-mail: mailurvarun@gmail.com

Background and Aim: Harlequin syndrome (HS) is a rare dysautonomia syndrome characterized by hyperhidrosis on one side and anhidrosis on contralateral side of the body. We report a rare case of HS secondary to Herpes Simplex Encephalitis. Methodology: A 24-years old right-handed male presented with acute onset, moderate grade fever for 8 days, multiple episodes of focal seizures with bilateral tonic-clonic movements and altered sensorium for 4 days. On examination, Glasgow Coma Scale was E4V1M6, Pulse rate was 78/min, axillary temperature 390C, blood pressure 122/70 mmHg, and respiratory rate 20/min with normal oxygen saturation. Pupils were bilaterally symmetrical, normal in size and reaction to light. Papilledema was absent. Muscle power, tone and deep tendon reflexes were normal in all four limbs. Cardiovascular, respiratory and abdominal examinations were within normal limits. Result: On evaluation, he was diagnosed as a case of Herpes Simplex Encephalitis based on classical radiological and positive cerebrospinal fluid real time polymerase chain reaction test for herpes simplex virus 1. He received full course of intravenous acyclovir and discharged on two antiepileptics. During follow up, he developed hyperhydrosis on one half of face and anhidrosis over other half suggestive of Harlequin syndrome. However, these symptoms subsided by its own without any active intervention. Conclusion: To the best of our knowledge, this is the first report of Harlequin syndrome secondary to HSE.

ID: 503

Changes in Cortical Thickness are Associated with Cognitive Impairments in Patients with White Matter Lesions

Na Wei, Yuexiu Li, Decai Tian, Pei Dai, Huixian Yu, Yumei Zhang

Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China

E-mail: clare_wn@163.com

Background and Aim: White matter lesions (WMLs) play a role in cognitive decline and dementia. It has been hypothesized that cognitive impairment is related to cortical atrophy and cortical thickness network measurements in certain brain regions. The purpose of this research was to investigate the characteristics of cortical thickness in patients with WMLs and the relationship that these characteristics have with cognitive function. Methodology: In this study, a total of 76 patients with WMLs and 37 healthy controls (HCs) were enrolled and underwent magnetic resonance imaging (MRI) scans. Cortical reconstruction and segmentation were performed on the participants’ three-dimensional (3D) T1-weighted images using the FreeSurfer image analysis suite. The patients were further divided into two subgroups, namely, those with WMLs and nondementia vascular cognitive impairment (WML-VCIND) and those with WMLs and vascular dementia (WML-VaD). Cortical thickness analysis was performed. Pearson’s correlation analysis was performed to explore the relationship between cognitive manifestations and cortical thickness in patients with WMLs. Result: WML-VaD patients were significantly older than WML-VCIND patients and HCs (P = 0.01). There was a significant difference in Montreal Cognitive Assessment (MoCA) scores (P < 0.0001) and executive function (P < 0.0001) between the participants in the WML and HC groups. Significantly decreased cortical thickness in the left precentral, caudal middle frontal, rostral middle frontal, superior frontal, middle temporal, transverse temporal, insula, bilateral pars opercularis, and superior temporal regions was found in participants in the WML-VaD group compared with participants in the HC group (P < 0.05). The reductions in the cortical thickness of the above gyri were positively correlated with executive function in patients with WMLs. Conclusion: These cross-sectional results suggest that decreased cortex thickness in certain gyri in patients with WMLs might lead to cognitive decline. These findings have implications that could improve the present understanding of the relationship among WMLs, cortical morphology, and possible concomitant cognitive impairment.

Award ID: 504

A Case of Leukoencephalopathy in Adults Caused by COL4A1 Gene Mutation

Yicong Wang, Weiqi Chen, Yilong Wang, Yilong Wang, Yilong Wang, Yilong Wang, Weiqi Chen, Yilong Wang

Department of Neurology, Beijing Tiantan Hospital, Beijing, China

E-mail: wangyicongccmu@126.com

Background and Aim: adult onset leukoencephalopathy is a general term for all kinds of leukoencephalopathy. It can include cerebrovascular disease, infection, autoimmunity, tumor, metabolic poisoning and degenerative degeneration. The clinical symptoms and imaging manifestations of different types of leukoencephalopathy are similar to each other to a great extent. COL4A1 is involved in encoding type IV collagen fibers α Its gene mutation is associated with small vessel disease, especially cerebral white matter disease, intracerebral hemorrhage and cerebral infarction. Methodology: a case of leukoencephalopathy with clinical manifestations of dizziness to be examined, imaging manifestations of adult onset and posterior head was comprehensively evaluated and detected by imaging, enzymology, very long chain fatty acids and genes, so as to provide basis for clinical diagnosis and treatment in the future. Result: dizziness was the clinical manifestation of this patient, with complex family history, including multiple system involvement such as intracranial artery tortuosity, scoliosis and double renal cyst. Imaging showed white matter lesions in the posterior head. Genetic metabolic diseases caused by abnormalities such as enzymology and very long-chain fatty acids were excluded. The final gene test showed that it was hereditary cerebrovascular disease caused by COL4A1 heterozygous mutation. Conclusion: COL4A1 gene mutation can lead to thickening of vascular basement membrane, coarseness, disorder and degeneration of collagen fibers, and most clinical manifestations are nonspecific. This case of multiple system involvement suggests that the genetic cerebrovascular disease caused by COL4A1 gene mutation should be considered in the symmetrical leukoencephalopathy with adult onset and multiple system involvement.

Award ID: 505

Vaginal recurrence of cervical cancer with Neoplastic Lumbosacral Radiculoplexopathy presenting as acute flaccid paralysis: A case report

Alex, Yugandhar Samireddypalle, Kumbha Dhanusha, Nangadda Narmada

Department of Neurology, All India Institute of Medical Sciences (AIIMS), Mangalagiri, Andhra Pradesh, India

E-mail: dralexrebello@gmail.com

Background and Aim: Acute flaccid paralysis (AFP) denotes flaccid weakness, less than 4 weeks, reaching its maximum severity in days to weeks. The causes include infections, immune mediated, toxins, ischemia and trauma. Neoplastic lumbosacral radiculoplexopathy (nLRP) is a rare cause of AFP. It can occur in association with pelvic malignancies like carcinomas of colon, prostate and cervix or other malignancies like breast and lung cancers.1 Methodology: We describe a case of nLRP associated with vaginal recurrence of cervical cancer presenting as AFP. Result: A 48-year-old lady presented with weakness of left lower limb for 1 week without any bowel or bladder disturbances. She had no fever, backache or trauma. She had undergone hysterectomy 3 years back. She had pain in left lower limb for 3 years which was unevaluated. On examination, there were severe weakness (grade:0/5 power), atonia, areflexia and sensory loss in whole left lower limb. CEMRI showed plaque like thickening involving left lumbosacral plexus and a vaginal vault mass lesion along with pelvic lymphadenopathy. FNAC from mass lesion showed moderately differentiated squamous cell carcinoma (SCC). Her post-hysterectomy biopsy had been reported as well differentiated cervical SCC, but she didn’t undergo any further treatment after hysterectomy. Conclusion: There are a few cases of nLRP associated with cervical cancer reported in the literature.2,3 However, none of them presented with AFP of the whole lower limb unlike this case. The proposed mechanisms for nLRP associated with pelvic malignancies include direct invasion, compression by lymph nodes and perineural spread along splanchnic nerves from the organs to the lumbosacral plexus.3,4

Award ID: 506

A Rare Case of LGMD2J / LGMDR10 TTN Gene Mutation from India

Aswin Surjit, Boby Varkey Maramattom, Boby Varkey Maramattom, Boby Varkey Maramattom, Hansashree Padmanabha, Ravindranadh Mundlamuri, Sneha Kamath, Boby Varkey Maramattom, Kamalesh Chakravarty, Vivek Lal, Parampreet singh kharbanda, Sucharita Ray, Aastha Takkar Kapila, Abdul Qavi, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Abdul Qavi, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Abdul Qavi, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Saraf Udit Umesh, Sudheeran Kannoth, Vivek Nambiar, Siby Gopinath, Anand Kumar Anandakuttan, Annamma Mathai

Internal Medicine, Aster Medcity, Kochi, Kerala, India

E-mail: surjitaswin@gmail.com

Background and Aim: TTN gene defect was first described in a Finnish family and later in Romania, Hungary and China. It is a rare autosomal recessive condition causing Tibial Muscular Dystrophy, Cardiomyopathy and Limb Girdle Muscular Dystrophy. Here we report a case of Limb Girdle Muscular Dystrophy due to TTN mutation (Titinopathy). Methodology: A 45 year male born of non-consanguineous marriage came with complains of fatigue and progressive lower limb weakness for past 8 years. He underwent L4 – S1 laminectomy 12 years back. His maternal uncle had possible similar illness. On examination he had bilateral lower limb proximal muscle weakness, DTR reduced and no sensory involvement. Investigations - CPK 1671 U/L. EMG showed irritable myopathic potentials. MRI reported atrophy of bilateral paraspinal muscles. Muscle biopsy showed scattered atrophic fibers with multinucleation. Result: He was evaluated for inflammatory muscle disease, metabolic disorder and genetic evaluation of LGMD. Mitochondrial genetic testing and metabolic screening was negative. The whole exome sequencing showed two heterozygous variants in the gene TTN (OMIM*188840) - Variants of Unknown Significance. ECHO showed borderline LVH. Conclusion: Titin is a major component of muscle sarcomere affecting skeletal and cardiac muscles. LGMD2J is a very rare condition and varied outcomes ranging from slow progressive muscle weakness to sudden respiratory depression. The patient could present with features of inflammatory muscle disorders or metabolic storage disorders. The evaluation of genetic profile will confirm the diagnosis. The patient should also be evaluated for cardiomyopathy and respiratory muscle depression.

Award ID: 507

A Clinical Study of Assessment of Cognitive Functions in Multiple Sclerosis Patients in a Tertiary Care Centre

Esther Rani, Lakshmi Narasimhan Ranganathan

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: estherrani.p@gmail.com

Background and Aim: Cognitive dysfunction affects 40–65% of multiple sclerosis (MS) patients and can occur in the early stages of the disease. This study aimed to study the prevalence of cognitive impairment by means of assessing Addenbrooke’s Cognitive Examination (ACE-III) Paced auditory serial addition test (PASAT 3), trail making test (part B) Methodology: Observational study. Inclusion criteria: 1. 18 or older, 2. clinical diagnosis of Multiple Sclerosis by revisedMc Donald criteria (2017) in the recent past,3. primary education Mini Mental State Examination (MMSE) score ≥ 26. Exclusion criteria: 1. MS patients in relapse, receiving pulse steroids; 2. patients with severe motor disability; severe visual impairment; 3. hypertension, diabetes mellitus, connective tissue disorders, thyroid, renal or hepatic impairment, major psychiatric illness; Result: 30 patients with recently diagnosed Multiple sclerosis were assessed for cognitive impairment. The prevalence of cognitive impairment was (18 out of 30) 60%. Their age 25 to 45 years with a mean age 34.6 ± 5.9 years. Male : female ratio (0.76). Seventeen (56%) received primary education, ten (33.3%) received secondary education, and three received university education (10%). ACElll showed impairment in ACE scores of attention (33%) memory (40%) and language (60%). Paced auditory serial addition test (PASAT 3) to evaluate working memory and information-processing speed (23%) and trail making test (part B) to evaluate executive functions (16%) were less affected. Conclusion: Cognitive domains which were observed to be affected in our patients were attention, memory, language with relative preservation of visuospatial, working memory, and executive functions. MS-related cognitive dysfunction results from domain-specific disconnection phenomena. Reduced functional connectivity between cortico-cortical and cortico-subcortical cognitive processing regions Detecting impairment of cognitive functions at an early clinical stage of disease could be useful to promptly enroll MS patients in targeted rehabilitation.

Award ID: 508

Long-Term Surgical Outcome and Utility of FDG PET in Presurgical Evaluation of MRI Negative Frontal Lobe Epilepsy

Rutul Shah, Shameer Aslam, Ramaiah Rajeshkannan, Sumana Pallegar, Siby Gopinath, Ashok Pillai

Neurology, Amrita Advanced Epilepsy center, Amrita Institute of Medical Sciences, Kochi, Kerala, India

E-mail: rutul0076@gmail.com

Background and Aim: Presently, MRI negative frontal lobe epilepsy represents one of the most challenging frontiers in epilepsy surgery.1,2 This study describes the surgical outcome, histopathological findings, and utility of FDG PET in MRI negative frontal lobe epilepsy Methodology: This retrospective study included 35 drug-resistant MRI negative epilepsy patients from a single centre who underwent frontal lobe resection for epilepsy following invasive EEG monitoring. The patients were followed up for at least 12 months postoperatively, and the seizure outcome was classified using Engel’s classification of postoperative outcome. Histopathological examination was done for all the patients and was compared to the surgical results of the patients. Correlation of Brodmann area brain atlas mapping between post-resection MRI and pre-resection PET was done in 19 patients Result: Eighteen patients (54%) reported Engel class 1 seizure-freedom. Four (12%) had Engel class 2 outcome, 6 (18%) had Engel class 3, and 5 (15%) had Engel class 4 outcomes. Two patients were lost to follow-up. Twenty-two patients (63%) showed features of focal cortical dysplasia. Of the seizure-free patients, 13 (76.5%) had histological evidence of dysplasia, and the remaining 4 (23.5%) had nonspecific reactive gliosis. We observed that the mean percentage of area of PET hypometabolism included in resection was maximum in the patients with Engel 1 outcome. Conclusion: This study suggests that post-surgical outcomes in MRI negative patients undergoing frontal lobe resection were better when the maximum area of PET hypometabolism was resected • This study provides data for long-term seizure outcomes in patients with MRI negative FLE

Award ID: 509

Unusual Neurological Infections after Corona Virus (SARS-CoV-2) Infection

Vijay Chenna, Manasa Voodi

Neurology, KIMSICON Hospital, Visakhapatnam, Andhra Pradesh, India

E-mail: ch.vijay22@gmail.com

Background and Aim: Neurological symptoms like anosmia, headache, ageusia, stroke, seizures, and altered sensorium were common with SARS-CoV-2 disease. We aimed to present a report of three cases of rare neurological infections after SARS-CoV-2 infection and discuss the management strategies. Methodology: Three patients aged 60 (X), 23 (Y), and 16 (Z) years visited our out-patient department and were hospitalized in our tertiary care center with a chief complaint of neurological symptoms after SARS-CoV-2 infection. Seizures, ataxia, and altered sensorium were the main symptoms of X, Y, and Z, respectively, and two of them had changes in brain MRI. The median time of recovery from SARS-CoV-2 was 12 days. COVID IgG antibodies were positive, while RT-PCR was negative for SARS-CoV-2 in all three patients. Result: Nocardia (X), Epstein Barr virus (Y), and Human Herpes Virus 6 (Z) were isolated from these patients. X was surgically treated with aspiration of the infection and medical management with Cotrimoxazole. Y had Opsoclonus Myoclonus syndrome. There was a substantial improvement after a pulse dose of methylprednisolone. Z had altered sensorium and was intubated but did not respond to treatment and succumbed to death after one week. Conclusion: This report highlights the importance of immunity derangement after SARS-CoV-2 infection, leading to post COVID infections and may be seen in all ages. Physicians could encounter significant challenges during the diagnosis and management of such uncommon infections.

Award ID: 510

CSF Indices in Autoimmune Encephalitis: Promising Predictor of Treatment Response

Sudheeran K, Ekaterina Popova, Pranav Nair, Annamma Mathai, Sruthi Sasikumar, Siby Gopinath, Vivek Nambiar, Anandkumar Anandakuttan, Udit Umesh Saraf, Jyothi Vikramanpillai Leelamaniamma

Neurology, Amrita Institute of Medical Sceinces, Kochi, Kerala, India

E-mail: sudheeran_k@yahoo.co.in

Background and Aim: No data is available on the use of CSF indices in prognosticating autoimmune encephalitis (AE), so we aimed to study these indices for prognosticating AE Methodology: This retrospective, single-center (AIMS, Kochi, India) cohort study included 60 adult patients with autoimmune encephalitis who were first admitted from August 2016 to November 2021. Patients’ CSF routine findings, oligoclonal bands, and CSF indices- albumin index, IgG index, IgG synthesis rate, IgG local synthesis, and Integrative Parameter of Local IgG Synthesis proposed by Ziadie M. et al were compared with treatment outcome. Result: There were 60 cases (median age-60 years, 30 females,6 sero positives)) A statistically significant association was found between good fast treatment response with a total count of cells in the CSF > 4 cells/mm3 (OR 4.571, 95%CI 1.31–15.956, p = 0.017), IgG Local Synthesis (OR 7.273, 95%CI 1.562–33.863, p = 0.011), and Integrative Parameter of Local IgG Synthesis (OR 5.318, 95% CI 1.271–22.250, p = 0.022). The good fast response was defined as an improvement with a single-agent from the first-line therapy by mRS-9Q of at least 3 points in case of severe disease and at least 2 points in case of moderately severe disease at the time of discharge. Higher Albumin Index values were associated with higher odds of having a poor GCS Score (OR 1.165, 95%CI 1.011–1.343, p = 0,035). Conclusion: Local IgG Synthesis in CNS and CSF cell count > 4 cells/mm3 and Integrative parameter of local IgG synthesis showed association with good and fast treatment response in patients with AE.

Award ID: 511

Acquired Hepato Cerebral Degeneration

Yamuna Gurugubelli, Sundara Chary

Neurology, Guntur Medical College, Andhra Pradesh, India

E-mail: yammi2357@gmail.com

Background and Aim: Acquired Hepato Cerebral Degeneration (AHD) is a N0n-Wilsonian chronic encephalopathy, first reported by van Woerkem in 1914. Liver diseases give rise to variable degrees of neurological impairment ranging from hepatic encephalopathy to a chronic progressive encephalopathy called acquired hepatocerebral degeneration characterised by Parkinsonism, cognitive decline and movement disorders. The disease appears after one or more episodes of hepatic coma or it begins insidiously following a decline in hepatic function. Movement disorders are the most common neurologic manifestations of AHD. About 20% have parkinsonism. Methodology: A 52 year male presented with progressive tremulousness of hands and dizziness followed by unsteadinesss of gait and slowing of daily activities for 4 months. History of cirrhosis of liver on medication. Examination revealed icterus, gynaecomastia, decreased blink rate, action postural tremor. Result: Investigations revealed hyperbilirubinemia, hypoalbuminemia, hypermagnesemia. Endoscopy showed esophaeal varices. MRI imaging showed hyperintensities in bilateral basal-ganglia Conclusion: AHD is an under-diagnosed neurological condition found in many forms of advanced liver disease (independently of etiology), especially those with portosystemic shunting, which are either surgically or spontaneously induced. AHD-related parkinsonism is mostly characterized by symmetric parkinsonism, early gait disturbance or falls, prominent postural or action tremor, mild cognitive impairment, and variable response to levodopa therapy. Manganese deposition in basal nuclei appears to be a key factor in AHD as it has high affinity for DMT1, which is highly expressed in the basal ganglia. Liver tranplantation is mainstay of treatment. Also trientine has been reported to successfully reduce clinical symptoms.

Award ID: 512

“Guitar Pick Sign”: A Valuable Prognostic Radiological Sign in Rhinoorbitocerebral Mucormycosis

Priyanka D, Muralidhar Reddy Y, Subhendu Parida, Shyam K. Jaiswal, Lalitha P, Abhinay Kumar G, Sreekanth Reddy Y, Bharathi Reddy S, Santosh Kumar B, Syed Osman, Murthy JMK

Neurology, Institute of Neurological Sciences, Care Hospitals, Banjara Hills

E-mail: priyankadiddi53@gmail.com

Background and Aim: “Guitar pick sign”, also referred to as posterior globe tenting, is a radiological surrogate marker of tense orbit and profound vision loss. We report a case series of Coronavirus disease - 19 associated rhino-orbito-cerebral mucormycosis (C-ROCM) with “guitar pick sign” and discuss the clinico-radiological features, causative pathomechanisms, severity grade and the clinical relevance Methodology: We studied features of seven orbits with “guitar pick sign” from six subjects of C-ROCM in this prospective tertiary care hospital-based outpatient study carried out between April 2021 and June 2021. Demographics, comorbidities, symptomatology, duration of disease, ocular examination findings, posterior globe angle, degree of proptosis, radiological findings, definitive therapy, vision and life salvage, causes of death were studied. The posterior globe angle and proptosis were measured on T2-weighted axial images of orbit. Result: Mean (range) age of the cohort was 43.6 years (25-60). All had diabetes, and four had renal dysfunction. Four had diabetic ketoacidosis at presentation. All of them presented within a week of symptom onset. Complete ophthalmoplegia, proptosis and absent perception of light in the affected eye were consistent findings in the whole cohort. Four underwent orbital exenteration and extensive debridement of the nasal cavity and paranasal sinuses. While one died awaiting surgery, the family made a shared decision against surgical intervention in the patient with bilateral global tenting. Conclusion: The “guitar pick sign” is a notable radiological finding which flags non-salvageable vision in C-ROCM. This finding is presumably independent of the COVID-19 association.

Award ID: 513

Spinal Dural AV Fistula Presenting as Progressive Paraplegia

Harini R, Saravanan S

Neurology, Tirunelveli Medical College, Tamil Nadu, India

E-mail: harini87@gmail.com

Background and Aim: Spinal Arteriovenous-malformations (AVMs) are rare developmental or acquired abnormal communication of radiculomedullary arteries with enlarged tortuous radiculomedullary veins. We wish to present a case of Spinal AVM presenting as progressive paraplegia. Methodology: 45 year old male patient presented with paresthesia of feet and gait instability for 7 months. Gradually he developed bilateral lower limb proximal muscle weakness over 6 months. 15 days prior to admission, patient had transient back pain and developed complete weakness of both lower limbs and decreased sensations below hips, with no bladder/ bowel symptoms, however he was catheterised in view of inability to walk. On examination, he had hypotonia of the right lower limb with a power of 2/5 and spasticity of the left lower limb with a power of 1/5. On eliciting knee jerk in the right lower limb, there was crossed reflex of left quadriceps. Right ankle jerk was absent. Left knee and ankle jerks were exaggerated. Plantar was mute on right and extensor on left side. Sensations of pain, touch, temperature and vibration were decreased by 80% below the level of L1. Result: MRI of Dorso-lumbar spine with contrast showed Spinal dural AV fistula from D9 to L1 vertebral level with cord edema, possibly due to venous hypertensive myelopathy Conclusion: Dural AV fistula is the most common among spinal vascular malformations, with an incidence of five patients per million. Dural AVFs are frequently misdiagnosed as they can mimic myelitis. Spinal AVMs are rare, but treatable cause of progressive paraplegia, hence timely diagnosis is important.

Award ID: 514

Study Of Post Stroke Cognitive Impairments (PSCI) and the Effect Yoga on PSCI

Manshi Kashyap, Nirendra Kumar Rai, Ruchi Singh, Ankur Joshi, Abhijit Rozatkar

Neurology, AIIMS, Bhopal, Madhya Pradesh, India

E-mail: kashyapmansi58@gmail.com

Background and Aim: Post Stroke Cognitive Impairment (PSCI) is a clinical entity that encompasses all types of cognitive impairment following an index stroke1. Yoga has been reported to have beneficial effect on risk factors of stroke and cognitive functions2,3. Hence, we aimed to study the spectrum of PSCI, and to assess the effect of yoga on PSCI in recent ischemic stroke. Methodology: It was a hospital based open-label, randomised, control study. Patients with age ³18 years, with ischemic stroke within past 1 month, and Modified Rankin Scale (MRS) ≤ 3 were included. With block randomisation, 40 patients each, were assigned to Yoga Arm and Control Arm. Routine standard of care was given to Control Arm, while for Yoga Arm, in addition, structured yoga therapy was provided for 6 months. Cognitive assessment was done at baseline and then repeated at 6 months with help of validated tools- Montreal Cognitive Assessment (MOCA), Frontal Assessment Battery (FAB), Clinician rated Dimensions of Psychosis Symptom Severity (CDPSS). In addition, Caregiver Burden Scale (CBS), MRS, and cognitive evoked potential (P300) were also assessed at baseline and at 6 months. Primary Outcome was defined as change in MOCA or FAB by 2 points. While secondary outcomes were MRS, CDPSS, CBS and P300. Result: Median MOCA score was significantly higher in Cases (25.5, IQR 22-27) than in Control group (24, IQR 20-25.75, p < 0.05). Median FAB score was also higher in the Cases (15.5, IQR 14-17) than in Control group (14, IQR 12-15.75, p < 0.05). Improvement in median CDPSS score was significantly better in the Yoga Arm than the Control Arm (p < 0.05). However, improvement in the MRS score and CBS score was not statistically significant. No significant difference between both the groups was seen in the p300 latencies. Conclusion: The spectrum of Post Stroke Cognitive Impairment includes deficits in multiple domains, including visuospatial, language, naming, programming, conceptualisation, mental flexibility, sensitivity to interference and inhibitory control. Our study shows that early yoga intervention in stroke survivors cam significantly improve their cognitive abilities.

Award ID: 515

Success of Day Rehabilitation Program for Young Stroke Survivors in Community Reintegration

Ping Kwan Ng, Po Wan Polly Chan, Chui Ming Doris Lam, Wai Lok Cheung, Tsz Ying Joyce Cheung

Occupational Therapy, Hospital Authority, Hong Kong

E-mail: npk477@ha.org.hk

Background and Aim: Stroke is often considered as an aging disease; however, around 30% of stroke admissions were adults aged below 65 according to Hospital Authority, Hong Kong (2016). Day Rehabilitation Management (DRM) program, a multi-disciplinary day rehabilitation program, of our hospital has filled the service gap for limited resources designated for young age neuro-rehabilitation and promoted early discharge. Occupational Therapy service in DRM has strived to achieve a seamless training program for young stroke patients from in-patient to community reintegration. ObjectivesTo review the functional outcomes in DRM of Tuen Mun Hospital in Hong Kong. Methodology: Stroke patients aged below 65 discharged from DRM from 1 April 2021 to 30 September 2021 were reviewed. Outcome measures included Modified Barthel Index (MBI), adapted Lawton IADL, Functional Test for the Hemiplegic Upper Extremity (FTHUE), Action Research Arm Test (ARAT), Nine Hole Peg Test (9-HPT) and cognitive function by Montreal Cognitive Assessment Hong Kong Version (HK-MoCA). Result: In the review period, 42 stroke patients (mean age 50.5) were discharged from DRM after receiving average 11.5 sessions. Significantly improvements were found in functional status (p < 0.001 with mean MBI score from 90.9 to 95.0; adapted Lawton score from 12.7 to 16.4), upper limb function (p < 0.05 in FTHUE, ARAT and 9-HPT) and cognitive function (p < 0.001 in HK-MoCA). Among 42 patients, 81.0% (N = 34) were working before stroke. 32.4% (N = 11) of them could directly resume previous job after receiving DRM training in 5.43 weeks. 44.1% of them continued OT rehabilitation in out-patient settings. 20.6% were referred to non-governmental organizations for further rehabilitation and job exploration. Conclusion: The findings showed promising rehabilitative outcomes for OT service in DRM. Seamless rehabilitation was achieved from hospital to early community reintegration which is essential for young stroke patients.

Award ID: 516

Circulating Cell Free Mitochondrial DNA as a Real Time Diagnostic and Prognostic Molecular Tool for Acute Ischemic Stroke

Sandhya Manorenj, Nusrat Fathima, Aleem Ahmed Khan, Nusrat Fathima, Aleem Ahmed Khan, Nusrat Fathima, Aleem Ahmed Khan, Sandeep Kumar Vishwakarma., Rindha Venepally Rao, Reshma Sultana Shaik, Bushra Naaz

Neurology, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Acute Ischemic stroke (AIS) occurs as a consequence of sudden cessation of blood supply to brain. Cascades of pathophysiological events occur leading to release of various neurobiological markers into circulations. Circulating cell-free deoxyribonucleic acid (DNA) has emerged as a potential biomarker of stroke. However their roles remain limited in clinical practice. To determine the role of plasma cell free mitochondrial DNA (cf-mtDNA) measured early after acute Ischemic stroke and its correlation with clinical status of the patients compared to healthy controls Methodology: Case control study, included 44 AIS patients; 44 healthy controls. All patients were subjected to detailed history and examination and received appropriate medical therapy. Severity of stroke was assessed using NIHSS score and functional outcome using modified rankin scale. Plasma cf-mtDNA levels were successively evaluated using quantitative polymerase chain reaction test (SYBR green assay) at onset, 24 and 72 hours. Result: Levels of cf-mtDNA were significantly increased at stroke onset and at 24 hours and 72 hours in AIS patients when compared to healthy controls (P value < 0.0001). This correlated with stroke severity. ROC curve analysis of cf-mtDNA showed significant association, linear response in cases (AIS) when compared to control at onset and 24 hours (69.7% sensitivity & 76.3% specificity; 64.6% sensitivity & 55.9% specificity). Conclusion: Plasma cf-mtDNA levels increases in acute setting of ischemic stroke. The study provides a primitive platform for non-invasive and relatively lower cost molecular tool for diagnosis and prognosis of patients with AIS using cf- mtDNA quantification and validation.

Award ID: 517

Cortical Blindness Owing to Moyamoya Syndrome as a Rare Presentation of Cerebral Rheumatoid Vasculitis

Sandhya Manorenj, Reshma Sultana Shaik, Rindha Venepally Rao

Neurology, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Background Central nervous system affection in rheumatoid arthritis (RA) is rare. The most frequently encountered neurological complications with rheumatoid arthritis are peripheral neuropathy and atlantoaxial subluxation. Cerebral rheumatoid vasculitis (CRV) is an unusual complication and accounts only 1–8%. The incidence of moyamoya syndrome in patients with RA is unclear. Literature of moyamoya syndrome in CRV is meager. We describe a case of moyamoya syndrome due to CRV presenting as cortical blindness in a young male. Methodology: Description of rare presentation of Central nervous system involvement of Rheumatoid arthritis Result: Case presentation A 36-year-old male with history of rheumatoid arthritis for 4 years and not on any disease modifying therapy presented with sudden onset, painless loss of vision of both eyes. Magnetic resonance imaging brain showed bilateral sub cortical parieto-occipital infarcts with gliosis. MR angiography showed bilateral MCA, ACA and left ICA narrowing at supraclinoid region. Digital subtraction cerebral angiography (DSA) showed moyamoya syndrome angiographic pattern as sequelae of intracranial vasculitis. The patient did well with conservative management. Conclusion: CRV is rare, but can lead to grievous damage like blindness in our case and can cause life threatening stroke. Our case showed moyamoya syndrome angiographic pattern with DSA, denting chronic intracranial vasculitis.. Early diagnosis, proper treatment with low dose aspirin and initiation of disease modifying therapy and regular follow-up visits are essential in preventing serious complications

Award ID: 518

Deep Learning Models for Rapid Identification of Bacterial Meningitis Based on Clinical Examination and Information

Donghua Mi, Siqi Chen, Guanghui Zheng

Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China

E-mail: midonghua131@163.com

Background and Aim: Bacterial meningitis (BM) is a neurologic emergency. The mortality of acute BM increases by approximately 30% for each hour of delay in treatment. However, current conventional detection requires 5-10 days of bacterial culture of the cerebrospinal fluid, which is time-consuming and has high false-negative rate. Methodology: We randomly enrolled 8,876 patients who underwent meningitis-related examinations at Beijing Tiantan Hospital between 2015 and 2020, and retrospectively collected total 16 predictors (no significant correlations between them) from clinical laboratory data and electronic health records (EHR). We integrated all predictors with diagnosis from EHR and established a random forest model (predicted target: BM diagnosis) after data cleaning and model screening. Then, each group of text data derived from ICD-10 codes of diagnosis and chief complaint from EHR was transformed through a one-hot matrix transformation into data by Doc2Vec. Finally, combining the data with clinical tests and electronic data, we trained and established a deep neural network (DNN) (predicted target: prognosis). Result: The random forest had great positive prediction value (96.3%), precision rate (93.2%) and well-performed training curve for BM diagnosis (yes/no). The DDN, with a combination of above 16 predictors with new predictors derived from the ICD-10 code by TF-IDF transformation, had great positive prediction value (98.1%), precision rate (98.3%) and excellent-performed training curve for prognosis (death/not). Conclusion: The pioneer study integrated the information from the random forest and the convolutional neural network, to obtain a joint model for rapid detection of BM and prediction of prognosis, with over 98% prediction accuracy.

Award ID: 519

Unusual Sway that Showed us a New Way

T. L. Geetha, Gottipati Bindu Narmada

Neurology, Guntur Medical College, Andhra Pradesh, India

E-mail: geethareddy.tamilisetti@gmail.com

Background and Aim: Episodic ataxias (EA) is a clinically heterogeneous group of disorders that are characterized by recurrent spells of truncal ataxia and incoordination lasting minutes to hours. Most have an autosomal dominant inheritance pattern.8 subtypes have been defined according to clinical and genetic characteristics, and five genes are known to be linked to EAs. Both EA1 and EA2, which are caused by mutations in KCNA1 and CACNA1A, account for the majority of EA, but many patients with no identified mutations still exhibit EA-like clinical features. Methodology: 15 year old female born out of non consanguinous parentage with normal developmental milestones without any preceding illness, presented with complaints of sudden onset of swaying while walking and reeling sensation which lasted for 3 days. Three such episodes over two months. Result: 15 year old female born out of non consanguinous parentage with normal developmental milestones without any preceding illness, presented with complaints of sudden onset of swaying while walking and reeling sensation which lasted for 3 days. Three such episodes over two months. On examination patient had gait ataxia and nystagmus without any motor weakness. MRI brain is normal. genetic analysis showed CACNA1A mutation Conclusion: 1. EA2 is caused by mutations involving CACNA1A on chromosome 19p13, which encodes Cav2.1, the α1 subunit of the P/Q-type voltage-gated calcium channel. 2. Over 60 different heterozygous point mutations have been described in EA2 patients.3. Episodic ataxia to be suspected on clinical grounds with history of recurrent ataxia, slurred speech for several hours, and interictal nystagmus.

Award ID: 520

“Venous Stroke Profile of 238 Patients from a Tertiary Care Center.”

K. Joy Mounica

Neurology, Guntur Medical College, Andhra Pradesh, India

E-mail: joykanakapudi@gmail.com

Background and Aim: • This study was aimed to assess the clinical profile, etiology, radiological findings and treatment outcome of the patients with CVT. Methodology: It is a Hospital based study, done during January 2016-July 2020. Patients presenting with clinical and radiological features of CVT are included in the study and analysed. Result: • In the present study, CSVT is found more frequently in men. 3rd decade is the common age group • Headache (94.95%) is the most frequent and most of the times, the earliest symptom of CVT, followed by vomiting (50%) and seizures (47%). • 14 patients had isolated headache or benign intracranial hypertension like presentation, all of them had transverse sinus thrombosis with no parenchymal involvement. • 3 patients with headache and 6th nerve palsy had transverse venous thrombosis with no parenchymal involvement. • 90% of patients with isolated seizures showed predominant involvement of superior saggital sinus and cortical vein thrombosis. • Papilledema (61.7%) was the most common clinical sign seen in patients with sub acute and chronic presentation but is less common in acute cases. • In our study alcohol was the most common risk factor in males with less than < 40 yrs age followed by hyperhomocystinemia. • In females (<40 yrs) age group postpartum state was the most common risk factor followed oral contraceptive usage. • In patients with > 40 yrs age group - DKA, CNS infections were the common risk factors observed in our study. Superior saggital sinus was the most commonly involved sinus in our study followed by right transverse sinus thrombosis as observed in other studies. In hospital mortality was seen 14 cases, 8 of them had deep venous thrombosis, 6 cases had multi-sinuses involvement. Conclusion: Poor GCS score, deep venous involvement, multi-sinuses involvement was associated with poor outcome.

Award ID: 521

Rare Case of Thoracic Myelopathy Secondary to Ossification of Posterior Longitudinal Ligament

Vinuthna Kodi, M. R. Manivannan, P. K. Murugan, C. Justin

Neurology, Madurai Medical College, Madurai, Tamil Nadu, India

E-mail: drvinnu05@gmail.com

Background and Aim: Ossification of posterior longitudinal ligament (OPLL) is a rare disorder manifested by thickening of the ligament followed by calcification. It is most commonly seen in cervical spine. Involvement of Thoracic or lumbar spine is rare. The index case is a thoracic myelopathy secondary to OPLL Methodology: 45 yr old female known Diabetic, Hypertensive presented with back pain, numbness in both lower limbs, dark incoordination with B/L symmetrical spastic weakness (distal > proximal), decreased sensations below the trunk. On examination patient had pyramidal pattern of weakness with loss of pain, touch, vibration, temperature up to D5 level. Her sensory-motor function and reflexes were normal in both upper limbs and Hoffman’s sign was negative with normal bladder and bowel. Result: Blood investigations were normal. Radio imaging of spine showed ossification of posterior longitudinal ligament compressing the thoracic cord. Radiograph of both forearms showed normal. Patient was taken for surgery and postoperatively patient’s power showed no improvement. Conclusion: It is essential to make a distinction between OPLL and spondylosis because failure to recognize OPLL resulting in inappropriate surgery can exacerbate the situation. Causative factors include diabetes, spondylosis, trauma, irradiation, fluorosis. Mechanical stress leading to increase in prostaglandin I2 was thought to play a role in ossification. Conservative treatment is indicated for patients with mild paresthesia and no evidence of muscular weakness. Early decompressive surgery should be recommended to patients with progressive neurological deficits or when there is compression of the spinal cord.

Award ID: 522

A Rare Case of Stiff Person Syndrome

Yamuna Gurugubelli, Yamuna Rani Gurugubelli

Neurology, Guntur Medical College, Andhra Pradesh, India

E-mail: yammi2357@gmail.com

Background and Aim: Stiff Person Syndrome (SPS) also known as Moersch-Woltman syndrome first described in 1956 with prevalence 1 in 1 million, characterized by progressive rigidity of truncal muscles and proximal lower limbs with superimposed spasms, and an exquisite sensitivity to external stimuli. SPS is commonly associated with high anti-glutamic acid decarboxylase (GAD) antibody titers and other autoantibodies targeting antigens in neurons of the brain and spinal cord at synapses using gamma-aminobutyric acid (GABA), a mechanism underlying the excessive motor neuron firing resulting Co-contractions of agonist and antagonist muscles at rest. Methodology: A 47 year male presented with 4 years duration of insidous onset lower backache with progressive stiffness of both lower limbs at the begiining of walking to start with and later became persistent but absent in sleep, then stiffness of total lower body and abdomen triggered by sudden touch and noise. History of hypothyroidism and left ventricular dysfunction on treatment. History of sudden respiratory arrest during a febrie illnes from which he was resuscitated. There is dramatical response to benzodiazepines. Examination is normal except for hypertonia in all limbs with bilateral babinski positive. Result: Investigations revealed increase in TSH, anti TPO antibodies, anti GAD 65 antibodies with normal imaging and electromyography revealed involuntary firing of motor units. TREATMENT:Benzodiazepines, intrathecal baclofen, Plasma exchange, high dose corticosteroids or IVIG. Conclusion: An extremely rare condition. Can be mistaken for non compressive myelopathy because of hyperreflexia and Babinski. Diagnosis is established by clinical findings with supportive evidence from electrophysiology and serological testing that show elevated anti-GAD antibodies.

Award ID: 523

Transcriptional Activation of Frataxin by Targeting Transcription Factors Associated with Repressor Region: A Plausible Therapeutic Approach in Friedreich’s Ataxia

Vishnu Swarup, Deepika Gupta, Vikas Kumar, Himanshu Narayan Singh, Inder Singh, Achal Kumar Srivastava, Vikash Singh, Achal Kumar Srivastava

Neurology, AIIMS

E-mail: vishnuswarup@gmail.com

Background and Aim: Aberrant transcription is a crucial factor for low frataxin levels causing Friedreich’s ataxia (FRDA). Transcription factors (TFs) binding to cis-acting DNA elements (repressors/enhancers) in FXN gene regulate its expression. Several under-trial drugs have been shown to improve frataxin levels. The aim of the present study is to investigate the interaction between under-trial drugs and TFs binding to the repressor region in FXN gene. Methodology: TFs binding to the repressor region (chr9:69026436-69026607) were identified using footprintDB database. Three drugs under trial were selected from www.curefa.org, an authentic database of therapeutic molecules under trials in FRDA. Three-dimensional structures of drugs and TFs were obtained from the PubChem repository and Uniprot database. Molecular docking simulations (MDS) were performed using Autodock Vina Software. After docking, the structures were examined by Protein-Ligand-Interaction-Profiler (PLIP) webserver. Result: Three TFs, IRF1, NR5A2, Oct1 were identified from footprintDB database and three drugs, dimethyl fumarate (DMF), omaveloxolone, and resveratrol were selected from curefa.org. The MDS results showed that the omaveloxolone binds most strongly to Oct1 (Ka = 3.27x106; ΔG = -9.0 kcal/mol) than NR5A2 (Ka = 1.98x106; ΔG = -8.7 kcal/mol) and IRF1 (Ka = 9.87x104; ΔG = -6.9 kcal/mol). Resveratrol (Ka = ~104 mol-1) and DMF (Ka = ~102 mol-1) showed low affinity towards all TFs. Omaveloxolone and resveratrol also interact with multiple amino acids in TFs. Figure-In-silico interaction of three TFs with selected drugs. Conclusion: In this maiden in silico study, under-trials drugs were investigated for their interactions with TFs. The strongest interaction of omaveloxolone than resveratrol/DMF to TFs at multiple locations poses its highest potential as therapeutics. Further, in vitro work is required to validate these results.

Award ID: 524

Longitudinally Extensive Transverse Myelitis in Tuberculous Meningitis

Mohamed Saleem EK, Shobana N, Sadeeshkumar V, Selvakumar CJ, Joby Thomas Mammen

Department of Neurology, Government Medical College, Coimbatore, Tamil Nadu, India

E-mail: drsaleemek@gmail.com

Background and Aim: Mycobacterium tuberculosis (MTB) is a very rare cause of Longitudinally Extensive Transverse Myelitis (LETM)(1). Tuberculous meningitis (TBM) is considered the most severe of all tuberculous presentations (2). The rare complication of transverse myelitis affects approximately 5 per million people, and its association with TBM goes largely under reported (3) Methodology: A 32 year old female, diagnosed as a case of TBM by CSF analysis (Gene-Xpert) and MRI (basal meningeal enhancement) who was initiated on ATT and tapering steroids from elsewhere, developed speech impairment, altered behaviour, lower cranial nerve palsies and quadriparesis after one month of initiation of treatment. Imaging showed CSF collection around cerebellum causing mass effect on CV junction following which patient underwent foramen magnum decompression from the same hospital. Post procedure patient improved clinically (MRS-3/6) and patient was continued on ATT. After 6 months the patient had worsening of the symptoms. MRI brain revealed multiple tuberculoma in bilateral cerebral hemispheres and MRI spine showed LETM extending from CV junction to D3 segment. Repeat CSF analysis showed a high protein and lymphocytic pleocytosis. Patient’s NMO and MOG antibody assays turned out to be negative. Subsequently patient got admitted in our facility (with a MRS score of 5/6 and muscle strength of 1/5). Later patient was started on CAT-2 ATT and intravenous steroids following which clinical improvement was noticed. Result: Conclusion: LETM is a rare complication of TBM. The pathogenesis can be due to direct infection of MTB or immune response (4). Modified ATT regimen and long term steroids help in recovery.

Award ID: 525

A Novel Presentation of NBIA in Neurology Department of a Tertiary Care Centre in South India

Ajith Thampi, Saravanan Sankaralingam

Department of Neurology, Tirunelveli Medical College, Tamil Nadu, India

E-mail: drajithdavidthampi@gmail.com

Background and Aim: A disturbed brain iron metabolism may damage brain and trigger disorders known as Neuronal brain iron accumulation (NBIA). Pantothenate kinase-associated neurodegeneration (PKAN) is an autosomal recessive disorder caused by mutation in the PANK2 gene. Methodology: A 51-year-old gentleman who was a known case of moderate intellectual disability and seizure disorder presented with abnormal involuntary movements of neck and both hands for the last 8 months, slowness of activity and speech disability for 6 months. He had a significant family history of retinitis pigmentosa in older brother and younger brother having intellectual disability with seizure and committed suicide. He had dysarthria, generalized dystonia, oromandibular dystonia as well as the presence of hyporeflexia and spasticity of the lower limbs, with flexor plantar and extra pyramidal features. Tests for strength, coordination and sensitivity were normal. Neuro-ophthalmologic exam was normal. Serum ferritin was decreased, and MRI showed decreased intensity in globus pallidus, substantia nigra and dentate nuclei supporting the clinical diagnosis. Result: The differential diagnosis considered was PKAN and its variants and HARP. Considering the late age of onset and different Phenotypic presentations in 3 siblings neuroferritnopathy was considered as the most probable cause of all PKAN types. Conclusion: Genetic evaluation is mandatory for atypical forms of NBIA as their offsprings could be obligate heterozygotes. Carrier testing for at risk family members requires prior identification of pathogenic variants in family.

Award ID: 526

Autonomic Dysfunction and Anemia in a Mouse Model of Prodromal Parkinson’s Disease

Tomoyuki Taguchi, Masashi Ikuno, Hodaka Yamakado, Ryosuke Takahashi

Neurology, Kyoto University Graduate School of Medicine, Japan

E-mail: riverotter@kuhp.kyoto-u.ac.jp

Background and Aim: Parkinson’s disease (PD) is one of the most common movement disorders. Along with typical motor symptoms, patients with PD exhibits a variety of prodromal non-motor symptoms such as constipation, frequent urination, and orthostatic hypotension. Anemia is also reported in patients with PD and subjects with multiple prodromal symptoms of PD. Methodology: We have previously reported a mouse model of Parkinson’s disease (A53T-SNCA-BAC Tg mouse), which exhibits the olfactory dysfunction and RBD-like symptom. In this study, we investigated whether A53T-SNCA-BAC Tg mice have signs and symptoms observed in prodromal PD patients. Result: There is a decrease in the number of red blood cell and hemoglobin in A53T-SNCA-BAC Tg mice from 7 months of age. A53T-SNCA-BAC Tg mouse also showed an increased whole gut transit time by at least 9 months old. Pathological analysis showed that proteinase K (PK)-resistant phosphorylatedα-synuclein (α-syn) was accumulated in the Auerbach›s and Meissner›s plexus of the intestinal tract. Moreover, accumulation of PK-resistant phosphorylated α-syn was also observed in the adrenal medulla, salivary gland, and lacrimal gland, which are under autonomic control. Conclusion: A53T-SNCA-BAC Tg mice showed anemia and decreased intestinal motility associated with phosphorylated α-syn accumulation. The abnormal accumulation of α-syn in glandular tissues such as salivary gland and lacrimal gland suggests the importance of pathologies of the autonomic nervous systems and is now attracting attention as peripheral biomarkers of PD. The relationship between anemia and systemic α-syn accumulation is now under investigation.

Award ID: 527

PARKIN Variant, Early Onset of Parkinson’s Disease (PD): A Study among Eastern Uttar Pradesh (UP) Population

Surajit Malakar, Parimal Das

Center for Genetic Disorders, Banaras Hindu University

E-mail: surajitmalakar92@gmail.com

Background and Aim: PARKIN (PARK2) located on chromosome 6q26, is a recessive gene associated with juvenile and early onset of Parkinson Disease (PD), the frequency of PARKIN mutation may be as high as 49% while the reported range is 15% to 18% in patients without familial history of PD. This PD associated gene spans approximately 1.38 Mb and encodes for an E3 ubiquitin ligase, PARKIN of 456 amino acids long, helps in mitochondrial maintenance and autophagy of dysfunctional mitochondria [1][2]. The present study is to investigate and in silico characterization of variants on PARKIN gene in early onset of PD patients from eastern part of Uttar Pradesh (UP), the prior knowledge of PARKIN as an important candidate gene for PD is necessary to be study within a population. Methodology: A total 50 unrelated subjects of age group 30 to 50 years from eastern part of UP diagnosed as PD was enrolled for study. Intravenous blood was collected from the patients with informed written consent. DNA was isolated from the blood samples and sequencing of all exons of PARKIN gene was done by Sanger Sequencing method, followed by in silico characterization of variant. Result: A missence variant c. G928A on PARKIN gene was identified in one of the early onset PD patient, which found to destabilise the secondary structure of mRNA and tertiary structure of protein. Conclusion: The PARKIN variant observed in our study produces a misfolding protein of lower stability, prevent autophagy of disfunctional mitochondria and accumulation of Reactive Oxygen Species result in death of Dopaminergic neurons leading to PD pathophysiology.

Award ID: 528

A Case of Early Infantile Epileptic Encephalopathy

Amarendhar Malreddy, Bindu Narmada

Neurology, Guntur Medical College, Andhra Pradesh, India

E-mail: amarendhar48@gmail.com

Background and Aim: Epileptic syndromes at early infantile onset are electroclinical syndrome in which seizures unexpectedly arose between the fourth and sixth days of life. The EEG picture often shows a characteristic burst-suppression pattern. KCNQ2 related encephalopathy where Partial seizures unexpectedly begin by the third day of life. Early infantile epileptic encephalopathy (EIEE), also known as Ohtahara syndrome, is characterized by intractable spasms often consist of tonic forward flexion often occurring in clusters in the setting of a severe encephalopathy and a burst-suppression background pattern on EEG. Methodology: 14 months male born of non consanguineous parentage presented with seizures from second day of birth with sudden tonic postures, EEG showed burst-suppression pattern with normal imaging. Genetic analysis confirmed KCNQ2 mutation Result: 14 months old male born of non consanguineous parentage presented with seizures from second day of birth with sudden tonic postures lasting for less than 1 min. He was on multiple anti-epileptic drugs, inspite of which child had seizure recurrence of 8-10/day with occasional seizure free days in between. On examination child is conscious and irritable but doesn’t make eye contact, no neurocutaneous markers, no focal motor deficits. EEG showed burst-suppression pattern with normal imaging. Genetic analysis confirmed KCNQ2 mutation. Conclusion: Ohtahara syndrome is a rare clinico-EEG syndrome. The EEG picture often shows a characteristic burst-suppression pattern. Gold standard– genetic analysis. Refractory to any mode of treatment including ketogenic diet and surgery. Highest mortality rate.

Award ID: 529

Atypical Presentation of Disseminated TB as CNS Demyelination

Jaberlin Sneha

Neurology, Tirunelveli Medical College and Hospital, Tamil Nadu, India

E-mail: jaberlinsneha11@gmail.com

Background and Aim: CNS involvement in TB is considered as a devastating form of TB comprising 10% of TB cases and caries risk of permanent neurological sequalae and mortality. Methodology: We report a case of 33 years female, known case of muscular dystrophy (LGMD2A), presented as insidious onset progressive double vision on looking to right with B/L painless blurring of vision of 1 month duration. Physical examination revealed right lateral rectal palsy, VA Rt (6/6) and Lt (6/9), Normal fundus without fever and neck rigidity. She has a power of 3 in all four limbs owing to her pre-existing illness. MRI brain showed hyper intensities in cortex, medulla and pons with diffusion restriction suggestive of demyelination/Infarction. With the clinical history and distribution of radiological lesion we considered demyelination as first probability and started the patient on steroids with which she showed improvement. After a week, she developed severe disabling back pain. MRI LS spine showed TB spondylodiscitis at D12-L1 level with epidural abscess compressing conus medullaris, suggestive of Potts spine. CT chest, sputum AFB, CSF analysis were unremarkable. she was started on ATT and steroid was continued. Patient showed considerable improvement in her symptoms with follow up imaging showing reduction in the size of lesions. Result: TB has varied presentation and can mimic numerous other conditions including demyelination. Conclusion: Rarity of presentation as concomitant non -meningeal cranial and Spinal TB without pulmonary involvement in an immunocompetent individual pose significant diagnostic dilemma.

Award ID: 530

To Study the Effect of Immunotherapy in Antibody Negative Patients with Clinical Syndrome of Autoimmune Encephalitis in Comparison with Seropositive Cases in Tertiary Level Institute Between 2019 – 2021

Sruthi Sasikumar, Sudheeran Kannoth, Anand Kumar, Siby Gopinath, Vivek Nambiar, Saraf Udit Umesh, Annamma Mathai

Neurology, Amrita Institute of Medical Sciences

E-mail: sruthianoop1991@gmail.com

Background and Aim: Autoimmune encephalitis refers to a collection of inflammatory central nervous system illnesses that are linked to neuronal autoantibodies or other central nervous system autoimmune indicators. Despite the advancement of Autoimmune Encephalitis diagnostic technologies, knowledge of antibody-based diagnosis is limited. primary objective of my study is To determine the number of cases which are antibody negative but fulfilling the clinical diagnostic criteria of autoimmune encephalitis and to analyse the outcome of patients who were antibody negative but were still initiated on immunosuppressive measures as per the current treatment guideline for antibody positive autoimmune encephalitis. Methodology: This is a prospective study of patients with clinical evidence of autoimmune encephalitis conducted at AIMS hospital, a tertiary referral hospital, in South India, between August 2019 to September 2021. The study was approved by the AIMS ethics committee. Informed consent was obtained from all patients when they had full sensorium or from a family member, parent, or guardian, if impaired. Result: In this study, we analysed a total of 52 patients who fulfilled the clinical criteria for diagnosis of autoimmune encephalitis. Antibody levels were tested for all and we assigned into seronegative and seropositive groups. There were a total of 16 seropositive cases and remaining were negative in the selected time frame of the study. We have shown that seronegative has equal and slightly greater prevalence in our center. All the parameters we used, including seizure frequency, behavioural changes, 9qMRS scores, MMSE and GCS assessment all showed an improvement which was non-inferior to the improvement shown in the antibody positive cohort. Conclusion: Here we show in this data from a single center trial, that the encephalitis previously classified as idiopathic can be attributed to an immune mediated aetiology. Early diagnosis using clinical criteria, MRI and CSF to rule out infection is important. Early diagnosis can help in early initiation of treatment, which has produced good outcomes. The current guideline which gives undue importance to presence and detection of antibodies need to be changed.

Award ID: 531

Sleep and Headache Characteristics in Patients with Idiopathic Intracranial Hypertension (IIH) - An Observational Study

Vaishali Sharma, Kamalesh Chakravarty, Vivek Lal, Parampreet Singh Kharbanda, Sucharita Ray, Aastha Takkar Kapila

Neurology, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India

E-mail: svaishali1996@gmail.com

Background and Aim: Background - IIH is characterized by elevated intracranial pressure without an identifiable etiology. Patients with IIH frequently have headaches and co-existing sleep abnormalities. Aim- To assess the sleep and headache characteristics in patients with IIH. Methodology: 51 cases enrolled. Baseline clinical characteristics were noted. Assessment of headache was done using HIT-6 and MIDAS questionnaire. Assessment of sleep was done using the Berlin questionnaire, Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS), and RLS scale for restless leg syndrome. Result: The mean age of patients was 33.72 ± 9.5 years and BMI 27.32 ± 5.99 kg/m. 84.31% were female. Mean headache frequency was 14.9 ± 9.9 days/month, and headache severity (VAS) was 7.02 ± 2.28. 37.25% patients had a holo-cranial headache, followed by Temporal-21.57%, Frontal-15.69%, Vertex-5.89%, and Occipital-1.97%. 17.65% patients had no headache at all. Headache was majorly throbbing (60.8%), followed by dull aching (15.7%) and pulsating (5.9%). Transient vision obscurations (TVO’s) were present in 51% of the patients and only one patient was presented with Aura. The mean CSF opening pressure was 24.5 ± 8.37 cmH20. For headache, MIDAS and HIT-6 scores were calculated which were 10.9 ± 21.9 and 48.92 ± 13.89 respectively. The mean ESS score was 6.0 ± 3.9. 47.05% patients reported poor sleep quality (PSQI score - ≥5), 60.78% patients had no RLS, 23.53% patients had mild RLS, 11.77% patients had moderate RLS, 1.97% patients had severe RLS, and 1.97% patients had very severe RLS. According to Berlin questionnaire, 31.37% patients had high-risk for OSA and 21.57% had low-risk for OSA. Conclusion: Patients with IIH suffer from moderate to severe headaches and also have a higher association with obstructive sleep apnea.

Award ID: 532

Study of Sexual Dysfunction in Parkinson’s Disease

Chennappan

Department of Neurology, Dhanalakshmi Srinivasan Medical College, Perambalur, Tamil Nadu, India

E-mail: drchenni78@yahoo.co.in

Background and Aim: BACKGROUNDParkinson’s disease (PD) is a movement disorder. Apart from motor manifestations nonmotor symptoms are also present, but are not well recognised unless specifically enquired. Among nonmotor manifestation sexual dysfunction is one of the most common autonomic disorder. It is well recognized in Parkinson’s disease (PD). However the incidence of this condition is not well established. AIM: To Study the incidence of sexual dysfunction in patients with PD Methodology: This is a case control (questionnaire based) study. 100 patients with PD and 50 age matched controls were included in this study. Questionnaire based on Sakakibara R et al study regarding the three sexual dysfunction was applied to the patients and controls. The findings were compared with age matched control subjects. All PD patients were on levodopa without dopamine agonists. The questionnaire assessed the sexual function (three questions for women, five for men). Each question was scored from 0 (none) to 3 (severe) with an additional quality of life (QOL) index scored from 0 (satisfied) to 3 (extremely dissatisfied). Result: Sexual dysfunction PD (M, F) Control (M, F) Decreased libido 72% (55, 17) 18% (8, 10) Decreased intercourse 79% (61, 18) 35% (23, 12) Decreased orgasm 54% (42, 12) 9% (5, 4) Decreased erection 59% 10% Decreased ejaculation 50% 9% Conclusion: CONCLUSION This study showed that sexual dysfunction are present in majority of patients with PD and often under reported. The symptoms of sexual dysfunction should be specifically sought for during assessment and effectively treated in view of improving the quality of life.

Award ID: 533

A Rare Case of Disseminated Neurocysticercosis

Balapradeep Boyidapu, Sivakumar S, Thiruvarutchelvan K, Chandrasekharan P, Muthukumaran D

Neurology, Government Mohan Kumaramangalam Medical College, Tamil Nadu, India

E-mail: drbalapradeep@gmail.com

Background and Aim: Background: The most common parasitic infection causing focal lesions in the CNS is neurocysticercosis (NCC) caused by Taenia solium. The most common presentation is seizures, although stroke, vision loss, cognitive decline may be seen. We have encountered a rare case of NCC presenting with vision loss with ocular cysts and multiple larval cysts in muscle. Methodology: Case summary: 27 yr old lady presented with diminished vision in left eye since 3 months, insidious onset, gradually progressive, painless, not associated with double vision, headache or fever. H/O recurrent seizures for 2 yrs, diagnosed as NCC for which she took albendazole, steroids short course and is on antiepileptics till present. O/E conscious, oriented, Higher mental functions normal. Pupils-right-3mm RTL, left-5mm RAPD, EOM equal b/l in all directions. Fundus-Right-normal, Left-pale disc with inferior subtotal retinal detachment, vision-right eye 6/6, left eye-HM+ MRI brain-diffuse thickening of retino-choroidal lining of left eyeball with ring enhancing lesion s/o left ocular cysticercosis. Innumerable thin-walled cystic lesions containing eccentrically placed scolex seen in b/l cerebral cortex, basal ganglion, thalami, pons and midbrain with surrounding vasogenic edema s/o disseminated neurocysticercosis in vesicular and calcified stages. MRI muscle s/o multiple intramuscular T2 hyperintense cystic lesions entire muscle in both thigh s/o NCC of both thigh. Result: Discussion: NCC can present as solitary or multiple cystic lesions in brain, muscles and other organs. This patient has visual loss due to cyst in retina which is a rare cause of uniocular vision loss. Conclusion: Conclusion: In the background of NCC, if patient has visual loss, intraocular cysts should be diligently searched as a cause of blindness.

Award ID: 534

Evaluation of Risk Factors Associated with Severe Ischemic Stroke: An Institution Based, Analytical Cross-Sectional Study

Ananya Sengupta, Krishnendu Roy, Anirban Dey

Neurology, Institute of Neurosciences, Kolkata, West Bengal, India

E-mail: annesgpt@gmail.com

Background and Aim: Stroke is a leading cause of mortality and the most important cause of permanent disability globally. Ischemic stroke accounts for majority of such cases. We aimed to evaluate risk-factors associated with severe ischemic stroke. Methodology: We included 100 patients presenting with acute ischemic stroke within 24 hours of symptom-onset. Each patient was subjected to detailed history regarding demography and examined for co-morbidities- diabetes mellitus (DM), hypertension and obesity. Biochemical investigations included mean platelet volume (MPV), platelet count and platelet distribution width (PDW), measured within 24 hours of admission. mRS score was calculated on day-5 to identify severe stroke (mRS > 3). Result: Among 100 patients with acute ischemic stroke [median (IQR) 65 (56-68.5) years, M:F 1.7:1), 31% (n = 3) had severe stroke. Advanced age (p = 0.02), higher BMI (p < 0.001), DM (p = 0.0004) and hypertension (p < 0.0001) showed a significant association with severe stroke. Mean ± SD platelet count was significantly reduced in severe stroke, compared to non-severe stroke (1.8 ± 0.9 vs. 2.9 ± 1.2, p = 0.03). In contrast, mean ± SD values of MPV (fL) and PDW (fL) were higher in severe stroke, none being statistically significant (p’s 0.3 and 0.1 respectively). Multivariate regression analysis revealed DM (AOR 6.02 [95% CI 1.1-32.1], hypertension (AOR 9.2 [95% CI 2.3-37.3] and high PDW (AOR 7.3 [95% CI 1.5-35.3] to be significant risk-factors for severe stroke. Conclusion: Patients with diabetes, hypertension and high PDW need regular screening to prevent severe ischemic stroke. This would facilitate optimum utilization of available health resources.

Award ID: 535

Iron Deficiency is Significantly Higher in Post-Partum CVST Patients, Cause or a Bystander?: A Retrospective Study from a Tertiary Care Center

Ashutosh Tiwari, Ishita Desai, Mritunjai Kumar, Niraj Kumar

Neurology, AIIMS Rishikesh, Uttarakhand, India

E-mail: ashutiwari19@gmail.com

Background and Aim: This study was planned to find pattern and frequency of risk factors and causes of Cerebral sinus venous thrombosis (CSVT) in Indian patients. Methodology: This is a single center, retrospective study involving patients with diagnosis of CVST, admitted at a tertiary care university hospital in Northern part of India, from May 2018 to May 2020. Risk factors, clinical features, investigations and outcomes were collected and analyzed. Result: Thirty-six patients with CVST with mean age 31.94 (range 20-70) years were included. Among younger subgroup of patients (< 37 years), females preponderance was noted (female 71% vs 68%, p = 0.02). Gynaecological cause was seen in 11 (30.5%) (Including 25% in puerperium and 5.5% on oral contraceptives), hyper-homocysteinemia in 13 (36.2%) and iron deficiency anaemia in 9 (25%). Comorbidities such as TB, dengue fever, ischemic heart disease, hypothyroidism, connective tissue disorders and stroke, were seen in 11 (30.5%) patients. Thrombophilia was observed in 7 (19.4%) patients. iron deficiency was significantly higher in postpartum group compared to non-post-partum group (88.9% vs 18.5%, p < 0.001). Cut-off value for iron deficiency was < 60 mcg/dL (normal = 60 to 170 mcg/dl). Conclusion: Iron deficiency may be an additional preventable risk factor in post-partum CVST cases.

Award ID: 536

Pregnancy Outcomes Following Cortical Venous Thrombosis

Atif I. A. Shaikh, Deepti Bal, Sanjith Aaron

Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India

E-mail: dr.atifshaikh@gmail.com

Background and Aim: Pregnancy is a pro-thrombotic state and in patients who had cortical venous thrombosis previously, subsequent decision to plan pregnancy can be difficult. We planned this study to aid decision making in patients who already had cortical venous thrombosis and wish to plan pregnanc Methodology: All female patients who were diagnosed to have cortical venous thrombosis in 15 years from Jan 2003 to Dec 2017 were included. Complete records with antenatal details were followed up Result: A total of 575 patients were screened. 74 new pregnancies occurred in a total of 50 patients. 26 [52%] of patients had developed CVT in the post-partum period, and nine patients [18%] had developed CVT during pregnancy. Mean age of patients was 25.06 years [18-36]. 30 patients [60%] had normal deliveries, with no maternal or fetal complications.17 patients had another known risk factor for thrombosis. 38 patients [76%] patients received anti-thrombotic prophylaxis. Four patients had major bleeding. 2 patients had fetal loss, and 1 patient had 2 fetal losses. 3 patients had intrauterine growth retardation and 1 patient had microcephalic child. 5 patients had pre-ecclampsia. 9 patients had a total of 17 abortions, with 1 patient having 5 and two patients having 3 abortions. 2 patients had recurrent cortical venous thrombosis and one patient had deep venous thrombosis. Conclusion: Thrombotic events are uncommon in pregnancy following cortical venous thrombosis. Appropriate thromboprophyaxis is generally safe, although close monitoring is advised

Award ID: 537

Visual Recovery in Patients with Lebers Hereditary Optic Neuropathy (LHON)- A Peek into Future Perspective of Treatment

Prabhjit Kaur, Aastha Takkar, Megha Varshney, Sofia Singh, Aastha Takkar, Basavaraj Tigari, Karthik Vinay Mahesh, Vivek Lal, Kathirvel S, Ramandeep Singh, Paramjit Singh, Amit Kaur, Aastha Takkar, Valliappan Muthu, Kathirvel S, Paramjit Singh, Ramandeep Singh, Vivek Lal

Neurology, Post Graduate Institute of Medical Education and Research (PGIMER)

E-mail: missghotra.ghotra@gmail.com

Background and Aim: Background: The maternally inherited blinding disease Leber’s hereditary optic neuropathy (LHON) is characterized by bilateral sequential acute or sub-acute, painless, loss of central vision typically presenting between 15- 35 years of age along with temporal pallor disc. Currently, there is no effective treatment available but taking vitamins B12 and vitamin E along with CoQ10 medications are being considered for the patients with little or no improvement in visual acuity. Aim: Visual Recovery in patients with LHON – A peek into future perspective of treatment Methodology: A 17/M presented with sudden sequential vision loss (Right > Left) in 15 days with no co-morbidity. Patient had a family history of vision loss in brother who was evaluated as Optic neuritis (Vision loss lasted few days). Similarly, a 20/F presented with sudden onset blurring of vision followed by sequential diminution of vision (Left > Right) in 4-6 weeks. She was a first degree cousin of the above mentioned patient. Examination: Visual Acuity at presentation was 6/60 and 6/12 which further deteriorated to FC at 3 MT and FC at 5 MT. Bilateral discs were temporal pallor with primary optic atrophy. Patient was tested positive for T14484C LHON mutation. Case 2: Visual acuity at presentation was 6/6 and 6/60 which progressively deteriorate to 6/60 and FC at 1 MT. Hypermic Discs with hypertensive retinopathy changes were present bilaterally. She was tested positive for T14484C mutation. Treatment: High dose pulse methylprednisolone (IVMP) was given to both the patients for five days followed by tapering course of oral steroids starting from 60 mg. Result: Improvement in visual acuity was observed at six months upto 6/6 bilaterally in Case 1. In case 2, improvement was observed upto 6/36 and 6/60 at one month follow-up. Conclusion: Though LHON is considered to be a blinding disease, these two cases provide a hope and future perspective for its treatment in young patients.

Award ID: 538

The Novel Mice Model to Investigate the Formation of Glial Cytoplasmic Inclusions (GCIs) in Multiple System Atrophy (MSA)

Tomoyuki Ishimoto, Hodaka Yamakado, Miki Oono, Seiji Kaji, Takashi Ayaki, Takakuni Maki, Shu-ichi Matsuzawa, Ryosuke Takahashi

Neurology, Kyoto University Graduate School of Medicine, Japan

E-mail: tishimoto@kuhp.kyoto-u.ac.jp

Background and Aim: Glial cytoplasmic inclusions (GCIs), which are aggregates containing insoluble alpha synuclein (αS) in oligodendrocytes (OLGs), are pathological hallmarks of multiple system atrophy (MSA). Recent studies have reported that in mice inoculated with synthetic αS fibrils, the accumulation of αS in OLGs is observed long after the accumulation of αS in neurons. This result suggests the possibility of neuronal origin of αS aggregates in MSA, but it is still controversial because immunohistochemical evaluation of the formation of early αS aggregates in OLGs is technically difficult. The aim of this study is to create novel mice model for investigating the GCIs formation in MSA by the sensitive and specific detection of early αS aggregates in OLGs. Methodology: We have generated transgenic mice that express human αS-GFP fusion proteins in OLGs under the control of CNP promotor (CNP-SNCAGFP Tg mice). In these mice the aggregation status of endogenous αS in OLGs can be monitored by GFP dot signals. CNP-SNCAGFP Tg mice were inoculated αS fibrils (N = 18) or PBS (N = 3), and these mice were sacrificed after 1 month, 4 months, and 12 months, respectively. Result: CNP-SNCAGFP Tg mice inoculated with αS fibrils into the striatum started to display GFP dots initially in the processes of OLGs, and the number and size of GFP dots were increased overtime in the centripetal manner. Conclusion: CNP-SNCAGFP Tg mice are useful for detecting the spreading of αS aggregates in the intracellular space of oligodendrocytes and investigating the formation of GCIs in MSA.

Award ID: 539

“Journey of Guillain Barre Syndrome from Pre Pandemic Era to COVID 19 Pandemic and Vaccine Era: A Four-Year Retrospective Study”

Deepinder Maini, Satyan Nanda, Rajiv Anand, Anubhuti Dixit

Neurosciences, BLK Hospital

E-mail: Docdeepk@gmail.com

Background and Aim: GBS is known to get triggered with antecedent infections like Campylobacter jejuni, Zika virus. Our study aims to compare the incidence of GBS cases during pre-pandemic and pandemic/vaccine era; to study the clinical profile of GBS cases in both pre pandemic and pandemic/vaccine era and to compare the disease severity and its outcome in pre-pandemic and pandemic/vaccine era. Methodology: Database of patients diagnosed were taken from MRD of a tertiary care hospital at New Delhi, India from March 2018 till March 2022 covering four-year period with 2018 and 2019 taken as pre pandemic era while 2020 and 2021 taken as pandemic and vaccine era. Result: N = 25 in pandemic/vaccine era while N = 49 in pre pandemic era. Mean duration of hospitalization was significantly higher during pandemic/vaccine era (10.68 ± 6.67 days) compared to pre pandemic era (7.59 ± 3.55 days). There was statistically no significance in age (p = 0.56), gender (p = 0.70), GBS variants (p = 0.40), clinical spectrum, antecedent infection (p = 0.91) HDS on admission and discharge (p = 0.93 & p = 0.52 respectively), respiratory involvement requiring ventilator (p = 0.19), mortality (p = 0.26) in either of the eras. Conclusion: Duration of hospitalization in pandemic/vaccine era was significantly more than that of prepandemic era (statistically significant). There was no significant difference in electroclinical variants, clinical spectrum, respiratory involvement requiring mechanical ventilation, Hughes disability score, and mortality in both eras.

Award ID: 540

POLG-Mitochondrial Disorder – A Case Report

Jyothsna Gedda

Neurology, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

E-mail: jyothsnapavanigedda@gmail.com

Background and Aim: POLG gene participates in the replication and repair of mitochondrial DNA. POLG mutations are the most common cause of inherited mitochondrial disorder with heterogenous phenotypic presentations. We report a case with progressive external ophthalmoplegia with sensory ataxic neuropathy. Evaluation of a case with progressive external ophthalmoplegia, sensory ataxia, peripheral neuropathy and proximal weakness of limbs Methodology: A 42 year old presented with progressive drooping of both eyelids without any double vision/squint since 8 years. Since 4 years he developed unsteadiness of gait with frequent wash basin attacks, slippage of chappals without awareness and cotton wool sensation while walking on ground along with reduced perception of clothes over his legs. His wife noticed stimulus sensitive myoclonic jerks involving upperlimbs. Born out of non consanguineous marriage, his elder sister developed similar complaints of sensory loss in both lower limbs, unsteadiness of gait and drooping of eyelids at an age of 30 years. She is presently bedridden. Result: Neurological examination revealed Bilateral ptosis, Restriction of eye movements in all directions of gaze and normal pupillary reflex, absent DTRs, impaired joint, position and vibration sense in lower limbs and positive Rombergs sign. His metabolic workup and Imaging were normal. In view of the positive family history and multiple axes of the nervous system being involved, a mitochondrial disorder was suspected. Genetic analysis revealed POLG homozygous mutation in exon 7 location. Conclusion: Mutations in POLG, a nuclear DNA gene can cause a quantitative and/or qualitative mtDNA defect. Prenatal diagnosis will possible if the pathogenic variant is known.

Award ID: 541

Adult Polyglucosan Body Disease with CIDP like Presentation-Case Report Dr Chintada Mounika (Pg 2nd yr), Dr. G Butchiraju (Prof-HOD), Dr S Gopi (Associate Preofessor), Andhra Medical College, Visakhapatnam

Mounika Pydi

Neurology, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

E-mail: chinnamouni93@gmail.com

Background and Aim: Adult polyglucasan body disease is caused by a mutation in the GBE1 gene, encoding the glucan (1, 4-alpha-) branching enzyme 1 (GBE). Without this enzyme, glycogen is not synthesized properly, regulation of glycogen synthase is dysfunctional and deposition of amylopectin-like polyglucosan bodies in neural tissue occurs. We report a case of APGB with CIDP Llike presentation. Methodology: A 50 yr old woman presented with progressive weakness of both lower limbs in th form of proximal weakness in difficulty in climbing upstairs and difficulty in getting up from squatting and distal weakness in form of difficulty in walking on heels and toes which started symmetrically along with paresthesias of both lowelimbs in the form of glove and stocking with no bladder and bowel of 2 yrs duration. Result: we excluded causes of chronic neuropathy with protei electrophoresis, ANA profile, Nerve conduction stidies, and nerve biopsy of sural nerve suggestive of adult polyglucosan body disease and evaluated for pathogenic variants in GBE gene through genetic analysis which turned out to be positive. Conclusion: we report a rare case of adult polyglucosan body disease with CIDP like presentation.

Award ID: 542

Cardiac MRI in Duchenne and Becker Muscular Dystrophy

Manu S G, Atchayaram Nalini, Ashita Barthur, Seena Vengalil, Deepak Menon, Saraswathi Nashi

Neurology, NIMHANS, Bengalure, Karnataka, India

E-mail: manumsg@gmail.com

Background and Aim: Cardiac disease is a common manifestation of DMD and BMD. CMRI can be used as a non-invasive technique for early detection of cardiac involvement in DMD and BMD. We sought to determine the prevalence of cardiac involvement in Duchenne and Becker Muscular Dystrophy (DMD and BMD) using Cardiovascular MRI (CMRI) and its association with clinical and laboratory parameters. Methodology: A prospective, observational study was conducted on genetically confirmed 38 DMD and 8 BMD patients, who underwent cardiac MRI between March 2020 and March 2022. Result: Mean age of DMD patients was 10.79 ± 3.03 years, whereas that of BMD was 22.00 ± 6.07 years; mean age of disease onset was 3.6 ± 1.73 years and 15.38 ± 0.29 years in the DMD and BMD group respectively. In CMRI, 23.68% DMD patients and 62.5% BMD patients had Left Ventricular (LV) systolic dysfunction. CMRI revealed late gadolinium enhancement (LGE) of LV wall, suggestive of myocardial fibrosis in 16/38 (42.1%) DMD patients and 5/8 (62.5%) BMD patients, but only 3/46 (6.52%) was symptomatic. Myocardial fibrosis is confined to inferolateral LV wall in early stages, with progressive involvement of LV apex, anterior and septal wall later. Multivariate analysis showed that the only clinical/laboratory parameter associated with myocardial fibrosis in CMRI was a low QRS amplitude in lead V6 in ECG. Age, disease duration, clinical severity, functional status, genotype, serum creatine kinase levels and use of ACE-Inhibitors or steroids showed no association with CMRI findings. Conclusion: There is a high prevalence of subclinical cardiac involvement in DMD/BMD patients which can be detected using CMRI, which helps in initiating early cardioprotective therapy in this subgroup.

Award ID: 543

A Rare Case of Chorea

Sudarshan Reddy

Neurology, Osmania Medical College, Hyderabad, Telangana, India

E-mail: meetdrsudha@gmail.com

Background and Aim: Chorea is a movement disorder, is a common yet challenging problem. Usually due to vascular, hereditary, metabolic or drug induced causes. We describe a 54 year male presented with chorea, dysphagia. Differential diagnosis and treatment will be discussed. Methodology: Chorea, dysphagia in our patient was evaluated. W did blood investigations, imaging, CSF analysis, CAG repeats. Result: We suspected etiologies like Huntington disease, Adult onset alexander disease etc. After extensive evaluation, we found a rare etiology for chorea in our patient. Which is going to be discussed. Conclusion: Chorea needs to be evaluated. Sometimes we may find a rare cause as in this patient.

Award ID: 544

ECG in Duchenne and Becker Muscular Dystrophy

Manu S G, Atchayaram Nalini, Ashita Barthur, Seena Vengalil, Deepak Menon, Saraswathi Nashi

Neurology, NIMHANS, Bengalure, Karnataka, India

E-mail: manumsg@gmail.com

Background and Aim: Cardiac disease is a common manifestation of DMD and BMD. ECG changes in DMD/BMD can be attributed to myocardial fibrosis, fibrosis of cardiac conduction system and impaired cardiac autonomic innervation. We sought to determine the prevalence of various ECG changes in Duchenne and Becker Muscular Dystrophy patients (DMD and BMD) and to analyse its correlation to Cardiac MRI (CMRI). Methodology: ECG of 18 DMD/BMD patients who underwent CMRI between March 2020 and March 2022 were compared with ECG of 23 healthy age-matched children. Result: Mean age of DMD/BMD patients was 12.11 ± 3.92 years, whereas that of control group was 10.52 ± 2.61 years. DMD/BMD patients had tachycardia, short PR interval, prolonged QTc interval, higher QRS amplitude in lead V1 and lower QRS amplitude in Lead V6, irregular rhythm and deep Q waves in inferolateral leads in comparison with age-matched controls. Among DMD/BMD patients, there was significant QTc prolongation among those with myocardial fibrosis in CMRI. The only ECG parameter correlated with CMRI was QRS amplitude in lead-V6, which had an inverse correlation with the extent of myocardial fibrosis in CMRI. Patients with moderate/severe cardiac involvement in CMRI showed characteristic ECG changes corresponding to region of myocardial fibrosis – a. myocardial fibrosis confined to a smaller region in inferolateral wall - moderate elevation in QRS amplitude in V1, moderate reduction in QRS amplitude in V6; b. fibrosis involving larger area in inferolateral LV wall – QRS amplitude in V1 is increased, QRS amplitude in V6 reduced; c. fibrosis spreading to involve the septum and anterior LV wall – appearance of deep Q waves in inferolateral wall. Conclusion: ECG serves as an inexpensive and highly useful complementary tool for cardiac evaluation in DMD/BMD patients.

Award ID: 545

Epidemological Study of Stroke in Tertiary Care Centre, South India

A. Madhuri

Neurology, Osmania General Hosptial, Hyderabad, Telangana, India

E-mail: madhuri2528@gmail.com

Background and Aim: Stroke is defined as “ Abrupt onset of neurological deficient that is attributable to focal vascular cause. stroke is the second leading cause of death and disability worldwide. The estimated prevalence rate range from 84-246 / 100000 in rural and 334 – 424 /100000 in urban areas. Stroke units are predominantly available in urban areas that too in private hospitals. IV and IA thrombolysis are commonly used in India. OBJECTIVE: To study epidemiology and risk factors in stroke patients in Tertiary care centre Methodology: A series of 560 patients with stroke admitted in Tertiary care centre, Department of Neurology were evaluated by clinical history, temporal profile, complete neurological examination. Investigated using Routine blood work, CT Brain, 2DECHO, Carotid doppler, MRI Brain with Angiogram and other necessary investigations. Result: Of these 560 patients, Males were 364 and females were 196. MC age group is 50- 60 year 356 patients were having Anterior circulation stroke and 204 having posterior circulation stroke. Most common symptom in Anterior circulation stroke is Hemiparesis and posterior circulation stroke is Headache. 377 were ischemic stroke and 183 were Haemorrhagic stroke. MC Risk factor is Hypertension. Out of 560, Cardioembolic strokes were 60. Conclusion: The burden of stroke is increasing in India as compared to previous because of changing life styles and non modifiable risk factors. Conducting epidemiological survey is a daunting task. The date on the risk factors and most prevalent stroke subtypes would guide in preparing stroke treatment protocols according to the prevalence of various risk factors in a community1.

Award ID: 546

AMPA Receptor in the Dentate Gyrus Regulates Reward Memory Recall

Nagashree Bhat, Deepa Rotti, Amul Sakharkar, Sneha Sagarkar

Department of Zoology, Savitribai Phule Pune University, Pune, Maharashtra, India

E-mail: nagashreesb@gmail.com

Background and Aim: Reward is goal-directed behavior. Contextual memories for rewarding stimuli increase probability to access reward. The molecular mechanism regulating the association of reward with contextual stimuli is mostly studied in mesolimbic neurocircuits. Although, contextual features of rewarding stimuli are known to alter hippocampal activities. The molecular underpinning of reward memory recall is understudied. With this, we hypothesize that glutamatergic neurotransmission in the dentate gyrus (DG) is important for reward memory recall. Methodology: In this study, male Wistar rats were conditioned using a nose-poke operant conditioning model for 8 days. Glutaminase siRNA was stereotaxically administered in the DG and the memory probe trial was conducted. Furthermore, to check the subtype of receptor involved, antagonist for NMDA (MK801) and AMPA (CNQX) was used. To check the downstream signaling during reward memory recall, the tet1-BDNF axis was manipulated. Result: Number of active nose poke by conditioned rats was reduced with infusion of glutaminase siRNA in the DG during memory probe trial. Moreover, infusion of AMPA/Kainate receptor antagonist (CNQX) also reduced number of active nose pokes, while infusion of NMDA receptor antagonist (MK801) didn’t affect it. BDNF infusion restored the glutaminase siRNA/CNQX induced reduction in the number of active nose pokes. Interestingly, reward memory recall increased the levels of DNA demethylation enzyme, Tet1. The partial knockdown of tet1 using siRNA reduced number of active nose pokes, which was restored with BDNF administration. Conclusion: These data support the role of glutamate neurotransmission via AMPA receptor in the DG during reward memory recall.

Award ID: 547

An Unusual Case of Opoclonus Myoclonus Syndrome

Aravind Visweswaran, Arun Kumar, Uma Maheshwari, Bala Subramanian, Lakshmi Narasimhan Ranganathan

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: aravindgautham.v@gmail.com

Background and Aim: •The opsoclonus–myoclonus syndrome (OMS) is a rare and distinct neurological disorder characterised by rapid multidirectional conjugate eye movements, myoclonus and ataxia, along with behavioural changes in adults and irritability in children. •It may commonly occur with para-infectious brainstem encephalitis, neuroblastoma in children and small cell carcinoma of the lung in adults. •Herein we describe a patient with opsoclonus myoclonus syndrome and ataxia along with atypical presentation of Anti-N-Methyl-D-Aspartate Receptor (Anti NMDAR) encephalitis. Methodology: · 30 Year old female presenting with involuntary jerky movements involving all limbs, gait ataxia with behavioural abnormality in the form of anger outbursts for one year. She had no motor weakness, preserved reflexes. She had opsoclonus, truncal ataxia with broad based gait disturbances. · She was found to have CSF NMDA receptor positivity with ovarian teratoma. Following Salphingo oopherectomy, She continued to have progressive ataxia and opsoclonus. She was started on rituximab and she developed anaphylaxis requiring mechanical ventilation. She was later treated with bortezomib/ dexamethasone and monthly cyclophosphamide following which her gait improved. Result: Opsoclonus myoclonus has been reported in patients with NMDA encephalitis with ovarian teratoma. Prompt diagnosis of this disorder is important because after tumour removal and immune modulatory therapies it has a relatively good prognosis. Conclusion: Here we report an unusual case of a young woman who had NMDAR antibody positive, teratoma-related, isolated OMS without encephalopathy who developed anaphylaxis to rituximab requiring other immuno-modulators, following which she improved.

Award ID: 548

Stranger in the Mirror

Naveen, Balakrishnan R

Neurology, PSG Institute of Medical Science and Research, Tamil Nadu, India

E-mail: peenav007@gmail.com

Background and Aim: Cortical blindness is an important cause of blindness due to damage to the occipital cortex. It is commonly associated with posterior circulation stroke. Here we are going to see 2 interesting case of cortical blindness Methodology: CASE 1:a 60 year old male came to the op with complaints of hiccupps for 7 days, recent worsening for 3 days, patient also complaints of transient loss of blindness in both eyes, patient underwent cataract surgery recently, on examination Both Eyes pseudophakia present, moving all four limbscase 2:50 yr old female came to the op with complains of complete loss of vision from morning 6 AM to evening 7:00PM and recovered back the eye sight and she tells another person behind her when she looks to the mirror, patient a known case of CAD on treatment, on examination patient is consious, oriented, moving all four limbs Result: In both the cases patient had a transient loss of vision and visual hallucination on further probing the history and imaging was done one case it revealed bilateral occipital lobe infarct, and second one rightt sided occipital lobe infarct Conclusion: Patients cannot go through their normal activities. On top of that, treatment is also very time-consuming, and the result may not be satisfactory. which can cause depression and other psychiatric problems. educating the health workers is the most important work

Award ID: 549

Ipsilateral Isolated Trochlear Nerve Palsy due to paramedian thalamic Infarction

Sandhya Manorenj, Rindha Venepally Rao, Reshma Sultana Shaik

Neurology, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Background: Isolated trochlear nerve palsy is a very rare phenomenon in Ischemic stroke. Each superior oblique muscle is supplied by nerve fibres from the trochlear nucleus of the opposite side. There are no reports of ipsilateral trochlear nerve palsy caused by acute infarction of thalamus. Aim & Objective: Here we report a case of ipsilateral isolated trochlear nerve palsy as a result of small medial thalamic infarction in a 30 year-old man Methodology: We describe clinical and radiology features of ipsilateral trochlear nerve palsy in aute paramedian infarction. Result: Case presentation: A 30 year old man presented with sudden onset vertical diplopia more on looking down with improvement while the head was tilted to left. There was no other complaints. He had history of young onset hypertension and habituated to alcohol and tobacco use. Examination showed right eye hypertropia with worsening of diplopia on head tilt to right side. Park 3 -step test and bielschowsky head tilt confirmed right superior oblique palsy. There was no other clinical manifestation. MRI brain showed acute right paramedian thalamic infarction. Conclusion: Conclusion: Trochlear nerve palsy due to disturbance of nucleus usually affects the contralateral side. In the present case, the trochlear nerve palsy occurred to be on the ipsilateral side of the infarction, which we believed to be because the trochlear nerve was damaged after crossing though there was no midbrain infarction. This is the first case report in literature of ipsilateral trochlear nerve palsy due to paramedian thalamic infarction.

Award ID: 550

Acute Ischemic Stroke Due to Free Floating Thrombus: A Short Case Series

Siva Sankar M, Muralidhar Reddy Y, Shyam K. Jaiswal, Subendhu Parida, Lalitha Pidaparthi, Santosh Kumar B, Syed Osman, Abhinay Kumar Gattu, Jagarlapudi M. K. Murthy

Neurology, Care Hospitals, Banjara Hills, Hyderabad, Telangana, India

E-mail: dr.siva.sm@gmail.com

Background and Aim: Acute ischemic stroke (AIS) due to free-floating thrombus (FFT) of carotid or vertebral arteries is an uncommon entity. These strokes are at increased risk of progression. However, it is still not clear if medical or surgical management is superior. We aimed to study the clinical course and natural history of such patients presenting to our center. Methodology: Study type: retrospective, longitudinal follow-up; Study site: tertiary care hospital; Study period: January 2018 to January 2022; Inclusion criteria: All consecutive patients of AIS due to FFT in the artery of infarction. Parameters studied: demographic features, clinical features at admission, early neurological deterioration, risk factor profile, NIHSS at admission and discharge, arterial territory involved, acute and long term treatment and outcome. Result: Total number of patients: 8; Age (Mean ± SD): 53.37 ± 12.75 years; M:F: 5:3; Risk factors: hypertension (5), diabetes (2), coronary artery disease (2), COVID-19 (2), hyperhomocysteinemia (2), hyperlipidaemia (1); Stroke severity: mild (3), moderate (5), moderately severe and severe (0); NIHSS at admission (Mean ± SD): 7.25 ± 3.53; Early neurological deterioration (1); NIHSS at discharge (Mean ± SD):6.25 ± 4.17; Arterial system involved: Common carotid artery (7), vertebral artery (1); Acute treatment: dual antiplatelet (5), anticoagulants (3), carotid endarterectomy (2); Long term treatment: anticoagulants (2); dual antiplatelet (6); 3-month functional outcome: mRS 0-2 (6), mRS 3-5 (2), deaths (0). Conclusion: AIS due to FFT are more frequent in men with hypertension, COVID-19 and hyperhomocysteinemia. They are usually moderate in severity and majority have good functional outcome at 90 days.

Award ID: 551

Atypical Presentation of Stroke

Naveen, Balakrishnan R

Department of Neurology, PSG Institute of Medical Science and Research, Tamil Nadu, India

E-mail: peenav007@gmail.com

Background and Aim: Methodology: here we present a different cases of stroke and varied presentation Result: ØStroke can have an unusual presentation and can often not be immediately recognized. Ø Diagnostic accuracy in stroke has been increased by improvement in imaging techniques. Patients present more often with milder strokes or coma, fewer focal signs, and cerebellar strokes and have higher disability and mortality rates at 12 months. Conclusion: Precise identification of stroke at the time of presentation can be difficult. ØFurthermore, coexisting medical conditions and the performance of physicians influence the correct diagnosis. ØThe full workup (including magnetic resonance imaging) of all patients that may have a stroke is probably not feasible, and an initial clinical evaluation is still an important screening tool.

Award ID: 552

Case Report: Familial Amyotropic Lateral Sclerosis 10 with Mutation in TARDBP (+) c.943G>C (p.Ala315Pro)

Priya Sutar, G. Butchi Raju, S. Gopi, T. Sateesh Kumar, S. Gopi, U. Aruna Kumari

Department of Neurology, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

E-mail: priyavish17@rediffmail.com

Background and Aim: 1 To study the prevalence of Neuropsychiatric comorbidities and white matter intensities on MRI brain imaging and compare them in patients with migraine and tension type headache. Methodology: All patients of migraine and TTH who were diagnosed by ICHD 3 beta version criteria, aged 18-55 years, attending neurology OP in King George Hospital between July 2020 and January 2021 were included. Patients with complicated migraine like migrainous infarction, primary headaches other than migraine and TTH, secondary headaches, major Psychiatric illness, pregnancy, major neurocognitive disorder and patients with DM, HTN were excluded. Instruments Scales administered by authors -- IHS verbal 4 point scale, Mini Mental Status Examination [MMSE], Montreal Cognitive assessment scale [MOCA], Neuropsychiatric inventory questionnaire, Insomnia severity scale [ISS], Epworth Sleepiness scale [ESS], Headache impact test (version 1.1)[HIT], Fazeka’s scale for white matter lesions. Result: Present study included 53 patients with migraine and 52 patients with TTH. In migraine group, 18 patients were with migraine without aura,18 patients of migraine with aura, 3 patients with chronic migraine and 4 patients with probable migraine. In TTH group, 38 patients were with ETTH and 14 patients with CTTH. In present study 35.85% cases were seen with cognitive impairment in migraine and 44.23% in TTH group. In migraine group prevalence of insomnia was 52.83% and 39.62% cases in TTH group. In migraine group 24.52% cases were seen with WHM whereas 7.55% cases in TTH Group. Statistically no significant difference was observed between cognitive impairment, insomnia and WHM and both groups. Although no statistically significant difference was observed between prevalence of psychiatric features and both groups, statistically significant correlation was observed between irritability and both groups (p = 0.023). In migraine subgroups, statistically significant correlation was observed between cognitive impairment and migraine with aura and migraine without aura group. Statistically significant correlation was observed between insomnia and migraine with auraand chronic migraine group. In TTH subgroups statistically no significant difference as per cognitive impairment, insomnia, psychiatric features and white matter intensities Conclusion: In conclusion, neuropsychiatric features and white matter intensities on MRI brain imaging are found in both migraine and TTH.

Award ID: 553

Association of Stroke and Cardiovascular Mortality with Cerebral Microbleeds: A Systematic Review and Meta-Analysis

Manabesh Nath, Astha Rai, Shubham Misra, Deepti Vibha, Pradeep Kumar

Neurology, All India Institute of Medical Sciences

E-mail: manabesh.nath@gmail.com

Background and Aim: Cerebral microbleeds (CMBs) are a unique characteristic of ischemic stroke and hemorrhagic transformation with significant cognitive, functional, and overall mortality. The current study investigated the role of CMBs in cardiovascular and stroke-related mortalities. Methodology: A comprehensive literature search was performed in various electronic databases, including PubMed, Embase, Cochrane Library, CINAHL, and Google Scholar up to 30th April 2022. Pooled Odds ratios (ORs) with 95% Confidence Intervals (CIs) were calculated to determine the association. Sensitivity analysis was performed to detect the heterogeneity between studies, and Begg’s funnel plot assessed any publication biases. Result: We identified seven retrospective cohort studies with 1127 CMB and 6350 non-CMB patients, all from the Caucasian ethnicity. Our findings revealed a significant protective association between CMB presence and cardiovascular associated mortality (OR = 0.45, 95% CI = 0.29-0.70). However, no significant relationship was observed between stroke associated mortality with the presence or absence of CMB (OR = 0.72, 95% CI = 0.31-1.64). When subgroup analysis was conducted based on the CMB location in the brain, a significant protective association was observed between lobar (OR = 0.35, 95% CI = 0.20-0.60), non-lobar (OR = 0.22, 95% CI = 0.07-0.77), and deep (OR = 0.22, 95% CI = 0.07-0.70) CMBs with cardiovascular associated mortalities. No such significant relationship was observed between the CMB types and stroke-related mortalities. Conclusion: The results showed a protective association between cardiovascular-related mortalities and the presence or absence of CMBs in patients. Large-scale studies investigating the long-term outcomes of CMBs are necessary.

Award ID: 554

Association of all-Cause Mortality with Cerebral Microbleeds: A Systematic Review and Meta-Analysis

Astha Rai, Manabesh Nath, Shubham Misra, Pradeep Kumar

Neurology, AIIMS, New Delhi, India

E-mail: astharai185@gmail.com

Background and Aim: Cerebral microbleeds (CMBs) have been associated with stroke severity, causing significant cognitive, overall mortality and long-term functional outcome leading to disability. The current study investigated the role of CMBs in all-cause mortalities in stroke patients. Methodology: A comprehensive literature search was performed in various electronic databases, including PubMed, Embase, Cochrane Library, CINAHL, and Google Scholar up to 30th April 2022. Pooled Odds ratios (ORs) with 95% Confidence Intervals (CIs) were calculated, along with subgroup analysis based on ethnicity (Caucasian vs. Asian). Sensitivity analysis was performed to detect the heterogeneity between studies, and Begg’s funnel plot assessed any publication biases. Result: We identified seven retrospective cohort studies with 2005 CMB and 8531 non-CMB patients, including 9 from Caucasian and 4 from Asian populations. Our findings revealed a significant protective association between CMB presence and all-mortality (OR = 0.63, 95% CI = 0.48-0.81). Moreover, a significant protective relationship was also observed between all-cause mortality and presence of lobar (OR = 0.55, 95% CI = 0.42-0.71), deep (OR = 0.08, 95% CI = 0.04-0.16), and mixed (OR = 0.07, 95% CI = 0.04-0.15) CMBs. However, no such significant association was observed for all-cause mortality and the presence of non-lobar CMBs. Subgroup analysis depicted a significant protective association between lobar CMB presence and mortality in Caucasians (OR = 0.56, 95% CI = 0.42-0.75) but not in the Asian population (OR = 0.98, 95% CI = 0.60-1.60). Conclusion: The results indicated a protective association between all-cause mortalities and the presence or absence of CMBs in patients. Large-scale studies investigating the long-term functional and cognitive outcomes of CMBs are essential.

Award ID: 555

Covid Immunity and Autoimmunity?

Bijoy Jose, Saraf Udit Umesh, Sudheeran Kannoth, Vivek Nambiar, Siby Gopinath, Anand Kumar Anandakuttan, Annamma Mathai, Cornelius Justin, M. R. Manivannan, Kamalesh Chakravarty, Vivel Lal, Parampreet Singh Kharbanda, Aastha Takkar Kapila, Sucharita Ray, Reshma Sultana Shaik, Sandhya Manorenj

Department of Neurology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

E-mail: bijoyjose89@gmail.com

Background and Aim: A significant rise in the number of autoimmune diseases is seen in people after COVID infection and vaccination. We noticed a few cases that were CASPR-2 antibody-positive which developed after COVID vaccination. This association is not described in the literature till now. Methodology: We screened the hospital records for patients reported to have CASPR-2 antibody disease. In this, we selected the patients who were detected to have antibody positivity after recent COVID vaccination. The patients who had clinical features of CASPR disease and were negative for the antibody were excluded. Antibodies were detected by indirect immunofluorescence on HEK 293 transfected cells (EUROIMMUN AG, Luebeck, Germany) in our patients. Result: We could identify 3 cases of CASPR2 autoantibody diseases all of which were triggered by COVID vaccination. The first case is a 37-year-old gentleman without any known comorbidities who presented with severe cramping pain all over the body which started after 1 month of COVID vaccination. Examination showed rippling movement in the calf muscles and EMG showed myokymia. Serum was strongly positive for CASPR2 antibody and he responded well to IV steroids. The next 2 CASPR2 cases presented with late-onset seizures that developed after COVID vaccination. They also responded well to steroids. Conclusion: This is the first report of CASPR2 autoimmunity following COVID-19 vaccination. Although causation cannot be established, it can help to create awareness about potential side effects that will likely emerge as more people are vaccinated.

Award ID: 556

The Relationship between Periodontal Disease (PD) and Recurrent Vascular Events in Ischemic Stroke/Transient Ischemic Attack (TIA) Patients - A Hospital based Cohort study

Ashish Sharma

Neurology, AIIMs Bilaspur, Chhattisgarh, India

E-mail: ashishsharmamd@yahoo.co.in

Background and Aim: In this cohort study, the association between periodontal Disease (PD) and recurrent vascular events was determined among the subjects with ischemic stroke/TIA, and the extent and severity of periodontal disease was estimated among these subjects. Methodology: This prospective, longitudinal, hospital-based cohort study included 153 individuals who had a stroke or TIA. They were divided into two groups: HPD (high periodontal disease) (N = 55, mean age: 59.40 ± 12.21) and LPD (low periodontal disease) (N = 98, mean age: 53.03 ± 12.82). Clinical attachment loss (CAL) and probing pocket depth (PPD) were used to measure the severity of the periodontal disease. TOAST criteria were used to determine the ischemic stroke etiology, and the NIH Stroke Scale was used to determine the ischemic stroke severity (NIHSS). A follow-up survey found that vascular incidents recurred. Result: HPD individuals exhibited a higher median NIHSS (8) than LPD patients (7) in a subset of stroke population (N = 23). 38 cardiovascular events occurred in the first three months after enrollment, including 23 strokes and 7 TIAs, and 5 MIs. There were three mortalityfrom vascular causes. There was a non significant association between PD and composite vascular events (HR 1.06, 95% CI, 1.03 to 1.09, p = 0.71). Compound vascular events were not related to severe HPD (HR 1.31, 95% CI 0.54 to 3.16, p = 0.07). Conclusion: In stroke/TIA patients, there is no direct association between high periodontal disease and recurrent vascular episodes. The proportions of stroke subtypes were not substantially different between HPD and LPD.

Award ID: 557

Boomerang Sign: In Acute Scrub Typhus Encephalitis

Anand Kumar, Deepika Joshi, Varun Kumar Singh

Neurology, Banaras Hindu University, Institute of Medical Sciences, Varanasi, Uttar Pradesh, India

E-mail: anand.2005.02@gmail.com

Background and Aim: Involvement of splenium of corpus callosum presented as signal changes in magnetic resonance imaging was reported in various different neurological conditions. Although, this is a non-specific sign in presence of different clinical situations this may be a clue for clinicians. Methodology: NA Result: A 37 years-old- woman presented with fever for the last 15 days followed by altered sensorium for the last 5 days. The patient also had 3-4 episodes of right focal seizures with impaired awareness. On presentation, the GCS was E2M4V3 with right hemiparesis. There were no signs of meningeal irritation. Her blood investigations including the complete blood count, renal function test, liver function test, Viral markers, chest X-ray, and electrocardiogram were normal. Another fever workup including malaria, dengue, and typhoid were negative. CSF studies were normal along with negative for bacterial, tubercular, fungal stain and culture. Pan-viral PCR testing was negative for all neurotropic viruses. IgM Scrub typhus was found to be positive with very high titer. MRI brain axial T2-weighted images revealed subtle hyperintensity in the splenium of corpus callosum with diffusion restriction in splenium (“Boomerang sign”) and left paramedian thalamus. The patient was started on injection of doxycycline 100 mg twice a day with levetiracetam loading dose of 40 mg/kg intravenously followed by 20 mg/kg in two divided doses along with supportive treatment. Conclusion: This case highlights the fact that “Boomerang Sign” is a non-specific sign. A case with this particular finding should be evaluated based on other clinical and laboratory features rather than a non-conclusive radiological finding.

Award ID: 558

Harlequin Syndrome Associated with Migraine

Anand Kumar, Deepika Joshi, Varun Kumar Singh, Abhishek Pathak, Vijaya Nath Mishra, Rameshwar Nath Mishra

Neurology, Banaras Hindu University, Institute of Medical Sciences, Varanasi, Uttar Pradesh, India

E-mail: anand.2005.02@gmail.com

Background and Aim: Harlequin syndrome is a rare autonomic disorder characterized by unilateral facial flushing and sweating with contralateral anhidrosis induced by exercise, heat, and emotion. It is usually idiopathic but could be the first manifestation of several serious underlying medical conditions. Here, we are presenting a rare case of Harlequin syndrome associated with migraine. Methodology: NA Result: A 17-year-old young female presented with a 4-month history of left hemicranial headache associated with reddish discoloration of left half of the face. Pain is pulsatile in character associated with photophobia, phonophobia, and occasional nausea. Duration varied from 4 to 12 hours. Reddish discoloration of the face was proportionate to the pain intensity and disappeared on subsidence of pain. Pain and discoloration were precipitated by any mental stress like solving mathematics, and subsided by sleep or taking analgesics. Due to this unusual reddish discoloration of their face, she avoids going outside and felt embarrassed. No history of associated redness of eyes, nasal congestion, or watering. Biochemical parameters and magnetic resonance imaging with angiography (MRI with MRA) of the brain, were normal. The patient was diagnosed as a case of migraine with Harlequin syndrome and started on migraine prophylaxis. After one month of follow-up, her headache and redness frequency and intensity were reduced significantly. Conclusion: Harlequin syndrome is a rare cranial autonomic dysfunction. Here we report a very rare association with migraine. With proper diagnosis and appropriate management, such patients improve clinically as well as psychologically.

Award ID: 559

Current Trends in Diagnosis of Tuberculous Meningitis

Kiran Bala, Sonia Ahlawat, Anil Kumar Chhillar, Machiavelli Singh

Neurology, Pt BD Sharma PGIMS, Rohtak, Haryana, India

E-mail: balakiran56@gmail.com

Background and Aim: Background: Tuberculous meningitis (TBM) is the most severe manifestation of Central Nervous System tuberculosis (CNS-TB). Early diagnosis and prompt treatment are the cornerstones to prevent severe neurological complications. Aim: To develop an immunoassay that has the sensitivity of ELISA along with immense nucleic acid amplification capacity of PCR for an early diagnosis of TBM. Methodology: Methodology: The study was done at department of Neurology, PGIMS, Rohtak in collaboration with Centre for Biotechnology, MDU, Rohtak, and Amity Institute of Biotechnology, Amity University Haryana, Manesar. Mycobacterium tuberculosis-specific early secretory antigenic target-6 (ESAT-6) was detected using indirect Immuno-PCR (I-PCR), and results were compared with analogous ELISA. Out of 225 CSF samples collected from PGIMS, 162 CSF samples were included in the study. CSF samples were centrifuged, and the supernatants were used for ESAT-6 detection using indirect I-PCR. All patients were HIV negative. Result: Results: 33.33% (54 out of 162) patients received a final clinical diagnosis of TBM, 9.25% (5 out of 54) patients had definite TBM (confirmation with positive GeneXpert Mtb/Rif along with clinical findings), 90.74% (49/54) were categorized as probable and possible TBM. The detection limit for indirect I-PCR with purified ESAT-6 was 1 pg/ml, which was 103 times lower than ELISA. The sensitivity and specificity of indirect I-PCR (63%, 82.4%) for TBM diagnosis were determined; smear and culture were negative for all. Conclusion: Conclusions: 1. Indirect I-PCR showed superiority over ELISA for ESAT-6 detection in the CSF of TBM cases. 2. The antigen detection revealed an efficient diagnosis by indirect I-PCR with limited CSF volumes.

Award ID: 560

Devic’s Dance- Unusual Movement Disorder in NMOSD

Kaushik S, Lakshmi Narasimhan, Manikavasagam V, Shanmugasundaram K, Vinoth Kannan V, Lakshmi Narasimhan, Tamil pavai, Nantha Kumar, Nithyanantham V

Neurology, MMC

E-mail: kaushik1944@gmail.com

Background and Aim: History: 15 year old female presented with complaints of blurring of vision in right eye since 1 week. Acute in onset and progressive in nature. Methodology: Examination: Visual acuity- 20/200, 20/30 Colour vision impaired on the right side with fundus examination suggestive of right optic disc edema. Serum Aquaporin 4- Positive Given IV Methylprednisolone and rituximab and discharged Result: •1 week later patient presented with history of involuntary movements- random, continually present suppressed during sleep/ •On examination she had Choreo- athetoid movements in both upper limbs with left upper limb dystonia. •MRI Brain s/o T2 hyperintensity in right optic nerve •MRI spine s/o C2- C7 hyperintensity. Conclusion: Discussion: Movement disorders have been reported in NMOSD patients. It is highly under reported. Spinal movement disorders are most common- Tonic spasms, focal dystonia, spinal myoclonus, tremors of spinal origin. Chorea has bee rarely reported. The mechanism of choreiform movement in NMOSD is probably due to deafferentation.

Award ID: 561

Diffusion Restriction in Bilateral Thalami: Beyond Artery of Percheron Infarct

Sandhya Manorenj, Reshma Sultana Shaik

Neurology, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Background: Bilateral thalamic infarction especially the medial thalamus has been characteristically described in “Artery of Percheron” affection. However bilateral thalamic restriction is described in many entities beyond artery of Percheron infarction. Aim: Here we describe a case series of infective causes of bilateral thalamic diffusion restriction lesion from a tertiary care centre in Tropical India Methodology: It was a prospective, cross sectional study done during the period of one year collecting cases showing bilateral diffusion restriction on MRI who did not have thalamic infarcts and their outcomes Result: Bilateral thalamic lesion can occur in both bacterial and viral infections. Thus we intend to add infections, as one of the etiologies in the ever expanding list of conditions which produce bilateral thalamic restriction Conclusion: All the acute symmetric thalamic lesions are not always vascular insult. Accurate assessment and prompt diagnosis can prevent unnecessary delay in treatment

Award ID: 562

Tandem Lesion as a Rare Presentation of Cerebral Rheumatoid Vasculitis: A Case Report

Sandhya Manorenj, Reshma Sultana Shaik, Rindha Venepally Rao

Neurology, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Background: Tandem lesion or tandem occlusion refer to the simultaneous presence of high grade stenosis of the extracranial (cervical) internal carotid artery and occlusion of the intracranial terminal internal carotid artery or its branches usually M1, M2 branches of middle cerebral artery. Tandem lesion accounts for 30% of large vessel occlusion strokes and carries poor prognosis if untreated. Rheumatoid cerebral vasculitis occurs in 2-8% cases and usually involve medium and small sized cerebral blood vessels. Large vessel vasculitis in rheumatoid arthritis is very sparse and reported in two scenario, first in rheumatoid arthritis patient on anti-tumour necrosis factor inhibitors, secondly chronic inflammation producing secondary moyamoya disease. Aim: Here we describe a case of young stroke with denova detected rheumatoid arthritis with tandem occlusion. Methodology: A case study. Result: Case report: A 45 year-old woman presented with acute onset of left ataxic hemiparesis. She had a history of multiple joint pain since two years for which she did not seek any medical attention. She did not have any other comorbidities. MRI brain showed diffusion restriction in right frontal and parietal cortex. Angiogram brain showed tandem occlusion, M3 and M4 narrowing with vasculitis changes and proximal cervical ICA showed significant narrowing. Rheumatoid factor was strongly positive. She responded dramatically to treatment with pulse steroids 1 gm daily for 5 days followed by maintenance immunotherapy (steroids and methotrexate) Conclusion: Conclusion: Cerebral rheumatoid vasculitis is very rare. To our knowledge present case is first in literature showing tandem occlusion in rheumatoid cerebral vasculitis that responded well to immunotherapy

Award ID: 563

An Interesting Case of Late Onset Congenital Myasthenic Syndrome

R. Pazhani, Pushkar P, Kavya S

Neurology, Pazhani Neuro Centre, The Tamilnadu Dr MGR Medical University, Tamil Nadu, India

E-mail: pazran@hotmail.com

Background and Aim: Congenital myasthenic syndrome is usually young age in onset. An interesting case of genetic test proven late age onset congenital myasthenic syndrome is presented here. Methodology: Seventy four year old gentleman presented with h/o weakness in both upper and lower limbs, mainly in the proximal part for the past 20 years. It was gradual in onset and slow in progression. He is wheel chair bound for the past 3-4 years. There was no family h/o similar illness. His children were normal. He was diagnosed to be having limb girdle syndrome, probable motor neurone disease. His past work up was analysed and remaining tests done this time to conclude the diagnosis. Result: He had routine blood investigations which were unremarkable. His serum CPK was normal. Nerve conduction study was normal. MRI cervical spine with routine spine screening was normal. This time clinical exome sequencing test was done. The report came as follows - Gene transcript - DOK7(+)-Location Exon 7-variant c.1120_1121insGCCT (p.Ala378SerfsTer30)-Homozygous-Congenital myasthenic syndrome-10, Autosomal Recessive-PathogenicHe responded to salbutamol and fluoxetine. He started walking with less support. Conclusion: The disease started at the age of 54 years and the diagnosis was missed for 20 years. It is an interesting case of late age onset, genetically proven congenital myasthenic syndrome with DOK 7 mutation. The patient responded to the given medications. The genetic test in this case helped in diagnosing the uncommon presentation of congenital myasthenic syndrome.

Award ID: 564

A Rare Presentation of Neuro-Behcet’s Disease as Optic Neuritis

Sushma Chandragiri, Jawahar M, Lakshmi Narasimhan Ranganathan, Marian Jude Vijay

Neurology, Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: sushchandragiri03@gmail.com

Background and Aim: Behçet’s disease is a multi-systemic vasculitic disorder of unknown aetiology, presents with recurrent oral aphthae, genital ulcers, variable skin lesions, arthritis, uveitis and thrombophlebitis. Neurological involvement occurs in around 5-10% of cases, with CNS being the main target. Methodology: We report an isolated case of Neuro-Behcet’s disease presenting as a optic neuritis. Result: We had a 32 year old female patient admitted with history of 2 episodes of diminished vision in left eye with pain 8 months before, with partial recovery after the second episode, and history of fever of 2 months duration, swaying to either sides on sitting and while walking, with tremulousness of both hands and slurring of speech of 2 weeks duration. Past history of recurrent oral ulcers present. Examination showed left optic disc pallor, jaw jerk present. Minimal weakness in pyramidal pattern on both sides upper and lower limbs. All DTR exaggerated. Pan-cerebellar signs. With normal cognitive, sensory and extra pyramidal system examination. Routine lab investigations were under normal range, fever work up is negative, demyelination work up is negative. Pathergy test was negative. Imaging showed left optic nerve T2/FLAIR hyper intensity, symmetrical T2/FLAIR hyper intensities in thalamocapsular region, bilateral corticospinal tract. HLA-B51 is positive. Patient has been treated with pulse dose inj. Methyl prednisolone followed by oral steroids and then added on steroid sparing agent. Patient improved symptom wise and is under regular follow up. Conclusion: CNS involvement in Neuro-Behcet’s disease can present with Parenchymal or non-Parenchymal involvement. With subacute meningoencephalitis being the most common. Whereas the presentation as optic neuritis is rare with around <0.6-0.8% of cases being reported in the literature.

Award ID: 567

Sarcoidosis Presenting as CIDP: A Rare Neurological Masquerader

Pratik Patel

Neurology, AIIMS, Jodhpur, Rajasthan, India

E-mail: patelpratikk304@gmail.com

Background and Aim: Neurosarcoidosis is rare entity. The meninges, brain parenchyma, spinal cord, peripheral nerve, and muscle can be involved in neurosarcoidosis. CIDP as a prototype presentation is very rare in neurosarcoidosis. Methodology: A 61-year-old hypertensive and diabetic gentleman presented with insidious onset slowly progressive LMN type quadriparesis of two years duration, burning paraesthesia over B/L thigh and calves; bulbar palsy and significant weight loss. Examination revealed diminished gag reflex, wasting of B/L thenar and hypothenar, long finger flexors and supraspinatus, power in B/L wrist flexor and extensor & B/L hip extensor:4-/5, weak grip, and B/L ankle reflexes were absent. There was palpable thickening of both ulnar, superficial peroneal and sural nerves. The red flag features were: gegenheltan type of paratonia and left palmomemtal reflex. NCS was consistent with electrophysiological criteria of CIDP. CSF was suggestive of albumiocytogenic dissociation (protein:177 mg/dl and total cells 15/cumm). CEMRI and PET brain and spine were normal. CECT and PET chest and abdomen were suggestive of perihilar hypermetabolic lymphadenopathy. Hyperprolactinemia (1046 mIU/L) and severe orthostatic hypotension were also present. Lymph node and left sural nerve biopsy showed non-necrotizing granulomatous pathology. ACE level was 33 microgram/L. With steroid pulse therapy his muscle power and tone improved completely. Result: Conclusion: Sarcoid related polyneuropathy is an unusual presenting feature of sarcoidosis which rarely mimics CIDP. It is important to recognise red flags in CIDP like presentations such as weight loss, systemic features, autonomic and additional levels of involvement of the neuraxis.

Award ID: 568

MOG Antibodies - Associated Demyelination Presenting as ADEM as an Initial Manifestation Secondary to COVID 19 in a Child: A Case Report

Sandhya Manorenj, Suma Kandukuri

Neurology, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Background: ADEM like illness occurs in Anti-MOG associated demyelination disease and is commonly reported in children. COVID 19 disease and Anti-MOG antibody related optic neuritis and encephalitis has been reported in adults in the literature. However COVID 19 infection in children presenting as ADEM and Anti- MOG antibody positivity is not yet reported in literature. Aim: Here we report a 9 year old child who presented with ADEM, found to have RT PCR positivity for COVID-19 and his MOG antibody was positive. Methodology: Methods: Prospective case study with clinical and radiological features. Result: Results: 9 year-old developmentally normal male presented with complaints of double vision, difficulty in walking, fever and altered sensorium since 3 days. During his hospital course, he became somnolent with autonomic instability. Contrast brain Magnetic resonance imaging (MRI) revealed extensive bilateral asymmetrical white matter involvement and bilateral deep gray matter thalami and right cerebellar involvement without DW1 restriction, and without enhancement on contrast. Nasopharyngeal swab SARS-CoV-2 polymerase chain reaction (PCR) was positive and Covid antibodies were negative. Cerebral spinal fluid (CSF) analysis showed 100 cells with 50% neutrophils; proteins, sugar and ADA were within normal limit. CSF and serum Oligo clonal band and serum (anti-NMO) anti-aquaporin antibodies were negative, while MOG antibodies was positive Conclusion: Conclusions: The case highlights a possible association between inflammation due to COVID-19 and secondary autoimmunity with MOG antibodies and ADEM. Clinician should be aware of these rare association of COVID 19, ADEM and MOG antibody and indicates role of immunotherapy in the treatment.

Award ID: 569

Vertebral Artery Stenting in Stuttering Vertebrobasilar Ischemic Symptoms

Vivek Iyer, Meenakshisundaram U, Rithesh Nair Ramankutty, Ravi Kumar, Deva Abinaya V, Rahul Vitthal Kulkarni, Pujari Shripad Suryakant, Rahul Vitthal Kulkarni, Shripad Suryakant Pujari

Neurology, SRM Institutes for Medical Sciences

E-mail: drvivekiyer1984@gmail.com

Background and Aim: To highlight the importance of timely VA stenting in case of stuttering vertebrobasilar symptoms Methodology: We describe 2 patients with recurrent vertebrobasilar ischemic symptoms -a hemodynamic phenomenon due to occlusion of vertebral arteries. Case 1: 70 years old gentleman presented with one-day history of dysarthria and right hemiparesis. Examination revealed palatal weakness and right-sided ataxic hemiparesis with NIHSS of 4/42 and mRS of 4/6. MRI revealed acute right cerebellar infarct. MRA showed faint opacification of basilar artery and non-visualization of bilateral VA, left ICA and MCA branches. Inspite of dual antiplatelets and good hemodynamic permissive hypertension, he had clinical worsening, he underwent DSA which showed right VA occlusion from right PICA origin and left VA non-visualization, he underwent right VA stenting with stabilization of his symptoms. Case 2: 51 years old gentleman, a known diabetic and hypertensive, presented with one-day history of right sided limb weakness and dysarthria with NIHSS of 3/42 and mRS of 3/6. MRI showed right PCA territory infarct, MRA showed faint opacification of basilar artery with bilateral VA occlusion. He developed worsening of symptoms, so he underwent Angiogram, which showed total occlusion of right VA and near total occlusion of left VA. He underwent stenting of near-total occlusion of the left VA with stabilization of his symptoms. His mRS at discharge was 1/6 Result: Timely endovascular intervention resulted in good functional outcome in vertebro-basilar ischemia. Conclusion: Timely endovascular intervention in case of large vessel posterior circulation stroke cannot be undermined.

Award ID: 570

A Rare Case Report Anteriar Spinal Artery Thrombosis in a Young Boy

Devireddy Prasoona, Uma Maheswari, Arun kumar, Balasubramanian S, Lakshminarasimhan Ranganathan

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drprasoonareddy@gmail.com

Background and Aim: Introduction: Anterior spinal artery (ASA) Thrombosis syndrome is less frequent. overall annual incidence can be estimated as of 12 in 100,000. It results in motor weakness and dissociated sensory loss below the level of lesion, accompanied by bladder dysfunction. Methodology: Case Scenario: A 13 year old boy presented with sudden onset of paraparesis associated with numbness below the chest and tight band like sensation over abdomen preceded by exertional activity in the form of excessive outdoor play, on the previous day. There was no symptoms suggestive of cranial nerve involvement, extra pyramidal system involvement. However, he had urinary retention. on examination, General examination and vitals were stable, neurological examination showed, normal higher mental functions with cranial nerve examination. Motor system examination showed flaccid paraparesis, with areflexia, with extensor plantar, with dissociated sensory loss, with sensory level at T6. MRI Cervical spine with whole spine screening showed T2 hyperintensity from D1 to D6 level suggestive of anterior spinal artery thrombosis. Procoagulant and other Rheumatological work up Negative. He was given inj.methyprednisolone for 5 days, and he improved and became ambulatory Result: Discussion: The most common cause of anterior cord syndrome is iatrogenic, namely thoracic and thoracoabdominal aortic aneurysm repair. Aortic dissection is a rare cause of spinal cord ischemia. In children minor trauma is a cause of ischemia related to fibrocartilagenous emboli and also spasm. it is often associated with post-infectious or post-vaccination myelopathy and downs syndrome, hematological disorders. Conclusion: Conclusion: this case is a young healthy boy with no underlying hematological abnormalities, presented with ASA thrombosis syndrome due to trauma, of rare etiology and anterior spinal artery thrombosis syndrome prognosis is poor. So Early suspicion and management is necessary to prevent poor outcome.

Award ID: 571

A Rare Case of Levodopa Responsive Painless Legs and Moving Toes Syndrome

Guruprasad Yellamsetty, Jawahar M, Marian Jude Vijay, Sivaji M, Balasubramanian S, Lakshmi Narasimhan Ranganathan, Uma Maheswari E, Arun Kumar Natesan

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: guruprasadyellamsetty@gmail.com

Background and Aim: Introduction: Painless legs moving toes syndrome is a rare disorder characterized by flexion, extension, abduction, adduction and torsion of toes in the absence of pain. It is considered as a variant of painful leg and moving toes syndrome. Methodology: Case scenario: 45 old female without any comorbidities, addictions, drug or natural medicine intake presented with 2 year history of involuntary movements of bilateral foot present throughout the day more on rest than standing almost absent during walking and sleep. No history of slowness of walking, falls. On examination patient was conscious, oriented with MMSE of 30/30. Lobar functions were normal. Motor, sensory and cranial nerve examination were normal. EPS examination showed no bradykinesia, hypokinesia, cogwheel rigidity. Routine blood investigations and MRI brain with MRA and MRV were normal. Result: With provisional diagnosis of Painless legs and moving toes syndrome she was treated with Levodopa+ carbidopa (100/10) after which she improved symptomatically. Conclusion: Early recognition, evaluation and promt treatment is very essential in treating this rare but treatable disorder. Other drugs which showed beneficial were clonazepam and gabapentin.

Award ID: 572

A Case of Syphilitic Optic Neuritis

Jaikumar Rajagurunathan, Nirmal Surya

Neurology, Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: jaikumar24691@gmail.com

Background and Aim: Syphilis is sexually transmitted disease caused by spirochetes, treponema pallidum. Optic nerve involvement can be the first manifestation of syphilis and hiv co infection. Methodology: Diagnosis of syphilitic optic neuritis based on following criteria; 1. History of visual loss secondary to optic neuropathy, 2. postive serological testing for VDRL and (or) TPHA, 3. Elevated csf protein and/ or elevated csf total leucocyte count and/or csf VDRL positive and/ or csf TPHA positive, 4. No evidence of other known causes of csf abnormalities or optic neuropathies. Result: A 25 year old male with no prior comorbities presented with subacute onset progressive neurological illness of 2 months duration in the form of blurring and cloudiness of vision in both eyes. On examination patient having b/l disc edema with visual acuity of 20/40 in both eyes, color vision and field of vision normal in both eyes with no rapd. Conclusion: Here we report a case of syphilitic optic neuritis in hiv positive patient.

Award ID: 573

Optic Neuropathy in Paget’s Disease of Bone

Aiswarya Kumar, Balasubramaniam S, Uma Maheshwari, Arun Kumar, Lakshmi Narasimhan R

Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: aisu5000@yahoo.com

Background and Aim: Introduction: We report a case of Paget’s disease of bone leading to optic canal narrowing with compressive optic neuropathy. Paget’s disease of bone is associated with involvement of both the central and peripheral nervous system. Neurologic complications occur because of mechanical compression of neural tissues or due to ischemia. Methodology and Result: Case Scenario: 49-year-old female admitted with diminution of vision of left eye since 5 days, painless, sudden onset, associated with dyschromatopsia. History of dental extraction 3 years back for dental caries, subsequently patient developed left jaw osteomyelitis for which she underwent debridement surgery. Post-surgical phase was complicated by left oroantral fistula. On examination, left eye visual acuity was counting fingers only with impaired color vision and RAPD. Left eye fundus showed temporal disc pallor with central scotoma on visual field testing while VEP was not obtained in left eye. MRI brain and orbit showed bilateral optic nerves normal with no contrast enhancement, normal brain parenchyma and diffuse sclerotic changes in skull. CT facial bones showed diffuse sclerosis and obliteration of diploea with bilateral optic canal narrowing -right optic canal 2.9 mm and left optic canal 2.77 mm. Conclusion: Most common focal cranial neurological manifestation in Paget’s disease is hearing loss. Other cranial mononeuropathies include disease of the cribriform plate that can disrupt olfaction as well as optic neuropathy, trigeminal neuralgia and hemifacial spasm which are much less frequent. Our patient had narrowing of left optic canal leading to compressive optic neuropathy in background of Paget’s disease of bone, although the optic neuropathy of Paget’s disease cannot be solely explained on basis of bony compression.

Award ID: 574

Emerging Role of Novel Blood-Based Biomarkers in Neurodegenerative Dementias

Faheem Arshad, Paras Borse, Poojja M, Subasree Ramakrishnan, Rajani Battu, Sarada Subramanian, Suvarna Alladi

Neurology, National Institute of Mental Health and Neurosciences Neurology

E-mail: faheem2285@gmail.com

Background and Aim: The aetiology of neurodegenerative dementia is complex, and clinical methods lack sensitivity and specificity due to overlapping symptoms. This underscores the need of novel serum biomarkers in diagnosis of dementia. Serum glial fibrillary acidic protein (GFAP) and neurofilament light chain (NfL) are non-amyloid blood-based biomarkers indicative of ongoing inflammatory and neurodegenerative process. We aimed to assess and validate their diagnostic value in patients with Alzheimer’s dementia (AD) and Frontotemporal dementia (FTD) in the Indian context. Methodology: Ninety-nine participants recruited in the study underwent detailed cognitive assessment and basic brain imaging. Blood samples were collected from healthy controls (n = 29), patients with AD (n = 28) and FTD (n = 42). Quantitative determination of serum GFAP and NfL was carried out with the Simoa platform following a 2-step digital immunoassay method as per the manufacturer’s instructions using an HD-X analyser. In addition, retinal parameters using Optical Coherence Tomography (OCT) were also studied. Result: Serum GFAP and NfL were significantly higher in patients with dementia compared to healthy controls (p < 0.001). Serum GFAP levels were higher in AD (p < 0.001), while NfL levels were significantly elevated in AD and FTD compared to controls (p < 0.05). OCT showed that TMV (total macular volume) and retinal nerve fibre layer (RNFL) thickness in the naso-inferior (NI) quadrant in both eyes were significantly lower in dementia than in controls. Conclusion: Our results suggest that, while serum GFAP and NfL seem to have an emerging role in differentiating AD and FTD from healthy controls, prospective cohort studies are required to confirm the findings.

Award ID: 575

Neurological and Functional Outcome with Hyperbaric Oxygen Therapy in Severe Hypoxic Ischemic Encephalopathy

Faiz Mohamed, Abhishek Srivastava, Navita Vyas, Tushar Sonawane

Physical Medicine Rehabilitation, Sakra World Hospital, Bengalure, Karnataka, India

E-mail: thefaiz@hotmail.com

Background and Aim: The prevalence of severe Hypoxic Ischemic Encephalopathy (HIE) is increasing and their prognosis for meaningful recovery is poor. Adjunctive Hyperbaric Oxygen therapy (HBOT) has been used in patients with severe disorder of consciousness but studies to evaluate benefits are scarce. Aim was to evaluate the changes in level of consciousness, neurological status and functional status in patients with severe hypoxic ischemic encephalopathy (HIE) who underwent HBOT. Methodology: This is a retrospective study involving 54 patients with severe HIE. Validated outcome scales (Coma Recovery Scale-Revised for level of consciousness, Glasgow Coma Scale for neurological status, and Glasgow Outcome Scale for functional status) prior to starting HBOT and at discharge were evaluated. Clinical Data, Duration of injury, Number of HBOT sessions and medical complications attributed to HBOT were analysed. Wilcoxon sign ranked test was used for statistical analysis. Result: Fifty two percent patients had change in level of consciousness with 13% achieving consciousness. Fifty percent patients had improvement in neurologic Status. 35% of patients showed functional improvement with 13% patients achieving independence for ADLs at discharge. Higher initial GOS score significantly increased the odds of having higher GCS and level of consciousness at discharge. Younger age, female gender, HBOT session started within a month of injury and higher GOS score significantly increased the odds of functional recovery at discharge. No major complications were attributed to HBOT. Conclusion: There is scope for improvement in consciousness, neurological status, and functional status after severe HIE. HBOT is safe and well tolerated by persons with severe HIE.

Award ID: 576

Utilizing MSProDiscuss to Monitor Disease Progression in MS: Preliminary Data

Meenakshi-Sundaram Salvadeeswaran, Karthik Nagendra Somalinga, Suresh Pandi, Muthukani Sankaranarayanan, Nandhini Nallasamy, Suganya Velayutham

Department of Neurosciences, Apollo Speciality Hospitals, Madurai, Tamil Nadu, India

E-mail: drsundarsms@gmail.com

Background and Aim: Patients with relapsing-remitting multiple sclerosis (RRMS) need vigilant clinical and radiological follow-up to look for evidence of clinical/subclinical disease progression. A disease monitoring tool will offer clinicians early signs of disease progression and aid in treatment decisions, making it essential to both patients and physicians. Aim To assess the usefulness of MSProDiscuss tool in patients diagnosed with RRMS newly or are under follow-up. Methodology: MSProDiscuss (A questionnaire-based, physician-completed digital tool) was used in the present study. Result: MSProDiscuss was administered to 15 RRMS patients (10 females, 5 males). The age group was 21-50 years. Duration of illness ranged from 6 months to 17 years. Ten patients were currently on disease-modifying therapy (DMT). Based on MSProDiscuss participants were categorized regarding the likelihood of progression into green (unlikely, n = 11), yellow (possibly likely, n = 4), and red (likely, n = 0) groups. The MSProDiscuss results and the decision taken by the clinician independent of MSProDiscuss were concordant in 11/15 patients. It takes only 2-4 minutes to complete MSProDiscuss per patient. Based on the tool’s results on disease progression, a decision was made regarding the efficacy of existing DMT, the necessity to switch to a different one versus continuing the same, and the need to commence therapy in individuals who were not on DMT. Conclusion: MSProDiscuss is a simple and valuable tool to assess disease progression in patients with RRMS.

Award ID: 577

Neuromyelitis Optica Spectrum Disorder Anti-MOG positive: Initial Presentation as Acute Desseminated Encephalomyelitis (Clinically/Radiologically)

Nishanth Raju C

General medicine, PSG IMSR, Coimbatore, Tamil Nadu, India

E-mail: hemnishanth@gmail.com

Background and Aim: Neuromyelitis optica spectrum disorders are inflammatory disorders of the central nervous system characterized by severe, immune mediated demyelination and axonal damage targeting optic nerves and spinal cord. The prevalence in adults ranges from 0.37 to 10 per 100,000.(1) Methodology: A 37 yrs female with no known co morbidities, presented with history of intermittent global headache for 20 days, high grade fever with chills for 3 days followed by acute retention of urine, bowel and paraparesis, blurring of vision for 3 days. CNS examination revealed bilateral lower limb weakness and exaggerated reflexes. MRI whole spine and MRI brain contrast showed subtle patchy enhancement in cervical and thoracic spine and hyperintense white matter lesions in bilateral cerebral hemispheres. CSF analysis showed pleocytosis, normal protein and glucose levels. Initial diagnosis of acute encephalomyelitis was made. Autoimmune workup showed Anti beta 2 microglobulin positive. Anti NMO-Aquaporin4 and anti MOG antibody done. Anti MOG antibody was positive. Visual evoked potential showed bilateral demyelinating optic neuropathy. A final diagnosis of neuromyelitis optica spectrum disorder-anti MOG positive was made. Result: Patient was started on steroids and showed improvement in symptoms. During the subsequent follow ups there was no relapses. Conclusion: This case underscores the varied initial presentation of Neuromyelitis optica spectrum disorder both clinically and radiologically. Acute disseminated encephalomyelitis associated with Anti-MOG IgG positive patients is uncommon in adults and low risk of relapse.(2)

Award ID: 578

A Near Missed Case of Idiopathic Intracranial Hypertension due to an Atypical Presentation - A Case Report

Shania Gunasekera, Bimsara Senanayake

Neurology, Institute of Neurology, National Hospital of Sri Lanka, Sri Lanka

E-mail: shania.g89@gmail.com

Background and Aim: Bilateral papilledema in a young female poses several differential diagnoses. Idiopathic intracranial hypertension (IIH) can present without classic manifestations like headache and episodic visual obscurations, when the diagnosis can be missed, causing detrimental effects on vision. We present a case of a young female, who presented with a hypertensive emergency masquerading a sight threatening IIH. Methodology: A 33 y old female with systemic hypertension and chronic kidney disease, presented with bilateral visual impairment without a headache for one-month. Her visual was impaired with significant bilateral visual field defects. Optic fundus revealed bilateral grade IV papilledema with hypertensive vascular changes. Her blood pressure was 220/ 110 mmHg, therefore managed as malignant hypertension. Due to her marked visual impairment which is atypical for hypertensive retinopathy, an MRI – brain was done that excluded Posterior reversible encephalopathy syndrome and bilateral occipital lobe infarctions, but it showed features of IIH. Subsequent lumber puncture revealed an cerebrospinal fluid (CSF) opening pressure of 370 mm. Uremia and anemia were thought to be causing secondary intracranial hypertension. She was treated with Topiramate with some recovery of vision. Result: IIH is increased intracranial pressure without a structural brain lesion or abnormality in CSF. Intracranial hypertension can occur secondary to systemic causes such as anemia, uremia, metabolic derangements and certain drugs.(1) Conclusion: Although classic presentation of IIH is an obese female with headache and episodic visual obscuration (2), it should be considered as a main differential in any patient with bilateral papilledema and visual impairment, especially in the presence of a secondary cause.

Award ID: 579

Neuromyelitis Optica in Aids – Therapeutic Challenges during Covid-19 Pandemic

Raina Deshlahra, Aralikatte Onkarappa Saroja, Karkal Ravishankar Naik

Internal Medicine, KLE, JNMC, Belagavi, Karnataka, India

E-mail: rainadeshlahra94@gmail.com

Background and Aim: Neuromyelitis Optica spectrum disorders (NMSOD) is characterised by severe immune-mediated demyelination of optic nerve and spinal cord. HIV infection and AIDS produce immunodeficiency state and occurrence of autoimmune neurological disorders including multiple sclerosis and NMSOD is rare in AIDS. We report a rare coexistence of NMOSD and AIDS. Methodology: A 38 year old female known case of HIV since 10 years on treatment presented during the first Indian COVID-19 pandemic with progressive paraparesis with sphincter involvement evolving over three weeks. She had asymmetric quadriparesis one year ago with near-complete recovery after oral steroids and eight months ago had painless diminution of vision on right eye which improved with short course of oral steroids. MRI revealed longitudinally extensive myelitis involving lower cervical and upper dorsal spinal cord. Serum NMO by cell-based assay was strongly positive and was MOG negative. Cerebrospinal fluid showed 10 lymphocytes with increased proteins. CD4 count was 226 with viral load of 1,061 copies. Result: She was treated with methylprednisolone resulting in moderate recovery Long term treatment with oral steroids along with mycophenolate was initiated. Low CD 19 and 20 counts contraindicated the rituximab therapy. During follow-up of two years she had one minor relapse which responded to steroid therapy. Conclusion: Institution of aggressive immunomodulation is essential to prevent recurrences in NMSOD. It was challenging to initiate and continue long-term immunomodulation in an immunodeficient person in the presence of ongoing COVID-19 pandemic.

Award ID: 580

Recurrent Stroke with Ipsilateral Hemiparesis: An Unusual Phenomenon

Camelia Porey, Sanjeev Kumar Bhoi, Menka Jha

Neurology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

E-mail: cporey@gmail.com

Background and Aim: Supratentorial strokes are commonly associated with contralateral hemiparesis due to pyramidal fibers decussation. But in some recurrent stroke episodes ipsilateral hemiparesis is caused by ipsilateral hemisphere lesions due to pyramidal fiber reorganization after prior stroke events. Methodology: Here we present an adult male in his forties presenting initially with an acute onset left hemiparesis with left UMN facial weakness due to an acute right anterior cerebral artery (ACA) infarct. After 4.5 years there was a second episode with acute onset right hemiparesis with right UMN facial weakness due to left gangliocapsular bleed. Recovery was incomplete with residual spasticity. After 5 months patient presented to us with a recurrence of right hemiparesis and right UMN facial palsy. However imaging revealed a right putaminal acute bleed extending partially to posterior limb of internal capsule. Result: Transcranial magnetic stimulation (TMS) was done to over each motor cortex and corresponding motor evoked potentials (MEP) was noted in upper and lower limb. Bilateral MEP was elicited on each of the hemisphere stimulation supporting the hypothesis of post stroke reorganization. Conclusion: This is a rare phenomenon of post stroke reorganization and should be further investigated in a larger studies to delineate the exact mechanisms and pathogenesis.

Award ID: 581

Craniovertebral Junction Anomaly as a Cause of Acute Ischemic Stroke in Young

Soaham Desai, Devangi Desai

Neurology, Shree Krishna Hospital Pramukhswami Medical College, Gujarat, India

E-mail: drsoahamdesai@yahoo.com

Background and Aim: Ischemic stroke in young often have unusual causes which require evaluation and management. Methodology: We describe a case of posterior circulation ischemic stroke due to unusual mechanism. A 30-year-old laborer presented with sudden neck pain, headache, vomiting, imbalance and left side weaknessafter lifting 20 kg weight on head. He had left Horner’s syndrome, cerebellar nystagmus, dysarthria, left ataxichemiparesis with decreased pain- temperature sensation on right half of body, with truncal ataxia. He had shortneck, low hair line, cervical scoliosis, hemi-body atrophy suggestive of Klippel-Feil Syndrome Result: His MRI revealed acute infarct in left cerebellum, left medulla, right occipito-parietal cortex with dissection inV1-V2 segment of left vertebral artery and hypoplastic right vertebral artery. Also, partial occipitalization ofatlas vertebra with left lateral atlanto-dental dislocation, focal cord edema at level of body of axis vertebra andblocked C6 and C7 vertebrae were seen on cervical spine imaging. He did not have any traditional risk factors for stroke. He recovered with stroke unit care, dual antiplatelet, statin and rehabilitation. CT angiography at 6 weeks revealed resolution of vertebral artery dissection . Conclusion: Vertebral artery dissection can be a rare manifestation of CV junction anomaly leading to stroke.(2,3) Carefulclinical examination is important in ischemic stroke in absence of traditional risk factors

Award ID: 582

GNE (Nonaka) Distal Myopathy: A Case Report in a Resource Limited Setting

Dilini Chandrasiri, Dilini Prarthana Chandrasiri, Arjuna Fernando, Senaka Bandusena, S. R. Constantine

Neurology, National Hospital Sri Lanka, Sri Lanka

E-mail: Dilini28@gmail.com

Background and Aim: GNE myopathy is an autosomal recessive disorder of the muscle due to mutations in GNE gene. The diagnosis should be considered primarily in patients presenting with slowly progressive distal lower limb weakness in early adulthood, with marked sparing of the quadriceps. Characteristic histological findings include “rimmed” vacuoles, aggregation of various proteins, and fibre size variation. The diagnosis is confirmed by sequencing of the GNE gene. Here we report a case of probable GNE myopathy diagnosed in a resource limited setting. To our knowledge, this is the first reported case of GNE myopathy in Sri Lanka. Methodology: A 26-year-old male presented with progressive weakness of the bilateral distal lower limb muscles over six years. There was no family history of muscle disorders, but he was a product of a consanguineous marriage. On examination there was bilateral symmetrical distal muscle wasting more prominent in the lower limbs, bilateral foot drop with marked sparing of the quadriceps. Neuro-electrophysiological studies were compatible with a distal predominant myopathy. Muscle biopsy revealed myofibre atrophy with occasional rimmed vacuoles consistent with GNE myopathy. Immunohistochemistry for GNE protein is not available in Sri Lanka and the patient was unable to afford genetic studies for mutations of the GNE gene. Result: Conclusion: Co-relating clinical presentation with muscle biopsy findings can be useful in diagnosing rare inherited muscle disorders in resource limited settings.

Award ID: 583

Prevalence of Essential Temor in Rural Gujarat, India

Soaham Desai, Devangi Desai, Andrea Chin, Swati Roy, Shyamsundar Raithata, Utkarsh Shah, Annindita Deb

Neurology, Shree Krishna Hospital Pramukhswami Medical College, Gujarat, India

E-mail: drsoahamdesai@yahoo.com

Background and Aim: Worldwide prevalence of essential tremor (ET) ranges from 0.4-8.6%, affecting about 1% of the general population and 4-5% of individuals over 65 years old. However, studies estimating prevalence of ET are few, with no previous studies amongst rural Indian communities. India’s diverse population makes data extrapolation inaccurate, presenting a need for population-based surveys. Here, we assess the prevalence of ET in 10 villages of Anand district in rural Gujarat, India. Methodology: We utilized a pre-validated modified screening questionnaire for movement disorders, including ET. Participants who screened positive underwent videography and a detailed clinical examination by a neurologist to confirm the ET diagnosis based on the 2018 MDS Tremor Task Force criteria. Result: Of 18,896 individuals screened, 54 participants screened positive and underwent formal neurologic evaluation; 17 were diagnosed with ET. The crude prevalence of ET in this population was 89.96 per 100,000 (0.09%) and the age-specific prevalence in the age group of the 60s and above was 676.69 per 100,000 (0.67%). Conclusion: This study is the only prevalence assessment of ET conducted in a rural based population in the Gujarat state of India. Further studies of prevalence utilizing the MDS diagnostic criteria will help improve awareness, diagnosis, and treatment of ET for the rural Indian population and generate more understanding of the prevalence of ET throughout India and globally.

Award ID: 584

Isolated Intracranial Hypertension Without Cerebral Venous Sinus Thrombosis in Apla Syndrome: An Unique Association

Camelia Porey, Binaya Kumar Jaiswal, Sanjeev Kumar Bhoi, Menka Jha

Neurology, All India Institute of Medical Sciences, Bhubaneswar

E-mail: cporey@gmail.com

Background and Aim: Antiphospholipid antibody (APLA) syndrome is an autoimmune disorder predisposing to thrombotic complications affecting CNS either by arterial vasoocclusion or venous thrombosis. Cerebral venous sinus thrombosis (CVST) secondarily produce raised intracranial pressure (ICP). However intracranial hypertension without evidence of CVST is rare entity. Methodology: We present two cases of elevated ICP with absence of identifiable CVST. Case 1, a 28 year old female presented with a 2 months history of headache followed by bilateral vision loss. CSF opening pressure and fundoscopy along with MRI was suggestive of IIH and patient improved with diuretics. 5 months later she presented with acute onset right sided hemiparesis. MRI showed acute left MCA infarct with normal MRV. APLA came positive with high titres of Anti cardiolipin and Beta 2 glycoprotein both IgG and IgM. Case 2, a 23 year old female presented with headache and diplopia of 2 months duration and, similar to Case 1, on basis of CSF, fundoscopy and MRI brain was diagnosed as IIH and she too responded to diuretics. 2 years later she presented with recurrence of headache and APLA profile showed elevated beta 2 glycoprotein IgG and IgA. Result: Both the cases had no evidence of CVST yet presented with features of intracranial hypertension and diagnosed as APLA syndrome Conclusion: This is an important non thrombotic complication of APLA syndrome and requires further large scale study for insight into the pathogenesis and early recognition to avoid future complications.

Award ID: 585

A Case of Mitochondrial Type 1 Deficiency Nuclear Type 26 Presenting as Dopa Responsive Dystonia

Priya Sutar, G Butchi Raju, S Gopi, T Sateesh Kumar

Department of Neurology, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

E-mail: priyavish17@rediffmail.com

Background and Aim: NDUFA9 (NADH:ubiquinone oxidoreductase) is a Q-module subunit required for complex I assembly or stability. Mitochondrial complex I deficiency nuclear type 26 is caused by homozygous or compound heterozygous mutations in the NDUFA9 gene. Phenotypes include macrocephaly with progressive leukodystrophy, nonspecific encephalopathy, hypertrophic cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. AIMS AND OBJECTIVE We report a case of Mitochondrial complex 1 deficiency presenting with Dopa Responsive Dystonia. Methodology: CASE DESCRIPTION A 11-year-old girl born to non-consanguineous parents after an uncomplicated full-term pregnancy came with Slowness of all routine activities, Slurring of speech and Abnormal posturing of hands and jaw with occasional oculogyric crisis for 8 months No fever, trauma, No H/O drug or toxin exposure No F/H/O similar complaints. Examination revealed mask facies and decreased blink rate No KFring or hepatosplenomegaly. HMFs were normal, mild dysarthria was present. Motor examination showed normal strength and tone at rest. Mild degree of dystonic posturing both hands and jaw closure dystonia present. Tests for bradykinesia were positive. Prompt but illsustained response to Levodopa was present. Result: RESULTS Initial blood investigations revealed normal Hemogram, normal LFTs, RFTs, Sr. Cereluplasmin level, Sr Lactic acid level were normal, Nerve Conduction Studies were normal. Imaging of the brain was unrevealing. Genetic testing revealed heterozygous NDUFA9 gene mutation in Exon 1 consistent with Mitochondrial complex 1 deficiency, nuclear type 26. Sanger sequencing confirmed above deficiency. Conclusion: Movement disorders (MoD), either hypo- or hyperkinetic type, are reported in MD, Dystonia is the main extrapyramidal movement disorder in pediatric MD patients; whereas parkinsonism is the most prevalent hypokinetic disorder in adult MD patients

Award ID: 586

Efficacy And Safety of Injection Tenecteplase in 4.5 To 24 hours imaging Eligible Window patients with Acute Ischemic Stroke

Arti Jatwani Jatwani, Arti Jatwani, Awadh Kishor Pandit, Achal Kumar Srivastava, M. V. Padma Srivastava, Vishnu V. Y., Leve Joseph Devarajan S., Ajay Garg, S. B. Gaikwad, Rohit Bhatia, Rajesh Kumar Singh, Deepti Vibha

Neurology, All India Institue of Sciences

E-mail: arti.jatwani123@gmail.com

Background and Aim: Patients with Acute Ischemic stroke (AIS) up to the window period of 4.5 hours have the scope of receiving intravenous thrombolysis, while intervention for 24 hour patients treated with mechanical thrombectomy (only large vessel occlusion). By understanding the potential of Tenecteplase in terms of safety and efficacy reported in the previous study with the small size of patients, the present study is planned to evaluate the safety and efficacy of injection of Tenecteplase in AIS patients (with or without large vessel occlusion) in window period of 4.5 to 24 hour in a randomized controlled trial. Methodology: The methodology is based on imaging eligibility with perfusion lesion-ischemic core mismatch greater than 1.8 between the volume of hypoperfusion and the volume of the ischemic core, absolute difference in volume should be greater than 10 ml, and ischemic-core volume should be less than 70 ml. Some more criteria are considered an inclusion criterion like patients will be >18 years in age, the period would be 4.5 to 24 hours with NIHSS > 5, and having pre-stroke mRS 2 or less. Patients will be enrolled based on advanced imaging results. One hundred eligible patients will be randomized to Tenecteplase (0.25 mg/kg; maximum 25 mg) and placebo in a ratio of 1:1 administered as a single bolus injection (cases 50 and 50 controls). An independent data safety and monitoring board (DSMB) will share responsibility for regular oversight of patient safety during the study. Result: Conclusion: The study is a prospective, randomized controlled trial design. The safety endpoints along with efficacy of Tenecteplase using the modified Ranking Scale (mRS) score at Day 90 will be determined.

Award ID: 587

A Case Series on Myoclonus

Noel James, TamilPavai N, Nithyanandam Allimuthu, Nanda Kumar V, Lakshmi Narasimhan Ranganathan

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: noeljames.e@gmail.com

Background and Aim: We aim to present a case series on 4 cases of myoclonus of differing etiologies an to analyze the pattern of progression of symptoms in each case. Methodology: This is a case series on myoclonus caused by 4 differing etiologies- Creutzfeldt Jakob disease, Subacute sclerosing Panencephalitis, Parainfectious opsoclonus myoclonus syndrome and multifocal myoclonus due to metabolic etiology. Result: The pattern of myoclonus changes with progression of disease as the pathologic process process involves additional anatomical structures. In CJD and SSPE, the myoclonus is initially off cortical origin and the jerks are chaotic and focal. As the disease process involves the deeper subcortical structures as the disease progresses, the myoclonus becomes periodic and generalized. Other symptoms also correlate with the type of myoclonus with learning difficulty and psychiatric manifestations dominating in the initial stages of disease, and the subcortical spread is marked by Parkinsonism, lethargy and terminally, the akinetic mute state. Conclusion: Study on the type of myoclonus in certain diseases like SSPE may help in identifying the extent of the spread of the disease process. A differing response to drugs may be present.

Award ID: 588

COVID -19 Pandemic and Neurosciences in India- The CoIN Survey: Impact on Neuroscience Research and Early Career Researchers

Mahammad Mondal, DIVYA KP, Jitupam Baishya, Divyani GARG, A Elavarasi, Vaibhav Wadwekar, Souvik Dubey, Kshiteeja Jain, Bhavya Pahwa, Ajay Asranna1

Neurology, National Institute of Mental Health and Neurosciences,

E-mail: msmondal788@gmail.com

Background and Aim: In the wake of the pandemic, an explosion of COVID19-related research is being witnessed. However, its ramifications on ongoing and planned research in neurosciences have not been studied. We conducted a survey to analyze the impact of COVID-19 related disruptions on neurosciences research in India. Methodology: A country-wide online survey involving medical trainees, post-doctoral fellows, PhD-students, early-career-faculty members, and basic-neuroscience researchers was conducted using a structured, self-administered, pre-validated questionnaire, pertaining to ongoing/planned research activities of the respondents, impact of COVID-19 pandemic, and concerns related to future research. Result: Total 204 responses were received. Difficulty in recruiting patients (62.3%) and loss to follow-up (34.3%) leading to delays in completion of projects were reported by a majority (65.4%). Significant deviations from planned protocol including changes in study methodology (29%), sample-size reduction (28%), and substitution of physical to virtual follow-ups (24%) were reported. Most of the researchers (64.5%) were diverted from regular services to COVID-19 related services. An increase in workload leading to a reduction in research time was reported by 34.3%. Funding constraints were experienced by about a third. In comparison to the pre-pandemic period, 32.3% of researchers perceived greater ease in presenting their research in conferences during the pandemic period. Though a majority attributed this to the adoption of the online mode of the conference, 41.6% of respondents still disagreed on replacing conventional off-line conferences with virtual /Hybrid modes. Conclusion: This serious impact of the COVID-19 pandemic on neurosciences research warrants attention and concerted efforts of global bodies and stakeholders.

Award ID: 589

Three Terittory Sign - An Unusual Cause of Stroke in Operated Carcinoma Breast Patient

Dhileeban P, Justin C

Department of Neurology, Madurai Medical College, Madurai, Tamil Nadu, India

E-mail: drdhileeban24@gmail.com

Background and Aim: Multiple cerebral territory infarcts of undetermined origin are typically attributed to cardioembolism; and most frequently atrial fibrillation. Three territory sign as a radiographic marker of stroke due to malignancy is under recognized. Here we report a case of common presentation of stroke with uncommon etiology Methodology: 64 yr female with history of carcinoma breast operated two years back presented with sudden onset of right sided upper limb and lower limb weakness. MRI was s/o multiple infarcts in ACA, MCA and b/l PCA territory. Patient was investigated in suspicious of cardioembolic stroke where ECG, Holter monitoring and 2Decho were normal and treated with antiplatelets, statins and physiotherapy. Result: This case report reminds us of the importance of not only thorough history taking and clinical examination but also MRI Brain with 3 terittory sign will help in early diagnosis and management. Three territory sign is a highly specific marker and 6 times more frequently observed in malignancy-related ischemic stroke than atrial fibrillation- related ischemic stroke Conclusion: Evaluation for underlying malignancy and its associated hypercoagulable states in patients with Three territory sign is reasonable with undetermined etiology.

Award ID: 590

Neurosyphilis with Rapidly Progressive Dementia as Presenting Manifestation

Surbhi Mahajan, Ravi Jakhar, Aastha Takkar, Vivek Lal

Neurology, Post Graduate Institute of Medical Education and Research

E-mail: mahajan_surbhi5@yahoo.com

Background and Aim: Neurosyphilis, known as the Great Chameleon of Neurology, can manifest at any stage with varied presentations. Dementia occurring in the Late Neurosyphilitic stages, has been well reported but presentation with rapidly progressive dementia and behavioural complaints, is rare. Hence, To consider Neurosyphilis in a patient with neurobehavioural and cognitive complaints is paramount. Methodology: 32 year old male with high risk sexual behaviour and recreational smoking drug use presented with 11 months history of insidiously progressive speech and gait impairment followed by behavioural disturbances including decreased social interaction. Social disinhibition along with inappropriate laughter since 9 months and forgetfulness since 6 months with complaints of loss of his whereabouts and tracking of things. On Examination, his MMSE was found to be 16/30. V/A was 6/6 bilaterally. Pupils showed the presence of Anisocoria which was more in dark. Pilocarpine Test done revealed constriction of large pupil (6 mm in the left eye). No EOM Restriction was seen. However, saccades were slow. Motor Examination revealed presence of spasticity bilaterally with brisk reflexes. Cerebellar Examination revealed impairment Axial > appendicular ataxia. In view of high clinical suspicion, diagnostic testing for neurosyphilis was done and came out to be positive. Result: Treatment with Inj. Ceftriaxone was started and improvement was noted with MMSE improving to 22/30. Conclusion: Neurosyphilis should be considered as a possibility in cases with neurobehavioural symptoms with cognitive decline, as a presenting feature.

Award ID: 591

Inclusion Body Myopathy with Early Onset Paget Disease and Frontotemporal Dementia (IBMPFD): A Case Report

Aakash Garg, Pankaj Saini, Sanjeev Maheshwari

Resident doctor M.D Medicine, J.L.N. Medical College and Hospital, Ajmer, Rajasthan, India

E-mail: aakash148101@gmail.com

Background and Aim: Inclusion body myopathy with early-onset Paget disease and frontotemporal dementia (IBMPFD) is a condition that can affect the muscles, bones and brain. Here we present a genetically confirmed case of IBMPFD, the first of its kind in India. A 45 year known case of DM and hypothyroidism female presented with bilateral lower limb followed by upper limb muscle weakness which was more proximal than distal and gradually progressive in nature. The patient complained of multiple bone site pain mostly involving the long bones of the arms and legs. Later she presented with mask like facies, abnormal extraocular movement, restricted vertical gaze, slow saccades impaired vertical OKN, mildly dysarthric speech, unable to swallow or speak properly, hyperreflexia and equivocal plantar reflex, postural tremors, perioral fasciculatory tremors and dystonic posture of neck. The Aim was to establish a diagnosis of the patient in order to provide effective treatment. Methodology: This is a case report study. The patient was evaluated with MRI, PET scan, NCV, EMG and whole exome sequencing. Result: MRI Brain and PET Scan were suggestive of Paget Disease of calvarium with no significant atrophy of brain. NCV and EMG study was suggestive of anterior horn cell involvement. Whole exome sequencing detected a heterozygous missense variation in exon 5 of VCP gene, suggestive of IBMPFD. Conclusion: The first symptom of IBMPFD is often muscle weakness starting with difficulty to climb stairs and later involving other muscles of arms and legs. About half of the patients also develop Paget disease of the bone. IBMPFD can be associated with frontotemporal dementia and rarely with ALS and Parkinson disease.

Award ID: 592

Frequency of Osmophobia among Patients with Migraine and Tension Type Headache

Shiv Saini, Bhupender Kumar Bajaj

Neurology, ABVIMS and Dr Ram Manohar Lohia Hospital, New Delhi, India

E-mail: shivsaini0@gmail.com

Background and Aim: International Classification of Headache Disorders, 3 rd Edition, (ICHD-3) deleted osmophobiafrom the diagnostic criteria for migraine. Some of the recent studies report that osmophobia issignificantly more common and specific observation in migraineurs. We undertook this study toprospectively estimate and compare the frequency of osmophobia among patients with migraineand those with tension type headaches (TTH). Methodology: Study Design: Prospective Observational studySetting: Outpatients in Department of NeurologyNinety seven patients with Migraine (with and without aura) and TTH diagnosed as per ICHD-3 criteria were assessed for history of osmophobia and other headache related characteristics atbaseline. All the patients prospectively maintained a migraine diary including details ofosmophobia till documentation of 4 headache attacks or 2 months follow-up period after initialrecruitment. Descriptive and analytical statistics using MS Excel and SPSS Version 21 were usedto analyze data. Result: We could recruit 61, 34 and 2 patients with TTH, migraine without aura and migrainewith aura respectively. Osmophobia was reported for 19.7% of the total headache attacks. Osmophobia was significantly more frequently associated with migraine than with TTH (p value < 0.05). Osmophobia started before onset of the pain phase of headache and ended before the endof it in all the patients. Conclusion: Osmophobia is more frequent among patients with migraine compared to that among patientswith TTH. Further studies on a larger sample of patients are needed for building a strong case forre-inclusion of osmophobia as a diagnostic criterion for migraine.

Award ID: 593

Clinico-Radiological Profile and Comparison of Recurrence and Functional Outcomes in Patients with Best Medical Management vs Carotid Stenting in Symptomatic Carotid Artery Stenosis

Charvitha Gangula, Aparna R. Pai, Dushyanth Babu Jasthi, Nisha Nithyanand Shenoy

Neurology, Kasturba Medical College, Manipal, Karnataka, India

E-mail: charvitha@gmail.com

Background and Aim: To study the clinic-radiological features of stroke, functional outcomes and recurrences of stroke in patients with symptomatic carotid stenosis in patients who are medically managed vs who underwent stenting. Methodology: This was a prospective observational study conducted in Department of Neurology Kasturba medical college, manipal in patients with infarction confirmed by imaging caused by symptomatic carotid stenosis were selected and their risk factor-radiological profile, outcomes between best medical management group vs carotid stenting at 3 months was studied Result: 120 patients of acute ischemic stroke were assessed and 40 were included in the study as per inclusion and exclusion criteria. Mean age was 61.9 years. 21 had diabetes, 30 had hypertension, 25 had dyslipidaemia, 6 had IHD, 8 were smokers. Main modality of vascular imaging was CT angio which is done in 35. 11 had stenosis 50%-69%, 21 had 70-99%, 8 had 100% stenosis. 32 (80%) preferred best medical management and 8 (20%) opted for carotid intervention. 8 had NIHSS Score of <4, 18 had 4-15, 14 >/16, discharge mRS is 0-2 in 13, 3-5 in 27. 1 patient in medical management group, 0 in stented group had Recurrent stroke at 3 months. Mean mRS at 3 months is 2.3 in medical management group and 1 In stented group. Conclusion: The risk factor profile for symptomatic carotid stenosis was hypertension, dyslipidemia, diabetes, smoking. Radiological profile showed highest patients were in 70-99% stenosis group. Overall, there was no statistically significant difference in the rate of recurrence between the medical management and the carotid stenting.

Award ID: 594

A Rare Case of Severe Iron Deficiency Anemia as a Cause for the Cerebral Venous Sinus Thrombosis

Anuradha Jayakody, Bimsara Senanayake

Department of Neurology, National Hospital Srilanka

E-mail: januhasjay@gmail.com

Background and Aim: Cerebral venous-sinus thrombosis (CVT) is a rare cause of young stroke which is strongly associated with thrombophilia, connective tissue disorders, infections, malignancies and drugs (oral contraceptives). We present a rare occurrence of severe iron deficiency anemia (IDA) with CVT which was a diagnostic and therapeutic challenge Methodology: A 30-year-old male presented with severe headache and two generalized seizures in a span of two days along with papilledema. Subsequent neuroimaging showed extensive CVT involving superior sagittal, right transverse and sigmoid sinuses. He had a microcytic and hypochromic anemia with a hemoglobin of 6.8 g/dl. Serum Ferritin was 5.5 ng/mL. Bidirectional endoscopy and radiological studies didn’t reveal any gastro-intestinal bleeding. Paroxysmal nocturnal hemoglobinuria (PNH) was excluded by flowcytometry. He was negative for thrombophilia, malignancies, connective tissue disorders, vasculitis, inflammatory bowel disease and coeliac disease. Patient was transfused with packed red cells along with iron replacement. Anticoagulating was commenced with heparin and followed by warfarin. Result: CVT and IDA can coexist. IDA is an independent risk factor for cerebral venous sinus thrombosis. PNH and inflammatory bowel disease should be excluded in this context where they can cause both thrombosis and iron loss simultaneously. Osophageal varices secondary to coexisting portal or hepatic vein thrombosis should be excluded in this situation before commencing anticoagulants Conclusion: We excluded all the possible causes for CVT and finally concluded that it was secondary to severe IDA. This association was rarely reported and IDA plays a significant role in pathogenesis of the CVT

Award ID: 595

A Rare Co Existence of Neuromyelitis Optica and Anti-Nmdar Encephalitis – Case Report

Ravi Jakhar, Aastha Takkar, Sahil Mehta, Vivek Lal, Saravana Sukriya, Megha Varshney, Neetika Sharma, Chamanjot Kaur, Suresh Kumar

Neurology, PGIMER

E-mail: dr.ravijakhar23@gmail.com

Background and Aim: Neuromyelitis optica spectrum disorder (NMOSD) is a chronic inflammatory disorder characterized by anti-aquaporin antibody-mediated demyelination and neuronal destruction. It typically presents with recurrent episodes of focal neurological deficits with optic neuritis and myelitis being the most common manifestation. Since the description of NMOSD as a separate clinical entity, there are reports of other conditions such as systemic autoimmune diseases, neurological diseases and malignancies occurring concurrently with NMOSD. The genesis of this association is unclear and attributed to underlying immune mechanisms. Anti-N-methyl-D-aspartate-receptor (anti-NMDAR) encephalitis is mediated by IgG antibodies against GluN1 subunit of NMDA receptor. It is characterised by psychiatric manifestations, seizures, dyskinesia, and encephalopathy. Though prevalent in children, it also affects young adults and has association with ovarian teratoma. Other malignancies include testicular germ cell tumor, non-Hodgkin lymphoma, small cell carcinoma lung and autoimmune disease such as myelin oligodendrocyte glycoprotein associated disease (MOGAD) are infrequently reported with anti-NMDAR encephalitis. Methodology: We here in report a young male who was diagnosed with NMOSD in view of recurrent myelitis presented one year later with personality changes, hypersomnolescence and was diagnosed to have a concurrent anti-NMDAR encephalitis based on a positive serum anti-NMDAR antibody. Result: He was treated with 5 sessions of plasma exchange followed by injection rituximab. One and half years in the follow-up he showedsignificant clinico radiological improvement. Conclusion: So it is crucial to identify the autoimmune diseases occurring concurrently as it influences disease management, prognosis and outcome.

Award ID: 596

Multiple Cranial Nerve Palsies as Neurological Manifestation of Neurosjogrens: An Atypical Scenario

Surbhi Mahajan, Ravi Jakhar, Aastha Takkar, Vivek Lal

Neurology, Post Graduate Institute of Medical Education and Research

E-mail: mahajan_surbhi5@yahoo.com

Background and Aim: Cranial Neuropathy as a presentation of Sjogren’s Syndrome is infrequent. Although any cranial nerve can be involved in Sjogren’s Syndrome, with Trigeminal nerve being the commonest, involvement of 3rd and 7th Cranial Nerves involvement as neurological manifestation is exceedingly rare. Hereby, we present a case of young female with CKD, with multiple cranial nerve palsies as manifestation of Sjogren’s Syndrome. Methodology: 17 year old female having CKD (BD-UK), (etiology: unknown) with Hypertension presented with 2 months history of focal neurological deficits in form of facial deviation towards right side with incomplete closure of left eye, followed by headache and subsequently partial left third nerve palsy with background of vision loss in left eye 5 years back (self recovered) On Examination, Visual acuity-6/6 on right, 6/9 on left. Anisocoria was present with equal reaction in both light and dark. Left Medial Rectus/Inferior Rectus /Inferior Oblique Restriction was present along with Left LMN facial palsy. Rest of the neurological examination was normal. CEMRI Brain with MRA was suggestive of contrast enhancement and thickening of left 3rd and 7th nerves. Autoimmune work revealed Anti Ro positivity (3+). The diagnosis of Sjogren’s Syndrome was further confirmed by histological evidence with Lip Biopsy and correlated by salivary scintigraphy. Result: Immunomodulation with Injection Cyclophosphamide showed significant improvement in gaze paresis. Conclusion: Sjogren’s Syndrome should be considered in patients presenting with multiple cranial nerve palsies, even in the absence of sicca symptoms.

Award ID: 598

MOG Antibody-Associated Demyelination Following Traumatic Brain Injury – A Case Report

Farsana MK, Saraswathi Nashi, Suvarna Alladi, Girish B. Kulkarni, Srijithesh PR

Neurology, NIMHANS, Bengalure, Karnataka, India

E-mail: ayshafmk@gmail.com

Background and Aim: Myelin oligodendrocyte glycoprotein antibody associated disorder (MOGAD) is an autoimmune demyelinating disorder that is associated with acute disseminated encephalomyelitis and occurs commonly following infection or vaccination. This is a case report of MOGAD after traumatic brain injury. Methodology: We evaluated and managed this patient in a tertiary neurology center in South India and followed up for eight months from October 2021 to May 2022. Result: A 45-year-old gentleman presented with areflexic quadriparesis, sensory loss below C5 dermatome and urinary retention for one-day. Patient sustained traumatic brain injury 2 weeks prior to the presentation with left temporal contusion managed surgically. MRI of the spine showed longitudinally extensive transverse myelitis from C6 to T10. Laboratory workup showed strongly positive serum MOG-IgG with oligoclonal bands in the CSF. CSF showed pleocytosis with elevated protein. Other etiologic workup was negative. Diagnosis of MOGAD was considered and treated with intravenous methylprednisolone pulse followed by oral steroids, 5 cycles of large volume plasma exchange and Mycofenolate mofetil. The patient improved symptomatically with resolution of spinal cord lesion. Conclusion: Traumatic brain and spinal cord injuries trigger complex series of secondary events, activating pathways for reactive gliosis, necrosis, apoptosis, immune response and degeneration. Traumatic demyelination of damaged axons occurs at the lesion site in primary injury, while demyelination following secondary processes may extend to regions far from the initial insult. To date, there are no cases of monophasic MOGAD following TBI though cases of ADEM has been reported. Whether trauma can result in MOG associated demyelination is a matter to be resolved by further studies. We postulate that an immune response could have been triggered by the brain trauma that subsequently resulted in the acute demyelination.

Award ID: 600

A Case of Absent Pulses and Ophthalmoplegia

Aditya Nayan, Sai Teja, Sandeep Reddy

Neurology, PGIMER

E-mail: uniquely.aditya@gmail.com

Background and Aim: Cavernous sinus syndrome is an entity with many aetiologies including neoplasms, infections, inflammatory masses, structural causes. Methodology: Here we report a 22-year female with Takayasu arteritis presented with unilateral cavernous sinus syndrome. She also had history of inflammatory joint pain, suggesting a possibility of a complex autoimmune pathology in the background. Result: This association is a rare entity with less known line of management. This case highlights the need to review patients with cavernous sinus syndrome to look for vasculitis as both have different therapeutics. Conclusion: A rare disease with a rare association

Award ID: 601

Sleep Problems in Children with Neurocutaneous Syndromes: A Cross-Sectional Study

Pradeep Kumar Gunasekaran, Lokesh Saini, Swetlana Mukherjee, Prahbhjot Malhi, Arushi Gahlot Saini, Renu Suthar, Jitendra Kumar Sahu, Naveen Sankhyan

Pediatrics, AIIMS Jodhpur, Rajasthan, India

E-mail: pradeepdoc93@gmail.com

Background and Aim: The prevalence and patterns of sleep disturbances in neurocutaneous syndromes are variable and understudied. Methodology: Cross-sectional study for 18 months at a Tertiary Care Pediatric hospital, involving 100 children with neurocutaneous syndromes aged between 4-10 years using the Children’s Sleep Habits Questionnaire-Abbreviated (CSHQ-A), DSM 5 criteria was used for Autism Spectrum Disorder (ASD), Attention Deficit Hyperactivity Disorder (ADHD), and Intellectual Disability (ID). Intelligence quotient (IQ) was assessed using Malin’s Intelligence Scale for Indian Children (MISIC). Result: In 100 children with neurocutaneous syndromes, 47 (47%) had significant sleep problems. In subgroup analysis, 7/17 children with Neurofibromatosis-1, 24/63 children with Tuberous Sclerosis Complex, 10/12 children with Sturge-Weber syndrome, 2/3 children with Linear Nevus Sebaceous Syndrome, and Each one of the children with Hypomelanosis of Ito, McCune Albright Syndrome, Megalencephaly Capillary Malformation Syndrome, and Unclassified neurocutaneous syndrome had significant sleep problems. A total of 11 children had ASD, 5 children had ADHD and 24 children had ID. The mean IQ/DQ scores using age-appropriate MISIC / DP-3 were 70.92 (SD = 22.63) in the study population of 100 children and 64.04 (SD = 24.25) in 47 children with sleep disturbances. There was a significant association between ASD and sleep problems (p < 0.011). The association between ADHD and sleep problems, Intellectual disabilities and sleep problems was not significant. Conclusion: Our study population of neurocutaneous syndromes did not have sleep problems more than that observed in the general population. However the presence of underlying neurodevelopmental disorders specifically autism makes them vulnerable to sleep problems independently. Specific profiling of the phenotypical sleep patterns in each of the neurocutaneous syndromes is required to tailor the evaluation and management individually.

Award ID: 602

A Man With Progressive Leptomeningitis

Swati Sharma, Joy Desai, Batuk Diyora

Neurology, Jaslok Hospital, Mumbai, Maharashtra, India

E-mail: swati.mdmedicine@gmail.com

Background and Aim: Primary diffuse leptomeningeal gliomatosis (PDLG) is characterized by infiltration of the meninges by glial cells without evidence of primary tumor in the brain or spinal cord parenchyma. We present a case of chronic meningitis, that showed no response to conventional treatment, diagnosed as a case of PDLG on biopsy. Methodology: A 59 year male presented with chronic progressive bifrontal headache, one episode of seizure and progressive imbalance on walking. CT Brain showed communicating hydrocephalus for which VP shunt was inserted. He showed improvement with antitubercular therapy and steroids, but subsequently developed cognitive deficits. His brain imaging, CSF and biopsy was done at JHRC. Result: MRI Brain and Spine done revealed diffuse leptomeningitis and ventriculitis with spinal meningitis, with cauda equina thickening. CSF showed neutrophilic pleocytosis with high protein and low sugar. Biopsy done revealed mitotically active nuclei staining positive for GFAP and S100, suggestive of high grade glioma. Conclusion: PDLG is often misinterpreted as being of infectious or autoimmune origin with tuberculous meningitis being considered as the most common clinical diagnosis. PDLG should be suspected in cases of unexplained subacute or chronic meningeal processes.

Award ID: 604

Correlations of Serum Cystatin C Level and Long-Term Cognitive Impairment after Acute Ischemic Stroke and Transient Ischemic Attack

Lijun Zuo

Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China

E-mail: zuolijun1987@126.com

Background and Aim: Cystatin C (Cys C) has been found as a novel biomarker of neurodegenerative diseases, such as dementia and Alzheimer’s disease. We aimed to explore the possible correlations of serum cystatin C level with cognitive impairment in patients who had mild stroke and TIA after 1 year. Methodology: We measured serum CysC levels in 1025 participants with a minor ischemic stroke/TIA from enrolled from the Impairment of Cognition and Sleep (ICONS) study of the China National Stroke Registry-3 (CNSR-3). They were divided into four groups according to quartiles of baseline CysC levels. Patients’ cognitive functions were assessed by MoCA-Beijing at day 14 and at 1 year follow-ups. Multiple logistic regression models were performed to evaluate the relationship between CysC and PSCI at 1 year follow-up. Result: Cognitive impairment was defined as MoCA-Beijing ≤22. Most patients were in 60s (60.96 ± 10.75 years old) with a median (interquartile range) National Institute of Health Stroke Scale score of 3.00 (4.00) and greater than primary school level of education, and 743 participants (72.48%) were male. Among the 1025 participants, 671 (65.46%) patients suffered PSCI at 1 year follow-up. A U-shaped association was observed between CysC and 1-year PSCI [quartile (Q)1 vs. Q3: adjusted odds ratio (aOR) 1.50, 95% CI 1.12-1.99, p = 0.0061; Q2 vs. Q3: aOR 1.17, 95% CI 0.89-1.54, p = 0.2483; Q4 vs. Q3: aOR 1.38, 95% CI 1.04-1.82, p = 0.0273]. Moreover, the A U-shaped association aslo was found between CysC level and the subscore of attention and language. Conclusion: CysC showed a U-shaped correlation with 1-year Overall cognitive function, as well as attention and language. It is probable that measurement of the serum cystatin C level would aid in the early diagnosis of VCI

Award ID: 605

Clinical Features, Brain Structural Changes and Cognitive Impairment in Stroke Patients with Basal Ganglia Infarcts

Lijun Zuo

Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China

E-mail: zuolijun1987@126.com

Background and Aim: Post stroke cognitive impairment (PSCI) is very common and is considered as an increased risk of dementia. The mechanisms underlying PSCI are unclear. Identifying imaging markers of PSCI will help to develop prevention strategies against post-stroke dementia. Methodology: Total of 23 PSCI patients with basal ganglia infarcts after two weeks and 29 age-matched controls underwent magnetic resonance imaging (MRI) measuring cortical thickness and volumes changes and a formal neuropsychological test battery. CI was defined as having a performance score <1.5 standard deviation for normally distributed scores. We compared the differences in Z-scores in different cognitive domains and cortical thickness and volumes in two groups. Multiple linear regressions were performed between cortical thickness and volumes and neuropsychological tests. Result: The majority of PSCI patients were in their 50s (55.19 ± 8.52 years). PSCI patients exhibited a significantly decreased Z score in multiple domains, such as memory, language, visuomotor speed and attention/executive function. The volumes of middle posterior corpus callosum, middle anterior corpus callosum and hippocampus in PSCI patients were remarkably decreased than controls. The thickness of right inferior temporal cortex and insula were significantly smaller than controls. Linear regression analysis revealed that the reduced right hippocampus was related to executive dysfunction; The atrophy of hippocampus was a risk factor of language impairment (p < 0.05) for PSCI patients with basal ganglia infarcts. Conclusion: These findings demonstrated that brain structure is likely to be compromised before ischemic stroke and different gray matter structural changes could lead to specific cognitive decline in PSCI patients with basal ganglia infarcts. The atrophy of right hippocampus, potentially serves as an imaging marker for early executive function of PSCI.

Award ID: 606

Treatment of Steroid-Resistant Sequential Optic Neuritis by Plasma Exchange at a Tertiary Care Centre in Mumbai

Nirbha Ghurye, Sachin Adhav, Swaleha Nadaf, Rahul T. Chakor

Neurology - House Officer (MBBS), Nair Hospital

E-mail: nirbhaghurye@gmail.com

Background and Aim: Plasma exchange (PLEX) is undertaken in acute central nervous system inflammatory demyelinating diseases failing treatment with high-dose corticosteroids. 1 Recommendations on the timing of PLEX after symptom onset remain unclear. 2 We would like to document the average time to PLEX and the outcomes of our three patients with steroid-resistant sequential optic neuritis receiving PLEX. Methodology: This is a retrospective study of patients who presented with steroid-resistant sequential optic neuritis and underwent PLEX. Detailed history and clinical examination was conducted for all patients along with AQP4, MOG antibody, and CSF OCB testing. Patients underwent MRI demyelination protocol. 5 days of IV methylprednisolone (1000 mg/ day) was administered before the five PLEX on alternating days. Result: The median interval between symptom onset in the second symptomatic eye and PLEX was 47.6 days (range from 19-97 days). One patient who tested positive for MOG antibody had two previous episodes of bilateral optic neuritis with partial improvement on steroids. Two patients tested negative for AQP4, MOG, and CSF OCB. Optic disc involvement was seen in all. As MRI demyelination protocol showed optic nerve enhancement suggestive of active disease without any improvement on steroids, PLEX was performed without any adverse events. All three patients had improvement in colour vision after five cycles. The two antibody-negative patients with their first episode of sequential optic neuritis showed an objective improvement in visual acuity. Conclusion: PLEX may lead to functionally important neurological recovery in patients showing active demyelination despite time from symptom onset and needs to be explored further.

Award ID: 607

“Epidemiological, Clinical and Radiological Spectrum of Connective Tissue Disease Related Neurological Disorders — An Ambispective Observational Study.”

Deepak Yadav

Neurology, AIIMS, New Delhi, India

E-mail: deepak.manta@gmail.com

Background and Aim: There is a paucity of properly conducted studies regarding neurological manifestations of connective tissue disorders in Indian population. No study has yet examined effect of neurological manifestations on quality of life in connective tissue disorder in Indian population. To study the epidemiological, clinical, biochemical and radiological features of connective tissue disease having neurological manifestations. -To study impact of neurological manifestation on Quality Of Life of connective tissue disorder patients. Methodology: Ambispective study of 66 patients of CTD with neurological manifestations. Their epidemiological, clinical and radiological data was collected and Quality of life was assessed with Euro QOL 5D and patients were followed up after 3 month and outcome was noted. Result: SLE, sjogren syndrome, systemic sclerosis were commenest. PNS manifestations, found in 35 patients f/b CNS manifestations in 34 patients and psychiatry manifestations in 11 patients. Headache f/b seizure disorder, myelopathy, CNS demyelination were commenest. Myopathy f/b polyneuropathy were PNS manifestations. Anxiety disorder f/b mood disorder were psychiatry manifestations. 50 (75.7%) patients had a positive ANA profile. The various antibodies to ENA seen were SS-A (31.8%), U1-RNP (6.1%), dsDNA (22.7%), Scl-100 (10.6%), SS-B (27.3%), RF (28.8%), APLA (6.1%), histones, centromere and Jo-1 in small proportions. QOL were severely impaired in patients of APLA, Neurobehcet, MCTD and were less affected in RA. Mean mRS after 3 months was 1.88+1.47, which showed disability due to neuropsychiatry manifestation. Conclusion: Conclusion- This study highlights the neurological manifestations of patients having connective tissue disorders. In 40 patients (60.6%), neurological manifestation was the presenting feature leading to the diagnosis of CTD. As CTD is an uncommon disorder, this study is important to understand the various neurological manifestations associated with it in Indian population.

Award ID: 608

A Family of Rare Genetic Disorders

Chandra Bhardwaj, Chandra Mohan Mohan bhardwaj, Chandra Mohan Bhardwaj

Medicine, SGT Medical College, Gurugram, Haryana, India

E-mail: bhardwajchandramohan322@gmail.com

Background and Aim: McLeod neuroacanthocytosis syndrome is a ultra rare genetic disorder that occurs almost exclusively in males. It affects brain and spinal cord. Affected individual develops jerky movements of arms, legs, dystonia of face, tongue It is also characterized by abnormal star shaped cells called acanthocytes on peripheral blood smear. We report a family where mother died due to abnormal neurological disorder (not documented), daughter has huntington chorea, middle son has McLeod neuroacanthocytosis. Methodology: Mc leod neuroacanthocytosis is caused by mutation in XK gene. People with this syndrome have reduced muscle mass, muscle strength, suffers from peripheral neuropathy along with dilated cardiomyopathy. It is a very rare genetic disorder with 150 cases reported worldwide. It is a X- linked recessive disorder. It is predominantly present in males.1,2 Mean age of onset is 30 -40 years. . MRI brain will show atrophy of caudate nucleus, putamen. There is no treatment for his condition. Result: A 35 year old male presented with chief complaints of irregular body movements since 2 years with slurring of speech since 3-4 months with facial grimacing since 1 year. Patient was apparently asymptomatic 2 years back when he complained of irregular body movements sudden in onset involved upper and lower limbs and trunk simultaneously. Patient also had 2 episodes of seizures 1 month back which were generalized tonic clonicPatient gives a unique history of mother dying at age of 55 years due to abnormal neurological disease (not documented), sister having huntington chorea which was recently diagnosed, and his elder brother who died of parkinsonism 5 years back. Conclusion: Early diagnosis is necessary to prevent future transmission.

Award ID: 609

“Asymptomatic Moyamoya”- Where Do We Stand?

Shambaditya Das, Souvik Dubey, Biman Kanti Ray

Neurology, Bangur Institute of Neurosciences, West Bengal, India

E-mail: drshambadityadan@gmail.com

Background and Aim: An ambiguous definition of “asymptomatic” Moyamoya Angiopathy (aMMA) of absence of ischemic/hemorrhagic episodes in MMA patients, has led to its variable adaptation in the limited past-studies. Objective: To observe the clinic-radiological characteristics and prospective follow-up of apparently “asymptomatic” MMA, and to determine if it is truly asymptomatic or not. Methodology: An observation, cohort study of 122 angiographically proven MMA over 6 years was undertaken from a single, tertiary-care-center to observe the clinico-radiological characteristics, prospective follow-up of apparently aMMA. Amongst them, 6 had an initial diagnosis of aMMA following evaluation by atleast one post-graduate doctor, which were further scrutinized by 3 different neurologists for epidemiological, clinical, radiological characteristics and subsequent follow-up. Data were analyzed using descriptive statistics. Result: Mean age was 23.7 ± 13.14 years. 3 of 6 underwent brain-imaging for evaluation of non-migraine-like headache, 1 for dizziness, 2 as part of familial screening for MMA. 4 of 6 patients had specific-triggers for aggravation of symptoms. Brain-imaging revealed old vascular insults and ivy sign in 5 of 6 each (83.3%), mean suzuki staging was 3.6 ± 0.82. 4 of 6 underwent cerebral perfusion study, all had hypoperfusion. Revascularization surgery was done in 2 of 6, rest were managed conservatively. None had any new-onset neurological deficit or radiological progression over a mean follow-up period of 22.3 ± 20.22 months Conclusion: Apparently aMMA may not be truly asymptomatic and often have subtle “paroxysmal events” precipitated by specific-triggers, indicative of transient ischemic symptoms. Thus, warrants for a more precise definition to avoid misclassification of aMMA.

Award ID: 610

Carotid Endarterectomy for Symptomatic Carotid Stenosis: A Developing Country Perspective on Patient Characteristics and Predictors of Periprocedural Adverse Events

Naveen Paramasivan, Sylaja P N, Sajith Sukumaran, Sapna Erat Sreedharan, Shivanesan Pitchai

Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India

E-mail: naveenkumal@gmail.com

Background and Aim: Carotid Endarterectomy (CEA) is the standard of care for patients with symptomatic carotid stenosis. Data from Low- and Middle-Income Countries is sparse on CEA and its outcome. We aimed to describe the profile of our patients, and predictors of periprocedural events in patients with symptomatic carotid stenosis who underwent CEA at our institute. Methodology: A retrospective review of patients with symptomatic carotid stenosis (50-99%) who underwent CEA between January 2011 and December 2021 was done. Clinical and imaging parameters and their influence on periprocedural events were analyzed. Result: Of the 319 patients (77% males) with a mean age of 64 years (SD ± 8.6), 207 (65%) presented only after a stroke. The majority (85%) had high-grade stenosis (≥70%) of the symptomatic carotid. The mean time to CEA was 50 days (SD ± 36), however, only 26 patients (8.2%) underwent surgery within 2 weeks. Minor strokes and TIA occurred in 2.2% of patients in the periprocedural period, while major strokes and death occurred in 4.1% of patients. None of the clinical or imaging parameters predicted the periprocedural adverse events except for the presence of hemodynamic infarcts (12.8% vs 4.8%; P = 0.019). The presence of co-existing significant (>50%) tandem intracranial atherosclerosis (24%) and contralateral carotid occlusion (7.5%) did not influence the periprocedural stroke risk. Conclusion: There is a delay in patients undergoing CEA for symptomatic carotid stenosis. The majority had high-grade carotid stenosis and presented only after a stroke reflecting a lack of awareness. CEA can be performed safely even in patients with significant intracranial tandem stenosis and contralateral carotid occlusion.

Award ID: 611

Neural basis of Functional Movement Disorders- A Neurophysiological Study

Sattwika Banerjee, Supriyo Choudhury, Akash Roy, Praveen Kumar, Subrata Naskar, Hrishikesh Kumar

Neurology, Institute of Neurosciences Kolkata, West Bengal, India

E-mail: sattwikabanerjee@gmail.com

Background and Aim: Patients of Functional movement Disorder (FMD) are conventionally not known to have organic involvement. However, recent reports suggest FMDs might have some organic component too. Basal ganglia and associated network control stopping ability of an ongoing movement. It can be objectively estimated through a behavioral paradigm which finally derive an index called ‘optimal combination Stop Signal Reaction Time’ (oc-SSRT). In the current study, we aimed to indirectly examine Basal Ganglia involvement in case of FMD through this stop-signal paradigm while comparing it with healthy matched control subjects. Methodology: In this cross-sectional study, we recruited 20 FMD patients and 22 healthy subjects from the movement disorders and psychiatry outpatient departments. Clinical assessments were performed through Psychogenic Movement Disorder Rating Scale Score (PMDRS) and Montreal Cognitive Assessment (MoCA). The estimation of oc-SSRT was performed through a portable device programmed to execute and analyse a stop signal task. It could also measure median reaction time. Result: FMD patients demonstrated a significantly prolonged oc-SSRT compared to healthy controls (306.6 ± 89.5, 243.7 ± 72.6 p = 0.019). Median reaction time was not significantly different between the groups (p = 0.087). We did not find any significant association of oc-SSRT with the PMDRS Score or MoCA score (p = 0.819, 0.267) Conclusion: These preliminary results indicate that contrary to classical teaching, the neural circuits controlling the response inhibition were not normal in FMD, but it did not show an association with severity of the disease.

Award ID: 612

Barriers in Stroke Care Pathway on the Way to a Tertiary Care Centre: A Hospital-Based Study from Eastern India

Sujoy Kabiraj, Biman Kanti Ray, Gargi Podder, Deepanjan Dutta, Babuji Santra, Souvik Dubey, Gautam Das, Alak Pandit, Biman Kanti Ray

Neurology, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, West Bengal, India

E-mail: DRSUJOYTHECAREGIVER@GMAIL.COM

Background and Aim: Every year the increasing burden of Stroke Survivors in India makes it imperative to streamline our Stroke Care Pathways. In order to elucidate the various barriers faced by a stroke patient in terms of knowledge barrier or infrastructure, our study aims to identify the nature of barriers to acute stroke care for patients presenting to our hospital in Kolkata, India. Methodology: A prospective cross-sectional study comprising of acute stroke patients from December 2021 to May 2022 admitted to our hospital. Either patients or their family members were registered and made to fill up Patient Informed Consent - Case Report Form involving a questionnaire to objectively describe their journey from onset of stroke to hospital bed and about their basic idea about stroke. Result: We surveyed 303 participants. Only 5 out of every 100 patients arrived to our hospital within 4.5 h of stroke onset. The significant factors (P <.05) causing delay were: knowledge about stroke symptoms amongst family members; failure to recognize the stroke symptoms by the Primary Care Physician; long distance (>15 km) from hospital; visiting hospitals where CT scan facilities are not available; prolonged Door-to-CT times in tertiary care hospital(s); poor knowledge, attitude and practice regarding stroke thrombolysis amongst Emergency Care Physicians in tertiary care hospital(s); non-availability of beds in tertiary care centres. Factors significantly associated (P <.05) with early arrival were: living near (<15 km) the hospital; higher NIHSS score at presentation. Conclusion: Various prehospital barriers to reaching hospital and in-hospital barriers for definitive stroke treatment were identified along with poor understanding of stroke amongst both common man and primary care physicians which demands an array of intensive efforts to strengthen the stroke care pathway in our community.

Award ID: 613

Case of Progressive Ataxia and Palatal Tremors

Maha Jayaraman, Balaji G, Hariharan S, Elangovan S

Department of Neuromedicine, Govt Chengalpattu Medical College Tamilnadu, India

E-mail: mahaswethaa@gmail.com

Background and Aim: Palatal tremor is involuntary, rhythmic and oscillatory movement of the soft palate. Palatal tremor can be classified into three subtypes; essential, symptomatic and palatal tremor associated with progressive ataxia PAPT. Here is a case of PAPT with cognitive impairment Aims: Highlight the sign of palatal tremor in cerebrotendinous xanthomatosis (CTX) Methodology: Case Report: 37 years male a school dropout, subdue intelligence, married with 3 children, worked as a manual labour. Brought by wife with C/O progressive walking difficulty, dysarthria and dysphagia and nasal regurgitation of food, which started insidiously and gradually worsened over 9 months. He stopped going for work and had to be assisted for activities of daily living. Past he was diagnosed as case of bilateral cerebellar stroke. h/o right eye cataract surgery at 8 yrs age. O/E low IQ, left eye cataract, EOM full, slow saccades, spastic dysarthria and cerebellar gait ataxia, B/L Palatal tremors, volitional palatal movements decreased and exaggerated gag reflex, tendon xanthoma in b/l Achilles tendon. CT brain b/l cerebellar hypodensity and MRI brain cerebellar atrophy and t2 hyperintensity of dentate nucleus. Result: Phenotyped as PAPT with childhood cataract, cognitive impairment and tendon xanthoma and a diagnosis of CTX was made. His cholesterol levels were low normal. Whole genome sequencing sent and reports awaited. Pt started on chenodeoxycholic acid. Conclusion: CTX is a rare AR disorder characterized by infantile or early childhood onset of chronic diarrhea, tendon xanthomas, cataracts& multiaxial neurological problems. Although there is no h/0 chronic diarrhea in this patient rest of the signs and imaging fit in. Since it’s a relatively treatable hereditary ataxia the need for an early diagnosis is crucial.

Award ID: 614

Wilson’s Disease with an Initial Manifestation as Peripheral Neuropathy

Aswin Babu, S Elangovan, Balaji G, Hariharan S

Neuromedicine, Chengalpattu Medical College, Tamil Nadu, India

E-mail: Ashwinbharat810@gmail.com

Background and Aim: Early recognition of wilsons disease is difficult because of it’s diverse manifestations. Peripheral neuropathy is rarely reported in context of wilsons disease, but initial manifestation as peripheral neuropathy is very rare Methodology: Case report Result: Wilsons disease may initial present as treatable peripheral neuropathy Conclusion: In this case a 20 year old male patient with history of difficulty in walking and difficulty in holding slippers since 15 years of age, involuntary movements of both hands and frequent falls since 17 years of ageOn examination:Distal muscles of upper and lower extremities are weak 4/5 bilateralSensory examination graded decrease in pain and temperature below hip and below shoulders bilaterally, decreased joint position and proprioception below ankle on both sides. Bilateral KF ring positive, USG abdomen altered echotexture of liver with elevated liver enzymes, serum ceruloplasmin 6.6 mg/dl, 24 hour urine copper excretion increased 120 mcg/day.mri brain T2/flair hyperintensities in B/L putamen, thalamus, caudate and midbrainNerve conduction study revealed primary demyelination with secondary axonal changes. After starting treatment with D -penicillamine and pyridoxine patient frequency of falls and walking and involuntary movements improved

Award ID: 615

Convulsion Without Cause- A Case of Complete Recovery

Nitisha Goyal

Neurology, Paras Hospitals, Gurugram, Haryana, India

E-mail: nitishagoyal@gmail.com

Background and Aim: Introduction: Cryptogenic -New Onset Refractory Status Epilepticus (C-NORSE) is a clinical presentation, not a specific diagnosis, in a patient with new onset of refractory status epilepticus, when the etiology remains unknown despite extensive work-up. Methodology: Case: A 38 years male patient presented to ER with generalized convulsive status epilepticus ongoing for last 40 mins. He had 6 days’ antecedent history of fever, headache and nausea with occurrence of 1 episode of generalized seizure each on day4 and day5 of illness. Generalised seizure activity responded to intravenous Lorazepam and Levetiracetam bolus, and revealed focal left lower limb clonic seizure. Focal seizures persisted and patient loaded with Phenytoin, but in vain. Subsequently, patient was intubated, shifted to ICU and started on midazolam infusion under EEG monitoring. Hemogram -leukocytosis with neutrophilia (WBC = 28,520/µL; 90% Neutrophils). MRI Brain- bilateral insular cortex, external capsule, medial temporal lobe and hippocampus T2/FLAIR hyper-intensity. CSF examination -unremarkable. Over next 3 days, multifocal and generalized electrophysiological seizures continued. Patient received further anti-epileptics including Valproate, Lacosamide, Perampanel, Topiramate, Carbamazepine and Ketamine infusion in an attempt to achieve burst-suppression on EEG. Attempts to taper Ketamine or Midazolam infusion were unsuccessful. Result: A diagnosis of possible autoimmune encephalitis was considered. Patient was treated with iv methylprednisolone 1 g daily for 5 days followed by plasmapheresis. 12 hours later, EEG normalized and patient regained consciousness. At 1-month follow up, patient had attained mRS2 without any seizure recurrence. Conclusion: Although the prognosis in NORSE is dismal, early identification and immunotherapy may disrupt the refractoriness of seizures and improve the outcome.

Award ID: 616

Mixed Movement Disorder- An Interesting Case Report

Sujaya Raghavendra

Madras Institute of Neurology, RGGGH & Madras Medical College, Chennai, Tamil Nadu, India

E-mail: sujayar30@gmail.com

Background and Aim: Paediatric movement disorder consists of a comprises a large group of disorders (tics, myoclonus, tremor, dystonia, chorea, Parkinsonism, ataxia), often with mixed phenotypes, difficult to diagnose, ultimately requiring genetic testing to diagnose. Treatment is challenging, and rehabilitation at the right stage improves the quality of life of the child. Methodology: In our case study, a 13-year-old female with history of involuntary posturing of all 4 limbs intermittently for 4 months with poor mathematics skill and slow learning since childhood. there was no similar family history. On examination patient was conscious obeying to commands. She had generalized dystonia and choreiform movements involving right upper limb than lower limb. This used to disappear at night during sleep. She also had postural tremors Result: Routine Investigations done were normal. Ophthalmology examination revealed arcus juvenilis, no KF ring. Serum ceruloplasmin and Serum copper were normal. USG abdomen done was normal. ECHO showed prolapsed mitral valve prolapse. MRI brain was normal she was treated with benzodiazepines, dopamine agonists, anticholinergics and other supportive medication. She was symptomatically better. TMS showed hyperarginemia and serum ammonia levels were mildly elevated, genetic analaysis including dystonia panel and whole genome sequencing has been sent. Conclusion: Hyperargenemia in paediatric and early adolescent population causes spastic paraplegia, seizures, intellectual disability with transient hyperammonimia. Presentation of chorea has also been seen but a mixed movement disorder is rare. This fascinating case study tells us the need of genetic screening of such children and IQ assessment for them to lead a good quality of life along with timely treatment

Award ID: 617

FDG PET Brain Findings in Various Phenotypic Variants of Progressive Supranuclear Palsy

Priyanka sehrawat, Achal Kumar Srivastava, Madhavi Tripathi, Roopa Rajan, Awadh Kishore Pandit, Divya MR, Animesh Das, Ayush Aggarwal

Neurology, All India Institute of Medical Sciences

E-mail: priyankasehrawat9609@gmail.com

Background and Aim: Heterogeneous clinical findings characterize Progressive Supranuclear Palsy (PSP), an atypical parkinsonian syndrome. Movement Disorders Society criteria 2017 classified PSP into different phenotypic variants based on clinical findings. We aimed to classify PSP patients into different phenotypic variants and determine whether these have differential findings on FDG PET brain imaging. Methodology: We conducted a cross-sectional, observational study at a tertiary care centre in India. Patients were divided into different phenotypes and their clinical characteristics were assessed. FDG PET brain imaging was performed. Visual analysis and cortex ID measurement were used to analyze the imaging characteristics. Areas of hypometabolism were compared across variants using the Kruskall Wallis test and p value of <0.05 was taken to be statistically significant. Result: Out of 82 patients of PSP, PSP-RS constituted 34.1% cases followed by PSP-P (22%), PSP-CBS (19.5%), PSP-F (15.9%), PSP-SL (4.9%). 82.9% of patients had slow vertical saccades, 75.6% had vertical supranuclear palsy, 21.9% had repeated unprovoked falls, 15% showed levodopa responsiveness. FDG-PET brain imaging indicated statistically significant hypometabolism in midbrain in PSP-RS and PSP-P (p value = 0.004), left putamen in PSP-CBS and PSP-P (p value = 0.022) and right temporal cortex in PSP-CBS (p value = 0.001). Conclusion: The FDG PET brain patterns vary among subtypes of PSP. FDG PET brain may be used as a complementary tool for the diagnosis and classification of PSP. The potential of FDG PET brain as a biomarker needs to be explored in large prospective studies.

Award ID: 619

Fulminant Idiopathic Intracranial Hypertension with Blindness, Complete Ophthalmoplegia and Polyradiculopathy

Pramod S, Sowmini P R, Malcolm Jeyaraj K, Sakthi Velayutham S, Viveka Saravanan R, Mugundhan K

Neurology, Stanley Medical College, Tamil Nadu, India

E-mail: pramod.sam13@gmail.com

Background and Aim: Idiopathic Intracranial Hypertension (IIH) is characterised by elevated intracranial pressure in the absence of a structural, parenchymal, or cerebrospinal fluid (CSF) abnormality and absence of focal neurologic deficits.1,2 Sixth nerve palsy is an exception which is seen most often as a false localising sign1. Fulminant IIH is defined by its acute onset and rapid worsening of vision over days1. Extensive radiculopathy has rarely been reported in the setting in intracranial hypertension3. We present a unique case of fulminant IIH complicated by complete ophthalmoplegia, blindness and polyradiculopathy. Methodology: A 23-year-old lady presented with raised ICP headache of 3 weeks duration. Later she developed double vision, gradual decrease in vision and neck pain, radiating to both upper limbs associated with numbness in medial 3 fingers bilaterally. Examination revealed diminished visual acuity and fundus showed bilateral established papilledema. Extra Ocular Movements (EOM) were completely restricted in all directions. Deep Tendon Reflexes were absent in all four limbs with normal bulk, tone and power. Sensory and cerebellar examinations were normal. Result: Nerve conduction study revealed normal CMAPs and SNAPs with absent F-waves in bilateral median, ulnar, tibial and peroneal nerves. CSF study revealed grossly elevated opening pressure with normal protein and sugar levels and was acellular. She underwent emergency theco-peritoneal shunting and post procedure her EOM improved significantly with moderate improvement of vision in both eyes. Conclusion: We propose that in particularly severe cases of intracranial hypertension, an under-recognised syndrome of polyradiculopathy with absent or prolonged F-waves may result along with complete ophthalmoplegia and blindness. CSF shunting may lead to a better outcome than medical management in these patients, particularly with regard to preservation of vision.

Award ID: 620

A Rare Case of Steroid Responsive Encephalopathy Associated with Autoimmune Thyroiditis Presenting with Gyriform Restriction

Marian Vijay, Jawahar M, Swathi T, Durgam Sathya Tejaswini

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: neuromarian@gmail.com

Background and Aim: Hashimoto’s encephalopathy (HE), a rare condition, presents as acute or subacute encephalopathy with high levels of anti thyroid antibodies. MRI brain may be normal or can show T2/ FLAIR hyper intensities in sub cortical white matter, generalised cerebral atrophy, dural enhancement. Gyriform resyriction is rarely reported in these patients. With this background, we report a case of 43 year old gentleman with steroid responsive encephalopathy presenting with gyriform restriction. Methodology: We report a case of 43 year old gentleman, with h/o seizures, presented with break through seizures followed by behavioural disturbances and cognitive decline. Examination revealed impaired lobar functions, axial and appendicular rigidity, dystonic posturing, release reflexes. Result: Aetiological workup was done. Imaging showed diffusion restriction in bilateral basal ganglia and gyriform restriction in temporal lobe. Evaluation showed high anti thyroid antibodies. Other work up was negative. He was treated with pulse Methyl prednisolone, followed by IVIG. Patient improved and repeat imaging showed resolution of diffusion restriction. Conclusion: Our case is unique of its kind, where in there is rare imaging finding of gyriform restriction in steroid responsive encephalopathy (SREAT) with autoimmune thyroiditis. To conclude, there can be varied imaging findings in SREAT and high index of clinical suspicion is required to diagnose this condition. Prompt treatment will resolve the clinical picture.

Award ID: 621

Biotinidase Deficiency- A Treatable Neurometabolic Disorder in Paediatric Population at a Tertiary Care Hospital

C. Leema Pauline, Neeraj Elango, Jered Livingston

Paediatric Neurology, Institute of Child Health and Hospital for Children, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: leema.pauline@rediffmail.com

Background and Aim: Background: Biotinidase deficiency is an autosomal recessively inherited disorder in which the vitamin, biotin, cannot be appropriately recycled. It is a rare congenital metabolic disorder with an incidence of 1 in 60,000 births. Biotinidase deficiency is one of the causes for metabolic epilepsy which is easily treatable. Herewith we present the clinical features of nine children with biotinidase deficiency. Methodology: Methods: Children diagnosed as biotinidase deficiency and on follow up in the neurology outpatient department of our hospital from 2015 to 2021 were included and their clinical features, and outcome were analysed. Result: Results: There were nine children diagnosed as biotinidase deficiency during this period. Six children (66.6%) had onset of seizures in the first three months of life. The female: male ratio was 2:1. Generalised seizures were the predominant seizure type in 5 (55.6%) followed by focal in 3 (33.3%) and myoclonic in one (1.1%) child. None had neonatal seizures. Cutaneous manifestations-seborrheic dermatitis and or alopecia was seen in 5 (55.6%) children. One (1.1%) presented with metabolic acidosis. Hypomyelination in neuro imaging was noticed in 3 (33.3%) children, and abnormal electro encephalogram in 4 (44.4%). Eight children (88.9%) had profound and one (1.1%) partial deficiency. All showed complete control of seizures in 48 hours. Four (44.4%) patients had developmental delay and spastic diplegia. Three (33.3%) had optic atrophy, and 2 (22.2%) hearing loss. Conclusion: Conclusion: Biotinidase deficiency should be considered in any child presenting with seizures with alopecia and/or seborrheic dermatitis and in infants less than 3 months of age with recurrent seizures, even without neurocutaneous manifestations. Universal neonatal screening to identify this treatable neurometabolic disorder is essential to prevent the development of permanent neurological sequelae.

Award ID: 622

CT Angiographic Imaging Patterns Differentiate Pseudo and True Occlusion of the Proximal Internal Carotid Artery in Acute Ischemic Stroke

Sayooja Sachithanandan, Harikrishnan Ramachandran, Sylaja P. N., Sapna Erat Sreedharan, Jayadevan E. R., Harikrishnan Ramachandran, Sylaja P. N., Sapna Erat Sreedharan, Jayadevan E. R., Santosh Kannoth

Comprehensive Stroke Care Program, Department of Neurology, Sreechitra Tirunal Institute of Medical Sciences and Technology, Kerala, India

E-mail: sayooja.04@gmail.com

Background and Aim: Identifying pseudoocclusion (PO) of the proximal internal carotid artery (ICA) from true distal ICA occlusion has implications on successful recanalization and outcome after endovascular thrombectomy (EVT) in acute ischemic stroke (AIS). The aim of this study was to identify the CT angiographic patterns in differentiating a true occlusion from PO and to analyze the rate of successful recanalization after EVT Methodology: This was a retrospective cohort study of patients with AIS and proximal ICA occlusion who underwent mechanical thrombectomy from 2014 to 2021. The patterns of carotid occlusion in CT angiogram (beak, dome or flat pattern) were reviewed and correlated with Digital subtraction angiography (DSA) images and categorized into PO and true occlusion. The rate of successful recanalization in PO and true occlusion were analyzed Result: Of the 24 patients 16 (66%) had proximal ICA PO and 8 (33%) had true occlusion in DSA. A beak pattern of the proximal ICA on CTA was significantly higher in the PO group (87.5% vs. 25%, p value = 0.005), and a flat pattern was significantly higher in the true occlusion group (50% vs. 12%, p value = 0.005). A gradual contrast decline of the proximal ICA on CTA images was seen only in the PO (85.7%, p value = 0.05). There was no significant difference in the rate of successful recanalization between PO and true occlusion (81.25% vs 62.5%, p = 0.362) Conclusion: The imaging patterns of proximal ICA in CTA can differentiate PO from true occlusion. This can help in planning intervention strategies and prognostication

Award ID: 623

NUMB CHIN Sign: Think of the Omnious

Chitrangada Yadav

Neurology, All India Institute of Medical Sciences, Delhi, India

E-mail: ychitrangada@gmail.com

Background and Aim: Numb chin syndrome (NCS) is a condition where there is altered sensation along the distribution of the mandibular branch of the trigeminal nerve without any obvious odontogenic cause Methodology: A 19 years-old-girl presented with complaints of intermittent fever, 5 kilograms weight loss since the past 2 months and sudden onset numbness of chin for 20 days. Examination was non-revealing apart from a numb chin. Routine blood investigations and cerebrospinal fluid examination (including malignant cytology) were normal. Magnetic resonance imaging of her brain (with contrast) revealed enlargement, along with contrast enhancement of the left Meckel’s cave. Serum ANA, ENA profile, ACE and viral markers were also non revealing. Bone marrow biopsy revealed hypercellular marrow with sheets of medium sized atypical cells consistent with acute leukemia of B lymphoid origin. She was treated with chemotherapy for the same but unfortunately succumbed to the illness. Figure 1 A- Numb chin B- MRI Brain (T1+C) showing deposits in the left Meckel’s cave C- IHC stain revealing atypical cells (blasts) with round nuclei, indistinct nucleoli and scant cytoplasm Result: Though rare, numb chin sign may be the manifestation in the presentation of an underlying malignancy 2. The most common primary malignancy being lymphoma or breast cancer that has metastasized to mandible 1,3 Conclusion: Presentation with numb chin, especially in young individuals should raise alarm bells and lead to a thorough search for an underlying malignancy.

Award ID: 624

“Early Neurological Deterioration after AcuteIschemic Stroke and Long Term Functionaloutcome- A Prospective Cohort Study”

Archana Sharma, Awadh Kishor Pandit

Neurology, AIIMS, New Delhi, India

E-mail: archanasharma0411@gmail.com

Background and Aim: Early neurological deterioration (END) in acute ischemic stroke is associated with increased risk of functional disability and mortality. In the present study we evaluated the association between END in patients with acute ischemic stroke and their long-term functional outcome (modified Rankin Scale - mRS) at three months. To study the association between the END in patients with acute ischemic stroke and long-term functional outcome at three months. Methodology: An observational prospective study was carried out where 190 patients of acute ischemic stroke were enrolled and assessed for END within first five days of onset and assessed at three months for outcome in the form of mRS. END was defined as a decrement of least one point in motor power or increment in National Institute of Health Stroke Scale (NIHSS) ≥2 points within the first 3 days after admission. We explored the association between END and long term outcome and the clinical, biochemical and radiological risk factors of END. Result: 17.8% of the acute ischemic stroke patients experienced END during the acute phase and 94.1% showed poor functional outcome and 64.7% had died. END was associated with high blood glucose at admission and low serum albumin after adjusting for related factors like hypertension, diabetes, NIHSS at admission and laboratory parameters, including direct bilirubin, total cholesterol, low-density lipoprotein, glucose, CRP and homocysteine. Conclusion: END was associated with a poor functional outcome and increased mortality at three months. High blood glucose at admission, low serum albumin were independent risk factors of END in the present study.

Award ID: 625

Thiamine Polyradiculo Neuropathy Mimicking as Guillian Barre Syndrome

Balasubramanian Samivel, Nageswaran B, Arun Kumar N, Uma Maheshwari E, Lakshmi Narasimhan Ranganathan

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: balaprammu@gmail.com

Background and Aim: Introduction: Nutritional neuropathy is a well recognised clinical entity. Suspecting them in appropriate clinical settings and treating them will yield better results. We are here by discussing two cases of thiamine polyradiculo neuropathy mimicking as GBS. Methodology: Case vignette 1: A 32 year old antenatal mother with history of Hyperemesis gravidarum presented with weakness involving all four limbs since 10 days with severe burning parasthesias involving both upper and lower limbs. On examination she had distal more than proximal weakness with generalized hyporeflexia. She also had severe hyperalgesia involving both upper and lower limbs. Result: Case vignette 2: A 42 year old male who is a chronic alcoholic presented after a binge of alcohol intake with acute weakness involving all four limbs with burning parasthesias involving both hands and feet. On examination he had areflexic flaccid quadriparesis with severe hyperalgesia involving both hands and feet. Conclusion: Both patients were admitted with provisional diagnosis of Acute poly radiculoneuropathy. Nerve conduction studies showed features of sensory motor axonal neuropathy. With history of symptoms developing after a binge alcohol drink and multiple episodes of vomitings, thiamine polyradiculo neuropathy was suspected. Both patients were treated with IV Thiamine following which proximal muscle power improved first followed by distal muscles and finally sensory symptoms. In appropriate clinical setting with severe sensory symptoms nutritional neuropathy should be suspected which can mimic as Guillian-Barre Syndrome. So If recognised early and treated promptly it will give good results.

Award ID: 626

Does Locomotion Relates to the Reproduction of Rhythm in Patients with Parkinson’s Disease

Suchismita Majumdar, Tanisha Majumdar, Supriyo Choudhury, Asit Baran Bayen, Piali Chatterjee, Hrishikesh Kumar, Stuart Baker, Mark Baker, Hrishikesh Kumar

Department of Neurology, Institute of Neurosciences, Kolkata, West Bengal, India

E-mail: suchismita.biotech@gmail.com

Background and Aim: Gait impairment is frequently seen in patients with Parkinson Diseases (PD). It could be associated with deficiency in overall sense of rhythm as bipedal human locomotion is a rhythmic activity. In the current study, we compared the rhythm-reproduction between PD patients and healthy control (HC) through a neurophysiological task and examined the correlation of the gait with rhythm-reproduction for PD patients. Methodology: 30 PD patients and 20 age matched HC were evaluated on a rhythm task where participants were initially asked to tap fifteen times with a simple beep-sound (equally spaced) and then asked to continue the tapping for another fifteen times maintaining same rhythm in absence of the beep-sounds. The participants were presented with three different time intervals (800 ms, 1500 ms, 1800 ms) between beep-sounds. Their gait parameters were objectively assessed through a gait mat (GAITRite) embedded with grids of pressure sensors. Result: Unexpectedly, the finger tapping of Parkinson’s patients was rapid (by 295 ms and 245 ms) than healthy in the continuation phase of rhythm task (800 ms, 1500 ms, p < 0.05) and they went faster (by 260 ms) while synchronizing with rhythms of 800 ms (p < 0.001). This distorted perception of rhythm correlated with gait impairment; velocity (ρ = 0.427, p < 0.05) and stride length deviation (ρ = -0.538, p < 0.005). Conclusion: The suboptimal sense of rhythm of PD patients in our cohort and its correlation with impairment of gait, possibly attributes to the commonality in anatomical substrates of these two activities (gait and rhythm production). Clinically useful tools could be developed in future for screening and therapy of gait impairment through simple rhythm tasks.

Award ID: 627

Concurrent Inflammatory Myopathy and ART Induced Myopathy in a Patient with HIV/AIDS

Pavan Kumar Reddy PG, Sakthivelayutham S, Sowmini PR, Malcolm Jeyaraj K, Viveka Saravanan R, Mugundhan K

Neurology, Govt Stanley Medical College, Chennai, Tamil Nadu, India

E-mail: pavanpg99@gmail.com

Background and Aim: Neuromuscular complications are common in HIV/AIDS patients. Myopathy in a HIV patient can be due to HIV myositis, inflammatory myopathies, ART therapy or other Secondary myopathy may also occur, due to metabolic, infectious, neoplastic and vasculitic processes (1) Methodology: 54 year old gentleman known case of diabetic and HIV on ART from 12 years admitted with complaints of chronic symmetrical proximal muscle weakness of lower limbs since 4 years, and upper limbs since 2 years. He was on ZLN regimen for 10 years and on TLD regimen from past 2 years. He did not have any fasciculations, muscle pains. He also had numbness, paraesthesia of all 4 limbs, since 2 years. On Examination patient has severe wasting of bilateral thigh (predominantly quadriceps) and gluteal muscles, and also minimal wasting noted in legs, bilateral deltoid, forearm and hand muscles. Thigh and gluteal muscle wasting was out of proportion to weakness. Deep tendon reflexes were absent. Sensory examination revealed graded sensory loss in all 4 limbs Result: Laboratory inestiagtions demonstrated a CD4 count of 1040 cells/mm3, and elevated creatinine kinase levels 1020 U/L with normal renal function tests. Vasculitis work up was normal. Nerve conduction studies showed sensory motor axonal neuropathy of all 4 limbs. Electromyography showed myopathic pattern. Muscle biopsy of left vastus lateralis performed which showed features of inflammatory myopathy and ragged red fibers suggestive of mitochondrial pattern. Patient was started on oral steroids and on follow up. Conclusion: We present this case for its rarity of myopathy in HIV infection and to highlight the importance of comprehensive workup which may have therapeutic implications.

Award ID: 628

A Case of Tuberous Sclerosis Presenting with Gelastic Seizures

Naga Srinivas PS, Malcolm Jeyaraj, Sowmini P. R., Sakthi Velayutham S, Vivek Saravanan, Mugundhan K

Department of Neurology, Govt. Stanley Medical College and Hospital, Chennai, Tamil Nadu, India

E-mail: nagasrinivas.amc@gmail.com

Background and Aim: Gelastic seizures, an uncommon epileptic seizure type, with laughter as the main ictal manifestation, have been mostly described in association with hypothalamic hamartomas but laughter may also occur in association with complex partial seizures of frontal or temporal lobe as well as the cingulate gyrus (1). Here we present a child with tuberous sclerosis complex (TSC) who presented with gelastic seizures. Methodology: Case Vignette: A 13 years girl, born out of non-consanguineous marriage with normal birth and development without antecedents, presented with recurrent seizures since 7 years of age, characterized by behavioural arrest, screaming followed by uncontrolled laughter leading on to generalized seizures. The frequency was 6-7 episodes per day. She had facial adenoma sebaceum. MRI brain showed Non enhancingfoci of T2 hyper intensities in right frontal lobe in the parasagittal location and left precentral gyrus suggestive of cortical tubers and on CT correlation, subependymal calcified tubers were noted. EEG showed interictal epileptiform abnormalities in bilateral frontotemporal regions. Result: Discussion: The literature on TSC with gelastic seizures is scarce. Case report of Cook et al, stated that only 2 cases of TSC with gelastic seizures have been reported before (2). However, a clear anatomic pathway for laughter still remains unclear. In our case, gelastic seizures were well controlled with the regimen of lamotrigine, levetiracetam and clobazam. Conclusion: ConclusionWe are reporting this case since gelastic seizures in TSC is a rare phenomenon.

Award ID: 629

Straight Sinus Thrombosis with Bilateral Internal Watershed Infarcts Mimicking Large Vessel Arterial Disease

Sivaroja Yellaturi, Sakthi Velayutham S, Sowmini P R, Malcolm Jeyaraj K, Viveka Saravanan R, Mugundhan K

Neurology, Govt Stanley Medical College Hospital, Chennai, Tamil Nadu, India

E-mail: rojayellaturi@gmail.com

Background and Aim: Watershed area infarcts are a rare occurrence in cerebral sinus venous thrombosis. So far there have only been few cases of watershed infarcts due to thrombosis of deep cerebral venous system reported in the literature.[1] We report one such case of a 17 year old boy with deep cerebral venous sinus thrombosis with internal watershed infarcts and the possible mechanism is discussed. Methodology: A 17 year old boy was brought to our emergency department with history of headache, vomiting and decline in sensorium without accompanying fever or seizures. On examination patient was in confused state without papilloedema, cranial nerve palsy or neck stiffness. Rest of the neurological examination was unremarkable. Result: Magnetic Resonance Imaging of the patient showed multiple small foci of bilateral internal watershed area infarcts in centrum semiovale and corona radiata regions. Susceptibility weighted imaging showed blooming of straight sinus with non-visualisation of straight sinus on venography. Magnetic resonance angiography appeared normal with no evidence of stenosis suggesting venous etiology for the infarcts. His vasculitic profile and coagulation parameters were normal. Patient was started on anticoagulation therapy with which he showed marked improvement. Washida et al have reported a patient with straight sinus thrombosis, who at onset had multiple deep white matter hyperintense lesions on DWI, but the patient subsequently developed hyperintensities in bilateral thalamus and basal ganglia regions.[2]In CSVT, venous pressure is increased secondary to venous obstruction, which leads to decreased capillary perfusion and cytotoxic oedema resulting in the development of diffusion restricting areas.[3] Conclusion: Our case highlights straight sinus thrombosis as a diagnostic possibility in patients with internal watershed area infarcts, especially when the clinical setting is favouring CSVT.

Award ID: 630

Myasthenia Gravis, Thymoma and Candidiasis – A Rare and Unique Association

Kamatchi Sundaramoorthy, Sowmini P. R., Sakthivelayutham S, Malcom Jeyaraj K, Viveka Saravanan R, Mugundhan K

Stanley Medical College and Government Hospital, Stanley Medical College and Government Hospital, Chennai, Tamil Nadu, India

E-mail: kamatchis28@gmail.com

Background and Aim: BACKGROUND: Myasthenia gravis is a well-known and extensively studied autoimmune disorder. About 10% of these patients have associated thymoma1. Here we present a case of myasthenia gravis and thymoma presenting with significant weight loss, oral mucocutaneous and oesophageal candidiasis mimicking an immunodeficiency state. Methodology: CASE REPORT: A 37 year old female came with complaints of right eyelid drooping with diurnal variation of 2 years duration, vomiting immediately after taking food over the past 1 year and she had lost 15 kg body weight over this period. AchR Ab was more than 8 nmol/L. Serum IgG, IgM, IgA levels, CD4 and CD8 ratio were normal. RNS showed decremental response of more than 10% in both orbicularis oculi. CT chest showed lobular mass in the anterior mediastinum without evidence of local infiltration suggestive of thymoma. Upper gastrointestinal endoscopy revealed mid and lower oesophageal candidiasis. In view of oropharyngeal and oesophageal candidiasis workup for HIV, HbSAg, HCV and Good syndrome were done and were found to be negative. Result: DISCUSSION: Very few cases have been published in literature showing association of myasthenia with thymoma and candidiasis. Good syndrome is a rare acquired immunodeficiency syndrome with mortality ranging between 44-50%. However In our case workup for Good syndrome was negative. Conclusion: CONCLUSION: The coexistence of myasthenia gravis, thymoma and candidiasis in the same patient with features of autoimmunity and immunodeficiency proved to be a diagnostic and therapeutic challenge. Recognition of this rare association is essential to decide long term management of these patients.

Award ID: 632

Carbamazepine Induced Abnormal Eye Movements

Ravi K

Neurology, SIMS Hospital, Chennai, Tamil Nadu, India

E-mail: doctorravikumark@gmail.com

Background and Aim: To describe abnormal eye movements associated with carbamazepine administration Methodology: Two patients who were taking carbamazepine presented with abnormal eye movements to a tertiary care hospital in south India Result: Here we describe 2 patients on carbamazepine who presented with different abnormal eye movements which subsided after withdrawal of carbamazepine. One was a direct effect and the other an indirect effect. Case1 45 year old female, a known epileptic on Carbamazepine presented with complaints of dizziness and shaky vision. On examination she had down beating nystagmus. On investigation she had high levels of carbamazepine in the serum. The dosage of carbamazepine was decreased leading to complete resolution of the nystagmus. Case2 57 years old male, known case of seizure disorder on valproate, presented with history of giddiness and headache for a duration of 15 days, associated with gait unsteadiness. He had been recently started on carbamazepine in addition to sodium valproate. Examination revealed oculogyric crisis and ataxic gait. Baseline investigations revealed deranged liver parameters. Serum Ammonia level was high (111). Serum Carbamazepine level was normal. After carbamazepine was stopped, Oculogyric crisis subsided within 24 hours. His ataxia also subsided. He was able to walk without support. Conclusion: Downbeat nystagmus due to carbamazepine is a rare phenomenon. Very few case reports have been published. Oculogyric crisis due to hyperammonemia (in this case caused by addition of carbamazepine) is also a rare phenomenon. Care should be exercised when adding a hepatotoxic drug to another as this can precipitate hyperammonemia

Award ID: 633

Sensory Ataxic Neuropathy With Dysarthria & Ophthalmoplegia (SANDO) - A Multisystem Mitochondrial Disorder

Usha Budumuru, Sowmini PR, Malcolm Jeyaraj K, Sakthivelayutham S, Vivekasaravanan R, Mugundhan K

Neurology, Stanley Medical College/ Hospital, Chennai, Tamil Nadu, India

E-mail: usha.budumuru113@gmail.com

Background and Aim: Mitochondrial diseases are clinically heterogeneous and have complex inheritance patterns resulting in incorrect or delayed diagnosis. We report a case of Mitochondrial cytopathy with sensory ataxic neuropathy, dysarthria and ophthalmoplegia in a middle aged female Methodology: A 36 year old lady presented with insidious onset, gradually progressive neurological illness in the form of distal, symmetrical, flail weakness of both upper limbs and lower limbs (lower limbs > upper limbs) of 8 years duration, associated with numbness and paresthesias in all 4 limbs of 6 years duration Neurological examination revealed mild dysarthria, mild ptosis in left eye, restriction of eye movements in all directions with out double vision, absent deep tendon reflexes in lower limbs, and glove and stocking pattern of graded sensory loss for all modalities of sensations in all 4 limbs. Vibration perception over spine was reduced. Romberg’s sign was positive Result: Laboratory investigations revealed normal blood parameters with out muscle/ liver enzyme elevation. Vitamin B12 levels were normal. Vasculitic workup was negative. NCS demonstrated severe sensory motor axonal neuropathy of all 4 limbs. Needle EMG showed features of chronic denervation. MRI Brain was normal. Muscle biopsy of peroneus brevis muscle demonstrated ragged red fibres and COX negative fibres, features suggestive of mitochondrial myopathy. Superficial peroneal nerve biopsy shows features of severe axonal neuropathy. Conclusion: The term “SANDO” syndrome characterized by an adult-onset and severe form of sensory ataxic neuropathy, dysarthria, and chronic progressive external ophthalmoplegia, results from mitochondrial dysfunction and is due to mtDNA depletion in muscle and peripheral nerve. The phenotype is largely variable. Mitochondrial disorders should always be considered in the differential diagnosis of neurological diseases with multi axial involvement.

Award ID: 634

An Atypical Case Presentation of Leukodystrophy

Vaishali Bagrodia, Purba Basu, Soumava Mukherjee, Nilam Singh, Supriyo Chowdhury, Hrishikesh Kumar

Neurology, Institute of Neuroscience

E-mail: vaishalibagrodia@gmail.com

Background and Aim: Leukodystrophies are rare, genetic disorders characterized by abnormal white matter growth in the brain. The aim is to describe a unique case of leukodystrophy that presented with movement disorder. Methodology: A 42 year old female was born out of non-consanguineous parentage with full term forceps delivery and delayed cry. All developmental milestones were delayed. By age 3, her speech was dysarthric and gait was abnormal. She developed bowed knees and was wheelchair bound by age 9. These symptoms did not significantly worsen for next 30 years. Cognition was preserved. Cerebral palsy (CP) was diagnosed due to history of birth asphyxia and delayed milestones. Since 2020, she complained of posturing, intermittent spasms and abnormal movement of her right arm. Examination revealed generalized dystonia, ataxia in right upper limb, dysarthria and brisk DTRs. Intermittent tremor with dystonic posturing was observed in the right arm. Complains of electric shock like sensation was also noted in the limbs. No family history of similar illness. Result: MRI shows extensive white matter hyperintensity sparing grey matter, brain stem, cerebellum consistent with leukodystrophy. Clinical exome sequencing detected a pathogenic variant of a homozygous single base pair duplication in exon 2 of the MLC1 gene. Conclusion: This is a unique case because leukodystrophy presenting with movement disorder is a rare phenomenon. Furthermore, the patient was diagnosed with CP which could suggest a dual pathology but no such case has been previously reported. Provisional cases of CP should be however be further investigated for heredodegenerative diseases if symptoms change or progressively worsen.

Award ID: 635

Assessment of Praxis in Healthy Individual to Obtain Normative Data and to Validate in Patients with Dementia

S Sandeep Kumar, Atanu Biswas, Adreesh Mukherjee

Department of Neuromedicine, BIN, IPGMER

E-mail: sandeep1987.ssk@gmail.com

Background and Aim: Apraxia is a disorder of learned skilled movement in the absence of motor or sensory deficits or any general cognitive impairment such as inattention to commands, object recognition deficits or poor oral comprehension. Due to lack systematic culturally validate instruments we here tried to correlate demographic influence over limb praxis assessment and negate these influences by obtaining normative data in a standard praxis assessment scale (TULIA). Methodology: This is a cross sectional observational study. Healthy adults above 18 yrs without any neurological or cognitive impairment along with cases who are diagnosed as dementia as per DSM 5 criteria were included inner study. We used TULIA for assessment of praxis in our subject and case. Result: Among healthy subjects non symbolic and intransitive gestures are performed better than transitive gestures. Most common error committed while performing transitive pantomime tasks was spatiotemporal error. We assessed the validity of our tool by applying it on dementia patient. We found that patient with dementia scored less than healthy subjects. Conclusion: This test can be administered easily and was able to differentiated healthy individual from patient with apraxia.

Award ID: 636

Extra Medullary Intra Cranial Hematopoeisis - A Rare Cause of Seizure

Sucharitha M V, Thomas Mathew, G. R. K. Sarma, Raghunandan Nadig, Sagar Badachi, Delon Dsouza, Sonia Shivde

Neurology, St Johns Hospital

E-mail: sachu.bunty@gmail.com

Background and Aim: Extramedullary hematopoiesis (EMH) is described in patients suffering from bone marrow failure. Most common cause is often thalassemia in children and myelofibrosis in adults. EMH mostly arises in the liver, the spleen, the lymph nodes, or in the mediastinum; it rarely affects the dura. Intracranial EMH is an even rarer entity. Meningeal EMH may present as headache, drowsiness, disorientation, epilepsy and hemiparesis. Here we report a case of aplastic anemia with intracranial extramedullary EMH presenting with seizures. Methodology: A 48 y /female, diagnosed case of transfusion dependent Aplastic anemia presented with history of tremulousness of both lower limbs, on examination she had spasticity with brisk reflexes. MRI brain was advised which revealed features suggestive of EMH in the parafalcine region. Tissue biopsy was planned but was deferred in view of refractory thrombocytopenia. Few months later she had focal seizures of the lower limbs. Result: She was started on anti epileptics and cranial irradiation was given. Metabolic work up for cause of seizures was done, which was normal. She received 5 cycles of cranial irradiation, follow up scan revealed decrease in the size of EMH. Seizure frequency has decreased and she is symptomatically better. Conclusion: In a patient with aplastic anemia presenting with seizures neuroimaging must be advocated to look for any Dural or meningeal EMH as it can be treated with irradiation or surgery.

Award ID: 637

Worsening Motor Weakness in Genetically Proven Congenital Myasthenic Syndrome: A Novel Association with Hidden Culprit

Sandhya Manorenj, Reshma Sultana Shaik, Rindha Venepally Rao

Neurology, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Background: Congenital Myasthenic Syndrome (CMS) are a group of rare genetic disorders of the neuromuscular junction, which typically manifests in early childhood. Although cognitive impairment, cerebellar dysfunction and epilepsy have been described in CMS, psychiatric manifestations are seldom reported. Aim: Here we report a case of genetically proven CMS with Schizophrenia Methodology: Method: A case report study Result: Case report: A 32 year old gentleman, presented with worsening of weakness since 6 months. He had a history of fatigable weakness in the form of limb girdle weakness with partial ptosis, since the age of 5 years for which he was on Acetylcholine-esterase inhibitors (AChEI) with partial improvement. He had history of formed visual, auditory hallucinations, and paranoid delusions since the age of 30 years and was diagnosed as “Paranoid Schizophrenia” by the psychiatrist for which he was on olanzepine 10 mg daily since 8 months. Examination revealed bifacial weakness bilateral ptosis, fissured tongue and high arched palate. Genetic testing revealed homozygous mutation in CHRNE. He was treated with salbutamol along with the AChEI. Olanzepine was stopped and amisulpride 100 mg /day was started for chronic schizophrenia. At 2 weeks he showed complete remission of his motor weakness Conclusion: Conclusion. The anticholinergic effects especially of the atypical antipsychotic agents though show a highly selective muscarinic receptor blockage, they cause a small but clinically significant blockade of acetylcholine at the nicotinic receptor sites, thus exacerbating the weakness. Clinician should be aware of this entity and should be careful while initiating antipsychotics in such rare associations (CMS and schizophrenia).

Award ID: 638

A Misdiagnosis of Cerebral Palsy in a Case Of PLA2G6 Associated Neuroregression

Sabyasachi Pattanayak, Deepika Joshi, Anand Kumar, Vijay Nath Mishra, Rameshwar Nath Chaurasia, Abhishek Pathak, Varun Kumar Singh

Neurology, IMS-BHU, Varanasi, Uttar Pradesh, India

E-mail: sabya.pattanayak4@gmail.com

Background and Aim: the infantile neuroaxonal dystrophy form of PLA2G6 associated neurodegeneration spectrum usually presents by the age of three months to six years. Children usually present with psychomotor delay, spastic quadriparesis and visual difficulty. In presence of confounding history of any birth insult, misdiagnosis is a possibility. In this report we present a case of PLA2G6 related INAD along with the diagnostic challenges and clinical features Methodology: The patient was a four year old female who presented to us with spastic quadriparesis and neck floppiness with progressive cognitive decline for past 2 years. She was borne out of a non-consanguineous marriage a with history of birth asphyxia. She had achieved all her milestones till 2 years of age following which she had deterioration. She had been diagnosed as cerebral palsy due to the confounding birth history. We reassessed her history and clinical examination and a similar history in the younger sibling prompted us to assess for a genetic condition Result: Her genetic test results reported an likely compound heterozygous variant for gene transcript for PLA2G6 in exon 13, exon 3 respectively. Conclusion: INAD is an important differential diagnosis to consider in patients with early, rapid cognitive and motor regression. An high index of suspicion for a genetic disease is necessary in these cases.

Award ID: 639

An Unusual Case of Vascular and Neural Conflict

C. Lalawmpuia, Amit Batra, Sanjay Saxena

Neurosciences, Max Superspeciality Hospital Patparganj, Delhi, India

E-mail: lalawmpuia63@gmail.com

Background and Aim: Vascular etiologies of radiculopathy/neuropathy are underestimated as they have atypical presentation. Most of published literatures have reported vascular radiculopathy in the context of aneurysm, malformation, hemorrhage or hematoma. Abdominal aortic thrombus is rare vascular cause of neuropathy, with unclear pathophysiology and variable presentations. Here, we report a 63-year-old male, with clinical presentation suggestive of left common peroneal nerve neuropathy secondary to left popliteal and aortic bifurcation thrombus, needing urgent surgery. Methodology: A 63-year-old patient CAD Post PTCA with complaints of acute lower back pain, paraethesia of both limbs, left > right, with left lateral severe leg pain with partial left foot drop. MRI Spine was suggestive of marginal lumbar osteophytes with minimal PIVD L4/5, L5/S1. NCV was suggestive of left common peroneal neuropathy with entrapment across knee. MRI Leg done showed neurogenic/denervation oedema in left T. antrerior but no neural compression/ entrapment. USG arterial Doppler showed echogenic thrombus filling left popliteal artery. CT PA was done which showed infrarenal aortic occlusion extending up to bilateral common iliac and left popliteal artery thrombotic occlusion. His Echo cardiogram was normal. Result: Patient underwent left popliteal embolectomy and femoral artery embolectomy with gradual improvement in pain and lower limb power and was discharged on oral anticoagulants. Conclusion: It is important to include vascular aetiology as differential diagnosis of acute leg pain especially when MRI spine is normal, as it may require urgent intervention.

Award ID: 640

A Study on Correlation Between Retinal Nerve Fiber Layer (RNFL) Thickness in Spectrum of ALS Patients in a Tertiary Care Hospital

Bijendra Mohanty

Neurology, NRS Medical College and Hospital, Kolkata, West Bengal, India

E-mail: bijenmohanty@icloud.com

Background and Aim: The primary goal of this study is to provide evidence regarding retinal changes in ALS patients and identify correlation between RNFL thickness in spectrum of ALS patients and its association with disease duration and severity. Methodology: Study was designed as cross-sectional study and the diagnosis of ALS was made based on the basis of El Escorial criteria and included along with healthy controls. ALSFRS-R score was used to assess functional status of patients. RNFL thickness in the four quadrants were measured with a spectral domain OCT & all data were analysed. Result: We included 30 cases and 10 controls having mean age of 49.5 years (65%-middle-aged 41 to 60 years). This study has found statistically significant differences in RNFL thicknesses between ALS patients & controls. On segmental analysis, right eyes superior, nasal quadrant and in left eye superior, inferior and nasal quadrant were significantly affected along with gross asymmetry between left and right eye among ALS patients. On comparative analysis we got significant decrease in average RNFL thickness in definite ALS patients with significantly reduced average RNFL thickness in moderate to severe group. On correlation analysis disease duration showed good negative correlation and ALSFRS-R score demonstrated good positive correlation with bilateral average RNFL thickness. Conclusion: The analysis showed significant decrease in RNFL thickness in ALS patients in comparison to controls and it decreases significantly with disease severity and duration. Therefore, the use of OCT in ALS patients could be recommended as a biomarker of neurodegeneration and to assess progression of disease.

Award ID: 641

To Identify Clinical and Neuro-Ophthalmological Predictors of Visual Outcome in Patients with Ethambutol-induced Optic Neuropathy (EtON)

Megha Varshney, Prabhjit Kaur, Aastha Takkar, Karthik Vinay Mahesh, Basavaraj Tigari, Vivek Lal

Neurology, Post Graduate Institute of Medical Education and Research

E-mail: meghavarshney87@gmail.com

Background and Aim: Background: Ethambutol-induced Optic Neuropathy (EtON) has been recognized since 1962 and with the change in treatment guidelines in India increased incidence of EtON has been observed. Thus, early diagnosis or recognizing the predictors of worse progression is of utmost importance which may help in altering natural course and reducing visual morbidity. Aim: To Identify clinical and neuro-ophthalmological predictors of visual outcome in patients with EtON Methodology: 20 patients diagnosed clinically with EtON were recruited. Detailed neurological, ophthalmological and radiological examination were carried out in patients including Visual Acuity, Color Vision, Visual Fields, Fundus Photography, OCT-RNFL, OCT-GCL, and CEMRI-Brain with Optic Nerve Cuts. Result: Mean age (SD) of the patients were 43.63 (13.75), male: female ratio = 1:1.31 and time to onset after initiation of ethambutol was 5.51 ± 4.1781 months. 10 patients had pulmonary TB and 10 had EPTB. 15 patients developed symptoms even at dose of 15-20 mg/kg/day with diabetes in 16 patients and hypertension in 10 patients. At presentation, patients presented with bilateral, symmetrical and severe vision loss (93% with >6/60) which improved to Grade 1 in follow-up. The average OCT RNFL and GCL thickness observed in our study were 95.8 ± 22.4 mm and 39.65 ± 13.5 mm respectively and it was found that despite stopping the drug RNFL and GCL layer thinning in all quadrants continued to occur suggesting structural damage to the optic nerve. Conclusion: Duration of symptoms onset after ethambutol intake for 5.96 months was associated with poor visual outcome. Moreover, lower thickness of RNFL and GCL in temporal quadrant at baseline found to be statistically associated in predicting poor visual outcome with a sensitivity of 85.2% and 93%.

Award ID: 642

Spastic Paraplegia Type 8 (SPG8): First Report from India

Rohan R. Mahale, Sneha Kamath, Ravindranadh Mundlamuri, Hansashree Padmanabha

Neurology, National Institute of Mental Health & Neurosciences (NIMHANS)

E-mail: rohanmahale83@gmail.com

Background and Aim: Spastic paraplegia type 8 (SPG8) is an autosomal-dominant form of hereditary spastic paraplegia (AD-HSP) caused by a mutation in the KIAA0196 gene. It accounts for 1% of less of all AD-HSP. Hereby, we report a middle-aged male who presented with spastic paraparesis of 3 years duration with similar history in his father (age at onset-60 years). Whole exome sequencing showed missense mutation in WASHC5 gene (p.Val626Phe) which is a pathogenic variant. SPG8 has not been reported from India. Methodology: Case report of one patient who had missense mutation in WASHC5 gene suggestive of SPG8. Result: A 45-year-old male patient born out of non-consanguineous parentage presented with slowly progressive stiffness in bot lower limbs with urinary urgency of 3 years duration. There was no wasting, sensory symptoms, vision, hearing loss, cognitive disturbance or ataxia. His father had similar symptoms at 60 years of age and was wheelchair bound by 5 years. MRI spine and brain was normal. Whole exome sequencing showed missense mutation in WASHC5 gene (p.Val626Phe) NM_014846.4(WASHC5):c.1876G>T. The p.Val626Phe missense variant is predicted to be damaging by both SIFT and PolyPhen2. The valine residue at codon 626 of WASHC5 is conserved in all mammalian species. The nucleotide c.1876 in WASHC5 is predicted conserved by GERP++ and PhyloP across 100 vertebrates. The clinical phenotype of the proband matches with that of the disorder caused by pathogenic variants in WASHC5 gene. For these reasons, this variant has been classified as Pathogenic. Conclusion: We report first case of SPG8 from India.

Award ID: 643

A Rare Case of Reversible Splenial Lesion Syndrome with Atypical PRES in a Post Natal Woman

Jayasurya Suresh, Saravanan Sankaralingam

Department of Neurology, Tirunelveli Medical College, Tamil Nadu, India

E-mail: drjayasuryasuresh@gmail.com

Background and Aim: Reversible splenial lesion syndrome (RESLES) is a rare clinico-radiological neurological syndrome which involves acquired reversible lesions in the splenium of the corpus callosum, which occurs secondary to infections, toxins exposure, metabolic disturbances, seizures etc. Methodology: 29 year old female, P1L1, with history of gestational hypertension from 7th month of gestation, with significant past history of native medicine exposure and history of skipping Labetalol on her own for one week post delivery, presented with sudden onset gradually progressive neurological illness in the form of dysarthria, deviation of angle of mouth to right side, drooling of saliva, dysphagia, peri oral tremors and hypo aesthesia over left half of the body of one day duration with no evidence of motor weakness, higher mental function involvement, bowel bladder involvement or meningeal involvement. MRI brain with contrast showed T2 and FLAIR hyperintensity with diffusion restriction in bilateral centrum semi ovale, corona radiata and splenium of corpus callosum. Patient was diagnosed as RESLES secondary to toxin exposure with atypical PRES. The patient was treated with Labetalol, Clonazepam and THP. Patient improved significantly in the due course of hospital stay. Result: Toxin exposure and atypical PRES lead to the neurological symptoms in this patient which responded well to the treatment Conclusion: RESLES secondary to toxin exposure is a treatable condition and atypical neurological presentation of PRES responds well to BP control. This kind of co existent neurological clinical presentation is rare in a post natal woman and hence presenting this case.

Award ID: 644

A study on Depression and Migraine Disability among Migraine Patients

Vishnu M S, Lakshmi Narasimhan Ranganathan, Chandramouleeswaran V, Lakshmi Narasimhan R, K. K. Shanmugasundaram, J. K. Manickavasagam, Jawahar M, Lakshminarasimhan Ranganathan

Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: vishnumsath@gmail.com

Background and Aim: Migraine is the second most prevalent primary headache. Even though it is a major cause of disability, studies done to understand the burden and impact of migraine on daily living remains less. Migraine is associated with depression. Hence understanding the effects of migraine on QoL and severity of disability it causes as well as estimating the prevalence of depression among these patients will enable us to better manage the disease. Methodology: In this descriptive study, 50 consecutive migraineurs were studied. Headache impact, migraine disability, migraine-specific quality of life and depression among the patients were studied using validated questionnaires like HIT6, MIDAS, MSQoL and HAM-D. Result: Migraine was seen in 36 females and 14 males with most being in the age group of 20–40 years. Headaches had considerable to severe impact on the sufferers. The mean HIT score in the study was 65, indicating headaches had substantial to severe impact on them. The mean MIDAS score was 22 and most were found to have a grade III to grade IV disability indicating significant migraine-related disability. Mean MSQoL score was 68. The MSQoL in all the domains was affected significantly. The prevalence of depression among patients with migraine was 52%, with 6% of patients having severe depression and associated with Grade IV disability. Conclusion: Migraine was more frequently seen in young- and middle-aged females. Headaches had considerable to severe impact on the sufferers, causing significant disability and detrimental effects on QOL. The prevalence of depression among them was 52%.

Award ID: 645

To Assess the Levels of Cytokines and Chemokines in the Patients with Idiopathic Intracranial Hypertension

Aastha Takkar, Karthik V. Mahesh, Chirag Ahuja, Basavaraj Tigari, Ramandeep Singh, Sucharita Ray, Biman Saikia, Manoj Goyal, Vivek Lal

Department of Neurology, Postgraduate Institute of Medical Education and Research (PGIMER)

E-mail: draastha49@yahoo.com

Background and Aim: Idiopathic Intracranial Hypertension (IIH) is a disorder characterized by elevated intracranial pressure (ICP) without an identifiable etiology. Headache, double vision and subsequently visual loss are the common symptoms. The primary aim of this study was to measure the levels of pro and anti- inflammatory cytokines in serum and CSF of patients with IIH. Methodology: This is a prospective ongoing case control study wherein an interim data of 54 Indian patients of IIH has been studied as yet. The patients were recruited randomly from neurology department and outpatient department in PGIMER. During Lumbar puncture, opening pressure was noted and 3 ml of CSF & serum was collected for assessment of interleukins using BD™ Cytometric Bead Array (CBA). Result: Mean ± SD values of IL 6 (in pg/ml) in CSF and serum was found to be 12.5 ± 49.3 and 61.3 ± 271.1 respectively, which was significantly higher when compared to the control CSF and serum IL6 levels 9.8 ± 6.8 and 4.29 ± 3.46 respectively. Significantly high levels of proinflammatory cytokines in CSF of these patients may suggest the role of inflammatory cascade in IIH. Most of our patients have not completed their final follow up (Ongoing study – Interim Data). We wish to assess the visual prognosis and outcome of these patients and compare it with their baseline inflammatory markers. Conclusion: Our interim analysis suggests that inflammation may be a prominent pathophysiological determinant in patients of IIH. Our study also explains so as to why a subgroup of IIH patients (with worse visual parameters at presentation) responds well to steroid (anti-inflammatory) treatment. Our study may disclose the determinants of worse prognosis in patients developing IIH (specifically patients of fulminant IIH).

Award ID: 646

Delayed Neuropsychiatric Syndrome - A Rare But Reversible Manifestation of Carbon Monoxide Toxicity

Adreesh Mukherjee, Dwaipayan Bhattacharyya, S. Sandip Kumar, Debaleena Mukherjee, Sougata Bhattacharya

Neurology, Bangur Institute of Neurosciences, Institute of PostGraduate Medical Education and Research, Kolkata, West Bengal, India

E-mail: adreesh03@yahoo.co.in

Background and Aim: Exposure to burning fuels in closed room can result in Carbon monoxide (CO) poisoning. There is varied clinical manifestation of CO poisoning. Delayed neuropsychiatric syndrome following acute CO poisoning is a rare but reversible manifestation. Methodology: We present a case of cognitive and behavioral dysfunction manifesting about 1 month after acute CO poisoning which improved with appropriate management. Result: A 34-year-old gentleman had history of acute exposure to CO from burning coal indoors resulting in altered sensorium. He was resuscitated at a local hospital with rapid improvement and he resumed his duties after 1 week. However, about 1 month later, he developed rapidly progressive cognitive impairment and behavioral symptoms. There was apathy with impaired attention, executive dysfunction and disinhibition. He also had memory dysfunction and visuospatial deficits. Motor examination revealed bradykinesia and mild rigidity (axial and appendicular). MRI Brain showed bilateral globus pallidus and diffuse white matter T2/FLAIR hyperintensities. He was managed conservatively with high flow oxygen following which there was remarkable recovery over the next few weeks. Conclusion: Delayed neuropsychiatric syndrome is a rare complication of CO toxicity. It is usually associated with a period of pseudo recovery ranging from 2 to 40 days followed by rapid cognitive and behavioral deterioration. It is potentially reversible with conservative management, especially oxygen therapy.

Award ID: 647

Correlative study of VEP, OCT AP and Funduscopy in Optic Neuritis

Jasmine P, S. Saravanan, S. Saravanan

Neurology, Tirunelveli Medical College Hospital, Tamil Nadu, India

E-mail: jasminekalyani31@gmail

Background and Aim: The correlation of OCT, AP, VEP and funduscopy in optic neuritis with clinical disability are that these tests are potential markers of the disease burden in optic neuritis and NMO spectrum disorder. Methodology: Diagnosis of optic neuritis based on visual loss, RAPD when unilateral, serologic studies for AQP4-IgG, MOG-IgG, CSF studies for MS panel and test to r/o other possible causes -VDRL, anti HIV, RA factor, anti Ro/La, thyroid function test were done ophthalmologic evaluation including visual acuity, Funduscopic examination, VEP, Automated perimetry for assessment of visual field and OCT. Result: There were significant but opposite changes in RNFL thickness and mfVEP amplitude. The average asymmetry of RNFL thickness between affected and fellow eyes increased from 16.5 ± 11.5 to20.1 ± 12.8 µm (P = 0.0003), indicating progressive axonal loss, whereas mfVEP amplitude asymmetry decreased from 44.2 ± 30.8 to 38.3 ± 31.1 nV (P = 0.0015), indicating ongoing recovery of the optic nerve. Optic disc edema was found in 1/3 of cases. Automated perimetry was done. RNFL thickness was correlated with visual field defects and visual acuity.11 patients with normal visual fields in AP had reduced RNFL thickness. (p < 5%) Conclusion: Remyelination Helpss lesional repair, protection of axons, and restoration of conduction velocity. Recent studies have suggested that myelin plays an important role in providing trophic support to axons and protects them from inflammatory mediators and immune effector cells. Therefore, it is concluded that remyelination has effect on axonal conductivity and is responsible for the increase in cortical amplitude. Hence VEP and its topographic relationship to OCT in which VEP proves to be superior. OCT can detect optic neuritis even when visual field is normal in AP

Award ID: 648

A Foxing Case of Dyscognitive Seizures, Ultimately Found to be due to Ring Chromosome 20

Neetu Choudhary, Parampreet Singh kharbanda, Jitupam Baishya, Kamalesh Chakravarty

Neurology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

E-mail: Neetumeel@gmail.com

Background and Aim: Background- Dyscognitive seizures cause impairments in perception, attention, emotion, memory or executive function. The more common causes of such seizures, like hippocampal sclerosis, are easily picked up. Some other uncommon etiologies, however, may be a challenge to diagnose. We present details of one such case. Aim-To discuss detail clinical examination, investigation findings of a case of Dyscognitive seizure due to ring 20 chromosome. Methodology: A 16/F presented with episodes of sudden onset unresponsiveness lasting for 2 minutes. Duration of such episodes gradually increased to up to 45 -60 minutes during which she would be partially responsive to external stimuli, and had difficulty in performing cognitive tasks. She was evaluated with necessary investigations including video-EEG and karyotyping. Result: VEEG showed daily multiple episodes of habitual events with ictal onset at bilateral anterior temporal region (left predominant), as rhythmic slowing in theta-delta range lasting for 30-45 minutes. Her blood investigations including autoimmune panel were noncontributory. Her fasting CSF sugar was also normal. Her initial karyotype with analysis of 20 metaphase count was normal. However, in view of high suspicion of Ring 20 chromosome disorder, karyotyping was repeated with 200 metaphase counts which showed presence of ring 20 chromosome in 2 metaphases (1%). Patient has been tried with multiple antiseizure medicines, without significant improvement in seizure control. Conclusion: Ring chromosome 20 is an important cause of Dyscognitive seizures, which may be missed in initial work-up. High suspicion of ring 20 chromosome disorders should be considered in appropriate clinical setting and karyotyping should be repeated if necessary with more metaphase count in such cases.

Award ID: 649

Inhibition of Neuronal Activity with Perampanel Blocks α-Synuclein Transmission in Parkinson’s Disease Models

Jun Ueda

Department of Neurology, Kyoto University Graduate School of Medicine, Japan

E-mail: junueda1987@gmail.com

Background and Aim: The pathological feature of Parkinson’s disease is progressive neuronal degeneration and the presence of Lewy bodies composed of misfolded α-synuclein. The intercellular transmission of pathogenic proteins plays a crucial role in Parkinson’s disease. Previous studies have demonstrated the correlation between neuronal activity and the neuronal uptake of pathogenic proteins in experimental models of neurodegenerative diseases. Therefore, we hypothesized that the inhibition of neuronal activity could modulate the dynamics of α-synuclein, inhibit the propagation of α-synuclein pathology, and attenuate the progression of Parkinson’s disease. Perampanel, an antiepileptic drug, is an AMPA receptor antagonist that inhibits neuronal activity. The objective of this study is to verify the efficacy of perampanel on α-synuclein transmission in Parkinson’s disease models. Methodology: Mouse primary hippocampal neurons were transduced with α-synuclein preformed fibrils to examine the effect of perampanel, NBQX, and tetrodotoxin on the neuronal uptake of α-synuclein preformed fibrils. An in vivomodel of Parkinson’s disease was used to examine the effect of oral administration of perampanel on the neuronal uptake of α-synuclein preformed fibrils and the development of α-synuclein pathology. Result: Perampanel, NBQX, and tetrodotoxin blocked the neuronal uptake of α-synuclein preformed fibrils by inhibiting macropinocytosis. Furthermore, we showed that oral administration of perampanel inhibited the developmentof α-synuclein pathology in in vivo Parkinson’s disease model. Conclusion: Neuronal uptake of α-synuclein preformed fibrils could be mediated by an activity-dependent mechanism, and targeting neuronal activity with perampanel could represent a new therapeutic strategy for Parkinson’s disease.

Award ID: 650

Stroke Unit Treatment and Risk Factor Control Reduce Recurrent Stroke Risk

Shani SD, Ravi Prasad Varma, Sankara Sarma P, Sreelakshmi RS, Harikrishnan R, Raman Kutty V, Sylaja PN, Gurpreet Singh, Sapna Erat Sreedharan, Deepa Damayanthi, Srinivas Gopala, Madhusoodanan UK, P. N. Sylaja, Ravi Prasad Varma, Sankara Sarma P, Sreelakshmi RS, Harikrishnan R, Raman Kutty V, Sylaja PN

Neurology Stroke, SCTIMST, Kerala, India

E-mail: shanisd82@gmail.com

Background and Aim: The risk factor control is an important predictor of risk of stroke recurrence. The attributable fraction which estimates the excess risk among the exposed stroke survivors has not been studied previously. We studied the attributable fraction for stroke recurrence in consecutive incident cases of recurrent stroke. Methodology: A case control study with incident cases of recurrent stroke and controls matched for age and post stroke period was done. Structured interview was done to collect data on sociodemographic variables, lifestyle and medication adherence. The risk factors, treatment of index stroke and outcome were collected. Logistic regression analysis was done to find out the factors associated with stroke recurrence. Attributable fraction and average attributable fraction were calculated. Result: Among the 103 matched pairs, more than 70% were rural residents. Male gender (OR 2.59; 95% CI 1.05-6.42), presence of depression (OR 8.67 95% CI 2.80- 26.84), memory problem (OR 10.12 95% CI 2.48- 41.34), uncontrolled diabetes (OR 3.19 95% CI 1.42- 7.19), cardioembolic stroke (OR 4.45 95% CI 1.12- 17.62) and index stroke not being treated in stroke unit (OR 6.60 95% CI 2.86- 15.23 were associated with increased risk of stroke recurrence. The maximum average attributable fraction for stroke recurrence risk was attributed to index stroke not being treated in stroke unit and uncontrolled diabetes. Conclusion: The index stroke treated in a comprehensive stroke care unit and control of risk factors can reduce recurrent stroke risk among stroke survivors. This population attributable risk is important in planning secondary stroke prevention strategies.

Award ID: 651

The Utility of FDG-PET in the Differential Diagnosis of Parkinsonism

Arun Mani, Kancha Naresh, Appaswamy Thirumal Prabhakar, Sanjith Aaron, Anup Joseph Devasia, Aditya Nair, Ajith Sivadasan, Sanjith Aaron, Mathew Alexander, Vishnu A R, Julie Hephzibah, Sanjith Aaron

Neurology Unit, Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India

E-mail: arunmm18@gmail.com

Background and Aim: Differential diagnosis of parkinsonism can be difficult on clinical grounds, especially in the early stage. Recent advancements in 18-F-fluorodeoxyglucose positron emission tomography (FDG-PET) imaging reveals different patterns of regional glucose metabolism in various parkinsonian disorders, which may help to differentiate between these conditions. Methodology: We did a retrospective chart review of patients ≥ 18 years who had been diagnosed with parkinsonism and subsequently underwent an FDG-PET between 2015 and 2019. Z-score maps of FDG-PET were made using 3-dimensional stereotactic surface projections with comparison to age group–matched controls. Brain region mean Z-scores with magnitudes ≥ 2.00 were interpreted as significant. Result: A total of 34 patients were identified, with 25 males and 9 females. On clinical categorization according to the published clinical diagnostic criteria, 17 (50%) patients were classified as PSP, 9 (26.5%) as CBS, 4 (11.8%) as MSA, and 4 (11.8%) as IPD. FDG-PET was abnormal in 25 (73.5%) patients. On categorization of FDG-PET according to published criteria based on the characteristic patterns of glucose metabolism, 3 (8.8%) patients were classified as PSP, 5 (14.7%) as CBS, and 3 (8.8%) as MSA. 19 (55.9%) patients had an inconclusive FDG-PET. Using the clinical criteria as the gold standard, the sensitivity of FDG-PET was 17.6% for PSP, 55.6% for CBS, and 75% for MSA; while the specificity was 100% for each. Conclusion: FDG-PET may help to differentiate between the various parkinsonian disorders. In the current study, the diagnostic accuracy of FDG-PET is the highest for MSA, followed by CBS, and is the least for PSP.

Award ID: 652

“A Case of Early Infantile Epileptic Encephalopathy”

Amarendhar Malreddy

Neurology, Guntur Medical College, Andhra Pradesh, India

E-mail: amarendhar48@gmail.com

Background and Aim: Epileptic syndromes at early infantile onset are electroclinical syndrome in which seizures unexpectedly arose between the fourth and sixth days of life. The EEG picture often shows a characteristic burst-suppression pattern. KCNQ2 related encephalopathy where Partial seizures unexpectedly begin by the third day of life in neurologically normal-appearing patients. Early infantile epileptic encephalopathy (EIEE), also known as Ohtahara syndrome, is characterized by intractable spasms often consist of tonic forward flexion that lasts 1 to 10 seconds often occurring in clusters 10 to 300 times per day in the setting of a severe encephalopathy and a burst-suppression background pattern on EEG. Methodology: We reporting a case of 14 months old male presented with history of seizures from second day of birth with epilepic encephalopathy Result: 14 months male born of non consanguineous parentage presented with seizures from second day of birth with sudden tonic postures lating for 1 min. He was on multiple anti-epileptic drugs, inspite of which child had seizure recurrence of 8-10/day. On examination child is conscious and irritable but doesn’t make eye contact, no neurocutaneous markers, no focal motor deficits. EEG showed burst-suppression pattern with normal imaging. Genetic analysis confirmed KCNQ2 mutation. Conclusion: Ohtahara syndrome is a rare clinico-EEG syndrome. The spasms often consist of tonic forward flexion that lasts 1 to 10 seconds often occurring in clusters. The EEG picture often shows a characteristic burst-suppression pattern. Gold standard– genetic analysis. Refractory to any mode of treatment including ketogenic diet and surgery. Highest mortality rate.

Award ID: 653

A Rare Cause of Quadriplegia- Bilateral Medial Medullary Infarction with Heart Sign on MRI.

Thahseen S, Saravanan S, Saravanan S

Neurology, Tirunelveli Medical College, Tamil Nadu, India

E-mail: milmikach@gmail.com

Background and Aim: Medial medullary infarction (MMI) accounts for <1% of all brain infarctions. Bilateral MMI (BMMI) is even rarer. Common clinical presentations are motor weakness, dysarthria, hypoglossal palsy. Patient may also present with nystagmus, respiratory failure, dysphagia, ataxia, Horner syndrome. Stroke mechanism is vertebral artery atherosclerosis, perforator occlusion or arterial dissection. Clinical outcome is poor with high mortality and dependency. Methodology: A 57 year old diabetic and hypertensive male presented with acute onset right hemiplegia and dysarthria of 6 hours duration. Two hours later, he developed paucity of movements of left upper and lower limbs. He was conscious and extra ocular movements were full. No facial or tongue weakness was noticed. Subsequently he developed dysphonia. Palatal contractions were diminished bilaterally. He had flaccid tetraplegia with brisk DTRs and bilateral extensor plantar; sensory findings were inconsistent. Initial NCCT was normal. Patient was started on anti-platelets, heparin and supportive management. MRI Brain showed T2 FLAIR hyper-intensity with diffusion restriction in anterior medulla in heart shape. Result: Patient was treated with anti platelets, statins, anti hypertensives insulin and discharged with Ryles tube, bladder catheter and rehabilitative therapy. Power continued to be 0 in upper limbs and 1 in lower limbs and he is under follow up. Conclusion: Acute BMMI may present with flaccid progressive tetraplegia mimicking neuromuscular disorders. Typical heart sign on ventral medulla is unique to BMMI. Early diagnosis based on clinical and radiological findings can facilitate thrombolysis to decrease morbidity following this rare stroke subtype.

Award ID: 654

Predictors of Drug Induced Hepatitis in Patients of CNS Tuberculosis

Ajay Emani, Abdul Qavi, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha

Neurology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

E-mail: murthyad1@yahoo.co.in

Background and Aim: Most of the studies on anti-tubercular drug induced hepatitis are on patients with pulmonary tuberculosis. A high frequency of drug induced hepatitis has been observed in patients with CNS tuberculosis. Frequency and predictors of drug induced hepatitis in CNS tuberculosis has been evaluated in this study. The study was aimed to evaluate the frequency and risk factors, primarily, associated with drug induced hepatitis in patients of CNS tuberculosis. Methodology: This prospective study was carried out in a tertiary care teaching hospital. The diagnosis of DIH was based on five times elevation of alanine amino transferase (ALT) in asymptomatic and three times in symptomatic patients, serum bilirubin 1.5 mg/dL or more occurring after 3 days of antitubercular drugs without any other cause of liver dysfunction. Liver function tests were repeated at least once in 2 weeks. Along with that inflammatory markers and other blood workup was sent. Multivariate logistic regression analyses were used. Result: A total of 32 patients of CNS tuberculosis were evaluated. 10 (31.25%) patients developed DIH. After discontinuation of hepatotoxic ATT, the liver function normalized within a median of 7 (3-24) days. DIH was associated with seizures, low serum albumin and tuberculomas on neuroimaging. Conclusion: DIH occurs in 31.25% patients with CNS TB and is related to hypoalbuminemia and seizure. Attention should be paid to manage under-nutrition and avoid enzyme inducing antiepileptic drugs.

Award ID: 655

Clinical Profile, Neuroimaging Features and Outcome Study in Histologically Proven Focal Cortical Dysplasia: A Retrospective Analysis

Abhishek Gohel, Rutul Shah, Siby Gopinath, Ashok Pillai

Neurology, AIMS

E-mail: abgohel@gmail.com

Background and Aim: Focal cortical dysplasia (FCDs) constitute constitute a significant proportion of drug resistant epilepsies (DRE). This study describes the clinical profile, histopathological findings, MRI features and surgical outcome in DRE with FCD. Methodology: This retrospective study included 124 patients with drug-resistant epilepsy and histologically proven FCD. These patients were from a single centre who underwent resective surgery after EEG and MRI correlation. The patients were followed up for at least 12 months postoperatively, and the seizure outcome was classified using Engel’s classification of postoperative outcome. Histopathological examination was done for all the patients and was compared to the surgical results of the patients. Result: Out of 98 patients, 75 (76.5%) reported Engel class 1 outcome. 14 (14.3%) had Engel class 2 outcome, 8 (8.1%) had Engel class 3, and 1 (1%) had Engel class 4 outcomes. FCD type 2A was reported in 34 patients and type 2B was reported in 24 patients. Type 3B was reported in 2. Conclusion: This study underscores the longterm post-surgical outcomes in DRE patients with FCD.

Award ID: 656

Clinical Utility of Volumetric MRI and FDG PET Asymmetry in Temporal Lobe Epilepsy

Shameer Aslam, Ramiah Rajeshkannan, Sandhya C J, Manjit Sarma, Siby Gopinath, Ashok Pillai

Neurology, Amrita Advanced Epilepsy Centre, Amrita Institute of Medical Sciences and Research Center

E-mail: aslamshameer165@gmail.com

Background and Aim: The volumetric MRI and interictal 18F fluorodeoxyglucose (FDG) PET are the most important imaging modalities in presurgical evaluation of epilepsy. Quantitative analysis may further increase their yield in evaluating temporal lobe epilepsy (TLE). In this study, we statistically analyzed hippocampal volumetry and FDG PET metabolic asymmetry to detect TLE. Methodology: This study included 36 epilepsy patients who underwent resective surgery following the depth electrode implantation in the hippocampus. All patients were seizure free for more than 12 months. Patients were divided into Group I (n = 18; temporal lobe resection) and Group II (n = 18; extratemporal lobe resection). Amygdala and hippocampus were manually segmented from volumetric T1 MRI. The segmented amygdala and hippocampus along with Brodmann area template were used as regions of interest to determine the mean regional metabolism in PET. ROC (receiver operating characteristic) analysis was used to find a clinically relevant cutoff asymmetry value in hippocampal volumetry and temporal metabolism Result: ROC curve analysis identified a hippocampal volume asymmetry of 4.21% with sensitivity of 88.89% and specificity of 100% (p < 0.001). PET asymmetry analysis revealed the statistically significant asymmetry of 5.77% in hippocampus and 8.36% in Brodmann area 38 with sensitivity of 72.7% and specificity of 77.8% (p < 0.001). The asymmetry higher than this cutoff would indicate the temporal lobe seizure onset. Conclusion: Using statistical asymmetry analysis, we derived the cutoff asymmetry values to evaluate the hippocampal volumetry and FDG PET. This technique strengthened the utility of MRI and PET in non-invasive detection and exclusion of TLE. The derived threshold parameters warrant further validation in prospective studies.

Award ID: 657

The Friendship of White Matter and Red Matter

B. Naveen Reddy

Neurology, Guntur Medical College, Andhra Pradesh, India

E-mail: drnaveenreddy22@gmail.com

Background and Aim: Methylenetetrahydrofolate reductase (MTHFR) deficiency has an autosomal-recessive inheritance pattern and is the most common inherited disorder of folate metabolism. Biochemically, there are elevated plasma homocysteine, homocystinuria and low methionine levels Methodology: 17 year gentleman born of consanguineous parentage with developmental delay presented with complaints of progressive weakness of both lower limbs. He also had history of headaches and was diagnosed to have cerebral venous thrombosis in the past. Neuroimaging showed features of leukodystrophy, blood investigation showed raised homocystine levels. Genetic analysyis showed MTHFR mutation. Result: 17 year gentleman born of consanguineous parentage with developmental delay presented with complaints of progressive weakness of both lower limbs. He also had history of headaches and was diagnosed to have cerebral venous thrombosis in the past. Examination showed subnormal intelligence, spastic paraparesis, exaggerated tendon reflexes, extensor plantar response. Neuroimaging showed features of leukodystrophy, blood investigation showed raised homocystine levels. Genetic analysyis showed MTHFR mutation. He was treated with oral anti coagulation, betaine and folate, pyridoxine supplementation. Conclusion: MTHFR deficiency is a potentially treatable disease, early diagnosis is crucial and betaine treatment should be started as early as possible. It is therefore imperative for clinicians to be aware of the clinical patterns of early- and late-onset forms of the disease. Hyperhomocystinemia is an important independent risk factor of unprovoked CSVT. One should consider this treatable condition if CSVT is associated with leukoenchephalopathy.

Award ID: 658

Differential Regulation of Excitatory Synaptic Transmission in the Hippocampus and Anterior Temporal Lobe by Src in Temporal Lobe Epilepsy

Nitin Yadav, Priya Rajput, Sneha Anand, Yogesh Aggarwal, Savita Singh, Jyotirmoy Banerjee, Sanjeev Lalwani, M. C. Sharma, Fouzia Siraj, Manjari Tripathi, P. Sarat Chandra, Aparna Banerjee Dixit

ACBR, University of Delhi, Delhi, India

E-mail: nitinyadav.hrc@gmail.com

Background and Aim: Src family kinases are a crucial point of convergence for various signalling pathways. NMDA receptors (NMDARs) play a significant role in excitatory synaptic transmission in the brain and are critical in the process of epileptogenesis which is regulated by Src kinase. Therefore, this study is designed to test the hypothesis that altered Src kinase functions may contribute to hyperexcitability in MTLE Methodology: For this study, surgically resected hippocampal and ATL tissue specimens were obtained from MTLE patients. mRNA & protein expression in both human and TLE rat models (acute & chronic) was studied by Western blotting on Hippocampus and ATL regions. Functional Src activity was measured by kinase assay. Immunohistochemistry and Histopathological studies were performed on paraffin-embedded tissue sections. Functional validation using EPSCs was done using Patch-clamp. Result: Src was found to be upregulated in the histopathological, immunohistochemical and protein level findings of the hippocampus. Kinase activity was higher in the TLE model as compared to the control. A significant increase in Src was observed in the chronic model of TLE as compared to the respective control with no significant changes in the acute TLE model. PP2 blocker resulted in alterations in EPSC in MTS patients. Conclusion: Our results are indicative of the role of Src in hyperexcitability via modulating the regulation of NMDA receptors in MTLE. These findings will greatly improve our understanding of the molecular mechanisms and synaptic plasticity involved in the pathogenesis of MTLE, and Src may represent new potential therapeutic drug targets.

Award ID: 659

Differential Expression of Histone deacetylase 6 (HDAC 6) in Pilocarpine Model of Temporal Lobe Epilepsy

sonali Kumar, Arpna Srivastava, Ozasvi R. Shanker, Jyotirmoy Banerjee, Manjari Tripathi, P. Sarat Chandra, Fouzia Siraj, Aparna Dixit

Dr. B.R Ambedkar Centre for Biomedical Research, University of Delhi, Delhi, India

E-mail: sonalikumar281@gmail.com

Background and Aim: Histone modifications have emerged as important mechanisms regulating gene transcription in the brain, and altered histone acetylation may also be associated with epilepsy and the epileptogenic process. Previous studies have shown that HDAC inhibitors can be effectively used as anti-epileptic drugs. The present study was designed to investigate the region-specific alterations in the level of HDAC6, a cytoplasmic isoform of histone deacetylases in the pilocarpine model of temporal lobe epilepsy (TLE). Methodology: The pilocarpine model of temporal lobe epilepsy was generated using male Sprague Dawley rats after IAEC approval and the seizures were quantified on a Racine scale. Histopathological analysis using hematoxylin and eosin staining was performed to observe the pathological changes occurring in the animal model upon seizure generation. The mRNA levels of HDAC6 were assessed using Quantitative Real-time PCR and protein levels were investigated using western blot in three different regions of the brain i.e. the neocortex, hippocampus, and anterior temporal lobe (ATL). Result: Hemotoxylin and eosin staining confirmed pathological changes associated with epilepsy in the lithium pilocarpine model of epilepsy. There was an upregulation in the mRNA and protein levels of HDAC6 in the hippocampus of the experimental model as compared to the control whereas a downregulation was observed in the neocortex. Conclusion: As a result of the present study, we have demonstrated region-specific alterations of HDAC6 in the pilocarpine model of TLE suggesting the existence of independent epileptogenic networks in the temporal lobe structures and a possible molecular mechanism mediated by HDAC6 that might be contributing to epileptogenesis.

Award ID: 660

An Interesting Case of Intracranial Hypertension

Jenix Nathan Y. A., Shobana N, Sadeesh Kumar V, Selvakumar C. J., Joby Thomas Mammen

Department of Neurology, Coimbatore Medical College Hospital, Tamil Nadu, India

E-mail: jenixnathan@gmail.com

Background and Aim: INTRODUCTION:Idiopathic intracranial Hypertension which is a syndrome characterized by raised Intracranial pressure usually occurs in obese women in child bearing age group. Methodology: CASE PRESENTATION:35 years old female patient got admitted with complaints of persistent diffuse headache for one month associated with Nausea, vomiting, photophobia and phonophobia. History of diplopia present. There was no history of trauma, drug intake or use of oral contraceptives. On examination her BMI was 24. Her ocular examination revealed both eyes vision 6/6, Extra ocular movements both eye abduction restricted. Fundus examination showed established papilledema. MRI Brain features suggestive of Idiopathic intracranial Hypertension. Result: Investigation revealed Hb-12.7, TC-87.2 X10 3, Plat – 6.3 lakhs. RFT, LFT were within normal limits. Peripheral smear examination revealed marked leukocytosis. Differential count suggestive of evolving phase of CML in Chronic phase. USG abdomen showed mild Hepatosplenomegaly. CSF opening pressure was raised 40 cm of water, with normal protein, sugar and no cells. BCR ABL1 gene (Philadelphia chromosome) positive. She was treated with osmotic diuretics like mannitol, Acetazolamide and started with chemotherapy drugs like Imatinib and allopurinol. Conclusion: DISCUSSION: Idiopathic Intracranial Hypertension can be associated with number of conditions. Common associations include Thyroid disorders, Hypervitaminosis and systemic Hypertension.1 It is also associated with hematological disorder like CML, Polycythemia. However, association with CML is rare. There is very few case reports of CML presenting as IIH. Raised WBC count can lead to hyper viscosity of blood which in turn interfere with CSF absorption in subarachnoid granulations. 2, 3 This can lead to raised ICP and Papilledema in our patient. CONCLUSION: Hematological disorder like CML need to be kept in mind while evaluating a case of Idiopathic Intracranial Hypertension. After treatment with osmotic diuretics and treating underlying cause there is resolution of symptoms.

Award ID: 661

A Rare Case of Refractory Epilepsy- Double Cortex Syndrome

Jeyashree S P, Sownthariya R, Edwinraj Thomas A, Kannan B

Neurology, Thoothukudi Government Medical College and Hospital, Tamil Nadu, India

E-mail: jeyashreesankar16@gmail.com

Background and Aim: INTRODUCTION: Double cortex syndrome or Subcortical band heterotopia is a neuronal migration anomaly in which focal or diffuse collections of ectopic neurons in the cerebral hemispheres. Methodology: CASE REPORT: In this case report, we present a case of 17 years old girl presented with status epilepticus, who was on treatment with 4 antiepileptic. On admission, she was in post ictal state. The past history reveled that seizures began when she was of 5 years of age and had presented as multiple episodes of drop attacks, atypical absences and generalized tonic-clonic movements. She had mild intellectual impairment and had learning disabilities. Birth and family history were unremarkable. Electroencephalogram was done. Magnetic resonance Imaging of brain showed Thick symmetric band of grey matter and sub cortical white matter of bilateral cerebral hemispheres- Band heterotopia. Result: DISCUSSION: Band heterotopia is a rare cortical malformation secondary to early arrest of neuronal migration which may be familial with an incidence of 1 in 2, 00,000. Most common in women. Common cause is due to missense mutation in the double cortin (DCX) gene with X-linked dominant pattern of inheritance. Affected individuals present in their first decade with multiple seizure types refractory epilepsy and mental retardation. MRI is the mainstay in diagnosis showing double cortex appearance. Conclusion: We presented this case because of its rarity and one of the causes of refractory seizure, to be evaluated and treated at the earliest. Drug refractory patients can be referred at earliest for surgical therapy. Recognition of MRI pattern helps in prognostication, proper treatment and rehabilitation in patients.

Award ID: 662

Role of Autoimmunity in Atypical Parkinsonism- A Case Control Study

Sudheeran K, Annamma Mathai, Jyothi Vikramanpillai Leelamaniamma, Vivek Nambiar, Siby Gopinath, Anandkumar Anandakuttan, Suguvavan Kalingavarman, Ullas Mony, Manu Raj, Renjitha Bhaskaran

Neurology, Amrita Institute of Medical Sciences, Kochi, Kerala, India

E-mail: sudheeran_k@yahoo.co.in

Background and Aim: Presence of inflammation in atypical parkinsonism is well described but no data is available on autoimmunity. Objective of this study is to determine the role of autoimmunity in atypical parkinsonism Methodology: Consecutive patients with a diagnosis of atypical parkinsonism attending the neurological services of Amrita Institute of Medical Sciences, Kochi, India were included in the study as cases and age and sex matched healthy controls were recruited (December 2018 to May 2019). Fifteen cell/cytokine parameters [T regulatory cells, human retinoid orphan receptor γt (RORγt), Interleukin (IL) -2, IL4, IL-6, IL10, tumor necrosis factor (TNF), interferon gamma, IL-17A, IL-21, IL-22, IL-23, Granulocyte Monocyte Colony stimulating Factor (GM-CSF), nuclear factor kappa B (NFkB) and transforming factor beta (TGFB)] were measured in blood (using flowcytometry, and ELISA) and compared between cases and controls. Result: There were 26 cases (mean age 67.77 + 7.45 years,16 male) and 15 healthy controls (68.07 + 3.45 years,10 males). [ progressive supranuclear palsy-15, multiple system atrophy-1, fronto temporal dementia with parkinsonism-2, and 8 as atypical parkinsonism]. RORγt mean fluorescence intensity (1383.73+501.62 vs 914.94+457.07, p = 0.041), IL-6 (14.28+31.36 pcgm/ml vs 0.92+ 1.24 pcgm/ml, p = 0.004), tumour necrosis factor (0.32 + 0.46 pcgm/ml vs 0.01 + 0.05 pcgm/ml, p = 0.020), IL 10 (0.60+1.12 pcgm/ml vs 0.08+ 0.19 pcgm/ml p = 0.027) and IL-4 (0.34 + 0.70 pcgm/ml vs 0, p = 0.048) levels were significantly elevated in atypical parkinsonism compared to controls. Conclusion: Our study shows elevated blood levels of RORγt, IL-6, TNF, IL10, 1L-4 in cases pointing towards a potential underlying autoimmune pathway in atypical parkinsonism.

Award ID: 663

Altered Expression of Protein Tyrosine Kinase 2 (PYK2) in the Pilocarpine Model of Temporal Lobe Epilepsy

Ozasvi Shanker, Sonali Kumar, Jyotirmoy Banerjee, Manjari Tripathi, P. Sarat Chandra, Fouzia Siraj, Aparna Dixit

Dr. B.R Ambedkar Centre for Biomedical Research, University of Delhi, Delhi, India

E-mail: ozasvi.mhg.bhu16@gmail.com

Background and Aim: PYK2 is a non-receptor tyrosine kinase known to be highly expressed in forebrain neurons. Pyk2 is known to be activated following stimulation of glutamate receptors, neuronal depolarization, and increased intracellular free calcium. Previous evidence has reported a transient increase in the levels of PYK2 in neurons is associated with delayed microglial activation following seizures. The present study thus sought to investigate the region-specific alteration of PYK2 in the pilocarpine model of epilepsy to understand its contribution to the process of epileptogenesis. Methodology: The pilocarpine model of epilepsy was developed after IAEC approval using male Sprague Dawley rats. Seizure quantification was done using the Racine scale and histopathological changes were observed using H&E staining. The mRNA levels of PYK2 were assessed using quantitative Real-time PCR and the protein levels and activity of P-(Tyr402) PYK2 were analyzed using western blot and kinase assay respectively. All the experiments were carried out using three different brain regions - neocortex, anterior temporal lobe, and hippocampus resected from the animal model. Result: The H&E staining confirmed the presence of epilepsy-associated pathological changes in the brain of the animal model. The mRNA levels of PYK2, its activity as well as the protein level of its activated form were found to be upregulated in the hippocampus but not in the cortex or ATL. Conclusion: The upregulation of PYK2 in the hippocampus of the brain of the animal model suggests its potential contribution to the pathogenesis of the disease and the region-specific alteration provides evidence for the existence of independent epileptogenic networks.

Award ID: 664

A Case Report of Hoffmann’s Syndrome - Rare Facet of Hypothyroid Myopathy

Srujanitha Tadi, Cornelius Justin, Ramu S, Aditya Bellala

Neurology, Madurai Medical College, Madurai, Tamil Nadu, India

E-mail: anithatadi@gmail.com

Background and Aim: Hypothyroid myopathy is characterised by myalgias, muscle cramps, fatigue, and proximal muscle weakness aggravated on exertion, there are 4 subtypes of hypothyroid myopathy : myasthenic syndrome, atrophic form, Kocher-Debre-Semelaigne syndrome, Hoffman syndrome. Hoffmann syndrome is characterized by pseudohypertrophy, painful spasms, proximal muscle weakness, and stiffness. Here we report a case of hypothyroid male with Hoffmann’s syndrome. Methodology: A 20 yr male with chronic progressive proximal muscle weakness of both lower limbs, muscle cramps, stiffness and easy fatiguability, with history of hypothyroidism in mother. Examination revealed dry and coarse skin, facial and periorbital puffiness, large and thick tongue, mild pedal edema, Sinus bradycardia, bilateral symmetric proximal muscle weakness of both lower limbs, non tender calf muscle hypertrophy, grip and percussion myotonia, delayed relaxation of bilateral ankle jerks. Result: Investigations revealed TSH-922 µIU/ml; Anti TPO - positive, CPK level is 1250 IU/l, LDH is 250 U/l. ECG showed low-voltage complexes and sinus bradycardia, echocardiography showed mild pericardial effusion. MRI bilateral lower limbs showed severe hypertrophy with T2 hyperintensity involving gastrocnemius and soleus suggestive of myotonia, MRI Brain showed pituitary hyperplasia. Nerve conduction study was normal. Electromyography showed small-amplitude myopathic motor unit potential. Based on above findings a diagnosis of Hoffmann syndrome is made and patient is started on thyroxine and he showed partial symptomatic improvement. Conclusion: Hypothyroidism has been a common endocrine disease and clinicians should be aware of this atypical and rare presentation of hypothyroid disease spectrum. Hoffman’s syndrome represents those few forms of myopathy that completely reverse on prompt therapy and, hence, has a good outcome.

Award ID: 665

An Interesting Case of Central Pontine Myelinolysis in a Patient with Type 2 Diabetes Mellitus

Archana M, Saravanan Sankaralingam

Neurology, Tirunelveli Government Medical College Tamil Nadu, India

E-mail: archanamkiran@gmail.com

Background and Aim: Central pontine myelinolysis (CPM) is a non inflammatory demyelinating lesion of pons. Although there are pronounced fluctuations in serum osmolarity, CPM is rarely seen in diabetics. This is a case report of CPM associated with hyperglycemia Methodology: A 55 year old male patient with uncontrolled Type 2 diabetes mellitus, systemic hypertension and who is a chronic alcoholic presented with one month history of progressive weakness of all four limbs (lower limb > upper limb) and slurring of speech. His previous medical records revealed multiple admisssions for high blood sugar levels for which he received iv correction. Patient was noncompliant with treatment. On examination, patient had dysarthria, spastic quadriparesis with power grade 4- in lowerlimbs and grade 4 in upper limbs. Laboratory evaluation showed S.Sodium 136 meq/l, S.potassium 4.2 meq/l, RBS 676 mg/dl and serum osmolarity 319 mosmol/kg. MRI brain demonstrated bilateral symmetrical T2 FLAIR hyperintensities with diffusion restriction in pons and sparing of peripheral region and transverse pontine fibres. A diagnosis of Central pontine myelinolysis secondary to hyperglycemia was made. He was managed conservatively. Result: During follow up after 8 weeks, patient had near normal recovery. Conclusion: Case report highlights the importance of adequate blood glucose control in diabetics. Physicians should be aware of complication like CPM, which can present atypically in diabetics and is only diagnosed in presence of a high index of clinical suspicion.

Award ID: 666

Autoimmune Encephalitis - High Degree of Suspicion Counts the Most

Prasanthi K, Shobhana N, Selvakumar CJ, Sadeesh Kumar V, Joby Thomas Mammen

DM Neurology, PG Resident, Coimbatore Medical College and Hospital, Tamil Nadu, India

E-mail: katragaddaprasanthi@yahoo.com

Background and Aim: Autoimmune encephalitis (AE) constitutes a group of neuropsychiatric manifestations in which antibodies produce neuronal dysfunction by targeting the neuronal surface or synaptic antigens. The presentation varies depending on the target antigen. Knowledge regarding different types of autoimmune encephalitis and high degree of suspicion based on clinical presentation are necessary for early diagnosis and management. To highlight the importance of knowing clinical manifestations of NMDAR/ non NMDAR encephalitis for its early diagnosis and treatment. Methodology: AE was diagnosed based on clinical manifestations and confirmed by the presence of positive antibodies in csf and serum. Result: 3 patients presented with generalised tonic clinic seizures, abdominal pain, aggressive behaviour, apathy, paucity of speech, altered sleep wake pattern, myoclonic jerks, ataxia. EEG showed triplets, doublets. CSF came as positive for NMDA antibody, AMPA antibody, VGKC antibody. Treatment given were IV steroids, IV Immunoglobulin, IV Rituximab. Conclusion: A high degree of suspicion is required for early diagnosis of autoimmune encephalitis. Mri brain, eeg and csf analysis may be normal initially, it is the clinical evolution of symptoms and signs that counts the most. Lack of proper awareness of these diseases can result in patient diagnosed as nonorganic psychosis or malingering.

Award ID: 667

An Unsual Case of Post Viral Paraparesis

Rindha Venepally, Reshma Sultana Shaik, Sandhya Manorenj, Shalini Akunuri

Neurology, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: rindha7.rao@gmail.com

Background and Aim: Neurological manifestations following varicella infection is a well-known entity. However syrinx as a post viral complication of varicella is a rare phenomenon and little is known in the literature. Here we describe a case report of a child who presented with post varicella paraparesis secondary to syrinx formation. Methodology: Descriptive study of a single case report from a tertiary care centre in south india. 5 year old female child presented with difficulty in walking 5 days prior to the presentation predominantly as buckling. Two weeks earlier child developed varicella exanthem. Examination showed asymmetrical weakness of bilateral lower limbs and grade 1 spasticity with brisk reflexes and bowel and bladder disturbances. Right plantar was extensor and left equivocal. A diagnosis of post viral patchy myelitis was considered and evaluated Result: Routine labs: normal; 2D echo: normal; MRI Dorso lumbar spine with screening of whole spine and brain with contrast was done which revealed a syrinx formation at D5-7 level. CSF analysis : varicella virus was detected The child was started on intravenous acyclovir and solumedrol and had a prompt recovery Conclusion: Post varicella syrinx formation is a rare entity. It is usually reported as a sequelae of post viral transvere myelitis. The mechanism of syrinx as reported in the literature could be due to swelling of the spinal cord causing narrowing of sub arachnoid space or breakdown of blood spinal cord barrier and venous congestion due to the inflammatory process.

Award ID: 668

MSPro Discuss: Constructive Approach to Monitor Progression in MS

Rahul Tryambak Chakor

Neurology, T N Medical College B Y L Nair Hospital, Mumbai, Maharashtra, India

E-mail: rahulchakor@gmail.com

Background and Aim: MSProDiscuss is a novel algorithm-based online tool that assists neurologists in identifying early indications and symptoms of MS progression. The tool utilizes the information from patients’ EDSS, relapses, MRI lesions, symptoms, and associated impact over the last six months to generate a report that supplements discussion regarding disease progression. The purpose of this study was to determine the tool’s clinical utility in a busy Govt Neurology practice for monitoring early progression. Methodology: We assigned two dedicated members of staff from the institution and trained them to utilize the tool. Developed a protocol and data collection sheet for a pilot study with 50 MS patients to evaluate disease progression during follow-up visits. Result: The average time to complete MSProDiscuss was 4-5 minutes per case. As of April 30, 2022, the tool had been used in 25 MS patients, with the majority of our patients showing yellow signal (may progress), few showing green signal (unlikely to progress), and red signal (high likelihood of progression). We continued the same treatment for patients with green signal, however for patients with yellow & red signals we considered treatment switch and provided options to patients and recommended close follow-up. Conclusion: MS progresses even in the absence of relapses and MRI activity. Detecting subclinical indicators of disease activity is critical for preventing progression to SPMS. This tool assists in identifying some of these early symptoms of disease progression and allows for more in-depth discussions with patients, especially those who did not have frequent relapses or MRI activity. This tool has shown to be beneficial and adaptive in our Govt hospital.

Award ID: 669

Trigeminal Nerve Block for Treatment of Refractory Trigeminal Neuralgia: A Case Series

Kuljeet Anand, Vivek Loomba, Alfarid Shahid Ali

Neurology, Dr. RML Hospital, New Delhi, India

E-mail: kuljeet_anand@rediffmail.com

Background and Aim: BACKGROUND Trigeminal neuralgia is a neuropathic condition characterised by recurrent brief episodes of sudden stabbing (lancinating) facial pain involving the trigeminal nerve. The condition is mostly unilateral and can involve one or more branches of the trigeminal nerve. Few patient are non responsive to conventional medications. AIM- To report the effect of trigeminal nerve block on refractory trigeminal neuralgia (rTN) Methodology: Twelve patients (male:female = 10:2, Age range-52-74 years) of rTN who were refractory to multiple (2 or more) conventional medications were included in the study. All patients underwent trigeminal nerve block. A 22 G spinal needle was inserted through the mandibular notch under fluoroscopic guidance and the affected nerve (V1, V2 and/or V3) was blocked with 0.25% bupivacaine and triamcinolone. The pain symptoms, duration, frequency of attacks per day and pain severity (VAS) were recorded before and after the procedure. Result: All patients reported significant relief of symptoms. One patient had oropharyngeal carcinoma and died within 6 months of procedure. Another patient had a recurrence of symptoms after 7 months when he was treated with radiofrequency ablation of trigeminal nerve. Rest of the patients were pain free for over one year on follow up. There were no complications. Conclusion: Trigeminal nerve block is safe and effective in treatment of rTN.

Award ID: 670

Management of Refractory Chronic Migraine with Sphenopalatine Block: Case Series

Kuljeet Anand, Vivek Loomba, Alfarid Shahid Ali

Neurology, Dr. RML Hospital, New Delhi, India

E-mail: kuljeet_anand@rediffmail.com

Background and Aim: BACKGROUND Migraine is a common, often disabling neurological disorder. The pathophysiology of migraine is complex, involving activation of the trigeminovascular system that leads to inflammatory changes in the pain-sensitive meninges. Given the complex neurobiology of this disorder, treatment of migraine is often difficult and refractory. In migraine, activation of parasympathetic synapses within the Sphenopalatine Ganglion (SPG) releases vasoactive peptides that contribute to neurogenic inflammation, vasodilation, and the symptoms of migraine and cranial autonomic symptoms may be mediated by this parasympathetic outflow of the SPG. SPG block may result in parasympathetic and sensory outflow attenuation, thereby treating migraine and its associated autonomic symptoms. This is a case series of six patients of refractory migraine who underwent SPG block resulting in long term relief of symptoms AIM To report the efficacy of Sphenopalatine Ganglion Block in refractory chronic migraine. Methodology: Six patients of Refractory Chronic Migraine (rCM) (Male:female = 0:6; Age-22-35 years) were included in the series. All patients underwent SPG block under fluoroscopic guidance with a 22 gauge quincke needle via the mandibular notch approach using 0.25% bupivacaine and 40 mg triamcinolone. Patients were assessed before and after intervention for frequency and severity of headache. Result: Four out of six patients reported significant decrease in frequency and severity of migraine attacks (>50% from baseline). Two patients had reported total cessation of migraine symptoms for over 2 years after the block. Conclusion: The present series shows that SPG Block is safe and effective intervention in rCM

Award ID: 671

MRI Finding in Hyper Ig E Syndrome (Job’s Syndrome)

Leena Rajani, Sownthariya R, Edwinraj Thomas A, Kannan B

Neurology, Thoothukudi Government Medical College And Hospital, Tamil Nadu, India

E-mail: leena3011rajani@gmail.com

Background and Aim: Introduction- The Hyper IgE syndrome comprise a group of primary immunodeficiency disorders that exhibit markedly elevated IgE levels, recurrent staphylococcal skin abscesses, eczema and pulmonary infections. It has a incidence of 1/1,000,000. In addition to immunologic abnormalities there are connective tissue and morphologic abnormalities in many patients including characteristic facies, scoliosis, joint hyperextensibility, retained primary dentition, craniosynostosis, osteopenia and pathologic fractures. The great majority of abnormalities that we found in autosomal dominant or sporadic Hyper IgE range from focal Hyperintensities and Chiari I malformations to lacunar infarcts and CNS infections. Both Autosomal dominant and Autosomal recessive forms of the disorder have been described. Most autosomal Dominant Hyper IgE syndrome have been found due to mutation in STAT3 whereas DOCK 8 mutation have been identified in Autosomal recessive Hyper IgE syndrome. Methodology and Result: Here I report a case of 12 year old boy born out of second degree consanguineous marriage with the eventful history of delayed cry after birth. Patient was diagnosed as a case of Hyper IgE syndrome at the age of 10 years on the basis of recurrent history of pulmonary and skin infections along with clinical findings. Patient presented in our OPD with the chief complaint of headache in which MRI Brain showed T2 Hyperintensity in periventricular white matter predominantly peri-trigonal regions. Hyperintensities on MRI Brain is the common manifestation of this uncommon disease. Conclusion: This rare syndrome has got very pertinent CNS abnormalities peculiar in Hyperimmunoglobulin E syndrome. Focal T2 Hyperintensities not appreciated previously represent a prominent feature of this rare disease that may assist in clinical diagnosis.

Award ID: 672

Clinical and Genetic Spectrum of Congenital Myasthenic Syndromes

Saraswati Nashi, Nalini Atchayaram, Seena Vengalil, Kiran Polavarapu, Akshata Huddar, Gopi U, Abel Thomas Oommen, Dipti Baskar, Preethish Kumar Veeramani, Gautham Arunachal Udupi

Neurology, NIMHANS

E-mail: nandanashi@gmail.com

Background and Aim: Congenital myasthenic syndromes (CMS) are heterogeneous group of rare, inherited neuromuscular junction (NMJ) disorders. We aimed to describe the clinico-genetic spectrum of a cohort of 154 suspected CMS patients from 140 families to understand the prevalence of different CMS subtypes in India and develop an efficient diagnostic pipeline. Methodology: Ambispective Result: Age of onset ranged from as early as at birth to as late as fourth decade with a mean age of 6.6 ± 9.9. M:F-85:69. The mean age at presentation was 19.1 ± 12.9 (1-56 yrs). All patients presented with fatigable weakness of either ocular, cranial or limb muscles. Limb symptoms (predominantly lower limb fatigable weakness) were most reported in 140 patients (90.9%) followed by ocular symptoms (ptosis / ophthalmoparesis) in 118 (76.6%). Bulbar involvement was reported in 72 (46.7%) and 23 patients (14.9%) had respiratory symptoms. Mutational SpectrumOverall, 87 unique CMS gene variants have been reported in this cohort out of which 46 are novel variants identified as disease causing for the first time in this study. The most common group of CMS genes affected was post-synaptic (87/127- 68.5%) followed by glycosylation defects (30/127- 23.6%). Among the individual CMS genes, most common association was CHRNE (51/127–40.2%) followed by DOK7 (19/127- 15%), DPAGT1 (13/127- 10.2%), GFPT1 (10/127- 7.9%), MUSK (8/127- 6.3%), GMPPB (7/127- 5.5%), COLQ (6/127- 4.7%), VAMP1 (3/127- 2.4%), CHRND (3/127- 2.4%), AGRN (2/127- 1.6%) and one each family (0.8%) of SLC25A1, CHRNA1, CHRNB1, RAPSN and MACF1. Conclusion: The incidence and prevalence of CMS in India is currently unknown. To date, there are only a few case reports and three small cohort studies. We describe a large cohort of CMS from the Indian Population and the common clinco-genetic variants prevalent.

Award ID: 673

Next Generation Sequencing Revealed Oligogenic Variants in a Young Onset ALS Patient from North India

Saileyee Roychowdhury, Deepika Joshi, Anand Kumar, Parimal Das

Centre for Genetic Disorders, Banaras Hindu University, Varanasi, Uttar Pradesh, India

E-mail: saileyee.rc@gmail.com

Background and Aim: Amyotrophic lateral sclerosis, a devastating neurodegenerative condition affects the motor neurons of the brain and spinal cord with an extremely difficult diagnosis due to the enormous degree of clinical variability and no known cure. The aim of the present study is identification of the genetic cause of the disease in a young-onset ALS patient from North India. Methodology: A young onset apparently sporadic ALS patient aged 25 admitted to Neurology ward, IMS, BHU with a rapid and progressive course of the disease without any cognitive decline was screened for mutations in major ALS candidate genes by Whole exome sequencing. Variants detected were reconfirmed by Sanger Sequencing. Result: Two heterozygous missense variants one each in the ANXA11 (p.R452W) and in the SIGMAR1 (p.R208W) respectively were identified. The minor allele frequency of p.R452W variant was <0.01 in large population databases. However, the p.R208W variation is a frequent single-nucleotide polymorphism seen in the Asian population, accounting for around 5% of all genetic variants. Both of these variants were predicted to be damaging by several major In silico prediction tools. Conclusion: We identified a potentially pathogenic rare variant that did previously not have reported in ALS patients along with a common but damaging SNP in an ALS patient. These findings add to our knowledge of the ALS genetic architecture of Indian ALS patients and serve as a foundation for future research into the pathogenic mechanisms/pathways of these variants that underlie the cause of sporadic ALS pathophysiology.

Award ID: 674

Serotonin Syndrome: A Spectrum of Toxicity with Prompt Response to Cyproheptadine

Neelam sahu, Pushpendra nath Renjen, Dinesh Mohan Choudhari

Neurology, Indraprastha Apollo Hospital, New Delhi, India

E-mail: drneelamsahu6@gmail.com

Background and Aim: Serotonin syndrome is an adverse drug reaction that results in mental status changes, and autonomic and neuromuscular dysfunction due to excessive activation of postsynaptic serotonin receptors. Methodology: A 60 years old gentleman with sudden onset tachycardia, generalized body aches, extreme fatigue, weakness, uncontrollable twitching, tremor, and hyperreflexia. Over the next 24 hours, the patient became drowsy and had to be intubated for mechanical ventilation. He had a history of Fournier’s gangrene four years back and developed neuropsychiatric symptoms in the last one year. The patient was being treated with multiple antipsychotics. On examination, patient was diaphoretic, hyperthermic, tachycardic, hypertensive, and exhibited bilateral mydriasis, tremors, extrapyramidal features and clonus. Result: Serotonin syndrome was diagnosed using the Hunters criteria. All antipsychotic medicines were stopped. The patient was managed with iv lorazepam along with Cyproheptadine with an initial dose of 12 mg through Ryle’s tube, followed by 2 mg every two hours until a clinical response was achieved and later on maintenance dose. The patient showed significant improvement in all the neurological symptoms. The patient was extubated after a few days and was discharged in a stable condition later. Conclusion: Serotonin syndrome is a clinical diagnosis; serotonin concentrations do not correlate with clinical findings, and no laboratory test confirms the diagnosis. The diagnosis of serotonin syndrome is made solely on clinical grounds using the Hunter Criteria; a detailed history and thorough physical and neurologic examination are essential.

Award ID: 675

A Prospective Case Control Study on the Role of Pro-Inflammatory Bio-Markers in Serum, in Predicting High Risk Subsets of Multiple Sclerosis (MS)

Sreehari Surendran, Ravi Anadure, Amit Sreen

Neurology, Army Hospital Research and Referral, New Delhi, India

E-mail: 06.sreehari@gmail.com

Background and Aim: Subtle diffuse intrathecal inflammation is undetectable by clinical disability scales & conventional neuroimaging and could influence MS disease course. Aim of this study was to explore the role pro-inflammatory CNS biomarkers in serum of patients with Multiple Sclerosis in detecting subclinical central inflammation making them amenable to early & appropriate therapeutic intervention. Methodology: 77 cases of MS and 77 age & sex matched controls were included in the study. Serum levels of IL-8, IL-1β & TNF-α for both cases and controls were obtained by ELISA. Result: The mean value of IL-8 (pg/ml) for cases was 312.62 ± 409.69 pg/ml and controls was 120.88 ± 92.5 pg/ml. Mean IL-8 levels were significantly elevated in cases as compared to healthy control with P value of 0.001. There was no statistically significant difference in the mean values of IL-1β and TNF-α between cases and controls. By using this 90th percentile cut off value in the IL-8 subgroup analysis, there was significant association (Odds Ratio of 2.93) when patients were having a greater number of MRI Spine lesions. Conclusion: Pro-inflammatory CNS bio-markers in serum - IL8, IL-1 β & TNF-α were studied in of Multiple Sclerosis patients with a case control design. IL-8 levels were significantly elevated in cases as compared to healthy controls. We can infer that IL-8 can be used as a surrogate marker of CNS inflammation specifically for disease activity in patients with MS with increased lesion load in spinal cord. It remains a non-specific marker of CNS inflammation and can be used as an additional investigation in MS cases.

Award ID: 676

Acute Flaccid Paralysis – A Rare Presentation of Arsenic Poisoning

Midhun Mohan, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Abdul Qavi

Neurology, Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

E-mail: midhunmohan07@gmail.com

Background and Aim: Acute poisoning from inorganic arsenic in humans affects all organ systems. Chronic, non-lethal levels of inorganic arsenic poisoning have different characteristics. Neurological symptoms include peripheral neuropathy mimicking Guillain-Barre syndrome, cognitive impairment, confusion and memory loss. These manifestations of chronic, non-lethal arsenic poisoning occur at levels of 0.001 to 0.1 mg/kg/day, if exposed to inorganic arsenic > 365 days. Methodology: Case Presentation- We report the case of a 31 year old female presented to neurology department with acute onset sensorimotor quadriparesis without bowel, bladder involvement with hyperkeratotic skin lesions over both palms and family history of similar presentation in the relatives. Nerve conduction study on admission demonstrated axonal sensorimotor polyneuropathy. CSF analysis showed normal cell count with mildly elevated protein. 24 urine arsenic levels were elevated and confirmed the diagnosis of arsenic neuropathy. Result: The patient was treated symptomatically with antioxidants and physiotherapy. Chelation was done with Penicillamine. There was no worsening of the patient’s symptoms. Conclusion: The possibility of arsenic neuropathy should be considered in patients with acute or subacute peripheral neuropathy of undetermined etiology

Award ID: 677

An Unusual Neurological Presentation of Maliganant Melanoma with Unknown Primary Posing a Dignostic Dilemma: A Case Report

Amreen, Muralidhar Reddy, Muralidhar Reddy, Muralidhar Y. Reddy, Vijaya Tourani, Tvrk Murthy, Sainath Bethanabotla, Subhendu Parida, Shyam K. Jaiswal, Lalitha P, Abhinay G. Kumar, JMK Murthy

Neurology, Care Hospitals

E-mail: amreen.33@gmail.com

Background and Aim: Leptomeningeal metastasis results from metastatic spread of cancer to leptomeninges. Breast and lung cancer, and melanoma are the most frequent causes. We report a patient with unassuming clinical presentation due malignant melanoma with no obvious primary disease. Methodology: we evaluated this patient with serum serology, CSF analysis, cytology, imaging. He was further evaluated with whole body PET CT, S1 root and arachnoid biopsy Result: A 37-year-old male patient presented with gradually progressive right lower limb radicular pains of 3-months duration, numbness of left thigh of one-month duration and progressive weakness of right lower limb followed by left lower limb of 15-days duration. Initial evaluation showed LETM and abnormal CSF (Glucose 34 mgs%, Proteins 286mgs%, 24 cells and 100% lymphocytes). CSF GeneXpert and TB-PCR, serum ANA, HIV and MOG antibodies were negative. Serum aquaporin antibodies were positive and ACE levels elevated. He was treated with steroids with no clinical improvement and presented to us. He was re-evaluated with MR imaging of brain and spine which now showed nodular enhancing lesion in the left occipital horn of lateral ventricle, nodular thickening of bilateral V, VII and VIII nerves, nodular enhancing lesions over the whole spinal cord and clumping of cauda equina. PET-CT showed hypermetabolic occipital horn lesion. Bone marrow biopsy was normal. Repeat CSF analysis showed atypical cells with melanin pigment. He underwent S1 root and arachnoid biopsy which showed features of malignant melanoma. His clinical condition deteriorated rapidly to quadriplegia and bulbar palsy. He was administered intrathecal Nivolumab and oral B-RAF inhibitors. He died within two months. Conclusion: Meningeal metastasis should be considered in the differential diagnosis of rapidly evolving cumulative and multiaxial CNS disease.

Award ID: 678

POEMS Syndrome: An Atypical Presentation with Chronic Diarrhoea

Sridhar Balaga, Butchiraju G, Gopi S, Sateesh Kumar T

Neurology, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

E-mail: sridharbalaga@gmail.com

Background and Aim: POEMS syndrome is a rare paraneoplastic condition associated with polyneuropathy, organomegaly, monoclonal gammopathy, endocrine and skin changes. Evaluation of a case with diarrhoea, peripheral neuropathy, sensory ataxia, hyperpigmentation of skin and organomegaly. Methodology: A 46 year old woman known hypothyroidism patient presented with 1 year history of diarrhoea (3 times/day, without blood, mucus or pus), accompanied by diffuse abdominal pain, anorexia and weight loss (10% of her body weight). 6 months history of pain, paresthesias, weakness of limbs, pigmentation of skin. Result: On examination hyperpigmentation over face, hand, lower abdomen and foot, hepatomegaly, peripheral nerve thickness, absent DTRs, impaired vibration and joint sense, rombergs sign positive. PCNA weakly positive, ANA weakly positive, serum electrophoresis- monoclonal gammopathy, nerve biopsy - chronic axonopathy with demyelination and inflammation. Conclusion: POEMS syndrome should be suspected in the presence of peripheral polyneuropathy associated with monoclonal gammopathy; diagnostic workup is challenging and delay in treatment is very common. Gastrointestinal involvement is not frequent and initial presentation with chronic diarrhoea is unusual and rarely mentioned in the literature.

Award ID: 679

Case series of Rare Isolated Extraocular Muscle Palsies due to Strategic Infacrts

Sabeeha Naaz, Ramachandra Ramesh, Kamera Sateesh Kumar

Neurology, Gandhi Medical College/Hospital, Secunderabad, Hyderabad, Telangana, India

E-mail: drsabeehanaaz@gmail.com

Background and Aim: Isolated unilateral extraocular muscle palsies are very rare. Very few such cases have been reported. However, most of them have other features of third nerve palsy like ptosis /mydriasis. Here we present three cases of unilateral isolated extraocular muscle palsies due to strategically placed infarcts. Methodology: 65 year old male presented with complaints of diplopia since 5 days, sudden, painless more while seeing near objects with maximum horizontal separation of images on looking to extreme left. Examination showed right medial rectus palsy. 40 year old female presented with vertical diplopia on seeing right up with head tilt to right. Examination showed left inferior oblique palsy. 28 year old female presented with horizontal diplopia on seeing left. Examination showed right medial rectus palsy with nystagmus of left abducting eye. All three patients had no other signs of third nerve involvement and no other neurological abnormality Result: MRI in all three cases showed strategically placed infarct involving only isolated fascicle of 3rd nerve supplying right medial rectus, left inferior oblique and right medial longitudinal fasciculus respectively. Conclusion: Unilateral ocular palsy is usually seen with lesions in the orbit or muscle disease. These cases are unique with an unusual presentation due to strategic location of infarct in brain stem involving medial rectus, inferior oblique subnuclei and medial longitudinal fasciculus. Hence, systematic clinical approach with history and examination is needed to avoid missing a serious diagnosis like cerebrovascular accident.

Award ID: 680

To assess the Feasibility and Inter-Rater Reproducibility of MS Pro Discuss Tool in Indian People with RRMS.

Manish Mahajan, Sumit Singh, Prabhjot Kaur

Division of Neuroimmunology, Department of Neurology, Artemis Agrim Institute of Neurosciences

E-mail: drmanishneurology@gmail.com

Background and Aim: Multiple Sclerosis Progression Discussion Tool (MSProDiscuss) is a questionnaire-based free online tool for healthcare providers, available at https://msprodiscuss.com/. and developed to help the practicing neurologists to identify progressive MS at an early stage in people with relapsing remitting MS (pwRRMS). The objective of this single center study was to assess the feasibility and inter-rater reproducibility of this tool in Indian pwRRMS by a MS nurse and a neurologist. Methodology: 40 pwRRMS from our MS clinic were administered this tool by a MS nurse followed by a MS physician independently at the same visit. The time for administration of the tool and the inter-rater reproducibility in discriminating pwRRMS into green, yellow and red subgroups between both the raters were assessed. All the participants provided feedback about the convenience of administration and relevance and completeness of the tool. Result: In our cohort, the median duration of MS was 6 years and median age was 36 years. No statistical significant differences were found between both the raters for the time of administration and the discrimination of pwRRMS into green, yellow and red subgroups using MS Pro Discuss tool. Most of the participants found this tool to be convenient, relevant and complete. Conclusion: MSProDiscuss is a feasible, convenient and relevant tool in Indian settings that can assist healthcare providers with minimal training in identifying progressive phase in pwRRMS at an early stage.

Award ID: 681

Real world Evidence of Erenumab In Refractory Migraine in Indian Setting

Ramakrishnan Ramalingam, Jaya M. P. Krishnan, Saleem Akhtar

Neurology, KG Hospital

E-mail: priyarams01@gmail.com

Background and Aim: Background: Erenumab is a novel CGRP receptor blocker that has been available in India since 2021, but no real-world Indian evidence on its use in chronic migraine has been published. In this paper, we examined its efficacy and safety in chronic migraine refractory to conventional migraine prophylactics. Methodology: Methods: This is a single-center retrospective real-world analysis. The trial included patients with chronic migraine who received erenumab as monotherapy or as an add-on medication between August 2021 and March 2022. The treatment response was assessed using the Monthly Migraine Days (MMD), Acute Migraine Specific Medication Days (AMSMD), and the Headache Impact Questionnaire – 6 (HIT-6). Result: Results: This study included 10 patients who had a history of chronic migraine. The participants’ disease duration was 12.8 years on average, and ~3.6 earlier preventive treatments failed. The average MMD, AMSMD and HIT-6 score at baseline were 21.2, 19.9 and 63.8 and they were reduced to 5.8, 4.9 and 36.8 respectively by month 3. 7 out of 10 patients achieved >70% of reduction in MMD at month 3. There were only a few adverse events (1 constipation: 2 injection site reaction). Conclusion: Conclusion: - In real-world use, erenumab was found to be efficacious and safe for the prophylaxis of refractory migraine.

Award ID: 682

Interesting Case of Quadriplegia

Naveen, Balakrishnan R

Neurology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India

E-mail: peenav007@gmail.com

Background and Aim: Guillain, Barré and Strohl described the main clinical features of Guillain-Barré syndrome (GBS): motor weakness, areflexia, minor paresthesias and cerebrospinal fluid (CSF) albumino-cytological dissociation. Common subtypes of GBS are acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, acute motor-sensory axonal neuropathy and Miller Fisher syndrome. Fulminant cases of GBS mimicking brain death have been reported in which a rapid clinical deterioration occurs over hours to days This clinical presentation is very rare, disease diagnosis can be challenging and patient mortality rate is high. Methodology: A 65 year old female a known case of DM, HTN, DEPRESSION presented to the emergency Department with complaints of tingling and numbness in both lower limb and left upper limb she also had weakness of left upper and lower limb she was initially treated as stroke with in few hours of admission she had worsening of symptoms and difficulty in breathing and aspiration she was shifted to ICU Result: Her general examination was normal but neurological examination suggestive of Areflexia, B/L Plantar -extensor, NCS suggestive of demyelination and imaging was with in normal limits she was started on IVIG on the second day of her admission she was fully quadreplegic with ophthalmoplegia and pupils not reacting Conclusion: Fulminant GBS has a rapidly deteriorating clinical course associated with high mortality. Patients treated with multiple course of immunotherapeutic agents and persistent supportive care can have good outcome.

Award ID: 684

Paraneoplastic Neurological Syndrome: A Case Series from a Tertiary Care Centre in Hyderabad

Sandhya Manorenj, Reshma Sultana Shaik

Neurology, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Background: Paraneoplastic syndrome are very rare. Literature suggests that paraneoplastic syndrome occurs in up to 8% of cancer patients. There are only limited case series on paraneoplastic neurological syndromes (PNS). Aim: We present a case series study of PNS during a period of one year at our centre Methodology: Methods: It was an observation Ambispective case series study that included clinical features and evaluation for the primary malignancy and treatment response Result: Results: Five cases met the definitive criteria for PNS. 80% had no known neoplasm. Mean age was 52.6 ± 10.2 years with near equal sex predilection 3: 2 [M: F]. PNS and associated antibody were lambert eaten Myasthenic syndrome – anti VGCC antibody, limbic encephalitis – anti CASPR, pan cerebellar syndrome – anti Yo, limbic encephalitis with extrapyramidal syndrome – anti Ma 2 antibody and opsoclonus myoclonus ataxia syndrome –anti Ri antibody respectively. Whole body PET scan was negative in 80% cases. MRI brain with contrast was normal in all the five cases. All the patients received immunotherapy (pulse steroids : n = 5; Azathioprine : n = 2, plasma exchange : n = 3; pulse cyclophosphamide: n = 1; rituximab = 1; 3,4 DAP : 1). There was complete recovery in CASPR related limbic encephalitis, near complete improvement in Ma 2, Ri and VGCC antibody associated PNS. Treatment response was partial in Yo antibody associated PNS. Conclusion: Conclusions: PNS are rare and are the early manifestation of occult malignancy. PET scan can be negative in majority. Majority benefitted with Immunotherapy which is the mainstay of treatment. Choice of immunotherapy should be individualized based on age, gender and type of PNS.

Award ID: 685

A Rare Case of Heterotopic Ossification

Parvathy K S, Lakshmi Narasimhan R, Lakshmi Narasimhan R, K Shanmugasundaram, Jawahar M, Lakshminarasimhan Ranganathan, J Manickavasagam, Vinoth Kannan

Madras Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: ksparvathysaneesh@gmail.com

Background and Aim: We report a case of neurogenic cause of heterotopic ossification (HO) of left knee following head injury in a patient after road traffic accident. Neurogenic cause of HO is rare and the incidence in patients with traumatic brain injury is about 20%. Methodology: 26 year old male with history of road traffic accident 18 months back had sustained head injury quadriparesis and left mid shaft of humerus fracture. MRI brain showed diffuse axonal injury and subarachnoid hemorrhage with cerebral edema. His GCS was E1VTM5 on admission. He was in prolonged period of coma in ICU care for about 4 months. Left arm fracture fixation was done. GCS improved to E4V2M6 and he was discharged. Quadriparesis improved and he could walk with support. Physiotherapy was continued. Since past 6 months he developed progressive stiffness of left knee joint with restricted range of movements and severe tenderness. Routine investigations were within normal limits except for elevated Alkaline phosphatase. CT scan of knee joints showed heterotopic ossification of left thigh and knee. He developed this despite having no fracture in the left leg Result: HO is a pathologic phenomenon in which ectopic lamellar bone forms in soft tissues. It develops when multipotential connective tissue cells transform into chondroblasts and osteoblasts presumably under the influence of locally induced growth factors. Recent studies show patients who suffered bone fracture combined with traumatic brain injury are at significantly increased risk of HO Conclusion: Our patient developed HO in a non fractured site after a period of 12 months after traumatic brain injury which is a morbid complication to be anticipated

Award ID: 687

Balloon Angioplasty and Stenting in Critical Stenoses of Bilateral ICA - A Case Study

Vivek Iyer

Neurology, SRM Institutes for Medical Sciences, Chennai, Tamil Nadu, India

E-mail: drvivekiyer1984@gmail.com

Background and Aim: Symptomatic internal carotid artery stenosis is associated with a high risk of recurrent stroke and usually managed conservatively and rarely EC-IC bypass in selected patient. Methodology: We discuss the patient with critical stenoses of bilateral ICA causing hemodynamic acute stroke. Case1:43 years old female, diabetic and recently covid recovered, had right hemiparesis, left facial paresis, severe giddiness & dysarthria. Her mRS was 5/6. Her MRI revealed acute non-hemorrhagic infarct in left corona radiata and centrum semiovale. CT Angiogram showed complete occlusion of left terminal ICA with left MCA filling through ACom and mild to moderate stenosis of right ICA at carotid level. CT Perfusion showed ischemic penumbra in left MCA territory. She underwent DSA which revealed total occlusion of left ICA supraclinoid segment and critical stenosis of right ICA at caverno-clinoid junction. In view of sudden worsening of symptoms, she underwent balloon angioplasty and Stenting of bilateral ICA. There were no periprocedural complications. Post-procedure, she had improvement in the right hemiparesis. She was discharged with dual antiplatelet and statin. Check angiogram after 6 months showed good instent flow. Her mRS was 1/5 Result: Timely endovascular treatment of bilateral ICA stenoses resulted in good clinical outcome. Percutaneously angioplasty and stenting is technically feasible and can be an alternative for EC-IC bypass Conclusion: Intervention for total occlusion is done rarely. This case is reported as the patient had significant improvement after intervention for total occlusion of left ICA. Bilateral ICA stenosis can be treated with stenting with good technical and clinical results.

Award ID: 688

Relapsing Cerebral Ring Enhancing Lesions – The Diagnostic Duel Between Toxoplasmosis and Multiple Sclerosis

Udari Samarasiri, Thashi Chang

Neurology, National Hospital of Sri Lanka, Sri Lanka

E-mail: Udarit@gmail.com

Background and Aim: Multiple sclerosis with closed ring enhancing lesions is rare. We report a case of recurrent cerebral ring enhancing lesions and discuss how a demyelinating disease was differentiated from cerebral toxoplasmosis. Methodology: A 44-year old female was evaluated for recurrent presentations with neurological deficits over 15-years, including left optic neuritis, cortical, cerebellar and spinal cord involvement. The Magnetic resonance imaging (MRI) on each episode revealed closed ring enhancing lesions of brain and in one episode a short segment white matter lesion in spinal cord which responded to steroid therapy. She was initially treated as for cerebral toxoplasmosis for a course of 2-years based on serum Toxoplasma Immunoglobulin M positivity and MRI appearance of the lesions. However, other serological and histological investigations did not show any conclusive evidence. Result: Although closed ring enhancing lesions were atypical and cerebrospinal fluid was negative for oligoclonal bands, multiple sclerosis was diagnosed based on relapsing remitting clinical course, response to steroids and fulfilling of current diagnostic criteria after excluding other possible differential diagnoses. Conclusion: Multiple sclerosis may present as closed ring enhancing lesions of brain. It will pose a challenge leading to diagnostic delay.

Award ID: 689

Safety and Efficacy of Perampanel in Difficult to Treat Patients with Epilepsy

Mehul, Nirmal Surya, Nirmal Surya

Neurology, Bombay Hospital

E-mail: drmehuldesai07@gmail.com

Background and Aim: Global prevalence of epilepsy is approximately 1% and that of India mirrors global data. Perampanel is a newer first in class anti-seizure drug directed against AMPA receptors. As almost 1/3rd of epilepsy patients has difficult to treat epilepsy, current study was undertaken to find safety and efficacy of Perampanel in difficult to treat patients of epilepsy. Methodology: It is a prospective observational study conducted at Epilepsy Foundation, Mumbai over a period of 2 years. We enrolled all the patients with difficult to treat focal or generalized epilepsy above age 12 years and started them on Perampanel as adjuvant therapy. Patients were monitored for seizure reduction, seizure freedom and drug tolerability. Those with status epilepticus, age < 12 years and absence seizures were excluded from study. Result: Out of total 40 patients enrolled in the study, 6 were lost to follow up hence removed from final analysis. Amongst 34 patients 44% were female and 56% male. Perampanel was started as first add on in 8%, 2nd add on in 38%, 3rd add on in 39% and 4th add on in 15% patients. Patients in whom drug was started as first or second add on had highest seizure reduction. Conclusion: Perampanel is a drug with novel mechanism which is well tolerated and safe. Drug is most effective when started early in the course of treatment.

Award ID: 690

A Rare Case of Extra Pontine Myelinolysis in a Postnatal Woman

Ravi S, Saravanan Sankaralingam

Department of Neurology, Tirunelveli Medical College, Tamil Nadu, India

E-mail: drsravi83@gmail.com

Background and Aim: Osmotic demyelination refers to the phenomenon of apoptosis of myelin from the neural cells, especially oligodendrocytes due to abrupt osmotic shifts between intracellular and extracellular compartments. Extrapontine myelinolysis (EPM) is a form of osmotic demyelination, a rare yet feared complication of hyponatremia correction treatment which develops due to rapid correction of hyponatremia. Methodology: 27 year old female, P2L2, postoperative day 15, with significant past history of hyponatremia correction and gestational hypertension referred from outside hospital, developed acute onset gradually progressive neurological illness in the form of fever and altered sensorium for 3 days with no history of headache or seizures. On examination, the patient was disoriented, with bilateral upper limb tremor, pyramidal pattern of weakness and rigidity in all the four limbs with exaggerated deep tendon reflexes and extensor plantar response without any meningeal signs. MRI brain showed T2/FLAIR hyperintensities with subtle diffusion restriction in cerebellum, hippocampus, posterior limb of internal capsule, basal ganglia and splenium of corpus callosum. Hence a diagnosis of extra pontine myelinolysis was made and was treated with correction of dyselectrolytemia, low dose of dopa and other supportive management. The patient improved in due course. Result: EPM is a rare complication of rapid correction of hyponatremia. Correction of dyselectrolytemia is the main stay of treatment but parkinsonian symptoms of EPM can respond to low dose dopa. Conclusion: EPM can present with pyramidal and extra pyramidal features. EPM makes full recovery with symptomatic support and cautious dyselectrolytemia correction.

Award ID: 691

Identification of Novel Single Nucleotide Variations using Whole Exome Sequencing in Indian ASD patients

Prashasti Yadav, Saileyee Roychowdhury, Nilanjan Mukherjee, Reema Mukherjee, Soumen Bhattacharjee, Sudipta Kumar Roy, Sumedha Mondal, Parimal Das

Centre for Genetic Disorders, Institute of Science, Banaras Hindu University, Varanasi, Uttar Pradesh, India

E-mail: prashastiy@gmail.com

Background and Aim: Autism Spectrum Disorders (ASD) is a neurodevelopmental disorder with high phenotypic and genetic variability. Whole exome sequencing has shown that single-nucleotide variations (SNVs) play an important role in its pathogenesis. The present study aimed to search for novel SNVs using whole exome-sequencing in ASD patients. Methodology: Two Indian ASD patients were identified on the basis of clinical diagnosis. One is 19 years male patient presented with severe neurodevelopmental regression since the age of 11 years and he also have seizure disorder. Another patient is 3 years female child with physical features- round face, macrocephaly, low set ears, hypertelorism, thin upper lip and she also has the history of neonatal jaundice and febrile convulsion at the age of 1 year. For recognizing mutated gene (s) whole exome sequencing (WES) was performed for these two patients and variants were analysed by autosomal dominant mode. Sequences were analysed and variants were identified. Result: Mutation in six genes were identified viz. HECW (NM_020760.4) c.1234C>T, SON (NM_032195.3) c.2150C>G, from female patient and KIF1A (NM_004321.8) c.3839C>T, SETD5 (NM_001080517.3) c.314A>C, ERMARD (NM_018341.3) c.1523G>A, MAPK8IP (NM_015133.5) c.2849C>T from male patient. A novel mutation in ERMARD c.1523G>A was identified as pathogenic variant which is predicted to cause the loss of protein function through truncation. Conclusion: The findings of this study suggest that mutation in ERMARD (NM_018341.3) c.1523G>A underlie the ASD susceptibility. Further investigation for functional studies in cell and animal models are needed to understand the mechanism of action of this variant in the pathogenesis of ASD.

Award ID: 692

A Peccant Tale of an Innocent Investigation - Unmasking of Idiopathic Intracranial Hypertension (IIH) Associated Spontaneous CSF Leak Following an RT PCR Swab Test for SARS COV 2

Chandra Reddy Singireddy, Reshma Sultana Shaik, Sandhya Manorenj

Neurology, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: scr.5656@gmail.com

Background and Aim: Cerebrospinal fluid (CSF) leaks can be categorized based on the underlying etiology, anatomic site of leak, and underlying ICP. Unusual causes of CSF rhinorrhea have resurfaced during the current pandemic like a traumatic nasal swab test. Here we describe a patient with a spontaneous CSF leak in presence of an otherwise asymptomatic IIH, that was unmasked after a CSF leak repair. Methodology: A 40-year-old obese lady presented with complaints of watery discharge on bending forward, from the left nostril for 15 days. She had undergone an RT PCR swab test for COVID 19, 2 days prior to the onset of her complaints. History was otherwise unremarkable. A diagnosis of traumatic CSF rhinorrhea secondary to RT PCR swab testing was considered. On examination, her BMI was 36. Fundus examination showed Friedman grade I papilledema. General and Systemic Examination was otherwise normal. Result: MR Cisternography had no demonstrable evidence of CSF leak into the sinuses or nasal cavity. CT cisternography showed a focal defect in the left posterior cribriform plate with an active CSF leak. MRI Brain showed features of IIH with kinking of optic nerves and increased perioptic CSF space, empty sella configuration with an unremarkable MRV. The patient underwent a CSF leak repair, following which she developed holocranial pulsatile headache unmasking her IIH. She was advised for weight loss, started on acetazolamide and kept under close follow-up. Conclusion: 1. Isolated CSF rhinorrhea can be the only presenting symptom of an asymptomatic IIH 2. CSF leak repair may be associated with resurfacing of symptoms related to raised ICP, which were previously nonexistent

Award ID: 693

Minipolymyoclonus and Hirayama -Like Disease as the Atypical Clinical Manifestation of Cervical Compressive Myelopathy

Sandhya Manorenj, Rindha Venepally Rao, Reshma Sultana Shaik

Neurology, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Background: Hirayama disease is a cervical flexion myelopathy that typically have male predilection and causes upper extremities weakness and wasting involving C 7, C8 and T1 myotomes. Cervical extramedullary compressive myelopathy usually presents with radicular pain with myelopathy features with definite sensory and motor findings are usually described. Very rarely isolated motor weakness and wasting reported. Hirayama disease like clinical features are reported very sparse in literature. Aim: Here we present a young male with clinical features of Hirayama disease with radiological features of cervical compressive myelopathy due to prolapse disc Methodology: Methods: A case report study showing clinical and radiological features of the study case. Result: Results: A 25 year male presented with left hand shaking and difficulty to lift weight since 2 years. He had occasional occipitonuchal headache. Neurological examination revealed asymmetric (left >> right) wasting of muscles of hand (thenar, hypothenar and intrinsic muscles), and extensor muscles of forearm sparing brachioradialis showing oblique atrophy. Motor weakness was noted in the atrophied muscles. There was minipolymyoclonus of left fingers. Rest of the neurological examination was normal. Dynamic MRI cervical spine with contrast did not show any radiological features of Hirayama disease. Instead MRI showed significant central and right disc prolapse at C2-C3 and C3 – C4 level with cord compression and myelomalacia changes opposite to the side of clinical severity. Conclusion: Conclusions: Pure motor symptoms and minipolymyoclonus are unusual clinical features of cervical compressive myelopathy. All Hirayama like disease clinically, are not true Hirayama disease. Gadolinium contrast flexion dynamic cervical spine MRI confirms Hirayama disease

Award ID: 694

Primary Sjogren Syndrome with Isolated Trigeminal Neuropathy as Initial Presentation-A Rare Presentation

Dulari jith, Arun Kumar, Uma Maheshwari, S. Bala subrahmanian, R. Lakshmi Narasimhan

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: dularijith@gmail.com

Background and Aim: Introduction: We report a case of primary sjogren syndrome with initial presenting symptom itself as trigeminal small fibre sensory neuropathy causing burning mouth syndrome. Methodology: Case Scenario: 54 year old female with no prior comorbidities presented with chronic progressive neurological illness of 2 years duration in the form of burning paresthesia inside the mouth, tingling and pin pricking sensations around the mouth. Patient later developed tightness of both cheeks causing difficulty in opening with incomplete closure of mouth and foreign body sensation in both eyes and dry lips. On examination patient has thickened skin over face and extremities, salt and pepper pigmentation over cheeks on both sides, decreased facial sensation for touch, pain and temperature inside mouth and entire face sparing periphery Result: Onevaluation autoantibodies for sjogrensyndrome were positive with lipbiopsysuggestive of sjogrens disease. MRI Brain with CISS sequence for trigeminal nerve was normal. Patient improved dramatically with steroids. Conclusion: Discussion: Burning mouth syndrome is a chronic and intractable orofacial pain syndrome characterized by the presence of burning sensation of the oral mucosa in the absence of specific oral lesion. This condition mainly affects middle aged and elderly woman and is multifactorial in orgin. In our case trigeminal sensory neuropathy is the cause for the same. Primary sjogren syndrome presenting as isolated trigeminal neuropathy is rare. This case suggested that the signs of simple trigeminal lesions, especially those with atypical manifestations could be the early manifestation of other systemic diseases. So enough attention should be paid to identification of the pathogeny of the primary disease to achieve early identification, diagnosis and proper treatment.

Award ID: 695

A Clinical and Laboratory Profile of Neuromyotonia- A Study of 15 Patients at a Tertiary Care Hospital in Mumbai

Nirbha Ghurye, Darshan Pandya, Riddhi Patel, Khushnuma Mansukhani, Satish Khadilkar

House Officer Neurology- MBBS, Nair Hospital

E-mail: nirbhaghurye@gmail.com

Background and Aim: Neuromyotonia is a rare condition characterized by peripheral nerve hyperexcitability due to a varied etiology. 1,2,3,4 We would like to document the clinical and etiological spectrum of Neuromyotonia through our study of 15 patients with electromyographically confirmed Neuromyotonia. Methodology: This is a retrospective study of patients who presented to a tertiary care center between 2010 to 2022 with neuromyotonic discharges on electroneuromyography. They underwent a detailed history and clinical examination. Blood tests like VGKC complex antibody, electrolytes, total CPK levels, and thyroid function tests were conducted along with heavy metal studies in selective cases. PET scan was also used for evaluation. Result: A total of 15 patients were analyzed. The mean age at symptom onset was 34.67 years, with a range from 12 to 61 years. A gender predilection for males was observed (14/15 cases). Twitching was the most common symptom in all cases, followed by pain and sweating in 13 and 12 cases respectively. Central symptoms were seen in only four cases. An autoimmune etiology with serum VGKC antibody, predominantly, CASPR2 antibody-positive was seen in nine cases. Elevated blood mercury levels were seen in one patient, and a thymoma on PET-CT was detected in another, who recovered completely post thymectomy. Management-wise, all patients received a steroid trial. Amongst the steroid-refractory cases, two received plasmapheresis, Inj. IVIG and azathioprine were also administered in two different cases Conclusion: Neuromyotonia is a rare condition, most likely due to an autoimmune etiology. One should be familiar with this possibility as it is a potentially treatable cause.

Award ID: 696

Etiology of Posterior Circulation Stroke

Meet Raval, Umangkumar Patel, Sudhir Shah, Mayank Patel, Shalin Shah, Pranav Joshi

Neurology, SVPIMSR, Ahmedabad, Gujarat, India

E-mail: meet110592@gmail.com

Background and Aim: Clinical profile and prognosis of ischemic stroke depends on the site of involvement of arteries. Identifying etiopathology of posterior circulation stroke is essential so that appropriate preventive therapies can be instituted. Methodology: This is a prospective observational study conducted at tertiary care hospital from November 2019 to December 2021, where patients with >12 years of age, with acute ischemic posterior circulation stroke presented within 14 days. Etiology PC stroke was categorized according to TOAST classification. Result: Out of 56 patients of PC stroke, 36 (64.28%) were males. Mean age was 63.8 years. 41 (73.2%) patients were of the age > 60 years. Unilateral limb weakness was seen in 9 (16.6%), headache in 7 (12.5%), crossed sensory loss in 4 (7.14%) and diplopia in 2 (3.6%). Most common risk factors were hypertension (69%), dyslipidemia (54.7%) and diabetes mellitus (42.5%). The most common etiology found was large artery atherosclerosis (LAA) in 28 (50%), small artery occlusion (SAO) in 6 (10.71%) cardio embolism in 7 (3.57%) and stroke of other determined aetiology in 2 (3.57%) patients. 19 (33.92%) patients had PC stroke due to undetermined etiology. Posterior cerebral artery involvement was seen in 27 (48.21%) and basilar artery involvement in 12 (21.43%), vertebral artery occlusion in 10 (17.86) and posterior inferior cerebellar artery (PICA) in 7 (12.5%) patients. Conclusion: Most common etiology of PC stroke was found to be LAA followed by SAO. Around one third of PC stroke had undetermined etiology. Posterior cerebral and basilar artery were most commonly involved vessels.

Award ID: 697

Clinical Profile of Patients with Refractory & Super-Refractory Status Epilepticus

Kinjal Patel, Amey Bhise, Sudhir Shah, Mayank Patel, Shalin Shah, Pranav Joshi

Neurology, SVPIMSR, Ahmedabad, Gujarat, India

E-mail: kpatel71234@gmail.com

Background and Aim: Refractory SE (RSE) & Super refractory status epilepticus (SRSE) are neurological emergencies with high morbidity and mortality. Therefore, aim was to study the demographic features, clinical profile, etiology and outcome at the time of discharge of patients presenting with refractory & super-refractory status epilepticus. Methodology: In hospital prospective observational study conducted at Tertiary care center, during 2019- 2022, where all patients above 12 years of age presenting with RSE & SRSE were enrolled. Those that occurred secondary to chronic neurodegenerative and chronic neurometabolic diseases (such as mitochondrial disorders, inborn errors of metabolism etc.), secondary to alcohol withdrawal were excluded. Result: Out of 44 patients with status epilepticus, 13 (29.5%) were of RSE & 6 (13.6%) were of SRSE. Median age for RSE was (37 years) & SRSE was (45.33 years). Convulsive status epilepticus was the most common type in both subgroups. CNS infection was the most common etiology. Minimum 4 AED’s were required in RSE & 6 in SRSE for its adequate control. Mean duration of status was 6.3 hours in RSE & 49.5 hours in SRSE. In both the groups majority of patients 17 (89.5%) had higher STESS on admission & longer ICU stay. Total 8 (42.1%) patients could not survive. Conclusion: Acute symptomatic seizure was most the most common presentation for both RSE & SRSE. Despite aggressive management, mortality was high.

Award ID: 698

Stroke in Young Secondary to Dissecting Vertebral Artery Aneurysm- A Rare Entity

Vikashini Kanagaraj, Balakrishnan R

General Medicine, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India

E-mail: Vikashinikanagaraj@gmail.com

Background and Aim: Vertebral arteries are the chief arteries of medulla and also supplies posterior part of cerebellar hemisphere. Approximately 20% of strokes originate from the vertebrobasilar system and are due to specific vertebral artery pathology, including atherosclerosis, fibromuscular dysplasia, neurofibromatosis, arteritis and dissection. The risk factors leading to vertebral artery dissection include smoking, hypercholesterolemia, hypertension, Diabetes, neck trauma.16% of vertebral artery dissecting aneurysms are due to neck trauma. Methodology: This 36 year old female presented with complaints of giddiness, left sided facial numbness and severe neck pain with history of neck manipulation. CNS examination revealed cerebellar signs. MRI Brain revealed acute Left cerebellar/ left posterolateral medulla infarct. Patient was diagnosed as lateral medullary syndrome and started on antiplatelets, anticoagulants, statins. Workup for young stroke revealed no significant abnormalities. CT angiogram of brain was done which revealed two focal segments of severe luminal narrowing in V3 segment of left vertebral artery. DSA revealed Left vertebral artery V3 segment dissecting aneurysm. Result: Follow up DSA after 12 weeks revealed completely absent dissecting vertebral artery aneurysm Conclusion: Spontaneous dissection of the vertebral artery is a potentially disabling and probably underdiagnosed cause of stroke mainly affecting young adults. A young patient presenting as posterior circulation stroke with a history of neck injury or manipulation, Vertebral artery dissection must be ruled out. Clinical outcomes in such cases are more favourable.

Award ID: 699

Diagnostic MRI Clue in the Diagnosis of HSP

Amarendhar Malreddy, Amarendhar Reddy Malreddy

Neurology, Guntur Medical College, Andhra Pradesh, India

E-mail: amarendhar48@gmail.com

Background and Aim: The “ears of the lynx” MR imaging sign has been described in case reports of hereditary spastic paraplegia. mostly associated with mutations in the spatacsin vesicle trafficking associated gene, causing Spastic Paraplegia type 11 (SPG11). This sign corresponds to long T1 and T2 values in the forceps minor of the corpus callosum, which appears hyperintense on FLAIR and hypointense on T1-weighted images. Methodology: This is a case of 25 years old male presented with complaining of difficulty walking with slurring of speech since five years. MRI brain showed T2/FLAIR hyperintensity in the region of forceps minor. Genetic analysis showed SPG 11 mutation. Result: A 25 years old male born out of consanguineous parentage presented with progressive weakness of both lower limbs and slurring of speech since five years. On examination stiffness in both lower limbs with extensor plantors with no cerebellar or extra pyramidal signs. MRI brain showed T2/FLAIR hyperintensity at the tip of the frontal horns of lateral ventricles. Genetic analysis showed SPG 11 mutation. Conclusion: The ears of the lynx sign on FLAIR MR imaging is highly specific for the most common genetic subtypes of hereditary spastic paraplegia. When this sign is present, there is a high likelihood of a genetic mutation, particularly associated with SPG11 or SPG15.

Award ID: 700

A Rare Case of Marinesco Sjogren Syndrome in a 11 Year Old Male Child

Saravanan S, Ravi Subbiah

Department of Neurology, Tirunelveli Medical College, Tamil Nadu, India

E-mail: drsaravananneuro@gmail.com

Background and Aim: Marinesco-Sjogren syndrome (MSS) is a rare disease which belongs to the group of autosomal recessive cerebellar ataxias. (OMIM 248800). It’s clinico-pathological and genetic features are still ambiguous Methodology: 11 year old male child, born out of third degree consanguinous marriage, with significant past history of bilateral cataract excision and similar family history in elder brother, presented with chronic progressive neurological illness in the form of microcephaly, psychomotor developmental delay, ataxic gait and dysarthria for 5 years, pyramidal pattern of motor weakness and sensory impairment of touch, pain and temperature below knee for 3 years with significant pan cerebellar and posterior column signs. The child had also syndromic facies and skeletal deformities. The child was diagnosed as a case of Marinesco Sjogren syndrome and genetic analysis was sent. MRI brain showed cerebellar atrophy. EMG showed myopathic features and NCS showed normal CMAP with decreased SNAP. Result: The index patient had additional atypical findings of sensory system involvement which is rarer in this rare disease along with features of MSS and hence we present this case. Conclusion: Marinesco Sjogren syndrome is a rare autosomal recessive cerebellar ataxia with a wide range of multiple neuraxial presentation and multi system involvement. Features of MSS include tetralogy of cerebellar ataxia, congenital cataract, intellectual disability and progressive muscle weakness.

Award ID: 701

Isolated Central Variant of Posterior Reversible Encephalopathy Syndrome Involving Posterior Circulation, Coexisting with Acute Stroke in Anterior Circulation: A Diagnostic Challenge and Poor Prognostic Marker

Sandhya Manorenj, Reshma Sultana Shaik, Reshma Sultana Shaik

Neurology, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Introduction: Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiological entity that predominantly manifests on imaging as cortical/subcortical vasogenic edema within the cerebral hemisphere, with a parieto-occipital predominance. Isolated brainstem, basal ganglia, thalamus involvement alone or in combination, without cortical or subcortical involvement is very rare. Objective: Here we describe the clinical and radiological features of central variant of PRES involving brainstem, thalamus, basal ganglion and cerebellum without cortex involvement co-occurring with anterior circulation acute ischemic stroke in an elderly male. Methodology: Methods: We report a case of isolated central variant of PRES associated with acute lacunar stroke. Result: Clinical history and radiological features were extensively studied. Patient had low GCS (Glasgow coma scale-7), accelerated hypertension (210/110 mm Hg), hypertensive retinopathy with features of sepsis and mild azotemia. Cause of central PRES and stroke was due to high blood pressure MRI showed acute lacunar infarct in semi-centrum ovale (middle cerebral artery territory) infarct with central variant of PRES features. Despite best medical management (antihypertensive agents, antiplatelets, and antibiotics) and ventilator support, patient succumbed to his illness. Conclusion: Though PRES is considered as a completely reversible condition, co-occurrence of stroke and PRES (central variant in the absence of cortical/subcortical involvement) can be a marker for poor clinical outcome as observed in this case. High index of clinical suspicion and radiological clues on MRI can guide one in early recognition of this rare entity and plan an appropriate management. Antihypertensive treatment should be individualized.

Award ID: 702

A Case of Cortical Venous Thrombosis Masquerading as GBS - Radiculopathy as a False Localizing Sign

Arun S, Chandramouleeswaran V, Ravi L A, Krishna Kumar, Lakshmi Narasimhan Ranganathan

Madras Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: arunsaravanan@gmail.com

Background and Aim: Intracranial venous sinus thrombosis can have multiple manifestations which include isolated intracranial hypertension, acute or subacute encephalopathy and focal neurological symptoms or signs. However, radiculopathy as a false localizing sign is uncommon. Methodology: A 53 Year old male, chronic alcoholic, hypertensive presented with, headache, blurring of vision and weakness of all four limbs since 5 days. On examination, patient had normal higher mental function, reduced visual acuity in both eyes, bilateral Ophthalmoparesis and bilateral papilledema. Pupils were normal. Other cranial nerves were normal. Spinomotor system examination showed hypotonia and weakness of all four limbs (MRC grade of 4 in both upper limbs and 3 in both lower limbs), generalized areflexia and bilateral plantar showed flexor response. Sensory examination was normal. There was no neck rigidity or cerebellar signs. Result: MRI BRAIN with MRA and MRV showed thrombosis of the superior sagittal sinus, right lateral sinus, sigmoid sinus and venous infarct in right occipital region with no midline shift. NCS showed Bilateral absence of F waves in bilateral peroneal nerves. CSF analysis was deferred as patient had been started on Inj. heparin Our patients showed marked improvement in symptoms with medical management of CVT. Distension and distortion of the subarachnoid space around the nerve roots and venous ischemia of the nerve roots secondary to impaired venous outflow may cause radicular dysfunction. Conclusion: In a patient presenting with flaccid areflexic quadriparesis and papilledema, the possibility of a potentially reversible dysfunction of the cranial nerves and spinal nerve roots due to a marked rise in intracranial and intraspinal pressure must be recognized. Timely recognition is necessary failing which results in deterioration of clinical status and emerges as life threatening condition.

Award ID: 703

Is it Clippers or a Mimic; A Controversy

Nadeesha Premarathna, Arjuna Fernando, Senaka Bandusena

Department of Neurology, National Hospital Sri Lanka, Sri Lanka

E-mail: nnpremarathna@gmail.com

Background and Aim: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) represents a rare central nervous system inflammatory disorder with predominant T cell pathology which characteristically involves the pons. It is characterized by multiple curvilinear or punctate gadolinium enhancement peppering the pons in Magnetic resonance Imaging (MRI) of the brain which is a hallmark radiological feature. Patients present with predominant brainstem syndrome and responds to immunosuppressives including glucocorticoids. Methodology: Fifty-three years old patient with long standing diabetes presented to us with subacute onset of unsteady gait with tendency to fall to left side for two weeks. Meanwhile, he has had some slurring of speech at the outset which was improving gradually. Examination revealed mild left dysmetria with impaired tandem walk in the presence of glove and stocking type of sensory loss. Result: The MRI brain of the patient revealed patchy enhancement with peppering in the pons. The susceptibility weighted imaging and magnetic resonance angiography were normal. He had an ESR of 70 mm/1st hour. There was normocytic normochromic anemia with some rouleux formation. Patient had a significant clinical improvement with high dose methylprednisolone but didn’t return for the follow up imaging. Conclusion: This entity is having a lot of differential diagnoses (CLIPPERS mimics). Therefore, clinicians should be careful in evaluating the patients for possible central nervous system lymphoma, vasculitis or other rheumatological conditions (Behcets disease), paraneoplastic and demyelinating disorders or inflammatory conditions (sarcoidosis) which remain common amongst the many of the CLIPPERS mimics. This is especially true in the presence of systemic symptoms.

Award ID: 704

Comparison of Clinical Features of PSP and MSA Patients Presented at a Tertiary Center

Jay Chaudhari, Zubin Shah, Sudhir V. Shah, Mayank A. Patel, Shalin D. Shah, Pranav B. Joshi

Neurology, NHLMMC/SVPIMSR

E-mail: drjc008@gmail.com

Background and Aim: Atypical parkinsonism is a group of heterogeneous degenerative disorder with Overlapping clinical features. Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are commoner disorders. Hence We aimed to study difference between clinical profile of PSP and MSA patients Methodology: We reviewed clinical features of PSP patients (n = 24) and MSA (n = 22) patients diagnosed according to commonly accepted clinical criteria in cross sectional observational study at tertiary center. Result: Mean age at disease onset for PSP group And MSA group was 65.5 years and 56.9 years respectively. Apart from Parkinsonism (100% in both group) common motor manifestations were falls, present In 87.5%patients of PSP group, 50% patients of MSA group; Ataxia was seen In 72.7% patients of MSA group and 12.5% patients in PSP group. Axial rigidity observed in PSP and MSA group was 87.5% and 9.1% respectively. All 24 patients in PSP group had vertical gaze restriction. FNF was positive in 12.5% in PSP group and 77.3% in MSA group. In non-motor symptoms, REM sleep behavior disorder (RBD) was seen in 20.8% In PSP group and 54% In MSA group; autonomic symptoms like postural symptoms (72.3% vs. 16.7%), sexual dysfunction (63.6% vs. 0%), urinary symptoms (95.4% vs. 58.3%) were more common in MSA group. Mean MMSE score observed in PSP, MSA group was 21.4, 25.4 respectively. Conclusion: autonomic symptoms, RBD and cerebellar signs were more common in MSA, While Falls, Vertical Gaze restriction, axial rigidity and memory impairment were common in PSP.

Award ID: 705

A Rare Presentation of Tubercular Meningitis as Flaccid Paraplegia

Shreyansh Bardiya, Pratik Mittal

General Medicine, MMIMSR, Mullana, Ambala, Haryana, India

E-mail: Shreyansh200993@gmail.com

Background and Aim: Tuberculous Meningitis (TBM) has been found to be associated with paraplegia which is usually always spastic type. Potts’s Spine classically involves the dorsal spine. However, flaccid paraplegia due to lumbosacral region involvement can also occur in TBM and spinal arachnoiditis remains an important treatable cause. Methodology: 31 years female presented to us with history of fever and vomiting since 3 weeks and altered sensorium since 1 week. CEMRI brain revealed basal exudates, early hydrocephalus and tuberculoma. CSF showed lymphocyte predominant pleocytosis. Patient was started on ATT with steroids. Patient gradually recovered; however, she developed paraplegia of grade 1/5, DTRs not elicitable and tone decreased. Possibility of Pott’s Spine was kept and CE-MRI Spine was done which s/o Arachnoiditis, cervical and dorsal spine was however unremarkable. She was therefore given IV Methylprednisolone for 3 days, following which her paraparesis improved and she started walking unassisted. She was discharged on oral steroids, ATT was continued and no other antibiotics were used. Result: TBRM has been used as a generic term to include cases of arachnoiditis, intradural spinal tuberculoma, granuloma and spinal cord complications of tuberculous meningitis. The various pathological forms of spinal TB can cause paraplegia. Tuberculomas within the spinal cord, as well as in intra- and extradural locations, and encasing granulomatous arachnoiditis (radiculomyelopathy) with cord compression and vasculitis of spinal cord vessels has been often reported. In our case, there was a marked improvement with IV Methylprednisolone. It could be possible that this patient had vasculitis leading to ischaemic changes and/or granulomatous reaction of the spinal leptomeninges (both of which have been outlined) as pathophysiology behind the root lesions in TB Arachnoiditis. Conclusion: TB Spinal Arachnoiditis may present as a flaccid paraplegia. Therefore, a high index of suspicion, CEMRI LS spine and early institution of IV Methylprednisolone may be considered.

Award ID: 706

Newly Diagnosed Celiac Disease Presenting as Myeloneuropathy from vit B12 Deficiency

Amruthreddy Muduganti, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshrestha, Abdul Qavi

Neurology, Dr Ram Manohar Lohia Institute of Medical Sciences

E-mail: wizard.amruth@gmail.com

Background and Aim: Celiac disease (CD) is a systemic autoimmune condition triggered by dietary gluten in genetically susceptible individuals [1]. Although historically considered a gastrointestinal disorder, approximately half patients present with extraintestinal symptoms [1]. Several neurologic disorders have been associated with CD [2–4]. As per literature review, Myeloneuropathy from Copper deficiency due to previously diagnosed CD is noted, we are presenting case report of Newly Diagnosed CD presenting as myeloneuropathy due to vit B12 deficiency. Methodology: 15 yrs old male, presented with c/o tingling sensation and paraesthesia in both hands and feet since 6 months, c/o progressive weakness in all 4 limbs since 3 months, c/o incomplete bladder emptying and constipation since 1½month. On detailed history, diagnosed as insidious onset B/L symmetrical gradually progressive sensorimotor quadriparesis (UMN and LMN) with Bowel/Bladder involvement. Past History – intermittent diarrhea and abdominal bloating episodes since 3 yrs. On Physical examination, knuckle pigmentation and B/L foot drop was present. On clinical examination, motor system - B/L Spasticity in both LL. Distal weakness greater than proximal weakness. DTR – Exaggerated with absent ankle reflex and Bilateral plantar extensor. Sensoryexamination – Impaired distally > proximally, Rhomberg sign positive. Diagnosis of Myeloneuropathy most likely Nutritional deficiency. Result: On investigations, S.Homocysteine -significantly elevated (57.78 micromol/L), S.VitB12 = 300 pg/ml (low normal), others normal. As patient is not affordable, serum copper not done. Imaging -NAD. NCS - Axonalpattern. Nerve biopsy = s/oAxonopathy. Duodenal Biospy = villous atrophy with marked increase in intraepithelial Lymphocytes – s/o celiac disease. Conclusion: Initially patient diagnosed as Myeloneuropathy due to VitB12 deficiency and furtherly detected as Celiac disease by biopsy. VitB12 deficiency mostlikely due to Celiac disease. Patient improved on followup and advised glutenfree diet.

Award ID: 707

Cranial Nerve Palsies Half a Dozen but What Could be the Reason?

Srividhya S, Lakshmi Narasimhan, V Chandramouleeshwaran, L. A. Ravi, Krishna Kumar

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: srividhyashankaran@gmail.com

Background and Aim: Skull base chordomas constitute less than 0.2% of all intracranial tumours. There is infiltration and destruction of surrounding bone and soft tissue. Here we look to identify the cause of consecutive cranial nerve palsies from 6 through 12. Methodology: 65 year old male presented with complaints of left sided, insidious onset and progressive headache for the past 2 years. He has double vision in the left gaze, left ear tinnitus and hearing loss with change in voice and slurring of speech for the past 6 months. Left eye abduction restriction, left lower motor neuron type of facial palsy, left ear sensorineural hearing loss, vestibular signs such as nystagmus, vertigo, and Romberg’s sign were present. Direct laryngoscopy revealed left vocal cord palsy. There was drooping of the left shoulder and deviation of tongue to the left side on protrusion. These examination signs corresponded to multiple cranial nerve palsies on the left including 6,7,8,9,10,11, and 12, localizing the lesion to skull base. Result: MRI brain confirmed an expansile lesion involving the clivus, bilateral occipital condyle, left greater wing of sphenoid, petrous part of temporal bone, lateral mass of atlas causing obliteration of jugular fossa, and inlet of left auditory canal with mild heterogenous enhancement. PET-CT also showed no enhancement anywhere else thereby confirming a primary skull base tumor. Patient was transferred to neuro-surgery later and biopsy revealed a skull based chordoma. Conclusion: Skull based chordomas are rare slow growing tumors. The close proximity of this tumor to cranial nerves and vessels makes it a highly challenging tumor to resect. Aggressive surgery and adjuvant radiotherapy are the main modalities of management.

Award ID: 708

Frequency and Spectrum of Premonitory Symptoms in Patients with Migraine

C. Sruthi, Bhupender Kumar Bajaj, Bhupender Kumar Bajaj

Neurology, ABVIMS & Dr. RML Hospital

E-mail: sruthireddy81188@gmail.com

Background and Aim: Migraine has a significant disease burden globally with a constellation of non-painful symptoms apart from headache. Premonitory symptoms, despite their recognition as contributing factors in migraine associated disability, are often under reported. The betterappreciation of spectrum and occurrence of the premonitory symptoms has potential to improve our understanding of migraine pathophysiology and to direct therapeutic interventions. Most of the information available on prevalence of premonitory symptoms inpatients with migraine are based on retrospective studies and are likely to be affected by recall bias. There are no prospective studies available from the Indian subcontinent about premonitory symptoms. We undertook this study to estimate the frequency and observe the spectrum of premonitory symptoms in patients with migraine. Methodology: Study Design: Prospective ObservationalSetting: Outpatients in Department of NeurologyDemographic and headache characteristics of 83 patients with migraine were recorded. Patients maintained a migraine diary including details of premonitory symptoms till documentation of 4 headache attacks or 2 months follow-up period. Descriptive and analytical statistics using MS Excel and SPSS Version 21.0 were used to analyse data. Result: Premonitory symptoms were observed in 87.83% of patients with migraine and the mean number of premonitory symptoms in a patient with migraine was 5.07 (3.658). Migrainous/sensory symptoms (89.23%) followed by mood/cognitive premonitory symptoms (78.46%) were the most common. Autonomic premonitory symptoms (29.23%) were the least frequently reported. Severity of migraine was observed to positively correlate with the no. of premonitory symptoms (p < 0.001). Conclusion: Premonitory symptoms are common and their presence correlates with greater severity of migraine.

Award ID: 709

Long Segment Myelitis Secondary to Neuro Melioidosis

Vithoosan Sahathevan

Institute of Neurology, National Hospital of Sri Lanka, Sri Lanka

E-mail: vithoosan@gmail.com

Background and Aim: Neuro-melioidosis carries a risk of high morbidity and mortality. We describe two Sri Lankan patients presenting with long segment myelitis secondary to melioidosis. Methodology: Case1: 47-year-old male presented with right side hemiparesis which progressed rapidly to quadriparesis. Initial CSF analysis revealed protein 76 mg/dl and glucose 72 mg/dl but without a cellular reaction. MRI spine revealed long segment myelitis. He was treated with IV MPP and plasma exchanges on suspicion of an immune mediated myelitis but without success. A repeat MRI revealed high signal changes in the brain stem and along the entire spinal cord with contrast enhancement. MRI brain after treatment with MPP/PLEX showed enhancing hyper intensities along the corticospinal tracts. The repeat CSF revealed protein 1187 mg/dl, glucose 78 mg/dl, lymphocytes 1600/mm3 and neutrophils 10200/mm3. CSF culture has become positive for Burkholderia pseudomallei. Serum melioidosis antibody titre was 1: 320. He was started on IV meropenem with oral cotrimoxazole for 12 weeks followed by oral co trimoxazole. But he had poor clinical recovery. Result: Case2: 47-year-old female presented with bilateral lower limb weakness for 1-week duration. On examination, she had flaccid paraparesis with a sensory level at T11. CSF analysis revealed protein 50 mg/dl with lymphocytes 172/mm3. MRI pan spine revealed a long segment myelitis. Serum melioidosis antibody titre was 1: 640. She was treated with IV meropenem for 8 weeks followed by oral co-trimoxazole with an excellent clinical and radiological response. Conclusion: These cases signify the importance of considering melioidosis as a differential in patients with long segment myelitis especially in endemic areas.

Award ID: 710

Prevalence of Stroke in Mucormycosis Patients with Covid-19 Infection in India

Poorvi Tangri, Awadh Kishor Pandit, M. V. Padma, A. K. Srivastava, V. V.Y., R. Ranjan, R. K. Singh, D. M.R., Animesh Das, A. Agarwal, Shubham Misra

Department of Neurology, All India Institute of Medical Sciences, New Delhi, India

E-mail: poorvitangri1@gmail.com

Background and Aim: During the second wave of COVID-19, India suffered from a catastrophic outburst of cases and rapid transmission of disease due to the highly infectious delta strain (B.1.617.2). Patients infected with this strain underwent prolonged hospitalisations, suffered from severe symptoms. A sudden surge of fungal infections, primarily Mucormycosis was observed. Methodology: We conducted a case-control study to study various risk factors and form of intracranial involvement in cases of Mucormycosis. Result: Amongst the COVID-19 associated mucormycosis cases, a total of 31/72 (43.05%) cases showed intracranial involvement. Out of the 31 cases, 58.06% (18/31) were males and 41.93% (13/31) were females. Mean age of presentation was 47.54 years (age range 26 to 71 years). Patients with COVID-19 associated mucormycosis were divided into different COVID-19 grades: mild 64.28% (18/28), moderate 32.14% (9/28) and severe 7.14% (2/28). Infarcts were observed in 57.14% (16/28) of the cases, Haemorrhage was detected in only three patients 11.53% (3/26). Abscess formation was seen in 26.92% (7/26) patients, three of the patients displayed meninges involvement and thrombosis was seen in 44% (11/25) of the total patients. Thrombosis was observed in internal carotid artery (ICA) in six patients, in cavernous sinus in two patients and in superior ophthalmic vein in one patient. Patients were treated with administration of Amphotericin B and Posaconazole. Telephonic follow-up was conducted after a time period of about 90 days and their health condition was recorded on basis of modified ranking scale (mRS). Out of the Mucormycosis infection patients displaying the occurrence of stroke, 10 patients could not survive. Conclusion: Intracranial Mucormycosis in COVID-19 patients presenting with stroke were observed frequently and had mortality in about one-third cases.

Award ID: 711

Post-Stroke Fatigue and its Relationship with Clinical Factors Among Chronic Stroke Survivors: A Cross-Sectional Study

Akhila Jagadish, John M. Solomon

Physiotherapy, Manipal College of Health Professions, Manipal, Karnataka, India

E-mail: akjag07@gmail.com

Background and Aim: Post-stroke fatigue (PSF) is considered one of the top research priorities and a least explored symptom among community-living stroke survivors. It is highly associated with impaired functional activity, reduced quality of life, and mortality. Only a few studies in India have looked at the influence of clinical factors on fatigue in chronic stroke. Hence the study aimed to determine the prevalence and relationship of PSF with clinical factors among chronic stroke survivors Methodology: A cross-sectional survey was conducted among the first-time stroke survivors within six months to 2 years. Records were screened and included for telephonic interviews if the participants met the criteria of age >18 years, were cognitively sound, and had no coexisting neurological or systemic condition that would influence fatigue scores. Telephonic consent was obtained, and an interview was conducted to obtain the scores for fatigue, sleep quality, depression, functional activity & disability. Result: A total of 309 subjects were included, of which 196 completed the interview to date. The mean age was 60.53 ± 12.76, with the median stroke duration being 11 months (range, 16 months). The proportion of PSF was 15.8% (n = 31). Among 31, 19.3% (n = 6) reported minimal to minor depression, 29.1% (n = 9) moderate to severe ADL dependency and 45.2% (n = 14) significant disability. Fatigue scores showed a weak correlation with depression (r = 0.20) and mRS (r = 0.36). Conclusion: Most of the PSF patients presented with ADL dependency and disability. They did not show any significant correlation with the clinical factors but with disability scores among chronic stroke survivors.

Award ID: 712

Mild Encephalopathy with Reversible Splenial Lesion in Paratyphoid Infection: A Case Report

Quyen Pham, Thang Ba Nguyen, Uyen Ngoc Le Ha, Chien Cong Phan

Neurology, Ho Chi Minh City University Medical Center

E-mail: quyen.ptn@umc.edu.vn

Background and Aim: Mild encephalopathy with reversible splenial lesion (MERS) is an uncommon clinico-radiological syndrome reported mainly in children. There is limited document on MERS in aldults, especially in Salmonella infection. We present a case of MERS in an aldult patient with blood culture-proven Salmonella paratyphi A. Methodology: A 33-year-old man with persistent fever for one month presented with one week of diarrhea and altered mentation. Physical examination showed a temperature of 39 degrees Celsius, slurred speech, drowsiness, recent memory disturbance with intermittent hallucinations and otherwise normal vital signs. Abdominal CT examination showed slight symmetrical thickening of the wall of the terminal ileum, cecum, ascending colon. Brain MRI showed well- defined irregular lesion of the splenium was seen on DWI/ADC with restricted diffusion and no enhancement on T1 with Gadolinium. Blood cultures grew Salmonella paratyphi A associated with normal results in cerebrospinal fluid examination. Figure 1. Brain MRI revealed well- defined irregular lesion with restricted diffusion of the splenium on DWI (A) and hypointensity on apparent diffusion coefficient map (B) (yellow arrows). Result: Patient responsed with Levofloxacin treatment. Repeat brain imaging one week following revealed resolution of splenial lesion of the corpus callosum with complete neurologic recovery. Conclusion: No specific treatment is recommended for MERS besides indicated treatment for the underlying infectious disorder. Clinicians should be aware of this disorder and include it in their differential of encephalopathy, especially in tropical regions with various infectious diseases.

Award ID: 713

A Rare Case of Central Pontine Myelinoysis Associated With Hypokalemia and Hyperparathyroidism

Vishnu M S, Balasubramanian S, Lakshminarasimhan Ranganathan

Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: vishnumsath@gmail.com

Background and Aim: Central pontine myelinolysis (CPM) is one of the rare non-inflammatory demyelinating diseases of the pons and very rarely it can involve extrapontine structure as well. Rapid correction of hyponatremia has been attributed as a most common factor, but alcoholism, malnutrition, diuretics, psychogenic polydipsia and hypokalemia have also been attributed. Methodology: 22 year old female, P1 L1, with hyperemesis gravidarum in present pregnancy and spontaneous expulsion of fetus at 16 wks and no previous comorbidities, presented with disorientation, memory impairment, bilateral gaze evoked nystagmus, scanning dysarthria, incoordination of upper and lower limbs, truncal and stance ataxia, hypotonia and weakness of all four limbs. She had a history of hypokalemia (S. K - 2.1) which was corrected in another hospital. She was found to have hypercalcemia (S. Calcium – 13.3) and primary hyperparathyroidism (S. PTH – 255), with no hyponatremia, with MRI Brain showing central pontine myelinolysis and Parathyroid scintigraphy showed enlargement of parathyroid glands. Patient was treated with IV dexamethasone, IV thiamine and IV Zolendronic acid, with improvement of symptoms and normalization of calcium levels. She underwent subtotal parathyroidectomy and histopathology was suggestive of parathyroid adenoma and hyperplasia. Result: Most CPM cases have been reported in association with rapid correction or overcorrection of hyponatremia. Association of CPM with hypokalemia has been reported in patients of anorexia nervosa after starting feeding in them. Conclusion: Our patient had hypokalemia, following correction of which she presented with CPM and found to have hypercalcemia due to primary hyperparathyroidism, which is a rare presentation.

Award ID: 714

Mimicker of Cavernous Sinus Syndrome

Rajagembeeran Venugopal, Lakshmi narashiman R, Shunmugasundaram K, Manickavasagam J, Vinoth Khanna V

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: rajagembeeran01@gmail.com

Background and Aim: Subdural empyema is intracranial infection located between the dura mater and the arachnoid mater. It has a tendency to spread rapidly through the subdural space until limited by specific boundaries (eg, falx cerebri, tentorium cerebelli, base of the brain, foramen magnum). Methodology: 45 year female without any comorbities presented to our department on March 2022, with chief complaints of headache, drooping of left eyelid, double vision and decreased sensation over left periorbital region for last one month without diurnal variation. On examination, patient had left side complete 3rd nerve palsy, left IV nerve palsy and sensory impairment in V1 distribution also noted. Her basic investigation including CSF study are all normal. MRI Brain (plain +contrast) showed cisternal segment left third nerve thickening and left subdural abscess in tentorium cerebelli. She was started on inj. Vancomycin and ceftriaxone along with dexamethasone. Her symptoms stared improving Result: Subdural empyema usually presents with signs and symptoms of infection such as fever, headache, vomiting, focal neurological deficits, and mental status changes. Rarely palsies of cranial nerves III, IV, or V seen if the abscess is near a petrous portion of the temporal bone. At the free margin tentorium cerebelli cranial nerves III, IV and V pass through toward the lateral wall of the cavernous sinus. This explains the clinical correlation of our patient Conclusion: This unusual case of acute tentorial cerebelli subdural abscess involving III, IV, V1 suggests the need for increased clinician awareness of the different ways that subdural empyema present

Award ID: 715

A Case of Rapidly Progressive Encephalopathy with Demyelinating Neuropathy

Parnika Nangla, Bhavik Bansal, Jerry A. George, Megha Brijwal, Mehar C. Sharma, Vaishali Suri, Manjari Tripathi, Deepti Vibha, Rajesh Kumar Singh, Saumya Saha, Arunmozhimaran Elavarasi

Undergraduate, All India Institute of Medical Sciences, New Delhi, India

E-mail: parnikanangla@gmail.com

Background and Aim: Rapidly progressive encephalopathy in a young adult has a range of differentials requiring an exhaustive workup. We present a man with a history of encephalopathy with neuropathy and a rare diagnosis. Methodology: A 40 year-old man presented with a 6-month history of cognitive dysfunction and an asymmetric gradually progressing ascending quadriparesis with the involvement progressing in the order of motor, sensory, autonomic and bulbar. MRI of the brain showed diffuse atrophy. Nerve conduction studies revealed sensorimotor neuropathy affecting all four limbs. CSF showed a negative autoimmune panel and no signs of an infectious etiology. The patient was started on plasma exchange with a provisional diagnosis of an immune mediated disorder. Result: A nerve biopsy showed axonolysis and demyelination. EEG showed delta wave slowing and periodic discharges. A possibility of SSPE was considered, although peripheral neuropathy is an atypical presenting feature, following which the measles IgG antibodies titers in CSF and serum were found to be raised at 377.2 U/mL and 197 U/mL respectively. He had persistent sepsis and several episodes of respiratory distress requiring invasive mechanical ventilation. He was started on intrathecal interferon with a period of objective stability after which the sensorium gradually deteriorated and the outcome was fatal. Conclusion: Subacute sclerosing panencephalitis (SSPE) is a progressive disorder caused by a persistent defective measles virus and causes death several years after the measles infection. It is suspected based on characteristic clinical features, EEG findings and demonstration of measles antibodies in CSF. The diagnosis is made based on Dyken’s criteria. (1)

Award ID: 716

Comparative evaluation of Clinical, Electrophysiological and Ultrasonographic parametersin diagnosing and assessing severity of Carpal Tunnel Syndrome- A Study from a Tertiary CareCentre in North India

Khushboo Gyanchandani, Neera Chaudhry

Neurology, VMMC and Safdarjung Hospital, New Delhi, India

E-mail: khushboogyan619@gmail.com

Background and Aim: To investigate the diagnostic value of different electrophysiological and ultrasonographic parameters in Carpal Tunnel Syndrome (CTS) patients and to assess a correlation between clinical, electrophysiological and ultrasonographic abnormalities. Methodology: Sixty-two symptomatic hands and sixty-two asymptomatic hands were included. Historical-objective (Hb-Ob) scale was used to assess clinical severity. Nerve Conduction Studies (NCS) were done and following parameters were assessed; Distal median motor latency, distal Median Motor Latency – distal Ulnar Motor Latency difference, Median Sensory Latency– Ulnar Sensory Latency difference, Comparison of Lumbrical And Interossei Latency, Palm –wrist conduction comparison. Ultrasonography of wrist was done and following parameters were measured; Cross-sectional area (CSA) of median nerve at inlet and outlet, inlet-outlet ratio (IOR), Flattening Ratio (FR), Palmar bowing (PB). Sensitivity and specificity of all electrophysiological and ultrasonographic parameters was evaluated. Correlation between clinical severity, electrophysiological and ultrasonographic parameters was assessed. Result: Electrophysiological parameters have high degree of sensitivity and specificity in diagnosing CTS. Ultrasonographic parameters have high diagnostic value comparable to nerve conduction studies. Significant correlation was found between clinical, electrophysiological and ultrasonographic parameters. Conclusion: Ultrasonographic measurements offer comparatively high diagnostic accuracy for CTS and ultrasonographic study could be considered as a non-invasive, alternative, and complimentary diagnostic modality for the evaluation of CTS.

Award ID: 717

The Enigma of Autoimmunity: A Case of Subacute Sclerosing Panencephalitis in a Patient Diagnosed with Systemic Lupus Erythematosus

Al Inde John Pajantoy, Pia Teresa Atienza Camara-Chua

Center for Neurological Sciences, Department of Adult Neurology, Quirino Memorial Medical Center, Philippines

E-mail: alindejohnapajantoy@gmail.com

Background and Aim: To report a case of Subacute Sclerosing Panencephalitis (SSPE) with co- existing Systemic Lupus Erythematosus (SLE); propose the mechanism of their causation and association of their pathogenesis. Methodology: Detailed description of the clinical presentation and review of literature. Result: A- 19- year old female had multiple hospital admissions in our institution due to recurrent seizure episodes associated with progressive decline in cognitive, motor, and language functions. The patient satisfied two major and one minor Dyken’s criteria which included elevated anti- measles antibody titers in the Cerebrospinal fluid, a typical chronic evolution of neurologic manifestations, and a characteristic occurrence of periodic complexes in the electroencephalographic findings. A co- existing Systemic Lupus Erythematosus by clinical and serologic examinations was also noted as shown by positive ANA and Anti DsDNA. In this case report, the contribution of measles in the development of SLE is elucidated and whether SLE and therapeutic immunosuppression has led to the reactivation of measles leading to SSPE. To date, this is the first documented case of SSPE with concomitant SLE recorded worldwide. Conclusion: SSPE may be due to the down-regulation of host- immune responses that resulted to incomplete measles viral clearance. The persistence of immune complexes facilitated autoimmunity and the potential development of SLE. This led to the downregulation of T- cell responses and accelerated loss of antibodies formed against measles virus hence, the evolution to SSPE.

Award ID: 718

Primary Spinal Cord Hemorrhage During COVID19 Illness: A Novel Addition to the Endless Spectrum of Neurological Manifestations?

Mehul, Nirmal Surya

Neurology, Bombay Hospital

E-mail: drmehuldesai07@gmail.com

Background and Aim: Since its discovery in December 2019 severe acute respiratory syndrome coronavirus-2 (SARS-COV2), coronavirus disease-19 (COVID19) pandemic has opened up pandora’s box and put the entire health-care industry globally under trial by fire. Spinal cord involvement in COVID19 although very well documented, overall remains uncommon. Here we present an interesting case of spinal cord hemorrhage in a patient with COVID19 positive status, first of its kind to our knowledge in this ever so expanding spectrum of nervous system manifestations of SARS-COV2. Methodology: An elderly lady suffering from COVID19 illness developed acute onset paraplegia on day-3 of illness. Initial MRI revealed spinal cord intramedullary hemorrhage and raised possibility of ?Dural A-V fistula. DSA done to evaluate same did not reveal any abnormalities hence follow up MRI was performed which showed resolving cord edema and hemorrhage from D9-12 levels. She had received therapeutic dose of LMWH for 3 days and her PT/INR was 1.5 and d-dimer levels were 3600 ng/ml. Although spinal cord involvement in COVID19 illness is well-known, primary spinal cord hemorrhage is not yet documented in literature. Result: Both thrombotic and hemorrhagic complications are well documented during COVID19 illness and represent underlying coagulopathy state. Intracerebral, intraperitoneal and adrenal hemorrhage, all are reported in various case reports and case series. However here we present a unique case of primary spinal cord hemorrhage during COVID19 illness. Conclusion: As such unusual complications may have catastrophic prognosticating implications on patient’s overall quality of life, we would like to emphasize the judicious use of anti-coagulation therapy in only absolutely indicated cases to prevent occurrence of such cases in future.

Award ID: 719

Prevalence of Stroke and Intracranial Involvement in Mucormycosis Patients with Covid-19 Infection in India

Awadh Kishor Pandit, M. V. Padma, A. K. Srivastava, Shubham Misra, M. V. P. Srivastava, V. V.Y., R. Ranjan, R. K. Singh, D. M.R., A. Das, A. Agarwal, Poorvi Tangri

Department of Neurology, All India Institute of Medical Sciences, New Delhi, India

E-mail: akpandit.med@gmail.com

Background and Aim: India during the second wave of COVID19 faced a rapid and sudden surge of not only COVID19-delta variant cases but also mucormycosis making the infection even more fatal. We conducted a study to determine factors associated with occurrence of mucormycosis in patients with COVID-19 Methodology: This case-control study comprised of 121 patients; 61 were cases (mucormycosis with COVID-19) and 60 controls. Patients were included from April 10, 2021 onwards. Follow-up was conducted after about 90 days and health-status were recorded on basis of modified-Rankin-Scale (mRS) Result: Mucormycosis with COVID-19 cases had median (IQR) age 49 (43-59) years with 65.6% males and were older (95%CI1.015-1.075; p = 0.002) than in the control group with median (IQR) 38 (29-55.5) years and 66.6% males. Baseline raised serum creatinine (OR = 4.963; 95%CI1.456-16.911; p = 0.010) and D-dimer (OR = 1.000; 95%CI 1.000-1.001; p = 0.028) were independently associated with occurrence of mucormycosis in COVID-19 patients, also Diabetes Mellitus (OR = 26.919;95%CI 1.666-434.892; p = 0.020) was associated with poor outcome and increased mortality in patients with mucormycosis with COVID-19 as per multivariable analysis. 30/61 mucormycosis patients had intracranial involvement. Intracranial involvement was observed either in the form of infarct (33.33%), haemorrhage (6.66%), meningitis (6.66%), abscess (10%) or thrombosis (26.66%). Conclusion: The study concludes that baseline raised creatinine and D-dimer may be associated with the occurrence of mucormycosis in COVID-19 patients. However, future studies may be conducted to establish this cause-effect relationship.

Award ID: 720

Teenager with Ophthalmoparesis and Dysphagia

Kamalesh Tayade, Sohini Chakraborty, Manjari Tripathi, Deepti Vibha, Rajesh Kumar Singh, Arunmozhimaran Elavarasi

Neurology, All India Institute of Medical Science, New Delhi, India

E-mail: drkamalesh21@gmail.com

Background and Aim: Pharyngeal-cervical-brachial (PCB) variant is the rare presentation of Guillain-Barre syndrome (GBS), and there is a handful of case reports of overlap of PCB variant with Miller-Fisher (MFS) variant of GBS. This overlap produces varied symptoms that may be confusing and challenging for physicians to diagnose timely and start appropriate treatment. Methodology: A 18 yrs boy, presented with a three-days history of acute onset, a progressive bulbar symptom followed by bilateral ptosis and diplopia followed by an imbalance while walking. A week prior to this, the patient had fever with a dry cough. Patient had bilateral ptosis, restricted extraocular movements, jaw weakness, bifacial weakness, absent cough and gag reflex. No limb weakness. There was generalized hyporeflexia, limb and gait ataxia. Result: His ice pack test, neostigmine test and acetylcholine receptor antibody all were negative. Nerve conduction studies, Repetitive nerve stimulation (RNST), CSF and MRI brain were normal. The patient was treated with high-dose methylprednisolone and five cycles of plasma exchange following which patient improved completely. Conclusion: MFS manifests as acute onset ophthalmoplegia, ataxia and areflexia. Clinical features of the PCB variant of GBS include acute weakness of the oropharyngeal, neck and shoulder muscles with swallowing dysfunction. Our patient had overlapping clinical features between MFS and PCB variant of GBS. In case series608 of 100 patients of PCB, 26 patients showed overlap with MFS. Symptoms of PCB and MFS overlap syndrome may confuse with symptoms of botulism, myasthenia gravis, diphtheria, Wernicke’s encephalopathy, brainstem stroke and neuro-Behcet’s, so careful history and examination is required for ruling these out.

Award ID: 721

A Case of Sub Acute Pancerebellar Ataxia - Anti YO Positive Pan Cerebellar Degeneration

Abbigari Vardhan

Neurology, Madurai Medical College, Madurai, Tamil Nadu, India

E-mail: harsha.abbigari3@gmail.com

Background and Aim: Para neoplastic cerebellar degeneration (PCD) is a rare non metastatic neurological complication in cancer patients. Anti YO is one of the anti –onconeural antibodies found in PCD patients. It is believed that Anti YO occurs almost always in women and his most likely associated with gynaecological or breast cancers, although exceptions exist. Methodology: A 52 year old female with out any co morbidities presented with sub acute onset, gradually progressing neurogical illness, characterized by imabalance while walking, had a tendency to sways to both sides while walking, feels imbalance while sitting, incordination in both upper limbs, speech abnormality in the form of slurring with increased pauses between words and syllables especially longer words. No history of sensory, cognitive behavior abnormalities, cranial nerve dysfunction or weakness of limbs. No positive etilogical history for ataxia. On examination Nystagmus, hypometric saccades and broken pursuits are present. Bilateral cerebellar signs present, gait is wide based and ataxic. Result: Routine blood investigations with in normal limits. ANA profile, auto immune profile, viral markers negative, serum TSH is normal. para neoplastic pannel strongly Anti YO positive. MRI brain with whole spine screening is normal, NCS normal study. PET CT highly metabolic nodule present in lower quadrant of right breast. Conclusion: Anti YO antibody –positive PCD usually presents as symptoms of subacute cerebellar degeneration. The main clinical manifestation is cerebellar ataxia of trunk and limbs and speech abnormality, which last for weeks to months.

Award ID: 722

Paraneoplastic Neurological Disease Manifesting as Spinal Segmental Myoclonus

Laxmi Khanna, Ankkita Sharma Bhandari, Mandaville Gourie-Devi

Neurophysiology, Sir Ganga Ram Hospital, Delhi, India

E-mail: laxmikhanna@hotmail.com

Background and Aim: BACKGROUND: Anti-CRMP5 antibodies cause central and peripheral neurological diseases as a consequence of autoantibody mediated neuronal damage and a T-cell mediated immune response. OBJECTIVE: Paraneoplastic Neurological Diseases are clinically challenging with a wide spectrum of manifestations. We came across an unusual case of painful myoclonus involving the proximal muscles of both shoulders caused by antiCRMP5 mediated neuronal injury. Methodology: METHODS: Besides clinical examination, serum and cerebrospinal fluid autoimmune antibody assay, electroencephalogram, electromyogram, nerve conduction studies, Magnetic Resonance, FDG-PET- CT scans and tissue biopsy were used to confirm the diagnosis. Result: RESULTS: CRMP5 antibodies were detected in the cerebrospinal fluid and FDG PET-CT scans revealed mediastinal lymphadenopathy. Ultrasound guided endoscopic bronchoscopy revealed a small cell carcinoma of the lung. Chemoradiation, immunotherapy and symptomatic therapy with Tab Levetericetam and Tab Clonazepam gave symptomatic relief. The myoclonic jerks subsided after the first course of chemotherapy. Conclusion: CONCLUSION: Anti-CRMP5 mediated antibodies affect the nervous system and present with limbic encephalitis, encephalomyelitis, myasthenia gravis, cerebellar ataxia, Parkinson’s disease, peripheral neuropathy and Spinal Segmental Myoclonus involving the proximal muscles of both upper arms. As anti-CRMP 5 mediated neurological damage is rare, we considered reporting this case. This patient responded to chemoradiation with immunotherapy and supportive treatment.

Award ID: 723

A Case of Spinal AV Malformation AMD Klippel Trenauney Syndrome

Swati Sharma, Pettarusp Wadia, Shaji Marar

Neurology, Jaslok Hospital, Mumbai, Maharashtra, India

E-mail: swati.mdmedicine@gmail.com

Background and Aim: Klippel-Trenaunay-Weber syndrome (KTWS) is characterized by a triad of port-hwine stain, varicose veins, and bony and soft tissue hypertrophy involving an extremity. Clinical presentations of spinal cord AVMs can involve either progressive myelopathy or acute hemorrhage (subarachnoid or intramedullary) Very few cases of spinal arteriovenous malformations with Klippel Trenauney Symdrome have been described. We present a case of a 24 year old male, diagnosed as KTS, who presented with gait difficulty, diagnosed to have a spinal AV malformation, that was embolised. Methodology: A 24 year male, diagnosed as KTS since age of 9 years, presented with difficulty in walking and imbalance since 4 months. He had dragging of right leg, with massive lower limb edema, macrocephaly, asymmetric facial hypertrophy, cutaneous heamangiomas and organomegaly. We did an MRI Spine and DSA at JHRC. Result: MRI Spine revealed T2 hypointense flow voids in epidural space from D2-D5 levels suggestive of AVM, with compressive myelopathy. DSA confirmed presence of AVM, which was embolised. The patient showed improvement in his gait after the procedure. Conclusion: Patients with KTS should be screened for underlying spinal AVMs. Intramedullary spinal AVMs have been reported as most commonly associated with KTWS. Close differentials include Parkes-Weber, Cobbs and Cloves syndrome. Embolisation of AVM via DSA can lead to symptomatic improvement in gait difficulties.

Award ID: 724

Belly Dancer Syndrome due to Cortical Myoclonus. A Rare Cause

Sandhya Manorenj, Bushra Naaz

Neurology, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Background: Belly dancer’s syndrome (BDS) or diaphragmatic flutter refers to myoclonic jerks involving one or both hemidiaphragms. BDS is an extremely rare phenomenon characterised by involuntary repetitive often rhythmic contraction of the diaphragm either unilateral or bilateral, causing undulating movements of the abdomen resembling a belly dancer It is usually accompanied with discomfort in the upper abdomen or lower chest, but clinical symptoms are highly variable often resulting in late diagnosis. Aetiology is usually segmental myoclonus, rarely a central cause. Aim: Here we describe a rare case of BDS. Methodology: Methods: Descriptive case report study Result: Results: 21 year-old girl who presented with acute onset abdominal quiver of 5 days duration following febrile illness. Clinical examination showed abdominal jerky movements with preserved awareness. These abdominal movements were rhythmic, continuous, undulating movements of the abdomen and lower trunk markedly noted while lying [Video 1]. The movements persisted on manoeuvres like deep inspiration and breath-holding. She could not voluntarily control this abdominal movements. MRI brain and spine was normal. Electroencephalogram (EEG) showed interictal polyspike wave discharges from bilateral frontocentral region with adjacent spread consistent with cortical myoclonus. These abdominal myoclonic jerks disappeared dramatically on treatment with Lacosamide, a third generation antiepileptic drug. Conclusion: Conclusion: BDS can be due to cortical myoclonus origin instead of the widely described segmental myoclonus form. Clinician should be aware of this rare phenomenon with multiple clinical presentation, as usually BDS is misdiagnosed. Present case EEG helped to confirm the diagnosis and appropriate treatment

Award ID: 725

Protean Manifestations of IgG4 Disease

Swati Sharma, Joy Desai, Azad Irani, Pettarsup Wadia, Reshik Kannan Korakkaran, Aditya Aundhkar

Neurology, Jaslok Hospital, Mumbai, Maharashtra, India

E-mail: swati.mdmedicine@gmail.com

Background and Aim: IgG4-related disease (IgG4-RD) is an immune-mediated, chronic, fibro-inflammatory condition that causes tumefactive lesions with characteristic histopathological features. We present two cases of IgG4 related disease with different CNS manifestations. Methodology: One 66 year female (case A) presented with chronic occipital headcahe, vomiting, one seizure and behavioural changes, Another 57 year female (case B) presented with frontal headache and orbital pain. We did imaging, CSF and serum investigations. Result: Conclusion: Isolated central nervous system (CNS) involvement in IgG4 related diasease, though rare, has been described in the literature. Diagnostic imaging, with clinical, laboratory and histopathological data, provides a fundamental diagnostic tool in the diagnosis of this entity. The first-line treatment currently consists of steroid therapy. Rituximab, azathioprine and mycofenolate mofetil can be used as second line therapy.

Award ID: 726

The Panoramic Manifestations of Neurosjogren: A Retrospective Case Series

Sohini Chakraborty, Baikuntha Panigrahi

Neurology, AIIMS Delhi, India

E-mail: sohinichakraborty84@gmail.com

Background and Aim: Neurological manifestations occur in 8.5-70% of those with pSS (Primary Sjogren’s syndrome) 1In 25-60% neurological symptoms precede diagnosis of pSS by 2 years, whereas in the rest appeared 6-8 years post pSS diagnosis 2 Our aim is to demonstrate the Panoramic neurological manifestations studied in two pSS patients in our center. Methodology: Patient 1: Mr. K, 38 years old gentleman, presented with 2 episodes of pure motor acute flaccid Quadriparesis (time to peak of 2-4 hrs and time to resolution of 10-12 hrs) 30 years back followed by insidious onset gradually progressive paresthesias over bilateral hand and feet, sensory ataxia since last 20 years and right foot drop since last 7 years. On examination left ulnar and bilateral common peroneal nerve were thickened associated with wasting of bilateral intrinsic muscles of the hand, right foot drop and graded sensory loss over bilateral hand and feet. ANA, anti-SSA, Schirmer’s, salivary gland Scintigraphy were positive along with NCS showing demyelinating Sensorimotor polyneuropathy of all 4 limbs. Patient 2: Miss N, 19 years old female, presented with inflammatory arthritis, oral ulcers, alopecia, CIDP, vertical nystagmus and paresthesias. On examination there was bilateral foot drop, generalised atrophy. Schirmer’s, salivary Scintigraphy, RA, ANA, U1RNP and anti Ro were positive. Result: Both of our patients qualified as pSS according to 2016 ACR/EULAR CLASSIFICATION CRITERIA. They were started on pulse steroid with monthly cyclophosphamide and showed considerable improvement. Conclusion: Neurological involvement in pSS is a negative prognostic factor, where more intensive treatment is indicated. Sensory polyneuropathy is the most common complication3CNS involvement is in 2-25%4

Award ID: 727

Studies on Selected Mitochondrial Gene Expression in the Peripheral Blood Mononuclear Cells of SCA 12 Patients

Sabbir Ansari, Jyoti Rungta, Rakhi Pal, Supriyo Choudhury, Rebecca Banerjee, Sanjit Dey, Hrishikesh Kumar

Department of Neurology, Institute of Neuroscience, Kolkata, West Bengal, India

E-mail: sabbirhzb@gmail.com

Background and Aim: Spinocerebellar ataxia type 12 (SCA12) is an autosomal dominant neurodegenerative disorder attributed to CAG repeat expansion mutation in a 5’ UTR of the ppp2r2b gene; encodes regulatory subunits of protein phosphatase 2A. Mitochondrial dysfunction has been reported in most neurodegenerative diseases including SCA12. Therefore, we chose key mitochondrial genes such as ndufs5, tim22, fis1, dnm1l to understand alterations in mitochondrial functioning in peripheral blood mononuclear cells (PBMCs) from SCA12 patients. Methodology: Ten genetically confirmed SCA12 patients and ten healthy controls were recruited prospectively following institutional ethical clearances and informed consent. Functional impairment, cognitive function, and depression were assessed using the International Co-operative Ataxia Rating Scale (ICARS), Montreal Cognitive Assessment (MOCA), and Beck’s Depression Inventory (BDI). PBMCs were isolated using density-gradient separation techniques. Gene expression profile of ndufs5, tim22, fis1, dnm1l and gapdh was performed using ABI7500. Result: The mean age of the SCA12 patients was 62.5 (±9.19) and 46.9 (±10.33) years for controls. The scores of the ICARS, MOCA and BDI scales for the patients were 33.4 (±20.97), 22.0 (±6.76), and 9.88 (±6.5) respectively and for the healthy, the value was within the normal range. The mean CAG repeat length was 55.55 (±6.34). The mitochondrial genes i.e. ndufs5, tim22, fis1 and dnm1l show similar expression between the SCA12 patients and the healthy controls. Conclusion: The interim analysis shows that the mentioned mitochondrial gene has no role in the disease pathology or maybe the differential expression occurring at the protein level or in other cell types.

Award ID: 728

Spectrum of Neurological Adverse Events Following COVID-19 Vaccination: An Observational Study from a Tertiary Care Centre of Eastern India

Uddalak Chakraborty, Soumita Sadhukhan, Arijit Roy, Souvik Dubey, Saumen Bhat, Rajdeep Saha, Sujoy Kabiraj, Aishee Bhattacharyya, Ajitava Dutta, Shambaditya Das, Dwaipayan Bhattacharyya, Arindam Santra, Manasi Das, Biman Kanti Ray, Alak Pandit, Gautam Gangopadhyay, Atanu Biswas, Tejendra Nath Kundu, Adreesh Mukherjee, Samar Biswas, Gautam Das, Jayanta Ghosal, Sumanta Sarkar, Uma Sinha Roy

Neurology, Bangur Institute of Neurosciences, IPGMER & SSKM Hospital Annex-1, West Bengal, India

E-mail: uddaalakchakraborty@gmail.com

Background and Aim: As the world tends to recover from the social, economic and health crisis created by the COVID-19 pandemic, vaccines have served as one of the most valuable tools of combat and large-scale immunization has helped to tide over the catastrophe. However, several reports of post vaccination minor to severe neurological complications around the globe have surfaced up in recent literature. Methodology: A descriptive observational study was carried out in our institute from March 2021 to February 2022 including 25 patients who were admitted with a new onset neurological illness within 4 days to 42 days of vaccination. Result: The mean age of the population was 36.64 years and the mean latency of onset of neurological events was 14.32 days. GB syndrome was documented in 11 patients; majority were AMAN (45.45%) followed by AIDP (36.36%) and AMSAN (18.18%). CNS demyelination was reported in 9 patients; two patients had ADEM, 2 patients were diagnosed with MOGAD, one patient with NMOSD, and the rest were labelled as seronegative CNS demyelination. Vascular events were noted in 5 patients comprising of 2 cases each of CVT and ICH and a case of Moyamoya angiopathy. There was antecedent history of Covishield vaccination in all patients barring one presenting with AMSAN, who received Covaxin. All patients were conservatively managed and had a complete to partial recovery suggestive of a favorable outcome after 3 months. Conclusion: The pathophysiology of post vaccination neurological complications is still elusive and any causal association attributable to vaccination warrants further research. Compared to the enormity of the vaccinated population, the adverse effects are miniscule hence any misconception to hinder vaccination shall be strongly discouraged.

Award ID: 729

Mosquito Born Alpha Virus Infection a New Cause of Stroke in Adult: Chikungunya Related Cerebral Vasculitis

Sandhya Manorenj

Neurology, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Background: Infections have been associated with stroke risk and are often neglected. Viral infectious vasculitis is a rare cause of stroke. Varicella zoster virus, Epstein barr virus, Herpes simplex virus and Zika virus are known to cause cerebral vasculitis and stroke. Stroke is less frequently described in Chikungunya infection. Neurological syndrome frequently described in chikungunya infection are encephalitis, myelitis, encephalomyelopathy, neuropathy, guillain barre syndrome, encephalomyeloneuropathy, acute disseminated encephalomyelitis and neuro-occular diseases.. Literature showed combination of Zika virus and chikungunya linked to stroke. Aim: Here author describe a case presenting with acute chikungunya infection that was associated with stroke Methodology: Methods: A case report describing clinical and radiological features of chikungunya associated vasculitis and Ischemic stroke. Result: Case report: 45 year old woman without any co morbidities presented with three days of high grade fever with chills, facial flushing, polyathralgia and one day history of acute left hemiparesis. MRI brain showed acute infarct in right corona radiate region with angiogram showing narrowing of right MCA M3 branch with vasculitis features. Serological test, confirmed the presence of IgM and IgG anti-chikungunya antibodies. Routine stroke works up tests (2D Echo, fasting lipids, and Doppler neck vessel) were negative. Patient was treated with parenteral steroids, and antiplatelets. She. She recovered completely by 2 weeks Conclusion: Conclusions: Stroke following chikungunya infection is rare. Cerebral vasculitis in chikungunya should be considered as one possible mechanism of Ischemic stroke in chikungunya infection and should be evaluated and treated. Tropism of virus to cerebral vessel endothelia could have lead to stroke

Award ID: 730

An Interesting Case of Unilateral Isolated Total 3rd Nerve Palsy

J. B. Agadi, Sharath G G

Neurology, Apollo Hospital Sheshadripuram, Bengalure, Karnataka, India

E-mail: drjbagadi@gmail.com

Background and Aim: Extra axial compression of the 3rd nerve presents with total third nerve palsy and the classical teaching is that one should rule out aneurysm of the posterior communicating artery by DSA or a tumour pressing on the third nerve. Patients with acute stroke presenting with primarily visual complaints can often be misdiagnosed and inappropriately triaged. Prompt recognition is critical due to the time-sensitive nature of potential acute stroke therapy, such as intravenous thrombolytics or thrombectomy. Utilisation of advanced neurovascular imaging has to be more deliberate. Methodology: We describe about a patient with painful 3rd nerve palsy on the right side. The history, clinical features, MRI findings and findings of CT angio and management will be discussed. WE will also highlight the vascular supply of the third nerve. Result: MRI scan coronal section of the brain with a b value of 2000 demonstrated ischemia of the right 3rd nerve on the right side to account for the total 3rd nerve palsy and it also showed acute infarct in the watershed territory of the right frontal region. CT angiography of Head and neck showed a floating thrombus in the cavernous segment of the right internal carotid artery. Conclusion: Relevant neuro imaging played a crucial part in managing this case. 3rd nerve is supplied by meningohypophyseal artery and inferior cavernous sinus artery both of which are branches of internal carotid artery. Timely CT angio dictated the management of this case. To our knowledge such case reports are rarely found in the literature.

Award ID: 731

Burden of Depression in Epilepsy: An Observational Study in a Tertiary Care Centre

Meera Radhakrishnan, Nandakumar Vasudevan, Nithyanandam A, Thamilpavai N, Lakshminarasimhan Ranganathan

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: meerasusanth@gmail.com

Background and Aim: Depression is the most frequent type of psychiatric disorder encountered in patients with epilepsy. Screening of patients presenting to the epilepsy clinic for depression is essential for early identification and timely intervention Methodology: The study included patients aged more than 18 years, admitted to the Neurology ward between September 2020 and February 2021 and those attending OPD for follow up visits. A total of 100 consecutive patients with epilepsy were screened and 60 patients were included in the study based on the inclusion criteria. It was an observational study Result: 60 patients were enrolled. 46 subjects had depression, of which females (65%) constituted the majority. Majority were on more than two antiepileptic drugs. Severe depression was noted in patients using more than one group of antiepileptics. Multivariate regression analysis was done and a significant association was found only between depression and seizure frequency and depression and the use of multiple antiepileptics, with a p value of 0.029 and 0.004 respectively Conclusion: A routine screening for depression is mandatory for patients on multiple antiepileptics and with poor seizure control. Association between seizure control and depression suggests need for adequate control of epilepsy. Use of multiple AEDs and the significant association with depression in our study suggests the need for further studies on depression and interactions between multiple AEDs, when used in combination for seizure control

Award ID: 732

Unusual Chimera- MS and MOG

Manickavasagam Janarthanam, Lakshmi Narasimhan, Shanmugasundaram K, Vinoth Kannan, Kaushik S

Neurology, MMC

E-mail: drjmanicks@gmail.com

Background and Aim: •24 year female •History: Presented with complaints of numbness of left perioral region, numbness over both feet, reduced flow of urine since 2 weeks since 2 weeks. Numbness of perianal region since 1 week. Methodology: •Past H/o: a) 1st episode- Numbness below chest region 1 year ago, spontaneously resolved 1 week later. b) 2nd episode- Blurring of vision in right eye since 10 months age, resolved on methylprednisolone. Result: •O/E: Visual acuity- Normal Colour vision- Normal •Motor system Normal •Sensory: Pain, touch reduced in both soles of feet and perianal region (R > L) Anal wink- absent, Anal tone- Reduced•CSF OCB- 4-10 bands (October 2021) •Aquaporin- Negative •MOG- Positive Conclusion: Discussion:•Typically MS cases have negative MOG antibody. •Case reports of MOG antibody positive MS have been reported. •They have atypical features such as: a) bilateral optic neuritis b) longitudinally extensive transverse myelitis c) moderate pleocytosis

Award ID: 733

Atypical Skull Base Osteomyelitis of Tubercular Etiology

Nandakumar Vasudevan, Thamilpavai Arulnambi, Lakshmi Narasimman Ranganathan

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: vnkneuro@gmail.com

Background and Aim: This is a case report intended to highlight an unusual cause of skull base osteomyelitis caused by Tuberculosis spreading superiorly from a retropharyngeal abscess Methodology: This is a case report of a patient with progressive subacute and sequential involvement of multiple cranial nerves Result: 62-year-old male presented with Headache localized to the left side of the face involving the forehead initially, dull aching, continuous, a/w numbness of face which began from the forehead and then progressed to involve the cheek and chin. 4 days later, he developed restricted eye movements over the left side. This was associated with diminished vision in his left eye. It was Progressive in nature. Blurring of vision more for near objects compared to distant objects. No diurnal variations. Protrusion of left eye is present which is progressive. Conjunctival congestion was present. No h/o diplopia. No color vision defect. 5 days later, he developed deviation of angle of mouth to right side. 5 days later, he developed deviation of angle of mouth to right side. General Examination revealed Enlarged cervical lymph nodes- firm, discrete mobile, largest measuring 2*3 cm. Neurological examination revealed left 7th, 9th, 10th, 5th, 3^rd and 6th cranial nerve palsy. MRI brain with contrast showed clival enhancement with thin retropharyngeal abscess. Multiple necrotic lymph nodes were also noted. He was initiated on ATT and was followed up. He showed partial improvement in symptoms on follow up. Conclusion: Tuberculosis can cause atypical skull base osteomyelitis through a retropharyngeal abscess spreading superiorly to involve clivus. It may also involve the pterygoid plexus of veins and cause cavernous sinus involvement causing bilateral cavernous sinus involvement

Award ID: 735

Secondary NPH as a Sequela of Cryptococcal Meningitis

Sampurna Chowdhury, Elavarasi Arunmozhimaran, Kokkula Praneeth, Saranya B. Gomathy, Ajay Garg, Manjari Tripathi, Deepti Vibha, Animesh Das, Rajesh Kumar Singh

Neurology, AIIMS

E-mail: sampurna21@icloud.com

Background and Aim: The triad of Gait impairment, urinary incontinence and cognitive impairment is associated with Normal pressure hydrocephalus, which may be secondary to diseases of the subarachnoid space. Secondary NPH may be due to various causes of acquired hydrocephalus like SAH, Head trauma, intracranial malignancies, chronic meningitis and cerebrovascular disease. Methodology: Here we report a case of cryptococcal meningitis presenting as secondary normal pressure hydrocephalus (NPH) in an immune-competent female. A 60 year old immunocompetent female was diagnosed with cryptococcal meningitis based on clinical symptoms, imaging and CSF findings including (Increased protein with lymphocytic pleocytosis and low glucose with fungal culture and Cryptococcal antigen test positive) 4 years back and received a 2-week course of antifungal therapy with which there was improvement in sensorium. Over the next 3 years, she developed slowness of gait, increased frequency of urination and cognitive impairment with bradyphrenia. CT head was suggestive of ventriculomegaly disproportionate to the degree of cortical atrophy. CSF tap test was done which showed significant improvement in gait and urinary symptoms. Result: Following non-programmable VP shunt insertion patient showed considerable improvement in her speed of walking and urinary symptoms. Bradyphrenia also improved. Conclusion: We share our experience in managing a sequlae of Cryptococcal meningitis in an immunocompetent individual. Secondary NPH can occur years after the insult even in successfully treated immunocompetent individuals and highlights the need to promptly use VP shunt to prevent further cognitive decline.

Award ID: 736

An Interesting Case of Stroke – Common Presentation of a Rare Disorder

Krishnakumar Balaraman, Aravind Gautham, Chandramouleeswaran V, Lakshmi Narasimhan R

Institute of Neurology, Madras Medical College & RGGGH

E-mail: kichazmd@gmail.com

Background and Aim: The presentation of Stroke with the involvement of multiple cranial nerves is a common clinical scenario in neurological practice. We report a case of rare disorder with common presentation Methodology: A 53 Year old male presented with C/O of sudden onset severe, continuous, bi-frontal headache for a week. Then he noticed drooping of Rt eyelid & restricted outward movement of Rt eye with horizontal & vertical diplopia. Later, he developed deviation of angle of mouth to Rt side with difficulty in closing left eye and was associated with left hemi ataxia and left hemiparesis. On the 10th day, he noticed dysphagia for both solid & liquid foods. He had difficulty in manipulating food & protruding tongue with altered sensorium Past H/o Uncontrolled diabetes, HTN and dyslipidemia. O/E: He had Bil.abduction restriction, right eye adduction restriction, Impaired VOR, spared pupils, motor weakness of 5th nerve, Left LMN facial palsy, Bil. 9,10,12 cranial nerve palsy, weakness of right trapezius & both sternocleidomastoid, Lt hemiparesis followed by right hemiparesis, no sensory, bowel and bladder dysfunction. Result: MRI Imaging revealed diffusion restriction noted in left red nucleus, bilateral pons, left cerebellar peduncle - Acute infarcts. His serum IgG4 levels were elevated (IgG4:184 mg/dl). Based on the above findings, a diagnosis of probable IgG4RD was made. Conclusion: Our patient presented with multiple cranial nerve palsies with Bilateral hemiparesis1. Multiple infarcts in pons, could indicate underlying vasculitis. There have been only a small number of cases reporting cerebral vasculitis/stroke associated with IgG4-RD2. While this disease is not entirely understood, it is important for clinicians to be aware of this unique presentation and to have a high clinical index of suspicion when presented.

Award ID: 737

AA Case of Arachnoid Cyst in Cervical Spinal Cord

Shunmuga Sundaram, lakshmi Narasimhan, Manikavasagam J, Vinoth Kannan, Jaikumar R

Neurology, MMC

E-mail: tskssundaram@rediffmail.com

Background and Aim: Arachnoid cyst are CSF filled sacs formed between parenchyma of brain/ spinal cord and Arachnoid layer of meninges. They are the most common type of brain cystsTypes : 1. Primary 2. SecondarySite : Intracranial Arachnoid cyst (middle cranial fossa, posterior cranial fossa, suprasellar region), Spinal Arachnoid cyst. Here we report a case of Arachnoid cyst in cervical cord Methodology: A 50 year old male with past history of extrapulmonary tuberculosis (pleural effusion) presented with Insidious onset gradually progressive neurological Illness of 2 year duration in the form of asymmetrical (left > right) spastic quadreparesis with reduced perception of sensations of all modalities below C5 level, with positive Lhermitte sign and UMN bladder. Spinomotor system Examination showed wasting of small muscles of both hand (left > right) power upper limb 4+/4-, lower limb 2/1, hypertonia of both lower limbs, exaggerated deep tendon reflexes and bilateral extensor plantar reflex. MRI spine showed Intradural extramedullary T1 hypointense and T2 hyperintense lesion with cord compression and thinning. Contrast revealed filling defect without contrast enhancement. Patient was taken up for surgical intervention and post decompression of the cyst power of lower limbs improved and patient walked home. Result: Finally it is a case of spinal arachnoid cyst presenting as a cervical compressive myelopathy. Post decompression patient was improved well. Arachnoid cyst is common in intracranial location and mostly asymptomatic and detected incidentally. Spinal arachnoid cyst is uncommon and most common site is dorsal spine presenting as intradural extramedullary compressive myelopathy. Herewe report this case because of rarity and early recognition & intervention gives excellent prognosis. Conclusion: We report a case of young male who presented with spastic quadriparesis along with sensory loss and bladder involvement with intact higher mental functions, cranial nerves, cerebellum and extrapyramidal system Imaging revealed intradural extramedullary lesion With posterior displacement and thinning of cord in the cervical region along intradural filling defect in contrast

Award ID: 738

Drug Adherence for Antihypertensive Medications and its Determinants Among Hypertensive Stroke Patients in a Tertiary Care Government Hospital of West Bengal

Adrija Ray, Tapobrata Guha Ray, Biman Kanti Ray, Jyotirmoy Pal, Souvik Dubey

Rotatory Intern, R G Kar Medical College and Hospital, West Bengal, India

E-mail: adrija.ray211@gmail.com

Background and Aim: Uncontrolled hypertension due to suboptimal adherence to antihypertensives is a major risk factor of stroke. There is dearth of drug adherence studies among stroke patients in Eastern India. This study aimed to identify adherence to antihypertensive medications and its determinants among admitted study subjects. Methodology: This observational, descriptive crosssectional study was conducted for 6 months among a calculated sample of 529 study participants using predesigned and pretested schedule, the metric “Proportion of days covered (PDC),” and the Morisky, Green, and Levine (MGL) Scale. Data were compiled and analyzed using SPSS software (version 20.0). Result: Adherence rates were 33.2% and 45.6% based on the MGL scale and PDC method, respectively. Higher adherence was significantly associated with increasing age, greater literacy, longer interval between diagnosis of hypertension and present symptom, increased gap between antihypertensive treatment initiation and present symptom, receiving advice on regular drug intake and prescription of the medication by a registered medical practitioner. Conclusion: Higher literacy rate, longer duration of antihypertensive treatment, accessibility to the registered medical practitioner, and proper explanation by healthcare providers regarding the necessity of regular intake of medicine are the key issues to ensure good adherence to prescribed drugs.

Award ID: 739

Therapeutic Response in Subgroup of Idiopathic Inflammatory Myopathy

Aravinda Mandalapu, Sruthi s Nair, Raja lakshmi P, Satya Kishore Chivulula

Neurology, Sree Chitra Tirunal Institute for Medical Scinces and Technology, Trivandrum, Kerala, India

E-mail: aravinda@sctimst.ac.in

Background and Aim: Background: Idiopathic inflammatory myopathies (IIM) are rare, treatable muscle disorders with a heterogeneous presentation. The newer diagnostic criteria have used combined clinical symptomatology, myositis autoantibodies, histopathology and extra muscular manifestations for classification. Aim: To study the relative frequencies of subtypes of idiopathic inflammatory myopathy based on the EULAR-ACR 2017 criteria and descriptively analyze the disease course and therapeutic response of the subgroups. Methodology: We reviewed the electronic case records of inpatients with the final confirmed diagnosis of idiopathic inflammatory myopathy for 12 years from January 2011 to January 2022, with a minimum follow-up period of 90 days. The clinical, investigational and follow up data were extracted with a structured proforma and analyzed descriptively. Result: The study cohort consisted of 55 patients with IIM. The subgroup classification was Polymyositis in 27 (49.1%), Dermatomyositis in 14 (25.5%), Overlap myositis in 7 (12.7%), Necrotizing autoimmune myositis in 4 (7.3%) and Inclusion body myositis in 3 (5.4%). Females constituted 54.5% (n = 30) in the study. The mean age at diagnosis was 39.30 (+ 17.94) years, with a mean duration of symptoms of 12.65 (+ 20.02) months, and the median follow-up duration of 38 months. 15 (27.3%) had antecedents in the form of infection or recent vaccination. Limb-girdle pattern of weakness was noticed in 89.1%(n = 49). Elevated creatinine phosphokinase was present in 92.7% (n = 51). Myositis panel was performed in 27 individuals, out of which 21 (77.7%) had positive autoantibody status. Steroids were administered in 52 (94.5%). Among them, steroid monotherapy was given in 32 (58.1%), and dual therapy with additional immunosuppressive agents in 17 (31%) and intravenous immunoglobulin or plasma exchange in 9 (16.3%). 8 individuals (14.5%) required escalation of immunotherapy.39 patients (70.9%) had objective clinical improvement on follow up by Medical Research Council (MRC) sum score. 15 (27.7%) patients had suboptimal improvement in the form of persistent disability on last follow up, and one patient expired. Polymyositis group 53.8% (n = 21) had the best outcome. Conclusion: Polymyositis was the most common subgroup in this cohort and the one with the best therapeutic outcome. Suboptimal therapeutic response was noted in more than a quarter of the patients.

Award ID: 740

A Case OF Anti-N-Methyl-D-Aspartate Receptor Encephalitis Associated With Renal Cell Carcinoma With Ectopic Acth Secretion

Ishu Goyal, Arun B. Shah, Harshvardhan S. Bhamare

Neurology, H N Reliance Foundation Hospital, Mumbai, Maharashtra, India

E-mail: drishu.goyal@gmail.com

Background and Aim: Association of Anti-N-Methyl-D-Aspartate Receptor Encephalitis (anti NMDAR) with various tumours is known. We describe a case of anti NMDAR encephalitis presenting as difficult to treat psychosis and paraparesis, diagnosed 4 years after treatment for renal cell carcinoma, associated with ectopic ACTH secretion and pancytopenia. Methodology: Presenting complaints were recorded and a detailed neurological evaluation was done. Appropriate laboratory and radiological tests were conducted for diagnosis and a multidisciplinary approach was followed. Immunotherapy and cortisol suppression therapy was given and response to treatment noted. Result: The cause of psychosis was evaluated and anti NMDAR antibodies were found in serum and CSF. It was treated with 5 cycles of plasmapheresis followed by a course of intravenous immunoglobulin. Paraparesis improved after correction of hypokalemia (serum potassium-1.7 mmol/L). He had high serum cortisol (207 mcg/dl), high ACTH (120.5 pg/ml) and positive overnight dexamethasone suppression test raising the possibility of endogenous Cushing’s Syndrome. PET scan was negative for recurrence of renal cell carcinoma but had bilateral adrenal hyperplasia and brain findings in favour of NMDAR encephalitis. He later developed pancytopenia and had CMV PCR positive. Conclusion: We describe a case of anti NMDAR encephalitis with a background of clear cell renal carcinoma, an association not previously described. Simultaneous presence of ectopic ACTH secretion causing hypercortisolemia and hypokalemia raises the possibility of some covert malignancy or paraneoplastic syndrome which triggered these pathologies.

Award ID: 741

Non- Megalencephalic Leukoencephalopathy with Subcortical Cysts

Muralidharan Kamalakannan, Sowmini P.R, Sakthi Velayutham S, Malcolm Jeyaraj K, Viveka Saravanan R, Mugundhan K

Neurology, Government Stanley Medical College Hospital, Chennai, Tamil Nadu, India

E-mail: sridhardude37@gmail.com

Background and Aim: Non- Megalencephalic leukoencephalopathy with subcortical cysts is an autosomal-recessive condition manifesting in early childhood with psychomotor retardation, seizures, spasticity and hearing loss. Normocephaly or microcephaly is seen. On imaging, it is characterized by bilateral anterior subcortical temporal lobe cysts and extensive multifocal white matter hyperintensities in a normal sized brain. We report one such rare case of Non-Megalencephalic leukoencephalopathy with subcortical cysts in a fourteen year girl. Methodology: A fourteen year girl born out of second degree consanguineous marriage with normal birth history, delayed milestones and poor scholastic performance presented to us with one episode of generalized seizures. Examination revealed poor IQ, learning disability and attention deficit and no spasticity. EEG and PTA were normal. MRI Brain showed diffuse cerebral and cerebellar atrophy, subcortical cysts in bilateral anterior temporal lobe and hypomyelination of subcortical white matter fibres. The caudate nucleus was fused with lentiform nucleus. Anterior limb and genu of internal capsule were not visualized, while posterior limb is visualized and hypomyelinated. Calvarium was normal. All these features were suggestive of non-megalencephalic leukoencephalopathy with subcortical cysts. Result: The identification of patients with this new white matter disease depends on the recognition of the characteristic MRI findings of bilateral anterior temporal lobe cystic lesions and symmetric white-matter lesions. Conclusion: Non- Megalencephalic leukoencephalopathy with subcortical cysts is an unusual entity with very fewer cases are reported. It was first described by Olivier et al. in 1998 and since then, fewer than 30 cases have been described The closest differential would be Van der Knaap disease, where megalencephaly is a cardinal feature

Award ID: 742

A Rare Case of Spastic Paraplegia Type 35 in Indian Sub-continent

Anushree Pal, Purba Basu, Hrishikesh Kumar

Neurology, Institute of Neurosciences Kolkata, West Bengal, India

E-mail: anushreepal57@gmail.com

Background and Aim: Autosomal recessive spastic paraplegia (SPG) Type 35 is a rare hereditary spastic paraplegia characterized by childhood onset limb spasticity, spastic dysarthria and cognitive decline. In India, genetically confirmed cases of SPG Type 35 is scarce in literature. We report one such case. Methodology: We present the case of a 22 yrs old male, absolutely healthy till 5 yrs of age with normal developmental milestones. After 5 yrs, he started having progressive gait difficulty with imbalance, followed by stiffness of limbs and slurring of speech. He had episodes of focal seizure involving the right side of his body with impaired consciousness and postictal Todd’s palsy. On examination, spasticity and hyperreflexia were elicited. Bilateral cerebellar signs were also present. On investigation, Spinocerebellar ataxia panel was negative. Testing for Friedreich’s Ataxia, VLCFA estimation, and Anti gliadin IGA was negative. Anti GAD was mildly elevated. Result: Whole exome sequencing revealed homozygous variation (c.88G>T) in exon 1 of the FA2H gene. Sanger sequencing from the parents’ sample showed the presence of same heterozygous allelic variation in both parents. Homozygous c.88G>T variation in FA2H gene causes a change from Glycine to Tryptophan at position 30 (p.Gly30Trp) in the Fatty Acid 2 Hydroxylase, hampering the formation of neural tissue and myelin sheath. Besides the usual presentation of Spastic Paraplegia Type 35, this patient also had focal seizures and limb dystonia, which is not commonly seen with this disease. Conclusion: We report a rare case of autosomal recessive spastic paraplegia type 35, with unusual presentation.

Award ID: 743

Methotrexate Induced Leukoencephalopathy; A Case Report

Kopal Maheshwari, Aakash Garg, Pankaj Saini, Sanjiv Maheshwari

Mbbs Student, JLN Medical College Ajmer, Rajasthan, India

E-mail: Kopalmaheshwari18051999@gmail.com

Background and Aim: Methotrexate (MTX) is an antimetabolite that is used both intravenously and intrathecally for the treatment of many cancers. MTX is also commonly used for CNS prophylaxis which is important during therapy of childhood cancers. Unfortunately, MTX can generate neurotoxic side effects such as leukoencephalopathy (LE), which may present as focal weakness, seizures, headaches, and confusion. It is important to be able to distinguish MTX-induced LE from a stroke in an emergency setting to aid appropriate triage and treatment. The Aim was to establish a diagnosis of the patient in order to provide effective treatment. Methodology: This is a case report study. The patient was evaluated with MRI. Result: An MRI of the brain revealed hyperintensity in the periventricular white matter, asymmetrically more on the right, and also involving the genu of the corpus callosum1).. The transient LE findings were thought to be secondary to intrathecal MTX-induced LE, and symptoms resolved with leucovorin, methylprednisolone. Conclusion: High-dose intrathecal methotrexate has shown to decrease CNS disease or metastasis in childhood ALL and non-Hodgkin lymphoma. Although MTX-related neurotoxicity is rare, acute and subacute neurological symptoms including seizures, sudden onset of transient paresis, blurred vision, aphasia, anarthria, pseudobulbar palsy, loss of consciousness, opisthotonus and confusion. While these side effects can occur days or a few weeks after treatment with intrathecal MTX, a delayed form of MTX-induced neurotoxicity can manifest several months to years after treatment with intrathecal MTX. Symptoms in acute–subacute MTX-induced LE are transient and can resolve within a few days with clinical recovery occurring 1–10 days after the onset of LE

Award ID: 744

FBXO7 Mutation: A Rare Cause of Early Onset Parkinsonism with Prominent Impulse Control Disorder

Sahil Mehta, Surbhi Mahajan, Vivek Lal

Neurology, PGIMER

E-mail: mehtasahilpgi@gmail.com

Background and Aim: Early onset parkinsonism account for 3-5% of all cases of Parkinson’s disease. It can be a manifestation of a broad range of genetic and metabolic etiologies. Methodology: We describe a young patient who presented with impulse control behaviour and parkinsonism and was found to have a compound heterozygous mutation in the FBX07 gene. Result: A 30-year-old male product of non-consanguineous marriage presented with impulse control behaviours since early childhood in the form of keeping his bags and books in a particular pattern along with an excessive craving for sweets. He used to steal money to buy sweets and would eat the whole box at once. At 20 years of age, he developed gradually progressive parkinsonian features in the form of right-hand tremors with bradykinesia and rigidity progressing lately to the extent of postural instability and falls. Examination revealed frontal lobe dysfunction with MOCA 25/30. MRI brain revealed mild cerebral atrophy. FDOPA PET revealed asymmetric reduced striatal uptake more on the right side. Treatment with levodopa and dopaminergic agents even in small doses was complicated by severe exacerbation of verbal and physical aggression. Clinical exome sequencing revealed a compound heterozygous non-sense variant in exon 9 of the FBX07 gene [c.1213 G>T {p. Glu405Ter}]. Conclusion: FBX07 is a rare autosomal recessive monogenic form of parkinsonism. The median age is 17 years with variable response to levodopa and characterized by pyramidal involvement and prominent neuropsychiatric manifestations. To conclude, FBX07 mutation should be included in the differential diagnosis of early onset parkinsonism with prominent psychiatric manifestations.

Award ID: 745

Peripheral Nervous System Involvement in a Patient with Multiple Sclerosis

Swamy Kondapally, Sowmini PR, Malcolm Jeyaraj K, Sakthivelayutham S, Viveka saravanan R, Mugundhan K

Neurology, Govt Stanley Medical College Hospital, Chennai, Tamil Nadu, India

E-mail: swamykondapally2@gmail.com

Background and Aim: Multiple sclerosis (MS), a common acquired demyelinating neurological disease, is traditionally regarded as restricted to the CNS. However, various studies have also shown that the peripheral nervous system (PNS) can be involved in MS, expanding the clinical spectrum outside the brain and spinal cord. Here we report a case of RRMS who presented with acute flaccid areflexic weakness of left lower limb. Methodology: A 27 year old female with RRMS on IFN therapy, developed acute onset LMN type of weakness in left lower limb. NCS of all four limbs showed severe axonal neuropathy of left tibial nerve, peroneal nerve and left sural nerve. EMG showed fibrillation and chronic denervation potentials.. MRI brain revealed no new white matter lesions. MRI whole spine showed diffuse cord atrophy. ENA profile was negative Result: It is widely accepted that pathological changes in MS are restricted to the CNS and cranial nerves.. A potential factor that might contribute to the occurrence of PNS lesions might be that PNS lesions are the result of Walleriandegeneration caused by spinal cord lesions in MS.1 Shefner JM et al, suggested that demyelination in the region of the ventral root exit zone may account for LMN findings.2 Conclusion: The fact that Lower motor neuron damage may also occur in MS patients is not widely accepted. Involvement of the peripheral nervous system in MS as seen in our case, may offer new insights into the pathophysiology and treatment of MS.

Award ID: 746

Olfactory Groove Meningioma Masquerading As Chronic Isolated Apathetic Syndrome

Rindha Venepally, Sandhya Manorenj, Reshma Sultana Shaik

Neurology, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: rindha7.rao@gmail.com

Background and Aim: Tumors of brain can present with myriad of symptoms. Psychiatric symptoms may be the only manifestations of certain tumors like meningiomas or gliomas. Thus, it becomes crucial for clinicians to suspect intracranial growth in patients with new-onset psychiatric symptoms. An early diagnosis helps in appropriate management. Available literature shows very few case reports. Methodology: A 49 yr old male patient with no comorbidities presented with a history of lack of interest in daily activities for 10 months. Patient was taken to multiple psychiatrists where he was diagnosed to have depressive disorder and was started on medications for the same. On reviewing the history, patient also gave history of decreased sense of smell since 1 year. However there was no history of headaches, seizures, urinary incontinence, loss of consciousness or any systemic symptoms. On the basis of the above complaints a detailed neurological workup including neuropsychology was done and was normal. Patient had hyposmia on examination. Result: In view of his persisting apathy and anosmia MRI brain with contrast was advised and revealed a well defined bifrontal extraaxial space occupying lesion invading into the floor of the anterior cranial fossa showing restriction on DWI with a sun burst appearance and diffuse and homogenous enhancement on contrast. A diagnosis of likely atypical meningioma was made. Conclusion: Our case illustrates the need for prompt and detailed assessment including brain imaging studies when patient presents with atypical psychiatric symptoms. It is important for clinicians to have an index of suspicion of brain tumour in patients with new-onset psychiatric symptoms, atypical presentations and treatment resistance.

Award ID: 747

Two Rare Cases of Genetically Proven Parkinsonism

Soumya A, G Butchi Raju, S Gopi Seepana, T Sateesh Kumar

Department of Neurology, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

E-mail: soumya_arapreddy@yahoo.co.in

Background and Aim: Two cases of young onset parkinsonism were evaluated for cause Methodology: CASE 1: A 24 yr old female presented with insidious onset stiffness, decreased utilily and rest tremors of right upper limb since 1 year duration. She had difficulty in carrying out activities of daily living due to decreased dexterity of right limb. History of slowness of activities was noted where she used to take more amount of time to walk / eat along with history of micrographia. Examination revealed mask like facies, decreased blink rate, positive tests of bradykinesia, cogwheel rigidity in right upper limb and rigity of right lower limb. MMSE was 24, detailed cognitive examination revealed executive dysfunction, impaired working and episodic memory. Examination of power, reflexes, sensory and cerebellar system was normal. Tests for autonomic dysfunction were normal. CASE 2 : A 29 yr old male presented with insidious onset gradually progresssive parkinsonism which was symmetric along with spastic ataxia, stimulus sensitive myoclonus. He had dysarthria and GTCS. No evidence of chorea / dystonia. examination revealed parkinsonism, normal autonomic testing, MMSE of 20, EEG showed generalised epileptiform discharges. Result: Routine blood work did not reveal any abnormality. MRI Brain was normal. No KF ring. Serum cooper and ceruloplasmin was normal. No acanthocytes on peripheral smear. In view of inconclusive workup, a clinical exome sequencing was sent which revealed a heterozygous mutation for VPS13C i.e PD-23 in case 1 and ATP13A2 in case 2 Conclusion: Case 1 showed PD23 as the cause for young onset parkinsonism and case 2 showed Kufor rakeb disease as the cause

Award ID: 748

A Rare Presentation of Lithium Toxicity

Arun Kumar N, Balasubramanian S, Balasubramanian S, Lakshminarasimhan Ranganathan

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drarun1809@gmail.com

Background and Aim: Lithium salts are widely used in treating bipolar disorder. Adverse effects of lithium include tremor, ataxia, hyperreflexia, downbeat nystagmus, gaze evoked nystagmus and gaze palsies. Factors leading to lithium toxicity are dehydration, infection, altered renal function. We are presenting a case of lithium induced nystagmus. Methodology: A 56 year old male with chronic bipolar disorder treated with quetiapine, was recently added lithium bicorbonate 300 mg bd for 3 months. He developed unstable vision in the form of difficulty in fixing the gaze in primary position. On examination, patient had downbeat nystagmus, upgaze restriction, slow saccades & exaggerated tendon reflexes. Structural causes were ruled out on MRI brain, CVJ and spine. Metabolic panel with vitamin B12 and magnesium levels were normal. Serum lithium level was 0.7 mmol/L, which was in normal therapeutic range. Result: Symptoms were improved with adequate hydration and gradually diminished and subsided over a period of 2 weeks, after discontinuation of lithium. Conclusion: Downbeat nystagmus is a rare adverse effect of lithium toxicity. It can occur even with therapeutic range of lithium level. This should be considered once other structural and metabloic causes are ruled out.

Award ID: 749

Case report of Two Genetically Proven Congenital Myasthenic Syndromes

Soumya A, G Butchi Raju, S Gopi Seepana, T Sateesh Kumar

Department of Neurology, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

E-mail: soumya_arapreddy@yahoo.co.in

Background and Aim: To evaluate two cases of external opthalmoplegia and proximal muscle weakness Methodology: CASE 1 : A 7 yr old boy presented with insidious onset progressive ptosis, restricted eom, no diplopia, frequent respiratory crisis and proximal muscle weakness with east fatiguability and improvement with rest. no history of birth asphyxia CASE 2 : A 33 yr old female presented with symmetric ptosis and opthalmoplegia with blindness since 4 yrs of age. Mild dysphagia and proximal muscle weakness of all limbs and no history of respiratory crisis. Anti AcHR Ab were negative in both. RNS showed decremental response with 2 Hz. Ice pack test and neostigmine test was negative Result: In view of inconclusive workup, whole exome sequencing was sent which revealed a pathogenic mutation in CHRNE, fast channel congenital myasthenic syndrome in both patients. Conclusion: Fast channel syndrome (FCS) comprises around 5% of genetically confirmed CMS. Majority of fast channel syndrome present as a severe form of CMS with associated sudden, life-threatening apnoeas on a background of persistent generalised muscle weakness. Patients may have an initial dramatic response to cholinesterase inhibitors but over time this reduces. Addition of 3, 4-Diaminopyridine can be beneficial.

Award ID: 750

Harlequin Syndrome Secondary to Herpes Simplex Encephalitis

Abhishek Dixit, Abhishek Pathak, Varun Singh, Vijaya Nath Mishra

Neurology, IMS BHU, Varanasi, Uttar Pradsh, India

E-mail: abhidixi88@gmail.com

Background and Aim: Harlequin syndrome is a rare dysautonomia syndrome characterized by increased sweating on one side and anhidrosis on the contralateral side of the body. Methodology: 24 years old male presented with fever, multiple episodes of focal seizures with bilateral tonic clonic movements and altered sensorium. On evaluation, he was diagnosed as a case of Herpes Simplex Encephalitis based on classical radiological and positive cerebrospinal fluid real time polymerase chain reaction test for herpes simplex virus. He received full course of intravenous acyclovir during hospital stay and discharged on two antiepileptics. During follow up, he developed increased sweating on one half of face and anhidrosis over another half suggestive of Harlequin syndrome. However, these symptoms subsided by its own without any active intervention during further follow up. Result: Harlequin syndrome is rare association with the heroes encephalitis syndrome Conclusion: To the best of our knowledge, this is the first case report of Harlequin syndrome secondary to Herpes Simplex Encephalitis.

Award ID: 751

A case of MRI Negative Myelopathy - MTHFR Mutation Causing Myeloneuropathy

Vedang Desai

Neurology, AIIMS, New Delhi, India

E-mail: drvedangdesai@gmail.com

Background and Aim: The methyl tetrahydrofolate reductase (MTHFR) gene provides instructions for the body to make the MTHFR protein, which helps process folate, which in turn is needed to make DNA and modify proteins. MTHFR is a key regulatory enzyme in folate and homocysteine metabolism. Research performed during the past decade has clarified our understanding of MTHFR deficiencies that cause hyperhomocysteinemia with homocystinuria. Rarely, they may present with features suggestive of non-compressive myelopathy/ myeloneuropathy and are reversible if diagnosed and treated early. We describe one such case. Methodology: A 25 years old gentleman presented with insidious onset, gradually progressive asymmetric spastic, pure motor quadriparesis of 3 months duration. Examination revealed decreased power (MRC- --/5), brisk deep tendon reflexes, extensor plantars with normal sensory examination. His routine investigations were normal including viral markers, B12 and folate, thyroid and ENA profile. However, his serum homocysteine levels were elevated. MRI brain and spine was suggestive of changes consistent with Vitamin B12 deficiency. Result: In view of these findings, Genetic analysis for MTHFR deficiency and mutation were sent, which came positive for MTHFR deficiency. Conclusion: The patient was started on Vitamin B12, Pyridoxine and folate replacement and showed remarkable improvement in 3 months. Power improved from 2/5 in joints of lower limb to 4/5 in just one month with independence in ambulation. Thus, MTHFR mutation should always be considered as a differential when MRI negative Myelopathy is being assessed, even more so when associated Demyelinating Polyneuropathy is detected.

Award ID: 752

A Study on Clinical Profile of Non Parkinsonian Tremor with Electrophysiological Correlation

Seema Shahi

Department of Neurology, Artemis Hosptal, Gurugram, Haryana, India

E-mail: findshahi@gmail.com

Background and Aim: Introduction- Tremor is most common movement disorder but the diagnosis of different tremor syndrome is challenging. Objective- To study the clinical profile of various types of non-parkinsonian tremor disorders and the electrophysiological characteristics of various non-parkinsonian tremors. Methodology: Method- As per inclusion and exclusion criteria, a total of 50 patients were enrolled in the study. Personal and demographic particulars of all the patients will be Noted. Surface EMG was used to distinguish between different types of tremors. A clinical and statistical correlation will be made between the clinical and electrophysiological diagnosis. Result: Results- Among essential tremor patients, 6 (19.3%) and 25 (80.6%) patients had alternating and synchronous pattern on surface EMG respectively. All cerebellar, enhanced physiological tremor, alcohol withdrawal tremor and drug induced tremor patients had synchronous pattern on surface EMG whereas dystonic tremor and psychogenic tremor patients had alternating pattern on surface EMG. Mean surface EMG frequency was 11.1 ± 0.7 hz for essential tremor whereas for Cerebellar - 4.2 ± 0.4 hz (Range: 3 - 5 hz), Enhanced Physiological Tremor -9.1 ± 2.2 hz (Range: 5 - 11 hz), Dystonic Tremor - 6.5 ± 0.6 hz (Range: 5 - 7 hz), Alcohol withdrawal Tremor - 9.8 ± 0.4 hz (Range: 9 - 10 hz), psychogenic Tremor - 7 ± 0.5 hz (Range:6 - 7 hz) and for Drug Induced Tremor – 9 hz. Conclusion: CONCLUSION- Among all types of tremor, essential tremor is the most common cause. In most cases of tremor, there is no diagnostic laboratory test to confirm or exclude a particular type of tremor and the diagnosis heavily relies on physician’s thorough clinical history as well as clinical examination. Neurophysiological techniques (EMG) and imaging, can be useful to differentiate different types of tremors.

Award ID: 753

Biotinidase Deficiency Mirror as a Case of Neuromyelitis Optica Spectrum Disorder

PoojaTripathi, Hardeep singh Malhotra, R. K. Garg, Neeraj Kumar, Sanjeev Verma

Neurology, KGMU Lucknow, Uttar Pradesh, India

E-mail: docpoojachildcare@gmail.com

Background and Aim:Metabolic and inflammatory conditions may lead to neurological disorder. We report a case of biotinidase deficiency mirror as case of NMOSD. Biotinidase deficiency is a rare, autosomal recessive, metabolic disorder. Clinical features are heterogenus LETM is one of them and can easily misdiagnosed as NMOSD. Methodology: We report a case of biotinidase deficiency mimic as case of NMOSD Result: 13 yr old male presented with c/o fever with difficulty in walking along with drowsiness since 1 month. He had past h/o rashes along with drooling of saliva and decreased scholastic performance over the years. On neurological examination he was having spasticity with features of sensory ataxia with impaired joint position sensation. His CSF routine examination was normal and CSF virology came negative but CSF lactate was high along with increased serum lactate. MRI brain showed hyperintensity in b/l basal ganglia and mamillary body and MRI spine showed LETM from cervicomedullary junction to C7 involving anterior., lateral and posterior column of spinal cord. He developed quadriparesis and needs ventilatory support. His NCS study, RNST was normal so was NMO-MOG profile. In view of decreased scholastic performance with intermittent episodes of falls and fluctuating coarse metabolic disorder become 1 st differential. His Urine GCMS showed severe biotinadase deficiency. Conclusion: Correct diagnosis of Biotinidase deficiency can prevent spinal disability when identified early and treated aggressively.

Award ID: 754

Tacrolimus- Induced Atypical Posterior Reversible Encephalopathy Syndrome (PRES) after Liver Transplantation : An Interesting Case Report

Sri Sai Srujana Puppala, Anuja Patil

Neurology, Krishna Institute of Medical Sciences

E-mail: patilanuja1986@gmail.com

Background and Aim: With the increasing trend of organ transplantation rates, use of immunosuppressive agents such as cyclosporine and tacrolimus has also risen significantly. Posterior reversible encephalopathy syndrome (PRES) is a serious complication associated with immunosuppressive agents and solid organ/ stem cell transplantation. Methodology: A 58 year gentleman with decompensated liver disease underwent deceased donor liver transplantation. On postoperative day 13, he developed tremulousness of both hands. Next day he had focal onset GTCS with automatisms and behavioural arrest followed by post ictal confusion. He was on steroids, tacrolimus and mycophenolate for immunosuppression. Upon examination, he was Conscious, oriented with mild dysarthria, Bilateral extensor plantar response, mild postural and intentional tremors. Tandem walking was impaired. Result: His metabolic parameters and tacrolimus levels were normal. EEG showed encephalopathy and MRI Brain had T1 isointense lesions in inferior right parieto- occipital sulcus, right middle occipital gyrus and left middle temporo-occipital gyrus with corresponding T2/ FLAIR hyperintense lesions and facilitated diffusion suggestive of atypical PRES. After stopping tacrolimus, his symptoms resolved and follow-up MRI was normal. These changes could be due to reversible vasogenic edema. Conclusion: Tacrolimus toxicity presenting as PRES can be reversed completely with discontinuation/ decrease in dose of the drug or switching to another alternate agent. We want to highlight importance of high index of suspicion of PRES in the patients who are on tacrolimus and value of MRI in prompt recognition of this syndrome.

Award ID: 755

Fragile X-Associated Tremor Ataxia Syndrome in an Adult Male: A Rare Case Report

Sri Sai Srujana Puppala, Subhash Kaul

Neurology, Krishna Institute of Medical Sciences

E-mail: srujana.pss@gmail.com

Background and Aim: The primary features of FXTAS are action tremor and gait ataxia occurring in older males. It is caused by full mutation expansion in excess of 200 repeats of a CGG, located in the 5’ untranslated region of the fragile X mental retardation 1 (FMR1) gene. Methodology: A 56 year old gentleman patient came with complaints of progressive symptoms of ataxia, tremor, rigidity and cognitive dysfunction. The neuropsychological tests confirmed cognitive impairment. His elderly brother had similar complaints. Result: MRI Brain showed symmetrical T2/FLAIR hyperintensities in subcortical white matter of bilateral parietal lobes, bilateral middle cerebellar peduncles, paramedian aspect of bilateral cerebellar hemispheres and splenium of corpus callosum. Diffusion restriction was noted in bilateral middle cerebellar peduncles (MCP) and splenium of corpus callosum, features suggestive of FXTAS. The progressive symptoms and the MRI features suggested a degenerative disease, due to fragile X premutation. The genetic tests, conducted with Southern blot and PCR, showed an expansion of CGG triplets in the 5’ untranslated region of the Fragile X Mental Retardation gene. Conclusion: FXTAS associated with fragile X premutation, has been noted to manifest in elderly men as the presence of tremor, ataxia, dyssynergia, cognitive decline, rigidity, and impotence. FXTAS is a rare neurological disorder with varied clinical symptoms including motor, sensory, and cognitive findings that may mimic other degenerative diseases, especially MSA C. MRI typically reveals generalized brain atrophy with characteristic MRI findings of MCP sign. However, a genetic test is necessary to look for presence of the pre-mutation.

Award ID: 756

A Case of Ataxia in an Elderly Lady Associated with Amiodarone: A Case Report

Sri Sai Srujana Puppala, Subhash Kaul

Neurology, Krishna Institute of Medical Sciences

E-mail: srujana.pss@gmail.com

Background and Aim: Amiodarone is widely prescribed in patients with tachyarrhythmias and its neurological side effects like ataxia though rare can be very disabling. Older age group individuals are more vulnerable to this side effect. Methodology: A 64-year-old woman with a past history of diabetes mellitus, HTN, CRHD post MVR, CAD post PTCA to LAD (6 months prior to the current symptoms) with AF, presented with the complaints of imbalance while walking with multiple falls, action tremors, slurred speech since 3 months. Her medications included verapamil, amiodarone, metformin and warfarin. Result: On examination, she was oriented with intact memory and slurred speech. Horizontal and vertical nystagmus was present. Her motor and sensory examination was normal. There was finger-nose-finger dysmetria, titubation, dysdiadochokinesia and heel shin test impairment. She had wide based gait and truncal ataxia. MRI brain showed no significant cerebellar lesion or cerebrovascular disease. TSH and metabolic parameters were normal. A review of her medications revealed that she was given loading dose of amiodarone followed by maintenance dose of 200 mg/day for AF. Once amiodarone-related neurotoxicity was considered, it was discontinued. Six weeks after the discontinuation of amiodarone, her gait disturbance completely resolved. Conclusion: The lowest possible dose of amiodarone to control the arrhythmia should be used, especially in older people. Withdrawal or reduction of amiodarone therapy may lead to considerable improvement in neurological side-effects. Elderly patients on amiodarone should have regular screening and assessment of neurological function.

Award ID: 757

Unusual Etiology of Cranial Neuropathies

Natarajan Elamurugan, Lakshmi narasimhan Ranganathan, Thamil pavai Arulnambi, Nithyanandham Alimuthu, Nandhakumar V

Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drnataraj91@gmail.com

Background and Aim: Cranial neuropathies are one of the most commonly encountered neurological condition in the hospital settings. The etiology of this entity could be various ranging from infections, inflammatory and infiltration etc. Clinical picture varies according to the nerves involved and site of lesion. Methodology: In this we present two cases of cranial neuropathies of unusual etiology. Both these patients were subjected to routine investigations and imaging and their results are submitted here. Result: For both the patients MRI contrast showed granulomatous etiology. CNS TB has been ruled out with CSF CBNAAT. ACE levels were also normal in both these patients. These patients were initially labelled as Tolosa hunt syndrome. Then we did IgG4 assay for these patients which found to be high in both the patients. Hence we made a diagnosis of IgG4 disease presenting as cranial neuropathy. Conclusion: Granulomatous cranial neuropathies can have various etiologies. IgG4 related disorder should also be considered in differential diagnosis in any patient suspected to have cranial neuropathies of granulomatous etiology.

Award ID: 758

Safety and Efficacy of Perampanel in Difficult to Treat Patients with Epilepsy

Mehul, Nirmal Surya

Neurology, Bombay Hospital

E-mail: drmehuldesai07@gmail.com

Background and Aim: Global prevalence of epilepsy is approximately 1% and that of India mirrors global data. Perampanel is a newer first in class anti-seizure drug directed against AMPA receptors. As almost 1/3rd of epilepsy patients has difficult to treat epilepsy, current study was undertaken to find safety and efficacy of Perampanel in difficult to treat patients of epilepsy. Methodology: It is a prospective observational study conducted at Epilepsy Foundation, Mumbai over a period of 2 years. We enrolled all the patients with difficult to treat focal or generalized epilepsy above age 12 years and started them on Perampanel as adjuvant therapy. Patients were monitored for seizure reduction, seizure freedom and drug tolerability. Those with status epilepticus, age < 12 years and absence seizures were excluded from study. Result: Out of total 40 patients enrolled in the study, 6 were lost to follow up hence removed from final analysis. Amongst 34 patients 44% were female and 56% male. Perampanel was started as first add on in 8%, 2nd add on in 38%, 3rd add on in 39% and 4th add on in 15% patients. Patients in whom drug was started as first or second add on had highest seizure reduction. Conclusion: Perampanel is a drug with novel mechanism which is well tolerated and safe. Drug is most effective when started early in the course of treatment.

Award ID: 759

Rare Case of Extrapyramidal Emergency

Anand N, Saravanan S

Neurology, Tirunelveli Medical College, Tamil Nadu, India

E-mail: aohawk89@gmail.com

Background and Aim: Antipsychotics are common causes of drug induced extrapyramidal syndromes. Drug induced movement disorders can range from tremors to life-threatening syndromes. They usually present with bilateral extrapyramidal symptoms. Flupenthixol Decanoate is a antipsychotic drug of the thioxanthone group available in tablet and intramuscular injection form used in maintenance therapy of schizophrenia. It is an inhibitor of both D1 and D2 Dopamine receptors in the brain. Methodology: 29 years old male apparently normal a month back had initial complaints of fatigue and sleep disturbances. He consulted a neurologist and was treated. His illness progressed such that he developed giddiness and had sensation of worms crawling inside his head. He was referred to psychiatric clinic where he was diagnosed with schizophrenia and was treated with flunarizine, escitalopram, clonazepam and intramuscular injection of flupenthixol decanoate. Following day he developed stiffness in all limbs, bradykinesia and dysphagia. MRI Brain was normal and patient was referred to a neurologist. Patient presented to us with above complaints. Following day of admission patient symptomatically worsened with agitation, generalized rigidity and tremors. Patient was immediately shifted to ICU and was given intravenous diazepam, fluids and promethazine and oral trihexyphenidyl. Patient improved in due course of hospital stay. Result: Patient was diagnosed with drug induced extrapyramidal syndrome presenting as an acute emergency. Flupenthixol may induce life threatening extrapyramidal syndrome presenting with marked generalized tremors, rigidity and agitation. Conclusion: It is common in psychiatric clinics to give depot preparation of flupenthixol to patients of Schizophrenia. Neurologists should be aware that intramuscular depot preparations of flupenthixol may present as an acute emergency with extrapyramidal symptoms. Early recognition is essential for prompt intervention and faster recovery. Management includes stopping the offending drug, supportive care, and pharmacological treatment.

Award ID: 760

Migraine and Metabolic Syndrome

bharat Bhushan, Jayanti Kalita, Vinay Kalluri, Sneha Thakur, Rajesh Deoghuria

Neurology, Novartis Healthcare Pvt Ltd

E-mail: bharat-1.bhushan@novartis.com

Background and Aim: Migraine disease and metabolic syndrome (MetSyn) are both pro-inflammatory disorders with vascular changes. Therefore, coexistence of migraine and MetSyn requires careful management to treat patients with both concomitantly without worsening either underlying conditions. This article reviews the relationship, prevalence, pathology and challenges in management, including more modern management options for treating migraine when co- existing with MetSyn. Methodology: We did database research with specific keywords on Pubmed, google scholar and URLs of major organization Result: An association between migraine and vascular disorders such as coronary heart disease and stroke has been reported; obesity and diabetes as metabolic disorders also have been associated with migraine. Insulin resistance plays an important and major role in the development of metabolic syndrome, further leading to several cardiovascular factors Conclusion: MetSyn is a chronic disorder and has a very protracted underlying disease course on which every episode of migraine could hasten the progression of the underlying vascular pathology. The coexistence of these conditions gradually make treatments less effective. Screening for an underlying MetSyn must be considered as a routine part of management of migraine patients. Management of life stressors is one of the preventive healthcare strategies being implemented, although the exact benefits in patients with migraine and metabolic syndrome following this method is unclear. Some of the existing migraine prophylactic treatment options have contraindications in especially obese individuals, diabetic patients and patients with hypertension. The choice of therapy may have impact on the management of metabolic syndrome; however, much is yet to be learned in this regard.

Award ID: 763

Acute Presentation Of CNS Vasculitis: Unravelling the Mystery

Shweta Koshy

Internal Medicine, PSG Institute of Medicine Science and Research, Coimbatore, Tamil Nadu, India

E-mail: Shwetakoshy@gmail.com

Background and Aim: Primary angitis of central nervous system is a rare small and medium sized vasculitis resulting in inflammation and destruction of the blood vessels of the brain, spinal cord and meninges without any systemic vasculitis. It commonly presents as subacute and insidious onset. Most common presenting symptoms were headache 60%, cognitive impairment, stroke and TIA in 30-50% of cases. Methodology: This 50 year old female with uncontrolled T2 DM / SHTN / s/p hysterectomy/ CA- thyroid s/p thyroidectomy, not on regular medication, presented with slurring of speech with dysarthria for 1 day. Neuroimaging showed acute infarcts in right corona-radiata and capsuloganglionic region and started on routine stroke treatment. On 3rd day of admission, patient developed left hemiparesis with left UMN facial palsy. Repeat MRI showed increased size of the infarct. Two days later she developed further worsening of left hemiparesis, dysarthria and dysphagia in spite of being on antiplatelets and anticoagulants and CT carotid angiogram was done which revealed multiple blocks at different sites due to - ?Atherosclerosis with left temporal lobe bleed. High suspicion of CNS vasculitis arose in this setting of recurrent ischaemic infarcts and intracerebral haemorrhage despite the background of risk factors for stroke and stroke medications. The antiplatelets and heparin were stopped in view of ICH. DSA revealed classical beading appearance in B/L middle cerebral artery (M2 branches) and distal branches of both anterior and posterior circulation with basilar artery stenosis. A clinical and radiological based diagnosis of CNS vasculitis was made. She wasn’t willing for meningeal biopsy. Patient was started on IV steriods and mycophenolate mofetil and showed an improving trend. She developed B/L lower limb weakness and dysphagia and repeat MRI showed new infarcts in midbrain and hemipons while on steriod coverage and hence she was started on 1 dose of Inj. Rituximab. Result: PACNS, having a kaleidoscopic array of presentations, makes it more difficult to identify it immediately. Patient improved drastically on starting Inj. Rituximab and was able to walk without support and take oral feeds. She is on regular follow up. Conclusion: Initially presenting as a single stroke evolving into strokes involving different anatomical territories makes this case more baffling.

Award ID: 764

A Case of Hereditary Motor Sensory Neuropathy with Vocal Cord Palsy And Parkinsonism

Abbigari Vardhan

Neurology, Madurai Medical College, Madurai, Tamil Nadu, India

E-mail: harsha.abbigari3@gmail.com

Background and Aim: Hereditary neuropathy constitute a complex heterogenous group of diseases that usually share the clinical features of insidious onset and indolent course over years to decades, with long standing neuro muscular symptoms and skeletal abnormalities, electrophysiological evaluation and more importantly testing for confirming genes are essential in identifying inhereditary neuropathy Methodology: A 55 year old male with no similar illness in the family presented with insidious onset, slowly progressive neurological illness characterized by flial weakness of all four limbs (distal > proximal) with bilateral foot drop along with deformities of hand, feet and spine and bony deformities of wrist and ankle associated with severe wasting of muscles of hand, feet and leg, lower thigh muscles over 35 years. History of numbness in bilateral lower limb with dark incoordination and wash basin phenomenon without loss of other sensory modalities, tremors of hands, slowness of daily activities with frequent falls forward since 8 months. On examination thickened nerves present, with severe kypho scoliosis, hammer toes, clawing of fingers and toes, bilateral foot drop. and inverted champagne bottle legs, wasting of small muscles of hand, feet, calf and leg muscles. Areflexia of DTR with rigidity, resting tremors, reduced blink rate, pull test positive, sensory ataxia, left vocal cord palsy Result: MRI brain with spine shows cerebral atrophy with spondylotic changes. NCS -uniform absence of motor and sensory study in all four limbs. Genetic analysis report awaited. Conclusion: HMSN related movement disorders are associated with disturbance of the CNS, as like our patient is having parkinsonism like features resting, rigidity, bradykinesia and postural instability.

Award ID: 765

Myopathy as an Initial Manifestation of Secondary Hyperparathyroidism

Rupesh Kumar M, Manickavasagam J, Shunmugasundaram K, Lakshminarasimhan R, Vinoth kannan, Manickavasagam J, Shunmugasundaram K, Lakshminarasimhan R

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drmrupeshkumargmail.com

Background and Aim: 17 year old female with no known comorbidities presented with insidious onset gradually progressive symmetrical weakness of both lower limbs for 6 months duration. She also presented with truncal weakness. No history of upper limbs weakness, facial muscle weakness, diurnal variation, fatigability, ptosis, dysarthria, dysphagia, twitching, involuntary movements. Methodology: O/E there were no neurocutaneous markers, no skeletal abnormalities, no facial dysmorphism. Higher mental functions, cranial nerve examination, anthropometry was normal. There was symmetrical weakness of both lower limbs (proximal > distal) with neck & truncal weakness. DTR in all 4 limbs was brisk. Bilateral plantar flexor response and Gower’s sign was positive with waddling gait. There was no Sensory, cerebellar, and autonomic nervous system involvement. Result: LFT showed increased ALP – 1757 IU/L and decreased phosphorus 1.9 mg/dl. Calcium level was 8.7 mg/dl. NCS of all four limbs was normal. EMG showed myopathic pattern. MRI cervical spine with whole spine screening was normal. MRI brain was normal. Skeletal survey showed increased end plate convexity- fish mouth vertebra and diffuse osteopenia with prominent bony trabeculations with focal areas of radiolucency. DEXA scan showed osteoporosis. Parathyroid hormone was raised 376.8 pg/ml, Vitamin D was low 2.5 ng/ml. FGF 23 was normal 9.18 pg/ml. Conclusion: She was diagnosed with osteomalacia due to vitamin D deficiency with secondary hyperparathyroidism and proximal myopathy and was started on Calcium – 1.5-2 gms/day and Vitamin D3 – 50,000 IU weekly for 3-12 weeks followed by maintenance therapy for 800 IU daily.

Award ID: 766

Pramipexole Induced SIADH in a Patient with Young Onset Parkinson’s Disease

Baikuntha Panigrahi, Divya Madathiparambil Radhakrishnan, Roopa Rajan, Achal Kumar Srivastava, Awadh Kishor Pandit, Ayush Agarwal

Neurology, AIIMS New Delhi, India

E-mail: baikunthap199@gmail.com

Background and Aim: Syndrome of inappropriate anti-diuretic hormone (SIADH) is an extremely rare complication of dopaminergic agonists (DA). Pramipexole, a DA, by its increased affinity for D4 receptors stimulates the secretion of arginine vasopressin (AVP) in the supraoptic nucleus of the hypothalamus. This report highlights a rare but reversible adverse effect of the commonly used DA, pramipexole. Methodology: This case report describes a patient of young onset Parkinson’s disease (YOPD) who developed new onset hyponatremia due to pramipexole-induced syndrome of inappropriate anti-diuretic hormone (SIADH) within 3 months of starting the drug. Result: Initial management with salt tablets and fluid restriction were ineffective. In view of multiple case reports incriminating pramipexole as a cause of SIADH in patients with PD and its ability to act on D4 receptors, the doses of pramipexole were reduced by 50% to 3.15 mg/d. Rest of the drugs were continued as before and by 5th day of reducing dose of pramipexole, the sodium levels raised to 135 mEq/L. Conclusion: SIADH is a rare, but reversible side effect of DAs like pramipexole. The limited number of cases in the literature despite the widespread use of DA in PD suggests that the condition is unrecognized or under reported. Hyponatremia can cause worsening of both motor and nonmotor symptoms in PD and, if present, should prompt an evaluation for drug induced SIADH and optimization of medication

Award ID: 767

A Rare Case of Levodopa Responsive Painless Legs and Moving Toes Syndrome

Guru Yellamsetty, M. Jawahar, M. Sivaji, Marian Jude, M. Jawahar, R. Lakshmi Narasimhan

Neurology, Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: gurumedhasvi@gmail.com

Background and Aim: Introduction: painless legs and moving toes syndrome is a rare movement disorder characterised by Flexion and extension, abduction, adduction, and torsion of toes in the absence of pain and it is considered as a variant of painful legs and moving toes syndrome Methodology: Case presentation: 45 y old female with out any comorbidities, addictions, with out any drug and native medicine intake presented with non rhythmic, repititive, involuntary movements of both toes, in the absence of pain, these movements are present throughout the day more on rest and standing, disappears on walking and sleep on examination conscious and oriented with normal higher mental functions, cranial nerve and motor and sensory, cerebellar, EPS examination normal except above involuntary movements of toes with out any cogwheel rigidity, bradykinesia, hypokinesia, negative pull test Result: All necessary investigations was done, routine blood investigations with serum vitB12 levels, serum iron, ferritin, thyroid profile, serum calcium and magnesium, phosphorus, NCS of all four limbs MRI BRAIN and SOINE SPINE screening were normal Conclusion: Idiopathic painless legs and moving toes syndrome diagnosis was made and treated with combination of levodopa and carbidopa (100+10 mg), patient improved symptomatically

Award ID: 768

Clinical and Diagnostic Profile of Myopathy in a Tertiary Care Centre-Case Series of 15 Cases

Vinuthna Kodi, P. K. Murugan, S. Ramu, M. R. Manivannan, C. Justin

Neurology, Madurai Medical College, Madurai, Tamil Nadu, India

E-mail: drvinnu05@gmail.com

Background and Aim: Myopathies are disorders which occur due to dysfunction of muscle fibre. They can be acquired or inherited. They include congenital myopathy, muscular dystrophy, mitochondrial, inflammatory, metabolic, infectious, drugs and toxin induced myopathies. Methodology: Observational cross-sectional study of 15 cases referred to territory centre Madurai. The cases were evaluated clinically with appropriate laboratory investigations and electrophysiological tests were done. Result: Out of 15 patients 11 patients were males and 4 patients were females. Differential weakness was seen in certain types of myopathies. Weakness of extensors and abductors of hip, extensors of knees are more commonly involved in dystrophinopathies. Calf hypertrophy, knee flexors weakness, positive hip abductor sign with winging of scapula is seen in sarcoglycanopathies. Knee plantar flexor weakness with diamond on quadriceps sign, hypertrophy of calf seen in dysferlinopathies. Winging of scapula, hip extensor weakness with muscle atrophy seen in calpainopathies. Scapular winging, polyhill sign, Beevor sign is seen in FSHD. Calf hypertrophy with proximal weakness is seen in endocrine myopathy. Out of 15 cases 4 cases were dystrophinopathies, 3 cases FSHD, 3 cases of calpainopathy, 2 cases of dysferlinopathy,1 calpainopathy, 1 Emery Dreifuss muscular dystrophy, 1 thyroid myopathy. Electrodiagnostic tests showed fibrillation potential with small MUP and early recruitment. Conclusion: Myopathies are characterized by various pattern of weakness depending on the underlying disorder. Electrodiagnostic studies serves as extension of neurological examination

Award ID: 769

LETM; Our Experience with a Case Series of 6 Patients

Sajad Tak, Bashir Ahmad Sanaie, Shiekh Hilal Hilal

Neurology, Superspecislity Hospital (SSH), GMC Srinagar, Jammu & Kashmir, India

E-mail: taksajad83@gmail.com

Background and Aim: To study the etiology and outcome in patients with LETM (Longitudinally extensive transverse myelitis. Methodology: Study Design: prospective observational study. Study period : August 2020 to February 2022. All the patients admitted with LETM in the Department of Neurology, Superspeciality hospital, GMC Srinagar were included in the study. A total of 6 patients with LETM presented during this study period. Result: Out of 6 patients, four were females and 2 we’re males. Two patients were diagnosed as NMOSD, one patient as Sjogren Myelitis, one as MOGAD, one as Post Covid Myelitis and other one as idiopathic LETM. Conclusion: Although NMOSD is the commonest cause of LETM, patients with LETM must b evaluated for Sjogrens syndrome and other autoimmune disorders. Females are more predisposed to LETM as compared to males.

Award ID: 770

Comparison of Clinical and Electrophysiological Characteristics of Very Early with Early/Late Guillain-Barré Syndrome

Mritunjai Singh, Ashutosh Tiwari, Nikita Dhar, Jagbir Singh, Vinayak Jatale

Neurology, AIIMS, Rishikesh, Uttarakhand, India

E-mail: mritunjaisingh68@gmail.com

Background and Aim: To compare clinical, electro-diagnostic (EDX) features and long-term outcome between patients with very early Guillain-Barré Syndrome (VEGBS) and early/late GBS. An additional objective was to validate Uncini’s newer EDX criterion. Methodology: 100 patients with GBS were categorized into VEGBS (symptom onset ≤ 4 days) and early/late GBS (> 4 days). All patient underwent detailed clinical and EDX evaluation. Distal and proximal compound muscle action potential (CMAP) amplitude, distal motor latency (DML), duration of distal CMAP (d-CMAP), Proximal/distal (p/d) CMAP amplitude and duration ratio, motor nerve conduction velocity (MNCV), F-wave and sensory nerve action potential (SNAP) were assessed. Uncini’s criteria were used to categorized patients into acute inflammatory demyelinating poly-radiculo-neuropathy (AIDP) and acute motor axonal neuropathy (AMAN) acute motor sensory axonal neuropathy (AMSAN), and equivocal. Result: VEGBS patients had higher peak disability (median 5 vs 4; P = 0.02), frequent in-hospital disease progression (42.9% vs 19.0%, P = 0.009), needed mechanical ventilation (50% vs 22.4%; P = 0.004), less frequent cerebrospinal fluid dissociation (52.4%vs74.1%; P = 0.02). and poor 6-month outcome (52.4%vs27.6%; P = 0.01) compared to early/late GBS. EDX studies were abnormal in all patients. DML was common in early/late GBS (36.2% vs 25.4%; P = 0.02), while F-waves were frequently abnormal in VEGBS (43.3% vs 31.9%; P = 0.03). Uncini’s EDX criteria resulted in only 5% equivocal categories. Conclusion: VEGBS patients are more disabled and have poor six-months outcome compared to Early/Late GBS. Distal CMAP and F-waves are frequently abnormal. Uncini’s criteria resulted in fewer equivocal subtypes.

Award ID: 771

A Study of Etiology and Clinico-Radiologic Profile of Longitudinally Extensive Transverse Myelitis in a Tertiary Care Centre in South India

Srujanitha Tadi, Cornelius Justin, M. R. Manivannan, Vaibhav Kumar Somvanshi, Murugan P. K., Ramu S, Cornelius Justin, P. K. Murugan, Ramu S, Justin Cornelius

Neurology, Madurai Medical College, Madurai, Tamil Nadu, India

E-mail: anithatadi@gmail.com

Background and Aim: LETM is defined as contiguous inflammatory lesions of spinal cord extending to three or more vertebral segments. Neuromyelitis optica (NMO) is the most common cause of LETM. Other causes of LETM are infective, neoplastic, autoimmune diseases and connective tissue disorders. The aim of this study is to study etiological, clinical and radiological profile and treatment outcomes in patients of longitudinally extensive transverse myelitis (LETM) Methodology: This is a hospital based cross sectional prospective study of patients presenting with LETM. Detailed analysis of their clinical features, radiological findings, serum aquaporin 4 (AQP4), MOG antibodies, etiological workup is done. All patients have been followed up for one year for prognosis Result: The mean age in our series was 35.05 ± 15.69 years. There is female predominance. Thoracic and cervical spinal cord segments are the most commonly affected. Optic nerve involvement is seen in three patients (15%). A total of ten patients (50%) were clinically diagnosed as NMOSD, 4 patients are postinfectious myelitis, one case is LETM secondary to SLE, The patients with AQP4 NMOSD and extensive involvement of cord with gray matter necrosis showed residual deficits and relapses are more common with AQP4 antibodies positive NMOSD. Conclusion: LETM is a heterogenous clinico-radiologic spectrum with varied aetiologies, early Identification of the etiology plays a major role in best therapeutic management, prognosticate and prediction of risk of recurrence.

Award ID: 772

Cerebral Venous Sinus Thrombosis Score - A Reliable Prognostic Indicator in CVST?

Chandra Reddy Singireddy, Sandhya Manorenj, Reshma Sultana Shaik

Neurology, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: scr_5656@yahoo.com

Background and Aim: Cerebral venous sinus thrombosis (CVST) is an important cause of stroke in developing countries.1 There is a paucity of studies evaluating the extent of CVST in determining clinical severity, MRI findings, and outcome. A study conducted in Northern India showed no association between CVST score and outcome.2 In this study, we report the role of CVST score and its correlation with the outcome from a tertiary care center in Southern India. Methodology: A prospective observational study was conducted between September 2019 to November 2021 at Princess Esra Hospital, Hyderabad. Consecutive patients during the above period with a confirmed diagnosis of CVST were enrolled in the study after informed consent was taken. These patients were followed up until discharge/death and at 3 and 6 months. The study was approved by the Institutional Ethics committee. CVST Score was scored based on the distribution of clots in the sinuses and 1 point was given for each sinus involved. The functional outcomes at discharge and telephonic follow-up at 3/6 months were assessed using modified Rankin scale (mRS). Result: 39 patients were included in our study. In these patients, CVST score was correlated with various clinical, radiological, and outcome parameters. CVST scores correlated well with the duration of onset of symptoms (p < 0.05) and seizure (p < 0.05). However, no correlation was observed between CVST score and outcome. Conclusion: In our study, CVST score did not correlate with the outcome parameters and it alone may not be an ideal scoring system to assess the severity and outcome. However, CVST score in addition to other clinical parameters may serve the purpose and help in formulating a standardised scoring system for better prognostication and management of CVST.

Award ID: 773

Cognitive Decline and its Assessment in Idiopathic Parkinson Disease and its Correlation with Age in PD Patients

Navneet Kumar, Gaurav Gupta, Pooja Shukla, Richa Giri

Neurology, GSVM Medical College, Kanpur, Uttar Pradesh, India

E-mail: neuroindia3@gmail.com

Background and Aim: 1) To assess cognitive decline in Parkinson Disease. 2) Correlation of cognitive decline with age in PD patients. Methodology: Hospital based cross sectional study conducted among 52 patients of idiopathic Parkinson disease, 61 to 80 year of age (fulfilling UKPDS Brain bank criteria) at GSVM Medical college, Kanpurduring Feb 2020- oct 2021 using SCOPA COG scale. Result: Using SCOPA COG score in 52 patients,17 (33%) patients found with declined cognition with mean score 31.23 in age group 61 to 70 and 27.11 in 71 to 80. Range of score in 61 to 70 is 10-43, in 71 to 80 is 10-40, with p value 0.319. There was negative correlation found between SCOPA COG: Assembling Pattern and Age (Years) (rho = -0.37, p = 0.006). for every 1 unit increase in Age (Years), the SCOPA COG: Assembling Pattern decreases by 0.22 units. Conclusion: No significant association found betweenincreased age and overall cognitive decline in Parkinson patients. Significant decline foundin visuospatial function with increasing age in Parkinson disease patients.

Award ID: 774

Apolipoprotein E Genotype Association with the Risk of Hemorrhagic Stroke: A Meta-Analysis

Pradeep Kumar, Manabesh Nath, Shubham Misra, Astha Rai

Neurology, AII India Institute of Medical Sciences, New Delhi, India

E-mail: pradeepguptaneuro@gmail.com

Background and Aim: Apolipoprotein E (ApoE) alleles have been associated with the risk of Hemorrhagic stroke (HS). We conducted a meta-analysis to determine the association of ApoE alleles with HS risk. Methodology: A literature search was performed till April 07, 2022, in various databases using the keywords: (‘Apolipoprotein E’ OR ‘ApoE’ OR ‘ApoE genotype’) AND (‘Single Nucleotide polymorphisms’ OR ‘SNP’) AND (‘Hemorrhagic stroke or ‘HS’ or ‘Intracerebral Cerebral Hemorrhage’ or ‘ICH’). Pooled Odds Ratio (OR) and 95% Confidence Interval (CI) were determined. Statistical analyses were performed using STATA version 13.0. Result: Twenty-two studies with 7209 HS and 23625 control subjects were included in the study. Pooled analyses revealed a significant association of ApoE ε3/3 with overall HS risk [OR = 1.11, 95% CI = 1.03 – 1.19]. However, significant protective association of ApoE ε2/2 [OR = 0.52, 95% CI = 0.35 – 0.77], ApoE ε2/4 [OR = 0.54, 95% CI = 0.33 – 0.89], ApoE ε2/3 [OR = 0.89, 95% CI = 0.80 – 1.00], ApoE ε 4/4 [OR = 0.63, 95% CI = 0.48 – 0.82, I2 = 0%], and ApoE ε4 [OR = 0.78, 95% CI = 0.66 – 0.93, I2 = 65.9%] was observed with HS occurrence. Population sub-group analyses revealed significant association of ApoE ε3/3 with HS risk in Caucasians [OR = 1.11, 95% CI = 1.01 – 1.22]. Conclusion: The meta-analysis indicates that HS risk is associated with only ApoE ε3/3 genotype, and ApoE ε2/2, ε2/4, ε2/3, and ε4/4 had a protective association with HS, suggesting the significant role of ApoE SNPs in the occurrence of HS.

Award ID: 775

Clinical Profile and Predictors of In-Hospital Mortality in De Novo Convulsive Status Epileptic us in the Elderly Populace

Archana Verma, Kiran K, Pooja Pathak

Neurology, All India Institute of Medical Sciences, Munshiganj, Dalmau Road, Raebareli, UP, India

E-mail: archanashiva2010@rediffmail.com

Background and Aim: Elderly constitute the largest and fastest-growing segment among the patients with Status epilepticus. To study the clinical characteristics and the predictors of in hospital mortality in de novo convulsive status epileptic us (CSE) in the elderly populace. Methodology: Seventy seven elderly (≥60 years of age) hospitalized patients with de novo CSE were evaluated for clinical profile, aetiologies and predictors of in-hospital mortality. Result: The average age of the participants in the study was 65.96 ± 6.72 years. In de novo CSE, the most common aetiologies were acute symptomatic in 68.8% of cases, followed by remote symptomatic in 24.7%. In-hospital mortality in the de novo CSE in the elderly was 30 (38.9%) in our series. Stroke was the leading cause of death among them (acute stroke in 23 cases, old infarct in one case), followed by posttraumatic (n = 4), CNS infection (n = 2). On multivariate analysis, it was found variables significantly related to mortality in de novo CSE were low Glasgow Coma Scale (GCS) (AOR = 53.5, 95% CI = 5.17-555.14; p = 0.001) and lack of response to first line treatment (AOR = 0.06, 95% CI = 0.01- 0.50; p = 0.01). Conclusion: In-hospital mortality in de novo CSE patients was linked to a low GCS and a lack of response to first-line therapy. The most efficient strategy to prevent in-hospital mortality in the elderly is to treat de novo CSE promptly and aggressively in the setting of stroke.

Award ID: 776

Alteration in miR-24-3p and miR-186-5p Contributes to Modulation of GABAA Receptors in Focal Cortical Dysplasia (FCD)

Arpna Srivastava, Aparna Banerjee Dixit, P. Sarat Chandra, Manjari Tripathi, Jyotirmoy Banerjee

Neurology, All India Institute of Medical Sciences, New Delhi, India

E-mail: arpnasrivastava@gmail.com

Background and Aim: Enhanced spontaneous GABAA receptor activity is associated with FCD. miRNAs are potentially involved in the regulation of GABAA receptor subunit expression and activity. This study aimed to determine the expression of miRNAs in FCD and to correlate their expression level with mRNA levels of GABAA receptor subunits. Methodology: The expression of GABAA receptor subunits (α1 & α4) and miRNAs (miR-155, miR-186, and miR-24) were evaluated using real-time PCR in surgically resected brain tissues of FCD patients. miRNAs levels were also determined in the serum of FCD patients. GABAA receptor activity was measured using patch-clamp technique Result: Significant increase in GABAR α1 (p < 0.01), GABAR α4 (p < 0.0001) and miR-155-5p (p < 0.0001) and decrease in miR-24-3p (p < 0.01) and miR-186-5p (p < 0.001), was observed in tissue samples of FCD. Expression of miR-155-5p (p < 0.01) was found to be increased also in serum of FCD patients, whereas expression of miR-24-3p and miR-186-5p were found to be unaltered. MiR-24-3p (r = 0.8835; p < 0.0001) and miR-186-5p (r = 0.9206; p < 0.0001) expression were inversely correlated with GABAR α4 protein expression in resected tissues of FCD patients, whereas miR-186-5p (r = 0.6093; p < 0.05) expression was also inversely correlated with GABAR α1expresion. MiR-24-3p (r = 0.5002; p < 0.05) expression in serum was also correlated significantly with GABAA receptor α4 subunit expression in resected tissues of FCD patients. We also observed an increased frequency of GABAA receptor-mediated synaptic transmission in brain samples of FCD patients. Conclusion: Dysregulation of miRNAs and significant negative correlation with GABAA receptor subunits designated a regulatory role for miRNAs in GABAA receptor-mediated GABAergic activity in FCD. Collectively, we provided evidence supporting that alteration in GABAA receptor subunit expression may be mediated in part by microRNAs in FCD.

Award ID: 777

Transcranial Doppler Study in Migraine and Tension Type Headache

Leena Rajani, Sownthariya R, Kannan B, Edwinraj Thomas A

Neurology, Thoothukudi Government Medical College and Hospital, Tamil Nadu, India

E-mail: leena3011rajani@gmail.com

Background and Aim: To use Transcranial Doppler Sonography to determine Hemodynamic abnormalities in patients with Migraine compared with patients who have Tension Type Headache in a tertiary care Hospital Methodology: This observational study comprises of Twenty five Migraine patients and Twenty five Tension type Headache patients (Age Range-20 to 60 years) who were diagnosed according to the criteria of International Classification of Headache-3; participated in the study during the time period from 1 January 2022 to 30 March 2022. Exclusion criteria were -Arterial Hypertension, Diabetes Mellitus, other brain disorders (such as tumor), pregnancy, Metabolic and pulmonary diseases, Alcohol abuse and patients not on any medications Patients gave their informed consent to participate in the study and were recruited consecutively. Transcranial Sonographic Doppler examination was performed with a 2 MHz pulsed Doppler probe and a dedicated pulsed Doppler system (RMS SPECTRA 102 SI. No. AO 3M5 4OP). Mean velocity, peak velocity, Pulsatility index and Resistive Index were measured via Transcranial Doppler sonography in Middle cerebral artery. Result: 1. Mean velocity and peak velocity was higher in patients with Migraine. 2. There was no difference in Pulsatility Index and Resistive Index in both the groups. Conclusion: Findings in our study support the arteriolar vasodilation theory in patients with Migraine. This study highlights the ability of Transcranial Doppler in differentiating Migraine from Tension Type Headache through hemodynamic profile. However, further studies are needed to confirm this association.

Award ID: 778

Role of Pain Related Evoked Potential in Diagnosis of Trigeminal Neuralgia and Trigeminal Neuropathy

Murali Rayani, Appaswamy Thirumal Prabhakar, Atif Shaikh, Deepti Bal, Rajkumar Ponnana, Sanjith Aaron, Mathew Alexander, Jebalin Abigayil G, Anupama Roy, Paul Premkumar, Tharan Suresh

Neurology, CMC Hospital

E-mail: rayini_murali@yahoo.com

Background and Aim: Trigeminal neuralgia (TN) is a common neurological condition that presents with recurrent brief unilateral stabbing pain over the face whereas Trigeminal neuropathy (TNP) is a rare cranial-neuropathy that presents with pain and numbness over the face which should be diagnosed, as etiology of latter can be sinister. Standard electrophysiological tests for Trigeminal neuralgia were technically demanding and unreliable. We aimed to study the role of pain-related evoked potentials (PREP) in the diagnosis of Trigeminal neuralgia and Trigeminal neuropathy. Methodology: It was a retrospective study over 2 years in a tertiary hospital. Patients with Trigeminal neuralgia, Trigeminal neuropathy and normal volunteer were included. Pain related evoked potential was recorded by stimulating skin over malar eminence and recording from the cortex at Cz. Result: Total of 47 subjects were included of which 30 were normal, 14 had Trigeminal neuralgia and 3 had Trigeminal neuropathy. Mean pain related evoked potential (PREP) amplitude was 67 (21.5) microvolt in the normal nerves and 15 (15.7) microvolt in Trigeminal neuralgia and the difference in the mean was statistically significant (p = 0.025). ROC curve analysis was done using the PREP amplitude and the area under the curve was 0.68. To differentiate the normal from Trigeminal neuralgia the optimal cut-off point for PREP amplitude was 33 microvolt with sensitivity and specificity of 73% and 70% respectively. Pain related evoked potential was not elicitable in trigeminal neuropathy. Conclusion: Pain related evoke potential (PREP) can differentiate between Trigeminal neuralgia and trigeminal Neuropathy. PREP amplitude was lower in Trigeminal neuralgia when compared to normal subjects.

Award ID: 779

Bruxism- A Vascular Cause

Praveen Annadata, Shobhana N, Sadeesh Kumar V, Selva Kumar C.J.

Neuromedicine, Coimbatore Government Medical College and Hospital, Coimbatore, Tamil Nadu, India

E-mail: praveenannadata1989@gmail.com

Background and Aim: Background: Bruxism is a diurnal or nocturnal oral parafunctional activity, including clenching, bracing, grinding or gnashing movements of the teeth. It can lead to toothwear, headaches and depression. Methodology: Case presentation 35 yr female, who habitually snuffs tobacco, without any comorbidities presented with sudden onset fall with transient loss of consciousness for about 2 mins. On awakening, there was paucity of movements of all four limbs and speech, with minimal response to environmental stimuli, with preserved visual tracking movements. She subsequently developed grinding movements of teeth which were present during wakefulness, and reduced during sleep. Result: InvestigationsRoutine blood investigations were obtained. MRI Brain revealed bilateral acute infarcts in the parasagittal frontoparietal regions with occlusion of an Azygous Anterior cerebral artery (ACA). TreatmentShe was treated for acute stroke and was given clonazepam, levodopa and trihexyphenidyl which led to resolution of her bruxism. Conclusion: The estimated prevalence of bruxism is between 5–25%. 1 While bruxism has been associated with a number of neurological diseases, it has not been highlighted following cerebral infarction. It has been previously reported in a patient with bilateral basal ganglia infarcts. 2 It has also been reported in a hispanic women with moya moya disease, who had bilateral ACA and Middle cerebral artery infarcts.3 Similarly, our patient also had bilateral ACA infarcts due to occlusion of an azygous ACA. Various drugs have been used to treat bruxism like benzodiazepines, baclofen, levodopa, zolpidem, bromocriptine, metoclopramide with varied results. Refractory cases have been found to respond favourably to botulinum toxin injection into the masseter. In our patient a short course of clonazepam, levodopa and trihexiphenydyl was tried with alleviation of her symptoms.

Award ID: 780

Covid 19 Vaccine Triggered Relapsing Immune Dysregulation-An Observational Study

Chandra S R, Thomas Gregor Isaac, Abhirami M A

Neurology, S.K. Hospital, Trivandrum, Kerala, India

E-mail: drchandrasasi@yahoo.com

Background and Aim: Vaccination efficacy and safety needs field years. Hospital based study done at Trivandrum analysed patients who presented with immune mediated disorders in temporal correlation to COVID19 vaccine. Methodology: Study period 20 months March 2020 - November 2021; 3235 patients; 1007 (31.12%) patients had vaccination. Single dose in 511 (50.7%) and both 496 (49.3%). All patients were evaluated with imaging, CSF, evoked responses, serology, ANA, TPO, RA and autoimmune panel in affordable only. Those with H/O previous neurological illnesses were excluded. Chi-Square test was utilised for determining the association between Neurological sequelae and the type of vaccine administered. Data was compared between Covaxin and Covishield as only 2 patients had taken Sputnik. Result: 18 had Neurological sequelae and 16 had Monophasic pattern. Recurrent autoimmune features were seen in 2 patients who took sputnik vaccine. Anti- MOG associated recurrent optic neuritis in one patient and tongue atrophy, pyramidal signs followed by ulcerative colitis and arthritis after 2 months with C-ANCA + after Sputnik. The association between neurological manifestation and indigenous vaccines (Covishield and Covaxin) was checked and the Chi square (X2 = 0.0466) and (p = 0.8) and there was no significant association of Neurological sequeale meaning both the vaccines are safe. Conclusion: Vaccination is an effective public health intervention. But, it can also probably trigger a recurrent and multisystem event by immune dysregulation in individuals with genetic susceptibility. The association is a postulate needs longer, larger follow up with genetic study of silent predisposing factors in individuals with family history of autoimmune disorders, which is important to ensure safety.

Award ID: 781

Utility of F-Wave Studies in Facial Neuropathy

Ponnana Raja Kumar, Prabhakar Appaswamy Thirumal, Atif Shaikh, Anupama Roy

Department of Neurological Sciences, Christian Medical College and Hospital, Vellore, Tamil Nadu, India

E-mail: drraj64505@gmail.com

Background and Aim: Electrophysiological evaluation of the facial f-wave is a useful but uncommonly performed test in the evaluation of facial neuropathy. It may be used as an early diagnostic tool in the evaluation and prognosis of facial neuropathy. There is limited data on normative values or their utility. We aimed to study facial F-wave recordings in early facial neuropathy (< 7 days from symptom onset). Methodology: Patients with an established clinical diagnosis of facial-neuropathy within one week of symptom onset and normal volunteers, were recruited. All subjects underwent NCS which included facial nerve and facial f-waves recording. F-wave minimum latency and the latency and amplitude of the Facial CMAP were obtained. Result: The total cohort included 58 subjects with a mean age of 38.9 (15.7) years of which 27 (46%) were males. A total of 106 facial nerves were studied. The mean CMAP amplitude and F latency were 5.2 (1.4) mV and 10.3 (2.5) ms in the normal nerves (p-value - < 0.01) and 3.7 (1.9) mV and 19.6 (6.0) ms in facial neuropathy (p-value - 0.016) respectively with statistically significant p values. In ROC analysis the area under the curve was 0.89 for F-min latency in making a diagnosis of Facial neuropathy. The optimal cut-off point identified was 13.5 msec. At this cut-off point, the sensitivity and specificity for making a diagnosis of facial neuropathy were 81.3% and 97.3%. Conclusion: Facial F-wave recording is a reliable electrophysiological test in the early detection of facial neuropathy.

Award ID: 782

Profile of Strokes in First and second Waves of COVID-19: A Study from Tertiary Care Centre

Rajendra Kumar Sureka, Ishank Goel, Amit Agarwal

Neurology, Mahatma Gandhi Medical College, Jaipur, Rajasthan, India

E-mail: rsureka@rediffmail.com

Background and Aim: COVID-19 (Novel corona virus) continues to wreak havoc across China, European countries, USA, India and now seems to be heading towards the fourth wave. The aim of this study was to explore the association between neuroimaging findings of brain, COVID-19 infection and non COVID-19 patients who presented with neurological manifestations during the first and second waves. Methodology: The present study is a retrospective, hospital-based, descriptive study of neuroimaging findings (NCCT head, HRCT thorax) in COVID-19 and non COVID-19 patients admitted with neurological manifestations in the Department of Neurology at a tertiary care centre in Rajasthan (India) between July 2020 till June 2021. Result: The average age during the second wave was less as compared to the first wave (53.87 vs 63.26 years) along with male preponderance during both the waves of COVID-19. Olfactory dysfunction (26.0% vs 35.9%), headache (47.8% vs 12.8%), altered sensorium (78.3% vs 17.9%), paresis (87.0% vs 51.3%), seizures (17.4% vs 2.6%), dysarthria/aphasia (87.0% vs 56.4%) were found to be the major symptoms during both the waves at presentation. Overall, ischemic infarcts were the most common finding in 50% patients. Patients who presented with encephalopathy had a poor prognosis. Conclusion: The most common neurological manifestations in COVID-19 patients were headache, olfactory dysfunction, strokes, altered mental status and seizures in both the waves. Among ischemic stroke, large vessel occlusion was more common during both the waves of COVID-19 infection. Deaths were more common in COVID-19 positive patients with stroke than in non-COVID-19 patients.

Award ID: 783

A Rare Case of Rapidly Expanding ACOM Aneurysm With CNS Vasculitis Presenting as Stroke and Intractable HICCUP

Dinesh Chaudhari, Pushpendra Nath Renjen, Nishchint Jain, Abhas Kumar

Neurology, Indraprastha Apollo Hospital, New Delhi, India

E-mail: dineshneurology@hotmail.com

Background and Aim: The main culprit lesion causing hiccup in patients with ischemic stroke is thought to involve the medulla oblongata, but some cases of hiccups caused by damage to the supratentorial cortex have been reported. Methodology: A 44 years old gentleman presented intractable hiccups, after a few weeks he developed a sudden onset of right-sided weakness. On examination, the patient had extensor right plantar response with motor weakness (3/5) of the right upper and lower limb. Result: An immediate MRI brain revealed a left MCA acute infarct. MR Angio of brain vessels was suggestive of a huge saccular left ACOM aneurysm (47*37 mm) with the altered intensity of 18*16.5 mm around the aneurysm suggestive of the false lumen with partial thrombosis. The aneurysm was not present in the MRI brain done for transient headache one year back. A cerebral DSA was performed which revealed circumferential eccentric multifocal areas of enhancement in left MCA branches and similar bilateral patchy vessel wall enhancements; suggestive of CNS vasculitis. Endovascular coiling of saccular left ACOM aneurysm was performed and the patient was treated with five days of pulse therapy of high dose IV Methylprednisolone. The patient has a complete resolution of hiccups and right-sided weakness improved significantly. The patient has not experienced any new neurological symptoms on long-term follow-up. Conclusion: We report a rare case of rapidly expanding ACom aneurysm with CNS vasculitis presenting as stroke and intractable hiccup.

Award ID: 784

Impact of Primary Dysmenorrhea on Postural Sway among Medical Undergraduate Students- A Comparative Pilot Study

Ishani Prasade, Nawaj Mehtab Pathan, Chandan Kumar

Physiotherapy, MGM School of Physiotherapy Aurangabad, Maharashtra, India

E-mail: ishaniprasade@gmail.com

Background and Aim: Primary Dysmenorrhea is considered the most important issue among college-going students. This is mainly identified as abdominal pain, backache, and muscle cramps, and believed that it is under influence of the estrogen hormone the muscle tone and their elastic properties get alters along with soft tissue laxity. These collectively result in postural control difficulties during the menstrual phase which leads to an increase in postural control difficulties. This study is intended to find out the postural alterations during menstruation among college-going undergraduates having a history of primary dysmenorrhea. Methodology: This pilot study comprising of 10 participants, who are aged between 16-and 25 years old medical graduates. The study participants were randomly divided into 2 groups where Group one had primary dysmenorrhea and group two had no history of primary dysmenorrhea. All study participants were assessed by administrating 1 minute Romberg test with eyes open and closed on the Balance Master Force Platform. © C90 area, Romberg Quotient, Std X & Y deviations, Trace Length & Std Velocity were the parameters considered for analysis purposes. Result: The Balance Master Force Platform© parameters such as C90 area and Romberg Quotient i.e. area of postural sway and impact of visual input on balance respectively were significant and indicating, there is an increase in postural sway among girls suffering from primary dysmenorrhea. Conclusion: There are noteworthy postural alterations that occur during dysmenorrhea. This study also concludes that blocking visual output escalates more postural sways.

Award ID: 785

To Study the Spectrum of Hospital Acquired Infections in Patients Admitted in a Neurology Setting

Raghav Kumar, Pradeep Kumar Maurya, Ajai Kumar Singh, Abdul Qavi, Manodeep Sen

Neurology, DR.RMLIMS, Lucknow, Uttar Pradesh, India

E-mail: raghav.sem@gmail.com

Background and Aim: Health care-associated infections (HAIs) has pronounced significance on morbidity and mortality among neurologically injured patients (1,2) The current study aimed to review such infections in a neurology unit and identify the most prevalent causative agents and burden of such infections on patient outcome. Methodology: This prospective study was carried out in a tertiary care teaching hospital. Patients with hospital acquired infections were broadly classified according to site of infection acquired (Urinary tract, Pulmonary system, Skin and soft tissue infections, Blood stream related infections, Device related infections)[3] Culture and sensitivity from site of infection was sent to determine microbiological etiology and estimate diversity of microorganisms. Patients were followed throughout the entire length of stay at the hospital to assess the outcome. Result: Amongst 50 cases, Urinary system involvement was the most prevalent. Overall prevalence of Urinary system, Hematological system & Pulmonary system involvement was found to be 44%, 24% & 38% respectively. Amongst Urinary system involvement (n = 44), most commonly detected pathogen was E coli (13.6%) followed by Candida & Enterococcus (11.4%). Amongst 11 blood stream related, Staphyloccus, Acinetobacter & Coagulase negative Staph, was detected among 27.3% cases each. Most cultured organisms from ET were Actinetobacter baumanii & K pneumoniae. Multiple sites of infection were found among 24% of cases. Mean average length of hospital stay was 22.8 days. Mortality was prevalent among 14% of cases. Conclusion: Most prevalent site of infection was the urinary system followed by pulmonary system and haematological infections. Multi-site infection was detected in 24% of cases. A greater length of stay at the hospital and mortality of 14% was detected in patients who developed hospital acquired infections.

Award ID: 786

An Observational Study of Etiological, Clinical and Radiological Profile of Noncompressive Myelopathy in Tertiary Care Centre of Central India

Nitin Agrawal, Ritu Gupta, Pawan Soni

General Medicine, NSCB MCH Jabalpur, Madhya Pradesh, India

E-mail: nitin.agrawal13295@gmail.com

Background and Aim: Aim-To Study Etiological, Clinical and Radiological Profile of Noncompressive Myelopathy In Tertiary Healthcare centre of Central India Methodology: Study Design-Observational study. Sample Size-50. Study Period-Oct 2020 till January 2022. Study Area-N.S.C.B MCH JABALPUR. Study tools-Detailed History, Clinical Examination and MRI Spine and Brain. Special Investigations-EMG, VEP AND NMO Antibody. All patients with age above 15 who were admitted with etiology as Noncompressive Myelopathy were included in study. Result: 50 Patients with Noncompressive Myelopathy were studied from Oct 2020 till January 2022 At N.S.C.B MCH Jabalpur of Which 33 were Men and 17 were Women. Hospital Incidence was 0.25/1000. Presentation was acute in 24%, Subacute in 56% and Chronic in 20%. Majority had progressive illness. All patients presented with Motor weakness. 75% had numbness and Tingling and 50% had Urinary symptoms. 72% had Plantars as Extensors and rest being mute. Tone was increased in 64% and was decreased in rest. DTR were Exaggerated in 72% and rest had Diminished Reflexes. In MRI Study 74% of patients had Demyelination, majority had long segment involved at Cervical level. As per etiology is concerned Maine cause of Noncompressive myelopathy were Multiple Sclerosis and NMOSD followed by Idiopathic Transverse Myelitis and ALS followed by SACD. Other rare causes included Vascular cause, MOG associated Myelopathy, TB Myelitis and HIV Associated Myelopathy. Conclusion: 1) Multiple Sclerosis and NMOSD were the most common cause followed by Idiopathic Transverse Myelitis and ALS. 2) Majority had Demyelination in MRI of spine involving Long segment and at Cervical level.

Award ID: 787

A Study of Genetically Confirmed Limb Girdle Syndromes

Jyothsna Gedda, Butchi Raju Garuda

Neurology, Andhra Medical College, Visakhapatnam, Andhra Pradesh, India

E-mail: jyothsnapavanigedda@gmail.com

Background and Aim: In the study, we define limb girdle syndrome as chronic progressive proximal weakness of upper limbs/lower limbs/both. Our aim is to study the epidemiology, clinical, laboratory profile of such patients presenting with limb girdle syndrome to tertiary care hospital, KGH and to perform targeted genetic analyses. Methodology: STUDY POPULATION: Consisted of patients with limb girdle syndrome presenting from April 2019 to April 2022 with chronic progressive (>1 yr) proximal weakness of both upper limbs/lower limbs/both with age of onset before 50 yearsSTUDY DESIGN: Cross-sectional hospital-based observational study SAMPLE SIZE : 22 Result: Out of the total 22 patients, 17 (77.27%) were males and 6 (27.27%) were females. Mean age of onset of the disease was 10.6 years of age. There was developmental delay observed in 5 out of 22 (22.7%) patients and a history of consanguineous parentage in 8 out of 22 (36.3%). One out of the 22 (9.09%) patients is non-ambulatory at present. The genetic diagnoses were DMD/BMD in 7 of 22 (31.8%), LGMD in 8 of 22 (36.36%), Emery Dreifuss muscular dystrophy in one of 22 (4.54%), Myotonia congenita in one of 22 (4.54%), Nonaka myopathy in one of 22 (4.54%), Congenital Myasthenic Syndrome in 2 of 22 (9.09%), Spinal Muscular Atrophy in 2 of 22 (9.09%). ECG and Echocardiography was normal in all the cases. The mean CPK was 2931 U/L with the highest observed in DMD. Conclusion: We recommend targeted genetic analysis to diagnose conditions like SMA, Pompes disease, CMS for which proven treatments are available. Genetic analysis also aids pre-conceptional genetic counselling and helps classify the subtype for prognostication.

Award ID: 788

Overview of Necrotising Autoimmune Myopathy with a Focus on the Utility of the Clinico-Serological Profiles in Diagnosis and Treatment with a Rare Case Report

Tamphasana Wairokpam

Neurology, American Oncology Institute, Imphal, Manipur, India

E-mail: drtamphasanaw@americanoncology.com

Background and Aim: BACKGROUND: Necrotizing autoimmune myopathies (NAM) are relatively new and increasingly recognised subset of myopathies. Patients generally present with subacute onset of proximal myopathy and significantly elevated creatinine kinase (CK) levels. This review and case report on NAM aims to highlight the heterogeneity of this entity and focus on the distinct clinical presentation, biopsy findings, specific auto-antibodies implicated and available treatment options with prognosis. Methodology: Meta-analysis of literatures and a case report outlining the clinical presentation, biopsy findings, antibodies implicated and management of a patient with NAM. Result: Two biomarkers, anti- Signal recognition protein (SRP) and anti- hydroxyl methylglutaryl-coenzyme A reductase (HMGCR) Abs, have been found to have an association with NAM in about 2/3rd of cases. Biopsy shows muscle fibre necrosis without inflammation. There are reports of Statin induced NAM where progression of myopathy has been seen even after discontinuation of Statins, pointing towards an underlying immune mechanism. Most cases are refractory to corticosteroid monotherapy. Immunosuppressive therapy with other immunotherapeutic agents has a role in the treatment of NAM. Conclusion: CONCLUSIONS: Given the heterogeneity of NAM, it appears that NAM is not just a single entity but consists of many different forms and its classification and a thorough understanding of underlying mechanism and clinic-serological profile remains an evolving field. Clinical correlation with antibodies associated in NAM is essential for efficacious management and disease prognostication.

Award ID: 789

α4-Containing GABAA Receptors as Therapeutic Target in Drug-Resistant Epilepsy Pathologies?

Jyotirmoy Banerjee, Devina Sharma, Ramesh Doddamani, M. C. Sharma, P. Sarat Chandra, Manjari Tripathi, Devina Sharma, Sanjeev Lalwani, Aparna Banerjee Dixit, Ramesh Doddamani, M. C. Sharma, Sanjeev Lalwani, P Sarat Chandra, Manjari Tripathi

Biophysics, All India Institute of Medical Sciences (AIIMS), New Delhi, India

E-mail: jyotirmoybanerjee1@gmail.com

Background and Aim: GABAA receptor is hetero-pentameric protein consisting of 2α, 2β, and a δ/γ subunit. GABAA receptors are target for anti-seizure drugs like Benzodiazepines (BZDs). BZDs bind at the interface of α-and γ- subunit of the GABAA receptors, with selectivity of BZD determined by the type of α subunit. α1-, α2-, α3-, and α5-containing GABAA receptors are sensitive to BZDs, while α4-, and α6-containing GABAA receptors are insensitive to BZDs. Here we investigated altered subunit configuration of GABAA receptors in various drug-resistant epilepsy (DRE) pathologies and its possible contribution to pharmaco-resistance. Methodology: Cortical specimens were obtained from patients with various DRE pathologies like temporal lobe epilepsy, cortical dysplasia, Rasmussen’s encephalitis, dysembryoplastic neuroepithelial tumor and ganglioglioma undergoing resective surgery. Differential gene and cellular expression of GABAA receptor subunits were evaluated using qPCR and immunohistochemistry. Whole-cell patch-clamp recordings were performed on pyramidal neurons of cortical samples of DRE patients to measure the effect of BZD-binding site antagonist, flumazenil, on spontaneous GABAergic activity. Result: We observed upregulation of α4-containing GABAA receptors both at mRNA and protein level in DRE samples as compared to controls, while α1-containing GABAA receptors remain unaltered. Flumazenil did not affect the kinetics of GABAergic events in samples of DRE pathologies, but significantly reduced the frequency and amplitude of GABAergic activity recorded from pyramidal neurons of non-seizure control samples. Conclusion: Our results suggest that α4-containing GABAA receptors may be predominant in patients with DRE, which could contribute to BZD resistance in these patients. Further, α4-containing GABAA receptors could be potential therapeutic target for DRE pathologies.

Award ID: 790

Central Nervous System Involvement in Chronic Myeloid Leukemia - A rare complication

Surekha Dabla, Neeraj Kumar, Kiran Bala

Department of Neurology, PGIMS Rohtak, Haryana, India

Introduction: Chronic myeloid leukemia (CML) is a clonal myeloproliferative disorder characterized by the presence of a reciprocal translocation between the long arms of chromosomes 9 and 22, t(9;22)(q34:q11)(Philadelphia chromosome) leads to the fusion of the break point cluster region (BCR) with the ABL gene, which forms an oncogene, the transcript of which is an oncoprotein with a tyrosine kinase (TK) function. Methodology and Results: A 38 yrs old lady diagnosed in year 2020 with chronic myeloid leukemia (CML-chronic phase). Initially she was started on Dasatinib one month later she started having dyspnea and generalised weakness then Dasatinib stopped. This time her CH showed Hb-11.1gm/dL, TLC 3800/cumm with 2% blast, Platelet counts- 2,60000 lacs/cumm. Her peripheral smear done suggested Lymphoid blast crisis B cell type. She was switched on Nilotinib, 2 weeks later no blast and it continued. She remained stable till 30/1/2021. Then again she landed in blast crisis (27%), started on Dasatinib 70 mg twice a day with prednisolone 80 mg tapered to 30 mg after one month, she improved no Blast in peripheral smear. Her LFT/ KFT normal. BCR ABL 91.95% with Cytogenetic 46 xx (t9:22), Imatinib resistance analysis (IRMA) showed resistance to both Imatinib and Nilotinib. In view of this she received two doses of High dose MTX 3 gm/m2 at 2 weeks interval. around 3 months later she underwent Allogenic BMT. She tolerated well with no GVHD, In august 2021 she developed cyclosporin induced high blood pressure and amlodepin was added and again oral Ponatinib was started. In oct 2021 She had sudden onset B/L vision loss with vertigo and papilledema her MRI Brain s/o extra axial dural based homogenously enhancing lesion along with left tentorium and right high frontal dural leukemic deposits and perioptic sheath prominent. This time in view of Lymphomatous Meningitis IT-Cytarbine 50 mg given by intraventricular route (shunt placed) for maintenance doses. In Feb 2022 she suddenly developed seizures and started on oral carbamazepine and was doing well till May 2022 when she had altered sensorium, confabulation, loss of inhibition, apraxia, aphasia, and excessive sleep. MRI brain done [Figure 1]. Her Serum sodium and Serum carbamazepine levels were. CSF was negative for neuroviral panel, without any blast cells and NMO, MOG and autoimmune panel was also negative. She was started on Methyl prednisolone and IT MTX 12.5 gm given. Presently patient is alive, accepting orally but has severe cognitive decline and vision loss. CNS blast crisis usually presents with headache and vomiting, and their presentation should prompt clinical suspicion of CML relapse. Papilledema remains the most common finding on fundoscopy, and leptomeningeal enhancement is the most common finding on imaging. however, it occurs with greater frequency in ALL than in myeloid leukaemia. Dasatinib is a second-generation inhibitor of tyrosine kinases SRC and BCR-ABL that has been used in the treatment of imatinib-intolerant or -resistant Ph+ leukaemia and since it crosses the blood brain barrier it is considered as an effective therapy for Ph+ CNS leukemia. However why some patients fail to respond or having CNS blast crisis the mechanisms could be nonadherence, acquired mutations in the BCR/ABL fusion protein, the expression of TKI efflux systems by tumor cells, and failure of epigenetic silencing of the BCR/ABL transcript. In our patient’s despite of regular chemotherapy with Allogenic BMT she had a very severe CNS relapse and presently in cytogenetic and complete hematological response but clinically she had complete vision loss with severe cognitive decline. Conclusion: CML patients rarely present with a CNS blast crisis, mostly reported in patients treated with imatinib due to the poor penetration of the drug through the blood-brain barrier. We report a patient undergoing treatment for CML with various TKIs except Imatinib and allogenic BMT had a very unusual presentation of relapsed disease, presenting with CNS involvement, despite compliance to therapy.

Award ID: 791

Brown-Vialetto-Van-Laere Syndrome: A Report of Two Cases

Swati Parida, Deepika Joshi, Anand Kumar, Rameshwar Nath Chaurasia, Varun Kumar Singh, Vijaya Nath Mishra, Abhishek Pathak

Neurology, IMS BHU, Varanasi, Uttar Pradesh, India

E-mail: swatiparida1989@gmail.com

Background and Aim: Brown-Vialetto-Van Laere Syndrome (BVVLS) is a rare neurodegenerative disorder characterized by progressive pontobulbar palsy and sensorineural deafness (SNHL). Mutations in the genes SLC52A2 and SLC52A3 encoding riboflavin transporter RFVT2 & RFVT3, respectively are seen in BVVLS. Methodology and Results: We report two cases, a 13 year old boy and 9 year old girl who presented with gradually progressive hearing loss for one year. The boy and girl had bilateral facial weakness, difficulty in swallowing, moving the tongue sidewise and slurring of speech for 8 months and 6 months, respectively. Two siblings of the girl had expired in the first decade due to pneumonia. On examination anthropometric measurements were normal in both cases. Central nervous system examination revealed VII, VIII, IX, X and XII cranial nerve involvement with diminished deep tendon reflexes in all four limbs in both cases. Girl had postural tremors of both hands and atrophy of thenar muscles. Sensory examination was normal. Baseline investigations like complete hemogram, renal and liver function test, serum creatine kinase were normal. MRI of brain, cervical spine and nerve conduction studies were normal. Electromyography revealed a neuropathic pattern with chronic denervation and reinnervation changes in facial muscles. Pure tone audiometry revealed bilateral SNHL. A heterozygous missense variation in exon 2 of the SLC52A3 gene and SLC52A2 gene was detected in whole exome sequencing. They were started on 25 mg/kg of riboflavin. On follow up visit at three months, they reported improvement. Conclusion: It is important to identify BVVLS at early stage as it responds to high doses of riboflavin.

Award ID: 792

Tuberculous Longitudinally Extensive Transverse Myelitis – Review of 6 Cases: A Case Series

Shreyasi Banerjee, Indranil Dutta, Alapan Paul, Debsadhan Biswas

Neurology, Medical College, Kolkata, West Bengal, India

E-mail: shreyasibanerjee100@gmail.com

Background and Aim: Longitudinally extensive transverse myelitis (LETM) affects three or more vertebral segments on spinal magnetic resonance imaging (MRI). Mycobacterium tuberculosis is an extremely rare cause of LETM. We aim to study the clinical and radiological profile of tuberculous LETM. Methodology: This study was conducted in the Neurology department, Medical College, Kolkata from March 2020 to March 2022. The data of 6 cases of LETM of tubercular origin (diagnosed by clinical examination, CSF analysis, Imaging) were collected and their clinical profile, imaging & outcome were analyzed. Compressive etiology was ruled out by imaging. Result: Among 6 (n) patients, 5 were male with mean age 28.8 ± 9.54. One patient was immunocompromised with very low CD4 count. Two had miliary Pulmonary TB and one TB lymphadenitis. All had dorsal cord involvement with spinal meningeal and roots enhancement. Lesions extended over 5 or more segments in all. On contrast MRI Brain, 5 had meningeal enhancement and among them 3 had brain tuberculoma. One patient had spinal tuberculoma only. 4 patients (3 immunocompetent) presented with myelitis, as manifestations of paradoxical reaction after starting anti tubercular therapy. Most of them had very high CSF protein (>1 gm/dl). CSF CBNAAT was negative in all patients. Five patients responded markedly to steroid and ATD as started early in disease course. Conclusion: Tuberculous LETM commonly involve dorsal cord in young immunocompetent male and usually associated with other CNS TB manifestations. It occurs commonly due to paradoxical reaction after starting ATD. Early diagnosis and therapy may predict outcome.

Award ID: 793

The Prevalence and Clinical Phenotype of Dual Positive Neuromyelitis Optica Spectrum Disorders (NMOSD) at a National Reference Centre

Fida Faisal, Anjali Yadav, Sudheeran Kannoth, Vivek Nambiar, Sibi Gopinath, Anand Kumar, Saraf Udit Umesh, Jino Vincent, Sruthi Anoop, Annamma Mathai, Suprabha Panicker

Neurology, Amrita Institute of Medical Sciences

E-mail: falsafa711@gmail.com

Background and Aim: Neuromyelitis Optica Spectrum Disorders (NMOSD) is an autoimmune syndrome that shows positivity for Aquaporin 4 (AQP4) IgG or Myelin Oligodendrocyte Glycoproteins (MOG) IgG. Dual positivity is rare. We aim to assess the prevalence of dual positive NMOSD and outline its clinical phenotype. Methodology: This is a retrospective cross-sectional study conducted at a tertiary healthcare center between 2018 and 2021. The serum or CSF samples of suspected cases of NMOSD were tested for both AQP4-IgG and MOG-IgG using Indirect immunofluorescence test on transfected cells. Result: 1935 cases of NMOSD were tested for both antibodies- 64 patients (3.3%; 57 females and 7 males) tested positive for AQP4-IgG, 217 patients (11.21%; 122 females and 95 males) tested positive for MOG-IgG and 3 patients (0.15%; 2 females and 1 male) showed dual positivity. There was a strong female preponderance in all three groups. This study identified 3 patients with dual positivity. The first patient (42 years, Male) presented with area postrema syndrome initially and relapsed by developing right-sided numbness of the temporal area and limbs. The second patient (27 years, Female) presented with bilateral optic neuritis (left > right) initially and relapsed following an episode of a seizure with left-sided hemiplegia and right-sided facial deviation. The third patient (25 years, Female) initially presented with acute bilateral optic neuritis and later developed left-sided hemiplegia post-recovery. Plasmapheresis and/or rituximab injections produced a better response than management with methylprednisolone. Conclusion: We conclude that the prevalence of dual positive NMOSD is 0.15% and its clinical phenotype is more similar to NMO rather than MOG- associated disease.

Award ID: 794

Subcellular Therapies Improve the Outcome of Ischemic Stroke in Preclinical In-Vivo Studies: A Meta-Analysis

Sunil K. Narayan, Simy Grace Cherian

Neurology, Jawaharlal Institute of Post Graduate Medical Education and Research (JIPMER), Puducherry, India

E-mail: sunil.narayan@jipmer.edu.in

Background and Aim: Subcellular therapy has been theorized to be safer, more effective, and less cumbersome in replacing stem cell therapies for tissue repair and regeneration. Exosomes administration has been found effective in the treatment of cancers, diabetes and renal disease both in in-vivo and clinical studies. However, there remains considerable uncertainty on effectiveness and safety of this therapy for cerebral infarction. In a first of such an exercise, we aimed to systematically explore the evidence from preclinical studies in rodent models. Methodology: We reviewed existing literature on exosome therapy in rodent stroke models from EMBO, Web of Science, PubMed, EMBASE, ProQuest, SCOPUS, and Cochrane reviews, and reviewed the participant characteristics, interventional measures, and outcome measures systematically, with changes in the infarct volume and functional scores as outcome parameters. The quality of individual studies was assessed by the CAMARADES checklist. 17 studies were found qualitatively acceptable. We used RevMan v5.3. Result: Of 7 controlled studies, with administration of exosomes within 24 hours of stroke induction, the standardized mean difference in infarct volume was 3.02 [95% CI:1.68,4.36] ML; P < 0.0001) and Heterogeneity test, I2 82%. For four studies with exosomes after 24 hours of stroke, the difference was 1.21 [0.00,2.42], P = 0.05, where heterogeneity, I2 = 59%. On Foot-fault test, difference on D28, 5.30 [4.01, 6.77], P < 0.00001 with I2 of 0%. For enriched exosomes D28 difference 6.44 [3.81, 9.06], P < 0.00001, I2 = 8%. Safety assessments were seldom addressed. Conclusion: The pooled analysis showed effectiveness compared to the controls for reducing infarct volume and improving functional recovery. But, there was insufficient data to draw robust safety conclusions. Further preclinical studies should be done to higlight safety and enhancement methods, before translation into human trials.

Award ID: 795

A Rare Case Report of CMT4C Presenting with Trigeminal Neuralgia

Swati Parida, Deepika Joshi, Anand Kumar

Neurology, IMS BHU, Varanasi, Uttar Pradesh, India

E-mail: swatiparida1989@gmail.com

Background and Aim: Charcot–Marie–Tooth disease (CMT) is a group of clinically and genetically heterogeneous hereditary motor and sensory neuropathies. CMT4C is one of the most common forms of autosomal recessive demyelinating neuropathies caused by a mutation in the SH3TC2 gene. Typical clinical features include early-onset distal motor and sensory neuropathy, scoliosis, and cranial nerve involvement. Methodology: Case report: A 46 years old male presented with sudden onset excruciating pain on his left side of face lasting for few seconds that aggravated on chewing and speaking without any sensory loss for 5 days. He had gradually progressive imbalance while walking, high steppage gait, painless hearing loss in both ears and bilateral symmetrical postural tremors for last 20 years. He had difficulty in sitting with both feet on the ground together; he would tend to fall backwards if he assumed this posture for last 30 years. He had one episode of generalized seizure before 1 year. On examination he had pes planus, kyphoscoliosis, short stature, shortened tendoachilles. There was no nerve thickening. His power at hip, knee and ankle joints was 4/5 and 5/5 in both upper limbs. Sensory examination revealed impaired joint and vibration sense below both malleoli with positive Romberg’s sign. Pain and temperature sensation were normal. There was diffuse areflexia. He had bilateral severe sensorineural hearing loss. A homozygous missense variant in exon1 of SH3TC2 gene was detected on gene sequencing. Result: None Conclusion: Our patient had atypical presentations. It is crucial to know the subtype of CMT to provide valid prognosis.

Award ID: 796

Study to Determine the Proportion of Amyotrophic Lateral Sclerosis Patients Having Non Motor Symptoms

Arpit Agrawal, Jyoti Garg, Kuljeet Singh Anand

Neurology, Atal Bihari Vajpayee Institute of Medical Sciences and Dr Ram Manohar Lohia Hospital, New Delhi, India

E-mail: arpit.10dec@gmail.com

Background and Aim: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons. The pathology is not limited to initially affected motor neurons but may spread to involve other non motor regions of the brain leading to non motor symptoms (NMS). This study was conducted to determine the proportion of ALS patients having NMS and to find correlation between NMS and functional status of the patient. Methodology: It was an observational study conducted at department of neurology, Dr RML hospital, New Delhi. 41 patients of ALS were included as per revised El Escorial criteria. Non motor symptoms were evaluated using NMS questionnaire, included 30 questions of different domains (Gastrointestinal, urinary, cardiovascular, Neuropsychiatric, sleep) and the functional status of patients was assessed using the revised amyotrophic lateral sclerosis functional rating scale (ALSFRS-R). Result: NMS were reported in 92.7% (38 cases) patients. The most prevalent NMS were gastrointestinal (78%), Neuropsychiatric (75.6%), sleep disorder (75.6%). The less prevalent NMS were Cardiovascular (14.6%) and urinary system (9.8%). Mean ALSFRS score in patients with or without non motor symptoms were (32.82 ± 7.24) and (46.0 ± 1.73) respectively (P < 0.05). The difference between mean ALSFRS in these two groups was maintained while comparing each domain separately. Conclusion: we concluded that NMS were very frequent in ALS and their prevalence increases with the progression of disease and resulted into poor functional status. Early identification and management of NMS in ALS will help in rehabilitation and improvement of quality of life.

Award ID: 797

Impact of Various Disability Domains in Patients Who Recovered From GBS

Nirumal Khumar, Thamil Paavai, NIthyanandham, Nandha Kumar, Lakshminarasimhan Ranganathan

Insitute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: nirmalindiaganguly@gmail.com

Background and Aim: Guillian barre syndrome is a immune mediated polyneuropathy that disables a person and contributes to morbidity as much as it contributes to mortality (1). The aim of this study is to assess various factors causing disability in patients with GBS and attempt to quantify the disability based on validated scales. Factors causing disability include pain, motor weakness and psychological factors including depression and anxiety Methodology: We aim to assess the factors causing disability with the help of validated questionnaires concerning various domains causing disability. 50 GBS patients were included in the study, their baseline characteristics were recorded and disability assessment was done at 6 months. Kessler’s psychological score (2) was used for grading psychological disability, pain disability index was used to assess pain disability (3) and modified hughes disability score was used for assessment of motor disability. Statistical analysis of frequencies and correlation between different grades and domains of disability will be done using SPSS version 20.0 Result: Of the patients included in the study 54% patients had severe pain related disability (PDI more than 42), 41% patients had moderate disability (PDI more than 27).9% had only mild disability. Significant improvement in motor function was observed in those who were using physiotherapy at home with a mean improvement in Hughes disability score after 6 months by 1 point Conclusion: Based on the above study it can be concluded that pain and psychological distress contributes to as much disability as motor weakness and earlier initiation of IVIG/PLEX is likely to reduce disability in all domains.

Award ID: 798

Cardiac Arrhythmias in Refractory Status Epilepticus

Deepika Saroha, Samhita Panda

Neurology, AIIMS Jodhphur, Rajasthan, India

E-mail: deepikasaroha91@gmail.com

Background and Aim: Refractory SE (RSE) is a medical emergency associated with increased mortality. However, cardiac complications may also contribute to worse outcomes with increased mortality. We aimed to study the frequency of cardiac rhythm disturbances in RSE. Methodology: All patients with RSE admitted between January 2021 to May 2022 in the Department of Neurology were included. RSE was defined as generalized convulsive or non-convulsive status epilepticus (SE) that continued despite adequate benzodiazepines and at least one antiepileptic drug (AED), irrespective of time. Patients with age <18 years, coronary artery disease, heart failure/cardiomyopathy, congenital heart disease, on pacemaker, and implantable cardiac defibrillator were excluded. Cardiac-specific diagnostic studies (6 hourly ECG, Holter and 2D echo) were obtained. These were reviewed for cardiac arrhythmias classified into ventricular tachycardia/fibrillation, atrioventricular block, atrial fibrillation/ flutter, sinus bradycardia, sinus tachycardia and QTc prolongation Result: We recruited 18 (male:female::1.2:1) patients with RSE with mean age 48.8 range 34- 72 years). Cardiac arrhythmias were observed in 100%. These were ventricular tachycardia/fibrillation (n = 2,11.4%), atrioventricular block (n = 1,5.5%), atrial fibrillation/flutter (n = 1,5.5%), sinus bradycardia (n = 3,16%), and sinus tachycardia (n = 18,100%). QTc was prolonged in 27.7% (n = 5). In-hospital mortality was 50%. Outcome was worse with combination of arrhythmias but difference was not statistically significant. Conclusion: Cardiac injury is common in patients of RSE. The proposed hypothesis is the brain-heart relationship leading to hemodynamic changes due to intense activation of sympathetic nervous system and resulting overstimulation of cardiac adrenoreceptors during seizures. This study highlights the importance of monitoring for cardiac arrhythmias in RSE so as to reduce cardiac-related mortality.

Award ID: 799

Bimelic Symmetric Hirayama Disease – A Mimicker of ALS - Case Series

Dhileeban P, Manivannan M R, Murugan P K, Justin C, Subramanian R

Department of Neurology, Madurai Medical College, Madurai, Tamil Nadu, India

E-mail: drdhileeban24@gmail.com

Background and Aim: Focal amyotrophy, is a condition that presents clinically with juvenile onset of focal atrophy and weakness most commonly of a single hand and arm and rarely of a leg. It’s Male predominant disorder. Recovery doesn’t occur, but considered to be benign since they don’t progress to systemic motor neuron disease. Methodology: Three patients in the adolescent age, presented with insidious and progressive neurological symptoms for more than a years, in the form of flail type weakness and wasting of both hands, first started in one side and progressed to other side without sensory symptoms, cranial nerve/HMF/ cerebellar/ extrapyramidal or bladder symptoms and also without significant past history. Examination showed wasting of bilateral thenar and hypothenar/ lumbricals and flexor compartment of forearm with relatively preserved bulk of extensor compartment, Hypotonia in both upper limb, absent deep tendon reflexes in biceps, triceps and supinator with normal sensory, and normal lower limb, truncal motor system. Minipolymyoclonus present in two patients. Result: Nerve conduction studies showed chronic denervation in form of prolonged latency, impersistence and reduced amplitude with unrecordable F waves in atrophied muscles. EMG showed features of active and chronic denervation. MRI Cervical spine in neutral position showed abnormal T2-weighted signal of the spinal cord at site of maximum forward shift or normal cord intensity. But all 3 patients MRI cervical spine in flexion position showed posterior dural sac crescent appears as high signal intensity on T1- and T2-weighted sequences. Conclusion: Bilaterally symmetric hirayama disease is a severe form of classic disease which remained undiagnosed in most patients. Even though Hirayama disease is irreversible condition, early diagnosis and treatment can reduce the morbidity.

Award ID: 800

Study of Retinal Nerve Fibre Layer Thickness in Parkinson’s Disease and it’s Correlation with the Severity of Disease

Rahul Jain, Kuljeet Singh Anand

Department of Neurology, Jain Hospital

E-mail: drrahul@jainhospital.com

Background and Aim: The retinal nerve fibre layer (RNF) changes have been found in several neurodegenerative diseases, particularly Alzheimer’s disease and Parkinson’s disease. By assessing RNFL thickness with the help of Optical Coherence Tomography (OCT), the early detection of such diseases is possible. With this background, we compared the RNFL thickness of Parkinson’s disease patients with that of healthy individuals. Methodology: A cross-sectional, observational study was conducted at a tertiary care hospital in New Delhi to determine and compare the RNFL thickness in Parkinson’s disease (PD) patients (n = 35) with that of age and sex matched healthy controls (n = 35). All patients from 45-80 years age and fulfilling UKPDS criteria of PD diagnosis were included in the study. RNFL thickness was evaluated using Spectral domain OCT (SD-OCT). Result: In PD patients, the mean duration of disease was 5.7+3.2 years, duration of treatment was 5.6+3.2 years and MDS UPDRS score was 39.7+17.8. RNFL thickness compared between control and PD groups were as follows: temporal superior 111.77+21.74 vs 95.76+34.2 (p = 0.0012); nasal superior 106.74+24.12 vs 93.22+34.37 (p = 0.0079); temporal 57.27+15.17 vs 58.46+18.72 (p = 0.6812); nasal 71.76+22.12 vs 65.13+13.64 (p = 0.0346); temporal inferior 117.33+25.51 vs 103.31+34.22 (p = 0.0068); nasal inferior 105.86+27.78 vs 95.78+32.24 (p = 0.0496); and average global RNFL thickness 87.47+11.25 vs 79.41+18.58 (p = 0.0023). There was a very weak negative correlation of average global thickness with severity of PD using MDS-UPDRS 3 score (r = -0.1379, p = 0.255). Conclusion: With this being the first study of its kind on Indian population, we found overall thinning of RNFL in the PD patients than the age and sex matched controls. RNFL thinning was significant in all quadrants except the temporal quadrant. But correlation between RNFL thickness and severity of PD was very weak negative one. In order to show that RNFL thinning occurs in PD patients and is a predictive factor, longitudinal studies in healthy persons progressing to PD should be conducted.

Award ID: 801

A Cross Sectional Study to Evaluate the Correlation of Gross Motor Function, Hand Function and Caregiver Reported Quality of Life with Intelligence in Children with Cerebral Palsy

Shiv Yadav, Fathima Haneena P

Physical Medicine & Rehabilitation, AIIMS, New Delhi, India

E-mail: slyaiims@yahoo.co.in

Background and Aim: Cerebral palsy (CP) is a group of permanent, but often changing disorders of movement and posture and motor function. Intellectual disabilities are common in cerebral palsy. The objective of this study is to assess the correlations of gross motor and hand functions with intelligence quotient (IQ) in children with CP. Methodology: CP children of the age group 6 to 12 years were enrolled after taking informed consent. All patients underwent detailed physical examination. Motor function and hand function were assessed by Gross Motor Function Classification CP children of the age group 6 to 12 years were enrolled after taking informed consent. All patients underwent detailed physical examination and Gross Motor Function Classification Result: 112 CP children were enrolled in the study. The mean age was 8. 24 ± 1.75 years. 75% children were male and 25% were female. Major comorbidities present in these children included seizures, impaired speech, low IQ and involuntary movements. Topographically, 46.4% were diplegic, 28.6% were hemiplegic and 25% were quadriplegic type of CP. Majority of CP children belonged to GMFCS levels and MACS level II. The verbal functioning, performance ability and total IQ of CP children with GMFCS III, IV and V were low. The verbal functioning of GMFCS II was higher than that of GMFCS I, while the performance ability and total IQ of GMFCS I were greater than GMFCS II. The differences were statistically significant (p value < 0.05). Conclusion: In children with limited motor activity, the performance IQ was more affected than verbal IQ. Caregivers of CP children with low IQ experienced higher burden.

Award ID: 802

Quantification of Carotid Atheromatous Plaque

Kiren Koshy, Veerendra Kumar, Subasree Ramakrishnan, Raghavendra Kenchaiah, Arvinda H R

Neurology, NIMHANS Bengaluru, Karnataka, India

E-mail: kirenkoshy@gmail.com

Background and Aim: Carotid atheromatous disease is a major etiology of ischemic stroke. Aim: To study carotid atherosclerosis in patients with stroke and in healthy subjects in terms of carotid intima media thickness (CIMT) and quantitative and qualitative plaque analysis Methodology: Type of study: observational descriptive Sample size: 12 subjects with stroke or TIA, 10 healthy controls Inclusion criteria: patients with ischemic stroke in the age group of 20 to 70 years based on clinical profile and imaging, age and sex matched healthy controls Experimental procedure: Carotid ultrasonography (2D and 3D) along with doppler assessment. Vascular Plaque Quantification (VPQ) software was used to find plaque volume. Result: Twelve Patients with stroke/TIA and 10 controls were studied. CIMT was similar in both cases and controls. Systolic/diastolic ratio was lower in ICA (2-93 ± 0.6) compared to ECA (5.92 ± 1.55) p < 0.001. Atheromatous plaques were present in 66.7% of patients with stroke and 30% of controls. A total of 18 plaques were studied. Based on plaque characteristics (presence of echolucency, homogeneity, presence of surface irregularity or ulceration), plaques were categorized into stable and unstable plaques. Seventy percent of unstable plaques were symptomatic while only 16.7% of stable plaques were symptomatic. Mean plaque volume in patients was 179.82 ± 310.3 mm3 while that in controls was 56.75 ± 69.6 mm3. Maximum cross-sectional area reduction fell within the limits of estimated stenosis by Doppler criteria for all 18 plaques. Conclusion: Qualitative characterisation of carotid plaques is helpful in predicting their stability. 3D plaque quantification in terms of plaque volume can be done with specialised software and is helpful in quantiying the plaque with high degree of precision.

Award ID: 803

Clinical Profile, Outcome and Electrophysiological Correlates of Disorders of Consciousness

Farsana MK, Subasree Ramakrishnan, Ravindranath Chowdary, Raghavendra K, Dhaval P. Shukla, Sonia Bansal

Neurology, NIMHANS, Bengalure, Karnataka, India

E-mail: ayshafmk@gmail.com

Background and Aim: There is an increase in number of patients surviving severe brain injury and entering into coma, unresponsive wakefulness syndrome (UWS) or minimally conscious state (MCS) across the world. Electrophysiological assessment could identify patients with higher chances of clinical improvement which will help with various aspects of decision- making like prognostication, rehabilitation, resource management and therapeutic trials. This study examined clinical profile, qualitative EEG, median nerve somatosensory evoked potentials, recovery pattern and correlation with outcome in patients with disorders of consciousness (DoC). Methodology: This prospective study was conducted in 45 patients above 18 years with DoC due to traumatic and non-traumatic ethology who got admitted to NIMHANS neurology and neurosurgery departments from May 2021 to April 2022, after obtaining ethical committee approval and written informed consent. Diagnosis of DoC was made based on standard clinical diagnostic criteria. Acute reversible causes of encephalopathy and coma mimics were excluded. EEG amplitude, dominant frequency, reactivity to stimuli, amplitude of median nerve SSEP were analysed and integrated to various rating scales. Coma Recovery Scale Revised (CRS-R) and Glasgow outcome scale were used to assess outcome. Data was analyzed using IBM SPSS software version 22. Result: Patients with delta frequency and reduced amplitudes showed less improvement in CRS-R scores and poor outcome at 3 and 6 months compared to patients with normal amplitudes and alpha frequency. Patients with EEG reactivity showed greater improvements in CRS-R scores than patients without reactivity. Median nerve SSEP did not show any significant predictive value. Conclusion: Delta frequency and reduced EEG amplitudes correlated with poor recovery and worse clinical outcome, while alpha frequencies and reactivity to stimuli correlated with better outcome in disorders of consciousness.

Award ID: 804

Myeloblastic Meningeal Neoplasm – An Extraordinarily Long Natural History

Saranya Gomathy, Elavarasi A, Aanchal Kakkar, Ritu Gupta, Devasis Panda, Ajay Garg

Neurology, AIIMS Delhi, India

E-mail: gomathysubramoniam@gmail.com

Background and Aim: Myeloid sarcoma is a rare extramedullary malignant tumor, associated with AML. It is usually associated with skin and soft tissue deposition of myeloblastic cells in the setting of bone marrow and blood involvement. There have been only a very few case reports of isolated CNS involvement by myeloid sarcoma. Even rarer is a smoldering presentation restricted to the CNS. We report this patient who presented with headache, progressive vision loss and raised ICP, which was going on for more than two years. Methodology: 24-year-old man presented with episodic headaches and vomiting for two years, progressive diminution of vision in both eyes for one year with complete loss since three months. He had a painful swelling in the left thigh 15 years back, diagnosed with myeloid sarcoma and treated with Radiotherapy. He remained asymptomatic for next 13 years after which he developed intermittent headache and vomiting-evaluated to have intracranial hypertension and meningeal lesions on MRI. When he presented to our institute, he could not perceive light in either eye, with grade IV papilledema and neck stiffness. We considered CVT, meningeal leukemia and meningeal melanomatosis in our differentials. Result: Hemogram, RFT and LFT were normal. Peripheral smear didn’t reveal blasts. CSF opening pressure was high with lymphocytic pleocytosis and elevated protein. Flow cytometry revealed myeloid blasts. MRI brain revealed T1 hyperintensities, SAH and leptomeningeal enhancement. Bone marrow examination didn’t show involvement by blasts. Conclusion: He was diagnosed with myeloid sarcoma of CNS, treated with multiple therapeutic CSF taps and intrathecal cytarabine and craniospinal irradiation. Her reported very minimal improvement in vision.

Award ID: 805

Cerebral Hemodynamic Evaluation in Children with Sickle Cell Disease in India

Erum Khan, Bhakti Gajjar, Pranita Sharma, Pawan Jain, Vikas Goel, Arvind Neral, Arvind Sharma, Sanjay Sharma, Vijay Sharma

Department of Medicine, B.J.Medical College, Civil Hospital, Ahmedabad, Gujarat, India

E-mail: erum98khan@gmail.com

Background and Aim: India has the second highest number of cases of sickle cell disease (SCD), many belonging to low socioeconomic status, spread across a horizontal belt from Gujarat to Odisha. Despite high prevalence of SCD in India, information about cerebral hemodynamics among children with SCD remains scarce. We performed transcranial Doppler (TCD) to assess cerebral hemodynamics among Indian SCD children and examined their association with clinical and hematological parameters. Methodology: Children aged 3-18 with SCD living in Raipur and Ahmedabad were recruited. TCD was performed to obtain flow velocities from middle cerebral, intracranial internal carotid and basilar artery. Associations were evaluated between timed-average-mean-maximum velocities (TAMMV), end diastolic velocity (EDV), and peak systolic velocity (PSV) with clinical and hematological parameters. Result: In this prospective study, a total of 62 consecutive children, previously diagnosed with SCD were recruited. Study population was 9.8 ± 3.9 years old and 31 (50%) were male. Hb was 8.64 ± 1.34 Gm/dL while the mean HbS was 6.28 ± 1.06%. 11% of participants showed TAMMV of > 200 cm/s, higher than previous studies across the world. Higher HbS level, history of iron chelation therapy, history of blood transfusion and stroke showed a trend towards having higher TAMMV. A positive spearman correlation was observed among TAMMV, EDV and PSV (p < 0.001 for all vessels). All correlations were well above 0.7, with PSV having the strongest correlation with TAMMV (more than 0.9 in all vessels). Conclusion: Stroke and cerebral hemodynamic alterations are common among Indian children with SCD. A High TAMMV in Indian SCD patients could be due to coexisting variants of other hemoglobinopathies. Our findings, consistent with previous studies, support recommendation of performing TCD according to the STOP trial. This will help in risk stratification and optimization of blood transfusion to reduce stroke and physical disabilities among Indian SCD patients, especially among socioeconomically disadvantaged communities.

Award ID: 806

Cerebral Venous Thrombosis (CVT) Etiology, Radiological Features and Outcomes – A Retrospective Multicenter Study

Dulari Gupta, Rahul Kulkarni, Anup Bende, Sripad Pujari, Shankar Prasad Gorthi

Department of Neurology, Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

E-mail: dularigupta@gmail.com

Background and Aim: CVT is an uncommon cause of stroke. In recent times, better awareness and easy access to imaging has increased the diagnosis of CVT. Methodology: This study is a retrospective observational multi-center study in two tertiary care hospitals in Pune, Western India. All consecutive adult patients admitted with CVT and CVST (Cerebral Venous Sinus Thrombosis) were included from January 2014 to June 2022. Their demographic details, clinical features, laboratory findings, etiologies, imaging findings and outcomes were recorded. All patients were contacted telephonically at the end of the study to assess outcomes via telephonic modified Rankin Scale (mRS). Result: A total of 400 patients with CVT were studied. Males 73%, mean age 35 years +/-5.75. Headache and seizures were the commonest clinical presentations. Alcohol consumption, and hyperhomocystianemia were the commonest risk factors in males, whereas anemia, OCP usage and post-partum were the commonest risk factors in females. Thirty percent had intercranial hemorrhage; superior sagittal sinus followed by transverse sinus were the commonest sinuses to be involved. In-hospital mortality was 8%. Half the patients could be contacted by telephonically at the end of the study and their mRS was recorded. Eighty percent had mRS < 2, suggestive of good outcomes. Conclusion: CVT is an important cause of strokes in the young. Males are more affected than females. In hospital mortality is 8% whereas long-term follow up showed good prognosis (mRS < 2) in 80%.

Award ID: 807

Endovascular Treatment of Cerebral Venous Thrombosis – A Case Series

Sucharitha M V, Thomas Mathew, Gosala R K Sarma, Raghunandan Nadig, Saikanth Reddy, Sharath Kumar G G, Sagar Badachi, Delon D. Souza, Sonia Shivde, Poonam Awatare, Sunitha Palasamudram

Neurology, St Johns Hospital

E-mail: sachu.bunty@gmail.com

Background and Aim: Cerebral venous sinus thrombosis (CVT) accounts for 0.5%–1% of all stroke cases. Anticoagulation, anti-oedema, anti-epileptics are the main stay of treatment. Endovascular procedure can be considered for those who worsen on anticoagulation or have high risk of poor outcome. It aims to reduce thrombus burden rapidly either by locally administrating fibrinolytic agents or mechanically removing it. Based on the prognostic variables, they are classified into low risk and high risk of poor risk of outcome and the decision for endovascular intervention is taken. To enumerate the indications for endovascular treatment and the outcome of procedure. Methodology: Case records of pateints who underwent endovascular procedure for CVT between June 2019-May 2022 was studied, details regarding clinical presentation, treatment, radiological investigations were studied and descriptive statistics applied. Result: In our study 8 patients with CVT had undergone interventional procedure for CVT. Most common sinus involved was superior sagittal sinus. Thromboaspiration was done in all patients and one underwent thromboaspiration with venoplasty. Out of eight patients 2 had risk score of 5, 4 had risk score of 3, the 2 had risk score of 1. Two out of eight had complications in the form of cortical vein rupture that was managed conservatively. Worsening of hemorrhage was noted in one patinet that was managed conservatively. Seven out of eight patients improved and currently their Modified Rankin scale is 0. 1 patient succumbed to illness. Conclusion: Systemic anticoagulation is the mainstay of treatment but endovascular treatment is a useful alternative for patients with risk score more than 3 or who worsen on anticoagulation. Close monitoring of GCS, development of new symptoms or worsening of existing symptoms is important for timely intervention.

Award ID: 808

A Study of Clinical and Imaging Features of Refractory Epilepsy in Patients Attending Tertiary Care Centre, South India

Sudarshan Reddy

Neurology, Osmania Medical College, Hyderabad, Telangana, India

E-mail: meetdrsudha@gmail.com

Background and Aim: Epilepsy is a chronic noncommunicable disease. More than 50 million people have epilepsy. Epilepsy is characterized by an enduring predisposition to generate epileptic seizures and by the neurobiological, cognitive, psychological, and social consequences of this condition. Drug resistant epilepsy defined as failure of adequate trials of two tolerated and appropriately chosen and used AEDs to achieve sustained seizure freedom for a sufficiently long period of time (three times the longest inter-seizure interval for that patient, or 1 year, whichever is longer). Early identification of patients is essential in selecting patients for investigations and treatment and to prevent toxicity from overdose and costs of AED. Aim of the study is to identify the risk factors, clinical and imaging features in Refractory Epilepsy. Methodology: A series of 105 patients with symptomatic epilepsy who are on two or more AEDs and who attended Neurology OPD and inpatients in neurology ward were evaluated by history, examination. Investigated using Routine blood work, EEG, CT Brain or MRI Brain. Result: Of these 105 patients, males were 70, females were 35. Most Common age group is 21- 30 years. Majority (44%) had onset below 10 years. Focal seizure type (68) was the most frequent type. 58 (55.5%) patients had developmental delay. 44 (42.4%) had Status epilepticus. 70% had abnormal EEG. Most common abnormality on imaging in children is congenital anomalies, in adults it is calcifications. Conclusion: Many risk factors identified for refractoriness. The seizure episode should be evaluated and treated with appropriate AEDs. Complete evaluation to identify the cause helps in early treatment intervention and better quality of life.

Award ID: 809

The Time Perception and Time Production Abilities in Parkinson’s Disease

Avanteeka Ganguly, Supriyo Choudhury, Tanisha Majumdar, Hrishikesh Kumar, Supriyo Choudhury, Hrishikesh Kumar

Department of Neurology, Institute of Neurosciences Kolkata, West Bengal, India

E-mail: avanteekaganguly@hotmail.com

Background and Aim: Cholinergic and dopaminergic neurotransmissions are associated with time estimation in healthy. Therefore, their depletion in Parkinson’s Disease (PD) might lead to a distortion in time perception. Learning and memory could be closely related to time perception due to supposedly common neurochemical substrate. In this study, we compared the subjective perception of time and time-production between PD and healthy. We also explored if learning ability has impact on the time perception in PD patients. Methodology: A “Time Perception Questionnaire” was developed and administered to 42 PD patients and 27 age-matched healthy participants. Participants went through screening using MoCA, and PD group was scaled on MDS-UPDRS III. 20 patients from PD group, and the healthy group were also administered a simple “Non-sense Syllable (NS) Test” to assess their learning and retention abilities. Result: 15% and 20% of the PD patients and healthy respectively reported a subjective slowness of passage of time in their daily lives (p = 0.9). Time production tasks showed a significant underproduction of time for both the 5s and 30s intervals in PD group (p < 0.05). Healthy control performed significantly better compared to PD patients in nonsense syllable test (p < 0.05). However, no correlation could be obtained between the NS test scores and time production task performance. Conclusion: A significant underproduction of the instructed time interval in PD patients was noted. The distortion in objective time-keeping in PD is not reflected in their subjective experience of time interval. PD patients displayed a diminished learning and retention ability, even after being screened for cognitive impairment.

Award ID: 810

Experience of Botulinum Toxin Clinic During the Covid-19 Epidemic: A Retrospective Chart Review

Saurabh Nandwani, Anumeha Mishra, Ganeshgouda Majigoudra, Sanjay Pandey

Neurology, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research

E-mail: nandwanisaurabh@gmail.com

Background and Aim: Clinical services were severely affected during the COVID 19 epidemic, globally. This study was conducted to characterise the experience of botulinum toxin (BTX) clinic during the Covid period Methodology: This is a retrospective chart review of patients who received injections of BTX during the Covid period. Result: A total of 105 patients received an injection of BTX, out of which 76 (72.4%) were male. The mean age of patients was 47.9 (SD: 15.1) years. The most common indication for BTX injection was dystonia [ 79 patients (75.2%)] followed by hemifacial spasm [ 22 patients (21%)] and miscellaneous [ 4 patients (3.8%)]. Amongst dystonia most frequent was focal [ 45 patients (42.9%) followed by segmental [ 24 patients (22.9%)]. Generalised dystonia and hemi-dystonia were 8.6% and 1% respectively. Amongst focal dystonia most frequent were cervical dystonia (44.4%), blepharospasm (17.8%), and writer’s cramp (15.6%). The miscellaneous group included 4 patients (3.8%) with trigeminal neuralgia, Holme’s tremor, dystonic tics, and hemi-masticatory spasm. The mean age of patients in dystonia, hemifacial spasm, and the miscellaneous group were 47.7 (SD-14.9), 49.2 (SD:14.0), and 44.2 (SD: 26.0) years. The mean BTX dose used was 131.6 U (SD:104.1). The mean BTX dose used for the dystonia group was 158.7 U (SD: 105.3), for hemifacial spasm was 40.1 U (SD:11.3), and for the miscellaneous group was 100.0 U (SD: 70.7) Conclusion: The majority of patients were males. The most frequent patients were of dystonia (75%), with the most being focal dystonia (43%).

Award ID: 811

Profile of Young Patients Presenting with Stroke to Tertiary Care Hospital in Kashmir

Irfan Yousuf Wani, Shakeel Ahmad Bhat, Parvaiz Ahmad Shah

Medicine, Government Medical College, Srinagar, Jammu and Kashmir, India

E-mail: drirfanyousuf@gmail.com

Background and Aim: Indian studies have shown that about 10% to 15% of strokes occur in people below the age of 40 years1,2. No data is available from our region. so we did this study. Methodology: This study was done over a period of one and a half years. All stroke patients in the age group 18-45 years who were admitted in SMHS Hospital during the study period were included in this study. Result: Our study comprised of sixty patients,35 males and 25 females. The mean age in our study was 32.7 ± 7.69 years. Dyslipidemia was the most common risk factor (28 patients). 25 patients were smokers. 43 had ischemic stroke while as 17 had hemorrhagic stroke. Etiology of stroke was unexplained in fourteen patients. In ischemic strokes, cardioembolism was most common (11 patients). Arterial dissection was present in five patients. Three patients were found to have thrombophilic disorders. Out of seventeen hemorrhagic stroke patients, hypertensive bleed was most common. In our study mRS at three months was favorable in forty three patients mRS (0-1). Conclusion: Dyslipidemia, Smoking and hypertension are the three leading modifiable risk factors in our study suggesting that increased public awareness and public health system interventions could reduce the overall burden due to young stroke. There is a need to highlight the awareness of some specific biochemical and imaging investigations to recognize etiologies like arterial dissections and thrombophilic disorders early in the evaluation of young strokes.

Award ID: 812

A Randomized Controlled Study to Compare Antidepressants in Mild to Moderate Depression in Person with Epilepsy

prashant Bhatele, Manjari Tripathi

Neurology, All India Institute of Medical Sciences, New Delhi, India

E-mail: prshntbhatele@gmail.com

Background and Aim: Depression is the most common psychiatric disorder in PWE and often goes unrecognized and untreated (1). However, RCT for direct comparisons of newer antidepressants (Escitalopram and Venlafaxine) in PWE with depression are lacking. Thus, Randomized Controlled Clinical Trials are necessary to assess the efficacy and safety of newer antidepressants in PWE. The primary outcome was to study difference in efficacy based on the change in the scores of Hamilton depression rating scale (HAM-D) and adverse event profile at week 8. Secondary outcomes included QOLIE-31, seizure frequency and adherence to treatment using Medication Adherence Rating Scale. Methodology: In a prospective, double-blind RCT involving 90 PWE with mild to moderate depression, were randomly assigned in a 1:1 ratio to receive escitalopram and venlafaxine. Allocation was done by using pharmacy controlled randomisation method. Result: Out of 350 PWE screened by NDDI-E scale, 90 eligible PWE were enrolled. The mean (+SD) scores on the HAM-D at baseline were 13.53 + 3.27 in escitalopram group and 13.02 + 3.57 in venlafaxine group. The mean difference (95%CI) in scores of HAM-D at week 8 were significant in both groups (p < 0.001)(ITT: Escitalopram 8.21 (7.39,9.03), Venlafaxine 7.75 (6.75,8.79). 92.9% in escitalopram group and 90% in venlafaxine group adhered to the treatment at week 8. The change in depression scores on HAM-D at week 8 did not differ significantly between trial groups. Other secondary outcomes favored both escitalopram and venlafaxine. The incidence of adverse events was more in venlafaxine group. Fatigue, decreased appetite and dry mouth were significantly associated with venlafaxine. An increase in seizure frequency was observed in 2.2% in both groups. Conclusion: Among PWE with mild to moderate depression, both escitalopram and venlafaxine are similar in efficacy, considering high burden of adverse events and cost of venlafaxine, escitalopram represents a better choice in treating depression in PWE.

Award ID: 813

The Study of Somatosensory Temporal Discrimination Threshold (STDT) in Writer’s Cramp - Inter Hand Difference And Correlation with Severity of Dystonia

Merugu M, Venkata Navya Gopal, Rukmini Mridula Kandadai, Rupam Borgohain

Neurology, Nizams Institute of Medial Sciences, Hyderabad, Telangana, India

E-mail: akhil.m673@gmail.com

Background and Aim: Somatosensory temporal discrimination threshold (STDT) measures a subject’s ability to discriminate paired sensory stimuli temporally in time. in this study we evaluated STDT in affected and unaffected hand of patients and correlated it with severity of disease. Aims To investigate the STDT abnormalities and inter-side difference in patients with writer’s cramp and to correlate STDT with severity of dystonia. Methodology: This is a single center prospective case control study conducted from January 2021 to December 2021, in Department of Neurology, NIMS. We included 69 WC patients and 29 controls. STDT was recorded in both hands independently in graded increasing intervals and mean STDT values were recorded. Severity of WC was assessed using ADDS score. STDT values were compared between patients and controls and were correlated with severity and duration of disease using appropriate statistical tests. Result: Out of 69 patients, there were 37 dystonic and 32 simple writer’s cramp patients. Mean STDT values of affected hand in dystonic WC and simple WC patients were significantly higher than that of controls. Mean STDT values of unaffected hand in dystonic WC and simple WC were significantly higher than that of controls. Mean STDT values of affected limb in both dystonic and simple WC patients were significantly higher than that of unaffected limb. Mean ADDS score of dystonic WC was lower than that of simple WC. Conclusion: We found that STDT was significantly prolonged in both affected and unaffected hands of dystonic and simple WC patients compared to healthy controls. STDT values in affected hand were significantly higher compared to unaffected hand. STDT values showed significant correlation with severity of disease and duration of disease.

Award ID: 814

Clinico-Radiological Profile of Patients with Pyruvate Dehydrogenase Deficiency – PDHA1 Mutations

Karthika Valaparambil, Soumya Sundaram, Ramsekar Menon

Neurology, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Trivandrum, Kerala, India

E-mail: karthikaajitv@gmail.com

Background and Aim: Pyruvate dehydrogenase (PDH) deficiency is a disorder of energy metabolism with variable clinical presentations, ranging from severe infantile lactic acidosis to milder chronic neurological disorders. The pyruvate dehydrogenase (PDH) complex is a mitochondrial multienzyme that catalyses the irreversible oxidative decarboxylation of pyruvate to acetyl-CoA. Mutations that lead to decreased PDH activity have been reported in PDHA1, PDHB, and in the genes encoding E2, E3-binding protein with the most frequent cause of PDH deficiency being mutations in PDHA1. AIMS To review the clinical, biochemical, radiologic and genetic profile of patients with PDH deficiency harbouring PDHA1 mutations Methodology: Retrospective analysis of patients with Pyruvate Dehydrogenase (PDH) deficiency harbouring PDHA1 mutations attending PediatricNeurology Division in our Institute. Result: We report 4 patients with Pyruvate Dehydrogenase (PDH) enzyme deficiency harbouring PDHA1 mutations. Among the 4 patients, 2 were males. Age of onset of illness was observed during the infantile period. Varying clinical phenotypes observed were Epileptic encephalopathy, recurrent ataxia, generalised chorea with dystonia. Lactate levels were uniformly elevated with normal lactate pyruvate ratio. Varying radiological presentation was noted which included ventriculomegaly, thinned out corpus callosum and hyperintensities in b/l dentate nucleus of cerebellum and basal ganglia. As per ACMGE classification, pathogenic variants, likely pathogenic and variants of unknown significance were observed in 1, 1 and 2 patients respectively. Target gene identified was PDHA1. The 2 mutations identified as Variants of unknown significance (VOUS) were novel mutations as they had phenotype genotype match. Among the mutations 2 deletions, 1 insertion and 1 misense variants were detected. Patients were initiated on thiamine and ketogenic diet and showed response on follow up. Conclusion: The genotype-phenotypic spectrum of PDH deficiency is expanding. High index of clinical suspicion will help in arriving at this potentially treatable entity.

Award ID: 815

Assessment of Autonomic Function During Ictal and Interictal Period of Migraine

Pooja Anand, Achal Kumar Srivastava

Neurology, All India Institute of Medical Sciences

E-mail: pooja.anand264@gmail.com

Background and Aim: There are studies which suggest that a degree of autonomic dysfunction is implicated in pathophysiology of migraine but none of them have commented in detail. This is a pilot study for assessment of autonomic status during ictal and interictal phases of migraine. Methodology: Tests of sympathetic function (pressure changes in Head-Up-Tilt test) and parasympathetic function (heart rate response to Deep Breathing and Valsalva Maneuver) were performed, each during ictal and interictal period and statistical analysis done. Result: A total of 10 patients were studied. In Deep breathing test, the Expiration:Inspiration [E:I] ratio and change in heart rate (∆HR) was significantly lower in the ictal period than interictal state (1.19 ± 0.07 vs 1.35 ± 0.11, p = 0.006; 16.24 ± 4.44 vs 25.19 ± 6.81, p = 0.006), suggesting significantly impaired vagal response. The Valsalva ratio and 30:15 ratio in Head-Up-Tilt test were not statistically significantly different between the ictal and interictal state (1.57 ± 0.2 vs 1.68 ± 0.29, p = 0.21; 1.09 ± 0.08 vs 1.26 ± 0.4, p = 0.25), indicating intact sympathetic function. Conclusion: There is significant parasympathetic dysfunction in the ictal state as compared to headache-free periods. There is no objective evidence of sympathetic dysfunction in the ictal period as compared to headache-free periods. Therefore, this study provides new insight into the genesis of the ictal state of migraine.

Award ID: 816

Assessment of Autonomic Function during Ictal and Interictal Period of Migraine

Pooja Anand, Achal Kumar Srivastava

Neurology, All India Institute of Medical Sciences

E-mail: pooja.anand264@gmail.com

Background and Aim: There are studies which suggest that a degree of autonomic dysfunction is implicated in pathophysiology of migraine but none of them have commented in detail. This is a pilot study for assessment of autonomic status during ictal and interictal phases of migraine. Methodology: Tests of sympathetic function (pressure changes in Head-Up-Tilt test) and parasympathetic function (heart rate response to Deep Breathing and Valsalva Maneuver) were performed, each during ictal and interictal period and statistical analysis done. Result: A total of 10 patients were studied. In Deep breathing test, the Expiration:Inspiration [E:I] ratio and change in heart rate (∆HR) was significantly lower in the ictal period than interictal state (1.19 ± 0.07 vs 1.35 ± 0.11, p = 0.006; 16.24 ± 4.44 vs 25.19 ± 6.81, p = 0.006), suggesting significantly impaired vagal response. The Valsalva ratio and 30:15 ratio in Head-Up-Tilt test were not statistically significantly different between the ictal and interictal state (1.57 ± 0.2 vs 1.68 ± 0.29, p = 0.21; 1.09 ± 0.08 vs 1.26 ± 0.4, p = 0.25), indicating intact sympathetic function. Conclusion: There is significant parasympathetic dysfunction in the ictal state as compared to headache-free periods. There is no objective evidence of sympathetic dysfunction in the ictal period as compared to headache-free periods. Therefore, this study provides new insight into the genesis of the ictal state of migraine.

Award ID: 817

Covid-19 Related Central Pontine and Extra Pontine Myelinolysis

Peerzada Shafi, Rashmi Devaraj

Neurology, Apollo Hospital Bangalore, Karnataka, India

E-mail: peerzadashafi@gmail.com

Background and Aim: The novel severe acute respiratory syndrome associated coronavirus (SARS-nCoV-2), the causative organism for the coronavirus disease (COVID-19) pandemic, has caused a major healthcare crisis of an unprecedented scale worldwide. As the number of cases grow at an exponential rate, the medical fraternity has witnessed a diverse range of new neurological manifestations in these patients. While some of these neurological issues manifest during the acute phase of the COVID infection, a few occur during the convalescent phase as well. Methodology: We report three patients who had an acute COVID infection of variable severity from which they recovered completely. But 2 to 4 weeks later these patients presented with altered sensorium and were found to have hyponatremia. Despite slow correction, they developed features of locked-in-syndrome with similar neurological findings of dysarthria, quadriparesis, rigidity, dysphagia, pyramidal signs and tremors. MRI showed varying combinations of central pontine and extrapontine myelinolysis. Patients were managed with steroids and/or intravenous immunoglobulin resulting in significant recovery with mild residual extra-pyramidal symptoms. A better prognosis may be expected in COVID related myelinolysis. The possibility of immune-mediated etio-pathogenesis of myelinolysis should be considered, as they show a good response to steroids and/or IVIG. Result: Conclusion: A high index of suspicion for Central Pontine Myelinolysis and Extra Pontine Myelinolysis has to be kept for patients who present with altered sensorium and hyponatremia in the post-COVID phase. The plausible mechanisms for hyponatremia and Pontine and extra-pontine myelinolysis in the convalescent period of COVID infection needs to be explored.

Award ID: 818

Quantitative Analysis of Disorder of Consciousness Patients based on Scalp EEG using Machine Learning Methods

sreelakshmi Raveendran, Subasree R, Bini A A, Raghavendra K, Ravindranath Chowdary, Sonia Bansal, Farsana M K

Electronics and Communication Dept., IIIT Kottayam, Kerala, India

E-mail: slr.sreelu.slr@gmail.com

Background and Aim: Disorder of consciousness (DOC) being described by impaired wakefulness and awareness can be categorized into Coma, Unresponsive Wakefulness Syndrome (UWS), and Minimally Conscious State (MCS). Resting-state EEG could significantly differentiate between the DOC class further aiding in the diagnosis and prognosis of DOC patients. EEG-based study amounts to reducing the error rate of around 40% in DOC classification using behavioral assessment. We aim to study the classification of DOC patients based on resting-state EEG using machine learning classifiers. Methodology: Twenty controls and 60 DOC patients were enrolled in this study from NIMHANS. The DOC was categorized further by a consultant neurologist based on the CRS-R (Coma recovery Scale-Revised) and GCS (Glasgow Coma Scale) scores. The control and the DOC underwent resting-state EEG followed by extraction of band power, permutation entropy, and sample entropy for each channel to be input for classifiers. Data augmentation was done to increase the yield. SVM, LDA, QDA, LR, Naive Bayes, KNN, Random Forest, and Decision Tree were used as binary and multiclass classification. One way ANOVA method determined the discriminative ability of the features with the post hoc Least Significant Difference (LSD) test. Two-group comparisons were performed using the student’s t-test (p < 0.05). Result: Relative band power and sample entropy across occipital, parietal, frontal, and central regions showed more significant differences (p < 0.05) in delta, alpha, and beta bands between groups. The decision tree classifier gave the highest classification accuracy of 87.5% for both binary and multiclass classification. Conclusion: Relative power and sample entropy serve as important features that distinguish the DOC category.

Award ID: 819

Electrical Source Localisation (ESL) of Epileptiform Activity in Children with Neurodevelopmental Disorders (NDD)

murugan Nair, Vishnu Prasad, Amal Ahammed, Sanjeev Thomas, Mary Iype, Amal Ahammed, Varna V. Nair, Mary Iype, Sanjeev Thomas

Department of Neurology, Institute of Communicative and Cognitive Neuroscienses (ICCONS)

E-mail: murunair@gmail.com

Background and Aim: The relationship between NDD, epileptiform activity in EEG, and clinical seizures remain uncertain, although several hypothesis have been developed. To characterise the ES of epileptiform activity on an MRI template for children with NDD. Methodology: Children with NDD and epileptiform discharges in the EEG were further evaluated with BESA software to localise the ES on an MRI template. The ES were correlated with their clinical characteristics obtained from the medical records. Result: There were 12 Children (Male 9). Age range - 2 to 12, Mean 7 +/- 2.69. Autism spectrum disorder (ASD) 4, Developmental learning disorder (DLD) 4, Attention deficit Hyperactive disorder (ADHD) 3. The ES was localised to the Frontal lobe (2), Temporal lobe (2), Fronto-Temporal (4), Multifocal (4) ESL correlated with MRI lesion in 3 of the 4 children with MRI abnormality while 7 children with no MRI abnormality demonstrated clear ESL. The ES was localised to Temporal+ (2 Temporal, 1 Fronto-temporal) location in 75% of ASD cases and 33% of ADHD. It was localisable to Extra temporal location for four children (1 ASD, 2 ADHD, and 1 DLD). Conclusion: The ES can be successfully localised from EEG in children with NDD with epileptiform activity. The source was localised to the temporal lobe and associated areas for most children with ASD and DLD and was more widespread with ADHD and GDD.

Award ID: 820

Distortion in Temporal Processing in Parkinson’s Disease: A Neurophysiological Study

Tanisha Majumdar, Avanteeka Ganguly, Supriyo Choudhury, Stuart N. Baker, Mark R. Baker, Suchishmita Majumdar, Hrishikesh Kumar

Neurology, Institute of Neurosciences Kolkata, West Bengal, India

E-mail: tanishamajumdar11@gmail.com

Background and Aim: Central dopaminergic activity plays a crucial role in processing of temporal information. The aim of the present study was to investigate the distortion in temporal processing in Parkinson’s Disease assessed by motor timing tasks -Time Production and Reproduction. The study also aims to calculate potential correlations among task performance in PD patients and measures of disease severity and cognitive abilities. Methodology: Patients diagnosed with PD and healthy individuals (n = 24) matched by age and sex were tested for time production (n = 25) of short (3s), medium (5s) and long (10s) duration and time reproduction (n = 45) of standard stimuli 500 ms, 1.5s, 3s. Motor symptoms were assessed using UPDRS (part III) and MoCA was employed for cognitive screening. Result: In the ‘Time Reproduction Task’, we observed that PD patients demonstrated a significant over-production of time interval for 500 ms standard stimuli and a significant underproduction of time interval for 03s standard stimuli than healthy individuals (p < 0.05). No significant difference was observed in the ‘Time Production task’ performance. A significant negative correlation was observed in Time reproduction task performance at 03s (r = -0.490, p 0.007) and UPDRS score. Conclusion: We observe an abnormality in temporal processing in PD patients and a dissociation in temporal reproduction abilities in sub-second and supra-second ranges which may be thought to arise from altered basal ganglia function. An increase in disease severity may contribute to an increased distortion in temporal reproduction abilities. Further studies will help us assess whether deficits in temporal processing correlate with the clinical progression of the disease.

Award ID: 821

A Comparative Analysis of Coverage of Epilepsy under Rashtriya Bal Swasthya Karyakram (RBSK) with Community and School based Surveys in Faridkot District in North India

Sulena, Gagandeep Singh, Divesh Tyagi, Ravinder Garg, Preeti Padda, Rajinder Kumar, Kiranjit Kaur, Harmandeep Kaur

Neurology, Guru Gobind Singh Medical College, Faridkot, Punjab, India

E-mail: sulenasingh@yahoo.co.in

Background and Aim: Treatment gap for Epilepsy varies between 22% (1) and 90% (2) in India. The Rashtriya Bal SwasthyaKarayakaram (RBSK) is a program to bridge this immense treatment gap (3). The study aims to estimate the coverage of epilepsy in children under RBSK as compared to prevalence in community and school survey in Faridkot district Methodology: The study was undertaken in a rural district in North India from 1st December 2019 to 31st March 2022. Screening of 45,075 children from Health screening register by Mobile Health Team (MHT) under RBSKwas done. Door to door community based survey of 10,279 children and school based survey of 7,627 childrenafter randomization using probability proportional to size method of sampling. Subjects were screened using a validated screening questionnaire for epilepsy. All screen positive children were invited to the hospital for further neurological evaluation included magnetic resonance imaging, electroencephalographic study. Diagnosis of epilepsy and its classification was done using guidelines by International League Against Epilepsy (2017). Result: Out of 42 children screened positive in Health visit register of MHT, 34 were confirmed cases of epilepsy with prevalence of epilepsy 0.75 per 1000 children. Community and school survey showed 77 and 13 confirmed cases of epilepsy with prevalence of 7.4 and 1.7 per 1000 children respectively. Generalized epilepsy was most common type in all the surveys. Conclusion: Coverage of epilepsy as per RBSK was lower as compared to community and school based survey. Stigma associated with epilepsy prevents parents from disclosing the condition to school authorities and RBSK personnel.

Award ID: 822

Retrospective Study of Clinical and Neuroradiological Features of Myelin Oligodendrocyte Glycoprotein Antibody Disease from a Tertiary Care Centre

Swathi Sanjee, Thomas Mathew, Raghunandan Nadig, GRK Sarma, Sharath Kumar GG, Saikanth Deepalam, Suneetha Palasamudram, Delon Dsouza, Sagar Badachi, Sonia Shivde, Poonam Awatare

Neurology, St Johns Medical College Hospital, Bengaluru, Karnataka, india

E-mail: swathi.s.sanjee@gmail.com

Background and Aim: Introduction: Myelin oligodendrocyte glycoprotein-immunoglobulin G associated disease (MOGAD) was initially described as a subtype of neuromyelitis optica spectrum disorder (NMOSD) with antibodies against MOG. However, it has recently been described as a separate disease entity with clinical and radiological features that overlap those of multiple sclerosis (MS) and NMOSD. The clinical features of this disease phenotype remain undetermined as compared to NMOSD and MS where disease specific manifestations are described. Aim : To describe clinical and neuro-radiological features of MOGAD Methodology: Retrospective study was conducted at a tertiary care Hospital. All patients evaluated by Neurology department between January 2018 and March 2022 with MOG antibody being positive were included in the study. Detailed clinical history, examination and neuroimaging findings were noted and descriptive statistics were applied. Result: 35 patients were MOG-positive. 45% were females. Most common presentation was Optic neuritis (60%). One case of ON presented with chiasmatic lesion mimicking NMOSD. One case of bilateral ON showed assymetric T2 hyperintensities in bilateral optic tracts, right optic radiation, splenium and midbrain which was atypical. Second most common presentation was transverse myelitis (20%). Focal meningoencephalitis was noted in 14% of cases. One case had extensive white matter changes on MRI suggestive of Leucoencephalopathy. One child presented with pyrexia of unknown origin showed hypothalamic demyelinating lesion. Acute demyelinating encephalomyelitis (ADEM) was observed in 14% of cases. One case of MOGAD on immunosuppression presented as isolated Limbic encephalitis. Conclusion: The phenotype and radiological features of MOG antibody-positive cases demonstrated varied spectrum of overlapping features with MS, NMOSD, ADEM, focal meningoencephalitis and Limbic encephalitis. These diverse range of manifestation highlights the need to consider MOGAD as an important differential diagnosis, as it a potentially treatable condition.

Award ID: 823

Serum Lipid Profile and Prognosis in Spontaneous Intracerebral Hemorrhage

Farsana MK, Sanghamithra P, Sueann Zachariah

Neurology, NIMHANS, Bengalure, Karnataka, India

E-mail: ayshafmk@gmail.com

Background and Aim: The clinical importance of spontaneous intracerebral hemorrhage (ICH) derives from its high incidence and 30-day mortality of 50%. Lower serum cholesterol levels were found to be an independent prognostic marker for ICH outcome in several studies worldwide. This study investigates the lipid profile abnormalities in ICH and the effects of serum lipid profile at admission on the mortality and morbidity outcomes of primary spontaneous ICH. Methodology: This prospective study was conducted in 100 patients above 18 years with spontaneous ICH who got admitted to Government medical college, Kottayam, Medicine department from August 2017 to July 2018, after obtaining ethical committee approval and written informed consent. Fasting lipid profile was estimated on the next day of admission. Short term outcome was measured in terms of modified Rankin scale (mRS) at one month follow up. Data was analysed using IBM SPSS software version 22. Result: 74% of the study population had good outcome and 26% had bad outcome with 30 day mortality of 13%. Patients with total cholesterol below 200 mg/dL, LDL below 130 mg/dL, and TG below 150 mg/dL were seen to have bad outcome. The mean total cholesterol levels in bad outcome group was significantly lower than those with good outcome (176.46 ± 38.37 mg/dL & 200.45 ± 51.56 mg/dL respectively). Serum total cholesterol and LDL were seen to have statistically significant association with outcome in spontaneous ICH. Conclusion: The mean lipid profile values in the study population was found to be in the normal range. Serum total cholesterol and low density lipoprotein levels at admission were found to be an independent predictor of outcome in spontaneous ICH, lower levels being associated with bad outcome.

Award ID: 824

Profile of Male Cerebral Venous Sinus Thrombosis Patients in a Tertiary Care Hospital in South India

Anush S, Subasree Ramakrishnan, Girish Baburao Kulkarni, Veerendrakumar M

Neurology, NIMHANS

E-mail: anush25292@gmail.com

Background and Aim:

Methodology: Retrospective analysis of CVST patients treated in our hospital from May 2020 to Febraury 2022 was done. Clinical data (risk factors, admission mRS, follow up mRS etc.,) and images of patients were obtained from case files and PACS respectively. Percentages were used to express the data Result: Out of the 151 patients admitted with CVST, 72 (47.6%) were males. Mean age was 35.68 (SD:10.8) years. Commonest clinical syndrome was Focal neurological deficits in 28 (43.9%), followed by raised ICT syndrome in 27 (38%). Most common risk factor was alcoholism in 54 (76.1%) followed by hyperhomocysteinemia in 53 (74%). Mean mRS at admission was 3.59 (SD:1.26), at discharge was 2.22 (SD:1.35). Superficial venous sinus involvement was seen in 68 patients (95%), deep sinuses in 4 (4.8%). Among the patients with superficial sinus involvement, combined Superior sagittal sinus (SSS) and lateral sinus involvement was seen in 38 (53.4%). SSS was the most common isolated sinus involved in 54 (76.1%). Mass effect was seen in 54 (76.1%), midline shift was seen in 32 (45.1%) while only 6 (8.5%) required surgery. All patients were treated with unfractionated heparin for 10 days with acenocoumarol overlapped on 6th day. INR was maintained between 1.5-2. At discharge 44 patients (62%) improved, 26 (36%) remained status quo and 3 (7.3%) expired Conclusion: Male CVST is relatively common in South India. Hyperhomocysteinemia, and alcohol consumption were plausible risk factors.

Award ID: 825

Comparison of Language Mapping by Using Magnetoencephalography (MEG) with fMRI

Vathsala A

Neurology, NIMHANS

E-mail: vats.369@gmail.com

Background and Aim: Resective surgery benefits in about 20-30% of patients with epilepsy. Language localisation helps in presurgical planning to minimise the post-surgical cognitive dysfunction. Aim of this study was to compare language localisation on MEG as compared with that of fMRI Methodology: This was a prospective-retrospective observational study conducted in the Department of Neurology and Neuroradiology, NIMHANS. Patients with refractory epilepsy who were above 18 years of age were included in the study after informed consent. After fMRI studies, MEG was done including a picture naming task and word recognition task. Event related field waveforms were used for source localisation. Dynamic statistical parametric mapping (dSPM) model was used for scattered pattern of activity. The agreement index was measured for localisation by using kappa statistical methods. Result: This study included 20 patients. Mean time-interval for activation of primary cortex was 122 msec. Localizations of Wernicke and Broca areas was observed in all patients with fMRI and in 15 patients with MEG. Wernicke area activity was most consistent in supratemporal sulcus (83%) and Broca area in inferior frontal gyrus (72%). Conclusion: Results from MEG and fMRI were concordant for language localisation.

Award ID: 826

The Clinico-Radiological Profile of Neuro Myelitis Optica Specrum Disorders (NMOSD)- A Cohort Study from a Tertiary Care Center in South India

Sri Sai Srujana Puppala, Anuja Patil

Neurology, Krishna Institute of Medical Sciences, Secunderabad, Hyderabad, Telangana, India

E-mail: srujana.pss@gmail.com

Background and Aim: To study the clinico-radiological pattern and treatment response among our patients with NMOSD. Methodology: This is an ambispective study who presented with an acute attack of NMOSD. The clinic- radiological patterns and the treatment response was studied. Result: 34 patients (F = 61.8%, M = 38.2%) were included and mean age at onset was 29.67 ± 12.85 (11- 59 years). The common presenting phenotype in isolation was LETM (29.4%), followed by ON (26.5%) and brainstem (11.8%). Central cord T2 hyperintensities were common in the dorsal cord. AQP4 Abs were positive in 32.3%, MOG Ab were poitive in 8.8% and 58.8% were seronegative. 16% had positive ANA profile. All the MOG positive had brainstem involvement. Delay in initiation of treatment showed significantly higher rate of relapse (38.5 weeks vs 9 weeks; p = 0.008). Rituximab (RTX) was commonly prescribed in 44.1% followed by azathioprine in 23.5%. Relapse was seen in 23.5% out of which 87.5% were females, 50% had LETM at onset, 75% were seronegative and 75% were on RTX. Follow up VEP showed improvement in 32.3% and worsened in 0.02% (LETM at onset and on RTX). Follow up MRS showed improvement in 88.2% and no improvement in 11.7% (seronegative, male patients, with LETM at onset and on RTX). Conclusion: Our study showed that females were commonly affected and LETM (dorsal cord) was the common initial presentation. Seronegativity, female gender, LETM at onset and RTX therapy were frequently seen in patients with relapse but were statistically insignificant. Delay in initiation of treatment resulted in higher relapses.

Award ID: 827

Sexual Dysfunction and Semen Analysis in Male Patients with Epilepsy on Anti-Seizure Drugs

Rajendra Kumar Sureka, Ishank Goel, Amit Agarwal

Neurology, Mahatma Gandhi Medical College, Jaipur, Rajasthan, India

E-mail: rsureka@rediffmail.com

Background and Aim: The etiology of disturbances of reproductive and sexual health in patients with epilepsy appears to be multifactorial, and both epilepsy itself and drugs used to treat it are implicated. The aim of this study was to assess the effect on anti-seizures drugs on semen analysis and sexual functions in male patients with epilepsy. Because the effects of AED therapy on human sperm motility are not well understood, this study was conducted. Methodology: This is a retrospective, hospital based, descriptive study to correlate between epilepsy, AEDs with semen analysis and sexual functions which was conducted in the Department of Neurology at a tertiary care center in Rajasthan (India) from May 2017 till Jan 2020. Result: The most common type of convulsions in all the age groups (15-25, 25-35 and >35 years) were GTCS type (76.6%, 85.7%, 66.6% respectively). The duration of epilepsy had statistically significant effect on liquefaction time (p < 0.05). Multiple types of seizures combined had statistically significant effect on the sperm count (p < 0.05). Valproate alone or in combination with other drugs had poor effect on the sperm parameters. With increasing duration of epilepsy, more patients had infertility, premature ejaculation and loss of libido. Conclusion: The most common type of seizures were GTCS in all age groups. The duration of epilepsy, combination of different types of seizures and polytherapy had poor effect on the sperm study parameters. The duration of epilepsy had poor effect on the sexual functions like infertility, premature ejaculation and loss of libido.

Award ID: 828

A Study of Long Term Remission in Juvenile Myoclonic Epilepsy (JME)

Phanindra Surapaneni, Sita Jaya Lakshmi, Anuja Ghogre Patil

Neurology, Krishna Institute of Medical Sciences, Secunderabad, Hyderabad, Telangana, India

E-mail: phanindrababu12345@gmail.com

Background and Aim: One third of the people with Juvenile myoclonic epilepsy (JME) do not respond to anti-seizure medications (ASMs) and in the remaining, seizure relapses are not uncommon when attempting to withdraw the ASMs. Aim is to analyze the long term remission in people with Juvenile Myoclonic epilepsy (JME) and to determine the predictors of long-term remission Methodology: This is a retrospective observational study of persons with JME with a follow-up of 5 to 20 years. The clinical details, inter-ictal EEG and ASM response details were recorded and statistically analysed. Result: A total of 231 patients were included; 64% of the patients had an age of onset of 11-18 years (mean age 14.6 years). 38% had positive family history. Nearly 52% patients had a delay in the diagnosis of JME by > 1 year. Among the seizure types, GTCS was noted in 96.1% patients while absence seizures was reported in 24.5%. Nearly 22% patients had all types of seizures. 36% had praxis induced seizures. Photo Paroxysmal Response (PPR) was noted on the EEG in 47% patients; Focal EEG abnormalities were noted in 29% patients. Motor seizures during sleep was reported in in 41.5% patients. The longest freedom of any seizure type for more than 10 years was achieved in 11%, more than 5 years achieved in 40%. Conclusion: Presence of all three seizure types, praxis induced seizures, positive Photo Paroxysmal Response and focal EEG abnormalities were the predictors of poor remission in people with JME.

Award ID: 829

A Case of Classical Stiff Person Syndrome with Cervical Spondylotic Myelopathy

Abbigari Vardhan

Neurology, Madurai Medical College, Madurai, Tamil Nadu, India

E-mail: harsha.abbigari3@gmail.com

Background and Aim: Stiff person syndrome is a rare neurological disorder, characterized by fluctuating muscle rigidity associated with painful spasms. It was described by Moersch and Voltman in 1956. Methodology: A 64 year old male, with history of Vitiligo in him and his daughter and son. Presented with multiple episodic waxing and wanning type of neurological illness characterized by rigidity in lower back muscles and bilateral lower limb muscles with painful muscle spasms, precipitated with tactile stimulation, unexpected noise, anxiety, not relieved by medication. No history of sensory, cerebellar, cranial nerve, higher mental function, extra pyramidal or psychiatric symptoms. On general examination, vitiligo present. On CNS examination, abdominal, paraspinal rigidity with spastic weakness of all four limbs. DTR is brisk in upper limb and exaggerated in lower limb, plantar extensor bilaterally. Gait is spastic with excessive lumbar lordosis. Result: Investigations - MRI brain and spine with contrast shows C5, C 6, and C7 disc protrusion with compression of spinal canal. On laboratrory blood investigations anti GAD positive 2246 IU/ML, ANA, auto immune profile, serum anti TPO are negative. EMG shows simultaneous and continuous firing of para vertebral motor units and reduced motor unit activity by administration of Diazepam and increased with unexpected sounds. Conclusion: Stiff person syndrome is highly heterogenous in clinical presentation, diagnostic findings disease progression and treatment response, but in most of the cases it is very diabling leading to a significant reduction of quality of life. It is divided into three forms : Classic SPS, Paraneoplastic SPS and Variants. In our case it is classic SPS with cervical spondylotic myelopathy (CSM).

Award ID: 830

Delayed Perihematomal Edema in Patients with Spontaneous Intracerebral Hemorrhage

baikuntha Panigrahi, Awadh Kishor Pandit, Achal Kumar Srivastava, Divya Radhakrishnan, Ayush Agarwal, Sohini Chakraborty, Bhargav Prajapati

Neurology, AIIMS New Delhi, India

E-mail: baikunthap199@gmail.com

Background and Aim: Perihematomal edema (PHE) occurs in patients with spontaneous intracerebral hemorrhage (sICH) due to both early and delayed neurological injury. The early PHE peaks at 4-5 days after sICH. Delayed PHE can be seen upto two to four weeks after the onset of ICH. Although the precise mechanisms causing delayed perihematomal edema are still unclear it is believed to occur due to erythrophagocytosis which leads to delayed neuronal cell death. The proposed mechanisms of delayed PHE include higher initial hematoma volume, hypertension, altered cerebral blood pressure regulation, decreased baroreceptor sensitivity. The objective of this observational case series is to identify potential causes of delayed PHE in sICH. Methodology: We describe three interesting cases of delayed PHE after sICH and their outcome. Demographic data and imaging data of these patients were recorded. Treatment received and mRS outcomes (as per treating doctor) were noted. Result: All three patients were young 38-45 years age, non-hypertensive, non-alcoholic and were having no conventional risk factors. PHE occurred at 45 days, 25 days and 20 days after onset of sICH respectively (range-20-45 days). All three patients continued to improve on medical management and are currently having mRS at 3 months (case 1 and 2) of 2 and 1 respectively and for case 3 mRS at 2 months is 5. Conclusion: PHE should be suspected in young patients following onset of sICH and can be treated with good outcome.

Award ID: 831

Quantitative Analysis of Strengths of Various Muscle Groups and its Implications in Practice

Hiral Halani, Hiral Amrut Halani, Satish Vasant Khadilkar, Harsh N. Oza

Neurology, Bombay Hospital and Institute of Medical Science

E-mail: heerdymnd@gmail.com

Background and Aim: Muscle strength assessment is done manually using Medical Research Council grading in clinical practice. Our study was designed to test the hypothesis that manual muscle testing (MMT) can cause erroneous judgment of actual weakness. This possibility generates out of the inherent variations in strengths of different muscle groups, which when tested against examiner’s pushing strength (supposedly constant), can potentially lead to misinterpretation of different muscle strengths. Methodology: A cross-sectional observational study was conducted at a tertiary-care centre. Quantitative muscle strength was assessed using Hand-Held Dynamometer (HHD) for 16 muscle groups. Statistical analysis was performed to identify any difference between mean strength of individual muscle groups and examiner’s pushing strength. Result: Total of 80 healthy volunteers in the age group of 18-60 years (40 males, 40 females) were assessed. The mean muscle strengths for different muscle groups ranged from 7.15 kg to 30.85 kg, maximum in the knee extensors. The average push strength of examiner was 16 kg as measured by HHD. A statistically significant difference was identified between the examiner’s push strength and the mean strength of different muscle groups; particularly of knee extensors, hip abductors and adductors, hip extensors, and shoulder abductors (p < 0.0001). Conclusion: Our study presents one of the few attempts at quantitative muscle strength assessment in the Indian population. This knowledge of inherent variation of different muscle strengths is important while doing MMT. The important clinical implications are discussed. This will help better assessment of the degree and distribution of actual muscle weakness in clinical practice.

Award ID: 832

Kiloh Nevin Syndrome and COVID -19 Infection

Shivani Bhagwat

General Medicine, KLES Dr Prabhakar Kore Hospital and MRS

E-mail: shivani.bhagwat2013@gmail.com

Background and Aim: Anterior Interosseous Nerve is a motor branch from the Median nerve, which innervates the pronator quadratus, flexor digitorum profundus 1 and 2, and flexor pollicis longus. The purely motor palsy of these muscles is called Kiloh Nevin Syndrome. It is a rare syndrome, affecting less than 1% of individuals. With the increasing number of coronavirus disease 2019 throughout the world, the neuromuscular complications are on the rise. Peripheral nerve injury can occur in patients with COVID-19 secondary to postinfectious inflammatory neuropathy, prone positioning, compression injury, or systemic neuropathy. Methodology: A 46-year-old right-handed gentleman presented with pain in his left upper limb for 3 weeks. It was dull constant ache in his left arm radiating to the forearm, severe, interfering with his daily activities. Later he developed difficulty in buttoning of his shirt with his left hand, when he noticed the inability to flex the terminal phalanx of the thumb, index and middle finger. Prior to this, he was infected with COVID 19, and treated symptomatically. A neurological examination along with a complete hemogram, serum creatinine, ESR, electromyography nerve conduction study was done. Result: On the basis of the history, the neurological examination and ENMG, a diagnosis of Kiloh Nevin syndrome was made. He received a course of prednisolone. Conclusion: With the onset following his recent COVID-19 infection, we can consider this to be a post-infectious sequelae. Hence as we still continue to fight the coronavirus battle, let’s keep the eyes open for peripheral neuropathy in addition to various other neurological sequelae.

Award ID: 833

Neuropsychological Outcome of Temporal Lobe Epilepsy Surgery: A Comaprision of Presurgical and Postsurgical Cognitive Functions

Yeruvena Ashok

Neurology, Nizams Institute of Medical Sciences, Hyderabad, Telangana, India

E-mail: drashok34@gmail.com

Background and Aim: Temporal lobe seizures represent approximately two thirds of intractable seizure population coming to surgical management. Resective surgery is the most effective treatment for TLE especially drug resistant epilepsy. To achieve better seizure control without causing loss of brain function is the goal of surgery. So neuropsychological assessment is needed before surgery to assess postoperative outcome after epilepsy surgery. AIMS; 1. To compare pre and post surgical neuropsychological functions of patients with temporal lobe epilepsy (TLE) surgery patients. 2. To analyse the predictors for a good neuropsychology before and after surgery. 3. To study quality of life and seizure outcome post surgery in patients with TLE Methodology: 30 patients with refractory TLE who underwent surgery in NIMS were included in the study. Neuropsychological assessment was done before and 6-12 months after surgery. The protocol evaluated attention, verbal memory, visual memory, executive function, visuomotor integration and intelligence before and after surgery Result: 83.3% of patients had improved neuropsychology after surgery. Distribution of neuropsychology outcome after the surgery is statistically significantly associated with drug dose reduction, seizure free status and improved quality of life in the study group (P-value < 0.05). Distribution of neuropsychology outcome after surgery is not statistically significantly associated with several factors such as age group, sex, side involved, age of onset, no. of drugs, frequency of seizures and duration of seizures (P-value > 0.05]. Conclusion: 1. There is improved neuropsychological functions of patients with temporal lobe epilepsy after successful surgery irrespective of having bilateral or unilateral temporal cognitive dysfunction before surgery. 2. There is significant reduction in drug dose, number of seizures and improvement in quality of life with surgery which significantly impacts the improvement of neuropsychological function post surgery. 3. Factors like age, age of onset of epilepsy, gender, number of AEDs, duration of seizures, side of involvement, frequency of seizures are not able to predict the neuropsychology significantly either before or after surgery.

Award ID: 834

A Case Report of Gaint Axonal Neuropathy

Salijamala Hari, C. Justin

Neurology, Madurai Medical College, Madurai, Tamil Nadu, India

E-mail: sahasra.moni@gmail.com

Background and Aim: Giant axonal neuropathy is a rare hereditary neurodegenerative disorder characterised by accumulation of excess neurofilaments in the axons of PNS and CNS which hampers signal transmission. It manifests in infancy and early childhood and slowly progressive. They have characteristic curly kinky hair, everted feet and crouched gait Methodology: A 11 year female child, product of non consangenious marriage with out birth asphxia presented with insidious onset progressive weakness of proximal and distal muscles of both upper limbs and lower limbs since the age of 8 years with out sensory symptoms, cranial nerves involvement and seizures with family H/o of similar illness in her younger brother. On examination curly kinky hair, Scoliosis, bilateral Foot drop, Pes planus, Hammer toes Trophic ulcers, Hypotonia, wasting of small muscles of both hands and foot muscles Muscle power shows distal weakness more than proximal waekness in all 4 limbs and Absent DTR. Result: NCS shows features of sensory motor axonal neuropathy and SRS is absent in both UL and LL Autonomic involvement. ENMG s/o Neurogenic pattern. Genetic testing positive for GAN gene exon3 Conclusion: Treatment is mainly supportive, GAN progresses slowly as neurons degenerate and die. Brain and spinal cord involved slowly causing gradual decline in mental function and seizures. children become wheelchair dependent in second decade of life If GAN gene testing performed in first case such that a prenatal diagnosis could be performed in second case, the birth of the index cases could be avoided.

Award ID: 835

A Case of Area Postrema Syndrome

Poornam Naveena Venkateswaran, Balaji Gopalan, Hariharan S, Elangovan S

Neurology, Govt Chengalpattu Medical College and Hospital, Tamil Nadu, India

E-mail: vpnaveena30@gmail.com

Background and Aim: Although area postrema syndrome is a core clinical feature of NMOSD, it is frequently misdiagnosed as gastroenteritis or other systemic diseases. Here I describe the diagnostic challenges in an area postrema syndrome with accompanying bulbar palsy and dysautonomia. Methodology: A 25 year old previously healthy female presented to us with complaints of vomiting for the past 20 days which was treated as gastroenteritis elsewhere and did not show improvement. She also complained of dysphagia and slurring of speech for 3 days. She had a history of diplopia intermittently during the evenings. Examination revealed normal spinomotor examination with lingual dysarthria, depressed gag reflex and tongue weakness. MRI brain was normal. On day 2 of admission she had acute worsening of dysarthria and dysphagia and had labile bp variations. Examination revealed absent gag, tongue weakness, hypotonia and pyramidal pattern of weakness. She was started on IvIg suspecting GBS and also given a trial of pyridostigmine. Repeat MRI brain revealed T2 and FLAIR hyperintensities in dorsal medulla and occipital regions. Her serum Aquaporin 4 was negative. However since she fit into the criteria for NMOSD, she was started on steroids and showed improvement. Result: A presentation of subacute vomiting and hiccups, with acute bulbar symptoms eventually developing motor manifestations and dysautonomia with negative serum Aquaporin 4 eventually diagnosed as a case of NMOSD. Conclusion: Neuromyelitis Optical spectrum of disorders has a varied clinical presentation predominantly transverse myelitis (59%) and optic neuritis (17%). Acute brainstem syndrome as the presenting complaint has been reported in 14%. Here we highlight the accompanying features of dysautonomia in a case of NMOSD

Award ID: 836

Clinical Presentation, Predisposing Factors, Radiological Features and Outcome of Unusual Posterior Reversible Encephalopathy Syndrome: A Descriptive Case Series Study from a Tertiary Care Centre from South India

Sandhya Manorenj, Reshma Sultana Shaik, Sravan Kumar Marupaka, Rindha Venepally Rao

Neurology, Princess Esra Hospital, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Background:Posterior reversible encephalopathy syndrome (PRES) classically involves bilateral parieto-occipital lobes and is usually reversible. Classically occur due to disruption of the blood-brain barrier secondary to elevated intracranial pressures or endothelial injury. These results in subcortical vasogenic edema well appreciated in T2/ FLAIR sequence of MRI brain. Atypical variant of PRES includes involvement of brainstem, cerebellum, corpus callosum, basal ganglia, thalami, periventricular white matter, and other cerebral areas, with more common areas including the frontal lobes and inferior temporal lobes. Aim:To describe the clinical and radiological features of unusual PRES. Methodology: Methods: A case series study conducted over a period of one year, collecting predisposing factors, clinical presentation, radiological features and outcome among PRES with atypical variants and unusual presentations Result: Results: Among six cases of unusual PRES identified, age range was 1-60 years. Clinical presentations were cortical blindness and seizures [n = 2]; low GCS of 7 [n = 1]; acute vestibular syndrome:[n = 1]; generalized seizure with autonomic dysfunction:[n = 1]; giddiness and ataxia [n = 1]. Predisposing factors were accelerated Hypertension [n = 3]; hypotension [n = 1], sepsis [n = 3]; bingealcoholism [n = 1]. Unusual risk factor was ulcerative colitis on infliximab treatment [n = 1] but with typical PRES. 83.3% cases had multiple predisposing factors. Atypical radiological features were optic nerve involvement [n = 1], brainstem involvement [n = 1], brainstem PRES with acute stroke [n = 1]; cerebellum involvement and frontal involvement [n = 1]; cerebellum involvement with obstructive hydrocephalus [n = 1]. Death occurred in two cases Conclusion: Conclusions: Study enhances the understanding of atypical PRES. Prompt recognition is important to avoid delay in diagnosis and treatment. Treatment of predisposing factors serves to reverse PRES symptoms and optimize outcome

Award ID: 837

A Rare Presentation of NMOSD as a CP angle Lesion- Isolated Case Report

Sivaji Muruga, Jawahar M, Lakshmi Narasimhan Ranganathan, Sushma Chandragiri

Neurology, Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: Sivajimuruga@gmail.com

Background and Aim: NMOSD is an inflammatory disease associated with antibodies to aquaporin-4 (AQP4), which has distinct clinical, radiological and pathological features. Methodology: Here we report a case with such atypical presentation. A 48 year old male patient with diabetes and hypertension presented with 2 weeks history of swaying to either sides while walking, tremulousness of right hand, right side hard of hearing and followed 5 days later by deviation of angle of mouth to left side with difficulty in closing right eye. Examination showed right LMN 7th palsy, right sensory neural hearing loss, bilateral exaggerated DTR with normal power and flexor plantar. Bruns nystagmus is present. Right cerebellar signs with gait ataxia present. Result: Investigations done- CSF analysis includes protein of 8.7 mg/dl, glucose is 121 mg/dl, acellular smear. VEP P100 latency prolongation in right eye. Vasculitic work up and serum ACE is negative. Work up for primary demyelination negative. MRI of brain with spine screening showed T1 hypointense and T2/FLAIR hyperintense lesion in right MCP and adjacent cerebellar hemisphere without any diffusion restriction and on contrast the lesion shows incomplete ring enhancement. Another similar lesion is noted in splenium of corpus callosum. Ill defined T2 hyper intensity noted in spinal canal at C4 vertebral level. These findings suggestive of possibly active demyelination. Conclusion: Apart from the typical MRI sites like LETM, optic nerve involvement, periventricular lesions, other atypical imaging sites were reported in the literature. Here we report a case with such atypical presentation.

Award ID: 838

Anti-Epileptic Drugs and Hyperpigmentation

Bhavna Pandey

MBBS Student, II Year, SVIMS-SPMC (W), Tirupati, Andhra Pradesh, India

E-mail: bhavnap2021@gmail.com

Background and Aim: Anti-epileptic drugs are commonly associated with adverse events; however, their dermatological side effects have been rarely reported. This study aims to review the literature on hyperpigmentation caused by anti-epileptics. Methodology: A PubMed search was done on 29 May 2022, using keywords, [Antiepileptics and hyperpigmentation]. A total of 45 articles appeared that were referred for the review. Result: Epilepsy is characterised by the type of seizure. Anti-epileptic drugs are the first treatment option in patients with epilepsy. One of the typical adverse reactions of antiepileptic drugs is hyperpigmentation. Commonly used anti-epileptic drugs, valproic acid, lamotrigine, gabapentin, and phenytoin all have been reported to cause cutaneous photosensitivity. A 2017 case report described a 23-month-old child who presented with hyperpigmentation and onychomadesis post valproic acid treatment.1 Another case has been reported from Kolkata describing the development of a purple, painful, edematous hand 2 hours after I.V. Phenytoin sodium was loaded to treat generalised tonic-clonic seizures in a 45-year-old female.2 Skin pigmentation is induced as a result of various mechanisms like the formation of a drug-pigment complex, induction of hyper melanosis, or direct accumulation of the drug in the skin. Conclusion: Commonly used antiepileptic drugs, valproic acid, lamotrigine, gabapentin, and phenytoin all have been reported to cause cutaneous photosensitivity. Obtaining a thorough medical history is crucial for making a diagnosis of drug-induced pigmentation. Awareness of hyperpigmentation as a common complication of anti-epileptic drug administration and preventing it’s use in susceptible patients is important.

Award ID: 839

Diagnostic Yield of Combined Sensory Index in Patients with Symptoms of Mild Carpal Tunnel Syndrome

Soumya Parne

Neurology, Osmania Medical College, Hyderabad, Telangana, India

E-mail: parne.soumya@gmail.com

Background and Aim: Combined sensory index is a sensitive index which utilizes the sum of latency differences between the median and ulnar; and median and radial sensory nerve action potentials across the wrist. It is useful when the routine median sensory and motor nerve action potentials are normal, but the clinical suspicion of carpal tunnel syndrome is high. The aim is to study the diagnostic value of combined sensory index in patients with symptoms of mild carpal tunnel syndrome with normal routine median nerve action potentials. Methodology: A total of 100 patients who presented to Osmania General Hospital with complaints of neck pain or numbness or paresthesia of upper limbs with normal clinical examination and routine median conduction studies. A set of 3 tests as described below was done for all the patients. First test: Median Vs Radial thumb, Technique: Antidromic Second test: Median Vs Ulnar ring finger, Technique: Antidromic Third test: Median Vs Ulnar-palm to wrist, Technique: Orthodromic The combined sensory index is a sum of these three latency differences. A value of more than 0.9 ms is considered as positive for carpal tunnel syndrome. Result: Out of 100 patients, 12 patients had a CSI >0.9. In 5 of these patients, ultrasonography of wrist was suggestive of carpal tunnel syndrome. Conclusion: This study shows that Combined sensory index is a useful diagnostic test for patients with high index of suspicion for carpal tunnel syndrome but normal clinical examination and normal routine median sensory conduction study.

Award ID: 840

Sleep Pattern and Overnight Polysomnographic Findings in Stroke Patients in the Acute and Chronic Stages

Mythirayee Sivasubramanian, Srijithesh PR, Ravi Yadav, Doniparthi Venkata Seshagiri, Nitish L. Kamble, Girish B. Kulkarni, Sanjib Sinha

Neurology, National Institute of Mental Health and Technology

E-mail: mythugranger@gmail.com

Background and Aim: Background In the acute stage of a stroke, sleep disruptions are common. However, there is limited literature on the changes in sleep architecture in chronic stage of these patients. Aim To evaluate the sleep parameters in patients with acute stage of stroke and follow-up. Methodology: In this study ischemic stroke patients was prospectively recruited to evaluate changes in sleep architecture. Patients underwent overnight polysomnography twice- initially within 2 weeks of stroke onset and later after 3 months. Sleep was scored according to AASM criteria. Result: The records of 56 patients who had a follow-up PSG were analyzed in the study. 23 patients had AHI < 5 (41%), 15 patients had AHI between 5-14 (26.7%), 11 patients had AHI between 15-30 (19.6%) and 7 patients had AHI >30 (12.5%). There is no significant difference between the AHI of the initial and follow-up study. The number of long duration of obstructive apnea (15%), central apnea (11%), and hypopnea (23%) reduced during follow-up. There was a significant difference between total sleep time [304.7 ± 105.4 vs 350.6 ± 81] and REM periods [44.3 (24,62.5) vs 63 (96.3, 34)] between the studies. The sleep efficiency [64.7 ± 22.1 vs 74.7 ± 15.8] tends to improve in the follow-up study. There is no significant difference between the other sleep parameters of the first and follow-up study. Conclusion: There was no significant difference in the AHI in the first and follow-up study. There was a reduction in long-duration apnea and hypopnea as well as a trend of improvement in sleep efficiency during the chronic stage of stroke.

Award ID: 841

Eltrombopag Related Cerebral Venous Thrombosis (CVT) and Ischemic Stroke in Refractory Immune Thrombocytopenic Purpura (ITP) Patients – A Case Series

Rohit BG, Vivek Nambiar, Jaffer Wali

Neurology, Amrita Institute of Medical sciences

E-mail: raghuthedeathknight@gmail.com

Background and Aim: Eltrombopag is a thrombopoietin-receptor-agonist (TPO-RA) approved for treatment of patients with chronic ITP who are unresponsive to immunosuppressive therapy1. Adverse effects of the agent include thrombotic episodes due to the release of platelet materials into the circulation2. Methodology: We report incidence of eltrombopag related CVT and CVA in 5 chronic ITP patients. Result: We report 5 cases diagnosed with chronic ITP on Eltrombopag. Two of them developed CVT within 3 days of initiation of Eltrombopag. Two of the patients died due to raised intracranial pressure secondary to extensive CSVT. One patient developed Acute CVA while on Eltrombopag. One patient developed CVT while on Eltrombopag later found to have covid infection andBeta2glycoprotein antibodies. Conclusion: A review on safety profile and efficacy of Eltrombopag study revealed 2–6% of patients receiving Eltrombopag had thromboembolism3. Interestingly, in our case series all patients were female. Female genders have higher risk of CVT than male genders, owing to hormonal factors and high frequency of autoimmune illnesses. We could infer that thromboembolism associated Eltrombopag was not uncommon from these findings. Platelet activation has been proposed as the possible mechanism in Eltrombopag related thromboembolic events4. Patients with thromboembolic risks should be aware and use Eltrombopag with caution. The diagnosis is important as the decision to anti-coagulate is vital. The decision to anti-coagulate in patients especially with low platelet count poses real challenge. Physicians should be aware of this adverse event and warn patients when prescribing Eltrombopag.

Award ID: 842

Serum 25-Hydroxy Vitamin D Level in Diabetic Peripheral Neuropathy with Clinical and Electro-Physiological Correlation

Akash Kharat

Neurology, B.R.D Medical College, Gorkhpur, Uttar Pradesh, India

E-mail: aakashkharat123@gmail.com

Background and Aim: Diabetic peripheral neuropathy (DPN) is one of the most common troublesome complications of diabetes mellitus (1). Apart from role of vitamin D in calcium and bone metabolism, researches have shown its relationship with certain neurological disorders like multiple sclerosis (2), Parkinson’s disease and cognitive decline in elderly. However in recent years there has been accumulating evidence regarding its role in development of diabetes and its complications like peripheral neuropathy. Methodology: Total 100 patients was recruited in this study from out patient and in patient department. Patients of diabetes mellitus were assessed for diabetic peripheral neuropathy by clinical and electrophysiological criteria and evaluation of serum level of 25(OH) vitamin D levels was done. The neuropathy disability score (NDS) and neuropathy severity score (NSS) which clinically assessed severity of neuropathy where included in study. Result: In our study, Neuropathy disability score was severe in 39 patients (39%), moderate neuropathy disability score was present in 53 patients (53%) and mild neuropathy disability score was present in 8 patients (8%). In our study most of patients (n = 68, 68%) scored severe on Neuropathy severity score, while 29 patients (29%) had Neuropathy severity score in moderate range, 3 patients (3%) had mild score on neuropathy severity score. Vitamin deficiency was present in majority of patient (n = 53, 53%), Insufficient level of vitamin D was present in 39 patients (39%). Most of patient (n = 87, 87%) had abnormality on nerve conduction studies, while 13 patients (13%) of them had normal nerve conduction study. Conclusion: The present study with previous clinical studies demonstrated that vitamin D deficiency has a significant role and independent association with DPN. Also vitamin D serum levels correlate with the severity of neuropathy in patient with T2DM.

Award ID: 843

A Retrospective Study Comparing the Clinico-Radiological Characteristics of NMOSD Presenting with Letm & Double Sero-Negative Letm in a Tertiary Care Hospital in Eastern India

Ankur Banik, Indranil Dutta, Amrit Chattopadhyay, Srabani Ghosh, Debsadhan Biswas

Neuromedicine, Medical College, Kolkata, West Bengal, India

E-mail: banik.ankur@gmail.com

Background and Aim: Longitudinally extensive transverse myelitis (LETM) is characterized by spinal cord lesion extending ≥ three vertebral segments. LETM is classically related AQP4-Ab and MOG-Ab. However, some patients are double sero-negative (DSN). We aim to study and compare the clinical, etiological and radiological profile of NMOSD and DSN LETM. Methodology: This retrospective study was conducted at Neurology department, Medical College, Kolkata. The following information of all LETM cases from March 2020 to March 2022 were obtained: demographic profile, clinical presentations, neuroimaging, AQ4 and MOG serology and other relevant investigations. Result: Amongst a total of 27 LETM patients,12 were NMOSD and 15 were DSN. Majority of NMOSD patients were females (66.67%) in contrast to DSN group (males 60%). Predominant presentation was quadriparesis (58.33%) in NMOSD group while paraparesis (60%) in DSN group. The disability severity was more in NMOSD group (91.67% mRS 3 or 4 vs 66.67%) and there was no correlation between the severity and the number of segments involved in either of the two groups. Predominantly central cord involvement was seen in as high as 83.33% patients of NMOSD, unlike in the DSN patients (66.67%). Optic nerve (41.67% vs 6.6%) and brain involvement (58.33% vs 6.6%) were significantly more in NMOSD group. Conclusion: NMOSD is the most important and devastating cause of LETM, predominantly affecting females. DSN LETM affects males more and differ in clinical profile. Optic neuritis, quadriparesis, presence of brain lesions and central cord involvement are more common in NMOSD.

Award ID: 844

Are Salivary Oxidative Stress Markers Altered in Parkinson’s Disease?

Jyoti Rungta, Akash Roy, Supriyo Choudhury, Sabbir Ansari, Piali Chatterjee, Rochana Pramanik, Sanjit Dey, Hrishikesh Kumar

Department of Neurology, Institute of Neurosciences Kolkata

E-mail: jyotirungta95@gmail.com

Background and Aim: There is a hunt for identifying a reliable non-invasive tool to diagnose Parkinson’s disease (PD). Blood samples of PD patients show enhanced oxidative stress, and it increases with the severity of the disease. The aim of this study is to examine if salivary oxidative markers are also raised in PD and if it has any relation with disease severity or phenotype. Methodology: Twenty-four PD patients and twenty-four age-matched healthy controls were recruited. Motor and cognitive severity assessments were undertaken. The PD patients were categorized into Tremor dominant (TD) and Postural instability and gait disorder (PIGD) phenotypes from UPDRS III. Standard spectrophotometric techniques were used to determine the activity of salivary antioxidant enzymes such as reduced glutathione (GSH), superoxide dismutase-1 (SOD-1) and catalase. Result: Salivary Catalase activity was found significantly lower in PD compared to Control (p < 0.01). Salivary reduced GSH concentration was found significantly lower in PIGD compared to TD. However, there wasn’t any association of these markers with the disease severity of PD. Conclusion: This study detected increased oxidative stress (catalase activity) in PD salivary samples. Although we could not observe any association with disease severity but reduced GSH was significantly different in PIGD and TD phenotypes.

Award ID: 845

Automated Hypometabolism Detection in FDG PET Improves Epileptogenic Zone Localization in Drug Resistant Epilepsy

Shameer Aslam, Natesan Damodaran, Ramiah Rajeshkannan, Manjit Sarma, Siby Gopinath, Ashok Pillai

Neurology, Amrita Advanced Centre for Epilepsy, Amrita Institute of Medical Sciences and Research Center

E-mail: aslamshameer165@gmail.com

Background and Aim: Interictal 18F FDG PET is an important imaging modality to localize the epileptogenic focus in drug resistant epilepsy patients. In this study, we described an automated interhemispheric PET asymmetry analysis technique to detect hypometabolism. Methodology: This study included 19 drug resistant epilepsy patients who underwent PET-MR acquisition for epilepsy presurgical evaluation. All patients were followed-up for > 12 months of postoperative response. Voxel-based asymmetry index of PET was calculated following the interhemispheric anatomical asymmetry correction. The asymmetry index images were converted to z-score and thresholded at z>3, 4 and 5 with and without clustering. All patients were analyzed using the described technique and the results were retrospectively validated from the surgical intervention. Result: We found that 16 of 19 patients with Engel 1 postsurgical outcome had high degree of overlap between the detected hypometabolism (with z>4 threshold) and resection cavity. One patient with Engel 2 and two patients with Engel 3 outcome had no overlap of detected hypometabolism and the resection cavity. Conclusion: The novel technique of voxel-based PET hypometabolism detection was proved to be efficient in automatically detecting and delineating the PET hypometabolism independent of normative control PET data. Prospectively, this technique may help in planning the intracranial electrode placement or resective surgery for better outcome.

Award ID: 846

Deciphering the Role of Casein Kinase 2 (CK2) in Mesial Temporal Lobe Epilepsy

Priya Priya, Nitin Yadav, Arpna Srivastava, Ridhima Tandon, Jyotirmoy Banerjee, Sanjeev Lalwani, MC Sharma, Fouzia Siraj, Manjari Tripathi, P Sarat Chandra, Aparna Banerjee Dixit

Dr. B.R Ambedkar Center for Biomedical Research, University of Delhi, New Delhi, India

E-mail: priyarajput291@gmail.com

Background and Aim: NMDA receptors (NMDARs) are key mediators of excitatory synaptic transmission in the brain. Studies have shown that CK2 regulates the NMDAR activity through the phosphorylation of GluN2B Ser1480 resulting in phosphorylation-dependent endocytosis of NR2B and an increase in the synaptic NR2A expression. Therefore, this study is designed to test the hypothesis that altered CK2 functions may contribute to hyperexcitability in MTLE. Methodology: For this study, surgically resected hippocampal tissue specimens of 23 patients and 17 controls were obtained. mRNA levels of CK2α, CK2β, NR2A and NR2B were evaluated by quantitative real-time PCR and the expression of proteins was studied by western blotting. CK2 activity was measured by kinase assay. Expression of mRNA and protein were also evaluated in the acute and chronic pilocarpine model of TLE in the Hippocampus, ATL and Neocortex regions of the brain. Result: A significant increase in CK2α1, CK2β1 and NR2A expression was observed in MTLE patients. Kinase activity was significantly higher in MTLE patients. A significant increase in CK2α2 was observed in the chronic model of TLE as compared to the respective control with no significant changes in the acute TLE model. Conclusion: Our results suggest that casein kinase 2 may contribute to hyperexcitability via modulating the regulation of NMDA receptors in MTLE. This new information greatly improves our understanding of the molecular mechanisms and synaptic plasticity involved in the pathogenesis of MTLE, and CK2 may represent new potential therapeutic targets.

Award ID: 847

A Rare Case of LGMD2J / LGMDR10 TTN Gene Mutation from India

Aswin Surjit, Boby Varkey Maramattom, Boby Varkey Maramattom, Boby Varkey Maramattom, Hansashree Padmanabha, Ravindranadh Mundlamuri, Sneha Kamath, Boby Varkey Maramattom, Kamalesh Chakravarty, Vivek Lal, Parampreet Singh Kharbanda, Sucharita Ray, Aastha Takkar Kapila, Abdul Qavi, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Abdul Qavi, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Abdul Qavi, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Saraf Udit Umesh, Sudheeran Kannoth, Vivek Nambiar, Siby Gopinath, Anand Kumar Anandakuttan, Annamma Mathai

Internal Medicine, Aster Medcity, Kochi, Kerala, India

E-mail: surjitaswin@gmail.com

Background and Aim: TTN gene defect was first described in a Finnish family and later in Romania, Hungary and China. It is a rare autosomal recessive condition causing Tibial Muscular Dystrophy, Cardiomyopathy and Limb Girdle Muscular Dystrophy. Here we report a case of Limb Girdle Muscular Dystrophy due to TTN mutation (Titinopathy). Methodology: A 45 year male born of non-consanguineous marriage came with complains of fatigue and progressive lower limb weakness for past 8 years. He underwent L4 – S1 laminectomy 12 years back. His maternal uncle had possible similar illness. On examination he had bilateral lower limb proximal muscle weakness, DTR reduced and no sensory involvement. Investigations - CPK 1671 U/L. EMG showed irritable myopathic potentials. MRI reported atrophy of bilateral paraspinal muscles. Muscle biopsy showed scattered atrophic fibers with multinucleation. Result: He was evaluated for inflammatory muscle disease, metabolic disorder and genetic evaluation of LGMD. Mitochondrial genetic testing and metabolic screening was negative. The whole exome sequencing showed two heterozygous variants in the gene TTN (OMIM*188840) - Variants of Unknown Significance. ECHO showed borderline LVH. Conclusion: Titin is a major component of muscle sarcomere affecting skeletal and cardiac muscles. LGMD2J is a very rare condition and varied outcomes ranging from slow progressive muscle weakness to sudden respiratory depression. The patient could present with features of inflammatory muscle disorders or metabolic storage disorders. The evaluation of genetic profile will confirm the diagnosis. The patient should also be evaluated for cardiomyopathy and respiratory muscle depression.

Award ID: 848

A Case of Metronidazole Encephalopathy & Peripheral Neuropathy

Mangalapalli Vijay, Sakthi Velayutham S, Malcom Jeyaraj K, Sowmini P R, Viveka Saravanan R, Mugundhan K

Neurology, Stanley Govt College and Hospital

E-mail: vijaymangalapalli@gmail.com

Background and Aim: Metronidazole induced neurotoxicity is very rare and presents as peripheral neuropathy, headache, ataxia, seizures, and encephalopathy1. We report a patient who consumed Metronidazole for 1 month and presented with neurotoxicity. Methodology: A 37 year old male presented with complaints of unsteadiness while walking, tremors of hands, crural dystonia, emotional and mild cognitive disturbances of 1 month duration. Patient also had tingling, numbness & distally predominant flail type of weakness of upper and lower limbs. Patient had history of gastroenteritis 1 month back, took antacids and Tab. Metronidazole 800 mg per day for 1 month duration. Gastritis improved but later he developed neurological symptoms. Result: MRI brain on admission showed symmetric areas of T2 and FLAIR hyperintensities and restricted diffusion in bilateral dentate, red nuclei, dorsal pons, and caudate nuclei and splenium of corpus callosum2. NCS revealed sensory motor axonal changes of all limbs. Metronidazole toxicity was suspected and metronidazole was stopped. Patient’s neurological symptoms improved gradually over the next 2 weeks. Repeat MRI was done after 2 weeks was normal. Conclusion: Metronidazole causes reversible brain lesions. Metabolites of metronidazole inhibit RNA protein synthesis which can lead to neurotoxicity. Once the medication is discontinued, the syndrome improves within days or weeks. Physicians should be aware about neurologic side effects of metronidazole, a commonly prescribed antibiotic.

Award ID: 850

Unmasking of Elderly Onset Dementia

Sravanthi Guduru

Neurology, NIMHANS

E-mail: sravanthireddyguduru@gmail.com

Background and Aim: Dementia is one of the common neurological illness in elderly. A 68 year old male presented with progressive cognitive decline followed by parkinsonism with a history of severe RTA 10 years before cognitive symptoms. Methodology: We evaluated patient for reversible causes of dementia. Serum b12, folate, homocysteine was normal. HIV, HBsAg, HCV, VDRL was negative. Serum ANA, ANCA were negative. Serum autoimmune and paraneoplastic panel was negative. EEG showed left sided focal slowing. Serial MRI brain suggestive of progressive increase in white matter changes started in frontal lobe asymmetrically and later involved all lobes associated with mild chronic subdural hematomas. Possibilities thought was post traumatic neurodegeneration vs genetic causes. Whole exome sequencing was sent. Result: Whole exome sequencing suggestive of CSF 1R Gene mutation. Conclusion: This patient was atypical for CSF 1R in view of age. Diffuse progressive white matter changes with hematomas in first instance would favour post traumatic encephalomalacia changes. In view of almost 10 years gap between trauma and cognitive decline other causes were also included.

Award ID: 851

Opsoclonus Myoclonus Ataxia - Rare Case

lakshmi Prathyusha Mandapati, Balaji G, Hariharan S, Elangovan S

Neurology, Chengalpattu Medical College and Hospital, Tamil Nadu, India

E-mail: lprathyusha11@gmail.com

Background and Aim: INTRODUCTION : The opsoclonus–myoclonus–ataxia syndrome (OMS) is a neurological disorder, first described in infants by Kinsbourne. Most cases in adults are associated with a neoplasm or a viral infection; however in few cases no underlying aetiology can be identified. It is hypothesised to be due to an underlying autoimmune mechanism, multiple antibodies have been linked to it Methodology: CASE REPORT : A 47 yr female known case of hypothyroidism and diabetes presented with subacute onset tremulousness involving all 4 limbs and unsteadiness 1 wk following an episode of fever, which progressed over a period of 3 days and she required the help of others for her daily activities. On examination : Her general examination and vital signs were normal. Her eyes showed fast frequency, high amplitude, multidirectional conjugate movements in primary position and conjugate gaze. Myoclonus present involving all 4 limbs. On testing co ordination she had limb ataxia left > right and truncal ataxia. Investigations showed normal hemogram, liver and renal function tests. TSH 31 mIU/ml, TPO ANTIBODIES 338.76 IU/ml. HIV positive. CSF studies showed normal sugar and elevated protien. Serum EBV positive. AntiGAD ANTIBODY 55.37 IU/ml (positive) MRI BRAIN plain was normal and contrast showed enhacement in the left middle cerebellar peduncle. In view of opsoclonus myoclonus ataxia pt was treated with IV methylprednisolone for 5 days. Sodium valproate and leviteracetam was started for myoclonus. Pt improved symptomatically. Result: DISCUSSION : This is a case of opsoclonus myoclonus ataxia syndrome. OMAS is a syndrome that was first described in infants. In adults, the majority are paraneoplastic origin, but other aetiologies include demyelination, infections, drug induced and other autoimmune processes. But classically, the autoimmune hypothesis of OMAS has been the mainstream in this field. The main evidence stems from multiple classes of antibodies that have been linked to OMAS. In this case it is linked to multiple antibodies Conclusion: OMAS is reported in infective/auto immune /para neoplastic aetiologies however established markers are detected only in a few patients to establish a diagnosis. There were multiple antibodies detected in our case - HIV/TPO/ANTI GAD/ EBV each one of which are individually reported as the cause of OMAS. This establishes the underlying autoimmune process.

Award ID: 852

An Interesting Case of Hypoglossal Nerve Palsy Secondary to Neurovascular Conflict

Ravi L A, Chandramouleeswaran V, Lakshmi Narasimhan R, Krishnakumar B

Institute of Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: laravi92@gmail.com

Background and Aim: Isolated hypoglossal nerve palsies are by themselves a rare entity but a hypoglossal nerve palsy caused by a neurovascular conflict due to anomalies of the vertebro-basilar system has been reported in very few instances, less than 15 cases reported in literature. This case is presented to emphasize neurovascular conflicts as a reversible causes of isolated hypoglossal nerve palsy. Methodology: we present the case report of a 49 years old male who presented with left sided unilateral headache for the past 2 months followed 15 days later by sudden onset deviation of tongue to the left on protrusion, difficulty in manipulating food on the left side of the tongue, slurring of speech and thinning of left half of tongue. On examination, there was wasting of left half of tongue with prominent fasciculations. There were no other neurological deficits. Result: He was thoroughly evaluated for other causes, including infections, neoplasms, connective tissue disorders and carotid artery dissection. MRI brain contrast with CISS sequence revealed enhancement of the left hypoglossal nerve at the hypoglossal canal and a twig from the vertebral artery seen compressing the nerve. The patient was treated with a pulse course of methylprednisolone and showed improvement. He was planned for microvascular decompression by neurosurgeon. Conclusion: Though our case report rare, we emphasise on keeping neurovascular conflicts as one of the reversible causes of isolated hypoglossal nerve palsy.

Award ID: 854

Neurocognitive Profile in Anti-LGI1 Associated Limbic Encephalitis : Pre and Post Immunotherapy Clinical Correlation, Rehabilitation and Outcome

Sonal Vijay Chitnis, Sujit Aabaji Jagtap

BVDU School of Audiology Speech Language Pathology, Bharati Vidyapeeth Deemed to be University Medical College Pune, Maharashtra, India

E-mail: sonalc123@gmail.com

Background and Aim: Anti LGi1 associated autoimmune limbic encephalitis (ALE) cognitive behavioural profile is clinically challenging with it’s heterogenous presentation across amnestic, aphasic, apractic, dysexecutive spectrum with fairly preserved activities of daily living vs few typical rapidly progressive dementia profiles. There is scarcity of literature discussing clinical diagnosis, intervention, rehabilitation and outcome in anti LGI ALE. Current study aimed to investigate neurocognitive profile, clinical correlation, immunotherapy, rehabilitation and overall pre clinical and post outcome. Methodology: 7 Out of 18 (mean age 58 years, mean age of onset 54 years) confirmed cases with autoimmune encephalitis were carefully investigated and analysed retrospectively (2016-2021) for their clinical history, imaging, neurocognitive behavioural profile in present study. ACE -III Marathi, CDR, detailed clinical history, imaging, VEEG, pre and post immunotherapy were explored to understand ALE. Cognitive communicative therapy for recall, dysfluencies, Verbal DDK speech non speech sensory motor integration along with specific case based taillor made intervention was provided. Result: Anti LGI ALE present with seizures, cognitive decline however LGI1 limbic encephalitis remains rare, difficult to identify but often treatable if timely intervened. Poor working memory, apractic amnestic skills, faciobrachial dystonic seizures (FBDS), and hyponatremia seen in 80% population. 90% cases had no significant decline on ADL but overall altered cognition behavior, sensory motor skills for ADI. Post immunotherapy improved seizure outcome, memory recall, orientation, ADI however not all anti LGI ALE had complete reversible cognitive outcome. Inter & intra individualistic factors varied among complex Indian rural and urban patients as per their physical, social, mental, environmental dietary habits and reserve. Conclusion: Timely detection, right referral, diagnosis, imaging and neurocognitive work up as well as immunotherapy and neurorehabilitation can improve outcome in anti LGI limbic encephalitis. Anti LGI1 ALE remains one of the intricate rapidly progressive dementia and interdisciplinary team management can help patient, caregivers and overall outcome in Indian neurology practice.

Award ID: 855

Multiple Cranial Nerve Palsy with Embolic Infarcts in a Patient with Rhino-Cerebral Mucormycosis

Subhadeep Basu, Suprotim Ghosh, Krishnendu Choudhury, Shibendu Ghosh

General Medicine, Jagannath Gupta Institute of Medical Sciences and Hospital, Kolkata, West Bengal, India

E-mail: subhadeep_basu_2007@yahoo.co.in

Background and Aim: Mucormycosis is an opportunistic fungal infection occurring in immunocompromised persons. Rhino-orbital Mucormycosis can damage cranial nerves and invade cerebral vasculature. We present this patient with diabetes with fungal sepsis in oral and nasal cavity complicated with polycranial neuropathy and embolic infarcts from vascular invasion. Methodology: 1. MRI & MRA to establish evidence of stroke.2. Tissue biopsy from fungating ulcer of hard palate and PNS to establish Mucormycosis. Result: A 35 year old obese woman presented with fever, diarrhea and headache with sudden visual loss and slurred speech. She developed weakness in her right upper and lower limbs. She also had painful deglutition with sores in mouth. She was a known diabetic on irregular medication. Signs :Patient was febrile, conscious, oriented, dysarthric, absent meningeal signs. Neurologically : Cranial nerves: Left II, III, IV, VI, VII palsy. Sensory loss along left maxillary (V2) Right hemiparesis, with plantar extensor. Ulcers in oral, nasal cavity. Investigations :Blood : Polymorph leukocytosis, hyperglycemic, hyperlipidemia. MRI [Figure 2]: infarcts in left parietal, temporal, frontal region; MRA [Figure 3]: occlusion in left ICA with poor visualised left MCA. Echocardiogram normal. CT- PNS [Figure 4 A&B]: pansinusitis, mucosal thickening. Initial microscopy [Figure 4F] hyphae with branching. Tissue biopsy [Figure 4E] later confirmed mucormycosis. Provisionally diagnosed with stroke and sepsis, treatment started with antibiotics, statin; antiplatelets added as MRI showed non-hemorrhagic infarcts. Mucormycosis was treated with Liposomal Amphotericin B, renal function monitored. Anticoagulant LMWH was added and antibiotics upgraded to contain cavernous sinus infection and thrombosis. DISCUSSION :Sudden visual loss, and ptosis of left eye and absent extraocular movement and pupillary dilatation, infraorbital sensory loss consistent with polycranial neuropathy. The corrosive lesions in the palate, mouth, nose and paranasal sinus proven to be Mucormycosis on tissue biopsy, which eventually invaded the left internal carotid artery causing embolic infarcts in three different cerebral lobes. Conclusion: Rapid progression and vascular invasion in mucormycosis can lead to embolic infarction of the brain, and polycranial neuropathy, particularly in immunocompromised persons with uncontrolled diabetes. Early diagnosis and treatment is essential for patient survival.

Award ID: 856

Normal Pressure Hydrocephalus in an Elderly Patient with Chronic Cerebral Small Vessel Disease

Suprotim Ghosh, Subhadeep Basu, Krishnendu Choudhury, Shibendu Ghosh

General Medicine, Jagannath Gupta Institute of Medical Sciences and Hospital, Kolkata, West Bengal, India

E-mail: suprotim_ghosh@rediffmail.com

Background and Aim: Normal pressure hydrocephalus (NPH) is a syndrome of gait ataxia, dementia and urinary incontinence with dilatation of the ventricles with normal cerebrospinal fluid (CSF) pressure. We describe here an elderly patient presenting with features of NPH as well as chronic cerebral small vessel disease (CSVD). Methodology: 1. CLINICAL ASSESSMENT2. MRI BRAIN3. THERAPEUTIC CSF DRAINAGE BY LUMBAR PUNCTURE. Result: An 88 year old man, known hypertensive for 20 years, on antihypertensive, presented with gradually progressive dysarthria and gait disturbances over last 3 months. He was unable to speak fluently due to impaired articulation which made him depressed. He also had inability to walk steadily with tendency to fall, so that currently he requires a cane while walking. He had no dysphagia, hoarseness or dyspnoea. No impairment in memory, executive functions. On Examination :He was conscious, oriented, no postural hypotension, depressed. Memories intact. Articulation was severely impaired - suggestive of spastic dysarthria particularly with letters “b, d, m, k”. Occasionally his speech was loud, staccato, explosive in nature. Cranial nerves: Jaw-jerk exaggerated. Tongue movements normal with no fasciculations. Motor : power 4+/5 in all four limbs with hypertonia, DTR exaggerated. Gait was slow to start, hesitant, broad based, having tendency to sway and near normal swing but turning en bloc, able to walk with a cane in his right hand. MRI brain revealed multiple sub-cortical and bilateral pontine lacunar infarcts with diffuse ischemic changes with features of NPH. Normal NCV, EMG study excluded MND. He was given antiplatelets, physiotherapy and speech therapy. Therapeutic CSF drainage via lumber puncture done. His gait improved, was able to walk without swaying or help of cane. With continuous speech therapy, his dysarthria showed mild improvement. Discussion :The gait was ataxic, hesitant, broad based frontal type seen in NPH, Dementia and urinary incontinence were absent in this patient, but the MRI features were consistent with NPH. The presence of multiple sub-cortical and pontine lacunar infarcts with spastic dysarthria was consistent with corticobulbar involvement due to the patient’s diffuse cerebrovascular disease. Conclusion: Cerebrovascular disease (CVD) is a common comorbidity in NPH, and prevelent cerebrovascular insults may contribute to the development of NPH, making it an important risk-factor consideration in diagnosis and relevant prognostic factor.

Award ID: 870

Osmotic Demyelination Syndrome with Neuropsychiatric and Extrapyramidal Manifestations - A Case Report

shivangi MS, Srikant Kumar Sahoo, Srikanta Kumar Sahoo, Ajit Prasad Mishra

Neurology, IMS and Sum Hospital, Bhubhaneshwar, Odisha, India

E-mail: drshivangisingh13@gmail.com

Background and Aim: Osmotic demyelination syndrome mainly occurs in the setting of chronic illness. It occurs exclusively in patients admitted to the hospital and was not described before the advent of intravenous fluids, supporting an iatrogenic etiology. Rapid intravenous correction of hyponatremia is the main cause, and in alcoholic and malnourished patients, hyponatremia need not be severe and the correction need not be rapid to give rise to ODS. I, hereby here, present a case of a 22-year old male who presented to us with behavioural abnormalities and extrapyramidal symptoms in form of oromandibular dystonia, bradykinesia, rigidity and gait disturbances for 1 month. He has history of alcohol binge drinking. Methodology: This case report follows the whole case from clinical evaluation, investigations, diagnostic dilemma to outcome of the patient after successful pulse steroid therapy. Result: The patient showed good response with resolution of extrapyramidal symptoms to intravenous pulse steroids. Neuropsychiatric manifestations subsided markedly but with persistence of episodes of inappropriate laughter on follow up after 2 months. Conclusion: Osmotic demyelination syndrome to be suspected early in cases of Alcoholic patients and to consider intravenous pulse steroids as a beneficial treatment modality.

Award ID: 871

Distal Diamond on Quadriceps - A Novel Sign in a Patient with Calpainopathy

Rindha Venepally, Reshma Sultana Shaik, Sandhya Manorenj, Abdul Qavi, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Abdul Qavi, Ajai Kumar Singh, Pradeep Kumar Maurya, Dinkar Kulshreshtha, Cornelius Justin, Kiran K, M. R. Manivannan, Birla Pavalam

Neurology, Deccan College of Medical Sciences, Hyderabad, Telangana, India

E-mail: rindha7.rao@gmail.com

Background and Aim: Diamond on quadriceps is a documented sign seen in patients with dysferlinopathy. Here, we report a case of distal diamond sign on the medial aspect in a patient with calpainopathy, which has not been reported before AIM : CASE REPORT 25-year-old lady presented with chronic progressive muscle weakness, proximal more than distal, lower limb onset since 4 years. Patient was born to second degree consanguineous parents. On examination, patient had bilateral proximal more than distal muscle weakness. Scapular winging was seen. When asked to stand with bent knee, there was a bulge seen on the medial aspect of the distal quadriceps Methodology: Routine labs: normal;Sr CPK: 884; ENMG: suggestive of primary muscle disease; MRI of muscle: Bilateral distal medial asymmetrical bulge over the vastus medialis muscle was noted; Whole exome test: A homozygous, autosomal recessive mutation was detected in the exon 5 of CAPN3 Result: Patients with calpainopathy exhibit disproportionate weakness of the hip extensor and adductors, knee flexors; scapular winging is also common but asymmetrical bulge of the muscles was not reported before. Diamond on qudriceps sign given by Dr sunil Pradhan, is seen in patients with dysferlinopathy, as asymmetrical diamond shaped bulges in the antero lateral aspects of the thighs involving the vastus lateralis muscle, with wasting of the muscle above and below. This sign indicates selectivity of dystrophic process not only among different muscles but also within a muscle Conclusion: Diamond on qudriceps sign seen in patients with dysferlinopathy over the vastus lateralis muscle. We report the first case of distal diamond sign over the vastus medialis muscle in a patient with calpainopathy. Hence all patients with muscular dystrophies should be examined to look for any asymmetricity in the muscle pattern as it may give clue to the diagnosis. Clinical phenotypes of the muscle diseases can help in specific genetic testing

Award ID: 872

Case Report on CNS Involvement in Acute Myeloid Leukemia -A Rare Complication

Surekha Dabla, Neeraj Kumar, kiran bala

Neurology, PGIMS Rohtak, Haryana, India

E-mail: surekhadabla@yahoo.co.in

Background and Aim: - Infiltration of CNS in patients with AML is a rare in comparison to ALL. Here we present a A 36 yrs old female diagnosed with CML and blast crisis one year back, Received various chemotherapeutic agents and Allogenic bone marrow transplant. Three months back she started on intrathecal Methotrexate and 2 months later she suddenly deteriorate and brain imaging showed diffuse infiltration in brain which is not a common occurrence. Methodology: None Result: None Conclusion: None

Award ID: 873

Myopathy as an Initial Manifestation of Secondary Hyperparathyroidism

Rupesh Kumar M, Vinoth Kannan, Manickavasagam J, Shunmugasundaram K, Lakshminarasimhan R

Neurology, Madras Medical College, Chennai, Tamil Nadu, India

E-mail: gowri13priya@gmail.com

Background and Aim: 17 year old female with no known comorbidities presented with insidious onset gradually progressive symmetrical weakness of both lower limbs for 6 months duration. She also presented with truncal weakness. No history of upper limbs weakness, facial muscle weakness, diurnal variation, fatiguability, ptosis, dysarthria, dysphagia, twitching or involuntary movements Methodology: O/E there were no neuro cutaneous markers, no skeletal abnormalities, no facial dysmorphism. Higher mental functions, cranial nerve examination, anthropometry was normal. There was a symmetrical weakness of both lower limbs (proximal > distal), weak neck & truncal muscles, increased tone in both lower limbs with brisk DTR in all 4 limbs. Bilateral plantar flexor response and Gower’s sign was positive with waddling gait. There was no Sensory, cerebellar, and autonomic nervous system involvement. Result: INVESTIGATIONS : LFT showed increased ALP – 1757 IU/L. Phosphorus level was decreased - 1.9 mg/dl. Calcium level was normal- 8.7 mg/dl. NCS of all four limbs was normal. EMG showed myopathic pattern. MRI C- spine with whole spine screening and MRI brain was normal. Skeletal survey showed increased end plate convexity- fish mouth vertebra and diffuse osteopenia with prominent bony trabeculations with focal areas of radiolucency. DEXA scan showed osteoporosis. Parathyroid hormone level was raised - 376.8 pg/ml. Vitamin D was low- 2.5 ng/ml Conclusion: She was diagnosed as osteomalacia due to vitamin D deficiency with secondary hyperparathyroidism and proximal myopathy and was started on Calcium – 1.5 gm /day and Vitamin D3 – 50,000 IU weekly. Patient showed significant improvement after treatment.

Award ID: 874

Binasal Visual Field Defects In Intracranial Hypertension

Megha Varshney, Vikas Lakhanpal, Aastha Takkar, Ramandeep Singh, Vivek Lal

Neurology, Post Graduate Institute of Medical Education and Research

E-mail: meghavarshney87@gmail.com

Background and Aim: Raised intracranial hypertension primary or secondary presents with severe visual disturbances. Binasal visual field defects are rarely seen in patients with raised intracranial pressure. Methodology: A 28-year-old morbidly obese female (BMI-32.8 kg/m2) with a history of vascular headache since 8 yrs, presented with rapidly progressive acute onset bilateral visual loss over 7 days duration along with recent worsening of headache for 5 months, TVOs and pulsatile tinnitus. On examination, she couldn’t perceive light from either eye and had grade IV papilloedema. Patient was diagnosed as a case of chronic CVT with raised ICP based upon imaging findings and CSF opening pressure of 40 mm of H2O. Improvement in visual acuity occurred over 1 month after therapeutic CSF drainage and on CSF lowering agents. On follow up ophthalmic examination her vision improved initially to BCVA of 6/36 in both eyes but later She noticed worsening of vision despite on treatment. Her repeat BCVA was 6/18 in both eyes associated with binasal field loss. CSF diversion procedure was planned but an intraoperatively spontaneous CSF leak was detected, and CSF leak repair was done. Following surgery, significant recovery occurred with incomplete resolution of visual field loss. Result: Occurrence of binasal visual field defects is a rare manifestation reported in raised ICP1. The possible mechanism being compression of non-crossing fibres of optic nerves against the supraclinoid portion of ICA in raised ICP causing binasal field defects however literature still lack the definite causal relationship 2. Conclusion: This unusual field defects increase our understanding of various possible types visual field loss in patient with raised ICP and importance of monitoring visual field defects

Award ID: 875

Dengue and the Brain - A Case Series of Uncommon Neurological Sequelae of Common Disease

Sathya Tejaswini Durgam, Jude Vijay Marian, Shivaji M, Jawahar M, Lakshmi Narasimhan R

Neurology, Madras Medical College and RGGGH

E-mail: teju.durgam@gmail.com

Background and Aim: Neurological manifestations of Dengue fever account for 0.5-7.4%, with most common associated serotypes being DENV 2, 3. These include encephalitis, encephalomyelitis, myelitis, Guillain Barre syndrome, myositis, neuritis, stroke etc., However there are very few case reports regarding post dengue parkinsonism, cerebellitis. With this background, we report case series of 3 individuals with rare neurological manifestations following Dengue fever. Methodology: We report 3 cases of varied neurological manifestations after Dengue fever. All the 3 individuals had typical symptoms of Dengue fever and were seropositive for Dengue. Of the 3 individuals, one was a 16-year-old girl, with nil comorbidities, developed ataxia, scanning speech, incoordination of all 4 limbs on day 7 of dengue fever. she was thoroughly evaluated for other causes, diagnosed as post dengue cerebellitis. Other 2 individuals were middle aged, with nil known comorbidities, developed slowness of activities, slowness of speech, rigidity, bradykinesia, few days following dengue fever. They were diagnosed as post dengue Parkinsonism, after ruling out other aetiologies. Result: 1 st patient was given pulse Methyl prednisolone therapy and other 2 were given supportive therapy. All 3 individuals recovered in varied timelines. Through our case series, though rare, we emphasise on keeping Dengue as one of the aetiologies in cases of parkinsonism, cerebellitis following febrile illness. Conclusion: Steroids can be tried in selected cases. Long term use of L Dopa can be avoided in such cases.

Award ID: 876

An Interesting Case of Early Lance Adams Syndrome-A Prompt Diagnosis Enabled Rapid Functional Recovery

Rashma Mohammed, Fazal Ghafoor, Firosh Khan, Shafeeq Usman, Arun Pranav, Ahamed Subir H, Mohammed Rafeeque

Neurology, MES Medical College, Perinthalmanna, Kerala, India

E-mail: rashmamohammed@gmail.com

Background and Aim: Early Lance-Adams syndrome (LAS) is a rare entity that appear after a period of cerebral hypoxia, typically a respiratory arrest characterised by debilitating action myoclonus taking chronic course. Differentiating status myoclonus from early LAS, the latter being characterised by generalised action myoclonus accompanied by dysmetria, dysarthria and ataxia, with relative preservation of cognition and electroencephalographic (EEG) differences helps in prompt treatment and rapid functional recovery. To date, less than 150 cases have been reported. Methodology: MATERIALS & METHODS An interesting Lance Adams Syndrome case description, investigations, pretreatment, post- treatment recovery videos. Result: A 50-year-old was referred with multifocal myoclonus after an episode of cardiopulmonary resuscitation following cardiac arrest in background of asthma exacerbation sustained a month back. Initially treated with antiepileptics for post hypoxic encephalopathy with status myoclonus, he became bedridden due to severe progressive myoclonus and ataxia with dysarthria and dysphagia. O/E, severe action induced multifocal myoclonus all over the body and unable to sit or stand. He had intention tremors with overlapping myoclonus and left-sided nystagmus. A possibility of LAS was considered based on history and action myoclonus with dysmetria, dysarthria and ataxia, with relative preservation of cognition. His brain CT, MRI, EEG, were within normal limits suggesting a LAS. (Video of patient before treatment and with recovery available) COVID IgG showed raised titres, CSF viral panel was negative. He was started and up titrated on levetiracetam, Piracetam, valproate and clonazepam. His mRS score improved from 5 to 0 within 2 weeks. Conclusion: LAS is rarely diagnosed as the myoclonus with the ataxic component is misjudged to be a part of post-hypoxic encephalopathy or status myoclonus. Dysfunction in neural circuits rather than structural pathology is documented, and hence investigations are normal. Hence high suspicion with prompt diagnosis and differentiation of LAS from other disorders will help in the early treatment and rapid recovery of the debilitating symptoms as highlighted in our case.

Award ID: 877

A Rare Case of Stroke Causing Isolated Webino

Swathi T, Swathi T, Mariam Jude Vijay, Jawahar M

Neurology, MMC

E-mail: swathi1089@gmail.com

Background and Aim: The WEBINO syndrome (wall-eyed bilateral internuclear ophthalmoplegia) - characterized by alterations of extrinsic ocular motility - bilateral deficit in adduction, horizontal nystagmus to abduction, and presence of exotropia in primary gaze. The most common etiology being multiple sclerosis. However, we report a case of WEBINO caused by stroke The physiopathology of this syndrome involves compromise of both longitudinal medial fascicles, superior mesencephalic tegmentum and possible compromise of subnuclei of oculomotor nerves the coordination of which is crucial for adequate execution of horizontal conjugate gaze. Methodology: Case report 63 y male DM came with complaints sudden acute onset of reeling sensation of self and surroundings with horizontal diplopia for both near and distant vision with occasional hazy vision with swaying to left side with out motor sensory, other cranial nerve disturbances with out headache, fever trauma, non progressive improving status On examination : impaired bilateral adduction with abducting eye nystagmus with exotropia on primary position impaired horizontal saccades, VOR, OKN, convergence impaired tandem walking swaying to left side INVESTIGATION MRI Brain – Diffusion Restriction in paramedical ponto-mesencephalic junction Demyelination work up - Negative Discussion May complain of diplopia due to limitation of adduction or skew Paramedian artery on one side on reaching the region of the MLF on its side divides into two almost equal size branches, one of which cross the midline to run to the region of the MLF on the contralateral side and the other branch staying on its own side and running to the ipsilateral MLF Cause - Demyelination, InInfection, AV malformations, CV junction, Wernickes, drugs Multiple treatment options from alternative closure of both eyes with secondary prevention with oral antiPlatelets, antihypertensive, rehabilitation with aggressive treatment like surgery, and botox injection are available Result: Conclusion: WEBINO syndrome is a rare independent occurrence, being aware of it existence facilities the determination of the anatomical location which helps to indicate supplementary examinations to determine its etiology. Here in our case it was in the form of - STROKE

Award ID: 878

Electrophysiology and Magnetic Resonance Neurography Findings of Non-Traumatic Common Peroneal Neuropathy

Divya Madathiparambil Radhakrishnan, Achal K. Srivastava, Kishan Raj, Parthiban Bala, Ajay Garg, Ayush Agarwal, Awadh K. Pandit, Animesh Das

Neurology, AIIMS New Delhi, India

E-mail: dr.divyamr@gmail.com

Background and Aim: Common peroneal nerve (CPN) is frequently involved in lower limb neuropathies across the age groups1. Exploring non-traumatic common peroneal mononeuropathies beyond electrophysiological studies helps to identify etiology and plan management2, 3. Aim: To describe the magnetic resonance neurography (MRN) and electrophysiological findings in patients with nontraumatic common peroneal mononeuropathy Methodology: Consecutive patients presenting with features suggestive of nontraumatic common peroneal mononeuropathy were recruited. Hansen’s disease was ruled out in all patients and investigations were done to rule out specific causes of mononeuropathy (e.g. vasculitis). Nerve conduction studies and electromyography were done in all subjects. Patients with electrophysiological localization along the CPN underwent MRN. Result: Electrophysiological evaluation was done in all 34 patients (27 males) with a clinical diagnosis of non- traumatic common peroneal mononeuropathy. Median age at presentation and time from onset to electrodiagnostic tests were 37.28 years (range 9-70 years) and 14.4 months (range: 1-18 months) respectively. Electrophysiology could localize lesion along the CPN in 32 patients (94.12%); two patients had isolated involvement of deep peroneal nerve. MRN was done in 20 (62.5%) patients. MRN could confirm the involvement of CPN and identify a lesion in 19/20 patients. Hyperintensity of CPN in T2W & FLAIR images, suggestive of peroneal neuritis, was the commonest MRN finding, followed by nerve sheath tumour (n = 4). Compression of CPN by ganglion cyst, osteochondroma and neurofibroma were found in 3,2,1 patents, respectively. In 19 (95%) patients, electrophysiology and MRN agreed to CPN localization. Conclusion: Our study identified various aetiologies of common peroneal mononeuropathy; peroneal neuritis was the commonest cause identified followed by nerve sheath tumour. MRN can be a complimentary to electrodiagnostic tests in the evaluation and management of patients with mononeuropathies2.

Award ID: 879

Dystonia, Catatonia and Parkinsonism A Rare Triad

Vinoth V, Lakshmi Narasimhan, Shanmugasundaram K, Lakshman S

Neurology, MMC

E-mail: drvka86@gmail.com

Background and Aim: Anti-NMDA receptor encephalitis is a form of autoimmune encephalitis occurring primarily in women and is associated with antibodies against NR1 or NR2 subunits of NMDA receptor. Methodology: 19 year old female presented with subacute onset (2 months), gradually progressive symptoms of hypersomnia, behavioural changes in the form of aggression, irritability, inappropriate dressing, lack of insight and apathy and stiffness of both upper and lower limbs, resting tremors involving left hand, lower lip and jaw and abnormal posturing of the left upper limb and postural instability. Result: Neurological examination revealed an alert and oriented patient, with impaired immediate and recent memory (MMSE score was 17/30), speech with reduced fluency and normal comprehension, impaired lobar function tests for frontal, parietal and temporal lobes Conclusion: Motor system examination revealed lead pipe rigidity in both elbows and lower limbs and cogwheel rigidity in both wrists. Power of all muscle groups in both upper and lower limbs were normal. Deep tendon reflexes were 2+ in all 4 limbs and plantar reflex was bilateral flexor. She had abnormal forward bending of neck while standing and sitting which disappears on sleep. She also had abnormal posturing of left upper limb, in which her elbow, wrist and fingers remain flexed. Waxy flexibility was elicited and on testing gait, she had short stepped gait with the tendency to turn en bloc. Her arm swing was also reduced. Serum cerulopasmin was normal. MRI brain was normal. USG abdomen did not reveal teratoma. Video EEG was normal. CSF examination revealed normal cell count, protein, sugar. CSF examination was also negative for HSV and JE infection. Autoimmune encephalitis profile done in CSF revealed positive NMDA antibodies.

Award ID: 880

Safety Profile of Mycophenolate Mofetil in Autoimmune Neurological Disorders at a Tertiary Healthcare Center in Kerala

Anjali Yadav, Sudheeran Kannoth, Deepali Arora, Rajender Kandikonda, Vivek Numbiar, Sibi Gopinath, Anand Kumar, Gopikrishan Unnikrishnan

Department of Neurology, Amrita Insistitue of Medical Sciences and Research Center

E-mail: anjaliiyadav05@gmail.com

Background and Aim: Despite increased usage of Mycophenolate mofetil (MMF) in autoimmune neurological diseases, a paucity of literature exists regarding its side effect profile (1)(2). Hence, we aim to estimate the frequency of adverse drug reactions of Mycophenolate mofetil in patients diagnosed with Neuroimmune disorders. Methodology: Retrospective analysis of electronic medical records from January 2010 – May 2022. Result: Mycophenolate toxicity was analyzed in 398 (188 females; mean age 49.43 ± 16.15 years, mean duration of therapy - 15.94 ± 18.56 months). Patients were given a standard dosage of 500 mg twice daily, which was increased to a maintenance dosage of 1 to 1.5 g daily. One or more adverse effects (AE) were noted in 195 patients {48.99%}. The most frequent AEs were: elevated liver enzymes {49 patients, 12.31%}, infections {41 patients, 10.30% [ urinary tract infections (36 patients, 9.04%), sepsis (2 patients, 0.50%), candidiasis (1 patients, 0.25%), necrotizing fasciitis (1 patient, 0.25%) and Cytomegalovirus (CMV) and Herpes zoster infection (1 patient, 0.25%)]}, gastrointestinal discomfort {40 patients, 10.05%}, cough {23 patients, 5.77%}, leukopenia {4 patients, 1%}, and febrile neutropenia {1 patient, 0.25%}. Conclusion: In our study, 48.99% had one or more AEs -mostly mild. Clinically significant AEs requiring prolonged hospital admission were reported in 4 (1%), without any mortality.

Award ID: 881

Neglected Limb Weakness- An Unusual Presentation of Infective Endocarditis

Sukriti Arora, V. H. Ganaraja, Menaka Vishwanathan, Suresha Kodapala, Jayashankar C A

General Medicine, Vydehi Institute of Medical Sciences, Bengaluru, Karnataka, India

E-mail: sukritiarora9@gmail.com

Background and Aim: Bacterial endocarditis (IE) is a complex disease, associated with significant morbidity and mortality, despite improved diagnostic tools and expanded therapeutic options. However, limb weakness as initial presentation of IE is unusual. This report aims to exhibit an unusual manifestation of infective endocarditis. Methodology: A 42-year male, known diabetic, presented with the history of difficulty in gripping objects with the left hand since one month, which was sudden in onset and non-progressive in nature. At the same time, he was also noticed to have multiple episodes of loss of consciousness, each, lasting for 1-2 minutes. He was not evaluated for his current symptoms. Examination revealed left handgrip weakness, rest being unremarkable. On cardiac auscultation, pansystolic murmur was noted in pulmonary area. During the time of evaluation, patient had developed sudden onset breathlessness with heart rate of 180/min and respiratory rate of 34 cycles/min, which required intensive care. Result: Brain MRI showed multiple nodular cystic lesions in the right temporal, parietal and frontal vertex suggestive of brain abscess. CT abdomen showed splenomegaly with multiple hypodense lesions suggesting splenic infarcts. Three consecutive blood cultures showed methicillin resistant staphylococcus aureus. Echocardiography showed vegetation on anterior mitral leaflet with eccentric mitral regurgitation. A diagnosis of infective endocarditis was made and showed good response with injectable antibiotics. Conclusion: It is important to suspect IE in patients presenting with symptoms of septic emboli. The timely diagnosis and treatment of patients will possibly help with combatting the further worsening or development of complications including death.

Award ID: 882

The Effectiveness of Anti-CGRP Monoclonal Antibodies on Hemiplegic Migraine : A Case Series in a Tertiary Headache Center in Japan

Daisuke Danno, Kumiko Ishizaki, Shoji Kikui, Takao Takeshima

Department of Neurology, Headache Center, Tominaga Hospital, Osaka, Japan

E-mail: daisuke_danno@yahoo.co.jp

Background and Aim: Monoclonal antibodies (mAbs) targeting the calcitonin gene-related peptide (CGRP) are novel migraine preventive treatments showing promising efficacies in normal migraine cases; however, there are no reports regarding the efficacy in hemiplegic migraine. Methodology: We reviewed the medical records of consecutive hemiplegic migraine cases treated with CGRP mAbs in our tertiary-care headache center between May 2021 and April 2022. Result: We had six hemiplegic migraine cases treated with galcanezumab, and the average duration of the follow-up was 5.5 months, and the number of days each month with headache of at least moderate severity was reduced in 4 cases. The number of days each month with motor weakness was also reduced in five cases. Further, the Patients’ Global Impression of Change and change in migraine disability assessment score were improved in five cases. On the other hand, the number of days with motor weaknesses was increased in one case. No adverse events directly related with the treatment were reported. Conclusion: Regarding the mechanism underlying the improvement in aura symptoms, we speculate that a small amount of CGRP mAbs directly acts in the central nervous system. Another explanation is that blocking of the CGRP pathway in the periphery might secondarily inhibit cortical spreading depression (CSD). We need to be prudent, but galcanezumab was still generally effective for headache and motor aura in hemiplegic migraine cases and well tolerated. Further prospective studies will elucidate the effects of CGRP mAbs in hemiplegic migraine.

Award ID: 883

Clinical Features of Malignant Lymphoma Accompanied by Central Nervous System Involvement

Keiichi Mizushima, Shinichi Shirai, Ikuko Iwata, Masaaki Matsushima, Hiroaki Yaguchi, Ai Shimizu, Ichiro Yabe

Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University.

E-mail: kmizu@pop.med.hokudai.ac.jp

Background and Aim: Malignant lymphoma accompanied by central nervous system (CNS) involvement is diagnostically challenging because definitive diagnosis necessitates CNS biopsy, which is highly invasive. In this study, we investigated the clinical characteristics of malignant lymphoma accompanied by CNS involvement. Methodology: We retrospectively investigated 9 patients (6 men, 3 women, mean age 57 years) admitted to our department between August 2010 and October 2021, with CNS symptoms and histopathologically confirmed diagnosis of malignant lymphoma. Result: The mean soluble interleukin (IL)-2 receptor (sIL-2R) levels in the serum and cerebrospinal fluid (CSF) were 1,860.9 U/mL and 160.2 U/mL, respectively. Magnetic resonance imaging (MRI) revealed CNS lesions in the cerebral white matter in 6, in the spinal cord in 2, and in the cavernous sinus in 1 patient; all 6 patients who underwent contrast-enhanced imaging showed lesion enhancement. Notably, 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) revealed enhanced accumulation in CNS lesions in 6 of 9 patients. Histopathological evaluation revealed diffuse large B-cell lymphoma in 8 and follicular lymphoma in 1 patient. Conclusion: This study showed elevated sIL-2R levels in the CSF and lesion enhancement in all 6 patients who underwent contrast-enhanced MRI. Although 18F-FDG PET showed increased tracer accumulation in the lesions in most patients, it is suggested that malignant lymphoma cannot be ruled out even in the absence of increased tracer uptake. Further accumulation of cases and detailed descriptions of clinical features are warranted to enable accurate diagnosis of malignant lymphoma accompanied by CNS involvement.

Award ID: 884

Concern - A Prospective Study of Burden Among Care Giver of Patients Who Underwent Decompressive Hemicraniectomy Following Malignant Middle Cerebral Artery Infarction

Vibhor Upadhyay, M. V. Padma Srivastava, Rohit Bhatia, Ayush Agrawal

Neurology, AIIMS, New Delhi, India

E-mail: docvibhor@gmail.com

Background and Aim: Background – 10-15% patients with middle cerebral artery (MCA) territory cerebral infarction requires decompressive hemicraniectomy (DH) as a life saving measure causing moderate to severe disability leading to high levels of caregiver burden (CGB). Aim - To study CGB, employment status and level of change of social functioning of principal caregiver of stroke survivor of malignant MCA infarction underwent DH and correlate it with severity of stroke, functional disability, and depression, anxiety and stress score. Methodology: An observational prospective study was carried out where 47 patients and their caregiver with malignant MCA infarction post DH were recruited and assessed after 3 months for mRS and Barthel index. Employment status and social function of the caregiver was analysed using a 6-item social function scale developed for the study. 25-point scale for Burden assessment named CBS-IP was used to assess the CGB along with DASS21 scale to assess depression, anxiety, and stress among caregivers. Result: Spouse was the principal caregiver for patient f/b kin. Employment loss had a statistically significant association with CGB. There was an overall decline in social functions of caregivers. Of the CGB, economical domain was worst affected f/b physical domain and least affected was social domain. The stroke severity and functional disability of the stroke survivors was having statistically significant positive correlation with overall CGB. Mental stress was predominantly seen in caregiver and having positive significant correlation with CGB. Conclusion: CGB significantly associated with stroke severity and functional disability. Employment loss of stroke survivors and caregiver alongwith mental stress was associated with high CGB.

Award ID: 885

Diagnostic Value of Munix and Nerve Ultrasound in Charcot-Marie-Tooth Patients

Elham Dirandeh, Farzad Fatehi, Shahriar Nafissi

Neurology Department, Tehran University of Medical Sciences, Dr. Shariati Hospital, Isfahan, Iran

E-mail: dirandehelham@gmail.com

Background and Aim: Charcot-Marie-Tooth disease (CMT) is the most common inherited sensory-motor neuropathy diagnosed by electrodiagnostic and genetic studies. Motor unit number index (MUNIX) and nerve ultrasound (NUS) are noninvasive new noteworthy techniques. The objective was to delineate the application of these tests in CMT and their association with clinical scales. Methodology: Thirty-three CMT patients and 20 healthy subjects entered this cross-sectional study. We assessed clinical outcome measurements as well as MUNIX (in abductor pollicis brevis (APB), abductor digiti minimi (ADM) and tibialis anterior (TA)) and NUS on median, ulnar, tibial, peroneal and sural nerves and cervical nerve roots in both patients and control groups. Result: Mean age of the patients was 30.3 ± 10.3 years. The most common subtype was CMT1A (38.1%). Electrodiagnostic study revealed demyelinating change in 72.7%. MUNIX of all examined muscles was significantly lower than that of controls. According to NUS, median and ulnar nerves at mid-arm, tibial and peroneal nerves at popliteal fossa, sural nerve at ankle and C6 and C7 roots had enlarged cross-sectional area (CSA) which was significantly higher than controls. There was a significant correlation between MUNIX sum score and clinical outcome measurements and also between APB MUNIX and median nerve CSA at forearm, antecubital fossa, mid-arm / ADM MUNIX and ulnar nerve CSA at mid-arm / TA MUNIX and peroneal nerve CSA at popliteal fossa. Conclusion: Considering significant difference of MUNIX and NUS between patients and controls and significant associations of MUNIX with clinical scales and NUS findings, these tests could be used as additive outcome measurements.

Award ID: 886

Extensive Vasculitis in Tuberculous Meningitis

Rajarshi Chakraborty, Rajesh Verma, Ravindra Kumar Garg

Neurology, King George Medical College, Lucknow, Uttar Pradesh, India

E-mail: satyalung@gmail.com

Background and Aim: Tuberculous meningitis is a common cause of morbidity and mortality in tropical countries. It can complicate with hydrocephalous, vasculitic infarcts, tuberculomas, abscess, optochiasmatic arachnoiditis etc.1. Vasculitis in tuberculosis is basically at the level of lenticulostriate arteries supplying the basal ganglia and terminal cortical branches.2 In this case report, we present a unique case of tuberculous meningitis with extensive vasculopathy. Methodology: She was evaluated thoroughly for chronic meningitis. In view of new-onset stroke post-ATT, we tried to explore the element of vasculitis furthermore in the patient. Result: This 26 year old post puerperal day 13 lady presenting with fever, persistent headache with intermittent vomitting for 1 and half months, blurring of vision for 1 month and history of altered sensorium for 15 days was diagnosed as tuberculous meningitis based on clinicopathological and radiological evidence and she showed initial improvement with antitubercular treatment and steroids. However on day 6, she developed new-onset seizures followed by sudden onset right sided classical hemiplegia with inability to speak which came out to be left Internal carotid artery occlusion. She was extensively worked out for vasculitis which came out to be negative and she was diagnosed as extensive vasculopathy in tuberculous meningitis. Conclusion: The element of very high levels of protein in cerebrospinal fluid might predispose to such an event. The exact pathogenesis of such extensive involvement in tuberculous meningitis substantiates the need for further future studies.

Award ID: 887

Neurobrucellosis: A Forgotten Entity

Rajarshi Chakraborty, Imran Rizvi, Ravindra Kumar Garg

Neurology, King George Medical College, Lucknow, Uttar Pradesh, India

E-mail: satyalung@gmail.com

Background and Aim: Zoonotic infections are important entities oftenly underdiagnosed in developing nations. Neurobrucellosis affects the entire nervous system with a plethora of neurological manifestations starting from encephalitis to peripheral neuropathies.1 Methodology: A 45 year old male presented with progressive tremulousness of limbs for 8 months, followed by progressive hearing loss for 5 months with low grade fever on and off for a year. There was no history of altered sensorium, convulsion, headache, paresthesia, falls, weakness, altered behavior or sensorium, vomiting, change in voice or difficulty in speaking, swallowing, vision or chewing. He was a security-guard but lost his job due to his illness. He had pets in his house in the form of dogs, cow and hen. His general examination was normal. Neurological examination showed a GCS of 15, sensorineural deafness of right ear along with cerebellar signs. Rest of examination was normal. He was thoroughly evaluated. Result: His neuroimaging showed patchy meningeal enhancement with sensorineural pattern of deafness in right ear. He was serologically tested for brucella which turned out positive. He was treated with rifampicin and doxycycline. On follow-up at 3 months, there was substantial improvement in tremulousness with mild improvement in hearing. Conclusion: Neurobrucellosis is a treatable zoonotic infection with guarded prognosis. A strong suspicion of disease can lead to early diagnosis and favourable outcome. Public awareness needs to be considered for early detection and better prevention.

Award ID: 888

Seasonal Variation of Guillain-Barré Syndrome in Iranian Patients: A Retrospective Study

Elham Dirandeh

Neurology Department, Tehran University of Medical Sciences, Dr. Shariati Hospital, Isfahan, Iran

E-mail: dirandehelham@gmail.com

Background and Aim: It seems that the incidence of Guillain-Barré syndrome (GBS) has seasonal trends depending on weather as well as environmental and demographic factors such as upper respiratory tract infection (URI). The aim of this study was to evaluate seasonality of GBS and its electrophysiological subtypes. Methodology: In this cross-sectional study, the records of all admitted patients to all wards of Dr. Shariati Hospital from March 2009 to March 2019 according to ICD-10 codes for GBS and other similar neuropathies were investigated and 87 patients were registered based on fulfillment of Brighton criteria and symptom onset during the recent 4 weeks. Statistical analysis was performed by IBM SPSS version 20. A p-value < 0.05 was considered statistically significant. Result: Most of the patients (63.2%, n = 55) were men. The mean age of them was 49.1 ± 19.2 years, and 41.3% (n = 36) and 10.3% (n = 9) participants had recent URI and gastrointestinal infection, respectively. The frequency of GBS in different seasons was 35.6% (n = 31) patients in the winter, 27.6% (n = 24) in the autumn, 19.6% (n = 17) in the spring, and 17.2% (n = 15) in the summer. The most frequent electrophysiological subtype was acute inflammatory demyelinating polyneuropathy (AIDP) in all seasons. The most common GBS disability score was 1. Conclusion: The highest and the lowest occurrence was seen in the winter and summer, respectively. AIDP was the most common electrophysiological subtype in all seasons. More studies are suggested to evaluate other aspects of GBS on more details.

Award ID: 889

Acute Sensorimotor Polyneuropathy: Think Beyond GBS

Rajarshi Chakraborty, Imran Rizvi, Ravindra Kumar Garg

Neurology, King George Medical College, Lucknow, Uttar Pradesh, India

E-mail: satyalung@gmail.com

Background and Aim: Peripheral neuropathy is an important entity in neurological clinics.1 The presentation of acute-onset areflexic progressive sensorimotor quadriparesis usually point towards Guillain barre syndrome (GBS) or myelitis and is a potential neurological emergency that heralds early and prompt management. In this case, we discuss a 62-year-old man presenting with acute-onset paresthesia and ascending weakness of limbs, treated with IVIG but was evaluated at the same time beyond GBS based on certain findings. Methodology: This 62-year-old, nondiabetic, nonhypertensive, retired teacher presented with weakness of all limbs for 5 days which is acute onset, gradually progressive, symmetric, ascending in nature with distal to proximal progression and involving the upper limbs over 3 days along with tingling sensation of both lower limbs of similar duration. Result: There were presence of multiple nontender circular hyperpigmented patches in both the lower limbs. His neurological examination showed normal higher functions, normal cranial nerves, generalised hypotonia with power of 4/5 in upper limbs and 2/5 in lower limbs with areflexia and flexor plantars, sensory signs were prominent for all modalities in glove and stocking pattern, normal cerebellar function with nonpalpable peripheral nerves; with decreasing single breath count of 15. He was diagnosed as AMSAN type GBS. However, electrophoresis showed MGUS with IgG immunophenotyping in kappa region. Bone marrow study showed atypical plasma cells with hemophagocytosis & skeletal survey was unremarkable. He was treated with intravenous immunoglobulin. Conclusion: GBS is a treatable prototype of immunological neuropathy. However, in presence of some clinical clues, the diagnostic domain should be explored.

Award ID: 890

Manifestations and Novel Mutations of Iranian Patients with CMT4C

Elham Dirandeh

Neurology Department, Tehran University of Medical Sciences, Dr. Shariati Hospital, Isfahan, Iran

E-mail: dirandehelham@gmail.com

Background and Aim: Charcot-Marie-Tooth disease (CMT) is the most common inherited neuropathy diagnosed by electrodiagnostic (EDx) and genetic studies. CMT4C is a demyelinating neuropathy, frequently characterized by spine deformities and cranial nerve involvement, associated with recessive mutations in SH3TC2 gene. Motor unit number index (MUNIX) and nerve ultrasound (NUS) are also new valuable techniques. The main objective of this study was to describe manifestations of CMT4C. Methodology: We reported five CMT4C patients from neuromuscular department of Dr. Shariati hospital. All patients underwent physical and neurological examination, EDx and genetic testing. We also assessed clinical outcome scales as well as MUNIX and NUS on different muscles and nerves, respectively. Result: Four patients were men. The median of current age and age of onset were 38 and 10 years, respectively. Family history of 3 patients was positive. All patients complained about distal limb weakness and paresthesia. The examination of cranial nerves and bulbar muscles was normal. All of them had pes cavus and distal atrophy and 2 patients had kyphoscoliosis. The median of clinical outcome scales were mentioned as follows: 9-HPT: 33.81 sec, 10MWT: 8.52 sec, ONLS: 3.5, CMTNS: 18. MUNIX score was lower than normal limit. According to NUS, median nerve at all sites had enlarged cross-sectional area in 2 patients. Different mutations in SH3TC2 gene were detected in all patients. Conclusion: The phenotype of CMT4C appears to be enriched by a number of features unusual for broad CMT category. A new era of genetic testing has led to recognize individuals with CMT4C and highlights accurate diagnosis.

Award ID: 891

A Gray Matter Volume Network in Alzheimer’s Disease

Koji Fujita, Munkhzaya Chuluunbat, Daiki Matsuda, Maki Otomo, Yoichi Otomi, Kohsuke Kudo, Masafumi Harada, Yuishin Izumi

Department of Neurology, Tokushima University

E-mail: kfujita@tokushima-u.ac.jp

Background and Aim: This study aims to identify and validate a gray matter volume network in patients with Alzheimer’s disease (AD). Methodology: To identify a disease-related network, a principal component analysis-based algorithm, Scaled Subprofile Model, was applied to gray matter volume data derived from structural T1-weighted magnetic resonance imaging of the training sample that consisted of nine patients with AD (women, four; dementia, seven; mild cognitive impairment, two; age, 66.7 ± 8.8 [mean ± SD] years) with positive 18F-flutemetamol amyloid positron emission tomography and eight age-matched healthy controls obtained on-site. The network expression scores were calculated by topographic profile rating in the validation sample obtained via the Open Access Series of Imaging Studies and comprised 12 patients with AD dementia (women, four; age, 70.0 ± 3.7 years) and 12 age-matched healthy controls. Result: A significant network from the training sample, for which subject expression differed between the groups (permutation test, P = 0.006; sensitivity and specificity, 100%; area under the curve, 1), was identified. This network was represented by the principal components 1, 2, and 3 and showed a relative decrease in the inferior parietal lobule including angular gyrus, inferior temporal gyrus, premotor cortex, amygdala, hippocampus, and precuneus. It significantly differed between the groups with a sensitivity, specificity, and area under the curve of 83%, 91%, and 0.85, respectively, in the validation sample (P = 0.003). Conclusion: An AD-related gray matter volume network that captured relevant regions was identified in amyloid positron emission tomography-positive patients and validated in an independent sample.

Award ID: 892

Myasthenic Storm Severely Breaking the Heart, A Case Report

Khaled Kariminejad, Farnaz Sinaei, Bentolhoda Ziaadini

Neurology Department, Shariati Hospital

E-mail: xalidknnkarimi@yahoo.com

Background and Aim: Takotsubo cardiomyopathy is a grave clinical condition triggered by emotional or physical stress. Myasthenic crisis is a rare cause of this condition due to the severe respiratory failure. Methodology: Here we report a case of myasthenia gravis presenting with respiratory failure. Result: A 45 year-old women who was previously diagnosed as a thymomatous myasthenia gravis presented with rapidly progressive dyspnea, nasal speech and ptosis after a 1-hour walk. She was immediately intubated. Her chest CT revealed significant pulmonary edema and cardiac evaluations showed Ejection Fraction of 10%, global Hypokinesia, left Ventricular Ballooning and normal serial cardiac enzymes. On ECG she had diffuse ST depression and T inversion. The patient experienced an episode of atrial tachycardia treated by amiodarone infusion and also an occasion of cardiac arrest returning back after 20 minutes CPR. IVIg and 1 mg/kg prednisolone were administered for the underlying myasthenic crisis and treatments for cardiac failure was initiated. After 48 hours she was extubated and her follow up echocardiography showed marked improvement. Takotsubo cardiomyopathy was considered and she was discharged after 25 days. Further outpatient follow up echocardiogram after 16 days was normal. Conclusion: Acute cardiac failure can rarely be triggered by myasthenic crisis. The clinician must be aware of this condition to avoid administration of inappropriate drugs and unnecessary investigations. Serial ECG and close cardiac monitoring should be done and helpful in early detection of disease. Our patient had improved myocardial function and clinical outcomes.

Award ID: 893

Autism Spectrum Disorder with and Without Epilepsy: A Comparative Study on Clinical, Developmental, Electrophysiological and Radiological Profile

Sayooja Sachithanandan, Ramshekhar N. Menon, Sajitha K.V., Soumya Sundaram

Neurology, Sreechitra Tirunal Institute of Medical Sciences and Technology, Kerala, India

E-mail: sayooja.04@gmail.com

Background and Aim: Epilepsy coexist with autism spectrum disorders suggesting common mechanistic link. However, limited literature exists on autism with epilepsy (ASD-E) phenotypes and this study is aimed to compare and contrast the clinical, electrophysiological and radiological profiles among ASD-E and autism without epilepsy (ASD) Methodology: Retrospective study of autism patients attending a tertiary care Hospital between 2011 -2021. The demographic, clinical, developmental, electroencephalography (EEG) and neuroimaging parameters were collected and were compared between ASD-E and ASD groups using Pearson’s chi square test. Regression analysis was applied to statistically significant variables (p < 0.05), to find out predictors of epilepsy in autism Result: Out of 441 autism patients, epilepsy was present in 191 (43.3%). 319 patients (72%) were males and the mean age of presentation was 88 ± 56 months. Global developmental delay (GDD) (78% vs 22%, p < 0.001), global regression of developmental milestones (34.3% vs 10.3%, p < 0.001), abnormal EEG (83% vs 39%, p < 0.001), background slowing (62% vs 17%, p < 0.001), interictal discharges (IEDs) (81% vs 35%, p < 0.001) and structural abnormality (48% vs 32%, p 0.013) were higher in ASD-E than in ASD group. Female sex (OR 2.744, 95%CI 1.145-6.576, p 0.024), GDD (OR 2.852, 95% CI 1.185-6.862, p 0.019), background slowing (OR 4.141, 95% CI 1.621-10.581, p 0.003) and IEDs (OR 4.282, 95% CI 1.641-11.171, p 0.003) were predictors for ASD-E phenotype Conclusion: Developmental deviance in all domains, EEG abnormalities and structural lesions in neuroimaging are more frequent in ASD-E and these factors could predict occurrence of epilepsy in autism

Award ID: 894

Toll-Like Receptor 3 Genetic Polymorphism in Dengue Encephalitis

Rajarshi Chakraborty, Rajesh Verma, Shantanu Prakash, Atul Pandey, Amita Jain, Ravindra Kunar Garg, Anuj Verma

Neurology, King George Medical College, Lucknow, Uttar Pradesh, India

E-mail: satyalung@gmail.com

Background and Aim: Dengue is an important vector-borne viral disease affecting human with diverse manifestations. Toll-like receptors (TLRs) are pattern recognition receptors which influences innate immunity against microbes.1 TLR3 plays critical role in controlling the innate immune response mediated by flavi-viruses like dengue.2 We aimed to investigate the role of TLR3 polymorphism in cases of dengue encephalitis (DE). Methodology: Out of 248 patients with dengue illness, we recruited 29 patients of DE as cases and equal number of age and sex-matched dengue fever patients without neurological features as controls. All cases and controls were examined for TLR3 polymorphism with the help of polymerase chain reaction and genetic sequencing method. Result: The findings revealed that the genotypic distribution of TLR3 Leu412Phe polymorphism for mutant genotype Phe/Phe (TT) demonstrated increased risk for DE [31.03% vs 6.8%, p value 0.019, Odds Ratio (OR) 6.075, 95% Confidence Interval (CI) 1.181-31.245]. However, the number of heterozygous (H) genotype (Leu/Phe–CT) variance was found to be statistically insignificant (6.8% vs 24.13%%, OR 0.233, 95% CI 0.043-1.279, p value 0.07). The frequency of mutant (M) + heterozygous (H) genotype was compared in cases with controls, but the difference was statistically insignificant. (37.93%vs 31.03%, OR 1.36, 95% CI 0.458-4.027, p value 0.65). The frequency of mutant Phe allele (T) was found to be higher in cases than controls but the difference was statistically insignificant (34.48% vs 18.97%, OR 2.290, 95% CI 0.98-5.30, p-value 0.052). Conclusion: The presence of mutant TLR3 Leu412Phe polymorphism increases the propensity to have dengue encephalitis in patients with dengue infection in Indian population.

Award ID: 895

Sensory Neuronopathy Shouting out Hodgkin Lymphoma and Fading Away by its Treatment, A Case Report

Bardiya Ghaderi Yazdi, Farnaz Sinaei, Bentolhoda Ziaadini

Department of Neurology, Shariati Hospital, Tehran University of Medical Sciences

E-mail: Bardiyaghaderi@gmail.com

Background and Aim: Paraneoplastic neurological syndromes are rare manifestations of Hodgkin and non-Hodgkin lymphomas. Among all, sensory ganglionopathy is still a rare presentation. Methodology: Here we present a patient with an undiagnosed skin lesion since 8 years ago who has been referred due to a 14 months history of gradually worsening ataxia. Result: The patient was a 46-year-old woman with a complaint of paresthesia in distal extremities and ataxia. On neurologic examination, the deep sensation was profoundly impaired and generalized areflexia was evident. In electro-diagnostic evaluation all sensory potentials were absent. In her extensive workup, several lymphadenopathies in the thorax and abdomen were detected in the PET scan. Biopsy of the lymph nodes revealed Hodgkin Lymphoma (HL) and treatment of the underlying lymphoma was immediately started. Surprisingly, the skin lesion and the sensory symptoms responded dramatically to the remedies. Conclusion: Sensory neuronopathies are very rare presentations of HLs. This case may represent the immunologic basis of some neurological syndromes in hematologic malignancies. Appropriate work-up and early detection of the underlying disease prevent long-term disabling sequels in these patients.

Award ID: 896

Resting State Network (RSN) Disturbances in Hypothalamic Hamartoma (HH) using Magnetoencephalography

Viswanathan Lakshminarayanapuram Gopal, Bhargava Gautham, Mariyappa N, Ravindranadh Mundlamuri, Raghavendra Kenchaiah, Ajay Asranna, Karthik Kulanthaivelu, Sanjib Sinha

Department of Neurology, NIMHANS Bangalore, Karnataka, India

E-mail: vishwanath.iyer89@gmail.com

Background and Aim: Cognitive impairment in HH is not uncommon and is believed to be due to widespread network disruptions.(1) The aim of this study is to identify if there are anomalous patterns of resting state networks in patients with hypothalamic hamartoma using MEG Methodology: This is a retrospective study which included 10 patients. All had drug resistant epilepsy M:F – 7:3, (Age at onset – 3.32 ± 3.23 years, Duration – 13.46 ± 10.77 years). Volume of the hamartoma was calculated from 3T MPRAGE sequences. Resting MEG data was acquired using Neuromag Triux MEG system using standard protocols. The filtered data in various frequency bands were projected onto source space using sLORETA using the segmented cortex of the individual patient MRI. Network topology was analyzed using graph theory metrics using clustering coefficient (CC) and small worldness (SW). Result: Decrease in small worldness (σ < 1) was seen in the delta and theta frequency bands. CC was increased in the delta, beta and gamma bands in nodes involved default mode network, attention and primary sensory regions. Significant correlation was observed between CC in beta band and hamartoma volume (r: -0.6987) and SW in the gamma band with disease duration (r:0.6743). Conclusion: Network analysis in HH patients showed deviation from small-world structure theta and delta bands. Network dysfunction was directly related to the volume of hamartoma and scaled with disease duration. HH tends towards a hyperconnected network with a poor hub structure and this configuration might be responsible for the wide spread cognitive decline in HH.

Award ID: 897

Mechanisms of Visual Misperceptions in Parkinson’s Disease

Gajanan Revankar, Yuta Kajiyama, Tomohito Nakano, Noriaki Hattori, Etsuro Mori, Tetsuya Shimokawa, Masahito Mihara, Hideki Mochizuki

Neurology, Osaka University

E-mail: revankar@neurol.med.osaka-u.ac.jp

Background and Aim: Parkinson’s disease (PD) patients susceptible to visual hallucinations encounter perceptual deficits in the form of pareidolias. Pareidolias are visual misperceptions in which ambiguous forms or shapes appear meaningful. Our aim was to reveal brain mechanisms influencing top-down visual processing in PD patients using a novel, multimodal, testing paradigm. Methodology: 21 PD patients and 12 healthy controls were evaluated. Tests for cognition, attention and executive functions were performed. Pareidolias were evaluated using a computerized pareidolia test with synchronized EEG and eye-tracking. PD patients were divided into those with pareidolias (N = 10) and those without (N = 11). Pre-stimulus EEG connectivity were evaluated using graph networks in the spectral domain (theta, low-alpha, and high-alpha frequencies). Result: PD patients with high pareidolias were older and under-performed on neuropsychological tests. Eye-tracking data showed longer visit (saccade-dependent) and fixation durations for pareidolic stimuli ‘during’ active search task. Eye-tracking synched EEG showed higher presaccadic potentials on frontal electrodes. Pre-stimulus EEG low-alpha band networks showed a tendency towards higher frontal activity in PD with pareidolia group. Conclusion: We conclude pareidolias in PD are due to an abnormal top-down modulation of visual processing which are defined by frontal cortex alterations that affects visual attention and guidance to ambiguous stimuli due to a dissonance between patients’ internally generated mental processing with external stimuli. Our results highlight the importance of the use of pareidolia as a screening tool for neurodegenerative diseases, as well as implications for interventions in the future.

Award ID: 898

The Development of a Novel Drug Platform with Safety Using Artificial Cationic Oligopeptides to Regulate Genes in the Central Nervous System by Systemic Injection

Masahiro Ohara, Tetsuya Nagata, Rintaro Iwata Hara, Kazunori Takagi, Kazuki Sato, Yusuke Maeda, Takeshi Wada, Takanori Yokota

Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Tokyo, Japan

E-mail: masa.ohara0204@gmail.com

Background and Aim: We previously reported that cholesterol conjugated heteroduplex oligonucleotides (Chol-HDO) have a very high gene silencing effect compared to single-stranded antisense oligonucleotides (ASO) (1-4), even in the central nervous system (CNS) by crossing blood-brain barrier (BBB) (5). However, CNS gene inhibition requires high-dose systemic administration of Chol-HDO, resulting in some side effects. L-2,4-diaminobutanoic acid oligomers (DabO) were developed as artificial cationic peptides binding to the major groove of HDO (6). We aimed to develop a novel drug platform which reduces these side effects while maintaining high efficacy of our HDO technology. Methodology: To generate HDO, equimolar concentrations of ASOs and complementary RNAs were hybridized by denaturing them at 95 °C for 5 min and then slowly cooled to 37 °C. DabO/Chol-HDO complex was spontaneously formed by their mixing. aPTT and complement activation was analyzed by validated methods. Quantitative real-time PCR analysis was performed to evaluate gene silencing effects. Result: Both in vivo and ex vivo study showed that the DabO/Chol-HDO complex statistically ameliorated aPTT prolongation and complement activation. Furthermore, behavioral abnormality or brain focal necrosis was not observed in DabO/Chol-HDO complex injected mice while hypoactivity and focal necrosis in brain were noted in Chol-HDO injected mice. Intriguingly, pharmacokinetics and pharmacodynamics study of DabO/Chol-HDO complex showed that DabO did not affect gene knockdown effect and BBB-crossing property of cholesterol ligand. Conclusion: Our results showed that DabO reduced the toxicities with maintaining the silencing efficacy of Chol-HDO. DabO/Chol-HDO complex can be a novel platform to overcome the side effects of BBB crossing HDO technology.

Award ID: 899

Rare Case of Familial MuSK Positive Myasthenia Gravis in Father and Son

Naushad Ravjani, Rukmini Mridula

Neurologist, Nizams Institute of Medical Sciences, Hyderabad, Telangana, India

E-mail: naushadravjani@gmail.com

Background and Aim: Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular junction. Hoch et al in 2001, discovered that antibodies to muscle specific tyrosine kinase (MuSK) were responsible for 70% of AchR negative Myasthenia gravis. There are some case reports showing familial occurrence of autoimmune myasthenia gravis. First case of familial MuSK myasthenia gravis was reported in a Sardinian family consisting of two sisters. We report rare familial MuSK positive myasthenia gravis from India in father and son. Methodology: We are presenting case report of father and son who presented with bulbar and ocular symptoms. On evaluation they had AntiMusk antibodies positive.Studies have reported these type of familial AntiMusk MG cases. Result: Routine haematological and biochemical investigations were normal. Atropine-neostigmine test was positive. RNS showed decremental response in trapezius muscle. AchR antibody was negative. Anti MuSK antibody was positive (10.51 mol/LAfter the treatment, his dysphagia, neck and proximal muscle improved. Conclusion: This is a rare case report of familial Musk positive myasthenia gravisin father and son.

Award ID: 900

Common Presentation of Uncommon Disease- Not to be Missed

Atith Neware, Vikram Huded, Anirudh Kulkarni, Gopal Krishna Dash, Kuldeep Shetty, Vivek Jacob Philip, Radhika Manohar, Chintan Prajapati, Dileep R

Neurology, Narayana Hrudayalaya Hospital

E-mail: atithn@gmail.com

Background and Aim: DAVF is rare type of acquired intracranial vascular malformation with pathological shunt in dura matter of the brain. Has various clinical signs and symptoms. location and venous drainage determines clinical presentation and potential for serious sequelae. Common Sites-Transverse/ Sigmoid-71%, Cavernous sinus-10%, Middle fossa-2%, Convexity-4%, Anterior cranial fossa-2%, Tentorial-3%, SSS-3%, Others-7%. Aim to report a case, presented with features of raised ICP, followed by seizure’s, altered sensorium and cognitive decline, found to have uncommon diagnosis, which is treatable. Methodology: Elderly male had history of recurrent transient visual obscurations-1 year, no other complaints, had papilledema, initial MRI Brain with venogram- normal, few month’s later-worsening of visual complaints, CSF LP opening pressure-280mm, no response to acetazolamide, underwent TP shunt in other centre for severe papilledema, Later Had Covid- fever, seizure, altered sensorium for few days. TP Shunt closed. Treated as PRES. later had deterioration in vision, had established papilledema, underwent RE ONFS in other centre. Later had recurrence of seizures on anti-epileptics and Cognitive impairment. Presentation to our institute: Impaired calculation and recall, MMSE 23; BE secondary optic atrophy. Result: MRI Brain venogram contrast study- Chronic CVT along bilateral transverse and sigmoid sinuses with straight sinus, with fistulous communication at left distal transverse and sigmoid sinuses. DSA showed Left transverse-sigmoid sinus DAVF (Cognard type 2a+b). Underwent endovascular and patient showed signs of improvement. Conclusion: DAVF should be suspected in cases of raised Intracranial pressure, and before undergoing any major surgeries, this treatable condition has to be ruled out.

Award ID: 901

Does Optic Nerve Sheath Diameter as Determined by Transorbital Sonography and MRI Correlate with CSF Opening Pressure in Patients with Idiopathic Intracranial Hypertension?

Vineet Todi, Ashish Kumar Duggal, Debashish Chowdhury, Ankit Sharma, Kalpana Bansal

Neurology, G B Pant Hospital, New Delhi, India

E-mail: drvineet103@gmail.com

Background and Aim: Background: Although measurement of opening pressure is the gold standard for diagnosis of IIH, because of the invasive nature of LP, reliable and non–invasive methods of detecting elevated intracranial pressure are needed. Aim: To determine the correlation between Optic Nerve Sheath Diameter (ONSD) as determined by transorbital sonography and CSF opening pressure in patients with Idiopathic intracranial hypertension. Methodology: This was a cross-sectional study that included 30 patients with IIH diagnosed by Friedman criteria[1]. All patients underwent detailed clinical examination and optic nerve sheath diameter was determined by Transorbital ultrasound and MRI. Cerebrospinal fluid opening pressure (CSFop) was determined by lumbar puncture performed in the lateral decubitus position. Correlation between the variables was evaluated using the Pearson correlation coefficient for parametric data whereas Spearman’s rank correlation coefficient was used for non-parametric data. Result: On the basis of Friedman Criteria, 30 patients were diagnosed as IIH. The mean age of the patients was 31.73 ± 8.57 years and 90% of patients were females. The mean CSFop in IIH patients was 301.5 ± 60.92 mm H2O (95% CI = 247.38 to 303.45). The mean ONSD as measured by USG was 6.38 ± 0. 55 mm and had a moderate positive correlation with CSFop (Pearson correlation coefficient of 0.67 [p = 0.0001]). The mean ONSD as measured by MRI was 6.13 ± 0.57 mm and had a moderate positive correlation with the CSFop with a Pearson correlation coefficient of 0.61 (p = 0.0003). Conclusion: ONSD as measured by USG or MRI has a moderate positive correlation with CSFop.

Award ID: 902

Tegmental Involvement In Herpes Encephalitis

Garima Sharma, Gagan Saxena

Internal Medicine, Rama Medical College, Uttar Pradesh, India

E-mail: drgarimasharma1996@gmail.com

Background and Aim: Encephalitis is defined as an inflammation of the brain caused either by infection, usually with a virus, or from a primary autoimmune process. (1) The most commonly identified viruses causing sporadic cases of acute encephalitis in immunocompetent adults are herpes viruses (herpes simplex virus [HSV], varicella- zoster virus [VZV], Epstein –Barr virus [EBV]) (1). Purpose is to assess rare tegmental involvement in herpes encephalitis. Methodology: case report of 25 years old pregnant female Result: Atypical finding of direct involvement of tegmentum of mid brain on MRI Brain due to HSV encephalitis. Conclusion: 1. Viral Encephalitis should be considered in differential diagnosis of acute encephalitis syndrome particularly in endemic country like India. 2. In such cases, clinical examination, CSF analysis and neuroimaging along with other relevant investigation should be done to support diagnosis and rule out other possible etiologies.

Award ID: 903

Spectrum of Involvement and Long-term Outcomes of Cognitive Impairment in Autoimmune Encephalitis

Shreyashi Jha, Netravathi M, Ravindranadh Mundlamuri Choudhary, Suvarna Alladi

Neurology, NIMHANS

E-mail: shreyashi.jha11@gmail.com

Background and Aim: Cognitive impairment is a core symptom of autoimmune encephalitis (AE), however, literature on long term cognitive outcomes are sparse. We conducted a prospective study to assess cognitive long-term outcomes, domains involved and identify predictors. Methodology: Cognitive assessment (≤ 12 years: Vineland Social Maturity Scale (VSMS), 13-18 years: Montreal Cognitive Assessment (MoCA) and >18 years: Addenbrooke’s Cognitive Examination-III and repeated after six months in patients with AE Result: Among 74 patients, 38 (51%) were less than 18 years of age, 27 (37%) adults and nine (12%) late onset AE (more than 45 years) aged 21.8 (21.8 ± 17.0) years. 45 (61%) patients were females. Cognitive impairment was seen in 100% of late onset AE, 85% adults and 58% children, 53 (89.8%) {anti-LGI-1 (100%), anti-NMDAR (73%), anti-CASPR2 (71%)} and nine (60%) seronegative patients (p = 0.005). Cognitive impairment was seen in 60% at follow-up. All median scores improved (MMSE: 12 v 28, ACE-III: 34 v 72, MoCA: 12 v 22, VSMS social age: 32.8 v 36.8 (p = 0.007). Domains of memory, (10 v 14), fluency (5 v 9), attention (5 v 8) were affected to a greater extent than language (16 v 18) and visuospatial (12 v 23) domains. Higher mRS scores, abnormal background on EEG and epileptiform discharges correlated with lower scores on MMSE, MoCA and ACE-III. (p = 0.001, p = 0.036 and p = 0.001). Late onset, mutism, psychiatric features, altered consciousness, ICU admission, abnormal MRI brain associated with residual cognitive impairment (p < 0.05). Conclusion: Cognitive impairment is common in AE; especially late onset and anti-LGI-1, globally affects all domains in the acute phase and shows incomplete recovery of attention, fluency, and memory.

Award ID: 904

Therapeutic Effects of Gait Corrected Multimodel Exercise on Gait Dysfunction Shaking Palsy-A Single Case Study

Karthikeyan T

Physiotherapy, NIMHANS

E-mail: karthik_77in@yahoo.co.in

Background and Aim: Back ground: Shaking palsy also called as Parkinson. It is a central nervous system disorder characterized by reduced muscular power, loss of voluntary movement, posture & propulsive gait. Objective: To find out the therapeutic effects of gait corrected multimodel exercise on gait dysfunction sp. Methodology: A single case study is a 58 years old diagnosed with shaking palsy and referred to neuro rehab care for further management. Initially physiotherapy assessment carried out followed by gait corrected multimodel exercise introduced & recorded through gait characteristic changes noted mainly on stride length, step length, and cadence. Therapeutic exercise consists of Gait corrected multimodel exercise, gait, balance corrected exercise, graded exercise and stretching weekly 6 days/1 hours duration (15 minutes (Warm up) +45 minutes (Therapeutic Treatments)/6 weeks period, totally 36 session. Treatment tools used in this study treatment chair, inch tape, paint marker & stop watch, treatment couch. Inclusion: shaking palsy medicine & other rehab services (OT, PT, PMR, psychology) on, Cross leg standing with 30 sec. Exclusion: Shaking palsy medicine off, Cross leg standing with less than 30 sec Any injury, dislocation & subluxataion to lower extremity Research outcome: TUG. Result: Pre TUG score 9.5s and post Post TUG score 15.6 (p < 0.5). Conclusion: The investigator concluded that post 6 weeks tailored made this therapeutic form of gait corrected multimodel exercise which help to minimizing Parkinson motor symptoms, changes in gait character in stridelength, step length, cadence for SP.

Award ID: 905

Meningitis Caused by Shigella Flexneri-A Case Report

Shraddha Shrivastava, Neha Rai, Swarupa Bansode, J. S. Kathpal, Dhanraj Panjwani

Neurology, Choithram Hospital and Research Centre Indore, Madhya Pradesh, India

E-mail: shraddha1509@gmail.com

Background and Aim: Bacterial meningitis carries high mortality risk and even among survivors, the incidence of neurological after effects approaches 20% worldwide. Meningitis caused by Shigella flexneri is quite rare and till date, there have been very few case reports of Shigella flexneri associated with encephalopathy. We describe here our experience with this entity in a young female patient and her successful treatment. This case highlights the need for thorough microbiological diagnosis for satisfactory management of meningitis. Methodology: Our patient was a 28 year old woman with a background history of pyoderma gangrenosum. She presented with a reduced level of consciousness in the setting of fever, vomiting and seizure episodes. Imaging was suggestive of meningitis. Lumbar puncture was performed and a sample of cerebrospinal fluid (CSF) was obtained. Although microscopy was negative, culture grew Shigella flexneri. We treated her with Iv antibiotics. Result: Our patient responded well to treatment with dramatic improvement in neurological status. She was discharged with no residual neurological deficit Conclusion: We report a case of meningitis with rare etiology. A high index of suspicion led to accurate diagnosis and treatment. Moreover, the coexistence of pyoderma gangrenosum and meningitis (Shigella flexneri) has been very seldom reported. The treatment of pyoderma gangrenosum is largely based on suppressing the immune response and associated inflammation with corticosteroids and other immuno-suppressants. The use of these drugs in the context of associated infection warrants caution and careful monitoring.

Award ID: 906

Prevalence of Restless Leg Syndrome (RLS) in Parkinson’s Disease and its Correlation with Serum and CSF Iron and Ferritin Level

Divya Reddy, Divya Bandlapalli, Rukmini Mridula

Neurology, NIMS Hospital Hyderabad, Telangana, India

E-mail: dr.divyareddy2013@gmail.com

Background and Aim: Restless legs syndrome (RLS) is a common non motor symptom of Parkinson‟s disease (PD) and has been found to be associated with low levels of iron and ferritin in blood. PRIMARY OBJECTIVES 1. To assess the prevalence of RLS in PD. 2. To assess the severity by RLS rating scale. 3. To see correlation between RLS severity and levels of iron and ferritin in CSF and serum. SECONDARY OBJECTIVES 1. To look for correlation between levels of iron and ferritin in CSF and their corresponding values in the serum. 2. To look for correlation between RLS and clinical features of Parkinson‟s disease, Nigrosomal MRI, other sleep disorders and apathy and fatigue. Methodology: : 100 PD patients were included and divided into PD with RLS group and PD with no RLS group. Serum iron and ferritin were assessed in all. In patients with RLS and in a subset of RLS negative PD patients, lumbar puncture was done and CSF samples were collected for iron levels Result: PD-RLS frequency was 9%. PD-RLS group had significant association with PLMS, and lower levels of iron and ferritin in the CSF. In PD-RLS group, RLS-RS score had no significant correlation with levels of iron and ferritin in the serum or CSF. Levels of serum iron were positively significantly correlated with that of CSF levels of iron. Conclusion: RLS was associated with low levels of iron and ferritin in the CSF (even in the presence of normal serum iron and ferritin) and has strong association with PLMS

Award ID: 907

Greater Occipital Nerve Blockade for the Preventive Treatment of Chronic Migraine: A Randomized Double-Blind Placebo-Controlled Study

Apoorva Tomar, Debashish Chowdhury, Apoorva Tomar, Vaibhav Deorari, Ashish Duggal, Anand Krishnan, Arun Koul

Neurology, GIPMER

E-mail: apoorvatomar1991@gmail.com

Background and Aim: Greater occipital nerve blockade (GONB) for the prevention of chronic migraine (CM) has a limited evidence base.1-3 To study efficacy and tolerability of GONB for prevention of CM. Methodology: This double-blind, placebo-controlled, parallel-group trial, following a baseline period of 4 weeks, randomly assigned patients of CM 1:1 to receive 4-weekly bilateral GONB with either 2 ml of 2% (40 mg) lidocaine (active) or 2 ml of 0.9% saline (placebo) injections for 12 weeks. The primary and key secondary efficacy endpoints were a change from the baseline in the mean number of headache and migraine days and the achievement of ≥50% reduction in headache days from baseline across the weeks 9-12. Safety evaluations included reporting of serious and other adverse events. Result: Twenty-two patients each were randomly allocated to the active and the placebo group. Mean headache days at baseline (±SD) in the active and the placebo group were 23.4 ± 4.4 and 22.6 ± 5.0 days respectively. The active group compared to the placebo had a significantly greater least-squares mean reduction in the number of headache and migraine days [-4.2 days (95% CI: -7.5 to -0.8; p = 0.018) and -4.7 days (95%CI: -7.7 to -1.7; p = 0.003) respectively]. 40.9% of patients in the active group achieved ≥50% reduction in headache days as compared with 9.1% of patients receiving a placebo (p = 0.024). Overall, 64 mild and transient adverse events were reported. Conclusion: Four-weekly GONB with 2% lidocaine for 12 weeks was superior to placebo in decreasing headache and migraine days in patients with CM with a good tolerability profile.

Award ID: 908

Reverse-INO in PSP? – A Review of Nystagmus Patterns in the Disease

Vaibhav Mathur, Dinesh M. Khandelwal, Mridula M. Singh

Neurology, Jaslok Hospital, Mumbai, Maharashtra, India

E-mail: drmathurvbhv@gmail.com

Background and Aim: Progressive supranuclear palsy (PSP) features parkinsonism with early falls and prominent eye movement abnormalities, mainly saccadic slowing, followed by gaze limitation. Nystagmus is not considered typical for PSP, being more commonly associated with MSA. Gaze evoked nystagmus (GEN) however, has been described in PSP. The different patterns of GEN can be : (i) GEN accompanying intranuclear ophthalmoplegia (INO) like findings; (ii) GEN with lateral gaze impersistence (Pseudo/Reverse INO) and (iii) GEN present only in specific body positions. Methodology: We saw a 65 year male, with 2 years of Slowness of daily activities, occasional backward falls, apathy, behavioral complaints, and double vision without any diurnal variation. Higher mental functions – FAB 10/18, Subcortical Frontal affection. Speech was hypophonic and slurred. Oculomotor findings were supranuclear vertical > horizontal gaze paresis, slow horizontal saccades, bilateral lateral > medial gaze restriction (lateral gaze impersistence) with gaze evoked nystagmus. Tone was increased, axial > limbs. Deep tendon reflexes were bilaterally brisk with mute plantar responses. No sensory and cerebellar signs were elicited, there was a slow, short stepped, extrapyramidal gait. Result: In our patient, a diagnosis of PSP was evident with clinical scenario and MRI findings. GEN is an overlooked feature of PSP, probably as it only appears transiently, when horizontal saccades are not fully affected. Conclusion: Central nystagmus is present in more than a third of patients with PSP. It may present as symmetrical or asymmetrical gaze-evoked nystagmus or as dissociated gaze-evoked nystagmus related to internuclear ophthalmoplegia and probably arises from neurodegeneration of the neural integrator.

Award ID: 909

Intravenous Immunoglobulin in Guillain Barre Syndrome: A PRESS-ing Case

Vikas Prabhu, Ravindra Kumar Garg, Imran Rizvi, Neeraj Kumar, Ravindra Kumar Garg

Neurology, KGMU Lucknow, Uttar Pradesh, India

E-mail: vikasneurokgmu@gmail.com

Background and Aim: Guillain-Barré syndrome is an acute demyelinating polyneuropathy and IVIG remains a commonly administered treatment. Several risk factors are known to predispose to Posterior reversible encephalopathy syndrome (PRES) which manifest with headache, seizure, altered sensorium and visual disturbances. Some of the rarer known risk factors among them are IVIG therapy and GBS associated dysautonomia. We report a similar case of PRES post IVIG therapy in a patient of GBS. Methodology: We report a case of PRES in patient of GBS post IVIG therapy Result: A 67-year-old female patient with no prior comorbidities presented with acute onset ascending flaccid areflexic quadriparesis of 5 days duration. Nerve conduction study done showed acute sensorimotor axonal neuropathy. CSF done showed albumino-cytological dissociation. Patient was diagnosed with Guillain Barre Syndrome (AMSAN) and was started on IVIG as per bodyweight for total of 5 days. One day after completion of her IVIG course, patient started complaining of bilateral vision loss and then went on to develop alteration of mental status along with raised blood pressure reading. Neuroimaging done then showed bilateral occipital hypodensities and patient was diagnosed with PRES. Patient was managed accordingly with gradual resolution of symptoms. Conclusion: A high index of suspicion for PRES should be kept in patients of GBS who develop neurological symptoms and signs, especially visual disturbance after IVIG infusion. Interplay as well as independent causation of both GBS and IVIG therapy plays a part in PRES and early recognition of same may prove vital.

Award ID: 910

EEG Background Activity in Cortical Myoclonus: Reappraisal of “Spiky Alpha” as a Biomarker Candidate

Maya Tojima, Masao Matsuhashi, Katsuya Kobayashi, Takefumi Hitomi, Kiyohide Usami, Akihiro Shimotake, Ryosuke Takahashi, Akio Ikeda

Neurology, Kyoto University Graduate School of Medicine, Japan

E-mail: maya0826@kuhp.kyoto-u.ac.jp

Background and Aim: Patients with cortical myoclonus (CM), especially progressive myoclonus epilepsy (PME) including benign adult familial myoclonus epilepsy (BAFME), are anecdotally known to have increased amplitude and sharpness of background activities in EEG, used to be called as “spiky alpha”. We aimed to evaluate posterior background activities in CM patients. Methodology: This retrospective study included patients who were 1) diagnosed CM (PME n = 35, non-PME n = 11) and juvenile myoclonic epilepsy (JME) (n = 24), and 2) clinically underwent EEG. In each EEG, 5 segments of 5−10 sec duration with clear posterior dominant rhythm (PDR) were selected. Band-limited power of 4 frequency ranges (operationally called, alpha: 8−13, beta1: 13−30, beta2: 30−59, beta3: 61−100 Hz) of posterior area (P3/P4, O1/O2), and PDR frequency and power at O1/O2 electrodes were calculated by power spectrum analysis. We compared clinical and EEG findings between 1) CM (PME and non-PME) and JME, 2) BAFME and non-BAFME in PME and 3) 2 groups with or without benzodiazepines (BZ) in PME. Result: 1) PME patients significantly had higher beta1 and 2 power than JME patients (all p-values were <0.01). In PME, 2) BAFME patients significantly had higher beta1 and 2 power than non-BAFME patients (p = 0.002, 0.008, 0.04), whereas 3) there was no significant difference between patients with or without BZ. Conclusion: PME especially BAFME patients had significantly higher beta power of posterior area, which may be responsible for appearance of “spiky alpha” and it may be a practically easy and accessible biomarker candidate.

Award ID: 911

Assessment of the Role of Continuous EEG Monitoring in Critically Ill Patients with CNS Infection

Divya B, Jabeen S. A.

Neurology, NIMS Hospital Hyderabad, Telangana, India

E-mail: dharani7591@gmail.com

Background and Aim: To determine the prevalence of EEG abnormalities and electrographic seizures in critically ill patients with primary CNS infection and study the correlation between electrographic abnormalities and patient outcome. Methodology: 41 patients with the diagnosis of primary CNS infection from November 2017 to October 2018 included. 40/41 were diagnosed as bacterial 7.5% (n = 3), fungal 2.5% (n = 1), tubercular 45% (n = 18), viral 40% (n = 16) and parasitic infection 5% (n = 2). cEEG was performed in all these patients and analysed. Result: Of 40 patients, 12.5% (n = 5) patients had ESz and periodic discharges were recorded in 12 patients (30%). Out of these Generalised Periodic discharges (GPD) were seen in 6 patients (15%), Lateralised periodic discharges (LPD) in 4 (10%) and bilaterally independent periodic discharges (BIPD) in 2 (5%). Notably 50% (n = 20) out of 40 cases were observed with major EEG abnormality in the form of ESz (n = 5; 25%), PDs (n = 12; 60%) and background suppression (n = 3; 15%). Higher age is associated with major EEG abnormality (p = 0.032). Clinical seizures prior to cEEG (p = 0.002) and Stupor or coma prior to EEG (p = 0.044) also associated with EEG with major abnormality. On the bases of Glasgow outcome score male patients were shows more prone to poor outcome (p = 0.003). Conclusion: ESz and PDs were independently associated with poor outcome which was not seen in our study. The reason could be small sample size and prospective design of the study which has led to inclusion of wider spectrum of cases than in the previous study

Award ID: 912

Retinal Nerve Fibre Layer Thickness as a Biomarker for Parkinson’s Disease and its Correlation with Rapid Eye Movement (REM) Sleep Behavior Disorder and Cognitive Function

Shivangi MS, Lulup Kumar Sahoo, Srikanta Kumar Sahoo

Neurology, IMS and Sum Hospital, Bhubhaneshwar, Odisha, India

E-mail: drshivangisingh13@gmail.com

Background and Aim: Parkinson’s disease is a neurodegenerative condition primarily due to dopaminergic depletion encompassing a wide variety of non motor symptoms which often manifests before the classic motor symptoms. Dopamine dysfunction is also seen in retina, particularly in the horizontal and amacrine, bipolar, and ganglion cells. OCT is a non invasive tool which measures Peripapillary retinal nerve fiber layer (pRNFL) and macular scans. Various studies have demonstrated significant retinal thinning in PD patients and its correlation with disease severity. This study is aimed at understanding the neurodegenerative process and offer RNFL as a biomarker for Parkinson’s disease and its correlation pertaining to REM sleep behaviour disorder and cognitive function. Methodology: A Crosssectional, singlecenter study conducted from February 2022 to June 2022 with a sample size of 20 consenting Idiopathic PD patients who were selected on the basis of MDS-PD criterion. HNY staging was done to assess the severity of disease. MDS-NMS, RBD screening questionnaire and MOCA scoring was used to categorize patients and OCT was performed to assess the RNFL thickness. The collected data was compared with similar parameters recorded from the eyes of age matched healthy controls. Result: Severity of RNFL thinning paralleled the clinical severity of Parkinson’s disease. Patient with RBD and cognitive decline were found to have greater RNFL thinning than other PD patients. Conclusion: OCT can be a non invasive biomarker in PD patients and provide a window of opportunity for therapeutic intervention in PD patients with non motor symptoms in early course of disease.

Award ID: 913

The Alteration of Lymphatic System after Ischemic Stroke in Mice

Yasuhiro Kuwata, Takakuni Maki, Ken Yasuda, Naoki Takayama, Shintaro Toda, Ryosuke Takahashi

Neurology, Graduate School of Medicine, Kyoto University, Japan

E-mail: y_kuwata@kuhp.kyoto-u.ac.jp

Background and Aim: Stroke remains one of the world’s most serious health problems despite recent progress in thrombolytic therapy and intravascular thrombectomy. Recent study has shown that the pathway from meninges to cervical lymph nodes is involved in the detrimental interaction between brain injury and peripheral immune activation. However, how the ischemic lymphatic system contributes to neurovascular damage and repair remains elusive. Therefore, the aim of our study is to examine the roles of lymphatic endothelial cells (LECs) after ischemic stroke. Methodology: Immunohistochemistry was performed to assess the behavior of LECs and their interactions with other cell types in a mouse model of transient middle cerebral artery occlusion (MCAO) at different time points on day7, 10, 14 after MCAO. To identify LECs, we used primary antibodies as follows: lymphatic vessel endothelial hyaluronan receptor-1, prospero homeobox protein-1 and vascular endothelial growth factor receptor-3. Result: The number of LECs started to increase in ischemic region on day10 after MCAO and most of LECs were located around the vasculature. On day14 after MCAO, the number of inflammation-related cells (macrophages, microglia, dendritic cells, and CD4 positive T cells) were also increased in the ischemic side of meningeal, perivascular, and parenchymal areas, and LECs were located in close proximity with these cells. Conclusion: Our data suggests that reactive LECs may actively participate in the damage and repair processes after cerebral ischemia. Further analysis of the neurovascular unit-lymphatic crosstalk would enable us to find novel therapeutic strategies against ischemic stroke.

Award ID: 914

Cryptogenic New Onset Refractory Status Epilepticus (NORSE)-A Case Report

Shraddha Shrivastava, C. B. Pandey, Shubhanjali Joshi

Neurology, Choithram Hospital and Research Centre Indore, Madhya Pradesh, India

E-mail: shraddha1509@gmail.com

Background and Aim: Cryptogenic NORSE is a relatively recent term describing the occurrence of refractory seizures with no discernible etiology. We describe here our experience with this entity and its successful treatment with plasma exchange (PE). Given the excellent results, we advocate early consideration of PE in all cases of cryptogenic refractory status epilepticus (RSE). Methodology: Our patient was a 24 year old woman with no past history suggestive of a seizure disorder. She presented with RSE, having received phenytoin, fosphenytoin, valproic acid, levetiracetam and topiramate prior to being referred to us. Magnetic Resonance Imaging, cerebrospinal fluid analysis and electroencephalogram provided no clue. Viral serology and polymerase chain reaction for viral nucleic acids were negative. Tuberculous meningitis was ruled out. Thyroid function was within normal limits. She was initially treated with intravenous midazolam and thiopentone. This was reasonably successful but seizure activity returned on attempting to wean off. With suspicion of autoimmune etiology, corticosteroids were trialed but were also unsuccessful. Plasma exchange was ultimately considered Result: PE brought about an instant response with complete cessation of seizures, even on discontinuing midazolam and thiopentone. She regained consciousness and with no further adverse events, she was ultimately discharged. She remains under our follow up and has shown no neurological sequelae till date Conclusion: NORSE frequently results in death and debilitating neurological sequelae. We strongly recommend early institution of PE for successful management of this condition

Award ID: 915

Potential Embolic Sources in Embolic Stroke of Undetermined Source Patients with Patent Foramen Ovale

Benjamin Tan, Keith Toh, Ming Yi Koh, Kathleen Ong, Vijay K. Sharma, Ching Hui Sia

Division of Neurology, Department of Medicine, National University Hospital, Singapore

E-mail: benjaminyqtan@gmail.com

Background and Aim: A patent foramen ovale (PFO) may co-exist with other potential embolic sources (PES) in patients with embolic stroke of undetermined source (ESUS), leading to difficulty in attributing the stroke to either the PFO or other PES. We investigate the prevalence of concomitant PES in ESUS patients with PFO. Methodology: A retrospective cohort study was conducted in a tertiary stroke centre. Consecutive patients with ESUS and a concomitant PFO admitted between 2012 and 2021 were included in the study. Baseline characteristics and investigations as part of stroke workup including echocardiographic and neuroimaging data were collected. Potential embolic sources were adjudicated by 2 independent neurologists after reviewing the relevant workup. Result: Of 1,487 ESUS patients, 309 patients who had a concomitant PFO with mean age of 48.8 ± 13.2 years were identified. The median Risk of Paradoxical Embolism (RoPE) score was 6 (IQR 5-7.5). Amongst the 309 patients, 154 (49.8%) only had PFO, 105 (34.0%) patients had 1 other PES, 34 (11.0%) had 2 PES, 16 (5.2%) had 3 or more PES. The most common PES were atrial cardiopathy (23.9%), left ventricular dysfunction (22.0%) and cardiac valve disease (12.9%). The presence of additional PES was associated with age ≥60 years (p < 0.001), RoPE score ≤6 (p < 0.001) and the presence of comorbidities including diabetes mellitus (p = 0.004), hypertension (p < 0.001) and ischemic heart disease (p = 0.011). Conclusion: A large proportion of ESUS patients with PFO had concomitant PES. Concomitant PES was associated with older age and lower RoPE score.

Award ID: 916

A Diagnostic Dilemma

Krishna Vani Nemalidinne, Rajiv Anand, Varun Rehani, Sujatha Das

General Medicine, BLK MAX Hospital

E-mail: nkrishnavani@gmail.com

Background and Aim: Hypothyroidism may present with a wide range of symptoms ranging from minor fatigue to life-threatening myxedema coma. Here we present a case of Hoffman’s syndrome aka hypothyroid myopathy presenting as generalized fatigue and pure motor quadriparesis with proximal muscle predominance. Methodology: A 21-year male presented to us in a wheelchair with difficulty walking for 5 years who was labeled as a case of Beckers muscular dystrophy on the basis of calf hypertrophy, elevated creatine phosphokinase levels (CPK), and myogenic pattern on electromyogram from a tertiary care government hospital. Initial evaluation was suggestive of pale dry skin, coarsened facial features, brittle nails, and alopecia. Clinical examination revealed calf hypertrophy, pure motor quadriparesis with proximal muscle predominance (power 4/5), waddling gait, and sluggish ankle jerk. Laboratory investigations revealed a severely deranged thyroid profile (FT3 <0.7, FT4<1.0 pmol/L, TSH 108 IU/ml) with elevated CPK (15024 U/L) and liver enzymes (SGOT: 95, SGPT:87). Result: When he presented to us, Prima facie suggested something else. An urgent thyroid profile reported an alternative diagnosis in just a few hours of his visit. He was started on thyroxine supplements (75 mcg). Now our patient is ambulatory with a significant improvement in his quality of life. Conclusion: Hoffman’s syndrome is a rare entity of hypothyroidism, which presents with pseudo-hypertrophy of calf muscles and elevated CPK levels. Our take-home message is that all treatable secondary causes should be ruled out before labeling a syndrome with a fatal outcome.

Award ID: 917

A Case of Complete Ophthalmoplegia Secondary to Herpes Zoster Ophthalmicus

Nareng Padun, Kishore Rajendran, Rajasekaran Meenashisundaram, Vignesh Karunaharan

Neurology, KAPV Government Medical College

E-mail: padunnareng@gmail.com

Background and Aim: Based on a review of well-documented reported cases, herpes zoster ophthalmicus (HZO) is rarely associated with complete unilateral ophthalmoplegia. Our aim is to report a case with complete ophthalmoplegia after herpes zoster ophthalmicus. Methodology: A 73-years man had been diagnosed with herpes zoster ophthalmicus 2 weeks before presentation to our department. seven days after stopping oral aciclovir, he presented with periorbital pain and swelling, ptosis and complete ophthalmoplegia of right eye. On examination, afebrile, and vital were stable. On right side a well-demarcated healing vesicular, crusting, rash corresponding to the distribution of the first branch of the trigeminal nerve (V1) and V2 was seen, visual acuity in his right eye was 6/12 with a relative afferent pupillary and complete ophthalmoplegia. Other examination was normal. Magnetic resonance imaging (MRI) showed no obvious pathology in the region of the cavernous sinus and superior orbital fissure. Cerebrospinal fluid analysis study was normal and the patient was treated with a 2-week course of acyclovir and corticosteroids for one month. Result: The patient was fully recovered from the complete ophthalmoplegia in five months after the onset of the cutaneous lesion. Conclusion: Complete ophthalmoplegia is a rare ophthalmic complication of herpes zoster infection. Therefore, an evaluation of extraocular muscle and lid function should be performed during the examination of herpes zoster patients in order to screen for ophthalmoplegia

Award ID: 918

Late onset Cobalamin C Disease: An unusual masquerader of Acute psychosis

Prachi Mohapatra, Rohit Bhatia, Anu Gupta, Roopa Rajan, Padma M. V. Srivastava, Mamta Bhusan Singh, Venugopalan Y. Vishnu

Neurology, AIIMS New Delhi, India

E-mail: prachimo1994@gmail.com

Background and Aim: Cobalamin C disease is a rare inborn error of cobalamin metabolism. 90% cases present early (<1 year) but the late onset (>4 years) presentation is rare and often misdiagnosed because of varied phenotypic presentations. Methodology: 31 year old female with no prior comorbidities presented with 1.5 months history of acute onset neurobehavioral symptoms in form of delusions of persecution, reference, misidentification, overgrooming, overspending, auditory hallucinations and on/off episodes of acute confusion. She also had urinary frequency and urge incontinence. On examination, lobar functions were intact and apart from generalized hyperreflexia there was no other positive finding. She was initially diagnosed as acute psychosis and treated with antipsychotics but with no improvement in symptoms. MRI was done which showed T2/flair hyperintensities in b/l centrum semiovale, coronal radiata and cervical cord following which the possibility of demyelination was kept. Work up for primary and secondary demyelination including NMO/MOG, CSF analysis, EEG, VEP/BERA, autoimmune encephalitis panel were inconclusive. VitB12 and folate levels were normal but homocysteine was elevated. Urinary and serum MMA were also elevated. Considering her clinical phenotype, VitB12 remethylation defect was suspected and clinical exome sequencing was done which showed heterozygous mutation in MMACHC gene confirming the diagnosis of Cobalamin C disease. The patient was treated with parenteral VitB12 following which there was significant improvement in her symptoms. Result: VitB12 remethylation defects can present only as acute psychosis without overt neurological features. Conclusion: Late onset cobalamin C disease is a rare but treatable inborn error of metabolism. A high index of suspicion should be kept for early diagnosis and treatment.

Award ID: 919

Efficacy of Steroids in Seizure Control and Dipole Stabilisation in Drug Resistant Electrical Status Epilepticus in Sleep (ESES) due to Structural Cause

Neetha Balaram, James Jose

Neurology, Government Medical College, Kozhikode, Kerala, India

E-mail: neethabalaram@gmail. com

Background and Aim: Symptomatic causes of ESES and treatment options are underdiagnosed and under reported in literature. Neurocognitive regression and worsening of seizures difficult to assess in this group. Development of new EEG tools other than spike wave index (SWI) more crucial. Assessing dipole stability is a good method to assess the efficacy of treatment. Methodology: Twenty children included 2-12 years with drug resistant epilepsy 1. ESES based on electrical criteria of >50% Spike-wave index (SWI). 2. Neurological regression in atleast two domains. 3. MRI brain showed structural lesions. EEG assessment and stability quotient done at 0, 3, 6 months and one year of starting methylprednisolone using a source localisation software (Besa Research 7.1). Stability quotient = Total number of Averages÷ Number of spikes (N). Intravenous Methylprednisolone pulse therapy given at 30 mg/kg/day for three consecutive days for six months. Seizure control defined as atleast 50% reduction in seizure frequency. Dipole stabilisation defined when both Stability-quotient ≥ 0.8 and Spike-wave index< 50%. Result: 1 .18 children (90%) had seizure control at 6 months and 15 of 20 children (75%) maintained this at 12 months. 2. 18 children had stability quotient of <0.8 before starting pulse and 14 (77.78%) on follow up improved their stability quotient to ≥ 0.8 at 6 months and 12 (66.67%) at 12 months. 3. 15 children (75%) had SWI <50% at 6 months and 13 (65%) at 12 months. Conclusion: Steroids help in clinical and EEG (dipole) stabilisation in ESES dueto structural causes. Thus can serve as effective bridging therapy. Assessing stability quotient as a measure of dipole stability gives better association with seizure control.

Award ID: 920

Down with the Dancing Chin: An Intriguing Case Series of Masseter Spasms

Darshan Pandya, Satish Khadilkar, Jamshed Lalkaka, Khushnuma Mansukhani

Department of Neurology, Bombay Hospital Institute of Medical Sciences,??

E-mail: darshanpandya1994@gmail.com

Background and Aim: Masseter spasms are rare movement disorders characterized by involuntary jaw clenching, which can be unilateral/ bilateral. They typically manifest as a symptom of a generalized movement disorder or in isolation. We analyzed clinical and electromyographic data from six patients with unilateral or bilateral masseter spasms. Methodology: This is a retrospective study of 6 patients who presented to BH with masseter spasms from 2002 to 2022. Detailed electromyographic recordings and electrophysiological studies of the facial and masticatory muscles were performed on all patients. Result: Out of 6 patients, five were male. The mean age was 35.2 years. Two patients had hemimasticatory spasms with hemifacial atrophy, one on the left and the other on the right. One patient had left spasm with hypertrophy, and one had neither hypertrophy nor atrophy. One patient had cephalic tetanus and couldn’t open his mouth. In all, apart from the tetanus patient, bursts of motor units were seen during masseter spasms. In two patients with hemiatrophy, silent period was absent during the spasm. All five patients improved after local botulinum toxin injections, except one. Conclusion: Masseter spasm is a rare condition with varying possible etiologies that can be severely disabling or potentially reversible with botulinum toxin injection locally.

Award ID: 921

Hereditary Spastic Paraplegia type 76: First Cases from India

Rahul Oinam, Ayush Agarwal, Achal Kumar Srivastava

Neurology, AIIMS Delhi, India

E-mail: rahuloinam@gmail.com

Background and Aim: Affecting about 2 in 100,000 people, Hereditary spastic paraplegia present as pure spastic paraparesis or in a complicated form (1,2). A majority of HSPs are due to SPAST gene mutation. SPG76 due to CAPN1 mutation is an uncommon cause of HSP. Only 35 cases have been reported in literature with none from India (3). Methodology: A 40-year-old gentleman from North India presented with progressive stiffness and scissoring of gait since 27 years of age followed by imbalance and swaying. Nine years into the illness he developed urinary urgency and incontinence along with a slurred speech. Examination revealed slowed vertical and horizontal saccades, lower limb predominant UMN signs, cerebellar dysarthria, cerebellar signs in both upper and lower limbs, and gross impairment of tandem walking. Cognition, sensory function and general physical examination were unremarkable. His sister also had a similar presentation and clinical findings except for dysarthria and onset at 22 years of age. Result: MRI brain of both showed T2/ FLAIR hyperintensity at tips of frontal horns of lateral ventricles and mild cerebellar atrophy while MRI spine revealed no pathological findings. Both tested positive for CAPN1 mutation. Conclusion: We report the first two cases of HSP76 from India. In comparison to previous case series our patients had a similar presentation with onset in 3rd decade, prominent cerebellar ataxia and upper limb spasticity (3). Ocular motor abnormality in our patients was the only clinical feature which has not been commonly seen in the previous cases reported.

Award ID: 922

An Imaging Clue for a Treatable Metabolic Myelopathy Mimicking Neuromyelitis Optica Spectrum Disorder

Hansashree Padmanabha, Sameetha Prabhu, Rohan Mahale, Jitender Saini, Rita Christopher, Pooja Mailankody, P. S. Mathuranath

Neurology, National Institute of Mental Health and Neurosciences,

E-mail: hansa777@gmail.com

Background and Aim: The authors would like to present a case of a treatable metabolic myelopathy mimicking NMOSD. Methodology: Retrospective review of a case of treatable metabolic myelopathy Result: 9-year-old boy, premorbid well, studying in 4th std had presented to us with sub-acute onset, bilateral painless vision loss, urge incontinence and features of autonomic symptoms of 45 days duration. Modified Rankins score of 5 at admission. Clinical Possibility of Longitudinally extensive transverse myelitis with optic neuritis with autonomic dysfunction and PRES was considered. The possible aetiology considered was primary demyelination ?NMOSD/MOGAD, others being inflammatory disorders or metabolic myelopathies. His routine investigations including hemogram/biochemical profile/B12/Homocysteine/folate/vasculitis work up (ANA profile, ANCA) /demyelination work up (NMO/MOG)/infective work up were negative. CSF lactate was markedly elevated and TMS showed mildly elevated methylmalonylcarnitine/C4DC/C5-OH. Neuroimaging showed LETM with optic neuritis with additional involvement of mammillary bodies, fornix and tectal plate was unusual for a demyelinating pathology. Hence possibility of a metabolic myelopathy was considered and serum biotinidase levels were sent which came deficient and genetic analysis confirmed the same. Hence a final diagnosis of Biotinidase deficiency induced metabolic myelopathy was considered and child received high dose oral biotin. At six months follow up child is able to walk with assistance with MRS of 3-4. Conclusion: Biotinidase deficiency though rare is a treatable metabolic myelopathy and should be investigated in seronegative LETM’s with radiological involvement as described.

Award ID: 923

A Systematic Review of Randomized Controlled Trials for Effectiveness and Tolerability of Greater Occipital Nerve Blockade for the Prevention of Chronic Migraine

Samiran Chowdhury

General Medicine, Vivekanand Polyclinic and Institute of Medical Sciences

E-mail: samiranchowdhury15@gmail.com

Background and Aim: The effectiveness of greater occipital nerve blockade (GONB) for the prevention of chronic migraine (CM) is uncertain. We aimed to systematically review the literature on the effectiveness and tolerability of GONB for the prevention of CM. Methodology: We searched articles published in the English language in PubMed between 1969 and March 2022 using “greater occipital nerve block,” “chronic migraine,” “migraine,” “headache,” and randomized controlled trials (RCTs) as keywords. RCTs involving only CM patients were included. Result: Out of potential 540 articles, 7 RCTs were included that had significant methodological heterogeneity.1-7 Of these, 4 were double-blind (DB), 1 compared GONB with and without steroids, 1 compared two ultrasound (USG) guided GONB techniques and 1 was open-label. A DB-RCT found single GONB injection provided a greater reduction in headache frequency at 1 week.1 Of the three long-duration DB-RCTs, one study reported a positive result of GONB at month 1;2 the other two were negative,3,4 one of which, however, showed a positive result at month 3.4 One RCT found no benefit of adding steroids to GONB.5 A recent RCT found that the combination treatments of topiramate with GONB (with and without steroids) were more effective than topiramate monotherapy at month 3.6 Proximal USG-guided GONB significantly reduced the pain scores at months 1 and 3 compared to the central group.7 GONB was reported to be well tolerated by all the studies. Conclusion: Although promising, further trials are needed to find the efficacy of GONB for the prevention of CM.

Award ID: 924

A Patient with Recurrent Attacks of Demyelination

Tejal Agarwal

Neurology, Nizams Institute of Medical Sciences, Hyderabad, Telangana, India

E-mail: tejalagarwal007@gmail.com

Background and Aim: case report Methodology: A 46 year old male presented with altered sensorium for 6 days with Past history of gait ataxia and optic neuritis 1 ½ year and myelitis 8 months back and he was treated with steroids for these episodes. O/E He was drowsy and contractures present in both elbows, wrist, knee and thigh. All Reflexes are exaggerated with plantar reflex extensor on both sides. Imaging showed -Multiple hemispheric demyelinating lesions with open ring enhancement and demyelinating lesion extending from C3-C5 vertebral level. Serum MOG IgG Positive. He was also found to have cirrhosis of liver. Result: Anti MOG IgG syndrome with alcoholic Chronic liver disease with portal Hypertension was diagnosed. Patient is treated with Pulse dose of IV methylprednisolone and maintenance dose of oral steroids and azathioprine. Conclusion: Anti-MOG IgG syndrome is a Monophasic illness. We describe a case of relapsing Anti-MOG IgG syndrome.

Award ID: 925

A Rare Case of Atypical Atypical Parkinsonism

Sumirini Puppala, Sumirini Puppala, Surjyaprakash S. Choudhury, Srikanta Sahoo, Srikanta Sahoo, Lulup Kumar Sahoo

Neurology, IMS and Sum Hospital, Bhubhaneshwar, Odisha, India

E-mail: psumirini@gmail.com

Background and Aim: Rarely, a number of genetic parkinsonian conditions have been recognized that share some features with the clinical syndromes of progressive supranuclear palsy (PSP), cortico-basal degeneration (CBD), and multiple system atrophy (MSA), the classic phenotypic templates of atypical parkinsonism but do not typically fit into either of the diagnostic criteria as well, labelled as “Atypical Atypical Parkinsonism”. Methodology: This case report follows a 60-year old female with slowness of ADL, head reeling and repeated falls for 8-10 months. Result: Her clinical diagnostic criteria and her MRI findings correlated to her being categorized as ‘Atypical atypical parkinsonism’. Conclusion: We describe her clinical features, evaluation and radiodiagnosis for the same

Award ID: 926

A Cross Sectional Study on Drug Resistant Epilepsy in Patients Attending T.D. Medical College Alappuzha

Rohit Kumar, Shaji C. V., Kabeer K. A

Neurology, T D Medical College, Alappuzha, Kerala, India

E-mail: rohitramkumar106@gmail.com

Background and Aim: To throw some light on cases presenting as drug resistant epilepsy Methodology: This was a cross sectional study. We studied 150 patients over a span of 2 yrs from May 2020 to June 2022. We assessed the etiology of drug resistant epilepsies presenting to us. Result: Out of the 150, maximal no of DRE were noted due to PNES (32%), followed by ischemic seizures (22%), drug default seizures (16%), post traumatic epilepsy (10.66%), CPS (10%), ICSOL (6.6%), Epilepsy and Intellectual disability disorder (2.6%). Among PNES cases 53% were having GAD, followed by depression, BPAD etc. 3% had history of sexual abuse in childhood. Among males with DRE, 60% continued to take alcohol. Among drug default seizures, the most common factor was financial burden followed by casually forgetting to take drugs. 3 patients with partial seizures with impaired awareness had hippocampal sclerosis on follow up. Conclusion: Drug Resistant epilepsy poses a serious threat to the patient as well as the treating physician. It can cost a person’s life and has to be dealt with seriousness. Globally PNES amounts to around 25% of DRE. With good support from psychiatry department, we might be able to decrease the pill burden in them.

Award ID: 927

Left Ventricular Diastolic Dysfunction is Associated with Poor Functional Outcomes after Endovascular Thrombectomy

David Tang, Tony YW Li, Ching-Hui Sia

Neurology, National University Hospital, Singapore

E-mail: dirassa@hotmail.com

Background and Aim: With the advent of endovascular thrombectomy (ET), patients with acute ischemic strokes (AIS) with large vessel occlusion (LVO) have seen vast improvements in treatment outcomes. Left ventricular diastolic dysfunction (LVDD) has been shown to herald poorer prognosis in conditions such as myocardial infarction. However, whether LVDD is related to outcomes in ischemic stroke remains unclear. Methodology: We studied 261 consecutive LVO AIS patients who had undergone ET at a single comprehensive stroke centre. All patients had a valid transthoracic echocardiogram for analyses. We correlated LVDD to short-term mortality (in-hospital death) as well as good functional outcome defined as modified Rankin Scale of 0–2 at 3-months. LV diastolic dysfunction was assessed in accordance to international guidelines considering four main parameters including (a) an average E/e’ of > 14, (b) septal e’ velocity < 7 cm/s or lateral e’ velocity < 10 cm/s, (c) TR velocity > 2.8 m/s and (d) LA volume index (LAVI) > 34 ml/m2. Result: LVDD indicated poorer outcomes in terms of functional recovery at 3 months (OR 2.18, 95% CI 1.04-4.54, P = 0.038) but was not associated with increased in-hospital mortality (OR 2.18, 95% CI 0.60-7.99, P = 0.240) after adjusting for various confounders. Conclusion: In addition to conventional echocardiographic indices such as left ventricular ejection fraction, LVDD may portend poorer outcomes after ET, and this relationship should be investigated further.

Award ID: 928

Analysis of Spatio Temporal Propagation of Occipito-Frontal Spikes in Childhood Epilepsies by 3D Sequential Voltage Mapping and Dipole Localisation

Neetha Balaram, James Jose

Neurology, Government Medical College, Kozhikode, Kerala, India

E-mail: neethabalaram@gmail.com

Background and Aim: 1. To study the spatio temporal propagation of occipito-frontal spikes in childhood epilepsies by sequential voltage mapping and dipole localisation. 2. Identify types of occipito-fronto spikes based on onset, propagation and stability of the dipoles. Methodology: Sleep EEG of 10 children with Occipito-frontal spikes;five with Panayiotopoulos syndrome and five with symptomatic epilepsies. 150 manually selected successive individual occipito-frontal spikes selected and using a source localisation software, they were averaged in automated pattern matching with a threshold of 80% and sequential 3D voltage maps of averaged spike analysed. Stability quotient calculated as Total number of Averages/150. Stable dipole defined as Stability-quotient ≥ 0.8. Dipole analysis done by Principal component analysis using age appropriate template head model. Result: 3 types of occipito-frontal spikes identified: 1. Narrow Occipito-frontal spikes with stable dipoles seen in all five patients of Panayiotopoulos syndrome. Apparently ‘synchronous and bilateral’ clone like spikes with short occipito-frontal lag of 10-30 ms and similar rapid propagation pattern from an unilateral medial parietal region to ipsilateral medial frontal region. 2. Wide Occipito –frontal spikes with stable dipoles seen in one symptomatic patient with occipito-frontal lag of 45 ms. 3 Wide Occipito–frontal spikes with unstable dipoles seen in four symptomatic patients, has occipito-frontal lag of >50 ms and complicated propagatory patterns with intraspike and interspike variability. Conclusion: ‘All the way’ propagation from occipital to frontal region is not necessary in an occipito-frontal spike. It is possible to differentiate idiopathic from symptomatic patients by analysing the stability of dipoles and the occipito-frontal lag period.

Award ID: 929

A Case of Paraparesis with Sensorineural Hearing Loss – Venturing Beyond Tuberculosis into Neurobrucellosis

Vikas Prabhu, Imran Rizvi, Neeraj Kumar, Ravindra Kumar Garg

Neurology, KGMU Lucknow, Uttar Pradesh, India

E-mail: vikasneurokgmu@gmail.com

Background and Aim: Neurobrucellosis may develop at any stage of Brucella disease and may have widely variable manifestations. Diagnosis of neurobrucellosis remains elusive and is usually made 2–12 months after the onset of symptoms in most cases. We look at the nuances of a similar presentation. Methodology: We report a case of Neurobrucellosis presenting with paraparesis and hearing loss Result: 36-year-old male patient presented with history of gradually progressive bilateral lower limb weakness of one year duration. Associated with paraesthesias. On further probing, history of decreased hearing for 1 year was also elicited along with an undulating febrile illness. On examination features of myeloradiculopathy were noted. Patient was extensively evaluated. CSF study showed lymphocytosis with elevated protein and decreased glucose and was negative for malignant cells. MRI brain showed leptomeningeal enhancement with focal bilateral signal changes. CBNAAT and VDRL reported negative. PTA and BERA done showed bilateral profound sensorineural hearing loss. On further testing, serum IgM for brucella came to be positive and patient was diagnosed with neurobrucellosis and was managed accordingly. Patient improved significantly and is on follow up with mild residual sequelae. Conclusion: Neurobrucellosis is a deceptive infectious disease in India and needs to be recognised and differentiated from tuberculosis which remains a commonly encountered close counterpart. Hearing loss due to vestibulocochlear nerve involvement, deep grey matter involvement, and extensive white matter lesions on neuroimaging mimicking demyelinating disorders seems to be unique for brucellosis. When the patient presents with undulating fever and neuropsychiatric features, neurobrucellosis should be considered as a differential diagnosis.

Award ID: 930

Revisiting Choreoacanthocytosis – A Mimicker of Huntington’s disease

Vikas Prabhu, Neeraj Kumar, Ravindra Kumar Garg

Neurology, KGMU Lucknow, Uttar Pradesh, India

E-mail: vikasneurokgmu@gmail.com

Background and Aim: Neuroacanthocytosis is a rare neurodegenerative disease characterized by orofaciolingual dyskinesias, involuntary choreiform movements and erythrocyte acanthocytosis. Clinically and radiologically the disease can be mistaken for Huntington’s disease. Methodology: We report a case of Neuroacanthocytosis closely mimicking Huntington’s Result: A 26-year-old male patient presented with history of gradually progressive abnormal choreiform movements since 8 months. He also had orolingual dyskinesias which were associated with tongue and lower lip biting. The movements worsened during stress and improved with sleep. He also had vocal tics in the form of hissing sound which interfered with talking. On examination speech was slurred and hyporeflexia was noted. Patient was extensively evaluated. Workup for Wilsons, vasculitis and rheumatic chorea were negative. Initial peripheral smear done outside did not show acanthocytes. MRI brain showed caudate head atrophy with focal dilatation of the frontal horns. Although no family history elicited, a possibility of Huntington’s was kept. However, Gene study for Huntington’s reported negative. Serum CPK was noted to be elevated and nerve conduction studies done showed sensorimotor polyneuropathy. EEG done showed normal activity. Possibility of Neuroacanthocytosis was reconsidered in accordance with the clinical presentation; peripheral smear study was resent and analysed which showed significant numerous acanthocytes and diagnosis of neuroacanthocytosis was made Conclusion: Neuroacanthocytosis should be considered in case of adult onset chorea with classical associated features. It remains a close mimic to Huntingtons disease. Reliance on clinical acumen and judgement is needed when genetic studies are not possible. Paying close attention to the subtle differentiating features helps identify the condition

Award ID: 931

Decoding the Genetic Mimics of Acquired Demyelinating Disorders

Kshiteeja JAIN, Netravathi M, Maya Bhat, Netravathi M

Neurology, National Institute of Mental Health and Neurosciences,

E-mail: kjain30@gmail.com

Background and Aim: Mitochondrial disorders are rare genetic disorders whose clinical presentation can mimic acquired demyelinating disorders. Methodology: We present a series of 5 cases with distinct imaging features and clinico-genetic correlation in 3 confirmed cases. Result: Case 1: 35-year-old lady presented with event of subacute onset quadriparesis in 2017 and with bladder disturbances, bilateral vision loss and acute onset paraplegia in 2018. Clinical exome sequencing revealed two heterozygous missense variants in exon 5 and exon 13 of the DARS gene (VUS). Case 2: 13-year-old girl presented with relapsing remitting illness since 2018 with episodes of right lower limb weakness, right upper limb weakness, bladder involvement and seizures. Clinical exome sequencing revealed heterozygous pathogenic variant in exon 15 of CSF1R gene. Case 3: 35-year-old lady presented with 6-month history of cognitive decline in 2019. MRI showed T2/FLAIR hyperintensities of bilateral fronto-parietal periventricular and subcortical white matter. Clinical exome sequencing revealed likely pathogenic homozygous variant in exon 4 of AARS2 gene. Case 4: 27-year-old male presented with subacute onset spastic paraparesis with T4 sensory level in 2022. MRI showed T2/ FLAIR hyperintensities in bilateral corticospinal tract, crus cerebri, pons, anterior medulla and cervicodorsal spinal cord. Genetic test results are awaited. Case 5: 33-year-old lady presented with sequential optic neuritis followed by partial myelitis in May 2022 with MRI showing hyperintensity of bilateral optic nerves and bilateral corticospinal tract in cervicodorsal cord. Genetic test results are awaited. Conclusion: The characteristic imaging abnormalities can help in the diagnosis of mitochondrial disorders provided a high degree of suspicion is maintained.

Award ID: 932

A Novel SLC16A2 Gene Mutation: Delayed Myelination and Dysthyroidism

Aakash Mahesan, Abin Sherif, Ankit Kumar Meena, Kaushik Ragunathan, Gautam Kamila, Atin Kumar, Pawan Ghanghoriya, Biswaroop Chakrabarty, Prashant Jauhari, Rachna Seth, Puneet Chaudhary, Sheffali Gulati

Pediatric Neurology, AIIMS

E-mail: aakash191293@gmail.com

Background and Aim: Guillain-Barré syndrome is an acute demyelinating polyneuropathy and IVIG remains a commonly administered treatment. Several risk factors are known to predispose to Posterior reversible encephalopathy syndrome (PRES) which manifest with headache, seizure, altered sensorium and visual disturbances. Some of the rarer known risk factors among them are IVIG therapy and GBS associated dysautonomia. We report a similar case of PRES post IVIG therapy in a patient of GBS. Methodology: We report a case of PRES in a patient of GBS post IVIG therapy Result: A 67-year-old female patient with no prior comorbidities presented with acute onset ascending flaccid areflexic quadriparesis of 5 days duration. Nerve conduction study done showed acute sensorimotor axonal neuropathy. CSF done showed albumin-cytological dissociation. Patient was diagnosed with Guillain Barre Syndrome (AMSAN) and was started on IVIG as per bodyweight for total of 5 days. One day after completion of her IVIG course, patient started complaining of bilateral vision loss and then went on to develop alteration of mental status along with raised blood pressure reading. Neuroimaging done then showed bilateral occipital hypodensities and patient was diagnosed with PRES. Patient was managed accordingly with gradual resolution of symptoms. Conclusion: A high index of suspicion for PRES should be kept in patients of GBS who develop neurological symptoms and signs, especially visual disturbance after IVIG infusion. Interplay as well as independent causation of both GBS and IVIG therapy plays a part in PRES and early recognition of same may prove vital.

Award ID: 933

All in One” -A Rare Case Report Vitamin B12 Deficiency Induced Subacute Combined Degeneration of Cord and Cerebral Venous Thrombosis in a Young Male

Pooja Tripathi, Imran Rizvi, R. K. Garg, Shubham Dubey

Neurology, KGMU Lucknow, Uttar Pradesh, India

E-mail: docpoojachildcare@gmail.com

Background and Aim: We report a case of Vitamin b12 deficiency induced subacute combined degeneration of cord and hyperhomocysteinemia, lead to, cerebral venous thrombosis in a young male. Methodology: Result: 26 yr old male presented with complain of headache along with difficulty in walking since 1 month.neurological examination showed sensory ataxia. His investigations showed anaemia with raised MCV, hyperhomocysteinemia along with low b12 level. MRI with MRV showed sinus thrombosis along with hemorrhagic stroke. MRI spine showed T2 hyper intensity along the posterior aspect of cord in cervical region. Discussion -B12 deficiency presents with hematological, gastrointestinal and neuropsychiatric manifestations. Neuropsychiatric manifestations commonly associated with deficiency include myelopathy, neuropathy, dementia, neuropsychiatric abnormalities. Our case report is very unique as our patient developed SACD and CVT, establishing the fact that hyperhomocysteinemia due to vitamin b12 deficiency is a independent factor to develop CVT. SACD due to vitamin B12 deficiency is a established entity but for CVT, establishing direct causal relationship, there are very sparse literature. 1) Sen A, Chandrasekhar K. Spinal MR imaging in Vitamin B12 deficiency: Case series; differential diagnosis of symmetrical posterior spinal cord lesions. Ann Indian Acad Neurol. 2013 Apr;16(2):255-8. [PMC free article] [PubMed] 2) den Heijer M, Rosendaal FR, Blom HJ, et al. Hyperhomocysteinemia and venous thrombosis: a meta-analysis. Thromb Haemost 1998;80:874–7. Conclusion: Correct diagnosis of Biotinidase deficiency can prevent spinal disability when identified early and treated aggressively.

Award ID: 934

Snake Envenomation and Stroke – Do We Know Enough?

Kshiteeja Jain, Jitender Saini, Netravathi M

Neurology, National Institute of Mental Health and Neurosciences

E-mail: kjain30@gmail.com

Background and Aim: Hemorrhagic strokes are reported to be extremely rare complications of snake envenomation with more severe clinical course and higher mortality rates. Angiographic imaging findings has been described sparingly in these cases. To our knowledge, vessel wall imaging has not been described. Methodology: We describe novel vessel wall imaging findings in a patient with recent vasculotoxic snake bite. Result: 65-year-old gentleman presented with acute onset headache and language disturbances of 2 days duration. He had a history of vasculotoxic snake bite to his left ring finger 3 weeks ago. At the time patient only had local symptoms and received 30 vials of anti-snake venom (ASV) in peripheral hospital. At presentation to our centre, he was conscious, oriented, GCS E4M6V4 with sensory aphasia and no other focal deficits. CT brain was suggestive of left parieto-temporo-occipital hemorrhage with surrounding edema and mass effect. MR venogram showed hypoplastic left transverse and sigmoid sinuses with no evidence of thrombosis. MR vessel wall imaging revealed concentric enhancement in bilateral vertebral V4 segment, basilar artery, bilateral PCA P1/P2, bilateral MCA M1/M2 and terminal internal carotid arteries. Coagulation parameters at admission were normal. Patient was given antiedema measures and symptomatic treatment following which he showed improvement. Conclusion: In presence of normal coagulation parameters, vessel wall imaging may provide additional clues for the presence of a possible toxic vasculitis in patients with vasculotoxic snake bite.

Award ID: 935

Early Post Stroke Seziures Following Acute Ischemic Stroke: A Cross Sectional Study from a Tertiary Care Centre in Kerala

Ann Thomas

Neurology, Govt. TD Medical College, Alapuzha, Kerala, India

E-mail: annpriyathomas@gmail.com

Background and Aim: Background:Stroke is the most common cause of epilepsy in the adult population1. Post-stroke seizures can significantly affect the clinical outcome and duration of hospital stay. Aims and objectives: 1. Clinical profile of patients with early post stroke seizures 2. Asses the risk factors for early post stroke seizures Methodology: Inclusion criteria All consecutive patients who were admitted under neurology department during the study period between June 2020 to May 2022 with acute ischemic stroke (confirmed by CT/MRI) were included in the study. Exclusion criteria All patients with a previous stroke, transient ischemic attacks, hemorrhagic stroke, cerebral venous thrombosis, prior history of seizures, or any other epileptogenic comorbidity. Result: Patients with ES were younger, had a more severe stroke at presentation (NIHSS and mRS), had a cortical location of the infarct, involving the MCA territory, and underwent lesser recanalization procedures (thrombolysis or mechanical thrombectomy). Large artery disease and cardioembolic subtypes were more common in patients with ES. Conclusion: Significant risk factors revealed in our study were cortical location, mRS at admission, anticoagulant use. We seek to determine those individuals who would be at high risk for ES, and therefore, can be given prophylactic antiseizure drugs (ASD) along with the standard of care. By preventing a seizure, we will not only decrease their infarct volume but also reduce the morbidity, mortality and duration of hospital stay for the patient

Award ID: 936

Untangling CNS Demyelination – Experience from a Single Tertiary Care Centre in Southern India

Mandara Ganganakudige Manjappaiah, Kamakshi Dhamija, Kshiteeja Jain, Priya Treesa Thomas, Maya Bhat, Geetha Desai, Anita Mahadevan, Netravathi M

Neurology, National Institute of Mental Health and Neurosciences

E-mail: gmmhebbar123@gmail.com

Background and Aim: Central Nervous System demyelinating disorders (CNS-dd) is a chronic immune mediated condition. We aimed to study the demographic and treatment profile of these patients. Methodology: Prospective study of patients attending the Neurology services at a tertiary University hospital from Southern India. Result: We enrolled 119 patients with CNS-dd from February 2022-July 2022 (6 months). Mean age of patients was 32?10.7 years (2.84-female:male). Multiple Sclerosis (MS) was diagnosed in 83 patients, 19 had AQP4+ve NMOSD (Neuromyelitis Optica Spectrum Disorder) and 17 had MOGAD (Myelin Oligodendrocyte Glycoprotein Antibody Associated Disease). Among the MS patients, 73 were relapsing remitting, 6 were secondary progressive and 4 were primary progressive type. The mean age of onset of symptoms was 26.6?10.7 years. The mean duration between onset of illness and diagnosis was 2.2 (range:5 days-17.5 years) years. The initial presentation was spinal cord involvement (46.2%), followed by optic nerve (41.2%), and 15.1% had brainstem manifestations. The average number of relapses during the disease course was 3.45 (range:0-10). Among the MS patients; the disease modifying agents (DMAs) administered were: Rituximab (57), dimethyl fumarate (29), natalizumab (9), & other DMAs (29). Among the AQP4+ve NMOSD and MOGAD patients, 63.2% and 47.1% received rituximab, respectively; whereas mycophenolate mofetil and azathioprine were the other immunomodulators. Conclusion: Multiple sclerosis was the commonest CNS demyelinating disorder at our Institute. Maintenance of a dedicated clinical database provides a unique opportunity to study the clinical features, phenotypes of CNS demyelinating disorders and optimize outcomes in patient care and management.

Award ID: 937

Mechanical Thrmobectomy in Inflammed Arteries: A Special Case

Kishore Ramachandraiah

Neurology, Narayana Health City

E-mail: kishore.imcore@gmail.com

Background and Aim: Takayasu arteritis is a chronic large vessel inflammatory disease leading to progressive stenosis and pathological occlusion, cerebral ischemia or infarction and other symptoms are well known in supra-aortic occlusion. Steno-occlusive disease especially in the subclavian and carotid arteries, which if untreated may result in hemiplegia or death. Procedures for stenotic lesions can be performed using percutaneous transluminal angioplasty, which is less invasive. Here we report of Takayasu arteritis with left MCA stroke who underwent mechanical thrombectomy and left CCA angioplasty. Methodology: A young female presented with right sided weakness and NIHSS -16. Initial imaging revealed left MCA infarct ASPECTS 7, left MCA occlusion, left CCA occlusion. Underlying aorta-arteritis was considered with asymmetric BP measurement in upper limbs, and evidence of small kidney. Left CCA angioplasty and mechanical thrombectomy was performed. She was started on anti-platelets. Diagnosis of Takayasu arteritis was confirmed from CT aortogram. Steroids and methotrexate were initiated and the further hospital course remained uneventful. Result: MR brain with angiography showed left MCA occlusion, Left CCA occlusion and left ICA faint flow. Conclusion: There are only few cases of Takayasu arteritis where Mechanical thrombectomy has been done. The range of serious complications are inevitable if left untreated.

Award ID: 938

Moyamoya Disease: A Case Series

Tariq Harris, Kishore Rajendran, Rajasekar Meenakshisundaram, Kannan Nithyanantham, Vignesh Karunakaran

Neurology, KAPV GMC, Trichy, Tamil Nadu, India

E-mail: tariqharris7@gmail.com

Background and Aim: Background: Moyamoya disease (MMD) is a chronic progressive cerebrovascular disease characterized by bilateral stenosis or occlusion of the arteries around the circle of Willis with prominent arterial collateral circulation. “Moyamoya” is a Japanese word meaning puff of smoke in the air. The term was used to describe the smoky angiographic appearance of the vascular collateral network. Objective: To profile 2 children diagnosed to have Moyamoya disease. Methodology: Methods: 1st patient is a 4-year-old female child who presented with sudden onset right hemiparesis, aphasia. Child had associated anaemia, was also found to be Covid 19 antibody positive. Imaging MRA showed bilateral internal carotid artery supraclinoid segment occlusion. CT Angiography confirmed the findings. 2nd patient 6 year old male patient presented with sudden onset of weakness of right upper and lower limb, aphasia. There is history of left hemiplegia one year back. Child had multiple café- au- lait spots and evaluated and found to have neurofibromatosis type 1. MRI brain showed diffusion restriction in left frontal lobe, centrum semiovale and corona radiata. MRA showed bilateral internal carotid artery supraclinoid segment occlusion. Result: Results: Both patients were treated with aspirin and supportive care and showed clinical improvement. Conclusion: Conclusion: Association of Moyamoya disease neurofibromatosis 1 has been described, we need to evaluate the role of anaemia, post covid 19 state as risk factors

Award ID: 939

Is There a True Spinal Tap Responder in Progressive Supranuclear Palsy?: The First Prospective Study

Masahiro Ohara, Takaaki Hattori, Takanori Yokota

Department of Neurology and Neurological Science, Tokyo Medical and Dental University, Tokyo, Japan

E-mail: masa.ohara0204@gmail.com

Background and Aim: Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disease, but sometimes shows idiopathic normal pressure hydrocephalus (iNPH)-like presentations (1,2,3). However, there have been no prospective studies to evaluate spinal tap responsiveness including placebo effect (4) in patients with PSP. Methodology: Clinically diagnosed PSP patients and suspected iNPH patients were prospectively enrolled. All patients underwent placebo spinal tap (5) and spinal tap test. Gait was evaluated before and after each test. We defined patients with 10% improvement in any gait parameters (TUG total time, stride length and stride velocity during 15 m straight walking) as “tap responder”. Primary outcome is the ratio of tap responders in PSP patients. Result: Eleven PSP patients, 12 probable iNPH patients and 5 control patients (Alzheimer’s disease, septo-optic dysplasia and cervical spondylosis) were finally evaluated. Seven of the 11 PSP patients, 10 of the 12 probable iNPH patients, and 1 of the 5 control patients responded to spinal tap test. On the contrary, 6 of the 11 PSP patients, 8 of the 12 probable iNPH patients responded to placebo spinal tap test while no control patients responded. A patient who was diagnosed as probable PSP-Richardson syndrome responded to spinal tap test beyond placebo effect and even to shunt operation. Conclusion: This is the first study to prospectively demonstrate that some PSP patients can develop iNPH-like clinical features including spinal tap responsiveness. We also exhibited the usefulness of placebo spinal tap test and PSP patients with “true” spinal tap responsiveness beyond placebo effect can response to shunt-operation.

Award ID: 940

Ring Enhancing Lesion-A Case Report

Sahil Mittal, Gagan Saxena

General Medicine, Rama Medical College, Hapur, Uttar Pradesh, India

E-mail: sahilmittal725@gmail.com

Background and Aim: Multiple ring-enhancing lesions in the brain is difficult to reach the true diagnosis. The causes are many neoplastic, infectious, vascular, inflammatory and demyelinating conditions. The two important causes of multiple ring-enhancing lesions in the brain are neurocysticercosis andtuberculomas in developing countries. This casereport illustrates how multiple ring-enhancing lesions cause a diagnostic-dilemma between neurocysticercosis and tuberculoma. Methodology: We report a case of 39 yrs. Male admitted in RMCH Hapur with c/o fever, headache and difficulty in walking since 3 months, which were gradually progressive, fever in evening, low grade fever a/s with chills and sweating, patient tends to fall towards right side of body, headache a/s with nausea and vomiting. This prevented him from carrying out his dailyactivities and a/s with anger and decreased sleep. Patient is a tobacco chewer and non-vegetarian and eats uncooked pork meat 20 days back initiation of symptoms and chronic alcoholic, non-diabetic, non-hypertensive. Result: GPE:-WNL, NR-absent Pt conscious. oriented and MMSE of 22. Normal cranial nerve except facial, left facial deviation. Ant 2/3 taste sensation lost. Sensory deficit present on right side of face and shoulder, normal power and tone of muscles, normal reflexes Nystagmus + right side, Romberg + towards right, heel shin test +, ataxic gait+ Patient investigation revealed- Normal fundus examination. No cysticerci seen and tubercular nodule. HIV- NR, ESR raised, CSF analysis show glucose-40, micro protein-119, WBC count 05, lymphocyte-100%, tuberculin positive. Lat. X-ray of thigh-no cysticerci. Conclusion: Thus a diagnosis of tuberculoma was made with cerebellar and facial nerve involvement and patient was symptomatically improved by giving Anti-tubercular drugs.

Award ID: 941

A Novel Method for Gait and Tremor Quantification in Parkinson’s Disease Patients

Yeruvena Ashok, Rupam Borgohain, Rukmini Mridula

Neurology, Nizams Institute of Medical Sciences, Hyderabad, Telangana, India

E-mail: ashokyadav.yadav@gmail.com

Background and Aim: Tremor and gait disturbances are one of the key features in Parkinson’s Disease. There is an unmet need to evaluate the complex motor disturbances, especially tremor, gait and balance disturbances, that manifest and progress with the duration of disease. Till date there exists no validated system which can quantify gait and tremor, the values of which can be used clinically to assess patients AIM:To quantify gait and tremor, the values of which can be used clinically to assess patients. Methodology: To analyze the tremor, we used MEMS based triaxial accelerometers attachable to the region of interest, mainly knuckles. And for gait assessment, we used snug fit shoes attached with switches at prefixed points. Data was collected and analyzed offline in MATLAB. Result: The parameters obtained were correlated with the clinically administered scales while the subject is on and off the anti-Parkinson’s medication. The results were also comparable to the literature available as of date. Conclusion: With this proposed instrument, we were successfully able to measure gait and tremor quantitatively. This can be used to analyze other kinds of tremors and gait related disorders quantitatively, after appropriate validation, apart from those pertaining to Parkinson’s disease. The analysis can further help in development of gait rehabilitative devices or as a reminder to take medication, when the effects are wearing off. Also, the tremor device can be used intraoperatively while stimulation the patient at various currents to select the best fit electrode in DBS.

Award ID: 942

Methodology of Development of a Branching Logic Questionnaire and a Computer Based Assessment Tool (COMBAT) for the Diagnosis of Primary Headache Disorders using International Classification of Headache Disorders Third Version (ICHD3)

Vaibhav Deorari, Debashish Chowdhury, Kolin Paul, Pritesh Srivastava, Apoorva Tomar, Ashish Duggal, Vaibhav Deorari, Kolin Paul, Pritesh Srivastava, Apoorva Tomar, Ashish Duggal

Department of Neurology, G B Pant Institute of Post Graduate Medical Education and Research, Maulana Azad Medical College, New Delhi, India

E-mail: vaibhav2498@gmail.com

Background and Aim: A digital tool for the diagnosis of Primary Headache Disorders shall be useful [1,2] though none is available in India. We aimed to develop a branching logic questionnaire-based computerized tool (English and Hindi), COMBAT for the diagnosis of primary headache disorders using ICHD3. Methodology: The development of COMBAT was done in four phases. In phase 1, an assessment of the core diagnostic features of primary headaches was done followed by the development of the questionnaire and the algorithm in phase 2. In phases 3 and 4, validation of the tool was done using different sets of users against the diagnosis provided by two headache experts (gold standard). Result: 250 case records were analyzed for core diagnostic features of primary headaches. We created 15 versions of the COMBAT and improved upon its features with each version and finally included 143 questions. COMBAT provided three levels of output for headache diagnosis for each patient. At Level 1, the patients were diagnosed as having episodic or chronic headaches based on the frequency of headaches. At level 2, output was whether the patient had primary, secondary, or possible secondary headaches based on an assessment of red flags in history and examination. Level 3 provided the final diagnosis of the primary headache (or ‘unclassified headache’ if the criteria were not fulfilled). The validation of COMBAT was done in 770 headache patients. Conclusion: COMBAT, the first digital tool for the diagnosis of primary headaches from India was successfully created.

Award ID: 943

ReCOVer study: A Cross-Sectional Observational Study to Identify the Neuro-Rehabilitation Needs in Post-discharge COVID-19 Survivors

Raktim Swarnakar, Shiv Lal Yadav, Srikumar Venkataraman, Kapil Dev Soni, Richa Aggrawal, Anjan Trikha

Department of Physical Medicine and Rehabilitation (Pmr), All India Institute of Medical Sciences (AIIMS), New Delhi, India

E-mail: raktimswarnakar@hotmail.com

Background and Aim: People recovered from Coronavirus disease-2019 (COVID-19) are now facing multiple health issues including neurological complications [1]. Considering post-COVID scenario, neurorehabilitation need identification is a key unmet need currently [2]. The aim was to evaluate the neurorehabilitation needs in post-discharge COVID-19 survivors by assessing the International Classification of Functioning, Disability, and Health (ICF) data set, COVID-19 Yorkshire Rehab Screen (C19-YRS) tool, Post-COVID-19 Functional Status (PCFS) scale. Methodology: Around 320 screened patients, 90 COVID-19 survivors (minimum one-week post-discharge, irrespective of age and sex) were recruited from COVID Centre data register (tertiary care hospital) through the telerehabilitation facility. Result: Among 90 participants (58% male, 42% female, age: 31 ± 18.2 years) 51% had comorbidities. Post-COVID 58% experienced pain and 67% are still facing mild-moderate functional limitations in PCFS scale. In the ICF data set, cardiopulmonary and neuro-musculoskeletal systems are mostly affected. C19-YRS and ICF data revealed difficulties in activities of daily living in >60% of cases, anxiety-depression in 89% of cases. Conclusion: This is the first study to identify neurorehabilitation needs in post-discharge COVID-19 survivors. Findings suggest that comorbidities influence the severity of long-covid symptoms. Neurological complaints are important problem areas where long-COVID rehabilitation interventions should be prioritized early to prevent functional limitations.

Award ID: 944

Implementation of Visual Evoked Potential for the Detection of Preretinopathy and Prognostication in Diabetes Mellitus: Observational Study in Southern India

Leena Rajani, Sownthariya R, Kannan B, Thomas Edwinraj A

Neurology, Thoothukudi Government Medical College and Hospital, Tamil Nadu, India

E-mail: leena3011rajani@gmail.com

Background and Aim: Diabetic Preretinopathy is one of the leading disabilities caused by Diabetes Mellitus in the age group of 20 to 74 years of age and may be preventable by glycemic control. Diagnosis of retinal changes and determination of prognosis can be done by analysing the pattern of visual evoked potential. In many cases the patient is asymptomatic until its too late for effective treatment. Our Aim of the study was to analyse the potential of visual evoked potential (VEP) in detecting preclinical neurodegenerative changes in patients with diabetic preretinopathy by studying changes in VEP, in terms of P100 wave latency in diabetic patients without retinopathy. Methodology: The study included 40 diabetic patients without retinopathy and 40 non diabetic controls. Patients with History of Hypertension, cataract, glaucoma and optic atrophy were excluded. VEP study was done in subjects and controls and the latency of P100 was analysed. Mean P100 wave latency of both groups were compared and analysis was done checking for any variation in latency of P100 with respect to duration and control of diabetes. Student t test and ANOVA tests were applied. Result: 1) There was significant prolongation of P100 wave latency in Diabetics without Retinopathy. 2) There was positive correlation between prolongation of P100 wave latency and duration of diabetes. Conclusion: 1) Analysis of P100 wave of VEP is helpful in the detection of diabetic Preretinopathy changes. 2) It could be a better tool for detection of very early retinal changes before any clinical evidence of Retinopathy.

Award ID: 945

Exploring the Protective Role of Biliteracy in Alzheimer’s Disease

Snehasree Neogy, Snehasree Neogy, Tanisha Majumdar, Arinjoy Bhattacharjee, Koustav Chatterjee, Supriyo Choudhary, Hrishikesh Kumar, Siddharth Shankar Anand

Department of Neurology, Institute of Neurosciences Kolkata, West Bengal, India

E-mail: snehasree949@gmail.com

Background and Aim: The act of writing involves multifaceted cognitive domains beyond language processing. Studies show that bilingualism (speaking two languages) reduces the progression of dementia in Alzheimers disease (AD). However, the effect of biliteracy (ability to speak, write and read) has been rarely studied in AD. Hence, we took an initiative to examine the effect of biliteracy in clinical outcomes of AD patients. Additionally, we explored the association of biliteracy with subcortical and cortical volumes through neuro imagining studies in a subset of patients. Methodology: In this ongoing cross-sectional study, 30 bilingual AD (12 mono-literate, 18 biliterate) patients underwent cognitive assessments through Montreal Cognitive Assessment (MoCA) and Mattis Dementia Rating Scale (Mattis-DRS). MRI-morphometric analysis was performed on eight AD patients using FreeSurfer Pipeline. Result: We observed that the MoCA and DRS scores (p=<0.05) were significantly less impaired in the biliterate group, particularly in the domains of attention (digit backward), language, conceptualization, construction and memory. However, we did not observe any delay in onset of symptoms in biliterate group in our limited sample cohort. In our preliminary neuroimaging study we found correlation between the volume of cortical and subcortical areas with age of onset. Conclusion: The findings of the current study reflect a higher cognitive reserve in biliterate AD patients suggesting its protective role in the disease. This ongoing study demonstrated cortical and subcortical correlates of onset of dementia symptoms in AD patients. Adequately powered study is underway to achieve a higher confidence.

Award ID: 946

Efficacy of Michigan Neuropathy Screening Instrument and Nerve Conduction Studies for Diagnosis of Diabetic Distal Symmetric Polyneuropathy: Observational Study in Southern India

Leena Rajani, Sownthariya R, Kannan B, Thomas Edwinraj A

Neurology, Thoothukudi Government Medical College and Hospital, Tamil Nadu, India

E-mail: leena3011rajani@gmail.com

Background and Aim: Distal Symmetric Polyneuropathy (DSP) is the most frequent type of Neuropathy found in association with diabetes. About 50% of patients with diabetes develop disease during progression and 20% present symptoms of DSP at diagnosis. The aim of the present study was to assess the prevalence of DSP in our cohort and to characterise it depending on glycemic control plus to find an easy method for detection of DSP which could be used more effectively. Methodology: We performed a cross sectional study in Government Thoothukudi Medical College and Hospital,(Tamilnadu) during the time period from 1 January 2022 to 30 April 2022. 100 patients of Diabetes Mellitus were enrolled in the study and evaluation was done consisting of Clinical Examination, Michigan Neuropathy Screening Instrument (MNSI) and Nerve conduction studies (NCS). Inclusion criteria- 65 males and 35 females with Diabetes Mellitus of age group 45 -85 years were enrolled in the study. Exclusion Criteria- Patients with Chronic Alcohol Abuse, Neurotoxic drugs, Chronic kidney disease, Vitamin B12 deficiency, Hepatic dysfunction and Hematological diseases were excluded. Statistical analysis was done using t test and Regression Analysis. Result: (1) Among the patient Investigated 85% had DSP. (2) 65% of them had poor glycemic control (HbA1C>7%) (3) Mean MNSI score was 2.5 (4) Motor NCS revealed longer distal latencies and reduction in amplitude and sensory NCS showed reduction in amplitude. Conclusion: MNSI is a simple validated diagnostic tool and it has got strong correlation to electrophysiological parameters. Daily implementation in practice could help identify and follow patients at risk for DSP.

Award ID: 947

DWI-Aspects – ASL Mismatch for Selecting Patients for Endovascular Therapy in Late Window Strokes

Sameer Arora, Dhananjay Gupta, Devasheesh Kamra, Tariq Matin, Anurag Gupta

Consultant Neurologist, Narayana Superspeciality Hospital

E-mail: dr.samarora@gmail.com

Background and Aim: A number of methods like diffusion-flair (DWI-FLAIR) mismatch, DWI-PWI mismatch and CT perfusion imaging have been previously described to help in patient selection for revascularization therapy (endovascular mechanical thrombectomy) for patients with acute stroke who present in the late window (6-24 hours). Arterial spin labelling (ASL) is a non-invasive, non-contrast MRI based technique which indicates the perfusion deficit in the ischemic area. To retrospectively analyze the outcomes of late window stroke patients, who were selected for endovascular therapy based on mismatch between DWI-ASPECTS and ASL on MRI imaging. Methodology: 20 patients with acute stroke with late window presentation (6-24 hours from the last known normal), underwent MRI based stroke imaging, including diffusion imaging (DWI), ADC maps, FLAIR imaging, ASL maps and MRI angiography. 14 patients with large or medium vessel occlusion mismatch between DWI-ASPECTS and ASL maps (larger area of hypoperfusion shown by ASL compared to infarct core demonstrated by DWI) were selected for endovascular revascularization therapy. Result: The mean age of patients was 53.4 ± 15.2 years. Mechanical thrombectomy resulted in TICI 2b- 3 reperfusion in all patients. There were no major intra-operative complications. 10 patients showed significant clinical improvement in the immediate post-thrombectomy period with decrease in NIHSS score within first 3 days. A modified mRS score of ≤2 was achieved in 7 of the 14 patients at 3 months follow-up. Conclusion: ASL is a non-invasive, non-contrast MRI technique which can help in patient selection for endovascular therapy in acute strokes with late presentation.

Award ID: 948

Indian Natalizumab Study

Thomas Mathew, Indian Natalizumab Study Group INSG

Neurology, St.John’s Medical College and Hospital Bangalore, Karnataka, India

E-mail: chakkuthom@hotmail.com

Background and Aim: Natalizumab (NTZ) is increasingly being used in Indian multiple sclerosis (MS) patients. There are no reports on its safety and efficacy from India. The aim of this study is to describe the patient characteristics, treatment outcomes, and adverse events, especially the occurrence of progressive multifocal leukoencephalopathy (PML) in NTZ-treated patients. Methodology: A multicentre ambispective study was conducted across 18 centres, from January 2012 to December 2021. Patients ≥ 18 years treated with NTZ were included. Descriptive and comparative statistics were applied to analyse data. Result: During the study period of 9 years, 116 patients were treated with NTZ. Mean age of the cohort was 35.6 ± 9.7 years; 83/116 (71.6%) were females. Relapse rate for the entire cohort in the year before NTZ therapy was 3.1 ± 1.51 while one year after was 0.20 ± 0.57 (p = 0.001; CI 2.45 -3.35). EDSS of the entire cohort in the year before NTZ was 4.5 ± 1.94 and one year after was 3.8 ± 2.7 (p = 0.013; CI 0.16–1.36). At last follow up (38.3 ± 22.78 months) there were no cases of PML identified. Conclusion: NTZ is highly effective and safe in Indian MS patients, with no cases of PML identified at last follow up. A short course of natalizumab and lack of prior exposure to immunosuppressant therapy may be the reason for the lack of PML in the present study. Initiating NTZ early, before other immunosuppressive medications and keeping the duration of treatment shorter is important for optimum safety and efficacy.

Award ID: 949

Medication Satisfaction Survey in a Unique Cohort PD Patients Shifted from L-dopa + Carbidopa to L-dopa +Benserazide

Krisnendu Das, Tanisha Majumdar, Avanteeka Ganguly, Supriyo Choudhury, Hrishikesh Kumar

Neurology, Institute of Neurosciences Kolkata, West Bengal, India

E-mail: krishnendas96@gmail.com

Background and Aim: Fixed dose combinations (FDC) of l-dopa and carbidopa (LC) are arguably the most widely prescribed drug in treating patients with Parkinson’s disease (PD). Benserazide is an alternative drug to carbidopa but less frequently used than LC. Levodopa pharmacokinetics differ after administration of LB and LC, but its clinical significance is unclear. Recently, due to unavailability of l-dopa and benserazide (LB) combination we identified a unique cohort where PD patients were on a stable dose of LB but currently shifted to LC. In the current study we compared the medication-satisfaction between LB and LC. Methodology: On reviewing the outpatient records for last 5 months, we observed that 89 PD patients were on stable dose of LB but switched to LC due unavailability of the former combination. We interviewed consecutive 39 patients from this cohort over telephone based on a validated medication satisfaction questionnaire. Result: Out of 39 patients, 28 (71%) reported that LB was superior in treating their condition compared to LC, 10 (25.6%) patients reported that LB and LC were equivalent and only 1 (2.5%) patient reported that LC is superior to LB. Out of 39 patients, 17 (43.6%) reported some Adverse Drug Reaction to LC. Conclusion: We observed that most of the patients were relatively more satisfied with LB and they faced challenges due to the unavailability of the medication. The result should be interpreted with caution, as some of the patients in this cohort were initially prescribed LB due to unresponsiveness to LC.

Award ID: 950

Impaired Finger Individuation in Patients with Parkinson’s Disease

Asit Bayen, Supriyo Choudhury, Suchismita Majumder, Stuart Maitland, Mark Roger Baker, Stuart Nicolas Baker, Hrishikesh Kumar

Department of Neurology, Institute of Neurosciences Kolkata, West Bengal, India

E-mail: asitbaranbayen@gmail.com

Background and Aim: BACKGROUNDPyruvate dehydrogenase (PDH) deficiency is a disorder of energy metabolism withvariable clinical presentations, ranging from severe infantile lactic acidosis to milder chronicneurological disorders. The pyruvate dehydrogenase (PDH) complex is a mitochondrial multienzyme that catalyses the irreversible oxidative decarboxylation of pyruvate to acetyl-CoA and is thus important in energy metabolism. AIMS To review the clinical, biochemical, radiologic and genetic profile of patients with PDH deficiency Methodology: Retrospective analysis of patients with Pyruvate Dehydrogenase Deficiency was done in Pediatric Neurology Division of our Institute Result: We report 4 patients with PyruvateDehydrogenase (PDH) enzyme deficiency. Among the 4 patients, 2 were males. Age of onset of illness was observed during the infantile period. Varying clinical phenotypes observed were Epileptic encephalopathy, recurrent ataxia, generalised chorea with dystonia. The prominent phenotype was episodic decompensation during febrile episodes. Lactate levels were uniformly elevated with normal lactate pyruvate ratio. Varying radiological presentation was noted which included ventriculomegaly, thinned out corpus callosum and hyperintensities in b/l dentate nucleus of cerebellum and basal ganglia. As per ACMGE classification, pathogenic variants, likely pathogenic and variants of unknown significance were observed in 1,1 and 2 patients respectively. Target gene identified was PDHA1. The 2 mutations identified as Variants of unknown significance (VOUS) were novel mutations as they had phenotype genotype match. Among the mutations 2 deletions, 1 insertion and 1 misense variants were detected. Patients were initiated on thiamine and ketogenic diet and showed response on follow up Conclusion: Th genotype-phenotypic spectrum of PDH deficiency is expanding. High index of clinical suspicion will help in arriving at this potentially treatable entity

Award ID: 951

A Study of Clinical and Radiological Features of NMOSD in the Indian Pediatric Population

Konika Bansal

Consultant Pediatric Neurologist, Vivekanand Polyclinic Institute of Medical Sciences

E-mail: konikabansal2@gmail.com

Background and Aim: Neuromyelitis Optica Spectrum Disorder (NMOSD) is an inflammatory autoimmune disease of the central nervous system (CNS), primarily affecting the optic nerves and spinal cord. The classic presentation is optic neuritis and transverse myelitis. This is mostly triggered by autoantibodies against aquaporin 4 (AQP4) and MOG (myelin oligodendrocyte glycoprotein). Methodology: This is a retrospective study of 14 patients diagnosed with NMO/ NMOSD with or without NMO antibody positivity. All indoor and outdoor patients presented with demyelination on MRI are included in the study fulfilling the diagnostic criteria published by The International Panel for NMO Diagnosis (IPND) in 2015 and after exclusion of alternate diagnosis. Result: Out of 14 patients, 9 patients (66.6%) were girls and 5 patients (33.4%) were boys. The mean age of onset was 8.1 ± 4 years. The most common presentation was gait disturbance (64.2%%) with hemiparesis (43.2%) and paraparesis (21%) and vision impairment (35.7%). 28.5% (4/14) patients were positive for anti NMO antibody. 6/14 (42.8%) were anti MOG antibody positive. Only 3/15 patients had high CSF proteins and 6/15 had high CSF IgG. 9/14 patients had pleocytosis with lymphocyte predominance in CSF while 5/14 patients had no cells. All 5 patients presenting with vision impairment had abnormal VEP. 5/9 patients presenting with features of myelitis showed spinal cord lesions MRI. Conclusion: NMOSD is an inflammatory CNS infection with female predominance. The response to immunosuppressive drug therapy is good. The risk of recurrence is higher with serum positivity either with NMO or MOG antibody hence, requires long term follow up and preventive therapy.

Award ID: 952

Do People with Parkinson’s Disease Fall More than the General Population? A Case Control Study of Possible Risk Factors for Falls

Charulata Sankhla, Karan Sankhla, Mahendra Thakre

Department of Neurology, P D Hinduja National Hospital

E-mail: charusankhla@gmail.com

Background and Aim: To determine the frequency and risk factors associated with falling in Parkinson’s Disease patients. Settings and Design: Case control retrospective observational cohort study. The study was conducted in the Movement disorders clinic of tertiary care hospital Methodology: A total of 121 PD patients in the age group of >18 years were included between July 2018 and June 2019. Age and sex matched (n = 123) normal healthy persons were also studied for frequency of falls. UPDRS Motor part III, Postural Instability, Freezing of Gait Questionnaire (FOG-Q), Schwab and England activities of daily living, Hoehn and Yahr staging, Tinetti’ Balance and gait test, Timed get-up-and-go test performed were assessed. Statistical analysis used: The χ2 test was used to assess associations between categorical variables. logistic regression analyses used to determine independent variables. Receiver operating characteristic analyses was performed to assess the sensitivity and specificity. Result: Seventy-one PD patients (58.68%) reported at least one fall, and 40 of these patients were recurrent fallers (33.05% of patients with falls). In controls there were 24 falls (19.5%) and only 2 (1.6%) recurrent falls. Conclusion: PD patients tend to fall almost 3 times more than normal healthy control. Higher Timed Up and Go test score (≥11.88 seconds) is independent risk factor for falls, while advanced age (≥60 years), akinetic rigid subtype at onset, symptomatic orthostasis, Modified Hoehn and Yahr advanced disease stage (≥3), lower Tinetti functional total score (≤ 18) are the independent risk factors for recurrent falls.

Award ID: 953

Effect of Yoga as an Adjunct Therapy in People with Long Term Epilepsy: A Randomized Control Trial

Divyasiny Sharma, Balaji Patil, Hitav Someshwar

Student and Yoga Teacher, Epilepsy Foundation India

E-mail: divyasinysharma@gmail.com

Background and Aim: Epilepsy is a disorder in which recurrent seizures are caused by abnormal electrical discharges in the brain. As there are many Non-pharmacological remedies tried to evaluate their impact on the Epilepsy and Seizure and on Quality Life of People with Epilepsy. However, there is no conclusive data has been published to establish the positive impact of non-pharmacological intervention on Epilepsy or related quality of life. As there is lack of randomized trials on effect of Yoga on Epilepsy, we designed this study to evaluate the effect of Yoga on EEG, WHOQOL-brief and seizure frequency. Methodology: This is prospective, single centre, Double blinded randomized control trial, and the individual assessing the pre and post values of the patients is blinded to which group the patient belongs and at the same time the yoga instructor is blinded to the pre and post assessment values of the subjects. Total 50 patients will be randomized in to interventional and control group in 1:1 ratio. WHOQOL BREF, Seizure frequency, EEG changes will be the outcome measures seen Pre inclusion, after 3 months and after 6 months for the subjects based on inclusion exclusion criteria. Result: results will be presented during the conference. Conclusion: If effective, yoga may be used as an adjunct to pharmacotherapy for treatment of non-refractory epilepsy. Conclusion: This study will put light on the benefits of Yoga if any improvement is seen in quality of life, EEG findings and seizure frequency.

Award ID: 954

Identifying the Vulnerable Population in Epilepsy

H. R. Radhakrishna

Consultant Neurophysician, Medicover Hospital, Hyderabad, Telangana, India

E-mail: dr.hariradhakrishna@gmail.com

Background and Aim: Epilepsy is a disease associated with significant disability and social stigma. This problem is more severe in the villages. A study of epileptic subjects in the villages across one district in Andhra Pradesh state was taken up and followed for nearly five years from 2013. Methodology: The study consisted of visiting 55 remote interior villages of West Godavari district, once in 2 months, identifying the epileptic subjects, confirming from semiology and investigations (wherever available), counselling them, and prescribing affordable antiseizure medications. Result: A total of 323 patients were studied. Nearly 35 subjects died during the follow up due to drowning, burns, or accidental injuries. Some migrated to different villages on account of marriage, employment or family problems. A few cases of Tuberous sclerosis, Sturge-Weber’s syndrome, Lennox- Gastaut syndrome were identified. Such patients and parents were counselled regarding long term prognosis, and precautions to be taken. There was resistance from the family members for surgical options, getting the subjects married or sometimes even to identify the long term nature of the disease. Though public is accepting that epilepsy is not contagious, they refuse to interact with epilepsy patients for different reasons. Treatment gap is high preventing access to effective antiseizure medication. Discussion: Rearing an epilepsy subject is a long term commitment requiring patience, time and finances from sympathetic attendants, mostly family members. Conclusion: Long term follow up is necessary by sympathetic doctors to educate, counsel and manage epilepsy subjects, preferably with support of an organization.

Award ID: 955

PARK-EASE Trial: A Double-Blind, Randomized, Controlled Trial to Study the Efficacy of Exercises in Early-Stage Parkinson’s Disease

Raktim Swarnakar, Sanjay Wadhwa, Srikumar V, Vinay Goyal, Sreenivas V

Department of Physical Medicine and Rehabilitation (PMR), All India Institute of Medical Sciences (AIIMS), New Delhi, India

E-mail: raktimswarnakar@hotmail.com

Background and Aim: Exercises as neurorehabilitative interventions have neuroplastic effects. The key unmet need in Parkinson’s Disease (PD) research is to address those areas which can delay the disease progression or modify it. Methodology: To evaluate the efficacy of exercises in early-stage PD by assessing Unified PD Rating Scale (UPDRS) III (motor), UPDRS-I (mentation, behaviour, and mood), UPDRS-II and VI (Schwab and England ADL Scale) at baseline, 4-weeks, 8-weeks, and 12-weeks and PDQL (Parkinson’s Disease Quality of Life) at baseline and 12-weeks. Design: Double-blind, randomized, controlled trial Setting: Tertiary rehabilitation care centre Participants: Forty individuals (≥18 years) with newly-diagnosed PD (Hoehn and Yahr stage ≤ 2) on a stable dose of PD medications were randomized to the intervention group (structured exercises) and control group. Result: : The intervention group showed statistically between-group significant (P <0.05) improvement in UPDRS-I (P = 0.035; minimal clinically important differences [MCID] >2 points), UPDRS-III (P = 0.023; MCID >6 points) at 12 weeks, and in UPDRS-VI at 8 weeks (P = 0.004) and 12 weeks (P < 0.001) but PDQL had no between-group significant results statistically. PDQL showed improvement in mean scores in intervention group. Conclusion: This is the first trial to study effectiveness of structured exercises in early-stage PD. Structured exercises are efficacious in improving the ADL, motor symptoms, and mentation in early-stage PD. Exercises start to show improvement when they are properly followed for at least 3 months. Exercises should be started as an early neurorehabilitation intervention in the early stages of PD soon after the diagnosis

Award ID: 956

Quality of Life in Persons with Spinal Cord Injury and Close Person of Spinal Cord Injured

Deval Pathak, Loganathan S

Physical Therapist, Jaya Rehab Institute & Research Center, Gujarat, India

E-mail: devalpathak2018@gmail.com

Background and Aim: 1. To investigate the changes in quality of life (QOL) in persons with spinal cord injury (SCI) and their close persons during the first 2 years post injury. 2. To compare QoL between with spinal cord injury (SCI) and their close persons Methodology: Longitudinal multiple wave panel design. Data included 55 patients recruited from Kutch district Gujarat and 50 of their close persons. Questionnaire was administered at 12 weeks 6 months 1 year and 2 years after injury to both samples. Quality of life was assessed using the (WHOQOL-BREF, Gujarati version). Result: WHOQOL-BREF domains in individuals with SCI and found differences mostly in the physical domain indicating that QOL increases for persons with SCI from onset. compared individuals with SCI to their close persons and found differences in the physical environmental and social domains over time. The scores on the psychological dimension did not significantly differ between the persons with SCI and their close persons over time. Conclusion: QOL measured by the WHOQOL-BREF shows that QOL changes during rehabilitation and after discharge. Apart from the physical dimension the persons with SCI and their close persons seem to experience a similar change in QOL. Further longitudinal research is suggested to clarify the mutual adjustment process of people with SCI and their close persons

Award ID: 957

Subclinical Hypotonia of Forearm muscles in the Unaffected Side in Chronic Hemiplegic Stroke - Objective Measurement with a ‘Myotonometer’

Supriyo Choudhury, Mark Baker, Sattwika Banerjee, Stuart Baker, Hrishikesh Kumar

Institute of Neurosciences Kolkata, West Bengal, India

E-mail: choudhurydrsupriyo@gmail.com

Background and Aim: Hypertonia in the affected side is a common consequence of hemiplegic stroke. Reorganisation of descending motor tracts with bilateral innervation is generally accepted as the cause for this change. There is evidence for this bilateral post-stroke reorganisation from physiological studies on the contralateral ‘unaffected’ side; these have shown reduced average grip strength, reduced stretch reflex threshold and absent beta-band cortico-muscular coherence. Methodology: We screened patients who had partially recovered from stroke at least six months before the date of assessment. Patients taking prescription muscle relaxants were excluded. In addition to the clinical assessment (Modified Ashworth Score) of both upper limbs, a precise estimation of forearm muscle tone was performed using a motorised robotic arm, which passively moved the semi-pronated hand while recording the applied torque and position data through integrated sensors. Result: Out of 95 stroke patients, 26 patients were selected for the study and 21 age matched healthy participants were recruited for comparative analysis. We observed a linear trend of agreement between the clinical and derived index of muscle tone in the affected limb, up to the point at which passive movement became difficult. Interestingly, the average calculated index of muscle tone in forearm muscles of the ‘unaffected’ side, derived from the instrumented robotic device, was reduced, consistent with hypotonia. Conclusion: This is the first objective evidence of subclinical hypotonia in unaffected limbs of stroke survivors. The pathophysiological basis is unknown but speculatively could be a mix of central and peripheral mechanisms.

Award ID: 958

Virtual Reality and Cognitive Rehabilitation: An Experimental Study

Deepa Sundareswaran

Faculty of Occupational Therapy, Meenakshi Academy of Higher Education & Research, Chennai, Tamil Nadu, India

E-mail: otdeepa@gmail.com

Background and Aim: Virtual Reality (VR) can be viewed as an advanced form of computer interface that allows user to interact with and become immersed in a computer generated environment. The study aimed at identifying the effect of specific virtual reality in improving cognitive skills, functional performance of persons with cerebro vascular accident Methodology: Experimental study design where persons with neurological disorders who had cognitive impairment were included for this study. Ethical consent and institutional review board approval was sought. The study was undertaken after receiving informed consent TOOLS: Lowenstein Occupational Therapy Cognitive Assessment Tool and Occupational Performance measure. Pre test was administered. Intervention protocol included specific functional tasks and exer games through use of immersive technology. Each session lasted for 45 minutes. The duration Of training was 3 months. The sessions were held every alternate days of a week by a trained clinical therapist. Result: A total of 60 participants took part in the study. There were 35 men and 25 women in the study. There was significant improvement in areas of Praxis, Visuo spatial skills and thinking operations. Improvement in thinking operations was better in younger age group than higher age group. There was minimal improvement in visuo spatial abilities. Overall improvement noticed in functional performance following training using VR. Conclusion: Virtual reality facilitates significant interest and motivation in persons with stroke. Specific training using virtual reality improves cognitive functioning and functional performance. Virtual reality serves as promising technology in cognitive rehabilitation by occupational therapists.

Award ID: 959

Rehabilitating Persons with Aphasia (PWA), along with Empirical Research Based on Data Derived from Those Activities

Apporva Pauranik

Neurology, PauranikAcademy of Medical Education, Indore, Madhya Pradesh, India

E-mail: apauranik@gmail.com

Background and Aim: Many activities have been undertaken for rehabilitating persons with Aphasia (PWA), along with empirical research based on data derived from those activities. Methodology and Result: 1. Assessment of burden on Significant Others (SO) of PWA. 2. A survey of Supportive Communication Strategies (SCS) used by PWA and SO to facilitate communication. 3. Development of a module in Hindi for “Communication Partner Training” targeted to Health Care Professionals and SOs, as a part of an international collaborative effort. 4. Hindi adoption of LEAP-Q (Language Experience and Proficiency Questionnaire). 5. A Hindi/English bilingual digital application for Assessment of Handedness. 6. A Hindi-English bilingual digital version of a large compendium of Home-Based Exercises for PWA and SOs. 7. An online card-matching game (Shabd-Chitra) for Intensive Language Action Therapy in a Group setting. 8. Creation of a large corpus of education literature in Hindi and English for doctors, the general public, PWA and SOs. 9. Creation of standardised and validated tools for assessment of Aphasia and Dyslexia in Hindi. 10. City Based meetings (Gwalior, Jabalpur, Raipur, Bilaspur, Ahmedabad, Udaipur, Kota) with three activities at each venue a. Clinical case presentations by RMOs about the examination of a patient with aphasia b. Joint Forum between Speech-Language Pathologists, PWA and SOs. c. CME for Doctors of that City 11. Organised an Expert Group meeting on aphasia for the Indian Academy of Neurology. 12. Guest Edited a special supplement on Aphasia for Annals of the Indian Academy of Neurology. Conclusion: We have described many activities which have been undertaken for rehabilitating PWA.

Award ID: 960

Facial Emotion Recognition Database: An Artificial Intelligence Based 3D Avatars of 99 Faces of Indian Origin

Gowthami Nair, Soumya Sundaram, Uttama Lahiri, Raviprasad Varma, Sruthi S. Nair, Pragya Verma

Department of Neurology, Sree Chitra Tirunal Institute for Medical sciences and Technology, Thiruvananthapuram, Kerala, India

E-mail: gauthaminair.13@gmail.com

Background and Aim: Recognising emotions from facial expressions is an important aspect in any form of human communication and social interactions. Facial emotion recognition is defective in many neuropsychiatric disorders including schizophrenia and autism. Lack of freely available Indian facial emotion database and ethical concerns of using photographs of persons prompted us to create a database of 3D Indian face avatars expressing various emotions. Methodology: For the 3D avatar creation, consenting participants of Indian origin were photographed in a standard setting and this was uploaded to the FaceGen Modeller Pro 3.29 software. Utilising the Facial Action Coding System (FACS), an artificial intelligence based application for discerning facial movements, the images were modified to display 7 basic emotions (happy, sad, anger, fear, disgust, surprise and neutral). Item content validity index (I-CVI) based on modified kappa (k) and content validity ratio (CVR) using the Lawshe method was calculated. Items with k > 0.74 (excellent) and CVR > 0.99 were included in the final database. Result: 19 participants of age group 22 to 50 years, with a male to female ratio of 11:9 were photographed, generating 133 3D avatars of various emotions. Content validation by 6 experts in the field of cognitive sciences was conducted. 99 faces were found have k > 0.74 and CVR > 0.99 which was included in the database. Conclusion: This facial emotion database was created for the free use for researchers working in the field of cognitive sciences especially in neurological and neuropsychiatric disorders and has both clinical and research applications.

Award ID: 961

Acute Hyperkinetic Movement Disorder and Mass Lesion in MRI Brain: Unusual Presentation of SSPE

Goyal Divyam

Medical student, Saraswati Institute of Medical Sciences, Hapur. Consultant, Institute of Neurosciences, Medanta, Gurugram. Assistant Professor, Department of Neurology, AIIMS, New Delhi, India

E-mail: gauthaminair.13@gmail.com

Background and Aim: Subacute Sclerosing panencephalitis (SSPE) is a relentlessly progressive neurological disorder caused by persistent mutant measles virus infection of the brain characterised by behavioural disturbances, generalised periodic slow myoclonus progressing to stupor and coma culminating in death in a mean period of 4 years. Methodology: This 11 year boy, without any comorbidities, non-consanguineous parentage, presented with history of asymmetrical onset choreoballistic movements interfering with the activities of daily living along with speech arrest with preserved comprehension. On examination patient was able to follow commands and respond to yes/ no questions. There were continuous choreoballistic movements on both sides (right > left with right sided spasticity and extensor plantar. Later in course he developed classical asymmetrical slow myoclonus. Result: Routine biochemical and haematological parameters were within normal limits. Initial MRI Brain was T2 / FLAIR hyperintensity involving the left external capsule, insula and parasagittal area without contrast enhancement. ANA, Autoimmune, paraneoplastic, ENA profile and Wilsons workup was negative. CSF analysis was within normal limits. EEG showed intermittent generalised delta activity with normal background. Initially, he was treated with streoids with no responce. Plamapheresis was also tried. Later in course, repeat MRI brain showed increase Hyperintense T2/ FLAIR signal involving left frontal cortical subcortical, Peeriventricular, deep white matter with gyral swelling with extension in to the right frontal region. Brain biopsy showed encephalitis vs demyelination without any neuronal inclusions. Clinically patients condition evolved and he developed classical slow myoclonic jerks. Subsequently repeat EEG showed classical rademacker complexes with slowing of background (6-7 hz). On follow up, CSF measles IIG was sent which came out to be positive. Patient was started on Isoprinosine and weekly interferon alpha 2 b (6 million units weekly) Patient has received 3 doses of interferon. Conclusion: Unilateral onset and acute chore ballistic movement at presentation in SSPE is uncommon. Initial MRI and EEG were nonspecific which delayed diagnosis. A typical presentation with fulminant course is well known in SSSP and a high degree of suspicion is required in patients with acute onset hyperkinetic disorder.

Award ID: 962

Chronic Inflammatory Demyelinating Neuropathy: A Comparative Study on Magnetic Resonance Neurography And High-Resolution Nerve Ultrasound

Jayanth Shimoga Shanthakumar

Senior Resident, Department of Neurology, NIMHANS, Bengaluru, Karnataka, India

E-mail: jayanthss98@gmail.com

Background and Aim: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a demyelinating disease of the peripheral nerves, including sensory and motor nerves (1). High-resolution ultrasound (HRUS) is an emerging, cost-effective, complementary diagnostic tool in CIDP. HRUS has been found to show multifocal, non-homogenous enlargement of peripheral nerves, brachial and lumbosacral plexus, and cervical nerve roots as well as distinct echo-intensity patterns and focal fascicular involvement which may help in differentiating it from other inherited and acquired neuropathies (2–7). HRUS is especially useful in establishing the diagnosis in patients in whom electrodiagnostic features are elusive (8). MR Neurography has been reported as clinically valuable to evaluate pathological conditions of the peripheral nerves (9). However, enlargement of proximal segments of peripheral nerves or gadolinium enhancement of the brachial or lumbosacral plexus in magnetic resonance neurography (MRN) is only a supportive criterion for CIDP diagnosis according to the European Federation of Neurological Societies ‘guidelines (level C recommendation)(1). A comparative study of HRUS and MRN showed a fair degree of correlation of CSA of all nerves/ nerve segments between the two methods. CSA in HRUS also correlated with signal intensity increase and fractional anisotropy on diffusion tensor imaging (10). CSA also showed an inverse relation with conduction velocity implying a higher degree of demyelination in those nerves. Inexcitable nerves also showed a higher CSA implying secondary axonal damage rather than primary axonal damage (10). Before these neuroimaging techniques can be introduced into everyday practice, studies comparing their imaging potential and their correlation with clinical parameters, as well as with electrophysiological parameters, and with the age and sex-related controls, as the ‘gold standard’ are crucial. To systematically compare High-Resolution Ultrasound (HRUS) and MR Neurography (MRN) findings in patients with CIDP Methodology: It was a prospective, observational, cross-sectional, monocentric study with an estimated duration of 24 months to be done at the Department of Neurology, NIMHANS. The locally appointed ethics committee of the Nimhans reviewed and approved this study and its research protocol. We obtained written informed consent from all included subjects. We enrolled 20 patients with features of typical CIDP who visited our hospital either on an outpatient or inpatient basis between April 2020 till April 2022. All the patients between the age group of 18 to 70 fulfilled the typical CIDP (EFNS/PNS criteria)(11). We excluded patients with paraproteinemia, vasculitic neuropathies, other causes of neuropathies, inherited neuropathies, and variants of CIDP. All the patients underwent the required ancillary investigations, including laboratory testing and NCS. Muscle strength was assessed using a previously described protocol based on the Medical Research Council scale (3,12). MRC Sum score and INCAT disability score were calculated for all the patients. All the patients underwent MR Neurography and High-Resolution Ultrasound of the brachial plexus. Magnetic resonance imaging studies of the brachial plexus were performed using a 3-Tesla system (Philips, Best, The Netherlands)(13). We used a protocol that consisted of T1-weighted spin-echo images, fat-suppressed coronal T2-weighted short tau inversion recovery images and sagittal T2-weighted spin-echo images bilaterally (13,14). MRIs were assessed by a single neuroradiologist. High-resolution ultrasound studies of the brachial plexus trunks were performed bilaterally according to a previously described protocol (3,12) with a Philips iU22 (Philips Medical Instruments, Bothell, WA, USA) and 5–17-MHz linear array transducer. Trunks were identified at the supraclavicular region and scanned along their length up to root level, and nerve size (cross-sectional area) was obtained in the anatomical space between scalene muscles on transverse images using the ellipse tool (15–17). The following upper limit of normal for nerve size was based on disease controls (3): cross-sectional area of any trunk of brachial plexus > 8 mm2. Result: Clinical Characteristics A total of 20 patients participated in the study. Among them, 13 were male and 7 were female. The mean age of patients was 39.75 years. 60% of the patients were in the age group between 18-and 39 years. The mean duration of progression of symptoms was 4.3 months. MRC sum score was in the range of 28 to 58, the maximum score is 60. The mean INCAT disability score was 5.65. All the characteristics are summarized in Table 1. Nerve conduction studies All the patients satisfied the electrophysiological criteria (EFNS/PNS criteria) for CIDP. Distal latencies were prolonged in all the patients. F wave latencies were prolonged in 50% of the patients and absent in the others. Brachial plexus imaging Magnetic resonance imaging showed enlargement and/or a pathological T2-hyperintense signal of the brachial plexus in 18/20 patients with CIDP. The mean cross-sectional area of all the trunks of the brachial plexus measured in MRI was 0.1735 cm2. Brachial plexus sonography High-resolution ultrasound showed enlargement and/or a pathological T2-hyperintense signal of the brachial plexus in 18/20 patients with CIDP. The mean cross-sectional area of all the trunks of the brachial plexus measured in high-resolution ultrasound was 0.1668 cm2. Combined analyses: brachial plexus magnetic resonance imaging and sonography The mean cross-sectional area of the brachial plexus in MR Neurography and High-Resolution Ultrasonography was 0.1735 cm2 and 0.1668 cm2. The students t-test was -0.006. The confidence interval was -0.021 to 0.008. P-value was 0.3385. Applying both MRI and HRUS identified thickness of brachial plexus in 18/20 (90%) patients with CIDP, further enhancing the diagnostic performance of neuroimaging and indicating that they could be used as complementary adjunctive diagnostic tools. Mean and standard deviation between MR Neurography and High Resolution Ultrasound is shown in Table 2. Conclusion: We performed a comparative study of brachial plexus abnormalities as detected by MRI and HRUS in patients with typical CIDP. The mean age of patients was 39.75 years. 60% of the patients were in the age group between 18-and 39 years. Up to 90% of patients had abnormal imaging study results of the brachial plexus. We found a high level of concordance between these neuroimaging techniques. Brachial plexus MRI is an accepted technique for the diagnostic work-up of both CIDP, in particular in the case of abnormal NCS that do not fulfill all electrophysiological consensus criteria for Background and Aim: Thrombotic complications leading to cerebrovascular events occurring in conjunction with Covid-19 vaccination though rare, is well documented. Moyamoya Angiopathy is a progressiveintracranial vasculopathy leading to recurrent strokes. Methodology: Result: We present two index cases of young patient presenting with stroke and TIA following Covid-19 vaccination (COVISHIELD) leading to unmasking of Moyamoya Angiopathy. Conclusion: Arterial stroke following Covid-19 vaccination though reported, is uncommon. However, in the background of a vasculopathy, it may not be so rare. Moyamoya Angiopathy has been closely studied in the model of inflammatory pathophysiology in genetically predisposed patients leading to progressive vaso-occlusive disease. Few reports of Covid-19 infection potentiating Moyamoya Angiopathy symptoms are also documented. Thus, in extrapolation of the inflammatory etiopathogenesis of Moyamoya Angiopathy, Covid-19 vaccination can similarly affect the Moyamoya symptomatology. These two index cases open new lines of enquiry regarding the interplay of Covid-19 vaccination and neurological destabilization in patients with probable underlying vasculopathy.

Award ID: 963

Moyamoya Angiopathy Unveiled Following COVID-19 Vaccination (Covishield)

Ajitava Dutta, Shambaditya Das, Souvik Dubey, Biman Kanti Ray

Department of Neurology, Bangur Institute of Neurosciences, IPGMER and SSKM Hospital, 52 1/A Sambhu Nath Pandit Road, Bhowanipore, Kolkata, West Bengal, India

E-mail: ajitava.dutta1990@gmail.com

Background and Aim: Thrombotic complications leading to cerebrovascular events occurring in conjunction with Covid-19 vaccination though rare, is well documented. Moyamoya Angiopathy is a progressiveintracranial vasculopathy leading to recurrent strokes. Methodology: Result: We present two index cases of young patient presenting with stroke and TIA following Covid-19 vaccination (COVISHIELD) leading to unmasking of Moyamoya Angiopathy. Conclusion: Arterial stroke following Covid-19 vaccination though reported, is uncommon. However, in the background of a vasculopathy, it may not be so rare. Moyamoya Angiopathy has been closely studied in the model of inflammatory pathophysiology in genetically predisposed patients leading to progressive vaso-occlusive disease. Few reports of Covid-19 infection potentiating Moyamoya Angiopathy symptoms are also documented. Thus, in extrapolation of the inflammatory etiopathogenesis of Moyamoya Angiopathy, Covid-19 vaccination can similarly affect the Moyamoya symptomatology. These two index cases open new lines of enquiry regarding the interplay of Covid-19 vaccination and neurological destabilization in patients with probable underlying vasculopathy.

Award ID: 964

Progressive Cerebellar Ataxia: A Rare Presentation of Multicentric Castleman’s Disease

Aishee Bhattacharyya

Neuromedicine 3rd year Resident, Bangur Institute of Neuroscience, West Bengal, India

E-mail: aishee1234@gmail.com

Background and Aim: Castleman’s disease (Angiofollicular lymph node hyperplasia) is a rare non clonal lymphoproliferative disorder that affects lymphoid organs and rarely extranodal tissues in any region of the body caused by impaired immunoregulation leading to abundant B cell and plasma cell proliferation. Clinically it may be unicentric (localized) or multicentric (generalized or multifocal) and pathologically classified as Hyaline Vascular, Plasma Cell type, Mixed type and HHV8 associated.1 CNS manifestation is uncommon and therapeutic guidelines are not well established owing to the rarity of this condition. Methodology: We describe a 58 year old female with history of subacute onset progressive ataxia with dysarthria for 2 months. She also had a history of prolonged intermittent low grade fever associated with reduced appetite and significant weight loss over last 1 year. She had pallor, generalized lymphadenopathy with splenomegaly and neurological examination showed features of cerebellar ataxia, dysarthria and right upper limb intention tremor. MRI Brain - showed :T2 hyperintensity in bilateral cerebellar hemisphere and right cerebral peduncle without diffusion restriction or contrast enhancement. Cervical lymph node biopsy was consistent with the diagnosis of Hyaline Vascular type of Castleman’s disease. IHC was negative for lymphoma and HHV 8. Whole Body PET CT showed hypermetabolism in multiple lymph nodes, spleen as well as right cerebellum supporting the diagnosis of multicentric Castleman’s disease. She was initially treated with High dose IV methylprednisolone followed by Tocilizumab with partial remission, however she succumbed to pneumonia and sepsis 1 ½ years after initial diagnosis. Result: CNS manifestation in Castleman’s disease occurs mostly in 4th or 5th decade, most commonly associated with the Hyaline Vascular type. 5 year Survival rate is 50-77% in idiopathic Multicentric Castleman Disease1 but survival rates among those with CNS involvement in unknown. Among the cases reported in literature – the predominant CNS manifestations are a. Infiltration- parenchymal involvement or meningeal infiltration b. Vascular:cerebral vasculitis 2 c. Immune dysregulation- eg immune mediated cerebellitis 3, autoimmune hypophysitis. Treatment guidelines of idiopathic MCD are not well established but include : Tocilizumab or Siltuximab in conjunction with steroids as 1 st line therapy followed by Rituximab in non responders. Conclusion: CNS involvement in Castleman disease is an exceedingly rare entity usually suspected in those with presenting with CNS mass lesion or meningeal infiltration in association with clinical and histopathological evidence of the disease. However the learning points of this case include that CNS involvement in Castleman’s disease may radiologically mimic a demyelinating pathology. Thorough screening for systemic features in imperative in all patients presenting with suspected demyelination. b. Early detection and probably more aggressive management is required for the subset of patients with CNS involvement and Castleman’s disease.

Award ID: 965

Study on Etiology and Clinical Presentation of Stroke in Tertiary Care Hospital

Priscilin Vinita J.

E-mail: priscilinvinita0707@gmail.com

Background and Aim: Stroke is one of the leading causes of death and disability in India. Early diagnosis and prompt treatment can reduce the morbidity. Knowledge about the clinico-demographic profile of patients with stroke will improve the stroke services. To find out the clinical, demographic, imaging features and risk factors of stroke in adults in a tertiary care hospital Methodology: Retrospective cohort review of all cases with a diagnosis of stroke between January 2022 to june 2022 from Velammal Medical College, Madurai. Result: There were 50 patients with stroke during this period. Forty-two (84%) had ischemic stroke and 8 (16%) had haemorrhagic stroke. The male: female ratio was 3:1. Majority (40%) belonged to 51-60 years age group. The commonest risk factor identified was hypertension (50%) followed by diabetes mellitus (22%). Headache (30%) was the most common symptom followed by giddiness (20%). Clinical examination revealed right hemiparesis in 21 (50%), left hemiparesis in 10 (25%), ataxia in 9 (18%), aphasia in 7 (14%), sensory loss in 4 (8%), and Horners syndrome in 1 (2%) patient. Among the 42 patients with acute ischemic stroke, anterior circulation was involved in 31 (75%) and posterior circulation in 11 (25%). Among the eight patients with hemorrhagic stroke, 6 (75%) had hemorrhage in the capsuloganglionic region and 2 (25%) in the brainstem. Out of 42 patients with ischemic stroke, only two arrived during the window period and underwent intra venous thrombolysis. One underwent mechanical thrombectomy Conclusion: Awareness about stroke Symptoms and window period (4.5 hrs) is important for the prompt treatment, thereby reducing the morbidity and mortality of stroke

Award ID: 966

Touch-Me-Not: A Study Analyzing the Effect of Chronic Smartphone Usage on the Fingertips of Normal Individuals

Sidharth M. Rahejaa

Final Year MBBS Velammal Medical College Hospital & Research Institute

E-mail: sidharthrahejaa.13@gmail.com

Background and Aim: In today’s world we can access the wealth of the universe’s knowledge via a device that fits in the palm of our hand: the smartphone. Through gestures we ingress on the benefits of this technological marvel. The skin of our finger tips is the chief mode of interaction to utilize the smartphone’s features. There have been numerous studies that have studied the various effects of smartphone usage on different physiological systems of the body, none have analyzed the effect of chronic smartphone usage on the fingertip sensations of the hand. The authors tried fill this lacuna in understanding through this particular study. To study the effect of smartphone touchscreens on the fingertips of normal individuals in terms of sensory perception change. By tracking the mobile phone usage time of the participants and measuring sensory perception changes utilizing 2 parameters: Two Point Discrimination and Pain threshold measurement. Methodology: The study involved 70 participants in the age group of 20-25 years of both genders. Smartphone usage behaviors was reported by the participants through a self-designed questionnaire to gauge whether appropriate for criteria. Smartphone usage time was tracked via a smartphone application. In Group A, participants thumb & index finger were tested for pain & touch sensations. In Group B, the participants middle, ring and little fingers were tested for the same. Digital Algometer and Compass Aesthesiometer are used to measure the sensations. The results were analyzed statistically using AVOVA. Result: A significant difference (p < 0.001) was observed in the pain threshold values and 2-Point discrimination values was observed between Group A and Group B. It was observed that there existed a significant difference the male and female participant values of parameters measured of Group A. Conclusion: Chronic smart phone usage decreases pain and touch sensations

Award ID: 967

Guanidino Acetate Methyl Transferase Deficiency - A Treatable Cause of Developmental Delay

Caroline Silvia Lawrence

Dept. of Paediatrics, Mahatma Gandhi Medical Hospital and Research Institute, Pondicherry, India

E-mail: silcarol693@gmail.com

Background and Aim: Developmental delay is a common cause for referral to paediatric neurology services. Guanidino Acetate Methyl Transferase deficiency -a creatine deficiency syndrome, is a treatable cause of developmental delay, necessitating early diagnosis. Methodology: A four-year-old girl, firstborn of third-degree consanguineous parents presented with two episodes of generalized seizures. She had global developmental delay, of which language, social and adaptive domains were markedly involved. She had poor attention span, minimal eye to eye contact, microcephaly, appendicular ataxia and a wide-based gait. Thyroid profile, vitamin B12 levels, ammonia, lactate, ophthalmological examination were normal. MRI brain showed symmetrical hyperintensity in globus pallidi. TMS, urine organic acids were normal. Considering a possibility of Leigh syndrome, she was started on mitochondrial cocktail in addition to anticonvulsants, occupational and speech therapy. Six months later, she developed generalized choreoathetosis following two days of fever. Repeat MRI brain including MRS revealed absent creatine peak. Clinical exome sequencing identified a homozygous mutation in GAMT gene confirming the diagnosis of GAMT deficiency. She exhibited significant developmental gains following creatine supplementation and protein restricted diet. Result: GAMT deficiency-one of the three creatine deficiency syndromes is characterized by global developmental delay, autistic features, seizures, and movement disorders. Early detection and prompt treatment improves the developmental outcome. Absence of creatine peak in MRS is a hallmark of creatine deficiency syndromes. Conclusion: GAMT deficiency should be suspected in any child with unexplained developmental delay, and MRS should be done to look for absent creatine peak.