IANCON 2023 Poster Abstracts

Abstract ID: 40

Fat-fraction Quantification Using Dixon Technique in Duchenne Muscular Dystrophy

Manisha Mohanty, Deepak Menon, Manoj Kumar, Jitender Saini, Seena Vengalil, Saraswati Nashi, Atchayaram Nalini

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: manisha4236@gmail.com

Background and Aim: Quantitative magnetic resonance image (MRI) using Dixon MRI to measure muscle fat fraction (MFF) is more precise and reliable method than visual fat fraction methods for longitudinal follow-ups of patients of Duchenne muscular dystrophy (DMD). The current study aims to detect any correlation between a one-time FF estimation using MRI Dixon technique as a biomarker of clinical phenotype. Methodology: The study was a single centre prospective observational study. Boys with genetically confirmed DMD, aged between 6 and 18 years with independent ambulation, were recruited. Age, muscle strength assessment, MRC sum score, North Star Ambulatory Assessment (NSAA), 6 minute walk test (6MWT) were evaluated. MRI was obtained and a single axial slice at 50% distance between hip to knee joint was chosen for examining the quadriceps, adductors and hamstrings. Another axial section at the femoral head was chosen for gluteus maximus. The images were processed by MATLAB based codes. Results: Thirty-five boys with a median age of 8 years (mean 8.34 years, 6 -12 years) were included. The highest MFF was found for gluteus maximus (mean -51.11 ± 23.56 and 49.38± 24.85 for right and left respectively) followed by quadriceps (mean -26.51 ± 20.58 and 26.35 ± 21.25 for right and left respectively). There was a positive correlation between MFF of muscles groups and age, strongest being that of quadriceps (r = 0.619, p <0.001 and r = 0.597 and p < 0.001 for right and left quadriceps respectively). The MFF of all the muscle group correlated significantly with all the clinical parameters (MRC sum score, NSAA, 6MWT). Discussion: Gluteus maximus had the highest MFF. The quadriceps MFF had the strongest correlation with the MRC sum score, 6MWT and NSAA score at cross-section. Conclusion: MFF using Dixon technique shows excellent correlation with other clinical measures of DMD assessment.

Abstract ID: 135

Effectiveness of a Training Program in Improving Neurological Skills among Primary Health Care Professionals: A Karnataka Brain Health Initiative (KaBHI)

G. Sarath, Pooja Mailankody, Rajani Parthasarathy, D. Randeep, Girish Rao, Avanthi Paplikar, J. Annie, Feba Varghese, Rehan Shahed, Girish Kulkarni, Sanjib Sinha, Suvarna Alladi

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: gsarath1995@gmail.com

Background and Aim: Neurological disorders, as reported by the WHO, are the leading causes of mortality and disability. A public health model “the Karnataka Brain Health Initiative” was developed in Karnataka, India to implement an evidence-based comprehensive model to prevent, diagnose, and treat neurological disorders at all healthcare levels. Many common neurological disorders can be identified and treated at the primary healthcare level. The primary challenge in treating neurological disorders at this level is the insufficient knowledge and skills of PHC professionals. This study aimed to evaluate the effects of training PHC professionals on diagnosing and treating major neurological disorders. Methodology: We adopted a pre- and post-test study design without a control group. A hybrid training program was conducted for PHC professionals in Chikkaballapura, Kolar, and Bengaluru South over a period of 2 months which included both in-person workshops (4 to 4.5 hours/ workshop) and online training sessions (90 minutes/session). The impact of the training program was assessed by the KaBHI Primary Health Care Physicians Training questionnaire. Results: A total of 120 PHC professionals participated in the pre-and post-training. The average age of the participants was 34.9±6.63 years. The paired-t test showed that there was a significant increase in the questionnaire test scores before and after the training program (p<0.001). A significant improvement was also found in different neurological disorders such as headache, epilepsy, stroke, and dementia (p<0.001). Discussion: PHC professionals of the study had limited knowledge and awareness of the diagnosis and treatment of neurological disorders before the training program. After undergoing training, the PHC professionals were efficient to diagnose and manage common neurological disorders. Conclusion: In conclusion, this study assessed the impact of a comprehensive training on PHC professionals in India for common neurological disorders. The program improved knowledge and clinical skills in diagnosing and managing common neurological conditions.

Abstract ID: 136

ICF-CY: A Better Tool to Assess Disability in Children with Neurodevelopmental Disorders

Mary Iype, Sanjeev Thomas, Anish Ts, Hamza Mullath, Murugan Nair

DM Neuro Completed, Other

E-mail: maryiypedr@gmail.com

Background and Aim: Disability in children with neurodevelopmental disorders is measured using tools that are copyrighted and expensive. ICF-CY, developed by the WHO, is a tool that is universally available and user-friendly, that can record disabilities and impairments of developing children. We aimed to characterize the diversity in impairment of children who are challenged for communicative and cognitive. Methodology: Methods: All children between 2 years and 17 years-of-age referred to our centre with a cognitive disorder from January 2014 to June 2022 were recruited. The medical records were screened and the family was interviewed. ICF-CY was translated and then applied on the cohort. Their impairment and disability were represented by ‘b scores’ and ‘d scores’ respectively. Results: Inclusion criteria were met by 300 children (221 with Autism-spectrum-disorder (ASD) and 79 with other disorders including ADHD, Cerebral-Palsy, language delay, learning-disability and intellectual-disability). There was a negative correlation between VSMS and ‘d’ ratio (Odds ratio – 7.7; 95% CI 3.73-15.86: Spearman correlation 0.354; SE 0.7; p value <0.001) and a positive linear relation between the ‘b’ ratio and the ‘d’ ratio (Odds ratio – 32.14 95% CI 14.3-72.36: Spearman correlation 0.61; SE 0.7; p value <0.001). Comparing the cohort of children with ASD vs. the rest, the difference in disability scores between the two groups was significantly better brought out by the ‘d score’ when compared to the VSMS score. Discussion: ICF-CY is a tool that represents the disability of differently-abled children better than a standard tool (VSMS). Besides, we have demonstrated that there is a negative correlation between the ‘d scores’ and the VSMS score. Conclusion: ICF-CY can be used universally as it is available in the public domain and free of cost. The tool will possibly in the future, make comparison of the disability of groups seen at different centres easy.

Abstract ID: 137

Care Seeking Pathways for Common Neurological Disorders in Karnataka, India: Data and Vignettes from a Cross-Sectional Analysis of Patients Presenting to a Quaternary Referral Center

P. Suhas, Rahul Sastry, G. Sarath, Suvarna Alladi, Girish Rao, M. Pooja, Faheem Arshad, Priya Thomas, M. Miluitty, T. Atheera

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: suhasvp24@gmail.com

Background and Aim: Neurologic disorders are responsible for the greatest burden of death and disability in low- and middle-income countries, including India. Well established functional health care systems and networks of care are necessary for the efficient and appropriate care. The goal of this study was to identify barriers to efficient care provision and opportunities for health system development and innovation for patients with neurologic disorders. Methodology: A cross-sectional study of adult patients presenting to the outpatient neurology clinic of a single hospital (NIMHANS) from either Bengaluru city or 2 neighboring districts was conducted. In concordance with diagnostic and treatment protocols for primary and secondary health centers developed as components of the Karnataka Brain Health Initiative (KaBHI), a contemporary capacity building, training, and care pathway development plan being implemented in the Bengaluru, Chikkaballapura, and Kolar districts.Patient’s demographics and care seeking pathways were collected and evaluated for appropriateness of treatment and pathway of care. Results: In total, 74 patients met inclusion criteria for this study. Among these, 10 (13.5%) carried a primary diagnosis of dementia, 23 (31.1%) were epilepsy, 21 (28.4%) were headache, and 20 (27.0%) were stroke. Thirty (40.5%) of initial patient presentations were deemed to be inappropriate and 17 (23.0%) resulted in inappropriate treatment or management . Majority of rationales for choice of initial treatment center were made on the basis of proximity (54.0%) and reputation (39.2%). A minority of the were made on the basis of referral by another doctor (37.5% and 29.0%, respectively). Although urban patients were significantly more likely to be closest to a private hospital , there were no significant differences in the proportion of urban and rural patients who initially presented to a private hospital. Discussion: In results. Conclusion: There are significant gaps in the existing healthcare system for patients with neurological diseases. There are significant opportunities for improvement.

Abstract ID: 138

Congenital Myasthenic Syndromes: A Case Series – A Compendium of Rare Cases

Indhuja Karunakaran, J. Manickavasagam, G. Balaji, S. Hariharan

DM Neuro Pursuing, Other

E-mail: drindhuja@yahoo.com

Background and Aim: Congenital myasthenic syndromes (CMS) are a diverse group of disorders characterized by abnormal neurotransmission at motor endplate. Here we present a series of eight patients with CMS. Aim: To describe the clinical features and genetic profile of CMS patients diagnosed in our hospital during the last 18 months. Methodology: Patients having myasthenic features with atypical clinical presentation and AchR antibody (Acetylcholine receptor antibody) negative status were clinically evaluated and followed up with genetic analysis. Results: There were 8 patients. (three male and five female). Age at diagnosis varied from 19 day old infant to 56 years (mean-21 years). Almost all patients had ocular weakness while 6 patients had associated bulbar and limb weakness. Three patients had consanguineous parentage. Neonatal respiratory distress and delayed motor milestones were observed in four patients. Positive family history was observed in only one patient. Unique features like : associated kyphoscoliosis was noted in two patients; One patient had LGMD like phenotype; Claudication like severe proximal muscle weakness was seen in one patient; Two patients had waddling gait; Two patients had syndromic facies with high arched palate; One patient had subnormal IQ and seizures . Three patients had CHRNE mutation while there were one of each : DOK7, GMPPB, COLQ, Chr 6q and SLC18A3. All reported good response to treatment. Discussion: CMS includes a heterogenous group of disorders as shown above. Patients had mutation in all three pools: pre-synaptic, synaptic and post-synaptic CMS. None had affected siblings. Some patients did not report much fluctuation in motor weakness. All showed decremental pattern in RNS (Repetitive Nerve Stimulation). Conclusion: This series is unique in their varied onset, ranging from newborn to adulthood, uncommon associated features, diverse mutations including the rarer SLC18A3 and a relatively benign course with good response to medical management.

Abstract ID: 139

Strokes with Monogenic Inheritance

Preethiswary Subramanian, J. Manickavasagam, G. Balaji, S. Hariharan

DM Neuro Pursuing, Other

E-mail: preethiswary@gmail.com

Background and Aim: We describe five stroke patients from two different families with mutations representing CADASIL and CARASIL. Methodology: We provide a detailed clinical, imaging and genetic studies conducted in these patients. Two siblings (brother and sister) from the first family with similar phenotype were evaluated and diagnosed with CARASIL. Members from three successive generations of second family were diagnosed with CADASIL by genetic analysis of the index patient. Result: Both siblings exhibited early onset stroke, attacks of low backache and disc herniation, premature baldness and diffuse leukoencephalopathy involving cerebral white matter, bilateral external capsule and anterior temporal pole consistent with CARASIL. HTRA1 DNA sequencing using targeted genomic sequencing revealed homozygous mutation as c.904C>T in the HTRA1 gene resulting in stop codon and premature truncation of arginine at codon 302. The son, mother, and grandmother from the second family exhibited recurrent ischemic stroke and cognitive decline. Genetic testing showed Arg332Cys mutation in exon 6 of NOTCH 3. The classical involvement of the anterior temporal pole in MRI is less common with this specific mutation, and this finding is consistent with our patient. Discussion: Incidentally both siblings of CARASIL were operated for disc prolapse with one patient undergone revision surgery twice. Our CADASIL patient displayed several distinct features, including an earliest onset of symptoms than previously reported cases,an absence of migraine history, and hyperintensities in corpus callosum and infratentorial regions, which have also been documented in association with this novel mutation. Conclusion: There are only few case reports of CARASIL in Indian literature and only three familial cases were confirmed with mutational analysis. We have only three globally reported cases for this mutation at c.904C>T in the HTRA1 gene of CARASIL and none reported in India. Regarding CADASIL, this is the first reported case in India of this rare Arg332Cys mutation in exon 6 of NOTCH 3.

Abstract ID: 140

An Iconic Variant of an Ionic Disease

S. Mohinish, C. Leema Pauline, Neeraj Elango, Jered Livingston

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drmohinish92@gmail.com

Background and Aim: Introduction: SCN1A is the most common epilepsy gene mutation (80%) causing Dravet syndrome. The present case is a rarest entity in childhood onset disease of multiple hyperkinetic movement disorder in SNC1A – mutation with no predominant seizures and cognitive impairment. Methodology: Case discussion of the rarest presentation and probably the first reporting of such presentation. Results: Case Presentation: A 12 year old boy with a history of isolated motor delay and insidious onset of gait ataxia since childhood, almost static till five months ago, presented with sudden onset of right focal seizures with secondary generalization as status epilepticus. This was followed by insidious onset of multifocal dystonia: blepharospasm, oro-facial, laryngeal, cervical, and left-limb dystonia, lingual chorea, and parakinesia classically with diurnal variations. No further seizures noted. MRI revealed severe cerebellar atropy. Was initially suspected as mitochondrial / NCL, GM2 gangliosidosis. When started with anti-epileptics and anti-spasmodics, especially with carbamazepine, dystonia and ataxia were aggravated thereby posing a diagnostic challenge. Genetic work-up showed pathogenic variant of SCN1A- loss of function mutation in exon 28 as Dravet variant. Later, when carbamazepine was stopped and tetrabenazine was given, a dramatic improvement of symptoms was noticed. Discussion: Phenotype-genotype correlation of multiple hyperkinetic movement disorder in SCN1A- mutation opens the gate to think beyond classical Dravet syndrome. Detailed molecular studies of SCN1A and related channels are need of the hour. Conclusion: Association of hyperkinetic movement disorder with cerebellar atropy in SCN1A mutation is not studied in the pediatric population till now. Early identification by genetic analysis is needed. Targeted treatment is required in these cases emphasizing the need for precision medicine.

Abstract ID: 147

Embolic Strokes of Undetermined Source: Stroke Registry Based Study from a Tertiary Care Hospital

Gautam Bhausaheb Kale

DNB Neuro Completed, Other

E-mail: drgbkale@gmail.com

Background and Aim: Background: ESUS comprises non-lacunar infarct in absence of extracranial or intracranial atherosclerosis causing >50% luminal stenosis in artery supplying ischemic region or major cardioemebolic sources or no other specific cause of stroke. Aims: To study frequency and clinical characteristics of ESUS among patients presenting with first ever acute ischemic stroke in tertiary care center. Methodology: This study is a prospective observational study. Study cohort includes patients presenting to tertiary care hospital with first ever acute ischemic stroke. Study period is twenty months (July 2016 to February 2018). Subjects were enrolled after a written informed consent and institutional ethics committee approval was obtained. Analysis of history, clinical examination and investigations was done to establish mechanism of ischemic stroke. Results: Of 340 cases with first ever ischemic stroke, 54 (15.88%) patients had ESUS. Mean age was 60.6 years, 81.48 % were males. Median NIHSS at admission was 4, median mRS at discharge was 1. Frequency of vascular risk factors were: hypertension (62.96%); diabetes (46.30%); coronary artery disease (14.81%); dyslipidemia (35.18%); hyperhomocysteinemia (35.18%). Common minor risk potential embolic sources of ESUS were PFO (29.6%); moderate to severe left atrial enlargement (12.9%); and moderate LV dysfunction (12.9%). Discussion: Reported frequency of ESUS in various registries varied widely from1% in Amsterdam to 42% in Portuguese. In our study, ESUS accounted for 15.88%. Hypertension was commonest vascular risk factor in our study similar to many other studies. In ESUS Global Registry and systematic review of ESUS by Hart et al, ESUS patients were younger, and strokes were less severe; similar to our study. PFO (29.6 %) was commonest minor risk potential embolic source in our study, similar to ESUS Global Registry (25%). Conclusion: Frequency of ESUS is around 16%. Clinical characteristics of ESUS included younger age (mean 60 yrs), male preponderance (81.5%), minor strokes (median NIHSS score 4), and a good functional outcome (median mRS 1 and no deaths).

Abstract ID: 148

Clinical and Radiological Characteristics and Predictors of Outcome in Cerebral Venous Sinus Thrombosis

Lakshmi Madhuri Nallapaneni, Pradeep Raji

MS Ramaiah Medical College, Bengaluru, Karnataka, India

E-mail: nl.madhuri7@gmail.com

Background and Aim: The incidence of cerebral vein and dural sinus thrombosis (CSVT) is estimated to be between 0.2 and 1.57 per 1,00,000 per year ; CSVT represents between 1 - 5% of all strokes and is associated with variable outcome. This study is aimed to predict the factors associated with poor outcome and hospital fatality based on clinical and radiological characteristics. Methodology: In this retrospective study, we included all patients with the diagnosis of CSVT, who were admitted in our hospital from January 2018 to April 2023. Demographic data, clinical characteristics, radiological findings, risk factors, treatment given and outcome according to modified Rankin Scale (mRS) on discharge were assessed. The factors associated with hospital fatality and poor outcome (mRS>2) were investigated through univariable and multivariable analyses. Adjusted odds ratio (OR), 95% Confidence interval (CI) and p values were reported. Results: Among 82 patients , 44 were female. The mean age was 38.8+/- 10.2 .Total of 12 patients had poor discharge outcome and 2 patients died in hospital. Older age (OR – 1.041 , CI = 1.000-1.08), decreased level of consciousness, (OR = 5.46,CI = 2.17-13.72) focal neurological deficit (OR=5.63,CI=2.14-14.77) and expansion of intracranial hemorrhage (OR = 9.13 ,CI =1.96-42.64) were predictors of poor outcome. Older age (p =0.02), focal neurological deficit (p=0.005), deep venous thrombosis (p value =0.002), intracranial hemorrhage (p value = 0.005) were associated with hospital fatality. Discussion: Age, decreased level of consciousness and focal neurological deficit on admission, intracranial hemorrhage are predictors of poor outcome. In the current study, the result is in concordance with the ISCVT (International study on cerebral vein thrombosis). Conclusion: As CSVT can lead to death, early diagnosis and initiation of treatment are of utmost importance. Hence, patients at high risk of unfavourable outcome should be recognised and closely monitored to reduce mortality and morbidity.

Abstract ID: 149

Study of Interarm Blood Pressure Difference in Predicting Early Neurological Deterioration in Noncardioembolic Stroke Patients

Raamprasath Chandrasekaran, S. Saravanan

DM Neuro Pursuing, Other

E-mail: raamprasath.281292@gmail.com

Background and Aim: Interarm systolic and diastolic Blood Pressure difference though found in normal population, their importance in stroke patients especially in predicting Early Neurological Deterioration has rarely been studied. This study aimed to find the association between interarm systolic and diastolic blood pressure difference in predicating Early Neurological Deterioration. Methodology: Consecutive patients with Non cardioembolic stroke were taken into study. Cardioembolic stroke is diagnosed based on -Abrupt onset -Maximum deficit at onset -Involvement of multiple arterial territories -Evidence of other emboli in peripheral vessels -Defined source of emboli -Cortex involvement Blood pressure was measured in both arms at 12 hours from onset of stroke. END (Early Neurological Deterioration) was assessed at 72 hours of stroke according to NIHSS score. END is defined as increase in NIHSS score of ≥ 2 points since admission or subscore for level of consciousness ≥1 or any new neurological deficit. Results: Demographic characteristics comparison of patients with and without END did not differ significantly. Interarm systolic and diastolic pressure difference of ≥10mmhg was related to END. Causes of END were symptomatic hemorrhagic transformation, stroke progression, stroke recurrence and other medical complications like Myocardial infarction, pneumonia with major cause for END in this study being stroke progression. Discussion: This study suggests that interarm Blood Pressure difference of ≥10 mmhg could be useful in predicting Early Neurological deterioration. Conclusion: Causes of END were symptomatic hemorrhagic transformation, stroke progression, stroke recurrence and other medical complications like Myocardial infarction, pneumonia with major cause for END in this study being stroke progression.Interarm Blood Pressure difference of ≥10 mmhg could be useful in predicting Early Neurological deterioration.

Abstract ID: 150

Case Series of Rescue Stenting for Acute Ischemic Stroke Due to Intracranial Atherosclerotic Stenosis Related Major Vessel Occlusion

Vibhor Upadhyay, A. Thacker, Ritwiz Bihari, Sudhakar Pandey, Pradeep Gupta, Rohit Agrawal, Rakesh Mishra, Amrin Ir

All India Institute of Medical Sciences, New Delhi, India

E-mail: docvibhor@gmail.com

Background and Aim: After mechanical thrombectomy fails, intracranial stenting implantation is a crucial rescue option for the management of intracranial atherosclerosis-related occlusion (ICAS-O), but its safety and efficacy are yet unknown. For emergent major artery occlusion (LAO) with underlying ICAS, we looked into the effectiveness and safety of rescue intracranial stenting. This case series presents the outcomes of patients who underwent intracranial stenting for acute ischemic stroke. Methodology: We gathered information on patients who, between July 2022 and March 2023, underwent direct angioplasty at our facility as the initial treatment for ICAS-related LVO in the anterior circulation and were known pre-existing ICAS patients. We looked at procedural information, the level of reperfusion, functional results, and complications. A modified Treatment in Cerebral Ischemia score of 2b or higher was considered to indicate successful reperfusion. Modified Rankin Scale scores were used to evaluate functional outcomes after 90 days. Results: Seven patients data, with a mean age of 51.6±11 years, were examined. The median amount of time from the beginning of symptoms and recanalization was 299±35.6 min. Five patients had occlusions in the first segment of the middle cerebral artery, and two patient had in the basilar artery. Five patients (72%) experienced successful reperfusion. Discussion: The results of our case series are consistent with previous studies, which have shown high rates of successful recanalization and good clinical outcomes. Intracranial stenting for acute ischemic stroke in the window period is a safe and effective treatment option. The results of our case series are consistent with previous studies, which have shown high rates of successful recanalization and good clinical outcomes. The ultimate mTICI for the remaining patient (28%) was 2a after intracranial stenting was used. Conclusion: In individuals with acute ischemic stroke caused by known ICAS-related LVO, direct angioplasty may represent an alternative treatment plan.

Abstract ID: 161

Substance Use-related Vertigo: A New Entity and An Unrecognized Mimic of Functional Vertigo

Thomas Mathew, Surabhi Garg, Shagun Bhardwaj, Pranjali Ektare, Sindhu Nambiar, Molly George, Raghunandan Nadig

St. Johns Medical College, Bengaluru, Karnataka, India

E-mail: chakkuthom@hotmail.com

Background and Aim: Here we report the demographic and clinical features of five cases of substance use-related vertigo (SURV) mistaken as functional vertigo. Methodology: All patients presenting with the diagnosis of functional vertigo were enquired about the use of products containing pro-convulsant substances especially essential oils of camphor and eucalyptus. Patients with benign paroxysmal positional vertigo, migraine, Meniere’s disease, and structural brain abnormalities on MRI brain were excluded. A vertigo diary was used to know the frequency, duration, and characteristics of vertigo. All patients were asked to stop the exposure to the products containing pro-convulsant substances and were reassessed weekly till complete recovery. Results: Over a period of 6 months, we had 5 cases of substance use-related vertigo. The mean age was 49.6 ±18.6 years with a male: female ratio of 3:2. The mean duration of symptoms was 5.3 ±3.2 months. For 3 patients the attacks were episodic while 2 had continuous vertigo. Episodic attacks had a mean duration of 99.7 ±110.6 minutes with a frequency of 1-5 attacks per day. They had exposure to pro-convulsant substances through toothpaste. Substances implicated were camphor, cineole, thujone, and sodium fluoride. The mean duration of exposure was 6.2 ±3.8 years. The mean time taken for onset of improvement was 3.8±1.8 days and complete to near complete improvement was 9 ± 4.8 days. Discussion: In this case series, we report substance-use-related vertigo, a hitherto unrecognized entity. The exact mechanism by which these substances trigger vertigo is not known but may be due to either hyperexcitability of vestibular neurons or Purkinje cell dysfunction. Conclusion: Substance use-related vertigo is an unrecognized cause of vertigo and can mimic functional vertigo. Clinicians should be aware of this entity as complete recovery is possible on cessation of exposure to these pro-convulsant substances.

Abstract ID: 162

Early Outcome of Therapy with IV Edaravone and Oral Riluzole in Motor Neuron Disease Patients: Experience from a Tertiary Referral Centre in South India

Sunil K. Narayan, Jayaram Saibaba, Ramkumar Sugumarane

Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

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

Background and Aim: Motor Neuron disease is a fatal neurodegenerative disease characterized by rapidly progressive muscular paralysis caused by degeneration of motor neurons leading to muscle fasciculations, muscle weakness, wasting and spasticity. Respiratory failure limits survival to 2-5 years after disease onset. We studied the clinical profile and progression of disease in MND patients from Tamil Nadu with amyotrophic lateral sclerosis phenotype who were treated with IV Edaravone and oral Riluzole. Methodology: After ruling out ALS mimics by neuroimaging, patients who fulfil El Escorial criteria, were given IV Edaravone 60 mg once a day for 14 days followed by 14 days drug free period with subsequent treatment cycles of 60 mg once a day IV infusion for 10 days out of 14 day periods cycles. Tablet Riluzole 50 mg BD was given simultaneously. Age, onset age, symptom duration to start of therapy, postintervention follow up duration, pre-treatment ALS FRS scale and post-treatment ALS FRS scale at minimum 6 months follow up, Outcome of patients (proportion) remaining improving, static, progressing to respiratory, bulbar involvement or expired. Adverse events were also noted. Results: All patients recruited completed Edaravone course and were fully drug compliant to Riluzole. 18 patients who fulfilled the El-escorial criteria had mean age: 48.83 years ; Males – 12 (66.6%); Mean time to start of therapy 24.7 months. The mean pre-treatment ALS FRS scale was 34.8. Post-treatment ALS FRS at 6 months was 33.4. Only two patients had mild improvement after the ALS treatment regime. 77.7% had progressed to have bulbar involvement, 50% developed respiratory involvement and 5% expired. Discussion: Both Riluzole and IV Edaravone have been approved by many regulatory bodies for treatment of MND. Long term follow up data are scanty. From India no published data. Conclusion: The prognosis of ALS continues to remain poor despite the use of Riluzole and IV Edaravone regimen.

Abstract ID: 163

Juvenile Parkinsonism: An Unusual Clinical Presentation of Hereditary Spastic Paraparesis

Divya Rani, Shishir Chandan

Vardhman Mahavir Medical College, New Delhi, India

E-mail: divyarani270791@gmail.com

Background and Aim: Hereditary spastic paraparesis is a genetically and clinically heterogenous group of disorders. It’s estimated prevalence is very low, but it’s worldwide prevalence is actually underestimated because of benign nature of the disease and it’s varied clinical presentation. Our aim is to study the clinical phenotype of a case of HSP-11 as it is often misdiagnosed because of lack of suspicion. Methodology: A 15 years old male was evaluated for presenting complaints of asymmetric and insidious onset gradually progressive tremors associated with bradykinesia for last 1 year. The patient was worked up extensively to rule out common causes e.g. Wilson disease , NBIA etc. Later on , whole exon sequencing, was done which showed that patient is homozygous for a pathogenic variant in the SPG11 gene associated with HSP-11. Results: Patient was diagnosed as a case of autosomal recessive form of HSP, HSP-11, by genetic analysis. Discussion: SPG11 is an autosomal recessive form of HSP, which usually have onset in adolescence. It commonly presents with spasticity and paraplegia with additional features like mental impairment, cerebellar ataxia, Parkinsonism and structurally with a thin corpus callosum. Conclusion: SPG-11 is an autosomal recessive form of HSP, which predominantly presents with symptoms of spasticity and paraplegia. In our case patient, presented with parkinsonian features and was suspected to have juvenile Parkinsonism and later on patient was diagnosed as HSP-11 by whole exon sequencing. Therefore, HSP-11 should always be considered as an important differential diagnosis in any young adult presenting with juvenile Parkinsonism.

Abstract ID: 164

Scapular Winging: A Novel Finding in Spinocerebellar Ataxia

Suman S. Khushwaha, Deepti Gupta, Panda Kumar, Mridula Rustogi, Ashish Kulkarni, Rajinder Dhamija

DM Neuro Pursuing, Other

E-mail: sumankushwaha@gmail.com

Background and Aim: The autosomal dominantly transmitted Spinocerebellar Ataxias (SCAs) are a subset of inherited cerebellar ataxias. They are progressive neurodegenerative diseases, found to have many associations like ophthalmoplegia, spasticity, basal ganglia symptoms, tremors, peripheral neuropathy, amyotrophy, psychosis, seizures, visual loss and intellectual disability. Methodology: We report 2 cases of spinocerebellar ataxia with scapular winging. Case 1: 26-year male presented with 1.5-year history of gradually progressive cerebellar symptoms along with generalized dystonia, and cognitive dysfunction. On examination patient had bilateral scapular winging apart from ataxia, square wave jerks, slow saccades, brisk reflexes, bradykinesia, generalized dystonia. Case 2: 22-year male presented with 2-year history of pancerebellar ataxia with slow saccades. He had right medial scapular winging on examination. Result: To discern the cause for ataxia, reversible causes were ruled out and SCA panel was sent to look for neurodegenerative etiology which showed expansion in CAG repeats (ATXN3 in case 1 and ATXN2 in case 2). Discussion: SCAs are a class of neurodegenerative diseases with regional variations that share ataxia as a common clinical feature as a result of the cerebellum’s gradual degradation but can also affect other related areas. The usual causes of scapular winging include trauma, facioscapulohumeral dystrophy, calpainopathy, dystrophinopathy, late onset pompe disease, poliomyelitis, and iatrogenic but it`s presence in patients with SCA has not been described up till now. This can be postulated due to differential amyotrophy of the scapular muscles. Conclusion: Our case demonstrates a previously unreported unique finding of scapular winging in patients of SCA2 and SCA3. It highlights on the importance of examining for scapular winging in all cases of suspected spinocerebellar ataxia which can provide a direction for further phenotypic relation and subtyping.

Abstract ID: 172

The Impact of Sleep Disturbances in Patients with Migraine – A Prospective Observational Study from a Tertiary Care Specialty Hospital from South India

Sahil Mathur

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: sahil.mathur94@gmail.com

Background and Aim: Migraine is a common primary headache disorder that impairs the quality of life of patients. The inter-relationship between headache and sleep has not been studied in detail in Indian population. AIM: To study the impact of sleep disturbances in patients with migraine. Methodology: 117 adult patients of migraine visiting the neurology OPD, not on any prophylactic medication for migraine, were assessed by personal interview. Headache severity was recorded by the MIDAS questionnaire. A comprehensive questionnaire was developed for the characterization of headache. The subjective sleep quality was recorded by the Pittsburgh sleep quality index. Insomnia and excessive daytime sleepiness was graded by the ISI and ESS scales respectively. Co-morbid anxiety and depression were screened by the GAD-7 and PHQ-9 questionnaire respectively. Results: Out of 117 migraine patients, 85 were female (70.8%), with a mean age at presentation of 35.6 ± 9.8 years, mean duration of headache being 6.5 years. 90/117 patients had chronic migraine (76.9%), with a mean MIDAS score of 42.5. Mean ISI score was 10.2, and 34/117 patients had clinical insomnia. The mean PSQI score was 7.22 and 64/117 patients had poor sleep quality (PSQI score 5 or more). There was a significant correlation of MIDAS score with ISI score (r-0.735, p- 0.001); overall sleep quality – PSQI (r–0.657, p-0.001). The overall SF-36 score had a significant correlation with the total MIDAS score (r–0.568, p-0.001), total ISI score (r–0.788, p-0.001), and total PSQI score (r– 0.859, p-0.001). Discussion: The results of this study shows a correlation between headache severity, sleep disturbances, and health-related quality of life. This study emphasises the need to address the sleep disturbances associated with migraine to improve the quality of life and reduce the overall disability related to migraine. Conclusion: In patients with migraine, the severity of headache correlates with the severity of insomnia and the overall sleep quality.

Abstract ID: 501

A Case Report of HaNDL (Headache with Neurological Deficits and Elevated CSF Lymphocytes) Syndrome

Rajagembeeran Venugopl, Arun Kumar

Smt. B.K. Shah Medical Institute and Research Centre, Vadodara, Gujarat, India

E-mail: rajagembeeran01@gmail.com

Background and Aim: The syndrome of headache with neurological deficits and elevated CSF lymphocytes (HaNDL) is a rare, benign, and self-limiting condition. It is predominant in young adults and characteristically present with migraine-like headaches, transient neurologic deficits such as hemiparesis, hemiparesthesia, and dysphagia, and lymphocytic pleocytosis in the CSF. This syndrome is grossly underrecognized and underreported. Methodology: We had a case of 28 years female who presented with fever for one week which is mild and intermittent. She also had headache which is holocranial and throbbing in nature present more in day time lasting for more than 2 hours. She also had vomiting and photphobia. She had intermittent parasthesia over her right hand and leg which lasted for 10 minutes .The episodes were occurring about 5 to 10 episodes per day during which she could not move her right side upper and lower limb. These episodes got spontaneous remission after 5 to 10 minutes.On admission she was drowsy. Her neurological examination revealed no deficits. Results: Initially acute encephalitis with new onset seizures was considered. Other differentials considered were hemiplegic migraine and recurrent TIAs. Empirical treatment started. CT taken immediately which was normal. CSF opening pressure was about 35 cm which is high. CSF analysis showed 30 lymphocytes. CSF protein was 43mg/dL and glucose 56 mg/dL Other investigations including EEG, MRI brain with contrast were normal. Her conditions improved on next day itself. She became alert afebrile and her hemisensory symptoms also stopped..She was observed for a week and dischargerd with diagnosis of HaNDL (headache with neurological deficits and elevated CSF lymphocytes) syndrome base on clinical and investigations. Discussion: Migrainous pathophysiology such as vasomotor changes in the middle cerebral artery may play a role in HaNDL syndrome, causing asymmetrical pulsations in the blood flow. Some studies demonstrate a reduction in cerebral blood flow to the brain at the contralateral side of neurological deficits. Conclusion: On the other hand, few authors suggest antibodies formed after viral or autoimmune diseases may have led to aseptic inflammation affecting leptomeningeal vessels causing CSF pleocytosis and neurologic deficits.

Abstract ID: 502

A Rare Case Report of Sensorineural Hearing Loss and Gait Ataxia

Raajneesh Tamilarasan, B. Krishnakumar, V. Nandhakumar, M. Jawahar, R. Lakshminarasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: traajneesh@outlook.com

Background and Aim: Superficial siderosis is a neurodegenerative disorder resulting from recurrent leakage of blood into the subpial space. Not all patients have an identifiable source of leak. The prevalence is around 1 in 1 million individuals, and it is categorized under National Organization of Rare Disorders. Common neurological manifestation includes ataxia and sensorineural deafness. There are two types – infratentorial and cortical superficial siderosis. Methodology: Case study. Results: Case Report: 62 years old male presented to us with chronic progressive neurological illness in the form of worsening of bilateral hard of hearing and unsteadiness of gait for the past 4 years. On examination patient had bilateral sensorineural deafness and gait ataxia, Tandem Romberg’s positive and Tandem walking impaired. Patient did not have any other cerebellar, vestibular, or sensory symptoms. MRI brain revealed T2/FLAIR hypo intensities in bilateral parieto-occipital and bilateral cerebellum along with extensive gradient blooming of meninges and CSF sulcal spaces features suggestive of superficial siderosis. CSF analysis showed elevated protein levels. Discussion: The possible sources of leak include brain or spine trauma, neurosurgery, cerebral aneurysms or vascular malformations, cerebral amyloid angiopathy or spinal dural defects. In our case, there was no previous history of trauma or any neurosurgery, CT Cerebral Angiogram and MRI spine revealed no significant abnormality and no evidence of arterio-venous malformation or any feeder vessel. Hence, patient was started on Deferiprone and is on follow up currently. Conclusion: The average time from the clinical manifestation of early symptoms to diagnosis maybe as long as 10 years. Due to overlapping similarities, it is most often misdiagnosed a multiple sclerosis, Parkinson’s disease, or multiple system atrophy, but the current imaging modalities and recognizing this rare disease can lead to earlier diagnosis and appropriate management to stop the leakage can prevent the ongoing neurodegenerative process.

Abstract ID: 503

An Atypical and Rare Presentation of CNS Tuberculoma

Deepak Rajendran, V. Nandakumar, B. Krishnakumar, M. Jawahar, R. Lakshmi Narasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drdeepakrajendran@gmail.com

Background and Aim: CNS tuberculoma can have different manifestations like headache, seizures, focal deficits, cranial neuropathies and signs of raised intracranial pressure. Here we report the case of a 15 year old boy with CNS tuberculoma presenting only with bilateral ptosis. Our case report is unique in that bilateral ptosis as the sole manifestation of tuberculoma has been rarely reported in literature. Methodology: Case report. Results: 15 year old boy with past history of childhood tuberculosis presented with subacute onset of bilateral ptosis of 1 month duration. He had no history of headache, fever, altered mental status or symptoms of any other involvement of neuraxis. There was no history of diurnal variation or fatiguability. Examination revealed bilateral symmetric partial ptosis with no other positive findings. Extraocular movements, pupils, visual acuity and fundus were normal. Examination of rest of the nervous system were normal. Tests of fatigability were negative. MRI brain with IV contrast revealed peripheral ring enhancing T2 hyperintense lesion measuring 8.5 by 6.4 mm in dorsal midbrain with surrounding oedema causing mild extrinsic compression over the quadrigeminal plate, with no restricted diffusion, and was reported as possible granulomatous lesion. He was treated with steroids and ATT. Following therapy, ptosis has resolved over a period of 2 weeks. Discussion: In this case, bilateral ptosis is likely due to the involvement of caudal central subnucleus in the midbrain innervating levator palpebrae superioris muscle bilaterally, without affecting medically placed superior rectus subnucleus. Usually lesion in such a location involve caudal central subnucleus along with ipsilateral superior rectus subnucleus and the fascicle coming from contralateral superior rectus subnucleus. For CNS tuberculoma, ATT is indicated for a period of 12 months. Steroids are given if there is perilesional oedema. Conclusion: Bilateral symmetric ptosis without any other neurological features is rare in CNS tuberculoma. Tuberculoma should be considered as DD of ptosis.

Abstract ID: 504

Extrapontine Myelinolysis Presenting As Acute Onset Parkinsonism Even with Slow Correction of Hyponatremia

Rahul Gandhi, S. Velusamy, R. Lakshmi, Nithyanandam

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: rahulirg10@gmail.com

Background and Aim: Rapid correction of hyponatremia is a known risk factor for osmotic demyelination symdrome. However even with slow correction of hyponatremia results in osmotic demyelination syndrome. Here we present a case hyponatremia that was corrected slowly developed extrapontine myelinolysis which was treated with intravenous immunoglobulins. Methodology: 64 yrs old hypertensive male with previous history of admission for UTI found to have hyponatremia (112 mEq/dl) which was corrected over next 5 days to 133 mEq/dl. 2 days later patient developed acute onset progressive neurological illness of 3 days with akinetic state with complete mutism. On examination patient awake, obeys commands with no verbal output with bilateral upgaze restriction with axial and appendicular rigidity and 2+ DTR of all 4 limbs, bilateral plantar flexion and choreiform movements of left upper and lower limbs with dystonia of right lower limb toes. Results: MRI brain showed diffuse symmetrical T2/ FLAIR hyperintensities with no diffusion restriction and no enhancement in bilateral lentiform nucleus and anterior aspect of caudate nucleus. CSF analysis showed elevated protein with normal glucose level and no cells. CSF viral panel showed JE IgM equivocal which was repeated after 4 weeks was negative. Autoimmune encephalitis panel done was negative. Toxins history was ruled out. Discussion: Patient presenting with acute onset extrapyramidal features with symmetrical basal ganglia hyperintensities with background of hyponatremia even with slow correction of hyponatremia sugesstive of extrapontine myelinolysis after ruling out other causes. Conclusion: Hence with report we conclude that osmotic demyelination which is usually seen with rapid correction of hyponatremia can occur even with slow correction of hyponatremia.

Abstract ID: 505

Low Incidence of Neoplasm in Anti-glutamic Acid Decarboxylase Antibodies Associated Cerebellar Ataxia: A Descriptive Case Series from India

Rohan R. Mahale, Ravi Yadav, M. Sandeep, Anita Mahadevan

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: rohanmahale83@gmail.com

Background and Aim: Background: Anti-glutamate decarboxylase 65 antibody-associated cerebellar ataxia (anti-GAD65Ab-associated CA) is the most widely studied immune-mediated CA. The occurrence of neoplasm is rarely reported. Objective: To study the clinical, radiological profile, associated neoplasm, treatment outcome and prognosis in patients diagnosed with anti-GAD65Ab-associated CA. Methodology: The study was retrospective hospital-based descriptive analysis of cohort of patients diagnosed as anti-GAD65Ab-associated CA. Anti-GAD65Ab-associated CA was diagnosed as subacute or chronic CAs associated with serum and/or CSF anti-GAD65 antibody positivity. The baseline demographic details, clinical presentation, biochemical, serological, CSF, radiological profiles and treatment details were recorded from the hospital case records. Qualitative assessment of Serum anti-GAD65Abs were done by using Immunofluorescence method. Results: 13 patients were selected for analysis with female predominance (70%). The mean age at presentation was 47.5 ± 11.1 years (range- 29-65 years), and median duration of symptoms was 4 months. All 13 patients (100%) had gait ataxia. Ten patients had limb ataxia along with gait ataxia (75%). Seven patients had cerebellar dysarthria (54%). Four patients (31%) had gaze-evoked jerky nystagmus. Brain magnetic resonance imaging was normal in 7 (54%) and pure cerebellar atrophy in 6 patients. One patient was detected to have colon neoplasm. All 13 patients received intravenous methylprednisolone followed by oral steroids over 3 months. Six patients (50%) received plasma exchange along with intravenous methylprednisolone. Favourable outcome (mRS scores ≤ 2) was seen in 5, poor in 2 and 6 patients lost to follow-up after first admission. Discussion: The incidence of systemic neoplasm in our cohort was very low (8%) which is similar to other cohorts described earlier. Ariño et al (2014) reported 3 cases of neoplasm (34 patients). Conclusion: Anti-GAD65Ab-associated CA is one of the treatable IMCAs which presents as subacute to chronic progressive CA with favourable outcome with immunotherapy and low incidence of systemic neoplasm.

Abstract ID: 506

Paroxysmal Pain and Neuropathic Pruritus in a Case of Neuromyelitis Optica

Nageswaran Bala Ramachandran, S. Velusamy, A. Nithyanandam, V. Aravind Gautham, R. Lakshmi Narasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: dr.nageswaran@gmail.com

Background and Aim: More than 85% of NMOSD patients have reported pain as a major symptom. Painful tonic muscle spasms and neuropathic pain are the most common pain types in NMOSD. Paroxysmal pain and the presence of pruritus in the craniofacial regions in NMOSD are rarely reported. Methodology: Case report. Results: We report a case of a 60 yr old lady who presented with constipation, urinary retention, followed by weakness of both upper and lower limbs. Patient had developed severe intermittent burning pain on her right forehead area that lasted for 12 days, which was provoked by a slight touch. The pain frequency varied from five to ten times a day, with each attack lasting 5 to 15 minutes. After 12 days, she developed pruritus on the same area which lasted an hour and recurred on the average of 5 times a day, for a week. It was worse at night and was associated with pain. Scratching provided temporary relief for a few seconds. patient had sensory loss of pain and temperature below xiphisternum level. Vibration sense was diminished in bilateral lower limbs and upper limbs till elbow in a graded manner. Patient had long extensive lesions in the spinal cord from C2 to D7 and D9 to L1 levels and found positive for aquaporin 4 antibodies. Discussion: Pain occurs due to increased glutamate and BDNF in synapses and decreased GABA andendocannabinoids.Neuropathic pruritus in NeuromyelitisOptica may reflect the typical central cord lesions affecting dorsal horn neurons, being rich in mediators of pruritus. Conclusion: Neuromyelitis Optica is a rapidly disabling disease. The prompt identification of a relapse is crucial. We believe that paroxysmal facial pain and pruritus are manifestations of involvement of sensory pathways in this condition. Unexplained pain and pruritus may therefore, on occasion herald an oncoming relapse of Neuromyelitis Optica.

Abstract ID: 507

Clinico- radiological Profile of Stroke Due to Immune Mediated CNS Vasculitis- Role of Vessel Wall Imaging – A Prospective Cohort Study from a Tertiary Care Center

Goli Ashwini, George Patil, George Patil, Gudipati Anantaram

KIMS Hospital, Hyderabad, Telangana, India

E-mail: drashwini2810@gmail.com

Background and Aim: CNS vasculitis poses a great diagnostic challenge. Vessel wall contrast enhancement aids in diagnosis. Our objective is to study the clinical and radiological profile of patients with immune mediated CNS vasculitis and to assess the role of high resolution vessel wall imaging (HR VWI) in diagnosis and treatment. Methodology: Patients with suspected CNS vasculitis either primary or systemic were prospectively enrolled between August 2022 to May 2023 at a tertiary care center. Demographic details, examination, neuroimaging, treatment, and functional outcomes were assessed. Results: A total of 28 patients satisfying inclusion criteria were taken into study. Of which 13 were primary and 15 were systemic CNS vasculitis. The mean age at presentation 39.46 (20-59) years and mean age at symptom onset was 36.07 (18-57) years, with a mean diagnostic delay of 3.39 years. Among 28 patients, male to female ratio is 1:4.6. 23 (82.1%) patients presented with hemiparesis at initial presentation followed by 6 (21.4%) patients with seizures and 4 (14.2%) patients with vision disturbances. Among 16 who underwent HR VWI, 14 (87.5%) had positive findings. All of them received steroids, maintenance immunomodulators given AZT (11), MMF (2), RTX (1), CYC (9), MTX (2). Favorable mRS (0-2) was seen in 20 (71.4%) at 6 months follow up (76.9% primary and 66.6% systemic vasculitis). VWI at 6 months of treatment could be done in 7 who showed significant resolution. Relapse is seen in 3 [2 primary and 1 systemic vasculitis]. Overall mortality rate is 10.7%. Discussion: Our study has younger age at presentation with primary CNS vasculitis having higher relapse rate compared to systemic vasculitis where mortality and poor functional outcome is seen. Conclusion: This study showed the utility of vessel wall imaging in etiological work up, diagnosis, neuroimaging surveillance and targeted treatment.

Abstract ID: 508

Rare Manifestation of Rare Autoimmune Disorder

Praveen Panicker, Keerthi Balachandran, Thomas Iype, M. Madhusudanan

DM Neuro Completed, Other

E-mail: panicker85@gmail.com

Background and Aim: Stiff-person syndrome usually presents with thoracolumbar or limb stiffness. They can also present with cerebellar ataxia and epilepsy. Stiff-person syndrome is more common among females with age of onset in third decade. Methodology: A 52 year female had right lower abdomen cramps producing rectus abdominis tear ten years back. She had GAD antibody in serum and was diagnosed as focal stiff person syndrome. She improved with oral medications -baclofen and diazepam- and was completely symptoms-free. She now presented with left-side ataxia and oscillopsia. Examination: The patient had downbeat nystagmus and left upper and lower limb dysmetria. Results: Investigations: Her serum GAD antibody titre was 245 IU/mL (IU/mL). The paraneoplastic workup was unyielding. MRI brain showed cerebellar atrophy. Management: Ataxia improved on intravenous Methylprednisolone pulse, followed by rituximab. However, she presented with a generalised onset seizure two weeks later, requiring anti-seizure medicine. Her ataxia is improving. Discussion: Focal stiff person syndrome may be easily misdiagnosed as focal muscle cramps unless specifically evaluated. Our patient remained asymptomatic for 10 years after initial presentation. This emphasizes the need for proper follow up. Our patient had stiff-person syndrome plus disorder as she progressed to develop cerebellar ataxia and brainstem dysfunction in the form of downbeat nystagmus. Her last symptom was generalized onset seizure. Usually, serum titre of GAD antibody >500 IU/mL is regarded as risk for poor outcome. Our patient with lower titres responded to rituximab therapy. Conclusion: Focal stiff person syndrome involving abdominal muscle is rare and the same progressing to cerebellar ataxia with downbeat nystagmus and seizure is still more rare. We are highlighting the importance of long-term follow up as patient may need immunosuppression therapy later.

Abstract ID: 509

Risk Factors for Relapse of NMOSD – A Tertiary Care Centre Experience

Himaja Sanganabhatla

Osmania Medical College, Hyderabad, Telangana, Insia

E-mail: himaja.sanganabhatla@gmail.com

Background and Aim: Neuro Myelitis Optica spectrum disorder (NMOSD) is a rare autoimmune inflammatory disorder of the central nervous system (CNS) that is mainly associated with serum autoantibodies against Aquaporin-4 (AQP4) in astrocytes. It is an aggressive inflammatory disorder characterized by recurrent attacks of bilateral ON and LETM which results in long term visual and motor disability; 60 % become functionally blind at 5 years and 50% become wheel chair dependent. Here, we aim to study the risk factors associated with relapse in NMO. Methodology: 33 patients who were admitted under the Department of Neurology in a tertiary care centre between April 2017 to April 2023 fulfilling Wingerchuk criteria of NMOSD were selected and data was collected retrospectively, including age of onset, clinical profile, investigations, functional recovery, relapses and details of treatment and follow-up. Results: Out of 33 patients, 32 were female and one patient was male Majority of patients were in the age group of 21-30 yrs. Majority of patients presented with paraparesis – 11 (33%), followed by Quadriparesis – 8 (24%), Optic neuritis – 6 (18%). Least common symptoms were Monoparesis and Brainstem syndrome. Discussion: All patients who had relapse (16 out of 33) were AQP4 positive and all had Tonic spasms at presentation. (100% of relapses) All these patients were young females (93% of relapses) except for one young male and the clinical presentation was paraparesis with imaging showing LETM in 13 patients (81% of relapses). Patients who had longer segments of cord involved on MRI had higher risk of relapse. Three out of six patients with ON had relapse (18% of relapses). Four patients had Area Postrema syndrome at presentation of which only one patient had relapse (drug defaulter). Conclusion: In a resource limited setting, early detection of risk factors can potentially decrease the relapses of NMOSD, thus improving quality of life.

Abstract ID: 510

Heart in the Brain

Saurabh Sultania, Birinder Paul, Dhananjay Gupta

Dayanand Medical College, Ludhiana, Punjab, India

E-mail: saurabh211311@gmail.com

Background and Aim: Bilateral medial medullary infarction (MMI) is a rare stroke subtype. It usually leads to quadriplegia, sensory disturbance, hypoglossal palsy, bulbar paralysis, etc. We encountered a patient with rapidly progressive quadriparesis in elsberg’s pattern. Diffusion-weighted imaging (DWI) exhibited a “heart appearance” sign in the bilateral medial medulla. Methodology: Case description of a patient with acute onset progressive quadriparesis with bulbar palsy. Results: 51 year old male, k/c/o hypertension, presented with sudden onset weakness over the right upper limb followed by lower limb along with slurring of speech over the 1st 24 hours On examination his GCS was 15, dysarthria was present, tongue was deviated towards the left side, power in the right upper and lower limb was 3/5. Plantar was extensor over the right side and flexor over the right side. Non contrast CT head was normal, CT angiography head and neck didnt reveal any significant large vessel occlusion. Patient was started on antiplatelets and statins Over the next 48 hours he developed weakness over the left lower limb and left upper limb along with bulbar weakness.. Mri brain was done which showed diffusion restriction in bilateral medial medullary region (heart appearance) s/o acute infarct. Discussion: Medial medullary syndrome is found in approximately 1% of total posterior circulation strokes, and bilateral medial medullary syndrome is even rarer. Clinical Presentation may mimic GBS, brainstem encephalitis etc. Conclusion: This Patient had rare presentation of medial medullary syndrome, in the form of acute onset progressive quadrparesis in elsberg pattern. Recognition of this entity is important as it carries a poor prognosis.

Abstract ID: 511

Relationship between Acute Ischemic Stroke and Neutrophil to Lymphocyte Ratio: A Prospective Study from a Tertiary Care Centre in South India

Rindha Venepally, Sandhya Manorenj

DM Neuro Pursuing, Other

E-mail: rindha7.rao@gmail.com

Background and Aim: To examine the association between acute ischemic stroke and the Neutrophil to Lymphocyte Ratio (NLR) in various aspects, including stroke severity at admission, functional outcome at 3 months and risk of recurrent stroke. This study aims to evaluate how NLR could be used as a potential biomarker or prognostic indicator in these areas of ischemic stroke. Methodology: The prospective study was conducted in a tertiary care center in South India from September 2021 to January 2023.The study included patients between the ages of 18 and 80 years, whose diagnosis was confirmed by imaging. Patients with hemorrhagic and venous stroke, pregnant women, and individuals with febrile illness accompanying the stroke were excluded from the study. The study enrolled a total of 110 patients who provided informed consent. Within 24 hours of admission, NLR was calculated. NIHSS was assessed at the time of admission and MRS at three months. Statistical analysis was done using SPSS V20.0 and p value was significant at < 0.05. Results: A total of 110 patients with AIS fulfilled the criteria. The mean age of patients was 58.2 ± 11.22 years old and 76 (69.1%) patients were men. We found that 83.6% of patients had abnormal NLR value. Normal NLR value is between 1-2. Patients with higher NLR had more moderate to severe stroke, worse functional outcome and increase risk of recurrence. There was a positive correlation between NLR and NIHSS score (P < .001), MRS (p value 0.001) and stroke recurrence. Discussion: NLR is a simple marker that reflects the ratio between neutrophils and lymphocytes and recent studies have supported NLR as a predictor of short-term mortality and severity in AIS patients. Very limited studies are done in India. Conclusion: The findings of this study suggest that NLR can serve as an indicator for predicting several aspects related to stroke.

Abstract ID: 512

Exploring the Significance of DWI-FLAIR Mismatch Analysis in Ischemic Stroke Patients Presenting Beyond the 4.5-Hour Window Period: A Comparative MRI Study

Eluka Reddy, Sandhya Manorenj

DM Neuro Pursuing, Other

E-mail: e.mounikareddy20@gmail.com

Background and Aim: Determining symptom onset time in acute ischemic stroke (AIS) is challenging, especially for patients with unknown or delayed presentation. This poses difficulties in administering thrombolytic therapy within the recommended 4.5-hour window. However, the DWI-FLAIR mismatch concept provides potential solutions for extending thrombolytic therapy eligibility in these cases. Methodology: This comparative MRI study explored the role of DWI-FLAIR mismatch in AIS patients presenting within 24 hours but beyond the 4.5-hour thrombolysis window. The study included patients who underwent MRI imaging within 24 hours of admission, excluding specific conditions. Demographic, risk factor, and National Institutes of Health Stroke Scale (NIHSS) data were collected. Results: Out of the initial 52 patients diagnosed with AIS, MRI imaging could be conducted in 34 cases. The mean age was 51.94 ± 10 years, with a majority of males (76.47%). Anterior circulation involvement was more common (64.71%) than posterior circulation involvement (35.29%). The most prevalent risk factor was hypertension (88.23%), followed by diabetes (58.82%). The mean time from symptom onset to MRI imaging was 11.24 hours ± 4.88 hours. Among the analysed cases, 12 (35.39%) exhibited a DWI-FLAIR mismatch pattern, distributed across different time windows. Discussion: The presence of a DWI-FLAIR mismatch pattern in AIS patients beyond the thrombolysis window has treatment implications. Among the analysed cases, 35.29% exhibited a DWI-FLAIR mismatch pattern, suggesting potential eligibility for thrombolytic therapy. However, the study’s limitations warrant further research to validate the findings and explore the clinical implications of the DWI-FLAIR mismatch concept. Conclusion: The study demonstrates that the presence of a DWI-FLAIR mismatch pattern in acute ischemic stroke patients presenting beyond the thrombolysis window suggests potential eligibility for thrombolytic therapy. Further research is needed to validate and explore the clinical significance of this finding.

Abstract ID: 513

Clinical, Laboratory and Radiological Profile of Patients with Embolic Stroke of Undetermined Source – A Bispective Observational Cohort Study

Keerthi Gopu

KIMS Hospital, Hyderabad, Telangana, India

E-mail: kirthy.reddi.g@gmail.com

Background and Aim: Upon completion of the workup for stroke, etiology cannot be identified in about 1/3rd of the patients, with ESUS accounting for around 50% of these cryptogenic strokes. This study is done to identify characteristics of ESUS in our hospital. Methodology: This is a single centre study conducted in a tertiary care hospital. All patients admitted in Neurology wards and OPD from August 2022 to April 2023 with a diagnosis of ESUS, > 18 years age, fulfilling the criteria of ESUS and willing to participate in the study were enrolled. Results: Of the total 270 patients admitted (IP) with a diagnosis of ischemic stroke, 25 (9.25%) patients were ESUS, 103 (38.1%) large vessel disease, 39 (14.4%) cardioembolic, 58 (21.4%) small artery occlusion, 33 (12.2%) incomplete workup cases and 12 (4.4%) strokes with other determined etiology. Out of 60 patients with ESUS (both from OP and IP), males - 39 (65%), mean age - 58.7 years. Hypertension (n=40, 66.6%) and dyslipidemia (n=35, 58.8%) were significant risk factors. Mean NIHSS score -3.69, mean mRS - 2.8. Limb weakness (n=31, 51.6%) and slurring of speech (n=19, 31.6%) were major presentations. On MRI Brain, single lesion (n=15, 25%), multiple lesions (n=45, 75%), multi-territorial (n=20, 30.3%), cortical (n= 46, 76.6%), subcortical (n=34, 66.6%), watershed/border zone (n=8, 13.3%) lesions observed. ELR for 2 weeks done in 22 patients, AF was not detected. TEE done in 30 patients, PFO (n=3, 10%), ascending aorta (n=3, 10%) and arch of aorta (n=3, 10%) atheroma, IAS aneurysm (n=1, 3%) were observed. Recurrence in 7 patients (11.6%) cases. Discussion: Majority of ESUS patients had mild strokes, low NIHSS score, multiple cortical and multi-territorial infarcts. Causes of ESUS identified were PFO, arch or ascending aorta atheroma, atrial cardiopathy. Conclusion: ESUS prevalence is 9.25% in our study and ELR of 2 weeks didn’t have much utility in diagnosing paroxysmal AF in our study.

Abstract ID: 514

Study of Carotid Artery Disease Pattern in Patients with First Ever Ischemic Stroke

Amreen, Lalitha Pidaparthi, Jmk Murthy, Subhendu Parida

DNB Neuro Pursuing, Other

E-mail: amreen.33@gmail.com

Background and Aim: This study aims to evaluate carotid artery disease patterns, both intra-and extracranial, in patients with first-ever Anterior circulation ischemic strokes (ACiS), using CT angiography. Methodology: This is a prospective observational study was conducted in a tertiary care hospital between February 2021 and January 2023 with the Institute Ethics Committee’s approval. We included all the consecutive patients with first-ever ACiS aged above 18 years presenting within one week of stroke onset. Demographic details, risk factor profile, neurologic findings, stroke subtype, and MR infarct patterns were documented in pre-designed proforma. Carotid disease patterns were classified into ICAS, ECAS, and both based on findings on CT angiography. In addition, the degrees of stenosis and asymptomatic disease patterns were also documented. Further, we categorized patients by age into 45 years and below and patients above 45 years. Results: Of the 578 (80.50%) patients with ischemic stroke, 74 (36.36%) had first-ever ACiS. The mean age was 59.91 years. 33 (44.59%) had ICAS, 25 (33.78%) had both ICAS and ECAS, and 16 (21.62%) had ECAS. A significant number of patients with ICAS had hypertension whereas patients with ECAS had dyslipidemia . The contralateral asymptomatic carotid disease was seen in 20.27% of patients. In our study, 12.16% of patients were young adults for whom smoking, alcoholism, homocysteinemia, and obesity were the common risk factors. Discussion: The mean age was 59.91 (27-84) years, similar to studies from India and South Asia, but lower than the reported age from the developed countries. Most of the patients presented with motor weakness (87.83%) followed by language (35.13%) and speech (12.16%) involvement. In our study the stenosis was labeled as intracranial when the lesion was distal to the ophthalmic artery after piercing inner dura similar to the studies by Suh et al. and Gorelick et al. In our study even though the intracranial atherosclerosis was the most common stroke subtype, the proportion is less compared to other Asian studies mostly because of different criteria used to identify intracranial carotid artery in different studies. Conclusion: This study showed ICAS was the common stroke subtype in this study population in South India, similar to other Asian countries. In addition, the frequency of extracranial carotid disease was also high.

Abstract ID: 515

TOAST Classification and NIHSS Score in Acute Ischaemic Stroke in a Tertiary Care Hospital – A Prospective Study

Abbigari Vardhan

DM Neuro Pursuing, Other

E-mail: harsha.abbigari3@gmail.com

Background and Aim: Stroke is a heterogeneous disease with more than 150 known causes. Categorization of subtypes of ischemic stroke has considerable study, but definitions are hard to formulate and their application for diagnosis in an individual patient is often problematic. Advances in diagnostic technology have allowed us to identify the potential underlying causes of stroke in patients. The fundamental goals of ischemic stroke classification are to make a correct diagnosis, enable prompt treatment, and predict future risks in subgroups of certain discrete features. Methodology: It is a prospective observational study to assess the categorisation of acute ischemic stroke patients treated at the Neurology Department of Madurai Medical College, Madurai , from October 2021 to September 2022. Results: In our study, 250 ischemic stroke patients were consecutively included and classified according to TOAST. The assessment was supplemented with cerebral small vessel disease (SVD) score, based on Magnetic Resonance Imaging (MRI) . Markers of small and large vessel disease were present in all TOAST groups. Carotid stenosis and atrial fibrillation were associated with their respective TOAST groups (p = 0.023 and p < 0.001, respectively). We found no association between the SVD score and the small vessel occlusion TOAST group (p = 0.59). NIHSS score compared among patients with TOAST and severity has been assessed.An operational classification that contains quantification of each vascular pathophysiology in the individual patient is pivotal for future research and development of personalized medicine. Discussion: Stroke is a global epidemic and an important cause of morbidity and mortality. It is the second most common cause of death and may soon become the leading cause of death worldwide8 . The National Institutes of Health Stroke Scale (NIHSS) is a well-validated, reliable scoring system for use specifically with stroke patients. The National Institutes of Health Stroke Scale (NIHSS) can be used as a standard measurement instrument by physicians to evaluate the severity of a patient and outcome6,7. We had expected the SVD score to be associated with the SVO group. Thus, inclusion of additional vascular markers did not improve the TOAST classification for the individual patient. As expected, ipsilateral carotid stenosis _ 50% and atrial fibrillation were associated with the TOAST classification. The need for a classification system that includes all underlying pathophysiological mechanisms in the individual ischemic stroke patient has been raised in an Asia-specific context 9, but this need is general, especially in terms of research (e.g., into genetics and biomarkers), choice of treatments, and further development of personalized medicine. Conclusion: Cerebral SVD were present across all TOAST groups and objective measures of large and small vessel disease (carotid atherosclerosis, ABI, RHI and eGFR) were not associated to specific TOAST groups. Only strokes in patients with ipsilateral carotid stenosis _ 50% and atrial fibrillation, were associated with TOAST.

Abstract ID: 516

Peripheral Blood Neutrophil-to-Lymphocyte Ratio for Predicting the Prognosis of Hemorrhagic Stroke

Ruchika Tandon Tandon, Anadi Mishra, Vimal Paliwal, Sanjeev Jha

Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

E-mail: rtlib161080@gmail.com

Background and Aim: We explored the blood neutrophil-to-lymphocyte ratio (NLR) as a prognostic marker and its correlation with Modified Rankin Scale (mRS) score at 3 months following ICH. Methodology: The investigators calculated the NLR of 90 adult patients within 3 days of onset of stroke with evidence of hemorrhagic stroke in brain imaging and correlated it with in-hospital mortality and mRS at 3 months following stroke. Results: Out of 90 individuals, there were 54 (60%) males and 36 (40%) females. The mRS score at 3 months and in-hospital death significantly related to the admission NLR ratio. Conclusion: A high NLR ratio predicted worse outcomes in terms of mortality, morbidity at 3-months following haemorrhagic stroke. Hence, NLR can predict morbidity and mortality following an acute haemorrhagic stroke.

Abstract ID: 517

Electrophysiological Evaluation of Subclinical Neuropathy in Diabetes Patients

Abbigari Vardhan

DM Neuro Pursuing, Other

E-mail: harsha.abbigari3@gmail.com

Background and Aim: Diabetic neuropathy is defined as the presence of symptoms and signs of peripheral nerve dysfunction in diabetes after exclusion of the other causes .Diabetes is the leading cause of peripheral polyneuropathy in developed countries2 . In general ,to define diabetic neuropathy ,should be based on symptoms , objective signs and EDX confirmation .Nerve conduction study is widely used for the diagnosis of diabetic neuropathy. Nerve conduction abnormalities exist in subclinical stages of neuropathy that can be detected ata an early asymptomatic stage by various electrodiagnostic test3 .Because peripheral nerves have the capability to regenerate ,ealy diagnosis and timely intervention will reduce the morbidity in diabetes . This study is used to evaluate the electrophysiological profile in type 2 diabetes patients for the identification of subclinical neuropathy . Methodology: The study is conducted at government Rajaji Hospital ,Madurai from April 2021 to April 2022 . 30 patients diagnosed as diabetes ,who were asymptomatic regarding neuropathy attending the diabetology outpatient department are examined at neurology out patient department . Results: Regarding duration of diabetes ,majority of the patients were between 2-5 years ( 50 %) .The next group to follow s < 2 years duration (33.3% ) and finally > 5 years duration ( 16.7 % ) . Out of 30 cases , 40 % cases showed abnormal Median –CMAP . Discussion: Diabetic neuropathy is one of the complication that contribute to the morbidity of such diabetic patients .There are evidence stating that earlier subclinical diabetic neuropathy diagnosis can result in fewer diabetic. Conclusion: The results of the present study done in diabetics without complications with duration of diabetes, indicate that there is a significant impairement of mtotr and sensory nerve parameters even though they are asymptomatic .

Abstract ID: 519

Finger Drop Sign and Gower Sign in a Quadriparetic Child

Rajarshi Chakraborty, Rajesh Verma

King George’s Medical University, Lucknow, Uttar Pradesh, India

E-mail: satyalung@gmail.com

Background and Aim: Quadriparesis is an important neurological disorder in clinical practice. Childhood quadriparesis can involve acute flaccid paralysis (Guillain Barre Syndrome), myelitis, muscular disorders, etc. Finger drop sign and Gower sign are distinct entities in clinical neurology Methodology: This 12 year-old-boy presented with acute onset quadriparesis of 8 days-duration. He complained of ascending weakness of all four limbs symmetrically. He was unable to get up from squatting position without help. His general examination was unremarkable. Nervous system examination showed a normal higher function, no cranial nerve involvement with normal sensory and cerebellar finding. Motor examination revealed symmetric quadriparesis with Gower sign, Finger drop sign, generalised areflexia and flexor plantar response. Other system involvement was negative. Results: He was worked up in the line of acute motor quadriparesis with distal and proximal involvement. His nerve conduction study showed axonal pattern of Guillain Barre Syndrome (GBS). There was albuminocytological dissociation in cerebrospinal fluid as well. He was treated conservatively as he showed spontaneous recovery. He recovered completely in 1month of follow-up. Discussion: He was worked up in the line of acute motor quadriparesis with distal and proximal involvement. His nerve conduction study showed axonal pattern of Guillain Barre Syndrome (GBS). There was albuminocytological dissociation in cerebrospinal fluid as well. He was treated conservatively as he showed spontaneous recovery. He recovered completely in 1month of follow-up. Conclusion: Finger drop sign and Gower sign can be observed in GBS rarely. Awareness about such distinct signs in neurology should be further advocated.

Abstract ID: 520

Study of Clinical and Immunological Profile of Primary Demyelinating Disorders of Central Nervous System in North East India

Papori Borah, Nidhin George

DM Neuro Completed, Other

E-mail: paporiborah31a@gmail.com

Background and Aim: Demyelinating disorders of CNS are characterized by inflammation and selective destruction of central nervous system (CNS) myelin. In spite of advancements in diagnostic techniques, studies on Indian population are rare. The current study aims to document the clinical and immunological profile of primary demyelinating disease of CNS in patients presenting to a tertiary care hospital. To study the clinical manifestations and immunological profile of patients of primary CNS demyelinating disorders and categorizing them into its various types viz. multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis (ADEM). Methodology: An observational study was conducted for 1 year May 2022 to April 31 2023 including old and newly diagnosed cases and detailed evaluation of clinical and immunological profile was done. Results: Of the 32 patients included, 19 (59.2%) were cases of NMOSD (18 patients were Aquaporin 4 antibody positive and 1 patient was Anti MOG (myelin oligodendrocyte) positive), 10 (31.25%) of MS, 3 (9.3%) of ADEM. 25 were female and 7 were male (ratio 3.57 :1). Among the clinical manifestations, motor abnormalities (87.5%), visual loss (60%) bladder dysfunction (45.5%), were the most common presentations. In MS, the most common manifestations were motor dysfunction followed by sensory symptoms, optic neuritis (mostly unilateral), and bladder dysfunction. In NMOSD, the most common clinical manifestations were motor dysfunction, followed by bladder dysfunction, optic neuritis and sensory abnormalities. In ADEM, most common manifestations were headache, motor abnormalities, altered sensorium, and visual loss. Discussion: Similar study was done in Karnataka showed MS more common than NMOSD.But our study shows NMOSD is more common. Conclusion: Demyelinating disorders of the central nervous system are common among younger age group females. A wide spectrum of clinical presentations in various disorders of primary CNS demyelination was found with maximum patients presenting with motor dysfunction, sensory disturbance and optic neuritis.

Abstract ID: 521

Serial Electrophysiological Study and Clinical Outcome in Patients of Guillain Barre Syndrome

Amrit Chattopadhyay, Shreyasi Banerjee

Kolkata Medical College, Kolkata, West Bengal, India

E-mail: ursrit@gmail.com

Background and Aim: The diagnosis of Guillain-Barre syndrome (GBS) depends on clinical criteria supported by electrophysiological studies and CSF findings. Our aim was to study the serial clinical and electrophysiological profile and its relation to outcome of GBS. Methodology: This observational study was conducted in Department of Neurology, Medical College and Hospital, Kolkata. All patients with GBS who were admitted in Neurology department from January 2022 to January 2023 were recruited. Detailed clinical profile and electrophysiological profile at appropriate interval (Admission and 1,3,6 months after disease onset) analyzed using Rajabally criteria for GBS subtype diagnosis. Results: Among total 22 (n) patients, after serial electrophysiology 6 patients had diagnostic change in sub groups occurring within 4 weeks between two NCS. Finally, 10 patients each were suffering from AIDP and AMAN, 1 AMSAN and 1 AMAN-RCF variety. DML progressively decreased whereas DMA and CV progressively increased and took nearly 6 months to normalize. 60% had sensory involvement electrophysiologically at admission of which 70% patient had demyelinating character in motor NCS. Those patients who had low DMA (<50% of LLN) at admission took more time to recover than those who had value above 50%. Discussion: During presentation AIDP was the major subgroup, which however became equal with AMAN group during final classification, which is different from western countries where AIDP is the predominant pattern. Not a single patient of axonal group had sural sparing sensory involvement. Most patients (86.36%) improved and most of them could walk independently within one month. Axonal variety took longer duration to recovery then demyelinating variety. Conclusion: Serial electrophysiological study is helpful to ascertain correct GBS subtype diagnosis for assessing prognosis. Degree of DMA reduction may predict poor outcome. Clinical recovery is much earlier than electrophysiological improvement and motor parameter improved earlier to or simultaneously with sensory.

Abstract ID: 522

Brain Ain’t Immune to Autoimmune – A Case Series of Auto Immune Encephalitis

M. V. Sucharitha, Raghunandan Nadig, Grk Sarma, Thomas Mathew, Sagar Badachi, Sonia Shivde, Akshatha Huddar, V. Sindhu, Surabhi Garg

St. Johns Medical College, Bengaluru, Karnataka, India

E-mail: sucharithavasu91@gmail.com

Background and Aim: Autoimmune encephalitis is among the most common form of encephalitis of noninfectious etiologies accounting for 20% of the encephalitis cases.To describe the clinical profile, treatment strategies and outcomes in patients diagnosed with Autoimmune encephalitis. Methodology: It was a retrospective study done in a tertiary care center. Patients diagnosed with autoimmune encephalitis during 2018-2023 were included. Demographic details, clinical presentation, laboratory investigations, radiological. Investigations and treatment modalities was analyzed and descriptive statistics applied. Results: A total of 40 cases were analyzed. 24 Were diagnosed to have sero positive autoimmune encephalitis, 14 were sero negative. Most common presentation was seizure seen in GTCS was the commonest type of seizure. Behaviour disturbance was mostly in the form of aggressiveness, impulsive behaviour, confusion. Most common finding was medial temporal lobe hyperintensity seen in 54% of patients. NMDA was the common antibody detected followed by LGI-1. 83% received IVMP as first line of treatment,13% received IVIG as first line, and 4% recieved PLEX. Majority of the NMDA patients required prolonged ICU care and hospitalization. Discussion: A delay in presentation was observed in patients with NMDA encephalitis, which may be attributed to the late recognition of symptoms as they are mostly behavioural and cognitive and may be subtle to start with. CSF and serum should be tested in suspected NMDA encephalitis as serum can be negative in 14% of the cases. Most commonly used mode of immunomodulation was steroids to which there was significant response. 29% of patients required second line of immunomodulation, 70% of whom were NMDA positive. Conclusion: NMDA encephalitis may require aggressive and prolonged immunomodulation, hence close follow up of patients is essential. CSF studies to be mandated in case of AE.

Abstract ID: 523

Autosomal Dominant Ullrich Congenital Muscular Dystrophy: A Rare Case Report

Suranjana Basak, Sudesh Prabhakar, Amit Singh, Jeenendra Singhvi, Harpreet Kaur, Harpreet Mann

E-mail: suranjana_basak@hotmail.com

Background and Aim: There are multiple overlapping clinical syndromes with Collagen VI-related dystrophies (COL6-RDs) namely Betlem myopathy (BM) in the milder form of the disease, and Ullrich congenital muscular dystrophy (UCMD) as the more severe manifesting disease.There is phenotypic and genetic overlap between these two disorders. Methodology: We report a further case of AD-UCMD, confirming that dominant mutations are not as rare as previously believed in patients with UCMD. Results: A 9-year-old boy complaints of difficulty in walking and getting up from sitting position since 2years.On motor system examination, B/L lower limb muscles had generalised atrophy,flaccidity and hypotonia.Power at bilateral hip flexors and extensors 3 and hip abductors,adductors 4-,knee 4-. Ankle dorsiflexion and plantar flexors were weak bilaterally. Deep Tendon Jerks Normal except hyporeflexic knee and ankle jerks. Severe scoliosis noted. He had Total CPK 8000 IU/L, CKMB 601.6IU/L.Whole Exome Sequencing suggestive of Autosomal Dominant heterozygous nonsense in COL6A3. His genetic analysis for multiples ligation probe amplification (MLPA) tested negative for Duchenne, Becker’s, and Spinal muscle atrophy.Left thigh muscle biopsy reported as-Moderate increase in perimysial and endomysial connective tissue with fat infiltration and mild fibrosis.Many atrophic, regenerating, degenerating and hypertrophic fibres noted with few showing splitting Foci of myophagocytosis are seen.On IHC for muscle proteins,dystrophin shows variable expression with loss of expression in few myofibrils and reduced expression in few myofibres. Discussion: UCMD is characterized by muscle weakness,proximal joint contractures,hypermobility of distal joints with AR inheritance.BM is generally a mild and late proximal myopathy with finger flexion contractures with both AD,AR inheritance.Studies have shown that heterozygous de novo deletions in COL6A1, COL6A2, and COL6A3 cause both severe UCMD and mild BM. Conclusion: There are similarities between clinical and genetic phenotypes of AD and AR inheritance of UCMD.The severity of clinical manifestation of UCMD has been directly linked to COLVI mutation just as much in AD.

Abstract ID: 524

A Rare Case of MRI-Negative Myelitis, Seizures, and Cognitive Impairment as a First Manifestation of SLE

P. Kumar, Himanshu Shakya, Deepti Grover, Suman Kushwaha, Aldrin Dung Dung, Rajinder Dhamija

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi, India

E-mail: ashwin.larzish@gmail.com

Background and Aim: Myelitis in SLE is a rare manifestation seen in about 1-2% of cases only, and MRI-negative myelitis is rarer. The purpose of presenting this case is to highlight some unique features associated with neurological manifestations of SLE. Methodology: We present a case of a 27-year-old female admitted to the neurology ward at IHBAS, Delhi with generalized tonic-clonic seizures, decreased attention, acute onset spastic quadriparesis, loss of joint position/vibration in the lower limbs, and bladder symptoms, without any evidence of MRI brain and spinal cord imaging abnormalities. Results: The patients was diagnosed to have Systemic lupus erythematosus. It was the first clinical presentation of SLE as MRI negative myelitis which the white matter of the spinal cord (postero-lateral cord) and seizures, with mild cognitive impairment. Her SLEDAI-2K score was 24. She was started on levetiracetam, steroids (intravenous followed by oral steroids tapering), and mycophenolate mofetil. She had significant improvement in motor, sensory and bladder symptoms. Discussion: The unique points in our case were: 1) SLE Myelitis, presenting as a selective tractopathy 2) MRI-negative myelitis as a first clinical presentation of SLE 3) CSF hypoglycorrhachia can be a clue in such cases. 4) Only case in literature with seizure and mild cognitive impairment associated with MRI negative Myelitis 5) No other organ involvement prior to SLE myelitis 6) Significant recovery post treatment. Conclusion: Neurological manifestations may be the sole symptoms in a patient with SLE. “MRI negative myelitis with selective tract involvement” may be a distinct subtype of lupus myelitis. Central nervous system involvement (seizures and decreased attention) may be simultaneously present along with SLE myelitis.

Abstract ID: 525

Anti SRP Antibody Necrotising Autoimmune Myopathy: A Rare Case Report

Suranjana Basak, Harpreet Mann, Sudesh Prabhakar, Harpreet Kaur, Jeenendra Singhvi, Amit Singh

DNB Neuro Pursuing, Other

E-mail: suranjana_basak@hotmail.com

Background and Aim: Immune-mediated necrotizing myopathy (IMNM) or necrotizing autoimmune myopathy (NAM) represents a heterogeneous subset of myositis that is characterized by the presence of necrotic fibres on muscle biopsy along with elevated serum CK levels and abnormal electromyogram (EMG). Methodology: We report a patient with necrotizing autoimmune myopathy with positive anti-SRP autoantibodies,p-ANCA antibody positive,Anti Ro-52 antibody positive,Anti Centromere Antibody positive. Results: 47 years female complaints of multiple episodes of loose stools,fever since 2 months,weakness of all four limbs since 2 weeks.Weakness of all 4 limbs was insidious,rapidly progressive, characterised by difficulty in getting up from the chair, climbing stairs, lifting arms, combing hair. It worsened over next few days to the extent that she couldn’t switch positions in bed, couldn’t get up from squatting position. Also had difficulty in lifting her head from pillow. CNS examination normal except atrophy of bilateral quadriceps, hypotonia of all limbs. Bifacial muscle weakness, neck muscle weakness, truncal weakness, power 2/5 in all 4 limbs with weak grip, weak ankle movements. Both plantars-flexors. Labs:LeptospiraIgM+, ANA+ (1:320), Anti Ro-52Ab+, Anti Centromere Antibody+, p-ANCA +. Electromyography-prominent spontaneous activity in the form of positive sharps / fibs,short duration low amplitude MUAPs suggestive of myogenic involvement.Right Thigh MRI-muscle edema involving muscles of right thigh esp.adductor group with minimal myofascial planes likely myositis.Muscle biopsy-skeletal muscle tissue with viable and necrotic fibres randomly distributed and temporarily heterogeneous.Focal acute necrosis,myophagocytosis and regenerative muscle fibres seen.Features suggestive of Necrotising Myopathy.SRPAb+++.She was treated with Pulse Steroids,IVIG,MMF,Inj.Rituximab with gradual recovery and succumbed to acute myocardial infarction. Discussion: The clinical picture, pathological features, autoantibodies of IMNM differ markedly from antisynthetase syndrome and non-specific myositis. Muscle weakness, dysphagia, cardiac involvement are common. Statin use, malignancy, connective tissue disorders are known risk factors. IVSteroids is first line of treatment followed by IVIG, Azathioprine, Rituximab. Conclusion: Initial treatment for SRP myopathy usually includes corticosteroids and immunosuppression agent, added at the same time or within one month (depending on severity and response to treatment). For severe cases of SRP myopathy, rituximab should be added within six months if other strategies are failing.

Abstract ID: 526

Ovarian Teratoma and Teen Hysteria: A Case of Anti-NMDA Receptor Encephalitis

Degapudi Sruthi, S. Balasubramaniam, Uma Maheshwari, Arun Kumar

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: sruthidegapudi@gmail.com

Background and Aim: NMDA receptor encephalitis is the most common cause of autoimmune encephalitis and progresses from psychotic symptoms and cognitive impairment to seizures, movement disorder, autonomic instability and loss of consciousness. Methodology: A 19-year-old college going girl presented with a 15-day history of abnormal behaviour heralded by generalized tonic clonic seizures. Two days later she developed psychiatric symptoms which were described to have been hallucinations of being trapped in a forest fire. She began to use abusive language without provocation and developed insomnia. She was unable to recognise family members or familiar surroundings. Three days after the onset of symptoms, she became fully dependent and was unable to carry out activities of daily living. She was admitted in to the hospital on day 14 of illness. On examination, she was irritable, with intermittent orofacial dyskinesias and no neck stiffness. Considering the clinical presentation, encephalitis was suspected and detailed evaluation was performed to rule out infective and metabolic causes. Serum NMDA receptor antibodies were positive and were also present in high titres in the CSF. Magnetic resonance imaging of the brain was normal. EEG showed intermittent diffuse slow waves in bilateral hemispheres. High dose IV methylprednisolone for days and plasma exchange following which patient improved symptomatically. Tumour screening with ultrasound and CT abdomen revealed dermoid cyst in the left ovary measuring 2.9 cm x 2.7 cm with O-RADS-2 and patient was planned for surgery. Results: Ovarian teratomas are one of the most common gynecologic tumours among adolescent patients. Discussion: Anti-NMDAR encephalitis is a rare form of autoimmune paraneoplastic encephalitis. Conclusion: Early treatment is essential for favourable cognitive outcome in anti-NMDA receptor encephalitis associated with ovarian teratoma. Immunomodulatory therapies with corticosteroids, plasmapheresis and immunoglobulin infusion (IVIG), are first-line, as well as tumor removal.

Abstract ID: 527

Incidental Vestibular Schwannoma, Personality Change and Autoimmune Encephalitis – A Coincidence Which Delayed Diagnosis

Surekha Dabla, Naveen Ranga, Meghna Pawar

DM Neuro Completed, Other

E-mail: surekhadabla@yahoo.co.in

Background and Aim: A 55 yrs old gentleman presented with history of behavior change with background history of occasional opium intake only once or twice in last 7 days , Never had addiction in past . His MRI Brain showed B/ L Vestibular Schwannoma. Initially we had impression about opium addiction but Toxic Screen came negative. Then a possibility of acute psychosis with Vestibular Schwannoma was kept and he was treated with antipsychotics. But after 3-4 days of treatment his condition deteriorated, his paraneoplastic panel and Autoimmune encephalitis panel sent. Later PET Brain also confirmed Autoimmune Encephalitis. Patient received both Inj Methyl prednisolone as well IVIG for 5 days and he improved dramatically later discharged in Stable mental and neurological condition. Methodology: NA. Results: NA. Discussion: NA. Conclusion: NA.

Abstract ID: 528

Therapeutic Plasma Exchange in Immune Mediated Neurological Disorders – Single Centre Experience

Gayathri Petluri Umasankar, Umasankar Kalai

DM Neuro Completed, Other

E-mail: gayathri_petluri@yahoo.com

Background and Aim: Plasmapheresis or Therapeutic Plasma exchange (TPE) is useful in management of many acute immune mediated neurological disorders especially in situations where IVIG availability is limited due to cost or logistics. Methodology: We did retrospective analysis of the patient admitted in our icu for plasmapheresis . Among them 20 patients who underwent 86 sessions of TPE for various neurological emergencies were analysed. Plasmapheresis was performed by centrifuge plasmaperesis machine NIGALE (Sanum Sanguine ). Results: Among 20 patients, 11 had Guillain Barre Syndrome (GBS), 3 had Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 4 had myasthenic crisis ,1 with NMO and 1 with long segment myelitis (post COVID). Number of sessions of TPE range from minimum of 3 to maximum 5. Age of patients range from 24 to 76 years. Complications during TPE are allergic reactions like itching, urticaria (46 percent), paresthesia of limbs (11.6) , transient hypotension (4.6 percent ) and poor outflow in central vein (1.1). Clinical improvement started in all our patients post 2 sessions, all recovered at the end of 2 weeks of last TPE. Discussion: Both Intravenous Immunoglobulin and TPE are approved for patients with GBS, CIDP and myasthenic crisis. In our country plasmapheresis is performed either through dialysis machine using filter or centrifuge plasmaperesis machine. However TPE is safe and cost effective option for these patients. In patients with NMO early initiation of TPE reduces significant disability. In patients with generalized myasthenia planned for thymectomy , preoperative TPE, reduced the need for prolonged ventilator requirement postoperatively. Most common side effects noted are allergic reactions possibly due to fresh frozen plasma related (FFP) as it is commonly used as replacement fluid. One patient we used peripheral vein for TPE, as central venous catheter had poor flow. Conclusion: TPE is extremely safe and cost effective for treatment of immune mediated neurological disorders even in small cities.

Abstract ID: 529

A Curious Case of Isolated Complete Oculomotor Nerve Palsy

P. Kumar

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi, India

E-mail: ashwin.larzish@gmail.com

Background and Aim: Cranial neuropathies (CN) are one of the neurological manifestations of autoimmune diseases like SLE, Sarcoidosis, Rheumatoid arthritis (RA), Sjogren’s, Systemic Sclerosis. Isolated complete oculomotor involvement in rheumatoid arthritis has been rarely reported in the literature. Methodology: 32-year female, a known case of RA from the past 10 years on irregular treatment presented to us with left hemi-cranial headache of 2 weeks followed by complete ptosis and complete left oculomotor palsy (dilated fixed pupil) without other cranial nerve involvement. Results: The routine investigations were normal with raised ESR, RA factor and Anti CCP. Rest investigations including CEMRI Brain, MR Angiography of brain, CECT chest and abdomen, ANA profile, serum IgG4, and VEP was normal. CSF showed 10 cells with normal sugar and mildly raised protein. Patients was started on 5 days of IV steroids followed by oral steroids and methotrexate. Patient showed slow improvement with significant improvement after 4 months. Discussion: The common acquired causes of 3rd nerve palsy can be deciphered according to the site of the lesion. The localisation of unilateral complete third nerve palsy with pupillary involvement is between midbrain and the orbit, the most common cause of which is posterior communicating artery aneurysm. After extensive set of investigations, aneurysm, infections, compression were ruled out. Rheumatoid arthritis is not commonly reported to involve the third nerve. It affects CN II, V, VII, VIII, XI. The exact pathophysiology is not known but is attributed to the immune mediated vasculitis and or drug related complication. Therefore, a plausible explanation in our case was due to perineuritis of the 3rd nerve due to pupillary fibers involvement. The patient responded reasonably well to steroid and methotrexate treatment. Conclusion: We hereby report a rare case of isolated third nerve palsy in a known case of rheumatoid arthritis most likely due to perineuritis.

Abstract ID: 530

Use of Transorbital Ultrasound and Optical Coherence Tomography to Assess Disability in Multiple Sclerosis

Poornima Nair, B. Srikumar, Thomas Iype, M. S. Sunil

DM Neuro Completed, Other

E-mail: pnairbhavana@gmail.com

Background and Aim: Axonal injury appears to be an important contributor to disability in MS. Aims To study the -Association between Optic nerve diameter (OND),optic nerve sheath diameter (ONSD), Retinal nerve fibre layer (RNFL) thickness & Ganglion cell complex (GCC) thickness with EDSS -Diagnostic accuracy of TOS to predict disability compared to Optical Coherence Tomography (OCT). Methodology: A prospective observational study was done in 28 MS patients. The baseline data, details of disease duration, relapses were collected & baseline EDSS calculated. All patients underwent OCT & TOS to measure RNFL, GCC, OND & ONSD respectively. Result: Mean OND was 3.8 v/s( >2 ) 4.3 mm( 2) 4.9 mm( 2) 17.6 microm( 2) 51.9 microm(2. Discussion: The optimum cut-off values resulting in the highest sensitivity & specificity for detecting patients with EDSS >2 were 1.≤ 42 µm for GCC 2. ≤ 4.35 mm for OND 3. ≤ 4.62 mm for ONSD 4. ≤ 12µm for RNFL. Conclusion: RNFL, GCC, OND & ONSD were significantly reduced in the better eye irrespective of prior optic neuritis. RNFL & OND were found to be independent predictors of EDSS>2. TOS was found to be reliable & an economical alternative to OCT.

Abstract ID: 531

Tales of Triumph over Acute Psychosis

S. S. Muthukani, A. Madhuri

All India Institute of Medical Sciences, New Delhi, India

E-mail: mkani86@gmail.com

Background and Aim: Anti NMDAR Encephalitis It is increasingly being recognized as one of the common causes of encephalitis, but is frequently misdiagnosed especially in resource-constrained settings. With a simple test available to diagnose the disorder and prospects of good recovery following early immunotherapy. The disorder should be kept as a differential diagnosis in patients presenting with unexplained behavioral/psychiatric symptoms and progressive encephalopathy with movement disorders. Methodology: Study Setting : Apollo Main Hospital , Chennai . Study Design : Prospective study Study Duration : From May 22 -May 23. Results: Patients with anti-NMDAR encephalitis respond to treatment regardless of the severity of the disorder. Patients with a teratoma who are treated early in the course often respond to removal of the tumor and corticosteroids, IVIG, or plasma exchange. Discussion: Encephalitis has numerous and varied causes, but in resource-constrained settings, most cases are assumed to be infectious in etiology. Despite infections being a common cause, immune-mediated encephalitis is increasingly being recognized as a significant contributor to encephalitis cases (20-30%). Most frequent antibody associated encephalitis. Most common in young women and children who represent 80% of patients but also occurs is men and older patients (Dalmau et al., 2008). Characteristic clinical picture : facilitates diagnosis of antiNMDAR encephalitis. Patients experience a viral-like prodrome. Conclusion: Anti-NMDA receptor autoimmune encephalitis is often misdiagnosed as primary psychotic or substance induced disorder. High index of suspicion and work up for autoimmune encephalitis needed before labelling as pseudo seizures, someone with altered behaviour and loss of consciousness. We have pursued and identified para neoplastic cause of ovarian teratoma and had it been excised with good outcome.

Abstract ID: 532

Role of Early Rituximab in Treatment of NMS with NMDAR Encephalitis: Case Report and Review

P. Kumar, Sourav Hazra, Chirag Gupta, Suman Kushwaha, Aldrin Dung Dung

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi, India

E-mail: ashwin.larzish@gmail.com

Background and Aim: The neuroleptic malignant syndrome (NMS) is an idiosyncratic reaction to neuroleptics with overlapping features of Anti-NMDAR encephalitis. Despite prompt management of NMS in an Anti-NMDAR encephalitis permanent residual neurological sequelae can occur. Titulaer et al in their seminal paper emphasize that early initiation of immunotherapy impacts the outcome of the disease. We present a case and the literature review to iterate the need of early institution of second line immunotherapy to prevent permanent disabilities. Methodology: We present a case of a 18 year old boy who presented with NMS following antipsychotic initiation, diagnosed and treated for Anti MMDAR encephalitis. We also present a literature review of NMDAR presenting as NMS on Pubmed. We analysed the cases (n=13) with emphasis on the antipsychotics used, treatment received and residual disability in the cases. Results: Our patient was given multiple antipsychotics which lead to NMS. On diagnosis of NMDAR he was given first line (Steroids and IVIG) and second line (Rituximab, within 4 days) leading to complete recovery on follow up. Thorough review of literature showed 13 cases of NMS with NMDAR. 46% patients received atypical antipsychotics. 54% were on monotherapy. Only those who received second line (rituximab, n=1) or surgery (n=3) recovered completely (n=4/13, 30%). Discussion: NMDAR antibodies cause dopamine blockade, which may lead to NMS like symptoms. Typical antipsychotics and polytherapy may increase this risk by the same mechanism, however literature review revealed that atypical antipsychotics and monotherapy can lead to NMS. Poor GCS has negative co-relation with survival in patients of NMDAR. Therefore, NMS patients with NMDAR should be started on second line therapy or surgery without waiting for treatment failure of first line. Conclusion: 1. Atypical antipsychotics and monotherapy can cause NMS in NMDAR. 2. The case and literature suggests the role of early initiation of second line therapy in resolution of symptoms.

Abstract ID: 533

Series of Ideopathic Hypertrophic Pachymeningitis

Viresh Nashte, Chinmay Kumbhar, Rahul Kulkarni, Shripad Pujari, Vishal Deshpande, Rushikesh Deshpande

Deenanath Mangeshkar Hospital and Research Center, Pune, Maharashtra, India

E-mail: viresh.nashte28@yahoo.com

Background and Aim: Hypertrophic pachymeningitis (HP) is rare form of dural inflammatory disease that cause thickening of dura matter that can be idiopathic or secondary to conditions, like RA, syphilis, tubercular and cancer. Clinically presented with headache, cranial nerve defect, hydrocephalus, uncommonly CVST or can be asymptomatic There are few series of idiopathic subtypes have been reported. Aim: To report idiopathic Hypertrophic pachymeningitis with clinical and laboratory evaluation, course, treatment and correlation between clinical finding and MRI and to review literature on IHP. Methodology: Pachymeningitis was established based on contrast MRI and/or CSF finding. Retrospective case series of 9 patients, who had IHP by imaging studies Relevant clinical features, laboratory evaluation, contrast MRI treatment and outcome were documented in all cases. follow up MRI in some cases. Results: Main clinical presented were Headache (6 cases), Cranial nerve involvement (2 case), secondary CVST (2 cases), DOV (2 cases),diplopia (1 case) hydrocephalus (1 case) On initial MRI -location of abnormal enhancement of dura matter correlated with clinical finding. documented Routine lab, CSF, RPR, IgG4 level, ACE level were negative for identifiable etiology. one patient underwent dural biopsy to confirm diagnosis. All treated with steroid, 3 required Azathioprine (steroid sparing agent). Discussion: Common clinical presentation headache, cranial nerve defect, CVST, hydrocephalus . So high degree of suspicious of this disease should be kept in such clinical and MRI finding . MRI showed cortical thickening , basal thickening . with abnormal CSF,Note CSF WBC may not be highly abnormal, unlike Infective meningitis. Clinical radiological concordance like hydrocephalus for dorsal meningeal thickening and cranial nerve involvement for basal meningeal thickening seen. Conclusion: Diagnosis of HP established on clinical feature , cMRI or CSF examination. IHP is diagnosis of exclusion after ruling out auto immune ,inflammatory, infective causes. Definite biopsy may not needed in an all. Some patient may relapse /worsen after steroid tapering, required steroid sparing agent.

Abstract ID: 534

Neuropsychological Profile and MRI Correlates of Multiple Sclerosis

Vinayaka Yadav, Shantala Hegde, M. Netravathi, Manoj Kumar

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: vinayakavitthal@gmail.com

Background and Aim: Multiple sclerosis (MS) is a chronic, autoimmune disease of the central nervous system. In addition to physical health complications, deficits in neurocognitive functions, mood, and quality of life is also observed. Comprehensive neuropsychological evaluation and correlation with MRI findings become essential in treatment plan for this clinical population. Details of the pilot phase of a larger ongoing study are presented here. Aim was to examine neuropsychological and brain volumetric profiles in relapsing-remitting MS patients (RRMS) and to study the correlation between tested variables. Methodology: The sample comprised college educated female patients with RRMS (n=5). RRMS undergone structural volumetric MRI and were assessed on overall IQ, executive functions, anxiety, depression, fatigue and quality of life. Results: RRMS performed poorly on various neurocognitive tests compared to matched Indian normative data. Deficits on verbal-visual learning & memory and working memory (n=4), attention switching (n=3), full scale IQ ranged from falling in the category of borderline to dull normal (n=4). Mild to moderately severe depression, with moderate to high trait anxiety was reported (n=2). Mild to moderate fatigue was reported with impact on quality of life (n=3). Brain volumes for left putamen (Mdn=4084mm3) left accumbens (Mdn=481mm3) showed significant negative correlation with trait anxiety. Discussion: The neural subcortical structures have significant underpinnings in cognitive processing and information consolidation, with impact on emotional regulation. In view of very limited research in the given area, the current study contributes to a better understanding of neuropsychological consequences, mood, and quality of life in MS patients. Conclusion: Neurocognitive deficits and a significant negative correlation between trait anxiety and volume for different brain regions were recorded. The ongoing study with larger sample would shed further light in to establishing our hypothesis.

Abstract ID: 535

Rituximab in Rapidly Progressive Inflammatory Myopathy: Case Series

Gayathri Petluri Umasankar, Umasankar Kalai

DM Neuro Completed, Other

E-mail: gayathri_petluri@yahoo.com

Background and Aim: The patients with idiopathic inflammatory myopathies (IIMs) might respond poorly to first-line treatment with glucocorticoids and other immunosuppressive therapy. Here we share our experience of utility of anti CD20 B Cell antibody ,Rituximab (RTX) in our 3 patients of rapidly progressive inflammatory myopathy. Methodology: Case 1 55 year old diabetic female presented with progressive proximal muscle weakness and neck muscle weakness. She had elevated CPK (447U/L), muscle biopsy showed necrotizing myositis. PET CT showed extensive inflammation in all muscles. Myositis panel showed elevated SRP Ab, diagnosed with Immune Mediated Necrotizing Myopathy (IMNM) . She was managed with pulse methylprednisolone followed by oral steroids and methotrexate. Subsequently Rituximab was given 2 gm (500mg every 2 weeks ) Case 3 22 year female presented with h/o arthralgia for 2 years followed by progressive weakness of proximal muscles and neck muscles . She had elevated CPK (12530U/L), ANA and anti Sm antibody positive. Muscle biopsy showed inflammatory myopathy, diagnosed as Overlap Myositis She was managed similar to case 1 Case3 34 year female with weakness of proximal muscles ,neck muscle and bulbar muscles with rash over neck and digital ulcers. Evaluation showed elevated CPK , muscle biopsy showed inflammatory myopathy, Diagnosis Dermatomyositis . She was managed with steroids , Intravenous immunoglobulins followed by RTX. Results: All the patients showed improvement in power at 1 month post infusion and are on tapering dose of steroids. Discussion: Many patients with IIM respond poorly to first-line treatment with glucocorticoids and other immunosuppressive agents .Our case series highlights that RTX induction is beneficial in subset of patient based on clinical severity (bulbar and respiratory )and with specific myositis antibody profiles (AntiSRP) in reducing the disability. Conclusion: Our case series shows that Rituximab is effective and safe option in IIM patients poorly responding to conventional treatment.

Abstract ID: 536

The Panoramic Manifestations of Neurosjogren- A Single Centre Case Series

Sohini Chakraborty, Baikuntha Panigrahi, Ayush Agarwal, Rajesh Singh, Deepti Vibha

All India Institute of Medical Sciences, New Delhi, India

E-mail: sohinichakraborty84@gmail.com

Background and Aim: It is a systemic chronic inflammatory disorder characterised by lymphocytic infiltrates in exocrine organs. We are elaborating few cases with rarer manifestations, laboratory and radiological evidence suggestive of Sjogren’s and reporting treatment response. Methodology: Mr. S, thirty eight years old, with history of dry eyes, mouth, recurrent self-limiting episodes of acute flaccid quadriparesis, presented with paraesthesia over bilateral hand and feet, slippage of slippers without awareness and right foot drop since last one year. On examination he had bilateral weak handgrip, right foot drop (common peroneal nerve), graded pattern of sensory loss over bilateral lower limb. ANA, anti-Ro, Schirmer’s and work-up for renal tubular acidosis were positive. Conduction studies showed mononeuritis multiplex. Miss N, nineteen years old, with oro-ocular dryness, recurrent oral ulcerations, alopecia, polyarthralgia, presented with insidious onset progressive symmetric proximal followed by distal upper and lower limb sensorimotor weakness since eight months associated with pan-cerebellar signs, vertical nystagmoid eye movements, bilateral foot drop and large fibre involvement on examination. ANA, Anti-Ro, La, Schirmer’s were positive. Conduction studies showed axonal sensorimotor polyneuropathy. MRI Brain showed multiple hyperintense periventricular, subcortical white mater and brainstem lesions. Mrs. P, thirty-three years old, with history of spontaneous first trimester abortions, presented with insidious onset gradually progressive sensory ataxia, patchy sensory impairment, urinary urgency, incontinence, flaccid dysarthria and regurgitation of fluids for four years. On examination, apart from generalised hypo- areflexia, there was spasticity in bilateral lower limb, Holme Adie’s pupil, right seventh, ninth, tenth and twelfth cranial nerve involvement. ANA, Anti-Ro, La were positive. Imaging showed long segment non enhancing dorsal column hyperintensity and brainstem lesions. Results: All three patients improved on steroid, cyclophosphamide and IVIg. Discussion: Prevalence and type of central and peripheral nervous system involvement in Sjogren’s remains controversial, making the diagnosis challenging. Conclusion: Treatment is decided on an individual basis according to disease severity.

Abstract ID: 537

Clinical profile, Treatment and Outcome in Patients with Symptomatic Internal Carotid Artery Occlusion in a Tertiary Care Centre

Sanjo John, N. Shobana, M. Sacratis, V. Sadeeshkumar, C. J. Selvakumar, M. Joby Thomas

DM Neuro Pursuing, Other

E-mail: sanjokalathil24@gmail.com

Background and Aim: Atherosclerosis is the most common cause of ischemic stroke and 6-15% of all acute ischemic stroke is due to internal carotid artery occlusion. Ischemic stroke with ICA occlusion has high risk of in hospital recurrence, early death and poor outcome. In the absence of best medical treatment, high incidence of stroke recurrence has been reported. Aim: This study aims to determine clinical profile ,treatment of patients with symptomatic internal carotid artery occlusion and their outcome after 6 month followup. Methodology: This is an observational study on Individuals between 40-60 years of age, diagnosed with acute ischemic stroke with internal carotid artery occlusion more than 50%. 25 patients were analyzed and was given best medical therapy as per guidelines and their clinical profile and treatment and outcome after 6 months was analyzed. Results: Out of the 25 cases majority were males (92%). Mean age was 54.4 years ( range : 35-72) .The mean duration of hospital admission was 1.64±4.99 days . Mean MRS score at time of discharge was 2.52 and 6 months follow up score was 1.28.21 patients had acute infarct and 4 had subacute infarct.28 % had diabetes and 32 % had hypertension and CAD . In Doppler imaging majority ( 24%) had complete Right ICA occlusion. All patients were treated with dual antiplatelet for 3 months .Upon 6 months follow up 24 cases had improved MRS and one had recurrent stroke. Discussion: More than 50% occlusion of internal carotid artery is found in 15-20% of patients presenting with ischemic stroke. Best medical therapy has lowered atherosclerosis related morbidity and mortality. Conclusion: This study demonstrates the effectiveness of dual antiplatelet therapy and high dose statin therapy in patients with stroke with internal carotid artery occlusion . Follow up and adherence to medical therapy is needed to prevent stroke recurrence or complications.

Abstract ID: 538

A Cross Sectional Analytical Study to Estimate Prevalence of Poor Functional Outcome in Posterior Circulation Stroke Cases

Pranav Mehta, Sreehari Dinesh, Gargee Pore

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

E-mail: drmehtapranav@gmail.com

Background and Aim: Functional dependence or independence or mortality are different outcomes of any stroke. The outcomes of posterior circulation stroke (PCS) are varied and there is a paucity of reviews with no definite conclusion. Thus, we undertook this study with the aim to study the prevalence of poor functional outcome of PCS patients at the end of 3 months as assessed by modified Rankin Scale (mRS) and the risk factors associated with them. Methodology: In this prospective and retrospective observational analytical study, acute PCS patients was evaluated. NIHSS and Post NIHSS was calculated on admission for prospective cases. Posterior Circulation vascular anatomy was characterized. Functional outcomes was assessed at Day 90 post onset of stroke using mRS. Results: Among the 150 patients studied in total, mean age was 60.4 years and 68% were male. 54 people had Hypertension, 49 had type 2 DM and 32 had both amongst most common risk factors. Most common vascular anomaly noted was ectasia of vessels. Functional outcome analysis at the end if 90 days showed almost 36% of patients had mRS ≥3. Discussion: PCS have been generally considered to have a greater mortality and morbidity than Anterior circulation stroke (ACS). The outcome is dependent on the severity of the neurological signs, location and extent of infarction and mechanism of ischemia. The immediate mortality rate in PCA stroke is 3–4%, and the major disability is 18% (NEMC-PCR). Conclusion: Our study showed poor functional outcome in almost 36% patients at the end of 90 days (mRS > 2). For a country where stroke is a leading cause of mortality, very few studies of PCS have been performed. Hence there is a need for further research in this field, especially in Indian Population.

Abstract ID: 539

A Retrospective Study on the Clinical Characteristics of Idiopathic Double Seronegative LETM in a Tertiary Care Hospital in Eastern India

Ankur Banik, Indranil Dutta, Amrit Chattopadhyay, Debsadhan Biswas, Srabani Ghosh, Sandip Pal

Kolkata 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.Although LETM is classically related to AQP4-Ab,of late,MOG-Ab & a number of other neurological & systemic causes are recognised.However,in many patients the diagnosis remains elusive & there is no management consensus available for this group. We aim to study the clinical profile of this idiopathic double seronegative (DSN) LETM. Methodology: This retrospective study was conducted at Neurology department,Medical College,Kolkata.Details of all LETM cases from September 2021 to December 2022 were obtained and the idiopathic DSN patients were studied in terms of their demographic,clinical,radiological & therapeutic profile. Results: Amongst a total of 37 LETM patients,idiopathic DSN was the most common cause in 15 patients (40.54%),while 12 patients had NMOSD (32.43%).Amongst the 15 idiopathic DSN patients, as high as 66.67% were males.The predominant presentation was paraparesis with dorsal spinal cord being most commonly affected and there was no correlation between the severity and the number of segments involved.Central cord involvement was the most common pattern of radiological involvement (66.67%).Optic nerve and brain involvement were absent in all patients. Discussion: Although NMOSD is the most commonly perceived devastating causal association of LETM, Idiopathic DSN LETM was the most common cause among all cases of LETM in our study. It affected males more with great variation in clinical presentation and severity. In the absence of definite management guidelines, various therapeutic strategies were tried with variable response. Conclusion: idiopathic DSN LETM is very common in clinical practice with extremely variable presentation. They require further research to arrive at a much needed management consensus.

Abstract ID: 540

A Retrospective Study on Outcome of Thrombolysis in Patients with Acute Ischemic Stroke in Tertiary Care Hospital

Salijamala Hari, Manivannan

DM Neuro Pursuing, Other

E-mail: sahasra.moni@gmail.com

Background and Aim: Stroke is a major rising concern worldwide. Thrombolysis has shown a new light for treatment of acute ischemic stroke with good results especially if used in 4.5 hours of the window period.To determine demographic profile, co morbidities, clinico radiological profile, efficacy and safety of intravenous alteplase (0·9 mg/kg) in study population. Methodology: AIS patients who underwent thrombolysis with alteplase. Data collection was retrospective and analysed from January 2021 to December 2022.Primary outcome was measured with NIH Stroke Scale (NIHSS) at admission and at discharge; and secondary outcome was measured with modified Rankin score (mRS) and NIHSS at three months. Results: We collected data of consecutive 51 patients who received alteplase for thrombolysis. Mean age was 53.84 years with majority of them being males.The most common risk factors were alcohol, smoking, hypertension, tobacco and diabetes, dyslipidemia followed by CAD. In our study mean window period was 186.6+/- 0.64 minutes . Primary outcome achieved 35 patients (68.63%) and poor outcome in the remaining16 patients (31.37%). Discussion: Thrombolysis within time has shown an improvement in quality of life. In our study average age was 53.84/-10.90 years males predominantly underwent thrombolysis .mean window period was 186.6 +/-0.64 minutes.We found no significant correlation when we evaluated those arriving within 2.5 hours and those arriving within 2.5-4.5 hours and mRS scores.The mean NIHSS score at admission was 8.92 ± 3.78.The mean mRS after three months was 2.22 ± 1.05 The primary result was 68.63%, which was more than 65% in the study by Padma et al. 16 Intracerebral hemorrhage was seen in 4 patients (7.84%). Conclusion: Most patients who took alteplase achieved their primary goal, which was of reduced morbidity and mortality, In our study, NIHSS and mRS scoring scales were crucial in determining the morbidity and outcome.

Abstract ID: 541

Temporal Trends, Clinical Profile and Outcome of Patients Receiving Intravenous Thrombolysis in Acute Ischemic Stroke

Sandhya Manorenj, Mounika Reddy

DNB Neuro Completed, Other

E-mail: drsandhyamanorenj@gmail.com

Background and Aim: Background: Stroke is the second most common cause of mortality .Intravenous thrombolysis is the approved treatment in patient with acute ischemic stroke (AIS) with in window period of 4.5 hours. Health outcome data of thrombolysis in patients with acute ischemic stroke in real life-settings in India are scarce. Aims &Objective: To explore the temporal trend, clinical profile and outcome of patient with AIS who received intravenous thrombolysis. Methodology: Ambispective study observational study conducted for a period of 5 years at hospital located in Charminar region of Hyderabad. Patients with acute ischemic stroke coming with in window period of 4.5 hours who received intravenous thrombolysis were studied. Neurological deficit were assessed using NIHSS, and functional disability using MRS scale. Results: A total of 3958 patients were diagnosed to have AIS. Among them only 126 patients (3.18%) presented to our hospital within window period. Out of 126 patients, 56 patients (44 %) underwent intravenous thrombolysis (IVT) within 4.5 hours of the window period. Among them 49 (87.5%) patientsreceived tenecteplase and remaining alteplase (12.5%). Discussion: We found a trend in improvement in arrival to the hospital early as years passed from 2018 to 2022. Average arrival time was 72 hours in year 2018, 48 hours in year 2019, 28 hours in 2020, 18 hours in 2021 and 12 hours in 2022 among patients with AIS. Mean age was 55.56±11.05 years. Average door to needle time was 70 minutes.Mean NIHSS score at presentation was 12 (NIHSS range 4 - 19). Mortality was noted in six patients Favorable MRS (0-2) at 90 days was observed . Conclusion: Though there is encouraging trends in IVT over the years indicative of increasing community awareness of stroke,still we are far behind as only a minimal percentage of patients with AIS reach with in window period of 4.5 hours of symptom onset.

Abstract ID: 542

Factors Delaying Door to Needle Time in Thrombolysing Acute Ischemic Stroke and the Strategies to Minimize the Delay

Priyanka Kashyap, Akanxa Yadav

All India Institute of Medical Sciences, Bhopal, Madhya Pradesh, India

E-mail: dr_pvinod@yahoo.co.in

Background and Aim: Stroke in window period suffers various hurdles in achieving successful thrombolysis. These measures if identified sincerely and rectified improve the effective thrombolysis both qualitatively and quantitatively. Moreover, improves the stroke outcome and quality of life in stroke patients and thus decreasing the society burden. Aims: The authors aimed to identify the barriers faced in achieving door to needle time in 1 year period (December 2021 to December 2022) in Neurology department in AIIMS Bhopal. Methodology: Setting and study design: Prospective observational study at AIIMS Bhopal for a one-year period Materials and Methods: Patients with AIS reaching hospital within window period were identified. The eligibility for thrombolysis was assessed, their clinico epidemiological characteristics were noted. Types and severity of stroke were identified, their baseline NIHSS, NIHSS at 1 hour, 24 hours, at discharge, and modified Rankin Scores (mRS) at 0, 1 and 3 months were recorded in a structured proforma. The different time matrix indicating door to needle time was fractionated and sub grouped and barriers were identified. Statistical analysis used: Categorical variables were presented as proportions and continuous variables as mean ± standard deviation (SD). Non-parametric tests were used for analysis. Continuous and categorical data were analyzed using an independent sample t-test and chi-square (or Fisher exact) tests, respectively. A value of p <0.05 is considered statistically significant for all tests. Results: Results: This study is the first study from Central India to study the door to needle time (DTNT) of stroke patients and the barriers or factors resulting in delay in thrombolysis. Out of the 300 patients, 21 patients (8.6%) were thrombolysed. Males outnumbered the gender distribution ,13:8 (61.91%:38.09%) and the mean age was 60.81 years. Hypertension (57.9%), diabetes mellitus (36.8%) and smoking (26.3%) were the most common risk factors. Time matrix included mean onset to door time, door to neurology call and door to imaging and door to needle time were noted. Out of total 21 patients, 8 were female (38.09%) and 13 were male (61.91%) and ranged from 21 years to 85 years (mean age of 60.81 years). Two-third (66.67%) of our patients (14 out of 21) reached our center from within 10 km distance radius highlighting the accessibility of centers providing thrombolysis facility. Most were anterior circulation stroke, MCA territory and only one patient had posterior circulation stroke. Dual territory anterior stoke was also rare in our study. complete MCA involvement (66.67% of total) was most common, the mean NIHSS was 12 (2-22). Two had disabling stroke with score of 2 and 4 of our patients presented with wake-up stroke who received thrombolysis. The mean time of onset to presentation to door (Dt) was 115.48 minutes (40 minutes to 210 minutes, and to recognition of stroke by emergency department resident was 10.48 minutes (5 minutes to 20 minutes) and further mean time from ictus confirmation by neuro-resident was 14.76 minutes (5 minutes to 110 minutes). Door to imaging (DI) varied from 30 minutes to 50 minutes (mean time 39 minutes) and then mean DTNT (door to needle time) was 106.67 minutes (65 minutes to 140 minutes). 18 patients received Tenecteplase as being cost effective and easy availability near the center. All our patients showed reduction in mean NIHSS scores from 12 to 9.88. Successful thrombolysis achieved in 5 (23.8%) and eleven out of 21 patients (52.38%) showed no improvement in NIHSS at 24 hours. 4 patients died because of sepsis and upper gastrointestinal (UGI) bleed. Three patients showed haemorrhagic transformation in repeat NCCT at 24-hour post thrombolysis and were all managed conservatively (asymptomatic Intracranial Haemorhage). Most common barrier for delay in effective DTNT was drug procurement from outside as not available with emergency unit and relatives counselling and when time matrix fraction was analyzed patients who reached early had longer DTNT than those who came late as decision was quick in them also the patients who had high NIHSS had lesser DTNT than who had low NIHSS. Discussion: Identifying factors delaying door to needle time in thrombolysis of AIS can help the clinician to specifically note the barrier points and to frame a module including rectification and enablers of reducing the golden door to needle time that can help in increasing successful thrombolysis. Conclusion: Thrombolysis in window period is a must for today’s world as it will reduce the disability resulting from stroke and improve the quality of life thus will decrease the burden in the society. Now a days cost-effective and safer thrombolytics to be made available and awareness programme in prehospital settings at common public and at primary physician level to be started and at in hospital settings establishing stroke code stroke alarm units to be started in hospital. Regular training programmes among medical paramedical staff, with technical staff in neuroimaging will help to achieve the target of thrombolysing within window period. Regular assessment of the barriers and enablers to be looked for. One important strategy at hospital level is to make the drug available at the EM which can effectively reduce the window period.

Abstract ID: 543

Seated Tadasana as a Rehabilitative Measure in Post Stroke Patients – Study of 50 Patients

R. Pazhani, P. Bavani

DM Neuro Completed, Other

E-mail: pazran@hotmail.com

Background and Aim: In post stroke patients, apart from therapeutic measures rehabilitation is important. Yogasana is a cost effective rehabilitative measure. The upper limb shoulder and finger stiffness are main difficult areas of correction. In this study seated Tadasana was taught to the patients. Fifty patients were taken for this study and the results were analysed 6 months later. Methodology: Fifty post stroke patients, with in 30 days of stroke were taken for this study. Thirty are male, twenty are female. Forty patients had ischemic CVA and 10 had hemorrhagic CVA. Patients who had predominant upper limb weakness were taken for this study. Thirty two had right upper limb weakness and 18 had left upper limb weakness. All had subcortical CVA. Seated Tadasana was taught to the patients and the attenders. The patients were followed up once a month for 6 months for study purpose. Results: The outcome was analysed after 6 months. The power improved significantly in all the patients. Forty five patients shoulder movements were full, as the seated Tadasana was initiated early. There was no finger stiffness and pain at the end of 6 months. As the patient was encouraged to do the seated Tadasana as many time as possible in a day, it made significant difference. The remaining five patients who started late, developed finger and shoulder stiffness already, but relatively they done better. Discussion: Seated tadasana relaxes the mind and body. It is ideal for meditation.Improves posture and build core strength. It helps to align neck, shoulders and spine. In this study, seated Tadasana helped the patients as it can be done by themselves with less supervision. Conclusion: The seated Tadasana is simple to do, cost effective and has clinical benefit. It can be included as part of the rehabilitative measures in post stroke patients.

Abstract ID: 544

Transforming Stroke Care: Evaluating the Impact of Mechanical Thrombectomy through Systematic Review and Network Meta-analysis

Pradeep Kumar, Manabesh Nath, Shubham Misra

All India Institute of Medical Sciences, New Delhi, India

E-mail: pradeepguptaneuro@gmail.com

Background and Aim: Acute ischemic stroke is a devastating condition with limited treatment options. Mechanical thrombectomy has emerged as a game-changer in stroke management, but there is a need to consolidate the existing evidence and explore its comparative effectiveness. This systematic review and network meta-analysis aimed to evaluate the efficacy and safety of mechanical thrombectomy as compared to other interventions for acute ischemic stroke. Methodology: A comprehensive literature search was conducted across multiple databases to identify relevant studies published up to the present. Inclusion criteria encompassed randomized controlled trials reporting outcomes of mechanical thrombectomy in acute ischemic stroke patients. A network meta-analysis was conducted to compare the relative effectiveness of mechanical thrombectomy with other treatment modalities. Results: Our network meta-analysis revealed that mechanical thrombectomy exhibited superior functional outcomes compared to standard medical therapy (p<0.001), intravenous thrombolysis (p<0.01), and medical therapy plus intravenous thrombolysis (p<0.05). Additionally, mechanical thrombectomy was associated with significantly lower mortality rates compared to standard medical therapy (p<0.01) and medical therapy plus intravenous thrombolysis (p<0.05). The analysis also demonstrated favorable rates of successful reperfusion with mechanical thrombectomy. Discussion: The findings indicate that mechanical thrombectomy surpasses standard medical therapy and other interventions in terms of functional outcomes, mortality reduction, and successful reperfusion. These results underscore the significance of mechanical thrombectomy as a leading treatment option for acute ischemic stroke. However, the study highlights the need for further investigation to optimize patient selection criteria and assess the long-term outcomes and cost-effectiveness of this intervention. Conclusion: Our findings advocate for the integration of mechanical thrombectomy into clinical guidelines as a preferred treatment for acute ischemic stroke. Future research should focus on refining patient selection criteria and evaluating the long-term benefits and cost-effectiveness of this groundbreaking intervention.

Abstract ID: 545

Clinico-immunological Profile and Outcome of Central Demyelination in a Pediatric Tertiary Care Hospital

Muhammed Shadique, Leema Pauline, Neeraj Elango, Jered Livingston, S. Mohinish

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drshadique@gmail.com

Background and Aim: Pediatric central demyelination is rare with an incidence of 0.9 per lakh population. Studies with a proper treatment protocol are lacking. This necessitates a study to understand the varied presentation and course of these disorders for better treatment protocols and follow-up. Methodology: To study the spectrum of various primary CNS demyelinating disorders; categorizing them as NMOSD, MOGAD,ADEM, optic neuritis, Transverse myelitis; treatment response; followup.Children who presented to our Institute with features of central demyelination between May 2018 and December 2022 were included in the study ; clinical details were recorded. Neuroimaging, CSF analysis, anti-Aquaporin4 & anti- MOG Antibody serology, VEP were done. Children were treated with IVIG/IVMP as indicated. Oral steroids/ 2nd line agents were given if necessary. Children were followed up with repeat imaging and managed appropriately in case of relapses. Data was assessed using SPSS 23.0 software. Results: Of the 41 patients included, 15(36.5%) were ADEM, 17(41.4%) were Optic neuritis and 9(21.9%) were Transverse myelitis. Male and female children were 19 and 22 (0.8:1). Among the clinical manifestations, visual manifestations (41.4%), weakness (29.2%), Ataxia (14.6%),Cranial nerve palsies (9.7%), Bladder dysfunction (7.3%), sensory abnormalities (2.4%), were common presentations. Discussion: One child (2.4%) presented as encephalitis.NMO-Ab was positive in 4(9.7%) and MOG antibody in 31 cases (75.6%); 6 children (14.6%) were double seronegative. 7 children relapsed, of which 6 were MOG positive and 1 was NMOSD. VEP and CSF abnormalities were noted in 30 and 3 children respectively. All children received IVMP followed by oral Prednisolone; one child with encephalitic presentaton was treated with IVIG.18 children needed Azathioprine to maintain remission. Conclusion: As not many studies on Pediatric central demyelination are available in our region, this study can help us understand the natural course and predict the outcome of pediatric central demyelinating disorders there by guiding optimal management.

Abstract ID: 546

Study of Blink Reflex in Bell’s Palsy and Its Correlation with Clinical Severity and Prognosis

Suresh Palanisamy, K. Thiruvarutchelvan, S. Sivakumar, C. Selvaraj

Pursuing MD Paediatrics, Other

E-mail: sureshhhcp@gmail.com

Background and Aim: Blink reflex studies are useful for assessing the pathway from trigeminal nerve through the brainstem upto facial nerve. Electrophysiological findings in bell’s palsy are known to have prolonged latencies or absent wave in blink reflex. Literature about the role of blink reflex in correlating clinical severity and its role as prognostic value in clinical improvement of bells palsy shows conflicting results. Aim: To study the usefulness of Blink reflex study in correlating clinical severity of Bell’s palsy and its prognostic value. Methodology: Study design:Prospective observational study in outpatient of Neurology department GMKMCH, Salem. Objectives: To study blink reflex response in Bell’s palsy within 7 days of onset and to evaluate clinically using House-Brackmann (HB) grading of facial function and correlate electrophysiological parameters with clinical findings. Duration:1 year. Results: Out of 26 patients with Bell’s palsy, based on clinical HB grading, grade 3-4 (moderate) included 14 patients and grade 5-6 (severe) included 12 patients. Among 14 patients with HB grade 3-4, blink reflex study showed latencies were prolonged in 8 patients (meanR1-14.25ms, R2i-44.38ms, R2c-45.50ms) and absent in 6 patients. Among patients with HB grade 5-6, latencies were prolonged in 9 patients (meanR1-14.44ms, R2i-45.44ms, R2c-46.44ms) and absent in 3 patients. No significant difference (using chi square test and Fisher exact test) in either R1 or R2 latencies of blink reflex observed between moderate or severe facial palsy (p value 0.45). Discussion: Studies on blink reflex to be used as prognostic test to predict outcome of facial nerve palsy recovery showed variable results in the past. Our study demonstrated that blink reflex has no value in predicting the clinical severity of Bell’s palsy and hence no value in prognosis of clinical recovery although it can be used to confirm facial nerve palsy. Limitations are small sample size. Conclusion: Blink reflex in Bell’ palsy cannot predict the clinical severity and has no prognostic value.

Abstract ID: 547

Visual Snow Syndrome – Excerpts from a Clinical and Radiological Perspective

Sohini Chakraborty, Anu Gupta, M. V. Padma Srivastava, Madhvi Tripathi, Senthil Kumaran

All India Institute of Medical Sciences, New Delhi, India

E-mail: sohinichakraborty84@gmail.com

Background and Aim: Visual Snow Syndrome (VSS) encompasses the clinical spectrum of visual static along with palinopsia, entoptic phenomena, photophobia and nyctalopia, having a validated diagnostic criterion by International Classification of Headache Disorders (ICHD). In a cohort of 1,104 patients described by Puledda et al, average age of onset was twenty-nine years and black and white static was the most commonly described feature, followed by floaters, after-images and photophobia. The pathophysiology underlies thalamo-cortical dysrhythmia and cortical disexcitability. Methodology: A 22 years old male presented with subacute onset progressive tinnitus in right followed by left ear, perceiving visual static (TV-static like images), palinopsia (afterimages and trailing of objects in the dark)D, enhanced entoptic phenomena, spontaneous photopsia, photophobia, haloes and nyctalopia for four months, preceded by an episode of upper respiratory tract infection. On neurological examination no deficit was elicitable. Secondary causes of visual snow were ruled out with negative infectious, autoimmune, paraneoplastic panel, normal MRI Brain, electroretinogram (ERG), optical coherence tomography (OCT), contrast sensitivity, visual field,visual evoked potentials, electroencephalogram, fundus examination and absent psychotropic drug intake history. FDG-PET and PET MRI fusion showed right lingual gyrus hypermetabolism. fMRI with DTI (Diffusion tensor imaging), showed BOLD activation of left> right fusiform, lingual gyrus and cuneus, suggestive of significant structural and metabolic abnormalities in the visual and limbic system. Results: Our patient was given a trial of injectable steroid for five days followed by oral steroid and topiramate. However, no meaningful improvement was noted in our patient. Discussion: Hyperexcitation of cortical visual pathways and decreased saliency threshold produce scotopic hypersensitivity, leading to visual snow and associated phenomena. Conclusion: Visual snow likely represents a clinical continuum, with different degrees of severity. In the future, further studies are needed to enhance our understanding of the underlying neurobiology of VS and consequently to move toward an era of better management of the condition.

Abstract ID: 548

Spectrum of OCT Retinal Nerve Fiber Layer Thickness at Onset and Three Months Follow up in Patients with Positive Aquaporin Antibody, Anti MOG Antibody and Double Seronegative Non MS Acute Optic Neuritis

Indranil Dutta, Debsadhan Biswas

Kolkata Medical College, Kolkata, West Bengal, India

E-mail: indracmc@gmail.com

Background and Aim: As there is sparse data of acute as well as follow up optic neuritis (ON) and ON due to non-MS (Multiple Sclerosis) far outnumber the MS patients in our country, our aim is to identify the non-MS acute ON patients based on their baseline OCT RNFL thickness, follow up at 3 months with RNFL values and corroborate the same with the visual outcome. Methodology: In this prospective study patients with ON attending Neurology Department over 1 year (September 2021 to august 2022) were evaluated and considered acute if presented within 2 weeks. They were categorized into 3 groups based on serology – AQP-4 +ve, MOG +ve and Double Seronegative (DN), but MS patients were excluded. Informed consent was taken for each subject, and approval of the Institutional Ethics committee was available. Results: We had 11 (19 eyes) MOG +ve, 3 (5 eyes) AQP-4 +ve and 10 (15 eyes) DN patients, of which 8 MOG (14 eyes), 2 AQP-4 (3 eyes) and 5 DN (7 eyes) patients had 1st acute attack of ON. Majority of acute MOG and all acute AQP-4 patients had thickening of RNFL. Only 2 acute DN patients (2 eyes) had high values of RNFL thickness. Discussion: At follow up all patients had significant thinning. Chronic (>2 weeks) or recurrent attack patients had baseline thinning of RNFL. Chronic patients had further thinning of RNFL at follow up while those with recurrent attacks had no further changes as the nerves were already thinned out. Of all the segments temporo-inferior segment was most involved followed by temporal segment. Conclusion: Baseline thickening in RNFL in acute ON suggests AQP4 or MOG antibody associated disease. Among the recurrent attack patients, visual prognosis is better for MOG patients compared to AQP4 +ve and DN patients, though all showed significant thinning of RNFL at follow up.

Abstract ID: 549

Clinical, Radiological Profile and Follow-up of Patients with Idiopathic Intracranial Hypertension

Mohamed Azharudeen, Ravi La, K. Shanmugasundaram, Marian Vijay, V. Chandramouleeswaran, Lakshmi Narasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: azharudeen1226@gmail.com

Background and Aim: Idiopathic intracranial hypertension (IIH) is a syndrome of elevated intracranial pressure (ICP) without any identifiable brain pathology. IIH is one of the preventable cause of blindness. The aim of our study is to describe the clinical and radiological profile of IIH cases and response to treatment at the end of 6 months. Methodology: This was a prospective study carried out on 49 patients presented to our institute with the symptomatology of IIH. All the patients were subjected to complete history taking, neurological examination, lumbar puncture, and MRI brain and MR cerebral venography. Patients with secondary causes of raised ICP were excluded. The patients were followed-up for 6 months to assess treatment response. Results: Patients’ ages ranged from 18 to 60 years, the majority were females, and 48% of cases had grade 1 obesity. Headache was the presenting manifestation in 100% of the patients. 2 had IIH without papilledema. About 50% of the patients were either misdiagnosed or had a delayed diagnosis. There was a significant positive correlation between CSF opening pressure, the severity of a headache, and the grade of papilledema. Empty sella turcica is the most common MRI abnormality occurring in 47%. The majority of cases responded to medical management, and three cases of fulminant IIH underwent lumbar-peritoneal shunt. At the end of 6 months, 60% had persistent headache. Only 8.7% had persistent visual symptoms, and 2 had optic atrophy. High grades of papilledema correlated with CSF opening pressure. Discussion: In our study, we found there was a significant positive correlation between CSF opening pressure, the severity of a headache, and the grade of papilledema. The highest grades of papilledema correlated with CSF opening pressure. These findings were consistent with the Asian studies published earlier. Conclusion: Headache is the most common presentation of IIH. Early diagnosis and appropriate treatment can prevent blindness.

Abstract ID: 550

Impact of Nonpharmacological Intervention on Quality of Life in Patients with Idiopathic Parkinson’s Disease

Kiran Bala, Areca Wangnoo, Surekha Dabla

DM Neuro Completed, Other

E-mail: balakiran56@gmail.com

Background and Aim: Quality of life in (QoL) patients with Idiopathic Parkinson’s disease (IPD) remains compromised due to its motor and non-motor manifestations. Aim:of our study was to assess the impact of non-pharmacological interventions on QoL in patients with IPD. Methodology: A prospective study on 54 IPD patients attending Movement disorder Clinic in a tertiary hospital enrolled after written informed consent. Intervention group comprised of 19 patients in stage 1-3 Modified H&Y, without any major orthopaedic, cardiovascular or cognitive impairment. Motor and non-motor functions, severity and QoL evaluated using MDS-UPDRS, Modified H&Y Staging and PDQ 39 respectively. Intervention given by health education, dietary modification, raj yoga meditation and exercise. Follow up assessment done at 3 months and 6 months. Results: Mean age and disease duration were 60.2 and 5.69 years respectively, M:F ratio 1.45:1. Most common motor and non-motor symptom was tremor (55.6%) and urinary symptom (16.7%) respectively. Mean MDS-UPDRS total score was 80.07±37.47. Mean PDQ39SI was 29.78±18.06. QoL was worst affected in activities of daily living (46.62) and emotional well-being (37.79). MDS-UPDRS positively correlated with PDQ39 (r=0.814,p<0.001). Discussion: Activities of daily living and emotional well-being were the worst affected dimensions of QoL as also reported by Martinez-Martin et al. Significant positive correlation of quality of life PDQ 39 with levodopa dose and dyskinesias was found similar to that reported by Chapuis et al. Improvement in QoL after nonpharmacological intervention (Excercise ,mindfulness meditation) has also been reported by others (Van der Kolk et al,Advocat J et al and Srikumar V et al). Conclusion: Quality of life was worst affected in the domains of activities of daily living, emotional well-being and mobility. ¬ The mean overall PDQ39 scores in the intervention group showed statistically significant reduction after 3 months and 6months. (p = 0.007 at 3 months and p=0.013 at 6months).

Abstract ID: 551

A Feasibility Study of Game-Based Rehabilitation System for Upper-Limb in Acute Neurological Care Setting

Sudhindra Vooturi, Panem Sumanth, Habib Ali, K. G. Sreehari

KIMS Hospital, Hyderabad, Telangana, India

E-mail: sudhindragupta@gmail.com

Background and Aim: Background: Game-based rehabilitation systems (GBRS) are being increasingly utilized in Out-Patient Rehabilitation units across the world. It is well established that Early Initiation of Rehabilitation improves the chances of functional recovery. However, feasibility of GBRS is rarely investigated in In-Hospital / Acute Care Settings. Aim: To evaluate the feasibility of GBRS for Upper Limb recovery in Acute Neurological Care Settings. Methodology: Methods: Ninety-Nine patients with acute Neurological ailments and resultant upper-limb motor dysfunction assessed by Action Research Arm Test (ARAT) score of <10 were included. Fugl-Myer assessment for Upper-Limb function (score of 0-2 per task) was done in the hospital once the patient was haemodynamically stable and repeated at discharge from the hospital. The game-based exercises aimed to engage the participant in shoulder flexion, shoulder horizontal abduction/adduction, elbow flexion/extension, and grasp. The sessions were done two times a day as part of the daily physiotherapy session. Results: The average age of the study population was 60.14±15.4 years with 72 (70.3%) men. The average length of stay was 11±17 days. There was significant improvement in shoulder flexion (0.46 vs. 0.64; p<0.001), shoulder elevation (0.79 vs 0.97; p<0.001), elbow extension (0.84 vs. 1.00; p<0.001), grasp abduction of thumb (0.47 vs 0.61; p<0.001), elbow supination and pronation (0.67 vs 0.85; p<0.001) during the stay in the hospital. Discussion: The results reported in this study suggest the feasibility of employing the computerized game-based rehabilitation to deliver correct arm and hand movements during early phases of rehabilitation. Our findings should be validated with randomized-control studies. Conclusion: Our results suggest that Game-Based Rehabilitation are a feasible to be initiated immediately after initial insult in Neurological Conditions after ensuring haemodynamic stability.

Abstract ID: 552

Cognitive Impairment in Adult Onset Primary Focal Dystonia

Indu Kiran, Atanu Biswas

Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

E-mail: indu.mbbs07@gmail.com

Background and Aim: Dystonia is a motor disorder that results from dysfunction of basal ganglia dysfunction. Studies have shown many non motor symptoms are part of clinical symptoms. We conducted a prospective observational study to determine the domains and magnitude of cognitive impairment in patients of adult onset primary focal dystonia. Methodology: A set of neuropsychological tests were applied to patients of primary focal and segmental dystonia with age of onset >/= 18 years. The tests included- ACE-Bengali/Hindi, Digit span test, Trail making test A & B, Tulia, Verbal learning test, Verbal fluency test and Picture naming test. Results: The study was done on 31 cases of primary dystonia (fulfilling inclusion and exclusion criteria), and it was found that as compared to healthy controls of similar age, sex, educational qualification and socio-economic status, patients with primary dystonia showed deficit in working memory functions revealed by digit span test, new learning as revealed by verbal learning test, deficit on information processing speed and set-shifting capacity revealed by Trail Making Test A and B. Discussion: Dystonia has been considered as a pure motor disorder arising due to basal ganglia dysfunction, but recent studies have shown plethora of non-motor symptoms often present in patients with dystonia like cognitive impairment in domains of visuospatial functioning, verbal memory and set shifting; sleep problems; pain and psychiatric symptoms like anxiety, depression, phobia, OCD and alcohol dependence. These non-motor symptoms further add to the disease burden and quality of life of the patients. The occurrence of cognitive dysfunction can be explained by dense connection of basal ganglia to prefrontal cortex which is involved in higher cognitive function and social behaviour. Conclusion: We found that patients of primary dystonia have impairment in specific cognitive domains relative to attention, working memory, processing speed and short term memory.

Abstract ID: 553

Clinical profile of Drug Induced Parkinsonism

Praveen K. Yadav

DM Neuro Completed, Other

E-mail: dr.praveen4u@gmail.com

Background and Aim: DIP is a reversible condition in most of the cases therefore have to be identified and treat early. In our study we presented the dominant pattern of DIP and the medications causing it. Methodology: This is a retrospective study done on outpatient basis from1st January 2022 to 30th November 2022 at a super speciality neuro clinic. In this study all patient, satisfying the criteria of DIP were included. The inclusion criteria were: 1. All patients satisfying more than equal to two of the four criterias of Parkinsonism- bradykinesia, rigidity, tremor and postural instability. 2. Patients had no symptoms of Parkinsonism before taking drug. 3. There is direct relation of drug intake and symptom onset, and the Exclusion Criteria were: 1. Other causes of secondary parkinsonism- NPH, vascular parkinsonism, brain tumour, toxin induced etc by appropriate investigations. 2. All other drug induced motility disorder without the features of parkinsonism. The age, sex and other demographic criterias were studied. The dominant pattern of involvement- tremor, akinetic rigidity and mixed were noted. Results: Out of 32 patients 19 (59.38%) were female and 13 (40.63%) were male. The mean age was 62.37 years with standard deviation of 13.09 years. The most common clinical pattern observed in our study was tremor (50%) followed by akinetic rigidity (34.38%) and mixed pattern (15.63%). Orofacial dyskinesia along with bradykinesia, rabbit syndrome, head and jaw tremor along with parkinsonian features were all seen in our study with equal percentage of (6.66%). Atypical Parkinson with features of Parkinson plus syndrome is also seen in 6.66%. The most common drug associated with DIP in our study was pro-kinetic drug levosulpiride (46.88%) followed by flunarizine (25%), valproate and haloperidol (6.25%), sulpitac and itopride (3.13%). The combination drugs causing DIP was also seen in 9.36% Discussion: same. Conclusion: DIP is a reversible and cause of parkinsonism if identified early.

Abstract ID: 554

A Case of Wilsons Disease with Atypical Radiological Features

Sathya Tejaswini Durgam, S. Kaushik, Marian Jude Vijay, K. Shunmuga Sundaram, R. Lakshmi Narasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: teju.durgam@gmail.com

Background and Aim: Wilsons disease (WD), a rare autosomal recessively inherited disease of copper metabolism, is one of the few treatable metabolic disorders if diagnosed early. Neurological manifestations of WD are varied, few of which include Akinetic rigid syndrome with Parkinsonian features, generalised dystonia, ataxia with intentional tremor. White matter hyperintensities are rarely reported in WD. With this background, we report case of WD with atypical imaging features. Methodology: A 15-year-old boy born of 3rd degree consanguineous marriage, with normal birth and developmental history presented with slurring of speech, scissoring of gait, dystonic posturing of all 4 limbs progressed over last 4 years. He progressed to such an extent that he could barely speak, walk with support. On examination, he has KF ring, vacuous smile, slow vertical saccades, spasticity of all limbs, extensor plantar, dystonic of tongue, feet. Results: He was thoroughly evaluated. KF ring was confirmed by slit lamp examination. MRI brain showed symmetrical T2, Flair white matter hyperintensities in frontal subcortical region, parieto temporal region with cystic change, hyperintensities in bilateral basal ganglia, midbrain with no diffusion restriction and with thinning of corpus callosum. Serum Ceruloplasmin was low (<4). USG abdomen showed coarse echotexture of liver. He was started on Tab Penicillamine, Tab Zinc. He is under follow up. Discussion: MRI brain usually shows hyperintensities in lentiform nucleus, globus pallidus, mid brain with sparing of red nucleus and substantia nigra. Giant Panda sign in mid brain, miniature panda sign in pons have been described. Symmetrical involvement with cystic change is very rare and the underlying etiology is not clearly known. Conclusion: Though its rare to have extensive white matter involvement in wilsons disease, it is essential to rule out wilsons disease in such cases, as it is a potentially treatable disease. Through our case, we highlight the atypical MRI features of wilsons disease.

Abstract ID: 555

Acute Disseminated Encephalomyelitis with Pulmonary Tuberculosis: Coincidence or Cause?

Sohini Chakraborty, Saman Fatima, Mamta Singh, Rohit Bhatia

All India Institute of Medical Sciences, New Delhi, India

E-mail: sohinichakraborty84@gmail.com

Background and Aim: Acute disseminated encephalomyelitis (ADEM), as defined by the International Paediatric Multiple Sclerosis Study Group (IPMSSG), is an acute demyelination syndrome, presenting clinically with encephalopathy and polyfocal CNS symptoms showing demyelination on brain MRI, occurring mostly in children. It is predominantly a post-infectious disorder, having an identifiable trigger in 50-85%. However, evidence for tuberculosis as a trigger is scarce in literature. We are describing an adult male presenting with ADEM with microbiologically confirmed pulmonary tuberculosis. Methodology: Mr. F, nineteen years old male, having low-grade fever and dry cough for two weeks, presented with subacute onset progressive left hemiplegia involving the right upper and lower limb within two days of onset. On examination he had generalised hyper-reflexia, extensor plantar, left hemiplegia and bronchial breath sounds in right upper zone. X-ray Chest showed right apical consolidation. HRCT Thorax showed cavitary lesions, tree in bud appearance, nodules with mediastinal lymphadenopathy in bilateral upper and middle lobe (Right>Left) suggestive of active Koch’s. MRI Brain and Spine showed bilateral large, asymmetrical (Right>Left), poorly demarcated, non-enhancing, T2 FLAIR hyperintense lesions in parietal lobes. As sputum analysis came negative, Bronchoscopy guided lavage done, GeneXpert came positive for acid fast bacilli with Rifampicin sensitivity. Work-up for primary and secondary demyelination, including NMO, MOG, CSF analysis and infective panel, came negative. Results: He was started on pulse steroid and four drug anti-tubercular therapy (Isoniazid, Rifampicin, Pyrazinamide and Ethambutol) with Pyridoxine, following which there was significant clinical and radiological improvement. MRI Brain repeated at an interval of one month showed reduction in size of lesions and cavitations. Discussion: Tuberculosis may be considered an antecedent trigger for ADEM in countries where tuberculosis is frequent. The neurological symptoms may be confused with tuberculous meningitis, which was excluded in our case. Conclusion: However, whether it was the cause or just a mere coincidence in our scenario, is debatable.

Abstract ID: 556

A Study of Clinical Profile of CVT Admitted in a Tertiary Care Hospital

P. Prasanth, M. R. Manivannan, C. Justin, M. R. Manivannan

DM Neuro Pursuing, Other

E-mail: pkumartheboss@gmail.com

Background and Aim: X linked adrenoleukodystrophy (X-ALD) is an inherited disorder of peroxisomal metabolism, biochemically characterised by accumulation of saturated very long chain fatty acids. Accumulation of these fatty acids is associated with cerebral demyelination, peripheral nerve abnormalities, and adrenocortical and testicular insufficiency.The various phenotypes have been recognized to occur within the same pedigree. The cause of the phenotypic variability is unknown. The first case of cerebral ALD was published by Haberfeld and Spieler in 1910. Methodology: A girl presented to us at eleven years of age with a history of nonconsanguious marriage,2nd order birth with normalbirth history and normal milestones till 5 years of age and positive family history presented with spastic weakness of both lower limbs in the term of difficulty in standing, climbing up & down staircase, associated with tightness in both legs (distal>proximal), buckling of knee present.Neurological examination showed bilateral extensor , ankle clonus-present, increased tone, accelerated lumbar lordosis-present. Younger brother and paternal aunt are affected with the same condition. All routine laboratory investigations produced normal results. MRI brain report showed symmetric T2/Flair hyperintense noted in bilateral periventricular white matter and posterior limb of internal capsule possibility of metachromatic leukodystrophy.Genetic analysis revealed homozygous variant of uncertain significance. Results: Accurate information about the relative frequency of the various forms of ALD is needed for counselling and for the selection and evaluation of therapeutic approaches. Discussion: Risk-benefit analysis of bone marrow transplantation requires knowledge of the phenotype frequencies. Conclusion: Promising results from an ongoing gene-therapy trial and recent antioxidant therapy investigations and the increase in newborn screening programs provide hope for patients with ALD.

Abstract ID: 557

TB or not to be - That is the question

Navendu Jana

DNB Neuro Pursuing, Other

E-mail: navendujana@gmail.com

Background and Aim: Four atypical cases of tuberculosis have been highlighted. Methodology: Case#1 Toughest thing in life to achieve - balance 51-year-old female presented with severe gait instability with no fever or meningism. MRI brain: Leptomeningeal enhancement. CSF: 65 cell/cumm mostly lymphocytes, elevated protein (211.4 mg/dL) and low normal Glucose 54 mg/dL and CBG134) CT scan Thorax: enlarged paratracheal lymph nodes. EBUS TBNA: ill formed granulomas. CASE#2 PUKE, PUKE AND MORE PUKE A 27-year-old male presented with intractable vomiting for one month and normal gastroenterology workup. MRI Brain: multiple lesions in brainstem, cerebellum and medulla which were thought to be neoplastic and whole body PET CT scan was unfruitful. CSF cytology and biochemistry: normal. CBNAAT for TB in CSF: Positive. Case#3 This case merits a ‘pres’ release A 51-year-old female Presentation: headache and blurring of vision, on antitubercular medications for spastic paraparesis due to spinal arachnoiditis. Initial MRI brain was normal only for it to reveal lesions akin to PRES when she suffered seizures. Associated with spuriously high blood pressure at that time with probable secondary causes ruled out. Case#4 Don’t mention the unsolved ones, Watson!! A 51-year-old male presented with spastic paraparesis due to spinal arachnoiditis and spinal subarachnoid hemorrhage. All possible inflammatory, infective, neoplastic, vascular causes ruled out. Minimal pleural effusion was exudative in nature and biopsy of pleural surface nodules revealed ill formed granulomas. CSF remained hemorrhagic on subsequent taps. Results: First three patients responded well to anti tubercular drugs but the fourth one remains a mystery with no improvement in CSF picture. Discussion: Manifestations of Tuberculosis can be dubious and challenging, needing sincere workup. Conclusion: Idiopathic in west can be Tuberculosis in the Indian Subcontinent. With great power in advancement in diagnostics and specific drugs, comes great responsibility to use them in a judicious and evidence based scenario.

Abstract ID: 559

Effect of Antiseizure Medication on Migraine in Patient’s with New Onset Epilepsy

Saurabh Sultania

Dayanand Medical College, Ludhiana, Punjab, India

E-mail: saurabh211311@gmail.com

Background and Aim: Background: Epilepsy is characterized by unprovoked seizures that require long term or lifelong therapy. It is seen that epilepsy is associated with migraine but is often under recognized. Anti seizure medications (ASM’s) are known to have additional effects in comorbidities mainly migraine. FDA has approved few AEDs as a prophylactic treatment in migraine. Aim and Objectives: .To study the frequency of migraine in patients with new onset epilepsy and to study the effect of various AEDs on frequency, severity, pattern and disability in patients of migraine. Methodology: Patients suffering from epilepsy and migraine who were on AEDs monotherapy were recruited from the neurology and epilepsy OPD after prior approval from institutional ethic committee. Follow up was done for 3 months after dose stabilization. Patients were asked to maintain a migraine diary to record the frequency of migraine attacks per month. Severity of migraine attack was assessed by using VAS and GAMS scale. Results: In this study of 102 patients, predominant age group was 21-40 years. Majority of patients were of GTCS . Significant decrease in frequency and severity was seen with Valproate and levetiracetam. Discussion: Based on the results, it was found that AEDs like LEV and VPA showed significant decrease in migraine frequency and severity. The effect of AEDs was augmented with propranolol in reducing frequency and severity of migraine (with or without aura). Conclusion: Migraine is an important co morbidity in epilepsy, its recognition is important as epilepsy patients who have comorbid migraine can be treated with antiseizure medications having antimigrainous effects, hereby reducing there morbidity.

Abstract ID: 560

Burden of Comorbid Conditions in People with Epilepsy

Harish Chandika, S. Saravanan

DM Neuro Pursuing, Other

E-mail: harishchandika21@gmail.com

Background and Aim: World health organisation estimates that there are 50 million people living with epilepsy worldwide. People with epilepsy have challenges in daily activities. Comorbidities associated with epilepsy like headache, insomnia, pain syndromes, hypertension and diabetes mellitus play an important role in the management and health outcomes for people with epilepsy. Methodology: All patients older than 18 years with epilepsy attending to neurology department, TVMC hospital were enrolled for the study. Comorbid conditions like headache, insomnia, pain syndromes, hypertension and diabetes mellitus in people with epilepsy, their demographic data and clinical profile were noted through structured proforma. Results: The collected data was entered into Microsoft Excel and analysed using the statistical software SPSS version 23.0. Comorbid conditions negatively affect seizure outcome and quality of life. Discussion: The burden of comorbidity in people with epilepsy is high. Understanding the basis for the association of epilepsy and comorbid conditions poses important challenges for epilepsy diagnosis and management. Several mechanisms explain how epilepsy and comorbidities are associated, including shared risk factors and bidirectional relations. Conclusion: Comorbid conditions affects quality of life in people with epilepsy. Better quality of life to the patients can be given by addressing the comorbid conditions along with the primary disease of epilepsy.

Abstract ID: 561

A Battle with Norse- A Challenging Management

Sujaya Raghavendra, M. Jawahar, R. Lakshminarasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: sujayar30@gmail.com

Background and Aim: New onset refractory status epilepticus as we all know is a clinical presentation of refractory status epilepticus. Among adult patients without cryptogenic NORSE, the most often identified cause is autoimmune encephalitis, either nonparaneoplastic or paraneoplastic. Infections are the most prevalent etiology of pediatric NORSE it has high mortality and morbidity if not managed well , and it becomes challenging as the status progresses. Methodology: This 19 year old female with no previous comorbidities came with acute onset progressive neurological illness presenting initially with unilateral right sided headache with high grade fever for 3 days followed by GTCS later after admission in evolving into right sided focal seizures with secondary generalisation. . She stayed for 1 month and 7 days in outside hospital and referred with refractory seizures to RGGGH for further managment . Results: on examination patient was under sedation and intubated . higher mental functions could not be assessed . vitals showed tachycardia and was normotensive general examination done showed .Perioral movements with involuntary movements of the tongue present along with uprolling of eyes along with right sided irregular low amplitude writhing movements involving the distal hand.Doll s eye was sluggish and pupils were sluggishly reacting to light. Discussion: her routine investigations and CSF analysis was normal . CSF virology ,autoimmune encephalitis panel ,vasculitic profile and paraneoplastic workup was negative.MRI brain done showed hyper intensities in the medial cingulate gyrus and right medial temporal lobe. she was treated according to SE protocol. we started her on IVIG after which her seizures subsided. she remained seizure free for 1 month but again she developed seizures for which PLEX was done. currently she is recovering after a battle of 5 months. Conclusion: cryptogenic NORSE is life threatening condition and the co existence of seronegative Autoimmune encephalitis with no underlying malignancy needs immediate treatment with immunosuppressants.

Abstract ID: 562

A Study of Peripheral Nervous System Manifestations of Systemic Cancer and Cancer Therapy

G. Sreerangalakshmi, Himaja Sanganabhatla

Osmania Medical College, Hyderabad, Telangana, India

E-mail: rangalakshmi2000@gmail.com

Background and Aim: Involvement of PNS is common in patients with cancer and any part including motor neurons,ganglia,roots,plexus,peripheral nerves can be affected. Different mechanisms can initiate damage which include infiltration,infections,treatment related toxicities, paraneoplastic neurological syndromes. Type of cancer is a further determinant of PNS disorder.Methodology: 50 patients who presented to outpatient department of neurology between October 2022-March 2023 with known systemic cancer were evaluated by clinical history, detailed neurological examination and investigated using routine blood work, NCS, and other necessary investigations. Neuroimaging was done where necessary. Results: In our study, among 50 patients, the most common cancer were hematological malignancies where peripheral neuropathy was the most common PNS manifestation. In solid tumors, infiltration was the most common PNS manifestation. Treatment related toxicities were seen in both solid and hematological malignancies in which pain was the presenting feature. Discussion: Pain and paresthesia were the most common presenting symptoms and effected quality of life significantly in patients with malignancies. Carcinoma breast and lung were the most common solid organ tumors. Most hematological cancer patients had chemotherapy related adverse effects while most patients with solid organ cancers had manifestations related to local invasion or metastasis. Conclusion: Neurological complications in patients with malignancy can significantly impact quality of life. The spectrum of complications is broad and includes a variety of direct and indirect complications. Early detection of these complications can help in discontinuation or modification of treatment regimen and permanent neurological injury can be prevented if early treatment is initiated.

Abstract ID: 563

Longitudinal Study on Neurological Disorders among Mothers in Early Peuperium in a Tertiary Care Hospital

K. Jai Ganesh, J. Manickavasagam, G. Balaji, S. Hariharan

DM Neuro Pursuing, Other

E-mail: k.jaiganeshmbbs@gmail.com

Background and Aim: Neurological disorders are a significant cause of morbidity and mortality during the early peuperium.Hormonal and physiological changes in early puerperium is known to exacerbate already existing neurological disorder or precipitate new ones. To study the clinical profile of mothers presenting with Neurological disorders during the first 2week of peuperium. Methodology: Prospective observational study Duration: 1 year Study population: Mothers in the first 2 week of puerperium who presented with neurological symptoms and were diagnosed with a Neurological disorder (New or Preexisting) at Chengalpattu medical college hospital OPD/ IP wards. Results: A total of 83 mothers of ages between 17 to 30 yrs were diagnosed to have a Neurological disorder in the first 2 week of puerperium. Cortical venous Thrombosis was the commonest disorder (30 of 83) of which 10 presented only with headaches while others with headache and seizures.PRES was the diagnosis in 17 patients among whom Seizures followed by a prolonged LOC was the presentation in the majority. Discussion: The other diagnosis being Postpartum Eclampsia in 10 , exacerbation of Migraine in 9, Post-spinal Headache in 6, Epileptic Seizures in 4 ,Postpartum psychosis and LS plexopathy in 2 each and foot drop and arterial stroke in 1each. Conclusion: Headaches and seizures are the major neurological symptoms in early puerperium and evaluation in our cohort of mothers in the first 2 week of puerperium yielded the above-mentioned spectrum of diagnosis.

Abstract ID: 564

Prognosis and Treatment Outcome Study of Guillain Barre Syndrome from a Rural Tertiary Care Hospital

P. Rashma Mohammed, Fazal Ghafoor, Aswathy Sasidharan, S. Firosh Khan

DM Neuro Pursuing, Other

E-mail: rashmamohammed@gmail.com

Background and Aim: Guillain–Barre syndrome (GBS) is an autoimmune disease with polyneuropathy involving motor, sensory and sometimes autonomic nerves. The present study was aimed to identify the clinical findings, outcomes and variations amongst the patients with GBS diagnosed in our hospital.Course in hospital and response to treatment was followed up with MRC scale and modified Rankin scale. Methodology: A retrospective analysis of cases diagnosed as GBS were conducted.Diagnosis was based on Alber’s criteria.Time period was one year from April 2022 -april 2023. Medical records and the data related to age, sex, antecedent illness, duration of symptoms before admission, muscle power graded by the Medical Research Council scale, functional scores, details of Intensive Care Unit complications and need for ventilation were obtained. Results: The mean age of the patients was 46 yr. (60 %) patients had a history of preceding illness. Fourteen (70%) patients showed quadriparesis as most common complaint. More than 50 % had a preceding illness.One had a preceding history of COVID infection Ten (50%) patients were of acute inflammatory demyelinating polyneuropathy (AIDP) type in NCS. 2 patients required ventilatory support.Follow up was monitored with modified Rankin scale.80% showed improvement in modified rankins scale. Discussion: It is a rare but recognized cause of generalized progressive paralysis worldwide. Incidence is 1-2/100,000 population. It is a post-infectious immune-mediated neuropathy.It can present with sensory symptoms such as numbness and ascending paralysis. Treatment modalities available include plasmapheresis and IVIG. In our study AIDP was the commonest type.Prognosis was poor for AMAN. Conclusion: GBS seems to affect all age groups.Most common presenting feature was paraparesis .AIDP was the common type followed by AMAN.Prospective studies with follow up of GBS patients including repeat CSF and NCS is essential.This study aims to identify preceding factors profile and outcome of GBS patients.

Abstract ID: 565

Unilateral Asterixis – A Localizing Sign in Thalamic Infarct

Justin, P. Dhileeban

DM Neuro Completed, Other

E-mail: drjustin2001@yahoo.com

Background and Aim: Asterixis is an important clinical sign commonly seen in patients with hepatic encephalopathy and known as hepatic flaps. Bilateral asterixis is seen in encephalopathies such as uremic encephalopathy, carbon dioxide narcosis, drug-induced sedation, etc. Unlike bilateral asterixis, unilateral asterixis is associated with structural lesions in the brain. We hereby revisit this important neurological sign and present a patient with isolated unilateral asterixis. Methodology: A 65-year-old right-handed woman, with history of diabetes mellitus for 10 years on oral hypoglycaemic agents, was admitted in our emergency room. Clinical examination revealed minimal weakness with unilateral asterixis of the right upper limb, as well as reduced muscle tone on the right side and a positive Babinski sign. The cranial nerve examination, sensory examination, and deep tendon reflexes were normal. Results: Laboratory data were also completely unremarkable. Cranial CT imaging at admission did not show any abnormality. An MRI scan showed acute infarction involving the left thalamus. There were no abnormal lesions in the internal capsule, the basal ganglia, or the cerebellum. Discussion: Asterixis is an arrhythmic, abrupt negative myoclonus due to irregular intermittent interruption of tonic muscle contractions evoked usually in the upper limbs when the arms are outstretched. It is predominantly associated with metabolic diseases. Hyperkinetic movement disorders, such as tremor, chorea, dystonia, and asterixis, may occur in 1–4% of ischemic stroke cases. The pathophysiology of unilateral asterixis occurring in thalamic stroke remains unclear. Due to the different lesions of symptomatic unilateral asterixis, one can propose that there are cortico-subcortical pathways involving the basal ganglia, motor thalamus, cerebellum, and frontal-parietal neocortex to control postural tone and movement. Conclusion: We conclude that asterixis is a common, useful clinical sign, and unilateral asterixis has a clearly lateralizing value in neurological examination.

Abstract ID: 566

Validation of Cerebral Collaterals on Vascular Imaging in Acute Anterior Ischemic Stroke in Relation to Functional Outcome

Sreehari Dinesh, Shankar Gorthi, Dulari Gupta

Bharati Vidyapeeth Medical College, Pune, Maharashtra, India

E-mail: sreehari4657@yahoo.co.in

Background and Aim: Background: Brain collateral status in patients with acute ischemic stroke due to anterior circulation occlusions has become an important predictor of functional outcomes. Aim: To assess the cerebral collaterals using CT angiography with various scoring systems and to correlate with functional outcome in cases of acute anterior ischemic stroke. Methodology: This is a prospective observational analytical single-centre study done to describe the cerebral collaterals and various scores associated with it among patients from a tertiary care referral centre in Western India from July 2023 to April 2023. Results: Our study had 48 patients of acute anterior ischemic stroke. Mean age of patients were 55.36 ± 16.4 with 72.9% being males. The average NIHSS of patients was 10.18 ± 7.6. Hypertension was the most common risk factor seen in 59.6% cases followed by diabetes mellitus in 34% cases and tobacco consumption in 25.5% cases. Atrial fibrillation and ischemic heart disease was seen in 6.4% patients each. 17.8% cases underwent intravascular thrombolysis, 7% endovascular thrombectomy and 9.3% required decompressive hemicraniectomy.95.6% cases received aspirin and 8.9% cases received heparin. mCTA and CT ASPECTS had the best sensitivity in predicting the outcome with area under the curve of 0.6. Good outcomes (modified Rankin Score at 3 months <=2) were seen in 60% of cases. Discussion: Collateral status in patients with acute ischemic stroke due to anterior circulation occlusions has become an important predictor of functional outcome of patients. Most of the cerebral collateral scoring systems have not been validated and our research correlates favorable brain collaterals as visualized and scored using these scoring systems on CT Angiography and the improved functional outcome of patients in acute anterior ischemic stroke. Conclusion: Good cerebral collateral has been associated with good clinical outcome.

Abstract ID: 567

A Rare and Interesting Case of Stroke – “Thirteen and a Half Syndrome with Hemiparesis”

Rajeev Katti, Sree Lakshmi, N. Veena, Archana Bethala, Akhilesh Kumar

Osmania Medical College, Hyderabad, Telangana, India

E-mail: rajeevkatti9@gmail.com

Background and Aim: Thirteen and a half syndrome (13 ½ )is a newly described syndrome. It is an extension of one and a half syndrome (1 ½ syndrome).Presence of LMN facial nerve palsy with 1 1/2 syndrome is called Eight and a half syndrome (8 1/2 syndrome). 13 ½ Syndrome is an extension of 8 ½ syndrome with involvement of V cranial nerve nucleus (8 ½ + 5 = 13 ½ ).Only 2 cases of thirteen and a half syndrome are described in the literature till now and both were secondary to space occupying lesions. Here we report a case presenting with 13 ½ syndrome with hemiparesis and secondary to Stroke.To the best of our knowledge this is the first case of 13 ½ syndrome associated with hemiparesis and presenting secondary to stroke. Methodology: 40 year old gentleman presented to our hospital with left hemiparesis of one day duration. The patient also complained of diplopia, deviation of the mouth to left and inability to close right eye, Results: On examination the patient was found to have right conjugate gaze palsy with right INO and abduction nystagmus of the left eye. The patient also was found to have reduced sensations on the right side of the face with absent corneal reflex on right. The patient also had right LMN facial nerve palsy with left hemiparesis MRI showed an infarct extending from the dorsal part of the pons to the ventral part on the right side. Discussion: There are many variants of one and a half syndrome i.e.,8 ½ syndrome, Nine syndrome, 15 ½ syndrome, 13 ½ syndrome. Only 2 cases of 13 ½ syndrome are reported so far and none of the patients had hemiparesis and none were secondary to stroke. Conclusion: This case report widens the spectrum of 8 ½ syndrome to include 13 ½ syndrome as a variant.

Abstract ID: 568

Study of Language Disorders and Clinicoradiological Correlation of Stroke in a Tertiary Care Hospital

Maheshpandian Mariappan, Ramu Sekar, K. Murugan

DM Neuro Pursuing, Other

E-mail: dr.mahesh.neurologist@gmail.com

Background and Aim: To study the clinical and anatomical correlation in different types of aphasia presenting in stroke. Methodology: Patients attending OPD or admitted with a clinical diagnosis of stroke with aphasia were included in the study. Inclusion criteria 1) Acute vascular syndrome with aphasia, 2) Presentation within 3 months of stroke, Exclusion criteria 1) Prior history of stroke, 2) History of cognitive decline or progressive language disturbance preceding the event. Clinically aphasia was evaluated at the bedside and WAB-R. Results: A total of 80 patients were selected for the study. Patients presenting as Global Aphasia had large lesions with involvement of insula, cortical, subcortical, temporoparietal operculum, cortical frontal operculum predominantly, also having involvement of Putamen, AG, STG. In Wernicke’s aphasia, STG was involved predominantly. Discussion: Sensitivity of imaging modality, MR is superior to CT in documenting anatomical substrate of aphasia. Most important observation regarding global aphasia were that patients had extensive lesions in the perisylvian cortex and subcortical white matter. In addition, involvement of basal ganglia structures and internal capsule was also seen in a significant proportion of patients. This is in concurrence with the previous MR and CT based studies. Also, Wernicke’s aphasia had fairly consistent pattern of involvement of cortical and subcortical temporoparietal operculum and STG. Similar pattern of involvement has been observed in neuroimaging studies. Conclusion: 1.Most common aphasia in acute stroke is Global Aphasia and they tend to have a large lesions involving cortical and subcortical areas on the left hemisphere. 2.Wernicke’s Aphasia had involvement of Temporoparietal operculum and STG mostly. 3.Putamen and anterior limb of internal capsule were commonly involved in pure subcortical aphasia.

Abstract ID: 569

Movement Disorder Emergencies: A Case Series of 11 Cases

Dinesh Kumar Manna, Ankur Banik, Dilip Roy, Debsadhan Biswas, Sandip Pal, Ande Mihira Chakravarty

Kolkata Medical College, Kolkata, West Bengal, India

E-mail: drdkmanna@gmail.com

Background and Aim: Although traditionally movement disorders are attributed to chronic conditions,acute presentations are often encountered, posing diagnostic and therapeutic challenges.Studies on acute movement disorders are relatively sparse and especially so in our socio-demographic background.In this study we describe the clinical profile of 11 cases of movement disorder presenting in our emergency. Methodology: Patients attending ER of Medical College, Kolkata with movement disorder as the sole or predominant presentation within 4 weeks of disease onset have been considered as acute movement disorder emergency and enrolled in our study after proper consent.Detailed history,phenomenology,video recording,general and neurological examination and relevant investigations were obtained. Results: Out of 11 patients,7 had hyperkinetic disorders,while 2 each had hypo kinetic and mixed movement disorders.Among the hyperkinetic group,there were 2 patients each of hemichorea and generalised choreodystonia and 1 each of status dystonicus,tremor and cortical myoclonus.Of the 2 patients with hypokinetic prsentation,both had Catatonia and the other 2 cases had acute Parkinsonism with craniocervical dyskinesia and oculogyric crisis.Etiologically we diagnosed 2 patients each with hyperglycaemia, stroke, drug induced conditions and neuropsychiatric disorders.We also diagnosed a patient each of status dystonicus presentation of PKAN,functional movement disorder and autoimmune encephalitis. Discussion: Our findings were corroborative with other studies on acute movement disorders,suggesting drugs, neuropsychiatric,vascular and metabolic causes as the most common causes.We found that vascular and metabolic causes predominantly have hyperkinetic presentation,while neuropsychiatric causes have hypo kinetic presentation in the form of catatonia and drug induced causes have mixed picture in the form of Parkinsonism with dyskinesias and oculogyric crisis. Conclusion: Movement disorder emergencies are often encountered with a wide variety of clinical presentations ranging from hyperkinetic to hypokinetic to mixed movement disorders.Awareness of the different presentations and their prompt recognition is of utmost importance in diagnosing the etiology and initiating appropriate treatment.

Abstract ID: 570

EEG and Neuro-behavioural Assessment Scales as Predictors of Outcome in Disorder of Consciousness

M. K. Farsana, Subasree Ramakrishnan, C. M. Ravindranath, K. Raghavendra, Sonia Bansal, Dhaval Shukla

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: ayshafmk@gmail.com

Background and Aim: Patients surviving severe brain injury and entering coma, unresponsive wakefulness syndrome (UWS), or minimally conscious state (MCS) is increased recently. Electrophysiological assessment could identify patients with higher chances of clinical improvement. This study evaluated the possible diagnostic and prognostic role of clinical, and electrophysiological parameters in patients with disorders of consciousness (DoC). Methodology: This prospective study was conducted on 60 patients with DoC from May 2021 to November 2022. Diagnosis of DoC was made with standard diagnostic criteria. EEGs were analyzed using 2021 ACNS terminologies and various rating scales were applied. Patients were followed up serially for 6 months. Coma Recovery Scale-Revised (CRS-R) and Glasgow outcome scale was used to assess outcome. Data were analyzed using IBM SPSS software. Results: The median age of the study population was 42.50 years (IQR-31,55). The median hospital stay was 14 days. Patients in MCS and UWS had GCS, FOUR and CRS-R total scores higher than those in coma (p: <0.001). Thirty-two (53.3%) patients improved whereas 28 (46.7%) patients did not. Patients with alpha and theta frequency of the background activity were found to have MCS and better outcome than those with delta frequency (p:0.039). Discussion: Patients with Synek I, AFR 7, and Estraneo <3 had a high median CRS-R score and better outcome (p:0.041). On univariate analysis age, type of DoC, ventilator requirement, median GCS, FOUR, CRS-R scores at initial assessment, frequency of the EEG background activity, and Estraneo EEG score <3 were found to have a significant association with outcome. Conclusion: Younger age, higher FOUR score, and Estraneo <3 (presence of normal and mildly abnormal EEG background activity) were found to be independent predictors of a better outcome. The prognostic validity of the CRS-R remains unproven in the acute phase. EEG can be used as a bedside diagnostic and prognostic tool in patients with DoC.

Abstract ID: 571

Case Series of Posterior Reversible Encephalopathy Syndrome as a Complication of Pregnancy

Justin

DM Neuro Completed, Other

E-mail: drjustin2001@yahoo.com

Background and Aim: Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological syndrome characterized by a headache, seizures, altered mental status and visual loss and characterized by white matter vasogenic edema affecting the posterior occipital and parietal lobes of the brain predominantly. Common triggering factors include blood pressure fluctuations, renal failure, eclampsia, exposure to immunosuppressive or cytotoxic agents and autoimmune disorders. Reversibility of the symptoms and characteristic imaging findings helps us to diagnosis of PRES and prevents the morbidity. The present study highlights the clinicoradiological spectrum of PRES in patients with Eclampsia. Methodology: Observational cross-sectional study of 50 cases of pre-eclampsia and eclampsia patients in a territory care centre, Madurai. The cases were evaluated clinically with appropriate investigations including Neuroimaging. Results: Out of 50 patients 34 cases had eclampsia and 16 cases had severe Preeclampsia. Most common age group affected was between 20-25 years old and incidence was more in multigravida. 22 patients developed PRES in antepartum Period and 28 patients developed PRES in postpartum period. Headache with visual obscuration and seizures were the most common manifestation in our patients. Out of 16 patients with seizures GTCS was the most common seizure type and only 2 patients had multiple episodes. 3 out of 50 cases developed encephalopathy and required intensive care. on imaging 32 patients have typical features of PRES and 18 patients have atypical features involving cerebellum, basal ganglia, brainstem with hemorrhages. Discussion: It is of particular importance not to exclude PRES as a possible diagnosis when we have the appropriate clinical presentation which is not accompanied by the typical radiological findings since this clinical-radiological syndrome can often be manifested with atypical MRI findings. The success of the therapy lies primarily in recognizing these findings. Conclusion:-.

Abstract ID: 572

Predictors of Good Functional Outcome in Patients with Acute Ischemic Stroke at Three Months from the Event: A Single-center Prospective Cohort Study

Sohini Chakraborty, Awadh Pandit, Ayush Agarwal, Ajay Garg, Rohit Bhatia, M. V. Padma Srivastava, Venugopalan Vishnu

All India Institute of Medical Sciences, New Delhi, India

E-mail: sohinichakraborty84@gmail.com

Background and Aim: Stroke is the fourth leading cause of death and fifth leading cause of disability in India, causing a significant proportion of patients to have chronic sequelae and persistent deficits. Prediction of recovery might act as warning and help early planning of rehabilitation. A practical and accurate tool will be monumental for predicting functional recovery in acute ischemic stroke patients. Our aim is to assess clinical, laboratory and radiological predictors of good functional outcome (mRS 0-2) at three months in acute ischemic stroke patients. Methodology: This is a single-center prospective-cohort study recruiting two-hundred adult≥18 years acute ischemic stroke patients with pre-stroke mRS≤1, within seventy-two hours of ictus, fulfilling the inclusion and exclusion criteria, from February 2022- September 2023. Detailed history, demographic data, onset to hospital arrival, glasgow-coma-scale at presentation, NIHSS at presentation (NIHSSa), at twenty-four, seventy-two hours, systolic, diastolic blood pressure, significant past comorbidities, NCCT ASPECTS, multiphase-CT angiogram (mCTA) collateral score, perfusion study, vessel wall imaging with plaque characteristics, time to thrombolysis/ thrombectomy, decompression, single/ dual anti-platelet, statin, electrocardiogram, echocardiography, holter, hemogram, electrolytes, coagulogram, lipid, thyroid, homocysteine and glycemic parameters, mRS at discharge (mRSd) and at three months will be assessed. Factors conferring good prognosis (mRS 0-2) at three months will be identified. Results: Ninety-nine patients recruited so far have been followed. Mean age was 54.56±16.7 years, comprising of 68% male, 42% hypertensive, 26% diabetic, 34% and 16% consumed tobacco and alcohol respectively. Mean NIHSSa and mRSd was 11 and 4 respectively. Twenty-nine, fifty-four and sixteen patients had mRS 0-2, 3-5 and 6 at three months respectively. Rest of the analysis is ongoing. Discussion: Apart from predicting functional recovery at three months, our study will demonstrate pattern of recovery among moderate-severely disabled acute stroke patients and determine mortality burden at ninety days. Conclusion: Improving our knowledge about predictive factors might enhance rehabilitative procedures in acute ischemic stroke patients.

Abstract ID: 573

Pattern and Predictors of Caregiver Burden in Stroke Patients Undergoing Decompressive Craniectomy

Sudhindra Vooturi, Anand Karnati, Praveen Yada, Sita Jayalakshmi, Subhash Kaul, Panem Sumanth, K. G. Sreehari, Habib Ali

KIMS Hospital, Hyderabad, Telangana, India

E-mail: sudhindragupta@gmail.com

Background and Aim: Background: Quality of life of stroke survivors (SS) who undergo decompressive craniectomy (DC) is intertwined with the care they receive and hence the burden on care-giver forms an inevitable determinant of recovery after stroke. Understanding the burden of delivering care to SS is often neglected. Aim: To evaluate patterns of burden on care-givers of SS and analyse predictors of this burden. Methodology: Methods: In this prospective observational study, burden of care-givers of 198 SS who underwent DC between September 2019 and 2021 were evaluated using Zarit Burden Interview, a scale of 1-5 where 1 represents no burden to 5-always burdened. The association between the reported burden was evaluated against the clinical characteristics of SS and extent of recovery of SS using modified Rankin Score. Results: Results: The average age of the study population was 47.00 ± 11.2 years. Post DC, poor outcome was observed in patients with a pre-DC GCS score of <=8, those on anti-platelets, diabetes and those who were discharged to a rehabilitation care. 58.0% of caregivers reported that they are always burdened and 16% reported that they are frequently burdened providing care. 33.3% of caregivers reported to do a retrospective review of consent. The burden increased linearly with mRS score, suggesting higher the mRS score, more the burden. Discussion: The decision to perform a DC for patients with stroke is challenging, particularly in developing countries like India, where families providing care for such stroke patients are under the perspective that saving a life is of priority despite the risk of disability. Conclusion: Conclusion: Post-DC nearly 75% of care-givers reported to being frequently or always burdened of providing care and one-third of care-givers suggested to review the consent for DC retrospectively. A higher mRS score was associated with a greater burden on care-giver.

Abstract ID: 574

Retrospective Hospital Based Study of Recanalization of Venous Sinuses after Cerebral Venous Thrombosis and Its Various Outcomes

Rohin Dubbal, Girish Kulkarni, R. Aravind, R. Subasree, M. Pooja

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: rohin.dubbal@gmail.com

Background and Aim: Cerebral venous thrombosis (CVT) is a rare form of stroke due to blockage of venous sinuses which leads to various clinical presentations. The aim of the study is to explore the rate of recanalization of thrombosed sinuses, factors a“ecting it and clinical outcomes. Methodology: In this retrospective largest single-centre cohort study, we included 150 patients of CVT-treated at NIMHANS hospital, India, who had initial and follow-up imaging (4 months or later). Recanalization of venous sinuses was classified as complete, partial or no recanalization. Outcome was measured in terms of modified Rankin Scale (mRS). Results: Among 150 patients (median age, 34 years; 55.6% females), more than half (59.2%) of the patients had left hemispheric haemorrhagic infarct with involvement of transverse (78.5%), superior sagittal (68.5%) and sigmoid (49.7%) sinus. Average duration of symptom onset to initial and follow up imaging was 9.6 days and 14.2 months respectively. Complete recanalization was seen in 55(36.5%), partial in 80(54.3%), and no recanalization in 15(10.2%) patients. Among these, 20.6% had signs of raised ICP. All the patients received subcutaneous unfractionated heparin for an average of 11.9 days and oral anticoagulation for an average of 15.4 months. Most common risk factor was anaemia (50.7%) followed by hyperhomocysteinemia (46.4%). In our study, 61.8% achieved mRS of 0 and 35.1% had mRS of 1, with headache being the most common symptom. Discussion: This study focused on recanalization in cerebral venous thrombosis (CVT) and its impact on patient outcomes. The findings provide valuable insights into the prevalence and sinus involvement in CVT. Additionally, the study identified common risk factors and highlighted the importance of anticoagulation for favourable functional outcomes. Further research is warranted to validate these findings. Conclusion: Recanalization (partial or complete) was seen in 89.8% patients with long term anticoagulation and correction of risk factors. There was no significant di“erence in the outcome with respect to partial or complete recanalization.

Abstract ID: 575

A Study of the Etiology, Clinical Profile and Outcome of Non Traumatic Coma in Patients Attending Emergency Department in a Tertiary Care Hospital in South India

Usha Budumuru, Padmaja Sowmini, K. Malcolm, Sakthi Saravanan, Kannan Vellaichamy, Viveka Saravanan, K. Mugundhan

DM Neuro Pursuing, Other

E-mail: usha.budumuru113@gmail.com

Background and Aim: Non traumatic coma remains one of the commonest emergency presenting to casualty. In this study we aimed to identify the etiological profile of patients presenting at emergency department with non traumatic coma and to assess the outcome pattern and clinical factors that can be useful in the prediction of overall prognosis. The prediction of prognosis will be beneficial as management of such patients can be expensive. This is of great relevance in developing countries especially for better effective utilisation of limited resources. Methodology: All patients aged > 18 years with signs and symptoms of non traumatic coma with GCS scores < 12 were enrolled in this study. Demographic characteristics, risk factors, general examination , neurological examination, and requisite investigations were taken. Results: Total of 63 patients who presented at emergency department of the tertiary care centre were included in the study. Out of the 63 cases 61.9% were males and 38% were females. The majority were in the age group of 50-60 years (22.2%). Abnormal neurological examination was found in 28.5% . Out of which motor weakness was the commonest finding noticed in 15 patients (83.1%). MRI Brain/ CT Brain was done in 52 patients. 25 patients showed abnormal findings. Discussion: In Our Study, The Common Etiologies Of Non Traumatic Coma identified in descending order of frequency were Neurological Causes- In 26 Patients (41.2%), Metabolic In 16 Patients (25.3%), Multifactorial In 09 Patients (14.2%). The mortality rate was 36.5%. CVA was the commonest cause of death identified in 10 patients out of 22 patients. 2nd most common cause of death was multifactorial etiologies. Conclusion: This study helps us to determine the therapeutic interventions needed to improve the prognosis of non traumatic coma. Seeking medical care within early hours of altered sensorium along with timely diagnosis could reduce the mortality and improve outcomes.

Abstract ID: 576

Pure Neuritic Leprosy – Lessons Learnt and Strategies Moving Forward

Shrimantika Sarkar, Angel Miraclin, Aditya Nair, Geetha Chacko, A. T. Prabhakar, Ajith Sivadasan, Vivek Mathew, Sanjith Aaron

Christian Medical College, Vellore, Tamil Nadu, India

E-mail: shrimantika96@gmail.com

Background and Aim: ‘Pure neuritic leprosy’ is a distinct subset of leprosy, involving the peripheral nerves without cutaneous stigmata. It has poorer prognosis, treatment course is complicated by periods of neuritis and significant proportions develop residual deficits, despite treatment. We, in this study report our experience with pure neuritic leprosy, with emphasis on long term outcomes. Methodology: This is a retrospective observational study from a tertiary care centre in South India, where patients fulfilling the criteria for ‘pure neuritic leprosy’ were recruited. The demographic, clinical, electrophysiological data and biopsy findings were tabulated. The primary outcome was proportion with unfavourable outcomes at long-term follow-up along-with their predictors. Results: From January 2003 to May 2023, 99 patients had diagnosis of pure neuritic leprosy. The median age (yrs±SD) was 42 ±15 years with male preponderance (77%). Fifty three (53%) patients presented within 1 – 5 years. Sensory deficits (76, 76.8%) were most common followed by foot drop (49, 49.5%) and ulnar claw (36, 36.4%). Ulnar nerve thickening was found in 57 patients (57.5%) with bilateral involvement in 53.5%. Nerve biopsy showed borderline tuberculoid pathology in 75(75%). Electrophysiological pattern shows mononeuritis multiplex in 50 (50%). 96 patients had WHO disability grade 1 or 2 at diagnosis. All patients received with multi drug therapy (MDT) and 41 patients developed lepra reaction, necessitating steroids. The mean follow-up period was 3 years (IQR: 0.6 – 10 years), and 60% of the patients had disability. Development of lepra reaction is an important predictor of higher disability at follow-up. Discussion:-. Conclusion: A high index of suspicion and prompt management of lepra reactions are mandatory as pure neuritic leprosy presents late. Despite treatment, significant residual disability requires better therapeutic decisions tailoring to infection burden and host immune status. Management strategies of promise include high resolution ultrasound, polymerase chain reaction, steroid dose- duration and second line immunosuppressants.

Abstract ID: 577

Rare INO Variants- A Case of Lutz Posterior INO and A Case of Stroke Presenting as Isolated WEBIN

D. Dularijith, A.marian Vijay, K. Shanmugasundaram, M. Jawahar, Lakshmi Ranganathan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: dularijith@gmail.com

Background and Aim: To study the different patterns of INO. Methodology: Case vignette 1- 63 y male, Type 2 DM came with complaints of sudden acute onset of reeling sensation of self and surroundings with horizontal diplopia for both near and distant vision .On examination: Impaired bilateral adduction with abducting eye nystagmus with exotropia on primary position, impaired horizontal saccades, VOR, OKN, convergence, normal up gaze. MRI Brain – Diffusion Restriction in paramedian Ponto-mesencephalic junction Demyelination work up – Negative Case vignette 2- 23-year-old man with no prior comorbidities presented with acute onset of numbness of left upper limb and left lower limb associated with double vision on looking towards right. On examination he has right eye abduction restriction with adducting nystagmus on left eye,normal convergence with mild hypertonia of left upper limb and lower limb and normal power. Sensory and cerebellar examination normal. MRI Brain revealed T2/FLAIR hyperintense oval lesions suggestive of demyelination in pontomedullary junction, right cerebellar peduncle, bilateral centrum semiovale.Primary demyelination work up came negative. Results: WEBINO syndrome is an infrequent occurrence,and commonest etiology being demyelination.Here in our case it was vascular event and stroke presenting as isolated WEBINO is very rare.The second case is reported due to its rarity of presentation. Discussion: 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 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. In our case, the aetiology is ischemic stroke presenting as isolated WEBINO, which is very rare. Lutz Posterior INO is now known as INO of abduction characterised by abduction restriction occasionally associated with adduction nystagmus of the contralateral eye. The prenuclear origin of the disorder is based on morphologic and neurophysiologic evidence of an ipsilateral inhibitory connection between the PPRF and oculomotor nucleus running close to but separated from the MLF.Thus an ipsilateral midbrain or rostral pontine lesion may cause a supranuclear paresis of abduction. Conclusion: Careful evaluation of each patient with a gaze deficit should alert the examiner to the appropriate diagnostic work up.

Abstract ID: 578

In or Out? - A Case of Spinal Extradural Arachnoid Cyst

Muralidharan Kamalakannan, Malcolm Jeyaraj Krishnasamy, Mugundhan Krishnan

DM Neuro Pursuing, Other

E-mail: sridhardude37@gmail.com

Background and Aim: Spinal extradural arachnoid cysts are uncommon and symptomatic cysts are rare. The most common site is thoracolumbar region. We present one such case, which posed a clinical challenge, which was then better accounted by clinic radiological correlation. Methodology: 17 year boy, presented with flaccid paraparesis of one year duration. It started first in distal lower limb on right side, followed few months by buckling of both knees and then proximal weakness of both lower limbs. It was associated with thinning of both thighs and legs, and twitching in both thighs. There was no history of weakness in upper limbs, trunk and neck. There was no sensory disturbances, no bladder and bowel disturbances. Based on history, the possibility of cauda equina syndrome was considered. Examination showed wasting of intrinsic hand muscles, arm, thigh and leg and fasciculations were noted in both thighs over anterolateral aspect. Muscle power examination showed asymmetric weakness in lower limbs, right more weaker than left and there was differential weakness even within same segments. There was no objective weakness in upper limbs, trunk and neck . All reflexes were absent. After examination, possibility of anterior horn cell disease was considered. Results: MRI Dorsolumbar spine showed a large extradural arachnoid cyst in the dorsolumbar region, with minimal extension into cervical region, which could explain all the clinical findings. He was subjected to laminectomy with excision of the cyst, and the dural rent was repaired. Discussion: This case report highlights the rarity of extradural arachnoid cyst, which is caused by defect in the dura mater, leading to cyst formation outside the dura. Conclusion: We also emphasize the possibility of considering it in the differential diagnosis when patients presents with features of anterior horn cell- LMN syndrome or cauda equine syndrome, as a definite management is available.

Abstract ID: 579

Bilateral Thalamic Infarcts Due to Deep Venous Thrombosis – Two Cases, Two Outcomes

Navendu Jana, Amlan Mandal, Kaushik Sundar

DNB Neuro Pursuing, Other

E-mail: navendujana@gmail.com

Background and Aim: Two cases with bilateral thalamic infarcts due to deep venous thrombosis have been described here with extreme, different outcomes. Methodology: Case#1 The fortunate case 51-year-old, hypothyroid female was admitted with sudden onset severe headache and altered sensorium.Brain imaging (MRI, NECT) revealed bilateral thalamic infarcts.Infective,inflammatory,hypercoagulable,neoplastic lesions were ruled out by appropriate investigations involving CSF analysis,viral PCRs,imaging of other organs etc.CT angiography of cerebral circulation revealed deep venous sinus thrombosis involving vein of Galen,internal cerebral veins,inferior sagittal sinus,straight sinus and transverse sinus.She was put on anticoagulation and later on DOACs. Case#2 The difficult case A 47 year old male without any co morbidities was admitted with history of altered sensorium with fever for about three days.Brain imaging (MRI) revealed bilateral thalamic infarcts with involvement of basal ganglia and internal capsule.Similar workup was done as in the above case.CT angiography of cerebral circulation in this case too revealed deep venous thrombosis.However,he did not respond to anticoagulation as did the above case.He subsequently suffered a right thalamic hematoma.DSA was done to localize and confirm the venous obstruction which revealed a Galenic dural AVF with feeders from left occipital artery.Anticoagulation had to be stopped for the hematoma,and the AVF got blocked due to local thrombosis for the same.It was restarted so that the defect could be visualized.After recanalization,the fistula was closed by coil embolization of occipital feeder. Results: The fortunate patient showed remarkable improvement and is leading an independent life.However the difficult case patient showed an extremely slow recovery,though now has improved over time. Discussion: The cases demonstrate the varied outcomes due to different circulatory characteristics of the venous circulation. Conclusion: Bilateral thalamic infarcts have a specific list of differentials of which deep venous thrombosis needs to considerd with adequate weightage.The chicken egg situation of the fistula causing the thrombosis or vice versa needs to be kept in mind.

Abstract ID: 580

Opercular Syndrome Due to Bilateral Corona Radiata Infarcts

Deepak Rajendran, N. Arunkumar, E. Uma Maheswari, S. Balasubramanian, R. Lakshmi Narasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drdeepakrajendran@gmail.com

Background and Aim: Bilateral voluntary facial, pharyngeal, lingual, masticatory paralysis with automatic-voluntary dissociation is the hallmark of “Foix Chavany Marie Syndrome” (FCMS), also known as opercular syndrome. Classical FCMS results from the involvement of bilateral anterior opercular cortex. Here we present a case of FCMS with bilateral corona radiata infarcts, which is an atypical topographical location for FCMS. Methodology: Case study (case report). Results: 46 year old woman with no comorbid illnesses and with past history of left corona radiata infarct 6 months back with no residual disability, presented with acute onset slurring of speech and minimal left hemiparesis. She presented with in window period and was thrombolysed with Alteplase. MRI brain revealed acute infarct in right capsuloganglionic region and chronic infarct left corona radiata. She was subsequently discharged with residual dysarthria and minimal left hemiparesis. 3 weeks later, she presented with altered sensorium in the form of decreased responsiveness to her relatives. On examination, she was anarthric, her mouth was half open, with an inability to voluntarily close it fully, but she was able to yawn. She was unable to protrude her tongue, chew and swallow solid as well as liquid feeds, but she was able to laugh to jokes. She was able to move both upper and lower limbs. She had preserved gag reflex. MRI brain revealed acute infarct right corona radiata and subacute infarct left corona radiate. Discussion: Our patient had features of suprabulbar palsy with “automatic-voluntary dissociation” characterized by preservation of automatic, reflex movements (yawning and gag reflex) with paralysis of voluntary action of muscles innervated by cranial nerves. This is a hallmark feature of FCMS. Conclusion: We highlight that FCMS can also be caused by bilateral infarcts in the corona radiata. Greater awareness of this rare is essential to avoid delay in diagnosis and neurorehabilitation.

Abstract ID: 581

A Rare Case of O’Sullivan McLeod syndrome

Swetha Kannadhasan, V. Nandakumar, B. Krishnakumar, M. Jawahar, R. Lakshmi Narasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: sswethacmc26@gmail.com

Background and Aim: There are several disorders that primarily affect the motor nervous system and mimic amyotrophic lateral sclerosis (ALS) . These disorders are often referred to as atypical motor neuron disorders . One such disorder is O’Sullivan Mcleod syndrome. Our aim here is to present an extremely rare type of motor neuron disease. Methodology: Thorough workup with electrophysiology and neuroimaging were done to rule out other diseases with similar presentation. Results: No confirmatory test available. Discussion: Our patient was a 56 year old male whose illness started at around 33 years of age, initially with wasting and weakness of small muscles of right hand which progressed and involved similar muscles of left hand 4 years later, with proximal weakness of bilateral upper limbs for the past 4 years. On examination he was found to have wasting of bilateral hands, right more than left with fasciculations and polyminimyoclonus. EMG of small muscles of hand was suggestive of chronic denervation and MRI of cervical spine revealed features of AHC disease. Literature search was suggestive of O’Sullivan McLeod syndrome. O sullivan Mcleod syndrome is characterized clinically by slowly progressive weakness and amyotrophy of the distal parts of the upper limbs, mainly the hands, but sometimes extending into the forearms, in the absence of sensory or pyramidal signs. Most cases are of juvenile or early-adult onset. The main DDx include Hirayama disease, distal hereditary motor neuronopathy and multifocal motor neuropathy with conduction block. Neurophysiological studies of O’Sullivan–McLeod syndrome have revealed chronic denervation with normal NCS. Neuroimaging studies are usually unremarkable, although they can display symmetrical hyperintensity in the anterior horn (the so-called ‘snake-eye sign’) of the cervical spinal cord in T2- weighted sequences. Conclusion: Recognition of these atypical motor neuron disorders is important because several are potentially treatable or may not carry the same grave prognosis as ALS.

Abstract ID: 582

Where less is more: A Case of Reversible Cerebral Vasoconstriction Syndrome

Degapudi Sruthi, S. Balasubramaniam, R. Lakshmi Narasimhan, Uma Maheshwari, R. Lakshmi, K. Shanmugasundaram, S. Kaushik

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: sruthidegapudi@gmail.com

Background and Aim: Reversible cerebral vasoconstriction syndrome is characterized by segmental narrowing and dilatation of multiple intracerebral arteries lasting days to weeks. Methodology: 34-year-old woman with no medical illnesses was admitted with frequent headaches of two weeks’ duration. These were excruciating holocranial headaches of aching type, reaching peak intensity within few seconds of onset, in keeping with thunderclap headaches. They lasted between 10-60 mins and were occasionally associated with vomiting, and relieved by over-the-counter medication. Similar headaches had been occurring daily at a frequency of 2-3 per day. Examination was unremarkable with no signs of raised ICP, meningeal irritation or neurological deficits. Head CT showed focal hemorrhage in the left temporal region. CT cerebral angiography was done in suspicion of vascular malformation due to the atypical location of the bleed which showed multifocal alternate dilatation and narrowing in multiple branches resembling beaded appearance consistent with vasculitis. However, her presentation with recurrent thunderclap headaches, along with an RCVS2 score of 6 suggested a high likelihood of RCVS rather than CNS vasculitis. Blood investigations were normal and autoimmune screen was negative. MRI Brain revealed FLAIR dot sign and sulcal hyperintensities, which are highly specific radiologic features of RCVS. The patient was treated with analgesics and the headaches resolved over 2 weeks. CT cerebral angiography was repeated after 14 weeks which showed complete resolution of segmental narrowing. Results: Recurrent thunderclap headaches are pathognomonic of RCVS. Focal arterial narrowing is often misunderstood as being diagnostic of vasculitis. Discussion: Many patients with RCVS are subjected to brain biopsy and lifelong immunosuppressive therapy due to misinterpretation as CNS vasculitis and some others as ruptured brain aneurysms due to overlapping features of cerebral ‘vasospasm’ associated with thunderclap headache. Conclusion: This case highlights RCVS as a close differential to CNS vasculitis in the right clinical context and emphasizes the importance of differentiating the two.

Abstract ID: 583

Bilateral Corona Radiata Infarcts Masquerading as Opercular Syndrome

Deepak Rajendran, N. Arunkumar, E. Uma Maheswari, S. Balasubramanian, R. Lakshmi Narasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drdeepakrajendran@gmail.com

Background and Aim: Bilateral voluntary facial, pharyngeal, lingual, masticatory paralysis with automatic-voluntary dissociation is the hallmark of “Foix Chavany Marie Syndrome” (FCMS), also known as opercular syndrome. Classical FCMS results from the involvement of bilateral anterior opercular cortex. Other locations are opercular-subopercular syndrome, subopercular syndrome, unilateral anterior syndrome and posterior syndrome. Here we present a case of FCMS with bilateral corona radiata infarcts, which is an atypical location for FCMS. Methodology: Case study. Results: 46 year old woman with past history of left corona radiata infarct 6 months back with no residual disability, presented with acute onset slurring of speech and minimal left hemiparesis within window period and was thrombolysed with rt-PA. MRI brain revealed acute infarct in right capsuloganglionic region and chronic infarct left corona radiata. She was subsequently discharged with residual dysarthria and minimal left hemiparesis. 3 weeks later, she presented with altered sensorium in the form of decreased responsiveness. On examination, she was anarthric, mouth was half open, an inability to voluntarily close it fully, but able to yawn, was unable to protrude tongue, chew and swallow feeds, but was able to laugh to jokes. She was able to move both upper and lower limbs. She had exaggerated gag reflex. MRI brain revealed acute infarct right corona radiata and chronic infarct left corona radiata. Discussion: Our patient had features of pseudobulbar palsy with “automatic-voluntary dissociation” characterized by preservation of automatic, reflex movements (yawning and gag reflex) with paralysis of action of muscles innervated by cranial nerves. This hallmark feature of FCMS is seen in this case having bilateral corona radiata infarcts. The voluntaryautomatic dissociation in FCMS is explained by the existence of alternative pathways connecting the amygdala and hypothalamus to the brainstem. Conclusion: We highlight that FCMS can also be caused by bilateral corona radiata infarcts. Awareness of this rare presentation is essential to avoid delay in diagnosis and neurorehabilitation.

Abstract ID: 584

Toxoplasmosis - Unexpected Uninvited Guest Causing Chaos - A Case Report

G. Tamil Kumaran, V. Aravind Gautham, A. Nithyanandam, S. Velusamy, R. Lakshmi Narasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: tamil00660@gmail.com

Background and Aim: Toxoplasmic encephalitis is a common presentation of Toxoplasma gondii infection of the central nervous system in the late stage in AIDS patients with low CD4 count. Here, we present a case of CNS toxoplasmosis in an HIV patient with normal CD4 count. Methodology: Case study with review of literature. Results: A 36 years old male patient recently diagnosed to have HIV and on ART, presented with new onset headache and right focal seizures. MRI Brain revealed a left frontal SOL with surrounding edema. His CD4 count was 312 cells/mm3. He was empirically started on ATT and steroids and was discharged as he showed clinical improvement. After 2 weeks, he presented to us again with headache and altered sensorium for 2 days. Repeat imaging showed multiple ring enhancing lesions with eccentric nodules causing significant edema and mass effect in both cerebral hemispheres , right cerebellar hemisphere and left basal ganglia. CNS toxoplasmosis was considered after imaging and he was positive for serum toxoplasma IgG. He was started on trimethoprim sulfamethaxozole and steroids. He showed significant improvement and repeat imaging after 2 weeks of anti parasitic therapy showed partial resolution of lesions with minimal contrast enhancement and significant resolution of edema when compared with previous imaging. Discussion: Toxoplasmic encephalitis (TE) is caused by reactivation of latent infection by the protozoan Toxoplasma gondii because of progressive loss of cellular immunity. Approximately 90% of patients with TE have CD4+ T-Lymphocyte count less than 200 cells/mm3 and 75% have CD4+ T-Lymphocyte count less than 100 cells/mm3 at the time of clinical appearance. The most general signs contain fever, confusion, headache, and lethargy. Seizures develop in up to 30% of patients. 70% have focal neurologic symptom such as ataxia, sensory deficits, and hemiparesis. Conclusion: CNS toxoplasmosis should be considered in differential diagnosis even in HIV patients with normal CD4 count.

Abstract ID: 585

Doppler Evaluation of Carotid Arteries in Acute Ischemic Stroke

Venkatesh Kondle, M. R. Manivannan, P. K. Murugan, C. Justin

DM Neuro Pursuing, Other

E-mail: venkatesh.kondle@gmail.com

Background and Aim: Stroke is focal neurological deficit of vascular origin. Doppler ultrasound is safe, non-invasive modality in carotid evaluation. It estimates the degree of stenosis, characterizes and identifies plaques with risk of embolization and haemorrhage. Methodology: Observational cross-sectional study of 100 cases of AIS patients in a tertiary care centre, Madurai from October’22 to April’23. Study was done using ultrasound capable of B-mode imaging, pulsed–wave duplex scanning, and Doppler flow imaging. Patients with haemorrhagic stroke were excluded. Results: Out of 100 patients, 60 were males and 40 were females. Mean age was 65. Majority (n=82,80%) were aged >50 & 18(20%) were <50. Plaques were seen in 75(75%) cases. B/L ICA plaques were seen in 38(50%). 22(30%) patients showed plaques in right ICA only and 14(20%) patients showed plaques only in left ICA. Out of these 112 plaques, 56(50%) were in carotid bulb region, 36(32.5%) in proximal part of ICA, and 20(17.5%) in distal part of CCA proximal to carotid bulb, demonstrating carotid bulb to be common site for plaque formation. Out of the 112 plaques, 16(14%) showed >230cm/sec PSV (>70% stenosis), 26 had PSV of 125-230cm/sec (>50%stenosis) and 70 had <125cm/sec (<50% stenosis).Most (n=54; 48%) of plaques were heterogeneously hyperechoic (type III). 23((21%) were uniformly hyperechoic (Type IV), 22(20%) were type II (heterogeneously hypoechoic)and 12(11%) were type I (uniformly hypoechoic). Discussion: Age >50, male sex, smoking, HTN, diabetes, and hyperlipidemia were associated with an increased rate of stenosis. IMT> 0.8mm represents the earliest change of atherosclerosis. An atheromatous carotid plaque is described as a focal increase in the IMT (>1.2 mm). Conclusion: Doppler is a very useful, non-invasive modality in detecting site, morphology of plaque, quantifying degree of stenosis. Early detection of stenosis by carotid Doppler in highrisk patients would be of great importance in managing and preventing ischemic stroke.

Abstract ID: 586

Analysis of Transcranial Doppler Velocimetry in Middle Cerebral Artery Stroke in Comparison with Magnetic Resonance Angiography

Hrishikesh Pai, B. Kannan, Thomas Raj, Soumya Das

DM Neuro Pursuing, Other

E-mail: hrishikeshhrishi30@gmail.com

Background and Aim: Transcranial Doppler is non Invasive,Inexpensive and safe technique providing real time information of Intracranial blood flow patterns.It can be done bed side.Magnetic Resonance Angiography is commonly done as part of MR imaging in stroke work up for defining location and extend of vascular compromise.Aim:To analyse usefulness of Transcranial Doppler which is relatively inexpensive to Magnetic Resonance Angiography in Middle Cerebral Artery Stroke patients. Methodology: Prospective descriptive study in Tertiary care centre in south India.Study duration of 10 months.230 patients of stroke screened and 52 patients meeting Inclusion criteria and Exclusion criteria analysed.Demographic profile including age ,sex,medical history,risk factors,type and duration of stroke,investigations was noted after informed consent.Transcranial Doppler to analyse Peak flow Velocity,Mean flow velocity,Pulsatility Index,Resistance Index and compared with Magnetic Resonance Angiography.Findings described in simple descriptive manner.SPSS Version 24 used for statistical analysis. Results: 52 Patients Male 65.3%(34) Female 34.6%(18),Mean age 60years.35-50yrs 3.8%(2)50-70yrs 82.6%(43),>70yrs 13.4%(7)Hypertension 25%(13),Diabetes 28.8%(15),smoking 26.9%(14),Alcohol 23.07%(12),Dyslipidemia 34.61%(18).No temporal window 15.3%(8).Right MCA territory infarct 44.2%(23),Left MCA Infarct 55.7%(29),Transcranial Doppler flow abnormalities Increased Peak and Mean flow velocity 59.6%(31),MRA abnormalities 53.8%(28),Carotid Extracranial doppler abnormalities 38.4%(20).46.1%(24) having both Transcranial and MRA Abnormalities.Bilateral MCA Increased Peak and mean flow velocities 13.4%(7),Increased Pulsatility and Resistance Index 9.6%(5).Peak Flow Velocity 80-100cm/sec-34.6%(18),100-120cm/sec-19.2%(10),>120cm/sec-5.7%(3).Mean flow velocity 80-100cm/sec-53.8%,>100cm/sec-5.7%(3).11.5%(6)had normal flow velocities,13.4%(7)had increased peak flow and mean flow parameters in contralateral side with decreased flow pattern on the side of infarct. Discussion: Of the 52 patients screened with Transcranial doppler 53.8%(28) had MRA showing occlusion and 42.3%(22)had stenosis on carotid doppler.85.7%(28) of abnormal MRA had Transcranial doppler flow abnormalities.Mean flow of >80 cm/sec,peak flow of >100 is significant.Flow rate of >100cm/sec and Peak flow>120 is suggestive of significant stenosis. Conclusion: Transcranial Doppler flow study can be used to assess patients of stroke with good reliability in resource limited settings and can be also used for prognostication.

Abstract ID: 587

Polyradiculopathy in Patients with Raised Intracranial Tension- A Case Series of 3 Cases

Shreyasi Banerjee, Amrit Chattopadhyay

Kolkata Medical College, Kolkata, West Bengal, India

E-mail: shreyasibanerjee100@gmail.com

Background and Aim: Raised Intracranial tension either idiopathic or secondary to any cause usually presents with headache, vomiting, visual blurring, ophthalmoplegia etc. Polyradiculopathy in a case of raised ICT is rare. Here we report 3 cases with raised ICT presenting with polyradiculopathy. Methodology: This hospital based observational study was conducted in the dept of Neurology, Medical College, Kolkata from March 2022- March 2023 among the patients presenting with features of raised ICT. Necessary data were collected from detailed history taking, Clinical examination, relevant blood investigations, Imaging e.g. MRI brain and Cervical Spine, MR venogram, CSF study and NCS. Results: Here we report 3 cases who, besides having usual manifestations of raised ICT (Headache, LR palsy, Papilledema), showed some atypical features in the form of- Case 1 ( 23 years female)- 3rd cranial nerve palsy, weakness of right upper limb and generalized hyporeflexia Case 2 ( 42 years male)-areflexic quadriparesis Case 3 (46 years male)-Right LMN 7 th palsy with areflexic quadriparesis In all cases CSF pressure was high (>25 cm H2O), CSF routine study was normal, MRI brain and spine does not reveal any structural lesion, MR venogram was normal in case1 and it showed thrombus occluding left transverse sinus in case 2 and Superior sagittal sinus in case 3. NCS showed absent or impersistent F wave in all cases. Discussion: Polyradiculopathy in case of raised ICT occurs in case of acute rise in CSF pressure and patients usually present with rapid deterioration. Except for F wave abnormalities, rest of the NCS is normal. With therapy it is quite reversible. Conclusion: Hence polyradiculopathy can be a manifestation of raised ICT. Recognition of this entity is required as it can narrow down the investigation burden on the patient and with early intervention it shows favorable outcome.

Abstract ID: 588

Carotid Endarterectomy, Without Shunt, Under Continuous Scalp EEG Monitoring

Jayanth Shanthakumar, Sujith Kumar, Ravi Mohan Rao

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: jayanthss92@gmail.com

Background and Aim: Carotid endarterectomy (CEA) is indicated in patients with symptomatic or asymptomatic carotid stenosis (>70%). Methodology: Consecutive patients of symptomatic or asymptomatic carotid stenosis (>70%) based on CT angiogram, presenting to Apollo Hospitals, Seshadripuram, Bangalore, from May, 2012 to January 2023 were included. All patients underwent continuous scalp EEG monitoring during CEA. Carotid cross clamping times were recorded. All patients had postoperative neuroimaging & angiograms. Results: 60 patients underwent carotid endarterectomy under GA. The age of patients ranged from 33 to 87 years. 47 patients were male. 23 patients underwent CEA for asymptomatic carotid stenosis, while 32 for symptomatic stenosis. 17 patients with coronary disease, underwent simultaneous CEA & Coronary artery bypass grafting. 13 patients had bilateral carotid stenosis. The carotid cross clamping times in 60 patients ranged from 22 minutes to 49 minutes with an average of 32 minutes. No intraoperative EEG changes were noted in 59 patients. One patient, who had bilateral cervical ICA occlusions and underwent ST-MCA anastomosis for recurrent TIA’s, needed external carotid endarterectomy on the side of EC-IC bypass had reduction of EEG amplitude, which resolved on removing the cross clamp after CEA. No patient required insertion of carotid shunt during the cross-clamping time. No patients had postoperative strokes. One 87-year old patient, with symptomatic carotid stenosis, who showed no EEG changes during surgery, developed hemiplegia post operatively. His MRI showed ischemic reperfusion injury. Discussion: Intraoperative EEG monitoring during carotid endarterectomy assesses for acute ischemia following clamping. Conclusion: Intraoperative EEG monitoring predicts safety of CEA under GA. Meticulous CEA technique & not the cross-clamping time is the key.

Abstract ID: 589

Reflexes on the the Ability to Inhibit a Response on the Performance of Flanker Task in the Subjects of Cerebrovascular Accidents

Harshdeep Singh, Kuljeet Anand

Pursuing MD Medicine, Other

E-mail: harsh.gill07@gmail.com

Background and Aim: Cerebrovascular Accidents (CVA) cause abnormal or asymmetrical reflexes contributing to motor impairments. Since the tendon reflexes are mediated at the spinal cord its effects on the ability to inhibit a response is overlooked. This study aims to find the contributions of reflexes on the ability to inhibit a response on the performance of Flanker task. Methodology: A total number of 46 subjects of CVA were recruited for study. Deep tendon reflexes (DTR) of the both upper extremities were assessed. Later, Flanker task was performed on all the subjects and the mean Reaction Time (RT) along with both the congruent and incongruent stimuli were evaluated. For the final analysis Kruskal Wallis test was performed to see the difference between the DTR and performance of the Flanker Task. Results: The Kruskal Wallis test results showed significant difference between the DTR scores, X2(2) = 11.328, p= 0.023 with the mean RT of the flanker task and X2(2) = 9.531, p= 0.049 with mean RT of the Incongruent Stimuli. Whereas the result found non-significant difference for mean RT of the Congruent Stimuli. Discussion: Each DTR score is distributed differently with the mean RT of the flanker task and for the incongruent stimuli as well but not for the congruent stimuli. Conclusion: The abnormal tendon reflexes in CVA may be contributing to the ability to inhibit a response on the performance of Flanker Task and may be an indicator of the abnormal cognitive performance. Further research is needed to evaluate how the RTs are distributed with each DTR score.

Abstract ID: 590

Neutrophil-Lymphocyte Ratio as a Prognostic Marker for outcome in Acute Ischemic Stroke Thrombolysis with Alteplase

Arvind Visweshwar, Manivannan, Ramu, Senthur Pandian

DM Neuro Pursuing, Other

E-mail: dr.arvind.visweshwar@gmail.com

Background and Aim: The purpose of this study is to evaluate the relationship between neutrophil-to-lymphocyte ratio (NLR) at admission with safety and efficacy outcomes in acute ischemic stroke patients undergoing thrombolysis with IV ALTEPLASE. Methodology: 100 Patients presenting to GRH, Madurai with stroke symptoms were subjected to CT Brain and was classified as acute ischemic stroke and hemorrhagic stroke. Patients with ischemic stroke in window period were evaluated with NIHSS and ASPECT Scores and blood samples for Neutrophil and Lymphocyte ratio were drawn. These patients were subjected to IV thrombolysis with ALTEPLASE and was monitored for 24 hours. These patients were reviewed for outcome with NIHSS score 24 hours post thrombolysis and 7 days post thrombolysis. Primary outcome was an improvement in NIHSS score and improvement of motor power according to MRC scale. Results: out of 100 patients 65 patients had ischemic stroke and 55 patients presented within window period and was treated with thrombolysis. 35 patients with NLR <3.8 were associated good outcome post thrombolysis and 20 patients with NLR >4 were associated with poor outcome post thrombolysis. Discussion: our study has shown that patients with low NLR ratio were associated with better outcome than the patients with higher NLR ratio regardless of the reason for neutrophil increase. A. Conclusion: by using NLR ratio as a prognostic marker in acute stroke thrombolysis, outcome of the procedure can be predicted, an option of mechanical thrombectomy in patients with higher NLR ratio.

Abstract ID: 592

Study of Status of Intracranial and Extra Cranial Vasculature and Brain Parenchyma in Patients Presenting with Lacunar Infartcs

Sreenath Nair

DM Neuro Pursuing, Other

E-mail: dr.sreenathgnair@gmail.com

Background and Aim: Lacunar infarcts are small subcortical infarcts due to occlusion of a single perforating artery. Many with lacunar infarcts have hypertension,diabetes , dyslipidemia , and Leukoaraiosis (WMH) and cerebral microbleeds (MB) as common associations. Stenosis of cranial vessels are also seen .We studied the correlation between the common risk factors and changes in brain parenchyma and intra ( IC)and extracranial (EC) vessels in lacunae infarct patients.To study the changes happening in intra cranial and extra cranial vasculature and brain parenchyma in lacunar infarcts. To find out the correlation between vascular and brain parenchymal changes and common risk factors of lacunar infarcts. Methodology: Symptomatic lacunar infarcts were classified into 5 syndromes .History of common risk factors were obtained. 1.5 T MRI with venography of brain used to assess parenchymal changes like WMH and MB. Patency of IC vessels were assesed using MR angiogram while carotid and vertebral doppler used for EC vessels. Changes in IC and EC large vessels and brain parenchyma were correlated with common risk factors. Results: Majority of the lacunar infarcts were asymptomatic , located in the basal ganglia and were single .Strongest associations with lacunar infarcts were for age, high bloodpressure,Internalcarotid artery stenosis , more pack- years of smoking, diabetes mellitus and female sex. Age, cardiac diseases and diabetes mellitus were independently associated with brain parenchymal changes. Dianetesmellitus,high bloodpressure ,cardiac disease,advanced age and female sex had more stenosis in intracranial large vessels. Discussion: Data was assessed using SPSS 23.0 software. Risk factors , changes in IC and EC vessels and brain parenchyma are represented in frequency table. Association between variables were found using Regression analysis and chi square .Results were compared with the standards and the differences pointed out. Conclusion: Female patients with advanced age , diabetes mellitus and cardiac disease have more changes in intracranial and extracranial large vessels and brain parenchyma .

Abstract ID: 593

Circadian Features of Episodic Cluster Headache

Navneet Kumar, Mohd Amzarul

DM Neuro Completed, Other

E-mail: neuroindia3@gmail.com

Background and Aim: Cluster headache (CH) is the most common type of the TACs and is considered one of the most severe pain syndromes in humans. The most common presentation of cluster headache is the episodic nature followed by a complete remission for months or years. In this article, we present that the circadian system may be abnormal in patients with cluster headaches and investigation into these circadian abnormalities may be important in elucidating the full causative mechanism of this disorder and future treatments for the disease. Methodology: In this study, 60 patients with cluster headache were recruited between April 2019 and Nov 2021 from Government Medical College, Kannauj. We evaluated the patients for circadian rhythmicity and time of cluster headache attacks. Results: Of the 60 patients seen, 39 (65%) had circadian rhythmicity with respect to timing of day and 27 (45.8%) patients had bouts during months of March to May with pain free remission period rest of the year. The most frequent timing of the episode was daytime distribution and was reported in the afternoon ( 11 am to 2pm). Discussion: Cluster headache is not very common but the clinical history of these patients was very typical of cluster headache. Conclusion: Cluster headache has a circadian relevance and understanding of this can help us in future chronotherapy and development of new potential clock modulating compounds.

Abstract ID: 594

Dural Arterio-venous Fistula in a Case of CVT

Raajneesh Tamilarasan, A. Nithyanantham, V. Aravindgowtham, S. Velusamy, R. Lakshminarasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: traajneesh@outlook.com

Background and Aim: The incidence of CVT is estimated to be 1.3 – 1.6 cases per 100,000 individuals. Dural arteriovenous fistulas (DAVFs) are abnormal connections between the dural sinuses and the venous system, which can occur as a complication of CVT. The incidence of DAVF after CVT is reported to be between 0.9 and 13%. It is believed that a thrombus in the cerebral venous system causes stagnation of blood flow leading to an increase in venous pressure and causing enlargement of pre-existing physiological arteriovenous shunts or neo angiogenesis, resulting in the development of a DAVFs. Methodology: Case study. Results: Case report: 30 years old male known case of CVT and Polycythemia diagnosed 3 years back, lost to follow-up for past 1 year, now came with complaints of headache of moderate intensity predominantly in the occipital region for 1 month. Initial imaging taken three years back revealed occlusion of Right transverse sinus and Sigmoid sinus with thrombosis extending into the IJV. Now the current imaging revealed multiple tortuous dilated vessels in the subarachnoid spaces on both sides and dural venous sinus appears dilated with multiple dilated tortuous vessels draining into – features suggestive of DAVF. Discussion: It is difficult to establish the causal relationship between CVT and DAVFs since most of the low grade DAVFs are asymptomatic. In our case there is a clear temporal relationship between both, since there is a well-documented history and imaging which showed CVT previously. High grade DAVFs are more serious condition and are associated with intracranial hemorrhage and other neurological problems. Treatment options of DAVFs include Endovascular embolization, surgical ligation, and radiation therapy. Conclusion: This highlights the importance of long-term follow-up imaging in patients with CVT to detect any potential complications such as DAVF, especially in high-risk patients. Early diagnosis and treatment of CVT can prevent these complications.

Abstract ID: 595

Vascular Risk Factors as Predictor of White Matter Disease in Patients with Acute Ischemic Stroke

Venkatesh Kondle, M. R. Manivannan, P. K. Murugan, C. Justin

DM Neuro Pursuing, Other

E-mail: venkatesh.kondle@gmail.com

Background and Aim: Background: White matter disease,also called Leukoriarosis, is detected by hyperintensities on T2 or FLAIR MRI. They are typically caused by ischemia. Both stroke and WMD are interlinked and share common risk factors. Stroke is considered a risk factor for WMD and vice versa. The purpose of this study was to assess the relationships between the extent of WMD and established vascular risk factors in acute ischemic stroke (AIS) patients. Aim: To investigate the association of cerebral WMD on MRI with vascular risk factors and laboratory findings in AIS patients and to study association of them and WMD. Methodology: Study population is the acute ischemic stroke patients admitted in Department of Neurology, Madurai medical college between August 2022 and April 2023. Patients were considered eligible for further analysis if MRI is completed within 2 weeks after stroke onset. All patients provided serum samples on hospital admission. Results: A total of 150 patients were analysed, Mean age was 57,112(75%) study population are males and 38%(25%) are females; 16(10.4%) had cardioembolic stroke, 40(27%) had Lacunar stroke; 97(65%) had HTN; 26(17.5%) are smokers; 124(82.5%) are nonsmokers; 96(64%) had diabetes; 27(18%) had CAD; 54(36%) had Dyslipidemia, 21(14%) had AF. Discussion: It is well known that age and hypertension are consistently associated with occurrence of white matter disease. Our study corroborates the evidence for the same. Conclusion: In ischemic stroke patients, other than age, hypertension diabetes and current smoking predicted the severity of white matter disease. Better control of hypertension and diabetes may ameliorate the white matter changes.

Abstract ID: 596

Cross Sectional Study of the Outcome after Stroke Thrombolysis in Patients Treated with in 3 Hours vs > 3- 4.5 Hours

Nisskala Harikrishnan, S. Saravanan

DM Neuro Pursuing, Other

E-mail: nisskala@gmail.com

Background and Aim: The use of recombinant tissue plasminogen activator with in 4.5 hours is an established therapy for acute ischemic stroke. Ever since the results of the International Stroke Thrombolysis registry (SITS-ISTR) confirmed the extension of the time window from within 3 hours to 4.5 hours, it became essential to study the importance of onset -to- treatment on the outcome. This study is to assess the outcome after stroke thrombolysis in patients treated with in 3 hours VS > 3- 4.5 hours. Methodology: All acute ischemic stroke patients who present with in window period of 4.5 hours are taken for study after obtaining informed consent. Patient is assessed for the fitness of thrombolysis using inclusion and exclusion criteria. CT brain is taken to rule out Haemorrhage.IV thrombolysis is done using INJ.Alteplase 0.9 mg/kg. NIHSS and CT brain is repeated after 24 hours to look for any worsening. Modified Ranking scale (mRS) is assessed at the time of discharge. Results: The patients who has been thrombolysed with in 3 hours has better outcome, lower risk of haemorrhage than patient who has been thrombolysed in 3- 4.5 hours window period. Discussion: There was significant difference in rates of functional independence (mRS<2) in patients treated within the 3 hour and 3-4.5 hour time windows. Early treatment is essential. The effect size of thrombolysis is time dependent. Conclusion: The door-to-needle time remains paramount and must be kept as short as possible to increase the chance of a positive outcome.

Abstract ID: 597

A Study of Etiological Spectrum, Clinical Profile, and Site of Cerebral Venous Sinus Thrombosis

P. S. Apsara, P. K. Murugan, M. R. Manivannan

DM Neuro Pursuing, Other

E-mail: apsara.aps73@gmail.com

Background and Aim: Cerebral vein and sinus thrombosis are rare compared to arterial stroke and often occur in young individuals. AIM: 1. Identify the etiology in patients with cerebral vein thrombosis. 2. Correlation between the site of venous occlusion and clinical parameters. Methodology: This study was performed on 50 patients as a hospital-based retrospective and prospective observational study. All patients with the final diagnosis of CVT, confirmed by imaging MRI/MRV or angiography were included. Results: The majority of patients were in the third decade of their age. Systemic diseases like anemia, polycythemia, OCP pills use and infections were the major risk factors identified, present in 5 out of 50 cases of each in CVT.26 patients had papilloedema,24 patients had focal deficits,30 patients had seizures,headache in 32 patients, cranial nerve involvement in 5 patients.MRV findings showed Superior sagittal sinus (the commonest sinus involved) involvement in 40 patients, Transverse sinus was involved in 32 patients followed by sigmoid sinus in 22 patients. Discussion: Patient of CVT usually presents with headache, seizure, papilloedema, altered sensorium, and focal deficits due to thrombosis of intracranial veins and sinuses resulting in hemorrhagic infarctions and raised intracranial tension.CSVT is an important and treatable cause of stroke. Conclusion: When an attempt was made to correlate the clinical profile with the topographic Radiological substrate like the involvement of the superficial/deep venous system or the pattern of infarction, there was no significant correlation to evolve a pattern of diagnostic significance, correlating with the involvement of sinus.

Abstract ID: 598

A Study on Predictors and Outcome in Post-stroke Seizures

V. S. Veerappan

DM Neuro Pursuing, Other

E-mail: veerasomu@gmail.com

Background and Aim: Stroke is one of the most common causes of epilepsy in elderly. The frequency of seizures after stroke is variously estimated at 4% to 10%, the risk of epilepsy is greater in patients with late onset of the initial seizure after stroke, and early-onset seizures are considered a risk factor for stroke-related death. So our aim was to study the predictors and clinical outcome of seizures following an episode of stroke. Methodology: This is a hospital-based, prospective, observational study conducted among eligible consecutive patients admitted in the Neurology department with acute stroke. Patients were studied for the type of stroke (ischemic vs. hemorrhagic; cortical vs. subcortical strokes), timing of seizures, seizure semiology and seizure recurrence (during follow-up), duration of hospital stay, and mortality. Relevant investigations done. Data was collected and correlated to various risk factors. Results: Out of the patients enrolled, ischemic stroke (IS) was the most common stroke subtype, followed by hemorrhagic stroke (HS), and IS with hemorrhagic transformation (ISH). Seizures mainly occurred in patients with hemorrhagic stroke followed by Ischemic stroke with hemorrhagic transformation. Majority of patients developed early seizures, of which most of them had immediate post-stroke seizures. Discussion: In Patients with HS the incidence of early seizures was higher than late onset seizures. Focal motor seizures was the predominant types of seizures both in early onset and late onset group. Both in ischemic and hemorrhagic stroke involvement of the cerebral cortex was associated with the development of seizures. Patients with post stroke seizures had a longer mean duration hospital stay and increased disability. Conclusion: Our Study Showed hemorrhagic stroke, cortical involvement, previous history of stroke, were associated with the development of post stroke seizures and they had more disability in follow-up.

Abstract ID: 599

LDL Levels and Ejection Fraction in Ischemic Stroke Patients: A Hospital Based Cross-sectional Study

Soumya Das, B. Kannan, Thomas Raj, Hrishikesh Pai

DM Neuro Pursuing, Other

E-mail: soumyakantidas@gmail.com

Background and Aim: Ischemic stroke is a top contributor to mortality amongst non-infectious diseases. Atherosclerosis is a major risk factor. This study aims to assess LDL levels and ejection fraction in ischemic stroke patients. Methodology: This descriptive cross-sectional study was conducted in Government Thoothukudi Medical college from November, 2022 to april, 2023. 35 patients admitted in the neuromedicine ward and diagnosed as acute ischemic stroke were included in the study after informed consent and ethical clearance. Patients with acute neurological deficit and evidence of infarct on diffusion-weighted MRI were included. Patients with no evidence of infarct on DWI, those already on lipid lowering therapy, patients with a history of cerebrovascular disease or transient ischemic attacks were excluded. Blood for lipid profile taken after 8 hours of overnight fasting. Echocardiography done for all patients. Mean age calculated. Categorical data represented as percentages. Results: Of the 35 patients, 20 (57%) were men, mean age was 62.4 years. 57% ischemic stroke patients had their LDL levels elevated, 28.6% had decreased HDL, 28.6% had total cholesterol elevated, and in 14.3% triglycerides was elevated. This study also observed the cardiac status of the patients which showed 29 % patients had decreased ejection fraction. Discussion: From result, more males had ischemic stroke which is in line with available evidence. Triglycerides was normal in majority of the patients. In close to one third patients HDL levels were lower than normal. LDL levels were high in 57 % cases which corroborates with available literature .10 subjects, all with elevated LDL levels, had low ejection fraction. This reinforces the potential of LDL accumulation as a risk factor for atherosclerosis. Small sample size was a limitation in this study. Conclusion: This study concluded that most patients had elevated LDL levels which is an independent risk factor of ischemic cerebrovascular disease. Larger sample sizes with follow-up studies needed.

Abstract ID: 600

A Study of Diffusion Tensor Imaging in Central Post Stroke Pain: Travelling Beyond the Pain Pathways

Camelia Porey, Sanjeev Bhoi, Menka Jha, Suprava Naik

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

E-mail: cporey@gmail.com

Background and Aim: Central post stroke pain, seen in the aftermath of stroke, is an underdiagnosed entity but nevertheless quite a disabling complication. All the postulated theories regarding the pathogenesis of CPSP points to its origin in the central pain pathways. However this study attempts to demonstrate the role of other contributing areas in the generation of CPSP. Methodology: In this single centre tertiary care hospital based study, 24 patients with both ischaemic and haemorrhagic stroke of variable durations were recruited and Magnetic Resonance Imaging (MRI) imaging with Diffusion tensor imaging (DTI) acquisition was done. Fractional anisotropy (FA) and Apparent diffusion coefficient (ADC) values of the Spinothalamic tract ( STT), Corticospinal tract (CST), Superior thalamic radiation ( STR), basal ganglia ( BG) and primary somatosensory cortex ( SSC) were compared between normal and abnormal sides and also in extrathalamic lesions separately. Results: Significantly difference with lower FA were noted in STT, CST, STR and SSC and higher ADC value in BG, STR, CST and SSC on comparison between the normal and lesion side. On individual sub analysis, ischaemic stroke had significant changes in the FA value of CST and ADC value of STR and CST while haemorrhagic stroke had significant changes in FA and ADC value of STR and SSC as well as FA value of STT. In analysis of the extrathalamic strokes the significance persisted in all the studied parameters except the BG. The CST abnormalities were evident even in patients with clinical motor improvement. Discussion: Among different studies for motor deficit in stroke, in a review by Moura et al fractional anisotropy (FA) measured at an early phase after stroke was stated as potential predictors of motor recovery in majority of the studies.(11,12). Werring et al in his study demonstrated reduced FA associated with cerebral infarction in CST remote from the lesion in ischaemic stroke.(13) Puig et al stated that recovery of motor function involves remodelling of the CST proper and/or a greater reliance on alternative motor tracts through plasticity. DTI-metrics represent promising clinical biomarkers to predict motor recovery and to monitor and predict the response to neurorehabilitative interventions. Conclusion: Contrary to the belief of pain pathways being solely responsible for CPSP, the role of other contributing areas are also evident by this study and this may also be evolved as a biomarker to prognosticate neurorehabilitation if done in a larger population.

Abstract ID: 601

A Case of Horizontal Gaze Palsy with Progressive Scoliosis

Kaushik Senthilnathan, Lakshmi Narasimhan, S. Velusamy, Nirumal Kumar, K. Shanmugasundaram, Vinoth Kannan, Manikavasagam

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: kaushik1946@yahoo.com

Background and Aim: 12 year old boy born of non consanguineous marriage presented with chief complaints of bending of spine since 6 months of age and restriction of eye movements since 1 year of age. Methodology: On examination patient had a scoliosis with convexity to the left. Extra ocular movements - Horizontal gaze palsy+, with intact vertical gaze and convergence. Results: Xray spine- Scoliosis+ MRI brain- Normal with intact facial colliculus. Discussion: Horizontal gaze palsy with progressive scoliosis is a rare autosomal recessive disease due to mutation in ROBO 3 gene. A combination of horizontal gaze palsy with progressive scoliosis presents in early childhood. Imaging is usually suggestive of uncrossed motor and sensory pathways. Horizontal gaze palsy is due to involvement of abducent nucleus or PPRF (Paramedian pontine reticular formation). • The pathophysiology behind scoliosis is less well understood, one hypothesis is poor development of extrapyramidal projections in the reticular formation. Conclusion: HGPPS is a rare condition and should be in the differential diagnosis of patients presenting with early onset gaze palsy with skeletal abnormalities. Genetic testing is the key to diagnosis.

Abstract ID: 602

Elsberg Syndrome in VZV Infection

S. Pramod, P. R. Sowmini, S. Lakshamanan, K. Mugundhan

DM Neuro Pursuing, Other

E-mail: pramod.sam13@gmail.com

Background and Aim: Elsberg syndrome is a rare, mostly self-limiting condition characterized by acute to subacute onset lumbosacral radiculitis, with or without myelitis confined to lower spinal cord. HSV-2, reactivation or occasionally primary infection is the most commonly reported cause of Elsberg syndrome, with VZV, West Nile and SARS – CoV being the other infectious causes. We report a case of clinically definite Elsberg syndrome, caused by VZV reactivation. Methodology: A 70-year-old lady presented with vesicular skin lesions in the perineal region, right buttock, right foot dorsal and plantar aspect. 4 days later she developed severe burning pain in the same regions followed by urinary disturbances in the form of urgency and increased frequency. Two days later she lost sensation of bladder fullness and developed retention of urine. On examination she had grouped vesicles in L5, S1 and S2 dermatomes. She had spasticity both lower limbs, with normal power and bilateral brisk knee and ankle jerk, and bilateral extensor plantar reflex. All modalities of sensations reduced in the right L5 to S5 distribution with absent anal wink. Results: Her routine blood investigations were normal. MRI lumbosacral spine (plain and contrast) didn’t show any evidence of radiculitis or myelitis. CSF analysis revealed elevated protein and lymphocytic pleocytosis. Discussion: She is fitting into the diagnosis of clinically definite Elsberg syndrome, as per the Salvodi diagnostic criteria, based on the signs and symptoms of cauda equina involvement, subacute onset and progression less than three months, coexisting herpes zoster infection and CSF pleocytosis. Conclusion: Elsberg syndrome accounts for 5 -15% of the combined cauda equina syndrome and myelitis. Although radiologic findings are non-specific, MRI spine helps to rule out competing diagnoses, such as dural arteriovenous fistula, sarcoidosis, and other conditions. Elsberg syndrome should be considered in patients presenting with urinary disturbances associated with lumbosacral radiculitis.

Abstract ID: 603

Rare Forms of Genetically Mediated Familial and Sporadic Amyotrophic Lateral Sclerosis: An Indian Experience

Saraswati Nashi, Anoop Cheriyan, Nalini Atchayaram, V. Seena, Deepak Menon, Deepti Bhaskar, Keerthi, Mainak Bardhan, Kiran Polavarapu, Preethish Kumar, Gautham Arunachal, Ram Murthy, Veeramani, A. Nalini

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: nandanashi@gmail.com

Background and Aim: Amyotrophic lateral sclerosis (ALS) or Motor Neuro Disease is largely a sporadic disorder, but a small percentage is familial and genetically medicated. Here we have attempted to describe the genetic forms of ALS, their clinical phenotypes and genetic patterns identified among Indians. Methodology: Next Generation sequencing (NGS) was performed for all probands and family members wherever possible. The in-silico prediction analysis tools used for the identified SNVs were Polyphen2-v2.2.2r398, MutationTaster2 based on build NCBI37/Ensembl 69, Combined Annotation Dependent Depletion (CADD) PHRED score-GRCh37-v1.6. Results: A total of 26 patients with genetically proven ALS (M: F- 19:7) were studied. mean age at onset, illness duration and age at presentation are 42.58 years, 3.41 years and 46 years respectively. Initial symptoms were: weakness of proximal lower limb (34.6%), bulbar (23.1%), distal LL (23.1%) , distal UL (11.5%), generalized weakness and behavioral changes (3.8% each). At presentation, dysarthria was present in 53.8%, dysphagia (42.3%), emotional liability (30.7%), behavioral changes (30.7%), stiffness of LLs (34.6%). Around 80.7% of were independently ambulant and remaining 19.3% were wheelchair bound. Most common mutations were: SOD1 (26.9%) and C9ORF72 (23.1%) followed by FUS (7.7%), CHCHD10 (7.7%), TARDP (7.7%). Others were SETX, SPG11, OPTN, VCP, TBK1, FIG4, SPG7 (3.8%each). Discussion: Onset of symptoms in this cohort was noted in most of the patients by 4 th decade which early compared to sporadic ALS patients who had onset of symptoms by 5 th decade, as observed in one of the largest cohort sporadic ALS from our centre. However in Comparision to European population with genetic ALS, symptom onset is one or two decade earlier in Asian patients with GALS. Conclusion: Most common genetic variants identified in our cohort were in C9ORF72 and SOD1.Early recognition of these genetic mutations are important for considering genetic counselling and better understanding of this fatal disease.

Abstract ID: 604

Post-stroke Recrudescence

Saurabh Sultania

Dayanand Medical College, Ludhiana, Punjab, India

E-mail: saurabh211311@gmail.com

Background and Aim: Post-stroke recrudescence (PSR) is a phenomenon characterized by the temporary aggravation of neurological deficits in individuals with previous strokes, despite the absence of acute stroke findings on neuroimaging. Despite its clinical significance, PSR remains under-recognized, under-reported, and poorly understood. In this study, we present two cases of chronic middle cerebral artery (MCA) infarction with recent worsening of neurological deficits, where acute stroke was ruled out by neuroimaging. Through these cases, we highlight the association between localized infections and transient exacerbation of neurological deficits in chronic stroke patients. Methodology: Methods: This study employed a case report design along with a comprehensive review of relevant literature. Two cases of chronic MCA infarction with worsening neurological deficits were examined. Detailed clinical information, including patient demographics, medical history, neuroimaging findings, and subsequent management, was recorded. A thorough review of the existing literature on PSR and related topics was performed to provide contextual background information. Results: Results: Case 1: A 70-year-old male with a previously diagnosed large right MCA infarct presented with a deterioration of left hemiparesis and facial weakness. Neuroimaging revealed a chronic infarct in the right cerebral hemisphere without evidence of acute stroke. However, further evaluation identified a urinary tract infection, which was promptly treated. The patient reported symptomatic improvement within 4-5 days. Case 2: A 56-year-old male with an old left MCA infarct presented with worsening right hemiparesis accompanied by aphasia. Neuroimaging did not reveal any acute stroke findings. However, evaluation revealed maxillary sinusitis, which was treated accordingly. The patient demonstrated improvement within 3-4 days following sinusitis management. Discussion: These two cases illustrate the occurrence of transient worsening of neurological deficits in chronic MCA infarction without evidence of acute stroke on neuroimaging. Through further evaluation, localized infections were identified as the underlying cause of the exacerbation. Prompt resolution of symptoms was observed following appropriate treatment of the infections. Therefore, we attribute the transient worsening in these cases to recrudescence rather than stroke recurrence. Conclusion: Conclusion: These findings emphasize the importance of considering localized infections as a potential etiology for transient neurological deterioration in individuals with chronic stroke. Further research is warranted to elucidate the pathophysiological mechanisms underlying PSR and optimize its diagnosis and management.

Abstract ID: 605

Correlation between Platelet-To-Neutrophil Ratio and Hemorrhagic Transformation in Acute Ischemic Stroke Treated with Thrombolysis

V. Aravind Gautham, A. Nithyanandham, S. Velusamy, Lakshmi Narasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: aravindgautham.v@gmail.com

Background and Aim: Inclusion criteria:(1) Age >18 years, (2) Acute ischemic stroke confirmed by Computed tomography scan or magnetic resonance imaging (MRI), and (3) Onset of stroke symptoms within 4.5 hours and treated with r-tPA. Methodology: Stroke severity was assessed on admission using the National Institute of Health Stroke Scale (NIHSS) score. All patients underwent emergent computerized tomography (CT) scan before IVT to rule out the possibility of hemorrhagic stroke. Baseline characteristics were evaluated.Venous blood samples were collected at admission .The PNR value was calculated according to platelet and neutrophil counts at admission prior to thrombolysis therapy. We defined the PNR value at admission as pre-IVT PNR, the images were categorized into hemorrhagic infarct (HI) and parenchymatous hemorrhage (PH) according to the ECASS II criteria as follows: HI1, small petechiae along the margins of the infarct; HI2, confluent petechiae within the infarcted area but no space effect; PH1, blood clots in ≤30% of the infarcted area with some slight space-occupying effect; and PH2, blood clots in >30% of the infarcted area with substantial space-occupying effect. Results: Patients with low platelet to neutrophil ratio had higher hemorrhagic transformation when compared to patients with no hemorrhagic transformation following thrombolysis.But there was no significant correlation between subgroups (HT1 and HT2) with PNR.Patients with high NIHSS at admission were found to have low PNR and higher hemorrhagic transformation following thrombolysis. Discussion: Pitfalls: 1)All data were collected only in one hospital, and it might lead to selection bias.2)In addition, infection and other diseases that may affect inflammation that occurred during treatment were not taken into consideration.3)We lacked dynamic PNR data, which would be considered a more effective predictor. Conclusion: As a new parameter put forward recently, the research of PNR in the stroke field is still rare. This finding could help neurologists predict and improve stroke outcomes after rtPA treatment in clinical settings.

Abstract ID: 606

Clinical Profile of Non Motor Manifestation in Young Onset Parkinsons Disease in Eastern Indian Population

Praveen K. Yadav, Praveen Yadav

DM Neuro Completed, Other

E-mail: dr.praveen4u@gmail.com

Background and Aim: Non motor symptoms (NMS) are extremely common in Young onset Parkinson disease (YOPD) with high incidence of neurobehavioral and autonomic dysfunction which should be given greater emphasis as it will affect the quality of life. Methodology: All patients satisfying the UPKRDS Diagnostic criteria for Idiopathic Parkinson’s disease (IPD) attending the Neurology Outpatient services at a super speciality neuro clinic during study period July 1,2021 to April 30,2022 were included. All secondary causes of parkinsonism like drug induced, multi-infarct state and normal pressure hydrocephalus were excluded. All patients were examined, and demographic data and non-motor symptoms were documented using the non-motor scale (NMSS) of International Parkinson’s and movement disorder society and modified H & Y staging used for staging. Results: A total 32 patients were diagnosed with YOPD during the study period. Out of which19 (59.37%) were males and 13 (40.32 %) were females. Majority are coming under the age group of 40-50 years (84%) and onset of illness in 1 month to 5 years (78.17%) with equal distribution in both 1 – 1.5 and 2 H&Y staging (37%) each. Various non motor symptoms are manifested by the patients where anxiety (71.87%), memory loss (59.37%), depression (56.25%) bieng the commonest. Most of the patients are shown one or more autonomic symptoms and sleep disturbances. Discussion: The most common NMS is anxiety (71.87%) followed by memory loss (59.37%),depression (56.25%) and constipation (46.87%) compared to a study conducted in Indian populations which shows depression (45.6%), anxiety (45.4%), and apathy (30.5%).^[23].A similar study in USA shows similar rate of depression (48.3%). Conclusion: Non motor symptoms are shown by all patients under study (100%) with anxiety, memory loss, depression and constipation are being the commonest.

Abstract ID: 607

Magnetic Resonance Imaging of Muscles: Pattern of Involvement in Inflammatory Myositis

S. Sridhar, Saraswati Nashi, Seena Vengalil, Karthik Kulanthaivelu, Aneesha Thomas, Vijay Kumar, P. V. Prathyusha, A. Nalini, Deepak Menon, Deepti Bhaskar

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: sridharshashi577@gmail.com

Background and Aim: Idiopathic inflammatory myopathies (IIMs) are immune-mediated disorders characterised by proximal weakness. There are very few studies on the pattern of muscle involvement in different subgroups of IIMs. We aim to study pattern of muscle involvement in sub-groups of IIM. Methodology: Ambispective and observational study on muscle MRI of 125 patients with IIM. Modified Stramare and Modified Mercuri scales were used to assess severity of edema and atrophy respectively. Results: 125 patients were divided into four subgroups: DM, IMNM, OM and seronegative groups. F:M - 1.6:1. Median age at presentation was 35 years. Median duration of illness was 8 months. In patients with DM, adductor group of muscles showed severe edema. In leg, 46.8% had moderate -severe edema in peronei. 65.6% had significant fasciitis. In IMNM, 52.1% had severe edema in rectus femoris, semimembranosus. In leg, 78.2% patients had no edema in peroneus muscles. In OM, there was no specific pattern of involvement. In leg, gastrocnemius had moderate edema. In seronegative, vastus lateralis had severe edema. In leg, deep posterior compartment and peroneal group of muscle had subtle to no edema. Discussion: On Muscle MRI, we observed a specific pattern of involvement in each subgroup. In DM, severe edema was observed in adductors and moderate edema in leg muscles. Involvement of peroneus muscles in leg was a feature of dermatomyositis; Peronei were strikingly spared in other subgroups. In IMNM, Rectus femoris and semimembranosus had severe edema with sparing of peroneus muscles. In OM, there was diffuse mild to moderate edema. In the Seronegative group, vastii and soleus displayed moderate edema. Muscle atrophy was mild in all groups. Conclusion: Current study is one of the largest cohorts of muscle MRI in IIMs . This study expands the imaging spectrum of IIM and brings out the novel concepts of selective muscle involvement in subgroups of IIM.

Abstract ID: 608

Anti-N-Methyl-D-Aspartate Receptor Encephalitis: Clinical Characteristics, Treatment and Outcome Predictors

R. Amod, Ashalatha Radhakrishnan

SCTIMST, Thiruvananthapuram, Kerala, India

E-mail: anaswarakrish@gmail.com

Background and Aim: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common type of autoimmune synaptic encephalitis and it often responds to treatment. We analyzed the clinical characteristics of anti-NMDAR encephalitis including investigations,treatment and outcomes. Methodology: Hospital based retrospective prospective observational study • Study period -2012-2022.Included all NMDA patients ( CSF/serum) in the Prospective AIE registry with minimum follow-up of 1 year and adequate data.Excluded all other causes of AIE/immune mediated encephalitis/encephalopathy including intracellular neuronal antigen related AIE (paraneoplastic).Outcomes were assessed using mRS. mRS <3 – good outcome, mRS 3 and >3 – bad outcome. Both 3 months (short term) and 1yr (long term) outcomes assessed. Results: 55 patients were analysed.Mean age was 20yrs and was female predominant with seizures (56%) mainly extratemporal followed by behavioural symptoms (38.2%) were the commonest presenting symptoms.80% also had movement disorder mainly perioral dyskinesias.MRI was abnormal in 45% mostly non specific FLAIR changes only.EEG was abnormal in 92% with extreme delta brush in 5%.CSF abnormality in only 16%.First line treatment administered were IVMP+IVIG in majority followed by PLEX+IVIG.36% received second line.Relapse occurred in 10% mostly seizures. Good outcome was seen in 88% at 3months and 92% at 1yr.Factors favouring good outcome were age more than 18yrs and favourable mRS at admission.Factors for poor prognosis was abnormal MRI at presentation. Discussion: This is the largest study on NMDA encephalitis from Indian published till date.As compared to previous study our study also showed similar clinical presentation.Majority had EEG,MRI abnormality similar to previous studies but CSF abnormality was only in16%(60%) may be due to previous treatment.Predictor of outcome were similar to previous studies.Limitations were non randomised data and non uniform treatment. Conclusion: NMDA encephalitis is prevalent in female patients of reproductive age group. CSF and Serum NMDA was equally sensitive and specific.EEG was abnormal in most patients.Favourable outcome was obtained in 86% patients managed with immunotherapy.

Abstract ID: 609

Gut Dysbiosis in Indian Multiple Sclerosis

Gurkeerat Kaur, Dheeraj Khurana, Rajesh Pandey, Pallawi Kumari, Priti Devi, Aparna Swaminathan, Ashok Kumar, Usha Dutta, Poonam Khanna, Deepak Sharma, Pallab Ray

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

E-mail: kaurgurkeerat6@gmail.com

Background and Aim: Gut microbiome dysbiosis has been linked to Multiple Sclerosis (MS) through bi-directional gut-brain axis. Western studies have established that certain taxa may be mediating immune and metabolic factors that are associated with either disease pathogenesis or progression. Indian diet (plant-based diet) is markedly different from western diet, therefore, this study aimed to characterise the gut microbiota in Indian MS patients. Methodology: 84 MS patients (both untreated and treated) from outpatients’ department and MS clinic were recruited along with age-gender matched 106 healthy controls (HCs). Major inclusions were relapsing-remitting multiple sclerosis (RRMS) patients as per 2017 Mc Donald’s criteria with an Expanded Disability Status Score (EDSS) < 5.5 and exclusions were progressive forms of MS and no associated co-morbidities. Demographics, MS characteristics and detailed dietary characteristics were recorded. Stool samples were taken for 16S rRNA sequencing. Results: Median (IQR) age of RRMS patients was 32.8 (27.9 - 38.6) years, with 63 (75%) females and 21 (25%) males. Firmicutes was the most abundant and dominant phylum observed in our population (47.06% HCs vs 48.47% MS patients). Significantly reduced differential abundance of bacterial species such as Bacillus species of probiotic potential, Monoglobus pectinilyticus (pectin-degrading bacteria) and Faecalibacterium prausnitzii (commensal, probiotic, and butyrate-producing bacteria) were observed in MS patients. Metabolic pathways involved in the generation of long chain fatty acids (LCFAs) were significantly enriched in MS patients. Discussion: Dominant short chain fatty acids (SCFAs)-producing bacterial species in the gut of HCs were from phylum Firmicutes due to their higher dietary fibre intake. The gut dysbiosis in MS may disrupt balance between SCFAs, which have anti-inflammatory and immunoregulatory effects, and LCFAs, which have pro-inflammatory and disease- promoting effects. Conclusion: Gut dysbiosis exists in North Indian MS patients which has a distinct microbiome signature compared to Western MS patients and it may be linked to a low dietary fibre intake.

Abstract ID: 610

Predisposed HLA-DRB1 Alleles and EBV Infection Increases MS Susceptibility

Gurkeerat Kaur, Dheeraj Khurana, Ranjana Minz, Ashok Kumar, Prabhsimran Singh, Jagdeep Singh

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

E-mail: kaurgurkeerat6@gmail.com

Background and Aim: HLA region on chromosome 6 is linked to autoimmune diseases like multiple sclerosis (MS), with 20-30% of genetic susceptibility found on HLA locus. Some HLA DRB1 alleles increase MS risk, while others may be protective. Carriers of HLA DRB1*15:01 allele are three times more likely to develop MS than non-carriers. Epstein-Barr virus (EBV) infection is likely a prerequisite for MS onset, supported by epidemiological,virological and serological data. In present study we aimed to evaluate the association of HLA-DRB1 alleles and EBV infection to MS susceptibility. Methodology: MS patients of 18-60 years of age were recruited from MS clinic of our center. Demographic and clinical data were recorded. Serum and DNA extraction was carried out. Genotyping for HLA-DRB1 by using sequence-specific oligonucleotide (SSO) typing kits was carried out with Luminex technology. Qualitative in-vitro assay for IgG/IgM to EBV antigens was carried out. Results: 101 MS patients and 100 healthy controls (HCs) were recruited. Median (IQR) age of MS patients was 34.3 (28.7 - 41.1) years, with 68.3% females and 31.7% males, whereas age of HCs was 28.7 (24.8 - 36.1) years,with 54% females and 46% males. Multivariate binary logistic regression (adjusting for confounders; age and gender) was done to evaluate the effect of predisposed HLA DRB1 alleles and EBV infection on MS susceptibility. Association was significant in presence of EBV infection along with *15:01 alleles (adjusted OR: 2.111, CI: 1.071 - 4.160, p=0.031) as well as in presence of EBV infection along with *04:04 alleles (adjusted OR: 8.733, CI: 1.015 - 75.145, p=0.048) as compared to HCs. Discussion: HLA DRB1 alleles and EBV infection contribute to increased MS susceptibility in Indian MS, supporting the theory of their involvement in MS onset in Caucasians. Conclusion: Predisposing HLA DRB1 alleles (*15:01 and *04:04) and EBV infection were observed to have an additive effect in susceptibility to MS.

Abstract ID: 611

An Observational Study of “Movement Disorders” Related to Infectious Diseases

Chandra Rawat

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi, India

E-mail: drcsrneurology@gmail.com

Background and Aim: Movement disorders can be the initial manifestations or part of the disease spectrum of various infectious diseases. Infectious causes account for 20.4 percent of all secondary movement disorders.[1] We conducted this study to investigate the frequency and types of various movement disorders, in a cohort of infectious disease patients admitted to our institute “G. B. Pant Institute,” in New Delhi, India. Methodology: A hospital-based cross-sectional observational study . Results: Among the 61 patients (mean age 24.45 years), the most frequent aetiology found in our cohort was CNS tuberculosis in 26 (42.6%) patients, followed by SSPE in 16 (26.2%) patients, HIV/AIDS in 05 (8.19%) patients, dengue in 03 (4.91%) patients, Neurocysticercosis in 02 (3.27%) patients, post-streptococcal in 02 (3.27%) patients, post-covid in 02 (3.27%) patients, post Japanese encephalitis in 01 (1.63%) patient, post chickenpox in 01 (1.63%) patient, post diphtheria in 01 (1.63%) patient, Ascaris encephalopathy in 01 (1.63%) patient, and enteric encephalopathy in 01 (1.63%) patient. Dystonia was the most common phenomenology present in 23 patients, followed by tremor in 22 patients and myoclonus in 19 patients, with mixed phenotypes observed in 13 patients. Discussion: In our study tremor was the most common movement disorder seen in CNS tuberculosis patients. similarly, tremor was the most common phenomenology observed by Alarcon et al. in 30 patients of tuberculous meningitis.[2] In SSPE cohort, most common non-myoclonus movement disorder was dystonia seen in 10 patients. In HIV/AIDS patients stereotypy was most seen in three out of five patients and in patients of dengue encephalitis two patients had dystonia-parkinsonism and one patient had left foot dystonia. Conclusion: This is the observational study, in which we have reported the wide spectrum of movement disorders associated with infectious agents prevalent in our region. This showed tremor and dystonia are the two most prevalent movement disorders observed in tubercular meningoencephalitis patients.

Abstract ID: 612

Clinical Profile and Outcome Predictors of Seronegative Autoimmune encephalitis – An Observational Study from a Tertiary Care Centre

R. Amod, Ashalatha Radhakrishnan

SCTIMST, Thiruvananthapuram, Kerala, India

E-mail: amodracl@gmail.com

Background and Aim: Seronegative autoimmune encephalitis is an immune-mediated syndrome, with sub-acute to chronic presentations with varied presentation and negative antibody testing.Aim is to describe clinical characteristics, imaging, EEG profile and outcome among seronegative AIE. Methodology: Hospital based retrospective prospective observational study • Study period -2012-2022.Included all seronegative autoimmune patients in the Prospective AIE registry with minimum follow-up of 1 year and adequate data.Excluded all other causes of AIE/encephalopathy including intracellular neuronal antigen related AIE (paraneoplastic).Outcomes were assessed using mRS. mRS <3 – good outcome,mRS 3 and >3 – bad outcome.Both 3 months (short term) and 1yr (long term) outcomes assessed. Results: 116 patients with adequate data studied.Mean age of presentation 24.5 yrs with equal gender. Most common presentation were seizures followed by behvaioural changes.MRI abnormality was seen in 68% (mostly flair HI temporal) and EEG abnormality (mostly focal or generalized slowing) in 82%. CSF was abnormal only in 14%. Most common first line treatment administered was pulse steroids.Secondline agent was required in 21%. Death occurred in 2.6%.Favourable outcome at 3months and 1year was in 86% and 88% respectively. 7.8% patient had relapse mostly seizures.Age less than 18 yrs, normal MRI at presentation,early initiation of treatment was associated with favourable longterm outcome and those patients with severe deficits at presentation has worst outcome. Discussion: This is the largest study on seronegative encephalitis from Indian published till date.As compared to previous study our study also showed similar clinical presentation. EEG, MRI abnormality was seen in almost 80% patient similar to previous studies but CSF abnormality was only in 14% (40-60%) may be due to previous treatment.Predictor of outcome were similar to previous studies. Limitation was of non randomised data and non uniform treatment. Conclusion: Seronegative AIE has excellent response to immunotherapy. Age less than 18 yrs, normal MRI at presentation, early initiation of treatment was associated with favourable longterm outcome and those patients with severe deficits at presentation has worst outcome.

Abstract ID: 613

IgG4- Related Disease Mimicking CNS Lymphoma: A Case Report

Sofia Singh, Aastha Kapila, Vivek Lal

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

E-mail: rajasingh0888@gmail.com

Background and Aim: IgG4-related disease (IgG4-RD) is a newly identified inflammatory autoimmune disease marked by IgG4+ plasma cell infiltration and systemic fibrosis. IgG4-RD is known to cause inflammatory pseudotumors in numerous visceral organs. An inflammatory pseudotumor has tumor-like morphological characteristics. Aim: To differentiate IgG4-related disease (IgG4-RD) from CNS Lymphoma on histological evidence. Methodology: We report a case of IgG4-related brain pseudotumor (IgG4-BP) in a 77-year-old woman, mimicking central nervous system lymphoma. She had a history of cataracts and presented to the outpatient department of Neurology with complaints of a progressive decrease of vision in a bilateral eye. Detailed ophthalmological, neurological, and radiological examinations were done. Consequently, she underwent a biopsy of the sellar region of brain. Results: Patient was PL negative in left eye and 6/60 in right eye. Magnetic resonance imaging (MRI) showed lesion of altered signals in the supra sellar and para sellar areas. FDG-PET showed FDG Avid mass in parasellar regions. Additionally, malignant cytology in the CSF showed lymphocytic pleocytosis. CSF IL-10 levels were raised. Based on these findings, we suspected CNS lymphoma and performed partial resection of the brain lesion. Pathological examination revealed prominent lymphocytic infiltration associated with plasma cell infiltration. Most of the plasma cells were immunoreactive for IgG4. Serum IgG4 levels were raised (1849 mg/L). The patient’s condition improved after surgical resection and high-dose intravenous methylprednisolone administration, followed by oral prednisolone therapy. Discussion: In our situation, it was difficult to identify IgG4-BP because the histological findings of the tissues collected during the original biopsy were summarised as nonspecific inflammation, and findings at presentation were comparable to those of CNS lymphoma. Conclusion: IgG4-related disease (IgG4-RD) could have several features that mimic CNS lymphoma on brain imaging, CSF analysis, and FDG- PET. Thus, a better understanding of the characteristics of both (IgG4-RD) and CNS lymphoma may improve the clinical outcomes of these diseases.

Abstract ID: 614

Clinicoradiological Spectrum of Primary CNS Vasculitis/Primary Angitis of CNS (PACNS): A Case Series

Bhaswar Bhattacharya, Kuntal Biswas, Soumozit Banerjee, Biman Ray

Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

E-mail: bhaswarbambi@gmail.com

Background and Aim: PACNS is a rare inflammatory disorder affecting the CNS with a heterogeneous presentation. In these cases, while brain biopsy remains the gold standard for diagnosis, prompt recognition of PACNS based on the clinicoradiological/ angiographic profile is necessary. We present 3 atypical cases of probable PACNS, diagnosed by clinical, laboratory and radiological data, in our series. Methodology: NA. Results: A 43 year male, presented with subacute worsening hemicranial headache and paraparesis. MRI Brain revealed left periventricular/subcortical and right thalamic T2/FLAIR hyperintensities, with cortical microbleeds, and short segment myelitis. MRA was normal, but DSA revealed classic beaded appearance. He was diagnosed as probable PACNS and improved on immunosuppression. A 47 year male presented with recurrent seizures and cognitive decline for 7 years and progressive spastic paraparesis for 4 years. MRI revealed an old right frontal haematoma, bilateral periventricular T2/FLAIR hyperintensities, diffuse microbleeds and vessel wall MRI showed multiple areas of concentric enhancement of bilateral MCA with dorsal myelitis. DSA showed characteristic picture of PACNS. He is currently on treatment. A 23 year old male presented with hemicranial headache for 5 years with acute right sided 3rd/6th cranial nerve palsy. MRI revealed multiple haemorrhagic white matter hyperintensities in right pons ,bilateral thalamus and periventricular areas. Vessel wall MRI showed concentric enhancement of bilateral ICA and DSA was suggestive of PACNS. Discussion: These cases highlight the importance of looking out for suggestive imaging findings with underscoring the role of concordant vessel wall MRI and angiographic findings in suspected cases of PACNS as virtual biopsy , where histopathological diagnosis is not rapidly available to institute prompt therapy. Further, isolated myelitis can be a sole presentation of PACNS, and the spinal cord was seen to be involved in our series as well. Conclusion: Clinicoradiological/angiographic studies of suspected PACNS patients is paramount for prompt diagnosis.

Abstract ID: 615

A Rare Case of CNS Vasculitis Presenting as Wemino

Maheshpandian Mariappan, Ramu Sekar, K. Murugan

DM Neuro Pursuing, Other

E-mail: dr.mahesh.neurologist@gmail.com

Background and Aim: Wall-eyed monocular internuclear ophthalmoplegia (WEMINO) is a rare variant of internuclear ophthalmoplegia (INO), consisting of unilateral INO and ipsilateral exotropia. This distinctive syndrome is probably associated with damage to the medial longitudinal fasciculus. Patients with classic INO have parallel visual axes in primary gaze. WEMINO is a rare variant of INO, consisting of unilateral INO and ipsilateral exotropia in primary gaze. Methodology: CASE REPORT: A 21 years female presented with complaints of double vision and abnormal position of eyes for one week. Patient had an acute febrile illness associated with headache 10 days prior to this symptom onset. On examination patient had exotropia of right eye, with adduction paresis of right eye and abducting nystagmus of the left eye with deficiency of convergence. Results: Investigation showed elevated ESR (85mm at 1 hour) and CRP (18), CSF study revealed elevated protein of 85mg/dl and 10 cells (8 lymphocytes and 2 Neutrophils), CSF TB PCR, was negative. Her ANA IF was Positive and C-ANCA, P-ANCA were negative. MRI revealed multiple infarcts in the cortical and subcortical areas including pontine tegmentum. Discussion: As this patient had multiple infarcts and ANA-IF positivity, patient was started on steroids. On regular follow up patient’s signs and symptoms improved. Conclusion: There have been only five other WEMINO patients reported over the past 25 years. The aetiology was acute ischemic stroke in three patients located exclusively in the pontine tegmentum affecting unilateral MLF, one involving midbrain tegmentum and neurocysticercosis in one patient. In our case the aetiology was Vasculitis and patient did respond to therapy well which makes our case interesting.

Abstract ID: 616

A Case of Fluroquinolone Triggered MUSK Antibody Postive Myasthenia Gravis: A Case Report

K. Mugundhan, Tino Baby

DM Neuro Completed, Other

E-mail: mugundhan69@gmail.com

Background and Aim: Muscle-specific tyrosine kinase (MuSK)myasthenia gravis (MG) is a severe, subtype of MG with different pathogenesis, and atypical clinical features.Such patients may suffer a worsening of symptoms upon exposure to a variety of medications. Fluoroquinolones of any generation may interfere with neuromuscular transmission and should be avoided in patients with MG. Methodology: A 24 year old female , known case of migraine ,presented with low grade fever and headache for 1 month ,for which csf analysis was done which showed mild CBNAAT positivity . She was then diagnosed as TB meningitis and was started on Levofloxacin based ATTregimen.After 1 month, patient developed double vision ,drooping of eyelids, unsteadiness while walking , dysphagia to solids and liquids and respiratory depression. Results: On examination, patient had bilateral ptosis and restricted eye movements.Ice pack test and neostigmine challenge test were positive. The patient’s routine laboratory investigations were normal.Anti Thyroglobulin and anti AchR were normal .MUSK antibody came as highly positive.CT chest was normal .RNS showed more than 10% decremental response in facial and limb muscles A working diagnosis of MUSK positive MYASTHENIA Gravis with impending crisis was made and was started on iv methylprednisolone 1 g for 5 days ,IVIG at a dose of 2gm/kg for 5 days and oral pyridostigmine. Patient did not show any improvement, so she was was given iv rituximab 1gm and patient was started on MMF .Patient didn’t show much response,hence she underwent plasma exchange and her symptoms improved dramatically and she was continued on MMF. Discussion: MuSK-MG is a subtype of MG with atypical clinical presentations causing a delay in diagnosis. MuSK-Ab testing confirms the diagnosis. Response to pyridostigmine is often poor from that expected in MG patients. Conclusion: Among all immunotherapies, plasma exchange is the cornerstone of treatment for MuSK-MG. Fluoroquinolone exposure may result in potentially life-threatening myasthenia gravis in patients with underlying disease.

Abstract ID: 617

NMOSD Presenting as Bulbar Symptoms and Paroxysms of Facial Pain with Preceding Attacks of Area Postrema Syndrome

Aubin Varghese

DM Neuro Pursuing, Other

E-mail: aubinmathew@gmail.com

Background and Aim: Unreported presentation of NMOSD as paroxysms of facial pain with bulbar symptoms. Reinforce the intractable nausea, vomiting and hiccups (INVH) symptoms of area postrema syndrome (APS) as harbinger of subsequent disabling attacks. Methodology: 32-year-old lady presented with 20-day history of bulbar symptoms, blurring of vision and paroxysms of lancinating facial pain above the angle of mouth, provoked by touch stimulus in the facial region. Prior to current symptoms, she had 2 attacks of INVH, initially as an inaugural attack 3 months back, when she was misdiagnosed. Results: Clinically she had bilateral 9th, 10th, 11th & 12th cranial nerve involvement with mild pyramidal pattern weakness of all limbs. AQP-4 antibody was positive, MOG antibody was negative. Brain imaging showed demyelination in AP of medulla. Patient improved after pulse methylprednisolone of 1 gm per day for 5 days, followed by IVIg therapy. She was continued on oral prednisolone & Rituximab was given 2 months later. Discussion: APS occur as inaugural or non-inaugural, isolated or associated attacks in NMOSD. As inaugural symptom, it is often misdiagnosed. Significant delay from INVH onset to MRI, leads to delayed immunotherapy. INVH of APS is continuous and severe without any other specific characteristic. Presence of INVH is highly specific for AQP4-IgG seropositive NMOSD, thus the importance of clinical rather than MRI features of APS. Facial pain in our patient could be possibly explained by rostro-caudal lamination arrangement of spinal nucleus of 5th CN. Associated bulbar symptoms & lower CN involvement with mild long tract signs points to a cervico-medullary junction lesion. Conclusion: Clinically approach to INVH would be to rule out other etiologies in acute or subacute NVH, followed by AQP4-IgG antibody testing and brain imaging with gadolinium contrast. It is important to diagnose early and correctly to prevent disability of ON or TM.

Abstract ID: 618

MOG Antibody Associated Disease with Brainstem Ring Enhancing Lesion and Short Segment Myelitis

Alex Rebello, V. C. Soumya, Pavan Rudrabhatla, Ashalatha Radhakrishnan, Mohd. Asif, Jasneet Kaur Sodhi

DM Neuro Completed, Other

E-mail: dralexrebello@gmail.com

Background and Aim: Myelin Oligodendrocyte Glycoprotein antibody Associated disease (MOGAD) is a demyelinating disease of the central nervous system. It usually has longitudinally extensive myelitis. Methodology: We describe a case of seropositive MOGAD with ring enhancing lesion in midbrain and short segment myelitis with contrast enhancement. Results: A 37-year-old female presented with 1-week history of gait imbalance, vomiting, vertigo along with paresthesias in left upper and lower limbs and urge incontinence. On examination, she had UMN signs and impaired tandem walking. Fundus examination was normal. Her routine blood investigations and viral markers were normal. Contrast-enhanced MRI showed lesions in midbrain and left centrum semi-ovale with diffusion restriction and enhancement with well-defined T2 hyperintensity in right hemicord at D6 level with homogenous enhancement and tiny enhancing nodule at right hemicord C6-C7 level. CSF analysis was normal. Serum MOG antibody was strongly positive while NMO antibody was negative. CECT chest and abdomen was done and ruled out any neoplastic lesion. She was treated with steroids and rituximab. Her symptoms improved over 2 weeks after treatment initiation. Discussion: MOGAD myelitis is usually extensive and without contrast enhancement in majority of cases. Ring enhancing lesions are also rare in MOGAD.2,3 However, in neuromyelitis optica and multiple sclerosis, the active lesions can enhance in most of the cases. In our case, there was ring enhancing lesion in brainstem and short segment myelitis in spinal cord which is unusual in MOGAD. Conclusion: There can be atypical MRI features in MOGAD and antibody testing may aid in diagnosis.

Abstract ID: 619

Uncommon Manifestations of Mogad (Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease)

Viresh Nashte, Chinmay Kumbhar, Shripad Pujari, Rahul Kulkarni, Vishal Deshpande, Rushikesh Deshpande

Deenanath Mangeshkar Hospital and Research Center, Pune, Maharashtra, India

E-mail: viresh.nashte28@yahoo.com

Background and Aim: MOGAD (Myelin oligodendrocyte glycoprotein antibody-associate disease) is rare auto immune inflammatory disease , targeting protein that is present on oligodendrocyte ,myelin on brain , spinal cord and and optic nerve . Classical MRI features: Optic neuritis: Myelitis: Acute disseminated encephalomyelitis (ADEM): Brain stem encephalitic syndromes: AIM: To report uncommon clinical and MRI manifestation of MOGAD. Methodology: Retrospective clinic-radiological data, serological reports ,treatment and outcome of 3 patients with established diagnosis of MOGAD were collected. Results: None of our patients had classic features as discussed in background . all 3 Patients had uncommon manifestation, First presented with subacute episode of hemiparesis and seizure since at age of 12 yr, however unlike ADEM like lesion, MRI showed b/l symmetrical FPO white matter lesion as seen in leukodystrophy, MOG came positive. second patient presented with subacute right hemiparesis and seizure. His MRI showed cortical encephalitis with Lepto-meningeal enhancement which is another unusual finding. Third one presented with subacute right hemiparesis ,MRI however shown ,contrast enhancing lesion in left ganglio capsular region (never seen in MS also usual in MOGAD) and its is complete ring. Discussion: Apart from classic features, rarely leukodystrophic picture seen can be seen and give way is an episodic encephalopathy or focal deficit and contrast enhancement . MS and NMO never have cortical encephalitic like lesions and in contrast it is well seen finding in MOGAD, When unilateral, cortical FLAIR hyperintense lesion in MOGAD associated encephalitis with seizures is referred to as FLAMES. Complete ring enhancing lesion and also in ganglio-capsular region is very unusual finding in both MS and NMO and seen uncommonly even in MOGAD. Conclusion: Apart from classic clinico-radiological features ,neurologist should also be in look out for leukodystrophic, cortical encephalitis , ring enhancing lesion as unusual presentation of MOGAD.

Abstract ID: 620

Fulminant Guillain -Barre Syndrome in Ulcerative Colitis -A Double Whammy

S. Arunan, Elanthiraiyan Chelvakumar, R. Kalpana, S. Robert Wilson, K. Aruna Kumari

DM Neuro Completed, Other

E-mail: drarunan@yahoo.com

Background and Aim: Guillain Barre Syndrome (GBS) occurring in Ulcerative Colitis is extremely rare and has a high mortality .We present a patient who is a known case of Ulcerative Colitis who developed Fulminant GBS. Methodology: A 41 years old male, who is a known case of Ulcerative Colitis in remission on treatment with Sulfasalazine presented with acute onset of weakness of all four limbs (proximal> distal).It was preceded by paresthesias in both hands and feet.Clinical examination revealed flaccid areflexic quadriparesis with no cranial nerve involvement.Nerve Conduction Study showed axonal sensory motor polyradiculoneuropathy.CSF studies revealed albumino-cytologic dissociation.MRI whole spine and Brain with contrast was normal.A diagnosis of AMSAN variant of GBS was made. Results: He was initiated on IV Immunoglobulin.His limb weakness improved.5 days after the completion of IVIG he developed worsening weakness of the limbs.He was started on Pulse Methyl Prednisolone.Three days later he developed brainstem involvement in the form of bilateral ptosis,bifacial involvement and bulbar weakness.He was initiated on Plasma Exchange.He subsequently developed respiratory distress and autonomic instability.He was then intubated and ventilated.Inspite of the aggressive treatment he succumbed to the illness. Discussion: Apart from bowel disease Ulcerative Colitis is notorious for extraintestinal manifestations in 40% of cases.Neurological manifestions in Ulcerative Colitis are rare and occurence of GBS is even rarer.Extra intestinal manifestations in Ulcerative Colitis occur during the active phase of the disease.Paradoxically GBS occurs during the remission phase in Ulcerative Colitis.Immune dysregulation plays a keyrole in pathogenesis of GBS in Ulcerative Colitis. Other possible mechanisms include Ulcerative Colitis associated vasculitis,B12 deficiency secondary to malabsorbtion,Opportunistic infections like Campylobacter/EBV secondary to immunosuppression. Conclusion: We present this case to highlight the extremely rare occurence of GBS in Ulcerative Colitis.GBS occurring in Ulcerative Colitis is associated with poor prognosis and has a high mortality.

Abstract ID: 621

Tumefactive Demyelination or Tumor: A Diagnostic Conundrum

Surbhi Mahajan, Aastha Kapila, Sofia Singh, Vivek Lal, Chirag Ahuja, Vivek Lal

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

E-mail: mahajan_surbhi5@yahoo.com

Background and Aim: Demyelinating diseases, especially those presenting as large lesions, can be mistaken for a neoplasm (either spinal or cerebral), both clinically and radiologically, which can possess diagnostic challenge in regards to absolute diagnosis, and hence affect further treatment and prognosis. Here, we summarize the presenting features of 5 patients with pathology confirmed inflammatory demyelinating disease of the central nervous system which initially mimicked as tumoral lesions. Methodology: 5 patients included in our study, had clinical presentation mimicking neoplasm and biopsy confirming the diagnosis of Demyelination. Routine detailed examination and Laboratory investigations were performed in all cases. Work up to rule out Demyelinating etiology was sent, including Serum NMO and MOG levels. Patients also underwent CSF work up including OCBs and VEP/BAER studies. Pre and post operative scans (computed tomography (CT) or magnetic resonance imaging (MRI) was examined. Results: Median age at onset was 29 years, with a female to male ratio of 1.5: 1. 4 patients presented with spinal cord lesions whereas 1 patient had lesion involving cortical grey matter and subcortical white matter on their pre-biopsy magnetic resonance imaging (MRIs). Clinical course prior to biopsy was a first neurological event in all cases. After the diagnosis, patients were started on steroids and/or immunomodulation, and showed significant recovery, as determined by EDSS scale. Discussion: In our study, we reported majority of the cases with Spinal cord involvement, which is in contrast to relatively frequently reported tumefactive lesions in the brain. After treatment, lesion regression, as evidenced by post biopsy imaging scans, was associated with improved EDSS. Conclusion: Comprehensive information from the patient’s history, imaging and response to steroid and/or immunomodulatory therapy in addition to histopathology, provide clues for the final diagnosis in tumefactive demyelination. Hence, it is mandatory to consider demyelinating etiology as one of the differential diagnosis of tumour-like lesions.

Abstract ID: 622

Parry-Romberg Syndrome - still Progressing over 20 Years of Disease Course

Rajesh Singh, Ashem Thoibisana, Biswamohan Mishra, Arunmozhimaran Elavarasi, Deepti Vibha, Shailesh Gaikwad, Manjari Tripathi

All India Institute of Medical Sciences, New Delhi, India

E-mail: drrajeshrims679@gmail.com

Background and Aim: Parry-Romberg syndrome (PRS) presents with atrophy of one half of face involving skin, soft tissue, muscle and bone. It usually progresses slowly over 2-20 years and finally stabilizes. Neuropsychiatric abnormalities are subtle, seen in in about 10% of patients. Methodology: We describe the case of a young female who had progressive brain atrophy over 20 years of disease course and presented with focal seizures and recent onset progressive cognitive impairment. Results: 39-year lady presented with atrophy of left face for 20 years, seizure for last 4 years and forgetfulness for last 3 months. 20 years back, she noted atrophy in the left maxillary area which gradually involved other parts of face including periorbital area and forehead within 2 to 3 years and was static since then. Higher mental function evaluation revealed predominant impairment of frontal and temporal lobe functions. Brain Magnetic Resonance Imaging (MRI) showed progressive left cerebral hemiatrophy in the form of prominent sulcal spaces of left cerebral hemisphere and ex vacuo dilation of left lateral ventricle. Patient was given steroids and was started on Methotrexate 7.5 mg weekly for long term immunosuppression. Discussion: Seizures, headache, facial pain, limb weakness, oculomotor dysfunction and neuropsychiatric manifestations are common symptoms in PRS. Neuropsychiatric abnormalities are subtle, seen in in about 10% of patients. Our patient also presented with predominant memory impairment and naming difficulty without any psychiatric disturbances. Our case was unique as brain atrophy was progressing over 20 years of disease course and she had developed recent onset progressive cognitive impairment. Conclusion: Progressive brain atrophy in PRS can manifest with cognitive Impairment. Detailed neurological investigation with serial brain imaging should be considered in PRS to look for subtle cognitive impairment and progressive brain atrophy and timely intervention with immunosuppression to halt the disease progression.

Abstract ID: 623

Eyes: Gateway to the Brain

Yuvraj Lahre, Sanjeev Bhoi, Menka Jha, Suprava Naik, Priyanka Samal

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

E-mail: yuvi.10170@gmail.com

Background and Aim: CNS vasculitis is well known cause of recurrent strokes in young patients. Both primary and secondary vasculitis have similar CNS presentations. Routine eye examination in such patient are always helpful in such patients. Here we report a rare case of secondary CNS vasculitis who presented with recurrent strokes and chorioretinitis. Methodology: 32 year old, chronic alcoholic and smoker male patient presented to us with history of chronic headache, and recurrent neurological deficits. He had acute onset cerebellar ataxia 3 year ago then right hemiparesis 10 month back, and forgetfulness with abnormal behavior 8 months back. All deficit improved spontaneously with mild residual deficit. Detailed evaluation of patient was done. Results: MRI brain showed multiple old lesions, old bleed in rt cerebellum and lateral temporal area and old infarct in left gangliocapsular area. Fundus examination showed bilateral multifocal choroiditis, patches involving central and peripheral fundus. DSA and brain biopsy were suggestive of chronic vasculitis. He was treated with injection cyclophosphamide and all symptoms improved. Discussion: CNS vasculitis with choroiditis is rare occurrence. Common causes of secondary CNS vasculitis like SLE, RA do not have chorioretinitis. Diseases like posterior multifocal placoid pigment epitheliopathy, Vogt-Koyanagi-Harada disease can present with CNS vasculitis and chorioretinitis. VKH patient generally have diminution of vision which was not present in our patient. FFA and OCT were done and were showing features of multifocal placoid pigment epitheliopathy. Ischaemic strokes have been reported with this disease, our patient has both ischaemic and haemorrhagic strokes. Conclusion: Possibility of posterior multifocal placoid pigment epitheliopathy, Vogt-Koyanagi-Harada disease should be taken into consideration in young patients with recurrent stroke. Eye examination is crucial in diagnosing CNS vasculitis.

Abstract ID: 624

Chronic Inflammatory Demyelinating Polyneuropathy : An Uncommon Association with Lupus Nephritis

K. Priya

DNB Neuro Pursuing, Other

E-mail: drpriyachandran@gmail.com

Background and Aim: Review of literature disclosed that glomerulonephritis seems to be not uncommon in AIDP, but association with CIDP appears to be rare. Also CIDP is an uncommon manifestation of SLE. We here in report a case of glomerulonephritis secondary to SLE diagnosed in the course of evaluation of sub acute demyelinating polyneuropathy who later progressed to CIDP. Methodology: 33 year old gentleman presented with 1 month history of progressive paraesthesias of fingers and toes bilaterally, followed by gait ataxia and weakness of all four limbs 1week prior to admission. He also gave a history of hematuria and frothy urine for last 3 months with intermittent fever, malaise, rash and significant weight loss of around 10 kgs. On examination, motor power- grade 3/5 in proximal muscles, 2/5 in distal muscles in all four limbs with poor hand and toe grip bilaterally. DTRs: absent in all limbs. Romberg’s was positive. Gait was ataxic. Investigations revealed pancytopenia with increased triglycerides, proteinuria and ANA positivity , ESR: 75, CRP:15. Serum calcium was low. CSF study showed albuminocytological dissociation. NCS showed severe demyelinating polyradiculopathy. Nephrology opinion was sought and renal biopsy was done which showed class III lupus nephritis. Viral markers and VDRL were negative. Serum C3 was reduced. ANCA with GBM was negative. Patient was treated for subacute inflammatory demyelinating neuropathy secondary to SLE with high dose pulse methyl prednisolone for 5 days along with IV Cyclophosphamide . Results: Though the patient initially showed some improvement in his hand grip and muscle power he later progressed to CIDP. He was then started on monthly maintenance dose of IVIG (2gm/kg) after which the patient showed significant improvement. Discussion: Compared with AIDP, the association of CIDP and GN is rarely reported. Also CIDP is an uncommon manifestation of SLE . CIDP responds to high dose glucocorticoids, but not all patients improve on this treatment. Our patient showed only modest improvement with steroids and improved only after IVIG treatment. Conclusion: Though early diagnosis of CIDP can predict a good response to steroids, some patients may not respond to steroids. IVIG, plasmapheresis or immunosuppressive therapy should be considered if there is no response to steroids.

Abstract ID: 625

Acute Bulbar Palsy Plus Syndrome: A New Entity or an Overlap Guillain- Barré Syndrome

Bharat Sahu, Swapan Gupta, Babu Challepalle

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi, India

E-mail: bksahu.sahu0@gmail.com

Background and Aim: Acute bulbar palsy plus (ABPp) syndrome is characterized by acute bulbar palsy combined with other cranial symptoms or ataxia without limb and neck weakness. We aim to investigate characteristics of 4 patients with ABPp syndrome and analyze its nosological position within the GBS spectrum. Methodology: A total of 4 patients were included in our study. All the patients had preceding infection. Out of 4, 3 had URI and 1 had history of acute gastroenteritis. All had presented with acute bulbar palsy. The other main accompanying symptoms in combination were ataxia in 2 patients, ophthalmoplegia in 1 patient, altered sensorium in 1 patient. All the tested patients were seronegative for antiganglioside antibodies with normal CSF composition. On electrophysiological assessment, one patient had normal study, 2 had bilateral decreased sensory nerve action potentials (SNAPs) with sural sparing and one had sensory motor axonal neuropathy with sural sparing beyond cranial nerves. Results: All were given IVIG with additional steroids in 2 patients. The outcome was favourable in 2 patients. They became asymptomatic at 2 weeks; 1 patient was ambulatory with minimal support but delayed at 10 weeks whereas there was death of 1 patient due to respiratory failure at 10 weeks.Discussion: The clinical manifestations do seem to appear a combination of subtypes of GBS but with variable electrophysiological and serological parameters, raising the possibility of a new subtype with unique antibody profile. Conclusion: ABPp syndrome is more likely to be a different subtype of GBS than merely overlap of distinct subtypes and should instigate further research in the diagnostic algorithm of GBS.

Abstract ID: 626

Unusual Case of Autoimmunity: Multiple Sclerosis with Myasthenia Gravis

Vibhor Upadhyay, Anoop Thacker, Ritwiz Bihari, Sudhakar Pandey, Pradeep Gupta, Rakesh Mishra

All India Institute of Medical Sciences, New Delhi, India

E-mail: docvibhor@gmail.com

Background and Aim: Reporting an unusual case of 51-year-old man who was k/c MS and 16 years later developed MG. Methodology: we report an unusual case of autoimmunity in which patieny developed MS and later on remission developed MG. Results: Although MS is primarily mediated by T lymphocytes and MG primarily involves the destruction of the neuromuscular junction by antibodies, there are evidences that support both cell-mediated and humoral immunity are involved in the pathogenesis of both the diseases. Discussion: We report an unusual case of 51-year-old man who was k/c MS and 16 years later developed MG. He had previously been diagnosed with hypothyroidism and relapsing-remitting multiple sclerosis (RRMS), which was discovered in 2006 (at the age of 34) after the initial presentation of internuclear ophthalmoplegia (INO) and optic neuritis. He was on DMF for next 6 yrs. i.e. upto 2012 after which he did not have any other recurrences and he stopped the treatment own his own. Now he presented to us with complains of blurred vision, diplopia, and drooping of both eyelids.He did not have any generalised weakness or walking difficulty. He was thought initially to be exacerbation of MS (INO) and his MRI brain with spine with contrast was done. As there was no active lesions in MRI brain, comprehensive labs tests were submitted including panels for CK, TSH, serum NMO antibodies, AChR and anti-Musk antibodies, paraneoplastic, and autoimmune diseases. He was started on pulse IV methylprednisolone after which patient had significant improvement so potential diagnosis of myasthenia gravis was kept. Later his AChR Ab also came positive and he was started on MMF and oral steroids. patient after 2 months follow up is doing good. Conclusion: This case highlights the rare co-occurrence of MS and MG and underscores the importance of careful evaluation of patients with overlapping symptoms esp ocular and bulbar.

Abstract ID: 627

Handy Hints in Autoimmune Encephalitis

S. Mohinish, C.leema Pauline, Neeraj Elango, Jered Livingston

Madras Medical College, Chennai, Tamil Nadu

E-mail: drmohinish92@gmail.com

Background and Aim: Introduction: Autoimmune encephalitis (AIE) is closely associated with antibodies against neuronal cell-surface proteins, receptors, and ion channels. It has broad spectrum of symptoms including alteration of behavior, psychosis, catatonic, insomnia, memory deficit, seizures, abnormal movements, autonomic dysfunction. These disorders are highly responsive to immunomodulatory therapies. Since early initiation of treatment has been shown to improve outcomes, it is important that these symptoms are promptly recognized. Methodology: Case Report: This comprehensive case report discusses about 2 cases of AIE with unique hand manifestations. Results: Case 1: A 9 year old child presented with acute onset of gait abnormality and dystonic posturing of right upper limb in form of pronation and flexion at wrist, flexion at metacarpophalangeal (MCP) joints, and extension at interphalangeal (IP) joints , with progressive clawing noted. This presentation is consistent with “thalamic hand.” CSF panel revealed anti- NMDA receptor antibody positivity. Treated with immunotherapy and is on follow-up. Discussion: Case 2: A 5 year old child presented with sudden onset of left focal seizures initially followed by hemi-asomatognosia and motor neglect involving left hand leading to secondary parietal alien hand phenomenon. Manifestation as purposeless and restless hand. CSF panel revealed anti- NMDA receptor antibody positivity. Immunotherapy was initiated irrespective of which gradual severe dystonia emerged in his left hand with spreading of fingers in a “star fish” pattern. Currently on second line therapy with Injection Rituximab. Conclusion: Conclusion: Dystonic hand posturing or hemi-asomatognosia, although a rare presentation, should always arouse a suspicion of AIE especially when presenting along with focal seizures, oro-facial dyskinesias, and/ or behaviour changes.

Abstract ID: 628

A Curious Case of IIH and GBS

Mohit Anand, Abhinav Nair, Sumit Singh, Manish Mahajan, Archana Sharma, Rimpi Rajrana, Shubhi Katyal

All India Institute of Medical Sciences, New Delhi, India

E-mail: mohitanand42@gmail.com

Background and Aim: To evaluate an atypical case of IIH with GBS. Methodology: A 29 year old, obese female complained of headaches on and off without any nausea or vomiting. She also complains of tingling sensation of B/L toes that spread to B/L feet. History of Occasional black spots in front of her eyes was also present. Fundus showed b/l papilloedema. MRI was suggestive of empty sella and tortous optic nerves. A possibility of IIH was kept and lumbar puncture was done. Results: CSF opening pressure was 54 cm of water CSF ; glucose : 52.00 mg/dl , lactate : 11.50 mg/dl, protein : 88.00 mg/dl , cell count : 10 cells/cumm (Neutrophils - 05% Lymphocytes - 95%). Rest of CSF investigations were normal. She was started on mannitol and acetazolamide. 2 days later she developed numbness and paresthesia of b/l lower limb with proximal and distal weakness of b/l lower limb. Nerve conduction studies were suggestive of demyelinating polyneuropathy of all 4 limbs. Discussion: In view of predominant sensory symptoms and raised ICT, workup for GBS mimics was sent with vasculitic profile and serum electrophoresis. Serum electrophoresis showed M spike of gamma globulin. PET was suggestive of Metabolically active destructive lytic lesion with associated intraosseous soft tissue in left pubis and left inferior ischio-pubic ramus. Bone marrow aspiration : Cellular marrow shows trilineage haematopoiesis and 3% plasma cells. A diagnosis of MGUS associated paraproteinemic neuropathy was kept and she was started on IVIG followed by bortezomib. She had complete reversal of all her symptoms and is doing well at 2 months followup. Conclusion: Atypical presentation of GBS with IIH should be considered a red flag. Although many case reports exist for association of raised ICT with GBS, a predominant sensory presentation followed by thourough workup helped us clinch the diagnosis in this case.

Abstract ID: 629

A Febrile Illness Causing Paraplegia and Pneumonia

Jenu Santhosh

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: jenusanthosh@gmail.com

Background and Aim: Spinal epidural abscesss is a loculated purulent collection that may cause cord injury via compression or vascular compromise. If untreated, it may produce sensory symptoms, weakness and ultimately paralysis. It is frequently underdiagnosed because of the nonspecific signs and symptoms. Early detection will have improved outcomes. Methodology: 60 year old female Mrs V presented with complaints of difficulty in using both lower limbs of 20 days duration in Feb 2023. The illness started with a fever following which she got admitted to a local hospital from where IV fluids and medications were administered. Subsequently, the patient developed swelling, and pain warmth at the IV cannula insertion site for which drainage was done. The Patient developed difficulty in using both lower limbs associated with bladder disturbances in the form of retention of urine. On examination, the Patient was conscious, oriented , afebrile with a GCS- 15/15 . No pallor, not icteric, mild pedal edema No lymphadenopathy. Cranial nerves examination was normal. Spinomotor system examination showed decreased power in both lower limbs with hypotonia and absent reflexes. Sensory examination revealed diminished touch, pain sensation below the T10 level. Spinal vibration was decreased below the T8 level. No neck stiffness was noted. Results: Results: MRI spine taken showed T2 signal intensity in the cord from D2 to D9 levels with collection noted posteriorly abutting the cord from D7 to D10. CT chest showed evidence of septic emboli. Blood culture yielded growth of Staphylococcus Aureus. Discussion: This is a case of septic emboli causing spinal epidural abscess with cord compression and paraplegia and a septic pulmonary emboli, the source of infection being the peripheral IV cannula insertion site in the hand. Conclusion: Spinal epidural abscess is a misdiagnosed condition. Early identification and timely intervention will result in improved outcome.

Abstract ID: 630

A Case Report of Neuromyotonia: LGI1 and CASPR2 Antibody Positive Isaac’s Syndrome

Vignesh Kumar, Marian Vijay, R. Lakshminarasimhan, R. Lakshminarasimhan, Marian Vijay

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: vigneshkumar.r@gmail.com

Background and Aim: We report a case of Issac’s syndrome, which is characterised by spontaneous discharges from motor nerve fibres and dysautonomia. Methodology: A 19 year old boy developed debilitating pain and muscle twitching over both thighs, gluteal regions and calves following a minor trauma. Patient had significant weight loss, hyperhydrosis and developed burning sensation over both legs over a period of two months. There was no significant family history of similar complaints. Examination revealed myokymia involving bilateral calves and deltoids with weakness of lower limbs (proximal>distal). Patient had postural orthostatic tachycardia. Sensory, extrapyramidal, and cerebellar examination were normal. Results: Routine blood investigations were normal. MRI of muscles was suggestive of focal myositis in bilateral gluteal region. Muscle biopsy showed no diagnostic pathology. Nerve conduction studies were normal. EMG showed continuous muscle fibre activity in the form of recurrent bursts. Autoimmune panel was strongly positive for CASPR-2 and LGl1. Discussion: Issac’s syndrome or Neuromyotonia, cramp-fasciculation syndrome (CFS), and Morvan’s syndrome are the most common primary peripheral nerve hyperexcitability syndromes. They generate extensive symptoms and signs without the presence of an obvious peripheral nerve illness. Most PNH syndromes are immunological while some are hereditary. The main symptoms of neuromyotonia and CFS are muscle twitching and stiffness, which differ only in intensity and the presence of dysautonomia in the former. Several patients exhibit cramps, pseudomyotonia, hyperhidrosis, other autonomic abnormalities, and minor positive sensory experiences in Issac’s syndrome. Morvan’s syndrome is characterised by central nervous system involvement including encephalopathy, insomnia, amnesia and confusion. Conclusion: The diagnosis of PNH syndromes necessitates a high level of suspicion as well as familiarity with the symptoms associated with each PNH condition. The majority of patients have antibodies against voltage-gated potassium channel complexes. Research into optimal treatment and understanding the complete aetiology of PNH disorders are needed.

Abstract ID: 631

Parry-rhomberg Syndrome and Fascicular Trigeminal Neuropathy in a Patient of Morphea: A Rare Case Report

Sandeep Joshi, Y. Muralidhar, P. L. Chandravathi, Subhendu Parida, K. Shyam, Laitha Pidaparthi, G. Abhinay, M. K. Jagarlamudi

DNB Neuro Pursuing, Other

E-mail: sandeepjoshi.brn@gmail.com

Background and Aim: Introduction: Parry-Romberg syndrome (PRS), characterized by progressive facial hemi-atrophy, is a rare neurocutaneous disorder of unknown origin. It is associated with neurological disorders such as trigeminal neuralgia, migraine and epilepsy. We present a case of PRS with neurological involvement in a patient diagnosed with Morphea. Methodology: Case Report: 32 year-old-female presented to neurology out-patient for left-sided facial twitching and neuralgic pain over the jaw for six months. She developed left-sided cheek tightness and nodular thickening of the skin over the cheek and jaw two years ago when she consulted a dermatologist and was diagnosed with Morphea (skin biopsy). She has been on hydroxychloroquine and methotrexate since then. There was no history of seizures or cognitive deficits. The general examination showed left hemi-facial atrophy. Neurological examination showed wasting and fasciculation in left temporalis and masseter muscles. EMG of both muscles showed fasciculation. MR brain and orbits revealed thickened left mandibular nerve at the foramen ovale and inflammation of left-sided recti muscles. Anti-Scl 70 was negative. She was treated with oral prednisolone, methotrexate, intralesional tacrolimus and carbamazepine with partial relief. Result: -. Discussion: -. Conclusion: Trigeminal neuropathy is a common manifestation of PRS and limited scleroderma. Awareness of this association can help in early diagnosis and management.

Abstract ID: 632

Unmasking the Dementia Masquerader - Temporal Lobe Meningioma

J. N. Brijesh, M. Jawahar, R. Lakshmi Narasimhan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: jnbrijeshremin711@gmail.com

Background and Aim: Secondary Dementia can present as a slow progression of clinical symptoms that mimic Alzheimer Dementia. Here we report a 70 year old female with slowly progressive memory impairment caused by an intracranial Benign tumor -Meningioma strategically located in Left temporal lobe. Methodology: This is a elderly female with Diabetes for 10 years who presented with memory impairment for 2 years in the form of difficulty in remembering what was told to her , misplacing and repeated searching of commonly used household objects , which progressed to way finding difficulty , cooking. She did not have any headache , seizures ,vomiting ,trauma or fall , exposure to drugs/toxins delusions and hallucinations . On examination, patient was conscious, oriented and attentive .MOCA score was 10/30 indicating apparant cognitive decline. Language domain was normal.Frontal and executive functioning was normal. There was severe impairment in working, recent , remote and paired associative memory. There was visuospatial disorientation . Examination of cranial nerves , cerebellum, extrapyramidal , autonomic and spino motor system was normal. Results: Routine blood investigations , Thyroid , vitamin B12 were normal . CT Brain normal. MRI Brain with contrast showed well defined extraaxial enhancing lesion with dural tail noted in the left temporal region suggestive of meningioma without any obvious hippocampal or global atrophy. Discussion: Brain tumours can cause Dementia due to mass effect on adjacent structures . Meningiomas are most common Benign intracranial tumours. The risk of developing a Meningioma increases with age , female sex and exposure to radiation. Since they are slow growing , usually asymptomatic and the presenting signs and symptoms depends on the size and site of the tumour. Conclusion: Various factors can lead to dementia and exhaustive workup is needed for early identification and treatment of the reversible ones. In our report we describe a slow growing Meningioma leading to dementia which is potentially treatable by surgical treatment .

Abstract ID: 633

A Bewildering Case of Hypertension: Neurogenic Supine Hypertension Due to Baroreceptor Failure

Eluka Reddy

DM Neuro Pursuing, Other

E-mail: e.mounikareddy20@gmail.com

Background and Aim: To describe a perplexing case of uncontrolled hypertension in a young male presenting with acute hemiplegia. Methodology: A descriptive case study of rare cause of uncontrolled hypertension in a patient diagnosed with left basal ganglion bleed. Results: A 45-year-old man with a history of hypertension presented with a dense right-sided hemiplegia. Brain imaging revealed bleeding in the left basal ganglia extending to the thalamus. Despite being on three antihypertensive medications, his blood pressure remained persistently high during hospitalization (ranging from 180-200/100-130 mmHg). Labetalol infusion was initiated but did not effectively control his blood pressure even after five days of titration. Investigations for renal artery stenosis, arterial dissection, and pheochromocytoma were negative. Blood epinephrine and catecholamine levels were normal. Fluctuations in blood pressure recordings were observed, leading to the consideration of baroreceptor failure. The patient’s blood pressure dropped from 180/110 to 110/80 mmHg when sitting up, suggesting neurogenic supine hypertension. The diagnosis of neurogenic supine hypertension was confirmed, possibly due to baroreceptor failure. Management involved using short-acting antihypertensive medication at night, elevating the head during sleep, and having the patient sit in an inclined chair during the daytime. Discussion: Neurogenic supine hypertension (nSH) is seen in most patients with neurogenic orthostatic hypotension (n0H). nSH is usually asymptomatic or may present with nonspecific symptoms. Rarely present with hypertensive emergencies. We postulates that baroreceptor failure might have led to haemorrhagic stroke in our case while in sleep. Conclusion: In uncontrolled hypertension one need to monitor supine, sitting and standing blood pressure to arrive at a proper diagnosis. Diagnosis of nSH secondary to baroreceptor failure prevented hospital stay and multiple antihypertensive therapy in our case.

Abstract ID: 634

A Case of Adrenomyeloleukodystrophy

P. Prasanth, M. R. Manivannan

DM Neuro Pursuing, Other

E-mail: pkumartheboss@gmail.com

Background and Aim: X linked adrenoleukodystrophy (X-ALD) is an inherited disorder of peroxisomal metabolism, biochemically characterised by accumulation of saturated very long chain fatty acids. Accumulation of these fatty acids is associated with cerebral demyelination, peripheral nerve abnormalities, and adrenocortical and testicular insufficiency.The various phenotypes have been recognized to occur within the same pedigree. The cause of the phenotypic variability is unknown. The first case of cerebral ALD was published by Haberfeld and Spieler in 1910. Methodology: A girl presented to us at eleven years of age with a history of nonconsanguious marriage,2nd order birth with normalbirth history and normal milestones till 5 years of age and positive family history presented with spastic weakness of both lower limbs in the term of difficulty in standing, climbing up & down staircase, associated with tightness in both legs (distal>proximal), buckling of knee present, difficulty in gripping sandal.Neurological examination showed bilateral extensor , ankle clonus-present, increased tone, accelerated lumbar lordosis-present. Younger brother and paternal aunt are affected with the same condition . Results: All routine laboratory investigations produced normal results. MRI brain report showed symmetric T2/Flair hyperintense noted in bilateral periventricular white matter and posterior limb of internal capsule possibility of metachromatic leukodystrophy.Genetic analysis revealed homozygous variant of uncertain significance. Discussion: Accurate information about the relative frequency of the various forms of ALD is needed for counselling and for the selection and evaluation of therapeutic approaches. Conclusion: Risk-benefit analysis of bone marrow transplantation requires knowledge of the phenotype frequencies. Promising results from an ongoing gene-therapy trial and recent antioxidant therapy investigations and the increase in newborn screening programs provide hope for patients with ALD.

Abstract ID: 635

A Case of Anti-Jo-1 Myositis with Inclusion Body Myositis Phenotype

Sivaroja Yellaturi, P. R. Sowmini, K. Mugundhan

DM Neuro Pursuing, Other

E-mail: rojayellaturi@gmail.com

Background and Aim: Idiopathic inflammatory myopathies are a heterogenous group of autoimmune diseases that affect various organ systems including lungs, joints, muscles and skin. Anti-Jo-1 antibody is one of the most common myositis specific antibody described in this group and it is directed against the antihistidyl-tRNA synthetase that catalyses the binding of histidine to tRNA during protein synthesis. This antibody is associated with a characteristic phenotype called anti-synthetase syndrome (ASS) which include interstitial lung disease (ILD), arthritis, myositis, fever, mechanic’s hands and Raynaud’s phenomenon. Here, we report the case of a patient with anti-Jo-1 antibody myositis who came to us with an interesting pattern of weakness. Methodology: A 32 year old Lady presented to us with two month history of asymmetrical onset proximal weakness of left followed by right lower limb with difficulty in getting up from squatting and multiple episodes of falls due to buckling at knees. Two weeks later, she developed asymmetrical distal weakness of upper limbs with difficulty in holding objects. Upper limb motor examination showed wasting of forearm, thenar and hypothenar muscles; distal weakness of both upper limbs with predominant involvement of finger flexors. Lower limbs showed proximal weakness of both limbs with predominant wasting and weakness of quadriceps. Rest of the neurological examination was normal. Results: Routine laboratory investigations were normal. Creatine phosphokinase levels were elevated at 775 IU/mL. Electromyography showed myopathic pattern and muscle biopsy demonstrated features suggestive of necrotizing myopathy. Myositis antibody panel was positive for anti-Jo-1 antibody. CT chest and pulmonary function tests done to evaluate for ILD came back normal. Discussion: Our patient was first treated with parenteral methyl prednisolone for 5 days, for which she did not show any improvement. Later oral steroids were continued with mycophenolate mofetil. She was given two doses of intravenous rituximab with which showed gradual improvement and is currently on regular followup. At the initial presentation, the pattern of weakness in this patient with predominant finger flexor and quadriceps involvement raised a suspicion of Inclusion body myositis. But a relatively earlier age of presentation and rapidity of disease progression prompted for the consideration of other possibilities. Conclusion: Our case demonstrates an interesting pattern of muscle weakness in anti-Jo-1 antibody myositis mimicking as IBM phenotype. This case highlights the need for recognition of atypical presentations of such inflammatory myopathies in view of their therapeutic implications.

Abstract ID: 636

A Case of atypical skull Base Osteomyelitis

Senthil Kumar, N. Thamilpavai, Lakshmi Narasimhan, L. Ravi

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: senthilmk16@gmail.com

Background and Aim: Skull base osteomyelitis is mostly seen in immunocompromised elderly patients, especially diabetics. Here we present a case of Central/atypical skull base osteomyelitis in a patient with no known comorbidities following otitis media. Methodology: This is a 59 year old female, with no known comorbidities, presenting with Right ear pain and discharge of 3 weeks duration followed by progressive swelling of Right eye with restricted eye movements, pain, excessive lacrimation, and gradual vision loss of 2 weeks duration. 4 days after hospital admission, patient developed Swelling and redness of left eye despite IV antibiotics. On examination, the patient had periorbital swelling with proptosis of both eyes , right more than left, with restriction of extraocular movements in all planes with no light perception in the right eye. Restricted abduction and elevation noted in left eye, with a normally reacting pupil and 20/100 Visual acuity. 2 days later, patient had developed left LMN facial palsy and sensory impairment along V2 distribution of left side of face with bilateral palatal palsy. Results: MRI brain revealed Right otitis media, with central skull base osteomyelitis with right orbital cellulitis and right optic nerve infarct. HRCT temporal bone revealed supportive findings. DNE / High nasal swab and Aural swab revealed no organisms. This patient was treated with broad spectrum IV antibiotics and IV amphotericin along with Retrobulbar amphotericin following which the patient showed significant recovery. Discussion: SBO often affects diabetics or immunocompromised individuals. SBO - central with cranial nerve paresis indicates progression of the disease. Conclusion: SBO presents a challenge for diagnosis and treatment. Early aggressive medical treatment, even in immunocompetent individuals, is necessary for better outcomes.

Abstract ID: 637

A Case of Congenital Myasthenic Syndrome

Noonavathu Vamshi Krishna, M. R. Manivannan, C. Justin

DM Neuro Pursuing, Other

E-mail: vamshikrishna948@gmail.com

Background and Aim: The congenital myasthenic syndromes (CMS) are inherited disorders of neuromuscular transmission is impaired by one or more specific mechanisms. These disorders are characterized by muscle weakness which is worsened upon exertion. The age of onset, severity of presenting symptoms and distribution of muscle weakness are variable. Most CMS are transmitted by autosomal recessive inheritance,a few specific subtypes are transmitted by autosomal dominant inheritance. Methodology: A 9 years old male child 2nd degree consanguineous marriage with full term NVD with no developmental delay presented with complaints of difficult in swallowing since 3yrs of age and with history of bilateral ptosis improves daily in the morning however worsens as the day progresses,h/o exertional dyspnoea and fatigue after walking for a few steps,h/o laryngomalacia. No history of regurgitation,no recurrent aspiration. On examination child is conscious oriented,bilateral ptosis present,EOM – restricted,Tone – Normal,DTR – depressed,b/l plantar- flexor. Results: Investigations: EMG is normal. CPK – 118. CK-MB – 16. Serum Calcium – 9.5. ESR – 12mm/hr. TSH – euthyroid. Acetylcholine receptor Ab <0.11, negative, ANA– negative, RA factor– negative. On Whole exome sequencing CHRNE gene mutation detected resulting in Autosomal recessive congenital Myasthenic syndrome associated with acetylcholine receptor deficiency. Discussion: CMS associated with acetylcholine receptor deficiency (AChR) is caused by mutation in the CHRNE gene on chromosome 17p13. The age of symptom onset ranges from infancy to adulthood.Clinical manifestations include hypotonia,respiratory insufficiency, weakness of ocular and bulbar muscles and skeletal deformities. EDx findings are variable and depend on the severity and distribution of weakness. RNS studies usually demonstrate a decrement but the decrement may be absent or restricted to facial muscles in mild cases. Conclusion: Patients with mutations in the CHRNE gene may have compensatory increased expression of the fetal subunit CHRNG and may respond to treatment with pyridostigmine or amifampridine. The index case has shown improvement with the treatment.

Abstract ID: 638

A Case of Dystonia

Shamili Devi, Senthur Pandiyan, Manikandan

DM Neuro Pursuing, Other

E-mail: shamili3119@gmail.com

Background and Aim: Fahr’s Syndrome also known as idiopathic basal ganglia calcification or Bilateral Striato Pallido Dentate Calcinosis (BSPDC) is an uncommon neurodegenerative disorder characterized by abnormal calcified deposits in the basal ganglia and other parts of the central nervous system. Methodology: 31 Years old male, k/c/o mild intellectual disability/ seizure disorder for past 15 years on antiepileptics presented with abnormal involuntary posturing of neck, hands and trunk and abnormal involuntary posturing of toes while walking for past 1 year along with tremulousness of all four limbs for past 1 year. H/o tingling and numbness in both hands and feet on and off. Past H/o cataract surgery present. On Examination patient had Generalized dystonia along with increased tone in all four limbs and cogwheel rigidity present. Results: Investigation includes MRI BRAIN shows bilateral symmetrical basal ganglia calcification along with bilateral thalamus and white matter calcification. Viral markers were negative. Serum calcium was 6.38 mg/dl, serum phosphorus -5.29 mg/dl and PTH -2.7ng/dl suggestive of hypoparathyroidism. Discussion: The calcification in Fahr’s syndrome is bilateral and symmetrical most commonly in the globus pallidus of the basal ganglia but may also occur in the thalamus, dentate nuclei, centrum semiovale, and rarely other intracerebral structures. The clinical presentation includes varying paresthesia, neuropsychiatric, dystonia, parkinsonism, convulsive seizures, and cerebellar symptoms. The pathogenesis and etiologies of Fahr’s syndrome are not clearly understood but endocrine abnormalities of calcium metabolism (Parathyroid), toxins, infections, and genetic causes may be involved. Parathyroid hormone (PTH) is important in maintaining calcium levels in the cerebrospinal fluid and preventing phosphorus deposition in the periventricular regions, which may partially explain why hypoparathyroidism coincides with the occurrence of Fahr’s syndrome in some patients. Conclusion: Fahr’s Syndrome is as yet considered to be incurable and management strategies are mainly supportive to relieve symptoms and treatment of the causative etiology. Evidence suggests that early diagnosis and treatment of the causative etiology may reverse the calcification process and hence improve neurological dysfunction.

Abstract ID: 639

A Case of Extensive Central Skull Base Osteomyelitis Due to Delayed Diagnosis: Rare But Life-threatening Illness

Babitha Sangaraju, R. Narasimhan, Marian Vijay, K. Sundaram

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: babithakool@gmail.com

Background and Aim: Central skull base osteomyelitis (CSBO) is a rare but fatal disease involving sphenoid, occipital, temporal bones. Diagnosing and treating CSBO is challenging. Here we would like to report a case of CSBO involving all the Cranial nerves on left side except I, V, XI which is very rare. Methodology: 50Y male k/c/o DM presented with subacute onset gradually progressive neurological illness in the form of left sided headache following tooth removal which subsided followed by multiple cranial nerve palsies over a period of 4months.On examination, he has visual loss in the left eye, left ptosis, all EOM in the left side were restricted, left LMN facial palsy, conductive hearing loss in the left ear, reduced palatal movements in the left side, weakness and atrophy of left half of the tongue. Results: Imaging studies with CT Skull base revealed right tegmen tympanic defect, left otomastoiditis. MRI Brain with orbital cuts revealed central skull base osteomyelitis with extensive involvement upto left orbital apex anteriorly and hypoglossal canal posteriorly. DNE revealed mucopurulent secretions from left middle meatus and excision biopsy from the same were sent for microbiological and histopathological examination. HPE revealed invasive mucormycosis, though repeated cultures yielded negative. Discussion: CSBO is a rare but life-threatening disease occurring as a complication of nasal/paranasal, oral cavity, pharyngeal infection without any otogenic source and accompanied by venous sinus thrombosis, meningitis, abscess, cranial neuropathies, and carotid invasion as complications. It is a rare entity that can demonstrate confounding radiologic, clinical, and laboratory data leading to a delay in diagnosis. Conclusion: The morbidity and mortality for skull base osteomyelitis are both high if left untreated, thus a high clinical suspicion and rapid diagnosis for prompt medical and surgical treatment even though of longer duration is life saving.

Abstract ID: 640

A Case of Fungal Abscess Mimicking as Space Occupying Lesion

M. Rupesh, Uma Maheshwari, S. Balasubramanian, R. Lakshmi

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drmrupeshkumar@gmail.com

Background and Aim: Even though fungal abscess usually occur in immunocompromised patients, fungal abscess can occur in immunocompetant individuals with history of IV drug abuse. Methodology: A 22 years old male who is a chronic alcoholic and IV drug abuser presented with Acute onset weakness of right upper limb and lower limb with deviation of angle of mouth to left and hemisensory loss over right upper limb and lower limb, right half of face and trunk. Results: On examination patient had normal higher mental functions, he had right UMN facial palsy, motor examination showed power of 0/5 right upper limb and lower limb, 5/5 on left side with right hemi sensory loss and right extensor Plantar. Discussion: Neuro imaging showed T2 / FLAIR Heterointense leison in left basal ganglia, thalamus with mid line shift, DWI showing restricted diffusion only in the periphery of the lesion with intracavitory projections with ring enhancement suggestive of fungal abscess. Conclusion: Histopathology showed PAS positive septate hyphae, suggestive of Aspergillosis. Patient was treated with antifungal medications and showed significant improvement.

Abstract ID: 641

A Case of Lead Intoxication with Acute Encephalopathy Motor Neuropathy and Anemia

Siddharth Maheshwari, Aparna Thomas, Mridula Rana, Suman Kushwaha, Aldrin Anthony

DM Neuro Completed, Other

E-mail: drsiddharthvds@gmail.com

Background and Aim: -Lead intoxication may develop either acutely or chronically.Plenty of cases of lead poisoning were reported in industrial era from 1965-1990.However recognition of lead as health hazard and adoption of several lead poisoning prevention policies in 90 s have drastically reduced cases of lead poisoning in 21st centuary with very few cases reported in last decade. Methodology: A 24 y old male patient admitted with complaints of headache of 1 month duration followed by 3-4 episodes of GTCS and altered sensorium on examination patient was stuporous, bilatral LR palsy present, his blood report showed moderate microcytic hypochromic anemia ,csf-protein elevation (87) with no cells and normal sugar. Contrast MRI brain showed generalized leptomeningeal enhancement On detailed enquiry patient had history of recurrent abdominal colic and occupational exposure to lead as a worker in invertor battery factory for past 8 yrs and serum lead level came 136 microgm/dl. NCV showed non recordable F waves (R) peroneal .Patient treated initially with anticonvulsant, antiedema measures followed by iv chelation therapy with EDTA for 5days followed by oral penicillamine and patient’s condition improved in the form of improvement in sensorium and became seizure free. Results: Patient’s condition improved symptomatically after initiation of chelation therapy and serum lead levels also decreased. Discussion: Emergency care like seizure control and management of raised ICP can be life saving in acute lead encephalopathy. Chelating agents to be given as early as possible. Conclusion: Lead encephalopathy should be considered in patients with encephalopathy who is likely to have occupational exposure.

Abstract ID: 642

A Case of Neurosarcoidosis Mimicking as Chronic Inflammatory Demyelinating Polyneuropathy

S. Swathy, Archana Netto, S. Praveen Kumar, D. C. Janardhan, Anita Mahadevan, Reghu Patil

Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India

E-mail: justswathy@gmail.com

Background and Aim: Sarcoidosis is a rare disease of granulomatous inflammation of unknown etiology. Nervous system involvement is only in 5–15% of patients. Sarcoid polyneuropathy is a rare initial presentation of sarcoidosis. Methodology: We report the clinical,electrophysiological,and pathological findings of a patient who presented as chronic inflammatory demyelinating polyneuropathy (CIDP) and subacute encephalopathy but was found to have sarcoid polyneuropathy with systemic sarcoidosis. Results: A 58-year-old man presented confused disoriented behaviour of 2 weeks and progressive ascending numbness and weakness of the bilateral lower and upper extremities for 3 months. Examination showed a delirious patient with lower motor neuron quadriparesis suggestive of polyradiculoneuropathy. Biochemistry was positive for hypercalcemia, high alkaline phosphatase, hyperammonaemia, and acute kidney injury. Electrophysiology showed sensorimotor polyradiculoneuropathy of mixed axonal and demyelinating variety. CSF had albuminocytological dissociation. Paraneoplastic evaluation revealed mediastinal hilar and cervical lymphadenopathy which were metabolically active in PET. Biopsy of superficial peroneal nerve, peroneus brevis, skin confirmed sarcoidosis granulomas in muscle with vasculitic neuropathy. The patient is a case of definite neurosarcoidosis as per the modified Zajicek criteria. He improved with steroid and methotrexate therapy. Discussion: The world prevalence of sarcoidosis is 5–50 per 100,000 highest being in Northern Europe. In India, the incidence or prevalence of sarcoidosis is unknown. Due to its multisystemic nature, the diagnosis of this condition is often difficult and delayed. Peripheral nerve involvement is also rare (15% to 20%). Axonal sensorimotor polyneuropathy is the most common, but mononeuropathy, mononeuritis multiplex, Guillain-Barre syndrome, and sensory small fibre ± autonomic neuropathy can be seen. Conclusion: Neurosarcoidosis is treatable and early and appropriate treatment can avoid patient having severe residual neurological impairments. Thus, in patients with atypical polyneuropathy, with associated systemic symptoms, we recommend clinical and laboratory testing for sarcoidosis including nerve and muscle biopsy. Aggressive treatment with a combination of steroids and immunomodulators should be considered early on.

Abstract ID: 643

A Case of Poirier–Bienvenu Neurodevelopmental Syndrome Due to Novel Missense Mutation CSNK2B

Swapan Gupta, Challepalle Baburao, Bharat Sahu

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi, India

E-mail: gupta.swapan@gmail.com

Background and Aim: Poirier–Bienvenu Neurodevelopmental Syndrome (POBINDS) is a rare neurodevelopmental disorder related to loss- of -function mutations of CSNK2B. Most common clinical features of POBINDS are drug refractory epilepsy and developmental delay and intellectual disabilities. Methodology: We present case of a 10 years old boy born out of non-consanguineous parents with normal birth history and history of motor delay and speech delay and intellectual disability. With multiple seizures, semiology of generalised tonic clonic and myoclonic absence and recurrent myoclonic absence status epilepticus episodes, epilepsy was drug refractory. MRI was unremarkable. Inter ictal EEG was suggestive of generalised 3-4 HZ irregular, polyspike wave discharges with abrupt onset and offset and fragmentation during sleep and accentuation of polyspike during sleep. Results: Novel CSNK2B missense mutations identified (p. Arg130Pro; ENST00000375882.7) as case of Poirier–Bienvenu Neurodevelopmental Syndrome (POBINDS). Patient responded with valproate, levetiracetam, zonisamide and clobazam with >50 % reduction in seizure frequency and complete remission of status episodes. Discussion: POBINDS is a rare neurodevelopmental disorder related to loss- of -function mutations of CSNK2B. To date this is a first described case of Poirier–Bienvenu Neurodevelopmental Syndrome due to Novel CSNK2B missense mutation. Conclusion: Clinician should be aware of Poirier–Bienvenu Neurodevelopmental Syndrome in which child present with developmental delay and drug refractory epilepsy especially myoclonic absence status.

Abstract ID: 644

A Case of Rapidly Progressive Spinal cord Disease with Asymptomatic Intracranial Lesion

B. R. Sindhu

MBBS completed, Other

E-mail: sinbin419@gmail.com

Background and Aim: A 48 yr old male on treatment for left trigeminal neuralgia presented with pain in the right arm which was insidious onset, progressive associated with insidious onset of weakness in the right lower limb followed by left lower limb in the next 2 days associated with sensory level at T4 and urinary retention. A compressive myelopathy was diagnosed. On imaging, patient was found to have extra dural lesion, an extension of right Pancoast tumour. Incidentally MRI brain with contrast study showed lesion in right frontal and temporal lesion suggestive of metastasis. Methodology: After informed consent, detailed history was taken, thoroughly examined. Relevant investigations were done. Imaging of brain , spinal cord and thorax was taken. Results: A 48 yr old male on carbamazepine for trigeminal neuralgia presented with compressive myelopathy at thoracic level , metastasis from Pancoast tumour along with intracranial spread. Discussion: Pancoast tumour aka superior pulmonary sulcus tumour presents with shoulder pain and horner syndrome. Pulmonary symptoms are usually late as the tumour is placed peripherally. Neurologic complications can be C8 and T1 root involvement affecting the intrinsic muscles of the hand. 5% develop spinal cord compression causing paraplegia. In our patient , intracranial metastasis were present along with spinal cord compression masked by anti-epileptics. Needle guided biopsy is done to diagnose and later followed by chemoradiotherapy with surgical resection. Conclusion: Pancoast tumour can cause rapidly progressive spinal cord compression in 5% of the cases. Timely evaluation and treatment is required.

Abstract ID: 645

A Case Report of an Isolated Nodulr Infarction Mimicking a Peripheral Vestibulopathy

Srujanitha Tadi, S. Ramu, P. Murugan

DM Neuro Pursuing, Other

E-mail: anithatadi@gmail.com

Background and Aim: Isolated nodulus infarction a rare entity commonly mistaken to be of peripheral vestibular disorder. Differentiating a central lesion from a peripheral lesion is not only important in planning a management strategy but also prevents long-term residual deficits. Here we report a case of isolated nodulus infarction mimicking a vestibular neuropathy. Methodology: A 52 year old male k/c/o diabetes mellitus presented with sudden onset of giddiness, vomiting and unsteadiness while walking and sitting of 9hrs duration. Neurologic examination showed truncal and gait ataxia and horrizantal jerky gaze evoked nystagmus with normal head impulse test, cranial nerves, tone, power and reflexes. Results: MRI Brain showed acute infarct in nodulus of cerebellum. Patient was managed conservatively and is under follow up. Discussion: Cerebellar nodulus connects the peripheral vestibular system and central vestibular system. Isolated nodular infarction is rare. Lesion presents with acute onset postural vertigo, nystagmus, ataxia mimicking peripheral vestibular disorders. Treatment with anti edema measures, anticoagulants and antiplatelets improves the outcome. Conclusion: Re-evaluation is required for proper diagnosis of a patient with unexplained giddiness with discordance of symptoms and clinical examination. Early and prompt treatment prevents complications and improves outcome.

Abstract ID: 646

A Case Series of Hereditary Motor Sensory Neuropathy in a Family

Nisskala Harikrishnan, S. Saravanan

DM Neuro Pursuing, Other

E-mail: nisskala@gmail.com

Background and Aim: Charcot Marie Tooth disease is the most common inherited neuropathy with an estimated prevalence of 1 per 2500 individuals. CMT may be classified by mode of inheritance, electrophysiological studies, chromosomal locus and causative genes. CMT 1 and the vast majority of subtypes of CMT 2 display AD inheritance. Methodology: Here we analyse the clinical features of 3 CMT patients belonging to a same family. Results: Patient A – a 29 years old female presented with complaints of difficulty in walking , weakness of both hands associated with deformity of both hands and legs since childhood. On examination there is pescavus deformity in both legs. Wasting noted in B/L FDI, hand muscles and legs. There was hypotonia in both wrist and ankle. There was symmetric distal more than proximal weakness with brisk reflexes and high stepping gait. B/L plantar was mute. JPS and vibration sense was decreased. NCS showed demyelinating polyneuropathy. H/o similar complaints in father, Younger sister and her niece Patient B – a 53 year old male who is the father of patient A also had similar complaints of deformity and difficulty in walking since childhood. There was no H/O weakness of upper limbs. He was born to non consanguinous marriage with nil significant birth history Patient C – A 20 days old female child born to non consanguineous marriage who is the niece of patient A. parents noticed deformity of both legs. She was born through LSCS and there was H/O birth asphyxia. O/E there was hypotonia in both ankle . Child moved all 4 limbs. Discussion: There is no cure for CMT, but physical and occupational therapies may help with the disabling symptoms of the disease. Conclusion: Ongoing research on CMT includes efforts to identify more of the mutant genes and develop interventions to slow down the disease process.

Abstract ID: 647

A Case Spinal Cord Ischemia Presenting as Quadriparesis after Intra-abdominal Postoperatively

Rahul Gandhi, Thamil Pavai, Lakshmi Narasimhan, L. A. Ravi

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: rahulirg10@gmail.com

Background and Aim: Spinal cord ischemia is rare but devastating disease that present with acute paraparesis or quadriparesis diagnosed by clinical and neuroimaging. Here is the case report of spinal cord ischemia in a patient presenting with acute quadriparesis post intra-abdominal surgery. Methodology: 41 years old diabetic female had history of intestinal obstruction due to jejunal gangrene underwent jejuno-jejunal anastomoses developed acute onset non-progressive weakness of both lower limb on postop day 3 with history of truncal weakness, numbness of both lower limb below hip with unaware fecal incontinence with altered sensorium. On examination after recovering from altered sensorium, patient found to had hypotonia, areflexia and weakness of bilateral upper and lower limbs, loss of pain and temperature below xiphisternum, impaired vibration and position sense in both lower limb till D1 spine level. Results: MRI spine done showed long segment hyperintensity in the dorsal cord at D3 to D12 level. NMO and MOG antibodies with Vasculitis workup were negative. Repeat MRI spine taken 1 month later showed T2 intramedullary hyperintensity in dorsal spine with owl’s eye appearence hyperintensities on axial cuts with hyperintensities on D3 to D7 vertebral body suggestive of vertebral body infraction. Discussion: Patient presenting with acute quadriparesis with MRI imaging showing intramedullary spinal cord hyperintensity with vertebral body infarction suggestive of etiology of spinal cord ischemia. Conclusion: Hence with this report we conclude that in any patient with acute nontraumatic myelopathy with non inflammatory CSF analysis with spine imaging showing intramedullary T2 hyperintensity with associated vertebral body infarction, spinal cord ischemia should be considered especially in postoperative period, after excluding other causes.

Abstract ID: 648

A Cross-sectional Analytical Study of Autonomic Dysfunction in Migraine Patients in a Tertiary Care Centre

N. Anand, S. Saravanan, S. Ravi

DM Neuro Pursuing, Other

E-mail: aohawk89@gmail.com

Background and Aim: Patients with Migraine headaches are associated with many symptoms which are behavioral, neurological and systemic. Autonomic dysfunctions like yawning, sweating, flushing, and diarrhea are associated with migraine which occurs during the time of headache as well as in pain-free intervals. They affect the quality of life and determine the type of drugs to be used for their other ailments like associated hypertension and diabetes. Knowledge about their association is important in providing prophylactic measures for them. Aims To study the frequency of ANS disturbances occurring in migraine patients. To correlate the ANS disturbances with the type of migraine the patient is suffering from. Methodology: This is a Cross-sectional study that included 134 patients of Migraine attending neurology department from August 2021 to July 2022. Patients are diagnosed as migraine on the basis of ICHD and migraine headache is typed. Patients suffering from other headaches, diabetes, primary autonomic disorders and taking drugs affecting autonomic functions are excluded from study. Bedside autonomic function tests are done to find out the ANS system disturbances. The tests include Blood pressure and heart rate response to standing, sustained hand grip and immersion in cold water,Valasalva changes in heart rate and blood pressure. Results: Mean Age was 31.41 and most of the subjects were females.63 % had Migraine without aura and 37% had migraine with auras. Observation showed 31% of patients had autonomic dysfunction. Majority of the patients with autonomic dysfunction had migraine with aura. The difference was statistically significant. The commonest Autonomic disturbance was the abnormal Valsalva ratio and is more common in migraine with aura. Discussion: Autonomic disturbances are present in proportion of individuals with migraine. Migraine with auras is more commonly associated with autonomic dysfunction. Conclusion: Autonomic disturbances affect quality of life and profession. In the long run untreated migraine may lead on to permanent neurological changes.

Abstract ID: 649

A Curious Case of Young Stroke

Karthik Harisankar, Sahil Mehta, Vivek Lal, Sucharitha Ray

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

E-mail: karthik.hshankar@gmail.com

Background and Aim: 40 year old male with diabetes for past 10 years presented with complaints of progressive right hemiparesis for past 1 and half years with progressive bulbar symptoms with severe dysphagia and anarthria and pseudobulbar affect.There was prominent spasticity with brisk reflexes in the upper limb with extensor plantar response.There was mild wasting in upper limbs. Methodology: MRI Brain showed Altered signal intensity is seen involving white matter of bilateral cerebral hemispheres, internal and external capsule, bilateral cerebral peduncles, brainstem along the cortico spinal tracts, bilateral cerebellum and right middle cerebellar peduncle with patchy diffusion restriction at places.CSF showed normal cells with protein 62 and normal sugars. Infective workup was negative. The possibility of an infiltrative pathology like Lymphoma was considered. But CSF malignant cytology was negative and CSF IL-10/IL-6 was not suggestive. FDG PET did not show definite PET-CT evidence of clinically significant abnormal hyper metabolism. The possibility of CADASIL was also considered in view of the temporal lobe involvement.Adult onset leukodsytrophy was also considered. Patient received a course of 5 days of methyl prednisolone pulse during the hospital stay. There was improvement in his power from 2 to 4 in his right upper limb. There was mild improvement in his bulbar symptoms ; was able to tolerate semisolids with minimal improvement in spastic dysarthria. Results: Whole genome exome sequencing revealed NOTCH-3 mutation.Likely pathogenic. Discussion: The presentation of the patient was in form of progressive bulbar symptoms and spasticity.There was no family history of strokes with imaging showing diffuse white matter changes.The patient even showed mild response to steroids. Conclusion: Genetic stroke syndromes need to be kept in back of mind when dealing with atypical presentations with prominent white matter changes.

Abstract ID: 650

A Diagnosis Hiding in Plain Sight – A Rare Case of Acute Hepatic Porphyria with Leucoencephalopathy

B. Thejus, Praveen Kumar, Archana Netto, D. C. Janardhan, K. Pramod

Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India

E-mail: apunkrockangel@gmail.com

Background and Aim: The porphyrias are metabolic disorders caused by altered activities of enzymes within the heme biosynthetic pathway.Porphyrias can cause neurovisceral manifestations (eg, motor and sensory peripheral neuropathy, neuropsychiatric changes) or cutaneous photosensitivity (chronic and blistering).We report an interesting case of an acute hepatic porphyria. Methodology: We report a case of Acute hepatic porphyria with leucoencephalopathy. Results: A 54 year old male born out of a 3rd degree consanguineous marriage initially presented with acute onset altered sensorium with agitated delirious state and on physical examination had waxy periorbital hyperpigmentation , chronic blistering cutaneous blistering lesions , post inflammatory scarring in the sun exposed areas of his limbs and face with a significant family history of similar skin lesions in his first cousin brother , patient underwent routine blood investigations which were normal , MRI BRAIN showed frontal lobe atrophy with leukoencephalopathy , NCS of all 4 limbs showed motor axonal polyneuropathy of all 4 limbs , Urine Porphobilinogen was positive and urine 24 hour total porphyrin levels was elevated , skin biopsy done also was consistent with cutaneous porphyria. Discussion: The patient matched the clinical and investigational profile of an acute hepatic porphyria with leukoencephalopathy and was treated with I.V dextrose infusions , antipsychotics and other supportive measures and showed clinical improvement. Conclusion: A high index of suspicion should be present in any patient presenting with acute neuropsychiatric manifestations and chronic blistering lesions in the sun exposed areas for acute hepatic porphyria if both these combinations exist clinically to specifically look for either Hereditary coprophyria or variegate porphyria.

Abstract ID: 651

A Diagnostic Dilemma in a Patient with Ethambutol-induced Optic Neuropathy and MOG Antibody Positivity: New Insights

Prabhjit Kaur, Karthik Mahesh, Basavaraj Tigari, Aastha Kapila, Ramandeep Singh, Paramjeet Singh, Vivek Lal

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

E-mail: missghotra.ghotra@gmail.com

Background and Aim: 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. We investigate the patients who presented as EtON with MOG antibody came out to be positive during evaluation. Methodology: Patients who were on anti-tubercular treatment (ethambutol-based) and clinically diagnosed with EtON were recruited. Detailed neurological, ophthalmological, and radiological investigations were carried out. Results: Mean age ± SD of the patients were 39 ± 14.09, and time to onset after initiation of ethambutol was 5.12 ± 2.4 months. All of them developed symptoms even at dose of 15-20 mg/kg/day. At baseline, three patients presented with severe vision loss (>6/60) and one with mild vision loss (6/18). On Fundus Examination, three were having temporal pallor and one having optic disc atrophy with majority of the patients having optic chiasma hyperintensity and chiasma atrophy on radiological examination. Three patients were having bilateral lower limb numbness and parasthesia. Complete blood and CSF investigations were normal except anti-MOG antibody which came out to be strong positive (++). Patients were treated with high dose pulse steroids (IVMP) for five days and immunomodulators. Improvement was seen in visual acuity of three patients with improvement in peripheral numbness on treatment. Discussion: EtON is a well-known side effect of ethambutol since it had been in use. MOG-IgG antibody positivity correlates with underlying inflammatory pathway at the optic nerves resulting in bilateral, painless and progressive vision loss. Conclusion: Though the etiology of EtON is still unknown, the positivity of anit-MOG antibody point towards the underlying inflammatory pathology which precipitates upon ethambutol exposure but it needs further evaluation for downregulatory pathways.

Abstract ID: 652

A Rare Case of CNS Granulomatous Angitis Presenting as Cortical Myoclonus in a Middle Aged Female

Nareddy Sandeep Reddy, Neeraj Balaini, Thatipally Manod Reddy, Jitupam Baishya, Vivek Lal

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

E-mail: sandeepnsr32@gmail.com

Background and Aim: cns granulomatous angitis can present as focal neurologicl deficits as seizures , hemiparesis , monoparesis but there are not many cases where it predominantly presented as focal myoclonus. Methodology: we present here a case of 47 year old female patient had been previously evaluated for headache in 2016 when she was found to have pachymeningeal enhancement in left fronto-temporo-parietal region and left cerebellar and tentorium cerebelli , she was treated with steroids and immunomodulated with azathioprine 100 mg /day but she herself had stopped medications and started CAM intake for 5 years (most likey had steroids ) as she had developed cushingoid features with weight gain. Results: 47 year old female presented with h/o vascular headache since past 7 years as mentioned , presented with history of left upper limb focal seizures with secondary generalization since past 6 months and also history of action and stimulus sensitivr myoclonus of left upper limb and lower limb since past 1 month , on examination she was spastic hemiparesis of left upper limb with motor power of 4/5 and on extra pyramidal examination there is sudden brief jerky movements noted in left distal upper limb precipitated by action and stimulus and disapperas in sleep. Discussion: she was evaluated with csf analysis including gene expert for tb which was negative , ESR 23, CRP 50.5, RA factor 95.3, serum ACE level is normal , ANA blot is negative , her CT chest was normal . MRI brain showed pachymeningeal enhancement over right frontoparietal region with nodular patchy thickening suggestive of granulomatous etiology she underwent open brain biopsy and histopathology showed small well formed granulomas/multinucleated giant cells and granulomatous inflammation with destruction of vessel wall , PAS/ZN stain is negative for AFB and no evidence of malignancy. Conclusion: she was diagnosed as a case of CNS Angitis primary.

Abstract ID: 653

A Rare Case of DHTKD1 Mutation Presenting as Isolated Respiratory Muscle Weakness

Manisha Mohanty, Deepak Menon, Saraswati Nashi, Seena Vengalil, Atchayaram Nalini

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: manisha4236@gmail.com

Background and Aim: DHTKD1 is a nuclear gene that encodes ‘dehydrogenase E1 and transketolase domain-containing 1’, which is essential in mitochondrial metabolism. It was first identified in the patients of 2-amino-apidic and 2 oxoapidic aciduria. Rare cases of mutations in this gene have been described in patient presenting as Charcoat Marie Tooth disease and Amyotrophic Lateral sclerosis. These isolated case reports and series implicate the pleotropic phenotypic presentation of DHTKD1 mutation in neurodegenerative diseases. Here we present a 72 year old patient with progressive respiratory and neck muscle weakness with brisk reflexes found to have a heterozygous mutation in the DHTKD1 gene. Methodology: A 72 year old man presented to us with two years history of progressive respiratory muscle weakness without speech or swallowing difficulty or limb weakness. Patient was dependent on non-invasive ventilation by the end of one year after symptom onset. On examination he had wasting of the upper limbs predominantly distal with presence of minipolymyoclonus and fasciculations. Power was normal except neck flexor (grade 4). Deep tendon reflexes were brisk. The likely possibility of a motor neuron disease was considered. Electrophysiological studies showed reduced motor and sensory amplitudes with needle electromyography showing denervation pattern in cervical, paraspinal and lumbosacral segments. Clinical exome sequencing showed heterozygous mutation in exon 8 of DHTKD1 gene, variant c.1455T>G(p.Tyr485Ter). Patient was offered rehabilitative care and palliative care. Results: This is the second case of DHTKD1 mutation being reported from India. Discussion: This rare phenotype of isolated respiratory muscle weakness with sparing of bulbar and limb muscles is being described for the first time with this particular mutation. Conclusion: The gene DHTKD1, which has been recently identified, is implicated in a range of neurodegenerative disorders. This report contributes to the broadening of the clinical characteristics that are linked to mutations in the DHTKD1 gene.

Abstract ID: 654

A Rare Case of Childhood-Onset Dystonia

Ande Adarsh, Ramesh Ramachandra, Sateesh Kamera, Naveen Saddanapu

DM Neuro Pursuing, Other

E-mail: andeadarsh@gmail.com

Background and Aim: Childhood-onset parkinsonism-dystonia-3 (PKDYS3) is due to homozygous or compound heterozygous mutation in the WARS2 gene located on chromosome 1p12.Along with PKDYS3,NEMMLAS (Neurodevelopmental disorder, mitochondrial, with abnormal movements and lactic acidosis, with or without seizures) is also caused by the same gene mutation.Both the diseases share some come features .Compared to PKDYS3, NEMMLAS is the more severe disease.PKDYS3 patients usually present with Parkinsonism and dystonia.Mild cognitive impairment or psychiatric disturbances can be seen in some patients. Parkinsonism features may be responsive to treatment with levodopa. Methodology: Case Report: A 15 year old male, born to non-consanguinous parents presented with c/o tremor of upper limbs, altered speech and dystonic posturing of the neck, trunk and limbs since 4 years of age which were insidious in onset and gradually progressive. Clinical examination revealed hyperextended neck with deviation to right side and scoliosis.There is mild cognitive impairment, rigidity of all four limbs, gait and appendicular ataxia with no signs of sensory, cranial nerve, or autonomic dysfunction.MRI brain showed diffuse cerebellar atrophy.Nerve conduction studies, Serum Lactic acid and & fundoscopy were normal. Exome sequencing was performed to identify pathogenic sequence variants, associated with primary dystonia/ related disorder.A heterozygous WARS2 gene mutation with variation c.37T>G (p.Trp13Gly) and c.882C>A (p.Ser294Arg) were identified.The patient was diagnosed as PKDYS3.He was started on levodopa and trihexiphenydl with mild improvement in symptoms. Result: -.Discussion: Childhood-onset parkinsonism-dystonia-3 (PKDYS3) is a neurodegenerative disorder which is of autosomal recessive inheritance.It usually begins in infancy or early part of childhood.Along with parkinsonism,the patients may also present with dystonia,myoclonus,ataxia,tremors and also ballismus in PKDYS3. Conclusion: Parkinsonism-dystonia-3 is a rare genetic disorder caused by WARS2 gene mutation.Only few case reports were available in the literature.Here we report a rare such case.

Abstract ID: 655

A Rare Case of Dysphagia in a Middle Aged Female

Arvind Visweshwar, P. K. Murugan, Ramu

DM Neuro Pursuing, Other

E-mail: dr.arvind.visweshwar@gmail.com

Background and Aim: Chorea-acanthocytosis (ChAc), as the most common subtype of neuroacanthocytosis syndrome, is characterized by the presence of acanthocytes and neurological symptoms. It is thought to be caused by the VPS13A (vacuolar protein sorting-associated protein 13A) mutations. We discuss a case of middle-aged female with movement disorder and difficulty eating and swallowing. Methodology: A 34-year-old, women presented with gradual, progressive neurological illness in the form of abnormal choreiform movements starting in perioral region progressed to involve head, tongue, limbs and tongue disappearing during sleep without any posturing, with difficulty eating associated with involuntary tongue protrusion associated with dysarthria and frequent anger outbursts, tongue and lip biting with negative family history and drug history. On examination had MMSE of 24, with perioral dyskinesia, darting tongue, feeding dystonia, milkmaid grip and generalized chorea with hypotonia and depressed DTR with normal sensory, cerebellum and autonomic function. Results: MRI showed caudate atrophy and dilation of anterior horns of the lateral ventricles and peripheral smear showed presence of acanthocytes, NCS showed low SNAP with normal conduction velocities and normal motor nerve conduction studies. Discussion: This is a case with classical presentation of Chorea-acanthocytosis. Chorea-acanthocytosis is a rare autosomal recessive disorder characterized by onset in young adulthood of chorea, oro-lingual-facial dystonias, tics, seizures and distal muscle wasting. Conclusion: No formal diagnostic criteria for chorea-acanthocytosis exist, the disorder is suspected on basis of clinical symptoms and presence of acanthocytosis. No curative or disease modifying treatment for chorea-acanthocytosis currently exist.

Abstract ID: 656

A Rare Case of Friedreich’s Ataxia Variant

Sanjo John, N. Shobana, M. Sacratis, V. Sadeeshkumar, C. J. Selvakumar, M. Joby

DM Neuro Pursuing, Other

E-mail: sanjokalathil24@gmail.com

Background and Aim: Friedreichs ataxia is the most common autosomal recessive ataxia. Harding described typical form of friedreichs ataxia with essential criteria consisting of onset of symptoms before 25 years, progressive ataxia of gait and limbs, absent knee and ankle jerks, reduced motor wave conductions, extensor plantar reflex and late onset of dysarthria . AIM To present a case of Friedreich’s ataxia variant. Methodology: CASE SUMMARY A 34 year old male presented with history of unsteadiness in gait at the age of 16 years.Five years later patient had sensory disturbances and scanning speech.Patient also had severe stiffness of both lowerlimbs and was also diagnosed with diabetes mellitus. On examination, hammer toe, pescavus and scoliosis were present , Cranial nerve examination revealed nystagmus and square wave jerks. Tone was increased in lower limbs.with scissoring of limbs, all reflexes were exaggerated except ankle jerks and plantars were bilateral extensor. sensory disturbance were present in bilateral lower limbs . Cerebellar involvement like impairment of finger finger nose test and dysdiadochokinesia and rebound phenomenon was present bilaterally. There was cerebellar scanning speech, nystagmus and square wave jerks. NCS was suggestive of sensoryaxonal neuropathy in both upperlimb and lowerlimb.2D ECHO revealed hypertrophic cardiomyopathy.MRI Brain showed cerebellar atrophic changes and cervical spinal canal narrowing. Results: Friedreichs ataxia mutational analysis was done by real time PCR method which revealed homozygous expansion of GAA repeats (> 65 repeats) in the frataxin gene of this patient. Discussion: Classical friedreichs ataxia patients have absent reflexes. Atypical phenotypes of Friedreichs ataxia are late onset friedreichs ataxia (LOFA) , very late onset friedreichs ataxia ( VLOFA), and friedreichs ataxia with retained reflexes. Conclusion: Knowledge of atypical presentations of friedreichs ataxia is very important in clinical practice. It will help us differentiate friedreichs ataxia from other differential diagnosis.

Abstract ID: 657

A Rare Case of GNE Myopathy with Vasculitis

Jonnalagadda Mohankrishna, Srikanth Gadam

DM Neuro Completed, Other

E-mail: mohankrishna88@gmail.com

Background and Aim: To present a rare case of GNE myopathy with vasculitis. Methodology: We report case of 25 years old male who presented with slowly progressive weakness of limbs over 2 years.Weakness started in lower limbs, initially in the left leg followed by right (distal >proximal) followed by weakness of upper limbs ( only distal)after one year.There were no sensory symptoms.On examination had Quadriparesis (distal>proximal),bilateral foot drop,atrophy of muscles of hand, no hypertrophy.Sensory system was intact.On evaluation NCS showed decreased CMAPs and normal SNAPs.EMG was suggestive of myopathy.Serum CPK was mildly elevated (350iu/ml).ANA profile and other metabolic workup was normal.Muscle biopsy (left TIbialis Anterior )showed features of myopathy with rimmed vacuoles and lymphocytic infiltration of blood vessel wall causing collapse suggestive of vasculitis.Genetic analysis showed a Heterozygous Pathogenic mutation of c.208G>A p.val696met on Exon 12 of GNE gene. Results: 25 years old male presenting with progressive weakness of limbs,distal>proximal, clinically suggestive of distal myopathy.Electrophysiolocal studies suggestive of myopathy.Histopathological examination showed myopathy with rimmed vacuoles with vasculitis.Genetical studies showed a heterozygous pathogenic mutation of GNE gene. Discussion: GNE myopathy is rare distal myopathy also known as Hereditary inclusion body myopathy or Nonakas myopathy or Distal myopathy with rimmed vacuoles or Quadriceps sparing myopathy. It is an Autosomal recessive disease that presents as in homozygous or compound heterozygous forms with predominantly compound heterozygous mutations in Indian subcontinent.Is is caused by mutations in the UDP-N-acetylglucosamine 2-epimerase and N-acetylmannosamine kinase (GNE) gene on chromosome 9.clinically the most striking feature is distal leg weakness sparing the quadriceps distinguishing it from other myopathies.The histopathologic hallmark of DMRV is the presence of rimmed vacuoles predominantly in atrophic fibers. Conclusion: we report a rare case of distal myopathy with clinical,histopathological and genetic studies suggestive of GNE myopathy with Vasculitis.

Abstract ID: 658

A Rare Case of Idiopathic IX and X Cranial Nerve Palsy in a Child

S. Saravanan

DM Neuro Completed, Other

E-mail: drsaravananneuro@gmail.com

Background and Aim: Multiple cranial Neuropathy are uncommon in pediatric patients, more so is lower cranial nerve palsy. We report one such case. Methodology: We report a case of 8 year old female child, who presented with acute onset, slowly progressive neurological illness of one week duration in the form of nasal regurgitation, cough on swallowing liquids with dysarthria and nasal twang to her voice. There was no associated painful swallowing, weakness of limbs, unsteadiness of gait, headache or fever. Parents deny preceding history of fever, URTI and diarrhoea. On examination, her vitals were stable. Neurological examination revealed decreased palatal movements on the right more than left side, deviation of uvula to the left side with absent palatal sensation and gag reflex on the right side. The other cranial nerves were normal. Motor system, cerebellum and sensory examination were unremarkable. Results: Blood investigations including ANA, CRP, ESR were all normal. Pharyngeal swab, video laryngoscopy were within normal limits. CSF analysis and NCS done in the second week of illness were normal. CT skull base, MRI brain contrast with CISS sequence were unremarkable. we considered inflammatory pathology versus demyelination as our provisional diagnosis and started her on IV steroids followed by oral steroid tapering for 6 weeks. Patients showed drastic response to steroids and recovered completely within a week. Discussion: Common cranial nerve palsies reported in children are third, fourth and sixth. Most common cause being neoplasm, followed by meningitis and demyelination. Conclusion: Isolated idiopathic ninth and tenth cranial nerve palsy in children are rarely reported in the literature.

Abstract ID: 659

A Rare Case of Primary CNS Vasculitis Presenting with Recurrent Hemiparesis

Raamprasath Chandrasekaran, S. Saravanan

DM Neuro Pursuing, Other

E-mail: raamprasath.281292@gmail.com

Background and Aim: Primary angitis of the central nervous system is an inflammatory brain disease first described in 1959. 3-5% of cerebrovascular events in young patients are caused by primary CNS vasculitis. Calabrese and mallek coined the term primary angitis of the central nervous system and proposed the diagnostic criteria which includes 1) History or clinical findings of an acquired neurological deficit of unknown origin. 2) Cerebral angiogram or CNS biopsy with classic features of vasculitis, 3)No evidence of systemic vasculitis. Methodology: A 32 year old female a known hypertensive developed left hemiparesis 2 years back treated with antiplatelets and statins now presented with right hemiparesis, spastic dysarthria and cough on food intake for past 5 days. She was having both proximal and distal weakness involving all four limbs in current presentation. No significant family history. She had spastic dysarthria, left UMN 7th cranial nerve palsy with exaggerated gag reflex, spastic weakness involving all four limbs with exaggerated Deep Tendon Reflex with ankle clonus on left side. Bilateral plantar were extensor. Sensory system and cerebellum was normal. Results: CT brain showed multiple infarcts over right MCA territory and left MCA and left MCA-PCA territory. Cerebral angiogram showed focal constriction and dilatation of vessels with beaded appearance of M1 left MCA. Immunological and coagulation workup was negative. CSF analysis showed elevated protein. she was treated with prednisolone along with antiplatelets and was discharged with improvement in speech with advice to continue steroids for atleast 6 months. Discussion: she was treated with prednisolone along with antiplatelets and was discharged with improvement in speech with advice to continue steroids for atleast 6 months. Conclusion: 3-5% of stroke in young patients has primary CNS vasculitis as underlying etiology. It is important to evaluate for the same and early administration of steroids in such patients is beneficial.

Abstract ID: 660

A Rare Case of Rapidly Progressive Dementia

P. S. Apsara, P. K. Murugan, Ramu Sekar

DM Neuro Pursuing, Other

E-mail: apsara.aps73@gmail.com

Background and Aim: Creutzfeldt–Jakob disease (CJD) is a rare and fatal human neurodegenerative disorder characterized by rapidly progressive dementia, myoclonus, cerebellar, pyramidal, extrapyramidal, visual symptoms, and psychiatric manifestations. Pathological changes in the disease include vacuolar lesions that give a spongiform appearance to the brain, preferably in the basal ganglia, thalamus, cerebellum, and cerebral cortex. Methodology: A 49-year-old female was in her usual state of health until 3 months ago when her family members noticed that she seemed to have easy forgetfulness and worsening functional impairment. She started experiencing visual hallucinations. Later she also developed behavioral abnormality in the form of urinating and undressing in public places. Myoclonic jerks were noted over the left side of the face. Results: Routine laboratory investigations including serology for hepatitis B, HIV, and syphilis were all negative. Lumbar puncture for CSF evaluation along with culture revealed insignificant findings. Thyroid hormones, vitamin B12, and folate levels were all within normal limits. Chest X-ray, electrocardiogram, and abdomino pelvic ultrasound were otherwise unremarkable. EEG report showed random paroxysmal, sharp, and slow wave discharges seen bilaterally.MRI of the Brain revealed a cortical ribbon sign. The autoimmune encephalitis panel was found to be negative.CSF protein 14-3-3 is 2.3ng/ml (positive). Discussion: Rapidly progressing dementia is an interesting clinical scenario with many diagnostic possibilities.sCJD can be clinically heterogeneous, especially if initially presented with behavior and personality changes that obscure neurological findings. Conclusion: A high index of suspicion, awareness of specific biomarkers, and radiologic signs are crucial in diagnosing this condition. Unfortunately, there are no effective treatment options for prion diseases.

Abstract ID: 661

A Rare Case of Rapidly Progressive Dementia Parkinsonism

Bharat Sahu, Swapan Gupta, Babu Challepalle, Prince Kumar

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi, India

E-mail: bksahu.sahu0@gmail.com

Background and Aim: Rapidly progressive dementia parkinsonism is a group of disorder which is commonly seen in clinical practice and is characterised by rapidly progressing cognitive decline and parkinsonism with variable psychiatric, extrapyramidal and pyramidal features. Methodology: A 12year female patient born out of second degree consanguineous marriage with normal birth and developmental history presented with acute onset cognitive decline for 2 months. Patient had worsening in her symptoms after an acute febrile illness for 2 to 3 days with development of Parkinsonism in the form of rigidity and bradykinesia with development of generalised dystonia along with dysautonomia making her bed bound and mute over 15 days. MRI showed diffuse atrophy. Results: After ruling out other possible other causes like Wilson’s disease, mitochondrial, infections etc. NGS was done which came out to be positive for VPS13C mutation. She was started on Levodopa therapy, to which she has responded to an extent that she is able to walk with some support. Discussion: VPS13C mutations is a rare cause of early onset Parkinson’s disease characterized by rapid and severe disease progression and early cognitive decline and show moderate response to levodopa therapy. To date only 16 clinically described cases of VPS 13C -related PD cases have been reported in literature. Conclusion: Clinician should be aware of VPC13C mutation where patient can present with rapidly progressive dementia Parkinsonism and it would be worth trying levodopa therapy in such patients.

Abstract ID: 662

A Rare Case Report of Subacute Sclerosing Panencephalitis Presenting with Balint’s Syndrome and Sinus Bradycardia

Swati Parida, Deepika Joshi, Anand Kumar

Banaras Hindu University, Varanasi, Uttar Pradesh, India

E-mail: swatiparida1989@gmail.com

Background and Aim: Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence. It is caused by persistent defective measles virus. Here we present a case with Balint’s syndrome in whom visual agnosia, optic ataxia and praxis deficits symptoms were the initial presenting features of SSPE. Methodology: A 22 years male presented with inability to recognise faces of persons, objects, pictures, colours although he was able to describe their features for last eight months. He had difficulty in identifying the big letters but was able to read the small letters inspite of good visual acuity. He was able to recognize some part of a picture, but not the whole. When he was given a cup/ glass/ pen to hold he would spread his hands here and there to grasp it. Six months into the illness he developed generalized myoclonus and cognitive decline in the form of apathy, behavioural problems and forgetfulness. He had past history of measles at the age of 5 years. Blood pressure was 110/70mmHg and pulse rate was 48/minute. Higher mental functions could not be accomplished due to poor attention. Fundoscopy revealed pale bilateral optic discs. He was treatment naïve patient so probability of drug induced bradycardia was ruled out. Results: Routine blood tests were normal. Cerebrospinal fluid analysis revealed an IgG measles titre =16,578 U/ml (serum IgG measles titre=6365U/ml and CSF/Serum quotient=4.34). Brain MRI was suggestive of bilateral posterior parietal and occipital atrophy. Electrocardiography showed sinus bradycardia. 2D-echocardiography, Holter and cardiac MRI were normal. Discussion: Zuhal Yapici reported the first case of SSPE presenting as Balint’s syndrome in 2005. Conclusion: This is probably the first SSPE case from India presenting as Balint syndrome with sinus bradycardia. Sinus bradycardia probably could be due to dysautonomia.

Abstract ID: 663

A Rare Presentation of Tuberculoma

Deepak Rajendran, M. Jawahar, V. Nandakumar, B. Krishnakumar, R. Lakshmi Narasimhan, M. Jawahar, Lakshmi Narasimhan Ranganathan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drdeepakrajendran@gmail.com

Background and Aim: CNS tuberculoma can have different clinical manifestations like headache, seizures, papilloedema or other signs of raised intracranial pressure. Here we report the case of a 15 year old boy with past history of childhood tuberculosis presenting with bilateral ptosis. Our case report is unique in that bilateral ptosis as the sole manifestation of tuberculoma has been rarely reported in literature. Methodology: Case study. Results: 15 year old boy with past history of childhood tuberculosis presented with insidious onset bilateral ptosis of 1 month duration. There were no symptoms suggestive of other cranial nerve involvement , long tract signs and features suggesting raised ICT. Examination revealed bilateral symmetric ptosis with no other positive findings. Tests of fatigability were negative. MRI brain with IV contrast revealed peripheral ring enhancing T2 hyperintense lesion measuring 8.5 by 6.4mm in the dorsal midbrain with surrounding oedema causing mild extrinsic compression over the quadrigeminal plate, with no restricted diffusion, and was reported as possible granulomatous lesion. He was treated with steroids and ATT (antituberculous therapy). Following steroid therapy, his ptosis has resolved. Discussion: Bilateral ptosis is likely due to the involvement of caudal central subnucleus in the midbrain innervating levator palpebra superioris muscle without affecting fibers innervating superior recti. Antitubercular therapy (ATT) is indicated for a period of 12 – 18 months. Steroids are given if there is perilesional oedema causing neurological deficits. Conclusion: Bilateral symmetric ptosis without any other neurological symptoms or signs is a rare presentation of tuberculoma and it should be considered as a differential diagnosis of ptosis especially in endemic regions.

Abstract ID: 664

A Study on Clinical Profile of Patients Presenting as an Emergency with Movement Disorders to a Tertiary Care Center – A Preliminary Report

Chintha Sriram, Atanu Biswas, Arijit Roy, Adreesh Mukherjee

Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

E-mail: sriram16rocks@gmail.com

Background and Aim: Movement disorders often present as emergencies in the casualty ward and can be challenging to diagnose and manage due to their varied presentation and underlying etiologies. Emergency departments (EDs) are often the first point of contact for patients presenting with movement disorders. The clinical profile of these patients can vary widely, depending on the underlying condition. There is dearth of studies on pattern of various movement disorders observed in emergency. We studied the varied spectrum of movement disorders, their etiology and outcome in patients presenting as an emergency to our hospital. Methodology: This is an ongoing prospective, descriptive and observational single center study. Patients presenting as an emergency with movement disorders to our tertiary care center during the last 4 months were included in the study. Informed consent from the patient and Institutional ethical committee approval were taken. Result: 40 patients presented as an emergency with movement disorders during the study period. Thirteen (32.5%) patients were female. Mean (SD) age was 45.62 (+/-19.14) yrs with age range 5-78 years. Myoclonus (32.50%), Chorea (30%) were found to be the most common movement disorders in emergency. Seven patients were < 18yrs. Myoclonus was the most common movement disorders in emergency among them. Discussion: The most common etiology of myoclonus was found to be Metabolic. Commonest cause for Chorea was found to be Uncontrolled Diabetes (53.8%) and the control of glucose levels resulted in resolution of the movement disorders with 2 patients requiring symptomatic therapy as well. Conclusion: This study showed Myoclonus was the most common movement disorder presenting in the emergency. Early diagnosis, identifying the etiology and treating the reversible causes helped in better outcomes.

Abstract ID: 665

A Twist in the Search for Riboflavinopathy

Naresh Kumar, Ramesh, Satheesh Kamera

DM Neuro Pursuing, Other

E-mail: nareshserigudem@gmail.com

Background and Aim: Introduction : Madras Motor Neuron disease (MMND), Brown- Vialetto - Van Laere (BVVL) syndrome , Fazio-Londe syndrome (FLS) are rare motor neuron disorders of childhood closely resembling each other. BVVL is an autosomal recessive inherited disease caused by mutations in SLC52A3 , SLC52A1 and SLC52A2 which are intestinal riboflavin transporter genes, while MMND has no identifiable etiology. Methodology: Case report : A 25 yr old female presented with 16 years of insidious onset gradually progressive inability to close both the eyes with drooling of saliva, hearing loss, dysarthria, asymmetric distal weakness of the upper limbs, stiffness of the limbs associated with abnormal gait. On examination bilateral (b/l) LMN facial palsy, b/l SNHL, b/l tongue atrophy with fasciculations, distal predominant wasting of the both the upper limbs right > left, spasticity with exaggerated reflexes in the lower limbs with no signs of involvement of other cranial nerves, sensory system, autonomic system, cerebellum. Fundus examination was normal. Routine blood investigations were normal. Needle EMG showed Neurogenic pattern , MRI brain and spine were normal. Genetic analysis came negative for BVVL . Hence diagnosed as Madras Motor Neuron Disease. Results: -. Discussion: The Madras motor neuron disease (MMND) is characterized by wasting and weakness of limb muscles, sensorineural deafness and multiple cranial nerve palsies usually affecting cranial nerves VII, IX and XII. Dysfunction of cranial nerves III and VI has not been reported in MMND. BVVL is another closely related condition. Only about 15% of cases of MMND are familial, compared to 50% in BVVL The other most closely related condition is the progressive bulbar paralysis of Fazio-Londe, where the only distinguishing feature from BVVL is the absence of deafness. Conclusion: Differentiating MMND and BVVL is important as BVVL can be treated with high dose Riboflavin.

Abstract ID: 666

A Very Rare Type of Autosomal Dominant Spinocerebellar Ataxia Masquerading as Parkinsonism

Shishirakumar Goudar, Jagadish Agadi, Sujit Kumar, N. Pramod, S. Jayanth, M. Sindhu, Raja Kakollu

DNB Neuro Pursuing, Other

E-mail: shishir.a.goudar@gmail.com

Background and Aim: A 48 year old lady presented with insidious onset gradually progressive parkinsonism with ataxia of 2.5 years duration with autonomic disturbances. Methodology: Her clinical features, investigation details, genetic workup of the case will be presented. Result: Her clinical features, investigation details, genetic workup of the case will be presented. Discussion: A 48 year old lady presented with insidious onset gradually progressive parkinsonism with ataxia of 2.5 years duration with autonomic disturbances. To our knowledge not many such cases are reported from India. Conclusion: Autosomal Dominant spinocerebellar ataxia masquerading as parkinsonism.

Abstract ID: 667

Extensive Cerebral Venous Thrombosis, Subdural Hematoma and Bilateral Adrenal Haemorrhage – A Triptych of Complications due to Vaccine Induced Immune Thrombotic Thrombocytopenia

Shanaz Km, Fathima Hakeem, Boby Maramattom

MBBS completed, Other

E-mail: drshanaz@outlook.com

Background and Aim: Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a rare complication of ChAdOX1 (Oxford - AstraZeneca) vaccine. The incidence is reported to be one case per 26,500 to 127,3000 with first doses of ChAdOx1 nCoV-19 and one case per 518,181 with second doses of ChAdOx1 nCoV-19 administered. Methodology: . Results: A 41 year old woman with T2DM came with fever and throat pain for two days. No history of other symptoms. She received ChAdOX1 covid 19 vaccine one week back. Her initial blood investigations showed a platelet count of 39000/uL, d-Dimer of 5410 ng/mL and other inflammatory markers were also raised. Her covid 19 antigen test was positive. She developed severe abdominal pain on day two and CT abdomen taken showed adrenal haemorrhage. During evaluation for thrombocytopenia and its complications, she was found to have cerebral venous thrombosis. IVIg and anticoagulation was started with a provisional diagnosis of immune thrombotic thrombocytopenia. Patient developed seizures and was mechanically intubated and ventilated. Anti PF4 antibody test was found to be positive, hence a diagnosis of VITT was made in view of the clinical features and laboratory findings. She continued to develop extensive cerebral venous thrombosis along with pulmonary embolism, deep vein thrombosis and subdural haemorrhage. She underwent mechanical venous thrombectomy and middle meningeal artery embolization. Discussion- . Conclusion: Our case report demonstrates the rare but serious adverse reaction associated with ChAdOX1 vaccination like venous and arterial thrombosis with haemorrhage at multiple sites. The identification of VITT is by using American Society of Haematology criteria, in which anti PF4 antibody testing is the most suggestive one.

Abstract ID: 668

“Heart Appearance” Infarction of the Pons: A Case Report

Thahseen Sahubar Sadique

DM Neuro Pursuing, Other

E-mail: nilofarsahamed@gmail.com

Background and Aim: Heart appearance on magnetic resonance imaging is a unique presentation of bilateral medial medullary infarction. In contrast, “heart appearance” infarction of the pons has rarely been featured in the medical literature. In this paper, we present a case of “heart appearance” infarction of the pons with its MRI findings. Methodology: The patient was a 72-year-old female who presented with sudden onset fall and unresponsiveness soon after awakening. She was a hypertensive for 15 years on treatment. At admission, Patient’s GCS was 5; dolls eye movement was impaired; no anisocoria; plantars bilateral extensor. BP was 148/96 mm Hg. She needed supplemental O2 to maintain spo2 of 96%. Results: Sugars and electrolytes were normal. CT brain showed subtle hypodensity in left cerebellar hemisphere without any hydrocephalus. MRI Brain showed diffusion restriction in left cerebellar hemisphere and heart shaped restricted diffusion in pons (paramedian pons , tegmentum) with ADC correlation. MRA showed features of diffuse atherosclerosis of vertebral and basilar arteries. Discussion: Arterial territories involved were anterior inferior cerebellar artery, superior cerebellar artery and perforator territory of basilar artery. A diagnosis of acute bilateral pontine infarction with a “heart appearance” and left cerebellar infarct was made. Conclusion: For the heart sign to occur, there should be bilateral involvement of anteromedial and anterolateral territories, sparing the lateral territories. This may occur due to arterial disease involving bilateral paramedian and short circumferential branches and sparing of long circumferential branches.

Abstract ID: 669

“Shall I Also Become Dependant Like My Brother, Doc?” – Tale of three Siblings from the Himalayas

Subhadeep Banerjee, Ananya Sengupta, Avik Mukherjee, Anindya Sengupta, Jayanta Roy, Soutrik Das

DM Neuro Completed, Other

E-mail: subhadeepdr@gmail.com

Background and Aim: Nemaline myopathy is a rare disease with a reported prevalence of 1/50,000 live births. Severity is variable, with the most common genetic mutations involving NEB and ACTA1 genes. Autosomal recessive is the commonest mode of inheritance. We present a case of nemaline myopathy presenting in early adulthood with varying involvement in two siblings. Methodology: CLINICAL HISTORY: A 27 year old lady from Bhutan presented with slowly progressive pure motor quadriparesis since early childhood, proximal more than distal, along with truncal and neck weakness but without extraocular, facial or bulbar involvement, with no atrophy or fasciculations. Weakness started from lower limbs in childhood, while upper limb weakness started around 18 years of age. She had a younger brother who had become wheelchair bound by age 20, with quadriparesis and prominent atrophy of all four limbs along with facial weakness, while her 22 year old sister had only mild walking difficulty. On examination, the patient had global atrophy of shoulder and hip girdle muscles, with hammer toes and high arched foot without any facial dysmorphism, with normal muscle tone and preserved reflexes. Results: EMG showed myopathic pattern, with normal serum creatine kinase levels. MRI of thigh showed global atrophy. Muscle biopsy was suggestive of myopathy. Whole genome sequencing revealed compound heterozygous mutation in NEB gene. The patient is currently on a tailored rehabilitation program and doing well. Discussion: Congenital myopathies can have a wide variety of presentation. The clinical clue to narrow down the the differential in this case was the presence of significant atrophy and weakness of bilateral sternoclidomastoids muscle. Conclusion: Nemaline myopathy can have a wide variety of presenting symptoms and milder forms can even show minor progression in adult life.

Abstract ID: 670

Acute Cerebral Venous Thrombosis: A Rare Complication of Primary Varicella Zoster Virus Infection – A Case Report

S. Sangeethameena, J. Manickavasagam, G. Balaji, S. Hariharan

DM Neuro Pursuing, Other

E - mail: drsangeethameena@gmail.com

Background and Aim: Chickenpox is a benign illness caused by varicella-zoster virus, predominant in childhood. The illness presents with fever and characteristic exanthematous Vesicular skin rash. Though it is a self-limiting disease, occasionally, serious complications can occur. Neurological complications frequently encountered are cerebellar ataxia and encephalitis. Less frequent complications are Guillain–Barré syndrome, meningoencephalitis, transverse myelitis, aseptic meningitis, ventriculitis, optic neuritis, post-herpetic neuralgia, herpes zoster ophthalmicus, and peripheral motor neuropathy. Methodology: Case report. Results: CVT is a rare complication of Primary Varicella Zoster Virus Infection. Discussion: A 19-year-old female with a recent history of chickenpox, presented to us with a focal neurological deficit in the form of weakness of the right upper limb and lower limb with right UMN seventh nerve palsy and speech difficulty. She had two episodes of seizures, followed by altered sensorium and fluctuating behavioral symptoms. On examination, she had right hemiplegia and higher mental functions revealed sustained attention deficit and frontal lobe dysfunction with Neck stiffness. Imaging showed Cortical Venous thrombosis in both frontal lobes with hemorrhagic infarct in the left frontal lobe. She was treated with antiviral, anticoagulants, antiedema measures, anti-epileptics, and physiotherapy. Her CSF varicella IgM was positive. She improved well and was discharged with anticoagulants and anti-epileptics. Conclusion: Venous thrombosis following chickenpox is very rare. The exact pathogenesis of varicella Venous thrombosis is unknown, but similar to varicella zoster virus arteriopathy, activated varicella may migrate transaxonally to infect the meninges and Venous sinuses of the brain. The mechanisms underlying cerebral vascular events after VZV infection could be vasculitis, thrombosis due to direct endothelial damage, and acquired protein S deficiency. These patients require anti-viral treatment with IV Heparin and oral anticoagulants. With this case, we wish to add to the existing literature, that CVT is another neurological complication after varicella infection.

Abstract ID: 671

Acute Necrotizing Encephalopathy with RANBP Mutation: Report of Two Cases

Siddharth Chand, Karthik Vm, Ritu Shree, Manish Modi, Manoj Goyal, Vivek Lal

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

E-mail: siddharthchand@gmail.com

Background and Aim: Acute necrotizing encephalopathy (ANE) is a severe form of encephalopathy, precipitated after acute febrile illness of infectious etiology. ANE1 is a subset of ANE that is characterized by genetic origin with missense mutation in RANBP2 gene. Methodology: 23-year-old female presented with rapidly progressive encephalopathy after fever. On examination she has decerebrate rigidity, anisocoria, non-reacting pupils with absent doll’s eye and deep tendon reflexes. Her NS1 antigen for dengue was positive. MRI brain showed T2/FLAIR hyperintensities in bilateral basal ganglia, thalami, lentiform nuclei and medial temporal lobes with evidence of hemorrhagic necrosis in thalami. Possibility of dengue encephalitis was very strongly considered as NS1 antigen was positive. Other possibilities like acute hemorrhagic encephalomyelitis, adult onset Leigh disease, biotin responsive basal ganglia disease were kept. Her father had similar symptoms at 40 years of age with similar radiological findings. The possibility of genetic predisposition to ANE was considered and clinical exome sequencing was sent. She was pulsed with intravenous methylprednisolone 1 gram daily for 3 days and was also given immunoglobulin 2 gram/kg. Results: Clinical exome sequencing detected heterozygous mutation in c.1754C>T of RANBP2 gene. She improved gradually and currently wheelchair bound with significant deficit in the form of cerebellar signs and spasticity. Discussion: Missense mutation in RANBP2 has been reported to be associated with ANE1. The mutation predisposes to ANE1; however, an environmental trigger commonly a viral infection such as influenza A and B, parainfluenza, etc is essential for clinical manifestation. It is postulated that mutations in RANBP2 cause mitochondrial metabolic disorders or hyperinflammatory state leading to cytokine storm. Conclusion: ANE1 is a distinctive type of encephalopathy which is caused by genetic predisposition. ANE in adults has rarely been reported. The treatment options are limited, and further studies are needed for better outcome.

Abstract ID: 672

Adult Onset Vanishing White Matter Disease presenting with Spastic Hemiparesis

Devavrata Sahu, Bhupender Bajaj, Jyoti Garg, Kuljeet Anand

DM Neuro Pursuing, Other

E - mail: devsahu.90@gmail.com

Background and Aim: Vanishing white matter disease is an autosomal recessive inherited leukoencephalopathy. Adult form is rare and presents mainly with cerebellar ataxia, spastic paraparesis, and cognitive difficulties. This is a case of adult-onset vanishing white matter disease, presenting with progressive spastic hemiparesis. Methodology: A 32-year-old female patient presented with insidious onset, progressive spasticity of the left lower limb followed by the left upper limb, of 1½ years duration. She also had primary infertility, and had developed menopause at 19 years of age. Neurological examination showed spastic hemiparesis, and cognitive dysfunction involving the frontal, parietal, and temporal lobes. Results: With a picture of adult-onset insidious and progressive neurological dysfunction with primary infertility and premature ovarian failure, possibility of Vanishing White Matter disease was kept. MRI Brain showed changes suggestive of the disease. Genetic testing confirmed the patient to be a compound heterozygote for mutations in the EIF2B3 gene. Discussion: Vanishing white matter disease can affect people of all ages. It occurs due to mutations in the subunits of eukaryotic initiation factor 2B. The disease occurs more commonly in females. Initial manifestations in adults include seizures, presenile dementia, and psychiatric symptoms. Affected women can present with a combination of leukoencephalopathy and ovarian failure. Our patient remained undiagnosed as a case of Vanishing White Matter disease for 11 years, prior to onset of motor neurological symptoms. To the best of our knowledge, hemiparesis has not been reported previously as a manifestation of the disease in adults. One of the mutations noted in the EIF2B3 gene has been reported only once. Conclusion: Adult-onset Vanishing White Matter disease is rare. To date, presentation with hemiparesis has not been reported. The presence of ovarian dysfunction and infertility in a patient with cognitive decline may aid in diagnosis.

Abstract ID: 673

An Atypical Case of Neurobrucellosis preSenting as L5 S1 Disc Prolapse with Aseptic Meningitis, Ophthalmoplegia with Cerebral Venous Sinus Thrombosis Leading to Fulminant Intracranial Hypertension Causing Bilateral Vision Loss

Prince Kumar, Rahul Agarwal, Arunav Garg, Arunav Garg, Neera Chaudhry

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi, India

E-mail: prince.agr90@gmail.com

Background and Aim: A young girl presented with intermittent low-grade fever with anorexia for 3 months with L5 S1 radiculopathy and 2 months later developed secondary headache with ophthalmoplegia followed by rapid diminution of vision in both eyes. Methodology: After an informed consent, the patient underwent detailed history taking with clinical examination and relevant investigations. Results: She was diagnosed clinically with acute meningitis and intracranial hypertension with involvement of 3,4, and 6th cranial nerve on the left side and 6th on the right side. CSF examination revealed raised opening pressure. MRI brain was normal. MRV showed bilateral sigmoid sinus thrombosis. After ruling out other possible etiologies, she was worked up for Brucellosis. Brucella tube agglutination test and IgM antibody were also positive. She was managed with antibiotics ( IV Ceftriaxone, PO Rifampicin, and PO Doxycycline) with SC heparin followed by oral rivaroxaban and pulse steroid followed by rapid tapering in view of vision loss. She partially responded to the treatment and is being followed up. Discussion: Brucellosis is a chronic granulomatous infection. The most common complication of Brucellosis is in the form of osteoarticular involvement. The patient presenting with disc herniations has been reported very rarely. Nervous system involvement may also be seen in 5-7 percent of cases. Meningitis has been most commonly reported, however, cases of aseptic meningitis are rarely reported. Cases of cerebral venous thrombosis have been reported in neurobrucellosis. There have been a few case reports of vision loss due to raised ICP. Vestibulocochlear nerve involvement is frequently involved in causing sensorineural hearing loss. In our patient, 3,4, and 6th cranial nerve was involved and the 8th cranial nerve was not involved. Conclusion: Clinicians should be aware of neurobrucellosis, and consider workup when a patient presents with signs and symptoms involving multiple axes with systemic involvement and common causes are ruled out.

Abstract ID: 674

An Atypical Presentation of Tangier’s Disease – Expanding the Clinical Spectrum

Rohin Dubbal, Seena Vengalil, Saraswathi Nashi, Deepak Menon, A. Nalini, Nandheesh Bn, Dipti Bhaskar, Aneesha Thomas

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: rohin.dubbal@gmail.com

Background and Aim: Tangier’s disease is a rare autosomal recessive metabolic disorder characterized by abnormal lipid metabolism and low levels of high-density lipoprotein (HDL-C). Neurological manifestations primarily involve peripheral neuropathy, but anterior horn cell (AHC) involvement is rare. This case report aims to describe a novel phenotype of Tangier’s disease with predominant AHC involvement. Methodology: A 16-year-old girl presented with progressive weakness and atrophy of the right leg, waddling gait, and characteristic dysmorphic features. Clinical examination, laboratory investigations, nerve conduction studies, electromyography, and genetic testing were performed to confirm the diagnosis. Whole exome sequencing identified a novel likely pathogenic mutation in the ABCA1 gene. Results: The patient exhibited orange tonsils, dyslipidemia, and asymmetric weakness and atrophy of the lower limbs. Nerve conduction studies and electromyography indicated a neurogenic pattern, while muscle MRI revealed fatty infiltration. Genetic testing by whole exome sequencing confirmed a homozygous likely pathogenic mutation at c.2542+1 G>A, in the ABCA1 gene identified at intron 17, which is a novel variant. reported in our patient which is also a novel variant. Discussion: Tangier’s disease commonly presents with peripheral neuropathy, but this case highlights a unique phenotype with AHC involvement. The underlying pathogenesis involves ABCA1 deficiency and cholesterol ester accumulation in muscle fibrils and Schwann cells. Autopsy studies have previously shown AHC involvement due to metabolic defects in Tangier’s disease. The presence of orange tonsils and dyslipidemia aided in suspecting the diagnosis. Conclusion: This case expands the clinical spectrum of Tangier’s disease by demonstrating AHC involvement. A high index of suspicion, along with genetic testing and lipid profile assessment, is essential for accurate diagnosis. As there is no specific treatment, management primarily focuses on screening for and managing vascular complications.

Abstract ID: 675

An Interesting Case of a Rare Photosensitive Epilepsy: Sunflower Syndrome

B. Thejus, Archana Netto, Praveen Kumar, D. C. Janardhan, K. Pramod

Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India

E-mail: apunkrockangel@gmail.com

Background and Aim: Sunflower syndrome is a rare photosensitive epilepsy characterized by highly stereotyped seizures. Patients with Sunflower syndrome look toward a light source, often the sun, and wave one hand with abducted fingers in front of their face. Often, there is associated eye fluttering or blinking during these episodes. We report a case of a 7 year old female with sunflower syndrome. Methodology: We report a case of sunflower syndrome in a 7 year old female. Results: A 7 year old female presented with history of hand waving in bright sunlight , patient was unresponsive to commands when instructed not to do hand waving actions during such episodes and also appeared to have impaired awareness of surroundings and people around her during such episodes , patient was born out of consanguineous parentage and had no similar history among siblings , on examination patient had no focal neurological abnormality , EEG [interictal] showed generalized spike and wave discharges with photosensitivity , MRI Brain [epilepsy protocol] was unremarkable. Discussion: The patient had features consistent with sunflower syndrome and was started on syrup sodium valproate and was prescribed protective eyewear with photoprotective filters following which clinically the seizure episodes stopped. Conclusion: A high index of suspicion should be present when young patients between the ages of 3 to 10 years with female gender have specific gestures such as hand waving to be attributable to an epilepsy syndrome especially when it occurs in sunlight which may suggest heliotropism and sunflower syndrome.

Abstract ID: 676

An Interesting Case of Auditory Agnosia

Sreenath Nair

DM Neuro Pursuing, Other

E-mail: dr.sreenathgnair@gmail.com

Background and Aim: Auditory agnosia is a rare otoneurological manifestation that refers to the defective recognition of auditory stimuli in the context of preserved hearing and language functions. It is divided into (1)pure word deafness (2)Pure auditory nonverbal agnosias (3) Phonagnosia and (4) Pure amusia. Methodology: 33 year old male , with past history of provoked DVT and dilated cardiomyopathy has presented with recurrent stroke. First episode 1 year back presented as altered sensorium, features of frontal syndrome and deafness to words not to sounds. There was partial recovery with respects to the frontal syndrome but auditory agnosia persisted. Second presentation 6 months later as acute psychosis,hallucinations,religious ecstasy and he had partial recovery , 3rd episode with transient dysarthria and paraphasia ,4th with palilalia and paraphasia . Currently patient has palilalia, complex calculation difficulty , agraphasthesia and astereognosis on left side and general auditory agnosia with intact lip reading.Reading and writing normal. Tyere was evidence of bipyramidal involvment. CT brain showed chronic infarct in bilateral parieto-temporal region. MRI deferred due to intramedullary nail insitu in left leg .Hearing assessment including audiogram, inner ear test and BERA were normal. Echocardiogram showed left ventricular clot. Results: The patient was diagnosed as recurrent cardioembolic stroke with general auditory agnosia.He was given standard treatment for stroke along with speech therapy and auditory stimulation therapies. Patient is under follow up. Discussion: Auditory agnosia commonly caused by bilateral temporal cortex infarcts but it can also caused by unilateral involvement also. Conclusion: Bilateral involvement of temporal lobes can cause auditory agnosia in patients who develop stroke, especially in cardioembolic origin. The clinical diagnosis of auditory agnosia may be missed or misdiagnosed . Close observation and variable evaluation tools of cognition, speech and auditory function are important in exact diagnosis and treatment of auditory agnosia in stroke patients.

Abstract ID: 677

An Interesting Case of Dancing Eyes

R. Harini

DM Neuro Pursuing, Other

E-mail: harini87@gmail.com

Background and Aim: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is an inflammatory disease of the central nervous system (CNS) characterized by attacks of immune-mediated demyelination. We wish to present a case of possible MOGAD presenting like recurrent ADEM, the last episode presenting with opsoclonus myoclonus ataxia. Methodology: A 23 year old male presented with a remitting relapsing neurological illness of two year duration, with the first episode presenting as seizures, second episode presenting with cognitive impairment, bradykinesia and urinary incontinence; third episode presenting as involuntary ocular movements; fourth episode presenting with mania, urinary urgency and the fifth and the most recent episode presenting with increased ocular involuntary movements, intentional tremors and gait ataxia. On examination, he had opsoclonus. He had bipyramidal and bilateral cerebellar signs. Gait was spastic ataxic. He had occasional myoclonic jerks and bradykinesia. Index MRI had shown a butterfly shaped T2/ FLAIR hyperintense lesion in Genu, body of corpus callosum and bilateral frontal parafalcine subcortical white matter. He was given steroids during the first event. Subsequent MRIs showed resolution of lesions. Patient was not compliant with medications. MRI during current episode showed gliosis in the right frontal and basi-temporal lobes and ex-vacuo dilation of ventricles. Results: Patient was likely to have cerebral poly-focal deficits like presentation of MOGAD. Although anti-MOG antibody was negative, he was given pulse methylprednisolone and oral prednisolone was continued. His opsoclonus and ataxia reduced drastically with steroids. Discussion: This patient had cerebral polyfocal deficits which can be a presentation of MOGAD. It was a unique case as none of the attacks involved optic nerve or spinal cord. Resolving lesions on MRI and dramatic response to steroids favoured our diagnosis of central demyelination. Conclusion: MOGAD can present like a multiphasic ADEM and a timely diagnosis is important as it responds well to steroids.

Abstract ID: 678

An Interesting Case of Dermatomyositis

M. Archana

DM Neuro Pursuing, Other

E-mail: archanamkiran@gmail.com

Background and Aim: Dermatomyositis is an idiopathic inflammatory myopathy involving predominantly proximal muscle weakness and characteristic skin rash. Classification of dermatomyositis for a definitive diagnosis requires characteristic rash and other criteria’s like proximal muscle weakness and muscle enzyme level elevation. Muscle biopsy helps in establishing the diagnosis. Methodology: We would like to present the case of a 30 year old female, who presented with insidious onset gradually progressive neurological illness, in the form of dysphagia for both solids and liquids* 1.5 months, drooling of saliva and pooling of secretions in mouth*1.5 months, proximal upper limb and lower limb weakness, with generalized myalgia with no evidence of higher mental function, Cranial nerves, sensory, bowel and bladder, autonomic, cerebellar or raised ICT features. Results: O/E: Frontal balding, Hyperpigmented lesions over dorsal interphalangeal joints (Gottron’s papules),and over nape of neck, upper back &chest, ,Generalized wasting+ ,HMF- normal, Speech - Hypophonic with nasal twang, Cranial nerves:7- minimal deviation of angle of mouth to right,9,10 -Nasal twang of voice, palatal movements normal. Motor: Right winging of scapula+ Inferior border of scapula deviates medially Bulk- generalized wasting, proximal muscle weakness of both upper and lower limbs,, DTR - absent bilaterally, No sensory ,cerebellar complaints,gait- Waddling gait. Discussion: Investigations - ESR-80mm/1 h. Urine analysis, renal function tests ,liver function tests and blood glucose were normal. Creatinine phosphokinase-6564 IU/L , S. lactate dehydrogenase (LDH)-1468 IU/L, ANA- neg ,thyroid function tests were normal. Chest X-ray, ECG, 2D echocardiography, CT chest, ultrasound abdomen and pelvis were normal. Electromyography (EMG) showed early and complete recruitment with polyphasic and low-amplitude motor unit action potential, suggestive of myopathic process. Muscle biopsy showed skeletal muscle fibres with scattered degenerative changes and perivascular collections of lymphocytes .A provisional diagnosis of dermatomyositis was made. Conclusion: Patient was started on prednisolone 60mg/day,following which there was a marked improvement in weakness at the end of 3 weeks.

Abstract ID: 679

An Interesting case of Diplopia with Vision Loss

Margeret Aishwarya, Kingsly Jeba Singh, A. Sethuram, Shankar Ganesh

Pursuing MD Medicine, Other

E-mail: draish87@gmail.com

Background and Aim: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune, Astro cytopathic CNS disease, mainly involving the optic nerves, spinal cord and brain stem regions. NMOSD constitutes Aquaporin 4 antibody disease (9:1) and MOG antibody disease. Methodology: Case Discussion : A 21-year-old female presented with subacute onset drooping of both eye lids (right > left) with restricted eye movements and diplopia. Also associated with diminution of vision and colour desaturation. No history of limb weakness and sensory loss. On examination, there was bilateral internuclear ophthalmoplegia (INO) and bilateral ptosis ( Right > left ) , bilateral optic neuritis with normal motor and sensory system. Results: Investigations : Considering ocular myasthenia , Ice pack test was done – negative. Serological tests for Viral markers were negative. CSF – Aquaporin4 Ab was positive. MRI Brain was suggestive of bilateral optic nerve and mid brain demyelination – favouring NMO. VEP showed marked prolongation of P100 latencies in both eyes ( right > left ). NCS of limbs was normal. As per IPND criteria , our patient was fitting into Aquaporin4 Ab positive NMO disorder. Patient was treated with iv methyl prednisolone pulse therapy followed by immunosuppressants. Patient recovered well and discharged. Discussion: Our case was unique, as there was additional involvement of bilateral 3rd nerve ( ptosis ) along with bilateral INO & optic neuritis without pyramidal signs. Conclusion: NMOSD has wide range of presentations, like in our case which presented with ptosis, diplopia , bilateral INO and bilateral optic neuritis sparing the pyramidal tracts.

Abstract ID: 680

An Interesting Case of Flail Paraparesis Masquerader

Ajith Thampi

DM Neuro Pursuing, Other

E-mail: drajithdavidthampi@gmail.com

Background and Aim: 37 year old female, with significant past h/o CAD, newly detected Diabetes mellitus, presented with acute onset progressive neurological illness, in the form of asymmetric onset flail weakness of both lower limbs for 3 days, left> right, symptoms suggestive of acute lower limb ischemia, left > right with no involvement of HMF, cranial nerves, bowel and bladder, posterior column, cerebellar or features of raised ICT .She also had breathlessness which was worsening since 1 day. O/E:Left lower limb above knee amputated and femoral pulse was not felt Right lower limb- femoral pulse, posterior tibial, dorsalis pedis feeble HMF, CN - Normal Motor- tone decreased in right lower limb. Power- both upper limbs 5 Right lower limb 1 Left lower limb - not tested DTR -absent in both lower limbs. UL - Normal reflexes Sensory- normal. Methodology: Investigations: D dimer -1247,CK, CK MB - elevated, INR- elevated CT LL angio- complete occlusion of abdominal aorta in infra renal level before bifurcation. R. external iliac occlusion B/l common iliac artery occlusion at the origin CT Chest-peribronchovascular consolidation with interstitial thickening. Results: Treatment given - Bilateral transfemoral embolectomy heparin, dual antiplatelets, Acitrom, Cilastazole. Discussion: Diagnosis: Acute paraperesis , Acute lower limb Ischemia ? secondary to cardio embolic etiology. Motor and reflex level L. Conclusion: Discussion: This patient was brought to ER with progressive weakness and breathlessness. Examination revealed a flail weakness .She was initially suspected as GBS outside and was referred for ivig to our medical college. However the absence of femoral pulses in let lower limb and feeble right femoral pulse, posterior tibial, dorsalis pedis made us suspect paraparesis due to ischemia. CT chest for breathlessness showed pulmonary embolism and patient improved with medical management. Bilateral transfemoral embolectomy was done and patient was discharged in a stable condition.

Abstract ID: 681

An Interesting Case of Opthalmoplegic Neuropathy

Padmapriya Sakthivel, N. Shobana, V. Sadeeshkumar, C. J. Selvakumar, M. Joby

DM Neuro Pursuing, Other

E-mail: spriya.mimi@gmail.com

Background and Aim: Opthalmoplegic migraine described as migraine with oculomotor nerve palsy.Opthalmoplegic migraine was replaced by recurrent painful Opthalmoplegic neuropathy in ICHD 3 based on headache, subsequent cranial neuropathies,MRI features and the fact that cases show response to steroid treatment. Methodology: 19 years male presented to our hospital with complaints of drooping of right eyelid and double vision for 2 days.Before adminission he had been experiencing Migraine for past 3 years then concurrent oculomotor paralysis for one month .Was improved of symptoms over weeks with IV steroids There is no family history of migraine. General examination was normal.Neurological examination revealed complete paralysis of right oculomotor nerve .Serology and CerebroSpinal Fluid analysis were normal .MRI Brain with contrast shows no significant abnormalities. Results: Identifying and early treatment allows better outcome in Opthalmoplegic neuropathy. Discussion: He had his second attack on 3 months later the first attack at his 14 years of age .and presented with same complaints of headache, moderate to severe intensity, right sided headache with conjunctival injection followed by drooping of right eye .Examination showed right third cranial nerve palsy .other ranial nerve examination normal. Investigations done normal. Patient was treated with Intravenous steroids and improved of symptoms in 2 weeks .He had his third attack at 15 years of age in 2019. He presented with right eye pain,throbbing type moderate to severe intensity associated with right sided headache .No history of any aggravating factors,releived by medications following which he developed drooping of right eye .At the age of 18 he had his 4th attack with similar complaints and treated with Intravenous steroids and improved of symptoms over 3 weeks Presently on follow up and is symptom free for past 6 months . Conclusion: Initially Opthalmoplegic migraine now replaced by recurrent Opthalmoplegic neuropathy is presented for its rare and complete resoulution of symptoms with steroids suggesting demyelination as etiology.

Abstract ID: 682

An Interesting Case of Painful Ophthalmoplegia

R. Harini, S. Saravanan

DM Neuro Pursuing, Other

E-mail: harini87@gmail.com

Background and Aim: Tolosa-Hunt syndrome is a rare syndrome with an estimated annual incidence of one case per million per year. It is characterized by painful ophthalmoplegia and is caused by an idiopathic granulomatous inflammation of the cavernous sinus. We report a case of a middle aged lady presenting with painful ophthalmoplegia. Methodology: A 40 year old female presented with a three month history of neurological illness which started as right sided headache and right peri-orbital pain. One month later she developed diplopia and numbness on the right side of forehead. Two weeks later, she developed drooping of the right eyelid. She had no fever. On examination, she had complete ophthalmoplegia of the right eye. Left eye was normal. She had decreased facial sensations on V1 and V2 distribution of the right trigeminal nerve. Results: MRI Brain showed a prominent cavernous sinus on the right side. She was started on high dose steroids. Within a week, patient showed signs of improvement. She was discharged with slow tapering dose of steroids and will be on close follow up. Discussion: In our case, after excluding other causes of painful ophthalmoplegia like aneurysms, tumours, infections and autoimmune causes, we considered Tolosa hunt syndrome and started our patient on steroids, to which she responded well. Conclusion: Tolosa hunt syndrome, though rare, is a disease warranting timely diagnosis as patient can have resolution of symptoms with steroids.

Abstract ID: 683

An Interesting Case of Tremor

R. Gopi Krishnan

MBBS completed, Other

E-mail: gkr.619@gmail.com

Background and Aim: An interesting case of tremor. Methodology: After informed consent, detailed history was taken, thoroughly examined. Relevant investigations were done. Imaging of brain , spinal cord was taken. Results: A 58 yr old male with no comorbidities presented with insidious onset progressive rubral tremor, palatal myoclonus with left cerebellar signs with metastatic lesion in left cerebellar hemisphere and left frontal lobe. Discussion: Cerebellar metastasis are considered negative prognostic factor in patients with systemic cancers. Usually the primary site is from lung, breast or GIT. A patient with palatal myoclonus ,rubral tremor is due to the lesion involving guillian mollaret triangle affecting the connection between red nucleus, ipsilateral inferior olivary nucleus and contralateral dentate nucleus. It is also seen after few months of initial insult like posterior fossa surgery, brainstem strokes. These leads to hypertrophic olivary degeneration. Conclusion: Lesion involving guillian mollaret triangle can be due to tumor per se or an old insult like stroke/ surgery leading to hypertrophic olivary degeneration.

Abstract ID: 684

An Interesting Case of Vision Loss in an Adolescent Girl

Arvind Visweshwar, Manivannan, P. K. Murugan, Birla Pavalam, Chezhian, Justin, Ramu, Subramanian

DM Neuro Pursuing, Other

E-mail: dr.arvind.visweshwar@gmail.com

Background and Aim: Extramedullary haematopoiesis is a compensatory proliferation of haematopoietic element outside the bone marrow because of insufficient bone marrow erythropoiesis. Compressive optic neuropathy is an extremely rare complication. Here we present a case of visual impairment because of EMH in a patient with beta thalassemia. Methodology: A 16-year-old Saurashtra origin female born of 2-degree consanguineous marriage presented with history of headache and slowly progressive painless vision loss. On examination she was short statured with, macrocephaly, fundus examination showed bilateral pale optic disc with RAPD on right side. Per abdomen examination revealed hepatosplenomegaly which was later confirmed by ultrasound and CT BRAIN showed features of extramedullary haematopoiesis. Orbital CT showed significant enlargement of diplopic space of sphenoid bone resulting in bilateral narrow optic canals. Later patient revealed to have received multiple blood transfusion from the age of 5. Results: Blood investigations showed normocytic hypochromic anaemia with Mentzer index of 12 and anisopoikilocytosis. Peripheral smear showed presence of target cells and Beta thalassemia was confirmed by HPLC. Patient was subjected to repeated blood transfusions and was followed up for 6 months and subsequently reported improvement in vision with periodic blood transfusions. Discussion: Although compressive optic neuropathy secondary to extramedullary haematopoiesis is extremely rare. Beta thalassemia or thalassemia intermedia patients should have periodic comprehensive ophthalmic assessment especially those with inadequate blood transfusions. Conclusion: Favourable visual outcome can be achieved with early recognition and prompt management with blood transfusions combined with low-dose radiotherapy.

Abstract ID: 685

An Unusual Cause of Seizure in a 2 Year Old Child

Venkatesh Kondle, M. R. Manivannan, P. K. Murugan, C. Justin

DM Neuro Pursuing, Other

E-mail: venkatesh.kondle@gmail.com

Background and Aim: Glycogen storage disease IX characterized by phosphorylase kinase deficiency which is necessary to breakdown glycogen. Individuals with GSD-IX cannot break down glycogen, excess amounts accumulate in liver, muscles. GSD-IX is categorized into liver and muscle form. Methodology: 2 year old male child, born out of consanguineous marriage , first in birth order, normal delivery cried immediately; no perinatal asphyxia; with motor developmental delay presented with seizures (GTCS) frequently occurring in early mornings between 5-6 am; 4 months back he had first episode, labelled elsewhere as unprovoked seizures, started on AEDs; On examination there is doll like facies, hypotonia, enlarged liver and spleen. Results: In hospital child had similar early morning seizure, hypoglycemia was documented during the episode; routine tests showed mild transaminitis, mild anaemia; Hb Electrophoresis was done which is normal, MRI brain didnot show any structural lesion. Discussion: Considering growth-delay, hypotonia, organomegaly, we suspected an underlying GSD and genetic studies done, which revealed mutation in PHKG2 gene on short arm(p) of chromosome 16, that is suggestive of GSD-9C. Conclusion: GSD 9c must be included in differential diagnosis who present with growth delay, organomegaly, seizures after prolonged fasting; this case is of particular interest because of novel mutation in PHKG2 which is very rare.

Abstract ID: 686

Analysis of Barthel Index and Care giver Strain Index in Stroke Patients in Tertiary Care Hospital in South India

Hrishikesh Pai, B. Kannan, Thomas Raj, Soumya Das

DM Neuro Pursuing, Other

E-mail: hrishikeshhrishi30@gmail.com

Background and Aim: Stroke patients have high rate of mortality and morbidity and survivors suffer from physical,cognitive and emotional issues.Quality of life of stroke survivors depends on severity of disability.These disability or morbidities not only affect stroke survivors but also has impact on physical and psychological wellbeing of caregivers providing support for daily routine.Aim:Analyse the Barthel Index in Stroke patients and Care giver strain (CSI)among caregivers. Methodology: A Cross sectional descriptive study in a tertiary care hospital.Study duration of 5 months (July 2022-Nov 2022).150 Patients admitted for stroke meeting the Inclusion criteria were included for the study.Demographic profile including age,sex,medical history,risk factors,relationship with care giver,type and duration of stroke was noted.Berthel Index and Care Giver strain index questionnaire was filled by Interviewer with Caregiver.Findings were described in simple descriptive manner.SPSS version 24 was used for statistical analysis. Results: 150 patients male 64%Female 36%.Ischemic stroke 65% Hemorrhagic 35%.Mean age of the patient 62.25+/-6.31yrs.Patients age group<40yrs 3%,41-60yrs 45%,61-80yrs 51%,>80yrs 1%.Care giver included male 38% female 62%.Care giver strain Score of <7 (40%),8-14(42%),>14(18%).Sleep deprivation 68%,Physical strain 85%,family adjustment problems 32%,work adjustment problems 67%,financial strain 76%,Emotional adjustment problems 33%,personal plans problems 44%,upsetting behavior problems 44%.17% termed it as Inconvenient,24% completely overwhelmed,29% Confining.Barthel Index of>80 (13%),61-80 (32%),41-60 (26%),21-40 (13%),<20 (16%). Discussion: Care giver strain index more than 14 was found in 18%.Major cause of stress being physical strain,sleep deprivation,financial strain.Nearly 30% patients were dependent fully on caregivers for routine activities.Stress including emotional,psychological was more in this group compare to 13% with Barthel Index more than 80 who were able to their daily activities. Conclusion: In stroke along with care of patients physical and psychological issues among caregivers also need to be considered.Their apprehensions need to be addressed and need for proper counselling regarding prognosis and further management need to be taken to help them to adjust to the situation.

Abstract ID: 687

Anticoagulant Induced Spinal Epidural Hematoma with Spontaneous Recovery

S. Haritha, S. Lakshmanan, P. R. Sowmini, K. Mugundhan

DM Neuro Pursuing, Other

E-mail: haritha199212@gmail.com

Background and Aim: Spinal epidural hematoma is a rare cause of compressive myelopathy, of which anticoagulant induced bleed account for less than 11.8% of the cases of spinal epidural hematoma, the incidence of which is more nowadays with the advent of MRI. Methodology: A 72 year old gentleman, known case of rheumatic heart disease, on acenocoumarin, presented with sudden onset of tight band like sensation at the level of upper back, weakness and numbness of both lower limbs and acute retention of urine. On examination he had 0/5 power in both lower limbs, with absent deep tendon reflexes, and decreased perception of all modalities of sensations below L1. Results: MRI thoracolumbar spine revealed spinal epidural hematoma from D3 to D8 level. His INR was 9.21. He was treated with Injection Vitamin K stat dose, following which he was advised emergency surgery. Patient was not willing for surgery, hence surgery was deferred. But patient clinically improved with symptomatic and supportive management. He had complete improvement of symptoms. He was discharged in a fully recovered state. Repeat MRI done one month later revealed decrease in the size and extent of hematoma. Discussion: Spinal epidural hematoma is a rare condition that accounts for less than 1 % of the causes of spinal epidural space occupying lesions. It can occur at any level, but is most common at the level of thoracic and cervical spinal cord. Conclusion: Although spinal epidural hematoma is a rare clinical entity, it should be considered among the differential diagnosis for compressive myelopathy as it a surgical emergency, which is associated with significant morbidity of left untreated. However, in the presence of early recovery over the first few hours or continuing recovery patients may be kept under observation for worsening before proceeding with surgery, as in our case, hence avoiding a major surgery in such patients.

Abstract ID: 688

Arsenic Intoxication Presenting as Guillain-Barré Syndrome

Sunderka Meet, P. K. Maurya, A. K. Singh, Dinkar Kulshreshtha, Abdul Qavi, Vijay Varman, Anand Singh

DM Neuro Pursuing, Other

E-mail: meetsunderka@gmail.com

Background and Aim: Acute poisoning from inorganic arsenic in humans affects multiple organs.Neurological symptoms of acute intoxication include peripheral neuropathy mimicking Guillain-Barre syndrome, light headedness, delirium, encephalopathy and muscle weakness or cramping.The most common presentation includes a distal, symmetrical, sensorimotor axonal polyneuropathy often misdiagnosed at initial presentation. We report a case of acute arsenic intoxication presenting as GBS. Methodology: We report the case of a 43 year old male presenting with acute onset sensorimotor areflexic predominantly distal quadriparesis of 4 days duration without bowel bladder involvement without sensory level . This was preceded by prolong diarrhoea of about 1 week. Motor examination revealed power of 2/5 in all 4 limbs. GBS disability score was 4. Nerve conduction study on admission demonstrated axonal sensorimotor polyneuropathy. CSF examination was normal. Possibility of GBS was kept .IVIG was initiated but symptoms worsened and patient got readmitted after 2 weeks.Repeat course of IVIG was given but there was no improvement. A short course of steroids was also given with no improvement.There was a history of multiple herbal medication use. Hyperkeratotic skin lesions and mees lines on nails appeared during hospital admission. Results: Patient was investigated thoroughly. Secondary causes were ruled out.. However,serum toxicology panel demonstrated high arsenic levels (2190 micrograms/L, normal<50). A 24 hr urine arsenic levels were also found to be elevated ( 138.81 micrograms,normal<50) and confirmed the diagnosis of arsenic neuropathy.The patient was treated symptomatically with neuropathic agents, antioxidants and physiotherapy. Chelation was done with penicillamine as patient was found to be sensitive to BAL. He was able to walk with support (GBS disability score 3) at 3 month follow up. Discussion: Arsenic neuropathy may present as GBS and may often be misdiagnosed initially.It must not be overlooked especially in patients with history of herbal medication use.Chelation with penicillamine has been found to be effective and efficacy of treatment should be monitored clinically and biochemically with urinary arsenic levels. Conclusion: The possibility of arsenic neuropathy mimicking GBS should be considered in patients with acute or subacute peripheral neuropathy of undetermined etiology and history of herbal medication use.

Abstract ID: 689

Assessment of Bladder Dysfunction in Neurological Disorders

Naman Agrawal, Samhita Panda, S. Bhaskar, Taruna Yadav, Sarbesh Tiwari, Deepak Bhirud

All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

E-mail: naman.mack91@gmail.com

Background and Aim: There is paucity of bladder symptom questionnaires that assess nature of neurogenic bladder. Though Neurogenic bladder symptom questionnaire (NBSS) demonstrated good validity and reliability in neurological disorder patients, more reliable questionnaire is needed to localize, assess the level of neurogenic bladder. This study was planned to assess neurogenic bladder dysfunction based on clinical, laboratory and urodynamic evaluation with aim to create a questionnaire. Methodology: This prospective observational study was conducted from July 2021 to December 2022 after seeking approval from the Institutional Ethics Committee. Patients above 18 years with non-obstructive bladder dysfunction due to neurological disorders were included after written informed consent. Detailed clinical and bladder history, examination findings were collected and NBSS was administered. Urine routine-microscopy, culture sensitivity, and radiological evaluation was done. Urodynamic studies (UDS) were performed in selected patients. Results: 72 patients were enrolled with mean age- 48.8 years (70.83% male and 29.16% female). Neurological disorders were extramedullary spinal cord lesions (40%), demyelinating disorders (23%), parkinsonian disorders (17%), dementia (12.5%) and others (17%). Clinically, 84%, 11% and 6% had UMN bladder lesion, LMN type and UMN bladder in shock, respectively. Bladder involvement was localised to frontal in 12%, suprapontine (35%), suprasacral (37.5%), and sacral/peripheral (12%). Radiological diagnosis was concordant with clinical bladder localization in 87%. Discussion: History and examination of neurological and bladder symptoms as elicited in our study is strongly concordant with actual level of localization along the neuraxis. Fairly good degree of consistency was observed using radiological and laboratory evaluation to confirm level of involvement. On other hand, NBSS only helps in evaluating severity and complications associated with bladder dysfunction. Conclusion: This study has demonstrated high degree of consistency of bladder-related symptom-based hypothesis regarding level of involvement in the neuraxis with final diagnostic localization. This information can be now used to create bladder-specific questionnaire for use in neurological patients.

Abstract ID: 690

Ataxia-Telangiectasia in a Family of Three Daughters_Identical Twins and Their Elder Sibling: A Case Report

Thahseen Sahubar Sadique, S. Veerappan, S. Saravanan

DM Neuro Pursuing, Other

E-mail: nilofarsahamed@gmail.com

Background and Aim: Ataxia-telangiectasia is multisystem, complex disorder characterised by progressive cerebellar ataxia and cutaneous telangiectasia since childhood. The incidence is about 1 in 100,000 live birth. Males and females are equally affected and there are no racial or regional preferences. Immune dysfunction and lymphoreticular malignancies are frequent accompaniments. Methodology: Our index case is a13 year old girl, first born to 2nd degree consanguineous parents, brought with complaints of unsteady gait and progressive slurring of speech since childhood. She performed above average in school and had recently attained menarche. On examination, child had bilateral pan-cerebellar signs including nystagmus, scanning dysarthria, symmetrical appendicular dysmetria of all 4 limbs, stance and gait ataxia, and impaired tandem gait. She had oculomotor apraxia and bilateral bulbar conjunctival telangiectasias. Neuroimaging showed profound cerebellar atrophy. Serum alpha fetoprotein was elevated. Results: She had younger, identical twin sisters who were 8 years old. Parents denied any complaints in the twins. The children came to us upon repeated request to the parents. Discussion: On examination, both of them had bilateral mild pan-cerebellar signs and bulbar conjunctival telangiectasias; One had subtle upper limb dystonia. They performed average in school. In all three children, diagnosis of AT was made based on Diagnostic Criteria formulated by Ataxia-Telangiectasia Clinical Center at the Johns Hopkins Medical Institutions. Conclusion: AT is mainly a clinical diagnosis and costly investigations can be avoided. Management includes genetic counseling, examination of all the family members, regular surveillance of the heterozygote for malignancy, symptomatic management of immune dysfunction, other supportive therapies.

Abstract ID: 691

Atypical Presentation of Cidp- A Case Report

Supraja Vasu, Neha Rai, Js Kathpal, Dhanraj Panjwani, Shraddha Shrivastava

DNB Neuro Pursuing, Other

E-mail: supsvasu24@gmail.com

Background and Aim: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired, immune-mediated neuropathy affecting peripheral nerves and nerve roots, characterized by a relapsing-remitting or progressive course of symmetric weakness of proximal and distal muscles that exceeds the extent of sensory loss. Here we elaborate upon an atypical presentation of the same. Methodology: A 43 year old female, with no known comorbidities presented with sudden onset of weakness in her right upper limb-expressed as difficulty in gripping objects in her hand since 7 days. On further probing she revealed history of tingling and numbness over both her soles and palms since the past seven years which was insidious in onset, static in nature and thus ignored by the patient. Examination revealed right handed weakness of thenar, hypothenar and intrinsic muscles, absent Biceps and Knee reflexes bilaterally and no sensory deficit. On workup her HbA1c was found to be 8.6 diagnosing her with newly detected Diabetes, and remaining routine investigations were normal. Autoimmune workup and ENA Panel were negative. Nerve conduction study showed Mixed sensorimotor neuropathy (predominantly demyelinating) involving all 4 limbs (LL>UL) and absent F-responses. MRI Spine done showed diffuse thickening along the filum terminale and cauda equina with clumped nerve roots showing patchy nodular post contrast enhancement - CIDP. Results: She was treated with IV Methylprednisolone pulse dose therapy and counselled regarding Iv Immunoglobulin administration. She was also started on appropriate management for her newly detected Diabetes. Discussion: She underwent preliminary screening modalities-prior to initiation of immunomodulatory therapy (Including TPMT assay for initiating Azathioprine). She was counselled regarding the nature of her illness, the need for follow up and long-term management modalities. Conclusion: An atypical presentation of a common disease should be considered before thinking of a common presentation of a rare illness.

Abstract ID: 692

Atypical Presentations of 2 Cases of Trigeminal Autonomic Cephalalgias

Ande Mihira Chakra Varthy, Debsadhan Biswas, Sandip Pal

Kolkata Medical College, Kolkata, West Bengal, India

E-mail: mihira94@gmail.com

Background and Aim: TACs are a specific group primary headaches characterized by unilaterality, ipsilateral cranial autonomic features ,and specific duration of pain. These include cluster headache, paroxysmal hemicranias (PH), SUNCT/SUNA and Hemicrania continua (HC). Here we describe a couple of cases of TACs with atypical presentation. Methodology: Detailed history, general as well as neurological examination, CEMRI were done in 2 cases which were admitted at Neurology ward of Medical College, Kolkata. Results: Case 1: 46yr old male smoker presented with Right sidelocked severe throbbing pain from occipital to frontotemporal region lasting for 15-20mins occuring atleast 10 times/day associated with restlessness but without any autonomic or migranous features, gets releived by Nsaids and occurring almost every day since 6 years and there is excellent response to indomethacin suggestive of chronic PH. Case 2: 45yr old male smoker presented with left sidelocked severe shock like pain in retroorbital and periorbital region lasting for 60-90mins following alarm clock pattern occuring 3 times/day associated with autonomic features like lacrimation,redness,nasal stuffiness and eyelid edema on left side with pacing and suicidal tendency almost everyday since 7 months suggestive of chronic cluster headache But this patient also had a background mild-moderate severity continuous left sidelocked periorbital pain since past 2 years with intermittent exacerbations lasting 60-90mins once in a while and had excellent response to indomethacin suggestive of HC . Discussion: Case 1: The patient having restlessness in the absence of autonomic features satisfies criteria of ICHD3beta of chronic PH. Case 2: The background continuous pain and incomplete response to cluster headache treatment raised the suspicion of HC even though the presentation being typical of chronic cluster headache. Conclusion: TACs can present without autonomic features and Exacerbations of HC can mimic cluster headache and Indomethacin response will help in differentiating TACs.

Abstract ID: 693

Autoimmune Encephalitis- A master Mimic

V. S. Veerappan

DM Neuro Pursuing, Other

E-mail: veerasomu@gmail.com

Background and Aim: Anti-NMDAr related encephalitis is a well-described dysimmune entity of the central nervous system and the most frequent of the auto-immune encephalitis (AIE). Methodology: Here we present a 70 years old female with adequately controlled diabetes mellitus for the past 10 years who presented to the hospital with 10 days history of acute onset irrelevant speech and memory impairment with no prodrome or autonomic instabilities. On examination she had poor attention span and responded only to simple verbal commands, with impairment of both recent and remote memory. Other Neurological examination was normal. Initial blood investigations and CT brain done was normal. MRI Brain showed T2 FLAIR hyperintensity and subtle diffusion restriction noted in bilateral medial temporal lobe and hippocampus. EEG done showed diffuse slow wave activity. Cerebrospinal fluid (CSF) analysis showed an acellular smear with slightly elevated protein, normal glucose. She was started with IV acyclovir empirically, however CSF was sent for HSV PCR turned negative. CSF NMDAR antibody was positive while Serum NMDAR antibody was negative. Results: She was treated with a 5-day course of IV methylprednisolone and continued on IV acyclovir. She started improving after the initiation of steroids and her attention span and spontaneous speech improved dramatically. Discussion: Initially mimicking stroke, however later on with classically different imaging, and CSF findings similar to HSV encephalitis, Anti NMDAr encephalitis can curtain the eye twice. We would like to highlight that the classic prodrome may be absent. Speech and memory impairment may sometimes be the only presenting features. Conclusion: CSF NMDAr analysis is more predictive and should be chosen over serum NMDAr analysis in resource limited settings. Anti NMDAr encephalitis, a master mimic should still be thought of in patients with even atypical or incomplete presentations.

Abstract ID: 694

The Importance of MRI Imaging of the Ocular Muscles – Three Interesting Cases

R. Pazhani, P. Pushkar, S. Kavya

DM Neuro Completed, Other

E-mail: pazran@hotmail.com

Background and Aim: Ptosis, double vision, prominent eyeballs and restricted ocular muscles are some of the symptoms, patients presenting with to the outpatient service. Three patients with some of the above complaints, where the MRI imaging of the ocular muscles made the diagnosis are discussed here. Methodology: Case 1 – Seventeen-year-old girl presented with right side partial ptosis, was initially diagnosed as seronegative ocular myasthenia. She was not responding to pyridostigmine. Case 2 – Fifty-year-old gentleman presented with prominent eyeballs, left more than right associated with right side partial ptosis. Case 3 – Forty-year-old lady presented with bilateral partial ptosis, left more than right with restricted ocular movements. Results: Case 1 – MRI imaging of the ocular muscles showed, cysticercus granuloma in the right side levator palpebrae superioris muscle. Case 2 – MRI imaging of the ocular muscles showed, enlargement of all the ocular muscles on either side, suggestive of thyroid ophthalmopathy. Case 3 – MRI imaging of the ocular muscles showed, thinning of all the ocular muscles on either side suggestive of Complete Progressive External Ophthalmoplegia. Discussion: Case 1- Imaging was done, as she is not responding to the myasthenia medication. Case 2- Had a combination of ocular myasthenia and thyroid ophthalmopathy. Acetylcholine receptor and TPO antibodies are elevated. Case 3 - The clinical suspicion was CPEO. Conclusion: In all the three cases presented here, the MRI imaging of the ocular muscles confirmed the diagnosis. It is important to do MRI imaging of the ocular muscles in special clinical situation.

Abstract ID: 695

Back to the Basics

Ibrahim Hussain, Anand Kumar, Deepika Joshi

Banaras Hindu University, Varanasi, Uttar Pradesh, India

E-mail: ibrahimhussainetu@gmail.com

Background and Aim: Takayasu’s arteritis is a chronic vasculitis affecting large and medium vessels. Aim of this presentation is to describe a case report of Takayasu’s arteritis with multiple atypical features. Methodology: A case report on Takayasu arteritis. Results: A 15 years old patient presented with history of 2 episodes of transient loss of consciousness lasting for about 1minutes over the last 6 months and was receiving antiepileptic from elsewhere suspecting generalized seizures. She had history of recurrent vertiginous sensation after writing her class examination along with tingling and numbness over the right upper limb. She had significant weight loss over the last 6 months. Bilateral radial pulsation were absent. Right lower limb pulses were feeble. Bruits noted over right carotid and right Renal artery. BP was not recordable over bilateral upper limbs. And170/90 in left lower limb. CT angiography showed widespread involvement in bilateral subclavian, carotids, SMA , Right renal artery and Infrarenal aorta. Classified as Takayasu’s arteritis as per ACR/ EULAR criteria. She was treated with Pulsed steroids with tapering dose of oral corticosteroid along with methotrexate. Discussion: Vasculitis can present as syncope and symptoms of vertebro basilar insufficiency. Subclavian steel phenomenon is a rare manifestation of Takayasu’s arteritis. Bilateral phenomenon is even rarer. All the manifestations of Takayasu’s arteritis is seen in this case that include syncope, vertebrobasilar symptoms, renovascular hypertension along with limb claudication. Limb claudication can present as paresthesia which may mislead the clinician. Conclusion: Examination of peripheral pulses and blood pressure of all the 4 limbs is extremely important in any patient coming with symptoms of vertebrobasilar insufficiency or transient alteration in consciousness.

Abstract ID: 696

Bimelic Symmetric Hirayama Disease – A Mimicker of ALS

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

DM Neuro Pursuing, Other

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. Results: 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. Discussion: 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.

Abstract ID: 697

Boomerang in the brain!!

Akash Walinjkar, Payal Patil

DNB Neuro Pursuing, Other

E-mail: akash.walinjkar@gmail.com

Background and Aim: To study the CNS adverse effects of 5 Flurouracil (5FU) and its appearance on MRI. Methodology: It is a retrospective observational study. Study area- Fortis Hospital, Mulund, mumbai. Patients known case of GI malignancy on chemotherapy containing 5FU who had any CNS symptoms were included in the study. Results: Six patients who were known to have some form of GI malignancy and were on FOLFIRINOX chemotherapy protocol which has 5FU presented with slurred speech, dysarthria and ataxia. MRI brain with diffusion and FLAIR was done suggestive of Areas of diffusion restriction in bilateral centrum semiovale, fronto parietal deep white matter and splenium of corpus callosum with ADC drop. All the areas were FLAIR negative suggestive of a boomerang sign. Discussion: Six patients with GI malignancy and on FOLFIRINOX chemo regimen presented with dysarthria, ataxia, had difficulty in reading. On examination all the patients had alexia without agraphia, dysarthria and ataxia with no paresthesia or motor weakness. MRI brain diffusion and FLAIR done suggestive of areas of diffusion restriction in bilateral centrum semiovale, fronto parietal deep white matter and splenium of corpus callosum with ADC drop, all the areas were FLAIR negative. this is known as Boomerang sign. This is due to cytotoxic edema, which is seen as a toxic effect of a chemotherapy agent (in this case it was 5 FU). There are many causes of boomerang sign- drug toxicity, vascular, demyelination, nutritional, infective etc. Conclusion: All aphasias, dyarthria, ataxias and focal neurological deficits are not due to stroke. Some may be drug induced. In the case of GI malignancies with acute onset focal deficits look for 5FU toxicity and a BOOMRANG in the brain.

Abstract ID: 698

Chromosome 9 Open Reading Frame 72 Repeats are Not Uncommon in Frontotemporal Dementia Spectrum Disorders in the Indian Context

Faheem Arshad, Keerthana Somashekar, Subasree Ramakrishnan, Mohammed Faruq, Suvarna Alladi

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: faheem2285@gmail.com

Background and Aim: The hexanucleotide repeats of the C9orf72 gene is found to be the most frequent cause of Frontotemporal Dementia (FTD), Amyotrophic lateral sclerosis (ALS) and FTD overlap syndromes. Its prevalence in all FTD cases varies from 4-29%, with 20-25% in familial cases and about 6-8% in sporadic FTDS. Unlike Caucasians, this mutation is rare in the Indian population as per a 2015 study. We aim to describe the clinical profile of patients with FTD due to C9ORF72 repeat expansions in a single tertiary dementia centre. Methodology: Patients with FTD included in the study underwent detailed cognitive assessment using validated tests in the Indian context along with MRI. Repeat-Primed PCR (RP-PCR), an often missed but necessary protocol for the detection of C9ORF72, along with Whole Exome Sequencing was done for genetic analysis. Results: Out of 45 patients with FTD who underwent genetic testing, 4 patients were detected to have C9or72 hexanucleotide repeat expansion. Clinical presentations of these 4 patients were progressive cognitive decline, behavioural disturbances such as apathy, emotional lability, speech difficulty, weakness, impaired frontal lobe functions, visuospatial and language difficulties with bradykinesia, upper and lower motor neuron weakness and dysarthria. A family history of behavioural disturbances was reported among multiple family members of two patients. Brain imaging showed atrophy in the frontal and temporal regions, in addition to the anterior cingulate gyrus. The above clinical and radiological findings were suggestive of FTD spectrum disorder. Diagnosis of behavioural variant FTD, FTD-ALS, FTD-ALS-Parkinsonism, ALS with behavioural impairment (ALS-bi) was made for the mentioned patients. Discussion: Despite previous studies reporting a low incidence of c9orf72 cases in India, this first-of-its-kind Indian report shows that c9orf72 mutations are seen in 10% of patients presenting with FTD spectrum disorders with FTD-ALS with/without parkinsonism. Conclusion: Our results emphasize the need for c9orf72 mutation testing for patients with FTD in India.

Abstract ID: 699

CAA-RI, A Little Known but Treatable Disease: A Case Report

Manju Yadav

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi, India

E-mail: mishty125@gmail.com

Background and Aim: Cerebral Amyloid Angiopathy Related Inflammation (CAA-RI) is a rare , aggressive subtype of Cerebral amyloid angiopathy (CAA ) with diverse clinical presentations and characteristic radiological findings. Rapidly progressive acute or subacute onset of cognitive decline or behavioural changes are the most common symptoms of CAA-RI due to pronounced perivascular or transmural inflammatory infiltration. Methodology: We report a case of CAARI with a favourable outcome. Results: A 69 years male without any significant co-morbidities presented with rapid cognitive decline in form episodic memory impairment, apathy, behavioral changes, executive dysfunction and recurrent episodes of acute onset focal neurological deficits. His MMSE and MOCA scores on admission were 7 and 4, respectively. Speech was slow with exaggerated deep tendon reflexes. MRI brain T2/FLAIR was suggestive of multi-focal, slightly asymmetric white-matter hyperintensities in cortico-subcortical areas along with periventricular white matter lesions. GRE sequence showed numerous cortico-subcortical macro and micro hemorrhagic lesions. T1 contrast images showed no enhancement of lesions. Inflammatory markers were elevated. CT angiography, MR vessel wall imaging and digital subtraction angiography of intracranial vessels, and brain PET-CT were normal. Discussion: A diagnosis of possible CAA-RI was made based on Auriel criteria. He was treated with IV methylprednisolone and cyclophosphamide followed by oral maintenance prednisolone. He showed significant improvement in his general and mental functions. Repeat MMSE was 13 (6 points improvement), and MOCA was 9 (5 points improvement). Conclusion: In patients over 40 years of age with a rapid and progressive dementia accompanied by headache, seizures, or focal neurological deficits, with patchy or confluent T2/FLAIR hyperintensity and cerebral microbleeds and cortical siderosis, a diagnosis of CAA-RI should be suspected. Glucocorticoids with or without immuno-suppressants can result in a dramatic reversal of cognitive decline as happened in our case.

Abstract ID: 700

Case Report of TubulinA4A Missense Mutation Presenting as Lower Motor Neuron Disease

Divya Bayatapalle, J. Manickavasagam, G. Balaji, S. Hariharan

DM Neuro Pursuing, Other

E-mail: bayatapalledivya@gmail.com

Background and Aim: Amyotrophic latera sclerosis (ALS) is a neurodegenerative disease that selectively affects motor neurons of the cortex, brain stem and spinal cord. Most common genetic mutations reported causing ALS are SOD1, C9ORF72, TARDBP, FUS. Several other genes assosciated with ALS have been reported. Here we present a case report of novel missense mutation of tubulinA4A presenting as pure motor LMN syndrome. Methodology: A 47-year-old gentleman known diabetic presented with progressive asymmetric lowerlimb onset flaccid quadriparesis over the past one year with transient dysphagia. Significant occupational history suggestive of heavy metal exposure was present. Examination showed LMN signs (wasting, fasciculations, hypotonia, global areflexia) in all 4 limbs with no sensory, pyramidal, autonomic, cerebellar, eps signs. Evaluated for a pure motor syndrome – LMN type. CBC, RFT, LFT, ESR, CRP, Blood sugars, electrolytes were normal. CPK of >1000(1yr ago), but repeat CPK was 256. NCS shows b/l peroneal axonal neuropathy. EMG shows neurogenic pattern MRI brain with spine, CSF analysis – normal Heavy metal screening – Lead – 20.4 MLPA for SMN1 gene – Negative CAG repeat analysis – 21 (not significant) While body PET – Normal. Results: Suspected of ALS – LMN type and sent for genetic analysis Whole exome sequence revealed heterogygous missense variation in exon 4 of TUBA4A gene (p. Ala289ser). Discussion: As this patient presented with Asymmetric progressive flaccid quadriparesis investigated for all causes of pure lmn syndromes which turned out to be negative as mentioned then sent for genetic analysis suspecting MND which showed missense mutations in TUBA4A gene. On Literature review there have been reports of sporadic and familia ALS with this gene. We might have picked up patient at very early stage in whom UMN signs are yet to appear (to be followed up). Conclusion: Perhaps we are the 1st ones to report case of tubulin mutation presenting as LMND.

Abstract ID: 701

Case Report: Ataxia, Movement Disorder, and Epilepsy in a Young Male - An Interesting Combination Diagnosed as Genetically Confirmed DRPLA

Sumit Kharat, Pawan Ojha, Sarika Patil, Afroz Ansari, Vikram Aglave, Shashank Nagendra

Grant Medical College and JJ Hospital, Mumbai, Maharashtra, India

E-mail: sumit_kharat2000@yahoo.com

Background and Aim: We hereby present a case of a 25-year-old male with a 3-year history of insidious onset, gradually progressive seizure disorder, cerebellar ataxia, and choreiform movements. Methodology: Patient had history of unknown onset bilateral tonic-clonic seizures 3 years back which were initially controlled with antiepileptic medications. He presented with recent increase in the frequency of seizures with a history of progressive imbalance while walking for last 1.5 years with choreiform, dystonic, and myoclonus-like movements. There was history of mild tremors and subtle myoclonus-like movements in his father. There was history of death of a paternal aunt in her late twenties due to similar progressive illness. Results: Based on the above clinical picture with significant family history, the patient was suspected to have Huntington’s chorea, Neuroacanthocytosis, Wilson’s Disease, and other inherited ataxia. Among acquired causes, drug toxicity, nutritional, viral, and other etiologies were considered. Routine investigations did not have any significant findings. MRI Brain and spine were normal. CSF studies were normal. PBS for acanthocytosis was negative. There was no KF ring on ophthalmological examination and Serum Ceruloplasmin was normal. Serum Alpha-fetoprotein, Antithyroid antibodies, and Total CPK was normal. EEG was not suggestive of SSPE. NCS was normal. Hence the genetic etiology was suspected. CAG repeats for HD were normal. Discussion: After all the above workup and possible autosomal dominant inheritance, SCA 17 and DRPLA were suspected. Test for these conditions was not available in the routine SCA panel. Further genetic testing showed 63 CAG repeats for DRPLA in ATN1 gene on allele 2, which confirmed the diagnosis of DRPLA in the above clinical scenario. Parental testing showed 55 CAG repeats in father of patient. Conclusion: This is probably the first case of DRPLA in Western India. A Triad of seizure disorder, movement disorder and ataxia with autosomal dominant inheritance are the key features for diagnosis.

Abstract ID: 702

Case Series IgG4 Spectrum Disorders.Meninges to Nerves

Devireddy Prasoona, V. Chandra, N. Thamil, L. Ravi, Lakshmi Ranganathan

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drprasoonareddy@gmail.com

Background and Aim: Introduction: Immunoglobulin G4-related disease represents an immune-mediated fibroinflammatory condition with a characteristic histopathological appearance that can affect various organs. CNS manifestations include hypertrophic pachymeningitis, involvement of the pituitary gland, inflammatory orbital pseudotumor, pterygopalatine fossa, and cranial nerve involvement . However, cranial nerve involvement is rare. exclusion of other conditions are important for accurate diagnosis. Here we are reporting a varied syndromes of igG4 Related disorder, including hypertrophic pachymeningitis ,para cavernous syndrome, cavernous syndrome. Methodology: Case Scenario: Case1:A 63 yr old male presented with dysphagia,Dysarthria,diplopia,with numbness over face since 1yr,on Examination decreased sensation over the left face on V1,V2 distribution ,left abduction restriction, uvula deviated to right,decreased gag reflex on left with normal motor examination.MRI BRAIN showed hypertrophic pachymeningitis,2DECHO suggestive of Right atrial mass ,ultrasound neck showed fibrotic strands in thyroid, elevated igG4 levels . CASE 2:A 33yr old female presented with pain and numbness over the left half of face since 6months,diplopia,difficulty in chewing since 2months.on examination, left eye abduction restriction, Decreased sensation over V1,V2,distribution over left face,with weakness of masticatory muscles.MRIBRAIN showed contrast enhancement in paracavernous region with elevated igG4 LEVELS. Results: Case3:A 34 yr old f,presented with headache, since 6 months,ptosis and diplopia since 2wks.examination showed left horners syndrome with abduction restriction MRIBRAIN showed circumferential thickening of cavernous part of ICA,with contrast enhancement suggestive of cavernous sinus syndrome with elevated igG4 LEVELS. Discussion: Despite recent advances in the identification of the underlying immunological processes, its pathophysiology is incompletely understood.The diagnostic workup is complex and requires a combination of clinical examination, imaging, histological, and serological analysis.However, no finding alone is specific for Diagnosis.Traditional treatment includes glucocorticoids and immunosupressants,now B cell targeted therapies like rituximab is emerging but single dose is not sufficient to maintain remission.hence maintanance therapy is needed. Conclusion: Hence high index of suspicion is needed for early diagnosis.Early treatment has good prognosis.

Abstract ID: 703

Case Series Study on Cytotoxic Lesions of Corpus Callosum

G. Aswin, Senthilvel Murugan, A. Prabhu, K. Vignesh, R. Kishore

DM Neuro Pursuing, Other

E-mail: aswingbahaar@gmail.com

Background and Aim: Cytotoxic lesions of the corpus callosum (CLOCCs) are a clinical-radiological spectrum of disorders that can be caused by several conditions. They were formerly known as “mild encephalopathy with reversible splenial lesions,” “transient splenial lesions,” “reversible splenial lesion syndrome.it is characterized by restricted diffusion in the splenium of the corpus callosum on MRI and accompanying neurological symptoms. we aimed to evaluate the clinical and radiological characteristics of CLOCCs. Methodology: or this case series study, we reviewed the MRI in our hospital from January 2022 to April 2023 to identify splenial lesions with diffusion restriction. primary lesions of CC were excluded. CLOCC’s were divided into infection associated, metabolic disorder associated, epilepsy associated and trauma associated. Data were collected from medical database. Results: twelve patients were determined to have CLOCCs. 6 (50%) were infection associated,4 (34%) were epilepsy associated,2(16%) were metabolic associated. all patients had a known etiology among infection group,3 cases with dengue and 2 cases with meningitis and 1 case with ARDS. All patients with infection related CLOCCs and epilepsy related CLOCCs recovered totally with good neurological outcome and one patient among the metabolic group had bad outcome. 5 patients required ICU care. Discussion: CLOCCs are associated with causes including seizures, sudden withdrawal of AED, metabolic disturbances, infections, trauma, and other entities. They are ovoid and located in the splenium but more extensive, with involvement of the body of the corpus callosum and the genu. Conclusion: CLOCCs are frequently but not invariably reversible. Although they are non-specific with regard to underlying cause, additional image findings and the clinical findings can aid in making a specific diagnosis. When they are present, their underlying cause should be sought and addressed.

Abstract ID: 704

Cerebral Venous Sinus Thrombosis Due to Iron Deficiency Anaemia in an Adolescent Girl

Venkatesh Kondle, M. R. Manivannan, P. K. Murugan, C. Justin

DM Neuro Pursuing, Other

E-mail: venkatesh.kondle@gmail.com

Background and Aim: CVST is an uncommon diagnosis in children and adolescent. Cause of CSVT include: thrombophilias, infections, dehydration, intracranial surgeries, hematological diseases and systemic illness such as nephrotic syndrome, SLE. We present here a 15-year adolescent girl who presented with persistent headache and vomiting. Methodology: 15-year-old adolescent girl presented with episodic headache for 1 week, not associated with nausea, vomiting, fever. There were no abnormal physical findings on examination. Hence, oral analgesic was prescribed. One week later, she presented with persistent headache, vomiting, diplopia. On examination, she had pallor, B/L papilledema and right abducent nerve paresis. There was no focal neurological deficit. Results: Investigations showed severe microcytic hypochromic anemia Hb 6.5g/dL, MCV 60.30fl, MCH 15.70pg, MCH 25g/dL, RDW 20%(high), and low ferritin 4.66µg/, PC 2.5 lakhs. CT brain was done, showed extensive thrombus in right transverse sinus extending to sigmoid and IJV, then confirmed on MRV. There was no evidence of infarction. Workup for thrombophilia was negative. She was treated with IV Heparin, Mannitol and 2 units PRBC was transfused. Acetazolamide was added. She was discharged on the D5. A week later, she was examined in OP where she had complete resolution of headache, diplopia, and papilledema. Acetazolamide was stopped then. LMW heparin was prescribed for 3 months. Patient had good outcome after iron supplementation. Discussion: IDA usually manifest as fatigue, exertional dyspnea and malaise. Neurological manifestations are not common. CVST is an uncommon diagnosis in children and adolescents. Conclusion: Our case is of particular interest because of unusual cause of CVT i.e., iron deficiency anaemia and the other reason is it is not associated with thrombocytosis.

Abstract ID: 705

Cerebrotendinous Xanthomatosis - A Report of Two Cases

S. Saravana Sukriya, Rasmi Sahoo, Shree Hari, Pradeepto Patro, Ashish Gupta, Ashish Gupta

All India Institute of Medical Sciences, Raipur, Chhattisgarh, India

E-mail: sukriyasampathkumar@gmail.com

Background and Aim: NA. Methodology: NA. Results: CASE 1 A 30-year-old female presented with complaints of progressive weakness of bilateral lower limbs associated with recurrent falls of one year duration. Examination revealed non-tender, non-erythematous subcutaneous swelling involving bilateral Achilles tendon, left eye cataract, spasticity involving bilateral lower limbs with power of MRC 4/5, brisk deep tendon reflexes, bilateral ankle clonus and extensor plantar. Biopsy of the swelling revealed cholesterol clefts and multinucleated giant cells, suggestive of xanthogranulomatous lesion. Axial T2 weighted MRI brain showed hyperintensities involving bilateral dentate nucleus and whole spine screening was unremarkable. A clinical possibility of cerebrotendinous xanthomatosis (CTX) was considered. Genetic studies were deferred due to financial constraints. She was managed symptomatically with baclofen and is on regular follow up. CASE 2 A 20-year-old male presented with progressive painless subcutaneous swellings over both knees, heels and dorsum of right great toe and blurring of vision for 8 years. Examination revealed high arched feet, global cognitive impairment, and bilateral lenticular opacities in slit lamp examination. Histopathological examination of the swelling revealed foamy histiocytes, cholesterol clefts and touton giant cells, suggestive of xanthoma. Axial T2 weighted MRI brain showed bilateral symmetrical cerebellar dentate nuclei hyperintensities and susceptibility weighted imaging revealed hypointensities due to mineralization. Whole exome sequencing revealed heterogenous pathogenic mutations on exon 3 (c.526delG) and exon 7 (c.1213C>T) in the CYP27A1 gene suggestive of CTX. Discussion: CTX is an autosomal recessive lipid storage disorder with diverse clinical manifestations including infantile onset diarrhea, cataracts, tendon xanthomas, neurological dysfunction manifesting as cognitive impairment, spasticity, cerebellar degeneration, parkinsonism, peripheral neuropathy, and seizures. Elevated plasma and tissue cholestanol with normal or low plasma cholesterol, decreased chenodeoxycholicacid (CDCA), increased bile alcohols, and raised cholestanol and apolipoprotein B in cerebrospinal fluid are usually seen. Early detection and treatment with CDCA normalizes plasma and CSF concentration of cholestanol and improves outcome. Conclusion: NA.

Abstract ID: 706

Characterizing the Clinical Profile and Treatment Approaches for MOG-associated Disease: A Retrospective Analysis

Thomas Mathew, Surabhi Garg, Swathi Sanjee, Molly George, Sindhu Nambiar, Sonia Shivde, Sagar Badachi, Gosala Raja Kukkuta Sarma, Raghunandan Nadig

St. Johns Medical College, Bengaluru, Karnataka, India

E-mail: chakkuthom@hotmail.com

Background and Aim: Myelin oligodendrocyte glycoprotein antibody-associated disorder (MOGAD) is increasingly being recognized as having an expanding clinical spectrum. However, treatment options remain controversial. The aim of this study was to analyze the demographics, clinical profiles, and treatments of MOGAD patients. Methodology: Registry of our MOGAD patients was analyzed for demographic information, clinical presentations and the various treatment strategies employed. Results: Our cohort consisted of 46 MOGAD patients with a mean age of 30 ± 14.7 years. There was a relatively equal distribution between genders with 24 males (52.8%) and 22 females (47.8%). The initial clinical presentation was with optic neuritis in 43.5%, myelitis in 21.7%, MOG-encephalitis in 15.2%, and ADEM in 15.2%. Relapsing disease was present in 32.6% and the median number of relapses was 2. Among steroid sparing maintenance treatments tried, rituximab was used in 34.8%, tofacitinib in 6.5%, and methotrexate in 4.3%. In a subgroup of patients (4.3%) with truly refractory disease, Tocilizumab was used. However, low dose steroids had to be used in these patients. Discussion: Unlike other CNS inflammatory conditions like Multiple Sclerosis and NMOSD with female predominance, MOGAD has a relatively equal to slightly male predominant gender distribution. While it can be monophasic in the majority, a certain group has relapsing disease. A small subgroup of patients can be truly refractory to all previously tried immunotherapies, and in these tocilizumab may be useful. Conclusion: In our cohort, MOGAD appears to have a male predominance with optic neuritis and myelitis as common presentations. Response to standard immunotherapies like rituximab is not uniform with minority of patients being truly refractory. Tocilizumab may be a useful option in this subgroup. Further studies are required to explore long-term outcomes and assess the efficacy of different treatment approaches in MOGAD patients.

Abstract ID: 707

Clinical and Radiological Profile of Trigeminal Neuralgia

Praveen K. Yadav, Praveen Yadav

DM Neuro Completed, Other

E-mail: dr.praveen4u@gmail.com

Background and Aim: Trigeminal Neuralgia also called as Tic doulorex,is a chronic pain condition characterized by recurrent,unilateral,brief,painful,electric shock like pain episodes in trigeminal distribution which is abrupt onset and termination. Methodology: All patients from December 2021 to October 2022 presented to superspeciality neuroclinic with symptoms suggestive of Trigeminal Neuralgia were included in this study after consent. Clinical pattern and MRI brain findings along with other risk factors and comorbidities were studied.MRI brain with 3D CISS/FIESTA Sequence was done in every case to rule out any secondary causes . Results: Out of 30 patients 17(56.67%) were females and 13(43.33%) were male.Most common age group was 51-60 years (53.33% ) followed by 61-70 years (26.66%). MRI Brain showed vascular loop in 20 % wile the majority did not have any compressive etiology.Most common clinical presentation is sudden severe shooting pain in one side of face last for few seconds to 2 minutes which is triggered by eating, drinking, brushing . Out of 30 cases , 3 had atypical Trigeminal Neuralgia , two had bilateral Trigeminal Neuralgia. Discussion: unilateral and may affect one or more divisions of trigeminal nerve which is ophthalmic , maxillary, mandibular[9]. In review literature male to female ratio is 1 to 1.5 to 1 to 1.7.mean age of onset 53-57 year Contrary In this study we found that Male to Female ratio ranges from 1 to 1.3 and most common age affected are 51 – 60 year. On MRI we found that more than 80% cases no secondary cause like vascular loop was found as a cause of trigeminal neuralgia. Conclusion: Trigeminal neuralgia is mainly a clinical diagnosis.Neuroimaging should be done in all cases to rule out secondary causes.Refractory cases are common.

Abstract ID: 708

Clinical Characteristics of Patients with Early Onset Dementia from a Memory Clinic

Atri Chatterjee, Ankur Abhinav, Amul Agarwal, Sonali Aggarwal, Aparna Kathait, Siddhartha Dhar, Sanghamitra Laskar

Vardhman Mahavir Medical College, New Delhi, India

E-mail: chatterjee.atri@gmail.com

Background and Aim: Early onset dementia (EOD) is a term used to describe all forms of dementia that manifest before the age of 65. The prevalence of EOD ranges from 6.9% to 44% among studies conducted on dementia patients in different parts of the world. In this study, we aimed to investigate the clinical characteristics of a cohort of patients with EOD. Methodology: We reviewed the medical records of all patients (N= 181) who visited the Cognitive Neurology Clinic at VMMC, New Delhi between 2018 and 2022, and selected patients who met the criteria for dementia and had an onset of symptoms before the age of 65. Patients with an unclear diagnosis or confounding variables such as a history of traumatic brain injury were excluded from the study. 79 patients matched the selection criteria and their demographic characteristics, initial symptoms, diagnosis and cognitive testing scores were extracted for analysis. Results: 63.3% male and 36.7% female patients had an average of 8.4 years education (SD ± 5.3). Age of symptom onset was 54 years (SD ± 6.35); at baseline, patients were 57.2 years (SD ± 7.14) and had a 3.2-year delay for baseline assessment. Common diagnoses were Alzheimer’s disease (37.9%), vascular cognitive impairment (30.4%), and frontotemporal dementia (10.1%). Memory impairment was the commonest initial symptom (63%), followed by altered behaviour (36.8%) and visuospatial symptoms (18.4%). The mean MoCA score at baseline was 12.7 (SD ± 7.35). Discussion: A considerable proportion of individuals in India experiencing dementia exhibit symptoms at a relatively young age. Alzheimer’s disease (AD) is the most frequently occurring form of dementia, followed by vascular dementia and frontotemporal dementia. The most commonly reported initial symptom among patients is memory impairment, with moderately impaired Montreal Cognitive Assessment (MoCA) scores being observed after an average of 3.2 years from the onset of symptoms. Conclusion:-.

Abstract ID: 709

Clinical Profile of Headaches in Patient of Headache with Irritable Bowel Syndrome: An Observational Study

Bishal Shah, Ashutosh Tiwari, Itish Patnaik, Ravi Kant

All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

E-mail: shahbishal24@gmail.com

Background and Aim: IBS and headache affect approximately 15-20 % of the general population which comprises of young females most commonly.Our aim was to assess the pattern of headaches in patients of headache with IBS,the onset of gastrointestinal and headache symptoms and association between headache type and type of IBS,if any. Methodology: The study was approved by institutional ethics committee (IEC/21/394). Consecutive 100 patients from inpatient & outpatient Department of Medicine, Neurology and Gastroenterology at AIIMS Rishikesh having IBS and headache symptoms were recruited.A detailed history based on a semi structured interview was recorded.We used ICHD-3 and ROME-IV criteria for headache and IBS respectively. Results: Study population had 64 females (64%),mean age of 37.59 (±11.88)years.Headache in study population was predominantly bilateral (57%),pulsatile in quality (70%) of moderate intensity (54%) and occurred in high frequency i.e. >10/month (51%).Predominant headache type observed was migraine without aura (61%),TTH (27%),migraine with aura (9%) and rest were cluster headache (3%).46% patients had onset of IBS before headache (IBS onset headache). The analysis showed that IBS onset patients had predominantly bilateral (67.39%), pulsatile (56.2%) and tight-band like (32.6%), moderate intensity (52.17%) headache occurring at low frequency (i.e.<4/month).IBS onset patients had higher percentage of TTH patients (43.47% vs12.96%,P=0.003). Discussion: In our study we observed migraine in 70 (70%), TTH in 27 (27%),and cluster in 3 (3%) patients. Migraine was common type as in previous studies.To the best of our knowledge this is first study showing comparison of headache characteristics in a cohort based on symptom onset (IBS vs Headache onset).Presence of tight-band like, dull-aching quality headache of lower frequency and aggravation with stress was seen in IBS onset which was statistically significant compared to that of headache onset group. Conclusion: Our study showed that in addition to migraine,IBS patients also have other phenotype of headaches like TTH and cluster headache.IBS onset patients commonly have TTH type headache.

Abstract ID: 710

Clinical Profile of Patients from a Botulinum Toxin Injection Specialty Clinic

Adreesh Mukherjee, Sanjay Pandey

DM Neuro Completed, Other

E-mail: adreesh03@yahoo.co.in

Background and Aim: Botulinum neurotoxin (BoNT) injection is the standard therapy for disorders such as dystonia and spasticity. Recently, its use has expanded to include other disorders such as neuralgic pain. We aim to present the clinical profile of patients injected at a Botulinum toxin specialty clinic. Methodology: This is a cross-sectional study including consecutive patients who were injected with BoNT (OnabotulinumtoxinA) in the past 6 months. Electromyography (EMG) guidance was used where required. Results: 75 patients were included in the study, and 56% were female. The indications for BoNT injection were- oromandibular (±lingual) dystonia (OMD) (n=18, 24%), cervical (n=10) and segmental craniocervical (n=9) dystonia, hemifacial spasm (HFS) (n=8), and blepharospasm (n=4). Writer’s cramp and other limb dystonia comprised 4 patients each. The other indications included neuralgic pain (n=9), apraxia of eyelid opening (n=5), spasticity (n=2), head tremor (a patient of SCA12), and cervical dystonic tics (n=1). HFS (75%) and neuralgic pain (66.7%) were more prominent in females, while it was reverse for limb dystonia including writer’s cramp (75% male). EMG guidance was used for cervical, limb, and oromandibular dystonia, and spasticity. Discussion: This study demonstrates that a referral BoNT clinic usually comprises relatively difficult or complex indications such as cervical, limb, and oromandibular dystonia in addition to HFS and blepharospasm. Moreover, atypical indications such as neuralgic pain, apraxia of eyelid opening and tics are also encountered. While EMG guidance is routinely used for cervical, limb, and oromandibular dystonia, and spasticity, the additional cost sometimes becomes a barrier. Conclusion: BoNT is a useful treatment modality for several disorders, and the list is increasing. EMG guidance is valuable in some of the indications.

Abstract ID: 712

CNS Tuberculosis Presenting as Focal Vasculitis Causing Top of the Basilar Artery Syndrome

Iqbal Singh, Monika Singla, Gagandeep Singh

Dayanand Medical College, Ludhiana, Punjab, India

E-mail: driqbalsingh16593@gmail.com

Background and Aim: The most serious consequence of CNS Tuberculosis is the development of vasculitis in the vessels of the circle of Willis, the vertebrobasilar system, and the perforating branches of the middle cerebral artery, resulting in infarctions in the distribution of these vessels. We report a case of CNS tuberculosis with basilar artery vasculitis causing multifocal infarctions in respective vascular territory. Methodology: A 41-year-old male presented with the complaints of fever since 2 months, headache since 15 days, left sided weakness since 1 day. His neurological examination was remarkable for left hemiparesis and left upper motor neuron facial palsy. On 4th day of his hospital stay, his condition worsened. He had bilateral tonic clonic seizure preceded by neck turning towards left side followed by post ictal confusion, bilateral ptosis, and dilated, non-reacting left pupil. He was managed with mechanical ventilation and other appropriate supportive treatment. Results: CE-MRI Brain revealed well-defined extra axial T2W hypointense lesion with intense homogenous contrast enhancement along left tentorial leaflet, possibly ‘enplaque tuberculoma’ with acute vasculitic infarct involving right upper pons.CSF analysis revealed lymphocytic pleocytosis with elevated proteins and low sugar .CBNAAT was negative and patient was started on antitubercular drugs under steroid cover. Repeat MRI on 4th day revealed multiple acute infarcts in left occipital, left thalamus, left side of midbrain, and cerebellar hemispheres. CT angiography neck vessels revealed markedly attenuated distal basilar artery with short segment of faint opacification just proximal to its bifurcation. Discussion: Approximately two-fifths of tuberculous meningitis patients develop intracranial vasculitis. Vasculitis of the basilar artery as seen in our patient, is an extremely rare entity, and has been described very rarely in the literature till date. Conclusion: CNS Tuberculosis presenting as top of the basilar artery syndrome is a rare clinical entity. Future studies into this entity would guide us in elucidating exact pathophysiological mechanisms.

Abstract ID: 713

Clinico-radiological Profile of Idiopathic Intracranial Hypertension

Praveen K. Yadav, Praveen Yadav

DM Neuro Completed, Other

E-mail: dr.praveen4u@gmail.com

Background and Aim: Idiopathic intracranial hypertension (IIH) is a condition of raised intracranial pressure (ICP) in the absence of a spaceoccupying lesion. IIH patients usually present with typical symptoms and signs of increased ICP, such as, headache, vomiting, neck pain,double vision, transient visual obscuration and papilledema. Typical MRI findings include empty sella, optic nerve tortuousity, globe flattening and transeverse sinus stenosis. Methodology: All patients from July 2021 to June 2022 presented to the super speciality neurology clinic with symptoms suggestive of IIH were included in this study after consent. Clinical pattern and MRI brain findings along with other risk factors and comorbidities were studied. Resulst: Total12 patients were studied out of which all werefemale. The most common age group 31-340 years (41.6%).The most common clinical presentations were headache and Transient Visual Obscurations (TVO) followed by painless loss of vision, visual field changes and double vision. 7 out of 12 cases presented with headache (58.3%) out of which only 3 were suffering from migraine. TVOs was presenting complain of 7 patients (58.3%). Painless visual loss was there in 3 patients (25%), out of which only one case was bilateral (33%).In all cases (100%) MRI was suggestive of IIH. Transverse Sinus Stenosis in 3 cases (25%) and scleral flattening in 1 case (14.2%) were seen respectively. Discussion: Our study should similar findings as compared to previous studies in the clinical symptoms and MRI Features. Conclusion: IIH is most prevalent in females of age group 31-40 years. Headache (58.3%) and/or Transient Visual Obscurations (58.3%) are the most common presentation of IIH in our study.Clinical suspicion is of utmost importance for diagnosis of IIH to prevent complications. Every patient with headache and visual symptoms should undergo MRI Brain.

Abstract ID: 714

Congenital Diastematomyelia in Upper Thoracic Spine-rare Presentation

M. Kumar, M. R. Manivannan, P. K. Murugan, C. Justin

DM Neuro Pursuing, Other

E-mail: sathishmd89@gmail.com

Background and Aim: Diastematomyelia, also known as Split Cord Malformation is a congenital spinal anomaly in which there is longitudinal splitting of cord that requires early diagnosis. Two hemicords are separated by fibrous, bony or cartilaginous septum. Antenatal detection of cord abnormalities by USG can be limited by acoustic shadowing from spine, foetal position and the amount of liquor. We report a new born baby with split cord malformation diagnosed by clinical examination and imaging. Methodology: Here by we report a neonate born out of non consanguineous marriage by LSCS, first in birth order to a 28 year old female, Baby cried immediately after birth and has weight of 2.5 kgs. On examination there was a small midline swelling in the upper dorsal region. The swelling was soft and there was no discharge from the lesion without any other deficit. Results: MRI was done on day 8 of neonatal period showing division of spinal cord into two hemicords by a bony septum at the level of D4 surrounded by separate dural sac which extends up to D5 vertebra beyond which it fuses, suggestive of diastematomyelia-1. This band was seen extending up to the pedicle. This is associated with low lying conus, tethered cord at L2-L3 and cord ends as low as L4-L5 level. Central cord appears dilated for a length of 9.3mm extending from D3 to D4. Discussion: Diastematomyelia may be an isolated finding or seen associated with myelomeningocele, lipoma etc. Females are affected commonly than males. It usually occurs between D9 and S1 commonly at upper lumbar vertebra. Conclusion: Our case is of particular interest because of its atypical location i.e., upper dorsal region. So to prevent neural tube defects, mother should have regular antenatal check-ups,ultrasound examinations. These babies should be followed at regular intervals to identify any deficits, bladder dysfunction that can be developed.

Abstract ID: 715

Correlation of Clinical and Neuro-electrophysiological Parameters in Type 2 Diabetes: A Cross-sectional Study

Rajat Gupta

Dayanand Medical College, Ludhiana, Punjab, India

E-mail: rajat01121993@gmail.com

Background and Aim: Nerve conduction studies (NCS), Vibration perception threshold (VPT), Sympathetic skin response (SSR) and Ankle-brachial index (ABI) are commonly used to assess patients with Type 2 Diabetes Mellitus (T2DM). However, the correlation of these parameters with clinical variables such as HbA1c, duration of diabetes, diabetic nephropathy, retinopathy has not been extensively explored. This study aimed to investigate the clinical and neuro-electrophysiological parameters in patients with T2DM and to assess the associations between them. Methodology: This observational, non-blinded, cross-sectional study enrolled patients aged over 40 years with a diabetes duration exceeding one year. Clinical assessment was done using the Michigan Neuropathy Screening Instrument (MNSI). Fasting blood glucose, HbA1c, and urine albumin-creatinine ratio (UACR) were measured. NCS abnormalities were diagnosed based on the Toronto Diabetic Neuropathy Expert Group criteria. VPT, SSR, and ABI measurements were obtained for all participants. Results: The study included 125 patients, with a mean age of 61 years and a female proportion of 31.2%. The mean duration of diabetes was 9.28 years, mean fasting blood glucose was 161 mg/dl, and mean HbA1c was 8.98%. Axonal sensorimotor polyneuropathy was the most prevalent type of neuropathy. NCS abnormalities were present in 83.2% of the participants and were significantly correlated with age, gender, duration of diabetes, HbA1c levels, nephropathy, retinopathy, MNSI scores, VPT, and SSR. Discussion: Medial/ Lateral plantar motor and Sural sensory were the earliest nerves to be involved in DPN and should always be a part of protocol for testing for DPN. Medial plantar CMAP cut-off value of 5.5 mV and Medial plantar SNAP cut-off value of 2 µV is a sensitive and specific test for predicting NCS abnormality. Conclusion: A combination of NCS parameters can effectively detect early DPN. VPT is a sensitive screening tool, SSR identifies autonomic dysfunction, and ABI detects vasculopathy in DPN patients.

Abstract ID: 716

Correlation of Thrombus Attenuation Score and Nihss in Acute Ischemic Stroke Due to Large Artery Atherosclerosis

Ronanki Priyanka, Muralidhar Yerasu, Subhendu Parida, Lalitha Pidaparthi, Shyam Jaiswal, G. Abhinay, J. M. K. Murthy

DNB Neuro Pursuing, Other

E-mail: priyanronanki108@gmail.com

Background and Aim: Large artery atherosclerosis (LAA) is the most common acute ischemic stroke (AIS) mechanism. Previous studies showed that thrombus density correlated with functional outcomes. Thrombus attenuation increase (TAI) is a radiological measure of thrombus permeability. It is calculated from the difference in the Hounsfield units of thrombus on non-contrast computed tomography (NCCT) and CT angiography (CTA). We aimed to study the correlation between TAI and admission-NIHSS in LAA-AIS. Methodology: This retrospective was conducted at CARE Hospital, a tertiary care hospital in Hyderabad, between October 2022 and March 2023. All consecutive patients of LAA-AIS admitted into the neurology unit and with a demonstrable thrombus of CT/CTA during the study period were included. Age, gender, risk factors, admission NIHSS, artery involved, and TAI were entered in proforma. TAI values were compared between minor stroke (NIHSS ≤4) and moderate/severe stroke (NIHSS ≥ 5). Results: Total number of patients - 43; Age (Mean±SD) -65+15.09 years; M:F – 2.3:1; Risk factors - hypertension 28(65.21%), diabetes 24(55.8%), coronary artery disease 15(34.8%). hyperlipidemia (5(11.3%), chronic kidney disease and peripheral vascular disease in 1 (2.3%); Artery involved: middle cerebral artery 14(32.5%), Internal carotid artery 12(27.9%), basilar artery 5(11.6%) anterior cerebral artery 4(9.3%), vertebral artery 7(16.3%), and posterior cerebral artery 1(2.3%); Admission NIHSS: ≤ 4 – 25 (58.1%), ≥ 5 – 18 (41.8%). TAI score did not differ significantly between both the groups of NIHSS (14.72±7.01 vs 16.32±10.02, P value -0.56). Discussion: -. Conclusion: Thrombus permeability did not correlate with NIHSS in patients with LAA-AIS in this study.

Abstract ID: 717

Creutzfeldt-Jakob Disease – Case Series from Tertiary Care Centre in South India

K. Ravi

DM Neuro Completed, Other

E-mail: doctorravikumark@gmail.com

Background and Aim: Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal neurodegenerative disorder caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. To report a series of patients with probable CJD from a tertiary care center in southern India. Methodology: Here we present a case series of 5 cases who were managed in a tertiary care center in South India. Patients who presented with rapidly progressive dementia fulfilling the diagnostic criteria for CJD were included. All were investigated in detail to find out any possible treatable cause including magnetic resonance imaging (MRI) of brain, electroencephalography (EEG) and cerebrospinal fluid analysis. Results: A total of 5 patients of probable CJD diagnosed using the CDC diagnostic criterion between December 2021 and January 2023. All patients presented with rapidly progressive dementia. While 60% of patients had the classical EEG findings, 100% had typical MRI features. Discussion: All patients presented with rapidly progressive dementia. While 60% of patients had the classical EEG findings, 100% had typical MRI features. Conclusion: High index of suspicion is required to identify the patients with classical manifestations of CJD. The clinical features were almost similar to other reported series.

Abstract ID: 718

Creutzfeldt-Jakob disease Masquerading as Psychosis - A Case Report

S. Shalini, R. Pradeep

MS Ramaiah Medical College, Bengaluru, Karnataka, India

E-mail: shalinis2594@gmail.com

Background and Aim: Creutzfeldt-Jakob disease is a rapidly progressive infectious disease resulting in death within 1 year of onset. Clinical features seen in patients with sporadic Creutzfeldt-Jakob disease (sCJD) may be varied and diverse. Owing to diverse clinical features, it’s important to differentiate the psychotic symptoms which is essential for the management. Methodology: In this abstract, we discuss the clinical case of a 67-year-old female patient presented with 1 month onset of withdrawn behaviour, irritability, cognitive decline, occasional myoclonic jerks and visual hallucinations. There was no past history or family history of psychiatric illness . Symptoms were rapid in onset, non pervasive and atypical to psychosis hence organicity was suspected. Results: MRI Brain showed Anterior Putamen and Caudate DWI Bright signal intensity (R>L) with extreme cortical ribboning in the parieto-temporal area EEG reports showed periodic generalised sharp wave discharges at rate of 1Hz, which is suggestive of CJD. Discussion: CJD clinical onset can present with mood and behavioural disturbances which can often be misdiagnosed as psychiatry disorders, in our case the initial symptoms were psychiatric symptoms. The exact mechanism for psychotic symptoms which occur in sCJD is not known, but it is thought to be related to the limbic system. Conclusion: Clinicians must be cautious in identifying the psychiatric symptoms which can be the earliest presenting signs and symptoms in CJD. One should be aware of different ways a CJD patient can present. Thorough history, examination and high grade of suspicion will help in diagnosis and management.

Abstract ID: 719

Crossed Aphasia in Dextrals

Ragasivamalini Balachandran, V. Balambighai

DM Neuro Pursuing, Other

E-mail: rsmalini.91@gmail.com

Background and Aim: Crossed aphasia is defined as a language disturbance after right hemispheric stroke in dextrals. Aphasia in right handed individuals are usually caused by left hemispheric stroke, and crossed aphasia following a right hemispheric lesion is rarely observed. The prevalence of CA in right handed individuals is around 0.38% – 3%. The aim is to present such a rare case as a poster in this forum. Methodology: Here, we are going to present a case of crossed aphasia in dextrals. Results: We present a case of right monoparesis with aphasia with right hand dominance. A diabetic and hypertensive woman in her early fifties presented with acute onset of weakness of right upper limb associated with slurring of speech and deviation of angle of mouth to right side. She also had mild memory lapses and Urinary urgency. Her MMSE was 20/30 at the time of admission. On Examination, her language assessment showed impaired comprehension and repetition, with impaired recent memory, with intact fluency. Other lobar functions were normal. Discussion: MRI brain showed acute infarct in right temperoparietal lobe, corona radiata, internal capsule and insular cortex. Carotid & vertebral Doppler showed non visualization of right internal carotid artery, probably -complete occlusion. CTA from arch of aorta to intracranial vessels showed complete luminal occlusion of right ICA. Left ICA (throughout the course) showed 40% -50% stenosis. Right ACA & Right MCA were reduced in caliber. she was started on dual antiplatelets, statins, LMW heparin. ESR was 22mm/hr. Other blood parameters including TSH, Fasting Lipid profile and renal functions were within normal limits. Cardiac evaluation was unremarkable. Her limb dominance was checked with Edinburgh Handedness inventory and was suggestive of right dominance. Conclusion: At the end of 2 weeks, her right upper limb weakness improved with MMSE of 24/30. Hereby, we conclude that our patient fulfilled the diagnostic criteria of CAD.

Abstract ID: 720

Crossed Aphasia in dextrals -A Case Report

Anandi Damodaran, Rehab Abdo Qasem, Devdutt Kotekar, Khalid Elgharib Attia

DM Neuro Completed, Other

E-mail: anandimuthuraj@hotmail.com

Background and Aim: Crossed aphasia in dextrals (CAD)is defined as aphasia after right hemispheric stroke. Usually, aphasia is caused by left sided stroke in right-handed individual. Prevalence of crossed aphasia in right-handed individual is 0.38% to 3%.CAD was diagnosed when following are present –aphasia, right handed patient ,no. Methodology: Here we present a case of crossed aphasia in a right-handed individual in addition to,she also presented with a global aphasia without hemiparesis. Results: She is 56-year-old female, known case of severe mitral stenosis with chronic AF on warfarin. she was brought to our hospital as she was not communicating. On initial examination patient is conscious, globally aphasic with left facial lag and moving all her limbs. She was not thrombolysed as she was out of window. She was treated conservatively with full dose anticoagulation and upon discharge started on oral anticoagulants. At the time of discharge patient was able to understand few commands, was having echolalia with non-fluent speech. She was reviewed again at 3 months, and she had full recovery of her speech. Discussion: CAD is a rare stroke syndrome with low prevalence. Even though all subtypes of aphasic syndromes were seen in CAD -Brocas aphasia was the common subtype and men are affected commonly. Most patients also present with apraxia and visuo -spatial neglect. Conclusion: Our patient presented with global aphasia without any weakness. she had all criteria for the diagnosis of CAD and showed rapid recovery. On recovery patient initially had echolalia and fluent speech. There is no apraxia or visuo-spatial neglect. Follow up of this patient after 3 month her speech has recovered completely. The cause in this patient is thought to be two different lesions involving language areas due to embolism from her heart. This case is presented for its rarity of CAD and GAWH in the same patient in right sided infarct.

Abstract ID: 721

Cryptococcal Meningitis in Immunocompetent Individuals Mimicking Rapidly Progressive Dementia: A Case Report

Abhishek Dixit, Abhishek Pathak, Vijaya Mishra

Institute of Medical Sciences, Varanasi, Uttar Pradesh, India

E-mail: abhidixi88@gmail.com

Background and Aim: Cryptococcal meningitis is a serious fungal infection of the brain and spinal cord that can cause a range of neurological symptoms. In rare cases, it can mimic rapidly progressive dementia, leading to misdiagnosis and delayed treatment. Early recognition and treatment of cryptococcal meningitis are critical to prevent neurological damage and improve outcomes. Methodology: A 57-year-old male with a history of chronic kidney disease (CKD) not on maintenance hemodialysis for the last 6 months with Insidious onset, subacute, progressive apathy with visual hallucinations, altered sleep and short-term memory loss with intermittent fever and headache without history of seizures/ myoclonus.his lab and neuroimaging was performed. Results: Lumbar puncture was performed to obtain CSF for studies. The patient’s CSF studies showed 240 cells with 60% lymphocytes and 40% neutrophils, protein levels of 120, and glucose levels of 26. His CSF cytology was negative for malignant cells. His CSF CBNAAT was negative. His serum cryptococcal antigen report was positive. Discussion: Diagnosis of CM requires a combination of clinical, laboratory, and imaging findings. Lumbar puncture with cerebrospinal fluid (CSF) analysis is crucial for diagnosis it has positive India ink staining, cryptococcal antigen testing, and fungal culture confirm the presence of Cryptococcus spp. Neuroimaging, such as magnetic resonance imaging (MRI) reveal meningeal enhancement or hydrocephalus .The management of CM in immunocompetent individuals involves antifungal therapy, typically with amphotericin B and flucytosine, followed by fluconazole for consolidation and maintenance therapy. Early diagnosis and prompt initiation of treatment is essential to prevent complications, such hydrocephalus, cerebral infarction and death. Conclusion: Cryptococcal meningitis in immunocompetent individuals rarely can present with atypical manifestations of rapidly progressive dementia causing significant diagnostic challenges. One should maintain a high index of suspicion and consider CM in the differential diagnosis of RPD and prompt diagnosis and appropriate antifungal therapy improve patient outcomes.

Abstract ID: 722

Cytomegalovirus Infection Causing Optic Neuropathy in Immunocompetant Patient

Sidharth Sharma, Abdul Qavi, Ajai Singh, Pradeep Maurya, Dinkar Kulshreshtha

DM Neuro Pursuing, Other

E-mail: sidharthsharma1992@gmail.com

Background and Aim: Cytomegalovirus (CMV) infection is common among patients of all age groups, but it has traditionally been considered as a problem in neonatal and immunosupressed patients. Methodology: We report the case of a 46 year old female presenting with acute onset headache and painful loss of vision with altered colour perception and a paracentral scotoma of left eye. Upon ophthalmological examination, best corrected visual acuity of the left eye was 6/24 with impaired coloured vision (00/16) with fundus examination suggesting papillitis. Right eye fundus examination was normal. There was left-sided relative afferent pupillary defect. VEP revealed abnormal pattern in left eye; the remaining neurological examination was unremarkable. There was no past history of HIV, DM or other immunosuppressive disease. Past history of similar episode dated 5 years back. Lab work up revealed a normal hemogram and normal complete metabolic panel. CSF analysis showed <5 cells with normal protein and sugar levels. NMO and MOG were negative. ANA and vasculitis profile was normal as well. On further investigation CSF real time PCR was positive for CMV with 1.4x104 IU/ml viral load. Results: Patient was treated with tab gangcylovir 450mg bd for 21 days. Follow up was done after completion of therapy which showed improvement in vision. Discussion: Cytomegalovirus infection in immunocompetent patients is usually subclinical. CMV eye infection presents as keratouveitis, retinitis, retinal branch angiitis or anterior optic neuropathy (papillitis), the most worrisome being CMV retinitis which can lead to blindness after macular involvement. Conclusion: The reason some immunocompetent patients develop a neuroretinal disease is unknown and CMV screening should be done in suspected optic neuritis. A possible benefit of the antviral treatment is seen in such patients.

Abstract ID: 723

Cytotoxic Lesion of Corpus Callosum in CNS Tuberculosis

Nikhil A. Kumar

Banaras Hindu University, Varanasi, Uttar Pradesh, India

E-mail: nikhilakmr@gmail.com

Background and Aim: To describe a case of Cytotoxic lesion of corpus callosum (CLOCC) in CNS tuberculosis. Methodology: Case report and review of literature. Results: 25-year-old female presented with 3-month history of acute onset severe headache followed by intermittent high grade fever. One month later she developed progressively worsening drowsiness with blurring of vision. The vision loss was acute onset, bilateral. She continued to deteriorate as her headache increased and she developed vomiting. At admission she was drowsy with bilateral papilloedema and left hemiparesis. MRI brain showed communicating hydrocephalus with a T2/FLAIR hyperintense lesion in posterior corpus callosum. CSF analysis was suggestive of chronic meningitis. CSF CBNAAT was negative. She underwent VP shunt for her raised intracranial tension and anti-tubercular therapy was started with steroids. Discussion: Cytotoxic lesions of corpus callosum (CLOCCS) is known by several other names like transient splenial lesions (TSL), mild encephalitis/encephalopathy with a reversible isolated SCC lesion (MERS), reversible splenial lesions and reversible splenial lesion syndrome (RESLES). Typical MRI features are hyperintense signal on T2, FLAIR and DWI with decreased apparent diffusion coefficient (ADC) values on ADC map, and hyper-isointense signals on T1 without contrast enhancement. The lesions typically disappear within 2 weeks. The lesion may involve the corpus callosum alone or it may extend into adjacent white matter. Transient splenial lesions have been described in viral encephalitis, drug use and hypoglycemia. It is thought to be due to IL-6 release causing breakdown of blood-brain barrier and activation of astrocytes. The release of glutamate into extracellular fluid leading to cytotoxic oedema. The peculiar involvement of corpus callosum is believed to be due to high glutamate content in this part. CLOCCs have been described only rarely in CNS tuberculosis infection. Conclusion: CLOCCs are a newly described radiological entity and neurologists must be aware of its existence.

Abstract ID: 724

Deciphering A Rare Case of Cerebral Arteriopathy in a Patient with ACTA 2 Gene Mutation

Abhishek Bhargava, Bhawna Sharma

Pursuing MD Medicine, Other

E-mail: bhargavaabhishekrohan@rediffmail.com

Background and Aim: We describe an interesting case of abnormal neurovascular vasculopathy which has not been reported previously in India. A 3-year-old male child presented to us with a history of delayed developmental milestones, and multiple episodes of drug-refractory focal seizures with impaired awareness and macrocephaly. Methodology: The patient’s MRI Brain showed bending and hypoplasia of the anterior corpus callosum with malformed and hypoplastic posterior corpus callosum manifesting as colpocephaly. On axial images, the anterior corpus callosum demonstrated a characteristic V-shaped appearance. There was abnormal radial gyration of the frontal, parietal, and temporal lobes. “Twin peaks sign” was seen in the pons. CT angiography of Brain and neck vessels showed abnormally straight morphology of proximal branches of the circle of Willis (broomstick appearance), vascular ectasia of the proximal internal carotid artery, and stenotic features in the terminal part of the internal carotid artery. Results: The patient’s genetic panel was positive for ACTA2 mutation. Discussion: Actin is one of the most essential proteins in eukaryotic organisms that play a pivotal role in the protein-protein interactions which is required for smooth muscle function. The occlusive disease observed in intracranial vessels is a result of fibrosis that results in the thickening of the vascular wall. A possible explanation for these brain imaging findings, which can be helpful in diagnosis, is a mechanical effect on the brain parenchyma during development by abnormal rigid vessels with the possible contribution of cross-regulation between different actin isoforms. Conclusion: Patients with these mutations are also predisposed to premature coronary artery disease, young onset stroke, epilepsy, and aortic dissections in their adulthood.

Abstract ID: 725

Deciphering Molecular Efficacy of Repetitive Transcranial Magnetic Stimulaiton in Treating Dementia in Low vs Himalayan High-landers: Protocol

Ashish Sharma

DM Neuro Completed, Other

E-mail: ashishsharmamd@yahoo.co.in

Background and Aim: Repetitive transcranial magnetic stimulation (rTMS), a non-invasive, electrodeless, focal brain stimulation technique, has heterogenous effects on dementia. Thus, an optimal protocol should be evaluated through a bio-marker, such as Clusterin (CLU) that directly correlates with rate of clinical progression. Objectives: 1. Scrutinizing the efficacy of single vs multi-session rTMS in improving cognitive performance. 2. Assessing effects of rTMS on temporal profile of CLU. 3. Examining the metabolomics profile of dementia-resilient high-lander over 60 years old. 4. Role of multi-session rTMS on high-lander Dementia patients. 5. Investigating differential metabolomics profile of dementia progression in low vs high-lander patients. Methodology: Study duration: 36 Month, Study design: Interventional & Multi-centre, Sample Size:208 AIIMS OPD or medical camp patients diagnosed with dementia will be enrolled in the study after fulfilling the recruitment criteria and giving their legal consent. Then the dementia patients will be randomly divided in the sham and intervention groups. Altogether there will be 6 groups. 3 groups of low-lander dementia patients: (i) Sham stimulation (SS), (ii) Single-session stimulation (SSS) and (iii) Multi-session stimulation (MSS). Fourth group contains high-landers with MS protocol. Group 5 and 6 are for healthy voulnteers consisting of low- and high-landers. Results: On day one, patients from each group will receive stimulation (sham or rTMS)and under go cognitive task. Blood will be collected before the stimulation session to get the baseline value of CLU biomarker. Patient from MSS will be coming every week for a month to the health care facility. After a month from the day of the recruitment, cognitive assessments will be done and blood will be collected for biochemical analysis During this entire process, patients will take the standard drug therapy as prescribed by the neurologist. Discussion: Novelty: a. Establishing an optimal protocol of rTMS in dementia patients. b. Deciphering a novel metabolic signature from dementia-resilient high-landers. Conclusion: Funding: Extramural – SERB (File Number: SPG/2022/000744 Dated 27-12-22).

Abstract ID: 726

Decompression Craniectomy in CVT: A Short Case Series

G. Manvitha, Y. Muralidhar Reddy, Shyam K. Jaiswal, Lalitha Pidaparthi, Abhinay Kumar Gattu, Subhendu Parida, Syed Osman, B. Santosh Kumar, Jagarlapudi M. K. Murthy

DNB Neuro Pursuing, Other

E-mail: manvithareddy01@outlook.com

Background and Aim: Hemorrhagic infarcts and raised intracranial pressure are the primary pathophysiological consequences of cerebral venous thrombosis (CVT). These can be rarely life-threatening and warrant decompression craniectomy (DC). We aimed to study the short and long-term outcomes of patients with CVT who underwent DC. Methodology: Study type - Retrospective; Study site - tertiary care hospital; Study period - April 2015 to March 2023; Inclusion criteria - consecutive patients of CVT who underwent DC; Diagnosis of CVT is based on MR/CT venogram; Data elements - age, gender, cause, clinical and radiological features, the timing of DC, duration of hospital stay and functional outcomes. Results: Total number of patients n(%) - 14; Age (Mean±SD) - 43.42±16.68 years; M:F - 3:11; Clinical features - headache 10 (71.4%), seizures 9(64.28%), focal deficit 4(28.57%), altered sensorium-9 (64.28%); Glasgow coma scale at presentation (range) 3-14; Day of DC from the admission (range) - 0-3 days; Radiological features: Involved sinuses superior sagittal sinus 9(64.28%), transverse/sigmoid sinus11(78.57%), straight sinus 2(14.28%), cortical veins 2(14.28%), deep venous system - 1 (7.14%), subarachnoid haemorrhage 2(14.28%), subdural hematoma 1(7.14%), hemorrhagic infarction 14(100%), mass effect 14(100%); Treatment with anticoagulation - 13(92.85%); Outcomes- duration of hospital stay (Mean±SD) - 12.21±5.93 days, death within 90 days - 5(35.7%), 90-day mRS (0-2) 6(42.85%), 180-day mRS (0-2) 6 (42.85%), 12-month mRS (0- 2) 8 (57.14%); 24-month mRS (0-2) 8(57.14%). Discussion: -. Conclusion: Patients of CVT with large hemorrhagic infarcts and mass effects, who underwent DC, showed good short and long-term functional outcomes.

Abstract ID: 727

Delineating Radiological Signs of Ethambutol-Induced Optic Neuropathy: A Prospective Study of 80 Patients

Amit Kaur, Prabhjit Kaur, Karthik Mahesh, Basavraj Tigari, Aastha Kapila, Paramjeet Singh, Vivek Lal

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

E-mail: kaur.amit333@gmail.com

Background and Aim: Ethambutol, an essential component of ATT, has been associated with optic neuropathy as a potential side effect, which may result in irreversible vision loss. Radiological investigations of ethambutol-induced optic neuropathy (EtON) can aid in early diagnosis, prevention, and management of this condition. To identify specific imaging findings associated with the development of the EtON. Methodology: 80 patients with EtON were enrolled in this study. Detailed neurological, ophthalmological and radiological examinations were carried out. MRI Brain with T1W, T2W, FLAIR images along with pre- and post-contrast gadolinium- enhanced T1W MRI images and optic nerve cuts with thin coronal and axial T1 and T2 fat- saturated sequences, as well as sagittal T2 fat-saturated sequences were obtained along with a post-contrast T1W image. Results: Mean age ± SD of the patients were 45.71± 14.60. Majority of the patients were taking 15-20mg/kg/day weight-based dose of ethambutol which is the recommend dose. On radiological examination, hyperintensities of optic nerve and optic chiasma was observed in majority of the patients (31.66% and 36.67%) with atrophy of nerves and chiasma in 18.33% and 21.67% of patients. Unilateral involvement was noted in 39.39%, bilateral involvement was present in 11%. Diffusion restriction could be studied in a minority of patients. Radiological parameters were compared to study the association of neuroimaging with other clinical and investigational parameters. Discussion: Studying radiological predictors in patients with EtON through a prospective case series may help clinicians identify patients at high risk, initiate timely interventions, and improve overall patient outcome. Conclusion: The findings suggest that optic nerve and optic chiasmic hyperintensities on MRI are important indicators of optic nerve damage. Early recognition of these radiological findings can facilitate timely intervention and close monitoring of patients on ethambutol therapy to prevent irreversible vision loss.

Abstract ID: 728

Diagnostic Deadlock: A Case of Disseminated Tuberculosis with Neuroleptospirosis

Sanchit Chouksey, Rameshwar Chaurasia, Varun Singh, Pranjali Batra

Institute of Medical Sciences, Varanasi, Uttar Pradesh, India

E-mail: drsanchit92@gmail.com

Background and Aim: Neuroleptospirosis may present with altered sensorium, generalised tonic clonic seizures, headache, and focal neurological deficit. Common neurological manifestations of tuberculosis include meningitis, tuberculoma and arachnoiditis. We report a case of a young male who presented with features of acute meningoencephalitis. On further evaluation, he was also found to have disseminated tuberculosis. This is the first case report of coexistent neuroleptospirosis and disseminated tuberculosis creating diagnostic dilemma. The case emphasizes the importance of revising one’s own diagnosis and further work up once the anticipated therapeutic response is inadequate. Methodology: Case Report. Results: 19-year-old male presented with features of acute meningoencephalitis. He had history of low birth weight, delayed milestones and seizure disorder and was initially diagnosed as neuroleptospirosis. The patient had partial improvement in sensorium despite optimum treatment with doxycycline. He was later found to have disseminated tuberculosis as evident from his neuroimaging that showed basal exudates and hydrocephalous for which VP shunting was done. He later developed refractory seizures and succumbed to death. Discussion: In present case, Neuroleptospirosis was thought as an aetiology for acute meningoencephalitis. However, persistence of fever despite standard therapy of leptospirosis led to suspicion of either coexistent other disease or false positivity of IgM leptospira owing to cross reactivity. A homologue of mammalian cell entry (Mce) protein, normally found in Mycobacterium tuberculosis, is also present in pathogenic leptospira species that may explain the cross reactivity. In present case, refractory seizure was responsible for the poor outcome. Conclusion: This is the first case report of coexistent neuroleptospirosis and disseminated tuberculosis creating diagnostic dilemma. This case highlights the importance of revising the diagnosis and further work up once the anticipated therapeutic response is inadequate.

Abstract ID: 729

Efficacy and Safety of Injection Tenecteplase in 4.5 to 24 Hours Imaging Eligible Window Patients with Acute Ischemic Stroke (East-AIS)

Awadh Pandit, Arti Jatwani, Poorvi Tangri, Bhoomika Arora, M. V. P. Srivastava, Rohit Bhatia, S. S. Kale, Shailesh Gaikwad, Achal Kumar Srivastava, Ajay Garg, Leve S. Joseph, Deepti Vibha, V. Y. Vishnu, Rajesh Kumar Singh, M. R. Divya, Animesh Das, Ayush Agarwal

All India Institute of Medical Sciences, New Delhi, India

E-mail: akpandit.med@gmail.com

Background and Aim: Stroke is second major cause of disability and third most common cause of mortality in India. Rate of thrombolysis within 4.5 hours onset is<5% worldwide including India. This study is conducted to evaluate the safety and efficacy of injection Tenecteplase in AIS patients (with or without large vessel occlusion) in the time window 4.5 to 24 hours of stroke onset. Methodology: This study is prospective Randomized Controlled Trial approved by Institute Ethics Committee. Patients included will be male and female with age >18 years, baseline NIHSS>5, pre-stroke mRS<2 and CT-perfusion showing penumbra-ischemic core ratio >1.8, absolute difference in volume >10 ml, and ischemic-core volume <70 ml. A total of 100 patients will be enrolled, 50 each in Tenecteplase-arm (0.25mg/kg bodyweight; maximum 25mg) and placebo-arm in a ratio of 1:1. An independent data safety and monitoring board will have regular oversight of study. A total of ten patients have been randomized in the trial till date. Results: Unmasking of treatment allocation was done due to adverse events. Five patients were randomized in the active drug, Tenecteplase group and five patients in the control group. Intracranial haemorrhage (ICH) and symptomatic ICH (two in active group and one in control group), decompressive hemicraniectomy (three in active group and two in control group), infarct growth (one in active group and two in control group), death (two in active group) was observed. Discussion: It is an interim analysis shall be done again after 25 patients have been randomized in the trial. Conclusion: It is an ongoing trial with a total sample size of 100 patients.

Abstract ID: 730

Elusive Eyes

Sai Gullapalli, M. Jawahar

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: saiprajna_gullapalli@yahoo.com

Background and Aim: A 48 year old female homemaker presented with complaints of sudden onset of double vision, giddiness and giving away of right upper and lower limbs along with slurring of speech while she was lying on a couch and watching TV at 4 pm. She later had drooping of both eyelids and swaying to her left and left side weakness while walking during examination in ER. She was unable to say when her right side weakness improved. Methodology: At 6 am :- c/o drowsiness, Right eye ptotic, pupils-4 mm, sluggishly reaction Left eye ptotic (R>L), pupil -1mm, questionable reaction to light. EOM- head tilt towards left side, primary gaze - right eye central, left eye down and in. Movements on looking to right horizontal gaze only preserved in both eyes. Left UMN facial palsy. Other cranial nerves- normal. Left pronator drift Sensory - normal. Cerebellar- left impaired. At 9am:- not drowsy, Left pupil normal in size and reacting. Left ptosis resolved. Power on left- 4/5. Next day:- Left eye can move down and out. Rest as above. Results: MRI Brain - Acute infarct in medial aspect of right thalamus and right midbrain. Discussion: The presentation of I/L ptosis and ophthalmoplegia, c/l skew deviation and esotropic eye, c/L UMN facial palsy, c/l hemiplegia, c/L cerebellar involvement suggests the involvement of right paramedian midbrain and thalamus probably involving thalamo -subthalamic paramedian artery. The above presentation and evolution of findings doesnot fit to any of the classically named syndromes like Claudes, vertical one and half syndrome, plus minus etc. Conclusion: Cerebrovascular accidents involving posterior circulation can have wide variety of presentations and evolution of findings.

Abstract ID: 731

Embolic Stroke Due to Idiopathic Pulmonary Vein Thrombosis: A Rare Case Report

Nikitha Kailas, Y. Muralidhar, Christopher Johan, Subhendu Parida, M. K. Jagarlapudi, Shyam Jaiswal, Lalitha Pidaparthi, G. Abhinay, Manvitha Reddy, Shilpa Reddy

DNB Neuro Pursuing, Other

E-mail: nikkikailas95@gmail.com

Background and Aim: Embolic stroke due to pulmonary vein thrombosis (PVT) is quite rare. It is often underdiagnosed in neurological practice. PVT is commonly associated with malignancy, lung and left atrial tumours and surgeries, atrial fibrillation and radiofrequency ablation. It is very rarely idiopathic and hence this case report. Methodology: A 23 year old male presented with acute onset diplopia, vertigo, left sided weakness, dysarthria and left sided facial numbness of two days duration. He reported one episode of vertigo with double vision five months before this event and was treated with valproate at an outside facility. General examination was unremarkable. Neurological examination revealed left partial third nerve palsy, labial dysarthria, and left ataxic hemiparesis. NIHSS at admission was 5. MR Brain showed acute infarct in the left side of the midbrain and left thalamus. CT angiogram of intracranial and extracranial arteries did not reveal any steno-occlusive disease. Transthoracic echocardiography was normal. Transoesophageal echocardiography showed a hyperechoic mass projecting from the right inferior pulmonary vein. Cardiac MRI revealed an acute thrombus in the right inferior pulmonary vein. Loop recorder did not reveal any atrial arrhythmia. RT-PCR for SARS CoV2 was negative. HRCT Chest did not show any significant abnormality of pulmonary parenchyma. Work up for the hypercoagulable state was negative. He was treated with Dabigatran for six months with complete resolution of thrombus. Results: -. Discussion: -. Conclusion: PVT is underdiagnosed and should be looked for, especially in young patients with embolic stroke with no atherosclerotic risk factors.

Abstract ID: 732

Essential Thrombocythemia Presenting as Chronic Cerebral Venous Sinus Thrombosis and Intracranial Hypertension – A Case Report

Swapan Gupta, Prince Kumar

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi, India

E-mail: gupta.swapan@gmail.com

Background and Aim: Essential thrombocythemia is a myeloproliferative neoplasm characterized by clonal proliferation of megakaryocytes in bone marrow leading to excessive platelet production with a tendency for thrombosis and bleeding. About one-half of ET patients remain asymptomatic. Thrombosis of cerebral venous system is an extremely rare complication of essential thrombocythemia (ET). Here we report a case of chronic CVT due to essential thrombocythemia. Methodology: A 35 years old male presented with headache and bilateral progressive loss of vision of one-year duration. Fundus revealed Grade IV papilledema. VEP was not recordable in both eyes. MRI brain and CSF examination revealed features of raised intracranial pressure. Rest CSF examination was within normal limits. On further evaluation he was found to have partial thrombosis of superior, inferior, bilateral transverse, right sigmoid sinus and proximal right internal jugular vein with formation of collaterals suggesting chronic cerebral venous sinus thrombosis (CVT). Extensive evaluation for the cause of CVT did not reveal anything except he was found to have persistently raised platelet counts. Other secondary causes of raised platelet count were ruled out. Results: Provisional diagnosis of essential thrombocythemia was made. While he had not shown any significant response with anticoagulation alone, he was started on hydroxyurea. His headache dramatically improved with modest improvement in vision. Essential thrombocythemia was subsequently confirmed with a positive JAK2 V617F mutation. Discussion: Persistently unexplained raised platelets counts and dramatic response to anti-neoplastic agents like hydroxyurea suggested the diagnosis. Conclusion: Chronic CVT is a rare presentation of essential thrombocythemia. Clinician should be aware of this entity.

Abstract ID: 733

Exploring the Genetic Profile of Mitochondrial Disorders: A Single Centre Based Study

Arushi Saini, Yashu Sharma, Prateek Bhatia, Savita Attri, Priyanka Srivastava, Naveen Sankhyan

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

E-mail: doc.arushi@gmail.com

Background and Aim: Mitochondrial disorders are an inherited, heterogeneous group of disorders that arise due to dysfunction of the mitochondrial respiratory chain or oxidative phosphorylation. They are caused by mutations in either the nuclear DNA or mitochondrial DNA. Methodology: The children with a clinico-radiological suspicion of mitochondrial encephalopathy were enrolled and evaluated. A custom gene panel involving 57 genes responsible for mitochondrial encephalopathies was designed based and next generation sequencing was performed to identify the genetic variations. Results: We had enrolled 32 children with suspected mitochondrial encephalopathy. Out of 32 children, 26 (81.25%) had mutation in the genes responsible for mitochondrial encephalopathy. The most common gene carrying mutation was NDUFV1 (30.76%). Other genes involved were SUCLG1, NARS2, MTO1, IBA57, FASTKD2, COQ9, NDUFS8, ACAD9, KCNQ2, LIPT2, PDHA, BTD, GLDC, DLAT, NDUFV1, GCH1, C19orf12, SLC22A5, HIBCH, HTT, NDUFS3, NDUFAF2 and POLG. Majority of cases were males 73.07% (n=19/26). Mean age of presentation was 3.5 years (1 month – 12 yrs). The most common symptoms were global developmental delay (84.6%), regression of milestones (46%), extrapyramidal symptoms (54%) and seizures (28%). MRS data was available for 14 children and 8 children (57.14%) had high lactate peak. Brain MRI showed T2 hyperintensity in mid brain and basal ganglia. Discussion: Mitochondrial disorders are an inherited, heterogeneous group of disorders that arise primarily as a result of dysfunction of the mitochondrial respiratory chain or oxidative phosphorylation. They are caused by mutations in either the nuclear DNA (autosomal recessive or dominant inheritance) or mitochondrial DNA (maternal inheritance). The mitochondrial respiratory chain is central to cellular energy machinery and its dysfunction results in mild to severe metabolic encephalopathies and variable neurological manifestations. Conclusion: Mitochondrial disorders have variable neurological presentations across the childhood. Availability of genetic testing in developing countries has helped in confirming the diagnosis as well as understanding the complex phenotypes.

Abstract ID: 734

Eye and the Brain; Schilder’s Disease Presenting as Tumefactive Demyelination with Concomitant Balo’s Like Lesions

Sanjay Kordcal, Tarang Panchal

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi, India

E-mail: sraokordcal@gmail.com

Background and Aim: Tumefactive mass lesions is a rare presentation of demyelinating disorders. Here, we are presenting a case of 20 year old male patient who presented to our institute with subacute onset apathy, aphasia and encephlopathy with past history of bilateral sequential optic neuritis subsequently diagnosed to have Schilder’s variant of Multiple Sclerosis. Methodology: case report of schilder disease. Results: MRI was reviewed, and a fresh MRI Brain was done which demonstrated Bilateral hyperintensities in periventricular white matter with irregular concentric hyper and isointense layers. Discussion: A 20 year old male with no previous comorbidities presented with complaints of blurring of vision associated with color desaturation 2 months back which was diagnosed as bilateral optic neuritis. Patient had received low dose steroids outside and had partial improvement in his symptoms. After 2 months, patient developed subacute onset right hemiparesis, apathy and motor aphasia which progressed over 2 weeks. Examination demonstrated. in view of the subacute onset of focal neurological deficit and previous attack of optic neuritis, a provisional diagnosis of recurrent demyelinating disease was kept. The previous MRI was reviewed, and a fresh MRI Brain was done which demonstrated Bilateral hyperintensities in periventricular white matter with irregular concentric hyper and isointense layers. CSF analysis was non-inflammatory alongwith negative Oligoclonal Bands. Antibodies to Aquaporin-4 and MOG were negative. After ruling out infections, in view of presumed demyelinating event, patient was started on IV MPS Pulse followed by sequential IV Immunoglobulin. Patient had improvement in his weakness and his word output. At the time of last follow up, patient is ambulatory on his own with significant improvement post treatment. A retrospective diagnosis of Schilder’s disease with concomitant Balo’s like lesions was kept. Conclusion: This case Highlights the spectrum of Rare variant of the multiple sclerosis with an aggressive presentation and characteristic imaging findings.

Abstract ID: 735

Fulminant Iih: Treading Uncharted Waters (A Case Series)

Subhadeep Mandal, Samar Biswas, Arijit Roy

Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

E-mail: 1knite@gmail.com

Background and Aim: Fulminant IIH is a severe form of IIH characterized by sudden symptoms and signs of intracranial hypertension, including rapid vision deterioration within a few days (within 4 weeks).It requires urgent treatment as a medical emergency. In this paper we report 3 cases of Fulminant IIH presenting with rapidly progressive visual loss. Methodology: We had three cases: a 34-year-old non-obese female presented with severe headache, painless bilateral dimness of vision, vomiting, and rapid progression to near blindness within 48 hours. The other two cases were 29 and 30-year-old obese females with moderate headache, double vision, transient visual obscuration, and rapid development of severe visual dimness resulting in perception of hand movements within 13 days. Results: All three cases showed grade 4 papilledema, enlarged blind spots with inferior hemifield defects on perimetry, and a constellation of tortous optic nerve, flattened posterior aspect of globe on MRI with an additional finding of partially empty sella in the 1st case. OCT revealed a thickened retinal nerve fibre layer in all the patients along with prolonged P100 latencies on VEP. CSF opening pressure was greater than 30cm of water in all cases, and venography revealed transverse sinus stenosis in all three, with bilateral stenosis in the first case. Due to lack of consent for surgery, the patients were promptly treated with acetazolamide and temporary CSF drainage, resulting in significant improvement in vision and headache. Discussion: Rapid recognition of fulminant IIH is of utmost critical importance and can be pinned down on the basis of certain characteristic findings. Hence without appropriate triage and intervention the patient is at risk of profound permanent visual loss. Conclusion: An anticipation of delay in active surgical intervention may prompt rapid initiation of medical therapy and temporary CSF drainage which arrests the irreversible vision loss.

Abstract ID: 736

GFPT1 – Congenital Myasthenic Syndrome: A Case Series of 2 Brothers

Chitrangada Yadav, Ayush Agarwal, Divyani Garg, Divya M. Radhakrishnan, Achal Srivastava

All India Institute of Medical Sciences, New Delhi, India

E-mail: ychitrangada@gmail.com

Background and Aim: Case series of two brothers with glutamine-fructose-6-phosphate transaminase 1 (GFPT1) gene mutation causing congenital myasthenic syndrome (CMS). Methodology: Nerve conduction study (NCS) ,Repetitive nerve stimulation test (RNST), Illumina next generation sequencing (NGS) systems. Results: A 14 year old male patient presented with gradual onset progressive difficulty in standing from squatting position since 5 years, requiring support from hand while standing. Since 2 years there was difficulty in lifting arms above shoulder. There was no history of distal weakness of upper or lower limbs. His weakness increased as the day progressed and was more in the summer. He had associated mild drooping of the left eyelid since childhood. NCS did not show after depolarization and RNST was normal. His younger brother had similar complaints. A possibility of congenital myasthenic syndrome was considered and clinical exome sequencing was ordered. He was homozygous for a pathogenic variant in the GFPT1 gene. He was treated with pyridostigmine with complete symptomatic relief. Discussion: CMS comprise a group of genetically and clinically heterogeneous inherited disorders affecting the transmission of signals at the neuromuscular junctions.[1,2] The hallmark of CMS is fatigable and fluctuating weakness. GFPT1 mutations contribute to an impairment of neuromuscular transmission by a defect in glycosylation in these proteins.[3,4] Conclusion: In a patient presenting with progressive proximal weakness in the 1st or 2nd decade of life, genetic testing for CMS should be done in patients presenting with a limb girdle syndrome.

Abstract ID: 737

“Gray Area” Revealed by White Matter – A Case of Metachromatic Leukodystrophy

Swamy Kondapally, S. Sakthi

DM Neuro Pursuing, Other

E-mail: swamykondapally2@gmail.com

Background and Aim: Metachromatic leukodystrophy (MLD) is a lysosomal storage disorder caused by mutations in the ARSA gene. Traditionally divided into three forms: late infantile, juvenile, and adult. Adult metachromatic leukodystrophy, which has onset in late adolescence through adulthood, is characterized by a slow progression of neuropsychiatric symptoms. Here we report a case of MLD who presented with progressive dementia without other neurological deficits. Methodology: A 47-year gentleman with no vascular risk factors, presented with 3 years duration of behavioral disturbances in the form of apathy, disinhibited, and aggressive behavior with executive dysfunction followed by recent and remote memory disturbances. His son had an intellectual disability and died at 14 yrs of age and was not evaluated. The examination suggested a behavioral variant of FTD, with other neurological examinations being normal. Results: MRI brain showed diffuse T2/FLAIR hyperintensity in bilateral frontal, parietal white matter with tigroid pattern and sparing of subcortical U-fibres, suggestive of MLD. The diagnosis was confirmed by the reduction of ARSA activity (15nmol/h/mg, normal values: 78–180), Genetic report is pending. Discussion: MLD is an autosomal recessive lysosomal disorder with typical onset in the first years of life. It is caused by mutations in arylsulfatase A (ARSA) or, exceptionally, in prosaposin (PSAP) which determine sulfatide accumulation in myelin cells leading to central and peripheral demyelination. The rare adult variant usually presents in the twenties with progressive behavioral and cognitive decline associated with varying degrees of pyramidal, cerebellar, and peripheral nerve involvement. Our case presented in the fifth decade of life with progressive dementia alone without other neurological deficits and vascular risk factors. Conclusion: This case highlights the need to include adult-onset leukodystrophies in the differential diagnosis of a patient presenting with behavior disturbances, especially in the background of unexplainable periventricular white matter changes.

Abstract ID: 738

GM1 Gangliosidosis - Infantile Type – Rare GLB1 Splice Mutation c.397-2A>G - A Case Report

Tariq Harris, M. Rajasekaran, N. Kannan, E. Arunraj, R. Kishore

DM Neuro Pursuing, Other

E-mail: tariqharris7@gmail.com

Background and Aim: GM1 gangliosidosis is an autosomal recessive lysosomal storage disease caused by deficiency of the lysosomal hydrolase, acid beta-galactosidase. The infantile form is characterized by progressive organomegaly, dysostosis multiplex, dysmorphic facies, and progressive neurologic deterioration within the first year of life. A variety of cutaneous signs have been described in children with GM1 gangliosidosis. Here we describe an infant with GM1 gangliosidosis with ventral and dorsal distribution of Mongolian spots with rare splice mutation. Methodology: Case of 8-month-old male patient born to non-consanguineous parents presented with global developmental delay, syndromic facies. Ophthalmology examination showed cherry red spot but no corneal clouding. He had hepatosplenomegaly, congenital hydrocele, hypotonia. In addition, large hyper pigmented, well demarcated macules. Xray shows bullet shaped phalanges. Results: Suspecting GM1 gangliosidosis in view of presence of cherry red spot, cutaneous markers, whole exome sequence was done which showed pathogenic variant mutation in gene GLB1- variant c.397-2A>G Exon 3, this is a splice mutation and Allele frequency is only 1-2%. Discussion: Clinical and genetic test evidence supported the diagnosis of GM1 gangliosidosis type 1 in our patient. He had numerous and diffusely distributed hyper pigmented lesions which is an unusual presentation for typical Mongolian spots. Affected infants who survive beyond 12 months usually exhibit decerebrate rigidity. Seizures can occur, generally during the later stages of disease. Death usually occurs by 2 years of age from respiratory failure and bronchopneumonia. Conclusion: GM1 GANGLIOSIDOSIS infantile variant is very rare genetic disorder even though at present no approved treatment available, but treatment including enzyme replacement therapy, gene therapy clinical trials are showing promising results. Our patient had one of the rarest splice mutation variant c.397-2A>G.

Abstract ID: 739

Guillain Barre Syndrome: An Unusual Manifestation of Scrub Typhus Infection

Mukund Agrawal

Institute of Medical Sciences, Varanasi, Uttar Pradesh, India

E-mail: mukundagrawal010@gmail.com

Background and Aim: Guillain Barre Syndrome (GBS) is an autoimmune acute polyradiculoneuroathy manifesting as a rapidly evolving areflexic paralysis with or without sensory & lower cranial nerve involvement. Scrub typhus is a common vector-borne disease caused by Orientia tsutsugamushi. Common neurological manifestations include meningoencephalitis & aseptic meningitis. Here, we report GBS (AMSAN variant) as an unusual neurological manifestation of scrub typhus. Methodology: Case report. Results: A 72 years male patient presented with intermittent fever with yellowish discoloration of eyes & urine for 20 days, tingling sensation in both lower limbs for 15 days & progressive ascending weakness of all 4 limbs for 5 days. Examination revealed icterus, weakness in all four limbs (MRC grade 4/5), global areflexia, impaired pinprick & temperature sensation till bilateral ankle joints, impaired joint position & vibration sensation in bilateral upper limbs till wrist joints & in bilateral lower limbs till knee joints with positive Romberg’s sign. There was no cranial nerve involvement. He had normal complete hemogram, serum potassium & CK levels with deranged S.bilirubin (6.4 mg%), SGOT (230 IU/L), SGOT (560 IU/L), INR (2.28). IgM Scrub typhus was positive. Dengue, malaria & Leptospira serology were negative. NCS showed sensorimotor axonal involvement in all 4 limbs. CSF showed albumino-cytological dissociation. Due to economic constraints IVIG could not be given. He improved with doycycline & pulse Methylprednisolone. Discussion: There are few case reports & series of GBS associated with Rickettsia conorii, Rickettsia rickettsii & Orientia tsutsugamushi. Pathogenesis of GBS may be due to molecular mimicry, toxins, or immune dysregulation. In our patient symptoms developed 5 days after onset of fever & improved with doxycycline & methylprednisolone. Conclusion: Although rare, but scrub typhus may precipitate Guillain barre syndrome. It should be searched for when GBS presents with systemic signs & symptoms suggestive of scrub typhus infection.

Abstract ID: 740

Hallucination - A Bird’s Eye View

S. Arun, N. Thamil, A. Ravi, R. Lakshmi

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: arunsaravanan@gmail.com

Background and Aim: Creutzfeldt-Jakob disease (CJD) although rare, is the most common of the human prion disease.Rapidly progressive mental deterioration and myoclonus are the two cardinal clinical manifestations of sCJD.We present a case of CJD who had initial predominant visual symptoms. Methodology: 61 year old male with previously no comorbidities had developed acute onset progressive neurological symptoms with giddiness before 14 days followed by swaying to both sides while walking,diminution of vision in both eyes, intermittent involuntary posturing of both upper limbs (right > left) with twitching,behavioural disturbances in form of paucity of speech,confused talking,not recognising family members,decreased sleep,self talking with formed visual hallucination,loss of interest for basic activities,involuntary micturition,agitated behaviour followed by decreased responsiveness.Patient was intubated and mechanically ventilated because of the poor sensorium.The Patient had complained of poor vision in both eyes for the last 6 months and underwent cataract surgery in the right eye,but had persistent visual symptoms. Results: On examination,the patient had decreased vestibulo ocular reflex,neck rigidity,bilateral upper and lower limb rigidity,flexion withdrawal to pain stimuli in all four limbs,brisk deep tendon reflexes,bilateral extensor plantar, dystonic posturing of both upper limbs and right lower limb with no release reflexes.The Patient had a progressive disease course and developed akinetic mute state and succumbed after 10 days of admission.MRI Brain showed T2 FLAIR hyperintensities in bilateral basal ganglia and bilateral medial temporal,occipital and parietal lobes. Discussion: A Probable diagnosis of CJD is made for the patient based on clinical manifestations and typical MRI findings.An infrequent variant of CJD with early prominent visual symptoms is called the Heidenhain variant which represents represents 10-20% of sCJD cases. Visual symptoms include visual field restriction,visual blurring,cortical blindness,visual hallucination,visual perception deficits and cortical blindness. Conclusion: In patients with visual disorders of unclear origin and signs of dementia,the differential diagnosis of the Heidenhain variant of CJD must be taken into consideration.

Abstract ID: 741

Unusual Manifestations of a Known Devil: A Case Series

Ananya Sengupta, Subhadeep Bannerjee, Avik Mukherjee, Jayanta Roy

Vardhman Mahavir Medical College, New Delhi, India

E-mail: annesgpt@gmail.com

Background and Aim: Crutzfield-Jacob disease (CJD) is a uniformly fatal neurodegenerative disorder caused by the transformation of the endogenous PrP (prion-related) protein into an abnormal conformation called the prion. It usually presents as a rapidly progressive dementia over weeks to months with behavioral and personality changes, motor symptoms, incoordination, visual or constitutional symptoms. Survival ranges from 6-18 months. We present two unusual cases of probable CJD with acute onset and unusually rapid deterioration. Methodology: NA. Results: Case 1: A 69 year old gentleman presented with acute onset left sided hemiparesis with focal myoclonic jerks, gait ataxia, progressive generalized rigidity and attention deficit, followed by left focal seizures with secondary generalization. MRI Brain at onset of symptoms showed true diffusion restriction in right caudate nucleus. Within two weeks, the patient became drowsy, with EEG showing 1 Hz triphasic slow waves. Repeat MRI Brain showed right parietal cortical ribboning. CSF for 14-3-3 protein was positive. In the absence of any reasonable alternative diagnosis, the patient was diagnosed as a probable case of CJD. He was tracheostomised, non responsive to commands and discharged to home care. Case 2: A 64 year old gentleman presented with acute onset disinhibited behavior, executive dysfunction and delusions followed by progressive gait ataxia, multifocal myoclonus, focal seizures and left spastic hemiparesis over next 3 weeks. MRI Brain initially showed true diffusion restriction in right caudate nucleus, which rapidly evolved into a cortical ribboning pattern. EEG showed 1.5Hz triphasic slow waves. CSF autoimmune panel was negative, while CSF 14-3-3 was positive. The patient became progressively drowsy, showing no response to a therapeutic IVIG trial. He was diagnosed as a case of probable CJD and discharged to home care post tracheostomy. Discussion: NA. Conclusion: CJD should be considered as a potential differential diagnosis even in acute stroke-like or psychosis-like presentations.

Abstract ID: 742

Unusual Presentations of Cerebral Dural Arteriovenous Fistulas

Aamna Maniyar, Rakesh Singh, Narayan Deshmukh

Grant Medical College and JJ Hospital, Mumbai, Maharashtra, India

E-mail: amaniyar1991@gmail.com

Background and Aim: Cerebral dural arteriovenous fistulas (AVF) involve abnormal communications between meningeal arteries and venous sinuses. Incidence is 10-15% of all intracranial vascular malformations. Common symptoms include headache, pulsatile tinnitus, visual disturbances, stroke like episodes. However unusual presentations can occur posing a diagnostic challenge. Methodology: A multicentric restrospective observational study was done over three years. Data analysis was done for 107 patients of cerebral dural AVF diagnosed with digital subtraction angiography (DSA) and treated by endovascular embolization. Post operative follow up was done over 3-6 months. Results: There were six unusual presentations in our cases which included: 1. Fifty five year male presented with rapidly progressive cognitive decline over one year 2. Sixty five year male with subacute history of slowness of activities, tremors and gait difficulty suggestive of parkinsonism 3. Forty year female complained of acute unilateral facial pain, was on multiple neuropathic pain management for trigeminal neuralgia 4. Fifty year male presented as isolated tongue atrophy and considered as hypoglossal neuropathy 5. Thirty five year male presented with acute unilateral red eye 6. twenty seven year male came with acute CSF rhinorrhea. Discussion: Dural AVF is a rare treatable neurological condition. Arterialization of veins leads to venous hypertension and congestion resulting in ischemia causing various presentations. Catheter angiography is the gold standard for diagnosis. With endovascular embolization, most lesions are cured. Our cases showed significant reduction in their symptoms with post operative clinical improvement over 6 months. Conclusion: Natural history of dural AVF suggests it is aggressive. Delay in diagnosis and treatment can result in irreversible neuronal damage. This indicates need for prompt diagnosis and treatment since it is curable.

Abstract ID: 743

Vasculitic Neuropathy and Charcots Foot

Vishnu Sravya Vasipalli

DNB Neuro Pursuing, Other

E-mail: reddysravya21@gmail.com

Background and Aim: This is a case report about an uncommon presentation of vasculitic neuropathy with charcots joint. Vasculitic neuropathy corresponds to the occurrence of vasculitis at the level of vasa nervorum,resulting in ischemic damage of the peripheral nerve and axonal degeneration. There are three patterns of presentation: Mononeuritis multiplex- the most common presentation, Asymmetric polyneuropathy and Distal symmetric polyneuropathy. Methodology: Case Presentation - 42 year old female k/c/o Diabetes Mellitus since 1year presented with complaints of non traumatic swelling of left foot which progressed over 10 days. O/E there was edema, non tenderness on foot. Her HbA1C – 10.2%, she was managed conservatively with antibiotics and insulin. But there was worsening of swelling over the next 10 days with decreased sensation. MRI Foot has shown Lisfranc fracture of 2-5th tarsometatarsal joints with extensive edema in the foot extending till the ankle- Features s/o Charcots joint. Patient was advised NCS but didn’t follow up. She presented after 2 months with ulceration over foot. NCS has shown motor and sensory axonal neuropathy of lower limbs, sensory neuropathy in upper limbs. Relevant Blood investigations for neuropathy were uneventful except elevated CRP. Her ulceration has worsened, Left Below Knee Amputation was done. Left Sural nerve biopsy was done and shown features s/o Vasculitis-focal sparse perivascular lymphocytic inflammation around small vessels in epineurium. Endoneurium shows severe loss of nerve fibres with few hyalinised blood vessels. Meanwhile the whole genome sequencing has shown VUS of HSAN Type IV. Results: The diagnosis is considered as Vasculitic neuropathy presenting as Charcots Joint. Discussion: Charcots joint is an arthropathy affecting the lower extremity of patients with established peripheral neuropathy. It is characterized by bone and joint fragmentation of the foot and ankle. The most common etiology are Diabetic Neuropathy, Spinal cord injury, leprosy, chronic alcoholism, syphilis, poliomyelitis, syringomyelia. But there are no case reports in the literature till date with vasculitic neuropathy as a cause of charcots joint. Conclusion: This highlights Charcots Neuroarthropathy as a presentation in Non systemic Vasculitic neuropathy.

Abstract ID: 744

Vogt Koyanagi Harada Syndrome: A Case Report

K. S. Parvathy, S. Balasubramanian, E. Umamaheshwari

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: ksparvathysaneesh@gmail.com

Background and Aim: Vogt Koyanagi Harada (VKH) is an inflammatory disorder that typically presents with bilateral choroiditis and exudative retinal detachment. This condition may be missed by neurologists unless there is a high index of clinical suspicion . Methodology: 24 year old lady presented with history of redness of left eye following a trivial trauma of 1 month associated with headache (moderate intensity, throbbing type) and pain on moving eyeballs. Pain and redness subsided after 1 week spontaneously but recurred after 1 week. She had intermittent similar episodes in right eye followed by left eye .Also she developed fullness of right ear and hearing impairment in the right ear followed by left ear. Results: She had normal BMI ,normal visual acuity with Koeppe’s nodules in bilateral iris and bilateral papilledema. CSF profile normal with normal opening pressure. Pure tone audiogram showed bilateral sensorineural hearing loss. Automated perimetry normal. OCT showed bilateral disc edema with serous peripapillary retinal detachment and subretinal fluid collection. Fundus fluorescein angiography showed leaks in retinal pigment epithelium. Serology, vascultic profile and neuroimaging normal. She was treated with IV steroids and improved symptomatically. Discussion: VKH is an inflammatory disorder thought to be due to aberrant T cell mediated immune response directed against a self antigen on melanocytes. Conclusion: VKH is a rare treatable condition that may escape detection by a neurologist due to the normal CSF and imaging findings and this case emphasizes a thorough fundoscopy in patients presenting with headache and disc edema.

Abstract ID: 745

Young Male Presenting with Wilsons Disease and Charcot Marie Tooth Disease -1

R Siddharthan, J. Manickavasagam, G. Balaji, S. Hariharan

DM Neuro Pursuing, Other

E-mail: siddhu.1088@gmail.com

Background and Aim: Recognition of Wilsons disease is sometimes difficult because of it’s diverse manifestations. Peripheral Neuropathy is usually seen in later stages of the disease, but here we present rare presentation of Wilsons disease and Charcot Marie Tooth disease in a same person in young age. Methodology: CASE REPORT. Results: Coexistence of Autosomal Recessive Wilsons disease and X linked dominant Charcot Marie Tooth disease -1 proven by genetics in a young male. Discussion: 22 year young male presented with history of difficulty in walking , holding objects since 5 years ,frequent falls, involuntary movements of both hands since 3 years. -On Examination, Patient had slow saccades, Kayser-Fleischer rings present in both eyes with speech dysarthria. Patient had hypertonia of all four limbs, Positive cerebellar signs, intentional tremors with dystonic posturing of hands, Pes cavus both feet, Bilateral tendoachilles contractures, hammer toe with wide based ataxic gait and Rhomberg’s test positive with graded sensory loss of pain and temperature below hip , impaired joint position, vibration sensation below shoulder. - Blood Investigations revealed low serum ceruloplasmin with normal copper values with deranged liver function tests. -Ultrasound abdomen showed altered echotexture of Liver -Magnetic Resonance Imaging of Brain had features suggestive of Wilsons disease . Nerve conduction study of all four limbs showed evidence of primary demyelinating neuropathy with secondary axonopathy. - Genetic Sequencing showed pathogenic genes positive for Wilsons disease and Charcot Marie Tooth disease-1. Patient symptoms improving with D Pencillamine and Pyridoxine treatment and patient is on regular follow up. Conclusion: Clinical presentation of peripheral neuropathy due to Charcot Marie tooth disease -1 in a young male with features of Wilsons disease both of which is proven by genetics is indeed a rare combination, hence we report this case of Wilsons disease and Charcot Marie tooth disease in a young male.

Abstract ID: 746

Zuckerguss Sign – Sugar Coated Brain in a Case of Lung Adenocarcinoma

Rohit Keshav, Dinkar Kulshrestha, Ajai Singh, Pradeep Maurya, Abdul Qavi

DM Neuro Pursuing, Other

E-mail: rohitrevealed@gmail.com

Background and Aim: Meningeal carcinomatosis (MC) also called leptomeningeal carcinomatosis/neoplastic meningitis is defined as the spread of tumour cells within the leptomeninges and the subarachnoid space. It is seen in approximately 5% of patients with lung cancer. MRI appearance of extensive posterior fossa involvement with enhancement of leptomeningeal spaces of cerebellar foliae and brainstem can give the sugar-coated appearance or Zuckerguss pattern. Methodology: 61-year-old female came with presenting complaints of headache, giddiness and tinnitus for 1-1.5 months with acute onset confusional state for 10 days and vomiting for 1 day before admission. There was associated history of transient improvement in symptoms noted when patient was given pulse steroids elsewhere. Examination revealed no significant abnormality except left sided cerebellar findings. Results: Blood investigations and CXR were grossly normal except mild elevated neutrophilic predominant leucocyte counts and low sodium levels with raised Anti – TPO levels with normal TSH. MRI BRAIN revealed diffuse leptomeningeal enhancement with the classical Sugar-coated appearance especially along the occipital region and bilateral cerebellar folias with mild hydrocephalus. CSF analysis revealed elevated proteins and low sugar levels with normal cell counts. CSF bacterial culture as well as for common viral infections and cryptococcal antigen were negative. CSF malignant cytology came out to be positive. Detailed workup for malignancy subsequently revealed heterogeneously enhancing left upper lobe lung mass with mediastinal lymphadenopathy and wedge collapse of D2 vertebral body. CT guided FNAC and biopsy smears were suggestive of Adenocarcinoma of Lung. Discussion: Patient was managed with conservative measures and was referred to Radiation oncology Deptt. for further management. Conclusion: In an appropriate clinical scenario, MRI brain must be carefully analysed and a classical sugar-coated appearance of leptomeningeal enhancement should alert the clinicians for early diagnosis of meningeal carcinomatosis and plan appropriate management protocols.

Abstract ID: 747

Unraveling the Clinical Phenotype of Primary Dystonia: An Experience from a Quaternary Centre of India

Debjyoti Dhar, Riyanka Kumari, Vikram Holla, Nitish Kamble, Ravi Yadav, Babylakshmi Muthusamy, Pramod Pal

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: deb.dhar.india@gmail.com

Background and Aim: The comprehensive characterization of dystonia phenotypes in individuals with primary dystonia visiting movement disorder centres in India is currently lacking sufficient data. Methodology: Patients of primary dystonia were recruited from May 2021 to September 2022, after excluding acquired and secondary causes. They were evaluated using standard motor and disability scales [Global dystonia rating scale (GDS), unified dystonia rating scale (UDRS), Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS), when applicable]. The study was approved by the institute ethics committee. All patients were recruited after informed consent. Results: We recruited 65 patients (males=45, 69.2%), whose mean age at onset (AAO) and assessment (AAA) were 25.0 ±16.6 and 31.7±15.2 years, respectively. Majority of the patients belonged to early-adulthood onset (21 to 40 years) subgroup (n=23, 35.4%). The focal, segmental, generalized and multifocal groups comprised 23.1% (n=15), 13.0% (n=13), 55.4% (n=36) and 1.5% (n=1) patients, respectively. Neck was the most commonly affected first site across all the subgroups. Combined and complex phenotypes were more common in the generalized subgroup (n=13, 36.1% and n=9, 25%, respectively). Parkinsonism was the most common additional movement disorder reported (n=8, 12.3%) followed by chorea (n=7, 10.7%). Positive family history was observed in 19.4% (n=12) cases. Motor (GDS, UDRS, and BFMDRS) and disability scores were significantly higher in the generalized phenotype compared to the focal and segmental subgroups (p<0.001). There was significant negative correlation of motor scores with AAO (p<0.05) and AAA [BFMDRS (p<0.01), GDS (p<0.05) and UDRS (p<0.01)]. Patients with younger AAO (p<0.01) and AAA [BFMDRS-disability (p<0.05) and TWSTRS-disability (p<0.01)] had higher disability. Discussion: This study revealed the varied distribution of dystonia phenotypes and highlighted the differences in motor scores and disability levels among different subgroups of dystonia. Conclusion: Understanding the wide spectrum of presentations of primary dystonia can contribute to improved diagnosis and management strategies.

Abstract ID: 748

Tuberculous Myelitis: Rare and Rediscovered

Pramod Virbhadra Dhonde Dhonde

Grant Medical College and JJ Hospital, Mumbai, Maharashtra, India

E-mail: dhondepramod@gmail.com

Background and Aim: Central nervous system tuberculosis presents with meninigitis, hydrocephalus, spinal tubercuolmas and rarely cns vasculitis with headache followed by focal neurological deficits as clinical features. Spinal cord involvement is mainly bony involvement followed by cord compression in majority of cases. In era of modern diagnostics, still many questions remain unanswered in CNS TB. Isolated Myelitis as presentation is rare in spinal cord tuberculosis.To diagnose and differentiate tuberculous myelitis from various presentations of myelitis. Methodology: We are presenting two cases with isolated myelitis and one with myelitis and encephalitis. Diagnosis was based on particular CSF features and exclusion of other causes. Results: Two cases presented with long segment myelitis and one with encephalopmyelitis. Two cases showed very high proteins more than 1000 mg% and sugars were low below 20 mg%. One case was having CSF proteins upto 400 mg% and sugars below 20 mg%. Diagnosis was made by exclusion of other causes in one case and others were made based solely on CSF. CSF gene expert was negative all cases. Two cases significantly improved over six months with antitubercolous therapy for 9 months and steroids given for one month. One case not showed much recovery as diagnosis was delayed by almost 2 months and ATT started after 2 months. Discussion: Based on 1969 paper by Professor NH wadia and Darab Dastur, very high proteins and low sugars with apparently idiopathic myelitis cases, tuberculous myelitis can be considered as diagnosis in selected cases. Demyelinating diseases has not been described with such CSF picture in the current literature. To avoid delay in diagnosis and treatment as shown recovery significantly in two of our cases based solely on CSF scenario, TB myelitis to be considered. Still needs a larger data to prove this hypothesis. Conclusion: Tuberculous myelitis is a treatable disease with diagnosis based on very high proteins and low sugar. To avoid delay in diagnosis high degree of suspicion needed in selected cases. As this CSF picture is not seen in any of the CNS demyelinating diseases, with India having high prevalence of cases and recovery as shown our two cases with early diagnosis, further larger data can provide insight in better understanding of tuberculous myelitis.

Abstract ID: 749

Transient Ischemic Attack-there is more to it than What Meets the Eye

M. V. Sucharitha, Raghunandan Nadig, Grk Sarma, Thomas Mathew, Sagar Badachi, Sonia Shivde, Akshatha Huddar, V. Sindhu, Surahabhi Garg

St. Johns Medical College, Bengaluru, Karnataka, India

E-mail: sucharithavasu91@gmail.com

Background and Aim: Limb shaking transient ischemic attack (TIA) ,is a are manifestation of occlusive carotid disease.They are characterized by brief, arrhythmic, flailing or jerking movements of the leg or arm, and often misdiagnosed as focal seizures or movement disorder. The most important clinical clue is the precipitation of symptoms by maneuvers causing cerebral hypoperfusion. Focal cerebral arteriopathy (FCA) is a monophasic inflammatory stenosis most often affecting the supraclinoid internal carotid artery (ICA) or the proximal segment of the middle cerebral artery (MCA). It accounts for more than half of acute ischaemic stroke (AIS) cases in young children but is a less familiar entity among adults. Methodology: A 27 y/male patient with no known comorbidities presented with history of multiple episodes of transient jerky movements of right upper and lower limb with weakness lasting for 1-2 minutes with no sensory symptoms.The frequency of these events being 4-5 per day.Patient is well aware of the episodes. During the episodes patient is unable to communicate but able to comprehend.Resolution of symptoms was noted spontaneously.Clinically he was diagnosed to have limb shaking TIA. Results: MRI brain was done which was normal.MR Angiogram revealed a “Y” shaped stenosis involving the distal ICA and proximal MCA.Vessel wall imaging revealed circumferential enhancement of the vessel wall involving the stenosis. He was diagnosed to have focal arteriopathy. He was worked up for cause of focal arteriopathy. He improved with steroids. Discussion: -. Conclusion: Focal arteriopathy can present in adults with atypical manifesataion.In case of visualisation of tight large vessel “Y” shaped stenotic lesion on MR Angiogram in a young patient with no known co-morbidities, possibility of vasculitic etiology should be considered.

Abstract ID: 750

Tuberculoma of Spine Mimicking as a Intramedullary Tumour

M. Kumar, S. Ramu, M. R. Manivannan, P. K. Murugan

DM Neuro Pursuing, Other

E-mail: sathishmd89@gmail.com

Background and Aim: Spinal Tuberculosis presenting as intramedullary SOL is a rare that accounts for 1-2/1,00,000 patients with tuberculosis. Methodology: 28 year old male patient, presented with left arm and shoulder pain with numbness over right arm for 15 days .H/O low grade fever with evening rise of temperature . On examination, there is asymmetrical distal wasting left > right UL, with left UL spasticity and bilateral brisk DTR in UL with pain and temperature reduced over right arm and right half of trunk from C4 to T2 present. with b/l plantar withdrawal. Results: Investigation showed raised CRP. MRI Cspine shows enhancing intramedullary lesion C4-C5level over left side with surrounding edema from C3-T1. MRI chest shows multiple tree in bud opacities with granulomatous and mediastinal lymphadenopathy , Endoscopic ultrasound guided fine needle aspiration of mediastinal lymphnodes shows granulomatous inflammation and started on ATT. Patient symptomatically improving and he is on followup. Discussion: MRI is optimal tool for evaluating and diagnosis of intramedullary tuberculosis at an early stage and useful in followup. Usually conservative treatment is successful in achieving complete recovery over a period of 1 year. Surgical intervention is considered in those who are showing progressive defecits inspite of adequate medical management. Conclusion: Tuberculoma should be considered as differential diagnosis for intramedullary space occupying lesion in TB endemic countries irrespective of age.

Abstract ID: 751

Assessment of Clinical Outcomes in Transverse Myelitis Patients: A Retrospective Analytical Follow-up Study of Cases from 2018 to 2022

Pendem Bindu, Dhanya Sureddy

MBBS completed, Other

E-mail: pendemhimabindu@gmail.com

Background and Aim: Background and aims: Transverse myelitis (TM), a rare inflammatory condition affecting the spine, presents with rapid onset of bilateral motor, sensory, and bladder/bowel dysfunction. Recent studies are attempting to identify its improvement, worsening or conversion to multiple sclerosis, and the factors that determine these outcomes. The present study aims to assess the immediate and long-term outcomes of transverse myelitis and to determine the factors associated with them. Methodology: Methodology: the study involved retrospective review of hospital records of 30 patients diagnosed with transverse myelitis between 2018 - 2022, followed by telephonic interview to assess their present outcomes. Results: Results: Median age of the patients was 40 years (IQR = 30 - 48.5), with 53% males. About 76.7% had LETM. Onset was acute in 63.3%. Half (50%) of the patients had paraparesis. MRI spine showed involvement of long segment in 63.3% and short segment in 23.3%. At the end of treatment, 43.3% patients improved partially and 16.7% improved completely. At follow-up, nearly 30% of the respondents reported complete recovery while 8.3% reported worsening. Complete improvement at follow-up was higher among females, acute onset, and those treated with 1000mg intravenous steroid. One patient (3.33%), with acute onset TM, quadriparesis, bowel involvement, sexual dysfunction, and long spinal segment involvement, converted to Multiple Sclerosis at follow-up. 23.3% patients with initial partial improvement showed complete improvement at follow-up. Discussion: Contrary to our findings, Cobo Calvo et al (2002) reported conversion of idiopathic acute TM to MS but similar to our findings LETM was associated with poorer outcomes. Conclusion: Conclusion: Female gender, acute onset, and treatment with 1000mg intravenous steroid had higher chances of complete improvement at the time of follow-up. However, acute onset LETM cases can potentially convert to MS.

Abstract ID: 752

Diagnostic Challenge: Isolated Insidious Hiccups as CNS Tuberculosis Mimicking Medullary Glioma

Eluka Reddy, Sandhya Manorenj

DM Neuro Pursuing, Other

E-mail: e.mounikareddy20@gmail.com

Background and Aim: Central nervous system (CNS) tuberculosis presenting with isolated hiccups is exceedingly rare. We describe a case of a 67-year-old male with no known comorbidities who presented with intractable hiccups for 3 months and no other complaints. Initial treatment with antiemetics and muscle relaxants was ineffective, prompting further evaluation. This case highlights the diagnostic difficulties associated with isolated medullary lesions and emphasizes the importance of accurate differentiation from other conditions. Methodology: Comprehensive analysis included clinical and radiological features. Brain MRI, magnetic resonance spectroscopy (MRS), CSF analysis, and HRCT of the chest were conducted. Results: The MRI revealed a predominant right-sided space-occupying lesion in the medulla oblongata, adjacent to the right inferior cerebellar hemisphere. Contrast imaging demonstrated a conglomerate lesion with peripheral rim enhancement. MRS indicated an elevated lipid lactate peak suggestive of a tuberculous lesion. CSF analysis revealed elevated protein and adenosine deaminase (ADA) levels with low CSF glucose, and an elevated erythrocyte sedimentation rate (ESR). HRCT of the chest showed centrilobular nodular infiltrates consistent with pulmonary Koch’s. The patient responded well to anti-tuberculous treatment and steroids, with CSF culture confirming the diagnosis of CNS tuberculosis. Discussion: Isolated medullary lesions are rare in CNS tuberculosis, presenting a diagnostic challenge. Differential diagnoses include medullary glioma, meningioma, schwannoma, metastatic tumour’s and sarcoidosis. In this case, MRS and CSF analysis played pivotal roles in reaching the correct diagnosis. Conclusion: Our study reports a rare case of tuberculous lesions in the medulla oblongata, mimicking a medullary glioma. It emphasizes the necessity of maintaining a high index of suspicion for CNS tuberculosis and underscores the significance of appropriate diagnostic approaches for effective management. To the best of our knowledge, there are very few reported cases in the literature documenting such a rare presentation.

Abstract ID: 753

Three Cases of Primary Autonomic Failure - A Rare Entity

Rashmi Devaraj, Abdul Khan, Aliakbar Netterwala, P. Chandra

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: rashmid.326@gmail.com

Background and Aim: To study the Demographics, clinical spectrum, and outcomes of patients diagnosed as PAF. Methodology: Retrospective, Observational study conducted at Apollo Speciality Hospital, Jayanagar, Bangalore from 1st January 2022 to 31st March 2023. Patients diagnosed as PAF were included. Results: Here we report 3 cases of PAF. Patients were aged between 65 to 70 years. Two were male. Presenting features in all three patients was recurrent falls. Mean duration of symptoms was 40 months. Episode frequency falls ranged from once/week to once /day. Two patients had diabetes Mellitus and hypertension as comorbidities. All 3 of them had orthostatic hypotension. All 3 patients were examined thoroughly and had no features suggestive of extrapyramidal system involvement, or cerebellar signs. AFT in all 3 patients done from NIMHANS was abnormal. Fluoro DOPA PET was done in 1 patient and it showed no evidence of Parkinsonism. Patients were started on both non-pharmacological treatment and pharmacological treatment with fludrocortisone which was titrated on subsequent visits. All three patients are on regular follow up and have shown significant improvement in symptoms and are able to carry on their day to day activities and their occupation . Discussion: PAF is a rare neurodegenerative disorder characterized by Alpha Synuclein deposition in autonomic ganglia and nerves. Patients are typically middle aged or older and present with syncope or orthostatic hypotension. Other features like genitourinary, bowel and thermoregulatory dysfunction may also be present. Patients of PAF may progress to other synucleinopathies like Multiple system atrophy, Parkinson’s disease, Dementia with lewy bodies. Conclusion: PAF is a rare and debilitating illness with a few treatment options. A high index of suspicion is required for timely diagnosis. Long term follow up is required to monitor for progression to other synucleinopathies.

Abstract ID: 754

Headache Culminating in Parkinsonism

Shylaja Kaliappan, J. Kingsly, A. Sethuram, G. Shankar

Pursuing MD Medicine, Other

E-mail: shylgana@gmail.com

Background and Aim: Central pontine myelinolysis and extra pontine myelinolysis are two aspects of same disease osmotic demyelination syndrome, in recognition of the importance of osmotic shifts in its pathogenesis. Having same pathology, associations and time course but differ clinically. Methodology: 50-year-old female recently diagnosed hypertension presented with weakness of all 4 limbs and inability to speak since 3 weeks. Apparently normal 6 weeks ago she had headache and vomiting diagnosed to have Hypertension and put on drugs and low salt diet. Since her symptoms persisted, she was taken to nearby hospital and found to have hyponatremia of Na+ 122 and mild hypokalemia of 3.4 where correction was given with 3% NaCl. Results: A week later she had one episode of involuntary movements of all 4 limbs with posturing followed by tonic clonic movements, tongue bite and aggressive behaviour. After one day she developed weakness of all 4 limbs with increased tone with cog wheel rigidity with DTR’s exaggerated, plantars B/L flexor. Extrapyramidal signs include positive Myerson sign, slow saccades, broken pursuits, bradykinesia+. Discussion: CSF including autoimmune work up were negative. Blood c/s negative EEG showed diffuse slowing of background activity s/o electrophysiological dysfunction no sharp waves or epileptiform discharges MRI showed B/L symmetrical T2/FLAIR hyperintensities with diffusion restriction in putamen and caudate nucleus, B/L cerebellar hemisphere, superior frontal lobe sparing globus pallidus. Subsequent EEG did not show features suggestive of CJD. Conclusion: ODS usually presenting as pontine myelinolysis. Our Case was unique as there was extrapontine myelinolysis sparing globus pallidus involving corticospinal tract extrapyramidal connections and without the classical brainstem involvement.

Abstract ID: 755

Hemiakathisia in a Patient with Top of Basilar Syndrome

Arnesh Bhattacharya, Ashish Duggal, Jyoti Garg, Kuljeet Anand, Kamakshi Dhamija

DM Neuro Pursuing, Other

E-mail: jeet.arns@gmail.com

Background and Aim: Akathisia is an unvoluntary movement disorder characterized by sensory component of inner restlessness and consequent motor phenomenon in the form of repetitive stereotypic movements. Akathisia is most commonly drug induced though it is reported after thalamic strokes as well. We describe a case of unilateral akathisia developing after a Top of Basilar Syndrome. Methodology: A patient with history of posterior circulation stroke presented with feeling of irresistible urge to move the limbs of the left half of the body five months after stroke onset. These movements were, stereotypical, partially suppressible and would disappear during sleep. The phenomenology included flexion extension at shoulder and hands and fingers and foot tapping of left side. MRI brain was suggestive of bilateral thalamic lesion with a greater involvement of the left thalamus. Results: In view of the strong urge to move associated with stereotypic movements of the left side of body, a diagnosis of post stroke Hemiakathisia was made, and patient was started on benzodiazepine (clonazepam) with marked improvement in his symptoms. Discussion: Akathisia is one of the rarer movement disorders associated with thalamic strokes. Lesions of posterior thalamic nuclei could cause crosstalk of sensory and motor information leading to unvoluntary hyperkinetic movement disorders. Conclusion: Stroke is an important cause of movement disorders, hyper or hypokinetic, acute or delayed and there are complex underlying mechanisms responsible for changes in existing neurocircuits of movement pathways.

Abstract ID: 756

Hepatic versus Hypoxic Encephalopathy - A Diagnosis Delima Role of Neuroimaging

Baiakmenlang Synmon, Shri Sharma, Iada Tiewsoh

DM Neuro Completed, Other

E-mail: baiakmenlangsynmon@gmail.com

Background and Aim: Encephalopathy without a proper history from care giver possess a diagnosis challenge because of the wide range of etiology. metabolic or infective etiology always come to mind with a normal CT brain, however with a CT brain showing a diffuse cerebral oedema sparing the cerebellum points towards hypoxic encephalopathy whatever the preceding factor was. we here by reports a case report where MRI brain findings helps us in changing the etiological diagnosis of the case. Methodology: the patient was admitted to emergency department with a history of being found lying alone on the ground with vomitus around him. no significant past history except for ethanol abuse. patient was in altered sensorium with a GCS of 3/15, pupil mid dilated not reacting to light. His vitals were stable and afebrile. patient was intubated and shifted to ICU.CT brain done in emergency showed gross cerebral oedema so he was conservatively started on anti -odema, anti -epileptic and antibiotics. Results: patient routine investigation was normal, CSF analysis after fundus also did not show any gross abnormality. EEG was done which was also normal with no spike or slow wave. MRI brian was later done showing feature of hepatic encephalopathy. Discussion: Hepatic encephalopathy specifically refers to an encephalopathy occurring in patients with acute liver failure, a portosystemic shunt, or chronic liver disease. It is generally regarded as a reversible condition, although the long-term sequelae are being further studied. The CT brain was showing global cerebral oedema with sparring the cerebellum correlating with the diagnosis of hypoxic excephalopathy, later MRI brain showed global cerebral oedema sparing the peri-rolandic and occipital cortex of the cerebrum which was in favour of hepatic encephalopathy changing the treatment and prognosis of the patient. Conclusion: encephalopathy can poses a clinical challenge when all the routine investigation are normal. neuroimaging can aid in the diagnosis.

Abstract ID: 757

Horizontal Gaze Palsy with Progressive Scoliosis - A Case Report

Muhammed Shadique, Leema Pauline, Neeraj Elango, Jered Livingston, S. Mohinish

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: drshadique@gmail.com

Background and Aim: Horizontal gaze palsy with progressive scoliosis is a rare congenital disorder. We present a child with classical findings of HGPPS. Methodology: A 12 year old boy developmentally normal child born to non consanguinous parents presented with complaints of head nodding sideways and inability to move eyeballs sideways noticed by mother for the past 4yrs. No history of progression of symptoms. Child was able to look up and down without any difficulty. Results: On examination, child was conscious, oriented. Small amplitude sideways oscillations of the head was noted, bilateral pendular nystagmus was present, Scoliosis of thoracolumbar spine was present. Higher mental functions were normal.Cranial nerve examination showed absence of horizontal eye movements which was non maneuverable with preserved vertical gaze. Convergence was normal. Pupils were reacting well to light and visual acuity was normal with a normal fundus examination. There were no cerebellar signs. Other system examination was normal. MRI Brain showed the classical split sign of the pons and butterfly appearance of the medulla. Discussion: HGPPS is believed to be due to ROBO3 gene mutation in chromosome 11q.ROBO3 mutations may disturb brainstem morphogenesis by failing to promote decussation of long motor and sensory tracts in the pons and medulla.Impaired decussation of pontine oculomotor pathways could explain the absence of horizontal eye movements. MRI shows characteristic brainstem configuration with anterior and posterior midline clefts. The unusual anterior cleft at medullary level is accompanied by anteriorly flattened medulla which gives a butterfly like configuration in axial sections. The pons is flattened and the facial colliculi does not protrude into the fourth ventricle. Conclusion: We report this case as it is a rare congenital disorder and this case helps us to keep our possibilities wide open to arrive at a specific diagnosis.

Abstract ID: 758

Hyperosmolar Non-ketotic State Mimicking a Posterior Cerebral Artery Infarct: A Report of 2 Cases

Raja Kakollu

DNB Neuro Pursuing, Other

E-mail: yash_222284@yahoo.com

Background and Aim: Hyperosmolar nonketotic state is often seen in older people with poor Diabetic control. Field defects mimicking PCA infarcts are well known. We report 2 such cases. Methodology: Two patients who presented to the neuroophthalmology outpatient at Apollo Hospitals, Seshadripuram Bangalore, with an acute history of worsening of vision since 4 hours were recruited. Results: Both the patients had blood sugars more than 500. Discussion: Both patients presented to the neuroophthalmology outpatient at Apollo Hospitals, Seshadripuram Bangalore, with an acute history of worsening of vision since 4 hours. One patient had a history of left sided adversive movements followed by a secondarily generalized seizure. Clinical examination revealed a left homonymous hemianopia in both cases. MRI brain done, showed a diffusion restriction in the Right parieto occipital region in both cases with no changes on apparent diffusion resistance. T2 weighted hypointensities were seen in the right posterior head region corresponding to the diffusion restriction, suggestive of hyperglycemia. Blood sugars in both patients were in excess of 500. Treatment of hyperglycemia in both patients resulted in complete resolution of the field defect. Conclusion: It is imperative to look for hyperglycemia as a cause in patients presenting with acute onset field defects mimicking a PCA stroke, if they have T2 weighted hypointensities on MRI brain.

Abstract ID: 759

Hypoglossal Nerve Palsy – A Segmental Approach

Himaja Sanganabhatla

Osmania Medical College, Hyderabad, Telangana, India

E-mail: himaja.sanganabhatla@gmail.com

Background and Aim: Hypoglossal nerve palsy is an infrequent clinical presentation and usually occurs in combination with other lower cranial nerve palsies. It is divided into five segments – Medullary, Cisternal, Skull base, Carotid space and Sublingual. Each segment is usually affected by different disorders. Here, we present three cases of Hypoglossal nerve palsy due to involvement at different segments. Methodology: Three patients who came to outpatient department of Neurology with 12th cranial nerve palsy were evaluated by clinical history, detailed neurological examination, and investigated using routine blood work, Neuroimaging and other necessary investigations. Results: 1 – A 32 yr. old male presented with 9,10,12 cranial nerve palsy with atrophy of tongue with deviation to the left; clinical diagnosis was involvement of skull base segment of Hypoglossal nerve. MRI revealed a Glomus Jugulare tumour. 2 – A 35 yr. old female presented with 9,10,12 cranial nerve palsy with Horner’s syndrome with a hard mass in the neck; clinical diagnosis was involvement of carotid space segment of Hypoglossal nerve. CT Neck and FNAC confirmed Malignant salivary neoplasm with metastatic deposits. 3 – A 70 yr. old male presented with isolated 12th cranial nerve palsy with submandibular swelling; clinical diagnosis was involvement of sublingual segment of Hypoglossal nerve. PET CT and FNAC confirmed Squamous cell carcinoma of base of tongue. Discussion: Hypoglossal nerve palsy along with other cranial nerve involvement (9,10,11) can be due to infectious, inflammatory, malignant, metastatic and neurodegenerative disorders like motor neuron disease. Presence of associated features specific to these causes helps in planning further investigations and treatment protocols. Isolated hypoglossal nerve palsy is extremely rare and occasionally appears as the initial sign in extra or intracranial space occupying lesions, head and neck injuries or vascular abnormalities. Conclusion: Localizing a lesion to a particular segment narrows the differential diagnosis and helps in early management.

Abstract ID: 760

Idiopathic Intracranial Hypertension Associated with Cyclosporine Use in a Patient with a Plastic Anaemia

K. Priya, Sonali Bhattad

DNB Neuro Pursuing, Other

E-mail: drpriyachandran@gmail.com

Background and Aim: Idiopathic Intracranial Hypertension is characterized by raised intracranial pressure (>25 cm of H2O) with normal CSF composition and lack of intracranial lesion. The use of cyclosporine may lead to IIH in upto 35% of cases. We report a case of IIH secondary to the use of CsA after 2 years of therapy in a patient with aplastic anaemia, which resolved satisfactorily without any sequalae, after drug suspension. Methodology: 25 year old lady, diagnosed to have aplastic anaemia in January 2021 and started on oral cyclosporine, presented to our OPD with complaints of progressively increasing headache and diplopia since 1week. On examination, visual acuity , colour vision and visual field was normal. Extra ocular movements were full.Fundus examination showed bilateral papilledema. MRI Brain done showed features of IIH. In view of thrombocytopenia and high risk of infection, CSF study including measurement of opening pressure was withheld. Taking into consideration the clinical symptoms and radiological evidence, she was suspected to have IIH. Cyclosporine was temporarily stopped and she was started on oral acetazolamide following which her headache and diplopia completely resolved. Results: Papilledema resolved within 2 weeks after which she was restarted on low dose cyclosporine. Discussion: There are several etiologies such as recent weight gain, infections, endocrine changes and medication uses for IIH. For 2 decades, CsA has been reported as one cause of IIH. There are only few reports of IIH in patients on CsA after renal transplantation and none reported so far in a patient with aplastic anaemia. Conclusion: It is important to recognize the role that cyclosporine plays in raising intracranial pressure, especially in patients requiring immunosuppression. The basic treatment consists of either cessation of cyclosporine or reduction of dose with consequent improvement in the symptoms and decrease in ICP.

Abstract ID: 761

Internuclear Opthalmoplegia with Upbeat Nystagmus and Central Facial Palsy in Medial Medullary Syndrome-A Rare Case Report

K. V. Vandana, Lakshmanan Sankaranarayanan

DM Neuro Pursuing, Other

E-mail: vandana17.2011@gmail.com

Background and Aim: Internuclear opthalmoplegia and UMN facial palsy are rare occurrences in a case of medial medullary syndrome.Here we report a case of INO with upbeat nystagmus and UMN facial paresis in a case of medial medullary syndrome. Methodology: 55 years male who is a known diabetic and hypertensive came to us with complaints of giddiness and vomiting. He subsequently developed numbness and heaviness of left side of the body. 2 hours later he developed weakness of left side of the body. On examination, he was found to have right eye Internuclear Opthalmoplegia and upbeat nystagmus. He also had left central facial palsy, hemiparesis of left side and impaired vibration sense and joint and position sense on left side. Results: MRI brain revealed right medial medullary hyperintensity in the T2 weighted and flair sequences and showed diffusion restriction suggestive of acute infarct.MR Angiography was unremarkable. His four vessel doppler was normal. His ECHO showed concentric LVH with no RWMA with normal LV function and no clots. He was treated with antiplatelets, statins and other supportive management. Discussion: Central facial paralysis may result from a unilateral contralateral medullary infarction, suggesting that some of the facial corticobulbar fibers descend ipsilaterally before making a loop as low as medulla oblongata before decussating and ascending to the contralateral facial nucleus that innervates the perioral musculature. Upbeat nystagmus can occur due to dorsal extension of the lesion towards the medial longitudinal fasciculus or it can be because of lesion affecting the right nucleus intercalatus. Conclusion: Although diverse patterns of eye movements may be observed in lesions anywhere along the brainstem, medullary lesions mostly present various patterns of nystagmus and impaired vestibular eye movements without obvious ophthalmoplegia. In this case report ,we present a case of internuclear opthalmoplegia with upbeat nystagmus and UMN facial paresis in a case of medial medullary syndrome.

Abstract ID: 762

Intra Cerebral Haemorrhage as the Initial Presentation of Familial Hemophagocytic Lymphohistiocytosis

C. Leema Pauline, Neeraj Elango

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: leema_pauline@rediffmail.com

Background and Aim: Familial hemophagocytic lymphohistiocytosis, caused by a genetic mutation in one of the genes responsible for cytotoxic function of natural killer cells and cytotoxic T lymphocytes, usually presents as an acute illness with prolonged fever, cytopenias, and hepatosplenomegaly. Central nervous involvement as the initial presentation is rare. Methodology: Case summary. Results: A five-year old female child presented with intermittent fever, headache and vomiting for one month. There were no h/o seizures or ALOC. She was diagnosed as Tuberculous meningitis started on anti-tuberculous drugs with oral steroids and ventriculo-peritoneal shunt for hydrocephalus elsewhere. On examination, she was irritable, afebrile. Cranial nerves, tone, power, reflexes were normal. Neck stiffness was present. She had appendicular and gait ataxia. Other systems were normal. Perusal of MRI films revealed haemorrhage in both cerebellar hemispheres with mass effect on the fourth ventricle causing obstructive hydrocephalus. There was no meningeal enhancement. Tuberculous workup turned out to be negative. Coagulation profile was normal. MRA, MRV, CTA were normal. CSF analysis showed no cells, elevated protein, normal sugar. Vasculitic workup was negative. Repeat imaging showed resolving hemorrhage in the cerebellum and the child was discharged with anticonvulsants without any focal deficit. Six months later, she presented with acute onset of right hemiplegia. There was no fever, headache or vomiting. MRI brain showed an acute hemorrhage in the left capsuloganglionoic region with surrounding edema. Repeat coagulation workup including factor XIII assay was normal. Genetic mutation studies revealed a pathogenic mutation in perforin gene confirming a diagnosis of primary hemophagocytic lymphohistiocytosis (HLH). Child received steroids, etoposide and underwent hematopoietic stem cell transplant. She has residual right hemiparesis. Discussion: CNS involvement in HLH can be meningitis, ADEM like presentation, ring enhancing lesions and hemorrhage. Intracerebral hemorrhage as initial presentation is rare. Conclusion: Primary (familial) HLH should be considered as a possibility in cases of unexplained intracerebral hemorrhage.

Abstract ID: 763

Intractable Cough as an Initial Manifestation of Neurologic Wilson’s disease

Prakash Vellasamy, J. Manickavasagam, G. Balaji, S. Hariharan

DM Neuro Pursuing, Other

E-mail: thangamprakash_mmc@yahoo.com

Background and Aim: Wilson’s disease (WD) is a genetic disorder of copper metabolism. There is impaired biliary copper excretion, which leads to accumulation of copper in many organs mainly in liver, brain, and cornea. WD is known as a great imitator. Here, we present a case of proven neurologic Wilson’s disease, commenced only with intractable cough. Methodology: A 20-year female, developed intractable non-productive cough without fever or any other features of respiratory tract infection. Cough was not amenable to cough suppressants. Work up for the cough didn’t reveal any cause for the intractable cough. Subsequently, she developed subacute onset gradually progressive dysarthria with monotonous speech, slowness of activities, asymmetric tremors involving right hand. Clinical examination revealed hypophonic monotonous speech with symmetric cog-wheel rigidity at both wrists. The possibility of WD disease was contemplated with the onset of neurological findings. Results: Subsequent work-up revealed KF ring in both eyes by slit lamp examination and blood investigations were also positive for Wilson’s disease (Leipzig score was more than 4, diagnosis established). She was started on chelation therapy with D-penicillamine. Her speech was improving gradually and cough also got relieved. Discussion: Unusual isolated cough as an initial manifestation of neurologic WD, though rare, was reported in literature. It is hypothesized as a form of respiratory dyskinesia. Conclusion: Diagnosis of WD requires high index of suspicion, as the disease has got wide variety of presentations. Because, in contrast to many neurogenetic metabolic diseases, WD can be very effectively treated. Hence, we should be aware of its rare manifestations too.

Abstract ID: 764

Ischemic Stroke in Case of Scorpion Bite

Vikram Khardenavis, M. Manivannan

DM Neuro Pursuing, Other

E-mail: vikram_khardenavis@rediffmail.com

Background and Aim: A 54-year-old lady with no comorbidities, presented with scorpion bite on left hand. On shifting to hospital, her vitals were stable. She was immediately administered prazosin, intravenous antihistaminics, analgesics, diazepam, anti-inflammatory medications and intravenous fluids. Routine blood tests and coagulation profile were normal. 2 hours after admission, the patient developed dyspnea with palpitation. There was sinus tachycardia with hypotension. CKMB levels were elevated while Troponins was normal. 2-D echocardiography did not show any features suggestive of myocarditis. 6 hours after scorpion bite, the patient developed paucity of movements on the right side of the body with global aphasia. Methodology: clinical examination and MRI of brain with MRA was done. Results: MRI of brain showed large left MCA territory ischaemic stroke involving the cortical and subcortical areas. MRA showed complete non-visualisation of left ICA and MCA. Discussion: Pathophysiology of stroke in scorpion poisoning has been attributed to various mechanisms: 1.Autonomic storm induced sudden surge in blood pressure 2. Myocarditis induced cardioembolic stroke. 3. Venom induced DIC causing increased platelet aggregation. 4. (Venom induced vasculitis caused by endothelial damage. 5. Hypotension induced watershed infarcts. 6. Catecholamine excess induces increased endothelin resulting in vasoconstriction. The most likely mechanism of infarct might have been cardioembolic because: 1. The cerebral vascular event was preceded by tachyarrhythmia. 2. There is selective complete occlusion of ICA and MCA on the left side only, which is less likely to be due to vasospasm. Conclusion: Scorpion bite-associated stroke is a rare entity. It may manifest as intracranial bleed or ischaemic infarct. Voltage-dependent ionic channels are the target of scorpion venom. The case fatality rate in case of scorpion bite is between 3% and 22%.

Abstract ID: 765

Isolated Laughter in Parkinson Disease

Ankur Vivek, Vijaya Mishra, Abhishek Pathak

Institute of Medical Sciences, Varanasi, Uttar Pradesh, India

E-mail: ankurrock78@gmail.com

Background and Aim: Pseudobulbar affect (PBA) is characterized by uncontrolled episodes of crying and laughing which is associated with a variety of neurological diseases. Several neuroimaging and neurophysiological studies have reported that the cerebellum plays a key role in the development of PBA. Pathways from the cortex to the pons and cerebellum also are involved in affective and cognitive performance. Disruption of corticopontine-cerebellar pathways or cerebellum itself leads to impairment of emotional response. Methodology: A 69 years old male, with complaints of slurring of speech since 2008. The voice was hypophonic, low pitch and patient cannot speak the sentence in a flow. The speech output had decreased and had difficulty pronouncing consonants. Patient also complained of difficulty in walking from 2008. According to patient, there was difficulty in initiation of gait and the steps were short and shuffling with decreased foot clearance. There was decreased arm swing and used to walk with stoop posture. He also had difficulty in turning. Patient also complained of unwanted and excessive laughing since 2008. Patient attendant noticed that while having a conversation with someone, he used to laugh. He could not control the laughter. Whenever any bad news happened in the home, despite being sad, he would laugh. He would also have guilt so he would try to avoid those situations. Neither the patient nor the attendant gave history of unwanted or excessive crying. Results: Patient MMSE was 30/30, FAB-18/18. CN examination revealed slow saccades and broken pursuits .UPDRS III motor scale OFF score was 22, ON score was 44. There was 50% improvement. CNS-LS score was 20. Discussion: His routines were normal. MRI Brain suggestive of cerebral brain atrophy. Conclusion: PBA is a distressing neurological condition that is relatively common but underappreciated in the parkinsonian population. However, no one has reported the association of isolated laughter in Parkinson’s disease.

Abstract ID: 767

Knowledge Attitude and Practices regarding epilepsy and pathways to care of People with Epilepsy

Deepak Chadha, Sanjib Sinha, Ravindranath Mundlamuri, Ajay Asranna, K. Raghavendra, L. G. Vishwanath, Priya Thomas, K. S. Meena, Renibi Lepcha

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: deepakchadha1298@gmail.com

Background and Aim: Epilepsy is associated with rampant misconceptions, negative attitudes & stigma that hampers access to proper care. We aimed to study the KAP regarding epilepsy among PWE and the pathways of care they adopted to access care. Methodology: This prospective study was conducted at the Department of Neurology, NIMHANS, where 84 adult PWE were analyzed using a structured questionnaire, including a validated KAP questionnaire and details of first and subsequent referrals for understanding the pathway map for receiving epilepsy care. Results: Of the 84 PWE, 49 were male with a mean age of 30.7 +_ 5.2 years and a mean duration of epilepsy of 4.32 years. Among PWE, 25% were graduates, while 10 % were illiterate. About 46% belonged to the low socio-economic category. Misconceptions related to epilepsy were common; 41% believed epilepsy to be a mental health disorder, 12% felt that epilepsy is contagious, and some attributed it to a curse (15%) or possession by evil spirits (10%). A poor attitude towards epilepsy was noted; 45% believed that epilepsy had no treatment, 20% believed PWE should never marry & can never bear children (24%). Most PWE consulted a medical practitioner for epilepsy care (73%), among whom 26% were neurologists, while a minority sought care from faith healers (10%) & traditional medicine practitioners (7%). Correlation analysis of the choice of care pathway seeking faith healers/ non-medical practitioners showed a significant negative correlation with family income (r=-0.04, p=0.004) & a positive correlation with the duration of epilepsy (r=0.194, p= 0.0007). Discussion: Combined with result. Conclusion: We found that misconceptions related to epilepsy were prevalent among PWE despite seeking care in a tertiary hospital. Though most PWE sought modern medical treatment for initial epilepsy care in our study,those with a lower family income and higher duration of epilepsy were more likely to seek alternative modes of treatment.

Abstract ID: 768

Lentiform Fork Sign in Central Pres with Hellp Syndrome

Venkatesh Kondle, M. R. Manivannan, P. K. Murugan, C. Justin

DM Neuro Pursuing, Other

E-mail: venkatesh.kondle@gmail.com

Background and Aim: Posterior Reversible Encephalopathy Syndrome (PRES) is a clinic-radiological entity characterised by headache, seizures, altered mental status, visual disturbances occurring in predisposing clinical conditions such as hypertension, uraemia, autoimmune diseases, and neoplasms. Pathophysiology of PRES is controversial, dysfunction of cerebrovascular autoregulation and vasogenic edema mainly being incriminated. Methodology: 32 year old female G2P2L1 38 weeks of GA presented with 3 days history of B/L pedal oedema, headache, on examination BP was 160/100 and her labs were suggestive of hemolysis elevated liver enzymes, low platelet counts. The very next day one hour after delivery, she throwed one episode of GTCS; following which she was in postictal state. Results: CT showed bilateral symmetric hypodensity of basal ganglia. MRI showed hypointense areas on T1 WI, hyperintensity on T2WI, FLAIR consistent with vasogenic oedema involving symmetrically lenticular and caudate nuclei, with extension to internal and external capsule. After pregnancy interruption, intensive antihypertensive and supportive treatment, and recovering from HELLP syndrome and pre-eclampsia, patient experienced rapid, complete recovery. Follow-up MRI 3 weeks later showed resolution of previous findings. Discussion: Basal ganglia involvement was reported in around 10% of cases. This is an example of atypical central variant. In central variant, there is preferential involvement of brainstem, thalami and periventricular white matter with sparing of frontal, parietal and occipital cortex. Conclusion: Symmetric basal ganglia oedema with extensions to internal and external capsules, associated with some mass effect, surrounded by hyperintense rim delineating the lentiform nucleus, was previously described as the “lentiform fork sign”. This sign has been reported in acute uremic encephalopathies, and in metabolic acidosis. However, in our case no uraemia or metabolic acidosis was present, which makes this of particular interest.

Abstract ID: 769

Leukodystrophy Presenting as Frontotemporal Dementia

Sachin Adhav, Rahul Chakor

Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India

E-mail: drsachinadhav@gmail.com

Background and Aim: Frontotemporal dementia (FTD) is common cause of dementia. Mutations in some leukodystrophy genes namely TREM2, TYROBP, CYP27A1, CSF1R, NOTCH3 and TMEM106B can present as FTD. We present three patients who fulfilled clinical criteria for FTD, however next generation sequencing (NGS) detected CSF1R and NOTCH3 gene mutations. Methodology: We conducted comprehensive clinical, imaging and genetic analysis of three cases suspected to have FTD. Whole exome sequencing using NGS, and sanger sequencing was conducted. Findings were analysed with literature review. Results: Case 1 – 48 years old male presented with progressive language impairment with word finding difficulty and impaired single word comprehension. He fulfilled clinical criteria for semantic variant FTD. MRI revealed bilateral confluent white matter changes. NGS detected Notch 3 gene (NM000435.3, EXON 11) mutation. Case 2 – 32 years old female presented with behavioural disinhibition, executive dysfunction, and apathy. She fulfilled clinical criteria for behavioural variant FTD. MRI showed T2/FLAIR hyperintensities in periventricular region with corpus callosum thinning. NGS detected Notch 3 gene mutation (NM000435.3, EXON 25). Case 3 – 45 years old female presented with aggressive behaviour, executive dysfunction, perseveration, and social disinhibition. She fulfilled clinical criteria for behavioural variant FTD. MRI revealed T2/FLAIR hyperintensities with persistent restricted diffusion in bilateral frontoparietal white matter. NGS detected CSF1R gene mutation (chr5:149433963delC). Discussion: Our Patients didn’t have typical clinical features or MRI characteristic of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) NOTCH3 or hereditary diffuse leukoencephalopathy with spheroids (HDLS) CSF1R. However, their relatively young age with white matter changes on MRI prompted us to evaluate for genetic testing. Conclusion: Spectrum of FTD mimics is expanding. Clinical diagnosis of HDLS and CADASIL presenting as FTD is challenging. Recent advances of NGS technology have led to identification of rare gene mutations. This has helped in accurate diagnosis of these leukodystrophies.

Abstract ID: 770

Lighting Along the Tract: HIV-associated Pseudobulbar Palsy: An Illustrative Case Report

Arijit Pal, Debashish Chowdhury, Ayush Mohan

Govind Ballabh Pant Institute of Postgraduate Medical Education, New Delhi, India

E-mail: drariz1993@gmail.com

Background and Aim: Hyperintensity along the Corticospinal Tract has a wide differential diagnosis including but not limited to motor neuron disease, demyelinating disorder, leukodystrophy, chronic infections and toxic disorders. We are presenting the case of a 21 year old male who presented to our institute with rapidly progressive pseudobulbar palsy. Methodology: Case Presentation: The patient had a history of fever 3 months back following which he developed difficulty in speech, nasal regurgitation of food and inability to swallow over 3 months. He had high risk sexual behaviour. Examination revealed UMN type 5th, 7th, 9th, 10th and 12th nerve palsy along with subtle pronator drift in the Left Upper Limb. Patient was evaluated for the same and routine blood investigations were sent. MRI Brain demonstrated T2/FLAIR Hyperintensity along the corticospinal tract (Right>Left). HIV by ELISA came out to be positive. A CD4 count was sent with a value of 167 cells/µl. Patient was started on first line ART regimen (Dolutegravir + Lamivudine+ Tenofovir) along with prophylaxis for opportunistic infection. In view of the significant bulbar symptoms, the patient underwent Percutaneous Endoscopic Gastrostomy (PEG). Patient is currently in follow up with modest improvement in symptoms. Results: -. Discussion: -. Conclusion: This case serves to highlight two important points- first, in patients with hyperintensity along corticospinal tract on MRI, the importance of keeping a wide differential diagnosis and second, pseudobulbar palsy can be a neurological manifestation of HIV.

Abstract ID: 771

Limb Girdle Muscular Dystrophy like Presentation in Spinal Muscular Atrophy

Gaurav Chaudhary, Pawan Ojha

Grant Medical College and JJ Hospital, Mumbai, Maharashtra, India

E-mail: doc101gau@gmail.com

Background and Aim: Differential weakness is protype for a muscle disease, and splaying is one of the sign depicting differential weakness which is characteristic for lgmd, here we report three patients of spinal muscular atrophy presenting in differential weakness mimicking LGMD. Methodology: It is an observational study, done at tertiary care hospital with chief complaints of pure motor weakness in all four limb, and after detailed history and clinical examination were thought to be autosomal recessive LGMD but they had tremors in hand. Routine blood investigation and serum cpk level, EMG was done and clinical exome was sent. Results: The EMG study was consistent with neuropathic pattern suggestive of SMA. Clinical exome shows deletion in exon 7 of SMN1 gene. Discussion: Spinal muscular atrophy is a disorder of alpha motor neuron with phenomenology of progressive pure motor muscle weakness, atrophy with reduced tone, pattern of muscle weakness is symmetrical with proximal more than distal weakness with neurogenic tremors in hands and there is no differential weakness as found in LGMD i.e. “Hip-abduction sign” or splaying of thighs in which hip adductor is weaker than abductor.i.e. Differential weakness .Selective involvement and sparing of muscle is characteristic LGMD or myopathic pattern of weakness but not described in SMA. Conclusion: Though muscle diseases have a clinical phenotype of differential weakness i.e. splaying but this is not specific for muscle diseases and can be found in other condition such as SMA, as in our case. Neurogenic tremors in patients who present with myopathic pattern of weakness suggest SMA.

Abstract ID: 772

Lipidomics in Idiopathic Intracranial Hypertension: A Prospective Pilot Study

Kirandeep Kaur, Aastha Takkar, Karthik Mahesh, Chirag Ahuja, Basavaraj Tigari, Vivek Lal

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

E-mail: kiranworld99@gmail.com

Background and Aim: Idiopathic intracranial hypertension (IIH) is characterized by increased intracranial pressure (ICP) occurring predominantly in women with obesity without an identifiable etiology. It causes blindness (in up to 24% of individuals) and long-term disabling headaches. Recently, it has been reported that adipose tissue in IIH is transcriptionally and metabolically primed toward depot-specific lipogenesis. Moreover, serum lipid metabolite pathways were seen to be significantly increased in IIH. In this study, we present the diagnosis, investigation, and management of IIH patients. The lipid levels were evaluated in these patients and association with clinical parameters was assessed. Methodology: The patients presenting at PGIMER (Chandigarh) and fulfilling the revised diagnostic criteria (modified Friedman criteria, 2013) for IIH were included in this prospective pilot study carried out during the 4-month period (Feb, 2023-May, 2023). They were assessed for neurological, visual and radiological outcomes. The demographic and clinical findings including the lipid profile {Cholesterol, Low Density Lipoproteins (LDL), Very Low Density Lipoproteins (VLDL), Triacylglycerols (TAGs)} in these patients were explored & statistical analysis was performed. Results: All the patients reported headache of throbbing nature with intensity ranging from severe to moderate. Patients reported visual loss with CSF opening pressure being above the normal range. The lipidomics was studied in these patients & the statistical analysis was performed. The lipid profile was differentially dysregulated in these patients. Discussion: The etiology of IIH is poorly understood. The IIH patients are generally obese but not all the obese individuals develop IIH. Interestingly, we noted the differential increase in lipidogram in these patients suggestive of dysregulated lipid metabolic pathways. Conclusion: This pilot study provides a basis to identify metabolic targets requiring further study.

Abstract ID: 773

Marchiafava-bignami Disease Presenting as Akinetic Extrapyramidal Syndrome

Kheta Sharma, Hemant Luniwal, Samhita Panda, Sucharita Anand, Anka Arora, Sarbesh Tiwari

All India Institute of Medical Sciences, Jodhpur, Rajasthan, India

E-mail: kheta.sharma29@gmail.com

Background and Aim: INTRODUCTION- Marchiafava-Bignami disease (MBD) is a rare toxic disease associated mostly with chronic alcohol intake and characterized by progressive demyelination and necrosis of the corpus callosum and occasionally centrum semiovale and cerebral cortex. Diagnosis of the disease is made on the basis of clinical findings in combination with neuro-imaging. In MBD, the tempo of onset, progression and clinical symptoms are variable. Methodology: SINGLE CASE REPORT. Results: A 57 years old male farmer by occupation with alcohol use disorder since 40 years (180 ml/day of country-made liquor) had acute onset psychosis, cognitive decline, unsteady gait and urinary incontinence since 30 days. He had developed extrapyramidal symptoms in form of rigidity, bradykinesia, decreased blink rate and mask-like facies since 25 days, becoming akinetic mute since 10 days. MRI brain revealed diffusion restriction with corresponding T2/FLAIR hyperintensities involving entire corpus callosum predominantly involving the central layers with relative sparing of the dorsal and ventral parts, consistent with diagnosis of MBD. Thiamine was started in therapeutic dose with low dose levodopa/carbidopa and the patient had significant improvement in symptoms. Discussion: Most patients diagnosed with MBD have a history of alcoholism and poor nutrition and rarely uncontrolled diabetes mellitus. There may be acute, subacute or chronic onset of neurological deficits. Patients may present with stupor or coma, seizures, dementia, gait problems, psychiatric disturbances, incontinence, hemiparesis, aphasia, dysarthria, apraxia, and signs of interhemispheric disconnection. Some uncommon manifestations include intermittent alien hand syndrome and extrapyramidal symptoms. Conclusion: This case highlights a rare presentation of MBD as an akinetic mute state reversible with thiamine supplementation and levodopa.

Abstract ID: 774

MEAK (Myoclonic Epilepsy and Ataxia due to Potassium Channel Mutation) KCNC1 Mutation: An Extremely Rare Phenotype of Progressive Myoclonic Epilepsy: A Rare Case Report

S. Sidharth, Biswamohan Mishra, Deepti Vibha, Rajesh Singh, Manjari Tripathi, A. Elavarasi, Shailesh Gaikwad, Madhavi Tripathi, Manjari Tripathi, Deepti Vibha, Rajesh Singh, A. Elavarasi, Biswamohan Mishra

All India Institute of Medical Sciences, New Delhi, India

E-mail: drsidharth27@gmail.com

Background and Aim: Progressive myoclonic epilepsy (PME) include phenotype leading to myoclonic jerks, seizures and progressive neurologic impairment. KCNC1 is an extremely rare potassium channel mutation known to cause PME with ataxia. Methodology: 24 year old male born of a non-consanguineous marriage with low birth weight and near normal developmental milestones. At the age of 4 years, he developed multiple episodes of generalized myoclonic jerks responsive to valproate. At the age of 7 years , he developed tremulousness of both hands on activities, scanning dysarthria, gait instability and mild cognitive impairment . He had generalized tonic clonic seizures (GTCS) along with myoclonic jerks. His seizures were controlled since 14 years age on medications. On examination, he had gaze evoked horizontal nystagmus, broken saccades with normal fundus. Motor examination normal. Cerebellar examination revealed abnormal signs bilaterally. Blood investigations, Lactate levels were normal. MRI brain revealed diffuse cerebellar atrophy. Skin biopsy didn’t reveal any Lafora bodies. EEG showed generalized epileptiform discharges. Whole exome sequencing revealed KCNC1 c.959G>A variant at exon 2 with autosomal dominant pathogenic variant. Results: MEAK identification in a PME phenotype has unrevealed many of the unclassified PME spectrum diseases and the use of whole exome sequencing is useful for making the diagnosis. Discussion: Cohort of 84 patients of unconfirmed PME were taken in a multicentre study and found KCNC1 mutation encoding a potassium channel which was the first known human disease caused by KCNC1. The disruption of GABAergic neurons and degeneration of cerebellar neurons lead to syndrome of MEAK. Our patient had myoclonic epilepsy, GTCS, ataxia and was evaluated for common PME which were non revealing. He was tested for Exome sequencing where the pathogenic variant causing mutation in KCNC1 gene was revealed. Conclusion: Whole exome sequencing in PME with MEAK phenotype can help in diagnosing rare mutation of KCNC1.

Abstract ID: 775

MicroRNAs as Biomarkers for Distinguishing Stroke Subtypes: A Systematic-review and Meta-analysis

Manabesh Nath, Deepti Vibha, Pradeep Kumar

All India Institute of Medical Sciences, New Delhi, India

E-mail: manabesh.nath@gmail.com

Background and Aim: MicroRNAs (miRNAs) have emerged as potential biomarkers for diagnosis and differentiation of stroke. miRNAs have a significant role in the pathophysiology of ischemic stroke (IS) and hemorrhagic stroke (HS) and its dysregulation can depict disease risk in individuals. Methodology: A comprehensive literature search was performed in various electronic databases, including PubMed, Embase, Cochrane Library, CINAHL, and Google Scholar up to 17th May 2023. Pooled Standard Mean Deviations (SMDs) 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. Results: We identified 13 case-control studies with 1223 stroke patients (1065 IS, 158 HS) and 1445 control subjects within 24 hours, involving 11 Asian and two Caucasian population studies. Our findings revealed a significant association between the differential expression of miRNAs and diagnosis of overall stroke (SMD=2.85, 95% CI=1.34 to 4.35). Moreover, a significant relationship was observed between differentially regulated miRNAs and IS event within 24h (SMD=3.20, 95% CI=1.65 to 4.76). No such significance could be seen in case of HS (SMD=0.43, 95% CI=-4.71 to 5.58). Subgroup analysis depicted a significant association between dysregulated miRNA expression and stroke event in Caucasians (SMD=12.21, 95% CI=4.87 to 19.56) but not in the Asian population (SMD=-0.51, 95% CI=-2.97 to 1.95). Discussion: Our systematic review and meta-analysis provides evidence that miRNAs play a role in the pathogenesis of stroke. However, the underlying mechanisms of miRNAs in stroke pathogenesis are still not fully understood and require further investigation. Conclusion: The results indicated a significant association between differentially regulated miRNAs and the occurrence of stroke in patients. MiRNAs can serve as biomarkers for diagnosis of acute stroke.

Abstract ID: 776

MOG Antibody Associated Disease Presenting with Longitudinally Extensive Transverse Myelitis of the Dorsal Cord and Basal Ganglia Involvement in a Preschooler: A Case Report

Thahseen Sahubar Sadique

DM Neuro Pursuing, Other

E-mail: nilofarsahamed@gmail.com

Background and Aim: Myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is a Demyelinating disease presenting with encephalitis, optic neuritis, transverse myelitis. Typical radiological features include demyelinating cerebral and spinal lesions, cortical involvement, leptomeningeal enhancement. Here we present a case of a young child with encephalopathy, longitudinally extensive transverse myelitis and basal ganglia involvement with MOGAb positivity. Methodology: CASE REPORT: A 4 year old boy presented with low grade fever without focus for a day; was asymptomatic for next 2 days following which he became progressively encephalopathic. Vitals were stable. Preliminary blood tests normal and fever workup negative. On Day 5 child became pain responsive. Fever subsided; neck stiffness +. Acute encephalitis syndrome was considered .CSF analysis showed 10 lymphocytes, other parameters normal; started on meningitic dose of antibiotics and Acyclovir. Next day, he developed acute myelitis - Urinary retention and paraparesis. No RAPD. In view of rapidly progressive multifocal (cortex and cord) deficits, ADEM was considered. Results: MRI brain was normal; MRI spine showed subtleT2 hyperintensity from D1-D10. LETM work up was positive for Serum Anti MOG antibody. IV Methyl Prednisolone was given for 5 days without clinical improvement. So treatment was escalated with Plasma exchange. Encephalopathy improved; motor power improved. 3 weeks into the illness, child developed new onset dystonia in Upper Limbs. Repeat MRI showed subtle T2 hyperintensity in bilateral basal ganglia with normal spinal cord. In view of new lesions, Rituximab 375 mg/ Sq.m/week was given for 4 cycles. Movement disorder responded to anticholinergics. Discussion: At discharge he recognised parents, Walked with support and was on Azathioprine and Steroids. He returned to baseline during follow up. Conclusion: We present our case with the following unique points - Basal ganglia involvement; LETM in age < 9 years; dorsal cord involvement as against conus lesion; Steroid unresponsive MOGAD (+) ADEM mandating treatment escalation.

Abstract ID: 777

Orbital Infarction Syndrome a Rare Complication of Mechanical Thrombectomy

Pritam Raju, Girish Kulkarni, Deepak Menon, Manisha Gupta

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: pritamraja007@gmail.com

Background and Aim: Background: Orbital infarction syndrome is defined as ischemia of intraorbital and intraoccular structures secondary to occlusion of the ophthalmic artery or its branches. The ophthalmic artery supplies the optic nerve, extraocular muscles, and orbital fat. The ophthalmic artery arises from the supraclinoid segment of the ICA. It is a rare complication of mechanical thrombectomy as anastomoses of ophthalmic artery branches with the branches of the external carotid artery are extensive. Methodology: To report two cases of orbital infarction syndrome following mechanical thrombectomy. Results: Case 1: Fifty-five years old gentleman, case of an ischemic stroke involving left MCA territory presented within 2 hours of onset with nihss score of 20. He underwent thrombolysis followed by mechanical thrmobectomy, and tici 2b recanalisation was achieved. Immediately after the procedure, he developed periorbital odema, restricted eye movement, ptosis and complete loss of vision in the left eye with an unreactive pupil. Follow up after 2 months of symptom onset; he was status quo. Case 2: thirty years old lady a case of ischemic stroke involving right MCA territory presented within 9 hours of onset with nihss score of 11. She underwent mechanical thrmobectomy, and TICI 1 recanalisation was achieved. After 24 hours of the procedure, she developed sudden loss of vision in the right eye with visual acuity of finger counting from 1 meter with unreactive pupil. She was status quo at the time of discharge. Discussion: we Presented a rare case of orbital infarction syndrome resulting from distal emboli during mechanical thrombectomy for treatment of acute ischemic stroke. The other possible mechanism are hypotension and cca occlusion . Conclusion: Orbital infarction syndrome is a unusual but devastating complication of mechanical thrombectomy.

Abstract ID: 778

Orbital Apex Syndrome: A Series on Clinical Presentation and Outcome

Chayan Mondal, Uddalak Chakraborty, Soumozit Banerjee, Biman Ray, Alak Pandit

Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

E-mail: mondal.chayan2152@gmail.com

Background and Aim: Orbital Apex syndrome is caused by involvement of various structures at the region of orbital apex. The important neurovascular structures in the orbital apex are mainly 2nd,3rd,4th,6th and V1 cranial nerves with ophthalmic artery and vein. The etiologies of orbital apex syndrome commonly ranges from granulomatous inflammation, infective, infiltrative disorders. The authors hereby describe the spectrum of clinical presentation and outcome of 20 cases of orbital apex syndrome. Methodology: The authors describe a series of clinically and radiologically diagnosed orbital apex syndrome based on their clinical presentations and outcomes. Results: Among 20 cases, 14(70%) were male and 6 were female. 9 patients (45%) were diabetic. The patients were diagnosed after clinico-radiological correlation and tissue biopsy. Among the etiologies, fungal infection (Aspergillosis) was seen in 5 patients ( 25%) followed by tubercular infection in 4 patients (20%). Among non-infective granulomatous etiologies, sarcoidosis was seen in 3 patients (15%) followed by IgG4 disease in 2 patients (10%) and 5 patients (25%) had idiopathic granulomatous disease. One patient had lymphomatous infiltration (DLBCL) of orbital apex. Among clinical presentation, infranuclear ophthalmoplegia involving the 6th and 3rd cranial nerves was the commonest presentation (70%). Subacute to chronic vision loss was documented in majority of the patients (80%) and acute visual loss was seen in 20% patients. The median latency of diagnosis of orbital apex syndrome from the onset of clinical symptoms was 32 days. Majority of patients had a favourable outcome at the end of 6 months whereas 2 patients (10%) succumbed. Discussion: Orbital apex syndrome is commonly attributable to inflammatory followed by neoplastic infiltration and idiopathic causes. A constellation of involvement of 3rd,4th,5th (V1) and 6th cranial nerve in addition to involvement of 2nd cranial nerve localises the lesion to orbital apex in contrast to superior orbital fissure and cavernous sinus. Conclusion: The clinician must be vigilant in assessing the symptoms and treating the etiologies accordingly to ensure a favourable outcome.

Abstract ID: 779

Opsoclonus as an Association of Scrub Typhus

Aruna Kotteti

DM Neuro Pursuing, Other

E-mail: arunakotteti123@gmail.com

Background and Aim: To high light an ophthalmological sign associated with a CNS infection. Methodology: We came across a case with febrile illness with severe headache .fever investigations were negative the patient had ataxia and eye movement abnormality.by investigating further causes of fever we found that he was positive for IgM antibody to scrub typhus in blood.he was evaluated and was found to have cerebellar ataxia and opsoclonus. Results: He was diagnosed to have cerebellar ataxia and opsoclonus clinically and he improved spontaneously with treatment for scrub typhus with doxycycline. Discussion: Srub typhus is an acute febrile illness caused by orientiatsutsugamushi .scrub typhus results in multisystem involvement.neurological compromise is present in about 20%of scrub typhus patients.it affects both central and peripheral nervous system in the form of aseptic meningitis ,meningoencephalitis,cerebellitis,transversemyelitis,gullianbarresyndrome,polyneuropathy,opsoclonus is a rare neurological sign seen in patients with neurological involvement. Conclusion: Opsoclonus and cerebellar ataxia though were rare occurences in scrubtyphus ,it can occur in paraneoplastic conditions also,,but scrub typhus induced opsoclonus resolve spontaneously opsoclonus of the paraneoplastic usually doesnot regress unless immunotherapy is initiated.this case is presented for its rarity.

Abstract ID: 780

ODS - Not Salt Can Be Sugar

Harish Chandika, S. Saravanan

DM Neuro Pursuing, Other

E-mail: harishchandika21@gmail.com

Background and Aim: Osmotic demyelination syndrome (ODS) is a rare non-inflammatory CNS demyelinating disease. It is reported commonly with rapid correction of hyponatremia but it can also occur with other conditions causing rapid changes in serum osmolarity. Methodology: A 45 year old diabetic male presented with complaints of sudden onset unsteadiness of gait and slurring of speech of 7days duration. Examination showed dysarthria, nystagmus, bilateral impairment of finger nose and heel knee tests, dysdiadochokinesia and impaired tandem gait. Results: RBS : 540, HbA1c : 9.8, Na+ : 136, CSF : Normal, MRI Brain : T2 and FLAIR hyperintense signal with T1hypointensity showing facilitated diffusion restriction in pons, middle and inferior cerebellar peduncles. Discussion: Hyponatremia followed by its rapid correction is the most common cause of ODS although any kind of electrolyte imbalance can be the cause. Spectrum of clinical features varies based on anatomical site involved. Patient was managed symptomatically for high blood sugars and other supportive measures. On follow-up patient improved with no deficits and his sugar levels were under control. Conclusion: Not only rapid correction of hyponatremia, but also sudden or rapid change in extracellular fluid osmolarity due to diabetes mellitus can cause ODS. The exact mechanism for the development of ODS and appropriate glycaemic correction rate needs further evaluation. ODS can be missed in clinical setting; as it can be fatal and debilitating, it must be considered in any kind of disorder associated with change in osmolarity.

Abstract ID: 781

Occulomotor Abnormalities - A Diagnostic Clue in PME Spectrum Disorders

Rohit Keshav, Vijaya Varman, Ajai Singh, Pradeep Maurya, Dinkar Kulshrestha, Abdul Qavi, Sangam Singh

DM Neuro Pursuing, Other

E-mail: rohitrevealed@gmail.com

Background and Aim: Gaucher disease (GD) is an autosomal recessive condition caused by deficiency of lysosomal enzyme β-glucocerebrosidase. Patients with GD type 3 (chronic neuronopathic Gaucher disease) constitute about 5% of the population of Gaucher patients and affects fewer than 1 in 100 000 people. Methodology: 10-year-old child came with presenting complaints of recurrent falls with multiple seizure types (myoclonus, GTCS, partial seizures) with age of onset at 4 years. Since age 8 years, patient attendants had also noticed decline in scholastic performance and behavioural changes. Examination revealed multiple hyperpigmented brownish plaques around his back, inguinal region, wrist and ankle joints and mild splenomegaly. Neurological examination revealed B/L conjugate horizontal gaze restriction and a delayed saccadic initiation with compensatory head thrusts and preserved vertical gaze. Motor examination revealed spasticity and brisk reflexes with flexor plantar responses. Gait ataxia was present with left sided cerebellar and normal sensory findings. Examination of sister aged 2 years revealed B/L conjugate horizontal gaze restriction with head thrusts. Results: Blood investigations including serum lactate levels was grossly normal except mild normocytic normochromic anaemia. EEG was suggestive of high amplitude polymorphic delta – theta activity with intermittent multifocal generalized polyspike and wave complexes. VEP, BERA, NCS, MRI Brain was normal. CSF analysis was also normal. Axillary skin biopsy and muscle biopsy ruled out Lafora’s disease, NCL and MERRF. Whole Exome sequence was then sent suspecting Gaucher’s Disease which revealed pathogenic heterozygous missense mutation in coding region of GBA1 gene. Dried blood spot assay was subsequently done to establish the diagnosis which suggested deficient glucocerebrosidase activity. Patient was meanwhile managed with AED’s and is being planned for enzyme replacement therapy. Discussion: Horizontal Supranuclear gaze palsy may aid in the diagnosis of GD. Conclusion: Oculomotor abnormalities may serve as an important diagnostic clue in Gaucher’s Disease type 3C patients presenting as PME spectrum of disorders.

Abstract ID: 782

Normal Pressure Hydrocephalus - Revisited

Rashmi Devaraj, Aliakbar Netterwala, P Chandra, Abdul Khan

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: rashmid.326@gmail.com

Background and Aim: To Study the Demographics, clinical profile and outcomes of patients admitted with NPH. Methodology: Retrospective, Observational study conducted at Apollo Specialty Hospital, Jayanagar, and Bangalore from 1st January 2020 to 31st March 2023. Cases of MRI Proven NPH were included. Detailed History and Clinical examination were done, supplemented with LP-Tap Test and Neuroimaging. Patients showing clinical improvement underwent CSF Flow Diverting Procedures and outcome was noted. Results: Out of 14 cases of NPH satisfying the criteria, Average Age was 74.9 Years with only 1 Female. Average duration of illness was 21 months. 13 had gait impairment and 8 had urinary incontinence. Average MMSE at Baseline was 18/30, 9 patients who underwent Large Volume CSF Drainage improved in symptoms. Five underwent Shunt Surgery - 3 Theco-peritoneal, Shunt 1 Conventional Ventriculo-Peritoneal (VP) and 1 Programmable VP. 3 declined for procedure, 3 were deferred due to other causes 3 were lost on Follow-up. Their follow-up data to be presented. Discussion: Normal Pressure Hydrocephalus (NPH) presents with the triad of progressive Cognitive decline, gait difficulty and Urinary Incontinence. However, the definitive diagnosis and treatment may be challenging at times due to secondary causes of NPH, co-existing other degenerative, vascular and metabolic causes of similar clinical presentations. Recent advances in MRI i.e., TIME –SLIP (time-spatial-labeling-inversion-pulse) and diffusion tensor imaging (DTI) have shown promising applicability in diagnosis of NPH. Shunt surgery is the easiest and most common treatment strategy for NPH but over drainage and underdrainage are frequent problems and these complications can be avoided with the help of a Programmable /adjustable shunt. Conclusion: NPH is a treatable cause of cognitive decline and gait disorder. Advancement in flow diverting procedures has improved outcomes with lesser adverse events. While Neurodegenerative causes may accompany Primary NPH, any patient with improvement after CSF drainage deserves therapeutic intervention.

Abstract ID: 783

Nonketotic Hyperglycemia Presenting as Reflex Occipital Seizures and Left Homonymous Hemianopia

M. Bharath Kumar, S. Saravanan

DM Neuro Pursuing, Other

E-mail: dr.bharath10@gmail.com

Background and Aim: NKH (Nonketotic hyperglycemia) presents with neurological manifestations including chorea-hemiballimus, Epilesia partialis continua, focal deficits. One hypothesis states Cortical GABA Deficiency lowers seizure threshold since GABA is metabolized as the brain energy source in NKH. Another hypothesis states transient focal ischemia due to hyperglycemia leads to focal deficits. Methodology: A 60 years female with history of type2 Diabetes on treatment, presented with bilateral painless progressive Diminution of vision over 10 days. She had several episodes of complex visual hallucination involving human faces, each lasting few minutes. Vitals were within normal limits. General examination was normal. She could only perceive hand movements on both sides and field defect of left homonymous hemianopia was present. Remaining Cranial nerves, Spinomotor & sensory examination was normal . Patient developed left focal seizure in form of twitching of left eye lids whenever asked to look to left side. Results: Basic blood biochemistry was normal except RBS-424mg/dL , urine and blood ketones- negative. CT BRAIN, CSF STUDY- NORMAL. CSF Viral/autoimmune panel-NEGATIVE. MRI BRAIN (plain and contrast)- T2 hypointensity and flair suppression involving subcortical white matter of right posterior parietal and right occipital lobes with adjacent gyri showing hyperintensity. Illdefined lepto-meningeal contrast enhancement of right occipital lobe predominantly gyral based. Discussion: Patient was started on insulin for strict glycemic control, iv fluids for hyperosmolar state and symptomatic antiseizure medications. Vision normalised to 6/6 in 2 weeks and she became seizure free. MRI brain repeated after 4 weeks became normal. Conclusion: Partial seizures featuring visual symptoms are rarely reported. Reflex seizures denotes paroxysmal events consistently elicited by a specific stimulus, in our case looking to left .This case highlights and reiterates the dual pathophysiology of Nonketotic hyperglycemia (cortical GABA deficiency and focal ischemia ) in causing neurological manifestations (reflex seizures and Left hemianopia respectively) . This condition is completely reversible with treatment.

Abstract ID: 784

Neurosyphilis Presenting as Acute Ischaemic Stroke in Young

Satinder Deswal

DM Neuro Pursuing, Other

E-mail: satinderdeswal@gmail.com

Background and Aim: Neurosyphilis can be classified into early forms and late forms. The early forms typically affect the cerebrospinal fluid (CSF), meninges, and vasculature, while the late forms affect the brain and spinal cord parenchyma. Methodology: A 32 years gentleman with no comorbidities presented to neurology OPD with chief complaints of sudden onset double vision, instability in walking, severe vomiting episodes and slurring of speech 10 days back. His complaints were persisting but had improved since onset. He was admitted. On neurological examination, signs of meningeal irritation were present. There was medial gaze restriction on right side with left eye abducting nystagmus along with vertical nystagmus. There was left sided cerebellar ataxia. Rest examination was normal. Diagnosis of meningitis with vasculitic infarct was kept. Results: His routine stroke workup was normal. ESR was 38. HIV ELISA was positive and CD4 count came out to be 198 after ICTC centre registration. MRI Brain was s/o acute ischaemic infarct in right midbrain and pons with meningeal enhancement along prepontine cistern. CSF showed 300 cells/cumm with 90 % lymphocytes, proteins-225 mg/dl, ADA- 6U/L, sugar- 94 mg/dl, CALAS and India ink-negative, gene expert- negative. His serum RPR and serum TPHA were positive and CSF VDRL was negative. History of high-risk sexual behaviour and primary syphilis were later elicited. CSF viral markers for CMV, EBV, JE, HSV, JC and VZV were negative. Serum TORCH was negative. Discussion: Patient fulfilled the US CDC 2018 criteria for probable neurosyphilis. He was treated with a 14 days course of injection ceftriaxone and thereafter started on ART and Septran. On follow up patient condition improved and CSF after 6 months was completely normal. Conclusion: Neurosyphilis should be considered in cause evaluation of young stroke particularly if patient is retropositive.

Abstract ID: 785

Neuro Ichthyosis – A Case of SLS

Kirthika Kannan

DM Neuro Pursuing, Other

E-mail: aakokithika.thedoc@gmail.com

Background and Aim: Sjogren Larsson syndrome is a very rare Autosomal recessive inherited neurocutaneous disorder caused by mutations in ALDH3A2 gene. The prevalence of this condition is 1 per 2,50,000 individuals. It is important to identify associated features in a patient presenting with spastic paraplegia namely skin changes in order to diagnose the disease and predict the outcome for the case. Methodology: Case report. Results: SLS is a rare neurocutaneous disorder in South Asian population. Discussion: 17 year old male presented to the OPD with history of seizures. On examination he was found to have spastic dysarthria along with spastic paraparesis and Intellectual disability. On general examination, he had ichthyosis all over the body (which was present since his birth), wide spaced teeth and kyphoscoliosis. MRI Brain done showed white matter changes suggestive of leukodystrophy. Genetic analysis showed Exon-2 homozygous mutation of ALDH3A2+, highly suggestive of SLS. Conclusion: SLS is due to defect in fatty aldehyde dehydrogenase complex resulting in abnormal lipid accumulation in skin and white matter resulting in spastic diplegia, generalized hyperkeratosis of trunk, joints and dorsal aspect of hands and feet along with macular dystrophy and decreased vision.

Abstract ID: 786

Multisystem Atrophy Presenting with Dyskinesia- An Unusual Case Report

Jagdeep Singh, Sahil Mehta, Neeraj Balaini, Vivek Lal

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

E-mail: Rajagdeep94@gmail.com

Background and Aim: Multiple system atrophy (MSA) is a rare progressive neurodegenerative condition that primarily affects adults and has a male-to-female ratio of 1.3: 1. The specific cause of MSA is unknown. Many recent investigations have shown links between MSA development and aberrant protein-synuclein (Syn) accumulation in the glial cytoplasm. Aim- Dyskinesia can occur in atypical parkinsonian syndromes such as MSA, though less frequently than in Parkinson’s disease. Methodology: 63 year female with bronchial asthma and constipation presented with tremoulness while eating present throughout the range of motion and it was distal predominant. She had imbalance while walking in form of lateral flexion to right side. She had rigidity from past 10 months –axial rigidity initially started in neck then upper limbs which was mild with neck in extended position. Rigidity was associated with slowness of activity. Patient was admitted in Neurology ward with above mentioned complaints and was evaluated in view of multi system atrophy type c. Results: On neurological examination, cranial nerves were within normal limits. There was rigidity in tone in both bilateral limbs. FDG PET showed decreased uptake in B/L striatum right > left. MRI showed cerebellar atrophy with mild middle cerebellar peduncle degeneration. UPDRS Scaling was done there was improvement in % score of only 22% on Syndopa challenge. Patient was started with fludrocortisone 0.1mg for postural hypotension and TAB amantadine 50 mg , there was significant improvement in levodopa induced neck and truncal dyskinesia. Patient has been suspected to have MSA C based on above evaluation. Discussion: MSA manifests clinically as a variety of neurological signs and symptoms such as cerebellar impairment, autonomic nervous system dysfunction, and dopa-low-reaction Parkinson-like symptoms. Conclusion: The diagnosis is often confused with many diseases, so it is necessary to closely coordinate clinical, MRI as well as patient follow-up to achieve an accurate diagnosis.

Abstract ID: 787

Movement Disorders in the Setting of Dengue Infections in a Rural Medical Teaching Hospital in Gujarat, India

Soaham Desai, Devangi Desai

DM Neuro Completed, Other

E-mail: drsoahamdesai@yahoo.com

Background and Aim: Dengue fever is a common viral infection that can cause a range of symptoms, including fever, headache, and muscle pain. While movement disorders are not typically associated with dengue fever, there have been reports of these conditions occurring in some patients. This study aims to provide a comprehensive understanding of the occurrence of movement disorders in patients with dengue fever. Methodology: In this ambi-spective study, we collected series of all patients presenting with movement disorders in the setting of dengue infection in our hospital. We collected data all patients diagnosed with dengue fever between Jan 2015 to Dec 2022 . The patients with movement disorders were identified and their demographic data, clinical presentation, laboratory parameters and imaging findings were analyzed. The outcomes of the patients were noted at 3 months after discharge from the hospital or last follow-up visit. Results: Out of the total 736 patients with presenting with dengue ,17 (2.31% ) had movement disorder. The manifestations seen were parkinsonism (7), ataxia (5), isolated opsoclonus (1), parkinsonism with dystonia (2), opsoclonus myoclonus ataxia (OMAS) (2). All the patients developed the movement disorder after a duration of 5-15 days after onset of febrile illness. The patients with parkinsonism and dystonia had severe acute demyelinating encephalomyelitis with basal ganglia involvement following dengue shock syndrome, had prolonged course and needed steroids. All others had only dengue fever followed by subacute onset of movement disorder after a latent period suggestive of a parainfectious autoimmune process. Patients with isolated ataxia or parkinsonism had a normal imaging or Reversible splenial lesion[ RESLES or CLOCCs]. 7/17 patients had mild lymphocytic pleocytosis on CSF with raised protein and normal glucose, while others had normal CSF. All patients with ataxia or OMAS had spontaneous resolution over 2 weeks. Discussion: The types of movement disorders seen in these patients varied widely and included parkinsonism, isolated ataxia, isolated opsoclonus, OMAS, and parkinsonism with dystonia. However, the majority of patients had good outcomes, with complete resolution of symptoms within 4-6 weeks. Conclusion: Our study found that movement disorders can occur in patients with dengue fever, with a frequency of 2-3%.

Abstract ID: 788

Orbital Pseudotumor with Associated Optic Neuropathy in a Patient with Hashimotos Thyroiditis

Rosy Thomas, S. Velusamy, A. Nithyanandam, Aravind Gautham, V. Aravind, A. Nithyanandam, S. Velusamy, R. Lakshmi

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: thomas.rosy17@gmail.com

Background and Aim: Orbital pseudotumor also known as idiopathic orbital inflammatory syndrome is benign process confined to orbits usually but extraorbital involvement can occur. It is the third most common orbital disease. Patients present with diplopia, conjunctival chemosis, proptosis and may have associated optic neuropathy. Methodology: Clinical case study with review of literature. Results: 40 year old lady, known case of hypothyroidism on thyroxine replacement presented with complaints of left eye periorbital pain and decreased vision for past 8 months prior to presentation. She complained of swelling of left eyelid and proptosis. Patient’s attenders noticed that her left eye was not moving while looking sideways or vertically. The symptoms were progressive over past 8 months. No history of double vision. There was no diurnal variation of symptoms. It was not associated with any preceeding history of trauma. There is no history of fever, weight loss, loss of appetite. There was no associated headache or vomiting. On examination she had left eye proptosis with ophthalmoplegia which was non manouverable with relative afferent pupillary defect . Her visual acuity was decreased in left eye. In view of left sided 2,3,4,6 cranial nerve involvement a retroorbital pathology involving superior orbital fissure was kept and MRI brain done which revealed features suggestive of orbital pseudotumor. CSF analysis was normal and vasculitic profile was negative. Her anti TPO antibody were positive. She was initiated on pulse I.v methylprednisolone as per protocol with which her symptoms improved. Her visual acuity improved and ophthalmoplegia resolved. Discussion: Orbital pseudotumor is the most common differential of TAO. It denotes non specific benign orbital infiltration.It rarely can have a malignant course with extraocular muscle involvement and optic nerve damage. In orbitopathy related to hashimoto’s thyroiditis TRAb is positive. Conclusion: Orbital pseudotumor and related optic neuropathy has a good response to steroids.

Abstract ID: 789

Patent Foramen Ovale and Acute Ischemic Stroke – A Short Case Series

A. Shilpa Rani, Y. Muralidhar Reddy, Shyam K Jaiswal, Lalitha Pidaperthy, G. Abhinayumar, Subendhu Parida, Syed Osman, B. Sathosh, Jagarlapudi M. K. Murthy

DNB Neuro Pursuing, Other

E-mail: shilpareddy2409@gmail.com

Background and Aim: Case–control studies have demonstrated an association between PFO and cryptogenic stroke, especially in young adults. We aimed to study clinical and radiological features and outcomes of acute ischemic stroke (AIS) patients due to PFO. Methodology: METHODOLOGY: Study type - Retrospective; Study site - tertiary care hospital; Study period - April 2022 to March 2023; Inclusion criteria - consecutive patients of AIS associated with PFO; Diagnosis of AIS is based on MR/CT, and the PFO was diagnosed using trans oesophageal echocardiography. Data elements included age, gender, comorbidities, NIHSS at admission, ROPE score, arterial territory involved, large vessel occlusion, PFO characteristics, and 90-day modified Rankin Scale. Results: RESULTS: Total number of subjects (n) - 10; Age (Mean ±SD) - 65.8±5.9 years; M: F -7:3; Comorbidities and risk factors n (%) - hypertension 7(70%), diabetes 7(70%), tobacco consumption 2 (20%), alcohol consumption 3(30%), coronary artery disease 5(50%), hyper homocysteinemia 1(10%), migraine 0(0), hypothyroidism 2(20%); NIHSS at admission n (%) - ≤ 4 - 5 (50%); 5-15 - 5 (50%); ROPE score was less than 7 in all; Arterial territory involved n (%) - only anterior circulation 6 (60%), only posterior circulation 2(20%), both anterior and posterior circulation in 2(20%); Large vessel occlusion 1 (10%); PFO size n (%)- less than 2mm 3 (30%), 2-3.9 mm 2 (20%), ≥4 mm 5 (50%); Inter atrial aneurysm 3 (30%); 90-day mRS n(%) - [0-2] 8(80%), [2-5] 2(20%). They are usually seen in the anterior circulation and have a favourable 90-day functional outcome. Discussion: -. Conclusion: PFO-associated ischemic strokes in our cohort were seen predominantly in elderly males.

Abstract ID: 790

Personalised Therapeutics for Neurogenetic disorders

Noel Gomes, Rahul Chakor, Swaleha Nadaf

Topiwala National Medical College and BYL Nair Charitable Hospital, Mumbai, Maharashtra, India

E-mail: noelg03@gmail.com

Background and Aim: Diagnosis of rare, complex, neurogenetic disorders with variable phenotypes has been made easier with NGS (next generation sequencing). Genetically confirmed diagnosis can help initiate tailor made therapy. We present four cases earlier labelled as non-treatable degenerative disorders. NGS technology confirmed genetic diagnosis and subsequently definitive therapy led to improved clinical outcome. Aims- To discuss how NGS can help initiate specific therapy in neurogenetic disorders. Methodology: Whole exome sequencing by NGS technique was done for 70 patients of suspected neurogenetic disorders over a period of two years. Results: whole exome sequence report of patients, case 1-MTHFR mutation (EXON 3), case 2- PRRT 2 mutation, case 3- SLC52A3 mutation, case 4-ATP13A2 mutation. Discussion: Case 1: 35-year-old male presented with spastic paraparesis and cognitive decline for five months. There were white matter hyperintensities suggestive of leuocoencephalopathy on MRI brain. His sister had spastic paraparesis, renal and Cerebral venous sinus thrombosis. NGS detected MTHFR mutation. There was significant hyper-homocysteinemia. Patient improved after treatment with folinic acid, pyridoxine and Betaine. Case 2: 15 years old female presented with generalized tonic clonic seizures and axial jerky movements suggestive of myoclonus. She was being treated with levetiracetam and valproate without any benefit. NGS detected PRRT 2 mutation. The jerks subsided completely with carbamazepine. Case 3: 9 years old boy presented with 2 years history of hearing loss, seizures, facial weakness and tongue atrophy. NGS detected SLC52A3 mutation causing Brown Vialetto Van Laere syndrome. He was treated with riboflavin (dose 100 mg/day) with significant improvement. Case 4: 26 years old male presented with spastic gait, bulbar weakness, mild extra pyramidal symptoms for 2 years. He was clinically suspected to have juvenile ALS. NGS detected mutation for Kufor Rakeb syndrome. His gait and bulbar symptoms improved with levodopa. Conclusion: NGS gives a genetic diagnosis and guides in potentially life-changing tailor-made therapy.

Abstract ID: 791

Polysomnographic Study of Sleep Disorders in Idiopathic Parkinson’s disease

Ramanan Chandrasekaran, M. Rajasekaran, N. Kannan, R. Kishore

DM Neuro Pursuing, Other

E-mail: dr.cramanan@gmail.com

Background and Aim: Parkinson’s disease (PD) patients are very commonly affected by insomnia, which is the most common non-motor symptom known to reduce the quality of life in patients as well as caregivers and can exacerbate other motor and non-motor symptoms of the disease. Our aim is to study Sleep abnormalities in idiopathic Parkinson’s disease patients with Polysomnography. Methodology: We studied 50 Patients fulfilling clinical diagnosis of PD using UK PDS Brain Bank Criteria for PD admitted in the neurology ward in Mahatma Gandhi Government General Hospital from October 2022 to March 2023. Patients who are bedridden and with concomitant major cardiac and respiratory ailments were excluded. All patients were subjected to detailed clinical assessment and staging and severity scoring done using Hoehn and Yahr staging system and MDS-UPDRS Scale respectively. Patients were subjected to a sleep questionnaire Parkinson’s disease sleep scale (PDSS) and overnight polysomnographic study using Medicaid Sleep Care SC-32 Polysomnographic system and parameters like EOG, EEG, EMG, ECG, Oxygen saturation, Respiratory effort, airflow, body position were studied. Results: Sleep disturbances were observed in majority of patients. Frequent micturition and nocturnal painful cramps were observed as major causes of fragmented sleep. Total sleep time was reduced in 41 (82%) patients. A strong correlation was observed between Total sleep time and disease duration, staging and severity. Sleep latency was prolonged in 31 (62%) patients and normal sleep efficiency was observed in only 13 (26%) of our patients. REM sleep onset latency was prolonged in 35 (70%) patients. Periodic limb movements in sleep were observed in 11 (22%) patients. Discussion: Polysomnography helps to characterize the nature and severity of the sleep problem and identify contributing factors for successful treatment. Conclusion: A polysomnographic study of sleep disorders among PD patients could help us manage them better.

Abstract ID: 792

Post-tussive Bilateral Vertebral Artery Dissection: A Rare Case Report

Sravya Reddy, Y. Muralidhar Reddy, Subhendu Parida, Shyam Jaiswal, Lalitha Pidaparthi, Abhinay Gattu, Jagarlapudi Murthy

DNB Neuro Pursuing, Other

E-mail: dr.sravyareddy17@gmail.com

Background and Aim: Cervical artery dissection is a rare cause of large artery stroke in young individuals. A severe cough can rarely result in either carotid or vertebral dissection. Bilateral vertebral artery dissection following cough has not been reported in the literature. our aim is to present a rare case of bilateral vertebral artery dissection due to a common symptom seen in our practice. Methodology: This is a retrospective single case report of a 31year old female patient admitted under neurology department in care hospitals, banjara hills. she was evaluated with HRCT chest,MRI brain,MR and CT angiogram of the neck and intracranial vessels.IgG anticardiolipin antibodies,IgG phospholipid antibodies,ANA profile by immunoblot method were done. Results: the patient had B/L vertebral artery dissection in V2 segment. Discussion: A 31-year-old female presented with occipital headache and neck pain for 15 days. She also complained of a persistent dry cough for two months. She was evaluated by a chest physician for cough (and no specific cause was found) and was treated with anti-tussive syrups and antibiotics. The cough decreased significantly when she presented to us with a headache. General and Chest examination was unremarkable. Neurological examination showed gaze-evoked nystagmus to either side. MR Brain showed acute infarcts in bilateral cerebellar hemispheres. Transthoracic echocardiography was normal. MR and CT, angiogram of neck and intracranial vessels, showed bilateral vertebral artery dissection with acute thrombus in the V2 segment. RT-PCR for SARS CoV2 was negative. The vasculitis profile, anti-cardiolipin antibodies, and prothrombotic profile were negative. She was treated with low molecular weight heparin and later on warfarin. Her symptoms resolved. She is under follow-up. Conclusion: Cervical artery dissection should be ruled out in patients with embolic stroke following persistent cough, especially in younger individuals with no atherosclerotic risk factors.

Abstract ID: 793

Postpartum Longitudinally Extending Transverse Myelitis – A Rare Presentation of MOG Associated Disease

M. Abirami, J. Manickavasagam, G. Balaji, S. Hariharan

DM Neuro Pursuing, Other

E-mail: abiramitvmc3@gmail.com

Background and Aim: LETM is defined by a spinal MRI indicating a lesion extending over three or more vertebral segments.The clinical course of longitudinally extensive transverse myelitis is characterised by single or multiple attacks of paraparesis or tetraparesis, sensory deficits and bowel/bladder disturbances and in severe cases can lead to respiratory failure.The early distinction between possible LETM etiologies is crucial in providing accurate prognosis and guiding therapeutic strategy. Methodology: Case report. Results: MOGAD is also a rarest etiology of postpartum LETM . Discussion: 24yrs primi para ( P1L1) 60days postnatal mother,developed sensory symptoms in lower limbs progressed over 2 days,with a band like sensation around chest and sudden onset weakness in both proximal and distal group of muscles with bladder,bowel involvement along with a shock like pain from cervical region to lower limbs, examination revealed sensory deficit involving all modalities of sensation lower limb more than upper limb,Paralysis of lower limb initially flaccid then became spastic and imaging revealed LETM extending from C2-T6,serum positive myelin oligodendrocyte glycoprotein antibody.Patient treated with pulse steroid followed by oral steroids symptomatically improved and walks without support. Conclusion: After delivery, pregnancy-related hormones decrease sharply, and inhibited immune response begin to be active,resulting in relapse of previous IDD or new onset demyelinating disease.Among the postnatal demyelinating diseases, MOGAD are rarely reported when compared to MS and NMOSD. Abbreviations LETM-Longitudinally extending transverse myelitis MOGAD-Myelin oligodentrocyte glycoprotein antibody –associated disease NMOSD - Neuromyelitis optica spectrum disorders MS- Multiple sclerosis, IDD- Immune related demyelinating disease.

Abstract ID: 794

Portosystemic Shunt Presenting as Spastic Paraparesis in a Patient with Non Cirrhotic Portal Hypertension

Swathi Sanjee, G. R. K. Sarma, Raghunandan Nadig, Thomas Mathew, Sagar Badachi, Sonia Shivde, Akshatha Huddar, V. Sindhu, Surabhi Garg,Y. M. Pavan

St. Johns Medical College, Bengaluru, Karnataka, India

E-mail: swathi.s.sanjee@gmail.com

Background and Aim: Portosystemic myelopathy (PSM) is a rare complication seen in patients with chronic liver disease and in those with portosystemic shunts manifesting as pure motor spastic paraparesis. Myelopathy can be reversed with liver transplantation or selective embolization of shunt if diagnosed in early stages. Methodology: A 55-year-old female with presented with difficulty in walking with progressive stiffness in both lower limbs for 6 months. No back pain, fever, involuntary movements, or sphincter involvement. No history of jaundice, malena, hematemesis or altered sensorium. No history of any drug, alcohol or toxin exposure in past. Examination revealed moderate splenomegaly. MMSE was 30/30. Spasticity in both lower limbs of grade 3, with brisk knee and ankle jerks and Babinski’s sign was positive. There were no cerebellar signs and sensory examination was normal. Upper limb sensory and motor system examination was normal. Results: CSF analysis and NCS were normal. Autoimmune encephalitis, Immunoline, and paraneoplastic panel were negative. MRI spine was normal. MRI brain showed T1 hyperintensity of basal ganglia. CECT abdomen showed normal liver, CBD stent in-situ with two calculi in gall bladder. Spleen was grossly enlarged. Portal vein dilated (17.5 mm) with tortuous porto-systemic collaterals noted in paraduodenal and right suprarenal region draining into inferior vena cava. Left moderate pleural effusion. Discussion: The pathogenesis of PSM involves symmetrical demyelination or axonal loss, predominantly involving the lateral corticospinal tracts which generally occurring not higher than cervical cord. In portosystemic myelopathy, shunting of blood may allow nitrogenous products or neurotoxins to bypass the liver and possibly enter the venous system of spinal cord leading to selective damage of spinal cord neurons. Conclusion: Spastic paraparesis, hypoalbuminemia, blood ammonia elevation and absence of compressive myelopathy changes are diagnostic indicators of PSM. This condition should be suspected and diagnosed early for halting the disease progression to late irreversible myelopathic features and improving the morbidity.

Abstract ID: 795

Progressive Multifocal Leukoencephalopathy Secondary to Post Chemotherapy

Kalpana Perumal, Thamil Pavai, Lakshmi Narasimhan, L. A. Ravi

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: dr.kalpanaperumal@gmail.com

Background and Aim: Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease preferentially affecting the central nervous system caused by reactivation of John Cunningham polyomavirus (JCV). Methodology: 33 years male presented with insidious onset gradually progressive neurological illness in the form of unsteadiness while walking for 10 months duration, behavioural disturbances in the form of offensive language, beating wife, suspicion on wife, breaking household objects with memory disturbance for three days duration with background TB Spine in 2016, Non Hodgkin’s Lymphoma of vertebra in 2017 Post CT/ RT was on CHOP regimen. On examination he was conscious, MMSE 9/30, FAB – 6/18, impaired attention, recent /visual memory impaired , with optic ataxia, occulo motor apraxia, finger agnosia, right left disorientation, paratonia, hyperreflexia with cerebellar signs, wide base gait and gibbus present in D7. Results: Investigations revealed normal basic blood investigations. viral markers were negative. Thyroid profile and vitamin B12 level were normal. Paraneoplastic panel and autoimmune panel was negative. CSF analysis revealed normal protein and glucose with csf JCV PCR positive. Imaging revealed B/l asymmetrical white matter hyperintensities involving predominantly parietal lobe with involvement of subcortical U fibres. Discussion: PML is an uncommon demyelinating disease of the CNS characterized by the destruction of oligodendrocytes due to the reactivation of JC virus. It has a rapid clinical course with an extremely poor clinical outcome, the overall median survival without treatment being a mere 3.5 months. Conclusion: The present case report describes a rare but deadly complication of chemotherapy for non-hodgkins lymphoma. In cases with a relatively rapid progressive neurological disorder affecting the entire nervous system and mainly involving the white matter, in a setting of immunocompromise, it is important to consider PML to obtain an early diagnosis.

Abstract ID: 796

Proximal Muscle Weakness with Myotonia -Not always PROMM

Shakthi Govindarajan, Lakshmi Narasimhan Ranganathan, K. Shanmugasundaram, A. Mariam Jude Vijay

Madras Medical College, Chennai, Tamil Nadu, India

E-mail: neuroshakthi@gmail.com

Background and Aim: Early distal muscle weakness and myotonia are typical clinical presentations in type I myotonic dystrophy (DM1). We present a DM1 case with unusual predominant proximal weakness with action myotonia. Methodology: Detailed history taking and clinical examination was done. Results: Confirmed by genetic analysis. Discussion: Our 42-year-old male patient was normal until his early 30s after which he initially had progressive difficulty in raising his arms which was followed 2 years later by difficulty in getting up. For the past 4 years his weakness is further progressive to distal muscles of both upper and lower limbs with difficulty in letting objects go off after gripping them, with difficulty in holding head steady for past 1 year . On neurological examination his cognitive function was fair , shoulder and hip girdle group of muscles were weaker than distal muscles, in addition to facial involvement . Further Clinical Examination revealed action myotonia in hand, along with tongue myotonia being readily inducible by percussion. The diagnosis of DM1 was later supported by electromyography and confirmed by molecular genetic testing of CTG repeat tract of DMPK gene analysed by the repeat length analysis of Triplet Primed PCR using three primers. The examination findings in this patient had atypical prominent proximal weakness which is quite unusual for DM1. Conclusion: The unusual presentation of this DM1 patient suggests the importance of comprehensive neurological examination including percussion of thenar and tongue muscles, even in a patient with atypical distribution of muscle weakness and without a clear personal and family history of myotonia. In addition to molecular testing and electrophysiology clinical examination was supportive in making the diagnosis.

Abstract ID: 797

Proxysmal Kinesigenic Dyskinesia: An Easily Treatable but often Missed Condition

Mukund Agrawal

Institute of Medical Sciences, Varanasi, Uttar Pradesh, India

E-mail: mukundagrawal010@gmail.com

Background and Aim: Paroxysmal kinesigenic dyskinesia (PKD) is characterized by recurrent, attacks of abnormal movements [dystonia (most common), chorea, athetosis or ballism or a combination], triggered by sudden movement, acceleration or intention to move. Multiple attacks occur every day, lasting for seconds upto 1 minute.Attacks can be unilateral or bilateral.Maximum cases are idiopathic or familial. Secondary PKD accounts for very few cases. Methodology: Case report. Results: A 10 year old boy with normal birth & developmental history presented with recurrent, sudden onset involuntary twisting movements (distal>proximal) of left followed by right upper limb for 5 months, appearing only during activities, lasting for 10-15 seconds, occurring 15-20 times/day, subsiding spontaneously & never at rest. There was no premonitory sensation, pain, altered sensorium, tonic clonic movements, falls, facial involvement, emotional lability, scholastic deterioration, weakness of any limb, bradykinesia, tremulousness. Apart from these movements patient was absolutely normal. There was no history of preceding fever, rashes, joint pain, diurnal variation. Events occurred at the same frequency (whether alone or in group).He was previously considered as a case of PNES.During examination he had precipitation of dystonic & choreiform movements in the left upper limb on getting up from chair after sitting quietly for 1 hour.Apart from this, examination was normal. Complete hemogram, liver & renal function tests, calcium, ESR, CRP, Anti Streptolysin O titre, 2D Echo, MRI brain & Video EEG were normal. He had complete remission from carbamazepine (empirical).Genetic analysis revealed heterozygous mutation in PRRT 2 gene. Discussion: PRRT2 is inherited in autosomal dominant fashion with variable penetrance. In sporadic form there is a higher male prevalence (4:1). Age of onset is usually 7-15 years. PKD has excellent response to ASMs (Carbamazepine, oxcarbazepine, phenytoin, levetiracetam, valproate). Conclusion: PKD is an easily treatable movement disorder & requires high index of suspicion.

Abstract ID: 798

Questionnaire-based Assessment of Sleep Abnormalities in Patients with Migraine: A Cross-Sectional Study with a Comparison Group

Vaishali Sharma, Kamalesh Chakravarty, Sucharita Ray

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

E-mail: svaishali1996@gmail.com

Background and Aim: Background-Patients with migraine frequently have co-existent sleep-related abnormalities. This underlying sleep abnormality might be a contributing factor to the increased disease burden. Aims-To evaluate sleep abnormalities in migraine patients using validated and worldwide accepted questionnaires compared with healthy controls. Methodology: 60 cases and 30 healthy controls enrolled. Baseline clinical characteristics noted. Headache assessment was done using the MIDAS questionnaire. Sleep was assessed using Epworth Sleepiness Scale (ESS), Pittsburgh Sleep Quality Index (PSQI), RLS scale for restless leg syndrome, and Berlin questionnaire for OSA. Results: The mean age of patients was 35.17 ± 10.02 years and controls 32.70 ± 7.50 years. The severity of headache (VAS) mean was 7.89 ± 0.84. MIDAS Score was 18.74 ± 28.57. 55% of cases had chronic migraine, 40% had migraine without aura, and 5% had migraine with aura. Type of headache was mostly Throbbing type in 85% patients, followed by dull aching headache in 10% patients and Lancinating headache in 5% patients. The mean headache duration (for each attack) was 33.34 ± 24 hours. PSQI score>5 (indicating poor sleep quality) was found in 60.56% migraine patients and 23.34% controls (p=0.0001). Mean ESS score was 7.97 ± 4.61 in cases and 5.60 ± 4.04 in controls (p=0.009), 31.67% of cases and 10% of controls were found to have daytime sleepiness (p=0.0242). Restless leg syndrome (RLS) was found in 35% migraine patients and 23.34% controls (p=0.2597). High risk of Obstructive sleep apnea (OSA) was found in 8.34% of patients and 0 controls according to the Berlin questionnaire. Discussion: Sleep and headache are considered closely related. The likelihood and severity of sleep problems increase proportionally with migraine frequency and disability. Studies on the Western population have shown that poor-quality sleep can result in headaches, including migraines. Conclusion: Migraine patients tend to have poor sleep quality as compared to healthy controls.

Abstract ID: 799

Rare Manifestation of Retroviral Disease

Shamili Devi, Senthur Pandiyan

DM Neuro Pursuing, Other

E-mail: shamili3119@gmail.com

Background and Aim: Progressive multifocal leukoencephalopathy (PML) caused by the polyomavirus, JC virus (JCV), is one of the most dreaded complications of HIV-1 infection. Unlike other opportunistic infections, PML may present while blood CD4+ T cells remain above AIDS-defining levels and while patients receive combined antiretroviral therapy (cART), either shortly after starting or, more rarely, during chronic successful treatment. Methodology: 35 years old male presented with acute onset, difficulty in using right upper limb and right lower limb in the form of difficulty in holding things, difficulty in combing hair, difficulty in buttoning and unbottuning shirts, difficulty in standing and walking along with deviation of angle of mouth to left and drooling of saliva for past 10 days and history of headache for 4 days which is holocranial, subsided by medications , not associated with vomiting . On examination patient had right homonymous hemianopia, decreased tone in right upper limb and right lower limb, power 4/5 in right upper limb and right lower limb, plantar extensor on right side along with deviation of angle of mouth to left. Results: Investigations were done. His blood counts, renal and liver parameters were normal, MRI BRAIN Shows features of Progressive Multifocal leukoencephalopathy. CT chest shows features of pneumonitis. He turned out to be HIV positive and his CD4 count is 167. Discussion: Progressive multifocal leukoencephalopathy is an infection of the central nervous system by the polyoma virus, JC virus , that destroys oligodendrocytes and their myelin processes. Classically, PML presents insidiously with focal neurological deficits that vary depending on lesion localization. There may be hemisensory defects or hemiparesis ,visual field loss and hemianopsia (occipital lobes or optic radiations), aphasia , or ataxia and dysmetria. Some patients develop seizures, in association with lesions adjacent to the cortex . Headache and fever are usually absent. Conclusion: PML should be considered in any HIV-1-infected patients presenting with acute or insidious onset of focal neurological symptoms, both cART untreated or treated. If the diagnosis of PML is achieved, effective cART needs be immediately instituted.

Abstract ID: 800

Rare Presentation of LMN Facial Palsy in Cerebral Venous Thrombosis: A Case Report

K. Mugundhan, K. V. Vandana, S. Haritha, K. V. Vandana, Surya Prakash Kanithi, P. K. Syed Munnaver

DM Neuro Completed, Other

E-mail: mugundhan69@gmail.com

Background and Aim: We report a patient with raised ICP headache and lower motor neuron facial palsy with CVT showing resolution on treatment with anticoagulation , highlighting a rare atypical clinical presentation of CVT.Facial nerve palsy in cerebral and cortical vein thrombosis is rare with an unclear pathophysiology. Although involvement of bilateral sixth cranial nerve due to raised ICP and other ocular motor nerve involvement has been previously described , other cranial nerves such as facial nerve involvement is less documented. Till now only around 6-7 case reports have been described. Methodology: we report a case of a 44 year old male, a chronic alcoholic admitted with complaints of severe headache and raised ICP features. He developed left sided facial nerve palsy with impaired taste perception on the left side. Neurological examination revealed left LMN facial palsy with Bells phenomenon and bilateral established papilledema. Results: Mri brain and MR venography showed evidence of cerebral venous thrombosis of late subacute stage involving superior sagittal sinus, straight sinus, right transverse and right IJV with features of intracranial hypertension. Discussion: CVT is defined as the thrombosis of the superficial or deep venous system of the brain . Risk factors that are commonly seen include prothrombotic state ,chronic alcoholism, dehydration OCP use ,vasculitis, infection, congenital heart disease, pregnancy and immediate postpartum period, anemia, and polycythemia. Conclusion: Our patient had risk factor of chronic alcoholism and dehydration. Facial palsy in our patient is considered to be due to transient neuropraxia in the intracranial segment of the nerve. This case highlights facial palsy as a rare clinical presentation of cvt.

Abstract ID: 801

Rare Presentation of LMN Facial Palsy in Cerebral Venous Thrombosis: A Case Report

P. K. Syed Munnaver

DM Neuro Pursuing, Other

E-mail: syedfm@gmail.com

Background and Aim: We report a patient with raised ICP headache and lower motor neuron facial palsy with CVT showing resolution on treatment with anticoagulation , highlighting a rare atypical clinical presentation of CVT. Methodology: Facial nerve palsy in cerebral and cortical vein thrombosis is rare with an unclear pathophysiology. Although involvement of bilateral sixth cranial nerve due to raised ICP and other ocular motor nerve involvement has been previously described , other cranial nerves such as facial nerve involvement is less documented. Till now only around 6-7 case reports have been described. Results: we report a case of a 44 year old male, a chronic alcoholic admitted with complaints of severe headache and raised ICP features. He developed left sided facial nerve palsy with impaired taste perception on the left side. Neurological examination revealed left LMN facial palsy with Bells phenomenon and bilateral established papilledema. Mri brain and MR venography showed evidence of cerebral venous thrombosis of late subacute stage involving superior sagittal sinus, straight sinus, right transverse and right IJV with features of intracranial hypertension. Discussion: CVT is defined as the thrombosis of the superficial or deep venous system of the brain . Risk factors that are commonly seen include prothrombotic state ,chronic alcoholism, dehydration OCP use ,vasculitis, infection, congenital heart disease, pregnancy and immediate postpartum period, anemia, and polycythemia . Conclusion: Our patient had risk factor of chronic alcoholism and dehydration. Facial palsy in our patient is considered to be due to transient neuropraxia in the intracranial segment of the nerve. This case highlights facial palsy as a rare clinical presentation of cvt.

Abstract ID: 802

Red as White in MRI – An Interesting Cases of Stroke

Nareng Padun, Vignesh Karunaharan, A. Prabhu, R. Kishore, E. Arunraj

DM Neuro Pursuing, Other

E-mail: padunnareng@gmail.com

Background and Aim: Stroke is the third leading cause of mortality and morbidity worldwide. Ischemic stroke may be the first presenting symptom of Polycythemia Vera in 15% or more of those affected. we are reporting a cases of Polycythemia Vera presenting with acute ischemic stroke as the initial symptom. Through this case, we aim to highlight polycythemia vera as a cause of ischemic stroke and the importance of blood counts in the routine evaluation of the same. Methodology: case vignette 1: 50-year-old woman, normotensive non diabetic who presented with features of acute onset posterior circulation stroke- headache, vertigo and ataxia, MRI showed multi- infarct state. Case vignette 2: 79 y/f presented with aphasia with right hemiparesis, MRI showed left capsule-ganglionic infarct. Case vignette 3: 65 y/f acute left hemiplegia, MRI showed right capsulo-ganglionic region. All three patient’s Hematologic investigations revealed a hyper viscous state with features of polycythemia. Genetic studies were positive for polycythemia vera mutation-JAK2(exon 14) and positive bone marrow, other investigations including ECHO were normal. Results: symptom improved with antiplatelet agents, adequate hydration and chemotherapy with hydroxyurea. Discussion: Polycythemia vera (PV) is a myeloproliferative disorder associated with an increased risk of cerebrovascular diseases. The ischemic stroke resulted as the first manifestation of PV in up to 16.2% of cases. Cerebral ischemic events are due to increased blood viscosity and platelet activation within the central nervous system arterial vessels. An early diagnosis could lead to prompt treatment with phlebotomy, treatment with hydroxyurea, and low-dose aspirin to decrease the risk of recurrences. Conclusion: close attention to routine blood investigation is very important in all cases, which in these cases turned out to be cause but a simple routine test.

Abstract ID: 803

Refractory Headache Due to Mycobacteria Presenting as Aspetic Hypertrophic Pachmeningitis

V. Ganaraja, Suresha Kodapala

Vydehi Institute of Medical Sciences and Research Centre, Bengaluru, Karnataka, India

E-mail: ganaraj.v.h.91@gmail.com

Background and Aim: Tuberculosis infection of the central nervous system is diagnosed in a variety of forms. However, rarely in certain cases, the inflammatory response may become exuberant leading to a hypertrophic form of pachymeningitis. Methodology: Hereby we report a case of refractory headache due to tubercular hypertrophic pachymeningitis presented to our centre. Results: A 36 year old male presented to us with features of headache of one month duration. It was present all over the head, continuous dragging type, disturbing his daily activities. There was no fever or vomiting, no triggering factors for headache. This headache was not responding to any medicines including injectable analgesics. His routine blood investigations were within normal limits. Serological tests for HIV,Hepatitis B and C were negative. MRI brain showed diffuse dural thickening with enhancement in frontal and parietal convexity. Cerebrospinal fluid analysis showed 6 lymphocytes, sugars of 76mg/dl ( elevated) , protein 195mg/dl (elevated). CSF gram stain, gene xpert for tuberculosis meningo-encephalitis panel and autoimmune panel were negative. Meningeal biopsy from right frontal dura-mater showed features of chronic Ill formed granulomatous inflammatory lesion with acid fast bacilli consistent with tuberculous pachymeningitis. Discussion: This report highlights the importance of considering mycobacterial infection in the differential diagnosis of hypertrophic pachymeningitis. Conclusion: Hereby we report a rare treatable disorder and early identification is very crucial for good clinical outcome.

Abstract ID: 804

Risk Factors in Paediatric Stroke: A Tertiary Care Hospital Based Cross-sectional Study

Soumya Das, B. Kannan, Thomas Raj, Hrishikesh Pai

DM Neuro Pursuing, Other

E-mail: soumyakantidas@gmail.com

Background and Aim: Stroke is relatively rare in children and the clinical presentation of paediatric stroke is often subtle. Treatment of childhood stroke and preventive strategies depend upon each child’s clinical profile. The aim of this study is to characterize risk factors, and to identify possible common factors. Methodology: This hospital based study was done at Thoothukudi Government Medical College Hospital. 40 patients of age 1 month to 15 years attending the paediatric and neurology OPD or admitted in paediatric or neurology ward with evidence of stroke were assessed for possible risk factors. Study duration was of 1 year (May 2022-May 2023). All established paediatric stroke cases, ischaemic or haemorrhagic where included in the study. Congenital hemiplegia and cerebral palsy patients were excluded. Risk factors were assessed using clinical, radiological and laboratory data. Results: Out of 40 patients, 20 (50%) were male and 20 (50%) female. 14 out of 40 (35%) had no recognizable risk factors. Median age of the patients was 7.5 years. 8 (20%) were haemorrhagic and 32 (80%) were ischemic stroke. The risk factors identified were dyslipidemia (10%), sickle cell (10%), vitamin K deficiency (5%), nephrotic syndrome (5%), infective cause (10%), hyperhomocysteinemia (10%), vasculitis (10%), prothrombotic state (5%). Discussion: The data supports the view that childhood stroke is multifactorial. This is in agreement with studies that suggest presence of multiple risk factors can increase chances of recurrent stroke. Priority should be to identify modifiable risk factors such as homocysteinemia and dyslipidemia. Risk factors could not be identified in around 35% cases which is in line with other studies. Conclusion: Although common patterns of risk factors could not be identified, several risk factors occurred more commonly. Clinical presentation seemed to vary. Hemiparesis seemed to be more common in younger children whereas seizures were commoner in older children. More studies with larger sample sizes needed.

Abstract ID: 805

Risk and Protective Factors for Dementia: A Study from a Large Dementia Cohort from Two Centres

Faheem Arshad, S. Kumar, Shailaja Mekala, Aparna Somaraj, Nithin Thanissery, Sarath Govindaraj, Subasree Ramakrishnan, Subhash Kaul, Suvarna Alladi

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: faheem2285@gmail.com

Background and Aim: A greater burden of risk factors and an increase in life expectancy are associated with an increasing trend in the number of dementia patients in India. Identifying risk and protective factors is essential for creating efficient management and prevention plans. The aim of the study was to determine the contribution of factors such as education, vascular risk factors, and the number of languages known in a large dementia cohort in South India. Methodology: Patients with cognitive impairment from the dementia registries of two centres in South India were included. All patients underwent clinical, cognitive, and imaging studies, and standard criteria were used for diagnosis. Vascular risk factor profiles, education status, and the number of languages spoken by the patients were noted. Results: Of the total 3440, VCI was the most common subtype (29%), followed by FTD (19%) and AD (17.8%). MCI comprised 11.3% of patient diagnoses, DLB 5%, mixed dementia 3.5%, questionable diagnosis 6%, and secondary dementia 6%. Overall, hypertension (51%), and diabetes (35%), were the most prevalent vascular risk factors. Other risk factors were stroke (28%), cardiac ailments (15%), chronic alcohol (13.4%), and smoking (12%). About 29% of patients had > 12 years of education, 26% had 7-12 years, 12.3 % had 1-6 years of education, and 11 % were illiterate. Among bilinguals, a higher proportion (55%) of patients had >12 years of education compared to monolinguals who had a higher proportion of no or low education status (27.1 & 21.2%). Both educational status and bilingualism have an inverse relationship with age at onset and severity; the effects of bilingualism are independent of other factors, including education. Discussion: Our results demonstrate a huge burden of vascular risk factors, especially hypertension, diabetes, and stroke, in both neurodegenerative as well as vascular dementia, and that bilingualism has protective effects across all subtypes of dementia. Conclusion:-.

Abstract ID: 806

Role of Vascular Endothelial Growth Factor in Tuberculous Meningitis: A Prospective Study form a Tertiary Care Centre in North India

Riya Sharma, Tanish Modi, Devesh Kumar, Manoj Goyal, Ritu Shree, Manish Modi, Deependra Singh, Rajeev Ranjan

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

E-mail: riya.090501@gmail.com

Background and Aim: The role of vascular endothelial derived factor (VEGF) in pathogenesis of tuberculous meningitis (TBM) remains far from clear. We prospectively evaluated role of serum and cerebrospinal fluid (CSF) VEGF in TBM. Methodology: This prospective case control study was conducted at a tertiary care centre in North India from Jan 2018 to June 2019. Consecutive drug naive patients (n=82) of TBM diagnosed on basis of modified Ahuja’s Criteria were included in the study. The results were compared with 49 control subjects (n=49). Serum and CSF VEGF were done in all the cases and controls. Follow up serum VEGF levels were done in 34 patients after three months of completion of antitubercular therapy. The VEGF level estimation was done using the human VEGF ELISA KIT. Results: Mean age of TBM patients was 29.9 ± 13.1 years. Study group consisted of 33 (40.2%) men and 49 (59.8%) women. Bactec MGIT960 was positive in 15 (18%) patients while multiplex tuberculosis polymerase chain reaction (PCR) was positive in 73 (89%) patients. Discussion: Serum and CSF levels of VEGF in TBM patients were not elevated when compared to controls. There was no association between final outcome in TBM and decrease in serum levels of VEGF at follow up. Conclusion: VEGF may not be playing a significant role in pathogenesis of TBM. Future studies with larger sample size may help in clearing role of VEGF further in TBM.

Abstract ID: 807

Scrub Typhus Presenting an Acute Hemorrhagic Leukoencephalitis

Arpan Mitra, Anand Kumar, Deepika Joshi, Anand Kumar, Deepika Joshi, Abhishek Pathak

Institute of Medical Sciences, Varanasi, Uttar Pradesh, India

E-mail: mitraarpan25@gmail.com

Background and Aim: Scrub typhus is a rickettsial infection caused by Orientia Tsutsugamushi, transmitted to the humans by the bite of trombiculid mite. Central nervous system (CNS) involvement is a known complication of scrub typhus, includes meningoencephalitis (most common), meningitis, encephalitis, encephalopathy, Seizure, myelitis, cranial neuropathies, mononeuritis multiplex, brachial plexopathy, GBS, and rarely ADEM and stroke. AHLE is a rare demyelinating disease of CNS marked by rapid progression and acute inflammation of the white matter. It is regarded as the most severe form of ADEM. The most frequent antecedent history is that of an upper respiratory infection. It usually occurs after a bacterial or viral infection, or post vaccination. Here I am sharing a case report of scrub typhus presenting as AHLE. Methodology: 12 year old girl presented with acute onset fever, headache, focal seizure with altered sensorium for 15 days. She was diagnosed as TBM based on CSF report outside and started on ATT. There was no significant improvement and she was brought to our institute. Extensive work up done to evaluate cause including repeat CSF analysis, MRI brain and all necessary investigations. Results: Patient’s IgM scrub typhus came positive. CSF showing lymphocytic pleocytosis with raised protein and reduced sugar. MRI showing features of hemorrhagic leukoencephalitis. CSF neuroinfection panel came negative. We stopped ATT, started on pulse steroid therapy and inj. Doxycycline for 14 days. There was significant improvement clinically and CSF parameters after treatment. Discussion: Though neurological manifestation is common in scrub typhus, there are very few literatures suggesting scrub presenting as ADEM. And there is no literature about scrub presenting as AHLE. Conclusion: ALHE is very severe and fatal disease. Many a times it remains undiagnosed due to lack of knowledge regarding its etiology. This case report will open our eyes to think of scrub typhus as a cause of ALHE.

Abstract ID: 808

Seizure in SCA 12: A Case Report

Sabyasachi Pattanayak, Deepika Joshi, Anand Kumar, Atanu Roy, Suyash Tripathy, Sanjeev Singh, Deepika Joshi, Anand Kumar, Varun Singh, Abhishek Pathak, Vijaya Mishra, Rameshwar Chaurasia

Banaras Hindu University, Varanasi, Uttar Pradesh, India

E-mail: sabya.pattanayak4@gmail.com

Background and Aim: Spinocerebellar ataxia type 12 (SCA12) is a very rare subtype of type I autosomal dominant cerebellar ataxia. It is characterized by the presence of action tremor associated with relatively mild cerebellar ataxia. Associated pyramidal and extrapyramidal features are reported. Other features include peripheral neuropathy, eye movement abnormalities and dyscognitive features. We report a SCA 12 patient with focal seizure, which is probably the first report of this presentation. Methodology: case report. Result: The patient had complaints of initial voice tremors, followed by upper limb tremors and head tremors with complaints of mild gait difficulties since 2009.these tremors and gait difficulties didn’t affect her quality of life much till 2014.on detailed history she gave similar family history and genetic analysis revealed 53 CAG repeats in PPP2R2 beta gene establishing the diagnosis. she remained in close follow up. In 2023, she presented with sudden onset multiple episodes of right sided focal seizures with secondary generalisation. She was thoroughly evaluated for vascular, infectious and metabolic aetiologies. Her MRI reveals gross atrophy with EEG showing focal slowing. she responded well to antiepileptics and gradually improved. Discussion: Myoclonus has been reported as a rare presentation in few studies, however to the best of our knowledge this is the first report of focal seizures. The significant cerebral atrophy maybe hypothesized to be a possible cause in the patient. Conclusion: This report is expected to throw new light into the ever expanding spectrum of SCA 12.

Abstract ID: 809

Severe Hyponatremia and CNS Manifestations

Vignesh Neethiarasu, Muthukumar Jeyaraj, P. K. Murugan

DM Neuro Pursuing, Other

E-mail: vigneshshantham@gmail.com

Background and Aim: Hyponatremia is one of the most common electrolyte abnormalities you encounter in hospitals.It is usually divided based on the volume status into hypovolemic,euvolemic,hypervolemic.Based on the time taken for the development of symptoms it is divided into acute and chronic.It is usually corrected using isotonic 0.9% normal saline or hypertonic 3% normal saline.The most common age group affected is elderly.This study aims at classifying the patients who presented with central nervous system manifestations and to evaluate the causes for it. Methodology: This prospective observational study includes patients who are aged more than 18 years with severe hyponatremia (defined as serum sodium levels <=120 meq/L and first clinical presentation was due to hyponatremia.Baseline patient characteristics (age,gender,prior medical conditions ,presentation symptoms and outcomes were analysed. Results: A total of 108 participants were analysed and it was found out that as per definition hypovolemic (n=60, 55.56%), euvolemic (n=33, 30.6%) and hypervolemic (n=2, 1.9%). cases which could not be classified as per definition were classified as undifferentiated (n=13, 12.1%).The varied presentation of symptoms range from altered sensorium which was the most common symptom present in 46 patients followed by giddiness which was present in 21 participants.8 patients had slurring of speech and 8 presented with metabolic seizures. Participants also presented with ataxia,, tremors, numbness ,limb weakness. Discussion: The presentation of acute hyponatremia in the form of central nervous system manifestations is a matter of concern because it is reversible and improves with either isotonic or hypertonic saline based on its volume status.identified Participants in this study have had subdural hematomas,craniectomy done secondary to road traffic accident,old cerebrovascular accidents and also malignancies. Conclusion: In this study the most common type of symptomatic hyponatremia was secondary to hypovolemia (55.56%).The most common CNS manifestation was altered sensorium (42.76%).The most common cause for euvolemic hyponatremia was SIADH.The most common prior medical illness was diabetes mellitus.

Abstract ID: 810

Shaking Up the Norm: Myoclonus Dystonia as an Unexpected Manifestation of SSPE in a Young Male

Abhishek Dixit, Abhishek Pathak, Vijaya Mishra

Institute of Medical Sciences, Varanasi, Uttar Pradesh, India

E-mail: abhidixi88@gmail.com

Background and Aim: Rare neurological conditions Myoclonus Dystonia (MD) and Subacute Sclerosing Panencephalitis (SSPE) often occur separately. This case demonstrates a rare MD manifestation in a young male patient with underlying SSPE. The goal is to raise awareness of this unusual pairing and the significance of etiological research in dystonia sufferers. Methodology: 19-year-old patient had dystonia and myoclonic jerks that had gotten worse during the previous 10 months. A thorough neurological examination, electroencephalogram, magnetic resonance imaging, and cerebrospinal fluid (CSF) investigation, which included measles antibody titers, were all performed. Results: Initial examination revealed right upper limb dystonia with myoclonic jerks. EEG was normal, and MRI was normal with some nonspecific white matter changes, whole exome sequence was sent which was normal. The diagnosis was confirmed when the CSF analysis revealed significantly elevated measles antibody titers, confirming a diagnosis of SSPE. The patient’s MD was considered secondary to SSPE. Discussion: This case emphasizes the potential for SSPE to manifest as MD, a rare presentation. The disease’s progression underlines the necessity for early measles vaccination, which could prevent the development of SSPE. Conclusion: This case highlights the necessity of thorough etiological evaluation in patients presenting with myoclonus dystonia in a child. SSPE, although rare, should be considered in the differential diagnosis when assessing patients with MD, particularly in regions where measles is prevalent. This emphasizes the importance of vaccination against measles to prevent serious neurological complications.

Abstract ID: 811

Silver Syndrome – Rare Case of Hereditary Spastic Paraparesis

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

DM Neuro Pursuing, Other

E-mail: drvinnu05@gmail.com

Background and Aim: Hereditary spastic paraplegias (HSPs) are a complex group of neurodegenerative disorders characterized by lower-limb spasticity and weakness. When spastic paraplegia is the only clinical feature, the disease is described as “pure” HSP; if accompanied by other neurological features, such as deafness, dementia, and mental retardation, the disease is described as “complicated” HSP. Silver syndrome (SS) is a disabling dominantly inherited form of HSP, complicated by amyotrophy of the hand muscles. Methodology: 8 years male child born of consanguineous marriage first order by birth with normal birth history and mild development delay in early childhood and intellectual disability, presented with history of spastic weakness of both lower limbs distal more than proximal For the past 3years . On examination patient had decreased subcutaneous fat, pes cavus , with atrophy of small muscles of hands and legs and spastic weakness of both Lower limbs distal > proximal and exaggerated reflexes. Pt had hepatosplenomegaly. His cranial nerves, sensory system, cerebellum, autonomic system was normal. Results: Routine blood investigation were normal. Imaging MRI brain with screening of spine was normal. Electrophysiological test also were normal. Genetic study revealed BSCL2 gene mutation. Discussion: The index case is a rare case of Silverman syndrome. Occurs due to mutation of BSCL2 gene that encodes seipin, a protein that is mainly localized to the endoplasmic reticulum membrane. The pathological feature common to both subtypes is axonal degeneration involving the longest fibers of the corticospinal tracts and of the dorsal columns, which may be associated with a loss of anterior horn cells. Conclusion: Mutations in BSCL2 gene cause two other allelic disorders, which are congenital lipodystrophy type 2 and autosomal dominant distal hereditary motor neuropathy type VA. Our patient had features of both lipodystrophy and Hereditary spastic paraparesis.

Abstract ID: 812

Skeletal Muscle Ion Channelopathies – Therapeutic Benefits

Anuradha Mahto, Kamlesh Jagiasi

Grant Medical College and JJ Hospital, Mumbai, Maharashtra, India

E-mail: anuradha.mahto@yahoo.in

Background and Aim: Skeletal muscle ion channelopathies have varied genetic mutations and impact on muscle function. They include, non-dystrophic myotonias (NDM), periodic paralysis (PP), and congenital myasthenic syndromes (CMS). The aim of the study emphasizes the importance of early recognition and appropriate treatment though symptomatic can have positive impact on the quality of life. Methodology: We analyzed 6 patients who presented with myotonia or weakness where investigated and treatment response was observed. Results: There is therapeutic benefit observed with acetazolamide, mexiletine and salbutamol in sodium/calcium channel, chloride channel mutation and CMS DOK7 respectively. Discussion: NDM typically occur in the first two decades. They present with stiffness of muscles on voluntary contraction in the absence of weakness and wasting. They include several disorders with clinical overlap. Mexiletine has been empirically used and is now approved as an orphan drug to treat both sodium and chloride mutation myotonia. Specific myotonias respond well to acetazolamide. PP present as generalized weakness and can be either due to hypokalemia or aggravated by potassium. Acetazolamide is effective in prevention of PP. DOK7 CMS clinically present as fluctuating limb girdle weakness, respiratory weakness without ophthalmoplegia that begins in early childhood (can present in adolescence or adulthood). Unless treated, symptoms progress with time. Salbutamol, the most effective treatment of DOK7 CMS, works by bypassing the step in receptor aggregation pathway that requires DOK7. Conclusion: Though the treatment of NDM and PP are symptomatic, early recognition and diagnosis can improve the quality of life. CMS should be considered in patients with fluctuating limb girdle weakness as initiating early treatment can prevent further muscle injury and disability. Genetic testing is paramount to confirm the diagnoses.

Abstract ID: 813

Spectrum of Neurological Manifestations, Inflammatory Markers and CSF Parameters in COVID 19 Patients: A Hospital Based Study

Amrut Dhulappanavar, Chandrashekhar Agrawal, Chand Wattal

Pursuing MD Paediatrics, Other

E-mail: amrut7760@gmail.com

Background and Aim: On March 11th 2020, the WHO officially announced COVID as a pandemic disease.COVID is a “multisystem disease” involving a complex interaction of the immunological, inflammatory and coagulative cascades. Differences in the host immune system lead to wide heterogeneity in the clinical picture, course and outcome.Neurological manifestations are due to direct viral neuro-invasion of nerve , vascular endothelium, leucocyte migration across blood brain barrier, and indirect mechanism includes hyper immune response, autoimmune response, hypercoagulable state, and secondary to hypoxic, metabolic and treatment related complications. Methodology: All the 150 RTPCR positive COVID patients of age more than 18 year as well as COVID recovered patients presenting with neurological manifestations within 6 weeks were enrolled for the study. As per standard diagnostic criteria clinical profile of the patients was described. All relevant investigations as per the diagnosis were done including inflammatory markers. CSF analysis was done in 94 selected patients who had encephalopathy, encephalitis, myelitis, AIDP , CNS demyelination , CNS mucormycosis. Results: Patients with severe COVID illness and of elderly group were more commonly affected. Patients with neurological problems and severe COVID disease have significantly elevated inflammatory markers Elevated CSF protein, lymphocytic pleocytosis and presence of COVID antibodies in CSF are more commonly seen in COVID patients with neurological manifestations. Discussion: Inflammatory biomarkers and CSF parameters are of great help in prognosis and plan of management. Various studies have decribed neurological manifestations and inflammatory markers in COVID patients globally and in India. Elevated inflammatory markers in COVID are useful in early suspicion of disease, classification of disease severity, identification of high risk population, prediction of outcome and prognosis. Conclusion: Our study observed important clinical information on COVID, that would raise clinician’s awareness regarding involvement of different neurological axis and its manifestations which helps in preventing mortality and morbidity.

Abstract ID: 814

Spectrum of Varied Presentatation of Dural AV Fistula

Naveen Prabhu, Balakrishnan Ramasamy, Smitha Chandran, S. Elango, S. Mohanakannan

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

E-mail: peenav007@gmail.com

Background and Aim: Dural arteriovenous fistula (DAVF) is an abnormal connection between arteries and veins within the dura mater, the outermost layer of the brain. This abstract aims to explore the diverse clinical spectrum of DAVF presentations, investigate the underlying pathophysiology, evaluate diagnostic methodologies, and summarize treatment outcomes. Methodology: Here we analysed a 5 cases which presented to the emergency with varied presentation. Results: The spectrum of DAVF presentations encompassed a wide range of neurological and ocular symptoms. Common manifestations included pulsatile tinnitus, headache, visual disturbances, and intracranial hemorrhage. Diagnostic modalities, such as digital subtraction angiography (DSA), magnetic resonance imaging (MRI), and computed tomography angiography (CTA), played crucial roles in confirming the diagnosis and assessing the angioarchitecture of DAVFs. Discussion: the varied presentation of dural arteriovenous fistula (DAVF) reflects the complex nature of this vascular abnormality and poses a diagnostic challenge for healthcare professionals. Understanding the diverse clinical manifestations is crucial for timely diagnosis and appropriate management. In this discussion, we delve into the factors contributing to the wide spectrum of DAVF presentations and their implications for clinical practice. Conclusion: Dural arteriovenous fistula presents with a broad spectrum of clinical manifestations, reflecting the complex nature of this vascular abnormality. Prompt and accurate diagnosis is crucial for appropriate management. Advanced imaging techniques, such as DSA, MRI, and CTA, are essential in delineating the angioarchitecture of DAVFs. Treatment decisions should be tailored to individual patients, considering the location, angioarchitecture, and symptoms associated with the DAVF.

Abstract ID: 815

Specturm of Fungal Infection of Cavernous Sinus in Immunocompatent Patients: A Series of 3 Patients

Soumozit Banerjee, Chayan Mondal, Uddalak Chakraborty, Arijit Ray, Souvik Dubey

Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

E-mail: soumozit.banerjee@gmail.com

Background and Aim: Cavernous sinus is one of important structures in the venous system of brain as multiple structures including internal carotid artery with sympathetic trunks, multiple cranial nerves are adjacent to it . Symptomatically cavernous sinus syndrome can present with fever, secondary headache, multiple cranial nerve palsies, exophthalmos. Fungal involvement of cavernous sinus is often associated with various immune compromised states. But in this article, the authors are going to present 3 patients , otherwise Immune-competent, diagnosed as fungal infection of cavernous sinus. Methodology: In this case series, the first patient is 26y-old- female presented with mild headache with blurring of vision for last six months. The second patient, 45y-old-female, presented with headache, progressive ptosis and Diplopia. The third case was 59 yr old male presented with headache followed by ptosis and dimness of vision for last 6 months followed by sudden onset hemiparesis. Results: the first 2 patients were diagnosed having aspergilosis by biopsy and are under our follow up. The 3rd patient was diagnosed having fungal infection on the basis of imaging finding and was managed accordingly but succumbed to death. Discussion: Among the different etiology of cavernous sinus syndrome, fungal involvement by Aspergillus and Mucor is worth mentioning. Indolent course, involvement of cavernous sinus related structures and neuro-imaging findings including diffusion restriction and contrast enhancement, poly sinusitis, angio-invasion characterise fungal etiology but still biopsy is gold-standard. In our cases, all 3 patients were biopsied and 2 of them revealed Aspergillus but 1 of them was provisionally diagnosed by imaging and exclusion of other possibilities and managed with anti-fungals. Conclusion: Fungal etiology is to be considered in every case of cavernous sinus syndrome depending on clinical and radiological scenario.

Abstract ID: 816

Spontaneous Intracranial Hypotension - A Case Series of Six Patients Treated with Targeted Epidural Patch Therapy

Bhuvaneswari Maddali, V. L. Arul Selvan

Sri Venkateswara Institute of Medical Sciences, Tirupati, Andhra Pradesh, India

E-mail: Brinda13m@gmail.com

Background and Aim: Spontaneous Intracranial Hypotension (SIH) is a relatively rare cause of headache, with incidence of <5 in 100,000 in general population. Despite the classic features of orthostatic headache, low CSF pressures (<6 cm CSF) and corresponding findings on neuroimaging, it is not uncommon to encounter a more varied presentation. Targeted epidural blood patching provides effective management of this condition, as outlined in this case series. Methodology: Six cases of SIH are presented here. All patients underwent brain imaging, CT myelography to demonstrate CSF leak followed by targeted epidural blood patching for symptom resolution. Results: All six patients presented with headache worsening on erect posture. While CT myelography showed contrast extravasation in four patients, no demonstrable leak was found in two patients. All patients responded to targeted epidural blood patching at cervicodorsal level. Discussion: SIH is actually because of decrease in volume of the CSF from a spontaneous spinal dural leak that leads to decrease in CSF pressure and sagging of the brain within the skull. Resultant traction on pain sensitive structures causes a clinical syndrome with postural headaches, but non postural headaches and other neurologic symptoms may also occur. SIH is more common in women aged between 45 to 55 years. Associated clinical features of diplopia, tinnitus, photophobia, phonophobia, nausea and vomiting are not uncommon, as were observed in our patients. MRI Brain shows characteristic findings of “brain sagging” in the skull and subdural hygromas. A CT myelography may often, but not always, confirm the diagnosis by identifying the site of leak, usually at cervicodorsal level. Targeted epidural blood patching provides good clinical response. Conclusion: History of orthostatic worsening of a chronic daily headache should raise a high index of suspicion for SIH. Once diagnosed, targeted epidural blood patching should be considered in patients not responding to conservative management.

Abstract ID: 817

Story of Three: An Amoebic Tale

Debarup Das, Souvik Dubey, Arijit Ray, Sanjoy Chatterjee, Kuntal Biswas, Kokila Banerjee

Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India

E-mail: debarup1992das@gmail.com

Background and Aim: Acanthamoeba is a free a living amoeba which known to cause keratitis, encephalitis, disseminated infection in human beings. Granulomatous amoebic encephalitis (GAE) is classically seen in immunocompromised hosts. Methodology: Here, the authors presents 3 patients with meningoencephalitis presenting from eastern India which subsequently was proved to be a cases with acanthamoeba associated meningoencephalitis. Results: First patient had a very acute fulminant presentation, like acute encephalitis syndrome with background history of fresh water drowning, second one presented with typical granulomatous amoebic encephalitis (GAE) and third with concurrent Central Nervous System (CNS) tuberculosis and acanthamoeba. Acanthamoeba spp. was identified on CSF wet mount examination and subsequently proven by culture and PCR (polymerase chain reaction) test. Magnetic Resonance imaging (MRI) showed multiple hemorrhagic infarcts with leptomeningeal enhancements in first case, vasculitic infarct with meningeal enhancement in second case, and the last case had basal meningitis, vasculitic infarcts and spinal subdural collection. CSF showed neutrophilic pleocytosis in all three patients, who were apparently immunocompetent as was inferred by negative triple serology, euthyroid, non-diabetic status , absence of history of immunosuppressive drug intake or any immunological diseases. Discussion: Acanthamoeba related meningoencephalitis can be fatal as it was in our first case if not presented and diagnosed early. Among three cases only one had history of fresh water drowning. Interestingly, we had neutrophilic pleocytosis in all cases which is very odd in such cases. Following microbiological diagnosis of Acanthamoeba we had started the patients on combined regimens with fluconazole, sulfamethoxazole-trimethoprim and rifampicin. Amongst the three, last two have responded very well to our combined regimen but the patient with fulminating presentation succumbed to the grave infection with in a few days. Conclusion: Acanthamoeba as an etiological agent must be suspected even in immunocompetent hosts. Early diagnosis and appropriate antibiotic regimen coverage is pertinent in these scenarios.

Abstract ID: 818

Subacute Encephalopathy with Seizures in Alcohol Consumers (SESA): A Case Series

Soaham Desai, Devangi Desai

DM Neuro Completed, Other

E-mail: drsoahamdesai@yahoo.com

Background and Aim: Subacute Encephalopathy with Seizures in Alcohol consumers (SESA) syndrome is a rare condition characterized by subacute onset of encephalopathy with seizures, which occurs in chronic alcoholics who abruptly stop or reduce their alcohol intake. The purpose of this case series is to describe the clinical and electrophysiological features of SESA syndrome presenting as focal seizures mimicking dyskinesias and myoclonus, and to emphasize the importance of early recognition and management. Methodology: We report a case series of 9 patients with SESA syndrome who presented to a medical teaching hospital in rural Gujarat, India, between 2016 and 2021. Results: The patients had a mean age of 42 years (range 28-62), and all had a history of chronic alcoholism. The most common presenting symptom was focal seizures, which were misinterpreted as chorea-ballism or stroke in some cases. Myoclonus was also a common finding. Electroencephalography (EEG) showed diffuse slowing and epileptiform discharges. All patients received treatment with thiamine and anti-epileptic drugs, resulting in significant improvement in symptoms and resolution of abnormal EEG findings. Discussion: SESA syndrome can present with focal seizures mimicking dyskinesias and myoclonus, which may be misinterpreted as movement disorders. Early recognition and treatment with thiamine and anti-epileptic drugs can improve prognosis. The limitations of this case series include the small sample size and the fact that it was conducted in a single center. Conclusion: Clinicians should consider SESA syndrome in the differential diagnosis of patients with subacute encephalopathy and seizures, particularly in chronic alcoholics.

Abstract ID: 819

Status Epilepticus as a Presenting Feature of Posterior Reversible Encephalopathy Syndrome: Retrospective study from Tertiary Care Centres

Pratibha Prasad

All India Institute of Medical Sciences, Patna, Bihar, India

E-mail: pratibhaprasad12@gmail.com

Background and Aim: Background Though epilepstic seizures are common in Posterior reversible encephalopathy syndrome ( PRES) , status epilepticus as the presenting feature is rare. Objective: To study the clinical profile and outcome of patients with status epilepticus as presenting feature of posterior reversible encephalopathy syndrome. Methodology: The diagnosis of PRES was established by a neurologist on the basis of characteristic clinical features and radiologic changes on brain MRI (n=40). Six months follow-up imaging was obtained to confirm the resolution of imaging findings. Patients with Status epilepticus (SE) as the initial manifestation of PRES were identified. We collected information on clinical presentation, seizure phenotype, brain MRI findings, EEG studies, antiepileptic treatment, previous history of seizures, associated comorbidities, presumed etiology of PRES, and long-term clinical outcome. Results: Mean age of the patients at presentation was 23 yrs in females and 46 yrs in males. Most common presentation was seizures, seen in 31 patients (77.5%). Primary etiologies of PRES included pre-eclampsia, or eclampsia (n = 33 [82.5%]), hypertensive encephalopathy (n = 3 [7.5%]), SLE , AIP, and CRF in one patient each (n= 01, [2.5%]). MRI brain showed parieto occipital lobes ( n=33 [ 82.5%]) involvement in maximum cases. SE was the presenting feature in 8 cases [20%] All had generalized convulsive SE. Among them , 5 [0.5%] had a history of chronic epilepsy. In remaining three patients, SLE and acute intermittent porphyria, CRF precipitated the SE. Discussion: Though SE in the context of PRES carries a favorable prognosis but requires timely recognition and treatment of the course of PRES. Role of antenatal care is important for identifying, and treating associated comorbidities; BP control ,ensurance of proper compliance of AED and proper councelling. Conclusion: This study highlights that awareness of varying spectrum of clinical presentations, and etiology of PRES leading to early diagnosis and management carries a favourable prognosis.

Abstract ID: 820

Targeted Metabolomics in Children with Autism Spectrum Disorder

Chakkera Priyanka

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: priyankachakkera@gmail.com

Background and Aim: Early diagnosis and intervention in children with autism spectrum disorder (ASD) is crucial. Diagnosis of ASD is primarily based on subjective assessment tools. Identifying metabolic biomarker will aid in early diagnosis. Aim is to conduct targeted metabolomic analysis and determine the plasma metabolic features that can discriminate children with ASD from healthy controls (HC). Methodology: A prospective, case-control study was conducted in Department of Neurology, Child and Adolescent Psychiatry, NIMHANS, Bengaluru between June 2021- March 2023 after obtaining ethical approval. Fasting plasma sample was obtained from 70 children with ASD and 58 HC, aged 2 to 12 years after written informed consent. ASD children were classified into ASD with regression (n=36, 51.4%) and without regression (n=34, 48.4%). Samples were quantitively analysed for 55 targeted metabolites (13 amino acids (AA), 40 acylcarnitine levels, adenosine, 2-deoxyadenosine levels) using tandem mass spectrometry. Statistical analysis was done by biostatistics department. Results: Total of 26 metabolites (11 AA’s, 14 acyl carnitines and adenosine) were found significantly different between ASD and HC. Among cases, short and long-chain acyl carnitine levels were significantly decreased, medium-chain acyl carnitine levels were significantly increased. Adenosine and eleven out of thirteen AA levels were significantly decreased among ASD. Octenoylcarnitine (Cut-off value-0.025 mmol/L, AUC-0.683, p value <0.05, sensitivity-55.7%, specificity-79.3%) and Adenosine (Cut-off value-0.025 mmol/L, AUC-0.673, p value <0.05, sensitivity-57.1%, specificity-72.4%) could predict ASD from controls. ASD could be classified using metabolites by machine learning models like Support vector machine (Sensitivity-75%, Specificity-74%) and Random Forest models (Sensitivity-75%, Specificity-82.6%). Discussion: Both decreased amino acid and acylcarnitine levels in ASD can be due to malnutrition or gastrointestinal disturbances. However, we were not able to find significant differences within ASD subgroups. Conclusion: Amino acids, acylcarnitine and adenosine metabolism are mainly altered in ASD compared with HC. Octenoylcarnitine and adenosine might be potential biomarkers of ASD.

Abstract ID: 821

Takayasu Arteritis: A Rare Cause of Convulsive Syncope

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

Banaras Hindu University, Varanasi, Uttar Pradesh, India

E-mail: anand.2005.02@gmail.com

Background and Aim: Takayasu arteritis is a stenotic, inflammatory disease of medium-large sized arteries involving predominantly aortic arch and its branches. Symptoms may vary from mild constitutional symptoms to serious ischemic symptoms. Methodology: NA. Results: A 15-years-old female presented to neurology outpatient department with complaints of frequent loss of consciousness for six months. Patient visited multiple paediatricians and started on anti-seizure medications. Despite good compliance and multiple antiseizure medication her symptoms not resolved. After admission at out institute patient reported episodes of black out before loss of consciousness. She also reported increased episodes of loss of consciousness after head tilting or turning. On examination there were no pule in both radial artery and brachial artery very feeble pulse. Blood pressure is not recordable in both upper limbs while 90 mm of Hg in right lower limb and 170/98 mm of Hg in left lower limb. A palpable thrill present over right carotid artery and there are laude bruits noted on right carotid and left renal artery. Rest of all cardiovascular system examination was normal. No other obvious neurological deficit was observed. Blood investigations showing anaemia with haemoglobin 10.2 gm/dl, raises Erythrocyte sedimentation rate (ESR), and C-reactive protein, with normal liver and renal function tests. Computer tomography angiogram done showing significant stenosis of both lest and right common carotid artery proximally. Along with bilateral subclavian complete stenosis with extensive collaterals, right pulmonary artery, narrowing in right common iliac and internal iliac artery. Abdominal aorta narrowing infra renal (D9-L2) luminal stenosis around 60% along with right renal artery ostial Stenosis. MRI brain and EEG was normal. Final diagnosis was made as type V Takayasu arteritis with syncope and convulsive syncope. Discussion: NA. Conclusion: Detail history and proper clinical examinations can helps in diagnosing serious underlying pathology like Takayasu arteritis causing syncope, mimicking epilepsy.

Abstract ID: 822

Successful Treatment of Superior Cerebellar Artery Arteriovenous Malformation in a 3-year-Old Child by Onyx Embolisation

H. Ahamed Humayoon, P. Rashma Mohammed, Fazal Ghafoor, Aswathy Sasidharan, Firosh Khan, Fazal Ghafoor, Anil Karapurkar, Rakesh Singh, Riddhiben Patel

DM Neuro Completed, Other

E-mail: ahamedsubirh@yahoo.com

Background and Aim: Occurrences of cerebral AVMs are uncommon in the pediatric age group. Management of cerebral AVMs can be done with embolization, surgical removal and radiosurgery. Embolic agents like N-butyl cyanoacrylate (n-BCA) were used before but with a success rate of only 10%. A newer liquid embolic agent called Onyx made up of ethylene-vinyl alcohol dissolved in dimethyl sulfoxide (DMSO) is able to penetrate deeper into the nidus. We present a case of a 3-year-old child who presented with headache and vomiting followed by loss of consciousness. He was having ataxia, which on evaluation revealed a ruptured right superior cerebellar artery AVM and was successively managed with Onyx embolization. Methodology: Patient details and images of CT, MRI, MRA and angiography were collected for reporting. Results: Our patient presents with right-sided ataxic features with hemorrhagic right cerebellar stroke due to the ruptured superior cerebellar AVM. The AVM was further draining to the cerebellar veins. Detachable tip microcatheter over Hybrid 008 microwire was advanced to the right superior cerebellar artery and placed close to the AVM under road map guidance. Onyx 18 was injected into the AVM under a blank roadmap. Complete occlusion of the AVM and aneurysm was obtained. Post-procedure significant improvement in the gait and ataxia was noted in 2 days. The child presented after 2 weeks of follow-up with complete resolution of neurological deficits. Discussion: Pediatric AVMs constitute about 3% of all AVMs.Studies have indicated that AVMs trigger about 2% of all strokes and 4% of hemorrhagic types. Endovascular embolization is a radiological treatment modality that can rapidly eradicate angiographic risk factors. The prognosis of pediatric AVM is usually good if well-managed. Conclusion: We successfully treated this 3-year-old child with ruptured superior cerebellar AVM using Onyx embolization with complete resolution of the neurological deficits.

Abstract ID: 823

Subhyaloid Hemorrhage: A Rare Manifestation of CVT

Mangalapalli Vijay, Sakthi Velayutham, K. Mugundhan

DM Neuro Pursuing, Other

E-mail: vijaymangalapalli@gmail.com

Background and Aim: Cerebral venous thrombosis (CVT) is a cerebrovascular disorder that causes venous infarction and intracerebral hemorrhage (ICH) due to occlusion of cerebral veins or venous sinuses. Approximately, one-third of the patients with CVT develop cerebral hemorrhage. Here, we present a rare case of CVT presenting with subhyaloid hemorrhage in the eye. Methodology: An 26-year-old gentleman presented with complaints of holocranial headache, 1 episode of vomiting, 2 episodes of seizures, and sudden painless diminution of vision in the right eye of 10 days duration. Ophthalmic examination revealed constriction of visual fields in right eye with visual acuity reduced to finger count and impaired colour vision. Fundus examination revealed bilateral papilledema with large preretinal subhyaloid bleeding in the temporal retina of the right eye. Results: MRI brain imaging with MRA & MRV was done. MRI brain showed tortuous optic nerves & Magnetic Resonance Venography showed thrombus in right transverse sinus, right sigmoid sinus, straight sinus, posterior 1/3rd superior sagittal sinus and right internal jugular vein. Procoagulant and vasculitic workup was negative. Discussion: CVT is a cerebrovascular disorder that causes venous infarction and ICH due to occlusion of cerebral veins or cerebral venous sinuses, accounting for 0.5% to 1.0% of all strokes1. Diagnosis and management can be difficult because of diversity of underlying risk factors and variation in clinical presentation2. Headache, generally indicative of an increase in intracranial pressure, is the most common symptom of CVT3. Among patients with CVT, cerebral hemorrhage is seen in 30–40% of patients4. Our patient had right eye subhyaloid hemorrhage without any evidence of intracerebral hemorrhage which is a very rare presentation of CVT. Anticoagulation therapy in CVT helps to prevent thrombus formation and facilitates recanalization. Conclusion: CVT with subhyaloid hemorrhage is a very rare presentation. High index of suspicion is needed for diagnosis of CVT presenting with subhyaloid hemorrhage. Identification of these patients is important as early medical intervention can cause early resolution of symptoms and thereby salvage the vision.

Abstract ID: 824

Pediatric Arterial Ischemic Stroke – Role of Infection/Inflammation

Manoj Prabhakar, Kingsley- Jebasingh, Sethuram, Shankar Ganesh

Pursuing MD Medicine, Other

E-mail: mailtodr.manojprabhakar@gmail.com

Background and Aim: Perinatal stroke encompasses cerebrovascular event that occurs as early as 20 weeks of gestation up to 28 days. Methodology: nil. Results: nil. Discussion: We herein report a case of a 45-day-old premature female, severely underweight, stunted infant with a history of respiratory distress syndrome after stabilization had undergone a procedure for retinopathy of prematurity and during the post-op period developed involuntary movements of limbs that lasted for 15 minutes and referred to higher center. On arrival, she was febrile, apneic, and lethargic and was intubated. Clinically severe pallor, fair hydration, anterior fontanelle full, head circumference- 33 cm, no neck stiffness, hypotonia, spontaneous eye opening, sluggishly reacting to light. She was started on intravenous levetiracetam and midazolam infusion MRI done before intubation showed acute infarct with hemorrhagic transformation in bilateral frontal, temporal, parietal with a thin rim of subdural hemorrhage in bilateral frontal convexity. Hemogram revealed Hb- 6.5 g/dl, TC- 6500, Platelet-29000, with liver function showing indirect hyperbilirubinemia. Multiple packed cell and platelet transfusions were done. The blood culture showed pseudomonas and was treated with appropriate antibiotics. Counts improved, weaned off the ventilator, converted to oral antibiotics, and discharged with oral AED. Follow up MRI- showed cystic encephalomalacia. Conclusion: Infections play an important role in the pathogenesis of acute ischemic stroke, even minor childhood infections are associated with increased risk, Agents such as parvovirus B19, dengue, covid. Herpes may lead to focal cerebral arteriopathy. Most cases of intracerebral hemorrhage are idiopathic, but some are attributed to thrombocytopenia, hemophilia, coagulopathies, vascular anomalies, and aneurysms. We report this case suffering from infarct with hemorrhagic transformation secondary to severe sepsis and thrombocytopenia implicating infection and inflammation as primary pathogenesis. A better understanding of the role of the host immune response in stroke pathogenesis, paired with targeted immune-modulating therapies, may lead to more effective interventions with fewer side effects.

Abstract ID: 825

Complete Recovery in Cryptogenic NORSE with Early Immunotherapy - A Case Report

Miriyala Deepthi, Pamidimukkala Vijaya, Ramachandra Yelamanchi

MBBS completed, Other

E-mail: deepsmiriyala@gmail.com

Background and Aim: Background: Norse is defined as a clinical presentation with refractory status epilepticus in a previously healthy individual without structural, toxic, and metabolic causes. Around half of the NORSE patients are found to be cryptogenic. Although the exact pathology of cryptogenic NORSE is not defined, an immune mediated mechanism is suggested. Objectives: To describe a case of 15 years old presented to a tertiary care center with cryptogenic NORSE, characteristic clinical and EEG findings, and Response to aggressive immune therapy and IV anesthetic agents. Methodology: A descriptive case report. Results: We describe a 15-yr-old boy presented with history of seizures following a febrile illness which were infrequent at presentation and gradually progressed to status epilepticus. His seizures had a shifting ictal activity with different semiology. His Interictal EEG showed Beta delta complexes resembling extreme delta brush. His Ictal EEG showed prolonged fast activity followed by spike and wave ictal rhythm. His MRI brain was normal. CSF study, metabolic, Infective and Autoimmune workup were negative. Patient was treated with AED, IV MP started on day3 for 5 Days along with Plasmapheresis and Inj Rituximab. Later patient was treated with IV anesthetics to attain burst suppression. The patient made a complete recovery without any neurological sequalae and recurrence of seizures. Discussion: Here we present a young boy presented with cryptogenic NORSE following a febrile illness who had characteristic clinical and Electroencephalographic findings of NORSE. He was treated with early aggressive immunotherapy and IV anesthetic later. However, patient made a complete recovery without any sequelae. Conclusion: Diagnosis of NORSE is given late in a clinical course. Identification of characteristic clinical EEG clues described in proceedings of the First International new-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) Symposium, and early aggressive Immune therapy and IV anesthetics helps in reducing significant mortality and morbidity.

Abstract ID: 826

ARSACS- Autosomal Recessive Spastic Ataxia of Charlevoix–Saguenay- A Rare Diagnosis in Indian Scenerio

Sidharth Sharma, Abdul Qavi, Ajai Singh, Pradeep Maurya, Dinkar Kulshreshtha

DM Neuro Pursuing, Other

E-mail: sidharthsharma1992@gmail.com

Background and Aim: ARSACS is a neurodegenerative disorder with the characteristic triad of cerebellar ataxia, spasticity, and peripheral neuropathy. It was originally described in 1978 from Charlevoix-Saguenay-Lac-St-Jean region, Quebec in Canada. Subsequently, it has been reported from other parts of the world, including India. However, only a few genetically proven cases of ARSACS have been reported from India. Methodology: 21 year old male presented with progressive difficulty in walking since 6 years of age associated with simultaneous stiffness of both the lower limbs . After about 2 years, patient developed distal weakness followed by swaying to either side while walking. On examination Pes cavus and Hammer toe was present along with Gaze evoked nystagmus , Hypermetric Saccades. Spasticity present (LL>UL). Vibration –Impaired up to bilateral ankle joint , Pain and temperature –intact. DTRs Right - BJ3+,TJ3+,SJ3+KJ3+AJ- ; Left- BJ3+,TJ3+,SJ3+KJ3+AJ- , Plantar –bilateral extensor, Cerellar signs were present. Rest Systemic examination was normal. Routine investigations were normal; NCV- demyelinating motor polyneuropathy while sensory NCS was not recordable. MRI Brain showed Superior vermis atrophy along with LinearT2/FLAIR hypointensities in pons s/o striped Pons, suggesting the possibility of ARSACS. Results: Diagnosis was confirmed by Clinical Exome sequencing showing heterozygous frameshift mutation in Exon 10 of SACS gene. Discussion: ARSACS is a neurodegenerative disorder, with the characteristic triad of cerebellar ataxia, spasticity, and peripheral neuropathy . Disease onset is in early childhood, leading to delayed walking because of gait unsteadiness in very young toddlers. Recent advances in molecular genetic testing confirmed it to be one of the most prevalent autosomal recessive ataxia disorders worldwide. Conclusion: Taking into consideration, ARSACS is being reported increasingly from all parts of the world, it should be considered in the differential diagnosis of spastic ataxia syndromes. The typical clinical features and neuroimaging findings should be followed by clinical exome sequencing to identify variations in the SACS gene.

Abstract ID: 827

Autosomal Recessive Spastic Ataxia of Charlevoix- Saguenay- A Case Series

Mit Raval, Vikram Holla, Sneha Kamath, Nitish Kamble, Jitender Saini, Ravi Yadav, Babylakshmi Muthusamy, Pramod Pal

National Institute of Mental Health and Neuro-Sciences, Bengaluru, Karnataka, India

E-mail: mitraval1994@gmail.com

Background and Aim: Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS) is a rare neurodegenerative condition that presents with cerebellar ataxia, lower limb spasticity and neuropathy with characteristic MRI findings. This study aims to describe the clinical, radiological, and genetic profiles of four cases of ARSACS. Methodology: A chart review. Results Four cases with a median age at onset of 5 years and a median age at presentation of 23 years (range: 15-23 years) were studied. Three were born from consanguineous parentage, and one had a positive family history. Imbalance while walking was the most common presenting symptom with additional dysarthria, poor scholastic performance, dexterity issues, and dystonia. Three of four were cognitively preserved, two had dysmetria, two had hypermyelinated fibers radiating from the optic disc on the fundus with thickened RNFL on OCT, three had gaze-evoked nystagmus and all had ataxic- dysarthria. Bilateral lower limb spasticity was seen in all with additional distal hypotonia in one, mild lower limb weakness in two, brisk reflexes in three, absent ankle reflex in two, and extensor plantar in three. Sensory examination showed impaired JPS and vibration in lower limbs in two patients. Abnormal cerebellar signs with spastic-ataxic gait were noted in all four patients. MRI Brain revealed classical transverse T2 hypointensity in the pons with bulky pons, hyperintensity in the perithalamic region, and superior cerebellar atrophy in all patients along with atrophy of the cord in three patients. NCS showed sensorimotor axonal and sensorimotor demyelinating neuropathy. Clinical exome sequencing revealed disease-causing variants in SACS gene in all four patients, homozygous in three (c.6000_6004del;p.Arg2002CysfsTer25, c.8240T>A;p.Ile2747Asn, c.13531del;p.Glu4511AsnfsTer9), and compound heterozygous in one (c.10686_10689delCTTT;p.Phe3562Leufs*8 and c.3810delT;p.Phe1270Leufs*4). All patients were managed with supportive medication and physiotherapy. Discussion: Combined with results. Conclusion: A comprehensive understanding of the clinical, radiological, and genetic profiles associated with ARSACS is crucial for early diagnosis and effective management.

Abstract ID: 828

To Evaluate the Effectiveness of Add on Repetitive Transcranial Magnetic Stimulation in Management of Post-stroke Spasticity: An Open Label Randomized Control Trial

Yuvraj Lahre, Sanjeev Bhoi, Menka Jha, Suprava Naik, Priyanka Samal

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

E-mail: yuvi.10170@gmail.com

Background and Aim: Spasticity is a common complication of stroke limiting patients’ daily activities. Management of spasticity in chronic stroke patients is necessary to improve their quality of life. Transcranial magnetic stimulation is a painless and non-invasive method that can potentially reduce post-stroke spasticity. In this study we want to evaluate the effectiveness of 3 sessions of low Hz rTMS therapy in reducing the spasticity by analysing the changes in modified Modified Ashworth Scale (mMAS). Methodology: Patients with upper limb spasticity after stroke of 3 months duration were randomised into active and sham groups. In the active group three treatment session, 1000 pulses of 1 Hz rTMS at an intensity of 90% of resting motor threshold were applied to the primary motor area of the contra-lesional hemisphere. mMAS was measured pre and post therapy in each group and also at one month follow up. Results: 106 patients were screened of which 50 patients were selected. 25 patients allotted to active group and 25 to sham group. We found significant reduction in spasticity across all joints in rTMS group. Mean mMAS score before and one month after therapy were 2.83±1.16 vs 2.17±.92 across shoulder, 3.33±.96 vs 2.83±.96 across elbow, 3.08±1.06 vs 2.29±1.04 across wrist and 2.54±1.25 vs 1.83±1.00 across finger joints with p value of .001 for all joints. No significant change was found in sham group. Discussion: We found that three sessions of rTMS therapy can be helpful in reducing the spasticity among post-stroke patients. Previous studies have shown similar results but our study is first among Indian population. We also evaluated the effectiveness of rTMS based on size, location and type of stroke which was not done in previous studies. Conclusion: rTMS is safe and feasible method for management of spasticity among post-stroke patients. Further studies are needed to confirm these findings.

Abstract ID: 829

Outcomes of Axonal Guillain Barre Syndrome in an Indian cohort: Is it Time to Revise Strategy?

Sowri Rajan Rajendran, Ajith Sivadasan, Sanjith Aaron, A. T. Prabhakar, Aditya Vijayakrishnan Nair

Christian Medical College, Vellore, Tamil Nadu, India

E-mail: rajanrajen92@gmail.com

Background and Aim: Axonal GBS forms a unique subset with different immunopathogenesis and could respond differentially to different immunotherapies . Few studies described the clinical profile and outcomes in axonal GBS in the Indian population To study the outcomes of axonal GBS and compare efficacy of IVIG compared to plasma exchange in this cohort. Methodology: A retrospective observational study. Among 206 consecutive GBS patients admitted during the study period (2018-23), 44 axonal GBS were included based on electrophysiological criteria . Demographic data, clinical details, outcomes and hospitalisation costs were analysed. Results: Anti ganglioside antibodies were positive in 53.8%. The median duration of illness was 7 days .Hughes grading on admission, discharge and follow up were 3.51 (SD 1.08),2.55 (SD 1.11) and 0.88 (SD 1.25) respectively.33 patients received IVIG and 11 underwent therapeutic plasma exchange. Between IVIG and PLEX cohorts, age (33 vs. 36,P = 0.10),duration of illness (10.12 vs. 8.4 ,p=0.87),Hughes admission grade (3.52 vs. 3.50,p=0.24),discharge (2.48 vs 2.8,P=0.07) and follow up (0.9 Vs 0.7, p=0.48) and hospitalization duration (10.03 Vs 13.4, p=0.19) were comparable. On subgroup analysis, patients who received IVIG early (< 7 days) had reduced hospital stay (8 vs. 11.9, P=0.004*) and better Hughes grade at discharge (2.09 Vs 2.81, P=0.03*). Discussion: In contrast to demyelinating GBS with complex immune mechanisms, axonal GBS is antibody mediated and may present with severe disease with significant morbidity and mortality. IVIG has specific action whereas therapeutic plasma exchange non- specifically removes antibodies and immune mediators. In our study, we found early administration of IVIG in axonal GBS is associated with early recovery, reduced hospital stay with good safety profile without the complications of plasma exchange. Conclusion: Administration of IVIG early in disease course in axonal GBS may be associated with improved outcomes and offers attractive treatment strategy with lesser burden and utilization of health care resources.

Abstract ID: 830

Case Series of Idiopathic Hypertrophic Pachymeningitis

Sridevi Natrajan, M. Rajasekaran, N. Kannan, E. Arunraj, R. Kishore

DM Neuro Pursuing, Other

E-mail: srintrjn.4@gmail.com

Background and Aim: Hypertrophic pachymeningitis is a fibrosing inflammatory process causing thickening of the dura mater. ‘Primary’ or ‘idiopathic hypertrophic pachymeningitis where no identifiable cause is found and ‘secondary’ where identifiable causes like infective, inflammatory, infiltrative pathology is present. Here three cases of idiopathic hypertrophic pachymeningitis are reviewed. Methodology: This is a retrospective review of three cases admitted in KAPV Govt Medical College,Trichy and diagnosed to have Idiopathic Hypertrophic Pachymeningitis. All three patients had headache and Two of three patients presented with optic neuropathy and one of the three patients presented with proptosis and Papilledema of both eyes. Clinically no patients had signs of meningeal inflammation. On MRI, dura mater appears to be thickened in all three patients which was isointense on T1WI and hypointense on T2WI with uniform enhancement of the thickened meninges. Clinical or biochemical and CSF evidence of any predisposing condition causing hypertrophic pachymeningitis were ruled out in all three patients. All three patients were treated with steroids. Results: On follow up all three had resolution of clinical symptoms and MRI findings. Discussion: Hypertrophic pachymeningitis causes recurrent cranial neuropathies and headaches, cerebellar dysfunction and may produce focal neurologic deficits . Secondary causes are syphilis, tuberculosis, and Lyme disease, IgG4-related disorder, rheumatoid arthritis, sarcoidosis, Behcet disease, and Sjogren syndrome, ANCA assosciated vasculitis and malignant conditions. Idiopathic hypertrophic pachymeningitis is, thus, a diagnosis of exclusion. Idiopathic variety is treated with steroids and Secondary causes are treated specifically. Conclusion: Hypertrophic pachymeningitis can be diagnosed with MRI contrast of brain when there is a high index of suspicion. Exclusion of secondary causes and treatment of idiopathic variety with steroids will prevent permanent neurologic deficit.

Abstract ID: 831

An Atypical Presentation - Descending Guillain- Barre Syndrome

Manoj Prabhakar, Kingsley Jebasingh, Sethuram, Shankar Ganesh

Pursuing MD Medicine, Other

E-mail: mailtodr.manojprabhakar@gmail.com

Background and Aim: Guillain-Bare syndrome and its variants form a continuous spectrum of overlapping syndromes. We herein report a case of a nine-year-old male child with an unusual presentation of Guillain-Barre syndrome. Its presentation is usually of symmetric ascending type of paralysis. Methodology: nil. Results: nil. Discussion: The developmentally normal nine-year-old presented with a change in voice, difficulty in articulation, pooling of secretion, and dyspnea for one day. Over the course of his stay the next day he developed symmetrical flaccid weakness of the upper limbs which gradually progressed to involve both lower limbs, with no bowel or bladder involvement. One week before the illness he had a fever with respiratory symptoms, treated with oral antibiotics. Clinically he had normal higher mental function with, bifacial weakness, diminished gag reflex, pooling of secretions, hypotonia of all four limbs, with the power of 3/5 and neck weakness, areflexia of all deep tendon reflexes, with no sensory involvement. MRI spine -normal, all basic blood investigations were normal. CSF analysis showed albuminocytological dissociation with 0-1 lymphocytes, sugar- 80, and protein- 83. NCS showed prolonged latency with normal amplitude and conduction velocity suggestive of early GBS. The patient was electively intubated and started on immunoglobulin for 5 days. He showed good improvement and responded well and was discharged after the closure of the tracheostomy walking with support. Unusual features of GBS include papilledema, facial myokymia, hearing loss, meningeal signs, vocal cord paralysis, and mental status changes. There are several case reports of Miller-Fischer syndrome with botulism toxin ingestion presenting as descending paralysis. Conclusion: This report adds to the evolving spectrum of GBS, descending involvement extremely rare in children. Atypical presentations of AIDP as in this case may delay diagnosis. It is important to be aware of the multitude of variations of presentation of GBS in order to prevent delays in treatment.

Abstract ID: 832

Low Cobalamin Levels – An Underestimated Cause of Demetia - A Case Report

D. Subburaj

DM Neuro Pursuing, Other

E-mail: dsubbu26@gmail.com

Background and Aim: Vitamin B12 deficiency is widely known for its hematological and neurological manifestations. Neuropsychiatric manifestations of vitamin B12 deficiency include dementia, delirium, cerebellar ataxia, psychosis, neuropathy, and mood disorders . Dementia caused by vitamin B12 deficiency may not be accompanied by anemia and it may be the sole manifestation of cobalamin deficiency. Methodology: Case report A70-year-old woman , premorbidly normal, presented with rapidly progressive cognitive impairment over a period of 7 months in the form of short term and episodic memory disturbances ,decreased interaction, withdrawn behavior, apathy, way finding difficulties and sleep disturbances . Neurologic examination revealed a MMSE score of 9/30. Tests for attention and sustained attention were impaired. Speech and language examination revealed reduced word output and impaired animal naming. Both immediate and recent memory were impaired. Lobar examination revealed frontal and temporal involvement. The patient’s initial laboratory investigations indicated mildly decreased hemoglobin (10.2 g/dL) and normal MCV -98 fL. There were very low levels of vit B12 (116 pg/mL). MRI Brain showed age related cortical atrophy. Results: The patient was treated with parentral methycobalamin initially then switched to oral cobalamin .After a period of three months of treatment, MMSE was reassessed and was 16/30.She was fully oriented and had significant improvement in her memory and behaviour. Discussion: Patients presenting with neuropsychiatric symptoms due to vitamin B12 deficiency have a less severe presentation of megaloblastic anemia . Conclusion: The role of vitamin B12 deficiency as one of the few treatable causes of dementia, however, is still controversial. The purpose of this case report is to show that if vitamin B12 deficiency remains undiagnosed, serious sequelae may occur. Assessment of B12 levels should be included as a standard evaluation in patients presenting with dementia. Therefore, prevention, early detection, and management of this reversible state are of profound importance.

Abstract ID: 833

Multiple Spinal Level Cerebrospinal Fluid Leaks Presenting with Spontaneous Intracranial Hypotension

D. Srilekha

DM Neuro Pursuing, Other

E-mail: srilekhakartik@gmail.com

Background and Aim: Intracranial hypotension is an under recognised clinical condition and usually occurs as a result of spinal cerebrospinal fluid leakage, which may be iatrogenic, traumatic or spontaneous. Here we report a case with multiple level spinal leaks as a rare cause of intracranial hypotension. Methodology: A 35 year old female without any previous comorbidities presented with orthostatic headache of one and a half months duration. Her general and neurological examination was normal. Results: On investigating her routine blood panel, liver and renal functions were normal. MRI brain with contrast revealed features suggestive of intracranial hypotension like subdural hygroma in bilateral frontoparietotemporal and bilateral cerebellar hemisphere convexities, sagging of midbrain with reduced midbrain-pons angle of 32 degrees, tonsillar herniation and diffuse pachymeningeal thickening and contrast enhancement. Computed tomography cisternogram revealed dural leaks at multiple levels including cervical, thoracic and lumbar levels. Autologous epidural blood patch was done for this patient. Patient showed complete symptomatic improvement following lower thoracic and lumbar epidural blood patch. Repeat MRI done after three months showed partial resolution of features of spontaneous intracranial hypotension. Discussion: Spinal CSF leaks are often implicated as a cause of spontaneous intracranial hypotension, but they are rarely demonstrated radiologically. The choice of treatment depends on the severity of symptoms ranging from conservative management to surgical correction of the dural tear. Conclusion: Spontaneous CSF leaks at multiple spinal levels are less frequently demonstrated and so it needs to be carefully looked for while performing spinal imaging in patients with spontaneous intracranial hypotension. It is also important for clinicians to keep this diagnosis in mind and review treatment modalities used in patients and explore possible explanation for failure of initial therapy.