Friday, September 28, 2018, 09:00-10:00 hrs, E-Poster designated area: E Poster Session – 01: Miscellaneous Disorders

. 2018 Sep;21(Suppl 2):S72–S114.

Friday, September 28, 2018, 09:00-10:00 hrs, E-Poster designated area: E Poster Session – 01: Miscellaneous Disorders

PMCID: PMC6174656

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP1/51: Van der Knaap disease: A case report

Introduction: Megalencephalic leukoencephalopathy (MLC), also known as van der Knaap's disease, is a rare autosomal recessive degenerative disorder characterized by megalencephaly, leukoencephalopathy, deterioration of motor function with ataxia, spasticity, seizures, and delayed onset of slowly progressive neurological deterioration. Most of the cases reported in India belong to Agarwal community with consanguinity. Magnetic resonance imaging (MRI) is the investigation of choice and features include extensive symmetrical white matter changes with subcortical cysts in the anterior temporal region and frontoparietal subcortical area. We report this rare case of a 12-year-old boy belonging to this ethnic background presented with a history of delayed milestones, ataxia with recurrent falls, poor scholastic performance, and seizures.

Case Report: The patient is a 12-year-old male born from nonconsanguineous marriage in Agarwal community by normal vaginal delivery with a history of delayed milestones, intellectual disability, and recent-onset seizures. There is a history of progressive difficulty in maintaining balance while walking without any history of sensory, bladder, and bowel involvement and family history. On examination, the patient had macrocephaly (head circumference – 56 cm) (>97^th percentile), dysarthria, and spastic paraparesis with positive Babinski sign. Routine blood investigations along with creatine kinase and lactate levels were normal and TORCH screen was negative. MRI brain showed diffuse white-matter hyperintensities and subcortical cysts in anterior temporal region. The patient was treated symptomatically with well-controlled seizures and is under regular follow-up.

Conclusion: MLC should be included in the differentials of macrocephaly and leukoencephalopathy with characteristic MRI findings. Precise diagnosis helps clinician for better management of the child and to prognosticate its benign course to the parents.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP2/83: Etiological profile of noncompressive myelopathies in a tertiary care hospital in Central Tamil Nadu

Jayasankar VR ¹, Thangaraj M ¹, Balamurali K ¹

Materials and Methods: An observational study was carried out in Thanjavur Medical College. Patients of noncompressive myelopathies were segregated into two categories: degenerative and nondegenerative.

Results: The study had 46 patients with male:female ratio of 1.3:1. Presentation was acute in six patients (13%), subacute in 4 (8.6%), chronic in 31 (67.3%), and history of relapse and remission in 5 (10.8%) patients. Degenerative etiology was found for 25 (54.35%) and others were nondegenerative. There was an history of electrocution in two cases (6.66%). Cerebrospinal fluid study carried out in seven cases revealed rise of protein in 6 (1.3%) patients. Magnetic resonance imaging (MRI) study carried out showed signal changes in 32 cases (69.5%) which included myelomalacia, demyelination, atrophy of cord, infarction of cord, and in 14 cases (30.4%), no abnormality could be detected. Etiological diagnosis could be established in 44 (95.6%) cases such as amyotrophic lateral sclerosis in 11 (23.9%) cases; other degenerative myelopathies such as hereditary spastic paraparesis, spinocerebellar ataxias, Hirayama disease, and syrinx; nondegenerative etiologies such as acute transverse myelitis were seen in three cases (6.5%); demyelination was seen in six cases (20%); other causes included neuromyelitis optica spectrum disorder (3; 6.52%) and multiple sclerosis (3; 6.52%), vascular cause in one (2.17%); physical (electrocution) in two cases (2.42%), and nutritional causes in five (16.66%) cases (Vitamin B12 deficiency in 4 cases and copper deficiency in 1 case). In the remaining two cases (6.66%), no etiological factors could be found.

Conclusion: The underlying etiology was found in 95.6% patients of noncompressive myelopathy. Clinical features combined with MRI findings are helpful in defining the cause of noncompressive myelopathies.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP3/133: An interesting case of a child with spastic diplegia

Kumar S ¹, Velayutham S ¹, Jeyaraj M ¹, Sowmini PR ¹, Arunan S ¹

Background: Spastic diplegia is a neurological disorder characterized by spastic paraparesis with scissoring of gait. Cerebral palsy is the most common cause of spastic diplegia. Rarely, inborn errors of metabolism (IEM) such as arginase deficiency and urea cycle disorder can cause spastic diplegia. We report here a case of arginase deficiency presenting with spastic diplegia, regressive milestones, and stunted growth.

Case Report: A 13-year-old boy born out of 3^rd-degree consanguineous parentage presented with drowsiness for 2 days. Till 2 years of age, his developmental milestones were normal after which there was a regression. At 3 years of age, he developed generalized tonic clonic seizures and was on valproate 400 mg with good seizure control. His Glascow Coma Scale score was 9/15 (E2V2M5). Morphometry revealed stunted growth, underweight, and microcephaly. Lower limbs were spastic and hyperreflexic with heel and knee contractures and upgoing plantar. Biochemical investigations and magnetic resonance imaging brain were normal with electroencephalogram showing diffuse nonspecific electrophysiologic dysfunction. Valproate-induced hyperammonemic encephalopathy was considered and was replaced by clobazam. Sensorium improved and he became alert. Progressive nature of the deficit and hyperammonemia with low-dose valproate created a suspicion of IEM. Tandem mass spectrometry revealed high arginine levels and red blood cell arginase activity was low. Considering arginase deficiency, we started him on low-protein diet and he showed improvement in language skills.

Conclusion: Arginase deficiency presents in the 1^st or 2^nd decade with a unique clinical feature of progressive spastic diplegia. Low-protein diet with inclusion of essential amino acids forms the mainstay of treatment.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP4/162: Traumatic carotid-cavernous fistulas: Can we wait?

Nagendra S ¹, Soni G ¹, Gutte A ¹, Jagiasi K ¹, Patil S ¹

Carotid-cavernous fistula (CCF) is an abnormal communication between the cavernous part of internal carotid artery (ICA) or one of its branches or external carotid artery and the cavernous sinus. It is classified etiologically into posttraumatic or spontaneous CCF. Traumatic CCF (TCCF) is a direct communication between cavernous portion of the ICA and cavernous sinus due to tear in ICA; it accounts for 75% of all the cases of CCFs. Apart from ophthalmoplegia and vision impairment, these high-flow TCCFs may result occasionally in life-threatening intracranial hemorrhage or epistaxis. Most of the cases need endovascular embolization; spontaneous resolution of high-flow, direct TCCFs is extremely rare (<6% and usually with indirect CCF). We report two cases of high-flow, direct TCCF (Barrow type A) that resolved spontaneously and confirmed by digital subtraction angiography.

MiEP5: Not found

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP6/217: A case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids with spinal cord presentation

Aglave V ¹, Faldu H ¹, Ojha P ¹, Jagiasi K ¹, Soni G ¹

Background: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is an inflammatory condition primarily involving the pons and adjacent structures and presents with cerebellar and brainstem signs such as dysarthria, ataxia, cranial nerve palsies, and long tract signs. It has characteristic magnetic resonance imaging (MRI) findings and clinical and radiologic responsiveness to steroids. The pathogenesis is unknown and it is a diagnosis of exclusion.

Case Report: A 53-year-old male patient presented with 1-month history of progressive imbalance while walking requiring support to stand and walk. He had numbness and paresthesia below mid-trunk level with urinary urgency and constipation. However, there were no signs or symptoms referable to the brainstem.

Results: MRI spine with brain revealed characteristic gadolinium-enhancing punctate lesions without any mass effect in brainstem and cerebellum as well as the cervicothoracic cord and cerebral white matter. Autoimmune, malignancy, and infection workup were negative. Cerebrospinal fluid analysis revealed 30 cells/mm³, 92% lymphocytes along with protein 90 mg%. Positron emission tomography-computed tomography of the whole body revealed hypometabolism in the brainstem and cerebellum. The patient was started on intravenous methylprednisolone 1 g/day for 5 days followed by prednisolone 60 mg/day. He showed significant improvement and follow-up MRI scan at 2 months revealed clearing of all lesions. Secondary immunosuppression with methotrexate was added later.

Conclusions: Typically, chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids patients present with symptoms and signs referable to the brainstem and cerebellum; however, here, we present a case with predominant spinal cord presentation. Our patient was extensively worked up for alternate causes and showed typical clinical and radiologic responsiveness to steroids.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP7/241: Intracranial plasmacytoma associated with multiple myeloma presenting as multiple cranial nerve palsies: A case report

Mohan CK ¹, Perumal SS ¹, Velayutham S ¹, Jeyaraj M ¹, Mohan A ¹

Background: Central nervous system involvement in multiple myeloma is rare (1%). Few cases present as intracranial plasmacytoma. Isolated cranial neuropathies had been reported in solitary clival plasmacytoma. Multiple cranial neuropathies had been reported in late stages of multiple myeloma. Intracranial plasmacytoma associated with multiple myeloma presenting as multiple cranial nerve palsies is a rarity. We report a case of multiple cranial nerve palsies as the first presentation of intracranial plasmacytoma.

Case Report: We had a 44-year-old female presenting with left hemicranial throbbing headache for 6 months. Since 3 weeks, she developed dysphagia, nasal regurgitation, and hoarseness of voice, deafness, tinnitus, and diplopia. On examination, the patient had lower motor neuron type of left 6^th, 7^th, 8^th, 9^th, 10^th, 11^th, and 12^th cranial nerve palsies. Initial investigations showed bicytopenia (hemoglobin 8.3 g/dl, platelet count: 45,000), white blood cell count was normal. Erythrocyte sedimentation rate was 68 mm/h. Uric acid levels were high (7.1 mg/dl). Magnetic resonance imaging brain showed soft-tissue component with enhancement on contrast in clivus and left petrous apex pushing encroaching foramen ovale and crossing the midline extending to the right side. Urine Bence Jones proteins were positive. Serum protein electrophoresis showed M spike in gamma globulin region (4.66 g/dl). Serum light chain assay showed increased free kappa levels with free light chain ratio of 26.2. Bone marrow biopsy showed clusters of plasma cells and immature forms with immunohistochemistry positive for CD138+. Hence, the patient was started on bortezomib, thalidomide, and prednisolone regimen.

Conclusion: We present this case of a female with intracranial plasmacytoma associated with multiple myeloma, presenting as multiple cranial nerve palsies, which is a rarity.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP8/257: A rare cause of Parkinsonism-manganese toxicity

Yadav PK ¹, Roy P ¹

A 43-year-old male presented with 6-month history of gradual-onset and progressive bradykinesia, tremors of limbs, and imbalance while walking. He also had slurring of speech with hypophonia. Neurological examination revealed slow broken saccades, rigidity with cogwheeling, bradykinesia and short shuffling, and festinant gait. He had action and postural tremors of upper limbs. Pull test was positive. Thus, a diagnosis of young-onset Parkinsonism was considered. Occupational history revealed that he was working in blast furnace of a steel plant for the past 14 years. He was evaluated with magnetic resonance imaging brain which showed symmetrical hyperintensities involving basal ganglia and subcortical white matter in T2-weighted images. T1-weighted images showed evidence of basal ganglia hyperintensities probably related to mineral deposition. Kayser–Fleischer rings, serum ceruloplasmin, liver function tests, and ultrasound abdomen were normal. Peripheral smear did not show any acanthocytes. Renal function and thyroid function tests were normal. Antithyroid antibodies were normal. Cerebrospinal fluid was normal. In view of occupational exposure and clinical features, a possibility of manganese toxicity was strongly considered. Serum manganese was done which was elevated (twice of upper limit of normal). The patient was started on symptomatic medications such as levodopa and trihexyphenidyl. He had stopped working which eliminated occupational exposure to manganese. His Parkinsonism gradually started to improve over 3-month period of follow-up. Plan is to repeat the serum manganese level after 3 months.

Conclusion: Occupational history of manganese exposure is very important in cases of Parkinsonism. Early removal from the work environment can lead to improvement of symptoms and prevent permanent neurological deficits.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP9/259: Synaptic nuclear envelope protein 1 ataxia: The first genetically confirmed case from India and a novel homozygous mutation in the SYNE 1 gene

Kothari K ¹, Chakor R ¹, Nadaf S ¹, Patil A ¹, Bharote H ¹

Synaptic nuclear envelope protein 1 (SYNE1) ataxia is a slowly progressive autosomal recessive cerebellar ataxia first described in Quebec, Canada, and subsequently in European countries. In Asian countries, SYNE 1 ataxia is described in 1 Japanese family. We report the case of a 24-year-old female who presented with a 10-year history of slowly progressive cerebellar ataxia with anterior horn cell (AHC) involvement. On examination, she had pan cerebellar involvement, bulbar, as well as limb AHC involvement (fasciculations and wasting) as well as pyramidal dysfunction. Her younger sibling had a similar clinical phenotype. Next-generation sequencing revealed a novel hitherto unknown homozygous pathogenic variant in exon 125 of SYNE1 gene. It was responsible for frameshift mutation resulting in a truncated protein with loss of function. SYNE 1 is one of the largest genes in the human genome, with the longest isoform comprising 146 exons that encode the 8797 aminoacid synaptic nuclear envelope protein 1. This first genetically confirmed case report from India suggests that this unique phenotype should alert clinicians to consider SYNE 1 mutation in this part of the world.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP10/274: Sickle-cell anemia in association with Moyamoya syndrome: Revisited

Dixit NS ¹, Sharma S ¹, Sharma P ¹

Background: Moyamoya syndrome (MMS) is a chronic progressive cerebrovascular occlusive disease of the bilateral internal carotid arteries that leads to compensatory abnormal vascular network at the base of brain. MMS occurs in a wide range of disorders including hemoglobinopathies such as sickle-cell anemia. MMS is a rare and potentially fatal/devastating outcome of sickle-cell disease (SCD).

Case Reports: We present two cases of MMS associated with SCD.

Case 1: A 7-year-old girl presented with painless decline in vision for few days. She was diagnosed to have SCD at about 2 years of age. She suffered two episodes of stroke with right hemiparesis in the past 2 years. She also had seizures. Magnetic resonance imaging (MRI) brain showed acute right posterior cerebral artery (PCA) infarct, chronic infarcts in left PCA and middle cerebral artery (MCA) with multiple flow voids suggestive of Moyamoya phenomenon.

Case 2: A 14-year-old boy presented with right-sided headache and blurred vision for 4 days. He was diagnosed to have sickle-cell anemia at 2 years of age. He had icterus, palpable liver, ejection systolic murmur, and right homonymous hemianopia. MRI brain showed acute right PCA infarct and magnetic resonance angiography was suggestive of Moyamoya phenomenon. Transcranial Doppler showed right MCA-peak systolic velocity (PSV) 156, end-diastolic velocity (EDV) 38.6, and mitral valve (MV) 75.5; left MCA-PSV 207, EDV 49.5, and MV 98.9.

Conclusion: Sickle-cell disease patients are at risk for recurrent stroke. MMS can be diagnosed with appropriate history, physical examination, and MR/MRA brain.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP11/296: Headache and neurological deficits with cerebrospinal fluid lymphocytosis: Case series

Patil S ¹, Ojha P ¹, Soni G ¹, Barvalia P ¹, Aglave V ¹

The syndrome of headache with neurologic deficits and cerebrospinal fluid lymphocytosis (HaNDL) is a self-limited and rare entity which is a diagnosis of exclusion. We present four cases of HaNDL with different clinical presentations.

Patient 1: A 61-year-old male presented with meningoencephalitis-like presentation with normal imaging and cerebrospinal fluid (CSF).

Patient 2: A 53-year-old male presented with a severe headache with agitation and delirium. His imaging, CSF profile, and detailed blood workup were negative.

Patient 3: A 35-year-old male with rheumatic heart disease presented with severe headache and slurring of speech. The imaging did not show evidence of acute stroke and angiography was normal.

Patient 4: A young male patient presented with an acute-onset severe headache which fulfilled the diagnostic criteria for migraine. All the patients improved completely with sodium valproate. HaNDL mimics infectious causes, but it is transient and no infectious cause can be demonstrated. This clinical syndrome is underrecognized. Increased awareness with early diagnosis of HaNDL can help predict the prognosis and unmerited investigations can be limited.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP12/322: Rare late-onset presentation of glutaric aciduria type I

Reddy PV ¹, Kannan V ¹, Chandramouleeswaran V ¹, Narasimhan LR ¹

Background and Purpose: Glutaric aciduria type I (GA type I) is a recessive disorder that results from a mutation on chromosome 19 due to deficiency of mitochondrial matrix enzyme glutaryl-CoA dehydrogenase, leading to accumulation of glutaric acid (GA) and 3-hydroxyglutaric acid. There are two clinical presentations, both occurring early in infancy or in the neonatal period. The most common is progressive encephalopathy beginning in the first 3–4 months and presents as psychomotor retardation, cerebellar ataxia, extrapyramidal symptoms, macrocephaly, and seizures. These symptoms, once started, can rapidly progress. The other presentation consists of an acute encephalopathic crisis that mimics hypoxic or ischemic encephalopathy, especially in the neonatal period. Patients usually present with seizures, high anion gap metabolic acidosis, and hypoketotic hypoglycemia. It is rapidly progressive and may lead to death in the acute phase if undiagnosed. The characteristic magnetic resonance imaging (MRI) findings include T2 hyperintensities of the basal ganglia, dilated sylvian fissures with open opercula (bat wing sign), widespread restriction in the white matter in diffusion-weighted MRI. The diagnosis can be confirmed by elevated urinary 3-hydroxyglutaric acid in urine and tandem mass spectrometry.

Case Description: We report a rare late-onset presentation of 30-year-old male, presenting with acute onset of delirium of 2-day duration, no localizing signs, MRI showing T2 fluid-attenuated inversion recovery hyperintensities in bilateral basal ganglia, dilated sylvian fissures – bat wing appearance, normal metabolic parameters, urine was negative for organic acids and tandem mass spectrometry was suggestive of GA type 1.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP13/329: Autosomal recessive spastic ataxia of Charlevoix–Saguenay: A report of a novel variant from South India

Murali S ¹, Kumar PS ¹, Netto A ¹, Nagaraj K ¹, Janardhan DC ¹, Abhinandan KS ¹

Autosomal recessive spastic ataxia of Charlevoix–Saguenay (ARSACS) is a progressive neurological disorder reported from the Saguenay and Charlevoix regions of Quebec, Canada, but is rarely reported from other parts of the world, including India. We report the case of a 32-year-old female, born to asymptomatic nonconsanguineous parents, who manifested with progressive ataxia and dysarthria of 10 years’ duration. She was earlier evaluated at other centers, for many years as Friedrich's phenotype and hereditary motor sensory neuropathy (HMSN). Her systemic examination, ocular movements, and optic fundi were normal. Routine laboratory test results including metabolic workup were normal. Her magnetic resonance imaging showed T2/fluid-attenuated inversion recovery transverse pontine hypointensities with superior vermian atrophy and cervical cord atrophy. Her optical coherence tomography showed thickening of the nerve fiber layer of the retina. A diagnosis of ARSACS was considered. A targeted sequencing of the SACS gene revealed a novel, homozygous, pathogenic variant (c.12529dupT) in the poly A region of exon-10 on the SACS gene on chr13q11 which leads to a loss of function. This report is shared to impress upon the need for identifying novel variants for this relatively rare disease, which in Indian context may have a relatively benign course.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP14/330: An unusual case of mitochondrial myopathy due to complex 1 deficiency with acute exacerbations responding to intravenous immunoglobulin: Case report

Awatare P ¹, Murgod U ¹

Mitochondrial myopathies are heterogeneous group of disorders due to defect in enzymes of respiratory chain. The most frequently encountered one is complex I deficiency. Mitochondrial myopathy can occur in a wide range of age groups. It has wide clinical spectrum including Leigh's disease, cardiomyopathy, fatal infantile lactic acidosis, hepatopathy, and myopathy. Isolated COX deficiency associated with a relatively mild clinical phenotype comprising myopathy, demyelinating neuropathy, premature ovarian failure, short stature, hearing loss, pigmentary maculopathy, and renal tubular dysfunction. While adult-onset patients have clinical features mainly ataxia, seizure, deafness, dementia, and peripheral neuropathy. There is no definite treatment for mitochondrial myopathy. We report a case of 38-year-old female patient of mitochondrial myopathy who had chronic proximal weakness with exercise intolerance from the age of around 16 years. She had presented twice with acute exacerbation of weakness leading to respiratory compromise. Both time, she responded to intravenous (IV) Ig improving her back to baseline status. There have been very few case reports of adult-onset Leigh syndrome responding to immunotherapy. This is a rare and unusual case of adult-onset mitochondrial myopathy which responded to IV Ig. This suggests adult-onset mitochondrial myopathy which may have an underlying autoimmune mechanism amenable to immunotherapy. Further research on the role of the inflammation/immune pathway activation is needed in order to explore the role of immunotherapy in these conditions.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MiEP15/335: Hemiparkinsonism hemiatrophy: An interesting case for the discerning eye

Chakravarty K ¹, Ray S ¹, Pattanayak SV ¹, Baliani N ¹, Takkar A ¹, Mehta S ¹, Lal V ¹

Hemiparkinsonism hemiatrophy (HPHA) syndrome is a very rare entity characterized by early-onset unilateral parkinsonism, hemiatrophy of the affected side, and accompanying dystonia. We report a case of 50-year-old female presenting with gradual-onset, progressive rigidity, and dystonia of left half of the body for the last 4 years without any cognitive decline, apraxia, alien hand phenomenon, or myoclonus. There was no past history of any perinatal insult or strokes. Clinical examination revealed a normal higher mental function without any cranial nerve involvement. There was hemiatrophy on the left including the face. There was cogwheel rigidity and action dystonia on the left upper and lower limbs. Magnetic resonance imaging brain revealed atrophy of the contralateral mid brain and internal capsule with preserved cortical structures. Positron emission tomography findings revealed asymmetrical reduction of tracer uptake suggestive of unilateral presynaptic dopaminergic hypofunction. She showed only partial response to levodopa.

Conclusion: The diagnosis of HPHA is primarily clinical and requires a high level of suspicion. It should be considered an important differential in all cases presenting with unilateral dystonia and Parkinsonism because of its poor response rates to dopamine agonists.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

Friday, September 28, 2018, 13:00-14:00, E-Poster designated area: E Poster Session – 02: Miscellaneous Disorders: SEP1/19: Sex differences in risk factor profile, stroke subtype, and outcome in acute ischemic stroke

Mitta N ¹, Sylaja PN ¹, Sreedharan SE ¹, Sarma PS ¹

Background: There is increasing evidence to suggest that sex differences exist in the risk factor profile, treatment, and outcome of stroke.

Aim: We analyzed the sex difference of ischemic stroke in India.

Methods: We included 742 patients within 1 week of onset of first ever ischemic stroke during 2014–2017. The demographic, clinical details, and risk factors were documented. The stroke severity was assessed using the National Institute of Health Stroke Scale (NIHSS) at onset. The stroke subtype classification was done using Trial of Org 10172 in Acute Ischemic Stroke criteria. The 3-month functional outcome was assessed using modified Rankin scale (mRS) and excellent outcome was defined as a mRS ≤2.

Results: Of 742 patients, 250 (33.7%) were females. The mean age, clinical presentation, and rate of thrombolysis did not differ between men and women. Women suffered more severe strokes compared to men (mean NIHSS 9.5 vs. 8.4, P = 0.03). The etiological stroke classification showed large artery atherosclerosis was more common in men (21.3% vs. 14.8%, P = 0.03) and cardioembolic strokes were more common in women (27.2% vs. 19.7%, P = 0.02). Men had a better 3-month functional outcome compared to women (68.6% vs. 61.2%, P = 0.04).

Conclusion: Our data provide insights about the sex differences of stroke in India. Identification of these differences has implications in planning treatment and prevention strategies.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP2/52: Cerebral venous sinus thrombosis in scrub typhus infection: A rare presentation

Shringi P ¹, Sardana V ¹

Introduction: Scrub typhus is an acute febrile infectious illness that is caused by rickettsia species Orientia tsutsugamushi. Scrub typhus has diverse clinical manifestations ranging from a nonspecific febrile illness to severe multiorgan dysfunction. The spectrum of neurological complications in scrub typhus includes aseptic meningitis, meningoencephalitis, cerebellitis, myelitis, cerebral hemorrhage, and cerebral infarction. This report describes a case of scrub typhus with cerebral venous sinus thrombosis (CVT), an unreported complication of scrub typhus to the best of our knowledge.

Case Details: A 17-year-old young male was admitted in neurology department with throbbing headache, high-grade fever, and periorbital pain for 15 days without a history of vision difficulty. On neurological examination, he was conscious with normal fundus examination, no meningeal signs on neurological examination. Hematological analysis shows increased leukocyte counts. Liver function tests and renal function tests were normal. IgM antibody was positive for scrub typhus by immunochromatographic method. Magnetic resonance (MR) venogram brain showed acute thrombus in superior sagittal sinus, T2 and fluid-attenuated inversion recovery images of MR imaging (MRI) brain showed white-matter edema in bilateral high frontoparietal region. The patient was managed with doxycycline and azithromycin along with subcutaneous low-–molecular-weight heparin. Later he was switched to nicoumalone and targeted international normalized ratio was achieved. During treatment, the patient improved his headache significantly reduced along with fever.

Conclusion: This case highlights the significance of rare presentation/association of scrub typhus with CVT. Early search and prompt management of scrub typhus and CVT may be rewarding, since scrub typhus is a treatable disease and so is CVT.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP3/56: Clinical profile and risk factors of massive middle cerebral artery infarction at a tertiary care center in Central Tamil Nadu

Palliyali S ¹, Thangaraj M ¹, Elangovan S ¹

Aims and Objectives: The aim and objective was to study the clinical profile and risk factors of massive middle cerebral artery (MCA) infarction and to study the relation of alcoholism and its pattern to massive MCA stroke.

Materials and Methods: Patients admitted with massive MCA infarct with an early ischemic change on computed tomography affecting at least >50% of the MCA territory within 48 h of symptom onset were included in the study. Stroke mimics and those with a history of previous stroke were excluded from the study. Study design was a cross-sectional, observational study.

Results: A total of sixty cases were analyzed. Mean age was 55.4 years. Nearly 35% of patients had National Institute of Health Score Scale (NIHSS) >20. Diabetes was present in 18 patients. Nearly 60% were alcoholics, and 83% of males were alcoholics. Thirty-one patients were heavy drinkers. Binge drinking was present in 30% of patients. The mean duration of alcohol was 9.5 years. Average intake per day was 120 ml. Mean Alberta Stroke Program Early CT Score (ASPECT) was 3. Twenty-five patients had a modified Rankin score of 4 at discharge. Craniotomy was done in six cases. The total number of deaths was 17 (29%). Aspiration pneumonia was the common cause of death in 5 patients.

Conclusion: More number of adverse outcomes were in age >50 years (14 deaths). Mean NIHSS was 18. NIHSS 20 or more was associated with adverse outcome (P < 0.05). Nearly 60% were alcoholics. Binge intake was present in 30% of patients (associated with adverse outcome P < 0.05). Non high-density lipoprotein >130 was the significant lipid abnormality observed. Mean ASPECT score was 3 (3 or less was associated with adverse outcome P < 0.05).

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP4/80: Factors associated with hospital arrival time of acute stroke patients in Emergency Department of All India Institute of Medical Sciences, New Delhi: A cross-sectional study

Misra S ¹, Talwar P ¹, Kumar P ¹, Rai A ¹, Kumar A ¹, Aggarwal P ¹, Mohania D ¹, Pandit AK ¹, Prasad K ¹, Vibha D ¹

Background: Acute ischemic stroke (IS) is an emergency, and time-sensitive treatment decisions are often hampered by the delays in the presentation of patients to the hospital.

Objective: The objective of this study was to investigate the factors associated with early hospital arrival time of acute IS patients.

Methods: From July 4, 2017 to April 27, 2018, consecutive IS patients admitted to the Emergency Department of All India Institute of Medical Sciences, New Delhi, India, were recruited in this ongoing cross-sectional study. Patient information was registered using a structured questionnaire including time-to-hospital, neurological symptoms at onset, previous history of stroke, season at attack, and demographic data.

Results: A total of 128 IS cases have been recruited in the study till date with a mean age of 52.94 ± 16.73 years. There were 86 males and 42 females in the study. The median time-to-hospital arrival was 5.37 h (interquartile range: 2–18.5). A total of fifty (39.68%) patients presented to the hospital within 4 h. Only 20.31% of patients received thrombolytic therapy and only 15.45% of patients used ambulance as their preferred mode of transportation. Patients with a high National Institutes of Health Stroke Scale score >15 at admission, unconsciousness at onset, and higher education status had significantly less time-to-hospital arrival (within 4 h).

Conclusion: Our study found considerable delay in the arrival of stroke patients to the emergency department. Adequate measures need to be taken to increase the stroke awareness along with more use of ambulance services for early treatment and management of stroke.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP5/82: To study the occurrence of unrecognized diabetes mellitus and prediabetes in patients of acute ischemic stroke

Rani P ¹, Chaudhry N ¹, Puri V ¹

Objective: The objective was to study the occurrence of unrecognized diabetes mellitus and prediabetes in patients of acute ischemic stroke.

Methods: A total of 75 patients of acute ischemic stroke with no previous diagnosis of diabetes were included in the study. In all patients, insulin levels and HbA1C were measured during hospital stay, along with daily fasting plasma glucose. The degree of insulin resistance was measured by the Homeostasis Model Insulin Resistance Index (HOMA). Patients were categorized into three groups: normoglycemics, prediabetics, and diabetics. All investigations were repeated at the end of 3 months to assess any change in their glycemic status.

Results: Out of 75 patients, 31 (41.3%) were normoglycemic and 44 (58.6%) had hyperglycemia. Of these 44 hyperglycemics, 7 (9.3%) were diabetic and 37 (49.3%) were prediabetes. Sixteen patients were lost to follow-up and so reevaluation at 3 months was performed in 59 patients. Out of these 59 patients, 26 (44.0%) were normoglycemic, 27 (45.7%) were prediabetics, and 6 (10.1%) were diabetic initially. At 3 months, all normoglycemics continued to remain so. Out of 27 prediabetics, 4 developed diabetes, while another 6 patients became normoglycemic. HOMA at initial evaluation was 1.66 + 1.11 in normoglycemics, 3.56 + 3.04 in prediabetics, and 11.7 + 8.98 in diabetics.

Conclusion: The reactionary hyperglycemia in stroke is found only in a few patients in our study, while majority have an abnormal glucose metabolism. It can be identified using simple tests and should be done during early course of stroke for a more effective and aggressive stroke prevention.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP6/212: Noninvasive measurement of vascular flow changes in ischemic strokes using transcranial Doppler

Masilamani S ¹, Jawahar M ¹, Tushar VP ¹, Manickavasagam J ¹, Narasimhan LR ¹

Background: Transcranial Doppler (TCD) ultrasound is a noninvasive, safe, and cost-effective bedside test for evaluating cerebrovascular circulation.

Aim: The aim was to study the role of TCD in the study of vascular flow changes in ischemic strokes.

Methodology: Forty-two patients admitted with ischemic stroke in the neurology department were included in the study. Parameters assessed using TCD include mean flow velocity (MFV) and Pulsatility index (PI). The primary sign of stenosis is focal increase in MFV at the site of luminal narrowing. Secondary TCD signs of stenosis include decreased velocity and increased PI in upstream vessels distal to the occlusion.

Results: Of the 42 patients studied, 34 were males and 8 were females. Decrease in MFV in vasculature distal to occlusion was seen in 34 patients accounting for 75% of abnormality (P < 0.05). Increase in PI in distal vasculature was seen in 24 patients accounting for 44% (P < 0.05) abnormality. In internal carotid artery and middle coronary artery (MCA) occlusion, the vasculature most commonly involved is ipsilateral MCA followed by ipsilateral anterior cerebral artery. Among the posterior circulation stroke, the MFV and PI were not correlative to clinical picture. Among the 42 stroke patients, TCD was normal in 11 patients. Among the anterior circulation strokes, MFV and PI measurements were found to be statistically significant in ischemic strokes.

Conclusion: TCD is a noninvasive, portable, nonionizing examination of intracranial arteries for the detection and quantification of flow changes in ischemic stroke. It also estimates the prognosis and recovery with collateral flow and velocity changes.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP7/216: Finasteride-induced cerebral venous sinus thrombosis: Rare drug to cause cerebral venous thrombosis used for alopecia

Prajapati J ¹, Machhavada K ¹, Parmar M ¹, Sharma A ¹

Introduction: Cerebral venous thrombosis (CVT) is common in young age group. Common causes are dehydration, prothrombotic state, drugs, and malignancy. Drugs such as oral contraceptive, corticosteroids, thalidomide, and tamoxifen are the common causes of cerebral venous sinus thrombosis. Here, we present a case of finasteride (5-α-reductase inhibitor)-induced CVT, rare drug to cause CVT.

Case Discussion: A 30-year-old male patient presented with new onset of acute severe headache with multiple episodes of vomiting and intermittent blurring of vision. On neurological examination, the patient had papilledema without any focal neurological deficit. Magnetic resonance imaging brain with contrast venogram was suggestive of right transverse, sigmoid, and jugular sinus thrombosis. His pro-coagulant workup including serum homocysteine, antiphospholipid antibody, anticardiolipin antibody, lupus anticoagulant, protein C, protein S, factor 5 Leiden mutations, and MTHFR mutation were normal. The patient had a history of male pattern baldness for which he was treated with finasteride. Finasteride is a 5-α-reductase inhibitor used for benign prostatic hyperplasia, androgenic alopecia, and female hirsutism. Finasteride has the common side effect of erectile dysfunction, decreased libido, breast enlargement, and ejaculation disorder. On reviewing literature, finasteride found to be a risk factor for CVT. The patient was treated with standard anticoagulation and improved. It was assumed to increases in estrone and estradiol levels induced by finasteride lead to thrombosis development.

Conclusion: Even though CVT is common in country like India, we should take detail drug history to rule out any rare possible drug as a cause of CVT.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP8/218: Homocysteine and serum B12 level in cortical venous thrombosis

Sharma S ¹, Kamran K ¹, Koka RP ¹, Sharma P ¹

Background: There is increasing evidence that mild hyper-homocysteinemia (Hcy) is a risk factor for venous thrombosis. Epidemiological studies have suggested that hyper-Hcy is associated with atherosclerotic arterial disease and venous thromboembolism. Relation with cortical venous thrombosis is less clear. Genetic and nutritional factors are important determinants of homocysteine metabolism. Blood levels of folate, Vitamin B12, and to a lesser extent Vitamin B6 are related inversely to homocysteine, anyone with a nutritional deficiency of these vitamins is at increased risk of hyper-Hcy.

Materials and Methods: We studied consecutive 38 patients of cortical venous thrombosis between June 2017 and June 2018 for fasting serum homocysteine level and serum B12 level. Hyper-Hcy is further classified into mild 15–30 nmol/mL, moderate 30–100 nmol/mL, and severe >100 nmol/mL. B12 levels are classified into normal range (190–900 pg/ml), mild deficiency (100–189 pg/ml), severe deficiency (900 pg/ml).

Results: The study included 26 males and 12 females with age ranging from 21 to 75 years (average 36.78 years). Nearly 73.68% (28/38) of patients were young between 24 and 40 years of age. The mean homocysteine level was 26.534 (normal = 12, mild = 10, moderate = 16, and severe = 0) and serum B12 level was 992.594 (normal = 13, mild deficiency = 9, severe deficiency = 1, and high levels = 15).

Conclusions: Cortical venous thrombosis in the present study is associated with mild-to-moderate hyper-Hcy.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP9/225: A study on poststroke depression at a tertiary care hospital in Eastern Tamil Nadu

Kumar A ¹, Balamurali K ¹

Introduction: Stroke is significantly associated with a high rate of mortality and morbidity. According to an estimate, about one-third of the poststroke patients suffer from substantial depressive symptoms. The WHO has predicted cerebrovascular disease as the second highest cause of disability worldwide by 2020. Different studies on the prevalence of poststroke depression (PSD) report different frequencies and results vary considerably across studies. The prevalence as reported in different studies ranges from 9% to 60%. Study of PSD is of vital importance to avoid long-term adverse consequences of PSD.

Materials and Methods: Totally 52 patients were selected to serve as sample in this study who fulfilled the inclusion criteria. This study was carried out at the Neurology Outpatient Department of Thanjavur Medical College Hospital, Thanjavur, from February 1 to June 30, 2018, with the help of neurologist and psychiatrist. The consent of patients to participate in study was sought and they were also assured about confidentiality of the information.

Conclusion: Depression is a common psychiatric disorder after stroke. The occurrence of PSD observed in this study more than 6 months after stroke was 60%. Demographic variables are not significantly associated with PSD. Patients having ischemic stroke are at higher risk of developing depression after stroke. There is a significant association was found between PSD and left middle cerebral artery infarction. These findings highlight the necessity to investigate, diagnose, and treat PSD, which is a risk factor for morbidity and mortality of patients after stroke.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP10/226: Thrombolysis for stroke at a tertiary care hospital in Hyderabad

Surisetti BK ¹, Narisetti V ¹, Kamera SK ¹

Ischemic strokes account for 80% of total stroke events. The prevalence rate of stroke in India ranges from 2.24 to 4.22 per 1000. The 30-day case fatality for stroke ranges from 25% to 40% in India. Early treatment of stroke (for patients who presented within window period [4.5 h]) with intravenous (IV) thrombolysis (rtPA or tenecteplase) may prevent long-term neurological disability.

Objective: The objective was to study the outcomes of IV thrombolysis.

Methodology: All patients who came to the emergency department within window period with signs and symptoms suggestive of acute ischemic stroke were included in the study. Plain computed tomography (CT) brain was done to rule out intracranial hypertension. Baseline glucose and blood pressure were recorded. National Institute of Health Stroke Scale (NIHSS) score was calculated. A patient whose score was between 5 and 25 was given IV thrombolysis with rTPA or tenecteplase. Drug was given according to availability and it was free of cost.

Results: A total of fifty patients were thrombolysed. Sixteen were females and 34 were males. Door-to-needle time ranged from 30 to 75 min. NIHSS was calculated at baseline, after 2 h, and after 7 days. NIHSS at admission ranged from 5 to 22 mean 12.80. NIHSS at discharge 3-16 mean 9.46. Difference in NIHSS 3.34 points. Modified Rankin score (mRS) was calculated at discharge and after 3 months. Mortality was in four patients. Significant improvement in mRS score was seen in twenty patients.

Conclusion: Thrombolysis provides significant improvement for acute ischemic stroke in terms of improved disability and early functional recovery. There was no difference in outcome between two drugs.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP11/242: Clinical and laboratory profile of patients with cerebral venous sinus thrombosis related to alcohol intake at a tertiary care center in South India: A retrospective study

Prashob PS ¹, Sarala G ¹, Ranganathan LN ¹, Nithyanandam A ¹

Introduction: The association of alcoholism with stroke is known. However, acute cerebral venous sinus thrombosis due to alcoholism is on the rise. It is important to keep the possibility of CVT in mind while dealing patients with chronic alcohol intake presenting with neurological symptoms. This study was done to assess the clinical, laboratory, and radiological profile and assessment of associated risk factors in patients with CVT related to alcohol intake.

Methods: All cases of radiologically proven CVT patients with alcohol dependence or symptoms following a binge drink, admitted in the time period of 6 months (January–June 2018), were included. Their clinical and laboratory profile and radiological investigations were assessed.

Results: Thirty patients fulfilling the above criteria were studied. All of them were males. The mean age of the patients was 35±4.6 years. The common symptom was headache (86.7%) followed by seizures (56.6%). Among the patients, 43.33% (13) were alcohol dependent by CAGE questionnaire. Nearly 56.66% (17) of the patients had alcohol binge prior to the symptom onset. Computed tomography brain was normal in 33.3% (10) and showed hemorrhage in 46.66% (14).Transverse sinus was the most common sinus involved (60%) followed by superior sagittal sinus (28%). Vitamin B12 deficiency in 56.6% (17), hyperhomocysteinemia in 63.3% (19), and polycythemia in 10% (3) were also seen in these patients.

Conclusion: CVT is common in alcohol-dependent patients with binge drinking of alcohol. Headache was the most common symptom. Binge alcohol drinking was identified as a risk factor for CVT. Vitamin B12 deficiency and hyperhomocysteinemia associated with chronic alcohol intake were also found to be risk factors for CVT.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP12/243: Clinical profile of cavernous sinus syndrome at a tertiary care center in Eastern India

Rodge V ¹, Das SK ¹

Introduction: Cavernous sinus syndrome (CSS) is defined as involvement of at least two of the third, fourth, fifth (V1, V2), sixth cranial nerve (CNs), or involvement of only 1 of them in combination with a radiologically confirmed cavernous sinus lesion. Cavernous sinus pathology can be divided into vascular (aneurysm, fistulas, and thrombosis), tumors, and inflammatory disorders.

Objective: To evaluate clinical features of CSS and to analyze the etiology of CSS.

Materials and Methods: Patients admitted to Neuromedicine department between March 2016 and October 2017 with features of CSS were evaluated excluding the patient.

Results: This is an ongoing study: out of thirty patients, twenty were female and ten were male. The right side was involved in 17 patients; the left side in 11, and 2 patients had bilateral involvement. Two patients had recurrent CSS. The most common presentation was headache followed by ptosis and diplopia. Out of the thirty patients, most commonly involved CN was CN III. Etiologically, four were fungal, three were tubercular, one was vascular, and in 22 cases no definitive cause was found (idiopathic granulomatous).

Conclusion: In our study, females were affected more than males and right side involvement was more common. The most common symptom is headache and most common CN affected is CN III followed by CN IV. Etiologically inflammatory pathology constituted major chunk.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP13/244: Occurrence and pattern of intracranial atherosclerotic disease in acute ischemic stroke and its impact on outcome: An observational study at a tertiary care center

Sharma P ¹

Introduction: Intracranial atherosclerosis (IA) is believed to be commoner than extracranial atherosclerosis (EA) in Indian/Asian acute ischemic stroke (AIS) patients. Prospective Indian data are sparse.

Aim: The aim was to study the occurrence, pattern, and outcome of AIS with IA.

Methods: AIS patients (age ≥20 years) underwent MR angiography (MRA/time of flight) and carotid ultrasonography to diagnose IA and EA, respectively. Thirteen arterial segments were evaluated. Nearly ≥50% stenosis was considered significant. Stroke severity by National Institute of Health Stroke Scale (NIHSS) and diffusion-weighted imaging volume of infarct and outcome by modified Rankin score at 3 months was measured.

Results: A total of 100 AIS patients were studied (mean age 54.48 ± 13.31 years; M:F 65:35; 31% had large artery, 24% small artery, 18% cardioembolic, 2% other determined, and 25% undetermined etiology). Overall 55/100 had atherosclerosis (13% only EA, 33% only IA, and 9% both). In large artery disease strokes, figures were 26%, 52%, and 22% respectively. Overall (107/1300), 8.23% arterial segments had atherosclerosis. IA was seen in intracranial-internal carotid artery 12, middle cerebral artery 16, anterior cerebral artery 6, vertebral artery 16, basilar artery 4, and posterior cerebral artery 10. NIHSS score and mean DWI volume were higher in patients with significant IA (odds ratio = 3.37, P = 0.01) and (32.02 ml³ vs. 14.05 ml³; P = 0.03). Patients had worse outcome as the number of arterial segments with IA increased from 1 to 5.

Conclusion: IA was commoner (42%) in Indian AIS irrespective of stroke subtypes. Patients showing IA suffered more severe strokes. Greater IA involvement leads to worse outcome.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP14/247: Analysis of risk factors, clinical features, and outcome of stroke in patients with Vitamin B12 deficiency

Veerappan M ¹, Shanmugasundaram N ¹, Balasubramanian S ¹, Chandramouleeswaran V ¹, Narasimhan LR ¹

Aim: Hyper-homocysteinemia is an independent risk factor for stroke due to its pro-atherogenic effect. Studies relating the association between B12 deficiency and strokes are limited. In this study, we analyzed the risk factors, clinical features, and outcome of both arterial and venous stroke in patients with Vitamin B12 deficiency.

Materials and Methods: This prospective study was conducted in 165 patients with arterial and venous stroke admitted at the Institute of Neurology, Madras Medical College, Chennai, over a period of 1 year, between May 2017 and May 2018. Vitamin B12 and homocysteine were sent within 72 h of onset of symptoms and analyzed by radioimmunoassay.

Results: Among 165 patients, the male:female ratio was 4:1. A total of 115 patients had venous stroke and 50 patients had arterial stroke. Nearly 71% of patients belonged to young stroke (<45 years). Among dural venous sinus thrombosis (DVST), superior sagittal sinus (83%), and transverse sinus (78%) were the commonest dural venous sinuses involved. Headache and seizures were the commonest manifestation among CVT. Among fifty patients with arterial stroke, the most common presentation was hemiplegia and language disturbances. Alcoholism (P 0.046) and hyperhomocysteinemia (P 0.049) had significant correlation with low B12 in both arterial and venous strokes. Nearly 90% of DVST patients recovered completely with only 10% among arterial stroke improved completely.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP15/248: A comprehensive study of “outcomes and their predictors” in patients of acute ischemic stroke

Raghuvanshi S ¹

Background: Outcome measures other than disability and their predictors have not been studied rigorously in Indian acute ischemic stroke (AIS) patients.

Aim: This study aimed to study short- and long-term outcomes and their predictors in AIS: mortality, recurrence, disability (modified Rankin score [mRS]), cognitive dysfunction, and Stroke-Specific Quality of Life (SSQOL).

Methods: AIS patients were evaluated for short- (1 month) and long-term (3 and 6 months) outcome measures. Age, gender, risk factors, baseline National Institute of Health Stroke Scale (NIHSS), biochemical parameters, infarct volume, and presence of depression and anxiety were assessed as predictors of outcome.

Results: 92 AIS patients studied (mean age 54.05 ± 14.34 years; M:F 2.5:1). Etiology: Large vessel (39.1%), lacunar (15.2%), cardioembolic (15.2%), other determined (3.3%), and undetermined etiology (27.2%). Cumulative mortality: 6%, 9%, and 14% at 1, 3, and 6 months; predictors were high NIHSS and raised inflammatory markers (thin-layer chromatography, uric acid) (short term) and age >65 years (long term). Recurrence: 1%, 2%, and 4% at 1, 3, and 6 months. Worse disability: 84%, 75%, and 59% (mRS 2–5) at 1, 3, and 6 months; predictors were high NIHSS and depression. Cognitive dysfunction: 40%, 35%, and 31% (Mini–Mental State Examination < 22) at 1, 3, and 6 months; predictors were female gender (short term) and high NIHSS (long term). SSQOL: 38% had poor SSQOL; predictors were high NIHSS and depression.

Conclusion: Nearly 2/3^rd had significant disability, 1/3^rd poor cognitive functions and quality of life and 1 in 7 died at 6 months. High baseline NIHSS was the major predictor of all outcomes.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP16/249: Predictors of recanalization of cerebral sinus venous thrombosis

Rangam RP ¹, Sweetha R ¹

Introduction: Cerebral sinus venous thrombosis (CSVT) is a rare phenomenon frequency in young patients, multifactorial, gender-related specific causes, with a wide clinical presentation, highly variable clinical spectra, difficult diagnosis, variable etiologies and prognosis that requires high suspicious index.

Objective: The objective was to investigate recanalization in the first 6 months after CSVT and to know the cause of CSVT.

Methods: Twenty-six consecutive patients presenting with CSVT were enrolled in the study. Diagnosis was made by magnetic resonance imaging (MRI) and magnetic resonance venography (MRV). Patients were initially treated with intravenous heparin; acenocoumarol was given for 6 months. Cerebral MRI and MRV were done at 6 months to know persisting venous thrombosis, and factors causing CSVT were evaluated.

Results: Outcome at 6 months was good, with a median MRS score of 0 (range 0–2); 21 patients (82%) had no residual deficits, 2 patients suffered recurrent CSVT during follow-up. After 6 months, 57% of patients had complete recanalization and 43% of patients had partial recanalization. Nearly 65% of superior sagittal sinuses, 65% of transverse sinuses, 35% lateral sinus, 73% jugular veins, and 41% sigmoid sinuses were involved. Headache is the most common symptom in 25/26 patients followed by vomiting in 12 of 26, the least was loss of consciousness. Polycythemia was present in four patients and anemia was present in 15 patients. Four patients had vacuities profile positive. One patient had JAK4 mutation. One patient had ulcerative colitis.

Conclusions: The results suggest that complete recanalization occurs in 57% of patients and partial recanalization was seen in 43% of patients irrespective of oral anticoagulation.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP17/255: Short case series of patients thrombolyzed with tenecteplase in tertiary care settings

Shivde S ¹

Background: Tenecteplase is an advanced thrombolytic agent with novel benefits. We present here short case series of patients thrombolysed with tenecteplase comparing their outcomes.

Materials and Methods: All six patients presented with stroke within window period were thrombolysed with 0.2 mg/kg of tenecteplase as per protocol. National Institute of Health Stroke Scale (NIHSS) score and modified Rankin score (mRS) were calculated for each patient at the time of admission, at 24 h, at discharge, and at 3 months. All patients underwent baseline stroke workup and risk factor assessment.

Results and Discussion: Out of six patients included in the study, four were males and two were females. Two patients were stroke in young. All patients had one or two risk factors in the form of nicotine abuse, diabetes mellitus, or hypertension except one female. One patient had atrial fibrillation (cardioembolic stroke) and three patients had large-artery occlusions. NIHSS and mRS scores compared at the time of admission and discharge showed significant recovery. The scores compared at the interval of 3 months showed betterment in long-term outcomes. Only one patient had hemorrhagic transformation on repeat imaging and one patient had minimal gastrointestinal bleed. No mortality was reported.

Conclusion: Tenecteplase was associated with higher chances of early major neurological recovery and a trend for better functional outcome at 3 months. Lower cost, single intravenous bolus injection, more fibrin specificity, and less chances of bleeding make it an ideal thrombolytic agent in the Indian context.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP18/260: Bilateral hypertrophic olivary degeneration: A rare and delayed complication following pontine hemorrhage

Yadav P ¹

Introduction: Hypertrophic olivary degeneration is a rare transsynaptic degeneration that appears usually after 3–4 weeks of an injury to the Guillain–Mollaret triangle. The common causes are hemorrhage, infarct, trauma, surgery, or tumor. Usually, it is unilateral in structural causes. Here, the author presents a case of bilateral olivary nucleus degeneration following pontine bleed.

Case: A 37-year-old hypertensive, smoker with a history of pontine bleed 6 months back with residual left spastic hemiparesis, able to walk without support after 2 months of initial stroke. Now presented with 6 weeks history of ataxia and thereby difficulty of walking. There were severe tremors involving the left upper and lower limbs and head tremors. It was gradually progressive and he became wheelchair bound for the past 2 weeks. On examination, he had gaze-evoked nystagmus with severe postural and action tremors of the left limb. Rest tremor was also present. Motor power was gr 4+ in the left upper and lower limbs with mild spasticity. He had yes-yes type of head tremor. Reflexes were brisk on the left side with extensor plantar. There was no palatal myoclonus. In view of progressive deficits, computed tomography brain was done which was normal. Magnetic resonance imaging brain showed bilateral hypertrophic olivary degeneration. GRE sequences showed old hemosiderin deposits in pons. Thus, the cause of delayed worsening could be explained by hypertrophic olivary degeneration. Bilaterality can be explained by old bleed effecting both central tegmental tract and superior cerebellar peduncle.

Conclusion: Hypertrophic olivary degeneration should be considered in patients with delayed progressive worsening following pontine strokes. Appropriate imaging can lead to early diagnosis.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP19/272: Clinical and imaging profile of patients with cerebral venous thrombosis

Suhana S ¹, Gadad V ¹

Background: Cerebral venous thrombosis (CVT) is an uncommon form of stroke affecting young patients. It is defined by thrombosis of dural sinuses and cerebral veins. It has varied clinical findings, etiology, imaging findings, and outcomes. Numerous systemic and local causes are known in CVT.

Aims: The aim was to study the clinical and etiological spectrum of patients with CVT.

Methods: Fifty patients with clinical features suggestive of CVT were studied. Institutional ethical clearance was obtained. Details about demography, age, gender, onset, and symptoms were noted. They were evaluated for cause of CVT through hematological and biochemical studies and thrombophilia profile when required. Magnetic resonance imaging (MRI) brain with magnetic resonance venography (MRV) were performed.

Results: The mean age was 35 years. The common symptom was headache (92%) and sign was papilledema (66%) and hemiparesis (30%). Ophthalmoplegia was seen in 20% of the cases. The most common cause of CVT was obstetric (puerperium 28%, oral contraceptive pill use 10%), alcohol abuse (22%), and hyperhomocysteinemia (12%). Others were meningitis (10%), mastoiditis (2%), protein C deficiency, and antiphospholipid syndrome (2%). The sinus commonly involved was superior sagittal sinus (72%), transverse sinus (64%), and cortical veins (58%). Infarct (74%) and hemorrhage (54%) commonly involved parietal (58%) and frontal lobes (48%), respectively.

Conclusion: Clinical presentation of CVT is varied and a high index of suspicion is needed. Ophthalmoplegia was an uncommon finding in our patients. MRI brain with MRV is the diagnostic modality of choice. Early diagnosis and timely therapy can reduce the risk of acute complications and long-term sequelae.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP20/277: Risk factors and mechanism of stroke in 536 young patients at a tertiary referral hospital

Arepareddy PK ¹, Reddy N ¹, Tandra S ¹, Praveen ¹, Jabeen SA ¹, Kaul S ¹, Meena AK ¹, Borgohain R ¹

Objective: The objective of this study was to assess the risk factors, mechanism, and predisposing factors of stroke in young patients.

Materials and Methods: This is a retrospective study carried out from January 2010 to May 2018, in which young patients, who were admitted to Nizam's Institute of Medical Sciences, Hyderabad, were evaluated. Inclusion Criteria: All patients with ischemic stroke of <45 years. Exclusion criteria: Age >45 years.

Results: Of the total 536 patients, 61 were of <15 years’ age group and 475 were of 16–45 years’ age group. The distribution of mechanism of strokes was specific etiology (49%), undetermined (37%), large artery (8.%), small vessel (3.27%), and cardioembolic (1.6%) in <15 years. In 16–45 years’ age group, distribution was large artery disease (33%), specific etiology (23.78%), undetermined (26.10%), cardioembolic (8.84%), and small vessel (7%). In large artery disease of 16–45 years’ age group, the common risk factors seen are hyperhomocysteinemia (65.5%), alcohol (45.7%), smoking (39%), hypertension (35.5%), diabetes (26.2%), and dyslipidemia (16%). Cholesterol levels were higher in patients with large artery group. Common specific etiologies in <15 years’ age group included Moyamoya – 10, tuberculous meningitis (TBM) – 5, secondary vasculitis – 3, MELAS – 2, flow-mediated dilation – 2, and in age group 16–45 years, TBM – 46, secondary central nervous system vasculitis – 33, human immunodeficiency disease – 15, arterial dissection – 10, Moyamoya – 12, and antiphospholipid antibody – 7.

Conclusion: In this study group, in children, specific etiology was the most common cause of ischemic stroke, while in young adults, focal large artery disease was the predominant cause. Hence, we consider the possibility of early atherosclerosis is contributing to the cause of focal large artery disease.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

Friday, September 28, 2018, 13:00-14:00 h, E-Poster designated area: E Poster Session – 03: Autoimmune Disorders: AEP1/79: Treatment response over 6–8 months in acute demyelinating disorders of brain and spinal cord

Darole P ¹, Sundar U ¹, Kuchekar N ¹, Salunkhe M ¹, Karre A ¹

Aims: This study aimed to assess treatment response over 6–8 months in acute demyelinating disorders of brain and spinal cord.

Materials and Methods: This was a 2-year prospective observational study with serial recruitment of forty patients with acute and subacute demyelinating central nervous system disorders, after excluding multiple sclerosis. Clinical and radiological features were assessed on review at 6–8 months.

Operational Definitions: Optic nerve demyelination was graded as multisegmental/unisegmental. Extensive involvement of cord was graded as over five segments. Motor power improvement was by over 2 grades and normal visual acuity on review was graded as good clinical outcome. Among forty patients, 14 (35%) patients had optic neuritis, 10 (25%) patients had acute transverse myelitis (ATM), 8 (20%) patients had neuromyelitis optica (NMO), and 8 (20%) had acute demyelinating encephalomyelitis (ADEM). All patients in acute phase received steroids, two received intravenous immunoglobulin, and one plasmapheresis, additionally. In addition, two received azathioprine, seven received rituximab, and two received cyclophosphamide (NMO positive with ANA and dsDNA positive).

Results: (1) 62.5% (5/8) of patients with ADEM, 50% (4/8) of patients with NMO, 50% (5/10) of patients with ATM, and 78% (12/14) of patients with optic neuritis showed significant clinical improvement at 6–8 months, on treatment. (2) Among patients with myelopathy, presence of NMO antibody and spinal shock at presentation correlated significantly with extensive multisegmental involvement of cord (P 0.03 and 0.03, respectively). Spinal shock at diagnosis correlated with poor motor improvement at 6–8 months. Disease-modifying treatments could not be assessed for correlation with outcome due to inadequate numbers in subgroups.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP2/90: Clinical features and response to treatment in eight patients of autoimmune encephalitis from a tertiary care center in Eastern India: A prospective case series

Sawale V ¹, Bhat A ¹, Biswas A ¹

Aims and Objectives: The aim and objective was to study the clinical features and response to therapy in the cases of autoimmune encephalitis admitted to Bangur Institute of Neurosciences, Kolkata, West Bengal, India.

Design: prospective study.

Materials and Methods: We studied eight patients who presented with extrapyramidal features, status epilepticus (SE), epilepsy, behavioral abnormalities, and other neurological symptoms. The patients were positive for various autoantibodies. Clinical features were noted, and various investigations such as brain magnetic resonance imaging (MRI), cerebrospinal fluid (CSF) analysis, and screening for underlying malignancies were done. Patients were given intravenous immunoglobulin and/rituximab as treatment and the response to treatment was noted.

Results: There were eight patients of autoimmune encephalitis. The mode of onset was acute in four patients, subacute in 2, and 2 patients presented as SE. The most common clinical presentations were seizures followed by behavioral abnormality, and movement disorders. Brain MRI was done in all patients. It was normal in four patients and abnormal in rest. CSF was done in all patients; it was normal in five patients, had antibodies to N-methyl-D-aspartate receptor, while two had antibodies to voltage-gated potassium channel. Patients received immunotherapy, steroids, intravenous immunoglobulin, or rituximab. There was marked improvement in the clinical course in 50% of patients, while there was no relapse in the rest. There was one death also.

Conclusion: Autoimmune encephalitis is a relatively uncommon but underdiagnosed clinical entity which if diagnosed early can be treated with now widely available modalities.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP3/221: Morvan's syndrome after conventional scrotal tapping

Sahu CS ¹, Sharma S ¹, Sharma P ¹

Background: Occurrence of Morvan syndrome (MoS) after scrotal tap and injection of sclerosing agent for the treatment of hydrocele were earlier reported by our group. The present case is reported because of positive VGKC titer in scrotal fluid.

Case Report: A 50-year-old man presented with low backache radiating to both thighs anteriorly of 1-month duration associated with marked anxiety and decreased sleep. He had a history of hydrocele tapping with chemical instillation in scrotal sac 2 months prior to symptoms. On examination, he was profusely sweating on the face and hands. He was febrile, with elevated blood pressure of 150/90 mmHg, strength was normal in all four limbs, and deep tender reflexes were depressed. Fine tremors of outstretched hands and anterior abdominal wall were noted with intermittent twitching observed over the calf and thigh muscles. Blood investigation revealed sugar level of 249 mg/dl and presence of CASPR-2 antibody. Serum sodium was 132 mmol/l and serum creatine phosphokinase was normal. Electromyography study showed spontaneous grouped discharges in the form of doublets and triplets. Scrotal ultrasound revealed epididymitis and orchitis with interstitial fluid collection. 10 ml yellowish fluid was aspirated from contralateral scrotum and sent for VGKC detection that turned out to be positive for both CASPR2 and LGI1 antibodies of VGKC complex.

Conclusion: The present case has positive CASPR2 VGKC antibody titer in serum and positive CASPR2 and LGI1 antibody titers in scrotal fluid. We postulate that testis may be another site for VGKC complex antibody production in response to strong antigenic challenge.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP4/222: Is hypertrophic pachymeningitis really idiopathic?

Barvalia P ¹, Patil S ¹, Singh R ¹, Jagiasi K ¹, Soni G ¹, Aglave V ¹

Background: Hypertrophic pachymeningitis is a disorder characterized by localized or diffuse enlargement of the dura mater. It is seen in conditions such as sarcoidosis, syphilis, tuberculosis (TB), rheumatoid, Wegener's granulomatosis, fungal, meningioma, lymphoma, Tolosa–Hunt syndrome, IGG4, and idiopathic.

Objective: The objective was to determine various etiologies of pachymeningitis, clinical profile, and treatment response.

Materials and Methods: Twenty-two patients with clinical and radiological diagnosis of pachymeningitis were included in the study. The study was performed at a tertiary care center from January 2014 to July 2018, followed up over 1–2 years. Thorough workup for secondary causes was done. Patients were treated as per etiology.

Results: The mean age was 48 years with a male:female ratio of 1:1.4. Secondary causes (3 cases – IGG4, 2 – fungal, 1 – Wegner's granulomatosis, 1 – lymphoma, 2 – neurosarcoid, and 2 – TB) were found in 11 out of 22 cases and 11 cases were idiopathic. All presented with headache, 16/22 cases-multiple cranial nerve involvement except 1^st cranial nerve. 3rd-most common. ESR-2-120. Biopsy-5 cases, Steroids given to all except fungal, AKT-7 cases, second line immunosuppression-8/11 idiopathic cases. All had relief in headache and cranial nerve except, two patients expired, and two had no change in vision. Disease recurred in five cases.

Discussion: Idiopathic pachymeningitis is a diagnosis of exclusion. Hence, thorough workup is required to rule out secondary causes. It is highly responsive to steroids.

Conclusion: In our study, headache was the universal symptom, which responded to immunotherapy. We found secondary causes in 11 cases; etiological workup is of utmost importance as treatment changes. Recurrence is high, and long-term immunotherapy is required. The disease is treatable.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP5/250: Optical coherence tomography and visual-evoked potential in optic neuritis: Which predicts early?

Shivaraman AM ¹, Ranganathan LN ¹, Sarala G ¹, Nithyanandan A ¹, Rajan M ¹, Mugundhan ¹

Background: Optic neuritis, an inflammatory condition, produces acute vision loss. Etiologies include demyelinating diseases, autoimmune diseases, infections, or toxins. Optic neuritis is a common manifestation of multiple sclerosis. Optical coherence tomography (OCT), a noninvasive imaging, is an anatomical measure of anterior visual pathway. OCT measures retinal nerve fiber layer (RNFL) thickness. Visual-evoked potential (VEP) measures the response (measures latency and amplitude) to light stimulus.

Aim: The aim of this study was to assess the sensitivity of VEP and OCT in diagnosing optic neuritis and also to assess the pattern of RNFL changes in optic neuritis.

Methods: Twenty-four patients with optic neuritis due to multiple sclerosis and other etiologies were included in the study. Five multiple sclerosis patients without optic neuritis were also included. Patients underwent detailed clinical examination, neuroimaging, cerebrospinal fluid analysis, VEP, and OCT.

Results: 18/24 (75%) patients had abnormal VEP (prolonged P100 latency/reduced amplitude) and 6/24 (25%) had normal VEP. All the patients (100%) had retinal RNFL thinning. Average RNFL thickness of temporal quadrant was 50.1 + 4.45 µm, nasal quadrant was 77.15 + 7.47 µm, superior quadrant was 110.07 + 73.04 µm, and inferior quadrant was 111.76 + 14.32 µm. Three of the five multiple sclerosis patients without optic neuritis had normal VEP but RNFL thinning in OCT.

Conclusion: OCT is more sensitive than VEP in diagnosing optic neuritis. RNFL thinning irrespective of etiology was more in temporal quadrant. OCT detects early changes during the subclinical changes (optic nerve) in multiple sclerosis even before VEP shows abnormality.

Limitations: Larger cohort is needed to extrapolate the data.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP6/251: Spectrum of idiopathic inflammatory demyelinating diseases of the central nervous system

Saraf N ¹, Ganguly G ¹, Pandit A ¹, Das SK ¹

Objectives: The objective was to review clinical characteristics in primary demyelinating disorders such as multiple sclerosis and neuromyelitis optica spectrum disorders.

Design: Observational, prospective case series.

Setting: Bangur Institute of Neurosciences, Kolkata, West Bengal, India.

Materials and Methods: In this prospective analysis, sixty patients who presented with varied neurological findings, were subsequently diagnosed as primary demyelination of the central nervous system. Demographic and clinical characteristics were recorded. Brain and spine magnetic resonance imaging (MRI), cerebrospinal fluid analysis (CSF), and anti-neuromyelitis optica (NMO), cerebrospinal fluid (CSF) oligoclonal bands, and evoked potentials were done. Treatment received and responses (categorized as per patients and treating doctor's information) were noted and some were started on immunomodulation.

Results: There were sixty (females = 7) patients with primary demyelination, NMO spectrum disorder (NMOSD) (9), and multiple sclerosis (14). The mean age of presentation was 32 years (range: 11–63 years). The most common onset was subacute (64%). Predominant clinical presentations were optic neuritis (40%). CSF was done in all patients and were subsequently analyzed, 7 (9) NMOSD patients had anti-NMO positivity, there MRI were analyzed depending on the radiological criteria and site, all of them were given intravenous (IV) methylprednisolone, with two patient of NMOSD did not show any improvement and one was given plasmapheresis and one was given IV immunoglobulin, and showed significant improvement. Multiple sclerosis patients were put on interferon therapy.

Conclusions: Our study shows that demyelination diseases are prevalent in eastern part of India and our analysis and results are comparable to international studies.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP7/252: Clinical profile of neuromyelitis optica spectrum disorder at a tertiary care center

Ajit K ¹, Sarala G ¹, Shunmughasundaram K ¹, Balasubramanian ¹, Shanmughasundaram N ¹, Narasimhan RL ¹

Background: Neuromyelitis optica is an idiopathic, severe, demyelinating disease that preferentially affects optic nerve and spinal cord. It is now recognized as a broader spectrum, ranging from monophasic illness to polyphasic illness; disease is initially confined to optic nerve and spinal cord is now known to involve brainstem and cerebrum.

Objective: The objective was to study epidemiological characteristics, clinical profile, recurrence rates, and treatment of patients with neuromyelitis optica spectrum disorder (NMOSD) at a tertiary care center.

Methods: This was an observational, retrospective study done at the Institute of Neurology, Madras Medical College, from the prospectively maintained database of patients with NMOSD during January 2016–June 2018 who satisfied the International Consensus Diagnostic Criteria for NMOSD.

Results: There were 26 patients: females 88% and males 12%. The mean age of the symptom onset was 32 years (range 13–60 years, standard deviation ±11). Seven patients (27%) had monophasic illness and 19 patients (73%) had polyphasic illness with recurrences. The median recurrence rate was two per patient in the polyphasic group. Twenty patients with NMOSD were aquaporin 4 positive (77%), while six patients were seronegative. Longitudinally extending transverse myelitis (LETM) was the predominant magnetic resonance imaging (MRI) finding (57%). All patients received intravenous methylprednisolone and two patients were initiated on plasmapheresis in view of steroid resistance. All the patients were initiated on disease-modifying agents (mycophenolate mofetil – 3, azathioprine –19, and rituximab – 4).

Conclusion: NMOSD is a demyelinating disorder with female predominance, having polyphasic course, LETM being the most common MRI finding, with NMO antibody positivity in 77% of patients.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP8/253: Systemic lupus erythematosus presenting as aseptic meningitis and subdural hemorrhage

Saraf N ¹, Roy BK ¹, Dubey S ¹

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease affecting multiple organ system. Central nervous system manifestations of SLE are diverse. Aseptic meningitis and subdural hemorrhage are known to be induced by SLE, but primary presentation of either of them as an initial manifestation is very rare. Apart from immunological causes and drugs, the aseptic meningitis group can include some unidentified viral infections. There are no case reported in literature wherein patient presented with both meningitis and subdural hemorrhage (SDH) as the initial manifestation of SLE.

Case Report: An 18-year-old woman presented with a history of headache, fever, and joint pain rashes with acute-onset bilateral diminution of vision; the patient was found to have pallor, petechiae, ecchymosis, optic neuropathy, and meningeal signs. On investigation, there was bicytopenia with evidence of serositis; neuroimaging studies showed meningoencephalitis with SDH, cerebrospinal fluid was unremarkable, and dsDNA and antinuclear antibody were positive with raised erythrocyte sedimentation rate. Diagnosis of SLE was established based on ACR criteria. Once a diagnosis of SLE with meningoencephalitis with SDH was established, the patient was started on corticosteroids, and platelet transfusion was given. The patient showed marked improvement with the therapy, with decrease in headache and slight improvement in visual acuity.

Conclusion: We report a rare case of SLE with aseptic meningitis and subdural collection.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP9/254: A rare case report of MuSK myasthenia gravis in a human immunodeficiency virus patient

Faldu H ¹, Aglave V ¹, Ansari A ¹

Background: Autoimmune disorders have been frequently described in human immunodeficiency virus (HIV) patients, but there has been little research on the association between HIV and myasthenia gravis. MuSK myasthenia gravis coexisting with HIV is an even rarer entity. We report the case of a HIV patient who presented with MuSK myasthenia gravis.

Case Report: A 50-year-old female with HIV and baseline CD4 count of 170, on antiretroviral therapy from 1.5 years, presented with complaints of difficulty swallowing, change in voice, and intermittent double vision for 10 months. There was a history of fluctuations. On examination, she had bilateral mild lateral gaze palsy with mild asymmetric bifacial weakness. Her gag reflex was weak on both sides. Her CD4 count done 6 months back had increased to 477 and her magnetic resonance imaging brain was normal. Myasthenia gravis was strongly suspected. Repetitive nerve stimulation showed a decremental response in bilateral median innervated muscles. Her MuSK antibody was positive and Ach receptor antibody was negative, which confirmed the diagnosis of MuSK myasthenia gravis. There was improvement with addition of pyridostigmine, and she was later discharged on pyridostigmine, prednisolone, and azathioprine. On follow-up, the patient is showing sustained improvement.

Conclusion: Autoimmune disorders are being increasingly reported with HIV infection since the advent of highly active antiretroviral therapy. MuSK myasthenia gravis with HIV has been reported in very few case reports. The use of immunosuppressants in the treatment of these conditions should be carefully evaluated.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP10/266: A study on patients of vasculitic neuropathy attending tertiary care hospitals in Eastern India

Trivedi S ¹, Biswas D ¹, Pandit A ¹, Pal S ¹, Das SK ¹

Objective: The objective was to study the clinicopathological and electrophysiological profile of patients of vasculitic neuropathy.

Materials and Methods: Study Area: Neuromedicine Department, Bangur Institute of Neurosciences and Medical College and Hospital, Kolkata, West Bengal, India.

Study Population: Patients diagnosed with vasculitic neuropathy attending Neuromedicine department.

Study Duration: 3 years.

Design: Descriptive cross-sectional study.

Study Technique: Patients admitted or attending Neuromedicine department with features of vasculitic neuropathy were evaluated for this study. Apart from detailed history, clinical examination, routine biochemical and hematological investigations, and electrodiagnostic tests to define the topography and extent of sensory motor deficit were done. Nerve biopsy and specific etiology-guided investigations such as protein electrophoresis and vasculitis profile were done.

Results: In this study, 27 patients diagnosed as vasculitic neuropathy were included. The mean age of presentation was 43 years, with female sex being more commonly affected (70%). In most of the patients, large-fiber sensory symptoms predominated (70%) as the presenting complaint, followed by weakness (69%). Pain was present in 66.66% of patients, small-fiber symptoms were present in 55.55% of patients, and cranial nerve involvement was seen in five of the patients. Nerve biopsy could be done in 17 out of 27 patients. In nerve conduction study, the most common symptom was distal symmetrical axonal sensorimotor polyneuropathy (44.44%) followed by mononeuritis multiplex (37%), asymmetric sensorimotor neuropathy (18.51%), and mononeuropathy in one patient. Treatment given was either prednisolone alone or in combination with immunomodulatory therapy.

Conclusion: Vasculitic neuropathy may not always present as mononeuritis multiplex as most patients when reach neurologist have overlap polyneuropathy which may delay diagnosis and treatment.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP11/269: Hashimoto encephalopathy with abnormal movements

Kar M ¹, Padhy BP ¹, Radhakrishna H ¹

In Hashimoto's thyroiditis, the antithyroperoxidase (TPO) and antithyroglobulin antibodies disrupt thyroid function resulting in encephalopathy which can be accompanied by ataxia and involuntary movements. All these can occur despite a biochemically euthyroid state. We hereby present such a case.

Case Report: A 64-year-old male rheumatologist came with a history of altered sensorium, had a fall at bathroom on May 21, with level of consciousness for few minutes with irrelevant talk from May 26 with acute psychosis. There was no history of vomiting, nausea, diplopia, blurring of vision, and trauma. There was also no history of fever, chest pain, cough, and breathlessness. He had hypotension on arrival with no inotropic support and fluid management was started, but blood pressure was very labile. He was very restless at admission with involuntary movements of all the four limbs with right more than left. Magnetic resonance imaging with contrast was unremarkable. Cerebrospinal fluid study was done which showed raised protein with normal cell count and sugar level. His anti-TPO level was high, which was considered Hashimoto's/autoimmune/viral infection. Electroencephalography was within normal limits. Hashimoto's autoimmune encephalitis was kept and the patient was started on steroids (methylprednisolone 5 doses). Psychiatry consultation was sought for psychosis and started on antipsychotic. He was advised to continue oral steroids for short course. He responded very well to the steroids and resumed back to work.

Conclusion: As neuroimaging and encephalogram can be normal in patient having encephalopathy due to abnormalities caused by thyroiditis. Hence, it is worth doing a workup for thyroid antibodies as it is a steroid-responsive encephalopathy associated with autoimmune thyroiditis.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP12/302: Relevance of anti-thyroperoxidase antibodies in the clinically unexplained encephalopathy: Is it underreported or a mere coincidence?

Karthikeayan V ¹, Sudha AP ¹, Chander P ¹, Sathishkumar V ¹, Dineshnayak ¹, Narayanan L ¹

Background: Hashimoto's (steroid responsive) encephalopathy, associated with autoimmune thyroiditis, is an established subclass of autoimmune encephalitis. In a suspected case of encephalitis, in which differentials have been ruled out, antithyroid antibodies are validated.

Objective: The objective was to describe the clinical spectrum and conundrums in the management of patients with anti-thyroperoxidase (TPO) antibody (ATPOAb)-positive encephalopathy.

Materials and Methods: Study design: Case series. ATPOAb was screened for in ten patients who presented with encephalopathy, after ruling out structural lesions, metabolic insults, and neuronal antibodies. Five cases significantly positive for ATPOAb were described.

Results: Four of the five cases were males. Two males presented with status epilepticus and both responded only after steroid therapy. Seizure recurrence was observed upon steroid withdrawal. Another patient presented with headache. He had a past history of encephalopathy, during which he was diagnosed with hypothyroidism, treated with meningeal doses of antibiotics and steroids. On follow-up, significant ATPOAb titer was detected, but steroid was deferred since encephalopathy features were absent. The fourth male had noncirrhotic hyperammonemic encephalopathy. Search for the source of hyperammonemia was futile; however, ATPOAb was significantly elevated. The final patient presented with subtle ataxia and was considered as functional prior to her antithyroid antibody positivity. Presently, she is under follow-up for response to steroids.

Conclusion: Excluding the two cases with seizures, other cases portray discordance between the clinical scenario and antibody status, despite satisfying criteria for Hashimoto's encephalopathy. Scientific statement on approach to encephalopathy associated with anti-TPO antibodies is the need of the hour.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP13/341: Autoimmune encephalitis: Case series from a tertiary care hospital

Reddy RJ ¹

Introduction: Autoimmune encephalitis forms an important group of modifiable neurologic disorders. These are being increasingly recognized due to the availability of serum markers and have been better characterized in recent years. These disorders have various clinical patterns which correlate with antibodies.

Objectives: The objective was to describe clinical features, antibody profile, and treatment outcomes of seven such patients admitted to our hospital.

Materials and Methods: Patients admitted to Government General Hospital, Guntur, between September 2017 and June 2018 and in whom autoimmune encephalitis was suspected were included in the study. Patients were identified on the basis of clinical history, examination findings, and investigations. Data were reviewed from imaging (magnetic resonance imaging), cerebrospinal fluid analysis, electroencephalography, and malignancy workup.

Results: The presenting symptoms in the cohort were cognitive and constituted behavioral disturbances (n = 4), seizures (n = 7), and altered sensorium (n = 1). Five patients were positive for anti-LGI1 antibodies, anti-CASPR2 antibodies were positive in one patient, and one patient was positive for anti-N-Methyl-D-aspartate receptor antibodies. All patients received immune therapy, five of them improved.

Conclusions: Autoimmune encephalitis should be promptly considered in a patient presenting with altered sensorium, seizures, and memory disturbances when the initial investigations for infectious causes are negative. Serological studies are valuable in arriving at diagnosis. Immune therapy should be considered early for good outcome.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP14/353: Clinical features, disease course, outcome, and comparison as per serostatus and application of International Consensus Criteria in neuromyelitis optica spectrum disorder

Bhatia R ¹, Singh N ¹, Bali P ¹, Srivastava MVP ¹, Goyal V ¹, Dash D ¹, Tripathi M ¹, Prasad K ¹, Garg A ¹, Joseph L ¹, Sreenivas V ¹, Singh V ¹

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a demyelinating disorder of the central nervous system. In this ambispective study, we evaluated clinical characteristics, imaging features, response to therapy, and disability outcomes in patients with NMOSD.

Methods: This is an ambispective study of NMOSD patients following up at AIIMS, New Delhi, India. Detailed patient data were collected.

Results: A total of 97 patients (28 males/69 females) with NMOSD were evaluated. The mean age of onset was 29 years (standard deviation ±11.2 years). A total of 64 patients (65%) were positive for acquaporin 4 antibody. The most common syndrome at disease onset was longitudinally extending transverse myelitis (55.7%) followed by optic neuritis (34.02%) with/without brainstem and cerebral involvement. Mean time until diagnosis of NMO was made was 27 months and median attacks until the diagnosis were 2. Azathioprine was the most common immunotherapy (82.47%) followed by rituximab (6.1%) and mycophenolate mofetil (4.1%). Significant decrease in number of relapses was observed postazathioprine (P = 0.00). Visual recovery was complete in 25%, mild in 23.4%, moderate in 12.5%, and severe in 25% of patients. Motor recovery was complete in terms of walking without support in 67.27%, with support in 44.6%, and no ambulation in 21.4% of patients. Nearly 37% more patients could now be classified as NMOSD after application of the new criteria; seven continued to remain nonclassifiable. There was no difference in age of onset and gender and visual and motor recovery as per the antibody status.

Conclusions: Clinical pattern of NMOSD is similar to the reported literature. Newer criteria are more sensitive to diagnose NMOSD. Serostatus does not affect treatment response.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP15/359: Autoimmune encephalitis: A case series

Trivedi S ¹, Datta A ¹, Kumar H ¹, Anand SS ¹, Shobhana A ¹, Das C ¹

Out of 11 patients of autoimmune encephalitis, 9 were positive for anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, 1 for anti-voltage-gated potassium channel, and 1 for anti-PNMA antibody. Nearly 72.2% of autoimmune encephalitis patients present in the 2^nd and 3^rd decades of life. Around 72.2% of patients of autoimmune encephalitis were female. Almost 44% of anti-NMDAR encephalitis patients were presented with seizure at onset and 44% of patients with altered mental status and behavioral problems. Perioral automatic movement with or without limb automatism was present in 66% of anti-NMDAR encephalitis patients. One female patient of anti-NMDAR encephalitis had ovarian dermoid cyst. Delta brush was present in 22% of patients with anti-NMDAR encephalitis; other patients had generalized slowing with or without epileptiform discharges. Cerebrospinal fluid (CSF) showed lymphocytic pleocytosis in three patients and increased CSF protein in one patient. Magnetic resonance imaging brain showed signal changes in two patients. Immunomodulatory therapy (methylprednisolone, immunoglobulin, rituximab, and/or cyclophosphamide) with multiple antiepileptic drugs has been done with variable outcome.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

AEP16/AWPN5: Typical and atypical neuromyelitis optica spectrum disorder and its implications

Chaitanya Koduri VK ¹, Nagarjunakonda VS ¹, Uppala V ¹, Gajula R ¹

Background: Neuromyelitis optica (NMO) has evolved from Devic's classical description to a broader disease spectrum of NMO spectrum disorder (NMOSD), from monophasic illness to a polyphasic illness with multiple recurrences; the disease is confined to optic nerve and spinal cord to now brain stem, cerebrum, and even with endocrinopathy due to hypothalamic involvement (1), coexisting infections (2), and a variety of autoimmune diseases, including nonorgan-specific autoimmune diseases and co-existent autoantibodies without diseases (3).

Objectives: The objective was to report the epidemiological characteristics, clinical presentations, recurrence rate, treatment, and response to therapy in 23 patients with NMOSD among the Indian population.

Materials and Methods: An observational, retrospective analysis of our prospectively maintained database of patients during the period of May 2018–June 2018 who satisfied International Panel for NMO Diagnosis revised criteria (4) of NMOSD was done.

Results: Twenty-three case records of NMOSD were retrieved. Fourteen cases were aquaporin 4 positive and nine were aquaporin 4 negative (1) Mean age was 39.6 years, median age was 42 years (range 17–64 years), and female-to-male ratio was 1:2.8; (ii) Clinical presentations included longitudinally extensive transverse myelitis being the most common followed by optic neuritis, area postrema syndrome, seizures, and brainstem encephalitis; (iii) Two had associated herpes simples virus encephalitis and one had cerebrospinal fluid (CSF)-Venereal Disease Research Laboratory positivity. Two had associated retroviral disease; (iv) Two had antinuclear antibody and anti-dsDNA antibody-positive vasculitis; and (v) one had CSF oligoclonal bands in CSF and an open ring enhancement on magnetic resonance imaging suggestive of tumefactive demyelination.

Discussion and Conclusions: Contrary to the current literature, unusually, there is a male preponderance in our study population (1), (5), (6) (*). Atypical lesions or course (red flags) should suggest additional workup for associated diseases and prompt treatment which can lead to significant recovery (*). Aquaporin 4 antibody positivity, severity at presentation, associated diseases, and relapse rate determine the prognosis.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

Friday, September 28, 2018, 17:00-18:00 h, E-Poster designated area: E Poster Session – 04: Movement Disorders: MEP1/7: Hemimasticatory spasm with facial hemiatrophy and localized scleroderma: Is it strictly unilateral?

Radhakrishnan D ¹, Goyal V ¹, Shukla G ¹

We report a rare case of a 45-year-old female with a 15-year history of progressive left facial thinning with frequent episodes of involuntary jaw closure and almost continuous rippling movements over her left-sided masticatory muscles. There was localized scleroderma, left facial hemiatrophy, and left hemimasticatory spasm (HMS). Localized scleroderma was proven histopathologically. Electrophysiological studies revealed normal blink reflex on both sides. Her masseter inhibitory reflex was absent bilaterally and surface electromyogram (EMG) showed spontaneous bursts of high-frequency activity over bilateral masseter and left temporalis muscles. The patient responded remarkably with bilateral botulinum toxin injection. This case highlights the presence of rare bilateral involvement of HMS, especially on EMG and excellent response to botulinum toxin.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP2/34: Isolated hemichorea as a presenting feature of Fahr's syndrome due to iatrogenic hypoparathyroidism

Desai K ¹, Walzade P ¹, Ravat S ¹, Jain N ¹, Agarwal P ¹

Basal ganglia calcification (BGC) can be physiological (seen in up to 20% of routine computed tomography scans) or pathological. Fahr's “disease” denotes a primary (sporadic or familial) cause of BGC, an unraveling of the genetic underpinning of which is underway in recent years. Fahr's “syndrome” is the term suggested for secondary causes of BGC, and the latter include hypoparathyroidism, which itself could be either idiopathic/primary or secondary to inadvertent removal of parathyroid glands during thyroid surgery. Chronic hypocalcemia in these cases triggers a metabolic cascade, leading to deposition of calcium in the brain, potentially causing neurological problems including movement disorders that are usually bilateral in keeping with symmetrical lesions. We report a case of isolated unilateral chorea with bilateral BGC, presenting 20 years after thyroidectomy-induced iatrogenic hypoparathyroidism.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP3/50: Regional susceptibility of the brain in copper toxicity

Kumar V ¹, Kalita J ¹, Misra UK ¹, Bora HK ¹

Introduction: The human prototype of copper (Cu) toxicity is Wilson's disease, in which corpus striatum is commonly involved followed by thalamus and midbrain. Animal studies mostly region specific and none of the studies evaluated regional susceptibility of brain in Cu toxicity.

Methods: Twelve male Wistar rats (205 ± 10 g; 10 weeks old) were included: six were exposed to 100 mg/kg body weight for 4 weeks orally and six were control received saline water orally by gavage. Their neurobehavioral studies (ratarod, grip strength, spontaneous locomotor activity (SLA), Y maze) were done at baseline and at 1 month. At 1 month, rats were sacrificed and their corpus striatum, thalamus, frontal cortex, and hippocampal region were dissected and used for assay of tissue Cu, oxidative stress (glutathione [GSH], total antioxidant capacity [TAC], and malondialdehyde [MDA]) and glutamate.

Results: Experimental group reduced in weight compared to control. Tissue Cu (1.58 ± 0.04 vs. 2.89 ± 0.02 µg/g wet tissue weight; P < 0.01), MDA (2.67 ± 0.12 vs. 5.04 ± 0.13 nmol/mg protein; P < 0.01), and glutamate (5.03 ± 1.11 vs. 6.46 ± 1.16 nmol/mg protein; P = 0.01) were highest in corpus striatum. This was followed by thalamus, hippocampus, and frontal cortex. GSH (2.94 ± 0.27 vs. 2.29 ± 0.12 mg/g tissue weight; P = 0.02) and TAC (2.41 ± 0.07 vs. 1.90 ± 0.07 nmol Trolox Eq./g tissue weight; P = 0.01) levels were reduced. These levels correlated with grip strength, ratarod retention time, Y maze attention score, and SLA parameters.

Conclusion: Cu toxicity has regional affinity for corpus striatum and thalamus, revealing high Cu, oxidative, and excitotoxicity damage of these regions. These intracranial affinities are similar to magnetic resonance imaging changes in Wilson's disease.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP4/44: An interesting case of symptomatic cervical dystonia in a patient with acute cerebellar hematoma

Mahale R ¹, Mehta A ¹, Javali M ¹, Acharya P ¹

Dystonia is a hyperkinetic movement disorder characterized by involuntary sustained muscle contractions leading to abnormal postures and repetitive movements. It is a disorder of motor programs controlling semiautomatic movements or postures. It was originally claimed as a disease of basal ganglia. At present, dystonia is regarded as a “network” disorder including the cerebellum. Dystonia secondary to a cerebellar lesion is uncommon. Hereby, we report a 64-year-old male patient who presented with headache, inability to stand, and imbalance while walking of 1 day duration. On admission, he was conscious, oriented. Fundus examination showed papilledema in both eyes. Speech was slurred, scanning in quality. Bilateral gaze-evoked horizontal jerky nystagmus was present. Motor examination showed hypotonia with hyporeflexia. He had mild incoordination in both upper limbs, impaired bilateral knee-heel coordination. Gait was ataxic with mute plantar response. Computed tomography head showed hyperdense lesion, suggestive of bleed in the left vermian/para-vermian region with fourth ventricular extension and hydrocephalous. The patient was taken up for ventriculosubgaleal shunt for cerebrospinal fluid diversion. The patient tolerated the procedure well. During the postoperative day 2, there was abnormal posturing of neck in the form of head tilt to the right (laterocollis), suggestive of cervical dystonia. He was started on trihexyphenidyl (total dose of 8 mg) and tetrabenazine (50 mg). At 15 days following discharge, he had total improvement in the cervical dystonia. Cerebellar bleed can rarely cause dystonia, with very few reported cases of cervical dystonia following bleed.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP5/119: Frontal assessment battery in patients with Parkinson's disease and correlations with formal measures of executive functioning at a tertiary care center in Western India

Saini PK ¹, Sharma CM ¹, Kumawat BL ¹

Introduction: Parkinson's disease is a neurodegenerative disorder characterized by motor and nonmotor symptoms. Cognitive impairment in Parkinson's disease primarily consists of executive dysfunction. Frontal assessment battery (FAB) is a validated and easily performed tool for assessment of executive functions consisting of six subsets to explore different functions related to the frontal lobe.

Aims: This study was performed to evaluate the clinical usefulness of the FAB in identifying executive dysfunction in Parkinson's disease and to determine if FAB scores in Parkinson's disease are correlated with formal measures of executive functions such as fluency tests and trail making test in a group of Indian patients with Parkinson's disease.

Materials and Methods: This study was conducted at SMS Medical College, Jaipur, from December 2016 to March 2018. Cases were taken on the basis of random sampling from neurology outpatient department. We included 60 patients diagnosed with Parkinson's disease and 40 healthy age-, sex-, and education-matched controls in our study.

Results: In our study, among 60 cases, there were 36 men and 24 women with Parkinson's disease. In cases, the mean age was 56.8 ± 7.4 years, mean education was 10.6 ± 4.8, mean duration of disease was 6.64 ± 3.88, mean Mini–Mental Status Examination (MMSE) was 26.2 ± 2.6, and mean FAB was 10.8 ± 4.2. The mean FAB scores were significantly decreased in cases as compared to controls, and significantly correlated with age, trail making test, and MMSE scores.

Conclusion: FAB is a useful screening test to detect executive dysfunction in Parkinson's disease, and it can be used to detect subtle cognitive deficits in these patients.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP7/183: Distinctive upper limb dystonia as a correlate to the “eye-of-the-tiger” magnetic resonance imaging sign

Chheda A ¹, Khadilkar S ¹

Background: The study of phenomenology in movement disorder helps classify movement into tremor, dystonia, chorea, myoclonus, etc., based on the rate, rhythm, range, region involved, and the maneuvers or situations that bring out or suppress the abnormal movement. Further study of the characteristics of movement can help predict etiology, e.g., tic tac chorea in Sydenham's disease, slow myoclonus in subacute sclerosing panencephalitis, etc. We describe the characteristic upper limb dystonia as a correlate to “eye-of-the-tiger” brain magnetic resonance imaging (MRI) sign seen in pantothenate kinase-associated neurodegeneration (PKAN).

Methods: The authors examined children <15 years with upper limb dystonia having MRI correlate of “eye-of-the-tiger” sign and age-matched controls with dystonia due to any cause and MRI not showing “eye of the tiger” over a period of 2 years.

Results: Five cases of upper limb dystonia were recognized having MRI correlate of “eye-of-the-tiger” sign. All of them had distinctive pattern of upper limb dystonia with extension at shoulder, flexion at elbow, and at times pointing index finger, appearing as if it is going behind back. The dystonia was asymmetric and action induced initially, and later appeared at rest. Children also showed quasi-purposive quality of dystonia by keeping dystonic hand over the waist. Only one control group patient out of 15 examined showed the characteristic dystonia.

Conclusions: “Eye-of-the-tiger sign” in children is highly specific for PKAN. Therefore, the representative upper limb dystonia is a special feature of PKAN and helps in its early and accurate diagnosis.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP8/185: Pantothenate kinase-associated neurodegeneration: A rare clinical entity

Malaka M ¹

Introduction: Pantothenate kinase-associated neurodegeneration (PKAN) is an autosomal recessive disorder caused by the absence or deficiency of PANK2 gene located on chromosome 20p13-p12.3. It is a motor disorder of extrapyramidal type associated with dystonia, rigidity, and involuntary movements (choreoathetosis and tremor).

Case Report: A 15-year-old male patient born of third-degree consanguineous marriage with normal developmental mile stones presents with complaints of abnormal posturing of both upper limbs since 1 year, unclear speech since 6 months, and abnormal posturing of both lower limbs since 2 months. General examination revealed dystonic posturing of both hands in the form of flexion of wrist and fist, occasional dystonic smile, and task-specific dystonia. Neurological examination higher mental functions normal, tone increased in all limbs, power 5/5 in all groups of muscles, reflexes 2+ (DTR) plantar flexor response bilaterally. There was no sensory and cerebellar signs. Fundoscopy revealed retinitis pigmentosa and macular dystrophy in both eyes. Magnetic resonance imaging (MRI) of brain revealed focal altered signal intensities symmetrically in globus pallidus which appeared hyperintense on T2-weighted images with surrounding areas of low signal intensity in all sequences (eye-of-the-tiger sign), which was suggestive of neuronal degeneration, probably Hallervorden–Spatz disease.

Conclusion: PKAN is a rare syndrome which has to be suspected in child with dystonic posture and MRI features. Currently, treatment is symptomatic. The ongoing involvement of physical, occupational, and speech therapists can delay complications of disease.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP9/205: Inherited movement disorders in children: The enigma of neurotransmitter disorders

Saini A ¹, Gowswami J ¹, Attri S ¹, Suthar R ¹, Sahu J ¹, Sankhyan N ¹, Saini L ¹, Singhi P ¹

Objective: Pediatric neurotransmitter disorders refer to an inherited group of neurometabolic syndromes that are an important cause of movement disorders in children.

Methods: Retrospective review of case sheets over the past 5 years identified 5 families (8 cases) of monoamine synthesis (tyrosine hydroxylase, aromatic L-amino acid decarboxylase, and BH4 deficiency); 5 families (6 cases) of gamma-aminobutyric acid metabolism (succinic semialdehyde dehydrogenase (SSADH) and pyridoxine deficiency); and 1 case of glycine metabolism disorders.

Results: A 10-year-old girl had delayed motor milestones, recurrent hypoglycemic encephalopathy, oculogyric crisis, ataxia, limb dystonia, and autonomic instability secondary to c.475G>A p. Ala159Thr homozygous variation in DDC Dopamine decarboxylase gene. A 9-month-old boy presented with neuroregression and dystonia. Similar symptoms were noted in younger sibling and father's 21-year-old first cousin. Compound heterozygous variations were detected in TH gene. Two severe cases of BH4 synthesis were diagnosed from two children with neuroregression and dystonia. One case of GTP cyclohydrolase I deficiency was diagnosed in a 5-year-old boy with fluctuating dystonia and wide-based gait. Two 10-year-old twin brothers presented with seizures and global developmental delay associated with SSADH deficiency. Variations in ALDH7A1 gene in four children resulted in pyridoxine-responsive seizures and secondary neurotransmitter deficiency. A case of hereditary hyperekplexia due to homozygous variation in the SLC6A5 gene, p. L460P, affecting glycine metabolism, was identified in a 7-month-old girl.

Conclusion: Neurotransmitter disorders represent an enlarging group of inherited movement disorders in children often requiring specialized diagnostic procedures for detection.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP10/232: A case of neuroacanthocytosis

Bandari RK ¹, Narisetti V ¹

A 45-year-old female presented with a history of behavioral disturbances for 28 years and involuntary movements for 4½ years. Illness started with behavioral disturbances, being meticulous about cleanliness. Becomes anxious and restless until she does the intended act. History of eating excessive amount of food and vomiting voluntarily with the fear of becoming obese was noted in the patient. There was involuntary repetitive grunting sounds. The patient was unable to suppress the movements completely, but subsiding during sleep. There was a history of seizures – generalized tonic-clonic seizures in semiology. Since 2 years, they are well controlled with medication. Family history included similar complaints in other family members. There were behavioral disturbances and involuntary movements in her elder sister and behavioral disturbances in her younger brother.

Examination: Normal except for involuntary movements: chorea, dystonia, and vocal tics. Gait: variable – stance, stride length, arm swing with abnormal twisting posture of foot.

Investigations: CBP – normal, peripheral smear – acanthocytes (10%); liver function test, renal function test, and electrolytes – normal, creatine phosphokinase – normal (174 U/L), thyroid profile: normal (thyroid-stimulating hormone – 5.46; T3 – 97.74; and T4 – 6.8), lipid profile: total cholesterol – 209; low-density lipoprotein – 109; high-density lipoprotein – 40; triglycerides – 297; very low-density lipoprotein – 59, ceruloplasmin – 0.46; copper – 126, 2D ECHO: normal, NCS – axonal neuropathy, and magnetic resonance imaging brain – atrophy of caudate and putamen.

Review of Literature: Autosomal recessive disorder. Progressive syndrome includes movement disorder and behavioral and cognitive disturbances. It is caused by various mutations of gene VPS13A on chromosome 9, coding for chorein. Characteristic phenotype includes chorea, a very peculiar “feeding dystonia” with tongue protrusion, orofacial dyskinesias, involuntary vocalizations, dysarthria, and involuntary tongue and lip biting. Neurodegeneration affects predominantly the caudate nucleus, putamen, and globus pallidus.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP11/291: The Parkinson's disease sleep scale: evaluation of sleep disturbances in Parkinson's disease patients

Shakthi C ¹, Sritharan B ¹, Manivannan MR ¹, Muthuveeran M ¹, Justin C ¹, Ganesan K ¹, Amalraj E ¹, Chezhian D ¹, Pandian GD ¹, Pavalam B ¹, Raja SP ¹, Muthukumar J ¹

Introduction: The pathology of Parkinson's disease extends far beyond the nigrostriatal pathway and results in nonmotor symptoms in addition to the common motor symptoms. The most common are autonomic disturbances, mood disorders, and sleep problems. Sleep disturbances occur in 96% of patients with Parkinson's disease and appear to be due to neurochemical and neurodegenerative process involving central sleep regulatory centers such as forebrain, thalamus, and midbrain dopamine neurons.

Methods: We enrolled 103 patients with Parkinson's disease and were given Parkinson's disease sleep scale (PDSS) questionnaire addressing 15 items which included quality of sleep, sleep onset, nocturnal restlessness, nocturnal psychosis, nocturia, nocturnal motor symptoms, and daytime sleepiness.

Results: PDSS was impaired more in advanced than early stages.

Conclusion: Sleep disturbances in Parkinson's disease especially daytime sleepiness is associated with vehicle accidents and impairs the quality of life. PDSS can be used as a screening tool to subject patients to sleep studies and treating them.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP12/298: Assessment of pain and its correlation with motor disability in Parkinson's disease

Agrawal AK ¹, Anand KS ¹

Objective: To assess the prevalence of pain, pain types, and its correlation with motor disability in Parkinson's disease (PD).

Background: Pain is common, distressing often overlooked nonmotor symptoms in PD.

Methodology: One-hundred PD cases were recruited, staged by unified Parkinson's disease rating scale (UPDRS) III, V, and VI. Pain was assessed by King's PD pain scale (KPPS).

Results: The mean age was 62.70 ± 11.18 years, with age range of 38–85 years. Male:female ratio was 73:27. The mean duration, age of onset, and levodopa equivalent daily dose (LEDD) were 56.94 ± 46.22 months, 57.77 ± 12.05 years, 414.11 ± 318.99 mg, respectively. Mean ± standard deviation of UPDRS III, V, and VI scores were 15.01 ± 7.55, 2.39 ± 0.72, and 79.57 ± 18.70, respectively. Pain prevalence was 70% with mean KPSS of 5.23 ± 6.42. Different pain types included musculoskeletal (47%), chronic pain (13%), fluctuation related (35%), nocturnal (27%), orofacial (12%), discoloration (11%), and radicular (17%). Patients with pain had lower mean age, more disease duration, younger age onset, high LEDD and higher UPDRS III scores compared to patients without pain but insignificant (P > 0.05); however, the difference in mean UPDRS V and VI between the two groups was significant (P < 0.05). KPPS scores showed positive correlation with UPDRS III and V with r = +0.494 and + 0.347, respectively, while the correlation with UPDRS VI was negative with r = −0.461 and these correlations were significant (P < 0.05).

Conclusion: Nearly 70% of patients had one or more types of pain with statistically significant correlation of pain with motor disability, staging of disease, and functional disability (UPDRS III, V, and VI).

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP13/309: Interrater and intrarater agreement on the 2018 consensus statement on classification of tremors

Rajan R ¹, Pandey S ¹, Reghu A ¹, Srivastava A ¹

Background: Well-defined criteria are essential for the diagnosis and classification of tremors as they are predominantly diagnosed clinically. Whether the criteria set forth in the new 2018 consensus statement on the classification of tremors achieves reliable and consistent differentiation of tremor syndromes is unknown.

Objective: To assess the interrater and intrarater agreement on the 2018 consensus statement on classification of tremors.

Methods: Two independent movement disorder specialist raters reviewed videotaped recordings of tremor patients and applied the 2018 consensus classification criteria to classify the tremors into Axis 1 syndromes. Intraclass correlation coefficient (ICC) and Cohen's κ were calculated for interrater and intrarater agreement.

Results: Forty-nine structured videotaped neurological examinations were reviewed. For interrater agreement, ICC = 0.468 (95% confidence interval [CI]: 0.223–0.658; P < 0.001) and Cohen's κ = 0.388 (95% CI: 0.192–0.584; P < 0.001). For intrarater agreement, ICC = 0.689 (95% CI: 0.510–0.812; P < 0.001) and Cohen's κ = 0.670 (95% CI: 0.494–0.846; P < 0.001). Majority of the interrater discordant pairs (19/49) involved a classification of dystonic tremor syndrome (13/19) or essential tremor plus (11/19).

Conclusion: The current consensus classification achieves modest interrater agreement and better intrarater agreement. Majority of the discordant classifications involved a judgment on the presence or absence of dystonia.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP14/327: Brothers with paroxysmal kinesigenic and paroxysmal nonkinesigenic dyskinesia: A case report

Kumar CA ¹

A 15-year-old male patient presented with a history of sudden attacks of abnormal movements of all four limbs lasting for <30 s occasionally associated with falls without loss of consciousness. The episodes were associated with physical activity. He had 8–10 such episodes in a day. He did not have any abnormalities during examination. Blood investigation and radiological investigations were unremarkable. An attack during video electroencephalogram (EEG) recording did not reveal any abnormality in the EEG waveforms. Based on the unremarkable findings during clinical examination and investigations, paroxysmal kinesigenic dyskinesia was suspected. He responded to treatment with carbamazepine. A retrospective history revealed similar attacks of abnormal movements in his elder brother, but not associated with physical activity and with a lesser frequency (1–2 episodes/month), but lasting for a longer duration (approximately 5 min). His clinical examination and investigations (blood/radiological/EEG) were unremarkable. Even he was treated with clonazepam and carbamazepine. Frequency of dyskinetic attacks reduced. Paroxysmal kinesigenic dyskinesia is a rare neurological disorder characterized by brief episodes of dyskinetic movements provoked by physical activity. Paroxysmal nonkinesigenic dyskinesia is associated with sudden attacks of abnormal movements which lasts for minutes and is not triggered by physical activity. Both the disorders have a genetic basis. This case report is important as it shows the occurrence of both paroxysmal kinesigenic and nonkinesigenic dyskinesia within a single family. It highlights the importance of awareness about this clinical entity among clinicians as this condition can be misdiagnosed as a psychosomatic illness.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

MEP15/AWPN25: Prevalence and spectrum of pain in Parkinson's disease

Meenakshisundaram US ¹, Samivel B ¹, Natarajan T ¹, Ranganathan LN ¹, Sundaram T ¹, Kannan V ¹, Venkataraman C ¹

Background: Pain is one of the recognized nonmotor symptoms in Parkinson's disease (PD). However, not much research has been done into definitively defining pain and its management in PD. No specific characteristics or correlation for pain have been described.

Aim: To study the prevalence, type, and severity of pain in patients with Parkinson's disease.

Methods: This was an observational study that included 66 patients with PD from the movement disorder clinic. A structured interview which included questions about the presence, type, and severity of pain was administered. The disease characteristics such as duration and stage of PD were also noted. The severity of pain was graded using the visual analog score.

Results: Of the 66 patients included in the study, 42 (63.6%) had complaints of somatic pain. Pain was present more commonly in females with P < 0.5. The most common localization of pain was to the lower back (33.33%) followed by the lower limbs (21.21%). In the visual analog score, 63.64% of patients had a score between 5 and 8, indicating moderate severity. Duration since onset of motor symptoms and stage of PD did not significantly correlate with the presence of pain, but good correlation was noted with severity of pain.

Conclusion: Pain is a common symptom in patients with PD, but has varying characteristics with subjective differences between patients and poor correlation with disease characteristics, except for females and duration of disease.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

Saturday, September 29, 2018, 09:00-10:00 h, E-Poster designated area: E Poster Session – 05: Epilepsy: EEP1/38: Dacrystic epilepsy

Raybarman C ¹

Dacrystic epilepsy was first named by Offen et al. in 1976. Since then, dacrystic seizures have been studied and reported infrequently. The author came across a case of healthy 8-year-old boy presented with dacrystic seizures for 4 years, some of which accompanied with brief series of high-speed pedaling-like movement, which could not be arrested by manual restraint. Electroencephalography showed generalized epileptiform activity. Computed tomography of the brain was normal. He had excellent outcomes with treatment with oxcarbazepine. His seizure freedom was continued for 2 years. There was more than 4-year treatment gap in our case because of unusual semiology mimicking crying spells as behavior of a demanding child for which child care professionals was reluctant and underestimated crying as an ictal manifestation. The ictal onset zone in our case may be in the supplementary motor area (SMA), with spread to the temporal lobe or may be in the temporal lobe, with spread to the SMA. To my knowledge, this is the first report of dacrystic seizures accompanied with epileptic pedaling-like motions. On this background, the author reviewed and discussed dacrystic epilepsy, because dacrystic seizures may be more common than reported in the literature. In conclusion, not all crying spells in children are benign; some of them may be ictal crying which is treatable. Hence, the child care professionals should be aware of this.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP2/46: Clinical and electroencephalography profile in “febrile seizures in children aged between 6 and 12 years”

Bose JA ¹, Thangaraj M ¹, Rajendran A ¹

Febrile seizures are the most common epileptic disorder in children aged <5 years. In some children, febrile seizure occurs after the age of 6 years, although a well-reported phenomenon of the clinical and electroencephalogram (EEG) features is less clear.

Aim: To analyze the clinical and EEG profile in febrile seizures in children aged between 6 and 12 years.

Materials and Methods: All patients of typical and atypical febrile seizures in the age group of 6–12 years attending the Neurology Outpatient Department, Thanjavur Medical College Hospital, were included. Patients with structural lesions, central nervous system infections, and those with previous a febrile seizures were excluded. Thirty patients fulfilling the criteria were included in the study. A careful neurological examination with appropriate laboratory tests, imaging, and EEG was performed.

Results: Of 30 children, 22 were in the age group of 6–9 years and the rest in the age group of 10–12 years. Twelve girls and 18 boys were included in the study group. Twenty-two (73.3%) patients presented with generalized tonic-clonic seizures, 3 with focal seizures, and 5 with febrile status. Family history of febrile seizure was present in 40% and family history of epilepsy in 26.6% of patients. Four (13.3%) children had behavioral problem and 33% had learning difficulty. EEG was abnormal in 60% of the study population. Hyponatremia was found in 36.7% of the patients. Imaging abnormality was noted in three patients.

Conclusion: Abnormal EEG had significant positive association with learning difficulty (P < 0.05). Hyponatremia had significant association with the occurrence of febrile status.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP3/48: Novel KCNQ2 mutation presenting as refractory childhood epilepsy: A case report

Mundlamuri RC ¹, Kenchaiah R ¹, Sinha S ¹, Katragadda PK ¹, Mariappa N ¹, Bhargava GK ¹

Introduction and Aims: Early infantile epileptic encephalopathy-7 and benign familial neonatal seizures-1 are caused by heterozygous mutations in the KCNQ2 gene. Hereby, we report a novel mutation in the intron of KCNQ2 gene presenting with refractory childhood epilepsy.

Materials and Methods: Clinical phenotype, electroencephalogram (EEG), video EEG, imaging, magnetoencephalography, treatment response, and genotype characteristics of a child presented with refractory seizures were described.

Results: A 3-year-old boy born of nonconsanguineous parentage with normal birth and development presented history seizures since 2 years of age. The seizure frequency was varying from daily multiple episodes (10–20 episodes) to few days of remission. Semiology of seizure was either generalized myoclonic jerk or asymmetric tonic seizures involving both UL and tonic deviation of the eyes. Seizures used to occur in clusters. He developed hyperactivity and behavioral problems after the onset of seizures. However, he had normal cognition. His magnetic resonance (MR) imaging brain was normal. EEG showed frequent interictal discharges in the central leads. Megalencephaly showed diffuse dipoles in B/L frontoparietal temporal leads. His next-generation sequencing revealed a heterozygous 3’ splice site variation in intron 12 of the KCNQ2 gene. He showed a good response to carbamazepine, had no response to sodium valproate, and worsening of seizures with clobazam.

Discussion and Conclusion: This novel mutation found in our patient adds to the existing clinical phenotype and genotype variation related to KCNQ2 gene mutation. It emphasizes the need for clinical exome sequencing while evaluating MR negative children with refractory epilepsy to avoid presurgical evaluation.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP4/47: Efficacy and tolerability of perampanel in the treatment of epilepsy based on concomitant antiepileptic drugs

Haldar A ¹, Gupta A ¹, Kamle M ¹, Patil C ¹

Aim: Perampanel is a first-in-class AMPA receptor antagonist, approved in India for adjunctive therapy of partial onset seizure (POS).

Methods: Databases such as PubMed and Google were searched for perampanel published abstracts and articles till June 2018 were included. Pooled data of 3 POS phase III trials and real-world experience were considered for this analysis.

Results: In phase III studies, responder rate 50% for all POS with concomitant carbamazepine, valproate, lamotrigine, and levetiracetam was up to 31.3%, 38.3%, 35.6%, and 43.0%, respectively, and the same for secondarily generalized seizures was up to 51.5%, 67.4%, 68.2%, and 66.7%, respectively. Overall responder rates with concomitant enzyme-inducing antiepileptic drug (EIAED) were 27.1%, 32.4%, and 32.9% in perampanel 4, 8, and 12 mg, respectively, compared to 35.1%, 45.3%, and 54.3% with perampanel 4, 8, and 12 mg, respectively, with non-EIAED. Patients taking 1 AED at baseline had higher responder rate compared to 3 AEDs at baseline. The overall incidence of TEAE was similar regardless of the presence of EIAEDs or number of AEDs at baseline. The rate of hostility and aggression observed among patients was irrespective of receiving levetiracetam/topiramate/levetiracetam + topiramate compared to those not. Real-world data suggest that responders to perampanel on concomitant non-EIAEDs versus patients on EIAEDs were 29.1% and 23.7%, respectively. No differences were observed in responder rates between patients on non-EIAEDs versus EIAEDS in groups <6 mg/day, 6 mg/day, and >6 mg/day. There were no differences in AE during 12 months of perampanel use in patients taking SCBs and not receiving SCBs. There were no differences in the rate of overall AEs or rate of psychiatric AEs in patients receiving or not receiving levetiracetam.

Conclusion: Perampanel with novel MOA can be combined rationally with any AED. Perampanel shows efficacy and safety in combination with commonly prescribed AEDs as well as irrespective of the presence or absence of EIAEDs.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP5/64: Is immunosuppression therapy helpful in new-onset refractory status epilepticus/febrile infection-related epilepsy syndrome?

Choudary GV ¹

Status epilepticus (SE) represents a true neurologic emergency that requires immediate attention and treatment to stop seizure activity and evaluation to treat potentially treatable cause. Here, we studied ten patients who presented with SE from March 2016 to March 2017. Of them, five patients had history of genetic generalized epilepsy and had drug withdrawal followed by SE, which were well controlled with medication. However, one patient who presented with SE with underlying HSE (right side) causing mass effect and was cured by decompressive craniectomy along with antiepileptic drugs. Three patients (two females and one male) between 12 and 17 years of age group in whom no cause was found and the possibility of febrile infection-related epilepsy syndrome/new-onset refractory status epilepticus (NORSE) were considered. By routine diagnostic protocol among three patients with NORSE stayed longtime in hospital and recovered well. Had immunosuppressive therapy a pivotal role? Two patients (females) improved well but one (male) patient had occasional partial seizures with some cognitive deficit.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP6/85: Knowledge, attitude, practice, and drug utilization pattern study in North Indian epilepsy patients

Sethi A ¹, Mishra V ¹, Joshi D ¹, Chaurasia R ¹, Pathak A ¹, Rath S ¹, Mishra A ¹, Singh V ¹

Objective: To study knowledge, attitude, practice, and drug utilization pattern in North Indian epilepsy patients.

Materials and Methods: One-hundred patients were administered a questionnaire of 25 questions in English/Hindi whichever the patient was proficient with. Responses were recorded in yes/no/don’t know. Drug utilization pattern was analyzed from the patient's case sheets.

Results: Approximately 30% were young population (<30 years), 66% were male, and 34% were female. Literacy rate was low; 72% of patients studied up to school level. Good knowledge was seen as 94% patients had heard of epilepsy; 86% thought that it was treatable by modern drugs. Poor knowledge was seen as 24% believed that it was contagious. Positive attitude was seen; 72% thought that epileptics can be studied, 84% felt that they can work, 92% felt that they can marry. Negative attitude was seen as 58% felt discriminated by schoolmates and 40% by their teachers; 52% did not want to disclose epileptic condition of their daughters before marriage. Good practices were seen; 98% knew that epilepsy patients should be taken to hospital. The most common seizures were generalized (40%), followed by focal (30%) and then focal with secondary generalization (26%). 4% had juvenile myoclonic epilepsy. Common drugs used were levetiracetam (50%), valproate (40%), clobazam (28%), oxcarbazepine (16%), carbamazepine (12%), and phenytoin (12%). 96% patients were controlled with antiepileptic drugs.

Conclusion: The study showed that epilepsy patients in North India had good knowledge about epilepsy, but negative attitude and incorrect practices are still prevalent. There is a need to educate people about epilepsy. Most of the patients were controlled with antiepileptic drugs.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP7/207: Doose syndrome: A case report

Reddy MVH ¹, Reddy HRK ¹

A 7-year-old male child presented with recurrent forward falls, with a nonhealing forehead wound. The falls were associated with brief period of unresponsiveness and eye blinking. Such episodes were 15–20/day. Earlier treatment with carbamazepine only increased the number of falls. Imaging and electroencephalographic findings were suggestive of Doose syndrome. He was started on divalproex sodium, topiramate, and ketogenic diet.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP8/208: Electrocardiography and vascular risk profile abnormality in people with epilepsy on long-term antiepileptic drugs

Muppalla LL ¹, Garuda BR ¹, Seepana G ¹

Aims and Objectives: People with chronic epilepsy who are on long-term antiepileptic drugs (AEDs) may be associated with various metabolic abnormalities and sudden cardiac arrest, so the aim of this study is to investigate whether the changes of electrocardiography and metabolic vascular profile abnormalities are associated or not in people with epilepsy on long-term AEDs.

Methods: This is a case–control study, from May 2016 to December 2017, conducted in Andhra Medical College, Visakhapatnam.

Results: There were 72 cases and 59 controls. Among the studied vascular risk parameters, total cholesterol (187.4 ± 38.4 mg/dl), triglycerides (147 ± 28 mg/dl), low-density lipoprotein cholesterol (116.15 ± 38 mg/dl), and homocysteine (13.19 ± 6.3 µmol/dl) were significantly noted with carbamazepine and its combination followed by phenytoin (P < 0.05). The studied electrocardiogram (ECG) parameters were within the normal limit in both the cases and controls, but the mean values of PR-interval (199 ± 0.03 ms) and QT-interval (409 ± 19.7 ms) were higher side normal in cases with significant P value. The mean values of ST segment were normal in both the groups. J-wave like recording is seen in 18.6% of controls and 8.3% of cases. Brugada-type ECG recording is seen in 0.7% of cases and 1.04% of controls.

Conclusion: Vascular profile and ECG abnormalities were prevalent among epileptic patients who are on long-term AED. Routine evaluation of chronic epileptic patients with long-term AEDs must warrant the addressing of these complications and prevent the unwanted sudden cardiac arrest.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP9/304: Psychosocial functioning is associated with adjustment to seizures and requirement for more antiepileptic drugs in persons with epilepsy

Paila R ¹

Results: A total of 324 consecutive patients with epilepsy (PWE) were included, and the mean age of the study population was 25.90 ± 6.22 years with 176 (54.3%) women. The average age at onset of epilepsy was 14.29 ± 7.65 years. Whereas, 121 (37.3%) PWE did not have psychosocial issues, 60 (18.5%) had depression, 73 (22.5%) had anxiety, and 70 (21.6%) had both anxiety and depression. Among four groups, there were no significant differences between groups for age, gender, age of onset, literacy, socioeconomic class, and marital status. The mean number of antiepileptic drugs (AEDs) was 3.26 ± 1.23 and PWE with depression need more number of AEDs than normal PWE (2.87 ± 0.97 vs. 4.10 ± 1.47; P < 0.05) and those with anxiety (2.72 ± 0.83 vs. 4.10 ± 1.47; P < 0.05). Emotional adjustment was significantly better in normal PWE (14.36 ± 2.29) than PWE with depression (16.43 ± 2.67) and PWE with anxiety (17.41 ± 2.04). Interpersonal adjustment was better in normal PWE than those with depression (3.64 ± 1.00 vs. 4.22 ± 0.97; P < 0.05). Similarly, vocational adjustment (4.50 ± 1.71 vs. 3.45 ± 1.64; P < 0.05) and financial adjustment (1.13 ± 0.36 vs. 1.43 ± 0.64; P < 0.05) were better in normal PWE than those with depression. Importantly, adjustment to seizure was higher in normal PWE (11.00 ± 1.87 vs. 10.15 ± 1.97; P < 0.05).

Conclusion: Psychosocial maladjustment is observed in two-third of PWE, thus making adjustment to seizure difficult in them and hence requiring higher number of AEDs.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP10/305: Clinical features and role of psychological counseling in nonepileptic attack disorders

Kumar BNM ¹, Jayalakshmi S ¹, Somayajula S ¹, Vooturi S ¹

Purpose: To evaluate clinical features and neuropsychology in patients with nonepileptic attack disorders (NEADs).

Methods: Data of 98 consecutive patients identified as NEAD on video electroencephalogram were collected. Patients were followed up to 12 months over telephone or at outpatient epilepsy clinic.

Results: The average age of the study population was 22.74 ± 12.6 years, with 74 (75.5%) women. Auras were reported in 41 (41.83%) patients and NEAD was similar to habitual events in 79 (80.6%) patients. Onset was gradual in 33 (33.7%) patients, eyes were open throughout the event in 30 (30.6%) patients and limb movements were observed in 39 (39.79%) patients. Whereas, on suggestion, events were observed in 51 (52.0%) patients and postictal confusion was seen in 4 (4.1%) patients. Seventy-seven (78.6%) patients had NEAD, and the remaining 21 (21.4%) had both seizures and NEAD. On neuropsychological assessment, the most common diagnosis was dissociative disorder in 77 (78.6%) patients, followed by major depression in 10 (10.2%) and anxiety in 8 (8.1%) patients. The remaining 3 (3.1%) patients had stress disorders. Fifty-six (57.1%) patients received antiepileptic drugs (AEDs). All the patients received psychological therapy. Eight (8.1%) patients were lost to follow-up. Six (6.1%) patients continue to get the attacks and the remaining 84 (85.7%) patients were free of NEAD.

Conclusion: Establishing adequate psychological counseling programs at outpatient epilepsy clinics could eventually improve the outcome in patients with NEAD.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP11/308: Hemiconvulsion–hemiplegia epilepsy: A case report

Bondada RA ¹, Jeyaraj M ¹, Velayutham S ¹, Sowmini ¹, Arunan S ¹

Introduction: Hemiconvulsion–hemiplegia epilepsy (HHE) is an uncommon outcome of prolonged febrile focal status epilepticus in childhood. It is followed by hemiplegia in the side of convulsions and later could develop drug refractory seizures. It is accompanied by radiological evidence of acute cytotoxic edema in the affected hemisphere followed by chronic atrophy of that hemisphere.

Case Discussion: A 7-year-old male child with normal birth and developmental milestones at 2½ years of age had high-grade fever followed by right focal status epilepticus. After 2 days, he developed right hemiplegia with aphasia. Magnetic resonance imaging (MRI) brain shows edema of the entire left hemisphere with diffusion restriction with normal magnetic resonance angiography. He was treated with antiepileptics, antiedema measures, and steroids. Seizures were controlled after 1 week. Subsequent antinuclear antibody and antineutrophil cytoplasmic antibody profile were normal. The patient was discharged after 1 month with residual hemiparesis. Repeat MRI brain after 2 years showed diffuse left cerebral atrophy with volume loss of subcortical structures. The patient was seizure free for 4½ years. The patient presented with drug refractory complex partial seizures for 2 months. He was treated with newer antiepileptics and seizures got controlled. With occupational and speech therapy, he was able to walk, speak, and communicate. The final diagnosis of HHE was made based on the clinical and radiological findings.

Conclusion: HHE is rare but serious disorder in pediatric population. It can greatly impact the quality of life for patients. Morbidity of HHE syndrome may be reduced by rapid resolution of febrile seizures, aggressive, and early treatment with antiedema measures. HHE is rare cause of drug refractory seizures which might need surgery if not controlled medically.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP12/369: Cognitive and neuropsychological assessment in people with epilepsy: A case–control study

Kesireddy S ¹, Garuda BR ¹, Seepana G ¹, Kumar ST ¹

Aims and Objectives: To study the incidence of cognitive and neuropsychological aspects in people with epilepsy attending a tertiary care hospital of North coastal Andhra Pradesh.

Materials and Methods: This is a case–control study conducted from April 2017 to May 2018 on 52 patients with epilepsy from the Department of Neurology, KGH, Visakhapatnam. Patients with acute symptomatic seizures, major psychiatric illness, major systemic illness, progressive neurological condition, and pregnancy with epilepsy were excluded from the study. Mini–Mental Status Examination (MMSE), Montreal Cognitive Assessment (MOCA), Epworth Sleepiness Scale, MINI Neuropsychiatric Inventory were the scales used.

Results: In epilepsy patients (n = 52), majority were male (57.6%). The mean age of symptom onset was 17 years and the mean duration was 11.7 years. Juvenile myoclonic epilepsy (JME) is seen in 21%, temporal lobe epilepsy (TLE) in 28%, focal seizures with bilateral spread in 8%, and other seizures types with generalized tonic-clonic seizures as predominant seizure type in the rest. Patients with TLE had low scores with MMSE and MOCA and high scores on Epworth Sleepiness Scale. We found a significant association between the long duration of TLE and poor scores with MMSE and MOCA–P.

Conclusions: In our study, the incidence of cognitive dysfunction is seen more with TLE patients. JME patients performed well in the cognitive scales. This is one of the cross-sectional studies to show the incidence of cognitive and psychiatric abnormalities in different types of epilepsy, which needs to be further validated in large population studies.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP13/371: A long-term follow-up of epilepsy patients after a single neurologist visit: A case–control study

Prajapati C ¹, Singh M ¹, Srivastava MVP ¹, Goyal V ¹, Shukla G ¹, Vishnu VY ¹, Sreenivas V ¹

Objective: To determine the long-term effectiveness of an epilepsy outreach clinic by establishing antiepileptic drug (AED) continuation rates after a single neurologist consultation.

Methods: A retrospective case–control study was conducted from March 2016 to July 2018. Cases were consecutive epilepsy patients from two outreach clinics conducted in 2014 while epilepsy patients registered in the same year at AIIMS Outpatient Department (OPD) were enlisted as controls. The primary outcome was to determine if patients were still taking AEDs. Cases were contacted telephonically to determine the primary outcome. For controls, hospital records were reviewed and phone calls made to those patients who had not followed up for more than a year.

Results: A total of 304 cases and 372 controls were enrolled. During telephonic follow-up, 110 cases responded, of them 82 (75%) complained about the medicines advised. Among controls, 166 were regularly followed up in OPD. Of 205 cases who were not followed up, 64 responded to telephonic contact and 45 of them complained about the medicines advised. As 141 patients lost to follow-up in control group and were nonrespondent to telephonic contact, it was inferred that 211 (92%) of remaining 231 patients complained about medicines.

Significance: In resource-poor countries of the world, mobile epilepsy clinics can be an option for delivering epilepsy care. However, innovative follow-up mechanisms need to be developed for ensuring medication compliance and thus better epilepsy management.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP14/372: A study of the treatment gap in epilepsy

Bhoi KK ¹, Jaiswal D ¹

Purpose: Treatment gap (TG) is defined as the percentage of individuals with active epilepsy who at the time are not receiving appropriate treatment. High TG is a burden to the society in underdeveloped and developing countries. We aimed to study the TG and factors responsible for it.

Methods: We studied 504 patients with epilepsy attending Neurology Outpatient Department, Shri Balaji Hospital, Raipur, from December 2016 to December 2017. The study included the locality of the patient, their awareness and attitude about epilepsy, literacy rate, social stigma prevailing in that community, use of traditional remedies, and compliance to antiepileptic drug (AED). Other aspects such as availability of health-care service and cost of treatment were assessed.

Results: It included 360 (77.4%) patients from rural and 144 (22.6%) patients from an urban population, male 330 (65%), female 174 (35%), and 400 (80%) literates. One hundred and fifty (30%) patients had a lack of proper knowledge of epilepsy. One hundred (20%) patients were on traditional remedies, and 130 (26%) had delayed initiation. One hundred and fifty (30%) patients had poor compliance to AEDs. The significant difference in the TG in rural–urban patients was due to lack of awareness and attitude, social stigma, low literacy rate, unavailability of health-care service, and high cost of treatment.

Conclusion: Epilepsy awareness is needed. Free or subsidized AEDs should be provided. Epilepsy should be recognized as a public health problem.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP15/AWPE1: Counseling by an Indian neurologists for various risks involved in epilepsy including sudden unexpected death in epilepsy

Diwan AG ¹, Pawar V ¹, Diwan GA ¹

Objective: (1) To survey the attitudes toward counseling about various risks related to epilepsy with special emphasis on sudden unexpected death in epilepsy (SUDEP) and (2) to determine factors responsible for discussing and not discussing SUDEP.

Methods: Web-based questionnaires (www.surveymonkey.com) were sent via e-mail to approximately 1200 neurologists through the President, Indian Academy of Neurologists, in May 2016 and a reminder for the same was e-mailed in July 2016. Questions were asked regarding experience of SUDEP among treated epileptic patients: when and how often they discussed SUDEP, and its risk factors, and reasons for not discussing SUDEP.

Results: In total, 100 surveys were completed (76% adult neurologists, 15% neurologists with additional epilepsy training, 5% pediatric neurologists, and 4% nonneurologists [intensivists/physicians]). A minority of 6% of patients reported that they counseled all of their patients (>90%) on SUDEP, 8% counseled most of their patients (50%–90%), 20% sometimes (10%–49%), 50% rarely (1%–9%), and 16% reported not counseling about SUDEP at all. In contrast, 77% of patients reported that they counseled all patients (>90%) about risks in daily life activities. Refractory epilepsy (70%) and noncompliance for the medications (62%) were the most common reasons to counsel for SUDEP.

Conclusion: Very few neurologists discussed premature mortality due to SUDEP in their epileptic patients. Neurologists with special training in epilepsy were significantly higher in proportion to discuss SUDEP. At the same time, majority of these responders counseled for prevention of injuries in daily life, e.g., falling from heights, working in hostile environment. Discomfort in discussing premature death due to SUDEP is related to many factors such as lack of time, no definite preventive measures, fear of emotional reaction, or adding anxiety to patients with poor benefit to risk ratio.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP16/AWPE3: New-onset interictal headache in people living with epilepsy: a case–control study

Mohan Rao JS ¹, Sahu J ¹, Nair PP ¹, Aghoram R ¹, Wadwekar V ¹

Introduction: Antiepileptic drugs (AEDs) are the mainstay of treatment for people living with epilepsy (PWE). A headache as a side effect of AEDs is easily overlooked in everyday clinical practice. In our personal experience, headache is a frequent complaint in PWE receiving AEDs, carbamazepine (CBZ) in particular. There is hardly any systematic study on AED-associated headache other than a few case reports and mention in the package inserts. The objective of our study was to assess the association of new-onset interictal headache in PWE with CBZ compared to other AEDs and to study other factors such as clinical and demographic factors associated with it.

Materials and Methods: Two thousand PWE on AEDs were screened, of which 165 having headache were identified. Eight PWE were excluded due to comorbid illnesses such as significant hypertension (4 patients), sinusitis (2 patients), and tuberculous granuloma (2 patients) which could potentially contribute to a headache. Finally, 157 were recruited for the study. One hundred and fifty-seven age- and sex-matched PWE on AEDs, without a headache, were recruited as controls. Headache questionnaire based on the ICHD-II criteria was used to classify primary headaches such as migraine and tension-type headache. Demographic factors, seizure characteristics, type and dose of AEDs, and investigation findings such as electroencephalogram (EEG) and magnetic resonance imaging/computed tomography were recorded from the case file and by interview.

Results: A total of 157 (7.8%) of 2000 PWE had some form of new-onset interictal headache. Among PWE with headache, the majority (88 [56.1%]) were on CBZ compared to other AEDs, but the same was noted among controls also. Seventy-one (45%) persons with headache had the migrainous characteristic, of whom 23 (14.6%) had migraine without aura. There was a significant difference in seizure frequency between the persons with the headache and without a headache. PWE who had a frequency of seizure of <1/month had a higher frequency of headache. EEG abnormalities such as epileptic form discharges and focal slowing were more common in PWE without headache compared to those with headache. In multivariate analysis, lower seizure frequency and lower EEG abnormalities were found to be independent predictors of a headache.

Conclusions: CBZ was not found to have any predilection for a headache compared to other AEDs. This study showed that an interictal headache is a significant issue in PWE, nonmigrainous headache more common than migrainous type. A lower frequency of seizures and EEG abnormalities in PWE with a headache may suggest that seizures contribute less to the development of headache and further may support the hypothesis that AEDs may be significant contributing factors for the development of headache.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP17/AWPE5: Variations in electroencephalography with mobile phone usage in medical students

Tandan R ¹, Parmar K ¹, Kumar N ¹, Garg RK ¹

Background: Electromagnetic fields emitted by cellular telephones may cause neurological ill effects such as cognitive dysfunction, emotional instability, and even brain tumors. Slowing of brain activity on electroencephalography (EEG) has been shown. However, these findings need further validation.

Objectives: The study was conducted in the Department of XXX on the students of the XXX, from August 2017 to October 2017 to analyze EEG changes and adverse effects experienced following cellphone use.

Materials and Methods: Twenty-one students underwent video-EEG recording before and after application of Samsung GT-56312 dual-SIM smart phone in switched off, switched on and switched on mode with conversation. Average EEG frequencies and amplitudes were calculated for different brain regions. Chi-square tests and Wilcoxon signed-ranks test were used for comparison between variables.

Results: The mean age of 7 (33.3%) males and 14 (66.7%) females was 20.76 ± 1.48 years. The average EEG frequencies following mobile phones application with conversation were higher and the amplitudes lower than baseline values. Frequencies were greater on the right side. Frontal slow waves were detected in 38.1%, parietal in 33.3%, occipital and temporal in 19.1%, and generalized slow waves in 9.5% of students. During experiment, 23.8% of students experienced headache, 19% experienced irritation, and 9.5% felt drowsy. Headache and loss of concentration (33.3%), sleep disturbances (28.6%), and fatigue (19%) were frequent in daily life.

Discussion: Experimental application of mobile phones may lead to some EEG changes and certain ill effects on the well-being.

Conclusions: Prolonged use of these gadgets warrants caution.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP18/AWPE7: To evaluate cognitive dysfunction in patients with juvenile myoclonic epilepsy

Chawla T ¹, Chaudhry N ¹, Puri V ¹

Background: Juvenile myoclonic epilepsy (JME) is considered as benign epilepsy, though sensitive imaging techniques and autopsy studies have provided evidence of multiregional cerebral dysfunction, involving primarily the frontal lobes and the thalamus in JME. We attempted to study the cognitive dysfunction in patients with JME and its correlation with various clinical and electrographic features.

Methods: Fifty JME patients and 50 age- and sex-matched healthy controls above 12 years of age were subjected to a set of cognitive tasks including Mini–Mental Status examination (MMSE), frontal assessment battery (FAB), executive interview (EXIT), PGI memory scale, clock-drawing test, cube-copying test, and Nahor–Benson test. The performance of cases and controls on the various tests was compared. The results obtained for the cases were also correlated to the age of onset, duration of epilepsy, monotherapy/polytherapy, electroencephalographic features, and status of seizure control.

Results: JME patients performed significantly worse on MMSE (P = 0.001), PGI memory scale (P = 0.001), FAB (P = 0.001), EXIT (P = 0.001), clock-drawing test (P = 0.02), and cube-copying test (P = 0.001) when compared to the controls. JME patients had impaired attention, verbal fluency, design fluency, verbal memory, visual memory, conceptualization, set shifting, mental flexibility, response inhibition, and visuospatial functions.

Conclusion: JME patients demonstrate cognitive impairment in the frontal and parietal lobe dysfunction. Hence, detailed higher mental function tests supplemented by functional neuroimaging studies should be done in JME patients for a comprehensive management of this group of apparently benign epilepsy.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP19/AWPE9: Comparison of clinical, imaging, and electrophysiological features in focal cortical dysplasia type 1 and type 2

Bapu PS ¹, Jayalakshmi S ¹

Objective: To characterize clinical, imaging, and electrophysiological features of type 1 and type 2 focal cortical dysplasia (FCD) and their effect on long-term outcome.

Methods: One hundred ninety-two consecutive patients with a diagnosis of FCD were included to the analysis of factors differentiating FCD type 1 and type 2 groups and their effect on long-term outcome.

Results: Magnetic resonance imaging (MRI) showed clear lesion in 80.7% of FCD type 1 patients and 92.8% of FCD type 2 patients (P = 0.021). Regional interictal electroencephalography (EEG) pattern observed in 43.1% of FCD type 1 patients and 57.8% of FCD type 2 patients (P = 0.055). Positron emission tomography (PET) showed hypometabolism in 81.7% of FCD type 1 and 91.6% of FCD type 2 patients (P = 0.060). PET showed hypermetabolism in 15.6% of FCD type 1 and 6.0% of FCD type 2 patients (P = 0.042). Seizure freedom was achieved in 54.1% of FCD type 1 patients and 74.7% of FCD type 2 patients (P = 0.004). Among 27 patients with subtle MRI, ¹⁸F-fluorodeoxyglucose-PET helped in localizing the lesion in 19 and ictal single-photon emission computed tomography in four more. The predictors for seizure freedom were clear lesion on MRI, regional interictal EEG pattern, and completeness of resection.

Conclusion: Surgical intervention in carefully selected patients may facilitate favorable seizure outcome leading to better quality of life. FCD type 1 group present with early onset of seizures, subtle MRI lesion, hypermetabolism on PET, and poor surgical outcome. Type 2 FCD groups present with late onset of seizures, clear MRI lesion, regional interictal EEG pattern, hypometabolism on PET, and good surgical outcome.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

EEP20/AWPE10: Social cognition abilities in patients with chronic temporal lobe epilepsy

Manjeera K ¹, Suryaprabha ¹, Hemalatha ¹, Rukmini ¹, Borgohain R ¹

Background: Social cognition is a high-level cognitive function that includes all the processes that are used to understand and store information about the interactions with other people in a social context. Perception of social signals pertaining to others’ mental states is essential to act appropriately in that social context. Low performances in mentalizing abilities on different Theory of Mind tasks have been reported in patients with temporal lobe epilepsy (TLE).

Aim: To investigate the social cognition abilities in patients with TLE.

Materials and Methods: Thirty-two consecutive patients with TLE (19 males; mean age = 27 ± 11.7 years) and 30 controls (mean age 25.24 ± 5.9 years) are taken into the study. Demographic profile, age at onset, ictal semiology, seizure frequency, and response to treatment are studied. All underwent prolonged video-electroencephalography monitoring and magnetic resonance imaging brain to locate the side of lesion. Affective and cognitive aspects of faux pas in the form of 20 stories are assessed in both patients and controls.

Results: Patients with TLE showed significantly lower performances on both social, cognition tasks when compared to controls.

Conclusion: Patients with TLE are found to be deficient in perceiving other's feelings in social context that may impair daily living.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

Saturday, September 29, 2018, 13:00-14:00 h, E-Poster designated area: E Poster Session– 06: Stroke: SEP21/285: Comparison of risk factors and severity of anterior circulation stroke versus posterior circulation stroke

Aravind S ¹, Reddy PV ¹, Chandramouleeswaran V ¹, Lakshminarasimhan R ¹, Kannan V ¹

Background: Anterior circulation (AC) strokes account for about 70%–80% of all strokes while the rest 20%–30% occur in posterior circulation (PC). We sought to explore the differences between AC and PC strokes concerning their risk factors and severity.

Methods: This is an ongoing cross-sectional study done in the Institute of Neurology, Madras Medical College, starting from January 2018. Strokes in AC and PC were diagnosed by clinical and neuroimaging findings.

Results: The analysis included 326 patients, of which 175 had AC stroke and 151 had PC stroke. Among AC strokes, 58.2% (n = 102) were male, while 62.2% (n = 94) were male in PC stroke and the average age was 67.2 years and 63.5 years in AC and PC, respectively. Hypertension and diabetes mellitus were present in 45.7% and 43.4% of AC strokes and 50.4% and 41% of PC strokes, respectively. Hyperlipidemia and hyperhomocysteinemia were present in 34.2% and 12.9% of AC strokes and 29.8% and 6.4% of PC strokes, respectively. Alcoholism and smoking were associated with 34% and 30.2% of AC strokes and 28% and 34.4% of PC strokes, respectively. Atrial fibrillation was present in 12.5% AC strokes and 7.2% PC strokes. The mean National Institutes of Health Stroke Scale (NIHSS) and modified Rankin Scale (mRS) score at presentation was 10.4 and 3.8 in AC strokes and 7.2 and 3.2 in PC strokes, respectively.

Conclusion: Risk factors including hypertension and smoking were more common in PC stroke while diabetes mellitus, hyperlipidemia, hyperhomocysteinemia, alcoholism, and atrial fibrillation were more common in AC stroke. AC strokes were more severe as evidenced by high NIHSS and MRS score.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP22/286: A case report of combined central retinal artery with central retinal vein occlusion and bilateral carotid artery disease in a patient with protein C deficiency

Chakravarthi D ¹, Chandramouleeswaran V ¹, Sarala G ¹, Ranganathan LN ¹, Kannan ¹

Introduction: A combination of central retinal artery (CRAO) and central retinal vein occlusion (CRVO) is a rare entity of retinal vascular disease that is associated with poor prognosis and can cause sudden visual loss. Here, we report a rare case of combined CRAO with CRVO and associated bilateral carotid artery disease in a patient with protein C deficiency. Only few cases of arterial thrombosis have been reported previously due to protein C deficiency.

Case Report: A 45-year-old male patient presented with sudden-onset visual loss in his right eye with visual acuity of only perception of hand movement. Ophthalmic evaluation showed combined CRAO with CRVO. Subsequently, he developed multiple episodes of transient ischemic attack in the form of weakness involving left upper limb and lower limb, for which he was evaluated and found to have bilateral carotid artery disease with reformatted flow from circle of Willis. Further investigations showed decreased protein C assay. All other hypercoagulable workups were negative. He was started on anticoagulation and aspirin demonstrating substantial improvement in the neurological status.

Conclusion: combined CRAO and CRVO is a disease which is associated with devastating visual outcome and is not often an isolated ocular event. A detailed investigation is always needed mainly in younger patients as ocular pathology may be followed by severe cerebrovascular and cardiovascular events.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP23/288: Case report of cerebral hyperperfusion syndrome

Reddy MVH ¹, Sangani HR ¹

Introduction: Cerebral hyperperfusion syndrome (CHS) is known to be a rare but devastating complication of carotid artery revascularization. CHS is defined as an increase in cerebral blood flow of over 100% compared to baseline, in a patient with new-onset headache ipsilateral to the carotid revascularization, focal neurological deficits, and seizures.

Clinical Details: A 67-year-old male patient admitted on June 23, 2017, with multiple episodes of right upper and lower limb focal seizures followed by weakness of the right side of the body. A history of recurrent transient ischemic attack in the form of slurring of speech lasted 10–15 min. He underwent left carotid endarterectomy on June 13, 2017. Magnetic resonance imaging brain with perfusion scan (ASL) s/o CHS. The patient was managed with antiepileptics, ventilatory support, improved gradually, and discharged in stable condition.

Discussion: CHS can develop at any time from immediately after surgery to up to a month later, but most patients develop symptoms within the first few days. The main reason to perform carcinoembryonic antigen (CEA) is removal of the source of emboli originating from carotid plaques. However, in a small subset of patients, cerebral hyperperfusion or reperfusion causes postoperative neurological dysfunction.

Conclusion: Cerebral hyperperfusion or reperfusion syndrome is a rare, but a serious complication following CEA.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP24/290: Our experience with stroke thrombolysis in a tertiary care public hospital

Somkuwar A ¹

Objective: A report of thrombolysis in acute ischemic stroke in our hospital from March 2016 to June 2018.

Methodology: We present the data of patients undergoing thrombolysis for acute ischemic stroke from March 2016 to June 2018 in a tertiary public hospital. Thirty-three patients were thrombolyzed, 31 with alteplase, and 2 with tenecteplase.

Results: The average age of the patient was 55.90 years. There were 24 (72.72%) males and 9 (27.27%) females. The main risk factors were hypertension (54.54%), diabetes mellitus (DM; 18.18%), ischemic heart disease (IHD; 9.67%), and atrial fibrillation (9.67%). Of 33 cases, 31 (93.93%) cases were due to anterior circulation stroke. The mean National Institutes of Health Stroke Scale (NIHSS) on admission was 10 and on discharge was 5. The mean door-to-needle time was 1 h, 1.8 min despite logistic hurdles associated with a public hospital. Two (6.06%) patients had hemorrhagic transformation type 2. Two (6.06%) patients had expired; the causes were symptomatic hemorrhage and cardiac complication. Seven (21.21%) patients required post-thrombolysis Intensive Care Unit (ICU) care. The mean duration of hospital stay was 13 days. Nearly 29 (87.87%) patients paid for tPA while three (9.67%) patients did not pay due to financial problems. The tPA was sanctioned from hospital for 1 (3.03%) patient.

Conclusions: NIHSS improved by mean of 5 in thrombolyzed patients. The main risk factors were hypertension, DM, IHD, and atrial fibrillation. Implementation of stroke thrombolysis protocol helped achieve door-to-needle time of an hour. Furthermore, close monitoring of stable postthrombolysis patients in wards may yield similar outcomes if ICU cares not available in a hospital.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP25/292: Transcranial Doppler in sickle cell anemia at Chhattisgarh

Sharma S ¹, Sharma P ¹, Goyal V ¹, Jain P ¹

Background: Sickle cell disease is widely prevalent in the state of Chhattisgarh. It is observed that the prevalence of sickle cell anemia (SCA) was 2.1% and sickle cell trait was 10% among different tribes. Stroke is a devastating complication of SCA, particularly during childhood. The use of transcranial Doppler (TCD) identifies children with SCA who are at high risk for stroke.

Materials and Methods: We screened 19 children with a symptomatic SCA between the ages of 2 and 16 years and stratified for risk of stroke on the basis of elevated cerebral blood flow as measured by TCD screening tests. The transtemporal head diameter is measured and recorded. The middle cerebral artery (MCA) is tracked to as shallow a depth as possible (depth <40 mm). The velocity scale is set to 200–250 cm/s. TCD signal is optimized and recorded at proximal MCA near to its origin. Maximum velocity in an MCA was classified as low cerebral blood flow velocity (CBFV) <70, normal CBFV <170, low conditional between 170 and 184, high conditional between 185 and 199, and abnormal between 200 and 219 with imminent risk of stroke.

Results: The average left MCA mean velocity was 68.047 that of right MCA 58.047.

Conclusion: None of asymptomatic child with SCA has abnormal TCD as defined by mean velocity <170, consistent with the low prevalence of stroke in Indian subset of SCA.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP26/294: Case report of primary angiitis of the central nervous system

Dasari V ¹, Hari R ¹

Introduction: Primary angiitis of the central nervous system (PACNS) is a rare disorder resulting in inflammation and destruction of CNS vessels without evidence of vasculitis outside the CNS.

Clinical Details: 45/F with complaints of headache from 1 month, facial deviation from one day. Magnetic resonance imaging (MRI) brain (P/C)-Findings s/o Granulomatous likely Tuberculosis, less likely differential of vasculitis. The patient was started on ATT and discharged. The patient was readmitted on August 24, 2017, with headache from 1 week and left focal seizures with generalization. MRI brain-Acute infarct in right dorsomedial thalamus possibly vasculitic infarct. Focal closely placed short segmental mildly stenotic areas causing mild beading in the horizontal segment of right middle cerebral and left vertebral arteries (Atheromatous/? Vasculitic etiology). Biopsy of right temporal dura s/o vasculitis. The patient started on steroids and cyclophosphamide pulse therapy. Her neurological deficits improved and the patient discharged in stable condition.

Discussion: Among mimics of PACNS, exclusion of infectious arteritis is especially important because many infections are treatable with specific drugs and empirical immunosuppression can have disastrous consequences. Tuberculosis is an important mimic of PACNS and should be excluded by the use of appropriate microbiological studies.

Conclusion: PACNS is a rare disease, but has been increasingly recognized in recent years. Prompt diagnosis either by biopsy of CNS tissues or by angiography with rigorous exclusions is essential.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP27/306: The correlation of vascular endothelial growth factor-A and brain-derived neurotrophic factor levels with early neurological deterioration in acute ischemic stroke patients

Praksh D ¹, Kulshrestha M ¹, Kulshrestha D ¹, Thacker A ¹, Singh A ¹, Maurya P ¹, Tiwari V ¹

Introduction: After stroke, survivors remain with degree of disability. Predicting outcome at admission could improve the patient's condition through prevention and rehabilitation. The study aimed to find biomarkers of survivors whose concentration at admission can give insight into patient outcome.

Methodology: AIS within 3 days of onset confirmed by CT/MRI were included. The study was conducted in Dr. Ram Manohar Lohia Institute of Medical Sciences, Lucknow. Serum levels of vascular endothelial growth factor (VEGF)-A and brain-derived neurotrophic factor (BDNF) were quantified. The National Institutes of Health Stroke Scale (NIHSS) scores were taken at the time of admission and after 72 h. Early neurological deterioration (END) was taken as an increase in the NIHSS score ≥2 points in the first 72 h after admission.

Results: Fifty-eight AIS patients were included in study. 77.6% (n = 45) were male and 22.4% were female. 50% (n = 28) were hypertension. 29.39 (n = 17) were type 2 diabetes mellitus, 25.5% (n = 09) were alcoholic, and 10.3% (n = 6) were smoker. No patient had history of stroke or cardiac failure, renal failure, hepatic failure, and sepsis. Stroke is secondary to chronic infections or head trauma or venous infarcts. END was noted in 17.2% (n = 10) of patients. VEGF and BDNF levels were significantly lower (P < 0.05) in patients with END in comparison to without END. These showed that higher concentration at admission was associated with a worse neurological outcome.

Conclusion: Strong evidence was found for VEGF and BDNF. All of these showed the same trend: a lower concentration at admission is associated with a good outcome.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP28/315: A case series of malignant middle cerebral artery infarction

Meduri PC ¹, Shankar V ¹, Sundar S ¹

Introduction: “Malignant middle cerebral artery (MCA) infarction” is the term used to describe rapid neurological deterioration due to the effects of space-occupying cerebral edema following MCA territory stroke involving >50% area of MCA territory. Treatment includes antiedema measures and surgical decompression to relieve pressure effects.

Aims and Objectives: To study the clinical profile, risk factors, and outcome of patients with malignant MCA infarct.

Materials and Methods: It is a retrospective study for the duration of 3 years in patients with malignant MCA infarct in tertiary hospital.

Results: Thirty patients were included in our study. The age range is from 38 to 70 years. Nineteen were male and 11 were female. Hemisphere involved right (14) and left (16). Risk factors are diabetes (12), systemic hypertension (15), dyslipidemia (24), coronary artery disease (10), and atrial fibrillation (5). The Glasgow Coma Scale on admission is from 3 to 13. Of the 13 patients conservatively managed, death occurred in 10 (76%) patients. Death occurred from day 2 to 14 days with mean of 7^th day of admission and three patients recovered with severe deficits at discharge. Of 17 patients who were operated, 12 were taken within 48 h with mortality in 3 (25%) and 5 were taken after 48 h with mortality in 3 (60%). Stay of hospital in operated patients ranged from 10 days to 30 days.

Conclusion: Patients undergoing early decompression have good outcome and decreased mortality.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP29/321: Hemichorea in a patient with recent ischemic stroke

Vasagam P ¹

The term choreoathetosis describes slow chorea typically seen in patients with cerebral palsy. There are numerous causes for chorea. The important causes of hemichorea include infarction or hemorrhage, arteriovenous malformation, moyamoya disease, polycythemia rubra vera, migraine, following cardiac surgery with hypothermia and extracorporeal circulation in children. A 52-year-old female patient came with gradual onset progressively worsening left hemichorea. The patient developed left upper limb and left lower limb weakness 3 months before onset of hemichorea; she was also a diabetic and hypertensive controlled on oral medications for the past 6 years. Blood investigations showed hemoglobin – 11.1, total leukocyte count – 12,100, erythrocyte sedimentation rate – 32. Blood sugar – 240 mg/dl, and liver function test and renal function test were normal. Echocardiography showed left ventricular hypertrophy. Computed tomography brain (old) showed right capsuloganglionic and thalamic acute infarct. Magnetic resonance imaging brain showed right thalamic subacute lacunar infarct, mild cerebral atrophic changes, and right thalamic old lacunar infarcts. She was treated with subcutaneous insulin with adequate hydration and blood sugar was controlled appropriately. In view of persisting hemichorea, the patient was initiated on oral haloperidol, trihexyphenidyl, sodium valproate, and topiramate, including antiplatelets and statins. There was good symptomatic control of hemichorea. Ischemic stroke is an important cause of hemichorea in the 5^th to 6^th decade of life, requiring control of metabolic parameters such as hyperglycemia as well as optimal management of stroke and its risk factors. Dopamine antagonists and antiepileptic drugs like sodium valproate and topiramate are very useful for symptomatic control in choreoathetosis

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP30/326: Raised blood urea nitrogen/creatinine ratio is associated with increased mortality in patients with intracerebral hemorrhage

Pathak A ¹, Joshi D ¹, Mishra VN ¹, Chaurasia RN ¹, Priya ¹

Objective: Dehydration may impair cerebral oxygen delivery and worsen clinical outcome in patients with acute intracerebral hemorrhage (ICH). We evaluated if elevated blood urea nitrogen-to-creatinine ratio (BUN/Cr) as a marker of dehydration was associated with death in acute ICH.

Methods: We conducted a prospective cohort study using a stroke registry enrolling all emergency department patients with ICH from October 2016 through June 2018. Poor clinical outcome was defined using modified Rankin scale (mRS) score at discharge. BUN/Cr ratio of 15 was considered elevated.

Results: A total of 108 consecutive patients of ICH were categorized into two groups (Group I including mRS 01–05 and Group II including mRS with score of 6, i.e., in hospital death). Twenty-three (21.2%) patients had death during hospital stay. The mean and standard deviation of the National Institutes of Health Stroke Scale (NIHSS), Glasgow Coma Scale score, and urea/Cr ratio were found to be statistical significantly associated with the mortality outcome in ICH patients. High NIHSS score (odds ratio [OR] = 1.20; 95% confidence interval [CI]: 1.08–1.33), history of diabetes (OR = 5.86; 95% CI: 1.47–22.85) variables associated with a poor clinical outcome OR 6.5 (3.6–11.8), history of diabetes OR 2.7 (1.5–5.0), and BUN/Cr ratio (OR = 1.03; 95% CI: 1.05–1.06).

Conclusion: An elevated BUN/Cr ratio in patients with ICH is associated with poor outcome at discharge. Further study is needed to see if acutely addressing hydration status in patients with ICH can alter outcome

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP31/333: Cerebral venous sinus thrombosis in elderly patients at tertiary care center

Patel K ¹, Alexander M ¹, Benjamin R ¹, Aaron S ¹

Background and Purpose: Cerebral vein and dural sinus thrombosis in elderly patients have different presentation, etiology, and prognosis. We need a high index of suspicion to diagnose elderly cerebral venous sinus thrombosis.

Methods: It is a retrospective observational study of patient from 60 years of age and above from 2008 to 2018.

Results: A total of 993 patients were analyzed and 40 (4%) patients were above 60 years of age with the mean age of 65.1 ± 5.2 years. Most common clinical presentation in elderly patients was seizures (65%), followed by stroke-like presentation (60%). Encephalopathy was more common presentation than isolated intracranial hypertension. The mean duration of symptoms was 8.2 ± 15.4 days. The mean duration of presentation with headache was 15 days and without headache was 1.3 days. Hyperhomocysteinemia (45%) was the most common cause for elderly cerebral venous thrombosis. We also found prothrombotic factors (22.5%), infections (10%), neoplasms (5%), and polycythemia (2.5%) as other risk factors in the elderly. Duration of stay in hospital was around 2 weeks. The rate of intervention of 7.5% was less frequent in elderly patients. Nearly 62.5% of patients showed improvement or residual deficits (17.5%) at discharge. On univariate analysis, the absence of headache (P = 0.0018), altered sensorium at admission (P = 0.018), infection (P = 0.004), polycythemia (P = 0.043), and no intervention (P = 0.036) are independent predictors of poor outcome.

Conclusion: Cerebral venous sinus thrombosis in the elderly has varied presentation. Hyperhomocysteinemia was the most common risk factor. Patients with absence of headache, altered sensorium at presentation, infection, and polycythemia have poor outcome.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP32/337: Bifurcation of common carotid artery: A computed tomography angiogram-based study from Central India

Sinha H ¹, Sinha MB ¹, Nayak N ¹

Introduction: The anatomy of common carotid artery (CCA) bifurcation is of paramount importance in both surgical and medical specialties. However, on account of significant variation seen in its anatomy, we believed that the topography of CCA bifurcation now needs to be readdressed, especially for surgical procedures of neck. The aim of our study is to substantiate the available knowledge about the anatomy of CCA and to analyze its any unusual occurrence in the population of Central India. Furthermore, very few computed tomography (CT)-based studies are available on CCA.

Materials and Methods: The study was done on 24 patients retrospectively. Randomly, nonconsecutive CT carotid angiogram was analyzed.

Results: Anteriorly, we found the most common location of bifurcation of common carotid artery (CCB) at the level of greater cornu of hyoid bone (38.63%) and above the greater cornu of hyoid bone (38.63%). Posteriorly, the most common vertebral level was detected at lower part of vertebra C4.

Conclusion: In our study, we found that the higher carotid bifurcation is the most common bifurcation. The implication of this finding is that any surgery in this region could be potentially risky, particularly if the surgeon is not familiar with this variation. Furthermore, this study supplements data regarding the level of division of CCA in situ by CT angiography as compared to previous cadaveric studies.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP33/339: Cerebral venous sinus thrombosis as a complication of varicella-zoster infection

Sundaram SN ¹, Balasubramanian S ¹, Ranganathan LN ¹, Sindhuja L ¹, Prabaharan U ¹, Gupta D ¹, Ajith K ¹

Introduction: Cerebral venous sinus thrombosis (CVST) is a rare complication among varicella-zoster-related neurological complications as varicella infection can be associated with coagulopathy.

Case Report: A 32-year-old male patient who had varicella-zoster infection with recovering skin lesions presented with acute onset severe headache with vomiting on the 8^th day and recurrent episodes of seizures of the left focal with secondary generalization on the 11^th day with postictal weakness which improved. Examination revealed bilateral papilledema without other cranial nerve or sensory motor deficit. Imaging revealed superior sagittal sinus and bilateral transverse sinus thrombosis. There were no parenchymal hyperintensities or infarct or bleed. Cerebrospinal fluid analysis was not significant. Serum immunoglobulin M antibodies were positive for varicella-zoster virus (VZV). Procoagulant workup showed reduced protein S levels. ENA profile was negative. The patient improved with anticoagulation and antiviral medication.

Discussion: Coagulopathy with CVST can occur during or in the recovering phase of varicella-zoster infection. The possible mechanism of CVST after VZV infection could be a postinfectious immunologic reaction with vasculitis, thrombosis due to direct endothelial damage, and acquired protein S deficiency. Considering and in spite of the unknown status of preexistent hypercoagulable state in our patient, there is certainly an attributable relationship between varicella infection and CVST due to the immune-mediated mechanisms.

Conclusion: Patients with primary VZV infection are prone for hypercoagulable state. CVST should be considered in them when they present with related symptomatology.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP34/340: Study of complications in patients on Vitamin K antagonists

Kumar S ¹, Misra UK ¹, Kalita J ¹

Background: Vitamin K antagonists are associated with complications. Few studies have evaluated the role of genetic polymorphism in the development of complications.

Objective: To study the genetic and nongenetic predictors of complications in patients on oral anticoagulation (OAC) who attended neurology service between March 2013 and February 2016. Detailed history, clinical examination, and investigations were done. Regular international normalized ratio monitoring was done. Complications were noted. Genomic DNA extraction was done using standard salting-out method. Genotyping for polymorphisms of VKORC1 (-1639G>A) was performed by polymerase chain reaction.

Results: A total of 157 patients were available for analysis. The mean age of patients was 40.87 ± 15.0 years. Ninety-two patients were taking OAC for cardioembolic stroke, 62 for Cerebral venous sinus thrombosis, and 3 for deep vein thrombosis. There were 36 complications, 16 had thrombosis, and 20 had bleeding. The rate of thrombosis and bleeding was 11.16/100 person years and 13.94/100 person years, respectively. On univariate analysis, the occurrence of bleeding was related to low educational status, vegetarian diet, and use of diltiazem and digoxin. Use of diuretic was related to thrombosis. On multivariate analysis, the occurrence of thrombosis was related to use of polypill and diuretic, while bleeding was related to male sex and low educational status. Survival plot analysis revealed that the mean time of occurrence of first complication in patients with AA genotype is 20.5 months, and GA and GG is 28.5 months and 30 months, respectively.

Conclusion: Complications are related to male sex, vegetarian diet, polypills, drug interactions, and low educational status. Variant genotype may require stringent follow-up to reduce complications.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP35/345: A retrospective study of young stroke etiology in tropics

Shanmugam S ¹, Sridharan B ¹, Muthuveeran N ¹, Manivannan MR ¹, Justin C ¹, Amalraj ¹, Ganesan K ¹, Chezhian D ¹, Pandian G ¹, Pavalam B ¹, Pandian SR ¹, Subramanian ¹

Background and Purpose: Conventionally, young stroke is the cerebrovascular accident (CVA) occurs to patients <40 years. It differs from the geriatric stroke in many aspects, one of which is the young who sustained the disability is the breadwinner of the family, so many other differences, very important is the etiology, where elderly has atheroembolic phenomenon as the common culprit, the younger has diverse etiological factors causing young stroke. Hence, the study aimed to find out the most common etiological factors.

Materials and Methods: Our study is a retrospective study, all the comorbidities and risk factors, whether directly caused the stroke or act in synergy studied in detail. The source of the patients, are taken from the stroke registry of our neuro and general medicine departments, who comes under the definition of young stroke, i.e. below 40 years with CVA, hemorrhagic and ischemic strokes taken in to consideration, l it is found that that among young ischemia scores over bleed. Duration of the study was 2 years, 2016–2018.

Results: (1) RHD (valvular heart disease) - 67%, male 35, female 42, mitral common in females multivalvular common in males. (2) In female's vasculitis (3%), one case of aplas, (3) cryptogenic-three male strokes, with agitated saline contrast echo, one case– PFO.

Conclusion: Should aim at early detection of RHD, which is still the most common cause for stroke in tropics, should be repaired or replaced with proper anticoagulation, thus to prevent stroke in young.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP36/346: Study of initial electrolyte abnormalities in acute stroke patients

Sudheer P ¹, Shankar V ¹

Aims and objective: To evaluate the initial electrolyte abnormality in patients with acute stroke.

Materials and Methods: A cross-sectional study was done over a period of 6 months from October 2017 to March 2018.

Inclusion Criteria: Patients above 18 years of age with first episode acute stroke admitted within 48 h of onset and confirmation of stroke by imaging studies.

Exclusion Criteria: Patients with previous stroke, transient ischemic attack, head injury, stroke mimics, and tumors.

Results: One-hundred patients with acute stroke, 90 patients had ischemic and 10 patients had hemorrhagic stroke of 90 patients with ischemic stroke, 27 patients had sodium abnormality of which 23 had hyponatremia, 4 had hypernatremia, 4 had hypokalemia and 1 had hyperkalemia one had hypocalcemia and 3 patients had hypercalcemia.10 with hemorrhagic stroke 4 had hyponatremia and 5 had hypernatremia, 9 had hypokalemia and 1 had hyperkalemia. In 23 patients with hyponatremia in ischemic stroke in which the arterial distribution was as follows, 17 had infarct in the left middle cerebral artery (MCA), 3 in the right MCA, and 2 had posterior circulation (PC) infarct, 1 had left anterior cerebral artery infarct. Four had hypernatremia of which 3 were PC and 1 had large MCA infarct. One patient had both hyponatremia and hypokalemia. The age of the patient with ischemic stroke with sodium abnormality was as follows: >70 years in 9, 60–70 years in 6, 50–60 years in 7, <50 years in 1. Patients with chronic kidney disease 5 patients of which 4 had hyperkalemia. Two patients succebed both of them had hyponatremia and along with sepsis and were above 70 years.

Conclusion: Hyponatremia is most common dyselectrolytemia in stroke. Dyselectrolytemia is more common in hemorrhagic stroke than ischemic stroke. Electrolyte abnormality is also related to other comorbidities of the patient.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP37/356: Multiple cranial neuropathy and radiculopathy: An unusual presentation of cerebral venous sinus thrombosis

Behuria P ¹, Mallick AK ¹, Samanta M ¹

Introduction: Cerebral venous sinus thrombosis (CVST) has variable mode of onset and clinical manifestations such as headache, seizure, encephalopathy, isolated intracranial hypertension, and focal neurological symptoms. Atypical presentations such as cranial nerve palsies and false localizing signs due to raised intracranial pressure (ICP) have also been reported. Radiculopathy as a false localizing sign is rare.

Clinical Scenario: A 35-year-old female presented with gradual-onset holocranial dull aching headache, difficulty in deglutition, and deviation of angle of mouth to right 12 days after delivery. Two days later, she complained of blurring of vision, diplopia, and weakness of limbs. O/E-B/L papilledema, B/L 6^th, Left LMN 7^th, 9^th, 10^th Cranial nerve palsies and areflexic quadriparesis (proximal > Distal) was noted. On investigation, routine tests were normal. Cerebrospinal fluid (CSF) study was normal except elevated opening pressure. MRV showed thrombosis in the posterior superior sagittal sinus, torcula, and B/L transverse sinus. The nerve conduction study showed prolonged F-waves. She recovered partially with anticoagulants.

Discussion: Cranial nerve palsy in CVST has been attributed due to the raised ICP, extension of thrombosis to venous channels, or direct pressure from the clot. Lateral sinus (transverse plus sigmoid portion) drains blood from the cerebellum, brainstem, and veins from cranial nerves in the posterior fossa. Thrombosis of the lateral sinus can produce venous congestion and hypoxia of the cranial nerve veins. Severe raised ICP may lead to sixth cranial nerve palsy. Radiculopathy is due to elevated CSF pressure distending the subarachnoid space around the nerve roots resulting in mechanical stretching and venous ischemia.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP38/358: Atypical Wallenberg's syndrome due to arterial thrombosis associated with gastric cancer

Balaji BS ¹, Chandramouleeswaran V ¹, Kannan V ¹, Ranganathan LN ¹

Purpose and Background: Various patterns of sensory disturbance have been observed in Wallenberg's syndrome. The typical sensory impairment is ipsilateral facial and contralateral trunk and limb thermal hypesthesia and hypoalgesia (crossed sensory pattern). Tactile sensation is not generally diminished. Here, we report a case of 39-year-old male patient with gastric cancer on chemotherapy presented with Wallenberg's syndrome having atypical sensory impairment and discuss its neuroanatomical correlation.

Case Description: A 39-year-old man, nonsmoker, nondiabetic male patient presented with acute spinning sensation, dysphagia, unsteadiness, and numbness with profuse sweating of left half of body including face progressing over 2 days. The patient was on chemotherapy for gastric carcinoma. Neurological examination revealed gait ataxia with falling to the right side; right hypotropia and miosis, spontaneous and left gaze evoked nystagmus, deviated uvula to left, right dysmetria, both tactile and thermal/pain hypesthesia of the right and left half of face, thermal/pain hypesthesia of left UL, trunk and LL, intact vibration, and position sense. Brain magnetic resonance imaging revealed acute infarcts in the right dorsolateral medulla and bilateral cerebellum and occlusion of distal basilar and both vertebral arteries.

Conclusion: We present a case of Wallenberg's syndrome which describes atypical features of tactile and thermal/pain hypesthesia on both ipsi- and contralateral side of the face and thermal/pain hypesthesia on contralateral limbs and trunk. We attributed this atypical pattern to lesion of dorsolateral medulla with ventral extension and protopathic tactile sensory pathway. This case report highlights the presence of atypical presentations of LMI that may initially challenge the physician's diagnostic reasoning and rare occurrence of arterial thrombosis associated with cancer.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP39/360: Aortic computed tomography angiographic abnormalities in acute ischemic stroke

Nardla MR ¹, Venkatasubramanian S ¹

Background: Thrombotic disease in aortic arch is an uncommon cause of stroke. With the use of computed tomography angiography (CTA), aortic thrombosis is picked up incidentally. Aortic abnormalities in patients of acute ischemic stroke (AIS) in whom CTA was done were studied.

Aim: The aim of this study was to analyze CTA findings in the arch of the aorta in patients with AIS, treatment and followed for 3 months for outcome and complications.

Methods: A total of 104 patients with AIS who underwent CTA during the period of May 2017 to May 2018 were studied. Aortic abnormalities, clinical features, treatment, and follow-up were studied.

Results: A total of 104 patients with AIS in whom CTA was done, four patients had aortic abnormalities. Of the four patients (M = 3 and F = 1), three had thrombus and one had ulcerated plaque in the aorta. Among four patients, three had dyslipidemia, two had systemic hypertension, two had diabetes, and one had a history of ulcerative colitis. Out of four, two patients had anterior circulation and two had posterior circulation strokes. Only one patient had isolated aortic thrombus with no other lesions and remaining three patients had atherothrombosis in arteries corresponding to the site of infarct. All patients with aortic thrombus were started on oral anticoagulants. No embolic phenomena were seen during the follow-up.

Conclusion: Incidence of 3.8% was seen in the study. Dyslipidemia, diabetes, systemic hypertension, and history of ulcerative colitis are the risk factors observed in the study. In patients with no other cause, aortic thromboembolism could be cause for stroke for which treatment is anticoagulation and hence should be evaluated with CTA or transesophageal echocardiogram which would otherwise be missed.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP40/367: Stroke mimics: A tertiary care teaching hospital experience

Alexander PT ¹, Nandish A ¹, Sivadasan A ¹, Mathew V ¹, Aaron S ¹, Alexander SM ¹, Alexander M ¹

Introduction: Stroke mimics are a major diagnostic challenge that presents as important differential diagnoses in the setting of acute ischemic strokes which require thrombolytic management.

Aim: The aim of this study was to identify masqueraders that present as acute strokes within thrombolytic window period.

Materials and Methods: We conducted a retrospective, observational study including 709 patients who presented as acute strokes to the emergency services and as in patients in Christian Medical College, Vellore, from June 2017 to May 2018. We defined acute stroke as a patient presenting with focal neurological deficits within 6 h of onset – the window period for acute management with thrombolysis and/or endovascular thrombectomy.

Results: About 20% (135) of the total numbers were identified as stroke mimics. The most common mimics identified were seizures (15%) and cardiogenic etiology (15%). Other common etiologies included encephalopathies secondary to infections (10%), metabolic etiology (10%), hypertensive emergencies (10%), and peripheral causes of vertigo (10%). The less common causes identified were toxins and drug overdose (5%), cerebral venous thrombosis (5%), and malignancies (5%).

Conclusion: This study highlights the significance of the number and variety of mimics that can present with clinically similar presentation as an acute ischemic stroke, and the importance of identifying them using a thorough clinical history and examination, and supported by radiological evidence and laboratory parameters – as their management will follow an entirely different algorithm, not involving thrombolysis or thrombectomy – which is the essence of using magnetic resonance imaging acute stroke protocol as a screening tool for acute ischemic strokes.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

Saturday, September 29, 2018, 13:00-14:00 h, E-Poster designated area: E-Poster Session – 07: Stroke: SEP42/AWPN2: Hypoadiponectinemia, an acute phase response to stroke: Correlation between adiponectin and severity/functional outcome of stroke

Verma (Khushwah) A ¹, Kathpal JS ¹, Rai VK ¹

Background and Objective: Role of hypoadiponectinemia as cardiovascular risk factor is well established, but in scenario of stroke, there are very few studies to establish the association. In the current study, we evaluated correlation between level of adiponectin and neurological severity and functional outcome of stroke.

Materials and Methods: A cross-sectional, analytical, observational study was performed including 105 cases of stroke. Clinical information comprising stroke severity was obtained on day 1 and 3 using the National Institute of Health Stroke Scale. Functional outcome was measured on day 3 and 90 using the Modified Rankin Scale. Laboratory investigations including adiponectin level (using sandwich assay technique) were assessed on day 1.

Results: High adiponectin has statistically significant and positive correlation with severity (Pearson coefficient = 0.243 and P = 0.013) and outcome of stroke (Pearson coefficient = 0.199 and P = 0.042). It implies that a rise in adiponectin level tends to occur in patients with severe stroke and poor outcome. In the absence of more than two risk factors, high adiponectin level is in fact associated with severity and outcome of stroke.

Conclusions: Adiponectin by virtue of its antiatherogenic and anti-inflammatory actions protects endothelial cells and prevents cerebral vascular inflammation. We can postulate that in the event of stroke, adipo-vascular axis responds through acute pathophysiological changes leading to increase in level of adiponectin. More the severity of stroke, more will be rise in adiponectin, to counter the pathological damage. Poststroke hypoadiponectinemia is not associated with severity and outcome of stroke.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP43/AWPN3: To assess the cognitive function in patients with carotid artery stenting

Gupta A ¹, Huded V ¹, Phillip VJ ¹

Background: Patients with transient ischemic stroke or strokes due to carotid artery stenosis are commonly seen in acute neurological critical care situations. In the management of these patients, carotid artery stenting is done to help lower the risk of stroke. Carotid artery stenting is one of choice of treatment for carotid artery stenosis. There is little data regarding the changes in cognitive functions in a patient who undergo carotid artery stenting.

Materials and Methods: Study design: This was a prospective observational study that was done at a tertiary care center, Narayana Hrudayalaya hospital, Bangalore, in admitted patients. Inclusion criteria: Patients admitted for carotid artery stenting were included in the study. Exclusion criteria: (1) Patient with previously diagnosed cognitive impairment or dementia, (2) illiterate patient, (3) patient with eyesight problem, speech problem, and hearing problem, (4) preexisting psychiatric illness, (5) drug or alcohol abuse, and (6) noncooperative patient were excluded from the study. Study period: The study period was 12 months, June 2017 to May 2018, and the number of patients was 12.

Method: Demographic information from all patients was collected in a predefined format. All patients who underwent carotid artery stenting and who satisfied inclusion and exclusion criteria were enrolled for the study. We assessed cognitive functions of each patients before and after 3 months of carotid artery stenting according to Addenbrooke's cognitive examination scale. Then, we compared cognitive parameters among them. Data collection and statistical analysis: Descriptive analysis of data and comparison done using Shapiro–Wilk test.

Results: Cognitive scores on the memory and language were improved after 3 months of follow-up. No significant differences (P value) on cognitive functions including the memory (0.156), verbal fluency (0.715), attention (0.680), and language (0.195) were observed preprocedural and 3 months after carotid reopening through carotid artery stenting.

Discussion: In our study, we found that the scores on the memory and language were improved after 3 months of follow-up, but it was not associated with a significant P value. We also observed that there were no recurrent ischemic events in any patients during 3 months of follow-up periods.

Conclusion: Carotid artery stenting was not associated with significant cognitive function improvement. There were no recurrent ischemic events in any patients during 3-month follow-up period.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP44/: Withdrawal: SEP45/AWPN26: A novel wearable device for motor recovery of hand function in chronic stroke survivors

Choudhury S ¹, Shobhana A ¹, Singh R ¹, Sen D ¹, Anand SS ¹, Shubham S ¹, Baker MR ¹, Baker SN ¹, Kumar H ¹

We have previously demonstrated in monkey that reticulospinal connections to hand and forearm muscles are strengthened following corticospinal lesion, contributing to recovery of function (Zaaimi et al., 2012). Reticulospinal neurons can be activated by auditory click stimuli, probably partially through the vestibular apparatus (Fisher et al. 2012). Pairing clicks with electrical stimulation of a muscle induce plastic changes in motor pathways (probably the reticulospinal tract) through spike-timing-dependent plasticity mechanisms (Foysal et al. 2016). In this study, we tested whether pairing clicks with muscle stimulation could improve hand function in stroke survivors. Clicks were delivered through a miniature earpiece, and electrical stimuli at motor threshold were given over the forearm extensor muscles. Stimulation was delivered using a wearable electronic device, as in Foysal et al. (2016), which allowed the patient to receive stimulation at home while performing normal daily activities. We recruited 95 patients who had suffered a cortical or subcortical stroke at least 6 months previously. Patients were randomized to three groups: (1) wearable device stimulation at ~0.67 Hz, where muscle stimuli were given 12 ms before click, (2) wearable device stimulation, where click and shock occurred independently at random, also at 0.67 Hz, and (3) standard care. Those allocated to the device used it for at least 4 h per day, every day for 4 weeks. Hand function was assessed at baseline (week 0), and weeks 2, 4, and 8, using the Action Research Arm Test (ARAT) which has four domains – grasp, grip, pinch, and gross.

Severity across three groups was comparable at baseline (mean total ARAT in three groups – 18.09, 10.75, and 17.25, P = 0.194). Group 1 improved in the total ARAT (mean week 0: 18.09 and week 8: 19.77, P = 0.019) unlike the other two groups. The grasp subscore also improved significantly (mean week 0: 5.84 and week 8: 7.18, P = 0.004) only in Group 1. Other ARAT subdomains showed no significant changes in any groups. Only one device-related adverse event occurred, which was a contact dermatitis associated with the adhesive surface electrodes; this improved with topical steroid cream with no interruption of treatment. We conclude that a wearable device delivering paired clicks and shocks can produce a significant improvement in hand function in stroke survivors, probably through an action on the reticulospinal tract.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP46/25: Utility of video-electroencephalogram monitoring in a tertiary care epilepsy center

Chatterjee A ¹, Chandran V ¹, Nair RK ¹, Radhakrishnan K ¹, Gorthi SP ¹

Long-term video-electroencephalogram (VEEG) monitoring is an important tool and an essential component of a comprehensive epilepsy care program. A routine 30 min EEG detects abnormalities in only 50% of the patients who are ultimately diagnosed with epileptic syndromes. Furthermore, very rarely is the patient's habitual event recorded. Clinical applications of VEEG may be classified as (1) differentiation between true epileptic versus nonepileptic events, (2) localization of epileptic foci, (3) characterization of epileptic syndromes, (3) presurgical evaluation of patients with medically refractory epilepsy, and (4) to monitor therapeutic response and/or modification of antiepileptic drug therapy. In this study, we aimed to determine if the specific clinical reason as to why the VEEG was recommended was indeed answered at the end of the study. The first 400 patients admitted to the VEEG unit at a tertiary care hospital were retrospectively reviewed and whether the stated goal was achieved or not was determined. We found that long-term monitoring was successful in accomplishing the goal of admission in 84% of the patients. The main reason for failure in the rest was primarily due to failure to capture the habitual event (23.30%) or lack of interictal discharges (5.5%) of the cases. About 25% of the patients of drug-resistant epilepsy who were referred for presurgical evaluation were referred for epilepsy surgery. No adverse events were noted. Our study confirms that VEEG, when utilized appropriately, is highly productive in patients with difficult to treat epilepsy.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP47/AWPN15: Neurocardiac axis in central vertigo

Moorthy MP ¹, Srinivasan AV ¹, Bhanu K ¹

Methods: This study is done in the Department of Neurology, Coimbatore medical college hospital, Tamil Nadu, from January 2018 to May 2018. Inclusion Criteria: All patients presented with central vertigo were included in the study. Exclusion Criteria: Patients presented with peripheral vertigo were excluded from the study. All patients presented with central vertigo were undergone electrophysiological studies includes cervical vestibular-evoked myogenic potential (cVEMP), electroencephalography (EEG), and electrocardiography (ECG).

Results: This study is done in 40 patients presented with central vertigo in the age group of 20–60 years, males (70%) mean age (46 years) and females (30%) mean age (43.6 years). cVEMP-(P¹³ to N²³) amplitude mean in normal side (33.25 µv) and abnormal side (21.38 µv). Patient's neurocardiac axis was analyzed using cVEMP, EEG, and ECG. cVEMP showed statistically significant drop in (p¹³ to N²³) amplitude (P < 0.00001) and left axis deviation in ECG (P < 0.00001). All patients with central vertigo showed normal EEG pattern.

Conclusion: Electrophysiological evaluation of neurocardiac axis using cVEMP, EEG, and ECG is useful for early detection and treatment of central vertigo.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP48/AWPN17: Association between gender, involved hemisphere, and outcome after stroke and influence of socioeconomic status on stroke outcome: An ambispective cohort study

Kapoor N ¹, Prasad K ¹, Shukla G ¹, Srivastav A ¹, Vibha D ¹

Objectives: The objectives of this study were to determine the effect of gender and involved hemisphere on outcome after stroke.

Methods: We did an observational ambispective cohort study at AIIMS hospital, New Delhi, in Northern India from February 2016 to August 31, 2017. A total of 282 patients were enrolled, out of which 180 were prospectively recruited and 102 with retrospective chart review, who were admitted in neurology ward. Consecutive patients, male or female, with age 18–70 years, presenting with ischemic or hemorrhagic stroke, within 1 week of onset of stroke were recruited. Patients with posterior circulation stroke, brainstem stroke, cerebellar stroke, or recurrent stroke were excluded from the study. Outcomes were analyzed by assessing modified Rankin's Score (mRS) after 3 months. Poor outcome was defined as mRS score >3 points at 3 months. Other relevant variables, prognostically important for stroke, were recorded for all patients.

Results: A total of 282 patients were enrolled out of which 16 patients were lost to follow-up and hence were excluded from the analysis. Data of 202 male and 64 females were analyzed. Mean age of patients in entire cohort was 51.24 ± 13.04 years. Logistic regression analysis showed that males with left-sided stroke had the worst outcome than right side male stroke, both in infarct (not significant) and intracerebral hemorrhage (ICH), ICH: OR 7.00, (95% CI 1.856–26.448, P = 0.004) (Adjusted: age and volume of bleed). Right side female stroke had a worse outcome than right side male stroke, ICH: OR 20.543, (CI: 1.882–224.176, P = 0.013) (Adjusted: volume of bleed, age, and midline shift). However, in infarct, they did better than right side males ischemic strokes, (not statistically significant). Left side female had a worse outcome than right side male in infarct, OR 3.60, (CI: 0.965–13.488, P = 0.05) (Adjusted: NIHSS, ASPECT, and age). Same results were observed for ICH (not statistically significant).

Conclusion: This study shows difference in outcomes after stroke in both sexes when different hemispheres were involved with left-sided hemispheric strokes in men having a poor outcome as compared to right side hemisphere strokes in men.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP49/: SEP50/AWPN20: To study the occurrence of unrecognized diabetes mellitus and prediabetes in patients of acute ischemic stroke

Rani P ¹, Chaudhry N ¹, Puri V ¹, Singh B ¹

Objective: The objective of this study was to study the occurrence of unrecognized diabetes mellitus and prediabetes in patients of acute ischemic stroke.

Methods: A total of 75 patients with acute ischemic stroke with no previous diagnosis of diabetes mellitus were included in the study. Insulin levels and HbA1C were measured during h2511 hospital stay along with daily fasting plasma glucose. Insulin resistance was estimated by homeostasis model insulin resistance index (HOMA). Patients were categorized into three groups: normoglycemics, prediabetics, and diabetics. All investigations were repeated at the end of 3 months, to assess any change in their glycemic status.

Results: Out of the 75 patients, 25 (33.33%) were normoglycemic and 50 (66.66%) had hyperglycemia initially. Of these 50 hyperglycemics, 7 (14.0%) were diabetic and 43 (86.0%) had prediabetes. Totally 16 patients were lost to follow-up and so reevaluation was performed in 59. Out of 59 patients, 20 (33.8%) were normoglycemic, 33 (55.9%) prediabetic, and 6 (10.1%) were diabetic. At 3 months, all normoglycemics and diabetics continued to remain so. Out of 33 prediabetics, four developed diabetes while six patients became normoglycemic. HOMA at initial evaluation was 1.57 + 1.07 in normoglycemics, 3.26 + 2.86 in prediabetics, and 11.7 + 8.98 in diabetics.

Conclusion: The reactionary hyperglycemia at stroke presentation is found only in a few subjects as seen in our study, while majority have an abnormal glucose metabolism. This can be identified using simple and inexpensive investigations and should be done during the early course of acute stroke for a more effective and aggressive stroke prevention protocol.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP51/AWPN22: Parietal drift: A useful clinical sign

Nagendra S ¹, Soni S ¹, Jagiasi K ¹, Ansari A ¹

Background: Clinical signs that help detect subtle weakness are invaluable in reaching early localization of lesion and diagnosis. The parietal drift is a useful clinical sign to localize lesions in the parietal lobe.

Objective: The objective of this study was (1) to characterize the parietal drifts which are observed on the basis of extent, direction, and hand and finger movements and (2) to study the association between the direction of the drift and the underlying causative lesion in the brain parenchyma.

Materials and Methods: Hand drift, fingers movement, and any abnormal posturing of fingers and wrist were noted by the two assessors separately, for a period of 20–30 s each. The drift is described in each patient on the basis of six parameters such as (1) curling of fingers, (2) outstretching of thumb, (3) outward drift of hand, (4) upward drift of hand, (5) downward drift of hand, and (6) pronation of hand.

Results and Conclusions: (1) Patients having lesions in parietal lobe demonstrated a hand drift called as the parietal drift which consisted of hand movement in the outward, upward, or rarely in downward direction. (2) An attempt can be made to localizing the site of lesion while awaiting radiological images by a simple characterization of this clinical sign. (3) The parietal drift consisted of outward and upward movement of the hand in patients having lesion in the parieto-occipital regions. (4) The parietal drift consisted of upward but not outward movement of the hand in patients having lesion in the parieto-temporal regions.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP52/AWPN23: An interesting interplay of atrial fibrillation clinical, radiological, and prognostication profile of cerebrovascular accident in a tertiary care hospital

Shasthara P ¹, Harsha S ¹

Background: “Cryptic strokes” in the past are increasingly being attributed to the sine qua non-atrial fibrillation. Interplay of stroke and atrial fibrillation with respect to etiology, types, and clinicoradiological correlation is done in the present study.

Objectives: The objectives of this study were Primary – To determine in patients with stroke whether atrial fibrillation influences clinical features and prognosis, contribution to the causation of different types of stroke and to evaluate the cause of atrial fibrillation. Secondary –- To document the clinical outcomes and quality of life of patients of atrial fibrillation with stroke and attempt to determine whether atrial fibrillation influences the outcome (30 days) and to evaluate socioeconomic implications in this cohort.

Materials and Methods: An observational clinical study conducted on 200 stroke patients based on exclusion and inclusion criteria. Descriptive and inferential analysis has been carried out in the present study. Student t-test and Chi-square test have been used.

Results: Out of 200 patient's studied, permanent type of AF and hypertensive heart disease were the most common type of atrial fibrillation and its cause, respectively. Of types of stroke in patients with AF, 63.5% were thrombotic and 35% were hemorrhagic including 1.5% of subarachnoid bleed. Morbidity scores, early recurrent strokes, and case fatality were higher in patients with AF.

Discussion: Patients of atrial fibrillation have all varieties of stroke including thrombotic, hemorrhagic, and subarachnoid bleed. They are likely to have compromised quality of life, morbidity-mortality aspects, and higher early recurrence of stroke.

Conclusion: It is important to address the issue of looking for the most common and treatable arrhythmia to bring down morbidity and mortality in stroke patients.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP53/AWPN28: Cerebrovascular reactivity in ischemic stroke patients: A case–control study

Shah UK ¹, Narayan SK ¹

Background: Neurovascular unit is an evolving concept to understand cerebral hemodynamics which is found to be impaired in ischemic stroke. The integrity of neurovascular unit can be assessed by cerebrovascular reactivity. Data in this regard are lacking in Indian population; therefore, we investigated cerebral autoregulation in ischemic stroke patients.

Method: This was a case–control study. Study participants were 50 patients of middle cerebral artery (MCA) territory ischemic stroke (within 1^st month of stroke) and 50-age- and sex-matched healthy controls. Transcranial Doppler was used to assess breath-holding index (BHI) from both MCAs simultaneously, in supine position, using standard technique. Primary outcomes were BHI mean values which were compared between case and control groups. Secondary outcome was to find out association of ischemic stroke risk factors with low cerebrovascular reactivity.

Results: Mean age of participants was 56 years, with 88% males. The stroke and control groups were similar in characteristics except for significantly higher frequency of smoking and alcoholism in stroke group. Majority of patients in our study had mild strokes (median NIHSS-5). The predominant subtype was lacunar stroke (48%), followed by large artery atherosclerosis (44%). The MCA BHI was 47% ipsilateral to the side of stroke, 61% on the contralateral side, while in the controls right and left MCA BHI were 89% and 86%, respectively. In the stroke patients, although there was a bilateral impairment of BHI, the degree of impairment was significantly more on ipsilateral (damaged side 0.47) when compared with contralateral (nondamaged side, 0.61) with P value 0.015. Mean BHI of both sides in stroke patients was 54%, while in controls, it was 88% with P value 0.003. On multivariate analysis, the presence of stroke and age was independently associated with impaired BHI (<0.69) with P value 0.026 and 0.027, respectively.

Conclusion: Vasomotor reactivity was impaired bilaterally in MCA territory ischemic stroke patients within the 1^st month of stroke. Presence of stroke and age was independently associated with low BHI.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP54/AWPN30: Vitamin B12 deficiency and headache: coincidence or much beyond?

Varsha A ¹, Surya N ¹, Kadu SP ¹

Background: Vitamin B12 deficiency is a systemic disease affecting the entire nervous system. In India, Vitamin B12 deficiency is seen in about 3.8% of population, largely linked to predominantly vegetarian diet.

Methods: The present study was an observational study done over 11 years (2005–2016) at an urban tertiary neurology clinic. We studied demographics, clinical features, biochemical, imaging, and electrophysiological characteristics (wherever applicable) of all patients who presented with neurological complaints and had low serum Vitamin B12 levels.

Results: Out of 5100 patients presenting to the clinic, 290 patients (males – 161/females – 129) had low Vitamin B12 levels. The mean (±SD) age at presentation and serum Vitamin B12 levels were 39.73 ± 14.23 years and 171.29 ± 98.42 pcg/ml, respectively. Majority were vegetarians (87.6%). The most common presenting complaint was headache (43. 1%), followed by giddiness/vertigo (15.5%), tingling and/or numbness in extremities (13.7%), gait imbalance and disturbances (10.35%), seizures (4.82%), tremors/involuntary movements (3.79%), memory disturbances/sleep disturbances (5.17%), abnormal behavior (2.06%), and unilateral facial weakness (1.38%). Acute headache was noted in 20/125 (16%) patients and chronic headache in 105/125 (84%) patients, among the subgroup presenting with headache. The mean (±SD) age and serum B12 levels were 36 ± 10.56 years (range: 14–67 years) and 162.93 ± 61.2 pcg/ml (range: 59–686 pcg/ml). Majority of these patients had migraine (28.8%); the other diagnoses being: chronic headache (tension type, muscle contraction, autonomic cephalgias, and others) (37.6%). The various secondary causes included sinusitis, vasculitis, tuberculous meningitis, venous thrombosis, and glaucoma.

Conclusion: Vitamin B12 deficiency presents with varied neurological manifestations. Whether low vitamin B12 levels in patients with primary headache, especially migraine is an incidental or a causal finding, remains yet to be explored.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP55/AWPN31: The care and cost of acute ischemic stroke in a stoke unit of a tertiary care hospital in Mumbai

Agarwal K ¹, Surya N ¹, Someshwar H ¹

Aim: The aim of this study was to evaluate the current status of care and cost of acute ischemic stroke in Mumbai. The hospital-based analysis at a tertiary emergency care hospital with a 24 h neurology team and stroke care unit.

Methods: During 9-month period of May 2017 to January 2017, consecutively hospitalized 72 patients with acute ischemic stroke data were collected. We examined the demographic data, in-hospital care, length of stay, outcome at discharge, and hospital costs. The medical cost data were collected from official hospital medical accounts department, which calculated direct medical costs for beds, staff, radiological and blood examination, medications, and rehabilitation.

Results: The mean age was 60.81 years, and 81.12% were male. The mean National Institutes of Health Stroke Scale Score (NIHSS) was 8.07 points on admission. All patients underwent magnetic resonance imaging (MRI) angiography of brain on admission. All patients were treated with ischemic protocol (Antiplatelet, Neuroprotector, LMWH, and Statin and treatment of comorbidity) in stroke unit. Almost 52.72% patients were admitted to neurological Intensive Care Unit. Overall, 100% patients received in-hospital rehabilitation; mean length of stay was 10.77 days. In hospital, mortality rate was 0.03%. The mean hospital cost per patient was 89,610 INR (8320.3INR/day). The mean ICU cost per patient was 13,495 INR per day. Of which 44.03% was attributable to the costs for beds, doctor, and staff, 9% for medicine, 3% for rehabilitation, 16% for imaging studies, 12% for surcharge, and 24% for laboratory examinations. National Institutes of Health Stroke Scale score on discharge were 4.07 points.

Conclusion: Despite the single hospital-based analysis, this study provided the current precise data on short-term inpatient care and costs of acute ischemic stroke in a tertiary care hospital in Mumbai. We can conclude that early effective and proper management of stroke leads to a better and cost-effective outcome.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

Saturday, September 29, 2018, 16:30-17:30 h, E-Poster designated area: E-Poster Session – 08: Infections, Neuromuscular Disorders, Stroke: IEP1/78: CNS granulomas: Correlation of clinical and imaging features with histopathology at initial evaluation and follow-up at 4–6 months

Sundar U ¹, Darole P ¹, Karre A ¹, Kuchekar N ¹

Background: Central nervous system granulomas may be of varied etiologies, and worsening clinical/radiological features on treatment prompt consideration of IRIS, drug resistance, or wrong diagnosis.

Aims: The aims of this study were to correlate clinical, radiological features magnetic resonance imaging (MRI), and histopathology, at diagnosis, and 4–6 months later in case of radiological and clinical worsening.

Materials and Methods: This study was a prospective observational study, with ethical clearance and patient consent. Radiological diagnosis was made on features of size, conglomeration, T2 features, calcification, and scolex appearance. Stereotactic biopsy was done only if lesion was indeterminate, or worsening clinically and radiologically at 4–6 months, and in noneloquent area and accessible. H and E, acid-fast, and resistance studies were done.

Results: Among 30 patients (22 women), 10/30 (33%) had single and 20/30 (67%) had multiple lesions. MRI lesions over 2 cm in 4/30 (13%), conglomerate in 17/30 (57%), central T2-hypodensity in 20/30 (67%), scolex in none, and calcification on CT in 6/28 (22%) seen. A combination of T2-hypodensity, lesions over 2 cm, and conglomerate lesions was seen only in 2/30 (7%). Among seven treatment-naïve biopsied patient, six patients had TB and one NCC. Among the former, a combination of T2 hypodensity, large lesions, and conglomerate appearance was seen in only one patient. At review, 23/30 (77%) had clinical improvement and 7/30 (23%) patients worsened. Seven out of nine patients with MRI worsening had clinical worsening (78%). Biopsy of worsening lesions showed that three-fourth (75%) patients treated initially as NCC had tuberculosis.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

IEP2/215: Dengue encephalitis: Diagnosed by dengue dot sign with positive cerebrospinal fluid dengue polymerase chain reaction

Sharma A ¹, Parmar M ¹, Prajapati J ¹

Introduction: The neurological manifestations seen in dengue are encephalitis, meningitis, encephalopathy, stroke, and Guillain–Barré syndrome. Dengue encephalitis is a rare disease. Here, we present a case of dengue encephalitis diagnosed with dengue dot sign and cerebrospinal fluid (CSF) polymerase chain reaction (PCR) positivity.

Case Discussion: A 15-year-old young boy came to the hospital with a complaint of high-grade fever with chills, headache, and increased drowsiness with irritability for 2 days. On examination, the patient was conscious, oriented, irritable, power 5/5 in all limbs, sensory normal, and DTR + 2, fundus examination shows mild papilledema. MRI brain showed dot-like lesion over splenium of corpus callosum, previously described as dengue dot sign. Routine blood investigation showed thrombocytopenia. Dengue serology was negative, but dengue RT PCR was positive. CSF routine micro examination was normal. With suspicion of viral infection HSV 1 and 2, Chikungunya and dengue RT PCR performed out of these tests dengue RT PCR in CSF was positive. Although RT-PCR method is not validated for CSF, sample processed with internal control.

Conclusion: Common infections in routine clinical practice that cause fever with neurological symptoms in young age in our country are cerebral malaria, herpes encephalitis, and pyogenic meningitis. Dengue encephalitis should be considered in the differential diagnosis of fever with neurological symptoms, especially in countries like India, where dengue is rampant. Dengue encephalitis can be diagnosed with the help of MRI as well as CSF dengue PCR even though CSF routine examination is normal.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

IEP3/265: Challenges in management of Aspergillosis: A series of interesting cases

Ray S ¹, Chakravarty K ¹, Takkar A ¹, Pattanayak SN ¹, Baliani N ¹

The Aspergillus species are a ubiquitous saprophytic fungi, whose main ecological niche is in the soil or decaying vegetation. They are important contributors to global carbon and nitrogen cycling. Central nervous system (CNS) involvement of Aspergillus is one of the most severe forms of the disease in which the mortality rates can be as high as 88%. However, it is important to note that Aspergillus can occur even in the immunocompetent host and can present a big challenge to treatment. In this perspective, we present four cases to demonstrate the varied challenges in presentation and treatment of Aspergillosis. The cases look into the role of empirical therapy in Aspergillus infections, persistence of Aspergillus infection in human body despite treatment, resistance to antifungals and recurrence after long periods of inactivity.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

IEP4/273: The man with postoperative infection

Shobhana A ¹, Pradhan D ¹

Postspinal surgery infections are common. Identifying the organism from the site of infection is the key to appropriate therapy. We report one of the rare forms of postspinal surgery infection which is still bothering the patient as well as the treating physicians. A 33-year-old male presented with right-sided cervical radiculopathy. He had a C1-C2 intradural extramedullary SOL. A C1 laminectomy with excision of the tumor was done. Two weeks after discharge, he had features of meningitis. On imaging, intramuscular collection in the retrocervical region was found. The aspirate was suggestive of a purulent infection and so was the cerebrospinal fluid (CSF). He had 2 weeks of parenteral meropenem and vancomycin. Later, he had a GTCS. A hydrocephalus had developed. External ventricular drain (EVD) was placed and later the spinal wound reexplored. He had to undergo a ventriculoperitoneal (VP) shunt. Patient continued to have severe rigors. He had a history of rheumatic heart disease. A transesophageal echocardiography revealed a tiny vegetation. Meanwhile, CSF grew coagulase-negative staphylococcus; he was discharged in a stable condition. After a month, he started having gait imbalance. Brain magnetic resonance imaging (MRI) revealed a conglomerate ring-enhancing lesion in the right temporal and cerebellar region. Empirical ATD was started. After 4 months, the cerebellar lesion had gone, but fresh lesions appeared throughout the spinal cord with severe arachnoiditis and cord edema. Opinions varied. Meanwhile, he is on ATD to cover MOTT as well as antibiotics to cover fastidious organism. We plan to reimage him in about a month time now.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

IEP5/278: Idiopathic hypertrophic pachymeningitis: Case report

Dasari V ¹, Sangani HR ¹

Idiopathic hypertrophic pachymeningitis (IHPM) is a rare disorder of diverse etiology. It is rare form of diffuse inflammatory disease that causes thickening of the dura mater. It can involve the cranial or the spinal dura or both. It presents with headaches, cranial neuropathies, and ataxia occurring alone or in combination. A 40-year-old female presented with episodic binocular diplopia for 10 years, difficulty in swallowing (liquids more than solids) and nasal twang since 6 months, and headache for 1 month. In last 10 years, she had similar episodes of 5–6 times. Initially, diagnosed to have MG and started on steroids. The patient improved symptomatically later on steroids was stopped due to adverse effects. Symptoms relapsed after stopping steroids. Investigations – CSF – 90% lymphocytes, ACE level – 28.2U/L [normal]. ANA, HIV ELISA, VDRL were negative. MRI brain with contrast suggestive of smooth linear pachymeningeal enhancement overlying the right temporal convexity and tentorium cerebelli predominantly right tentorial leaflet. Meningeal biopsy revealed no significant inflammation or features suggestive of malignancy. There are no prominent plasma cells. Serum IgG 4 was normal. Diagnosis of IHPM was made after excluding all other possibilities. The patient started on steroids and azathioprine. Follow-up after 1 month had significant improvement.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

IEP6/318: Tuberculoma: A therapeutic paradox?

Muppala JP ¹, Nityanandam A ¹, Sarala G ¹, Narasimhan LR ¹

Central nervous system (CNS) involvement in tuberculosis is seen in 10% of patients, common in developing countries. In patients with tuberculous meningitis, during the course of antituberculous drug therapy, parenchymal tuberculomas may develop paradoxically. Tuberculomas in and around the optic chiasm and optic nerves is a quite rare phenomenon which may lead to severe visual loss if not treated properly. We describe a 26-year-old female presented with fever, headache, and seizures (GTCS) with documented tuberculous meningitis being treated with first-line antituberculous drugs, antiepileptics, and steroids. Initial magnetic resonance imaging (MRI) brain showed gyral thickening with white matter edema in the left frontoparietal region, diffusion restriction in left temporal region with prominent temporal horns. The patient then developed sudden painless, bilateral, and persistent visual impairment 2 months after starting the treatment. Subsequent MRI revealed multiple ring-enhancing lesions around the suprasellar and perimesencephalic region with surrounding edema compressing the optic chiasm, suggestive of multiple tuberculomas. Biopsy shows granulomatous lesion. Ethambutol toxicity and other causes for granulomatous lesions were ruled out. The patient was treated with corticosteroids, antiepileptics, and antituberculous therapy with streptomycin. The patient did not show significant clinical improvement in terms of vision and is on regular follow-up. Visual impairment developing in a patient on treatment with antituberculous drugs should give rise to a suspicion of rare optochiasmatic tuberculomas; this necessitates urgent contrast-enhanced MRI of the brain and prompt treatment with steroids.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

IEP7/373: Central nervous system involvement in syphilis

Sahu S ¹, Chhatani L ¹, Bhoi KK ¹

Ten cases of syphilis with central nervous system involvement reported. All patients had HIV negative status. Seven patients tested positive for treponemal antibody in both serum and Cerebrospinal fluid (CSF). Five patients had progressive dementia with prominent psychiatric symptoms. All developed extrapyramidal syndrome after exposure to antipsychotic medication. Two patients had meningoencephalitic illness. Neuroimaging showed cortical atrophy more marked at frontotemporal region with ventricular prominence. CSF revealed increased protein and lymphocytic pleocytosis. One patient presented with recurrent stroke. None of them improved due to advanced stage of disease. Multisegmental demyelination noted in two patients predominantly involving dorsal cord; they were tested positive in serum only. One had good recovery and other showed no improvement in deficit.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

IEP8/357: CEDNIK Syndrome: A rare syndromic intellectual disability

Prajapati A ¹, Prajapati J ¹

Introduction: CEDNIK (Cerebral dysgenesis, neuropathy, ichthyosis, and keratoderma) syndrome refers to a unique constellation of clinical manifestations including microcephaly, severe neurologic impairment, psychomotor retardation, failure to thrive, and facial dysmorphism, as well as palmoplantar keratoderma and late-onset ichthyosis caused by homozygous mutation in the SNAP29 gene.

Case Discussion: A 4-year-old male child with nonconsanguineous parents, normal delivered with birth weight of 2.3 kg, prenatally detected right ventriculomegaly on 24 weeks scan presented with severe global developmental delay, hypotonia, infantile onset seizure, facial dysmorphism, hypopigmented light color skin, and ichthyosis. On evaluation, magnetic resonance imaging brain suggestive of bilateral frontal and parietal lobe polymicrogyria with normal metabolic work up and normal karyotype. In view of cortical malformation, associated with ichthyosis and dysmorphism underlying syndromic intellectual disability was suspected and exome sequencing was sent by NGS to test all genes associated with this phenotype which showed homozygous mutation (c.418_487insA) in exon 3 of SNAP3 gene confirms diagnosis of CEDNIK Syndrome. This is a gene that is located on chromosome 22q11 and it follows autosomal recessive inheritance with recurrence risk of 25% in next pregnancy.

Conclusion: Cerebral cortical dysgenesis has been associated with many genetic syndromes. Patient with polymicrogyria or any cortical developmental malformation with ichthyosis and dysmorphism should be evaluated for underlying genetic etiology where possibility of CEDNIK syndrome should be considered. Molecular confirmation of syndrome is crucial for diagnosis, prognosis, and genetic counseling to offer prenatal diagnosis in next pregnancy.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

IEP9/350: Cerebellar ataxia due to occupational methane poisoning

Dhonde P ¹, Deshmukh S ¹, Chaudhari K ¹, Tiwari N ¹

Introduction: Occupational exposure to inhaled methane in workers of mines, sewers, and landfills poses known hazards such as asphyxia, headache, confusion, and death. Current understanding of toxicity of relatively nontoxic methane is limited to asphyxia due to oxygen displacement with scant reporting of neurotoxicity. We report a rare case of cerebellar ataxia due to methane poisoning in sewage engineer.

Case Report: A 24-year-old male presented with headache since last 6 days; after 15 days’ work in an ill-ventilated sewage plant for 10 uninterrupted hours each day. He developed drunken dysarthric voice and ataxic gait 4 days back. He was asymptomatic during his prior 2 months’ work at a well-ventilated sewage plant. Magnetic resonance imaging revealed T2/FLAIR hyperintensities without restricted diffusion in bilateral cerebellar lobes, superior cerebellar peduncles, and dentate nuclei. Other causes of ataxia and stroke were ruled out.

Discussion: Methane being water insoluble limits itself to lung parenchyma but concentrates in brain since it is also an aliphatic gas. Neurotoxic effect of methane is usually indirect, by displacing oxygen in blood. Direct toxicity is due to fluoride derivatives and methanol that alter redox milieu (NADP+/NADPH ratio) causing oxidative damage. Hypoxic-ischemic insult of cerebellum causes dose-dependent dendritic and Purkinje cell loss causing the cerebellar symptoms.

Conclusion: Time-dose relationship of exposure to sewer gases preceding the symptoms corroborated by neuroradiographic evidence of cerebellar ischemia suggests a causal role of methane in ataxia. Large occupational hazard trials are necessary to understand mechanism of methane neurotoxicity.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

IEP10/: NEP1/4: Familial Madras pattern of motor neuron disease: A case report from western Rajasthan

Prasad P ¹, Bhargava A ¹, Shubhakaran K ¹

Introduction: Madras motor neuron disease (MMND) is characterized by young onset, weakness, and wasting of the limbs, multiple cranial nerve palsies particularly involving the 7^th, 9^th to 12^th and sensorineural hearing impairment.

Case Report: A 25-year-old female of Pali, Rajasthan, presented with insidious onset gradually progressive bilateral deafness for 4 years. After 2 years, she developed dysphagia to liquid, unable to close both eyes completely followed by wasting and weakness in distal limbs 1 year later. Similar complaints were present in her brother since last 2 years. On examination, bilateral sensorineural deafness, fasciculation and atrophy of the tongue, bilateral facial involvement, and tenth cranial nerves were involved. There was wasting of the deltoid, small hand muscles, and bilateral foot drop with normal bulk in lower limbs. The plantar responses were flexor with hyperreflexia in all limbs. Sensory and gait were normal. Hematological and biochemical profile and imaging were normal. Audiometry showed bilateral cochlear deafness. Brainstem auditory evoked potential latencies were delayed, but the latencies of the visual and somatosensory evoked potentials were normal bilaterally. Electromyographic studies showed neurogenic pattern in the deltoid, first dorsal interosseous, and tibialis anterior muscles. Nerve conduction studies were normal.

Discussion: The clinical features, family history, examination, and electromyography, brainstem auditory evoked response findings were consistent with the diagnosis of familial MMND. Although the disease is rare and has a unique geographic distribution in the southern states of India, it should always be kept as a differential diagnosis of multiple cranial nerve palsies.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

NEP2/12: Peripheral neuropathy in children with Thalassemia Major: A study from tertiary care center in North India

Bala K ¹, Kaushik JS ¹, Verma A ¹, Sharma H ¹, Yadav ¹

Background: Neurological complications including peripheral neuropathy have been described in 20%–52% of adults with thalassemia major. However, its prevalence in children and impact of effective chelation is largely undetermined.

Objective: The objective of this study was to determine the prevalence of peripheral neuropathy in children aged 5–15 years with transfusion-dependent thalassemia major and to compare the electrophysiological parameters across those with effective and ineffective chelation group.

Design: This study design was a cross-sectional study.

Methods: Baseline demographic and clinical details for evidence of peripheral neuropathy were recorded. Hemoglobin and serum ferritin levels were determined. For the purpose of research, based on serum ferritin level, “effective chelation” was considered when serum ferritin value was <2499 ng/ml and “ineffective chelation” when the value was more than 2500 ng/ml. Nerve conduction study including motor (median, ulnar, peroneal, and tibial) and sensory (median, ulnar, and sural) nerves including F-wave studies were performed. Electrophysiological parameters were recorded in terms of compound muscle action potential (CMAP), sensory nerve action potential (SNAP), and conduction velocity (CV).

Results: A total of 50 children were enrolled. Mean (standard deviation) age was 10.5 (4.5) years. None of the children had either clinical or electrophysiological evidence of peripheral neuropathy. Electrophysiological parameters including CMAP, SNAP, and CV of the tested motor and sensory nerves were comparable between the two groups of effective and ineffective chelation.

Conclusion: Contrary to adults, children with transfusion-dependent thalassemia major did not show evidence of peripheral neuropathy and their nerve conduction parameters were not affected by the degree of effective chelation.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

NEP3/111: Clinical spectrum of chronic inflammatory demyelinating polyneuropathy among patients with and without diabetes mellitus in Western Rajasthan

Awasthi P ¹, Bhargava A ¹, Shubhkaran K ¹, Puri I ¹

Introduction: The present study was conducted to assess the clinical spectrum and treatment response among chronic inflammatory demyelinating polyneuropathy (CIDP) patients with and without diabetes mellitus.

Method: A hospital-based cross-sectional study was conducted among the CIDP cases admitted in the Neurology Department of MDM Hospital, Dr. SN Medical College, Jodhpur, between the period of August 2016 and June 2018. Patients having CIDP with diabetes (CIDP + DM) and without diabetes (CIDP-DM) underwent clinical examination and nerve conduction studies. The groups were compared using c2 analysis.

Results: Clinical spectrum, disease duration, and disease severity in a total of 35 CIPD patients were assessed. Out of the 35 patients, 11 were CIDP + DM and 24 were CIDP-DM. There were 26 males and 9 females. The mean duration of CIDP at the time of reporting was 10.8 ± 11.12 months. After applying Modified Ranking Scale to the treatment outcome, it was observed that there was a statistically significant difference in treatment response in CIDP-DM and CIDP + DM. CIDP-DM responded better to the treatment compared to CIDP + DM (P < 0.05). There was no significant difference in the treatment outcome among patients who were treated with oral steroids and I. V. methylprednisolone weekly. Response rate was significantly (P < 0.05) associated with the duration of disease at the time of presentation, as the patients responded better with early initiation of treatment.

Conclusion: The clinical spectrum and treatment response rate of CIDP patients differs among diabetics and nondiabetics.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

NEP4/94: Clinical profile, complications, and outcome of myasthenic crisis patients at a tertiary care center

Chalasani V ¹, Venkatsubramanian S ¹, Shanmugam S ¹

Introduction: Myasthenic crisis (MC) is a major complication of myasthenia gravis. The clinical course, therapeutic response, and complications are heterogeneous in MC. We studied the demographic, clinical, and treatment-related characteristics of patients who developed MC.

Materials and Methodology: All patients who were diagnosed to have a myasthenic crisis between August 2016 and May 2018 were included in the study.

Results: There were 19 patients who presented as the myasthenic crisis, out of whom 3 (15%) of them never had a prior diagnosis of myasthenia. The median age of patients was 65.7 years. About 16 (84%) of them are male and 3 (16%) of them were female. Nearly 16 (84%) patients presented with MC within 2 years of onset of illness. Infection was most common precipitating factor seen in about seven patients (36%) followed by cholinesterase inhibitors, noncompliance was noted in 6 (31%) patients and steroid initiation in 3 (15%) patients. Two (10%) of them were drug-induced. Mean duration of ventilator support is 8.3 days. Three (15%) patients required only BiPAP support. About 16 (85%) patients were treated with IV immunoglobulin and three patients (15%) only received plasma exchange. Cardiac complications were noted in four patients (21%). Urosepsis was seen in four patients and bacteremia was found in three patients. Mortality was seen in 2 (10%) patients.

Conclusion: This study reflects various precipitating factors responsible for the myasthenic crisis and also highlights the importance of aggressive Intensive Care Unit management and identification of various complications which help in improving the outcomes of patients.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

NEP5/209: Puzzling cases of peripheral neuropathy

Sangani P ¹, Hari RK ¹

Peripheral neuropathies can have varied presentations making it difficult to diagnose at early stages. We are presenting two cases: one unusual presentation of Hansen's disease and another case of chronic inflammatory demyelinating neuropathy.

Case 1: A 57-year-old female came with complaint of numbness of left foot for 2 years. After 3 months, she developed painless ulcers over the dorsal aspect of her left toes. Nine months later, she developed numbness in the right foot. NCS showed symmetrical sensory neuropathy in lower limbs. Hypopigmented patch over left elbow was present with normal sensation. Sural nerve biopsy showed lepra bacilli. The patient was started on steroids, rifampicin, and clofazimine and improved.

Case 2: A 63-year-old male hypertensive, presented with a complaint of left-hand numbness and dropping objects for 3 years duration. Nerve conduction studies (NCSs) showed mild asymmetrical sensory motor neuropathy. The patient was given a short course of steroids and improved. One year later, he developed right foot drop. NCS was done again and it showed severe sensory and motor neuropathy. Again steroids were given for 1 month duration and he improved. Two years later, he presented with imbalance while walking. Sural nerve biopsy was done which showed nonuniform involvement of fascicles with fiber loss with remyelination indicating acquired demyelinating neuropathy suggestive of chronic inflammatory demyelinating polyneuropathy. The patient was advised IVIG.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

NEP6/214: Spectrum of peripheral nerve vasculitis at tertiary care center

Chhirolya R ¹, Kalita J ¹, Misra U ¹

Background: Due to the paucity of studies on peripheral nerve vasculitis, we report the clinical spectrum, treatment response, and outcome.

Subjects: Patients with vasculitic neuropathy (VN) were taken from vasculitis registry. Vasculitis was diagnosed by respective diagnostic criterion and categorized into three groups: Group-I (nonsystemic VN), Group-II (VN with systemic features), and Group-III (VN with CNS involvement). Their demographic, clinical, and laboratory parameter were recorded. They were treated with prednisolone with or without disease-modifying therapy. Their disability was assessed on admission and follow-up (3 and 6 months) with the Modified Rankin Scale (MRS). Outcomes were termed as significant improvement/minimal improvement (MRS ≤ 4/≤2), respectively.

Results: There were 24 patients with a median age of 53.5 (24–70) years with females (58.3%). Onset was acute (57.1%) in Group-I and subacute/chronic (76.5%) in Groups-II/III. Nerve biopsy was suggestive of VN in 10 (83.33%). Disability was mild-to-moderate (MRS ≤ 3) in Groups-I/II, severe (MRS ≤ 5) in Group-III at admission. All patients in Group-I were treated with prednisolone only while half of the patient in Group-II/III required DMT. On 3 months, significant improvement was seen in 85.7/71.4/0%, minimum improvement in 14.3/28.6/100% in Groups-I/II/III, respectively. On 6 months, significant improvement was seen in all patients of Groups-I/II/III.

Conclusion: Patients in Group-I presented early had the mild disability, responded to prednisolone alone compared to Groups-II/III.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

NEP7/240: A 50-day follow-up study on the effect of edaravone on motor neuron disease with respect to ALS-FRS (revised) score

Raj JP ¹, Shaji CV ¹

A total of 12 patients were enrolled. They were evaluated before giving edaravone, after 3^rd and 6^th doses of edaravone and 10, 25, and 50 days after completion of the course of the drug. They were evaluated using ALS-FRS revised score. Maximum score is 48. Score of 40 or below considered as severe disease, 41–44 as moderate, and 45 or more as mild disease. All were given edaravone 60 mg per day for 10 days. Such 6 cycles were given 2 weeks apart. Average initial score was 39.5 with males having 39 and females having 40.5. Bulbar and limb onset had equal average of 39.5. After 6 cycles of edaravone, total average increased. Bulbar onset had 39.75 and limb onset had 40.375. After 50-day follow-up, three patients expired. One was having bulbar and the other two having limb-onset disease. All were having ALS-FRS revised score <40 initially. Remaining showed that bulbar onset had average of 34.33 limb onset had 41.0. Severe diseases patients initially showed average improvement of 1 score after 6 cycles. On follow-up of 50 days, 2 out of 4 expired and the remaining showed decrease of 1.5 score on average.

Conclusion: This study shows that there is a transient subjective improvement with the use of edaravone. However, 50-day follow-up shows that this improvement persists in only mild and moderate diseases of limb onset. Severe disease and bulbar onset variety showed rapid deterioration after stoppage of medication.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

NEP8/263: Follow-up analysis of clinical and electrophysiological parameters in patients with Guillain–Barre Syndrome: A short-term study

Jayaharan N ¹, Shanmugasundaram N ¹, Balasubramanian S ¹, Sarala G ¹, Ranganathan L ¹

Background: Guillain–Barre syndrome (GBS) is a common cause of acute flaccid paralysis. Electrophysiological studies are used at various stages of the disease. In this study, a electrophysiological assessment during course and follow-up was undertaken to assess the changes and correlate with the clinical course.

Methods: This study included 15 patients with GBS admitted in the period between November 2017 and April 2018 in Madras Medical College, Chennai. Clinical examination was done in these patients and the Medical research council (MRC) score and Hughes disability score were noted at admission and 3-month follow-up. Electrophysiological parameters studied included distal latency, compound muscle action potential (CMAP), conduction velocity and F waves. A comparison of the findings noted before and after 3 months was studied.

Results: In this study, GBS occurred in ages between 23 and 60 with a male-to-female ratio of 1.1:1. The mean Hughes disability score at admission was 4.25 with the average MRC score being 23. Electrophysiologically distal latency was prolonged, CMAP amplitude was reduced, conduction velocity was reduced, and F-wave was prolonged/absent in at least one nerve in 14 patients. The mean disability score after 3 months was 1.75 and the mean MRC score was 51.91. Among the 13 patients showing improvement, distal latencies had normalized in all nerves in 4, CMAP in 3, conduction velocity in 4, and F-wave in 4 patients.

Conclusion: In our study, we observed that despite adequate clinical improvement, electrophysiological abnormalities persisted in majority of the patients.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

NEP9/314: Hirayama disease: A case series from a tertiary care center

Vemuri R ¹, Venkata SN ¹, Veeramma U ¹

Background: Hirayama disease is benign focal amyotrophy of the upper limbs, affecting primarily young males in second to third decades of life. It is often misdiagnosed as motor neuron disease. Routine magnetic resonance imaging (MRI) is often reported as normal.

Aims and Objectives: The aims of this study were to study the clinical, radiological, and electrophysiological profile of upper limb wasting confirmed as Hirayama disease after exclusion of other causes.

Materials and Methods: Patients presenting with upper limb wasting and weakness between September 2017 and July 2018 were evaluated. Thorough clinical examination was performed and other causes of upper limb monoparesis such as plexopathy, stroke were excluded from the study. Imaging and ENMG were done in all the cases.

Results: A total of five cases were studied. All patients were males and were between 19–29 years of age. Upper limb wasting was present in all cases. Polyminimyoclonus was seen in two cases. Duration of illness ranged from 1 year to 3 years. MRI in neutral position showed loss of cervical lordosis in three cases and normal in two cases. MRI in flexion position showed prominent posterior epidural space with anterior displacement of dural sac. Focal cord atrophy seen in two cases. Electromyography showed denervation changes in affected myotomes.

Conclusion: All patients were in the second and third decades of life. There is preferential involvement of ulnar innervated muscles. Electrophysiology was suggestive of chronic denervation due to anterior horn cell involvement. Early collar therapy prevented progression.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

NEP10/AWPN12: Electrophysiological and Leeds Assessment of Neuropathic Symptoms and Signs Pain scale score relationship in patients with carpal tunnel syndrome

Kuppusamy K ¹, Govindharajan S ¹, Balasubramanian S ¹, Shanmugasunsundaram K ¹, Lakshminarasimhan ¹, Jayakumar H ¹, Lakshminarasimhan S ¹

The aim of the study was to study the relationship between the presence of neuropathic pain assessed by the Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) scale and electrophysiological findings in patients with carpal tunnel syndrome (CTS).

Methods: We studied 50 cases of idiopathic CTS with pain complaints involving hand and wrist. All cases were assessed by the LANSS with which a score of 12 or more is defined as pain dominated by neuropathic mechanisms. These hands were assigned to minimal, mild, moderate, severe, or extreme severe groups according to the results of the median nerve conduction studies.

Results: A LANSS score ≥12, suggestive of pain dominated by neuropathic mechanisms, was defined in 50 CTS hands. Pain intensity was significantly higher in CTS hands with a LANSS score ≥12 (P < 0.001). Among electrophysiological findings, compound muscle action potential amplitude was significantly lower and in hands with a LANSS score ≥ 12 compared with hands with a LANSS score <12 (P = 0.002). Severity of CTS was significant between LANSS ≥12 and LANSS <12 groups. Electrophysiological severity is significantly correlated with LANSS pain scale score.

Conclusion: We suggest that the presence of pain dominated by neuropathic mechanisms in CTS is significantly related to electrophysiological CTS severity. Neuropathic pain should be assessed carefully in patients with CTS. The detection of neuropathic pain is useful for selecting the optimal medication and surgical treatment.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

NEP11/AWPN13: How the phenotypic variants differ from classical Guillain–Barre syndrome

Manasa A ¹, Sreerangalakshmi G ¹, Ramesh ¹, Archana B ¹, Anilkumar T ¹

Background: Classical Guillain–Barre syndrome (GBS) and its variants exist as several subtypes with different neurological features and presentations. Although the typical clinical features of GBS are well recognized, current classification systems do not comprehensively describe the full spectrum of variants. There are no definitive diagnostic criteria for phenotypic variants. In this study, we used criteria from multiple studies. This could facilitate early clinical diagnosis and initiation of appropriate immunotherapy.

Objectives: The objectives of this study were to differentiate classical cases of GBS from variants and describe their detailed clinical and electrophysiological features.

Materials and Methods: study design: This study design was a observational study. 75 patients admitted for acute-onset rapidly progressive weakness in Osmania General Hospital from January 2017 to May 2018.

Results: Classical GBS – 60 cases, variants – 15 cases. Antecedent events: 42 in classical, 4 in variants. Cranial nerves – 44 in classical, 5 in variants. CSF albuminocytological dissociation: 44 of classical, 6 of variants. NCS – classical [demyelinating-31, axonal-19], variants – [demyelinating-7, axonal-2]. Six of variants had normal NCS, they were diagnosed by means of absent H reflex, abnormal facial conductions, and prolonged temporal dispersions.

Discussion: Acute inflammatory demyelinating polyneuropathy is most common. AMSAN has more severe presentation. Variants had milder presentation, mostly spontaneous recovery, and good prognosis.

Conclusion: Early identification and treatment of variants are necessary for proper management. New variants like GBS with retained reflexes need to be identified.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

NEP12/: NEP13/AWPN21: Role of ultrasonography to monitor carpal tunnel syndrome patients treated with local corticosteroid

Wagh SJ ¹, Chakkalakkoombil SV ¹, Nair PP ¹, Barathi D ¹, Kumar H ¹

Introduction: Assessed the role of ultrasonography (USG) in monitoring carpal tunnel syndrome (CTS) patients treated with corticosteroid injection.

Methods: Included CTS patients who underwent USG before and after treatment. Cross-sectional area (CSA), anteroposterior diameter (APD) of median nerve at the carpal tunnel (CT), clinical and electrophysiological data were collected from records. Assessed whether changes in USG measurements before and after treatment correlated with clinical and electrophysiological parameters and their significance.

Results: Included 39 wrists for 1 month and 17 wrists for 3 months analysis. Boston symptom severity score, functional score, motor latency, sensory velocity, differences in motor, and sensory latency showed significant changes over 1 and 3 months. Although changes in CSA and APD correlated with some clinical and electrophysiological parameters, they were insignificant.

Discussion: USG parameters APD and CSA of median nerve at CT inlet were not useful to monitor CTS patients treated with corticosteroid injections.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

NEP14/AWPN24: Importance of A waves in predicting recovery in Guillain–Barre Syndrome

Lakshminarasimhan S ¹, Chandramouleeswaran V ¹, Kannan V ¹, Balasubramanian S ¹, Sarala G ¹, Jawahar M ¹, Lakshminarasimhan R ¹

Background: A wave is a late response recorded between the motor and F responses. They have been described to occur in polyradiculoneuropathy. There have been few studies describing the significance of A waves in Guillain–Barre syndrome (GBS).

Objective: The objective of this study was to study the characteristics of A waves in acute inflammatory demyelinating polyradiculoneuropathy and determine their significance in clinical recovery.

Methods: 84 patients with acute inflammatory demyelinating polyradiculoneuropathy were enrolled in this study. Their clinical features, electrophysiological features, and disability grading at admission and at 6-week follow-up was assessed.

Results: A total of 58 patients (69%) had the presence of A waves in at least one nerve studied. Pattern of A waves in descending frequency was multiple A waves, A waves in the absence of F waves followed by A waves occurring between motor and F response. Tibial nerve was the most common site of occurrence of A waves. Patients with A waves had higher Hughes grade (P = 0.003) and lower MRC sum score at 6-week follow-up (P = 0.04) as compared to patients without A waves.

Discussion and Conclusion: A waves are a common occurrence in demyelinating form of GBS. Their presence correlated with prolonged time to recovery in short-term follow-up. Further long-term follow-up is necessary to determine the clinical course in these patients.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP41/231: Polycythemia and ischemic stroke

Umachandran P ¹, Sundaram SN ¹, Samivel B ¹, Ranganathan LN ¹, Venkatraman C ¹

Objective: The objective was to study the clinicoepidemiological and radiological aspects of ischemic stroke associated with polycythemia.

Methodology: Patients admitted in Madras Institute of Neurology, with arterial or venous stroke with polycythemia, were included in the study. Clinical, laboratory, and radiological data were analyzed.

Results: A total of 42 patients with polycythemia were studied. Median age was 42.5 years (range: 20–72). Out of 42 patients, 30 (71.4%) were smokers. 36 patients (85.7%) had arterial stroke (AS), and 6 (14.2%) had venous stroke (VS). Mean hemoglobin was 18.36 ± 0.88 g/dL in AS and 19.12 ± 1.66 gm/dL in VS. Mean PCV was 53.14% ± 3.41% in AS and 55.38% ± 4.73% in VS. Mean serum erythropoietin measured in 24 patients was 3.43 ± 1.73 mU/mL. Recurrent CVA with AS occurred in 15 patients (41.7%). Nine patients (25%) had multiple or bilateral infarct. Out of 36, AS patients 9 (25%) had ASPECT score <7 (100% – smokers), 27 (75%) had ASPECT score >8 (66.6% smokers). About 28 patients (77.8%) had NIHSS score <10 (67.8% smokers) and 8 (22.2%) had NIHSS >10 (100% smokers). Using TOAST classification, 19 (52.8%) had small vessel disease and 16 (44.4%) had large vessel disease.

Conclusion: We observed that in patients with polycythemia and stroke, age of onset is younger, AS is commoner than VS, multiple and small vessel infarct are common (25%). Recurrence rate is higher (41.7%). Although high ASPECTS and low NIHSS score were observed in majority of patients (75%), large infarcts were more commonly noted in smokers.

Ann Indian Acad Neurol. 2018 Sep;21(Suppl 2):S72–S114.

SEP56/AWPN04: Triad of clinical findings localizes to cortex

Vijayan BV ¹, Gourisanker PT ¹, Vijayan CP ¹, George J ¹

Introduction: Cortical lesions cause a variety of deficits. Somatosensory cortical lesions cause subjective numbness of face and pinna, differential sensory impairment of face and pinna, with preserved touch, impaired pain and temperature sense, and impaired corneal reflex. These findings help in localization and redefine sensory homunculus.

Background: Cortical stroke presenting as isolated numbness of face leads to identification of nine cases.

Materials and Methods: Stroke patients presenting as facial and ear numbness without aphasia or long tract signs with MRI brain showing cortical infarcts were included in the study. Index patient presented at 2014. The last patient recruited was in June 2018.

Results: Ten patients, including index, had subjective facial with pinna numbness, differential facial sensation of preserved touch, but impaired pain and temperature appreciation, severe over lower/middle part and mild over upper face. Corneal reflex was affected in all and pinna in nine. Spastic hand was seen in seven. Recovery for upper face and corneal reflex was early and for lower face and hand was late.

Discussion: Cortical stroke can cause LMN-like (trigeminal neuropathy-like) clinical features. Pinna involvement, facial weakness, sparing motor trigeminal, and frequent co-occurrence of spastic hand are clues for cortical localization. Primary or secondary somatosensory cortex lesions cause these deficits, which have neurophysiologic and anatomic significance.

Conclusion: Awareness of trigeminal neuropathy-like stroke presentation should be emphasized (diagnostic errors are common). Abundant extraocular muscle proprioceptive and dense corneal sensory afferents cause extensive bilateral representation of upper face and eye, resulting in mild upper facial involvement with early recovery. It is relevant to seek if upper face (with eye) has equal area of somatosensory cortical representation as lower face (with lips and tongue) in sensory homunculus.

PERMALINK

Friday, September 28, 2018, 09:00-10:00 hrs, E-Poster designated area: E Poster Session – 01: Miscellaneous Disorders

MiEP1/51: Van der Knaap disease: A case report

Bhattiprolu RK

Sardana V

MiEP2/83: Etiological profile of noncompressive myelopathies in a tertiary care hospital in Central Tamil Nadu

Jayasankar VR

Thangaraj M

Balamurali K

MiEP3/133: An interesting case of a child with spastic diplegia

Kumar S

Velayutham S

Jeyaraj M

Sowmini PR

Arunan S

MiEP4/162: Traumatic carotid-cavernous fistulas: Can we wait?

Nagendra S

Soni G

Gutte A

Jagiasi K

Patil S

MiEP6/217: A case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids with spinal cord presentation

Aglave V

Faldu H

Ojha P

Jagiasi K

Soni G

MiEP7/241: Intracranial plasmacytoma associated with multiple myeloma presenting as multiple cranial nerve palsies: A case report

Mohan CK

Perumal SS

Velayutham S

Jeyaraj M

Mohan A

MiEP8/257: A rare cause of Parkinsonism-manganese toxicity

Yadav PK

Roy P

MiEP9/259: Synaptic nuclear envelope protein 1 ataxia: The first genetically confirmed case from India and a novel homozygous mutation in the SYNE 1 gene

Kothari K

Chakor R

Nadaf S

Patil A

Bharote H

MiEP10/274: Sickle-cell anemia in association with Moyamoya syndrome: Revisited

Dixit NS

Sharma S

Sharma P

MiEP11/296: Headache and neurological deficits with cerebrospinal fluid lymphocytosis: Case series

Patil S

Ojha P

Soni G

Barvalia P

Aglave V

MiEP12/322: Rare late-onset presentation of glutaric aciduria type I

Reddy PV

Kannan V

Chandramouleeswaran V

Narasimhan LR

MiEP13/329: Autosomal recessive spastic ataxia of Charlevoix–Saguenay: A report of a novel variant from South India

Murali S

Kumar PS

Netto A

Nagaraj K

Janardhan DC

Abhinandan KS

MiEP14/330: An unusual case of mitochondrial myopathy due to complex 1 deficiency with acute exacerbations responding to intravenous immunoglobulin: Case report

Awatare P

Murgod U

MiEP15/335: Hemiparkinsonism hemiatrophy: An interesting case for the discerning eye

Chakravarty K

Ray S

Pattanayak SV

Baliani N

Takkar A

Mehta S

Lal V

Friday, September 28, 2018, 13:00-14:00, E-Poster designated area: E Poster Session – 02: Miscellaneous Disorders: SEP1/19: Sex differences in risk factor profile, stroke subtype, and outcome in acute ischemic stroke

Mitta N

Sylaja PN

Sreedharan SE

Sarma PS