Annals of Indian Academy of Neurology
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Year : 2019  |  Volume : 22  |  Issue : 5  |  Page : 45-157


Date of Web Publication19-Sep-2019

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How to cite this article:
. Abstract. Ann Indian Acad Neurol 2019;22, Suppl S1:45-157

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. Abstract. Ann Indian Acad Neurol [serial online] 2019 [cited 2020 Jan 27];22, Suppl S1:45-157. Available from:

Friday, October 04, 2019, 13:30 PM-20:00 PM

Poster Session 1: Award Posters; Poster Hall Area;

PP1/28: Patient zero: Containment at source

Sundararajan AR, Maramattom BV

Department of Neurology, Aster Medcity, Kochi, Kerala, India

This is a 21 year old male, an engineering student, who presented with fever of 7 duration associated with involuntary jerky movements and gross ataxia of limbs and trunk. Standard infection parameters were found to be negative and MRI shows scattered micro infarcts all over the cortex and pons bilaterally in diffusion weighted sequence. CSF Encephalitic panel turned out to be NIPAH positive. This abstract is about the presentation of Nipah encephalitis and the acute management in hospital and prevention of outbreak.

PP2/30: Autoimmune glutamic acid decarboxylase 65 antibody associated cerebellar ataxia

Chinta VR, Murgod U, Maloo R

Department of Neurology, Manipal Hospital, Bengaluru, Karnataka, India

Introduction: The cerebellum is a vulnerable target of autoimmunity in the CNS. The category of immune-mediated cerebellar ataxias (IMCAs) was recently established, and includes in particular paraneoplastic cerebellar degenerations (PCDs), gluten ataxia (GA) and anti-GAD65 antibody (Ab) associated-CA, all characterized by the presence of autoantibodies. We hereby present one such patient. History: A 36 year old female patient presented to MHB following complaints of double vision, incoordination while walking; and difficulty in reaching for and holding objects for the past few weeks. This was insidious in onset, bilateral and slowly progressive. There was no weakness of limbs or cognitive decline or memory disturbances or bowel and bladder symptoms. There was no antecedent illness or fever or trauma or drug use or family history of such illness. Examination: On examination she was conscious, alert and oriented. Higher Mental Functions were normal. Pupils were bilateral equal and reactive to light. EOM's were full. She had bilateral horizontal gaze evoked nystagmus. Motor examination showed, normal bulk, tone, power, reflexes with bilateral upper limb ataxia left more than right. There were no sensory abnormalities. She had ataxic gait, requiring support to walk even a few steps. Methods/Investigations: She was extensively worked up for the cause of her neurological symptoms. Routine blood tests including CBC, Renal profile, TSH, Liver functions, B12 were normal. Autoimmune workup was negative. MRI brain plain and contrast was normal. LP CSF was unremarkable. NMO/MOG antibodies was negative. PET CT was normal. GAD 65 antibodies were strongly positive. Tissue Transglutaminase Antibodies were weakly positive. Hence she was diagnosed to have autoimmune GAD 65 antibody associated cerebellar ataxia. Treatment and Follow-up: She was initially treated with pulse IVMP followed by an oral taper. She showed moderate improvement of her symptoms. However over the next few months she had worsening of her symptoms after the tapering of steroids. This time she was diagnosed to have uncontrolled diabetic ketoacidosis which required inpatient therapy. She was commenced on IVIG 2G/Kg body weight infused over 5 days after adequate sugar control was achieved. She had mild improvement of diplopia and gait ataxia over the next month, but still required assistance to walk. Following this she was commenced on IV Rituximab given at a dose of 1gm twice 15 days apart. She had slow progressive improvement of her symptoms. Currently she persists to have mild limb ataxia and mild gait ataxia, but is independent for ADL's. She is off steroids, but requires increasing doses of insulin for her diabetic control. Summary: This patient highlights a few of the disorders which present in the spectrum of disorders in patients presenting with GAD (Glutamic Acid Decarboxylase) 65 positivity and the challenges in their immunomodulatory treatment. GAD 65 antibody positivity is known to be associated with a variety of neurological and non-neurological manifestations including Autism, Type 1 DM, LADA, Cerebellar Disorders, Stiff Person Syndrome and Neuropathic pain.

PP3/35: A study of clinical spectrum of dystonia in a tertiary care center of India

Prasad R, Joshi D

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

Aims and Objectives: To study the clinical charecteristics of patients with Dystonia attending the neurology OPD or admitted in neurology ward of Sir Sundarlal Hospital, IMS-BHU, Varanasi which primarily caring for patients coming from Eastern part of India from Jan 2018 to Jan 2020. Materials and Methods: All the consecutive Dystonia patients fulfilling the inclusion criteria were subjected to a detailed clinical history, physical and neurological examination as per the standard protocol prepared by us after an informed consent. Neuroimaging were performed in selected patients according to need. Patients were classified according to the consensus update on phenomenology and classification of dystonia (Axes 1 and 2). In Axis 1 patients were classified according to: Age distribution [Infancy (birth to 2 years), Childhood (3–12 years), Adolescence (13–20 years), Early adulthood (21–40 years)], Late adulthood (>40 years), Body distribution (focal, segmental,multifocal,hemidystonia and generalized), Variability (persistent, action, diurnal &paroxysmal), Isolated or combined with other movement disorder or other neurological and systemic disease. In Axis 2 classified as: Neurodegenerative or structural, Inherited or acquired. We calculated the proportion of patients classified into one of the defined dystonia syndromes: Early-onset isolated generalized dystonia, Focal or segmental isolated dystonia with onset in adulthood, Dystonia  Parkinsonism More Details and myoclonus dystonia. Results: Till now a total of 183 patients with dystonia were included with 1.6:1 male & female ratio. Mean age of onset of dystonia were 35.9 years (9 months to 91 years) with average duration of illness was 4.67 years (2 days - 38 years). Age of onset of dystonia was infancy in 3.8% of patients, 15.3% had childhood onset, 12% were adolescent at onset, 25.2% were in early adulthood and 43.7% had late adulthood onset. Among them Focal were most common (48%) followed by segmental (22.4%) then generalized in 19.12% and among focal, brachial (48.8%) were most common, followed by blepharospasm (26%) and least were cervical (2.27%). Task specificity was documented in 48.8% of focal dystonias. Diurnal variation was found in 1.1% of cases and 2.2% had paroxysmal dystonia. Tremor was present in 38.2%. Among total patients 71.6% patients had isolated, 3.82% had combined dystonia and other neurological and/or systemic disease were present in 24.6% of patients. Etiologically (Axis 2), 8.2% patients had positive family history, Neurodegeneration as a cause was present in 11.5% and most of them were wilson's disease (81%), Acquired causes were found in 26.7%, among them drug was most common cause (32.65%) followed by Birth insult & vascular causes (22.4% each). Two patients had early onset generalised dystonia,10.4% patients came under dystonia parkinsonism syndrome (only two patients had clinical suspecion of dopa responsive dystonia) and focal or segmental isolated dystonia with onset in adulthood was found in 44.3% of patients. Conclusion: Focal or segmental isolated dystonia with onset in adulthood was most common dystonia syndrome. Writer's cramp was the most common isolated dystonia and Wilson's disease was most common degenerative cause of dystonia.

PP4/44: A case of early onset cerebellar ataxia with cerebellar atrophy: A less recognized entity

Mahale R, Reddy N, Pavagada M

Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

The Deoxyribonucleic acid (DNA) damage response (DDR) or DNA repair defects cause rare inherited diseases, termed as ''DNA-repair defects” or ''DNA-repair deficiency”. Among them is ataxia telangiectasia (AT). The ATM (Ataxia –telangiectasia mutated kinase) gene mutated in AT is central to DDR signalling. Other genes implicated in DDR signalling are MRE11A (Meiotic recombination 11) and NBN (NBS1) genes 1. Mutation of these genes results in ataxia-telangiectasia-like disorder 1 (ATLD1) and Nijmegen breakage syndrome respectively. The clinical presentation of ATLD is similar to AT but without telangiectasia, raised serum alpha fetoprotein and immunodeficiency 2. Here, we report a boy who presented with mild cerebellar ataxia and dystonia with cerebellar atrophy on brain imaging. Clinical exome sequencing showed compound heterozygous variants in MRE11 gene. He was diagnosed as ATLD which has not been reported in Indian subcontinent so far. A 14-year-old boy born out of non-consanguineous parentage with normal perinatal history and motor and language milestones was brought with the history of imbalance while walking since the age of 6 years. The imbalance while walking was insidious in onset and slowly progressive for 3-4 years and static till the presentation to us. There was no diurnal variation. He was able to play with peers. There was no history of incoordination of upper limbs, speech disturbance, tremulousness of head, abnormal posturing of limbs, myoclonus or seizures. He had younger male sibling of 6 years of age who was asymptomatic. There was no history of similar complaints in other family members. Systemic examination was unremarkable. There was no oculocutaneous telangiectasia, cataract, tendon xanthomas, scoliosis or high arched feet. Neurological examination showed normal cognition and speech. Fundus examination was normal. Visual acuity and visual fields was normal. There was no external ophthalmoplegia. Vertical and horizontal saccades had normal latency, velocity but were hypometric. He had horizontal and vertical saccadic pursuits. Other cranial nerves were normal. Motor examination showed hypotonic limbs with hyporeflexia. Muscle power was grade 5/5 in both upper and lower limbs. Sensory examination was normal. There was mild finger-nose and knee-heel incoordination on both sides. Tandem gait was impaired. Gait was slightly wide-based and ataxic. Romberg's sign was negative. Plantar response was flexor. There was mild finger dystonia of both outstretched hands with mild fidgetiness while sitting. Serum total creatine phosphokinase was normal. Serum alpha fetoprotein, immunoglobulins G, A and M and vitamin E levels were normal. Nerve conduction study was normal. Brain magnetic resonance imaging (MRI) showed pan cerebellar atrophy (Figure 1). Clinical exome sequencing showed compound heterozygous variants in the MRE11 gene. A heterozygous 5´ splice site proximal variation in intron 4 of the MRE11 gene that affects the positions 4 to 7 nucleotides downstream of donor splice site of exon 4 (c.314+4_314+7 del) was detected. The sequencing of variant was not carried out in parents in order to confirm the significance due to financial constraints.

PP5/46: An unsual case of acute onset quadriplegia

Sharma S, Panda S, Jain S, Tiwari S

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

Background: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterised by insidious onset and slow progression over 2 months. However upto 16% of CIDP may show acute presentation similar to Guillain Barre Syndrome (GBS), hence termed Acute-onset CIDP (A-CIDP). Case Report: A 18-year-old male presented with low back pain and weakness of both lower followed by upper limbs without sensory, cranio-bulbar, bladder or respiratory involvement for 11 days. There was generalized hypotonia, loss of muscle bulk and generalized areflexia with signs of meningeal irritation. Proximal power was 2/5 upper limbs, 1/5 lower limbs, 0/5 distally. Initial diagnosis of axonal variant of GBS was made. CSF was abnormal [pressure 28cm H2O, turbid with coagulum, protein 5.9 gm/dL, sugar 68 mg/dL (RBS-92), cell count 45 (all lymphocytes)]. Nerve conduction study suggested severe degree of distal and proximal, symmetrical small and large fibre motor axonal polyradiculoneuropathy. Serum ganglioside antibodies were negative. Contrast-enhanced MRI whole-spine revealed enhancement of multiple nerve roots in lower dorsal and cauda equina region suggestive of polyradiculopathy. Revising diagnosis to A-CIDP, patient was started pulse methylprednisolone followed by oral steroids. Repeat CSF (after 7 days) showed protein 120 mg/dL, sugar 86 mg/dL, and cell count 2 (all lymphocytes). There was progressive improvement and patient was ambulatory with 1-person support after 1 month with concomitant improvement in nerve conduction and MRI studies. Conclusions: Differentiating GBS and A-CIDP maybe challenging. Clinical, CSF, electrophysiological and neuroimaging features and response to treatment will give clues for A-CIDP. This case suggests further possible variant of A-CIDP with motor axonal involvement of acute onset, very high CSF protein and remarkable response to steroids.

PP6/56: Clinico-radiologic spectrum of MOG-IGG associated disease from a tertiary care institute

Agrawal S, Ravat S, Jain N

Department of Neurology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, Maharashtra, India

Introduction: The clinical spectrum of MOG-IgG-associated disease has expanded from paediatric patients with acute disseminated encephalomyelitis (ADEM) to adult patients presenting as NMO-SD. It has predominantly relapsing course and significant visual system damage after clinical optic nerve damage. Indian data on this new disease is insufficient. It is important to diagnose this disease as treatment strategy is different from other demyelinating diseases particularly MS which is a close differential. Materials and Methods: 10 adult (Age >12 years) patients who presented to a tertiary care institute from august 2017 to May 2019 with NMO-SD and were seropositive for MOG-IgG antibodies were prospectively evaluated for clinical and radiologic spectrum and response to treatment. Results: 70% (7/10) had monophasic and 30% (3/10) had a relapsing course. 70% (7/10) patients had optic neuritis either alone or in combination with LETM. 50% (5/10) had only optic neuritis, 10% (1/10) presented with only LETM, 10% (1/10) with brainstem demyelination and 10% (1/10) with cortical lesions. 20% (2/10) had LETM and optic neuritis. 30% (3/10) had relapsing optic neuritis prior to diagnosis. All patients who had relapsing course had optic neuritis. Only 10% (1/10) patients had bilateral ON at presentation. Patients on immunosuppression with a mean 12 months follow-up did not show a single relapse. Conclusion: This study reflects MOG-IgG-Ab associated disease data from India. Majority have monophasic illness which suggests a better prognosis than other demyelinating disorders. It is important to diagnose these patients as it will prevent incorrect labelling as multiple sclerosis and wrong treatment in the form of initiation of DMDs. Limitations: Small sample size and short follow-up owing to a government set-up with limited resources. Further Scope: Long term study with more number of patients and their prospective follow up will identify the course of disease with and without second line immunosuppression. There is an immense scope in India with regards to MOG-IgG associated diseases as they are relatively new and uncommon than other demyelinating disorders.

PP7/58: Superior orbital fissure syndrome: A case series

Badveli A, Gadad V, Krishnamurthy L

Department of Neurology, S.S. Institute of Medical Sciences and Research Centre is a Medical School, Davanagere, Karnataka, India

Introduction: Superior Orbital Fissure Syndrome (SOFS) is a clinical entity caused by the inflammation of structures anterior to orbital apex, presenting as multiple cranial nerve palsies viz nasociliary nerve, oculomotor nerve complex and ophthalmic vein. Etiologies include Tolosa–Hunt syndrome, infectious, neoplastic, iatrogenic/traumatic, or vascular processes. Aim: To study the clinical presentation, etiology and treatment outcome of SOFS. Methods: We report 5 cases of this rare entity selected from patients attending the outpatient department at our tertiary care centre from October 2018 to March 2019. Results: All the patients presented with a subacute onset of symptoms with painful ophthalmoplegia. On examination, they had varying involvement of unilateral 3rd, 4th and 5th cranial nerves. CSF analysis of all 5 patients was normal. Neuroimaging findings of two patients was normal, while 3 patients had contrast enhancing signal intensity changes of superior orbital fissure extending to cavernous sinus and/or orbital apex. All the patients were treated with intravenous methylprednisolone for 5days followed by oral steroids which were gradually tapered over a period of three months and stopped. All five patients had complete recovery over next three months with no residual deficits. Conclusion: Most cases of SOFS are idiopathic or inflammatory and respond well to corticosteroids with complete clinical recovery. Treatable causes need to be ruled out before considering neoplastic etiology. Early recognition of the symptoms and prompt treatment will result in better outcome.

PP8/63: Varicella myelitis: A case series from a tertiary care center

Choudhary A, Khichar S, Bhargava A, Puri I

Department of Neurology, Dr. S. N. Medical College, Jodhpur, Rajasthan, India

Introduction: Myelitis has been rarely seen after varicella infection in adults with a frequency of 0.3%. It has been postulated to occur either as a direct viral invasion of the spinal cord or through an immune mediated allergic mechanism. No diagnostic test is completely accurate, since VZV cannot usually be isolated from blood or cerebrospinal fluid (CSF) in VZV myelitis. We had unusually high number (four) of patients presenting with varicella myelitis in a short period. Aims and Objectives: The aims of this study were to observe and analyze the clinical, radiological profile and therapeutic response in patients presenting with myelitis after varicella infection. Materials and Methods: Patients presenting with myelitis during or after varicella infection between January 2019 to May 2019 were evaluated. Patient developing weakness within 2 week of rash onset and MRI spine showing myelitis were considered as varicella myelitis. Serum VZV antibody was tested in patients. All patients were treated with acyclovir, methylprednisolone. Results: A total of four cases presented between january-may having myelitis after varicella infection. Three were female and one male, with age between 17 – 30 years. The duration of developing myelitis after onset of varicella was 4 days to 14 days. Three patients presented with quadriparesis and one with paraparesis, all four patient had long segment myelitis. Only two patients could afford serum VZV antibody test and both of them tested positive. One patient improved with steroid and acyclovir, two patients didn't show any improvement with them so were given cyclophosphamide after which they responded .One patient developed ascending demyelination and expired. Conclusion: In our study, we observed that myelitis developed between 4-14 days after primary varicella infection. All patients had long segment myelitis and female to male ratio was 3:1. Two patients improved after cyclophosphamide who didn't respond to steroid and acyclovir suggesting plausible immune mediated mechanism for myelitis. Ascending demyelination lead to death of one patient. Limitations: VZV antibodies couldn't be done afforded by 2 patients. Further Scopes: Since neurological complications in adult onset varicella is no longer rare and even can lead to mortality, adults should be vaccinated against this preventable disease.

PP9/72: A case of chicken pox presenting as ischemic stroke due to vasculopathy: Rare neurological complication of varicella

Lakesar A, Khichar S, Bhargava A, Puri I

Department of Neurology, Dr. S. N. Medical College, Jodhpur, Rajasthan, India

Vasculopathies caused by varicella zoster virus (VZV) are result of a productive virus infection in cerebral arteries after either reactivation of VZV (shingles) or primary infection (chickenpox). Vasculopathy can cause infarction of the brain and spinal cord, as well as aneurysm, subarachnoid and cerebral haemorrhage, carotid dissection, and, rarely, peripheral arterial disease. VZV vasculopathy can be unifocal or multifocal, deep-seated or superficial infarctions. Grey–white matter junction lesions on neuroimaging favour diagnosis. Large and small artery vasculopathy is more common than that of either alone.Mostly patient have CSF pleocytosis, often with RBCs. Anti-VZV IgG antibody in CSF is found more frequently than VZV DNA. We report a case of post chicken pox ischemic stroke. Case: 17 year old male had history of fever with maculopaplar, vesicular rash all over body since 3 days followed by acute onset right sided faciobrachial weakness since 1 day.His routines were normal.Serum ANA=negative. MRI brain suggested acute infarcts in left frontal and parietal lobe. MR Angiography suggested partial stenosis in left M1 segment. CSF suggested pleocytosis. CSF VZV PCR was negative. His serum VZV IgM antibody was positive. We treated patient with acyclovir for 14 days with prednisolone 40mg for 5 days. Patient improved in 1 month. Conclusion: VZV vasculopathies are caused by productive viral infection in cerebral arteries. CSF anti-VZV IgG more sensitive than VZV PCR .Patient responds well to acyclovir and steroids. Accurate diagnosis is important for the effective treatment of these disorders.

PP10/76: Unusual cause of rest tremor

Veera S, Lakshmi SR, Ramesh, Archana, Tirumal

Department of Neurology, Osmania Medical College, Hyderabad, Telangana, India

Background and Introduction: Acquired hepato cerebral degeneration (AHD) refers to a chronic neurological syndrome in patients with advanced hepatobiliary diseases or porto systemic shunts. The prevalence of AHD in chronic liver disease is estimated to be 1–2%. Typical features include Symmetric parkinsonism, early gait disturbance or falls, prominent postural or action tremor, mild cognitive impairment,and variable response to levodopa therapy. Case Report: 51 yr female presented with rest tremor involving right hand, lower lip and left foot since 2 months, without rigidity and bradykinesia with past history of jaundice and splenomegaly, for which splenectomy was done at the age of 26 yrs, alcoholic since 10 yrs. USG Abdomen showed chronic liver disease. Arterial ammonia levels, serum manganese levels are elevated, MRI showed T1 hyperintensities in globus pallidi, hypothalamus, tegmentum of mid brain, PET CT showing hypermetabolism in the bilateral basal ganglia, left precentral gyrus, left insular cortex. Conclusion: Our case presented with isolated rest tremor. Rest tremor is rarely observed in parkinsonism related to AHD and is mostly accompanied by postural or action tremor in one or both hands. The high signal lesions on T1WI are thought to be the result of manganese accumulation.

PP11/81: Unusual aetiology of acute febrile encephalopathy

Halappanavar A, Ahmad F, Nanda S

Department of Neurology, Armed Forces Medical College, Pune, Maharashtra, India

Our patient was a 42 years old lady, who developed acute febrile illness with intermittent headache and vomiting. There were no other accompanying symptoms in form of running nose, cough, dyspnoea, chest pain, pain abdomen or dysuria. She was operated for Pituitary Macroadenoma by transnasal-transsphenoidal route in 2009 and underwent gamma-knife radiosurgery in 2012 and was on treatment for Ulcerative Colitis and Diabetes Mellitus for the past 04 years. On the third day of fever, she was noticed to be in altered sensorium by family members and became stuporous over next 7 to 8 hours after which she was brought to a tertiary care military hospital at Pune. She was noted to febrile with temperature of 103F, Pulse 112/min, respiratory rate of 22/min with normal blood pressure. SpO2 was 95% on room air. There was no pallor, icterus, cyanosis, rashes, petechiae or any lymphadenopathy. CNS examination revealed an unconscious patient and her GCS was E1M4V1; right pupil 3mm and left pupil 2mm, both not reacting; moving limbs to noxious stimuli with bilaterally extensor plantar response. Other systems were unremarkable. Peripheral smear and Paracheck for malaria, NS1Ag and IgG/IgM for dengue were negative. Her initial Chest X Ray was normal. She was started on broad-spectrum injectable antibiotics and intravenous Acyclovir and Artesunate and apart from supportive care. Her contrast enhanced MRI Brain showed T2/FLAIR hyperintensities involving bilateral posterior basifrontal region, mammillary bodies, anterior column of fornices, hypothalamus, anterior thalamus on left side and splenium of corpus callosum with diffusion restriction of above areas and no contrast enhancement. Cerebrospinal fluid examination revealed clear in appearance with WBC of 08/cumm, RBC of 120/cumm, Protein of 58 mg/dl, sugar of 93 mg/dl (blood sugar of 112 mg/dl) and globulin was not raised. Her arterial blood gas was normal. Her urine routine and microscopy, and serum procalcitonin were normal. In view of no clinical improvement and persistent fever, limited MRI brain was performed which showed increase in the size of the lesions which were seen previously along with involvement of bilateral basal ganglia and right side of midbrain. Her urine culture showed no growth and blood culture grew Pseudomonas aeruginosa, sensitive to Colistin. Throat swab for H1N1 by Taqman RT-PCR was positive (received on day 5 of hospitalization). Her antibiotics were modified and she was started on Tab Oseltamivir. In spite of all possible efforts, her condition went downhill and finally succumbed on day 5 of hospitalization. CSF for Herpes Simplex Virus by DNA-PCR and Japanese Encephalitis virus IgM by ELISA were negative (received post-mortem). Conclusion: Acute influenza-associated encephalopathy/encephalitis mostly occurs in the paediatric population. However, it can also present in adults without usual flu-like symptoms as in our case. This highlights the importance of considering the diagnosis with present H1N1 endemicity in an acute febrile illness with unusual presentations involving any organ system, to avoid misdiagnosis/ delayed diagnosis that may lead to a fatal outcome.

PP12/85: Meningioma presenting as nummular headache

Wani I, Shah P, Dar I, Maqbool M, Mehraj I

Department of Neurology, Government Medical College, Srinagar, Jammu and Kashmir, India

Nummular Headache is a rare type of primary headache that presents as pain of variable duration, often chronic, localised to a small circumscribed area of the scalp which is 1 – 6 cm in diameter with a round or elliptical shape. Hypaesthesia, dysaesthesia, paraesthesia, allodynia and/or tenderness may be present in the region of the affected scalp. Nummular headache has been reported in underlying brain tumors like pituitary tumor, infratentorial meningioma and other conditions like arachnoid cyst, pagets disease. Here we a report a middle aged female who presented with nummular headache and on evaluation was found to have a frontoparietal meningioma that completely responded to surgical exisicion of the mengioma thereby signifying the importance of diagnosing and evaluating patients presenting with this type of headache.

PP13/88: Optical coherence tomography and diffusion tensor imaging of optic tract in optic neuritis

Shivaraman AMM, Ranganathan LN, Ravi R, Peter BS, Rajan M

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

Background: Optic neuritis is an inflammatory condition producing 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 non-invasive imaging which is an anatomical measure of anterior visual pathway, retina and macula. OCT measures retinal nerve fibre layer (RNFL) thickness. Visual evoked potential (VEP) measuresthe response (measures latency and amplitude) to light stimulus. Diffusion tensor imaging of optic tract measures the fiber density and direction of optic radiation aschanges in optic radiation occurs in patients with optic neuritis. Aim: To assess the sensitivity of VEP,OCT in diagnosing optic neuritis. To assess the pattern of retinal nerve fibre layer changes in optic neuritis. To assess the fractional anisotropy and mean diffusivity values of optic tract in optic neuritis. Methods: 24 patients with optic neuritis due to multiple sclerosis and other etiologies were included. Five multiple sclerosis patients without optic neuritis were also included. Patients underwent detailed clinical examination, neuroimaging, CSF analysis, VEP and OCT. Magnetic resonance imaging of brain acquired with 3-tesla MRI and diffusion tensor imaging was synthesized using tractography software and reconstructed to obtain cranial 3D images based on DEC maps. Results: 18/24 (75%) patients had abnormal VEP (prolonged P100 latency/reduced amplitude), 6/24 (25%) had normal VEP. All the patients (100%) had retinal RNFL thinning. Average RNFL thickness of temporal quadrant was 50.1+4.45um, nasal quadrant was 77.15+7.47um, superior quadrant was 110.07+73.04um and inferior quadrant was 111.76+14.32um. 3 of the 5 multiple sclerosis patients without optic neuritis had normal VEP but RNFL thinning in OCT. Tractography demonstrated decreased number of fibers in optic radiation in optic neuritis patients compared with age matched controls. Fractional anisotropy values were significantly lower and mean diffusivity and radial diffusivity were significantly higher in both optic radiations in optic neuritis patients. Conclusion: Optical coherence tomography is more sensitive than visual evoked potential in diagnosing optic neuritis. RNFL thinning irrespective of etiology was more in temporal quadrant. OCT detects early changes during the subclinical phase even before VEP shows abnormality. The optic radiations undergo microstructural changes following optic neuritis reflecting neurodegeneration. Diffusion tensor imaging is sensitive to these changes and fractional anisotropy values serve as useful and valuable surrogate biomarker for assessing changes in demyelination and optic nerve involvement. Limitations: A large cohort is needed to extrapolate the data.

PP14/103: Ross syndrome – Not so Rosy!!!

Manju Surendran, Kabeer KA, Shaji CV

Department of Neurology, Government Tirumala Devaswom Medical College, Alappuzha, Kerala, India

A 60 year old female presented to our department with 5 years history of anhydrosis in the right side of face. She had no surgeries or trauma prior to the onset of anhydrosis. Her Examination revealed left sided dilated pupil with sluggish light reflex and normal accommodation (Adie's pupil). She also had absent knee and ankle reflex with no other significant neurologic abnormality. Laboratory investigations including blood count, Erythrocyte Sedimentation Rate, renal function test, liver function test and vasculitis work up were normal. Although neuroimaging including MRI BRAIN and CSF studies were non contributary, her skin biopsy showed absent sweat and sebaceous glands on the right side of face suggestive of segmental anhydrosis. With the triad of areflexia, tonic pupil and segmental anhydrosis, Ross syndrome was confirmed in our patient.

PP15/114: Charot–Marie–tooth disease type 2O: First case report from India

Furqan Mohd Khan, Dave D, Nirhale S, Rohatgi S, Rao P, Naphade P

Department of Neurology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India

 Charcot-Marie-Tooth disease More Details is a hereditary motor and sensory neuropathy, characterized by length-dependent axonal degeneration with distal sensory loss and weakness, deep-tendon-reflex abnormalities, and skeletal deformities. Charcot-Marie-Tooth hereditary neuropathy type 2 (CMT2) is an axonal (non-demyelinating) peripheral neuropathy characterized by distal muscle weakness and atrophy, mild sensory loss, and normal or near-normal nerve conduction velocities. We report a case of 43 year old female with genetically confirmed CMT-2OA disease having clinical involvement of facial nerve and dysphonia. These atypical features widen the clinical spectrum of CMT-2O, leading to interesting observations about DYNC1H1 gene related disorders and varied clinical expression of similar genetic mutations.

PP16/121: A study on clinical profile and automatisms in complex partial seizures and its radiological correlation in 120 patients at Thanjavur Medical College

Nandini Priyanka Balasubramani, Thangaraj M

Department of Neurology, Government Thanjavur Medical College and Hospital, Thanjavur, Tamil Nadu, India

Introduction: Complex partial seizures are now preferably called as “focal impaired awareness seizure”. This is the seizure, with longest duration of signs and symptoms, which helps in localising and lateralising the lesion. Hence, this study is taken to analyse various features of the seizure among people in central tamilnadu at a tertiary health center. Aims and Objectives: To study the clinical profile of complex partial seizures, nature of automatisms in complex partial seizures, about lateralising and localising values of automatisms and other semiology features and its radiological correlation. Materials and Methods: 120 Patients with complex partial seizures were evaluated for their aura, automatisms, semiology of seizures and its relation with EEG, CT-Brain, MRI Brain is correlated. It is a Cross Sectional Observational Study. Inclusion Criteria: All patients with semiology and clinical features suggestive of complex partial seizures. Exclusion Criteria: Patients with all other tyoes of seizures. Results and Conclusion: MC age group-10-20 years, Males were more common, MC aura–vertiginous>somatosensory>cephalic>epigastric, MC automatism- gestural/manual>vacant Staring>oroalimentary>verbal>bipedal>behavioral arrest, Mc type of epilepsy- Tle>Fle>multiple semiology, Tle with symptomatic lesions in MRI :50.72%, correlation was significant with p value of <0.005, Fle with symptomatic lesions in MRI: 17%, correlation was significant with p value of <0.005, MC MRI findings were Normal>MTLS>Gliosis>ICSOL>HIE, Tle with abnormal eeg: 33%, Fle with abnormal eeg: 47%. Thus, significant lateralizing and localizing nature of automatisms concluded with radiological findings.

PP17/140: Idiopathic CD4+ T cell lymphocytopenia presenting with atypical meningitis

Sanjiv Sharma, Panda S, Jain S, Tiwari S

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

Background: Idiopathic CD4+ T cell lymphocytopenia (ICL) is an unusual disease with CD4+ T lymphocyte count < 300 cells/mm3 or <20% total T-cells without evidence of HIV or other immunosuppressing condition that may lead to fungal, parasitic, and other serious opportunistic infections. Case report: A 23-year-old married lady presented with new onset headache, vomiting, decreased appetite and weight loss for one month with preceding right focal motor seizures for 2 years. Examination revealed grade 2 papilledema, hypotonia and decreased power at right ankle (4-/5) and brisk right ankle jerk without other deficits. Initial suspicion was cerebral venous thrombosis. MRI brain showed cortical based hetero-intense lesion with coarse calcification focus in left paracentral lobule with diffuse smooth leptomeningeal enhancement along fronto parietal convexity and cerebellar folia. On decongestant therapy, right foot drop improved but severe headache, neck pain and meningeal signs appeared over 2 weeks. CSF done was clear, pressure 28 cm H2O, protein 119 mg/dL, sugar 9 mg/dL with 20 lymphocytes. Gram's, Acid fast stain, Bacterial culture, India ink, Cryptococcal antigen and culture were negative. HBsAg, Anti HCV, VDRL and HIV tests and other hematological and biochemical tests were negative. CSF mycobacterial culture revealed growth of nontubercular mycobacterium (NTM) on 11th day and line probe assay was positive for  Mycobacterium abscessus Scientific Name Search Absolute Lymphocyte Count (639/uL) and CD4 count was reduced (137/uL; 21.38%) with normal CD8 count while P24 assay, HIV RNA PCR, HBV DNA PCR, HCV RNA PCR were negative. A diagnosis of Idiopathic CD4 T-Lymphocytopenia was made and patient started Clarithromycin based therapy with linezolid and amikacin resulting in improvement in vomiting, headache, appetite and CSF picture. Conclusion: Mycobacterium abscessus Scientific Name Search  complex is a rare non-tuberculous mycobacteria species that causes meningitis or cerebral abscess more in HIV-negative individuals. Its indolent presentation with seizures, mass lesion and meningitis in Idiopathic CD4 T Lymphocytopenia Syndrome is a yet unreported association.

PP18/141: Whole exome sequencing in young ischemic stroke: A study protocol

Ankita Maheshwari, Sagar R, Mohd F, Srivastava AK, Prasad K

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

Background: Stroke is the second leading cause of death globally and a major public health problem of India. Ethnic variation in disease susceptibility loci is well known. 10-30% of Ischemic stroke occur in age group between 18-45ys. Exome Sequencing help in identifying the disease causing rare genetic variants. Role of rare or low frequency variants with high penetrance and large effect size was observed in mulifactorial polygenic disease stroke. This is the first study from India up to best of our knowledge to specifically addresses the genetic causality of young ischemic stroke through Whole exome sequencing. Objective: To identify rare functional exonic variations and genetic diagnostic markers for young onset Ischemic stroke. Materials and Methods: Study protocol was approved from the Ethics committee, AIIMS, New Delhi. Young (18-40ys) stroke cases classified by TOAST were recruited after obtaining consent from neurological OPD/Ward. The clinical evaluation was done under a senior neurologist. 5ml blood was collected in EDTA coated vials for DNA isolation. DNA isolation from whole blood was done by the phenol-chloroform method. Whole Exome Sequencing was done on illumina Hi Seq X TEN. The study was approved from Indian Council of Medical Research (ICMR), Government of India. Results: A total of 122 cases were recruited in the study. Exome sequencing is being done on 100 cases from 78 families. The variants will be segregated into rare variations according to the minor allele frequency (<1%). Gene burden analysis will be carried out by SKAT-O for an association between variants and phenotypes. Family-based segregation analysis will be done on identified variations. Conclusion: Whole exome sequencing has been done in 100 cases of young ischemic stroke. This is probably the first study from India to unravel the genetic cause of ischemic stroke in young.

PP19/149: Carbamazepine induced noncirrhotic hyperammonemia

Rishabh Maloo, Reddy V, Manjunath M, Roy AK

Department of Neurology, Manipal Hospital, Bengaluru, Karnataka, India

Introduction: Carbamazepine is a commonly prescribed antiepileptic and mood stabilizer for the treatment of acute mania, bipolar I disorder and in neuropathic pain. Its common adverse effects include drowsiness, dizziness, gait instability, nausea, vomiting, hypotension, hyponatremia, nystagmus and confusion. Severe adverse reactions include aplastic anaemia, agranulocytosis and dermatological reactions which include toxic epidermal necrosis and  Stevens-Johnson syndrome More Details. Hyperammonemia is an uncommon adverse event that can lead to impaired consciousness, lethargy, coma and death. Early symptoms include appetite loss, nausea, insomnia, agitation, personality changes and clinical signs of hyperammonemia which occur at concentrations >60 μmol/L. Decreased ammonia elimination can be secondary to hepatic failure, inherited defects of enzymes in the urea cycle, defects in fatty acid oxidation (carnitine deficiency), hematologic diseases and adverse effects of medications. Medications commonly associated with hyperammonemia include valproic acid, fluorouracil, salicylates and asparaginase. We hereby report a patient, who presented with hyperammonemia on therapeutic dosage of carbamazepine; which improved after discontinuation of carbamazepine. Case Report: 62 yr old male who was known to have Hashimoto's encephalopathy and seizure disorder, on medication, presented with history of increased drowsiness and disorientation since 4 days. Family history and drug abuse history were not contributory. Patient was currently on carbamazepine 600 mg/day with levetiracetam 1 gm daily orally for seizures. Liver function tests, renal parameters, complete blood count, serum electrolytes, lactate, thyroid function test, HIV, CSF routine, EEG and MRI Brain with contrast were within normal limits. Serum ammonia concentration was found to be 119 μmol/L (normal reference level 11-35) while carbamazepine levels were within therapeutic range 8.2mg/L (4-12 mg/L). Carbamazepine was discontinued and the patients sensorium slowly improved with gradual resolution of asterixis in the next two days, whereas the serum ammonia level decreased to 47 μmol/L. USG abdomen and fibroscan were normal. Subsequently patient was started on a combination of levetiracetam and lamotrigine with adequate seizure control. Discussion: Temporal correlation of development of asterixis, confusion and elevated ammonia levels with the use of carbamazepine, which resolved completely with the discontinuation of the medication indicate the causal relation between the drug and side-effects. There were no other probable causes of hyperammonemia like hepatic failure or hematological disorders. The possible mechanism of hyperammonemia due to carbamazepine is unknown and some researchers have postulated it to have a similar mechanism as that of valproic acid by disruption of urea cycle. An isolated increase in ammonia without an increase in liver enzymes indicates either hepatocellular dysfunction without cellular damage or isolated mitochondrial dysfunction. The treatment for hyperammonemia is to discontinue the precipitant. L-carnitine has been used in cases with urea cycle disorders and valproic-acid-related toxicities to reduce serum ammonia levels. Other options directed at lowering serum ammonia levels are oral lactulose and consumption of a low-protein diet. Conclusion: Carbamazepine induced hyperammonemia is not an uncommon side-effect and can present with the presence of asterixis, disorientation and confusion.

PP20/152: Herpes zoster ophthalmicus with retrobulbar neuritis

Chandu P, Hazeena P, Sugumar T, Sundar S, Shankar V

Department of Neurology, Sri Ramachandra Medical College and Hospital, Chennai, Tamil Nadu, India

Background: Herpes zoster Ophthalmicus with Retrobulbar neuritis in young adults has been seldom reported in the literature. Objective: To report the case of a young adult who presented as Herpes Zoster ophthalmicus (HZO) with retrobulbar neuritis showing clinical features, examination findings, investigation and the subsequent clinical response. Case Report: A 24 year old male presented with herpes zoster ophthalmicus, who on the fifth day of the disease, developed painless blurring of vision over left eye progressed to non perception of light over 24 hours. Examination findings showed vesiculo-papular rashes over left eyelid and forehead. MRI findings were suggestive of post contrast enhancement of optic nerve with normal fundus picture and VEP showing delayed P100 responses, suggesting Retrobulbar neuritis. Patient presented to us two weeks after the onset, were he had received only antiviral therapy and had no clinical response to intravenous methyl prednisolone 1gm/day for five days. Conclusion: Young adult presenting with Herpes Zoster ophthalmicus with retrobulbar neuritis is very rare. HZO with retrobulbar neuritis most commonly seen in advancing age, immunocompromised state and patient mostly on chemotherapeutic drugs. Prompt treatment with systemic antivirals and steroids improve the visual outcome.

PP21/154: Glial fibrillary acidic protein levels in differentiating ischemic stroke from healthy controls, stroke mimics and intracerebral hemorrhage: A systematic review and meta-analysis

Shubham Misra, Talwar P, Kumar P, Kumar A, Rai A, Pandit AK, Prasad K, Vibha D

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

Introduction: Acute stroke is an emergency and time sensitive treatment decisions depend on the diagnosis of correct stroke type. Clinically, it is difficult to diagnose and differentiate ischemic stroke (IS) from intracerebral hemorrhage (ICH) or other mimicking conditions in acute stages. Glial Fibrillary Acidic protein (GFAP), a cytoskeleton filament is rapidly released in intracerebral hemorrhage (ICH) and has the potential to differentiate IS and ICH in acute stages. Aims: To conduct a systematic review and meta-analysis of all the published studies till date to determine the role of GFAP biomarker in diagnosing and differentiating IS from controls, mimics and ICH within 24 hours of symptom onset. Materials and Methods: A literature search was conducted till 29 June 2019 in PubMed, Google Scholar and Cochrane databases using following keywords: ('Glial Fibrillary Acidic Protein') AND ('GFAP') AND ('Ischemic stroke') AND ('Healthy Controls') AND ('Stroke mimics') AND ('Intracerebral Hemorrhage') AND ('Blood biomarkers'). Standardized mean difference and 95% Confidence Intervals were calculated. If the I2value was < 50% then fixed-effects model was used, else a random effects model was used. The quality assessment of all the studies included in our meta-analysis was done using the modified version of Quality Assessment of Diagnostic Accuracy Studies (QUADAS) questionnaire. All the statistical analysis was carried out using the STATA version 13.0 software. Results: GFAP levels in eight diagnostic test studies having 727 IS and 312 ICH cases were found to be significantly lower in IS as compared to ICH (SMD -1.04; 95% CI -1.30 to -0.77) within 24 hours of symptom onset. After conducting a stratified analysis including six studies, the GFAP levels significantly differentiated IS from ICH within 6 hours of symptom onset as well (SMD -1.02; 95% CI -1.36 to -0.69). In three studies having 363 IS and 268 controls, the GFAP levels were significantly higher in IS and it successfully differentiated IS from healthy controls (SMD 0.27; 95% CI 0 to 0.53) within 24 hours of symptom onset. However, GFAP did not differentiate IS from stroke mimics in two diagnostic studies (SMD 0.09; 95% CI -0.28 to 0.46). The levels of GFAP biomarker were also significantly higher in total stroke as compared to healthy controls (SMD 0.66; 95% CI 0.50 to 0.81) in 3 studies having 481 stroke and 268 control subjects. Conclusions: GFAP is an extremely promising biomarker with huge potential to be used in clinical settings for stroke diagnosis. Our results of our meta-analysis conclude that GFAP levels were significantly lower in IS as compared to ICH and differentiated IS from ICH within 6 hours and 24 hours of symptom onset. GFAP levels also differentiated IS from healthy controls but failed to differentiate IS from stroke mimics. GFAP could be used as a diagnostic biomarker to exclude ICH in clinical settings. However, further well-planned diagnostic test studies with high sensitivity and specificity of GFAP are required to be conducted before using GFAP as a diagnostic biomarker in routine clinical practice.

PP22/157: Sturge– Weber syndrome More Details presenting as status epilepticus

Murugan P, Pravina L, Vasagam LN

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

Sturge–Weber syndrome (SWS), also called as encephalotrigeminal angiomatosis, is a rare non-hereditary condition characterized by a facial cutaneous vascular nevus (nevus flammeus or port-wine stain) in association with leptomeningeal angiomatosis. When the upper and lower eyelids are involved in port-wine birthmark, the risk of developing glaucoma becomes as high as 50%. Type I: individual has a facial PWS, leptomeningeal angioma, and may have glaucoma. Type II: individual has a facial PWS, no leptomeningeal angioma, and may have glaucoma. Type III: individual has leptomeningeal angiomatosis, no facial PWS, and, rarely, glaucoma. SWS is an developmental anomaly of embryonic origin because of malformation in mesodermal and ectodermal development. It results due to failure of regression of a vascular plexus around cephalic portion of neural tube which is destined to become facial skin. This vascular plexus normally forms at 6th week of intrauterine life and regresses by 9th week. Failure of its regression results in residual vascular tissue which forms angiomas of leptomeninges, face and ipsilateral eye. Due to this there is abnormal flow of blood which in turns causes vasomotor phenomenon, resulting in ischemia, gliosis, atrophy, and calcification of the cortical tissues. The aim of this article is to report a case of Sturge–Weber syndrome and add to the existing literature. 11 month old child presented with Status epilepticus. Seizure semiology was left focal seizure with dyscognition. Patient was ventilated for 2 days and given Midazolam infusion after which seizure abated. Patient was stabilized with Inj Phenytoin, Inj Levetiracetam and Inj Phenobarbitone. Patient's parents gave a history suggestive of discolouration of right side of face, body as well as feet, since birth. Physical examination showed portwine stain involving right side of face, body, legs and feet, there was right Exophthalmos. MRI Brain showed Right parietooccipital volume loss, T2 FLAIR hyperintense signals, enhancing choroid plexus. MRI CONTRAST showed diffuse leptomeningeal enhancement in Right temporal, posterior parietooccipital region suggestive of pial angiomatosis. Right globe sclera enhancement. Management: Anticonvulsant medications in combination with aspirin can be given for management of neurologic consequences. Regular treatment of the patient with Sturge-Weber with carbamazepine 600 mg results in long term seizure free. A successful early treatment results in control of seizures and prevention of complications. Additionally, we strongly emphasize that professional counseling and support in addition to drug treatment can provide proper assistance to patients and their family to overcome their problems and improve the outcome of the treatment. SWS presents with varied clinical features. Available latest treatment modalities & thorough knowledge of this syndrome is very important in management of this condition. It is a multidisciplinary team work to deliver proper medical care to the patient. Complete understanding regarding the entire disorder should be imparted to caregivers. It is important that caregivers should perceive consequences of complications so that the patients are treated quickly and effectively. Several controversies exist in the management of seizures in Sturge Weber syndrome. It is necessary to improve our understanding of the cause and molecular neuropathology of Sturge Weber syndrome.

PP23/161: An unusual cause of ulnar neuropathy

Venkatesh Dasari, Radhakrishna Hari, Bimal Prasad Padhy

Department of Neurology, CARE Hospitals, Hyderabad, Telangana, India

Introduction: Recurrent subluxation of the ulnar nerve at the elbow is uncommon (16.2%), occurring about equally in young and old, male and female. The probable cause of such a dislocation is congenital laxity of supporting ligaments. Being more vulnerable to injury than normally positioned nerves, however, complicating neuritis can occur. Case Details: A 40 year male, right handed, presented with numbness of left ring and little finger, and medial aspect of left palm since 1 yr. Thinning of left palm since 8 months. Clawing, decreased sensations since 6 months. No h/o trauma. Evaluated for above symptoms outside and diagnosed as ulnar neuropathy due to Hansens disease and started on medications. Consulted our hospital for second opinion. On examination, weakness of hypothenar, interossei, 3rd and 4th lumbricals and FCU, FDP. Reflexes were normal. Sensory - decreased pinprick in ulnar nerve distribution. Investigations: EMNG showed left ulnar neuropathy with active denervation. HRUSG of left elbow was normal. MR Neurography of left upper limb showed thickened ulnar nerve with hyperintense signals at elbow and midforearm. Biopsy of left ulnar s/o chronic axonopathy. Dynamic Ultrasound of elbow showed- on flexion, left side ulnar nerve showing snapping/transient dislocation over the medial epicondyle which appears to relocate on extension. Conclusion: Accurate diagnosis of the ulnar nerve dislocation requires a careful history taking, a thorough physical examination followed by appropriate anatomical and functional imaging investigation such as dynamic sonography of the elbow.

PP24/162: A case of isoniazid induced cerebellitis in a patient with chronic renal failure

Vaishal Shah, Sardana V

Department of Neurology, Government Medical College, Kota, Rajasthan, India

Introduction: CKD and tuberculosis have been proven to be interrelated. An increased incidence of tuberculosis, as well as side effects of antituberculosis therapy, has been reported in patients with CKD, especially in those on haemodialysis. INH induced peripheral neuropathy, seizure and neuropsychiatric menifestations are well known but only cerebellitis without other manifestation is rare and its occurrence was reported in CKD patients undergoing dialysis. We report a case of isoniazid induced cerebellitis in CKD patient not on dialysis who had isolated bilateral symmetrical dentate nucleus T2/FLAIR hyperintensity with diffusion restriction on magnetic resonance imaging with recovery after treatment modification. Case Report: A 65 year old man presented to us with complaints of sudden onset of slurring of speech and mild clumsiness in right upper limb since 3 days. He was known hypertensive since 20 years and CKD stage 4 since 1 year. 10 days prior to presentation, he was started on DOTS category 1 without pyridoxine for left axillary lymph node tuberculosis which was biopsy proven with INH dose being 375mg/day which was higher than recommended dose of 300mg/day. Central nervous system examination showed dysarthria with subtle impairment in right finger nose test. MRI brain showed symmetrical diffusion restriction in bilateral dentate nuclei with T2 and FLAIR hyperintensity. Cerebellitis due to INH neurotoxicity secondary to reduced renal clearance and pyridoxine deficiency were then considered as a possible aetiology after ruling out other reported causes of similar MRI picture. Patient improved completely within 2 weeks after stopping INH and adding pyridoxine. Conclusion: INH induced neurotoxicity should be considered in CKD patients who present with cerebellar symptoms after starting antituberculosis therapy. Prophylactic pyridoxine should always be prescribed especially in patient who are prone for INH induced neurotoxicity. INH induced neurotoxicity should be included in differential diagnosis of bilateral symmetrical dentate nucleus T2/FLAIR hyperintensities with diffusion restriction.

PP25/163: Discrepancy between ventricular and lumbar CSF in chronic meningitis

Bhavin Patel, Sardana VS

Department of Neurology, Government Medical College, Kota, Rajasthan, India

Introduction: Meningitis is inflammation of coverings of brain and spinal cord. Raised intracranial pressure can be part of clinical presentation in acute or chronic meningitis. Diagnosis of meningitis can be confirm by CSF fluid analysis which usually obtain from lumbar puncture. In case of acute hydrocephalus urgent placement of external ventricular drainage is required to relieve raised intracranial pressure. In such cases CSF can be obtained during the operation and sent for investigation. Sometime this CSF results come out normal which leads to delay in diagnosis and treatment. Case Report: 35 year old male patient went to other hospital with history of moderate intensity generalized headache and vomiting associated with low grade fever for 15 days. Patient investigated for same and on imaging communicating hydrocephalus was found. Ventriculo-peritoneal shunt was performed and CSF was sent for analysis which came out to be normal. Patient was treated with conservative treatment and symptomatically improved. After 15 days of VP shunt patient again started having persistent headache and vomiting and came to our hospital. On examination he was not having any focal deficit. Considering possibility of shunt block repeat imaging was done which showed no hydrocephalus. In view of possibility of chronic meningitis repeat CSF was sent which showed 200 counts with lymphocytic predominance and high protein. CSF ADA was 16.4. Empirical Antitubercular was started and patient improved clinically. Conclusion: Our case highlights that difference between ventricular and lumbar CSF can be present in tubercular meningitis hydrocephalus. Early diagnosis and appropriate treatment can make significant impact on morbidity in meningitis patient. It is advisable to do lumbar CSF in suspected case of meningitis even if ventricular CSF is normal to avoid risk of delay in diagnosis and treatment.

PP26/164: Wilson disease presenting as resistant rickets without fanconi syndrome

Bhavin Patel, Sardana VS

Department of Neurology, Government Medical College, Kota, Rajasthan, India

Introduction: Wilson's disease (hepatolenticular degeneration) is an autosomal recessive disorder of biliary copper excretion due to mutation in the ATP7B gene on chromosome 13. Wilson disease is a disorder of copper metabolism which usually present with neurological or hepatic manifestation. Wilson disease presenting as musculoskeletal disorder is rare. Arthropathic manifestation of Wilson disease occurs in late stage and that too in 25-50% of patients. Arthropathic manifestation mostly occurs secondary to hypoparathyroidism, Wilson disease or copper deposition. Case Report: A 12 year old male child born by nonconsanguineous marriage with normal birth history and developmental milestone presented with complaint of insidious onset progressive in intensity bilateral knee joint pain with difficulty in walking since 3 years followed by slurring of speech since 6 months. On examination patient had genu valgus with richatic rosary and mild hepatomegaly. Neurological examination revealed dysarthria without other neurological deficit. Slit lamp examination shows KF ring. Investigations suggestive of High 24 hr urinary copper level (267.40). MRI brain showed hyperintensity in T2 and FLAIR images with hypointense T1 image without any diffusion restriction in bilateral thalamus, basal ganglia, dorsal midbrain and pons. In view of KF ring with high 24 hour urinary copper and MRI brain changes diagnosis of Wilson disease was kept and patient treated with D-penicillamine. Conclusion: High index of suspicion for WD should be kept in Case of resistant rickets. Hightened awareness in clinicians of possible rachitic presentation of WD results in early diagnosis and timely treatment. Delay in treatment increases morbidity in WD.

PP27/166: A case of hemiparesis and head myoclonus associated with hypoglycaemia

Pallav Jain, Vijay Sardana, Vaishal Shah

Department of Neurology, Government Medical College, Kota, Rajasthan, India

Introduction: Hypoglycemia has been associated with various neurological symptoms including confusion, coma, seizure. Presentation as a hemiparesis is also seen in hypoglycaemia, in about 4.2% of cases. Few reports of dyskinetic movement including chorea, hemiballismus have been also reported. Myoclonic seizure have not been commonly reported. We are reporting 64 year female who was known diabetic on metformin 1000mg. glimepiride 4mg was added 1 week prior, who presented with hemiparesis preceded by TIA like episode and also developed jerky movement involving head with documented hypoglycaemia during hospital stay. She had complete recovery with correction of hypoglycaemia. MRI brain showed diffusion restriction in bilateral internal capsule. Conclusion: Existence of cases in which hypoglycaemia cannot be distinguished from stroke on imaging studies suggests the importance of measurement of blood glucose level when stroke symptoms are first recognised. Our case highlights the occurance of head myoclonus along with hemiparesis during hypoglycemic episodes.

PP28/169: Outcome following intravenous thrombolysis in anterior versus posterior circulation ischemic stroke: An Indian tertiary centre experience

Amit Shankar Singh, Sidhu AS, Bhutani V, Mann HS

Department of Neurology, Fortis Hospital, Mohali, Chandigarh, India

Introduction: Intravenous thrombolysis by recombinant tissue plasminogen activator is an approved treatment for anterior circulation stroke (ACS) and posterior circulation stroke (PCS) if patient arrives within window-period. Though, 20-25% of total stroke are due to posterior circulation involvement, still not much studies are available comparing outcome in ACS and PCS. Aim: To assess the variability in clinical outcomes in ACS versus PCS, through known outcome scales. Methods: We conducted a single center, observational cohort study in 54 consecutive ischemic stroke patients arriving in our emergency within window-period. NIHSS score was assessed at presentation and 24 hours post-thrombolysis. Clinical outcome was assessed at 1 and 3 months by modified Rankin score (MRS), Barthel index (BI), Glasgow outcome score (GOS). Results: Out of 59 patients, 33 were ACS and 12 were PCS, rest were lacunar stroke. Average door to needle time in ACS and PCS was 28.93 and 28.66 minutes respectively. There was statistically significant difference between ACS and PCS with respect to MRS (at one month) (P<0.05). There was no statistically significant difference between ACS and PCS with respect to NIHSS (at presentation and after 24 hours), BI (at 1 month and 3 months), GOS (at 1 month and 3 months), and MRS (at 3 months) (P>0.05). Mortality in ACS was 15.15% and none in PCS. Conclusions: Our study suggests that outcome in ACS and PCS is comparable. Therefore, early identification of PCS is important so that early thrombolysis can prevent morbidity as in ACS.

PP29/175: A case of pres-sci (spinal cord involvement) in renal artery stenosis patient

Ram Abhishek Bondada, Jeyaraj KM, Velayutham SS, Sowmini PR, Mugundhan K, Arunan S

Department of Neurology, Stanley Medical College, Chennai, Tamil Nadu, India

Introduction: Posterior reversible encephalopathy syndrome (PRES) is a common entity which occurs frequently in hypertensive patients and around 20- 30% in normotensive patients. It is one of the treatable encephalopathy which needs appropriate management. PRES-SCI is a new terminology which is a rare occurrence along with spinal cord involvement (SCI). Although PRES is commonly encountered clinicoradiological syndrome, very few cases have been reported with spinal cord involvement on MRI. Case Report: We report a case of 19 yr old male patient who presented with sudden onset headache, vomiting, multiple episodes of seizures with altered sensorium with accelerated hypertension. MRI brain and spinal cord shows T2/Flair hyperintensities noted asymmetrically in cortical and subcortical areas of parieto –occipital, temporal regions and along with involvement of medulla and cervical spinal cord without diffusion restriction, hemorrhages and contrast enchancement. Ultrasound and renal artery Doppler study showed right renal artery stenosis with right contracted kidney. Patient was treated with antihypertensives and antiepileptics. Patient has improved clinically and there is complete resolution of radiological findings after 1 week. Clinically he had no features suggestive of spinal cord involvement inspite of radiological findings. Conclusion: We present a unique case of posterior reversible encephalopathy syndrome which involved both brain and spinal cord (PRES-SCI) secondary to undiagnosed renal artery stenosis. This case highlights unique distribution and etiology of PRES in a young boy.

PP30/176: Effectiveness and safety of tenecteplase versus alteplase in acute ischemic stroke: A prospective study from a rural tertiary care centre

Ahamed Subir, Ghafoor F, Krishnadas NC

Department of Neurology, MES Medical College, Malappuram, Kerala, India

Introduction and Background: Stroke is the second leading cause of death worldwide with its incidence and mortality increasing in past two decades in the resource poor countries. Tenecteplase is a recently approved recombinant tissue plasminogen activator (r-TPA) for acute ischemic stroke. This study is first of its kind to be reported from rural tertiary care centre with 24 hour stroke care facility with I.V thrombolysis and advanced endovascular procedures from a developing country like India which observes the clinico-epidemiological as well as in-hospital parameters and compares the immediate and the 3 month functional outcomes with these two drugs. Methodology: 47 acute ischemic stroke patients receiving r-TPA [alteplase (N=28) and tenecteplase (N=19)] presenting within 6 hours of symptom onset were enrolled in our prospective observational study. Institutional ethics committee approved the study and written informed consent was obtained from all study participants. Evidence of haemorrhagic stroke, age<18 years, transient ischemic attack and participants with seizures at stroke onset were excluded. NIHSS (baseline, 1 hour, 24 hours and 7 days) and mRS (1 and 3 months) were recorded in structured proforma and were analysed using SPSS. Wilcoxon signed rank test & Friedman test were used as test of significance, p <0.05 was considered statistically significant. Results: Significantly lower NIHSS at 1 hour compared to baseline was observed among participants receiving alteplase (p<0.001). Significantly lower NIHSS at 1 hour (p=0.008), 24 hours (p=0.01) and at 7 days (p=0.009) compared to baseline was observed among participants receiving tenecteplase. Significantly lower mRS at 3 months compared to 1 month was observed among participants receiving alteplase (p<0.001) and tenecteplase (p=0.005). No significant difference in change in NIHSS (p=0.3) and mRS (p=0.5) between alteplase and tenecteplase was detected. 10.7% and 5.3% receiving alteplase and tenecteplase respectively had symptomatic intracranial haemorrhage (sICH). Conclusion: Tenecteplase was non inferior to alteplase in reducing NIHSS and mRS scores but with the lower risk of symptomatic Intracranial hemorrhage. This goes hand in hand with NORTEST results but disagrees to EXTEND 1A TNK & ATTEST results. In this era of endovascular stroke care, faster bridging time along with its other advantages makes it the preferred thrombolytic. Limitations: The limitation of this study was that it was an observational nonrandomized study with a small sample size. Further Scopes: This observational study is being continued in our centre looking into other parameters. Studies regarding dosage of the thrombolytics are to be done on Asian/Indian population especially in rural population. Study comparing endovascular procedures with thrombolytic therapy are being planned.

PP31/180:  Chiari malformation More Details in craniosynostosis – A rare case report

Mariappan Veerappan, Nithyanandam A, Govindarajan S, Balasubramanian S, Lakshminarasimhan R

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

Craniosynostosis results from premature fusion of skull sutures which affects 1 in every 2500 live births. Premature fusion restricts the growth of the skull perpendicular to the affected suture and compensatory growth of skull occurs parallel to affected suture to accommodate the growing brain. Syndromes most commonly associated with craniosynostosis includes Apert, Crouzon, Pleiffer, Carpenter and Saethre-Chotzen which is due to de novo mutations involving FGFR and TWIST genes. Crouzon syndrome results in premature fusion of coronal, sagittal sutures and lambdoid sutures which starts in first year of life. Premature fusion of lambdoid suture results in small posterior fossa which leads to formation of CM-1. Here we report a case of a 22 years male presented with characteristics features of crouzon syndrome withbilateral proptosis, hypertelorism, strabismus, flat forehead, long slender nose, high arched palate, mandibular prognathism, broad lower lip, low set ears with small external auditory meatus and midfacial hypoplasia with symptoms of syringomyelia and associated Chiari malformation type 1.

PP32/181: Familial amyloid polyneuropathy with transthyretin P. 173 V mutation: A report of the first case from India

Manoj Bharath, Taly AB, Periyasamy G, Kumar P, Archana NB

Department of Neurology, Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India

Mrs.B, a 40 years old lady presented to us with history of weakness and parasthesias of both lower limbs for 5 years, weakness and parasthesias of both upper limbs for 2 years, exertional dyspnoea, orthostatic giddiness,grittiness of both eyes, dry mouth and constipation for 1 year. In addition, she had occasional burping, vomiting and early satiety for one year. She also had a significant weight loss of 12 kilograms in the past one year. On examination, she was emaciated with a BMI of 17.08 kg/metre square. She had normally sized, but sluggishly reacting pupils bilaterally, hypotonic areflexic flaccid quadriparesis and length dependent pan sensory loss of all four limbs. Bedside autonomic function tests showed significant dysautonomia. Her Barthel's index was 45/100 and Compass-31 score for autonomic dysfunction was 39/100. Work-up showed anaemia, thrombocytopenia, elevated ESR, Positive Schrimer's test bilaterally and absent response on sympathetic skin stimulation in all four limbs. Her cardiac evaluation showed changes suggestive of hypertrophic cardiomyopathy with speckled appearance of the interventricular septum. ANA profile, ANCA, serum protein electrophoresis by immunofixation were all negative. A previous nerve biopsy done in a centre elsewhere showed severe axonal neuropathy with no evidence of regeneration, however, no etiology could be identified. In view of the typical clinical profile, we went ahead with a lip biopsy as the patient had significant symptoms of dry mouth suggesting possible involvement of the minor salivary glands. Lip biopsy was positive for amyloid deposits with a typical apple green birefringence. We then proceeded with targeted genetic analysis for Transthyretin gene,which showed missense variation A-G on exon 3 at codon 277 in TTR gene, replacing Proline for Valine. Concluding, this is the first time the mutation is reported in India and only the third time globally. Familial amyloid polyneuropathy should be considered in the differential diagnosis in pateints with a classical clinical presentation, even in the absence of a positive family history as has happened with our patient. Nerve biopsy may be erroneously negative in amyloidosis and sequential biopsy helps. Traditionally, abdominal fat pad, rectum, liver, kidney and bone marrow are the chosen sites for biopsy in amyloidosis, however, it is noteworthy that the symptoms present in the pateint in question can guide towards the choice of the biopsy site, as has happened with our patient where dry mouth guided us towards a less invasive and higher yielding lip biopsy. Lastly, though usually a painful peripheral neuropathy, familial amyloid neuropathy can be a painless neuropathy and the previous two case reports harbouring this particular mutation also had painless neuropathy.

PP33/182: Analytical study to find out incidence of hematoma expansion in ICH patients and its clinical and radiological variable deciding hematoma expansion

Rajesh Chaudhary, Singh KK, Bhutani N

Department of Neurology, Santokba Durlabhji Memorial Hospital, Jaipur, Rajasthan, India

Introduction: Incidence of hematoma expansion varies in different studies. Radiological predictors have been studied so far but data regarding clinical and radiological predictors of hematoma expansion along with their impact on outcome of patients with ICH still lacking. Aims: To determine incidence of hematoma expansion and review the clinical and radiological variable predicting hematoma expansion along with prognosis after hematoma expansion. Methods: Total 94 ICH patients admitted to SDM hospital over 18 months who presented within 24 hour of symptom onset. Initial ICH volume measured using ABC/2 method, and repeat CT was done within 72 hour or when clinically indicated. Clinical and radiological parameters related to expansion noted and looked for the prognosis of the patient after expansion. Results: Total 14 (15%) patients showed expansion [10 male (71%), 4 female (29%)]. In clinical profile headache (P=0.04) and hemiparesis (P<0.001), radiologically marginal irregularity (P<0.001), hypodensities (P<0.001), ovoid (P=0.0046) and circular shape (P=0.04) showed significant results. While initial hematoma volume (P=0.16), SBP (P= 0.28), fluid level (P=0.15), cerebral edema (P=0.18), and IV extension (P=0.98) were more in expander group but didn't reached to statistically significant level. This expansion affected the prognosis with prolonged hospital stay (P=0.05), detoriation of ICH score (P=0.02) and mRS (P=0.54). Conclusions: Considering certain clinical and radiological predictors of hematoma expansion can improve the prognosis of patients with spontaneous ICH.

PP34/184: A descriptive study of the etiology, clinical profile and management of first seizure an adult patients presenting to a tertiary care hospital

Rohit Kumawat. Bhutani N, Agarwal P

Department of Neurology, Santokba Durlabhji Memorial Hospital, Jaipur, Rajasthan, India

Background: Aetiological and clinical profile of first seizure can vary with age, accordingly appropriate treatment needed to prevent recurrence. Objective: To study aetiology, demographic, clinical profile and outcome of first seizure in adult patients. Methods: Prospective observational study conducted at tertiary care hospital of from April 2017-Oct 2018. Total 114 adult patients of either gender, aged 18 years or above, presenting within 15 days of new onset seizures or developed first seizure in hospital during study period, were included. Results: New onset seizure most common in 18-29 & 30-49 years. GTCS most common seizure type (70.2%) followed by focal to bilateral tonic clonic (14.9%), focal onset with awareness (10.5%). 61.4%, 25.4% and 13.2% cases presented with single seizure, seizure clusters and status epilepticus respectively. Nearly 3/4th (74.6%) had provoked seizures. Cerebrovascular aetiology was most common (36.8%) followed by neuroinfections (22.8%) and systemic/metabolic aetiology (18.4%). Infraction most common cerebrovascular cause followed by CVT. Neurocysticercosis most common infection followed by tuberculosis. In systemic/metabolic causes, most common aetiology PRES followed by hyponatremia. 11.4% had idiopathic seizure. 71.9% had abnormal neuroimaging. 62.3%, 21%, 12.3% and 4.4% cases treated with single AED, two AED, >2 AED and without AED respectively. Most commonly used AED levetiracetam (68.4%) followed by fosphenytoin (35.1%). At 3 months follow up seizure recurred in 20.2% cases. Conclusion: New onset seizures, common during middle age & aetiology varying with age. Not all patients required treatment, recurrence was seen in treated patient, hence AED need to be individualized.

PP35/187: A study of cognitive function in young patients with type 2 DM in a tertiary care hospital in North West India

Prashant Gandhi, Singh KK, Varma AR, Shah A

Department of Neurology, Santokba Durlabhji Memorial Hospital, Jaipur, Rajasthan, India

Background: T2DM is associated with CVD, leading to sub cortical dysfunction. These changes manifest later, if we can predate it; prevent progression. Aim: Explore cognitive functions in 35-55 years T2DM and analyse domains involved. The self report of cognitive dysfunctions, any relation of decline with factors like demography, education, duration and control of T2DM. Methods: Observational study in 100 T2DM of 35-55 years. Subjects and Carers were Asked (SQID): 'Have you been more forgetful in the past 12 months to the extent that it has signi?cantly affected your life? And classified as SQID positive and negative. HMSE test, Digit span, Clock drawing, neuropsychological (Weigl's block, Picture sequencing, Luria motor sequence, Word fluency test and combined score WPLW). Orientation and delayed loss of information scores calculated. Correlation between HMSE and WPLW score with variables. Results: 24 were SQID positive. HMSE score of SQID positive was 26.5 ± 1.93vs.27.22 ± 2.61 of negative (P=0.038). HMSE score uneducated 22.4 ± 2.80vs.graduate (28.34 ± 1.61) (P<0.001). Forward span in SQID positive was 4.08 ± 0.58vs.negative 4.47 ± 0.58 (P=0.014). CDT score in SQID positive was 2.83 ± 1.37vs.Negative 3.5 ± 1.34 (P=0.048). Picture sequence score in SQID positive 4.58 ± 1.02vs.Negative 5.11 ± 0.99 (P=0.026). WPLW score in SQID positive patients was 9.5 ± 1.69vs.Negative 11.22 ± 2.27 (P<0.001). HMSE and WPLW scores were positively correlated. HbA1c and HMSE scores negative correlated. Conclusion: This suggests underlying frontal subcortical dysfunction in T2DM.

PP36/193: Riboflavin responsive lipid myopathy due to a homozygous missense variation in the ETFDH gene masquerading as myasthenia: A case report

Vinanthi Prabhuraj, Agadi JB, Kumar S, Pramod MN,

Pradeep YV, Yasha TC

Department of Neurology, Apollo Hospitals, Department of Neuropathology, NIMHANS, Bengaluru, Karnataka, India

Disorders of lipid metabolism affect several tissues including skeletal and cardiac muscle tissue. Lipid storage myopathies are a heterogeneous group of genetic disorders that present with abnormal lipid storage in multiple body organs, typically muscle. Lipid storage myopathy is pathologically characterised by prominent lipid accumulation in muscle fibres due to lipid dys metabolism. Making an accurate diagnosis by specific laboratory tests including genetic analysis is important for the management. Patients can clinically present with cardiomyopathy, skeletal muscle weakness, myalgia and extreme fatigue. An early diagnosis is crucial as some Lipid storage myopathies can be managed by a simple nutritional supplementation. Here we present a case of easy fatigue with mild ptosis and symmetrical proximal muscle weakness which was earlier being treated as case of Myasthenia gravis. Biopsy of the muscle proved it to be a case of Lipid storage myopathy. Genetic analysis showed a homozygous missense variation in the Exon 8 of the ETFDH gene. Suitable treatment abolished all the signs and symptoms of the disease.

PP37/203: A study of depression and quality of life in headache patients

Sreejith Paul, Narasimhan L, Jawahar M, Jayakumar M

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

Objective: To investigate the frequency of depression among headache patients and to assess their quality of life. Introduction: Headache is one of the most common ailments affecting mankind. There is inadequate data, especially native data, on psychiatric comorbidities like depression in chronic headache patients and it's impact on quality of life. Methods: Consecutive patients attending the head ache clinic were recruited. Headaches were classified according to ICHD3 criteria. Hamilton depression scale was used to assess depression and CDC HRQOL4 to determine quality of life. Patients with other chronic illnesses including primary psychiatric illnesses were excluded from study. Results: Totally 128 patients were recruited. 85.9% were women. The mean age was 47 years. Among these patients 58.5% patients had migraine, 36.7% patients had tension type headache. 4.6% had other types of headache (2.3% cluster headache and 2.3% IIH attributed head ache. Excluded from further analysis due to low numbers). Depression was present in 57% of the total patients. 32% had mild, 17% moderate and 7.8% severe depression. 56% of migraine patients were found to have depression (6% severe, 16% moderate, 29% mild depression) while 65% of TTH patients were detected with depression (10% severe, 15% moderate and 40% mild depression.) On CDC HRQOL4 self rated health only 30.4% of the total patients, rated their health in excellent, very good or good category where as 69.5% stated their health in fair/poor category (poor-25.7%, fair-43.7%). 65.3% of migraine patients and 78% of TTH patients rated their health in fair/poor category. Migraine patients reported 8.2 physical unhealthy days, 3.41 mental unhealthy days, 9.37 total unhealthy days and 7.9 days of limited activity due to ill health, the preceding month. TTH patients reported 10.4 physical, 4.54 mental and 12.2 total unhealthy days. They reported 5.3 days of limited activity. Patients with at least moderate depression reported 14 total unhealthy days and 13.5 days of limited activity compared to 11.74 total unhealthy days and 9.5 days of limited activity among patients without depression. Discussion and Conclusions: Depression is highly prevalent in chronic headache patients. Depression and the primary disease itself has a significant impact on quality of life of the afflicted patients. In this study patients with depression were found to have lower quality of life compared to their counterparts without depression. This factor has to be considered in the management. Limitations: The number of patients recruited in headache types other than migraine and TTH were limited. CDC HRQOL4 could not differentiate between effect of chronic headache and depression individually on quality of life. Further Scope: Effect on quality of live in patients who also receive treatment for depression in addition to headache need to be compared with who get treated only for headache. Further study is required to understand the interrelationship between headache and depression as cause and effect. As the study continues, less common headaches like cluster headache, IIH attributed head ache etc. can be analysed similarly.

PP38/205: A case of cranial autonomic dysfunctions predominant migraine: Migraine sans ache

Amit Shankar Singh, Singhvi JP, Singh HS, Sidhu AS

Department of Neurology, Fortis Hospital, Mohali, Chandigarh, India

Introduction: Pain is the essential part of migraine headaches along with other features. What if other features of migraine are present but pain is absent? One such variation in migraine symptomatology is reported in this case, where pain was not there but cranial autonomic symptoms/signs (CAS) were predominant along with other features of migraine. Aim: To present a case with significant variation in migraine symptomology. Methods: A 28 year women presented with episodes of CAS in form of redness and tearing from eyes, bilateral facial and forehead flushing, and bilateral aural fullness without any pain in head or neck since last 8 months. Duration of these episodes was 12-24 hours. These episodes were associated with triggers like travelling, hunger or decreased sleep. Photophobia and phonophobia was not present but nausea was present during few episodes. Patient had no pain, but she felt restlessness and discomfort during these episodes. Some activity restriction was also present during these episodes. Family history for headaches was negative. Neurological examination was normal. MRI brain with MRA was normal. Results: Patient was started on anti-migraine treatment for these CAS episodes and was advised to follow-up with diary pointing out CAS in terms of onset, severity, laterality and frequency. Her CAS with other features gradually improved and completely subsided over 3-4 months. Conclusion: The possible mechanism for such variation (CAS predominant episodes without pain) in this case may be de-sensitisation of afferent loop (comprised by nociceptors in extracranial and intracranial pain sensitive structures supplied by trigeminal nerve) and direct activation of trigemino-cervical complex and brainstem structures causing dissociation of pain from rest of the features of migraine.

PP39/206: Outcome following intravenous thrombolysis in anterior versus posterior circulation ischemic stroke: An Indian tertiary centre experience

Amit Shankar Singh, Sidhu AS, Bhutani V, Mann HS

Department of Neurology, Fortis Hospital, Mohali, Chandigarh, India

Introduction: Intravenous thrombolysis by recombinant tissue plasminogen activator is an approved treatment for anterior circulation stroke (ACS) and posterior circulation stroke (PCS) if patient arrives within window-period. Though, 20-25% of total stroke are due to posterior circulation involvement, still not much studies are available comparing outcome in ACS and PCS. Aim: To assess the variability in clinical outcomes in ACS versus PCS, through known outcome scales. Methods: We conducted a single center, observational cohort study in 54 consecutive ischemic stroke patients arriving in our emergency within window-period. NIHSS score was assessed at presentation and 24 hours post-thrombolysis. Clinical outcome was assessed at 1 and 3 months by modified Rankin score (MRS), Barthel index (BI), Glasgow outcome score (GOS). Results: Out of 59 patients, 33 were ACS and 12 were PCS, rest were lacunar stroke. Average door to needle time in ACS and PCS was 28.93 and 28.66 minutes respectively. There was statistically significant difference between ACS and PCS with respect to MRS (at one month) (P<0.05). There was no statistically significant difference between ACS and PCS with respect to NIHSS (at presentation and after 24 hours), BI (at 1 month and 3 months), GOS (at 1 month and 3 months), and MRS (at 3 months) (P>0.05). Mortality in ACS was 15.15% and none in PCS. Conclusions: Our study suggests that outcome in ACS and PCS is comparable. Therefore, early identification of PCS is important so that early thrombolysis can prevent morbidity as in ACS.

PP40/210: Post herpes simplex N-methyl D-aspartate receptor encephalitis presenting as kluver bucy syndrome

Jain S, Panda S, Sharma S, Tiwari S

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

Introduction: Herpes simplex virus (HSV) encephalitis (HSE) is a frequent cause of severe, potentially fatal encephalitis. Relapsing neurologic symptoms develop in 12–27%, either as a true viral relapse or rarely immune-mediated. Kluver-Bucy syndrome (KBS) is a neurobehavioral syndrome seen in association with a variety of neurological disorders. We present an adult case of post HSE N-methyl D-aspartate receptor (NMDA-R) antibody associated KBS. Case Report: A 29-year patient presented with progressive mania, irritability, hyperorality, social disinhibition, urinary and faecal incontinence for 10 days. KBS was diagnosed based on triad of hyperorality, hypersexuality and hyperphagia. He had been treated for HSE two months ago for fever, seizures and drowsiness. HSV-1 PCR was positive and he was treated with acyclovir and steroid. Patient recovered slowly over 1 month with residual altered executive functions (mRS 2). Currently, his attention span and concentration were markedly reduced, needing continuous help for his daily activities (mRS 4). Patient was abusive and thrust any object into his mouth. No movement disorders were observed. CSF was acellular, protein-60mg/dl, sugar-49 mg/dl and negative for HSV-1 PCR. Suspecting immune-related relapse, CSF was tested and was positive for NMDA–R antibody. MRI brain showed atrophy and gliosis involving bilateral limbic area. EEG showed only excessive beta activity. He was treated with intravenous methylprednisolone and IVIG for 5 days followed by oral steroids and showed progressive improvement in behavioural symptoms and hyperorality though hyperphagia persisted till last followup after 6 months. Conclusion: Patients with HSE should be carefully followed for any symptom relapse, worsening of deficits, or development of psychiatric or movement disorders. Any of these symptoms should raise concern for a viral relapse or an immune-mediated complication. KBS is a rare complication of post HSE NMDA-R encephalitis and eminently treatable illustrating the importance of determination of CSF and serum neuronal cell surface antibodies.

PP41/212: Time to think beyond thiamine in alcoholic

Agrata Sharma, Rai N, Kohat AK

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

Introduction: Deficiency of multiple vitamins in an alcoholic and malnourished patient is like a situation in which to expect the unexpected. Wernicke encephalopathy due to B1 deficiency characterized by triad of global confusion, ataxia and ophthalmoplegia. Pellagra from niacin deficiency is rare but under diagnosed condition due to B 3 deficiency. Acute pellagric encephalopathy (APE) is manifested by nervousness, insomnia, and hallucinations; and can confound confusion, oppositional hypertonus, myoclonus and ataxia. The differential diagnosis between pellagra and non specific erythaema is important because of the reversibility of pellagra and prevent fatal condition, if left untreated. Materials and Methods: Mr A 30 year old man, heavy alcoholic, presented with history of imbalance while walking from last 1 year which gradually worsened in last 6 months. Also in the last one year he had problems in maintaining attention with delayed response to any questions. Also had 2 to 3 episodes of GTCS along with intermittent hallucinations with episodes of self talking in last 6 months. Despite the treatment with multivitamins he developed multiple episodes of diarrhea that lasted for 1 month along with scaly itchy rashes. Rashes were extremely itchy and painful with heightened response to touch. Rash was described as dull dark erythema with overlying loose scale mainly in dorsal arms and legs; sparing the clothing bearing areas of chest and upper arms and legs. Within few days he developed diplopia with bilateral gaze restriction with auditory and visual hallucinations and myoclonus. On examination he was conscious oriented with incoherent speech. And had delusions and hallucinations. He had horizontal as well as vertical eye movement impairment. Motor examination revealed 5/5 power with preserved reflexes. Cerebellar features were present in the form of finger nose in coordination and disdiadokinesia and impaired gait. His hemoglobin was 7.9 gm/dl. Metabolic encephalopathies were excluded by normal laboratory tests. CSF examination was normal. MRI brain revealed diffuse cerebral with cerebellar atrophy. Results: Nicotinamide 300 mg BD was given along with higher intravenous dose of thiamine. Thiamine was increased to 500 mg intravenously 3 times daily for 3 days followed by 250 mg iv for next 5 days followed by 100 mg orally TDS during hospital stay. In view of response to nicatinamide plus thiamine with pellagra skin lesion diagnosis of pellagrous encephalopathy with wernickes disease was considered. Conclusion: Pellagra is nutritional disorder that occurs as a result of a severe cellular deficiency of niacin. The diagnosis of pellagra is clinical and confirmed by rapid resolution of symptoms after starting niacin replacement. Our patient's symptoms resolved only after high doses of intravenous thiamine; so wernickes encephalopathy was obvious despite the patient showing myoclonic jerking which are more common in pellagric encephalopathy. APE can present with other alcoholic encephalopathies. Possibility of coexistence of multiple vitamin deficiency with pellagrous as well as wernickes encephalopathy should be considered. Niacin should be administered prior to thiamine with adequate intravenous dosages to prevent development of APE.

PP42/213: Varied presentation of hereditary spherocytosis in the family: Unmasked by a stroke in young

Sharma A, Rai N, Kohat AK

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

Introduction: Hereditary spherocytosis (HS) is the genetically transmitted disease as a result of heterogeneous alterations in genes that encode for proteins such as spectrin and ankyrin involved in vertical associations that tie the membrane skeleton to the lipid bilayer. It is rare for HS to be associated with cerebrovascular event secondary to moya moya. Moya moya is the chronic progressive non atherosclerotic non inflammatory and non amyloid cerebrovascular disorder, defined by progressive stenosis of intracranial vessels. Materials and Methods: 23 year old male non smoker presented to the hospital with weakness involving left upper and lower limbs which was insidious in onset, gradually progressive associated with slurring of speech. Patient had no family history of consanguinity or cerebrovascular accidents. His examination revealed no skin anomalies evocative of phacomatosis and no dysmorphic features. He was fully conscious and oriented. Power was decreased in left upper and lower limb (0/5) with exaggerated deep tendon reflexes and mute planter. Complete blood test revealed anemia Hb 7.0gm/dl, MCV 65.2 fl, MCHC 28 gm/dl, MCH 18.3 pg. Peripheral smear showed marked anisopoikilocytosis with predominantly microcytic hypochromic RBCs along with numerous elliptocytes and few polychromatophills and pencils cells. Platelet count was raised 9 lakhs. Biochemistry indicated hemolysis; total billirubin of 4.47 mg/dl, and uncojugated billirubin being 3.89 mg/dl with raised LDH. Corrected reticulocyte count was 4%. Iron profile was within normal limits. ESR and CRP was within normal limits. Autoimmune work up was negative. Direct and indirect coombs test; and sickling test was negative with normal G 6 PD levels. Osmotic fragility was increased. 2D Echocardiography and ECG was absolutely normal. Coagulation studies and chest radiograph was unremarkable. MRI brain revealed acute infarct in right fronto parietal region. CT angiography revealed significant stenosis of supraclinoid segment of right ICA with reformatting of ACA and MCA via collaterals. MR angiographic findings are suggestive of moya moya syndrome. It was diagnosed as moya moya associated with hereditary spherocytosis presenting as a stroke in young patient. Testing for family members was done which revealed anemia with elliptocytosis in mother who was asymptomatic with mild pallor on examination. Results: Patient was started on dual antiplatelet drugs with pentoxiphylline and transfused 2 units of whole blood. By the end of 1 month power improved to 3/5. Conclusion: MMD is rarely associated with HS. Severe hemolysis may trigger endothelial injury and contribute to oxidative damage through nitric oxide scavenging. In addition, less deformability of the red blood cells and increased blood flow associated with anemia contribute to endothelial proliferation, which progresses slowly and produces stenosis followed by occlusion. Prevention of anemia is crucial because it is an important risk factor for ischemic events. Both the abnormal rheology of spherocytes and chronic anemia led to the formation and progression of cerebral vasculopathy. It is important to consider the possibility of MMD in a patient with HS presenting with stroke.

PP43/214: A rare case of moyamoya syndrome and hemoglobin E/beta-thalassemia

Nikhil Repaka, Guha G, Misra AK, Bhattacharjee A, Nanda S, Agasti N, Ghosh S, Roy M

Department of Neurology, NIL Ratan Sircar Medical College, Kolkata, West Bengal, India

Moyamoya disease causes occlusion or stenosis of the bilateral internal carotid arteries, which cause elaborate arterial collaterals at the base of the brain and is differentiated from Moyamoya syndrome, which occurs when similar magnetic resonance imaging (MRI) findings of stenosis or occlusion are found secondary to genetic or acquired conditions. Moyamoya syndrome is a rare diagnosis that has been linked to a small number of haemoglobinopathies. Children wth Moyamoya syndrome tend to present with transient ischemic attacks, mental deficiency, and/or neurological deficits. HbE/b-thalassemia causing Moyamoya syndrome is very rare in the literature till date. Here we describe a case of a 7 year old male with HbE/b-thalassemia who was found to have a moyamoya syndrome as a part of an evaluation of recurrent weakness of left upper and lower limb with blurring of vision. The link between Moyamoya syndrome and HbE/b-thalassemia may be multifactorial, but Moyamoya syndrome is an important consequence of to consider in children with HbE/b-thalassemia.

PP44/215: A prospective observational study of diagnostic accuracy of GeneXpert MTB/RIF in tubercular meningitis

Prashant Shringi, Sardana V, Maheshwari D, Bhushan B, Kiran RB

Department of Neurology, Government Medical College, Kota, Rajasthan, India

Introduction: Tubercular meningitis accounts for 5% of all extrapulmonary tuberculosis (TB). Infection of the Central nervous system is the most severe extrapulmonary manifestation of tuberculosis, with a high mortality rate and residual neurologic sequelae, even with adequate treatment. In low resource settings, limited access to health care, limited diagnostic capacity, and economic constraints frustrate early treatment initiation, So need for a test which is easily available, low cost, has acceptable diagnostic yield, givesearly result. This study shows diagnostic accuracy of GeneXpert MTB/RIF test in suspected tubercular meningitis (TBM). Objectives: Aim of the study is to prospectively determine the diagnostic accuracy of CBNAAT (Xpert MTB/RIF) in patients with suspected TBM. Design and Materials and Methods: Prospective observational study done on 100 patients of clinically suspected TBM using clinical consensus diagnostic criteria. Results: 100 patients of clinically suspected tubercular meningitis studied and divided into probable and possible TBM which were 62 and 32 respectively. CBNAAT was found positive only in 12 patients, 8 patients found to be positive in probable group and 4 patients in possible group. In this study Sensitivity of Gene Xpert was 12.5 % and specificity was 88.23%. Conclusion: This study shows low sensitivity and high specificity for CBNAAT. This study results shows that Xpert MTB/RIF may be a good rule-in test for the diagnosis of TBM. Our study shows low sensitivity as compared with other studies due to low sample size so further studies needed with larger sample size along with adequate volume of CSF.

PP45/217: Stroke as initial manifestation of visceral malignancy – Trousseau syndrome

Karnati Mrudula, Emani Srikanth Reddy, Kamlesh M Chawda

Department of Neurology, Kamineni Hospitals, Hyderabad, Telangana, India

Introduction and Background: Stroke & Cancer have complex relation. 15% of cancer patients have pathologically proven ischemic stroke & only half are seen clinically. 3-4% of stroke patients have cancer as major risk. However Conventional vascular risk profiles for stroke in cancer patients are same. In few, cancer is the risk factor for stroke & in few, stroke is the manifestation of occult cancer. Unfortunately detection, prevention, and treatment of stroke in cancer patients have been largely understudied. Hence this case series to when to suspect and how to screen for occult malignancy in patients with acute stroke. Materials and Methods: This is Case series of 4 patients who have no or well controlled vascular risk factors for stroke, presented to Kamineni hospitals, Hyderabad (jan 2017 to dec 2017) diagnosed as recurrent acute ischemic stroke with bilateral embolic infarcts with no identifiable embolic source. On evaluation D dimer & tumor markers are elevated and finally diagnosed with visceral malignancy. Patient 1: A 79Yr old male non alcoholic, nonsmoker, known hypertensive, well controlled on regular treatment. Presented with left hemiparesis. MRI Brain showed multiple bilateral embolic infarcts. 3weeks later he presented with altered sensorium, icterus, abdominal distension. Repeat MRI Brain showed bilateral infarcts with large infarct in left frontotemporoparietoccipital region. Repeat Alkaline phosphatase is elevated. D dimer is elevated. CT Abdomen showed Hepatomegaly with multiple hypo dense Lesions in both lobes of liver - ? Metastasis. Serum CA 19.9 is - >2044 U/ml. Finally diagnosed as liver metastasis. Patient 2: A 56Yr old female, known hypothyroid on regular treatment. Past h/o hospital admission 8mon back in view of recurrent hypoglycemia, right hemiparesis – bilateral acute infarcts and bilateral lung consolidation. Presented to our hospital with acute left hemiparesis. MRI Brain showed acute infarcts bilaterally and multiple T2/FLAIR hyperintensities. 2D ECHO showed dilated RA & RV with moderate PAH with no septal defects. CT Chest showed Multiple focal consolidations bilaterally. During stay she developed DVT of left common femoral & popliteal vein in spite on LMWH prophylaxis. D DIMER is elevated >800. CT Guided Lung biopsy done was suggestive of Bronchoalveolar Carcinoma. Patient 3: A 62Yr old male non alcoholic, nonsmoker, known hypertensive, well controlled on regular treatment. Presented with acute aphasia and right hemiplegia. MRI Brain showed bilateral acute infarcts. Alkaline phosphatase is elevated. CT Abdomen contrast suggestive of ? Cholangiocarcinoma. Serum CA 19.9 is elevated. FNAC of liver done was s/o Metastatic squamous cell carcinoma. Patient 4: A 80Yr old male non alcoholic, nonsmoker, known hypertensive and diabetes mellitus, well controlled on regular treatment. Presented with acute dysarthria and gait ataxia. MRI Brain showed multiple bilateral embolic infarcts. Ultrasound abdomen showed grade III prostatomegaly. Serum PSA is elevated. CT abdomen contrast showed : sclerotic metastasis in left acetabulum. Prostatectomy was done, histopathology – s/o adenocarcinoma. Conclusions: Suspect Occult malignancy in c/o Multiple B/l acute embolic infarcts with no identified embolic stroke or Cryptogenic Stroke. Screen with D dimer and confirm with tumour markers, CT Chest, abdomen & pelvis.

PP46/219: A case report of panencephalopathic type of creutzfeldt jakob disease

Justin Cornelius, Kannan V, Ramraj M, Kasirajan MP, Jeyaraj M

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

Introduction: Creutzfeldt Jakob disease, the most common form of human prion disease is clinically characterised by rapidly progressing dementia, cerebellar and extrapyramidal signs and myoclonus. The sporadic type of CJD shows a predominant grey matter involvement. Apanencephalopathic type of CJD is characterised by the extensive involvement of the cerebral white matter as well as the grey matter. This present CJD case is unusual because of widespread white matter degeneration in addition to moderate, but typical CJD gray matter changes. Case Report: A 38 years old female presented with the chief complaints of slowness in doing daily activities and walking, with stiffness of all 4 limbs and the trunk and low volume speech for past 1½ years. She was apparently normal 1½ years ago and was working as a supervisor and was active. She was found to be slow in her work, slow to walk as observed by her coworkers, her handwriting had changed and become small, she took longer time to respond, however was able to carry out her daily activities slowly. Two months later, shesubsequently became slow in cooking, took longer time to cut vegetables, wash utensils and clean clothes. She became slower in walking especially in turning and getting up from chair. Her speech became slow, took longer time to respond, spoke less, low volume but was able to understand and read. 2 months later she developed gradually worsening memory disturbances. She became functionally dependant and apathetic with history of unconcerned micturition. She developed mixed type of seizures (right focal, GTCS and myoclonic jerks) for past 9 months. Now she isbedridden for past 1 month. No history of similar illness in any of her family members. No history of diabetes, hypertension or seizures in the past. On examination she was awake, not aware, mute, in a persistent vegetative state, with extrapyramidal features like mask facies and cogwheel/axial rigidity. Disuse atrophy and contractures of hands and feet were noted. No evidence of alopecia or KF ring. Vitals were stable. Cranial nerves examinations were normal. Investigations including HIV, ANA, VDRL were negative, thyroid tests and serum ceruloplasmin were normal. Cerebrospinal fluid cell count, protein, autoimmune panel, antimeasles antibody were normal. Her EEG revealed periodic synchronous sharp waves with generalised slowing. At the disease onset, MRI revealed T2 FLAIR hyperintensities in the periventricular region and mild cortical atrophy. 14 months later, MRI showed evidence of T2 FLAIR hyperintensities in bilateral periventricular, right thalamus and right side of pons. The lesion is asymmetrical and bilateral in subcortical white matter and perirolandic region. The lesions show predominant white matter involvement of bilateral corona radiata, centrum semiovale and splenium of corpus callosum. She was clinically diagnosed as a case of panencephalopthic type of CJD and treated with supportive measures, antiparkinsonian drugs, antiepileptics and low dose steroids.

PP47/222: To review an uncommon cause of stroke in young presenting with a dramatic cascade of events

Heena Kathuria, Doomra K, Pattanayak SN, Goel A, Ray S, Modi M, Lal V

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Introduction: The etiology of stroke in young is difficult to find out as there are varied causes of stroke in young. Aim: To review an uncommon cause of stroke in young presenting with a dramatic cascade of events. A 46 year old female presented with sudden onset loss of consciousness with left hemiparesis and multiple episodes of vomiting .On presentation on next day, she was euglycemic, pupils were asymmetrical, non-reactive to light with bilateral ptosis, bilateral brisk deep tendon reflexes and extensor plantar response. Possibility of cerebrovascular accident was kept. Her neurological and radiological findings indicated that she had ischemic stroke and was eligible to undergo mechanical thrombectomy as she was out of window period for intravenous thrombolysis with NIHSS of 28/42, MRI brain showing acute infarct in right cerebellum, left paramedian mid brain and bilateral thalamus. CT angiography showed top of basilar and bilateral posterior cerebral artery occlusion. She underwent mechanical thrombectomy at 23 hours from the onset of stroke with post thrombectomy TICI score 3/3. NIHSS improved to 10 from 28 and gradually her sensorium improved. Later on, she was evaluated further for the cause of stroke in young and had normal blood parameters causing cerebrovascular risks. Procoagulant work up including APLA workup was negative. Therefore, possibility of cardio embolic stroke was kept and she underwent 2 D Echocardiography which showed a mass attached to atrial septum with possibility of Myxoma and Cardiac MRI showed homogenous contrast enhancement suggestive of atrial myxoma. 24 hour Holter was normal. CECT chest and abdomen to look for associated tumours was normal except atrial myxoma. Tumour was resected by trans right atrial and trans septal left atrial complete myxomectomy with no intra or post- operative complications and biopsy specimen confirming myxoma. She was discharged in hemodynamically stable condition with mRS of 4. Conclusion: Cardiac myxoma stroke is rare. Often, it affect young females. For an improved diagnostic accuracy, magnetic resonance imaging of the brain and echocardiography are imperative for young stroke patients in identifying the cerebral infarct and determining the stroke of a cardiac origin. Immediate thrombolytic therapy or mechanical theombectomy may completely resolve the cerebral stroke and improve the neurologic function of the patients. An early surgical resection of cardiac myxoma is recommended in patients with not large territory cerebral infarct.

PP48/226: Low CD4 counts across gamut of neurological disorders: An immunological association or an epiphenomenon?

Dhananjay Gupta, Anish Mehta, Pradeep R, Rangasetty Srinivasa, Purshottam Acharya

Department of Neurology, Ramaiah Medical College, Bengaluru, Karnataka, India

Introduction: Idiopathic CD4+ lymphocytopenia (ICL) is a rare syndrome characterised by low CD4+ counts in absence of HIV infection and other immunodeficiency states or treatment. Most patients are asymptomatic and are serendipitously diagnosed during evaluation of other clinical presentations. The aetiology, pathogenesis, and management of ICL remain poorly understood. Case reports of ICL associated neurological disorders are limited to opportunistic infections and immune mediated sensory-motor polyneuropathy. We report three patients, who on evaluation were found to have ICL. Cases: The first patient, 50-year-old gentleman, presented with progressive cognitive decline and personality changes characterized by errors in financial transactions, decreased social interaction and crying spells for 2 months. Subsequently, he developed mild right sided weakness. Neurological evaluation revealed a conscious patient with impaired comprehension and reduced attention span. He had right upper motor neuron facial palsy with right pronator drift and a right extensor plantar. Neuroimaging revealed MRI T1 hypo intense, FLAIR hyperintense, confluent, subcortical white matter lesion in the left fronto-parietal region, without post-contrast enhancement. Baseline blood tests were normal. Viral serologies were negative. Absolute CD4 count was 69/ μL and CD8 count 120/μL. Clinically, a possibility of progressive multi focal leukoencephalopathy (PML) was considered, which was confirmed on brain biopsy. His CSF analysis and John Cunningham virus serology were normal. The second patient, a 40-year-old lady, presented with pins and needle sensation, followed by weakness of all limbs over a period of 15 days. The weakness was initially distal, followed by proximal limb weakness, truncal and eye closure weakness, without respiratory or bulbar weakness. On examination, she had bilateral facial weakness, areflexic quadriparesis (proximal > distal, power 3/5 on MRC scale) with mild impairment of joint position and vibration up to ankle joint. Nerve conduction study showed sensory motor demyelinating and axonal neuropathy (LL > UL). MRI brain and spine were normal. CSF analysis showed albumin-cytological dissociation. Hence, she was managed as a case of Acute Inflammatory Demyelinating Polyradiculoneuropathy with Corticosteroids and Intravenous Immunoglobulins. Subsequently she showed improvement in weakness. She had 2 similar episodes within next 3 years, with improvement after corticosteroid therapy. Repeated testing for common causes of neuropathy and viral serologies were negative. Except, her CD4 levels were 123 cells/ μL. Despite detailed evaluation, no other cause could be found for her low CD4 counts. The third case, a 65-year-old gentleman, was diagnosed to have generalized myasthenia based on fatigable ptosis, proximal limb weakness and high Acetylcholine receptor antibody levels. He was managed with corticosteroids, but did not show improvement. Baseline blood tests were normal. Incidentally, he was diagnosed to have CD4 counts of 80/μL, with negative testing for HIV-1 and 2. This complicated the clinical scenario, as we were hesitant to use immunosuppressants. Hence, he was managed with IVIG and showed improvement. Discussion: These 3 cases describe distinct neurological presentations with ICL. Whether low CD4 counts contribute to pathogenesis or are an associated finding in such patients, is debatable.

PP49/239: Comparative study of clinical outcome following only intravenous thrombolysis versus intravenous thrombolysis plus mechanical thrombectomy in acute ischemic stroke

Singh AS, Sidhu AS, Bhutani V, Mann HS, Singh AS

Department of Neurology, Fortis Hospital, Mohali, Chandigarh, India

Introduction: Both intravenous thrombolysis (IVT) by recombinant tissue plasminogen activator and mechanical thrombectomy (MT) are approved treatments for acute ischemic stroke (AIS). IVT has limitation of time (less than 4.5 hours) but extension of time limit has been experimented recently in MT according to clinical and imaging parameters. Bridging with MT is indicated in selected patients and found helpful in past studies. Aim: To compare clinical outcome following only IVT versus IVT plus MT in AIS. Methods: This is a single center, observational cohort study conducted in 54 consecutive AIS patients arriving in our emergency. Patients were divided in to two categories on basis of treatment: patients underwent only IVT (n = 51) and patients treated with MT post IVT (n = 8). NIHSS score was assessed at presentation and 24 hours post-thrombolysis. Clinical outcome was assessed at 1 and 3 months by modified Rankin score (MRS), Barthel index (BI), Glasgow outcome score (GOS). Results: In terms of outcome, early outcome measure NIHSS at 24 hours was better and statistically significant in IVT group. But long term outcome parameter BI at 1 month and 3 month was better and statistically significant in IVT plus MT group. MRS and GOS were comparable in both groups at 1 and 3 months. Conclusion: In AIS where mechanical thrombectomy is indicated and preformed in selected patients post-thrombolysis, though initial outcome appear to be worse because of more severe disease and large vessel involvement but long term outcome measures are comparable or may be better. Therefore bridging with MT is very important in selected patients arriving early at stroke unit.

PP50/242: Acute hepatitis E virus infection presenting with LGBS

Sarath Aleti, Naresh Tandyala, Sucharita Ray, Navneet Sharma, Vivek Lal

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Introduction: Hepatitis E is one of the most common cause of hepatitis worldwide. Neurological mainfestations are a rare complication of this infection. GBS is one of the least common complications of the same. Case History: A 52 year old gentleman presented with fever, vomitting and jaundice followed by pure motor lower motor neuron areflexic quadriparesis with bulbar involvement which progressed over 5 days. Conduction studies were consistent with diagnosis of LGBS. Anti HEV IgM antibodies were positive in serum. He recieved IVIG at 2g/kg and his weakness improved. Discussion: Transmission of Hepatitis E virus is by faeco-oral route. It rarely presents with neurological manifestation such as GBS[MOU1], Neuralgic amyotrophy, transverse myelitis and meningitis. Only 5.5% of patients of acute and chronic HEV cause neurological manifestations. In India 10% of GBS cases are caused by HEV. A recent literature review showed 53 cases of GBS associated with HEV till now in world literature. Pathogenesis is related to GM1, GM2 and GQ1b antibodies. IVIG has been used successfully in treating these people. Conclusion: GBS is an emerging extrahepatic manifestation of HEV. Needs to be considered strongly in people presenting with elevated transaminases and areflexic flaccid paralysis.

PP51/246: Prognostic significance of red cell distribution width and neutrophil-to-lymphocyte ratio in acute ischemic stroke

Rahi Kiran Bhattiprolu, Sardana V, Maheshwari D, Bhushan B, Shringi P

Department of Neurology, Government Medical College, Kota, Rajasthan, India

Stroke is the second leading cause of death worldwide. Red Cell Distribution Width (RDW) and Neutrophil Lymphocyte Ratio (NLR) have role in predicting severity of Acute Ischemic Stroke (AIS). Our study was designed to find out role of RDW and NLR as prognostic biomarkers for AIS. Objectives: Evaluate the association between RDW and NLR with MRS, GCS and NIHSS scores in patients with AIS. Design, Materials and Methods: This is a Prospective observational study including 100 patients of AIS admitted <48 hours of symptom onset. On admission, NIHSS and GCS scores were calculated along with RDW and NLR values. MRS scoring was done at the time of discharge or death and after 1 month followup. Using Pearson's correlation coefficient, Student t test and ANOVA, association of RDW and NLR with various scores was evaluated. Results: Mean age is 61.16 years including 58 males and 42 females. 53% had MRS≤2, 47% had MRS>2. Hypertension and diabetes mellitus had no influence over various scores, RDW and NLR values. Mean RDW was 15.43 and NLR was 3.5.36% patients had RDW <14,64% had >14, 52% had NLR≤3 and 48% had >3. The association between RDW and NLR with NIHSS, MRS and GCS suggests highly significant correlation in prognostication of AIS. Conclusion: Our study highlights the prognostic value of readily available inexpensive biomarker RDW and NLR in AIS patients and their correlation with various scores in assessing the severity. Higher values on admission are predictors of severity and poor prognosis.

PP52/259: Transforming growth factor – beta gene polymorphism and risk of ischemic stroke in North Indians: A case control study

Amit Kumar, Prasad K, Sagar R, Misra A, Rawar D, Raj R, Vibha D, Srivastava A, Pandit A, Goyal V, Gupta G

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

Background: Transforming growth factor –beta (TGF- β) is a prototype of a large family of cytokines and plays an important role in the inhibition of the inflammatory cascade, immune regulation, apoptosis and maintenance of blood pressure. Genetic variation in the gene encoding TGF- β could be important as genetic risk for the ischemic stroke. Aim and Objective: To investigate the relationship between TGF-β1 G800A gene polymorphism and risk of IS in North Indian population. Methods: In a case-control study, DNA was isolated by chloroform-phenol method and genotyping was performed by MALDI-TOF MassARRAY method for 540 patients and 540 age-sex matched controls. Frequency distribution of genotypes and alleles were compared between cases and controls through conditional logistic regression by using STATA software. Results: Mean age of patients and controls were 53.3±12.6 and 51.8±12.8. Frequency distribution of alleles was consistent with HWE. Conditional logistic regression analysis showed a statistically significant protective effect of TGF-β1 G800A and ischemic stroke under recessive model (OR=0.10; 95% CI 0.03 to 0.27; p<0.001). This association also remains significant in the multivariable analysis (OR 0.07 95% CI 0.02 to 0.25, P<0.001). Conclusion: TGF-β1 G800A gene polymorphism showed a protective role for ischemic stroke under recessive model in North Indians. Prospective adequately powered studies is required to reveal its precise effect.

PP53/261: POEMS syndrome and stroke

Vicky Thakkar, Benny R

Department of Neurology, Fortis Hospital, Mohali, Chandigarh, India

Introduction and Background: POEMS syndrome is a rare multi-systemic disease characterised by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Recurrent ischemic stroke is an extremely rare complication of POEMS syndrome. Association between POEMS syndrome and ischemic stroke has not been entirely understood. Materials and Methods: Two cases with recurrent ischemic stroke and diagnosed POEMS syndrome is presented. Comprehensive review and analysis of the literature were performed. Results: Two patients with recurrent ischemic strokes, one patient had multiple episodes of bilateral MCA territory infant with occlusion of the right MCA, other patient with left MCA territory infarct were identified. Common clinical manifestations of POEMS syndrome were less common in these patients as compared to other patients with POEMS syndrome. Both of these patients were investigated in detail to find out the cause of recurrent stroke. Conclusions: Comprehensive analysis of literature revealed several trends in patients with ischemic strokes and POEMS syndrome. There is a preponderance of cerebral vasculopathy and involvement of multiple cerebral arteries. Ischemic stroke may be a poor outcome predictor in patients with POEMS syndrome. Although atherosclerosis due to diabetes mellitus, hypertension, and dyslipidemia might as well have contributed to the cerebral artery occlusion in our patients, though the ischemic attacks continued even after appropriate antiplatelet therapy. Limitations: Stroke in patients with POEMS syndrome is rare. A larger cohort may help in better characterising and treating this rare complication. Further Scopes: POEMS syndrome should be considered in the differential diagnosis of the patients presenting with the combination of peripheral neuropathy and cerebrovascular events, particularly when a monoclonal protein in the serum is present. Cytokines, such as IL-1, IL-6, TNF alpha and VEGF, may play a role in the development of the disease and occlusion of the cerebral vessels. This would be an intriguing area to investigate further.

PP54/264: A rare case of hereditary motor sensory neuropathy with retinitis pigmentosa in a young male

Heena Kathuria, Tandyala N, Goyal M, Lal V

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Introduction: Hereditary motor sensory neuropathies are diagnosed very commonly these days in young patients with foot deformities and distal involvement of limbs. A very rare variant of the same is seen with night blindness and retinitis pigmentosa. Aim: To review the rare case of hereditary motor sensory neuropathy with night blindness. Case Report: A 23 years old male presented with difficulty in maintaining balance and ankle twisting in left ankle more than right during walking since 13 years of age with difficulty in walking on uneven surface and recurrent falls. He did not have any sensory, urinary and bowel symptoms. He had deformities in bilateral forefeet of same duration. He had progressive painless diminution of vision with more difficulty during night time since 6 years of age. His mother was having hammer toes without any neurological symptoms. On examination, there was mild kyphosis, bilateral pes cavus which was more in left foot as compared to right and bilateral hammer toes. Visual acquity was 3/60 in right and 4/60 in left eye. Posterior segment examination revealed bony spicules in both eyes with tilted disc and paripapillary atrophy bilaterally. There was distal lower limb weakness with absent ankle reflexes with circumferential sensory loss till both ankles. Hematological investigations like complete blood count, liver and renal function tests, thyroid profile, vitamin B12, Serum protein electrophoresis, ANA were negative. Nerve conduction study revealed decreased sensory conduction velocity in bilateral ulnar, median nerves with non recordable SNAPS in bilateral sural nerve. Bilateral median and left peroneal nerve showed increased motor latency and decreased conduction velocity. Right peroneal and right tibial CMAPS are not recordable. Sural nerve biopsy revealed loss of myelination. NFP immunostain revealed intact axons with no evidence of vasculitis, amyloidosis or any granulomatous pathology. However, genetic analysis was not done. Conclusion: Hereditary motor sensory neuropathies are diagnosed commonly these days, but its few types are rarer of the rarest. These should be suspected based upon the associated clinical features in the patients presenting with distal neuropathies with skeletal deformities.

PP55/267: A mystic case of malignant intracranial hypertension

Soumya Medarametla, Kodapala S, Nataraju B

Department of Neurology, Vydehi Institute of Medical Sciences, Bengaluru, Karnataka, India

Introduction: Iron deficiency anemia is one of the rarer causes of IIH. It is a hypercoagulable state and has been associated with cerebral thrombosis. Although rarely reported, anemia alone has been associated with IIH with no signs of thrombosis. Aim: We present a case of life threatening malignant hypertension secondary to no other cause than anemia. Case Details: A 34 year old female, a known case of IIH since 2003, with history of multiple therapeutic lumbar punctures and blood transfusions for severe anemia in the past presented with three day history of severe throbbing headache, neck pain and vomitings. Her therapeutic lumbar puncture showed pressure of >39cms of H2O twice and it did not relieve her of the raised ICP and had to finally undergo a Bitemporal craniectomy to prevent coning. Investigations: She has been thoroughly worked up for the cause of malignant rise of ICP with a MRI Brain with MRV with contrast, CSF analysis, & blood tests. There were no signs of Infection or thrombosis. Her routine blood investigations revealed a Hb of 5.8gm/dl with Hb electrophoresis showing a HbE trait with low serum Iron. Results: This is the first case report in literature showing Iron deficiency as the only cause for Malignant IIH requiring a craniectomy. It is hypothesized that raised ICP may result from cerebral hypoxia and edema, secondary to low hemoglobin and inadequate oxygen-carrying capacity associated with decreased iron-dependent enzymes. Conclusion: So our case reinforces the concept that severe anemia may lead to life threatening malignant Intracranial hypertension. A simple investigation like a complete blood count may be obtained in patients with IIH, especially in the absence of known associated factors such as obesity or medications or when appropriate treatment aimed at lowering ICP fails.

PP56/268: The recurrent nightmare of Zoster: A case series showcasing the varied neurological presentations

Shiva Narayan Pattanayak, Ray S, Tandyala N, Goel A, Sukriya S, Goyal M, Chakravarty K, Lal V

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Introduction: Varicella Zoster infection usually self-limiting and improves without complications or sequelae but sometimes in adults it is associated with neurological symptoms in a varied way. Aim: To explore the neurological effects of Varicella Zoster infection. Materials and Methods: We present the following cases we encountered in the last 6 months highlighting the various catastrophic neurological manifestations of Varicella Zoster.

Discussion: Incidence of CNS complications in varicella ranges from 0.3-2%. Though common presentations are cerebellitis, may present in different manners as aseptic meningitis, transeverse myelitis, Guillain Barre syndrome, meningoencephalitis, ventriculitis, optic neuritis, delayed hemiparesis, post herpetic neuralgia and facial paralysis. The causes can be postulated as either direct viral invasion or through immune mediated mechanisms. Typically occurs within 1 week of rash but may occur upto 3 months. Here all patients are immunocompetent in middle ages. Confirmation done by viral Ag/Ab in CSF but can be negative indicating immunological basis. Conclusions: Focus must be on keen observation of symptoms and new modalities of investigations to predict severity of complications.

PP57/271: Rapidly progressive dementia a diagnostic dilemma?

Krishnaveni Vanapala

Department of Neurology, Vydehi Institute of Medical Sciences, Bengaluru, Karnataka, India

Introduction: Rapidly progressive dementias (RPD) are neurological conditions that develop subacutely over weeks to months, or rarely acutely over days. In contrast to most dementing conditions that take years to progress to death, rpd can be quickly fatal. It is critical to evaluate the rpd patient without delay, usually in a hospital setting, as they may have a treatable condition. Methods: A 64 y old male denovo diabetic & hypothyroid with history of exposure to pigeons & parrots presented with sub acute onset cognitive impairment and acute onset left focal seizures without any other neurological symptoms. Results: blood investigations showed tsh--41.3miu/ml(0.45-5.5), TPO antibodies: 256 (0-9), ANA 3+, CSF –mild protein rise .autoimmune & paraneoplastic panel negative. Mri brain – suggestive of ADEM , MR angio – normal. Pet scan Áno metabolically active lesion elsewere in the body to suggest malignancy. A diagnosis of hashimotos encephalopathy/adem was considered. He was treated with pulsed steroids for 5 days and was put on oral steroids at time of discharge. 1 month later patient was admitted again to our hospital with sudden onset of aphasia, altered sensorium for 1 week, repeat MRI showed increased number of large bilateral cerebral hemisphere infarcts with hemorrhages. The diagnosis was revised as cns vasculitis v/s CNS lymphoma, brain biopsy -reported as possibility of CNS vasulitis he was given iv pulse cyclophosphomide 1 gram. He was given iv immunoglobulin at 0.4gram/kg body weight over 5 days and still there was no improvement. Conclusion: vasculitides are often distinguished from other rpds by brain MRI abnormalities, such as infarctss and/or hemorrhage involving both the white or gray matter.when primary cns vasculitis is suspected, cerebral angiogram or brain and meningeal brain biopsy of the affected area may be required for diagnosis.

PP58/275: Prolonged magnesium sulphate infusion in the management of super-refractory status epilepticus in a probable GABA B Autoimmune Encephallitis

Somdattaa Ray, Srijithesh PR, Kulkarni G, Alladi S

Departments of Neurology and Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Introduction: Super refractory status epilepticus is a condition associated with high rate of mortality and morbidity. We report the treatment protocol of magnesium sulfate infusion adapted for the management of a case of super refractory status epilepticus that lasted for 4 weeks. Case Report: A young lady presented in altered sensoirium with history of fever followed by uncontrolled seizures of two weeks duration. Her serum was tested weakly positive for GABA b antibody. Her seizure was not controlled despite being on multiple antiepileptics. Anesthetic induction using propofol, ketamine, midazolam and thiopentone could not control the seizure. Intravenous magnesium infusion was initiated and serum magnesium was titrated up to a final target concentration of 3.8 to 6.5 mg/dl. Development of hyoptension limited the rate of up titration of magnesium sulphate infusion. Seizure reduction was achieved after nine days of the infusion. The patient was extubated after 34 days and discharged after 55 days. She is currently on 14 months follow-up on pulse methylprednisolone and oral azathioprine. At the last follow-up patient is independent on her activities of daily living. Discussion: This case is the longest reported successful use of magnesium sulphate infusion for control of super refractory status epilepticus. Magnesium sulphate could be used as a rescue drug for patients with super refractory status epilepticus.

PP59/285: An interesting case of ataxia telangiectasia

Saksham Jain, Samhita Panda, Sanjiv Sharma, Sarbesh Tiwari

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

Introduction: Ataxia telangiectasia (a-t) is a rare autosomal recessive neurodegenerative disease caused by mutations in the a-t gene (atm) on chr 11. It is characterized by progressive neurological dysfunction in association with multisystem abnormalities and cancer predisposition. It occurs in 1 out of 88,000 live births in the USA with onset of symptoms in infancy, particularly between the ages of two and five years. Classical neurological signs include progressive cerebellar ataxia; oculomotor abnormalities, particularly ocular apraxia; movement disorders, such as chorea; and cognitive dysfunction. Case Report: 20 years male patient presented with history of inability to walk since early childhood, abnormal body movements, altered speech and growth retardation. Psychomotor development was normal until the age of 2 years when trunk swaying and inability to walk was noticed by the parents. Patient also had an altered speech and lagged behind his peers in terms of physical growth. He had normal learning and cognition but found difficulty in writing due to incoordination of upper limbs Patient was taken to local practioners for treatment but did not improve. Patient's family history showed a normal younger sister (age 18 years) and two clinically positive siblings; a younger brother (age 15) having similar features of truncal ataxia, bilateral conjunctival telengiectasias and growth retardation and a younger sister who had conjunctival telengiectasias, ataxia, repeated chest infections and died at the age of 6 years with intestinal perforation. On examination, he was found to have severe appendicular and truncal ataxia, external beating nystagmus and bilateral conjunctival telengiectasias. The International Cooperative Ataxia Rating Scale score was 3 (normal 0). Examination for neurocutaneous markers showed only a single ash leaf spot on trunk. Sensory and motor nerve conduction velocities were normal. Brain MRI showed meningeal based bone eroding lesion. Serum IGF 1 levels were slightly decreased (118.7 ng/ml (N: 131-490 ng/mL)) while AFP level were markedly increased (986.85 ng/ml (n.v. <10 ng/ml)). Serum Ceruloplasmin, Ferritin and lipid levels were within normal limits. CT abdomen showed a lobulated mass lesion in segment V/VI of liver suggestive of telangiectatic adenoma/FNH. USG guided trucut biopsy of liver favoured a well differentiated hepatocellular neoplasm with possibilities of (1) telangiectatic FNH (2) Hepatocellular adenoma. On Follow up after 6 months patient came with one month history of increased abdominal swelling. On imaging patient was found to have cholangiocarcinoma with moderate ascites. Patient was advised treatment but patient refused citing financial difficulties. Conclusion: It is now known that various diseases associated with dna damage response (ddr) manifest with neurological symptoms and immunodeficiency. These can simulate a-t phenotypically and include diseases such as a-t-like disorder (atld - mre11 deficiency) and nijmegen breakage syndrome (nbs, or nibrin/nbs1 deficiency), with microcephaly and mental retardation, without ataxia, apraxia or telangiectasia, and a-t (fresno), a mixture phenotype (a-t and nbs) with mutations in the atm gene. Thus it is proposed that this important disease be named as atm syndrome.

PP60/289: Development of a novel diagnostic platform for noninvasive detection of Alzheimer disease using brain derived serum exosomes

Rajesh Reddy C, Anusha T, Yaswant, Meena AK, Prasad G, Manda S, Prabhakar S

Department of Neurology, Apollo Hospitals, Chennai, Tamil Nadu, India

Introduction: Development of traditional biomarkers for CNS diseases in CSF is challenging due to intrinsic difficulties associated with handling CSF environment. 2011 guidelines of National Institute of Aging and Alzheimer Association (NIA-AA) research frame work proposed the use of amyloid bdeposition (A), Tau deposition(T) and neurodegeneration (AT(N)) system, both at biomarker and imaging level. Exosomes are nano particles released by all living cells, and are known to contribute to the pathogenesis of neurodegenerative diseases including AD and Parkinson-disease. Exosomes host “biocargo” (RNA, DNA and proteins) from the cell of their origin. We harnessed this property of the neuronal exosomes and established a workflow for AD diagnosis.We incorporated (ApoE genotyping) and peripheral inflammation biomarkers i.e oxidation potential, Angiotensin converting enzyme levels, serum formaldehyde levels and redox potential. A comprehensive multianalyte biomarker profile was developed using comprehensive circulating biomarkers from the periphery for AD diagnosis. Aim: To develop a non-invasive exosomal marker diagnostic in order to differentiate AD from other forms of neurodegenerative diseases. Materials and Methods: A total of 60 samples which included clinically confirmed AD patients, age and gender matched controls and other neurodegenerative diseases were analysed. Plasma exosomes were extracted, precipitated, and enriched for neuronal source by anti-L1CAM and anti-MAPT antibody immunoprecipitation approach. Neuronal exosomes (NEs) were characterized as per the guidelines endorsed by the International Society for Extracellular Vesicles and included-analysis of exosomal (protein, lipid content and protein-lipid ratio) to determine EV concentration and neuronal exosome antibody array for assessing neuronal purity; bioanalyzer profile for their nucleic acid content; and transmission Electron Microscopy for visualization. NEs were quantified for their neuropathological load by ELISA and real-time PCR, bAmyloid (Amyloid 40, Amyloid 42), Tau (total Tau, Phospho Tau -181) and neurodegeneration markers (Neurogranin, Neuro filament light and REST). Each biomarker category is rated as positive or negative. An individual score might appear as A+/T+/N−, or A+/T−/N−, etc. Results: Plasma levels of NEs were higher in AD samples compared to the age matched controls. Biochemical, biophysical characterisation of NEs were conducted for their purity. NE protein and RNA levels of Tau, P-T181-tau, neurofilament light and Aβ1–42 were significantly higher, whereas those of neurogranin (NRGN) and the repressor element 1-silencing transcription factor (REST) were significantly lower in AD compared to cognitively normal controls (CNC). Plasma levels of ACE, GSH: GSSG were reduced, when compared to their healthy controls whereas plasma levels of HCHO were higher. Conclusion: The neuronal exosome platform (modified ATN along with genetic and peripheral blood markers) developed by us provides a basis for an easy-to-access biomarker assay for blood-based diagnosis of AD and has a potential to be extended for diagnosis of other CNS disorders as well. Additionally, it could also be used as companion diagnostic for new AD targets, and for patient stratification and disease/therapy monitoring.

PP61/308: Study of spectrum of inflammatory demyelinating disease of central nervous system

Furqan Mohd Akram Khan, Dave D, Rohatgi S, Nirhale S, Rao P, Naphade P

Department of Neurology, Dr. D. Y. Patil Medical College, Hospital and Research Center, Pune, Maharashtra, India

We report the observations of twenty-five patients from Pune diagnosed with inflammatory demyelinating diseases of CNS over a period of 1 year. The aim is to highlight recent observations in inflammatory demyelinating diseases in the central nervous system. We emphasize the discriminative nature of new clinical, imaging, immunopathologic data, the rising incidence of anti-MOG antibody associated central nervous system demyelination and atypical MS due to easy and cost effective availability of anti-MOG antibody test. In the light of new findings, typical multiple sclerosis may represent a small entity in spectrum of demyelinating diseases.

PP62/310: Methylenetetrahydrofolate reductase deficiency – A rare potentially treatable cause of adult-onset complicated hereditary spastic paraplegia

Furqan Mohd Akram Khan, Dave D, Rohatgi S, Nirhale S, Rao P, Naphade P

Department of Neurology, Dr. D. Y. Patil Medical College, Hospital and Research Center, Pune, Maharashtra, India

Hereditary spastic paraplegia (HSP) is a group of clinically and genetically diverse disorders that share a primary feature, which is the causation of progressive and generally severe lower extremity weakness and spasticity. No treatment is available for these disorders. We present case of 19 year old male with severe methylenetetrahydrofolate reductase (MTHFR) deficiency manifesting as a complicated form of adult-onset hereditary spastic paraparesis. Although juvenile- or adult-onset forms of severe 5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency manifesting as complicated hereditary spastic paraplegia have rarely been described, it is a potentially reversible cause of complex hereditary spastic paraplegia.

PP63/312: A review of 43 cases of head bath triggering migraine

Yogesh Kuntalwad, Murthy JMK

Department of Neurology, CARE Hospitals, Hyderabad,Telangana, India

Introduction: Of the many triggers for migraine, head or cold water bath is an uncommon trigger and mostly described in Asia. Head bath may be the only trigger or may be associated with other triggers. Methods: The study population included 210 consecutive subjects who met the diagnostic criteria of migraine proposed by the International Headache Society 3 beta. Total 210 subjects with migraine, 43 (20%) subjects head bath was the trigger for some attacks and also had other triggering factors. In 13 (30%) subjects hot water head bath was the trigger and in 30 (70%) patients cold water head bath was the trigger. The headache following the head bath had the same migrainous features as that precipitated by other triggers. Study aims in finding any differences between the two groups. Results: The mean age: HWHB:30.38+6.56 years; CWHB: 36.03+9.06 years. Cold water trigger was more common in women as compared to men (27/30(90%) vs. 1/13(7.7%); p=0.001). Hot water trigger was more common in men as compared to women (12/13(92.3%) vs. 3/30(10%);p=0.0001). Location of headache was hemicranial with hot water trigger (p=0.0002) and it was holocranial with cold water trigger (p=0.0002). There were no differences between the two triggers including psychiatric morbidity and quality of life as measured by MIDAS scoring. Conclusion: This study suggests distinct clinical difference in patients with migraine with cold water and hot water as triggers.

PP64/318: Status dystonicus in Wilson's disease

Shashank Jaiswal, Nataraju B

Department of Neurology, Vydehi Institute of Medical Sciences, Bengaluru, Karnataka, India

Status dystonicus is a rare but truly potentially life threating movement disorder emergency. SD presentation in wilsons disease is rare. SD induced by d penicillamine is a known entity. To our knowledge this is one of a few case series of a denovo SD presentation of wilsons disease. Case Report: We report 3 cases of status dystonicus. Case 1, 2: 10 and 8 year old males born out of non-consanguineous marriage with normal birth and developmental history presented with one month h/o acute onset progressive generalised dystonia, starting in right UL which within a month progressed to generalised dystonia with risus dystonicus. KF ring positive without any history suggestive of behavioral disturbance or jaundice. ATP 7B gene +. Case 3: 17 yr old female presented with acute onset progressive generalised dystonia since 6 years, evaluated elsewhere and diagnosed with wilson's diasease. Treated with physiotherapy, pencillamine, zinc, baclofen and pacitane. Although developed kyphoscoliosis and contractures of hip, knee and ankle within a year. Mri brain- case 1, 2, 3 - T2-weighted axial MR image shows bilateral symmetric high signal intensity in the putamen and caudate nuclei. All the cases were treated with pencillamine with a slow up-titration to a therapeutic dose. Zinc, baclofen, tetrabenazine and pacitane were given for adjuvant therapy. GAS scores at presentation of case 1 was 40/76 which improved to 30 upon discharge and 10 upon follow-up a year later. Case 2 and 3 also showed comparable modest improvements.

PP65/322: Interventions for postherpetic neuralgia

Praveen Yada, Patnala N

Department of Neurology, KIMS Hospital, Thiruvananthapuram, Kerala, India

Introduction: Post herpetic neuralgia is a serious complication after herpes zoster which impairs the quality of life. Many patients donot get relief even after using multiple medications . In such patients use of Nerve blocks, para -vertebral blocks, Dorsal root ganglion blocks, Epidural Injections provide significant relief. Materials and Methods: We describe procedures done on 10 patients referred to the Pain Clinic. Out of them 5 patients had pain in Ophtahlmic division of trigeminal nerve, 1 patient in all three divisions of trigeminal nerve, 3 patients with thoracic post herpetic neuralgia, 1 patient with pain over abdomen. All patients had used multiple medications for a significant time period. The procedures done were - Supraorbital and Supratrochlear nerve blocks, intercostal nerve blocks, selective nerve root block, DRG block, paravertebral block and trigeminal nerve block. Results: Two patients stopped all medications after intervention, 2 patients required repeat intervention for which Radiofrequency ablation was done. Other patients had substantial decrease in VAS Score and number of medications required. Discussion: Appropriate interventions are helpful in pain control, improving quality of life, decreasing medications in patients with post herpetic neuralgia. Neurologists donot use the procedures frequently and use them only as a last resort. Early referral to a interventional pain specialist can improve the quality of life and decrease the pill burden and their side effects to a great degree.

PP66/329: LGI 1 Encephalitis: Is epilepsy or movement disorder

Sujit Jagtap, Aurangabadkar K, Rathod M

Department of Neurology, Bharti Hospital and Research Center and Deenanath Mangeshkar Hospital, Pune, Maharashtra, India

Introduction: Limbic encephalitis (LE) is a disease associated with epilepsy, memory deterioration, behavioral & sleep disorders. LE, implicating (VGKCs), is a potentially reversible disease affecting the CNS & quickly responsive to immunotherapy. LGI1 LE, mainly non-paraneoplastic, tumor markers and radiological examinations are negative, & different from those of typical LE. Very few case studies' about LGI1 LE currently described in literature & its treatments are also only empirical and symptomatic. Materials and Results: To analyse the clinical features, course, evolution, radiological characteristics, and treatment and natural history of LGI1 limbic encephalitis (LE). Prospectively studied patients with LE with LGI1 anti body positive during period of 2016-2018, who underwent video EEG. Six patients had LGI1 LE; M:F 2:4. Mean age of onset was 57.8 years (42-74). All had faciobrachial dystonic seizures (FBDS). FBDS were alternating side in (3/6) patients, with frequency of 5-10/ hour during 24 hour video EEG period, each lasting for 10-15 sec. Mean duration of symptoms was 17 months (2-36 months). Three patients had recent memory loss. MRI brain was normal of all except one which showed bilateral hippocampal hyperintensity. LGI1antibodies in the blood and/or CSF were positive in all. Three patients had hyponatremia (serum Na 120). FBDS subsided within 48-72 hours of receiving steroids. Mean duration of follow up was 10 months (8-24 months). Memory also improved on follow up. Video EEG showed temporal slowing in two patients and ictal temporal rthym in one patient. WHOLE body PET scan was done in all patients, none showed abnormality, while barain PET showed basal ganglia hypermetabolism. Discussion: VGKCs are the potassium channels adjusting the neuronal excitability of CNS and PNS.VGKCs mainly include LGI1 and Casper-2 antibody. LGI1 is the glycoprotein released by presynaptic membrane, and interact with presyn memb ADAM. LGI1 antibody reduces the interaction of LGI1–ADAM and aggregation of AMPAR. FBDS, cognitive disorder (mainly recent memory deterioration), epilepsy, & hyponatremia, together with disorientation and sleep disorder are predominant symptomsof LGI encepahlitis. All our patients had FDS, interestingly careful video EEG analysis showed alternating FDS in all patients. One patient was initially misdiagnosed as stress related events but careful analysis helped for diagnosis FDS. PET showing BG hypermetabolism, in concordance with origin of FDS from BG. EEG did not show epileptiform abnormality and only one patient had temporal ictal rthym while FDS, so FDS is still movement disorder or epilepsy is not clear. Three had memory impairment which improved with treatment. Patients who had delay in diagnosis & initiation of immuno-modulatory treatment had memory impairment as compared to those who were diagnosed early. Out of 6 patients, MRIs was normal in all except one which showed B/L hippocampus hyperintensities. None had neoplasm which is again important feature of LGI encephalitis although 10% may have thymoma. Conclusion: LE is rare clinically and can be easily missed or misdiagnosed. Early diagnosis of LGI1 antibody encephalitis & immunotherapy lead to good recovery from FBDS as well as may prevent development of cognitive impairment.

PP67/330: Tenecteplase versus thrombaspiration versus dual antiplatelet therapy in acute ischemic stroke

Ahamed Subir, Ghafoor F, Krishnadas NC, Rafeeque M

Department of Neurology, MES Medical College, Malappuram, Kerala, India

Introduction and Background: Increasing stroke burdren among Asian population and timely treatment with newer modalities needs research. Tenecteplase, a recently approved recombinant tissue plasminogen activator (rt-PA) for acute ischemic stroke and has greater fibrin selectivity and greater resistance to plasminogen activator inhibitor. Thrombaspiration using ADAPT technique requires comparison with recently approved rt-PA and medical management. Materials and Methods: 78 participants with stroke were enrolled in our prospective observational study. 19 received tenecteplase, 10 underwent thrombaspiration technique and 49 received DAPT presenting within 6 hours of symptom onset were enrolled in our prospective observational study. IEC approved the study and written informed consent was obtained from all study participants. Evidence of haemorrhagic stroke, age<18 years, transient ischemic attack and participants with seizures at stroke onset were excluded. NIHSS (baseline, 1 hour, 24 hours and 7 days) and mRS (1 and 3 months) were recorded in structured proforma and were analysed using SPSS. Results: Significant reduction in NIHSS score over 1 week was not observed with thrombaspiration (p=0.1) and DAPT (0.07) and significantly lower mRS at 3 months was observed with both thrombaspiration (0.008) and DAPT (0.001). Significantly lower NIHSS at 1 hour (0.008), 24 hours (0.01) and 1 week (0.009) was seen in participants receiving tenecteplase. Significantly lower mRS at 3 months was observed with tenecteplase (0.005). Significantly higher change in NIHSS was observed for thrombectomy (0.01) compared to tenecteplase and DAPT. Change in mRS did not show any significant difference between groups (0.06). 1 participant each from thrombectomy (10%) and tenecteplase (5.3%) had symptomatic intracranial haemorrhage (sICH). Participants undergoing thrombaspiration were significantly younger (p=0.006). Conclusions: Tenecteplase and thrombaspiration is superior to DAPT. Endovascular procedures also showed significantly lower mRS scores at 3 months. Tenecteplase was superior to thrombaspiration in terms of sICH.

PP68/333: Limb girdle myasthenia – A rare entity to remember

Keerthi Vyas KS, Nataraju B, Kodapala S, Soumya M, Raju S

Department of Neurology, Vydehi Institute of Medical Sciences, Bengaluru, Karnataka, India

Introduction: Limb girdle myasthenic syndromes are rare disorders described under broad heterogenous group with mixed features of myasthenia and myopathy raising the problem of the definition of an unusual form of myasthenia gravis. They may be grouped as familial and autoimmune variants constituting only 3-4% of myasthenic population. These syndromes usually spare the cranial nerves especially the oculobulbar musculature. In view of predominant proximal muscle weakness, many patients may carry the diagnosis of limb girdle muscular dystrophy or unspecified myopathy, despite normal creatine kinase. It seems that the rate of positivity of acetylcholine receptor antibody is lower than in the common form of the disease. Aim of the Study: This is for rarity and difficulty encountered in diagnosis, as they are easily confused with muscular dystrophies. The case report of the patient was retrieved and extensive literature review was done. Informed consent was obtained for this case report. Methods: We report a case of 33 yr old male presenting with chronic progressive limb girdle weakness sincen 7 months. He had fluctuating symptoms induced by moderate exercise with no oculobulbar weakness or cranial nerve disturbances. The weakness was predominantly in the pelvic girdle than pectoral region and had waddling at hips while walking. The primary diagnosis was chronic progressive limb girdle myopathy. In view of fluctuating sympoms, absence of of marked wasting, and normal serum creatine kinase activity limb girdle myasthenia was suspected. Finally diagnosis was established by occurrence of a characteristic positive decremental response to repetitive stimulation of the proximal muscle, by positive neostigmine test, and positive acetylcholine receptors antibody. Muscle biopsy was essentially normal. Patient responded to acetylcholinesterase inhibitors with significant improvement in MRC motor power grading. Conclusion: The review of literature showed only 25 reported cases of autoimmune limb girdle myasthenia out of which only 12 presented with Acetylcholine receptor antibodies, similar to our case. However this unusual form of chronic limb girdle weakness has to be recognised to have a strong index of suspicion as it has therapeutic implications.

PP69/336: Lateral medullary syndrome (Wallenberg syndrome) causing persistent headache (trigeminal autonomic cephalalgia)

Murugan P, Justin C, Muthukumar, Pravina L, Vasagam LN

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

Headcahe can be an accompanying symptom of cerebrovascular disease (upto 38% of cases) mostly depending on stroke etiology and localization. It is very common in posterior inferior cerebellar artery infarction with a frequency of upto 76%. In patients with Wallenberg's syndrome headache resembling Trigeminal autonomic cephalalgia is induced in lateral medullary ischemia infarction. Case Report: 38 year old man, a cook by occupation came with left unilateral headache, swaying to left, nasal regurgitation of fluids, vertigo, drooping of left eyelid, decreased sensation on left side of face and right half of his body and unilateral absence of sweating on left side of face, They occurred 1 day prior to admission to the hospital. Patient was a tobacco chewer and chronic alcoholic. MRI showed T1 hypointense, T2 Flair hyperintense lesion and Diffusion restriction in left lateral medulla. MRA and MRV was done and Vertebrobasilar artery dissection and Cortical Vein Thrombosis were ruled out. Patient was treated with Aspirin and Atorvastatin. Symptoms of regurgitation, ataxia and vertigo improved during hospital stay except patient had persisten left unilateral headache. Patient was initiated on symptomatic drugs like Paracetamol, Diclofenac, Amitryptiline and Pregabalin. There was no relief of symptoms with persistent severe headache. Patient was initiated on gradually increasing dose of Verapamil till a maximum of 160 mg per day, following which headache subsided gradually over 2 days and patient was pain free from day 3. Discussion: TACs are primary headaches characterized by typical pain and autonomic features. Central nervous system lesions can rarely present with cluster-like or SUNCT-like symptomatology The hypothalamus, via the hypothalamospinal tract, is a regulatory centre for integration of sympathetic and parasympathetic systems. Experimental studies with functional MRI and PET showed hypothalamic activation during TACs attack. Moreover, stereotactic hypothalamic stimulation has been successfully used in drug-resistant patients, indirectly confirming the hypothalamic involvement in TACs' pathophysiology. Hypothalamospinal tract lies in dorsolateral medulla; it is constituted by first order neurons responsible for orthosympathetic innervation of ipsilateral half face and body and projects to peri-acqueductal gray matter, thus activating the trigeminovascular system that is a well-known pain generator of headaches. In this area, descending fibers of the hypothalamospinal tract carry sympathetic innervation to the pericarotid plexus. In the subacute phase of stroke, Horner's syndrome, as well as orthostatic hypotension, was symptomatic of a sympathetic impairment, while tearing and ocular injection reflected a parasympathetic activation, The persistence of pain attacks with vegetative involvement in a chronic phase of stroke was due to an aberrant activation of trigeminovascular system by hypothalamospinal tract via the peri-acqueductal gray matter. These clinical features resembled cluster headache. The weak response to diclofenac and acetaminophen and the dramatic improvement after verapamil therapy supported this hypothesis. In fact, while NSAIDS may be effective in treating paroxysmal hemicrania by inhibiting NO-induced dural vasodilation, verapamil acts mainly as neuromodulator in the hypothalamus. In summary, our patient's case supports the hypothesis of a dysfunction of the hypothalamospinal tract in the pathophysiology of both pain and autonomic features of TACs.

PP70/339: Recurrent seizures and acute fulminant leukoencephalopathy in endemic countries: What did I miss?

Arushi Saini, Singanamalla B, Kesavan S, Singh P

Department of Peadiatric Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: Subacute sclerosing panencephalitis (SSPE) may mimic several neurological and psychiatric syndromes including acute encephalitis syndrome, acquired demyelination, psychosis, catatonia, movement disorders including status dystonicus, visual los, pseudotumour cerebri and dementias. The course is typically sub-acute to chronic with a variable incubation period over years. Methods: An 11-year-old, developmentally normal and immunized boy for age presented with abnormal behavior followed by progressive loss of cognitive functions and rapid deterioration in consciousness over the past 2 weeks. There was no history of any exanthematous illness in the past. He was born to non-consanguineous parents and family history was unremarkable. On examination, he was in a minimally conscious state with generalized rigidity, brisk muscle stretch reflexes, bilateral Babinski sign, normal fundii and periodic eyelid myoclonus. Results: Magnetic resonance imaging showed diffuse white matter and thalamic hyperintensities with elevated lactate and diffusion restriction. Electroencephalograph showed periodic complexes. Cerebrospinal fluid analysis and serum showed elevated measles antibody titers. He was started on oral isoprinosine, levetiracetam, nasogastric feeding and supportive care. At 1 month follow-up, he is in stage 4. Conclusion: Our case highlights that a high index of suspicion is needed to detect SSPE in its atypical and rare forms, especially in endemic countries. Acute fulminant SSPE with diffuse leukoencephalopathy is an extremely unusual presentation in children.

PP71/342: Demyelinating peripheral neuropathy in a case of classical polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome with castleman-like histology: A rare, atypical but treatable presentation

Manish Mahajan, Bansal S, Singh S, Singh PK, Radhakrishnan R, Goel D

Department of Neurology, Artemis Hospital, Gurgaon, Haryana, India

Introduction: POEMS (Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes), a paraneoplastic syndrome due to an underlying plasma cell disorder (PCD), has 'demyelinating polyneuropathy' as a mandatory major diagnostic criterion. Between 11% and 30% of POEMS patients who have a documented clonal PCD also have documented Castleman disease (CD), a rare usually polyclonal lymphoproliferative disorder, or rarely Castleman-like histology. A different spectrum of peripheral neuropathies (PN) are associated with CD. CD-PN is sensory predominant and is the mildest phenotype, whereas CD-POEMS is a more severe sensory and motor neuropathy. Case Report: 45-year-old gentleman presented to us with a slowly progressive lower-motor-neuron type symmetrical ascending quadriparesis of one-and-a-half years duration associated with areflexia, distal wasting without fasciculations and length-dependent pattern of painful sensory impairment. He also noticed a painless left axillary and left supraclavicular lymphadenopathy for the last one-and-a-half months. Systemic examination added hypertrichosis, and mild hepatosplenomegaly that was confirmed on CECT abdomen. Optic fundi examination and echocardiogram were unremarkable. NCS showed predominantly demyelinating sensorimotor polyradiculoneuropathy without any evidence of conduction block. CSF analysis showed cyto-albuminological dissociation. TSH was 8.86 IU/mL. Serum protein electrophoresis (SPEP) detected abnormal M proteins, identified by immunofixation as IgG lambda at a concentration of 0.3 g/dL. LN biopsy revealed reactive and atretic follicles with onion skinning. There was expansion of interfollicular areas with infiltration by monoclonal plasma cells in sheets showing lambda-restriction on IHC. PET?CT found no hypermetabolic activity, and MRI total spine was negative for bony abnormalities. Bone marrow biopsy did not show any interstitial increase or aggregates of plasma cells. A diagnosis of demyelinating PN with classical POEMS syndrome with Castleman-like histology was considered. Cyclophosphamide-Bortezomib-Dexamethasone based chemotherapy regimen has been started with a plan for autologous stem cell transplant and survival is typically excellent. Conclusion: Appropriate and extensive diagnostic work-up for chronic demyelinating polyneuropathy is required and may be rewarding in accurately establishing the underlying hematologic disorder that can guide with the treatment and can have implications on the prognosis.

PP72/355: An interesting case of sarpagandha induced neurolept malignant syndrome

Monika Porwal

Department of Neurology, Mahatma Gandhi Memorial Medical College, Indore, Madhya Pradesh, India

Introduction: Neuroleptic malignant syndrome (NMS) is d/t blockade of dopamine receptors, which is potentially fatal adverse effect of neuroleptic drugs, often during 1st week or after increasing its dosage. It is characterized by muscular rigidity, fever, altered mentation and autonomic dysfunction. Rauwolfia serpentina a/k/a “Sarpagandha”, its roots contains 200 alkaloids, mainly Resperine, which is used in both Homeopathy and Ayurveda for managing hypertension and mental disorders including schizophrenia, bipolar disorder and insomnia. Case Report: A 52-year-old woman with no previous comorbidities got hospitalized for hyperthermia, perspiration, severe tremors of the limbs, head and jaw, generalized stiffness, and bradykinesia since 2 weeks. All blood investigations, chest X ray, USG abdomen were normal except for raised CPK levels & Lactate levels. And this case is particularly interesting because inspite of repeatedly enquiring we were not getting any clue to etiology for sub-acute onset extrapyramidal features. Finally we noticed that her husband offered her few dried roots to eat, and on enquiring he mentioned history of psychosis and irrelevant talking in patient since 8 to 10 years, and was taking “SARPAGANDHA ROOTS” from Ayurvedic medical shop in doses 1 -2 roots twice a day, since last few weeks she was eating 8 to 10 such roots 4 – 5 times a day (self overmedicating). That was the thing that clicked our minds and compelled us to read about these roots and allowing to finally diagnose NMS and eventually she got improved with the therapy offered. This is the FIRST CASE in literature mentioning NMS due to overdose of RESERPINE d/t content in sarpagandha roots.

PP73/357: A child with macrocephaly

Mahesh N

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

2 years old male child presented with H/o large head and developmental delay Has bipyramidal signs. H/o seizures by 4 years of age left focal, afebrile Started with AED/Levitiracetam, MRI brain showed evidence of leukodystrophy with subcortical cysts, Genetics showed mutation in MLC 1 gene as well as insignificant mutation over TREX1 for Aicardi Goutieres syndrome. Clinical correlation must be needed while interpreting genetics. Presented for rarity.

PP74/359: Clinical profile of patients with anti N-methyl-D-aspartate receptor encephalitis in India

Koustav Jana

Department of Neurology, Medical College, Kolkata, West Bengal, India

Introduction: Anti N-methyl-D-aspartate receptor (NMDAR) encephalitis is a potentially treatable encephalitis of autoimmune origin. Indian data on anti-NMDAR encephalitis is limited. Aims: The aim was to study the clinical profile of patients with anti-NMDAR encephalitis in India. Materials and Methods: 9 patients with symptoms compatible with anti-NMDAR encephalitis who tested positive for anti NMDA-receptor antibody in their cerebrospinal fluid (CSF) were enrolled in the study from January, 2018 to June, 2019. Relevant data including their clinical features, CSF findings, findings of contrast enhanced MRI of brain and electroencephalography (EEG) as well as the treatment received were noted. All the patients underwent radiological screening for teratoma by contrast enhanced CT scan of abdomen. Results: The mean age of the patients studied was 16.3 years (Range: 3-28 years) with a female preponderance (66.7%). 4 patients (44.4%) had fever at onset. All our patients excepting one had drug refractory motor seizures; of which 6 (66.7%) were noted to have a definite focal onset. 5 patients (55.6%) had psychotic symptoms; of which 4 (44.4%) had formed visual hallucinations. All our patients had some behavioral disturbance during the disease course. In fact, 4 of our patients (44.4%) initially went to psychiatrists for evaluation. 5 patients (55.6%) had abnormal involuntary movements; 4 patients (44.4%) had dystonia. 3 of our patients (33.3%) had catatonia. Only 3 patients (33.3%) had an abnormal brain MRI in the form of T2/FLAIR hyperintensities in the brain parenchyma; none had contrast enhancement. CSF study was normal in 5 of our patients (55.6%). Increased CSF protein was found in 4 patients (44.4%); CSF pleocytosis in 3 patients (33.3%). CSF protein in the study population ranged from 23-110 mg/dl whereas cells in the CSF ranged from 1-30/ Serum samples of 3 of our patients (33.3%) were negative for anti-NMDAR antibody by Indirect Immunofluorescence method, although their undiluted CSF samples tested positive by the same method. 7 of our patents (77.8%) had background slowing in EEG; 2 had delta brush; 2 had focal epileptiform discharges. Only one patient was found to have an ovarian teratoma. All patients received first-line therapy (intravenous pulse methylprednisolone, Intravenous Immunoglobulin or plasmapheresis). 5 patients (55.6%) were treated with second-line therapy (Rituximab). 1 patient, who was not responding to Rituximab, was treated with Tocilizumab. Conclusion: New onset psychiatric symptoms, drug refractory focal onset seizure or abnormal involuntary movement in a young female or child should raise the suspicion of anti-NMDAR encephalitis which may often have a normal CSF and MRI of brain.

PP75/371: AN interesting case of toxin induced peripheral neuropathy (mixed arsenic and lead toxicity) – Contaminated water source

Monika Porwal

Department of Neurology, Mahatma Gandhi Memorial Medical College, Indore, Madhya Pradesh, India

Background: Peripheral neuropathy have numerous causes and diverse presentations; hence, a systematic and logical approach is needed for cost-effective diagnosis, especially of treatable neuropathies. Lead is a ubiquitous and versatile metal that has been used by mankind since many years. It is a toxic heavy metal and considered as one of the most important environmental poisons in the world. Arsenic exposure may be occupational (smelting, mining, tanning, and numerous manufacturing processes), environmental (groundwater contamination, combustion fumes of contaminated fossil fuels and arsenate treated wood, and contact with arsenic containing pesticides and herbicides) and iatrogenic medicinal exposure including folk and herbal medicines. It has been recognized as a major public health risk, particularly in developing countries and exposure has a special consequence on neurotoxicity. A 24 year old Male had been working as labourer (Making buildings and boring Wells), 10-12 hours daily for 10 years. He got admitted to hospital for Pain, Numbness and Tingling sensation and Weakness of all four limbs (LL > UL) since 2 months. Also Dark pigmentation of skin of both lower limb since 1 month, Wasting of both hands with Nausea, Vomiting, Epigastric pain since 20 days. Hematological and clinical findings were indicative of lead and Arsenic toxicity. His lead and Arsenic levels were investigated which showed abnormally high levels (Lead = 260.54 μg/L and Arsenic levels > 10 μg/L). The nerve conduction studies revealed Severe Symmetrical Peripheral Neuropathy involving all four limbs (lower limbs>upper limbs) with both Sensory & motor fibres involved and Mixed Demyelinating and Axonal type. This case was finally treated as lead and arsenic toxicity with peripheral neuropathy. The important and interesting fact about this case is that there were Clustering of cases within same family (father, mother, sister including his wife and 2 miscarriages) suggesting Nutritional and toxin exposure on our priority list. All patients and drinking water sample had elevated lead and arsenic levels that were in the toxic range. The concerned regulatory bodies should make it mandatory for checking metal content of water used through boring and people using this water to undergo regular health checkups to detect signs of lead and arsenic poisoning and must ensure that people are aware about the ill effects of exposure to these metals. We recommend frequent toxin screening of patients presenting with severe peripheral neuropathy before they become severely and irreversely crippled. Chelation therapy removes such toxins from the blood and soft tissues and chronic exposure often requires repeated courses of treatment.

PP76/379: A simplified profiling of reading and writing errors in a bilingual person with mixed aphasia: A single case study

Sonal Chitnis, Mudgalkar V, Bhatnagar S, Pauranik A

Department of Neurology, Bharti Hospital and Research Center and Deenanath Mangeshkar Hospital, Pune, Maharashtra, India

Background: Alexia with agraphia is an acquired neurological disorder in which person exhibits partial or complete impairment of reading and writing skills. It can either be associated with aphasia or exists alone which is termed as pure alexia with agraphia. Objectives: The study is an effort to explore a comparative investigation between paralexic, paragraphic, and paraphasic errors in person with aphasia and discusses the same towards an aid into cognitive neurorehabilitation. Methods: The study presents systematic descriptive error analysis of generative reading and writing errors in 55 year old man who presented with mixed aphasia. Client also showed cognitive linguistic impairment along with sensory motor integration deficits. Marathi translated version of WAB and HRWIT, and standardized reading passage in Marathi were administered. Results and Discussion: Hindi Reading Writing Indore Test (HRWIT, Pauranik, 2013) results showed reading and writing impairment in both true and non word stimuli. ORLA PCA, haptic perception, imagery techniques helped on high imageability over low imageability stimuli elicited better responses. On testing function words both semantic and phonemic paralexias were observed along with neologism. He could write all the letters, numbers and also simple words, he could copy & write simple words but not phrases. Conclusion: Criterion based assessment and intervention which focuses on client's functional needs and skills in areas of reading and writing is important in individuals with aphasia for better therapeutic outcome and rehabilitation.

PP77/404: A case report on anti-NMDAR encephalitis

Sharath chandra Pasham

Department of Neurology, Mahatma Gandhi Memorial Medical College, Indore, Madhya Pradesh, India

Anti-NMDA receptor encephalitis is an immune mediated syndrome with antibodies to neuronal cell surface antigen causing profound dysregulation of neuronal transmission. It is frequently associated with ovarian tumors and young females are disproportionately affected. Patients may present with a constellation of signs and symptoms like psychiatric abnormalities, seizures, memory deficits, altered sensorium, dyskinesia and autonomic dysfunction. It's potentially fatal if left untreated and almost 80% have full recovery on treatment. We describe this syndrome in two different patients and review the literature for differential diagnosis, approach and treatment options and prognosis.

PP78/409: Fluctuating ophthalmoplegia in a patient with leptomeningeal carcinomatosis: An unusual presentation

Syed Osman, Krishnamani KV, Jampana PR, Kumar BS, Reddy MY, Jaiswal SK, Lalitha P, Murthy JMK

Departments of Neurology, Medical and Radiation Oncology, Institute of Neurosciences, CARE Hospitals, Hyderabad, Telangana, India

This report represents a rare case of fluctuating ophthalmoplegia in a patient of leptomeningeal carcinomatosis. Case Report: A 56-year-old female, a known case of carcinoma breast (diagnosed and treated in 2017) admitted with intermittent holocranial headache of 20-days duration and 2 episodes of right focal-onset unaware and bilateral tonic-clonic seizures. Examination revealed terminal neck stiffness. On day-2 of admission she developed diplopia with drooping of right eyelid progressing to complete oculomotor nerve palsy with dilated and non reacting pupil which improved within an hour. On day-3 she developed headache and left third nerve palsy which improved within an hour. Contrast MRI brain revealed leptomeningeal and cranial nerve enhancement and MRA was normal. Lumbar Puncture: normal opening pressure and CSF analysis: pleocytosis (480 cells with 75% Lymphocytes), malignant cells, elevated protein (710 mg/dl). EEG done at admission and during episode of ophthalmoplegia showed no epileptiform activity. She was treated with antiepileptic drug, dexamethasone and whole brain radiotherapy and supportive care. The possible explanation for the transient third nerve palsy would be intermittent entrapment of the cranial nerves in the highly vascular basal meningeal metastases.

PP79/415: Association of quality of life with lifestyle and metabolic profile in people with epilepsy: A correlative analysis

Sudhindra Vooturi, Kalva S, Yazala M, Jayalakshmi S

Department of Neurology, Krishna Institute of Medical Sciences, Secunderabad, Telangana, India

Background: People with epilepsy (PWE) often experience stigmatization and social isolation, resulting in a poor QOL, despite adequate seizure control on medications. However, the effect of poor QoL on quantifiable physical and mental components of health has rarely been investigated. We aimed to evaluate the association between poor QoL with physical fitness, lifestyle and metabolic parameters in PWE. Methods: In this prospective, observational study of PWE with seizure controlled on anti-epileptic drugs, 186 subjects aged 18–60 years recruited. Data on anthropometry, physical activity, sub-maximal exercise test and metabolic parameters (lipid profile, glycosylated haemoglobin, thyroid profile and vitamin profile) was obtained from the participants. Additionally, sleep and activity patterns were also documented. Self-reported SF-12 questionnaire was administered. Data from SF-12 questionnaires were used to calculate physical component score (PCS) and mental components score (MCS). Pearsons correlation was used to investigate the association between PCS and MCS with other study variables. Results: The mean age of the cohort was 26.49±9.94 years with 106 (57.0%) men. There were no differences between gender for scores on SF-12. The average score on PCS was 46.1±8.1 and MCS was 45.4±10.3, both lower than the population average. Importantly, 112 (60.2%) and 121 (65.5%) PWE had lower scores than population average on PCS and MCS respectively. A positive correlation was observed between PCS and distance walked in six minutes (r=0.296;p<0.001) and stepper test (r=0.266;p<0.001). Whereas a negative correlation was observed between PCS with LDL values (r=-0.158;p=0.034) and WBC (r=-0.160;p=0.035). Higher MCS were associated with longer duration of sleep (r=0.188;p=0.014) and lower glycosalated haemoglobin (r=-0.182;p=0.015). Conclusions: People with epilepsy have lower QoL than general population. Physical component score of SF-12 is associated with increased physical fitness and lower LDL. Whereas higher mental component score is associated with better sleep duration. Importantly, lower MCS and not PCS is associated with increased glycosalated haemoglobin levels.

PP80/416: LGI 1 autoimmune encephalitis and phenytoin induced generalised lymphadenopathy: A case report

Santhosh Kumar, Murthy JMK, Osman, Reddy M, Jaishwal S, Pidaparthy L

Department of Neurology, CARE Hospitals, Hyderabad, Telangana, India

A 62-year-old male presented with history of recurent severe hyponatremia with symptamatic seizures, progressive cognitive impairment, psychiatric disturbances, and faciobrachial dystonic seizures since 6 months. He was prescribed phenytoin sodium for seizures at other facility. While using phenytoin he developed generalised lymphadenopathy. Work-up for cognitive impairment revealed CSF and serum LGI 1 antibodies. MRI revealed bilateral mesial temporal lobe hyperintensities. Total body PET – CT showed bilateral mesial temporal and basal ganglia hypermetabolic activity. Biopsy of lymph node showed reactive lymphadenopathy (phenytoin induced). Antiepileptic drug was changed to levetiracetam. He was given immunomodulation therapy. At 2 months follow-up lymph nodes resolved. Faciobrachial dystonic seizures, psychiatric disturbances, and cognitive impairment, all resolved.

PP81/420: Clinical, imaging and treatment response of 100 consecutive patients with seizure emergency: Experience from a tertiary care hospital

Joydeep Mukherjee, Kishore P, Gautham B, Mundlamuri RC, Kenchaiah R, Asaranna A, Iyer V, Sinha S

Departments of Neurology and Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Introduction: Seizure emergency is one of the common presentations to the neurology emergency services. This can be new onset, cluster seizure or status epilepticus. Hereby we are presenting a descriptive analysis of 100 consecutive patients presented to our emergency services. Materials and Methods: Data was collected prospectively from the patients attending emergency services of NIMHANS, Bangalore in the month of May 2019 with a seizure-related emergency, after informed consent. Patients were treated with antiepileptic bolus therapy. Data regarding detail clinical, imaging, drug treatment response and side effect details were noted during their stay in emergency services ranging from 6-24 hours. Results: Among 100 patients presented in emergency room following seizures, male was 65 (65%) and female was 35 (35%), age range was 6-75 years. Ten (10%) patients presented in status, twenty-four (24%) as new onset seizure and sixty-five (65%) as cluster seizure. Seventy-three patients (73%) had GTCS and rest (27%) had focal seizure, seizure duration was from new onset seizure to 65 years, seizure episode lasted for 15 seconds to 2 hours, presented in emergency room within 0-4 days of last seizure. Triggers noted as alcohol 21% (n=21), sleep deprivation 5% (n=5), head injury 2% (n=2), hot water bath 1% (n=1), ganja abuse 1% (n=1) and fever 1% (n=1). Focal neurological deficit present in 9% (n=9). Poor anti-epileptic drug compliance was in 33 (33%) patients. Twenty (20%) patients were presented in drowsy state. Among them seven (35%) patients regained consciousness after antiepileptic bolus. Neuroimaging was grossly normal in 36 (36%) patients. Levetiracetam was given in 31 (31%), phenytoin given in 59 (59%), valproate given in 10 (10%) patients according to appropriateness. Bolus dose started within 10-30 minutes of patient's entering the busy emergency room. Adverse effects noted during infusion and hospital stay were valproate causing vomiting (20%) (n=2), drowsiness (10%) (n=1) and breathlessness (10%) (n=1); levetiracetam causing giddiness (3%)(n=1), vertigo (3%) (n=1), drowsiness (3%) (n=1) and vomiting (3%) (n=1); phenytoin causing drowsiness (10%) (n=6), vertigo (7%) (n=4), headache (2%) (n=1), vomiting (2%) (n=1), abdominal pain (2%) (n=1) and pain in injection site (2%) (n=1). 2 (2%) patients needed repeat bolus to control seizure. Conclusions: This study shows the spectrum of seizure emergencies, common triggers, treatment response and the side effects in a prospective consecutive cohort. Limitations: Limited number of patients (100) and a short study period (one month). Further Scopes: Patients will be followed up in OPD to note the sustained effect of antiepileptic treatment, adherence to treatment and long-term outcome.

PP82/421: Anterior cerebral artery strokes presenting with fluctuating weakness: A case series

Rithvik Ramesh, Shankar V, Shanmugam S, Hazeena P

Department of Neurology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Introduction: Strokes involving anterior cerebral artery are known for their predominant lower limb distribution of weakness apart from other additional distinctive features of the weakness such as motor neglect, anterior disconnection syndrome and alien hand sign. Fluctuation of neurological deficit has not been reported priorly. Review of literature shows that fluctuation of neurological deficits (defined as a change in NIHSS score of > 4) has been associated with reperfusion therapy, large vessel occlusion and subcortical strokes in MCA territory. We present a series of two ACA territory infarction cases, with waxing and waning of the weakness during the acute period, with eventual improvement. Case Vignette: Case 1: A 55-year male presented to the emergency room after 2 hours of onset of left hemiparesis, leg more than arm. Clinical assessment revealed rapid improvement of 6 points in NIHSS score (Hemiparesis) from the time of presentation. He was subsequently thrombolysed after CT assessment and shifted to ICU, where his NIHSS again dropped 5 points. MRI brain revealed right ACA territory infarct. In his further course, the patient had two more fluctuations (Mean change in NIHSS score 5), and was eventually discharged after 1 week with mRS of 1. Case 2: A 64-year male, recent stroke (right frontal infarct) three months back on antiplatelets, sustained a fall and developed left SDH. He was admitted under neurosurgery and underwent a procedure for SDH during which time the antiplatelets were withheld. Seven days after stoppage of the antiplatelets the patient developed weakness in his left lower limb more than upper which fluctuated over the course of two days (mean change in NIHSS 5). MRI revealed anterior cerebral artery infarct. Conclusion: We present this case series to highlight the possible pendular nature of fluctuations in the patient's weakness in ACA strokes leading to panic and additional imaging, which have been previously described only in MCA strokes.

PP83/424: Clinical profile of patients with distal bimelic amyotrophy – A retrospective study

Vaibhav Tandon, Nair SS, Wadwekar V, Nair M

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

Introduction: Distal bimelic amyotrophy (DBMA) is clinically a distinct entity although the age of onset and pattern of muscle involvement is characteristically similar to brachial monomelic amyotrophy (BMMA). Classically, adolescent and young adults are affected with symmetrical/asymmetrical onset of weakness and wasting of distal upper limbs. Our objective was to study the clinical profile, electrophysiological and radiological features in a large and distinct series of DBMA from a tertiary care centre in Kerala. Aim: To study clinical profile of patients with Distal Bimelic amyotrophy. Materials and Methods: We identified 24 subjects with bimelic amyotrophy among 56 patients who presented with distal onset upper limb amyotrophy between 2008 and 2018. The clinical, electrophysiological and radiological information was extracted from the medical records and analysed. Results: A significant majority of the patients (23/24) were males. Mean age at onset was 20.08 years, mean age of diagnosis was 22.65 years and mean duration of illness at presentation was 52.6 months. Onset was asymmetrical in onset in all cases. Wasting was present only distally in the hands in 12 (50%) and forearm and hand wasting was present in 10 patients (41.7%). Fasciculations were reported by 8 (33.33%) patients. Hand muscle weakness was seen in 18 (75%) patients and forearm and hand muscle weakness was seen in 5 (20.83%) patients. Deep tendon reflexes were normal in 15 patients (62.5%) and hyperactive in 5 (20.8%) patients. Nerve conduction studies were abnormal in 13 patients (54.1%), normal in 6 patients (25%) and were not available for analysis in 5 patients. EMG showed fasciculations, fibrillations and giant/large motor unit action potentials in all 15 patients where EMG was available for analysis. MRI carried out in 13 patients showed abnormalities in 12 patients and was normal in 1 patient. Abnormalities included localised cervical cord atrophy in 8 (61.5%), asymmetric cord flattening in 4 (30.7%), abnormal cervical curvature in 3 (30.7%), parenchymal signal changes in lower cervical cord in 4 (30.7%), forward displacement of posterior dura in 1 (7.6%), and epidural flow voids in 1 patient (7.6%). Conclusion: DBMA is a phenotypically distinct, but closely related clinically and radiologically to BMMA or Hirayama disease. The electrophysiological and imaging features point to a pathology at the cervical spinal cord level as the etiology similar to BMMA.

PP84/439: Charcot–Marie–Tooth disease: Mutation pattern in patients diagnosed at NIMHANS

Atchayaram Nalini, Polavarapu K, Kumar VP, Vengalil S, Raju S, Nashi S, Mahajan N, Arunachal G

Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Background and Aim: Reports on genetic studies in CMT from India is exceedingly rare. This study aims enumerates the frequency of mutations and genotype-phenotype correlates. Materials and Methods: Whole exome sequencing and clinical exome were performed in clinically suspected cases of CMT. 44 genetically confirmed cases were studied. Results: Putative disease-causing mutations were identified in 44 cases. The majority from all groups had onset in 1st decade of life. PMP22, FIG4, NEFL1 and GJB1 had adult to late adult onset. Most common causative genes identified are [SH3TC2 =8(c.3325C>T(p.Arg1109Ter); c.3511C>T(p.Arg1171Cys);c.1969G>A(p.Glu657Lys;c.385+1G>A(5'splicesite); c.3325C>T (p.Arg1109Ter),c.3325C>T(p.Arg1109Ter);c.1096_109del (p.Thr366SerfsTer5);c.3325C>T (p.Arg1109Ter); c.1267G>T (p.Glu423Ter); c.2036T>G (p.Leu679Arg)]; [GDAP1=7 (c.112C>T(p.Gln38Ter); c.500_501del(p.Thr167ArgfsTer3); c.817C>T(p.Arg273Ter); c.844C>T(p.Arg282Cys); c.361del(p.Val121TyrfsTer26); c.691C>T(p.Pro231Ser); c.1077G>T(p.Ter359TyrextTer14); [MFN2=7(c.787T>C(p.Ser263Pro); c.1687C>T (p.Arg563Cys); c.280C>T(p.Arg94Trp); c.1069A>G;p.Lys357Glu; c.310C>T (p.Arg104Trp); c.280C>T(p.Arg94Trp); c.383A>G (p.His128Arg); c.280C>T (p.Arg94Trp)]; [PMP22 =6], [GJB1=2 (c.415G>A (p.Val139Met), c.43C>T (p.Arg15Trp)]; [HSPB1 = 2 (c.452C>T(p.Thr151Ile),c.116C>T(p.Pro39Leu);[MTMR2=2(c.57_78dup(p.Gly27AsnfsTer20); c.304C>T(p.Arg102Ter), c.2908C>T (p.Arg970Trp). Mutations in other known CMT genes occurring in single cases included [DNAJB2 (: c.208_209insG (p.Glu71GlyfsTer48)]; [FGD4 (c.1672C>T (p.Arg558Ter)]; (FIG4 (c.272C>T (p.Ser91Leu)); GARS (c.843G>A (p.Met281Ile); HK1 ((c.2T>C); [NEFL (c.23C>G (p.Pro8Arg)); [PLEKHG5 ((c.307G>A (p.Val103Met)); [SCN9A (c.1642C>T (p.Arg548Ter)]; [SURF1 (c.870dupT (p.Lys291Ter)). In PMP22 cases, heterozygous duplications were identified in 5, and deletion in one case. In 3 cases with PMP22, GDAP1 and MTMR2 mutation, additional significant rare variants were identified in AARS, PDK3 and PRX genes respectively, suggesting a possibility of dual pathogenesis. A compound heterozygous mutation was identified in HGS (Hepatocyte growth factor related tyrosine kinase substrate) gene, c.700C>T (p.Pro234Ser); c.854C>T (p.Thr285Met), a novel gene that has not been reported in humans till date. HGS mutations in mice have been shown to result in abnormal myelination in peripheral nerves. Conclusions: The mutation pattern in 44 CMT cases from a single national institute is described in this report. The tests performed were Whole exome sequencing in Trios and Clinical exome sequencing in probands. The commonest disorder was SH3TC2, followed by GDAP1, MFN2 and PMP22. The majority had onset in the 1stdecade of life with more severe form of the disease and rapid course while the group with PMP22, NEFL1, GDAP1 and FIG4 had adult onset and milder course.

PP85/443: Mercury masquerading as motor neuron disease

Deependra Singh, Lal V, Kamlesh, Lakhanpal V

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: The causes of sporadic amyotrophic lateral sclerosis (SALS) and other types of motor neuron disease (MND) remain largely unknown.[1] Heavy metals have long been implicated in MND, and it has recently been shown that inorganic mercury selectively enters human locus ceruleus (LC) and motor neurons. Several case-reports of mercury poisoning have demonstrated convincing ALS-like clinical symptoms, leading to postulate a causal relationship.[2],[3],[4] Materials and Methods: We describe an Individual, who has presented with h/o developing abnormal movements of muscle over upper limb and along with tingling and numbness of both upper limb and lower limb with no other neurological complaints. Clinically he has fasciculation, over tongue and biceps, with normal power. Biceps, triceps and knee (3+), rest 2(+). Plantar B/L flexor, rest of the examination normal. He was also detected to have significant proteinuria. He under- went kidney biopsy which revealed secondary membranous glomerulonephritis. His EMG revealed – fasciculation noted in Biceps, Brachioradialis, vastus and gastronemiusÁ neurogenic EMG S/O ant horn cell disorder, NCS –normal study. Mercury level were high 24 hr Á 57.30 (< 20), His other work-up for motor neuron disease was negative. On repeated questioning to look for the cause of motor neuron disease to rule out other causes masquerading as MND, he came with h/o applying some “BHASM” over his dermatological condition which he developed approx 2- 3 months prior to developing symptoms. Discussion: Toxic metals, and mercury in particular, have long been suspected to play a part in the pathogenesis of motor neuron disease.[2],[4],[5] Mercury can initiate oxygen free radical formation, induce excitotoxicity, reduce DNA, RNA and protein synthesis, cause epigenetic changes, activate autoimmunity, and interact with microtubules,[4] all mechanisms that have been implicated in ALS.[6] Electron microscopic studies indicate that mercury binds selectively to intracellular sulfhydryl-rich membranes such as those of mitochondria, the nucleus, the Golgi apparatus, the endoplasmic reticulum and lysosomes,[6] organelles whose functions have been reported to be impaired in ALS. [5,7] In this individual, mercury not only presented with features of motor neuron disease, but it also caused kidney damage. His complete evaluation for other etiology for motor neuron disease was negative. Pt was put on chelating agents and subsequent to which his symptoms improved. His repeat mercury level came down to 30. Conclusions: Motor neuron disease is a relentlessly progressive disorder with median survival from the time of diagnosis is three to five years. So correct diagnosis is utmost important before committing on motor neuron disease. This case also tells about the importance of proper history taking in neurology to come to proper diagnosis. Pt is still on chelating agents and “ BHASM” is sent for evaluation of mercury level. (report awaited).

PP86/449: Role of endoplasmic reticulum stress in the postsurgical outcome of patients with refractory epilepsy and focal cortical dysplasia

Kishore Madamanchi, Phanithi PB, Panigrahi MK, Jayalakshmi S

Department of Neurology, University of Hyderabad, Hyderabad, Telangana, India

Intoduction: Endoplasmic reticulum (ER) stress is characterized by accumulation of unfolded and misfolded ER proteins. ER stress is an intricate mechanism that interferes with numerous brain damage responses in epilepsy. Aim: The present study aimed to evaluate the effects of Endoplasmic reticulum stress during accumulation of unfolded/misfolded proteins (UFP) on the long term outcome after epilepsy surgery. Materials and Methods: Autopsy Control brain and resected epilepsy surgery tissue samples with a pathological diagnosis of focal cortical dysplasia (FCD) were collected from HBTR-NIMHANS and KIMS, Secunderabad respectively. Tissues were fixed in paraffin blocks and thin sections were made for immunoassays and H&E studies, remaining tissue were used to prepare lysates using RIPA with Protease and phosphatase inhibitors for western blot (15) and biochemical assays, UFP levels were studied by Thioflavin-T assay (n = 28), Antioxidant levels (n = 30) through H2O2 Neutralization assay, total reactive oxygen species (ROS) levels (n = 28) by 2', 7'-Di Chloro Fluorescin Di Acetate (DCFDA) assay. Western blot studies were used to understand the expression pattern of ER transmembrane protein (IRE1α) and Chaperon (BiP) and its downstream regulators (XBP1, CHOP, NOX) during chronic ER stress due to UPR in the resected epilepsy surgery samples with a diagnosis FCD which showed distinct expression pattern compared to controls. Paraffin sections were used to carry out Immuno fluorescence to find the XBP1 localization in neuronal cells which is critical transcription factor to overcome ER stress. Postsurgical outcome of patients carried out with reference to Biochemical data obtained through Thioflavin-T, H2O2 Neutralization assay and DCFDA methods by comparing the seizures frequency. Results: At 2 years post-surgery follow-up significantly more number of patients with type II FCD were seizure free when compared to type 1 FCD (p<0.05). Patients with increased AO levels and decreased ROS & UFP levels in epilepsy surgery specimens with FCD were associate with good post-surgical outcome (ILAE class -I & II). Significant level of Processed XBP1 expression was seen in FCD type IIB type of histopathology (Figure). Phosphorylation of IRE1 was more in FCD type 1A histopathology. GRP-78 expression more in FCD type 1A when compared to control samples. Conclusion: These results suggest that AO levels and ER unfolded protein response are the important forecasters for the prediction of long term post-surgical seizure free (ILAE-I & II) outcome in epilepsy patients with type I and type II FCD. Further Scope: Studying the rest of ER stress markers ATF6, PERK responsible for survival and shutting down mechanisms ameliorating the necessity of medication and surgery.

PP87/457: Optic neuritis following anti-rabies vaccine

Ayush Agarwal, Garg D, Goyal V, Pandit AK, Srivastava AK, Srivastava MVP

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

Introduction: Anti-rabies vaccine related neurological complications are uncommon. The involvement of the optic nerve is extremely rare after anti-rabies vaccination. It has occasionally been reported after Semple's vaccine administration due to the presence of antigenic sheep brain tissue in it. This is the first case report of optic neuritis after chick embryo-derived anti-rabies vaccination. Case Report: A 15-year old male presented with vision loss in both eyes since last seven days. He had an unprovoked dog bite to his right foot three weeks back and had received four doses of chick embryo-derived anti-rabies vaccine, given intramuscularly, every alternate day. Three days following the last dose of the vaccine, he developed sequential painless loss of vision involving the right eye, followed after one day, by the left eye. This progressed to absence of perception of light over the next 2-3 days. He had concomitant complaints of poor colour vision and contrast perception. There was no history of eye redness, lacrimation or photophobia. Fundus revealed bilateral disc edema. The remainder of his neurological examination was unrevealing. Magnetic Resonance Imaging (MRI) of the brain revealed bilateral tortuous optic nerves, which were hyperintense on T2/FLAIR sequences (figure 1a) and showed enhancement on contrast administration (figure 1b). Cerebrospinal fluid (CSF) examination and all routine biochemistry was normal. RT-PCR done for rabies virus from the CSF sample was also negative. He was treated with intravenous methylprednisolone (1gm/ day, 5 days). During IVMP his vision improved to 6/60 over next 5 days and at last follow up at 2 weeks, his vision further improved to 6/6 with complete resolution of bilateral disc edema. He was not given oral steroids. Discussion: Neurological complications are rare after anti-rabies vaccination. The main ones reported are encephalitis and myelitis, alone or in combination (ADEM). Reported cranial neuropathy are: facial, oculomotor, vagus and glossopharyngeal nerves. Optic nerve involvement is extremely rare and generally forms a part of a ADEM. Only a few cases of isolated optic neuritis having been reported after Semple's vaccine.[1] The antigenicity of the Semple's vaccine is thought to be responsible for these which causes an immune complex-mediated reaction, leading to inflammation and demyelination. Spontaneous recovery usually occurs. However, incomplete visual recovery may result because of permanent macular damage. Our case received anti-rabies vaccine derived from chicken embryo cells, which is usually not associated with neuroparalytic complications, though one case each, of neuro-retinitis and Guillain Barre syndrome[12] have been reported. The manufacturer's information also does not mention the risk of neurological complications with the use of chick embryo vaccines. He received his vaccine doses on alternate days. Accelerated dosing regimen as given in this patient could have contributed to enhanced vaccine antigenicity, leading to an immune-mediated complication like optic neuritis. It seems prudent that recombinant vaccines be prepared and used as a permanent solution to this problem. Vaccination is advisable despite these potential neurological complications in view of their rarity.

PP88/463: H-sign: A rare sign of an increasingly common disease

Dhawal Narang, Mehta S, Lal V, Pattnaik SN, Takkar A

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Introduction: MOG antibody disease is a newly described entity and presents in a similar fashion to Neuromyelitis optica and multiple sclerosis in the form of myelitis and optic neuritis. However, few clinical and radiological differences are described in the literature. One such sign is a unique sign called as H-sign. It corresponds to a novel T2 hyperintensity confined to gray matter seen on axial T2W images. We describe a patient with positive MOG antibody with this classical sign. Case Summary: A 51 year old woman, with no previous comorbidities presented with complaints of acute onset progressive bilateral lower limb weakness and numbness of seven days duration. It was associated with urinary retention and had to be catheterised. There was a history of acute febrile illness with multiple loose stools 7 days prior to the onset of weakness. There was no history of any upper limb weakness, cranial nerve involvement or cognitive symptoms. On examination, she was conscious and oriented. Her visual acuity was 6/12 in both eyes. Fundus revealed mild blurring of nasal margins bilaterally. Cranial nerves examination was unremarkable. Motor examination showed spastic weakness of bilateral lower limbs with exaggerated deep tendon reflexes. Sensory examination revealed loss of sensations below trunk with a sensory level at D6. Rest of neurological and other systems examination was normal. Imaging of the spinal cord showed patchy multisegment T2 hyper intense signal changes throughout the cord most marked in the cervical region extending from C4-T1. On axial images the hyper intensity was involving the centre of the cord and mainly restricted to the grey matter distribution without significant gadolinium uptake giving the appearance of H-sign (Figure). Cerebrospinal fluid was acellular with increased proteins. Visual evoked potential showed prolonged distal latency in right optic nerve (P100- 122 msec). Her aquaporin antibody was negative. However, MOG antibody came out to be positive.She was given 5 cycles of plasma exchange after which she reported marked improvement in her symptoms and became ambulant on discharge. Discussion: We demonstrate a unique sign found in MOG-Ab related myelitis. This sign if elicited in a spinal MRI can be helpful to differentiate MOG-Ab related myelitis from NMOSD and MS. In a recent series by Dubey et al, 15/51 MOG-Ab positive patients were found to have H sign compared to only 3/39 patients with NMO and none of the patients with MS (p=0.007). Only 8 patients had gadolinium enhancement which was patchy and faint. Conclusion: We emphasise the importance of this newly described unique sign in MOG myelitis.

PP89/464: Multifaceted presentation of mitochondrial disease – Story of 3 siblings

Muralikrishna Konda, Suresh Babu P, Mohan Das S

Department of Neurology, KIMS Hospital, Thiruvananthapuram, Kerala, India

Aim: To present a rare mitochondrial cytopathy in 3 siblings with variable presentation. Materials and Methods: Three siblings with variable age and symptoms presentation. Results: 27 year old gentleman with normal birth and development presented with drowsiness and ataxia following a febrile illness. Followed by patient developed psychiatric manifestations including suicidal tendencies. MRI Brain suggestive of bilateral symmetrical basal ganglionic T2 hyperintensities. Family history suggestive of almost similar complaints in both the elder sisters at an early age. Thirty four year old first sibling with normal birth and delayed development in all domains suddenly worsened since last 2 years developed gait ataxia with psychiatric manifestations, urinary and fecal incontinence. Now she is dependent in ADL. On examination cerebellar ataxia and absent DTR were present. MRI Brain suggestive of diffuse symmetrical cerebellar atrophy with T2 hyperintensities in bilateral cerebral pedencles of the midbrain. Thirty year old second sibling with normal and intial development had sudden onset recurrent falls and ataxia at the age of 3 years followed by rapid worsening which led to bed ridden status within few years with severe truncal hypotonia. MRI Brain suggestive of bilateral symmetrical T2 hyperintensities in putamen and caudates. All the 3 siblings routine laboratory investigations were normal. Whole genome exome sequencing of the third sibling revealed autosomal dominantly inherited heterozygous mutation in ATAD3A gene on chromosome 1 causing a rare distinct syndrome Harel-yoon Syndrome. The same gene mutation was targated in family members which was present in father and elder sister. Due to variable penetration of autosomal dominantly inherited disorder variable age and symptoms presentation had seen and inspite of harbouring the mutation father didnot develop any symptoms till now. ATPase family AAA-domain containing protein 3A (ATAD3A) is a nuclear-encoded mitochondrial membrane protein implicated in mitochondrial dynamics, nucleoid organisation, protein translation, cell growth and cholesterol metabolism. Harel-Yoon syndrome is a syndromic neurodevelopmental disorder characterized by delayed psychomotor development, intellectual disability, truncal hypotonia, spasticity, and peripheral neuropathy. Other more variable features such as optic atrophy may also occur. Laboratory studies in some patients show evidence of mitochondrial dysfunction. Conclusions: Any patient presenting with symmetrical basal gangionic lesions with positive family history requires extensive evaluation. Detailed family history and extensive evaluation are required for extact diagnosis.

PP90/475: Cerebral venous sinus thrombosis in children: A study from a tertiary care hospital of eastern India

Priyabrata Nayak, Mallick AK, Mohanty G, Nayak SD, Mishra S

Department of Neurology, Srirama Chandra Bhanja Medical College and Hospital, Cuttack, Odisha, India

Background: Cerebral venous sinus (sino-venous) thrombosis (CSVT) in childhood is a rare, but under recognized, disorder, typically of multifactorial aetiology, with neurologic squeal apparent in up to 40% of survivors and mortality approaching 10%. The objectives of this study were to enlist the patients diagnosed as CVST below 14 years of age and to diagnose the aetiology along with radiological correlation. Materials and Methods: Prospective clinical study for 2 years in the department of neurology and department of paediatrics of SCB Medical College, Cuttack. All the patients were enlisted in a pre-structured format with detailed clinico-radiological evaluation. Treatment was done according to recent guidelines. Outcome after 3 month was analysed. SPSS software version 20.0 was used for statistical analysis. Results: Total numbers of patients included in the study was 15. Out of them 3 patients were neonates. Most common association was TB meningitis. Phototherapy after neonatal hyperbilirubinemia was prominent cause in neonatal age group. Multiple sinus involvement was seen in the most of the patients. Transverse sinus was the most common sinus to be involved. Conclusion: Cerebral sinovenous thrombosis is an underdiagnosed but important cause of stroke in childhood occurring most often in the neonatal period. Mortality and morbidity are significant. Infective and hypercoagulation are the two primary association. MR Venogram is the investigation of choice. Early diagnosis with management along with plan for secondary prevention can save from catastrophic consequences.

PP91/478: Peek through the smoke: Case series of moya moya disease

Nardla Maheedhar Reddy, Venkatasubramanian S, Sundar S, Hazeena P

Department of Neurology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Introduction: The term moyamoya means “Hazy, cloudy puff of smoke appearance” and is defined as Nonatherosclerotic, noninflammatory, and nonamyloid vasculopathy characterized by chronic progressive stenosis or occlusion of the terminal internal carotid arteries (ICA) and/or the proximal portion of the anterior cerebral arteries (ACA) and/or middle cerebral arteries (MCA). It remains largely an angiographic diagnosis. We are presenting 5 cases of Moya Moya in view of diverse clinical, radiological presentation and varied methods of management. Materials and Methods and Results: An Observational study of moya moya patients admitted and treated in SRI RAMACHANDRA MEDICAL COLLEGE in one year period. 5 patients were diagnosed with Moya Moya of which 2 are females and 3 were males. Both the females are less than 20 years age group and and all men are aged more than 40 years. Three patients presented with focal ischemia, one patient with recurrent TIA and one had seizure with intraventricular bleed. 4 patients had bilateral internal carotid (ICA) stenotic occlusion, unilateral ICA occlusion in one patient and one of the patients also had posterior cerebral artery stenotic occlusion. Only one patient had 3 flow related aneurysms with largest one measuring 4.5 x 2 mm size. Two patients were are planned for STA-MCA bypass and remaining three patients are managed conservatively without any further event in follow up.

PP92/481: Anterior horn cell involvement in Sjogren syndrome – An exceptional presentation of an autoimmune disorder

Deependra Singh, Chakravarty K, Ray S, Lakhanpal V, Lal V

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: The causes of sporadic amyotrophic lateral sclerosis (SALS) and other types of motor neuron disease (MND) remain largely unknown.[1] Sjogrens syndrome may present as peripheral sensory neuropathy or more rarely as mononeuritis multiplex, but MND is rare. We here present two cases who presented to us with clinical features suggestive of Motor Neuron Disease that ultimately proved out to be secondary to Sjogrens Syndrome. Materials and Methods: We describe two interesting cases that were definitely ALS, but later turned out to be due to secondary Sjogren's syndrome. Case 1 - 47 yr old female presented with 5 months history of progressive bulbar symptoms and proximal and distal pure motor weakness of all limbs with tongue fasciculations along with increased reflexes B/T/K – (3+) and S/ A ( 2+). On evaluation, electrophysiologically – definite ALS, Anti Ro (+), ANA 2(+), speckled, lip biopsy S/o Sjogren's syndrome. His PET scan, salivary scintigraphy – normal. Her other work-up for motor neuron disease was negative. CASE 2 - 50 years old female presented with 3 months history of progressive bulbar symptoms and weakness along with wasting. Was diagnosed with Sjogren's syndrome 5 months back. Electrophysiology was highly suggestive of anterior horn cell involvement. She had high titers of ANA(2+) and Ro(2+) positivity. Steroids brought temporary improvement in her symptoms. Discussion: Sjogren's syndrome is a multisystem autoimmune disorder with neurological involvement in up to 20% of patients.[2] In approx 25-92% of this group, these symptoms may precede dry eyes and mouth.[3] MND is a rare presentation of Sjogren's syndrome, mentioned in only a very few case reports. UMN signs are more predominant than LMN signs and occur early in Sjogren's syndrome patients with MND. There are two possibly two mechanisms underlie the upper motor neuron involvement in patients with Sjogren's syndrome.[4] One possible mechanism could be attributed to the autoimmune origin of UMN involvement as in such patients the presence of various anti-neuronal antibodies is reported in the literary works. Subclinical Sjogrens syndrome plays an additive role in leading to another possibility for the progression of upper motor neuron dysfunction. Conclusions: Neurological symptoms occur commonly in Sjogrens syndrome, although anterior horn cell involvement is exceptional. Immunosuppressant's should be tried in such cases because inflammation is the main reason for the anterior horn cell involvement.

PP93/494: Interesting etiological spectrum of vasculitic neuropathy

Neelam Patil, Chakor R, Bharote H, Endole D

Department of Neurology, Krishna Institute of Medical Sciences, Karad, Maharashtra, India

Vasculitis can affect the peripheral nervous system alone (nonsystemic vasculitic neuropathy) or can be a part of primary or secondary systemic vasculitis. In cases of pre-existing systemic vasculitis, the diagnosis can easily be made, whereas suspected vasculitic neuropathy as initial or only manifestation of vasculitis requires careful clinical, neurophysiological, laboratory and histopathological workout. The typical clinical syndrome is mononeuritis multiplex or asymmetric neuropathy, but distal-symmetric neuropathy can frequently be seen. Standard treatments include steroids, azathioprine, methotrexate and cyclophosphamide. We had three female patient presented with different clinical presentation and etiologies. Patient 1, 33yrs, F presented with asymmetrical onset sensorimotor weakness with joint pain and tremors in hands progressed over 3 months. Nerve biopsy s/o vasculitic neuropathy, ANA (1:1280 titres, cytoplasmic homogenous pattern) and Anti-MPO positive. improved with cyclophosphamide pulse therapy and steroids. Patient 2, 38 yrs, F presented with asymmetrical onset distal burning sensation in all 4 limbs since 6 months. Nerve conduction study s/o asymmetrical axonal sensorimotor neuropathy, autoimmune work up was negative. Nerve biposy s/o vasculitic neuropathy. Treated as c/o nonsystemic vasculitic neuropathy. She is undergoing pulse therapy with cyclophosphamide with steroids. Patient 3, 47 yrs, F presented with h/o asymmetric onset sensory loss in all 4 limbs since 4 yrs followed by distal weakness since 3 yrs. NCS s/o generalised axonal sensory neuropathy. Her c-ANCA came positive. She was started on steroids f/b tab azathioprine and improved significantly. Conclusion: About 30–50% of all vasculitis patients exhibit signs of peripheral neuropathy. Neuropathies associated with systemic vasculitis should be treated according to the guidelines of the underlying disease. Non-systemic vasculitic neuropathy will be treated with steroids, or in severe/progressive cases, Cyclophosphamide pulse therapy. Some patients need long-term immunosuppression.

PP94/496: Association of CYP2C19 and UGT1A6 genetic polymorphisms with valproic acid induced weight gain in persons living with epilepsy from South India

Balachandran Mani, Nair P, Kesavan R

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

Introduction and Background: The association between genetic factors and valproic acid induced weight gain is not studied in the South Indian population. Previous study had shown that most of the current Indian population has its roots in two genetically divergent and distinct ancient populations viz. Ancestral North Indians and Ancestral south Indians. Further the role of UGT 1A6 polymorphism (552A>C) in VPA induced weight gain has not addressed so far. Hence, we propose to study the effect of CYP2C19 polymorphism and UGT1A6 polymorphism in South Indian population with epilepsy. Materials and Methods: We included People with epilepsy (PWE) within 1 month after initiating VPA monotherapy. The cases (those with CYP2C19 polymorphism) and controls (those without CYP2C19 polymorphisms) were in 1:2 ratio. They were followed up monthly for assessing the weight gain or any other adverse effects for 6 months. We measured height, weight, BMI, food frequency, control of seizures and drug compliance and physical activity. Sample size was calculated for 95% CI, 80% power, and % outcome in controls 33% and relative risk of risk of 1.7 with ratio of cases to control of 1:2 then we had to include 50 cases and 99 controls. Target weight gain was defined as the increase of BMI > 1 from the baseline. We divided the participants into those with and without weight gain. Genetic analysis for identifying CYP2C19 and UGT1A6 polymorphisms were done. During the interim analysis of this prospective genetic association study allele and genotype frequencies were compared between the patient sub groups with and without VPA toxicity using the chi-square test. The association of weight gain with UGT1A6 and CYP2C19 genotypes was carried out by using Chi square test. Results: We screened 48 PWE till now, and 27 PWE on valproate monotherapy were included. Out of 27 PWE, 23 had CYP2C19*2 (681G>A) genotype polymorphism and 4 did not have this polymorphism. 17 had weight gain. The mean age was 32.39 years. Majority were male (n= 21, 77.7%). The demographic parameters like age, gender, and initial BMI were not significantly different between the groups. The generalized seizure was the major seizure type among the participants. The relative risk of CYP2C19*2(681G>A) genetic polymorphism on valproate-induced weight gain is 0.13 (95 % CI: 0.39 – 3.2, p=0.8) and UGT1A6 (552A>C) genetic polymorphism is 0.711 (95 % CI: 0.352-1.435, p=0.34). The study did not show any significant association of CYP2C19*2 (681G>A) and UGT1A6 (552A>C) polymorphism with VPA induced weight gain in south Indian population having epilepsy (p-value 0.8 and 0.7 respectively). Conclusions: There is no clinically significant association between CYP2C19*2 (681G >A), UGT1A6 (552A>C) genotypes and VPA induced weight gain in PWE from South India. Limitations: The target sample size is not achieved. Further Scope: The availability of genetic results will help in personalized medications and thereby reduce the occurrence of side effects.

PP95/503: Characteristic of epilepsy in an improvised cohort: Population based study

Paul BS, Singh G, Singhal S, Bansal RK, Sharma S

Department of Neurology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India

There are about 50 million diagnosed cases of epilepsy worldwide, of which 40 million live in low- and middle-income countries (LMICs). We undertook a population-based survey to identify people with epilepsy in a resource-limited, impoverished community in India. We also surveyed drug resistant epilepsy, existing medical and/or psychiatric co morbidities in this population. Methods: Community Interventions For Epilepsy is population-based initiative to provide care for people with epilepsy through primary and community health workers. A cluster-randomized controlled trial with support from ICMR is underway. A population-based survey was conducted in two-step: (1) door to door screening for epilepsy (n=55,953). People screened positive in first step (n=556) underwent evaluation by neurologist with EEG and MRI (234;125). People screened negative for epilepsy (NES, single seizures, syncope) were excluded (n=96). People who did not show up for step 2 of evaluation were also excluded (n=67). Community was divided into 9 zones, (2-3 clusters per zone) and total eligible patients (n = 254) from 24 clusters were enrolled. These subjects were followed up for 12-19 months. We undertook to describe socio-demographic features of this cohort, to characterize seizure types and epilepsy syndromes (2017 ILAE classification). We also used current ILAE criteria (2010) to label among this cohort, those with DRE. Finally, we used a previously used inventory to associate various medical and psychiatric comorbidities with each of cases (2009). Those with febrile seizures, neonatal seizures, single seizures and acute symptomatic seizures were excluded. Results: A total of 254 people with epilepsy out of 55,953 surveyed were included. Mean age 25.78 (+/- 15.21) years with 170 (67%) males. Duration of epilepsy varied between 16 and 742 months (median:132 months). The seizure types, syndromes and etiological conditions will be described. Epilepsy was labelled as controlled in 97 patients (38.18%) and fulfilled criteria for drug-resistance in 79 (31.10%). Drug-resistance/control could not be determined in 78 (30.70%). Psychiatric Co Morbities were: developmental delay of varying degrees in 20 (7.87%), depression in 13 (5.11%), alcohol abuse in 7 (2.75%), other drug abuse in (7; 2.75%); tobacco use 4 (1.5%) and 4(1.5%) people had suicidal ideations. Amongst other psychiatric conditions found in this population were dementia, ADHD, behavioral disorders, interictal psychosis, and sleep disturbances. Neurological disorders were also common: perinatal insult (n=25; 9.84%), neurocysticercosis (n=24; 9.44%), traumatic brain injury (n=19; 7.4%), hemiplegia (n=6,2.36%) and migraine (n=2,0.78%). Others seen in this population –dermatological conditions 8 (3.54%), GIT infections 7 (2.75%), tuberculosis (n=5,1.96%), menstrual problems 4 (1.57%), dengue 3 (1.8%), and chest infections (0.78%) amongst others. Six people in cohort experienced status epilepticus during the follow-up period. Conclusions: A large proportion of people in this cohort had DRE. The large numbers with various psychiatric, neurological and medical comorbidities mean that these conditions must be included in any programs directed towards control of epilepsy in LMICs.

Friday, October 04, 2019, 07:30AM-13:30PM,

Poster Hall Area,

Poster Session 02: Stroke

SP1/19: Profile of dysphagia in acute stroke: A prospective pilot study in a Territory Care Hospital

Desai M, Surya N

Department of Neurology, Bombay Hospital and Medical Research Center, Mumbai, Maharashtra, India

Purpose: Post stroke dysphagia is one of the common under-estimated complication of acute stroke. Although multiple advances have been made in hyper-acute treatment of stroke and secondary prevention, the management of dysphagia post-stroke remains neglected area of research. The current study is designed to understand profile of dysphagia in acute stroke patients. Methods: In this prospective study, consecutive acute stroke patients admitted to a tertiary care hospital were screened using Nair Hospital Bed side swallowing assessment and Nair Hospital swallowing ability scale at admission and discharge along with it severity of stroke based on National Institutes of Health stroke scale was assessed. Based on this assessment patients were categorised from scale of 0 to 7 (normal swallow to complete dysphagia). We investigated the relation between dysphagia and severity of stroke. Results: A pilot study of 22 patients was done and 6 (27.27 %) out of 22 patients were diagnosed with dysphagia on admission. 3 (13.6 %) out of the above had persistent dysphagia at time of discharge. Average severity of stroke on admission based on NIHSS was 6.75 (2-18) and on discharge it was 3.87(1-12). There was strong positive correlation between stroke severity and dysphagia. Conclusion: The prevalence of dysphagia based on this pilot study is 27.7 % in acute stroke patients. Majority cases of dyspahgia in acute stroke were transient in nature. Dysphagia has positive correlation with severity of stroke and those with mild to moderate dysphagia improved significantly by the time of discharge.

SP2/20: Clinical profile of stroke patients admitted to a Territory Health Care Center in Mumbai, Maharashtra: A prospective cross-sectional study

Agrawal K, Someshwar H, Surya N

Department of Neurology, Bombay Hospital and Medical Research Center, Mumbai, Maharashtra, India

Introduction: Cerebrovascular stroke is one of the leading causes of morbidity and mortality in adult life, third to cancer and coronary heart disease. Objective: To study the clinical presentation, pattern of brain strokes, areas of brain affected as per radiological findings in patients admitted to a territory health care center. Methods: This is a prospective study, patients admitted to the stroke care unit of a territory care unit were included in the study. Demographic data, neurological assessment and routine blood and radiological investigations were advised to the patient. Results: The cerebrovascular stroke was more common in males (76.15%) than females (23.85%). The most common age group was 61-70(43.9%) years of age. 54.26% patient's required Intensive care stay in the hospital. Most common clinical feature was hemiplegia (78.3%). Most common risk factor was Hypertension (34.1%), Diabetic (27.3%), smoking (14%), Dyslipidemia (11%). 42% patients had more than one risk factor associated, hypertension and smoking (62%) being the most common combination. Most common type of stroke was the ischemic type (84.6%). In ischemic stroke, MCA territory (76.1%) was involved to maximum followed by ACA territory (11.3%). In ischemic stroke the parietal lobe (43.9%) followed by the frontal lobe (23.7%). In hemorrhagic stroke, ventricular area (33.8%) was more involved followed by thalamic area (17.1%). NIHSS score on admission was 8.37. Conclusion: Cerebrovascular stroke cases were having male pre dominance with hypertension was the most common risk factor and most common type of stroke was ischemic type in the parietal area.

SP3/24: Alterations in polysomnographic profile in ischaemic stroke patients

Ravi SV, Packiaseli CR, Saravanan S

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

Introduction: Sleep disturbances in stroke patients have been described in literature. Polysomonographic evaluation of sleep in stroke patients are far and few. Objective: To study the changes in Polysomnographic profile in ischaemic stroke patients. Materials and Methods: This prospective study included 30 ischaemic stroke patients admitted in the neurology ward of TVMCH, Tirunelveli. Inclusion Criteria: (1) age more than 18 years (2) completed ischemic stroke confirmed by CT or MRI; (3) Informed consent from patient. Exclusion Criteria: (1) Severe stroke with NIHSS >24 (2) patient on any type of ventillatory support (3) previously diagnosed OSA on therapy (4) co-existing CNS diseases such as dementia, psychosis, parkinsonism, epilepsy (5) Those with hemorrhagic strokes or cortical venous thrombosis. Then patients were subjected to overnight PSG during the period of their hospital stay and the results are interpreted. Results: PSG analysis revealed that poor sleep efficiency of <85% was present in 84.1% of patients (mean: 63.8 ± 18.1%). The latencies to sleep onset (mean: 43.4 ± 61.0 minutes) and stage 2 sleep (35.6 ± 12.9%) were prolonged while slow wave sleep was shortened. Respiration during sleep was significantly impaired in which 39.4% had impaired apnoea hyperpnoea index (AHI) ≥5, mean AHI: 8.1 ± 10.9). These patients had periodic leg movement (PLM) disorder (54.8% had PLM index of 5 or more, mean PLMI: 25.78 ± 48.12). Conclusions: Sleep macro-architecture is deranged frequently and also variably in ischaemic stroke patients.

SP4/27: Asymptomatic remote bilateral basal ganglia hemorrhage as a complication of intravenous thrombolysis for acute stroke: A rare complication

Gupta A, Kushwaha S, Sachan M, Gupta C

Department of Neurology, Shree Aggarsain International Hospital, Rohini, Delhi, India

Introduction: Hemorrhage after intravenous thrombolysis for acute ischemic stroke is an uncommon event. This may be intraischemic or extraischemic. Extraischemic, in contrast to intraischemic, is relatively rare. Presentation of asymptomatic extraischemic bilateral basal ganglia hemorrhage is rarely reported in literature. Case Report: A 55 years old previously hypertensive male presented within 2 hours with acute onset of giddiness and imbalance bilaterally, more to left side. No history of DM, TIA, smoking, alcohol. On examination, he was conscious and oriented (GCS of 15). Speech was dysarthric. There was no motor or sensory deficit and NIHSS was 4. His blood glucose was 145mg/dl and BP was 210/120 mmHg. The patient had no contraindications to thrombolysis except his raised BP. The patient was thrombolysed immediately, after lowering his BP. His symptoms improved partially. His NIHSS score was 2. After 24 hours of thrombolysis, a routine NCCT head of Brain was done which had acute bilateral cerebellar infarct and bilateral basal ganglia hemorrhage. Since patient was asymptomatic to this event, he was monitored in ICU.MRI brain showed acute infarct involving left cerebellar hemisphere with acute infarcts also involving left high parietal, left occipital and right middle cerebellar peduncle and acute hematoma in bilateral basal ganglia. On serial evaluation, his bleed was reabsorbed gradually. Discussion: Large data and management guidelines are present for symptomatic ICH but not for asymptomatic ICH. Risk factors for extraischemic hemorrhage are female sex, age and history of previous stroke (>3 months). The management and follow up of such patients is still an area of research.

SP5/38: Clinical profile and predictors of outcome in CSVT patients with intracerebral haemorrhage

Kalyani K, Veena N, Satish K

Department of Neurology, Gandhi Medical College, Hyderabad, Telangana, India

Background: Cerebral venous thrombosis (CVT) is responsible for 1-2% of all strokes in adults and affects all age groups. Although ICH is common in patients with CVT and leads to worse outcome there is paucity of studies regarding predictors especially in Indian scenario. Objective: To study varied clinical presentations and outcome among patients of CSVT with bleed also study the location of bleeds, sinus thrombosed and their outcome. Methods: prospective observational study done in pts admitted with CSVT with ICH in medicine and neurology dept after obtaining appropriate consent. Results: Total of 67 patients were included with males around 55%(37) and females 44.7%(30) with acute presentation seen in around 37% and subacute in 63% and majority of pts had headache (91%),vomiting (76%) seizures (45%) also aphasia (64%) papilloedema, diplopia seen in around 36%,around 43% pts were either drowsy or comatose at presentation. Common risk factors were anemia in 22.3%, hyperhomocysteinemia in 18%, alcoholism in 26.8%, oral contraceptive pill intake in 9%, postpartum state in 12%. In pts with CVT with ICH have advanced age, headache (91%), consciousness disturbances (43%), seizures (45%), and language deficits (64%) were significantly higher when compared to other studies done in CVT including non hemorrhagic CVT. Patients with hemorrhagic lesion were more dependent or died (32%). Conclusion: Headache, convulsion, behavioral disorder, seizures, and speech disorders were the most frequent clinical symptoms of patients with hemorrhagic CSVT. Specific risk factors, including puerperium, extended thrombosis of large sinus, multiple sinuses are associated with unfavorable outcome.

SP6/40: Non valvular atrial fibrillation in patients with recurrent stroke and multiinfarct state among patients admitted in a Tertiary Health Care

Remanan R, Shaji CV, Kabeer KA

Department of Neurology, Government Thirumala Devaswom Medical College, Alappuzha, Kerala, India

Introduction and Background: Atrrial fibrillation accounts for a large proportion of cases with recurrent stroke, a significant proportion of the af being paroxysmal may be missed on clinical examination and routine ECG examination. Detection of af in these patients will warrant them to be on anticoagulation, thus significantly altering the chances of further recurrences. Aim: 1. to find out the prevalence of atrial fibrillation in patients with recurrent ischemic stroke and multiinfarct states among patients admitted in the neurology wards in Government T.D Medical College, Alappuzha during one year period from 1/4/2018- 31/3/2019 2. To find out the prevalence of other rhythm abnormalities in the study population. 3. To find out the proportion of patients with paroxysmal af that are missed on convential ECG monitoring and clinical examination. Materials and Methods: Study Design: Hospital based cross sectional study. Study Tools: A proforma, ECG recorded using 12 lead ECG monitor, 2d echocardiography,72 hours of continuous ambulatory holter monitoring done with an ambulatory automatic holter monitor. Study Period: 1 year. Sample Size: All patients admitted during the study period were included in the study as the predetermined sample size of 286 could not be obtained. Inclusion Criteria: 1 .all patients admitted with recurrent ischemic stroke. 2. All patients with multiinfarct state on ct brain. Exclusion Criteria: 1. Unwillingness to give the consent. 2. Hemorrhagic stroke. 3. valvular heart disease on echocardiogram. Results: total 74 patients presented during the study perion with either a recurrent ischemic stroke or a multiinfarct state, out of which 8 were excluded as they had a valvular heart lesion in the echocardiography. 34 of the patients were males (53.1%) and 30 of them were females (46.8) 45 of them had a previous stroke with no multiinfarct state on ct brain (70.3%), 5 had previous stroke and a multiinfarct state on ct brain (7.8%), 14 had multiinfarct state on ct brain with no previous history of stroke (21.87%). 13 out of 64 (20.3%) had atrilal fibrillation detected by holter monitoring, out of which 9 (14%) had paroxysmal af that was not detected on clinical examination and ECG recording taken at the time of admission. Atrial fluttrer was seen in 8 of 64 patients (12.5%), nonsustained ventricular tachycardia in 28 of 64 patients (43.75%), ventricular ectopics in i2 of 64 patients (18.75%), heart block in 5 of 64 of patients (7%). Conclusion: This study shows that a significant proportion of patients with recurrent stroke are having atrial fibrillation with a high proportion being paroxysmal af. Further Scopes: Significant impact on management of patients with recurrent stroke and also in preventing further stroke episodes.

SP7/51: A study on memory and language functions in right hemisphere stroke

Saravanan S, Kiruthiga K

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

Among 60 patients presenting with left hemiplegia due to stroke were studied at the Department of Neurology at Tirunelveli Medical College Hospital, Tirunelveli, from the period August 2017 to September 2017. The study group consisted of 91.66% above 40 years, 66.5% males and 33.5% females. White matter infarct (48.33%) & parietal cortical infarct (30%) constituted majority of the MRI findings Hypertension (58.33%), smoking (41.66%), Alcoholism (38.33%) and diabetes (35%) were the commonest risk factors. 76.66% showed some form of right hemisphere dysfunction which was more in females (85%) than males (72.5%) and more in age group above 40 years ( 81.81%). The commonest abnormalities detected were in inference test (56.66%), written metaphor test (50%), picture metaphor test (45%) and visual memory test (60%). Emphatic accent test was unaffected (0%). Severe test abnormalities were found in 21.73%. The lesions more frequently associated with right hemisphere memory and language dysfunction were periventricular ischemic change (100%), capsular infarct (89.66%) and parietal cortical infarct (72.22%). Parietal cortical infarct (38.46%) and diffuse periventricular ischemic changes (33.33%) were associated with severe right hemisphere involvement (>5 test abnormality) when compared to infarcts in white matter areas (11.54%). The commonest abnormalities detected were in inference test, metaphor test and visual memory test. The most commonly affected tests for language dysfunction are Inference test, written metaphor test and picture metaphor test. The most commonly affected test for memory dysfunction is the visual memory test. So these tests can be considered as representative tests for right hemisphere language & memory dysfunction.

SP8/78: Normotensive state during acute phase of hypertensive intracerebral haemorrhage

Mulmuley M, Pradhan S, Anand S

Department of Neurology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Background: Hypertensive haemorrhage is a leading cause of intra-cerebral bleed (ICH) although some of these patients may not present with high blood pressure at the time of ICH. Methods: This retrospective study included patients with history of hypertension presenting with ICH. Patients with systolic blood pressure recording of more than 140 mmHg were included in hypertension group (group I). Patients whose blood pressure rose to hypertension range after fluid correction were included in group II and patients with blood pressure less than 140 mmHg on consecutive one-week blood pressure recordings were included in group III. Clinical features including volume of ICH of all the three groups were noted. Outcome in the form of mortality was analysed. Chi square test was used for categorical variables and independent t test for continuous variables. Results: Ninety-two ICH patients with history of hypertension were included. Of them, 20 patients (22%) presented with blood pressure less than 140 mmHg systolic at the time of ICH. After fluid correction it rose to hypertensive range in nine (10%) but remained in normal in eleven patients (12%) during consecutive recordings for one-week post admission. On comparing normotensive and hypertensive groups, significant difference was seen in survival and volume of ICH. Conclusion: There is a subset of hypertensive patients who may present with normal blood pressure recording during acute ICH. The blood pressure rises subsequently with the correction of hypovolemia in some. The volume of haemorrhage in normotensives is relatively small but whether this translate into better prognosis needs further studies.

SP9/83: Optimisation of neuroimaging in patients of acute stroke under arogyasree scheme of Telangana state

Chaudhary N, Laxmiprasad N

Departments of Neurology and Neuropathology, National Institute of Mental health and Neurosciences, Bengaluru, Karnataka, India

Introduction: Acute stroke is neurological emergency which has been approved by the Telangana government under aarogyasri scheme. This gives fixed Rs 20,000/- per patient for stroke management (10 days hospital stay / food / medications). The neuroimaging is must for diagnosis and getting approval for cashless treatment from aarogyasri authority. The major cost in management of stroke lies with CT/MRI scan of brain (Rs 3000/-, Rs 6000/-). Aims: To study the effectiveness of CT scan in diagnosis of acute stroke in lessor cost. Material and Methods: Life line hospitals Warangal is superspeciality hospital with authorisation from aarogyasri trust of Telangana to treat the acute brain stroke. 209 patients of acute stroke were admitted from December 2017 to May 2019 in this hospital. Retrospective study of the neuroimaging pattern was analysed. Results: 209 patients underwent CT Scan of brain within 24 hours of paralysis attack. 46 patients showed haemorrhagic pattern. 145 patients showed infarct. 18 patients with neurological deficit and normal CT Scan of brain, underwent MRI Scan which showed brainstem infarct and infarct in other area in some cases who underwent CT Scan within 12 hours of paralysis. Conclusions: CT Scan of brain is cheep neuroimaging option in contexts limited fund. It gives diagnosis in around 90% of the stroke patients (100% in haemorrhagic stroke). Only 11 % of the ischemic stroke patients needed MRI Scan for diagnosis. The limitation of study is twofold. None of the patient was given the option of interventional angioplasty or of thrombolytic therapy in ischemic stroke due to less fund in aarogyasri package.

SP10/86: A study of predictors, prognosis and outcome in recurrent stroke in hospitalised patients in a Tertiary Care Hospital in Eastern India, Bin, Kolkata

Sahu D, Ray BK

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

Introduction: Cerebrovascular-disease is one of the leading causes of mortality & morbidity in adults worldwide. The incidence of stroke-recurrence is significantly related to increased mortality and morbidity. Although risk factors are well-defined, the extent of control for modifiable risk factors by secondary treatment and success rate of treatment for the prevention of recurrent stroke have not been sufficiently investigated in our setup. Methodology: Aims and Objectives: The main purposes of our study were to determine the subtype of recurrent stroke in relation to subtype of index stroke, the prognosis of all stroke recurrences observed in our hospitals and the possible responsible risk factors and outcome was assessed. Definition of Population: Stroke patients who was admitted in Department of neuro-medicine, BIN and RDH (Annex), IPGME&R and SSKM Hospital, Kolkata. Study Type: Observational, cross sectional & prospective comparative hospital based study. Methods of Data Collection: By oral questionnaire method regarding relevant history from patient or patient party, using beside clinical instruments and sending routine investigation to the laboratory and plotting them all in Performa for case records. Definition of Problem: A recurrent stroke was defined as new focal neurological deficit with no apparent cause other than that of vascular origin occurring at any time after the index stroke, or with clinical evidence of sudden exacerbation of a previous focal neurological deficit occurring > 21 days after the index stroke. Results and Analysis: In this ongoing study we found majority of the study population (n=82) belongs to 61- 70 year of age group (28.84%) with male preponderance (65%). Among the ischemic recurrence stroke majority are harboring large artery atherosclerosis followed by lacunar pathology. And clinical syndrome of recent event is of partial anterior circulation in 60 % followed by total anterior circulation. Index stroke is hemorrhagic in 40% whereas ischemic in 60%. Among the hemorrhagic stroke recurrence parenchymal bleed is seen in 80% where subcortical bleed (volume 30-60 ml) is maximum. Index stroke is ischemic (63.64%). In our study predictive risk factors are hypertension (78.07%), diabetes (73.07%), cardiovasculardisease (42.3%), smoking (40.38%), significant alcoholism (28.84%), drug abuse (3.84%). Among the patients with known co morbidities drug nonadherence has been found in around 36%. In patients with cardiovascular disease atrial fibrillation (17.3%) is most important. Patients with lacunar ischemic stroke had milder maximal neurological deficits and better post stroke MRS compared to others. Hemorrhage (25.6%) and cardioembolic stroke (19.2%) had poorer functional status, more severe neurological deficits at the time of stroke, and post stroke depression (36%). Conclusion: Stroke recurrence is a non-negligible cause of disability and mortality. The most frequent pattern of recurrence was large vessel atherothrombosis in the same vascular territory, albeit any other pattern is possible. The differential diagnosis challenge between subtypes of infarct is not completely resolved. Limitation: Small number & discrepancies in completeness of case detection of both initial and recurrent stroke may affect the study, as very mild strokes may be missed. Further Scope: Subsequent long follow-up and well noted registry is required.

SP11/109: Clinical profile and risk factors of 104 patients with ischemic and hemorrhagic stroke and TIA patients in a Tertiary Care Hospital in Rajasthan

Mandal M, Sureka RK

Department of Neurology, Mahatma Gandhi University of Medical Sciences and Technology, Jaipur, Rajasthan, India

Introduction: The crude stroke prevalence in India is higher than developed countries.The traditional risk factors for coronary artery disease have been found to play lesser roles in stroke causation in various studies. Aim of Study: We studied the clinical profile and risk factors of 104 patients of stroke attending our hospital from October 2017 to September 2018. Materials and Methods: 104 patients of stroke admitted in Department of Neurology of Mahatma Gandhi Medical College were taken. Baseline NIHSS score and mRS score at discharge were calculated. Stroke patients were classified into hemorrhagic, ischemic and TIA groups based on clinical features and NCCT Head.Routine investigations like RBS, CBC, Serum creatinine, fasting lipid profile, HbA1c, Serum homocysteine were carried out. Patients suspected to have ischemic stroke were subjected to MRI Brain and CT angiography Brain and neck vessels. Data was analysed by the SPSS software. Results: Of 104 stroke patients, 2/3rd had ischemic stroke. Of 75 ischemic stroke patients, 64% were male, of 24 hemorrhagic strokes, 50% male and 5 TIAs 3 were male and rest were female. Mean age of patients with hemorrhagic stroke was 57.1yrs, in ischemic stroke 60.6yrs and TIAs 63.8yrs. Smokers constituted 61% of ischemic strokes, 33% hemorrhagic and 100%TIAs. HDL levels were lower in ischemic stroke patients and TIAs. High NIHSS in 20% ischemic and 33% hemorrhagic, functional independence in 48% ischemic and 33% hemorrhagic strokes. 78% ischemic strokes were in MCA territory. Conclusion: Ischemic stroke was more common than hemorrhagic stroke. Middle cerebral artery was most commonly involved. Low HDL more common in ischemic stroke.

SP12/110: Stroke: subtypes and associated risk factors in a Tertiary Care Hospital

Sriram Reddy Balankari, Lakshmi SRG

Department of Neurology, Osmania Medical College, Hyderabad, Telangana, India

Introduction and Background: Stroke is one of the leading causes of mortality and morbidity in India. Ischemic stroke is quite heterogeneous in its pathophysiological mechanisms, and this forms the basis of stroke subtyping. The importance of subtyping is highlighted by the fact that current guidelines for stroke prevention and treatment are subtype specific. Recently, genetic mechanisms underlying stroke have also been found to be subtype specific. Exploration of stroke subtype trends is necessary to provide physicians with vital insight for early diagnosis and treatment. The present study has been conducted in Osmania hospital, Hyderabad and is aimed to investigate the pattern of ischemic stroke subtypes and their associated risk factors. Objectives: To study about various subtypes of stroke. To study various risk factors associated with each type of stroke. Material and Methods: Inclusion Criteria: Patients attending to Osmania General Hospital with features acute strokeare included in the study. Exclusion Criteria: Patients with Neurological manifestations explained by other etiology which can mimic stroke are excluded from the study. It is a descriptive study carried out in OGH, Hyderabad (January 2018- June 2019). Patients admitted in Osmania hospital are taken into the study. For all patients, routine investigations, CT and MRI brain, 2D echo, carotid and vertebral Doppler were performed. In selected patients CT or MR brain Angiography, coagulation studies etc were performed. All patients provided informed consent. Results: A total of 401 patients of acute stroke were studied. Among them 319 were arterial strokes and 57 were venous strokes. Among arterial strokes 269 were ischemic and 55 were hemorrhagic strokes. Large artery atherosclerosis (LAA) were 150, small vessel occlusion (SVO) were 69 and cardio embolic strokes were 24. Stroke of other known etiology were 11 and unknown etiology were 35. Among hemorrhagic strokes 43 were parenchymal bleeds and 12 were subarachnoid hemorrhage. Stroke in young accounted for 63 cases in the study. Among cases of known etiology five patients were inherited thrombophilias, three were infective causes, one was a patient of coarctation of aorta, one was a patient of postpartum angiopathy and one was a patient of ischemic stroke post aneurysmal clipping. Among risk factors HTN and smoking were largely associated with large vessel strokes while DM accounted for more small vessel strokes in the study. Other risk factors like age, family history, dyslipidemias accounted evenly for both ischemic and hemorrhagic strokes. Conclusions: Large vessel atherosclerosis was the most common subtype of stroke. Among risk factors, smoking and HTN were associated with more large vessel and hemorrhagic strokes where as DM was predominantly associated with small vessel stroke in the study. Limitations: Due to financial constraints certain investigations like genetic studies were not done. Prognosis of each subtype was not assessed. Further Scope: Genetic studies can be conducted in patients who were unknown etilogy. Population based screening can be done based on demographic specific risk factors.

SP13/115: Demographics, risk factors, co-morbidity and clinical profile of ischemic stroke subtypes according to TOAST criteria at a Tertiary Care Center

Reddy PV, Chandramouleeswaran V, Narasimhan LR, Kannan V

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

Background: Stroke is a leading cause of adult disability, estimated incidence rate in India is 145 and 154 per 1,00,000/ year. The aim of the study was to determine the age-dependent differences in demographics, risk factors, co-morbidity, etiology and clinical profile of acute stroke in a 18 month long hospital?based registry at Rajiv Gandhi Government Hospital & Madras Medical college in Chennai. The cause of ischemic stroke was classified according to the TOAST criteria. Methods: The study was conducted on patients enrolled in the stroke registry of RGGH & MMC between July 2017 to December 2018. Results: The cohort comprised of 2100 patients: 1647 (78.4%) were ischemic and 453(21.5%) were hemorrhagic strokes. The most common ischemic stroke subtype was large artery atherosclerosis (LAA) comprising 42.8% (n = 898), followed by small vessel occlusion comprising 15.1% (n = 317) and cardio embolism 9.7% (n = 204), Stroke of other determined etiologies was 3.6% (n = 75) and stroke of undetermined etiology constituted 28.8%(n=604). The incidence of different risk factors were as follows: 1072 (51%) hypertensives, 637 (30.3%) diabetics, 258 (11.9%) dyslipidemics and 170 had (8.1%) TIA in the past. Conclusion: The study reveals a distinct pattern of ischemic stroke subtypes in the Indian scenario and has overlapping features of registries from asian countries. Ischemic stroke subtype according to the TOAST criteria was a significant predictor of prognosis. The study results have significant implications for developing preventive and management strategies for stroke care and research in India.

SP14/117: Effect of glycemic status on the severity and outcome of ischemic stroke in patients admitted with type-2 diabetes mellitus

Jayaprakash Muppala, Chandramouleeswaran V, Narasimhan LV, Kannan V

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

Background: Stroke is the second leading cause of long-term disability worldwide .Purpose of the study is to analyse the effect of Glycemic status at admission on severity and outcome of Acute Ischemic stroke in patients with diabetes. Materials and Methods: The study is a prospective, case-control, hospital based study done at Institute of Neurology, at the Madras Medical College & Government General Hospital, Chennai, for a period of 1½ year. Results: 60 acute ischemic stroke patients were studied. 40 were cases with diabetes.20patients were controlswithout diabetes. Case were sub categorized to good glycemic control (HbA1c<7) and poor glycemic control (HbA1c>7). Mean age of patients was 56.78 ± 12.925 years. 97.5% patients of poor glycemic control group has moderate to severe stroke severity >7 (NIHSS) at admission and 53.3% of good glycemic control patients has mild severity (NIHSS=1-6). Glycemic status indicatiors like FPG, PPG, HbA1C had very high statistical significance (0.0001) values among 3 groups of patients. 95% of good glycemic control group and 96.7% of non-diabetic control group patients have functional independence at 3,6 months of follow up. 47.5% of Poor glycemic control patients have functional dependence [>2 MRS] at 3,6 months of follow up. Conclusion: The current study was done to analyse and identify distinctly the clinical effects of diabetes mellitus on the outcome of acute ischemic stroke. Glycemic control has significant association on severity and outcome of ischemic stroke patients with diabetes.

SP15/118: An interesting case of recurrent stroke in young

Usha Kiran Behera, Sree Ranga Lakshmi G, Archana Bethala, Ramesh R

Department of Neurology, Osmania Medical College, Hyderabad, Telangana, India

Background: Delayed cerebral ischaemia (DCI) is a clinical syndrome of focal neurological, cognitive deficits, or both that occurs unpredictably in 3–14 days after the initial sub arachnoid haemorrhage. However the term “vasospasm” should be applied only to a temporary narrowing that is observed within 3 or 4 weeks after SAH. We present a case of a 35 year old lady, hypertensive non-diabetic, presented with acute onset right hemiparesis. She had an aneurysmal subarachnoid hemorrhage (H H grade 3) 3 months ago for which clipping was done. MRI revealed acute ischemic stroke with restricted diffusion in the left anterior and posterior watershed infarcts. MRA revealed a clip at the left MCA bifurcation and a fetal PCA noted. Neck vessel Doppler and 2D ECHO were normal. Coagulation profile normal. She was discharged on antiplatelets and her weakness improved. 3 months later she presented again with right upper limb monoparesis. Her drug compliance was good, and hypertension under control. MRI revealed acute infarcts in the left middle and posterior cerebral artery circulations. Whether this recurrent large vessel ischemia can be attributed to microvascular changes (fibrosis) produced by the clipping or is it a part of delayed cerebral ischemia following sub arachnoid hemorrhage. Such mechanisms have been suggested in earlier studies though the number is limited to a few cases. Conclusion: Thus we suggest that more research is needed to study the underlying pathophysiology and its implication in the long term disability of aneurysmal sub arachnoid hemorrhage inspite of clipping.

SP16/129: A study on the prognostic factors of cerebral venous sinus thrombosis

Lenin Sankar, Chandramouleeswaran, Lakshminarasimhan

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

Cerebral venous sinus thrombosis is rare in comparison with arterial stroke, and it is relatively common in Indian sub-continent when compared to west. The clinical characteristics, prognosis and outcome are different from other stroke subtypes. Data on natural history and prognosis of CVT is scarce. Despite improvements in diagnosis and treatment, dural sinus thrombosis may still cause death or permanent disability. According to International study on CVT risk factors for an unfavorable outcome were identified and included male sex, age >37 years, coma at presentation , intracranial hemorrhage on admission, thrombosis of the deep cerebral venous system. Our study aimed to assess the various poor prognostic factors of CVT in comparison with ISCVT. Our study identified the following the poor prognostic factors viz., age > 37yeras, coma at presentation, intracranial hemorrhage on admission and involvement of deep venous system which is in accordance with the ISCVT but failed to show male sex as a poor prognostic factor.

SP17/132: Usefulness of transesophageal echocardiography in ischemic stroke

Bimal Prasad Padhy, Hari RK

Department of Neurology, CARE Hospitals, Hyderabad, Telangana, India

Introduction: Aorta can be a source of embolic stroke and study of aorta can sometimes give us insight into the atheromatous process going on in the vascular tree.Left atrium and its appendage can be another important source for emboli . Transesophageal echocardiography (TEE) is useful in studying the different parts of aorta, mitral valve apparatus, inter-atrial septal identification and measuring patent foramen ovale, aneurysmal atrial septum and myxoma of left atrium. Objective: To study the findings of TEE in a cohort of ischemic stroke patients. Materials and Methods: The cases are collected from the digitized records of Care Hospital, Nampally, Hydeabad. The present study includes a total of 500 such TEE records. Results: Male:female ratio was 2.6 (males = 362, females= 138). The atrial septal abnormalities were seen in 18 cases. Clots or masses within the atria or ventricles were seen in 22 patients. The mitral valve was more often diseased in ischemic stroke patients, even multiple valve disease was also seen in many patients and the valve disease was of different grades. Conclusions: In the present study, TEE was useful in identifying an embolic source in 10% of patients and helped in initiating anticoagulation. It was also useful in identifying complex aortic atheroma in another 18.4% cases (92 patients) and helped in starting different antiplatelet drug combination, or anticoagulation. TEE helped in identifying the pathogenesis of strokes with difficult to identify mechanisms.

SP18/138: Knowledge, attitude and practice towards stroke risk factors and warning symptoms in caregivers coming to Madras Medical College

Jeyaram VK, Chandramouleeswaran V, Kannan V, Narasimhan LR

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

Background: Stroke is an acute ischemic or hemorrhagic event resulting in major morbidity and mortality rates. Adequate general knowledge is required for identifying the stroke symptoms and availabilty of thrombolysis treatment in stroke, thereby reduces the morbidity rates. Thrombolysis in the acute ischemic stroke treatment is now available in all government setup. Objectives: Assessing the knowledge, attitude and practice (KAP) of the patient caregiver's attending the Institute of neurology OPD, Madras Medical College. Methods: This is a cross sectional study. The questionnaire is made up of 10 questions based on knowledge, attitude and practice regarding stroke risk factors and knowledge about window period. We included 200 patient caregiver's attending with patient in Neurology OPD. Inclusion criteria includes both male and female , age > 20 years, literate and illiterate, stroke patient caregiver's and exclusion criteria includes age less than< 20years. Results: The majority of subjects had insufficient knowledge regarding the risk factors and warning symptoms of stroke. Both literate and illiterate caregiver's had insufficient knowledge. All of them had sufficient knowledge regarding myocardial infarction, but insufficient regarding stroke. None of the participants had awareness regarding thrombolysis treatment. Conclusion: The level of Knowledge, attitude and practice toward stroke risk factors and warning symptoms was inadequate among the majority of participants. To spread the knowledge regarding stroke risk factors, warning symptoms and thrombolysis treatment thro various modalities like social media, news, pamphlets can be used.

SP19/168: A therapeutic dilemma: Infective endocarditis and stroke

Venkatesh Dasari, Hari R, Padhy BP

Department of Neurology, CARE Hospital, Hyderabad, Telangana, India

Introduction: Infective endocarditis (IE) is defined as an infection of the endocardial surface of the heart, which may include one or more heart valves, the mural endocardium, or a septal defect. If left untreated, IE is almost inevitably fatal. Neurological consequences include stroke, TIA, SAH, Septic encephalopathy, Meningitis, Brain abscess. Case: 63 Y,M, K/c/o DM, HTN, CRHD admitted with Fever, SOB, orthopnoea, Chest pain. O/E Temp -1000 F, HR -104/min, BP -110/70, RR-28/min, CVS –Pan systolic murmur. Investigations Hb-10.3gm/dl, TLC-15500 /μl, Platelet count-1.52 lakhs, Urea-54, creat- 1.27, Na-135, K-4.2. 2D ECHO showed vegetations on AML & PML. Blood c/s showed sreptococcus viridans. Diagnosed as IE. Management: Antibiotics as per c/s report. Planned for CABG + MVR. On Day 15 patient had TIA. Sx postponed due to fever. On day 20 patient had left hemiparesis. MRI s/o acute infarct in Rt CG area. Surgery postponed due to acute stroke, AKI, septic encephalopathy. On day 31 patient had worsening hemiparesis. MRI s/o new infarct in midbrain. CABG and MV Replacement done on day 32. After surgery, patient had developed septicemia, AKI required intubation and prolong ventilation, tracheostomy. Gradually he recovered and was discharged on 22nd September [D 43] with residual left hemiplegia. Conclusion: This case management dilema in a patient with IE and stroke. It shows the need for early surgery irrespective of other complicating factors.

SP20/183: Prognosis of the patients of intracerebral hemorrhage with different noncontrast CT markers

Sharif Haji Mohammed M, Thacker AK, Singh AK, Maurya PK, Kulshreshtha D, Qavi A, Singh N

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

Intracerebral hemorrhage (ICH) is the third most frequent cause of stroke, following cerebral embolism and thrombotic disease. Common Etiologies Includes Hypertensive Primary (“Spontaneous”) ICH, ruptured saccular Aneurysm, Vascular malformation, ICH associated with the use of anticoagulants or thrombolytic agents. ICH Expansion occurs in up to one-third number of ICH patients and it is associated with poor outcome. Accurate stratification of expansion risk is therefore crucial, in order to identify patients with high likelihood to benefit from antiexpansion treatment. The CT Angiography (CTA) Spot Sign is a robust predictor of hematoma expansion, but this marker can only be evaluated on CTA, which is not widely available, carries the risk of iodine containing contrast allergy and increased radiation exposure. Several Noncontrast CT (NCCT) Markers associated with ICH expansion have been recently reported. These findings have many overlapping definitions and includes:-- Black Hole Sign, - Blend Sign, - Morphologic Appearance of the Hematoma (presence of Intrahematoma Fluid Level), - Heterogenous Density and Irregular Shape of the hematoma, - Swirl sign, - Island Sign. Axial Noncontrast CT (NCCT) scan of Head was done in all patients at presentation and Repeat NCCT Head was done after 48 hours of presentation in majority of the patients. ICH Score was calculated in all the patients. We Investigated the Prevalence of different NCCT markers. We Analysed the Presence of those NCCT markers with the Prognosis of the patients suffering from ICH. The Proportion of patients with Positive NCCT Markers who experienced ICH Expansion and had a Poor Functional Outcome at 90 days were identified. ICH Expansion was defined as volume increase >33% from the baseline hematoma volume OR an Absolute Increase in Hematoma Volume by 6 ml. Functional outcome was measured using modified Rankin Scale (mRS) and Poor Outcome was defined as mRS >3 at 3 months.

SP21/189: Correlation of TCD with vascular imaging in ischaemic strokes

Saranya Masilamani, Jawahar M, Narasimhan LR

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

Background: TCD using pulsed doppler transducer can show the spectral flow waveforms, blood flow direction, velocities andintensity in the intracerebral vessels. Objectives: To study correlation of transcranial Doppler with MR angiography and CV Doppler in ischaemic strokes. Methodology: 45 patients admitted with ischaemic stroke in neurology department were included in the study. TCD analysis showed decrease in mean flow velocity (MFV) in the upstream vessels distal to the occlusion with increase in pulsatility index (PI). Results: Of the 45 patients, 15 patients had extracranial and 24 patients had intracranial occlusion. In intracranial proximal ICA occlusion, the decrease in MFV in ipsilateral MCA was 70% and 40 % in ipsilateral ACA. In MCA occlusions the decrease in MFV in ipsilateral MCA was 60%. Among patients with intracranial ICA occlusions, the increase in PI in ipsilateral MCA and ACA was 58% and 47% respectively. In MCA occlusions the increase in PI in ipsilateral MCA was 40%. Among patients with extracranial CCA and ICA occlusion decrease in MFV and increase in PI was 70% and 60% in ipsilateral MCA and ACA respectively. Conclusion: The statistical coefficient for mean flow velocity was p=0.662/P<0.05 and that for pulsatility index was p=0.541/P<0.05. Thereby TCD provides insight into a wide range of intra and extracranial vascular pathologic conditions and their effects on cerebral hemodynamics.

SP22/204: Stroke in young a Tertiary Care Centre perspective from South India

Dhanya TS, Kannan V, Chandramouleeswaran V

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

Aim of Study: To study clinical profile and etiology of stroke in young in Madras Medical College. Materials and Methods: The study was conducted at Madras Medical College, Chennai. A total of 920 stroke cases were admitted at Rajiv Gandhi Government General Hospital during the period from October 2018 to March 2019 out of which 135 cases were aged less than 45 years and were included in study. Detailed clinical history along with neurological examination was done. Patients with neuroimaging evidence of acute infarct or intracerebral bleed were included in study. CBC, RBS, Renal function tests, liver function tests, fasting lipid profile, ECG, ECHO and 4 vessel doppler, procoagulant work up were done if indicated. Results: Incidence of stroke in young is 15%. Male to female ratio is 3:1. Out of young strokes 71% is ischemic strokes and 29% were haemorrhagic strokes. The commonest presentation was hemiplegia followed by head ache, seizures. The commonest predisposing factor was hypertension 12% followed by Diabetes Mellitus 5%. Mortality was higher in haemorrhagic strokes than in ischemic strokes. Conclusion: The pattern of stroke in young in our study is comparable with elsewhere in India. Preventable risk factors like systemic hypertension and diabetes Mellitus were the most common cause of stroke and life style modification along with treatment can reduce morbidity and mortality and social burden due to stroke. Detailed evaluation and management of other causes like cardiac arrythmias, vasculitis, prothrombotic states should be done in young patients.

SP23/216: Matrix metalloproteinase-9 levels for the diagnosis of ischemic stroke: A systematic review and meta-analysis

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

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

Introduction: Diagnosis of ischemic stroke (IS) is extremely difficult in acute phase. High levels of Matrix metalloproteinase-9 (MMP-9) are known to be associated with infarct extension and neurological deficits after IS. Aims: To conduct a systematic review and meta-analysis of MMP-9 levels in diagnosing and differentiating IS from healthy controls, stroke mimics and intracerebral hemorrhage (ICH) within 24 hours of symptom onset. Materials and Methods: A literature search was carried out until 28th June 2019 in PubMed, Google Scholar, and Cochrane database. Following combination of keywords was used: ('MMP-9') AND ('Ischemic stroke') AND ('Diagnosis') AND ('Blood Biomarkers'). Standardized mean difference and 95% CI were calculated. Statistical analysis was conducted using STATA version 13.0 software. Results: Four studies including 1533 IS and 282 ICH cases had borderline significantly higher levels of MMP-9 in ICH compared to IS (SMD -0.36; 95% CI -0.72 to 0.01). MMP-9 levels were found to be significantly higher in IS compared to mimics (SMD 0.48; 95% CI 0.13 to 0.83) in four studies including 1387 IS and 283 mimics. MMP-9 levels were also significantly higher in IS compared to controls (SMD 0.53; 95% CI 0.13 to 0.94) in five studies including 517 IS and 299 controls. Conclusion: MMP-9 has potential to be used as a biomarker to differentiate IS from controls, mimics and ICH within 24 hours of onset. Further diagnostic studies with large sample size need to be conducted for MMP-9 to be used in clinical settings.

SP24/244: Prevalence, risk factors and clinical profile of patients with cardio-embolic stroke in South India: A 5-year prospective study

Sandhya Manorenj, Jawalkar S, Bilal MS, Kumar A, Naaz B

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

Background: Stroke is the second leading cause of mortality and morbidity worldwide. Cardioembolic stroke accounts for 15-30% of Ischemic stroke. There have been very few clinical studies on cardioembolic stroke, especially from India. Our aim was to provide a demographic, risk factor and clinical profile of patients with cardioembolic stroke in South India. Methods: A prospective study was conducted at the Neurology Inpatient Department over a period of 5 years from January 2013 to January 2018 collecting necessary information from all cases diagnosed as cardioembolic stroke. The data collected were carefully analysed. Results: Out of 651 patients diagnosed to have acute Ischemic stroke during the study period, 76 patients (11.6%) were diagnosed with cardioembolic stroke, and 35 were females. Mean age was 53.5±12.2 years. Occurrence of cardioembolic stroke was higher (71% vs 28.9%, P value <0.0001) in patients of older age group (46 years or more). A significant proportion of patients of the older age group (37%) had hypertension (P value =0.0048). Coronary artery disease (CAD) was the most prevalent cardiac disorder, observed in 42 patients (55.2%); valvular heart disease in 22 patients (28.9%) and atrial fibrillation in 14 patients (18.4%). Baseline haemorrhagic transformation was noted in 10.5% of patients. 77.6% of lesions had cortical location. Conclusion: Prevalence of cardioembolic stroke in this region is lower than reported worldwide. While male predominance of cardioembolic stroke was noted, old age and hypertension were the major vascular risk factors identified. CAD was the major source of cardioembolism.

SP25/255: When a woman is (COL) led FO(U)R A-Weak- base: Recurrent stroke due to COL-4A mutation

Dhananjay Gupta, Mehta A, Pradeep R, Acharya P, Srinivasa R

Department of Neurology, Ramaiah Medical College, Bengaluru, Karnataka, India

Introduction: Strokes in young and middle-aged population are on a rising trend. They are particularly disabling and have a larger economic impact as the population involved is vocationally and reproductively active. The aetiology of stroke in young individuals is myriad and needs careful detailed evaluation. Recurrence of stroke is one of the important predictors of severe morbidity and mortality. Case: We encountered a 40-year-old lady, who presented with sudden onset spinning vertigo with severe headache and right sided visual loss. Examination revealed right homonymous hemianopia. MRI brain showed an Acute Left Posterior cerebral artery (PCA) infarct with a normal angiogram of brain. She was managed conservatively. A detailed evaluation including Serum homocysteine levels, immunological markers c-ANCA, p-ANCA, ANA profile, Thrombophilia profile and lupus anticoagulant were negative. 2d-Echocardiography and 24-hour Holter monitoring ruled out a cardiogenic origin of stroke and carotid vertebral doppler was normal. A CT angiogram of aorta, neck vessels and brain did not show any angiographic blocks. The patient improved with conservative management and was discharged on anti-platelet agents and statins. A month later, she presented with intense headache and sudden onset left hemiparesis (MMRC power 2/5) and dysarthria. Neuroimaging revealed an Acute Right Middle cerebral artery (MCA) infarct. All the previous investigations for aetiology of stroke in young were repeated. A trans-esophageal echocardiography did not show any cardiac defects. Lumbar puncture showed normal CSF analysis . Testing for Fabry disease and a Sucrose lysis test were negative. She was managed conservatively, but this time anti-coagulants and prednisolone were added to her treatment regimen, considering a possibility of CNS vasculitis. She improved over next 3 weeks and was able to carry out her daily activities. 2 months later, she developed acute right hemiparesis with global aphasia, secondary to a large Acute Left MCA territory infarct. CT angiogram and other tests for aetiology of stroke were normal. A PET-CT whole body did not show any metabolically active lesions. A meningeal biopsy was suggested to the family members; however, they were unwilling for the same. Excluding all other possibilities, we ordered a genetic profile to evaluate for a genetic cause for stroke and found that she had positive COL-4A1 mutation. We treated her with intravenous cyclophosphamide therapy, along with corticosteroids and anti-platelets. As of now, she has not had another stroke and continues to do well with rehabilitative therapy. Discussion: A literature search for COL-4A1 mutation revealed association with porencephaly, cerebral aneurysms, haemorrhage, retinal defects and cerebral small vessel disease, amongst others. Large vessel strokes have not been previously described with COL-4A1 mutation, though are reported with other collagen vascular disorders. Collagen-4A1 proteins are essential for basement membrane integrity and missense mutations lead to fragile basement membrane and vessel walls. The major site of damage is the brain and this leads to “COL-4A1 stroke syndrome” with weak basement membranes in otherwise strong adults.

SP26/257: Climate, and cerebral venous sinus thrombosis

Manoj Vasireddy, Mathew T

Department of Neurology, St John's Medical College, Bengaluru, Karnataka, India

Background: Cerebral venous thrombosis is an uncommon cause of stroke with varied clinical presentations and predisposing factors.The incidence of CVT is more in India compared to the west. Etiological factors are usually divided into acquired risks (e.g. pregnancy, puerperium, antiphospholipid syndrome, cancer, exogenous hormones) and genetic risks (inherited thrombophilia). Several studies have demonstrated seasonal and temporal variations in the incidence of arterial stroke; however, for cerebral venous sinus thrombosis (CVST), such study seems lacking. The main aim of this study was to investigate whether there is any seasonal variation for CVST, and association between CVST occurrence and temperature. Methods: We analyzed the seasonal distribution of disease onset in a tertiary care centre, with 110 consecutive CVT patients May 2017 to May 2019. In all cases, diagnosis was by MRI venography. Conclusions: We evaluated the number of new CVT cases per month and compared the distribution of new cases during the annual seasons. We found the highest numbers of new CVT cases during the summer months (April to July; n = 43) The number of cases during summer was significantly greater than during the remaining seasons (p ! 0.01).There have been no previous studies from India, to report the seasonal trends in the incidence of CVT. Studies in the wet and middle east previously conducted showed a biannual peak in summer and winter months, but that is not the case in India. The study would like to highlight that climate is the most dominant risk factor in the incidence of CVT, in India.

SP27/262: Unusual cause of cerebral venous thrombosis-AML

Sujana Gogineni, Pradeep R, Acharya PT, Srinivasa R, Mahendra JV, Mehta A

Department of Neurology, Rajiv Gandhi University of Health Sciences, Bengaluru, Karnataka, India

Introduction: Cerebral Venous sinus Thrombosis (CVT) is an uncommon cause of stroke with varied presentations and predisposing factors. Acute Myeloid Leukemia causing CVT is very rare and so far only one case is reported in association with CVT. We report an unusual presentation of AML as CVT A 44 year old lady with no previous medical comorbidities presented with c/o headache, neck pain and generalised body ache of 15 days duration and decreased vision of 5 days. On evaluation was found to be conscious alert and oriented. Bilateral papilloedema with decreased visual acuity was seen. There were no signs of meningeal irritation. MR venogram done showed an small acute infarct in left frontal parietal and temporal lobes with sigmoid sinus thrombosis. Her routine laboratory investigations done revealed leucopenia with total count of 2900 cells per mm3 and peripheral smear was normocytic normochromic anemia along with low Vit B12 levels done. Renal function and liver function tests including coagulation were within normal range. The patient was started on anticoagulants for CVT, antiedema measures and other supportive therapy Bone marrow biopsy was done and started on Vitamin B12 and folic acid supplementaion. The biopsy report was suggestive of AML. Cytogenetic analysis reports are awaited. CVT presenting as an intial manifestation of aml is rare. AML can masquerade as CVT. Clinical suspicion is highly important.

SP28/265: Prevalence of carotid artery disease in patients undergoing coronary artery bypass graft surgery for coronary artery disease

Phani Venkata Suman Modumudi, Rahul Kulkarni Vitthal

Department of Neurology, Deenanath Mangeshkar Hospital and Research Center, Pune, Maharashtra, India

Introduction: Carotid artery doppler is performed routinely prior to coronary artery bypass graft (CABG) surgery to identify the magnitude of carotid artery stenosis (CAS), risk of perioperative stroke and determine if carotid revascularization is required, but the role of preoperative carotid artery screening in identifying perioperative stroke risk is uncertain. Methods: We retrospectively reviewed 1791 patients who underwent CABG between 2009 and 2018. 1314 patients who underwent preoperative carotid doppler were included. Severity of CAS was stratified as: No stenosis,<50%,50-69%,70-99%, complete occlusion. Details of past stroke, perioperative stroke and carotid intervention were noted. Results: Right CAS of <50% seen in 174(13.2%), 50-69% in 148(11.2%), 70-99% in 51(3.9%), complete occlusion in 12(0.9%) patients. Left CAS of <50% stenosis seen in 173(13.1%), 50-69% in 144(11%), 70-99% in 63(4.8%), complete occlusion in 6(0.5%) patients. CAS prevalence was estimated to be 37%. Bilateral high grade stenosis(>70%) to complete occlusion was seen in 18(1.36%) patients. During peri-operative period 5 patients suffered stroke. 3 had no CAS, 2 had high grade CAS(>70%), of which 1 suffered stroke in same territory and 1 in different territory. Conclusion: This study shows high frequency of carotid artery disease among patients undergoing CABG, but proportion of patients with bilateral high grade carotid stenosis requiring carotid artery intervention and incidence of peri-operative stroke is significantly low. Routine screening of carotid arteries prior to CABG may not be justifiable.

SP29/273: Concurrent brain and spinal cord infarction as an unusual manifestation of posterior circulation stroke

Swapan Gupta, Kumar H, Khwaja GA

Department of Neurology, GB Pant Institute of Post Graduate Medical Education and Research, New Delhi, India

Introduction: Ischemic strokes usually follow a defined arterial territory distribution. Based on this principle, a clinician is usually able to define a specific syndrome and the involved vessel based on the constellation of clinical symptoms, especially in posterior circulation strokes. We present a posterior circulation stroke with unusual clinical features. Case: A 38-year-old male presented with sudden onset headache and vomiting with complete weakness of all four limbs. Half hour later, the weakness in both legs had spontaneously improved and when he tried to move, he noticed severe ataxia while standing and walking. Since then, the deficit had become static. On examination, he had right homonymous hemianopia. He had flail arms with complete weakness of both upper limbs. Motor examination of both lower limbs revealed power MRC Grade 4/5 with severe ataxia in both lower limbs. Sensory examination was within normal range. The patient was a known diabetic and hypertensive on irregular treatment. Imaging revealed acute infarcts in bilateral cerebellar, left occipital and cervical cord (C3-C6) region. Vascular imaging revealed non-visualization of right vertebral artery. Conclusion: Simultaneous brain and spinal cord infarction with such an unusual clinical presentation is exceedingly rare. Both embolic and thrombotic mechanisms seems likely in this case.

SP30/278: IVIG induced acute ischaemic stroke in treating GBS: A rare complication

Monika Sachan, Gupta A, Kushwaha S, Yadav A, Gupta C

Department of Neurology, Institute of Human Behaviour and Allied Sciences, New Delhi, India

Introduction: Intravenous immunoglobulin used to treat various autoimmune, infectious, and idiopathic diseases. Here we report a case of GBS who was treated with high dose of IVIG and developed an acute ischemic stroke. Case Report: A 40 year female presented with acute onset rapidly progressive weakness of all 4 limbs for 3 days and unable to close eyes bilaterally for 2 days. There was no history of preceding infection, recent vaccination or any toxic exposure. On examination, she had symmetric pure motor LMN type quadriparesis with generalised areflexia with facial palsy. Pt was started on Ivig. dose: 0.4 g/kg/day × 5 days], along with proper hydration. Nerve conduction studies showed diffuse sensory-motor demyelinating polyneuropathy. On day-12, patient suddenly developed acute painless loss of vision from both eyes. Patient GCS was 15/15 and vitals were stable. Initial NCCT head was normal. After two days, patient developed severe autonomic dysfunction in the form of fluctuating blood pressure and tachy-bradyarrthymia. Patient had sudden cardiac arrest and was put on ventilator support. A repeat NCCT head showed bilateral occipital lobe infract. In spite of best possible measures, patient expired next day. Conclusion: Complications such as acute ischemic stroke with IVIg administration is uncommon. Though these events are rare but they can significantly add to the disease burden in patients of GBS.

SP31/280: Thrombolysis in acute ischemic stroke: Actilyse versus tenecteplace- real world experience

Rajeswari Thirupathi, Kumaravelu S, Buddha SS, Bolla HB

Department of Neurology, Ramesh Cardiac and Multispeciality Hospital Pvt. Ltd., Vijayawada, Andhra Pradesh, India

Introduction: There are two molecules available for thrombolysis in acute ischemic stroke, actilyse and tenecteplase. There are no studies available in India comparing the two molecules. Aims: To compare intravenous tenecteplase with alteplase in acute ischemic stroke patients within the window period of 3 hours attending a tertiary care hospital. 100 patients were studied prospectively and retrospectively. Materials and Methods: Inpatients were studied over 6 years (June 2013 -May 2019). statistical analysis was done on Demography data, NIHSS score, comorbidities,and outcome at 7 days and 3 months. Outcome was stratified as good (mRS 0-2) and bad (mRS 3-6). Results: 102 patients were studied. 56 patients received actilyse; 46 patients received Tenecteplase. The Actilyse group had 41 males and 15 females (mean ages 57.95, 60.48). Base line NIHSS score -10.55 and at 7 Days-1.67. mRS at 3 months-good 42( 75%), Diabetes 30(53%), Hypertension 46(82%), hyperlipidaemia 13(23%) and smoking 9(16%). In the Tenecteplase group, males were 38, 8 female (mean ages 59.11,60.4), Baseline NIHSS score-10.63, NIHSS at 7 Days-3.78, mRS at 90 Days -37(82%), Hypertension25(54%), diabetes20(43%), hyperlipidaemia10(22%) and smoking 9(20%). Good outcome seen with actilyse and tenecteplase are equal (p =0.067). There were 2 symptomatic and 3 asymptomatic Intracerebral hemorrhage (ICH) in actilyse group as opposed to 2 asymptomatic ICH in the Tenecteplase group. Three patients in the actilyse group and one patient in the Tenecteplase group died. Conclusion: Tenecteplase was comparable to actilyse in Indian Stroke patients.

SP32/291: Grey areas of stroke management: Deviating from the tried and tested ischemic stroke guidelines

Shiva Narayan Pattanayak, Ray S, Khurana D, Rebello A, Kathuria H, Lakhanpal V, Chakravarty K, Takkar A, Mehta S, Lal V, Ahuja CK

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Introduction: Newer trials of ischemic stroke have emerged revolutionizing the management of acute stroke. We present the following cases encountered in the last year of our practice where the management was radical and different from the traditional ways of management. Material and Methods: A total of 110 patients presented in the window period of stroke. Of these 78 were thrombolyzed and 15 underwent mechanical thrombectomy. Seventeen patients could not be thrombolyzed due to incorrect time of onset or contraindication to thrombolysis or attendants refusal for ay intervention. Of these patients we present the following eighteen cases to highlight where clinical decision taken was different from established Class 1 level A guidelines for management of ischemic stroke. Intervention: The acute cases were managed with thrombolysis in 10 cases with significant clinical issues in the patient. One patient had ongoing ventricular tachycardia, a second patient was in the first trimester of pregnancy, renal failure was present in 3 cases, extremely delayed thrombolysis was attempted in one patient at 18 hours, re-thrombolysis was done in two patients. another patient was found to be a case of bilateral vertebral dissection due to craniovertebral anomaly. intraarterial alteplase was given in two cases. Outcome: All patients made an uneventful recovery. In some cases, recovery was prolonged and duration of hospitalization was prollonged. Follow-up NIHSS was significantly improved from the admission NIHSS. Conclusion: Apart from the traditional methods of stroke management, the existing stroke guidelines have several grey areas where clinical decision has a significant role to play to improve patient prospects.

SP34/307: Physical activity in urban population: Baseline data of 7137 participants from cohort study at AIIMS, New Delhi

Sachin Kumar, Verma V, Prasad K, Kant S, Dwivedi SN, Vibha D, Pandit AK

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

Background: A prospective population cohort study has been funded by Department of Biotechnology, Government of India. Objective: To determine risk factors associated with stroke and cognitive decline in community dwelling persons aged 50 years and above and to determine the proportion of participants with various level of physical activity. Methods: A systematic house to house survey in Vasant Kunj and Munirka Colony of South Delhi, India was conducted to recruit the eligible individuals. Those consented were administered a questionnaire to record medical history, dietary habits, physical activity, health status, ability to perform activities of daily living, sleep quality, neuro-cognitive functions and MRI brain. Results: The data of 7137 participants have been entered and analysed for the present study. Their physical activity is measured through International Physical Activity Questionnaire (IPAQ). It was found that 43% of the participants have low physical activity as per WHO standard. Among the participants 52% and 6% have medium and high physical activity respectively. Females are found to be less physically active as compared to males and this association is statistically significant (p<0.0001). Conclusion: Approximately 43% of participants in the urban population perform inadequate physical activity and thus at the increased risk of stroke and cognitive decline.

SP35/315: Unilateral cerebral deep venous thrombosis masquerading as thalamic glioma

Priyanka Samal, Satpathy DK, Rath A, Murmu S, Acharya S, Mishra B, Mitra C, Das BS

Department of Neurology, BR Life Kalinga Super-specialty Hospital, Bhubaneswar, Odisha, India

Introduction: Cerebral deep venous thrombosis usually presents as bilateral thalamic infarcts as the deep venous system drains both the thalami. Aberrant venous system may result in unilateral venous infarcts with edema which may be confused with neoplastic lesion. History: A 35-year-old pregnant female with 6 weeks amenorrhea presented with recurrent vomiting with headache. A week later she developed insidious onset rapidly progressive weakness of the left side of the body within 3 – 4 days. It was associated with behavior abnormality. Past History: Secondary infertility. Multiple hormonal injections prior to and after conception. Course of Illness: MRI Brain showed unilateral thalamic ICSOL with edema. The pregnancy was terminated and the patient was referred to higher center for stereotactic biopsy and further management. Previous imaging was reanalyzed with special emphasis on source image. Deep venous system could not be visualized. Repeat imaging showed extension of infarct to the opposite thalamus with hemorrhagic transformation. The thrombosis had extended into the superior sagittal sinus. The patient was immediately started on anticoagulants. Her sensorium gradually improved. Imaging: Will be provided Follow up: At 8 months follow up the patient was ambulatory, able to walk independently. Take home message: 1. Detailed history is a must and often provides crucial clue to the diagnosis. 2. One should analyze the venogram meticulously (including the source images). Technicians should be aware of the importance of deep venous system when reconstructing the venogram from source images. 3. Early detection and prompt initiation of anticoagulation results is favourable prognosis.

SP36/316: Non aneurysmal sub arachnoid hemorrhage in Takayasu's arteritis: A case report

Shashidhar Manchala, Jukkarwala A

Department of Neurology, Geetanjali Medical College and Hospital, Udaipur, Rajasthan, India

Background: Takayasu's arteritis is an inflammatory vasculitis of unknown etiology that involves the aorta and its major branches, the pulmonary arteries, coronary arteries and rarely cerebral arteries. Vascular inflammation may cause arterial stenosis, occlusion, thrombosis, dilatation or aneurysms. Takayasu's arteritis affects young people, generally women of reproductive age. Clinical manifestations vary depending on the site of blood vessels and effected organs involved . Cerebrovascular accident may be an important and predictive complication for the prognosis in such patients. Case Presentation: We report a case of 15 year old girl with recurrent low grade fever and sudden onset severe episodic headache for 1 day. Her clinical examination showed asymmetrical pulses, blood tests showed raised inflammatory markers and CSF study showed RBC predominance. MRI brain showed features suggestive of subarachnoid hemorrhage. Subsequent DSA study showed large extracranial vascular occlusion suggestive of large and medium size vessel vasculitis without aneurysm. She was diagnosed to have takayasu's arteritis with non aneurysmal subarachnoid hemorrhage. Results: She was started on steroids and in next 4-5 days she showed improvement clinically and radiologically. Conclusion: Takayasu's arteritis (TA) is to be suspected in a case of young female with asymmetric pulses, unexplained fever, headache with raised inflammatory markers. TA with SAH is a unique presentation, can be with or without aneurysms. Non aneurysmal SAH in takayasu's arteritis has rare presentation with good prognosis.

SP37/321: Carotid artery stenosis in first-ever ischemic stroke: An observational study

Praveen R, Rajan MDK, Unni MN

Department of Neurology, KIMS Hospital, Thiruvananthapuram, Kerala, India

Background: Carotid artery stenosis is a risk factor for ipsilateral ischemic stroke, artery to artery embolisation and TIA. Methods: A prospective observational study with 150 consecutive cases presenting with first-ever stroke. Eligible patients were aged between 50 to 75 years, had features of first episode of acute stroke affecting the anterior circulation, first episode of TIA or with radiological confirmation of acute ischemic stroke. Primary outcome was the prevalence of carotid artery stenosis in first-ever ischemic stroke determined by using duplex scan of the carotid artery, MRI and MRA. The Secondary outcome included other variables like socio demographic details, the relationship with hypertension, diabetes, smoking, exercise, coronary artery disease, dietary habit, ethanol consumption, etc. Observarions and Results: The mean age of patients in present study was 63.2 ±7.27 years, 65.3% were male and 33.7% were female, body mass index 24.2 ± 4.21, median NIHS score 7, mean blood sugar level of 158.5 63.8. 22% were smokers and 16% ethanolic. Diabetes was seen in 67.33%, hypertension 50.7%, dyslipidemia 93.3%, . 10% had CAD and 4% had AF. The prevalence of significant carotid artery stenosis was 22.67%. 12(8%) of the patients had 100% stenosis or total occlusion of the carotid artery. Conclusions: There was higher prevalence of total occlusion or 100% stenosis and more common in the age group less than 60 years of age. There was higher prevalence of carotid artery stenosis with older age, CAD, hypertension, smoking and obesity.

SP38/323: A study of clinical, radiological and thrombophilia profile in cerebral venous sinus thrombosis

Dhaval Dave, Khan F

Department of Neurology, Dr. D. Y. Patil Medical College, Hospital and Research Center, Pune, Maharashtra, India

Introduction: A prospective, observational study was done at a tertiary care hospital in Pune to describe the clinical, radiological and thrombophilia profile of cerebral venous sinus thrombosis in an Indian population. Methods: 30 patients with clinical and MRI features suggestive of cerebral venous sinus thrombosis (CVST), were studied with detailed clinical evaluation and pro-thrombotic work up. All were followed up monthly for 6 months, to assess the response to therapy and clinical outcomes. Results: Headache was the most common presenting symptom and papilloedema was the most common neurological sign. The most common sinus involved was transverse sinus in majority of cases. In the unprovoked CVST subset of patients prothrombotic states were commonly identified. Provoked and idiopathic CVST accounted in almost same percentage of patients in this study. Conclusion: CVST is an important yet under recognized cause of intracranial hypertension and stroke in young. Clinical presentation is extremely varied and a high index of suspicion is needed. Magnetic Resonance Imaging (MRI) brain with Magnetic Resonance Venography (MRV) is the current diagnostic modality of choice. Prothombotic states are common in cases of unprovoked CVST in an Indian population. Management with anticoagulants is safe and has excellent clinical outcomes.

SP39/350: All hempiparesis are not contralateral: A case report

Mahmood Ali Mohd, Reddy M, Parida S, Jaiswal S, Kumar S, Lalitha P, Mohd O, Murthy JMK

Department of Neurology, Institute of Neurological Sciences, CARE Hospital, Hyderabad, Telangana, India

Introduction: Supratentorial infarcts cause contralateral motor weakness due to decussation of corticospinal tracts at the level of caudal medulla. Ipsilateral hemiparesis following supratentorial stroke is rarity, hence this case report. Case Report: A 56 year old male known hypertensive and ischemic stroke 6 months ago with right hemiparesis presented with complaints of left hemiparesis of 2 hours duration. MRI brain showed acute infarcts in left MCA territory with no acute infarcts in the right hemisphere. MR angiography showed left middle cerebral artery (M2) stenosis. He was thrombolysed uneventfully. Diffusion Tensor Imaging done later in view of ipsilateral hemiparesis revealed no decussation of the corticospinal tracts (uncrossed pyramidal tracts). Conclusion: Ipsilateral hemiparesis following stroke is a rare finding and may be due to involvement of uncrossed corticospinal fibres. Additional imaging modalities like functional MRI or MR tractography can confirm this entity.

SP40/353: Outcome of mechanical thrombectomy from Tertiary Centre in artemis agrims neurosciences

Hilal Ganaie, Gupta V, Parthasarthay R, Londhi S, Jain N

Department of Neurology, Government Super Speciality Hospital, Srinagar, Jammu and Kashmir, India

Background: Mechanical Thrombectomy (MT) is Class I indication for acute stroke with Large Vessel Occlusion within 6-16 hrs. Aim: Outcome of MT from a Tertiary centre in Artemis Agrims Neurosciences. Methods and Results: A Total of 40 patients of acute Ischemic Stroke underwent mechanical Thrombectomy from September 2017- March 2019. The mean age of presentation was 59.28 yrs (21-88), male and female were 22 and 18. The mean onset to door time, door to imaging time and puncture to reperfusion time was 189 minutes, 31 minutes and 44 minutes respectively. the mean NIHSS, CT ASPECT and MR ASPECT were 14.7, and 7.1 and 6.8 respectively. Tandem ICA and MCA occlusion were 4 Cases, Terminal ICA 5 cases, MCA- M1 20 cases, MCA- M2 5 cases, MCA, Branch occlusion 2 cases and Basilar Top were 5 cases. Fifty seven % had TICI 3 and 35 % had TICI2b and only seven had TICI2a reperfusion rate. Solitaire stent retrieval was used In 27 MT, Aspiration technique in 7 MT, combined technique in 4 and rescue stenting in two cases. Eighty % of acute stroke patients after 3 months of MT were having MRS of less than or equal to 2 and only 20% patients had MRS of 3 or more. Four patients Expired. Conclusion: Our results of successful Mechanical Thrombectomy were compared to national and international data. Experience and Newer Techniques and patient selection are key factors for favourable outcome in Mechanical Thrombectomy.

SP41/374: Is the mechanism and aetiolgical spectrum of ischemic stroke changing in India: A hospital-based stroke registry study

Abhinay Gattu, Murthy JMK

Department of Neurology, CARE Hospitals, Hyderabad, Telangana, India

Introduction and Background: India is in a transition phase with major life style changes of the population. Population is aging. There has been in the prevalence of chronic rheumatic heart disease (CRHD). With these changes it is possible that the mechanism and aetiological spectrum may be changing in Indian population. Methods: This is a retrospective analysis of prospectively collected Institute Stroke Registry-based data. Mechanism and aetiological spectrum of ischemic stroke were compared between two time periods to determine any change in various variables. The two time periods were: T-A 2008-2010 and T-B 2016-2018. All the subjects registered in the Stroke Registry were comprehensively worked-up for the mechanism and aetiology of ischemic stroke. The data collected included: demographic data, cardiovascular risk factors, mechanism of stroke, and causes. Results: Total number (all age groups) of first ever ischemic stroke in the two time periods were: T-A 548 and T-B 450. The significant differences between the groups were: (1) The mean age was significantly higher in T-B when compared to T-A [60.83+13.95 vs.58.32+13.04;p=0.0035); (2) Large artery atherosclerosis significantly higher in T-B [212(47.14% vs. 224(40.8%);p=0.046]; and (3) Cardioembolic strokes due to nonvalvular atrial fibrillation (NV-AF) was significantly more T-B [13(11.6%) vs. 21(31.5%);p=0.0009]. Whereas there was no change in the incidence cardioembolic stroke due to CRHD-AF [30(26.7%) vs. 22(30.13%);p=0.6212]. Patients aged 50 years and above: The significant differences between the two time periods were (1) The mean age in the T-B was significantly higher in T-B [66.08+11.56 vs. 63.59+9.12;p=0.0009]; and cardioembolic stroke due to NV-AF was significantly more in T-B [23(41.07%) vs. 9(11.68%);p=0.001]. Patients aged 49 or less: (1) Stroke due to undetermined type was significantly more in T-A [29 (23.9%) vs. 9(11.1%);p=0.02]; and (2) Cardioembolic stroke due to CRHD was not in excess. Hyperhomocysteinemia was the most common cause in both the time periods: 81.8% vs. 57.14%. During T-B period more patients arrived in the window period for iv thrombolysis (100 vs. 56 p<0.0001) and more patients received iv thrombolysis (91 vs. 40 p<0.0001). Conclusions: There are some distinct differences seen between the two time periods.·Age spectrum is changing and stroke in the elderly increasing. Stroke due to large artery atherosclerosis is increasing more so in the elderly. Cardioembolic stroke due to NV-AF is significantly increasing. Recent global burden of the disease clearly suggests reduced prevalence of CRHD. But there was no significant reduction in stroke related. Hyperhomocystenimia is seen in all the age groups and vitamin B12 deficiency state. Utilizing of iv thrombolysis is increasing. Significance: This study suggests that with increasing age of the Indian population the burden of stroke would be huge.

SP42/378: Communicative effectiveness abilities among persons with aphasia: A preliminary study in Indian context

Sonal Chitnis, Adhyaru MK, Rao S, Jagtap S

Department of Neurology, Bharti Hospital and Research Center and Deenanath Mangeshkar Hospital, Pune, Maharashtra, India

Introduction and Background: Communicative effectiveness among persons with aphasia (PWA) is less investigated & measured. Present study aimed to develop an interview tool to study effectiveness of communication abilities among PWA. Stroke aphasia , bilingualism, caregiver's and patient's perspective were thoughtfully considered while constructing tool. In addition, the tool provides information regarding change in performance in the communication abilities post acute and chronic stroke aphasia over time. Materials and Methods: 32 Marathi speaking persons with aphasia ( 20 Males &12 females, mean age 64.5 yrs, mean education 9 yrs) irrespective of literacy, gender participated in the study . Further PWA were grouped into Recovering group (Acute stroke n=18) & Stable group (Chronic stroke n=14). Supporting partners of PWA rated the communication abilities on interview tool titled Assessment Tool of communicative effectiveness (ATOCE, Rao, Adhyaru & Chitnis, 2015) twice on initial and repeat testing after 6 weeks along with Marathi version of Bedside Western Aphasia Battery –Revised (WAB-R Marathi) to PWA. Ratings & WAB-R helped measure change in performance of communicative effectiveness of PWA over 6weeks. Results and Discussion: Assessment Tool of communicative effectiveness (ATOCE) showed high validity and internal reliability (α < 0.99) for both recovering and stable groups. The interrater reliability was high for recovering group (α < 0.99) and low for stable group (α < 0.42). This revealed that the communication effectiveness judged by the spouse and the children varied to a greater extent. Significant difference was observed for communication effectiveness between initial and repeat testing among Recovering group in all sub domains of ATOCE (p<.01) as compared to stable group PWA. Majority of non fluent aphasic patients revealed common recovery pattern of aphasia subtype evolution. Most of PWA irrespective of bilingual environment & language exposure revealed significant second language attrition more on expressive language. 78% Biliterates revealed complete loss of reading, writing, calculation in L2 whereas 40% PWA from recovering group revealed equal loss of language functions across modalities. Recovering group showed statistically significant change in performance( p<0.001) across domains of the ATOCE as well as in totality whereas, no significance was evident for the Stable group (p>0.05). There emerged high correlation (r=.98.p=0.001) between WAB R & ATOCE score for both recovering and stable groups on initial & repeat testing. Conclusion: This preliminary research from Indian aphasia perspective helped us understand Coomunicative impairment at various skills of PWA across language modalities, life skills ADL communication in Indian bilingual acute & chronic aphasia patients & their preserved vs regressed abilities . ATOCE is easy to administer outcome measurement tool. Limitation: Study couldn't control & compare across types of stroke pathology, lesion localization, therapeutic intervention and imaging to understand change in communicative efffectivness recovery pattern. Small sample size, lack of functional imaging markers. Future Scopes: Study can be extended over large clinical population considering & comparing vascular and degenerative aphasias. Functional neuroimaging and neurobiological aphasia intervention can be explored across groups to understand neural recovery among various aphasia patients and clinical predictors associated.

SP43/381: Association between methylenetetrahydrofolate reductase gene C677T gene and risk of ischemic stroke

Arti Gulati, Mishra A, Gulati S, Gulati S, Kumar A

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

Background: Stroke is second most cause of death or disability worldwide. Several conventional risk factors have been discovered for stroke; however, these risk factors do not completely explain the mechanismandpathophysiology of stroke. Study of Genetic risk factor is emerging as important aspect for understanding mechanism and discovery of population specific drug target for stroke treatment. Methylenetetrahydrofolate reductase gene (MTHFR) gene is an important gene for homocysteine metabolism and high homocysteine level is an important risk factor for stroke. Objective: To determine association between MTHFR C677T gene polymorphism and risks of ischemic stroke using meta-analysis approach. Methods: We searched electronic databases Medline, EMBASE, and Google Scholar. Pooled odds ratios (ORs) with 95% confidence intervals (CIs) computed using random effect model if heterogeneity was less than 50% otherwise fixed effects model. Results: A total of 56 case–control studies fulfilled our inclusion criteria comprising 18118 cases and 23637 controls. A total of 44 studies were from Asian population and 9 from Caucasian population. MTHFR gene polymorphism at 677 location is significantly associated with the increased risk of ischemic stroke (OR, 1.21, 95% CI 1.14 to 1.27, P < 0.001). Conclusion: Individual carrying MTHFR C677T gene polymorphism have higher chance to develop stroke as compared to controls.

SP44/383: Endovascular management of brain arteriovenous malformation: Ten years of experience

Sibasankar Dalai, Narayana RV, Kumar S, Pati R, Modi S

Department of Neurology, Seven Hills Hospital, Visakhapatnam, Andhra Pradesh, India

Introduction: Brain arteriovenous malformation (AVM) is a tangle of abnormal blood vessels connecting arteries and veins in the brain. Brain AVMs are rare and affect less than 1 percent of the population. Most people are born with them, but they can occasionally form later in life. Patients with brain AVMs experience signs and symptoms such as headache, seizures, intracranial hemorrhage etc. Brain AVMs can damage brain tissue over time. Aim: To analyse the outcome of embolization of brain AVM in past ten years in our institute. successful outcome, complications, embolization strategies and how to overcome complication will be discussed. Materials and Methods: Patients with brain AVMs presented to SevenHills Hospital in the last ten years were evaluated clinically, radiologically and treated by endovascular methods. Results: Total Brain AVMs treated -127, Total No. of sessions-81+110=191(14.6 per year), 46 AVMs treated for an average of 2.4 sessions. Rest of the AVMs (81/127) one sessions Embolization. Complete Embolization achieved in 78/81(96.2%) 20/46(43.4%) Total 98/127(77.1%). Complications (10.2%) Procedure related Death-8(6.2%) early venous occlusion, catheter pulll back, ICH, SAH, Morbidity-5(3.9%), Slurred Speech, Limb weekness. Conclusion: Endovascular treatment of brain AVMs are safe and effective.

SP45/423: Cortical blindness in a young female secondary to perimyocarditis leading to systemic thromboembolism

Sumit Verma, Uniyal R

Department of Neurology, King George's Medical University, Lucknow, Uttar Pradesh, India

Introduction: Although rare, left ventricular dysfunction due to perimyocarditis can lead to decompensated cardiac insufficiency. Embolic phenomena including loss of vision (cortical blindness) may be caused by multiple brain infarctions in the posterior cerebral circulation subsequent to cardiogenic embolism. Case History: We present here a case of a 14 year female with no prior history of rheumatic heart disease (RHD) who presented with a high grade fever 4 days back with bilateral painless loss of vision followed by left arm, face and leg weakness along with an altered sensorium on the same day. Next day, she developed petechial spots over bilateral palms, soles, digits and toes. Physical examination revealed multiple palmar and plantar petechiae, and gangrene of the medial two digits of the left hand. Cardiac auscultation did not reveal any murmur. MRI head showed acute infarcts in bilateral visual cortices along with an area of diffusion restriction in the right parietal region. Cardiac biomarkers (CPKMB and Troponin T) were elevated. 2D-ECHO revealed a clot (11x14mm) the left ventricle with LV dysfunction. A diagnosis of systemic thromboembolism with cardioembolic stroke secondary to perimyocarditis was considered. Conclusion: Perimyocarditis as a cause for acute heart insufficiency has to be taken into account in young patients without any history of cardiac abnormalities and preceding infections.

SP46/446: Risk factors and mechanisms of Ischemic stroke in young from a South Indian Tertiary Care Referral Hospital

Muralikrishna Konda, Kaul S

Department of Neurology, KIMS Hospital, Thiruvananthapuram, Kerala, India

Background: Stroke in young has devastating implications for the patient, family and the society. There is need to identify young adults at risk of stroke. Aim of the Study: To identify the risk factors and mechanisms underlying ischemic stroke in young. Materials and Methods: Young strokes constituted 15.3%(91) of all ischemic strokes (595). Ninety one consecutive patients of ischemic stroke (15-45 years age group) were enrolled in the study. All patients underwent hematolgical risk factor profile, MRI & MRA of brain, carotid & vertebral doppler, 2D Echo and TEE evaluation. Results: Among the risk factors the most frequent were hypertension - 27 (31%), Diabetes - 27 (31%) & smoking - 17 (23.3%). The most frequent mechanism of stroke in young was stroke of other determined etiologies in 22 (24.1%) comprised Moya - Moya- 6 (6.5%), hematological disese - 3 (3.25%), Aortic dissection- 2, FMD - 1, CADASIL - 1, Cocaine- 1, Migraine - 1 and RVD - 1. Second mechanism was Cardio embolism- 16 (17.5%) which included CRHD with AF - 8. Other mechanisms were Extra cranial atherosclerosis - 13 (14.2%), small vessel disease - 11 (12%) and stroke of unknown etiology-22 (24.1%). Conclusions: Conventional risk factors like Hypetension, Diabetes and Smoking were common risk factors even in young stroke. Unlike older adults, stroke mechanisms in young were stroke of other determined etiologies followed by Cardioembolic and Extra cranial atherosclerosis. Mechanism remained unknown in almost one fourth of all young strokes.

SP47/448: Risk factors and outcome of recurrent ischemic stroke: A prospective study in a Rurban Medical College

Ranjith Polusani, Reddy S

Department of Neurology, Prathima Institute of Medical Sciences, Nagunur, Telangana, India

Cerebrovascular disease CVD is the most common disease group among all the neurological diseases. The prevalence of strokes in India is increasing as found in community-based studies. Recurrent strokes are preventible yet increasing in prevalence and are disabling. Aim: We in the present study tried to evaluate the risk factors and outcomes of recurrent ischemic stroke in patients presented to a Rurban Medical college in Telangana. Methods: This study was conducted at Prathima Institute of Medical Sciences a tertiary care hospital located Nagunoor, Karimnagar. 100 consecutive cases admitted under Arogyasri scheme with first recurrent ischemic stroke are included in this study. A modified Rankin Scale was used to assess the outcome of the first recurrent ischemic stroke patients and also analyze various risk factors of recurrent ischemic stroke with reference to history, symptomatology, and investigations. Out of the 100 patients admitted during the study 22 were lost during follow up and 5 developed another recurrent stroke within three months of follow up and all these were excluded from the study and 73 patients were taken for evaluation and statistical analysis. Results: The mean age for men was 58.06 years women were 62.52 years the total number of the male in our study was 38 (52.1%) and the female was 35(47.9%). Out 73 studied Out of 73 16 (21.9%) were with hypertension and 6 (8.2%) were diabetic, 19(26%) were smokers and 9 (12.3%) were only alcoholic 7(9.5%) were both and 38 (52.1%) had no addictions. During the recurrent stroke 39(53.4%) patients had the weakness (hemiparesis or hemiplegia or monoparesis or monoplegia) as the only presentation, 10(13.6%) patients had both weakness and facial palsy as their presentation, 16(21.9%) of patients had both weakness and aphasia as their presentation. At the third follow up after second stroke every patient some disability (MRS 1-5) on comparison of the patients with their residual weakness and weakness at the third follow up 21(28.7%) patients had MRS 1 as their residual disability and 10(13.6%) patients had MRS 1 as their disability at follow up. Conclusion: It can be concluded that the risk factors and outcome of RIS in this study were comparable with other similar studies. The important risk factors were hypertension, diabetes mellitus, smoking, atrial fibrillation and decreased compliance with the treatment. Therefore an improvement in patient adherence to treatment is a single very important factor in addition to optimal treatment and follow-up in decreasing the incidence of RIS.

SP48/454: To determine the frequency of left atrial enlargement in different subtypes of ischemic stroke based on toast classification

Dinesh Chaudhari, Renjen PN, Saini R, Kumar A, Mishra A

Department of Neurology, Indraprastha Apollo Hospital, New Delhi, India

Introduction: The relationship between Left Atrial Enlargement (LAE) and stroke is complex. Rhythm disturbance that characterize atrial fibrillation is also associated with other atrial derangement such as endothelial dysfunction, fibrosis, impaired myocyte function and chamber dilation. Greater LA volume and reduced LA reservoir function are associated with subclinical cerebrovascular disease in subjects without history of stroke also. Background and Aims: The role and basic characteristic of LAE in acute cerebral infarction patient is not sufficiently described in literature, especially the relation between stroke subtype and left atrial enlargement. Hence this study was undertaken to look for the frequency of left atrial enlargement in acute stroke subtypes. Material and Methods: 119 patients with acute ischemic stroke admitted during the study period (June 2016 to March 18) were included. Patient's detailed history and thorough physical examination was performed. Routine blood tests, 12 lead ECG, Trans Thoracic Echocardiography were performed within 24 hours of admission. Using clinical data, radiological images and investigation results, stroke subtype of each patient was determined based TOAST criteria. P wave morphology in lead V1 of ECG was evaluated in each patient to look for left atrial enlargement and PTFV1 > 4,000 microvolt ms was considered to be left atrial enlargement by ECG voltage criteria. Results: Mean age of male and female stroke patients was 61.13±14.51 years and 62.11±13.34 years respectively. Percentage of stroke due to undermined etiology was highest (36.4%) followed by cardio embolic stroke (22.1%). Out of 154 patients, 64 (41.5%) had LAE. Small artery occlusion was found among 20.1% patients and large artery atherosclerosis was seen among 17.5% patients. Stroke of other determined etiology was noted in only 3.9% of stoke patient. Hypertension was most common risk factor among the stroke patient in this study (61.6%), followed by LAE (41.5%) and Diabetes mellitus (38.3%). Indexed left atrial diameter was significantly (p=0.02) higher among hypertensive (2.45±0.32) patients than non-hypertensive (2.20±0.24). Indexed LA diameter was significantly (p=0.001) higher in Cardio embolic group (2.67±0.30) than other groups. LAE was most frequent in the cardio embolic group (40.6%) followed by undetermined cause group (35.9%). Conclusions: Second Highest frequency of LAE found in undermined group raises the possibility cardiogenic origin of stroke, at least in some of these patients. Paroxysmal AF or left atrial pathology without AF may explain this phenomenon. As these patient would be prone to recurrent stroke, stroke patients of undermined etiology with LAE should be evaluated in detail including more prolonged holter. Further given the proven benefit of anticoagulant therapy in preventing left atrial thromboembolism in patients with AF, further studies may be worthwhile to determine optimal markers of atrial cardiopathy and the effect of anticoagulant therapy in patients with conclusive evidence of atrial cardiopathy, but no clear evidence of AF.

SP49/458: Fever, hyperglycemia, swallowing and hypertension management in acute stroke: A systematic review

Astha Rai, Amit Kumar, Shubham Misra, Prerna Dheer, Pumanshi Talwar, Kameshwar Prasad

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

Introduction: Management of fever, sugar, swallowing, and hypertension (FeSSH) is considered as effective intervention to improve outcome in acute stroke. Aims: To systematically review trials published on the management of fever, hyperglycemia, swallowing and hypertension in acute stroke patients. Materials and Methods: A literature search was conducted in PubMed, Cochrane and Google Scholar databases till 4thJuly2019. Following combination of keywords was used: ('Fever') AND ('Swallowing') AND ('Hyperglycemia') AND ('Hypertension') AND ('Management') AND ('Stroke'). Results: A total of 21 articles were searched and only two articles were found eligible for inclusion in our systematic review. Middleton et al. (2011) included 1696 patients and randomized 19 hospitals. They concluded that workshops/educational sessions for management of fever, hyperglycaemia, and swallowing in acute stroke during initial 72h of admission to hospital can result in decreased rates of death, dependency, and improved adherence to protocol. Another study by Wang et al. (2018) in which 40 hospitals were randomized involving 4800 acute ischemic stroke (AIS) patients. They observed statistically significant but small improvement in hospital personnel adherence to evidence-based performance measures in AIS patients when assessed as a composite measure, but not as all-or-none measure. Conclusion: There is great potential to manage stroke care using these quality care protocols. These types of trials are essential for countries like India. AIIMS, New Delhi has initiated a cluster randomized controlled trial in India in which 32 hospitals will be randomized to determine whether implementation of quality care protocol reduces mortality and improves stroke outcome.

SP50/482: Carotid atherosclerosis disease pattern in south Indians: CT-angiographic-based study

Mahmood Ali Mohd, Murthy JMK, Mohd O, Reddy M, Jaiswal S, Parida S, Lalitha P, Kumar S

Department of Neurology, CARE Hospitals, Hyderabad, Telangana, India

Background: The carotid atherosclerosis disease pattern is different in Asians as compared to western population. There are no quality studies from India that defined the carotid disease pattern. Methods: This is a retrospective study of prospectively collected data from the institute Stroke Registry. We analysed 272 consecutive CT-angiograms done in patients with ischemic stroke between January 2016 to December 2018. Any stenosis more than 50% was considered as critical stenosis for both intracranial and extracranial vessels. Results: During the study period 451 patients with ischemic stroke, 272 (60.3%) [mean age: 61.68 years, age <49 years 46 (17%); males 70-95%] received CT-angiography. Isolated extracranial disease (>50%-99%) was present in 9.55% of patients, only 20 (7%) were below <49 years. Pure intracranial large artery atherosclerosis was noted in 80 (29.42%) of patients. Significant intracranial atherosclerotic disease along with extracranial ICA disease was present in 29 (10.66%}. Young patients had lesser intracranial and extracranial disease. Conclusions: This study suggests that the carotid atherosclerosis disease pattern is different in south Indians, these people have more extracranial and along with intracranial and extracranial disease. This is probably first such study from India.

SP51/483: Study of early versus delayed neuro-rehabilitation in stroke and its correlation with functional outcome

Srinath R, Nanda SK

Department of Neurology, Armed Forces Medical College, Pune, Maharashtra, India

Aim: To study early versus delayed neuro-rehabilitation in stroke and its correlation with functional outcomes. Settings and Design: This single centre Prospective study done in a tertiary hospital studied the differences of Functional outcomes among the two groups of patients of early versus late neuro-rehabilitation in patients of Stroke both Ischemic & hemorrhagic. Materials and Methods: total of 60 patients were part of this study conducted at Command Hospital Kolkata. The Subjects were divided into two arms early (upto one wk) and delayed (more than one wk) depending on the introduction to Neuro-rehabilitation both active & passive. Functional disability was measured at 90 & 180 days. Results: It was observed that the delay in the start of Neuro-rehabilitation had statistically significant adverse functional outcome at 90 & 180 days. The early the initiation of the same the better. Conclusions: Early Neuro-rehabilitation, after adjusting for admission functional status and severity of stroke, remained to be important predictors of stroke functional outcomes.

SP52/485: Clinical and radiological study of cerebral venous thrombosis cases presenting to a tertiary care center

Naga Karthik Vanukuri, Hazeena P, Sugumar, Sundar S, Shankar V

Department of Neurology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Purpose of Study: This study was done to evaluate the clinical and radiological characteristics of CVT patients and assess the etiological factors involved. Study Design: This study was an observational study done on 92 CVT Patients confirmed by neuroimaging from August 2018 to June 2019 in Neurology Department, Sri Ramachandra Medical Centre. Results: Total patients 92, Males 55(59.78%), Females 37(40.22%). Mean age 36.8years (range,14-71years). The common clinical presentation was headache and vomiting in 85.7%, Seizures in 49.7%, altered sensorium 32% and focal deficits 15.75%. Papilledema in 78.3%. Multiple sinuses with Superior Saggital, Transverse and Sigmoid involved in 30.7%, TS and SS in 26.08%, cortical veins in 18.4%, SSS alone in 16.3% and deep cortical veins in 8.6%. Brain Parenchymal lesions present in 49.7%, SDH in 2.17%, SAH in 4.34%. Alcoholism was major factor affecting 61.4% of patients. In females severe anemia in 27% was the common cause followed by Pregnancy related in 5.4% and Hormonal pills usage in 4.2%. Protein C and S deficiency in 5.4% patients. Infections in 4.3%. Malignancy in 3.2%. JAK2 mutation in 1.08%. Conclusion: Headache followed by seizures are the common presenting features. Papilledema is consistently present. Multiple sinuses are involved in most of the cases with superior sagittal sinus being commonly affected. Deep venous system involvement resulted in increased morbidity. The major risk factor was alcoholism especially in young men. In females anemia and iron deficiency are the common causes while conventional risk factors like postpartum state are found to be less frequent.

SP53/504: Assessment of cost of acute stroke care and its determinants at tertiary Care Hospitals of Western Rajasthan: A cost of illness study

Inder Puri, Goswami D, Singh K, Bhardwaj P, Panda S

Department of Neurology, Dr. Sampuranand Medical College, Jodhpur, Rajasthan, India

Objective: A cost of illness study planned with aims to know various costs of acute stroke care and its determinants among non beneficiary and beneficiary of various social security schemes like public health insurance. Methods: We conducted a cross sectional study at government hospitals in western Rajasthan from March to May 2019. All consecutive stroke patients were enrolled. Data related to socio-demographic detail and cost were collected by patient/caregiver's interview. Primary outcome was description of direct and indirect cost of care among beneficiary and non beneficiary patients. Secondary outcome was description of determinants of cost. Results: A total 126 stroke patients were enrolled. Mean age was 57.67 + 15 years. Both beneficiary and non beneficiary patients were similar in baseline characteristic except monthly income (p = <0.01) Mean hospital stay was 6.52+2.23.The average Direct cost among beneficiary was INR 12727.21 (95% C.I. 8658.50, 16795.92) and among non beneficiary was INR 23649.68 (95%C.I. 18591.37, 28707.99).There was significant difference in direct cost of beneficiary and non beneficiary patients (p = <0.01). Mean Indirect cost among beneficiary was INR 12414.75 (95% C.I. 9691.13, 15138.37] and among non-beneficiary INR 16460 (95% C.I. 13044.81, 19875.19). There was no significant difference in Indirect cost (p = 0.06).Monthly income, stroke severity (modified Rankin score) and hospital stay were significant direct cost determinants. Conclusion: Public health insurance scheme reduced Direct cost of stroke care statistically significant. Severity of stroke and prolonged hospital stay were driven cost significantly.

SP54/505: Carotid endarterectomy: CARE Hospitals Hyderabad experience

Batchu Nikhila, Guptha PC, Atturu G, Sharma P, Murthy JMK, Jaiswal SK, Reddy M, Pidaparthi L

Department of Neurology, CARE Hospitals, Hyderabad, Telangana, India

Background: Endarterectomy is the treatment of choice in patients with extracranial internal carotid artery disease >70% – 99% on the symptomatic side; There are only few published studies on this topic from India. Materials: This is a retrospective review of case records of patient who underwent carotid endarterectomy of the extracranial ICA stenosis greater than 50% and less than 99% between January 2016 and December 2018. The data collected included demographics, degree of stenosis, cardiovascular risk factors, NIHS score, type of surgery, immediate postoperative complications and long-term complications. Surgical candidates: patients with NIHS score 0-8. and degree of stenosis 70%-99%. Results: During the study period 63 patients (M:F: 46:17; Mean age M:66.5+12.99years; F:65.7+11.06 years) underwent extracranial endarterectomy. Side of stenosis: 31 vs. 32; indication TIA in 3 and minor stroke in 60. Endarterectomy was done with protective device in 7 patients. Postoperative complications were minor and included numbness and pareshesias over face, chin, jaw and mandible in 6 patients. None of the patients had recurrence of TIA or stroke.. There was one death which was due to stroke-related complications. Conclusion: Carotid endarterectomy was safe and the results were comparable to the reported literature.

SP55/507: Endovascular management of posterior inferior cerebellar artery aneurysms: A singlecenter 7 years experience

Vinit Banga, Mahajan A, Chatterjee A, Goel G

Department of Neurointervention, Institute of Neuroscience, Medanta - The Medicity, Gurugram, New Delhi, India

Background and Purpose: Aneurysms of the posterior inferior cerebellar artery (PICA) are rare, comprising 0.5% to 3% of intracranial aneurysms. Because their anatomic location relative to the PICA origin is variable, several endovascular techniques are used in their management. The use of endovascular treatment as an alternative treatment to surgery has been increasing. The purpose of this study was to report our experience in managing the PICA aneurysms along with the clinical presentation. Materials and Methods: Retrospective analysis of records of all patients with ruptured VA-PICA junction aneurysms treated at our referral center between 2011 and 2016 was performed. During this duration, a total of 17 patients having PICA aneurysms were treated. Results: Mean age of the patients was 43 years; 58.8% (10/17) were women. All the patients presented with anacute intracranial hemorrhage on NCCT. No patient had previous history of any treatment. 5 (41%), aneurysms were located at the PICA origin, however, their relationship with the PICA and vertebral artery was variable. 12(70%) patients had distal PICA aneurysms. Treatment techniques included direct coiling, balloon assisted coiling, parent vessel sacrifice of PICA or stent assisted coiling. Complete occlusion was initially achieved in 17 of 17 patients. 1 patient had recanalisation at follow up and underwent re-treatment. There was no intra-procedural rupture of the aneurysms. Fourpatients developed cerebellar infarctions. One patient died during management. Outcome at 3 months in 16 surviving patients was good in 13(76.4%) of patients. Conclusion: In our experience, PICA aneurysms were challenging lesions. It requires a variety of techniques for their management which varies with regards to the morphology and location of the aneurysms as well as its relationship with vertebral artery.

SP56/508: Stent angioplasty as a rescue in posterior circulation stroke with basilar artery stenosis

Vinit Banga, Mahajan A, Chatterjee A, Goel G

Department of Neurointervention, Institute of Neuroscience, Medanta - The Medicity, Gurugram, New Delhi, India

Objectives: Stent angioplasty as a rescue in posterior circulation stroke with basilar artery stenosis. Background: Atherosclerotic Vertebrobasilar occlusion is associated with high morbidity and mortality with an unfavourable outcome if not timely treated. Methods: Clinical History, Neurological Examination, Neuroimaging and Follow up. 69 year female with altered sensorium and loss of consciousness. Results: A 69 year female presented to hospital within eight hours of sudden onset loss of consciousness and vomiting. She had past history of hypertension, old subdural haemorrhage and cholecystectomy. On examination she was comatose with low GCS. Keeping in mind the possibility of posterior circulation stroke, Code stroke was activated as per hospital protocol. Urgent neuroimaging in the form of NCCT head, CT perfusion and CT Angiography of neck and brain vessels revealed mismatched perfusion deficit involving bilateral thalamus, brainstem and bilateral temporo-occipital cortex along with occlusion of proximal basilar artery. Her MRI Brain showed scattered area of diffusion restriction in bilateral cerebellum along with thalamus, pons and hippocampi. She was taken for urgent mechanical recanalization. Cerebral angiography revealed basilar artery occlusion at left vertebrobasilar junction which was recanalised using aspiration technique. Subsequent check angiography showed severe proximal basilar artery stenosis with clot formation at the site of stenosis which was managed with Intra-arterial abciximab but lead to recurrent clot formation in subsequent check angiograms. It was decided to do stent angioplasty with solitaire stent which resulted in recanalization of basilar artery with reduction in stenosis. Patient did well clinically and was discharged in a stable condition. Conclusion: Patient with atherosclerotic disease involving vertebrobasilar circulation can present with posterior circulation stroke which can be life threatening and disabling. There have been reports of stent angioplasty as elective as well as emergency measure in managing such patients. Emergency stenting has its own limitations such as stent thrombosis. In our patient we used retrievable stent to circumvent such problems and also to avoid issues faced with balloon angioplasty such as perforator occlusion. Therefore, it probably gives us a new tool to deal with such patients in emergency setting and can be used to plan strategies in future.

SP57/98: Comparative study of transcarpal median sensory nerve conduction versus combined sensory index in the electrodiagnosis of carpal tunnel syndrome

Selvaraj V, Hammed TA, Manohar VV, Venkatesan V, Devanathan V

Department of Neurology, Saveetha Medical College, Chennai, Tamil Nadu, India

Aim: The aim of this study is to compare the gold standard Combined sensory index (CSI) technique with the Transcarpal median sensory nerve stimulation as an alternative diagnostic tool for patients with clinically suspected carpal tunnel syndrome. Methods: 62 subjects who have a clinical suspicion of CTS were chosen and NCS done in both the hands.On stimulating the third digit, an orthodromic median sensory nerve conduction was recorded at the palm and the wrist segment.The estimated Median NCV was recorded from the palm and wrist,the gold standard technique (CSI) was also done. Results: Among the 62 patients, 30 hands show normal conduction in both CSI and in Transcarpal median sensory nerve conduction and ,30 hands had abnormal elctrodiagnostic study in both CSI and transcarpal median sensory nerve conduction, 2 had normal CSI and abnormal in Transcarpal median sensory nerve conduction. The Sensitivity is 93.77% with the Specificity of 100% with the Positive Predictive Value (PPV) of 100% and the Negative Predictive Value (NPV) of 93.75%. Conclusion: When comparing with the gold standard CSI technique the transcarpal median sensory nerve conduction is a simple, reliable, time saving technique which can be highly recommended in the electrodiagnosis of carpal tunnel syndrome.

Saturday, October 5, 2019. 07:30AM-13:00PM, Poster Hall Area

Poster Session 3: Neuromuscular Disorders, Clinical Neurophysiology, Neurogenetics, Infections and Headache

NMP1/23: Clinical profile of fibromyalgia in a neurology clinic of South India

Vasanthy B, Nair VCP, Geethadevi M, George J

Department of Neurology, Government Medical College, Kottayam, Kerala, India

Fibromyalgia (FM) is a common disorder in general population & Neurology clinics, whose prevalence is not reported yet in India. Qualitative study showed increased number in neurology clinic who were treated with NSAIDs. Methods: Using 2016 revision of 2010/2011 American College of Rheumatology FM criteria, patients attending neurology clinic were screened. FM patients identified by screening questionnaire underwent physical examination. Clinical profile and proportion of patients were noted. With N=4pq/d2, sample size was calculated. Results: Screened patients were 2300. FM patients were 298. Proportion was 8.053%. Females were 93.6% and 53.6% were in 40-60 age group. Delayed diagnosis of more than a year occurred in 55%. Home-makers and unskilled laborers residing in rural areas formed 85.9%. Common comorbid illness were hypertension, hypothyroidism and diabetes. None were offered cognitive behavioral therapy. NSAIDs were given for 6.7%. Conclusion: Proportion of FM is more in Neurology clinic than in general population. Causes of concern are delayed diagnosis, affection of home makers and manual laborers, comorbid illnesses, and treatment with NSAIDs. Message: Neurologists should be sensitised to the presence and diagnostic criteria of FM. Patients should be diagnosed early to defer possible extensive investigations and NSAID treatment which can cause multisystem damage like renal failure, stroke and other prothrombotic disorders referred. They should be referred for early for cognitive behavioral therapy.

NMP2/59: Winging of scapula due to multifocal acquired demyelinating sensory and motor

Ponda A, Sharma M, Mudgerikar S

Department of Neurology, Apollo Hospitals International Limited, Gandhinagar, Gujarat, India

Introduction: MADSAM (multifocal acquired demyelinating sensory and motor neuropathy) has an insidious onset and slow progression. We present an extremely rare phenomenon of winging of scapula due to MADSAM. Case Report: A 32 year old housewife, presented with paraesthesia in all four limbs, asymmetric for past 6 months with difficulty in lifting object from overhead shelf with right arm and difficulty in combing hair with right arm for the past 1 month. There was no history of trauma or any incident leading to this weakness. A neurological examination revealed distal asymmetric sensory loss in lower limbs with weakness in flexion and abduction of right shoulder and there was winging of the medial section of right scapula especially after 120 degree elevation and when asked to push the wall with both hands. Her nerve conduction revealed asymmetric conduction block, temporal dispersion, prolonged distal latencies, slow conduction velocities, delayed or absent F waves in 1 or more nerves fitting in the criteria of MADSAM. Her family history was negative and she did not have any past medical condition. Her CSF was normal .Patient was treated with steroids 1mg/kg /day and patient completely recovered within 6 weeks. Discussion: Winging of scapula can be traumatic/muscular disease/ neuropathy related. In this case there was associated sensorimotor asymmetrical demyelinating neuropathies of both upper and lower limbs, hence the diagnosis of MADSAM was kept. In our case, as the treatment with high dose corticosteroids was initiated early, the recovery was faster and complete.

NMP3/70: Markesberry-griggs myopathy: A rare case of distal myopathy in two siblings

Jain R, Anand K, Garg J, Saran R

Department of Neurology, Dr. Ram Manohar Lohia Hospital, New Delhi, India

Background: Distal myopathies are rare and often confused with hereditary neuropathies or motor neutron disease. But one must consider a possibility of distal myopathy in late onset bilaterally symmetric distal weakness. This is a case report of a late onset distal myopathy with positive family history in his elder sister. Case Presentation: A 40-year-old man, presented with progressive, bilaterally symmetrical distal muscle weakness of all four limbs for last four years, without sensory complaints. Neurological examination revealed marked wasting of the muscles of anterior compartment of the leg and reduced power, distally in lower limb and upper limb. All DTR were normal. Plantars were not elicitable. On investigating, total CPK was borderline raised (396 U/L), EMG was suggestive of myopathic pattern, increased insertional activity, polyphasia, early recruitment of motor units. Muscle Biopsy from left gastrocnemius showed variation in size of muscle fibres, internal migration of some nuclei and rimmed vacuoles, suggestive of myofibrillar myopathy. There was no accumulation of desmin and ubiquitin. Similar clinical and histopathological findings were noted in patient's elder sister. Conclusion: Distal myopathies are rare presentations of myopathy and Markesbery-Grigs variant, is an autosomal dominant, congenital myopathy, presenting as progressive foot drop, usually after 35 yrs of age. It is caused due to mutations of ZASP, and has myofibrillar myopathy pattern on muscle biopsy. To the best of our knowledge, this is one of the first few cases of Markesbery-Griggs Myopathy reported from India.

NMP4/123: Rare treatable case of congenital myasthenia syndrome

Anusha Challa, Raju GB, Gopi S, Kumar TS, Kumari UA

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

Introduction: The CMS are diverse disorders linked by abnormal signal transmission at the motor endplate .Characteristics of COLQ CMS are Repetitive CMAP by single nerve stimuli and Delayed pupillary light response. Case Report: 5 year old male child, born to non consanguineous married parents presented with fluctuating drooping of eyelids and delayed attainment of motor milestones since infancy. There was weak cry at birth and feeding and sucking difficulties during infancy and recurrent hospitalisations for respiratory infections since his infancy. He attained head control and sitting at 1 year, walking at 2 years of age Ptosis characteristically worsened by the end of the day. Parents and siblings were healthy Examination: mask-like facies, wide-open mouth, bilateral ptosis, sluggish pupillary reaction, ophthalmoplegia ,generalized hypotonia, normal muscle stretch reflexes, and weakness of the trunk and proximal limbs. There were no fasciculations or contractures. Congenital Myasthenic Syndrome was suspected. Investigations: normal CPK (53 U/L), ECG and echocardiography. RNS test revealed second CMAP with nerve stimulation, characteristic of endplate AchE deficiency CMS. Genetic analysis using next-generation sequencing for Congenital Myasthenic Syndrome genes identified homozygous 3' splice site variation in intron 13 of the COLQ gene. Treatment: He was initiated on Salbutamol (0.1 mg/kg/day TID, increased to 0.2 mg/kg/day). On follow up, weakness in limbs improved and no recurrence in respiratory infections or hospitalizations. However, ptosis and ophthalmoparesis persists. Conclusion: Currently, COLQ mutation Congenital myasthenic syndrome is a rare treatable genetic disorder with good improvement in functional status.

NMP5/126: Proximal dominant hereditary motor and sensory neuropathy: First case report from India

Afroz Ansari, Ojha P, Ansari R, Jagiasi K, Soni G, Singh R

HMSN-P is a rare, degenerative disorder inherited in an autosomal dominant fashion. This disease has been described first in Japanese descendants from Okinawa and Shiga prefecture in mainland Honshu in 1997. The disease is characterized by adult onset of proximal weakness and atrophy, muscle cramps, fasciculations, areflexia, high incidence of elevated creatine kinase, hyperlipidemia and diabetes mellitus, resembling Kennedy disease, though mode of inheritance is autosomal dominant, rather than X- limked. Electrodiagnostic (EDX) studies typically reveal abnormalities consistent with sensorimotor neuronopathy. We examined a 56 years old male patient, with 6 year h/o insidious onset, slowly progressive, asymmetric onset, proximal, upper limb onset predominant motor, LMN neurogenic weakness and sensory features, predominat in lower limbs, diffuse fasciculations, minipolymyoclonus, facial myokymia and tongue fasciculations, with significant past H/o painful muscle cramps, for 10 years with autosomal dominant family history in 7 male and 2 female family members among 3 generations. Patient underwent EDX studies, revealing ongoing and chronic neurogenic denervation and motor unit instability,with large fibre sensory involvement, further defining the spectrum of EDX findings in HMSN-P. The Genetic features are also reviewed by whole exome sequencing and heterozygous missense variation in exon 8 of TFG (TRK-fused gene (TFG) gene (chr.3:100467026C>T; Depth: 76x) was detected, The appearance of HMSN-P in India and elsewhere calls for clinicians in non-endemic regions to be familiar with this rare disorder, which has typically geographically confined.

NMP6/155: Relationship between cerebrospinal fluid protein levels, electrophysiological abnormalities and severity of disease in Guillain-Barre Syndrome patients

Prashob PS, Chandramouleeswaran V, Kannan V, Lakshminarasimhan R

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

Introduction: GBS is a post infectious acute autoimmune polyradiculopathy. CSF study and nerve conduction studies aid in the diagnosis of GBS. This study was done to analyze the association between CSF protein level with clinical severity of disease and also with electrophysiological abnormalities. Materials and Methods: A prospective observational study of all diagnosed (clinically + electrophysiologically) GBS patients within 4 month time period. NCS and CSF protein levels were assessed together after one week of symptom onset. Clinical severity was assessed based on MRC sums score ranging from 0-60. CSF protein level of 40 mg/dl was used as upper cut off value of our laboratory. Results: Total 54 patients were admitted with diagnosis consistent with GBS. Mean CSF protein level was 53.7. CSF protein level above 40 was seen in 40 patients (74.%). MRC sum score (0-30) was present in 25 patients (46%), score 30-40 was present in 17 (31.4%) and more than 50 in 3 patients (5.5%). CSF protein more than 100 was observed in 3 patients. All the 3 patients had non stimulatable peripheral nerves. Of the 14 patients with normal CSF protein 9 patients had NCS suggestive of axonal change (64.4%) and rest five patients had demyelinating changes. Conclusion: This study shows CSF protein levels are elevated in GBS. CSF protein levels correlate with clinical severity of the disease. Very high CSF protein levels correlated with severe electrophysiological changes. Patients with normal CSF protein levels had predominant axonal changes in NCS.

NMP7/160: Guillain-barre syndrome: Clinical profile and seasonal variation at a tertiary care centre in South India

Jatin Pothuloori, Venkatraman C, Vellaichamy K, Samivel B, Ranganathan LN

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

Background and Objective: Guillain-Barre syndrome (GBS) is an autoimmune disease and a recognized cause of generalized progressive paralysis worldwide. This study was aimed to document the clinical findings, demographics and seasonal variations amongst the patients with GBS during the hospital stay. Methods: In this study, analysis of patients admitted in department of Neurology, Madras medical college, and diagnosed as GBS was conducted between the time period of November 2018 to June 2019. Demographics, clinical & revelant data were obtained. The patients were divided into two groups depending on the date of admission: S1 (Winter, November to February) and S2 (Summer, March to June) and parameters were studied. Results: A total of 75 patients were assessed, of which 48(64%) were male and 27(36%) females. Sex ratio being 1.77:1. Age of patients ranged from 13 – 75 years. Maximum patients were in the age group of 40-60 (37.3%). Quadriparesis (96%) was the most common presentation. Bulbar involvement was present in 56% of cases. Autonomic dysfunction in 7(9.3%) cases. 47(62.7%) cases were of AIDP variant. 17(22.7%) patients developed complications. Patients were treated with either IVIG or Plasmapheresis. Mortality was 5.3%. Higher number of cases were found in the S2 group (Summer) with a total of 42(56%) patients. Diarrhoea (15, 44.1%) was the most common prodrome among them. Conclusion: In this study, peak clustering of cases was noted in the summer months. Most cases with preceding diarrhoea occurred in summer months. AIDP was the most common variant. Age, sex distribution, clinical profile were similar between the two groups.

NMP8/179: Atypical presentations of hypokalemic myopathy

Shreeja KB, Jayakumar M, Balasubramanian S, Narasimhan LR

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

Introduction: Hypokalemic paralysis usually presents as acute/subacute flaccid symmetric quadriparesis with preserved sensation. Atypical presentations are rare. Two cases with atypical presentations are discussed. Case 1: A 37 years old male presented with bilateral wrist drop (R> L) and difficulty in getting up from squatting for 2 days. Previous H/o acute flaccid quadriparesis with complete recovery. Family H/o episodes of flaccid quadriparesis in patient's brother. Examination was normal except for BP 200/120 mmHg, bilateral wrist drop (Right > left) and Bilateral hip flexion 4.Investigations were normal except for hypokalemia;Serum CPK > 2000 U/L. NCS normal except for non stimulatable right radial nerve. In the background of Systemic hypertension; metabolic alkalosis; hypokalemia with normal aldosterone and renin levels, a diagnosis of hypokalemic myopathy with probable Liddle syndrome was considered. Case vignette 2: A 31 years old male presented with subacute flaccid quadriparesis of 15 days duration. Examination was normal except for bilateral LMN facial palsy, hypotonia with generalized diminished reflexes, quadriparesis and neck muscle weakness. Past H/o dimorphic anemia. Investigations were normal except for hypokalemia; hypocalcemia and compensated metabolic acidosis. NCS showed amplitude reduction in bilateral ulnar nerves and absent F waves in bilateral peroneal nerves. A diagnosis of hypokalemic myopathy with probable type 2 RTA was considered. Discussion: Atypical presentations in case 1 (predominant distal asymmetric weakness of upper limbs) and in case 2 (GBS like presentation) associated with electrophysiological changes cause diagnostic confusion.

NMP9/190: A rare case of statin associated ocular myopathy mimicking ocular myasthenia

Shaji CV, Prasanth SR

Department of Neurology, Government T D Medical College, Alappuzha, Kerala, India

Introduction: Statins are hypolipidaemic drugs.They inhibits conversion of HMG CoA to mevalonate. They inhibit the Rate limiting step in cholesterol synthesis. Statins are generally well tolerated. They are Indicated in Primary and secondary prevention of cerebrovascular and cardiovascular events. Muscle aches are the commonest adverse effect {10%}. Myopathy is the only serious adverse reaction {less than 1 in 1000}and Ocular muscle involvement is extremely rare. Ocular adverse effects include diplopia, ptosis, ophthalmoplegia. Average time from beginning of therapy to onset of symptoms was 8.3 months. Case Report: A 46 years old male, a Government employee Presented with bilateral ptosis since two weeks and diplopia since one week. Diplopia was more on looking to the left side with both eyes open and disappeared with one eye closed. There was no diurnal variation. Images were vertically separated. He was incidentally detected hypercholesterolemia on a routine medical visit and was on Atorvostatin 10 mg since last 8 months. Neurological examination-Restriction of left eye depression and intorsion suggestive of left superior oblique involvement with Bilateral ptosis. Investigations including Mri brain and orbit was unremarkable exept serum CPK level which was 220. Due to temporal association possibility of Statin associated ocular myopathy was considered. Drug was discontinued and there was Improvement in symptoms after one week and fully resolved by 3 weeks. Conclusion: Statin induced ocular myopathy should be considered in patients presenting with ocular symptoms when they are on statin therapy and it is a diagnosis of exclusion.

NMP10/209: A study on lmn facial palsy in a territory care center in Eastern Tamil Nadu

Santha Prabu Ramachandran, Thangaraj M

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

Introduction: Facial nerve palsy is common neurological disorder which is caused by Bell's palsy, infection, immune mediated, trauma, stroke, post operative injury etc. Bell's palsy is the appellation commonly used to describe an acute peripheral facial palsy of unknown cause. Materials and Methods: A sample of 75 cases of all age groups were studied in Neurology Department at Thanjavur Medical college from September 2018 to June 2019, Patients with facial paralysis secondary to trauma, post operative nerve injury, stroke were excluded from the study. Results: Of the 75 cases, 63 cases were diagnosed with idiopathic Bell's palsy, 8 cases with immune mediated, 2 cases with Herpes zoster otticus,1 case of recurrent facial palsy with ANA positive? Vasculitis and 1 case with bilateral facial weakness with HIV Male: 34 cases, Female: 41 cases, Right Side Palsy: 39 cases, Left Side Palsy: 36 cases, Risk factors include Diabetes mellitus: 18 cases, Diabetes with SHTN: 7 cases; winter season: 36 cases (Nov to Feb), summer season: 27 cases. Conclusion: In our study, bell's palsy was more commonly reported, paediatrics bells 6 cases were reported. One ANC case during third trimester was also seen. DM was found to have high association in the occurrence of Bell's palsy, about 40 % of cases were diabetic with higher incidence among males, 8 cases of BIlateral facial palsy were reported with immune mediated (GBS). 1 case of recurrent R sided facial palsy with ANA strongly +ve, 1 case of B/L facial palsy with HIV+ve.

NMP11/233: Corticosteroid induced myopathy-An ultrasound assessment

Raj VT, Narasimhan LR, Shunmughasundaram K, Manikavasagam J

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

Introduction: Corticosteroid myopathy is most common drug induced myopathy. Steroidmyopathy consist of non inflammatory toxic myopathy affecting postural muscles more than non postural with lowerlimb more than upperlimb and proximal more than distal involvement. Objective: The purpose of this study is to assess clinical usefulness of muscle ultrasound for steroid induced changes of muscle mass and structure. Materials and Methods: Study participants are patients with steroid exposure for minimum duration of one month who are admitted in MIN. They are assessed before starting steroids and after 1 month of treatment. patients were clinically assessed for muscle bulk and strength in all 4 limbs. sonological assessment were done with quantitative USG for muscle thickness, echogenicity and ratio of muscle thickness to subcutanous fat thickness in four main muscle groups – deltoid, biceps, vastus lateralis, tibialis anterior. Conclusion: Our findings suggest thatquantitative ultrasound may be able to quantify skeletal muscle changes associated with chronic steroid use. Muscle echogenicity can be used to track progression of steroid myopathyovertime and may help to indicate early response to therapuetic intervention.

NMP12/248: Isolated orthopnea as a presenting symptom of myasthenia gravis

Human Prasad Sinha, Kumar P, Maskey D

Department of Neurology, NH MMI Narayana Multispecialty Hospital, Raipur, Chhattisgarh, India

A 60 year old known hypertensive gentleman presented to our pulmonologist with sudden onset difficulty breathing in supine position. He was investigated extensively from pulmonary and cardiology view point but definitive diagnosis could not be made. However, the real time ultrasonography (USG) of chest showed reduced excursion of right hemidiaphragm and absent excursion of left hemidiaphragm. He required BiPAP support at night to sleep. Hence, he was referred for Neurology opinion after 4 days of onset of orthopnea. On evaluation his Neostigmine test was positive. 3 Hz repetitive nerve stimulation test of trapezius muscle was positive for post synaptic neuromuscular junction disorder. Acetyl choline receptor binding antibody was found to be positive (10.35; range-0.05-0.5). On Contrast enhanced CT chest there was no evidence of thymic hyperplasia or thymoma. He was put on pyridostigmine and he started showing dramatic improvement from the first dose itself. He was also initiated on low dose oral Prednisolone which was increased to 1mg/kg dose after 1 week. He responded well to treatment and no longer required BiPAP ventilation. His limb muscle weakness and ptosis also improved completely within 1 week of treatment. Later on he was also initiated on Azathioprine as immunosuppressant for long term management. Our case suggests that in an usual case of isolated orthopnea where other work up is non diagnostic one should always rule out myasthenia gravis.

NMP13/249: Pseudoneurfibromatous enlargement of spinal nerves and roots in a patient with chronic inflammatory demyelinating polyradiculoneuropathy

Bhupender K Bajaj, Goyal H, Bano S, Sharma B

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, Dr. Ram Manohar Lohia Hospital, New Delhi, India

Introduction: Hypertrophy of the nerves leading to thickened palpable peripheral nerves is known in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). There are only a few reports of massive hypertrophy of spinal roots and nerves in patients with CIDP. We present the case of 50year old female with longstanding CIDP who was discovered to have diffuse neurofibroma like spinal nerves and roots enlargement, and review the literature on the subject. Case Summary: A 50 year old lady diagnosed as a case of CIDP for 16 years on the basis of clinical, electrophysiological and cerebrospinal fluid findings, reported persistent low backache, neurogenic claudication, progressive weakness and tiredness predominantly in legs. She had been on immunosuppressive therapy with history of relapses on tapering or stopping of steroids. Magnetic Resonance Imaging of the spine revealed massive enlargement of spinal nerves and roots mimicking multiple neurofibroma. Clinical and radiological correlation suggested hypertrophy of spinal nerves and roots as being part of spectrum of involvement of nervous system in CIDP. Conclusion: Spinal nerves and roots may have massive enlargement mimicking plexiform neurofibroma in patients with CIDP. It may present with symptoms and signs of spinal canal stenosis, persistent and/ or worsening weakness.

NMP14/250: A case series of Schwartz-Jampel syndrome and variants in children

Singanamalla Bhanudeep, Kesavan S, Banerjee A, Suthar R, Saini AG, Sankhyan N

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Introduction and Background: Schwartz-Jampel syndrome (SJS) is a rare autosomal recessive disorder characterized by a facial dysmorphism, skeletal deformities, muscle stiffness, contractures and myotonic discharges without waxing and waning. Objectives: To describe the clinical, electro-physiology and genetics of children with Schwartz-Jampel syndrome (SJS). Materials and Methods: This was a retrospective case review of children with SJS from 2015 to 2018. Data were extracted as per predesigned proforma. Diagnosis was made on clinical and electrophysiological features, and confirmed by genetic analysis. Results: During this period, 4 children (boys: 3) presented with SJS phenotype. The median age of the cohort was 72 months (range: 24-132) and the median age of symptom onset was 21 months (range: 12-36). All the children had typical facial features with blepharospasm, skeletal deformities and both clinical and electrical myotonia. Out of the 4 children, 3 had neuromyotonia and 1 had myotonia in electromyography. All the children had normal compound motor action potentials in nerve conduction studies. Elevated muscle enzymes was seen in 50% of the cases. Genetic analysis revealed compound heterozygous mutation in HSPG2 in one case and missense mutation in SCN4A gene in another and could not be performed in remaining two cases. Conclusions: Presence of herculean appearance, fixed facies and skeletal deformities points towards diagnosis of SJS. However sodium channel myotonia with myotonia permanens also closely mimics SJS whose recognition is important due to management implications.

NMP15/254: Case report: Guillian barre syndrome as a rare sequela of neuroparalytic snake bite

Tarang Shah, Suthar N, Solanki D, Amdani S

Department of Neurology, AMC MET Medical College, Sheth LG Hospital, Ahmadabad, Gujarat, India

Introduction: Though many antecedent events like infection & vaccination has been attributed to development of Guillian Barre Syndrome (GBS); in extremely few instances has it been preceded by snake bite. This is a report of such rare phenomenon. Case: A 40 years old male presented with chief complain of difficulty in moving all four limbs, with ascending progression over the last 24 hours. 11 days before patient was admitted for neuroparalytic snake bite with respiratory compromise, for which after 7 days of treatment (including Anti-Snake Venom & ventilatory support); he was discharged without any Focal Neurological Deficit. No previous history of upper respiratory or gastrointestinal infection in previous month. On examination general condition was good & vitals were normal. All Deep Tendon Reflexes were absent, Plantar was bilaterally absent, Tone was decreased in all 4 limbs and Power was 3/5 in bilateral upper limb & 2/5 in bilateral lower limb. No sensory & autonomic abnormality seen. No bladder or bowel abnormality found. CBC, RFTs & LFTs were within normal range. MRI Brain & Spine was unremarkable. Nerve Conduction Velocity showed symmetrical, demyelinating polyneuropathy in all four limbs compatible with Acute Inflammatory Demyelinating Polyneuropathy (AIDP). CSF Protein was 800mg/dl without any leucocyte. CSF culture was negative for any infection. Patient was given Intravenous Immunoglobulin (IVIG) after which he improved. Conclusions: In around 40% cases of GBS the precipitating cause remains idiopathic, hence establishing snake bite as a cause of GBS further enhances our understanding of this clinical entity.

NMP16/277: Charcot-marie: Tooth disease masquerading as myeloneuropathy

Swapan Gupta, Tater P, Khwaja GA

Department of Neurology, GB Pant Institute of Post Graduate Medical Education and Research, New Delhi, India

Introduction: Charcot-Marie Tooth disease (CMT) is a rare hereditary neuropathy presenting as slowly progressive distal predominant weakness of limbs with no or minimal sensory complaints and decreased or absent deep tendon reflexes. Case: A 17 year old boy presented with gradually progressive sensory and motor deficits for last six years. There were both positive and negative sensory symptoms with “cotton wool feel” and sensory ataxia. The sensory loss was so severe that he would sustain unnoticed injuries over both feet. There was weakness and wasting of both upper and lower limbs more distal than proximal. On examination, he had evidence of chronic neuropathic changest. All deep tendon reflexes were brisk except ankle jerks. Sensations were impaired to all modalities in glove and stocking distribution. Bilateral gaze evoked nystagmus was present. With the clinical diagnosis of “myeloneuropathy”, extensive investigations did not reveal any etiology. Nerve conduction studies revealed severe axonal sensory motor polyneuropathy. Screening for asymptomatic family members revealed positive findings in his father. Nerve biopsy shows marked loss of large diameter nerve fibres with marked schwann cell hyperplasia and prominent onion bulb formation. Sample for genetic analysis and confirmation of CMT disease has been sent, but the clinical profile and nerve biopsy is suggestive of CMT- V. Conclusion: Prominent sensory as well as pyramidal signs can be present in CMT-V. Clinical presentation of myeloneuropathy with positive family history usually suggest adrenomyeloneuropathy but rarely can be CMT.

NMP17/281: Sjogren ganglionopathy: A case report

Mandar Waghralkar, Jukkarwala A

Department of Neurology, Geetanjali Medical College and Hospital, Udaipur, Rajasthan, India

Background: The most common presenting symptoms of Sjögren's syndrome (SS) are dry eyes, dry mouth, joint pain and fatigue. However, dorsal column involvement, reported as the most uncommon neuronopathy/ganglionopathy in SS, was the presenting manifestation of SS in this case. As such SS should be considered as differential diagnosis in patients presenting with sensory ataxia. Case Presentation: A middle-aged male presented with gradually progressive asymmetrical bilateral lower limb sensory symptoms and unsteadiness without any motor weakness with graded sensory loss of 18 years duration. He also had erectile dysfunction and sensory symptoms over face since last 10 years. Clinical diagnosis of sensory ganglionopathy was made. On follow up, patient had developed progressive symptoms of dry eyes & mouth since last 6 months. Although his antinuclear antibodies (ANA) done previously were negative, Schirmer's test came out to be strongly positive with subsequent sural nerve and labial biopsy favored Sjögren's syndrome with sensory ganglionopathy. Results: Patient was treated with intravenous methylprednisolone followed by oral prednisolone and azathioprine with significant improvement in his sensory symptoms and ataxia. Conclusion: Dorsal column involvement can precede the diagnosis of primary Sjögren's syndrome. As such Sjögren's syndrome or other connective tissue diseases should be considered in patients presenting with sensory ataxia. Appropriate immunosuppressive therapy can improve symptoms and function in these patients.

NMP18/298: Profile of recurrence of bell's palsy and hemifacial spasm in Idiopathic facial paralysis

Kamala Kant Bhoi, Chhattani L, Jain S, Sahu S

Department of Neurology, Shri Balaji Institute of Medical Science Pvt. Ltd., Raipur, Chhattisgarh, India

Fifty patients of Bells palsy were studied retrospectively for the recurrence rate and long term complication of hemifacial spasm. Recurrence is observed in 8 patients, with one patient having recurrence fourth time. Hemifacial spasm is observed in 5 patients (10%). Recurrence was more in patients female and during pregnancy and in patients having autoimmune disease as sarcoidosis, Scleroderma and Malkerson Rosenthal syndrome. The interesting cases will be discussed and brief emphasis on hemi facial spasm as a long term complication.

NMP19/304: Congenital muscular dystrophy-muscle eye brain disease: A case report

Velusamy Subramanian, Balaraman K, Ravi LA

Department of Neurology, Stanley Medical College, Chennai, Tamil Nadu, India

Congenital muscular dystrophies (CMD) are autosomal recessive disorders with incidence of 1:21500. A distinct feature of CMD from that of other muscular dystrophies is high association with brain malformations. Some subtype of CMDs like Muscle Eye Brain disease (MEBD) is also associated with eye abnormality. We report one such case. Case Report: 9 month old girl born for consanguineous parents presented with global developmental delay. Birth history was normal. Family history was negative. On examination, baby had microcephaly and mild dysmorphic facies. Baby was unable to fixate on objects. Fundus revealed mild disc pallor. There was hypotonia, diminished DTRs and paucity of movements in limbs. TORCH screening was negative. Serum CPK was high. MRI revealed bilateral pachygyria and white matter hyperintensity in frontoparietal region, hypoplasia of pons and cerebellum and multiple cysts in cerebellum all features suggestive of CMD. Targeted exome sequencing revealed mutation in POMGNT 1 gene consistent with CMD-MEB disease. Discussion: Major types of CMD are dystroglycanopathies, merosinopathy and collagenopathies. Subtypes in dystroglycanopathies are Fukuyama CMD, MEBD, Walker Warburg Syndrome (WES). Eye manifestations like cataract, retinal abnormalities are common in dystroglycanopathies. In merosinopathies, upgaze abnormalities are seen. White matter abnormalities are seen in FCMD and Merosinopathy, but pachygyria, cerbellar cysts, brainstem hypoplasia occur in MEB and are more severe in WWS. Conclusion: The case is reported for its rarity. In a child with global developmental delay and eye abnormalities, one should also consider this entity. MRI-Cortical malformations, hypoplasia of pons, cerebellum with cerebellar cyst are typical for CMD. Brainstem hypoplasia and cerebellar cysts are features of MEB and WWS.

NMP20/313: Plexiform neurofibroma: A case report

Justin Cornelius, Pandian G, Rajapandian S, Iyyadurai A

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

Introduction: Plexiform neurofibromas represent an uncommon variant(30%) of Neurofibromatosis type 1 in which neurofibromas arise from multiple nerves as bulging and deforming masses involving also connective tissue and skin folds. Case Report: We report a case of 6 years old male child, who presented with progressive swelling and weakness of right upper limb since 2 years of age, which is also painful on touching. Then he developed weakness of right lower limb since 3 years of age. History of hyperpigmentation over right half of trunk since birth. On examination, he was conscious with normal higher mental functions. Iris lisch nodules seen. Skin examination revealed multiple café au lait macules over the trunk and limbs. Giant melnocytic nevi with hypertrichosis on right upper half of the trunk and right upper limb hypertrophy with bag of worms feeling were noted. His motor examination revealed right hemiparesis with wasting of right chest and shoulder girdle muscles with winging of scapula. Three diagnostic criteria for NF-1(plexiform) were met, genetic testing was not done.MRI reports revealed plexiform neurofibroma involving right C3 to T1 nerve roots extending along the branches of brachial plexus. Intradural dumbbell components were noted at C4,C5,C6 causing compression of cord. The cervical and upper dorsal cord showed linear intramedullary syringomyelia. Conclusion: Diagnosis of plexiform neurofibroma is usually made clinically, especially if clinical hallmarks of NF-1 are present.Therapy is surgical,aiming at resecting deforming masses and cancerous tissue when malignant transformation occurs.

NMP21/337: Juvenile sporadic amyotrophic lateral scerosis presenting with flexor spasms

Mohammed Shujauzzaman Bilal, Jawalkar S, Manorenj S

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

Introduction: The initial presentation can vary between patients;some present with spinal-onset disease but other patients can present with bulbar-onset disease. Case Report: A 20 yr old female with Chief complaints of: Flexor posturing of bilateral upper and lower limb since 4 days. Was apparently well 12 months ago when she had an insiduos onset of weakness of left lower limb. The weakness slowly progressed and went on to involve the left upper limb then right lower limb and then right upper limb. She also developed difficulty in swallowing and change in voice over time. She also had 2 episodes of fall during the 12 month period. No family history of such disorder reported. Physical Examination: Higher mental functions normal. cranial nerves 7, 9, 10,11,12 affected by lower motor neuron paralysis. fundus normal. motor system examintaion revealed generalised wasting distal more than proximal,spaticity in all four limbs, spastic qudriparesis. sensory examination normal Deep Tendon Reflexes exaggerated. bilateral babinski sign positive and patellar and ankle clonus present. single breath count found to be 12. Investigations: needle EMG showed fascicultions and reduced recruitment. MRI imaging of the spinal cord and brain normal. Concluding Remarks: 'Juvenile ALS' refers to those with symptom onset consistently before age 25 years, typically in association with positive family history and slow progression. Bulbar-onset is less common in young-onset Amyotrophic lateral sclerosis.

NMP22/344: Psychosocial care for muscular dystrophy: The needs and interventions

Priya Thomas, Warrier M, Arun S, Bhuvaneshwari B, Nalini A

Departments of Neurology and Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Introduction: Muscular Dystrophies constitute a group of genetic diseases with varying onset, course and prognosis. They are generally characterized by the irreversible progression & increasing disability. In the absence of disease modifying therapies, accurate diagnosis, symptomatic management and rehabilitation form the basis of management. Methods: The paper proposes a psychosocial care model for intervention based on the identified needs. A cross sectional descriptive study was performed among thirty patients who attended the monthly neuromuscular clinic at a national tertiary referral centre for Neurological disorders. All the patients were diagnosed as Muscular Dystrophy based on clinical evaluation, confirmed by genetic analysis or muscle Immunohistochemistry. The diagnosis groups included Duchenne Muscular Dystrophy, Limb Girdle Muscular Dystrophy and other forms. Functionality, Needs and caregiver burden were assessed with standardized scales and in-depth interview. Results: Mean age of patients was 21.6 with 73% of them being male. There was significant variation in the felt needs, difficulties faced and support needed based on the type of and stage of illness. Information needs and genetic nature of the illness emerged as an important concern. Support for living with the genetic condition, reproductive decision making, family care and rehabilitation were reported as significant needs. The theoretical framework and model from a psychosocial care perspective is discussed. Conclusion: Psychosocial care initiated at the time of diagnosis focusing on development of self management skills would help patients and their families. The model presented has to be individualized and need to take the epochs in disease transition into consideration.

NMP23/347: Miller fisher syndrome with MRI findings

Mohammed Shujauzzaman Bilal, Jawalkar S, Manorenj S

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

A 38 year old right handed individual, admitted with acute onset diplopia and ataxia since 2 days preceeded by an episode of diarrhea. Extraocular movements testing showed Bilateral abduction and adduction paresis with nystagmus. Nystagmus was more prominant in horizontal gaze than vertical with fast component in the direction of gaze. Pupils were normal. Fundus was Normal. Rest of the cranial nerve examination was normal. Laboratory data was unremarkable. Nerve conduction studies showed demyelinating type of polyneuropathy with delayed F wave response. CT brain was normal. CSF analysis was normal. MRI brain showed a T2/FLAIR hyperintense lesion in the left cerebellar peduncle. Diagnosis of, Guillain-Barre Syndrome. Miller Fisher syndrome. Brighton criteria level 2 diagnostic certainty. was made and he was conservatively managed as per his informed decision. He was discharged after one week with no Diplopia and minimal vertigo.

NMP24/377: Mitochondrial cytopathy or congenital myasthenia: A dilemma

Surekha Dabla, Bala K

Department of Neurology, Pt BD Sharma Postgraduate Institute of Medical Sciences, Rohtak, Haryana, India

A 25 yrs old married female came with weakness of all four limbs with ptosis and diplopia which is there from last 20 yrs, recently it has been increased .o positive family history. Her CPK total is 431 IU, normal EMG ut RNST in Ocular muscles was strongly positive without any eviedece of other muscle weakness. She improved remarkably with pyridostigmine.

NMP25/419: Hyperacute thyroid myopathy: An unusual cause of metabolic dysfunction

Arun Kumar Singh, Malhotra HS

Department of Neurology, King George's Medical University, Lucknow, Uttar Pradesh, India

Introduction: Thyroid Myopathy is a well known entity in Hypothyroidism but hyperacute presentation is rare. Case History: We present here a case of a 15 year male with no prior history of Hypothyoidism presenting with Hyperacute onset of difficulty in getting up from sitting position since one day. On Examination: Vitals stable, GCS 15/15, cranial nerves were normal, motor examination bilateral upper limb was normal, bilateral lower limb had hypotonia with weakness of proximal muscles with diminished reflexes and intact sensory examination. Lab investigations TSH was elevated, CPK was slightly raised, Anti TPO/TG was elevated, Vit D was elevated, Potassium and Calcium were normal. USG Thyroid shows heterogenous enlargement of bilateral thyroid lobes, Ncv/Emg was normal. Patient was started on thyroid replacement and proximal weakness improved dramatically after about two months. Conclusion: This is being presented in view of rarity of hyperacte onest of proximal muscles weakness as presenting symptom of undiagnosed florid hypothyroidism.

NMP26/453: Hypokalemic respiratory paralysis as a presenting feature of sjgrens syndrome associated distal renal tubular acidosis: A report of 2 cases

Pradeep Kumar Maurya, Singh AK, Qavi A

Department of Neurology, Dr. RML Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Introduction: Hypokalemic periodic paralysis has diverse etiologies. It can be idiopathic but secondary causes in half of cases. Sjögren's syndrome is an autoimmune disorder of exocrine glands; however v extra glandular manifestations may occur. We report 2 cases of hypokalemic respiratory paralysis due to Sjögren's syndrome associated renal tubular acidosis. Case1. A 54 -years-old female presented with rapidly progressive weakness of all 4 limbs and respiratory distress since 2 days. Examination showed areflexic quadriparesis. She had hypokalemia (S. K+ 2 mmol/L) with biochemical profile suggesting distal renal tubular acidosis. She was diagnosed as Sjögren's syndrome as her anti Ro antibody and anti La antibody came significantly positive. After 2 days of ventilation she improved with potassium and alkali supplementation. Case2. A 60 -years-old female presented with weakness of all 4 limbs with carpopedal spasm and dyspnoea since 3 days. She had history of weakness with dry eye and joint pain. Examination revealed areflexic quadriparesis. Her S.K+ was 1.90 mmol/L and metabolic analysis suggestive of distal renal tubular acidosis. Schirmer's test was positive. Her anti Ro antibody and anti La antibody came strongly positive. She was diagnosed as Sjögren's syndrome and improved with ventilation (3 days), potassium, alkali and steroid therapy. Conclusion: Our cases highlight the importance of searching for underlying cause of renal tubular acidosis. Severe hypokalemia with metabolic acidosis prompted us to search for inherent cause. Sjögren's syndrome is a rare cause of renal tubular acidosis and needs to be considered in appropriate clinical setting.

NMP27/465: Statin-induced necrotising myositis: A case series

Sritheja Reddy Kambalapally

Department of Neurology, KIMS, Secunderabad, Telangana, India

Statins cause a wide spectrum of muscular adverse effects ranging from asymptomatic elevation of creatine Kinase, myalgias and exercise intolerance to rhabdomyolysis. Statin-induced necrotising myositis is increasingly recognised as part of statin induced myopathy spectrum, characterized by proximal muscle weakness with marked elevation of serum creatine kinase. 6 patients with severe muscle pains and weakness presenting to the hospital have been investigated and found to have myositis, rhabdomyolysis and recent history of statin administration. Patients were diagnosed to have statin induced necrotising myositis. Out of the 6 patients, 4 patients had a raise in liver enzymes and 3 patients had rhabdomyolysis. All the 6 patients showed similar EMG pattern in the form of increased insertional activity, runs of positive sharps and fibrillations. All the 6 patients had frequent pseudomyotonic discharges. Patients with renal failure required renal replacement therapy and were treated with intravenous steroids. Of the 6 patients, two patients expired, the remaining patients recovered and are on regular follow-up.

NMP28/492: A rare case of kern: Sayre syndrome

Sasank Gupta, Bhoi KK, Kumar S, Chhattani L, Jaiswal D

Department of Neurology, Shri Balaji Institute of Medical Science Pvt. Ltd., Raipur, Chhattisgarh, India

A 24 yr old male, presented with blindness since childhood, ptosis, ataxia since six months and chest pain since one month. He has hypothyroidism, diabetes, mentally retarded, short stature and cerebellar ataxia. ECG showed RBBB and prolonged IV conduction time, Echo: ischaemic cardiomyopathy. Deranged RFT and Increased creatinine phospokinase level, Fundus: showed atypical pigmentary retinopathy. And presence of myopathic changes on EMG. Muscle biopsy and genetics test suggestive of mitochondrial disorder. He diagnosed as a rare case of mitochondrial disorder: Kern-Sayre Syndrome.

NMP29/497: Sensory neuronopathy patients presenting in a Tertiary Care Hospital in central India

Surendra Kumar, Bhoi K

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

Background: Sensory neuronopathy is a rare condition associated with AIDP, CIDP, autoimmune conditions, paraneoplastic conditions. Materials and Methods: Patients who had diagnosis of sensory neuronopathy at AIIMS raipur for last one year were taken. Results: There were 3 patients. Two patients had sjogren syndrome, one of which also had hypothyroidism and dyslipidemia. The third case was of gullain Barre syndrome in a 70 year old gentleman which mimicked paraneoplastic condition but subsequently turned out to be gullain Barre syndrome. Sjogren syndrome patients were treated with steroids along with mycophenolate mofetil which improved her symptoms. The third case was treated conservatively. Conclusions: Sensory neuronopathy is a rare condition. Diagnosis of the condition and it's etiology helps in good recovery.

CNP2/445: Assessment of the role of continuous electroencephalographic monitoring in critically Ill patients with CNS infection (Prospective Study)

Pravallika Dutta, Manjeera K, Jabeen SA, Sailaja S, Tandra S

Department of Neurology, Nizams Institute of Medical Sciences, Hyderabad, Telangana, India

Introduction: Seizures, both clinical and subclinical are associated with poor outcome in acute CNS infections. Continuous electroencephalographic monitoring (cEEG) monitoring can detect electrographic seizures (ESz) including non convulsive status (NCSE) which could be the reason for unexplained decreased level of consciousness in these patients. Aims and objective: 1)To determine the prevalence of EEG abnormalities and electrographic seizures in critically ill patients with primary CNS infection 2)To study the correlation between electrographic abnormalities and patient outcome. Results: A total of 41 patients who fulfilled the inclusion criteria were included between November 2017 to October 2018. The mean age was 30.42yrs (range, 13-85). Infections were diagnosed as tubercular in 18(45%), viral in 16(40%), bacterial in 3(7.5%), parasitic in 2(5%) and fungal in 1(2.5%). The median duration of cEEG was 12 hrs (range 12-40hrs). Twenty patients (50 %) had major EEG abnormalities. This includes ESz in 5(12.5%), periodic discharges (PDs) in 12 (30%) and background suppression in 3 patients (7.5%). On univariate analysis a higher mean age of presentation [36.84 (+21.613), p= 0.032], presence of clinical seizures prior to EEG [14 (35%), p=0.003] and stupor or coma prior to EEG [17(42.5%), p=0.04] were associated with major EEG abnormality. There was no association between outcome (GCS at discharge) and major EEG findings (p value=0.55). Conclusion: In this study 50 percent of patients with CNS infections showed major EEG abnormality in the form of ESz, PDs and background suppression. History of seizures and unexplained sensorium should prompt for an early cEEG monitoring.

NGP1/201: A case series of Schwartz-Jampel syndrome and its variants in children

Singanamalla Bhanudeep, Suthar R, Sherawat IK, Padmanabha H, Kesavan S, Banerjee A, Saini AG, Sahu JK, Sankhyan N

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

Introduction and Background: Schwartz-Jampel syndrome (SJS) is a rare autosomal recessive disorder characterized by a combination of facial dysmorphism and skeletal deformities, muscle stiffness and contractures and myotonic discharges without waxing and waning. Objectives: To describe the clinical and electro-physiological features and mutation analysis of children with Schwartz-Jampel syndrome (SJS). Materials and Methods: This was a retrospective case review of children with SJS from 2015 to 2018. Diagnosis was made on clinical and electrophysiological features, and confirmed by genetic analysis. Results: During this period, 4 children (boys: 3) presented with SJS. The median age of the cohort was 72 months (range: 24-132) and the median age of symptom onset was 21 months (range: 12-36). Case 1: A 7-year-old boy, presented with stiffness of both upper and lower limbs, and frequent falls since 2 years of age. On examination, he had blepharospasm with narrow palpebral aperture with herculean appearance with proximal predominant weakness. Electromyography (EMG) showed characteristic dive-bomber sound consistent with myotonia. Skeletal survey showed lumbar scoliosis, and fracture of humerus. Next generation sequencing (NGS) revealed a compound heterozygous mutation in HSPG2 gene; c.1035 G > A, p. Arg 3452g at exon 75 and an unreported mutation c.1356-10 G > A at intron 11. Parents were both asymptomatic heterozygous carriers of the mutation. Case 2: A 2 yr old boy presented with daily facial twitching during awake and sleep for last 6 months and stiffness of the body for past 4 months. On examination he has facial myokymia along with typical facial features as described in first case. EMG showed abnormal decrementing repetitive discharges with pinging sound suggestive of neuromyotonia. Skeletal survey showed widening of long bone metaphyseal ends and hip dysplasia. NGS revealed a heterozygous missense variation in exon 6 of SCN4A gene(c.952T>T/C). Asymptomatic parents were not tested. Case 3: A 11 yr old girl presented with complaints of difficulty in opening of eyes since 1 yr of age and motor development delay since late infancy. EMG showed non-neuropathic pattern and myotonia with no waxing and waning. Skeletal survey revealed deformities of sternum, ribs and spine. Genetic analysis could not be done. Case 4: A 5 yr old boy presented with complaints of stiffness of the body since 3 yrs of age leading to recurrent falls. EMG showed myotonia with no waxing and waning. Skeletal survey revealed metaphyseal widening and misshapen capital femoral epiphysis. Genetic analysis could not be done. Conclusions: Although the presence of herculean appearance, fixed facies and skeletal deformities pointed towards SJS, variants of SJS like sodium channel myotonia and severe forms of myotonia congenital may present with similar clinical picture. Limitations: Despite the clinical, radiologic and neurophysiologic picture are similar to SJS, genetic analysis could not be performed in two cases. Further Scopes: The recognition of variants of SJS has treatment implications as cautious use of potassium supplements, exercise induced hypoventilation and risk of malignant hyperthermia.

NGP2/290: Utility and yield of genetic testing in neurology patients- from department of neurology of a Tertiary Care Hospital

Piyush Kumar Anshu, Ravat S, Jain N

Department of Neurology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, Maharashtra, India

Background: Since last few years genetic testing has become easily available in India, though the high cost is still the major prohibitive reason, especially in hospitals which caters to lower socio economic strata. We have been doing these in highly selective population where genetic basis is strongly suspected which helps in management and prognostication. Till date, several studies have addressed the utility of next-generation sequencing methods, reporting diagnostic yields of approximately 25%. Objective: To evaluate 1. Overall yield of molecular diagnostic methods in neurological diseases 2. Utility of Clinical Exome Sequencing (CES) in neurological disorders with a genetic background, 3. Impact of CES on management of Cases. Methodology: Retrospective analysis of all patients who were subjected to CES between 2016 and 2019 at neurology department of KEM hospital was done. It was done in patient in whom, 1. Clinical picture was highly suggestive of genetic etiology, 2. Genetic study is likely to change management, 3. Patient who afforded the test CES data was generated at standard genetic laboratory. Results: Exome data from 74 adult and child neurological patients were analysed. Likely or definite causative variants were found in 38 individuals, achieving an overall diagnostic rate of 51%. Molecular diagnoses could be established in 25/39 (64%) patients with neuromuscular disease, 4/9 (44%) with movement disorders, 3/6 (50%) with suspected neurometabolic conditions and 6/20 (30%) with epileptic disorders. In 8 patients the management changed in terms of specific drugs and diet. We also identified some genetic defects which were not reported from India. Conclusion: Yield of genetics was high in our study as we had done in highly selective population, selected after thorough clinical examination and discussion. Confirmation of diagnosis by genetics paves way to genetic counselling, avoids invasive diagnostic tests and further economic burden to the family and helps in management of disease. And in few cases outcome has improved to great extent.

NGP3/489: Lafora body disease: A form of progressive myoclonic epilepsy with a novel mutation in EPM2A gene

Jignesh Prajapati, Sowani A, Prajapati A, Patel B

Department of Neurology, Zydus Hospital, Ahmedabad, Gujarat, India

Introduction: Lafora body disease is a fatal, autosomal recessive, glycogen storage disease caused by mutations in one of two genes, Epilepsy, Progressive Myoclonus 2A EPM2A or Epilepsy, Progressive Myoclonus 2B (EPM2B). Case Summary: 12 years old developed 1st seizure at the age of 10 years with generalized discharge in EEG, diagnosed as primary generalized epilepsy and seizure was controlled on one AED for 2.5 years. Recently at the age of 13 years, she presented with, recurrent seizure including myoclonus progressively increasing and refractory to multiple AEDs with rapidly progressive cognitive decline and visual loss, memory loss and speech regression, where EEG showed multifocal, generalized spike and wave discharges with slowed background with normal MRI brain and VEP. In view of typical presentation, EEG findings, progressive myoclonic epilepsy was suspected and genetic analysis was sent which showed a novel homozygous variant of uncertain significance in EPM2A gene (c.4_39del(p.Arg2_Val13del) asoociated with Lafora body disease, to correlate and confirm the disease ,skin biopsy was sent which showed polyglucosan bodies. Discussion: Here we describe a case of a progressive myoclonic epilepsy with a novel homozygous variant in EPM2A gene which was uncertain significance however damaging by multiple predicted system and presence of polyglucosan bodies in skin biopsy. Conclusion: Lafora Disease manifests during adolescence with multiple types of seizures which progresses with dementia, apraxia, aphasia and visual loss, eventually leading to patients in a vegetative state and death, within a decade of initial seizure. Here novel variant increases mutation spectrum of the disease.

NGP4/491: Glycine encephalopathy - neurologist puzzle and urologist nightmare: A case report

Devashish Ruikar, Chavan G, Bharti V, Patil S, Panchal D

Department of Neurology, MIMSR Medical College, Latur, Maharashtra, India

Background: 1.5 percentage glycine irrigation solution is used regularly during various urological surgeries including TURP. It is supposed to improve endoscopic fields. Generally the fluid remains locally and in the peri-prostatic region and systematically not absorbed.Sometimes from open Venus plexus and blood vessel the fluid get absorbed in blood stream and gets Detoxified without significant sideffetcts. Post TURP syndrome is known entity with relatively benign manifestation. Glycine is known inhibitory neurotramister. Case Report: We report a 62yrs Patient who underwent TURP for BPH done at a Urological Center under spinal anaesthesia with Glycine irrigation as standard protocol. Two hours after the procedure patient had complaints of progressive giddiness followed by diplopia followed by dysathria then he became stuporous followed by respiratory distress which required Ventilator support. Post TURP within 6hrs patient became Comatose with GCS 3 and on Ventilator with no respiratory efforts. On supportive care he recovered after 36hrs and became conscious and coherent after 48hrs. Discussion: Glycine induced Encephalopathy is very rare entity and there are hardly any reported cases from India. Giddiness, Drowsiness, Headache, Hallucination etc. are reported as a post TURP syndrome. However Coma requiring ventilatory support is highly unusual as a part of Glycine Encephalopathy. Some patients have rare genetic deficiency (AR) to clear Glycine, who are prone to develop this syndrome. Glycine irrigation is used regularly in Urological surgeries. We report this case so that urology and neurological colleagues to be aware of this rare but potential lifethreating complication of routinely used irrigation fluid.

IP2/64: Etiological spectrum of febrile encephalopathy: A glimpse from a tertiary referral centre of north east India

Synmon B, Sharma SR, Hussain M

Department of Neurology, North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences, Shillong, Meghalaya, India

Introduction: Fever with altered sensorium is a common medical emergency. Its varied clinical presentation and wide range of aetiology make it a complex spectrum. Complete recovery depend on how the aetiology is promptly evaluated and managed. Aims of Study: we aim to analyse the different aetiology of febrile encephalopathy in this region. Materials and Methods: A prospective observational study was carried out for 6 months from November 2018-April 2019. All patients with features of febrile encephalopathy was enrolled irrespective of the duration. Routine investigation, cerebrospinal-fluid analysis, neuroimaging and other relevant investigation were done. Data was then analysed and interpreted. Results: 52 patient was enrolled, with age range of 12-79, mean 34 years. Twenty-four females and 28 males were included. Duration of illness varied from 2-90 days, acute in 35 patients and chronic in 17 patients. The most common symptom was fever (100%) and headache (98%). Focal neurological deficit was seen in 17 patients (32%). Tuberculosis was the most common aetiology noted in 34 (65%) acute presentation in 23 (67%) and chronic in 11 (32%) patients. Other aetiology was bacterial meningitis in 1(1.9%), septic encephalopathy in 2 (3.8%), HSV in 1(1.9%), Japanese encephalitis in 1(1.9%), viral in 4 (7,6%), cryptococcal meningitis in 3(5.7%) and non-infective cause like NMDA encephalitis in 2 (3.8%), CVT in 3(5.7%) and Sheehan syndrome (1.9%) in one patient. Conclusion: Tuberculosis being the commonest aetiology of febrile encephalopathy in this region of India calls a need for further research.

IP3/80: Epidemiology and clinical profile of neurological manifestations of dengue fever during an outbreak: Observations from a Tertiary Care Hospital

Cherukudal S, Nampoothiri V

Department of Neurology, Aster MIMS Hospital, Kannur, Kerala, India

Dengue fever is one of the most common arboviral infections in the world. Dengue virus is classically considered a nonneurotropic virus, but neurological manifestations can occur either as a direct effect of the virus or immune reactions. All patients admitted with the diagnosis of dengue fever in our hospital over a period of 4 months from May 1st 2018 to August 31st 2018 were studied. of 196 patients 35 had neurological manifestations. The most common neurological manifestation was aseptic meningitis in 20 patients. The other manifestations were dengue encephalitis, encephalopathy, cerebellitis, transverse myelitis, Guillain barre syndrome (GBS) and brachial plexitis. The 2 patients with GBS had ataxia and areflexia variant one of whom recovered with intravenous immunoglobulin within 5 days. The other patient with GBS had a prolonged course requiring a second course of immunoglobulin. One patient with encephalitis had a “Boomerang sign” in MRI Brain. Some patients had associated atypical manifestations like urinoma. It is important to recognise dengue and its atypical manifestations. The type of GBS might point a clue to molecular mimicry and immunological manifestations after dengue infection. Dengue fever diagnosis and interventions to prevent its spread are of great public health importance in South East Asian countries.


/90: Guillain barre syndrome: A rare complication of scrub typhus

Manchanda R, Kumar N, Divya M, Manchanda RS

Department of Neurology, All India Institute of Medical Science, Rishikesh, Uttarakhand, India

Background: Scrub typhus is a common infective disorder in the Northern part of India but its association with GBS has rarely been reported in the literature. Although respiratory involvement has been reported in 10- 30% of GBS patients, none of the scrub typhus related cases developed respiratory involvement. Mortality in GBS cases requiring respiratory support can be as high as 20%. Objective: To report a series of cases with Scrub typhus related GBS from a tertiary care centre in the Himalayan belt of India. Materials and Methods: Cases of acute flaccid paralysis fulfilling the Asbury criteria for GBS including clinical picture, CSF analysis and electrodiagnostic studies and presenting with concurrent history of fever were screened for scrub typhus infection. The diagnosis of scrub typhus was made in case with acute febrile illness with/without typical eschar on skin along with positive scrub IgM ELISA. Results: We report a series of three cases of scrub typhus related GBS. All were female in the age group of 40-56 years with a mean age of 45.6±8.9. All cases had acute areflexic flaccid quadriparesis, cranial nerve involvement along with albumino-cytological dissociation in CSF. While two of them had demyelinating pattern on nerve conduction studies, third had axonal involvement. Two of them had isolated 7th cranial nerve palsy, while third had multiple cranial neuropathy involving 5th, 7th, 9th and 10th cranial nerve. All three cases developed respiratory involvement requiring ventilatory support. We gave IVIG (2gm/kg over 5 days) in all three cases with one of them requiring a repeat cycle of IVIG. Before coming to us one of our cases received two sessions of plasma exchange without any improvement. All three required prolonged ventilatory support with a mean duration of 9.6±6.5 days. The time required for recovery from Hughes disability score grade 5 to 2 was 41±16.56 days. Conclusion: GBS like clinical picture in an acute febrile episode warrants testing for infections like scrub typhus especially in endemic region like ours. Such cases may progress to a severe form of illness requiring prolonged ventilatory support. They may benefit with longer duration of immunotherapy.

IP5/111: Tuberculous optochiasmatic arachnoiditis: A short series

Bhavana I, Hari Radhakrishna, Padhy BP

Department of Neurology, CARE Hospital, Hyderabad, Telangana, India

Introduction: Optochiasmatic arachnoiditis (OCA) is the one of the dreaded complications of TBM which presents with reduced vision and field defects. Case 1: 30 y lady k/c/o PTB came with fever & headache. She had b/l papilledema and neck rigidity. CSF s/o TBM, started on ATT & steroids. After 2 months she had reduced vision in both eyes. Acuity was 6/60 on right and 6/240 on left with RAPD and left hemianopia & b/l choroid tubercles. CECT brain showed OCA with tuberculomas and left CG infarct. She recieved 4 cycles of intrathecal Dexa with Hyaluronidase. Acuity improved to 6/12 on right & 6/36 on left. Case 2: 27 y lady came with fever, headache for 1 y. CSF s/o TBM & started on ATT. After 1 month, she had reduced vision in both eyes & left hemiplegia, so Ethambutol was stopped. O/E she had PL in both eyes with papilledema and MRI brain showed right thalamocapsular infarct. She recieved 4 cycles of intrathecal Dexamethasone with Hyaluronidase injections. After 15 months of ATT, her acuity is CF at 1 m on right & 6/240 on left. Discussion: In TB OCA, the basal exudate is most prominent in interpeduncular fossa which compress & occlude blood supply to optic nerve and chiasma. MRI brain contrast is preferred. Treatment of OCA is a challenge & the response unsatisfactory. ATT with steroids & intrathecal Dexamethasone with Hyaluronidase with Shunt surgery for hydrocephalus and microsurgical decompression of optic chiasma & optic nerves have been tried.

IP6/293: Progressive multifocal leukoencephalopathy: Immune reconstitution inflammatory syndrome in a patient with HIV: A case report

Chadalavada Kranthi Mohan, Velayutham SS, Sowmini PR, Jeyaraj M, Krishnan M, Arunan S

Department of Neurology, Government Stanley Medical College, Chennai, Tamil Nadu, India

Introduction: Progressive Multifocal Leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system occurring in the immunocompromised and it is caused by JC virus. The paradoxical clinical worsening of PML after initiating antiretroviral therapy (ART) therapy in HIV-infected patients resulting from restored immunity to JC virus is called as PML-immune reconstitution inflammatory syndrome (IRIS). Differentiating PML from PML-IRIS is crucial as the outcome and treatment strategies differ. We report a case of PML IRIS-in a HIV patient. Case Report: A 40 year old right handed male presented with subacute onset of right upper limb tremor initially followed by right lower limb of 2 months duration which was progressive, more during action, slurring of speech, unsteadiness of gait with swaying to right side. He had quit ART for 3 months and after1 month of restarting ART he noticed the above symptoms. He had scanning dysarthria and bilateral appendicular ataxia. MRI brain plain revealed T2 FLAIR hyperintensity in right cerebellum, middle cerebellar peduncle, left cerebellum without diffusion restriction, confined to white matter, with patchy irregular contrast uptake. The high CD4 count and low HIV RNA copies strengthened IRIS in PML lesions. We administered intra venous dexamethasone followed by oral prednisone starting for a period of 2 months along with HAART therapy. After 2 months Patient dysarthria improved, severity of tremor decreased and he walked independently without swaying. Conclusion: Recognition of PML IRIS has important therapeutic implications as corticosteroids may play a beneficial role in decreasing the neurological symptoms. PML-IRIS can be suspected in unexplained worsening of PML or development of new symptoms suggestive of PML in a HIV patient on ART. The contrast uptake in PML lesions inMRI serve as a surrogate marker for IRIS.

IP7/320: Lower limb monochorea as an unusual manifestation of motor cortex neurocysticercosis

Swapan Gupta, Bhattad S, Khwaja GA

Department of Neurology, GB Pant Institute of Post Graduate Medical Education and Research, New Delhi, India

Introduction: Neurocysticercosis (NCC) is rarely reported to present as movement disorder, chorea even rarer. Most are reported from basal ganglia lesions. We report a case of motor cortex neurocysticercosis with an unusual manifestation in the form of lower limb monochorea. Case Report: A 60 years hypertensive and diabetic female presented with sudden onset jerky movements of her right lower limb with preserved consciousness. There was no pain or abnormal sensation or weakness in the affected limb. The movements were continuous and persisted even during sleep. On examination, there were nonrhythmic, irregular, rapid, unsustained predominantly distal movements of the right lower limb suggestive of chorea. A possibility of stroke-related chorea or less likely metabolic chorea due to hyperglycaemia was entertained. However, her blood glucose levels, NCCT head and EEG were normal. She responded to haloperidol, and the movements subsided completely over next 3 days. Subsequent contrast MR imaging of the brain revealed a peripheral ring enhancing lesion with eccentric nodule in the left frontal parasaggital region suggestive of neurocysticercosis. The location of the lesion was consistent with the diagnosis of right lower limb chorea, as was the response to haloperidol. Conclusion: Monochorea as a manifestation of motor cortex neurocysticercosis is exceedingly rare. Hypothesis that the cortical lesions produce movement disorders through the modulation of the sensory motor circuit is suggested.

IP8/324: CNS tuberculosis masquerading as NMOSD

Kilani Aparna Swathi, Garuda BR, Seepana G, Talabhaktula SK, Upputuri AK

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

Introduction: The WHO estimated incidence of TB in India for year 2016 was 2.79 million. CNS TB accounts for 1% of all cases of TB. Pulmonary tuberculosis has been considered to present as NMOSD as the Mycobacterium tuberculosis, shares antigens with myelin basic protein. Direct infection of the central nervous system by TB may mimic NMO. It is important to differentiate CNS TB from NMOSD as misdiagnosis and treatment with immunosuppressants has serious repercussions. Case Report: 19 year old healthy adolescent presented with sudden onset of vomittings, hiccups, gait unsteadiness, blurred vision of both eyes with decreased colour perception, which progressed within 24 hours to ascending quadriplegia with urinary retention, urgency, along with nasal speech, dysphagia and nasal regurgitation. Investigations revealed ESR 25mm/hour, Chest X-ray and ultrasound abdomen normal, HIV- negative. MRI Brain and spine revealed LETM at cervical spine, demyelinating plaques in medulla, pons and midbrain. CSF analysis was normal with CSF ADA -0.5U/L except TB PCR which was positive. Discussion: Feng et al., described the benefits of anti-tuberculosis treatment in steroid-refractory Chinese NMO patients suggesting tuberculosis as etiology. Most reports suggest active pulmonary TB association. Cases of area prostrema syndrome, LETM due to TB have been described. The present case has no systemic features as fever and weight loss. The presentation was suggestive of NMOSD except for TB-PCR which clinched the diagnosis. Conclusion: NMOSD is not always a syndrome but a presentation where other possible etiologies as TB has been described. CNS TB if missed could lead to detrimental effects if immunosuppressants are used.

IP9/356: Disseminated tuberculosis of central nervous system: Rare case series of cranio-spinal tuberculomas

Kannan Vellaichamy, Venkatraman C, Samivel B, Ranganathan LN

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

Spinal tuberculosis, intramedullary tuberculoma (IMT) as a cause of spinal cord compression leading to paraparesis in association with central nervous system (CNS) involvement is rare. Authors report 5 cases presented with multiple CNS tuberculomas involving the spinal cord and the brain manifesting with corresponding neurological deficits. Magnetic resonance imaging for diagnosing IMT at an early stage can be very useful and is also useful in follow-up. IMT mostly responds to medical therapy though there are surgical options for selected patients. Here, we describe 4 patient with IMT and intracranial tuberculoma and 1 patient with cranial tuberculoma and epidural spinal involvement who improved clinically with antituberculous treatment, steroids and with surgery (when required).

IP10/368: A are case of dengue encephalitis

Rachel Packiyaseeli C, Vignesh Karunakaran

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

Dengue fever is the common disease which has a variable clinical spectrum ranging from asymptomatic to life threatening dengue hemorrhagic fever and dengue shock syndrome. However neurological complications in general are unusual. Dengue virus is classically considered as non-neurotrophic virus, neuronal infiltration and encephalitis due to dengue virus is very rare entity. We report an interesting case of dengue encephalitis with MRI brain abnormalities and its recovery during the follow up. A 14 year girl presented with acute onset of fever and altered sensorium with feeble peripheral pulses during epidemic session of dengue. Patient even though recovered from the shock but presented with one episode of convulsion, nystagmus, ataxia, finger nose incoordination, dysdiadokokinesia predominately on left side. Investigations shows leucopenia and thrombocytopenia. Her LDH also high. IgM dengue positive. MRI brain shows hyper intensity in T2 in thalamic region. Patient was on anti epileptics and physio therapy. This is a case presented to highlight the possible extensive involvement of the brain by dengue virus is rare manifestations of common also highlights the important fatal complications of the disease.

IP11/372: Fatal ameobic meningo-enchephalitis in immunocompetent host, due to eye drops: A case report

Ruikar Devashish, Darak G, Chavanda S, Kanade O, Ruikar P

Department of Neurology, Shree Neurocare Center, Latur, Maharashtra, India

We present a rare case of N.fowleri living amoeba meningitis in immunocompetent person. There was h/o patient used soil exposed eye drops for symptomatic relief for itching which was prescribed by some general practitioner long back. After using eye drops withing 3 days patient had fulminant course of disease and the patient expired within almost 4 days of onset of disease, diagnosis was made on basis of clinical signs of meningism, csf examination revealed neutrophilic predominance meningitis. We were able to make antemoratam diagnosis on light microscopy. Simple CSF wet mount light microscopy showed amoebic motility, PCR confirmation not done due to resource limited setting. N.fowleri infection appears to be quite rare compared to other diseases, the clinical manifestations of primary amoebic meningoencephalitis are devastating and nearly always fatal. Due to the rarity of N. fowleri infections in humans, there are no clinical trials to date that assess the efficacy of one treatment regimen over another. Most of the information regarding medication efficacy is based on either case reports or in vitro studies. This case is reported as Nagleria is rare and fatal cause of amoebic meningo encephalopathy there are very few case reports from India and almost all reported case are post mortom and after swimming in dirty water. There are hardly any case report with antemortam diagnosis Eye drops if not stored and used properly can lead to such a fatal infection. Simple test of CSF wet mount in light microscopy has diagnostic values in this rare infection.

IP12/376: Invasive breast aspergillosis presenting as wasted hand

Veenu Singla, Singh T, Goyal M, Bal A, Singh G

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

A 45 year female presented with history of pain in the medial aspect of right forearm and hand for the past 5 months, which was progressively worsening. The pain was intermittent, sharp, shooting in character initially and has progressed to severe burning paraesthesias. There was associated numbness of little and ring finger. For the past 1 month, the patient also noticed weakness of right hand grip along with thinning of the hand. Examination confirmed wasted hand with weakness of hand grip as well as intrinsic muscles of the hand. Possibility of brachial plexopathy secondary to malignancy was kept. Electrophysiological investigations revealed evidence of right brachial plexopathy in the form of reduced CMAPs and SNAPs in right Median and Ulnar nerve. Patient underwent CT and MRI of right brachial plexus, which revealed an infiltrating mass lesion, predominantly involving the right lower brachial plexus. Patient underwent Excision biopsy of the lesion, which revealed invasive aspergillosis causing brachial plexopathy. Patient underwent chemotherapy in the form of intravenous Amphotericin B along with symptomatic treatment, to which the patient responded well. This case highlights a rare manifestation of Aspergillosis presenting as Brachial plexopathy.

IP13/382: Abnormal movement, knuckle pigmentation and madness: Is it B12 deficiency?

Sanjeev Bhoi, Jha M, Naik S, Palo G

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

Background: Vitamin B12 deficiency neurological syndromes involves whole of neuraxis. The deficiency usually manifest with subclinical cognitive impairment, neuropathy to rarely sub-acute combined degeneration of cord and megaloblastic madness. Objective: Hyperkinetic movement disorder is rarely reported in adults with cobalamine deficiency state. Methods and Results: A 38-year-man presented with knuckle hyperpigmentation and behavioural abnormality of 8 months duration. He developed progressive neuro-psychiatry manifestations since 2 months. He had hyperkinetic movement in form of myoclonus probably of spinal origin (Video 1- pre treatment). Serum Vitamin B12 was 295 pg/ml, bone marrow showed megaloblastic erythropoiesis. Nerve conduction showed motor sensory axonal polyneuropathy, bilateral prolonged p100 latency (118 and 117 ms) absent cortical potential in tibial SEP. MRI spine showed increased T2 signal in posterior column of cervical and dorsal spinal cord. He was managed with 1000 μg vitamin B12 intramuscularly daily for 7 days followed by alternate day and then twice weekly. He showed clinical and laboratory parameters improvement and involuntary movements decreased (Video 2- post treatment). At 3 month followup he was normal and joined his duty. Conclusion: Abnormal hyperkinetic movement can occur in adult with cobalamine deficiency. Early diagnosis and supplementation of vitamin B12 reverses the symptoms.

IP14/406: Meningitis with vasculitis: Look beyond tuberculosis

Arun Seshu Vandanapu, Yada PK, Jayalakshmi SS

Department of Neurology, Krishna Institute of Medical Sciences, Karad, Maharashtra, India

Cryptococcal meningitis (CM), the most common manifestation of cryptococcosis among patients with impaired cell-mediated immunity, is caused by the encapsulated yeast organism Cryptococcus neoformans. Immunocompromising conditions that leave patients at risk of cryptococcal CNS infections include HIV infection, leukemia, post-transplantation immunosuppression, an organ failure syndrome, or innate immunologic problems, such as common variable immunodeficiency. Fungal infections of the CNS can manifest as meningitis, abscesses, granulomas, or infarction. Cryptococcal infection involving the CNS in non-immunocompromised patients is uncommon. To our knowledge, there have been only few reports of stroke in patients with CM. The usual diagnosis in patients having chronic meningitis with stroke is TB meningitis with vasculitis. Here we present a case series of 3 patients (2 immunocompetent, 1 immunocompromised) who presented meningitis with focal deficits, who were subsequently diagnosed to have Cryptococcal meningitis. Cryptoccal meningitis should be considered as a differential diagnosis in patients having meningitis with vasculitis even if they are immunocompetent especially if there is no clear evidence of TB. Missed Cryptococcal meningitis diagnosis can result in inappropriate management, affecting the patient's clinical outcome.

IP15/438: Varicella infection leading to intracranial venous thrombosis as an unusual presentation

Shubhada Khandare, Uniyal R, Garg R

Department of Neurology, King George's Medical University, Lucknow, Uttar Pradesh, India

This is interesting case report of Varicella induced cerebral venous thrombosis. chicken pox is benign disease of childhood, chicken pox related neurological complications are seen in less than 1% of cases. Most common known complications are ataxia, Meningoencephalitis, GBS and transverse myelitis. But there are very few reports of varicella induced cerebral venous thrombosis.This is 35 year old lady who presented with fever with rash which was centripetal in nature and on day 3rd of rash develop right sides weakness and right lower limb swelling, headache and vomitting. on day 7 she had 1 episode of generalised tonic clonic seizure. Her routine blood investigation including PT and aPTT was normal. CT suggestive of superior sagital sinus thrombosis and this finding was also confirmed on MR Venography. Her fundus showed papilledema. She was treated with anticoagulant , to which she responded very well. This showed that considering early possibility of cerebral venous thrmbosis and early treatment was life saving.

IP16/452: Comparison of regimens of dexamethasone in tubercular meningitis: A randomized control trial

Alvee Saluja, Vibha D, Prasad K, Srivastava A, Shukla G, Pandit AK, Rajan R, Dwivedi SN, Tripathi M

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

Introduction: The use of adjunctive corticosteroids may improve outcome by reducing the severity of neurological complications.Trials till date have not shown superiority of IV versus oral steroids. There has been no study which has compared between overlapping of IV with oral dexamethasone versus directly switching IV to oral dexamethasone. Methodology: Our study is a single center, single blinded, concealed allocation, randomized control trial conducted in 60 patients at AIIMS, New Delhi over 2 year period. Patients greater than 12 years of age with clinical features of TBM plus (any one) CSF or radiological or proven extraneural tuberculosis were included. Patients were randomized to two groups (direct switch versus overlapping of IV and oral dexamethasone) based on Thwaites' regimen.Patients with diagnosis other than tuberculosis or contraindications to steroids were excluded from the trial. Primary outcome measured was the resolution of clinical symptoms 1 month post randomization.Secondary outcomes like time to discharge, mortality at 6 months,mRS at 6 months were measured. Preliminary analysis is available for 42 patients. Clinical resolution was seen in 15 out of 21(71%) patients in direct switch arm which was similar to that overlapping arm (16/21 = 76%). Mean mRS and mortality at 6 months did no differ between the two groups. Mean time to discharge was 10 days (direct switch) versus 12.5 days(overlapping arm)(p=0.16). Conclusion: Direct switch of dexamethasone from iv to oral route leads to similar symptomatic resolution compared to slow overlap of iv and oral dexamethasone in TBM patients.

IP17/456: N-Methyl-D-Aspartate receptor antibody positive encephalitis in human immunodeficiency virus infected patients

Sumit Kharat, Ojha P, Soni G, Aglave V, Jagiasi K, Barvalia P

Department of Neurology, Grant Government Medical College and Sir J.J. Group of Hospitals, Mumbai, Maharashtra, India

We present 2 rare and interesting cases of N-Methyl- D-Aspartate receptor (NMDA-R) antibody positive encephalitis in Human Immunodeficiency Virus (HIV) infected patient. Only a few cases have been reported till now. Case 1: A 53-year-old male HIV patient with baseline CD4 1,113 on ART (Antiretroviral Therapy) presented to us with refractory facio-brachial seizures. The patient needed 6 different antiepileptic drugs for the control of Seizures. On investigations, CSF protein 79/dl; Glucose 66mg/dl; 7 cells all lymphocytes. CSF viral panel, MRI brain and work up for CNS opportunistic infections was done which was negative. On further workup, the patient's CSF was positive for NMDA-R antibody. The patient was subsequently treated with Intravenous immunoglobulins with the resolution of seizures. Case 2: A 36-year-old HIV infected woman on ART presented subacutely with altered sensorium, refractory seizures, and behavioral changes. Work up for CNS opportunistic infection and MRI Brain was normal. On further study, the patient's CSF was positive for NMDA-R antibody. The patient was initially treated with Intravenous immunoglobulins and later with Rituximab. However, the patient succumbed to the illness. Conclusion: HIV infected patients with introduction ART has now prolonged lifespan. There has been a relative decrease in opportunistic infections and an increase in the immunological manifestations. NMDA-R receptor encephalitis should be considered early in HIV patients with good CD4 count after ruling out infective etiology. Early diagnosis and management are crucial.

HP1/47: Rare cause of secondary headache in a young female: A case report

Alexander BK, Chandramouleeswaran V, Lakshminarasimhan R, Kannan V

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

Introduction: Headache is one of the most common neurological symptoms occuring worldwide. There are certain headache which is very severe and life endangering which can be cured and prevented. Here we present a unique case of reversible headache in a young female which will make us to consider the endemic factors as well as the red flags in headache while encountering such cases. Case Report: A young female who presented with severe unilateral left sided intractable headache associated with nausea and vomiting for 1 month duration. On clinical examination found to have bilateral papilledema without any focal neurological deficit and brain imaging showed 4th ventricular neurocysticercosis causing obstructive hydrocephalus, which was relieved on timely surgical excision and with medical management. Discussion: Neurocysticercosis (NCC) is the most common single cause of seizures/epilepsy in India and several other endemic countries throughout the world but it can present with headache as in this case report rarely. It is also the most common parasitic disease of the brain caused by the cestode Taenia solium or pork tapeworm. The diagnosis of NCC and the tapeworm carrier (taeniasis) can be relatively inaccessible and expensive for most of the patients. In spite of the introduction of several new immunological tests, neuroimaging remains the main diagnostic test for NCC. This is to highlight rare causes of secondary headache and various presentation of NCC, as it causes neurological morbidity which can be alleviated on appropriate treatment and thereby preventing economic hardship and improves quality of life.

HP2/97: Ischemic stroke and migraine in a tertiary care centre

Manickam V, Venkatraman C, Ranganathan L, Vellaichamy K

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

Objectives: To determine the characteristics of acute ischemic stroke (AIS) in patients with active migraine in a tertiary care centre. Methods: The authors studied the features of patients admitted to a tertiary care centre with active migraine. The diagnosis of previous episodes of migraine was made on answers to International Headache Society questionnaire. Those with migraine were divided into two age groups, one below 50 years and others above 50 years and compared by uni and multivariate analysis with age matched control subjects with AIS but without migraine. Results: Of 110 patients with acute AIS, 10 patients had active migraine (11%). 9 of them were younger than 50 and 3 were above 50 years. women proportion was higher in both groups of patients with migraine. In younger patients, PFO and posterior circulation stroke was characteristic. In older group, hypertension and smoking were common. In younger group, 1 patient developed AIS during active migraine. Conclusions: Migraine is common in young patients with AIS and infrequent in older patients. Patients with AIS and migraine are mainly women with stroke features that are age dependent

HP3/105: An exploratory study to understand association of emotional status with migraine disability

Rai NK, Singh R, Joshi A, Mitra A, Gupta A

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

Introduction: Migraine, a common pain syndrome, has a complex relationship with various emotional states. Aim: To evaluate association of various emotional states (depression, anxiety and stress) with migraine disability. Materials and Methods: In this hospital-based study, consecutive adults migraineurs (>18 years), fulfilling ICHD-3 criteria, were enrolled. Migraine Disability Assessment Test (MIDAS) and Depression-Anxiety-Stress scale (DASS-21) were used. Non-parametric tests were applied due to skewed data. “Quantile regression approach” was used, and its concordance was further assessed by “K-nearest neighbours” algorithm. Results: Total 132 patients (mean age 32.9±9.8, female (n=110; 83.3%) were included. Anxiety was most frequent (n=87; 65.9%) followed by depression (n=70;52%) and stress (n=52;39.4%). Severity of emotional states didn't change with increasing disability. For highest 2nd-3rd decile (70th-80th percentile) of MIDAS, only stress showed clear incremental gradient. “Mild-to-Severe disability” (N=98), had higher emotional state scores than “No-or-Minimal disability” (N=34){Mean Rank and p-values for depression (47.84 vs 72.97, p=0.001), anxiety (53.15 vs 71.13, p=0.018) and stress (48.09 vs 72.89, p=0.001) Mann-Whitney Test}. Conclusions: Severity of depression, anxiety and stress appears to be similar in all groups of “disabling migraine”, irrespective of its extent of disability. “Non-disabling migraine” had less severe emotional abnormalities. These observations suggest probably “all or none” association rather than severity of disability. Stress may have different relationship specially in more disabling migraine.

HP4/113: Evaluation of serum magnesium levels in patients with migraine

Rahul, Anand KS, Mahajan RK

Department of Neurology, Post Graduate Institute of Medical Education and Research and Dr Ram Manohar Lohia Hospital, New Delhi, India

Introduction: Migraine is a primary headache disorder causing significant disability. Magnesium has been implicated in many mechanisms underlying migraine development that includes central neuronal hyperexcitability, cortical spreading depression and nociceptive processing. Aim: To determine serum Magnesium levels in Migraine patients during attack and in between attacks and compare it with healthy controls. Methodology: Serum magnesium was measured in 50 migraine patients aged 18-45 years with or without aura diagnosed according to ICHD-III criteria for acute migraine headache along with same number of age and sex matched healthy controls. Results: In migraine patients, no significant difference was found in the mean Mg levels during and in between migraine attacks (1.82 ± 0.14 mg/dl versus 1.85 ± 0.13 mg/dl, p = 0.321) but mean Mg levels was significantly lower in migraine patients compared to the control group during attack (1.82 ± 0.14 mg/dl versus 2.09 ± 0.20 mg/dl, p < 0.0001) and in between attacks(1.85 ± 0.13 mg/dl versus 2.09 ± 0.20 mg/dl, p < 0.0001). In migraine group mean Mg levels were significantly reduced in patients having ≥4 attacks/month as compared to those having <4 attacks/month. Conclusion: Relatively low serum Magnesium levels in migraine patients as compared to healthy controls and inverse relation of serum Magnesium levels with frequency of Migraine attacks suggests that Magnesium plays an important role in pathogenesis of Migraine, which can be explored as a therapeutic option in migraine.

HP5/131: Assessment of visual evoked potential in patients with migraine

Thaseen Abdul Hameed, Selvaraj V, Jebarani M, Venkatesan V, Devanathan V

Department of Neurology, Saveetha Medical College, Chennai, Tamil Nadu, India

Introduction: Visual evoked potential is a reproducible and reliable parameter to assess the function of the visual pathway. It is a near-field potential from the visual cortices. Aim: In this study we aimed to assess the visual evoked potential (VEP) changes in migraine patients. Materials and Methods: In our study 30 consecutive patients attending our headache clinic, having Migraine according to International Headache Society criteria (10 patients with migraine with visual aura and 20 patients with migraine without aura) were included and compared with 50 healthy volunteers who had neither migraine (including other types of primary headache) nor familial history of migraine. VEP was assessed using checker board by monocular pattern reversal stimulation technique using Allengers Scorpio -4 device. We compared P100 latencies within the group and between groups. Results: The mean P100 latency in the healthy volunteers is 103±6.2 and the mean P100 latency in the migraine patients (with and without aura) is 106±4.3. The mean P100 latency was prolonged in 10 (p<0.05) of our migraine patients, of which 4 were migraine with aura and 6 were migraine without aura. The mean P100 latency was prolonged in both the subgroups of migraine as compared to the healthy volunteers. Conclusion: These results indicate involvement of the visual pathway in both groups of migraine patients, with and without aura.

HP7/195: Precipitants of headache in migraine patients attending neurology OPD in a tertiary care centre

Saravanan S, Mansoora

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

To identify the triggering factors of migraine we did Prospective observational for the period of three months from April 2019 to June 2019. We selected 100 patients who attended neurology OPD in Tirunelveli medical college hospital who fulfilled migraine case definition based on international headache society criteria. Patients demographic details, diagnosis, medication details and other medication taken will be collected in a specially designed proforma to identify the triggering factors of migraine. In our study majority of population belongs to the 31-40 years age group(28%) followed by the 51-60 years age group (23%) and Majority of the study population were females(83%) in sex distribution. Among the males majority were in the age group of 31-40 years (7%). Among the females also majority were in the age group of 31-40 years (21%). Majority of the migraine people (94%) have Grade IV headache in which females (95.2%) males (88.25) and most of them in the 31-40 years age group. There is no statistically significant trend is noticed with severity of headache between two groups. Chi square test for trends was used (p =0.21). Males who have history of exposure to excessive sunlight (80%) and history of journey (84%) less odds of having migraine and the difference is statistically significant. In conclusion, migraine common in females among them strong smell exposure precipitate migraine attack. In males excessive sunlight exposure and journey were precipitate migraine.

HP8/301: A rare manifestation of migraine in a south Indian family

Sreejith V Ravi, Saravanan S, Packiaseli RC

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

Introduction: Migraine can have various atypical manifestations that can lead to diagnostic dilemma. Here we report a rare presentation of migraine in a south Indian family. Case Summary: A 15 year old girl without any co-morbidities presented with history of sudden onset right sided throbbing type of headache associated with nausea and vomiting, followed by weakness of left upper and lower limbs. She also had intense photophobia. She had history of recurrent episodes of headache for the past two years associated with aura, lasting for few hours which subsided spontaneously. Last month also she had history of similar headache episode with left sided weakness which recovered after a night. There was no history of fever, altered sensorium or seizures. Neurological examination showed left sided hemiparesis. Her complete hemogram, liver, renal and function tests, electrolytes were normal. MR imaging of the brain with MRA and MRV didn't reveal any abnormality. Carotid artery Doppler was normal. ECG and Echocardiogram were also normal. Her EEG didn't show any epileptiform discharges. On enquiring detailed history, her mother and sister were having similar history of episodic weakness of unilateral extremities with normal brain imaging studies. She was diagnosed as a case of Familial hemiplegic migraine and was treated with analgesics. Her weakness improved and she was discharged after giving Flunarizine and Acetazolamide tablets. Conclusion: Here we report this case because of the rarity of this presentation of migraine in this particular family with three affected members so that it won't be confused with acute stroke.

Saturday, October 05, 2019, 13:00PM-20:00PM

Poster Session 4: Epilepsy, Dementia, Movement Disorders, Autoimmune Disorders

EP1/13: Seizure profile in poststroke patients in Eastern India

Humbi U, Ganguly G, Pandit A

Department of Neurology, Bangur Institute of Neurosciences, IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Introduction: Post stroke seizures greatly influence morbidity and mortality of stroke patients. The clinical spectrum of seizures in post stroke patients, with their radiological and laboratory profile in relation to different stroke subtypes in our population, is still to be understood and a study relating to this is an unexplored area. Objectives: This study is taken up to explore the profile of seizures in post stroke patients in our population, studying their semiology, time of onset, correlation of occurrence of seizures with anatomical location of stroke, and analyzing seizure occurence in different subtypes of stroke. Materials and Methods: This cross sectional study was conducted in Bangur Institute of Neurosciences from September 2017, including the patients who fulfilled the inclusion criteria after written informed consent. A correlation for exact clinical spectrum of seizures in post stroke patients, with the radiological and laboratory profile in relation to different stroke subtypes was then determined. Seizure semiology was determined using ILAE 2017 Classification of seizure types. Radiological assessment included CT Brain +/- MRI Brain. Stroke was classified into ischaemic, haemorrhagic or embolic type. Side, location and depth of the lesion was determined. Ischaemic strokes with demonstrable cardiogenic source of emboli were considered as embolic strokes. Ischaemic stroke was classified into cortical,subcortical or combined cortical and subcortical stroke. Infarcts <5 cms on CT Brain were considered to be small and >5 cms as large. Haemorrhagic CVA was classified into deep ICH +/- IVH +/- lobar extension or lobar ICH. Cortical bleed in ICH was defined as any bleed extending to cerebral cortex. Volume of ICH was calculated using ABC/2 score in CT Brain. Bleed volume <30 ml was taken as small and >/=30 ml large. Stroke severity was calculated using CNS score and associated comorbidities were accounted using Charlsons- Deyo Comorbidity index score. EEG findings were categorised into normal, diffuse slowing, focal +/- diffuse slowing, focal spikes, sharp waves, alpha coma and PLEDS. Statistical Analysis was performed using statistical software SPSS version 20. Results: Focal seizures form the most common semiology of post stroke seizures, usually with early onset of seizure occurrence. Cortical and combined cortical with subcortical lesions and larger lesions predispose frequently to seizures. Factors contributing to recurrence are late onset, post ischaemic seizures and EEG demonstrating PLEDs. Morbidity and mortality of post stroke seizures is influenced by comorbid conditions and increased with status epilepticus. Conclusion: Post stroke seizures greatly influence morbidity and mortality of stroke patients, hence such study is need of the hour to help assessing prognosis in post stroke epilepsy.

EP2/16: Occipital seizures imitating migraine aura

Thomas R, Rajesh A

Department of Neurology, Travancore Medical College Hospital, Kollam, Kerala, India

Introduction: Though occipital epilepsy and migraine are different disorders, they have symptoms in common. The visual phenomena that precede headache in migraine are also common in occipital lobe seizures. Distinction between them is not usually difficult. In epileptic seizures, elementary visual hallucinations are brief, lasting for three minutes at most; they are predominantly multicoloured, with circular or spherical patterns; and their onset is always on the same side. There is no photophobia. Post-ictal headache is common but rarely throbbing and severe. Here we report cases in which the visual features of occipital seizures resembled migraine aura. Case Reports: (1) 10 -year-old boy with history of severe throbbing headache preceeded by visual aura since one year. Visual aura consisted of flashes of light lasting for few minutes. There was no loss of consciousness or convulsion. On occasion his vision went black. He was on Flunarizine with no definite benefit. His MRI Brain was normal. EEG showed occipital discharges. He improved with oxcarbamazepine. (2) 14 old female presented with recurrent headaches preceeded by visual aura. The aura was described as bright, black flashing spots which appeared in a temporal hemifield moving towards the centre. This lasted for around thirty minutes. She felt as if her legs would give way which was followed by hemicrania lasting for four hours. She improved with carbamazepine. Conclusion: Elementary visual hallucinations, blindness, headache and vomiting, which are common manifestations of occipital lobe epilepsies, should not be unquestioningly equated with migraine.

EP3/36: A case of epileptic encephalopathy

Gowda C, Kumar PS

Department of Neurology, Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India

Introduction: Spectrins are a family of filamentous cytoskeletal proteins that function as essential scaffold proteins that stabilize the plasma membrane and organize intracellular organelles. Spectrins are composed of alpha and beta dimers that associate to form tetramers linked in a head-to-head arrangement. The encoded protein has been implicated in other cellular functions including DNA repair and cell cycle regulation. Mutations in this gene are the cause of early infantile epileptic encephalopathy. Early infantile epileptic encephalopathies (EIEEs) are a group of neurological disorders characterized by early-onset refractory seizures, severe electroencephalographic abnormalities, and developmental delay or intellectual disability. Recent progress in genetic analysis reveals that a significant proportion of cryptogenic epileptic encephalopathies are single-gene disorders. SPTAN1, the gene coding for the non-erythrocyte alpha-II spectrin, has been known as a rare cause of early-onset epileptic encephalopathies with hypomyelination and atrophy. More than half of the disease-causing variants in SPTAN1 are due to in-frame deletions and duplications. The major clinical features of SPTAN1 mutations include epileptic encephalopathy with hypsarrhythmia, no visual attention, acquired microcephaly, spastic quadriplegia and severe intellectual disability. Brainstem and cerebellar atrophy and cerebral hypomyelination, as observed by magnetic resonance imaging, are specific hallmarks of this condition. Mild cases have been reported, suggesting that different SPTAN1 variants can be associated with different clinical manifestations. Attempts to establish genotype/phenotype correlations have suggested that mutation site and type can play a role in conditioning development and severity of nervous system damage. Many mild cases had SPTAN1 mutations located far from the C-terminal region. In contrast, most of the severe cases had in-frame deletion/duplication mutations located in the last two αII spectrin repeats in the C-terminal region. Case Description: A developmentally normal child born to non-consanguineous parentage, presented with fever triggered seizures at the age of 1 year. From the age of 3 years she developed recurrent seizures most of which manifested as versive head turning to left with tonic-clonic movements of all four limbs associated with eyelid myoclonia. At the age of 3 and half years she developed marked truncal ataxia, tremors of hands and slurring of speech which stabilized relatively over a period of 8-9 months. She has a mild cognitive decline (IQ-85) and ADHD (Attention Deficit Hyperactivity Disorder). MRI brain revealed reduced white matter volume and EEG showed continuous spike and wave activity in slow wave sleep. She has been started on low dose oral steroids, lamotrigine, valproate and clobazam, and for ADHD she has been put on clonidine. At present seizures are well controlled, however mild ataxia and tremors persist. Results: Clinical exome sequencing showed a heterozygous 9 base pair deletion in exon 53 of SPTAN1 gene. The observed variation lies in the spectrin repeat domain of the SPTAN1 protein and has been previously reported in a patient with drug resistant focal epilepsy, mild cognitive impairment and ADHD. Conclusion: In patients with mild cognitive impairment, drug resistant seizures and ADHD consider SPTAN1 mutation.

EP4/48: A retrospective observational study of video electroencephalography among children with epilepsy

Swathi KA, Garuda BR, Seepana G, Talabhaktula SK, Upputuri AK

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

Introduction: Seizures are the commonest paediatric neurological disorders. Perinatal injury was strongly associated with epilepsy, suggesting that epilepsy may be preventable in a significant proportion in India. Aim: The aim is to describe the Video EEG and the clinical characteristics in children with epilepsy. Methods: A retrospective observational study of children with epilepsy between 1 month to 18years age referred for Video EEG between February 1st, 2019 to March 31st 2019, done in Department of Neurology, King George Hospital. Results: 147 children EEGs were analysed where 54% were males and 46% were females. Mean age of presentation was earlier in males (5.90±5.55) compared to females (7.68±6.11) with p-value-0.06.Most common age of presentation was less than 1 year age (40%). Perinatal injury observed in 46% cases. Developmental delay correlated significantly (p<0.001) with abnormal EEG suggesting it as a significant predictor of abnormal EEG record. Generalised seizures were the commonest semiology noted (55%), but focal epileptiform discharges (27%) were the commonest abnormal EEG recorded. 34% of 41 cases with generalised seizure semiology had focal epileptiform discharges where EEG was helpful in localisation. Epileptic encephalopathy was seen in 6 cases (3-LGS, 2-West syndrome, 1-Rasmussen encephalitis), 1case of SSPE and 1 case of PME. Most common drug used as monotherapy was phenytoin and as polytherapy was sodium valproate, in combination with clobazam, phenytoin, or carbamazepine each. Conclusion: Video EEG is important in accurate identification, localisation and syndromic classification of children with epilepsy. It also aids in appropriate treatment and prognostication of epileptic encephalopathies.

EP5/91: Study of the electroencephalography referral patterns at a tertiary care centre: An audit

Kawoosa A

Department of Neurology, Government Super Speciality Hospital, Srinagar, Jammu and Kashmir, India

Introduction: EEG has developed, over a past few decades, into a major diagnostic Neurology tool. However due to lack of proper EEG knowledge among the referring personnel, the valuable tool is often misused. Than in turn leads to loss of efforts, money and time; especially in a resource limited state. Aims and Methodology: We aimed to study the EEG referrals and yield at our tertiary care hospital by retrospectively performing an audit of the departmental records regarding EEGs a performed over a period of several months. Results: The frequency of EEG patient requests were maximum in the Children (26.2%) followed by the Adolescents (22.2%). There was no significant Gender difference among Normal EEG patients nor among Abnormal EEG patients. The most common referral indication overall were Seizures (33.6%) followed by Epilepsy (25.17%). A high proportion of EEG requests were done for Doubtful seizures/pseudosiezures (11.5%) and Syncopy and “Funny turns” (10.7%). Of all the EEGs done, 20.63% of EEGs were reported as abnormal. The highest percentage of abnormal EEGs were seen in patients referred for Encephalopathy (87.5%), followed by Epileptic patients with seizure Recurrence (41.1%). Lowest EEG abnormality yield was seen with Epilepsy patients on follow-up (8.2%), Syncopy and “Funny turns” (5.88%), Doubtful seizures/pseudosiezures (6.36%). Around one thirds (36%) of the referrals for EEG were deemed as “inappropriate” requests. Conclusion: A high proportion of patients are inappropriately referred for EEGs which leads to wastage of manpower, finances and time, especially in a resource limited place, like ours.

EP6/128: Epileptic spasms clinical characteristics and predictors of remission

Ramana Paila, Jayalakshmi SS, Patil A, Babu SP

Department of Neurology, KIMS Hospital, Hyderabad, Telangana, India

Background: Evaluate the clinical, EEG and imaging features in patients with epileptic spasms (ES) & their role in determining treatment response. Methods: Retrospective and prospective analysis of data of 111 patients with ES with minimum two months follow up was performed to predict remission. Results: Mean age at presentation was 7.99 ± 8.15 yr, while mean age at onset 2.67 ± 3.75yr, with 74 (66.7%) male patients. Delayed developmental milestones were noted among 67 (60.4%). partial seizures, absence and tonic seizures were common seizure types associated along with ES. Interictal GPFA was noted in 38.7%, while focal regional spikes were seen in 48.2% and multifocal sharp wave discharges were seen in 79.3% patients. Generalised burst of sharp waves followed by diffuse attenuation was noted at ictal onset in 37.9%. MRI brain was normal in 17 (26%), diffuse changes was noted in 22.5% of patients while 21.6% of patients had hemispherical involvement.Frontal lobe (23.4%) was commomnly involved and MCD was the most common abnormality in this lobe. parieto occipital lobes were involved in 13.5% of patients and most of them had gliosis. Valproate was most commonly prescribed AED. Patients with neonatal onset seizures (p-0.038), childhood insult (p-0.037), multifocal IEDs were associated with poor outcome, while generalised discharges (p=0.21) and focal/hemisherical gliosis (p=0.014) and who underwent surgery (p<0.001) associated with better remission. Conclusions: ES may occur with multiple etiologies well beyond infancy. Generalised IEDs, focal /hemispherical gliosis & who underwent surgery early had good prognosis.

EP7/156: Epilepsy: Aspects beyond seizures

Deepa Avadhani, Kannan V, Chandramouleeswaran V, Narasimhan LR

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

Objective: About 50 million people across the globe are living with epilepsy. Majority are in the developing nations. The impact of epilepsy on patients' lives is far beyond seizures. This study focuses on how epilepsy influences other aspects of life. Materials and Methods: It is an observational study. 60 patients with epilepsy in the age group of 18-50 years were asked to answer the questionnaires. Variables including sleep quality, excess daytime sleepiness, fatigue, sexual desire and anxiety and depression were assessed by Pittsburgh Sleep Quality Index, Epworth Sleepiness Scale, Fatigue Severity Scale, sexual desire inventory-2 and Hospital Anxiety and Depression Scale respectively. Results: 35% of patients scored high on fatigue severity scale. 48% of patients had poor sleep quality. 29% of them experienced increased daytime sleepiness. 68% had increased depression. 58% of patients experienced low sexual desire. Incidence of depression and sleep disturbances were more with patients on polytherapy as compared to monotherapy. Conclusion: Individuals with epilepsy need treatment in various other aspects apart from seizure control. Caregivers should be educated regarding the impact of the disease as well as drugs on patients' life. A comprehensive approach is needed to treat depression, fatigue and sleep disturbances in epilepsy along with treatment of seizures.

EP8/174: A study on clinical profile of status epilepticus in adults

Sridharan Muralidharan, Thangaraj M

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

Introduction: Status epilepticus is a life threatening neurological disorder with increased morbidity and mortality. Objectives: To study the aetiology, demographic features, clinical profile and outcome of SE in adult population. Study Design: Cross sectional observational study. Materials and Methods: Study was carried out from december 2018 to may 2019. 25 patients of SE above the age of 12 years were included in the study. Clinical history, systemic examination and appropriate laboratory investigations were used to categorise different variables. Results: Among 25 patients, maximum patients (40%) were between 20-40 years. Males (60%) had higher incidence when compared to females (40). Among the patients GTCS (76%) was the commonest semiology. Epilepsy was the most common diagnosis noted (56%). Among the cases of epilepsy, drug incompliance was the commonest cause (64%). Among the symptomatic seizures 10 (91%) had acute symptomatic seizure and 1 patient had remote symptomatic seizure. CNS infections were the commonest cause of symptomatic seizure (50%). Among the 25 patients 7 (28%) developed aspiration pneumonitis out of which 4 (57%) patients developed respiratory failure. In this series 3 (12%) patients died, all were acute symptomatic cases, 2 of whom were above the age of 60 years. Haemorrhagic stroke was the commonest cause (66%) of death. In this study poor response to first line drugs and longer duration of status epilepticus has poor outcome which is statistically significant. Conclusions: Aspiration pneumonitis, use of second line antiepileptic and prolonged seizure duration were the independent predictors of poor outcome in status epilepticus.

EP9/178: The road less travelled: A 7-year study of burns in epileptics

Archana Sachin Verma

Department of Neurology, MGM Medical College, Indore, Madhya Pradesh, India

Introduction and Background: In India, the epilepsy treatment gap ranges from 22% among urban, middle-income people to 90% in villages. Resultant effect is frequent attacks and vulnerability to injuries including burn. The burn injury sustained is deep owing to lack of escape attempt. Materials and Methods: longitudinal descriptive case record study of 3181 acute burns admitted in burn unit of central India from January 2009 to December 2015. Thirty-five cases studied to identify distribution and determinants of burn in epilepsy. Statistician noted and analyzed variables like age, sex, mode of burn, burn severity, burn site, seizure type and anti-epileptic compliance using SPSS software. Results: The mean age was 28.6years with standard deviation of 13.26. Epilepsy observed in 1.1% patients. Male: female ratio was 1:2.89. Flame burn (57.14%), scald (37.14%) and other burn noticed. Third degree (60%), second degree (25.71%) and first degree (14.2%) burn sustained. Upper limb (62.8%), lower limb, trunk (25.7%), head neck (11.4%) involved. GTCS (80%), absence (14.2%), complex partial seizures (5.71%) observed. Antiepileptic compliance <1 year (60%), >1 year (28.5%) and absolute compliance (11.42%) observed. Conclusions: Burn injuries in epileptics are preventable. Determinants are young females, flames due to kerosene, GTCS and non-compliance to antiepileptic. Burn injury sustained is very severe. An efficient burn prevention and management plan in epileptics requires avoidance of inflammable agents, optimization and regularization of antiepileptic treatment and special burn care units. Limitations: Retrospective non-analytical study. Further Scope: Formation of “State of Art Unit for Epileptic Burns.”

EP10/227: Study of occurrence of executive dysfunction in idiopathic epilepsy

Justin Cornelius. Chezian Deivanayagam, Manivannan Ramraj, Lourdes Pravina Vasagam

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

Epilepsy is a multi faceted disorder that affects people of all ages and both gender. Idiopathic epilepsy is defined as disorder in which there is no underlying cause or structural pathology other than a possible hereditary predisposition for generating seizures which can be generalised or focal in nature. This study aimed to measure neuropsychological performance in patients with Idiopathic epilepsy. To study the association of age, gender, seizure type, duration of epilepsy, age at seizure onset, with executive dysfunction in patients with idiopathic epilepsy. Study Population: All patients of epilepsy ≥18 years of age with normal imaging were included in the study for analysis. Results: 31 males (62%) were males and 19 (38%) were females, 10 (20%) had a seizure frequency more than 3 or more per year and 40 (80%) had a seizure frequency less than 3 per year, 13% were on polytherapy and 74% were on Monotherapy, Frontal Assessment Battery tests were used to assess executive dysfunction and 28 (46%) had normal scores and 22 (44%) patients demonstrated executive dysfunction. FAB score was abnormal in 84.61% cases on polytherapy compared to 43.24% cases were on monotherapy signifying more Executive dysfunction in the polytherapy group. The difference was statistically significant with a (P value =0.021). Conclusion: A significant proportion of patients with idiopathic epilepsy have executive dysfunction which adds to the seizure burden by reducing the capacity of an individual to successfully engage in self care, social, academic and occupational pursuits.

EP11/241: A study on clinical, electroencephalographic and imaging profile of patients with drug resistant epilepsy in a tertiary care centre

Jayakrishnan MP, Jeyaraj MK, Velayutham SS, Sowmini PR, Mugundhan K, Arunan S

Department of Neurology, Government Stanley Medical College, Chennai, Tamil Nadu, India

Introduction: Drug-resistent epilepsy (DRE) is defined as “failure of adequate trials of two tolerated and appropriately chosen and used antiepileptic drug (AED) schedules (whether as mono-therapies or in combination) to achieve sustained seizure freedom.” Aim: To evaluate the clinical, electroencephalographic (EEG) and imaging profile of 25 patients with DRE

Materials and Methods: 25 patients with DRE who attended the Epilepsy Clinic of Stanley Medical College were included in the study. They underwent detailed Clinical examination, prolonged video EEG monitoring and MRI Brain study under epilepsy protocol.

Results: Age of the patients included in study ranged from 4 to 42. 20% of the patients had abnormal birth history and 52% of the patients had antecedents (febrile seizure, meningoencephalitis, head trauma) preceding the development of epilepsy. 76% of patients had extra temporal complex partial seizures and 16% of patients had seizures with temporal lobe origin. EEG showed focal inter-ictal epileptiform discharges (IED's) in 60% of the patients and multifocal IED's in 40% of the patients. Brain imaging was abnormal in 76% of the patients among which 44% of the patients had gliosis and 20% of the patients had medial temporal lobe sclerosis.5 patients underwent successful epilepsy surgery in our hospital and are seizure free postoperatively. Conclusion: Our study shows that almost 60% of DRE patients have focal IED's on EEG and surgical substrates on imaging and are candidates for pre surgical evaluation. It indicates the continuing need for establishment of many new epilepsy surgery centers in our country.

EP12/245: Infantile onset epilepsy: Semiological, diagnostic and therapeutic challenges

Ramesh Konanki, Mohanlal S

Department of Neurology, Rainbow Children's Hospital, Hyderabad, Telangana, India

Introduction: Incidence of epilepsy in infancy (2months-24months) is highest of all age groups. The data on clinical spectrum, etiology and prognosis is sparse. Methodology: Infants with epilepsy onset between 2 months-24 months age enroled (January 2016-May 2018). Infants with epileptic spasms at onset and neonatal-onset epilepsies were excluded. Short-term seizure control and neurodevelopmental status after 3 months evaluated. Seizure control graded: completely controlled (>90%), partially controlled (50-90%), uncontrolled (<50%) Results: 90 infants were enrolled: M:F=57:33, mean age 6 months. 15 (17%) had consanguinity, 14 (16%) had perinatal complications, 18 (20%) had family history of epilepsy, 6 had neonatal seizures; Seizure semiology: focal onset 28/90 (31%), unknown onset 23/90 (26%), unclassified 14/90 (15%), generalised onset 15/90 (17%), 10/90 (11%) had unknown onset. 16 (18%) had status epilepticus. 30 (33%) had daily seizures. Etiology was structural in 21 (23%), metabolic in 5 (5%), genetic in 2 (2%), and unknown in 62 (68%). 48 (53%) were on 1 antiepileptic drug, 32 (35%) on 2, 10 (12%) on 3 drugs at enrolment. MRI abnormalities were seen in 28 (31%), Epileptiform discharges in 17 (19%). Treatment response: 43 (48%) completely controlled, 34 (38%) partially controlled, 13 (14%) uncontrolled. 2 (2%) developed epileptic spasms. 23 (25%) required more than 2 drug to control seizures. Conclusion: Semiologic classification (ILAE 2017) is challenging in infantile-onset epilepsy (unknown/unclassified onset in 41%). Despite extensive work-up, etiology remained unknown in 68%. Nearly 50% had complete seizure control.

EP13/251: CNS lupus: Masquarading as progressive myoclonic epilepsy

Manish Bhartiya, Pandit AK, Kanchana P, Prasad K

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

Progressive myoclonic epilepsies are a group of disorders which primarily present in the childhood with myoclonus, cognitive decline and se. The underlying etiology includes Lafora disease, myoclonic epilepsy with red ragged fibres (MERFF), NCL, and Gauchers disease. CNS lupus mainly presents with behavioral changes, seizures and cognitive decline. We present such a case of young female child who presented with syndrome of progressive myoclonic epilepsy and on evaluation had CNS lupus responding to the treatment. 11 years old girl, with normal birth and developmental history presented with complaints of recurrent episodes of myoclonic jerks, seizures and progressive cognitive decline without any history of antecedent fever, weightloss or any behavioral changes. At presentation she was bed ridden, with only verbalization in form of short sentences and had frequent myoclonic jerks with a frequency of 10-15 per day along with occasional GTCS, she was initially suspected as a case of Progressive Myoclonic epilepsy with possibilitis being MERFF or NCL. However, during the course of stay in the hospital she was noticed to have alopecia and malar rash. She was subsequently evaluated for possible autoimmune disorders, her ANA was strongly positive, elevated 24 hr urinary protein, MRI brain and CSF examination was normal. She underwent renal biopsy which revealed features suggestive of lupus nephritis. She was started on pulse methyl prednisolone followed by immune suppression with cyclophosphamide (after consent). She showed dramatic response to steroids and had reversal of congnitive symtoms and has been seizure free since last 04 months.

EP14/258: Seizures with bilateral basal ganglia and frontal calcification as the initial manifestation of Albright's hereditary osteodystrophy in a child

Akhilesh Kumar N, Jawalkar S, Manorenj S, Gubba SK, Bilal SU, Naaz B

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

Introduction:  Albright hereditary osteodystrophy More Details (AHO) is a rare hereditary metabolic disorder that presents with seizure secondary to hypocalcaemia. Characteristic presentations are short stature, obesity, brachydactyly, which are the phenotypic features of genetic mutation. Pseudo hypoparathyroidism (PHP) is observed in patients with AHD and is characterized by inability of the body to respond appropriately to parathormone, mainly characterized by hypocalcaemia, increased serum parathormone concentration, insensitivity to the biological activity of parathormone and hyperphosphatemia. Case Report: A 14 year old boy, product of non-consanguineous parentage, presented with history of two episodes of generalized tonic-clonic seizures in one hour duration. Physical examination showed hypertelorism, broad facies with moderately low IQ (48) for age, malerupted teeth, and subcutaneous calcification over the flexor aspect of left wrist. Anthropometry revealed height of 134 cm (less than 3rd percentile), arm span of 123 cm, weight of 35 kg, and widened bilateral thumb with short 4t and 5th fingers with dimpling of fist at 4th and 5th metacarpophalangeal joint suggestive of brachydactyly. The skin over sole of feet was dry and crackled. Nervous system examination showed positive chvostek's sign bilateral, positive trousseau's sign and positive woltman sign. Biochemical evaluation revealed hypocalcaemia with mild hyperphosphatemia, hypothyroidism, hypovitaminosis D3, and hyperparathyroidism. Computed tomography brain showed bilateral basal ganglion calcification and subcortical frontal curvilinear calcification. Conclusion: Careful examination of phenotype of patients presenting with seizure clues to the diagnosis of AHO. Metabolic work up should be done in cases where imaging brain shows basal ganglion and subcortical calcification.

EP15/311: Thalamic tuberculoma with gelastic seizures

Mohammed Shujauzzaman Bilal, Jawalkar S, Manorenj S

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

A 42 year old, right handed female, housewife from hyderabad, presented with the chief complaints of right upper and lower limb weakness in 2012. She was diagnosed as tuberculoma in the left temporal lobe with mass effect by Magnetic Resonance Imaging (MRI) of brain with contrast study. She was started on antitubercular (ATT) medications with HRZE. She improved over the next 15 days and was discharged. She used ATT for 2 months and stopped. She presented again in 2013 with inappropriate laughing in an episodic manner. Clinically diagnosed as gelastic seizures restarted on ATT and started on Anti-epileptic medications. She responded to anti epileptic medications. Used ATT for the next 3 years and the hemiplegia completely recovered. ATT and antiepileptic was stopped in June 2016. Reimaging was not done. In December 2016 she was readmitted with paraplegia and recurrance of gelastic seizures. She was then restarted on antiepileptic and ATT. Has been using these medications since 3 years and is currently asymptomatic except for body pains. Imaging in february 2019 showed persistant thalamic tuberculoma and she has been started on second line antitubercular drugs.

EP16/319: New onset seizures in the peripartum period - clinical characteristics, etiological spectrum and outcome: A hospital-based study in South India

Keshava Anand G, Jaiswal SK, Murthy JMK, Yerasu M

Department of Neurology, CARE Hospitals, Hyderabad, Telangana, India

Introduction: Seizures in the peripartum period are associated with significant morbidity and mortality to the women and their unborn baby. Methods: This is a retrospective study of consecutive pregnant women with newonset seizures during peripartum period admitted between January 2005 and December 2018.The data collected from the caserecords included demographic data; day of seizure(s) after delivery; seizure semiology, neurologic deficits; admission blood pressure (BP) and BP readings during the hospital course; aetiology; neuroimaging and EEG findings; and treatment outcomes. Results: During the study period 41 (mean age ± SD: 26.20 ± 4.22 years; range 1935 years) women were admitted for newonset seizures during peripartum period, 12 (29%) in the antepartum period and 29 (71%) in the postpartum period. Of the 41 women with peripartum newonset seizures, 20 (48.7%) women had hypertensive disorders of pregnancy (HDP). Seizure type was: generalized onset tonicclonic (88%); focal onset motor (7%); and unknown onset motor (5%). The seizure presentation was: single (39%); seizure cluster (54%); and CSE/NCSE (7%). Aetiology was: (1) epilepsy before pregnancy 3; (2) new nonpregnancyrelated seizure disorder 33; and (3) newonset pregnancy related seizure disorder 5; Posterior reversible encephalopathy syndrome (PRES) was the aetiology in 24 (58.5%). PRES was significantly more common in women with HDP as compared to normotensive women [18/20 (90%) vs. 6/21 (29%); p=0.0001]. Patients with PRES significantly present with seizure clusters when compared with patients with other aetiology [17/24 (72%) vs. 5/17 (29%); p= 0.0087].Conclusions: In this study PRES related to HDP was the common cause of seizures in peripartum period.

EP17/325: Fixation off sensitivity: Two case reports

Smitha Chandran K, Netto AB, Kumar P, Janardhan DC

Department of Neurology, Bangalore Medical College, Bengaluru, Karnataka, India

Mr. H, 23 year old gentleman, presented with seizures from 10 years of age. Semiology begins with aura of dark spots in the visual field lasting for 1-2 minutes followed by generalised tonic-clonic seizures or impaired awareness with ictal talking. Frequency being once in fifteen days. He had normal birth, developmental and family history. General physical examination was normal and neurological examination revealed borderline low IQ. EEG showed triphasic high amplitude discharges in the posterior quadrant bilaterally with distinct asymmetry (right > left). MRI brain was normal. Master S, a 6 year old boy presented with poor scholastic performance and inability to read from a distance, despite multiple refractive corrections. On probing he reported blurring of vision while trying to read, and mother has noticed short period of unawareness lasting for 2-3 seconds. He was born preterm and had neonatal asphyxia with left occipital gliosis. General physical and neurological examination was normal. EEG revealed, asymmetric back ground with slowing of alpha on the left and recurrent paroxysmal slow spike and wave activity from left occipital region with occasional homologous spread to opposite side. These were reactive to eye opening. Detailed EEG studies were conducted in both patients to analyse eye closure sensitivity (ECS), Fixation off sensitivity (FOS) and scoto sensitivity. ECS was ruled out as the discharges persisted more than 3 seconds after eye closure. Photo sensitivity, scoto sensitivity was ruled out by providing absolute darkness during EEG. FOS was tested with Frenzel glasses as well as with a white paper kept close to the eyes, to avoid fixation, and both the patients had FOS. The first patient was diagnosed as Gastaut childhood occipital epilepsy based on the age of onset, semiology, absence of autonomic symptoms and characteristic FOS with a normal MRI brain. With carbamazepine seizures were well controlled. Conclusion: FOS is a rare phenomenon (0.2% incidence) and is classically described in childhood idiopathic occipital epilepsies.The persistence of FOS in adulthood is further rare They are occasionally seen in symptomatic epilepsies also. We present two patients, with entirely different spectrum presenting with FOS.

EP18/335: Rasmussen encephalitis: Experience from a tertiary care centre

Guhan Ramamurthy, Kannan V, Ranganathan LN, Chandramouleeswaran V

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

Introduction: Rasmussen encephalitis, a rare neurological disorder is characterised by hemiatrophy, hemiparesis, drug resistant epilepsy and cognitive decline. Here we report 3 cases of Rasmussen encephalitis, and its therapeutic response from tertiary care centre. Cases: Case 1: 29 year old male presented with history of left focal seizures since 5 years of age with poor control recently requiring AED polypharmacy and was accompanied by weakness. Neurological examination revealed weakness and atrophy on left. Neuroimaging was suggestive of Rasmussen encephalitis. Patient was treated with immunotherapy and improved. Case 2: 17 year old male presented with right focal seizures since 6 years of age with poor control recently and on regular AED. He also developed gait and limb in coordination. The seizures were poorly controlled. Examination revealed right hemiparesis. Neuroimaging was suggestive of Rasmussen encephalitis. Patient was treated with immunotherapy and improved. Case 3: 9 year old boy presented with complaints of right focal seizures for 4 years duration. Examination revealed right hemiparesis with atrophy of right upper limb and lower limb. Neuroimaging was diagnostic of Rasmussen's encephalitis. Patient was treated with immunotherapy and improved. Discussion: In our series of Rasmussen's encephalitis, all the patients were males. All patients had epilepsy refractory to multiple antiepileptic drugs. Their seizure frequency reduced significantly with immunotherapy. The favourable response to immunotherapy favours immune mediated process in the pathogenesis of the disease. Conclusion: Rasmussen's encephalitis Is an important cause of refractory focal seizures. It is necessary to consider treatment with immunotherapy prior to considering surgical measures.

EP19/348: Treatment gap in epilepsy

Kamala Kant Bhoi, Tiwari A, Kumar S, Kumar S

Department of Neurology, Shri Balaji Institute of Medical Sciences, Raipur, Chhattisgarh, India

High treatment gap in epilepsy is a burden to society. We aimed to study factors responsible for it. We studied 1008 patients with epilepsy attending OPD of Shri Balaji Institute of Medical Sciences, Raipur from Dec'2016-Dec'2018. It included demographics, awareness, stigma, compliance to anti epileptics and health care facilities. High treatment gap is due to lack of awareness, high cost and poor health care in rural areas. Epilepsy should be a public priority by Government.

EP20/354: Medication adherence and seizure control in epileptic patients: A questionnaire based study

Sunitha Asula, Vengamma B, Prasad NSV

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

Introduction: In 2014 ILAE defined epilepsy as disease of brain defined by any of the following (1) At least 2 unprovoked seizures occurring >24hrs apart, (2) one unprovoked seizure and probability of further seizures and a probability of further seizures similar to general recurrence risk after two unprovoked seizures occurring over next 10 years and (3) diagnosis of an epilepsy syndrome. Controlled epilepsy was defined as a patient who is seizure-free for at least 18 months. Background: Patients with epilepsy coming to Sri Venkateswara Institute of Medical Sciences epilepsy camp are given free drugs, but they come with history of seizure recurrence. The present study is aimed to assess the relationship between medication adherence and seizure control. Methods: This is a descriptive observational study, participants are patients coming to monthly epilepsy camp, for free medication. They were asked to complete Morisky 8 item medication adherence scale questionnaire. Score of 0-2 is high adherence, 3-6 moderate adherence, 7-8 is low adherence. Results: Total of 195 participants underwent the study, among them males were 124 (63.6%), females were 71 (36.4), major seizure type was GTCS observed in 82 patients (42.1%). High adherence was seen in 177 (90.7%), low adherence 18 (9.2%) the relation between adherence and seizure control was statically significant. Remission was seen in 84 (43.1%) patients. Conclusion: Patients who have good drug compliance have seizure control.

EP21/366: Factors determining poor adherance in antiepileptic drugs in epileptic patients attensing tertiary care hospital

Sankaranaryanan P, Saravanan S

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

Adherence to antiepileptic drug therapy is important for controlling seizures in patients with epilepsy. This study aimed to identify the factors that influence the adherence. This is the Prospective observational study for a Period of one year in which Patients who have been diagnosed with Epilepsy were included. Treatment adherence was assessed using the Self-reported eight item Morisky Medication Adherence Scale. Among the100 patients we studied Majority of them were in the age group of 31 to 40 years. Mean age of the study population was 38.3 years (SD 15.6). Majority of the study population were males (52%). 28 % of the study population were illiterates. About 86% of the participants had studied up to middle school. Mean adherence score is 1.7 (SD 1.78). Prevalence of poor adherence of drug intake among patients with epilepsy is 54%. Those who have where more than 38 years of age had 1. 8 times more odds of having poor treatment adherence. Males had 1. 6 times more odds of having poor treatment adherence. Those who had education up to primary schooling had 1. 4 times more odds of having poor treatment adherence. Those who had taken anti epileptic drugs for more than 2 years had 1. 7 times more odds of having poor treatment adherence. However it is not statistically significant. This study found that a majority of patients with epilepsy have optimal rates of AED adherence. But male patient, age more than 38 years and education up to primary level had more poor adherence.

EP22/380: Semantic skills among adolescents and young adults with drug resistant epilepsy

Sonal Chitnis, Jain K, Jagtap S, Patil S, Kurwale N

Department of Neurology, Bharti Hospital and Research Center and Bajaj Allianz Epilepsy Centre, DMH, Pune, Maharashtra, India

Background: Semantic ability such as word retrieval in oral & written form is critical to succeed in academic, occupation and social domains. Intact lexical system is pre-requisite for word retrieval, literacy skills which are often observed to be impaired in dominant and non dominant hemispheric drug resistant epilepsy patients. Aim and Objectives of the Study: To investigate semantic skills in native Marathi speaking adolescents and young adults with epilepsy and healthy matched controls. Methods: 25 individuals with drug resistant epilepsy and 25 matched controls in the age range of 13-39 years were included in the study. A tool was developed in Marathi to assess semantic skills. MoCA and developed Indian semantic battery- Marathi were administered on all participants. Results and Discussion: There was significant difference in cognitive and semantic skills of individuals with epilepsy and matched controls (p<0.05). No significant difference in semantic skills was observed between TLE and ETLE, right hemisphere and left hemisphere epilepsy, right and left TLE and ETLE (Riva et al., 2005; Condret-Santi et al., 2014). There was moderate degree correlation observed between MoCA scores and scores of Indian semantic battery-M. Effect of literacy was observed on semantics and vice versa. Conclusion: Cognitive – linguistic networks get disrupted due on ongoing seizures. Age of onset, uncontrolled epilepsy, AED, frequency of seizures, literacy are important factors affecting semantic skills. Both right and left hemispheric drug refractory epilepsy reveal poor semantics and cohesion required for adequate learning & communication.

EP23/389: New onset occipital lobe seizures with subcortical T2 hypointensities in magnetic resonance imaging brain: A rare manifestation of nonketotic hyperglycemia

Sandeep Gurram, Prabhu AN, Gorthi SP, Pai AR

Department of Neurology, Kasturba Hospital, Manipal, Karnataka, India

Introduction: Hyperglycemia is a common metabolic cause of seizures. Focal occipital seizures are rare presentations of hyperglycemia. We describe the clinical and MRI findings of patients with non ketotic hyperglycemia (NKH) with occipital seizures as presenting symptom. Methods: We included patients who presented with clinical f/s/o occipital lobe seizures and had typical subcortical occipital hypointense lesions in MRI brain. They were followed up for 1 year after treatment with repeat imaging to look for resolution. Results: Of the 4 patients (3 male and 1 female), all patients presented with multiple episodes s/o occipital lobe seizures with visual aura of colorful circles. Clinical Examination revealed Homonymous hemianopia. All of them were found to have NKH. All had EEG evidence of focal occipital seizures; with secondary generalisation in two. All of them also showed unilateral occipital subcortical T2 hypo intensity on MRI. All of them improved clinically and radiologically after correction of hyperglycemia and with anti-epileptic drugs. Conclusion: Occipital seizures with visual deficits are rare initial manifestations of NKH. They are associated with specific subcortical T2 & FLAIR hypointensities on MRI that are frequently underreported. Hence we need to be aware of this rare clinical and radiological manifestations of hyperglycemia that is easily reversible and treatable.

EP24/390: Cognitive profile in drug-refractory temporal lobe epilepsy with unilateral hippocampal sclerosis

Mukheem Mudabbir MA, Sinha S, Mundlamuri RC, Mariyappa N, Alladi S, Aravind KR, Shivashankar N

Departments of Neurology and Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Objective: To study the cognitive profile in patients with drug-refractory Temporal Lobe Epilepsy (TLE) with unilateral Hippocampal sclerosis (HS) using ICMR cognitive test battery and comparison with age, gender & education level matched healthy controls. Methods: Thirty patients (M:F-17:13, age at onset of seizures-11.77 ± 8.75 years, duration of seizures-16.10 ± 9.61 years) with TLE-HS and 15 healthy age, gender & education level matched controls (M:F- 10:5, age- 28.13 ± 4.76 years) underwent ICMR cognitive test battery (Addenbrooke's cognitive examination-111, Digit forward and backward tasks, Indian Trail Making test A&B, Kolkata verbal learning task (KVLT), Modified Taylor complex figure (MCFT) test & Stick Figure Test copy& recall, Visual Object Naming, Stroop test, Neuropsychiatric inventory and Hospital Anxiety & Depression Scale). Results: The Cognitive scores in ACE -111, Digit Backward task, KVLT delayed recognition, MTCF test early recall, MTCF test delayed recall, Visual object naming was significantly lower in the patient group when compared to control group (P values: <0.05). The scores in Indian trail making test B (in seconds), NPI, NPI Care Giver Distress, HADS Depression, HADS Anxiety are significantly higher in the patient group when compared to control group (P values: <0.05). Conclusion: Patients with drug-refractory TLE-HS have cognitive deficits that extended beyond the medial temporal lobe functions possibly due to the effect of chronic epilepsy on neuronal networks.

EP25/403: Validation of the new International League against Epilepsy 2017 classification for determining seizure type in Indian patients

Sandhya Manorenj, Sagari N, Jawalkar S, Bilal MS, Kumar A, Naaz B

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

Introduction: Epilepsy is a common condition in neurology comprising a number of electro clinical syndromes and seizure disorders of varying known and unknown aetiologies that require variable diagnostic workup, treatment, and obviously have different prognosis. Therefore, for appropriate patient management, the best possible classification system for epilepsy is required. The International League Against Epilepsy (ILAE) is continuously working on this with latest classification given 2017. There is little knowledge about seizure type based on newer classification system in Indian patients. Aims and Objective: To test the applicability of the newer ILAE 2017 classification of epilepsy in determining seizure type in Indian patients, as for appropriate patient management, the best possible classification system for epilepsy is required. Materials and Methods: Prospective data of 155 consecutive patients with seizures, presenting in neurology department was collected from December 2018 to March 2019 and analyzed according to the newer systems of classification of seizures proposed by ILAE in 2017. Results: All 155 patients in age ranging from one year to 72 years with seizures could be classified according to the ILAE 2017 classification system. Focal onset seizure was observed in 33 patients (21.2%) while 122 patients (78.7 %) had generalised onset. Awareness was impaired in 131 (84.5%) patients. Motor onset seizure was observed in 139 patients (89.6%), while non motor seizure included absence seizure, sensory, cognitive and autonomic seizure. Conclusion: Present study showed that all patients could be classified using ILAE 2017 classification system. Generalised onset, predominantly motor type of seizure with impaired awareness was observed.

EP26/410: Eating epilepsy: A late manifestation of left basal ganglia bleed

Surekha Dabla, Bala K

Department of Neurology, Pt BD Sharma Postgraduate Institute of Medical Sciences, Rohtak, Haryana, India

Eating epilepsy is rare forms of reflex epilepsy and worldwide very few cases reported. We report the case of breakthrough eating epilepsy presenting with partial complex seizures since last 6 yrs with a background history of RSA, Left BG bleed with right hemiparesis in the year 2004 for which he remained in ICU for 2 months. His clinical examination suggestive of right hemiparesis with moderate dysarthria. His Interictal VEEG done while having meal which showed clinical and electrographic event. Brain MRI revealed chronic bleed with gliosis in left capsuloganglionic, He was treated with oral levitiracetam and valproate but he improved remarkably with tab clobazam during meals with dietary schedule.

EP27/414: Prevalence of over-weight, obesity and prediabetes is double in people with epilepsy than general population

Sudhindra Vooturi, Potharaj A, Kandula A, Jayalakshmi S

Department of Neurology, Krishna Institute of Medical Sciences, Secunderabad, Telangana, India

Background: Altered metabolic and bio-chemical factors is one of the proposed mechanics for higher cardiovascular mortality and morbidity in people with epilepsy (PWE). We aimed to quantify prevalence of risk factors of cardiovascular disease in PWE and compare with general population. Methods: In this prospective, observational study of PWE with seizure controlled on anti-epileptic drugs, 205 subjects aged 18–60 years recruited. Data on anthropometry, physical activity, sub-maximal exercise test and metabolic parameters (lipid profile, glycosylated haemoglobin, thyroid profile and vitamin profile) was obtained from the participants. A comparison for the above mentioned data in study participants was compared with Indian population average. Results: The mean age of the cohort was 26.45±9.85 years with 117 (57.0%) men. The mean body mass index was 28.75±6.73 and waist-hip ratio of 0.97±0.08. The prevalence of over-weight (28.5%vs12.5%; p<0.001), obesity (44.8%vs21.0%; p<0.001) and pre-diabetes (15.5%vs8.7%; p<0.05) was significantly higher in PWE than general population. Significantly lower fraction of PWE reported regular physical activity of (10.5% vs 46.4%; p<0.001). Additionally, obese PWE had significantly higher LDL (139.37±9.63 vs 100.97±14.96; p<0.01) and HbA1C levels (5.27±0.58 vs 5.11±0.42; p<0.05) than PWE with healthy weight. Conclusions: Prevalence of cardiovascular risk factors like obesity, pre-diabetes and physical inactivity is higher in people with epilepsy than general population. Obesity is also associated with higher LDL and HbA1C in people with epilepsy.

EP28/431: Effect of seizure on glasgow coma scale of patients in intensive care unit

Gursimran Singh, Singh G

Department of Neurology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India

Introduction: Seizures are frequent in neurological intensive care units (ICUs). In various studies, seizures are reported to occur in 3-50 % of patients admitted in neurological ICUs. The cause of seizures in neurological ICU are many and include stroke (hemorrhagic more than ischemic), meningitis, cerebrovenous sinus thrombosis, CNS tumours, aneurysms, AVMs and head injuries. Seizures often produce transient alterations in consciousness because of spread to cortices across both cerebral hemispheres thereby involving the cortical projections of the ascending reticular activating system diffusely. Aims and Objectives of the Study: To determine the effect of seizure on Glasgow coma scale (GCS) in patients with acute neurological insults in intensive care units. Methods: Subjects were enrolled if they had a seizure during admission to a neurological intensive care unit. Their last recorded GCS prior to the seizures was extracted from chart review and GCS monitored prospectively at 2, 4, 8, 24 and 48 hours after the seizure. Information on the age, gender, underlying neurological disorder, prior epilepsy was also noted. For comparison, subjects with matching GCS scores were also monitored for 48 hours to document fluctuations in their scores. The kinetics of GCS change after seizures were charted and their association with several demographic and clinical variables studied. Results: Mean pre-seizure GCS in cases (n=50) was (10? 4) and was similar to controls (n=50) (10±4). Immediately after the seizure, the mean GCS fell to (6?3) and thereafter increased progressively at 2 hours (8?4), 4 hours (9?4), 8 hours (9?4.),12 hours (9?5), 24 hours (9?5) and 48 hours (9±5). However the mean GCS did not return to pre –seizure values even after 48 hours. No deviations were noted in the control group. At the end of 2 hours 37 cases still had deranged GCS, end of 4 hours 26 patients had deranged GCS, at 8 hours 18 patients had deranged GCS, at 12 hours and 24 hours 16 patients and finally at 48 hours 13 patients still had deranged GCS. Conclusion: Seizures in the ICU lead to drop in GCS of those affected and this drop may persist even up to 48 hours. This may confound the assessment of GCS. Limitations: Sample size was small and Non convulsive status was not ruled out by an EEG. Future Scopes: Cases who were diagnosed as autoimmune encephalitis had prolonged unconsciousness, this needs further research work.

EP29/441: Outcome of epilepsy surgery in children with focal cortical dysplasia

Suresh Babu Pasangulapati, Vooturi S, Vadapalli R, Panigrahi M, Jayalakshmi S

Department of Neurology, KIMS, Secunderabad, Telangana, India

Aim: We evaluated the role of non-invasive multi-modality pre-surgical evaluation in determining seizure outcome in children below 16 years with intractable epilepsy due to FCD. Methods: We analysed clinical and demographic data, localization of epileptogenic focus on presurgical MRI, fluoro-deoxy-glucose positron emission tomography (FDG-PET), ictal single photon emission computed tomography (SPECT) and seizure outcome in 104 consecutive children at two years after epilepsy surgery. Seizure outcome was classified according to the Engel classification. Results: The average age at surgery was 10.29±5.15 years with 50 (48.0%) boys. Lesionectomy was done in 74 (71.1%), ATLAH in 11 (10.6%), multilobar resection in 17 (16.3%) children and posterior disconnection in two children. On histopathological examination, Type I FCD was found in 57 (54.8%), type IIa in 26 (25.0%), IIb in 15 (14.4%) and six (5.8%) children had a combination of Type I and II. At two years post-surgery follow-up 69 (66.3%) children had favourable Engels outcome. Clear cut lesion on MRI was observed in 92 (88.5%), and showed a trend of associated with favourable outcome (73.9% vs 41.6%;p=0.09).In the remaining 12 children with subtle MRI, localization in FDG-PET helped opt for surgery in eight (66.7%) and ictal SPECT in four (33.7%) more. All children with clear cut lesion on MRI or localized pattern on PET or ictal SPECT had favourable outcome. Amongst the 12 children with subtle MRI, favourable outcome was observed in five (41.6%). Conclusion: Pre-operative non-invasive multi-modality evaluation achieves seizure outcome similar to invasive EEG evaluation in children with FCD undergoing epilepsy surgery and may obviate the need for expensive invasive EEG monitoring.

EP30/474: Does change in name really changed the game? Prediction of seizure type with recent international league against epilepsy seizure classification - A video EEG study of 50 patients

Swapan Gupta, Bhattad S, Chowdhury D

Department of Neurology, GB Pant Institute of Post Graduate Medical Education and Research, New Delhi, India

Introduction: Recent international league against epilepsy (ILAE 2017) classification was devised for use in the clinics to eliminate the ambiguity and diagnose correct seizure type, which is an important parameter for anti-epileptic drug selection and localization for a potential surgical target. Onset (focal/generalized) and awareness during the event are the two most important components in the new classification. Aims: To classify the seizure type according to recent ILAE classification based on medical history and then compare with the seizure type recorded by the long-term video EEG (VEEG). Materials and Methods: The design was a hospital based longitudinal study conducted from June 2018 to June 2019. Patients were recruited from neurology OPD and epilepsy clinic. Patients of epilepsy of at least 1year duration and minimum of 2 seizures/month eligible for VEEG long term monitoring with reliable witness were included and interviewed by neurology residents trained for at least 1 year. Demographic data, details regarding seizure onset, frequency was collected. Seizure type according to recent ILAE classification was defined. All these patients were then subjected to long term VEEG monitoring after informed consent and minimum of two or more seizures were recorded. All the patients also underwent 3T MRI imaging with epilepsy protocol. Based on these investigations, actual seizure type was documented. The seizure types obtained by history and VEEG data were compared and concordance between the two was noted. Results: Fifty out of total 90 patients screened were included, out of which 35 were males. Mean age was 25 years (5-45 years) and 12 patients were children. On an average, about 3.5 (2-8) habitual seizures per patient were recorded. Three out of 50 patients had more than one seizure type on VEEG recording. Forty-five patients had focal onset seizures, 3 had generalized onset seizures and remaining 2 had psychogenic non-epileptic events (PNES) and combined PNES with focal onset seizures respectively. Out of 46 patients with focal onset seizures, preserved awareness was noted in 3 patients while impaired awareness was noted in 43 that also included 7 patients with partially preserved awareness (PPA). Brief behavioral arrest at the onset before motor manifestations was noted in 4 patients. Concordance between the seizure type by history and VEEG was noted in 56% (28/50). Conclusions: Prediction of correct seizure type with recent ILAE classification is low by the clinical history alone unless supplanted by video EEG information. Although easy to administer in a busy clinic, the recent classification appears oversimplified missing important links like partially preserved awareness and brief behavioral arrest at the seizure onset that are relevant in localization. Limitations: There might be hindrance in historical data owing to education and recall bias. Sample size was small. Future Scopes: Studies with larger sample size should be done. The quest for an ideal seizure classification should continue.

EP31/479: Clinical profile and outcomes of electrographically proven nonconvulsive status epilepticus in critically ill adult cohort from a tertiary care cente

Lakshmi Manasa Sajja, Chaterjee A, Chandran V, Gorthi SP

Department of Neurology, Kasturba Medical College, Manipal, Karnataka, India

Objectives: To study the clinical and electrographic profile in a consecutively collected cohort of adult patients in the intensive care unit with electrographically proven non-convulsive status epilepticus in a tertiary care center in South India and determine factors affecting functional outcome. Methods: This retrospective observational study was undertaken at Kasturba Hospital, Karnataka, from May 2015 to September 2018. Consecutive adult patients in the ICU, whose electroencephalogram fulfilled the Salzburg criteria for NCSE, were included. A structured questionnaire was used to gather relevant data. Results: A total of 65 patients were included in the analysis. Average age of the subjects was 48.16 years with 58% males. Median GCS was 9 (3-15). 26% had prior history of epilepsy. 28% had no convulsive seizures. 52% presented with generalized seizures, 11% and 8% had focal dyscognitive and simple partial seizures respectively. Electrographically, generalized NCSE was seen in 23% and focal in 77%, with continuous ictal activity in 40% of those with focal NCSE. Periodic discharges were seen in 49%. The commonest etiology was acute symptomatic (78%), followed by remote symptomatic (12.3%), idiopathic (8%) and progressive symptomatic (1.5%). Mortality rate was 26%; limited to the acute and progressive symptomatic groups (31%, 100% respectively). Complications were also highest in these two groups. Conclusion: Non-convulsive status epilepticus has a varied clinical profile in the ICU, depending on the underlying etiology. Poor GCS, acute symptomatic etiology and occurrence of medical complications were associated with higher mortality rates, whereas those with remote symptomatic and idiopathic epilepsies were more likely to reach baseline at discharge.

EP32/480: Types and treatment of epilepsy in a rural health centre from South India: A cross-sectional observational study

Khaja shakeeb Ahrar, Jabeen SA, Reddy ST, Mohan J, Bidinger P

Department of Neurology, Nizams Institute of Medical Sciences, Hyderabad, Telangana, India

Introduction: Seizure classifications, choice of antiepileptic drug and drug compliance are the most important determinants for optimal management and outcome of persons with epilepsy. Aims and Objectives: (1) To assess types and aetiology of epilepsy based on ILAE 2017 classification a rural health center prescription data base with a catchment area of 3,50,000 population. (2) To study the drug compliance and response to treatment with four antiepileptic drugs (phenytoin, carbamazepine, valproic acid and clobazam). Methods: This is a retrospective, cross sectional single centre study. Patients information is collected from a prescription based data base from 2010 to 2017 from a single IRHS (Institute of Rural Health Services) clinic in Dokur village of Mahaboobnagar district of Telangana state. Patients of all age groups and both genders who were diagnosed with epilepsy were included. Seizures due to alcohol withdrawal and other symptomatic seizures were excluded. All the patients were treated by a single neurologist using only 4 antiepileptic drugs (carbamazepine, valproic acid, Clobazam and phenytoin). Results: A total of 130 cases fulfilled the inclusion criteria and their data was analysed. Median follow up period was 2 years. Mean age of patients at presentation was 24.4 years; ranging from 3 to 56 years with a median age of 22 years. Mean age of onset of seizures was 14.7 years ranging from 0-53 years. Upon applying ILAE 2017 classification to this cohort, 67 (51.5 %) were of generalized onset seizures, 54 (41.5%) were of focal onset, 9 (7%) were of unknown onset type. In patients with focal onset seizures 34 (63%) were aware & 20 (37%) were unaware of their seizure onset. Epilepsy type classification according to ILAE 2017 classification showed 67 (51.5%) were having focal epilepsy, 32 (24.6%) had generalized epilepsy, 3 (2.3%) had both focal & generalized, 28 (21.5%) had unknown epilepsy. For the third level of classification based on investigations (neuroimaging, Electroencephalogram) 80 (61%) patients had unknown aetiology, 23 (18%) had genetic aetiology, 18 (14%) had structural aetiologies, 5 (4%) had metabolic factors & 4 (3%) had infectious aetiologies. Third level of ILAE classification i.e epilepsy syndromes diagnosis could be made in 23.8% patients among which Juvenile myoclonic epilepsy (45%) was the most common syndrome. Carbamazepine was precribed as first anti epileptic in 88 (69%) patients followed by valproate in 32 (25%), phenytoin in 4 (3%), lamotrigine & clobazam in 1 patient each. Eighty percent of patients under follow up were compliant to treatment prescribed. In our cohort; 94 % patients were seizure free in the follow up with 53% (of total cohort) becoming seizure on first anti epileptic drug. Eight (6%) patients had refractory / drug resistant epilepsy. Conclusion: ILAE classification of seizures 2017 from a single centre is similar to overall profile of seizures and epilepsy in the population. Excellent treatment response was noted with a limited number of antiepileptic drugs. Limitations: Small sample size and being a single centre study.

EP33/484: Neonatal-onset epilepsy: Clinico-etiological spectrum and short-term outcome

Ramesh Konanki

Department of Neurology, Rainbow Children's Hospital, Hyderabad, Telangana, India

Introduction: Neonatal-onset epilepsies are rare but distinct group with significant neurodevelopmental morbidity. The data on etiology and outcomes is scarce.

Methods: Prospective observational study of infants with epilepsy onset within 2 months of life. Results: Forty-two infants included over 12 months period-Males 22 (52%), mean age at onset of seizures 14 days (1-54 days). Encephalopathy at onset in 16 (38%). Consanguinity and family history of epilepsy was present in 24 (57%) and 17 (40.5%). The commonest seizure was focal clonic (73.8%); multiple seizure types seen in 15 (35.7%). Of 27 infants with complete work-up (biochemical/metabolic/imaging/genetic), 24 had confirmed diagnosis-yield of 88.8%: Eleven had structural abnormalities, three had metabolic disorders. Twelve of fourteen infants with suspected genetic etiology had confirmed genetic etiology. At 6 months follow-up, 24 of 36 infants (67%) had either died (3) or had severe developmental delay (21); 4 had mild delay and 8 were normal. Average age at seizure control in infants with mild delay or normal development was 11 months (1month -18 months). Infants required mean of 4 drugs for seizure control (2-8), corticosteroids in 9 and ketogenic diet in 4. Infants with delayed seizure control (p=0.009) and epileptic encephalopathy on EEG (p=0.028) had poor outcome (death or severe disability). Epileptic spasms, polymorphic seizures, age at seizure onset, MRI abnormalities, number of AEDs were not associated with short-term outcome. Conclusion: Neonatal-onset epilepsies have high diagnostic yield, and significant neuro-developmental morbidity.

DP1/79: Neuropsychiatric manifestations in patients with dementia

Thatikala A, Rangalakshmi SG

Department of Neurology, Osmania Medical College, Hyderabad, Telangana, India

Introduction: Neuropsychiatric symptoms (NPS) are commonly observed in patients with dementia. They appear frequently in very early and even prodromal phases of the disease and typically get worse as dementia progresses. The Neuropsychiatric Inventory (NPI) is the most commonly used scale for assessing NPS in cognitive disorders, allowing for the assessment of a broad spectrum of neuropsychiatric symptoms. Currently there is no medication that can cure dementia, but recognizing and treating NPS at the time of diagnosis and throughout the course of the disease could promote the well-being of persons with dementia and their families. Aim: To identify various causes of dementia and their neuropsychiatric manifestations. Materials and Methods: Study period: 2 years. Patients from Outpatient and Inpatient block, Department of Neurology, Osmania General Hospital. Patients with dementia aged >18 years are included. Patients with primary psychiatric illness, drug induced, post traumatic brain injury are excluded. All patients are evalulated with NPI questionnaire. Patients underwent routine blood investigations and CSF analysis, autoimmune encephalitis panel (whenever required), EEG (whenever required), and imaging of brain (CT/MRI). Results: 45 patients with dementia were included. Age of the patients ranged between 38 - 79 years. Majority of them were males (33). Most common cause of dementia was Fronto temporal dementia (18) followed by alzeimers dementia (12), normal pressure hydrocephalus (8). Most common neuropyschiatric symptom with high care giver distress is apathy (32) followed by irritability (18). Conclusion: Identifying neuropsychiatric symptoms at the time of diagnosis will help initiating prompt treatment at the earliest.

DP2/127: To study etiology and clinical profile of dementia

Sugali Rajesh Naik, Thangaraj

Department of Neurology, Thanjavur Medical College and Hospital, Thanjavur, Tamil Nadu, India

Introduction: Dementia is usually a disease of elderly and is characterised by progressive loss of memory and other cognitive domains with impairment of dailyliving activities. Aims and Objectives: To study etiology and clinical profile of dementia. Materials and Methods: 40 patients who satisfied DSM V Criteria for dementia were evaluated for etiology and clinical profile by neuropsychological assessment, neuroimaging and biochemical investigations and results were documented over study period. It's a cross sectional observational study. Results: In our study of 40 cases (60%) are male and (40%) are females. Early onset dementia seen in (35%), late onset in (65%). Alzheimers accounted for 50%, vascular dementia 20%, frontotemporal dementia 17.5%, dementia with lewybody 5% and 7.5% other causes. Both in Early and lateonset, Alzheimers is predominant, next seen is Frontotemporal dementia in young and vascular in lateonset dementia. Amnestic Alzheimers constitutes 80%, logopenic, frontal variant each 10%. In vascular dementia cortical infarcts seen in 50%, small vessel disease in 25%, strategic and multi infarct each in 12.5%. In frontotemporal dementia behavioural variant seen in 71.4%, semantic and progressive aggramatic nonfluent aphasia in 14.2% each. Dementia with lewy body constitutes 5%, Parkinson's Dementia, infective and lysosomal storage disorder in 2.5%. Episodic and recent memory impairment common in amnestic Alzheimers. In frontal variant Alzheimers executive dysfunction is predominant where in behaviour variant FTD behavioural symptoms predominant. Conclusions: Our study showed that Alzheimers is predominantly seen type of dementia in both early and lateonset, next was vascular dementia in lateonset and frontotemporal dementia in earlyonset. Cortical dementias and irreversible dementias are most common.

DP4/170: A study on cognitive impairment in chronic kidney disease patients

Prasanth SR, Shaji CV, Muhammed, Kabeer KA

Department of Neurology, Government TD Medical College, Alappuzha, Kerala, India

Introduction: Cognitive impairment (CI) is more common in patients with Chronic kidney disease (CKD) than in the general population. But it remains poorly recognized clinically. CI potentially affects multiple areas of patient care and quality of life. Aims and Objectives: (1) To assess the cognitive profile in CKD patients using Montreal cognitive assessment scale (MoCA). (2) To assess relationship between degree of cognitive impairment and stages of CKD. Materials and Methods: This was a descriptive study done in CKD patients aged 18 yrs to 60 yrs attending Departments of Nephrology and Neurology, Govt. TD Medical college, Alappuzha during the study period June 2017 to December 2018. Data was collected using a pre-designed proforma and cognitive assessment was done using MoCA. Results: 60% patients were males. Most common age group was 50-59 years (56%). 64% patients had either stage 4 or 5 CKD. 50 % of patients were on hemodialysis. Out of these 25 patients, 19 had stage 5 and 6 had stage 4 CKD. 52 % of patients (24% males, 28% females) had abnormal MOCA score (ie, < 26) suggestive of CI. Most common abnormality in cognition was for word fluency (in 78%) followed by executive function (in 56%) and abstraction (in 54%). MoCA score of patients on HD Versus non–HD group, showed CI more in HD group (p value: 0.009). Conclusion: Cognitive impairment (CI) is common in CKD patients, especially in HD patients. MoCA testing should be used, as most of the patients had only non – amnestic type of CI. MoCA score decreases with decline in eGFR.

DP5/200: Degenerative dementia subtypes in a tertiary care hospital

Shah G, Sikariya K, Shah S, Shah S, Patel M, Joshi P

Department of Neurology, Smt. NHL Municipal Medical College, Ahmedabad, Gujarat, India

Aim: To study the demographic and clinical pattern of degenerative dementia subtypes in a tertiary care hospital in Western India. Inclusion Criteria: All patients above 40 yrs diagnosed with dementia; according to DSM V criteria were included. Exclusion Criteria: Secondary causes of Dementia. Materials and Methods: This is a prospective study of dementia patients, presented at a tertiary care hospital from 2017-2019. 39 patients were further classified into the dementia types on the basis of standard criteria. Results: In our cohort, M:F ration was 8:5.The most common type of dementia was Alzheimer's (AD) (12/39), followed by Fronto-temporal (FTD) (10/39) and vascular (VaD) (7/39) subtype of dementia. FTD has earlier age of onset (mean 52), as compared to all other degenerative dementia (7th decade). Initial symptoms of memory impairment were common with AD, VaD and mixed dementia. Behavioral manifestations; especially apathy was commonest presenting symptom of FTD. 2 patients with rapidly progressive dementia were diagnosed to suffer from CJD; while one patient had DLBD. Along with routine investigation, patients under went MRI Brain with dementia protocol which correlated with clinical findings. Conclusion: In this small study, Alzheimer's disease was the most common dementia, followed by FTD. Mean age of onset of FTD was a decade earlier than other dementia. Degenerative dementia should also be kept in differential diagnosis of rapidly progressive dementia.

DP6/211: Subjective and objective assessment of occurrence of mind wandering in Indian population

Vidya Subhash, Narasimhan RL, Manikavasagam J, Shunmugasundaram K

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

Introduction: Mind wandering refers to disengagement of attention from the external environment and generation of thoughts unrelated to task at hand. Evidence suggests that mind wandering typically occurs at a significant cost to performance. But recent evidences suggests that it plays a crucial role in autobiographical planning and creative problem solving. Aim: (1) To obtain subjective as well as objective assessment of mind wandering. (2) To study the occurrence of mind wandering in patients and compare the frequency with that of normal controls. Materials and Methods: Patients admitted in the wards of Madras Institute of Neurology and Healthy volunteers and were taken as cases and controls respectively. They were assessed subjectively with MAAS (Mindfulness awareness assessment scale). Objective assessment was done with breath count task done for 10min. Pearson correlation were used to examine the relation between subjective assessment (MAAS) and breath count task (BCT) performance. All analysis were performed with IBM SPSS Software. Results: Our study includes found that patients have more frequent occurrences of mind wandering than normal individuals. Frequency of occurrence was less in individuals with high education status

Conclusion: We have conducted a study to validate breath count task as an objective measure of trait mindfulness.

DP7/228: Clinical correlates of leukoaraiosis with special emphasis on cognitive function

Kalluri Chinna Rao, Thacker AK, Singh AK, Maurya PK, Kulshreshtha D, Qavi A

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

Background: In india the correlates of leukoaraiosis (LA) have not been widely studies. LA appears on MRI Brain as hyperintensities on fluid -attenuated inversion recovery imaging. This study was designed to invesigate the factors which correlate with LA. Aim: To evaluate correlation of leukoaraiosis changes on MRI Brain with clinical features especially cognitive function, executive function and gait impairement. Materials and Methods: We included patients above 18 years old with LA on MRI who were given consent for study included and LA severity graded on the basis of Fazekas scale. The patients who were not given consent, patients with LA with infarct size more than 2 cms (suggesting large vessel disease) and patients with other white matter changes which mimic LA (CADASIL, MS, cerebral Amyloid angiopathy, HIV) were excluded. Results: Leukoaraiosis was common and under rated cause of disability. Different presentations include congnitive decline specially focussed on exexcutiion problems, gait disturbance, recurrent falls, speech difficulty (dysarthria), bladder, bowl disturbances and increased risk of stroke. The comorbidities include Diabetes milletes, Hypertension, dyslipidemia, smoking, tobacco use, alcohol consumption, hyperhomocystenemia, previuos stroke, ischemic heart disease, atrial fibrillation.

DP8/300: Nature of cognitive deficits in Parkinson's disease with and without psychosis

Mohit Gothwal, Hegde S, Sundar SA, Pal PK

Departments of Neurology and Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Introduction: Psychosis is one the debilitating non-motor symptoms of PD. Early stages of PDP is associated with minor hallucinations & illusions with preserved insight. In later stages, PDP is characterized by well-formed visual hallucinations (VH), non-VH & delusions with often insight affected. PDP has also been associated with more cognitive deficits (CD). These deficits affect day to day activities such as remembering their medication, focusing on the task, among others. Early detection of CD can help in identifying PD population which are at risk to develop PDP. Aims: Present study investigated nature of CD in PD patients with & without psychosis using neuropsychological method. Materials and Methods: Cross-sectional study Sample: 2 groups (PDP, n=15; PD, n=15) were group matched & assessed on clinical variables: duration of disease, UPDRS) Part-III, Levodopa daily dose & compared their performance on NIMHANS neuropsychology battery (Rao et al., 2004). Assessment measured performance across various cognitive domains. Inclusion criteria: Primary diagnosis of PD (MDS clinical diagnostic crtieria, 2015); age from 40-65 years; both gender; min. edu of 8 years; right handed; H&Y stage 1 to 2.5. & diagnosis of PDP in last 12 months (acc. to NINDS-NIMH criteria, 2007). Exclusion criteria: Dementia; severe anxiety and depression; individuals who had underwent DBS. Results: PDP found to have more severe CD in domains such as attention, executive, memory & visuoconstruction. Conclusions: PDP in comparison to PD is associated with severe CD.

MDP1/18: Scholastic performance in wilson disease patients-follow up of four patients

Ranganathan P, Pushkar P

Department of Neurology, Pazhani Neuro Centre, Chennai, Tamil Nadu, India

Background and Aims: Cognitive impairment in Wilson disease patients with neurological presentation is a known fact. Apart from the neuropsychological assesment, how these patients performed scholastically over a period of time is interesting to observe. Methods: Four Wilson disease patients, three male and one female with neurological presentation were followed up. Their presentation at the time of diagnosis and improvement in scholastic performance after treatment were assesed. Results: Age at neurological presentation of three male patients were 11, 13, 14 and female patient was 16. All of them had mild athetoid movements, bradykinesia and drop in the scholastic performance. Two male patients had behavioural disturbance in the form of anxiety and depression. The urine copper level was increased, ceruloplasmin was decreased and KF ring was preesent in all the four patients. MRI brain showed findings suggestive of Wilson disease. All the patients were treated with d penicillamine. They symptomatically became better. Two of the male patients who had behavioural disturbance needed psychiatrist help. Two male patients completed BE and in a good job now. One male patient completed MA. The female patient dropped out from the college. After treatment, she joined back the college and completed her BA. Conclusion: Scholastic performance in Wilson disease patients improves with treatment. Counselling in the form of constant motivation to complete the studies has additional benefit.

MDP2/43: Does gender difference have a role in determining quality of sleep in Parkinson's disease?

Mahale R, Ravi Yadav, Pramod Pal

Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Background: The occurrence of sleep disturbances in Parkinson's disease (PD) is common. Gender difference in non-motor symptoms of PD including sleep disturbance do occur. Objectives: To determine gender difference in patients with PD in relation to the quality of sleep. Methods: The study included 156 patients with PD with males with PD (n=119) and females with PD (n=37). The quality of sleep was assessed using Pittsburgh sleep quality index (PSQI), Parkinson's disease Sleep Scale (PDSS) and Epworth Sleep Scale (ESS). Results: Females with PD had higher frequency with insomnia (56.7%) as compared to males with PD (44.5%) (p=0.05), nightmares (16.8%) as compared to males with PD (32.4%) (p=0.04), greater PSQI component score of habitual sleep efficiency (p=0.008), sleep disturbance (p=0.03) and daytime dysfunction (p=0.02), higher global PSQI score (p=0.03), higher percentage of patients with global PSQI score >5 (64.8%) than males with PD (41.1%) (p=0.01), suggesting poor quality of sleep in females with PD. Higher total ESS score (p=0.03), higher frequency of females with PD with total ESS score >10 (45.9%) than in males with PD (26.9%) (p=0.02), and lower total PDSS score (p=0.009). Conclusions: Females with PD had a poor quality of sleep with higher frequency of insomnia, nightmares, and daytime sleepiness.

MDP3/50: Clinical profile of nonmotor symptoms in early Parkinson's disease patients in tertiary care centre in South India

Krishnasree KS, Ashok VP, Menon D, Rajan MD, Syamlal S, Suresh Chandran CJ

Department of Neurology, Kerala Institute of Medical Sciences, Thiruvananthapuram, Kerala, India

Introduction: Nonmotor symptoms are underrecognised in Parkinson's disease (PD) patients from early stage. Objective: To determine proportion of patients with NMS in early PD and to find an association of NMS with disease severity and motor disability. Methods: A cross - sectional prospective study of 100 patients who presented to Neurology Department from June 2016 to November 2017 with Idiopathic PD within 5 years of symptom onset were studied. NMS questionnaire was used as primary screen for nonmotor symptoms. Other methods of assessments included measures of motor disability (MDS-UPDRS), disease severity (modified Hoehn-Yahr staging), depression (Geriatric depression scale), global cognitive function (Minimental status examination and Montreal cognitive assessment). Results: We studied 100 patients out of which all patients except 2 reported at least one NMS. The most common NMS in the study were sleep symptoms (50%) followed by urinary symptoms - urgency (49%), nocturia (47%), memory disturbances (45%), orthostatic hypotension (44%), apathy (40%), sialorrhea (38%), difficulty concentration (36%) and constipation (33%). Females showed more depressive symptoms and sexual dysfunction was more seen in males. There was significant correlation between NMS with disease severity and motor disability. Nearly equal number of patients consumed levodopa alone (40%) and levodopa with dopamine agonist combination (39%). Those on higher doses of levodopa had increased attentional problems, restless leg syndrome and loss of ability to taste or smell. Conclusion: The study identified that NMS are very common in early PD. Careful characterization of NMS is important for understanding disease evolution from premotor to early motor phase.

MDP4/67: Musculoskeletal issues in Parkinson's disease

Anand KS, Agrawal AK

Dr. Ram Manohar Lohia Hospital, Post Graduate Institute of Medical Education and Research, New Delhi, India

Aims and Objectives: To assess the prevalence of musculoskeletal problems and type of musculoskeletal conditions in Parkinson's disease (PD) patients. Background: Musculoskeletal system involvement causes significant morbidity and contributes to poor quality of life in Parkinson's disease. They are often overlooked by both patients and treating physicians causing under-evaluation and under-treatment. The present study evaluates the prevalence and type of musculoskeletal problems in PD patients. Methodology: A total of 100 PD patients were recruited and staged by UPDRS III, V and VI. Musculoskeletal assesment included focused history and thorough physical examination. Results: n=100, mean age=62.70±11.18 years, age range=38-85 years. M:F=73:27. 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±SD of UPDRS III, V and VI scores were 15.01±7.55, 2.39±0.72 & 79.57±18.70 respectively. Prevalence of musculoskeletal problems was 53%. Most common involved site was lower back followed by shoulder and knee. Common conditions include low back pain, frozen shoulder, osteoarthritis and osteoporosis while cervical spondylosis, fractures and rotator cuff injury were less common. Older age and higher scores on UPDRS III, V and VI were more associated with musculoskeletal problems (p < 0.05). Conclusion: In the present study 53% of PD subjects had musculoskeletal problems which require proper evaluation and neurorehabilitation to reduce morbidity and improve quality of life.

MDP5/69: A rare and interesting drug induced movement disorder

Manikinda J, Yada PK, Surath M, Kaul S, Sattaluri SJ, Varalakshmi EA, Babu S

Department of Neurology, KIMS Hospital, Hyderabad, Telangana, India

Introduction: Serotonin syndrome (SS) is a life threatening condition caused by serotonin toxicity, and usually involves a combination of drugs that increase serotonergic transmission. Case Report: 58 years male patient, diabetic and hypertensive, on levodopa + carbidopa 125mg thrice daily for complaints of tremors, slowness of gait and activities since 6 months. 3 months back during OP review levodopa was stopped and patient was started on Amantadine and Rasagiline. Patient developed tremors involving both upper limbs and head after 1 month, along with delusions, hallucinations, memory disturbances and sleep disturbances. 1 month back as symptoms persisted, Escitalopram and clonazepam with amitriptyline was added to above medication. Olanzapine was also added when tremors and restlessness increased severely 10 days back. Patient's tremors increased further and he developed severe restlessness, irrelevant talk, sweatings and was brought to KIMS. On examination in ER patient had tachycardia, mild hypertension and was aggressive, delirious with irrelevant talk. Tremors involving head, limbs, chin tremor and myoclonic jerks. Patient was admitted to MICU, intubated and sedated with midazolam infusion. All offending medications were stopped and Syndopa plus was started. CPK was 839 U/L. Considering possibility of Serotonin syndrome, Cyproheptadine loading dose of 12 mg followed by 2mg every 2 hourly given and maintained on 8mg 6 hourly. Patient improved symptomatically and was discharged. Conclusions: Incidence of serotonin syndrome is rising, reflecting the growing number of serotonergic drugs available and the increased use of these agents in clinical practice.

MDP7/133: Nonmotor symptoms in patients with parkinsons disease-an observational study

Bulli Naveen Kumar M, Yada PK

Department of Neurology, KIMS Hospital, Hyderabad, Telangana, India

Aims and Objectives: To assess the Impact of disease duration, severity of disease, MS and NMS, on HR-QoL. Methodology: MS, NMS and HrQoL will be assessed.Impact of disease duration, severity of disease, MS and NMS on Hr-QoL will be assessed. Results: In total, 40 PD subjects were enrolled in this cross sectional study. The mean age of all patients was 55.93/-9.29 years. Mean duration of PD symptoms was 4.6+/-2.84 years, and H&Y stages ranged from 1 to 4. The mean NMSS score was 20.55, with a range from 1 to 80. There was a significant correlation between NMSS and UPDRS III score (rS = 0.622, P <0.001; and H&Y staging (rS = 0.502, P = 0.001;. Significant correlations were also observed between PDQ-39 SI and the disease duration, PDQ-39 SI and disease severity that was measured by the H&Y scale, and PDQ-39 SI and MS that were measured by the UPDRS III (rS = 0.487/0.614/0.685, P = 0.001/<0.001/<0.001). Stepwise multiple linear regression with Hr-QoL as the dependent variable showed that NMSS explained more of the variability in Hr-QoL than UPDRS III suggesting that NMS has strong association with lower Hr-QoL. Conclusion: We found that 100% of our PD patients have NMS with significant effect on Hr-QoL along with MS, So overall evaluation of PD patients should include MS and NMS, focusing beyond the conventional MS to improve the Hr-QoL.

MDP8/137: Sleep disorders in early parkinson disease: A comparative study

George J, Sasidharan S, Chandran S

Department of Neurology, Kerala Institute of Medical Sciences, Thiruvananthapuram, Kerala, India

Introduction: Sleep disorders are now recognised as common nonmotor symptoms in Parkinson′s disease (PD) significantly impacting on patient′s life. During the prodromal phase of PD, patients often demonstate sleep alterations. The aim of this study is to evaluate the incidence and types of various sleep disorders in early Parkinson′s disease. Materials and Methods: A prospective comparative study of 31 patients with Idiopathic Parkinson's disease and 40 age and sex matched controls without PD was done in KIMS Hospital, Trivandrum, Kerala. Sleep disorders were assessed using various questionnaires. Results: Majority of patients and controls were in the age group between 60 and 70 years. The average disease duration was 3 years. The prevalence of sleep disorders among patients was 77% compared to 22% among controls. The most common reported sleep problem was sleep initiation problems (45% among cases and 7.5% among controls). Sleep maintenance problems were also noticed among 41% patients and 5% of controls. EDS and RBS were found in 16% and 32% of patients respectively. None of the controls had EDS or RBD. Out of the 31 patients, 52% were of tremor dominant phenotype whereas 48% were of PIGD phenotype. Sleep disorders were more prevalent in tremor dominant phenotype (88%). Conclusion: Sleep disorders are common in early Parkinson's disease. Therefore early intervention to identify sleep disorders is required in every case of PD, which can improve the quality of life of the patient.

MDP9/143: Imaginary cues in freezing of gait in patients with Parkinson's disease

Prabaharan Umachandran, Lakshmi Narasimhan Ranganathan, Manikavasagam J

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

Background: Freezing of gait (FOG) is a disabling feature of Parkinson's disease (PD). Several studies have proven that implementation of visual, auditory cues have improved FOG. Objective: Our aim is to evaluate the effect of imaginary cues in FOG in patients with Parkinson's disease. Methodology: 24 patients PD with freezing of gait were included in the study. Patients were asked to perform the 50 feet walk test. They have to walk 25 feet, turn around, and walk back 25 feet. The number of freezing episodes and the time taken to walk 50 feet were measured. The 50 feet walk test were performed again by imagine themselves that they are riding a bicycle. The effect of test-retest variability was assessed. Results: Time taken to walk without cues was 52.45 ± 7.84 and with cues was 46.59 ± 8.51 seconds. The number of FOG without cues is 2.12 ± 0.48 and with cues was 1.6 ± 0.71. There is statistically significant difference (p<0.05) between before and after imaginary cues. Conclusion: This study reiterates the fact that such a simple measure that requires no special instruments, can improve the mobility & the quality of life in PD. Further studies are required to assess sensitivity/specificity.

MDP10/185: Profile and severity correlation of ataxia patients in a tertiary care institute in Eastern India

Subhas Bhuin, TN Kundu, Pandit A, Ganguly G

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

Introduction: The clinical profile and severity correlation of ataxia patients in our population is still varied. This study is taken up to explore this aspect. Methods: A cross-sectional study was conducted at XXX. Clinical parameters with severity correlation (SARA scale) in selected patients was determined. Results: In 188 patients, 127 had cerebellar ataxia (SCA 2, 3, 1, 6, 12, MSA C, Wilsons disease), 42 had sensory ataxia mainly Sensory ataxic GBS, CIDP variant (CISP), Sjogrens and 19 had Mixed cerebellar sensory ataxia (Multiple sclerosis, Vitamin E deficiency). Cerebellar ataxias had higher disability at presentation (mean SARA score- 21.87) than mixed ataxias (SARA – 19.68). Increase in SARA score was seen more in mixed ataxias (3.64) than cerebellar (3.01). Disability progression is more in MSA C and SCA 2 has lesser degree of progression (despite highest SARA score- 28.5 at presentation). Conclusion: MSA C, SCA, Multiple sclerosis are majority in respective ataxia groups. Maximum disability and progression is seen in MSA and SCA (SARA scale). Chances of early detection and knowledge about progression of ataxia spectrum in our population is attempted. Limitations: Further follow up of the patients with ataxia required to get a clear insight. Further Scope: Early diagnosis and management strategies can be validated for better disease management and prevention of deterioration to make the otherwise debilitating ataxia disorders a preventable one.

MDP11/186: Effect of low frequency (1HZ) repetitive transcranial magnetic stimulation on levodopa induced dyskinesias in parkinsons disease

Varun Reddy Gundluru, Goyal V, Srivastava MVP, Srivastava AK, Singh MB, Vishnu VY, Rajan R

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

Low frequency (1Hz) repetitive transcranial magnetic stimulation (LF-rTMS) over the supplementary motor area (SMA) can be tried as an alternative approach for the reduction of levodopa induced dyskinesias (LIDs) in parkinson's disease (PD) patients, in whom deep brain stimulation is contraindicated. This was a pilot, investigator initiated, single blinded, parallel group, randomized trial including patients with PD and disabling LIDs. Participants were randomized into real stimulation (3000 and 2000 pulses) and sham stimulation groups, given over 4 consecutive days. 30 participants were recruited in this study with 10 participants in each group. There was a significant reduction in Unified Dyskinesia rating scale part-3 and 4 in 3000 pulses group one day post rTMS (27.1 ± 7.82 to 15.7 ± 6.11; t=3.63, p=0.001;s). Unified parkinson's disease rating scale (UPDRS) part-4 showed a significant reduction in 3000 pulses group (8.3 ± 3.16 to 5.40 ± 1.50, t=2.62, p=0.017;s). Duration of daily LIDs percentage showed a reduction in both the real stimulation groups during the period of stimulation, but the sham group did not show any reduction. This reduction was significant in 2000 pulses group (64.4% ± 9.23 to 45.8% ± 10.8, F=6.35, p=0.014, s). This was not maintained a week after stimulation. No adverse effects were seen in any group. LF-rTMS over SMA is a safe, effective method for reducing LIDs in patients with PD and disabling LIDs with no long lasting effects.

MDP12/197: A case of huntignton's with corticospinal involvement

Vignesh Karunakaran, Saravanan S

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

Huntington's disease is an autosomal dominant neuro degenerative disease characterized by chorea, cognitive dysfunction and mood disturbances. The mean age of usual onset is 35-42years. We report a 26 Year old female who presented with six years history of insidious onset progressive movement disorder characterized by semipurposeful, non stereotyped, non rhythmic, irregular involuntary movements of all 4 limbs including trunk and which also involved the face in the form of excessive staring, frowning and facial grimacing. She also had truncal dystonia, positive jack in the box, milkmaid sign and piano sign. The patient also had associated history of depression with suicidal ideation. She had a strong family history of suicides. Her father and her paternal aunt committed suicide in her third decade though none of them had similar movement disorders. On examination she had personality changes in the form of chronic irritability, adamant behavior and suicidal ideas. Over past three months she had developed spastic weakness, exaggerated deep tendon reflex with bilateral plantar extensor and spastic gait. Her complete blood count, lipid profile, level was normal. MRI Brain showed bilateral caudate atrophy. Her genetic study showed Huntington gene mutation detecting 17CAG gene repeat in one allele and 56 CAG repeat noted in another allele. We report the case because Huntington disease may present with spasticity during the disease progression. Spasticity related to CAG repeat length in the Huntington's gene.

MDP13/199: Clinical spectrum of spinocerebellar ataxia at tertiary care hospital

Andelwar S, Sumra M, Shah S, Patel M, Shah S, Joshi P

Department of Neurology, Smt. NHL Municipal Medical College, Ahmedabad, Gujarat, India

Aim: To assess the clinical profile of spinocerebellar ataxia presenting to our hospital. Inclusion Criteria: All the cases of spinocerebellar ataxia diagnosed on the basis of clinical assessment, family history and genetic study. Materials and Methods: This study is a retrospective review of patients with spinocerebellar ataxia, presenting to tertiary care hospital from January 2017 to May 2019. Detailed clinical history and examination along with laboratory investigations, neuroimaging, electrophysiological study and genetic testing were done. Results and Discussion: Total of 16 patients met the inclusion criteria. There was male preponderance with M: F of 13:3. Mean age at presentation was 34.7 years. Genetic testing was positive in 6 (37.5%), of the 16 patients. Out of these positive cases, 4 (25%) were SCA 2, 2 (12.5%) were SCA 1. SCA 3 was suspected in 4 patients based on clinical assessment, but genetic testing ruled it out. In our cohort 8 had positive family history. History of consanguinity was present in 3 patients, 2 of which were SCA 1 positive. Abnormal saccades and pursuits were seen in 44%; out of which, 2 patients were SCA 2 CAG repeat positive. 4 patients had peripheral neuropathy based on NCS, half of which were SCA 1 positive. 8 patients had pyramidal tract involvement; of which 1 was SCA 2 positive. Conclusion: In our study SCA2 was the most common genetically proven cerebellar ataxia, which is in concordance with other studies done in India.

MDP14/218: Sialidosis I without cherrryred spot and magical response to perampanel - A case report

Senthilkumar Vajramanickam, Kiruthika Sivaraj

Department of Neurology, SKS Hospital, Salem, Tamil Nadu, India

Introduction: Sialidosis type I is an uncommon cause for progressive myoclonic ataxia. We hereby report a young female who presented with progressive myoclonic epilepsy and was given a grave prognosis. Genetic testing helped to fix the diagnosis and after perampanel patient showed a dramatic response. Case Report: A 22 year old female born to III degree consanguinity presented with progressive ataxia, myoclonus, seizures, proximal weakness for 5years. She was diagnosed as a case of progressive myoclonic epilepsy and given a grave prognosis. On evaluation she had pancerebellar signs, action myoclonus, proximal weakness, recurrent seizures. She had normal cognition and required support to walk. Her investigations showed normal pyruvate, lactate, liver function, ANA profile. MRI brain and NCS were normal. VEP and SSEP showed giant potentials. Fundus was normal. Muscle biopsy, axillary skin biopsy were normal. She had increased TSH with normal anti-tpo antibody. Genetic testing showed a positive homozygous variation in exon 5 of NEU-1 gene suggestive of sialidosis. She was started on perampanel and there was a dramatic response in the form of cessation of seizures, marked decrease in action myoclonus and improvement in gait. At the dosage of 4mg bid she is now independent, looking after her children, house hold work and seizure, myoclonus free. Conclusion: This case is presented for following reasons. All PMEs doesn't carry poor prognosis. Genetic panels are helpful in syndromic diagnosis, Sialidosis can present without cherry red spots, Perampanel is a wonder drug for patients with PME/PMA.

MDP15/247: Neuropsychiatric manifestations and caregiver burden in Parkinson's disease

Abhishek Juneja

Department of Neurology, Dr. Ram Manohar Lohia Hospital, New Delhi, India

Background: Parkinson's disease (PD) is a neurodegenerative disorder with significant impact on the quality of life of patients and caregivers. Neuropsychiatric symptoms (NPS) are highly prevalent in patients with PD. The present study aims to study the association of neuropsychiatric symptoms and caregiver burden in PD. Methods: 80 patients of Parkinson's disease and their primary caregivers who fulfilled inclusion and exclusion criteria were recruited for the study after institutional ethical clearance. Diagnosis of PD was established using UK Brain Bank criteria; severity/staging of Parkinson's disease was done by Movement Disorder Society - Unified Parkinson's disease rating scale (MDS-UPDRS-III). The neuropsychiatric evaluation was done based on Neuropsychiatric Inventory-Questionnaire (NPI-Q). Caregiver burden was assessed with the Zarit Caregiver Burden Inventory (ZCBI). Results: 80 patients of Parkinson's disease with mean age of 61.48 ± 6.71 were enrolled in the study. 93.8% patients were on levodopa therapy in addition to other antiparkinsonian drugs. 77.5 % patients had a Hoehn and Yahr stage of 2. An equal number of primary caregivers were enrolled. Caregiver burden was significantly related to the age of the patient (p value <0.001), duration of illness (p value < 0.007), H & Y stage of Parkinson's disease (p value < 0.000) and total NPI score (p value < 0.000). While there was no significant relation found between caregiver burden and age of caregiver or type of relationship with patient. Conclusions: Caregiver burden in PD is high and can have impact on quality of life of patients and caregivers.

MDP16/272: Effect of low frequency (1HZ) repetitive transcranial magnetic stimulation on choreiform movements in Huntington's disease

Varun Reddy Gundluru, Goyal V, Srivastava MVP, Srivastava AK, Singh MB, Vishnu VY, Rajan R

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

Huntington's disease (HD) is a neurodegenerative disorder with chorea as the major disabling motor symptom. This is the result of cortico-striato-thalamocortical dysfunction that results in excess movements. Repetitive transcranial magnetic stimulation (rTMS) has the potential to affect these pathways and reduce abnormal movements. This was a pilot investigational study including HD patients with disabling choreiform movements. Low-frequency rTMS (1Hz) of 1800 pulses were given over supplementary motor area (SMA) for four consecutive days. Primary outcomes were the change in the Abnormal involuntary movement scale (AIMS) and Unified Huntington's disease rating scale (UHDRS) chorea & bradykinesia items post rTMS on day-5. Secondary outcomes were change in the AIMS and UHDRS scores at various time points daily after rTMS (0 min, 15 mins, 30 mins, and 90 min). There was no significant difference in AIMS (26.6 ± 7.61 to 28.0 ± 7.07; t=0.42, p=0.67; ns) and UHDRS scores post rTMS (20.7 ± 5.73 to 22.5 ± 5.13, t=0.74; p=0.46, ns), one day after the last rTMS session. During the days of rTMS, there was no significant reduction in AIMS and UHDRS at any of the time points, but there was an insignificant reduction that was maintained until about 30 mins following rTMS. No side effects were observed in any of the patients. Low-frequency rTMS (1Hz) over the SMA does not have any clinically meaningful effects on the choreiform movements in HD, despite being a safe, non-invasive procedure.

MDP17/276: Heterogeneity in IPD patients and OCT in parkinsonism patients

Shubana Ashraf, Verma M

Department of Neurology, Government Super Speciality Hospital, Srinagar, Jammu and Kashmir, India

Objective: To investigate the heterogeneity in IPD patients in a data driven manner in patients presenting to Indraprastha Apollo hospital between September 2014 to June 2016. To perform OCT in different parkinsonism patients (Typical and Atypical). Methods: Data on the demographic (including the age of onset and duration of disease), clinical profile (motor and non motor symptoms), cognition, medication intake, response to treatment, depressive symptoms were collected from 80 patients who were categorized as IPD and Parkinson plus patients. Motor symptom evaluation was done with UPDRS which was applied in off stage of the patients. UPDRS 3 was repeated in the on stage. Disease severity was assessed as per the total UPDRS score in the off stage. Rate of total disease progression was calculated as total UPDRS score divided by disease duration. Patient's tremor score and non tremor score was calculated using UPDRS scale variables. Motor phenotype was assessed by the ratio of total tremor score to the non tremor score. The subjects were divided thus into PIGD/NTD subtypes whose tremor /Non tremor ratio was less than 1.Those with a ratio more than 1.5 were classified as tremor dominant subtypes. Those with a ratio between 1to 1.5 were classified as intermediate subtypes. Non tremor symptoms were assessed using MMSE scale for cognition, HAMD scale for depression. Results: The analysis revealed five main subgroups of IPD patients that included, 1.Non tremor dominant patients with the highest cognitive impairment, thought disorder and apathy score, 2.motor phenotype variants with highest tremor score, 3.Patients with highest progression rate, 4.young onset Parkinson disease patients, 5.Non tremor dominant patients with highest severity and HAMD score. The optical coherence tomography performed in IPD patients and in Parkinson plus disease patients revealed significant degeneration of the retinal fibre layer in both the groups. However, there was no statistically significant difference in optical coherence tomography findings between the two groups.

MDP18/284: The enigma of curved women: Managing camptocormia in clinical scenario

Dhananjay Gupta, Javali M, Mehta A, Acharya P, Pradeep R, Srinivasa R

Department of Neurology, Ramaiah Medical College, Bengaluru, Karnataka, India

Introduction: Camptocormia is a rare, disabling neurological condition, characterised by involuntary, non-fixed, forward bending of spine while sitting, standing or walking with need of walking aids. It completely disappears in supine position and is known as “curved or bent spine syndrome.” Due to myriad causes, a detailed structured evaluation is necessary. We discuss two such women who presented with progressive forward bending while walking and had different causes for the same. Case 1: The first, a 40-year-old lady presented with progressive walking difficulty with forward bending after walking for few steps for 5 months prior to presentation. She also complained of difficulty in climbing stairs and recent difficulty in gripping objects. On examination, she was noticed to have proximal leg weakness (MMRC grade 4/5) with mild hand grip weakness. There was wasting of temporal- masseter and percussion myotonia was noticed in tongue. She was noticed to have difficulty in opening her hand after clenching her fist tightly. While walking, she was noticed to have progressive forward bending of throcao-lumbar spine. Baseline blood tests were normal. A typical “dive bomber” sound was heard in needle EMG, suggestive of myotonia. Hence, a genetic analysis was performed, which was positive for DMPK mutation, diagnostic for Myotonic dystrophy 1. Case 2: A 60-year old lady, presented with progressive slowness of walking with changes in posture for 5 years. On examination, patient was found to stand and walk with forward flexion of the trunk, a posture which disappeared completely while sitting and lying down. Examination of the cranial nerves and limb muscles was normal. Deep tendon reflexes were elicitable and she had bilateral flexor plantar. Baseline blood investigations were normal. Neuroimaging of the brain and spinal cord was normal. Needle EMG showed myogenic patterns. Serum CPK levels were normal. We performed an MRI of the paraspinal muscles, which showed atrophy and fibro-fatty infiltration of these muscles. Hence, she was diagnosed to have primary camptocormia and managed conservatively with physiotherapy and rehabilitative exercises. Discussion: A variety of musculo-skeletal, neurological and psychogenic causes leads to camptocormia. It is essential to evaluate the patient for neuro-degenerative disorders like parkinsonism, motor neuron disease, myasthenia and other myopathies. While being commonly encountered in inflammatory myopathies and muscular dystrophies, camptocormia as a presenting complaint of Myotonic dystrophy (MD) is rarely reported. MD may have an adult onset with distal limb and facial weakness. On basis of temporal-masseter atrophy, percussion myotonia and EMG pattern with a positive DMPK mutation, we diagnosed out first patient with MD. The evaluation for secondary causes of camptocormia was normal in our second case. With MRI suggested of chronic atrophy of paraspinal muscles, we diagnosed her as having primary camptocormia. With physiotherapy and gait therapy, she showed progressive improvement in posture and was able to stand and walk few steps with an upright posture. Hence, careful evaluation and management helps in making the curved spine straight.

MDP19/288: Status dystonicus: A movement disorder emergency

Sonali Bhattad

Department of Neurology, GB Pant Institute of Post Graduate Medical Education and Research, New Delhi, India

Introduction: Status dystonicus, also known as dystonic storm or dystonic crisis, is a rare but life-threatening neurological emergency in a movement disorder setting. It usually occurs as a continuum of worsening dystonia seen after weeks or months after a diagnosis of dystonia is established. It is defined as “increasingly frequent and severe episodes of generalised dystonia which necessitate urgent hospitalisation”. We present a case series of four patients with status dystonicus due to different etiologies and their clinical spectrum. Aims: To study the various etiologies of dystonic storm, their clinical profile, management, and outcome of patients. Materials and Methods: In this study we analysed a case series of four patients presenting with status dystonicus who were recruited prospectively through movement disorder clinic. Results: All the four patients were male children with age group ranging from ten to thirteen years. Out of the four children, the etiology in two patients was wilson's disease, the third patient was a diagnosed case of subacute sclerosing panencephalitis (SSPE) and the fourth patient was a case of adrenoleukodystrophy. The factors contributing to status dystonicus in two patients of wilson's disease were chelating agents i-e, zinc and pencillamine respectively. Infection was the contributing factor for dystonic spells in the SSPE and adrenoleukodystrophy patients. The duration of dystonia with superadded dystonic spells was longer (months) in wilson's disease patients and was shorter (days) in the non-wilson's status dystonicus patients. All the four patients had elevated creatine kinase levels. In the first patient of wilson's disease a suspicion of zinc induced dystonic storm was made and zinc therapy was slowly tapered and stopped. In the second patient of wilson's disease a suspicion of penicillamine induced dystonic storm was made and penicillamine therapy was slowly tapered and stopped. Both patients were given injectable BAL (Dimercaprol), and they were free from episodes of dystonic spells, but unfortunately they expired due to hematemesis. In the patient of SSPE and adrenoleukodystrophy, the underlying respiratory tract infections were responsible for the causation of dystonic spells which were managed with appropriate antibiotic therapy leading to marked reduction in the dystonic spells and the patients were discharged satisfactorily and are still under follow-up. Conclusions: Dystonic storm is a life-threatening emergency leading to fatal respiratory and metabolic disturbances. It is often a triggered event; main triggers include medication adjustment and infection and in about one-third of cases no obvious trigger is identified. Status dystonicus is commonly reported to occur in patients of wilson's disease but rarely encountered with SSPE and adrenoleukodystrophy. Status dystonicus patients are vulnerable to secondary complications such as sepsis appropriate septic screen is warranted. The outcome of status dystonicus is variable and unpredictable. Poor prognostic markers are male gender and tonic phenotypes. The evidence-based guidelines for management are lacking. There is a desperate need for formulating the medication selection, drug order, dose, polypharmacy, the role of intravenous sedation and intubation, as well as the timing and patient selection criteria for deep brain stimulation (DBS) and intrathecal baclofen.

MDP20/401: Restless leg syndrome in neuromyelitis optica

Tarun Nagpal

Department of Neurology, Nagpal Neuro and Arthritis Center, Jabalpur, Madhya Pradesh, India

We studied 30 patients of Neuromyelitis optica (serpositive for aquaporin 4) and 30 age matched controls for symptoms of restless leg syndrome. We administered questionnaire for RLS consensus criteria, RLS severity score and Epworth Sleepiness Scale. The frequency of RLS, it's severity and impact on sleep health was found to be significantly higher in NMO patients than age matched controls who suffered from RLS.

MDP21/402: Epilepsia partialis continua in Wilson's disease

Tarun Nagpal

Department of Neurology, Nagpal Neuro and Arthritis Center, Jabalpur, Madhya Pradesh, India

Seizures are uncommon manifestations in Wilson's disease. Generalised tonic clonic seizures have been reported with varying frequency and occasionally focal seizures have also been mentioned in literature. We report a case of epilepsia partialis continua lasting for 2 days in a case of advanced Wilson's disease. To our knowledge, this is the first reported case of refractory prolonged epilepsia partialis continua in Wilson's disease.

MDP22/418: Apraxia of eyelid opening in spinocerebellar ataxia-3

Niraj Kumar, Desai I, Shree R, Radhakrishnan DM

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

Introduction: Machado–Joseph disease (MJD)/spinocerebellar ataxia type 3 (SCA3), the most common SCA worldwide, is an autosomal dominant disorder with mutation in the ATXN3 gene. It has a heterogenous clincal presentation with cerebellar ataxia, pyramidal and extrapyramidal features and peripheral neuropathy being commonly reported. Dystonia has occasionally been described but not apraxia of eyelid opening (AEO) to the best of our knowledge. Case Report: A 23-year-old right-handed male, born of a non-consanguineous parentage, presented with mixed (spastic +ataxic) dysarthria of 11-year duration, progressive gait ataxia of 4-year duration and craniofacial dystonia with AEO of 2-year duration. AEO worsened over subsequent 6 months and significantly affected his gait, which was already compromised by ataxia. His father had gait ataxia and apraxia of eyelid opening. Neurological examination revealed normal cognition, slow saccades with AEO, brisk deep tendon jerks, bilateral ankle clonus and upgoing plantars along with limb and gait ataxia. Routine lab parameters including thyroid profile and serum vitamin B12 levels were normal. MRI brain revealed mild cerebellar atrophy. Test for SCA returned positive for SCA-3. Although patient failed to respond to levodopa trial, his AEO improved on wearing spectacles thereby improving his gait. Conclusion: We report AEO as an additional neurological feature of SCA-3. It improved with wearing spectacles and we deferred using botulinum toxin injection for the same.

MDP23/434: Rare dystonic presentation of glutaric aciduria type I

Manickavasagam Janarthanam, Reddy PKV, Shunmugasundaram K, Ranganathan L

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

Background and Purpose: Glutaricaciduria type I is a recessive disorder that results due to deficiency of mitochondrial matrix enzyme glutaryl-CoA dehydrogenase, leading to accumulation of glutaric acid and 3-hydroxyglutaric acid. There are two clinical presentations, most common is a progressive encephalopathy beginning in the first 3 to 4 months and presents as psychomotor retardation, cerebellar ataxia, extrapyramidal symptoms, macrocephaly and seizures. The other presentation consists of an acute encephalopathic crisis that mimics with hypoxic or ischaemic encephalopathy especially in the neonatal period. Patient usually presents with Seizures, high anion gap metabolic acidosis, hypoketotic hypoglycaemia. The characteristic MRI findings include T2 hyper intensities 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 dystonic and dyskinetic presentation of 10 yr female, presenting with acute onset of upper and lower limb dystonia with perioral dyskinesias of 2 months duration, examination showed dystonia, brisk DTR, no focal deficts, no encephalopathy,MRI showing T2 FLAIR hyperintersities 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 glutaricaciduria type 1.

MDP24/469: Clinical and radiological correlation in progressive supranuclear palsy

Gaganpreet Singh Taneja, Shankar V, Sundar S, Hazeena P, Sugumar T

Department of Neurology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

Objective: To determine the correlation between clinical features and MRI Brain Imaging findings in patients with Progressive Supranuclear Palsy. Background: Progressive supranuclear palsy (PSP) is a neurodegenerative tauopathy, the more characteristic features of which are vertical gaze dysfunction and early postural instability causing frequent falls. In this study, we investigated the clinical and radiological correlation in patients with Progressive Supranuclear Palsy. Methods: We retrospectively reviewed 13 patients, who underwent a 1.5 Tesla MRI Brain, as a part of their diagnostic evaluation for suspected PSP. The subjects were classified as Probable PSP, Possible PSP and Suggestive of PSP, as per the 2017 Movement Disorder Society (MDS-PSP) Criterion. The MR Parkinsonism Index (MRPI) requires measurement of the area of the pons (P) and midbrain (M) and width of the middle cerebellar peduncle (MCP) on sagittal T1-weighted MRI and the width of the superior (SCP) on coronal MRI. The index is calculated from the formula MRPI = (P/M) × (MCP/SCP). A value of >13.58 was considered positive for PSP by neuroimaging criteria. Each patient's clinical diagnosis was compared to his or her MRPI. Results: Five out of Six (83%) Probable PSP patients; and Four out of Five (80%) Possible PSP patients had Positive MRPI; while one out of the two (50%) patients with Suggestive of PSP diagnosis had a Positive MRPI. Conclusions: In our study, the MRPI correlated well with the MDS-PSP Clinical Criterion.

MDP25/495: A rare case of ataxia

Jayachandra Reddy R

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

18 years old male born to consanguineous marriage presented with gait disturbance in the form of ataxia, tremers of hand since childhood and progressive slurring of speech. Weakness of proximal muscles of lowerlimb of 6 months duration. Family history negative. He underwent bilateral cataract surgury at the age of 3 yrs on examination he has not yet attained secondary sexual characters. Weakness of proximal muscles of limb with power 2/5. He has cerebellar dysfunction in the form of limb ataxia, gait ataxia and scanning speech. bilateral plantar flexor response with sluggish deep tendon reflexes on imaging cerebellar atrophy was present. CPK levels elevated suggestive of myopathy and raised follicle-stimulating hormone nerve conduction studies suggestive axonal neuropathy. All these features suggestive of marinesco-sjogren syndrome, an autosomal recessive disorder.

MDP26/500: Patients of unusual secondary causes of hemifacial spasm presenting with multiple episodes of focal neurological deficit

Surendra Kumar, Bhoi K

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

Hemifacial spasm is a rare movement disorder caused by vascular compression over facial nerve. We noted two cases of hemifacial spasm which progressed to have multiple episodes of neurological deficit after which MRI revealed the diagnosis of vertebrobasilar dolicho ectasia.

MDP27/136: Clinico-etiological profile of young patients with dystonia admitted in the neurology ward of a tertiary care centre of eastern India

Das S, Mondal GP, Bhattacharya R, Ghosh KC, Pattem HK

Department of Neurology, Calcutta National Medical College, Kolkata, West Bengal, India

Introduction: Dystonic movements are caused by co-contraction of agonist and antagonist muscles. There have been rare studies on young dystonia patients from India. Aim: To study the demographic, clinical and etiological profile of dystonia in patients younger than 20 years. Methods: 56 patients with dystonia admitted in the Neurology ward between 1st January 2018 and 31st December 2018 were included. Data on the clinic-etiological profile was recorded and statistical analysis was done by using descriptive analysis (frequency distribution). Our diagnostic approach followed the 2 axes as per the 2013 Consensus guideline. The severity of dystonia was quantified using the Hypertonia Assessment Tool (HAT). The severity of posturing and involuntary movements in different body parts because of dystonia and the functional disability caused was assessed using the Barry-Albright Dystonia Scale (BDS). Higher scores signified more severe involvement. Results: Mean (SD) age of onset=10.36 (6.7), duration of symptoms=15.81 (14.14) months and HAT score=4.53 (1.2). Etiologies- Inherited diseases Metabolic diseases- (Wilson's disease-8, Lesch-Nyhan syndrome-2, PANK-1, MLD-1) Heredodegenerative- (Huntington's disease-4, SCA-2, Ataxia Telengiactesia-1) Primary dystonia-1 (DYT1 mutation) Cerebral dysgenesis- (Aicardi syndrome-1, Congenital hydrocephalus-2, polymicrogyria-1) Acquired diseases Post-infectious- (Post-encephalitis-4, TBM-4, Scrub typhus- 2, SSPE-3) Perinatal - (HIE-3, Kernicterus-1, Neonatal sepsis-2) Post-traumatic- 4 Neuroleptic drugs-2 Cervical syringomyelia-2 Demyelinating diseases-4 Cerebral infarct-1 Distribution- Focal-2, segmental-3, multifocal-19, hemidystonia-9, generalized-23. Temporal disease course- Static-29, progressive-26. All the cases had persistent dystonia. The metabolic, and heredodegenerative diseases, primary dystonia, SSPE patients, cervical syringomyelia and 1 patient with neuromyelitis optica had progressive course. Associated movement disorders-Chorea-11, athetosis-14, tremor-12, orofacial dyskinesia-11. Other associated movement disorders were commonest among patients with metabolic followed by heredodegenerative diseases. Other associated features- Developmental delay-10 and Epilepsy-8 (perinatal insults and cerebral dysgenesis), Neuro-regression-5 (metabolic diseases), cognitive deficits-27, cerebellar signs-4 (SCA and ataxia telengiactesia), self mutilation-2 (Lesch-Nyhan syndrome). BDS scores- Regional involvement-Mean (SD) scores- Eye=0.41 (0.78), Mouth=1.6 (1.5), Neck=1.66 (0.65), Trunk=2.23 (0.57), Upper extremity=2.28 (0.91), Lower extremity=2.28 (0.86). Maximum affection of eyes, neck, trunk and lower limbs occurred in Heredodegenerative diseases, upper limbs in metabolic diseases and mouth in drug induced dystonia. Functional involvement-Speech 1.77 (1.13), Handwriting 1.88 (0.96), Feeding 1.9 (1.19), Swallowing 1.17 (0.9), Hygiene 1.67 (0.99), Dressing 1.56 (0.97), Walking 2.38 (1.37). Maximum involvement of handwriting, feeding, swallowing, and hygiene occurred among post-infectious patients, dressing and walking among patients with perinatal insults and speech among drug-induced dystonia patients. Conclusion: Indoor patients with dystonia had the following characteristics. Predominantly due to secondary causes. Predominantly generalized followed by multifocal distribution. Metabolic followed by heredodegenerative diseases had higher incidence of associated movement disorders. Upper and lower extremity dystonia were severest. Impairment of walking, feeding, and handwriting were worst impairments.

AOP1/12: Beta-interferon therapy in relapsing and remitting multiple sclerosis - challenges in an emerging country

Yadav PK, Radhakrishnan S

Department of Neurology, Vivekananda Hospital, Durgapur, West Bengal, India

Multiple sclerosis (MS) is immune-mediated demyelinating disease, with increasing prevalence in India, as seen in recent studies. Beta-Interferon is the widely used treatment option. There is a paucity of studies on beta-interferon in relapsing remitting multiple sclerosis (RRMS) in India. Aims: To study the efficacy, compliance, side effect profiles of patients on beta-interferon, with a diagnosis of RRMS, and also look at other factors, such as, affordability, preference, and availability. Settings and Design: A retrospective-prospective design. Materials and Methods: Twenty patients with a diagnosis of RRMS were included in study. They were followed up regularly while on beta-interferon. All patients underwent magnetic resonance imaging (MRI) of brain, plain and contrast, and expanded disability status scale (EDSS) score was assessed at regular intervals. Results: The mean age was 36.25 ± 12.24 years, with a female:male ratio of 2.3:1. The majority were unemployed, and were dependent on their families for treatment. There was significant delay from diagnosis to start of interferon therapy, mean duration being 29.95 months. Oligoclonal bands were positive in 70%. Seventeen patients were on interferon beta 1a intramuscular and three patients on interferon beta 1a subcutaneous injections. The mean relapse rates per patient before and after starting beta interferon were 1.85 and 0.55 respectively. First dose fever was most common side effect. Headache, myalgia, and psychiatric symptoms were also seen. Periventricular and juxtacortical lesions were most common radiological sites. Conclusions: Beta-interferon is an effective and safe therapy in Indians with RRMS.

AOP2/29: Anti-NMDAR encephalitis with prominent orolingual dyskinesia and its association with cystadenoma of ovary – A very rare presentation

Pandey S, Borah NC

Department of Neurology, GNRC Hospitals, Dispur, Assam, India

Autoimmune encephalitis is relatively a new disease entity. Anti NMDAR encephalitis is most common and well studied autoimmune encephalitis. First case report of anti-NMDAR encephalitis was published in 2007 by Dr. Joseph from university of pennysylvania. It was reported in a young female patient and was associated with ovarian teratoma. We report a case of Anti-NMDAR encephalitis presenting with prominent orolingual dyskinesia and its association with large ovarian cystadenoma. A young female patient presented to us with sub-acute onset of behavioural changes and prominent orolingual dyskinesia. It was also associated with significant autonomic instability. On the basis of clinical symptoms and the age group of patient, she was suspected to have autoimmune encephalitis. Anti-nmda receptor antibody testing was done in csf and it came out to be positive. The screening of associated tumour showed a large cystic ovarian tumour. She was given complete course of IVIG (2GM/KG) as well as removal of tumour. Histopathology of the lesion concluded it to be a cystadenoma of ovary. The patient is now on monthly IVIG (0.4GM/KG) therapy and is doing well. This case is unique as this patient with anti-NMDAR encephalitis had prominent orolingual dyskinesia as initial presentation and it was associated with cysadenoma of ovary. The association of cysadenoma of ovary with anti-NMDAR encephalitis is very rare association. Most common tumour associated with anti-NMDAR encephalitis is teratoma of ovary. There are no reports till now showing association of anti-NMDAR encephalitis with cystadenoma of ovary.

AOP3/33: Expanding etiologic spectrum of painful ophthalmoplegias

Tambe S, Kumar PS, Archana NB

Department of Neurology, Bangalore Medical College and Research Institute, Bengaluru, Karnataka, India

Introduction: Painful ophthalmoplegia has diverse causes. Detailed clinical assessment and comprehensive evaluation is necessary for diagnosis. Tolosa Hunt syndrome is inflammatory process of unknown etiology, however its diagnosis requires exclusion of other etiology. With more understanding of Neuroimmunology and entities like IgG4 Related Disorders etiologic spectrum has expanded. Etiology is important as it is main determinant of treatment. Objectives: To study clinical, biochemical and radiological profile of painful ophthalmoplegias and to evaluate various etiologies of painful ophthalmoplegias. Methods: A tertiary neurology centre based prospective study. We enrolled consecutive patients presenting with painful opththalmoplegias between December 2017-May 2019. After detailed clinical assessment, all patients were subjected to radiological, haematological, biochemical investigations. Additional investigations like IgG4, nasal Scraping for fungal evaluation was done as needed. The clinical and investigational data was collected and analysed. Results: We identified 42 patients. 20 (48%) were female and 22 (52%) were male. Various etiologies included 2 (4.7%) Traumatic, 8 (19%) infectious, 17 (40%) Inflammatory, 2 (4.7%) Vascular, 4 (9%) Neoplastic, 1 (2.3%) TB/Metastasis, 1 (2.3%) Recurrent Opthalmoplegic Cranial Neuropathy, 1 (2.3%) Sphenoid Mucocele and 6 (14%) Diabetes. Of The Inflammatory Causes 3 patients had orbital Pseudotumor, 3 had Possible IgG4 Related Disorder, 2 patients had Pachymeningitis and 9 had Idiopathic cause. 6 (14.2%) patients had Recurrent Painful Ophthalmoplegias. Conclusion: Meticulous clinical evaluation and targeted investigations help in identifying etiology of Painful Opthalmoplegias and avoiding waste basket diagnosis as tolosa hunt Syndrome. Accurate diagnosis has therapeutic and prognostic implications.

AOP4/54: A rare case of anti-dopamine D-2 receptor antibody encephalitis

Farooq O, Farooq S, Basoo J, Rasheed S

Department of Neurology, Government Medical College, Srinagar, Jammu and Kashmir, India

Antibody-mediated encephalitides constitute a group of inflammatory brain diseases that are characterized by prominent neuropsychiatric symptoms and are associated with antibodies against neuronal cell-surface proteins, ion channels, or receptors. Common clinical features include a change in behavior, psychosis, seizures, memory and cognitive deficits, abnormal movements, dysautonomia, and a decreased level of consciousness. The estimated annual incidence of all types of encephalitis is approximately 5 to 8 cases per 100,000 persons, and in 40 to 50% of the cases, the cause cannot be established. A prospective, multicenter, population-based study suggests that autoimmune disorders are the third most common cause of encephalitis, after infections, usually viral, and acute disseminated encephalomyelitis. The frequency of the most common form of autoimmune encephalitis, the type with antibodies against the N-methyl-d-aspartate receptor (NMDAR), surpasses the frequency of any individual viral cause of encephalitis in young persons, and in one retrospective study, anti-NMDAR encephalitis accounted for 1% of all admissions of young adults to an intensive care unit. We present a rare Case of Anti Dopamine D-2 receptor antibody encephalitis. A 43 year old female with underlying hypertension presented with sub acute onset dysarthria, dysphagia and drooling of saliva of 5 days duration without any history of fever, rash, polyarthralgia, loss of consciousness, seizure, diplopia, facial weakness, numbness, limb weakness, bladder/bowel symptoms with examination revealing her to be conscious, comprehending verbal commands but little agitated, producing incomprehensible sounds with bilateral 9th and 10th cranial nerve palsy, generalized rigidity all over, brisk reflexes all over with upgoing plantars and rest of the examination was normal. Investigations revealing normal CBC, KFT, LFT, Lipid Profile, routine urine examination, chest x-ray, ECG. Widal,  Brucella More Details, vasculitic profile, wilson's profile, TFT, Anti TPO. CPK, lactate, iron profile, iPTH, tumour markers, triple serology was normal. USG abdomen/ pelvis/ breasts, USG Doppler neck, CECT chest abdomen pelvis, CT Head, MRA MRV Brain were normal. MRI brain was showing bilateral basal ganglia (caudate and lentiform nuclei) hyperintense signal changes without restricted diffusion/abnormal susceptibility. CSF showing neutrophilic pleocytosis, normal sugar, protein, ADA, Gram Staining, AFB Staining, fungal staining and CBNAAT negative, CSF HSV PCR negative. Paraneoplastic profile, autoimmune profile of CSF was negative except anti D2 receptor antibody. Patient was managed with ryles tube feeding, antiparkinsonian drugs in view of parkinsonian features and on the line of autoimmune encephalitis, she was given 5 doses of IV methyl prednisolone 1g/day with no improvement. Patient was then given IVIG 400mg/kg/day for 5 days and there was dramatic improvement in her symptoms and signs and within 10 days patient was able to take orally. In humans there are five subtypes of dopamine receptors which are rhodopsin like seven transmembrane G protein coupled receptors, and are divided into 2 groups D1 like group (D1R, D5R) and D2 like group (D2R, D3R, D4R) on the basis of their properties. Dopamine receptor expression, innervation in humans are prominent in the brain, important in regulation of psychological and neuromuscular functioning.

AOP5/66: Monitering of immunomodulatory therapy with Rituximab in CNS autoimmune disorders

Roy AK, Sadekar S, Srinivasan M

Department of Neurology, Manipal Hospital, Bengaluru, Karnataka, India

Objectives: To study the therapeutic efficacy and monitoring the levels of immunomodulatory drug Rituximab when used as a primary and rescue therapy in patients with CNS autoimmune disorders. Methods: Twelve patients, ocular myasthenia (2) and generalized myasthenia (post thymectomy) (3), long segmental myelitis (2) and multiple sclerosis (5) were treated with Rituximab infusions once a week for four weeks and levels of CD 19/CD 20 were monitored in these patients. Results: Rituximab is a humanized monoclonal antibody which acts on B cells bearing the CD 20 molecule which play a role in pathogenesis of autoimmune neurological disorders. All twelve patients improved after their first dose of Rituximab. The levels of CD 19/CD 20 were checked. Absolute count for CD 19 is 56- 414, for CD 20 is 74-441. % of CD 19 is 4-22 and for CD 20 is 5-22. Initially the levels were near the lower normal range. When the levels were checked after the second schedule, seven patients had value of 0 whereas others had values ranging between 0 and 5. 5. Nine patients had values of 0%, others had between 0% - 1%. These patients are in remission now for past two years. Conclusions: CD 19/CD 20 levels help in deciding a therapeutic endpoint and also show therapeutic efficacy of immunomodulatory therapy. Monitoring these levels will help the clinician to plan duration of therapy and also is economically beneficial to patients.

AOP6/87: A 10 years retrospective analysis of clinico-radiological features and treatment outcome among patients with longitudinally extensive transverse myelitis

Kannan KT, Ramasamy B, Perumal S, Madhavi K, Vallipalam JJ, Soumya GK

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

Introduction: Longitudinally extensive transverse myelitis (LETM) is an immune mediated neurological disorder affecting spinal cord. Sometimes it also involves brain and optic nerves. Aetiology includes Neuromyelitis optica spectrum of disorder (NMOSD), Connective tissue diseases, infective, Malignancy, Multiple sclerosis, ADEM and idiopathic. Clinical features, radiological picture and treatment outcome varies depending on the aetiology. LETM is a challenging disorder in this resource crunch country. Though similar studies have been done in western world, this disease was not assessed in this geographical distribution. Aim(s): To assess the differences in clinic-radiological features and treatment outcome among various aetiologies in patients presenting with longitudinally extensive transverse myelitis to a tertiary care centre in southern India. Materials and Methods: It is a retrospective cross-sectional observational study. A total number of 40 patients between the age of 8-80 years presented with LETM visited this medical centre from 2010-2019, were included in this study. Results: In this study of 40 patients, 70% are females and 30% are males. Among age groups, 14 patients are more than 50 years of age, 11 patients are less than 30 years of age. Among various aetiology, 16 patients has NMOSD, 7 were idiopathic, 5 had connective tissue disease and 4 were due to infective aetiology. Their clinical, radiological, CSF findings, relapses, treatment and disability at onset, 3 months and 6 months (based on EDMUS scoring system) were compared and presented in this study.

AOP7/89: Study on the outcome of rituximab therapy in refractory neuromyelitis optica

Kurian S, Marimuthu J, Ranganathan LN

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

Background: Neuromyelitis optica (NMO) is a severe autoimmune disease targeting optic nerves and spinal. The monoclonal anti-CD20 B cell antibody Rituximab is a treatment option in refractory NMO. The prevalence of NMO in South India is around 2.6/lakh population. This study was conducted to evaluate the outcome of Rituximab over 12 months in refractory NMO. Materials and Methods: 23 patients of refractory NMO treated with Rituximab who attended the demyelination clinic from January 2017 were studied. Refractory NMO were cases of NMO with at least one relapse on immunosuppressive therapy. A fixed treatment scheme of total four doses of weekly Rituximab at 375mg/m2 and the same repeated every six months, or earlier based on CD19 levels, was given for refractory NMO. The mean annualized relapse rate (ARR) and the median Expanded disability status scale (EDSS) were analysed before treatment and after one year on Rituximab. Results: After a mean follow up of 13 months, we observed a significant reduction of median Annualized relapse rate from 2.9 to 0.14 and of the median EDSS score from 5.2 to 3.3.Two patients had relapse while on Rituximab, which was due to inadequate follow up doses of Rituximab. Conclusion: The therapy with Rituximab, with proper follow-up, was found to significantly reduce the frequency of relapses, with subsequent stabilization or improvement in disability.

AOP8/107: A case series of primary angiitis of central nervous system

Bhavana I, Radhakrishna H, Padhy BP

Department of Neurology, CARE Hospital, Hyderabad, Telangana, India

Introduction: PACNS is a rare disorder causing inflammation and destruction of CNS without evidence of inflammation in tissues outside CNS. The disease is poorly understood and is difficult to diagnose. RCVS, posterior reversible encephalopathy syndrome and brain neoplasms are the common mimics. Materials and Methods: We are presenting a case series of five cases of CNS vasculitis presented to our hospital over one and half year. Adult patients of age >12 years and both genders are included who presented with varied clinical features and complications of CNS vasculitis are included in the study. The course of the illness and response to therapy has been studied. Discussion: PACNS affects patients of all ages but peaks at around 50 years of age and is most common in males. The clinical signs and symptoms are non-specific. The course of the illness is also variable with presentations ranging from hyperacute to chronic and insidious. Headache, cognitive impairment, stroke and TIAs are common presentations. Various subtypes are GACNS (granulomatous angitis), benign angiopathy of the CNS (BACNS), and atypical PACNS (Mass-lesion presentation, Amyloid-β-related cerebral angiitis). MRI is the main modality for the workup of patients. Sensitivity is 90–100%. Multiple cortical and subcortical infarcts are the most common lesions. Cerebral vasular imaging by angiography is used. Brain biopsy remains the gold standard. Histologically, the inflammation affects the medium & small arteries & arterioles of the meninges and cortex & rarely the veins and venules. Most studies suggest good outcome when treated with glucocorticoids +/- cyclophosphamide.

AOP9/134: Sjogren's syndrome: A rare cause of isolated vagus nerve palsy

Bhattacharjee A, Guha G, Agasti N, Repaka N, Ghosh S, Roy M, Nanda S

Department of Neurology, Nil Ratan Sircar Medical College, Kolkata, West Bengal, India

Primary Sjogren's Syndrome is an autoimmune connective tissue disorder which characteristically affects the exocrine glands besides having other systemic features. Neurological manifestations commonly include peripheral neuropathy, myelopathy, focal or diffuse brain lesions or multiple cranial neuropathy. Cranial nerve affection is less common as far as nervous system affection of this syndrome is concerned; Isolated Vagus Nerve Palsy is perhaps the rarest reported in literature with only one case reported thus far. Here we describe the case of a middle-aged lady who presented with isolated vagus nerve involvement as the sole neurological manifestation of Sjogren's syndrome with very subtle glandular involvement, without any other neuological manifestations commonly associated with Sjogren's Syndrome.

AOP10/146: Disseminated tuberculosis with longitudinally extensive transverse myelitis: Interesting case series of a rare presentation

Rini George, Shanmugasundaram N, Balasubramanian S, Narasimhan RL

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

Longitudinally extensive transverse myelitis is defined as inflammatory lesion extending to three or more segments of spinalcord. It is a rare presentation of Mycobacterium tubercular infection. Case vignette 1: A 45 year female with history of fever, back pain, difficulty in walking and contact with PTB with subsequent altered sensorium. On admission she had features of subacute meningitis and MRI Brain showed features of early hydrocephalus and meningeal enhancement. MRI spine showed a linear central hyperintensity from C3 to D12 spinal segment with T1 hypointensity and contrast enhancement. CSF analysis showed elevated protein, lymphocytic pleocytosis and positive for CSF AFB. She failed to improve with steroids and ATT. She improved after 5 cycles of therapeutic plasma exchange. Case vignette 2: A 28 year old male with history of fever, chronic cough, back pain, weakness of both lowerlimb. He was evaluated for flaccid paraparesis. Chest xray showed encysted pleural effusion which was exudative and positive for MDR TB. MRI spine showed T2 hyperintensity from C5 to C7 and D8 to D11. CSF analysis showed elevated protein with normal glucose and raised LDH and positive gene xpert. Patient failed to improve with ATT and steroids. He was given inj. IVIG and showed signs of improvement. Conclusion: Both these cases of tubercular LETM failed to improve with ATT and steroids but instead responded to immunomodulators suggesting an immune mediated mechanism rather than direct tubercular infection.

AOP11/151: Neuromyelitis optica spectrum disease with positive autoimmune indices: A case report

Kumar RK, Nityhanandam A, Sarala G, Narasimhan RL

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

Introduction: Neuromyelitis optica is a serious, idiopathic demyelinating disease of CNS selectively attacking the spinal cord or optic nerves. Case Report: Mrs. A, 31 year old female presented with flaccid quadriparesis, sensory level, bowel & bladder involvement. She had a past history of lung disease treated with steroids. MRI spine showed Long segment T2 hyperintesity of spinal cord from C5 to D11. Other investigations showed elevated counts, ESR elevated, normal electrolytes, CSF analysis was normal. Vasculitic profile showed- positive IgM Anticardiolipin antibody, positive P-ANCA and Anti-ds DNA – positive. Serum Aquaporin 4 antibody was positive, VEP – prolonged suggestive of Bilateral optic neuritis. CT Chest showed bilateral pleural effusion. Patient recieved methyl prednisolone followed by IVIG for 5 days and Physiotherapy. As there was no much improvement even after 2 weeks patient was subjected to plasmapheresis following which weakness improved minimally. Subsequently patient was treated with Rituximab and then weakness improved. Discussion: Longitudinal extensive transverse myelitis (LETM) may occur as an uncommon manifestation of SLE in only 2% of patients several years before the development of SLE. NMO and NMO-SD have rarely been reported as a first manifestation of SLE. Patients with NMO often have positive antibodies for lupus. If they have clinical symptoms suggestive of rheumatological conditions it is likely that there are two coexisting autoimmune syndromes. Conclusion: Patients with LETM should always be tested for NMO IgG even if clinical and paraclinical autoimmune indices are present since patients with NMO-SD may present features of coexisting systemic autoimmunity.

AOP13/191: The clinical, radiological and immunological profile of neuromyelitis optica spectrum disorders in a tertiary care centre in Kerala

Manju Surendran, Shaji CV, Kabeer KA, Prasanth SR

Department of Neurology, Government TD Medical College, Alappuzha, Kerala, India

Background: Neuromyelitis optica is a severe demyelinating disease that preferentially affects optic nerve and spinal cord. Disability following these attacks was often severe. Diagnosis of NMOSD is currently based on clinical, neuroimaging and laboratory features. Standard treatment is based on the use of steroids and immunosuppressive drugs and aims to control the severity of acute attacks and to prevent relapses of the disease. Objective: The objective was to study the clinical, radiological and immunological profile in patients with Neuromyelitis optica spectrum disorder (NMOSD) at a tertiary care center in kerala. Methods: This is a descriptive study including patients who presented to the Department of Neurology, Government T D Medical College Alappuzha between June 2018 to May 2019 who satisfied the Diagnostic Criteria for NMOSD and MOG encephalomyelitis. Results: There were 14 patients: 71.4 % females and 28.6 % males. The most common age group of symptom onset was 21-30 yrs. The most common clinical presentation was paraparesis (4) and defective vision (4). Most common radiological finding was longitudinally extensive transverse myelitis (6). Anti aquaporin antibody was positive in 28.57% (4) patients and MOG antibody was positive in 50 % (7) patients. Steroid response was seen in 68.28% (9) patients. 1 patient required plasma exchange and 5 required second line immunomodulatory agents. Conclusion: NMOSD is a demyelinating disorder with female predominance. Longitudinally Extensive Transverse Myelitis was the most common MRI finding with Anti aquaporin antibody positivity in 28.57% of patients and MOG antibody positivity in 50% of the patients in this study.

AOP14/198: A clinical profile of NMO and NMOSD in a tertiary care hospital in south Tamil Nadu

Karunakaran V, Saravanan S

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

To Assess the clinical profile, Seropostivity and outcome of NMO and it's spectrum disorders. We did Retrospective study in tirunelveli medical college from 2016 January to 2019 May. Patients satisfying the revised Wingerchuk diagnostic criteria for NMO (seropositive NMO and seronegative NMO) were included in this study. Among the 25 patients we studied, 28% were seropositive, 72% were seronegative. The female and male ratio was 2.57:1 (n=25). Median age of onset was 35 (24-45). The initial presentation was combined myelitis and optic neuritis (28%) was more common among seronegative patients when compared to seropositive patients (p=0.047). In laboratory tests CSF analysis median cell count was 54 (0-840) and median CSF protein was 58 (4-112). In patients with clinical evidence of myelitis 68% having cervico dorsal spinal cord involvement. All 25 patients treated with methylprednisolone initially among which 12 improved, 10 needed second dose of steroids, two of them improved with plamaphersis and one patient improved with rituximab. EDSS at the last follow up was lower among seronegative patients (median=3.5) on comparing to seropositive patients (median=5) But there is no statistically significant difference (p=1.4). This study revealed that combined myelitis and optic neuritis is more common among seronegative patients compared to seropositive patients. Patients with seropositive NMO had similar outcome when compared to seronegative patients.

AOP15/253: Morvan syndrome: Case series and review of the literature

Abhay Bagul, Jagiasi K, Ojha P, Soni G, Singh R, Kadam N, Barwalia P

Department of Neurology, Grant Medical College and Sir JJ Group of Hospitals, Mumbai, Maharashtra, India

The first description of Morvan syndrome was in 1890 by Augustin-Marie Morvan. Morvan syndrome is a rare autoimmune disorder characterised by peripheral nerve hyperexcitability, autonomic dysfunction and central nervous system (CNS) symptoms with strong association with autoantibodies to voltage gated potassium channel complexes. Very few case reports are available from India till now. Being a treatable disease, awareness and early diagnosis is important. We report 6 cases of Morvan syndrome admitted in tertiary care hospital from 2017 to 2019. All enrolled subjects were male. Features of CNS involvement as insomnia and peripheral nervous system hyperexcitability as myokymia were seen in all subjects, and autonomic dysfunction as orthostatic hypotension and hyperhydrosis was seen in 66% subjects. Seizures were absent in all subjects. Voltage gated potassium channel antibody was positive in 66% (n=4) of subjects, all four were positive for CASPER2 and 2 were also weakly positive for LGI1. Neuromyotonia was observed in all patients and confirmed with electromyography. MRI Brain, EEG and cerebrospinal fluid examination were normal in all subjects. Whole body PET CT scan for primary tumour was done in 4 subjects and was normal. Four subjects were treated successfully with steroids. However, 2 subjects required additional treatment with intravenous immunoglobulin. Pain was treated with phenytoin and carbamazepine. On follow up, all patients are well and no relapse was observed.

AOP17/269: Chronic hepatitis B related CNS demyelination with cerebellar ataxia responsive to steroids – A case report

Swapan Gupta, Shukla T, Khwaja GA

Department of Neurology, GB Pant Institute of Post Graduate Medical Education and Research, New Delhi, India

Introduction: Hepatitis B virus infection is known to cause demyelination of both central nervous system and peripheral nervous system. Cases of demyelinating polyneuropathies like AIDP and optic neuritis have been reported in association with hepatitis B virus infection. However, there is no case report of Hepatitis B virus related CNS demyelination with steroid responsive cerebellar ataxia to the best of our knowledge. Case: A 60 years old male presented with insidious onset gradually progressive imbalance while walking, slurring of speech and tremulousness in both upper limbs for last 7 months. Examination revealed features suggestive of severe cerebellar dysfunction. MRI brain revealed demyelinating lesions in both cerebellar hemispheres and bilateral middle cerebellar peduncles with no contrast enhancement or diffusion restriction. He was found to be HBsAg positive with a high HBV viral load. HBeAg was negative but IgG anti HBc antibody was positive suggestive of chronic active Hepatitis B. USG whole abdomen and fibroscan revealed coarse echotexture of liver with prominent spleeno-portal axis and splenomegaly. CECT chest and abdomen also revealed active tubercular infection however CSF analysis didn't show any evidence of CNS tuberculosis. Patient was initially started on Entacavir followed by ATT. Subsequently after initial stabilization, he was given pulse steroids followed by oral prednisolone. He showed dramatic response with improvement in cerebellar signs following steroid therapy. Conclusion: This case is a unique presentation of Hepatitis B presenting with demyelinating lesions in cerebellum leading to cerebellar dysfunction. Favorable response to steroids points towards an auto-immune mechanism.

AOP18/282: A study of clinico-radiological profile with special emphasis on cognitive changes in patients of multiple sclerosis

Swati Kumar, Ganguly G, Biswas A

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

Introduction: Multiple sclerosis (MS) is a demyelinating disorder of the central nervous system (CNS), characterized by inflammatory demyelination and axonal loss. Although cognitive impairment is a well-recognized disabling symptom of MS, occurring in up to 65% of patients, cognitive function is not routinely assessed neither in clinical practice nor in clinical trials. Aims: To study the clinical profile with special emphasis on cognitive changes in patients of Multiple sclerosis. Materials and Methods: This was a descriptive cross-sectional study involving MS patients, diagnosed as per 2010 MacDonald's criteria, admitted in Department of Neurology, BIN. Detailed clinical history was taken, clinical examination was done. Detailed cognitive testing was done using Mini-mental score examination (MMSE), Frontal assessment battery (FAB) and Kolkata cognitive battery. Relevant investigations were done, including MRI, VEP and CSF study. Data was analysed using appropriate statistical tools. Results: Of 28 patients, 18 (64%) were females. Mean age of patients were 34.7 (26-51). Median disease duration was 7.5 years (1.5-15). Most common clinical feature was optic neuritis (64%) followed by myelitis (50%). Median EDSS score was 3.5 (1-6.5). Most common cognitive domains affected were executive dysfunction (100%), attention (71%), visuo-constructional (57%), language (50%), memory (28%). Median MMSE was 29 (24-30), median FAB score was 15 (10-18). 78% patients had high lesion load on MRI with 35% Gadolinium enhancing lesions. Conclusion: Cognitive impairment though subtle was seen in a significant proportion of patients in our study as picked up by detailed cognitive assessment.

AOP19/328: Atypical multiple sclerosis with antibody to MOG

Dhaval Dave, Khan F

Department of Neurology, Dr. D. Y. Patil Medical College, Hospital and Research Center, Pune, Maharashtra, India

Introduction: We report a case of young female, who had MRI Brain lesions typical of Multiple Sclerosis with CSF Oligoclonal bands and had Definite Multiple Sclerosis based on revised McDonald criteria, but she also had atypical features of mild pleocytosis, brainstem and cerebellar peduncle involvement apart from optico-spinal involvement. She turned out to be positive for Anti-MOG antibody as well, hence diagnosed to have Atypical Multiple Sclerosis. This case highlights when to suspect Atypical Multiple Sclerosis and their management approach. Discussion: Typical MS cases are largely Anti MOG? negative. In a study of 50 Japanese cases with AQP4? IgG? negative opticospinal MS, just two were MOG? IgG? positive, but they had some features atypical for MS, such as bilateral optic neuritis, longitudinally extensive transverse myelitis or moderate pleocytosis. In another study Antibodies to MOG were found in about 5% (5/104) of preselected adult patients with MS. Patients with MS with antibodies to MOG showed typical MS lesions on brain MRI with concomitant severe brainstem and spinal cord involvement and had a severe disease course with high relapse rates. Conclusion: Any patient showing typical MS leisons on brain MRI with OCB present in CSF but has atypical features like mild pleocytosis with brainstem, cerebellar or optico-spinal involvement should also be tested for autoantibodies to MOG and if positive then requires aggressive treatment approach in the form of Plasma Exchange, if resistant to pulse steroid therapy followed by either Rituximab or Natalizumab rather than trying other DMT's.

AOP20/345: Iron deposition in deep subcortical nuclei in multiple sclerosis patients: Cause or effect?

Sheikh Hilal Ahmad, Agrawal CS, Sud S, Mahajan M

Department of Neurology, Sir Ganga Ram Hospital, Delhi, India

Background: Multiple Sclerosis (MS) is considered to be autoimmune inflammatory, demyelinating disease of the central nervous system (CNS). Iron deposition is known to occur in CNS of MS patients. This MRI based study was done to see iron deposition in deep subcortical nuclei and look for any correlation with anatomy of extracranial venous drainage system. Materials and Methods: This case control study had two groups of 25 cases each. One group being of consecutive MS patients and another of other neurological disease (OND). The two groups were age and sex matched. Iron stores in deep grey matter nuclei were quantified with SWI studies on MRI. Both the groups underwent MRV of neck vessels and azygous system and cross sectional areas of internal jugular veins, azygous veins were measured. Results: We found more iron in all the nuclei in MS group compared to OND group. But statistically significant difference between the two groups was seen in bilateral pulvinar thalami and red nuclei, right putamen, right caudate nucleus and left substantia nigra. The various predefined cross sectional areas of internal jugular veins (IJV) and azygous veins were comparable in the two groups. The number of subjects having “stenosis” in IJV (as per predefined cutoff) was more in OND group than MS group. Thus the absence of any difference in anatomical parameters in two groups goes against any vascular hypothesis of iron deposition in deep subcortical nuclei. Conclusion: The iron deposition may be an epiphenomenon of underlying disease rather than having to do something with etiopathogenesis.

AOP21/346: Stiff-person syndrome with anti GAD65 antibodies- Rare association with primary adrenal insufficiency

Srikanth Koguru, Vengamma B, Prasad SVN, Rao AA

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

Introduction: Stiff person syndrome (SPS) is a rare, insidiously progressive disease of the central nervous system. Patients present with appendicular and axial rigidity caused by continuous involuntary motor unit activity and superimposed stimulus-sensitive spasms. There is good evidence for a primary auto-immune aetiology. Anti-glutamic acid decarboxylase (anti-GAD) antibodies, specifically to the GAD65 isoform, are present in serum or cerebrospinal fluid of 60-80% of patients with SPS and its variants. Case: A 16 year old boy who is a known case of primary adrenal insufficiency since 6 years, on regular follow up in endocrinology department, came with stiffness in all the limbs from 10 days, double vision from 7 days, drowsiness from 1 day. On examination he was drowsy but able to comprehend and was cooperative, pursuits and saccades were impaired, with restriction of bilateral lateral gaze, severe spasticity at shoulders, elbows, hips, knees, with brisk deep tendon reflexes, bilateral plantar extensors. MRI brain was normal. EEG showed background slowing. EMG showed continuous motor unit potentials which decreased on administration of diazepam. CSF analysis was normal. Autoimmune panel was strongly positive for anti GAD65 antibody. He was treated with diazepam, methylprednisolone and Intravenous immunoglobulin. On follow up after 1 month and 3 months, patient showed improvement; there was no stiffness and he was able to walk independently.

AOP22/361: Interesting patients with anti MOG encephalomyelitis

Mahesh Narayanan

Department of Neurology, Apollo Children's Hospitals, Chennai, Tamil Nadu, India

13 year old female child presented with H /o weakness of both lower limbs and upper limbs with urinary incontinence; she was admitted in a private hospital at their native place MRI cervical spine with spine survey showed long segment demyelination with cord edema The child was treated with IVMP for 5 days followed by oral steroids. Oral steroids were tapered off by 6 weeks, had weakness of both Lower limbs once again along with weakness of upper limbs after a week of tapering of steroids. The child was admitted and repeat neuroimaging was done. MRI brain with spinal cord showed demyelination involving the cervical cord and demyelination over cerebellum and thalamus. CSF analysis showed lymphocytic pleocytosis. Serology showed positive anti MOG antibodies. Any child with recurrence of demyelination following steroid withdrawal, think of anti MOG antibody encephalomyelitis. Similarly any child with illdefined fluffy demyelination over brainstem and cerebellum, think of anti MOG antibody encephalomyelitis.

AOP23/362: An interesting case of magnetic resonance imaging mimics of multiple sclerosis

Balaji BS, Sarala G, Nityanandam A, Lakshminarasimhan R

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

Background: Diagnosing Multiple Sclerosis (MS) can be very challenging owing to its variable clinical features & lack of a definitive test & presence of numerous clinical mimics of MS. MRI is a core diagnostic tool in detection of MS lesions & demonstration of spatial and temporal distribution of disease. We hereby present a case of monocular visual loss whose MRI brain demonstrated disseminated in space (DIS) criteria of MS. Case Description: A 39 yr old man, non-smoker nondiabetic presented with acute painless diminution of vision in left eye which progressed to complete loss of vision over 8 hours and remained static for next three weeks. On examination, visual acuity was PL negative in left eye, normal in other; fundus revealed mild disc edema & pallor, peripapillary haemorrhage, arteriolar narrowing. Other Cranial nerve examination were normal except for left RAPD. These findings were s/o Left AION. Blood chemistry showed leucocytosis, absolute eosinophilia, elevated total cholesterol & triglycerides. CSF evaluation showed protein elevation, acelluar and normal sugar. Serum AQ-4 antibody and vasculitic profile was negative. Brain MRI revealed multiple T2/Flair white mattern hyperintensities (WMH) at callosal septum, left centrum semiovale, left parietal & frontal subcortical area with diffusion restriction in left optic nerve sheath complex. CT angio showed severe proximal and cavernous & supraclinoid ICA occlusion. VEP was not recordable from left optic nerve, normal on Right. Patient was treated with IVsteroids with no improvement. Patient lost on follow up. Conclusion: We present a case of AION with MR imaging satisfying DIS criteria for MS. Careful observation of distribution, location, morphology, size, enhancement pattern and vascular/perivascular pattern of WMH help in distinguishing MS from MS mimics.

AOP24/363: Preventing relapse of MS. In search of a low cost alternative

Mohammed Shujauzzaman Bilal, Manorenj S, Jawalkar S

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

A total of 16 patients from Dept. Of Neurology, Princess Esra Hospital, Hyderabad were recruited in the study over a duration of 15 months starting December 2017. 94 % of MS cases were in the 20-40 yrs age group. Male to female ratio was 2:1. Majority of the cases were relapsing remitting MS. Motor symptoms predominated among presenting features. Periventricular demyelinating lesions were present in about 84% of the cases followed by soinal cord lesions in frequency MRI lesions. Eight patients of MS were started on Azathioprine 2mg per kg body weight after an overlap of steroids for a duration of 8 weeks. None of the patients had a relapse over a follow up period of upto 15 months. No major adverse effects of azathioprine were obeserved. Conclusion: Azathioprine was found to be an effective and economical optionin our experience for DMT treatment of MS.

AOP25/364: An unusual case of rhombencephalitis

Sumi M Pillai, Velayutham S, Sowmini PR, Jeyaraj M, Krishnan M, Arunan S

Department of Neurology, Stanley Medical College, Chennai, Tamil Nadu, India

Introduction: Neuromelioidosis accounts for approximately 4% of all melioidosis cases. It is associated with a significant debilitating morbidity and mortality, reaching as high as 25%. It is caused by the gram-negative bacterium, Burkholderia pseudomallei. Case Report: We report the case of a 25 year old male patient, who had been working in Singapore. He presented with history of fever, myalgia, headache and vomiting for 3 days duration. He had doublevision with inability to close right eye. On examination patient was lethargic with bilateral abducent nerve palsies, right LMN facial palsy and right sided hemiparesis. During hospitalisation he had one episode of seizure followed by worsening of sensorium and respiratory distress for which he needed ventilator support. Patient was initially treated with ceftriaxone, vancomycin and acyclovir for broad spectrum coverage of a suspected CNS infection. Patient's MRI brain plain showed symmetrical T2/FLAIR hyperintensities in pons, cerebellar peduncles and medulla. It also showed features of frontoparietal cerebritis. MRI brain with contrast showed multiple ring enhancing lesions in parietal region. CSF analysis revealed lymphocytic pleocytosis, mild protein elevation and normal glucose levels. CSF was negative for bacteria/fungi/TB. Blood culture came positive for  Burkholderia pseudomallei Scientific Name Search n 3rd week of illness. Patient was treated wih prolonged IV and oral antibiotics. His general condition and sensorium improved but with significant morbidity. Discussion: This case is mainly reported in view of it's rarity. Eventhough it is rare in our part of the world, it should be considered as a possibility in patients with rhombencephalitis and needs prolonged antibiotic treatment to prevent relapses.

AOP26/405: Case series of 8 cases of classical stiff person syndrome

Chakradhar Reddy, Chaudhuri JR, Alluri S, Mohammed G

Department of Neurology, Yashoda Hospitals, Hyderabad, Telangana, India

Stiff person syndrome (SPS) is a rare autoimmune disorder. Most commonly associated with anti GAD (Glutamic Acid Decorboxylase) antibody in idiopathic/classic SPS. Rarely it can be paraneoplastic manifestation with anti amphiphysin/anti gephyrin antibodies in breast cancer, small cell lung cancer. Clinically characterized by rigidity and intermittent painful spasms of truncal and limb muscles described as tightness in lower back and thighs with difficulty in walking. Other autoimmune disorders diabetes, hypothyroidism/graves disease, pernicious anemia are associated with SPS. Here we describe Eight cases of SPS, all were females with mean age of 53.6yrs (range 31-74yrs) and had classical clinical presentation of tightness with intermittent spasm in low back, difficulty in walking fast. In addition 2 patients had spontaneous and stimulus sensitive spasms involving lower limbs. Mean duration of symptoms was 17 months (range6-48 months). Diabetes was associated in 2 patients and one had hypothyroidism at presentation. MRI LS spine was performed in 5 patients, none had significant spondylosis. Para spinal and limb muscle EMG performed in 5 patients showed spontaneous MUP activity. All the 8patients had high (3+) serum anti GAD antibody titers in Paraneoplastic panel testing. In addition Anti ZIC4, Anti Titin were weekly positive in one patient each. None of them had malignancy. All the patients were symptomatically better with immuno modulation (pulse methylprednisolone, Azathioprin), benzodiazepines and antispastic drugs. Our study highlights consideration of Stiff person syndrome as differential during assessing low back pain/stiffness cases particularly in those without significant lumbosacral spondylosis.

AOP27/425: Cerebral venous thrombosis mimicking as neuromyelitis optica spectrum disorder: An unusual case

Shinu Singla, Sharma PK

Department of Neurology, King George's Medical University, Lucknow, Uttar Pradesh, India

Typical presentation of cerebral venous thrombosis is headache, seizure, focal neurological deficit and papilledema. We had a case of cerebral venous thrombosis presenting with intractable nausea, and sequential vision loss mimicking as NMO spectrum disorder. Case– 11year old boy presented with headache and vomiting for 1 month and sequential vision loss (right then left) for 15 days. There was no history of fever, seizure, focal neurological deficit. On examination, Vitals were normal, GCS- E4V5M6, neck rigidity absent, vision: bilateral PL positive, pupillary reflex: normal. Bilateral INO was present. Fundus was normal. Rest examination was normal. His routine blood investigations, CSF and MRI brain were normal. Possibility of NMOSD (Area Prostrema Syndrome) was kept. Inj Methylpredisolone was started. Vision improved to bilateral hand movement. Oral steroid was started and patient was discharged. After 1 month, patient again presented with complaints of headache, persistent vomiting and deterioration of vision. There was no other focal neurological deficit except bilateral INO. On examination, vitals were stable, GCS- E4V5M6, vision: B/L PL positive, fundus – B/L papilledema. CSF NMO and anti MAG was negative. MRI brain with MRV showed cerebral venous thrombosis with petechial bleed. Serum APLA profile was sent. Anticardiolipin IGM antibody was positive. Anticoagulant was started. His vision started improving. Headache and vomiting resolved. Patient was discharged in a stable condition. Conclusion: CVT is a challenging condition due to its variability of clinical presentations. Outcome is favourable with early diagnosis and aggressive treatment.

AOP28/440: Clinical and radiological profile of patients with neuromyelitis optica spectrum disorders with a comparison between AQP4 positive/MOG Ab positive/seronegative groups

Reshma Sultana, Netravathi M, Bhat MD, Mahadevan A

Departments of Neurology and Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Background: Neuromyelitis optica spectrum disorders (NMOSD) is an immune mediated disorder consisiting of patients with AQP4/MOG antibodies. Few with antibody negativity to both called as seronegative (SN) NMOSD. Objectives: To study clinical & radiological profile of patients with NMOSD. Methods and Results: A prospective study of 42 patients with 21 (50%), 12 (29%) and 9 (21%) patients of AqP4, MOG and SN. The core clinical features affected in the patients were: (i) AQP4: myelopathy (71%), optic neuritis (ON; 52%), brainstem (24%) and area postrema (24%). (ii) MOG: myelopathy (83%), ON (67%), Brainstem (8%). (iii) SN: myelopathy (67%), ON (56%), brainstem (22%). MRI brain & Spine showed the following abnormalities in: (i) AQP4: Optic nerve hyperintensity (HI 59%), Obex (43%), Brainstem (38%), white matter (WM 33.3%), MCP (5%), Hypothalamus (5%). Spine: Cervical (52%), Dorsal (14%), Cervicodorsal (14%). (ii) MOG: optic nerve HI (41.7%), WM (25%), Obex (8%), brainstem (16.7%), MCP (8%), Spine: Dorsal (25%), Cervicodorsal (17%), whole cord (17%), Cervical (8%), Conus (8%). (iii) SN: Optic nerve HI (33%), WM (33%), Obex (22%), MCP (11%), Medulla (11%). Spine: Cervicodosal (22%), Dorsal (11%), Conus (11%). Conclusion: (i) Majority of the patients were AQP4 positive among the NMOSD group. (ii) Area postrema involvement was observed in only AQP4 patients, (iii) On MRI obex involvement was most commonly seen in AQP4 patients and very less in MOG and none in SN patients. The following differentiating features clinically and on imaging may help us in categorizing the patients prior to the availability of the antibody tests.

AOP29/447: Clinical and radiological profile of 57 myelin oligodendrocyte glycoprotein related neuromyelitis spectrum disorders patients

Netravathi M, Sheik RS, Oommen AT, Bhat MD, Anita M

Departments of Neurology and Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India

Background: Antibodies to myelin oligodendrocyte glycoprotein (MOG) have been suggested to play a role in a subset of patients with neuromyelitis optica and related disorders (NMOSD). Objectives: To study the clinical & radiological profile of NMOSD patients due to MOG-IgG. Methodology and Results: This is a prospective evaluation of 57 (M:F: 43.9%:56.1%) MOG-related patients between Jan 2018 and June 2019. Based on age group they were classified as pediatric (36.8%), adult-onset (59.6%) and late-onset (3.5%). There were total of 151 neurological episodes. Among them; ON constituted 44.4% followed by myelopathy (21.2%), brainstem affection (19.2%), Opticomyelopathy (4.6%) and others (10.6%). MRI optics showed hyperintensities (HI) in ON (56.1%), optic chiasm (16.7%), perioptic sheath fat stranding (16.7%), optic tract (14.6%). MRI brain showed HI affecting brainstem (68.8%), white matter (45.8%), grey matter (35.4%). MRI Spine: Dorsal cord was involved in 14.6% followed by dorsolumbar inc conus (10.5%), cervical (8.3%), cervicodorsal (4.2%), dorsolumbar (2.1%). In the acute period patients received only steroids in 20.3% and 76.3% received additional plasmapheresis. Maintenance treatment consisted of rituximab (47.5%), azathioprine (18.7%), mycophenolate (13.6%), steroids (13.6%), cyclophosphamide (3.4%). Conclusions: This study showed that: (i) male and female patients had almost equal predominance, (ii) The adult patients were in higher proportion compared to pediatric and adult-onset age group, (iii) Nearly half of the patients had optic neuritis as clinical manifestation, (iv) recurrent vomiting without hiccups was one of the salient clinical features. (v) Brainstem involvement was more common in MRI brain especially the pons and MCP.

AOP30/451: A case of LGI-1 encephalitis with guillain barre syndrome

Balambighai Vaiyapuri

Department of Neurology, Apollo Children's Hospitals, Chennai, Tamil Nadu, India

72 years old gentleman presented with weakness of both upper limbs and lower limbs, difficulty in walking for past one month. He had history of irrelevant talk for past ten days and acute retention of urine for one day. There was history of jerky movements of face and right upper limb one month back for which he was treated with oxcarbamazepine. He had history of taking ayurvedic medications for hypertension. On examination, he had bifacial weakness and power of 3/5 in both upper limbs and power of 2/5 in both lower limbs. His deep tendon reflexes were just elicitable in both upper limbs and were absent in lower limbs. Plantar response was flexor bilaterally. Blood investigations revealed low sodium levels which were corrected. MRI brain was normal and MRI cervical spine with whole spine screening showed spondylitic changes in cervical and lumbar levels. NCS showed demyelinating polyradiculoneuropathy of all four limbs (LL > UL). Heavy metal Screening was done which was negative. Serum and CSF autoimmune panel showed serum LGI-1 Antibody positive. A diagnosis of LGI-1 encephalitis with GBS was made and he was treated with IV steroids and plasma exchange. He improved with a power of 4/ 5 in upper limbs and 4-/5 in lower limbs and was discharged with oral steroids.

AOP31/470: One autoantibody (ANCA), different clinical manifestations, different imaging findings

Surendra Kumar, Kumar N, Bhoi K

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

Background: ANCA autoantibodies are associated with Wegener granulomatosis, Churg Strauss syndrome and Microscopic polyangiitis. ANCA associated vasculitis usually involves ear nose and throat, lungs, kidney, skin. Nervous system involvement is rare. Predominant or isolated nervous system involvement is rare. Materials and Methods: All patients admitted with ANCA positive vasculitis since last 1 year at AIIMS Raipur are taken. Results: We had 6 cases. There were 4 females. Mean age of onset of disease was 53.5 years. All patients were positive for p ANCA. Two patients had ptosis as the first symptom, in one patient gradually evolving with time to involve facial nerve, seizures, psychoses, encephalopathy over a period of 16 years. One patient had isolated vasculitic polyneuropathy. One patient had recurrent sinusitis, headache, hearing loss, dysarthria and altered sensorium. Two patients had headache, along with decreased attention and concentration and complex calculation difficulties in one and vertigo and hearing loss in the other. Three patients had past history of bronchial asthma and diabetes mellitus. MRI brain finding was different in each case. One patient had pachymeningitis, sialadenitis, pansinusitis. One patient had periventricular and subcortical T2 hyperintensities. One had fluffy cortical and subcortical white matter T2 and FLAIR hyperintensities. One patient had cortical and subcortical microbleed with infarct resembling cerebral amyloid angiopathy. In two patients, MRI brain was normal. All patients were treated with steroids and cyclophosphamide according to guidelines with marked improvement. Conclusions: ANCA associated vasculitis is an important rare group of diseases for neurologists. Diagnosis needs strong clinical suspicion.

AOP32/476: Clinical profile of anti-N-methyl-D aspartate receptor antibiody encephalitis and their one year folowup in a tertiary care hospital

Balambighai V, Dhanaraj M, Arulselvan VL

Department of Neurology, Apollo Children's Hospitals, Chennai, Tamil Nadu, India

Objective: To study the various clinical features of patients diagnosed with anti-N-methyl-D aspartate receptor (NMDA R) encephalitis and their one year follow up, admitted to Apollo Hospitals, Chennai over a period of two years. Introduction: Anti –NMDA receptor encephalitis is considered as a severe form of non infectious encephalitis due to immune response against the neuronal autoantigens with production of anti neuronal antibodies. These have been increasingly recognised due to availability of serum markers and better clinical characterizations. Methods: Patients admitted to neurology Department from January 2016 to January 2018 were studied retrospectively. Diagnosis of anti-NMDA receptor antibody encephalitis was based on diagnostic criteria for “definite” anti – NMDA receptor encephalitis. Results: Females were commonly affected than males. Clinical presentations include behavior problems, seizures and memory impairment in 100% of the patients, movement disorders in 55% of the patients, autonomic disturbances in 22 % of the patient. MRI showed temporal lobe hyperintensities in 55% of the patients. All patients were discharged with improvement of symptoms, however, one patient developed refractory seizures and secondary infection and expired after three months during follow up, all the remaining patients were almost 90% symptom free on one year follow up. Conclusions: Anti NMDA receptor antibody encephalitis is a potentially treatable cause of encephalitis. On one year follow up, 77% of the patients recovered completely in the aspects of behavioral and psychiatric manifestations. 77% of the patients were seizure free at one year. However memory impairment takes a longer time to recover.

AOP33/493: ANCA associated vasculitis mimicking as cerebral amyloid angiopathy in a 78-year-old gentleman

Surendra Kumar. Borkar D, Kumar CHA

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

A 78 year old gentleman presented with two episodes of seizures followed by post ictal confusion. He had similar history of seizure one year back which was followed by change in behavior, psychoses and dependence on others for daily activities of life. He was on anti epileptic drugs along with anti platelets, statins, donepezil and olenzapine. He was recently diagnosed diabetes for 6 months for which taking oral hypo glycemic agents. He had history of recurrent facial paralysis, first episode 12 years back and second episode 7 years back. He also had intermittent asthmatic attacks for last 6 year and taking intermittent inhalers for that. He also had history of left sided ptosis 16 years back. All this episodes improved with short course of steroids. His MRI revealed multiple microbleeds and chronic infarcts. His complete blood count was normal with normal eosinophil count. His ESR was 58mm in the first hour and CRP was high. His creatinine was between 1.5 to 1.6 for last 5 to 6 years. Thyroid function test was normal. EEG showed diffuse slowing suggestive of encephalopathy. Auto antibody panel showed strongly positive p ANCA with other ones negative. Patient was managed with steroids and cyclophosphamide according to guidelines. Patient showed improvement in sensorium such that now able to understand and follow commands. Importance: All cerebral amyloid angiopathy looking imagings are not hypertension related but it may also be related to Central nervous system vasculitis which offers chance to treate the patient differently.

Sunday, October 06, 2019, 07:30 AM-13:00 PM. Poster Hall Area

Poster Session 05: Miscellaneous

MiP1/11: Clinical and radiological profile of osmotic demyelination syndrome in a tertiary care hospital

Yadav PK, Roy AG

Department of Neurology, Vivekananda Hospital, Durgapur, West Bengal, India

Objectives: In this study the clinical and radiological features, etiological factors and clinical outcomes of patients diagnosed with osmotic demyelination syndrome (ODS). Methods: A total of 40 patients with clinical and radiological findings suggestive of ODS admitted in AIMS, Kochi from Jan 2009 - sept 2012 were included in the study. The data from these patients were analysed. Results: Out of 40 patients of ODS 17 patients had Extra pontine myelinolysis (EPM), 16 patients had Combined extrapontine and Pontine myelinolysis (CEPM) and 7 had Central pontine myelinolysis (CPM). 23 patients were males and 17 were Females. Mean Age was 56.8 yrs. Hyponatremia seen in 77.5% of study population. The most common cause was SIADH precipitated by infection mostly Urinary Tract Infection. 5 patients had evidence of Chronic Liver Disease and 6 were alcoholic. Clinical presentation varied and included Altered Sensorium in 19 patients, Extrapyramidal state (18), Locked in state (7), Ataxia (7), Dysphagia, Dysarthia and Dystonia in 3 patients each. MRI showed involvement of Thalamus and internal capsule commonly apart from the involvement of pons and basal ganglia. Diffusion Restricting lesions were seen in 9 patients. MRI was normal in 2 patients. 22 patients were treated with steroids. Dopaminergic drugs were used in most of patients with extra pontine myelinolysis. Botulinum Toxin was used in 2 patients with intractable dystonia. On discharge from hospital 15 patients were bedridden and 18 patients were ambulant. 2 of our patients had succumbed to their illness. Conclusion: ODS is a rare demyelination syndrome. Prompt diagnosis and management is needed for favourable clinical outcome.

MiP2/14: An unusual etiology of acute febrile encephalopathy

Halappanavar A, Ahmad FMH, Nanda S

Department of Neurology, Armed Forces Medical College, Pune, Maharashtra, India

A 42 years old lady, previously operated for Pituitary Macroadenoma by transnasal-transsphenoidal route (2009) became symptomatic with high grade fever and projectile vomiting. On day 03 of illness, she was noticed to be in altered sensorium by family members and brought to emergency. Initial evaluation revealed tachycardia, normal blood pressure and SpO2 96% at room air. CNS Examination: unconscious patient with GCS E1M4V1; anisocoria, non-reacting pupils and bilaterally extensor plantar response. Chest examination: normal. Blood smear, Paracheck for malaria, NS1Ag and IgG/IgM for dengue were negative. Initial Chest X Ray was normal. Started on broad-spectrum injectable antibiotics and supportive care. Contrast enhanced MRI Brain showed T2/FLAIR hyperintensities in bilateral posterior basifrontal region, mammillary bodies and hypothalamus. CSF examination revealed clear appearance, WBC 08/cumm, RBC 120/cumm, Protein 58 mg/dl, Sugar 93 mg/dl and globulin was not raised. Throat swab for H1N1 by TaqMan RT-PCR was positive (received on day 5 of hospitalization) and started on Tab Oseltamivir. However, her condition went downhill and finally succumbed on day 5 of hospitalization. CSF for Herpes Simplex Virus by DNA-PCR and Japanese Encephalitis virus IgM by ELISA were negative (received post-mortem). Conclusion: Acute influenza-associated encephalitis in adults is rare and a diagnostic challenge. It can present without usual flu-like symptoms as in our case which highlights importance of considering this diagnosis with present H1N1 endemicity.

MiP3/26: Serotonin syndrome: An observational study of 20 patients

Patel J, Prakash S, Rathore C

Department of Neurology, Dhiraj Hospital, Sumandeep Vidyapeeth, Vadodara, Gujarat, India

Introduction: Serotonin syndrome is characterized by a symptom triad of altered mental status, neuromuscular abnormalities, and autonomic dysfunction. The degree of symptoms can range from mild to severe. it occurs mostly due to excess intrasynaptic concentration of serotonin. There are limited number of studies available for serotine syndrome. Methods: This observational study was done in adult patients (>18 years) attending neurology and psychiatric department between August 2018 and Feb.2019 fulfilling the Hunter's criteria for SS. All patients were subjected to a detailed clinical history and examinations. Patients were subjected to appropriate investigations to find out the other causes. Results: The mean age was 36.6 years (range: 18–59 years). Sixty-five percent of patients were male and thirty five percent of patients were female. There were 15 different underlying clinical syndromes for which serotonergic drugs were started. Psychiatry conditions (45%) and cough/respiratory tract infection (15%) were the two most common clinical conditions for starting serotonergic drugs. We noted different symptoms and physical signs. Overall, tremor (60%) and dizziness (40%) were the two most common symptoms. Fever (30%) and dizziness (30%) were the two most common initial (or first) symptoms. Conclusions: This study represents a small clinic-based study on SS. SS is not rare in clinical practice. However, various aspects of this syndrome are still to be determined. All patients on serotonergic drugs should be physically examined for the presence of SS on the development of any new symptom.

MiP5/45: SSPE presenting as anton syndrome-interesting rare manifestation of a relatively rare disease

Saurabh K, Joshi D

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

SSPE usually presents with cognitive decline and myoclonus in a previously measles infected child or adolescent. But sometimes rare manifestations can leave us unsuspecting untill disease is confirmed by diagnostic tests. One such presentation is Anton syndrome (with patient denying any visual deficit). A patient named Tabassum Bano,17 yr/F was admitted in our ward with history of acute onset difficulty in vision, in form of banging into objects while walking, not able to find objects like tv remote placed in front of her etc, but patient outrightly denying any visual loss. On examination vital parametres were within normal limit, patient was not able to count fingers correctly, menace reflex was absent, pupils were normally reactive to light. After confirming bilateral occipital lesion on MRI, work up for differentials of Anton syndrome was initiated, with emphasis on demyelinating illness and stroke. EEG was also done which surprisingly showed large amplitude periodic complexes, leading to suspision of SSPE, which was later confirmed by CSF studies. This should not come as a surprise, as though SSPE usually presents with cognitive decline and myoclonus, it preferrentially involves parieto-occiptal region of brain. Anton syndrome is described as a condition in which patient deny their blindness despite objective evidence of visual loss and moreover confabulate to support their stance. It is a rare extension of cortical blindness, in which, in addition to injuries to occipital cortex, other cortical centres are also affected, with patient typically behaving as if they were sighted.

MiP6/55: Neurological disorders in puerperium: A tertiary care experience

Farooq O

Department of Neurology, Government Medical College, Srinagar, Jammu and Kashmir, India

Background: Puerperium is the period after child-birth during which the mothers reproductive system returns to its normal prepregnant state. It lasts six to eight weeks and end with the first ovulation and the return of normal menstruation. A variety of neurological disorders may be encountered during puerperium. The primary neurological disorders studied in relation to puerperum include stroke, epilepsy, benign intracranial hypertension, demyelinating diseases of central and peripheral nervous system and peripheral neuropathy. Materials and Methods: A prospective observational study wherein the clinical profile of patients presenting with neurological disorders was studied. Patients with pre-existing neurological disorders were excluded from the study. Detailed history, examination and relevant investigation like CT head, MRI brain, EEG. were done and statistical analysis using Microsoft Excel spreadsheet was done. Results: Out of 36550 hospital deliveries in 2 years, 74 patients had postpartum neurological complications giving an incidence 337 per lac deliveries. Majority of patients were in their 3rd and 4th decade of life. Majority of patients had PRES and almost equal percentage 15% patients had GBS and ischemic stroke and 12.2% had cerebral venous thrombosis. Majority of our patients had generalized tonic clonic seizures. Majority (44.6%) of our patients (60.8%) had undergone caesarean section while 29 (39.2%) had normal vaginal delivery. Conclusion: Posterior reversible encephalopathy syndrome PRES and ischemic stroke (PRESS) emerge as the predominant neurological disorders during puerperium with a substantial contribution by peripheral neuropathy, cerebrovascular disease and CNS infections. Appropriate management is required to optimize the maternal and foetal outcome.

MiP7/68: CNS histiocytosis - Rare disease with a rare presentation

Sadekar S, Chaudhary JR, Vani S

Department of Neurology, Manipal Hospital, Bengaluru, Karnataka, India

Four year old girl presented with an acute onset of diplopia for one week, no signs of raised ICT and a normal ophthalmological examination. On examination - slow ocular saccades, diplopia upon looking sideways and downwards. Entire blood and CSF work up was normal except for CSF sugar -32. MRI Brain revealed patchy enhancing ill defined lesions in tectal pons, medial cerebellum. Low Grade Glioma and demyelination were considered. PET CT was normal, she received two courses of pulse steroids, each a month apart following which there was transient clinical and radiological resolution with symptoms recurring after few days. The lesion was biopsied which showed abundant layers of foamy histiocytes (MIB 1 positive stain) this was a case of CNS histiocytosis. Prevalence of histiocytosis is 1-2/1,00,000, CNS involvement is very rare. Histiocytes are of dendritic cell lineage, histiocytosis results from abnormal proliferation of immune cells. Literature review shows diagnosed cases as girls between 2- 18 years, MRI features of granulomatous deposits in calvarium and long bones, hyperintensities in posterior pituatory and occasionally in the brainstem. Patients present with diabetes insipidus, body aches and headache (bone deposits) and visual loss if the granulomas involve the optic nerve. This patient did not have any of the above features. She presented with isolated involvement of cerebellum, with a fluctuating course of symptoms causing diagnostic dilemma. Histopathology and immunohistochemistry remain the mainstay of diagnosing CNS Histiocytosis.

MiP8/74: Rare case of camptodactyly

Veera S, Veera SRL, Veera R, Veera A, Veera T

Department of Neurology, Osmania Medical College, Hyderabad, Telangana, India

Camptodactyly is a relatively rare hand anomaly, and this condition involves varying degrees of flexion contracture of the fingers at the proximal interphalangeal (PIP) joint, unilaterally or bilaterally . The fifth finger is mostly involved. 15 yrs girl presented with deformity of all fingers since 5 yrs. Initially started in right hand, ring finger, progressed to other fingers in 1month. Static since then. No history of similar complaints in family members. On examination contractures of PIP joints in both upper and lower limbs, normal dorsal joint creases were oblitereated. Hallux valgus deformity present. Neurological examination is normal. Non syndromic camptodactyly of hands and feet is rare condition. Nonoperative and operative techniques have been proposed to treat this condition, depending on its clinical severity. These diverse techniques range from splinting or stretching exercises to release of tendons, fascial bands, transfer of muscles, and tenotomy.

MiP9/84: Clinico-radiological correlation of calcification in CT brain

Alagamuthu V, Rajasekaran

Department of Neurology, K.A.P. Viswanatham Medical College, Tiruchirappalli, Tamil Nadu, India

Introduction: Intracranial calcification is the most common finding in CT brain. Neurocysticercosis is an endemic disease of Tamil Nadu and one of the most common causes for calcification. Its clinical manifestations are variable may includes seizures, headaches and focal neurological deficit etc., which is influenced by number and location of the lesion. Aim: We aimed to assess the clinical profile of patients in relation to calcification in CT BRAIN who seeks medical advice. Materials and Methods: The study is conducted in the outpatients department in department of Neurology, Mahathma Gandhi Memorial Government Hospital-Trichy, between June 2018 to May 2019. All Patients with calcification in CT brain were enrolled in this study. Exclusion criteria: Bilateral Basal Ganglia, Physiological, Neoplastic, Intracranial Vacular calcification. As many as 70 patients with intracranial calcification, Average age of the patient in our study 35.74 with female predominance (80%). In which 36 (51.4%) showed single calcification, rest showed (48.6%) multiple calcification. On analysing of location of single calcification 38.8 % (14 patients) showed frontal, 22.22% (8 patients) showed parietal, 11.4 % (4 patients) showed occipital, 11.4% (4 patients) showed thalamic, 5.6% (2 patients) showed temporal calcification. Among the 70 patients about 34 (48.57 %) were reported to have headache, about 6 (8.57 %) were diagnosed with Seizure, 28 (40%) patients were presented with both the headache and Seizures. Conclusion: In our study headache (48.57%) is the most common clinical presentation followed by head ache and seizures in 40 % of the patients. Description of headache and location of calcification will be discussed.

MiP10/92: Clinical profile of IIH with radiological signs and correlate of these signs for diagnosis of IIH

Dharavath E, Veena N, Kumar KS

Department of Neurology, Gandhi Medical College and Hospital, Secunderabad, Hyderabad, Telangana, India

Introduction: IIH is a disorder characterized by raised CSF pressure and is primarily a diagnosis of exclusion. MRI features seen in cases of IIH include slit like ventricles, empty sella, flattening of the posterior sclera, dilatation or tortuosity of the optic nerve sheath or gadolinium enhancement of the optic disc and distention of perioptic subarachnoid space. Methods: Patients with the diagnosis of IIH satisfying the modified Dandys criteria, who presented to the Neurology Department, will be included in the study after obtaining a written informed consent. Results: In our study, middle aged obese females were commonly affected, the mean age being 40 years. The patients in our study presented with headache (94%), transient visual obscurations or blurring (68%), pulsatile synchronous tinnitus (58%), pain behind the eyes (44%), double vision (38%), visual loss (30%), pain with eye movement (22%). In our study, findings of slit like frontal horns of bilateral lateral ventricle and empty sella related to the raised intracranial pressure were found in all the patients. Findings related to optic nerve such as vertical kinking of optic nerves, prominence of CSF space around optic nerves were present in 15 out the 20 patients. Conclusion: According to our study, slit like ventricles, empty sella, vertical kinking of optic nerve, prominence of CSF spaces around optic nerve and venous sinus abnormalities detected on MR venography are important neuroradiological markers of IIH.

MiP11/93: A rare form of mercury neurotoxicity following native medications

Ganesh V, Kannan V, Chandramouleeswaran V, Narasimhan NR

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

Introduction: Morvan syndrome is an autoimmune disease of central and peripheral nerve system mediated by VGKC (voltage gated potassium channel) complex antibodies. Mercury toxicity following native medications has been reported to be associated with Morvan syndrome rarely. We report two cases of this rare syndrome following native medications. Case Report: Our cases took native medications for constipation and sinusitis and presented with subacute onset of symptoms such as tremulousness, diffuse body pain, generalised muscle twitching, sweating, minimal weakness and insomnia. Both the patients had elevated serum mercury level, positive serum CASPR2 Ab and normal CT chest and abdomen. EMG showed features of fasciculation potentials, myokymia and neuromyotonic discharges. Both cases were advised to stop native medications and treated with intravenous methylprednisolone, plasmapheresis/ immunoglobulin and put on oral prednisolone maintenance dose. Both the patients showed partial improvement in their symptoms while discharge and are on follow up. Discussion: Native medication uses heavy metals especially mercury after purification and detoxification in preparation of medicines. Mercury causes Morvan syndrome by (1) direct toxicity - damaging VGKC complex or (2) autoimmune mediated - inducing production of antibodies against VGKC complex proteins such as LGI1 and CASPR2. Mercury toxicity associated Morvan syndrome is treated by immunotherapy and native medications discontinuation. Conclusion: All patients with features of neuromyotonia should be enquired about native medications intake and screen for heavy metals as they carry good prognosis after stopping native medications.

MiP12/99: A study of etiological profile of extra ocular muscle and nervepalsies

Goli N, Rangalakshmi S, Ramesh, Archana, Rao T

Department of neurology, Osmania General Hospital, Hyderabad, Telangana, India

Objective: To evaluate the myriad causes ofextraocular muscle and nerve palsies. Materials and Methods: Inclusion criteria: Patients attending Department of Neurology, Osmania General Hospital with clinical features of extra ocular muscle palsy. Exclusion criteria: Traumatic cases were excluded from this study. It is a Descriptive study conducted over a period of 1 year from January 2018 to December 2018. Data regarding history and detailed physical examination was collected and required investigations were performed. Results: A total of 88 cases were studied. Among them Isolated 3rd cranial nerve palsy were 27, Isolated 6th cranial nerve palsy were 23 and one case was Isolated 4th cranial nerve palsy. Combined nerve palsies were 22, Myasthenia cases were 11, Myopathy cases were 4. Among isolated 3rd cranial nerve palsy, diabetes was the most common cause affecting 11 members. Among isolated 6th nerve palsy, common etiology was idiopathic affecting 8 people, followed by meningitis affecting 4 people. Most common etiology for combined cranial nerve palsies was cavernous sinus pathology. Conclusion: 3rd cranial nerve is the most frequently involved nerve and is mostly involved in diabetes. Cavernous sinus pathology and Myasthenia were common etiology for multiple cranial nerve and mucle palsies respectively.

MiP13/106: EEG pattern in somnambulism

Venkata Narayana Randhi, Chaudhary N

Department of Neurology, Seven Hills Hospital, Vishakapattanam, Andhra Pradesh, India

Introduction: Somnambulism is a parasomnia disorder in which a child, less often an adult, sleepwalks. It occurs almost exclusively during stages 3 and 4 of a NREM sleep. Very few cases have been reported to have abnormal EEG. In largest individual series of 22 patients Turan Atay M.D reported EEG abnormality in only 4 patients (PUBLISHED IN 2000). Aims: To present the EEG abnormalities of two young patients of somnambulism and to discuss its implication on treatment. Materials and Methods: Two young boys (aged 11 years and 13 years) of sleep walking and complex behaviour presented to our neuro clinic in last three years. Necessary investigations were done. Apart from sleep EEG and polysomnography. Results: Clinically first boy had history of multiple symptoms of parasomnia like enuresis nocturna, night terrors, nightmares, confusional arousal and bruxism. The second boy has the family history of parasomnia. In both the cases EEG was done. Abnormality was found in both awake and sleep EEG. It showed abnormal pattern with spikes and generalised epileptiform activity. One was treated with antiepileptic (clonazepam) and the second one was put on quetiapine. Both responded well to the treatment. Conclusions: Though abnormal EEG is rare in somnambulism but if found the patient responds well to anticonvulsant and atypical antipsychotic drug.

MiP14/108: Progressive multifocal leukoencephalopathy: An interesting case report

Nagaraj AR, Nithyanandam A, Sarala G, Lakshminarasiman R

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

Introduction: Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by reactivation of latent JC virus in immuno-compromised individuals. PML remains an important complication of HIV-1 infection. Long-term survival in patients with PML is increasingly common in human immunodeficiency virus (HIV)-infected people treated with highly active anti-retroviral therapy (HAART). Case Senerio: A 51yr old female presented to our institute with memory disturbance since 2 months and difficulty in using right upper and lower limb since 1 month. Past history of pulmonary koch's 25yrs back for which she was treated with ATT. Examination of lobar function test showed dorsolateral prefrontal cortex, bilateral parietal lobe and medial temporal lobe involement. Spinomotor system examination reveals Spastic ataxic hemiparesis .Right side cerebellar signs were present. Cranial nerves, autonomic system, extrapyramidal systems were normal. Investigations revealed HIV positive status, MRI brain with contrast shows asymmetrical T2 flair hyperintensity lesion noted in bilateral cerebral hemisphere (left> Right). CD4 count was 201 cells/mm3. CSF for JC virus was negative. Diagnosed as possible Progressive multifocal leukoencephalopathy based on compatible clinical features and radiological findings. She was treated with ART- TLE regimen and Co-trimaxazole prophylaxis. Conclusion: PML is rapidly fatal in cases of advanced HIV infection. This disease usually occurs in individuals with low CD4+ T-lymphocyte counts. As PML remains an important cause of morbidity and mortality in HIV-infected patients so high index of suspicion should be there for diagnosis and management.

MiP15/116: Unsual presentation of a third nerve palsy in a diabetic patient

Haritha Kumari Pachuru, Nithyanandam A, Sarala G, Narasimhan LR

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

In adults, the most common cause of acute oculomotor palsy is microvascular ischemic third nerve palsy. This occurs in the setting of vasculopathic risk factors such as diabetes, hypertension, dyslipidemia, and advanced age. Pain with diplopia, drooping of eyelid is a common feature of oculomotor cranial nerve palsies from vasculopathic causes or compressive lesion. Resolution of symptoms within 3 months and pupillary sparing is usual in vasculopathic cause. In our case persistant symptoms and signs with mid dilated irregular sluggishly reacting pseudophakic pupil, inspite of good glycemic control provoked us to search for structural lesion.

MiP16/119: To study the etiological profile of noncompressive myelopathies in a tertiary care hospital of North-West India

Yavnika Jain, Jain RS

Department of Neurology, SMS Medical College, Jaipur, Rajasthan, India

Introduction: Till now only 3 studies have been carried out on non- compressive myelopathy in 3 different zones: North-East India by Gauhati medical college, Eastern India – by Bangur institute of Neurology and S.S.K.M hospital and North India by PGIMER. Aim: 1. determine the causes of non-compressive myelopathies 2. To study the clinical and radiological features of non- compressive myelopathies. Methods: An observational study is being carried out in the Neurology Department of SMS Medical College, JAIPUR, from feb 2018 to 2020. All patients undergo MRI SPINE to rule out compression and are then segregated in 2 categories-acute-to-subacute myelopathy and chronic myelopathy. MRI brain, CSF analysis, and immunological, infectious, and metabolic profile and other additional investigations and imaging relevant to clinical cases are included. Exclusion criteria includes patients with myelopathy who donot undergo MRI spine, spinal cord compression, motor neuron disease, degenerative ataxias and trauma. Results: Out of 36 patients studied so far, 28 patients had LETM, 2 had myelitis (<3 spinal segments), 6 did not have any spinal lesion. Cause could not be determined in 14 (38.8%) out of 36 cases, however, remaining 22 showed maximum cases of tubercular myelitis (19.4%), MOG/NMO-SD cases (19.4%), Vit. B12 deficiency (SACD) cases (13.8%), arterio-venous malformation (5.5%), and 1 case of neurosarcoid. Conclusion: One of the important cause of noncompressive myelopathy emerged out to be tubercular origin in addition to NMO-SD . SACD is another important cause in Indian population due to food habits.

MiP17/122: Rare case of type 1 sialidosis with novel mutation in NEU 1 gene

Anusha Challa, Raju GB, Gopi S, Kumar TS, Kumari UA

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

Introduction: Sialidoses are rare autosomal recessive, lysosomal storage diseases caused by NEU1 gene mutations on chromosome 6p21.3. Incidence: 1 in 50,00,000 live births. Type 1 - “cherry red spot-myoclonus syndrome” develop myoclonic epilepsy, visual impairment and ataxia in second or third decade of life. Progressive Myoclonic Epilepsies were considered as differential diagnosis. Case Report: 14 year old boy, first child of non consanguineous married parents with normal natal history and developmental milestones had progressive gait & limb ataxia since 2 years, dysarthria, action induced myoclonic jerks, sensitive to auditory stimuli and normal cognition and vision. Parents and siblings were asymptomatic. Examination: scanned speech, limb, truncal and gait ataxia, gaze evoked nystagmus, hypotonia, hyper reflexia with ankle clonus, cherry red spot on fundus examination, extensor plantar reflex, gingival hypertrophy. No dysmorphic features & no hepato splenomegaly. Cognitive examination was normal. Myoclonic jerks were partially controlled with valproate and clonazepam. Investigations: EEG showed Temporo Occipital spikes, generalized poly spikes with video correlation of myoclonic jerks –Cortical Myoclonus. MRI Brain, NCS, Thyroid profile & ultrasound abdomen were normal. Genetic testing with Next Generation Sequencing revealed Homozygous Splice site proximal variation (c.1021+4A>T) in the NEU1 gene and variant is damaging by Mutation Taster 2 which concluded that this novel variant alters the protein function. Presently, this pathological variant was not found in homozygous state in the general population. Conclusion: This case highlights Classical phenotypic features of Sialidosis Type 1 with novel mutation in NEU1 gene.

MiP18/124: Atypical presentation of idiopathic intracranial hypertension

Livingston KJ, Nithyanandam A, Sarala G, Lakshminarasimhan R

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

Idiopathic intracranial hypertension is a secondary headache disorder characterized by headaches and visual symptoms. Most frequently occurs in obese women of childbearing age. Prompt recognition, evaluation and treatment are needed to prevent permanent visual loss. Case scenario: 38 year old obese women presented with complaints of headache, swelling of left eye for 4 months duration without visual symptoms. No history suggestive of CNS infection/head injury. Past history of migranous headache for 15 years. No history of OCP or steroid intake/thyroid disorder. General examination revealed left proptosis without thrill/bruit.Neuroopthal examination: visual acuity - 20/20 in both eyes, colour vision-normal, visual field - normal. Fundus - disc margin clear, extraocular movements free and full. MRI-brain with orbit showed bilateral tortuous optic nerves with prominent perioptic cistern, partial empty sella with relative proptosis on left side suggestive of idiopathic intracranial hypertension. MRA with MRV – normal. CSF opening pressure was 34 cm of H2O, acellular with normal protein. Automated perimetry showed no evidence of enlarged blind spot or field defect. CT - orbit normal. Routine investigations normal. Patient was started on acetazolamide and symptoms resolved gradually. Conclusion: Any patient with chronic daily headache with recent exacerbation even without visual symptoms,IIH has to be considered. It has to be diagnosed early to prevent visual loss. Peculiar feature in this case is presence of monocular proptosis without papilledema with tortuous optic nerve.

MiP19/130: Case of isolated velopharyngeal insufficiency: A rare presentation

Tushar VP, Jawahar M

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

Acquired isolated velopharyngeal insufficiency is a rarity. Usual presenting feature acute onset bulbar palsy- nasal regurgitation to liquids, nasal intonation and asymmetric palate on examination, more commonly in children. Most common cause is post viral infections like diphtheria. Here is a case of acute onset isolated velopharyngeal insufficiency in 13 year old boy. His presenting complaints were nasal regurgitation to liquids and nasal intonation of speech of 3 weeks. It was maximal at onset, non progressive. No difficulty in comprehension, fluency or repetition. No past, personal or family history. Examination, vitals stable. Higher function examination normal. On cranial nerve examination, no asymmetry in palatal arch. Uvula midline. On phonation, no palatal movements bilaterally. Gag reflex present bilaterally. Sensations preserved. Other system examination normal. Blood investigations like complete blood count, CSF studies, blood and throat swab culture, viral markers normal. Electrophysiological studies including NCS, RNS were normal. ENT consultation showed normal vocal cords. MRI brain normal. Isolated velopharyngeal insufficiency is rare and usually seen in 1st decade. The usual causes are trauma (tonsillectomy, adenoidectomy), infections (diphtheria, polio), Neuromuscular disorders (GBS, MND), cranial vessels (ICA aneurysm, Vascular insult), brainstem lesions (Syringobulbia, posterior fossa tumors). Usually runs a self limiting course and there is complete recovery in 85% cases. A medline search for similar cases done from 1960 to 2012 showed 36 cases of which 73% were males and were mainly in children. There was complete recovery in 2/3rd cases and without treatment in 89% of them. Our patient showed symptomatic improvement.

MiP20/139: Cervical flexion myelopathy: Atypical hirayama: A rare case report

Avinash Alashetty, Govindarajan S, Allimuthu N, Nagaraj AR, Ranganathan L

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

Hirayama disease (HD) is a rare disease commonly seen in India and Japan presenting as monomelic amyotropy. We report a case of a 25 year old male presented with weakness and wasting of right hand since 8 years and left upper limb extending up to shoulder and parascapular region since 6 years. The weakness was static after course of 2 years. He developed recent onset spastic weakness of both lower limbs since 2 months. Examination revealed wasting in thenar and hypothenar muscles and clawing of both hands, left shoulder girdle and parascapular muscles and right thigh muscles, with characterisitic sparing of the right brachioradialis. The power in right UL was 4 and 2 in left UL and LL power was 4. Stretch reflexes were absent in LUL, normal in RUL except for triceps which was absent and LL reflexes were exaggerated with sustained ankle and patellar clonus on right side and ill sustained on left side. No signs of sensory, autonomic, cognition, cranial nerves, cerebellar and extrapyramidal involvement noted. Cervical MRI revealed thinning of cord at C5C6 level with intramedullary hyperintensity with multiple flow voids in posterior dural space. Dynamic MRI showed widened posterior dural space on flexion and anterior disaplacement of posterior dura causing cord compression. We report a rare of HD with many atypical features. Our patient had bilateral upper limb involvement with parascapular involvement. Pyramidal sings were attributed to be cervical flexion induced myelopathy in long standing patients with HD. Cervical flexion induced myelopathy in Hirayama is a rare entity and few cases have been reported worldwide.

MiP21/144: Case report: A rare case of adrenoleukodystrophy

Noorul Ameen, Ameen SN, Shanmugasundaram N, Balasubramanian S, Narasimhan RL

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

Introduction: X-linked adrenoleukodystrophy is a metabolic, peroxisomal disease affecting the nervous system, adrenal cortex and testis resulting from inactivating mutations in ABCD1gene which leads to impaired peroxisomal beta-oxidation resulting in accumulation of very long chain fatty acids in various tissues. Case Report: A 22 year old male presented with complaints of difficulty in walking due to weakness and tightness involving both proximal and distal muscles of left lower limb and unsteadiness of gait since 7 years of age followed by reduced distant and colour vision 6 months later. By 13 years, patient started to develop tremulousness of both hands, urinary urgency, precipitancy and urge incontinence. For past 4 months patient had reduced penile tumescence and difficulty in swallowing both solids and liquids. Personal and family history was insignificant. On general examination, patient had generalised hyperpigmentation . His neurological examination revealed reduced visual acuity, impaired colour vision with normal fundus, bilateral pyramidal signs with left cerebellar signs without any sensory involvement. His basic investigations were normal while MRI brain showed bilateral symmetrical T1 hypointense, T2 hyperintense signals in peritrigonal, parietal, occipital, temporal lobes white matter regions suggestive of Adrenoleukodystrophy. His Serum cortisol, ACTH levels and nerve conduction studies were normal. Conclusion: Any young male patient with pyramidal signs, poor scholastic performance, visual or auditory impairment with or without a positive family history should raise a high index of suspicion for this condition. This case is being reported for its rarity.

MiP22/147: Thyrotoxic periodic paralysis: A rare case report

Ravi Patel, Suthar N, Shah T, Sharma P

Department of Neurology, AMC MET Medical College, Ahmedabad, Gujarat, India

Thyrotoxic periodic paralysis (TPP) is a rare complication of hyperthyroidism characterized by episodes of muscle weakness and hypokalemia. TPP is typically present in young Asian male, female, and non-Asian ethnic group can also be affected. TPP is a curable cause of hypokalemic periodic paralysis, can often be the first manifestation of thyrotoxicosis. Factors such as high carbohydrate diet, strenuous exercise, emotional stress, hypothermia and steroid can precipitate an attack of TPP. The presence of both hypokalemia and elevated level of thyroxine (T4) & Triiodothyronine (T3) are important diagnostic features during the acute episode. Treatment of TPP involves two steps, immediate action to reverse the paralysis by correction of hypokalemia followed by measures to prevent future attacks by restoration of a euthyroid state. We present the first case of TPP to our hospital of 32 year male presented with bilateral lower limb weakness followed by bilateral upper limb weakness for 3 days diagnosed with TPP soon after admission and successfully treated initially with potassium supplementation and put on regular treatment with carbimazole. (Restoration of euthyroid state).

MiP23/165: An etiological study of ventriculomegaly

Srikanth Loya

Department of Neurology, Osmania Medical College, Hyderabad, Telangana, India

Introduction: Ventriculomegaly is defined as dilatation of lateral ventricles at level of atria more than 10mm.Common causes of ventriculomegaly include structural causes obstructing Cerebrospinal Fluid outflow, Cerebral atrophy and infections. The prognosis depends on the underlying etiology Besides the cause, prognosis depends on progression of ventricular dilatation. Aim: To study various etiologies of ventriculomegaly. Methods and Methodology: This study was done in Osmania General Hospital. A total of 55 Patients with imaging finding of ventriculomegaly were included in the study. Study period is from January 2019 to June 2019. Results: Out of 55 patients included in the study, 53% were females and 47% were males. More than 65% patients in the study are observed in children and elderly age group. The various etiologies found in the study were obstructive hydrocephalus (46%), communicating hydrocephalus (16%), 11% cases were due to exvacuodilatation secondary to cerebral atrophy and 10% cases were Normal Pressure Hydrocephalus. Among the obstructive hydrocephalus patients 38% had aqueductal stenosis and 35% had posterior fossa tumours as etiology. Conclusions: The predominant cause of ventriculomegaly found in this study was obstructive hydrocephalus. Obstructive hydrocephalus was found commonly in infants and young age group. Ventriculomegaly secondary to cerebral atrophy were more common in elderly people.

MiP24/202: Nutrition and neurology

Rajiv Anand, Aarti, Handa R, Prasad A

Department of Neurology, BLK Super Speciality Hospital, New Delhi, India

We present two cases of B12 deficiency with atypical features and atypical response to supplement treatment with injectable Methycobalamine. First case is a young boy with pancytopenia and mania for which he was admitted with hematooncology as his brother had hairy cell leukaemia. He was also having mania and was on psychiatric treatment. He was investigated and treated for megaloblastic mania. Second case presented as a case of cervical myeloradiculopathy with B 12 deficiency not responsive to B12 supplementation. Investigation revealed hypothyroidism with anti TPO positive antibodies and required additional methylprenisolone for response.

MiP25/235: Acute intermittent porphyria presenting as pancreatitis, posterior reversible encephalopathy syndrome with hyperglycemia

Swathi Alluri, Reddy C, Chaudhuri JR, Goushuddin M, Rajini R

Department of Neurology, Yashoda Hospitals, Hyderabad, Telangana, India

Background: Acute intermittent Porphyria (AIP) is an inherited -metabolic syndrome of heme biosynthesis occurs due to deficiency of the porphobilinogen deaminase leading to cascades of accumulation of various metabolites in the blood. AIP can affect autonomic, peripheral and Central nervous system leading to varied clinical presentation. Hypertension leading to PRES is a rare clinical manifestation. Symptoms of AIP improves with increase in blood glucose as supported by decrease in acute episodes in patients with AIP after developing Diabetes. Here we report a adolescent girl with two rare features of AIP. 1-PRES, 2 -Hyperglycemia at presentation. Clinical Presentation: A 15yr old girl presented with recurrent episodes of abdominal pain, on evaluation diagnosed to have acute Pancreatitis. During symptomatic management after 5days She developed severe headache, seizures and bilateral visual blurring with Blood Pressure 170/110mmhg. Results: RBS 210mg/dl, Amylase and Lipase were elevated and CT Abdomen revealed swollen head of pancreas. MRI Brain revealed symmetrical parieto occipital T2 /flair hyperintensities suggestive of PRES. Urine for Porphobilinogen - spot positive and 24hrs elevated. Autoimmune profile was negative. Considering diagnosis of PRES with AIP, she was treated with iv dextrose, anti-hypertensives, leveteracetam. Symptomatically improved over 2 days. Conclusion: This case is a very rare association of AIP associated with PRESS, Pancreatitis and Hyperglycemia. First line of treatment for AIP remains to be Hematin/dextrose. Association of AIP and Hyperglycemia is indeed very rare.

MiP26/236: Wilsons disease with focal demyelinating lesion

Goushuddin Mohammed, Alluri S, Reddy C, Chaudhuri JR, Rajini R

Department of Neurology, Yashoda Hospitals, Hyderabad, Telangana, India

Wilson's disease is an autosomal recessive condition with ceruloplasmin deficiency, copper accumulation in liver and brain particularly in the basal ganglia, thalamus, midbrain. It presents with hepatitis or hepatic decompensation commonly. Neurologic manifestations are late and include movement disorders, personality changes and seizures. Magnetic resonance imaging (MRI) brain shows high signal changes in putamen, lentiform nucleus, thalamus, and brainstem. White matter lesions with mass effect are rare. We report a child of Wilson's disease diagnosed 3 years ago. Patient was treated with penicillamine and zinc. Patient was not tolerating penicillamine, so it was substituted with trientine. Patient discontinued treatment 6 months back. Now presented to us with headache, involuntary movements, left focal seizures and had large demyelinating lesion in right frontal lobe causing mass effect. After ruling out infective, primary demyelinating and infiltrative etiologies by MRS, CSF analysis, possibility of gyral and white matter involvement due to wilsons disease is considered. Atypical MRI characteristics should be considered in patients with clinical signs of neurological involvement in Wilson's disease as it is a devastating but treatable disease.

MiP27/237: Brown vialetto van laere syndrome: are we missing some genes?

Pramod Dhonde, Mayura Dhonde

Department of Neurology, Dhonde Hospital, Nanded, Maharashtra, India

19 yrs old female presented with holocranial headache followed by bilateral sensorineural hearing loss (SNHL). After 6 months she started having dyspnea on exertion with generalised fatiguability. She was worked up for cardiac, respiratory as well as for myasthenia which was negative. Her nerve conduction studies were suggestive of acute motor axonal neuropathy. She received immunoglobulins with prolonged ventilatory support from which she recovered partially. She remained mild dyspneic for 3-4 years. After 4 years she had significantly increased dyspnea and fatiguability, for which she required ventilatory support. On examination her vitals stable with persistent tachycardia. Upper limbs distal wasting present. Power in upper limbs proximal Gr IV and distal Gr III/IV and Lower limbs Gr IV. Deep tendon reflexes absent in both upper and lower limbs. Tongue atrophy noted with mild bifacial weakness. Electrophysiology revealed profuse fibrillation in tongue with chronic denervation seen in upper as well as lower limbs. Sensory conduction study normal. RNS was negative for decrement. Her Acetylcholine receptor antibody was negative. In view of Bilateral SNHL, tongue atrophy, dyspnea related to neuromuscular weakness, diagnosis of Brown Vialetto Van Laere (BVVL) syndrome made and started on carnitine and high dose riboflavin, but without much improvement. She has been tested for SLC52A2 and SLC52A3, both turned to be negative. In view of classical phenomenology with negative testing for BVVL syndrome, further evaluation and more understanding needed in this disorder.

MiP28/263: Acquired Ondine's Curse – A rare case report

Pankaj Kumar Sharma, Suthar N, Chauhan B, Solanki D, Amdani S

Department of Neurology, AMC MET Medical College and Sheth LG Hospital, Ahmedabad, Gujarat, India

We report and discuss rare case of a 65 year old man who presented with history of sudden loss of consciousness followed by a backward fall while he woke up for early morning chores. He was a known case of hypertension on treatment. Patient was attended in a stuporous condition and kept on ventilator support. After he became conscious it was observed that the patient was needed to be 'reminded' in order to take spontaneous breath. MRI Brain of the patient revealed normal supratentorial structures with type 1 CHIARI malformation with downward ectopia of cerebellar tonsils in to the foramen magnum. Tracheostomy was performed while patient still on mechanical ventilation. Treatment with medroxyprogesterone, steroids and acetazolamide was started. He was discharges after over a month of treatment breathing environmental air with no apparent episodes of apnea. Patient later returned to emergency department with hospital acquired pneumonia mutliple times and succumbed to septicaemia and septic shock later within 6 months. Conclusion: Ondine's curse is a rare form of central respiratory failure which includes severe loss of automatic breathing. Generally associated with Stroke of posterior circulation,in few instances in the past it has been associated with Chiari Malformation as well. Such a syndrome has rarely been reported in adults and the diagnostic criteria are not consensual in the reviewed literature. Thus any diagnostic confirmation should be flexible. There are many therapeutic options in such cases, ranging from pharmacological approach, use of bilevel positive airway pressure and implantation of diaphragmatic pacemaker.

MiP29/270: Essential thrombocythemia presenting as chronic cerebral venous sinus thrombosis and intracranial hypertension: A case report

Swapan Gupta, Shukla T, Khwaja GA

Department of Neurology, GB Pant Institute of Post Graduate Medical Education and Research, New Delhi, India

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

MiP30/334: A case report of neurofibromatosis with facial dysmorphism and mental retardation

Murugan P, Manivannan MR, Pavalam B, Subramani R

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

Introduction: Neurofibramatosis with facial dysmorphism and mental retardation represents a rare form of NF1. It is attributed to germline and mosaic 17q11 microdeletion of NF1 gene and its flanking regions caused by non-allelic homologous recombination. Case Report: We report a case of 20 year old male who presented with generalised multiple nodular swellings all over the body for the past 6 months. The nodules were increasing in size and number and werenon tender. On examination, the patient was conscious and oriented with an IQ of 59, suggestive of mild mental retardation. General examination revealed short stature, facial coarsening, prominent forehead, downslanting palpebral fissures, hypertelorism, broad nose, broad nasal bridge, low set ears, low hair line, micrognathia, webbed neck, maloccluded teeth, high arched palate, clinodactyly. Dermatological examination revealed multiple cutaneous neurofibromas, multiple freckles and café au lait spotsand a plexiform neurofibroma in left forearm. Slit lamp examination revealed lisch nodules in iris. The examination of other systems were unremarkable. MRI was deferred due to a metallic implant in the left mandible as details of the nature of implant were unavailable. Genetic testing was not done. The patient was given supportive physiotherapy and advised close follow-up. Conclusion: The plethora of signs of facial dysmorphism is a rare feature in NF1. Affected individuals have a 50% risk of transmitting the microdeletion, and prenatal and preimplantation genetic diagnosis is possible.

MiP31/341: Nine diseases affecting dentate nucleus

Sateesh Gudla, Vengamma B, Prasad SVN, Rao AA

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

Introduction: The dentate nucleus is a cerebellar structure involved in voluntary motor function and cognition. Dentate nuclear hyperintensities are rarely encountered; the formulation of a differential diagnosis can be difficult. Details: Case 1: 54/male, CKD on PD, recently diagnosed to have pulmonary tuberculosis. He was initiated on 4 drug regimen ATT with HRZ and levofloxacin. MRI brain showed bilateral dentate nuclear hyperintensities. Isoniazid was stopped and improved. Case 2: 28/male with a past history of haemorrhoids was prescribed Ornidazole for 3 months. MRI of brain showed symmetrical T2/FLAIR hyperintensities involving the dentate nuclei. Ornidazole was stopped and improved. Case 3: 39/female presented with complaints of fever with altered sensorium 15 days ago and recovered. MRI brain revealed T2 hyperintensities in bilateral cerebral peduncles, dentate nuclei, pons and midbrain. We considered possibility of ADEM. Case 4: 47/female, mentally retarded with bilateral juvenile cataracts, chronic diarrhoea with progressive neurological illness with predominant cerebellar ataxia with sibling of the similar phenotype (CASE5) and were diagnosed as Cerebrotendinous Xanthomatosis. Both cases had dentate nucleus hyperintensities. Case 6: 5/female with had incidentally dentate nucleus hyperintensities and clinically diagnosed as Neurofibromatosis. Case 7 and 8: 53/male, 60/female with hypoparathyroidism, presented with seizures, hypocalcemia had Fahr's disease their imaging showed bilateral dentate nuclear changes. Case 9: A 2/female with Krabbe's disease had dentate nuclear hyperintensities. Conclusion: Acute cerebellar syndrome is a common clinical presentation associated with dentate nuclear hyperintensities. Most cases of dentate nuclear hyperintensities are potentially treatable and reversible with early intervention.

MiP32/349: Extrapontine myelinolysis with locked-in syndrome: Case report

Mohammed Shujauzzaman Bilal, Manorenj S, Jawalkar S

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

58 year old male came with the chief complaints of Fever with drowsiness. Found to have Total leukocyte count 31000 with neutrophilia. Diagnosed as Sepsis with urinary tract infection. Further investigations revealed Hyponatremia with serum sodium of 97meq/l. Over the next two days it was corrected to 108meq/l and over next 24 hours to 124meq. Two days later it was 142meq. Initially recovering, on day 5 of admission he became progressively drowsy and was found to be unresponsive later in the day. He was intubated in view of depressed sensorium and inability to protect airway.

MiP33/365: A child with cholinergic crisis

Mahesh Narayanan

Department of Neurology, Apollo Children's Hospitals, Chennai, Tamil Nadu, India

A 3 year old child presented with h/o bilateral asymmetrical partial ptosis Child was diagnosed as Myasthenia gravis Showed good improvement following oral neostigmine Presented with excessive salivation, diarrhoea and worsening of weakness with pinpoint pupils and respiratory distress following recent increase in dose of neostigmine. Admitted in intensive care unit and intubated neostigmine was withdrawn and was given IV Ig. Shown good improvement subsequently neostigmine was introduced at a lower dose. Any child presenting with crisis in myasthenia, better to withdraw neostigmine and observed so that rare presentation of cholinergic crisis may not be overlooked.

MiP34/369: An intersting case reports of Fahr's syndrome

Jaison Ambrose F, Karunakaran V, Saravanan S

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

Fahr syndrome also known as fahr's disease or idiopathic basal ganglia calcification is a rare clinical disease whose most common cause is primary or postoperative is characterised by bilateral and symmetrical intracerebral calcifications. A 31 year old male admitted with first episode generalised tonic clonic convulsion lasting for 10 minutes without any family history of seizure. On examination he was neurologically normal. His CT Brain showed bilateral basal ganglia and intracerebral calcifications. His paratharmone seems to be very low. S. calcium level was 4mg/dl. He was treated with high dose of vitamin D and high dose of calcium supplements. His family members were also screened for hypoparathyroidisim it seems to be normal. Another case of young lady underwent total thyroidectomy 18 years back. She subsequently developed chronic symptomatic hypocalcaemia for which she was managed with calcium supplements. She presented to us with subacute onset of generalised, random, jerk like, quasi-purposive movements. On examination she had chorea and cerebellar ataxia. Chovstek sign was positive. Blood investigations showed severe hypocalcemia, along with hyperphospatemia and markedly low serum PTH. CT brain revealed extensive calcifications involving bilateral basal ganglia and cerebellar hemispheres. She was started on high dose vitamin D and her calcium dose was increased. Basal ganglia and cerebellar calcification can occur as a rare complication of chronic hypocalcemia,like our cases is primary or secondary hypoparathyroidism . It can be prevented by assiduous correction of hypocalcemia and hyperphosphatemia.

MiP35/388: WASP: A killer of heart and mind

Karthik Thamarai Kannan, Ramasamy B, Perumal S, Madhavi K, Vallippalam JJ

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

A 40 years old male with no comorbidity and no history of substance abuse, had history of multiple wasp sting over his forehead, arm and neck while traveling in a bike. He developed urticaria, after 6 hours he developed 2 episodes of GTCS and presented to EMD. On examination he was drowsy, hypotensive and had tachycardia. Neurological examination showed left hemiplegia. Electrocardiogram showed ST depression and T wave inversion in leads I, aVL, V2-V6. Patient underwent MRI brain, which showed acute infarct in right MCA territory, involving frontotemporoparietal regions, corona radiata and capsuloganglionic region. MRA showed nonvisualization of M1, M2 segments of right MCA. Carotid Vertebral Doppler was also normal. In view of ECG findings, trop T was done, which was grossly elevated (4668pg/ml). Echocardiogram showed global hypokinesia with EF of 40% without an apical clot. Blood investigations were done for evaluation of young stroke (ANA, APLA, ANCA, homocysteine) and was found to be normal. Follow up CT showed large MCA stroke causing mass effect and midline shift of 8 mm, patient was intubated and referred for decompressive craniotomy. In this patient, we discuss the occurrence of Acute coronary syndrome accompanying cerebrovascular accident following wasp sting, presenting as symptomatic seizures. It was first described by Kounis and Zavras in 1991 and was referred to as allergic angina or allergic myocardial infarction. Pathophysiology of this syndrome, could be due to release of cytokines and mast cell activation causing vasospasm.

MiP36/391: A rare case of unilateral isolated superior ophthalmic vein thrombosis

Tanu Arora, Sowmini PR, Sakthivelayutham S, Jeyaraj KM, Mugundhan K, Arunan S

Department of Neurology, Government Stanley Medical College, Chennai, Tamil Nadu, India

Superior ophthalmic vein thrombosis is a rare disease entity, predisposed by sino-orbital disease, vascular and coagulation anomalies. It usually presents with painful proptosis, chemosis and ophthalmoplegia. We present a case of superior ophthalmic vein thrombosis in a diabetic patient with a history of head trauma. A 66 years old gentleman, diabetic with reasonable glycaemic control presented with ptosis and reduced vision in right eye. He had history of head trauma, one month before the onset of present complaints, but was asymptomatic after that. Then, he started complaining of headache mainly in right frontal and periorbital region of moderate intensity, continuous type, associated with drooping of right eye for 10 days. He was having visual acuity of only light perception with no colour vision in right eye. Left eye and bilateral fundus examination were normal. All extraocular movements were restricted in right eye, pupillary response could not be assessed due to post operative changes of cataract surgery in both eyes. Sensations (all modalities) were decreased in right frontal and maxillary regions. He underwent all routine investigations and brain imaging. MRI Brain (orbital cuts) showed T2/Flair hyperintensity in right superior ophthalmic vein along with perioptic inflammation, suggestive of isolated right superior ophthalmic vein thrombosis. He underwent chest and abdomen imaging also to look for malignancy, but were normal only. He responded well to intravenous antibiotics, anticoagulants and steroids. This is a rare case of unilateral isolated superior ophthalmic vein thrombosis, which occured probably due to orbital inflammation induced by trauma.

MiP37/396: AN interesting case of complicated hereditary spastic paraplegia

Manivannan MR, Vasagam LPLN, Prashanth S, Kannan V

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

42 year old female patient came with complaints of weakness of Right lower limb followed by left lower limb weakness over a period of 8 months. There was limb flaccidity without wasting. There were history of episodic nocturnal leg cramps. Patient had a past history of Pulmonary Kochs (Sputum positive). She took Antituberculous drugs (Isoniazid, Rifampicin, Pyrazinamide, Ethambutol) for a period of 6 momths. She underwent Right pneumonectomy in view of post Tuberculosis sequelae, Bronchiectasis and persisitent Hemoptysis. Physical examination showed Lower limb weakness in corticospinal distribution, proximal more than distal. Deep Tendon Reflexes were brisk with Flexor Plantar response, reduced tone, without cerebellar or extrapyramidal signs. Spine and Cranium were normal. Patient was investigated. MRI SPINE showed a normal study. Complete Blood count, Renal function test, Liver function test were within normal limits. Nerve Conduction Study was normal. X ray Chest showed post pneumonectomy status. USG Abdomen was normal. HSP (or familial spastic paraparesis) is a genetically and clinically heterogeneous group of disorders rather than a single entity. Although most cases present in the second to fourth decades, onset is from infancy into the eighth decade. The clinical syndrome is broadly divisible into the pure form and the complicated form. In the pure form, patients develop only lower-extremity spasticity, but some of these cases eventually become complicated. However, the complicated form may also include optic neuropathy, pigmentary retinopathy, deafness, ataxia, ichthyosis, amyotrophy, peripheral neuropathy, dementia, autoimmune hemolytic anemia/thrombocytopenia (Evans syndrome), extrapyramidal dysfunction, intellectual disability, and bladder dysfunction.

MiP38/397: Severe 5, 10? methylenetetrahydrofolate reductase deficiency: A rare, treatable cause of complicated hereditary spastic paraplegia

Dhaval Dave, Khan F, Rohatgi S, Nirhale S, Rao P, Naphade P

Department of Neurology, Dr. D. Y. Patil Medical College, Hospital and Research Center, Pune, Maharashtra, India

Background and Purpose: Juvenile? or adult? onset forms of severe 5, 10? methylenetetrahydrofolate reductase (MTHFR) deficiency manifesting as complicated hereditary spastic paraplegia have rarely been described. Methods: 19 year old male with epilepsy and young stroke developed a progressive spastic paraparesis in his mid teen. His diagnostic assessment included extensive neurophysiologic, neuroimaging and metabolic studies. Results: Brain magnetic resonance imaging showed periventricular and posterior white matter alterations and centrum semiovale infarct, and electromyography documented a mild axonal polyneuropathy. Severe hyperhomocisteinemia (>400 μmol/L) associated with the characteristic amino acid profile that is low methionine levels suggested a diagnosis of severe MTHFR deficiency, confirmed by MTHFR direct sequencing. Treatment with betaine and vitamins benifitted patient. Conclusions: Severe MTHFR deficiency can be a rare, treatable cause of autosomal recessive complicated hereditary spastic paraplegia. Its screening should be part of the diagnostic flowchart for these disorders.

MiP40/413: Metronidazole induced acute cerebellar syndrome: Report of a rare case

Bhaskar Shukla, Uniyal R

Department of Neurology, King George's Medical University, Lucknow, Uttar Pradesh, India

Introduction: Neurological toxicity in the form of peripheral neuropathy is fairly common with metronidazole. However, only a few cases of central nervous toxicity in the form of isolated cerebellar involvement have been reported. Case History: We present here a case of a 37-year chronic alcoholic male and a diagnosed case of right lobe liver abscess who presented to neurology OPD with gait instability, dysarthria and involuntary, jerky eye movements of 5 days duration. For liver abscess, the patient had been taking metronidazole 400mg BD for 1 month. On general examination there was right upper quadrant tenderness. On neurological examination there was gaze evoked nystagmus, impaired finger nose test, dysdiadochokinesia, wide based gait with impaired tandem walking, suggestive of a pan cerebellar syndrome. Nerve conduction study was normal. MRI brain revealed bilaterally symmetric T2/FLAIR and DWI hyper intensity in dentate and facial nuclei. The patient's symptoms improved dramatically following discontinuation of the medication. Conclusion: The characteristic MR imaging findings of bilateral increased T2/FLAIR signal of the dentate nuclei should alert the clinician to enquire regarding metronidazole usage. With a higher index of suspicion, many more such cases are likely to be diagnosed in future.

MiP41/428: Gall bladder carcinoma presenting with ophthalmolplegia, hearing loss and areflexic paraparesis: A rare case

Haramohan Sahoo, Imran Rizvi

Department of Neurology, King George's Medical University, Lucknow, Uttar Pradesh, India

Introduction: Gall bladder carcinoma usually presents with abdominal pain, weight loss, fever, jaundice. Here we present a case of gall bladder carcinoma which presented with ophthalmoplegia, hearing loss and areflexic paraparesis. Case: A 60 year female presented with fever for 3 months, headache followed by double vision for 2 months, progressive deterioration of vision, hearing loss and gradually progressive difficulty of walking for 1month. Patient was diagnosed outside as tuberculous meningitis and was started on ATT but patient did not improve. O/E GCS:15/15, vitals stable, vision: hand movement positive, pupils: dilated and sluggishly reactive to light, CN examination: bilateral third, fourth, sixth, seventh and eighth cranial nerve palsies. Power in the upper limbs was 5/5 and in lower limbs was 3/5. There was generalised areflexia with flexor plantars and normal sensory system. Neck rigidity was present. There were multiple skin nodules over chest. Relevant investigation findings: CSF: lymphocytosis with malignant cells, USG abdomen: gall bladder mass, CECT abdomen: gall bladder mass infiltrating into liver parenchyma, MRI brain: thickening and enhancement of CNIII, IV, V, VI, VII, VIII roots, MRI spine: features of arachanoiditis, biopsy from skin nodules over chest: malignant cells on HPE. Patient was diagnosed as gall bladder carcinoma with metastatic involvement of cranial nerves and meninges of brain and spinal cord and was started on chemotherapy. Patient gradually improved. Conclusion: Cranial nerve palsies and spinal cord involvement can be a presentation of solid tumours. Early diagnosis and timely management can save life of a patient.

MiP42/429: A neurological facade of Niemann's pick disease

Umesh Bonala, Pai A, Gorthi SP, Prabhu A

Department of Neurology, Manipal University, Jaipur, Rajasthan, India

Niemann-pick disease type c (NPC) is a rare neurovisceral lipid storage disorder resulting from autosomal recessively inherited loss-of-function mutations in either NPC1 or NPC2. As npc is heterogeneous in presentation and rare, it is often misdiagnosed as other movement or psychiatric disorders, highlighting the need for better awareness of this disease among clinicians. We describe a adult-onset case of npc. A 45 year old lady born of second degree consanguineous marriage presented with ataxia with slurred speech of 7 years duration. On examination there was supranuclear vertical gaze palsy with slow saccades with mixed dysarthria (cerebellar and spastic) and cerebellar ataxia. Abdominal examination revealed splenomegaly. Her investigations for complete blood picture, renal and liver function tests were normal. Viral markers were negative. Mri brain was done was normal. In view of supranuclear gaze palsy, ataxia and splenomegaly, strong clinical suspicion of lysosomal storage disorder was made. Bone marrow biopsy showed lipid laden foamy histiocytes suggestive of niemann- picks disease. Early bonemarrow/genetic testing for np-c should be considered in patients suffering from atypical neurological/neuropsychological symptoms, even in cases of uncertainty. Early diagnosis and improving the knowledge of the disease among neurologists appears essential since emerging treatments like disease-specific drug miglustat recent has shown positive outcomes before the occurrence of widespread deep brain neurological lesions.

MiP43/432: Lindane induced toxicity

Subhangi Thakur Hameer, Khurana D, Ray S, Karthik, Heena

Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Background: There are numerous toxins which can affect the nervous system. Scabies is a known problem in developing countries and the use of Gamma benzene hexachloride (Lindane) is prevelant in these countries. Several case reports of lindane induced toxicity of the nervous system in animals have been reported. Materials and Methods: We describe a, 27 yr old male with skin lesions diagnosed as scabies who applied lindane twice daily for 15 days from the foot to the waist including the scrotum. Within a month he developed spastic paraparesis of bilateral lower limbs with loss of all the sensory modalities below the waist. CSF analysis was done which was normal. MRI brain was essentially normal. Vitamin B12 levels were slightly low. Nerve conduction study was done which was normal. His work up for other causes of myeloneuropathy was normal. A diagnosis of Lindane induced myelopathy was made. Discussion: Anti-scabietic agents frequently contain 1% solution of the pesticide Gamma Benzene Hexachloride i.e Lindane. The toxicity of the compound depends on the concentration and the route of administration. Overzealous application of this anti-scabietic agent can result in serious complications such as Myelopathy and it has to be ruled out as a cause in patients using this agent for various purposes. Conclusion: Dermatologists and neurologists should be aware of lindane as a cause of myelopathy and should be cautious while prescribing this agent.

MiP44/435: Characteristic MRI findings in genetically proven hereditary spastic paraplegia

Neelam Patil, Chakor R, Bharote H, Endole D

Department of Neurology, Topiwala National Medical College and BYL Nair Hospital, Mumbai, Maharashtra, India

Autosomal recessive Hereditary spastic paraplegia (HSP) characterized by autosomal recessive inheritance, slowly progressive spastic paraparesis and mental impairment, thinning of corpus callosum, cervicodorsal spinal cord atrophy revealed by CT/MRI and exclusion of other disorders by laboratory tests and MRI of spine and brain. Hereby presenting case series of three patients of HSP proven genetically with characteristic MRI findings. Patient 1 presented with spastic paraplegia with cognitive impairment and rigidity in left UL, MRI s/o thin corpus callosum, white matter abnormalities and dorsal spinal cord atrophy, diagnosed to have pathogenic variant mutation in SPG11 gene. Patient 2 presented with spastic paraplegia with bladder involvement, cognitive impairment and positive family history, MRI s/o thinning of corpus callosum diagnosed to have novel pathogenic variant mutation in SPG 11 gene. Patient 3 presented with spastic paraplegia with impaired joint position sense, positive family history, MRI s/o cervicodorsal spinal cord atrophy. In all three patients other causes of spastic paraplegia ruled out. Conclusion: Patient presenting with spastic paraplegia with thinning of corpus callosum, spinal cord atrophy helps to improve genetic diagnosis of autosomal recessive hereditary spastic paraplegia.

MiP45/436: A rare cause for sub acutely evolving hemiparesis in an IV mainliner

Githin Benoy George, Iype T, Paniker P, Devi SKL, Nandakumar G, Pai B, Rajeev R

Department of Neurology, Government Medical College, Thiruvananthapuram, Kerala, India

A 25-year-old male, an Intravenous mainliner presented with fever 20 days, and progressive left hemiparesis 15 days. MRI brain showed rounded 1.2 x 1.3-centimetre T2/FLAIR hypointense lesion, iso to hyperintense on T1, a small focus of central diffusion restriction, no blooming, with lactate peak and choline NAA ratio 1.2 on MRS in the right parietal cortex showing peripheral enhancement and perilesional oedema: squashing the right lateral ventricle with midline shift of 3 mm. His weakness initially improved on anti-tuberculous medication with steroids, followed by drop in GCS to E2V4M5 headache and dysarthria on tapering steroids. The largest lesion on repeat MRI brain showed T2/FLAIR hyperintense lesion with hypointense rim, T1 hypointense lesion with a hyperintense rim with Papillary projections, rim enhancement with peripheral diffusion restriction and peripheral blooming in SWI. The midline shift increased to 6mm. MRS showed Lactate peak with choline NAA ratio 1.8 and a suspicious Trehalose peak. Haematoxylin and eosin, PAS and Gomory silver stained brain biopsy showed fungal hyphae and spores, with surrounding histiocytes, foreign body giant cells and lymphocytic infiltrates. Lactophenol cotton blue preparation, KOH mount and Gram stain revealed numerous septate and darkly pigmented hyphae. Culture on SDA yielded velvety colonies, olive-grey to black in colour in five days. The Neurotrophism, morphology of brown septate hyphae with short conidiophores bearing sparsely branched oval conidia with the absence of darkly pigmented hila, growth at 430 C and lack of gelatinase activity confirmed the diagnosis of Cladophialophora bantiana.

MiP46/437: Peripheral T cell lymphoma presenting with isolated CNS manifestations

Prashant Kumar

Department of Neurology, MMI Narayana Multispeciality Hospital, Raipur, Chhattisgarh, India

Peripheral T cell lymphoma (PTCL) itself is a rare disease which manifest with systemic symptoms with nodal involvement. Skin and gastrointestinal tracts are most common extra nodal sites involved. It's manifestation with isolated central nervous system involvement without characteristic systemic symptom is very rare. We are reporting such a case here. A 66 year old man presented with 6 months history of progressive cognitive decline. As per history symptoms used to improve partially with steroids. He was drowsy but arousable, disoriented with global cognitive dysfunction. Sensory motor examination and other systemic examination were non contributory. Routine investigations were normal except mild anemia. MRI brain showed meningeal enhancement with exudates in bilateral frontoparietal sulci and interpeduncular cistern. PET CT was non contributory. CSF analysis showed 45 cells with lymphocytic predominance, raised protein and normal sugar level. CSF flow cytometry showed T cell neoplasm. His bone marrow examination showed atypical lymphoid cells. He was treated with intra-thecal chemotherapy with good initial response. In conclusion PTCL may present with isolated CNS involvement.

MiP47/461: Health a matter of chance or choice

Kiran Bala

Department of Neurology, GB Pant Institute of Post Graduate Medical Education and Research, New Delhi, India

Introduction: Biomedical Science that explores the relationship between emotions, nervous system, and immune system reveals that there is a close link between our states of mind and our states of health. Obsevations: Mind is the software working through TEAM ie Thoughts, Emotions, Attitude and Memories Biological clock/circadian rhythms control the internal milieu of body, hence influences our state of health. Discussion: Psycho-Neuro-Immunology shows close link between Mind (Software) and Brain (Hardware). Power/Magic of Water: Essential for life, Our thoughts and emotions affect the structure of water!, Music has impact! Even naming has impact!!, Remaining close to nature and natural syatems keeps the biological clock in order. Conclusion: Awareness, thoughts affect biological processes…and hence health, Thoughts and feelings affect water, Watch the water you drink, food you eat, Biological purity is not the only thing that counts. I am both 'in'-'charge' and 'response''able' for my health. Health like Destiny is a matter of CHOICE and not a matter of Chance.

MiP48/472: Idiopathic intracranial study: A 3 months longitudinal follow up study

Abhishek Pathak, Chaurasia RN, Joshi D

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

The purpose of this study was to evaluate the clinical presentation and monitor a 3-month course using frequent optical coherence tomography (OCT) evaluations, visual field testings and lumbar opening pressure measurements. A longitudinal study of 14 patients with newly diagnosed IIH and 20 healthy overweight controls were included. Peripapillary retinal nerve fiber layer thickness (RNFLT) and retinal thickness (RT) measurements and Humphrey visual field testing were evaluated at regular intervals. The diagnostic delay was 4.5 months and initial symptoms were headache (100%), visual blurring (90%) and pulsatile tinnitus (15%). Mean CSF pressure was 25.6 cm of CSF. Complete clinical remission was achieved in 71.4%, partial in rest. Total average RNFLT and RT decreased significantly during the follow-up period (p < 0.001 and p < 0.001, respectively). Changes in RNFLT and RT correlated with improvements in visual field mean deviation (MD). OCT with visual field testing may be a valuable objective tool to monitor IIH patients.

MiP49/486: Use of high dose oral baclofen in management of spasticity due to myelopathy

Kamal Kumar Jain

Department of Neurology, Moolchand Neuro Sciences Center, Hyderabad, Telangana, India

Baclofen remains the drug of choice for management of spasticity in cases of CNS demyelination and Myelopathy due to various causes. The recommended dose for management of spasticity is 20-120 mg/day orally. Higher doses of up to 300 mg/day have been used in cases of Alcohol Dependency. Two cases of Grade 3 Spasticty due to Myelopathy were treated with oral Baclofen 200 mg/day. Following treatment, spasticity improved to Grade 1 without any side effects. Therefore, high doses of oral Baclofen is a cost effective therapy and should be tried in cases of severe spasticity due to myelopathy before subjecting them to invasive procedures such as Baclofen pumps.

MiP50/487: Reversible parkinsonism in osmotic demyelination syndrome

Kamala Kant Bhoi, Kumar S, Pandit A, Jaiswal D

Department of Neurology, Shri Balaji Institute of Medical Science Pvt. Ltd., Raipur, Chhattisgarh, India

Parkinsonism with or without dystonia has been rarely described following central pontine myelinolysis and extrapontine myelinolysis. We report 5 cases of reversible parkinsonism and dystonia with imaging evidences of central pontine myelinolysis and extrapontine myelinolysis associated with hyponatremia from a tertiary center in India. Their presentations varied from mild masked facies to extra pyramidal syndromes characterized by progressive supranuclear palsy like feature and marked dystonia. Two cases presented with flaccid quadriplegia later evolved into spasticity and dystonia. The cause of hyponatremia was due to vomiting in two, diuretic-induced, nutritional and psychogenic polydipsia one each. The onset was acute in 4, and gradual in one from psychogenic polydipsia. They responded well to gradual correction of electrolyte imbalance, dopaminergic and antidystonic agents including botulinum toxin. The movement disorders of central pontine myelinolysis with extrapontine myelinolysis represent a treatable manifestation of the osmotic demyelination syndrome and rewarding result can be achieved.

MiP51/490: Sub acute cerebellar ataxia: A wobbly step towards the diagnosis

Bhargeshkumar Patel, Sowani A, Dave N, Kotadia T, Gajjar B

Department of Neurology, Zydus Hospital, Ahmedabad, Gujarat, India

A 72-year-old, right-handed woman presented on 1st may, 2019 with complaints of acute-onset rapidly progressive imbalance while walking and change in voice since last 1 month. She had history of weight loss (5 kg) in last 1 month. No history of visual involvement or difficulty in swallowing or hearing loss. No history of limb weakness or numbness. No history of fever, altered sensorium or convulsions. No history of drug or substance abuse. Her medical and surgical histories were non-contributory. On examination she was conscious, HF normal, mild dysarthria, torsional nystagmus present, FNT bilateral positive, marked truncal ataxia present along with spastic ataxic gait and extensor planters. The Physical examination was unremarkable, including a negative breast examination. Routine blood tests and chest x ray were within normal limits. MRI brain showed cerebral and cerebellar atrophic changes. USG abdomen showed enlarged lt ovary with heterogeneous echo pattern and few small cystic component. CT abdomen showed heterogeneous enhancing lesion in left adnexa not separated from left ovary, p/o ovarian carcinoma likely. Serum Paraneoplastic Antibody panel was positive for anti YO antibody. Patient underwent total laparoscopic Hysterectomy with bilateral salpingo oophorectomy with left sided mass removal (9/5/19). Histopathology report s/o High grade serous ovarian carcinoma. Our patient fulfilled the diagnostic criteria of PCD based on international guidelines. Patient was treated with iv methyl prednisolone (1 gm. /day) for 5 days. Patient's clinical condition deteriorate and due to carcinoma related complications succumbed after month (14/6/19).

MiP52/506: An interesting case of reversible cortical blindness

Vinit Banga, Mahajan A, Chatterjee A, Goel G

Department of Neurointervention, Institute of Neuroscience, Medanta - The Medicity, Gurugram, New Delhi, India

Objectives: An Interesting case of reversible cortical blindness. Background: Cortical Blindness also known as Anton Syndrome is a dreaded and potentially irreversible complication of posterior circulation stroke. Methods: Clinical History, Neurological Examination, Neuroimaging and Follow up. 71 year female with altered sensorium along with cortical blindness. Results: A 71 year female presented to hospital with acute coronary syndrome and was diagnosed to have single vessel disease and undergone stenting to LAD for the same which was uneventful. On second day of surgery, she developed acute onset cortical blindness which further progressed to confusion followed by altered sensorium over hours. On examination she was drowsy and was confirmed to have cortical blindness. Urgent neuroimaging in the form of NCCT head, CT perfusion and CT Angiography of neck and brain vessels revealed mismatched perfusion deficit involving bilateral thalamus, vermis and bilateral cerebellar hemispheres along with severe basilar artery stenosis. Her MRI Brain showed scattered area of diffusion restriction in watershed zone. She underwent urgent basilar artery stenting and recovered completely during hospital stay with no residual deficit and has been doing well over one year follow up. Conclusion: Patient with atherosclerotic disease involving vertebrobasilar circulation can present with posterior circulation stroke with symptoms such as cortical blindness along with other manifestations which can be life threatening and disabling. Cortical blindness, recovering after treatment has not been described in literature. Our patient had improvement in her symptoms after basilar artery stenting. The importance of timely recognition and treatment of posterior circulation stroke is to be emphasised to avoid serious outcomes.


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