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. 2015 Sep;18(Suppl 2):S18–S40.

Selected Abstracts of IANCON 2015

PMCID: PMC4784112
Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

Platform Session 01, Friday, October 02, 2015, 8: 30 AM- 09:30 AM Dr. Baldev Singh Hall (Hall A): Stroke: SO1/4: Contrast Staining Areas (CSA) on CT following Mechanical Intra-Arterial Thrombectomy in Acute Ischemic Stroke: Pathophysiology and Clinical Implications

Rahul Kumar

G S Neuroscience Clinic and Research Centre, Patna, Bihar, India

Introduction: Mechanical thrombectomy for ischemic stroke is the most effective technique to achieve recanalization. As with every reperfusion technique the risk of hemorrhage undermines the benefits from recanalization to some extent. Due to speed widespread availability and ease of interpretation CT scan is the method of choice to study post reperfusion hemorrhage.

Materials and Methods: Over a period of 6 months 11 patients suffering from anterior circulation large artery occlusion underwent mechanical thrombectomy. According to the protocol at our institution all the patients underwent CT scanning immediately post procedure 24 hours post procedure and 5 days post procedure and in between if the clinical status so necessitated. All patients underwent thrombectomy using the Solitaire Device.

Results: TICI2b or TICI3 flow was obtained in all the 11 patients. 4 patients developed hemorrhagic transformation of the infarcted region and decompressive surgery had to be performed in 2 patients. Age risk factors for stroke time of reperfusion and use of GPIIb/IIIa inhibitors were not significantly different among the 2 groups. 10 out of 11 patients demonstrated CSA's on the post procedure scans. The CSA's were of various patterns but none of the patterns demonstrated consistent association with hemorrhage.

Conclusions: CSA's are common after successful reperfusion. They do not reliably predict the risk of future hemorrhagic transformation. They are possibly secondary to the blood brain barrier becoming more permeable due to the ischemia induced foot process retraction of the Astrocytes. They most often resolve spontaneously between 24 to 48 hours post procedure.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

SO2/213: To Study the Effects of Opioids on the Outcome of Stroke

Monika Singla, Gagandeep Singh, ShivaniJuneja, Sandeep Kaushal, Kanchan Gupta

DayayanandMedical College & Hospital, Ludhiana, Punjab, India

Objectives: To evaluate the effect of self administration of opioids on the outcome of stroke.

Methods: A prospective observational study conducted on patients with stroke admitted in the Neurology ward of DMCH Ludhiana from Jan-June 2012. The data collected was recorded in a semi-structured proforma. The severity of the stroke was evaluated using NIHSS scale and outcome using MRS scale. The data was analysed using descriptive statistics and unpaired t-test.

Results: Out of 380 patients who presented with stroke 75 patients fulfilled all the inclusion criteria and consented to participate in the study. The patients were divided in two groups those who gave history of self administration of opioids (30) at the onset of stroke and those who did not take opioids (45). The average age of presentation was 57±15.9 years in non-opioid and 64±9.6 years in opioid group with male preponderance of 83.33% and 57.69% respectively. Most of the patients belong to lower middle class 54% (opioid) and 38% (non-opioid). The rural inhabitation is 54% in patients in opioid group and 27% in non-opioid group. The mean duration of stay of stroke patients was 8±9.8 days in opioid group and 7±6.3 days in non-opioid group.

Conclusions: The domiciliary practice of opioids administration is more in rural areas but has no effect on the outcome of stroke. The difference in the outcome (MRS) and severity (NIHSS) of stroke in both the groups was statistically insignificant (p=0.283 p=0.987 respectively). Thus a larger sample size would be required to substantiate our findings.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

SO3/149: Clinical Profile of Patients with Anterior Ischemic Optic Neuropathy (AION) Presenting To a Tertiary Care Centre in India

Anuja Patil, Vivek Lal, Amod Gupta, Paramjeet

PGIMER, Chandigarh, India

Introduction: Ischemic optic neuropathy is one of the major causes of blindness or seriously impaired vision. AION is the most common cause of acute optic neuropathy in older age groups. Non-arteritic AION is the most common form arteritic form being associated with giant cell arteritis. Indian data regarding this entity is still sparse despite the rising prevalance of medical comorbidities in India matching the western trends.

Objective: To assess the clinical profile of patients presenting with AION in a tertiary care hospital.

Materials and Methods: In an observational study all patients presenting with sudden loss of vision were evaluated. Inclusion criteria were disc edema or sectoral pallor on fundus examination consistent field defect on perimetry and filling defect on fundus fluorescein angiography. All patients underwent routine biochemical investigations along with a detailed ophthalmic evaluation.

Results: 12 patients have been enrolled in the study till now all with NA-AION. 7 of these were females and 5 males. 4(33.3%) had sequential bilateral involvement. 5 (41.6%) had diabetes 6 (50%) hypertension and 8 (66.6%) had BMI >30kg/m2. Of the 16 eyes with AION almost half had normal visual acuity at presentation.

Discussion and Conclusions: Non-arteritic form was the commonest cause for AION. Diabetes hypertension and obesity were among the common risk factors associated. Patients presented with normal visual acuity despite visual field defect. Final results will be analysed and revealed in IAN.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

SO4/28: Comparison of Clinico-Radiological Profile of Posterior Circulation Strokes with anterior circulation stroke - Study from a Tertiary Care Centre in Eastern India

Bhushan Joshi, BhushanJoshi, B.K. Roy, D.S. Guin

Bangur Institue of Neurosciences, Kolkata, West Bengal, India

Results: Of the total 100 cases of posterior circulation stroke 64 (64%) were males (mean age-48.7yrs) while 36 (36%) were females (mean age 44.4yrs); 68(68%) were ischemic while 32 (32%) were hemorrhagic. Among ischemic strokes30% had TIAs (18% had only posterior circulation TIAs). Large artery (hemodynamic) (36%) and embolism (36%) (26%cardiembolism 10% artery-to-artery embolism) were the most common stroke mechanisms. Distal posterior circulation territory infarcts (44%) were much common than the proximal(26%) or middle territory(30%) infarcts. For hemorrhagic strokes uncontrolled hypertension was the commonest mechanism while cerebellum and thalamus were the commonest sites. Commonest predisposing factors for ischemic stroke were tobacco addiction followed by hypertension while for hemorrhagic strokes it was hypertension. Out of 100 14 died(14% mortality) 10 of hemorrhagic strokes and 04 of ischemic stroke commonest causes being septic and metabolic complications of the total 100 cases of Anterior circulation stroke 70 (70%) were males (mean age - 49.7yrs) while 30 (30%) were females (mean age - 50.4yrs); 58 (58%) were ischemic while 42 (42%) were hemorrhagic. Among ischemic strokes34% had TIAs. Embolism (60%) (46%cardiembolism 14% artery-to-artery embolism) was the most common stroke mechanism. For hemorrhagic strokes uncontrolled hypertension was the commonest mechanism while Basal ganglia were the commonest sites. Commonest predisposing factors for ischemic stroke were tobacco addiction followed by hypertension while for hemorrhagic strokes it was hypertension. Out of 100 20 died (20% mortality) 16 of hemorrhagic strokes and 04 of ischemic stroke commonest causes being septic and metabolic complications.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

SO5/67: Natural history of Ishemic stroke not treated with IV thrombolysis because of mild or rapidly improving symptoms.

Methil Pradeep, TCR Ramakrishnan

KG Hospital & Post Graduate Medical Institute, Coimbatore, Tamil Nadu, India

Introduction: IV thrombolysis with TPA in the first four & half hours has been the standard of care for the management of acute ischemic stroke. The number of patients with an acute ischemic stroke receiving IV thrombolysis is small. one common reason cited for not treating with TPA is that the deficits are too mild or rapidly improving.

Aim: To investigate the natural history of patients not treated with IV TPA solely because of mild or rapidly improving symptoms and to assess predictors of an unfavourable out come.

Methods: Patients with acute ischemic stroke admitted between march 2009 & December 2014 who were not treated with IV TPA solely because of mild or rapidly improving symptoms were included. The MRS score & NIHSS were taken into consideration at the time of admission & 3 months later. Variables were obtained to evaluate predictors of an unfavourable outcome (MRS 2 to 6).

Results: 66 patients with an acute ischemic stroke did not receive IV TPA because of mild or rapidly improving deficits. There was an unfavourable out come in 30 percent of patients. 10 patients with initial mild or rapidly improving symptoms did receive IV TPA after deterioration in the ward & out of them 6 patients had an unfavourable out come after 3 months. Significant predictors of unfavourable outcome were high initial NIHSS score & unfavourable MRS(2-6) on admission as well as female sex.

Conclusion: The Natural history of patients with ischemic stroke & mild or rapidly improving symptoms is not always favourable.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

SO6/86: Subtypes of ischemic stroke and associated risk factors: a hospital based stroke registry from south India

G. Sivaram Naik, A. Usharani, Shailaja Mekala, SA Jabeen, Suvarna Alladi, Subhash Kaul

NIMS Hyderabad, Hyderabad, Telangana, India

Background: Subtyping ischemic strokes is crucial since it affects prognosis and management. Recent studies from Asia and the West report differences in stroke patterns but limited data exists from India.

Aim: To investigate the pattern of ischemic stroke subtypes and associated risk factors in a stroke registry from south India.

Material and Methods: In this decade-long (2001-2010) prospective study done at Nizam's Institute of Medical Sciences Hyderabad all patients with ischemic stroke were comprehensively evaluated and subtyped as per TOAST classification.

Results: 2174 patients (mean age: 54.1 years) with ischemic stroke were included. Frequency of large-artery atherosclerosis (LAA) (36.4%) was the highest (intracranial: 28.3% extracranial: 8.1%) followed by small vessel occlusion (SVO) (18.9%) and cardioembolism (10.6%). The most common associations observed were hypertension with SVO; diabetes mellitus with LAA and SVO; smoking and hyperlipidemia with LAA; and ischemic heart disease with cardioembolism.

Conclusions: The pattern of ischemic stroke subtype appears to be different from both Asian and Western stroke registries. The frequency of LAA is higher than reported from other countries (14-32%) probably because both intracranial and extracranial atherosclerosis are common. The frequency of SVO was however similar to the west (15-23%) but less than other Asian countries (25-43%). On the contrary lower frequency of cardioembolism was observed compared to west but similar to other Asian countries (10-26%). The unique pattern of stroke subtypes in India can be attributed to differences in race/ethnicity genetics demographic profile and risk factors profile.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

Platform Session 02, Friday, October 02, 2015, 8: 30 AM - 09:30 AM Dr. K. S. Mani Hall (Hall B): Epilepsy: EO1/42: A Study on the Impact of Clinical Depression on Medication Adherence and Seizure Control in Patients with Primary Generalised Epilepsy

Somasundaram Aadhimoolam Chinnadurai, M. Kilji, N. Shanmuga Sundaram, S. Bala Subramanian, K. Bhanu

Madras Medical College, Madras, Tamil Nadu, India

Background: Depression in epilepsy though fairly common receives less attention from clinicians who mainly target seizure control. Depression has a significant impact on medication adherence and can cause poor seizure control.

Objective: The main objective is to investigate the prevalence of depression in patients with generalised epilepsy and its impact on antiepileptic drug (AED) adherence. We also try to uncover if there is any correlation between depression and seizure control.

Methods: 100 consecutive patients with primary generalised epilepsy attending epilepsy clinic were recruited. Patients were arbitrarily divided into two groups depending on their seizure control “good control (<1 seizure per month) and poor control (>1 seizure per month). All patients were administered the Morisky Medication Adherence (MMA) questionnaire. Patients with a MMA score of < 1 were considered non adherent. The presence of depression in all patients was assessed using the Center for Epidemiologic Studies Depression Scale. Patients with a score of > 16 were considered depressed.

Results: Among 100 patients with generalised epilepsy 52% (52/100) were depressed 35% (35/100) were non adherent to AED and 30% (30/100) had poor seizure control. Among patients with depression 48.07% (25/52) were non adherent which was significant when compared to patients who were not depressed 20.83% (10/48) (p<0.0001). Depressed patients were also more likely to have poor seizure control (44.23% 23/52) when compared to patients without depression (14.58% 7/48) (p<0.0001).

Conclusion: The prevalence of depression in patients with generalised epilepsy is high and it significantly impairs medication adherence in these patients. Depressed patients are more likely to have poorer seizure control.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

EO2/58: Carbamazepine Monotherapy in GTCS: An Indian Experience

M Thangaraj, ApurvaGawai, Souat Sarkar, Venkatramana Nagire

Naveen Neuro Clinic, Thanjavur, Tamil Nadu, India

Background: Though carbamazepine is extensively used in the treatment of partial seizures data concerning its effectiveness and tolerability in treatment of children and adults with newly diagnosed generalized tonic-clonic seizures (GTCS) in Indian population is lacking.

Methods: A prospective open-label multicenter non-comparative observational study in 503 Indian patients across 50 centers to assess the change from baseline (BL) in GTCS controls (number of seizures) after 24 weeks treatment with carbamazepine. Percentage of patients reaching >50% and >75% reduction in number of seizures per month after 24 weeks treatment was assessed.

Results: Out of 503 subjects enrolled 493 (98.01%) subjects completed the study. The mean age of study subjects was 26.1years (range 1.5-60 yrs.). The mean± standard deviation (SD) seizure frequency at baseline and week 24 was 7.7± 17.83 and 0.2 ± 1.43 per month respectively. Carbamazepine treatment achieved significant reduction in seizure frequency (mean change from baseline 7.39 ± 18.38; p<0.001). The percentage of patients reaching ≥ 50% reduction and >75% reduction in number of seizures per month was 94.6% and 92.8% respectively. The study reported 88.8% complete responders 5.8% partial responders and 5.4% non-responders. Overall 11 adverse events (AE) were observed in this study the most common being somnolence.

Discussion: In treatment naive GTCS patient's carbamazepine monotherapy was found to be an effective and tolerable treatment option in routine clinical practice in India.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

EO3/256: Pseudotemporal epilepsy – A Case Series

Shaik Afshan Jabeen, SuchandaBhattacharjee, Ajith Cherian, Rukmini Mridula, AK Meens, RupamBorgohain

Associate Professor of Neurology, Hyderabad, India

Aim: To study the presurgical data and correlate with surgical outcome of three patients with medically refractory epilepsy who presented with hypomotorseizures and underwent surgery in our institute from 2012 t0 2014.

Materials and Methods: Presurgical data of three patients who underwent lesionectomy under ECOG guidance for a calcified granuloma at parietotemporooccipital junction and presenting with hypomotor seizures were analysed. All the clinical details Imaging (1.5 Tesla MRI Brain) Video EEG data and other investigations like interictal CT PET Brain were noted.

Results: All three patients had a strategic calcified granuloma located at parietotemporooccipital junction. These patients had significantly shorter duration of epilepsy and high frequency of seizures when compared with other patients with mesial temporal epilepsy. All three were operated based on careful interpretation of video EEG data with MRI and interictal CT PET scan. Two patients with 1 year follow up and one patient with 6 months follow up are seizure free (Engel class I outcome).

Conclusions: Patients with strategic located lesion and pseudo temporal type of presentation can be operated based on neuroimaging and video EEG with a good surgical outcome.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

EO4/92: Clinical Profile and Outcome of Refractory Convulsive Status Epilepticus in Children

Geereddy Ravindar, LokeshLingappa, Ramesh Konanki, Ravi Patel, Sudhindra Vooturi, Sitajayalakshmi

Krishna Institute of Medical Sciences, Hyderabad, Telangana, India

Aim: The current study evaluates the etiology clinical course and short term outcome of refractory convulsive status epilepticus (CSE) in children above two years of age admitted to Neuro intensive care unit from South India.

Methods: Retrospective analysis of data of 73 children with CSE aged 2-12 years was performed. Odds ratios were calculated between variables for clinical course and outcome. Mortality of the group was analysed using survival analysis and life tables.

Results: Thirty three (45.2%) children progressed to refractory status epilepticus (RSE). The most common etiology for CSE was acute symptomatic in 44 (60.3%) of which 37 had CNS infections. The odds of progressing to RSE were higher in children with acute symptomatic etiology (OR 2.62; CI-95%; 0.99-7.14; p=0.041) and those with fever at presentation (OR 4.97; CI-95%; 1.68-14.65; p=0.002). Overall mortality was 13.7% higher in RSE (21.2% vs.7.5%). Amongst the 63 surviving children followed for six months from discharge progression to RSE increased the odds of disability by seven times (OR 7.08; CI-95%; 1.71-29.31; p=0.004).

Interpretation: Acute symptomatic etiology was the commonest cause of CSE in children and increased the odds of progressing to RSE. RSE was significantly associated with disability at six months from discharge. What this paper adds: Clinico-etiological profile of children with refractory convulsive status epilepticus from India. The most common etiology for CSE in children was acute symptomatic. Acute symptomatic etiology increased the odds of progression to RSE.13.7% children died.¢RSE was significantly associated with disability at six months from discharge.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

EO5/102: Evaluation of Headache Disorders in Patients of Idiopathic Epilepsy

Ujjawal, Kundu TN, Roy A, Pandit A

Bangur Institute of Neurosciences, Kolkata, West Bengal, India

Introduction: Epilepsy is a condition characterized by recurrent seizures unprovoked by any immediate identifiable cause. Various types of headache especially migraine is considered a relevant co-morbidity in epilepsy. Headache in idiopathic epilepsy may have diagnostic and therapeutic implications.

Materials and Methods: Herein we report clinical data on 80 patients of Idiopathic epilepsy. All eligible patients of idiopathic epilepsy were recruited from general OPD or epilepsy clinic of Bangur Institute of Neurosciences. Headache was classified according to ICHD III and headache disability before and after treatment was calculated according to Henry Ford Headache Disability Index(HDI).

Results: Out of 80 cases recruited 46(57.50%) had some or the other type of headache. Maximum number of cases had Idiopathic Generalized Epilepsy(IGE) with Generalized Tonic Clonic Seizures(GTCS) only and most common headache type in these patients being post ictal followed by probable migraine without aura and infrequent episodic Tension type headache. Other types of seizure noted were Juvenile Myoclonic Epilepsy (JME) Benign Epilepsy with Centro-Temporal Spikes(BECTS) and Juvenile Absence Epilepsy(JAE). Additional drug requirement was high in cases of migraine without aura and in cases of episodic TTH. HDI was reduced to a significant amount in 80% of the cases after additional treatment.

Conclusions: This is an ongoing study. Overall apart from post ictal headache TTH and migraine was seen in patients of JME and IGE with GTCS only. We noted migraine in two out of 4 patients of BECTS as expected. Patients of JAE did not have any type of headache in the course of the disease. In majority of patients Migraine and TTH required additional therapy but surprisingly in majority of the cases there was significant reduction in the headache frequency and reduction/ improvement in HDI.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

EO6/130: The Dilemma of Arranged Marriages in People with Epilepsy. An Expert Group Appraisal

Gagandeep Singh, Caroline Selai, Renu Addlakha, John Duncan, Urvashi Shah, H.V. Srinivas, Debashish Chowdhury, Manish Modi, Yashoda Wakankar, Satish Jain

Dayanand Medical College & Hospital, Ludhiana, Punjab

Introduction: Patients with epilepsy (PWE) are confronted by a number of psychosocial challenges in their life but the most significant of these is during matrimony. Worldwide PWE are less likely to marry; however in arranged marriages their marital prospects are most seriously compromised. Arranged marriages have declined considerably in post-industrial westernized cultures but constitute nearly 80% of all marriages in many under-resourced societies. By coincidence 80% of PWE also reside in these under-resourced countries. Here we review the dilemma of arranged marriages in PWE to document marital prospects and outcomes.

Aims: To document marital prospects and outcomes in PWE who go through arranged marriage and to propose an optimal practice framework for counselling PWE contemplating arranged marriage based on expert opinion.

Results: The matrimonial repercussions are heavily biased against women who experience a high level of stigma during arranged marriages. Although divorce rates are generally not very high in PWE the divorce rates seem to be higher in PWE undergoing arranged marriages as hiding epilepsy during marital negotiations appears to be a risk factor for divorce. Hiding epilepsy appears common during marital negotiations in arranged marriages but is associated with many adverse consequences including poor adherence to medications and treatment gap besides poor marital outcome.

Conclusions: In under-resourced communities in which arranged marriages are common physicians caring for PWE are perhaps best resourced to counsel them about their marital prospects. The issue should be discussed between the physician and the family of PWE in proactive manner.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

Platform Session 03, Friday, October 02, 2015, 8: 30 AM- 09:30 AM Dr. M. P. Mehrotra (Hall C): Neurological Infections: IO1/299: Incidence of seizure disorder associated with solitary cysticercus granuloma and single calcific NCC: A Study among children accessing Government Schools in Hyderabad district

Shyam Krishnakumar Jaiswal, JMK Murthy, Padmanabh M. Reddy, Sri Krishna S

Background: Seizure disorder associated with all evolutive stages of neurocysticercosis (NCC) is highly prevalent in countries endemic to NCC.

Objective: To find incidence of seizure disorder associated with these common forms of NCC in children in Government Schools in Hyderabad.

Material and Methods: Study population included 7,408 school children aged between 5-15 yrs seeking education in 18 government primary schools randomly selected from 250 schools adopted by the XXX Foundation under School Health Program in Hyderabad. Study period was between January 2006 and December 2012. All new-onset cases of seizure disorder (>1 seizures) during study period were evaluated in a structured format. Data collected includes demographic profile, date of onset of seizure, number of seizures, seizure semiology, neurologic findings, EEG and CT findings, antiepileptic drug (AED) prescription, and seizure remission. Most of children were from slums with unhygienic surrounding and significant pig population. Seizure disorder associated with degenerative phase of NCC were categorized under acute symptomatic seizures

Results: Of the 58 new-onset seizures during study period, 24 (41.4%) had seizure disorder associated with one of the evolutive stages of NCC; 19 (32.75%) with solitary cysticercus granuloma (SCG) and 5 (8.6%) with calcific-stage NCC. Incidence of seizure disorder associated with both the forms of NCC was 46.28 per 100000 in total cohort (5-15 years); SCG (acute symptomatic seizures) 36.63 per 100000; and single calcific NCC (unprovoked seizures-epilepsy): 9.64 per 100000.

Conclusions: This study suggests incidence of acute symptomatic seizure associated with SCG and epilepsy associated with calcific-stage of NCC is significant in children in government primary schools, much of which could preventable.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

IO2/59: Estimation of Cerebrospinal Fluid Cortisol level as a Diagnostic Marker in Acute Bacterial Meningitis

Anish Mehta, Rohan Mahale, Ajuabraham John, Kiran Buddaraju, Abhinandan KS, Madhusudhan BK, Suryanarayana Sharma, Mahendra JV, R Srinivasa

Department of Neurology MS Ramaiah Medical College, Bangalore, Karnataka

Background: Meningitis remains a serious clinical problem in developing as well as developed countries. Delay in diagnosis and treatment results in significant morbidity and mortality. The role and levels of intrathecal endogenous cortisol is not known.

Objective: To study the cerebrospinal fluid (CSF) cortisol levels and to evaluate its role as a diagnostic and therapeutic marker in acute bacterial meningitis.

Methods: Thirty patients with acute bacterial meningitis with no prior treatment were evaluated. Cortisol levels were compared with twenty patients with aseptic (viral) meningitis and 25 control subjects.

Results: Mean CSF cortisol level was 13.85 3.47 and 1.05 in bacterial meningitis aseptic meningitis and controls respectively. Mean CSF cortisol level in bacterial meningitis was significantly higher as compared to controls (p<0.001). There was significant difference in CSF cortisol levels in bacterial and aseptic meningitis (P < 0.001).

Conclusion: Cortisol levels in CSF are highly elevated in patients with acute bacterial meningitis. This suggests that intrathecal cortisol may serve as a valuable rapid relatively inexpensive diagnostic marker in discriminating between bacterial and aseptic meningitis. This helps in earlier institution of appropriate treatment and thereby decreasing morbidity and mortality.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

IO3/74: A Study of Clinical Profile Outcome and Complications of Herpes Simplex Encephalitis

Manmeet Kaur, Suman Kushwaha, Sarabjeet Khurana, Rajeev Thakur, Om Prakash

Institute of Human Behaviour and Allied Sciences, New Delhi, Delhi

Background: Herpes simplex encephalitis (HSE) is the commonest sporadic encephalitis world wide carrying significant mortality and sequelae.

Objectives: To study the clinical profile outcome and neurobehavioral sequelae in patients with HSE.

Material and Methods: Thirty three patients with HSE were analysed. Of these 17 were prospectively followed up for six months while 16 were analysed retrospectively. Diagnosis was confirmed on the basis of clinical criteria and herpes simplex virus polymerase chain reaction (HSV-PCR) along with other investigations including neuro-imaging CSF and EEG. Outcomes were assessed using Glasgow Coma Scale (GCS) Glasgow Outcome Scale (GOS) measured at 1 3 and 6 months and the cognitive and neuro-behavioral sequelae were studied as per BCRS (Brief Cognitive Rating Scale) NPI (Neuropsychiatry Inventory) and MINI PLUS neuropsychiatric interview.

Results: Thirty- three patients enrolled. Mean age 36.8 ±15.7 years with duration of symptoms 8 ± 3 days with fever (97%) headache (85%) seizure (76%) and altered sensorium (67%) as presenting symptoms. HSV-PCR in CSF was positive in 28 of 30 patients. MRI was abnormal in all. All patients received Acyclovir. In hospital mortality was 15 % and 21% at 6 months. At 6 months no patient made a good recovery. Commonest symptom in the 25 survivors was depression (36%) anxiety (32%) mania (16%) and combined anxiety + OCD (8%) and mania +OCD (8%). On BCRS Global Deterioration Scale mild to moderate impairment in cognition in 57.4% and severe in 27%.

Conclusions: Age at presentation duration of symptoms and GCS on admission are associated with a poor outcome. Early diagnosis and treatment portend a good survival with empirical treatment with acyclovir started promptly on clinical suspicion until a diagnosis is clearly excluded.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

IO4/99: Mucormycosiswith Neurological Involvement- A Retrospective Study

Mohammed Shafi A, Dhanaraj M

Apollo Main Hospital, Chennai, Tamil Nadu, India

Aim: To study the clinical profile radiological features treatment and outcome of mucormycosis with neurological involvement.

Methods: All patients with mucormycosisconfirmed either by biopsy or by culture presenting with neurological involvement admitted between Jan 2010 and May 2015 were studied retrospectively. The risk factors clinical radiological featurestreatment and outcomes were analysed.

Results: Among 27 patients admitted with mucormycosis 11 patients had neurological involvement. All had type 2 diabetes mellitus including diabetic ketoacidosis in three had chronic kidney disease (CKD)one had chronic liver disease and one had chronic obstructive pulmonary disease on corticosteroid use. Major clinical presentations were headacheexternal opthalmoplegiaproptosis facial swelling fever and few had facial numbness visual loss and hemiplegia. Imaging showed sinusitisacute otitis mediacavernous sinus thrombosismultiple infarcts and intracerebral bleed. Four patients had both septate and aseptatefungi. Seven patients underwent surgical debridement FESS in six including eye exenteration in two and mastoidectomy plus tympanoplasty in one. Among the seven two patients died. One died due to late presentation another died due to inadequate treatment. Among those who did not undergo surgery two died due to rapid deterioration and the remaining two died after discharge against medical advice.

Conclusions: The predominant risk factors for mucormycosis with neurological involvement were uncontrolled diabetes mellitus and chronic kidney disease. External opthalmoplegia and proptosis with predominant involvement of sphenoid and ethamoid sinuses in imaging was the commonest presentation. Few patients had both septate and aseptate fungi. Early diagnosis early surgical debridement and early initiation of antifungal treatment carries better outcome. Diabetic ketoacidosis along with CKDcentral retinal artery occlusion and intracranial involvement carried poor prognosis.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

IO5/123: The Role of MRI Parameters as Prognostic Markers in the Outcome of Solitary Cysticercus Granuloma

Loveleen Aggarwal, Gagandeep Singh, KavitaSaggar, G.S. Dhooria

Dayanand Medical College & Hospital, Ludhiana, Punjab, India

Background: Solitary cysticercus granuloma (SCG) represents a degenerating single cerebral cysticercus cyst; is a common presenting form of Neurocysticercosis (NCC) particularly in India and United States; and may cause refractory seizures. Because of the epidemiologic importance of NCC an optimal magnetic resonance imaging (MRI) prognostic protocol is vital and advanced MRI techniques have recently been used in this context.

Aims and Objectives: To study selected MR imaging parameters as prognostic markers in the outcome of resolution of SCG.

Design/Methods: Patients with seizures and demonstrating intracranial SCG diagnosed in the department of Neurology DMC & Hospital Ludhiana over a period of one year were subjected to non-conventional MRI evaluation including diffusion weighted imaging with ADC calculation; proton magnetic resonance spectroscopy (MRS) and magnetization transfer (MT) imaging. Six months follow up MRI was done. The associations of various demographic and advanced MRI techniques in predicting the resolution of the SCG were analyzed.

Results: MRI features which favour the trend towards complete resolution of the SCG lesion are a supratentorial location lower creatine levels on MRS lower NAA/Choline and higher Choline/Creatine ratios on MRS higher perilesional MT ratio T1 values absence of perilesional gliosis and absence of seizure recurrence. But none of these variables except supratentorial location and higher Choline/Creatine ratio on MRS reached a statistical significance primarily because of the small sample size.

Conclusion: MR imaging is the investigation of choice for determining the prognostication of SCG. Advanced MRI techniques are complementary to conventional MR imaging for this purpose. However larger prospective studies with a longer duration of follow-up are needed to statistically establish the above observations.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

IO6/195: A comparative study of cognitive and neurobehavioral changes in Japanese encephalitis and herpes simplex encephalitis

Vinita Elizabeth Mani, Usha Kant Misra, Jayantee Kalita, Sanjeev Kumar Bhoi

Professor& Head, Department of Neurology, Lucknow, Uttar Pradesh, India

Introduction: Japanese encephalitis (JE) is the commonest endemic encephalitis and herpes simplex encephalitis (HSE) is the most common focal encephalitis without any regional or seasonal predominance. Both are primary neurotropic viruses with different clinical presentations. We report and compare the cognitive and behavioral sequelae in HSE and JE. Consecutive cases of proven HSE and JE over a four year period were included and their clinical and MRI findings at admission and neurological status at discharge ascertained. At 1 year their cognitive and psychometric evaluation was carried out which included Mini-mental status examination lobar function testing and Neuropsychiatric Inventory (NPI) besides other tests. There were 18 patients of HSE and 31 patients with JE and sequelae were seen in 17 and 25 patients respectively at 1 year. Patients with HSE had prominent memory deficits (89 %) behavioral abnormalities (28 %) perseveration (39 %) KluverBucy syndrome (22 %) and intractable seizures (33 %). On the other hand patients with JE had motor deficits including hemiparesis (16%) and quadriparesis (13 %) and movement disorders (68 %) including rigidity tremors dystonia and parkinsonism. They also had apathy abulia speech abnormalities and wasting. The MRI correlates included extensive basifrontal and temporal lobe involvement in HSE and gangliothalamic and midbrain involvement in JE. The sequelae of HSE patients include predominant memory loss and behavioral abnormalities which are not seen in JE patients who instead have motor deficits.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

Platform Session 04, Friday, October 02, 2015, 04: 00 PM- 05:00 PM Dr. Baldev Singh Hall (Hall A): Neuromuscular Disorders: NO1/15: Myastenia Gravis: Clinical Spectrum and Long Term Follow-up Study

Janardan, A. Bhargava, K. Shubhakaran, Md. Yasin, Ankit Shaha, K. Sachdev, Pallavi

Dr. S. N. Medical College, Jodhpur, Rajasthan, India

Aim: This study is to analyse the spectrum clinical profile disease course complications and long term outcome.

Materials and Methods: Retrospective study of patients in Dr. S. N. Medical College, Neurology Department, Jodhpur from August 2012 to May 2015.

Results: Data from 42 fairly representative of myasthenicanalysed. There were 30 males 12 females (2.6:1) mean age onset 48. Family history in 2.35%. During first month symptom onset confined to ocular muscles 38% bulbar muscle 8% and extremities 6.5% while 47.5% individuals have generalized MG. 16.66% patient with crisis at onset. Of the patient with ocular myasthenia onset 50% developed generalized within 6month those more than 1 year isolated symptom not progressed. At last follow up (mean 1 year) 38.09% patients asymptomatic 33.33% reported improvement and 26.16% (frequent crisis plus death). Intermittent follow up 19.04%. Drug free stable remission 9.5%. Thymoma in16 patient (38.09%) Thymectomy 11.9%. Better outcome in thymectomy.16.66% under gone crisis mainly to drug default / infection. Median interval onset to crisis18month. Recovery > 90% (Ventilatory support plus IVIg). We lost 2 patients > 60 years with multiple tuberculoma and diabetes herpes zoster encephalitis. One developed avascular necrosis another emphysematous pyelonephritis undergone nephrostomy saved. One developedKlebsiella Pneumonia (Azathioprin induced) later CIDP ??Incidental responded to IV Cyclophosphamide.

Conclusion: This study shows drug default / inadequate follow up as major cause for crisis/deterioration. Infection mainly respiratory tract co-morbid conditions like diabetes elderly long term side effect steroid/ immunosuppression were leading challenges.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

NO2/112: A Study on Clinical and Radiological Profile of Hirayama Disease in a Tertiary Care Hospital of Eastern India

Debsadhan Biswas, Alak Pandit, Deb Sankar Guin

Bangur Institute of Neurosciences Kolkata, West Bengal, India

Background: Hirayama disease (brachial monomelicamyotrophy) is a unilateral or grossly asymmetric bilateral disease. Though bilaterally symmetric involvement has been described still it is rare in India.

Methods: The consecutive patients presenting with insidious onset hand atrophy in a tertiary care hospital of Eastern India between December 2013 and May 2015 were evaluated clinically electro physiologically and with MRI cervical spine flexion contrast imaging to diagnose Hirayama disease. All those found to have bilaterally symmetric involvement on clinical and electrophysiologic basis were evaluated for clinical electrophysiologic and MRI correlates other than those required for the diagnosis.

Results: All patients (n=50) were males less than 23 years of age with median age 19 years. All (100%) had oblique amyotrophy 38 (76%) cold paresis 35 (70%) minipolymyoclonus. Neutral position MRI showed loss of cervical lordosis localized lower cervical cord atrophy in all patients asymmetric cord flattening in 44 (88%) and intramedullary hyperintensity in 12 (24%); flexion study showed loss of dural attachment anterior displacement of dorsal dura epidural flow voids and enhancing epidural crescent in 50 (100%). Eight patients (16%) were found to have bilaterally symmetric involvement. The important characteristics of this type of presentation included severe weakness and wasting in C7 C8 and T1 myotomes cold paresis and a very prominent bilateral minipolymyoclonus. MRI during complete flexion of neck showed severe bilateral flattening of lower cervical spinal cord against C5-C6 vertebral bodies and crescent-shaped enhancing epidural space on contrast study extending from C3 to T2 spine.

Conclusion: Bilaterally symmetric Hirayama disease is common in eastern part of India and it is a severe form of the classic disease which remains undiagnosed due to a common notion that it is a unilateral or grossly asymmetric disease.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

NO3/133: Clinical Profile of Patients with Charcot-Marie-Tooth Disease

Sruthi S Nair, Muralidhran Nair

Sree Chitra Tirunal Institute for Medical Sciences, Thiruvananthapuram, Kerala, India

Introduction: Charcot Marie Tooth disease (CMT) is a genetically diverse group of inherited peripheral neuropathies with scarce data from India.

Aim: The study aimed to investigate the clinical and electrophysiological profile of patients with CMT from a tertiary care centre in South India.

Methods: The study was a retrospective analysis of patients with a clinical diagnosis of CMT based on clinical criteria and ruling out of alternate etiological factors. The study period was 7 years from January 2006 to December 2013. The demographic profile clinical features electrophysiological and pathological data were extracted from the patient records using a structured proforma.

Results: 32 patients satisfied the inclusion criteria for CMT with male-female ratio of 1.28 (18:14). The mean age of onset was 11.15 years (range 1-33 years). Onset was with lower limb weakness or wasting in 25 patients (78.1 %) and upper limb symptoms in 6 patients (1.9 %). Sensory symptoms were reported only by 3 (9.4 %) patients however signs were noted in 19 (59.4 %). All patients were noted to have deformities. A positive family history was obtained in 14 (43.7%) patients. Electrophysiology studies revealed axonopathic pattern as the commonest presentation noted in 25 (78.1 %) patients. Sensory conduction abnormalities were noted only in 17 (53.1 %) patients. Nerve biopsy was done in 18 (56.2 %) patients which correlated in all the cases. Follow up of 25 patients for an average period of 3 years showed progression only in 2 cases.

Conclusions: Motor predominant polyneuropathy with deformities should raise the suspicion of CMT. Axonal neuropathy was the most common presentation in our study group. Clinical categorization of the disease is of great importance in view of the heterogeneity and cost of genetic analysis.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

NO4/168: Autonomic Function Testing and Antiganglioside Antibodies Assessment in Correlation with Short Term Outcome in GBS

A. Gopi Krishna, A. Gopi Krishna, U. ArunaKumari, T. Sateesh Kumar, S.Gopi, G. Butchi Raju

Neurology resident, Visakhapatnam, Andhra Pradesh, India

Background: Autonomic dysfunction (AD) is a common and important complication in Guillain-Barre syndrome (GBS) and may be the cause of significant morbidity or death. About 20% of all GBS patients have symptoms involving both sympathetic and parasympathetic fibers. This rate rises to 75% in patients with quadriplegia. Association of antiganglioside antibodies to axonal or demyelinating GBS is a matter of controversy.

Objectives: To clarify association of antiganglioside antibodies with axonal dysfunction and show the prevalence of AD in GBS and its association with the severity and outcome of disease.

Material and Methods: Forty patients with clinical and electrophysiological diagnosis of GBS were prospectively studied between Jan 2014 and May 2015. Serum antiganglioside antibodies were measured by immunoblot technique in single laboratory. The sympathetic skin response (SSR) and cardiovascular autonomic function tests by CANS machine are studied in all patients.

Results: Of the 40 patients45%had AD and 15% had antibodies positive. The severity and recovery of illness was similar in both axonal and demyelinating subtypes. In 12 patients SSR couldn’t be elicited and in 6 patients amplitude was decreased. Parasympathetic dysfunction was seen in 10 patients. The commonest ganglioside antibody was IgG GT1B.

Conclusion: The patterns of autonomic involvement are qualitatively different between AIDP and AMAN. Our results suggest that SSR and other autonomic tests may be used for early detection of any autonomic involvement in patients with GBS. Antibodies are positive in only 15% of the cases and didn’t show any correlation with subtypes of GBS or their outcome.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

NO5/182: Mitochondrial Respiratory Chain Disorders: Phenotypic Biochemical and Genetic study in Eighty Five Children

Kothari Sonam, ParayilSankaranBindu, Arun B Taly, Srinivas Bharath MM, Narayanappa Gayathri, Hanumanthapura R Arvinda, Madhu N, Sinha Sanjib, PeriyasamyGovindaraj, ThangarajKumarasamy

NIMHANS, Bangalore, Karnataka, India

Background: Mitochondrial respiratory chain enzyme deficiency accounts for a wide variety of neuromuscular disorders in children. There are only limited reports on mitochondrial respiratory chain disorders in children from India.

Patients and methods: The study included eighty five children (Mean age: 6.9±4.7yrs M:F::2:1) with respiratory chain enzyme deficiency seen over a period of two years (2011-2013). Respiratory chain complex assays were done by spectrophotometry. Genetic analysis included sequencing of mitochondrial genome SURF1 POLG1&2.

Results: Twenty three patients had syndromic diagnoses which included Leigh syndrome (12) mitochondrial encephalopathy with lactic acidosis and stroke like episodes (4) Leber's hereditary optic neuropathy (2) chronic progressive external ophthalmoplegia (4) and juvenile Alper's syndrome (1). The major non syndromic presentations included episodic encephalopathy (12) ataxia (12) extrapyramidal disorder (8) epilepsy (6) and myopathy (5). The imaging findings ranged from normal MRI (13) to spectrum of findings namely striatal lesions (18) brainstem lesions (18) leukoencephalopathy (16) and cerebellar atrophy (12). Most common deficiency was complex 1 (50) followed by multiple complexes (24) complex IV (8) and complex III (3). Histopathology revealed ragged red fibers with cytochrome oxidase (COX) deficiency (10) isolated COX deficiency (8) and type II fiber atrophy (24). Molecular analysis revealed mitochondrial DNA mutations (14) POLG1 (3) POLG2 (5) and SURF1 (5).

Conclusion: This study first of its kind from India highlights the wide range of clinical and imaging phenotypes and genetic heterogeneity in children with mitochondrial respiratory chain disorders. Knowledge of the same may facilitate targeted metabolic and genetic testing.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

NO6/214: A Treatable Myopathy with Respiratory Difficulty: A Study of Seven Index Cases

Amitkumar Pande, Satish Khadilkar, Jimmy Lalkaka, Nasli Ichaporia, Rakesh Singh, Ashish Bhutada, Nahush Patil

Bombay Hospital, Mumbai, Maharashtra, India

Introduction: Pompes disease is a rare progressive and fatal disease. The spectrum of clinical presentation varies depending on the age of onset rate of disease progression and organ involvement. Cases have been described from almost all over the world and Asia. To our knowledge the literature of Pompes in India is very little; adolescent and adult onset is uncommon and not described from India. Diagnosing early is particularly important because of the availability of the myozyme now in India which alters disease course. Here we present a short series of late onset Pompe disease and discuss the clinical and laboratory features of seven index cases.

Materials and Methods: Patients presenting to a tertiary care neuromuscular clinic in 20 years (1996 to 2015) were included. They underwent history examination CK electrophysiology and muscle biopsy tests. Biopsies were stained for H and E PAS and diastase. The confirmation of diagnosis was done by muscle biopsy demonstration of vacuolar myopathy with PAS positive inclusions. When feasible assay of enzymatic activity of acid ± glucosidase (GAA) and genetic studies were carried out.

Inclusion criteria: Clinical syndrome of a myopathy2) Patients who fulfilled the histopathology enzyme assay or genetic diagnostic criteria

Exclusion criteria: Other muscular dystrophies myositis and congenital myopathies.

Results: The age of onsetranges from 8-31 yrs (mean 19.5 yrs). The delay in diagnosis from time of onset was on an average 6 yrs and maximum was 14 yrs. All the patients had limb girdle syndrome with 5/7 having respiratory affection. Cardiac evaluation was normal in all. Enzymatic assay was available in 3/7 and muscle biopsy was positive for vacuolar change and pas positive inclusions in all 7/7. Genetic study was available only in 1/7. Three patients were started on myozyme and are stable with BIPAP.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

Platform Session 05, Friday, October 02, 2015, 04:00 PM- 05:00 PM Dr. K. S. Mani Hall (Hall B): Dementia and Cognitive Disorders: DO1/9: Harmonizing Research in Vascular Cognitive Impairment across Educational Levels and Languages

Suvarna Alladi, Gowri K. Iyer, Aparna Dutt, Ratnavalli Ellajosyula, Amitabha Ghosh, Ashima Nehra, P.S. Mathuranath, Subhash Kaul, Ramshekhar Menon, Manjari Tripathi

NIZAM's Institute of Medical Sciences, Hyderabad, India

Background: With the rising burden of cognitive impairment due to cerebrovascular disease it is vital to understand the pattern of disease to develop appropriate preventive and treatment strategies. Diagnosing Vascular Cognitive Impairment (VCI) using clinical and neuropsychological tests in heterogeneous socio-linguistic settings is challenging and harmonising research across diverse settings is crucial for this purpose. The diversity in the Indian subcontinent provides an opportunity where we can develop harmonisation strategies across these variables.

Aim: To develop validate and harmonize a comprehensive clinical and neuropsychological test battery for studying VCI across different literacy levels and languages.

Methods: A multicentric study across five cities worked towards harmonizing methods to study cognition and behaviour in the Indian context of cultural socioeconomic and linguistic diversity. Protocols were developed and harmonized in six languages: Indian-English Hindi Bengali Telugu Kannada and Malayalam among both literates and illiterates. Protocols were also harmonized for uniform diagnosis of stroke with normal cognition mild cognitive impairment and vascular dementia.

Results: A comprehensive protocol was developed based on VCI harmonization battery of NINDS-AIREN and Canadian Stroke Network consortium to assess cognition behavior and function in 6 languages. Existing tests were culturally adapted and novel tests for illiterates were also developed. The process of adaptation validation establishing psychometric properties of tests and measuring test invariance across languages was systematically conducted in a large cohort of 1200 subjects which included healthy controls and stroke patients.

Conclusion: A key outcome was to create a pan-Indian comprehensive validated clinical and neuropsychological test battery for VCI for use across a range of clinical and research settings.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

DO2/68: Bilingualism Improves On Cognitive Outcome after Stroke

Pesala Pavan Kumar, Suvarna Alladi, Thomas H Bak, Shailaja Mekala, AmulyaRajan, Jaydip Ray Chaudhuri, Rajesh Kroviddi, Bapiraju Surampudi, Vasanta Duggirala, Subhash Kaul

NIZAM's Institute of Medical Sciences, Hyderabad, India

Introduction: The question whether bilingualism can protect against cognitive decline is a topic of intense debate. Several studies reported a 4-6 year delay in the onset of dementia in bilinguals however its effect on other neurological disorders causing cognitive decline such as stroke is not known.

Objective: We studied cognitive outcomes in stroke patients in a well characterised ischemic stroke cohort.

Material and Methods: We examined 608 consecutive ischemic stroke patients from the NIMS stroke registry for cognitive outcomes 3-24 months after stroke using a validated neuropsychological protocol and standard diagnostic criteria.

Results: The 608 patients vascular dementia was diagnosed in 189 (31.1%) vascular mild cognitive impairmentaI in 159 (26.2%) aphasia in 67 (11.0%) and 193 (31.7%) were found to have normal cognitive functioning. A larger proportion of bilinguals had normal cognition compared to monolinguals (40.5 % vs 19.6 % p<0.0001) while the reverse was noted in VaMCI (Monolinguals 31.8 % vs Bilinguals 22.1 % p = 0.041) and VaD (Monolinguals 36.9 % vs Bilinguals 26.9 % p = 0.054). There were no differences in the outcome of aphasia between monolinguals and bilinguals (11.8 % of monolinguals vs 10.5 % of bilinguals p = 0.655). Bilingualism was found to be an independent predictor of post-stroke cognitive impairment on logistic regression analysis.

Conclusions: Our results support the notion that bilingualism leads to a better cognitive reserve with a positive effect not only on neurodegenerative dementias but also on stroke outcome.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

DO3/94: Increased prevalence of possible NPH in a cohort of hospital based dementia patients

Robert Mathew, Sauda

Pushpagiri Institute of Medical Sciences, Trivandrum, Kerala, India

Background: In spite of growing awareness of dementia patients in this part of the country still seek medical help more readily for physical disability than cognitive impairment. This may reflect in cohort of hospital based patients.

Objective: To study the dementia subtypes of a hospital based cohort of dementia patients.

Method: Cross sectional descriptive study (period 2010 to 2013). Patients availing neurology service in 2 tertiary referral hospitals were selected.

Results: Four hundred and twenty hospital based patients were screened positive for dementia. A diagnosis of dementia was confirmed in 283(63.78%). Detailed evaluation including neuropsychology was done in 158(37.5%). Mean age was 71.19(9.46) 250(59%) were males and mean education 8(SD4.4) years. Of the patients who underwent diagnostic work up 120(42.2%) had possible normal pressure hydrocephalus(pNPH) and 45(15.9) had Alzheimer's dementia (AD) and diagnosis was inconclusive in 57(20.1%). Mean age of patients with pNPH was 72.67 (SD 8.46) Male 74.5% education 8.13(SD 4.45). Mean duration of illness at the time of presentation was 2.62 (SD 2) mean ACE-R score was 37.34(SD 18.63). When compared with patients with AD no significant difference was seen in age sex duration of illness at the time of presentation or cognition scores (ACE-RMMSE) ANOVA p>0.5.

Conclusion: In this hospital based cohort of dementia patients the number of patients with possible NPH far out weighted patients with other dementias. This referral bias is possibly because of physical disability which is considerably more in pNPH than other degenerative dementias.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

DO4/95: Clinical Characteristics of Advanced Dementia

Sunitha Robert, Robert Mathew, Sauda

Pushpagiri Institute of Medical Sciences, Trivandrum, Kerala, India

Aim: To compare the clinical features of advanced dementia with early stage of dementia

Introduction: Clinical features of dementia changes as the disease advances. Not only that the existing deficits worsens new deficits appear as well.

Method: Prospective cross sectional study Setting: Data from patients availing dementia care services from 3 different tertiary care centres were analyzed.

Results: Data from 900 patients were analyzed. Neuropsychologic tests could be done in 283 patients and definite diagnosis was available in 279(29.7%). Based on ACE R score patients were divided into 3 groups. There were 140 patients with early dementia 40 with advanced dementia and 103 in the intermediate group. The mean ACE R score in the groups were 66.66±9.737.62±7.213.86±7.3 respectively. The mean age 69.88 (±11.3). Mean age of early dementia group (67.27±12.84) was significantly less than the advanced dementia group(73.42±8.1)p<0.001. Females were less than males. Mean duration of illness was 2.84 years (± 1.98) and was comparable across the groups (2.832.872.8 respectively). Mean education was 8.97± 4.5. Education was significantly more in early dementia group (11.12±3.53) than in advanced dementia(5.55±4)p<.001. Significant correlation was seen between dementia stage and age as well as education even after controlling for each other (P<.001). No correlation with duration of illness(p0.94).

Conclusion: Less educated patients tend to present at an advanced stage of cognitive impairment. Duration of illness at the time of presentation did not influence the degree of cognitive impairment probably because of the variability in progression of disease across various dementia syndromes.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

DO5/162: A Study on the Recognition of Facial Emotions in Patients with Right Hemispheric Stroke

Philo Hazeena P, K.Bhanu, V.Kannan

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

Background: Stroke is the leading cause of physical disability and it significantly affects the life of the patients. Stroke causes impairment in the motor control language memory and in addition can also produce a disruption in a patient's ability to perceive emotions. The right hemisphere has a critical role in the recognition of emotions.

Aims and Objectives: To study the ability of the right hemispheric stroke patients in recognizing the emotions from the facial expressions and to analyse the differences in the particular emotion affected in distinct cortical lesions.

Materials and Methods: This was a cross sectional descriptive study among 74 patients. We had assessed in them the recognition of emotions through facial expressions for happiness anger sadness disgust surprise and fear.

Results: Among all the six emotions evaluated in our study it was noted that the negative emotions were the most affected and in particular the emotion of sadness (50%). The emotion of sadness and anger was most affected in the parietal region (75%) fear in the thalamus (87.5%) and disgust in capsuloganglionic region (50%). The positive emotion of happiness was recognized by 90.5% of the patients.

Conclusion: In our study 63.5% right hemispheric stroke patients had difficulty in recognition of facial emotions and so they might react indifferently and inappropriately to a given situation. This could inturn have an impact on the interpersonal relationship. Hence recognizing these patients could help in the therapeutic and rehabilitative measures.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

DO6/293: Assessment of visual misperceptions in patients of Parkinson's disease using single and bistable percepts as testing tool

Ankur Wadhwa, Bhupender Kumar Bajaj

PGIMER & RML Hospital, Delhi, India

Background: There is paucity of standardized methods for evaluation of visual misperceptions in patients with Parkinson's disease (PD). The present study was undertaken to evaluate the utility of single and bistable visual percepts for detecting visual misperceptions in PD.

Aims & Objectives: To assess visual misperceptions in patients of PD using single and bistable percepts as testing tool.

Methods: Thirty patients with PD fulfilling inclusion-exclusion criteria and thirty age-sex matched healthy controls were recruited. The severity and stage of disease were assessed using UPDRS (Part- III) and modified Hoehn &Yahr scale. Higher cognitive functions were evaluated by Mini Mental State Examination (MMSE), Frontal Assessment Battery (FAB) and Trail making tests (TMT-A & B) in all the subjects. Visual misperceptions were detected using monstable and bistable monochromatic images.

Results: The mean age of patients and controls were 59.23 (±5.11) and 59.27(±5.11) years respectively. Both the groups, patients and controls, comprised of 24 males and 6 females. More than 80% of patients were in H&Y stage 1-2.5. Patients compared to controls had significantly higher visual misperceptions (Patients: 7.73± 2.24; Controls: 4.8±1.3; p<0.005). Visual misperceptions positively correlated with disease and treatment duration, UPDRS (Part-III) score, H&Y stage and TMT A& B (p<0.0001). Inverse correlation was observed between visual misperceptions and, MMSE and FAB score (p<0.0001).

Conclusions: Patients with Parkinson's disease are detected to have significantly more frequent visual misperceptions when assessed using single and bistable visual percepts as testing tools compared to healthy controls.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

Platform Session 06, Friday, October 02, 2015, 04:00 PM- 05:00 PM Dr. M. P. Mehrotra (Hall C): Headache: HO1/29: Is Palinopsia In Migraineurs An Enhanced Physiological Phenomenon?

Jayantee Kalita, R Uniyal, U K Misra

Lucknow, UP, India

Introduction: We evaluate the frequency of palinopsia in the patients with migraine and correlate this with migraine characteristics triggers and allodynia. This is a cross-sectional case-control study. 153 consecutive migraineurs were included and their clinical details including migraine triggers allodynia and migraine characteristics were noted. Presence of palinopsia was evaluated in migraineurs and 101 controls using a questionnaire followed by a novel method. The clinical characteristics of patients with palinopsia were compared with those without palinopsia. The predictors of palinopsia were derived by multivariate analysis. On questionnaire 9.8% migraineurs and none of the controls had palinopsia. Using novel method of testing 57.5% migraineurs and 12% controls had palinopsia. Migraineurs had palinopsia most frequently to red color (51.6%) followed by yellow (49.7%) blue (47.7%) green (46.4%) and least to white (30.7%). The control group also had palinopsia most frequently to red (9.9%) and least to white (3%) color. The duration of palinopsia in migraineurs was longer than the controls (32.68±20.24 Vs 5.92±4.55sec; P<0.001) and was longest for the red color and shortest for white. Noise (P<0.001) and allodynia were related to palinopsia (P=0.03). On multivariate analysis the predictors of palinopsia were frequency (OR=0.79 95% CI 0.67-0.93 P=0.005) and severity of headache (OR=0.59 95%CI 0.35-0.99 P=0.05) and presence of headache at the time of examination (OR=0.19 95%CI 0.07-0.50 P=0.001). 57.5% migraineurs had palinopsia and the pattern of palinopsia to different colors and its duration was similar to the controls although was more frequent and longer in duration.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

HO2/30: Idiopathic Intracranial Hypertension with Ayurvedic Medication Use

Kuljeet Singh Anand, Prashant Kumar, Arshad Yahya, Jyoti Garg

PGIMER & Dr. RML Hospital, New Delhi, Delhi, India

IIH is characterized by elevated intracranial pressure and papilloedema typically occurring in obese young women. Exact pathogenesis is unknown but disordered cerebrospinal fluid dynamics is implicated in its development. The main associated factors include obesity and drugs. We report two cases of IIH following ayurvedic medication use.

Case1: A-30-year-old male presented with holocranial non-throbbing early morning headache of moderate intensity without transient visual obscurations for 3 months. He had consumed an ayurvedic preparation - Viryashodhanwatiβ for Dhat syndrome for 3 months till one month prior to the onset of headache. On examination fundus showed bilateral papilloedema while VA and EOMS BMI neurological and systemic examination were normal. Routine investigations were normal. Brain MRI revealed prominent subarachnoid space around optic nerves flattening of posterior sclera and empty sella. MRV was normal. LP revealed elevated CSF pressure of >200 mmH2O. CSF was normal.

Case 2: A-26-year-old female Dentist presented with holocranial non- throbbing morning headache of mild intensity without nausea photophobia or phonophobia for 2 months and double vision more on looking towards left and distant objects for one week. She had taken some Ayurvedic preparation for menstrual irregularity for two months prior to the onset of headache. On examination fundus showed bilateral gross papilloedema with bilateral restriction of lateral gaze while VA and EOMS BMI neurological and systemic examination were normal. Routine investigations including Brain MRI and MRV were normal. LP revealed elevated CSF pressure of >300 mmH2O. CSF was normal. Both cases showed immediate relief in headache with drainage of 30 ml of CSF followed by resolution of all symptoms with Acetazolamide.

Conclusion: Certain ayurvedic medication may contribute to IIH and their intake must be inquired.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

HO3/39: Levetiracetam in Migraine Prophylaxis: A Randomized Placebo-Controlled Study in a Rural Medical Institute in Northern India

Archana Verma, Alok Kumar, Vineeta Singh, Dheeraj Srivastava

UP Rural Institute of Medical Sciences and Research, Saifai, Uttar Pradesh, India

Objective: Migraine is often a chronic and disabling disorder. The objective of our study was to assess the efficacy and tolerability of levetiracetam (LEV) in adult migraine prophylaxis.

Methods: We conducted a prospective randomized placebo-controlled study. A total of 65 patients were randomized in a 1:1 ratio to receive LEV (n = 32) or placebo (n = 33). Twenty-five patients completed the study in the LEV group and 27 patients in the placebo group. Thirteen subjects discontinued early during the trial. After a 1-month run in period LEV was started at a dose of 250 mg/d (or the matching placebo) and was increased by 250 mg/wk until the final dosage of 1000 mg/d was reached. The titration phase was followed by maintained phase of 3 months.

Results: In LEV group we found a significant reduction in the frequency (attacks per month) of migraine (from 5.17 [SD 1.19] at baseline to 2.21 [1.47] in the last 4 weeks) and also in severity of migrainefrom (2.75 [0.44] to1.29 [0.75]) as compared to the placebo group. Patients treated with LEV also reported a statistically significant reduction in the quantity of symptomatic drugs needed for symptom control as compared to the placebo group (P < 0.0001). The percentage of patients on LEV who experienced greater than or equal to 50% reductionin headache frequency was 64% compared with 22% for placebo.

Conclusions: Compared with the placebo group LEV offers improvement in headache frequency and severity as well as it lowers the requirement for other symptomatic drugs in adult migraine patients.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

HO4/105: A Study on Clinical Profile of Headache in Young Women

Ramkumar S, Sarala G, Bhanu K, ShunmugaSundaram K

Madras Medical College, Chennai, Tamil Nadu, India

Background and Purpose: Headache is one of the common disorders encountered by a neurologist in their hospital and it causes significant morbidity. This study was conducted to study the aetiological and clinical profile of various headache types among young women in the age group of 20 to 40 years attending our hospital.

Patients & Methodology: It is a cross sectional study. Hundred young women in the age group of 20 to 40 years presenting with primary complaint of headache to the neurology department at the institute of neurology madras medical college Chennai between December 2014 and may 2015 were enrolled in the study. Headache was classified using the international classification of headache disorders 3rd edition (beta version).

Results: Migraine was the most common headache type 45% followed by tension type headache 40%. The other types of headache were as follows post traumatic headache 4% trigeminal neuralgia 2% idiopathic intracranial hypertension 2% sinusitis 2% refractive errors 2% central nervous system lesions 1% hypertension 1% cervicalspondylosis 1%. Migraine without aura constituted 39% while with aura 6%. Among patients with migraine stress was the commonest triggering factor 20[44.4%] followed by fasting 13[28.8%] sleep irregularities 3[6.6%] menstruation 2[4.4%]cheese 2[4.4%] coke 2[4.4%] cold air 1[2.2%] sun 1[2.2%] fried food 1[2.2%].

Conclusions: Migraine was the most common headache type followed by tension type headache. Among migraine patients stress was the commonest triggering factor followed by fasting.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

HO5/109: Migraine: Clinical Features Pattern of Prescription & Adverse Drug Reaction Profile in a Tertiary Care Teaching Hospital

SubhransuSekhar Jena, Monalisa Jena, Mrutunjay Dash, Swati Mishra, Ishwar Chandra Behera

IMS, Bhubhaneswar, Odisha

Introduction: Due to lack of well-defined research in India regarding migraine's clinical symptomatology & anti migraine drugs used more research in this field is required.

Aims and Objective: Our study has aimed to focus on the clinical features pattern of prescription of drugs in the management of migraine & also adverse drug reactions (ADRs).

Materials and Methods: Cross-sectional observational study of migrainers attending both neurology and paediatric OPD over 2 year. Clinico-epidemiological data & ADRs were obtained from patients or their attendants and from records using standard case-report form & CDSCO form respectively.

Observations and Results: Study enrolled 1652 patients from April 2013 to March 2015. Migraine was reported predominantly in female population (68.1%) with frequent onset in 2nd - 3rd decade. Migraine without aura was the commonest presentation 1328(80.4%) while only 324(19.6%) had with aura. 32(1.9%) patients initially presented with status migrainosus. Medications were used for the acute migraine in 1540 patients. NSAIDs alone were prescribed in44.6% and combined with ergots and triptans in 33.8% cases. For acute control of migraine 2546 drugs were prescribed and ADR noted in 203(7.9%) patients. The prophylactic therapy in patients with migraine headache was initiated in 882(53.3%) patients with 1347 drugs. Monotherapy was used most commonly (55.3%) followed by dual and poly therapy. Beta-blockers were most commonly used (57.1%) followed by antidepressants (32.9%) and calcium channel blockers (22.2%). ADRs were reported in 132(14.9%) patients; antidepressants were most common 47(35.6%) followed by beta blockers 31(23.4%) and anticonvulsant 22 (16.6%).

Conclusion: Further large clinical as well as epidemiological studies must be conducted in our country to confirm and further enlighten our observations.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

HO6/191: Preliminary Experience of ICHD Criteria in the Diagnosis of Medication Overuse Headache (MOH)

Sanghamitra Laskar, Usha Kant Misra, Jayantee Kalita, Sanjeev Kumar Bhoi

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

Background: MOH is regarded to be rare in India. ICHD 3 has been proposed for the diagnosis of MOH and is to be finalized soon. We report our experience with the proposed criteria in the diagnosis of MOH.

Subject and Methods: The patient with headache frequency > 15 days/month and using 10 combined analgesic or 15 simple analgesic tablets /month and headache related to medication overuse for 3 months and no other explanation for headache was diagnosed as MOH. This criteria was compared with the established ICHDII R and the result of this observation are presented.

Results: We had 98 patients with chronic daily headache 81 (82.7%) of whom were females 48 (49%) were from urban areas and 50 (51%) from rural areas. Their median years of education was 12 (range 0 to 20) and they belonged to lower middle and middle socioeconomic class. The duration of headache ranged from 1 to 20 years. 96 (97.95%) used abortive medication (NSAIDS 94.9% and ergotamine 3.1%) and 22 patients were on migraine prophylaxis. 88 (89.8%) patients took simple analgesic 4 (4.1%) mixed analgesic and triptans 1 on analgesic and ergotamine and 1 patient ergotamine alone. Using ICHDI 2-R 56 (57.14%) patients were diagnosed as MOH. Using earlier criteria ICHD 12 (12.2%) patients were diagnosed as MOH. This demonstrates higher frequency of ICHD3 in MOH.

Conclusion: We for the first time report MOH form north India. The proposed ICHD3 criteria are more sensitive than earlier ICHDIIR.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

Platform Session 07, Saturday, October 03, 2015, 08:30 AM- 09:30 AM Dr. Baldev Singh Hall (Hall A): Movement Disorder: MO1/43: Opsoclonus Myoclonus Ataxia Syndrome in Children: a Clinical Imaging and Follow up Study from a Tertiary Care University Hospital in India

Akshata Huddar, Parayil Sankaran Bindu, Madhu Nagappa, Rose Dawn Bharath, Sanjib Sinha, Mathuranath PS, Arun B Taly

NIMHANS, Bangalore, Karnataka, India

Background: Opsoclonus-Myoclonus-Ataxia syndrome (OMAS) is a rare disorder with limited experience on clinical course and therapeutic response.

Aims and Objectives: To describe clinical profile investigations and therapeutic outcome in paediatric OMAS between 2005 and 2015.

Patient and Methods: Thirteen children (Age: 2+1.5 years; M:F=1:2.3) of OMAS were included. Results of abdominal USG (n=13) CT of abdomen (n=10) thorax (n=3) and neck (n=1) metaiodo benzyl guanidine (MIBG) (n=2) and 18-Fluoro-deoxyglucose positron emission tomography (FDG-PET) scan (n=2) were reviewed.

Results: Eight subjects reported antecedent event (respiratory infection: 6; vaccination: 2). Commonest referral diagnosis was acute cerebellitis (n=8). Hypotonia (n=9) abnormal behaviour (n=9) and neuro-regression (n=5) were frequent. Brain MRI cerebrospinal fluid and urinary vanillylmandelic acid were normal in all. Seven patients had underlying tumor (abdomen-4 thorax-2 neck-1) detected by ultrasound (n=4) and CT (n=7). CT identified tumor in two patients in whom MIBG was negative. All patients received steroids for 20±18 months (3 months to 5 years). OMAS preceded malignancy in two by 3-4 months. Eight required prolonged immunomodulation (>12 months). Complete remission after follow-up of 27± 20 months.(4 months to 5 years) was noted in four patients while rest had persisting behavioural and cognitive abnormalities. Twenty-five relapses were noted in nine patients related to inter-current infections (n= 6) tapering or discontinuing steroids (n=2). Patients presented with isolated symptoms or the full-blown syndrome during relapses.

Conclusion: OMAS in children runs an indolent course requiring careful monitoring and long-term immunomodulation. Abnormal behavior is common and outcome is variable. CT scores over other investigations in detecting underlying tumor.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

MO2/50: Quantifying Bradyphrenia at the bedside- a pilot study

Porrselvi A.P., U. Meenakshisundaram, V. Shankar

Sri Ramachandra University, Chennai, Tamil Nadu, India

Background: Bradyphrenia is a symptom of many neurological and psychiatric disorders. The currently used neuropsychological measures used to assess information processing speed have visual and motor components to them and cannot be used in many clinical populations. In a comparative analysis of five of the commonly used processing speed measures it was found that all the speed subtests were dependent on motor speed(1). Numerical and Figural competencies also had an influence on test performance (1). Thus these tests cannot be used in a low-literacy level population or at the bedside. Aside from the measures of information processing speed clinical and bedside measures of bradyphrenia are not yet available (2) to identify cognitive slowing and to empirically measure its severity in patients with or without motor deficits and of varying educational and cultural backgrounds. Also clinicians require a test that can be administered quickly in a busy clinic.

Materials and Methods: 22 community dwelling healthy volunteers were administered the Symbol Digit Coding Test currently considered the best measure of information processing speed from the NIMHANS Neuropsychological Battery (3)a 25-item questionnaire that was developed for the ongoing study to quantify bradyphrenia in the bedside the Stroop Colour and Word Interference Test and Trail Making Test Part A and B(4). Statistical correlation was done between the test performances on each of the tests.

Results: The reading task of the Stroop Colour and Word Interference Test (-0.66) and the 25-item bradyphrenia questionnaire (-0.57) had the highest correlation coefficient with the test performance in the Symbol Digit Coding Test. Both these tasks are free of any motor component other than for involvement of oculomotor and speech functions.

Conclusion: Thus these tests can be valuable bedside and clinical tools to screen for and quantify bradyphrenia in conditions like stroke PD etc. where motor function is impaired.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

MO3/84: Impact of Bilateral Subthalamic Nuclei Deep Brain Stimulation on Gait in Idiopathic Parkinson's disease Patients

Lakshmi Chaithanya Rangineni, Rukmini Mridula Kandadai, KagithaVenkat Padma Vani, Aneel Kumar

Puligopu, Afshan Jabeen, Meena A Kanikannan, RupamBorgohain

Introduction: Gait disturbances are main reasons for morbidity in Parkinson's patients. The role of bilateral subthalamic nuclei deep brain stimulation (STN-DBS) on gait is not clear.

Aim: To assess change in gait in patients with idiopathic Parkinson's disease after bilateral subthalamic stimulation from a tertiary care hospital.

Subjects and Methods: Patients with idiopathic Parkinson's disease (duration of >5 years response to levodopa UPDRS-III score of >30 points during off phase) who underwent STN DBS were included.

Results: Sixty patients underwent surgery with bilateral STN microelectrode implantation. Post surgery average levodopa dose decreased from 855±367mg/per day to 494±260mg (p < 0.01) at 1yr. On period improved from 6.7±2.2 to 16.1±2.4 hrs/day at one yr (p value < 0.01). Overall UPDRS-III scores in on and off state improved significantly from 55.4±14.1 and 16.5±8.7 to 32.2±12.5 (42 %) and 11.9±6.2 (31 %) respectively at one yr follow up. Gait and postural stability improved significantly in off state at 1year follow up (2.5+0.9 and 2.3+1.0 to 1.5+0.9(p<0.0001) and 1.9+0.9 (p<0.0001) respectively). Freezing episodes reduced significantly in both on and off states at 1year. Frequency modulation with lower rates (80-100 hz) seem to reduce festination while higher frequencies (160-200Hz) show a slight improvement in freezing.

Conclusions: DBS of bilateral STN improve gait and postural instability markedly in off state while freezing episodes were reduced both in off and on states. Frequency modulation may further help in stabilizing gait.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

MO4/88: Clinical phenotype of patients with SCA 12

Aakash Shetty, Pettarusp M. Wadia, Aakash S. Shetty, Prochi Madan

Jaslok Hospital and Research Centre, Mumbai, Maharashtra, India

Background: Spinocerebellar ataxia is an autosomal dominant ataxia. (SCA12) is characterized by tremor of the upper extremities. SCA 12 is seen exclusively in Agarwal community in India. It is secondary to CAG repeat expansion on chromosome 5q31-33 that is located within the 5q region of PPP2R2B a gene encoding a brain-specific regulatory subunit of protein phosphatase 2A.

Objective: To Describe clinical phenotype and nature of tremors in patients with SCA12.

Material and Methods: Retrospective study of Neurologic features of twelve genetically confirmed symptomatic patients from ten families of SCA-12 presenting with tremors was conducted. All the patients were referred to the movement disorder clinic at our institute for tremors.

Results: Out of 146 patients with non-parkinsonian tremors referred to our clinic from 2008 till date we had total 12 genetically confirmed SCA12 patients forming 8% of the total referrals for tremors (other than parkinsonian tremors). Age at onset ranged from 37 years to 60 (mean of 46.75 years) out of which 7 were males and 5 females.11 patients had postural upper limb tremors which were asymmetrical in 3 patients. Head tremors seen in 3 patients and vocal tremors in 2. Cerebellar dysfunction in the form of ataxia was seen in 8/12 patients.

Conclusion: SCA-12 is a slowly progressive autosomal dominant neurodegenerative disorder that differs from other SCA in that it typically presents with postural limb tremors. Cerebellar dysfunction including gait ataxia is relatively nondisabling in majority of the patients and is slowly progressive. Patients presenting with postural tremors from the Agarwal community need to be screened for SCA 12 mutations.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

MO5/156: The Study of Pulmonary Function Testing in the Form of Spirometric Changes in Idiopathic Parkinson's Disease Patient

Susanta Kr. Das, Kalyan Brata Bhattacharjee, Ritabrata Mitra

Bangur Institute of Neurosciences Kolkata, West Bengal, India

Introduction: The spectrum of respiratory involvement in patient with PD has broadened to include restrictive defect obstructive defect abnormal control of ventilation and pulmonary sequelae attributed to drugs used to treat the disorder.

Aims and Objectives: This study is intended to find out the pattern of respiratory involvement by pulmonary function testing in the form of spirometric changes in Idiopathic Parkinson's Disease patient.

Materials and Methods: 1. Study Area “NeuromedicineDeptt.” BangurInstitute of Neurosciences Kolkata. 2. Study Population- Diagnosed case of Idiopathic Parkinson's disease patient.3. STUDY PERIOD-February 2014- June 2015.4. SAMPLE SIZE- Minimum of 50 Diagnosed case of IPD and 50 controls. Study Design- Case control study. Parameters to be studied: Demographic profiles Clinical stage FVC (forced vital capacity) % FEV1(Forced expiratory volume in one second) FEV1/FVC ratio

Results and Analysis: Fifty cases and 50 control were included. Among these 35 were male and 15 were female. Spirometry were performed in 45 cases and 50 control. Among cases 10 patients showed obstructive pattern 26- restrictive pattern 5 mixed result and 4 were normalin spirometry. Among control group only 8 showed normal.

Conclusion: In my present study restrictive air way disease is more common in spirometry.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

MO6/284: Prevalence of Quantitaive Sensory Abnormalities and Correlation with Autonomic Disturbances in Patients with Idiopathic Parkinsons Disease and ParkinsonsPlus Syndromes

Rupam Borgohain, Rukmini Mridula Kandadai, Murali Krishna, AfshanJabeen, Meena A Kanikannan

Nizams Institute of Medical Sciences, Hyderabad, Telangana, India

Objective: To identify and assess the correlation between quantitative sensory abnormalities and autonomic dysfunction in patients with idiopathic Parkinsons disease (PD) and Parkinson plus syndromes

Background: Autonomic disturbances are seen in Parkinson's disease (PD) and parkinson plus syndromes. But the contribution of peripheral component and presence of sensory abnormalities in these syndromes are not clearly known. As the sensory deficits are mild and are usually missed on routine nerve conduction studies a more sensitive sensory examination may be helpful.

Methods: Thirty-nine patients attending movement disorders clinic with diagnosis of PD, progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) were included in the study. Patients with other causes of autonomic dysfunction were excluded. Detailed history, neurological examination, electroneuromyogram, cardiovascular autonomic function testing (AFT) and computer assisted sensory examination (CASE IV) studies were evaluated in all. CASE IV studied three modalities -vibration detection threshold (VDT), cold detection threshold (CDT) and heat pain (HP). A value corresponding to greater than 95th percentile (based on normal age and sex matched controls) were considered to be hypoaesthetic and abnormal.

Results: Among 39 patients, PD were 15 (38.4%), PSP 12 (30.7%), MSA 12 (30.7%). All had normal nerve conduction response. PD (53.3%) and MSA-P (41.6%) had significantly higher abnormalities on CASE-IV compared to PSP patients (8.1%). Overall 36% of all patients had QST abnormalities. PD (53.3%) and MSA-P (41.6%) had significantly higher abnormalities on CASE-IV compared to PSP patients (8.1%) Abnormalities in cold detection threshold was maximum (25%), followed by vibration detection threshold (20%) and hot pain (15%). AFT abnormalities were seen in 75% of MSA, 46% of PD and 33.3% of PSP patients on comparison of QST and AFT, there was significant correlation with Pearson correlation coefficient of 0.42.

Conclusions: CASE -IV picked up sensory abnormalities in more than 50% of PD patients and 40% of patients of MSA-P. Impaired cold detection was the most common disorder. Autonomic dysfunction was also more common in MSA and PD compared to PSP. There was a significant correlation between the two abnormalities suggesting impairment in small fibres. Sent from Surface.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

Platform Session 08, Saturday, October 03, 2015, 08:30 AM- 09:30 AM Dr. K. S. Mani (Hall B): Miscellaneous Disorders: OO1/37: Neurological Manifestations in Children Exposed To Aerial Endosulfan Spraying In Northern Kerala

Shaji CV

Government TD Medical College, Alappuzha, Kerala, India

Introduction: Endosulfan is an organochlorine insecticide used in insect control. It was widely used for aerial spraying in the cashew plantations in northern Kerala for 20years. This study reports the various neurological manifestations among children in these areas born during those years.

Methods: 72 families were studied. The antenatal history natal and postnatal history was obtained for the children in the study population. Symptoms pertaining to neurological system were obtained. Various disabilities were assessed.

Results: Emotional disabilities were the most common observed feature(65.3%) followed by attention deficit disorders (61%) and speech defects(58%). Stroke was reported in (30.6%) and seizure in (12.5%).62.5% of children were dependent.

Conclusion: Among children living in the places where aerial spraying of endosulfan was done for a prolonged time the neurological deficits were significant. Whether this is a causal association remains to be proven.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

OO2/269: Clinico-Genetic Study of 96 FRDA Patients From North India

Achal Kumar Srivastava, Inder Singh, Mohammed Faruq, Sunil Shakya, Varun Surolia, Garima Shukla, Vinay Goyal, MV Padma Srivastava, MadhuriBehari

AIIMS, New Delhi, Delhi, India

Background: Friedreich Ataxia (FRDA) is a common recessive ataxia with features of variable occurrence of cardiomyopathy (60-90%) musculoskeletal deformities (60-80%) and diabetes mellitus (6-32%). It is caused by bialleleic loss of function mutations in FXN.

Objective: To study the clinico-genetic features of Indian FRDA patients in a large case series.

Material and Methods: 91 FRDA patients underwent standard neurological evaluation and other paraclinical investigations. GAA-expansion/mutation screening was carried out by Triplet-repeat-primed PCR followed by expand-long-template PCR kit (TaKaRa). The study was approved from institutional ethics committee.

Results: A total of 91(male: female ratio; 57:34) FRDA patients from 83 families were shown to have a mean size of the expanded GAA allele of 812 ± 213. Five patients were found heterozygous for GAA repeat and speculated to harbour point mutation on the other FXN copy. The observed age at examination disease onset and duration of the disease among all patients with mean (SD) were as following 20.6(7.3)14.0 (5.7)6.2(4.4) respectively. GAA-lower-allele showed inverse correlation with disease onset. Cerebellar ataxia peripheral neuropathy and musculoskeltal deformities were predominant features observed in >80% of cases. Cardiomyopathy was observed with 35% frequency and impaired blood glucose level was observed only in four patients (Figure-1). Patient related variables were found to determine occurrence of some of the neurological features. Occurrence of cardiomyopathy have shown no correlation with any variable (age at onset disease duration or GAA alleles) as reported earlier.

Conclusion: Indian FRDA patients show clinical variability with lower frequency of non-ataxia features cardiomyopathy and diabetes mellitus.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

OO3/70: Atypical Presentations of Spino Cerebellar Ataxia-12 (SCA-12)

Suman Kushwaha, Vinit Banga, Lomesh Bhirud, Aldrin Anthony

Institute of Human Behavior and Allied Sciences, Delhi, India

Objectives: Atypical presentations of Spino Cerebellar Ataxia-12.(SCA-12)

Background: SCA-12 can have many different presentations. The rapid onset of initial presentation of SCA-12 with decreased cognition asymmetrical Parkinsonism and blepharospasm is uncommon.

Methods: Detailed clinical history neurological examination neuroimaging and genetic testing. Two middle aged patient's one male and another female presented with atypical manifestations.

Results: Our first patient a 48-year-old male presented with asymmetric tremulousness bradykinesia and history suggestive of festination. He also gave history of forgetfulness for recent events. The second patient a 55-year-old female presented with 13-year history of tremulousness of limbs trunk and face preceded by blephorospam. Both the patients have positive family history. Expanded CAG Repeats confirmed the diagnosis.

Conclusion: SCA-12 usually presents as tremor predominant disease with other cerebellar signs and a slowly progressive course. Though other symptoms in the form of dystonia and Parkinsonism can be found they are less common and are usually symmetrical. Cognition is involved only later in the course. Our patients presented as asymmetrical Parkinsonism along with decreased cognition and blepharospasm with fairly rapid progression in one patient which has rarely been described.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

OO4/73: Out-Patient Burden of Neurological Disorders: Prospective evaluation of 1500 patients

Uday Shanker, Thomas Mathew, Arvind Prabhu, Raghunandhan Nadig, Alexander Manuel Lobo, Sagar Badachi, DelonD'souza, GRK Sarma

Introduction: Neurologists spend a significant time in providing out-patient services. Newly joined residents are least trained to manage most of the common out-patient neurological disorders.

Objectives: The aim of this study is to determine the incidence of various neurological disorders in the out-patient department and to identify those in which residents need maximum training.

Materials and Methods: The study was conducted in the Out- patient services of a tertiary care medical centre. Clinical data of 1500 consecutive out-patients was collected over a period of 2 months from December 2014 to January 2015. Appropriate statistical methods were used for descriptive analysis.

Results: The most common complaints with which patients came to the Out- patient services were headaches backache neck pain musculoskeletal pain seizures hemiparesis/monoparesis tingling tremor and giddiness. Headaches accounted for 28% of all the cases. Migraine was the commonest headache disorder (46.6%) followed by Tension Type Headache (26%). Backache neck pain and fibromyalgia were seen in 25% of all the cases. 20% of the migraineurs had concurrent fibromyalgia. Seizures accounted for 12.5% while strokes/TIA was present in12%. Neuropathies accounted for 7.6% of which most common was carpel tunnel syndrome. Movement disorders accounted for 6% most common being Parkinson disease. Vertigo was seen in 2.8% while Dementia and demyelinating disorders accounted for less than 2% each.

Conclusion: Most common disorders for which residents need elaborate training are headache backache fibromyalgia stroke epilepsy and neuropathies. Of these paramount importance should be given for management of Migraine TTH and Fibromyalgia.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

OO5/107: Mitochondrial Neurogastrointestinal Encephalomyopathy -MINGIE: Phenotypic Spectrum and The Need for Algorithm in the Indian Context

Mathew Alexander, Anna Oommen, Ajith Sivadasan, Karthick Muthusamy, Vivek Mathew, Maya Thomas, Anil Patil, Sanjith Aaron

Christian Medical College, Vellore, Tamil Nadu, India

Background: MNGIE is a rare AR disorder characterized by onset <30y with Ptosis PEO GI dysmotility cachexia peripheral neuropathy and leucoencephalopathy. This disease is caused by the TYMP gene encoding Thymidine Phosphorylase. In the Indian context genetic testing may not be feasible and in house development of TPP activity from Buffy coat will help clinch diagnosis and initiate Rx in this potentially fatal disorder.

Design: Retrospective (2008-2015)

Methods: We reviewed the medical records of patients who fulfilled the Hirano Criteria and had TPP enzyme activity in leucocytes done by spectrophotometric analysis and compared with age matched controls (< 10%).

Results: 10 patients over a 8 year period/ Mean age was 24 (M7F3) FH positive in 50%. The phenotypic spectrum was wide 60% had GI symptom 80% weight loss 50% intestinal pseudoobstruction 30% failure to thrive 30% PEO Ptosis 30% and facial weakness in 60%. 60% had a Peripheral Neuropathy presentation, 70% Demyelinating neuropathy, 10% had Diaphragmatic weakness and 60% leucoencephalopathy. The problems of genetic tests in the developing world context and developing in house testing of TPP is stressed and the various treatment options like Dialysis Platelet transfusions and Stem Cell therapy are discussed. There was misdiagnosis as CIDP HMSN MLD and Amyloidosis. Intercurrent infections could worsen this Paroxysmal Disorder and treated; we discuss our treatment experience with Dialysis (CAPD) which has been life saving.

Conclusions: The need for high index of suspicion development of in-house testing disease based algorithms are important for this rare but treatable metabolic disorder.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

OO6/211: Neurological Manifestations in Dengue Seropositive Patients

Navneet Kumar, Gaurav Gupta, Atul Garg, Kanhaiya Agrawal

Ganesh Shankar Vidyarthi Memorial Medical College, Kanpur, Uttar Pradesh, India

Objective: To study the incidence and spectrum of neurological manifestations in dengue seropositive patients.

Background: Dengue is an infectious disease caused by a flavivirus. It is an acute febrile illness causing considerable morbidity and mortality. The neurological complications in dengue has been hypothesized through three different pathogenic mechanisms: (1) direct neurotropic effects leading to encephalitis meningitis myelitis and myositis (2) indirect effects due to metabolic complications resulting in encephalopathy and cerebrovascular complications due to thrombocytopenia and platelet dysfunction and (3) postinfectious immune-mediated acute disseminated encephalomyelitis Guillain Barre syndrome and optic neuritis.

Methods: This was a descriptive cross sectional study including seropositive patients diagnosed with Dengue fever (DF) Dengue with warning signs and severe dengue with neurological manifestations presenting to Medicine Department of LLR HospitalKanpur.

Results: 10 (2.6%) patients had neurological manifestations out of 383 seropositive patients. Out of ten nine patients were male and only one patient was female. Among them 10% patients come under category of classical dengue fever 10% patients suffered from dengue with warning signs and 80% with severe dengue.4 patients had encephalopathy3 other patients had encephalitis 2 patients presented with single episode of symptomatic generalized seizure and 1 patient presented as having an intra cranial hemorrhage.

Conclusions: Neurological manifestations of dengue are manifold and it is necessary to consider dengue as a cause for the above neurological presentations in endemic zones of the disease.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

Platform Session 09, Saturday, October 03, 2015, 08:30 AM - 09:30 AM Dr. M. P. Mehrotra (Hall C): Auto-immune Disorders: AO1/33: Beta-Interferon Therapy in Relapsing and Remitting Multiple Sclerosis: Challenges in an Emerging Country

Sarath Menon R, Suresh Kumar Radhakrishnan, Rajeswary Kalathil Padmajan, Praveen Kumar Yadav, Emmanuel James, Anandakuttan Anand Kumar

Introduction: Multiple Sclerosis (MS) is an immune-mediated demyelinating disease with an increasing prevalence in India as is seen in the recent studies. Beta-Interferon is the most 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 & also look at other factors such as affordability preference availability. Setting and Design-A retrospective-prospective design

Materials and Methods: Twenty patients with a diagnosis of RRMS included in the study. They were followed up regularly while on beta-interferon. All patients underwent magnetic resonance imaging (MRI) of the brain plain & contrast & the expanded disability status scale (EDSS) score was assessed at regular intervals. Statistical Analysis - Data analysis was done using the SPSS 15.0version.

Results: The mean age was 36.25+_12.24 yearswith a female:male ratio of 2.3:1. There was a significant delay from diagnosis to start of interferon therapythe mean duration being 29.95 months. Oligoclonal bands (OCB) were positive in 70% of the subjects. Seventeen patients were on interferon beta 1a intramuscular & three patients on interferon beta 1a subcutaneous injections. The mean relapse rates per patient before & after starting beta interferon were 1.85&0.55 respectively. First dose fever was the most common side effect. Headache myalgia & psychiatric symptoms were also seen. Periventricular and juxtacortical lesions were the most common radiological sites. Conclusion-Beta-interferon is an effective and safe therapy in Indian patients with RRMS. However very few patients have been able to continue it as a longterm therapy due to financial constraints.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

AO2/41: Relapsing Remitting Multiple Sclerosis in Bengaluru (India): a longitudinal observational study

Thomas Mathew, GRK Sharma, P Satishchandra, P Netravathi, Vikram Kamath, AK Roy, UdayMurgod, Murali S, RaghunandanNadig, Guruprasad

Introduction: There exists a lack of epidemiological data on Multiple Sclerosis (MS) in India. There is paucity of data on the various types of MS and treatment patterns across various centers.

Objectives: To study the demographic diagnostic and management patterns of patients with MS in Bengaluru.

Materials and Methods: This was a multicenter longitudinal observational study conducted to assess the characteristics and treatment patterns of MS. The patients who satisfied the inclusion criteria for MS were enrolled into the study. The physicians entered the collected data of the eligible patients on a standardized web portal.

Results: From April 2013 to December 2014 there were 104 MS patients from five major tertiary care hospitals in Bangalore. 75% were females (78/104). The mean age of the patients were 35.6 (SD ± 9.86) years. 69.2% of the total patients had RRMS 18.3% had CIS 7.69% had PPMS and 5.77% had SPMS. The mean duration of disease was 3.1 years. Coordination and balance problems was the most common symptom observed and was experienced by 78.8% of the patients followed by spasticity (77.8%) fatigue (70.1%) cognitive impairment (62.5%) bladder dysfunction (56.7%) and bowel dysfunction (7.6%). The mean duration of time between first documented relapse and the second documented relapse is 314.1 days. 62.5% of the enrolled patients had received non-conventional and other management therapies previously. Yoga was the commonest alternative therapy and was used for fatigue. Only 43.8% of total patients were on standard treatment for MS.

Conclusion: Multiple Sclerosis is not uncommon in Bengaluru India. Cognitive impairment appears to be common in MS patients. Majority of patients do not receive standard treatment for multiple sclerosis.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

AO3/79: Anti MOG Antibody associated disease is distinct from Anti AQP4-IgG associated NeuromyelitisOptica Spectrum disorders

Lekha Pandit, Sharik Mustafa, Chaithramalli, Anita D’Cunha, AkshathaSudhir

KS Hegde Medical CollegeNitte University, Mangalore, Karnataka

Background: Until recently the biomarker associated with NMOSD was anti aquoporin 4-IgG (Anti AQP4-IgG) which manifests with severe myelitis and or optic neuritis of relapsing nature. Anti myelin oligodendrocyte glycoprotein antibody (anti MOG-Ab) associated disorders have recently been characterized with distinct clinical presentation disease course and MRI features. We are reporting the first study from India.

Method: 125 consecutive patients fulfilling criteria for NMO/NMOSD were clinically characterized and biomarker assay done by fluorescence activated cell sorter (FACS) assay. Statistical analysis was done by Fisher exact test for categorical and Wilcoxon test for numerical data.

Results: 38patients (30.4%) were NMO-IgG positive while 25 cases (20%) were positive for anti MOG-Ab. Phenotypic differences were significant (p< 0.001). Typical NMO was seen in 10% of patients with MOG-Ab as compared to 84% of those with AQP4-IgG. Recurrent optic neuritis (44%) and monophasic myelitis(36%) dominated in the former. On MRI dorsal cord was involved more commonly in patients with MOG antibody and cervical cord in those with AQP4 IgG. In patients with anti MOG antibody male gender (p<0.001) was common mean relapse rate (p<0.005) mean EDSS (p<0.005) and visual functional score (p < 0.01) were significantly low in comparison with those with AQP4-IgG.

Conclusion: NMOSD with MOG antibody constitute a significant proportion of patients seronegative for antiAQP4 antibody. They are likely to be more benign with male predominance with monophasic / fewer attacks less severe visual involvement and better outcome when compared to those with AQP4 antibody associated disease.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

AO4/285: Steroid Responsive Encephalitis: In the Background of Acute Encephalitis

Bhoi SK, Kalita J, Misra UK

Sanjay Gandhi PGIMS, Lucknow,UP, India

Objective: To evaluate the clinical spectrum and outcome of patients with autoimmune encephalitisand contrast them with viral encephalitis patients.

Methods: Viral encephalitis wasdiagnosed on the basis of fever, altered sensorium with virological markers. Autoimmune encephalitis was diagnosed on the basis of clinical picture, steroid responsiveness and autoimmune markers in blood or CSF or both. Clinical information was obtained from a retrospective review of medical records of encephalitis patient where definite viral aetiology was negative were included. Thyroid peroxidise antibody (TPO), and other autoantibody (NMDA, VGKC and GAD antibodies) testing was done in selected patients. The clinical pointers differentiating viral andautoimmune encephalitis will be evaluated.

Results: There were 17patients of autoimmune encephalitis; whose median age was 48 (range 13-69) years and 8 (47%) were female. The median duration of illness was 30 (2-365) days and GCS was 12 (9-15). Behavioral abnormality was present in 7 (41.2%), movement disorder 8 (47.1%), seizure 11 (64.7%), focal neurological deficit in 6 (35.3%) patients. CSF was abnormal in 11 (67%), MRI was abnormal in 10 (59%). The clinical spectrum was Hashimoto's encephalopathy in 6, paraneoplastic limbic encephalitis in 2, autoimmune encephalopathy associated with rheumatoid arthritis and nephritic syndrome in one patient each. Clinical response to steroid was observed in 14 (82.4%) patients. One patient with malignancy died during hospital stay, 11 had good recovery 5 patients had moderate to severe disability. There were 120 cases with different viral encephalitides. The level of consciousness was less impaired, systemicmanifestations less common but movement disorder and hyponatremia more common in autoimmuneencephalitis

Conclusion: Autoimmune encephalitisisan uncommon condition, movement disorder, hyponatremia and associated endocrine abnormality and response to corticosteroid are suggestive.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

AO5/152: Clinical Experience of NatalizumabIn Multiple Sclerosis at A Tertiary Care Center in Delhi

Sitla Prasad Pathak, Brig SP Gorthi, Col PawanDhull

Background: Management of active relapsing-remitting multiple sclerosis (MS) continues to pose a therapeutic challenge. Natalizumab is a humanized monoclonal antibody that blocks trafficking of lymphocytes into the brain by alpha-4 integrin-mediated adhesion. Although it is generally considered to be particularly effective and well tolerated patients may develop JC virus antibodies allergic reactions PML.

Objective: A retrospective review was performed on all patients who received Natalizumab for treatment of RRMS from May 2012 to May 2015 at Army Hospital (Research & Referral) Delhi cantt.

Methods: A cohort of 11 MS patients receiving up to 32 monthly doses natalizumab (mean 14) was evaluated clinically by magnetic resonance imaging (MRI) and by laboratory monitoring.

Results: None of the patients had relapse disease progression PML or hypersensitivity while on treatment. Clinical status was better as assessed with mean EDSS change (mean EDSS before and after natalizumab treatment was 6.90 and 5.90 respectively) and MRI findings were essentially unchanged without any new active lesion. 4 patients (36.36%) discontinued treatment in view of JC virus antibody positivity.

Conclusion: Natalizumab was found to reduce the progression of disability and the occurrence of clinical relapse and suppresses the formation of lesions as visualized by MRI in patients with relapsing multiple sclerosis. Natalizumab mono therapy had good safety and tolerability profile. However continued assessments of long-term treatment with natalizumab will better define the safety profile of this effective therapy.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

AO6/241: Sleep Quality and Its Determinants in Women with Systemic Lupus Erythematosus

N. Arun Kumar, S. Fazal E Ilahi, V. Kannan, R.S.A. Alexander, S. Rajeswari, K. Bhanu

Institute of Neurology Madras Medical College, Chennai, India

Introduction: Difficulty in initiating sleep maintaining sleep and early morning awakening are components of sleep disturbances which are seen in rheumatologic conditions including Systemic Lupus Erythematosus. According to studies prevalence of sleep disturbances ranged between 65% and 75% and disease activity pain and depression are causal factors.

Objective: To determine the prevalence of poor sleep quality in women with SLE and its association with disease activity pain and depression.

Methodology: Female patients who attended Rheumatology OPD during April to June 2015 aged between 18 to 60 years who fulfilled the American College of Rheumatology criteria were included for study. Those who had other co-morbid illness were excluded. Sleep quality in 60 patients was assessed using Pittsburgh Sleep Quality Index (PQSI). Patients were also assessed for disease activity pain and depression using SLE Disease Activity Index (SLEDAI) Visual Analogue Scale (VAS) and Center for Epidemiological Study - Depression (CES-D) scale respectively.

Results: Among 60 patients 38(63%) had poor sleep quality(PQSI:5). 41(68%) had difficulty in falling asleep (p<0.005) 37(62%) had difficulty in maintaining sleep (p<0.05) 29(48%) had early morning awakening (P<0.05). Disease activity(SLEDAI >6) pain (VAS>5) and depression (CESD:16) individually contributes significantly to these various components of sleep disturbances.

Conclusion: A significant proportion of women with SLE suffer from poor sleep quality contributed by difficulty in falling asleep difficulty in maintaining sleep and early morning awakening. Disease activity pain and depression are the confounding factors for these sleep disturbances. By correcting these factors quality of sleep and thereby overall quality of life can be improved in patients with SLE.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

Platform Session 10, Saturday, October 03, 2015, 02:30 PM - 03:30 PM Dr. Baldev Singh Hall (Hall A): Epilepsy: EO7/139: Study of Frequency and Characteristics of Reversible Periictal MRI Abnormalities

Jogu Siva Kumar, Afshan Jabeen, PavanCherukuri, RupamBorgohain, A K Meena, Jyothsna Rani, Rukmini Mridula

Nizams Institute of Medical Sciences, Hyderabad, Telangana, India

Background: Reversible periictal magnetic resonance imaging abnormalities (RPMA) is a well described finding seen in patients following seizures. Most previous studies are retrospective hence the precise incidence and factors responsible for development of MRI changes is not well studied. We did a prospective study of MRI abnormalities focusing on diffusion weighted imaging in patients with cluster of seizures and status epilepticus (convulsive and non-convulsive).

Objective: To study the frequency imaging characteristics and clinical predictors for development of RPMA

Methods: We prospectively analyzed electroclinical and imaging characteristic of adult patients with cluster of seizures or status epilepticus and in whom the imaging was done within 48 hrs after the end of last seizure (clinical or electrographic)

Results: There were thirty patients who fulfilled the inclusion and exclusion criteria. Seventeen patients (56.7%) had RPMA following seizures. Nine patients (30%) did not have any MRI abnormality. All the patients with RPMA had abnormality on diffusion weighted imaging. Hippocampal abnormalities were seen in nine (53 %) perisylvian in two (11.7%) thalamic in five (30 %) splenium involvement in two (11.7%) and cortical involvement in the form of temporo-occipital parieto-occipital temporo-parietal fronto-parietal fronto-temporal is seen in sixteen (94.1%) patients. Complete reversal of MRI changes was noted in eleven (66.6%) patients and six (33.3%) patients showed partial resolution of MRI abnormalities.

Conclusions: MRI showed areas of abnormal diffusion that were highly concordant with the clinical semiology and with the EEG activity. The patients with longer duration of seizures/ status were more likely to have RPMA.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

EO8/141: Study on Prevalance of Migraine in Epilepsy

Preetha, Shunmuga Sundaram. K, Sarala. G, Bhanu. K

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

Introduction: Migraine is a common disabling primary headache disorder. In global burden of disease survey it was ranked as the third most prevalent disorder. Migraine has two subtypes 1.1 migraine without aura is a clinical syndrome characterized by headache with specific features and associated symptoms. 1.2 migraines with aura is primarily characterized by the transient focal neurological symptoms that usually precede or sometimes accompany the headache.

Objectives: The purpose of the study is intended to analyse the frequency of migraine related headaches in epilepsy patients.

Methods: The study was conducted in madras medical college institute of neurology between January 2015 to May 2015. 708 patients with epilepsy were recruited at our outpatient epilepsy clinic. All patients were diagnosed using the criteria of international classification of headache disorders.

Results: Of the 708 patients 203 patients had seizure associated headache of which 84 (41.3%) had migraine type of headache of which 74(88.1%) had migraine without aura 10(11.9%) had migraine with aura Of 203 patients 114(56.15%) were women and 89(43.84%) were men. The type of seizure headache does not correlate with the epilepsy syndrome.

Conclusion: High prevalence of migraine related headache was common in this study population. These data explains the prevalence of migraine in epileptic population.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

EO9/144: Clinical profile of patients presenting with elevated Serum Phenytoin levels in a tertiary care centre

Kaushik Sundar, U. Meenakshi Sundaram, C. U. Velmurugendran, V. Shankar

Sri Ramachandra University, Chennai, Tamil Nadu, India

Aim: To study the profile of patients who present with biochemically proven high serum phenytoin levels in a tertiary care centre.

Materials and Methods: Inclusion Criteria-Patients of any age group presenting within the last 1 year with high values of serum phenytoin levels were included in this study. Exclusion Criteria-Patients on multiple anti epileptic drugs were not included.

Results and Discussion: A total of 33 patients were included in this study. There was a male preponderance. There was evidence of phenytoin toxicity in all age groups;however it was more often seen between 11-30 yrs of age. For study purposes patients were divided into 2 groups-acute phenytoin toxicity wherein the patients had taken large doses of phenytoin tablets accidentally or with suicidal tendencies and chronic toxicity wherein patients who were taking phenytoin regularly as a part of their anti epileptic drug therapy. Serum phenytoin levels were done within the first 24 hours of admission. Patients with acute phenytoin toxicity always presented with altered sensorium whereas in chronic toxicity the most common presentation was instability/giddiness. The most common clinical sign that was seen in acute toxicity was low GCS whereas in chronic toxicityny stagmus was common. Cerebellar signs were seen only in patients with chronic toxicity. The biochemical levels of serum phenytoin had no correlation with the type of clinical features both in acute and chronic toxicity.

Conclusion: Phenytoin toxicity was seen most often in iatrogenic arm rather than accidental/suicidal poisoning. Acute phenytoin toxicity presented with altered sensorium. Chronic toxicity presented with gait in stability and giddiness. The most common clinical sign of chronic toxicity was Nystagmus. Cerebellar signs were only seen in patients with chronic toxicity. Serum phenytoin levels (both in acute and chronic) did not correlate with the type of clinical signs and symptoms.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

EO10/208: Frequency of SCN1A IVS5-91 G>A (rs3812718) and CYP2C9*3 1075 A>C (Ile 359 Leu Exon-7) gene polymorphisms in south Indian Epilepsy Population

Amareshwer Rao K, Dhairyawan Pokalkar, Veena N, Sateesh K, Sowjanya P, Srilatha S, Santhosh Kumar B, Manjusha M, Chandrashekhar P

Gandhi Medical College, Hyderabad, Telangana, India

Introduction: Phenytoin and carbamazepine both target Na Channels in brain for their Anticonvulsant action. Phenytoin and Carbamzepine are metabolized in liver by Cytochrome P450 Enzymes. Mutations in the genes for target channels have been shown to cause resistance and requirement for higher dose of AEDs. Metabolising enzyme variants have been shown to cause toxicity and adverse reactions.

Objective: To find the Frequency of Gene polymorphisms rs3812718 (G>A) in SCN1A Channel and 1075 (A>C) in CYP2C9*3.

Material and Methods: A total of 100 Epileptic Patients attending the Neurology OPD of Gandhi General Hospital were taken into the study. Patients consent for inclusion in the study and genotyping was taken according to the standard protocols. Genotyping for the SCN1A mutation was done using ARMS-PCR method. Genotyping for CYP2C9*3 was done using PCR-RFLP method.

Results: PCR results for SCN1A gene showed 39 out of 100 patients had Normal (G/G) Alleles. 56 patients showed heterozygosity (G/A) with mutation in one allele and 5 patients showed homozygosity (A/A) with mutation in both alleles. PCR results for CYP2C9*3 gene showed heterozygosity (A/C) with mutation in one allele in 5 patients remaining patients had normal Alleles.

Discussion: Previous studies for SCN1A mutation were done in European populations. The study done by Le Gal et al for SCN1A showed 26% had homozygosity for mutant allele and 54% showed heterozygosity. A similar study by Schachter et al in European population showed similar results. Our study in South Indian populaion showed heterozygosity in 56 % and homozygosity in 5%.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

EO11/243: Case Series of Jeavon's Syndrome

Karthikeyan S, U. Meenakshisundaram, C. U. Velmurugendran, V. Shankar

Sri Ramachandra University, Chennai, Tamil Nadu, India

Introduction: Jeavon's syndrome is an epileptic syndrome characterized by (1) Eyelid myoclonus with or without absences.(2)Eye closure induced seizures.(3)EEG with brief generalized polyspikes wave discharges at 3-6Hz.(4)Photosensitivity.

Aim: To explore the clinical manifestation Electroencephalograph characteristics and therapeutic Effects of drugs in patient with Jeavons syndrome and to determine if it represents a well defined. Epileptic syndrome or a non specific condition associated with other Epilepsies.

Results: All our patients fulfilled the criteria for Jeavons syndrome. Among 5 patients 3 were male and 2 were females. The typical clinical manifestation of this disease was brief fast and repeated. Eyelid myoclonus with or without absence seizures eye closure induced seizures the typical Electroencephalography showed brief generalized polyspikes wave discharges Enhanced on eye closure and Photic stimulation and all 5 patients had history of GTCS. 4 patients were treated with Sodium valporate as monotheraphy and 1 patient with Sodium valproate &Levetiracetam. Seizures were controlled in all patients. The Neurological examination & Neuroimaging were normal in all cases.

Conclusion: Jeavonassyndrome is one of the idiopathic and generalized Epileptic syndromes and characterized by Eyelid myoclonus with or without absence seizures Eye closure induced seizures with photosensitivity. EEG plays an important role in diagnosing Jeavona syndrome. Sodium valproate &Levetiracetam were effective in controlling seizures in Jeavona syndrome.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

EO12/64: Infantile Spasms: A prognostic Evaluation

Mary Iype, Geetha S, PA Mohammed Kunju, Devi Mohan, MKC Nair, Babu George

Government Medical College, Trivandrum, Kerala, India

Purpose: We aimed to follow up children with infantile spasms to study; a) Theetiology demographics semiology the EEG and radiological pattern. b) Seizure control psychomotor development and EEG resolution with treatment. c) The effects of various factors on control of spasms resolution of EEG changes and psychomotor development at 3 year follow up.

Methods: Fifty newly diagnosed cases with a 1-12 month age of onset and who had hypsarrhythmia in their EEG were recruited and 43 were followed up for 3years.

Results: Of the children followed up 51% were seizure free and 37% had a normal EEG at 3 year follow up. Autistic features were seen in 74%. Only 22.7% among the seizure free (11.6% of the total) had normal vision and hearing speech with narration writing skills gross and fine motor development and no autism or hyperactivity. On multivariate analysis two factors could predict bad seizure outcome - the occurrence of other seizures in addition to infantile spasms and no response to 28 days of ACTH. No predictor could be identified for abnormal psychomotor development.

Conclusion: In our study we could demonstrate two factors that predict seizure freedom. The cognitive outcome and seizure control in this group of children is comparable to the existingliterature. However the cognitive outcome revealed by our study and the survey of literature is discouraging.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

Platform Session 11, Saturday, October 03, 2015, 02:30 PM - 03:30 PM Dr. K. S. Mani Hall (Hall B): Stroke: SO7/98: Clinical Profile Severity Prognosis and Comparison of Clinical Impression with Wab Classification of Post-Stroke Aphasia in Indians

Aju Abraham John, Mahendra Javali, Rohan Mahale, Anish Mehta, Kiran Buddharaju, Abhinandan K.S., Srinivasa R

M.S. Ramaiah Medical College, Bangalore, Karnataka, India

Background: Speech disturbance is a common symptom of stroke a prompt identifier of the event and can cause devastating cognitive impairments. Relations between aphasia severity aphasia type lesion location and prognosis could be helpful for treatment as who should undergo more aggressive interventions. It can also access prognosis and help in rehabilitation planning.

Aim: To study the clinical profile and prognosis of aphasia in acute stroke. To determine if bedside clinical impression can reliably predict Western Aphasia Battery classification of aphasia.

Methods: 55 consecutive cases of acute ischemic stroke and aphasia were included in this study. A detailed clinical evaluation handedness by Edinburg Handedness Inventory and Western Aphasia Battery scoring at admission and discharge was done.

Results: 53 of 55 cases had a left hemispheric infarct and all were right handed individuals. 2 cases had crossed aphasia. The frequencies of the different types of aphasia in acute ischemic stroke were: global 36.4% Broca's 25.5% Wernicke's 16.4% transcortical motor 1.8% transcortical sensory 3.6% conduction 1.8% anomic 14.5% and isolation 0%. Significant recovery was seen in more than 50% of patients at 1 week. There was an agreement of 67.3% between patient's bedside clinical impression and WAB classification of aphasia with a p value of less than 0.001.

Conclusion: Clinical impression was fairly reliable as compared to WAB in assessing aphasia. Aphasia quotient may be used for prognostication and select patients for more aggressive management which may reduce the mortality and morbidity in patients presenting with acute stroke.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

SO8/17: The effect of Citicoline in Stroke: A Comparative Study from Eastern part of India

Saikat Ghosh, Kartik Chandra Ghosh, Ramesh Bhattacharyya, Gouranga Prosad Mondal

Calcutta National Medical College, Kolkata, West Bengal, India

Background: Citicoline is a novel neuroprotective agent used in acute stroke with significant favourable outcome.

Method: 100 patients of both ischemic and hemorrhagic stroke presenting to the hospital within 48 hours of stroke onset were enrolled for the study. Out of these 100 patients (age- and sex-matched) 50 patients were treated with citicoline along with standard stroke management and taken as cases. Other 50 patients with standard stroke treatment were taken as control. All the patients were studied at the baseline with National Institute of Health Stroke Scale (NIHSS). The patients were followed at the end of first month and third month of the commencement of the therapy and the outcome was assessed by Barthel Index Score (BI).

Results: Mean BI scores of all categories at 1st month and at 3rd month were significantly higher in Citicoline treatment group (p<0.001 at 1st month and p= 0.002 at 3rd month). In subgroup analysis both ischemic and hemorrhagic stroke patients (all categories of BI score) in Citicoline treatment group showed significant higher mean BI score at 1st month (Ischemic: p=0.003 Hemorrhagic: p=0.04) and also at the end of 3rd month (Ischemic: p=0.03 Hemorrhagic: p=0.03).

Conclusion: Treatment with citicoline in patients of stroke presenting within 48 hours of onset increases the probability of complete recovery and favorable outcome at 1st month and at the end of 3rd month in all stroke groups.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

SO9/24: Is Two Really Better than One? Examining the Superiority of Dual Modality Neurophysiological Monitoring during Carotid Endarterectomy: A Meta-Analysis

Parthasarathy D. Thirumala, KarthyThiagarajan, Hannah L. Cheng, Jessie Huang, Piruthiviraj Natarajan, Donald J. Crammond, Jeffrey R. Balzer

University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA

Background: Periprocedural stroke after carotid endarterectomy increases long term mortality. Intraoperative monitoring with electroencephalography and somatosensory evoked potentials helps predict perioperative stroke risk. However the sensitivity of each technique when used independently still remains low.

Aim: To determine if multimodal monitoring leads to an increase in sensitivity and diagnostic accuracy.

Methods: Relevant literature was obtained through a search of Embase PubMed and Web of Science databases. Data was extracted and pooled estimates of sensitivity specificity and diagnostic odds ratio were obtained for single and multi modality neurophysiological monitoring. A McNemar test was used to evaluate for any statistically significant differences in the sensitivities and false positive rates.

Results: The diagnostic odds ratio of dual modality monitoring was found to be 17.4. The specificity of concurrent EEG and SSEP changes in predicting perioperative strokes was calculated to be 96.8% (95% CI 94.1-98.3%). The sensitivity of combined monitoring with a change in either modality designated as significant was 58.9% (95% CI 41.2-74.7%). Multi modality monitoring with a change in either EEG or SSEP as the alarm criteria was 1.32 times more sensitive than EEG alone and 1.26 times more sensitive than SSEP alone.

Conclusion: The odds of having a change in either EEG or SSEP are 17 times more in patients with perioperative strokes. Dual modality monitoring is more sensitive at predicting perioperative deficits than EEG or SSEP used independently.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

SO10/164: Hemodynamic Assessment by Transcranial Doppler in Acute Ischaemic Stroke

Gopisrikanth Matta, Pradeep Kakarala, T.Sateesh Kumar, S.Gopi, G.Butchi Raju

Andhra Medical College, King George Hospital, Visakhapatnam, Andhra Pradesh, India

Background: Stroke is the second most common cause of death and fourth leading cause of disability worldwide. Transcranial Doppler (TCD) is a non-invasive real-time bedside portable modality of investigation to look into the intra cerebral blood flow parameters for evaluation in stroke patients.

Aim of the study: To study the Intracerebral Blood Flow Velocities of 9 intracranial arteries (2MCAs 2ACAs 2PCAs 2VAs & BA) in Acute Ischemic Stroke using TCD and analyze the status of extracranial vessels using Neck Vessel Doppler. To correlate the TCD findings of the individuals with CT Angiography (CTA)/ MR Angiography (MRA) brain findings.

Materials and methods: All Patients aged 18-85 years with the diagnosis of ischemic stroke confirmed by CT or MRI scan admitted within 5 days of symptom onset were included. TCD was performed on the day of admission and in a sub group of 25 patientsfollowup TCD was performed after 7 days of admission. TCD was performed on SONARA/tek Portable Transcranial Doppler System. Each vessel was studied for mean flow velocity (MFV) Peak systolic velocity (PSV) end diastolic velocity (EDV) and pulsatility index (PI).

Results: In our study 55 cases were analyzed according to demographic characteristics risk factors of stroke investigative profile stroke subtypes according to TOAST criteria the status of extra cranial and intracranial vessels based on imaging findings and TCD. TCD flow parameters have increased in the involved vessel when compared to the uninvolved counterpart.

Conclusions: Hemodynamic parameters obtained on TCD from patients of acute ischemic stroke showed a significant rise in the clinically involved vessel. TCD flow parameters alone would be less informative for both diagnosis and prognosticationit has to be combined with CTA/MRA neck vessel Doppler and 2D-Echo as early as possible from the onset of stroke for planning suitable management strategies.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

SO11/186: Prevalence of Moderate to Severe Obstructive Sleep Apnea among Patients with Ischemic v/s Hemorrhagic stroke

Anupama Gupta, Garima Shukla, Mohammed Afsar, Shivani Poornima, Vinay Goyal, Madhuri Behari

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

Introduction: Stroke is common and a leading cause of death and obstructive sleep apnea hypopnea syndrome (OSA) is independent risk factor for stroke. Many studies have shown that OSA is clinically significant after ischemic stroke and it produces early neurological deterioration poor outcome and mortality. In hemorrhagic strokes the apnea frequency decreases with clinical improvement; but it remains as frequent as in the acute phase following ischemic stroke. In very initial studies it was shown that the ischemic stroke is more common in sleep in comparison with hemorrhagic stroke. We carried out this study with the aim of evaluating prevalence of OSA and other clinical and sleep characteristics of patients with ischemic v/s those with hemorrhagic stroke.

Materials and Methods: All patients presenting to a single unit of the Department of Neurology All India Institute of Medical Sciences New Delhi with history suggestive of first arterial stroke (group1= ischemic stroke and group2=hemorrhagic stroke) within 6 weeks from ictus with CT Scan or MRI evidence of the same were enrolled in the study. All patients were interviewed for history suggestive of sleep disordered breathing prior to stroke and risk factors and then underwent polysomnography (PSG).

Results: Out 679 consecutive stroke patients who were screened 346 gave consent for clinical sleep evaluation and PSG. Of these 207(172M) had suffered from Ischemic stroke and 139(107M) had hemorrhagic stroke. Mean age of patients in group1 v/s group2 was 53.24 ±12.64 vs. 55.45±11.39 (p=0.39). Out of 346 only 114 patients turned up for PSG. Overall prevalence of OSA was 95% in group 1 v/s 89% in group 2 (p=0.21) while that of moderate to severe sleep apnea in group1 v/s group 2 was 83% v/s 59% (p=0.004).

Conclusion: OSA is highly prevalent in both ischemic as well as hemorrhagic stroke but the proportion of moderate to severe sleep apnea is much higher among patients with ischemic stroke.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

SO12/197: Prognostic Significance of Admission Hyperglycemia and Early Glycemic Control in Spontaneous Intracerebral Hemorrhage

Sinchu C Maniangatt, Soumya Sundaram, Sapna Erat Sreedharan, Sankara Sarma P, Sylaja P N

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

Background: Admission hyperglycemia is known to be associated with worse outcome in patients with acute intracerebral haemorrhage (ICH). There is controversy regarding whether early glycemic control is associated with better outcome at 3 months.

Aim: To determine whether admission hyperglycemia portends worse outcome and optimal control of early hyperglycemia leads to improved outcome in ICH.

Methods: All cases of ICH diagnosed by CT scan presenting within 48 hours of symptom onset (August 2010 -March 2015) were included. Mean daily blood glucose for the initial 7 days was calculated and optimal control of hyperglycemia was defined as average of 7 day blood glucose ≤120 mg%. Significant haematoma expansion was defined as > 12.5 ml increase or >33% increase in haematoma volume in the second scan done 24 hours later. The functional outcome was assessed by modified Rankin scale (mRS) at 3 months.

Results: We evaluated 128 patients (Male -75% Mean age- 57.5±13.41 years). Mean NIHSS at admission was 13.5±7.5. Mean volume of haematoma at admission was 29.6 mL and 24 (18.8%) patients had significant expansion of haematoma. Intraventricular extension was seen in 50 (39.1 %) patients and the mean ICH score was 1.32±1.25. Mean mRS was 3.3 at follow up and 3 month mortality was 24.4% in the whole cohort. Admission hyperglycemia was not associated with poor functional outcome at 3 months (p =0.2). Tight control of hyperglycemia was associated with significantly less haematoma expansion (p =0.05) low rates of intraventricular extension (p=0.009) and lower ICH score (p=0.04).

Conclusions: Optimal control of hyperglycemia in ICH is associated with lesser haematoma expansion lesser intraventricular extension and lower ICH score thus indicating a better outcome after ICH.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

Platform Session 12, Saturday, October 03, 2015, 02:30 PM- 03:30 PM Dr. M. P. Mehrotra Hall (Hall C): Clinical Neurophysiology: CO1/75: Myasthenia gravis: a hospital based study from South India

Parvathy G, Shaji CV, Suraj MM, Prasanth SR

Govt. TDMC, Alappuzha, Kerala, India

Introduction: Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction characterized by skeletal muscle weakness and fatiguability. We present the findings from a prospective hospital-based study on clinical profile of myasthenia gravis from South India using data collected over a period of 2 years.

Objectives: To study the clinical presentation age at onset gender distribution Ach receptor antibody status and thymic abnormalities in patients with myasthenia gravis.

Materials and Methods: All patients presenting with myasthenia gravis during the period September 2013 to March 2015 were included. Data including age at onset sex distribution presenting symptom were collected. Bedside fatiguability test icepack test neostigmine test RNS test and CT thorax were done in all patients. Acetyl choline receptor antibody assay was done whenever feasible.

Results: 34 patients studied 15 were males and 19 were females. The median age at onset was 44years (males 27 years and females 48 years). 23 (67.6%) patients had ocular and 11 (32.4%) had generalized myasthenia. Serological studies were done in 14 patients for Acetylcholine receptor (AchR) antibodies of which 9 (64.3%) were positive. CT thorax revealed thymoma in 1(2.9%) patient and thymic hyperplasia in 4(11.7%).

Conclusion: The gender distribution and seropositivity rate for Ach receptor antibody were comparable with previous studies. However the proportion of patients presenting with generalized myasthenia and the prevalence of thymic abnormalities were lower compared to previous hospital based studies. Whether the coastal areas and the cholinesterase inhibitors from seaweeds exert a protective effect and alter the epidemiology is yet to be studied.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

CO2/85: Absent REM sleep in SCA 2; Early pontine degeneration? / slow saccades?

Seshagiri D V, Ravi Yadav, Arun Sasidharan, Pramod Kumar Pal, Bindu Kutty M, Sanjeev Jain

NIMHANS, Bengaluru, Karnataka, India

Introduction: Spinocerebellar ataxia type 2 (SCA 2) is an Autosomal dominant Neurodegenerative disorder. These patients will have progressive degeneration of cerebellum and brainstem structures. Early pons involvement and slow saccades is a feature of SCA 2.

Objective: To identify and to characterize the sleep disturbances in patients with genetically proven spinocere bellar ataxias type 2.

Methods: Patient with progressive ataxia were identified and assessed clinically. Disease severity measured with International Ataxia Rating Scale (ICARS) scale. Sleep quality was assessed with Pittsburgh Sleep Quality Index (PSQI) Mayo Sleep Questionnaire (MSQ) Epworth Sleepiness Scale (ESS). Genetically confirmed cases underwent overnight polysomnography (PSG) using a 40 channel Polysomnography system.

Results: Patieonts (n=10 5 males) were recruited. Patient details (Mean  SD): Age (3510.33); ICARS (30.5  13); PSQI (3.7  2.9); ESS (2.8  2.13). Most of the patients reported good sleep. None of them reported history of REM Sleep Behaviour Disorder Restless Leg Syndrome or Excessive Daytime Sleepiness. Polysomnography findings — Sleep efficiency 79.4 13.5%; N1%=1510.7; N2%=62.211; N3%=2010.33; REM%=2.57.3; REM Sleep was absent in 80%(8) of patients Significant finding in this study is absent REM sleep in most of the patients even though they had good sleep efficiency and had no sleep complaints.

Conclusion: Brain stem pathologies and sleep abnormalities have been reported in SCA patients. Absent REM sleep may be due to involvement of REM sleep generators in the brain stem or these patients may not able to generate the rapid eye movements because of slow saccades. Further studies are required to draw conclusion.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

CO3/265: Beta-Band Intermuscular Coherence: A Novel Biomarker Of Upper Motor Neuron Dysfunction In ALS

Hrishikesh Kumar, Stephan R Jaiser, Michael Mackay, Banashree Mondal, Marium Umme Kulsum, Rebecca Banerjee, Sidharth S Anand, Mark Baker

Institute of Neurosciences, Kolkata, West Bengal, India

Determination of Lower Motor Neuron (LMN) dysfunction by routine electromyogram (EMG) is relatively easy in patients with Amyotrophic Sclerosis (ALS). But Electrophysiological tools measuring the Upper Motor Neuron (UMN) dysfunction in ALS are scarce and inadequate. We hypothesize that observation of Beta-band intermuscular coherence (BIMC) can be a useful potential biomarker marker of upper motor neuron (UMN) dysfunction in ALS. In this effect there are some published evidences.12 We present data collected from 30 patients with ALS attending the outpatient at Institute of Neuroscience Kolkata. Lower limb BIMC was measured between a calf muscle and an intrinsic foot muscle during an unrestrained ankle dorsiflexion task. Upper limb BIMC was similarly estimated between a forearm muscle and an intrinsic hand muscle during performance of an auxotonic precision grip task. Average BIMC was computed for each limb and subject. Control data was also collected from 30 healthy volunteers (age 20-50). The probability distributions of ALS and control data were modelled by variable kernel density estimates and these estimates used to construct receiver operator characteristic (ROC) curves. Analysis of these data showed that cumulative probability distributions of BIMC in ALS were similar in shape to that in healthy controls but shifted towards lower BIMC values and area under the ROC curve was approximately 75% for each limb. BIMC represents an easily tolerated and inexpensive method for electrophysiological assessment of UMN disease in patients with ALS.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

CO4/111: Patterns of Neuropathy in Sjogren's Syndrome

Sandeep Gampa, Meena A.K, Rupamborgohain, S.A. Jabeen, Rukmini Mridula

Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Sjogren's syndrome is a systemic autoimmune disease that apart from exocrine glands may affect any organ or system. Involvement of different sections of the peripheral nervous system results in a wide spectrum of neuropathic manifestations. We evaluated clinical electrophysiological patterns and pathological characteristics of 14 patients with SS associated neuropathy (11 females 4 males). Majority of patients with SS associated neuropathy have primary SS (n=9) and had neuropathy (n=11) as an initial manifestation of the SS 3 had exocrinopathy preceding neuropathy & 5 had only neuropathy without other systems being effected. We had observed seven patterns of neuropathy in our cohort viz. multiple mononeuropathy(n=6) length dependent polyneuropathy (n=4) ganglionopathy (n=3)radiculoneuropathy (n=1) trigeminal/cranial neuropathy(n=1) small fibre neuropathy (n=1) autonomic neuropathy (n=1). Onset in majority was insidious (n=9) & rest it was sudden (n=5). Progression in the most of patients was gradual (n=10) & others had stepwise (n=1) relapsing and remitting (n=1) and static (n=1). Serology showed both Anti Ro & La positive (n=7) only Anti Ra positive (n=2) only Anti La positive (n=1) both Anti Ro & La negative(n=2). Histopathologic features showed evidence of definitive vasculitis (n=3) possible vasculitis (n=6) chronic axonopathy (n=5) demyelinating (n=2). one patient in addition to neuropathy had myopathy. The present study describes various patterns of neuropathy associated with SS & their associated serologic and histopathologic features. Screening for SS should be performed thoroughly in various forms of neuropathy or cranial nerve (especially trigeminal) involvement. High index of suspicion is required in absence of systemic and serologic evidence to diagnose SS associated neuropathy.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

CO5/115: Electromyography Guided Botulinum Toxin Injection in Newly Diagnosed Writer's Cramp Patients

Sanjay Pandey, Neelav Sarma

GB Pant Institute, Delhi, India

Objective: Detailed evaluation of newly diagnosed writer's cramp patients and their response to first dosage of electromyography (EMG) guided Botulinum toxin (BTX) injection.

Background: Writer's cramp is a type of task specific focal hand dystonia. Uses of BTX have significantly improved the treatment of writer's cramp patients.

Methods: We did a retrospective evaluation of 22 newly diagnosed writer's cramp patients who presented to our movement disorder clinic over a period of 18 months. EMG guided BOTX A injection was given to all patients and dosage was decided as per the clinical evaluation (kept at lowest considering the first injection). Benefit was assessed on a subjective scale from 0 to 4 based on percent restoration of normal function. Post injection weakness was defined as at least 25% reduction in normal strength (patients self-assessed weakness) following the previous injection.

Results: 90% of the newly diagnosed writer's cramp patients were male and task specificity waspresent in 81% patients. In 2/3rd (63.63%) patients more than 3 fingers (generalized) were involved. Mirror dystonia in 72% and dystonic tremor was present in 27% patients. Flexor digitorum superficialis and flexor pollicis longus were most common muscles injected. 68% patients had benefit with BTX injection and 22% had post injection weakness which improved rapidly. 72% patients were willing for second injection. Dosages required by our patients were small considering their first injection.

Discussion: Writer's cramp is the only focal dystonia to be more common in males but this bias is more striking in Indian patients (90% in this study). Mirror dystonia was seen in 72% of the patients which is more than reported (35-50%) in the literature. We used small dosages for the injection but still the response rate was 68% which is very good. The possible reason may be better muscle localization due to use of electromyography.

Ann Indian Acad Neurol. 2015 Sep;18(Suppl 2):S18–S40.

CO6/289: Effect of Magnesium Supplementation on Glycemic Control and Electrophysiological Parameters in Diabetic Patients with Neuropathy

P K Maheshwari, M. Kumar, A. Pandey, M. Bansal, A. K. Singh

S N Medical College, Agra, UP, India

Introduction: Diabetes is the leading known cause of neuropathy in the world. So far most of the clinical and diagnostic studies on diabetic neuropathy have concerned only peripheral and autonomic nerve but recently with the refinement of evoked potential techniques detailed exploration of sensory pathway in central nervous system has been possible.

Aim of Study: To study the effect of magnesium supplementation on glycemic control and electrophysiological parameters (brainstem auditory evoked response) in diabetic patients with neuropathy.

Material and Methods: 120 cases of diabetic neuropathy patients were taken in this study which was again divided in two sub groups. Group I consists of diabetic neuropathy patients receiving metformin, gabapentin and Magnesium supplementation (n=60) and group II consists of diabetic neuropathy patients receiving metformin and gabapentin only (n=60). Symptoms regression, blood glucose level, effect on blood pressure and latencies of BAER and total outcome were monitored.

Results and Conclusion: Magnesium deficiency is significantly associated in patients of diabetic polyneuropathy. Magnesium supplementation significantly improves the fasting blood glucose level. Results for improvement in latency of BAER of both ears were significant in patients who received magnesium along with metformin and gabapentin however there is no significant effect on systolic blood pressure.

Articles from Annals of Indian Academy of Neurology are provided here courtesy of Wolters Kluwer -- Medknow Publications

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