Annals of Indian Academy of Neurology
: 2016  |  Volume : 19  |  Issue : 6  |  Page : 24--132



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. Abstracts.Ann Indian Acad Neurol 2016;19:24-132

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Platform Session 01, Epilepsy08:30-09:30 h, Friday, November 11, 2016Hall A


Clinical profile and health related quality of life in patients with alcohol related seizures

S. R. Prasanth, C. V. Shaji, K. A. Kabeer, R. Mohan, G. Parvathy

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

Introduction: Alcohol related seizures (ARS) are adult-onset seizures that occur in the setting of chronic alcohol dependence. The clinical profile of 124 patients with ARS and their health related quality of life (HRQOL) is reported here. Methods: In this hospital based cross sectional study, consecutive patients admitted with new onset ARS (from July 2014 to February 2016) were included. Patients with previous diagnosis of epilepsy, those with a proximate well-known provoking cause of seizure (except alcohol) and those not consenting were excluded. Details of drinking pattern were analyzed. Alcohol dependence was identified by Alcohol Use Disorders Identification Test (AUDIT) and score more than 8 was taken as positive. HRQOL was assessed using the Life Situation Survey (LSS) questionnaire, once acute withdrawal symptoms have settled. Detailed neurological examination, hematological investigations, liver and renal functions, serum electrolytes were done. A 19-channel digital electroencephalography (EEG) was done in all cases. A CT brain (MRI in focal onset seizures) was done in all patients to rule out head injury or symptomatic lesions. Results: Of the 124 study subjects, all were males. Mean age of the sample was 32.84 (±6.37) years. The average daily consumption of alcohol was 176 g (±140 g). Mean duration of alcohol intake was 17.24 (±5.24) years. 19 (15.3%) patients had a family history of seizure, of which 2 had idiopathic generalized epilepsy. Of the 9 subjects with focal seizures, neuroimaging revealed right temporal cavernoma in one and hippocampal sclerosis in 2 patients. Occurrence of multiple seizures (2 or more) correlated with higher AUDIT scores. Patients with LSS score <80 had significantly higher AUDIT score and longer duration of alcohol intake (P = 0.05). Conclusion: A protocol based evaluation of ARS is necessary, to identify etiologies other than withdrawal. MRI Brain is useful in focal onset ARS, to identify underlying symptomatic lesions (3/9; 33.3%). Patients with higher AUDIT scores are more likely to have multiple seizures and a poorer HRQOL.


Long-term outcomes of drug refractory epilepsy patients who were not candidates for epilepsy surgery - A cross sectional study

I. Puri, D. Dash, M. V. Padma, M. Tripathi

Department of Neurology, AIIMS, New Delhi, India

Background: This study planned with aim to know Quality Of Life (QOL) and disability status of drug refractory epilepsy (DRE) patients who are not candidates of epilepsy surgery. Materials and Methods: A cross sectional study was conducted for one year at epilepsy clinic of Indian national hospital. All consecutive drug refractory epilepsy patients both adult and children who were found not a candidate of epilepsy surgery after pre-surgical evaluation were enrolled. QOL was assessed in adults by Quality Of Life in Epilepsy Inventory (QOLIE-31) and in children by Quality Of Life (QOLCE) parent rating form. Primary outcome was description of QOL of adults and children. Secondary outcome was to correlate QOL with epilepsy related baseline variables. Results: Sixty three patients were enrolled including 43 adults and 20 children. Monthly median seizure frequency was 12 (2-60) in adults and 2.5 (4.5-127.5) in children. National hospital seizure severity (NHS3) mean score was 9.6 in adults and 13.6 in children. In adults 63% of patients perceived reduction in seizure frequency when compared to frequency at the time of pre-surgical evaluation while 45% of pediatric patient's parent reported decreased in seizure frequency. Seizure free patients were 7% of adults. In non surgical candidate adults and pediatric patients mean scores in various QOL domains were similar to poor seizure outcome group with statistically significant difference to good seizure outcome group in most of domains. Seizure severity instead of seizure frequency was correlated with most of domains of QOL of adults. In children epilepsy related variables have little correlation with most of QOL domains. Conclusions: QOL of persons with DRE (both adults and children) who were not candidates of epilepsy surgery is poor. In adults, most QOL domains can be predicted by seizure severity while in children, parent reported QOL and disability are difficult to predict only by epilepsy related variables.


A comparative study of low dose sodium valproate, levetiracetam and lamotrigine for the treatment of Juvenile Myoclonic Epilepsy

P. Ojha, V. Sardana

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

Background: Juvenile Myoclonic Epilepsy (JME) is among the most common type of Idiopathic generalized epilepsy, consisting 5-11% of all epilepsies. Lifelong medication is usually required and seizure relapse is seen in 80% cases on stopping the medication. Sodium Valproate is considered as the first-line drug but has many complications with resistance observed in 15% patients. Low dose Sodium Valproate has been found to be effective with fewer side effects. Lamotrigine (LTG) is preferred in female patients due to the teratogenic and endocrinologic side effects associated with valproate. Levetiracetam has also emerged as an effective alternative due to lower rates of complications and safety in pregnancy. Objective: To compare the long-term effectiveness of Low-dose Sodium Valproate, Lamotrigine and Levetiracetam monotherapy in treatment of patients with JME. Design: Prospective, observational, long-term, unblinded study. Methods: This is an ongoing study in the Department of Neurology, Government Medical College, Kota, Rajasthan since January 2016. Informed consent has been recorded from all patients. All patients underwent detailed history, neurological examination, biochemical and radiological investigations along with EEG at frequent intervals. Patients were divided into three groups: those receiving low dose Sodium Valproate (500 mg daily), Lamotrigine (50 mg twice a day, slowly titrated) or Levetiracetam (250 mg twice a day). Patients were followed up after 15 days, 1, 3 and 6 months to assess the adequacy of seizure control on the basis of history and follow up EEGs. Results: A total of 28 JME patients have been studied till date. Among which 20 patients were on monotherapy, the remaining 8 patients were excluded as they were on polytherapy. Mean age of the patients was 17.53 years with a female preponderance (M:F=2:3). Myoclonic seizures were present in all (100%) while GTCS were present in 19 (95%) patients. The number of patients in low dose Sodium Valproate, Levetiracetam and Lamotrigine group were 12, 5 and 3 respectively. Final results will be analysed and presented. Conclusions: Low dose Sodium Valproate, Lamotrigine and Levetiracetam are being compared for treatment of JME. Final results will be analysed and presented.


Epilepsy surgery for lesional Lennox Gastaut syndrome: A case series

A. G. Diwan, A. Pillai, M. Vaidya

Department of Neurology, Vasantrao Pawar Medical College, Nashik, Maharashtra, India

Objective: Epileptic Encephalopathy can result from structural lesion in up to 70%. West syndrome or LGS can be secondary to gliotic or other structural lesion like FCD or developmental anomaly. Even though epileptic discharges are seen diffusely or generalised or even prominent on contralateral hemisphere, surgical resection or disconnection of the lesion can result in to favorable outcome. We studied clinical profile and outcome in recently started epilepsy surgery programme in Nashik, India. Methods: Medically refractory epileptic patients having MRI lesional Epileptic Encephalopathy of "Lennox Gastaut syndrome" was prospectively studied for outcome. Surgeries were performed in Nashik, India. Results: During October 2015 to January 2016, 5 LGS patients were operated for medically refractory epilepsy. Clinical profile-5 male patients with age from 4 to 18 years undergone Epilepsy surgery for the medically refractory epilepsy resulting from neonatal hypoglycemia, hypoxic ischemic encephalopathy or strokes due to prematurity. Along with refractory epilepsy-other deficits like mental subnormality, cortical vision impairment, stereotypie and Attention-deficit/hyperactivity disorder (ADHD) was seen. Seizure frequency ranged from 1/day to 100/day of variable seizures types like-drop attacks, focal clonic jerking, asymmetric tonic seizures and motor arrest. All patients were on at least 3 or more AEDs for more than 3 to 4 years. 2-Corpus callosotomy, 1-TPO disconnection and 2-Hemispherotomy was carried out and all showed very good surgery outcome - Engel 1. Cognitive improvement and Parent satisfaction was excellent with score of 9/10. Conclusion: All 5 patients received seizure freedom from all types of seizures except case no. 3, who developed Rt facial tonic spasm in 1 st 2 weeks after surgery, but became seizure free after that period. Cognitive improvement in all children was very good and all parents were satisfactory after the Epilepsy surgery. Though follow up period is short, the results are very encouraging. No major or persistent post-op disability was seen in any of the patient.


Nonconvulsive status epilepticus amongst comatose and noncomatose patients: Should they be viewed similarly?

S. D. Desai, D. Desai

Department of Neurology, Government Medical College, Karamsad, Anand, Gujarat, India

Purpose: Nonconvulsive status epilepticus (NCSE) amongst patients with coma may represent sequelae of damaged/dying brain and may differ from NCSE in non-comatose patients. Methods: Retrospective study of patients with NCSE (based on Hirsch et al. 2005) in Shree Krishna Hospital, Karamsad between 1 July 2009-31 December 2015 based on data from Electroencephalogram (EEG) records. We divided patients into two groups: A: NCSE without coma and B: NCSE with coma. Clinical characteristics, presentation, aetiology, EEG findings, treatment and outcome were compared. Results: 63 patients (38 males), mean age 67 (31-89) years were studied. 23 patients had NCSE without coma (group A), 40 patients had NCSE with coma (group B). In Group A, 15 had preserved ictal speech with confusion disorientation while 8 had aphasic NCSE. In group B, 21/40 had subtle signs. In Group A, EEG demonstrated focal/unilateral hemispheric abnormalities which resolved with intravenous midazolam associated with recovery. In Group B, 5 had focal abnormalities, 11 had hemispheric rhythm abnormalities, 7 had PLEDs, 5 had GPEDS and 2 triphasic waves. 13 patients showed clinical recovery after intravenous midazolam (definite NCSE) while the rest only had resolution of EEG abnormality without clinical improvement (probable NCSE). 11 patients could currently be classified as boundary syndromes. In Group A, 15 had structural lesions, 3 had metabolic abnormalities. In Group B, 18 had multifactorial metabolic abnormalities, 12 hypoxic ischemic encephalopathy, 7 structural lesions and 3 meningoencephalitis. In group A, all patients recovered, 20 with single AED, only 3 required 2 nd AED. In group B, 15/40 recovered. 30 received ≥2 AED, 15 received 3 AEDS. 25/40 had mortality within 3 months. Conclusion: NCSE in comatose differs completely in presentation, EEG finding, treatment and outcome with NCSE in non-comatose and both should be viewed as completely different entities.


Inflammatory biomarkers in patients of epilepsy

A. S. Deshmukh, V. Puri, N. Chaudhry, V. K. Gupta, A. Gupta, Sujata

Department of Neurology, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India

Aims: Recently, the role of inflammation has been demonstrated in the pathogenesis of various chronic diseases. Experimental and clinical studies suggest the association of inflammation with epilepsy. The purpose of this study was (1) to examine the baseline serum concentrations of high-sensitivity C reactive protein (hsCRP), Interleukin 6 (IL-6) and Tumour necrosis factor α (TNF-α) in 52 patients of epilepsy undergoing a video-electroencephalography (VEEG) study compared with 52 controls and (2) To study the change in their levels within 48 hours after an index seizure in patients. Materials and Methods: hsCRP, IL-6 and TNF-α levels were measured in serum at the beginning of the VEEG recording and again at 3, 6, 12, 24, 36 and 48 hours after the index seizure (the first verified localized or generalised onset seizure). Patients were divided into groups based on epilepsy type as follows: Temporal lobe epilepsy (TLE, n=26), Extra-temporal lobe epilepsy (ETLE, n=9), Simple partial seizures (SPS, n=2, not used in statistical analysis due to small number of patients in this group) and Generalized epilepsy (GTCS, n=15). Results: Serum IL 6 and hsCRP were elevated at baseline (interictal period) in all subgroups of epilepsy patients ie. TLE, ETLE and GTCS, while TNF α was elevated in patients with ETLE and GTCS only. Post ictal rise in levels of inflammatory biomarkers was seen in patients with TLE (IL 6 and TNF α) and GTCS (IL 6 and hsCRP), but not in ETLE. Serum levels of biomarkers showed no correlation with the imaging abnormality, drug therapy and disease duration or seizure frequency. Conclusion: Study supports the existence of inflammation in patients of focal as well as generalised epilepsy. The inflammatory biomarkers express independently and do not follow a tandem pattern. The inflammatory response (particularly TNFα) varies with the type of underlying epilepsy.

Platform Session 02, Stroke08:30-09:30 h, Friday, November 11, 2016Hall B


Clinical profile and outcome of patients with lateral sinus thrombosis seen at stroke unit

G. B. Kulkarni, M. A. Mirza, V. Mustare, R. Yadav, A. K. Gupta

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

Introduction: Among patients with Cerebral Venous Thrombosis (CVT), Lateral Sinus is commonly involved, there is a paucity of studies involving large cohort of single center experience. Objective: To define the clinical profile and outcome of patients with LST seen at our hospital. Methods: Prospective, hospital based study of patients with clinical and imaging features of LST admitted to stroke unit of NIMHANS Bengaluru, during 2 years (2012-13). Ethics approval and informed consent were taken. Results: There were 100 (M:F: 44:56) patients of LST (Isolated-27, combined-73 (with SSS-37, SSS and cortical veins/deep vein-36) mean age 31.45 + 11.3 years among 180 patients of CVT. Median delay for presentation was 11 days. Headache (97%), Seizure (46%), language deficit (26%) and motor weakness (35%) were common symptoms. Risk factors (sometimes multiple) were Anemia (57%), Hyperhomocysteinemia (61%), Puerperium (39%), Polycythemia (15%) and Oral Contraceptive Pill (OCP) intake (29%). MRI was taken as gold standard for diagnosis, sensitivity of CT brain was 69%. The other advantages of MRI were predicting the age and extent of thrombus, detecting subtle parenchymal lesions, micro-bleeds and additional sinus involvement not seen on CT. All patients were managed with anticoagulants, anti-epileptics, anti-edema measures and supportive care. Neurosurgical interventions were required in 17% patients and comprised of decompressive craniectomy-12, thecoperitoneal shunt-2 and EVD-3. The mortality was 4% - meningitis-1, brain stem herniation-2, pulmonary embolism-1. Patients were followed up for a mean duration of 6.5 + 4.01 months, 86% made good functional recovery (MRS 0-2). Conclusions: LST is usually associated with sagittal sinus and cortical vein thrombosis. Patients present with recent onset of headache and focal deficits. MRI is preferred investigation for diagnosis. Majority improve with anti-coagulation and standard care, 17% require neurosurgical intervention suggesting these cases to be managed in a comprehensive center with neurosurgical facility, if required.


Clinical profile of cerebral venous sinus thrombosis in South-Eastern Rajasthan with special reference to prognostic factors

P. P. Moon, V. Sardana

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

Introduction: Cerebral vein and sinus thrombosis (CVT) is rare compared to arterial stroke often occur in young individuals especially females. Cerebral venous infarction is the most serious consequence of CVT. Aims: To study the clinical profile of cerebral venous sinus thrombosis and to assess prognostic factors for death and prolonged hospital stay. Materials and Methods: A total of 56 patients with CVT were subjected to a detailed medical history and neurological evaluation. A MRI brain was carried out on a 1.5 T scanner using T1, T2, and DWI sequences and two-dimensional TOF MR Venography. Various haematological parameters were noted. Results: A total 56 patients were enrolled. The mean age was 33.98 ± 13.20 years. Female: male ratio was 2.3:1. Average hospital stay was 9.1 ± 7.2 days. Three patients died during hospital stay. Headache was the most common symptom i.e. in 46 (82.1%) patients followed by vomiting in 28 (48.2%). Altered sensorium was present in 13 (23.2%), hemiparesis in 23 (41.1%) & seizures in 23 (41.1%) patients. MRI brain showed infarct in 39 (69.6%) patients, out of which 24 (42.8%) had hemorrhagic venous infarct. Sub arachnoid haemorrhage was present in 7 (12.5%) patients. Most common sinus involved was superior saggital sinus (71.4%) followed by transverse (64.3%). Internal cerebral veins involvement was seen in 8 (14.3%). Bilateral sinus involvement was seen in 11 (19.6%). Mean difference of mean D-dimer which was rising with the increasing in number of vein involvement in patients with CVT. Predictors for poor outcome and prolonged hospital stay were altered sensorium, seizures, more than 5 venous sinus involvement, deep venous sinus involvement, hemorrhagic venous infarct and elevated D-dimer. Conclusion: All the patients of CVT with altered sensorium, seizures, deep venous sinus involvement, hemorrhagic venous infarct, elevated D-dimer should be managed aggressively in critical care unit especially patients having more than 5 venous sinus involvement.


Stent-assisted coiling of wide necked intracranial aneurysms: An experience of 26 patients

S. D. Rajadhyax, B. Chauhan, V. Bohra, V. Karan, V. Huded

Department of Neurology, Narayana Health City, Bengaluru, Karnataka, India

Aim: To audit the clinical presentation, aneurysm characteristics and outcome of patients undergoing stent assisted coiling (SAC) for wide necked intracranial aneurysms at a dedicated neuro-intervention division of a South Indian referral hospital. Methods: Consecutive patients undergoing SAC for wide necked aneurysms from 2009 to 2015 were enrolled. Aneurysm characteristics were studied on 3D cerebral angiogram. After deploying the stent across its neck, the aneurysm sac was densely packed with platinum coils. Pre and post procedure antiplatelet agents were administered based on a fixed protocol. Outcome was assessed with modified Rankin Scale (mRS) at discharge and 3 months follow-up. Control cerebral angiogram was performed at 6 month follow-up. Results: A total of 26 patients showed mean age of 49 years, with female predominance (69.23%). Majority of patients had ruptured aneurysms (n=18). Median aneurysm size and neck diameter were 7.5 mm and 4 mm respectively. Internal carotid artery was the most common site for aneurysm (n=18, 69.23%). Stent deployment and complete exclusion of aneurysm from circulation was successful in all the patients. Four cases had minimal stent thrombosis which was treated successfully with intravenous abciximab. Good mRS (0 or 1) at discharge was seen in all except three cases, due to sub-arachnoid haemorrhage related complications. Twenty patients were followed up at three and six months, all having uncomplicated clinical course and angiograms. Conclusion: Our experience with stent assisted coiling in wide necked intracranial aneurysms is promising and justifies this modality as a safe and effective treatment option.


Intima-media thickness of carotid artery as a measure of hyperlipidemia and vascular risk factor on prolonged exposure to enzyme inducing antiepileptic drugs

T. K. K. Reddy, Jyothsna, A. K. Meena, Preetika, Vasavi, K. K. Reddy

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

Introduction: Enzyme inducing drugs like phenytoin, carbamazepine, valproic acid are associated with weight gain which in turn caused by alteration of lipid metabolism and prone to stroke or other vascular events. It is found that intima-media thickness of carotid artery is an indirect and early indicator of stroke risk factor which can be taken as prognostication for vascular risk factors. Aim: To measure Intima-Media Thickness of carotid artery in patients with epilepsy on chronic exposure to antiepileptic drugs who are on phenytoin, carbamazepine, and valproic acid. Materials and Methods: Consecutive 61 patients with epilepsy who attended epilepsy clinic in Nizam's Institute of Medical Sciences and who are on prolonged enzyme inducing antiepileptics drugs are taken into study. Serum levels of fasting lipid profile, hs CRP, fasting insulin, c-peptide, lipoprotein a levels, serum homocysteine levels are measured. Intima-Media Thickness of carotid artery is also assessed. Results: Out of 61 patients, mean age of presentation is 25.30 + 12.07 years. Statistically significant difference in levels of HDL-C/TC and HDL-C/LDL-C are noted when compared to those with less duration of treatment when compared to chronic therapy in adults and children. Intima-media thickness of carotid artery Lipoprotein A levels, homocysteine levels, hs CRP levels is higher when compared to normative data of age matched controls. Conclusions: Patients with epilepsy on chronic therapy with inducing AEDs have significantly higher levels of HDL-C/TC and HDL-C/LDL-C. Intima - media thickness of carotid artery is higher which is surrogate marker for vascular risk factor.


Blood biomarkers in the diagnosis of stroke and its outcome

R. Bhatia, P. Sisodia, P. Bali, Aanchal, M. V. Padma, K. Prasad

Department of Neurology, AIIMS, New Delhi, India

Introduction: Increasing work is being done on blood biomarkers to prognosticate stroke severity, etiology and outcomes. The present study looked at various blood biomarkers to differentiate the types of stroke and their relationship to outcomes. Methods: This is an ongoing prospective study being carried out on all acute stroke patients presenting within 24 hours of stroke onset, with a planned sample size of 250 patients. The different blood biomarkers being studied include S100, Interleukin 6 (IL6), brain natriuretic peptide BNP, Glial Fibrillary Acidic Protein GFAP and N-methyl-d-aspartate NMDA receptor antibody NR2 subunit. Details of demographics, imaging details, risk factors, treatment interventions required during hospital stay, etiological work up and 3 months outcomes on mRS scale are recorded. Results: As of now, 225 patients have been recruited. Details of the type of stroke and onset time, risk factors, work up details, treatment received, etiology and 3 months mRS is recorded. Biomarker assay has been completed in 180 patients and in the rest is underway. Statistical analysis will be performed to study the differences in blood biomarkers assay between the types of stroke and their relationship to outcome and other variables. The detailed results shall be presented at the meeting. Conclusions: Blood biomarkers may help us in differentiating the types of stroke and prognostication of outcome.


Dabigatran in cerebral venous sinus thrombosis: Report of 5 cases

C. K. Josehva, N. V. Chowdhury, S. K. Jaiswal, M. M. Reddy, J. M. K. Murthy

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

Dabigatran has been shown to be effective in cases of cerebral venous sinus thrombosis (CVT). Materials and methods: Retrospective review of case records of patients with CVT who received dabigatran alternate to coumadins in the last two years. Diagnosis of CVT was established by plain and contrast magnetic resonance venography (MRV). Results: Of the 35 patients with CVT admitted during the study period, 5 (14.2%) received dabigatran. All were males and age ranged between 24 and 52 years. The risk factors were hyperhomocystenemia in 3 and no risk factor in the remaining two patients. Indications for dabigatran were: allergy to coumadins (1); alcoholic hepatitis (1), failure to achieve adequate INR in spite of adequate dose of coumadins. (1), patient non-complaint with drug monitoring (1), and labile PT/INR (1). The patient with labile PT/INR (Case 5) had endovascular thrombectomy. All patients received dabigatran 150 mg twice a day. Except Case 4 all patients were symptom free and were off dabigatrin at 6 months. Case 4 had recurrence of symptoms and repeat MRV showed acute thrombosis of sinuses. He was put back on Coumadin. Conclusion: Dabigatrin is safe alternative for coumodins in select clinical settings.

Platform Session 03, Headache;08:30-09:30 h, Friday, November 11, 2016Hall


Prospective study of largest case series of paroxysmal hemicrania from India and a critical review of the ICHD 3β diagnostic criteria

D. Chowdhury, R. Arora, A. M. Singh, G. A. Khwaja

Department of Neurology, GB Pant IPGMER, New Delhi, India

Background: There is scarce data on Paroxsysmal Hemicrania (PH) from India, a rare subtype of Trigeminal Autonomic Cephalalgia. Objective: To assess clinical features, psychiatric co-morbidities & treatment outcome in PH and to critically review ICHD 3β Diagnostic Criteria. Methods: Patients attending headache clinic from August 2013 to March 2016 were prospectively evaluated for PH (by ICHD 3β) using a structured proforma. Treatment outcome evaluated at 3 months. Results: Demographics of 33 patients, 19 had chronic (CPH) and 14 had episodic form (EPH). 19 were probable and 14 definite PH. Mean age 39.18 ± 12.03 years and F/M ratio was 2.3:1. Mean diagnostic-delay was 4 years. Headache Characteristics: 84% of probable PH lacked the criterion of >5 attacks/day in 50% of attacks. The mean attack duration 22.6 ± 6.9 minutes and mean VAS was 9. Majority had sidelocked throbbing pain (84.6%) in orbitotemporal region (61.5%). Cranial autonomic symptoms (CAS): Seen in 96% patients, facial sweating (60%) and lacrimation (52%) were commonest; aural fullness in 48% patients. 90% developed CAS during all attacks. Restlessness: Motor restlessness (changing body position (81%) and rubbing head (69%)) and psychological restlessness (inner restlessness (66%), suicidal ideation (54%) and impending doom (31%)) was seen. Psychiatric Co-morbidities: Seen in 77% patients including anxiety (70%), depression (65%), panic attacks (42%) and somatization disorder (35%). Autonomic Function Testing: 85% had abnormal BP to sustained hand grip. SSR was un-recordable in 2 and 24 hour-Holter abnormal in 1. Treatment Response: All responded to indomethacin (150-225 mg/day). At 3 months, 65% had complete remission, 35% breakthrough headaches. Chief AEs were gastritis and dizziness. Conclusion: ICHD III β criterion >5 attacks/day for >50% of the time for diagnosing PH needs modification. Aural fullness should be retained as a CAS while restlessness during attack should be incorporated. Indomethacin response is absolute but long term compliance is difficult.


Writer's cramp: How much task specific?

H. Kumar, S. Choudhury, P. Chatterjee, M. U. Kulsum, B. Mondal, S. Chatterjee

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

Introduction: Writer's Cramp (WC) is considered to be task specific but many patients report difficulty in other tasks apart from writing. We tried to explore motor symptoms other than writing difficulty in all those patients of WC. Methods: Patients attending the outpatient department of a tertiary care neurology referral centre in the eastern part of India were recruited, who initially presented with writing difficulty and diagnosed as Writer's Cramp. Individual patients were assessed for functional impairment by Disabilities of The Arm, Shoulder and Hand (DASH) and Arm Dystonia Disability Scale (ADDS) scale. The clinical severity was assessed by specific Writer's Cramp Rating Scale. We excluded mild (included: moderate to severe) disabilities as it might not be clinically significant in all cases. Results: 30 consecutive patients with WC were recruited. Interestingly, all of them were male. The mean age of the patients was 54.83 years (SD 10.77). Mean disease duration for this cohort was 72.36 months. 29 out of 30 patients complained of moderate to severe writing difficulties. 40% patients were facing difficulties while carrying heavy objects (over 10 lb) and 16.7% while carrying a shopping bag/ brief case. Among WC patients 20% had problem while performing heavy household chores. 16.6% had difficulties in changing bulbs. 10% of patients had difficulties in preparing meal and 20% while using a knife while cutting food. 29.9% and 26.6% had difficulties in handling utensils/ feeding and grasping objects respectively. 43.3% complained of pain and 30% had stiffness in arm, shoulder or hand. The overall functional scales did not correlate with the specific clinical assessment scale (r=0.17, P = 0.35). Conclusion: Number of patients complained of difficulties in activities other than writing. About 40% of patients had difficulty in carrying heavy objects, possibly due to precipitated dystonia.


A prospective observational study of 12 patients with SUNCT/SUNA

P. C. Makwana, S. Prakash, C. Rathore, A. Dave, D. Shah, S. Shah

Department of Neurology, SBKS Medical Institute and Research Center, Vadodara, Gujarat, India

Introduction: The trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders characterised by unilateral headaches that occurs in association with ipsilateral cranial autonomic features. The TACs include cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks, and hemicrania continua. There are two subtypes of short-lasting unilateral neuralgiform headache attacks: (i) Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) (ii) Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). Materials and Methods: In this prospective study, patients fulfilling the ICHD-3β criteria for SUNCT/ SUNA were evaluated. The patients were recruited from a tertiary neurology OPD clinic. The study period was December 2015 to March 2016. All patients were subjected to neuroimaging to rule out secondary causes. The patients were evaluated for demographic data (age, gender, etc.) and clinical characteristics (frequency of stabs, mean duration of stabs, location, and associated cranial features, and timing of attacks, and any triggers). Results: We recruited 12 patients over 16 months. A clinical diagnosis of SUNCT was in 41.67% and SUNA in 58.33% patients. The mean age at the time of examination was 47.8±16.8 years (range 18-70 years). Male:Female ratio was 1:1. The mean duration of illness was 25.6 ± 23.9 months (range 3 months-5 years). All patients had pain in orbital area. The frequency was more than 10 attacks / day in half of the patients. All patients had headache attacks duration of less than 5 minutes. Only 16.6% had predominantly nocturnal attacks. Lacrimation (83 %) and conjunctival injection (75%) were two most common autonomic features. All patients received lamotrigine. More than 50% patients showed at least 50% response. Conclusions: This is the largest case series on SUNCT/SUNA from India.


Prevalence of medication overuse headache in primary headache cases

K. Venkatraman, S. Gobinathan, Chandramouleeswaran, R. Lakshminarasimhan, V. Kannan, J. Manikavasagam

Department of Neurology, Madras Institute of Neurology, MMC, Chennai, Tamil Nadu, India

Aim: To study the prevalence of medication overuse headache in patients treated for primary headaches in a Tertiary Care hospital. Background: Medication overuse headache is a dull aching headache that results from analgesic overuse in patients treated for primary headache disorders and other painful conditions. Materials and Methods: One forty two patients who were enrolled for a comparative study between episodic and chronic primary headaches were diagnosed on the type of headache using the ICHD-3 (beta) version and grouped into patients with isolated primary headache (group 1) and patients with primary headaches along with medication overuse headache (group 2). The prevalence of medication overuse headache was estimated. Results: 94 patients had isolated primary headaches and 48 patients had primary headaches along with medication overuse headache. Of the 48 patients only 5 patients had episodic headache (6%) and 43 had chronic headache (65%). All the 46 patients gave two different characters of headache, one satisfying their primary headache and other due to medication overuse. Patients in group 2 had statistically significant higher scores in MIDAS, HIT-6 and PHQ-9 scores. On careful withdrawal of the offending drug all the patients reported improvement in the medication Induced headache. Conclusion: A strong suspicion of medication overuse headache in primary headaches should be sought when the patient reports persistent and different character of headache especially on awakening. Proper counseling on avoidance of over the counter analgesic drugs and judicious use of analgesics in primary headaches helps prevent medication overuse headache.


Clinical, radiological, neuropsychological profile and management of 26 cases of normal pressure hydrocephalus - A retrospective case analysis from NIMHANS

S. Ramakrishnan, C. Prasad, M. Philip, Veerendrakumar

Department of Neurology, NIMHANS , Bengaluru, Karnataka, India

Aim: To retrospectively analyse the clinical, radiological, cognitive profile and management of 26 cases of Normal Pressure hydrocephalus (NPH) evaluated at NIMHANS. Patients and Methods: Case records of patients with ICD code 101.5/G 91.2 were evaluated from records since 2000. The clinical features, imaging parameters, response to drainage lumbar Puncture(LP), management and outcome were analysed using SPSS v16. Results: 24 patients were men. Age at onset ranged from 53 to 80 yrs (mean 68.3 yrs). The first symptom at presentation was gait difficulty in 16/26 patients (61.5%). Mean duration of symptoms was 37.3 ± 38 months. Hakim's triad was seen in 17/26 (65.4%). Cognitive dysfunction was seen in 21 (80.8%), behavioural dysfunction in 9 (34.6%), falls in 8 (30.8%), urinary incontinence in 17 (65.4%) and bowel incontinence in 6 (23.1%). Mean mini-mental state examination score was 19.7 ± 6.4. Detailed mental state examination revealed diffuse, frontal lobar dysfunction or sub cortical involvement in 24. Frontal release signs were seen in 8 (30.8%), parkinsonism in 24 (92.3%) and pyramidal signs in 11 (42.3%). In MR imaging studies, ventriculomegaly out of proportion to cerebral atrophy was seen in all. Mean Evan's index was 0.36 ± 0.05. T2 flow void sign was seen in 15 (57.7%), periventricular lucencies were seen in 16 (61.5%), deep white matter hyper intensities were seen in 12 (46.2%), enlarged sylvian and basal cistern was seen in 18 (69.2%) and effacement of sulcal spaces at high convexity was seen in 12 (46.1%). 21 had undergone drainage lumbar puncture once or more. After LP, gait improvement was seen in 16, cognitive improvement in 8 and bladder improvement in 3. Ten (38.5%) patients underwent surgery (Ventriculo-peritoneal shunt -7 and theco-peritoneal shunt - 3) and remaining were managed conservatively. Conclusion: Improvement in timed gait, stride length after drainage LP was an important predictor of surgery. Few patients improved temporarily with drainage LP. There is a need for uniform NPH scale/tool for management strategy.


Effect of high rate rTMS on somatosensory evoked potential in migraine

J. Kalita, S. K. Bhoi, U. K. Misra

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

Aim: Sensitization and impaired habituation of cortical neurons have been reported in migraineurs. Repetitive transcranial magnetic stimulation (rTMS) may change these phenomena and be the basis of therapeutic response. We report the effect of 10 Hz rTMS on sensitization and habituation of median somatosensory evoked potential (SEP) in migraineurs, and correlate these changes with clinical response. Methods: Migraineurs having four or more episodes of headache per month were included and their clinical details were noted. Three sessions of 10 Hz rTMS, 600 pulses in 412.4 seconds were delivered on the left frontal cortex corresponding to the hot spot of right abductor digiti minimi, on alternate day. Median SEP was done before and 30 minutes after the third rTMS session. Sensitization (block I N20 amplitude) and impaired habituation (if N20 amplitude of block 2 or 3 were not suppressed compared to block I) were noted. The reduction in frequency and severity of headache in the next month were noted and correlated with SEP changes. Results: Ninety-four migraineurs were included; 56 received true rTMS and 38 sham stimulation. Following stimulation, reduction in N20 amplitude of block 1 correlated with reduction in frequency and severity of headache at 1 month. The impaired habituation significantly improved in true rTMS group compared to sham stimulation, and correlated with reduction in severity of headache but not with frequency. Conclusion: In migraineurs, 10 Hz rTMS improves habituation and may be the biological basis of headache relief.

Platform Session 04, Movement Disorders16:30-17:30 h, Saturday, November 12, 2016Hall A


South Indian perspective on young-onset Parkinson's disease: An observational study

M. M. Suraj, C. V. Shaji, K. A. Kabeer, M. K. Ram, Robin, S. Meenakumari

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

Introduction: Young onset Parkinson's disease (YOPD) refers to cases presenting with a parkinsonian syndrome with onset before the age of 50 years or 40 yrs according to some authors. The clinical features and the response to treatment in YOPD differ from Idiopathic Parkinson's disease. Whether the clinical profile is similar in our geographic population is unclear. Aims and Objective: To study the epidemiology and clinical profile of YOPD, to study the various associations, and to analyze whether there exists a variation in the phenotypical expression of YOPD in the study population from its Western counterparts. Materials and Methods: This case series study was conducted in the neurology wards of a tertiary care centre in South India. All cases meeting the UK Parkinson's Disease Society Brain Bank clinical diagnostic criteria for Parkinsonism with age <50 yrs were included in the study. Cases with history of repeated stroke with stepwise progression of Parkinsonism, presence of Intra Cranial Space Occupying Lesions (ICSOL), early dementia, and early severe autonomic involvement were excluded. Collection of data was done in pre-defined performa. Clinical features, family history and levodopa response were obtained based on written questionnaire. Routine Investigations were recorded. Results: Seventeen cases were included in the study of which 9 cases (52.9%) were females. Mean age was 44.5 yrs (+6.5 yrs). Tremor was the most common presenting symptom (41.2%) and Hypokinesia (82.4%) was the most common clinical feature. Left side (52.9%) was slightly more involved than the right. The mean duration to presentation from the onset of symptom was 37 months. Family history of moment disorder was noted in 17.6% and chemical exposure in 23.5%. All cases with chemical exposure had developed tremor. None had cognitive impairment. Eye movement abnormalities were noted in 58%, were significantly associated with hypokinesia (p<0.05), and pull test was positive in 41.2%. Structural MRI abnormalities were noted in 41.2%, and had significant association with those cases presenting with gait abnormalities (p<0.05). Fatty liver was noted in 29.1% and all cases with fatty liver had hypokinesia (p>0.05) and all of them had deranged Liver function test values. Favourable response to levodopa was noted in 94% of cases and dyskinesias in 24%. Levodopa induced dyskinesias was least common in tremor predominant cases. Dental fluorosis was noted in 41.7% of cases. Discussion: The proportion of females with young onset Parkinson's disease seems to be higher than in other Western studies. Although tremor was the most common presenting symptom, Hypokinesia was the most common predominant clinical feature, which is in stark contrast to previous studies. A significantly higher proportion of cases were noted to have eye moment abnormalities, which was associated with hypokinesia. Upto 30% saccadic eye movement abnormalities have been described in previous studies, but our study shows even higher proportion. None of the cases had cognitive impairment. Fluorosis has been proposed in neuronal degeneration earlier; whether the higher prevalence of fluorosis in our cases had a causal effect needs to be evaluated further. Parkin gene mutations have been previously linked to liver dysfunction in mice. Whether the observed higher prevalence of fatty liver in our cases is due to a similar mechanism merits further evaluation. Conclusion: there is a striking difference between the epidemiologic and the clinical features of YOPD among patients in the study population and the previously reported cases. Whether fluorosis has a role in the causal mechanism need to be studied further.


Movement disorders in patients with Japanese encephalitis: Experience in a tertiary care hospital in North Eastern region of India

A. R. Barua, N. C. Borah, P. S. Deb

Department of Neurology, Guwahati Neurological Research Centre, Institute of Medical Sciences, North Guwahati, Assam, India

Objective: Movement disorders in Japanese encephalitis is known, but under reported. In this study we tried to analyze the spectrum of movement disorders in patients with Japanese encephalitis correlated with radiological findings in a tertiary care hospital in North Eastern region of India. Background: Japanese encephalitis has been emerging as a major health problem in South East Asia and several out breaks have been reported in South - East Asia. It has been reported first in India in the southern state of Tamil Nadu in 1955. The disease is appearing in sporadic or in epidemic outbreaks since 1976 in various parts of North Eastern region of India, particularly in the state of Assam. Geographic location, Climatic condition, abundance of potential vectors, adequate reservoir, amplifying hosts, socio-cultural behavior of the people, agricultural practices etc are contributing for a congenial atmosphere for the spread of Japanese encephalitis virus in the region. Methods: We reviewed prospectively 34 patients admitted in the hospital during the monsoon season (June through September) in two consecutive years (2014-15). Diagnosis of Japanese encephalitis was based on clinical, serological (Cerebrospinal fluid or serum) and radiological criteria. Samples (Cerebrospinal fluid/Serum) were tested for Japanese encephalitis virus specific IgM antibody using ELISA technique. Magnetic resonance imaging (MRI) of the brain (Using 1.5 Tesla) was done in all the patients. Among which 14 (41.1%) patients had presented with various forms of movement disorders. Results: Patient's age ranged between 7-85 years. Male:Female ratio = 9:5. 9 (64.2%) patients who were in coma had facial masking while coming out of coma. Tremor in 10 (71.4%), Generalized dystonia in 1 (7.1%), Rigidity in 7 (50%), bradykinesia in 8 (57.1%), choreiform movements in 2 (14.2%). In MRI brain studies, bilateral thalamic involvement was present in all the patients. Basal ganglial lesions were found in all the patients presenting with movement disorders. 3 (21.4%) patients had midbrain and pontine involvement and 2 (14.2%) patients had cerebellar involvements. Conclusions: Movement disorders are not manifested as a presenting symptom initially, but are not uncommon manifestations and typically related to the anatomical site of involvement.


Typical and atypical MRI features of multiple system atrophy with special reference to cerebellar and parkinonian variants

R. Tandon, S. Pradhan

Department of Neurology, SGPGIMS, Lucknow, Uttar Pradesh, India

Objectives: MRI features like 'Hot-Cross Bun' sign and hyper intense putamen rim are considered to be typical for MSA (Multiple System Atrophy) and some features like 'corpus callosal atrophy 'do occur in other degenerative disorders also. We studied their frequency in MSA patients, especially with regard to Parkinsonian (MSAp) and cerebellar variant (MSAc) of MSA. Methods: Clinical and MRI features of 53 MSA patients presenting to the Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, diagnosed according to 'Second consensus statement on the diagnosis of multiple system atrophy' were analyzed in context of MSAp and MSAc between August 2010 and August 2013 with regard to midbrain atrophy, cerebellar atrophy, 'Hot-Cross Bun' sign, hyperintense putamen rim and corpus callosal atrophy. Results: Of 53 cases (mean age: 59.53 ± 9.74 years), 16 (30.2%) had MSAc and 37 (69.8%) had MS Andhra Pradesh Midbrain atrophy was found in 37 (69.8%) MSA patients (70.3% of MSAp and 68.8% of MSAc patients), cerebellar atrophy in 45 (84.9%) MSA patients (81.1% of MSAp and 93.8% of MSAc patients), 'hot-cross bun' sign in 13 (24.5%) MSA patients (27% of MSAp and 18.8% of MSAc patients), hyperintense putamen rim in 19 (35.8%) MSA patients (37.8% of MSAp and 31.3% of MSAc patients) and corpus callosal atrophy in 39 (73.6%) MSA patients (75.7% of MSAp and 68.8% MSAc patients). Conclusion: Hyperintense putamen rim, midbrain atrophy and cerebellar atrophy may occur in high percentage of MSA patients. "Hot cross bun" sign may not occur in most of them. Callosal atrophy may occur in MSA. 'Morning Glory Sign', typical feature of progressive supanuclear palsy may also occur in MSA.


Bilateral subthalamic stimulation improves non motor symptoms in advanced Parkinson's disease

S. M. Zafer, K. K. Pisharady, R. Rajan, S. Krishnan, G. S. Sarma, A. Kishore

Department of Neurology, Sree Chitra Institute of Medical Sciences and Technology, Triavndrum, Kerala, India

Background: Bilateral deep brain stimulation (DBS) of Subthalamic Nuclei (STN-DBS) is an established treatment for patients with advanced Parkinson's disease (PD) with proven effects on motor complications and quality of life. Non-motor symptoms (NMS) are commonly seen and contribute to significant disability in advanced PD. Very few studies have quantified the effect of STN-DBS on NMS. Aim: To examine the effect of bilateral STN-DBS on different domains of NMS in advanced PD at 1 year follow up after surgery. Methods: 73 consecutive patients with PD, who underwent bilateral STN-DBS, were evaluated prospectively using the non-motor Symptoms Scale (NMSS), 1 week before surgery and 1 year later. Motor status, mood and cognitive functions were evaluated using the Unified Parkinson's Disease Rating Scale (UPDRS) Parts I-IV, Hoehn and Yahr (H and Y) Stage, Beck's Depression Inventory (BDI) and Addenbrooke's Cognitive Examination (ACE) Results: The total NMSS score showed significant improvement (p=0.005) after surgery. Among the individual domains cardiovascular (p = 0.007), sleep/fatigue (p=0.001), mood and cognition (p = 0.008), perceptual problems/hallucinations (p = 0.024), attention/memory (p = 0.013) and the miscellaneous items domain - addressing symptoms like pain, taste/smell, recent change in weight and sweating (p = 0.00) showed significant improvement. UPDRS parts II, III and IV also improved significantly (p<0.05 for all comparisons). Conclusion: Bilateral STN-DBS improved not only the motor functions but also had a positive impact on the non-motor symptoms of PD. Long term follow-up studies are needed to see whether the improvement in NMS is sustained parallel to the motor benefits.


Clinical profile and diffusion tensor imaging in parkinsonian syndromes

P. Kakarala, G. Butchiraju, S. Gopi, T. S. Kumar, U. A. Kumari

Department of Neurology, Anthra Medical College, Kings George Hospital, Visakhapatnam, Andhra Pradesh, India

Introduction: Parkinsonism is an extrapyramidal movement disorder which is characterized by rest tremor, bradykinesia, rigidity and postural instability secondary to degeneration of dopaminergic neurons in striatonigral region. Advances in MRI techniques have provided new tools for the diagnosis of PD in its early stages and have discriminated it from other atypical PD syndromes. Aims and Objectives: To study the clinical profile and neuroimaging aspects of patients presenting with parkinsonian symptoms and aiding in their diagnosis and management. To explore the role of DTI in early diagnosis and it's utility as a potential screening tool for parkinsonian syndromes. Materials and Methods: 50 patients aged above 18 yrs presenting with primary Parkinsonism, Parkinsonism plus syndromes, predominant Parkinsonism features in heredodegenerative Parkinsonism groups were included. Patients were subjected to detailed case proforma questionnaires and categorized under two groups typical and atypical Parkinsonism. Clinical assessment was done by Unified Parkinson's disease rating scale (UPDRS), Modified Hoehn and Yahr staging and a 750 wide bore 3 T GE made MRI with Echo planar and diffusion weighted imaging in 25 directions was used for imaging of the brain. In present study -25 patients and 10 controls in whom DTI was performed, fractional anisotropy (FA) values were obtained from regions of interest (ROI)- cingulate cortex, basal ganglia, corticospinal tracts, superior longitudinal fasciculus, substantia nigra, thalamus regions of brain. Results: In our study male population represented higher cases than females and mean age of presentation was 49.94 yrs. Typical PD patients constituted 84% and atypical PD 16%. Median UPDRS-III motor scale for typical PD patients was 36 and atypical PD patients were 55. The mean FA values of substantia nigra regions of PD patients when compared to controls, was lower but statistically not significant (0.4256 vs 0.44801, p=0.076). None of the FA values in studied regions showed statistically significant difference between patients and controls. Conclusion: Our study DTI findings using ROI analysis helped in differentiating typical vs atypical PD syndromes and also diagnosing PD patients at an early stage. Eventhough there was no statistical significance among core parameters obtained; this study reinforces the findings in terms of trends in FA values thereby establishing the role of DTI in diagnosing PD patients.


Therapeutic effect of non-invasive vagus nerve stimulation in gait disturbance and freezing in Parkinson's disease patients

B. Mondal, M. Baker, S. Choudhury, K. Chatterjee, P. Chatterjee, H. Kumar

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

Introduction: Freezing of gait (FOG) and other gait disturbances are common yet difficult to manage symptoms in Parkinson's disease (PD). Vagus nerve stimulation (VNS) is the use of galvanic stimulation of the Vagus nerve possibly modifying the cortical excitability. Trans-cranial Direct Current Stimulation (TDCS) has shown to improve gait in some studies. In the same vein, we hypothesize that VNS could improve FOG in PD patients by modulating cortico-subcortical interactions and abnormal patterns of cortical activation related to gait planning. Objective: In this observational pilot study we explored the novel therapeutic approach of non-invasive VNS to improve the gait pattern and FOG in PD patients. Methodology: Eleven patients of PD with gait disturbance were recruited from the movement disorders clinic of Institute of Neurosciences, Kolkata. VNS (GammaCore) was applied to each of them. The stimulation was given twice in 15 min interval. Pre and post VNS gait parameters were recorded using GAITRite, an electronic walkway. Differences between gait parameters before and after VNS were assessed using Wilcoxon Sign Rank test with significance level of P < 0.01. The parameters of FOG were independently assessed by video recording of the gait of the patients by independent raters using a rating scale adopted from FOGQ by Giladi et al. Results: The mean age of the patients were 65.72 and 73% of them were male. The mean UPDRS (III) score was 45 and H&Y was 2.78. Statistical results suggested significant improvements in gait parameters including velocity (Pre VNS 52.164, Post VNS 62.136, p value 0.012), swing time(Pre VNS 0.38, Post VNS 0.055, p value 0.001), stride velocity(Pre VNS 53.01, Post VNS 62.994, p value 0.008) and stride length(Pre VNS 65.408, Post VNS 74.624, p value 0.025). Video analysis of gait suggested objective improvement in Start hesitation, Freezing while walking on straight and freezing during after VNS. Conclusion: The study showed significant improvements in gait parameters and freezing in patients with PD after VNS. Long-term stimulation might be more effective in these patients. This could provide way for non invasive therapeutic alternative for freezing.

Platform Session 05, Neuromuscular Disorders16:30-17:30 h, Friday, November 11, 2016Hall B


Patterns of nerve enlargement in demyelinating neuropathies on high resolution ultrasound

M. Y. Reddy, S. K. Jaiswal, A. K. Meena, J. M. K. Murthy, N. V. Chaudary

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

Background: High resolution ultrasound (HRUS) is increasingly being used for evaluating mononeuropathies. Literature on the utility of neuromuscular ultrasound in polyneuropathies is limited. This study aims to describe patterns of nerve enlargement and fascicular architecture in various types of demyelinating polyneuropathies. Materials and Methods: This study was conducted at tertiary care hospital from Hyderabad, South India between May 2015 to March 2016. HRUS was performed on Philips HD15 with linear 12-3 probe. The cohort comprised total of eleven patients (seven CIDP, two HMSN, one AIDP and one MMNCB). Mean Cross sectional area (CSA) at specific sites, intranerve cross sectional area variability (INV) of 21 median, 19 ulnar and 19 posterior tibial nerves from 11 patients were studied. Pattern of enlargement of each nerve in a disease group was compared with normal controls. Results: Median CSA and INV values in mm 2 of various nerves in various disease groups: CIDP vs. HMSN vs. AIDP vs. MMNCB - Median at wrist: 15 vs. 5.3 vs. 18.3 vs. 9.55; median at mid forearm 12.7 vs. 7.0 vs. 5.8 vs. 8.95; Median at mid arm: 12.55 vs. 7.5 vs. 5.1 vs. 9.55; Ulnar below elbow: 7.4 vs. 5.6 vs. 5.75 vs. 10.75; Ulnar at elbow : 12.6 vs. 6.85 vs. 8.7 vs. 12.65; Ulnar above elbow: 17.8 vs. 6.1 vs. 5.0 vs. 13.0; Tibial at ankle: 22.6 vs. 13.3 vs. 9.0 vs. 13.5; Tibial at distal leg: 21.3 vs. 13.0 vs. 14.9 vs. 17.65; INV of median nerve: 9.75 vs. 2.1 vs. 13.2 vs. 1.35; INV of ulnar nerve: 7.4 vs. 1.75 vs. 4.0 vs. 9.55; INV of Tibial nerve: 22.6 vs. 1.0 vs. 5.9 vs. 4.15. Conclusions: This study shows profound and non uniform nerve enlargement in acquired demyelinating neuropathies compared to hereditary demyelinating neuropathies across all nerve trunks.


Biopsy proven peripheral vasculitic neuropathy is resistant to treatment and associated with poor prognosis

M. P. Kate, D. Arora, J. D. Pandian, V. Jaison, K. Kwatra, P. Paul

Department of Neurology, Christian Medical College, Ludhiana, Punjab, India

Introduction: Peripheral Vasculitic Neuropathy (PVN) presents with a wide spectrum of symptoms and signs. Further it is difficult to diagnose without nerve biopsy. Scarce data is available regarding the outcome of biopsy proven PVN. We aim to describe factors affecting the outcome of biopsy proven PVN. Methods: In this cross sectional observational study according (to strengthening the Reporting of Observational studies in Epidemiology (STROBE guidelines) we identified biopsy proven PVN from our institutional database. In structured case record form we abstracted clinical pathological and treatment details. All biopsy were re-assessed by pathologist (KK, PP). Results: Over a period of last 5 years 110 nerve biopsies were performed. A total of 35 were diagnosed to have vasculitis. Complete data was available for 26 patients. Mean age of patients was 53.4 ± 14.3 years and were predominantly male (69.2%). The most common presentation was polyneuropathy (65%) followed by mononeuritis multiplex (35%). The median (IQR) duration of symptom onset to diagnosis was 165 (567) days. The most common biopsy finding was Lymphocytic infiltration 52% and 48%% had associated demyelination. Sixteen (61.53%) were treated with prednisolone, for a mean duration of 7 ± 5 months. However only 2 (7.7%) patients had a complete remission at 3 months. Partial remission and no remission observed in 3 (11.5%) and in 22 (84.61%) respectively. Conclusion: PVN is rare disabling disease. It is usually resistant to prednisolone and long term outcome is poor. There is need for randomised control trial using multiple-immunomodulators.


Mutations in congenital myasthenic syndromes

A. Nalini, V. P. Kumar, K. Polavarapu, S. Vengalil, A. G. Engel, X. M. Shen

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

Background: Congenital myasthenic syndromes (CMS) are a group of treatable inherited neuromuscular junction (NMJ) disorders. Objective: To describe the clinical and genetic pattern in 11 cases of CMS. Methods: Genomic DNA was isolated from peripheral blood and sequenced to identify the mutation. Results: All were sporadic cases with onset in infancy/early childhood. RNS revealed decrement, neostigmine test was positive. Nine had homozygous mutations in AChR epsilon subunit Cholinergic Receptor Nicotinic Epsilone (CHRNE). All had early onset ptosis, severe opthalmoparesis and fatigable limb weakness. Two had novel missense mutations in pre-M1 (c.712C>T) and M2-M3 linker (c.905C>T) regions of extracellular domain respectively. One had homozygous null mutation (c.729C>T (p.R91L). One had a novel homozygous missense mutation in c.652G>G) in M1 domain. Novel frame-shifting mutations in four patients included: A five bp duplication (c.183_187dup) and four bp deletion (c.684_687delTGAC) in exon 2 and 7 of extracellular domain; a 24 bp deletion (c.1216_1219+20del24) and 1-bp deletion (c.1327delG) in exon 10 and 12 of long cytoplasmic loop. Two had c.1267delG in exon 12, a founder mutation described in Roma gypsies and Indian/Pakistani patients. One carried two heterozygous mutations in RAPSYN, common N88K mutation and a c.272G>A (p.Asp218Asn) in exon 5 of DOK7. These two had early onset limb fatigue, persistent weakness, ptosis and diplopia. All responded well to pyridostigmine except DOK7 case who responded well to salbutamol. Conclusion: CMS are potentially treatable conditions which are frequently mislabelled as myopathies. Early identification is essential to avoid morbidity and life threatening complications. All attempts to be made to have a genetic diagnosis as definitive treatment is decided based on the mutation pattern.


Etiological and biochemical profile of hypokalemic paralysis in North-West India

A. Garg, C. M. Sharma, B. L. Kumawat, D. Khandelwal, P. Agarwal, M. Shah

Department of Neurology, Sawai Man Singh Hospital, Jaipur, Rajasthan, India

Background: Acute neuromuscular weakness, a common neurological emergency may have varied causes, of which hypokalemia is an important metabolic cause, characterized by acute flaccid paralysis which can be life threatening if not treated on time, and frequently has secondary treatable causes. Objective: To study the etiology, clinical and biochemical features in patients of hypokalemic paralysis. Materials and Methods: All patients who admitted with acute flaccid paralysis with hypokalemia from September 2012 to December 2015 were included in the study. Serum electrolytes, arterial blood gas analysis, electrocardiogram, urine analysis, renal function tests, thyroid profile and nerve conduction study were carried out in all and investigations for secondary causes were tailored individually. All patients were treated with potassium supplementation and treatment of underlying cause. Results: The study included 15 female patients (male:female: 11:5) with total of forty eight patients aged 18-85 years (mean 33.62 ± 12.84). Secondary causes were detected in 33 (68.75%) patients, which include 15 patients with renal tubular acidosis, 8 with thyrotoxicosis and 3 with uncontrolled diabetes mellitus, 2 each due to alcoholism and dengue fever and 1 each with hypothyroidism and acute gastroenteritis. One patient was having acute pyrexia, cause of which could not be determined. Twelve patients were presented with atypical clinical features, which included paraparesis in 3, neck muscle weakness in 7 and facial weakness in 2 patients. Respiratory paralysis was present in three patients and none of them required ventilator support. Fifteen (31.25%) patients had Idiopathic hypokalemic paralysis, which included two patients with positive family history. 72.91% patients were presented in summer season. All patients dramatically improved with potassium supplementation. Patients with secondary causes had significantly more weakness, lower serum potassium levels and took longer time to recover. Conclusion: Majority (68.75%) of patients had secondary and potentially treatable causes of hypokalemic paralysis. The common causes were renal tubular acidosis and thyrotoxicosis. Thorough and specific investigations should be done in each patient to determine secondary causes.


Cardiac dysregulation in duchenne muscular dystrophy - An electrocardiogram analysis

S. Talakad, M. Rao, A. P. John, P. Kumar, A. Nalini, T. R. Raju

Department of Neurophysiology, NIMHANS, Bengaluru, Karnataka, India

Introduction: Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder. Arrhythmogenic cardiomyopathy is a frequent feature in DMD with an impending risk of Sudden Cardiac Death (SCD). Among all the investigations that are able to foresee the susceptibility to develop life-threatening arrhythmias, Electrocardiogram (ECG) is an accepted non- invasive measurement of Neuro- cardiac autonomic modulation. In this study we evaluated ECG of children with DMD. Methodology: This is a cross-sectional study. Lead II ECG recordings of 54 MLPA confirmed DMD were compared with 31 healthy volunteers. Data on DMD characteristics and cardiac co-morbidity were collected and general ECG variables assessed. ECG analysis was performed using AD Instruments. Bazett correction was used for the corrected QT interval (QTc). Independent samples 't ' test was performed to compare the study variables between the 2 groups using SPSS. Results: The mean age at presentation of DMD was 12.33 ± 0.6 years. Mean age of onset was 3.82 ± 2.07 years. Mean duration of illness was 4.62 ± 2.71 years. ECG analysis showed significantly reduced PR interval in children with DMD. The QRS and QT intervals were significantly increased in DMD compared to healthy children. The QTc was significantly decreased in patients but Tp-Te interval (p=0.010) was significantly increased. The P, Q and R amplitude were increased significantly along with ST height of ECG wave. Conclusion: ECG in DMD showed atrial hypertrophy changes (Increased P wave amplitude and QRS interval) indicating cardiac pathology in muscular dystrophy. An abnormally prolonged QT duration was observed implying an increased risk of ventricular arrhythmias. The significant shortened QTc in DMD might be related to the associated increase in ventricular rate and alteration of the autonomic tone. However the clinical significance of ECG abnormalities, observed in the present study in relation to the pathogenesis of SUDEP merits further investigation.


Nuclear mitochondrial intergenomic communication disorders - Phenotypic characterization of 44 patients from NIMHANS

S. Chiplunkar, P. Govindaraj, M. Purushottam, K. Sonam, R. Kumar, P. S. Bindu

Department of Clinical Neurosciences and Neuromuscular Laboratory, NIMHANS, Bengaluru, Karnataka, India

Background: Nuclear-mitochondrial Intergenomic communication disorders are characterized by multiple deletions in mitochondrial DNA (mtDNA). We have recently established long range polymerase chain reaction (PCR) to detect mtDNA deletions in skeletal muscle at neuromuscular laboratory, NIMHANS. Objective: To describe the phenotype of patients with nuclear- mitochondrial intergenomic communication disorders. Materials and Methods: Over a period of four years (2011-2015), a total of 430 patients underwent long range PCR in skeletal muscle tissue for suspected mitochondrial disorders. Mitochondrial DNA deletions were identified in 80 (multiple=63; single=17). Clinical, histopathological, biochemical and genetic information in 44 patients (M:F = 10:1, mean age: 26.5±18.1 years) were collated. Results: The phenotypes included Chronic Progressive External Ophthalmoplegia (CPEO) (n=13) and non-syndromic presentation (ataxia=9, myopathy =5, neuropathy =3, epilepsy=4, neuroregression=2 among others). Some of the patients had multiple symptoms. Serum lactate was elevated in 19 patients. Histopathological analysis showed COX deficient fibres only in 11 patients, while others had either no diagnostic pathology (n=12) or non specific findings (n=21). Respiratory chain complex (RCC) deficiencies were noted in 33 patients (Multiple complexes= 17, complex I =8, complex IV =4 and complex III=4). However there was no consistent correlation between RCC deficiency and phenotypes. Genetic analysis revealed mtDNA point mutations (6/16, novel=4, known=2), POLG1 (1/16, L304R) Clinical exome sequencing detected variations in 2/5 patients (APTX). Discussion and Conclusion: This study, first of its kind from India, highlight that patients with nuclear intergenomic communication disorders may have a predominant non syndromic presentation. It is noteworthy that several of these patients with non syndromic presentation did not have mitochondrial signatures in conventional diagnostic tests. Detecting mtDNA aberrations could be a potential, cost effective screening technique to identify intergenomic crosstalk defects which would guide a physician for targeted genetic testing.

Platform Session 06 Cognitive Disorders16:30-17:30 h, Friday, November 11, 2016Hall C


Cognitive reserve in frontotemporal dementia: Lifecourse factors play a role

S. Alladi, M. Shailaja, A. Rajan, B. Surampudi, J. R. Chaudhuri, S. Kaul

Department of Neurology, NIMHANS, Bengaluru, Karnataka, Karnataka, India

Background: Emerging evidence suggests that lifecourse factors influence clinical expression of dementia. Risk and protective factor studies have so far focussed on Alzheimer's disease and limited evidence is available for their role in Frontotemporal dementia (FTD). Aims: The aim of the study was to evaluate the potential impact of a range of lifecourse factors including education, occupation, bilingualism and urban/rural dwelling on the age at onset of FTD and its subtypes. Methods: 193 subjects evaluated in a memory clinic in Hyderabad, India underwent clinical evaluation, neuropsychological assessment and brain imaging. Diagnosis of FTD and its subtypes was made using FTLD consensus criteria. Results: Higher levels of education (p=0.044), bilingualism (p=0.017) and urban dwelling (p=0.042) were associated with a later age at onset of FTD. Among patients with behavioural variant FTD (n=67), higher levels of education (p=0.043), bilingualism (p=0.024) and urban dwelling (p=0.032) were associated with a later age at onset, while there was no association between any of these factors and language variants of FTD (progressive nonfluent aphasia and semantic dementia, n=62) and FTD syndromes with predominantly motor manifestations (Progressive Supranuclear Palsy, Corticobasal Degeneration and FTD-Motor neuron disease, n=64). Univariate general linear model showed that bilingualism was the only factor independently associated with age at onset of bvFTD (p=0.002). Conclusion: Our study adds to the body of evidence demonstrating a complex relationship between lifetime experiences and clinical expression of FTD. The differential role for individual factors such as bilingualism and education is relevant for understanding mechanisms underlying the concept of cognitive reserve in dementia.


Autonomic dysfunction in patients with Alzheimer's disease

N. Gupta, M. R. Rukmani, T. N. Sathyaprabha, S. R. Chandra

Department of Neurology, NIMHANS, Bengaluru, Karnataka, India

Objective: To look for evidence of cardiac autonomic dysfunction in Alzheimer's disease (AD). Rationale: Limbic structures are important components of central autonomic control, which undergo degeneration in AD. Acetylcholine is a major neurotransmitter of parasympathetic system and there is cholinergic depletion in AD. Methods: It is a prospective two group comparative study. 25 clinically probable Alzheimer's patients (Fulfilled Diagnostic and Statistical Manual of Mental Disorders (DSM) - 4 criteria and NINCDS-ADRDA criteria) were compared with 25 age and gender matched healthy controls. Short term heart rate variability (HRV), blood pressure variability (BPV) and baroreflex sensitivity (BRS) were assessed. Comparison between the groups was done using Mann-Whitney and Wilcoxon test. Spearman's correlation co-efficient was used to assess correlation between the disease severity and study parameters. Results: In Alzheimer's group, among frequency domain parameters of HRV, high frequency power in normalized units (HF nu) was low with p<0.05: low frequency power in normalized units (LF nu) and LF/HF ratio was high with p<0.05. Time domain parameters of HRV and various parameters of BPV and BRS did not show any significant difference between the groups. There was a negative correlation between HF nu and disease severity and a positive correlation between LF nu and LF/HF ratio and disease severity. Discussion: HF nu reflects parasympathetic activity; LF nu reflects predominantly sympathetic activity and LF/HF ratio reflects sympathovagal balance. An increase in LF/HF ratio reflects sympathetic dominance while a decrease in LF/HF ratio reflects parasympathetic dominance. Study results imply that there is a significant reduction in parasympathetic activity with sympathovagal imbalance with sympathetic dominance in AD. This may be due to central autonomic dysfunction as well as cholinergic depletion. In addition, cardiac autonomic dysfunction and disease severity are positively correlated.


A study on pattern of cognitive domain affection in different types of vascular dementia in hospital based setting

J. Ganguly, S. Pal, D. Basu, P. Sengupta, A. Pal, C. Chatterjee

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

Introduction: Vascular dementia (VaD) is one of the most common causes of dementia in the elderly, second only to Alzheimer's disease (AD). VaD can be classified as large vessel dementia, Small-vessel dementia, Ischemic-hypoperfusive dementia, Hemorrhagic dementia, mixed (usually with AD) and dementias resulting from specific arteriopathies. The presence of VaD remains largely unrecognized and untreated in the elderly. Aims and Objectives: To evaluate cognitive function in patients of vascular dementia and to characterize the cognitive domain affection in different types of vascular dementia. Materials and Methods: This observational study was conducted in Department of Neurology, Medical College, Kolkata and 25 patients fulfilling non-inflammatory neurological diseases NINDS-AIREN criteria of vascular dementia were included in the study. Patients underwent cognitive testing by using Kolkata cognitive screening battery (including BMSE) and other relevant tests. MRI brain was done in all patients. Statistical analysis was done using GraphPad Prism Software. Results: Of the total 25 patients, 8 patients (32%) had small vessel dementia, whereas 6 patients (24%) had large vessel dementia. Others had large + small vessel dementia (16%), hemorrhagic dementia (3%), AD-VaD overlap (16%). Mean age was 61.32 ± 8.12 years with male: female ratio of 17:8. Apart from memory (100%), problem in Executive tasks (88%) and Attention (80%) were noted in most patients. Other domains involved were Visuoconstructional (56%), Language (48%), Visuoperceptual (40%) and Calculation (40%). Other than Memory (100%), in small vessel dementia, cognitive domains mostly involved were Attention (100%) and Executive function (100%), whereas in large vessel dementia, Language (66.67%), Visuoconstructional (66.67%) and Executive function (66.67%) were mostly affected. Conclusion: Different cognitive domains are involved in VaD and different types of VaD may have predilection of particular domains.


Dementia free survival after bilateral subthalamic stimulation for Parkinson's disease

S. Krishnan, K. K. Pisharady, R. Rajan, G. S. Sarma, S. P. Sarma, A. Kishore

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

Background: Bilateral Subthalamic Nucleus Deep Brain Stimulation (STN DBS) is the standard of care in moderately advanced Parkinson's disease (PD) with motor complications and has been shown to improve activities of daily living and quality of life (QOL) remarkably. However STN DBS has no disease-modifying effects and can't postpone cognitive dysfunction and other DBS refractory symptoms stemming from relentless progression of PD pathology. The profound effects of dementia on QOL and functioning make it important to know the expected dementia free survival and the determinants of the same at the time of STN DBS. Aims and Methods: We did a retrospective review of systematically documented baseline clinical, demographic and neuropsychological data and the dementia status on follow-up, of the cohort of PD patients who underwent STN DBS at our center and had a minimum of two years of follow-up. Results: 116 patients with a mean duration of PD of 11.2 (Standard deviation - 4.2) years who underwent bilateral STN DBS at our center between 1999 and 2013 satisfied the inclusion criteria. During 536.0 person-years of follow-up, 30 patients developed dementia. The mean dementia free survival was 8.7 (95% Confidence Interval: 7.8-9.6) years. Analysis using a case-control study design revealed that those with earlier onset of dementia (dementia free survival ≤ 5 years) after STN DBS had longer duration of PD at the time of surgery, lesser improvement of activities of daily living and axial motor scores with levodopa, were more depressed and had worse fluency and executive functions at baseline compared to those who remained dementia free beyond five years of DBS. Baseline MMSE scores showed no significant difference. Conclusion: Earlier surgery before the onset of significant axial motor dysfunction is likely to result in a longer dementia free survival, in patients with PD considered for DBS.


The cognitive profile and executive function of progressive supranuclear palsy and multiple system atrophy patients

K. Chatterjee, S. Choudhury, M. U. Kulsum, S. Chatterjee, S. Shubham, H. Kumar

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

Introduction: Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are both degenerative diseases with Parkinsonian features. Cognitive profile might be distinct in these groups based on anatomical sites of involvement. Due to dearth of reports, we intended to explore the differential involvement of cognitive domains in these patients. Methodology: 8 MSA (diagnostic criteria of Gilman et al.) and 7 PSP (diagnostic criteria of Lees) patients were recruited till now in this ongoing study. They were screened using MMSE scale (MMSE score<18 were excluded). Cognitive parameters were assessed using Mattis-DRS and executive functions were evaluated using COWA, Stroop and n-back. PSPRS (PSP rating scale), UMSARS (Unified MSA rating scale) and UPDRSIII were used for estimation of motor impairment. Results: The age of onset (MSA 62 yrs; PSP 58.14 yrs) and the disease duration (MSA 41.25 months; PSP 30.86 months) for MSA group and PSP group were comparable. DRS total score did not show any statistically significant difference between groups, but we could appreciate a trend of higher DRS score for MSA (125.80) patients compared to PSP group (112.40). We observed a higher (though not significant) impairment of initiation/perseveration among PSP patients (raw score 26.20) compared to MSA (raw score 32.60) patients. Attention domain had also shown a similar trend of higher deficit in PSP group. The disease specific motor scales were largely in agreement with the UPDRS III scores while assessing the motor severity. Conclusion: This is the first report comparing the cognitive profile of PSP and MSA patients in this ethnic population. From the data yet analysed we got a suggestive trends of higher cognitive impairment in PSP groups, specifically involving the attention and initiation/perseveration domains. The study is ongoing and we may get more robust correlation with recruitment of more patients.


Neuro behavioral abnormalities in advanced dementia

R. Mathew, Sauda

Department of Neurology, Mookambika Institute for Medical Sciences, Trivandrum, Kerala, India

Introduction: Neurobehavioral abnormalities vary at various stages of dementia. Aim: To compare the neurobehavioral abnormalities of advanced dementia with that of early stage dementia. Methods: Prospective cross sectional study. Setting: Data from patients availing dementia care services from 3 different teretiary care centres were analyzed. All the patients were administered a neuropsychology test battery comprising of ACE-R, MMSE, ADL, HADS, IQCODE, CBI, Trail A and B. Results: 184 patients were selected during the period 2010 -2014. Based on ACER score they were classified as stage 1 (ACER ≥50 N 68), stage 2 (ACER 25-49 N 49) and stage 3 (ACER<25 N25). Patients in stage 3 were analyzed. Mean age was 72.41 SD 9.8, 62% males. Mean education 6.47SD4.65. Mean duration of illness at the time of presentation 2.42 (SD1.8) years. Cambridge behavioral inventory (CBI) was used to assess neurobehavioral abnormality. For purpose of comparison the total CBI score for each domain was converted into percentage of maximum score (PMS). The impairment at early stage as well as advanced stage was maximum for memory (PMS 46.91SD24.79vs48.07SD28.79). The least impaired domain was eating behavior at initial stages as well as in advanced stage (PMS21.61SD23.64vs21.87SD22.49). As cognition worsens worsening was seen in all the behavioral domains except in stereotypy and motor behavior. The amplitude of change was maximum for selfcare (14.43)and least for stereotypy and motor behaviour (−0.90). No correlation was seen between ACER total score and CBI total score (Pearson correlation p0.45). Conclusion: In this cohort of hospital based patients with advanced dementia neurobehavioral abnormalities appear more or less similar to that seen in early stages.

Platform Session 07, Clinical Neurophysiology08:30-09:30 h, Saturday, November 12, 2016Hall A


Central hypersomnolence - A case series from South India

M. Trivedi, S. E. Sreedharan, A. Radhakrishnan

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

Background: Narcolepsy and idiopathic hypersomnolence (IHS) are rare disorders. In Western populations, the reported prevalence of narcolepsy is 0.02%-0.05% From the Indian subcontinent, there are only below 4 publications on narcolepsy so far. Aim: We looked at the clinical and polysomnographic profile as well as their treatment outcomes of Narcolepsy/IHS. Materials and Methods: Patients presenting with excessive daytime somnolescence (EDS) attending sleep clinic from January 2010 to December 2015 were included. Patients were diagnosed with Narcolepsy type 1, Narcolepsy type 2 and IHS based on the ICSD-3 criteria. Patients with secondary causes of EDS were excluded. Results: Total 56 patients were included in the study (29 males, 27 females). The mean age of symptom onset was 29 years (males - 34 years, females - 24 years). 12 (21%) patients had Narcolepsy type 1, 5 (9%) patients had Narcolepsy type 2, while 38 (68%) patients had IHS. The average time from symptom onset to diagnosis was 71 months. The mean sleep onset latency was 3.1 minutes, while the mean REM latency was 7.2 minutes. Among those with IHS, 7 patients had cataplexy but did not satisfy the MSLT criteria for narcolepsy. Majority of subjects were treated with Methylphenidate with good symptomatic improvement. Conclusion: Patients presenting with EDS without any obvious cause, must undergo evaluation to rule out narcolepsy. There is significant delay in diagnosing narcolepsy indicating the need to increase awareness among physicians about this rare treatable disorder.


Complications in mechanically ventilated patients of Guillain Barre syndrome and their prognostic value

A. B. Netto, A. B. Taly, G. B. Kulkarni, G. U. Rao, S. Rao

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

Introduction: The spectrum of various complications in critically ill Guillain Barre Syndrome (GBS) and its effect on the prognosis is lacking in literature. This study aimed at enumerating the complications in such a cohort and their significance in the prognosis and mortality. Materials and Methods: Retrospective case record analysis of all consecutive mechanically ventilated patients of GBS in neurology ICU of a tertiary care institute for 10 years was done. Demographic, laboratory, treatment details and outcome parameters were recorded. Results: Among the 173 patients were 118 men and 55 women (2.1:1), aged 1 to 84 years. The average number of ICU complications per patient was 6.8 ± 1.8 (median =7, range = 1 to 12). The commonest complication was tracheobronchitis (128). Other pulmonary complications were found in 36 patients. The next was metabolic hyponatremia (115), hypokalemia (67), hypocalcemia (13), stress hyperglycemia (10), hyperkalemia (8), hypernatremia (9), Sepsis (40), Urinary Tract Infection(UTI) (47), dysautonomia (27), hypoalbuminemia (76), anaemia (75), seizures (8), paralytic ileus (5), bleeding (4), anoxic encephalopathy (3), organ failures (12), deep vein thrombosis (7) and drug rashes (1) were also noted. The complications, considered significant in causing death, Hughes scale ≤3 at discharge, prolonged mechanical ventilation (>21 days) & hospitalization (>36 days) were pneumonia, hyponatremia, hypokalemia, urinary infection, tracheobronchial infections, hypoalbuminemia, sepsis, anemia dysautonomia. Conclusion: Active monitoring and appropriate and early intervention by the clinician will improve the quality of life of these patients and reduce the cost of prolonged mechanical ventilation and ICU stay.


A study of sleep disturbances in epilepsy including drug naïve epilepsy

G. Godhavarma, K. Bala, S. Kushwaha, A. A. Dung, B. Kaul

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

Aim: Sleep disturbances are a common complaint in people with epilepsy and the co occurrence of sleep disorders and epilepsy is a known fact. The interactions between them are very complex and the role of antiepileptic drugs is difficult to gauge. The present study aims to evaluate the sleep disturbances in patients with epilepsy and relate with AED treatment. Methods: It's a questionnaire based study using Epworth Sleepiness Scale (ESS), Sleep Apnea Scale of Sleep disorders Questionnaire (SA-SDQ), Restless legs syndrome scale, Insomnia Severity Index, Pittsburg Sleep quality index (PSQI). A Total of 200 consecutive cases of epilepsy and 200 age and sex matched controls were enrolled into the study. The data was collected, compiled and analyzed using appropriate statistical methods in SPSS 17. Results: The mean duration of seizures was eight years. Generalized seizures were reported in 52% had and 44% had partial seizures. In our study 20.5% cases were not on any treatment prior to enrollment. Poor control of seizures was observed in 13% and good control in 66% of cases. History of EDS was reported by 33% of cases as compared to 17% controls. ESS scores, Sleep apnea scale score, Insomnia severity index and RLS scale were significantly different between the cases and controls (p=0.000). The treatment group differed significantly from those who were not on treatment for all the parameters studied. Poorly controlled seizures significantly differed from well controlled seizures in SA-SDQ and Day dysfunction of PSQI Scale. Significance: Sleep disturbances were more common in people with epilepsy than in the general population and treated more than untreated population.


An audit of the electro-clinical, aetiology and prognostic factors in patients with alpha coma

A. H. Patanvadiya, B. Chauhan, G. K. Dash

Department of Neurology, Mazumdar Shaw Medical Center, Narayana Health City, Bengaluru, Karnataka, India

Aim: To audit the clinical, etiological and prognostic variables in patients with alpha coma at a tertiary care hospital in South India. Methods: Between the years 2013 to 2016, we identified seven patients with alpha coma and analysed their clinical, EEG, etiological and prognostic factors. Outcome at discharge was documented in the form of modified Rankin Scale (mRS), with mRS more than 3 being poor outcome. Results: The etiology of alpha coma was post cardiac arrest hypoxic encephalopathy (3, 42.85%), metabolic encephalopathy (2, 28.5%) and drug intoxication (2, 28.5%). Clinically, Glasgow Coma Scale (GCS) was 8 or less (deep coma) in 6 cases (86%) and more than 8 (light coma) in 1 case (14%). Brain-stem reflexes were present in 5 out of 7 cases (71.5%). EEG in all cases showed diffuse alpha pattern, non-reactive to noxious stimuli. Five patients (71.5%) had poor outcome, which included all 3 cases of hypoxic encephalopathy and 1 case each of metabolic encephalopathy and drug intoxication. Conclusions: Majority of the cases with alpha coma had poor outcome (5/7, 71.5%). Neither the clinical variables nor EEG reactivity influenced the clinical outcome. Similar to prior studies, underlying etiology of alpha coma probably determined the severity of clinical outcome. Hypoxic encephalopathy was the most common etiology and was associated with poor outcome.


Sleep abnormalities in juvenile myoclonic epilepsy - A sleep questionnaire and polysomnography based study

S. Roshan, V. Puri, N. Chaudhry, A. Gupta

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

Background: Few studies have evaluated the sleep quality and architecture in patients of JME, and are limited by small numbers and confounding effect of antiepileptic drugs (AED). Objective: To evaluate sleep quality and architect in drug naïve and on treatment patients with Juvenile myoclonic epilepsy (JME). Materials and Methods: 99 patients of JME (36 drug naïve, 63 on AED), and 30 healthy controls were recruited. Sleep quality was evaluated with Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS) and NIMHANS comprehensive sleep disorders questionnaire. Overnight polysomnography (PSG) was done to access sleep architect. Scoring of sleep and related events was done manually as per the AASM 2012 version 2. Results: Mean age of patients was 21.3 ± 6.0 years, 66.7% were females. JME patients had significantly poor quality of sleep by PSQI (p = 0.02) and ESS (p=0.05). PSG revealed reduced sleep efficiency (p<0.001); increased sleep latency (p=0.02); increased %WASO (p<0.001); increased %N1 (p=0.01) and decreased % REM sleep (p=0.02) in patients, compared to controls. Sleep quality and architect were similar between drug naïve and on treatment groups, except that, the AED group had lower %N3 sleep compared to drug naïve group (p=0.002) and also, a lower N2 arousal index (p=0.049). Discussion: An absent to decreased %REM and increased %N1 sleep in a significant number of patients, along with instability of light NREM sleep in the drug naive group was noted. Conclusion: JME is associated with poor sleep quality and altered architect, irrespective of treatment status.


Utility of exome sequencing in adults with neuro-genetic disorders

M. Nagappa, P. S. Bindu, A. B. Taly, S. Sinha, P. S. Mathuranath

Department of Neurology, NIMHANS, Bengaluru, Karnataka, India

Background: There are limited studies of exome sequencing in neurological disorders from India. Aims and Objectives: To explore role of exome sequencing in delineating molecular basis of uncommon neuro-genetic syndromes. Methodology: Over two years, 176 patients underwent targeted exome sequencing for suspected neuro-genetic disorders. Prediction of possible impact of identified variants was done using online tools (Polyphen2, SIFT, Mutation Taster). Results of 38 adults (M:F 28:10, mean age = 29.63 + 11.5 years ) were analysed and grouped based on phenotype-genotype correlations. Results: Fifty variants in 34 genes were detected among 27 patients. In group I, patients (n=14) had mutations in disease-causing genes (i) homozygous (novel=4, recognised=2), (ii) heterozygous (novel=2, recognised=1), and (iii) compound heterozygous (novel variants: one allele=4, both alleles=1). Their clinical phenotypes were metabolic disorders=4, HSP=3, hereditary vasculopathy=3, NBIA=2, and neuromuscular disorders=2. Group II (n=4) with variants in two different genes associated with clinical syndrome included: MPZ and DNMT in neuropathy, COL6A3 and TTN in muscular dystrophy, NOTCH3 and COL4A1 in vasculopathy, and GPR98 and SCN9A in myoclonic epilepsy. Group III (n=6) included hitherto undescribed phenotype-genotype associations: (i) ataxic syndrome and mutations in WDR81, CDH23, C10ORF1 and SLC2A1, (ii) leukoencephalopathy and SBF2 mutation, and (iii) MND and SCN11A mutation. Significance of mutations in groups II and III need verification by segregation analysis and gene-expression studies. In three patients, variants were considered 'benign' as they were present in unaffected family members (n=2) or in heterozygous state (n=1). Additional nine 'benign' variants were detected in seven patients along with pathogenic mutations. In 11 patients no mutations were identified (neuropathy=4, ataxic syndromes=2, leukoencephalopathy=3, stroke=1, extrapyramidal syndrome=1). They require deep sequencing. Conclusion: Exome sequencing has transformed evaluation and understanding of neuro-genetic disorders, guiding therapy and prognosis. Detection of new variants expands genetic spectrum in Indian context. Being expensive, its utility in routine neurological practice requires critical analysis.

Platform Session 08, Infections08:30-09:30 h, Saturday, November 12, 2016Hall B


A clinicoradiological profile of varicella zoster encephalitis - A short case series

S. Osman, M. Y. Reddy, N. V. Chaudhary, S. K. Jaiswal, S. Parida, J. M. K. Murthy

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

Introduction: Varicella zoster virus (VZV) is an uncommon cause of acute viral encephalitis, accounting for 10% of cases. However little is known about the clinical characteristics and outcome of VZV-encephalitis. Materials and Methods: Retrospective analysis of case records of patients with VZV-encephalitis admitted between April 2014 and March 2016. Diagnosis VZV-encephalitis was based on presence of VZV antigen in CSF or VZV antibodies in serum (IgM) and clinical evidence of herpetic skin lesions. Results: During the study period six patients (mean age 67.5 years; range 55-77 years; M:F: 1:1) were admitted. Duration of the symptoms was 4 to 25 days. Of the 6 patients, 4 had diabetes and 1 had rheumatoid arthritis. Presenting features included: Fever (3); Headache (1); Altered sensorium (4); Seizures (2); Ataxia (2), Headache and decreased hearing acuity (1). The clinical syndromes were: meningoencephalitis in 5 (one with deafness and 2 with cranial neuropathies) and cerebellitis in one. MRI findings were leptomeningeal and seventh nerve enhancement in one patient and leptomeningeal and patchy parenchymal enhancement in one patient. All patients had abnormal CSF: pleocytosis and elevated protein. All patients received IV acyclovir for 10 days and two patients received IV methylprednisolone in addition. Conclusion: VZV-encephalitis is apotentially treatable encephalitis and should be considered in patients with encephalopathy and CSF pleocytosis.


Spectrum of central nervous system tuberculosis and its correlation with outcome

R. N. Ingole, R. K. Garg, R. Verma, H. S. Malhotra, P. K. Sharma, A. Jain

Department of Neurology, KGMU, Lucknow, Uttar Pradesh, India

Background: CNS tuberculosis is the deadliest form of extrapulmonary tuberculosis. The spectrum is wide involving meninges (Tuberculous meningitis), brain parenchyma (Tuberculoma and tubercular abscess), spinal cord (Pott's spine, tuberculoma, abscess, myelitis) and as a disseminated form (CNS with extra-CNS involvement). This study was planned to look for spectrum of CNS tuberculosis and its correlation with outcome. Methodology: This study was conducted (between august 2014-july 2016) to determine CNS tuberculosis spectrum, their clinical presentation and short-term outcome (3 months). Outcome assessment was also done for bacteriologically proven cases and disseminated group. Results: After screening and exclusion, 303 patients were included in the study of whom 178 (58.74%) were male and 125 (41.26%) were females. There were 171 (56.44%) patients of intracranial tuberculosis, 54 (17.82%) patients of spinal tuberculosis, 15 (4.95%) patients of both intracranial and spinal tuberculosis and 63 (20.79%) patients of disseminated tuberculosis. In intracranial tuberculosis group, 128 (74.9%) had tubercular meningitis, 9 (5.3%) had tuberculoma, 5 (2.9%) had tubercular abscess and 29 (16.9%) were having multiple intracranial pathology. In spinal tuberculosis group, 42 (77.8%) had Pott's spine, 10 (18.5%) had arachnoiditis and 2 (3.7%) were having tuberculous myelitis. Bacteriological confirmation rate was higher in disseminated tuberculosis group (58%) as compared to intracranial tuberculosis group (34.4%). Outcome at 3-month follow up was poorer (death or Modified Barthel Index <12) in disseminated tuberculosis group (43.36%) as compared to intracranial (27.12%) and spinal tuberculosis groups (31.56%). Conclusion: One-fifth patients of CNS tuberculosis have disseminated tuberculosis with higher rate of bacteriological confirmation and poorer outcome.


Evaluation of neuroendocrine dysfunction in cases of tuberculous meningitis

A. V. More, R. Verma, R. Garg, M. Mittal, Hardeep, S. Malhotra, N. Kumar

Department of Neurology, King George Medical College, Lucknow, Uttar Pradesh, India

Background: Neuroendocrine dysfunctions have been reported in tuberculous meningitis (TBM) due to basal exudates surrounding pituitary gland or direct involvement of hypothalamic pituitary axis by tuberculous process. We studied occurrence and pattern of neuroendocrine deficiencies in cases of TBM. Methodology: Our study is a cross-sectional observational study carried out in Neurology Department of King George's Medical College, a tertiary care referral hospital in Lucknow. Hormonal assays and imaging of hypothalamic pituitary axis was performed in newly diagnosed TBM patients between August 2014 and July 2016. The gonadotropic axis (Follicle stimulating hormone (FSH), Luteinizing hormone (LH), Testosterone, Estrogen, Progesterone levels), thyroid axis (Thyroid stimulating hormone (TSH), free T3, free T4 levels), Growth hormone (GH) levels, corticotropic axis (Adrenocorticotropic hormone (ACTH) and cortisol levels) and prolactin levels were evaluated along with evaluation of posterior pituitary functions by looking for evidence of SIADH and Diabetes Insipidus. Cases with preexisting endocrine disorders and those already on corticosteroids were excluded from the study. Results: A total of 100 patients (56 males and 44 females, age range 8-76 years) of newly diagnosed tuberculous meningitis were studied. Fifty-six patients had hormonal deficiencies out of which 26 had involvement of more than one pituitary axis and 44 cases had no hormonal deficiency. The commonest hormones found to be deficient were gonadotropins (n=38) followed by TSH (n=19), ACTH (n=14), and GH (n=8) whereas hyperprolactinemia was found in 26 patients. SIADH was noted in 11 cases. None of patients had Diabetes Insipidus. On brain imaging, 21 patients showed basal exudates around sellar region and 3 patients showed direct involvement of hypothalamic-pituitary axis by tuberculomas. Conclusions: Endocrine dysfunction was found in 56% of tuberculous meningitis patients out of whom 26% had multiple pituitary hormonal dysfunctions.


Evaluation of consensus criteria for diagnosing paediatric neurotuberculosis

C. Susha, B. Smita, M. Anand, V. A. Gafoor, A. V. Gopalan, J. Jose

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

Background: Tuberculous meningitis is the most common cause of death from TB. Early diagnosis and treatment is the most important factor influencing the recovery without neurological sequelae. Recently consensus criteria for early diagnosis of tuberculous meningitis have been developed and published in Lancet Infectious Diseases, November-2010. This criteria need validation in Indian population, a country with high incidence of TBM. Objectives: This study tests the validity of consensus criteria for early diagnosis of tuberculous meningitis in children. It also assesses the usefulness of adenosine deaminase assay (ADA) and CSF-PCR in diagnosing TBM. Materials and Methods: 23 children who satisfied the entry clinical criteria were enrolled into the study. CSF examination was done in all the subjects. Scoring points were given to each individual according to the consensus criteria and was classified into definite, probable, possible and not TBM groups. All the children were reassessed every 2 months for 12 months. The consensus criteria were evaluated against response to treatment and CSF-PCR using statistical methods. The diagnostic value of ADA was assessed by calculating the area under the receiver operating characteristic (ROC) curves. Results and Discussions: Validation against response to treatment showed for definite criteria, the sensitivity was 37.5% and specificity 100%. The positive and negative predictive values were 100% and 41.2% respectively. When definite and probable criteria were combined together, the sensitivity was 56.3% and specificity 85.7%; the positive and negative predictive values were 90% and 46.2% respectively. For the whole of the consensus criteria, the sensitivity was 87.5% and specificity 28.6%; the positive and negative predictive values were 73.7% and 50% respectively. Validation of the consensus criteria classes probable and possible against CSF- PCR showed 100% sensitivity for consensus criteria. Sensitivity of CSF PCR for diagnosing TBM was 37.5%, specificity was 100% (p value 0.05) and the sensitivity of CSF ADA for diagnosing TBM was found to be 31.3% and specificity 100% (p value 0.05). Conclusion: The consensus criteria have high sensitivity (87.5%) and are an effective screening tool for diagnosing TBM. Validation of the consensus criteria against CSF PCR shows 100% sensitivity for consensus criteria in picking up suspected TBM cases and reaffirm its effectiveness as a high quality screening tool. CSF ADA has comparable specificity to CSF PCR and can be used as a cost effective investigation in diagnosing TBM.


Assessment of gene xpert MTB/RIF test in the diagnosis of CNS tuberculosis

M. D. Thakkar, V. Mittal, A. K. Singh, P. K. Maurya, D. Kulshreshtha, A. K. Thacker

Department of Neurology, Ram Manohar Lohia Hospital, Lucknow, Uttar Pradesh, India

Background: The Gene X pert MTB/RIF test is a recent addition in the evaluation of suspected Tubercular meningitis. It not only confirms the diagnosis but also shows Rifampicin resistance. It provides quick results and is easily available because of the WHO installed Gene X pert MTB /RIF machines at various levels. Objective: To study the sensitivity & specificity of Gene X pert MTB/RIF in suspected CNS tuberculosis. Methods: 58 patients were evaluated where CSF Gene X pert MTB/RIF test was requested. These included patients with suspected TBM (group A) and those where the diagnosis of CNS Tuberculosis was planned to be excluded (group B). CSF was subjected to routine cytology, biochemistry along with smear for AFB, MGIT and Gene Xpert MTB/RIF & CALAS. Sensitivity and specificity of gene X pert was calculated. Results: Of 58 patients 32 were from group A and 26 were from group B. 11 patients from group A had Gene Xpert positive and all the patients of group B had negative Gene X pert. All patients from both groups were negative for smear for AFB, MGIT & CALAS. Sensitivity of Gene X pert test was 33.33% 95% CI (17.96% - 51.83%) in CSF samples tested, with a specificity of 100% 95% CI (86.17%-100%). Negative predictive value of the test was 54.17% 95 % CI (39.17- 68.63%). None of the patients showed Rifampicin resistance. Discussion and Conclusion: Gene X pert MTB /RIF is a useful test with 100% specificity, though the sensitivity in suspected sub acute meningitis is low.


NMO/NMO-SD in the Indian context- is it different?

J. Sachdeva, V. Lal, M. Goyal, A. Takkar

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

With the advancement in therapeutic and diagnostic armamentarium, the spectrum of neuromyelitis optica (NMO) in India has witnessed a momentous change. As NMO/NMO-SD as an entity, is undergoing significant changes (from diagnostic criteria to therapeutic options available); it is prudent to diagnose and treat patients early in the course of the disease. With this objective, we share our experience of NMO/NMO-SD patients from our ongoing "Neuro-ophthalmology registry". There are very few studies from India available on the clinic-epidemiological profile of this disabling disease. This is a retrospective and prospective data of 72 patients of NMO/NMO-SD describing the epidemiological features, clinical presentation, recurrence rates, radiological and serological profile, treatment and response to therapy. The median age of the patients in our data was 29.5 ±11.9 years comprising of 45 female and 27 male patients. 62.5% patients had a relapsing disease with a maximum number of relapses being 7. More than 90% of patients were given an alternative diagnosis before presenting to us. The time between onsets of symptoms to diagnosis was 1-6 months in the majority of patients reflecting the need for increasing the awareness of this disabling disease. 49.2% patients were seropositive for aquaporin antibodies. Though the data (being ongoing) is currently variable in follow up, we present salient features in terms of nature of the disease, relapses, time of onset to diagnosis, time of onset to the maximum deficit and impact of all these factors on the prognosis. Our data also reveals the differences in our set of patients with regards to epidemiology, the social bearing of the disease, treatment strategies, response to treatment and prognostic markers as compared to various other studies published worldwide.

Platform Session 09, Autoimmune Disorders08:30-09:30 h, Saturday, November 12, 2016Hall C


Autonomic dysfunction in Multiple System Atrophy: An experience at a tertiary care hospital

V. Kataria, A. Srivastava, V. Goyal

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

Objective: The objective of this study was to prospectively analyze patients with Multiple System Atrophy (MSA) to look for any differences in autonomic disturbances among the two variants (MSA-Parkinsonism and MSA-Cerebellar). Methods: We prospectively examined 45 patients with probable Multiple System Atrophy (23 MSA-P and 22 MSA-C) at a tertiary care hospital from March 2015 to November 2015. All patients were examined by single investigator with Movement disorder society-unified Parkinson's disease rating scale part III (MDS-UPDRS), unified MSA rating scale (UMSARS) and Scale for Outcomes in Parkinson's disease - Autonomic (SCOPA-AUT). Descriptive statistics were used to analyse the data. Mean and Standard deviation were calculated for normally distributed data. All continuous variables were analyzed using unpaired t-test. Results: The mean age of patients at disease onset was significantly higher in MSA-P (63.3 ± 9.1 years) than in MSA-C (52.6 ± 7.9 years) (p<0.001). However, the disease duration was similar in both the groups (36.5 ± 26.4 months in MSA-P and 35.1 ± 19.6 months in MSA-C, p=0.84). There were 16 males in MSA-P and 18 males in MSA-C group. The mean MDS-UPDRS part III score was significantly higher in MSA-P group (51.2 ± 16.3) than MSA-C group (35.2 ± 13.0) (p<0.001). However, there was no significant difference in total UMSARS part I, II & III score (52.5 ± 16.8 in MSA-P and 48.4 ± 12.4, p=0.35). In UMSARS part III score, there was no significant difference in orthostatic change in blood pressure and heart rate. Similiarly, there was no significant difference in SCOPA-AUT (25.1 ± 12.8 in MSA-P and 21.8 ± 10.1 in MSA-C group, p=0.34). Conclusion: In this prospective cohort, patients with MSA-C were younger at disease onset but there were no significant differences in MSA rating scale and autonomic scale in between the two groups.


Clinical, electrophysiological and radiological study of optic neuropathies

S. Bawri, M. Das, M. Goswami, L. J. Basumatary, S. Das, A. K. Kayal

Department of Neurology, Gauhati Medical College, Guwahati, Assam, India

The optic nerve contains axons of nerve cells that emerge from the retina, leave the eye at the optic disc and go to the visual cortex where input from the eye is processed into vision. Optic neuropathy refers to damage to the optic nerve due to any cause. Materials and Methods: (1) Ongoing prospective study conducted from august 2014-June 2016. (2) Aim is to evaluate the causes of optic neuropathies. (3) All adult non traumatic patients with optic nerve involvement were included. Results and Observation: A total of 96 patients were included among which 54 (56.25%) were male and 42 (43.75%) were female. Average mean age was 35 ± 10 years and duration of symptom was 2.4 weeks ± 4 days. Most common clinical features were visual loss (96%), colour vision loss (62%), headache (41%). Associated features were paraparesis (13.5%), Lhermitte sign (7%) hemiparesis (4%) and quadriparesis (4%). Relevant positive investigations were anti-aquaporin-4 antibody (8%), Antinuclear Antibody (3%) in serum and oligoclonal band (4%), Angiotensin Converting Enzyme (3%), venereal disease research laboratory (3%) in cerebrospinal fluid. Visual Evoked Potentials revealed mixed demyelinating and axonal (9.6%) and demyelinating pattern (91.4%). Optical coherence tomography revealed decreased retinal nerve fibre layer (91.4%), macular oedema (5%) and normal (3.6%). Neuroimaging revealed optic neuropathy (71%) and normal (29%). Out of 96 patients, 31.25% were unspecified demyelinating etiology, 11.45% were multiple sclerosis, Neuromyelitis optica spectrum disorder (17.70%), Leutic (3.125%), Sarcoidosis (3.125%), Nutritional (4.166%), Toxic (8.33%), Ischaemic (12.5%) and others (8.35%). Discussion and Conclusion: Visual loss due to visual pathway dysfunction can occur in a variety of clinical setting. In a tropical country like India, apart from the usual suspects, nutritional, toxic, infective should be considered. Multiple sclerosis is moderately prevalent in India. However, the differential diagnosis is extensive. Prognosis and treatment depends on the cause.


Retrospective study of long extensive transverse myelitis at tertiary care centre in North India

L. B. Bhirud, S. Kushwaha

Department of Neurology, IHBAS, New Delhi, India

Objective: To evaluate clinical profile, aetiology and management of long extensive transverse myelitis (LETM). Background: LETM is a characteristic manifestation of neuromyelitis optica (NMO). However, not all patients with LETM are positive for aquaporin-4 (AQP4) antibodies. We try to evaluate the causes of LETM. Methods: Clinical History, Neurological Examination, laboratory investigations including CSF study and Neuroimaging and Follow up. Results: Among 20 patients presented with LETM in year 2012-2014, in Neurology department of IHBAS Delhi, 5 were female and 15 were male patients (F:M=1:3). Age ranged from 12 to 57 years (average 29.4 years). These patients were undergone routine haematological, renal, liver functions tests and CSF studies including TB PCR and M.TB culture, ANA, anti-dsDNA antibody including anti-aquaporin 4 antibody, MRI Brain imaging and electrophysiological testing including Visual evoked potentials (VEP) and Brainstem auditory evoked response (BAER) studies. We found 5 patients with tubercular, 2 patients with NMO, 6 patients with subacute combined degeneration due to vitamin B12 deficiency, 1 patient with sjogren syndrome and 1 patient with SLE as cause for LETM. In 5 patients etiological diagnosis was not determined. Conclusions: LETM may be manifestation of a systemic disease and has various causes in itself. To note in this study that tuberculosis of spinal cord presenting as LETM should be considered as one of the treatable condition.


Comparison of the clinical profile of patients with anti NMDA-R and anti VGKC encephalitis in a tertiary care centre

S. Perumal, V. Shankar, P. Hazeena, T. Sugumar

Department of Neurology, Sri Ramchandra University, Chennai, Tamil Nadu, India

Introduction: Autoimmune encephalitis is a treatable cause of subacute onset of cognitive decline and confusion. Autoantibodies can be divided into those that target the extracellular domains of membrane molecules and those against intracellular targets. Common antibodies are Anti VGKC (LGI1 and CASPR2), NMDAR and GAD. Aim: To compare the clinical profile of patients with Anti NMDA-R and Anti VGKC encephalitis in a tertiary care centre. Methods: Study was done among 96 patients admitted under neurology department in Sri Ramachandra medical college with features of behavioural disturbances, cognitive decline and new onset seizures over 1 year. Detailed clinical evaluation, csf analysis, neuroimaging and Anti NMDA-R and Anti VGKC antibodies were done. Patients positive for Anti NMDA-R and Anti VGKC were Included. Results: Among the 96 patients, 11 patients were positive for autoimmune antibodies. 4 of them were positive for Anti VGKC LGi1 antibody, 1 for Anti VGKC - CASPR2 and 6 patients were positive for Anti NMDA-R antibodies. Patients with NMDA- R encephalitis were of younger age group (1 st and 2 nd decade) compared to VGKC encephalitis (3 rd to 8 th decade). Aggressive behaviour, speech disturbances and hallucinations being the most commonsymptoms in NMDA group, whereas cognitive decline (4 of 5 patients), faciobrachial dystonic seizures and hyponatremia (2 of 4 with LGi1 encephalitis) in vgkc encephalitis. 8 of them had abnormal EEG. Abnormal MRI was seen in 4 of 11 patients. In our study, patients with VGKC encephalitis showed good response with steroids and Iv immunoglobulin, whereas NMDA-R encephalitis required plasmapheresis and immunomodulators in addition to steroids and had more morbidity. Conclusions: Autoimmune encephalitis can be subdivided into diseases with different antigens and these often show slightly different responses to treatment. Given the good prognostic outcome with prompt initiation of immunotherapy, early recognition and diagnosis of this rare neuropsychiatric syndrome is crucial.


South Indian perspective of disease modifying therapy in patients with multiple sclerosis: A single centre observational study

C. Kona, C. Kona, S. Kumar, A. Kumar

Department of Neurology, AIMS, Kochi, Kerala, India

Introduction: Multiple Sclerosis (MS) is a common demyelinating disease causing disability in adults. Disease Modifying Therapies (DMT) are the main stay of treatment. However in emerging countries there are many hurdles to this treatment with regards to availability and cost. The present study attempts to look at the various DMTs available in the country and our experience with them. Methods: This study was done in the MS Clinic of a tertiary care hospital in Kochi. All patients with Relapsing and Remitting (RRMS) on DMTs were included in the study. Required information was collected from hospital database. The diagnosis of RRMS was made using Mc Donalds 2010 criteria. Progressive MS were excluded. Results: Out of 152 patients in our MS Clinic, 41 (26.9%) RRMS patients on DMTs were analysed. 26 females and 15 males, 2 patients were lost to follow up. Mean age at onset of symptoms was 36.2 years. The mean time to initiation of DMT from the first symptom was 32.57 months. Commonly used therapies were Interferon beta 1a intramuscular in 26 patients (63.4 %), Interferon beta 1 a subcutaneous in 6 (14.6 %), Glatiramer acetate in 4 (9.8 %), Natalizumab 3 (7.3%) and others 1 (2.4%). We made no attempt to compare the relative efficacies of the various DMTs. 4 patients had clinical relapse while on treatment and 5 had radiological progression. 17 patients are continuing DMT till date. Mean time of discontinuing the therapy was 26.77 months from initiation. Most common cause for discontinuation for DMT was due to financial constraints, others included altered LFT and decision for conceiving. Those who stopped prematurely some were continued on azathioprine or mycophenolate mofetil. Conclusions: Patients affording DMTs are few as high costs hinder their continuous therapeutic use. There is a considerable delay in initiation.


Spectrum and predictors of refractory status epilepticus in a developing country

U. K. Misra, J. Kalita, S. K. Bhoi, D. Dubey

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

Aim: Refractory status epilepticus (RSE) can influence the outcome of status epilepticus (SE). In the present study, the etiology predictor's outcome of RSE in a developing country is reported. Methods: It is a prospective hospital based study SE patients (continuous seizures for 5 min or more). Those who had SE persisting after 2 antiepileptic drugs (AEDs) were defined as RSE. The demographic information, duration and type of SE, its severity using status epilepticus severity score (STESS), etiology, comorbidities and imaging findings noted. Outcome of RSE was defined as cessation of seizures and condition on discharge as assessed by modified Rankin's scale (mRS). Results: 35 (42.5%) out of 81 patients had RSE. Median duration of SE before starting treatment was 2 (0.008-160) hours. The common causes of RSE were stroke in 5 (14.3%), central nervous system (CNS) infections in 12 (34.3%) and metabolic encephalopathies in 13 (37.1%) patients. 21 (60%) patients had comorbidities and STESS was favorable in 7 (20%) patients. 14 (20%) patients died but death was directly related to SE in 1 patient only. Ten patients had super refractory status epilepticus (SRSE) which was due to CNS infection in 5 (50%) and metabolic encephalopathy in 3 (30%) patients. On multivariate analysis, unfavorable STESS (p=0.05) duration of SE before treatment (p=0.01) predicted RSE. Metabolic etiology (p=0.05), mechanical ventilation (p<0.001) and age >60 years (p=0.003) predicted poor outcome. Conclusion: RSE occurred in 42.5% patients with SE. Of the RSE patients, 40% died and poor outcome was related to age above 60 years and metabolic etiology.

Platform Session 10, Epilepsy16:30-17:30 h, Saturday, November 12, 2016Hall A


Monthly epilepsy camp SVIMS Tirupati experience 1999-2015

A. R. Anumolu, B. Vengamma, T. Naveen

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

Background: Real time data regarding clinical presentation and management of patients with epilepsy are sparsely available from Andhra Pradesh, India. Methods: Cross sectional study of 450 patients diagnosed to have and treated for various forms of epilepsy at monthly epilepsy camp during the period 1999-2015 at our tertiary care teaching hospital in South India. Results: Their mean age was 28.12 ± 14.29 years; there were 288 (64%) males. Median (inter quartile range (IQR)) seizure frequency at initial presentation was 2 (1-6)/month. Most frequent seizure types were GTCS (42.2%), followed by focal evolving to bilateral convulsive seizures (26.0%) and focal seizures with dyscognitive features (12.7%). Median (IQR) (years) age at onset, duration at initial presentation, duration of follow-up were 12 (5-20), 4 (1-10) and 4 (2-7.55) respectively. Salient EEG findings were inter-ictal discharges (44.4%) and background abnormalities (20.7%). Brain imaging was abnormal in 171 (38%) Most frequent imaging abnormalities were calcified granuloma (38%) and gliosis (11.1%). In the etiology Structural or metabolic causes were found in 152 (33.8%) patients, electro convulsive syndrome in 87 (19.3%) patients and unknown etiology in 194 (43.1%) patients. Seizure remission was achieved in 334 (74.2%). Median (IQR) duration of remission was 1 (0.5-2) year. Of these 114 (34.1%) were controlled on a single drug, 157 (47%) were controlled on 2 drugs while 63 (18.9%) required ≥3 drugs. Most common factors for not attaining remission were poor drug compliance (40.2%) and lifestyle factors (28.9%). Median (IQR) quality of life index (QOLIE10) (n = 348) was 63 (48-76). Median (IQR) expenditure incurred for procuring medications for free distribution per patient per visit was 208 (117-328) rupees. Important adverse effects of anti epileptic drug treatment included (n=223) anger and aggression (59.2%), headache (49.7%), sleepiness (40.8%). Conclusions: Monthly epilepsy camp providing free medications to patients appears to be an effective innovative measure for treating the patients with epilepsy at community level.


Source localization of JME discharges using Magnetoencephalography

V. M. Gadad, S. Sinha, N. Mariyappa, V. Jayabal, P. Satishchandra, G. Chaitanya

Department of Neurology, NIMHANS, Bengaluru, Karnataka, India

Objective: The purpose of this study is to localize the sources of epileptiform discharges (EDs), in juvenile myoclonic epilepsy (JME) using Magnetoencephalography (MEG), at three different time instances and analyze the propagation of EDs, from onset to offset, forinferring the cortical and subcortical region of involvement. Methods: Twenty patients (age 23.5 ± 6.3 years old) with JME were recruited in this prospective study. MEG source analysis was performed on the independently collected EDs of each patient. The distributed source model was employed for source localization using low resolution electromagnetic brain tomography (LORETA). In each EDs, the onset (leading edge of the spike from baseline), peak and offset (trailing edge of the spike), with time window of 8 ms, were subjected for source localization in order to study the propagation of the EDs. The obtained source location coordinates, from each individual MRI, were transformed in Talairach space and the distribution of region of source involvement was analyzed. Results: The frequency pattern of lobar distribution at onset, peak and offset respectively suggest that discharges most commonly localized at onset from sublobar region, at peak from frontal lobe and at offset from the sublobar region. It was observed that the maximum involvement of sources from the sublobar, limbic and frontal lobes at different time instances. It indicates that the restricted cortical-subcortical involvement during the generation and propagation of EDs in JME. Significance: This MEG study supported the cortical-subcortical region of involvement and provided further insights inour understanding the network involvement in generation and propagation of EDs in JME.


Emotional burden amongst parents of patients with epilepsy

K. Bala, Kalpana, T. B. Singh

Department of Neurology, PGIMS, Rohtak, Haryana, India

Introduction: Expressed emotions and caregivers burden perceived by the family members has an impact on remission and relapses in patients with epilepsy. Aims and Objectives: This study was carried out with an aim to know the expressed emotions and burden perceived by the parents of patients with epilepsy living in nuclear families. Methodology: Parents of 60 patients with history of epilepsy (>3 yrs & without any comorbid disorder), in age range 17-27 yrs, attending Neurology OPD of IHBAS hospital were enrolled for interview after taking informed consent. Tools used were Burden assessment scale (BAS), Family over involvement (EOI) and criticism scale. Results and Discussion: 52 patients (35 M, 17 F) had both the parents while 8 had single parent. Mean BAS score for mothers and fathers of male patients were 56 ± 8.56 and 42 ± 10.34 respectively, for female patients were 56 ± 7.36 and 40 ± 9.25 respectively. Mean EOI scores for mothers and fathers of male patient's were3.99 ± 0.37 and 3.56 ± 0.49 respectively and for mothers and fathers of female patients were 4.16 ± 0.29 & 3.55 ± 0.51 respectively. Mean criticism score for mothers and fathers of male patients were 1, 97 ± 0.46 & 2.48 ± 0.31 respectively while for female patients were 1.85 ± 0.48 and 2.17 ± 0.49 respectively. Thus mothers and fathers of patients with epilepsy behave differently for perceived burden and expressed emotions and the response for female and male child is also different. Conclusion: Burden perceived (BAS score) by mothers is higher than by fathers for both male and female child. Mothers showed more Emotional involvement (more for male than female child). While fathers showed more criticism.


Attitudes towards marriage and epilepsy: A meta-propotion and regression analysis

G. Singh, H. Vohra, T. Rastogi, N. Wasal

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

Introduction: Attitudes towards marriage in people with epilepsy vary according to geographical region, community studies, Calendar period and type of study. Aim: To determine factors associated with attitudes towards marriage in relation to epilepsy in published KAP studies. Methods: Meta-analysis of published KAP surveys in different populations from 1980 onwards to generate a meta proportion followed by univariate and then step wise metaregression of the continent, income status of country of origin, calendar period, setting and community, gender and type of question posed. Results: We included 114 surveys with 99394 respondents. The overall proportion of positive responses was 48% ± 12%. In the step wise regression the two variables that were significantly associated with proportion were the continent of origin and the question posed (e.g., generic? should PWE get married? personal = would you object to your child getting married to someone with epilepsy? Conclusion: African and Asian communities are more likely to have negative attitudes to marriage in context of epilepsy, but the response also depends on the question posed.


Does loss of REM associated atonia have a protective role in focal epilepsy? - Observations from an EEG-polysomnography study

A. Gupta, G. Shukla, K. Chakraverty

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

Background: Seizures among patients with temporal lobe epilepsy (TLE) are known to differ from those with extratemporal lobe epilepsy (ETLE) mainly through more frequent generalization and occurrence during sleep in the latter. REM sleep is known to be protective for seizure spread and hence generalization. This study aims at identifying REM sleep characteristics and their relationship with the main features that differentiate TLE versus ETLE. Methods: The clinical and polysomnography (PSG) records of consecutive epilepsy patients who underwent EEG-Polysomnography during 2012 to 2015, were included. PSG studies were reviewed for REM percentage and for presence of REM sleep without atonia (RSWA). Patients who had one REM epoch with >50% with sustained chin tone were considered to have RSWA. Results: Among a total of 61 patients with technically acceptable PSGs, 35 (57.38%) out of which, had TLE while 26 (42.62) had ETLE. RSWA was found in 8 (25.81) patients in the TLE group and 7 (30.43) patients in the ETLE group. REM%< 2 was seen in equal percentage of patients in both the TLE 7 (20%)and ETLE 3 (11.54%) groups (p = 0.49). However, All 7 number of patients with generalization as well as seizure frequency (0.1 (0.05-240) vs. 3.5 (0.05-150), p = 0.02) was significantly greater in patients with ETLE. Interestingly, none of the patients with history suggestive of generalization of seizures had RSWA. Hence RSWA was only seen among patients who never reported generalization of seizures. Occurrence of seizures exclusively during sleep was reported by none of the TLE patients and 6/26 ETLE patients, only one among which had absence of REM sleep on PSG and only one had RSWA. Conclusion: REM sleep without atonia is frequently present with patients with epilepsy, and it may have a protective effect on seizure occurrence exclusively during sleep and secondary generalization in focal epilepsy.


Refractory epileptic spasms, clinical characteristics and surgical treatment: Experience from a tertiary care centre of South India

S. Gupta, S. Jayalakshmi, M. Panigrahi, S. Shailaja

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

Background: Epileptic spasms (ES) are a distinct seizure type with "uncertain" onset. When it is refractory to medical management, surgical treatment has been advocated with variable success. Objective: We reviewed our experience of clinical profile and outcome of surgery for epileptic spasms in 65 subjects. Methods: Detailed clinical, EEG and imaging data were reviewed from 65 (40 male) patients with ES who underwent surgery between 2004 and 2015; palliative cases like corpus callosotomy were excluded. Results: Out of 65 subjects included, 36 (55.4%) had onset of epilepsy before 2 years of age. The mean duration of epilepsy was 7.46 ± 6 years. Fifty four (83.1%) had daily seizure at presentation. Forty eight (73.8%) had mental retardation and 62 (95.4%) had poor quality of life. The lesion was temporal in 9 (13.8%) and extratemporal in 56 (86.2%). Interictal EEG was multifocal/generalized pattern in 50 (76.9%) and ictal EEG onset was generalized in 26 (40%). PET/SPECT scan were lateralizing/localizing in 40 (61.5%) and 18 (27.7%) respectively. Most common surgery performed was lesionectomy (53.8%) and most common histopathological diagnosis was focal cortical dysplasia (56.9%). Outcome was favourable including complete seizure freedom in 38 (58.5%). On outcome analysis, hemispherotomy was associated with favourableoutcome (β=0.415, p=0.001) while lesionectomy (β= −0.282, p=0.015) and generalized interictal EEG (β=−1.084, p≤0.001) were associated with poor outcome. Conclusion: Epileptic spasms carry significant morbidity, but are amenable to surgical measures with good results when refractory to medical management.

Platform Session 11, Stroke16:30-17:30 h, Saturday, November 12, 2016Hall B


Cerebral sino-venous occlusion in males: A retrospective analysis of clinical, radiological and treatment outcomes

A. K. Duggal, D. Chowdhury, A. Shrivastava, G. A. Khwaja

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

Introduction: Cerebral sino-venous thrombosis (CVT) has been well documented as an important cause of stroke in peri-partum females in India. Data on CVT in males is sparse. Aim: To study clinical, radiological and treatment outcomes in male CVT patients. Methods: Retrospective analysis of the clinical records of male CVT patients during 2010-2015. CVT diagnosed by MRI and MRV. All patients investigated as per standardized protocol including assessment of pro-thrombotic states. Results: Demography - Case records of 66 male CVT patients (mean age: 30.84 ± 9.8 years) were analyzed. Presentation: Acute or sub-acute presentation (mean symptoms duration 17.07 ± 13.01 days) seen. Presentations include focal neurological deficits (16), subacute encephelopathy (20), isolated intracranial hypertension syndrome (20), isolated migraine type headaches (5) and isolated seizures (5). Risk associations: 50% were smokers and 26% were consuming alcohol regularly. Other risk factors included: hyperhomocysteinemia (24), HIV infection (3), anabolic steroids use (3), past history of venous thrombosis (2), tubercular meningitis (2), polycythemia (3), and prothrombotic factor(s) abnormality (4). Immediate triggers included fever, dehydration and alcoholic binge in a minority. Anatomical Involvement: All sinuses were involved in 3, superior sagittal sinus alone in 36 and transverse sinuses alone in 13 patients. Rest had in combinations. Deep CVT was present in 3 patients. Treatment: All were treated with anticoagulants (LMWH + oral warfarin) for atleast 6 months. One refractory patient underwent intra - sinus thrombolysis with rt-PA but succumbed. Outcome: Overall good prognosis seen in 48 patients (72%) who achieved MRS ≤ 2 within 3 months. 16 patients had residual deficits. 2 patients succumbed to CVT (mortality rate of 0.03%). Delayed diagnosis and worsening encephalopathy prognosticated poor outcome. Conclusion: CVT in males is not uncommon. As presentations may vary, high index of suspicion is needed. LMWH followed by warfarin provides excellent results provided early diagnosis is made.


Cerebral venous thrombosis in males - The diverse manifestations, etiology, imaging and prognosis of an under recognized disease in males

A. S. Shah, V. Bhargava, K. Shubhakaran, J. Sharma, Pratibha, Nitti

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

Background: CVT is not uncommon in MALES that once thought. Interestingly, MALE CVT has different clinical spectrum, etiology, workup, prognosis and more frequently an underlying etiology is found in most patients. Aim: To study clinical profile including etiological spectrum of Cerebral Venous Thrombosis In males and Factors associated with poor outcome. Methodology: This study was performed as a hospital based retrospective & prospective observational study at MG hospital at Jodhpur, India. All patients hospitalized in between the period (2013 to 2015) with the final diagnosis of CVT (confirmed by imaging MRI/MRV or angiography) were included. A detailed proforma filled and entered into the computerized data sheet, viz; Demographic data dates of onset of symptoms, of hospital admission and of confirmation of the diagnosis by imaging symptoms and signs from onset and diagnosis. Results: Of the 40 male the mean age was 31.3 years (range 15-78 years). Onset was acute in 6, subacute in 24 and chronic in 10 patients. Most presenting symptoms were headache (95%), vomiting (85%), seizures (35%), hemiparesis (15%), altered sensorium (15%). On imaging, 20% patients had brain parenchymal lesions, sinus involved were SSS (62.5%), right transeverse (27.5%), left transeverse (32.5%), and deep sinuses (10%). Common risk factors were hyperhomocysteinemia in 7 (17.5%), alcoholism in 7 (17.5%), polycythemia (5%), trauma 2 (5%), dehydration (5%) and protein S deficiency in 1 (2.5%) patients. In-house mortality was noted in 1 (2.5%) and recurrence in 1 (2.5%) patient. Conclusion: Compared to the previous studies, prevalence of CSVT was higher in men. Hyperhomocysteinemia, alcoholism was the most common risk factors. Overall prognosis was good, but a small percentage of patients died or showed recurrence.


Delayed cortical venous flow following mechanical thrombectomy for supratentorial large artery occlusion - an early predictor for blood brain barrier breakdown

R. Kumar

Department of Neurology, G S Neuroscience Clinic and Research Center, Patna, Bihar, India

Background: 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. Objective methods to predict reperfusion hemorrhage are lacking. Newer developments in post processing of Digital Subtraction Angiography (DSA) images give an insight into the dynamics at the capillary bed. Methods: Data obtained during the thrombectomy of 61 patients suffering from anterior circulation large artery occlusion was analyzed retrospectively. All patients underwent thrombectomy using the Solitaire Device. Angio-Viz software from GE, USA was used to quantify the perfusion based on DSA images. Results: TICI2b or TICI3 flow was obtained in all the 61 patients. 12 patients developed hemorrhagic transformation of the infarcted region, and decompressive surgery had to be performed in 6 patients. Age, risk factors for stroke, time of reperfusion and use of GPIIb/IIIa inhibitors were not significantly different among the 2 groups. The hemorrhage group demonstrated a significantly delayed filling and subsequent emptying of the venous system. Conclusions: A delayed filling and emptying of the cortical veins is an independent risk factor for hemorrhagic transformation of an ischemic infarct. This finding possibly indicates significant disruption of the blood brain barrier associated with micro-vascular occlusion, and should alert the interventionalist to plan early decompressive surgery.


Long term incidence of macro-vascular events after first attack of ischemic stroke - A 5 year follow-up study

V. N. Nagarathinam, M. Dhanaraj, M. Vikash, Balasubramaniyam

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

Background: The available information on delayed macrovascular complications following first attack of stroke is scanty. It is essential to know these facts as well as the factors influencing it for better long term management of stroke patients. Aim: To find out the incidence of macrovascular events such as recurrence of stroke, myocardial infarction and other vascular events follow the first attack of ischemic stroke over a period of 5 years and the factors influencing it. Materials and Methods: This study was conducted in Apollo hospitals, Chennai. All acute ischemic stroke patients admitted from January 2010 to April 2011 were included. Cerebral embolism of cardiac origin was excluded. The standard risk factors such as diabetes, hypertension, dyslipidemia and smoking were collected. All of them were advised risk factor control, antiplatelets, statins and periodic follow up. Those who failed follow up were interviewed through telephone with reference to risk factor control, drug compliance, recurrence of stroke, myocardial infarction, other vascular disorders and sudden death. Data were collected and they were divided into two groups (Group1: with and Group2: without macrovascular complications) and comparative study was done between them. Results: A total of 149 first attack of ischemic stroke patients were seen, six of them died within 30 days and 30 could not be followed up. Hence the study consists of 113 patients, among them 46% (52) had macrovascular complications including recurrence of stroke 22% (25), myocardial infarction 10% (11), peripheral arterial disease 2% (3)and sudden death 12% (13). Mean age in Group1 and 2 were 65 and 59 years (p=0.037). Smokers in Group 1 and 2 were 46% (22) and 9% (6) with p value of less than 0.001. There was no statistical difference in relation to diabetes, hypertension, dyslipidemia, alcoholism and drug non-compliance between these groups. Conclusion: As high as 46% of stroke patients developed macrovascular complications over a period of 5 years. Increasing age and smoking were significant factors influencing it. Existing preventive measures are probably inadequate for the secondary prevention of macrovascular complications.


Botulinum toxin in Meige's syndrome patients: Video based case series

S. Pandey, S. Sharma

Department of Neurology, Govind Ballabh Pant Institute of Postgraduate Medical Education and Research, New Delhi, India

Background: Meige's syndrome is rare but the most common cause of cranial dystonia, characterized by blepharospasm and oro-facial dystonia. Injection Botulinum Toxin (BTX) is the treatment of choice. Aims: In the majority of the studies Meige's syndrome patients have been discussed with blepharospasm patients and there is a paucity of case based studies exclusively describing this syndrome. Hence we are publishing this case-series to characterize and define the evolution of the syndrome as well as response to BTX therapy. Methods: Eight Meige's syndrome patients who had never been injected with BTX in the past were evaluated at our movement disorder clinic with a structured questionnaire. Videotaping of abnormal movements was done for 5 minutes before the BTX injection and at 1-month follow- up. All patients received electromyography guided 'OnabotulinumtoxinA' and dosage was decided by clinical evaluation. Demography, clinical features and treatment response to BTX using Burke-Fahn-Marsden Dystonia rating scale (BFMDRS) were analyzed before injection and at one-month follow-up. Results: Peak age of symptom onset was 46.4 years with a Male:Female ratio of 1:1 and average duration of symptoms was 6.43 years. Majority (6/8) had symptom onset with blepharospasm and clonic blepharospasm was noted more frequently (5/8). Lingual dystonia (6/8) and pharyngeal involvement (4/8) were commonly noted. Sensory tricks were present in all, placement of fingers over eyelids being most common (7/8). Average BTX dose given was 51.58 units and peak onset of relief was noted at 8.62 days with duration of effect lasting 82.5 days. Only one patient reported, mild weakness of jaw muscle following BTX injection. The average BFMDRS was 27.18 and 13.37 pre and post BTX respectively. Conclusions: In this case series of Meige's syndrome patients tongue involvement was very common (6/8, 75%) and response to first dosage of BTX treatment was excellent with no major side effect.


Dysferlinopathy in India: A next generation sequencing based study

K. Polavarapu, A. Nalini, K. V. Preethish, S. Vengalil, R. Dastur, P. Gaitonde

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

Background: Mutations within DYSF gene located at 2p13.3, encoding a 2080AA transmembrane sarcolemmal protein Dysferlin, can result in Limb-Girdle Muscular Dystrophy2B (LGMD2B), Miyoshi Myopathy (MM) or rarely Distal Myopathy with Anterior Tibial onset (DMAT). Objective: A prospective study to identify genetic mutations in biopsy confirmed Indian patients with Dysferlinopathy. Methods: Diagnosis of Dysferlinopathy was confirmed by muscle immunohistochemistry in 40 clinically suspected patients. Targeted Next Generation Sequencing (NGS) was performed on DNA extracted from blood samples of patients after obtaining informed consent, using a 35 gene LGMD panel at Centre for Advanced Molecular Diagnostics in Neuromuscular Disorders (CAMDND) in Mumbai through Jain foundation's India LGMD2B diagnostic program. Pathogenicity was confirmed based on Emory Genetics Laboratory (EGL's) variant classification. Results: Targeted NGS testing revealed mutations in DYSF gene in 37 cases, while 3 had mutations in other genes. Homozygous variants identified in 35 patients and compound heterozygous mutations in 2 cases. Known pathogenic mutations found in 26 patients, while 18 had novel variants out of which 14 were pathogenic and 4 had Variants of Unknown Significance (VOUS). Mutations in intron 50 and 52 of pathogenic significance observed in 3 and 1 cases respectively. One patient had homozygous VOUS variant in POMGNT1. Three heterozygous mutations in GNE gene with one likely pathogenic variant found in one patient, while another patient had heterozygous VOUS mutations in SYNE1 and TTN genes. Males: Females-29:11; Consanguinity-26 (65.0%); Positive family history-13 (32.5%). Calf wasting observed in 36 (90.0%) patients: Proximo-distal phenotype-26 (65.0%) and Miyoshi myopathy-10 (25.0%). Proximal limb-girdle involvement without calf wasting observed in 4 (10.0%) patients. Conclusion: Clinically and pathologically confirmed Indian patients with Dysferlinopathy showed several novel pathogenic variants as identified by NGS. Targeted NGS enhances the ability to identify mutational pattern accurately in ARLGMDs like Dysferlinopathy and hence aid in efficient genetic counselling as well as enrolment in future therapeutic trials.

Platform Session 12, Miscellaneous16:30-17:30 h, Saturday, November 12, 2016Hall C


Neuro respiratology - A science of coherent breathing

S. R. Menon, A. A. Kumar, K. Nayar, A. G. Roy, C. S. Kumar, K. R. Sundaram

Department of Neurology, Amrita Institute of Medical Sciences & Research Centre, Kochi, Kerala, India

Background and Objective: Cerebrovascular accident is one of the most common causes of death and disability among elderly population. Both ICH (Intracerebral haemorrhage) score and APACHE-II (Acute Physiology and Chronic Health Evaluation) score are used to predict the mortality, where former is only used for ICH and later has application in other fields of medicine also. This study is a comparison between APACHE-II and ICH Score in predicting 30 days' mortality and to assess the importance of non neurological factors in addition to neurological parameters affecting outcome in hemorrhagic stroke patients in a tertiary care centre in Eastern India. Methods: This prospective observational comparative study was done with the Patients admitted in the neuromedicine indoor and critical care unit with hemorrhagic stroke through emergency and medical outpatient department. Results: Out of 104 patients recruited, majority were male (66.3%) and of 5 th to 7 th decade of age. History of smoking, lower income, lower education, rural inhabitant, low Glasgow Coma Scale (GCS) at admission and intracerebral hemorrhage with intraventricular extension are important predictors of 30 day mortality. From comparison of sensitivity and specificity between APACHE II score with cut off 19 and that of ICH score with cut off 3 in predicting mortality, it appears that sensitivity in both score is 76.5%. However specificity of ICH score is 90% and APACHE 2 score is 76.5%. Conclusion: Although sensitivity of these 2 scores is same but ICH score is far more specific than APACHE-II.


Respiratory complex deficiency in adults: A phenotype-genotype correlative study

A. B. Taly, R. Kumar, S. Kothari, P. Govindraj, C. Shwetha, P. S. Bindu

Department of Neurology, NIMHANS, Bengaluru, Karnataka, India

Background: The diagnosis of mitochondrial disorders is easy to suspect at bedside, but difficult to establish since it requires battery of tests. Spectrophotometric analysis of mitochondrial respiratory chain complex (RCC) activity is a recently established facility at NIMHANS for improving the diagnostic yield. Aims and Objectives: To describe profile & patterns of mitochondrial RCC deficiency in adults. Methodology: A total of 182 patients, including 73 adults, fulfilling Bernier's criteria for mitochondrial disorders underwent detailed evaluation over three years (2011-13). RCC were assayed by spectrophotometric method. mtDNA and POLG1 and POLG2 were sequenced using Sanger's technique and deletions in muscle mtDNA were studied using long-range PCR. Data of muscle histology, serum lactate, and brain MRI were collated. Results: Fifty three adults (M:F 1.3:1; age: 32.1+11.2 years; symptom-duration: 7.0+6.6 years, syndromic-20 & non-syndromic-33) were detected to have RCC deficiency: complex I =33, complex IV=4, complex III =1 and multiple complexes =15. Common phenotypes included CPEO)/CPEO plus (n=15), ataxia (n=10), myopathy (n=8), epilepsy (n=5), leukoencephalopathy (n=4), & MELAS (n=4). Key MRI abnormalities (n==34) were white matter hyperintensities (n=20), mineralisation of dentate nucleus (n=12), cerebral atrophy (n=10) & basal ganglia hyperintensities (n=10). Other salient features were abnormal nerve conduction parameters (n=27/41) and epileptiform discharges in EEG (n=6/20,). Mitochondrial DNA analysis revealed deletions in 16/44 (multiple: 12; single: 4) & point mutations in 9/24 (known pathogenic: 4, novel: 5). One patient had POLG1 mutation. Mitochondrial signatures like elevated serum lactate (n=15), lactate peak on MRS (n=7/34) and cytochrome c oxidase deficient fiber in muscle (n=20), were noted only in a fraction of the cohort. Conclusion: RCC assay should be integral in diagnostic work up of patients with suspected mitochondrial disease as it improves diagnostic certainty in a substantial proportion of patients particularly with non-syndromic presentation and ascribe pathogenicity.


Acoustic vestibular evoked ocular myogenic potentials: An important diagnostic tool for neuro-otology

K. Sundar, C. U. Velmurugendran, V. Shankar, U. Meenakshisundaram

Department of Neurology, Sri Ramachandra University, Chennai, Tamil Nadu, India

Background: After headache and back pain, vertigo is the commonest symptom that brings a patient to a Neurologist. Over the last few decades many electrophysiological tests have been used to clinically assess this symptom. Acoustic vestibular evoked myogenic potential (Ac oVEMP) is one such investigation that tests the vestibular nerve. In this study we look at Ac oVEMP as a test that can recognize vestibular neuronitis. Objective: To study the usefulness of acoustic vestibular evoked ocular myogenic potentials (Ac oVEMP) in patients with vestibular neuronitis. Materials and Methods: 100 subjects were included in this study (Group A 50 - normal subjects; Group B 50 patients with vestibular neuronitis). Ac oVEMP was performed on both the groups and the results were analyzed for difference and statistical significance. Patients in Group B were additionally subjected to cervical VEMP (cVEMP) and brainstem auditory evoked response (BAER). The results were tabulated and analyzed. Discussion: Ac oVEMP in Group A subjects produced normal n and p response that was consistent and reproducible. There was no interaural difference. Ac oVEMP in Group B showed prolongation of n and p latencies when compared with normal subjects (p≤0.01). 23 patients demonstrated abnormal Ac oVEMP (46%) whereas 10 had abnormal cVEMP (20%). This was attributed to the selective involvement of superior division of vestibular nerve in vestibular neuronits, which was tested by Ac oVEMP. Conclusion: Acoustic vestibular evoked ocular myogenic potential (Ac oVEMP) is a potent electrophysiological test in neuro-otology which can be used to study the vestibular nerve.


Factors predicting outcome in Gullian barre syndrome patients treated with IV immunoglobulins

V. Chenna, A. K. Meena, Rupam, S. A. Jabeen, R. Mridula

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

Introduction: Guillain-Barré syndrome (GBS) is an acute, immune-mediated polyneuropathy that often leads to severe weakness. Intravenous immunoglobulin (IVIG) is a proven effective treatment for GBS (class 1 evidence). Important clinical factors associated with poor outcome are age, presence of preceding diarrhea and the severity of disability in the early course of disease. Aim: (1) To analyze the predictors of outcome in patients admitted with gullian barre syndrome (GBS), who received IVIg treatment. (2) To analyze the outcome of patients based on serum immunoglobulin levels. Methods: Data collected prospectively from a cohort of 53 patients with GBS were used to identify risk factors of being unable to walk at 4 weeks, 3 months, and 6 months who received IVIg. Potential predictors of poor outcome (unable to walk unaided) were considered in univariable and multivariable logistic regression models. A standard dose of IVIG is not sufficiently effective in many GBS patients. We have checked pre and post serum immunoglobulin levels and compared with the outcome to know whether there is any correlation to outcome. Results: There were 35 males and 17 females. The mean age of presentation was 34.91±19.1 years. The subtypes of GBS were Acute Inflammatory Demyelinating Polyneuropathy (AIDP) (28/52), Acute Motor Axonal Neuropathy (AMAN) (15/52) and Acute Motor Sensory Axonal Neuropathy (AMSAN) (9/52). Ventilator requirement predicts poor prognosis. Age, duration of illness, prior GI / URTI, GBS subtype, MRC sum score, duration of hospital stay didnot find statistical significance. The measurement of immunoglobulin levels prior to and 2 weeks after IVIg administration reached statistical significance especially Ig G. Conclusions: The measurement of immunoglobulin levels can be used to predict the outcome and second dose IVIg can be tried in patients who did not improve.


Spectrum of neurological mitochondrial cytopathy: Experience at a tertiary care center in North India

A. S. Kumar, V. Lal, Modi, B. L. Gasper, R. K. Vasishta

Department of Neurology, PGIMER, Chandigarh, India

Introduction: Mitochondrial diseases are multiorgan system disorders and nervous system is most commonly affected, accounting for its high energy consumption. Multiple components of the neuraxis including brain parenchyma, spinal cord, peripheral nerve, neuromuscular junction and muscle can be affected. Genetic mitochondrial diseases may be due to mitochondrial or nuclear DNA defects. Materials and Methods: Retrospectively collected data of fifteen subjects with biopsy proven mitochondrial cytopathy with neurological features were isolated from the records of subjects visiting outpatient and inpatient department of neurology services, between 1 st June 2015 and 31 st June 2016 PGIMER, Chandigarh. Epidemiological data, clinical features and syndromic diagnosis were noted. Results: Mean age of the subjects was 35.07 ± 15.45 years. There were 8 males and 7 females in the study group. Thirteen subjects could be categorized into well-defined syndromes, while two belonged to undefined group. Among the defined syndromes, four subjects had MELAS (mitochondrial encephalopathy, lactic acidosis and stroke like episodes), one had MERRF (myoclonic epilepsy and ragged red fibre myopathy) and eight were diagnosed to be suffering from mitochondrial myopathy. In the uncategorized group, one subject presented with behavioural abnormalities and the other was noted to have seizure with cognitive decline and optic atrophy. Four subjects had cerebellar ataxia, with peripheral neuropathy noted among five. Diminution of vision was noted among seven, with seizure in six. Muscle biopsy was done in all cases with features suggestive of mitochondrial cytopathy being noted in all. Individuals in the study groups were treated with coenzyme Q10 and other mitochondrial metabolism intermediates. Conclusion: Subjects presenting with multi-neuroaxial involvement should be investigated on the lines of mitochondrial cytopathies.


SPECT study in migraine: A tertiary care experience

S. K. Bhoi, J. Kalita, P. K. Pradhan, U. K. Misra

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

Background: Regional cerebral blood flow (rCBF) alteration is reported in migraine patients during headache attack. Objective: To evaluate single photon emission computed tomography (SPECT) abnormality and study the regional cerebral flow (rCBF) in common migraine patients. Subjects and Methods: Technetium (Tc) 99m ECD brain SPECT CT scanning procedures was performed in 21 migraine patients. Their detail demographic and migraine parameters were noted as per International Headache Society (IHS). The SPECT images were evaluated semiquantitatively with regard to the degree of asymmetry of the rCBF between the two hemispheres and the ratio of the rCBF in regions of interest to the rCBF in two reference areas (cerebellum or frontal cortex). The ictal and interictal SPECT abnormality was noted. Results: Their median age was 28 (18-42) years and 17 (81%) were females. Median duration of headache was 5 (2-16) years. Headache location was holocranial in 12, hemicranial in 7, and one each of occipital and frontal. The median frequency of migraine was 8.5 (range 4-30) attacks per month. Ictal SPECT was done in 6 (28.6%) patients. SPECT findings were abnormal in 7 (33.3%) patients. Hypoperfusion noted in parietal lobes in 2, parieto occipital in 1, fronto-parieto-temporal in 1 and frontal lobe in 1 patient. Hyperperfusion was seen in occipital lobe in 2 patients. SPECT was abnormal in Right side in 3 and left side in 3 and 1 patient had bilateral involvement. Conclusion: One third of migraine patients showed SPECT abnormality. There was no significant rCBF asymmetries noted between ictal and interictal SPECT.

Platform Session 13, Award Papers08:30-09:30 h, Sunday, November 13, 2016Hall A


A randomized trial of safety and efficacy of lowering mean arterial pressurein acute spontaneous intracerebral haemorrhage

S. Gupta, A. K. Abbot, R. Srinath, A. K. Tewari, A. Gupta, S. P. Gorthi, C. S. Narayanan, S. I. Totlani, Y. S. Serohi, R. Annadure

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

Background: Hematoma expansion due to raised blood pressure in spontaneous intracerebral haemorrhage is a major determinant of morbidity and mortality. Aim: To determine the safety and efficacy of lowering blood pressure during the first 72 hours of spontaneous intracerebral haemorrhage. Methods: In this open label, multicentric trial patients ≥ 18 years with spontaneous intracerebral hemorrhage with no other cause were randomized within 72 hours of onset to tight BP control arm (mean arterial pressure ≤ 115 mm of Hg) and conventional BP control arm (mean arterial pressure ≤130 mm of Hg). The level was maintained for another 72 hours after which both arms had mean arterial pressure below 115 mm of Hg. Primary outcome was modified Rankin Scale at 90 days. Results: 118 patients, 59 in each arm were included. Follow up was available for all. Baseline characteristics were similar. At 90 days there was no significant difference between median mRS between the two arms. Odds Ratio for "poor outcome" (mRS 3-6) in the tight control arm (safety of the intervention) against "good outcome" (mRS 0-2) was not significant (OR 0.70 (95% CI 0.34-1.47) tight control arm was not significant (OR 1.43 (95% CI 0.68-2.99), p=0.35). Conclusion: In patients with spontaneous intracerebral haemorrhage who present within 72 hours of the onset of symptoms, mean arterial pressure can be safely lowered if it crosses 115 mm of Hg but it does not improve clinical outcome.


Fatty acid oxidation defects presenting as primary myopathy with prominent dropped head syndrome

S. Vengalil, V. P. Kumar, K. Polavarapu, R. Christopher, N. Gayathri, C. Prasad, A. Nalini

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

Background: Fatty acid oxidation (FAO) disorders presenting as primary myopathy is relatively rare. "Dropped head syndrome (DHS)" is reported only in single cases of carnitine deficiency and multiple acyl CoA dehydrogenase deficiency (MADD). Objective: To identify FAO defects presenting as primary myopathy. Materials and Methods: A retrospective and prospective collection of 18 patients of FAO disorders presenting as primary myopathy, between 2011 and 2016 at a neuromuscular disorder clinic. All patients underwent clinical examination and tandem mass spectrometry. Eight patients underwent muscle biopsy and MR Imaging of the muscle done in 4 cases. Results: Subgroup of FAO defects: Medium chain acyl CoA dehydrogenase deficiency (MCAD) = 4; Very long chain acyl CoA dehydrogenase deficiency (VLCAD) = 7; MADD = 5; carnitine uptake defect (CUD) = 1; short chain acyl CoA dehydrogenase (SCAD) deficiency= 1. The mean age at onset and the mean time to diagnosis for MCAD, VLCAD and MADD was 12.7, 11.6, 23.4 years and 3.5, 7.4 and 11.2 years respectively. The patients with CUD and SCAD had onset at 29 and 15 years of age. The dominant symptoms were exertion induced myalgia and progressive proximal limb weakness in all. 11/18 (61.1%) had DHS. Ptosis and bulbar weakness were present in a few. Discussion: FAO disorders presenting as primary myopathy may be under diagnosed. Conclusion: FAO disorders should be considered in the differential diagnosis of acute or chronic limb girdle muscular symptoms. Hitherto, unreported we describe DHS as a prominent phenomenon in MCAD and VLCAD.


Role of angiotensin converting enzyme gene polymorphism in cognitive deterioration in patients with cerebral small vessel disease

T. G. Issac, S. Retnaswami, R. Christopher, J. Rajeswaran, M. Philip

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

Introduction: Cerebral small vessel disease (SVD) is the major contributor to vascular dementia SVD is characterized by periventricular white matter hyper intensities, Lacunes and micro bleeds in imaging and is a common finding in asymptomatic elderly with only a third progressing to VaD. The mechanisms for the same is yet unknown though heritability has been documented in few studies. Unlike other degenerative dementias this condition is potentially treatable and preventable making it more important to determine the factors responsible for conversion to VaD. Patients and Methods: 202 patients having radiological features of SVD were selected over a period 3 years after informed consent for genetic studies and neuropsychological assessment. Neuropsychological assessment specifically designed for assessing executive dysfunction in SVD was carried out and follow up done after 1 year. Results: There was significant difference (p<0.001) across neuropsychological scores between various neuropsychological tests between the first and second follow ups. The Neuropsychological scores with regard to ACE I/D polymorphism had a detrimental effect on cognition being associated more with ACE D/D polymorphism whereas the odds for ACE I/I polymorphism to protect from conversion to VaD were significantly high. Discussion and Conclusions: The study corroborates the role of hypertension as a major risk factor for cerebral small vessel disease. The ACE d/d polymorphism was found to be detrimental with reference to cognitive function. SVD resembles degenerative dementia and is not similar to the presentation of dementia due to large vessel disease.


Sensory motor integration in focal hand dystonia

S. Kumar, M. Alexander, S. Mani, A. Sivadasan, V. Mathew

Department of Neurosciences, CMC, Vellore, Tamil Nadu, India

Background: Focal hand dystonia (FHD) is a task specific dystonia with variable degrees of dysfunction and various therapeutic strategies are tried with limited success. Abnormalities in sensory motor integration, neuroplasticity and surround inhibition have been described in the pathophysiology of FHD. Aims and Objectives: To explore the sensory abnormalities, sensory motor integration and to look at abnormal plasticity using functional MRI (f MRI)/Multichannel surface electromyography (SEMG) in FHD and correlate with dysfunction. Materials and Methods: 15 FHD patients/15 healthy controls were included in this study. Sensory dysfunction was assessed using JVP domes and Aristotle illusory paradigm. Sensory Motor integration was assessed using precision hand grip. The abnormal plasticity of brain was assessed using event related fMRI and SEMG. Results: Gap detection threshold was found to be significantly higher in index and little finger of both hands of FHD and normal in controls. Aristotle's illusion paradigm showed significant decreased perception of double stimuli in crossed position in FHD patients. Precision hand grip task showed higher peak grip force in FHD patients. SEMG revealed "early onset" of activity, co-contraction of agonist-antagonist muscles and "delayed offset" in both observational and imagery tasks in FHD. fMRI demonstrated significant abnormalities in motor imagery and observation in all groups of FHD patients. Discussion and Conclusions: FHD had sensory dysfunction leading to abnormal sensory motor integration and resultant maladaptive plasticity. The presence of anticipatory changes in EMG indicates dysfunction of premotor cortex and therapeutic targets could include: sensory retraining/biofeedback/r TMS to inhibit afferent discharges.


Predictors of outcome after resective surgery in drug-resistant frontal lobe epilepsy

P. J. Samuel, R. Menon, G. Vilanilam, M. Abraham, B. Thomas, S. V. Thomas, S. P. Sarma, A. Radhakrishnan

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

Background: Frontal lobe epilepsy (FLE) surgery is the second most common focal resective surgery in drug-resistant epilepsy. Not many studies are available regarding the long-term surgical outcome and its predictors. Objective: To study the longitudinal outcome and prognostic predictors of seizure outcome following FLE surgery in a sizeable cohort of patients. Materials and Methods: 73 consecutive patients who underwent FLE surgery between January 1997-May 2015 with a minimum of one year follow-up (range 1-16 years) were studied. Primary outcome was seizure freedom at last follow-up (Engel Class IA). Seizure freedom was defined as absence of seizures till last follow-up, excluding auras and acute post-operative seizures. Outcome predictors were subjected to multivariate analysis (Cox regression model). Using Kaplan Meier curves, we assessed the postoperative seizure freedom over time. Discussion: 25 patients (34%) were seizure-free till last follow-up. The seizure freedom was 45 % at the end of first postoperative year, 34 %, 26 %, 20 % and 14% at the end of 2 nd , 3 rd , 4 th and 5 th postoperative year consecutively. Predictors of seizure recurrence on multivariate analysis were older age at surgery (p= 0.032), longer duration of epilepsy (p=0.031), presence of interictalepileptiform discharges (IEDs) in postoperative EEG on 7 th day (p=0.005), at 3 months (p=0.005) and at 1 year follow up (p=0.0179). In a subgroup analysis, duration of epilepsy prior to surgery less than 2 years showed a significant predictor of achieving seizure freedom (p=0.029). Conclusion: These results emphasise the importance of early surgery for excellent outcome after frontal lobe epilepsy surgery. EEG remained a good post-operative variable predicting long-term outcome.


Do auras aid in surgical decision making in drug resistant temporal lobe epilepsy?

D. Menon, R. Menon, G. Vilanilam, M. Abraham, B. Thomas, C. Kesavadas, S. V. Thomas, A. Radhakrishnan

Department of Neurology, SCTIMST, Trivandrum, Kerala, India

Introduction: The essence of epileptic surgery is in identifying the epileptogenic zone. Beyond this goal, the significance of epileptic aura has been little explored especially regarding its impact on surgical outcome in refractory epilepsy. The current study focuses on the clinicopathological correlation of aura as well as the role of aura in predicting surgical outcome in temporal lobe epilepsy (TLE). Objectives: (1) To determine the surgical outcome in temporal lobe epilepsy in comparison between patients with and without aura and (2) to identify clinicopathological, radiological and surgical differences between the two groups. Materials and Methods: Consecutive patients who underwent presurgical evaluation for TLE and ATL-AH as per our protocol, from Jan 2009 to December 2015 were included. Patients were followed up at 3 months, 12 months and then annually. Results: Among 456 patients included in analysis, 344 (75%) had aura. Variables analysed included clinical, EEG and imaging data, side of surgery, ECoG, histopathology and post surgical outcome. Multivariate Logistic regression analysis showed prototype EEG (OR 2.12, 95% CI 1.18-3.78) and right side surgery (OR 1.82 (95% CI 1.08 to 3.06) p=0.012) were significantly associated with aura. While there was no difference in surgical outcome between the two groups, those patients with auditory aura (OR 7.28, CI 2.80-18.95, p= 0.000) and vertiginous aura (OR 3.01, CI 1.55- 7.85, p= 0.028) had a poor surgical outcome and. Bivariate analysis showed no lesion or indeterminate lesion in MRI (p= 0.025) and non MTS pathology (p=0.001) were significantly associated with auditory aura and vertiginous aura. Conclusion: Presence of preoperative aura collectively, do not affect the outcome of surgery in TLE. However presence of auditory and vertiginous aura is a predictor of poor surgical outcome. These patients may require more extensive screening for an extratemporal focus before selection before surgery.

Poster Session 01; Infections and Demyelinating Disorders08:30-11:00 h, Friday, November 11, 2016


Cystic brain tuberculoma mimicking intracranial hydatosis: A diagnostic dilemma

A. Gupta, S. Kushwaha, V. Banga, S. Singh, M. Sachan

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

Introduction: Intracranial tuberculomas are being reported as thick walled cavity on brain imaging. Here we report a case of 21 years old female with atypical brain tuberculoma mimicking multiple primary intracranial hydatosis. Case Report: We report a case of 21 years old female with right focal seizures with dyscognitive features for last 2 years. There was no history of fever, chronic cough, vomiting, altered bowel habits or ear discharge. Patients lacked any neurological deficit, meningism or papilledema. Routine hematological investigations, Chest X-ray, ultrasound abdomen and cerebrospinal fluid CSF findings were nonrevealing. Serology for Human Immuno Virus (HIV), Venereal Disease Research Laboratory (VDRL), toxoplasma and echinococcus were negative. Magnetic Resonance Imaging (MRI) of brain revealed multiple thin walled cystic lesions. These were in clusters and CSF attenuating with mild perilesional edema around the lesions. Findings up to this stage lead to a provisional diagnosis of multiple primary intracranial hydatosis. Patient was started on antiepileptics with steroid therapy. Due to lack of clear consensus regarding management of intracranial hydatosis patient was additionally started on albendazole for 14 days undercover of steroids with no improvement subsequently. At this stage MRS study was done which showed a lipid lactate peak with a possibility of tuberculoma. Tuberculoma possibilities lead us to cystic aspiration. Ziehl-Neelsen (ZN) staining and culture of aspirate showed mycobacterium tuberculosis. After adding anti-tubercular therapy patient become seizure free. Discussion: Multiple intracranial cystic lesions are infrequent finding on brain imaging. Possibility ranges from hydatosis to tuberculoma and metastasis. In Indian scenario, tuberculosis is the best possibility but absence of the peculiar findings and multiple thin walled cysts rarely reported as tuberculoma deviate towards primary multiple intracranial hydatosis which itself is the rarest of rare phenomenon. The current case reinforces that even though the multiple intracranial thin walled cysts are rarely reported as tuberculoma it should be kept in differential during evaluation of such multiple intracranial cystic lesions.


Unique neuroimaging features in varicella zoster lumbosacral myeloradicuitis

M. Kar, M. Y. Reddy, S. Parida, J. M. K. Murthy

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

Introduction: Varicella virus reactivation, especially in elderly and immune compromised causes zoster. Lumbosacral zoster is uncommon and literature regarding radiological features is scant. A 70-year-old female known case of diabetic, hypertensive and hyperthyroid presented with fever, vesicular rash and burning pain over right leg and foot, urinary retention and incontinence and difficulty in walking of 15-day duration. Examination revealed papulovesicular rash over right L5 and S1 dermatomes. Neurological examination revealed weakness of right hip abductors, knee flexors and foot dorsi-flexors, absent bilateral knee and ankle jerks and allodynia over L5-S1 dermatomes. Kerning's sign was positive. Contrast MR of lumbosacral spine revealed contrast enhancing enlarged right dorsal root ganglia of L5 and S1 and were hyperintense on T2-weighted imaging. The exiting nerve root at the level of S1 on right side was enlarged and hyperintense on STIR. Conus medullaris and cauda showed enhancement on right side. CSF analysis showed lymphocytic pleocytosis (75 cells/μL), elevated protein (75 mg %), and normal glucose. CSF VZV PCR was positive. She was diagnosed to have herpetic lumbosacral myeloradiculitis due to varicella zoster infection and was treated with intravenous acyclovir for 14 days. She had significant improvement in pain and is ambulatory with minimal motor weakness. Conclusion: Contrast MR-imaging may quite diagnostic of radiculomyelitis due varicella zoster infection.


An interesting mimic of Pott's spine

K. Kuppusamy, K. Pandiyan, N. Thamilpavai, K. Venkatraman, S. Gobinathan, Laksminarasimhan

Department of Neurology, Madras Institute of Neurology, MMC, Chennai, Tamil Nadu, India

Objective: To highlight the importance of suspecting fungal infection in immune compromised patients with vertebral osteomyelitis resistant to Anti tuberculous therapy. Tuberculosis infection is the most common cause for vertebral osteomyelitis in tropical countries, especially in India followed by fungal etiology in immunocompromised patients. We describe two case of aspergillus osteomyelitis (culture positive) who were initially diagnosed as TB spine but responded poorly to antituberculous drugs. Methods: Observational study of two cases in Institute of Neurology, MMC, Chennai. Results: Case I, 24 years old male with Post Renal transplant status developed sudden onset of rapidly progressive flaccid paraplegia after 3 months of renal transplant. Patient underwent decompression surgery and had no response to Empirical anti tuberculosis therapy. Anti fungal agents started based on HPE which reported aspergillus flavus. Case II, 66 year old male diabetic developed sudden onset of rapidly progressive flaccid paraplegia. Imaging showed osteomyelitis involving D6-D11 with soft tissue lesion in D8-D11. Patient was started on ATT after decompressive surgery, fungal culture favoured Aspergillus flavus. Antituberculosis medications were continued along with antifungal medication, the interactions between antifungal and ATT highlighted. Conclusion: Aspergillus vertebral osteomyelitis and extradural abscess is a rare cause of compressive spinal myelopathy. Early definitive diagnosis remains challenging and needs high suspicion. Aspergillus osteomyelitis requires combined medical and surgical Andra Pradesh therapy. Early diagnosis and initiation of treatment helps in improving the outcome of this aggressive condition in immunocompromised patients.


An interesting case of diagnostic dilemma Creutzfeldt-Jakob disease/Hashimotos encephalopathy

L. N. R. Baddam, B. Vengamma, S. K. Rao, T. Naveen

Department of Neurology, SVIMS Hospital, Tirupati, Andra Pradesh, India

Introduction: Creutzfeldt-jakob disease and Hashimotos encephalopathy often show similar clinical presentation, but Hashimotos encephalopathy is particularly important as it is treatable. Thus, in case of probable Creutzfeldt - Jakob disease with high of antithyroid antibodies, immune modulation is performed with expectation of complete recovery from Hashimotos encephalopathy. Herein we provide the rare case where there is no effect of immune modulation. Case Details: 71 year old female medical professional presented with involuntary jerky movements of all the limbs for the past 6 months and progressive decline in cognition for the past 4 months. On examination there is a myoclonic non startle jerk of limbs with dystonic posturing. On evaluation there is significant elevation of antithyroid antibodies in serum (anti TPO = 65.7 IU/ml; anti TG = 48 IU/ml); cerebrospinalfluid (anti TG = 3.2 IU/ml) and follow up (serum anti TG = 45 IU/ml), other neuronal antibody and collagen vascular profile was negative. No evidence of primary on whole body PET. Magnetic resonance imaging showed hyperintensities in bilateral cortex, caudate nucleus and putamen. Serial electroencephalogram showed periodic sharp and slow wave complexes at a frequency of 1-1.5 HZ. Inview of antithyroid antibodies being positive patient was treated with corticosteroid pulse therapy subsequently IVIG for which she did not respond and later with Rituximab; however she did not respond and landed in akinetic mute state requiring three antiepileptics with optimal doses to control her continuous myoclonic seizures. Conclusion: This is a rare case where there is no improvement with immune modulation inspite of elevated antithyroid antibodies in a case of probable Cretzfeltd-jakob disease with features of Hashimotos encephalopathy. So this case highlights the importance of considering the no effect of immune modulation which is documented in only one case till date.


Bilateral cerebral hemorrhage in herpes simplex encephalitis: Rare occurrence

A. Miryala, R. Mahale, A. K. Shankar, M. Javali, P. Acharya, R. Srinivasa

Departmant of Neurology, MS Ramaiah Medical College, Bengaluru, Karnataka, India

The most frequent cause of sporadic encephalitis is herpes simplex virus (HSV) encephalitis (HSE). It carries a mortality of 70%, if left untreated. With the usage of acyclovir in the management of HSE and early diagnosis, the mortality rate has reduced to 19% 2. It causes hemorrhagic necrotising encephalitis involving the frontal and medial temporal lobes. Petechial cortical haemorrhages have been reported in HSE, however frank haematoma is rare. Bilateral cerebral haemorrhage in HSE has not been reported so far. A 71-year-old man was brought to the emergency department with the history of fever of 5 days duration, headache of 5 days and altered level of consciousness of 1 day duration. Fever was moderate degree, intermittent, associated with chills. Headache was holocranial, throbbing, and intermittent lasting for 2-3 hours, relieved with vomiting. There were no seizures, loose stools or limb weakness. He lapsed in to altered level of consciousness a day before admission. He became drowsy, irritable and was not recognising family members. Brain magnetic resonance imaging (MRI) showed left parieto-occipital and right occipital haemorrhages. Cerebrospinal fluid (CSF) examination showed positive polymerase chain reaction (PCR) for HSV deoxynucleic acid (DNA). The intracerebral haematoma in the above mentioned case reports involved the temporal lobe which is the typical area affected by the HSV. Our patient had bilateral cerebral haemorrhage involving right occipital and left parieto-occipital lobe. Venography did not reveal venous sinus occlusion. These sites have not been reported to be involved in HSE. Bilaterality of haemorrhage in HSE has not been reported so far. The occurrence of intracerebral haematoma in adults with HSE is rare. The usual location of haematoma reported is medial temporal lobe and are unilateral. Our patient had bilateral cerebral haemorrhage involving parietal and occipital lobe which is unusual. This has not been reported so far.


Acute bilateral vision loss in a case of pancreatitis

R. R. K. Gorantla, V. Shankar, P. Hazeena, Tripthi

Department of Neurology, Sri Ramachandra University, Chennai, Tamil Nadu, India

We report the clinical and radiologic features of a 31-year-old male who suffered incongruous binasal visual field defects and severe sudden visual loss due to hypoperfusion of bilateral lateral geniculate bodies following pancreatitis induced by ethanol. Complete neuroophthalmologic examinations were performed for visual acuity, color vision, pupillary reflexes, and visual fields. Additional testing was performed by means of MR imaging of the brain. The Vision loss was acute and severe and bilateral at the onset. The patient recovered visual acuity of 3/60 within 7 weeks. Color vision was abnormal in both eyes but gradually improved. Visual fields were characterized by incongruous binasal defects, but they reduced progressively. Cerebral MR imaging confirmed the presence of symmetrical lesions confined exclusively within both lateral geniculate bodies. These lesions were best seen on bilateral symmetrical T2/FLAIR bright signals with diffusion restriction seen involving the posterior end of optic tract fibers and lateral geniculate bodies-ischemic neuropathy involving optic tract and lateral geniculate fibers. Blood tests showed acute pancreatitis, hepatitis, acute kidney injury with impaired glucose tolerance, cultures excluded any microbial infection. It is noted cellular that the dysfunction within the lateral geniculate bodies results in visual symptoms. We conclude that shock secondary to pancreatitis may induce a bilateral isolated ischemia of the lateral geniculate bodies, resulting in incongruous binasal visual field defects and severe visual loss.


Study of cerebral magnetic resonance venography in patients of tuberculous meningitis

A. A. Bansod, R. K. Garg, N. Kohli, A. Jain, H. S. Malhotra, S. Kumar

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

Introduction: Central nervous system tuberculosis is a major cause of morbidity and mortality in the developing countries. Clinical spectrum of central nervous system tuberculosis includes meningitis, meningo-encephalitis, tuberculoma, tubercular abscess and stroke due to vasculitis and cerebral venous sinus thrombosis (CVST). Vascular endothelial damage leading to cerebral venous sinus thrombosis is relatively unusual. It commonly results from infections (bacterial, tuberculosis, fungal), tumours, trauma and inflammatory conditions. Despite high prevalence of tuberculosis in India, its association with cerebral venous sinus thrombosis has been scarcely reported. We intended to study prevalence, clinical profile, predictors and prognosis of cerebral venous sinus thrombosis in patients of tuberculous meningitis. Methods: We studied all consecutive patients of tuberculous meningitis admitted from August 2015 to July 2016 in Department of Neurology KGMU, Lucknow. They were assessed clinically and with imaging (Cranial MRI with MR Venography). Results: Out of 80 patients of tuberculous meningitis (42 male: 38 female), cerebral venous sinus thrombosis was found in 6 (7.5%) patients. In four patients superior sagittal sinus was thrombosed while in two patients transverse sinus was thrombosed. Seizure occurrence was more common in patients with CVST group in comparison to non-CVST group (83.3% in CVST group vs 24.3% in non-CVST group) and this difference was statistically significant (P= 0.006). There was no significant difference in occurrence of fever, headache, altered sensorium and focal neurological deficits in patients with or without CVST. Mortality was higher in CVST group (2/6; 33%) in comparison to non-CVST group (4/74; 5.4%) but this difference was not found to be statistically significant (p=0.06). Conclusion: Cerebral venous sinus thrombosis is an unusual complication of tuberculous meningitis and found only in 7.5% of patients. Seizure occurrence was significantly associated with presence of CVST.


To study the role of proinflammatory cytokines in tubercular meningitis

R. Shree, S. Sharma, M. Modi, K. Sharma, V. Singla, N. Khandelwal

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

Background: The role of cytokines in pathogenesis of tuberculous meningitis (TBM) continues to be controversial. It remains to be determined whether cytokines actually play a role in pathogenesis of TBM or whether these are innocent bystanders merely reflecting an effect rather than constituting a cause of inflammatory response. Aim and Objectives: To determine role of various proinflammatory cytokines in pathogenesis of TBM. Patients and Methods: Current study included 146 patients with TBM (90-Definite TBM; 56-Probable TBM), diagnosed as per criteria of Ahuja et al. which were modified to include CSF nucleic acid based tests. Serum and CSF levels (n=140) of various proinflammatory cytokines (IL-1β, IL-2, IL-6, TNF-α and IFNγ) were compared with serum and CSF proinflammatory cytokines of 99 healthy volunteers. The serum and CSF levels of various proinflammatory cytokines were correlated with various clinical, radiological and CSF parameters. Results: Levels of various serum and CSF cytokines (IL-2, IL-4, IL-6, IL-1β, IFN-γ and TNF-α) were significantly elevated in cases as compared to controls. A significant correlation was also found between (a) Higher stage of TBM and various cytokines (except for serum IL-6 and CSF IFN-γ); (b) CSF levels of TNF-α, IL-4 and IL-1β and severity of hydrocephalus; (c) CSF levels of IL1β and IFN-γ with presence of exudates on MRI; (d) Serum and CSF levels of all the cytokines with poor outcome as determined by death or as defined by S and E ADL score or by GOS (except for interferon gamma); (e) Serum and CSF IL-4 and IL1β with infarcts on imaging ; (f) CSF and serum IFNγ with border zone encephalitis; (g) Cytokines with lower CSF glucose and high CSF pleocytosis and proteins. Conclusion: Various proinflammatory cytokines play an important role in the pathogenesis of TBM and contribute significantly to severity of disease.


An interesting case of Foix-Marie-Chavagny syndrome

H. S. Nair, S. Gobinathan, S. Harisudan

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

Introduction: Opercular syndromes results from insult to the opercular areas due to vascular, infective or developmental causes. Anterior or the fronto-parietal opercular syndrome is referred as Foix-Marie-Chavgny syndrome which is commonly due to vascular cause. Here we present a rare case of anterior opercular syndrome secondary to an infective sequel. Case Vignette: 12 yrs boy born of a nonconsanguinous marriage presented with recurrent generalised tonic clonic seizures following a febrile illness at 18 months of age treated as tuberculous meningoencephalitis and now on antiepileptic drugs is now unable to close his mouth and to swallow liquids associated with drooling for the past 4 years. On examination he had buccolinguolabial apraxia with automatic voluntary dissociation. His higher functions and spinomotor system, cerebellarand autonomic examinations were within normal limits. He had an exaggerated jaw jerk and gag reflexes. Snout, grasp and palmomental reflexes were present. MRI brain scan revealed bilateral frontoparietal opercular T1 hypo & T2 hyperintense, diffusion nonrestricted lesions suggestive of a chronic infarct with gliosis and volume loss. EEG revealed presence of bilateral frontoparital slowing with occasional sharp waves. He was diagnosed with bifronto-parietal opercular syndrome secondary to tuberculous meningoencephalitis and his antiepileptics were adjusted for seizure control and was provided with swallow therapy. Conclusion: Foix-Marie-Chavagny syndrome though rare is a serious disorder of CNS causing a longlasting morbidity and mortality due to seizures, asthenia and aspiration. A multidisciplinary approach is needed in the management of this condition.


Application of Thwaites criteria in the diagnosis of TB meningitis

R. Daggumati, N. V. Sundarachary, A. Sridhar, U. Veeramma

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

Introduction: Tuberculous meningitis (TBM) is a devastating disease, claiming many lives in our country. There are many pitfalls in its diagnosis. We analyse the Thwaites scoring system, a validated TBM score in an Indian setting. Methodology: We analysed 248 patients with central nervous system (CNS) infection over a 3-year period and classified them based on the clinician's diagnosis and the Thwaites classification. Patients with discordant results-reasons for discordance as well as differences in outcome were also analysed. Results: Among the 248 patients, the final diagnosis of the treating physician was TBM in 74.6%, acute CNS infections in 18.5%, pyogenic meningitis in 5.2% and aseptic meningitis in 1.3%. Among these 248 patients, (92.8%) were classified as "TBM" by the "Thwaites" score and the rest as "Pyogenic". There was moderate agreement between the unit diagnosis and Thwaites classification (Kappa statistic = 0.53), as well as the Lancet scoring systems. Conclusion: There was only moderate agreement between the Clinician's diagnosis and Thwaites' score. There is need to prospectively evaluate the cost effectiveness of simple but more effective rapid diagnostic algorithm in the diagnosis of TBM.


Bilateral vision loss in a case of tuberculous meningitis

S. Thirunavukkarasu, S. Gobinathan

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

Introduction: Decreased vision occurs in 27% of tuberculous meningitis patients and the main causes are optochiasmatic arachnoiditis and optochiasmal tuberculoma. Case Vignette: In September 2014, patient developed fever associated with irritability, headache and neck pain of 1 week duration, was admitted to hospital in Dubai. For suspected meningitis, lumbar puncture was done. Results were suggestive of TB meningitis (CSF PCR positive) and ATT (anti-tuberculous therapy) was started 2 days after admission along with steroids. After unspecified time, patient deteriorated, became comatose and was put on mechanical ventilation. He was kept in ICU for 1 week. CT brain was repeated which showed hydrocephalus with multiple cerebral infarcts. Ventriculo-peritoneal shunting was done and tracheostomy was performed. He was weaned from ventilator, shifted to general ward where he remained vegetative for 9 months. He started regaining consciousness slowly. Tracheostomy tube and nasogastric feeding tubes were removed. Patient had residual quadriparesis, was blind bilaterally with perception of light only, and was discharged with ATT. He got admitted to our institution after discharge from the hospital in Dubai. MRI done here after admission showed evidence of pachymeningeal enhancement. He was treated as a case of optochiasmatic arachnoiditis and was asked to continue the ATT and was restarted on steroids. Discussion: Impairment of vision in tuberculous meningitis may be due to primary involvement of optic nerve by tuberculous lesion leading to optic neuritis, optochiasmatic arachnoiditis (OCA), and tuberculoma in the chiasmatic region or in the optic pathways, chorioretinitis, and secondary to hydrocephalus / increased intracranial pressure or due to ethambutol toxicity.


Two unusual cases of heat stroke at uncommon site in neuro-imaging and clinical and neuro-radiological corellation

P. Dhull, J. Shah

Department of Neurology, Army Hospital R & R Center, New Delhi, India

A case of 23 years, young male without any previous co-morbidity, presented with breathlessness followed by alter consciousness after 12 km. run. On hospitalization he was having recurrent episodes of generalized tonic-clonic seizure with one episode of upper g.i. bleed with high grade fever. On examination GCS was E1VTM1. MRI BRAIN showing bilateral high frontal lobe infarct with FLAIR hyper-intensity in bilateral medial temporal with right occipital region. 2 nd patient was a 36 years young male presented with unconsciousness after heat exposure, his clinical and biochemical profile suggestive of heat stroke. MRI BRAIN showing bilateral basal ganglia hyper-intensity. Both the patient showing gradual recovery over a period of time with residual impairment.


Correlation of RDW and neutrophil lymphocyte ratio with NIHSS score in acute ischemic and hemorrhagic stroke

H. Jayakumar, S. Balasubramanian, S. N. Sundaram, K. Krishnamoorthy, L. Sindhuja, S. Gobinathan

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

Introduction: Red cell distribution width (RDW) is a measure of red blood cell size and volume. Studies have shown that RDW and Neutrophil lymphocyte ratio (NLR) at the time of admission is an independent prognostic indicator for mortality in patients with acute stroke. The aim of this study was to analyse the variation in RDW and NLR in patients with acute stroke - ischemic and hemorrhagic, and to evaluate which among these two, correlate best with NIHSS score. Methods: A total of 101 patients, (66 - ischemic and 35 - hemorrhagic stroke) were included. NIHSS score was calculated for all patients at admission. Blood was drawn for RDW, Neutrophil and lymphocyte counts at the time of admission. Using Pearsons correlation coefficient, the correlation between RDW - NIHSS score and NLR - NIHSS score were analysed in both ischemic and hemorrhagic stroke. Results: In patients with Ischemic stroke the mean RDW was 15.39 ± 1.48, mean NLR was 4.92 ± 3.50 and mean NIHSS score was 15.16 ± 5.11. In hemorrhagic stroke mean RDW was 14.35 ± 1.96, mean NLR was 8.02 ± 4.82 and mean NIHSS score was 19.14 ± 5.64. In patients with hemorrhagic stroke, the correlation coefficient between RDW - NIHSS was -0.07 (p>0.05) and between NLR-NIHSS was -0.01 (p>0.05). However in ischemic stroke, the correlation coefficient between RDW - NIHSS was 0.68 (p<0.01) and between NLR - NIHSS was 0.54 (p<0.01), which indicates that the correlation of RDW and NLR with NIHSS score was significant only in patients with ischemic stroke, and RDW correlated best with NIHSS score, than NLR. Conclusion: This study emphasizes the importance of RDW and NLR and how it correlates with NIHSS score in patients with ischemic stroke and how a simple blood test, which is often ignored in CBC, offers great prognosticvalue.


The clinical and radiological spectrum of posterior reversible encephalopathic syndrome

M. Murarishetty

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

Introduction: Posterior reversible encephalopathy syndrome (PRES) is a neurotoxic state coupled with a variety of symptoms and distinct MR imaging appearance. Common symptoms include headache, vision change, paresis, nausea, and altered mentation. Generalized seizures are common and may lead to coma. Moderate-to-severe hypertension is present in 70-80% of patients. On CT or MR imaging studies, symmetric hemispheric watershed vasogenic edema predominates in the parietal and occipital lobes. Aims and Objectives: To review the clinical and neuroimaging findings in PRES patients with special reference to its atypical features. The purpose of the study was to distinguish the PRES from other diseases, as well as to identify it early, and treat promptly for the "reversibility" of the disease. Materials and Methods: Patients prospectively diagnosed as having PRES between October 1, 2014 to May 2016, were pooled. We performed a detailed review of clinical information, including demographics, presenting symptoms, medical history, and risk factors with 6 months to 2 yrs follow up in all cases. All patients underwent computed tomography of the brain or magnetic resonance imaging. Results: 40 patients with PRES were analyzed and followed for 2 yrs. Mean age was 30 yrs. Mean SBP was 190mmHg and mean DBP 110 mmHg. Etiologies of PRES included eclampsia (60%), HTN (20%), CKD (10%), autoimmunedisease (5%), sepsis (3%), cytotoxic medication (1%). Clinical presentations include seizures (80%), headache (60%), visual disturbances (40%), encephalopathy (15%), paresis (10%). MRI showed parieto occipital involvement in majority of cases (90%), frontal (65%), cerebellum (50%), and temporal lobe (40%), basal ganglia (20%). Radiological resolution seen in 90% of cases at 3 months follow up. Conclusion: Majority of patients with early diagnosis and prompt therapy were seizure free after initiating AEDs which were stopped after 6 months except in 15% cases of which majority were CKD and autoimmune diseases. As PRES is reversible condition, early and aggressive management of hypertension helps to prevent sequale morbidity and mortality.


Predictors of brain magnetic resonance changes in eclampsia and outcome assessment

A. K. Verma, R. K. Garg, Y. Pradeep, N. Kohli, R. Verma, H. S. Malhotra

Department of Neurology, KGMU, Lucknow, Uttar Pradesh, India

Background: Eclampsia is one of the important causes of posterior leukoencephalopathy syndrome. The purpose of this study was to assess the predictors of brain magnetic resonance imaging (MRI) changes and outcome assessment. Methodology: This was a prospective observational study conducted between August 2014 and July 2016 in a tertiary care hospital. The demographic, clinical, biochemical and radiological data of all eclamptic patients were recorded. All patients were followed up at 1 and 3 month and disability was assessed with modifiedrankin scale. Results: Out of 104 eclampsia patients, 74 were having MRI features suggestive of posterior leukoencephalopathy, 17 patients had normal MRI and 13 patients were having MRI findings other than posterior leukoencephalopathy. Comparison between group with MRI features of posterior leukoencephalopathy and normal MRI group showed significantly higher incidence of altered sensorium (94.6% vs 70.6%, p=0.010), status epilepticus (63.5% vs 29.4%, p=0.014), GCS ≤ 7 (25.7% vs 0%, p=0.018), vision impairment (56.8% vs 5.9%, p<0.001), serum creatinine (1.26 ± 0.62 vs 0.92 ± 0.41, p=0.034), serum uric acid (8.52 ± 1.64 vs 7.38 ± 1.61, p=0.011), serum LDH (1401.40 ± 736.78 vs 912.05 ± 542.43, p=0.012), fetal death (47.3% vs 17.6%, p=0.030) and maternal death (12.2% vs 0%, p=0.2) in the former group. One-month outcome assessed by modified Rankin Scale (mRS) in posterior leukoencephalopathy and normal MRI group showed good outcome (mRS ≤2) in 47.5% and 100% respectively. Similarly, 3 months good outcome (mRS ≤2) in both groups were 94.8% and 100% respectively. Conclusion: Eclamptic patients with posterior leukoencephalopathy on MRI were having poor clinical presentation, more deranged biochemical markers and higher maternal and fetal deaths.


A novel gene in Niemann Pick's Cdisease

Bhardwaj H

Department of Neurology, Army Hospital R & R Delhi Cantt, New Delhi, India

Niemann-Pick disease type C (NP-C) is a rare, autosomal recessive and a progressive genetic lysosomal lipid storage disease which is caused by mutations in the NPC1 or NPC2 gene. NPC1 mutations accounts for the majority of the cases which are about 90%. This is a highly heterogeneous disease and has varied clinical manifestations in various combinations involving almost all the body systems. The age at onset and the course can vary greatly from one patient to another. The combination of multiple signs and symptoms shows more diagnostic specificity for NP-C, which may aid with disease detection. There are certain combinations of the signs and symptoms which could strongly favour the diagnosis. These include: splenomegaly and vertical supranuclear gaze palsy (VSGP) among others. VSGP is a hallmark of NP-C and becomes highly specific of the disease when it occurs in combination with other manifestations. Our patient presented with a history of decline in scholastic performance since last 4 years along with multiple episodes of seizures since last one year. A clinical suspicion of neimann pick disease type c was made based on the combination of the symptoms listed above and she was worked up for the same. Two germline variations were identified. One in genomic position chr18:21131633; C>C/G (HET), c DNA position c.1612G>G/C (ENST00000269228) with amino acid change of p.G538R at exon number 10. Second was in genomic position chr18:21118573- 21118575delCCT, c DNA positon c.2972_2974delAGG (ENST00000269228) with amino acid change of p. Gln991_Gly992delinsArg at exon number 20. The first variation has been identified in the literature previously as a cause of neimann pick's disease type C. The second variation has not been identified so far in the reported cases of the disease and could be a novel variation causing this disease.


An interesting case report of salmonella encephalopathy

M. P. Bagul, A. Kushwaha, V. K. Rai, K. R. Bagul, N. Rai, J. S. Kathpal

Department of Neurology, Choithram Hospital, Indore, Madhya Pradesh, India

A 18-year-old male presented to Emergency Department with 8 day history of high-grade fever with chills, cough with scanty expectoration & loss of appetite, 3 day history of loose motions (2-3 episodes/day, semisolid, non bloody, non foul smelling, yellowish). Subsequently walking difficulty with slurred speech since 2 days & confusion with altered mentation since 1 day & once focal facial dyscognitive seizures 1 hour after hospitalization. No h/o headache, vomiting, blurred vision or any other complaints. No significant past history. Patient was febrile (101.8 F) with tachycardia & tachypnoea, conscious but not fully oriented, slurred speech, conjunctival hemorrhage with normal reacting asymmetric pupils (?), Moving all limbs with no focal neurological deficit, no pronator drift, no cerebellar/meningeal signs, normal deep tendon reflexes, both planters flexors. Blood investigations s/o pancytopenia, deranged Liver enzymes, hypocalcemia, hypoproteinemia with normal renal functions, urine r/m, chest X ray & negative HIV, Hepatitis panel (HAV, HBsAg, HCV, HEV), Dengue, Malaria, Widal test (S Typhi "O" & "H" in ratio of 1:160 each). MRI Brain s/o diffusion restriction and cytotoxic edema at splenium of corpus callosum. Cerebrospinal fluid examination (CSF) was normal, clear, normal pressures, no cells, proteins = 27 mg/dl, glucose =79/121 mg/dl, HSV-DNA PCR negative. The prominent finding was generalized spike & sharp wave periodic discharges at interval of 3 to 7 seconds throughout electroencephalograph (EEG) record. USG Abdomen was s/o submucosal edema of ileo-caecal region, multiple enlarged lymph nodes, echogenic free fluid in right iliac fossa. Patient was managed with antibiotics, antiviral, antiepileptics & symptomatic treatment. After 48 hours, Salmonella typhii isolated from blood cultures & dianosis of salmonella encephalopathy was made. On 8 th day patient had Generalized tremulousness of all 4 limbs involving distal extremities > proximal with wide based gait. Repeat MRI Brain was normal & all abnormal findings have disappeared.


Optic perineuritis - The sheath is to blame

A. Takkar, A. Kumar, V. Lal, A. K. Pandit, J. Sachdeva

Department of Neurology, PGIMER, Chandigarh, India

Introduction: Optic perineuritis is an uncommon inflammatory disorder involving the optic nerve sheath. Most cases are isolated and idiopathic, but may occasionally occur as a manifestation of a specific infectious or inflammatory disorder, such as syphilis, Wegener granulomatosis or giant cell arteritis. Objectives: To present three cases of optic perineuritis with varied clinical presentations. Cases: (1) 19-year-old boy presented with right hemicranial headache, swelling over right eye and periorbital area for 2 months. Clinical examination revealed decreased visual acuity and disc edema in right eye. (2) 12-year-old girl presented with complaints of progressively increasing redness and swelling of right eye for 10 days, associated with diminution of vision and headache on the same side. She revealed history of two episodes in past with sequential involvement of one eye followed by the other, 2 years back. Optic fundus examination showed right disc edema with dilated tortuous vessels. (3) A boy in his teens presented with loss of vision in left eye for one day. He had vesicular lesions on left side of forehead and upper eyelid (suggestive of herpes zoster infection), which erupted 10 days ago. Left eye had hand motions vision, intraocular pressure 10 mm Hg, a relative afferent pupillary defect, optic disc edema, dilated and tortuous veins, and multiple retinal hemorrhages. Fluorescein angiography revealed extremely sluggish blood flow in the arteries, delayed venous filling and frosted branch angiitis in the posterior pole. MR imaging of orbits clinched the diagnosis in of optic perineuritis in all these cases. Conclusion: These case reports highlight optic perineuritis and stress the fact that all disc edemas with diminution of vision are not just optic neuritis. The sheath has to shoulder the blame too.


Relapsing and remitting anti-NMDA receptor encephalitis with prior spontaneous recovery- a rare entity?

A. Doraiswamy, S. Ramaratnam

Department of Neurology, SRM Institute for Medical Sciences, Chennai, Tamil Nadu, India

Introduction: Anti N-methyl D-aspartate receptor (NMDAR) encephalitis is a distinct autoimmune entity predominantly affecting children and young adults. Its clinical features include impaired cognition, psychosis, seizures, catatonia, movement disorders and autonomic instability. Although relapses following treatment have been noted in 20-25% of cases, recurrent episodes of complete spontaneous recovery are rare. Methods: A 34 year old lady was admitted in January 2016 with complaints of altered behavior and one episode of seizure, subsequently developed dystonia of her upper limbs, infrequent catatonic spells and sleep disturbances during the hospital stay. She first became symptomatic 10 years back, when she developed fever, altered sensorium and status epilepticus requiring intensive care for 3 months, and was discharged with a presumptive diagnosis of viral meningitis, also later developing transient depression. Following an asymptomatic period of 4 years, she developed status epilepticus during her pregnancy, followed by post-partum psychosis. She responded to routine medical management in both episodes and showed complete spontaneous recovery without immune modulatory therapy. Results: Investigations done during the current admission were repeatedly positive for anti-NMDAR antibodies. Anti-thyroid peroxidase and antinuclear antibodies were negative. Magnetic Resonance Imaging (MRI) of the brain showed cerebral atrophy. Video telemetry showed presence of diffuse cerebral dysfunction with more emphasis on the left side. PET scan of the brain showed global hypometabolism with relative sparing of the left parietal and temporal lobes; patchy areas of preserved metabolism in bilateral cerebellar cortex. Co-existent neoplastic disease was ruled out by Positron Emission Tomography (PET) scan of whole body. She was managed with intravenous steroids initially followed by intravenous immunoglobulin after which she showed minimal improvement. Conclusion: Anti NMDAR encephalitis presenting with recurrent and spontaneously recovering prior neuropsychiatric episodes without immunomodulatory treatment has only been rarely reported. As it is just 9 years since its first description, these could represent previously undiagnosed cases.


Meningo encephalitis clinical feature and etiological profile

A. S. Choudhari, A. Senapati, D. S. Guin

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

Intoduction: Acute meningitis and acute encephalitis constitute significant public health problems worldwide. Although bacteria are predominantly responsible for meningitis, viruses can cause both meningitis and encephalitis with equal frequency. It is often difficult to reliably differentiate meningitis and encephalitis clinically. Therefore, the term acute meningoencephalitis (AME) is used to denote both conditions. Despite advancements in diagnostic techniques and antimicrobial therapies, AME remains an emergency infectious disease with a high fatality. By conducting this study I will be able to know clinical features and different etiologies of meningoencephalitis and which will help to modify treatment modalities. Objective: To evaluate clinical features of meningoencephalitis and to analyze the etiology of meningoencephalitis. Materials and Methods: All patients admitted neuromedicine department BIN between march 2015 to may 2016 with features of meningoencephalitis were evaluated excluding the patient <12 years age and metabolic cause for altered sensorium. History and examination was performed in a pre structured proforma. Relevant investigation Imaging and CSF study and specific etiology guided investigation done. Results: This is ongoing study out of 80 patients studied 34 were female and 46 were male patients, among this 31 were TBM, 26 were PM, 12 were viral, 3 were cryptococcal and in 8 cases no definitive cause found. Conclusion: Meningoencephalitis is a heterogonous condition. Many causative agents can result in a very similar kind of presentation. In our study group TBM and PM constituted major chunk followed by viral and cryptococcal meningitis. Men were affected more than women.


Clinical profile of longitudinally extensive transverse myelitis with and without aquaporin 4 antibodies

R. Das, P. S. Deb, N. C. Borah

Department of Neurology, GNRC Hospitals ltd, Dispur, Assam, India

Background: LETM refers to inflammation of the spinal cord causing T2 hyperintensity on spinal MRI that extends over three or more vertebral segments. Although LETM is a characteristic feature of NMO, but such spinal lesions can also occur in various other autoimmune and inflammatory diseases that involve the CNS eg: Multiple sclerosis. Methods: Retrospective evaluation of all consecutive patients (15) admitted to Neurology Department, GNRC Hospital between 2012 to 2015 satisfying criteria for LETM was considered. Eligible patients were subjected to clinical, laboratory, spinal fluid analysis, brain & spinal cord MRI. Blood for Aquaporin- 4 Ab was done in selected cases. Results: Ten out of 15 patients were female; age ranges between (17-63). All patients had LETM extending 3 or more contagious vertebral segment in MRI. Optic neuropathy was present in 3 patients along with LETM. CSF study done in 5 patients showed lymphocytic pleocytosis with raised protein. Aquaporin-4 Ab was positive in 5 patients, negative in 4 patients & not done in 6 patients. All the patients were treated with IV methyl prednisolone (5 days) followed by tapering dose of oral prednisolone for 6 months and Azathioprine. Out of 15 patients, 8 showed significant improvement, 3 showed mild improvement of muscle power, 4 showed significant improvement but had relapse after one year. All the patients who had relapse were Aquaporin 4 Ab positive. Conclusion: LETM due to NMOSD is different from MS from treatment point of view and outcome. In our study as well as in the literature it has been found that relapse is more common in Aquaporin-4 Ab positive cases. However large study is required to describe different characteristics. So, Aquaporin-4 Ab is the preferred test at the present time to predict a risk of recurrent attacks of LETM.


Thymomatous mysthenia gravis-unusual associations: Report of two unusual cases

V. K. Reddy, M. Y. Reddy, J. M. K. Murthy

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

Introduction: Thymomatous myasthenia gravis may rarely be associated with other disorders. This paper presents two patients with thymomatous myasthenia with unusual associations. Case 1: A 31-yr-male was diagnosed as a case of thymomatous myasthenia gravis (anti-acetylcholine receptor antibody positive) 8 years back for which he underwent thymectomy (thymoma grade IIb) 5 years ago. He was in remission on prednisolone and azathioprine. In the recent two years he had fluctuating course for which he was admitted thrice in the hospital, once for myasthenic crisis requiring non-invasive ventilation and immunomodulation. He had one episode of malena for which evaluation revealed pancytopenia. Bone marrow biopsy was suggestive of aplastic anemia. Aplastic anemia was attributed to azathioprine and was stopped. He continued to be symptomatic and blood counts worsened. Diagnosis of aplastic anemia due to thymoma was considered and was started on cyclosporine with which anemia improved and blood counts improved at 4-month follow-up. Case 2: A 55-yr-male presented with bilateral ptosis, facial weakness and quadriparesis with diurnal fluctuations since 2 months. He showed significant decrement on slow RNS, elevated anti acetylcholine receptor antibodies and thymoma. He was noted to have significant microcytic, hypochromic iron deficiency anemia. Work-up revealed multiple chronic non-specific inflammatory ulcers in second and third part of duodenum and stomach. Contrast CT abdomen showed hypodense lesion in the head of pancreas and gastrin levels were elevated five times the normal range. Diagnosis of multiple endocrine neoplasia was made. Conclusion: Aplastic anemia is a rare complication of thymoma. Association with gastrinoma and multiple endocrine neoplasia is even more rarer and described in a case report in literature. Such associations must be screened in thymomatous myasthenia as they have management implications.


Neuromyelitis optica complicated with mixed connective tissue disease

N. Deepak, J. B. Agadi, A. Netto, N. Karthik, P. Kumar, D. C. Janardhan

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

Introduction: Neurological manifestations are rare in Mixed Connective Tissue Disease (MCTD). The most common of them are Trigeminal neuralgia, headache and aseptic meningitis. Incidence of NMO is 0.05 to 0.4 per lakh population. Only one case of MCTD with neuromyelitis optica (NMO) has been published till now. A 30-year-old lady presented with acute paraplegia with bladder and sensory involvement. Apart from this acute deficit, she had a progressive limb girdle syndrome for past 9 months that was not evaluated. She had Raynaud's phenomenon, tightness of skin, polyarthralgia and photosensitivity. Past history was significant in the form of an Area Postrema syndrome (persistent hiccups & vomiting) in 2007, optic neuritis in 2009, and longitudinally extensive transverse myelitis (LETM) in 2011 and hypothyroidism in 2015 from which she had recovered well. Personal history was significant for menorrhagia and three second trimester abortions. Examination revealed sclerodactyly, thick and dry skin, afferent pupillary defect with optic atrophy and hypotonia of upper limbs with spasticity of lower limbs. Power was grade 3 in upper limbs, grade 1 in lower limbs with brisk reflexes in lower limbs and extensor plantar. Sensory level was at C8. Clinical diagnosis was acute LETMs with polymyositis. Extensive evaluation showed raised levels of inflammatory markers & CPK and positive serum Aquaporin 4 antibodies and n-RNP. She was treated with steroids and disease modifying agents with good recovery. Conclusion: It is necessary for the clinician to actively evaluate for presence of coexistent NMO in patients with vasculitic and connective tissue disorders since the choice of immunomodulators and prognosis varies widely.


IgG4 related hypertrophic pachymeningitis: Report of two case

D. S. Deshmukh, M. Reddy, N. V. Chaudhary, S. K. Jaiswal, J. M. K. Murthy

Department of Neurology, The Institute of Neurological sciences, Care Hospitals, Hyderabad, Telangana, India

Introduction: IgG4-related disorders are rare and hypertrophic pachymeningitis (IgG4-RHP) is one of the forms of these groups of disorders. Case 1: A 52-yr-old male presented with holocranial headache of 4-year duration and decreased visual acuity left eye, and decreased sensation left side of face with paroxysms of electric shock like sensation of 2-year duration. Examination revealed left optic neuropathy and sensory trigeminal neuropathy. Contrast brain MRI revealed extensive contrast enhancing dural thickening along the left cerebral convexity, posterior third of falx, tentorial leaflets and dura of the posterior fossa. CSF analysis: lymphocytic pleocytosis and elevated protein. ESR was 86 mm 1 st hr. Rest of the relevant investigation were normal. Meningeal biopsy showed fibrocollagenous tissue with patchy dense lymphocytic infiltrates and few plasma cells and histiocytes infiltrates. Serum IgG4 levels were elevated (338 mg/dl). Initially he was treated with steroids, after IgG4 test, he is being treated with IV rituximab. Case 2: A 54-yr-old female known patient presented with holocranial headache and nasal stuffiness of 5-year and seizures of 3-year duration. Examination was unremarkable. Contrast brain MRI revealed extensive contrast enhancing dural thickening along the right frontal convexity, anterior interhemispheric fissure and pansinusitis. CSF analysis: lymphocytic pleocytosis and elevated protein. ESR was 55 mm/1 hr hour. Rests of the relevant investigations were normal. Biopsy showed fibrocollagenous tissue with patchy dense infiltrate composed of sheets of plasma cells. Immunohistochemistry for IgG4 was positive. Serum IgG4 levels were elevated (241 mg/dl). She was treated with four weekly doses of IV rituximab. Conclusion: Possibility of IgG4-RHP should be excluded in patients with hypertrophic pachymeningitis as it is a potential treatable condition.


Neurobehcet's disease: A report of four clinically distinct cases

K. R. Rana, C. M. Sharma, B. L. Kumawat, A. Vyas, D. K. Khandelwal, V. Sharma

Department of Neurology, SMS Medical College, Jaipur, Rajasthan, India

Introduction: Behcet's disease (BD) is a multisystem inflammatory disorder of unknown aetiology and characterized by the triad of recurrent oral and genital ulcers with uveitis. Neurological manifestations are seen in 10 to 20 percent of patients and classified into two categories: a parenchymal and none parenchymal involvement. Aim: To explore the clinical manifestations of Neurobehcet's disease. Results: All our four patients fulfilled the criteria for Behcet's disease. Among 4 patients 3 were male. All four patients had a history of recurrent oral ulcers and uveitis. Two patients presented with a chronic headache and diagnosed to have cortical venous sinus thrombosis. One of these patients also had brainstem manifestation. The 3 rd patient presented with myelitis. The 4 th patient had a recurrent generalized tonic-clonic seizure and chronic headache and her MRI brain was suggestive of multiple sclerosis-like picture and pachymeningitis. All patients had positive Pathergy test. All patients were treated with azathioprine and supportive treatment. All patients responded well to treatment. Conclusion: Though neurological manifestations of Behcet's disease are rare, a high index of suspicion is required. Any patient presented with a refractory headache and recurrent neurological illness, Neurobehcet's disease should be considered as a differential diagnosis because it is a potentially treatable condition. Therapeutic strategies include immunosuppressant therapy.


Seronegative anti-n-methyl-d-aspartate receptor encephalitis

A. G. Thomas, M. Madhusudanan, R. Thomas, P. Byju

Department of Neurology, Pushpagiri Institute of Medical Sciences and Research Centre, Tiruvalla, Kerala, India

Generally, a patient with limbic encephalitis (LE) is not investigated for an associated ovarian teratoma, if Anti-N-methyl-D-aspartate receptor antibody (antiNMDAR Ab) is negative. We report here a patient who had presented with classical clinical features of AntiNMDAR encephalitis with negative CSF NMDARAb, who was found to have associated ovarian teratoma and had remarkable clinical improvement with removal of the tumour. A 24 year old unmarried female, presented to us with fever and psychiatric symptoms in the form of anxiety, anger, insomnia, fear, delusions, hallucinations and hyper-religiosity. Her word output decreased gradually. Later she became catatonic and mute. The patient was also noted to have orofacial dyskinesia and urinary incontinence. On examination the patient was keeping eyes tightly closed, was grimacing to pain but not obeying commands. The blood pressure recordings and pulse rate fluctuated over a wide range. She had orthostatic hypotension and was sweating profusely. She had generalized rigidity with exaggerated tendon reflexes and extensor plantar response. MRI brain didn't reveal any abnormality. EEG showed generalized slowing. HSV polymerase chain reaction (PCR) was negative. Serum and CSF samples were tested negative for antibodies against voltage-gated potassium channel and N-methyl-D-aspartate receptors (NMDARs). Serum paraneoplastic panel of neuronal antibodies were also negative. But in view of her symptoms, the possibility of autoimmune encephalitis was strongly considered. So a CT Abdomen was done which revealed a teratoma left ovary. Hence CSF study was repeated which showed nervous system specific unclassified antibody, but anti NMDA antibody was again negative. Her tumor was removed and she was started on immunosuppressive therapy. The patient attained remarkable recovery in the next few weeks. The case is presented to highlight the fact that the neurophysicians should not defer from investigating for ovarian tumour in patients with a strong clinical suspicion of autoimmune encephalitis even if antiNMDAR antibody is negative. In all of these patients, immunosuppressive treatments should be seriously considered, once the alternate possibilities are reliably ruled out.


Neuromyelitis optica spectrum disorders - A retrospective comparative study of serum and CSF NMO IgG status in Indian patients

S. Kannoth

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

Background: CSF testing for neuromyelitis optica (NMO) IgG complements serum evaluation in the diagnosis of neuromyelitis optica spectrum disorder. However no data is available from Indian population regarding this. Objectives: To compare the NMO IgG status in serum and CSF in a series of Indian patients with suspected NMO. Materials and Methods: Retrospective observational study based upon the samples tested in the neuroimmunology laboratory under the department of neurology of Amrita Institute of Medical Sciences, Kochi during the period of May 2013 to December 2015. Patients were included in the study only if the serum and CSF samples were tested together for NMO IgG. Indirect immunofluorescence with cell based assay for AQP4 antibody was used. Results: During the study period, a total of 640 samples (595 patients) were tested for NMO IgG, 45 of them were tested with serum and CSF samples simultaneously. Only two (4.4%) of them were positive with serum testing while four (8.9%) patients were positive with CSF testing (p=0.632). Discussion: Our study shows that CSF IgG testing yields better results than serum testing though it was not statistically significant. Conclusion: Testing of CSF NMO IgG improved the diagnostic out come in our cohort of suspected NMO patients.


Steroid responsive encephalopathy with autoimmune thyroiditis: A rare treatable entity with varied manifestations

N. Jain, V. Sardana, P. Ojha

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

Background: Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT), also known as Hashimoto encephalopathy, is a poorly understood and often misdiagnosed entity. The most common clinical features include seizures resistant to anti-convulsive treatment, headache, hallucinations, stroke like episodes, impairment of cognitive function, behavioural and mood disturbances, ataxia and presenile dementia, together with the presence of high thyroid antibody levels, especially against thyroperoxidase (TPO). In most cases, the thyroid function is normal or decreased. Corticosteroid treatment usually provides a dramatic recovery. Steroid intolerant patients may respond to cyclosporine or azathioprine. Several case reports also describe good clinical results following plasma exchange and intravenous immunoglobulin. Cases: We hereby present three female patients diagnosed with this entity during last 11 months, who presented with varying neurological complaints and had an excellent clinical response to steroid therapy. (1) A 27 year old female presented with insidious onset gradually progressive truncal and limb ataxia since last 1 year. She was a diagnosed case of hypothyroidism on thyroxine with current normal thyroid profile and anti-TPO antibodies strongly positive. After one week of steroid therapy, her ataxia markedly improved. (2) A 60 yrs old euthyroid female presented with progressive declining cognitive function and psychotic symptoms since last one year with strongly positive Anti-TPO antibodies. After starting steroid therapy, patient had improvement in her MMSE scores and the psychotic symptoms. (3) A 25 yrs old female patient presented with progressive spastic paraparesis and was diagnosed to have LETM. Patient was a known case of hypothyroidism and was strongly positive for Anti-TPO antibodies. Patient had a dramatic clinical improvement with steroid therapy. Conclusion: Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a poorly understood and often misdiagnosed entity because of its varied presentation. A high degree of suspicion can lead to early diagnosis and good prognosis as it shows dramatic response to corticosteroid therapy.


Prevalence and profile of attention impairment in patients with Multiple Sclerosis using novel clinical and electrophysiological tools - a case control study from a tertiary care centre in South India

S. A. Chinnadurai, G. Shankar, S. A. Venkatesan, L. N. Ranganathan, B. Samivel

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

Objectives: Knowledge regarding the prevalence of attention impairment in MS and the value of tools used to detect it are lacking. This study aims to uncover the prevalence of attention impairment in MS and tries to identify effective clinical tests to assess attention in MS patients. Methods: 50 consecutive MS patients attending the Neurology OPD in Madras Medical College, Chennai from May 2015 to February 2016 with equal number of age, sex, and educational status matched controls were included. Disability was measured with EDSS (Expanded Disability Status Scale) and depression with CESD (Center for Epidemiologic Studies Depression) scale. Digit forward and backward span, symbol digit modalities test, paced auditory serial addition test (3 second version), and trail making test A and B, gorilla in the room test, and modified Stroop test in addition to P300 latency were used to assess attention. Results: Patients had a significantly poorer performance in digit backward span, trail making test B, modified Stroop test, and gorilla in the room test in addition to P300 latency compared to controls. 34% (n = 17) had impairment in three or more tests for attention. Patients with attention impairment were more likely to have progressive forms of MS, higher EDSS or CESD score. P300 latency was the most sensitive (94.1%) measure to detect attention deficits. Conclusion: Attention impairment is widely prevalent in MS and P300 latency is the most sensitive measure which can be used routinely as a screening tool to detect it.


Idiopathic hypertrophic spinal pachymeningitis

D. Chezhian, F. Jason Ambrose, M. Arasu

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

Our patient was a 42 year old gentleman who presented with history of low back ache radiating to the left thigh with numbness and abnormal sensations in the lateral aspect of left thigh. MRI lumbosacral spine showed an intradural mass lesion in the sacral canal extending from S1 to S5 level which was T1 isointense and T2 hyperintense with uniform contrast enhancement, reported as plexiform neurofibroma. Sacral laminectomy and excision biopsy of the mass was done which revealed features of hypertrophic pachymeningitis, sacral region. Serum IGG4 levels were also done and found to be markedly elevated. Hence he was diagnosed as Idiopathic Hypertrophic Spinal Pachymeningitis. Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a rare inflammatory disorder that leads to spinal cord or root compression. The tyical MRI finding is a long extramedullary mass of low T2 intensity with peripheral enhancement. Biopsy and serum IGG4 levels provide confirmation of the diagnosis. We present this case because of its rarity.

Poster Session 02; Demyelinating Disorders and Clinical Neurophysiology11:00-14:00 h, Friday, November 11, 2016


Clinical experience in a single case of NMO-connective tissue disorder overlap syndrome

M. Janarthanam, K. Venkatraman, G. Sarala, S. Gobinathan

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

Objective: To acknowledge the importance of diagnosing Neuromyelitisoptica in SLE patients presenting with myelitis and optic neuritis. Background: SLE-NMO overlap syndrome is a rare manifestation. Such cases pose a diagnostic and therapeutic challenge especially when complicated by multiple organ system involvement. Case Report: 27 year old female presented with headache, sudden onset weakness of all 4 limbs with difficulty in voiding urine. Examination revealed quadriplegia, optic atrophy of right eye and disc edema of left eye. Her past history revealed that she was a known case of SLE diagnosed 3 years back and treated with immunosuppressants. She was withdrawn of immunosuppresantson account of her stable clinical status and ANA negative titres. 6 months later she developed right eye optic neuritis and was treated with Intravenous steroids, discharged and lost follow up. Her imaging in the current admission revealed a C2-C3 central cord predominant transverse myelitis and left eye optic neuritis and superior sagittal sinus thrombosis. Herserology was positive for IgG aquaporin4 antibody and B2 microglobulin. Her weakness improved partially. She developed recurrent spasms of limbs and our investigation showedhypocalcemia and heavy proteinuria. Her renal biopsy revealed stage 4 lupus nephritis. She had SLE-NMO-APLA overlap. She succumbed to arrhythmia secondary to refractory hypocalcemia. Conclusions: NMO should be strongly suspected in SLE patients presenting with myelitis/optic neuritis. Positive NMO serology attributes such neurological manifestations to NMO. Heavy proteinuria can excrete ANA complex and can give false negative ANA values, therefore caution is warranted before weaning immunosuppresants. We describe a very rare presentation of three disorders namely SLE, NMO and APLA in a single patient.


A case series of isolated brain stem demyelination

R. Nadig, T. Mathew, G. R. K. Sarma, D. Desoza

Department of Neurology, St Johns Medical College, Bengaluru, Karnataka, India

Background: Inflammatory demyelinating diseases of the central nervous system include multiple sclerosis, ADEM, NMO disorder; isolated brainstem dysfunction due to demyelination is not commonly described. Objective: To describe etiology, clinical features and outcome of patients with isolated brainstem demyelination. Methods: We identified the patients affected by brainstem dysfunction related to a single Isolated Brain stem demyelination not satisfying the diagnostic criteria for Multiple sclerosis, ADEM, NMO disorder and osmotic demyelination. Results: Six (3 male and 3 Female) patients were included in the study, with median age of 42 years, duration of symptoms ranged from 3 days to 6 weeks. Three patients presented with ataxia and hemi paresthesia, one patient with diplopia and hemi paresthesia, one patient with diplopia and ataxia and one patient with intermittent hiccup and vomiting of 6 weeks. One patient had hypertension with dyslipidemia. There was no preceding history of fever in all these patients. Five patients were initially treated as stroke and one patient as dyspepsia elsewhere. Evaluation at our center with MRI brain and spine revealed demyelinating plaques in medulla in four patients, in pons in one patient and midbrain in other patient. CSF examination of all patients was normal with no oligoclonal bands. ANA, ANCA, APLA, VDRL and HIV tests were negative. Metabolic parameters, ACE levels and VEP were normal. Serum for Aquaporin-4 antibody was positive in only one patient. All patients were treated with methylprednisolone and showed a good response. Median follow up was for 6 months; four patients did not have new lesion on repeat neuroimaging. Patient whose NMO antibody was positive developed optic neuritis, one patient succumbed to aspiration pneumonia. Conclusion: Isolated brainstem demyelination is a rare entity. It could represent a variant of inflammatory demyelinating CNS disorder. They respond well to immunosuppression.


Fulminant spinal form of MS - A case report

M. Sundararaju, S. Gobinathan

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

Introduction: Marburg variant MS is a rare form of MS with a fulminant course, presenting with bilateral large acute lesions in the subcortex and brainstem progressing to death over a period of few months. There are no case reports of patients presenting with recurrent and fulminant demyelination involving only the spinal cord. Case Vignette: 25 year old female presented with numbness in the right lower limb followed by vision loss in left eye of acute onset, treated by an Opthalmologist with IV steroids for 5 days with partial improvement in vision. 15 days later she started to have numbness over her right upper and lower limb. MRI was done, which showed a small plaque with open ring enhancement in the left frontal region and spinal cord expansion with hyperintensity in the cervical cord C 5 level and dorsal cord D 10 level with contrast enhancement. She was admitted and started on IV methylprednisolone for 5 days followed by oral taper. CSF oligoclonal band was positive and aquaporin 4 negative. P 100 latency prolonged on left side. Vasculitis workup was negative. HIV and VDRL nonreactive. After 20 days of hospitalization she had right cerebellar signs. Next day she developed acute onset of quadriparesis with respiratory distress. Repeat MRI showed a new lesion in the upper cervical cord at the level of C2 with complete disappearance of lesion in the left frontal region with no new lesions in the brain. She was intubated and shifted to IMCU for ventilator support and was started on plasmapheresis. Conclusion: We report this case, as the patient had a very fulminant course while on treatment and had recurrent demyelination involving the spinal cord.


NMDA receptor Ab encephalitis presenting with acute psychosis - A case report

N. Elango, V. Chandramouleeswaran, V. Ramakrishnan, S. Gobinathan

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

A previously healthy 13 years old female child presented with single generalised seizure, followed two weeks later by mood and behavioural changes, altered sensorium, confusion, memory loss, jerky movements of limbs and repetition of words. There was no history of headache or fever. On examination, no cranial nerve palsy or limb weakness. Metabolic and infectious investigations (including CSF analysis), and rheumatological workup were all normal. MRI Brain shows normal study. EEG shows slow wave delta frequency, no sharp wave discharge and evidence of bilateral paroxysmal dysfunction. Child was started with antipsychotic and anticonvulsive medications. Serum and CSF tested positive for anti-NMDAR antibodies. Immunoglobulin i.v ig and i.v methylprednisolone given for five days followed by oral prednisolone for six months. Child had slow and progressive recovery and now completely normal. Conclusion: Anti-NMDAR encephalitis should be considered in differential diagnosis of encephalitis when acute behavioural changes, seizures or dyskinesias present in previously healthy children. It is a potentially treatable disorder and can be lethal, if it has been misdiagnosed.


Recently described but worth recognizing!

A. Choudhary, D. Naheed, M. Goyal, L. Balan, B. Radotra, V. Lal

Department of Neurology, PGIMER, Chandigarh, India

Background: IgG4 related disease has been recently recognized in literature and involves multiple vital organs. Hypertrophic pachymenigitis is one such manifestation of this treatable condition. Case History: A 40 year male presented with 4 months history of headache associated with multiple cranial nerve palsy in the form of right 5, 6, 7, 8, 9, 10 and 12 nerve involvements. Hemogram and serum biochemistry was normal. CSF examination revealed 60 cells, predominantly lymphocytes, high protein (128 mg/dl) and sugar of 56 mg%. All other investigation in CSF was unremarkable. MRI brain was performed which revealed pachymeningitis involving the right temporal convexity extending to the right temporal leaf into the posterior fossa and almost encassing the the spinal cord at C1-C2 level. ANA/ANCA, ACE levels were normal. Dural biopsy was performed which showed areas of extensive fibrosis and the infiltrates comprised of plasma cells, lymphocytes and histocytes. Numerous IgG positive plasma cell (120-130/hpf were seen with majority of them positive for IgG4 (70-80/hpf. IgG4 to IgG ratio was 60-70%. Serum levels of IgG4 were found to be very high (270 mg%). Patient was started on IV steroids with marked improvement in symptoms. Dicussion: IgG4 related disease frequently affects the pancreas, salivary glands and lymph nodes, but can involve almost any tissue. It is estimated that IgG4 related hypertrophic pachymeningitis may account for a high proportion of cases of hypertrophic pachymeningitis once considered idiopathic. The presence of oligoclonally restricted IgG4-positive plasma cells within inflammatory meningeal niches strongly suggests a specific response against a still unknown antigen. High-dose glucocorticoids are still the treatment of choice for this entity because other immunosuppressives have shown variable response in reducing hypertrophy. Conclusion: Many of the hypertrophic pachymeningitis once considered idiopathic, might be IgG4 related. Thorough investigations should be performed as this entity is highly amenable to treatment.


Neuro behcet presenting as rhombencephalitis - A case report

R. Rangaraju, S. Gobinathan

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

Introduction: Rhombencephalitis is the inflammation of hind brain - brain stem and cerebellum. Around 6% of Behcets syndrome presents with neurological manifestation as rhombencephalitis, Neurobehcets. Case Vignette: 27 yr old Mr. R from Thiruvarur, came with the complaints of double vision for 2 months, difficulty in swallowing with nasal regurgitation , weakness of left upper limb and lower limb, numbness of left half of body and face, unsteadiness while sitting unsupportedly, head shaking for 2 weeks with altered sensorium for 2 days prior to admission. Patient's symptoms started after an episode of febrile illness which lasted for a week. Work up for fever was normal. On examination patient had bilateral partial ptosis, primary gaze left exotropia, horizontal gaze restriction and up gaze restriction with vertical nystagmus, impaired convergence, bilateral LMN facial palsy, absent gag, head turned to Left, tongue movements restricted without deviation. Patient also had bipyramidal signs, left spinothalamic signs and bil cerebellar signs with dysarthria. Investigations including blood routine, CSF, calcium, S. ACE, vasculitic work up, autoimmune antibody panel including GQ1b were all negative. MRI showed T2, FLAIR hyper intensities in Right thalamus, brainstem region (red nucleus spared) and was contrast enhancing. Radiologist opined as Rhombencephalitis to R/O Neurobehcets. Retrospectively there were no h/o recurrent oral or genital ulcers. Other causes of Rhombencephalitis including listeria, TB, sarcoidosis, Bickerstaff encephalitis, and lymphomas were all negative. Our patient initially showed dramatic improvement with steroid but later succumbed to the illness related complications. Conclusion: This case presented with rhombencephalitic features, with imaging s/o nuerobehcets and is presented in view of its rarity and classical brainstem meningoencephalitic presentation of Neuro Behcets syndrome.


A study of spectrum and severity of autonomic dysfunction in NMO and NMO spectrum disorders

M. Alexander, T. V. Murthy, S. Babu, A. T. Prabhakar, A. Sivadasan

Department of Neurology, CMC, Vellore, Tamil Nadu, India

Background: In NMO and spectrum disorders, which has predilection for critical areas in brain and spinal cord leading to high prevalence of autonomic disturbances. Aims and Objectives: To asses the frequency, spectrum and spectrum of autonomic dysfunction in patients with NMO spectrum disorders. Materials and Methods: 20 patients were studied using the revised criteria for NMO and spectrum disorders (2006). The autonomic dysfunction was assessed with COMPASS and CASS score and Autonomic Function tests using HUTT with Finopres TM and the frequency and severity was assessed. Results: There were 20 patients (13 F 7M) and 95% had autonomic dysfunction (AD) during the active phase of the disease. On AFT, parasympathetic dysfunction (80%) was more involved than sympathetic (65%). There was correlation between EDSS scores in the active phase due to involvement of specific areas in brain and spinal cord. Conclusion: The prevalence of Autonomic dysfunction is very high in NMO and spectrum disorders. There was correlation between EDSS during active phases of disease with the MRI changes involving specific areas including hypothalamus, area postrema, and midbrain and periventricular regions.


Pediatric NMDAR syndrome: Clinical diagnostic clues and atypical features - Experiences from a tertiary care pediatric neurology centre

K. Devadathan, M. Sreedharan, P. A. M. Kunju, M. Iype, B. P. Vidyalekshmi

Department of Pediatric Neurology, Government Medical College, Trivandrum, Kerala, India

Introduction: After identification of antibodies against the NR1 subunit of the NMDA receptor in 2007, anti-NMDA receptor encephalitis is increasingly diagnosed in children. Here we discuss the clinical characteristics of a cohort of 16 children with antibody positive NMDAR encephalitis. Methods: Single-centre observational study of consecutive children admitted to our centre with antibody positive NMDAR Antibody encephalitis between January 2014 and June 2016. Results: There were 16 children diagnosed with anti NMDAR antibody encephalitis during this period. Age group ranged from 11/2 yrs-14 yrs (median 5 yr). 14/16 was females. All the children presented to us in the months of January to June and there were no cases between July to December. The common presenting symptoms were fever (12/16), seizures (14/16) movement disorders and behavioural manifestations (16/16). The behavioural manifestations ranged from extreme irritability, insomnia or altered sleep rhythm, continuous rocking movements in bed etc. Perioral dyskinesias were present in 14/16 children. Speech abnormalities (16/16) were often the presenting symptom in the form of decreased word output, echolalia to complete mutism. Unresponsiveness to pain with retained response to call and tactile stimuli was seen in 5/16 children. Atypical clinical findings included hemiparesis in 3, and hemichorea in one child. The mean hospital stay was 60 days. 3 children needed ventilator support. All the children received immunosuppressant (steroids and IVIG), 8 children received rituximab and 5 cyclophosphamide. The outcome was good in 10, 4 had moderate disability and 2 children were severely disabled. A benign mature ovarian teratoma was detected in one pt, aged 14 years, which was surgically removed. Conclusions: We would like to highlight the seasonal clustering of pediatric NMDA from January to June. The presence of mutism, extreme fluctuating irritability and insomnia were very characteristic in children. Absence of fever, unilateral neurological deficits are not against the diagnosis. A high index of suspicion helps in early diagnosis and timely treatment.


Differential immune responsiveness in CASPR associated Morvan syndrome, experience from four cases

A. Pathak, D. Joshi, R. N. Chaurasia, V. N. Mishra, Ranjeet, S. Kawale

Department of Neurology, IMS, BHU, Varanasi, Uttar Pradesh, India

Morvan syndrome (MS) is relatively a rare disease characterized by constellation of symptoms of peripheral nerve hyperexcitability along with dysautonomia, sleep abnormality and encephalopathy. The association has also been established with thymoma and other malignancies. Most of the cases of MS are associated with VGKC antibodies. Four patients were admitted with CASPR2 positive moron's syndrome. The mean duration of symptoms was 3 weeks and all were screened for any association with the malignancy. One patient was a female and rest three was males. EMG showed prominent neuromyotonia. All four had prominent sleep disturbance while none had any feature of encephalopathy or altered sensorium. PSG could not be done in any of the patient due to unavailability of the facility at our centre. Two of the four had prominent autonomic dysfunction, who responded to methyl prednisolone while other two patients had to undergo plasma exchange for the improvement. Prominent autonomic disturbance probably holds a better predictor for steroid responsiveness. Though a larger study would be needed to draw a final conclusion.


Clinical and functional evaluation of children with ADEM

M. Iype, P. A. M. Kunju, S. Geetha, T. S. Anish, S. Mini

Department of Pediatric Neurology, Government Medical College, Trivandrum, Kerala, India

Background: Acute Disseminated Encephalomyelitis (ADEM) is an immune mediated demyelinating condition predominantly affecting the white matter of the brain and spinal cord. There are few studies from India describing the profile of ADEM. Aim: To study the clinical and functional profile of children with ADEM. Design: Retrospective cohort study Materials and Methods: Patients with ADEM (fulfilling the International consensus definition for Childhood inflammatory demyelinating diseases) have been included and followed up. Clinical features and radiological findings were evaluated. Functional outcome at discharge was scored using modified Rankin Scale (mRS) and the Kurtzke Expanded Disability Status Scale (EDSS). Results: The inclusion criteria were met by 102 patients (mean age 6.16 ± 3.1 years; range of ages 1-13). Males outnumbered females (n=56); 61 had a preceding febrile illness or vaccination with mean 1.25 ± 1.6 weeks latent period to onset. Non-specific febrile illnesses were the most frequent trigger (52.9%). Commonest findings were pyramidal signs (n=93), ataxia (n=48), brain stem signs (n=42), seizures (n=30), bladder symptoms (n=26), abnormal limb movements (n=12), optic neuritis (n=10), mutism (n=8) and ocular flutter or opsoclonus (n=7). MRI scans demonstrated lesions in the deep white matter (80.4%), brain stem (28.4%), spinal cord (25.5%), cerebellum (20.6%), thalamus (19.6%), basal ganglia (17.6%), corpus callosum (13.7%), periventricular white matter (10.8%), and internal capsule (6.9%). Three patients expired. Fifty one (50%) had pleocytosis. Intravenous methyl prednisolone was the initial treatment in 79.4%; 22 (21.6%) were given both Intravenous immunoglobulin and IV methyl prednisolone. Plasma exchange was done in addition in 4. At discharge 29.4% were normal and 52.9% showed more than 50% improvement. Ventilation was required in 17.6% and 8.8% had ventilator associated pneumonia. The median mRSand EDSS scores at discharge were 2 each. Conclusions: ADEM has a wide range of presentations and has a fairly good recovery at discharge; the majority self ambulating at discharge.


Unilateral foot drop: An atypical presentation of a common disease

R. N. Chaurasia, A. Pathak

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

Objective: Peripheral causes of foot drop are well recognized. However, causes stemming from the central nervous system represent rare, important, and under-appreciated differential etiologies. We are reporting a rare case of isolated unilateral spastic foot drop secondary to intracranial tuberculoma as only manifestation. Case History: A 14-year-old girl presented with history of difficulty in walking for last three days after left foot drop. On Examination: On detailed neurological examination, power at the left ankle, dorsiflexion was grade 1/5 while planti-flexion was grade 5/5 on MRC scale. Left ankle reflex was brisk with asymmetry in plantar response. Sensory, cerebellum and peripheral nerve examinations revealed no abnormality. Investigation: Erythrocyte sedimentation rate was raised (54 mm in first hour). MRI brain revealed multiple conglomerate inflammatory granulomas in cerebrum and cerebellum, larger one in right parasagittal region with perifocal oedema. X-ray chest revealed milliary shadowing in bilateral lung fields. Discussion: The commonly observed causes of foot-drop are common peroneal nerve lesion entrapment, injury at the fibular head and lower lumbar disc prolapse with nerve root compression; other causes include peripheral polyneuropathies, systemic diseases such as connective tissue disorders, vasculitis and diabetes mellitus. Among the cerebral causes of foot drop, parasagittal meningioma, neuroschistosomiasis, high-grade glioma. Anecdotal report of granulomatous lesion including tuberculoma presenting as spastic foot drop has been described in literature. This case needs to be considered as a rare presentation of relatively common central nervous system infections. Results: Patient was put on standard dose four drug anti-tubercular drug combinations, steroid and prophylactic anti-epileptic drug. Her dorsiflexion improved up-to grade 4/5 after 3 weeks of treatment. Conclusion: Intracranial cause of isolated unilateral foot drop is very rare. There may be a delay in the diagnosis of the cause of central foot drop or patients with such lesions might be misdiagnosed and subjected to unnecessary interventions.


Rare mimicker of MND

M. Krishnan

Department of Neurology, Government Mohan Kumaramangalam Medical College Hospital, Salem, Tamil Nadu, India

Introduction: Motor Neuron Disease (MND) is a neurodegenerative disorder which affects upper or lower motor neuron or both. The diagnosis of MND is considered a death sentence due to lack of treatment and the mortality associated with it. It is important to consider MND mimics which could be treatable and may not carry a grave prognosis. We report 2 cases of carcinoma of prostate presenting with neurological manifestations mimicking motor neuron disease. Materials and Methods: Case Vignette 1: A 65 year's old male presented with two months history of progressive dysphagia and nasal regurgitation, His gag reflex was sluggish and had tongue wasting and weakness. In addition he also had unilateral lateral rectus palsy. Sensory and cerebellar systems were normal. Deep tendon jerks (DTR) were normal. Plantars were flexor. Careful clinical examination revealed enlarged left supraclavicular lymph node. MRI spine showed multiple sclerotic secondaries. Fine needle aspiration cytology of left cervical lymph node showed metastatic deposits. Prostatic gland biopsy showed features suggestive of grade 2 adenocarcinoma. CSF analysis showed 40 cells/cu mm, elevated protein and low sugar (20 mg %). Diagnosis of leptomeningeal metastasis was made based on the hypercellularity and hypoglycorrhachia of the CSF. Patient was subjected to orchidectomy and planned for hormonal therapy. Case Vignette 2: A 68-year old gentleman with long standing diabetes presented with severe wasting and weakness of both upper limbs of 4-5 months duration. He also had numbness of both lower limbs. His DTR were absent. Nerve conduction studies revealed mild sensory motor neuropathy of lower limbs and severe reduction in CMAP amplitude of upper limb nerves. MRI spine revealed multiple prostatic spine secondaries causing cervical cord compression. Discussion: Secondary causes should be considered and looked for while dealing with a clinical presentation mimicking MND. Extraocular muscle involvement in the first case and the disproportionate upper limb involvement unusual in diabetic neuropathy in the second case were the giveaways. MRI spine should be done to rule out secondary causes. Our cases demonstrate that prostatic carcinomas can present with neurologic manifestation mimicking MND because of secondary deposits in the spine and meninges. A high degree of clinical suspicion is required as these patients lack symptoms pertaining to the primary site of involvement.


Peripartum neuropathy

J. M. Kovoor, J. Jose, A. Gafoor, B. Smita, C. Susha, M. P. P. Rijosh

Department of Neurology, Calicut Medical College, Calicut, Kerala, India

We are presenting 3 cases of postpartum women, in age group 26-34 years who presented to us with painful burning type dysesthesia of both feet that started in the late trimester 8 th or 9 th month which often gets worsened following delivery, two of them were primi, one after second child birth. None of them were having diabetes, nor had family history of diabetes. None of them had any sensory loss or any motor or autonomic or systemic symptoms. All of them belonged to middle class and hence a dietary deficiency could be ruled out. None of them had any signs of motor or large fiber sensory neuropathy. We did vit b12 assay, ogtt, ana profile, hcv, hiv elisa which all turned out to be negative their electrophysiology studies showed normal sensory conduction. One among them underwent nerve biopsy during the postpartum period and revealed demyelinating pattern, with remyelination. The epidermal nerve fiber density in skin biopsy revealed normal study thus ruling out a small fiber neuropathy. We are presenting this case out of its unique correlation with young females of peripartum period without any functional weakness, or electrophysiological correlate, instead self limiting in nature with histopathological findings.


A clinical and electrophysiological study of Guillain Barre syndrome from Northeast India - A prospective analysis

A. Mahanta, M. Das, L. J. Basumatary, M. Goswammi, K. K. Ashok

Department of Neurology, Guwahati Medical College, Guwahati, Assam, India

Patients and Methods: We report a prospective analysis of 92 cases of Guillain Barr'e Syndrome (GBS). The diagnosis of GBS was made as per Asbury AK, 1990 criteria and Brighton Working Group clinical case definition of GBS. All the patients were subjected to detailed clinical evaluation, haematological/biochemical studies as per hospital protocol & Nerve Conduction Study (NCS). Results: The sex ratio in our study was 1.55:1 (Male:female). The age range was 10-69 (median 28). 54% patients presented during the Summer & Rainy season (Mar-Aug) while 24% presented during winter (Nov-Feb). The duration from onset to admission to was 2 to 27 (median 6) days. A preceding event could be identified in 41.3% which was as follows - fever 13.1%, cough and cold 10.8%, diarrhoea 8.7%, trauma and minor surgery 1.08% each. Six patients (6.5%) presented during pregnancy and all of them in third trimester. Time taken to reach the nadir of weakness was 2-28 (median 9) days. 78 patients (84.8 %) presented in Hughes grade 4, 12 (13.1%) in grade 3 & 2 (2.1%) in grade 2. Cranial nerve involvement was as follows - bilateral VII th in 27 (29.3%), bulbar weakness in 21 (22.8%) and jaw weakness in 1 (1.08%). Oedema of the lower limbs with or without pain was seen in 15 (16.3%). RLS was observed in 2 (2.1%). Reflexes in the weak limb were absent in 41 (44.6 %), diminished in 49 (53.3%) & remained intact in 2 (2.17%). Respiratory paralysis requiring ventilation occurred in 14 (15.2%). Electrophysiologically 48 (52.1%) were AIDP, 26 (28.2%) were AMAN, 16 (17.3%) were unclassifiable (equivocal and nonexitable motor nerves) and 2 (2.1%) were AMSAN. Only 12 (13%) could afford intravenous immunoglobulin (IVIG). 11 subjects (11.9%) died despite of getting ICU care, including 3 (3.2%) receiving IVIG.


Validation of clinical and electrophysiological criteria in the diagnosis of Guillain Barre syndrome - A retrospective study

S. K. Bagadi, A. K. Meena, G. S. Naik, R. Borgohain, S. A. Jabeen, R. Mridula

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

Introduction: The yield of clinical diagnostic criteria for GBS and MFS is variable. Supportive laboratory data are not always helpful. It is essential to diagnose GBS early for effective treatment. As there is no gold standard, we require a criteria to diagnose GBS based on simple clinical features. This study aims to compare the sensitivity of four different criteria (clinical and electrophysiological). Retrospective data from the case records of admitted patients meeting the inclusion criteria: progressive, symmetrical flaccid weakness, in>one limb, and/or ataxia, and/or ophthalmoplegia, at admission, during January 2011 to December 2013. Four different criteria NINDS, Dutch, Brighton and GBS classification group were applied and sensitivity evaluated. 147 cases were studied. 43 were excluded due to alternative diagnosis. Four diagnostic criteria were analysed in 104 patients. Mean age was 33.2 ± 17.3. Fever without localisation (21%), gastroenteritis (10%) and respiratory tract infections (3%) were antecedent infections. Quadriparesis in 81 (78.6%) patients, global areflexia 76 (73.8%) and normal reflexes 5 (4.8%) were observed. Albuminocytologic dissociation in CSF was observed in 23/26 (88.5%). Electrophysiological studies were done between 4-12 days. Four (3.9%) patients had normal electrophysiology. Sural sparing was seen in 57 (55.3%). AIDP was the most common electrophysiological subtype using Brighton criteria (47.6%). Sensitivity of different criteria was: NINDS criteria was 85.6%, Dutch and Brighton criteria was 87.5% each, and GBS classification group criteria was 99%. To conclude, diagnostic criteria proposed by the GBS classification group was found to be most sensitive for early diagnosis. The criteria can diagnose almost the entire GBS spectrum as compared to the other criteria. It is also useful in resource-poor countries and also at peripheral health care systems where NCS are not always readily available.


A study on cross sectional area of median nerve in patients with mild to moderate idiopathic carpal tunnel syndrome

R. Dileep, B. Ramacha, T. Iype

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

Objective: The study was conducted to detect cross sectional area (CSA) of median nerve at the level of proximal carpal tunnel, ratio of cross sectional area at level of proximal carpal tunnel to area 5 cm proximal to carpal tunnel using ultrasound and to find correlation between electrophysiological and sonographic parameters. Materials and Methods: 30 patients who presented with paraesthesia of hands, who were electrophysiologically confirmed cases of mild to moderate idiopathic cases of carpal tunnel syndrome between ages of 18 and 70 were included in the study. An initial assessment was made with Boston carpal tunnel questionnaire (Symptom severity scale (11 items) and Functional status scale (8 items). Ultrasound of the median nerve at the wrist was done with the high frequency probe (7-14 Hz) with the patient sitting comfortably, forearm and hand resting on a table with supinated forearm and extended fingers. First the probe was kept at volar aspect of wrist over the distal palmar crease (oriented perpendicular to the distal palmar crease). Using tracing technique, cross sectional area (CSA) of median nerve was measured at the level of proximal carpal tunnel. The probe was moved proximally to trace the median nerve. Cross sectional area at of median nerve at a point 5 cm proximal to the initial one was taken and ratio between two was calculated. Statistical analysis was done with SPSS. Results: Out of 30 patients with carpal tunnel syndrome, 90.90% were females and 9.09% were males. Paraesthesia on holding (90.91%), on elevation (63.64%), numbness (100%), pain that worsens at night (81.80%), nocturnal awakening (82%), weakness (72.72%) and sensory losses (18.19%) were the main clinical features. Majority of the cases were bilateral (55%) and dominant hand was more affected. Mean Nerve conduction parameters: SNAP amplitude (32.82 ± 2.97) microvolt, DML (4.98 ± 0.95) milliseconds, CMAP amplitude (10.87±1.09) millivolts, SPL (4.37±1.07) milliseconds were found in our patients. Mean Cross sectional area of median nerve at proximal carpal tunnel was 14 ± 4.79 mm 2 and ratio of it to the area of nerve at a point 5 cm proximal was 1.83 ± 0.64. There was correlation between sonographic parameters and electrophysiological parameters. Discussion: All the 30 patients had abnormal cross sectional area (CSA) of median nerve at the level of proximal carpal tunnel and abnormal ratio of cross sectional area at level of proximal carpal tunnel to area 5 cm proximal to carpal tunnel. The sonographic parameters were correlating with the electrophysiological data. Conclusion: Ultrasonography can be used as a diagnostic aid in patients with mild to moderate carpal tunnel syndrome.


Electrophysiological changes in hypertensive chronic kidney disease patients with uremic neuropathy

M. P. Moorthy, A. V. Srinivasan, K. Bhanu, Sivakumar, Ezhilarasi

Department of Neurology, GMKMCH, Salem, Tamil Nadu, India

Introduction: Uremic neuropathy is most common problem in chronic kidney disease patients. Electrophysiological evaluation helps us in the prognosis of neuropathy in pre dialysis, post dialysis and post transplant state. Aim: To study about the electrophysiological changes in hypertensive chronic kidney disease patients with neuropathy. Methods: This study is a prospective analytical study carried out in electrophysiological unit of Govt Mohan Kumaramangalam Medical College and Hospital, Salem, Tamilnadu from August 2015 to May 2016. 46 patients aged between 30 to 60 years were chosen for this study (35 males and 11 females). Inclusion Criteria: Hypertension CKD patients with uremic neuropathy in pre, post dialysis state, post transplant states. Exclusion Criteria: uremic neuropathy due to Diabetes mellitus and other causes of CKD are excluded. Results: This Study is done in 46 patients with uremic neuropathy. Nerve Conduct Study (NCS) done in pre dialysis, immediate post dialysis, post transplant state. In pre dialysis state mean-latency (4.8 m/s), amplitude (3 mv), velocity (38 m/s) in right median nerve. In Post dialysis state mean-latency (3.3 m/s), amplitude (4.5 mv), velocity (49.8 m/s). Post dialysis state showed statistically significant reduction in latency (p<0.001), increase in amplitude (p<0.001) and increase in mean velocity for a transient period. Post transplant patient showed normal latency (<3.1), amplitude (>5.2), velocity (>54). There is no significant change in F-wave pattern. Conclusion: Electrophysiological studies in hypertension CKD patients' revealed, uremic neuropathy in pre dialysis state, transient improvement in post dialysis state, and normal electrophysiological studies in post transplant state. This study shows uremic neuropathy itself is an indication of need of renal replacement therapy.


Tenofovir induced Fanconi syndrome presenting as hypokalemic paralysis

S. Sheetal, M. Madhusudanan, R. Thomas, P. Byju

Department of Neurology, Pushpagiri Institute of Medical Sciences, Thiruvalla, Kerala, India

Hypokalemic paralysis is characterized by acute flaccid paralysis associated with hypokalemia. Tenofovir induced Fanconi syndrome (FS) presenting as hypokalemic paralysis is an extremely rare complication in patients on anti-retroviral therapy. We report a 37 year-old male with acquired immunodeficiency syndrome, diagnosed 11 months back, on tenofovir-based anti-retroviral therapy who presented with acute onset quadriparesis. On evaluation, he was found to have hypokalemia with hypophosphatemia, glucosuria and proteinuria suggesting Fanconi syndrome. He regained normal power in limbs over the next 3 days following correction of hypokalemia and withdrawal of Tenofovir. So far only 2 case reports of Tenofovir induced Fanconi syndrome (FS) presenting as hypokalemic paralysis have been published.


Foot drop-common presentation with rare cause

S. Subramanian, V. Kannan, V. Ramakrishnan, V. Chandramouleeswaran, V. Gobinathan

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

Introduction: Hereditary Inclusion body myopathies are of two major types - Type 1 and Type 2 which are Autosomal dominant and Autosomal recessive forms respectively. Inclusion body myopathy type 2 is also known as Distal Myopathy with rimmed vacuoles which mainly affects the leg muscles and spares the quadriceps. We report a case of Hereditary Inclusion body myopathy type 2 presenting as foot drop. Case Report: 33 year old male patient presented with chronic pure motor weakness which started with Bilateral foot drop and progressed over a period of 8 years. Initially he had difficulty in bearing weight over his toes associated with tripping of toes and alteration in his gait resembling a feminine gait pattern. Over the next 4 years he developed bilateral weakness of both proximal and distal muscles of the lower limbs. He was diagnosed with Peripheral neuropathy, extensively evaluated and differentials Mononeuritis multiplex with cryoglobulinemia/Chronic inflammatory Demyelinating Neuropathy were considered and steroids commenced. But patient's weakness deteriorated with steroids and over next 3 years his weakness was slowly progressive with the development of wasting of upper limb muscles. At this stage he was re evaluated and primary myopathy was suspected and he was worked up for the same. His Magnetic Resonance Imaging of lower limb muscles revealed severe atrophy of all muscles except both the Quadriceps which were spared. Muscle biopsy was suggestive of Distal myopathy with rimmed vacuoles. Genetic analysis revealed putative homozygote for C.971A>G variant of unknown clinical significance. With these results he was diagnosed to have Inclusion body myopathy type 2. Conclusion: This case has been highlighted because of rare presentation of muscle disorder with foot drop because of which we were late in making the diagnosis and Inclusion body myopathy itself being a rare entity.


Demographic and clinical characteristics of motor neuron disease in North West India: A retrospective case series of 82 patients

K. Khandelwal, R. S. Jain, S. Prakash, T. Desai

Department of Neurology, SMS Medical College and Hospital, Jaipur, Rajasthan, India

Objective: To study demographic and clinical characteristics of Motor Neuron Disease (MND) from North West India. Methods: All patients of MND who attended neurology department were scrutinized and included retrospectively through discharge summaries during the period July 2012 to June 2016. Results: Total 82 patients were studied; of which males were 67 (81.7%) and females were 15 (18.3%). ALS was most common type with 71.9% (59 cases) followed by MMA (Monomelic amyotrophy) with 14.6% (12 cases), PMA with 8.5% (7 cases) and SMA with 4.9% (4 cases). Mean age at onset of symptoms in SMA was 5.9±6.7, MMA was 23.37±10.73, ALS was 49.74±13.68 and PMA was 54.3±11.1 years. In MMA, 41.6% patients had upper limb and 58.3% had lower limb involvement. In UL involvement, 80% cases had affection of dominant limb. Fasciculations were present in 25% cases. In SMA, 1 case each of infantile (positive family history) & intermediate variety and 2 cases of juvenile variety were there. In PMA, weakness started in UL in 70% cases with predominant distal involvement, with tongue fasciculations & bulbar involvement in 4 cases. In ALS, 42 (71.18%) patients had spinal onset and 17 (28.81%) had bulbar onset. M:F ratio in spinal onset was 5:1 while in bulbar onset was 1.4:1. Weakness and atrophy was more distally in all group. Fasciculations and tongue atrophy were present in 69.5% and 40.7% cases respectively. Two uncommon cases of ALS - Parkinsonism and juvenile onset ALS was also found in our study. Conclusion: In our study, MND was more prevalent in males. ALS was the most common subtype followed by MMA similar to other parts of country. Mean age of onset was earlier with more male preponderance similar to other studies from our country. Females more commonly had bulbar onset ALS than spinal onset.


Unusual cases presenting as acute flaccid paralysis

A. A. Bhatti, N. Jain, A. Bhutada, M. Shah, S. Ravat, S. Ravat

Department of Neurology, King Edward Memorial Hospital and Seth G. S. Medical College, Mumbai, Maharashtra, India

Patient presenting with acute flaccid paralysis is a neurological emergency. Acute flaccid paralysis is a relatively common mode of presentation but sometimes it may present with rather uncommon features making a definitive diagnosis difficult and thus dissuade a treating physician from treating with the standard treatment. Because the earlier the treatment the better is the outcome. Hence we would like to present a series of four cases all with acute flaccid paralysis as a core feature but with additional atypical features. These features included vision loss, myoclonus, myokimia and urinary retention. Majority (3 out of 4) had good prognosis. The exact incidence of such odd presentation is unknown as most of the literature includes case reports. This series highlights the importance of thinking on a broader perspective and rational treatment so that even patients with atypical features are treated appropriately. These unusual features, the necessary investigation and outcomes will be discussed in this presentation.


Guillain-Barre syndrome following coronary artery bypass grafting: A case report

S. Rajendran, S. Gobinathan

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

Guillain-Barre syndrome is a heterogenous disease characterized by rapidly progressive, symmetrical limb weakness with hyporeflexia or areflexia, sensory disturbance and cranial nerve deficits. Molecular mimicry, antiganglioside antibodies and complement activation are involved in the pathogenesis of GBS. Surgery, as a triggering factor is one of the rare presentations. Altered host defence mechanism, with the release of proinflammatory cytokines due to the pathophysiological changes during and following surgery are implicated in causation. Surgery leads to a transient immunosuppresion by activation of the neuroendocrine stress axis and increased production of adrenocorticotrophic hormone. We report here, a 41 years old female, known Diabetic and hypertensive, 1 month after coronary artery bypass grafting procedure, presented with subacute onset of weakness of all the four limbs, of ascending and flaccid type, suggestive of Guillain-Barre syndrome, as per clinical and electrophysiological evaluation. Patient improved with a course of plasmapheresis.


A-waves in Guillain Barre syndrome

S. Lakshminarasimhan, K. Krishnamoorthy, J. Harish, V. Ramakrishnan, V. Chandramouleeswaran, S. Gobinathan

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

Background: A wave is a late response occurring between the motor response and F response. They are associated with demyelinating neuropathies and reinnervation following axonal loss. Objective: To study the presence of A waves in Guillain Barre syndrome and their significance. Methods: Clinical and electrophysiological features of patients admitted with Guillain Barre Syndrome between December 2015 to May 2016 were analysed. Out of 62 patients, 49 patients (79%) had AIDP (acute inflammatory demyelinating polyneuropathy) subtype, 10 patients (16%) had AMAN (acute motor axonal neuropathy) subtype and 3 (5%) had AMSAN (acute motor- sensory axonal neuropathy) subtype. Results: Out of 49 patients with AIDP subtype, 15 patients (30.6%) had A waves noted in electrophysiological study. 8 were males (53.3%) and 7 were females (46.7%). None of the patients with AMAN and AMSAN subtypes had waves. The mean age of patients in whom waves were noted was 37.6±9.3 years. 2 out of 15 (13.3%) had antecedent respiratory illness and 4 out of 15 (26.7%) had preceding gastrointestinal illness. Nerve conduction study was done between 6 to 11 days of onset of illness in the study group (mean: 8±1 days). Persistence of A waves ranged between 60 and 100%. They were most commonly noted in median nerve followed by tibial nerve. 3 patients had only conduction block abnormality in addition to the presence of A waves whereas rest of the electrophysiological parameters were normal which was statistically significant (P=0.04). Conclusion: Waves are associated with demyelinating form of Guillain Barre syndrome. Presence of A waves with appropriate clinical suspicion in a patient with no preexisting peripheral nerve disease can be taken as a corroborative marker of Guillain Barre syndrome.


To study the effect of magnesium supplementation on brainstem auditory evoked response in diabetic patients with neuropathy

P. K. Maheshwari, A. K. Singh, M. K. Bansal, A. Pandey, K. P. Mishra, R. Jose

Department of Neurology and Medicine, SN Medical College, Agra, Uttar Pradesh, India

Aims: (1) To study the effect of magnesium supplementation on glycaemic control and Brainstem auditory Evoked Potential in diabetics (NIDDM) with neuropathy. (2) To assess the value of auditory evoked potential alterations in predicting the stability and severity of auditory pathway disease related to diabetes. Methods: Of the 120 diabetic neuropathy patients group I (n=60) consisted of those receiving metformin, gabapentin and magnesium supplementation (300 mg/day). Group II (n=60) consisted of those receiving metformin and gabapentin only. Serum magnesium level, fasting blood glucose and latency of brainstem evoked potential were investigated before and after treatment at 4, 8 and 16 weeks. Results: In group I -58.33% of patients belong to the 51-60 age group. 23.33% belongs to 41-50 age group and 18.33% belongs to 61-70 age group of patients. In group II - 51.60% belongs to 51-60 age group.31.6% belongs to 41-50 age group and 16.6% belongs to 61-70 age group. No case was found in > 70 years of age. Mean duration of diabetes in group I was 20.83±0.83 as compared to 22.83±0.83 in group II. In present study mean serum magnesium level in group I before treatment (i.e: first visit) was 1.51±0.0174 mg/dl as compared to 1.77±0.0184 mg/dl & values of fasting blood glucose before & after treatment were 144.96±0.095 mg/dl & 130.24±0.79. The difference was considered significant. Improvement in wave latency post magnesium supplementation was significant. Conclusion: Serum magnesium levels were lower in diabetics with polyneuropathy in both groups and supplementation produced significant improvement. BAER latency and blood glucose levels showed significant improvement on magnesium supplementation.


Study on clinical significance of acetyl choline receptor antibody in ocular myasthenia gravis

R. R. Vaddepally, A. K. Meena, Rupam, S. A. Jabeen, R. Mridula

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

Aims and Objectives: To study the clinical significance of Acetylcholine Receptor Antibody (AchR ab) in Ocular Myasthenia gravis(OMG) and to examine their utility in progression from OMG to Generalized Myasthenia Gravis (GMG). Materials and Methods: A retrospective cohort study is done in 99 patients diagnosed with OMG; data was collected about the age, sex, symptoms at presentation, AchR ab levels. Progression to generalized myasthenia (if they occurred) gravis were recorded. Multiple logistic regressions were used to measure the association between all clinical variables and antibody result. Results: Data collected from 99 participants, there were 64 males and 35 females. Mean age at presentation was 49.64 ± 20.27 years. Clinical presentation was Ptosis (34/99), Diplopia (22/99), both (43/99). AchR ab was positive in 67 patients. In an adjusted model, significant positive antibody test result were associated with elderly age at diagnosis (P = 0.001). Females were less likely to have a positive antibody test (0.007). Patients who developed symptoms of GMG had a significantly higher mean antibody level than those who did not develop symptoms but didn't reach statistical significance Conclusions: Sensitivity of AChR antibody testing is higher than previously thought in patients with OMG. Older age, male sex, were significantly associated with a positive antibody test result, and patients who progressed to GMG likely to have higher levels of antibody levels than who have not progressed.


Significance of Spielman's 3p model of insomnia in current clinical practice: A prospective study

S. Ray, G. Shukla, K. Prasad, A. K. Srivastava, D. Vibha, A. Gupta

Department of Neurology, AIIMS, New Delhi, India

Background: While newer models of insomnia attempt to explain the pathology in terms of genetic markers and predispositions, heuristic models taking a wholesome view on the same have slowly become obsolete. The study sought to explain the relevance of Spielman's 3P model of insomnia in contemporary sleep practice using a direct interview approach to patients and asymptomatic individuals. Methods: Consecutive patients were assessed by the investigator for the presence of predisposing, precipitating and perpetuating factors responsible for their problems. The results were then compared against the final diagnosis made by a sleep expert using standard tools of insomnia diagnosis. Blinding was observed at both stages of evaluation. Analysis: Of the 252 subjects enrolled, 117 (46.4%) were normal, 11 (4.4%) had primary, 56 (22.2%) had secondary, and 10 (4.0%) had both primary and secondary insomnia whereas 58 (23.0%) had other disorders. Patients with sleep disorders were likely to be older and males. The prevalence of predisposing, precipitating and perpetuating factors in these groups were as follows: 32 (27%), 16 (14%), and 23 (20%) in normal, 10 (91%), 5 (45%), and 5 (45%) in primary, 45 (80%), 28 (50%), 30 (54%) in secondary, 8 (80%), 4 (40%) and 4 (40%) in primary and secondary and 33 (57%), 34 (59%) and 34 (59%) in other sleep disorders. Results: Patients without sleep disorders had very few stressors. Predisposing conditions were high in primary and secondary insomnia but not in other sleep disorders. Precipitating conditions were more important for primary and mixed insomnia. The difference was significant between normal versus primary (p<0.001), normal versus secondary (p<0.001) and primary versus secondary (p<0.001). Conclusions: High prevalence of predisposing, precipitating and perpetuating factors in insomnia can help differentiate insomnia types and identify insomnia in mixed sleep disorders.


A study of optical coherence tomography in 14 patients with optic neuritis

M. V. Chandarana, S. D. Shah, S. V. Shah, M. A. Patel, K. Mirche

Department of Neurology, V.S. Hospital, Ahmedabad, Gujarat, India

Background: Optical coherence tomography (OCT) is a non-invasive imaging technique routinely used to visualize and quantify the layers of the retina. Quantification of the RNFL thickness by OCT provides an indirect measure of axonal and neuronal loss in the anterior visual pathways. Objective: To determine degree of changes in retinal nerve fiber layer (RNFL) thickness after optic neuritis (ON) & correlate OCT findings with visual acuity, Fundus, Visual evoked potential (VEP) & perimetry. Materials and Methods: We studied patients with optic neuritis, who attended neurology clinic of tertiary care hospital from July 2015 to May 2016. Both typical and atypical optic neuritis patients were included. Detailed clinical history was taken followed by fundii examination, OCT, VEP & Perimetry along with MRI Brain contrast with optic nerve cut. All Patients were followed up for the period of 6 months. Results: Out of 27 patients we screened, 14 patients were confirmed to have optic neuritis out of which 8 were females & age range (mean) was 45.2 years. There were 2 patients of typical ON. Out of 12 atypical ON patients, 3 were Aquaporin 4 antibody positive. RNFL thinning was seen in majority of patients (11/14) & it usually started after 3 months of disease onset. Optic atrophy occured in 8/14 patients. VEP was abnormal in 11/14 patients. Visual acuity was severely impaired in 7 out of 14 patients (Vision <6/60). RNFL thinning correlated well with severe impairment on corrected visual acuity & optic atrophy on fundus examination. Conclusion: This study provides an important differential OCT finding in Indian patients with optic neuritis, which may have clinical impact on treatment & outcome.


Study of clinical profile and treatment response in CIDP patients

K. C. Mirche, S. V. Shah, P. B. Joshi

Department of Neurology, VS General Hospital, Ahmedabad, Gujarat, India

Background and Objectives: Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a chronic, acquired immune mediated disorder of the peripheral nervous system. The typical form is characterised by progressive or relapsing proximal or generalized limb weakness and areflexia. Methods: All patients of CIDP including both typical and atypical variants were included in study. We studied clinical profile, nerve conduction studies and treatment response in typical and atypical CIDP at our tertiary care referral centre from October 2015 to June 2016. Results: We studied a total of 14 patients of CIDP, of which 11 were typical CIDP. Among 3 atypical CIDP, 2 were multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) and one patient had distal acquired demyelinating symmetric (DADS) neuropathy, all patients were above 45 years, with male: female of 4:3. Diabetes mellitus was present in 10 patients. All had albumino-cytological dissociation and electrophysiological evidence of neuropathy. 3 patients had paraproteinemia. Three out of three patients, on high dose pulse dexamathasone therapy improved over 4 months. Out of 5 patients on oral prednisolone 3 patients improved over 3 months, 2 patients on azathioprine and low dose omnacortil improved over 6 months. One patient on mycophenolate mofetil (MMF) improved over 6 months. One patient improved with strict diabetic control. Two patients did not continue the maintainance therapy in them one patient expired and other patient is clinically static. Conclusion: Various clinical presentations and therapeutic strategies available for CIDP make it difficult to study and interpret. This is our attempt to review high dose dexamethasone therapy in Indian CIDP patients.


Study of sleep disorders and polysomnographic evaluation among primary chronic daily headache patients

R. Verma, K. Nagar, R. K. Garg, H. S. Malhotra, P. Sharma, N. Kumar

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

Objective: Studies related to sleep disorders and polysomnography among chronic daily headache patients are rare. We studied this and compared chronic migraine with chronic tension-type headache. Methods: 83 patients were recruited. They were evaluated by semi structured interview, headache and sleep diaries along with Epworth sleepiness scale score and insomnia symptom score. Overnight polysomnography was performed and data compared. Results: Chronic tension-type headache was commoner than chronic migraine, both having female preponderance. Insomnia followed by excessive day time sleepiness was prevalent sleep disorder. Sleep efficiency and stage 3 sleeps was lower in chronic migraine compared to chronic tension-type. ESSS was significantly increased among chronic tension-type patients. No significant correlation was found among polysomnography parameters in patients with or without sleep disorders. Conclusion: Insomnia being commonest sleeps disorder among chronic headache population. Chronic tension-type headache had slightly better slow-wave sleep than chronic migraine and significantly increased day time sleepiness.


Phenotypic and imaging pattern of muscle involvement in GNE myopathy

S. Doniparthi, A. Nalini, P. V. Kumar, K. Polavarapu, S. Vengalil, J. Saini

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

Background: GNE myopathy is an autosomal recessive disease caused by mutations GNE gene. The disorder is characterised by adult onset symmetrical/asymmetrical foot drop progressing to involve proximal muscles. Quadriceps is spared even in advanced stages. There are very few studies on muscle imaging in GNE myopathy. Methods: Genetic confirmation was done by direct sequencing of coding exons and intronic regions. 1.5T AERA MR Scanner used for evaluating 37 muscles on each side. T1W images to look for degree of fibro fatty replacement (Mercuri score) and T2W STIR for myoedema (Borsato et al.). Results: 31 patients were examined from 2007-2015 and mutations (known and novel) in GNE gene were confirmed in all. The mean duration of illness was 7.1 ± 5.9 years. The mean age at presentation 32.9 ± 6.7 years and age at onset: 26.0 ± 5.4 years. The classical pattern of selectivity in hip muscles was; Flexors weaker than extensors-22/31, adductors weaker than abductors-27/31. Knee flexors weaker than extensors was recorded in 28/31 cases. The mean creatine kinase levels were 467.5 ± 339.8 IU/L. The median mercuri score of biceps femoris (short head), gluteus minimus, tibialis anterior, extensor hallucis and digitorum longus was four indicating complete fatty replacement even in early or atypical cases. The median scores of adductors, hamstrings, sartorius, medial gastrocnemius, tensor fascia lata was three giving a washed out appearance. The vastus lateralis was the only muscle spared in advanced stages (Median score-0), while the rectus femoris (2b), vastus intermedius and medialis (2a) showed variable signs of fatty replacement. Myoedema showed significant inverse correlation with fatty infiltration scores. Conclusion: The clinical features are similar to those reported elsewhere with different founder mutations. This suggests that it is a more homogenous disease unlike other distal myopathies. The study also supports the use of MRI as important tool for diagnosis.

Poster Session 03; Miscellaneous14:00-16:00 h, Friday, November 11, 2016


Utility of Ishikawa versus Jefferson classification of cavernous sinus lesions - A prospective study of 73 patients in a tertiary care centre in Northern India

S. R. Bhatkar, M. K. Goyal, K. K. Mukherjee, P. Singh, V. Lal

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

Objective: To classify the patients presenting with cavernous sinus syndrome (CSS) using Jefferson's and Ishikawa classifications and to compare the utility of these classification systems. Cavernous sinus syndrome is a common cause of painful ophthalmoplegia. These patients have diverse etiologies, presentations and outcomes and often pose a diagnostic dilemma as they are not easily amenable for biopsy. It has been difficult to classify cavernous sinus syndrome and till date many classification systems have been used, the commonest ones being the Jeffersons and Ishikawa classifications. There is a paucity of Indian data of patients with CSS. Methods: In this prospective observational study we included 73 consecutive patients satisfying the criteria of cavernous sinus syndrome i.e. involving any 2 of the 3 rd , 4 th , 5 th and 6 th cranial nerves or any one of them with radiological cavernous sinus involvement. All of these patients were classified as per the Jeffersons and Ishikawa classification scheme and their utilities were compared. Results: While we could classify only 46.6% patients using Jefferson classification, 95.5% patients could be classified using Ishikawa scheme. CSS was classified as anterior, middle and posterior in 17.8%, 21.9% and 8.2% patients respectively as per the Jefferson classification. As per the Ishikawa classification, 37% patients each showed anterior and posterior CSS, 16.4% showed middle CSS while 4.1% had whole CSS. Etiologically, middle CSS was significantly associated with presence of fungal infections (p = 0.045) as per Jefferson's classifications while anterior CSS was significantly associated with a vascular etiology (p = 0.005) as per Ishikawa's classification. Overall, inflammatory causes were the commonest cause for anterior CSS, while tumors accounted for maximum cases of posterior CSS. Conclusion: Ishikawa scheme provides a convenient and more specific system for classification of CSS compared to Jefferson system. Conflict of interest and disclosures: Nil


A rare cause of reversible neurometabolic encephalopathy: Adult onset nonketotic hyperglycinemia

V. Anbazhagan, M. Jeyaraj, S. Velayutham, Sowmini, G. Revathy, P. S. Kumar

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

Introduction: Non ketotic hyperglycinemia (NKH) is rarely encountered in adult neurology clinics. It is an autosomal recessive disorder presenting in two forms, typical neonatal form and atypical late onset form. Patients with late onset form may present with myriad of clinical manifestations eluding clinical diagnosis and thus delaying treatment for years. We report a rare case of late onset non ketotic hyperglycinemia. Case Report: A 17 year old boy, born of 2 nd degree consanguineous marriage presented with 3 month history of progressive cognitive decline, apathy, unsteadiness while walking. His mother noticed foul smelling urine and peculiar brownish discoloration and thinning of hair. There was no history of any drug intake. On examination patient was emaciated, apathetic with impaired attention and short term memory. He had spasticity of both lower limbs with extensor plantar responses, cerebellar examination showed intentional tremor and dysarthria. His gait was spastic and ataxic with choreoathetoid movements of both upper limbs. Routine investigation including serum ammonia, porphyria, blood pH, urine screen for inborn error of metabolism, ceruloplasmin were within normal limits. His blood tandem mass spectrometry showed elevated plasma glycine level- 736.13 umol (normal <505 umol), suggesting NKH. He was started on protein restricted diet and dextromethorphan. Patient showed marked improved in mental status and his chorea disappeared. Plasma glycine was repeated and it came down to normal range (476 umol). A few months later his hair texture, color became normal, his cognition and gait improved. He was able to resume school and continue his studies. Conclusion: We report this case for its rarity and the reversible nature of this neurometabolic disorder which may be overlooked in busy neurology clinics.


A clinical profile of optic neuropathy

D. Kiran

Department of Neurology, Osmania General Hospital, Hyderabad, Telanagana, India

Introduction: Optic neuropathy is a frequent cause of vision loss encountered in clinical practice. The history often points to the possible etiology of the optic neuropathy. A rapid onset is typical of demyelinating, inflammatory, ischemic and traumatic causes. A gradual course points to compressive, toxic or nutritional and hereditary causes. Aims and Objectives: (1) To study the clinical profile of optic neuropthies. (2) To determine the etiology of optic neuropathies. Inclusion Criteria: Patients with optic neuropathy either unilateral or bilateral are included. Exclusion Criteria: Patients with multiple cranial nerve involvement were excluded. Patients with other ophthalmological causes of visual loss and those with occipital infarcts were excluded. Materials and Methods: This prospective study has been conducted from January 2015 to June 2016 in Department of Neurology at Osmania General Hospital. Clinical history was taken and complete neurological examination performed on all patients. Routine investigations like Chronic Bacterial Prostatitis (CBP), Renal Function Test (RFT), and Cumulative Urinary Excretion (CUE) were done in all patients and relevant specific investigations like CT Brain, MRI Brain and Spine, MRI Orbits, and Visual Evoked Potentials were sent according to the patient's requirements. Clinical data and the data from investigations were analysed. Observation and Results: A total of 50 cases were included. Among them 26 cases were due to demyelination, 8 cases were due to ischaemic etiology, 4 cases due to trauma, 3 cases due to toxic causes, 3 cases due to hereditary causes, 3 cases due to compressive etiology, 2 cases due to familial optic atrophy, 1 case due to infiltrative cause. Conclusions: There is wide range of causes for optic neuropathy. Among them most common causes are demyelinating and ischaemic diseases. Less common causes are hereditary like Leber's hereditary Optic neuropathy and tumors. We can prevent visual disability if demyelinating and ischaemic cases are diagnosed and treated early.


Situs inversus and takayasu arteritis presenting with stroke - Understanding structural and functional discrepancies of brain

M. J. Shah, C. M. Sharma, B. L. Kumawat, D. Khandelwal, V. Sharma, A. Garg

Department of Neurology, Sawai Man Singh Medical College, Jaipur, Rajasthan, India

We report a rare case with hemispheric stroke as a presenting feature of Takayasu disease without preceding constitutional symptoms, along with incidental situs inversus totalis. 40 years old male patient, right handed, presented to us with right hemiparesis and Broca's aphasia on examination had absent peripheral pulse in left upper limb and diminished in right upper limb. MRI brain showed infarct in left fronto-parietal region. Computed Tomography (CT) angiography of aorta and both upper limbs was suggestive of bilateral subclavian artery occlusion, was thus diagnosed as a case of Takayasu arteritis. Patient was coincidentally found to have Situs inversus totalis. The structure and function of brain were assessed in view of effect of situs inversus totalis on brain. Discussion: Takayasu arteritis presenting with neurological symptoms is uncommon and that too with stroke is rare. Moreover, patient had coincidental situs inversus totalis. Reviewing literature on structural asymmetries of the brain, it was found that patient had reversal of frontal and occipital petelia corresponding to reversal of other organs of body. However, in spite of situs inversus, language function was localised to the left hemisphere, suggesting non-reversal on the functional aspect. This case thus aids in probing whether situs inversus causes inversion of merely structural aspects of brain or also influences function of left versus right hemisphere of brain. Conclusion: This unique case reports previously unreported and unknown co-occurrence of two rare conditions presenting with stroke.


An interesting case report of dural carotid - Cavernous fistula

J. Cornelius, Manivannan, Ramu, Muthukumar, J. Ambrose, Malaiarasu

Department of Neurology, Government Rajaji Hospital, Madurai, Tamil Nadu, India

Introduction: A carotid-cavernous sinus fistula (CCF) is an abnormal communication between the cavernous sinus and the carotid arterial system. CCFs can be classified by etiology (traumaticvs & spontaneous), velocity of blood flow (high vs low flow) and anatomy (direct vs. dural; Internal carotid vs external carotid vs both). Some fistulas are characterized by a direct connection between the cavernous segment of the internal carotid artery and the cavernous sinus. These fistulas usually are of the high flow type. Most often caused by a single, traumatic tear in the arterial wall, they are called direct carotid- cavernous sinus fistulas. Other CCFs are dural. Many of these lesions are actually congenital arteriovenous malformations that developspontaneously, often in the setting of atherosclerosis, systemic hypertension, connective tissue disease and during or after childbirth. Dural carotid-cavernous sinus fistulas consist of a communication between the cavernous sinus and one or more meningeal branches of the internal carotid artery, the external carotid artery, or both. These fistulas usually have low rates of arterial blood flow. Case Viginette: 46 Year old male patient, a chronic smoker and alcoholic, a known SHT, admitted with excessive daytime sleepiness for the last 6 months. No other positive history. Examination euvolemic no pallor or jaundice. Chorionic Villus Sampling (CVS) and Respiratory System (RS) examinations were normal. Central Nervous System (CNS) examination showed drowsy, extraocular muscles (EOM) full, flap present, Deep Tendon Reflex DTR +, plantar- bilateral extensor, no meningeal signs. Investigations were done. Complete hemogram, sugar, Liver Function Test (LFT), Renal Function Test (RFT) were normal. Oesophago-Gastro- Duodenoscopy (OGD) revealed grade 2-3 esophageal varices. Echo revealed concentric Left Ventricular Hypertrophy (LVH). Ultrasonogram (USG) abdomen revealed chronic liver disease with portal hypertension. Magnetic Resonance Imaging (MRI) brain done which showed dural arteriovenous (AV) fistula. Patient treated with antibiotics, neurosupportives, liver supportives and other supportive measures. Patient improved clinically. Repeat MRI taken which surprisingly showed normal study. A final diagnosis of Cirrhosis Liver with portal hypertension with reversible dural CCF (spontaneous remission). Summary: A carotid- cavernous sinus fistula (CCF) is an abnormal communication between the cavernous sinus and the carotid arterial system. CCFs can be classified by etiology (traumatic vs spontaneous), velocity of blood flow (high vs. low flow) and anatomy (direct vs. dural; internal carotid vs. external carotid vs. both). We herewith present a case of alcoholic cirrhosis which presented with dural CCF.


Intracranial dural arteriovenous fistula presenting as rapidly progressive dementia parkinsonism with prominent fluctuations

S. S. Badachi, T. Mathew, G. R. K. Sharma, R. Nadig

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

Introduction: Dural arteriovenous fistulas (DAVFs) consist of arteriovenous shunts between meningeal arteries and veins within the dura and account for 10-15% of cerebrovascular malformations. The presenting feature of DAVFs is related to the location and pattern of venous drainage. Clinical manifestations include headache, pulsatile tinnitus, proptosis, dementia, parkinsonism, intracranial haemorrhage, myoclonus. MRI is helpful to diagnose especially susceptibility weighted imaging. Digital subtraction angiography is gold standard modality for diagnosis. If diagnosed early and treated promptly clinical manifestations may be reversible. Here we report a rare case of intracranial DAVF presenting as rapidly progressive parkinsonism dementia with prominent fluctuating course not described in literature so far. A 70 year apparently normal male had history of intermittent double vision lasting 10 seconds, 6 -8 episodes daily, with spontaneous recovery each time since one year. History of slowness of activities, freezing of gait since 8 months. Difficulty in calculation, naming and attention since 6 months. He had a prominent fluctuations with symptoms resolving after assuming recumbent position initially. Symptoms were constantly present 8 months into the illness. On examination he had executive dysfunction. Complex attention and calculation was impaired. He had parkinsonism and gait freezing. MRI T2 Flair showed white matter changes suggestive of venous hypertension. Susceptibility Weighted imaging showed prominent dilated veins. Digital subtraction angiography confirmed diagnosis of DAVF. Endovascular treatment yielded dramatic improvement. Conclusion: DAVF is a great mimicker. It must be considered in differential diagnosis of rapidly progressive dementia syndrome. High index of suspicion is needed to diagnose this rare entity. Susceptibility weighted imaging helps in early diagnosis non invasively. Symptoms may be reversible if treated promptly.


Moyamoya disease - A case presentation

M. Ramarajan, Justin, Muthukumar, Ramu, Malaiarasu, Thirumalairajan

Department of Neurology, Government Rajaji Hospital, Madurai, Tamil Nadu, India

Introduction: Moyamoya disease is a chronic progressive occlusive disease of the circle of Willis which leads to the development of characteristic collateral vessels seen on imaging, especially cerebral angiography. By definition, the pathognomonic arteriographic findings are bilateral in moyamoya disease, although the severity can differ between the sides. Case Vignette: A 30 year old female presented to our hospital by July 2014 with acute onset of loss of consciousness (LOC) with weakness of right upper limb and lower limb. On detailed history evaluation, she presented with similar episode by 2008 for which she got treated outside and she improved. At present, clinical examination revealed unconscious, DEM +, PERRLA, right UMN facial lag+, right hemiplegia+ and plantar extensor right. Results of metabolic parameters and coagulation profiles were normal. Chest x-ray (CXR), Electrocardiogram (ECG), Echocardiogram (ECHO) was normal. Computed Tomography (CT) brain revealed left thalamic bleed with intraventricular bleed, subarachnoid bleed with mild obstructive hydrocephalus. Patient was treated with antiedema measures, antiepileptics, antibiotics and physiotherapy. Patient improved symptomatically and discharged after a period of 2 weeks. Conclusion: Moya moya disease is probably an inherited vascular disease first described in japan and now reported from all over the world. Most common presentations are-Ischemic stroke 50-75%, Transient ischemic attack (including drop attacks) 50-75%and Hemorrhage (in adults) 10-40%. Our patient presented with hemorrhagic stroke. Characteristic radiographic findings confirm the diagnosis, and recognition of the disease early in its course, with prompt institution of therapy, is critical in order to achieve the best outcome in patients. Revascularization surgery appears to be effective in preventing stroke in patients with moyamoya.


Gordon holmes ataxia with atypical retinitis pigmentosa: Old men with new features

V. Banga, S. Kushwaha, A. Gupta, R. Mistry, S. Singh, M. Sachan

Department of Neurology, IHBAS, New Delhi, India

Gordon Holmes described an autosomal recessive disorder presenting with ataxia and hypogonadism. Later on Boucher & Neuhauser described SCA with hypogonadotropic hypogonadism and chorioretinal dystrophy. With passing time various reports were published which reported Gordon Holmes ataxia with variable clinical presentations. Case : We hereby report a case of 34 year old female (XX Karyotype) born to a nonconsanginous marriage with underdeveloped secondary sexual characteristics and atypical retinitis pigmentosa. Our case presented with complaints of difficulty speaking since 1 year of age with mild cognitive impairement. By the age of 20 years, she started developing cerebellar signs in the form of intentional tremors and progressive ataxic gait. At 24 years, she started reporting diminution of vision and distal lower limb weakness. Examination revealed stunted growth, under developed secondary sexual characters (Tanner stage II, pubic hair 2/3, axillary hair 2/3) with MMSE of 27/30 and Frontal dysfunction (FAB score 4). On ophthalmologic examination, there was grade II nystagmus and visual acuity of 6/60 with fundus examination revealing atypical retinitis pigmentosa. Detailed sensory-motor examination revealed impaired joint position, vibration, absent DTRs, extensor plantar. Laboratory investigations revealed decreased LH, FSH with hypoplastic ovaries and uterus. There was pituitary hypoplasia and a positive response to GnRH stimulation. Discussion and Conclusion : Gordon Holmes syndrome may present with a range of clinical manifestations. The differentiating features in our case were presence of neuropathy, corticospinal tract involvement and atypical retinitis pigmentosa. To the best of our knowledge this is the first case report from India describing Gordon Holmes syndrome with neuropathy & atypical retinitis pigmentosa. The current case report reinforces that similar situation could arise in the near future with new variables fanning its clinical spectra with a dilemma that Gordon Holmes is a distinct clinical entity or a spectrum.


Rare association of sugar coating cerebellum and squamous cell lung cancer

K. B. Jadhav, K. Ramadoss, Balakrishnan, Prakash, A. Ninan

Department of Neurology, PSG Hospital, Coimbatore, Tamil Nadu, India

We present the case of a patient presenting with cough, weight loss and poor appetite. Treated outside as case of Pulmonary TB and started on ATT. After one week he was referred to our hospital with new complains of unsteadiness of gait, swallowing difficulty, headache. On examination he was disoriented, with neck stiffness, B/L nystagmus, Power Normal, B/L cerebellar signs. CSF analysis was normal, with no evidence of Tuberculosis. GENE XPERT Negative, BAL was done showed no bacterial growth. MRI Brain showed leptomeningeal enhancement Scattered over cerebellum 'sugar coated' manner. Classically seen in leptomeningeal carcinomatosis due to the spread of malignant cells through the Cerebro spinal fluid either from primary CNS tumors (drop-metastases) or from distant tumors mets i.e. haematogenous spread. USG neck showed supra clavicular lymph nodes. To look for primary carcinoma. Biopsy was done showing Squamous cell carcinoma. So we confirmed the sugar coating of cerebellum leptomeningeal carcinomatosis was due to primary in lung. Methods: We discuss meningeal carcinomatosis in lung cancer, and describe challenges to diagnosis and therapy. Discussion : Leptomeningeal carcinomatosis seen in approximately 5% of patients with lung cancer and incurs a bleak prognosis. Presenting neurologic symptoms can be varied and diagnosis is confirmed via lumbar puncture and cerebrospinal fluid cytology. However our case CSF analysis was normal. Few data exist regarding optimal treatment, although intrathecal chemotherapy has been shown to provide a modest improvement in median survival.


Reversible posterior reversible encephalopathy syndrome in a COPD patient

O. Farooq, I. Hussain, S. Rashid, J. Basoo

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

The posterior reversible encephalopathy syndrome press is an increasingly recognised disorder. Most patients have several symptoms, seizures are most frequent often multiple or status epilepticus a combination of seizures, visual disturbances and/or headache, in particular should lead to an early brain mri to reveal the typical pattern of press in the form of bilateral hyperintensities on flair imaging predominantly in parieto occipital regions. We describe a case of press in a 45 year old male known copd, who developed it following acute exacerbation of copd and recovered completely clinically and radiologically after managing copd exacerbation aggressively. This is a unique case otherwise press is described in renal disease accelerated hypertension cytotoxic therapies, ecclempsia, immunosuppression but it can occur with many diverse clinical entities. The pathophysiology of press is under debate, but it is related to disordered cerebral dysregulation. Since press is often unsuspected by clinicians, recognition of characteristic imaging findings by radiologists is key to diagnosing this syndrome and should prevent deleterious work ups or therapies.


A study of clinical profile outcome and complications of posterior reversible encephalopathy syndrome among pregnant women in a tertiary care hospital

S. K. Parameswaran, M. Jeyaraj, S. Velayudham, Sowmini, R. Sugantha, A. Vignesh

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

Introduction: Posterior Reversible Encephalopathy is a syndrome in which abrupt rise in blood pressure leads to a myriad of clinical manifestations. Objective: To assess the clinical profile, outcome and complications of PRES among pregnant women in a tertiary care hospital. Materials and Methods: A descriptive study conducted among pregnant women with clinical suspicion of PRES, admitted to Obstetrics and Gynaecology department, Stanley Medical College over a period of two years. Inclusion Criteria: patients having clinical and radiological features consistent with PRES. Exclusion Criteria: Sub cortical white matter lesions other than PRES. Results: The commonest etiology of PRES was Pregnancy induced hypertension. Among the 14 patients in the study, 5 had ante partum eclampsia (35.7%), 2 had severe preeclampsia (14.28%) and 7 had post partum eclampsia (50%). 8 patients out of the 14 cases were primigravida (57.1%). Papilloedema was seen in two patients (14.28%) Commonest lobes involved were the occipital (77.41%) and parietal (67.7%). Sub cortical white matter changes were noted in all the patients. 48.4% of the patients had both sub cortical and cortical involvement. In most of the cases the symptoms were reversible Irreversible complication (visual defects) was seen in one patient. Conclusion: A high index of clinical suspicion in appropriate settings along with radiological correlation, will help in early recognition and prompt management of PRES.


Methotrexate induced ataxia and isolated vermian hyperintensity - Case report

R. Y. Kalpana, J. B. Agadi, N. Karthik, N. B. Archana, P. S. Kumar, G. T. Subhash

Department of Neurology, BMC & RI, Bengaluru, Karnataka, India

A 47 years old lady with Rheumatoid Arthritis (RA) since 8 years without extra articular manifestations presented with persistent giddiness and swaying while walking since 1 week. There were no brainstem symptoms. She was on Methotrexate of 15 mg/week. Past history was uneventful. On examination, she had no active signs of RA; neurologically, she had gaze evoked nystagmus (birectional and horizontal) and gait ataxia. There were no appendicular cerebellar signs or tremors. MRI brain revealed T2 and FLAIR hyperintensity in vermis with no restriction on diffusion weighted images (DWI) and no enhancement on contrast. Hematological, biochemical and CSF examinations including the work up for paraneoplastic conditions and vasculitis were normal. Suspecting Methotrexate as culprit, MTHFR genetic study was done which showed heterogenous missense mutation of the gene at exon 8 (Glu to Ala). She received 5 days of IV Methyl Prednisolone 1 gram once daily and had improved minimally. She was asked to discontinue Methotrexate and continue Folinic acid. She was referred to rheumatologist for change of the disease modifying drug (DMARD). At one month follow up she is better with 75% improvement in symptoms. The rarities of the present case are that (1) the patient was on a low dose oral Methotrexate; (2) isolated vermian hyperintensity without diffusion restriction on MRI has not been described in literature till now; (3) there were no apparent risk factors (no age extremes, no renal or liver disease, no cranial radiotherapy recieved previously); (4) she was genetically susceptible to Methotrexate toxicity. Highlighting this case, we would like to emphasize the need for a biological marker to quantitate Methotrexate toxicity and correlate with its clinical presentation.


Abnormal body odour - A clue to diagnose neurometabolic disorder in a child

V. Subramanian, B. Krishnakumar, B. Subathra

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

Aim: Maple syrup urine disease (MSUD) is an autosomal recessive metabolic disorder involving catabolic pathway of the branched chain aminoacids (leucine, isoleucine and valine) leading to build up of their respective ketoacids. MSUD presents with classic 'maple syrup' odour over the body and urine. We report a child who presented with developmental delay and abnormal odour, diagnosed as MSUD by MRI and biochemical findings. Case Report: Eight months old boy, born for consanguineous parents presented with developmental delay and abnormal odour over the body and urine since birth. Antenatal and natal histories were uneventful. Examination showed microcephaly, hypertonia. MRI brain showed white matter hyperintensities in bilateral cerebrum, basal ganglia, midbrain, pons, cerebellum and cerebellar peduncles due to edema. MRS revealed a peak for branched chain aminoacids at 0.9 ppm. Tandem mass spectrometry showed elevated leucine, isoleucine and valine confirming the diagnosis of MSUD. Child was treated with thiamine and parents were advised to give leucine, isoleucine and valine restricted diet. Discussion: affects 1 in 185,000 infants. There are a few other metabolic disorders like phenyketonuria, isovaleric academia with characteristic urine and body odour. Classical MSUD presents in the neonatal period with poor feeding, vomiting and seizures. If untreated, child may have developmental delay, seizures and mental retardation. Conclusion: This case is reported for its rarity. All developmental delay is not cerebral palsy. History of abnormal body or urine odour should be elicited in all cases of developmental delay especially when the perinatal period is uneventful, to make an early diagnosis, so that morbidity and mortality can be reduced.


Kikuchi disease- A case report

M. Paulrajan, Ebenesar, S. Kumar

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

A 53 years old male with diabetes mellitus, coronary artery disease had drowsiness, fever with rigor, haematemisis. He was admitted with GCS 10, distended abdomen PEARL. Fundi-normal, moving all 4 limbs, no melingial signs, low HB, deranged electrolytes, elevated liver enzymes. UGI endoscopy was done which showed Oesophageal candidiasis. HIV, Hbsag negative CT-chest ground glass opacities in lower lobes noncalcified non necrotic bilateral multiple lymph nodes present. MRI showed diffuse cerebral atrophy. EEG-abnormal, bilateral cerebral dysfunction. CSF was done fungal hyphae, negative, gram stain few pus cells and no bacteria, CSF protein 66 mg/dl. CSF PCR for TB negative. CT abdomen multiple enlarged lymph nodes in para aortic, aorto caval, para caval, peripancreatic and parailiac region with fuzzy margin and no necrosis. CT-guided FNAC of lymphnode showing large discrete areas of necrosis with abundant karryorectic debri and presence of apoptotic bodies are surrounded by lymphocytes, histiocytes and plasmacytoid cell. No neutrophils identified. Few follicles with residual germinal center noted no granuloma or atypical changes seen. Features are suggestive of histiocytic necrotizing lymphadenitis (kikuchi disease) started on steroids and antibiotics. Patient improved well.


CNS - Inflammatory myofibroblastic tumor masquerading as CSOM

V. Agarwal, N. Singhal

Dr. Vinay Agarwal Neurology Clinic, Agra, Uttar Pradesh, India

IMT is a rare tumor in the CNS, mostly being extra cranial. Approximately 100 sporadic cases have been reported in the literature (1, 2). The rarity of the tumor, its various histopathological characteristics and its variable aggressive course render it difficult to diagnose and treat. Inflammatory myofibroblastic tumor is generally a histological diagnosis which is rarely suspected preoperatively. It mimics other intracranial tumors such as Giant cell tumor, haemangiopericytoma, anaplastic meningioma, plasmacytoma, lymphoma etc. Rarely, it can present with a clinical picture which mimics a benign infective process, Rosai-Dorfman Disease or an idiopathic hypertrophic pachymeningitis. High index of suspicion is required as total resection of this lesion is mandatory to prevent recurrence. Here we describe a case of 10 year old child which initially presented with clinical features mimicking ASOM and radiological presentation of a small intracranial abscess. He was initially treated by an ENT surgeon who started him on iv antibiotics but the patient was lost to follow up. He returned after 2 months with a large lesion at the same location. Histological examination revealed multiple spindle cells with plasma cells and lymphocytes scattered among these spindle cells. The spindle cells were immune-positive for smooth muscle actin and negative for epithelial membrane antigen, S100 and CD34.


Rare cases of cerebral creatine deficiency syndrome

P. S. Peter, S. Saravanan, Murugan, Radha, Raichal, T. E. Raj

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

Introduction: Cerebral creatine deficiency syndromes are a group of inborn error of creatine metabolism comprising two autosomal recessive disorders that affect the biosynthesis of creatine-i.e, arginine: glycine amidnotransferase deficiency and guanidinoacetate methyl transferase deficiency and an X-linked defect that affects the creatine transporter, SLC6A8 definciency. It should be suspected in a child with intellectual disability, behavioural problems, speech delay and seizures. Case Details: Here we are presenting two children of same family, a sister, 10 years old and a brother, 7 years old. They presented with generalized tonic clonic seizures. On examination, these two children are having expressive speech and language delay, intellectual disability, autistic like behaviour. MRI Brain with MR spectroscopy shows complete depletion of cerebral creatine. EEG shows diffuse hyper synchronization with multiple spikes. Treatment strategies include oral supplementation of high dose creatine-monohydrate. We are presenting this case for its rarity.


Spectrum of malignancies presenting with neurological manifestations

D. S. Batra, Malhotra, R. K. Garg, R. Verma, M. Kumar, M. Goel

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

Introduction: One of the most frequent and debilitating complications of cancer is involvement of the nervous system. Nearly 45% of patients with cancer require evaluation of a neurologic problem. Neurologic complications are the most common reason for emergency admissions of patients with cancers. This study intended to evaluate spectrum of neurological manifestations of malignancies in patients admitted in neurology ward. Methodology: We prospectively evaluated all admitted patients in neurology ward with the suspected malignancy. Their specific neurological symptoms and syndromic neurological diagnoses were recorded. This study was conducted in neurology department of a tertiary care centre from August 2014 to July 2016. Results: A total of 156 patients were included. The most common symptoms were headache (n=31), paraparesis (n=30), back pain (n=29), hemiparesis (n=27), altered sensorium (n=18), seizures (n=14) and, cranial nerve involvement (n=13). Primary malignancies accounted for 62 (39.74%) patients while 94 (60.26%) patients had secondary involvement of nervous system. Among the primary malignancies, brain was the most commonly involved site (67.7 %) followed by the spinal cord (27.4%) and peripheral nerves (4.8 %). Histo-pathological diagnosis with biopsy/bone marrow examination was possible in 48 patients. Out of these, two most common histo-pathological diagnoses were adenocarcinoma lung(20.8%) and multiple myeloma(14.6%). Others less common diagnoses were squamous cell carcinoma, non- small cell lung carcinoma, bronchoalveolar carcinoma, poorly differentiated carcinoma glioblastoma multiformae, craniopharyngioma, meningothelial meningiomaadeno carcinoma prostate, plasmacytoma, thymoma, insular carcinoma thyroid, adenoid cystic carcinoma, leiomyosarcoma, adenocarcinoma endometrium, adenocarcinoma caecum, uroepithelial carcinoma and chromophobe renal cell carcinoma. Conclusion: The most common neurological presentations were headache, paraparesis and back pain. Adenocarcinoma lung was the most common malignancy presenting with neurological manifestations.


Clinical profile of neurological complications of diabetis mellitus

V. L. Gaikwad, R. V. Aravinda, S. K. Das, A. Mandal, A. K. Senapati

Department of Medical College, BMCH, Burdwan, West Bengal, India

Diabetes mellitus is a chronic metabolic disorder now recognised as fast growing global problem with huge social, health and economic consequences. It causes multifold of neurological complications including both central and peripheral nervous system eg: Cerebrovascular stroke, seizures, neuropathy and so on. Diabetes not only affect patients with severely deranged glycemic profile but also with mild glycemic derangment. So it is necessary to recognise diabetes and its complications early, in order to decrease mortality and morbidity associated with the disease. The study was conducted on rural population of Burdwan with sample size of 100 diabetic patients visiting in Burdwan medical hospital and Anamoy superspeciality wing Burdwan, and the burden of neurological complications found were as stroke - Ischaemic 49%, Hemorrhagic 16%, Seizures 5%, Cranial neuropathy 7%, Autonomic neuropathy 1%, Radiculopathy 5%, Plexopathy 3%, Peripheral neuropathy 12%, Chronic inflammatory demylinating polyneuropathy 2%.


Postpartum hypernatremic encephalopathy with osmotic myelinolysis and rhabdomyolysis: A case report

Y. Paidimarri, B. Vengamma, P. Nataraja, T. Naveen

Department of Neurology, SVIMS Hospital, Tirupati, Andhra Pradesh, India

Introduction: The clinical syndrome involving hypernatremia producing simultaneous rhabdomyolysis and cerebral imaging abnormalities in postpartum women was a rare entity. We report a case of young postpartum female who presented with hypernatremic metabolic encephalopathy. Case Report: A 22 year old female in 15 days postpartum status presented to our hospital with altered sensorium since 2 days. On examination patient was drowsy with quadriparesis. Laboratory investigations revealed hypernatremia (Na-180) and raised CPK levels (5600) with acute renal failure (Blood Urea- 132, Sr. Creatinine-2.1) suggestive of rhabdomyolysis and MRI Brain showed showed bilateral symmetrical areas of restricted diffusion involving cerebellum, cerebral white matter, hippocampus, parahippocampus, thalami, internal capsule and brainstem suggestive of osmotic myelinolysis secondary to hypernatremic encephalopathy. After correction of hypernatremia with oral free water supplementation and 5% dextrose over a period of two weeks there was gradual improvement in the sensorium of the patient. At the time of discharge patient was conscious, oriented and ambulant. Conclusion: Postpartum hypernatremia is a potentially reversible metabolic dysfunction which can produce encephalopathy, osmotic demyelination and rhabdomyolysis. Early identification and management of hypernatremia can improve the morbidity and mortality.


Clinical spectrum of neurological manifestations of systemic cancer

K. K. Kommu, S. R. Lakshmi, Ramesh, Archana, K. Theja

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

Introduction: Direct neoplastic (metastatic) manifestations; Brain (usually parenchymal tumours), Spinal cord (usually compression), Leptomeningeal Peripheral and cranial nerves. Indirect (non-metastatic) manifestations; Metabolic Infections, Vascular disorders, Complications of cancer therapy, Paraneoplastic neurological syndromes. Objective of the Study: (1) To study about clinical spectrum of neurological manifestations of systemic cancer (2) To know about their their correlation with specific cancer. Inclusion Criteria: Cancer Patients presented with Neurological manifestations. Exclusion criteria: Patients with Neurological manifeatations explained with other aetiology are excluded. Materials and Methods: Source of data - Patients presenting with Neurological manifeatations to Osmania general hospital and Reffered from MNJ regional cancer institute-Hyderabad. Method of collection of data: A prospective study will be carried out in OGH Hyderabad (December 2014- August 2016). Approximately 50 patients will be included in the study and consent will be taken from them. Following information to be noted in semi structured proforma like presenting age, sex, symptoms relevant history, clinical examination findings. Basic and relevant investigations are done. The data was tabulated and analysed. Observation and Results: Most common neurological manifestation is brain parenchymal metastasis followed by spinalcord metastasis. Most common tumor is Lung carcinoma followed by breast. In two cases cause found to be paraneoplastic. Conclusions: Nervous System (NS) manifestations of cancer are common; their frequency is increasing and they are serious. The differential diagnosis between direct and indirect NS manifestations is often difficult since the different disorders may present with similar clinical signs, requiring meticulous evaluation to reach a definitive diagnosis. When a correct diagnosis can be made early in the course of the disease appropriate treatment frequently relieves symptoms and prolongs life.


An interesting case of treatable ataxia

N. Vasudevan, S. Gobinathan

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

Introduction: Cerebrotendinous xanthomatosis (CTX) is a autosomal recessive lipid storage disease characterized by infantile-onset diarrhea, childhood-onset cataract, adolescent-onset tendon xanthomas, and adult-onset progressive neurologic dysfunction (dementia, psychiatric disturbances, pyramidal and cerebellar signs, dystonia, peripheral neuropathy, and seizures) due to cholestanol accumulation in the CNS, heart, blood vessels, eye, and tendons. Case Vignette: 33 years old male patient on treatment for coronary heart disease (CAD) for 9 years was admitted with unsteadiness while walking for 8 years and defective vision for 3 years. Examination revealed bilateral cerebellar signs, xanthomas over both Achilles tendon, bilateral immature cataract. MRI showed T2 Hyperintensity in dentate nucleus and cerebellar white matter. Serum cholestanol levels were elevated (440 μg/dL). Biopsy of Achilles tendon xanthoma is consistent with cerebrotendinous xanthomatosis. He was started on chenodeoxycholicacid (CDCA) 750 mg/day. Patient's cerebellar signs improved over a period of six months. Conclusion: Patients presenting with chronic cerebellar symptoms with cataract should also be evaluated for treatable causes of ataxia like CTX, although it is rare in India. This case is presented for its rarity and for the excellent prognosis with CDCA.


Rare disease with late onset respiratory failure

A. Shah, A. K. Roy, E. Ratnavalli, S. Murali, U. A. Murgod, P. Krishnan

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

Objective: We report a rare case of late onset respiratory failure as a presenting feature of a rare disease. Case Vignette: A 66 years old gentleman presented with acute onset altered sensorium without any fever, headache or vomiting. In ER, blood gases revealed hypercarbia and he had seizures necessitating mechanical ventilation, antiepileptics and supportive therapy. Within 24 hours, sensorium improved with no focal deficits except inconsistent left sided ptosis. MRI brain, CSF study and EEG were unremarkable. Attempted extubation was difficult. Tests for autoimmune encephalitis, vasculitis and anti-thyroid antibodies were negative. HIV serology, ACE, RNS, AChR and anti-MUSK antibodies were negative. CPK was mildly elevated. He was readmitted with similar complaints within a week of discharge. On requestioning, wife recalled 2 years h/o RBD, 2-3 episodes of postural hypotension, episodic non-exertional shallow breathing with normal cardiac/pulmonary work-up, depression and weight loss of 8 kgs in the preceding year. Family history revealed that mother had died of sudden onset respiratory distress and altered sensorium. Elder brother, treated as Myasthenia, died following an episode of acute onset respiratory insufficiency with altered sensorium. Another brother had progressive, symmetrical (distal > proximal) weakness of all limbs without respiratory complaints and had been treated as inflammatory myopathy. In the hospital, patient developed bilateral complete ptosis, markedly restricted EOMs and significant rigidity which improved with levodopa. Muscle biopsy showed type-2 fibre atrophy. Acid-alpha-glucosidase activity assay in dried blood spot was unremarkable. Whole body PET CT, anti-paraneoplastic antibodies, Tandem Mass Spectrometry, tests for titinopathy and GAA mutations were negative. Genetic tests revealed dynactin gene (DCTN1) mutation which is the genetic cause of Perry syndrome. Discussion: Perry syndrome is characterized by progressive central hypoventilation, AD parkinsonism, depression and weight loss. There is severe neuronal loss in Substantia Nigra and selective loss of putative respiratory neurons in ventrolateral medulla and dorsal raphe nucleus.


Varied spectrum of bilateral clawing in a tertiary care hospital

P. Chellamuthu, J. Manickavasagam, V. Kannan, S. N. Sundaram, S. Gobinathan

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

Background: Unilateral claw hand is a commonly seen condition but bilateral clawing with small muscle wasting of hands as initial presentation is rarely seen. This study gives the spectrum of neurological diseases presenting with bilateral claw hand and wasting of small muscles as initial symptom with varying etiologies. Methods: Patients presenting with bilateral claw hand and wasting of small muscles admitted in neurology ward between January 2016 to June 2016 were included in the study. A total of 5 patients were included in the study. Case Vignette: Case 1 - A 30 year male presented with acute onset of severe pin pricking pain both hands with bilateral clawing. Clinical examination revealed nerve thickening with hypo pigmented patch hidden in his beard. On investigation split skin smear was positive for lepra bacilli. Case 2 - A 32 year female presented with chronic occipital headache with bilateral clawing and wasting of small muscles. Clinical examination revealed cerebellar signs and mirror movements. MRI Cervical spine revealed foramen magnum tumour with cervical extension. Case 3 - A 54 year male presented with bilateral clawing and wasting of small muscles with fasciculations without sensory disturbance. Clinical examination revealed presence of upper motor and lower motor signs suggesting amyotrophic lateral sclerosis. Case 4 - 18 year male presented with bilateral clawing and wasting of small muscles which is static for 4 years. Clinical examination revealed lower motor neuron signs without sensory symptoms. MRI cervical spine suggestive of Hirayama diseases. Case 5 - A 50 year male presented with bilateral clawing and wasting of small muscles with neck pain and sensory disturbances. MRI cervical spine revealed severe compression of roots at C7, C8 and T1 levels. Conclusion: This study shows various treatable and non treatable etiologyfor patients presenting with bilateral clawing.


Neuroimaging in hypoglycemia

J. Ahamed

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

Introduction: Hypoglycemia causes serious cerebral damage and results in irreversible neuronal loss. The cortex, hippocampus and basal ganglia are particularly vulnerable to hypoglycemia. Imaging abnormalities in patients with hypoglycaemia are uncommon and also very variable. About a fifth mimic acute ischaemic stroke. Very few reports are available on changes in the human brain after severe hypoglycemic injury. We present a series of cases of patients with neurological deficits following hypoglycaemia and their neuro imaging abnormalities. Objective: The aim of this study was to investigate the neuroradiological changes in patients after hypoglycemic episodes. Materials and Methods: The study was done among 10 subjects admitted in Sri Ramachandra medical college during a period of 6 months with documented blood sugar levels of less than 70 mg/dl. The duration of the hypoglycaemic episode were noted. The subjects underwent Neuroimaging with 1.5 T MRI and the abnormalities were observed. Results: Among the 10 subjects with hypoglycaemia, 4 subjects had abnormal imaging and the others were normal. The abnormalities noted were diffusion restriction in cortical and subcortical white matter in all the subjects. The MRI abnormality was noticed only in patients with prolonged hypoglycaemia. Recurrent, short lasting hypoglycaemia was not associated with any MRI findings. Conclusion: Prolonged hypoglycaemia is associated with extensive white matter changes in MRI. MRI findings were found in Parietal, frontal, Temporal, occipital lobes, corpus callosum, midbrain and ventrolateral thalami. Patients with imaging abnormalities had a worse prognosis than MRI normal subjects. The imaging abnormalities were noted in subjects where hypoglycaemia lasted more than 6 hours. Hyperacute short-term severe hypoglycemia does not show visible changes in the MRI.


Atypical presentation of Wilson's disease - Needs for a high index of suspicion

L. L. Muppalla, T. S. Kumar, U. A. Kumari, S. Gopi, G. B. Raju

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

Introduction: Wilson's disease is a potentially treatable autosoma l-recessive disorder which presents as various protean clinical manifestations. The initial presentation can be hepatic or neurological or other system dysfunction alone. No two patients of Wilson's disease may have similar clinical characteristics even among the common sib - ship, leads to delay in diagnosis. Materials and Methods: To analyze initial clinical presentation and rate of misdiagnosis by using FERENCI SCORE in 24 patients with Neuro Wilson's disease who admitted in The Department of Neurology in Andhra Medical College, Visakhapatnam in 4 year period. Results: In our study only 22% have initial Neurological clinical manifestations. Others were with 17% Fertility related-17%, Musculoskeletal complaints-17%, Behavioral abnormalities-17%, Psychosis-13% Jaundice -13%, Ocular-13%, Cognitive-4.8%. Conclusion: High index of suspicion is required to diagnose Wilson's disease in those who present with unexplained neurologic (especially extrapyramidal), psychiatric and other clinical profile at any age of presentation, but particularly at younger age.



K. Vangiliappan, L. R. Narasimhan, N. Thamilpavai, S. Subramanian, S. Gopinathan

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

Objective: Emesis is often thought of as a gastrointestinal motility disorder. It can be the sole presenting manifestation of an underlying neurological disorder. Materials and Methods: An observational study of 60 patients who presented with vomiting at our Institute of Neurology, Madras Medical College was analysed. Exclusion criteria: Patients with gastrointestinal, psychological, drug induced causes of vomiting were excluded. A detailed questionnaire was administered regarding emesis-time, frequency, duration, associated with nausea or not, other associated symptoms, nature of vomitus, aggravating and relieving factors. Detailed neurological examination was performed and appropriate investigations like MRI, NCS, and Autonomic function tests, Upper gastrointestinal Endoscopy, USG abdomen and routine blood investigations were performed. Results: In our study 10 patients presented with Migraine headache, 10 with Intracerebral hemorrhage, Subarachnoid hemorrhage in 6 patients, Cortical venous thrombosis in 5, 5 with Idiopathic Intracranial hypertension, 5 with Meningoencephalitis, 5 with Benign positional paroxysmal vertigo. 3 patients presented with lateral medullary syndrome, Brain abscess in 2 patients, Guillain barre syndrome in 2, Sjogren's syndrome in 1, Neuromyelitis optica spectrum disorder in 1, Low brain stem Glioma in 1 patient, GBS in Diabetes mellitus in 1, HIV with Tubercular brainstem abscess in 1, 1 with vestibular neuronitis, 1 with Meniere's disease. Conclusion: Better understanding of the emetic neural circuitry is important to provide appropriate therapeutic interventions as it is sometimes life threatening.


Comorbid sleep apnea in acute neurological disorders: Experiences from a tertiary care centre

A. Sivadasan, M. Alexander, S. Aaron, V. Mathew, A. T. Prabhakar, S. Babu

Department of Neurosciences, Christian Medical College, Vellore, Tamil Nadu, India

Introduction: Co-morbid obstructive sleep apnea (OSA) though frequent, is often under-diagnosed in stroke and epilepsy. OSA often predates and worsens during the acute stages with potentially adverse outcomes. Methods: 24 consecutive patients (2015-2016) with acute neurological disorders and diagnosed to have OSA based on overnight polysomnography and started on Positive airway pressure therapy were included in the study. Results: Of the 24 patients, 18 had acute stroke and 6 had epilepsy with worsening seizure frequency. In the stroke group, mean age was 52 (+15.9) years, 17 were males. Six (33.3%) were below 40 years. OSA was graded as: severe (11, 61.1%), moderate (6, 33.3%) and mild (1, 5.6%). Comorbidities included hypertension (66.7%), Diabetes Mellitus (27.8%), dyslipidemia (50%) and coronary artery disease (16.7%). Fifteen (83.2%) had acute ischemic stroke, two had cerebral venous thrombosis and one had a haemorrhagic stroke. Anterior circulation was involved in 13 (72.2%), posterior in 5 (27.8%). Majority had large artery disease (12, 66.7%). Intracranial atherosclerosis was noted in 16 (88.9%), 6 (33.3%) had extracranial disease. On follow-up, improvement in neurological outcome was noted in 16 (88.8%). Severe OSA was associated with stroke severity (p 0.02) and adverse outcomes (p 0.03). Six patients with epilepsy had worsening seizure frequency despite adequate anticonvulsants. All were males, mean age (42 years). Etiologies included hippocampal sclerosis (3), autoimmune encephalitis (1), cortical dysplasia (1) and neurocysticercosis (1). Reduction in seizure frequency was noted in all patients. Discussion: Untreated patients with co-morbid OSA have worse functional outcomes. Early diagnosis and treatment prevents subsequent cardiac and cerebrovascular events with improvement in outcomes. This also results in better seizure control in epilepsy. Being an under-recognised and modifiable risk factor, the routine screening for OSA in this subset of patients needs to be emphasized.


An unusual case of CNS meningioma - Presentation with three and half years of pure peripheral weakness

A. Kumar

Department of Neurology , BHU, Varanasi, Uttar Pradesh, India

Background: Clinical presentation of meningioma vary with the CNS location and usually presents with predominantly central manifestations. We report an unusual case of long standing triparesis without central manifestations who was found to have CNS meningioma. A 61 year old right handed lady, without significant past medical history, presented with three and half years history of progressive, spastic left upper limb and left lower limb weakness followed by right leg proximal weakness of 1 year duration with tingling in both legs without bladder disturbances or cognitive or behavioural or visual disturbances or seizures or features of raised ICT. On FAB and MOCHA testing and detail mental state testing was normal. Left biceps was 4+ and other DTR on left side were 3+ and normal on other side. Plantar response was bilateral flexor. On clinical suspicion of cervical myelopathy patient was advised MRI cervical spine which was normal. Patient was then subjected to MRI brain and was found to have large 8*6*4 cm right parietooccipital meningioma with surrounding oedema with transfalcine herniation. Patient was then referred for surgical intervention.


A case of orbital cysticercosis

M. Arasu, J. F. Ambrose

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

A 35 year old lady presented to us with recurrent headache and right retroorbital pain. Examination revealed no focal neurological deficit. There was no papilledema. No meningeal signs. MRI brain showed an enhancing cystic lesion in the right orbit suggestive of orbital cysticercosis. She was treated with albendazole and steroids and symptomatically improved.


Early electrophysilogical findings in Gullian Barre syndrome

R. R. Wahatule, A. B. Taly, M. Nagappa

Department of Neurology, NIMHANS, Bengaluru, Karnataka, India

Introduction: Electrophysiological studies are important in the diagnosis of GBS. Early diagnosis of GBS is important as it will decide the treatment and prognosification of illness. Studies on early electrophysiological changes in GBS are sparse. Aim: To describes the early electrophysiological changes in first week of GBS. Patients and Methods: This is prospective study done at university hospital. Patients fulfilling the NINDS criteria from December 2014 to April 2016 admitted under single neurology unit (n=130) were studied, out of which patients coming in the first week of illness included in the study. Electrophysiological testing was done on Median, Ulnar & Common peroneal nerve. Values beyond 2 standard deviations of normalative laboratory data were considered abnormal. Final categorisation was done according to Haddens criteria. Results: Cohort consisted of 52 patients with male to female ratio of 2.2:1. Mean age was 37.2 (Range 5 to 83) years with mean duration of symptoms of 4.5 days (range 2 to7), mean MRC score of 31 (±14.4) and mean HDS of 3.6 (±0.71) with mean time to do electrophysiological testing was 5 days (±1.09). Absent F waves: median 22 (42.30%), ulnar 31 (59.61%), common peroneal nerve 33 (63.46%). Reduced persistence of F waves: median 14 (26.9%); ulnar 8 (15.38%); common peroneal 8 (15.38%). Distal latencies were prolonged: Median 25 (48.07%): ulnar 27 (51.92%); common peroneal 25 (48.07%). Conduction velocities were reduced in: median 13.9 (26.73%); Ulnar 9 (17.30%); Common peroneal 13 (25%). Sensory nerve action potential was reduced in: Median 32 (61.53%); Ulnar 32 (61.53%); Sural 4 (7.6%). Sensory nerve conduction velocity was reduced in: Median 19 (36.53%); Ulnar 7 (13.46%); Sural4 (7%). Most common electrophysiological subtype observed was primary demylinating 29 (55.76) followed by Equivocal 15 (28.84%), Axonal 8 (15.38%) & Inexcitable 0. Conclusion: F waves were most common abnormality during the early phase of GBS. None of the patients showed inexcitable motor nerves in the first week. Relative sparing of sural amplitude & velocity was seen. Ealry diagnosis can differentiate between mimickers like Transverse myelitis and Hypokalemia which present as flaccid quadriplegia.

Poster Session 04; Movement Disorders16:30-19:30 h, Friday, November 11, 2016


Extra pyramidal syndromes in hyperosmolar hyperglycaemic state

A. K. Roy, R. Siddhesh, Hegde, S. Murali, Raghunandan, Nadig

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

Introduction: Extrapyramidal syndromes occur due to a focal vascular lesion, neoplasm, granuloma, in the contra lateral basal ganglia. Metabolic derangements especially HHS can also cause focal neurological dysfunction like hemiballismus, chorea, hemiparesis or a focal seizure. We present clinical profile and MRI brain findings of extrapyramidal syndromes due to hyperglycemia. Materials and Methods: Patients admitted with acute extrapyramidal syndromes were included. A detailed history and examination including Hemogram, Blood sugar, HbA1C, Renal function test, Lipid profile and MRI of the brain were done. Results: From May 2010 to April 2016 there were 9 patients admitted with hemichorea and 1 of hemiballismus. All had hyperglycaemia without ketosis. The median age of these patients was 60 years, mean duration of symptoms was 3 days and their mean HbA1c was 11.1. Extrapyramidal syndromes subsided once the hyperglycaemia was controlled. Case Report: 76 year old gentleman presented with flinging involuntary movements of right upper and lower limb since 3 days. Because of these movements he was unable to walk and had falls quite frequently. The involuntary movements were hemiballismic. He was found to have DM type II. His HbA1c was 18.6. MRI of brain revealed unilateral T1 hyper intensities in the contralateral striatum and normal T2W images. There was no Diffusion restriction, no blooming in gradient echo sequence and MRS revealed low NAA peak. His symptoms subsided with control of hypoglycaemia. Conclusion: Recognition of these unique clinical syndromes is important as correction of the underlying metabolic derangement will lead to rapid improvement.


Study on efficacy of zolpidem in progressive supranuclear palsy

S. B. Patil, S. Khadilkar, K. Jagiasi, G. Soni

Department of Neurology, Grant Government Medical College and Sir Jamshedjee Jeejeebhoy Group of Hospitals, Mumbai, Maharashtra, India

Introduction: Progressive Supranuclear Palsy (PSP) is a typical parkinsonian syndrome. PSP does not respond well to conventional Parkinson's disease treatment, thus a new treatment which will mitigate what is otherwise a relatively bleak prognosis is urgently needed. Zolpidem is gamma-Aminobutyric acid (GABA) agonist of benzodiazepine (BZ1) receptor, found in output structure of basal ganglia which may enhance the degenerating basal ganglia output activity and rescuing ocular and motor symptoms. A study done by Daniele et al. showed improvement in vertical saccades and motor symptoms in 10 patients of PSP treated with zolpidem. Aims: To study whether zolpidem is more effective in relieving motor and cognitive symptoms of PSP than current treatment of levodopa. To quantify improvement with zolpidem using standardized clinical severity scale for PSP. Materials and Methods: Twenty patients with a clinical diagnosis of PSP were included in the study. All patients were subjected to MRI of brain to exclude other causes. All patients were started on standard therapy of levodopa for at least 2 months and then zolpidem was added. They were subjected to PSP- clinical rating scale prior to start of therapy and then at regular intervals till 1 year. Data analysis was done by using Friedman test using Predictive Analytics SoftWare (PASW) statistics 18 software. Results: The mean age was 63.6 years with a male: female ratio of 2.3:1. The mean duration of symptoms was 1.7 years. The mean PSP clinical rating score compared shows rising trend with increased duration of treatment. This increase in score with duration was statistically significant (p value <0.001). Mean score for frequency of falls, vertical up-gaze palsy, mental score and limb rigidity also increased significantly (p value <0.001). The most significant side effect was giddiness, though it was not dose limiting. Conclusion: This study reveals no significant improvement in motor or cognitive symptoms of PSP with zolpidem.


Hypoparathyroidism and movement disorders

N. A. Nadkarni, K. Satish, H. Abhinay, P. Sarika

Department of Neurology, Grant Government Medical College and Sir Jamshedjee Jeejeebhoy Group of Hospitals, Mumbai, Maharashtra, India

Background: Hypoparathyroidism can cause hyperkinetic as also hypokinetic movement disorders. We present 4 cases of movement disorders due to hypoparathyroidism. Neuroimaging in them showed calcification in basal ganglia, thalami and cerebellum. Case 1: A 12 years boy with mental retardation presented with recent episodic involuntary movements involving all limbs and flowing from one body part to another with retained consciousness. They lasted 5-10 minutes, and occurred every 1-2 hours without any triggering factor. In early infancy he had seizures with documented hypocalcemia, but was seizure free thereafter. He was diagnosed to have Primary Hypoparathyroism with Paroxysmal Non-Kinesigenic Dyskinesia. Case 2: A 50 years old lady presented blepharospasm, broad based gait, mild bradykinesia and tremors since 3 months. She had cataract surgery in both eyes at 13 years age. She had Primary Hypoparathyroidism. Case 3: A 59 years old lady presented with involuntary movement in left hand, and imbalance to walk. She had staccato speech, ataxic gait, darting tongue, and left hemichorea. She has diabetes since 15 years, and undergone thyroidectomy 10 years back. Investigations revealed hyperglycemia (400 mg/dl) with low calcium and parathyroid hormone levels. Patient probably had multi-factorial metabolic etiologies (Secondary Hypoparathyroidism following Thyroidectomy and Hyperglycemia) for her movement disorder. Case 4: A 26 years man had undergone thyroidectomy 10 years back. He had progressive slowness in his activities since 4 months. Since 2 months, he started to have hypocalcemic seizures. Examination revealed bilateral bradykinesia, rigidity, intention tremor, dysdiadochokinesia and impaired tandem walk. He had developed Secondary Hypoparathyroidism following Thyroidectomy. Conclusion: Suspect movement disorder due to hypoparathyroidism if it occurs on background of hypocalcemic seizures, childhood cataract or post-thyroidectomy status.


Hypoxic ischemic encephalopathy presenting as status dystonicus

S. Ray, R. Yadav, P. K. Pal

Department of Neurology, NIMHANS, Bengaluru, Karnataka, India

Introduction: Status dystonicus (SD) is a term assigned to extreme, continuous, generalized muscle contractions that is poorly responsive to treatment. It contributes significantly to morbidity and mortality in patients with dystonia. Dystonia is a clinical feature in patients with a prior history of anoxic brain injury. Here we report a rare case of acute hypoxic ischemic encephalopathy presenting with SD. Case Report: A 13-year-old boy developed acute onset giddiness while riding his bicycle followed by unconsciousness. He was detected to have acute myocardial infarction with low ejection fraction. His Magnetic Resonance Imaging of brain showed extensive hypoxic damage. On day 16 he developed ophisthotonic posturing with dystonic spasms which were not responsive to treatment. He was managed with trihexphenidyl, haloperidol, promethazine, tetrabenazine, diazepam, levetiracetam and baclofen with partial resolution of the dystonic spasms. He eventually succumbed to cardiac illness. Serum creatinine and urine was normal at admission. Serum creatine phosphokinase was markedly elevated and urine was positive for myoglobinuria indicating organ damage secondary to SD. Discussion: The commonest cause of SD is primary torsion dystonia. Other causes are Juvenile cerebral palsy, Pantothenate kinase associated neurodegeneration, Wilsons disease, ARX syndrome, Megalencephalic leucoencephalopathy with subcortical cysts. SD is usually triggered by fever, infection, exposure to medication like dopamine blockers or their abrupt cessation. Status dystonicus, as a presenting clinical feature of hypoxic ischemic encephalopathy has not been described in literature. The standard treatment for SD consists of anticonvulsants, trihexylphenidyl, baclofen, midazolam and propofol infusion supported with intravenous hydration and ventilation. Deep brain stimulation is the next line of management in refractory SD. Conclusion: Management of brain anoxia is challenging, even more so when the presentation is compounded by refractory SD.


Huntington chorea - a misdiagnosis for adult onset chorea with dominant inheritance - Neuroferritinopathy

J. Kaur, G. Singh, B. S. Paul

Department of Neurology, DMCH, Ludhiana, Punjab, India


Neuroferritinopathy is an adult onset progressive movement disorder with dominant transmission caused by mutations in the ferritin light chain gene (FTL1). Features overlap with common extrapyramidal disorders: idiopathic torsion dystonia, idiopathic Parkinson's disease and Huntington's disease. Case History: A 31 year old Indian woman presented with complaints of involuntary movements for last 5 years. She started noticing sudden jerks in her right leg. The movements progressed rapidly to all her four limbs, making it difficult for her to wear her clothes, combing hair, wearing slippers, holding a glass of water or even breaking a chapati. It also hindered her walking, although she could walk but she had the fear of falling due to sudden jerks in her legs. The movements were noticed even during sleep. Her husband noticed change in her behavior in form of aggressiveness. There is family history of similar complaints in her grandfather, father, aunt and her children - suggestive of dominant inheritance. On clinical examination - her mean MMSE - 21/30 with Frontal assessment battery -12/18 suggested frontal dysexecutive type demetia. Speech is interrupted with sudden pauses and rapid inhalations of air. With normal muscle tone and strength; she has brisk tendon reflexes and bradykinesia. Her hyperkinetic movements are asymmetrical yet generalized with dominant movement being appendicular chorea and other movement is truncal dystonia. This is associated with milkmaid's grip and jack in the box (hands & tongue chorea). While walking she has wide based, irregular gait interrupted with spontaneous knee flexions. With normal routine lab work up, MRI brain with SW images suggested areas of iron deposition in bilateral Globi pallidi, Substantia nigra and Dentate nuclei. Discussion: An adult onset progressive chorea with family history consistent with dominant transmission and evidence of excess iron storage on brain MRI, we are suspecting neuroferritinopathy in this family. Conclusion: To the best of our knowledge, neuroferritinopathy has been mostly reported from United Kingdom and France. And this might be the first case report in a large Indian family. Genetic analysis has been sent for FTL gene and we are awaiting the results for the same.


1. Chinnery PF, Crompton DE, Birchall D, Jackson MJ, Coulthard A, Lombès A, et al. Clinical features and natural history of neuroferritinopathy caused by the FTL1 460InsA mutation. Brain 2007;130(Pt 1):110-9.


Secondary parkinsonism in liver disease

K. Gowthaman, T. P. Krishnaprasad, G. Sarala, K. Shanmugasundaram, P. Kumar, S. Gobinathan

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

Objective: To emphasize the importance of ruling out liver disease in patients presenting with Parkinsonism. Background: Non wilsonian liver disease may be associated with parkinsonian syndromes. Cirrhosis presenting as Parkinsonism is a distinct subset of acquired chronic hepato cerebral degeneration. The entity is not rare and unless suspected, cirrhosis can easily be overlooked. We describe three cases presented with Parkinsonism features that were diagnosed as cirrhosis of liver. Methods: Observational case series in Madras Institute of Neurology, Chennai during September 2015 to April 2016. Totally three patients are included. Results: All three patients initially presented to neurology department with chief complaints of bilateral symmetrical tremor associated with bradykinesia. Investigations for Wilsons were negative for all three patients. For first patient Ultrasound abdomen revealed hepatocellular carcinoma which was confirmed with contrast computed tomography (CT) abdomen and liver biopsy. For other two patients cirrhosis was evidenced by reversal of serum albumin to globulin ratio and abdominal ultrasound were consistent with cirrhosis. Grade II oesophageal varices on Oesophago-Gastro Deuodenoscopy (OGD) scopy was present in all three patients. Only one patient had MRI abnormality of symmetrical T1 hyperintensity in basal ganglia. They improved to variable extents after treatment for cirrhosis, along with dopa agonists. Conclusion: We stress the importance of recognizing this chronic acquired hepatocerebral degeneration syndrome. Investigating for cirrhosis should be considered for patients presenting with Parkinsonism.


Uncommon visual hallucinations and associated behavioural disturbance in five patients with parkinson disease

P. Ranganathan

Department of Neurology, Pazhani Neuro Centre, Pazhani, Tamil Nadu, India

Background and Aims: In these study five Parkinson patients with unusual visual hallucination, the associated behavioural disturbance and the management issues are analysed. Methods: All the five patients in this case study have Parkinson disease. The main problem is troublesome visual hallucinations and the behavioural disturbance. Results: Case 1 - Eighty five year old male patient with Parkinson disease, complaint of seeing spiders all around the house. He moves with a basket and catch the spiders which are not there, go outside and throw it. Case 2 - Seventy year old male patient with Parkinson disease started seeing snakes everywhere, due to which he tries to run away from the house.

Case 3 - Seventy two year old female patient with Parkinson disease noticed the people in the photographs on the wall are coming out of the photograph and talking to her.

Case 4 - Seventy five year old male patient with Parkinson disease started seeing eagles and he is able to describe the sound it makes. Case 1 - 4 patients responded by reducing the dose of L dopa and adding rivastigmine. Case 5 - Sixty year old gentleman with Parkinson disease started feeling patches of mites all over his body. He takes frequent bath for it. He became better with a small dose of antipsychotic. Conclusion: Parkinson disease patient with behavioural disease need extra time in a busy clinic, because it may be due to the visual hallucination. By adjusting the medications, adding appropriate medicines and by counselling a significant difference can be made to the patients and care takers quality of life.


Non motor symptoms in patients with writer's cramp

P. Chatterjee, S. Choudhury, B. Mondal, M. U. Kulsum, Chatterjee, H. Kumar

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

Introduction: Writer's cramp (WC) is essentially a motor disorder, involving the basal ganglia and its connections. It is well known that Non-motor symptoms (NMS) are coexisting features in a number of movement disorders involving basal ganglia. But NMS are not well documented in cases of WC. We aim to explore the non-motor features of WC patients and correlate those with motor symptoms. Methods: Patients attending the outpatient department of a tertiary care neurology referral centre in the eastern part of India were recruited, who presented with writing difficulty and diagnosed as writer's cramp by neurologist. Individual patients were thoroughly assessed through various motor (DASH, WCRS, ADDS) and non motor (MINI, MOCA, GAD7, PHQ9, OCI, SF36) scales. Audio visual recording of uniform writing tasks were performed for all patients. Results: 30 consecutive patients with WC were recruited. Interestingly, all of them were male. The mean age of the patients was 54.83 years (SD 10.77). Mean disease duration for this cohort was 72.36 months. The average motor disability of the patients was 18.4% of maximum possible disability (DASH score). 70% and 60% patients reported Generalized Anxiety Disorder (GAD) and Major Depressive Episodes (MDE) respectively in recent past. 50% had Obsessive Compulsive Disorder (OCD) with a mean OCI score of 2.22 (maximum mean score 4). Panic disorder and social phobia were present among 26.7% and 60% respectively. Out of 18 patients suffering from social phobia, 12 were phobic specific to writing tasks in public 95.2% patients with GAD and MDE had moderate to severe grade of anxiety and depression respectively. Cognitive impairment was present in 25 (83.3%) WC patients. DASH score is negatively correlated with total MOCA score and attention score. Conclusion: Anxiety, depression, social phobia and OCD are four most frequent non motor symptoms present in WC patients. Functional disability.


Non motor symptoms of Parkinson's disease in patients with REM sleep behavior disorder

S. Shubham, S. Choudhury, R. Banerjee, P. Chatterjee, B. Mondal, Kumar

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

Introduction: Rapid Eye Movement Sleep disorder (RBD) is commonly associated with synucleinopathis like Parkinson's disease (PD). Due to presumably common pathogenesis, non motor symptoms of PD are likely to be closely related to RBD in patients with PD. Hence, we undertook this study to evaluate the association of RBD with several non-motor symptoms of PD. Methods: In this cross sectional analytical study we evaluated 67 consecutive patients of PD and screened them for coexistence of RBD through a validated rating scale designed to detect RBD cases. We also thoroughly evaluated them for any concurrent non-motor symptoms and analyzed to explore the association of RBD with individual non-motor symptom. Results: RBD was detected in 23 (34.33%) patients of PD. Mean Unified Parkinson's Disease Rating Scale (UPDRS) score was comparable between these groups (RBD 40.26, Non-RBD 39.68, p value 0.50). PIGD (Postural Instability and Gait Disturbance) variant of PD patients were remarkably common among RBD patients (91.3%) compared to non-RBD patients (70.5%) with PD. Constipation (Odd's ratio 6.08) and hallucination (Odd's ratio 7.29) held a strong association with RBD. Conclusion: RBD was found to be a frequently associated non-motor coexisting condition with PD in our sample. The presence of RBD is independent of Parkinson's disease duration and stage of disease. However, few non motor symptoms (hallucination and constipation) were clearly associated with RBD therefore finding any one of these symptoms should alert the clinicians to look for RBD.


A rare case of movement disorder - Cortico basalganglionic degeneration

T. E. Raj, Saravanan, Murugan, Radha, Bobby, P. S. Peter

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

Introduction: Cortico basalganglionglionic degeneration is a rare disease presents with clinical features of movement disorders and cortical signs. Apraxia is an inability to correctly perform learned skilled movement. Case Vignette: A 53 years old gentle man presented wirh the complaints of inability to correctly perform actions required for dressing, difficulty in taking the food to the mouth, difficulty is writing, slowness of all activities and involuntary movements in the form of sudden jerky movemnts involving the right hand of 7 months duration. He had onset initially involving right upper limb, 3 months later left upperlimb. Examination revealed patient had difficulty in performing actions of pantomine to verbal command such as 'comb hair, brush teeth, flip coin, wave goodbye, salute, give the peace sign, Ok sign' etc and difficulty in performing "lmitation of gestures" He is able to identify the different actions performed by examiner. He could not do the action requiring to perpare a letter for mailing. He could not do limb kinetic movements. He has cortical sensory loss, ideomotor apraxia, dressing and constructional apraxia. There is no weakness, sensory impairment or cerebellar dysfunction. He had rigidity & bradykinesia. Investigations: MRI Brain revealed fronto parietal atrophy involving left side more than right sides. Corpus callosum & mid brain and normal. EEG showed amplitude is less in left fronto parietal region than right side. Discussion and Conclusion: Cortico basal degeneration is one of the rara cause of movement disorder, this patient fulfils the Criteria of definite CBD proposed by maraganore etal, Lang at al, kumar at al also the proposed criteria for the diagnosis of CBD.


Usefulness of a modified questionnaire as screening tool for swallowing disorders in parkinson disease: A pilot study

R. Sureshkumar, U. Menon

Department of Neurology, Amrita Institute of Medical Sciences and Research Centre, Kochi, Kerala, India

Background: Parkinson's disease (PD) involves swallowing disorder as a symptom, which is often unreported. The present neurological evaluation parameters do not identify this symptom adequately. There is the need for a dedicated tool for this. A questionnaire should be designed to get maximum information with minimum trouble to the patient. Aims: To check for correlation between our questionnaire scores and the two standard PD disability scores (UPDRS and Hoehn & Yahr), and to study the correlation between the various subgroups of our screening dysphagia questionnaire. Methods: A questionnaire was developed with eleven items, keeping in mind the most relevant swallowing issues in PD patients. The questions were subdivided into four groups representing the different stages of swallowing. Reliability and validity study was conducted for the questionnaire. For the study, this tool was administered to the PD patients visiting a dedicated PD clinic in a tertiary referral hospital in central Kerala. The scores were checked and tabulated. Corresponding UPDRS and H&Y scores were documented on the same day. All scores were entered in an Excel sheet and statistical analysis applied. Results: Correlation coefficient values (r) of questionnaire score with H&Y and UPDRS scores were more or less the same (0.339 and 0.384 respectively), indicating moderate correlation with significant p values, and those between the mean scores of various groups ranged from 0.56 to 0.73, indicating comparatively high degree of correlation among the different groups of questions. Conclusions: The association between worsening motor symptoms and swallowing difficulties has been documented in this study. Oral stage of swallowing remains the most affected in PD, and this can be recognised early by using our questionnaire.


Case series of palatal myoclonus

K. P. Kothari, R. Chakor

Department of Neurology, Topiwala National Medical College and BYL Nair Hospital, Mumbai, Maharashtra, India

Objectives: Case series of Palatal Myoclonus Background: Palatal Myoclonus is a rare form of segmental myoclonus which presents as involuntary movements of the muscles of soft palate, throat and other structures derived from the branchial arcs. It can be of two types - essential or symptomatic. Methods: 3 cases of palatal myoclonus presented to our movement disorder clinic in the span of January 2015 to December 2015. The patients were evaluated with detailed clinical history, neurological examination followed by neuroimaging and electrophysiology. Results: Our first patient, a 27 year old male presented with loud clicking sound in his ear and involuntary movements of his tongue since 9 months. He had palatal myoclonus of frequency 160-170/minute with a loud clicking sound audible to the examiner due to slapping of base of the tongue to the palate. His MRI brain was normal and thus was having essential palatal myoclonus. The palatal myoclonus responded to sumatriptan, having failed the trials of carbamazepine, valproate and clonazepam. Our second patient was a 65 year old male who presented with acute onset vertigo and ataxia. His examination revealed palatal myoclonus of frequency 50-60/minute without audible click. His MRI revealed a right lateral medullary infarction. The third patient, a 45 year old male was admitted with complaints of progressive speech difficulty, dysphagia and imbalance while walking since 2 years. In addition to palatal tremor he had dysarthric speech, restriction of upgaze and cerebellar signs. His nerve conduction studies were normal. MRI brain was suggestive of olivopontocerebellar atrophy with atrophy of cervical spinal cord. Workup for Spinocerebellar ataxias was negative and thus was diagnosed to have Progressive Ataxia with Palatal Tremor (PAPT). Conclusion: Palatal Myoclonus is a rare movement disorder with diverse causes and variable presentations.


Antiphospholipid antibody with Anti-Jo 1 antibody associated chorea-cause or association?

R. I. Mistry RI, S. Kushwaha, L. Bhirud, V. Banga

Department of Neurology, Institute of Human Behavior and Allied Science, New Delhi, India

The antiphospholipid antibody syndrome (APS), an acquired thrombophilia, is characterized by vascular thrombosis and/or fetal loss in the presence of antiphospholipid antibodies (APLAs). The most common neurologic manifestations of APLS are stroke or transient ischemic attacks, which were the initial presentation in up to 30% of adults with APLS in a large European cohort. In the paediatric APLS registry, migraines (7%), chorea (4%), and epilepsy (3%) were the most common nonthrombotic neurologic manifestations. The commonest antibody that binds to amino-acyl-t-RNA synthetases, that is anti-Jo-1, is directed against histidyl t-RNA synthetase which is seen in 15% to 30% of adults with polymyositis or dermatomyositis. The antisynthetase antibodies have specificity for clinically distinctive neuromuscular and systemic syndromes that include muscle weakness and pain, Reynaud's phenomenon, interstitial lung disease, arthritis, and skin disorders. Till date no case has been reported that had presence of both Antiphospholipid antibody and anti Jo-1 antibody positive in a patient with chorea. Here we report a case of generalised chorea with anti Jo-1 antibody with APLAs.


A study of pain in Parkinson's disease

S. U. Meenakshisundaram, S. Tito, S. Balasubramanian

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

Background: Pain is one of the recognised non-motor symptoms in Parkinson's disease. However, not much research has been done into definitively defining pain and its management in Parkinson's disease. Aim: To study the prevalence, type and severity of pain in patients with Parkinson's disease. Methods: This was an observational study that included 51 patients with Parkinson's disease from the Movement disorder clinic in the Institute of Neurology, MMC, Chennai. A structured interview which included questions about the presence, type, severity of pain was administered. The disease characteristics like duration and stage of PD was also noted. The severity of pain was graded using the visual analog score. Results: Out of the 51 patients included in the study, 33 (64.7%) had complaints of somatic pain. The most common localization of pain was to the lower back (33.33%) followed by the lower limbs (21.21%). Dystonic cramping pain (57.6%) was the most common type followed by dull aching type of pain (24.24%). In the visual analog score, 63.64% of patients had a score between 5 and 8 indicating moderate severity. Duration since onset of motor symptoms and stage of Parkinson's disease did not significantly correlate with the presence of pain. Conclusion: Pain is a common symptom in patients with Parkinson's disease but has varying characteristics with subjective differences between patients and poor correlation with disease characteristics.


Parkin mutations in IPD patients in rural eastern India

R. V. Aravinda, G. Vishal, S. K. Das, A. Senapati, R. Jharna, A. Biswas

Department of Neurology, Burdwan Medical College (BMCH), Burdwan, West Bengal, India

Idiopathic Parkinson's disease (IPD) was thought to be the disease of the elderly. But later, epidemiological studies showed that it is prevalent in the younger patients as well. This further led to the studies determining the genetic basis of the disease. Mutations were then seen in various genes in different loci and various mutations in a single gene. Most prevalent mutation was seen in the parkin gene. Among the parkin gene mutations, p.Gln34Arg and p.Arg334Cys missense were the most common. The present study was conducted to determine the prevalence of these two mutations and the clinical profile in the patients attending the outdoor and indoor of the Neurology department of Burdwan Medical College and Hospital, Burdwan (West Bengal) which caters for the significant portion of the rural population in Eastern India. 60 IPD patients were selected for the study with their consent after taking detailed history and performing through physical examination. Neuro-imaging was done as per requirement. 10 ml blood was withdrawn from the included patients into the vials containing EDTA. The nucleic acid material was then isolated from the blood samples using the standard method. The gene of interest was amplified by polymerase chain reaction. Then the sample was processed to detect the two most prevalent mutation in the parkin gene. These mutations were seen in about 12% of the IPD patients and p.Gln34Arg being more common. This mutation was also more prevalent in male patients and was associated with tremor predominant disease as compared to the p.Arg334Cys mutation which was associated with faster progression of the disease. However further studies with larger sample size are required in this part of the country.


Excessive daytime somnolence in Parkinson's disease in a tertiary care hospital: A cross-sectional study

A. Ghosh, V. Shankar, U. Meenakshisundaram, C. U. Velmurugendran, P. Hazeena, T. Sugumar

Department of Neurology, Sri Ramachandra University, Chennai, Tamil Nadu, India

Objectives: To ascertain the magnitude/proportion of Excessive Daytime Somnolence (EDS) in patients of Idiopathic Parkinson's Disease (PD) and to analyse its association with certain variables. Methods: 38 patients of Idiopathic Parkinson's Disease, who attended the Neurology outpatients department at Sri Ramachandra University between 1.1.2016 to 30.6.2016 were assessed for EDS. They filled up the Epworth Sleepiness Scale. Thereafter it was evaluated if certain variables influenced the development of EDS. The five variables taken were: sex, Hoehn and Yahr stage, concomitant use of Dopamine agonists, place of residence urban or rural and if the patient had a predisposition to sleepiness prior to the onset of PD. Results: Of the 38 PD patients, 20 (52.63%) suffered from EDS, out of which 14 had mild EDS (ESS 10-15) whereas 6 had severe EDS (ESS 16-24). 14 out of 22 males (63.6%) and 6 out of 14 females (42.9%) reported EDS. 2 out of 6 (33%) in Stage 1, 1 out of 6 (16.7%) in Stage 1.5, 3 out of 8 (37.5%) in stage 2, 4 out of 5 (80%) in stage 2.5, 6 out of 7 (85.7%) in stage 3, 4 out of 4 (100%) in stage 4 had EDS. 28 patients were on Dopamine agonists of which 17 (60.7%) reported EDS. 15 of the 25 urban patients (60%) and 5 out of 13 (38.5%) rural patients had EDS. 7 out of 20 (35%) EDS patients had a tendency to fall asleep prior to the development of PD. Conclusion: Our study suggests that EDS in PD is common (52.63%). It also suggests that it is commoner in males and in the later H&Y stages, afflicts the urban population more often, commoner in patients on Dopamine agonists and can be significantly present in patients before the onset of motor manifestations of PD.


Tetrahydroisoquinoline attenuates parkinsonian neurotoxicity

R. Banerjee, M. N. Ali, P. Kochupurackal, Mohanakumar, P. Jaisankar, S. C. Biswas

Division of Cell Biology and Physiology, Indian Institute of Chemical, Biology (CSIR), Kolkata, West Bengal, India

Parkinson's disease (PD) therapy remains symptomatic with L-Dopa causing significant unwanted side effects. Hence the search for novel molecules in PD treatment continues. Parkinsonism is described as 'kampavata' in ancient Indian Ayurveda medicine and traditional herbal therapy indicates improvement in PD patients. Apart from herbal L-Dopa content, other molecules might be neuroprotective in PD. In the present study, tetrahydroisoquinoline (TIQ) alkaloid, identified in Ayurveda for treating PD was evaluated for its neuroprotective potency in a cellular model. Co-culture of murine neuronal (Neuro2a), microglial (EOC20) and astrocytic (C8D30) cells simulating cell-to-cell communication in brain was established. Co-cultures were set by indirect contact of dopamine neurons (dbcAMP-induced differentiation of Neuro2a cells) in recipient wells with glia on insert membrane or by direct contact of neurons, microglia and astrocytes. Exposure of cells for 24 h with MPP+ a potent dopaminergic neurotoxin and active metabolite of MPTP, known to cause irreversible parkinsonism in humans led to 40-50% loss of cell survival. Different concentrations of TIQ (0.1-10 μM) incubated for 24 h post-MPP+ were tested for neuroprotection against parkinsonian neurotoxicity in vitro. Significant attenuation of the neurotoxin-induced loss of total cell viability achieved by TIQ (10 μM) treatment was demonstrated by conventional MTT cell viability assay. With prolonged exposure (48 h) to the neurotoxin such protection was not observed. A more sensitive flow cytometric procedure using a Live Dead Assay kit, confirmed that TIQ treatment caused a significant (37%) increase specifically in the number of live (Calcein AM-positive) differentiated neurons compared to MPP+ alone incubated for 24 h but not for 48 h. Further, MPP+ -induced mitochondrial accumulation of toxic superoxide radicals in dopamine neurons was significantly reduced (30%) by TIQ (10 μM) as determined by MitoSOX dye flow cytometry. These findings demonstrate the anti-parkinsonian neuroprotective potency of TIQ a novel Ayurveda molecule in vitro.


Transfer dysphagia due to focal dystonia

P. Samal, V. Goyal, M. B. Singh, M. V. P. Srivastava

Department of Neurology, AIIMS, New Delhi, India

Introduction: The inability to propel a food bolus successfully from posterior part of oral cavity into oropharynx is defined as transfer dysphagia. Transfer dysphasia can be due to paralysis of muscles, lower cranial nerve palsy, neuromuscular junction disorder (myasthenia) but rarely it can be due dystonia involving muscles of swallowing. Case History: We describe three cases of transfer dysphagia due to focal dystonia. The first patient was a 55 year old gentleman whose sole presenting complaint was transfer dysphagia in the absence of any obstruction or LMN palsy. He was previously being treated as psychogenic. He was given trihexyphenidyl and improved symptomatically. The second case was a 74 year old lady who had transfer dysphagia and spasmodic dysphonia. Dysphagia improved with oral symptomatic treatment but she required botulinum toxin for dysphonia. The third case was a 60 year old gentleman with oraomandiblar dystonia who developed transfer dysphagia and later developed blepharospasm and torticollis. Discussion: These three patients represent that transfer dysphagia as a form focal dystonia may appear as the sole presenting complaint or may present with other forms of focal dystonia or as a part of a spectrum that may precede generalized dystonia. A high index of suspicion and adequate drug trial could facilitate diagnosis and avoid unnecessary and recurrent investigations (Video to be presented).


The quantum soul

V. T. Haridas

Department of Neurology, Elite Mission Hospital, Thrissur, Kerala, India

Man has wondered about the presence of a soul which can exist outside the materialistic body. Hindu philosophy believes in the existence of a DEHI (soul) inside the DEHA (body). This soul is believed to be eternal and capable of entering and exiting bodies. Near death experience and rare cases of re-incarnation have been interpreted as a strong evidence of soul. With the advent of modern science, especially physics and physiology, the concept of soul was sidelined. Every conscious and unconscious experience was given solid explanations based on neurophysiology and conventional physics. But conventional physics has failed miserably to explain reincarnation and certain aspects of near death experience. A new group of neuroscientists have emerged who try to explain the soul on the basis of quantum physics. When it comes to the subatomic level, where we are dealing with distances in nanometers and miniscule masses, the laws of conventional physics are replaced by the rather bizarre quantum mechanics Microtubular system which forms the cytoskeleton of neurons is hypothesized to be the seat of soul. The interactions of these elements, which are separated by only few nanometers, are believed to function according to quantum principles and hence the concept - quantum soul. Various aspects of this theory and its impact on our concept of soul will be discussed.


Intermuscular coherence of patients with writer's cramp

S. Choudhury, P. Chatterjee, B. Mondal, M. Baker, S. Baker, H. Kumar

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

Background: Intermuscular coherence analysis is a simple and non-invasive electrophysiological test which has potential for clinical investigation. In addition, it might also indicate the origin of neural drive. Hence, we present our pilot study on the IMC pattern comparing writer's cramp patients with healthy volunteers. Methods: Surface EMG was recorded from three forearm and hand muscles while the participants performed a repetitive 0000 pinch grip on a force transducer; the target force was set to 10% of maximum voluntary contraction. IMC between first dorsal interosseous and both flexor digitorum superficialis and extensor digitorum communis were calculated from the period of steady contraction. Results: The average IMC plot for 10 healthy volunteers and 20 WC patients demonstrated a characteristically different pattern. A peak IMC was noted at 4-7 Hz in WC patients in contrast to healthy volunteers who had negligible IMC in that band (mean 4-7 Hz coherences 0.013 for healthy and 0.067 for WC, significantly different, P=0.009). No difference in IMC was noted between WC patients with or without writing tremor. A cumulative distribution plot comparing the two groups revealed that 4-7 Hz IMC above 0.03 (the largest IMC seen in healthy volunteers) was noted in 50% of WC patients. The area under the curve for the receiver operating characteristic was 0.865; this was significantly greater than the value of 0.5 expected by chance. Conclusion: We report, for the first time, a clear IMC at 4-7 Hz in WC patients. This is strongly indicative of dystonia and dystonic tremor present in patients with WC. The similar coherence patterns in patients with and without writing tremor possibly indicate that they belong to the same spectrum of dystonia with a diverging clinical presentation. This fast, simple and low-cost electrophysiological test has the potential for high diagnostic utility.


Interesting case of symptomatic hyperekplexia

M. Abid, S. Gobinathan

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

Introduction: Hyperekplexia is a rare, hereditary, non epileptic disorder characterised by two abnormal forms of response to unexpected auditory, visual and somesthetic stimuli, namely the sustained tonic spasm and exaggerated startle response. Case Vignette: 33 yr young male, house keeping staff by occupation came with a background probable history suggestive of birth asphyxia, delayed milestones, with childhood onset of unsteadiness and difficulty in walking with recurrent falls, difficulty in negotiating narrow pathways. difficulty in articualtion of speech with stammering, with difficulty in gripping chappal and unaware slippage of chappal, tremors and dystonic posturing of limbs with exaggerated jerking of entire body in response to loud sound since childhood with no worsening of symptoms since then. For the past 6 months the above symptoms had worsened, no h/o suggestive of cognitive, cranial nerve, spinomotor or ANS involvement on examination found to bilateral flat foot on general examination his detailed cognitive and lobar function normal, cranial nerve examination showed bilateral gaze evoked nystagmus, spinomotor examination suggestive of proximal weakness, with scanning dysarthria with dystonic posturing of both upper limbs in the distal joints, with broad based gait. His routine blood reports were normal, kf ring negative, smear negative for acanthocytes, csf lactate pyruvate were normal, nerve conduction normal, mir brain with whole spine screening normal, muscle and nerve biopsy were inconclusive but electron microscopy suggestive of mitochondrial myopathy wth subsarcolemmal aggregation of abnormal mitochondria varying in size with altered crista pattern. Discussion: Startle reflex" will be used to describe both motor responses evoked by sudden intense stimuli and the underlying neuronal pathways. Startle syndromes are heterogeneous in nature. startle reflex is a bilaterally synchronous shock-like set of movements. The most prominent features of the reflex are forceful closure of the eyes, raising of bent arms over the head, and flexion of the neck, trunk, elbows, hips, and knees. Symptomatic hyperekplexia is probably quite rare; reported causes mainly involve brainstem or cerebral damage. All cases of symptomatic hyperekplexia concern late-onset hyperekplexia without stiffness in the neonatal period and can therefore be clinically categorised as the minor form. Some patients that are symptomatic fall after the startle reflex, but whether this is the consequence of stiff ness or of the extreme startle movement itself is unclearThe minor form of hyperekplexia consists of excessive startle reactions without stiff ness. These startle reflexes never begin in the neonatal period. Reports indicate a start in early infancy, childhood, and puberty. In our case the patient also had difficulty since childhood with fluctuation in his complaint. Conclusion: We present this case of symptomatic hyperekplexia due to non syndromic presentation of mitochondrial disease due to its rarity. All sporadic cases to be worked up potential cause apart from genetic work up.


Comparative study of clinical profile of young onset and adult onset parkinson's disease

A. Satpathy, G. Ganguly, A. Biswas

Department of Neuromedicine, BIN, IPGMER, Kolkata, West Bengal, India

Background: Young-onset Parkinson's disease (YOPD) differs from adult-onset disease, but published information is scarce from India. Objective: to compare the clinical profile of YOPD patients with that of adult-onset patients. Methods: Sixty patients satisfying UK Parkinson's Disease Society Brain Bank's clinical criteria were recruited in the study from OPD and Movement Disorder Clinic of the Institute. Patients were assessed clinically and by using various subscales of Scales for Outcomes in Parkinson's disease (SCOPA) including SPES-SCOPA, SCOPA-COG, SCOPA-AUT, SCOPA-PC, and SCOPA-PS scales. Results: Of the 60 patients 16 (26%) were young onset and 44 (74%) were adult onset. Young onset patients had a more frequent (12% vs. 3.3%) family history of Parkinson's disease. Tremor was the predominant feature of adult onset group (63% vs. 25%) as compared to young onset. Frequency of treatment related dyskinesia or dystonia was more common in YOPD group (25% vs 9%). Cognitive deficit was more marked in adult onset group (27% vs. 12%). Conclusions: YOPD patients differ clinically from adult onset patients in frequency of family history and the early appearance of drug induced dyskinesia in contrast to cognitive deficit in later.


Frontal executive function assessment in patients with idiopathic Parkinson's disease in Indian population using frontal assessment battery

A. K. Datta, D. Das

Department of Neurology, R. G. Kar Medical College and Hospital, Kolkata, West Bengal, India

Background: Parkinson's disease is associated with motor as well as non-motor symptoms. Amongst the cognitive dysfunctions frontal dysexecutive symptoms are often seen in Parkinson's disease. The Frontal Assessment Battery (FAB) is a short tool for the assessment of executive functions consisting of six subtests that gives an idea of different functions related to the frontal lobe & its connection. Goals: To evaluate the clinical usefulness of the FAB in identifying different executive dysfunction in parkinson's disease; the types of executive dysfunction, to determine if FAB scores in PD are correlated with severity of Parkinson's disease & activity of daily living (ADL) scores. Methods: The study was conducted in R G Kar medical College. Cases were taken on the basis of random sampling from outdoor patients of neurology clinic of RG Kar Medical College & Hospital, Kolkata. Patients, who are able to read and write, were selected. Controls were taken from healthy relatives, hospital workers etc. Results: In the healthy subjects there were 41 men & 39 women. In healthy subjects mean FAB score were 15.85. In Parkinson's patients there were 117 men & 53 women. With age varied between 45 to 88 yrs. Mean age was 57.74 ± 8.42, mean education 8.63 ± 4.61. Mean duration of the disease was 5.56 ± 4.34 yrs. Mean MMSE 26.20 ± 3.80, mean UPDRS 35.60 ±20.56. & mean FAB score was 11.26 ± 3.67. Mean FAB scores were significantly decreased compared to controls, and correlated significantly with age & MMSE scores. Lexical fluency and Go- No go test showed most significant change. Conclusions: The FAB is a useful tool for the screening of executive dysfunction in Parkinson' disease. Normative data for the Indian population of this test will improve clinical use of FAB.


Adult onset primary lower limb dystonia

S. Mehta, A. Takkar, V. Lal

Department of Neurology, PGIMER, Chandigarh, India

The lower extremity is rarely involved in adult onset primary dystonia at the onset where as it is commonly affected in childhood onset cases. When the dystonia affects the lower limb at onset in adults, it usually has a secondary cause. We present two cases of adult onset primary lower limb dystonia seen over a period of 6 months in our institute. The first case is a 57 year old male who presented with action dystonia of right foot for the last 5-6 years. The second case is a 65 year old female who presented with right lower limb dystonia of 3 years duration. Family history was negative in both the cases. Neurological examination was otherwise normal with no evidence of Parkinson's disease. In both the cases extensive testing to rule out secondary causes including MRI brain came out to be normal. Genetic testing for DYT 1 was negative in the first patient. The first patient did not show any benefit with medical therapy (anticholinergics, levodopa) and Botulinum toxin. The second patient showed only marginal benefit with oral drugs. The second patient even underwent total knee replacement on the right side with no improvement postoperatively. To conclude, primary dystonia of the lower limb can occur in adults and awareness about this disorder can shorten the time to diagnosis and prevent unnecessary surgeries.


Prevalence of non-motor symptoms in parkinson's disease: A study from South India

J. R. Chaudhuri, K. R. Mridula, V. C. S. S. Bandaru

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

Background: Non-motor symptoms (NMS) play a vital role in Parkinson's disease (PD) and have become the leading cause of deterioration of quality of life and are associated with progression of disease. Limited studies are available on how antiparkinsonian medications affect NMS in disease duration of PD. Aim: to investigate the prevalence of NMS in PD and correlate it with disease duration. Methods: We selected 75 PD patients prospectively, from department of Neurology Yashoda hospital Hyderabad in the study period between June 2011 and May 2013. All PD patients were diagnosed by the UKPD Brain Bank criteria and all PD patients were trichotomized based on disease duration (below 4 years, 5-8 years and more than 9 years). NMS screening questionnaire comprising 30 items were completed by all patients. Results: Out of 75 patients men were 62 (82.6%), mean age 59.2±1.51 years with an age range of 45-69 years. Various NMS were subclassified into different categories. The prevalence of NMS in the three groups of <4 years, 5-8 years and > 9 years with number (%) are :- Gastrointestinal symptoms- 8 (38%), 12 (48%), 25 (86.2%), Cardiovascular dysfunctions -10 (47.6%) 11 (44%), 24 (82.7%), Urological problems - 8 (38%) 10 (40%) 21 (72.4%), Sexual disturbances- 9 (42.8%) 10 (40%) 23 (79.3%), Sleep disturbance- 8 (38%) 9 (36%) 22 (75.8%), Mood disorders - 7 (33.3%) 10 (40%) 23 (79.3%), Delusions and Hallucinations -7 (33.3%) 9 (36%) 21 (72.4%), Cognitive problems - 8 (38%) 8 (32%) 21 (72.4%), Miscellaneous - 7 (33.3%) 10 (40%) 17 (58.6%) respectively. All categories increased significantly as the disease duration increased. Conclusion: NMS is a common even in early disease and the prevalence increased significantly with the disease duration.


Drooling in parkinson disease

N. Sarma, S. Pandey

Department of Neurology, Jawaharlal Institute of Postgraduate Medical Education & Research, New Delhi, India

Background: Parkinson's disease (PD) is a neurodegenerative disorder consisting of both motor and non motor symptoms. Among the non motor symptoms, drooling of saliva is one of the most common manifestations. The prevalence of salivary drooling has been described to be between 10-73% among various studies. It is an important factor behind psychosocial impact of Parkinson disease on both the patient as well as caregivers. Aim: To study the prevalence of salivary drooling in PD. Materials and Methods: A total of 143 patients of PD were included in this study. Clinical history including presence of drooling of saliva was enquired. Patients underwent physical examination including Unified Parkinson's Disease Rating Scale (UPDRS). Results: Drooling of saliva was found to be present in 48 patients (33.56%). Majority patients in this group were males (72.9%). Only two (4.2%) out of those 48 patients had young onset Parkinson's disease (YOPD) (p<0.01). Tremor dominant PD was the most common phenotype seen among this group of patients (28/48, 58.3%) which was followed by mixed variety and then rigidity dominant (9/48, 18.7%). On comparing clinical characteristics between patients who did and did not have salivary drooling, a significant difference was found in Part III of UPDRS which was higher in patients with salivary drooling (p<0.01). Conclusion: Drooling was present in one third of our patients with PD, drooling is uncommon among patients with young onset PD, patients with salivary.


Prevalance of microembolic signals in acute stroke and correlation with the risk factors

S. Uppal, B. S. Paul, M. Singla, G. Singh, R. K. Bansal

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

Introduction: Trans cranial Doppler is an ultrasound guided diagnostic technique, which helps to detect the micro embolic signal in acute stroke patients, tells about the flow of blood through the vessels and the risk of recurrent stroke. Aims and Objectives: To study the incidence of Micro embolic Signals in Patients with acute anterior circulation stroke and its correlation with risk factors. Materials and Methods: Source of data, the study was conducted on indoor patients who were admitted with acute anterior circulation stroke in neurology department, DMC Ludhiana from May 2015-May 2016. All patients of acute stroke of anterior circulation diagnosed clinically and by ct head/Mri Brain above age of 18 were included and the monitoring was done for 30 -45 minutes in patient of acute stroke who has came in first 24 hours. Patients with inadequate temporal bone acoustic window, hemorrhagic stroke and posterior circulation infarct were excluded. Method of Collection of Data: Transcranial Doppler monitoring was performed with 2-MHz probe. The patients were examined in the supine or sitting position. The probes were fixed on the head with a specially designed head frame; this guaranteed a minimum of artifact disturbances and a constant angle of insonation. Results: In this study, 70 patients were enrolled and out of which 50 were male and 20 female. The maximum number of patients was of age group 60-70 years. The most of the patients were studied from 4-12 hours of onset of stroke. Out of 70 patients, MES were recorded in 25 patients (35.71%) and out of which 5 (20%) patients had restroke in 1 week of hospital stay. It was seen that MES were seen more in patients of atrial fibrillation, PFO, and in patients who had combined risk factors in form of smoking, diabetes and hypertension.


Manganism in patients with chronic renal failure on long term hemodialysis

D. R. Duberkar, R. Jawale, N. Aghor

Department of Neurology, Wockhardt Hospital, Nashik, Maharashtra, India

Porto-systemic encephalopathy secondary to chronic liver disease has manganese deposition in the basal ganglia. Similar basal ganglia deposition of manganese is seen in patients with chronic renal insufficiency who is on long term haemodialysis. We present 5 patients on haemodialysis who developed symptoms of managanism. 3 patients presented with symmetrical Parkinsonism and 2 patients presented with myoclonus. MRI brain disclosed bilaterally symmetrical T1 basal ganglia hyperintensity. Manganese levels were normal in 3 patients elevated in one patient and low in one patient. None of the patients received chelation. Patient had minor recovery in parkinsonian symptoms and myoclonus disappeared after intensification of haemodialysis. We must remember this entity in chronic dialysis patients who present with parkinsonian symptoms or severe myoclonus. This entity is not well described in literature and needs further studies for better elaboration of etiopathogenesis diagnosis and management.


Pattern and predictors of tremor in patients with primary dystonia

R. Rajan, S. M. Zafer, S. Krishnan, A. Kishore

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

Background: Tremor is now recognized to be a frequent accompaniment of dystonia. The pattern, location and predictors of tremor in patients with primary dystonia are not well established. Methods: We conducted a prospective, observational study including consecutive patients with primary dystonia attending the Movement Disorder clinic of our university hospital. Subjects underwent a detailed structured neurological examination to document the presence, distribution and clinical features of tremor and dystonia. Results: 129 subjects with primary dystonia were recruited including 24.0% with early onset dystonia. Tremor in at least one site was noted in 50.4%. Upper limb tremor was most common (41.1%) followed by head tremor (24.0%), voice tremor (7.0%), jaw tremor (3.1%), lower limb tremor (3.1%) and facial tremor (0.8%). Tremor and dystonia were in the same body part (dystonic tremor) in 78.5%, in separate regions (tremor associated with dystonia) in 4.6% and both of the above distributions were noted in 16.9%. Tremor preceded the onset of dystonia in 61.5%. Upper limb tremor was postural in 98.5%, at rest in 43.1% and kinetic in 40.0%. Null point was present in 40.0%. Subjects with tremor were older (p=0.028), had a longer duration of symptoms (p=0.008), more frequently had symptom onset in the upper limb (p=0.000) or non-cranial sites (p=0.000), were more likely to have cervical (p=0.022) or brachial (p=0.000) dystonia and a multifocal distribution (p=0.044), rather than upper facial (p=0.004), oromandibular (p=0.026) dystonia or a focal distribution (p=0.008). On multivariate analysis current age (OR=1.08 (95%CI 1.04-1.13), p=0.000) and onset at a non-cranial site (OR=12.42 (95%CI 1.05-146.49), p=0.045) were independent predictors of tremor. Conclusions: In this clinic based study, tremor was seen in upto one half of patients with primary dystonia. Advancing age and onset of dystonia at a non-cranial site were associated with tremor in this cohort.


Parinaud syndrome: Case series

E. G. Chelvakumar, V. Chandramouleeswaran, V. Ramakrishnan, S. Gobinathan

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

Introduction: Parinaud syndrome also known as dorsal midbrain syndrome is a supranuclear vertical gaze disturbance caused by compression of the tectal plate. It is characterized by a classic triad of findings. It consists of upward gaze palsy often manifesting as diplopia, pupillary light near dissociation and convergence retraction nystagmus. Patients also have eye lid retraction (Collier's sign). Its importance lies in that recognition of Parinaud syndrome localizes pathology to impingement of an origin in the tectal plate, most frequently due to a posterior commissure or pineal region mass. There is compression of the vertical gaze centre at the rostral interstitial nucleus of the medial longitudinal fasciculus (ri-MLF) at the level of superior colliculus. Recent case reports have been published stating that Parinaud syndrome can occur in thalamic lesions. We present two cases with Parinaud syndrome. The first patient had a midbrain glioma. The second patient had a right thalamic infarct. Case Vignette: Case 1 - 45 year old male presented with history of left sided weakness, double vision more for the distant vision, headache, slurring of speech, giddiness with swaying towards the left side. Clinical examination revealed dilated right pupil with sluggish reaction to light, upgaze restriction, convergence retraction nystagmus, left hemiparesis, stance and gait ataxia with swaying towards the left , left sided dysmetria and incoordination. Imaging revealed tumour in the dorsal mid brain with extension in to the right thalamus. Case 2: 48 year old male who is diabetic and hypertensive presented with acute onset of left hemisensory loss and left hemiparesis. Clinical examination revealed eye lid retraction, upgaze restriction with convergence retaction nystagmus. Imaging revealed right thalamic infarct with hemorrhagic transformation and bilateral subcortical infarcts imaging did not reveal any abnormality in the midbrain. Conclusion: Parinaud syndrome classically occurs in lesions involving the dorsal midbrain. We are presenting this case series to highlight the importance of lesions involving the thalamus presenting with Parinaud syndrome.

Poster Session 05; Epilepsy08:30-11:00 h, Saturday, November 12, 2016


Experience of levetiracetam versus carbamazepine as first add-on in drug resistant epilepsy patients in Eastern India

J. Mukherjee, S. P. Saha

Department of Neurology, Nil Ratan Sircar (NRS) Medical College, Kolkata, West Bengal, India

Introduction: High rise of drug resistant epilepsy cases (DRE) in India, a developing country, put the society in high burden of costly drugs for a prolonged period of time. We had compared the efficacy of costly drug levetiracetam with much cheaper carbamazepine, sometimes available free in Government hospitals, to find a cheaper but efficaceous alternative. Methods: In the intractable epilepsy clinic in a Medical College in Eastern India, all clinical and investigational details of DRE patients had been analysed and compared between levetiracetam and carbamazepine first add-on groups, with fisher's exact test. Results: No statistically significant difference had been found in the effects of Levetiracetam & Carbamazepine as first add-on drug in DRE patients. Conclusion: Though the number of cases were small in the study, a light of hope had came for poor DRE patients of India and abroad, as cheaper carbamazepine was no less efficaceaous to much costlier levetiracetam, setting a scope for future larger studies.


Clinical spectrum of siezures in infancy excluding neonatal siezures

V. Lakshmi

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

Aim: To study about clinical spectrum of Siezures in Infancy Excluding Neonatal Siezures - Prospective Study. Inclusion Criteria: Infants presented with Siezures. Exclusion Criteria: Neonates and Children more than 1 yr of age are excluded. Materials and Methods: Source of data: Patients presenting with Siezures to Osmania general hospital and Reffered from Niloufer Hospital-Hyderabad. Methods: A prospective study will be carried out in OGH Hyderabad (December 2014-January 2017). Approximately 50 patients will be included in the study and consent will be taken from them. Following information to be noted in semi structured Performa like presenting age, sex, symptoms relevant history, clinical examination findings. Basic and relevant investigations to be done. The data to be tabulated and analyzed.


Response to pulse steroids in Landau Kleffner syndrome

D. Nagabushana, J. B. Agadi, P. S. Kumar, A. B. Netto, K. Nagaraj, D. C. Janardhan

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

Introduction: The spectrum of various complications in critically ill Guillain Barre Syndrome (GBS) and its effect on the prognosis is lacking in literature. This study aimed at enumerating the complications in such a cohort and their significance in the prognosis and mortality. Materials and Methods : Retrospective case record analysis of all consecutive mechanically ventilated patients of GBS in neurology ICU of a tertiary care institute for 10 years was done. Demographic, laboratory, treatment details and outcome parameters were recorded. Results: Among the 173 patients were 118 men and 55 women (2.1:1), aged 1 to 84 years. The average number of ICU complications per patient was 6.8±1.8 (median =7, range = 1 to 12). The commonest complication was tracheobronchitis (128). Other pulmonary complications were found in 36 patients. The next was metabolic hyponatremia (115) hypokalemia (67), hypocalcemia (13), stress hyperglycemia (10), hyperkalemia (8), hypernatremia (9), Sepsis (40), Urinary tract Infections (UTI) (47), dysautonomia (27), hypoalbuminemia (76), anaemia (75), seizures (8), paralytic ileus (5), bleeding (4), anoxic encephalopathy (3), organ failures (12), deep vein thrombosis (7) and drug rashes (1) were also noted. The complications, considered significant in causing death, Hughes scale ≤3 at discharge, prolonged mechanical ventilation (>21 days) & hospitalization (>36 days) were pneumonia, hyponatremia, hypokalemia, urinary infection, tracheobronchial infections, hypoalbuminemia, sepsis, anemia dysautonomia. Conclusion: Active monitoring and appropriate and early intervention by the clinician will improve the quality of life of these patients and reduce the cost of prolonged mechanical ventilation and ICU stay.


Clinical relevance of headaches in epilepsy

P. R. Prakash, S. P. Turaga, A. Kumar, S. Kaul

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

Introduction: Epilepsy and headache are most common neurologic disorders affecting all ages worldwide. Interictal headache (IIH) is thought to be important co-morbidity to epilepsy in addition to psychiatric co morbidity. Perictal headache (PIH) is recently found to be of localising value especially for temporal lobe epilepsy. These headaches display burden to seizures Objective: To see the frequency and evaluate the potential predictors of Interictal and Perictal headache in patients with epilepsy(PWE) attending outpatient epilepsy clinic of NIMS. Materials and Methods: PIH and IIH are classified according to International Headache society II into migraine, tension type headache (TTH) and unclassified. All of them underwent interview by epileptologist regarding occurrence of headache and characteristics of each type of headache. Demographics of all PWE including their age of onset, duration of epilepsy, seizure types and epilepsy syndrome, seizure frequency, maximum remission period and current antiepileptic medication are assessed. Results: Out of 181 patients, headache is seen in 46.4% of epilepsy patients which is most common comorbidity. Interictal headache is seen in 25.96% of all (55.96% of headache) patients. In Interictal headache, migraine is seen in 38.5%, TTH is seen in 61.5% of all patients. Ictal headache was seen in 3.57% and has localising value in temporal lobe epilepsy. Postictal headache is seen in 16.57 %( 35 .7% of headache) of all mostly generalised/ secondarily generalised seizures. After univariate analysis, Female gender, type of epilepsy, and migraine has statistical significance for having interictal headache. Age of onset, migraine, type of epilepsy statistical significance for association with periictal headache. Conclusions: Postictal headache is most frequent, severe and underidentified form of Periictal headache. Low age of onset, generalised tonic clonic seizures are predictors for Periictal headache, commonest manifestation being migraine. Female gender, type of epilepsy (focal) has statistical significance for having Interictal headache.


Factors responsible for epilepsy treatment gap at a tertiary care hospital

M. V. Shah, S. Ravat, N. Jain, K. Panchal, A. Bhatti, S. Ravat

Department of Neurology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India

Introduction: Treatment gap or the percentage of people with active epilepsy not on treatment is a major concern especially in developing countries. Various factors responsible for treatment gap include those related to the patient (education, socioeconomic status, personal beliefs, etc.), the physician (misdiagnosis, negligence, unavailability) or treatment (high cost, adverse effects). We conducted this study to evaluate the incidence, demographic characteristics and determinants of treatment gap at tertiary care center and to assess healthcare-seeking behavior of patients. Materials and Methods: It was a cross-sectional observational study conducted at the outpatient epilepsy clinic at our center, after obtaining ethical clearance. 500 consecutive patients with active epilepsy attending our epilepsy clinic for the first time were included in the study after taking written informed consent. A self-structured goal directed questionnaire was administered to interview the patients. The treatment gap (TG) before the initiation of antiepileptics was taken as primary treatment TG and gap after initiation of antiepileptic as secondary TG. Data regarding type of disease, drug treatment and reasons for treatment gap was obtained and subsequently analyzed. Result: 265 of 500 (53%) epilepsy patients had a treatment gAndhra PradeshAmongst the factors responsible, patient related factors were most common (69.8%), which included socio-economic and educational factors, followed by physician related factors (21.57%) and treatment related factors (8.6%). Secondary (46.59%) TG was more common than primary TG (44.69%) and 9.09% patients had both gaps. Conclusion: The patient related factors were most commonly associated with treatment gap of which patient's beliefs, educational and financial statuses were important determinants. Appropriate counseling regarding nature of the disease, need for antiepileptic drug continuation and optimal prescription of AED based on affordability and availability may help to decrease secondary TG. Primary TG maybe decreased by increasing epilepsy awareness and by making appropriate healthcare available in all areas.


Wilson's disease presenting as a masquerade of progressive myoclonic epilepsy

M. Sachan, S. Kushwaha, V. Banga, A. Gupta

Department of Neurology, IHBAS, New Delhi, India

Introduction: Wilson's disease is an autosomal recessive hereditary disorder of copper metabolism which is characterised by deposition of copper in various tissues predominantly, liver and brain. Neurological involvement in Wilson's disease commonly presents as extrapyramidal, cerebellar and cerebral symptoms. Seizures have been reported in 6% of cases with myoclonic jerks being extremely rare. Myoclonic jerks as initial presentation has not been described till date. We report two families with Wilson's disease having myoclonic jerks along with cognitive decline as initial presentation masquerading as PME. Case Report: An 18 years old male, presented with 1 year history of generalised myoclonic jerks not sensitive to stimulus along with poor scholastic performance for 10 months followed by difficulty in speech and tremulousness of B/L upper limb since last 3 months. There was no history of dystonia, dysphagia, ataxia, jaundice, hematemesis, malena, blood transfusion or drug intake in past with normal developmental milestones. He has 8 siblings, amongst whom first 3 males died because of jaundice in their early childhood & the younger sister who also had history of myoclonic jerks with cognitive decline and other extrapyramidal symptoms. On examination, there were bilateral action tremors, dysarthria, spasticity & brisk reflexes along with B/L KF rings. His copper profile was suggestive of wilson's disease. Ultrasound abdomen shows coarse hepatic echo texture with splenomegaly. MRI Brain showed bilateral symmetrical T2 and FLAIR hyperintensity in basal ganglia and brain stem. Our second patient (14 year male) presented with almost similar complaints with positive family history. All the patients were managed conservatively and showed improvement. Conclusion: PME presents as myoclonic jerks with cognitive decline which was similar to presentation in our patients. Although a rare manifestation of Wilson's disease, Myoclonic jerks as a presenting manifestation should be kept in mind while evaluating for seizure disorder with extrapyramidal symtoms.


Eating epilepsy in North India: A case series and report of efficacy of clobazam in its management

P. Singh, A. R. Bansal

Department of Neurology, Institute of Neurosciences, Medanta-The Medicity Hospital, Gurgaon, Haryana, India

Background: Eating epilepsy is a form of reflex epilepsy where seizures are triggered by eating. It has been widely reported from southern part of Indian subcontinent. The cause of eating epilepsy is unknown. Pathophysiology proposed in this epilepsy is area of hyperexcitability at the perisylvian region including insular area of cortex irrespective of the side of involvement. It has been treated as focal epilepsy with polytherapy. Clobazam have been proposed in treating eating epilepsy as adjuvant therapy in very few studies. Objective: To determine seizure characteristics and effectiveness of clobazam in patients with eating epilepsy from north India. Methodology: All patients from January 2015 to June 2016 presenting to epilepsy clinic were evaluated. Those with eating epilepsy based on distinct history were noted. All relevant demographic parameters, seizure characteristics, investigation findings were recorded. Appropriate treatment along with clobazam trial was instituted to these patients and they were followed up till completion of study. Results: Eating epilepsy consisted of less than 1% (0.65 %, 10 in 1532) patients in our study. A male predominance of 9:1 was found. Complex partial seizure was the most common seizure type seen in 8 patients (80%). EEG and neuroimaging revealed predominantly perisylvian localization of eating epilepsy in 7 patients (70%). Addition of antiepileptic drug clobazam was found to be effective in reducing the frequency of seizures in 7 out of 9 patients (78%) who were already on polytherapy of antiepileptic drugs. Conclusions: We report a case series of 10 patients with eating epilepsy from north India. Clobazam taken ½ hour before meal was found be an effective adjuvant therapy in majority of these patients.


Clinical and radiological profile of post-stroke seizures

S. S. Kamble, V. Sardana

Department of Neurology, GMC, Kota, Rajasthan, India

Aims and Objectives: To determine the incidence of early vs. late post-stroke seizures. To study the clinical characteristics, risk factors for post-stroke seizures in relation with types, location and size of stroke with the help of neuroimaging. To study post-stroke seizure incidence in young vs old patients. Study Design: Observational study. Methodology: All patients presented with post-stroke seizures were included. Those with known epilepsy, sepsis, electrolyte imbalance and any other reversible precipitating factors were excluded. Age, co-morbid condition, details of seizures and radiological findings regarding type and location of stroke were collected. Association of variables was determined through chi-square test with significance at p < 0.05. Results: Out of 50 patients, 32 (64%) were males and 18 (36%) were females with mean age of 54.6 years including 9 (18%) young patients (<45 years). Thirty one (62%), 12 (24%), 14 (28%), 6 (12%) patients had hypertension, Diabetes, Ischemic Heart Disease and Dyslipidemia respectively. Twenty eight (56%) patients had early seizures (within 2 weeks) whereas 22 (44%) patients had late seizures (>2 weeks). Generalized seizures were seen most frequently i.e. in 37 (74%) patients, followed by partial motor with secondary generalization 9 (18%) and partial motor 4 (8%). Twenty nine (58%) patients experienced more than 2 seizures. Twenty nine (58%) patients had cortical lesion whereas 21 (42%) had subcortical lesions. Forty (80%) had an ischemic stroke including 37 (74%) arterial infarct and 3 (6%) venous infarcts. Intracerebral hematoma was seen in 10 (20%) of subjects. Comparison in early vs late onset seizures revealed significant association between IHD, hypertension and late onset seizures (p < 0.05). There was no significant difference in the occurrence of early compared to late onset seizures in young and old patients. Conclusion: Post-stroke seizures were more frequent in males, older age with history of hypertension, and with cortical ischemic strokes. Early seizures, multiple episodes and generalized seizure type were more common. History of IHD, hypertension showed strong relationship with the occurrence of late onset seizures.


A rare type of myoclonic epilepsy

S. S. Sankaralingam, P. Murugan, M. Radha, C. R. Packiaseeli, E. Bobby, T. E. Raj

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

A 15 years old girl presented with frequent spasmodic blinking of both eyes associated with upward deviation and absences lasting for 3 - 6 seconds which occured mostly in early morning and in sun. EEG showed brief generalised polyspikes. The disease responded to sodium valproate. Jeavon's syndrome is a rare idiopathic myoclonic epilepsy syndrome presenting with eyelid myoclonia. This myoclonus is photosensitive and is provoked by eye closure. It can be associated with or without absence seizure. Jeavon's syndrome being one of the distinctive epilepsy sundrome is often misdiagnosed as facial tics. The disease usually responds well to sodium valproate. The case is presented for its rarity.


Clinical phenotype of patients with juvenile myoclonic epilepsy and analysis of functional brain network using resting state functional MRI

E. R. Sunesh, M. Thomas, S. Yoganathan, Devakumar, S. V. Sudhakar

Department of Neurology, CMC, Vellore, Tamil Nadu, India

Aims: To study the clinical profile, neuropsychological profile and analysis of functional brain network using resting state functional MRI in patients with Juvenile myoclonic epilepsy. Primary Objective: To describe the clinical phenotype of patients with JME. Secondary Objectives: (a) To determine the cognitive profile of patients with JME. (b) To study the brain functional connectivity using resting state fMRI. Methods: It was a prospective descriptive study. All patients satisfying the inclusion criteria underwent detailed clinical examination, investigations and cognitive assessment by MOCA test. Ten randomly selected patients under went rsfMRI. Data was analysed and compared with age and sex matched healthy controls. Results: Forty one patients were enrolled in the study during the study period from November 2014 to March 2016. Mean age ± SD of study group was 18.14± 5.53 years. Sex ratio (F: M) in this study was1.05:1. Family history of JME was present in 4.87% of patients.. Myoclonic seizures were present in all patients and generalised tonic clonic seizure was the presenting seizure semiology in all JME patients. Mean age of onset ± SD of juvenile myoclonic epilepsy was 12.48 ± 3.59 years. Mean age at diagnosis ± SD was 15.29 ± 5.78 years. Mean delay in diagnosis ±SD in years was 2.85 ± 4.93 years. Precipitating factors for seizures identified were sleep deprivation, fever, watching television and menstruation. Seventy percent of patients were on monotherapy either with valproic acid (48.75%) or levetiracetam (19.51%). MOCA score revealed normal cognitive function in all patients. Normal EEG was observed in 15 patients, 23 patients had generalised paroxysmal bursts of spike and wave discharges of which 2 patients also had focal abnormality. Photo paroxysmal response was seen in two patients. Resting state fMRI of randomly selected 8 JME patients showed increased connectivity in different parts of the brain compared to the healthy controls.


Drug compliance (AEDs) study at Institute of Neurology in a tertiary care hospital

U. Vallatharasu, S. Gobinathan

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

Background: All the seizure patients attending to neurology op, inter-departmental referrals, cases near by other Government Hospitals in Tamil Nadu and also the neighbourhood states like Andhra Pradesh and Union Territory Pondicherry were all included in this study. Methods: All of them were examined proforma entry was made, excluding post traumatic, neuro surgical, neuro psychiatric disorders and paediatric age group. More than one year duration of AEDs up to 10 years was included. 3100 cases using 10 variables were analysed and the results of them were as follows. Results: Total number of patients not taken AEDs 3100; due to alcohol 595 (19.19%); native medicine 7 (0.22%); no awareness of AEDs 393 (12.67%); side effects from AEDs nil; out of station 829 (26.74%); pregnancy 28 (0.90%); forgotten to taken AEDs 561 (18.09%); death at home 112 (3.61%); missed to take AEDs due to college and school 438 (14.13%); missed to take AEDs due to tour 137 (4.42%). Conculsion: Addressing and advising to this common defective drug compliance with AEDs may definitely bring down the break through seizure percentage among the epilepsy and further this will help to bring down the incidence of recurrence and related morbidity.


Role of emotional perception and interpersonal behavior in detecting social cognition in patients with chronic temporal lobe epilepsy

P. K. Reddy, S. Turaga, S. Kaul, N. Hemalatha, D. Pabbu, Vijay

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

Background: In chronic temporal lobe epilepsy, it was found that there is impairment of social cognition in form of defective emotion perception with its effect on interpersonal relationship due to involvement of amygdala and structures in the anterior and medial temporal lobes. Compared to normal controls patients are less sensitive in recognition of fearful and disgusted facial expressions. There is need to explore in more detail the effects of these subtle recognition problems on daily social interaction. Aims and Objectives: Aim of this study was to see whether there is impairment of social cognition in the form of defect in perception of facial expressions and its effect on interpersonal behavior in chronic temporal lobe epilepsy patients. Materials and Methods: Consecutive 67 patients with chronic temporal lobe epilepsy and 33 controls who attended Nizam's Institute of medical sciences completed a forced-choice emotional recognition task, in which morphed facial emotional expressions were shown sequentially, and a self-report questionnaire of interpersonal behavior. Face perception and depressive symptoms were also taken into account. Results: Compared to normal controls, patients were sensitive in the recognition of fear and anger facial expressions, in line with previous reports. These impairments were not correlated with self-report interpersonal behavior. In all, unilateral damage to the amygdala and medial temporal lobe results in emotion recognition impairments, but these deficits does not appear to extend to self-reported impairments in everyday interpersonal behavior. Conclusions: Social cognition in form of defective emotional perception especially for fear and anger is noted in chronic temporal lobe epilepsy and it seems does not effect day to day life of them.


Evaluation of clinical and etiological features of epilepsy in elderly - A hospital based study

A. Verma

Department of Neurology, UP University of Medical Sciences, Uttar Pradesh, India

Purpose: To determine the clinical profile and etiology of epilepsy in elderly patients in rural population of India which is in a phase of demographic transition with steadily growing geriatric population. Methods: A cross sectional, hospital based clinicoepidemiological study was performed from October 2014 to November 2015. Patients who had experienced two or more unprovoked seizures after 60 years were included in the study. A total of 715 patients aged more than 60 years having different neurological disorders were evaluated in our hospital during this period. Out of this 258 patients had onset of epilepsy or epileptic seizure after sixties and above. We excluded the acute symptomatic epilepsy. One hundred and ten patients were enrolled and were divided into three standard subgroups: subgroup A (aged 60-70), subgroup B (aged 71-80), and subgroup C (aged 81-90). Results: Out of 110 patients 72 (65.45%) were male and 38 (34.54%) were female. The most common etiology was cerebrovasular disease (46.36%), followed by focal cerebral calcifications single or multiple (11.81%), tumors (9.09%), trauma (6.36%) dementias (6.36%) and unknown (16.63%). The most common type of seizure in the group studied was focal 59.09% followed by generalized seizures 37.27%. Hypertension was the most common co-morbidity found in 40 (36.36%) patients. Conclusions: Present study proposes that epilepsy in the elderly patients have etiological relationship with stroke, focal cerebral calcifications, tumors and dementias. CNS infections accounts for a significant number of cases of remote symptomatic epilepsy in elderly in our region where neurocysticercosis is endemic.


Perception of epilepsy stigma in school children and teachers awareness and attitude towards epilepsy - A South Indian cohort study

B. Ramavathu, Suryaprabha, Subashkaul, Hemalatha, Rajeswari, Vasavi

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

Aim and Objectives: To study the perception of epilepsy stigma in school children and the awareness and attitude of teachers towards epilepsy in Singareni colories, area of Telangana in South India. Materials and Methods: The stigma scale of epilepsy (SSE) was applied to school children and a standardised questionnaire was applied to teachers to assess the awareness and attitude towards epilepsy. A total of 508 school children and 30 teachers were enrolled in this study. The survey was performed on individual basis; an interviewer read the questions to the subject and wrote down the answers. The same procedure was used to all the subjects. Results: The overall mean score for epilepsy stigma perception was 54. The SSE score for women (55.8) was higher than that for men (51.5). Regarding awareness and attitude of teachers towards epilepsy, all respondents had heard of epilepsy although only 25% admitted to be well informed about epilepsy. 56% of respondents thought that epilepsy is a hereditary or contagious disease.73% objected to their children marrying a person with epilepsy and 23% respondents thought that epilepsy effected the education of a person. Conclusions: Perceived stigma was found to be a common problem in school children. Lack of awareness and negative attitude of teachers against people with epilepsy is likely to contribute to perceived feeling of stigma. The results reinforce the need for creating awareness among school children and teachers addressing misconceptions attached to epilepsy.


Clinical, electrographic and neuroimaging characteristics of a cohort of patients with drug-resistant epilepsies

A. Chatterjee, K. Radhakrishnan

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

Epilepsy is a disorder of the brain that is characterized not only by a lasting predisposition to generate seizures, but also by its neurological, cognitive, psychological, and social consequences. It has been estimated that about one-third of all patients who are diagnosed with epilepsy remain poorly controlled on antiepileptic drugs (AEDs). India, with over one billion people, has over one million people with poorly controlled epilepsies. International League against Epilepsy (ILAE) has proposed a consensus definition of drug-resistant epilepsy (DRE) as failure of adequate trials of two tolerated and appropriately chosen and used AED schedules either as monotherapy or used in combination to achieve seizure freedom for a minimum of 12 months or for a period three times the previous longest seizure-free period, whichever is longer. Some of the factors associated with refractory epilepsy are: neonatal seizures, mental subnormality, abnormal neurological signs, multiple seizure types, initial high seizure frequency, inadequate response to initial AED therapies, a grossly abnormal EEG and neuroimaging evidence of structural abnormality. However, in a significant proportion of patients with DRE, the cause of drug resistance remains elusive. This study was designed to identify factors which are associated with AED-resistance in a consecutively collected cohort of patients, so that, alternate treatments such as epilepsy surgery, ketogenic diet or vagus nerve stimulator can be considered earlier, rather than later. All consecutive patients who are diagnosed with DRE, attending the outpatient clinic of Department of Neurology, KMC Manipal, as per the ILAE definition will be included in the study. They will be screened on the basis of history to rule out pseudo-resistance. Those with truly AED-resistance will be included in the final analysis. Data collection will be done by means of a standard questionnaire. The study is currently ongoing and data is being collected and analyzed.


Association of GSTM1 and GSTT1 polymorphisms in patients with epilepsy in South Indian subjects

K. Rakesh, P. T. Surya, K. Kumaraswami, V. K. Kutala

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

Experimental studies suggest that oxidative stress is shown to be one of the contributing factors in the onset of epileptic seizures. Glutathione S-transferases (GSTs) are able to conjugate electrophilic compounds and thus possess neuroprotective role by removing exogenous and endogenous oxidants, detoxifying therapeutic drugs, environmental toxins through conjugation with glutathione (GSH). Several studies from different ethnic groups showed that the polymorphisms of the GST gene have been associated with Epilepsy. In the present study, we aimed to investigate the association of GST polymorphism in patients with epilepsy in South Indian subjects. A total 371 samples (110 cases and 261 controls) were genotyped for the GST1 and GSTM1 polymorphisms by the multiplex PCR method. We found a significant association of genotype and allele frequencies with GSTT1 null polymorphism in patients with epilepsy. The frequency of the GSTT1 null variant was found to be significantly higher in cases (35.45%) than controls (18.39%) (OR: 2.44; 95%CI: 1.4- 4.02 p<0.0001). In contrast, the frequency of the GSTM1 null variant was significantly lower in cases (11.81%) than controls (32.95%) (OR: 0.27, 95%CI: 0.14-0.51) indicating a protective role. These results indicated that individuals who have GSTT1 null variant are at higher risk for developing seizure than those who are positive genotyped for GSTT1 gene. On the other hand, individuals carrying GSTM1 null variant showed protective role against seizure. Further, these two null variants did not show any significant association with anti-epileptic drug-induced skin rash.


Role of neuroimaging in first unprovoked seizure

N. Kumar, G. Gupta, A. Verma, M. Singh

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

Objectives: The primary goal of neuroimaging in a first unprovoked seizure (FUS) is to identify a lesion that can explain the seizure and estimate the risk of epilepsy. This study aims to determine the diagnostic and prognostic role of MRI when CT is normal and to identify potential clues that predict positive MRI findings. Methods: This was a prospective study of 202 patients with FUS from January 2015 to March 2016. All patients clinical, imaging, and EEG findings were prospectively recorded into a database. Patients underwent CT imaging initially and a proportion of them went on to have MRI after further assessment. The reports were recorded as being normal or having potentially epileptogenic findings (PEFs). Patients were reviewed at the clinic 3 to 9 months after FUS regularly. All patients with symptomatic seizure due to neurocysticercosis were excluded. Results: CT brain was performed in 170 and 152 patients had an MRI brain. Overall, 60 patients (30%) had PEFs on neuroimaging, most commonly stroke, post-traumatic, or neoplastic lesions. Out of 120 patients with a normal CT and subsequent MRI, 14 (12%) were identified to have a PEFs on MRI. The pathologies most likely to be evident on MRI but not evident on CT were MTS, malformations of cortical development, and cavernomas. When CT was normal, the strongest predictor of an epileptogenic lesion on MRI was the presence of focal slowing or epileptiform abnormality on EEG (OR 21; 95% CI 4.5-97.7, p< 0.0001). Conclusions: MRI is clearly superior to CT in detection of PEFs in patients with FUS. EEG predicted an epileptogenic lesion identified by MRI when CT was normal. Our findings suggest that if MRI is readily available then it can replace CT in most patients with FUS and should be the initial diagnostic and prognostic modality of choice.


Seizures in women during postpartum period: A hospital based study

K. Manish, S. Harsha, B. S. Keshava, Ambarish Bhandiwad

Department of Neurology, JSS Medical College, Mysore, Karnataka, India

All patients who were admitted in our hospital with history of seizures in the postpartum period (within 6 weeks after delivery) were included in the study to ascertain the cause of seizures and there outcome, and women with prior history of epilepsy were excluded from this study. Among 87 patients recruited, primipara women (42 patients) had high risk of seizures. Women between the age group of 21-25 years constituted a large number of patients in this study (44.8%). Seizure occurrence was more on the first day following delivery (69%). Imaging of the patients showed posterior reversible encephalopathy syndrome (PRES) as the most common cause of seizure (34.5%), second most common cause was cerebral venous thrombosis (CVT) (31%). Eclampsia was equally common as CVT (31%). 2 patients had intracerebral hemorrhage (4.5%). Eclampsia patients had normal imaging with high blood pressure, high (+++) urine albumin and deranged hematological values. Women with PRES and eclampsia had good outcome with no mortality. While patients of CVT had a mortality of 30% in our study. Conclusion: In this hospital based study, pres was the most common cause of seizure in postpartum women (34.5%), followed by cvt and eclampsia (each 31%). Patients of pres and eclampsia had good outcome and patients of cvt had higher mortality. The other aspects of the results shall be elaborated.


Clinical features and outcome of autoimmune epilepsies

G. Ravindar, S. Jayalakhshmi, P. K. Yada, E. A. Varalakhshmi, S. Mohandas

Krishna Institute of Medical Sciences Ltd., Secundrabad, Telangana, India

Aim: To evaluate clinical characteristics and outcome of patients with autoimmune epilepsy. Methods: A retrospective analysis of 26 cases with autoimmune epilepsy was performed. The patients were grouped as auto antibody positive and negative (presumed) autoimmune epilepsy. The predictors of favourable outcome at 1 year were assessed. Results: There were 26 patients; 20 were females. All presented with seizures. Associated significant memory disturbances were noted 9, psychiatric and behavioural problems in 8, while 5 patients presented with refractory status epilepticus, 3 with non-convulsive status; one had epilepsia partialis continua while 2 patients had ataxia. The mean age of patients was 33.4 years; Autoimmune antibodies were detected in 11 patients (VGKC -3; NMDAR-3; anti-thyroid-3; anti GAD-1; both VGKC and NMDAR-1). The MRI brain was abnormal in 16; and showed cortical and subcortical hyperintensities -10, bitemporal hyperintensities -5 and hippocampal sclerosis-1. FDGPET done in 9 showed typical features of autoimmune encephalitis in 4 while 5 patients had bi-temporal/focal cortical hypo metabolism. All the patients received immunomodulation therapy with steroids, while 8 patients received IVIG and rituximab was given to 11 patients as 2 nd line therapy. At 1 year follow up, 15 patients were seizure free while 11 had persistent seizures. Predictors of favourable outcome (seizure free with normal cognition and behaviour) at 1 year were normal MRI, early treatment while status epilepticus was a negative predictor. Conclusion: Autoimmune epilepsy was more frequent in women. Normal MRI and early treatment were predictors of favourable outcome while status epilepticus was negative predictor.


Prevalence of migraine in epilepsy

A. Mayyar, G. Singh, R. Bansal, M. Singla

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

Introduction: Headache is commonly associated with seizures as an inter-ictal, preictal, ictal, or postictal phenomenon, and often neglected because of the dramatic neurological manifestations of the seizure. A prospective study was designed to identify epidemiological association between headache and epilepsy and to evaluate the types and frequency of seizure-associated headaches in patients with epilepsy on anti-epileptics. Aims and Objectives: To evaluate the type and frequency of headache in people with epilepsy and risk factors with special attention to the anatomic localization of the epileptogenic focus, seizure classification (focal versus generalized) while on antiepileptic drugs over a period of 3 months. Study Design: Study conducted at Outdoor Patient Department, of Department of Neurology, DMC&H, and Ludhiana. Patients ≥17 years with a diagnosis of epilepsy more than 2 years on single antiepileptic agent - with new onset headache, increase in frequency and severity of headache in epilepsy were taken. Headache data analyzed according to IHS criteria, Epilepsy classified according to the 2010 (ILAE) Commission report. Results: 73 cases (29 female, males 44) on antiepileptic monotherapy were taken. Mean age was 26 years, median age at epilepsy onset was 15 years, median epilepsy duration was 8 years. Generalized epilepsy seen in 48 (66%), focal epilepsy 25 (33%). 23 (30%) reported headache, inter-IH in 15 (40%) and peri- IH occurred in 12 ( 50%) of these 5 (18.3 %) had an associated inter-IH, 7 had headache related to the seizures, Carbamazepine, levetricetam, valproate, oxcarbamazepine, lamictal given in 17, 18, 29, 6, 2, patients 7, 5, 9, 1, 1 had headache. This is an ongoing study till sept 2016. Conclusion: carbamazepine and levetricetam are associated with headache more frequently.

EP21/ Withdrawn


Clinical, electroencephalographic and imaging correlation of seizures and epilepsy

L. Rajanala, N. V. Sundarachary, U. Veeramma, D. Rajeswari

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

Background: Despite a high prevalence, vast research and technological advancement including availability of genetic studies, epilepsy in India is either under diagnosed, inadequately evaluated and inappropriately managed. One important cause for this discrepancy is the lack of syndromic approach whichrequires the classification as determined by the seizure semiology, electroencephalographic pattern of abnormalities and imaging findings coupled with associated motor and cognitive dysfunction if any. Aims and Objectives: (1) To evaluate patients with seizures and epilepsy for their Demographic profiles, seizure semiology and clinical features. (2) To Study their Electroencephalographic (EEG) findings, and (3) To correlate the clinical and EEG findings with imaging findings. Materials and Methods: This is a prospective and Retrospective study at a government general hospital. Observations: (1) Total sample size: 1000. (2)There were 53% Male, 47% female in the total sample; 56% Male and 44% female among adults while in children , there was a slight female preponderance with 57% Vs 43 % Male. (3) The age of onset of first seizures was the single most important overriding factor in organizing patients with epilepsy. (4) Greater than 90% of our subjects could be placed into a diagnostic category by seizure semiology alone and after EEG another 1% could be classified. (5) The role of imaging was mainly to establish and confirm the clinical diagnosis and a mere 0.6% could be classified after imaging. Conclusion: In a culturally diverse, socially stigmatized but economically growing nation like India, the need to change our approach towards epilepsy can not be overemphasized. Also required is the research in this field and bringing together scientists from diverse fields onto the same platform to discuss the contemporary issues and controversies.


A comparative blinded study of cognitive effects of carbamazepine, valproate, lamotrigine and levetiracetam in adult epileptic patients

M. Goyal, V. Lal, R. Sandal, M. Modi, J. Sachdeva, P. Kharbanda

Department of Neurology, PGIMER, Chandigarh, India

Introduction: Optic perineuritis is an uncommon inflammatory disorder involving the optic nerve sheath. Most cases are isolated and idiopathic, but may occasionally occur as a manifestation of a specific infectious or inflammatory disorder, such as syphilis, Wegener granulomatosis or giant cell arteritis. Objectives: To present three cases of optic perineuritis with varied clinical presentations. Cases: (1) 19-year-old boy presented with right hemicranial headache, swelling over right eye and periorbital area for 2 months. Clinical examination revealed decreased visual acuity and disc edema in right eye. (2) 12-year-old girl presented with complaints of progressively increasing redness and swelling of right eye for 10 days, associated with diminution of vision and headache on the same side. She revealed history of two episodes in past with sequential involvement of one eye followed by the other, 2 years back. Optic fundus examination showed right disc edema with dilated tortuous vessels. (3) A boy in his teens presented with loss of vision in left eye for one day. He had vesicular lesions on left side of forehead and upper eyelid (suggestive of herpes zoster infection), which erupted 10 days ago. Left eye had hand motions vision, intraocular pressure 10 mm Hg, a relative afferent pupillary defect, optic disc edema, dilated and tortuous veins, and multiple retinal hemorrhages. Fluorescein angiography revealed extremely sluggish blood flow in the arteries, delayed venous filling and frosted branch angiitis in the posterior pole. MR imaging of orbits clinched the diagnosis in of optic perineuritis in all these cases. Conclusion: These case reports highlight optic perineuritis and stress the fact that all disc edemas with diminution of vision are not just optic neuritis. The sheath has to shoulder the blame too.


Incidence and classification (2010) of new-onset unprovoked seizures and epilepsy and epilepsy syndromes in a cohort of children accessing government primary schools: A study in south India

S. K. Jaiswal, J. M. K. Murthy, P. M. Reddy, S. Sri Krishna

The Institute of Neurological Sciences, CARE Hospital, Hyderabad, Telangana, India

Background: The incidence of unprovoked seizure and epilepsy in children has not been well studied in developing countries, hence this study. Materials and Methods: Study cohort included 7408 children accessing education in 18 government primary schools in Hyderabad, Telengana. The children registered on rolls as on January 1, 2006 were followed through first to fifth standard for new-onset seizure between January 1, 2006 to December 31, 2012. The data collected included demographic data, seizure semilogy, date of seizure onset, neurologic findings, CT and EEG findings, antiepileptic drugs, and follow-up data. The recent definition of epilepsy and system for organization of seizures and epilepsies by ILAE were used to organize the seizure disorders due to NCC. Results: During study period, 37 children had new-onset unprovoked seizure, mean annual incidence 5.28. The common seizure type was focal and motor. Of the 37 children with unprovoked seizures, 30 (80%) children fulfilled new definition of epilepsy. The annual incidence unprovoked seizures was 71.35 (95%CI 71.28-71.42) per 100,000 and epilepsy 57.85 (95%CI 57.79-57.92) per 100,000. Using new organization system epilepsies were organized into (1) electro-clinical syndromes (genetic): 11 (36.6%), annual incidence 21.21 (95% CI 21.17-21.25) per 100,000 (2) non-syndromic epilepsy due to structural lesion: 9 (30%), annual incidence 17.36 (95%CI 17.32-17.39) per 100,000 and (3) non-syndromic epilepsies of unknown cause: 10 (33.3%), annual incidence 36.64 (95% CI 36.59-36.69) per 100,000. The annual incidence of epilepsy due to single NCC was 9.64 (95% CI 9.62-9.67) per 100,000. Conclusions: This is probably first incidence study of epilepsy that used the new definition of epilepsy and 2010 system for organization of seizures and epilepsy. This study suggests that incidence of epilepsy and frequency of various epilepsies are similar to developed countries and also suggests that with seizure semiology, EEG, and contrast-CT most of the unprovoked seizures can be classified.


Pilot study of concomitant use of metformin in patients on sodium valproate with PCOD like symptoms

S. Dwivedee, K. Dwivedee, N. Kaul

Department of Neurology, FMRI, Gurgaon, Haryana, India

25 female full filling the criteria of PCOD and safety criteria for use of metformin as a concomitant medication if it is considered essential by the investigator to give sodium valproate for seizure control and who were seizure free for more than one year on sodium valproate with no pre treatment history of PCOD. PCOSQ scale was used to assess the severity of PCOD at baseline and at 3 and 6 months after the introduction of metformin with safety visits in between this period. More than 80 percent subjects showed improvement in PCOSQ scores and CGI scales. There were 3 patients who had adverse events, severe enough for a drop out from metformin usage. The pilot study suggestes further trials to use metformin in difficult situations where use of sodium valproate is unavoidable.


Clinicoetiological correlation of new onset seizures in older population

T. N. Dubey, R. Saxena, P. Gupta, P. Kalantri, R. Gayakwad

Department of Medicine, GMC, Bhopal, Madhya Pradesh, India

Acute seizure, epilepsy and status epilepticus have the highest incidence in those over the age of 60. Clinical profile and etiology of new onset seizures in old age are obscure. This study was undertaken to observe clinico- etiological correlation of the new onset seizures in patients above 60 years of age admitted at a tertiary care centre of Bhopal. Materials and Methods: Fifty patients above 60 years of age with new onset seizures were included in the study. All patients underwent renal functions, liver function and glycemic status. EEG and CT scan head (or MRI brain) were done. Lumbar puncture and serological tests were done in patients as indicated. Observation: Mean age of patients in the study was 68.8 ±6.1 years. Male to female ratio was 2:1.32% of study participants were hypertensive, 20% were diabetic and 10% were both hypertensive and diabetic. 24% patients were smoker. Stroke was the most common (46%) etiology among the study patients studied with predominance of ischaemia (38%) than haemorrhage (8%). Metabolic causes (alcohol withdrawl and hypoglycemia) constituted 16% of study patients. Others causes observed were CNS infections, space occupying lesions and gliosis. Cause was not found in 28% of cases. The genderwise distribution of causes didn't differ in all age groups. In this study stroke was more common cause of seizure in patients with hypertension (55% vs 40%) and diabetes (54% vs 43%) compared to nonhypertensives and nondiabetics respectively. GTCS was the most common seizure type (70%) followed by focal with secondary generalization (26%) and simple partial seizure (4%). Conclusion: Overall stroke is the commonest cause of new onset seizures (GTCS as well as focal) in the study participants irrespective of gender. Metabolic derangement is second most common cause of seizure in study participants with all cases having generalized seizure in this group. Focal seizures with or without secondary generalization were observed in patients with SOL and gliosis.

EP27/331. Withdrawn


Targeting elevated glutamate in tumour associated seizures: Ex vivo studies in human peritumoural neocortex

M. Cunningham, K. Chatterjee, V. Goka, D. Pradhan, H. Kumar, A. Datta

Department of Neurology, Institute of Neuroscience, Newcastle University, Newcastle upon Tyne, United Kingdom

Multiple lines of evidence support the notion that seizures in glial tumours stem from increased extracellular glutamate release from over expression of a cysteine/glutamate transporter (SLC7A11), system xc-, in the peritumoural region. We demonstrate that the intrinsic seizure generating potential of peritumoural tissue derived from patients undergoing neurosurgery for the removal of glioma is dependent on system xc-. Spontaneous inter-ictal discharges were significant reduced by sulfasalazine over a concentration range consistent with the drugs antagonistic effect on system xc-. In contrast, in slices that were not spontaneous active, inter-ictal discharges induced using a proconvulsant media, were significantly reduced at concentrations at which sulfasalazine is known to block N-methyl-D-aspartate (NMDA) receptors. In spontaneously active slices, a proconvulsant stimulus was capable of inducing ictal-like activity which was subsequently abolished by sulfasalazine. Ictal activity could not be induced in the non-spontaneously active tissue. Given that elevated glutamate would appear to play a role in epileptogenesis, we tested the efficacy of an orally active AMPA receptor antagonist, perampanel in human peritumoural neocortex. This novel antiepileptic drug was capable of significantly reducing spontaneous IIDs and induced ictal activity. In addition, we have demonstrated that perampanel was more effective than the commonly used antiepileptic drug, levetiracetam, in suppressing peritumoural spontaneous IIDs and ictal activities. These findings demonstrate, for the first time in human peritumoural tissue, that System xc- contributes to the intrinsic seizure generating potential of this neocortical tissue. Moreover, the presence of elevated extracellular glutamate in seizure generating peritumoural tissue provides mechanistic data that supports the use of perampanel in Brain Tumour Related Epilepsy (BTE) in glioma patients.


Clinical profile of acute symptomatic seizures: A retrospective study

G. L. Rynjah, G. L. Rynjah, M. Alexander

Department of Neurology, CMC, Vellore, Tamil Nadu, India

Introduction: Acute symptomatic seizures are events, occurring in close temporal relationship with an acute CNS insult, which may be metabolic, toxic, structural, infectious, or due to inflammation. In this series, we describe the etiologies, semiology and clinical characteristics of patients presenting with acute symptomatic seizures to a tertiary centre over a period of two years. Methods: Patients were identified from discharge summaries database with key words of symptomatic seizures and symptomatic epilepsy from January 2014 to March 2016. Results: A total of 165 patients were included in the study with 91 (55.2%) males, 26% (43/165) were aged 15 years or younger with second peak in age 25-50 years 42.4% (70/165). The mean age of patients presenting was 30.02 (±19) years. A total of 74 patints (44.8%) presented in Status Epilepticus requiring ICU care. The main etiologies identified were Vascular (including arterial and venous strokes and vascular malformations) in 33 (20%) patients, neurocysticercosis in 31 (18%), autoimmune epilepsies in 21 (13%), post traumatic in 10 (6%) and tubercular in 8 (5%). The majority of patients presented with generalized tonic clonic seizures with 16% presented with focal motor seizures, 2 with focal sensory seizures, 2 patients with multiple semiologies and 3 patients with myoclonic status. Imaging was abnormal in 87% of patients (145/165). 64% (104/165) required more than 1 antiepileptic drug for control of seizures at discharge. Conclusions: The majority of patients with symptomatic seizures presents with generalized seizures and require more than one antiepileptic drug for seizure control. Vascular causes, including venous and arterial strokes account for the maximum number of symptomatic cases.

Poster Session 06; Stroke; 11:00-14:00 h, Saturday, November 12, 2016


Acute deep cortical vein thrombosis: The varicella auto antibody syndrome

S. Koshy, A. Ninan, K. Ramadoss

Department of Neurology, P. S. G Hospitals, Coimbatore, Tamil Nadu, India

Cortical Venous Thrombosis (CVT) is a rare entity found in association with Varicella Zoster Infection. Isolated Deep Cortical Vein thrombosis is having poor prognosis due to bilateral/unilateral thalamic involvement with pyramidal tract involvement. The Patient may present with acute onset of rapidly progressing altered sensorium with stroke like presentation and signs of raised intra cranial tension. Evaluating secondary causes for CVT is essential for plan of long term management and outcome. Varicella Zoster infection is common in childhood; the incidence has come down due to effective vaccination. Central nervous system involvement in the form of encecephalopathy, headache and vomiting, clinician should have strong suspicion of Cortico venous sinus thrombosis, even for radiologically negative thrombosis. Transient anti phospholipid and coagulation protein antibodies were common with Varticella Zoster Viral infection but were not predictive of thrombotic complication. We present a case of young female who had presented with Deep Cortical Venous Thrombosis (DCVT) following a Varicella Zoster Infection.


Comparison between APACHE II and ICH score in predicting 30 days' mortality outcome in hemorrhagic stroke patients

K. Pan, T. N. Kundu, A. Pandit, B. Bhattacharyya, B. Das, A. Ghosh

Department of Neurology, Bangur Institute of Neurosciences, Institute of Post-Graduate Medical Education and Research, Kolkata, West Bengal, India

Background and Aims: In these study five Parkinson patients with unusual visual hallucination, the associated behavioral disturbance and the management issues are analyzed. Methods: All the five patients in this case study have Parkinson disease. The main problem is troublesome visual hallucinations and the behavioral disturbance. Results: Case 1- Eighty five year old male patient with Parkinson disease, complaint of seeing spiders all around the house. He moves with a basket and catch the spiders which are not there, go outside and throw it. Case 2 - Seventy year old male patient with Parkinson disease started seeing snakes everywhere, due to which he tries to run away from the house. Case 3 - Seventy two year old female patient with Parkinson disease noticed the people in the photographs on the wall are coming out of the photograph and talking to her. Case 4 - Seventy five year old male patient with Parkinson disease started seeing eagles and he is able to describe the sound it makes. Case 1- 4 patients responded by reducing the dose of L dopa and adding rivastigmine. Case 5 - Sixty year old gentleman with Parkinson disease started feeling patches of mites all over his body. He takes frequent bath for it. He became better with a small dose of antipsychotic. Conclusion: Parkinson disease patient with behavioral disease need extra time in a busy clinic, because it may be due to the visual hallucination. By adjusting the medications, adding appropriate medicines and by counseling a significant difference can be made to the patients and care takers quality of life.


A rare case of ischemic stroke

N. Sunkarineni, S. Jawalkar, N. Aparna, S. M. Kumar, M. Zoheb

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

Introduction: The physiology of respiration is modulated by autonomic efferent neurons and circulating hormones. A continuous ambulatory monitoring of various respiratory parameters helps in early identification of sympatho - vagal dysregulation. Objective: To compare respiratory patterns in normal individuals, individuals with anxiety spectrum disorders & in regular meditators. To study the effect of meditation on respiration. Materials and Methods: Prospective cross sectional study which included 52 subjects - normal (20 nos), anxiety (16 nos.), meditation (16 nos.) was conducted at Neurology department, Amrita Institute of Medical Science (AIMS), Kochi. A 24 hrs ambulatory monitoring was carried out in each subject during the sleep - wake cycle & phases of activities by Embletta MPR & XactTrace belts using the respiratory inductance plythesmography (RIP). Respiratory parameters like Respiratory rate (RR), Respiratory rate variability (RRV) & Thoracoabdominal ratio (%RC) were assesed. Results: Mean RR in sleep in normal, meditation, anxiety groups were 18.22 bpm, 13.21 bpm, 19.37 bpm respectively. Meditation group had significantly lower RR than normal, anxiety group (p≤0.001& p≤0.001). At awake rest state, meditation group had significantly lower RR than anxiety (p=0.001). Mean RRV during sleep in normal, meditation, anxiety groups were 36.34%, 43.60% and 23.51% respectively. The study showed subjects in meditation group showed higher respiratory rate variability than normal & anxiety groups (p=0.013 & <0.001 respectively) in sleep & significant higher values than anxiety group during awake rest (p≤0.001). In anxiety group, mean RRV% increased, when physical activities increased & the difference during high activity from no activity state was statistically significant (p=0.005). Mean % RC during sleep in normal, meditation, anxiety groups were 54.18%, 47.56% and 55.86% respectively. During awake no activity state, meditation group (55.53%) had significant lower %RC than anxiety (62.49%) (p=0.048). Thoraco-abdominal ratio was lowest in meditation group & highest in anxiety group throughout sleep-wake cycle. Conclusion: Our study showed the meditation group had greater vagal emphasis which was reflected in their respiratory patterns with lower respiratory rate, greater respiratory rate variability, deep effective abdominal breathing. In converse, the anxiety group had higher respiratory rate, lower respiratory rate variability, rapid shallow thoracic breathing. RRV & %RC were considered as surrogate markers for autonomic coherence. Our study showed importance of continous ambulatory respiratory monitoring in detecting symptho - vagal influence in respiration and use of meditation in regulating and modulating their synchrony. This study paves way to a new branch -'Neurorespiratology', understanding the Science of Coherent Breathing & importance of sympatho-vagal synchrony in respiration.


Protean manifestations of racemose neurocysticercosis - Stroke

M. Sankaranarayanan, M. B. Singh, Neha, Elavarasi, L. Joseph

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

Neurocysticercosis (NCC) is a central nervous system parasitic infestation with protean clinical presentations, rarely manifesting as an acute stroke. Early diagnosis requires a high index of suspicion. Hereby we report a case of Cisternalneurocysticercosis presented with acute large vessel ischemic stroke. Our case highlights the importance of considering NCC in the differential diagnosis of stroke in patients coming from endemic regions, especially in younger patients lacking the usual risk factors for cerebrovascular disease. Case Report: A 16-yr-old young man presented with recurrent left focal seizures and sudden onset left hemiplegia with altered sensorium. At admission the patient was drowsy with a GCS of 9. All peripheral pulses were palpable, heart and chest was normal on auscultation. He had B/L papilledema, left facial weakness and left hemiplegia without any signs of meningeal irritation. MRI brain showed multiple cystic appearing lesions of variable sizes in the basal cisterns, bilateral sylvian fissures and in the anterior interhemispheric fissure without any contrast enhancement of the lesions. The supraventricular system was dilated and a right MCA infarct was seen. MRA showed an abrupt cut-off at the level of the M1 segment of the right MCA. Contrast enhanced CT Chest and abdomen was normal. HIV, HBsAg, anti HCV, VDRL were negative. CSF was acellular with normal protein, glucose and cryptococcal antigens, India ink, gram stain, AFB, bacterial culture, Gene Xpert TB were all negative. Serum ELISA for anti cysticercalIgG antibodies was positive. The final possibility that remained viable after all investigations was cisternalracemoseneurocysticercosis complicated by a vasculitic infarct. He was managed conservatively and given antiedema measures, antiepileptic drugs, and steroids. Cysticidal therapy was not given due to the high risk of ependymitis/encepahalitis. His consciousness and papilledema improved and he started communicating verbally with subtle improvement of hemiplegia.


Awareness and recognition of ischemic stroke in young adults

S. Mohan, V. Sardana, D. Maheshwari, B. Bhushan, P. Moo

Department of Neurology, GMC Kota, Mumbai, Maharashtra, India

Stroke is a leading cause of death and disability. The awareness of stroke symptoms remains poor amongst the general population. Approximately 15% of all ischemic strokes occur in young adults. Only limited prior public health and research efforts have specifically addressed stroke in the young. Early diagnosis remains challenging because of the lack of awareness and the relative infrequency of stroke compared with stroke mimics. The causes of ischemic stroke in the young are heterogeneous and relatively uncommon. The study was conducted to assess the awareness of warning symptoms and risk factors of stroke amongst young population. Materials and Methods: A hospital based survey was conducted. The study subjects (N=100) were divided into young patients presenting as acute ischemic stroke (n =42) or their Relatives (n=28) less than 45 yrs of age. The control group (n=30) had normal individuals above 45 yrs of age. The subjects were interviewed regarding various warning symptoms, risk Factors, etiologies for young stroke and Treatment options available including thrombolysis. Results: Out of the 42 patients of young ischemic stroke, 20 (48%) patients were aware of the most common symptoms of stroke (sudden weakness of limbs or face). Only 19 patients (45%) could identify the risk factors for stroke. The risk factor most commonly identified was hypertension diabetes and dyslipidemia. The subjects had very poor knowledge of Cardioembolic stroke and other causes of Stroke in Young. Knowledge of Risk factors was significantly more in the literate (59%) than illiterate (32%) population. Only 23 patients (55%) recognized Brain as the organ for stroke. Only 7 subjects and 3 controls (older than 45 yrs) had knowledge that stroke can occur in Young. Younger patients were less likely to seek medical care and even after arrival at the Primary care it was more likely to be missed, as Stroke is considered to be a disease of the elderly. Awareness about thrombolysis remains poor in the young (8%) as well as Elderly population (6%). There was no significant difference in the awareness in patients or relatives (p>0.05). Conclusion: The Knowledge about stroke symptoms and risk factors was better in the literate, Urban and those with higher income. Younger patients having more diverse etiologies and lack of traditional risk factors are more likely to be misdiagnosed. It is recommended to develop health education programmes to improve the awareness of stroke.


Anemia as a risk factor for acute ischemic stroke and its association with stroke severity and outcome: A prospective case control study

S. Sharma, D. Chowdhury, B. Singh, G. Khwaja

Department of Neurology, Govind Ballabh Pant Institute of Post Graduate Medical Education and Research, Puducherry, India

Introduction: Anemia is an independent risk factor and predictor of in-hospital mortality in cardiovascular diseases. Its role in Acute Ischemic Stroke (AIS) remains uncertain. Methods: A prospective case control study on 200 AIS and equal number of age and sex matched healthy controls was done. Anemia diagnosed by WHO Criteria ((Haemoglobin (Hb) <12 g/dl (women) and <13 g/dl (men)) and graded into mild (11-11.9 g/dl (women) and 11-12.9 g/dl (men)), moderate (8-10.9 g/dl) and severe (<8 g/dl). Stroke severity assessed by NIHSS and log DWI volume; outcome by modified Rankin scale (mRS), Barthel index (BI), mortality and recurrence at 3 months. Statistical analysis done using Epi-info 7.0. Results: Demographics -145 males and 55 female AIS and 144 males and 56 female healthy controls studied. Anemia: Although overall, anemia occurred equally in the 2 groups (37% vs. 35%; p=0.754), moderate (33.78% vs 4%; p=0.003) and severe anemia (5.4% vs 0%) was significantly more common amongst AIS. Anemia was more common amongst female AIS compared to controls (60% vs 37.5%;p=0.02). Severity and Outcome: Stroke was significantly more severe in anemic compared to non anemic AIS (mean NIHSS-10.4±7.3 vs 7.9±6.6; p=0.011 and log DWI volume-6.4 ± 28.6 vs 2.7 ± 8.3; p=0.035). 11.5% of the cases died at 3 months; mortality in female AIS with anemia was significantly higher (24.24% vs 0%; p=0.035). Five out of 6 AIS belonging to the lowest Hb quartile died. Conclusion: All grades of anemia but only moderate to severe anemia is a risk factor for AIS in females and males respectively. Anemia at admission predicts more severe stroke and worse outcome in AIS patients.


A prospective study of clinical features and etiological factors of cerebral venous thrombosis in a tertiary care hospital in Eastern India

S. Ghosh, K. R. Manoj, Mahata, K. C. Ghosh, R. Bhattacharyya, G. P. Mondal

Department of Neuromedicine, Calcutta national Medical College, Kolkata, West Bengal, India

Background: Cerebral venous thrombosis (CVT) is an uncommon cause of stroke with extremely varied clinical presentations, predisposing factors, imaging findings, and outcomes. We conducted a prospective study of 50 patients of CVT admitted in our department for a period of 1 year. Results: The mean age of presentation was 35 years and there was a male preponderance (3:2). Headache was the most common presenting feature (80%) followed by seizures, altered sensorium, hemiparesis, cranial nerve deficits. The predisposing factors were predominantly noninfective and included inherited thrombophilias like protein C and S deficiencies, pregnancy, hyperhomocystinemia, malignancies. Infective cases were mostly bacterial meningitis. Investigations: MR Venography is the most definitive imaging modality and revealed lack of flow due to thrombosis in venous sinuses most notably in superior saggital sinus. CT Scans revealed delta sign, cord signs in few cases. D-dimer was elevated in another few cases (25%). Treatment: Anticoagulation is the cornerstone of treatment. LMW Heparin was given in all patients for 14 days and was followed by oral anticoagulant, mostly warfarin and in few cases by Dabigatran especially in protein C and S deficient patients. Appropiate antibiotics were used in selected cases where infection was the etiology. Interventional procedures like decompressive hemicraniectomy or endovascular thrombolysis were not required in any of the cases. Outcome: Only 2 patients (4%) died within 7 days of the illness despite initiation of therapy. Out of the surviving 48 patients, 8 (12.5%) are recovering from residual disability and the rest are doing well.


Smokeless tobacco and stroke - A preliminary clinicoepidemiological follow up study in a tertiary care teaching hospital

S. S. Jena, S. Kabi, B. N. Panda, B. C. Kameswari, Payal, I. C. Behera

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

Background: Among the modifiable risk factors for stroke, tobacco smoking is well recognized. In some studies the use of smokeless tobacco (ST) has also been contributed as a risk factor for Ischemic stroke. Use of ST is very common in Southeast Asia. The form of ST varies according to the geographical & cultural variation. Aim of Study: To study the various clinical symptoms and radiological findings of stroke due to different types of ST and follow up. Settings and Design: This is a prospective hospital based study carried out over two year's period. Materials and Methods: All the cases within age group of 16 - 60 years and with a clinical and radiological diagnosis of acute stroke were included in the study. The Fagerström Test for Nicotine Dependence for ST of more than 6 was included in the study. Patients having other addictions like smoked tobacco, alcohol etc and with important risk factors like hypertension, diabetes, dyslipidemia were excluded. The cases were extensively investigated and followed up for at least 6 months. Statistical Analysis Used: Analysis was done using the Statistical Package for Social Sciences (SPSS-version 16). Descriptive statistics like percentage, mean were used wherever appropriate. Results: During a period of 2 years a total of 54 patients were studied. Forty two (77.7%) were males and 12 (22.3%) were females. The mean age at presentation was 42.72 (± 8.6) years and among all 96.3% patients were diagnosed as ischemic stroke. Among ST, pan was most commonly used in 21 (38.9%) patients with an average of 14.6 (±3.27) years of addiction. Abnormal speech was the predominant symptom on presentation 33 (61.1%). According to Oxfordshire Stroke Classification, partial anterior circulation infarct was most common in 20 (38.4%). The mean Modified Rankin scale after 5 days of hospital stay was 3.83 (±1.03) and after 6 months of follow up was 2.1 (±0.8). The Barthel Index at presentation was mean 12.2 (±3.32) and at final follow up was 18 (±2.3). Patients were counseled for deaddiction and after 6 months follow up 48 (88.8%) patients had quit ST. Conclusion: ST is an important etiological factor for young ischemic stroke. We require specific serum biochemical analysis for nicotine to quantify the level of addiction that will help us to further prognosticate.


Bilateral supranuclear vertical gaze palsy in unilateral thalamo-mesencephalic infarct: vertical one-and-a-one syndrome-Report of 3 cases

R. Mahale, A. Mehta, K. Buddaraju, M. Javali, P. A. R. Srinivasa

Department of Neurology. M.S Ramaiah Memorial Hospital, Bengaluru, Karnataka, India

Vertical gaze abnormalities occur due to infarct in the unilateral thalamo-mesencephalic junction and isolated medial thalamus. Unilateral damage to the thalamo-mesencephalic junction is caused by stroke in the territory of the thalamo-subthalamic paramedian artery. Vertical gaze abnormalities that are reported due to damage to unilateral thalamo-mesencephalic junction include vertical one-and-a-half syndrome, contralesional monocular elevation palsy, co-existence of vertical and horizontal one-and-a-half syndrome and vertical half-and-a-half syndrome. Hereby, we describe 3 patients who presented with unique vertical gaze disorder in the form of acute bilateral conjugate supranuclear vertical gaze palsy. Case 1: A 42-year-old man presented with history of gait unsteadiness of 2 days duration. Oculomotor abnormalities were noted in the form of restricted up and down gaze in both eyes. Vertical vestibulo-ocular reflex was preserved. Horizontal gaze was preserved in both eyes. Brain magnetic resonance imaging (MRI) showed acute infarct in right antero-medial thalamus and right rostral ventral paramedian midbrain. Case 2: A 54-year-old lady presented with history of double vision of 1 day duration. Oculomotor abnormalities were noted in the form of restricted up and down gaze in both eyes. Vertical vestibulo-ocular reflex was preserved. Brain MRI showed acute infarct in left antero-medial thalamus. Case 3: A 67-year-old man presented with history of giddiness and vomiting of 1 day duration. Left homonymous hemianopia was present. Oculomotor abnormalities were noted in the form of restricted up and down gaze in both eyes. Vertical vestibulo-ocular reflex was preserved. Horizontal gaze was preserved in both eyes. Brain magnetic resonance imaging (MRI) showed acute infarct in right antero-medial thalamus and right rostral ventral paramedian midbrain. The occurrence of bilateral supranuclear vertical ophthalmoplegia in thalamo-mesencephalic infarct has not been reported. We named this vertical gaze abnormality as vertical one-and-a-one syndrome.


Etiological spectrum of extracranial internal carotid occlusion in young-atherosclerosis or else?

K. C. Padhiyar, P. Joshi, S. Shah

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

We frequently encounter extracranial internal carotid occlusion in young patients with ischemic stroke. There has been increasing prevalence of atherosclerotic risk factors in young and many of the extracranial Internal Carotid Artery (ICA) occlusions are presumed to be due to atherosclerosis based on presence of risk factors. We evaluated patients with ECICA occlusions in age group of 10-44 years. Despite the presence of atherosclerotic risk factors in patients, the etiology of ICA occlusion may be dissection or else. We also reviewed the literature of Extracranial Internal Carotid Artery (ECICA) occlusions and reviewed the radiolgical features of atherosclerosis, dissection and other etiologies. In young patient presenting with ECICA occlusion, certain radilogical features help differentiate atherosclerosis from other etiologies like dissection which are more common in young patients. Differentiating between the etiologies may have important long term therapeutic implications for secondary prevention of ischemic stroke.


Study of association of lipoprotein(a) levels with carotid atherosclerosis in young patients with ischemic stroke

P. Gupta, I. Anand, A. Batra, P. K. Sethi

Department of Neurology, Shri Mata Devi Narayana Hospital, Jammu, Jammu and Kashmir, India

Context: Lipoprotein (a) is an established risk factor for coronary artery disease but its role as a risk factor for stroke is not established. Aims: To estimate the prevalence of elevated levels of lipoprotein (a) in young patients (18-55 years) with ischemic stroke and to study the association between lipoprotein (a) plasma concentration and carotid atherosclerosis Settings and Design: Thisobservational, cross-sectional, single center study was conductedon 90 young (18-55 years)patients with imaging confirmed ischemic stroke for a duration of 1 year. Lipoprotein (a) samples were taken within 24 hours of presentation to the hospital and values >30 mg/dL were considered abnormal. Carotid Doppler was done by a cardiologist unaware of history of patients. Patients were divided into two groups: Group A (normal) and Group B (abnormal). Average values of lipoprotein (a) levels in both the groups were taken and compared using appropriate statistical analysis. Results: There were 65 males and 25 females in the study. Prevalence of elevated lipoprotein (a) in the study population was found to be 44.4%. It was more than the other traditional risk factors for stroke. Prevalence of carotid atherosclerosis and carotid stenosis in the study was 41.1% and 31.1% respectively. Elevation of lipoprotein (a) was strongly associated with carotid atherosclerosis (p=0.001). Mean values of lipoprotein (a) were statistically higher in the atherosclerosis group than in no atherosclerosis group (p=0.002). Lipoprotein (a) was positively associated with carotid stenosis in a graded manner but its association with CIMT was not significant. The association of lipoprotein (a) and carotid atherosclerosis was independent of other risk factors. Conclusion: Lipoprotein (a) is strongly associated with carotid atherosclerosis (especially carotid stenosis) in young patients with ischemic stroke and the association is independent of the traditional risk factors.


A study on public awareness of risk factors for stroke and history of transient ischemic attacks in middle cerebral artery ischemic stroke patients

R. Sugumaran, V. Chandramouleeswaran, K. Bhanu, V. Ramakrishnan, S. Gobinathan

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

Background and Purpose: This study was conducted to assess the public awareness of risk factors of stroke and history of transient ischemic attacks among middle cerebral artery ischemic stroke patients admitted in our hospital. Patients and Methods: It's a cross sectional study. Hundred middle cerebral artery ischemic stroke patients admitted in Rajiv Gandhi government general hospital, Chennai, India during December 2015 to February 2016 were enrolled in the study. Risk factors for stroke were interviewed among patients or their relatives. Diagnosis of transient ischemic attacks was made using ABCD2 score. Results: Risk factors for stroke were known to 64% of the patients or their relatives. Among the risk factors known systemic hypertension 48%, diabetes mellitus 40%, age 22%, family history 15%, dyslipidemia 7%, cigarette smoking 4%, cardiac disease 3%, alcohol consumption 3% and obesity 2%. People with primary, secondary education, higher income with knowledge of the risk factors for stroke were 75%, 80%, 77.8% respectively. People who were uneducated and low income with knowledge of risk factors were 60% and 60.97% respectively. History of transient ischemic attacks was present in 8% of patients. 6 patients had ABCD2 score ≥4 and 2 patients had ABCD2 score <4. Conclusions: Transient ischemic attacks are an important harbinger of stroke. People have less knowledge about risk factors of stroke while people with higher income and education have better knowledge.


Study of risk factors and pattern of stroke in young adults (15 - 45 years) in a tertiary care hospital of Eastern India

L. K. Sahoo, A. K. Mallick, G. Mohanty, K. P. Swain, S. D. Nayak, M. Samanta

Department of Neurology, S.C.B Medical College and Hospital, Cuttack, Odisha, India

Background: Stroke is the one of the most common cause of morbidity and mortality in India. Stroke in young adults can have a significant impact and long term burden on the patients, their families and the community in general. Objective: To study the risk factors and pattern of stroke in young adults. Materials and Methods: This is a prospective study conducted in Neurology Department of SCB Medical College, Cuttack, Odisha from September 2014 to June 2016. Stroke patients in age group between 15 and 45 years were evaluated by routine clinical examination, risk factors, neuroimaging, routine hematological and cardiological examination. A total of 52 patients were included. Results: There was female preponderance (60%). Mean age was 37.5 ± 7.9 years. Ischemic stroke was most common in 22 (42.3%) cases followed by hemorrhagic stroke in 20 (38.5 %) cases and cardioembolic stroke in 10 (19.2%) cases. Anterior circulation stroke was detected in 38 (73.1%) cases and posterior circulation stroke in 11 (21.2%) cases. Stroke involving both anterior and posterior circulation was seen in 1 patient and primary IVH in 2 patients. Single risk factor was present in 23 (44.2%) cases. Two risk factors were present in 13 (25%) cases and ≥3 risk factors in 3 (5.8%) patients. No risk factor was detected in 13 (25%) patients. Hypertension was the most common risk factor in 22 (42.3%) patients followed by diabetes mellitus in 11 (21.2%) patients. Among other risk factors valvular heart disease in 9 (17%) patients, dyslipidemia in 8 (15.4%) patients, coronary artery disease in 1 patient, vasculitis in 3 patients, sickle cell disease in 1 patient, chronic ITP in 1 patient and hyperhomocystinemia in 2 patients. Smoking and alcohol addiction was present in 2 patients each. The mean NIHSS score at admission was 12.4 ± 5.7. The mean MRS score at admission was 4.52 ± 1.0 and at discharge was 3.42 ± 1.4. Two of the patients died during hospitalization. Conclusion: The present study indicates that hypertension is the most common risk factor of young stroke followed by diabetes mellitus and valvular heart disease. So special attention should be given for primary prevention of hypertension and diabetes mellitus.


An uncommon cause of stroke in a tropical setting

T. Thekkumpurath, J. B. Agadi, N. B. Archana, P. S. Kumar, N. Karthik

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

Snakebite is one of the common life-threatening medical emergencies. In India more than 20, 00,000 snake bites are reported annually, of which 35,000 to 50,000 people die. The clinical characteristics include local cellulitis, renal failure and hemorrhagic manifestations including pituitary and intracranial haemorrhage. Cerebral ischemic infarction following viper envemation is rare. A 42 years old female was admitted with altered sensorium and aphasia following viper bite. On examination her GCS was E2V3M5. She was in altered sensorium moving all limbs to pain. She had a deep fang mark with erythema on her right foot. In view of derangement of coagulation parameters she was started on anti snake venom within 2 hours. She was given ventilator support in view of aspiration pneumonia. MRI brain revealed bilateral infarcts in the inferior division of MCA with normal angiogram of extra and intra cranial vessels. All other etiological factors for stroke like Hypertension, Fasting lipid profile, RBS, ANA, APLA protein C and S, anti-thrombinIII, factor 5 mutation, homocysteine were found to be negative. Her cardiac evaluation was also normal. She made a partial recovery with supportive treatment over a period of 3 weeks. Viper snake venom is a complex toxin affecting haemostatic mechanisms which exhibit both anticoaguͼlant and procoagulant effects. Venom in large doses causes massive intravascular coagulation, leading to occlusion of small and even large vessels, resulting in cerebral infarction. The possible mechanism for cerebral infarction in this case may be due to toxin induced vasculitis and endothelial damageand disseminated intra vascular coagulation.


Intravenous thrombolysis for acute ischemic stroke: Review of 97 patients

A. Shankar, R. Mahale, A. Mehta, M. Javali, P. Acharya, R. Srinivasa

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

Background: Intravenous thrombolysis (IVT) has now become a standard treatment in eligible patients with acute ischemic stroke who present within 4.5 hours of symptom onset. However, with the advent of intra-arterial thrombolysis (IAT)/endovascular mechanical thrombectomy (EMT) and demonstration of their superiority in the recent studies with respect to recanalisation of occluded vessels and outcome, intravenous thrombolysis (IVT) has lost its sheen among the interventional neurologists worldwide 3, 4. But, IVT still has a role to play in the management of AIS in the resource-poor settings of the developing countries. Objective: To determine the usefulness of IVT and the subset of patients who will benefit from IVT in AIS within 4.5 hours. Methods: Patients with acute ischemic stroke within 4.5 hours of symptom onset who underwent IVT were studied prospectively. The study period was from October 2011 to October 2015. Results: A total of 97 patients were thrombolysed intravenously. The mean onset to needle time in all patients was 177.2 ± 62 minutes (range: 60-360). At 3 months follow-up, favourable outcome was seen in 65 patients (67.1%) and poor outcome including death in the remaining 32 patients (32.9%). Factors predicting favourable outcome were age less than 65 years (p = 0.02), NIHSS <15 (p < 0.001), small vessel occlusion (p = 0.006), cardioembolism (p = 0.006) and RBS less than 250 mg/dl (p <0.001). Factors predicting poor outcome were diabetes mellitus (p = 0.01), dyslipaedemia (p = 0.01), NIHSS at admission >15 (p = 0.03), RBS >250 mg/dl (p = 0.01), DRAGON score >5 (p = 0.03), and occlusion of large artery (p = 0.02). Conclusion: Milder baseline stroke severity, blood glucose < 250 mg/dL, younger patients (<65 years), cardioembolic stroke and small vessel occlusion benefit from recombinant tissue plasminogen activator (rt-PA).


A rare etiology of oculomotor palsy

L. Singh, K. Bhuddharaju, J. V. Mahindra, P. T. Acharya, A. Miryala

Department of Neurology, MS Ramiah Medical College, Bengaluru, Karnataka, India

Introduction: Oculomotor nerve originates in midbrain at the level of superior colliculus and has a long intracranial course before supplying extra-ocular muscles, eyelid and parasympathetic innervation to pupil. In view of its long intracranial course, it can be affected at midbrain, sub arachnoid space, cavernous sinus and in the orbit. Etiologies can be multiple like compression by temporal lobe, aneurysm of post cerebral artery, superior cerebellar artery, internal carotid artery (ICA), infarction of nucleus and fascicles of 3 rd cranial nerve, infections, Tolosa hunt syndrome, orbital apex syndrome and local orbital pathologies. Ischemia of 3 rd nerve palsy due to ICA occlusion/stenosis is relatively rare. Here, we report a patient who developed oculomotor nerve palsy due to ICA occlusion. Case Report: 48 yr old male, presented with weakness of right upper and lower limb with deviation of angle of mouth to left side. Neurological examination showed right upper motor neuron facial palsy and right hemiparesis (Medical Reseach Council (mRC) grading 4/5) Magnetic Resonance Imaging (MRI) brain showed left Anterior Cerebral Artery (ACA)/Middle Cerebral Artery (MCA) watershed infarcts and MRI angiogram showed left internal carotid artery not visualized. During hospital stay he developed left third CN palsy with worsening of right upper and lower limb power (MRC grade 0/5). Repeat brain imaging showed increase in the size of watershed infarcts. Work up for other etiologies of 3 rd CN involvement like AntiNuclear Antibody (ANA-IF&Profile), Antinuclear and cytoplasmic antibody (C-ANCA& P-ANCA), Angiotensin converting enzyme (ACE) levels, infections, HIV, HbSAg, VDRL were negative. Patient was continued on antiplatelets, statins, diabetes and hypertensive medications. On follow up left 3 rd CN improved and he was able to walk with one person support. Discussions: Our patient had left 3 rd CN palsy with evolving right hemiparesis. Work up for other etiologies including midbrain infarcts, uncal herniation, aneurysms, infective arteritis, and collagen workup were negative. In view of temporal evolution of left third cranial nerve and right hemiparesis, possibility of ischemia of 3 rd CN is being considered. There are few reports of ischemic oculomotor palsy due to ICA occlusion. Conclusion: Oculomotor nerve palsy due to ischemia of third cranial nerve following ipsilateral ICA occlusion is relatively rare, and should be considered in the etiology of occulomotor palsy.


Recurrent anterior ischemic optic neuropathy as a cause for pseudo-Foster Kennedy syndrome

D. Naheed, A. Patil, V. Lal, A. Takkar

Department of Neurology, PGIMER, Chandigarh, India

Introduction: Nonarteritic anterior ischemic optic neuropathy (NAION) is one of the most common optic neuropathy. The probability of involvement of fellow eye varies among different studies. The subsequent involvement of fellow eye gives the clinical appearance with a pale disc and disc edema in presenting eye like Pseudo- Foster Kennedy (PFK) syndrome. Objective: To review recurrent NAION as a cause for PFK syndrome. Materials and Methods: In an observational study all patients presenting with sudden loss of vision were evaluated. We reviewed patients presenting with disc edema on one side and optic atrophy in contralateral eye on fundus examination. Their visual field defects and fundus fluorescein angiography was assessed. Results: Of the 5 patients evaluated 4 (80%) were females. Mean age at presentation was 55 years. Mean duration between the two episodes was 13.8 months. The visual acuity of presenting eye ranged from 6/12 to worse counting fingers close to face. There was no association between risk factors for NAION and recurrence. Discussion and Conclusions: Patients with NAION are at risk for recurrence in fellow eye, thereby presenting as PFK syndrome. The history of sudden acute often sectoral visual loss in the fellow eye may give the clue although poor visual acuity is not a rule.


Estimated glomerular filtration rate as a predictive factor for 30 day mortality in acute stroke

S. Mahadeva, B. Nataraju, S. Swamy, S. Kodapala, A. Gurucharan, S. Raju

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

Background and Purpose: The purpose of this study was to investigate Glomerular filtration rate as predictor of 30 day mortality in acute stroke. Methods: This was a prospective Observational study done in Vydehi Institute of Medical sciences and Research centre, Whitefield, Bengaluru, which is a tertiary teaching hospital. Participants included 100 consecutive acute stroke patients admitted to the hospital over a one year period from 2015 to 2016. Follow up period was after 30 days. Main outcome was all cause mortality. Results: The total number of deaths at the end of follow up period was 33 (33%). The mean calculated Glomerular filtration rate was 62.09 ± 34. 35, which was calculated by the Cockgroft - Gault equation. Stroke survivors had a higher mean GFR. Low GFR <60ml/min/1.73 m2, predicted higher risk of mortality. There was also a significant association between stroke severity by NIHSS and mortality. Conclusions: Renal dysfunction measured by low GFR, in patients with acute stroke, predicted short term, 30 day mortality. Renal dysfunction can therefore help stratify risk in stroke patients for further interventions. Though many studies have been done showing renal dysfunction to be an independent predictor of stroke mortality, it is still a parameter which is given less importance while determining predictors of mortality after acute stroke , especially so when renal function is assessed solely based on serum urea and creatinine, without estimating the Glomerular filtration rate. This is important as low GFR has been shown to predict mortality even when serum creatinine levels are within normal range. Hence, the findings of our study reiterate the significance of estimating the GFR in acute stroke patients and to bring this parameter at the forefront in predicting prognosis for mortality after acute stroke.


Subtypes of ischemic stroke and correlation with risk factors in Western Rajasthan

K. Sachdev, A. Bhargava, K. Shubhakaran, A. Shah, Pratibha, Nitti

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

Background: There is paucity of Indian data on subtypes of ischemic stroke and each subtype is characterized by different age group, risk factors, comorbidities, treatment strategies and outcome. Aim: This study was planned to characterize stroke subtypes based on clinical and MRA findings and to correlate each subtype with conventional risk factors and outcome. Methodology: This prospective observational study took place at tertiary care teaching hospital at Jodhpur from Jan 2015 to Mar 2016. A total of 100 admitted patients with acute ischemic stroke were clinically evaluated for conventional risk factors along with relevant blood investigations and MRI with MR Angiography which was considered significant if stenosis was ≥ 50%. Patients enrolled were categorized as per TOAST classification. Results: Among ischemic stroke patients; mean age was 60.84 (range 15-98 yrs) with male preponderance (79%). Most common subtypes was large artery atherosclerosis (44%) followed by small vessel disease (39%), cardioembolic (10%), other determined etiologies (4%) and undetermined etiology (3%). Large and small vessel disease was common in elderly (mean age-64.75 yrs) while remaining subtypes were more common in younger age group (mean age-37.81 yrs). There was predominance of intracranial (56%) than extracranial (23%) atherosclerosis which is the most notable difference from western studies whereas 21% patients had normal MRA study. Small vessel disease was more commonly associated with risk factors like Hypertension (76%), Diabetes (56%) and Dyslipidemia (53%) with a significant P value of < 0.05. Recurrent CVA, TIA, higher mean NIHSS score and mortality was more commonly associated with large vessel disease. Conclusion: Ischemic stroke is a disease with multiple etiologic factors with marked difference among subtypes regarding risk factors and outcome. Large artery atherosclerosis was predominant type and intracranial involvement was more common.


Intra abdominal bleeding due to oral anticoagulation in cerebral venous thrombosis: Report of two cases

P. Raj, G. B. Kulkarni, R. J. Singh, V. K. Mustare

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

Introduction: Patients who are on oral anticoagulation for cerebral venous thrombosis (CVT) are at risk for bleeding manifestations and need constant vigilance. Materials and Methods: Case report and review of literature on intra abdominal bleeding due to oral anticoagulation in CVT. Case Description: Case 1: 27 year old lady presented with signs and symptoms suggestive of CVT and was on anticoagulation. During the hospital stay she developed acute onset diffuse right leg pain and on examination had pain while extending right hip. MR Imaging showed a large collection in right iliac fossa within retroperitoneal space. Her Prothrombin time (PT) was 172.8 seconds with INR of 10.8. Patient was treated with vitamin K injection and fresh frozen plasma (FFP) and packed cell transfusion and improved. Case 2: 29 year old man with CVT underwent decompressive craniectomy with bone flap placement in abdomen. Post operatively had abdominal site infection which was treated with antibiotics. He was started on anticoagulation and 33 days later presented with acute onset fever with abdominal pain and discharge from abdominal surgical wound. Imaging showed rectus sheath hematoma. His PT/INR values were prolonged. He was managed with FFP and vitamin K injection followed by exploration and evacuation of hematoma and improved. Both the patients had acute onset new symptoms with prolonged coagulation parameters which led to suspicion of systemic bleeding. The early recognition using appropriate imaging helped in achieving good outcome. Conclusion: This report brings about rare presentation of systemic bleeding complications of anticoagulation in young patients with CVT and need for the physician to look for such causes in patients developing unexplained symptoms with prolonged coagulation profile while receiving anticoagulation and the importance of imaging for accurate diagnosis and management.


Stroke and acute lower limb ischemia following multiple bee sting

R. Rajendran, S. Gobinathan

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

Neurological events following bee stings are uncommon. We report a 60-year-old man who developed swelling all over the body following multiple bee sting for which he was hospitalized. On the second day of hospitalization he became drowsy & developed right sided hemiplegia with seventh cranial nerve palsy. MRI brain revealed multiple areas of diffusion restriction noted in pons, bilateral cerebellar hemisphere, thalamus, medial temporal and occipital lobes with normal MRA & MRV. Subsequently he developed blackish discolouration of right lower limb upto midthigh with absent distal pulses. Arterial Doppler showed right femoral & left tibial artery thrombosis. He was started on anti-platelets and low molecular weight heparin. Despite adequate and effective treatment measures, his general condition worsened and he expired following cardiorespiratory arrest.


Clinico-epidemiological profile of border-zone infarct: A study from North-West India

T. Desai, R. S. Jain, T. Srivastava, K. Khandelwal, K. Nagpal

Department of Neurology, SMS Medical College and Hospital, Jaipur, Rajasthan, India

Objective: To study clinical and epidemiological features of border-zone infarct (BZI). Methods: Retrospective analysis of 64 cases of border-zone infarct was done. Border-zone infarcts were selected based on diffusion-weighted imaging templates to identify vascular territories. Baseline characteristics (age, sex, risk factors, neuroimaging and relevant investigations) were compared between internal border-zone (IBZ), cortical border-zone (CBZ) and mixed infarct pattern. Results: Total 64 cases of BZI were collected, of which 43 were male and 21 were female. IBZ infarct was present in 59.37% cases (total 38, M=20, F=18), CBZ infarct present in 23.43 %( total 15, M=14, F=1) and mixed infarct pattern (IBZ + CBZ) present in 17.18% cases (total 11, M=9, F=2). In IBZ, CBZ and mixed Pattern infarcts, mean age of onset was 61.26±8.6, 55.93±13.44, 60.63 ±11.92 years respectively. Predominant risk factors in IBZ and CBZ infarct were HTN (55% and 60% respectively) and smoking (50% and 80%) respectively. In mixed pattern infarct cases, dyslipidemia and smoking were most common (63% each) risk factors. Coronary artery disease was present in only 3 cases overall (2 in IBZ and 1 in mixed). Recurrent TIA was present in 23.4% patients (8 in IBZ, 5 in CBZ and 2 in mixed pattern). In IBZ, CBZ, and mixed pattern infarcts, hemiparesis was present in 71%, 66% and 54% respectively while faciobrachial monoparesis was present in 26%, 26% and 36% respectively. Conclusions: IBZ infarcts are the most common type of BZI. Males are more commonly affected as compared to females with M: F=2:1. Patients most commonly present in 6th and 7th decade. Smoking and hypertension are the most common risk factors for BZI. Hemiparesis is the most common presentation followed by faciobrachial monoparesis. This is probably the first study on characterisation of border zone infarcts in India.


Vertebral artery hypoplasia in posterior circulation stroke

M. Pradeep, R. Chinaapan, Shreelakshmi

Department of Neurology, KG Hospital and Post Graduate Institute, Coimbatore, Tamil Nadu, India

Introduction: Vertebral artery hypoplasia (VAH) is prevalent in approximately 10% of normal individuals. In recent years increasing evidence has suggested that VAH contributes to posterior circulation ischemic events. This study aimed to investigate the prevalence and pathogenitic role of VAH in ischemic events of the posterior circulation. Patients and Methods: we analysed all patients admitted to our stroke unit over a 5 year period (2010-2015) with documented posterior circulation stroke. The patients were dived into two sub groups based on the presence or absence of VAH. The presence of VAH was evaluated by ultrasound and then confirmed by MRA/CTA/DSA. We analysed the clinical out come, risk profile & etiology of posterior circulation strokes in presence of VAH. Results: Out of 200 cases of Posterior circulation strokes, VAH was present in 28% of cases. The two sub group patients did not show significant differences in age, sex distribution or clinical out come. Posterior circulation strokes occuring in the presence of VAH were located in a more proximal territory. VAH of the right vertebral was more compared to the left vertrbral artery. Embolic strokes occured more frequently on the side contralateral to VAH, where as strokes caused by other mechanisms (hemodynamic & thrombotic) occured ipsilaterally. Conclusion: This study shows that VAH is more frequently seen among patients with posterior circulation ischemia than in the general population. The most frequent stroke pattern in patiients with VAH is non embolic.


An interesting report on 3 patients of transient symptoms with cerebral infarction

G. Kanniappan, A. E. Iyyadurai, A. T. C. Vijay, N. Muthuveeran, M. R. Manivannan, B. Sridharan

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

Introduction: Patients with transient symptoms who have evidence of infarction on diffusion-weighted imaging (DWI) have a higher risk for early recurrent stroke or TIA than either those with a completed ischemic stroke or those with transient symptoms and a normal DWI scan. Here we present 3 patients who presented with transient neurological symptoms with DWI evidence of acute cerebral infarction. Case Vignette 1: 80 years male presented with sudden onset of aphasia with right arm weakness of 1 hour duration and had left putamen & caudate infarct on DWI. His symptoms improved spontaneously in MR room without any deficit. His echo was normal and Doppler showed bilateral increased intimal media thickness. He was treated with antiplatelets, statins and on follow up. Case Vignette 2: 17 years girl presented with weakness of right upper limb with aphasia. Her symptoms improved spontaneously within 1 hour of onset. Her MR showed acute infarct in left putamen and corona radiate on DWI. Her cardiac evaluation, hemogram, were normal, viral markers, vasculitic work up were negative. She was treated with anticoagulants and antiplatelets. Case vignette 3: 55 years old female presented with reduced word output for 1hour. Her MR was taken elsewhere showed left perisylvian acute infarct on DWI and referred for thrombolysis to our hospital. Her symptoms subsided on her way to hospital. She was evaluated found to have concentric LVH and insignificant bilateral carotid stenosis. She was treated with antiplatelets, statins, and antidiabetics. Conclusion: Transient symptoms with infarction (TSI) may be a clinical entity separate from TIA, with a different prognosis. TIA patients who have DWI abnormalities seem to be the equivalent of unstable angina in cardiology -- high-risk patients who are on the verge of having a stroke, and who do go on to have a stroke in the next few days.


A study on the clinical profile of patients with carotid artery disease in a tertiary care center

M. I. Matheen

Department of Neurology, Sri Ramachandra University, Chennai, Tamil Nadu, India

Aims and Objectives: (i) To study the clinical profile of patients who presented to our tertiary care centre with symptomatic carotid artery disease. (ii) To assess an association between Carotid artery disease and LDL, HDL levels. Methods and Results: We retrospectively evaluated 45 patients diagnosed by CT Angiogram of Neck and Intracranial vessels to have carotid artery disease in the study period between 1.6.2015 to 1.6.2016. All the patients had presented with focal neurologicaldeficits and had been evaluated first by MRI brain followed by Echocardiogram and Carotid-Vertebral Doppler. If they were found to have significant reduction in blood flow on Carotid Doppler study, they were subjected to a CT angiogram of the neck and intracranial vessels. The subjects who were found to have more than 50% luminal narrowing on CT Angiogram were included in the study. Out of 45, 35 were males and 10 subjects female, with an average age of 54. 24 subjects were having type 2 diabetes. 20 patients were diagnosed to have systemic hypertension. Out of 45, only 2 patients had Amaurosis Fugax. On correlating the carotid artery disease and lipid levels it was noted that among 45 patients 38 had low HDL levels with near normal LDL levels. Conclusion: Our study shows that males were more prone to carotid artery disease. Almost half of subjects were diabetics. Visual symptoms were the least common clinical presentation, furthermore most of our patients (38 out of 45 i.e. 84.4%) had an altered HDL: LDL ratio with low levels of HDL and near normal levels of LDL.


Achard levi syndrome

U. Karadan, Hariprasad

Department of Neurology, Baby Memorial Hospital, Calicut, Kerala, India

Introduction: Isolated mid brain infarction is extremely rare. They can be classified into three categories (1) Classic ischemic brainstem lesions with cranial nerve lesions and contralateral signs of long tracts (2) Lacunar brainstem syndromes with isolated long tracts (3) Brainstem syndromes with isolated cranial nerve palsies. We report a rare case of mid brain infarct with isolated 3 rd cranial nerve palsy. Case Report: 60 years old female diabetic, hypertensive presented with acute onset vertigo and diminished vision. She also complained of vertical diplopia. Examination showed isolated pupil sparing 3 rd nerve palsy. No long tract signs or corticospinal or corticobulbar palsy. MRI showed small infarct in the midbrain. Conclusion: Isolated 3 rd cranial nerve palsy due to midbrain infarct is rare. It has to be recognized and differentiated from other conditions.


Midbrain bleed with nothnagel syndrome

V. Y. Vishnu, R. Shree, A. Choudhary, V. Lal

Department of Neurology, PGIMER, Chandigarh, India

Background: Crossed brain stem syndromes described in literature are rare to encounter in clinical practice in patients with brainstem stroke. Case History: 34 year-old lady presented with sudden onset altered sensorium followed by drooping of right eyelid and severe tremors of left upper and lower limbs. On examination, she had right third cranial nerve involvement with bilateral horizontal nystagmus, severe disabling intentional tremors of right upper and lower limbs with severe ataxia. Computed tomography of head showed midbrain haemorrhage. Discussion: The named brainstem syndromes described in literature are numerous, one of which is Nothnagel syndrome, described by Nothnagel in 1879. These brainstem syndromes serve to illustrate the possible neuroanatomical connections in the human brainstem. The close proximity of the oculomotor nuclear complex to the dendato-rubro-thalamic fibers (within the midbrain tegmentum) constitutes the accepted anatomic basis of this semeiological association. Conclusion: The classical brainstem Nothnagel syndrome, though commonly described in neurology textbooks is rare to be encountered in clinical neurology practice. The strategic area needs to be involved in the brainstem for such manifestations.


Serum albumin as a predictor of functional outcomes following acute ischemic stroke

R. K. Nair, K. Radhakrishnan

Department of Neurology, KMC, Manipal, Karnataka, India

Stroke is currently the leading cause of neurological disability in adults. Two-thirds of stroke survivors have residual neurological deficits that impair their function and approximately 50% are left with disabilities making them dependent on others for activities of daily living. Following a stroke, the central nervous system undergoes reorganization during the process of functional recovery. However, the degree of recovery is variable and the processes subserving this recovery are incompletely understood. Neurophysiological changes associated with recovery often begin within 1-2 weeks following stroke and may plateau within two and three months, depending on the degree and extent of the neurologic deficit. A large number of factors such as admission functional ability, hemineglect, incontinence, age and others may influence the functional outcome of stroke patients. In undernourished people, stroke like acute illness, may lead to a negative energy balance and greater nutritional demand. Stroke patients may not able to meet these increased demands. The level of serum albumin has been acknowledged to be a marker of nutritional status. It can be a useful measure when acute changes in nutrition need to be assessed. Albumin has multifaceted intravascular effects. It not only reduces haematocrit level but also influences erythrocyte aggregation. Albumin constitutes a major antioxidant defense against oxidizing agents. Actually albumin traps the plasma's oxygen radicals and retards the formation of highly reactive hydroxyl radical species. A component of the neuro-protective effect of human albumin in acute ischemic stroke resides in its antagonism of stagnation, thrombosis and leukocyte adhesion within postcapillary microcirculation in the early reperfusion phase. Hypoalbuminemia at admission may cause negative influences on neuro-protection in acute stroke patients. If this hypothesis is found to be true, treating patients having hypoalbuminemia in acute stroke might enhance their functional outcome. The study is ongoing.


Ankle brachial index in stroke: A hospital based study

A. Koul, S. P. Saha

Department of Neurology, GB Pant Hospital, New Delhi, India

Ankle brachial index (ABI), the ratio of ankle and brachial systolic blood pressure, is commonly used to confirm the diagnosis and assess the severity of peripheral arterial disease (PAD). Many studies show that low ABI independently predicts the risk of stroke events while other studies didn't find such an association. The ABI is considered an inexpensive, easy and a reliable tool to identify patients with PAD. Many reports on the prevalence of PAD in cerebrovascular diseases have been published in the west but there is hardly any data about the prevalence of PAD in the stroke patients in India. So, we undertook this study which looked at the association of ABI and ischemic stroke risk and outcomes at one month. We used the simple bedside method of palpation of foot arteries to detect PAD. The study was carried out in ischemic stroke patients attending the neurology department of a tertiary care centre in Kolkata over a one year period. A total of 158 consecutive ischemic stroke patients were enrolled in our study and 150 age and sex matched individuals were taken as controls. The stroke patients were divided into two groups based on the ABI values (< 0.9 & ≥ 0.9). We found that overall the patients with low ABI values were more likely to be suffering from ischemic heart disease, diabetes, hypertension & hyperlipidemia. The most intriguing fact of our study was that not even a single patient of PAD complained of symptoms suggestive of intermittent claudication emphasizing the fact that PAD is largely an asymptomatic and hence an under diagnosed disease. This is probably the first study from India which has looked at the association between ABI and stroke and it does show that ABI is an important risk factor for the development of ischemic stroke.


Hyperhomocysteinemia in young males with cortical venous thrombosis

K. Vellaichamy, C. Prabakaran, J. Manikavasagam, S. Gobinathan

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

Background: Cerebral venous sinus thrombosis is a common cause of stroke in young condition precipitated by various conditions. We studied the clinical and biochemical profile of young males due to various aetiologies. Methods: Male patients with cortical venous thrombosis admitted in neurology ward in Institute of neurology, Madras Medical College between January 2016 to June 2016 were included in the study. A total of 15 patients were included in the study. All patients had headache; 6 patients presented with seizures; visual symptoms were seen in 8 patients; clinical examination revealed papilledema in all patients; 8 patients had lateral rectus palsy and 2 patients had limb weakness. Neuroimaging revealed superior saggital sinus thrombosis with right transverse sinus thrombosis in 9 patients and the remaining patients had r transverse sinus thrombosis. Hemorrhagic infarcts were present in 5 patients. On biochemical evaluation, high homocysteine levels were found in 9 patients with normal vitamin B12 and folate levels. Conclusion: Serum homocysteine level should be done in all patients with cortical venous sinus thrombosis routinely apart from other investigations.

Poster Session 07; Stroke and Headache14:00 - 16:30 hrs, Saturday, November 12, 2016


Correlation of retinal vascular parameter-retinal arteriovenous ratio in ischemic stroke and it's subtypes

K. Patil, S. K. Narayan, R. K. Babu

Department of Neurology, JIPMER, Puducherry, India

Background: Retinal microvascular changes mirrors that of cerebral circulation as retinal vasculature is anatomically and embryologically part of the cerebral vasculature. Ophthalmic artery being the first branch of internal carotid artery studying its transcranial Doppler parameters and retinal microvascular changes by fundus photography may reveal the possibility of an association between ischemic stroke and retinal microvascular changes. Objectives: (1) To study the association between Ophthalmic artery transcranial Doppler (OA-TCD) and retinal arteriovenous ratio (AVR) parameters with Ischemic Stroke (IS). (2) To study the correlation between baseline vascular risk factors and determine whether baseline risk factors correlate with OA-TCD and retinal AVR. Methods: In a cross-sectional observational study between 25 IS cases and age and gender matched healthy controls, OA-TCD study done through the transorbital window with power reduced to 10% by Foreview RIMED Machine. Retinal Fundus photographs were acquired using the non-mydriatic 45-degree digital retinal camera. By using student's T-test comparison of mean OA-TCD parameters and retinal AVR were done between IS and control group after controlling the confounding variables. Fischer's exact test compared proportions of abnormal AVR (<0.66). Spearman's rho was used to find a correlation between OA-TCD parameters and AVR. Results: OA-TCD parameters did not show significant association with IS group though Pulsatility index (PI) showed a non-significant trend of association with stroke group (P=0.059). There was a significant difference between IS and control in retinal AVR (Mean AVR 0.66± 0.09 for controls and 0.53± 0.05 for cases (P=0.001)). On partial correlation studies, for IS group, AVR was not affected significantly by HT (P- 0.51) or DM (P- 0.35), smoking (P=0.27).


Clinico radiological correlation of acute pontine stroke - A case series of 6 patients

A. B. Abdullah, N. S. Sundaram, S. B. Subramanian, S. Gobinathan

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

Introduction: Pontine strokes account for less than 17 % of total strokes. It can have complex symptomatology. The various presentations include as vertigo, severe imbalance, double vision, slurred speech or motor weakness depending on the various topographical insults to different areas in the pons. Risk factors include smoking, hyperlipidemia, diabetes mellitus, hypertension and Atrial fibrillation herewith we present a series of 6 patients with symptoms of pontine stroke and analyse the clinical and radiological features. Objective: Clinical localization of various pontine lesions and the radiological correlation. Settings: Madras institute of neurology, MMC Chennai. Patients: 6 patients who presented with symptoms of brainstem stroke and who were diagnosed clinically to have pontine strokes and confirmed radiologically were analysed. Results: Of the 6 patients studied; all of them were above 60 years of age. 4 patients were male and 2 were female. 4 patients had ischemic sroke while the other 2 had hemorrhagic stroke the risk factors were hypertension in 3, diabetes in 3 and chronic smoking in 4. The clinical presentations were pure motor limb weakness (2), dysarthria (1), vertigo (2), eye movement abnormalities in the form of one and half syndrome in 1 and upbeat nystagmus and abduction defect in one patient cerebellar symptoms in 2 and altered level of consciousness and disordered breathing (1). All the lesions were localized in the pons clinically and were confirmed radiologically. Of all the six, five patients improved with treatment and one expired. Conclusion: We present these 6 cases of pontine strokes to emphasize the value of clinical localization in brain stem strokes with radiology showing good confirmation of the clinical localisation.


Experiences in intervention in acute stroke at Army Hospital Research and Referral, New Delhi

C. S. Manoj, T. Pal, U. Bhanu

Department of Neurology, Army Hospital R & R Center, New Delhi, India

There is data of 15 patients who underwent intervention for acute stroke at Army Horpital Research and Referral, New Delhi. Out of thses patients, 13 patients presented with acute anterior circulation stroke, one with acue basilar artery stroke, one patient had right MCA occlusion as a complication while undergoing Anterior communicating artery (ACOM) aneurismal coiling. In these 15 patients 9 of them had underwent mechanical thrombectomy. Combined mechanical thrombectomy and intraarterial thrombolysis has been done in 2 patients. Only intraarterial thrombolysis was done in 2 patients. Intra arterial sucction device has been used in 2 patients. Out of these patients, good functional outcome (mRs 0 to 2) has been achieved in 8 patients after intervention. In 5 patients post intervention mRs was 3 to 5. 2 patients unfortunately died out of hemorrhagic complication.


Retrospective study of clinical profile of stroke patients from an industrial hospital in Eastern India

M. N. Singh

Department of Neurology, Tata Main Hospital, Jamshedpur, Jharkhand, India

Background: The cerebrovascular stroke is one of the leading causes of morbidity and mortality in adult life. After coronary heart disease and cancer of all types, stroke is the third commonest cause of death worldwide. Indian studies have shown a stroke prevalence of 471.58/100,000 population. The objective was to study the clinical presentation, risk factors, neurological presentation, pattern of brainstrokes, areas of brain affected as per CT scan findings in patients. Methods: This is a retrospective study of all 3026 patients managed in the neurological wards of Tata Main Hospital, Jamshedpur from January 2013 to December 2015. Results: The cerebrovascular strokes are more common in Males (59.7%) than Females (40.3%). Most common age group was 41-60 years (32.8%). Most common clinical feature was Hemiplegia (72.6%). Most common risk factor was Hypertension (85%) followed by past history of Cerebrovascular stroke (15%), smoking (14%), Dyslipidemia (13%). Most common type of stroke was Ischaemic (74.6%) and Hemorrhagic was 2 nd (22.9%). In Ischaemic stroke the most common involved areas were Parietal (33.7%), Frontal (16.7%). In hemorrhagic stroke most common site was Basal ganglia (24.7%) followed by ventricular (17.5%). The mortality rate was 23%. Conclusions: The cerebrovascular stroke cases were having male predominance with Hypertension being the most common risk factor and most common type of stroke was Ischaemic.


Hyperhomocysteinemia and Vitamin B12 deficiency as a risk factor for Ischemic Stroke: A case control study from Northwestern India

B. L. Kumawat, C. M. Sharma, M. Shah, A. Jain, D. Khandelwal

Department of Neurology, S MS Medical College, Jaipur, Rajasthan, India

Background: Vitamin B12 deficiency, by virtue of causing hyperhomocysteinemia, may be implicated as an acquired risk factor of Ischemic stroke, which is also easily modifiable. There is scarcity of data from India regarding prevalence of B12 deficiency in Ischemic stroke (IS) patients. Objectives: To explore the relationship between B12 and folate deficiency, hyperhomocysteinemia and IS. Design: Case control study. Materials and Methods: Eighty cases of IS were compared with equal number of age and sex matched controls. Homocystiene, B12 and folate levels were analyzed as continuous data (Student's T - test) as well as categorical data (Chi-square test). Odds ratio for occurrence of IS were calculated for each variable. Correlation of homocysteine with B12 and folate was studied using Pearson's coefficient. Results: Mean Homocysteine was higher in cases compared to controls (20.49 ± 12.64 versus 13.09 ± 6.96 umol/L, P<0.001, while B12 (282.46 ± 161.52 versus 480 ± 248.92 pg/ml, P<0.001) and folate (10.49 ± 6.86 versus 17.00 ± 5.52 ng/ml, P<0.001) were lower. Both hyperhomocysteinemia (Odds ratio 4.82, 95% CI; 2.28-10.19, P + 0.0003) and Vitamin B12 deficiency (Odds ratio 4.48, 95% CI; 1.99-10.07, P + 0.0003) emerged as significant risk factors for occurrence of Ischemic stroke. Negative correlation of homocysteine with B12 (r-0.457, P < 0.0001) and folate (r -0.337, P=0.0001) was observed. Conclusion: Hyperhomocysteinemia appears to be an important risk factor for IS in Indian population. B12 deficiency is a major acquired cause for the same while folate deficiency is relatively rare.


Mechanical thrombectomy - Our experience

S. Medasani, S. Sitajayalaxmi, E. A. Varalaxmi, P. Yada, S. Mohandas

Department of Neurology, KIMS Hospital, Tela, Uttar Pradesh, India

Introduction: Mr Clean revolutionized ischemic stroke management in patients with large artery occlusion; it ushered in mechanical thrombectomy as the norm for dealing with big ischemic strokes. Objective: We tried to replicate the success of MR CLEAN at our institute. Materilas and Methods: In this study we present our results of patients undergoing mechanical thrombectomy in the past one year from February 2015 to February 2016. Resuts: 6/28 patients with ischemic stroke underwent mechanical thrombectomy, 5 patients underwent thrombolysis followed by thrombectomy, 1 patient underwent only thrombectomy. NIHSS of the patients undergoing thrombectomy ranged from 16-22. Post procedure NIHSS of 4 patients dropped by about 50 % in first two days. m RS of 4 patients undergoing thombectomy was 0-2 at three months period. One patient with CSVT, also underwent mechanical thrombectomy for worsening vision in spite of anticoagulation. Conclusion: Mechanical Thrombectomy is useful in treatment of patients with large vessel occlusion, and we could replicate the results at our institute. The reason for non response in two patients is possibly large area of diffusion restriction and decreased collaterals. The drawbacks are the need of expertise and cost.


"Stroke riskometer app" in Bengali for primary stroke prevention

D. K. Mandal

Department of Neurology, North City Hospital and Neuro Institute, Kolkata, West Bengal, India

While in developed countries the incidence of stroke has declined by 42% over the last 4 decades (1970-2008), its incidence rates in developing countries have increased by >100%. The people in developing countries including India also experience a higher stroke mortality rate, increased morbidity and greater proportions of hemorrhagic ones and, that too in a younger age group, due to lack of proper preventive strategies. Despite shorter life expectancies than in developed countries, 85% of global stroke deaths occur in developing countries. This resulted in huge loss of human and economic resources. The best way to solve the problem is to reduce the incidence of first ever stroke. Recent introduction of Stroke Riskometer app, using mobile technology is considered as the best device for primary stroke prevention. This app can be downloaded in smartphones. About 20 questions are to be answered to know one's risk factors, prediction of stroke in next 5-10 years, and the guidelines of managements of detected risk factors are also outlined (in Pro version). This app is available to more than 5.6 billion peoples globally in about 160 countries in about 13 languages including Bengali and Hhindi for the people of India and Bangladesh. The Bengali version of this app has been just completed, and will be of immense help to the Bengali speaking people of Eastern India and Bangladesh. Details of this Bengali Riskometer app will be discussed in detail as regards the principal modality using smartphones to reduce Primary stroke prevention in Eastern India and Bangladesh.


Factors influencing haemorrhagic transformation after ischemic stroke

S. F. E. Ilahi, G. Sarala, K. Shunmugasundaram, N. Arun Kumar, S. Gobinathan

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

Aim of the Study: The aim of the study was to study the factors influencing haemorrhagic transformation (HT) in acute ischemic stroke and the effect of starting antiplatelet therapy in these patients. Materials and Methods: This study included 32 patients who were admitted to stroke ward in Madras Institute of Neurology with acute ischemic stroke with haemorrhagic transformation on CT Brain. Complete blood count, Liver function tests, Blood sugar, coagulation profile, Microalbuminuria, 2D echo, CT on admission and MRI Brain within 1 week of admission were done for all the patients. Antiplatelet therapy was started for every patient except 2 patients. Aggravation of haemorrhagic transformation was considered as increase of HT within the infarcted area or neurological deterioration of the patient. Results: 32 patients were analysed. Of 32 patients 24 (75%) were male patients and 8 (25%) were female. 10 (31.2%) patients had evidence of atrial fibrillation or rheumatic heart disease on ecg or echo. 30 patients out of 32 had normal liver function tests. 28 patients had normal complete blood count. 20 out of 32 patients were diabetics and 13 out of 32 patients were hypertensives. Coagulation profile was normal in 29 patients. All patients except 2 were started immediately on antiplatelets and monitored for 1 week and later followed up for 1 month. Conclusion: In conclusion, the results suggest that Diabetes mellitus, Embolic stroke to be factors associated with HT. Liver function tests and coagulation profile were insignificant in these patients. Antiplatelet therapy can be safely initiated in patients with HT without any complications.


Artery of percheron infarct: A case report

A. Subbiah, N. Murugapandian, D. Meenal

Department of Neurology, Kilpauk Medical College Hospital, Chennai, Tamil Nadu, India

Aim: Percheron studied Thalamic vascularisation and described 3 anatomical variants. Its occlusion leads to symmetrical para median bithalamic infarcts with or without mid brain involvement. Main symptoms are vigilance disturbances lasting hours to days, pseudo hypersomnia and vertical gaze disorder. I am herewith presenting a rare case of artery of Percheron acute infarct which presented as coma. Case Report: 63 years old male with diabetes and hypertension on treatment was admitted in our hospital in a comatose stage of two days duration. All metabolic parameters were normal. CT Brain taken within 24 hours was reported as normal. Patient had mild fever prior to becoming unconscious and was referred by a General Practitioner as encephalitis. Clinical examination threw no light on the possible cause for coma and fever workup was also not contributory. Fever also was not there since admission to our hospital. MRI brain was taken and it revealed bilateral paramedian acute infarcts. He was given anti edema measures, neuro protective drugs, anti platelet drugs along with his regular anti diabetics and anti hypertensives. CSF analysis was normal. Patient's sensorium improved in 3 days and had hypersomnia for 2 days which also improved and he recovered completely. He was discharged on 6th day and he had no neurological deficit during discharge except tiredness which also improved. Subsequent review of his old CT brain film also showed bilateral thalmic hypo densities. MR Angiogram also did not pickup any abnormal artery as it would be a small artery which could be missed in conventional and CT angiogram even. Conclusion: This case is presented for the rarity, and absence of mid brain involvement which commonly occurs only in less than 15% of cases. The patient was initially misdiagnosed a encephalitis due to mild fever and coma.


Comparision of utilisation of Iv thrombolysis between two time periods by the same study team: A study from South India

N. Chaudhary, M. Reddy, S. Jaiswal, J. M.K. Murthy

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

Background: The utilization of IV-thrombolysis is suboptimal in resource poor countries. These study trends of utilization of IV-thrombolysis at two time periods from south India Materials and Methods: Study site: tertiary care hospital; Study period; period I: January 2008 to December 2010; period II: May 2014 to May 2016. Parameters studied: total number of patients with acute ischemic stroke (AIS), proportion of patients in window period and patients who received IV-thrombolysis, door to image time, image to needle time, door to needle time, complications and reasons for not thrombolysing. MRI/CT was the imaging modality. Results: Data comparison between period I vs. period II: mean age: 64.22 ±11.84 vs. 62.27 ±13.87; male: female: 41:9 vs. 54:31; patients arriving within window: period: 65/641 (10%) vs. 100/443 (22.5%); patients thrombolysed: 50 (7.1%) vs. 85 (19.1 %); mean NIHSS: 12.54+3.14 vs. 7.52 +4.41; mean time door-to-image: 9.5 + 2.08 vs. 17.62 + 18.55 minutes; mean door-to-needle time: 60.00 + 12.49 vs. 47.35 + 27.01; mean image-to-needle time: 51.5+10.41 vs. 30.02 + 14.18; poor outcome (mRS 5-6): 7(14%) vs. 4 (4.7 %); and non-thrombolysed: (21.54%) vs. 15 (15%). Reasons for non-thrombolysis: poor purchasing capacity: 12 (24%) vs. 8 (8 %); contraindications 3 (6%) vs. 7 (7 %); complications: sICH 2 (4%) vs. 3 (3%). Conclisions: This study shows positive trends in regard to patients presenting within window period, improvement in door to needle time, and improvement in the purchasing capacity of the patients in this part of the world.


Factors associated with delayed diagnosis and misdiagnosis in patients with migraine

N. K. Rai, R. Bitswa, R. Singh, D. S. Parahua

Department of Neurology, AIIMS, Bhopal, Madhya Pradesh, India

Introduction: Migraine is one of the most common chronic debilitating disorders, affecting 10-20% of the world's population. Sometimes, proper diagnosis is missed or delayed due to lack of awareness of treating primary physicians. Objective: To determine various factors associated with delayed diagnosis of migraine. Methods: Patients attending Neurology OPD of AIIMS Bhopal and satisfying diagnostic criteria of the International Headache Society (ICHD-II) for migraine were selected for study. Detailed clinic-epidemiological including data MIDAS and HIT-6 were collected. Associated Depression and anxiety was detected by using MINI, whereas ROME-III for functional GI disorders. First diagnosis was considered as "Appropriate" if patients would be aware about his diagnosis "migraine" or previously recorded as "migraine" otherwise labelled as "Inappropriate". Expenditures were calculated on the basis of patients reporting and was tried to justify by evaluating available investigations. Results: Hundred patients with clinical diagnosis of migraine were included in the study. Mean age (SD) was 32.42 (10.74) and number of female patients were 77. Median duration of illness (range) was 48 (3; 360) months. Diagnosis was "inappropriate" in 65 patients (65%). Number of inappropriate diagnosis were 10 (25%) out of 40 by neurologists; 35 (89.3%) out of 39 patients by physicians; 18 (100%) out of 18 patients by ophthalmologists and 1 (33%) out of 3 patients by AYUSH doctors. Factors associated with "Appropriate Diagnosis" were "Neurologist Vs Other Doctors" {30 (75%) Vs 5 (8.3%), p<0.001}; throbbing headache Vs other types of headache {33 (39.3%) Vs 2(12.5%), p=0.047} and temporal localisation Vs other sites of headache (frontal, occipital, orbital etc) of headache {12 (57.1%) Vs 23 (29.1%), p=0.017}. Patients with "Inappropriate Diagnosis" had delayed presentation, {60 (3, 360) months Vs 24 (6, 240) months, p=0.02}; and expended more money {7000 (1,500; 65000) INR Vs 4000 (500, 49500) INR, p<0.01}. HIT was high also high them {59.48 (10.37) Vs 54.94 (10.99), p=0.04}. Other clinical parameters including vertigo, cervical pain, anxiety, depression and functional gastrointestinal symptoms were not associated. Conclusion: Delayed diagnosis and misdiagnosis is very frequent in migraine, especially by a non-neurologist. It has significant personal, social and financial impact. Like migraine; other common disorders presenting in "OPD" need to be evaluated for delayed diagnosis and misdiagnosis.


Hemiplegic cluster headache: A typical presentation

A. Dave, S. Prakash, C. Rathore, P. Makwana, S. Shah

Department of Neurology, Smt B K Shah Medical Institute & Research Centre, Vadodara, Gujarat, India

Introduction: Cluster headache (CH) is a form of Trigeminal Autonomic Cephalalgia which is characterised by strictly unilateral headache with autonomic symptoms and restlessness. We hereby report a young male with CH in whom attacks were accompanied by transient hemiparesis. A 41-year old male had a 4-year history of severe excruciating episodic headaches on the left side. The duration of headache attacks was 30-90 minutes. Most episodes were accompanied by ipsilateral lacrimation, conjunctival-injection and rhinorrhoea. Restlessness was also present during attacks. The patient fulfilled the ICHD-3β criteria for episodic CH. The cluster period lasted for 3-4 months, with remission phase of 6-8 months. Approximately one-third attacks were accompanied by left-sided hemiparesis. Hemiparesis used to start during or immediately after the onset of headache attacks. The duration of hemiparesis was 2-24 hours. The patient was investigated on many occasions for the possible cerebrovascular accident and other causes. The patient was put on lithium as a preventive measure and oxygen inhalation for acute attacks. The patient responded to lithium and the frequency of headache attacks reduced markedly. Even the frequency of associated hemiparesis reduced greatly. Oxygen inhalation had positive effect in both headache and hemiparesis. The average duration of hemiparesis reduced significantly (less than 60 minutes) with oxygen inhalation. Discussion: Siow et al. (2002) described four cases of CH with accompanying hemiparesis that was ipsilateral to the pain in two patients and contralateral to the pain in one with the fourth patient having ipsilateral facial and contralateral hemiparesis. Yang et al. reported a similar case in 2012. The average duration of hemiparesis was 2 to 24 hours. Conclusion: This case report reveals unique presentation of CH having hemiparesis as associated symptom, which is commonly seen with migraine (First case from India).


Neurology and MRI findings in IIH

A. Sivakumar, Kannan

Department of Neurology, RGGGH, Chennai, Tamil Nadu, India

Introduction: Idiopathic intracranial hypertension (IIH) is a headache syndrome of unknown etiology characterized by raised CSF pressure in the absence of an intracranial mass lesion or ventricular dilation. hR predominantly affects women of child bearing age. Normal neurologic examination with papilledema on fundus examination of the eye is common. Methods: Patients diagnosed as study subjects with IIH were recruited according to the diagnostic criteria. All patients underwent MRI with MR venography to exclude venous sinus thrombosis or structural CNS lesions. Clinical, radiological imaging features of IIH of 27 patients were studied. Other causes of ICT intracranial tension were excluded. Secondary clinical features like headache were excluded. Secondary clinical features like Head ache were prominent. MRI findings were studied regarding pituitary and optic nerve. Results: A total of 27 cases were analysed during the period of July 2015 - June 2016. Frontal and Horocranial headache 37%, visual obstrurations and blurring of vision in 81.8%, Cranial nerve palsy (6 th and Facial Nerve) 1.6%, Bilateral papilloedema- 51.8%, MRI Findings- 51.9%, CSF Monometry showed elevated CSF pressure in all patients, MRI suggestive of IIH were seen in 51.9%, Perioptic space widening is (more common finding) 35%. Conclusion: No specific mortality risk is there in IIH. Obesity has a selective role in this group because of the strong prediction for female, fertile, obese patients; it is possible that optic patients, it is possible that optic nerve head change could predict future visual outcome n IIH patients regarding the visual loss. Patients are instructed to return to the neurologist when symptoms recur.


Intractable headache in a patient with carcinoma breast: Infiltrative or infective - A diagnostic challenge

P. K. Maurya, D. Kulshreshtha, A. K. Singh, S. Shukla

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

Introduction: Tubercular meningitis continues to be an important cause of chronic meningitis in our subcontinent and empiric anti tubercular therapy is started awaiting AFB confirmation. Carcinomatous meningitis (CM) is infiltration of leptomeninges by malignant cells and is reported in 5% of cases of breast cancer. The condition warrants a high degree of suspicion to ascertain the diagnosis. We report a patient of breast carcinoma presenting with intractable headache misdiagnosed as tubercular meningitis and diagnostic difficulties encountered. Case Report: A 36-year old lady presented with low grade fever with progressively worsening holocranial headache of 2 months duration associated with nausea and vomiting with 2 episodes of GTCS. She was operated 1 year back for carcinoma breast (stageT3N1M0) and was on oral Tamoxifen. She was evaluated elsewhere for recent complaints and MRI brain was normal while cerebrospinal fluid (CSF) revealed lymphocytic pleocytosis with elevated protein and normal sugars. She was started on anti tubercular treatment and steroids but headache continued to worsen. Her fundus was normal and signs of meningeal irritation were positive. A repeat CSF examination showed elevated CSF pressure with low sugars and normal cells and proteins. CSF ADA/CALAS/Gene Xpert test for MTB were negative. A possibility of Tamoxifen induced intracranial hypertension was considered but headache persisted on its withdrawal. Hence, with a high possibility of carcinomatous meningitis, a repeat lumbar puncture with large volume CSF was analyzed showed positivity for malignant cells. There was a small nodular enhancing lesion in left cerebellum on a repeat MRI brain. She was treated with chemotherapy and her headache improved. Conclusion: Our case highlights the diagnostic difficulties in cases of chronic headache in a setting of malignancies and importance of a large volume CSF and repeated lumbar punctures to achieve the correct diagnosis of carcinomatous meningitis which is crucial for optimal treatment and prognosis.


what triggers you? A study of migraine triggers in a tertiary care centre

P. S. Kumar, V. Kannan, J. Manickavasagam, S. Gobinathan, S. Balasubramanian, L. R. Narasimhan

Department of Neurology, Madras Institute of Neurology, MMC, Chennai, Tamil Nadu.

Introduction: Migraine is a primary headache that may be triggered by exposure to a potential 'trigger'. Methods: Migraine triggers in 111 patients with migraine who attended the headache clinic at the Madras Institute of Neurology were documented by a questionnaire approach. The questionnaire included 22 sets of migraine triggers. Results: Stress (45%) was identified to be the most common trigger followed by weather changes (20.7%) and cold drinks (15.3%) as possible triggers. 10.8% were unaware of any possible trigger and most were not aware of other triggers. All patients were educated about all possible triggers and were advised to maintain a headache diary. Conclusion: It is important to identify these triggers and avoidance of these triggers reduces the frequency of attacks. Management of stress will help in reducing the morbidity of headaches.


To evaluate brainstem dysfuntion in headache using the blink reflex

D. Joshi, A. Pathak, S. Kawale, S. Chaudhary, V. N. Mishra, R. N. Chaurasia

Department of Neurology, IMS, Bhuvneshwar, Odisha, India

Aims: Blink reflex is a non-invasive method that can provide information on peripheral and central neurological functions of trigemino-vascular system. Present study aimed to evaluate brainstem dysfunction in primary headache patients using the blink reflex. Materials and Methods: The present study was conducted on a cohort of 75 patients of primary headache at a tertiary care referral centre. Of the seventy five patients of primary headache there were (51) patients of migraine (group I), (16) tension type headache, (groupII), (8) cluster headache (group III) and 70 control patients group (IV). They were examined clinically and were subsequently evaluated using the blink reflex in the headache free period. Results: R1 response latency on both sides were not significantly prolonged as compared to controls whereas the R2 responses on both sides (R2i & R2c ) were significantly prolonged in all headache patients (p<0.001) as compared to controls. Also an intergroup comparison of all 3 groups with controls and a comparison of each headache subtype with control also revealed a significantly prolonged R2 i and R2 c (p<.0.001) while the R1 latencies were normal in all groups as compared to controls. Conclusion: Thus our study revealed prolongation of the R2i & R2c latencies in headache patients. This shows that the trigeminal afferents are altered in headache patients thus demonstrating an abnormality in the central nociceptive system which were present even during the interictal period.


A study on headache attributed psychiatric disorder

S. Murugaiyan, N. Shanmugasundaram

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

Introduction: Psychogenic headache is produced or caused by mental or emotional factors like stress, life events or psychosis. Aim: To study the psychological problems causing or influencing headaches. Methods: 100 headache Patients were randomly selected from neurology OPD, government general hospital, madras medical college Chennai, from December 2015 to june 2016 with normal investigation parameters and normal CT/MRI IMAGING with exclution of primary headaches, who are falling on criteria classified under the International Classification of Headache Disorders, 3 rd edition (beta version}- (IHS) 12. Headache attributed to psychiatric disorder. 12.1 Headache attributed to somatization disorder. 12.2 Headache attributed to psychotic disorder. These patients subjected for structured psychiatric clinical interview with ICD-10 mental and behavioural disorder and DSM-5 criteria. HAM-A, HAM-D, BDI-2, panic and delusional rating scales were administered. Results and Discussion: The study included 76% female 24%male population. Age group between 15- 47 years. Literacy rate range from illiterate 5% to 95% high school and degree level. 62 % were employed, 10% were student and 28% were not employed. 72% (55 female 17 male) showed significant score on rating scales. 65% (46.2) on HAM-D (82% scores 8-13 points-mild depression. 18% scores 14-18 points-moderate depression}. On BDI-2 62% scores 14-29 points. 33% (25.2) on HAM-A scores 18-24 points. 1% on panic rating scale and 1% on delutional rating scale. Based on ICD-10 and DSM-5 criteria and various clinical assessment scales the following psychiatric diagnostic entities were arrived. Depressive episodes-22 (30.5%), Dysthymia-5 (6.9%), somatization disorder-4 (5.5%), persistant somatoform pain disorder includes psychogenic headaches-15 (20.8%), Illness anxiety disorder-1 (1.38%), Acute stress disorder -11 (15.2%), Generalized anxiety disorder-5 (6.9%), Mixed anxiety and depressive disorder-7 (9.7%), panic disorder-1 (1.38%) and major depressive disorder with psychotic features-1 (1.38%). These may contribute or cause headache or aggravate existing headache. Structured psychiatric clinical interview revealed an Intensive, agonizing pain, often provoked by stress, psycho-social factors. The pain occurs most commonly in depression, dysthymia, somatoform, stress and anxiety disorders. Conclusion: Early identification and treatment of psychiatric condition is important for the proper management of these headaches A detailed evaluation by an experienced psychiatrist or psychologist is mandatory in treatment non responsive headaches.


Do episodic migraine patients also have cranial autonomic dysfunctions? A prospective tertiary care headache clinic study from North India

A. S. Singh, D. Chowdhury, G. A. Khwaza

Department of Neurology, GIPMER, JLN Marg, New Delhi, India

Background: Unlike trigeminal autonomic cephalalgia, cranial autonomic symptoms/signs (CAS) have not been studied well in migraine. Whether migraine patients with CAS differ from those without in terms of age, gender, attack frequency, duration, and severity is largely unknown. Aim: Occurrence and spectrum of CAS in episodic migraine and compare them with those without. Materials and Methods: Episodic migraine patients attending headache clinic from August 2014 to June 2016 were prospectively diagnosed (by ICHD 3β) and evaluated using a structured proforma. 10 CAS studied include lacrimation, conjunctival injection, eyelid edema, nasal congestion, rhinorrhea, facial/forehead sweating, facial/forehead flushing, drooping of the eyelid, aural fullness and miosis. Results: Demographics: 89 patients, mean age of 32 years (range 11-51), 81F:8M were studied. 9 had migraine with visual aura. 73% had holo-cranial headache, 15.7% side-locked headache and 11.3% side-shifting headache. Nausea (81%), vomiting (34%), photophobia (95%), photophobia (97%) and inhibition of activity (98%) were present. CAS Occurrence and Distribution: 49 (55%) had one or more CAS. Of these, 69.4% had bilateral while 30.6% had ipsilateral CA. Lacrimation was the commonest CAS (73.5%) followed by conjunctival injection (46.9%), aural fullness (40.8%), eyelid edema (28.6%), facial/forehead flushing (24.4%), facial/forehead sweating (22.4%), drooping of eyelid (12.2%), nasal congestion (4.1%), rhinorrhea (4.1%) and miosis in none. CAS Spectrum: Out of 10 CAS, 1 feature alone in 14 (15.7%) while combination of 2-7 features were seen in 15 (16.8%), 6 (6.7%), 8 (8.9%), 5 (5.6%) and 1 (1.1%) patients respectively. Comparison to those without CAS: No difference noted between those with CAS and those without regarding age, gender, attack frequency, duration, and severity. Conclusions: CAS is common in migraine. Mostly these are bilateral. Lacrimation and conjuctival injection were the commonest, occurring alone or in combination. CAS is not related to age, gender and other headache characteristics in migraine.


Clinical profile, aura characteristics, treatment outcome in patients of migraine with aura and their comparison with migraine without aura

A. Gupta, D. Chowdhury, G. A. Khwaja

Department of Neurology, Jawaharlal Institute of Postgraduate Medical Education & Research, New Delhi, India

Background: Migraine with aura (MA) is relatively uncommon. Few reports are available from India. A comprehensive evaluation will be useful. Aims and Objectives: To analyze clinical profile and treatment outcome in MA and compare them with migraine without aura (MO). Methods: 50 consecutive MA patients attending headache clinic were prospectively diagnosed (ICHD 3β), comprehensively evaluated by structured proforma and compared with 50 consecutive MO patients. Results: Demographics: Mean age of presentation: 30.6 years (range 12-60yrs), 12M:38F. 90% episodic, 10% chronic migraine. Mean illness duration 94.3 days. Headache Characteristics: The mean attack duration, attack frequency and severity were 23 hours, 8.7/month, VAS 7.5 respectively. 68% had holo-cranial, 14% side-locked and 18% hemi-cranial headaches. Throbbing character (86%), nausea (49%), vomiting (48%), photophobia (98%), phonophobia (90%), inhibition of activity (56%), allodynia (14%), vertigo (8%), dysautonomia (24%) was seen. Aura Characteristics: Visual auras (90%) were commonest and included scintillating scotomas (10%), fortification spectra (30%) flashes of bright-coloured light (50%) visual hallucinations (10%), blurred vision (24%), halos (12%), obscurations (4%) and micropsia (4%). Brainstem auras (8%) consisted of vertigo, ataxia, dysarthia. Sensory aura (2%) consisted of tingling from shoulder region to hand. Mean aura duration was 18.3 minutes. None presented with isolated auras. Aura preceded headache in 90%. Treatment Outcome: 64% received flunarizine, while rest received propanolol, topiramate and amitriptyline. At 3 months, 14% had complete remission, 70% > 50% reduction in attack frequency and 16% did not respond. Comparison with MO : None of the demographic and clinical features differed between the groups except that MA patients had more severe headaches (mean VAS7.5 vs 7.0; p = 0.03) and lesser vertigo (8% vs 32%; p =0.006). Conclusions: Visual auras were the commonest. 84% responded to treatment especially flunarizine. MA and MO probably share same patho-physiology as no significant differences between them were found.


Facial anaesthesia matters: A rare case of unilateral congenital trigeminal hypoplasia

P. S. Kumar, R. Y. Kalpana

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

Isolated unilateral corneal anesthesia is a rare clinical entity. A 20 year old lady was referred from ophthalmology department with conjunctival injection of left eye. History revealed recurrent episodes of painless redness of left eye since about 4 years of age. No history to suggest trauma, seasonal variation, allergies, oral ulcers, arthralgia, skin rashes or any infections. Examination revealed no corneal sensitivity on the left side and the left sided facial sensations were reduced in the distribution of all branches of trigeminal nerve. Corneal reflex was absent on left side- both direct and indirect. No other deficits were noted. Formal ophthalmic evaluation was otherwise normal. MRI brain showed left trigeminal nerve hypoplasia. Due to the rarity of this presentation we report this case. In cases with painless intermittent keratoconjunctivitis we should consider acquired or congenital trigeminal involvement which includes trigeminal nerve hypoplasia. This condition requires lifelong corneal ulcer prophylaxis and regular ophthalmologic checkups.


A prospective comprehensive study of a rare primary headache disorder: 10 cases of hemicrania continua

R. Chopra, D. Chowdhury, G. A. Khwaja

Department of Neurology, GB Pant Hospital, New Delhi, India

Background: There is scarce data on Indian patients suffering from Hemicrania Continua (HC). Objective: To assess clinical features, psychiatric co-morbidities & treatment outcome in HC. Methods: Patients were prospectively diagnosed (by ICHD 3β) and evaluated as per a structured proforma covering demographic details, clinical characteristics & psychiatric co-morbidities. Treatment outcome was evaluated at 3 months. Results: Demographics: 5 were remitting HC and 5 were unremitting HC subtypes. 80% were definite and 20% were probable HC (fulfilled all but 1 criterion). Mean age at diagnosis was 48 years (range 37-63); sex ratio 5M: 5F. Mean diagnostic delay was 5 years (median 4.5). All male patients were smokers and 20% consumed alcohol. Family history of headache found in 40% but none had HC. Headache Characteristics: Pain located in orbito-temporal region (60%); character was throbbing 80%. Headache was side-locked in 90%. Background continuous headache was mild (mean VAS 3.4). All had exacerbations; 4-5 hours in 40%, whole day in 50% and more than 1 day in 30%. Exacerbations occured 1-2/day to 1/week. Exacerbations were painful (mean VAS 8.5). Commonest triggers were body movements and emotional stress (70% each). Nocturnal exacerbations were seen in 40%. Of migrainous symptoms, ipsilateral photophobia was the commonest (60%). None experienced aura. Cranial Autonomic symptoms (CAS): Present in 100%. Ipsilateral eyelid-edema was commonest (70%) followed by lacrimation (60%). Behavioural restlessness (motor and psychological) reported by all during exacerbations. Co-morbidities: Psychiatric co-morbidities seen in 70% patients: depression (70%), somatization disorder (50%), anxiety (30%), and panic attack (10%). Suicidal thoughts were present in 50%. Outcome: 8 responded to indomethacin (150-225 mg/d) completely within a week while 2 didn't. These 2 patients showed significant but incomplete relief to Etarocoxib (120 mg/d). Conclusion: HC is commonly misdiagnosed as chronic migraine. Side-locked continuous headache with exacerbations, ipsilateral dysautonomia, restlessness and absolute response to indomethacin is characteristic.


A study on the effect of prophylactic treatment of migraine on sleep in South Indian population

K. T. Periyasamy, G. Sarala, K. Venkatraman, G. Kaushik, K. Shanmugasundaram, S. Gobinathan

Department of Neurology, Madras Institute of Neurology (MMC), Chennai, Tamil Nadu, India

Background: Migraine, a type of primary headache syndrome has several subtypes, multiple co morbities and variable trigger factors. Sleep disturbance is considered as one of the most common causes of migraine. Headache and sleep have a close relationship since sleep disturbance may trigger headache and headache in turn produce sleep abnormality. Study: 50 patients diagnosed with migraine headache were included in the study. Baseline visual analogue pain scale and Pittsburgh sleep quality index (PSQI) scores were obtained. The baseline visual analogue pain scale was 7.74 and PSQI score was 7.23. Poor quality of sleep was identified in 74% of the patients. Patients received prophylactic treatment with either Amitryptalline or Propronolol. The patients were followed up for a period of 12 weeks. At the end of 12 weeks patients had improvement in both visual analogue pain scale scores and PSQI scores. Conclusion: This study shows that in patients with migraine there is higher prevalence of poor quality of sleep and addressing this issue may result in improvement in migraine treatment.


Bad headache journeys

T. P. Arulnambi, L. R. Narasimhan, S. Gobinathan

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

Introduction: Airplane Headache is headache occurring exclusively on air travel. Inconsistent variation in the cabin pressure affected by the ambient pressure changes during taking off and landing is one contributing factor though the pathogenesis is complex. Case Report 1: A 45 yr old female business executive with no prior comorbities was a frequent flyer over the last few years. Now for the past 2 years she reported headache exclusively while travelling by airplane. It was left sided shearing retroorbital and frontal pain not accompanied by nausea, photophobia, phonophobia occurring 45 minutes prior to landing which subsided 2 hours after landing spontaneously. Her headache consistency continued to remain the same. Neurological examination and imaging normal. ENT opinon during these attacks were normal. Case Report 2: A 50 yr old male politician developed bilateral frontal and retro orbital pain 30 minutes after taking off and 45 minutes before landing occurring only during air travel. The headache subsided after half hour of landing. He is a diabetic, normotensive, alcoholic, and nonsmoker. Neurological examination and imaging normal. Self medicated with paracetamol relieved the pain. Conclusion: With increasing international and domestic travels the emotional impact of pain incapacitates the travel schedule of individuals. Awareness among the flying class about this entity avoides the discomfort of travel and their work schedule. Triggering factors and preventive measures should be undertaken for better performance.


Clinical profile and treatment outcome of migraine in children and adolescents: A 2 years follow up observational study from North India

A. Srivastava, D. Chowdhury, G. Khwaja

Department of Neurology, GB Pant Hospital, New Delhi, India

Background: Migraine in children and adolescents has distinct clinical profile. Few studies available from India involved small number of patients. A large cohort study comprehensively detailing headache characteristics, unusual syndromes and treatment outcomes will be useful. Objective: To elaborate the spectrum of clinical features and treatment outcome of migraine in children and adolescents (<18 yrs). Methods: The clinical profile and treatment outcomes of patients (<18 yrs) with migraine (diagnosed by ICHD3β) attending headache clinic were studied prospectively using a structured proforma over 2-year period. Results: 250 consecutive patients (<18 yrs) had a mean age of headache onset at 11.8±3.3 years. Diagnostic delay was of 2.1 years. Migraine was commoner in girls (54% vs 46%). However, more boys <12 years had migraine than girls. Mean attack duration was 10.68±10.12 hours but 15% had headache only up-to 2 hours. 53.6% had holocranial, 21.6% hemicranial, 17.6% hemi-holocranial, and 7.2% had side-locked headaches. Majority (84%) had severe headaches (VAS ≥6). 63.6% had a first-degree relative with migraine. Aura was present in 7.2%. 2.4% had episodic syndromes (abdominal migraine, cyclical vomiting). Mean attack frequency was 11.04±9.3 days/month. 28% had chronic migraine and 6% medication-overuse headache at presentation. Commonest trigger was sunlight exposure (30.4%) followed by stress (20.8%). 96% were treated with preventives. 80% received monotherapy. 78.4% had significantly reduced headache frequency (>50%) by 3 months while 21.6% failed to respond. By 6 months, only 10% could be withdrawn from preventives. Higher attack frequency was an independent predictor of poor outcome. Conclusions: Diagnostic delay of 2 years resulted in suffering of many children and adolescents with migraine. 15% patients did not fulfill ICHD3β 'duration' criteria and more than 50% had holocranial headaches. Majority (90%) needed to continue long-term preventives despite good response. As higher attack frequency predicted poor outcome, the need for early diagnosis and treatment can't be overemphasized.


ECG changes in patients with migraine

M. Kilji, S. Gobinathan, S. Balasubramanian

Department of Neurology, Institute of Neurology, MMC, Chennai, Tamil Nadu, India

Background: Autonomic system involvement (both decreased parasympathetic and increased sympathetic activity) responsible for many clinical features of migraine leads to derangement of circulatory system and autonomic balance. This imbalance causes atrial and ventricular repolarisation abnormalities and arrhythmias. These marked autonomic derangements during ictal period produces changes within heart and coronary vessels which may cause ECG abnormalities in the form of cQT interval and P-wave changes which may be predictors of atrial and ventricular arrhythmias. Aim: To assess of ECG changes in migraine both during attack and pain free periods. Methods: Patients with migraine were recruited according to International Headache Society classification from Madras institute of neurology, India. ECG variables of heart rate, Abnormalities of rhythm, P wave duration, PR interval, QRS duration, QT interval, corrected QT interval (cQT), ST changes and T wave inversion were analysed in migraine patients both during ictal and nonictal periods. Results: Out of 50 patients with migraine (males(12%), females (88%), 34 ictal (68%),16 nonictal (32%)), 4 had arrhythmias in the form of ventricular premature complexes (8%), 26 had T wave Inversion (52%), 19 had ST changes (38%), 21 had abnormal P wave duration > 0.10 sec (42%),4had abnormal cQT interval (8%) and 1 had abnormal PR interval of >20 sec (2%). In ictal group, 17 patients (50%) had abnormal ST changes, 23 (67.6%) had abnormal T inversion and 13 (38.2%) had abnormal P wave duration. In non ictal group, 2 (12.5%) had ST changes, 3 (18.8%) had T inversion and 8 (50%) had abnormal P wave duration. Only values in the ictal group for ST changes and T wave inversion were statistically significant (P value 0.011, 0.001). Conclusions: We conclude that ECG abnormalities are often noted in migraine patients. Further studies are needed to correlate ECG manifestations and occurrence of arrhythmias and related manifestations.


Pseudotumor cerebri syndrome: A rare complication of drug for medical abortion

S. K. Chaudhary, D. Joshi, R. Singh, S. Sagar, R. Kawale, A. Kumar, R. R. Pushkar

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

Background: Though Pseudotumor cerebri syndrome is a relatively common and underdiagnosed cause of headache and visual disturbances in young and middle aged female, it's association with drug for medical abortion is rare. Case Report: We describe a lady who presented with Pseudotumor Cerebri Syndrome, after intake of drug easily available for medical abortion (kit containing mifepristone and misoprostol). A 26 year married lady presented with moderate to severe frontal predominant headache associated with nausea, vomiting and decreased vision in both eyes of one month duration with negative history of fever and headache in the past. Fifteen days prior to onset of headache and visual disturbances, she has taken drug, easily available in kit formulation containing Mifepristone and Misoprostol. On examination the lady was of normal built, conscious, well oriented to time, place and person. General examination was normal except mild pallor. Systemic examination was normal except decreased visual acuity and bilateral papilledema. Routine investigations including blood sugar, renal, liver, thyroid function tests, calcium, and phosphorus and collagen profile were normal except mildly decreased haemoglobin. MRI-Brain with MR venogram was normal. A diagnostic lumbar puncture demonstrated an elevated opening pressure of 290 mm of water in lateral decubitus with normal CSF analysis. She received pulse therapy of methyl prednisolone followed by short tapering course of oral steroid and acetazolamide with significant improvement in her headache and visual complaint. Conclusion: We report a case that highlight the rare occurrence of PTCS with this drug, though levonorgestrel, used for post coital emergency contraception has been listed in drugs causing this disorder.


A tale of two headaches-migraine with tubercular meningoencephalitis with CVST

S. D. Nayak, B. K. Mohanty, A. K. Mallick, G. Mohanty, K. P. Swain, M. Samanta

Department of Neurology, IMS & SUM Hospital, Bhuvneshwar, Odisha, India

TB is the most important etiology of chronic meningoencephalitis in India. Several infectious etiologies are associated with cerebral venous thrombosis but only few cases have been reported with TB meningoencephalitis. We hereby report a 38 years old female who presented with 2 months history of fever, headache, recurrent vomiting, followed by vision loss, seizures and altered sensorium. There was change in the pattern of headache from primary (migraine) to secondary type. On evaluation CSF analysis corroborated with pictures of TB meningitis. MRI with MRV and MRA revealed left pan-sinus thrombosis and non-communicating hydrocephalus due to aqueductal stenosis-post inflammatory sequelae (TBM). She was evaluated for other procoagulant defects but results were negative. Patient underwent a rapid downhill course with development of loss of vision and DVT and ultimately death even after treatment with antiepileptics, antitubercular, anticoagulants and steroids. We should be watchful for conversion of primary headache disorders to secondary headache disorders. AssociatIon of TB meningoencephalitis with CVST is very rare and associated with high fatality if not treated easily.


Medically refractory trigeminal autonomic cephalalgia: Diagnostic and therapeutic challenge, when no clear cut line exists among them!

A. K. Singh, D. Kulshreshtha, P. K. Maurya, A. K. Thacker, M. D. Thakkar, A. Tiwari

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

Introduction: Trigeminal-autonomic cephalalgias (TAC's) are rare but very disabling conditions with a major impact on the patient's quality of life. Primary short lasting headaches with prominent autonomic features includes cluster headache, paroxysmal hemicranias, SUNCT and SUNA and with sparse or no autonomic features includes trigeminal neuralgia, primary stabbing, cough, exertional, hypnic and headache associated with sexual activity. Diagnosis of TAC's is based on diagnostic criteria by the international classification of headache disorders (ICHD) - three beta. Case Report: A 48 year old male patient presented with Right hemicranial continuouos moderate dull aching with episodic severe pricking headache lasting 5-10min, 40-60 times/day since 3 weeks associated with ipsilateral lacrimation, conjunctival injection without nausea vomiting, photophobia, phonophobia, reliving/aggravating factor or trigger point. History of similar symptoms 3 years back for 6 months unresponsive to anti migraine treatment. Secondary causes were ruled out. Patient was treated step by step with adequate doses and duration of different medications considering primary TAC's, with Indomethacin, Verapamil, High flow Oxygen inhalation, Injection Methylprednisolone, Lamotrigine, Topiramate, Gabapentin, Carbamazepine and Lignocaine. Patient was discharged with the diagnosis of Hemicrania Continua and SUNA with advice of Topiramate and Lamotrigine and Indomethacin. However patient was having modest response in headache severity without reduction in headache frequency. Conclusion: When there is more than one type of headache in the same patient, both type of headache needs to be treated simultaneously, sometimes it becomes difficult to diagnose and misdiagnosis is common. While managing patients of headache with overlapping features, stepwise consideration and rule of exclusion with patience is very important Still the point is debatable that delay in making diagnosis of headache and initial mismanagement make the simple headaches refractory or the refractoriness is inherent.


Cell phone associated headache: Is it new variant of chronic daily headache?

D. D. Ruikar, S. Mohandas, S. S. Jayalakshmi

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

Aims and Objectives: health related issue of mobile or cell phone are topic of debate and under active evaluation. There is hardly any data on headache associated with cellphones. We present case report of atypical cases who reported chronic headache related with cell phone use. Methods: This is case report of 4 patients from single Center from 2013 to 2016 followed prospectively for 6 months on opd basis. There MRI brain and all other parameters were normal and no evidence of secondary headache etiology. Results: During 2013 to 2016 we came across four patients who had stereotypical pattern of headache related to mobile or cell phone use. They reported use of mobile more than 6 hours per day for more than 3 years on regular basis. They reported headache more than fifteen days per month for more than six months associated with almost exclusively related to use of cell phone. Two reported with use of hands free device also. There was no history of Naushera, photo or photophobia. Dull aching headache satisfying IHS chronic daily headache criteria. Two reported starting associated with stress as wel and two denied any underlying stress. There was no significant relief with routine treatment and councelling. Discussions: Use of mobile or cell phone is part of everyday life for most of adults in India. Excessive use of cell phone with possible underlying stress may trigger form of chronic daily headache in few patients. They are tough to treat as wel. This entity needs further evaluation, follow up and more assessment. In chronic headache patient use and association of cell phone should be actively searched for.


Posterior reversible encephalopathy syndrome - A diagnosis of unity in diversity

R. Telugu, S. Sitajayalaxmi, E. A. Varalaxmi, P. Yada, S. Mohandas

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

Objective: To identify the clinical associations and radiologic findings of posterior reversible encephalopathy syndrome (PRES). Patients and Methods: Patients prospectively diagnosed as having PRES (Typical/Atypical) were pooled with retrospectively identified patients admitted from October 2015 to June 2016 in KIMS, Hyderabad. We performed a detailed review of clinical information, including demographics, presenting symptoms, medical history, and risk factors. All patients underwent magnetic resonance imaging. Findings on magnetic resonance imaging were analysed independently by neurologist & neuroradiologist. Results: We identified 13 cases of PRES from October 2015 to June 2016 (9 months). Minimum Age is 9 yrs and maximum age 82 yrs. Mean peak systolic blood pressure was 155 mm Hg (minimum-maximum, 110-200 mm Hg), and mean peak diastolic blood pressure was 90 mm Hg (minimum-maximum, 70-110 mm Hg). Etiologies of PRES included hypertension (n=2 (15%)), sepsis (n=2 (15%)), preeclampsia or eclampsia (n=5 (38 %)), scorpion sting (n=1 (7%)), autoimmune (n=3 (23%)). Clinical presentations included seizures (n=9(69%)), encephalopathy (n=3 (23%)), headache (n=2 (15%)), and visual disturbances (n=1 (7%)). In the 13 cases for which magnetic resonance imaging findings were available, the parieto-occipital regions were the most commonly involved (n= 13), followed by the frontal lobe (n=12), temporal lobe (n=3), and cerebellum (n=5), splenium(n=2), basal ganglia(n=2), thalamus(n=4), brainstem(n=2), internal capsule(n=1). Conclusion: In all the varied causes of PRES, the combination of seizures and high blood pressures without focal deficit should raise the suspicion of PRES and preferably MRI should be done for accurate diagnosis and to avoid unnecessary investigations.

Poster Session 08; Neuromuscular Disorders16:30-19:30 h, Saturday, November 12, 2016


Motor neuron disease: Its unusual form

K. G. Roopa, S. Hegde, Udaymurgod, A. K. Roy

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

Introduction: MND is a progressive degenerative disorder of upper and lower motor neurons leading to progressive weakness of bulbar and spinal muscles with relative sparing of extra ocular muscles and sphincter function. In addition to classical MNDs many unusual forms have been described. Kennedy's disease (KD) is a rare inherited disorder characterized by degeneration of both motor and sensory neurons. Phenotypically, patients present with proximal and distal weakness and wasting of the facial, bulbar and limb muscles with occasional sensory and endocrinologic disturbances. The golden standard for diagnosis is genetic analysis, demonstrating increased CAG-repeat. Materials and Methods: Mr. RJ, 64yrs old healthcare professional presented with history of progressive bulbar and predominant proximal limb weakness since 25 years. Proximal muscle weakness was preceded by tremors of hands, bulbar muscles weakness and muscle cramps on excertion. Fasciculations of tongue, facial and cervical muscles are prominent and also has significant wasting of proximal and distal upper and proximal lower limbs muscles. Patient has gynaecomastia and Diabetes mellitus type II. Mr. RJ has one affected member, one carrier and a chance carrier in his family. NCS showed reduced CMAP and SNAP amplitude without sensory impairment. ENMG showed characteristic features of denervation. Plasma creatine kinase levels were high initially which was later normalized. Genetic analysis is suggestive of the disease. Discussion: Mr. RJ has an adult-onset, X-linked recessive disorder, caused by expansion of a polymorphic CAG tandem-repeat of the androgen-receptor (AR) gene. This phenotype in its full form is only expressed in males and females are only carriers. Early diagnosis may be helpful for family counselling.


A family with congenital myasthenia syndrome

B. Sanampudi, B. Raju, Gopi, T. S. Kumar, U. A. Kumari

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

Introduction: Congenital myasthenia syndrome (CMS) is a rare, genetically determined disorder that results in impaired synaptic transmission at the neuromuscular junction. In this study we are presenting case report of a consanguineous family with congenital Myasthenia with all siblings affected and non-affected parents. Case Report: The affected brothers were 10 years and 16 years old and the affected sister was 14 years old. There was history of one still birth in the family. There were no other affected relatives in the family. Fetal movements were normal in all the siblings and had normal labor. All 3 cases presented with ptosis since birth which was not present on waking up in the morning, develops after exertion and more towards evening. All complained of blurred vision on prolonged staring and had restricted ocular movements. All are having bulbar symptoms with difficulty in swallowing more for liquids than solids. All are having proximal weakness of limbs after exertion which improved with rest. There is no history of respiratory distress or joint contractures or dysmorphic features. There was no progressive worsening of disease. RNS showed decremental response in all. No repetitive CMAPs on single stimulation were obtained. Acetylcholine receptor antibody was negative (done in 10 year old boy). Neostigmine test was positive. Hemogram, serum creatinine, thyroid function tests and X-ray chest were normal. All showed good response to treatment with pyridostigmine with stable course on follow-up without exacerbations. Conclusion: Acetylcholine receptor (AChR) deficiency is the most common form of congenital myasthenic syndrome. This family may be a case of AchR subunit mutations with mild expression. Further studies including genetic analysis are needed. Thorough clinical, electrophysiological and neostigmine test can help in differentiating different Congenital Mysathenic syndromes and their proper treatment.


Role of short exercise test in diagnosing and predicting genotype in case series of non dystrophic myotonias

S. M. Sharief, G. B. Raju, S. Gopi, T. S. Kumar, U. A. Kumari

Department of Neurology, Andhra Medical College, King George Hospital, Visakhapatnam, Andhra Pradesh, India

Introduction: Non-Dystrophic Myotonias are group of skeletal muscle channelopathies with distint clinical phenotypes with overlaps. Genetic mutations in voltage gated chloride channel and sodium channel gene are causative. Neurophysiology test in form of Short Exercise Test identify channel specific patterns and narrow down the diagnosis and predicting genetic analysis. Materials: Six patients, 3 male of 16yr/16yr /4 yr and 3 females of 65 yr/25yr/8 yr came with c/o of stiffness of both upperlimbs and lower limbs, with various age at onset. Among 6 patients, 4 belong to different generations of one family. Methods: Clinically there was generalised myotonia with no variation on cold exposure in all patients with no skeletal and systemic features. Nerve Conduction Studies (NCS) were normal and Needle electromyography (NEMG) shown electrical myotonia in all cases. The short exercise test before and after muscle cooling was done and genetic testing based on pattern was sent. Results: In short exercise test drop of > 30% in Compound Muscle Action Potentials (CMAP) from baseline. Without cooling and no further drop after muscle cooling in all patients s/o Fournier pattern II pointing Recessive Beckers Myotonia congenital. Mutation analysis sent in one patient because of financial implications which was positive showing chloride channel (CLCN1) mutation. Conclusion: Because of difficult to clinically narrow down the diagnosis. Short Exercise Test showing a specific pattern, aided in Chloride channel testing and confirming diagnosis in limited resources.


A case of neurosarcoidosis presenting as peripheral neuropathy

S. Meenakumari, C. V. Shaji, K. A. Kabeer, S. R. Prasanth

Department of Neurology, Tirumala Devaswom (TD) Medical College, Alappuzha, Kerala, India

A 48 year old female with no co morbidities presented to us with weakness and paraesthesia of both lower limbs of 3 months duration. There was no weakness of upper limbs, neck muscles, cranial nerve symptoms or bowel and bladder involvement. There was no fluctuation of symptoms or systemic symptoms. She had bilateral lower limb weakness, proximal more than distal, with clawing of toes. Routine blood tests, thyroid and vasculitic profile were normal and viral markers were negative. High resolution computed tomogram of chest and abdomen revealed multiple mediastinal and peri pancreatic lymph adenopathy with small foci of fibrosis lung and traction bronchiectasis and nodular broncho vascular thickening. Electro physiology was suggestive of axonal sensorimotor peripheral neuropathy. Serum angiotensin converting enzyme (ACE) was elevated and histo pathological confirmation of the diagnosis was obtained from sub carinal lymph node. Patient improved with steroids. The prevalence of nervous system involvement in sarcoidosis is 5% and peripheral nerve involvement in neuro sarcoidosis is 5 to 15%. This case is being presented since isolated multi focal peripheral neuropathy is a rare presentation of neuro sarcoidosis.


Mutation profile of GNE myopathy from a quaternary institute in India

P. V. Kumar, O. Pogoryelova, K. Polavarapu, J. Hudson, H. Lochmüller, A. Nalini A

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

Introduction: GNE myopathy is an autosomal recessive disease caused by mutations in uridine-diphosphate (UDP) N-acetylglucosamine 2-epimerase/N-acetylmannosamine kinase (GNE) gene. Founder mutations are described in Middle Eastern and Roma families whereas patients of other ethnicities are compound heterozygous or homozygous for different mutations. Aim: To interpret the most prevalent and novel mutations of GNE myopathy and their clinical characterization. Methods: DNA extracted from 54 clinically and histologically confirmed patients was sequenced for coding exons 1 to 13 and >10 nucleotides of 5'and 3' intronic sequences. Alamut-Mutation software used for predicting the pathogenicity of novel variants. Results: 13 patients were putative homozygotes and 38 had compound heterozygous mutations. 5 patients had only a heterozygous mutation in exon 12 while the other allele did not show any mutation possibly because of copy number variants. 46 had missense, 6 nonsense mutations resulting in stop codon, 2 frame shift mutations (1 delins and 1 del). 2 patients had c.1374+1_1374+2Del in intron 7 affecting splicing of exon 8 and an additional homozygous p.Val727Met mutation. 5/54 had intronic splice site mutations in one allele and rest all had exonic deletions in both alleles. 44/54 had at least one copy of p.Val727Met mutation. 33/54 carried novel mutations in the GNE gene. The mean duration of illness was 6.4±5.9 years. The mean age at presentation 32.7±8.4 years and age at onset 26.3±6.7 years. The M: F ratio was 2.4:1. The onset was distal lower limbs in 43 (79.6%), distal upper limbs 4 (7.4%) and proximal lower limbs 7 (12.9%). Symmetrical onset 29 (53.7%) and asymmetrical 25 (46.2%). 13 had affected family members and 7 born to consanguineous parents. Conclusion: p.Val727Met mutation is previously described in Middle Eastern Jews, Moslem and South East Asians. This study proves high prevalence of this mutation among south Indians. Identifying GNE myopathy is crucial in view of potential therapeutic treatment with sialic acid.


Functionality, quality of life and family burden among patients diagnosed with amyotrophic lateral sclerosis

P. T. Thomas, M. G. Warrier, A. Nalini, P. Rajaram

Department of Psychiatric Social Work, National Institute of Mental Health and Neuro Sciences, Bengaluru, Karnataka, India

Introduction: Amyotrophic Lateral Sclerosis is a progressive neuromuscular disorder that can have significant and debilitating impact on the affected patient and families. Few studies have looked into the situation especially in the sociocultural scenario that is unique to India. Aim: to understand the relationship between Functionality, Quality of Life and Family Burden among patients diagnosed with Amyotrophic Lateral Sclerosis. Methods: A cross sectional descriptive study was performed among the patients and family members of 30 patients diagnosed with ALS (Both Males and females) who were receiving treatment from a national tertiary referral center for Neurological disorders. All the patients were evaluated for their complaints and diagnosed as Definite, Probable or Possible ALS according to the El Escorial Criteria. Mean age at onset of illness in this group was 51.6. Mean duration of illness at the time of presenting to hospital was eleven months. The family members were spouses, son or daughter or siblings. Functionality, Quality of Life and burden were assessed with standardized scales including ALS Functional Rating Scale Revised: (ALSFRS- R), ALS Specific Quality of Life Scale (ALSSQOL-R) and Zarit Burden Interview. Results: The preliminary analysis showed that Functionality and Quality of Life was negatively correlated with Family Burden. Better quality of life in the domains of Negative Emotional state and interaction of the patient with family and environment was associated with lesser family burden. No significant association was noted between family burden and Intimacy, religiosity as well as physical symptoms domains of quality of life. Further results will be presented. Conclusion: Patients and caregivers would benefit from structured care plan that takes into account the impact of the illness on the specific domains of quality of life of the patient and the family, as well as the deterioration in the neurological functioning. A conceptual framework for intervention with patients diagnosed with ALS and their families based on a psychosocial typology of chronic illness will be discussed.


Nonaka distal myopathy - A rare myopathy

M. Patni, M. Y. Reddy, K. Shyam, Jaiswal, N. Choudhary, J. M. K. Murthy

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

Introduction: Nonaka distal myopathy orhereditary inclusion body myopathy is an autosomal recessive disorder characterized clinically by the preferential involvement of the tibialis anterior muscle with sparing of the quadriceps muscles. It is reported often in Jews of Persian origin. The disease is rare in India, hence this case report. Case Report: A 24-yr-old male borne out of non-consanguineous parentage presented with insidious onset and progressive difficulty in walking and thinning of the leg muscle of 8-year duration. There was no similar illness in the family. Neurologic examination revealed bilateral foot drop and wasting of leg muscles, (anterior> posterior compartment). He had weakness of bilateral glutei, hamstrings, tibialis anterior and calf muscles. Both quadriceps muscles were spared. Serum creatinekinase levels were ten times the upper limit of normal. Electromyography of tibial anterior and gastrocnemius showed small amplitude, polyphasic and brief motor unit potentials with early and good recruitment. Muscle biopsy of right tibialisanterior muscle showed rimmed vacuoles with Gomori's trichrome stain. Generic studies: compound heterozygous missense variations were detected in exon 7 and 12 of GNE (UDP-GlcNAc 2-epimerase) gene. Conclusion: Nonaka myopathy should be considered in patients with distal myopathy with predominant involvement of anterior compartment muscles. Genetic studies help in counselling the patient.


An interesting association of myasthenia gravis

R. Gopal, Sowmini, Shaktivelayutham, M. Jeyaraj, Vignesh

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

Introduction: Myasthenia Gravis is an auto immune neuro muscular disorder characterized by fluctuating weakness of skeletal muscles which worsens with exertion and improves with rest. Two thirds of the patients present with extrinsic ocular muscle weakness which may progress to involve bulbar and limb muscles resulting in generalized Myasthenia. Case Report: A 37 years old female presented with asymmetrical drooping of both eye lids with double vision of one year duration. She also had easy fatigability with diurnal variation. On examination she had vitiligo and features of myasthenia gravis-weakness of neck flexors and proximal muscles (power 4 to 4+) in all 4 limbs, with normal DTR, bilateral ptosis and horizontal diplopia. Lab shows AchR antibodies positive with 15.29nmol/l (normal <0.4), RNS show decremental response, blood sugar more than 500mg/dl, HbA1c 10.5%, To differentiate Type 1 DM or Type 2 DM AntiGAD Ab was done which was very high 1235.2 (normal <10 IU/ML) indicating Type 1 DM, AntiTPO Ab were also high with Euthyroid state. Other hormonal study ACTH, CORT II, FSH, LH - normal. CT Chest is negative for Thymoma. Test for ANA, RA Factor, AntiSS A and B, were negative. With these clinical features and laboratory features a diagnosis of Polyglandular Autoimmune Syndrome Type III was made. She is being treated with Cholinergics, tapering dose of steroids, immunomodulatory drug-Azathioprine and Insulin. Conclusion: This case is presented to emphasise the importance of identifying other coexisting autoimmune diseases in any patient with Myasthenia Gravis if there is a strong clinical suspicion for such association, which will provide a complete and holistic management for the patient.


Amyloid neuropathy - presenting as rapidly progressive severe painful small fiber & autonomic neuropathy - A case report

P. K. Shembalkar

Department of Neurosciences , Getwel Hospital & Research Institute, Nagpur, Maharshtra, India

Amyloid neuropathy is rare and only few cases are reported from India. We present a 26 year female who presented with severe burning paresthesias & allodynia from March 2016. The burning and paresthesias were so severe that she could not work or sleep. She has also blurring of vision in left eye and was diagnosed as having Adies pupils. She also noticed loss of sweating in palms and soles (used to perspire heavily). Family history is negative. On examination, left pupil was sluggishly reacting to light but was briskly reacting to accommodation. Power was normal. Ankle jerks were absent. Pin prick was diminished distally. Position sense was normal. NC study showed absent H reflex. Autonomic testing showed absent sympathetic skin response. Vasculitic work up is negative. CSF showed raised proteins and 10 lymphocytes. Protein immunofixation showed positive Kappa and IgG band. Amyloidosis versus paraproteinemias was considered. Her bone marrow biopsy is normal. Abdominal fat pad biopsy is positive for amyloidosis (Congo red). There is no evidence of systemic involvement. With pregabalin and tramadol pain is now tolerable. However, she is now complaining of increasing numbness and difficulty in fine movements. She has predominantly small fiber & autonomic fiber neuropathy with dissociate sensory loss which is progressive. In view of positive Kappa band, positive abdominal fat pad biopsy for amyloidosis, she is most likely having amyloid neuropathy. The pattern of neuropathy and lack of systemic involvement favors familial amyloid neuropathy. However, primary amyloidosis (light chain amyloidosis) needs to be excluded. Gene testing for transthyretin mutation is suggested. Conclusion: Amyloid neuropathy should be considered in patients presenting with neuropathy with autonomic involvement.


Dok-7 congenital mysathenic syndrome: A case report

G. B. Kale, M. Y. Reddy, V. Nilesh, Chaudhary, K. Shyam, Jaiswal, J. M. K. Murthy

Department of Neurology, Institute of Neurological Sciences, Care Hospitals Banjara Hills, Hyderabad, Telangana, India

Introduction: Dok-7 is a cytoplasmic activator of muscle-specific receptor tyrosine kinase (MuSK) both of which are required for neuromuscular synaptogenesis. Mutations in DOK-7 underlie a congenital myasthenic syndrome (CMS) associated with small and simplified neuromuscular synapses likely due to impaired Dok-7/MuSK signaling. Dok-7 myasthenia is a relatively common CMS and is potentially treatable. Case Report: A 26-year-old male with normal birth and developmental history, presented with insidious onset and fluctuating limb girdle weakness with mild drooping of eyelids with no sensory or bowel and bladder complaints since 5 yrs of age. He was investigated at another facility with slow RNS which showed significant decrement. Acetyl choline receptor and anti Musk antibodies were negative. He was started on pyridostigmine and later put on prednisolone and azathioprine with no benefit. He developed three episodes of myasthenic crisis requiring ventilation. He was also given benefit of plasma exchange and IV immunoglobulin. He presented at our facility one year back with severe proximal muscle wasting and weakness. Congenital myasthenia was suspected in view of limb girdle pattern of weakness and the course of illness. He was tested positive for Dok-7 mutation. He harbored compound heterozygous mutation in exon 7 and intron 3 of chromosome 4. He responded remarkably with oral salbutomol. MGFA disability scores prior and after salbutomol therapy were 15 and 2 respectively. Conclusion: CMG should be suspected in patients with myasthenic syndrome who fail to respond to immunomodulation therapy and should be tested for DOK 7 gene mutation which is potentially treatable.


Duchenne and becker muscular dystrophy: Genotype phenotype correlation in a large cohort

W. J. Mujawar, A. Nalini, S. Vengalil, V. P. Kumar, K. Polavarapu, M. Mahadevappa

Department of Neurology, NIMHANS, Bengaluru, Karnataka, India

Background: Large scale studies on MLPA confirmed cases of Duchenne and Becker muscular dystrophy regarding clinical characteristics, genotype and relation between reading frame and phenotype have been done in many countries. However, such a study is lacking from the Indian subcontinent. Methods: A retrospective analysis of 317 MLPA confirmed cases of DMD/BMD attending the neuromuscular clinic of a quaternary referral center in South India was undertaken to study the genotype-phenotype pattern. Results: Among the 317 patients, there were 279 cases of DMD (88 %), 32 cases of BMD (10.1 %) and 6 cases of intermediate phenotype (1.9 %). Deletions accounted for 91.8 % of cases while duplications caused the remaining 8.2 %. There were 254 cases of DMD (91%) with deletions, while 25 cases (9%) were due to duplications, 31(96.8%) cases of BMD were due to deletions while one case (3.2%) was due to duplication. All 6 cases of intermediate type were due to deletions. Single exon deletions was the most frequently identified mutation. Deletions of 6 exons and less constituted 68.8 % of cases. Exon 50 deletion was the most common finding. Reading frame rule was observed in 90% of DMD and 94% cases of BMD. A tendency towards lower IQ and earlier wheel chair dependence was observed with distal exon deletions though a definite correlation was not noted. Conclusion: Reading frame rule was observed in 90-94 % of cases and this finding is concordant with reports for other parts of the world. However, advanced studies including analysis of structure of mutant dystrophin is needed for a more accurate genotype - phenotype correlation. Better understanding of the structure will surely help us for development of potential targeted therapies and recruitment in drug trials.


Came with a "simple" foot drop only to uncover underlying mysterious "complex" pathology!

S. S. Kawale, D. Joshi, S. K. Chaudhary, A. Kumar, R. Singh, J. Sharma

Department of Neurology, Banaras Hindu University, Varansi, Uttar Pradesh, India

Traumatic and other causes of CPN injury have been well documented. However, we report an unusual case of foot drop secondary to the cold abscess most probably due to non tubercular mycobacterial infection. A 30 yr old man without significant medical history visited with mild left calf pain and sensory disturbances over dorsum of left foot who on examination was found to have left ankle dorsiflexion weakness and 70% sensory loss in distribution of superficial peroneal nerve without nerve thickening. NCS showed non recordable response in left CPN and left sural. USG and colour Doppler of left leg showed popliteal vein thrombosis and superficial vein thrombosis. Patient was investigated started on anticoagulant therapy to which he showed partial improvement in sensory and motor disturbances. Even on therapy, for last 6 months, he again developed progressive left foot drop and mild swelling over anterolateral aspect of lower left leg without any sinus or ulceration or discharge. MRI of left leg showed multiple abscesses out of which two larger sized were located at posterolateral aspect of midleg and anterolateral aspect of distal leg leading to compression of superficial and deep peroneal nerve along with thrombosis of superficial saphenous vein. Bacterial and mycobacterial cultures were negative. Considering as non tubercular mycobacterial skin and soft tissue infection, patient was started on anti-tubercular therapy. Patient improved after 6 months of therapy.


A series of 5 cases of chronic progressive external ophthalmoparesis

D. S. Abraham, D. A. Sweetson, N. Shanmugasundaram, Balasubramanian, R. L. Narashiman, S. Gopinathan

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

Introduction: Chronic progressive external ophthalmoplegia (CPEO) is a mitochondrial ocular disorder characterised by bilateral progressive ptosis and ophthalmoplegia. These ocular features can develop either in isolation or in association with other prominent neurological deficits (CPEO+). Case Report: Five patients were identified with this disease in the age group from 25 to 50 years. All patients presented with ptosis and ophthalmoparesis. In three patients it was asymmetrical and symmetrical in other two. Two patients were noted with mild dysphagia for liquids and hard of hearing. One patient had proximal muscle weakness and one patient with inguinal hernia and one patient with conduction defect. The CPK, acetylcholine receptor antibody levels and RNS were normal in all patients. CSF pyruvate lactate ratio was elevated in two patients and normal in other three patients. Muscle biopsy showed mitochondrial pathology in one patient. Discussion: Most of the patients have presented with ptosis and ophthalmoparesis in the 2 nd or 3 rd decade. CPEO is characterized by progressive paralysis of the extraocular muscles, tends to begin in young adulthood. Ptosis usually is the first clinical sign and ophthalmoplegia may not become apparent for months to years. CPEO in association with mutations in mitochondrial DNA may occur in the absence of any other clinical sign, but it is usually associated with skeletal muscle weakness. Extraocular muscles are affected preferentially because their fraction of mitochondrial volume is several times greater than that of other skeletal muscles. Conclusion: The possibility of mitochondrial disease to be considered in all patients with Ophthalmoparesis and ptosis who doesn't have RNS positivity, even if CSF pyruvate and lactate is normal. Muscle biopsy to be considered in such patients to identify mitochondrial disease. This case series is presented for documentation.


Mysterious toe walking

D. R. Chowdhury, B. Ghosh, A. Tiwari, D. Bandopadhyay, T. Chowdhury, S. Sengupta

Department of Neurology, B.R Singh Hospital, Kolkata, West Bengal, India

Introduction: Benign acute childhood myositis (BACM) is a rare disorder leading physicians into unnecessary workup. It is characterised by sudden onset of calf pain, refusal or difficulty to walk followed by rapid recovery within a week. Most commonly occur after influenza B but other viruses are also implicated. Case Description: Case 1- A 6 years old boy presented with acute onset pain in both lower limbs, difficulty in walking, suddenly started after getting up from bed in morning following 2 days of upper respiratory symptom. He was apyretic, denied any passive manipulation of leg due to pain. Whenever asked to stand or walk, he kept on toe walking. Other neurological examinations were normal. Creatinine phosphokinase (CPK) was 6518 IU/L. Other investigations were normal. Adequate hydration was maintained and during the 2nd day of hospitalization pain spontaneously resolved and normal gait was resumed. Case 2- A 4 ½ years old boy presented with acute onset calf pain, inability in walking just after awakening in morning on the 3 rd day of a febrile episode. The boy could barely stand on toes with support. There was calf tenderness on passive stretching of ankle. CPK was 9297 IU/L, Liver function test was deranged (SGOT - 272 IU/L, SGPT - 51.4 IU/L, ALP - 169 IU/L). Patient improved following 2 days. Discussion: Raised CPK is the most consistent finding in BACM Though level does not correlate with recovery. Leucopenia, altered LFT may be present indicating underlying viral infection. Viral tests for BACM-related viruses should not be routinely performed because of the time interval between infection and seroconversion. Moreover, not always etiological agents can be found, and antiviral drugs are not usually recommended.


Centronuclear myopathy in adult: A rare disease

N. Balaini, V. Y. Vishnu, J. Sachdeva, A. Kumar

Department of Neurology, PGIMER, Chandigarh, India

Introduction: Centronuclear myopathy (CNM) is a rare disease. It is an inherited neuromuscular disorder characterised by clinical features of a congenital myopathy and centrally placed nuclei on muscle biopsy. The incidence of X-linked myotubular myopathy is estimated at 2/100000 male births. Autosomal dominant forms have a later onset and milder course than the X-linked form, and the AR form is intermediate in both respects. Case Report : a 23 year old male was referred for evaluation of proximal muscle weakness since chikdhood with family history of 5 male deaths 6 to 12 months of age. He started walking at 3 years of age and used to lag behind children of his age in running or lifting weights, also having difficulty in standing fron sitting position. Examination showed mainly hip flexor weakness and shoulder abduction weakness with mild calf hypertrophy. CK-NAC was normal. EMG was myopathic. Muscle biopsy revealed centronuclear myopathy. Discussion: The X-linked form has been clinically well characterised and usually gives rise to a severe phenotype in males presenting at birth with marked weakness and hypotonia, external ophthalmoplegia and respiratory failure. It has been associated with mutations in the myotubularin (MTM1) gene on chromosome Xq28. The autosomal-recessive form is characterised by facial weakness including severe involvement of the masticatory muscles, and ocular abnormalities such as ptosis and external ophthalmoplegia and associated with mutation in BIN1gene on chromosome 2. Dominant forms reported to date appear to be associated with a mild clinical phenotype characterised by normal early motor developmental milestones, onset in adolescence and a slowly progressive course with loss of independent ambulation uncommon before the 6 th decade and associated with mutations in dynamin2 (DNM2) gene and ryanodine receptor (RYR1). Our patient was having milder form of disease possibly dominant or recessive form of disease.


Neuromyotonia - experience of 8 patients from a tertiary care centre in North India

G. Jain, M. Goyal, A. Choudhary, M. Modi, V. Lal

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

Introduction: Neuromyotonia is a clinical and electrophysiological syndrome of spontaneous muscle fiber activity due to hyperexcitability of peripheral nerves causing generalised visible myokymia and muscular cramps. Most commonly, it is an acquired immune mediated disorder showing elevated antibodies against presynaptic voltage gated pottasium channels. Objective: To share our experience of 8 cases of neuromyotonia with emphasis on clinical manifestations, electrophysiological characteristics and response to various therapies. Materials and Methods: Patients with characteristic clinical signs and symptoms of neuromyotonia were subjected to elctrophysiological studies, followed by testing for antibodies to VGKC (LGI-1 and CASPR 2). Results: Our cases included 7 males and 1 female with age ranging from 23 to 68 years. Visible muscular twitchings were seen in 7 patients, diffuse severe pain was present in 5 patients, behavioural abnormalities were present in 3 patients and myoclonic jerks were present in 2 patients. Electromyographic evidence of continous muscle fiber activity was seen in 7 patients. Serum antibodies to VGKC (Anti- CASPR 2 in all 8 and Anti LGI-1 in 2) were present in all patients. No evidence of any malignancy was found in any patient. All patients were started on immunotherapy (5 on IV steroids and cyclophosphamide and 3 on IVIG and steroids). All of them showed remarkable response to the initial treatment, however, one of them had a relapse and had to be given IV Rituximab, with which he responded. Conclusion: Neuromyotonia is a heterogenous, potentially treatable condition which can present in varied ways and one needs to be vigilant to pick up this elusive condition.


Clinical profile of becker muscular dystrophy, phenotypic and genotypic correlation

U. Das, G. Ganguly, D. S. Guin, A. Joardar

Bangur Institute of Neurosciences, IPGMER, Kolkata, West Bengal, India

Background and Objective: Becker muscular dystrophy (BMD) is an X linked recessive condition affecting 5/10000 males. The clinical phenotype of BMD is more varied than Duchenne muscular dystrophy. Dystrophin is a large gene (2.4Mb) with 79 exons (covering only 0.6%) with a coding sequence of 14 kb. Clinically BMD (incidence approximately1/12,000), is similar to DMD but milder with late onset and loss of ambulation. Becker muscular dystrophy patients carry mainly inframe deletions, which allow partial functioning of dystrophin protein and hence results in a milder phenotype, whereas in DMD, frameshift results in loss of dystrophin. This retrospective study has been designed to correlate the disease severity with genetic deletion. Methods: Study has been conducted in Neurogenetic clinic of Bangur institute of neurosciences, Kolkata. Results: Out of 30 pt taken 23came out as confirmed cases of BMD by genetic analysis. Possibilities of rest of the undiagnosed pts remained either point mutation and duplication or other rare variants of congenital dystrophy which mimic BMD clinically. In our study 18 showed deletion in distal exons and rest 5 showed deletion both proximal and distal exons. In our study distal hot spot region was much more commonly involved than proximal hot spot region. Conclusion: So we have concluded that any phenotype is possible in various genotype and it's very difficult to predict a particular phenotype in context with a certain genotype. However we need a larger sample size and proper follow up of pts to further corroborate our findings.


Light after groping in darkness - Response to rituximab in MUSK positive myasthenia gravis

M. Suryawanshi, M. Goyal, G. Jain, A. Choudhary, M. Modi, V. Lal

Department of Neurology, PGIMER, Chandigarh, India

Introduction: Generalised myasthenia gravis with MUSK (muscle specific receptor tyrosine kinase) positive antibodies is a difficult to treat entity. These patients have a much lower frequency of thymic pathology than the AchR (Acetylcholine receptor) antibody myasthenia gravis, further diminishing the treatment armamentarium. Objectives: To study the clinical characteristics and treatment response to IV rituximab in 6 patients of MUSK positive myasthenia gravis in a tertiary care centre in Northern India. Materials and Methods: We observed the clinical characteristics of MUSK positive myasthenia gravis patients who were refractory to various routinely used immunomodulation including IVIg and PLEX. The patients were admitted in Neurology ward in our centre and were administered IV Ritiximab, 375 mg/m2 weekly for 1 month followed by 6 monthly, to look for response. Results: Of the 6 patients studied, 3 were males and 3 females. Mean age of the patients were 47.6 ± 3.4 years (range 23-68 years). All 6 of them presented to us in myasthenic crisis with predominant oculo-bulbar involvement. All of them had received either plasma exchange or weight based IVIg or both with no or minimal response. For long term immunomodulation, 2 were on Azathioprine and rest 4 had been on MMF (mycophenolate mofetil). All were then administered IV Rituximab according to protocol. All 6 recovered from the crisis. 5 of them remained in remission. 1 out of the 6 died, likely due to secondary infection. Conclusion: IV Rituximab remains an effective option in MUSK positive myasthenia patients who have failed all other available treatment options.


Utility of an advanced diagnostic facility for autoimmune neurological and neuropsychiatric disorders at neuromuscular lab at NIMHANS

A. Mahadevan, M. Nagappa, P. S. Bindu, A. B. Taly, G. Yashwanth, G. G. Kumar, P. Satishchandra

Department of Neuropathology, NIMHANS, Bengaluru, Karnataka, India

Background: In the last decade, there is an expanding repertoire of autoantibody for diagnosing autoimmune neurological diseases with high sensitivity and specificity. The clinical utility of these is considerable, as accurate identification has therapeutic and prognostic implications. To make available these expensive tests at affordable cost to our patients with rapid turn around time, we established a state-of-art "Neuromuscular Lab' on a self-sustaining model, with advanced diagnostic facility for neuroimmunological tests (including ANA, ANCA, NMO, autoimmune encephalitis mosaic with NMDA, VGKC, AMPA and GABA antibodies, and paraneoplastic antineuronal antibodies). Objective: To investigate the prevalence of these disorders in routine neurologic practice and the utility of these neuroimmunology tests in diagnosis. Results: In the last 22 months, 6866 cases were tested (ANA - 2505, ANCA -1995, NMO -889, autoimmune encephalitis mosaic -981, paraneoplastic antineuronal antibodies-488). Most frequent were ANA positives (24%), followed by autoimmune encephalitis mosaic (12.9%), NMO (11.8%). P Araneoplastic antineuronal antibodies (4.7%) and ANCA (3.1%) were infrequent. Autoimmune encephalitis was diagnosed in 127/981 (12.9%) (NMDA in 102(80.3%), LGI in 12(9.4%, CASPR in 11(8.6%), GABA in 2(1.5%)). NMDA encephalitis was more frequent in children (63.7%) with female predilection (M: F=1:3.4). In eleven cases CSF and serum were tested and positive. In the NMOSD group, there was striking female preponderance (M: F=1:6; age range: 2-70 yrs; only 8 being children). Time to diagnosis was 8-120days in monophasic and 4-108 Mon (mean 26.8 mon) in relapsing group. Both CSF and serum tested positive in five cases. Paraneoplastic antineuronal antibodies were detected in 23/488 cases tested (4.7%). Anti GAD antibodies were most frequent (10/23) followed by anti Yo (5/23), anti titin (4/23) and once each of anti Ma, anti Ri, amphiphysin and Sox. Conclusion: The Neuromuscular Lab helped establish diagnosis in significant proportion that would have otherwise gone unrecognised. Testing in serum was as sensitive as CSF. Availability of these tests have also aided in research into these rare disorders.


Macrosleep architectural changes in myotonic dystrophy: A pilot study

S. Nashi, B. Kutty, G. Kumar, R. Jhankar, R. Yadav, A. Nalini

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

Introduction: The study is aimed to document the sleep architectural changes in patients of myotonic dystrophy type 1 (DM1) - an autosomal dominant trait with incomplete penetrance and variable expressivity caused by unstable CTG trinucleotide repeat expansion located on chromosome 19q13.3. Patients with DM1 are often diagnosed with hypersomnia which could be due to dysfunctional sleep pattern regulation by the pontine brainstem and thalamocortical network. In the present study, we have evaluated the sleep architecture in terms of sleep stage percentage and duration to assess the dynamics of sleep stability and hence the reasons for excessive daytime sleepiness. Methods: Whole night video monitored polysomnography of DM1 (n=12, mean age= 33.83 ±12.45years), and normal controls (CNT, n=12, mean age =32.75 ±10.02 years) were carried out in the semi-soundproof sleep cabin in the Human Sleep Research Laboratory of the Department of Neurophysiology. The sleep data was acquired using 32 channels Galileo Mizar 40 system and the data was converted to EDF format, scored in Polyman viewer, and the analysis of Wake periods, NREM stages and REM stage was done using scripts written in Matlab R2013a. Statistical analysis was done using Permutation-based t-test (10,000 permutations) and Bootstrap-based Confident intervals. Results: DM1 (vs CNT) showed decreased Sleep efficiency, decreased N2 percentage and increased N3 duration. DM1 had significantly longer actual sleep duration and an increasing trend in REM duration with more wake after sleep onset (WASO) episodes leading to increased wake percentage and duration. Conclusion: Reduced sleep efficiency and N2 duration together with a significantly increased wake activity (WASO) confirm the reasons for sleep instability, disrupted sleep and abnormal sleep macrostructure in DM1. Increase in N3 and REM duration depict the need of restorative sleep which may be due to an intrinsic CNS defect perhaps associated with defective thalamo-cortical mechanisms associated with the disease.

Poster Session 09; Cognitive Disorders08:30-11:00 h, Sunday, November 13, 2016


The burden of vascular insult in brain in Alzheimer's disease in the Indian population

U. Sundar, M. Abhilasha, R. Gala, Priyank, P. Darole, C. Londhe

Department of Medicine, Lokmanya Tilak Municipal Medical College (LTMMC), Mumbai, Mahrasthra, India

Aim: To evaluate vascular risk factors and vascular lesions on imaging in patients with AD, and correlate with severity of cognitive dysfunction. Methods: serial recruitment of newly diagnosed AD (DSM-4). Forty Eight patients were studied. 29 cognitively normal, age matched controls with no prior strokes had MRI data recorded. Exclusion Criteria : Patients with previous clinical strokes. Parameters Noted : Diabetes, Ischemic Heart Disease, Hypertension, High waist hip ratio (above 0.9), Tobacco and Alcohol abuse, MMSE, FAB score, MRI - lacunes, Deep white matter hyperintensities, Global cortical atrophy Stats- WPSS package version 16. Results: (1) Prevalence of vascular risk factors was - Diabetes - 33%, Hypertension - 43.8%, Coronary Artery Disease - 12%, Family history of stroke-27.1%, Alcohol abuse - 20.8%, Tobacco abuse - 45.8%, Smoking - 25%, Waist hip ratio >0.9 - 70.8%. Prevalence of Coronary artery disease, Tobacco abuse was significant higher as compared to controls. (2) MRI evidence of ischemia alone was seen in 4/34 patients (11.76%), Ischemia + Atrophy in 16/34 (47%) patients. Ischemia was due to lacunes in 13/34 (38.23%) and white matter changes in 19/34 (55.9%). However, the control group (HT 47%, DM 33%) also showed old infarcts in 47% subjects, and deep WM changes in 71% subjects. The 2 groups were statistically similar. (3) FAB and MMSE cores did not correlate significantly with MRI evidence of Ischemia in AD. Hypertension in AD patients correlated with MRI changes of both atrophy and ischemia (P<0.05).


Superficial siderosis of central nervous system - A rare cause of ataxia and cognitive impairment

M. Zoheb, N. Madhavi, N. Aparna, S. Jawalker

Department of Neurology, Deccan College of Medical Science, Hyderabad, Telangana, India

Superficial siderosis of Central nervous system is an extremely rare condition characterized by deposition of hemosiderin in subpial layer of CNS. The condition remains undiagnosed due to its prolonged clinical course and majority of the patients present in later irreversible stage. Once hemosiderin gets encrusted on the surface of brain, removing the source of bleed does little help in altering the course of the disease. Here we present a 28 years old male who presented with gradually progressive ataxia, sensory neural hearing loss and cognitive impairment. Due to the prolonged course and varying clinical picture it posed us diagnostic difficulties, however MRI brain with supportive investigations helped us in arriving at the diagnosis of Superficial Siderosis. In about 2/3 rd of the cases exact causal relationship for siderosis could not be established however due to its protracted and irreversible nature early identification and management would help in altering the course of the condition.


AD-CBS variant of Alzheimer's Disease - A case report

M. G. V. Aditya, R. G. Butchi, S. Gopi, S. T. Kumar, A. Kumari

Department of Neurology, Anthra Medical College, Kings George Hospital, Visakhapatnam, Andhra Pradesh, India

Introduction: Corticobasal syndrome (CBS) is a striking and unusual clinical manifestation of various neurodegenerative pathologies (CBD, AD, FTD etc.). CBS-AD usually present at an early age (<65 years), has long disease duration (10 years), myoclonus, apraxias, cortical sensory loss are common. Case Report: A 60-year-old right handed hypertensive woman who studied up to 6th class presented with insidious onset, gradually progressive episodic memory loss from 3 years, losing paths from 2 years, visual & immediate memory loss, acalculia from 1 ½ years, right upper limb action myoclonus, dressing apraxia from 6 months, difficulty in using routinely used tools with hands from 4 months. No history suggestive of cranial nerve involvement/motor weakness of limbs/sensory/cerebellar/autonomic system involvement. Cognitive examination showed word finding difficulty, decreased sustained attention, poor executive function, impairment of immediate, recent, visual memory and new learning ability, ideomotor apraxia, dyscalculia, right left disorientation, finger agnosia, constructional and dressing apraxia. Physical examination revealed loss of cortical sensations in right upper limb, snout and glabellar tap sign. Her MMSE was 14/30 and ACE-R 47/100. MRI Brain showed atrophy of bilateral parieto-temporal region. FDG PET CT Brain showed asymmetric reduced tracer uptake right > left parieto-temporal cortex with mild asymmetrical reduced uptake in left basal ganglia. A diagnosis of possible corticobasal syndrome (Armstrong criteria) with intermediate likelihood of Alzheimer's disease (NIA-AA criteria) was made. Conclusion: AD pathology is frequently found in patients with atypical cortical syndromes, suggesting that diagnosis of AD needs to be considered even in patients who present with CBS. Awareness of this kind of atypical presentation is important in order to make an early diagnosis, prognostication, adequate treatment & research in similar cohorts.


A study on the effect of severity of chronic obstructive pulmonary disease on cognitive function

C. V. Shaji, J. John, Venugopal

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

Introduction: Cognitive dysfunction is an important systemic effect of chronic obstructive pulmonary disease (COPD) affecting various cognitive domains. Our study aimed to evaluate the impact of disease severity and impairment of respiratory physiology on cognitive function in patients with established COPD. Methods: In a hospital based cross sectional study, we examined hundred consecutive COPD patients for their respiratory parameters and further graded them into groups based on severity of disease by BODE index (Body-mass index, Airflow obstruction, Dyspnea and Exercise capacity), a multidimensional scoring system. All the subjects were then assessed for cognitive skills of orientation, attention, memory, visuo perceptual abilities and executive functions by simple and flexible neuropsychological battery of tests, including Mini Mental State Examination (MMSE), Clock Drawing Test (CDT) and Trail Making Tests (TMT-A and TMT-B). Results: Out of the respiratory parameters resting oxygen saturation (SpO2), modified Medical Research Council (mMRC) and Chronic Obstructive Pulmonary Disease Assessment Test (CAT) scores correlated well with most of the cognitive domains. There was a significant correlation of resting SpO2 with Mini Mental State Examination (MMSE), orientation, registration, attention, recall, language and Clock Drawing Test (CDT). The hypoxemic (<90%) performed poorly than moderately hypoxemic (90-95%) and non- hypoxemic (>95%) COPD patients in MMSE, CDT, orientation and registration (p<0.05). mMRC correlated significantly with the tasks Trail Making Test (TMT-A and TMT-B), orientation and recall. CAT score correlated significantly with orientation and MMSE and CDT. Conclusion: The decline in pulmonary function is associated with cognitive dysfunction, which increases significantly with the advancement of the disease. Among the various cognitive domains, memory, attention, symbolic representation and visual processing (CDT), reproduction of numeric sequences, cognition flexibility and set shifting capacity (TMT-A and TMT-B) were the most affected.


A case of hartnup's disease

B. Sritharan, M. Arasu, A. F. Jason

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

We present a case of Hartnup's disease followed up in our institution for 15 years. The patient was 9 years old when he first presented with progressive ataxia, intellectual disability and skin rash in sun exposed areas. Urine chromatography showed excretion of excessive amounts of tryptophan. Following the diagnosis, he was started on niacin supplements and high protein diet. Over the last 15 years he has suffered episodic worsening of ataxia, correlating with periods of poor oral intake, which is characteristic of Hartnup's disease. Examination reveals prominent intention tremor along with gait and stance ataxia. Short stature and mental retardation were also noted. Repeat urine chromatography was done this year, and found to be negative for aminoaciduria. A literature review revealed that absent aminoaciduria in a patient with Hartnup's disease could be either due to altered tryptophan metabolism or heterozygosity of the evaluated patient.


Aphasia and its metamorphosis

V. Ganesh

Department of Neurology, Narayana Medical College, Nellore, Andhra Pradesh, India

Introduction: Aphasias are the common language disorder that is encountered in stroke patients. Identification of different kind of aphasias will help to some extent in the localizing the site of lesion. Aphasias may evolve over time from one kind to a different one. Aim: To study the type of aphasia in relation to the site of brain lesion and to study the evolution of aphasias over a period of 4 weeks. Materials and Methods: It was a prospective study conducted in patients attending with stroke and language disturbances to the department of Neurology, Narayana medical college, Nellore during a period of one year from 2015-2016. Language assessment was done with the help of Western aphasia battery (bedside version) at the time of discharge and at follow up of 4 weeks. Results: Among the 51 patients at admission 39 (76.4%) had global aphasia, 8 (15.6%) were with Broca's aphasia and anomic aphasia observed in 2 (3.9%). Transcortical motor and Wernicke's aphasias were found in 1(1.9%) patient each. Out of 39 global aphasia patients 13 patients lost follow up. Remaining 26 patients 20 (77%) left as global aphasics. Among 8 Broca's aphasics 2(25%) patients remained as Broca's aphasics. 2 of the global aphasic and 2 of the anomic patients became normal. Type of aphasia in relation to anatomical lesion is studied. Mean aphasia and language scores shown improvement from 10.47 to 38.05 and from 8.22 to 30.65 respectively. Conclusion: Global aphasia was the most common aphasia in our study which was associated with lesions of both cortical and subcortical involvement. At the end of 4 weeks of follow up Wernicke's aphasia was least to transform to other aphasia followed by global and Broca's aphasias. Further studies with large sample size required to conform our results.


Intravascular lymphoma: A very rare cause of rapidly progressive dementia

R. K. Agarwal, A. Rohatgi, V. Shah, S. Patel, D. Rana

Department of Neurology, Sir Ganga Ram Hospital, New Delhi, India

Intravascular Lymphoma (IVL) is a very rare subtype of extranodal diffuse large B-cell lymphoma, which has a predilection for the central nervous system. It is usually a fatal disease characterised by the intraluminal proliferation of lymphoma cells within blood vessels. The patients usually manifests as subacute encephalopathy, dementia, seizures, or multifocal cerebrovascular events. Due to its variability of clinical presentation and the lack of diagnostic protocols available, approximately half of IVL cases are diagnosed only after autopsy, and the antemortem diagnoses of IVL are still rare. Here, we highlight a case of a 71 year old, gentleman, who presented with rapidly progressive cognitive impairment over a period of 3 months, with a recent episode of complex partial seizures, and was later diagnosed as a case of CNS intravascular lymphoma.


Megalencephalic leukoencephalopathy with subcortical cysts (Van der Knapp disease): A case report

M. K. Mahata, S. Ghosh, K. C. Ghosh, R. Bhattacharyya, G. P. Mondal

Department of Neurology, Calcutta National Medical College & hospital, Kolkata, West Bengal, India

Megalencephalic leukoencephalopathy with subcortical cysts is clinically characterized by macrocephaly, mild motor developmental delay and seizures. Later in life, patients may develop gradual onset of ataxia and pyramidal features. Mental capacities are usually preserved but there may be a mild deterioration later. It is inherited in an autosomal recessive pattern and the gene locus has been mapped to MLC 1 gene at chromosome 22q. We recently identified the disease in a 20 years old girl admitted in our hospital. A 20-year-old Muslim girl born out of consanguineous marriage presented with progressive difficulty in walking and stiffness of all limbs. She had gross developmental delay. She had complex partial seizures with semiology suggestive of right temporal lobe with secondary generalization since 4 years of age and was initially responsive to Valproate. She had moderate impairment of cognitive and language abilities and could not go to school. Her siblings were however not affected. Megalencephaly was evident as the head circumference was 56 cm. The motor system examination revealed severe spasticity with grade 4 power of all limbs. She had brisk reflexes and bilateral plantars were extensor. She had severe incoordination and also had a spastic gait. Routine blood investigations, LFT, RFT, electrolytes were normal. NCS of 4 limbs was normal. Her MRI showed bilateral temporal and frontal subcortical cysts along with other characteristic features suggestive of the disease. CSF was essentially normal and glycine content was normal contrary to reports from Western countries where it was found elevated.


A case series of wernicke's encephalopathy

P. Purushothaman, N. Shanmugasundaram, S. Balasubramanaian, S. Gobinathan

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

Introduction: Wernicke's encephalopathy is an acute/subacute neurological disorder due to thiamine deficiency. CASE1-20 year female with hyperemesis gravidarum and spontaneous abortion at 12 weeks presented with unsteadiness of gait, blurring of vision and disorientation. On examination acute confusional state, disorientation, horizontal gaze evoked nystagmus. CASE2-24 year female 5months amenorrhoea (G2P1L1) presented with vomiting for 2months, altered sensorium, double vision, unsteadiness while walking. Findings included bilateral abduction restriction, horizontal gaze evoked nystagmus, gaitataxia. CASE3 - 37year male alcoholic, smoker developed diplopia memory disturbances for 3 days, icteric, impaired recent memory, opthalmoplegia with bilateral nystagmus and incoordination of both upper and lower limbs. CASE4 - 33 year female (G3P2L2) with hyperemesis gravidorum presented with altered sensorium, unsteadiness while walking, external opthalmoplegia horizontal gaze evoked nystagmus, mild dysarthria, FNT and FFNT incordination with gait ataxia. CASE5-21 yr male alcoholic with recurrent episodes of GTCS, altered sensorium, unsteadiness in gait with bilateral horizontal gaze evoked nystagmus, incordination in both upper and lower limb, gait ataxia. Serum magnesium was low, EEG showed sharp waves. CASE6-62year male alcoholic with forgetfulness, apathy, weakness in left half of body for 3months. Clinical picture revealed frontal lobe disturbances, naming defect, confabulation, amnesia, visual and verbalmemory impaired with upgaze restriction and upbeat nystagmus with normal power, broad based gait and gait ataxia. Brain imaging of all patients had hyperintensities in medial thalamus and periaqueductal region except one had hyperintensities in posterior midbrain and other was normal. All patients were treated with injection thiamine and improved. Discussion: All the patients in this series are in the age group of 20-40 years, of these 4 case were antenatal with hyperemesis gravidorum, and 3 cases were chronic alcoholism, all patients had the triad of wernicke's encephalopathy, except one patient who had only mild memory disturbances and one had rare feature of upbeat nystagmus. Brain imaging of all patients had hyperintensities in medial thalamus and periaqueductal region except two had hyperintensities in posterior midbrain and other was normal. Conclusion: This case series is presented for its documentation.


The clinical spectrum of patients presenting with rapidly progressive dementia to a tertiary care center in India

A. R. Patil, D. Naheed, V. Y. Vishnu, M. Modi, M. Goyal, V. Lal

Department of Neurology, PGIMER, Chandigarh, India

Introduction: Rapidly progressive dementia (RPD) has been variably described as developing over few months to 2 years. It warrants exhaustive evaluation to ascertain the cause. Although prion diseases have been described as the most common cause in literature, with the availability of diagnostic neuroimaging and biochemical investigations, non-prion and reversible causes are now being increasingly recognized. Objective: To study the clinical profile of patients presenting to a tertiary care center with RPD. Materials and Methods: We retrospectively reviewed the medical records and laboratory data of patients admitted to our center with cognitive decline fulfilling the DSM 5 criteria from January 2008 to June 2016. Of these those who had presented within 1 year of symptom onset were included in the study. The epidemiological, clinical features and laboratory data were analysed. Results: Out of 248 patients admitted with dementia, 136 had presented within 1 year of symptom onset. Of these 82 (51.8%) were males, mean age at presentation was 47.7±6.8 years (age range: 15-83years). Mean duration from symptom onset at presentation was 5.5±2.6 months. Attention and memory were the most common domains involved (68.2%). Neurodegenerative dementias including Alzheimer's, fronto-temporal dementia, cortico-basal syndrome, etc were the most common etiology; 39(24.6%), followed by autoimmune encephalitis; 27 (17.08%) and infectious 24(15.1%); like cryptococcal, neurocystecercosis, subacute sclerosing panencephatis. Vascular cognitive impairment was the leading cause in 21(13.29%) patients. Neoplastic or metastatic lesions presenting with prominent cognitive decline was seen with 18(11.3%) patients with lymphoreticular malignancies being the most common cause. 12(7.5%) patients were diagnosed with probable CJD. Discussion and Conclusions: Neurodegenerative dementias remained most common cause for RPD, unlike most of the major series proposing prion diseases in current literature. Our series also highlighted the need for evaluating and treating autoimmune encephalitis and infections as a major cause for RPD.


Assessment of premorbid cognitive abilities in the Indian context

A. P. Porrselvi, V. Shankar

Department of Neurology, Sri Ramachandra University, Chennai, Tamil Nadu, India

Cognitive assessment of an individual is a challenge for any clinician. Ideally, a person's cognitive status should be compared to his or her own previous cognitive abilities to identify any cognitive decline. Often the clinician has to make clinical decisions based on subjective insight into the patient's demographic and socioeconomic status as there may be no previous cognitive evaluation done. A common approach to determining the premorbid intelligence and cognitive reserve is by using demographic details such as education and occupation but being a multilingual society with varying educational backgrounds and the lack of one standardized pattern in our education makes it difficult to estimate the premorbid cognitive abilities of our population. Also, it has been shown through research that intellectual development can happen well into adulthood and hence these demographic details alone might not offer accurate estimates. The other approach is by assessing the over learned skills like reading that show resistance to cortical dysfunction. But this skill is dependent on education and some studies have shown that reading skills may not always be resistant to cognitive dysfunction. The objective of the present study was to develop an informant based questionnaire that can yield a valid estimate of the premorbid cognitive abilities of an individual. This resulting instrument includes questions addressing education, occupation, language skills, using technology and the ability to carry out everyday activities successfully and independently that can possibly indicate a person's intellectual capabilities and cognitive reserve when the individual was cognitively and functionally at his or her best, preferably in the past 10 year time frame. The validation was done in a study that included older adults with and without cognitive impairment. The scores of a comprehensive neuropsychological battery were adopted as construct criteria. Inter-informant reliability was also tested in a sub-study with two relatives for some of the participants.


Sentence characteristics of the mini mental state examination among patients with parkinson's disease: A retrospective analysis

S. Chatterjee, S. Choudhury, M. U. Kulsum, P. Chatterjee, Banashree, Mondal, H. Kumar

Kolkata, West Bengal, India

Background: Appropriate usage of language is a principal cognitive domain which may be jeopardized in patients with neurodegenerative disorders. Therefore, writing a meaningful complete sentence is one of the assessment criteria in popular cognitive screening scale MMSE. Our objective was to undertake an in depth analysis of the sentences (from MMSE) written by patients with Parkinson's disease (PD). This neurodegenerative disease often present with motor and cognitive impairment and both of these might separately influence sentence writing skill. Methods: In this retrospective analysis we selected 101 MMSE source file with complete sentence written on it. The UPDRS III score, MMSE score and socio demographic details of patients were also recorded. After digitalization, the number of words and number of characters in each sentence was determined. Raters evaluated every sentence for 'emotional polarity' and 'concerns of health'. The statistical significance of these derived variables in various demographic and disease severity groups were assessed. Results: PD patients with cognitive impairment wrote sentences with 4.23 numbers of words compared to 5.09 numbers of words among patients with intact cognition. 14.3 % patients with intact cognition expressed health concerns in their written sentences compared to 3.2 % of cognitively impaired PD patients. Emotional polarity was comparable between with or without cognitive impaired groups. MMSE score emerged as the sole determinant for writing short sentence in multivariate analysis (adjusted p value 0.002*). Conclusion: PD patients with cognitive impairment wrote shorter sentences compared to PD patients with intact cognition. Expression of health concern is perhaps a natural process followed by mostly patients with intact cognition. Motor impairment did not appear as a significant determinant of characteristic short sentence production by PD patients with poor cognition.


Cognitive profile of cognitively impaired advanced NPH patients

B. Peethambaran, R. Mathew, Souda

Department of Neurology, PIMSRC, Tiruvalla, Kerala, India

Background: Literature on cognitive profile in advanced NPH dementia is sparse. Objective: To study the cognitive profile of cognitively advanced NPH in a hospital based cohort. Materials and Methods: The patients included were those availing dementia care services from 3 different tertiary care centers during a period of 5 years from2010 to 2014. Patients were considered to have cognitively advanced NPH, if the ACE score was <50. Patients underwent neuropsychological tests like ACE, Trail A, Trail B, RAVLT, CBI, HADS, IQCODE and ADL. Results: Detailed neuropsychologic assessment was done in 23 patients with cognitively advanced NPH and 15 patients with advanced AD. Among the 23 patients with NPH, the mean age was 72.06±9.62 years. Thirteen were males, mean education level was 7.74±3.21 years and mean duration of illness was 2.73±2.7 years. The mean MMSE score was11.6±5.2 and mean ACE score was 27.26±1.3. Among ACE subscores, most severely affected was memory (mean score6.7±4, PMS 19.41±11.58) and least affected was language (mean score 15.56±8.25, PMS 37.06_/-19.63). Significant difference was not seen between ACE scores and sub scores, when compared with 15 patients with advanced AD. Discussion: The cognitive profile of NPH at advanced stage was similar to advanced AD. Conclussion: As advanced NPH shows cognition similar to cortical dementia, so the pathological correlate in NPH may not be hydrocephalus alone.


Two cases of vanishing white matter disease

A. N. Kumar, L. Pauline, G. Sarala, S. E. I. Fazal, K. Shunmugasundaram, S. Gopinathan

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

Introduction: Vanishing white matter disease, also known as Childhood Ataxia with Central Hypomyelination (CACH) is a rare inherited leukodystrophy and the presentation is preceded by head trauma or infection in childhood. Case 1: A 5 year old boy, who had normal developemental milestones, admiited with the history of fever, diagnosed to have viral encephalitis and treated. After 2 weeks, he started insidious onset, gradually progressing proximal weakness of the limbs, difficulty in walking with development of broad based gait and unsteadiness while walking with clumsiness, with normal cranial nerves, power >3/5 in all the limbs, brisk DTR with appendicular, gait and stance ataxia. MRI showed bilateral symmetric white matter hyperintensities, suppressed with FLAIR without contrast enhancement. Case 2: An 8 year old boy, with normal developmental mile stones except speech delay, had h/o fever with altered sensorium and seizures at 2 years, followed 1 month later, he developed insidious onset, gradually progressing weakness of all the 4 limbs, with bipyramidal spastic quadriparesis and appendicular ataxia of all the limbs. This course is further worsened by recent fever when he was found to have pneumonia. Patient developed oculomotor apraxia, patellar and ankle clonus. MRI brain showed bilateral periventricular, subcortical white matter hyperintensity, suppressed on FLAIR. Discussion: Most cases are encountered in childhood or adolescence. Presentation is preceded by minor head trauma or infection, in an otherwise normal or near normal developmental milestones. With repeated illnesses, patients accumulate neurological impairment and succumbing to the illness. Hyperintensities suppressed on FLAIR is the characteristic radiological finding. Cerebellar atrophy is variably present and ranges from severe to mild and typically involves the vermis. Conclusion: Although the disease is spontaneously remitting, each episode results in accumulation of further injury. Preventing head trauma and infection is main strategy to delay the onset of the disease.

DP15/186. Withdrwan


Comparison of frontal lobe behavior in patients of cortical (behavioural varient of fronto-temporal dementia) vs. subcortical (dementia with parkinsons disease) dementia with emphasis on significant discriminating symptomatology

D. S. Saini, A. Biswas, A. Roy

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

Background: Patients of behavioral variant of frontotemporal dementia (bvFTD) exhibit prominent behavioral abnormalities along with executive dysfunction. Whereas executive dysfunction is also common in subcortical dementias because of disturbance in fronto-subcortical connection behavioral symptoms are less frequent. Objectives: To determine the frequencies of behavioral symptomatology of patients with subcortical (PDD) and frontal cortical dementia (bvFTD) and to find out the discriminating value of behavioral characteristics in differentiating these two group of patients. Methods: Patients were consecutively recruited from Cognitive and Movement Disorder Clinics following standard diagnostic criteria. A total 11 bvFTD and 8 Parkinson's disease dementia (PDD) were recruited. Behavioral symptoms were noted from reliable caregiver following the questionnaire prepared by Bathgate et al. (2000). Results: Whereas eating and vegetative behaviours were more frequent in bvFTD (91% vs 49 %, p=0.068), the neuropsychiatric behaviors were more common in PDD group 87.5% vs27.2%, p=0.02). Although didn't achieve statistical significance, other symptoms found to be discriminating were (i) sensory behavior (p=0.16), (ii) cognitive mediated behavior (p=0.35), (iii) affect-social behavior (p=0.36) and (iv) compulsion-ritual behavior (p=0.37). The behavior related to environmental dependency were not found prominent in either groups (p =0.95). Conclusions: Behavioral abnormalities are more common in bvFTD group with prominent abnormality in eating & vegetative behavior, sensory behavior, cognitive mediated behavior, affect-social behavior, compulsion-ritual behavior and the PDD group showed marked neuropsychiatry behavioral abnormalities.


Behavioral and psychological symptoms of dementia in patients with various dementia

M. U. Kulsum, S. S. Anand, S. Choudhury, P. Chatterjee, B. Mondal, H. Kumar

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

Background: Behavioral and Psychological Symptoms are frequent coexisting symptoms in patients with Dementia. These symptoms ought to depend on the type and extent of dementia, as the pattern of anatomical involvement in these diseases often differs substantially. Majority of people with dementia experience aggression, apathy, and psychosis, these symptoms are known as the behavioral and psychological symptoms of dementia (BPSD) by the International Psychiatric Association. Objectives: (1) To find out the prevalence of neuropsychiatric symptoms in patients with dementia. (2) To find out the relationship between cognitive impairment and neuropsychiatric symptoms as well as the severity of caregivers distress in various neurological disorders. Methods: We recruited 178 patients with dementia s as per DSM IV TR criteria, from the neurology Out Patient Department of Institute of Neurosciences- Kolkata. Mini Mental State Examination (MMSE) was utilized to estimate the cognitive status of the patients. Objective and uniform identification of neuropsychiatric symptoms were performed using Neuropsychiatric Inventory (NPI). Results: The mean age of these patients was 64 years (SD 9.28) and 75% were male. In this study 37.1% patients were with Parkinson's disease (PD) that is due to more number of patients with Parkinson's disease has come to our tertiary care centre, 21.9% patients were with progressive supranuclear palsy (PSP), 15.7% were with Alzheimer's disease (AD), 9% patients were with vascular dementia, 3.9% patients were with multisystem atrophy, and 2.8% patients were with normal pressure hydrocephalous. There is difference in cognitive decline and neuropsychiatric symptoms, the difference are significant in delusion (p- 0.00*), aggression (p- 0.013*), apathy (p- 0.016*), disinhibition (p- 0.019*), and aberrant motor behavior (p-0.003*). Among neuropsychiatric symptoms, psychosis and anxiety is more in Parkinson's disease Dementia, where as aggression, apathy and disinhibion is more with the patients with AD and PSP. Caregiver distress for hallucination is severe in Parkinson's disease dementia, whereas in aggression, apathy, and irritability is severe in Patients with AD, PSP, and vascular dementia, and caregiver distress is severe in anxiety and depression is more in Parkinson's disease dementia and vascular dementia. Caregiver distress is found severe in delusion and hallucinations with the patients with Parkinson's disease were as it is severe in aggression and elation with the patients with vascular dementia, AD, PSP. Conclusion: All neuropsychiatric symptoms were comparatively more frequent among patients with cognitive impairment especially delusion, aggression and depression were significantly higher among them. Psychosis, aggression, and elation are responsible for severe caregiver's distress. The patients with dementia should be routinely screened for all neuropsychiatric symptoms using standard tests, as the prevalence of behavioral and psychological symptoms is higher in these groups. The presence of one symptom should alert the clinicians to look for other features.


Functional network mapping of language code switching

K. P. Patwardhan, P. S. Mathuranath, J. S. Saini, V. P. Vandana, L. George, R. Panda

Department of Neurology, NIMHANS, Bengaluru, Karnataka, India

Introduction: Study of switching between first learnt and subsequently acquired languages using functional imaging in bilinguals has been an interesting topic. The aim of this study was to evaluate the language code switching between Kannada and Telugu in late bilinguals. Methodology: 6 right handed healthy volunteers with a mean age - 30.83 ± 4.5years, mean no of education- 14±4years, mean no of languages known 3±1. Language experience and proficiency (LEAP) questionnaire was used for selection of language (L1-Kannada); International second language proficiency rating (ISLPR) for language (L2-Telugu). Structural and functional MRI was done for all. Forward switch from L1 to L2 was studied in task based fMRI with visual stimuli. Task was presented in block design paradigm consisting of 6 active and 6 rest blocks. Each active block was followed by rest block. In active block, L1 image was followed by L2 image. Subjects were asked to respond in Kannada or Telugu as per color cues of code switching. FMRI data was acquired with EPI sequence (Total Dynamics: 218, TR-2sec, TE-36sec, 3x3x3mm slice thickness); structural MRI with T1weighted images, acquired in a 3Tesla scanner (Skyra, Siemens). FMRI data was processed in SPM8 (software) using general linear model with HRF function by modeling the onset time and duration of stimuli. The group level analysis was done with p<0.05 level of significance with False Discovery Rate (FDR) correction. Results: We studied the areas recruited by the brain during switching from L1 to L2 and found that the predominant activation was in left hemisphere and mostly in the frontal lobe. Middle & Inferior frontal gyri (followed by inferior parietal lobe) were most consistently activated. Conclusion: Depth of brain activation in language switching showed that the left sided frontal gyri could be the seat of language switch from Kannada to Telugu in late proficient bilinguals.


Pathological laughter as a presenting feature in two cases of cadasil

G. Shankar, K. Venkatraman, M. G. Kaushik, V. Kannan

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

Objectives: To emphasize the importance of suspecting CADASIL in young patients presenting with sudden onset unprovoked laughter and crying and a positive migraine history. Background: Pathological laughter and crying (PLC) complex is an entity that arises due to lesions that damage frontopontine tracts that inhibit the putative laughter centre in the brainstem. Demyelination is the most common cause followed by motor neuron disease to cause PLC in young patients. Vascular etiology is commonly in elderly. We present two cases of CADASIL that presented with pathological laughter. Study Design: Observational case series done at Institute of Neurology, Madras Medical College. Case Vignette: Case 1: 35 year old male presented with unprovoked laughter for 1 month. On examination he had brisk deep tendon reflexes and reduced executive functions of the prefrontal cortex. MRI revealed bilateral periventricular white matter hyper intensities in the temperoparietal cortex suggestive of CADASIL. He had a strong history of migraine with aura since 20 years of age. Case 2: An elderly female presented with unprovoked laughter and crying for 20 days and a past history of migraine in her mid-age. She also had a brother who had a history suggestive progressive dementia, migraine and died 4 years ago. Her MRI showed bilateral subcortical white matter hyper intensities suggestive of CADASIL. Both the cases were earlier evaluated by psychiatrist and treated for behavioural disorder. Conclusions: CADASIL presenting as pathological laughter is a rare presentation. History of migraine and strong family history should invoke a suspicion of CADASIL in such patients along with other differentials consider.

DP20/33. Withdrawn

Poster Session 10; Miscellaneous

11:00-14:00 h, Sunday, November 13, 2016


Hypopigmented skin and hair in infants: An important diagnostic clue in neurology

M. Sreedharan, D. Kalpana, P. A. M. Kunju, M. Iype, S. Chalipat

Department of Pediatric Neurology, Government Medical College, Trivandrum, Kerala, India

Introduction: The aetiology for hypo pigmented skin and grey hair in infants are diverse. Some of them are associated with global developmental delay or regression. The common differentials include aminoacidurias like phenyl ketonuria and tyrosinemia. The three cases are presented here to create awareness of these rare conditions and to highlight the importance of accurate genetic diagnosis in prenatal counselling. Case Reports: 1) A 4 month old male baby with global developmental delay was admitted for respiratory distress. The parens were first cousins. The baby was very fair, floppy since birth and had white hair. The hair examination revealed pili torti making us suspect Menke's disease. The serum ceruloplasmin was very low and MRA showed marked ectasia of vertebrobasilar system. The diagnosis was proved by demonstration of ATP 7 B mutation. Baby was started on subcutaneous copper histidinate without much improvement. 2) 7 months old girl child, first born of non consanguineous parentage presented with global developmental delay generalized hypopigmentation of skin and hair. Child had microcephaly, dysmorphic features, cataract, hypotonia with brisk deep tendon reflexes. Hair microscopy was normal. Child had cardiomyopathy and corpus callosal agenesis. BERA was abnormal suggestive of severe sensory neural hearing loss. Immunodeficiency panel normal. The constellation of findings favoured a diagnosis of Vici syndrome. DNA sent for EPG5 mutation. 3) One year old girl came with global developmental delay, fair skin, hypopigmented brittle hair and seizures. The serum ceruloplasmin was normal and there was no immunodeficiency. The MR imaging showed moderate cerebellar atrophy. The examination of hair under polarized microscope showed the typical tiger tailed appearance suggestive of trichothiodystrophy. Conclusions: In infants with generalized hypopigmentation, examination of hair, s.aminoacid levels, serum copper, ceruloplasmin levels, MR imaging abnormalities and presence of immune deficiency helps in narrowing the differential diagnosis. Accurate genetic diagnosis is possible in most of these disorders.


Adrenal adenoma with acutely progressive hypokalemic quadriparesis: Case report and review of literature with emphasis on CPK

A. S. Verma, V. K. Rai

Department of Neurology, UP University of Medical College, Uttar Pradesh, India

We present a case of 46-year-old man presenting with fatigue, myalgias, and acutely progressive weakness with raised CPK level of 12328 U/L. He was not able to eliminate the effect of gravity during eating or combing. He had difficulty in standing up from sitting position and also acute onset (24 hour) difficulty in holding the neck. Previously also he was hospitalized for similar complaints of quadriparesis but of lesser intensity and that improved in a short span of time. He was hypertensive and diabetic. Findings of metabolic alkalosis and significant hypokalemia (1.5 mmoi/L) suggested hyperaldosteronism and CECT revealed a right adrenal adenoma. When rhabdomyolysis occurs in a patient with hypokalemia and metabolic alkalosis, primary hyperaldosteronism has to be suspected: if confirmed, an aldosterone-producing adenoma is the most probable cause.


A case series of VGKC positive neuromyotonia triggered by mercury toxicity

V. V. Iyer, R. Rithesh, R. Sridharan, Nagarajan, B. Shankar, D. Anusha

Department of Neurology, SRM Institute for Medical Sciences, Chennai, Tamil Nadu, India

Introduction: We are presenting a series of three cases of antibody positive neuromyotonia. Their clinical manifestatations and response to therapy is documented. Materials and Methods: Patients were included when they presented with features of motor neuron hyperexcitability and EMG showed neuromyotonic discharges. Results: Of the 3 patients, 2 were males and 1 was a female patient. Mean age at presentation was 34 years (range: 28-49 years). Mean duration of symptoms was 15 days (range: 12-20 days). All patients presented with subacute onset of pain and stiffness in legs, continuous muscle twitching, muscle cramps, hyperhydrosis and insomnia. Two of them had fluctuating blood pressures, with one developing seizures secondary to PRES. Interesting all 3 patients had consumed native ayurvedic medications for sinusitis, bone and jointrelated problems and had high mercury levels, with one patient having received hemofiltration for the same (21-44 microgram/litre, normal <5 microgram/ litre). All patients had widespread neuromyotonic discharges on EMG and positive CASPR-2 subtypeof VGKC antibodies. Patients received 5 days of IV Methylprednisolone 1gm for 5 days, followed by a short course of oral steroids. All patients were asymptomatic at a mean follow up of 8 months (range: 5-11 months). Discussion and Conclusion: Acquired neuromyotonia is a rare VGKC associated autoimmune channelopathy. The symptoms can be triggered by heavy metal exposure (eg: mercury). A high index of suspicion is required for the diagnosis. Patients show a good response to immunotherapy.

OP34/334. Withdrawn


Paradoxical worsening with antituberculous therapy in immunocompetent patients with cns tuberculosis

J. Goel, S. S. Nair

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

Background: Tuberculosis is still a major cause of serious illness in developing countries like India. The objective of this study was to assess the clinical and radiological profile of immunocompetent adult patients with central nervous system tuberculosis (CNS TB) who had paradoxical worsening with antituberculous therapy (ATT). Design: This was a retrospective study by chart review over a period from January 2010 to December 2015 in a single tertiary care centre. The subjects were the immunocompetent patients with CNS TB who developed paradoxical clinical, CSF or radiological worsening with ATT. All patients with the diagnosis of CNS TB were screened and those fulfilling the inclusion criteria were selected for the study. Results: Paradoxical response in the form of worsening of clinical features, and radiological features was seen in 33 out of 61 (54.1%) patients with CNS TB following initiation of ATT. The common clinical paradoxical responses were raised intracranial tension (8 cases), focal neurological deficits (9) and altered sensorium (9). The mean time to clinical worsening was 5.84 months (range 1 - 12 months). Treatment initiated in this group was ATT along with steroids in 23 cases (69.7 %) and ATT alone in 10 (30.3 %). Co-existing pulmonary tuberculosis was present in 9 (27.3 %) cases. Mean baseline CSF parameters in this group showing paradoxical worsening was CSF leucocyte count204 cells/cmm, CSF protein 311 mg/dl and CSF sugar / blood sugar ratio 0.44. Radiological abnormalities at baseline were seen in 19 cases, among these granulomas in supratentorial and infratentorial location in 11 cases, leptomeningitis (4 cases) and infarcts (4 cases). Radiological paradoxical responses most commonly observed were appearance of new granulomas (20 cases), leptomeningitis (26), fresh infarcts (6), appearance/ worsening of hydrocephalus (n-6), opto-chiasmatic arachnoidits (3) and syringomyelia (3). Radiological paradoxical worsening in absence of clinical worsening was seen in 3 (8.5 %) cases . Conclusion: Paradoxical reactions are common in immunocompetent individuals with CNS TB occurringin nearly half.


Clinical profile, evaluation, systemic associations and outcomes of patients with idiopathic intracranial hypertension in a tertiary referral center in Southern India

A. I. A. Shaikh, M. Alexander

Department of Neurology, Christian Medical College, Vellore, Tamil Nadu, India

Background: Idiopathic intracranial hypertension is characterized by features of raised intracranial pressure in absence of any other intracranial pathology such as mass lesions, infections or venous thrombosis. Intracranial hypertension has also been associated with systemic diseases. This study aims to describe the clinical profile of patients including imaging findings and intracranial and systemic associations. Patients and Methods: This is a case series of all patients diagnosed to have intracranial hypertension from Jan 2011 to Dec 2014 and had required admission for the same. Analyses were done retrospectively. 52 patients had diagnosis of idiopathic intracranial hypertension. Their clinical profiles were noted, images reviewed and investigations including CSF studies analyzed. Patients kept a follow up in outpatient clinic. Results: 52 patients were evaluated. Age group ranged from 14 years to 44 years. Most patients were females (49/52). Headache was present in most patients (47/52). Diplopia was present in 16/52. Vision loss was present in 39/52, of varying severity from subclinical to complete vision loss. 9/52 patients had vomiting. All patients had papilledema. Obesity was present in 22/52 patients. 12 patients had dysfunctional uterine bleeding requiring hormonal therapy. 8 patients had hypocortisolemia. Common imaging findings were that of optic nerve tortuosity, prominent perioptic subarachnoid spaces and empty sella (41/49, 12/49 and 23/49). Bilateral transverse sinus/transverse = sigmoid junction stenosis was very common and was seen in 39/49 cases. CSF opening pressure ranged from 21 cm of CSF to 40 cm. All patients were started on medical treatment with diuretics most commonly acetazolamide. Optic nerve sheath fenestration was done in 4 patients, and 6 patients required lumboperitoneal shunting.


An exploratory study to investigate the association between short-term exposure to air pollution on the occurrence of ischemic or hemorrhagic stroke in a case crossover study design

A. K. Pandit, K. Prasad, A. Kumar, P. Kumar

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

Background: A rise in solid or liquid pollutants suspended in the air, including nitrates and sulphates emitted from road traffic, construction, and industry, was found to adversely affect health in both the short and long-term. Levels of air pollution are effective factors in occurrence of stroke. In India, to our knowledge there is no study reported till date that has investigated the association between ambient air pollutant and occurrence of stroke or TIA. Methods: The study design was a case-crossover study. The case crossover design is a variant of the matched case control study and consists of only cases, which serve as their own controls in the analysis. The data collection was partly prospective and partly retrospective. Results: Out of 952 cases of stroke/TIA screened, 401 cases were included in the final analysis of the study. The data was collected prospectively from 2 nd September 2014 till 30 th September 2015, and retrospectively from 1 st January 2012 till 1 st September 2014. There were 74% male patients with male to female ratio of 3:1. The mean age of cases was 54.1±13.8 years. There was significant association between short term exposure of SO 2 and risk of occurrence of hemorrhagic-stroke among females OR 6.86 (1.7 to 27.5), p=0.007. A borderline significant association was also found between short term exposure of PM10 and ischemic-stroke among females OR 2.09 (0.975 to 4.512), p=0.058. However there was no statistical difference between risk of occurrence of stroke and mean level of various studied air pollutants; overall, depending on type of stroke, and subtypes of ischemic stroke based on TOAST classification. Conclusion: There is marked and close association of occurrence of ischemic stroke and hemorrhagic stroke among females with short term exposure to raised PM10 and SO 2 air pollutants levels respectively. Prospective studies with larger sample size needed for precise association.


Utility of IQCODE in normal pressure hydrocephalus

S. Raj, P. Sauda, R. Mathew

Department of Neurology, Sree Mookambika Institute for Medical Sciences, Kanyakumari, Tamil Nadu, India

Introduction: Being an informant based assessment, IQCODE may have limitation in patients with subcortical dementias where motor impairment may be substantial than cognitive impairment. Aim: To study the use of IQCODE in patients with Normal Pressure Hydrocephalus (NPH). Materials and Methods: The patients included those availing dementia care service from 3 different teretiary care centres during a period of 3 years from 2013 to 2016. Patents were diagnoised based on consensus criteria. In addition to ACE patients underwent a battery of other neuropsychologic tests including Digit forward test, Trail A and Trail B , The Rey Auditory Verbal Learning Test ( RAVLT), CBI, Hospital Anxiety and Depression Scale (HADS), IQCODE and ADL. Results: Of 153 patients with NPH, IQCODE was available in 27 patients with normal pressure hydrocephalus. Mean age was 77.19 (SD 5.7), 70% were males. Mean duration of illness at the time of presentation was 2.08 (SD 1.11). Total ACE score was 19.2 (22.6) thereby indicating advanced dementia. IQCODE rates each items from 1 to 5, values above 3 indicating impairment. Mean of total score was 100.43 (14.17) and the absolute score 3.86 (normal <3.4). The values for subscores were memory 3.75, executive dysfunction 3.88, language 3.94 and calculation 3.95 which showed that language was more involved than memory. For comparison purpose the ACE scores were converted into percentage of maximum score was for language (31.85) and minimum score for memory (12.05). Visuospatial was (20.9) and executive function (21.36). Good correlation was seen among ACE total score and subscores. Similarly good correlation was seen between IQCODE total and subscores. However no correlation was seen between ACE and IQCODE as far as total scores and subscores were considered. Conclusions: In this cohort of patients with advanced dementia no correlation was seen between cognition as assessed by IQCODE and ACE.eing a screening instrument may have limited utility in advanced dementia. IQCODE however the number of cases were small to come to definite conclusion.


Prevalence of peripheral neuropathy in chronic kidney disease patients

P. C. Shekar

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

Background: Chronic Kidney Disease (CKD) is recognized as a major health problem. Prevalence of CKD is rising continuously; mostly CKD is affecting the elderly aged population or patients with diabetes and hypertension. Present study was aimed to explore clinical manifestation and evaluate the prevalence of peripheral neuropathy and peripheral nerve dysfunction in CKD patients attending our hospital with reference to the severity and duration of the CKD. Methods: The present cross sectional study was conducted in 50 patients affected with chronic kidney disease, of di fferent age groups at GANDHI Hospital, Hyderabad. The presence of peripheral nerve dysfunction was assessed by nerve dysfunction clinically (motor or sensory symptoms and signs) and electrophysiological nerve conduction studies. Results: Out of 50 patients, 60% of study population was suffering from chronic kidney disease with peripheral nerve dysfunction. The results shown that the rate of prevalence of peripheral nerves dysfunction was observed higher in subjects with longer duration of CKD. Conclusion: This study enlightens the prevalence and clinical presentation of peripheral nerve dysfunction in patients with CKD. The CKD was found to cause peripheral neuropathy including overt and subclinical neuropathy, of which distal symmetrical sensory motor neuropathy was common in CKD. The prevalence of peripheral neuropathy was directly proportional to duration and severity of CKD.


Neurology and MRI Findings in IIH

S. Kumar

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

Introduction: Idiopathic intracranial hypertension (IIH) is a headache syndrome of unknown etiology characterized by raised CSF pressure in the absence of an intracranial mass lesion or ventricular dilation. IIH predominantly affects women of child bearing age. Normal neurologic examination with papilledema on fundus examination of the eye is common. IIH is also called as psuedotumor cerebri and benign intracranial hypertension. Methods: Patients diagnosed as study subjects with IIH were recruited according to the diagnostic criteria. All patients underwent MRI with MR venography to exclude venous sinus thrombosis or structured CNS lesions. Clinical, radiological imaging features of IIH of 27 patients were studied. Causes of ICT intracranial tension were excluded. Secondary clinical features like headache were excluded. Secondary clinical features like Head ache were prominent. MRI findings were studied regarding pituitary and optic nerve. Results: In all patients diagnosis was newly established. Bilateral papilloedema was documented in all patients by opthalmological examination including fundoscopy. Patients above 18 years older with no history of glaucoma and amblyopia or diseases of the optic nerve are included. A total of 27 cases were analysed during the period of July 2015 - June 2016. Conclusion: No specific mortality risk is there in IIH. Obesity has a selective role in this group because of the strong prediction for female, fertile, obese patients, it is possible that optic patients, it is possible that optic nerve head change could predict future visual outcome n IIH patients regarding the visual loss. Patients are instructed to return to the neurologist when symptoms recur.


Relationship between factor V leiden gene variant and risk of ischemic stroke: A case control study

A. Kumar, S. Misra, R. Sagar, P. Kumar, K. Prasad

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

Background: Factor V is most prevalent genetic variation which leads to prothrombotic state therefore considered as important gene for understating stroke mechanism. The present study aim to determine the association between factor V gene polymorphism and risk of stroke in North Indian Population. Methods: In a hospital based case control study ischemic stroke patients and controls were recruited from outpatient Department of Neurology, All India Institute of Medical Sciences, New Delhi, India. Two ml of venous blood samples were collected by venipuncture in vacuum tubes from both cases and controls. Demographic and risk factor variable were collected in standardized data collection form. Deoxyribose nucleic acid (DNA) was isolated by Phenol-Chloroform method. Polymerase chain reaction - Restriction fragment length polymorphism (PCR-RFLP) was used genotyping. Data were analyzed using software STATA version 13. Results: 250 cases and 250 control subjects were recruited in the present study. The mean age of IS patients were 52.83 ± 12.59 years, in control group were 50.97 ± 12.70 years. Genotypic frequency distributions were in accordance with HWE in both cases and controls. Hypertension, Diabetes, Dyslipidemia, Smoking, Alcohol intake, Low Socioeconomic Status, Family history of stroke and Family history of Diabetes were significantly associated with risk of stroke. Conditional logistic regression analysis showed an independent association between Factor V Leiden G1691A gene polymorphism with the risk of large vessel subtype of Ischemic stroke (OR 5.17; 95% CI 1.32 to 20.3, P value = 0.01). Conclusion: Present study suggests that Factor V Leiden G1691A polymorphism may be an important risk factor for large vessel subtype of ischemic stroke. Prospective studies with large sample size need to confirm the present findings.

OP42: Withdrawn


A study of clinical profile, risk factors, etiology and prognosis of patients with posterior circulation ischemic stroke

S. Edara

Department of Neurology, Aayush Hospital, Vijayawada, Andhra Pradesh, India

Background: Posterior circulation stroke accounts for approximately 20% of all strokes with varied clinical presentation which differ from strokes in anterior circulation with reference to etiology, clinical features and prognosis. Materials and Methods: We prospectively analyzed 121 patients of posterior circulation ischemic stroke from January 2014 to June 2016 at Aayush Hospitals, Vijayawada. Patients were analyzed for demographic characteristics, stroke risk factors, clinical characteristics, etiological subtypes and prognosis. Results: Our patients age range from 30 to 92yrs with mean age of 60±13. Maximum number of patients were in 56-65 age group (37.5%). Male to Female ratio of our study group was 3.3:1. Hypertension was found to be the most common risk factor (88%) followed by diabetes (63%) and active smoking (52%). Ataxia was the most common clinical symptom (49%) followed by motor weakness(44%) and vertigo(36%). 34% of patients had large artery disease, 31% had small artery disease 20% had documented cardioembolic source. Posterior cerebral artery was most commonly involved vessel. Topographically distal intracranial involvement was most frequent (66.25%). Mortality in hospital was 5%. 14% patients were severely morbid. Conclusions: This study from south India describes the pattern of risk factors, clinical characteristics, etiological subtypes and prognosis of posterior circulation ischemic stroke in our population. Hypertension was most common risk factor, ataxia was most common clinical presentation, large artery disease was most common stoke subtype and distal territory involvement was most common in our study.


Paradoxical worsening with antituberculous therapy in immunocompetent patients with CNS tuberculosis

Jitesh Goyal

Background: Tuberculosis is still a major cause of serious illness in developing countries like India. The objective of this study was to assess the clinical and radiological profile of immunocompetent adult patients with central nervous system tuberculosis (CNS TB) who had paradoxical worsening with antituberculous therapy (ATT). Design: This was a retrospective study by chart review over a period from January 2010 to December 2015 in a single tertiary care centre. The subjects were the immunocompetent patients with CNS TB who developed paradoxical clinical, CSF or radiological worsening with ATT. All patients with the diagnosis of CNS TB were screened and those fulfilling the inclusion criteria were selected for the study. The details of presentation, investigations and treatment were recorded using a structured proforma. Results: Paradoxical response in the form of worsening of clinical features, and radiological features was seen in 33 out of 61 (54.1%) patients with CNS TB following initiation of ATT. The common clinical paradoxical responses were raised intracranial tension (8 cases), focal neurological deficits (9) and altered sensorium (9). The mean time to clinical worsening was 5.84 months (range 1 - 12 months). Treatment initiated in this group was ATT along with steroids in 23 cases (69.7 %) and ATT alone in 10 (30.3 %). Co-existing pulmonary tuberculosis was present in 9(27.3 %) cases. Mean baseline CSF parameters in this group showing paradoxical worsening was CSF leukocyte count 204 cells/cmm, CSF protein 311 mg/dl and CSF sugar/blood sugar ratio 0.44. Radiological abnormalities at baseline were seen in 19 cases, among these granulomas in supratentorial and infratentorial location in 11 cases, leptomeningitis (4 cases) and infarcts (4 cases). Radiological paradoxical responses most commonly observed were appearance of new granulomas (20 cases), leptomeningitis (26), fresh infarcts (6), appearance/ worsening of hydrocephalus (n-6), opto-chiasmatic arachnoidits (3) and syringomyelia (3). Radiological paradoxical worsening in absence of clinical worsening was seen in 3(8.5 %) cases. In addition, 3 cases showed resistance to first - line ATT, necessitating initiation of second line ATT. The mortality rate seen in this group of patients was 2(6 %) cases. Conclusion: Paradoxical reactions are common in immunocompetent individuals with CNS TB occurringin nearly half of them. A significant proportion of patients who showed paradoxical worsening had radiological abnormalities at baseline.


An interesting case of diagnostic dilemma creutzfeldt - Jakob disease Hashimotos encephalopathy

B. L. Narsimha Reddy, B. Vengamma, K. S. Rao, T. Naveen

Department of Neurology, SVIMS, Tirupati, Andhra Pradesh, India

Introduction: Creutzfeldt-jakob disease and Hashimotos encephalopathy often show similar clinical presentation, but Hashimotos encephalopathy is particularly important as it is treatable. Thus, in case of probable Creutzfeldt- jakob disease with high of antithyroid antibodies, immune modulation is performed with expectation of complete recovery from Hashimotos encephalopathy. Herein we provide the rare case where there is no effect of immune modulation. Case Details: 71 year old female medical professional presented with involuntary jerky movements of all the limbs for the past 6 months and progressive decline in cognition for the past 4 months. On examination there is myoclonic non startle jerks of limbs with dystonic posturing. On evaluation there is significant elevation of antithyroid antibodies in serum (anti TPO = 65.7 IU/ml; anti TG = 48 IU/ml); cerebrospinal fluid (anti TG = 3.2 IU/ml) and follow up (serum anti TG = 45 IU/ml), other neuronal antibody and collagen vascular profile was negative. No evidence of primary on whole body PET. Magnetic resonance imaging showed hyperintensities in bilateral cortex, caudate nucleus and putamen. Serial electroencephalogram showed periodic sharp and slow wave complexes at a frequency of 1-1.5HZ. Inview of antithyroid antibodies being positive patient was treated with corticosteroid pulse therapy subsequently IVIG for which she did not respond and later with Rituximab; however she did not respond and landed in akinetic mute state requiring three antiepileptics with optimal doses to control her continuous myoclonic seizures. Conclusion: This is a rare case where there is no improvement with immune modulation inspite of elevated antithyroid antibodies in a case of probable Cretzfeltd-jakob disease with features of Hashimotos encephalopathy. So this case highlights the importance of considering the no effect of immune modulation which is documented in only one case till date.


Comparison of the clinical profile of patients with anti NMDA - R And Anti VGKC encephalitis

P. Santhosh, Philo Hazeena, Tripthi, V. Shankar

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

Introduction: Autoimmune encephalitis is a treatable cause of subacute onset of cognitive decline and confusion. Autoantibodies can be divided into those that target the extracellular domains of membrane molecules and those against intracellular targets. Common antibodies are Anti VGKC (LGI1 and CASPR2), NMDAR and GAD. Aim: To compare the clinical profile of patients with Anti NMDA-R and Anti VGKC encephalitis in a tertiary care centre. Methods: Study was done among 96 patients admitted under neurology department in Sriramachandra medical college with features of behavioural disturbances, cognitive decline and new onset seizures over 1 year. Detailed clinical evaluation, csf analysis, neuroimaging and Anti NMDA-R and Anti VGKC antibodies were done. Patients positive for Anti NMDA-R and Anti VGKC were included. Results: Among the 96 patients, 11 patients were positive for autoimmune antibodies. 4 of them were positive for Anti VGKC LGi1 antibody, 1 for Anti VGKC - CASPR2 and 6 patients were positive for Anti NMDA-R antibodies.

OP47/AB12: Long term outcome of drug resistant epilepsy in children

T. Raimalwalla, V. Udani, D. Mhatre

P.D. Hinduja National Hospital & MRC, Mumbai, Maharashtra, India

Objective: Our study aimed to determine the probability of seizure remission in children with drug resistant epilepsy (DRE) in a developing country. Methods: In a hospital-based child neurology clinic in Mumbai, India, patients diagnosed with DRE with age of seizure onset < 13 years and a minimum follow-up of 5 years from seizure onset were investigated for outcomes at last contact. Seizure remission was defined as no more than 1 seizure per year. Kaplan-Meier analysis was used to calculate the annual remission probability. Chi square /Kruskal Wallis tests were used to detect differences in clinical factors between those with seizure remission, those with improvement and those without improvement in epilepsy symptoms based on parent/care-giver reports. Results: Median total follow-up period was 11 years. Of the 55 patients, 22 (40%) were in seizure remission, 14 (25.4%) had improved and 19(34.5%) had not improved. While overall annual remission probability was 2.03%, remission probability in those with an IQ≥70 was 3% and in those with an IQ <70 was 2.48% per year. Children receiving trial of ≥5 AEDs were less likely to show seizure remission or improvement in epilepsy symptoms at last contact. Conclusions: The purpose of this study is to help prognosticate outcome for children with refractory epilepsy given today's medical advances. This long-term follow-up study of childhood DRE suggests that a significant improvement in seizure control occurs over time. The remission rate was not found to be significantly different between those with and without intellectual impairment. The only significant risk factor associated with a poor outcome was the use of ≥ five AEDs.

OP48/AB10: Withdrawn


Referral trends for epilepsy surgery in drug resistant temporal lobe epilepsy in India, 2000-2014

A. Asranna, A. Radhakrishnan

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

Purpose: Despite class I evidence for effectiveness of surgery in temporal lobe epilepsy (TLE) and recommendations for referral to surgical epilepsy centre on failing appropriate trials of first-line antiepileptic drugs (AEDs) in TLE, surgical treatment remains underutilized even in developed countries. We studied the patterns of referral to a tertiary epilepsy surgery centre with state- of- the- art facilities in India and analysed the trends in referral patterns over time . Materials and Methods: Data of patients who underwent long-term VEEG monitoring in our Institute, as part of pre-surgical evaluation were analysed from the prospectively maintained records. Patients who were older than 16 years and diagnosed to have intractable TLE after comprehensive evaluation were included in the study. They were divided into three groups comprising of five year periods each during which they underwent pre surgical evaluation, Group 1: Year 2000-2004; Group 2: Year 2005-2009; Group 3: Year 2010-2014. Referral data with particular reference to duration of epilepsy before referral, age at onset of seizures and number of AEDs tried before referral was analysed. Results: 1364 patients who fulfilled inclusion criteria were included in the study; 389 in Group 1, 487 in Group 2 and 488 in Group 3. There was a significant increase in age at onset of seizures (p=0.001) and age at evaluation (p= 0.009) but, the mean duration of epilepsy before referral to a surgical centre showed no significant change over time (p=0.612). Conclusion: Despite recommendations for early surgery in drug resistant TLE, no significant trend for earlier referral for pre-surgical evaluation was seen. This calls for intensification of efforts to increase awareness about benefits of early surgery in drug resistant TLE amongst medical practitioners.

OP50/AB02: Withdrawn


Multi-pronged treatment of insomnia - Outcomes from an apex sleep clinic in India

S. Poornima, G. Shukla, A. Gupta, M. Afsar, P. Agarwal

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

Introduction: This chart review examined the outcome of chronic insomnia patients coming to a quaternary care centre. Method: Study identified 102 patients with the diagnosis of insomnia during a study period of two years between 2013 and 2015. The distribution of patient's was done depending on the co-morbidity and treatment received. The treatment approach and life stress were identified and discussed. The outcome was then reviewed for those who followed up. Conclusion: It was observed that 91.4% of patient showed good response with the physician administered CBTi along with treatment for co morbid conditions.


Anaemia as a risk factor for acute ischemic stroke and its association with stroke severity and outcome: A prospective case control study

S. Sharma, D. Chowdhury, B. Singh, G. Khwaja

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

Introduction: Anaemia is an independent risk factor and predictor of in-hospital mortality in cardiovascular diseases. Its role in acute ischemic stroke (AIS) remains uncertain. Methods: A prospective case control study on 200 AIS and equal number of age and sex matched healthy controls was done. Anaemia diagnosed by WHO Criteria (Haemoglobin (Hb) <12 g/dl (women) and <13 g/dl (men)) and graded into mild (11-11.9 g/dl(women) and 11-12.9 g/dl(men)), moderate (8-10.9 g/dl) and severe (<8 g/dl). Stroke severity assessed by NIHSS and log DWI volume; outcome by modified Rankin scale (mRS), Barthel index (BI), mortality and recurrence at 3 months. Statistical analysis done using Epi-info 7.0. Results: Demographics: 145 males and 55 female AIS and 144 males and 56 female healthy controls studied. Anaemia: Although overall, anaemia occurred equally in the 2 groups (37% vs. 35%; p=0.754), moderate (33.78% vs. 4%; p=0.003) and severe anaemia (5.4% vs. 0%) was significantly more common amongst AIS. Anaemia was more common amongst female AIS compared to controls (60% vs. 37.5%; p=0.02). Severity and Outcome: Stroke was significantly more severe in anaemic compared to non anaemic AIS (mean NIHSS-10.4 ± 7.3 vs. 7.9±6.6; p=0.011 and log DWI volume-6.4 ± 28.6 vs. 2.7 ± 8.3; p=0.035)). 11.5% of the cases died at 3 months; mortality in female AIS with anaemia was significantly higher (24.24% vs. 0%; p=0.035). Five out of 6 AIS belonging to the lowest Hb quartile died. Conclusion: All grades of anaemia but only moderate to severe anaemia are risk factors for AIS in females and males respectively. Anaemia at admission predicts more severe stroke and worse outcome in AIS patients.

OP53/AB07: Witdrawn

OP54/226 Withdrawn

OP55/AB17: Withdrawn


Analysis of status epilepticus in a tertiary care center over a period of 5 years

S. Kohli, V. Mathew, M. Alexander, A. Sivadasan, K. Muthusamy, A. T. Prabhakar

Department of Neurology, CMC, Vellore, Tamil Nadu, India

Objectives: Primary Objectives: To find the proportion of RSE (Refractory status epilepticus) and SRSE (Super refractory status epilepticus) among the patients attending a tertiary centre over a period of five years; to analyse RSE and NRSE (Non refractory status epilepticus) in terms of frequency, etiology and outcome. Secondary objective: To find out early factors predicting RSE from SE. Methods: Patients were identified from discharge summaries database with key words of status epilepticus and records of the portable EEG machine from Jan 2011 to March 2016. Results: Total of 218 patients were included in study with 114 (52.3%) males, bimodal age preponderance age <5 years 30% (62/218), and second peak in age 15-65 years 52.8% (115/218), Pre-existing seizures were present in 34.4% (n=75). NRSE constituted 77.1% (n=168), RSE was 23% (n=50), out of 23% RSE 7.8% were SRSE (n=17 of 50 RSE). Acute symptomatic epilepsy 75.2 (n=164), GTCS 76.1 (n=166), coma 68.3 (n=149), unfavourable STESS (Status epilepticus severity score) 70.2 (n=153) were the most common presentations. Presence of coma (p=0.001, 95% CI 1.744-10.93, unfavourable STESS (3-6) (p= <0.001, 95%CI 1.883-13.274), abnormal EEG (electrographic seizures or persistent background abnormalities; p≤0.001, 95% CI 1.886-10.528) were the statistically significant predictors for RSE on bivariate analysis. The outcome variables of duration of hospital stay more than 7 days (p=0.033, 95% CI 1.1-9.6) and in hospital mortality (p =0.001, 95% CI 3.341-19.008) was statistically significant in RSE. On multivariate analysis unfavorable STESS (p=0.04, 95% CI 1.06-12.18 value) and abnormal EEG (p= 0.03, 95% CI 1.13-8.45) were predictive for RSE. Conclusions: RSE and SRSE constituted 15.1% (n=33) and 7.8% (n=17) of all SE. Unfavorable STESS score at presentation and abnormal EEG are predictors of RSE with predictability scores with AUC (area under curve) 73% (65%-80%) with a sensitivity of 96% and negative predictive value of 91%.


Chronic polyneuropathy - Clinical spectrum and etiological profile in a tertiary care hospital

Sweety Trivedi, A. K. Senapati, A. Pandit

Department of Neurology, Bangur Institute of Medical Sciences, Kolkata, West Bengal, India

Introduction: Polyneuropathy is a heterogenous condition involving nerves usually in symmetric length dependent fashion. The clinical pattern is heterogenous involving motor sensory and autonomic fibers. Chronic polyneuropathy afflicts 2.4%-8% of people of different age groups with an estimated incidence of 25-200/10000 persons per year and a prevalence of about 5%. There are few studies on chronic polyneuropathy in Eastern India. Hence we have decided to carry out this study. Aims of study: 1) To evaluate the cases of chronic polyneuropathy in local population of eastern India. 2) To explore the various etiological factors in this population. Materials and Methods: In this descriptive cross sectional study, consecutive patients of chronic polyneuropathy attending neuromedicine department in the study period between March 2015 to may 2016 were included with proper informed consent. Acute polyneuropathies and those who did not consent were excluded. Detailed history and neurological examination done according to pre structured proforma. Elctrophysiological study and relevant etiology guided investigations were performed. Analysis of data: using SPSS software. Results: In this study. Out of 83 patients so far analysed, 28 were female and 55 were male patients. Hereditary etiology was found in 8.4% patients. Among the patients with acquired causes, diabetes (29%) was the most common etiology followed by CIDP (18%) and vasculitis (9.6%). Other causes were paraproteinimic (6%), drugs and toxin (4.8%), MMNCB (6%), amyloid (3.6%). No definite cause could be elicited in10% patients. Conclusion: In this ongoing study diabetes was the most common cause of polyneuropathy but other causes like vasculitis and paraproteinemia should also be kept in mind due to their fairly treatable potential.


Comprehensive epilepsy programme: A preliminary experience from Indore

O. P. Lekhra, Rajneesh Kachhara, Atma Ram Bansal

Institute of Neurosiences, Medanta Hospital, Indore, Madhya Pradesh, India

Background: Epilepsy surgery for MTS patients is available in India for two decades in major cities. A beginning has been done to reduce the surgical treatment gap in this part of the country. Aim: To establish the epilepsy surgery program at Indore. Methodology: Two young adults of refractory seizures of MTS were selected as per criteria of the pre-surgical evaluation. Video EEG monitoring was done for three days to confirm the concordance of clinical semiology with radiological lesion. Psychometric analysis done to rule out underlying depression. Anterior temporal amygdalohippocampectomy was done. Intraoperatively EEG was done before and after resection to confirm that epileptogenic zone has been resected in toto. Results: Patients were continued with the same dose of antiepileptics and followed as per the protocol. Conclusion: Success of comprehensive epilepsy surgery programme involves multidisciplinary approach. The enormous surgical treatment gap can only be minimized by developing many more epilepsy surgery centers in India.


Clinical spectrum of central nervous system demyelinating disorders in a tertiary care centre of North Western India

M. Paul, A. K. Sharma

Department of Neurology, SMS Hospital, Jaipur, Rajasthan, India

Background: Central Nervous System (CNS) demyelinating disorders include Acute Demyelinating Encephalomyelitis (ADEM), Clinically Isolated Syndrome (CIS), Multiple Sclerosis (MS) and NMO Spectrum disorder (NMOSD). They have distinct clinical presentations and different long term treatment strategies. Aim: To analyze the spectrum of CNS demyelinating disorders, their clinicoradiological features and laboratory parameters to categorize them into a particular subtype. Materials and Methods: A total of 50 patients were retrospectively analysed who were diagnosed to have CNS demyelination based on clinicoradiological features. Results: CNS demyelination was more common in females (74%) and common age group was 30-40yrs (24%). Seventy nine percent of patients had monophasic illness. NMOSD was most common (39%), followed by MS (26%), ADEM (21%) and CIS (13%). Of all NMOSD patients 50% fulfilled the criteria for NMO, 68% presented with opticospinal symptoms, 80% had Long Extensive Transverse Myelitis (LETM) and NMO antibody was positive in 66%. All with NMOSD had P100 latency prolonged. The CSF cell count was high in 26% and CSF protein was raised in 46%. Relapse is seen in 2 NMO and 4 MS patients. ADEM is monophasic illness. One patient of ADEM has positive HIV serology. Discussion: CNS demyelinating disorders is more common in females in third to fourth decade. NMOSD is most common. NMO antibody positive and negative patient have similar clinical course. MS patients has frequent relapse followed by NMOSD. The patients with CIS should be kept under follow up for progression to MS. Conclusion: CNS demyelinating illness is a broad group of disorder. The clinical features, radiology and laboratory parameters helps in categorizing the illness which helps in long term management and follow up.

OP60/AB19: Withdrawn