NEU/01(P) CLINICAL PROFILE OF SEIZURE DISORDER IN CHILDREN AGED BIRTH TO 12 YEARS AND USEFULNESS OF VARIOUS DIAGNOSTIC MODALITIESHemant Gogia, Premila PaulVMMC & Safdarjang Hospital, Ansari Nagar, New Delhi

OBJECTIVE: to determine the etiology of seizure disorder in children (0-12 years) and usefulness of various diagnostic modalities used in the workup of seizure disorder METHODS:259 children attending hospital emergency room with primary complaint of seizure in the period between May 2004 to April 2005 were registered .A detailed history, clinical examination and relevant investigations like CSF examination (including PCR& Elisa for MTB& NCC), CXR,Mx, CECT Head, EEG were done and patient having a definite underlying cause such as suspected infective CNS pathology with abnormal CSF examination and with developmental, structural malformation were excluded, however patients having seizures with fever where no cause was found were included. RESULTS: out of 259 children, 144 (55.59% ) were males and 105 females (40.54% ). Maximum number of children were in the age group of 5-10 years (126). Children presented with GTCS 112 (43.24% ), 92 (35.52%) with complex partial seizures, 28 (10.81%) simple partial seizures ,6 (2.31%) with myoclonic seizures,3(1.15%) atonic seizures,2(0.77%) gelastic seizures and 3(1.15) had unclassified seizures. Among these there were 42 cases(16.21%) of febrile seizures, and 13 cases mimicked seizure activity and were subsequently classified as pseudo seizures : of which7 (2.71%) had breath holding spell and 6(2.31%) pseudo seizures ,so were not further investigated.112(43.24%) children presented in status epilepticus. 36 patients showed abnormal EEG. Out of which8were GTCS,24partial seizures and4 had myoclonic seizures.CECT head showed abnormality in 61(23.55%) cases(19 GTCS,42 partial seizure) and most common abnormality was single ring enhancing lesion (SECTL) 38(14.67%) cases. CXR and Montoux test were performed in all of them and were positive in 16(6.17%) and 19 (7.33%) children respectively. Out of 38 patients with SECTL, 18 had positive Elisa test for neurocysticercosis and 8 had positive PCR for Tuberculosis. CONCLUSION: Though in majority of the children diagnostic workup was negative and exact cause was not available. CTscan appeared to be most useful tool to find out probable etiology which can be supplemented by specific tests for neurocysticercosis and tuberculosis.

NEU/02(O) CLINICAL PROFILE AND OUTCOME OF PEDIATRIC NEUROCYSTICERCOSIS –A STUDY FROM WESTERN NEPAL Sriparna Basu, Uma Ramchandran, Anna ThapliyalThe Department of Pediatrics, Manipal College of Medical Sciences & Manipal Teaching Hospital, Phulbari, Pokhara, Nepal.

Introduction: Neurocysticercosis (NCC) is the commonest parasitic disease of central nervous system and a common pediatric neurological problem in Western Nepal. Objective: To define the magnitude of the disease in pediatric population and to determine the epidemiology, clinical features, investigations, response to treatment and follow up of the disease Materials and methods: Medical records of 124 children with NCC were evaluated. Diagnosis was primarily based on clinical features, CT scan features, electroimmunotransfer blot (EITB) assay and exclusion of other causes. All patients were treated with 28 days’ course of albendazole, anti-edema drugs and anticonvulsants. Follow up period varied from 1-3 years. Data were analyzed by mean and standard deviation Results: The commonest age group was 10-12 years, youngest being of 11 months. Commonest clinical presentation was partial seizures. The most frequent finding in CT scan was single ring enhancing lesion with perilesional edema. EITB was positive in 80.72% of serum and in 96.88% of CSF samples. Ninety-eight patients had complete clinical recovery and 87 patients had complete disappearance of lesion in CT scan at the end of one year. Recurrence of seizures was the only residual symptom found in 6 (4.83%) patients, all of them having calcified lesions. Conclusion: In endemic areas of NCC partial seizures in the pediatric age group is an indicator of the disease. A good diagnostic marker is EITB while CT scan remains the best investigation for confirmation. Recurrent partial seizure is the only residual symptom which may occur more commonly in patients with calcified lesions.

NEU/03(O) CLINICAL EPIDEMIOLOGY OF PERVASIVE DEVELOPMENTAL DISORDERSSharmila Banerjee Mukherjee, Monica JunejaMaulana Azad Medical College, Department of Pediatrics, Bahadur Shah Zafar Marg, New Delhi – 110002

Objectives: To study the clinical and neurodevelopmental profile of Pervasive Development Disorders in Indian Children. Design: Descriptive study. Setting: Child development and early intervention clinic of a tertiary hospital. Subjects: Children with developmental delay, speech delay, behavioural and/ or poor school performance were screened for Pervasive Development Disorders. Methods: All patients were reviewed with a detailed history including social and developmental history and complete examination was done. The quality of child’s play and social interaction was directly observed. The diagnosis was established using the criteria of Diagnostic and Statistical Manual of Mental Disorders, Fourth edition. Childhood autism rating scale and Autism behavior checklist were also used. Development/ Intelligence quotient and social quotient were assessed. Results: The number of children diagnosed as having Pervasive Development Disorders was 124. Autistic disorder was diagnosed in 117, Asperger’s disorder in 3, Rett’s disorder in 2 , Childhood disintegrative disorder and Pervasive Development Disorder Not Otherwise Specified in one patient each. The diagnosis had not been suspected in most children at referral. The mean age at diagnosis was 57.03 ± 35.56 months. The male female ratio was 2.02: 1. Global developmental delay/ Mental retardation was seen in 116 children. Conclusion: Pervasive Development Disorders are not uncommon in Indian children. For early diagnosis, developmental surveillance should be elaborate. The medical community must increase their awareness and ability to recognize Pervasive Development Disorders for appropriate and early intervention.

NEU/04(P) CLINICAL PROFILE OF PATIENTS WITH ACUTE FLACCID PARALYSIS IN A TERTIARY CARE TEACHING HOSPITALKalra Veena, Sharma Anita, Kamate Mahesh, Banerjee Bidisha, Chaudhry Rama, Aneja Satinder, Dua TarunDepartment of Pediatrics, Division of Neurology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029

Aims and Objectives: To study clinical profile of patients presenting with acute flaccid paralysis. Materials and methods:All patients less than 15 years fulfilling WHO definition of acute flaccid paralysis presenting between August 2003 to August 2005 included. History of complaint and preceding illnesses taken. Detailed examination & disability score at admission recorded. CSF examination and nerve conduction done in Guillain-Barre syndrome(GBS) patients Results:Total seventy patients enrolled, age ranging from 15 months to 13 years with 47 boys and 23 girls. Final diagnosis included 43 GBS, 6 transverse myelitis, 6 traumatic neuritis, 3 hypokalemic paralysis, others-12. No cases of poliomyelitis identified. In GBS duration of symptoms at presentaion varied from 1-30 days with median of 5 days. Symptoms < 7 days in 14, 7-14 days in 14, 14-28 days in 15. All GBS patients had motor weakness, 19 had paraparesis, 24 had quadriparesis, symmetrical 38 asymmetrical 5. Cranial nerve dysfunction in 8 and 2 required ventilatory support. Preceding illness in 14(33%), respiratory tract infection 9, diarrhoeal illness 6 and injections in 3 patients. Functional disability grade at admission ranged from 1-9 with median of 7 (tetraparesis). CSF analysis done in 31 patients. All had <10 cells/ul, normal sugar, proteins ranged from 13-400 mg% being normal in 10. Nerve conduction study done in 34 cases,19 had axonal GBS, 10 patients no response, 5 had demyelinating type. Those with axonal type showed improvement in CMAP on follow up. Out of 15 mild GBS (ambulatory) 9 were axonal. Conclusions:GBS is important cause of AFP with the ongoing active polio eradication programme. Unlike reports from western world we have seen greater proprtion of axonal variants with relatively good prognosis. Presence of traumatic neuritis cases stress need for injection safety in pediatric practice.

NEU/05(P) CEREBRAL PALSY IN CHILDRENPuneet Jairath, Kamaljeet Kaur, Shalini Soi, K.K.LochamDeptt. of Pediatrics, Govt. Medical College/Rajindra Hospital, Patiala – 147001

Objectives : Evaluation of clinical profile of cerebral palsy. Methods : The study included 7 children with cerebral palsy admitted to department of Pediatrics, Govt. Medical College, Patiala. Age, sex, residence, presenting complaints, type of seizures and prenatal, natal and postnatal risk factors were recorded in a predesigned proforma. Cerebral palsy was classified on the basis of topographic and physiological classification. Presence or absence of mental retardation was also recorded. Results : 7 children with cerebral palsy were admitted. 1(14.28%) child was in the age group of less than 1 year and 6 (85.71%) children were in age group of 1-3 years. 1 (14.28%) child was female and 6 (85.71%) were males. 1(14.28%) child was of urban and 6 (85.71%) children were of rural background. 6(85.71%) children presented with seizures while 5(71.42%) were having fever. 1(14.28%) child each presented with feeding problems and cough with fever. 5 (71.42%) children had generalized tonic clonic seizures and 1 (14.28%) was having focal seizures. Birth asphyxia in 3(42.85%) cases and meningitis in 2(28.57%) cases were the predisposing factors while birth asphyxia and meningitis both were the underlying causes of cerebral palsy in 2(28.57%) cases. 3 (33.33%) cases had hemiplegia and 4 (44.44%) had quadriplegia. 5 (71.42%) children had spastic type while the 2 (28.57%) had mixed type of cerebral palsy. All children were mentally retarded. Conclusions : Spastic type was the most common and birth asphyxia was the predominant cause of cerebral palsy. Mental retardation was associated in all the cases of cerebral palsy.

NEU/06(P) CYSTICERCOUS ENCEPHALITISMegha Consul, Sugandha AryaDepartment of Paediatrics,Safdarjung Hospital and VMMC,New Delhi

A 6-year-old girl presented to us with complaints of headache, fever, vomiting and dimness of vision since 3 months and deviation of eyes since a fortnight. Examination revealed papilledema and 6th nerve palsy. No abnormality was found on any serological investigation including CSF. Contrast enhanced CT scan revealed no abnormality except for generalized edema. On MRI, however, multiple neurocysticerci(starry sky appearance) were revealed and subsequent serum analysis was positive for NCC. Cysticercotic encephalitis is a rare disorder known to present in young children with no other abnormality except features of raised intracranial hypertension and starry sky appearance on MRI. Treatment involves steroids ,cysticercidal drugs and anti-edema measures .

NEU/07(P) CHILDHOOD ISCHEMIC STROKE; CLINICAL AND RADIOLOGICAL CORRELATION D. L. Jain, A.S. Pilkhane. Department of Pediatric, Government Medical College, Nagpur.

Childhood stroke is an important cause of mortality and chronic morbidity, but the studies in Asian countries are lacking, including India. Yet very little is known about the cause, treatment and prevention. OBJECTIVES: To study clinical presentation and probable etiology of ischemic stroke in children. To find out correlation between clinical presentation and radiological findings. To study short term outcome including survival and neurological sequalae. METHODOLGY: Design: It is a cross sectional study with follow up duration of eight weeks. Setting: Department of Pediatrics, Government Medical College and Hospital, Nagpur, a Tertiary Care Hospital in Central India. Sample: Fifty children in age group between one month to twelve years having radiologically documented ischemic stroke were included. Data Collection: Complete hemogram including Hb, cell count, hematocrit and screening test for sickle cell disease was done in all cases; while CSF study, liver function test was done in selected patients. All of them were subjected to contrast CT scan study. CT angiography was performed in 28 out of 50 cases and carotid Doppler in twenty two children. RESULTS: More than half of cases etiology remains unknown but anemia and hypercholestrolemia (in few cases). May be the risk factor for ischemic stroke. On CT scan cortical infarcts were more common forty eight percent and Middle Cerebral Artery affected in majority eighty four percent of them. Overall outcome pertaining to mortality, neurological sequalae or recovery was not significantly affected by cortical or sub-cortical infarction. The risk factors reported in immediate mortality were alteration in sensorium at the time of presentation, extent of area involved by ischemic infarct, hemorrhagic transformation and underlying etiology.

NEU/08(O) CONGENITAL MYOPATHIES: A CLINICOPATHOLOGICAL STUDY OF 16 CASESSheffali Gulati, M.C. Sharma, S Epari,Chitra Sarkar, Veena Kalra,Associate Professor, Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi

Congenital myopathies are a group of neuromuscular disorders, mostly of childhood onset, often of slow progression, but exceptionally having more rapid course. They show disease specific structural changes in the muscle which are detected by enzyme histochemistry, and electron microscopy but sometimes with immunohistochemistry. We report a series of 16 cases of congenital myopathy, which to the best of our knowledge is first largest series from India. Material & Methods: Conducted at Myopathy Clinic, Department of Pediatrics, All India Institute of Medical Sciences. All cases between January 2001 to December 2004 diagnosed as congenital myopathies were studied. Clinical data and muscle biopsies were reviewed. Results: During this period 16 patients were diagnosed as congenital myopathies: Central core disease 6; Multi mini core disease 4; Centronuclear myopathy 2; Nemaline myopathy 1; Congenital fibre type disproportion 3. Age ranged from newborn to 12 years. All presented in the first decade of life. The most common findings were difficulty in running and getting up followed by generalized hypotonia in 5 (31.2%), facial dysmorphism in 4 (25%), high arched palate in 2 (12.5%) and ptosis in one (6.2%). Family history of similar illness was present in two cases. Creatine phosphokinase levels were either normal or mildly elevated. Electromyography was done in 12 patients, of which 8 showed myopathic changes, 3 were normal and 1 was suggestive of neurogenic atrophy. Conclusions: We are able to categorize the various congenital myopathies with available investigations and one should have a high index of suspicion for them.

NEU/09(P) CEREBRAL VENOUS THROMBOSISPrachi Gupta ,Sujata,M.S.PrasadDepartment of Pediatrics, Safdarjung Hospital, New Delhi

Cerebral venous thrombosis has a wide range of age group distribution, but in children it is a rare but significantly serious complication of a very common illness : acute gastroenteritis with dehydration. We report a case of a 6 months old female child who presented with the above complication. A 6 months of female child developmentally normal for age presented to the hospital with the complaints of fever,loose stools and vomiting for 3 days and abnormal jerky movements of the left side of the body assosciated with uprolling of eyeballs for one day. There was no history of ear disease, rash, contact with Koch’s,head trauma. On examination child was drowsy and signs of severe dehydration were present along with left hemiparesis with no cranial nerve deficit and no signs of raised intracranial tension. The dehydration of the patient was corrected.Blood investigations including coagulation profile and FDP were normal except for microcytic hypochromic anemia, thus ruling out metabolic cause of seizures.Cerebrspinal fluid analysis was normal and cultures were sterile. CECT head clinched the diagnosis : (R) parietal cortical venous infarct along with superior sagittal thrombosis. Further investigations like protein C,S etc to rule out hypercoagulable state were within normal limits. With appropriate fluid and drug therapy and physiotherapy, the child recovered completely in 6 days and was discharged with no residual defects.

NEU/10(P) CLINICAL PROFILE OF SPEECH AND LANGUAGE DISORDERS PRESENTING AT A TERTIARY LEVEL HOSPITAL IN INDIA.Kashyap N, Khanna H, Sharma PChild Development Clinic, Apollo Centre of Advanced Pediatrics, Apollo Indraprashtha Hospital, Sarita Vihar, New Delhi 110044

Disorders of communication are the most common developmental problem in pre-school aged children and 7 to 10 % of children are considered to be functioning below the norm in some aspect of speech or language ability. Evaluation of speech and language delay is a challenge. Appropriate intervention is often delayed due to improper identification and lack of systematic approach to its recognition. This paper presents the clinical profile of speech and language disorders noted in a retrospective analysis of 715 cases seen at a tertiary level hospital in India. Material & Methods: A total of 715 cases were seen during Jan 2002 to Aug 2005 out of which 90 cases presented with speech and language delay as a presenting complaint. A developmental pediatrician undertook detailed clinical and developmental examination and speech and language assessment was done in a multidisciplinary setting involving ENT specialist and speech and language pathologist. The speech and language disorders were classified based on DSMIV criteria. Hearing was tested by BERA or audiometry. Results: 74 of 90 (82%) were male and 16 (18%) were females. The subjects were in the age range of 5months to 12 years. Age distribution indicated that majority of the cases (72%) were less than 5 years of age, with 30% of the total presented before three years of age. The diagnostic workup revealed that 47.8% were Autistic spectrum disorders, 13.3% had mental retardation, 11% had ADHD, 10 % had developmental language disorder, 6.7% had hearing loss, 3.3% had articulation disorder and 3.3% had associated neurological conditions. 6.7% could not be classified and were labeled as specific language impairment.Conclusions: Presentation of speech and language disorders can be diverse. Autistic spectrum disorders were the largest category recognized in our series probably due to the study being conducted at a tertiary level referral centre. A detailed clinical and developmental examination with appropriate clinical tools in a multidisciplinary setting is required to workup these conditions. 

NEU/11(P) GUILLAIN-BARRE SYNDROME WITH HERPES SIMPLEX VIRUS INFECTIONRajiv Kumar, Nomeeta Gupta, Rajesh Sardana, Bindu Shrivastava, Gurdeep Atwal, Sandeep PatelDepartment of Pediatrics, Batra Hospital & Medical Research Centre, New Delhi-110062.

Guillain-Barre Syndrome (GBS) is an acute flaccid lower motor neuron paralysis. Usually GBS occurs a few days or weeks after the patient had respiratory or gastrointestinal viral infections. GBS caused by Herpes simplex virus (HSV) infection is very uncommon in children. We report uncommon case of GBS with HSV infection in a 4 year old child. Case Report: A 4 year old male child admitted in our institution with history of fever, headache, vomiting and uprolling of eyeballs for 3 days; altered sensorium and unable to walk for 2 days. On examination, he was afebrile, sick, and comatose with stable vitals and blood pressure 130/90 mm Hg. No abnormality was detected on examination of cardiovascular, respiratory and gastrointestinal systems. Central nervous system revealed power grade 1-2/5 and hypotonia in all four limbs. Deep tendon reflexes and planters were not elicitable. There was minimal response to painful stimuli and to verbal commands. There was no focal neurological deficit. The sensorium deteriorated gradually and power became 0/5 in all four limbs. He eventually developed autonomic dysfunction and respiratory failure for which he was put on mechanical ventilation. The complete blood counts, electrolytes, urinalysis, blood sugar, serum CPK, and blood culture were normal. CSF examination showed 46 lymphocytes with protein 68 mg%, glucose 60 mg% and positive HSV IgM. Poliomyelitis was ruled out by serological test and stool culture. CT head scan revealed picture suggestive of Herpes simplex encephalitis. EEG was normal. Nerve conduction test revealed severe peripheral neuropathy. He was treated with intravenous broad-spectrum antibiotics, high dose intravenous immunoglobulin, intravenous acyclovir, intravenous mannitol and antihypertensive drugs. He regained consciousness gradually and started responding to verbal commands. Tone and power improved gradually in al four limbs. Nasogastric feeds were started. Physiotherapy was done and continued even after discharge. At the time of discharge, he could sit independently and was accepting orally but was unable to walk. After 3 weeks on follow-up, the child was walking and running.

NEU/12(O) HOME-BASED THERAPY IN CHILDREN WITH AUTISTIC DISORDERMonica Juneja, Sharmila Banerjee MukherjeeChild Development and Early Intervention Clinic, Department of Pediatrics, Maulana Azad Medical College, N. Delhi - 110002. Objectives: To evaluate the effectiveness of individualized home-based therapy in children with Autistic disorder. Design: Retrospective. Setting: Child development and early intervention clinic of a tertiary hospital. Subjects: Children with Autistic disorder (AD) who were exclusively attending the clinic for Behavioral and speech therapy for at least six months. Methods: Parents are trained in a one to one session and given written and pictorial instructions regarding therapy, which they implement at home. During follow up the counseling is reinforced and modified according to the goals achieved and compliance for therapy is assessed. For the purpose of this study, files of children fulfilling the inclusion criteria were reviewed. Pre and Post treatment development quotient, social quotient, expressive and receptive language quotients, Childhood autism rating scale and Autism behavior checklist scores were recorded. Statistical analysis was performed using SPSS software version 11. Results: Sixteen children were enrolled. The male: female ratio was 13: 3 and the mean age was 39.06 ± 22.05 months. According to the Kuppuswamy scale, 2 children belonged to class I, 9 to class II, 2 to class III and 3 to Class IV. Fifteen children had severe AD. Mental retardation was present in 81.25 %. The average duration of therapy was 19.5 ± 11.78 months. Compliance to therapy by the care givers was less than 25% in 5, 25 - 50 % in 3, 50 to 75% in 3 and 75 - 100% in 5 children. Post therapy it was seen that there was a statistically significant difference in the development quotient (p = 0.015), social quotient (p = 0.004), expressive language quotient (p =0.03), Childhood autism rating scale score (p = 0.001), and Autism behavior checklist score (p = 0.014). Severity of symptoms was also reduced. Conclusion: Home based behavioral therapy is effective, even in children with severe AD and those belonging to low socioeconomic classes.

NEU/13(O) LACUNAE IN THE DIAGNOSIS AND APPROPRIATE TREATMENT OF WEST SYNDROME AND ITS CORRELATION WITH LONG TERM FOLLOW UP.Anaita Hedge, Varsha Vaidya, Shilpa Kulkarni, V.Lakshmi Kumari.Bai Jerbai Wadia Hospital for Children, Achararya Donde Road, Parel Mumbai 400 012

Aims and objectives: To highlight the lacunae in diagnosis and appropriate treatment of West Syndrome. To see if early and appropriate treatment changes the long- term prognosis. Materials and methods: 1000 cases of unequivocal childhood epilepsy from a tertiary pediatric epilepsy clinic were reviewed. Detailed history regarding birth, family, and type of seizures, EEG, MRI, neurodevelopmental stAims and Objectives: To study clinical, electrophysiological and neuroradiological aspects of patients with neonatal hypoglycemic insult. Materials and methods: 2000 epilepsy patients from 1998–2004 visiting our epilepsy center were studied. Those with documented neonatal hypoglycemia on NICU discharge cards or neuroimaging suggestive of perinatal hypoglycemic insult(parieto-occipital damage) were analysed. Detailed clinical history, perinatal risks, age of onset, type of epilepsy and thorough neurological examination were obtained. Patients were subjected to electroencephalography (EEG), neuroimaging. Follow-UP period was 0-84 months (average 19.93). Results: 70/2000(3.5%)patients had neonatal hypoglycemic brain damage.59/70 (84.28%) showed NICU records of documented hypoglycemia. 11 patients perinatal hypoglycemia was suspected on neuroimaging during epilepsy workup. 19/59 (32.75%) had neonatal hypoglycemic seizures. Perinatal risk factors were intrauterine growth retardation in 31/70 (44.28%)& sepsis 26/70(37.14%). 7/70 (10%) had isolated hypoglycemia. All patients were seizure free on monotherapy at discharge from NICU. Age of onset of repeat seizures was 19.13 months (2-108 ) Partial seizures were 30/70(42.85%), infantile spasms 24/70(34.28%) and myoclonic in 7/70(10%). Development was delayed in 54/70(77.14%). EEG revealed focal epileptiform abnormalities in 27/70 ( 38.57%) , multifocal in 19/70 (27.14%),hypsarrythmia in 18/70 (25.71%). 6/70(8.57%) had normal EEG. 53/70 underwent neuroimaging studies. 20/70 (28.57%) showed isolated parieto-occipital damage suggesting neonatal hypoglycemic insult, 18/70 ( 25.71%) had combinations of parieto -occipital damage and different perinatal insults On follow up 28/70 (40%) were seizure free.19/70 (27.14%) had poor seizure control. Conclusions: Hypoglycemia is common, easily treatable condition in neonates. It is associated with significant neurological damage and epilepsy. Complex partial seizures and West syndrome were the commonest outcomes with poor prognosis in later.

NEU/14(O) EARLY PREDICTORS OF INTERACTABLE EPILEPSYVikas D. Bansal, B. B. JavadekarDepartment of Paediatrics, M.P.Shah Medical College, Jamnagar.

Epilepsy is affecting approximately 50 million people worldwide. Prognosis for majority of patients is good but upto 30% suffer from intractable epilepsy. Because interactability of epilepsy is said to develop early in the course of the disease and early aggressive management can prevent intractability, it is essential to identify children destined to develop intractable epilepsy early in their course. AIMS : To identify early predictors of intractable epilepsy. MATERIAL AND METHODS : A case control study of 67 patients following the epilepsy clinics, Department of Paediatrics G.G.H. Jamnagar comparing 32 cases and 35 controls. Cases were patients who averaged atleast 1 unprovoked seizure per month during an observational period of atleast 2 years. Controls were subjects having achieved remission for at least 1 year on current therapy. Both cases and controls were analysed for the factors which have been reported to have unfavourable prognosis like age of onset of seizures, type of seizures, number of seizures before starting treatment, episodes of status epilepticus, predisposing factor for epilepsy, types of epilepsy (idiopathic / probable symptomatic and symptomatic), developmental delay, abnormal neurological status, EEG findings and site of epileptic focus on EEG and CT scan. Strong assocaition were noted between intractability and general factors with early onset of seizures, focal type of seizures, more than 10 episodes of seizures before starting treatment, history of status epilepticus particularly more than 2 episodes, symptomatic epilepsy, presence of developmental delay, presence of abnormal neurology and microcephaly. Cases were significantly younger than controls at onset (0.87 Vs. 3.74 years) this was not solely to cases with onset during the first year of life but was an association apparent throughout the age range studied with P value < 0.01, focal seizures P < 0.01, multiple seizures episodes (> 10 episodes) P < 0.01, more than 2 episodes of status epilepticus, P < 0.05, symptomatic epilepsy P < 0.01, developmental delay P <0.01, abnormal neurology P<0.01 and microcephaly P< 0.001. CONCLUSION : Our study suggested that the risk of developing intractable epilepsy may be predicted at the time of initial diagnosis in children with early age of onset of symptomatic epilepsy with high seizure propensity with frequent episodes of status epilepticus, developmental delay with abnormal neurology especially microcephaly.

NEU/15(P) EPILEPSY FOLLOWING NEONATAL HYPOGLYCEMIAVarsha Vaidya, Shilpa Kulkarni, V.Lakshmi Kumari, Anaita Hedge.Bai Jerbai Wadia Hospital for Children, Achararya Donde Road, Parel Mumbai 400 012

Aims and Objectives: To study clinical, electrophysiological and neuroradiological aspects of patients with neonatal hypoglycemic insult. Materials and methods: 2000 epilepsy patients from 1998–2004 visiting our epilepsy center were studied. Those with documented neonatal hypoglycemia on NICU discharge cards or neuroimaging suggestive of perinatal hypoglycemic insult(parieto-occipital damage) were analysed. Detailed clinical history, perinatal risks, age of onset, type of epilepsy and thorough neurological examination were obtained. Patients were subjected to electroencephalography (EEG), neuroimaging. Follow-UP period was 0-84 months (average 19.93). Results: 70/2000(3.5%)patients had neonatal hypoglycemic brain damage.59/70 (84.28%) showed NICU records of documented hypoglycemia. 11 patients perinatal hypoglycemia was suspected on neuroimaging during epilepsy workup. 19/59 (32.75%) had neonatal hypoglycemic seizures. Perinatal risk factors were intrauterine growth retardation in 31/70 (44.28%)& sepsis 26/70(37.14%). 7/70 (10%) had isolated hypoglycemia. All patients were seizure free on monotherapy at discharge from NICU. Age of onset of repeat seizures was 19.13 months (2-108 ) Partial seizures were 30/70(42.85%), infantile spasms 24/70(34.28%) and myoclonic in 7/70(10%). Development was delayed in 54/70(77.14%). EEG revealed focal epileptiform abnormalities in 27/70 ( 38.57%) , multifocal in 19/70 (27.14%),hypsarrythmia in 18/70 (25.71%). 6/70(8.57%) had normal EEG. 53/70 underwent neuroimaging studies. 20/70 (28.57%) showed isolated parieto-occipital damage suggesting neonatal hypoglycemic insult, 18/70 ( 25.71%) had combinations of parieto -occipital damage and different perinatal insults On follow up 28/70 (40%) were seizure free.19/70 (27.14%) had poor seizure control. Conclusions: Hypoglycemia is common, easily treatable condition in neonates. It is associated with significant neurological damage and epilepsy. Complex partial seizures and West syndrome were the commonest outcomes with poor prognosis in later.

NEU/16(P) EFFICACY AND SAFETY PROFILE OF CLOBAZAM IN INDIAN CHILDRENRachna Seth, Veena Kalra , Devender Mishra, Anita Sharma, N C Saha,Department of Pediatrics, All India Institute of Medical sciences, New Delhi, India

Background. Clobazam, is a newer 1, 5-benzodiazepine used for the treatment of epilepsy. Most studies on adults and few on children have shown clobazam to be a useful oral adjunctive therapy in refractory epilepsy. It is reported to be better tolerated and less sedating than other benzodiazepines. Asian data on use of clobazam is limited being particularly scarce in children. Our aim was to evaluate the efficacy of clobazam in childhood refractory epilepsy and to characterize the adverse drug reaction profile in the Indian children. Methods: A cohort of 88 children (9 months to 12 years) with ‘refractory’ epilepsy was started on clobazam as add-on therapy. Diagnosis was established and seizure type recorded. Patients were followed at the Pediatric Neurology Clinic at All India Institute of Medical Sciences. Therapeutic response was recorded as ‘complete’, ‘good’, ‘fair’ and ‘no response’. Observed side effects were classified as ‘mild’, ‘moderate’ and ‘severe’. Results: Most children were on at least two antiepileptics. Seizures most identified were either partial (36.3%) or generalized tonic clonic (15.9%). Clobazam was effective against all seizure types. The dose ranged from 0.3-2 mg/kg/day (average 1 + 0.2 mg/kg/day). Complete seizure control was seen in 62.2% patients. Tolerance was seen in 5 (5.6%) patients. Side effects were seen in 23 (26%) patients and were ‘mild’ in 20/23 patients. Clobazam was stopped in three patients who developed ataxia, which resolved on stopping the drug. Conclusions: Clobazam is well tolerated and has a broad spectrum of antiepileptic activity. It is a safe add on antiepileptic with mild adverse drug reactions . The drug was well tolerated . Single daily dose and low cost of the drug were responsible for good compliance. Clobazam is recommended as a safe add on drug with low rate of undesired side effects in children with refractory epilepsy.

NEU/17(P) DEVIC’S DISEASE - CASE REPORTKaruna Thapar, Abha Midha, Sandeep Aggarwal, Gaurav DhawanDepartment of Pediatrics, Govt. Medical College, Amritsar-143001

Introduction: Neuromyelitis optica (devic’s disease) is an uncommon clinical syndrome comprising of unilateral/ bilateral optic neuritis and transverse myelitis within 8 weeks. Case Report: An 8 year female presented with cough, abdominal pain, backache and dysuria for 5-6 days. Pain in legs, inability to stand and walk, urinary retention and dribbling of urine for 3 days. History of fever of short duration 1 month back. Development of child was normal and child was immunized. On examination, vitals were stable, GPE was normal. Neurological findings included grade 1 power and hypotonia of lower limbs. All reflexes of lower limbs were absent. Sensory loss was present up to the level of umbilicus. There was no sensory and motor loss in upper limbs, cranial nerves were normal, so child was then diagnosed as a case of transverse myelitis. Child had a sudden loss of vision on 7 th day. This led to the possibility of devic’s disease. Fundus examination was normal. Routine investigations were normal. CSF examination showed 4 cells/cmm, predominant lymphocytes, proteins 34 mg%, globulin negative, sugar 45.5 mg%, and chloride 125.2 mg% with no microorganism. MRI spine showed myelitis at dorsal cord D8- D12 level, child was put on intravenous dexamethasone within 4 hours of loss of vision. Vision showed improvement in 24 hours and full vision was restored within 72 hours. Sensory loss of lower limbs progressively decreased and child was able to stand. On 15 th day child was able to walk without support. There was no residual neurological deficit.

NEU/18(P) BECKER MUSCULAR DYSTROPHY: PREVALENCE AND PATTERNS OF CARDIAC INVOLVEMENTSheffali Gulati, Anita Saxena, Vani Wazir, M.C. Sharma, Chitra Sarkar, Madhulika Kabra, Monica Tiwari, Veena Kalra,Associate Professor, Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi

Duchenne muscular dystrophy (DMD) is a severe X-linked genetic disease affecting one in 3500 boys. It is due to a lack of dystrophin, a submembrane protein of the cytoskeleton, which leads to the progressive degeneration of skeletal, cardiac, and smooth muscle tissue. A milder form of the disease, Becker muscular dystrophy (BMD), is characterized by the presence of a semifunctional truncated dystrophin, or reduced levels of full-length dystrophin. Cardiac disease is not as common or as severe as in DMD Objective: To assess cardiac involvement in BMD patients by clinical, radiographic, electrocardiographic and echocardiographic monitoring. Materials and methods: Thirty four patients diagnosed to have BMD amongst those attending the Myopathy Clinic, Department of Pediatrics, All India Institute of Medical Sciences were enrolled. A detailed clinical evaluation, CPK levels and gene deletion studies were carried out. Cardiac investigations included Chest X ray, 12 lead ECG and echocardiography. Results: Age at onset was between 4 to 18 years (mean 11.4 3.8 years). Clinical features included: calf hypertrophy in 73.5%, developmental delay in 14.7%, myopathic facies in 17.6%, gower’s sign in over 90% and a positive family history in 38.2 %. The CPK levels had a mean of 8009 IU/L 10482.9. The cardio-thoracic ratios were between 0.40 to 0.52 (mean 0.46 0.03). Ejection fraction was between 41 to 64 (mean 54.6 6.1). ECG showed a V1R> 4mm, q waves in lateral and inferior leads in 47.1%, 2.9% and 2.9% respectively. Gene deletion studies will be discussed. Conclusions: Cardiac dysfunction is less common in BMD than in DMD but early recognition is necessary to institute appropriate treatment thus reducing morbidity.

NEU/19(P) BRAIN STEM AUDITORY EVOKED RESPONSE AUDIOMETRY (BERA) IN HYPERBILIRUBINEMIC NEONATES Pramod Sharma, N.P. Chhangani,Keshram Meena,B.D. Gupta,Rakesh Jora ,Ravi BhatiaUmaid hospital,Dr SN Medical College, Jodhpur

Neonatal hyperbilirubinema places the affected neonate at increased risk for hearing impairment. Brain Stem Auditory Evoked Responses (BAER) is an effective, objective method of assessing auditory functions and abnormalities correlate with serum bilirubin levels. Further therapeutic interventions have been shown to improve BAER record. OBJECT: -To record BAER in icteric term neonates & derive values for various latency period & interwave distances. Methodology : A prospective case controlled study in which 30 icteric term neonates delivered at Umaid Hospital Jodhpur without any obstetrical complications were enrolled.Babies with abnormal events like severe birth asphyxia, pyogenic meningitis, septicemia, congenital craniofacial malformations or babies on mechanical ventilator were excluded. BERA recordings were made as per the technique described by Taylor et al.The records were obtained at the time of peak hyperbilirubinema, after therapy & on follow up at the age of 2-4 months. Results: Mean birth weight, mean gestational age & mean age at enrolment were 3.31+0.41 kg; 38.38+1.26 weeks and 4.16+0.77 days respectively. Mean serum bilirubin levels were 25.91+7.28mg% with 5 babies having Rh incompatibility. Mean latency of all waves was prolonged at all decibel levels before treatment & after phototherapy and /or exchange transfusion the latency showed a statistically significant decrease. Similarly interwave period was prolonged in all segments, which too showed significant reversion after therapy. Changes in BAER were significant at serum bilirubin above 25mg%. Abnormal BAER responses were seen in 73.3% babies with 20% patients showing absence of wave V at 30 db in both ears though it persisted in only 7 cases (23.3%) after treatment. On follow up tracings 16% continued to show abnormality at 3 months of age. Conclusion: Hyperbilirubinema is significant high risk factor causing hearing impairment. BAER can detect bilirubin encephalopathy relatively early and correlates well with serum bilirubin levels.

NEU/20(O) ANOMALIES OF CRANIAL VAULT IN PEDIATRIC AGE GROUPNiranjan K. Singh, Sushma Malik, Sushma Save, Surekha Joshi.Pediatric Neurology Clinic, Department of Pediatrics, T.N. Medical College & B.Y.L. Nair Hospital, Mumbai.

INTRODUCTION: Abnormal shape and size of the skull are frequently observed in early childhood. These are a major concern to both parents and pediatricians. AIMS & OBJECTIVES: To estimate the prevalence, spectrum & etiology of cranial vault anomaly (CVA). To study the correlation of clinico-demographic factors to morbidity. MATERIAL & METHODS: Detailed history, thorough physical and neurological examination including anthropometry was taken in all cases. Relevant investigations were done to ascertain the underlying etiology. All data were analysed statistically using Chi Square Test and Z-test with SPS software package. RESULTS: Of the 137 subjects enrolled, CVA was encountered most commonly in 0-2 years age group with slight female preponderance (Male: Female – 0.87: 1). Based on skull size anomalies microcephaly was seen in nearly 81% and macrocephaly in 19%. Microcephaly was either primary (53%) or secondary in nature (47%). The spectrum of shape anomalies ranged from plagiocephaly to brachycephaly, scaphocephaly and acrocephaly. Amongst these positional plagiocephaly was the predominant form noted. The etiology of various CVA was either – idiopathic (44.47%) or symptomatic. Clinical presentation in 20% of our cases was primarily related to the shape / size of the skull, whereas in 80% the symptomatology was related to their underlying illness. In 8% of our subjects, recognisable syndromes like Down, Crouzon, Aicardi, etc. were detected. CONCLUSION: Early detection, management and intervention of children presenting with CVA will ensure optimum brain growth and decrease the morbidity significantly. In cases of craiosynostosis, careful search for syndromic association and early neurosurgical intervention would prove very beneficial.

NEU/21(P) ACUTE DISSEMINATED ENCEPHALOMYELITIS (ADEM)- CASE REPORTKaruna Thapar, Abha Midha, Gaurav DhawanDepartment of Pediatrics, Govt. Medical College, Amritsar-143001

Introduction: ADEM is immunologically mediated inflammatory demyelinating disease of CNS. Highest prevalence is seen in prepubertal boys. Clinical manifestations include abrupt development of irritability and neurological signs in children recovering from a viral prodrome. Changes in long tract signs and mental status are commonly observed. Case Report: A 7 years old male child presented with intermittent fever for 25 days, headache for 15 days, irritability, excessive crying, vomiting, and pain abdomen. On examination vitals were normal, neck rigidity was present, pupils were bilaterally reacting, tone was normal and reflexes were present. CSF studies were normal. MRI revealed few small hyper intense lesions in the sub cortical white matter of cerebral hemispheres, left caudate nucleus and rostrum of corpus callosum on right side suggestive of acute disseminated encephalomyelitis. He was treated with intravenous methyl prednisolone with which he showed slight improvement and was discharged on oral steroids. The child was readmitted within three months again with complaints of decreased vision for 15 days, headache for 10 days, difficulty in micturation for 4 days and irrelevant talks for 1day. MRI brain revealed that the previous lesions had increased in number and size with involvement of the basal ganglia and the brainstem. The child was given intravenous methyl prednisolone (30mg/kg/day) for three days and then started on oral methyl prednisolone. The child showed dramatic improvement to this therapy and led to resolution of all his neurological deficits. Follow up for 5 months revealed no neurological deterioration.

NEU/22(P) ACUTE ENCEPHALITIC SYNDROME IN CENTRAL INDIA – AN OVERVIEWMahendra Jain, Navaneetha S., Piyush Chandel., Ankur Vikas Bopche, Jyotsna Shrivastava, G.S. Patel, Rashmi Dwivedi.Department of Paediatrics, Kamla Nehru Hospital, Gandhi Medical College, Bhopal.

Encephalitis is a common cause of morbidity and mortality. Objectives: To study the epidemiology, etiology and magnitude of acute encephalitis. Setting and Methods: A retrospective study was conducted including the period from Aug. 2003 to Aug. 2005. All children admitted with the acute encephalitic syndrome defined as acute onset of fever with seizures and altered consciousness excluding meningitis and cerebral malaria were included in the study. Data regarding history, clinical findings, investigations and outcome were collected and analysed as to the etiology and cause of death. Results: A total of 174 children were admitted (2% of in patients) 68 (39%) of them expired (5% of total mortality). An etiological diagnosis could be made in only 11 (6.32%) cases (no serological support) 5 (2.87%) were rabies (100% mortality); 3 (1.72%) were measles (0% mortality), 2 (1.15%) were varicella (0% mortality) and 2 (1.15%) were mumps encephalitis (50% mortality) of the 163 cases with unknown etiology; the majority 54 (33.12%) had a short history <24 hours; 92 (56.44%), 24-72 hours and 17 (10.43%) >72 hours. Associated gastrointestinal, respiratory symptoms were present in 42 (25.77%) and 32 (19.63%) cases, rash in 7 (4.29%); conjunctivitis in 23 (14.11%) focal seizures in 8 (4.91%); pyramidal tract signs in 123 (75.46%) extra pyramidal signs in 2 (1.23%). 12 (17.64%) of the 68 expires presented with status epilepticus; 17 (25%) with fluid and inotrope resistant shock and 19 (27.94%) with signs of raised ICT. CSF pleocytosis could be documented in 51 (31.29%) of the cases including post mortem LP. Maximum no. of cases and expiries were recorded during the months of May to August. Conclusion: Encephalitis is a major cause of morbidity and mortality, but the problem is under addressed. Facilities for serodiagnosis and intensive care should be made available atleast at the tertiary care level.

NEU/23(O) AN OUTBREAK INVESTIGATION OF ENCEPHALITIS IN GORAKHPUR, UP: 2004Neeru Gupta , Kunal Chatterjee , Shah Hossain, Bina Pani Das, S.Venkatesh and Shiv LalAssistant Director General, Division of RHN, ICMR, New Delhi 

Introduction: Japanese encephalitis is an acute disease (caused by an arbovirus) of major public health importance, which primarily attacks children and has a high case fatality rate of 20 percent or much more and in large number of instances sequelae occur among survivors. This viral encephalitis has reservoirs in pigs and water frequenting birds, is transmitted by Culex mosquitoes and has a potential for outbreaks. Gorakhpur is reporting outbreaks of Japanese encephalitis regularly for many years. In the year 2004 there was an outbreak too. National Institute of Communicable Diseases (NICD) constituted a team of four including epidemiologists, entomologists and clinicians to undertake epidemiological investigations. Objectives: The outbreak was investigated with the objectives to confirm the existence of an epidemic, to identify the source of the outbreak and to understand the circumstances due to which the epidemic was taking place. Methods: The team visited Gorakhpur from 14th to 17th Sept, 2004. The team interviewed the key persons and analyzed the records. They also did clinical examination of patients, eco-entomological survey and collection of blood samples of patients for serological survey methodology to confirm the outbreak and understand the epidemiological reasons behind the outbreak in order to suggest control measures and future prevention. Results: There were cases of encephalitis occurring in the district of Gorakhpur as well as in the adjoining districts in the region. The diagnosis was also established as IgM positive for Japanese Encephalitis at the NICD laboratory. Total number of cases in the district of Gorakhpur were117 and deaths were 24 from 12 th

July, 2004 to 13th September, 2004. Cases were still continuing to come. All the PHC areas and blocks in the district were affected. Entomological investigations revealed C tritaeniorhynchus at outbreak level density in the affected villages. The density was indoor 8 per man-hour and outdoor 60 per ten sweeps of 'mosquito catching net'. Amplifier host of Pigs and ardeid birds were common in the area. Conclusions: Gorakhpur is one of the districts with highest number of cases and deaths due to encephalitis and a programme is also launched to immunize 1-3 year old children in the district with 2 doses of killed JE vaccine in the same year. The age band may be increased as well as community compliance should also be ensured for sustainable results. Vector control measures in the district needed be intensified including focused IEC for source reduction (pigs segregation). Personal protection measures may be emphasized in a tailored campaign to protect the children from mosquito bite. Physical medicine unit, paediatric neurology and resuscitation teams may be organized in a dedicated unit for better case management and reduction in death and disability.

NEU/24(P) AN INTERESTING CASE REPORT OF HEMIPLEGIA IN CHILDHOODDilip Mukherjee, Asha Mukherjee, Arpan Agarwal, Saradindu Sarkar, Ramakrishna Mission Seva Pratishthan-Vivekananda Institute Of Medical Sciences, 99, Sarat Bose Street. Kolkata-700026

This communication deals with a case of hemiplegia in a child and its investigations. Raja Doloi,a previously healthy 21/2 yr old Hindu male residing in 24 Pgs district of West Bengal presents to the ED 12 hrs after developing sudden onset weakness followed by inability to move left upper and lower limbs and deviation of angle of mouth to right side. Mother informed that while playing with friends the previous afternoon, Raja suddenly fell on ground after which he was unable to get up and stand of his own. He remained irritable for whole night until next morning when parents observed facial asymmetry, aphasia,unable to move left limbs.No h/o blood transfusion,diarrhea, bleeding manifestation,prolonged fever, cong. cyanotic heart disease, joint pains, sore throat, TIA. Birth and Development history;Normal O/E: Healthy, alert,no malnutrition, no distress. Vitals: T=98.6;P=100/min, regular,equally palpable in 4 limbs; R=26; BP=148/92 mm Hg (severe hypertension) CVS & Resp Sys: NAD CNS: Cranial Nvs; Left sided UMN Facial Nv palsy. No other CN deficiet.; TONE:Increased in left upper and lower limbs; POWER: Left Upper 2/5; Left Lower 3/5; Right Upper and Lower limbs: 5/5 Plantar: Extensor on left side; DTR: All Brisk on left side. No sensory deficiet, involuntary movements,cerebellar or meningeal signs. Fundoscopy-normal CT Scan done urgently shows hemiatrophy of right hemisphere with focal infarcts on both right and left side corresponding to ACA & MCA territory.. We proceed to evaluate for ischemic stroke. Hematological tests(CBC; Electrolytes etc) reveled only moderate anemia ; ECG,ECHO: NAD; Collagen Vascular screen : Normal; Protien C & S and Antithrombin III- Not Deficient VDRL, HIV , Lactate, Pyruvate levels- normal Urinary VMA, USG Abd, Renal Artery Doppler: all normal (for hypertension evaluation) MRI and MR Angiography done: Reveals narrowing of distal portions of both lt. and rt. Internal carotid artery and basilar artery with non visualisation of proximal portions of lt. Aand rt. ACA, MCA, and PCA. Collateral vessels noted on the left side. This confirms the diagnosis of Moya Moya Disease as evidenced by MR Angio in a case of left sided hemiplegia with ipsilateral facial nerve palsy anh aphasia associated with severe hypertension.

NEU/25(P) A STUDY OF HYDROCEPHALUS BETWEEN AGE GROUP 0-10YRS WITH SPECIAL REFERENCE TO AEITOLOGY AND CLINICAL FEATURE.Joydeep Das, Swapan Mukherjee, Jayanti Chakraborty, Rajoo Thapa, Shantoo PramanikInstitute of child health,Park circus.Kolkata-17  Objective: Hydrocephalus is a major cause of childhood disability and it is one of the common cause of chronic illness of childhood.This study analyses clinical profile,pattern and spectrum of disease and aeitological diagnosis of patients presented with hydrocephalus between age group0-10 yrs. Method:This is a cross sectional randomised study of 48 pts.under 10 yrs. of age at a urban tertiary referal center over a span of one year from September2004 to September2005. Results and analysis:Out of 48 cases heading the list is congenital acqueductal stenosis with 12 pts(24%) followed by pyogenic , tubercular meningitis and hydrocephalus ex vacuo were 20%,18% & 12% respectively.5pts(10%) were diagnosed as ICSOL & 3pts(6%) were labelled as viral meningitis.Lastly, there were 4% cases of acqueductal stenosis with arrested hydrocephalus and one case of normal pressure hydrocephalus. Conclusion: As this study analyses the spectrum of clinical presentation alongwith aeitological diagnosis so it will help pediatrician and neurologist to plan a therapeutic approach and avoid further damage to the neural tissue as well as developing brain.

NEU/26(P) A STUDY OF MUSCULAR WEAKNESS IN CHILDREN IN NORTHERN INDIARahul Jain, Bibek Talukdar, Vinod Puri, Medha TatkeDepartment of Pediatrics, Neurology and Pathology, Maulana Azad Medial College and Associated G.B.Pant Hospital, New Delhi-110002

Introduction: Muscular weakness is an important pediatric problem but has not been studied well in India. Objective: A study was planned to find the disorders causing muscular weakness in children. Methods: Consecutive cases presenting with primary complaints of weakness of muscles of any part of the body, within a year (between march 2004 and February 2005) were enrolled for the study. Weakness associated with upper motor neuron diseases were excluded. All the cases were admitted and worked up. Besides clinical evaluation, the cases were subjected to investigations that included, besides others, estimation of serum creatine phosphokinase, serum glutamate pyruvate transaminase, serum glutamate oxalate transaminase, electromyographic studies, nerve conduction studies and histopathological studies of muscle biopsy specimens, as required, to confirm the diagnosis. Results: Most of the cases hailed from Delhi and the neighboring states. 41 Cases, who met the case selection criteria, were investigated. The etiologies found were acute flaccid paralysis 15 (36.5%), Duchenne muscular dystrophy 12 (29.26%), Becker muscular dystrophy 1 (2.44%), congenital muscular dystrophy 1 (2.44%), spinal muscular atrophy 5 (12.19%), hereditary motor and sensory neuropathy 2 (4.87%), myasthenia gravis 1 (2.44%), glycogen storage disorder type II 1 (2.44%), congenital Bell palsy 1 (2.44%), and congenital Erb’s palsy 1 (2.44%) and unclassified 1 (2.44%). Amongst the patients with acute flaccid paralysis, 13 (31.71%) had Guillain-Barré syndrome and 2 (4.87%) had diphtheritic polyneuropathy. Conclusion: The study shows that Guillain-Barré syndrome dominates the acute disorders while Duchenne muscular dystrophy the chronic disorders causing muscular weakness in children in North India.

NEU/27(O) INCIDENCE OF CSF ELISA POSITIVITY FOR NCC IN PATIENTS PRESENTING WITH FOCAL SEIZURES AND HAVING RING ENHANCING LESION IN BRAINSuman Lata,Jacob Puliyel,Nirmal Kumar,Sona Chowdhary,Vikas BhambhaniDepartment of Pediatrics,St. Stephen's Hospital,Tis Hazari, Delhi-54

BACKGROUND AND OBJECTIVES: This study is to look at the incidence of CSF-ELISA for neurocysticercosis positivity in those who have ring – enhancing lesion on CT scan .The study was conducted among twenty two consecutive children presenting with focal seizures to our pediatric ward. MATERIALS AND METHODS: Twenty three patients were enrolled in the study in the age group 6 months to 12 years who presented with focal seizures. Analysis was done to look at the incidence of ring – enhancing lesions in patients with focal seizures and to look at what percentage of patients have CSF ELISA positivity for neurocysticercosis among patients with focal seizures and ring – enhancing lesions. ELISA positivity was also co-related with red meat intake, literacy of parents and socio-economic status RESULTS: In our study 60.9% children who presented with focal seizures had ring – enhancing lesions in the brain. 38.5% of the patients who had focal seizures and ring –enhancing lesions in the brain had CSF ELISA positivity for neurocysticercosis. CONCLUSION: CSF ELISA for neurocysticercosis would be a helpful investigation in all children who present with focal seizures and have ring – enhancing lesions in CT SCAN as it will help in determining the etiology and further management of these cases.

NEU/28(P) INTERMITENT PROPHYLAXIS WITH ORAL DIAZEPAM IN FEBRILE SEIZUREAjoy Kumar SarmaOil India Hospital, Duliajan, Assam

Aim and objective : To evaluate the effects of oral diazepam as a intermitent prophylaxis in febrile seizure. Settings : Oil India Hospital, Duliajan, Assam Material and Methods: All children with febrile seizure presenting at the hospital from 01/04/2003 to 31/03/2005 were considered. Diagnosis is done by taking detail history, clinical examination, heamatological and biochemical studies and CSF analysis (when indicated). On discharge oral diazepam was advised 0.3mg / kg /day tid for 2 days for subsequent febrile episidoe, along with antipyretics. Results: We treated 82 patients aged between 6 months to 6 years. They have had simple or complex febrile seizures. Recurrence occurred in 22 patients (26%), none had a long lusting febrile convulsion. Transient side effects occurred in 21.95% of the cases.Conclusion : We conclude that oral diazepam is safe and effective drug for prophylaxis of febrile seizures when used as soon as any signs of illness appears.

NEU/29(P) IN ACHIEVING GOALS OF EARLY IDENTIFICATION AND INTERVENTION OF CHILDREN WITH HEARING IMPAIRMENTMohd. Shamim Ansari, Ashok kumar SinhaAli yavar Jung National Institute for the hearing handicapped, K.C. Marg, Bandra (w), Mumbai-400050.

Early detection and intervention of hearing loss of any degree is critical to the linguistic, social and educational development of children with auditory deficits. The present study was conducted to determine the ages of suspicion in children with hearing loss. The survey revealed that the average ages of detection of hearing loss by parents is about 11.45 months, which unexpectedly lower than the reported ages of Identification of hearing loss in Indian literature. The present study also found delay in suspicion of hearing loss, initiation of diagnosis and fitting of amplification essential to initiate and institute early remedial programs to facilitate development of speech and language skills in children with hearing impairment. Surprisingly these delays are found to be attributable to the professional failure to detect the presence of hearing loss at young age. Thus the study emphasizes that parents are at best position to detect the hearing problem in their children, hence they can be effectively utilized as manpower/ equal partners in achieving the goals of early identification of hearing loss. It also advocates for imparting the sufficient training to those who are involved in the Rehabilitation of children with hearing impairment.

NEU/30(P) INTERESTING CASES OF ATAXIASharma Anita, Kalra Veena, Banerjee Bidisha, Kamate Mahesh, Kabra Madhulika Department of Pediatrics, Division of Neurology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110 029

Background-Chronic or recurrent ataxia are of diverse etiologies – post infectious, paraneoplastic, degenerative, metabolic or structural. Identification of cause is based on identifying pointers in clinical phenotype. Three interesting prototypes cases of chronic ataxia are described. 15 Y/F Progressively increasing difficulty in walking, frequent falls, writing, numbness feet, truncal incurving, for 2 yrs. No cognitive decline or headache. Examination-Muscle mass & bulk normal except Wasting of small muscles of hands & feet. Tone and Power normal. All DTRs absent, Planter B/L mute. Pain & touch normal. Impaired position sense and vibration both LL. Romberg Sign Positive, MRI spine -spinal atrophy. Possibility Friedrich’s Ataxia 12- year-boy presented during third episode of acute intermittent ataxia. Present episode started two months back& was longest one. Examintion- FTT, no nystagmus or telengectasia. Power 4/5. DTR normal upper limbs, absent lower limbs. Plantar flexor. Pain and touch normal. Vibratory & joint position sensation impaired all limbs more in lower limbs. Romberg sign positive. MRI normal. Recurrent Intermittent ataxia with complete recovery suggested neurometabolic disorder. No response on motor and sensory nerve conduction.Urinary organic acids normal. Lactate / Pyruvate (L/P) ratio was normal. Oral glucose challenge test for dysmetabolic state done. Two fold rise in. blood lactate & pyruvate noticed. Urinary alanine by semiquantitative test positive. Reversible metabolic cause of intermittent ataxia demonstrated. Diagnosis –Pyruvate Dehydrogenase Complex deficiency considered. Patient put on thiamine and showed improvement within a week. 7 yr boy, one yr h/o ataxia, falls, dropping of objects. Static symptoms. No cognitive decline, skeletal abnormality or family history. Examination- Ambulatory with support, DTR sluggish, Sensory system normal, Cerebellar sign +. On MRI cerebellar atrophy, lactate, ammonia, organic acid, serum cholesterol, triglycerides normal. LDL & Apolipoprotein B low with normal Apolipoprotein A. Possibile diagnosis Hypobetalipoproteinemia. Conclusion- Paraneoplastic related to neuroblastomas and postinfectious were commonest.      

NEU/31(O) IN VIVO EVALUATION OF NEURONAL DENSITY AND MYELINATION CHANGES IN THE CEREBELLUM IN MALNUTRITION.Rachna Seth, Veena Kalra , Uma Sharma, N R JagannathanDepartment of Pediatrics and NMR, All India Institute of Medical sciences,New Delhi, India

Background. Animal studies and autopsy data have shown that malnutrition is associated with reduction in number of neurons and diminished myelination . These changes are most pronounced in the cerebellum. We attempted to assess Cerebellar neuronal density and myelination in children with malnutrition using magnetic resonance spectroscopy (MRS). Methodology. Children with PEM and normal age matched healthy controls underwent Proton MRS of the cerebellum. MRS was performed by 1.5 Tesla (Sonata, Siemens) using a CP array head coil. MRS was repeated after 3-6 months of nutritional rehabilitation and compared with baseline spectra. Vitamin E level estimation was also done and correlated with the level of neurometabolites IQ assessment was also done. Results. All the patients had late onset malnutrition with onset after 2 years in majority (85%). Two patients were low birth weight babies. The proton MRS spectra showed three major resonance peaks arising from N-acetyl aspartate (NAA) at 2.02 ppm representing neuron density, Creatine ( Cr) and Phospho creatine (PCr) at 3.04 ppm representing energy metabolism and from Choline (Cho) and Phosphoryl choline at 3.20 ppm representing myelination. . 72% PEM subjects had normal IQ while 22% had dull normal IQ. Head size and development was normal in all PEM subjects. The ratios of neuromatabolites (NAA/Cr & Cho/Cr) were comparable between the controls and PEM subjects. These ratios did not change after nutritional rehabilitation. Conclusion: This pilot study establishes similar baseline spectra of proton MRS in PEM subjects and age matched controls. Brain maturation occurs mostly during the first two years of life, and proton MRS is shown to reflect this development The metabolite ratios were similar in PEM subjects before and after nutritional rehabilitation thus reinforcing the concept of ‘brain sparing’ and proving it at the molecular level. Our data establishes that neuronal density and myelination were not affected in PEM acquired after two years of age. This proves that brain metabolites are spared in late onset chronic malnutrition.

NEU/32(P) INTRAVENOUS IMMUNOGLOBULINS (IVIG) IN SEVERE GUILLIAN - BARRE SYNDROME(GBS)S.S. Dhanawade V.K. Patki , B.B.Shahvkp31073@rediffmail.com

Aims and objective - To evaluate whether early use of IVIG can reduce need of mechanical ventilation and mortality in patients with GBS. Material and methods -This is a retrospective analysis of 21 children with severe GBS admitted to PICU of Wanless Hospital, Miraj from Aug.2000 to Aug.2005. All children received IVIG infusion in total dose of 2g/kg over 2 -5 days (unless death supervened). The need for mechanical ventilation, mortality and period between maximum disability to full ambulation were compared between the patients who were electively admitted and those who were referred late by using Student t-test for unpaired data. Results -The age of the patients ranged from 10 months to 15 yrs with mean of 4.5 yrs. Male to female ratio was 2.5. The children were admitted to PICU after a median of 2 days onset of weakness (range 1-4days ). The disease progression from onset to maximum disability was seen over a median duration of 2 days (range 1-4 days).13 children were electively admitted whereas 8 children were transferred from other hospitals in late stages. Fourteen children required mechanical ventilation. There was one death in children who admitted electively whereas 6 deaths in children who had been transferred late (p<0.01).Median duration of ventilation, median duration of PICU stay and median period from maximum disability to full ambulation were (11 days , 15 days & 60 days respectively) much higher as compared to that of elective admissions( 7 days , 9 days & 33days respectively).There was no side effect of IVIG therapy. Conclusion - Early use of IVIG could reduce the mortality and the need of intubation and mechanical ventilation.

NEU/33(P) MYASTHENIA GRAVIS- CASE REPORT Karuna Thapar, Abha Midha, Gaurav DhawanDepartment of Pediatrics, Govt. Medical College, Amritsar-143001

Introduction: Myasthenia gravis is a disease caused by an immune-mediated neuromuscular blockade. There is decrease in number of available Acetyl choline receptors due to circulating receptor antibodies. Disease is generally nonhereditary and is an autoimmune disorder. Case Report: 3 year old female child complained of drooping of left upper eyelid, difficulty in swallowing, slight difficulty in walking for 1 year. Patient took treatment from various local practioners, took steroids, and developed hirsutism so drugs were stopped. Patient reported in outdoor with drooping of left upper eyelid and mild dysphagia. On examination child was healthy with normal anthropometric measurements with drooping of left upper eyelid. Vitals were normal. Systemic examination including neurological examination was normal. No family history. No history of diplopia. Examination of eye revealed normal visual acuity, normal fundus examination, and normal sized pupil reacting to light. T-3, T-4 and TSH were normal. Anti acetylcholine esterase test could not be performed due to cost factor. ANA was negative. Serum CPK was normal. ECG was normal. Child responded to edrophonium chloride (1mg/kg). Child was then diagnosed as a case of myasthenia gravis and put on neostigmine methylsulphate (0.4mg/kg) orally every 4-6 hours.

NEU/34(P) MAGNETIC RESONANCE IMAGING IN PEDIATRIC NEUROLOGICAL DISORDERS Mukti Sharma, Pl Prasad, Ss Nawre Command Hospital (CC), Lucknow Cantt – 226 002

Background MRI of the brain and spinal chord is done for all kinds of neurological symptoms. We undertook a retrospective analysis of the records of all children who underwent a MRI of the CNS at our hospital and correlated the outcome to evaluate its utility in pediatric neurological disorders. Patients & Methods Seventy four children underwent MRI of the brain and spinal cord between Jan 04 to Aug 05, of which half were males and mean age was 6.4 (range 1 month to 10) years. Children with febrile seizures and trauma were excluded from the study. The clinical features, biochemical parameters and outcome of MRI were correlated. Results Of the 28 children evaluated for seizure disorders, nine (32%) had inflammatory granuloma, two (7%) had cerebral atrophy. Of 18 children with congenital anomalies; meningo-myelocoele (n=4), tethered cord (n=2), spinal diastometomyelia (n=1), sacrococcygeal mass (n=2), occipital encephalocele (n=4), hydrocephalus (n=4), congenital AV malformation (n=1) the abnormality could be delineated well in all. Of the 12 with developmental delay, 3 (25%) revealed effects of HIE and the rest were normal as were all 5 scans of children with cerebral palsy. Of the five paralytic children, one with infantile hemiplegia revealed gliosis in the brain scan, the rest were normal. All six children non-specific headache had normal brain scans Conclusions MRI scan is a useful diagnostic tool in seizure disorders and congenital anomalies of the CNS, however its role in children with developmental delay, cerebral palsy, paralysis and non-specific headaches is limited.

NEU/35(O) NEURODEVLOPMENTAL OUTCOME OF NEONATAL SEIZURE IN TERM NEONATES -9 MONTHS FOLLOW -UP STUDYSatheesh.C.T, Rathika Shenoy, Kamalakshi Bhat, Nutan Kamath.Kasturba Medical College, Mangalore, Karnataka – 575001

Aim: To analyze the neurodevelopmental outcome of Neonatal intensive care unit [NICU] term graduates with seizures at 9months. Methods: Prospective study on term neonates with seizure over period of 20months.The neurodevelopmental assessment using bayley scales of infant development (BSID) was scheduled at every 3months. Sequelae of seizures analyzed were mortality, motor and mental quotient<70% on BSID, microcephaly, seizure recurrence, visual and hearing impairment. EEG was done. Results: cohort of 61term neonates with seizures were enrolled. 62.29% of cases were followed up for 9months, there was male predominance and 28.9% were small for gestational age (SGA) in this series. Common seizure pattern observed were Subtle and multifocal .76.3% of cases had seizure onset in first 3days. Birth asphyxia was noted in50% of cases. Out come at 9 months: Seizures recurrence in[31.5%] of cases, Microcephaly[ 27%], Motor delay[43.25%], Psychomotor delay[37.8%], Visual impairment[ 27%], Hearing impairment[8.1%]. 72.7% of SGA babies had abnormal outcome. Seizures due to hypoxic ischemic encephalopathy (HIE) stage III, inborn error of metabolism (IEM), persistent hypoglycemia and CNS malformation had significant abnormal outcome. Majority of neonates with poor neurological status had abnormal outcome. Other factors associated with abnormal outcome were Myoclonic seizures, generalized tonic and presence of background EEG abnormality. Conclusion: 1. Seizure etiology and neonatal neurological status significantly determined long term neurodevelopmental outcome. 2. Neonates with seizures and associated co-morbid conditions require long term follow up.

NEU/36(P) NEONATAL SEIZURES: TOWARDS A MORE PRECISE DIAGNOSIS AND ITS IMMEDIATE OUTCOMEManinder S. Dhaliwal, Anuj Bansal, Arun S., Raghuveera K.Kasturba Medical College, Mangalore, Karnataka – 575001

Neonatal seizures continue to be a motive for multiple controversies: the diagnosis only by clinical approaches, the necessity to confirm with EEG record and their treatment and control. Objective: To establish the incidence of type of clinical neonatal seizures and their relation to the electroencephalogram(EEG), the underlying etiology with emphasis on immediate outcome. Design: A prospective hospital based study. Methods: All neonates with seizures admitted in Neonatal special care unit, to the constituent hospitals of Kasturba Medical College, Mangalore were studied from Aug. 2002 to Nov. 2004. Necessary investigation including EEG were carried out and analyzed. Immediate outcome was determined by global neonatal neurological assessment as proposed by Agustin Legido et al. Results: 111 neonates with seizures were studied wherein majority (71%) of seizures occurred in first two days of life. Hypoxic ischemic encephalopathy [HIE](38%), followed by infection and hypoglycemia were the common causes. Multifocal clonic seizure type (38%) was the commonest seizure pattern observed. Good immediate outcome was seen for focal seizures (p<.012) and hypocalcemia while poor outcome was seen for tonic seizures (p<0.0005), interventricular hemorrhage and HIE. Out of 111 cases, 66 EEG were recorded, 30 came abnormal. Maximum correlations between seizure type and EEG abnormality were found for mixed type (62.5%) and multifocal clonic(59%) seizures. Background abnormality in EEG was more common in cases of HIE (35%) and hypoglycemia (28%). Conclusion: Thorough clinical examination with appropriate laboratory investigations which includes EEG is essential in all cases of neonatal seizures for diagnosis, effective treatment and to prognosticate outcome.

NEU/37(P) NEUROCYSTICERCOSIS IN CHILDREN: CLINICAL CHARACTERISTICS AND OUTCOME. A STUDY FROM EASTERN INDIA. Abhijit Dutta, A.Mallick, J.K.De, D.P.BanerjeeCalcutta National Medical Collage & Hospital,Gora Chand Road, Kolkata-700014 INTRODUCTION & OBJECTIVE : Neurocysticercosis is the most common parasitic infection of the central nervous system. Majority of patients presents with seizure. Objective of this prospective study was to determine clinical characteristic and outcome of neurocysticercosis over a period of three years.METHODS: We studied 58 children under 15 years with neurocysticercosis. Diagnosis based mainly on CT scan and MRI of brain. Detailed history, seizure activity, neurological finding and respond to therapy were noted and they were prospectively reviewed with repeat CT scan of brain at 3-month interval over 3years. RESULTS:Among 58 childrens included in the study, 25 were male and 33 were female. Average age of presentation was 8.5 years. Focal seizure with or without secondary generalization was common mode of presentation. Headache was associated in 13(22.4%) children. Ring enhancing CT scan of brain was common radiological finding and 72.4% showed perilesional edema. Al the lesions were parenchymal and most (83%) were single and locating in the following order: parietal-30 (51.7%), fronto-parietal-15 (25.8%), frontal-9 (15.5%) and occipitel-4 (6.8%). Albendazole therapy given to some patients did not have any beneficial effect on seizure control. On follow up, 72.4% of lesions disappeared, 26% get calcified and 2.3% of lesions persisted at the end of one year. CONCLUSION: Neurocysticercosis is an important cause of convulsion in children specially between 6-9 years and it should be considered as differential diagnosis of childhood convulsion. In the majority of the patients the lesions disappeared spontaneously and the prognosis is good.

NEU/38(P) PAEDIATRIC CNS TUMOURS IN MADURAIJ. Balaji, D. Meikandan, P. Amutha rajeswari, M.L. Vasanthakumari, R.A. SankarasubramanianN. Ragavan, V.InbasekaranInstitute of Child Health and, Research Centre And Neurosurgery, Department, Govt Rajaji Hospital, Madurai Medical College, Madurai.

INTRODUCTION :Brain tumours are the most common solid tumours in children. It is the second most common neoplasm of childhood. AIMS & OBJECTIVES : To find out the prevalence of paediatric CNS tumours, to analyze clinical profile and various types. MATERIAL & METHODS : A prospective observational study was done for suspected cases of CNS tumours admitted in our hospital in one year (1st September 2004 to 31st August 2005) after institutional ethical clearance and with informed consent. Post operative cases were followed up. The clinical profile and types of CNS tumours were analysed by using simple descriptive statistics. RESULTS :Out of 6601 inpatients, 16 had CNS tumours by clinical and Radiological diagnosis. Out of which 11 cases were operated and biopsy proved. There is no gender variation (M:F = 8:8). Age group less than 3 years 50%, from 4 to 10 years 18.75% and above 10 years 31.25%. Clinical profile showed that head ache was present in 44%, vomiting 63%, seizures 25%, papilledema 81%, hydrocephalus 37.5%, Motor deficit 19 %, cerebellor signs 37.5%, cranial nerve palsy 18.75%, Meningitis like picture in 2 cases, Neurodegenarative disorder like picture 1 and Diabetes Inspidus in 1 children. Types of CNS tumours were medulloblastoma or PNET 43.75%, Astrocytoma 25%, craniopharyngioma18.75% and Ependymoma 6.25%. CONCLUSIONS :Infratentorial tumours were more common. Vomiting, Headache, papilledema were more common presenting features. The rise in incidence of paediatric brain tumours is due to high level of suspicion and better radiological diagnostic methods.

NEU/39(O) PAROXYSMAL NON EPILEPTIC EVENTS IN CHILDHOODSushma Malik, Pravin Jawale, Surekha Joshi, Sushma SavePediatric Neurology Clinic, Department of Pediatrics, T.N. Medical College & B.Y.L. Nair Hospital, Mumbai.

Introduction:-Paroxysmal nonepileptic events (PNE) are frequently encountered in children and adolescents. These episodes of movement, sensation or behavior closely mimic epileptic seizures but do not have a neurologic origin and therefore need to be differentiated from true seizures for appropriate management. Aims :-To determine the prevalence, demographic profile and spectrum of PNE in childhood. Setting :–Pediatric Neurology Clinic and wards. Design:- Prospective study. Methodology:- 61 cases of paroxysmal nonepileptic events (PNE) were enrolled in the study over one year. Diagnosis was primarily based on history, clinical evaluation, psychological assessment and investigations like CBC, 2D ECHO, IQ/DQ, Tilt test, and BERA. EEG, Video EEG and neuroimaging were done to rule out seizure disorder. All were analyzed using Chi Square test and standard error of proportion. Results:- The spectrum of PNEs observed in our study were breath holding spells (BHS) (45.9%), syncope(9.8%), pseudoseizures(6.6%), night terrors to tic disorders. On reviewing the demographic profile, a male preponderance (1.9:1) was noted and 82 % children were below 10 years. BHS was encountered below three years of age and 75% of pseudoseizures and 83.3% of syncope were found in children above 10 years. Amongst all cases of PNE, 7 subjects had associated epilepsy and nearly 1/3rd

of the cases had behavioral problems. The latter encompassed cases of aggressive behavior, temper tantrums, stubbornness, anxiety etc. Conclusions:- In a substantial proportion, a careful history and examination elucidates the nature of PNE. EEG &Video EEG monitoring is the key to successful and accurate diagnosis of PNE. Differentiation from true seizures is essential to provide appropriate psychological and psychiatric assistance.

NEU/40(O) PREDICTION OF MYOCARDIAL DYSFUNCTION IN DUCHENNE MUSCULAR DYSTROPHYRachna Seth, Veena Kalra, Sandeep Seth*, Mahesh Kamte, Bidisha Banerjee.Department of Pediatrics and *Cardiology, All India Institute of Medical Sciences, New Delhi, India

Background: Cardiac involvement occurs in 90% of DMD patients. Early detection of cardiac involvement may permit timely therapeutic intervention with vasodilators and beta blockers. Autopsy data has shown that the fibrosis starts in the posterior and lateral walls. Tissue Doppler imaging (TDI), a new echocardiographic imaging technique, picks early changes in regional systolic and diastolic function much before conventional echocardiography. Methods: Patients with DMD were evaluated using conventional echocardiography and tissue Doppler imaging to assess left ventricular systolic and diastolic function. The left ventricular lateral wall was specifically examined by both techniques to pick up regional myocardial dysfunction. The tissue Doppler and echocardiographic findings were compared with age matched controls. Results: None of the patients with muscular dystrophy had any cardiac symptoms. 80% of the non-ambulatory patients had left ventricular dysfunction by both conventional echocardiography and tissue Doppler imaging. Tissue Doppler evaluation of the lateral wall revealed reduced systolic velocities (mean ± SD: 7.8 ± 1.8 cm/sec vs controls 8.6 ± 1.8cm/sec) . 45% of ambulatory DMD patients had normal conventional echocardiographic evaluation of the lateral wall but tissue doppler revealed latent abnormalities of systolic function (systolic velocities <8 cm/sec ,p<0.05). In contrast, the left ventricular septum did not show any abnormalities by either conventional or tissue dopper echocardiographic abnormalities. All the patients with abnormal tissue Doppler findings were put on vasodilators and followed up with no worsening of cardiac function over the next one year. Conclusions: Almost half the patients with DMD had asymptomatic cardiac involvement (lateral wall) despite normal ejection fraction. TDI is a more sensitive tool to pick early myocardial dysfunction in DMD and enabled initiation of drugs like Angiotensin converting enzyme inhibitors which prevented overt LV dysfunction.     

NEU/41(O) THE ROLE OF NEUROIMAGING IN EVALUATING CHILDREN WITH FIRST AFEBRILE SEIZURES     Mritunjay Pao, Vineet Bhushan Gupta, Anil Kumar, Amita Mahajan, Praveen Khilnani, Atul ChopraApollo Centre for Advanced Pediatrics, Indraprastha Apollo Hospital, New Delhi

Neuroimaging is an expensive modality. Its place in the workup of child presenting with new onset afebrile seizure is unclear. Aims & Objectives: To determine the diagnostic utility of neuroimaging in children 1 months to 16 years of age with no prior neuropathy, presenting with the first episode of afebrile seizures. Methods: Retrospective study. Study period: 1/01/2004 to 31/08/2005. Inclusion criteria: 1) all children aged 1 months to 16 years presenting with first episode of afebrile seizure. Exclusion criteria: 1) all febrile seizures, 2) recurrent seizures, 3) primary diagnosis other than seizures e.g., trauma/meningitis etc., 4) nonseizure diagnoses, such as GER, breath-holding spells, and syncope. Results: A total of 1214 children aged 1 month to 16 years attended the emergency and neurology OPD of this hospital. Of these 356(29.32%) children presented with seizures, 138(38.76%) had first onset afebrile seizures and 104(75.36%) underwent neuroimaging. Sixty four were males and 40 females. Of these 104 children 38(36.5%) had a positive finding on neuroimaging. Thirty five (33.7%) had inflammatory granuloma. Three children had lesions, which required intervention including a giant aneurysm, fibrillary astrocytoma and aneurysm of the internal carotid artery. Thirty one children had NCC (neurocysticercosis) and 4 children had tuberculoma diagnosed by neuroimaging criteria and supported by serology. Patients with NCC presented with simple partial seizure (n=15), versive seizure (n=4), complex partial seizure (n=3), generalized tonic clonic seizure (n=3), simple partial seizure with secondary generalization (n=4), atonic seizure (n=2). Of the four children with tuberculoma 2 had simple partial seizure, (1), generalized tonic clonic seizure and 1 focal with secondary generalization. Conclusion: In comparison to published western data a significantly higher proportion of patients had positive neuroimaging in this study. Neuroimaging should be considered in children presenting with the first afebrile seizure in a country like India where tuberculosis and neurocysticercosis are endemic.

NEU/42(P) TOPIRAMATE AS AN ADJUNCT IN LENNOX - GASTAUT SYNDROME: A CASE REPORTV V Tewari, P L PrasadDept of Pediatrics, Military Hospital Namkum, Ranchi, Jharkhand – 834010

A 5 year old girl, presented with early childhood onset refractory seizures. She was diagnosed as Idiopathic Lennox - Gastaut syndrome and managed with Topiramate and conventional anti epileptic drugs. This report highlights beneficial effects of this drug combination. KEY WORDS: epileptic encephalopathy, spike and slow waves INTRODUCTION: Lennox-Gastaut syndrome is an epileptic encephalopathy in which the epileptiform abnormalities by themselves contribute to the progressive disturbance in cerebral function. CASE REPORT: A 5 year old girl child presented with intractable generalized tonic clonic and myoclonic seizures. In addition, there appeared atypical absences, loss of previously acquired language and fine motor skills. She was managed with Sodium Valproate and Clonazepam without much relief. Her MRI brain is normal and EEG showed recurrent bursts of spike and slow waves. The benzodiazepine was alternated between Clonazepam and Clobazam. Topiramate was added as an adjunct. DISCUSSION: This syndrome is considered as a prototype of a heterogenous group of disorders referred to as the ‘epileptic encephalopathies’. The EEG characteristically shows bursts of slow spike wave discharges (2.5 Hz or less). Sodium Valproate is the drug of choice. Benzodiazepines are also effective. Drowsiness is associated with more seizures, as happens with benzodiazepine usage. A combination of Sodium Valproate with benzodiazepines produces better results than either one used alone. Topiramate has been found to be effective as an adjunct and is a safer option. Topiramate was used intermittently with Sodium Valproate and Clonazepam or Clobazam. It was used at a starting dose of 2 mg/kg/day and slowly increased to 6 mg/kg/day. Once there was greater than 50% reduction in the seizure frequency, the benzodiazepine was discontinued and the child continued on two AED’s in order to reduce benzodiazepine tolerance. Currently available data suggest that Topiramate may have safety that is better than Lamotrigine.

NEU/43(O) SENSORY INTEGRATION THERAPY – A CENTRAL ROLE IN THE HOLISTIC MANAGEMENT OF CHILDREN WITH CEREBRAL PALSYParul Valia, Vijal Oza, Spoorthy Prabhu , Unnati DesaiKrishna Developmental Centre , Baroda.

Introduction:- Children with CP have multiple sensory integration (S.I.) problems which hinder their overall development. Aims & Objectives:- To study the extent of problems of S.I in patients with CP To study the benefits of S.I therapy. Material & Methods:- 17 children of CP were studied over a period of 3 years (May 02 –05) for their SI problems. They were tested using the SIPT (sensory integration and praxis tests). A questioner was also filled in by the parents & S.I. therapy was offered accordingly in conjunction with Physio Therapy, Neuro Development Techniques, Speech Therapy & Cognitive Stimulation SI therapy was given in the form of visual, auditory, oral, tactile stimulations & vestibular activity. Results:- Main problems were - (children affected / improved) Distractibility (10 / 3), Abnormal arousal (10 / 8), Altered sleep patterns (4 /0), Altered suck, swallow (6/ 4), Altered tactile defensiveness, discrimination & localization (14/10), Altered visual response (12 / 10), Altered oral response (6/ 4), Altered auditory response (13/ 10) Altered coordination (6/ 3), Altered proprioception (14/ 8), Poor praxis , motar planning (9/ 7), Affected bilateral motor skills (8 / 4), Imbalance in vestibular system , equilibrium (13/10 ) Conclusions:-1. Children with CP benefited from S.I therapy and there was a significant decrease in their sensory problems. 2. The level of achievement in their milestones was significantly better after giving all forms of therapy along with SI than that seen with giving physio, cognitive, speech therapy alone. 3. Their behavioral problems were tackled in a more efficient manner after giving SI therapy.4. They could handle their ADL (activities of daily living) more confidently.

NEU/44(P) SPECTRUM OF CORPUS CALLOSUM DYSGENESISAnju Aggarwal, S.K Sethi, M.M.A Faridi . Department of Pediatrics, University College of Medical Sciences and Guru Tegh Bahadur Hospital, Delhi –95

Introduction: Corpus callosum dysgenesis (CCD) refers to spectrum of callosal anomalies, which range from partial to complete absence of corpus callosum (Corpus Callosum Agenesis CCA) with an incidence of 2-3 per 100 developmentally disabled populations. It can occur either as an isolated malformation (isolated CCA) (49% of cases), or in association with another genetic syndrome or as a part of telencephalic dysgenesis. We are presenting a prototype of each. Case Series: An 8-month male child presented with features of Apert Syndrome and had corpus callosal dysgenesis that has incidence of 27% in Apert syndrome. A 4-month male child who was initially being treated as hypocalcaemia seizures presented with repeated seizures. On examination child had a head circumference (HC) of 38 cms (< 3rd centile) and slight developmental delay. Computed tomography revealed non lissencephalic cortical dysplasia with CCD . Third child was a 11 month old male child who presented with repeated right focal seizures, had a normal HC and normal development. Magnetic resonance imaging revealed isolated corpus callosal dysgenesis. Isolated CCA appears to be related with a good prognosis ( 80% normal outcome) .Genetic associations and prognostic factors that have implications on genetic counseling will be discussed. Conclusion- Corpus callosal dysgenesis, a radiological diagnosis has a wide spectrum of presentation and associations which should be considered for genetic counseling and management.

NEU/45(P) STATUS EPILEPTICUS – A CLINICO AETIOLOGICAL PROFILEShalini Soi, Puneet Jairath, Kamaljeet Kaur, Baljinder Kaur, K.K.LochamDeptt. of Pediatrics, Govt. Medical College/Rajindra Hospital, Patiala – 147001

Objectives : To assess the clinical and aetiological profile of children presenting with status epilepticus. Methods : Period of study was 6 months from February to July 2005. Seventeen children ranging from 1-15 years of age admitted in the department of Pediatrics, Govt. Medical College, Rajindra Hospital, Patiala were the subjects of study. Age, sex, presenting complaints, detailed history, clinical examination and laboratory parameters were recorded in a pretested proforma. Children were categorized into various age groups for the purpose of study. Results : Out of 17 children, 11 (64.8%) were males and 6 (35.2%) were females. Number of children presenting in the age group of 1-5 years, 5-10 years and 10-15 years were 10 (58.85%), 5 (29.4%) and 2 (11.8%) respectively. Children presenting with generalised seizures were 14 (82.3%), complex partial seizures 2 (17.7%) and simple partial seizures 1 (5.9%). Number of children suffering from meningo-encephalitis were 9 (52.9%), epilepsy 4 (23.5%), febrile seizures 1 (5.9%), neurocysticercosis 1 (5.9%), cerebral palsy 1 (5.9%) and cerebral hemiatrophy with arachnoid cyst 1 (5.9%). 6 (35.2%) had neurological deficit, 4 (23.5%) had features of raised intracranial tension. CSF was positive for meningitis in 8 (47.1%) of cases and 5 (29.4%) had abnormal CT scan/2-DMRI findings. 12 (76.5%) children responded to single drug therapy while 5 (23.5%) required multiple drugs for control of seizures. Conclusions : Generalised tonic clonic seizures are the most common type of seizures presenting with status epilepticus and the predominant aetiology is meningoencephalitis.

NEU/46(O) SPECTRUM OF NONEPILEPTIC EVENTS IN A PEDIATRIC NEUROLOGY CENTRERekha MittalCommand Hospital (WC)Chandimandir

Objectives: To study the spectrum of Nonepileptic events (NEEs) detected over a one year period in a Pediatric Neurology Centre. Material and Methods: All cases diagnosed as having NEEs were included in the study. Those children in whom the event had occurred only once were excluded from the study. A detailed history of the events and treatment history was recorded, , clinical exam carried out and EEGs was performed. Attempt was made to observe the event by precipitating the event by suggestion , suggestion with injection of normal saline as being an epiletogenic agent, or by creating situations which precipitated the events in the particular patient. Video EEG was recorded in those children where there was a doubt about the diagnosis of NEE despite history and clinical observation of the patient. Results: 34 cases were diagnosed to have Non epileptic events over a one year period. Of these 4 had reported directly to the Neurology OPD, while others were referred either by Pediatricians or General Doctors. The referred cases were given a referral diagnosis of suspected seizures in 6, seizures in 15 and uncontrolled seizures in 9 cases. 27 children (79%) were on anticonvulsants at the time of reporting. Two neonates had benign neonatal sleep myoclonus. In the 1 month – 5 years group, breath holding spells were commonest (8) cases followed by sleep myoclonus, while in the 5 – 12 years age group functional seizures (4), syncopal attacks (4) and motor tics (3) were seen. Diagnosis of the nonepileptic nature of the events could be made on the basis of history alone in 11 cases ( 32%), on clinical observation of the event in 12 cases (35%), and on video EEG in 11 (32%). Conclusion: Breath holding spells, sleep myoclonus, syncopal attacks and functional seizures are the common non epileptic events that may be mistaken for seizures. Two third of the cases could be diagnosed on the basis of history and observation of the event itself. Video EEG was useful in the remaining one third of cases. 79% were taking anticonvulsant drugs for NEEs at the time of reporting.

NEU/47(P) RADIOIMAGING IN CHILDHOOD SEIZRUESK.K.Locham, Seema Rai, Rahul Gandhi, Ravneet Kaur, Puneet Jairath, Kamaljeet Kaur     Deptt. of Pediatrics, Govt. Medical College/Rajindra Hospital, Patiala - 147001

Objectives : To study clinico-radiological profile in childhood seizures. Methods : 50 children admitted to the department of Pediatrics, Govt. Medical College, Rajindra Hospital, Patiala, were the subjects of study. The age ranged from 1 to 15 years. Detailed history, physical examination and laboratory investigations in the form of CSF examination, ESR, mantoux test, x-ray chest and CT scan findings were recorded in the pretested proforma. Results : Out of the 50 cases, 30 (60%) were males and 20 (40%) were females. 30 (60%), 8 (16%) and 12 (24%) were in the age group 1-5, 5-10 and 10-15 years respectively. 32 (64%) children presented with generalized seizures while 18 (36%) with partial seizures. 20 (40%) children had meningitis. Out of 20 cases of meningitis, 7 (35%) had tubercular meningitis and 13 (65%) had pyogenic meningitis. Neurocysticercosis was seen in 11 (22%) children. Of the 11 cases of neurocysticercosis, 2, 4 and 5 were of age group 1-5, 5-10 and 10-15 years respectively. All cases of neurocysticercosis presented with partial seizures. CT scan revealed inflammatory granuloma (neurocysticercosis) in 11 (22%) cases, subdural effusion in 1 case and infarction in 6 (12%) cases. Neurocysticercosis was seen in parietal lobe in 7 cases , one each in frontal, temporal and occipital lobe while one case had multiple neurocysticercosis. Conclusions : Meningitis was the predominant cause of seizures. Generalized seizures were the most common. Neurocysticercosis was the most common CT scan finding.

NEU/48(P) RECURRENT GUILLAIN BARRE SYNDROME      Prachi Gupta ,Sujata, D.K. TanejaDepartment of Pediatrics, Safdarjung Hospital, New Delhi

Recurrent G.B.S IS A RARE CONDITION and there is paucity of clinical and neurophysiological studies.We report a case of recurrent episode of G.B.S.in a 11 year old boy.Though the history and physical examination was similar to the initial attack,the severity of illness was much more this time. A 11 year old boy , diagnosed 3months back as a case of Acute Inflammatory Demyelinating Neuropathy on the basis of electrophysiological studies and cerebrospinal fluid analysis, given I.V.Immunoglobulin for 5 days and discharged with complete recovery presented again with similar complaints of sudden onset ,bilaterally equal , symmetrical , ascending and progressively increasing weakness involving the lower limbs, trunk and upper limbs,with no features suggestive of bladder,bowel, respiratory, cranial nerve,autonomic or sensory involvement. On examination child was conscious ,oriented with stable vitals. Motor examinatin revealed decreased power in both upper and lower limbs(3/5) , with generalized hypotonia and areflexia, with no sensory involvement and no meningeal signs. The routine investigations were within normal limits.CSF analysis revealed albuminocytological dissosciaton.Electrophysiological studies were suggestive of severe demyelination with axonal neuropathy. The patient was started on I.V.Immunoglobulin again.However the power continued to worsen,and the patient developed sensory and respiratory involvement by the 10th day of illness.He was put on ventilator.As the recovery began , he was weaned off in 7days.The patient improved gradually such that by the beginning of 3rd week he had power of 4/5 with sluggish reflexes and stepping gait.The patient was discharged with complete motor recovery.

NEU/49(O) USE OF CLOBAZAM FOR THE TREATMENT OF REFRACTORY EPILEPSY IN CHILDRENSheffali Gulati, Lokesh Guglani, Mahesh Maheshwari, Amandeep Salhotra, Veena KalraAssociate Professor, Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi

Clobazam is a 1,5 benzodiazepine whose anti-epileptic properties have been shown to be more potent than the “conventional” AEDs. The present study was conducted to evaluate the seizure control with Clobazam being used as an add-on drug for refractory epilepsy in children and to assess the safety profile. Materials and methods: Conducted at Pediatric Neurology Clinic, All India Institute of Medical Sciences, New Delhi. Children with refractory epilepsy, which was uncontrolled with two standard anti-epileptic drugs at maximum doses were enrolled. Ninety two children with refractory epilepsy were started on Clobazam and followed up to 1 year with assessment of seizure control, development of tolerance or side-effects. Results: A total of 92 children (64 males, 28 females), with ages ranging from 2 months to 22 years, with a mean age at onset of seizures being 23.6 months (range 0-143 months) were enrolled. The average dose of Clobazam was 0.7mg/kg (range: 0.2-2.0mg/kg) and mean duration of administration was 15.3 months (median 12 months). The drug had to be withdrawn in 4 (4.3%) due to severe side effects. Side effects were observed in 23 children (25% cases), with majority experiencing sedation and lethargy (20.7%), headache and stuttering (2.2%), behavioral problems, unsteadiness and speech disturbances (1.1% each). Tolerance was noted in 6 cases (6.5%). The overall response to the drug showed no seizures in 39 (42.4%) cases, partially controlled in 30 (32.6%), uncontrolled in 20 (21.7%) cases. Conclusions: Thus Clobazam is effective as an add-on drug for refractory epilepsy in children.

NEU/50(P) INFECTIVE VASCULITIS – THE MAJOR CAUSE OF STROKE IN CHILDREN.Navaneetha S., Piyush Chandel., Ankur Vikas Bopche, Mahendra Jain, Jyotsna Shrivastava, G.S. Patel, Rashmi Dwivedi.Department of Paediatrics, Kamla Nehru Hospital, Gandhi Medical College, Bhopal.Pediatric stroke has a different etiological and epidemiological profile compared to adults. Objective : To study the epidemiology and etiology of pediatric stroke in a tertiary care center in Central India. Material and Methods : All children presenting to the emergency room, Kamla Nehru Hospital, Gandhi Medical College, Bhopal over a period of 2 years from August 2003 to August 2005 with sudden onset of neurologic deficit were included in the study and evaluated as to the cause. After a thorough history and clinical examination, they were subjected to a CT Scan Head and/or lumbar puncture and further work up as necessary. Result : Of the total 57 cases, multiple infarcts including micro infarcts from vasculitis associated with meningitis, mainly tubercular 37 (65%) and pyogenic 11 (19%) turned out to be the major etiologies. Other causes included tuberculoma 4 (7%) Arteriovenous malformation 2 (4%); Thrombocytopenia causing intracerebral haemorrhage 2 (4%) (1 Aplastic Anemia; 1 ALL) sickle cell anemia 1 (2%) and SLE 1 (2%). 28% (16) of the cases were <1 year old; 20 (46%) 1-5 year old, and 15 were >5 year old (26%). Majority of the pyogenic meningitis cases 6 (55%) belonged to the 1 month – 1 year age group; 3 (27%) to the 1-5 year age group and 2 (18%) belonged to >5 year age group. Majority of TB meningitis cases 20 (54%) belonged to the 1-5 year age group; 9 (24%) belonged to the 1 month-1 year age group and 8 (22%) belonged to the >5 year age group. The mortality rates were 9% in pyogenic meningitis 11% in TB meningitis; 50% in brain abscess and 100% in intracranial haemorrhage. Conclusions : The bulk of neurologic morbidity and mortality are due to infective causes and hence early and prompt institution of therapy can prevent mortality and long term morbidity.

NEU/51(O) CLINICAL STUDY ON USEFULLNESS OF CYCLICAL SEDATION IN CHILDHOOD TETANUSM.Veera Shankar, Gopala Krishna Shanbhag.B.V, Yatheesha B.L, Srinivas.V, Champakamalini.SDept. of Pediatrics, VIMS, Bellary.

INTRODUCTION: Tetanus is an important endemic disease in India. It is an acute disease induced by exotoxin of CI. tetani, which is ubiquitous organism, so it can contaminate wounds easily. Clinically it is characterized by muscular rigidity which persists throughout the illness, punctuated by painful paroxysmal spasms of voluntary muscles. Mortality tends to be very high from 40 to 80 percent. OBJECTIVE: To study the clinical profile and outcome of tetanus with respect to cyclical sedation management. DESIGN: Prospective hospital based study of tetanus from August – 2002 to July – 2005.SETTING: DISTRICT HOSPITAL, DEPARTMENT OF PEDIATRICS, VIMS, BELLARY. METHODS: Children with clinical diagnosis of Tetanus admitted during study period are isolated and clinical profile including age, sex, residence, vaccine status. Source of infection, onset of symptoms, duration of cyclical sedation required and duration of hospital stay are noted. Cyclical sedation included appropriate doses of IV injections of Phenobarbitone, Promethazine, Chlorpromazine and Diazepam. RESULTS: Total number of Children during study period were 41 cases. 39 (95.12%) children were below 10 years of age. Male to Female ratio was 1.05:1. Rural to Urban ratio was 3.1:1 (31:10). Source of infection was traumatic in 46.34% (19 cases), otogenic in 17.7% (7 cases), unknown in 36.58% (15 cases). Unimmunized or incomplete immunization was present in all cases. Common presenting symptom was lock jaw; with mean duration of symptoms of 4 days. Mean duration of cyclical sedation was 16 days with mortality of 31.70% (13 cases), maximum mortality was noticed during 48-72 hours; common cause of death was aspiration pneumonia due to uncontrolled spasms. CONCLUSION: Mortality in our hospital was 31.70% (13 cases). Cyclical sedation is effective in preventing death from tetanus to the extent of 68.3% (27 cases) children in our hospital. Since tetanus is a vaccine preventable disease, immunization and health education has to be taken up in a massive scale to reduce the case incidence.

NEU/52(P) RAPIDLY PROGRESSIVE ATYPICAL NEUROCYTOMA OF THE SPINAL CORDNeeraj Awasthy, Avninder Singh, Karam Chand, Premila PaulDepartment of pediatrics, neurosurgery (safdarjung Hospital). Department of pathology(institute of pathology , ICMR)

Extraventricular neurocytomas are a well established entity. non-classical sites like cerebellum, pons and spinal cord parenchyma have been occasionally reported. we report a case of intramedullary neurocytoma at D2-D8 levels of the thoracic cord in an 8 yr -old boy who presented with Para paresis and paraesthesia. The pt underwent subtotal resection. the case is noteworthy not only for its rare site but also for atypical histopathological features like vascular endothelial proliferation mitosis and focal necrosis and high nuclear proliferation index MIB-1.the case highlights the significance of atypical histology , high MIB-1 and the importance of gross total resection and post op radiotherapy

NEU/53 (P) PROFILE OF ACUTE BACTERIAL MENINGITIS IN NEONATES AND YOUNG INFANTSBakulesh Chauhan, Ruby Chander, Niranjan Shendurnikar, Charu Agarwal, Bithika DuttaroyDepartments of Pediatrics & Microbiology, Govt. Medical College, Baroda - 390001

Introduction: Acute Bacterial Meningitis (ABM) is one of the serious infections associated with acute complications, mortality and risk of neurological sequelae. Objectives: The aims of present study were to evaluate the etiology and outcome of ABM and to study the antibiotic sensitivity of organisms responsible for ABM. Methods: The prospective study was conducted in children aged 0 to 3 months of age admitted with a clinical diagnosis of ABM. The diagnosis of ABM was established by suggestive CSF microscopy, biochemistry, Gram Stain and CSF culture. All the babies were treated with intravenous antibiotics for a minimum period of fourteen days. Results: The study included 75 neonates and young infants admitted to NICU/PICU with a clinical diagnosis of ABM. The most commonly observed symptoms were fever, seizures and refusal to feed. CSF culture grew organisms in 28 out of 75 cases and these included Klebsiella, S.pneumoniae, E.coli, S.aureus, Coagulase Negative S.aureus and acinetobacter. Positive and Negative Predictive Values of CSF-CRP was 45.4 % and 74.2 % respectively. Antibiotic sensitivity showed that 50 % of the organisms were resistant to commonly used antibiotics and none were sensitive to ampicillin. The mortality was 33.3%. Conclusions: ABM remains a common illness in developing countries contributing to high mortality. The microorganisms show high resistance to commonly used antibiotics used in the treatment of ABM.