P-EP026. Clinical profile of children with west syndrome: A retrospective chart review

Abstract

Introduction. This study was intended to document the clinical profile and treatment outcome of West syndrome in children attending a tertiary care center in Northern India. Methods. Data were collected by a retrospective chart review of children diagnosed with West syndrome between January 2017 to January 2018. Information was recorded pertaining to the age at onset and presentation, etiology, and associated co-morbidities; results of electroencephalography (EEG) and neuroimaging; treatment given; and final outcome. The following drugs were used for treatment: ACTH (n=7), prednisolone (n=17), vigabatrin (n=25), sodium valproate (n=28), clonazepam (n=30), and levetiracetam (n=13) and modified Atkins diet (n=7). The response was categorized as spasm cessation, partial improvement (>50% improvement), or no improvement. Results. Records of 30 children (21 boys) were analyzed. The mean (SD) age at onset was 4 (3, 6.5) months. The median lag time to treatment was 5 (2,14) months. Eight (26%) were premature, 2 (7%) were SGA, birth asphyxia in 56%, neonatal encephalopathy in 62%. EEG findings were compatible with hypsarrhythmia in 13 (43.3%) children and 9 (30%) had modified hypsarrythmia. MRI finding was periventricular leucomalacia (54.1%), cystic encephalomalacia (13.8%), normal MRI (20.7%) and 1 had arrested hydrocephalus. There was no improvement with valproate (93%), clonazepam (89%) and levetiracetam (78%). Cessation of spasm was achieved with vigabatrin (28 %), prednisolone (38.2%) and ACTH (42.8%). Hypsarrhythmia resolved with presence of background and other epileptiform abnormalities in 34 children. Conclusion. The present research highlights limited role of sodium valporate, levetiracetam and clonazepam in initial management of children with West syndrome. However, further studies with larger sample size are required before concluding its limited role.