West syndrome and other infantile epileptic encephalopathies - Indian hospital experience

Children with infantile epileptic encephalopathies, comprising 3.5% of the Pediatric Neurology Clinic registrations in a tertiary care hospital were r etrospectively analyzed. Data were retrieved from case records and analyzed for seizure semiology, prenatal and perinatal insults, developmental statu s and relevant investigations. The various therapeutic modalities and their influence on spasm frequency, longterm development and final seizure statu s were compared. The two primary outcome variables analyzed included final seizure status and developmental outcome. Of the 94 infantile epileptic enc ephalopathies, West syndrome was the commonest (55.3%), of which two thirds were symptomatic. Etiological factors were prenatal in 66.6% and perinatal in 33.3%. The initial response to ACTH was good in 54.5% with subsequent r elapse in 27.8% and for prednisolone was 52.9 and 44.4%, respectively, comp ared to 25.3% spasms control with conventional antiepileptic drugs. Disease category of infantile epileptic encephalopathies evolved in 4, i.e. early myoclonic encephalopathy to West syndrome 1, early infantile epileptic ence phalopathy to West syndrome 1, West syndrome to Lennox-Gastaut syndrome 2. Psychomotor retardation was seen in 88.2%, with 16.1% having normal develop ment at onset of spasms. Microcephaly was associated with delayed developme nt but did not influence final seizure outcome. Final seizure outcome was p oor in children with delayed development at onset (adjusted odds ratio (OR) = 4), delay in diagnosis >12 months (OR = 2.27) and in children with Lenno x-Gastaut syndrome (OR = 4.75). ACTH/prednisolone and antiepileptic treatme nt versus antiepileptics alone showed a good final seizure response in 36.6 % versus 20%. Development on follow up was delayed in children with initial psychomotor retardation (OR = 23.4) and abnormal electroencephalogram (OR = 7.46). Perinatal factors constituted one third of symptomatic West syndro me. The use of ACTH/corticosteroids resulted in good initial spasm control though final seizure outcome and development were unaffected. Prednisolone had similar response to ACTH in spasm control but higher subsequent relapse rate. Vigabatrin was useful though often unaffordable. The identification of a neurometabolic etiology, though uncommon, has significant therapy impl ications. Delay in diagnosis was common and negatively influenced final sei zure outcome. (C) 2001 Published by Elsevier Science B.V.