West syndrome: a university hospital based study from Oman

Forty-four children (20 male: 24 female) with West syndrome (infantile spas ms, mental retardation/regression and hypsarrhythmia) diagnosed at Sultan Q aboos University Hospital (Pediatric Neurology Division of the Department o f Child Health) are reported, with thirty-four (77.3%) children constitutin g the symptomatic group. All children were followed up for an initial 1 yea r at this hospital. Thirty-seven cases (84%) still continue their follow-up with us. The age of onset ranged from 1 to 14 months (mean, 6.0 months). D evelopmental delay before the onset of infantile spasms was noted in 29 (65 .9%) children. Brain computed tomography was abnormal in 29 (65.9%). Sodium valproate and vigabatrin were the most often used drugs, though other anti epileptic drugs were also used. Nine (24.5%) children achieved good seizure control, out of which five have normal development. Only one child could b e weaned off antiepileptic drugs completely. There was one death in the who le series, related to aspiration pneumonia. (C) 2001 Elsevier Science B.V. All rights reserved.