West syndrome: a university hospital based study from Oman

Abstract

Forty-four children (20 male: 24 female) with West syndrome (infantile spasms, mental retardation/regression and hypsarrhythmia) diagnosed at Sultan Qaboos University Hospital (Pediatric Neurology Division of the Department of Child Health) are reported, with thirty-four (77.3%) children constituting the symptomatic group. All children were followed up for an initial 1 year 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). Developmental 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 antiepileptic drugs were also used. Nine (24.5%) children achieved good seizure control, out of which five have normal development. Only one child could be weaned off antiepileptic drugs completely. There was one death in the whole series, related to aspiration pneumonia.

Introduction

A triad of infantile spasms, mental retardation/regression and hypsarrhythmia constitute West syndrome (WS). This condition is named after Dr W.J. West, who in a letter to the editor of ‘The Lancet’ in 1841, described the typical condition affecting his 4-month-old son [1]. About 100 years later, Gibbs and Gibbs [2] described the chaotic EEG pattern as hypsarrhythmia, which remains the gold standard in the diagnosis of WS. The clinical and EEG characteristics of WS are determined by the type and topography of the underlying pathology [3]. The two types of WS are: symptomatic, which forms two-thirds of all cases; and cryptogenic [4]. The overall prognosis continues to be poor in the majority of cases, however, the cryptogenic type has been postulated to have a better outcome. The literature on WS from the Gulf region is limited [5]. We report a series of 44 cases, with complete follow-up in 37 (84%).