The clinical profile and outcome of children with West syndrome in KwaZulu-Natal Province, South Africa: A 10-year retrospective review

Abstract

Background . West syndrome (WS) is a rare epileptic encephalopathy of infancy. There is currently no research on the incidence or prevalence of WS in Africa. Methods . We aimed to describe the outcome of children with WS at a quaternary-level hospital in KwaZulu-Natal, South Africa (SA). This was a retrospective chart review conducted on patients diagnosed with WS over a 10-year period. Eight children (males, n =7; African, n =6; Asian, n =2) identified with WS out of 2 206 admitted with epilepsy. The median age (range) at diagnosis was 7.5 (1 - 9) months. The average time between onset of epileptic spasms and diagnosis was 3.1 months. Results . Six patients had abnormal neuroimaging (atrophy ( n =2); corpus callosum agenesis ( n =2); tuberous sclerosis ( n =1); focal dysplasia ( n =1)). Drug management included sodium valproate ( n =8), topiramate ( n =7) and levetiracetam ( n =3). Subsequent definitive treatment was intramuscular adrenocorticotrophic hormone ( n =3), vigabatrin ( n =2) and oral prednisone ( n =4). Four (50%) patients had complete seizure remission (neuromigratory disorder ( n =2); tuberous sclerosis ( n =1); and idiopathic ( n =1)) and 4 had partial remission (neonatal complications ( n =3); idiopathic ( n =1)). Discussion. Most of our patients had symptomatic WS, with 50% remission on treatment. Outcomes were poorer in our study when compared with those in published data. Conclusion . Further collaborative studies are still needed to evaluate the true impact and prevalence of WS in SA.