National survey of West syndrome in Taiwan.

In the fall of 2000, the Taiwan Child Neurology Society performed a retrospective survey of West syndrome that occurred in 1998 and 1999 in Taiwan. Questionnaires were sent to the child neurologists in 15 major teaching hospitals or medical centers throughout Taiwan. There were totally 41 cases in these 2 years. Among these 41 cases, 35 had complete data and were enrolled for analysis. The male to female ratio was 1:1.19. The mean age of onset was 6.0+/-4.9 months old. Twenty nine percent were classified as cryptogenic and 71% were symptomatic. Adrenocorticotropic hormone (ACTH) was given to 40% of these patients. The initial response rate was 78.6% and the recurrence rate was 36.4%. For those patients not receiving ACTH, vigabatrin and valproic acid were two of the most common choices. At the final visit, 37% patients remained seizure-free, 29% were still suffering from the same seizure, and 34% changed to other seizure types. The final developmental status was normal in 14%, mildly retarded in 20%, and moderate to severely retarded in 66% of the patients.