National survey on West syndrome in Korea

To investigate and analyze the current epidemiological and clinical data for West syndrome (WS) in Korea, including treatments and outcome.

Prepared survey forms were sent to hospitals located all over Korea to collect data on cases of WS treated between January 1997 and June 2000. Data from 21 institutes were returned.

Data were collected for 358 children (219 boys and 139 girls) who were diagnosed and treated for WS. The incidences of WS were 112 cases in 1997, 104 cases in 1998, 108 cases in 1999, and 34 cases in the first half of 2000. Based on these figures, the presumed annual incidence of WS in Korea was derived to be one in 4000 live births, although there was a great inter-district variability. Regarding the etiology, 138 cases (38.5%) were cryptogenic. For the 220 symptomatic cases, hypoxic-ischemic encephalopathy (HIE) was the most common etiologic factor (50.8%), followed by central nervous system anomaly including malformation of cortical development (21.4%), and neurocutaneous syndrome (8.6%). Clinically flexor type spasms were most common (59.2%) and 199 cases (55.6%) revealed typical hypsarrhythmia, electroencephalographically. During 1997 and 1998, vigabatrin (VGB) was used most frequently for the treatment of WS, followed by valproic acid and adrenocorticotropic hormone (ACTH). In 1999 and 2000, the use of ACTH diminished and topiramate was used more frequently than ACTH. Spasms were controlled in 230 cases (64.2%) and evolution to other seizure types was observed in 44 cases (11.2%). Developmental and/or mental retardation were noticed in 270 cases (75.4%).

The yearly incidence of WS did not show significant change in Korea between 1997 and 2000. Among symptomatic WS, HIE was the most common cause. During the period of this study, VGB was the most popular therapeutic regimen possibly because of shortage of ACTH. Recently, topiramate has showed promising signs as a therapy for WS.