VIGABATRIN AS A FIRST-LINE DRUG IN WEST-SYNDROME - CLINICAL AND ELECTROENCEPHALOGRAPHIC OUTCOME

The cessation of infantile spasms and the disappearance of hypsarrhyth mia in sleep EEC are the criteria for successful treatment in West syn drome. In a prospective study at the Children's University Hospital, 2 8 children with West syndrome (17 symptomatic, 7 cryptogenic, 4 idiopa thic) were treated with vigabatrin monotherapy for at least 2 weeks. S even children received vigabatrin 150 mg/kg/day, and 21 children recei ved 65-75 mg/kg/day. Patients were classified as responders, when infa ntile spasms and hypsarrhythmia in sleep EEC disappeared within 2 week s. After 2 weeks, 18 patients were seizure-free. 14 without hypsarrhyt hmia. Fourteen children were responders (8 symptomatic, 3 cryptogenic, 3 idiopathic), and 14 nonresponders. The follow-up encompassed 6 mont hs to 5.3 years (mean 20.3 months). in the responder group, 12/14 pati ents (6 symptomatic, 3 cryptogenic and 3 idiopathic) remained seizure- free, no relapse of West syndrome occurred. In the non-responder group , ACTH was efficient in 11, valproic acid in 2 children and clonazepam in 1 child. Relapse occurred in 4 children after discontinuation of A CTH. At last visit EEG was normal in 8/14 responders and 1/14 non-resp onders. The efficacy of vigabatrin monotherapy was comparable to ACTH and occurred within 2 weeks.