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Vigabatrin as a First-Choice Drug in the Treatment of West Syndrome

Hospital J.P. Garrahan

Ricardo Cersósimo, MD

Hospital J.P. Garrahan

Roberto Caraballo, MD

Hospital J.P. Garrahan

Jorge Grippo, MD

Hospital R. Gutierrez

Susana Corral, MD

Hospital R. Gutierrez

Raúl H. Martino, MD

Hospital P de Elizalde Buenos Aires

Gabriel Martino, MD

Hospital P de Elizalde Buenos Aires

Maria Aldao, MD

Hospital V.J. Vilela

Pedro Caccia, MD

Hospital V.J. Vilela

Mirta Retamero, MD

Hospital V.J. Vilela

Maria Cristina Macat, MD

Hospital E. Perón, G. Baigorria Rosario

Marcelo A. Di Blasi, MD

Centro Neurológico Bariloche

J. Adi, MD

Hospital H. Notti Mendoza, Argentina

This is a prospective study designed to evaluate the efficacy and safety of vigabatrin as first-choice monotherapy in infants with West syndrome. One hundred sixteen patients with newly diagnosed West syndrome were studied in Argentina, from June 1994 to April 1998. The follow-up ranged from 17 to 40 months (mean, 23 months). Vigabatrin was administered upon diagnosis, starting with a 50-mg/kg/day dose and increasing 50 mg/kg every 48 hours to reach a maximum dose of 200 mg/kg/day. Twenty-nine percent of cases were considered to be cryptogenic or idiopathic West syndrome, while 70.7% were symptomatic. Response to vigabatrin treatment was measured according to five categories: (1) seizures free: 61.8% of cases for cryptogenic and 29.3% for symptomatic West syndrome, (2) more than 75% reduction in the number of infantile spasms: 14.7% for cryptogenic and 26.8% for symptomatic West syndrome, (3) from 50% to 74% reduction in the number of infantile spasms: 11.8% for cryptogenic and 24.4% for symptomatic West syndrome, (4) poor or null response: 11.8% for cryptogenic and 18.3% for symptomatic West syndrome, and (5) increase in the number of infantile spasms: one symptomatic case (1.2%). All seizure-free cryptogenic cases showed normal neuropsychic development. The most effective dose of vigabatrin was 150 mg/kg of body weight per day. The most frequent adverse events were somnolence in 19 cases and irritability in 15 cases, but none required treatment interruption. (J Child Neurol 2000;15:161-165).

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