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

Abstract

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.

Síndrome de West, experiencia con una serie de casos con acceso al tratamiento de primera línea, en Lima

“…La eficiencia del tratamiento se encontró entre los límites reportados en la literatura (66,7%) (15)(16)(17)(18)(19)(20)(21)(22)(23)(24)34). Para iniciar oportunamente el tratamiento, los padres consiguieron los medicamentos de primera línea de otras familias con niños enfermos de SW o importándolos directamente del extranjero con el permiso de las autoridades, ya que no se encuentran en el petitorio nacional.…”

“…Podemos afirmar, con la experiencia recogida, que la vigabatrina y el ACTH fueron los medicamentos más eficaces para controlar los espasmos, tal como se reporta en la literatura (15)(16)(17)(18)(19)(20)(21)(22)(23)(24). Fue interesante observar el efecto benéfico de levetiracetam para dos casos de niños prematuros con SW por leucomalacia periventricular y del topiramato para un niño con SW criptogenico.…”

“…Para Esclerosis Tuberosa es conocida la indicación desde hace varios años (15,20,(21)(22)(23)(24) sin efectos adversos. Para el grupo de enfermedades perinatales, post infecciosas, idiopáticas y criptogénicas, la experiencia con ACTH ha sido mejor que con vigabatrina, lo cual haría suponer en un posible mecanismo inflamatorio, que esperamos se investigue en el futuro.…”

“…No existe tratamiento específico para el SW; sin embargo, el ACTH y la Vigabatrina son las drogas que han dado los mejores resultados (15)(16)(17)(18)(19)(20)(21)(22)(23)(24)34). Lamentablemente estos medicamentos no se encuentran en el petitorio nacional.…”

Epileptic Syndromes With Possible Immunological Mechanisms (Rasmussen Encephalitis, FIRES, West Syndrome, Landau-Kleffner Syndrome)

“…[90] Semptomatik hastalarda %0-50, kriptojenik hastalarda %50-100 oranında etkin bulunmuştur. [91] Valproik asit, vigabatrinden sonra, ACTH ile birlikte kullanılması ve ACTH sonrası tedavinin devamında uygun bir ajandır. Yapılan birkaç çalışmada 25-100 mg/kg/gün dozlarında spazmların %73, hipsaritminin de %91 kaybolmasını sağladığı bildirilmiştir.…”