Therapeutic Trial of Vigabatrin in Refractory Infantile Spasms

Abstract

Infantile spasms usually start during the first year of life and constitute one of the most difficult types of epilepsy to treat. They carry a very poor prognosis for both epilepsy and mental development. Seventy children, including 47 infants, with intractable infantile spasms were entered into an open study with vigabatrin as add-on therapy to the usual anticonvulsant treatment. All were resistant to previous treatments, including corticosteroids (43 patients), carbamazepine, benzodiazepines, and sodium valproate. Two children withdrew from the study because of intolerance to vigabatrin (hypotonia or hypertonia) before evaluation of efficacy could be made. Of the remaining 68 children, 29 (43%) showed complete suppression of spasms. Forty-six children had a greater than 50% reduction in spasms. The best response was observed in those with tuberous sclerosis (12/14 compared with 12/18 with symptomatic infantile spasms of other origin and 22/36 with cryptogenic infantile spasms). Following the initial response to treatment of these patients (n = 68), a long-term response was confirmed in 75% of children with symptomatic infantile spasms and 36% of children with cryptogenic infantile spasms. In eight children, all other anticonvulsant medication could be definitively withdrawn. Tolerability appeared excellent, with 52 of 70 patients reporting no side effects. Somnolence, hypotonia, weight gain, excitation, and insomnia were the most common problems at the beginning of the study and were usually transient. Given the poor prognosis of this type of childhood epilepsy, vigabatrin appears to be a very interesting advance in the management of drug-resistant infantile spasms. (J Child Neurol 1991;6(Suppl):2S52-2S59).

A Pilot Study of Topiramate in the Treatment of Infantile Spasms

“…In an open-label, add-on trial of VGB in a similar population of children with refractory infantile spasms, 43% became spasm free (24). In our study, TPM had similar spasm-free efficacy results compared with VGB in like refractory populations (45% TPM vs.…”

“…Vigabatrin appears to be effective in the management of infantile spasms, particularly in patients with tuberous sclerosis (23,24). In an open-label, add-on trial of VGB in a similar population of children with refractory infantile spasms, 43% became spasm free (24).…”

Vigabatrin

“…[22][23][24][25][26] Results from European randomized, controlled, placebo and active trials, open-label prospective studies, and retrospective reviews suggest that vigabatrin is safe and has effectiveness in treating subjects with IS. [27][28][29][30][31] One study of vigabatrin treatment in IS provided substantial evidence of its effectiveness (70% spasm-free) with greater response rates (85% spasm-free) in patients with tuberous sclerosis for periods up to 33 months. 17 The United Kingdom Infantile Spasms Study evaluated the effects of vigabatrin versus hormone treatment on seizure control and neurodevelopment in 101 infants followed for 1 year.…”

Espasmos infantis: experiência em treze casos

“…Em estudo publicado em 1998, Masatoshi 8 observou baixa concentração do GABA no líquor de pacientes apresentando EI. Baseados nisto e no comprovado efeito da vigabatrina nos casos de EI secundários a esclerose tuberosa 9 em que, sem dúvida é a droga de primeira escolha, existem autores que defendem este tratamento de rotina em todos os casos 10,11 . Em nossos casos usamos apenas nas recorrências, confirmando sua eficácia, principalmente na manutenção das crises a longo prazo.…”