|[Stiripentol for the treatment of severe myoclonic epilepsy in infants (dravet’s syndrome)]|
|Mengarelli C, Bardach A, Pichon-Riviere A, Augustovski F, García Martí S, Alcaraz A, Ciapponi A, López A, Rey-Ares L|
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Mengarelli C, Bardach A, Pichon-Riviere A, Augustovski F, García Martí S, Alcaraz A, Ciapponi A, López A, Rey-Ares L. [Stiripentol for the treatment of severe myoclonic epilepsy in infants (dravet’s syndrome)] Buenos Aires: Institute for Clinical Effectiveness and Health Policy (IECS). Documentos de Evaluación de Tecnologías Sanitarias, Informe de Respuesta Rapida No 522. 2017
There is low quality evidence from two small randomized clinical trials suggesting that stiripentol might reduce the rate of seizures in severe myoclonic epilepsy in infants (Dravet’s syndrome). There is no evidence showing improvement in cognitive impairment or quality of life in this group of patients at long term. This is a drug classified as “orphan drug”, only approved in some European countries and Canada as adjuvant therapy in combination with clobazam and valproic acid for refractory generalized tonic-clonic seizures in patients with severe myoclonic epilepsy in infants (Dravet’s syndrome) whose seizures are not adequately controlled with clobazam and valproic acid. The clinical practice guidelines and the health sponsors from Europe and Canada suggest adding stiripentol for patients with Dravet’s syndrome whose treatment with valproic acid, clobazam or topiramate has not been successful.
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Subject indexing assigned by CRD
Dioxolanes; Epilepsies, Myoclonic; Humans; Infant
Country of organisation
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Address for correspondence
Institute for Clinical Effectiveness and Health Policy, Viamonte 2146 – 3 Piso, C1056ABH Ciudad de Buenos Aires, Argentina Tel: +54 11 49 66 00 82 Fax:+54 11 49 53 40 58 Email: email@example.com
Date abstract record published