[Stiripentol for the treatment of severe myoclonic epilepsy in infants (dravet's syndrome)]


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[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
 
 

Record Status

This is a bibliographic record of a published health technology assessment from a member of INAHTA. No evaluation of the quality of this assessment has been made for the HTA database.

Citation

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

Authors’ conclusions

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.

Final publication URL

www.iecs.org.ar

Indexing Status

Subject indexing assigned by CRD

MeSH

Dioxolanes; Epilepsies, Myoclonic; Humans; Infant

Language Published

Spanish

Country of organisation

Argentina

English summary

There is no English language summary available.

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: info@iecs.org.ar

AccessionNumber

32017000287

Date abstract record published

13/06/2017