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Lennox-Gastaut Syndrome: An Update on Treatment

Published online by Cambridge University Press:  02 December 2014

Lionel Carmant
Université de Montréal, Hôpital Sainte-Justine, Montreal, Quebec
Sharon Whiting
Faculty of Medicine, University of Ottawa, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada


Lennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy. Few current treatment options are effective in improving seizure control. This paper reviews the available treatments of LGS and discusses a new option in Canada, rufinamide. It is a wide spectrum anticonvulsant, approved in a number of countries for the treatment of LGS. In a randomized controlled trial in the LGS population, adjunctive rufinamide therapy has been shown to offer significantly greater reduction in total seizure frequency and tonic-atonic seizure frequency in comparison to placebo. Efficacy has been assessed over three years and appears to be sustained. Most adverse events were cognitive (e.g. somnolence) or gastrointestinal in nature and in many cases transient or mild. Based on the efficacy and safety data on rufinamide obtained to date, this medication will provide additional benefits to patients with LGS in Canada and is an important consideration for our patients in the adjunctive treatment setting.



Le syndrome de Lennox-Gastaut (SLG) est une encéphalopathie épileptique sévère. Peu des options thérapeutiques actuelles sont efficaces pour améliorer le contrôle des crises. Dans cet article, nous revoyons les traitements du SLG qui sont disponibles et nous discutons d'une nouvelle option thérapeutique disponible au Canada, le rufmamide. Il s'agit d'un anticonvulsivant à large spectre qui a été approuvé dans certains pays pour traiter le SLG. Un essai clinique randomisé chez des patients atteints du SLG a démontré qu'un traitement d'appoint par le rufmamide entraînait une diminution significativement supérieure de la fréquence totale des crises et de la fréquence des crises toniques-atoniques comparé au placebo. Son efficacité a été évaluée pendant 3 ans et semble persister. La plupart des incidents thérapeutiques étaient de nature cognitive (e.g. la somnolence) ou gastrointestinaux et ils étaient transitoires ou légers chez plusieurs patients. Les données actuelles sur l'efficacité et la sécurité du rufmamide indiquent que cette médication apportera des bénéfices additionnels aux patients atteints de SLG au Canada et constitue un traitement d'appoint intéressant à considérer chez nos patients.

Review Article
Copyright © The Canadian Journal of Neurological 2012


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