| Current therapeutic procedures in Dravet syndrome. | |
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MedLine Citation:
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PMID: 21504427 Owner: NLM Status: In-Process |
Abstract/OtherAbstract:
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Dravet syndrome is a highly pharmaco-resistant form of epilepsy. Valproate and benzodiazepines are the first-line treatment but are usually insufficient therapeutic options. Lamotrigine, carbamazepine and high doses of intravenous phenobarbital can aggravate seizures and should be avoided. Topiramate, levetiracetam, bromide and ketogenic diet also provide substantial efficacy as adjunctive therapy and procedures. Stiripentol is the only new drug to demonstrate efficacy when combined with valproate and clobazam, as shown in two independent double-blind controlled trials dedicated to Dravet children. In order to avoid side effects (mainly loss of appetite and loss of weight) resulting from the inhibition of cytochromes P450 by stiripentol, the prescribed doses of valproate and clobazam should be reduced. Stiripentol has a proper antiepileptic effect and enhances GABAergic neurotransmission by acting on the alpha-3 subunit of GABA(A) receptors. Stiripentol was approved as an orphan drug in Europe in 2007 for adjunctive therapy in Dravet syndrome. More than 500 Dravet patients have currently been satisfactorily treated and recent experiments in Japan have confirmed stiripentol's benefit. In practice, valproate should be initiated at the first onset of complicated febrile seizure in Dravet patients. Relapses justify the addition of clobazam and stiripentol when available. Topiramate and a ketogenic diet are alternatives in pharmaco-resistant cases. |
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Authors:
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Catherine Chiron |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Developmental medicine and child neurology Volume: 53 Suppl 2 ISSN: 1469-8749 ISO Abbreviation: Dev Med Child Neurol Publication Date: 2011 Apr |
Date Detail:
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Created Date: 2011-04-20 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0006761 Medline TA: Dev Med Child Neurol Country: England |
Other Details:
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Languages: eng Pagination: 16-8 Citation Subset: IM |
Copyright Information:
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© 2011 The Author. Developmental Medicine & Child Neurology © 2011 Mac Keith Press. |
Affiliation:
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Inserm, U663, Paris, Université Paris Descartes, Paris, France. catherine.chiron2@gmail.com |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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