2016
DOI: 10.1007/s40272-016-0171-7
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Pharmacotherapy for Dravet Syndrome

Abstract: Dravet syndrome (DS) is an intractable pediatric epilepsy syndrome, starting in early childhood. This disorder typically manifests with febrile status epilepticus, and progresses to a multifocal epilepsy with febrile and non-febrile seizures with encephalopathy. Most cases are due to a mutation in the SCN1A gene. This article reviews treatments for DS, with an emphasis on pharmacotherapy. While many medications are used in treating the seizures associated with DS, these patients typically have medically refrac… Show more

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Cited by 65 publications
(42 citation statements)
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“…). This analysis is consistent with what has been reported in the literature from other epilepsy centers …”
Section: Resultssupporting
confidence: 93%
See 1 more Smart Citation
“…). This analysis is consistent with what has been reported in the literature from other epilepsy centers …”
Section: Resultssupporting
confidence: 93%
“…Dravet syndrome also includes developmental delays, cognitive impairment, motor dysfunction, and an increased risk for sudden unexpected death in epilepsy (SUDEP) . Typical pharmacotherapies include benzodiazepines, valproic acid, and stiripentol, but response is usually suboptimal . Lamotrigine and carbamazepine, both sodium channel inhibitors, are not recommended for Dravet syndrome as they have been reported to exacerbate seizures …”
Section: Introductionmentioning
confidence: 99%
“…[26][27][28] CBD has been shown to attenuate seizures in a mouse model of Dravet syndrome and reduce seizure frequency and severity in various acute seizure models. [26][27][28] CBD has been shown to attenuate seizures in a mouse model of Dravet syndrome and reduce seizure frequency and severity in various acute seizure models.…”
Section: Discussionmentioning
confidence: 99%
“…There is an urgent need to introduce better treatment options for intractable epilepsies, particularly for Dravet syndrome, since less than 30% of patients respond to first-line treatments. [26][27][28] CBD has been shown to attenuate seizures in a mouse model of Dravet syndrome and reduce seizure frequency and severity in various acute seizure models. [29][30][31][32][33] Following several successful Phase III trials, CBD has been approved by the FDA and offers a novel therapeutic option for patients with Dravet syndrome.…”
Section: Discussionmentioning
confidence: 99%
“…По мнению A. Wallace и соавт. (2016), препаратами первого выбора в лечении син-дрома Драве следует считать вальпроаты и клобазам (не зарегистрирован в РФ), хотя также могут быть эффективны топирамат, леветирацетам, стирипентол (не зарегистрирован в РФ) в комбинированной те-рапии и кетогенная диета [22]. В соответствии с от-четом рабочей группы комиссии ILAE по педиатрии (2015) высокий доказательный уровень эффективно-сти при синдроме Драве имеет стирипентол (в соче-тании с вальпроатами и клобазамом); топирамат, зонисамид, вальпроат, бромиды и кетогенная диета «возможно эффективны» на низком доказательном уровне.…”
Section: Ch I Ld Neurology R U S S I a N J O U R N A L O Funclassified