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首页> 外文期刊>The Canadian Journal of Neurological Sciences: le Journal Canadien des Sciences Neurologiques >Treatment of Dravet Syndrome
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Treatment of Dravet Syndrome

机译：德拉韦综合征的治疗

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摘要

Dravet syndrome is among the most challenging electroclinical syndromes. There is a high likelihood of recurrent status epilepticus; seizures are medically refractory; and patients have multiple co-morbidities, including intellectual disability, behaviour and sleep problems, and crouch gait. Additionally, they are at significant risk of sudden unexplained death. This review will focus predominantly on the prophylactic medical management of seizures, addressing both first-line therapies (valproate and clobazam) as well as second-line (stiripentol, topiramate, ketogenic diet) or later options (levetiracetam, bromides, vagus nerve stimulation). Sodium channel agents-including carbamazepine, oxcarbazepine, phenytoin and lamotrigine-should be avoided, as they typically exacerbate seizures. Several agents in development may show promise, specifically fenfluramine and cannabidiol, but they need further evaluation in randomized, controlled trials. In addition to prophylactic treatment, all patients need home-rescue medication and a status epilepticus protocol that can be carried out in their local hospital. Families must be counselled on non-pharmacologic strategies to reduce seizure risk, including avoidance of triggers that commonly induce seizures (including hyperthermia, flashing lights and patterns). In addition to addressing seizures, holistic care for a patient with Dravet syndrome must involve a multidisciplinary team that includes specialists in physical, occupational and speech therapy, neuropsychology, social work and physical medicine.
机译：Dravet综合征是最具挑战性的电子临床综合征之一。复发性癫痫持续状态的可能性很高;癫痫发作是医学上难治的;并且患者有多种合并症，包括智力残疾，行为和睡眠问题以及蹲伏步态。此外，他们处于无法解释的突然死亡的巨大风险中。这篇综述将主要集中在癫痫的预防性医学管理上，涉及一线治疗（丙戊酸和氯巴沙姆）以及二线治疗（斯立喷妥，托吡酯，生酮饮食）或以后的治疗方案（左乙拉西坦，溴化物，迷走神经刺激） 。应避免使用钠通道剂，包括卡马西平，奥卡西平，苯妥英钠和拉莫三嗪，因为它们通常会加剧癫痫发作。几种开发中的药物可能显示出希望，特别是芬氟拉明和大麻二酚，但它们需要在随机对照试验中进行进一步评估。除了预防性治疗之外，所有患者都需要家庭自救药物和可以在当地医院进行的癫痫持续状态治疗方案。必须为家庭提供非药物治疗策略的咨询，以减少癫痫发作的风险，包括避免通常诱发癫痫发作的触发因素（包括体温过高，闪光灯和模式改变）。除了解决癫痫发作以外，对Dravet综合征患者的整体护理还必须涉及一个多学科团队，其中包括物理，职业和言语治疗，神经心理学，社会工作和物理医学方面的专家。

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