Lennox-Gastaut综合征的药物治疗进展

摘要

Lennox-Gastaut syndrome ( LGS) is an globally recognized refractory epilepsy,also known as an age-dependent epileptic encephalopathy. It is characterized by multiple seizure types, mental retardation, and slow spike-wave complexes discharges or fast rhythmic bursts during sleep on electroencephalographic re-cordings. Many antiepileptic drugs were reported for the treatment of LGS,but it was difficult to compare their efficacy because of the small sample size and design limitations. Valproate,topiramate and lamotrigine are rec-ommended to be the first-line drugs by most guidelines or expert consensus,and the combination of valproate and lamotrigine or the combination of lamotrigine and topiramate should be preferred as early as possible for initial treatment of LGS in children. Recent progresses of medical treatment of LGS are reviewed and some optical re-gimes are proposed in this paper.%Lennox-Gastaut综合征是一种国际公认的难治性癫疒间，也是一种年龄依赖性癫疒间性脑病。以多种发作形式并存、精神发育迟滞和脑电图的表现以发作间期慢棘慢波和睡眠期快波暴发为特征。用于Lennox-Gastaut综合征的药物很多，但由于病例数少及缺乏严格的设计，难以判断其疗效。多数指南或专家共识推荐丙戊酸、托吡酯和拉莫三嗪为治疗 Lennox-Gastaut 综合征的一线药物，对新诊断的 Lennox-Gastaut综合征推荐首先应用丙戊酸+拉莫三嗪或拉莫三嗪+托吡酯两种药物联合治疗。该文就近年来Lennox-Gastaut综合征的药物治疗进展进行综述，并提出药物治疗方案的选择。