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Dravet综合征(DS)是儿童期起病的一种癫痫性脑病,早期称为婴儿严重肌阵挛性癫痫,发病率约为1/20 000~1/4 000[1]。DS电临床特点包括特定起病年龄、发作类型多样及演变、脑电图(EEG)特点、特有的生长发育史及基因突变位点。DS可分为典型和非典型两种类型,非典型DS是指患儿符合DS临床特点但缺乏肌阵挛发作和不典型失神发作,约占25%。目前关于双胎DS,国内尚无文献报道,报告如下。
Dravet syndrome (DS) is a childhood onset of epileptic encephalopathy, earlier known as infants with severe myoclonic epilepsy, the incidence of about 1/20 000 to 1/4 000 [1]. DS clinical features include specific age of onset, seizure types and evolution, EEG features, unique growth and development and gene mutation sites. DS can be divided into two types of typical and atypical, atypical DS refers to the clinical features of children with DS but the lack of myoclonic seizures and atypical absences, accounting for about 25%. Currently on twins DS, there is no domestic literature, the report is as follows.