儿童睡眠中癫(痫)性电持续状态相关综合征及治疗.pdfVIP

国际儿科学杂志 2014 年 11 月第 41 卷第 6 期 Int J Pedi眩， Nov 2014 ,Vol. 41 ,NQ._(j .629 . ·综述· 儿童睡眠中癫病性电持续状态相关综合征及治疗 赵小佩(综述) 陆燕芬(审校) [摘要] 癫瘸性电持续状态是一种特殊的脑电现象，与之相关的综合征包括获得性癫病性失语、不典 型良性部分性癫病、癫病性脑病伴慢被睡眠期持续棘慢波。这些综合征代表着一个临床系谱，这个系谱疾 病拥有一些共同的特征。虽然癫瘸性电持续状态是自限性疾病，但是早期有效的治疗能改善电持续状态 从而改善神经心理预后。目前仍没有统一的推荐治疗方案，临床上常见的治疗方案包括抗癫瘸药物治疗、 激素治疗、静脉丙种球蛋白、生酣饮食、手术治疗等，但疗效不一。急需开展更多???多中心临床试验的开展 以获得最佳的治疗方案。 [关键词] 儿童;癫瘸;电持续状态;脑病 ClinicaI syndromes and treatment of children with electrical sta饥1S eplleptic皿 in sleep ZJWO Xiaopei , Lu Ym庐 n. Department 01 Neurology , Shanghai Children s Hospital Al声 liated to Shanghai Jiaotong Univers仰， shanghai2αχ162 ， China [ Abstract 1 Elec创cal status epilepticus in sleep ( ESES ) is a specific 出ctroencephalographic pattern, clin? ical syndromes related to ESES including Landau- Kl e伍ler syndrome , atypical benign p征tial epilepsy , epileptic encephalopathy with continuous spike waves during sleep. These disorders represent a clinical spectrum with sev? eral common features. Al由ough ESES is a self-limited disorder , but early treatrnent with effective therapy to ameliorade the ESES pattern is essential for improvement in neuropsychological outcome. There is no unified treatrnent plan recommended now. Common clinical treatrnent options include antiepileptic drugs , hormone thera? py ,intravenous immunoglobulin , ketogenic diet , surgical treatrnent , but the curative effect varies. More multi? cen臼:r studies are needed to fmd the most proper 田温trnent. [ Key words 1 Children ; Epilepsy ; Electrical status epilepticus; Ence庐山p础y 1971 年 Pa町等首先描述了 6 例伴随癫捕和认知 (即放电指数 SWI;;-;85% )作为诊断标准[町，但之后 障碍的患儿，脑电图纪录表明这些患儿在非快动眼睡 大量研究显示，各种不同 SWI 域值 (259毛- 1 ∞%) 眠期( non-rapid eye movem