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浙江大学学报（医学版）
浙江大学学报（医学版）  2020, Vol. 49 Issue (4): 425-430    DOI: 10.3785/j.issn.1008-9292.2020.08.04
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睡眠相关过度运动性癫痫的研究进展
徐佳慧1(),金搏2,张力三1,王爽3,*()
1. 浙江大学医学院附属邵逸夫医院神经内科, 浙江 杭州 310016
2. 浙江省人民医院神经内科, 浙江 杭州 310014
3. 浙江大学医学院附属第二医院神经内科, 浙江 杭州 310009
Advances in sleep-related hypermotor epilepsy
XU Jiahui1(),JIN Bo2,ZHANG Lisan1,WANG Shuang3,*()
1. Department of Neurology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou 310016, China
2. Department of Neurology, Zhejiang Provincial People's Hospital, Hangzhou 310014, China
3. Department of Neurology, the Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310009, China
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摘要：

睡眠相关过度运动性癫痫（SHE）的主要临床表现为睡眠中发生的不对称强直或复杂的过度运动发作，且具有短暂、频繁、丛集发作的特点，其发病率约为1.8/100 000。由于SHE在睡眠中多发，易误诊为异态睡眠，临床上应注意鉴别。绝大多数SHE患者病因不明，可能的病因包括基因突变及结构异常，不同的病因可影响患者疾病严重程度及预后。SHE的治疗主要包括药物治疗和手术治疗，其中药物治疗首选卡马西平，经选择的难治性癫痫患者手术治疗预后良好。本文就SHE的诊断、发病机制、治疗及预后方面的研究进展进行综述，旨在增加临床医生对该病的认识。
关键词： 癫痫;  睡眠相关过度运动性癫痫;  夜间额叶癫痫;  异态睡眠;  诊断;  发病机制;  治疗    
Abstract:

Sleep-related hypermotor epilepsy (SHE), formerly known as nocturnal frontal epilepsy, is characterized by asymmetrical tonic or complex hypermotor seizures during sleep, with transient, frequent and clustering attack. The accurate incidence is not known but somehow low, which is estimated about 1.8/100 000. The differential diagnosis between SHE and parasomnias may be challenging due to possible similarities between the two sleep-related manifestations. In a majority of patients, the etiology is unknown. Identified etiologies are heterogeneous and structural abnormalities, which are involved in the severity and prognosis of SHE. In terms of treatment, it mainly includes pharmacological therapy and surgery. Carbamazepine seems to be the drug of choice in SHE patients, and epilepsy surgery provides excellent results in selected drug-resistant SHE cases. This review will focus on diagnosis, pathogenesis, treatment and prognosis of SHE, aiming to promote its early diagnosis and appropriate treatment.
Key words: Epilepsy    Sleep-related hypermotor epilepsy    Nocturnal frontal lobe epilepsy    Parasomnia    Diagnosis    Pathogenesis    Therapy
收稿日期: 2019-12-26 出版日期: 2020-09-27
:  R742.1  
通讯作者: 王爽     E-mail: xyh19960302@163.com;wangs77@zju.edu.cn
作者简介: 徐佳慧(1990-), 女, 硕士研究生, 住院医师, 主要从事癫痫和睡眠研究; E-mail:xyh19960302@163.com; https://orcid.org/0000-0002-7661-6763
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引用本文:

徐佳慧,金搏,张力三,王爽. 睡眠相关过度运动性癫痫的研究进展[J]. 浙江大学学报（医学版）, 2020, 49(4): 425-430.

XU Jiahui,JIN Bo,ZHANG Lisan,WANG Shuang. Advances in sleep-related hypermotor epilepsy. J Zhejiang Univ (Med Sci), 2020, 49(4): 425-430.

链接本文:

http://www.zjujournals.com/med/CN/10.3785/j.issn.1008-9292.2020.08.04    或    http://www.zjujournals.com/med/CN/Y2020/V49/I4/425

临床表现 发作时间 发作频率 发作持续时间 复杂性 发病时意识 发作后意识朦胧 起病年龄 家族史 是否白天发作 受伤风险
睡眠相关过度运动性癫痫 不固定 可每晚数次 数分钟以内 相对简单 常保留 不明显 多样 常有 有时会 低
非快速动眼期异态睡眠 睡眠起90 min内 一周几次 1~30 min 多变且复杂 不固定 存在 儿童 常有 不会 高
快速动眼期睡眠行为障碍 至少在睡眠起90 min以后，主要在后半夜 不固定，可每晚出现 10 min以内 复杂 极少能回应 无 常50岁以上 罕见 不会 中度
表 1  睡眠相关过度运动性癫痫与异态睡眠的鉴别
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