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睡眠相关过度运动性癫痫诊疗进展OA

Diagnosis and management of sleep-related hypermotor epilepsy:an update

中文摘要英文摘要

睡眠相关过度运动性癫痫(sleep-related hypermotor epilepsy,SHE),曾被称为夜间额叶癫痫(nocturnal frontal lobe epilepsy,NFLE),是一种以睡眠期出现过度运动发作或不对称性强直/肌张力障碍姿势为特征的局灶性癫痫综合征.SHE的病因多样,可分为遗传性、结构性和尚不明确的病因三大类.虽然SHE发作多数起源于额叶,但也可起源于岛叶、颞叶、顶叶等额叶外区域.临床诊断主要依赖病史和典型的发作期视频脑电图记录,但头皮脑电图常因运动伪差或深部起源而缺乏特异性改变,从而给诊断带来挑战.治疗方面,卡马西平仍为首选药物,奥卡西平、托吡酯、拉考沙胺、吡仑帕奈等药物亦为临床提供了更多选择.随着人们对烟碱型乙酰胆碱受体在SHE发病中的作用认识不断加深,尼古丁透皮贴片和非诺贝特等能够调节该受体系统的药物也被尝试用于SHE治疗,但仍需更多证据积累.对于存在局灶性结构性病变的药物难治性SHE,癫痫外科手术是有效的治疗手段,有望获得较高的无发作率.尽管近年来在SHE诊断和治疗方面取得了一定进展,但仍存在诸多未解问题和挑战,需要更深入的研究与探索,以改善患者的预后与生活质量.

Sleep-related hypermotor epilepsy(SHE),previously termed nocturnal frontal lobe epilepsy(NFLE),is a focal epilepsy syndrome char-acterized by hypermotor seizures or asymmetric tonic/dystonic posturing occurring predominantly during sleep.SHE has heterogeneous etiologies,in-cluding genetic factors,structural brain lesions,and unknown causes.Although seizures in SHE com-monly originate from the frontal lobe,extrafrontal seizure onset zones—such as the insular,temporal,or parietal regions—have also been identified.Clini-cally,diagnosis largely depends on detailed seizure history and typical ictal video-EEG recordings.How-ever,scalp EEG often fails to provide specific find-ings due to movement artifacts or deeply localized seizure foci,presenting considerable diagnostic challenges.In terms of treatment,carbamazepine remains the first-line medication,but alternative drugs such as oxcarbazepine,topiramate,la-cosamide,and perampanel offer additional thera-peutic options.Advances in our understanding of nicotinic acetylcholine receptor(nAChR)involve-ment in SHE pathogenesis have prompted explo-ration of receptor-modulating treatments,such as nicotine patches and fenofibrate,though robust clinical evidence remains limited.For patients with drug-resistant SHE and clearly defined focal struc-tural lesions,epilepsy surgery is an effective option,often leading to excellent seizure outcomes.De-spite recent progress in diagnosis and management,substantial gaps and challenges persist in the un-derstanding and treatment of SHE,underscoring the need for further research to improve patient out-comes and quality of life.

翟菲菲;黄颜

中国医学科学院 北京协和医学院 北京协和医院神经科,北京 100730中国医学科学院 北京协和医学院 北京协和医院神经科,北京 100730

医药卫生

睡眠相关过度运动性癫痫夜间额叶癫痫过度运动发作抗癫痫发作药物癫痫外科

sleep-related hypermotor epilepsy(SHE)nocturnal frontal lobe epilepsy(NFLE)hyper-motor seizureantiseizure medicationepilepsy surgery

《中国临床药理学与治疗学》 2026 (2)

168-174,7

协和人才培育支持计划D类项目(UHB11978)国家自然科学基金青年项目(82201486)

10.12092/j.issn.1009-2501.2026.02.004

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