脊髓固有性肌阵挛患者的临床特征和神经电生理表现  

Clinical and Neurophysiology Characteristics of Patients with Propriospinal Myoclonus

作　　者：林楠[1] 高伟芳 孙鹤阳[1] 郝红琳[1] 黄颜[1] LIN Nan;GAO Weifang;SUN Heyang(Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences,Beijing 100730,China)

机构地区：[1]中国医学科学院,北京协和医院神经科,北京100730

出　　处：《中风与神经疾病杂志》2022年第3期202-205,共4页Journal of Apoplexy and Nervous Diseases

摘　　要：目的脊髓固有性肌阵挛(propriospinal myoclonus,PSM)是罕见运动障碍疾病,分为症状性、特发性和功能性,入睡期PSM是特发性PSM的一个亚型。本研究结合病例和文献总结其临床及电生理特点,加深对PSM认识。方法研究纳入5例不同类型PSM病例,包括2例入睡期PSM、2例功能性PSM、1例症状性PSM。结果2例入睡期PSM均为慢性病程,常有腹部抽动主诉,与入睡期相关。2例功能性PSM均合并情绪障碍,其中1例发作形式、起源部位多变。电生理检查显示功能性PSM肌阵挛持续时间更长,传导速度更快。此外,入睡期和症状性PSM可出现肌阵挛持续状态。结论不同类型PSM具有各自临床和电生理特点,临床诊疗上需进行鉴别。Objective Propriospinal myoclonus(PSM)is a rare movement disorder.Three types of PSM have been reported:symptomatic,idiopathic and functional PSM.PSM at sleep onset is a subtype of idiopathic PSM.The current study summarized the clinical and neurophysiological characteristics of different types PSM.Methods The current study included 2 cases of PSM at sleep onset,2 cases of functional PSM,1 case of symptomatic PSM.Results Two patients with PSM at sleep onset had chronic course.Abdominal jerk was the main complaint,occurring at sleep-wake transition.Mental disorders were happened in two cases with functional PSM,one of whom had various patterns of jerk and propagation.Neurophysiological examination showed that functional PSM had longer duration of muscle burst and faster velocity of recruitment.Moreover,myoclonic status was observed in PSM at sleep onset and symptomatic PSM.Conclusion PSM showed specific clinical neurophysiological characteristics according to different types.Differential diagnosis is required in clinics.

关 键 词：肌阵挛 睡眠障碍 神经电生理 

分 类 号：R338.63[医药卫生—人体生理学]