Lembert-Eaton 肌无力综合征患者11例运动易化试验  

A study on post-exercise facilitation in 11 patients with Lambert-Eaton myasthenic syndrome

作　　者：丁青云[1] 方佳[1] 管宇宙[1] 崔丽英[1,2] 胡右方 Ding Qingyun Fang Jia Guan Yuzhou Cui Liying Hu Youfang(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,100730 [2]中国医学科学院神经科学中心

出　　处：《中华神经科杂志》2016年第12期947-951,共5页Chinese Journal of Neurology

摘　　要：目的初步探索 Lambert-Eaton 肌无力综合征（ Lambert-Eaton myasthenic syndrome ， LEMS）患者运动易化试验（post-exercise facilitation， PEF）的具体操作方法及其临床意义。方法纳入我院2014年12月至2016年4月临床确诊的 LEMS 患者11例，均行PEF 和重复频率电刺激（ repetitive nerve stimulation ， RNS）检测。行PEF检测时，嘱患者收缩小指展肌，运动完成后立即刺激尺神经并记录复合肌肉动作电位（ compound muscle action potential ， CMAP），休息1 min后嘱患者再次收缩小指展肌并在运动完成后记录尺神经CMAP，小指展肌收缩时间从0 s开始，每次增加2 s，重复上述过程直至记录到的CMAP波幅不再增加，记录达到最大CMAP波幅所需的运动时间。在PEF及高频刺激两种方法达到最大 CMAP波幅后继续以0.5 Hz 的频率刺激尺神经并记录CMAP 直至CMAP波幅恢复至初始水平，记录两种方法中CMAP波幅恢复所需的时间。结果11例LEMS患者PEF后均出现CMAP波幅增高现象。记录到最大CMAP波幅对应的小指展肌收缩时间为6～20 s，中位时间12（10，14） s；将PEF时间设定为10～12 s时，敏感度为63.6％。高频刺激以及PEF检测后CMAP波幅恢复过程一致性较好，CMAP波幅恢复至初始水平的平均时间为（50.4±16.0） s。结论应用PEF检测能快速判断结果，且患者更易耐受。临床疑诊LEMS的患者可常规进行运动时间为10～12 s的PEF检测以替代高频刺激检测。当PEF后CMAP波幅较初始升高≥60％时可认为检测结果阳性；CMAP波幅递增≤24％则可认为为阴性；当出现可疑递增现象时（ CMAP波幅增加24％～60％），可进一步行高频刺激检测。而两次检测的间隔时间需大于（50.4±16.0） s。Objective To study the operation method of the post-exercise facilitation （ PEF ） in Lambert-Eaton myasthenic syndrome （ LEMS ） , and to seek for the meaning of PEF in clinical work.Methods Eleven patients with LEMS were included to accept the PEF and the repetitive nerve stimulation （ RNS） test.When performing the PEF test , compound muscle action potentials （ CMAPs ） of abductor digitiminimi were recorded after a specified time of exercise.The patients would get rest for 1 minute before the next test.The same exercises were repeated for several times.The exercise time started from 0 second , and 2 seconds was added each time until the CMAP did not increase.The PEF or RNS test was followed by a continuous stimuli at 0.5 Hz and the CMAPs were recorded to study the recovery process.Results The CMAPs of abductor digitiminimi increased significantly after PEF.The median time for CMAPs to achieve its maximum was 12（10,14） seconds.The recovery processes of PEF and RNS showed that the CMAP would return to normal after （ 50.4 ±16.0 ） seconds.Conclusions Patients with suspected LEMS might get a 10-12 seconds of PEF test instead of RNS for it is fast and painless.If the potential of CMAPs increased ≥60%, the result would be positive.If the potential of CMAPs increased ≤24%, the result would be negative.A suspicious increments of CMAPs （ increasing by 24%-60%） should warrant a further RNS test.And the interval between the two tests should be longer than （50.4 ±16.0） seconds.

关 键 词：郎伯综合征 运动易化试验 电刺激 电生理学 

分 类 号：R746[医药卫生—神经病学与精神病学]