Lapergue B, Lefaucheur J-P, Bourahoui A, Nordine T, Hosseini H, Créange A
Service de neurologie, hôpital Henri-Mondor, AP-HP, Université Paris XII, Créteil.
Rev Neurol (Paris). 2007 Dec;163(12):1227-31. doi: 10.1016/S0035-3787(07)78407-2.
Lambert-Eaton myasthenic syndrome (LEMS) is a rare presynaptic disorder of the neuromuscular junction. Electrodiagnosis is characterized by compound muscle action potentials of small amplitude at rest, normalizing immediately after brief exercise or high-rate nerve stimulation. Needle electromyographic (EMG) activity is classically described as normal or myogenic.
We report the case of a young patient with a non-paraneoplastic LEMS in whom the initial electroneuromyographic examination showed neurogenic changes in needle EMG concomitant with typical features of presynaptic neuromuscular junction disorder at single and repetitive nerve stimulation.
Neurogenic EMG abnormalities were not previously described in patients with LEMS and could result from "functional" reversible denervation, depending on the presence of anti-calcium channel antibodies at axon terminals.
Neurogenic features in needle EMG examination in case of subacute motor deficiency should not eliminate the possibility of a presynaptic neuromuscular junction disorder, such as LEMS.
兰伯特-伊顿肌无力综合征(LEMS)是一种罕见的神经肌肉接头突触前疾病。电诊断的特征是静息时复合肌肉动作电位幅度小,在短暂运动或高频神经刺激后立即恢复正常。针极肌电图(EMG)活动通常被描述为正常或肌源性。
我们报告一例非副肿瘤性LEMS年轻患者,其初始神经肌电图检查显示针极EMG有神经源性改变,同时在单神经和重复神经刺激时有突触前神经肌肉接头疾病的典型特征。
此前未在LEMS患者中描述过神经源性EMG异常,其可能由“功能性”可逆性失神经支配导致,这取决于轴突末端抗钙通道抗体的存在。
在亚急性运动功能缺陷病例中,针极EMG检查出现神经源性特征不应排除突触前神经肌肉接头疾病(如LEMS)的可能性。