多灶运动神经病病人的临床和神经电生理研究

The clinical and electrophysiological features of patients with multifocal motor neuropathy

目的：分析多灶运动神经病（multifocal motor neuropathy，MMN）的临床及神经电生理特点。方法：对13例MMN患者进行神经传导和常规肌电图（EMG）检查，采用微移（inching）技术判定是否有运动神经传导阻滞（CB）。结果：全部患者均隐袭起病，进展缓慢，临床上以单肢无力为首发症状，随病情发展，受累部位增加。常规神经传导检查可见所有明显萎缩肌肉的复合肌动作电位（CMAP）波幅下降；13例患者神经受累区域以下所支配肌肉EMG检查可见不同程度的神经原性损害。采用微移技术共测定出CB25处和拟诊CB6处。在3条临床及EMG测定均正常的神经发现5处CB，其余26处CB或拟诊CB均发生于轻度至中度无力的肌肉所对应的20条神经。结论：MMN是一种以远端神经受累为主的不对称性周围神经病，神经电生理检查对诊断和鉴别诊断MMN起重要作用，CB是MMN的主要神经电生理表现。

Objective：To analyze the clinical and electrophysiological features of the multifocal motor neuropathy（MMN）. Methods： Thirteen patients with MMN were examined for nerve conduction and conventional electromyography（EMG）. Then by inching technique the conduction block （CB） of motor nerves were determined. Results： The first symptom in all patients was weak in single limb without re markable sensory symptoms. With very slow progression of the disease, more and more areas were involved. Decreased distal amplitude of CMAP was found in all muscles with atrophy. EMG of 13 cases showed nervous damages in the muscles dominated by the affected nerves. CB in 25 segments and probable CB in 6 segments were detected. CB were presented in 5 segments of 3 nerves in which clinical lea tures and EMG were normal,CB or probable CB in other 26 segments were detected in 20 nerves which showed mild or moderately weak chinically. Conclusion： MMN is an unsymmetrical peripheral neuropathy which is affected mainly on the distal part of the nerves. Electroneurophysiology plays an important role in the diagnosis and discrimination of MMN. CB might serve as a characteristic of MMN.