多灶性运动神经病的临床和电生理特征

Clinical and electrophysiological features of multifocal motor neuropathy

目的研究多灶性运动神经病(MMN)的临床和电生理特征。方法回顾性分析16例MMN患者的临床资料和电生理检查结果。结果本组患者均为中青年起病,平均年龄(28.2±2.4)岁;16例患者均有非对称性四肢无力,肌力Ⅱ～Ⅳ级,上肢无力重于下肢,右侧重于左侧,4例有轻度肌萎缩,无感觉减退;肌电图检查发现所有患者均存在节段性运动神经传导阻滞(CB),病变神经的运动神经传导速度(MCV)均减慢,感觉神经传导速度(SCV)减慢1例。结论MMN是一种不对称的周围神经病,主要累及运动神经;神经电生理检查对诊断起重要作用,节段性运动神经CB是MMN特征性的神经电生理表现。

Objective To study clinical and electrophysiological features of multifocal motor neuropathy （MMN）. Methods The clinical features and data of electrophysiological examination from 16 patients with MMN were analyzed retrospectively. Results In the present study, the onset age was 14 to 47 years with the mean age of （28.2 ± 2.4） years. All the 16 patients presented with unsymmetrical weakness of extremities （ Ⅱ -Ⅳ grade of muscle strength） and no hypoesthesia. More serious weakness was found in the upper limbs and in the right extremities. 4 cases were accompanied with mild amyotrophy. Electromyography showed segment conduct block （CB） and slow down of conduct velocity of motor nerves in all 16 patients. Slow conduct velocity of sensory nerve was found only in one case. Conclusions MMN is an unsymmetrical peripheral neuropathy, which affects mainly on motor nerve. Nerve electrophysiology plays an important role in diagnosis of MMN and CB is the characteristic manifestation of nerve electrophysiological change in MMN.