14例多灶性运动神经病的临床、电生理和免疫特征分析

Clinical and Immunological Features and Electrophysiology of Multifocal Motor Neuropathy

目的探讨多灶性运动神经病(MMN)的临床、电生理以及免疫特征。方法对14例按照美国电诊断协会标准(AAEM)诊断为MMN的患者,通过临床、肌电图、脑脊液检查以及免疫治疗的结果分析其特点。结果14例患者临床特点均为肢体多灶性不对称性乏力,1例患者存在轻度感觉缺失;肌电图诊断11例(78.57%)患者存在肯定的运动传导阻滞(CB);7例(50%)脑脊液(CSF)里有神经节苷酯(GM1)抗体阳性,10例(71.43%)对丙种球蛋白治疗反应良好,肢体乏力症状得到改善。结论慢性进行性、不对称性下运动神经元瘫痪和多灶性运动神经传导阻滞及血清高滴度抗GM l抗体是MMN特征,治疗以丙种球蛋白为首选。

Objective To determine clinical and immunological features and electrophysiology of multifocal motor neuropathy. Methods Fifteen patients diagnosed as multifocal motor neuropathy（MMN） by the American Association of Electrodiagnostic Medicine （AAEM） were studied on clinics, electromyologram （ EMG）, cerebrspinal fluid （CSF） and pathology. Results 14 patients with progressive multifocal - antisymmetry motor impairment were studied, and 1 patient was minimal sensory loss; Electromyologram studies disclosed the presence of a definite conduction block（CB） in 11 patients; seven patients＇ganglioside GM1 were positive in CSF; Ten patients were effectively responded to intravenous immunoglobulin treatment and limb impairment was significantly improved. Conclusion The typical clinical features of MMN consist of chronic progressive antisymmetry lower motor neuron impairment with conduction block and high titer ganglioside GM1. The first choice of treatment is immunoglobulin.