多灶性运动神经病的临床研究

Clinical study in multifocal motor neuropathy

目的 探讨多灶性运动神经病 (MMA)的临床特点、神经电生理及病理变化的特异性。方法 结合文献分析 4例MMN的临床资料、肌电图、病理特征及治疗结果。结果 MMN的临床表现为慢性、非对称性远端肢体无力 ;电生理检查以运动神经传导阻滞或部分阻滞为特点 ;病理学特征为运动神经局灶性脱髓鞘性改变。结论 MMN应与运动神经元病和慢性炎性脱髓鞘性多发性神经根神经病鉴别。

Objective To explore the clinical features of multifocal motor neuropathy (MMN) and its specificity of electrophysiology and neuropathology.Methods To analyze the clinical symptoms and signs in 4 cases with MMN,as well as the findings of electromyogram,pathology and their treatment combining with related literatures.Results The clinical features with MMN are chronic asymmetrical weakness of distant limbs.The electromyogram showed general and partial motor nerve conduction block.The neuropathological feature was multifocal demyelination.Conclusions MMN should be differentiated from motor neuron disease and chronic inflammatory demyelinating polyneuropathy.Immunoglobulin infusion may be a useful therapy.