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  • Title: [Analysis of the clinical and electrophysiological characteristics of multifocal motor neuropathy].
    Author: Wang HF, Yang F, Cui F, Chen ZH, Ling L, Huang XS.
    Journal: Zhonghua Nei Ke Za Zhi; 2017 Nov 01; 56(11):842-845. PubMed ID: 29136715.
    Abstract:
    Ten patients diagnosed with multifocal motor neuropathy (MMN) were recruited in the Department of Neurology at Chinese PLA General Hospital from January 1, 2009 to August 31, 2015. The clinical and electrophysiological features were analyzed retrospectively. All patients complained of progressive asymmetric limb weakness, which was more severe in distal than in proximal. Five presented muscle atrophy. None had sensory disturbances. All suffered diminished or disappeared tendon reflex, whereas Babinski signs were negative. Multi-focal conduction block (CB) was confirmed by nerve conduction studies (NCS) in all patients and 7 showed spontaneous potentials in needle electrode electromyography. Abnormal sensory nerve conduction was seen in 3 patients. Laboratory test revealed anti-ganglioside GM1 antibody in cerebrospinal fluid (CSF) in 6 cases and elevated CSF protein in 7 cases. Limb weakness alleviated greatly in 9 cases after intravenous immunoglobulin (IVIg) treatment. But the other one reported poor response, who had long course of disease, serious limb weakness and obvious muscle atrophy. Motor nerve damage is the most important manifestation of MMN and sensory nerve damage may also appear. NCS is essential to the diagnosis of this disease, with CB as the characteristic electrophysiological feature. IVIg is an effective treatment. 选择2009年1月至2015年8月解放军总医院收治的10例多灶性运动神经病(MMN)患者，对其临床资料及神经电生理检查结果进行回顾性分析。10例患者均表现为慢性、不对称性肢体无力症状，远端重于近端，其中5例有肌肉萎缩；均无感觉障碍，腱反射均减弱或消失、无病理反射；运动神经传导检测均有多灶性传导阻滞；7例针极肌电图检查出现自发电位；3例有感觉神经传导异常。脑脊液检查中，6例抗神经节苷脂(GM1)抗体阳性，7例蛋白升高。经静脉注射免疫球蛋白治疗后，9例肌无力症状明显改善，1例效果较差，此例病程较长，肌无力症状重，肌肉萎缩明显。本研究显示MMN以运动神经受损为主要表现，可伴有亚临床感觉神经受累，神经电生理检查对该病的诊断有重要意义，运动传导阻滞为其特征性表现，免疫球蛋白治疗有效。.
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