Wang H F, Yang F, Cui F, Chen Z H, Ling L, Huang X S
Department of Neurology, Chinese PLA General Hospital, Beijing 100853, China.
Zhonghua Nei Ke Za Zhi. 2017 Nov 1;56(11):842-845. doi: 10.3760/cma.j.issn.0578-1426.2017.11.013.
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.
2009年1月1日至2015年8月31日期间,中国人民解放军总医院神经内科招募了10例多灶性运动神经病(MMN)患者。对其临床和电生理特征进行回顾性分析。所有患者均主诉进行性不对称肢体无力,远端比近端更严重。5例出现肌肉萎缩。均无感觉障碍。所有患者腱反射减弱或消失,而巴宾斯基征均为阴性。所有患者经神经传导研究(NCS)均证实有多灶性传导阻滞(CB),7例针电极肌电图显示有自发电位。3例患者感觉神经传导异常。实验室检查显示6例脑脊液(CSF)中有抗神经节苷脂GM1抗体,7例CSF蛋白升高。9例患者经静脉注射免疫球蛋白(IVIg)治疗后肢体无力明显缓解。但另1例病程长、肢体无力严重且肌肉萎缩明显,治疗效果不佳。运动神经损伤是MMN的最重要表现,也可能出现感觉神经损伤。NCS对本病的诊断至关重要,CB是其特征性电生理表现。IVIg是一种有效的治疗方法。