慢性运动轴索性神经病患者随访观察

Follow-up study of patients with chronic motor axonal neuropathy

目的探讨慢性运动轴索性神经病（CMAN）的特点及临床转归。方法对16例临床和神经电生理确诊的慢性运动轴索性神经病患者进行随访研究，包括生存与否，用药情况，体征变化，运动单位数目估计。同时对60例肌萎缩侧索硬化（ALS）患者进行相关研究，比较两组病人的预后。结果CMAN组经过丙种球蛋白治疗，随访18个月，6例进展为ALS，其余10例患者临床表现好转，MUNE值由68±17升为154±25，而ALS组肌无力、肌萎缩加重，MUNE值由64±6下降为55±9。结论慢性运动轴索性神经病可能与免疫相关，预后良好，易与早期肌萎缩侧索硬化混淆。

Objective To study the characteristic of chronic motor axonal neuropathy and the prognosis of it. Methods 16 chronic motor axonal neuropathy （CMAN） patients were diagnosed by clinical manifestation and neurophysiologic manifestation. Follow-up study was done including living state, medicine they taken, change of physical sign and motor unit number estimation. 60 patients with amyotrophic lateral sclerosis were studies at the same time. The prognosis of these patients was compared. Results After being treated with intravenous immunoglobulin therapy, the patients with CMAN showed a good clinical recovery except 6 patients who had the characteristic of ALS in the 18 months follow-up study, and the MUNE changed from 68±17 to 154 ±25. On the contrary, the patients with ALS had worse clinical manifestation, and the MUNE changed from 64 ± 6 to 55 ± 9. Conclusions Immunological factor may be involved in the mechanism of chronic motor axonal neuropathy which has a better prognosis and is difficult to differentiate from amyotrophic lateral sclerosis in the earlier stage.