多灶性运动神经病10例分析

Multifocal motor neuropathy 10 case report

目的 探讨多灶性运动神经病 (multifocal motor neuropathy,MMN )的临床特点和诊断标准。方法 回顾性分析 1998年～ 2 0 0 1年间本院神经内科收治的 10例 MMN患者的临床表现、电生理表现、化验检查、病理结果。结果 本组病例的临床特点是 :10例患者均为男性 ,平均发病年龄 42 .1岁 ,症状均以单肢无力首发 ,远端重于近端 ,伴有肌肉萎缩 ,半数有肌束颤动 ;9/ 10有腱反射减低 ,感觉症状少 ,颅神经不受累 ;5/ 10脑脊液蛋白增高 ,5/ 10血 CPK增高。电生理检查 7/ 10出现了典型的运动神经多发 CB,最常见的发生部位是正中神经、尺神经和胫神经 ;3 / 10病例经神经活检主要表现为脱髓鞘改变 ,伴有轻中度轴索损害 ,病程较长的可有髓球形成 ,无炎性细胞浸润。结论 应综合临床表现、电生理特点等作出

Objective T o investigate the clinical features and diagnostic criteria of multiple motor neur opathy(MMN).Methods Ten cases of MMN,in the department of Neuro logy Xuanwu Hospital from 1998 to 2001,were analyzed clinically, electrophysiolo gically,and pathologically.Results Ten cases were male,their on set age was mean 42.1,all of them had a single limb weakness,accompanied by musc le atrophy and half of them had fasciculation.Reflexes were reduced in about 90% of the patients.Sensory impairment and cranial involvement were rare.In 50% of patients,CSF protein concentrations and serum CPK levels slightly increased.Part ial motor conduction blocks,the electrophysiological hallmark of MMN,occurred in 7/10 of the patients, most frequently in the median, ulnar and tibial nerves.Th ree patient's undertaking biopsy in sural nerve showed mild axonal degeneration, demyelination or both without inflammatory infiltrates. Onion bulbs were seen in patients with longer course.Conclusion The diagnosis of MMN sh ould be made according to the clinical,electrophysiological,laboratory findings and morphological findings.