多灶性运动神经病的临床和神经电生理研究  

The clinical and electrophysiological features of multifocal motor neuropathy

作　　者：刘明生[1] 崔丽英[1] 李晓光[1] 陈琳[1] 汤晓芙[1] 郭玉璞[1] 李本红[1] 任海涛[1] 

机构地区：[1]中国医学科学院中国协和医科大学北京协和医院神经科,100730

出　　处：《中华神经科杂志》2004年第1期62-64,共3页Chinese Journal of Neurology

摘　　要：目的　分析多灶性运动神经病 (MMN)的临床及神经电生理特点。方法　对 7例MMN患者进行观察随访 ,常规测定感觉及运动神经传导速度、F波、常规肌电图及单纤维肌电图 ,采用常规节段性运动神经传导以及Inching技术测定部分性传导阻滞 (CB) ,分析电生理特点与临床之间的相关性。结果　全部患者均隐袭起病 ,进展较缓慢。临床上以单肢无力为首发症状 ,随病程发展 ,受累部位增加。常规神经传导检查可见所有明显萎缩的肌肉复合动作电位波幅下降 ;4例患者F波出现率下降或消失 ;在临床受累及部分未受累肌肉肌电图检查可见不同程度的神经源性损害。 4例患者单纤维肌电图表现为颤抖增宽和纤维密度增高。采用Inching技术共测定出CB 16处 ,拟诊CB 6处。在 2条临床及肌电图测定均正常的神经发现 5处CB ,其余 17处CB或拟诊CB均发生于轻度至中度无力的肌肉所对应的 12条神经。 1例患者血抗神经节苷脂抗体IgG和IgM阳性。 结论　临床症状及体征是诊断MMN的基础 ,CB测定是确定MMN诊断的最有价值的手段 ,但CB与临床症状和体征并非完全相关。Objective To analyze the clinical and electrophysiological features of the multifocal motor neuropathy (MMN). Methods All 7 patients with MMN were followed up for 0.7 to 3.4 years. The onset and development features were clinically summarized. Conventional motor and sensory nerve conduction, F wave, EMG, single fiber EMG (SFEMG), segment motor nerve conduction and Inching technique were performed. The relations between clinic and eletrophysiology were analyzed. Results The first symptom in all patients was weakness in single limb without remarkable sensory symptoms. With the very slow progression of the disease, more and more areas were involved. Motor findings including the asymmetric and distal were greater than the proximal weakness and showed various degrees of muscular atrophy. High titers serum IgG and IgM anti-GM 1 antibodies were found in only one patient. 16 segments with conduction block (CB) and 6 segments with probable CB were detected. CB were presented in 5 segments of the 2 nerves in which clinic features and EMG were normal, the other 17 segments with CB or probable CB were detected in 12 nerves which showed mild to moderate weakness clinically. Follow-up of segment motor nerve conduction in 1 patient showed that a new CB was detected in left ulnar nerve, while a CB identified in right median nerve 16 months ago disappeared due to a significant decrease in the distal CMAP amplitude. SFEMG showed an increased jitter and fiber density in 4 patients. Standard EMG showed neurogenic disorders in all muscles with weakness and atrophy, abnormal EMG was also found in some clinically normal muscles in 3 patients, but the percentage of the increased duration and amplitude of motor unit potential was discrepancy in different muscles innervated by different nerves in the same patient. F wave was absent in 3 nerves of 3 patients and frequency of F wave decreased in 1 nerve of 1 patient. Decreased distal amplitude of CMAP was found in all muscles with atrophy. Conclusion Clinical features are one of the most i

关 键 词：多灶性运动神经病 MMN 神经电生理 神经传导阻滞 单神经病 

分 类 号：R744.8[医药卫生—神经病学与精神病学]