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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 neuroelectrophysiological characteristics of multifocal motor neuropathy (MMN). Methods Seven patients with MMN were observed and followed up. Sensory and motor nerve conduction velocity, F wave, conventional electromyography and single fiber electromyography were measured routinely. Partial segmental conduction block was measured by routine segmental motor nerve conduction and Inching technique (CB), analysis of electrophysiological characteristics and clinical correlations. Results All patients were insidious onset, progress more slowly. Clinically, unilateral limb weakness as the first symptom, with the course of disease, affected parts increased. Conventional nerve conduction examination showed that all obviously atrophy of muscle compound action potential amplitude decreased; 4 cases of F wave incidence decreased or disappeared; in clinical involvement and partial involvement of muscle EMG can be seen varying degrees of neurogenic damage. In 4 patients, single-fiber EMG showed tremor and increased fiber density. A total of 16 CBs were determined by Inching technique and 6 were evaluated. In the two clinical and EMG determination of normal nerve found in 5 CB, the remaining 17 CB or suspected CB occurred in mild to moderate weak muscles corresponding to 12 nerves. One patient had positive anti-ganglioside IgG and IgM antibodies. Conclusions Clinical symptoms and signs are the basis of diagnosis of MMN. CB measurement is the most valuable means to confirm the diagnosis of MMN, but CB is not related to clinical symptoms and signs.