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目的 对 15例诊断为多灶性运动神经病 (multifocalmotorneuropathy ,MMN)的病例的临床、电生理和病理进行研究 ,并就诊断易混淆的问题进行讨论。方法 行肌电图检查 ,以ELISA法检测血、脑脊液抗GM1抗体 ,5例病人行腓肠神经活检。结果 上肢起病 7例 ,下肢起病 8例 ,肌无力以肢体的远端为重 ,与肌萎缩并不平行。 4例病人血抗GM1(IgM )抗体升高。所有病人均有运动传导阻滞。病理为不同程度髓鞘脱失 ,有轻度或散在的轴索变性。结论 结果表明MMN为免疫介导的脱鞘性周围神经病 ,有特征性的运动传导阻滞 (CB)。CB的诊断标准和判断的准确性对MMN的诊断具有决定性作用
Objective To investigate the clinical, electrophysiological and pathological findings of 15 cases diagnosed as multifocal motor neuropathy (MMN), and to discuss the confusing problems of diagnosis. Methods Electromyography, anti-GM1 antibodies against blood and cerebrospinal fluid were detected by ELISA, and sural nerve biopsies were performed on 5 patients. Results The onset of upper extremities in 7 cases, 8 cases of lower extremity onset, muscle weakness to the distal limbs as the most important, and muscle atrophy is not parallel. 4 cases of patients with anti-GM1 (IgM) antibody increased. All patients had motor block. Pathological demyelination to varying degrees, with mild or scattered axonal degeneration. Conclusions The results show that MMN is an immune-mediated demyelinating peripheral neuropathy with a characteristic motor block (CB). The diagnostic criteria of CB and the accuracy of the judgment play a decisive role in the diagnosis of MMN