多灶性运动神经病的临床、电生理和病理分析

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

Analysis of clinic and electrophysiology and pathology on multifocal motor neuropathy

Objective Fifteen patients diagnosed as multifocal motor neuropathy (MMN) were studied on clinic and electrophysiology and pathology,and relevant aspects of diagnosis were also discussed.Method Electrophysiological studies were performed.Anti-ganglioside GM 1 antibody titers were tested by ELISA.Sural nerve biopsies were performed on 5 patients.Result The disease began in arms in seven cases.The disease began in feet in 8 cases.Weakness was usually accentuated distally,and it was not parallel with amyotrophy.All patients showed motor conduction block (CB).Increased anti-GM 1 antibody titers were found in the serum of 4/15 patients.Pathological results showed demyelination.Conclusion These studies suggest that MMN is a demyelinating peripheral neuropathy induced by immune,which has an characteristic on motor CB.The diagnostic criteria and precise judge of CB play a decisive role in the diagnosis of MMN.