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目的探讨几种常见免疫介导性多发性周围神经病的临床特点,为早期诊断、鉴别诊断及有效治疗等提供帮助。方法回顾性分析102例免疫介导性多发性周围神经病患者的临床资料,总结其运动、感觉、自主神经受累情况、肌电图表现、脑脊液改变、药物治疗情况及效果。结果合计27例CIDP、49例GBS、6结缔组织病相关周围神经病、4例与中枢神经损害并存的周围神经病、2例POEMS综合征及14例可疑免疫介导周围神经病。免疫介导的多发性周围神经病具有典型的免疫功能紊乱指征,最明显的特点是具有明确的前驱感染史、脑脊液蛋白-细胞分离和对免疫治疗效果反应良好。肌电图可以一定程度上反应疾病恢复程度。结论在临床工作中有很多免疫介导的周围神经病。对病因不明者应该进行详细全面的检查,以提高患者的诊断率。
Objective To investigate the clinical features of several common immunocompetent polycystic peripheral neuropathy and to provide help for early diagnosis, differential diagnosis and effective treatment. Methods The clinical data of 102 patients with immune-mediated multiple peripheral neuropathy were retrospectively analyzed. The motor, sensory, autonomic nerve involvement, EMG findings, cerebrospinal fluid changes, drug treatment and effects were summarized. Results A total of 27 cases of CIDP, 49 cases of GBS, 6 connective tissue-associated peripheral neuropathy, 4 cases of peripheral neuropathy coexisting with central nervous system injury, 2 cases of POEMS syndrome and 14 cases of suspected immune mediated peripheral neuropathy. Immune-mediated polycystic peripheral neuropathy has typical indications of immune dysfunction, most notably the clear history of prodromal infection, CSF-cell isolation and good response to immunotherapy. Electromyography can to some extent reflect the degree of disease recovery. Conclusion There are many immune-mediated peripheral neuropathy in clinical practice. Patients with unknown etiology should conduct a detailed and comprehensive examination to improve the diagnosis rate of patients.