李琪 赵东杰 王玉华 董莎 常磊 张鹏 倪瑶

    [摘要] 慢性炎性脱髓鞘性多发性神经病(CIDP)是一种获得性免疫介导的周围神经疾病，其特征表现为无力和感觉障碍，可伴随其他神经功能障碍。CIDP除经典型外，还有诸多变异型，易误诊，提高变异型CIDP诊断十分重要，其诊断通常结合了临床表现、电生理检查及支持诊断，如神经活检、诱发电位、脑脊液检查、MRI、超声波等支持诊断。目前CIDP的一线治疗方法有皮质类固醇(CS)、静脉注射免疫球蛋白(IVIg)、血浆交换(PE)和免疫抑制治疗。本文旨在对CIDP的临床表现、诊断和治疗等方面的研究进展进行综述。

    [关键词] 慢性炎性脱髓鞘性多发性神经病；变异型；诊断；治疗

    [中图分类号] R744.5 [文献标识码] A [文章编号] 1673-7210(2018)02(b)-0037-04

    [Abstract] Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated peripheral neuropathy characterized by weakness and sensory disturbances that may be associated with other neurological disorders. In addition to the classic CIDP， there are many variants which easily lead to misdiagnosis， so that improving the diagnosis of variant CIDP is very important. Diagnosis usually incorporates clinical manifestations， electrophysiological examinations， and other supporting evidence， such as nerve biopsy， somatosensory evoked potential， cerebral spinal fluid examination， MRI and ultrasound support evidence， CIDP first-line treatment including corticosteroid (CS) ......