Kuwabara Satoshi
Department of Neurology, Graduate School of Medicine, Chiba University.
Brain Nerve. 2025 Jan;77(1):29-34. doi: 10.11477/mf.188160960770010029.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) includes a number of clinical subtypes. The major phenotype is "typical CIDP," which is characterized by symmetric and "proximal and distal" muscle weakness. Due to historical changes in the concept of CIDP, multifocal motor neuropathy, anti-myelin-associated glycoprotein (anti-MAG) neuropathy, and autoimmune nodopathy were excluded. International guidelines for the diagnosis and treatment of CIDP were first published in 2005 and revised in 2010. In 2021, the guidelines of the European Academy of Neurology (EAN)/Peripheral Nerve Society (PNS) were the second revision, reflecting changes in the disease concept and progress of electrodiagnosis, neuroimaging, and novel treatments. This review introduces the outline of the guidelines in addition to typical CIDP; related chronic demyelinating neuropathies were classified as CIDP variants. The diagnosis of CIDP is based on (1)the phenotype of a typical CIDP or variant, (2)electrophysiological evidence of peripheral nerve demyelination, and (3)exclusion criteria. The first-line treatments for typical CIDP are corticosteroids and immunoglobulin therapy. These guidelines recommend intravenous or subcutaneous immunoglobulin as maintenance therapy, as well as unresolved questions on the evolving concept of CIDP and future treatments.
慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）包括多种临床亚型。主要表型为“典型CIDP”，其特征为对称性以及“近端和远端”肌肉无力。由于CIDP概念的历史变迁，多灶性运动神经病、抗髓鞘相关糖蛋白（抗-MAG）神经病和自身免疫性结节病被排除在外。CIDP诊断和治疗的国际指南于2005年首次发布，并于2010年修订。2021年，欧洲神经病学学会（EAN）/周围神经学会（PNS）指南进行了第二次修订，反映了疾病概念的变化以及电诊断、神经影像学和新治疗方法的进展。本综述除介绍典型CIDP外，还介绍了指南大纲；相关慢性脱髓鞘性神经病被归类为CIDP变异型。CIDP的诊断基于：（1）典型CIDP或变异型的表型；（2）周围神经脱髓鞘的电生理证据；（3）排除标准。典型CIDP的一线治疗方法是皮质类固醇和免疫球蛋白疗法。这些指南推荐静脉或皮下注射免疫球蛋白作为维持治疗，以及关于CIDP不断演变的概念和未来治疗方法的未解决问题。
