Continuum (Minneap Minn). 2020 Oct;26(5):1205-1223. doi: 10.1212/CON.0000000000000907.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and its variants comprise a group of immune-mediated neuropathies with distinctive clinical presentations and electrodiagnostic features. Prompt recognition of these treatable disorders is mandatory as delays result in significant disability and morbidity. This article highlights the clinical presentation, pathophysiology, diagnostic evaluation, and treatment approach of these polyneuropathies.
The spectrum of CIDP is expanding with the recent characterization of neuropathies associated with nodal and paranodal antibodies. These neuropathies are distinguished by their unique presentations and are often refractory to IV immunoglobulin (IVIg) therapy. Subcutaneous immunoglobulins have recently been approved as a treatment option for CIDP and join corticosteroids, IVIg, and plasma exchange as first-line treatment.
CIDP is characterized by progressive symmetric proximal and distal weakness, large fiber sensory loss, and areflexia, with clinical nadir reached more than 8 weeks after symptom onset. Autoimmune demyelinating neuropathies fall on a continuum, with differences in the type of nerve fibers affected and pattern of deficits. Distinguishing between typical CIDP and its variants allows for selection of the most appropriate treatment.
慢性炎症性脱髓鞘性多发性神经病（CIDP）及其变异型是一组具有独特临床表现和电诊断特征的免疫介导性神经病。必须迅速识别这些可治疗的疾病，因为延迟会导致显著的残疾和发病率。本文重点介绍这些多发性神经病的临床表现、病理生理学、诊断评估和治疗方法。
随着与神经结和神经旁抗体相关的神经病的特征最近得到阐明，CIDP 的范围正在扩大。这些神经病的特点是其独特的表现，并且通常对静脉注射免疫球蛋白（IVIg）治疗有抗性。皮下免疫球蛋白最近已被批准作为 CIDP 的治疗选择，并与皮质类固醇、IVIg 和血浆置换一起作为一线治疗。
CIDP 的特征是进行性对称的近端和远端无力、大纤维感觉丧失和反射消失，在症状出现后 8 周以上达到临床最低点。自身免疫性脱髓鞘性神经病呈连续谱，受累神经纤维的类型和缺陷模式存在差异。区分典型 CIDP 和其变异型有助于选择最合适的治疗方法。
