Service de Neurologie, Centre de Référence Neuropathies périphériques rares, CHU Limoges, France.
Lancet Neurol. 2010 Apr;9(4):402-12. doi: 10.1016/S1474-4422(10)70041-7.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic neuropathy of supposed immune origin. Understanding of its pathophysiology has recently improved, although its causes remain unclear. The classic presentation of CIDP includes sensory and motor symptoms in the distal and proximal segments of the four limbs with areflexia, evolving over more than 8 weeks. Raised protein concentrations in CSF and heterogeneous slowing of nerve conduction are typical of the condition. In addition to this usual phenotype, distribution of symptoms, disease course, and disability can be heterogeneous, leading to underdiagnosis of the disorder. Diagnosis is sometimes challenging and can require use of imaging and nerve biopsy. Steroids and intravenous immunoglobulin are effective, and plasma exchange can be helpful as rescue therapy. The usefulness of immunosuppressants needs to be established. The identification of specific diagnostic markers and new therapeutic strategies with conventional or targeted immunotherapy are needed to improve the outlook for patients with CIDP.
慢性炎症性脱髓鞘性多发性神经病（CIDP）是一种推测由免疫原因引起的慢性神经病。尽管其病因仍不清楚，但对其病理生理学的理解最近已有改善。CIDP 的典型表现包括四肢远端和近端的感觉和运动症状，伴有反射消失，病程超过 8 周。CSF 中蛋白浓度升高和神经传导速度不均匀减慢是该病的典型表现。除了这种常见的表型外，症状的分布、疾病过程和残疾程度可能存在异质性，导致该病的诊断不足。诊断有时具有挑战性，可能需要使用影像学和神经活检。类固醇和静脉注射免疫球蛋白有效，血浆置换可作为抢救治疗。免疫抑制剂的有效性尚待确定。需要确定特定的诊断标志物和新的治疗策略，包括常规或靶向免疫疗法，以改善 CIDP 患者的预后。
