Said Gérard
Service de Neurologie, Hôpital de Bicêtre, Assistance Publique Hopitaux de Paris, Université Paris-Sud, 94275 Le Kremlin-Bicêtre, France.
Neuromuscul Disord. 2006 May;16(5):293-303. doi: 10.1016/j.nmd.2006.02.008.
Chronic inflammatory demyelinative polyneuropathy (CIDP) is an acquired neuropathy, presumably of immunological origin. Its clinical presentation and course are extremely variable. CIDP is one of the few peripheral neuropathies amenable to treatment. Typical cases associate progressive or relapsing-remitting motor and sensory deficit with increased CSF protein content and electrophysiological features of demyelination. In other instances the neuropathy is predominantly or exclusively motor or sensory, CSF normal and electrophysiological studies fail to show evidence of demyelination. In such cases conventional diagnostic criteria are not filled yet the patient may respond to immunomodulatory treatments. In this paper we review the diagnostic pitfalls and clinical variants of CIDP to illustrate the problems that may arise. The different therapeutic options are reviewed. Axon loss associated with demyelination is the most important factor of disability and resistance to treatment.
慢性炎症性脱髓鞘性多发性神经病（CIDP）是一种后天性神经病，可能源于免疫因素。其临床表现和病程极具变异性。CIDP是少数可治疗的周围神经病之一。典型病例表现为进行性或复发缓解性运动和感觉功能障碍，同时伴有脑脊液蛋白含量升高及脱髓鞘的电生理特征。在其他情况下，神经病主要或仅表现为运动或感觉障碍，脑脊液正常，电生理研究未显示脱髓鞘证据。在此类病例中，虽不符合传统诊断标准，但患者可能对免疫调节治疗有反应。在本文中，我们回顾了CIDP的诊断陷阱和临床变异情况，以阐明可能出现的问题。同时也对不同的治疗选择进行了综述。与脱髓鞘相关的轴突丧失是导致残疾和治疗抵抗的最重要因素。
