Leger Jean-Marc, Bombelli Francesco, Tran-Thanh Hung, Chassande Bénédicte, Maisonobe Thierry, Viala Karine
Xentre de Référence Maladies Neuromusculaires rares Paris Est, Bâtiment Babinski, Hôpital de la Salpêtrière, 47 bld de l'Hôpital, 75651 Paris, cedex 13.
Bull Acad Natl Med. 2010 Apr-May;194(4-5):753-64; discussion 764-5.
Since the first description of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) by PJ Dyck's group at the Mayo Clinic 35 years ago, a wide range of publications have underlined the clinical, electrophysiologic and histopathologic heterogeneity of this disease. Expert consensus opinion is that CIDP should be considered in any patient with progressive symmetrical or asymmetrical polyradiculoneuropathy whose clinical course is relapsing and remitting or progresses for more than two months, especially if there are positive sensory symptoms, proximal weakness, are flexia without wasting, or preferential loss of vibration or joint-position sense. Electrophysiologic features of demyelinating polyneuropathy (especially conduction blocks) and elevated protein levels in cerebrospinal fluid may assist with the diagnosis. However, various clinical pictures have been described in patients with CIDP including pure motor or sensory impairment, and distal, multifocal or focal distribution. Two specific points have recently been emphasized:--while most CIDP patients have chronic onset, acute onset resembling Guillain-Barré syndrome may sometimes occur;--pure sensory forms may require different diagnostic strategies, including the use of somatosensory evoked potentials showing abnormal proximal sensory conduction, and nerve biopsy showing macrophage-associated demyelination, onion bulb formation, demyelinated and partially remyelinated nerve fibres, endoneurial edema, endoneurial mononuclear cell infiltration, and variation between fascicles. Several sets of diagnostic criteria for CIDP have been proposed, with different sensitivities and specificities. The European Federation of Neurological Societies/Peripheral Nerve Society criteria strike a balance between specificity, which needs to be higher for research purposes than for clinical diagnosis, and sensitivity, which, if too low, might lead to some cases being missed. CIDP patients may have a variety of comorbidities, including diabetes mellitus and IgG or IgA monoclonal gammopathy of undetermined significance. Since the first clinical trial of prednisone in CIDP, several randomized trials have given rise to evidence-based therapeutic approaches. Treatment induction with corticosteroids or intravenous immunoglobulin should be considered for patients with disabling sensory and motor CIDP. Plasma exchange is similarly effective but may be less well tolerated. The existence of relative contraindications to these treatments will determine the choice. The advantages and disadvantages of available treatments should be explained to the patient, who should be involved in the decision-making process. If the response is inadequate or if maintenance doses of the initial treatment are poorly tolerated, alternatives include combination therapy or the addition of an immunosuppressant or immunomodulatory agent, but the evidence is too weak to recommend a particular drug.
自35年前梅奥诊所的PJ·戴克团队首次描述慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）以来，大量出版物都强调了这种疾病在临床、电生理和组织病理学方面的异质性。专家共识意见是，对于任何患有进行性对称性或非对称性多发性神经根神经病的患者，如果其临床病程呈复发缓解型或进展超过两个月，尤其是伴有感觉症状阳性、近端肌无力、无萎缩的腱反射减弱或振动觉或关节位置觉优先丧失时，都应考虑CIDP。脱髓鞘性多发性神经病的电生理特征（尤其是传导阻滞）以及脑脊液中蛋白水平升高可能有助于诊断。然而，CIDP患者出现了各种临床症状，包括单纯运动或感觉障碍，以及远端、多灶性或局灶性分布。最近强调了两个特定要点：——虽然大多数CIDP患者起病隐匿，但有时可能会出现类似吉兰-巴雷综合征的急性起病；——单纯感觉型可能需要不同的诊断策略，包括使用体感诱发电位显示近端感觉传导异常，以及神经活检显示巨噬细胞相关的脱髓鞘、洋葱球形成、脱髓鞘和部分再髓鞘化的神经纤维、神经内膜水肿、神经内膜单核细胞浸润以及束间差异。已经提出了几套CIDP的诊断标准，其敏感性和特异性各不相同。欧洲神经病学学会联合会/周围神经学会标准在特异性（研究目的所需的特异性高于临床诊断）和敏感性（如果敏感性过低可能会导致一些病例漏诊）之间取得了平衡。CIDP患者可能有多种合并症，包括糖尿病以及意义未明的IgG或IgA单克隆丙种球蛋白病。自从首次在CIDP中进行泼尼松的临床试验以来，几项随机试验产生了基于证据的治疗方法。对于有严重感觉和运动功能障碍的CIDP患者，应考虑用皮质类固醇或静脉注射免疫球蛋白诱导治疗。血浆置换同样有效，但耐受性可能较差。这些治疗存在的相对禁忌证将决定治疗的选择。应向患者解释现有治疗的优缺点，并让患者参与决策过程。如果反应不充分或初始治疗的维持剂量耐受性差，替代方案包括联合治疗或加用免疫抑制剂或免疫调节剂，但证据不足，无法推荐特定药物。
