Chronic immune demyelinating neuropathies
Chronic immune demyelinating neuropathies
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DOI：
10.1055/s-2008-1041070
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发表时间：
1994-01-01
影响因子：
2.7
通讯作者：
Pestronk, Alan
中科院分区：
文献类型：
--
作者：
Lopate, Glenn;Pestronk, Alan
慢性多发性神经病的名单很长。在临床实践中，免疫介导的脱髓鞘性多发性神经病是鉴别诊断的重要组成部分，因为免疫调节治疗通常可以改善这些疾病患者的功能和生活质量。免疫介导的脱髓鞘性多发性神经病的分类是必要的，因为每种综合征似乎对不同的治疗药物都有一个特征性的反应谱（表 1）。免疫介导的多发性神经病可通过以下定义进行分类：1) 涉及的临床模式，2) 疾病的组织或亚细胞靶标，以及 3) 血清自身抗体的抗原靶标。临床模式的诊断有用特征包括功能受累（例如，运动与感觉丧失）、缺陷的解剖分布以及多发性神经病的时间进程。免疫介导的多发性神经病的病理过程的靶标包括髓磷脂、轴突、细胞体和亚细胞成分。 I.'组织靶标最常通过电诊断和组织病理学测试来定义。血清抗体的测量也可用于定义多发性神经病综合征（表 2）。目前临床实践中使用的许多抗体检测需要进一步的技术修改和临床验证，以优化其特异性和敏感性。然而，血清自身抗体的诊断测试尤其重要，因为它可以将一些以前无法治疗的疾病识别为免疫介导的疾病，并提供合理治疗的途径。
The list of chronic polyneuropathies is long. In clinical practice, immune-mediated demyelinating polyneuropathies are important components of the differential diagnosis, as immunomodulating treatments often lead to improvements in function and quality of life of patients with these disorders. Categorization of immunemediated demyelinating polyneuropathies is necessary because each syndrome appears to have a characteristic spectrum of response to different therapeutic agents (Table 1). Immune-mediated polyneuropathies can be categorized by defining 1) clinical patterns of involvement, 2) tissue or subcellular targets of disease, and 3) antigenic targets of serum autoantibodies. Diagnostically useful features of clinical patterns include the functional involvement (for example, motor vs sensory loss), the anatomical distribution of deficits, and the time course of the polyneuropathy. Targets of the pathologic processes in immune-mediated polyneuropathies include myelin, axons, cell bodies, and subcellular constituents. I.'Tissue targets are most commonly defined by electrodiagnostic and histopathologic testing. Measurement of serum antibodies can also be useful in defining polyneuropathy syndromes (Table 2). Many of the antibody tests now used in clinical practice require further technical modifications and clinical validation to optimize their specificity and sensitivity. However, diagnostic testing for serum autoantibodies can be especially important, as it may identify some previously untreatable disorders as immune-mediated and provide an avenue to rational therapy.