Lang Bethan, Dale Russell C, Vincent Angela
Neurosciences Group, Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, University of Oxford, UK.
Curr Opin Neurol. 2003 Jun;16(3):351-7. doi: 10.1097/01.wco.0000073937.19076.d5.
Recently, central nervous system disorders have been shown to be associated with autoantibodies. This review summarizes the recent findings and assesses the evidence that these conditions are caused by the antibodies, using the criteria established for peripheral nervous system autoimmune diseases.
Over the last few years, antibodies to voltage-gated calcium and potassium channels, and to glutamate receptors, have been detected in the serum and cerebrospinal fluid of patients with ataxia, limbic encephalitis and certain forms of epilepsy. Some of these patients respond to immunotherapies, suggesting that the antibodies are pathogenic, but there are few demonstrations using the passive transfer approach that antibodies present in the serum can penetrate the blood-brain barrier and affect central nervous system function. Some patients have antibodies to intracellular proteins such as glutamic acid decarboxylase or specific ribonuclear proteins. The pathogenicity of these antibodies must be in some doubt, although intravenous immunoglobulin therapy has been shown to be beneficial in stiff man syndrome, consistent with an autoimmune aetiology for the disease. In only a few conditions, has IgG derived from patients been shown to produce pathogenic effects in vivo or in vitro.
There is much that needs to be done to define the role of these antibodies and to determine how they affect central nervous system function in vivo. These studies must be carried out so that appropriate treatments can be provided for the growing number of patients with possible antibody-mediated conditions.
最近研究表明，中枢神经系统疾病与自身抗体有关。本综述总结了近期研究结果，并根据外周神经系统自身免疫性疾病的既定标准，评估这些疾病由抗体引起的证据。
在过去几年中，共济失调、边缘性脑炎和某些类型癫痫患者的血清和脑脊液中检测到了针对电压门控钙通道和钾通道以及谷氨酸受体的抗体。其中一些患者对免疫疗法有反应，这表明这些抗体具有致病性，但很少有研究采用被动转移方法证明血清中的抗体能够穿透血脑屏障并影响中枢神经系统功能。一些患者体内存在针对细胞内蛋白如谷氨酸脱羧酶或特定核糖核蛋白的抗体。尽管静脉注射免疫球蛋白疗法已被证明对僵人综合征有益，这与该疾病的自身免疫病因一致，但这些抗体的致病性仍存在一定疑问。只有在少数情况下，患者来源的IgG在体内或体外显示出致病作用。
要确定这些抗体的作用以及它们如何在体内影响中枢神经系统功能，还有很多工作要做。必须开展这些研究，以便为越来越多可能患有抗体介导疾病的患者提供适当治疗。
