Linnoila Jenny, Pittock Sean J
Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts.
Department of Laboratory Medicine/Pathology, Mayo Clinic, College of Medicine, Rochester, Minnesota.
Semin Neurol. 2016 Aug;36(4):382-96. doi: 10.1055/s-0036-1585453. Epub 2016 Sep 19.
Autoimmune neurology is a rapidly evolving new subspecialty driven by the discovery of novel neural- (neuronal- or glial-) specific autoantibodies and their target antigens. The neurologic manifestations affecting the central nervous system include encephalitis, dementia, epilepsy, and movement and sleep disorders. Laboratory testing is now available for most of these neural-specific autoantibodies, which serve as diagnostic markers, in some instances directing the physician toward specific cancer types (e.g., N-methyl-D-aspartic acid receptor antibodies for teratoma, collapsin response mediator protein 5 for small-cell lung cancer) and assisting in therapeutic decision making. Antibodies targeting intracellular proteins serve as markers of cytotoxic effector T-cell-mediated injury, which is generally poorly responsive to immunotherapy. By contrast, antibodies targeting extracellular plasma membrane proteins may act as pathogenic effectors and often infer good responses to immunotherapy. Diagnosing these conditions and implementing treatment as early into the clinical course as possible ensures the best possible clinical outcomes. An adequate immunotherapy trial to assess maximum reversibility of symptoms, as assessed through objective functional measures, is crucial and can help to determine whether maintenance therapy is needed.
自身免疫性神经病学是一个迅速发展的新亚专业，由新型神经（神经元或神经胶质）特异性自身抗体及其靶抗原的发现所推动。影响中枢神经系统的神经学表现包括脑炎、痴呆、癫痫以及运动和睡眠障碍。现在大多数这些神经特异性自身抗体都可以进行实验室检测，这些抗体可作为诊断标志物，在某些情况下指导医生识别特定的癌症类型（例如，针对畸胎瘤的N-甲基-D-天冬氨酸受体抗体、针对小细胞肺癌的塌陷反应介导蛋白5），并协助进行治疗决策。靶向细胞内蛋白的抗体是细胞毒性效应T细胞介导损伤的标志物，这种损伤通常对免疫治疗反应不佳。相比之下，靶向细胞外质膜蛋白的抗体可能作为致病效应物，并且通常预示对免疫治疗有良好反应。尽早诊断这些疾病并在临床过程中尽早实施治疗可确保获得最佳临床结果。通过客观功能指标评估，进行充分的免疫治疗试验以评估症状的最大可逆性至关重要，并且有助于确定是否需要维持治疗。
