Continuum (Minneap Minn). 2024 Aug 1;30(4):960-994. doi: 10.1212/CON.0000000000001447.
The field of autoimmune neurology is rapidly evolving. This article reviews the epidemiology and pathophysiology as well as current approaches to clinical and paraclinical assessment, testing paradigms, and general principles of treatment.
Improved recognition of autoimmune diagnoses among patients who have phenotypically diverse, subacute onset neurologic presentations is facilitated by disease-specific antibody biomarker discovery. These antibodies have varying associations with paraneoplastic causation (from no association to greater than 70% positive predictive value), immunotherapy responses, and outcomes. To simplify assessment in an increasingly complex discipline, neurologic phenotype-specific serum and CSF antibody evaluations are recommended. Clinical trials have led to the approval of monoclonal therapies for neuromyelitis optica spectrum disorder (NMOSD) and are underway for N-methyl-d-aspartate (NMDA) receptor and leucine-rich glioma inactivated protein 1 (LGI1) encephalitides.
Autoimmune neurology is now a mainstream subspecialty, consisting of disorders with diverse presentations detectable using antibody testing of serum and CSF. Early and sustained immunotherapy (eg, corticosteroids, intravenous immunoglobulin [IVIg], plasma exchange) is recommended and may be supplemented by immune suppressants (eg, rituximab or cyclophosphamide) to sustain responses and optimize outcomes.
自身免疫性神经病学领域正在迅速发展。本文综述了其流行病学和病理生理学，以及目前临床和辅助评估、检测模式和治疗一般原则。
通过疾病特异性抗体生物标志物的发现，有助于提高对表现出多种亚急性发作性神经表现的患者的自身免疫性诊断的认识。这些抗体与副肿瘤病因（从无关联到阳性预测值大于 70%）、免疫治疗反应和结果的相关性各不相同。为了简化日益复杂的学科中的评估，推荐进行针对特定神经表型的血清和 CSF 抗体评估。临床试验导致批准了针对视神经脊髓炎谱系疾病（NMOSD）的单克隆疗法，并且正在进行 N-甲基-D-天冬氨酸（NMDA）受体和富亮氨酸胶质瘤失活蛋白 1（LGI1）脑炎的临床试验。
自身免疫性神经病学现在是一个主流的亚专科，包括使用血清和 CSF 抗体检测可检测到的多种表现的疾病。建议早期和持续的免疫治疗（例如，皮质类固醇、静脉注射免疫球蛋白[IVIg]、血浆置换），并可通过免疫抑制剂（例如利妥昔单抗或环磷酰胺）来维持反应并优化结果。
