Yucel Yavuz, Sidow Nor Osman, Yilmaz Ahmet
Department of Neurology, Dicle University Faculty of Medicine, Diyarbakir, Turkey.
Department of Neurology, Mogadishu Somalia-Türkiye Training and Research Hospital, Mogadishu, Somalia.
Medicine (Baltimore). 2025 May 23;104(21):e42472. doi: 10.1097/MD.0000000000042472.
Autoimmune encephalitis (AE) is an inflammatory disease of the brain parenchyma that is mediated by many specific autoantibodies and is not caused by bacterial or viral causes. A diverse and growing number of autoantibodies have been identified in association with different types of AE. These antibodies can target either intracellular or cell-surface antigens. Advances are being made in understanding the clinical spectrum and treatment of AE. The prevalence and incidence of AE are increasing, although they remain comparable to those of infectious etiologies. The clinical presentation and management of AE are complex, with overlapping features. AE should be considered in the differential diagnosis of treatment-resistant epilepsy. Prompt diagnosis and treatment are critical in preventing seizures from developing into epilepsy. However, the broad differential diagnosis, the inability to detect specific autoantibodies in every patient, and delays in receiving antibody test results impede early diagnosis and treatment. Immunosuppressive agents are primarily used in treatment; first-line options include corticosteroids, intravenous immunoglobulin, and plasmapheresis, while rituximab and cyclophosphamide are used as second-line treatments. This review aims to provide a concise and accessible summary of this topic for readers and researchers.
自身免疫性脑炎（AE）是一种脑实质的炎症性疾病，由多种特异性自身抗体介导，并非由细菌或病毒引起。与不同类型的AE相关的自身抗体种类繁多且不断增加。这些抗体可靶向细胞内或细胞表面抗原。在理解AE的临床谱和治疗方面正在取得进展。AE的患病率和发病率正在上升，尽管仍与感染性病因相当。AE的临床表现和管理很复杂，存在重叠特征。在治疗难治性癫痫的鉴别诊断中应考虑AE。及时诊断和治疗对于防止癫痫发作发展为癫痫至关重要。然而，广泛的鉴别诊断、无法在每个患者中检测到特异性自身抗体以及抗体检测结果的延迟都阻碍了早期诊断和治疗。免疫抑制剂主要用于治疗；一线选择包括皮质类固醇、静脉注射免疫球蛋白和血浆置换，而利妥昔单抗和环磷酰胺用作二线治疗。本综述旨在为读者和研究人员提供关于该主题的简洁易懂的总结。
