自身免疫性脑炎

Autoimmune Encephalitis.

作者信息

Guasp Mar, Dalmau Josep

机构信息

Neuroimmunology Unit, Department of Neurology, Hospital Clínic de Barcelona, University of Barcelona, C/ Casanova, 143; Floor 3A, Barcelona 08036, Spain; Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS)-CaixaResearch Institute, Barcelona, Spain; Centro de Investigación Biomédica en red, enfermedades raras (CIBERER), Madrid, Spain.

出版信息

Med Clin North Am. 2025 Mar;109(2):443-461. doi: 10.1016/j.mcna.2024.09.001. Epub 2025 Jan 2.

DOI:10.1016/j.mcna.2024.09.001

PMID:39893022

Abstract

Autoimmune encephalitides (AE) constitute a broad group of inflammatory brain disorders characterized by prominent neuropsychiatric symptoms, frequently in association with autoantibodies against neural (neuronal or glial) antigens. The most frequent AE are anti-NMDA receptor encephalitis, acute disseminated encephalomyelitis (associated with MOG antibodies in 60% of patients), and limbic encephalitis (with several immunologic subtypes, anti-LGI1 encephalitis being the most frequent). The first 2 predominantly affect children and young adults, whereas limbic encephalitis usually affects patients older than 50 years. Despite the severity of symptoms, prompt diagnosis and treatment lead to substantial recovery in most patients.

摘要

自身免疫性脑炎（AE）是一组广泛的炎症性脑疾病，其特征是突出的神经精神症状，常与针对神经（神经元或神经胶质）抗原的自身抗体相关。最常见的AE是抗N-甲基-D-天冬氨酸（NMDA）受体脑炎、急性播散性脑脊髓炎（60%的患者与髓鞘少突胶质细胞糖蛋白抗体相关）和边缘叶脑炎（有几种免疫亚型，抗富含亮氨酸胶质瘤失活1蛋白[LGI1]脑炎最为常见）。前两种主要影响儿童和年轻人，而边缘叶脑炎通常影响50岁以上的患者。尽管症状严重，但及时诊断和治疗可使大多数患者显著康复。