自身免疫性脑炎是自身免疫机制导致的脑部炎性疾病，其相关抗体可分为细胞内靶向抗原抗体和细胞表面靶向抗原抗体2 类。中枢神经系统特发性炎性脱髓鞘病（central nervous system idiopathic inflammatory demyelinating diseases，CNS-IIDDs）是一组免疫相关的特发于脑（包括视神经）和（或）脊髓的炎性脱髓鞘疾病，其与数种自身抗体（包括抗水通道蛋白4 抗体、抗髓鞘少突胶质细胞糖蛋白抗体等）相关。临床上，自身免疫性脑炎可与CNS-IIDDs 重叠。本文结合相关文献对自身免疫性脑炎和CNS-IIDDs 以及2者重叠的疾病特征进行总结，以期为临床医师提供相关诊治建议。如要明确2 者重叠的临床特征和发病机制，还需开展更多高质量的研究。

Autoimmune encephalitis (AE) is a relatively new category of immune-mediated disease involving the central nervous system. AE-related antibodies can be divided into two groups: antibodies targeting intracellular neuronal antigens and antibodies targeting cell-surface neuronal antigens. Central nervous system idiopathic inflammatorydemyelinating diseases (CNS-IIDDs) is an immune-related group of inflammatory demyelination diseases especially occurring in the brain (including the optic nerve) and/or spinal cord. It is related to several autoantibodies (including anti-aquaporin 4 antibodies and anti-myelin oligodendrocyte glycoprotein antibodies). AE may co-exist with CNS-IIDDs. In this paper, the characteristics of AE, CNS-IIDDs and AE co-existing with CNS-IIDDs were reviewed in combination with relevant literature, in order to provide relevant diagnosis and treatment recommendations for clinicians. However, more high-quality studies are needed to confirm its pathogenesis and clinical features.