Abstract: In recent years, the antibody-associated limbic encephalitis (LE) has attracted attentions of more and more clinicians. The associated antibodies mainly act on neuronal cell surface antigens, including the N-methyl-D-aspartate (NMDA) receptor, the α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) receptor, the γ-aminobutyric acid B (GABA_B) receptor, leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein-like 2 (Caspr2) and so on. The clinical manifestation is primarily defined by the subacute onset of short-term memory loss, seizures, confusion and psychiatric symptoms suggesting the involvement of the limbic system. These severe and protracted disorders can affect children and young adults, occurring with or without tumor association. Routine detection of serum and cerebrospinal fluid (CSF) and imaging tests show no specificity, but associated antibodies can be detected in serum and (or) CSF. The patients respond well to tumor resection and immunotherapies, including corticosteroids, intravenous immunoglobulin (IVIg), plasma exchange or combination of them, but may relapse. This article aims to study the clinical features and treatment of antibody-associated limbic encephalitis and to improve the diagnosis and prognosis of these diseases.

Key words: Encephalitis, Limbic system, Receptors, N-methyl-D-aspartate, Receptors, GABA-B, Tumor suppressor proteins, Review

摘要： 新型边缘性脑炎近年来日益受到临床医师的关注，相关抗体主要作用于细胞表面抗原，包括N -甲基-D-天冬氨酸受体、α-氨基-3-羟基-5-甲基-4-异唑丙酸受体、γ-氨基丁酸B 型受体、富亮氨酸胶质瘤失活基因1 及接触蛋白相关样蛋白-2 等。该病主要累及儿童和青年，临床表现多样，病情严重，可合并或不合并肿瘤，常规血清学、脑脊液和影像学检查无特异性，若血清和（或）脑脊液检测到相关抗体可明确诊断。对糖皮质激素类药物、血浆置换和免疫球蛋白等免疫疗法，以及外科手术切除肿瘤灶反应良好，但存在复发的可能。本文对新型边缘性脑炎的临床特点和治疗原则进行综述，旨在提高临床诊断准确率和改善患者预后。

关键词: 脑炎, 边缘系统, 受体, N-甲基-D-天冬氨酸, 受体, GABA-B, 肿瘤抑制蛋白质类, 综述