摘要： 目的 分析不同类型自身免疫性脑炎病程中癫痫发作特征，筛查具有早期诊断价值的标志物。方法 收集2014 年8 月至2021 年1 月于华中科技大学同济医学院附属同济医院就诊的91 例自身免疫性脑炎伴癫痫发作患者的社会人口学资料、发病特点、脑电图与影像学特征、治疗与预后等临床资料。结果 （1）抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎（58 例）、抗γ-氨基丁酸B 型受体（GABA_BR）抗体相关脑炎（12 例）和抗富亮氨酸胶质瘤失活蛋白1（LGI1）抗体相关脑炎（12 例）是自身免疫性脑炎的常见类型。（2）快速进展性痴呆［79.12%（72/91）］、睡眠障碍［79.12%（72/91）］和精神障碍［75.82%（69/ 91）］为主要非癫痫症状，而情绪障碍或言语障碍少见。（3）脑炎病程中76.92%（70/91）患者出现全面性强直-阵挛发作，为抗 GABA_BR 抗体相关脑炎（12/12）的特征性发作类型；而面-臂肌张力障碍发作则是抗 LGI1 抗体相关脑炎［4.40%（4/91）］的特征性发作类型。（4）视频脑电图主要表现为背景慢波［37.88%（25/ 66）］、慢波合并痫样放电［27.27%（18/66）］或痫样放电［18.18%（12/66）］，但快波活动增加［1.52%（1/66）］少见，亦可无异常脑电波［15.15%（10/66）］；δ 刷仅见于 2 例抗 NMDAR 脑炎患者。（5）MRI 异常［58.89%（53/90）］以海马、颞叶T₂WI 和FLAIR 成像高信号为主，尤以抗LGI1 抗体相关脑炎（9/12）多见。（6）治疗方案首选免疫治疗［97.80%（89/91）］和抗癫痫发作药物［94.51%（86/91）］。平均随访48.50 个月，病死率14.29%（10/70），以抗GABA_BR 抗体相关脑炎最高（4/9）；癫痫复发率为10.17%（6/59），抗DPPX 抗体相关脑炎患者远期复发率相对较高（2/3）。结论 不同类型伴癫痫发作的自身免疫性脑炎具有特定的临床特征，对早期诊断、判断预后具有重要意义。

关键词: 脑炎, 自身免疫性疾病, 癫痫, 脑电描记术, 磁共振成像

Abstract: Objective To analyze the seizure characteristics of epilepsy in the course of different types of autoimmune encephalitis (AE), and to screen the markers of early diagnosis value. Methods A total of 91 patients with AE with epileptic seizure admitted to Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology from August 2014 to January 2021 were collected. The clinical data included social demographic data, pathogenic characteristics, EEG and imaging characteristics, treatment and prognosis. Results 1) Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis (n = 58), anti-γ-aminobutyric acid receptor type B (GABA_BR) antibody-associated encephalitis (n = 12) and anti-leucine rich glioma inactivated protein 1 (LGI1) antibody-associated encephalitis (n = 12) were common types of AE. 2) Rapid progressive dementia [79.12% (72/91)], sleep disorders [79.12% (72/91)] and mental disorders [75.82% (69/91)] were the main non-epileptic symptoms, but mood disorders or speech disorders were rare. 3) In the course of encephalitis, 76.92% (70/91) of patients had generalized tonic-clonic seizure (GTCS), which were the characteristic seizure type of anti-GABA_BR antibody-associated encephalitis (12/12). While faciobrachial dystonic seizure (FBDS) was the specific seizure type of anti-LGI1 antibody-associated encephalitis [4.40% (4/91)]. 4) The main manifestations of VEEG were background slow waves [37.88% (25/ 66)], slow waves combined with epileptic discharges [27.27% (18/66)] or epileptic discharges [18.18% (12/ 66)], but the increase of fast wave activity [1.52% (1/66)] was rare. There was also no abnormal brain wave [15.15% (10/66)]; δ brush was observed in only 2 patients with anti-NMDAR encephalitis. 5) MRI abnormalities [58.89% (53/90)] were mainly found in hippocampus amd temporal lobe, characterized by T₂WI and FLAIR hyperintersity, mainly in patients with anti-LGI1 antibody-associated encephalitis (9/12). 6) Immunotherapy [97.80% (89/91)] and antiepileptic seizure medicine [94.51% (86/91)] were the preferred treatment options. The mean follow-up was 48.50 months, and the fatality rate was 14.29% (10/70), with the highest rate of anti-GABA_BR antibody-associated encephalitis (4/9). The recurrence rate of epilepsy was 10.17% (6/59), and the long-term recurrence rate of anti-DPPX antibody-associated encephalitis was relatively high (2/3). Conclusions Different types of AE with epileptic seizure have specific clinical characteristics, which is of great significance for early diagnosis and prognosis.

Key words: Encephalitis, Autoimmune diseases, Epilepsy, Electroencephalography, Magnetic resonance imaging