王丽 张颖 王婷婷 田小夫 陈会生

[摘要] 目的 總结和分析20例自身免疫性脑炎临床表现、实验室及影像学特点、治疗及预后。 方法 回顾性分析2013年4月至2019年7月北部战区总医院确诊为自身免疫性脑炎患者的临床资料。用SPSS 22.0统计学软件对治疗前及随访时改良Rankin量表评分进行分析。 结果 20例自身免疫性脑炎均急性或亚急性起病，临床多以精神异常、癫痫、记忆力减退为首发症状，可出现言语运动障碍、自主神经异常、意识水平下降等症状。3例分别合并亚临床甲状腺功能减低、自身免疫性溶血性贫血、系统性红斑狼疮。2例病理为恶性畸胎瘤。1例病理为小细胞肺癌。1例增强肺CT提示：右肺占位病变。常规脑脊液检查大多数正常。头影像学多以双侧或单侧边缘系统炎症改变。脑电图：发作尖波或慢波，抗NMDAR脑炎可出现异常delta刷。部分患者可复发，复发症状较前轻。经免疫治疗和对症支持治疗后：治疗前中位改良Rankin量表评分为3（3，5）分，随访时中位改良Rankin量表评分为1（0，2）分，差异有统计学意义（Z=3.296，P<0.05）。 结论 自身免疫性脑炎主要表现为边缘叶脑炎或弥漫性脑炎。少数病例可复发。脑脊液大多数正常，抗NMDAR脑炎脑电图可出现delta刷。影像学多为边缘系统炎症改变，可伴有良性或自身免疫性疾病、恶性肿瘤。早期诊断及给予免疫治疗预后较好。

[关键词] 脑炎;自身免疫性脑炎;副肿瘤综合征;免疫治疗

[中图分类号] R512.3          [文献标识码] B          [文章编号] 1673-9701（2021）07-0050-04

Clinical characteristics of 20 cases of autoimmune encephalitis

WANG Li1   ZHANG Ying2   WANG Tingting1   TIAN Xiaofu1   CHEN Huisheng1

1.Department of Neurology， General Hospital of Northern Theater Command， Shenyang   110016， China; 2.Tieling Health Vocational College， Tieling   112008， China

[Abstract] Objective To summarize and analyze the clinical manifestations， laboratory and imaging features， treatment and prognosis of 20 cases of autoimmune encephalitis. Methods The clinical data of patients with autoimmune encephalitis diagnosed in General Hospital of Northern Theater Command from April 2013 to July 2019 were analyzed retrospectively. SPSS 22.0 statistical software was used to analyze the scores of modified Rankin scale before treatment and during follow-up. Results 20 cases of autoimmune encephalitis had an acute or subacute onset， with clinical abnormalities， epilepsy， and memory loss as the first symptoms， which may include speech-movement disorders， autonomic abnormalities， and decreased level of consciousness. Three patients were complicated with subclinical hypothyroidism， autoimmune hemolytic anemia， and systemic lupus erythematosus， respectively. Two cases had pernicious teratoma pathology. One case was pathologically small cell lung cancer. One case of enhanced lung CT showed that the right lung occupied lesions. Routine cerebrospinal fluid examination was mostly normal. Head imaging is often characterized by bilateral or unilateral inflammatory changes in the limbic system. Electroencephalogram： sudden sharp wave or slow wave， abnormal delta brush may appear in anti-NMDAR encephalitis. Some patients can relapse， and the symptoms of relapse were lighter than before. After immunotherapy and symptomatic support， the median score of modified Rankin scale before treatment was 3（3， 5） points， and the median score of modified Rankin scale during follow-up was 1（0， 2） points， with statistically significant difference（Z=3.296， P<0.05）. Conclusion Autoimmune encephalitis is mainly manifested as marginal lobe encephalitis or diffuse encephalitis. A few cases can recur. Cerebrospinal fluid is mostly normal， and delta brush can appear in EEG of anti-NMDAR encephalitis. Imaging is mostly inflammatory changes of marginal system， which may be accompanied by benign or autoimmune diseases and malignant tumors. Early diagnosis and immunotherapy will produce better prognosis.

[Key words] Encephalitis; Autoimmune encephalitis; Paraneoplastic syndrome; Immunotherapy

自身免疫性腦炎（Antoimmune encephalitis，AE）指通过体液免疫机制引起的弥漫性或多发性脑实质相对可逆的神经元功能障碍。2007年发现抗N-甲基-D-天冬氨酸受体（N-methyl-D-aspmtate receptor，NMDAR）脑炎[1]，此后抗富含亮氨酸经典的胶质瘤失活蛋白l（Anti-leucine rich glioma inactivated protein 1，LGIl）抗体、抗γ氨基丁酸B型受体（Anti-gamma-aminobutyric-acid B receptor，GABAbR）抗体、α氨基-3-羟基-5-甲基-4-异噁哇丙酸受体（Alpha-amino-3-hydroxy-5-methyl-4-Isoxazolepropionic acid receptor，AMPAR）抗体等相关AE陆续报道。AE临床表现多样，部分重症AE需住重症监护病房，病死率为4.0%～5.2%[2]。……