抗γ-氨基丁酸B受体脑炎11例临床分析  

Anti-gamma-aminobutyric acid-B receptor encephalitis in 11 cases

作　　者：赵盼 陈亚楠 杨世俊 李明敏 赵婷 王娜 张悦 时英英 张杰文 韩雄 ZHAO Pan;CHEN Yanan;YANG Shijun;LI Mingmin;ZHAO Ting;WANG Na;ZHANG Yue;SHI Yingying;ZHANG Jiewen;HAN Xiong(Department of Neurology,Henan Provincial People's Hospital,Zhengzhou University People's Hospital,Zhengzhou 450003,China)

机构地区：[1]河南省人民医院神经内科,郑州大学人民医院,郑州450003

出　　处：《中华实用诊断与治疗杂志》2020年第12期1228-1230,共3页Journal of Chinese Practical Diagnosis and Therapy

基　　金：河南省医学科技攻关计划省部共建项目(SB201901074)。

摘　　要：目的探讨抗γ-氨基丁酸B(gamma-aminobutyric acid-B,GABAB)受体脑炎患者的临床特点、实验室检查结果、影像学特征、脑电图表现、治疗及预后情况。方法回顾性分析11例抗GABAB受体脑炎患者的临床资料。结果11例患者均表现有边缘叶脑炎的症状,其中癫痫发作10例,记忆力减退4例,精神行为异常10例,组织病理检查确诊为小细胞肺癌1例,影像学检查提示为肺部恶性肿瘤2例。脑脊液抗GABAB受体抗体检测阳性2例,弱阳性1例,阴性8例;血清抗GABAB受体抗体检测阳性9例,弱阳性2例。11例中4例头颅MRI异常,表现为海马或内侧颞叶的异常信号。脑电图表现为弥漫性慢活动增多,或颞区局灶性慢波或痫样放电,但未发现明显的特征性脑电模式。11例中2例单纯行抗癫痫药物治疗,9例同时接受免疫治疗,其中6例明显缓解,1例部分缓解,4例无效;7例随访3~24个月,其中预后良好5例,预后不良2例。结论抗GABAB受体脑炎可伴发肿瘤,对新发难治癫痫、认知功能障碍、精神行为异常等边缘性脑炎症状的患者应考虑抗GABAB受体脑炎的可能,及时应用免疫治疗对改善预后有积极作用。Objective To investigate the clinical features,laboratory results,imaging features,electroencephalogram finding,therapy and prognosis of anti-gamma-aminobutyric acid-B(GABAB)receptor encephalitis.Methods The clinical data of 11 patients with anti-GABAB receptor encephalitis were retrospectively analyzed.Results All patients were demonstrated with limbic encephalitis,in which there were 10 cases of epilepsy,4 cases of memory impairment,10 cases of mental behavioral abnormalities,1 case of histopathologically confirmed small cell lung cancer,and 2 cases of imaging diagnosed malignant lung tumor.Cerebrospinal fluid anti-GABAB receptor antibody test was positive in 2 cases,weakly positive in 1 case and negative in 8 cases.Serum anti-GABAB receptor antibody test was positive in 9 cases and weakly positive in 2 cases.MRI scan of the brain showed abnormal signal in 4 cases,which mainly involved the medial temporal lobe or the hippocampus.The electroencephalogram showed increased diffuse slow activity,focal slow wave or epileptiform discharge in the temporal region,but no obvious characteristic electroencephalogram pattern was found.Among these 11 patients,2 received antiepileptic drug therapy alone and the other 9 received immunotherapy simultaneously,among which 6 had significant remission,1 had partial remission and 4 had no effect.Seven patients were followed up for 3 to 24 months,in which 5 had good prognosis and 2 had poor prognosis.Conclusion Anti-GABAB receptor encephalitis may accompany tumors,and it should be suspected when the patients are found newly-onset intractable epilepsy,cognitive impairment,abnormal mental behaviors and other symptoms of limbic encephalitis.Prompt initiation of immunotherapy may have positive effect on the prognosis.

关 键 词：自身免疫性脑炎 抗γ-氨基丁酸B受体脑炎 脑电图 

分 类 号：R742.9[医药卫生—神经病学与精神病学]