21例抗GABABR脑炎的临床特征分析  

Clinical Characteristica of 21 Cases Series with Anti-γ-aminobutyric Acid B Receptor Encephalitis

作　　者：冯雪丹[1] 于莎莎 李阳 张玉婧 王怀璐 FENG Xuedan;YU Shasha;LI Yang(Department of Neurology,Beijing Youanmen Hospital,Beijing100069,China)

机构地区：[1]北京丰台右安门医院神经内科,北京100069

出　　处：《中风与神经疾病杂志》2020年第9期801-805,共5页Journal of Apoplexy and Nervous Diseases

基　　金：北京市丰台区卫生健康系统科研项目(No.2017-83)。

摘　　要：目的总结分析21例抗γ-氨基丁酸B受体脑炎(抗GABABR脑炎)的临床特点、治疗方案及临床预后。方法回顾性收集北京丰台右安门医院神经内科2012年10月至2019年12月连续收治的抗γ-氨基丁酸B受体脑炎住院患者的临床资料。通过门诊复诊或电话进行随访,良好预后定义为mRS评分≤2分。分析病例的临床特征、治疗及预后转归。结果共纳入21例患者,中位年龄为57(11~77)岁,男性14例,女性7例。主要临床症状表现为癫痫发作21例,认知障碍19例,精神症状19例,语言障碍/缄默14例,睡眠障碍13例,意识水平下降13例,不自主运动7例,自主神经功能障碍6例,共济失调1例,9例伴发肺癌。脑电图异常11例(11/14),头颅MRI异常12例(12/21)主要累及颞叶。21例患者血清及脑脊液GABABR抗体检测均阳性。17例给予一线免疫治疗,除3例显示治疗无效外,其他患者均治疗反应良好。中位随访16(3~30)m期间,11例预后良好,9例死亡,4例复发。结论本组抗GABAB受体脑炎发病以中老年男性多见,临床症状主要表现为边缘性脑炎症状,以癫痫和神经精神障碍症状为主,癫痫主要为难治性癫痫,患者合并肿瘤的比例高,尤其肺癌。多数患者对免疫治疗反应良好、预后较佳,合并肺癌的患者预后较差。Objective To summarize the clinical characteristics,treatment and outcome of 21 patients with anti-γ-Aminobutyric acid-B receptor(GABA-B)encephalitis.Methods The clinical data of inpatients with anti-GABABR encephalitis at Neurology Department of Beijing Youanmen Hospital from October 2012 to December 2019 were retrospectively collected.all patients were followed up by outpatient visits or telephone.The good prognosis was defined as a mRS of 0 to 2 points.The clinical feature,treatment and followed-up outcome were analyzed.Results A total of 21 patients were included in this analysis,with a median age of 57 years(range:11~77 years)and 14 males.The major clinical manifestations included seizures(21/21),cognitive impairment(19/21),psychiatric symptoms(19/21),involuntary movement(7/21),speech impairment/silence(14/21),sleep disorders(13/21),disturbance of consciousness(13/21),involuntary movement(7/21),autonomic nervous dysfunction(6/21)and ataxia(1/21).Lung cancer was detected in 9(9/21)patients.Electroencephalogram(EEG)showed mild to moderate abnormal changes in 11(11/14)cases.The head magnetic resonance imaging showed abnormal signals in 12(12/21)cases,with the most involved area in temporal lobe.All the 21 patients showed anti-GABABR positive in serum and cerebrospinal fluid.17 patients received the first-line immunotherapy,except 3 patients showing no effect,all the other patients responded well to the treatment.During a median follow-up of 16(range:3~30 months),11 cases had good prognosis,9 cases died,and 4 cases had recurrent encephalitis.Conclusion The cases series with anti-GABABR encephalitis and limbic encephalitis presentation were more common in middle-aged and elderly men.The main clinical symptoms were epilepsy and neuropsychiatric disorders,and most epilepsy were refractory epilepsy.The proportion of concomitant tumor in the patients with this disorder was high,especially lung cancer.Most of these patients responded well to immunotherapy and had a good prognosis,and the patients with lung cancer had

关 键 词：抗GABABR脑炎 边缘性脑炎 自身免疫 脑脊液 肺癌 

分 类 号：R373.31[医药卫生—病原生物学]