抗γ-氨基丁酸B受体脑炎临床特点分析  

Clinical features of anti-γ-aminobutyric acid B receptor encephalitis

作　　者：王灿灿 张元杏 杨辉丽 吴颖颖 时英英 高鑫雅 徐长水 赵建华 胡亚梅 史晓红 张杰文 

机构地区：[1]郑州大学人民医院神经内科,郑州450000

出　　处：《中华神经医学杂志》2017年第9期938-942,共5页Chinese Journal of Neuromedicine

摘　　要：目的分析抗γ-氨基丁酸B（GABA—B）受体脑炎患者的临床特点、影像学表现及预后情况。方法对自2015年9月至2017年3月郑州大学人民医院神经内科收治的13例抗GABA-B受体脑炎患者的临床资料进行回顾性分析。以改良的Rankin量表（mRS）评价预后，mRS≤2分定义为预后良好，mRS≥3分定义为预后不良。结果13例患者的平均发病年龄为58岁，男女比例为12：1，主要临床表现包括癫痫发作（12例）、精神行为异常（11例），认知功能障碍（7例），意识障碍（4例）等。5例患者头颅MRI异常，4例累及海马、杏仁体等边缘系统，1例累及脑桥及左侧颞叶。8例行PET-CT检查，5例提示代谢异常，其中4例表现为颞叶内侧高代谢，1例表现为全脑皮质低代谢。4例胸部CT提示肺部占位，其中2例经病理诊断为小细胞肺癌（SCLC）。3例患者合并副肿瘤神经抗体阳性。10例患者接受免疫调节治疗，3例仅给予对症支持治疗。电话随访12例患者1～20个月（平均8个月），7例预后良好；5例预后不良，其中2例合并肺癌。结论抗GABA-B受体脑炎多见于中老年男性，严重且难治的癫痫发作及边缘叶脑炎是该病主要的特点，免疫调节治疗有效，合并肿瘤者、副肿瘤抗体阳性者预后较差。Objective To analyze the clinical characteristics, imaging manifestations and prognoses of anti-GABAB receptor antibodies encephalitis. Methods The clinical manifestations, laboratory findings and radiological data of 13 patients with anti-GABAB receptor encephalitis, admitted to our hospital from September 2015 to March 2017, were retrospectively analyzed. Modified Rankin scale （mRs） was performed to evaluate the prognoses （good prognosis： mRs scores 〈 2; poor prognosis mRs scores ≥ 3）. Results These 13 patients had an average age of 58 years （ranged 49-76 years） with a male to female ratio of 12：1. The major clinical features, including epileptic seizure, were found in 12 patients, psychiatric symptoms in 11 patients, cognitive disorder in 7 patients, and disturbance of consciousness in 4 patients. Brain MR imaging showed abnormal signal in 5 patients： 4 were located in the hippocampus and amygdaloid, and one in the pons and left temporal lobe. Five patients showed abnormalities in PET-CT, including 4 with temporal hypermetabolism and 1 with cortical hypometabolism. Chest CT showed lung occupying lesions in 4 patients, of which 2 patients were diagnosed as having small cell lung cancer （SCLC） by pathological examination. Ten patients received immunomodulatory therapy, and three were with supportive care. After the average of 8 months of follow-up, 7 patients had good prognosis, 5 patients had poor prognosis and one patient lost of follow up. Conclusions Anti-GABAB receptor encephalitis frequently occurs in elderly male subjects and the main characteristic includes prominent refractory epilepsy and shows neurological improvement on immunotherapy. It can accompany by SCLC and have a relatively poor prognosis.

关 键 词：Γ-氨基丁酸 脑炎 癫痫 小细胞肺癌 

分 类 号：R742.9[医药卫生—神经病学与精神病学]