抗γ-氨基丁酸B受体脑炎临床特征及预后分析

目的总结并分析12例抗γ-氨基丁酸B(GABAB)受体脑炎患者的临床特征、实验室检查、影像学、治疗及预后。方法分析2015年2月至2018年8月郑州大学第一附属医院收治的12例抗GABAB受体脑炎患者的临床资料,用改良Rankin量表(mRS)评估疾病预后。结果12例均以癫痫发作为首发症状,其他主要临床表现有认知障碍、精神行为异常、意识障碍等。12例中6例(50.0%)合并其他自身抗体阳性(抗甲状腺抗体3例,1例抗核抗体、1例抗Ro52抗体1例抗Amphiphsin抗体);7例(58.3%)头颅MRI异常,以海马和颞叶最易受累;6例(50.0%)合并肺癌,其中3例在入院中即发现,3例随访时发现,5例病理证实为小细胞肺癌。12例患者均急性期给予免疫调节及抗癫痫等对症支持治疗,8例完全缓解,4例部分缓解,中位随访18个月,5例预后较好,6例死亡,1例失访。结论癫痫发作是抗GABAB受体脑炎最常见临床表现,免疫治疗可改善疾病预后。缓解期应重视肿瘤的筛查。

The clinical features and prognosis of anti-γaminobutyric acid B recepter encephalitis

Objective To summarize and analyze the clinical features,laboratory examinations,imaging,treatment,and prognosis of 12 patients with anti-γ-aminobutyric acid B(GABAB)receptor encephalitis.Methods The clinical data of 12 patients with anti-GABAB receptor encephalitis admitted to the First Affiliated Hospital of Zhengzhou University from February 2015 to August 2018 were collected.The prognosis was evaluated by modified Rankin scale(mRS).Results All the patients have seizureas the initial presentation in our cohort.Other clinical presentations included cognitive impairments,mental behavioral abnormalities,and disturbance of consciousness.The 6 paients had additional autoantibodies(3 anti-thyroid、1 anti-nuclear,1 anti-Ro52,1 anti-Amphiphsin).The brain MRI were found be abnormal in 7 patients.The hippocampus and temporal lobe were most commonly affected.Lung cancer were found in 6 patients,including 3 cases detected at onset and 3 cases during follow-up,5 of them had small cell lung cancer by biopsy.All the patients were treated with immune modulation and antiepileptic therapies in the acute phase,8 patients had complete remission and 4 patients had partial remission.After a median follow-up of 18 months,6 patients died,5 patients had a good prognosis,and one patient was lost during the extended follow-up.Conclusions Seizure is the most common clinical manifestation of anti-GABAB receptor encephalitis.Immunotherapy can improve the prognosis of the disease,and the screening of tumors should be emphasized during remission.