抗GABA B型受体脑炎38例临床分析

Clinical features of 38 patients with anti GABA B receptor encephalitis

目的探讨抗GABA B型受体(GABABR)脑炎患者临床特征、治疗及预后。方法对2015年6月至2020年4月郑州大学第一附属医院神经内科收治的38例抗GABABR脑炎患者临床资料进行回顾性分析,采用mRS进行预后评估。结果38例患者中位发病年龄为62岁(15~84岁),男女比例26∶12。主要的临床表现包括痫性发作(33例,86.8%)、认知功能障碍(24例,63.1%)、精神行为异常(22例,57.8%)、意识障碍(12例,31.5%)。头颅MRI发现异常14例,13例累及单侧或双侧颞叶内侧。16例发现肿瘤,15例胸部CT提示肺癌,8例病理活检证实为小细胞肺癌。32例患者接受免疫治疗,26例(81.3%)对免疫治疗有反应,获得部分或完全神经功能改善。最后随访时,中位随访时间7个月(1~50个月),16例预后良好,18例预后不良,其中17例死亡,4例失访。结论抗GABABR脑炎多见于中老年男性患者,新发难治性痫性发作为其突出的临床特点,易合并肿瘤,应积极行肿瘤筛查,大多数患者对免疫治疗有反应。

Objective To explore the clinical features,treatment and prognosis of patients with anti GABA B receptor(anti GABABR)encephalitis.Methods The clinical data of 38 patients with anti GABABR encephalitis admitted to the First Affiliated Hospital of Zhengzhou University from June 2015 to April 2020 were retrospectively analyzed,and the mRS was used to evaluate the prognosis.Results Among the 38 patients,the median age of onset was 62 years(15-84 years),with a male to female ratio of 26∶12.The main clinical manifestations included seizures(33 cases,86.8%),cognitive dysfunction(24 cases,63.1%),mental and behavioral abnormalities(22 cases,57.8%),and disturbance of consciousness(12 cases,31.5%).Brain MRI revealed abnormalities in 14 cases,and 13 cases involved unilateral or bilateral medial temporal lobes.Tumors were found in 16 cases,chest CT showed lung cancer in 15 cases,and pathological biopsy confirmed 8 cases of small cell lung cancer.Thirty-two patients received immunotherapy,and 26 patients(81.3%)responded to immunotherapy,with partial or complete neurological improvement.At the last follow-up,the median follow-up time was 7 months(1-50 months),16 patients had good prognoses,18 patients had poor prognoses,of which 17 cases died,and 4 patients were lost to follow-up.Conclusions Anti GABABR encephalitis is more common in middle-aged and elderly male patients.New-onset refractory epilepsy are its prominent clinical features.It frequently accompanies tumor and tumor screening should be actively performed.Most patients respond to immunotherapy.