抗γ-氨基丁酸B型受体脑炎五例分析

A case series of five patients with anti-γ-aminobutyric acid type B receptor encephalitis

目的:探讨抗γ-氨基丁酸B型受体(GABA BR)脑炎患者的临床特点、治疗及预后。方法:回顾性总结2017年9月至2019年6月香港大学深圳医院神经内科诊治的5例抗GABA BR脑炎病例,分析其临床资料、辅助检查、治疗经过,随访3.5~23.0个月并评估预后。结果:5例抗GABA BR脑炎患者(19~81岁)均急性起病,以难治性癫痫为主要临床表现,其中4例颅脑磁共振成像显示颞叶及海马为主的T 2/液体衰减反转恢复序列高信号。脑电图均提示慢波或癫痫样放电。4例肺部发现占位,病理均确诊为小细胞肺癌。5例接受一线免疫抑制治疗(激素联合丙种球蛋白或血浆置换)效果均欠佳,3例接受了二线免疫治疗(利妥昔单抗、环磷酰胺),其中2例合并肿瘤者同时接受了肿瘤治疗,接受二线治疗及肿瘤治疗的患者疗效明显好于单纯一线治疗者。结论:抗GABA BR脑炎主要表现为以难治性癫痫为特征的边缘性脑炎综合征。一线治疗效果欠佳的抗GABA BR脑炎尽快启动二线治疗可明显改善预后。

Objective To investigate the clinical features,treatment and prognosis of anti-γ-aminobutyric acid type B receptor(GABAB R)encephalitis.Methods Retrospective analysis of five patients of anti-GABABR encephalitis from the Department of Neurology,the University of Hong Kong-Shenzhen Hospital from September 2017 to June 2019 was carried out.Clinical manifestations,auxiliary examination,and treatment were analyzed.The patients were followed up for 3.5-23.0 months to assess their prognosis.Results Five cases of anti-GABABR encephalitis(19-81 years old)presented acute onset,with refractory epilepsy as the main clinical manifestation.There were hyperintensive signals on T2/fluid attenuated inversion recovery in four patients′temporal lobe and hippocampus.Electroencephalogram showed slow wave or epileptic discharge;Lung mass was found in four patients,and all were small cell lung cancer.Five cases had poor response to first-line immunotherapy(intravenous use of pulse methylprednisolone,high dose immunoglobulin or plasma exchange),then three patients received second-line immunotherapy(rituximab,cyclophosphamide),two of whom with tumor also received tumor chemotherapy.Patients who received second-line treatment and tumor chemotherapy showed better outcome than those who only received first-line treatment.Conclusions Anti-GABABR encephalitis present with limbic encephalitis syndromes characterized by refractory epilepsy.For patients with poor response to first-line immunotherapy,initiating second-line immunotherapy as soon as possible can improve the prognosis significantly.