30例抗γ氨基丁酸B受体脑炎临床特点分析

Clinical characteristics of 30 patients with gamma-aminobutyric acid-B receptor(GABAB)encephalitis

目的探讨抗GABA BR抗体脑炎的临床表现、脑脊液、头部MRI及预后特点。方法收集我院2015年12月-2019年6月收治的抗GABA BR抗体脑炎患者30例,总结其临床表现、脑脊液、头部MRI特点,并进行随访,评估预后。结果30例患者平均发病年龄为58岁(15~84岁),男性18例,女性12例。癫痫发作为最常见的首发症状,并以癫痫发作(26例,86.67%)、精神行为异常(26例,86.67%)、认知功能损害(24例,80.00%)为主要临床表现。脑脊液以淋巴细胞比例及白细胞计数升高为主要特点。头部MRI可见内侧颞叶、海马异常信号(10例,35.71%)。易合并肺癌(特别是小细胞肺癌),在平均随访11m后近半数患者死亡(13例,48.15%),死亡患者大多合并肺癌(9例,69.23%)。结论抗GABA BR抗体脑炎主要表现为癫痫发作,影像学可见颞叶内侧、海马异常信号,同肺癌(小细胞肺癌)密切相关,近半数患者预后不良。

Objective To explore the characteristics of clinical presentations,cerebrospinal fluid(CSF),magnetic resonance imaging(MRI)and prognosis of the anti-GABA-B receptor encephalitis.Methods The clinical data of 30 patients with anti-GABA-B receptor encephalitis were collected.To analysis the characteristics of clinical features,CSF,cranial MRI features and prognosis.Results Among the 30 patients,eighteen were male,and the mean age at presentation were 58 years(range:15~84 years).Seizure was the most common initial symptom.The major clinical features include seizure(26,86.67%),mental and behavioral disorders(26,86.67%),cognitive dysfunction(24,80.00%).CSF cytology test showed that the lymphocytic pleocytosis and the elevated white blood cell counts can be indicative of this disease.Magnetic resonance imaging showed abnormality in the medial temporal lobe and hippocampus in ten patients(35.71%).The disease is closely related to lung cancer(Especially small cell lung cancer,SCLC).During a median follow-up period of 11 months,thirteen patients died.Nine of the patients died with tumors.Conclusions The major clinical features of anti GABA-B encephalitis was seizures.Inflammatory response signals in the medial temporal lobe and hippocampus are image features of this disease.It is closely related to lung cancer(Especially small cell lung cancer,SCLC).Almost half of the patients had poor outcome.