抗γ-氨基丁酸B受体脑炎患者的临床特征分析  

The characteristics of clinical manifestations in patients with anti-γ-aminobutyric acid B receptor encephalitis

作　　者：张隽[1] 张阵[1] 樊春秋[1] 吉训明[2] 黄小钦[1] 

机构地区：[1]首都医科大学宣武医院神经内科,北京100053 [2]首都医科大学宣武医院神经外科,北京100053

出　　处：《中华神经科杂志》2016年第6期439-444,共6页Chinese Journal of Neurology

摘　　要：目的 分析我国成年人抗γ-氨基丁酸B（GABA-B）受体脑炎患者的临床特征和预后特点.方法 收集2013年3月至2015年12月首都医科大学宣武医院收治的12例抗GABA-B受体脑炎患者,总结其临床症状、实验室检查、脑电图检查、影像学资料,并进行分析.结果 12例抗GABA-B受体脑炎患者主要临床表现为癫痫发作12例,认知障碍10例,精神行为异常10例,睡眠障碍3例,视幻觉2例,不自主运动2例,意识障碍3例,小脑体征2例,发热2例.12例患者脑脊液及血清抗GABA-B受体抗体均阳性.4例患者显示中度及以上异常脑电图,出现癫痫异常放电.头颅MRI显示异常信号9例,病灶主要累及海马、颞叶、侧脑室旁、丘脑.有6例患者行PET-CT及SPECT检查,其中5例提示海马、颞叶葡萄糖代谢轻中度减低.平均随访14个月后,9例预后良好,完全恢复;3例预后不良,其中2例经病理活体组织检查诊断为小细胞肺癌.结论 抗GABA-B受体脑炎主要临床表现为癫痫发作、认知障碍和精神异常.MRI病灶分布不仅局限于边缘叶,早期诊断并免疫调节治疗预后较好.Objective To analyze the clinical features and prognosis in adult Chinese patients with anti-γ-aminobutyric acid B receptor （GABA-BR） encephalitis.Methods We reviewed the clinical manifestations,cerebrospinal fluid （CSF） examinations,brain magnetic resonance imaging and prognosis of 12 patients who were diagnosed as anti-GABA-BR encephalitis in Capital Medical University Xuanwu Hospital from March 2013 to December 2015.Results The major clinical features of anti-GABA-BR encephalitis patients included seizures （12/12）,cognitive disorder （10/12）,psychiatric symptoms （10/12）,sleep disorders （3/12）,visual hallucination （2/12）,involuntary movements （2/12）,decreased consciousness （3/12）,cerebellar signs （2/12）,fever （2/12）.GABA-BR-antibody was positive in CSF and serum of all the patients.Electroencephalogram revealed epileptic discharges in 4/12 patients.Brain MRI showed abnormal signal in up to 9/12 patients,located in the hippocampus,temporal lobes,thalamus and periventricular area.PET or SPECT indicated hypometabolism in 5/6 patients.After the average of fourteen months follow-up,9/12 patients had good prognosis,3 patients did not.Of 3 patients with poor outcome,2 had small cell lung cancer.Conclusions The predominant clinical features of the anti-GABA-BR encephalitis are seizures,cognitive disorder and psychiatric symptoms.The lesions are not only located in limbic system.Early diagnosis and immune modulation may provide a good outcome.

关 键 词：脑脊液 脑电图 磁共振成像 抗γ-氨基丁酸B受体脑炎 

分 类 号：R742.9[医药卫生—神经病学与精神病学]