抗γ氨基丁酸B型受体脑炎6例临床特点分析  

Analysis of clinical characteristics of 6 cases of anti-γ-aminobutyric acid type B receptor encephalitis

作　　者：李奕[1] 辛子凯 范佳 惠海霞 卜晖[1] 何俊瑛[1] 张文琳[1] LI Yi;XIN Zi-kai;FAN Jia(Department of Neurology,the Second Hospital of Hebei Medical University,Shijiazhuang 050000,China)

机构地区：[1]河北医科大学第二医院神经内科,石家庄065000

出　　处：《临床神经病学杂志》2020年第3期209-212,共4页Journal of Clinical Neurology

摘　　要：目的报道6例抗GABA B型(GABAB)受体脑炎,结合文献探讨其临床特点及诊治。方法回顾性分析我院收治的6例诊断为抗GABAB受体脑炎患者的临床资料,结合相关文献总结和分析该病的临床特点及诊治。结果6例抗GABAB受体脑炎患者为4例男性,2例女性,中位年龄64岁。6例患者均以癫痫为首发症状,其他临床表现包括意识障碍、精神行为异常、高级智能下降。头颅MRI可见边缘叶异常信号。实验室检查可发现血钠异常及自身抗体阳性。CSF可见白细胞计数及蛋白计数轻或中度升高,所有患者均在血液及CSF中检测到抗GABAB受体抗体。1例患者合并CSF抗HU抗体阳性;2例患者在病情加重时再次进行了血及CSF抗GABAB受体抗体检测,其中1例抗体滴度升高,另1例较前降低。6例患者均接受了免疫治疗,其中5例病情好转。结论抗GABAB受体脑炎以耐药性癫痫为主要临床表现,EEG和头颅MRI对其诊断有指导意义。其中部分患者可合并肿瘤及自身免疫性疾病,因此对于可能患有此类疾病患者,必须进行肿瘤及自身免疫性疾病检查。一旦确诊抗GABAB受体脑炎,应立即启动免疫治疗。抗体滴度与病情严重程度的关系以及影响预后的相关因素仍需进一步研究证实。Objective To report 6 cases of anti-GABA B type(GABAB)receptor encephalitis,and to analyze its clinical features,diagnosis and treatment.Methods The clinical data of 6 patients with anti-GABAB receptor encephalitis admitted to our hospital were retrospectively analyzed.The clinical characteristics,diagnosis and treatment of the disease were summarized and analyzed in combination with relevant literature.Results Six patients with anti-GABAB receptor encephalitis were 4 males and 2 females,with a median age of 64 years.Epilepsy was the first symptom in all the 6 patients.Other clinical manifestations included disturbance of consciousness,abnormal mental behavior and decline of intelligence.Abnormal signal of marginal lobe was seen on MRI scanning of the brain.Laboratory tests showed abnormal serum sodium and positive autoantibodies.CSF test indicated slight or moderate increase in intracranial pressure and white blood cell count.Anti-GABAB receptor antibody was detected in all patients in blood and CSF and anti-HU antibody in CSF was detected in one patient simultaneously.Two patients underwent anti-GABAB antibody test in blood and CSF again when exacerbated.The titer of antibody increased in one case and decreased in the other.Immunotherapy was given to all 6 patients,and 5 of them improved.Conclusions The main clinical manifestations of anti-GABAB receptor encephalitis are drug-resistant epilepsy,EEG and head MRI have guiding significance for its diagnosis.Part of patients suffer from tumors and autoimmune diseases Simultaneously,so cancer and autoimmune diseases must be checked for patients who may suffer from such diseases.Immunotherapy should be initiated once anti-GABAB receptor encephalitis is diagnosed.The relationship between antibody titers and severity of the disease and the related factors affecting prognosis still need further study.

关 键 词：抗GABA B型受体脑炎 耐药性癫痫 自身免疫性脑炎 

分 类 号：R512.3[医药卫生—内科学]