以癫痫发作起病的抗GABAB受体脑炎六例  

The 6 cases of clinical features of anti-GABAB receptor encephalitis

作　　者：徐秦岚 崔韬[1] 史伟雄[1] 王群[1] 李志梅[1] Xu Qinlan;Cui Tao;Shi Weixiong;Wang Qun;Li Zhirnei(Department of Neurology, the Beijing Tiantan Hospital, Capital Medical University, Beijing 100050, China)

机构地区：[1]首都医科大学附属北京天坛医院神经病学中心癫痫科,北京100050

出　　处：《脑与神经疾病杂志》2018年第7期405-410,共6页Journal of Brain and Nervous Diseases

基　　金：首都医科大学基础-临床(天坛专项)科研合作基金(17JL(TTZX)02)

摘　　要：目的总结抗r氨基丁酸B型(GABAB)受体脑炎的临床特征以利于早期诊断及治疗。方法对6例抗GABAB受体脑炎患者的临床、实验室、脑电图(EEG)及影像学资料进行分析,并对其进行10~19(13.5±3.1)个月的随访。结果该组患者均以癫痫发作起病,表现为部分性发作、全面强直阵挛发作。5例出现认知功能减退,4例出现精神行为异常。发作间期EEG 1例表现为右前中颞区棘慢波,余未见明显异常。头颅MRI示2例颞叶内侧、海马T2/FLAIR高信号,1例海马体积缩小。腰穿检查2例脑脊液(CSF)压力增高,1例白细胞数增多,4例蛋白升高。所有患者血清及CSF GABAB受体抗体阳性,1例患者血清Hu抗体弱阳性。胸CT示2例肺部占位、2例肺叶微结节、1例纵隔稍大淋巴结及肝脏顶部异常强化灶,4例经活检诊断为小细胞肺癌。抗癫痫药物对患者癫痫发作控制不佳,免疫治疗及抗肿瘤治疗有效。结论抗GABAB受体脑炎可以癫痫发作为首发症状,逐渐出现认知障碍和精神症状,需注意对该类患者进行肿瘤筛查及随访,早期免疫治疗及抗肿瘤治疗可以改善患者神经功能预后。Objective To summarize the clinical features of anti r-aminobutyric acid receptor type B （ GABAB ） receptor encephalitis in order to diagnosis and treatment as early as possible. Method We analyzed the clinical, laboratory, electroencephalogram （ EEG ） and imaging datas of 6 patients with anti-GABAB receptor encephalitis and the outcome from 10-19 （ 13.5 ± 3.1 ） months. Results All patients presented with seizure onset, including partial seizure and generalized tonic-clonic seizure. Five patients developed cognitive deficits, and four of them had mental and behavior disorder. Interictal EEG showed normal except one exhibited spike-slow wave in right anterior mesial temporal lobe. Cerebral MRI revealed TJFLAIR hyperintense in mesial temporal lobe and hippocampus in two cases, and hippocampus atropy in one case. Lumber puncture showed two cases of elevated cerebrospinal fluid （ CSF ） pressure, one case of increased WBC and four of high protein level. All patients manifested GABAB receptor antibody positive in both serum and CSF. One patient had Hu antibody positive in serum. Chest CT scan showed lung lesions in two cases, pulmonary lobar nodules in two cases and upper liver enhanced lesion with larger mediastinal lymph nodes in one case. Four cases were diagnosed small cell lung cancer by biopsy. While the response to antiepileptic drugs was poor, most patients responded favorably to immunotherapy and anti-tumor treatment. Conclusion Anti-GABAB receptor encephalitis may initially manifest with seizures and slowly progressive cognitive impairment and psychosis. Screening and following-up for tumor was necessary. Immunotherapy and anti-tumor treatment at early stage could improve neurological prognosis.

关 键 词：抗r氨基丁酸B型受体抗体 脑炎 认知功能障碍 精神异常 小细胞肺癌 

分 类 号：R742.1[医药卫生—神经病学与精神病学]