摘要
目的探讨抗GABA-B(γ-氨基丁酸-B)受体脑炎的临床表现、实验室及影像学特点、治疗及预后。方法回顾性分析5例经脑脊液免疫学检查诊断为抗GABA-B受体脑炎患者的临床资料。结果 5例均呈急性起病,临床首发症状为癫痫发作,癫痫发作后出现不同程度的认知功能障碍及精神行为异常。影像学特点:病灶累及颞叶内侧、海马,头部CT呈等密度或低密度影,头部MRI呈长T_1、长T_2信号,T_2 FLAIR高信号,DWI等或高信号。2例行头部核磁增强病灶无强化。1例行PET-CT示颞叶海马低密度影,代谢增高。脑脊液及血清免疫学检查均提示脑脊液GABAB-R-AB阳性(+),血清GABAB-R-AB阳性(+)。5例均伴有NSE不同程度增高。脑电图无特异性,同患者意识状态相关。5例行肺部CT均合并纵隔淋巴结肿大,发病时合并中心性肺癌3例,其余2例随访3个月后亦发现中心性肺癌。2例行支气管镜病理活检为小细胞肺癌。结论抗GABA-B受体脑炎常以癫痫为首发症状,影像学多数以颞叶内侧、海马免疫性炎症反应信号为主要表现,同肺癌(小细胞肺癌)密切相关,结合脑脊液免疫学检查及上述特点有助诊断。
Objective To investigate the clinical manifestations, laboratory and imaging features, treatments and prognosis of anti GABA-B receptor encephalitis. Methods We performed a retrospective analysis of the clinical data of five cases with anti GABA-B receptor encephalitis diagnosed by eerebrospinal fluid immunological examination. Results Five cases had acute onset. The initial clinical symptom was epileptic seizure. After epileptic attack, patients developed different degrees of cognition dysfunction, mental and behavioral disorders. Neuroimaging showed that the lesions mainly involved medial temporal lobe and hippocampus. The lesions ware equal or low density shadows in CT scan and long T1 and T2 signals, T2 high signals on Flair, equal or high signals on DWI in MRI scan. Cranial MRI enhancement showed lesions with no enhancement in two tested cases. One case took PET-CT and data showed a low density shadow of temporal lobe and hippocampus with increased metabolism. CSF and blood immunological examinations showed CSF GABAB-R-AB positive （＋）, blood GABAB-R-AB positive （＋）. Five cases were associated with increased NSE. EEG was nonspecific and related to the conscious state of the patient. Lung CT showed enlarged mediastinal lymph nodes in 5 tested cases. Central lung cancers were found in five cases including three cases at onset and two cases during three month follow-up. Bronchoscopic biopsy identified small cell lung cancer in two examined cases. Conclusion Epileptic seizure is the most frequent initial symptoms of anti GABA-B encephalitis. Inflammatory response signals in the medial temporal lobe and hippocampus are image features of anti GABA-B encephalitis. The disease is closely related to lung cancer （Especially c small cell lung cancer, SCLC）. Immunological examination of cerebrospinal fluid and the above characteristics are helpful for diagnosis.
出处
《中国神经精神疾病杂志》
CAS
CSCD
北大核心
2017年第10期591-596,共6页
Chinese Journal of Nervous and Mental Diseases
关键词
脑炎
自身免疫性脑炎
副肿瘤综合征
Encephalitis Autoimmune encephalitis Paraneoplastic syndrome
