抗γ-氨基丁酸B受体脑炎的临床与影像学特点

Clinical and imaging features of anti-GABA-B receptor encephalitis

目的探讨抗γ-氨基丁酸B(GABA-B)受体脑炎患者的临床表现、实验室检查及影像学特点。方法选取我院2021年5月~2022年11月临床资料完整、符合《中国自身免疫性脑炎诊治专家共识(2022年版)》中自身免疫性边缘性脑炎的诊断标准,并经免疫学检查确诊为抗GABA-B受体脑炎的患者3例,收集其临床表现、脑脊液检查、免疫学检查、脑电图、影像学检查等资料进行回顾性分析。结果3例患者均急性起病,以癫痫发作为首发症状或主要临床表现,伴不同程度的认知功能障碍及精神行为异常。3例患者发病时均合并肿瘤,分别为多发性骨髓瘤、肺癌及鼻咽癌。脑脊液及血清免疫学提示脑脊液和(或)抗GABA-B受体抗体阳性。脑电图均异常,呈癫痫样放电、散在分布或弥漫性慢波。影像学检查见病灶累及单侧海马,头颅CT呈低密度影,头颅MRI呈长T1、长T2信号,T2 FLAIR高信号,DWI高信号,ADC呈等或稍高信号。结论抗GABA-B受体脑炎患者以癫痫发作为主要表现,易合并肿瘤。抗GABA-B受体脑炎合并鼻咽癌为首次报道,扩展了疾病谱。

Objective To investigate the clinical manifestations,laboratory and imaging features of patients with anti-GABA-B receptor encephalitis.Methods Three patients with anti-GABA-B receptor encephalitis were selected from our hospital from May 2021 to November 2022 with complete clinical data and in accordance with the diagnostic criteria of autoimmune peripheral encephalitis in the Consensus of Chinese Experts on the Diagnosis and Treatment of Autoimmune Encephalitis(2022 edition).Clinical manifestations,cerebrospinal fluid(CSF),immunological,electroencephalogram and imaging examinations of 3 patients of anti-GABA-B receptor encephalitis were analyzed retrospectively.Results All the 3 patients suffered from acute onset with epilepsy as first symptom or main clinical manifestation,accompanied by cognitive dysfunction and mental behavior abnormality of different degrees.All the 3 patients were complicated with tumors,including multiple myeloma,lung cancer and nasopharyngeal carcinoma.Immunological examinations of CSF and serum indicated that CSF and/or serum anti-GABA-B receptor antibody were positive.Electroencephalogram were abnormal,showing epileptic discharge,scattered distribution or diffuse slow wave.Imaging examinations showed that the lesion involved unilateral hippocampus,brain CT showed low density,MRI showed long T1 signal,long T2 signal,high T2 FLAIR signal,high DWI signal,slight high or equal ADC signal.Conclusion Patients with anti-GABA-B receptor encephalitis suffered from epilepsy as main manifestation and are prone to tumor.We reported that anti-GABA-B receptor encephalitis combined with nasopharyngeal carcinoma for the first time,which expands the disease spectrum.