抗γ-氨基丁酸B受体抗体阳性边缘叶脑炎:1例报道及文献复习
GABABR antibody-positive limbic encephalitis: A case report and literature review
目的:提高对抗γ-氨基丁酸B受体(gamma-aminobutyric acid-B receptor,GABABR)抗体阳性边缘叶脑炎的认识。
方法:报道1例首诊误诊为缺血性脑卒中的抗GABABR抗体阳性边缘叶脑炎患者的临床诊治经过,重点探讨抗GABABR抗体阳性边缘叶脑炎的临床表现、影像学表现及鉴别诊断。
结果:1例抗GABABR抗体阳性边缘叶脑炎患者以缺血性脑卒中为首发表现,随后出现进行性认知功能下降及癫痫发作。脑脊液抗GABAB受体抗体阳性,影像学提示左侧海马异常信号,伴FDG代谢增高,未发现肿瘤证据。给予丙种球蛋白治疗1个疗程后,患者症状明显改善。
结论:抗GABABR抗体阳性边缘叶脑炎较罕见,其发病机制与自身免疫性抗GABABR抗体攻击GABABR相关。患者通常急性或亚急性起病,常有前驱感染史,临床表现为早期显著的癫痫发作及认知功能减退,常合并肿瘤。抗GABABR抗体阳性边缘叶脑炎的诊断有赖于详细的病史、体格检查及辅助检查,影像学可发现海马及颞叶异常信号,脑脊液检测出相应抗体能够提示诊断。早期诊断及给予免疫抑制治疗能够显著改善患者的病情。
Objective: To improve the understanding of the gamma-aminobutyric acid-B receptor (GABABR) limbic encephalitis in clinical practice.
Methods: This paper reports the diagnosis and treatment of a patient with anti-GABABR antibody-positive limbic encephalitis misdiagnosed as ischemic stroke at the first diagnosis, focusing on the clinical manifestations, imaging findings and differential diagnosis of anti- GABABR antibody-positive limbic encephalitis.
Results: A patient who was diagnosed as anti-GABABR antibody-positive limbic encephalitis presented with ischemic stroke as the first manifestation, followed by progressive cognitive impairment and seizures. The anti-GABABR antibodies were proved in the cerebrospinal fluid. Imaging findings revealed abnormal signals in left hippocampus, with FDG metabolism was elevated and no evidence of tumor was found. The patient improved significantly after administration of gamma globulin.
Conclusion: Anti-GABABR encephalitis is an uncommon autoimmune disease, which has been known to be often associated with cancer, and its pathogenesis is related to the autoimmune antibody attack on GABABR. Patients usually have acute or subacute onset, with a history of precursor infection, and the significant clinical manifestations are seizures and cognitive impairment. The diagnosis depends on a detailed history, physical examination and auxiliary examination. MR can find abnormal signals in the hippocampus and temporal lobe. The anti-GABABR antibodies proved in the cerebrospinal fluid can confirm the diagnosis. Early diagnosis and immunosuppressive treatment will improve the patient’s condition.
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