PDF(Pubmed)
Abstract：
Autoimmune encephalitis is a disease characterised by neural-specific antibodies. This case report presents a 20-year-old young man with a recent history of suspected viral encephalitis who presented with recurrent fevers and episodes of confusion. He was found to have anti-N-methyl-D-aspartate receptor (NMDAR) and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid 1 receptor (AMPAR1) positive autoantibodies and was diagnosed with autoimmune encephalitis. He subsequently developed global cerebral atrophy and was found to meet diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH). This patient\'s presentation was consistent with existing literature showing that autoimmune encephalitis may develop after an initial viral meningoencephalitis. However, concurrent anti-NMDAR and anti-AMPAR1 positive autoimmune encephalitis has not been reported in literature to date, and this case report represents one instance of its presentation. We speculate that multiple antibodies against neural surface antigens may increase the risk for systemic immune activation leading to HLH and acute cerebral atrophy.
摘要：
自身免疫性脑炎是一种以神经特异性抗体为特征的疾病。该病例报告介绍了一名20岁的年轻人，他最近有疑似病毒性脑炎的病史，他反复发烧和混乱发作。发现他具有抗N-甲基-D-天冬氨酸受体（NMDAR）和α-氨基-3-羟基-5-甲基-4-异恶唑丙酸1受体（AMPAR1）阳性自身抗体，并被诊断患有自身免疫性脑炎。他随后发展为全脑萎缩，并被发现符合噬血细胞性淋巴组织细胞增多症（HLH）的诊断标准。该患者的表现与现有文献一致，表明自身免疫性脑炎可能在最初的病毒性脑膜脑炎后发展。然而,同时抗NMDAR和抗AMPAR1阳性自身免疫性脑炎迄今尚未在文献中报道,此案例报告代表其演示文稿的一个实例。我们推测，针对神经表面抗原的多种抗体可能会增加全身免疫激活导致HLH和急性脑萎缩的风险。
