Wu Qian, Dai Shujuan, Zhu Lin, Zhao Charlie Weige
Neurology, Kunming Medical University, Kunming, Yunnan, China.
Department of Neurology, Yale University School of Medicine, New Haven, Connecticut, USA
BMJ Case Rep. 2021 Mar 17;14(3):e240659. doi: 10.1136/bcr-2020-240659.
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.
自身免疫性脑炎是一种以神经特异性抗体为特征的疾病。本病例报告介绍了一名20岁的年轻男性，近期有疑似病毒性脑炎病史，表现为反复发热和意识模糊发作。他被发现抗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型受体（AMPAR1）自身抗体呈阳性，被诊断为自身免疫性脑炎。随后他出现了全脑萎缩，并被发现符合噬血细胞性淋巴组织细胞增生症（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生。然而，迄今为止，文献中尚未报道过同时存在抗NMDAR和抗AMPAR1阳性的自身免疫性脑炎，本病例报告是其表现的一个实例。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。
