Department of Neurology, Aalborg University Hospital, Aalborg, Denmark.
Department of Clinical Medicine, Aalborg University, Aalborg, Denmark.
BMC Neurol. 2023 Mar 10;23(1):102. doi: 10.1186/s12883-023-03129-2.
α-Amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) and N-methyl-D-aspartate (NMDA) receptors mediate excitatory neurotransmission in the brain and may be targeted by autoantibodies, leading to autoimmune synaptic encephalitis (AE). AE can be associated with other autoimmune diseases. However, the cooccurrence of anti-AMPA and NMDA receptor AE together with myasthenia gravis (MG) is unusual.
A 24-year-old previously healthy male presented with seronegative ocular MG, the diagnosis of which was supported by single-fiber electrophysiology findings. Three months later, he developed AE, initially being positive for AMPA receptor antibodies and subsequently for NMDA receptor antibodies. No underlying malignancy was found. In response to aggressive immunosuppressive treatment, he recovered (modified Rankin Scale (mRS) score change from 5 to 1). Despite some cognitive problems at the 1-year follow-up, which were not revealed using the mRS, he was able to return to his studies.
AE may coexist with other autoimmune disorders. Patients with seronegative MG, including ocular MG, may develop autoimmune encephalitis with more than one cell-surface antibody.
α-氨基-3-羟基-5-甲基-4-异恶唑丙酸（AMPA）和 N-甲基-D-天冬氨酸（NMDA）受体在大脑中介导兴奋性神经递质传递，并且可能成为自身抗体的靶点，导致自身免疫性突触脑炎（AE）。AE 可能与其他自身免疫性疾病相关。然而，抗 AMPA 和 NMDA 受体 AE 与重症肌无力（MG）同时发生并不常见。
一名 24 岁的既往健康男性出现血清阴性眼肌型 MG，单纤维肌电图检查结果支持这一诊断。3 个月后，他出现 AE，最初 AMPA 受体抗体阳性，随后 NMDA 受体抗体阳性。未发现潜在的恶性肿瘤。在接受强化免疫抑制治疗后，他（改良 Rankin 量表评分从 5 变为 1）恢复。尽管在 1 年随访时存在一些认知问题（mRS 未显示），但他仍能够继续学业。
AE 可能与其他自身免疫性疾病同时存在。血清阴性 MG，包括眼肌型 MG，可能会发生具有多种细胞表面抗体的自身免疫性脑炎。
