Department of Clinical Medicine, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Zhejiang Province, Wenzhou, 325037, China.
Department of Neurology, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Zhejiang Province, Wenzhou, 325027, China.
Eur J Med Res. 2022 May 9;27(1):66. doi: 10.1186/s40001-022-00697-w.
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis can coexist with neuromyelitis optica spectrum disorder (NMOSD). Patients with overlapping anti-NMDAR encephalitis with positive NMDAR antibodies and aquaporin 4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD are rare but should not be ignored.
This report describes a unique case of anti-NMDAR encephalitis coexisting with NMOSD. A 27-year-old male presented with blurred vision, cognitive impairment, psychosis, dysphagia, gait instability and urinary incontinence. Brain magnetic resonance imaging (MRI) showed abnormal signals in the right cerebellar hemisphere, temporal lobe, and corpus callosum. NMDAR antibodies were positive in the CSF. AQP4-IgG antibodies were positive in the serum. The patient's condition was stable following intravenous gamma globulin, corticosteroids, immunosuppressants and symptomatic treatments.
This case provides further evidence for the occurrence of anti-NMDAR encephalitis overlapping NMOSD with AQP4-IgG-seropositive in a Chinese patient. However, the mechanisms underlying the occurrence of double-positive antibodies remain elusive.
抗 N-甲基-D-天冬氨酸受体（NMDAR）脑炎可与视神经脊髓炎谱系疾病（NMOSD）共存。同时存在抗 NMDAR 脑炎阳性 NMDAR 抗体和水通道蛋白 4 免疫球蛋白 G（AQP4-IgG）阳性 NMOSD 的重叠表现的患者较为罕见，但不容忽视。
本报告描述了一例抗 NMDAR 脑炎合并 NMOSD 的独特病例。一名 27 岁男性，以视力模糊、认知障碍、精神病、吞咽困难、步态不稳和尿失禁为主要表现。脑部磁共振成像（MRI）显示右侧小脑半球、颞叶和胼胝体异常信号。CSF 中 NMDAR 抗体阳性，血清中 AQP4-IgG 抗体阳性。患者经静脉注射丙种球蛋白、皮质类固醇、免疫抑制剂和对症治疗后病情稳定。
本病例进一步证明了中国患者中存在抗 NMDAR 脑炎与 AQP4-IgG 阳性 NMOSD 重叠的情况。然而，双阳性抗体发生的机制仍不清楚。
