视神经脊髓炎合并抗N-甲基-D-天冬氨酸受体脑炎1例

目的 探讨视神经脊髓炎合并抗N-甲基-D-天冬氨酸(NMDA)受体脑炎的临床特征。方法 报道本院收治的1例视神经脊髓炎合并抗N-甲基-D-天冬氨酸受体脑炎的临床特点及诊治经过,并结合文献分析该病的特征。结果 患者为21岁女性,表现为腹泻后急性起病的下肢麻木无力、昏睡、视物模糊; MRI示双侧基底节、海马、桥臂、齿状回、延髓背侧多发异常信号,同时伴有颈髓多发条片状长T₂异常信号。脑脊液NMDA受体抗体阳性,血清和脑脊液水通道蛋白4(AQP4)抗体阳性,合并心动过缓并发症,筛查未发现肿瘤; 经激素冲击、丙种球蛋白及免疫抑制剂治疗后症状逐渐好转至痊愈。结论 视神经脊髓炎合并抗N-甲基-D-天冬氨酸受体脑炎可以在感染后起病,症状和影像学主要表现为两种疾病的叠加,可出现心动过缓并发症,早期积极行免疫治疗效果较好。

A case of neuromyelitis optica combined with anti-NMDA receptor encephalitis

ObjectiveTo explore the clinical characteristics of neuromyelitis optica combined with anti-N-methyl-D-aspartate( NMDA)receptor encephalitis.Methods A case of neuromyelitis optica combined with anti-NMDA receptor encephalitis was reported,and the diagnosis and treatment were analyzed.The related literatures of this disease were reviewed.Results A 21-year-old woman suffered from sudden lower limb numbness and weakness,lethargy and blurred vision after diarrhea.MRI revealed multiple abnormal signals in bilateral basal ganglias,hippocampus,pontine arm,dentate gyrus and dorsalis medulla oblongata.Many abnormal long T2 signals were found in cervical cord.The antibody of NMDA receptor was positive in cerebrospinal fluid,and aquaporin-4 antibody was also positive in blood and cerebrospinal fluid.Bradycardia was also found in this patient without tumors.Impulsive therapy with steroid and immunotherapy with gammaglobulin achieved a good clinical outcome.Conclusion Neuromyelitis optica combined with anti-NMDA receptor encephalitis can occur after intestinal infection,and the symptoms and imaging characteristics are presented with the two clinical syndromes of neuromyelitis optica and anti-NMDA encephalitis.Bradycardia can also occur in this disease,and timely immunotherapy is effective.