Abstract:

Objective  To explore the clinical features, diagnosis, treatment and prognosis of patients with anti? N ? methyl? D ? aspartate (NMDA) receptor encephalitis.  Methods and Results  Three patients had psychiatric symptoms, intractable epilepsy, disturbance of consciousness and hypoventilation. Two patients had fever. Two patients had autonomic dysfunction (one presented diaphoresis, hypersalivation and sinus arrhythmia, the other one presented nodal tachycardia) and orofacial?limb dyskinesia. All cases were detectied anti?NMDA receptor antibodies positive in serum and cerebrospinal fluid (CSF). CSF white blood cell count and protein quantification presented as non?specific inflammatory changes. Tumor markers screening were negative, and the results of head MRI were abnormal, but not specific. EEG manifestation was non?specific abnormal in 2 patients, while the other one did not undergo EEG inspection. Two patients had clinical symptom improvement after receiving immunotherapy and symptomatic treatment, but the other one died.  Conclusions  Anti?NMDA receptor encephalitis is a type of autoimmune encephalitis with or without neoplasms. The clinical symptoms are diversified but not specific. It is easy to be misdiagnosed as mental disorder in early stage. Detection of anti?NMDA receptor antibody in serum and CSF is a distinctive method to confirm the disease. Most cases have good prognosis after receiving timely and comprenensive treatment, but the prognosis of patients with serious symptoms is usually poor.

Key words: Encephalitis, Receptors, N-methyl-D-aspartate

摘要：

目的 总结抗N-甲基-D-天冬氨酸（NMDA）受体脑炎的临床特点、诊断、治疗及预后。 方法与结果 3 例抗NMDA 受体脑炎患者临床均表现为精神病样症状、顽固性癫发作、意识障碍和中枢性通气不足，2 例有前驱发热史，2 例有自主神经功能障碍（1 例表现为出汗、唾液分泌增多和窦性心律不齐，1 例表现为窦性心动过速）、口面部和肢体运动障碍；血清和脑脊液抗N-甲基-D-天冬氨酸受体（NMDAR）抗体均呈阳性，脑脊液白细胞计数和蛋白定量呈非特异性炎症性改变，肿瘤标志物筛查均呈阴性；头部MRI 表现无特异性；2 例脑电图呈非特异性异常，1 例未行脑电图检查；经对症支持治疗和免疫治疗后2 例临床症状改善，1 例死亡。 结论 抗NMDA 受体脑炎是自身免疫性脑炎，可伴或不伴肿瘤，临床症状多样且无特异性，早期易误诊为精神病。血液和脑脊液抗NMDAR 抗体阳性是特异性生物学标记。早期免疫治疗多数患者预后较好，少数临床症状严重的患者若治疗不及时，预后较差。

关键词: 脑炎, 受体, N-甲基-D-天冬氨酸