20例抗N-甲基-D-天冬氨酸受体脑炎患者临床特点分析

目的回顾性分析总结20例抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎患者的临床特点,增强对抗NMDAR脑炎的认识。方法对90例临床疑似脑炎患者的血清和脑脊液进行抗NMDAR-IgG检测,分析确诊为抗NMDAR脑炎的20例患者的临床表现、实验室检查、治疗及预后。结果抗NMDAR脑炎患者男女比例为6:14,中位年龄24岁,首发症状及主要精神症状多有不同。20例抗NMDAR脑炎患者中有12例患者血清和脑脊液中抗NMDAR-IgG抗体均阳性,其他8例仅在血清或脑脊液中检测到抗NMDAR-IgG抗体。5例盆腔检查异常,其中1例病理确诊为成熟囊性畸胎瘤。6例患者脑电图异常,7例头颅MRI异常。除1例患者未接受免疫治疗死亡外,其余患者接受免疫治疗后症状均有不同程度的缓解,其中3例未伴畸胎瘤的患者用二线免疫治疗后复检血清和脑脊液中抗NMDAR-IgG抗体水平下降。结论抗NMDAR脑炎患者中年轻女性发病率较高。二线免疫治疗可能对不伴有畸胎瘤患者的疗效更好。CSF中的抗NMDAR-IgG抗体的阳性率高于血清,同时检测血清和脑脊液中抗NMDAR抗体可以提高疾病的诊断效率。

The analysis ofcharacteristics in 20 patients with anti-NMDA receptor encephalitis

Objective Retrospectively analyzed and summarized the clinical characteristics in 20 patients with anti-NMDA receptor encephalitis,to raise the awareness of anti-NMDA receptor encephalitis.Methods The anti-NMDAR-IgG was tested in both the serum and cerebrospinal fluid (CSF)of the 90 patients who were suspected encephalitis.We analyzed the clinical manifestations,laboratory tests,treatment and prognosis of the patients who were confirmed anti-NMDA receptor encephalitis.Results 20 patients were diagnosed with anti-NMDA receptor encephalitis in the 90 patients.The ratio of the male and female was 4:16,and the median age at disease onset was 24 years.The initial symtoms and the main mental symptoms were different among the patients.The anti-NMDAR-IgG was positive both in the serum and CSF in 12 patients.The antibody was detected only in the CSF or serum in other 8 patients.Pelvic examination were found abnormal in 5 patients,and one of them was diagnosed with mature cystic ovarian teratoma in the subsequent pathological diagnosis.Electroencephalogram were abnormal in 6 patients.MRI were abnornal in 7 patients.All the patients except 1 patient who died received immunotherapy and after that the symptoms got a certain remission.The serum and the CSF of 3 patients without ovarin teratoma who had suffered the second immunotherapy were tested again,and we found that the level of the antibody decreased.Conclusion Anti-NMDAR encephalitis is common in young woman.The second-line therapy may be more effective in the patient without ovarin teratoma.The sensitivity of anti-NMDAR antibody testing is higer in CSF than in serum,therefore,examine the antibody both in serum and CSF at the same time can improve the diagnostic efficiency.