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[摘要] 本文主要探讨抗N-甲基-D-天冬氨酸（NMDA）受体脑炎患者临床表现、磁共振、脑脊液特点、治疗及预后，以提高对该病的认识。本文回顾性分析6例抗NMDA受体脑炎患者的相关临床资料，并复习相关文献。6例患者中，男3例，女3例，年龄15～61岁，所有患者血清及脑脊液抗NMDA受体抗体为阳性，1例患者脑脊液抗NMDA受体抗体及抗GABA受体抗体均为阳性；3例患者以癫痫发作为首发症状，3例以精神行为异常为首发症状；5例患者伴有不自主运动症状；头颅MRI：1例双侧丘脑DWI高信号；1例左侧颞叶T2、FLAIR高信号；其余4例正常；脑脊液化验结果与病毒性脑炎脑脊液特点相似；2例女性患者伴有畸胎瘤，1例患者伴有小细胞肺癌，经过免疫药物治疗后，症状均有不同程度恢复。抗NMDA受体脑炎以精神异常、癫痫发作为首发症状多见，抗NMDA受体脑炎患者可合并有抗GABA受体抗体阳性；部分患者核磁共振及脑脊液化验结果缺乏特异性。伴有肿瘤的患者，肿瘤切除及应用一线免疫抑制剂治疗，可显著改善预后。

[关键词] 抗N-甲基-天门冬氨酸受体脑炎；临床表现；核磁共振；脑脊液；治疗

[中图分类号] R651.2 [文献标识码] A [文章編号] 1673-7210（2017）01（b）-0175-04

A report of 6 cases with anti-N-methyl-D-aspartate receptor encephalitis and literature review

ZHAO Chao ZHANG Zhuo DUAN Fengju YAN Qi ZHOU Qiong BAI Miao MIAO Jianting LI Zhuyi

Department of Neurology， Tangdu Hospital， the Fourth Military Medical University， Shaanxi Province， Xi'an 710038， China

[Abstract] To improve the recognition of anti-N-methyl-D-aspartate （NMDA） receptor encephalitis， this paper analyzes the clinical presentation， magnetic resonance imaging （MRI）， cerebrospinal fluid （CSF）， treatment and prognosis. This paper analyzes the clinical data of 6 cases with anti-NMDA receptor encephalitis and reviews relevant literatures. Among 6 patients， 3 cases were male， 3 cases were female. The range of age was 15-61 years old. CSF and serum anti-NMDA receptor antibodies were positive in all patients. Anti-NMDA receptor antibodies and anti-GABA receptor antibodies were both positive in one patient. The epileptic attack as first symptom was observed in 3 patients. The mental and behavior disorder as first symptom was observed in 3 patients. 5 patients showed abnormal movements. The cranial MRI showed that DWI high signal intensity in bilateral thalamic regions in one patient. In another patient， the MRI showed T2/FLAIR high signal intensity in left temporal region， anti-NMDAR encephalitis of the left 4 patients showed no changes in the MRI. The CSF findings were in most respects similar to viral encephalitis： 2 cases had teratoma， one case had small cell lung carcinoma， and the symptoms were recovered inordinately after treatment of immunoagents. The first symptom of anti-NMDAR encephalitis was often psychiatric or seizures. Anti-NMDA receptor encephalitis patients could be combined with anti-GABA receptor antibody. The CSF and MRI findings in some patients were not specific. Patients associated with ovarian tumors underwent tumor excision and immunotherapy had remarkable efficacy.

[Key words] Anti-N-methyl-D-aspartate receptor encephalitis； Clinical symptoms； Magnetic resonance imaging； Cerebrospinal fluid； Treatment

抗N-甲基-D-天冬氨酸（NMDA）受体脑炎[1]属于自身免疫性脑炎，于2007年首次由Dalman报道，主要临床表现为癫痫发作、精神行为异常、不自主运动等，容易误诊为病毒性脑炎和精神障碍疾病。本文对6例抗NMDA受体脑炎患者进行临床资料分析。

1 病例资料

1.1 一般资料

收集2015年3～12月于第四军医大学附属唐都医院神经内科确诊的抗NMDA受体脑炎患者6例，所有患者血清及脑脊液抗NMDA受体抗体均为阳性，1例患者合并有血清及脑脊液抗GABA B受体抗体阳性。……