抗N-甲基-D-天冬氨酸受体脑炎5例分析及文献复习

Virus infection and limbic encephalitis： Clinical analysis of 5 cases of anti-N-methyl-D-aspartic receptor encephalitis and literature review

目的通过对抗N-甲基-D-天冬氨酸（N-methyl-D-aspartic, NMDA）受体脑炎患者的临床资料进行分析和总结，并复习相关文献，以加强对该病的认识。方法回顾性分析2010年5月～6月首都医科大学附属北京友谊医院收治的5例抗NMDA受体脑炎患者的临床资料，包括临床表现、既往史、影像学表现、血清学和脑脊液检查结果、治疗和预后。结果5例患者中，3例为年轻女性，2例为中老年男性；5例患者均以精神行为异常起病，同时出现癫痫发作，以及面部和肢体的不自主运动或震颤等锥体外系症状；3例女性患者均出现意识障碍，低通气，需要呼吸机辅助呼吸；2例男性患者病情相对略轻，不需要呼吸机辅助呼吸。1例合并卵巢畸胎瘤，1例合并Vogt-小柳-原田综合征。5例患者脑脊液抗NMDA抗体均为阳性；3例血抗NMDA抗体阳性。同时合并血和脑脊液EB病毒IgM抗体阳性1例，血和脑脊液单纯疱疹病毒1（HSV-1型）IgM抗体阳性1例；血和脑脊液抗髓鞘少突胶质细胞糖蛋白（MOG）抗体阳性1例。5例患者从发病到确诊的时间间隔为10～37 d，平均（18.8±9.8）d。所有患者均接受静脉用丙种球蛋白（IVIG）治疗，3例女性患者同时接受激素冲击治疗和血浆置换治疗。合并Vogt-小柳-原田综合征患者从发病到开始治疗时间最长（37 d），最终死亡，其余4例患者基本痊愈。合并畸胎瘤患者随访7年，血清抗NMDAR抗体始终阳性，所有4例存活患者脑炎症状无复发。结论抗NMDA受体脑炎以精神症状起病多见，临床表现复杂，影像学和脑脊液常规检查可正常。可以同时合并单纯疱疹病毒I型或EB病毒感染，可合并抗MOG抗体阳性，积极治疗是关键。

ObjectiveTo have a profound understanding of anti-N-methyl-D-aspartic receptor （anti-NMDAR） encephalitis, through the clinical analysis of 5 cases of anti-NMDAR encephalitis, and literature review.MethodsThis is a retrospective analysis. Five cases of anti-NMDA receptor encephalitis treated from May 2010 to June 2015, in the Department of Neurology, Beijing Friendship Hospital affiliated to Capital Medical University, were included in this study. The clinical data, including clinical manifestation, past history, radiological features, serum and cerebral spinal fluid examinations, treatment and prognosis, were analyzed.ResultsAmong the 5 cases, 3 young female and 2 middle-to old-aged male. The clinical features of the onset was mental and behavior disorder, as well as seizure and extrapyramidal features, like facial and limbic involuntary movements or tremor. Coma and hypopnea was severe in 3 young female cases, needing assistance of mechanical ventilator, while the manifestation of 2 male patients was much mild, need not assisted respiration. 1 case had teratoma of ovary, 1 case had Vogt-Koyanagi-Harada syndrome. The anti-NMDA receptor antibody was positive in cerebraospinal fluid of all 5 cases, but in serum of 3 cases, serum and CSF Epstein-Barr virus （EBV） IgM antibody was positive in 1 case, while herpes simplex I virus （HSV-1） IgM antibody positive in another case, and anti-myelin oligodendrocyte glycoprotein （MOG） antibody was seen in serum and CSF in 1 case. The time interval from the onset to treatment was 10-37 d （18.8±9.8 d）. IVIG was used in all of the 5 cases, glucocoticoid in 4 cases, and plasma exchange in 3 cases. One case with Vogt-Koyanagi-Harada syndrome, having a long time before diagnosis and treatment, died, while the other 4 cases had good prognosis, and had no relapse.ConclusionsMental and behavior disturbance is common at onset of anti-NMDAR encephalitis. The radiological and lab examination may be normal. It may be accompanied with HSV-1 or EBV infection, anti-MOG antibody may be positive in this disease. Active treatment is important.