抗NMDA受体脑炎7例临床分析

Clincial analysis of 7 cases of Anti-NMDA receptor encephalitis patients

目的总结间接免疫荧光法检测抗NMDA受体IgG阳性脑炎患者的临床表现、诊治和预后特点,以提高该型脑炎的诊治水平。方法回顾性分析2015-06-2016-08在中山大学附属第一医院神经内科住院检测抗NMDA受体IgG阳性的7例脑炎患者的临床表现、辅查、诊治及转归情况。结果患者以年轻女性发病多见,临床表现精神行为异常5例,癫痫6例,意识障碍4例,认知功能下降3例;脑脊液常规、生化结果异常4例,多数是WBC稍高一点,多以单核细胞为主,脑脊液蛋白升高2例,值均不太高。头颅MRI病灶:4例在边缘叶,1例在脑膜,2例无病灶,具有"影像轻"、临床"病情重"的特点;脑电图:4例患者脑电图异常。同时查血清和脑脊液抗NMDA受体IgG的5例患者结果均阳性,且血清中抗NMDA受体的抗体滴度均高于脑脊液。抗NMDA受体脑炎合并肺癌并肺门、纵隔转移1例(14.3%),合并双卵巢多囊样改变1例。多数患者给予激素和(或)丙球治疗,个别给予血浆置换治疗,其中1例给予一线各种治疗效果不好后使用二线治疗(环磷酰胺和妥昔单抗)。所有患者经上述治疗后好转,但遗留症状也常见,其中遗留认知下降3例,不自主运动2例。住院时间(48.29±46.48)d。结论抗NMDA受体脑炎患者临床表现以精神行为异常、癫痫、肌张力障碍、意识障碍及认知功能下降为特点,MR可见边缘叶病灶,脑电图多有异常,脑脊液类似病毒脑炎改变,采用抗NMDA抗体IgG检测对诊断十分重要,诊断后应立即使用足量、足疗程的免疫球蛋白和(或)激素治疗和(或)血浆置换治疗,疗效不满意时可重复使用,甚至加用硫唑嘌呤等免疫抑制剂治疗,经上述治疗后一般有好转,但遗留症状也常见,住院治疗时间较长。

Objective The clinical manifestation,diagnosis and treatment, and prognosis of characteristics of IgG Antibody Positive of NMDA-receptor encephalitis patients determined by Indirect Immune Fluorescence Method were summarized here, ai- ming to improve the diagnosis and treatment level of the encephalitis. Methods A retrospective analysis of the clinical manifestation,auxiliary check, diagnosis- and treatment,and outcome for 7 encephalitis patients of NMDA receptor IgG positive resistance from Neurology hospital of the first affiliated hospital of Sun Yat-sen University between Jun 2015 and Aug 2016 was carried out. Results The encephalitis was more often appeared in young females. There were 5 cases in clinical manifestations of mental behavior, 6 cases of epilepsy, 4 cases in disturbance of consciousness, 3 cases in cognitive decline; Cerebrospinal fluid routine and bio- chemical results were abnormal in 4 cases, while most of them had more WBC in which monocytes accounted for the major proportion and cerebrospinal fluid protein increased in 2 cases with not-too-high values; Head MRI lesions： there were 4 cases in the limbic lobe, 1 case in the meninge, and 2 cases with no lesions, while characteristics of mild image and serious clinical manifestation were present;Electroencephalogram （EEG） ：4 patients had abnormal EEG. The results of five patients whose NMDA receptor IgG resistance in the serum and cerebrospinal fluid were both checked showed positive, and the antibody titers of IgG in the serum were higher than those in the cerebrospinal fluid. Two special cases were found in this study： one was an encephalitis patient of NMDA receptor IgG positive resistance and lung cancer with hilus pulmonis and mediastinum metastases, the other one complicat- ed with polycystic ovary syndrome. The majority of the patients were given hormone and （or） gamma globulin, and individual plas- ma exchange treatment. A patient was received second-line therapy （cyclophosphamide and rituximab） when all kinds of first-line therapy were useless. All the patients were better after the treatments, but remained symptoms were common in which 3 cases were cognitive decline and 2 cases were of no-automatic transport. The duration of hospital stays of these patients was （48. 29±46.48） dayS. Conclusion Clinical manifestations＇ features for anti-NMDA encephalitis patients were mental behavior disorders, epilepsy, dystonia, disturbance of consciousness and the decline in cognitive function. Limbic lobe lesions were often present in MR and Electroencephalogram can be seen abnormal. Virus encephaliti change occurred in CSF. Detection of IgG was very important for the diagnosis. After the diagnosing, enough immunoglobulin therapy hormonal therapy and （or） plasma exchange therapy should be used immediately. If the effect was not satisfactory, the therapy could be reused, and even the immunosuppressive agents such as azathioprine could be used. Patients were better after the above treatments, but remained symptoms were also common and the duration of hospital stays was long.