儿童抗N-甲基-D-天冬氨酸受体脑炎7例临床分析  

Clinical Analysis of 7 Children with Anti-N-Methyl-D-Aspartate Receptor Encephalitis

作　　者：陈丽婷 林希[1] 施晓容[1] 郑爱东[1] CHEN Liting;LIN Xi;SHI Xiaorong;ZHENG Aidong(Department of Pediatrics,The First Affiliated Hospital of Fujian Medical University,Fuzhou 350005,China)

机构地区：[1]福建医科大学附属第一医院儿科,福州350005

出　　处：《福建医科大学学报》2021年第2期145-148,共4页Journal of Fujian Medical University

摘　　要：目的分析总结儿童抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎的临床特点,提高诊治水平。方法收集抗NMDAR脑炎患儿7例,男童3例,女童4例,发病年龄29~166个月。回顾性分析患儿的临床资料。结果7例中,首发症状依次为癫痫发作4例,精神行为异常2例,不自主运动1例。7例行脑脊液抗NMDAR抗体检查均阳性;4例行血清抗NMDAR抗体检查,阳性者2例。7例均接受一线免疫治疗(甲泼尼龙琥珀酸钠联合静脉用丙种球蛋白),一线免疫治疗无效予二线免疫治疗(环磷酰胺或利妥昔单抗)3例。治愈4例,好转2例,自动出院1例,复发2例。结论抗NMDAR脑炎临床表现多样,如病程中累积出现多个抗NMDAR脑炎的主要症状(≥3个),需高度警惕。积极的免疫治疗是抗NMDAR脑炎良好预后的关键,对一线免疫治疗无效者选用二线免疫治疗效果显著。Objective To analyze the clinical characteristics of anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis,and to improve the level of diagnosis and treatment.Methods 7 children with anti-NMDAR encephalitis were 3 males,4 females,onset age ranged from 29 months old to 166 months old.The clinical data of children with anti-NMDAR encephalitis were retrospectively analyzed.Results Of the 7 children,the common initial symptoms include seizures(4 cases),mental behavior abnormality(2 cases),involuntary movement(1 case).The cerebrospinal fluid of all patients were positive for the NMDAR antibody,but 2 in 4 patients tested positive for the NMDAR antibody in the blood.Methylprednisolone and immunoglobulins were used as first-line therapeutic agents,all patients were treated with first-line immunotherapy.Rituximab and cyclophosphamide were used as second-line therapeutic agents,3 patients were given the second-line immunotherapy after the failure of first-line therapy.4 cases were cured,2 cases were improved,1 case was discharged automatically,and 2 cases were relapsed.Conclusion Anti-NMDAR encephalitis has a variety of manifestations,several principal clinical features were accumulated in coursed of the disease,suggested that the patient may be considered with anti-NMDAR encephalitis.Aggressive immunotherapy is the key to a favourable outcome.Patients with anti-NMDAR encephalitis showed a good response to second-line immunotherapy after the failure of first-line therapy.

关 键 词：抗N-甲基-D-天冬氨酸受体脑炎 儿童 免疫治疗 

分 类 号：R742.9[医药卫生—神经病学与精神病学]