儿童抗NMDAR脑炎诊治进展  

Advance in diagnosis and treatment of pediatric anti-NMDAR encephalitis

作　　者：王燕菲(综述) 何大可[1] 李玲(审校)[1] Wang Yanfei;He Dake;Li Ling(Department of Pediatric Neurology,Xinhua Hospital Affiliated to Medicine School of Shanghai Jiaotong University,Shanghai 200092,China)

机构地区：[1]上海交通大学医学院附属新华医院儿神经内科,200092

出　　处：《国际儿科学杂志》2020年第9期617-622,共6页International Journal of Pediatrics

基　　金：国家自然科学基金(81571180)。

摘　　要：抗N-甲基-D-天冬氨酸受体(N-methyl-D-aspartate receptor,NMDAR)脑炎是由自身抗体IgG作用于NMDAR的GluN1亚基引起的弥漫性脑炎,临床多以神经症状如运动障碍、共济失调和癫痫为首发症状,辅助检查可有脑脊液IgG抗体阳性、脑电图(electroencephalogram,EEG)慢波和氟脱氧葡萄糖正电子发射断层扫描(18F-fluorodeoxyglucose positron emission tomography,FDG-PET/CT)代谢改变,但目前临床上对症状不典型和抗体阴性的患儿缺少早期诊断的特异性标志物。免疫治疗对多数患儿有效而且早期免疫治疗能大幅度改善预后,同时联合抗精神病类药物和抗癫痫药物可改善患儿精神行为症状和癫痫发作,不过仍有一部分难治性病例即使应用新型免疫抑制剂仍遗留不同程度的后遗症,亟需进一步的研究来揭示该病的发病机制,为患儿提供早期诊断的生物标志物。该文从该病的发病机制、临床特点和诊疗进展方面进行综述。Anti-N-methyl-D-aspartate receptor encephalitis is severe diffuse encephalitis caused by cerebrospinal fluid(CSF)antibodies against the GluN1 subunit of the NMDAR.Pediatric NMDAR encephalitis is mainly characterized by neurological symptoms such as dyskinesia,ataxia and epilepsy.Auxiliary examinations of the patients show positive CSF IgG antibodies,EEG slow waves and metabolic changes in FDG-PET/CT.But there is lacking of clinical biomarkers for early diagnosis of children with atypical symptoms and negative antibody.Immunotherapy is effective for most children and early treatment can improve prognosis remarkably.At the same time,antipsychotic drugs and antiepileptic drugs can treat children with psycho behavioral symptoms and seizures.However,some patients are refractory to conventional therapies and have neurological sequela even after the application of multiple immunosuppressants.Therefore,more further studies are needed to clarify the pathogenesis and find biomarkers for early diagnosis of pediatric patients.This article reviews the pathogenesis,clinical characteristics,diagnosis and treatments of the disease.

关 键 词：抗N-甲基-D-天冬氨酸受体脑炎 儿童 诊治 

分 类 号：R74[医药卫生—神经病学与精神病学]