儿童急性播散性脑脊髓炎的研究进展  

Progress on acute disseminated encephalomyelitis in children

作　　者：张潇 富洋 陆凤娟 Zhang Xiao;Fu Yang;Lu Fengjuan(Pediatric Base for Standardized Training of Resident Physicians,National Children's Medical Center Children's Hospital of Fudan University,Shanghai 201102,China;Department of Hematology,National Children's Medical Center,Children's Hospital of Fudan University,Shanghai 201102,China;Department of Pediatric Rehabilitation,Shanghai Xinqidian Rehabilitation Hospital,Shanghai 201107,China)

机构地区：[1]国家儿童医学中心、复旦大学附属儿科医院住院医师规范化培训儿科专业基地,上海201102 [2]国家儿童医学中心、复旦大学附属儿科医院血液科,上海201102 [3]上海新起点康复医院儿童康复科,201107

出　　处：《国际儿科学杂志》2024年第10期680-684,共5页International Journal of Pediatrics

摘　　要：急性播散性脑脊髓炎(acute disseminated encephalomyelitis,ADEM)是一种常见于儿童的中枢神经系统炎性脱髓鞘疾病。其病因尚不明确,目前主要认为与感染后自身免疫反应的激活有关。ADEM通常是单相型病程,与其他脱髓鞘性疾病如多发性硬化和视神经脊髓炎谱系疾病不同。随着近年来对髓鞘少突胶质细胞糖蛋白抗体的认识、理解和检测的增加,ADEM的诊断和治疗可能进一步变化。ADEM的诊断主要为临床诊断,需排除其他感染性、炎症性、肿瘤性和遗传性病变。急性期治疗方法包括静脉注射大剂量糖皮质激素、治疗性血浆置换和静脉注射免疫球蛋白,多数预后良好。该文综述了目前关于儿童ADEM的发病机制、流行病学、临床特征、诊断及鉴别、治疗和预后的进展。Acute disseminated encephalomyelitis(ADEM)is a common inflammatory demyelinating disease of the central nervous system in children.The etiology is not yet clear,but it is currently mainly believed to be related to the activation of autoimmune reactions following infection.ADEM is typically a monophasic disease,unlike other demyelinating diseases such as multiple sclerosis and neuromyelitis optica spectrum disorders.With the increasing recognition,understanding,and detection of myelin oligodendrocyte glycoprotein antibodies in recent years,the diagnosis and treatment of ADEM may further evolve.The diagnosis of ADEM is clinical and requires exclusion of other infectious,inflammatory,neoplastic,and genetic conditions.The acute phase treatment methods include intravenous injection of high-dose corticosteroids,therapeutic plasma exchange,and intravenous immunoglobulin,with a generally good prognosis.This review summarizes the current knowledge on the pathogenesis,epidemiology,clinical features,diagnosis and differentiation,treatment,and prognosis of ADEM in children.

关 键 词：急性播散性脑脊髓炎 髓鞘少突胶质细胞糖蛋白抗体 多发性硬化 视神经脊髓炎谱系疾病 

分 类 号：R744.53[医药卫生—神经病学与精神病学]