Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical School, 5323 Harry Hines Blvd, Dallas, TX, 75390, USA.
Department of Pediatrics, University of Texas Southwestern Medical School, 5323 Harry Hines Blvd, Dallas, TX, 75390, USA.
Paediatr Drugs. 2021 May;23(3):213-221. doi: 10.1007/s40272-021-00441-7. Epub 2021 Apr 8.
Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system that typically presents in childhood and is associated with encephalopathy and multifocal brain lesions. Although ADEM is thought to be a post-infectious disorder, the etiology is still poorly understood. ADEM is often a monophasic disorder, in contrast to other demyelinating disorders such as multiple sclerosis and neuromyelitis optica spectrum disorder. With increasing awareness, understanding, and testing for myelin oligodendrocyte glycoprotein antibodies, this disease is now known to be a cause of pediatric ADEM and also has the potential to be relapsing. Diagnostic evaluation for ADEM involves neuroimaging and laboratory studies to exclude potential infectious, inflammatory, neoplastic, and genetic mimics of ADEM. Acute treatment modalities include high-dose intravenous corticosteroids, therapeutic plasma exchange, and intravenous immunoglobulin. Long-term outcomes for ADEM are generally favorable, but some children have significant morbidity related to the severity of acute illness and/or manifest ongoing neurocognitive sequelae. Further research related to the optimal management of pediatric ADEM and its impact on prognosis is needed. This review summarizes the current knowledge of the pathogenesis, epidemiology, clinical features, diagnostic evaluation, treatment approaches, and outcomes in pediatric ADEM.
急性播散性脑脊髓炎（ADEM）是一种中枢神经系统的炎症性脱髓鞘疾病，通常在儿童期发病，与脑病和多灶性脑损伤有关。尽管 ADEM 被认为是一种感染后疾病，但病因仍不清楚。ADEM 通常是单相疾病，与多发性硬化症和视神经脊髓炎谱系障碍等其他脱髓鞘疾病不同。随着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，目前已知该疾病是儿科 ADEM 的病因之一，也有可能复发。ADEM 的诊断评估包括神经影像学和实验室研究，以排除潜在的感染、炎症、肿瘤和遗传模拟 ADEM 的疾病。急性治疗方法包括大剂量静脉内皮质类固醇、治疗性血浆置换和静脉内免疫球蛋白。ADEM 的长期预后一般较好，但有些儿童因急性疾病的严重程度和/或出现持续的神经认知后遗症而存在较大的发病率。需要进一步研究儿科 ADEM 的最佳治疗方法及其对预后的影响。本综述总结了儿科 ADEM 的发病机制、流行病学、临床特征、诊断评估、治疗方法和结局的现有知识。
