Santoro Jonathan D, Chitnis Tanuja
Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, United States.
Department of Neurology, Harvard Medical School, Boston, Massachusetts, United States.
Neuropediatrics. 2019 Oct;50(5):273-279. doi: 10.1055/s-0039-1693152. Epub 2019 Jul 24.
Acute disseminated encephalomyelitis (ADEM) is a common yet clinically heterogenous syndrome characterized by encephalopathy, focal neurologic findings, and abnormal neuroimaging. Differentiating ADEM from other demyelinating disorders of childhood can be difficult and appropriate interpretation of the historical, clinical, and neurodiagnostic components of a patient's presentation is critical. Myelin oligodendrocyte glycoprotein (MOG) antibody-associated diseases are a recently recognized set of disorders, which include ADEM presentations, among other phenotypes. This review article discusses the clinical diagnosis, differential diagnosis, interpretation of data, and treatment/prognosis of this unique syndrome with distinctive review of the spectrum of MOG antibodies.
急性播散性脑脊髓炎(ADEM)是一种常见但临床异质性的综合征,其特征为脑病、局灶性神经学表现和神经影像学异常。将ADEM与儿童期其他脱髓鞘疾病区分开来可能具有挑战性,对患者临床表现的病史、临床和神经诊断要素进行恰当解读至关重要。髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病是一组最近才被认识的疾病,其中包括ADEM表现及其他表型。本文综述讨论了这一独特综合征的临床诊断、鉴别诊断、数据解读以及治疗/预后,并对MOG抗体谱进行了特别综述。