Tenembaum Silvia N
Department of Neurology, National Paediatric Hospital Dr. J. P. Garrahan, Buenos Aires, Argentina.
Clin Neurol Neurosurg. 2008 Nov;110(9):928-38. doi: 10.1016/j.clineuro.2007.12.018. Epub 2008 Feb 12.
The advent of MRI has contributed to increase the interest and awareness in childhood white matter disorders. A major priority is to distinguish transient and self-limited demyelinating syndromes like disseminated encephalomyelitis (DEM), from life-long diseases like multiple sclerosis (MS). However, the term DEM has been inconsistently applied across studies due to the lack of clear clinical and neuroimaging diagnostic criteria. The present review summarizes the available literature on DEM in children, outlines the main clinical and neuroimaging features at presentation, pathogenesis and outcome, and its differentiation from other conditions with acute impact in the CNS. The recently proposed clinical definitions for monophasic disseminated encephalomyelitis and its relapsing variants are discussed, and controversies surrounding the diagnosis of MS in children are addressed.
磁共振成像（MRI）的出现促使人们对儿童白质疾病的兴趣和认识有所增加。一个主要重点是将播散性脑脊髓炎（DEM）等短暂性和自限性脱髓鞘综合征与多发性硬化症（MS）等终身疾病区分开来。然而，由于缺乏明确的临床和神经影像学诊断标准，“DEM”这一术语在各项研究中的应用并不一致。本综述总结了关于儿童DEM的现有文献，概述了其发病时的主要临床和神经影像学特征、发病机制及转归，以及它与其他对中枢神经系统有急性影响的疾病的鉴别。文中还讨论了最近提出的单相播散性脑脊髓炎及其复发型的临床定义，并探讨了儿童MS诊断方面存在的争议。
