Koelman Diederik L H, Mateen Farrah J
Department of Neurology, Massachusetts General Hospital, 165 Cambridge Street, #627, Boston, 02114, MA, USA.
Academic Medical Center - University of Amsterdam, Amsterdam, The Netherlands.
J Neurol. 2015 Sep;262(9):2013-24. doi: 10.1007/s00415-015-7694-7. Epub 2015 Mar 13.
Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory, demyelinating disorder of the CNS. Only in the past 15 years have larger groups of patients from several geographical areas been reported for comparisons across studies. In spite of the increased recognition of ADEM, the diagnosis of ADEM remains clinical, aided by neuroimaging confirmation, because of the lack of a biological marker. The diagnosis may be difficult, given that several diseases may present similar to ADEM. The controversial existence of multiphasic forms necessitates a continuous evaluation of the diagnosis by tracking subsequent events. Despite proposed consensus criteria, the diagnostic criteria employed to characterize ADEM range widely among the largest reported cohorts to date. This review comprehensively evaluates the current knowledge and controversies that surround ADEM, with special consideration of the distinction between ADEM and other demyelinating diseases such as multiple sclerosis. In addition, we present implications of the current knowledge of ADEM for both research and clinical practice.
急性播散性脑脊髓炎（ADEM）是一种罕见的中枢神经系统炎性脱髓鞘疾病。仅在过去15年里，才有来自几个地理区域的较大患者群体被报道用于跨研究比较。尽管对ADEM的认识有所提高，但由于缺乏生物学标志物，ADEM的诊断仍然依靠临床诊断，并借助神经影像学确认。鉴于几种疾病可能表现得与ADEM相似，诊断可能会很困难。多相形式存在争议，因此需要通过追踪后续事件对诊断进行持续评估。尽管有提议的共识标准，但在迄今为止报道的最大队列中，用于界定ADEM的诊断标准差异很大。本综述全面评估了围绕ADEM的现有知识和争议，特别考虑了ADEM与其他脱髓鞘疾病（如多发性硬化症）之间的区别。此外，我们还阐述了ADEM的现有知识对研究和临床实践的影响。
