Acute disseminated encephalomyelitis
Acute disseminated encephalomyelitis
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DOI：
10.1212/01.wnl.0000259404.51352.7f
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发表时间：
2007-04-01
期刊：
影响因子：
9.9
通讯作者：
Hahn, Jin S.
中科院分区：
文献类型：
--
作者：
Tenembaum, Silvia;Chitnis, Tanuja;Hahn, Jin S.
急性播散性脑脊髓炎（ADEM）是一种免疫介导的中枢神经系统炎症性疾病，其特征是广泛的脱髓鞘，主要累及脑和脊髓的白色物质。这种情况通常是由病毒感染或疫苗接种引起的。表现特征包括急性脑病伴多灶性神经系统体征和缺损。儿童优先受到影响。在缺乏特异性生物学标志物的情况下，ADEM的诊断仍依赖于临床和影像学特征。虽然ADEM通常有一个单相过程，复发或多相形式已被报道，提高了诊断困难，以区分这些情况下，多发性硬化症（MS）。国际儿科MS研究小组提出了ADEM及其变体的统一定义。我们讨论了一些困难，在现有文献的解释，由于不同的术语和定义。此外，本文综述了ADEM的主要方面，包括其临床和放射学诊断特征，流行病学，发病机制和结果的当前知识。提供了儿童ADEM治疗的概述。最后，围绕儿科MS和ADEM的争议得到解决。
Acute disseminated encephalomyelitis ( ADEM) is an immune-mediated inflammatory disorder of the CNS characterized by a widespread demyelination that predominantly involves the white matter of the brain and spinal cord. The condition is usually precipitated by a viral infection or vaccination. The presenting features include an acute encephalopathy with multifocal neurologic signs and deficits. Children are preferentially affected. In the absence of specific biologic markers, the diagnosis of ADEM is still based on the clinical and radiologic features. Although ADEM usually has a monophasic course, recurrent or multiphasic forms have been reported, raising diagnostic difficulties in distinguishing these cases from multiple sclerosis ( MS). The International Pediatric MS Study Group proposes uniform definitions for ADEM and its variants. We discuss some of the difficulties in the interpretation of available literature due to the different terms and definitions used. In addition, this review summarizes current knowledge of the main aspects of ADEM, including its clinical and radiologic diagnostic features, epidemiology, pathogenesis, and outcome. An overview of ADEM treatment in children is provided. Finally, the controversies surrounding pediatric MS and ADEM are addressed.