儿童最初出现ADEM的疾病过程的特征和预测因素
Alice Rutatangwa1, Gregory Aaen2, Kristen M Krysko3
1UCSF Weill Institute for Neurosciences, Department of Neurology, University of California, San Francisco, CA, United States.
在儿童中诊断急性扩散性脑膜炎 (ADEM) 可能很困难. 较年轻的发病年龄和没有视神经炎或加多增强表明ADEM的单相过程.
科学领域:
- 儿科神经学 儿科神经学
- 神经免疫学 神经免疫学
- 脱线性疾病 脱线性疾病
背景情况:
- 急性扩散性脑膜炎 (ADEM) 呈现出急性多焦点神经症状,脑病变和多焦点脱,通常在儿童时期.
- 初步诊断ADEM具有挑战性,高达20%的患有多发性硬化症 (MS) 或神经omyelitis optica光谱障碍 (NMOSD) 的儿童最初被误诊为ADEM.
研究的目的:
- 描述单相ADEM儿童与复发性脱髓化综合征之间的特征.
- 为了确定临床和成像特征预测的单相过程在儿科ADEM.
主要方法:
- 多中心观察性研究涉及837名被诊断为ADEM,MS或NMOSD的儿童在12个儿科MS中心.
- 用描述性统计和后勤回归分析来分析患者特征,临床/成像特征和结果.
- 随访数据平均为5.7年,用于评估诊断稳定性和重新分类率.
主要成果:
- 与MS/NMOSD组相比,ADEM患者更年轻 (平均年龄5.2岁),主要是男性 (66%).
- 2年后,83%最初诊断的ADEM病例保持了他们的诊断.
- 发病时的年龄较大,视神经炎和加多增强性病变与从ADEM重新分类到MS,NMOSD或其他脱髓化疾病 (DDNOS) 有关.
结论:
- 患有单相ADEM的儿童在发病时通常较年轻,并且不太可能出现视神经炎或加多增强性病变.
- 这些特征可以帮助区分单相ADEM与儿童的其他脱髓化疾病,提高诊断准确度.
- 准确的初始诊断对于儿童脱髓化疾病的适当管理和预后至关重要.
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