Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children
Clinical and neuroradiologic features of acute disseminated encephalomyelitis in children
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DOI：
10.1212/wnl.56.10.1308
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发表时间：
2001-05-22
期刊：
影响因子：
9.9
通讯作者：
Kean, MJ
中科院分区：
文献类型：
--
作者：
Hynson, JL;Kornberg, AJ;Kean, MJ
目的：确定儿童急性播散性脑脊髓炎（ADEM）的临床和神经放射学特征。方法：对 1993 年 1 月至 1998 年 12 月期间因 ADEM 到墨尔本皇家儿童医院就诊的儿童的病历和 MRI 进行回顾性审查。结果：在本研究纳入的 31 名患者中，22 名 (71%) 经历过前驱疾病。两名患者 (6%) 在发病前 3 至 6 周接种了乙型肝炎疫苗。症状和体征通常会在几天内形成。共济失调是最常见的表现特征，发生在 20 名患者 (65%) 中。 MRI 结果各不相同，但病变最常见于额叶和顶叶的双侧且不对称。作者发现胼胝体和脑室周围变化的发生率较高，通常与多发性硬化症相关，但他们也发现深部灰质受累率较高（61% 的患者）。使用高剂量静脉注射甲基强的松龙通常与快速恢复有关。百分之八十一的患者完全康复，其余儿童仅留下轻微后遗症。结论：在缺乏生物标志物的情况下，在首次出现时无法确定 ADEM 和 MS 之间的区别，但作者认为，病毒前驱症状、早发性共济失调、MRI 上的高病变负荷、深部灰质受累以及寡克隆带缺失更能说明 ADEM。
Objective: To identify the clinical and neuroradiologic features of acute disseminated encephalomyelitis (ADEM) in childhood. Methods: A retrospective review was conducted of the medical records and MRI of children who presented to the Royal Children's Hospital in Melbourne with ADEM between January 1993 and December 1998. Results: Of the 31 patients included in this study, 22 (71%) experienced a prodromal illness. Two patients (6%) had received hepatitis B vaccine 3 to 6 weeks before developing their illness. Symptoms and signs typically evolved over several days. Ataxia was the most common presenting feature, occurring in 20 patients (65%). MRI findings were variable, but lesions were most commonly seen bilaterally and asymmetrically in the frontal and parietal lobes. The authors found a high incidence of the corpus callosal and periventricular changes more typically associated with MS, but they also found a high rate of deep gray matter involvement (61% of patients). The use of high-dose IV methylprednisolone was usually associated with rapid recovery. Eighty-one percent of patients recovered completely, with only mild sequelae recorded in the remaining children. Conclusion: In the absence of a biological marker, the distinction between ADEM and MS cannot be made with certainty at the time of first presentation, but the authors suggest that a viral prodrome, early-onset ataxia, high lesion load on MRI, involvement of the deep gray matter, and absence of oligoclonal bands are more indicative of ADEM.