Division of Pediatric Emergency Medicine, Department of Pediatrics, Dana-Dwek Children's Hospital, University of Tel Aviv, Israel.
Divisions of Pediatric Emergency Medicine and Clinical Pharmacology and Toxicology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Ontario, Canada.
Am J Emerg Med. 2019 Nov;37(11):2004-2007. doi: 10.1016/j.ajem.2019.02.022. Epub 2019 Feb 15.
Acute disseminated encephalomyelitis (ADEM) is an uncommon, treatable, primarily pediatric, immune-mediated disease. Diagnosis of ADEM requires two essential elements: typical clinical presentation and magnetic resonance imaging (MRI) findings. The aim of this study was to evaluate how clinical findings in the initial emergency department (ED) presentation influenced the timing of MRI.
A retrospective chart review was conducted of children diagnosed with ADEM, over a 12-year period, in a tertiary care pediatric center. Clinical presentation at ED admission was recorded and patients who underwent an MRI as part of their ED evaluation (early MRI) with those who had MRI performed during ward hospitalization (late MRI) were compared.
30 patients were diagnosed with ADEM during the study period. Encephalopathy and polyfocal neurological signs were described in 80% and 50% of patients ED charts, respectively. Seven patients underwent early MRI and polyfocal neurological signs were more common in this group (p = 0.006). Fever was more common in the late MRI group (p = 0.02). Following diagnosis, all patients were treated with immune-modulation therapy, improved clinically, and were discharged.
20% of ADEM patients were not encephalopathic at ED presentation. Polyfocal neurological signs and absence of fever at ED presentation were related to earlier MRI utilization and thus earlier diagnosis and treatment. Familiarity with the ADEM constellation of signs, and a high index of suspicion, may help the ED clinician in early diagnosis and treatment of this rare disease.
急性播散性脑脊髓炎（ADEM）是一种罕见的、可治疗的、主要发生于儿童的免疫介导性疾病。ADEM 的诊断需要两个基本要素：典型的临床表现和磁共振成像（MRI）结果。本研究的目的是评估初始急诊室（ED）表现中的临床发现如何影响 MRI 的时机。
对 12 年间在一家三级儿科中心诊断为 ADEM 的儿童进行了回顾性病历审查。记录 ED 入院时的临床表现，并将接受 MRI 检查作为 ED 评估一部分的患者（早期 MRI）与在病房住院期间进行 MRI 检查的患者（晚期 MRI）进行比较。
研究期间共有 30 例患者被诊断为 ADEM。脑病和多灶性神经体征分别在 80%和 50%的患者 ED 图表中描述。7 例患者接受了早期 MRI，该组中多灶性神经体征更为常见（p=0.006）。晚期 MRI 组发热更为常见（p=0.02）。确诊后，所有患者均接受免疫调节治疗，临床改善后出院。
20%的 ADEM 患者在 ED 就诊时无脑病表现。ED 就诊时出现多灶性神经体征和无发热与更早进行 MRI 检查以及更早诊断和治疗有关。熟悉 ADEM 的体征组合和高度怀疑可能有助于 ED 临床医生早期诊断和治疗这种罕见疾病。
