Abstract

We aimed to determine whether acute disseminated encephalomyelitis (ADEM) diagnosis in children is delayed, and if so, to identify the clinical risk factors of delayed diagnosis. Standardised data were collected from children with ADEM from 2003 to 2020. Overall diagnostic delay (time between symptom onset and ADEM diagnosis), physicians’ delay (between the first medical visit and ADEM diagnosis), and patients’ delay (between symptom onset and the first medical visit) were analysed. Thirty ADEM patients were identified, including 16 (54%) with neurological deficits at discharge. Overall, physicians’, and patients’ delays were 9 (interquartile range [IQR] 6–20.5), 5.5 (IQR 3–14), and 4 (IQR 2–8) days, respectively. Overall delay was significantly associated with physicians’ delay, but not with patients’ delay. There were 61 misdiagnoses among 25 (83%) patients, while 5 (17%) were diagnosed correctly at the first visit. The misdiagnoses of common respiratory and gastrointestinal infection and aseptic meningitis were associated with overall and/or physicians’ delay. Later onset of specific neurological features suggestive of ADEM was associated with all three diagnostic delays. A unique diagnostic odyssey exists in ADEM. Several clinical risk factors were associated with the diagnostic delay.

Highlights

  • We aimed to determine whether acute disseminated encephalomyelitis (ADEM) diagnosis in children is delayed, and if so, to identify the clinical risk factors of delayed diagnosis

  • Encephalopathy, the key to diagnosis based on International Pediatric Multiple Sclerosis Study Group c­ riteria[4], may present with vague, subtle, and transient sleepiness or irritability, especially in paediatric patients

  • Children with Acute disseminated encephalomyelitis (ADEM) may initially be treated with broad-spectrum antibiotics and antiviral a­ gents[2], since clinical features of ADEM often resemble those of acute central nervous system (CNS) infections, which may lead to drug-related adverse effects and additional costs

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Summary

Introduction

We aimed to determine whether acute disseminated encephalomyelitis (ADEM) diagnosis in children is delayed, and if so, to identify the clinical risk factors of delayed diagnosis. Later onset of specific neurological features suggestive of ADEM was associated with all three diagnostic delays. Acute disseminated encephalomyelitis (ADEM) is an acquired demyelinating disorder of the central nervous system (CNS) in children and young ­adults[1]. It typically affects the subcortical white matter and is characterised by monophasic encephalopathy and polyfocal neurological s­ ymptoms[2]. Our data and visual representation of the diagnostic timeline will help future physicians diagnose ADEM early in the disease course

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