Clinical outcome of children presenting with a severe manifestation of acute disseminated encephalomyelitis

Neuropediatrics. 2009 Oct;40(5):211-7. doi: 10.1055/s-0030-1247518. Epub 2010 Mar 10.


Background: Acute disseminated encephalomyelitis (ADEM) is an acute, inflammatory-demyelinating disorder of the CNS with a favourable outcome in the majority of cases.

Objective: The aim of this study was to examine the long-term outcome of children with an initially severe form of ADEM.

Methods: Children with ADEM according to the criteria of the International Pediatric MS Study Group (IPMSSG) referred to the rehabilitation centre Vogtareuth were included. Neurological impairment was evaluated with a standardized telephone-based interview assessing the EDSS score. Neuropsychological outcome was assessed with review of the medical records and a standardized parental questionnaire (KOPKIJ).

Results: Twelve children (1 year 9 months to 13 years of age) were included. All children had focal-neurological signs and changes in mental status at presentation and an MRI of the brain showing a range of white and gray matter lesions. 11/12 patients with a mean follow-up of 6.2 years (2-13.6 years) had a monophasic course of the disease. One child had a multiphasic ADEM. Two children had an EDSS score of 0, three an EDSS of 2, five an EDSS between 3 and 5 and two children had an EDSS score of 6 and 9. Results of a standardized parental questionnaire (KOPKIJ) revealed that 7 children had deficits in the categories alertness, memory, school performance, visual-spatial skills and/or impulse control.

Conclusion: The results of our study indicate that children with an initially severe manifestation of ADEM continue to have in the majority of cases neurological and neuropsychological handicaps.

MeSH terms

  • Adolescent
  • Brain / pathology
  • Child
  • Child, Preschool
  • Cognition Disorders / diagnosis
  • Cognition Disorders / etiology*
  • Cohort Studies
  • Encephalomyelitis, Acute Disseminated / complications*
  • Encephalomyelitis, Acute Disseminated / diagnosis*
  • Female
  • Humans
  • Infant
  • Magnetic Resonance Imaging / methods
  • Male
  • Neurologic Examination / methods
  • Neuropsychological Tests
  • Spinal Cord / pathology