Acute disseminated encephalomyelitis: current controversies in diagnosis and outcome


Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory, demyelinating disorder of the CNS. Only in the past 15 years have larger groups of patients from several geographical areas been reported for comparisons across studies. In spite of the increased recognition of ADEM, the diagnosis of ADEM remains clinical, aided by neuroimaging confirmation, because of the lack of a biological marker. The diagnosis may be difficult, given that several diseases may present similar to ADEM. The controversial existence of multiphasic forms necessitates a continuous evaluation of the diagnosis by tracking subsequent events. Despite proposed consensus criteria, the diagnostic criteria employed to characterize ADEM range widely among the largest reported cohorts to date. This review comprehensively evaluates the current knowledge and controversies that surround ADEM, with special consideration of the distinction between ADEM and other demyelinating diseases such as multiple sclerosis. In addition, we present implications of the current knowledge of ADEM for both research and clinical practice.

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We would like to thank Prof. Dr. E. T. Hedley-Whyte, Department of Pathology, Massachusetts General Hospital, for reviewing and photographing the pathology images of patients with ADEM seen in Fig. 1.

Conflicts of interest

The authors declare that they have no conflict of interest.

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Corresponding author

Correspondence to Farrah J. Mateen.

Appendix: Identification of cohorts

Appendix: Identification of cohorts

Literature review PubMed was searched using the key term: “Acute Disseminated Encephalomyelitis”. Results were limited to the English language. All titles that were identified during the final search that was conducted on December 1, 2014 were reviewed. Reference lists of related articles were reviewed in effort to identify additional studies. All cohorts that described more than 50 cases of ADEM were included. In case of overlap of published cohorts, the most recent cohort was selected. Cohorts that were not comprehensively described were excluded from the table, but mentioned elsewhere.

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Koelman, D.L.H., Mateen, F.J. Acute disseminated encephalomyelitis: current controversies in diagnosis and outcome. J Neurol 262, 2013–2024 (2015).

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  • Acute disseminated encephalomyelitis
  • Autoimmune diseases
  • Encephalopathy
  • Postinfectious