What are unusual clinical presentations of acute disseminated encephalomyelitis (ADEM)?

Updated: Nov 08, 2018
  • Author: J Nicholas Brenton, MD; Chief Editor: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS  more...
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Answer

There are unusual presentations for possible ADEM that have uncertain classification. More literature is supporting a continuum of acute demyelinating diseases in childhood and adulthood.

  • Cases of pediatric patients diagnosed with neuromyelitis optica presenting with a clinical and radiographic evidence of ADEM have been reported. [73, 74, 76]

  • Cases of patients with anti-NMDA receptor encephalitis and ADEM-like lesions on MRI have also been reported. [77, 75]

  • Additionally, pediatric cases of ADEM followed by recurrent or monophasic optic neuritis have been described. [78]

  • Young children may manifest a rapidly progressive demyelinating illness that may be fatal within days to weeks and is almost universally associated with profound permanent psychomotor deficits in those who survive. Brain images differ from those typical of juvenile MS and may demonstrate confluent symmetric areas (butterfly pattern) of bright signal abnormality on T2-weighted sequences.

    • Fulminant presentation with lesions showing significant degrees of ring enhancement after contrast administration may also be found.

    • Malignant brain edema may be present, manifested by sulcal and ventricular effacement.

  • Some patients with the large tumor-like lesions, acute MS, or Schilder disease presentations during childhood or adolescence do remarkably well as compared to adults with similar presentations.


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