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Updated: Jun 3 2019

Acute Disseminated Encephalomyelitis (ADEM)

  • Snapshot
    • A 8-year-old boy presents to the pediatric emergency department for altered mental status. One day prior to presentation he reported a headache with mild lower extremity weakness. He also developed nausea and 2 episodes of nonbloody, nonbilious emesis. Of note, he developed a viral illness approximately 2 weeks ago. On physical examination, the patient is oriented to self but not to time or location. He has left-sided lower extremity weakness and hyperreflexia in the affected limb. A CT scan of the brain demonstrates mild hypodense white matter lesions. A lumbar puncture demonstrates a lymphocytic lymphocytosis with mildly elevated protein. After he was stabilized, an MRI brain and spine demonstrated hyperintense white matter lesions.
  • Introduction
    • Definition
      • an autoimmune demyelinating disease affecting the central nervous system
        • commonly triggered by viral infections
  • Epidemiology
    • Incidence
      • most common in children (especially) and adolescents
  • Etiology
    • Viral infections
    • Vaccinations (less common)
  • Pathogenesis
    • An environmental stimuli (e.g., infection) in a genetically susceptible host leads to an autoimmune response against the central nervous system
  • Presentation
    • Symptoms/physical exam
      • nonspecific signs
        • headache
        • fever
        • nausea
        • vomiting
      • encephalopathy
        • altered mental status
        • irritability and confusion
        • psychosis
        • somnolence/coma
      • other neurological deficits
        • oculomotor deficits
        • dysarthria
        • sensory changes
        • seizures
        • aphasia
        • optic neuritis
        • meningismus
  • Imaging
    • MRI brain with and without gadolinium
      • indication
        • imaging modality of choice in stabilized patients
  • Studies
    • Lumbar puncture
      • mild lymphocytic pleocytosis with mildly elevated protein is suggestive of ADEM
  • Differential
    • Multiple sclerosis (MS)
      • differentiating factors
        • ADEM typically follows a viral illnes while MS usually does not
        • MS presents with recurrent attacks separated by time and space
  • Treatment
    • Medical
      • high-dose intravenous corticosteroids (e.g., methylprednisolone)
        • indication
          • first-line treatment for ADEM
  • Complications
    • Severe neurological impairment
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