Acute disseminated encephalomyelitis history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

ADEM has an abrupt onset and a monophasic course. Symptoms usually begin 1-3 weeks after infection or vaccination. Major symptoms include fever, headache, drowsiness, seizures and coma. Although initially the symptoms are usually mild, later in the course of the disease patients may even die, if they are not treated properly. Some patients recover completely, while others have permanent neurological impairments.

History and Symptoms

• The disease presents with progressive, often abrupt, neurologic deterioration. The clinical course is quite variable, and in some cases, rapid progression occurs over hours to days.
• ADEM classically follows a monophasic course, though there are now discussions in the literature about “multiphasic ADEM”
• Features include:

  • Somnolence, confusion, lethargy
  • Fever – that had previously resolved if there was a precipitating illness
  • Headache
  • Meningismus
  • Motor features may include ataxia, myoclonic movements, and choreoathetosis
  • Seizures
  • Decerebrite rigidity may develop in severe cases
  • Paraplegia, quadraplegia, absent deep tendon reflexes (DTRs), sensory levels, and bladder or bowel involvement suggest prominent spinal cord involvement
  • Coma

• Signs of disseminated neurologic disease are usually present

  • Motor findings:

    • Hemiparesis
    • Quadriparesis
    • Extensor plantar responses

  • DTR’s may be lost initially, and later become hyperactive
  • Sensory findings are commonly present
  • Brainstem involvement may be present
  • Cerebellar involvement is particularly prominent in ADEM that occurs as a consequence of chickenpox

• In post-exantham disease, the rash usually appears 2-4 days before the neurologic manifestations, and is typically fading at the onset of neurologic disease.
• Though most cases demonstrate widespread disease (brain, spinal cord, optic nerves, etc), neurologic features may be limited to spinal cord features (transverse myelitis), cerebellar features, etc.