Fulminant form of acute disseminated encephalomyelitis: successful treatment with hypothermia

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Published on: March 3, 2021

T Takata1, M Hirakawa, M Sakurai

  • 1Department of Neurology, Graduate School of Medicine, University of Tokyo, Japan.

Journal of the Neurological Sciences
|July 30, 1999
PubMed

Insights

Fulminant acute disseminated encephalomyelitis (ADEM) after mycoplasma pneumonia is often fatal. Systemic hypothermia showed marked recovery in a patient, suggesting its potential as a novel treatment for severe ADEM.

Area of Science:

  • Neurology
  • Infectious Diseases
  • Critical Care Medicine

Background:

  • Acute disseminated encephalomyelitis (ADEM) is a rare, immune-mediated inflammatory demyelinating disease of the central nervous system.
  • Fulminant ADEM presents with rapid neurological deterioration and has a high mortality rate.
  • Mycoplasma pneumonia is a known, albeit uncommon, trigger for ADEM.

Observation:

  • A 28-year-old male presented with fever, headache, and aphasia following Mycoplasma pneumonia.
  • Despite surgical decompression and corticosteroids, the patient experienced progressive neurological decline.
  • Systemic hypothermia was initiated, leading to significant clinical improvement, including preserved language comprehension and communication via gestures.

Findings:

  • Head MRI revealed extensive white matter demyelination consistent with ADEM.
  • Cerebrospinal fluid analysis showed polymorphonuclear dominant pleocytosis, mimicking acute hemorrhagic leukoencephalitis.
  • Brain biopsy results were inconclusive, highlighting diagnostic challenges in fulminant ADEM.

Implications:

  • Systemic hypothermia may be a viable therapeutic option for fulminant ADEM, potentially mitigating brain edema and immune responses.
  • This case expands treatment strategies beyond corticosteroids and intravenous immunoglobulin for severe ADEM.
  • Further research is warranted to establish the efficacy and safety of hypothermia in managing fulminant ADEM.

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