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Tumefactive Acute Disseminated Encephalomyelitis.

Michael G Z Ghali1

  • 1Department of Neurological Surgery, Houston Methodist Hospital, 6560 Fannin Street, Houston, TX, USA.

Neurology India
|March 5, 2020
PubMed
Summary
This summary is machine-generated.

Tumefactive demyelination in acute disseminated encephalomyelitis (ADEM) mimics tumors. Distinguishing tumefactive ADEM from multiple sclerosis and neoplasms relies on clinical history, imaging, and biopsy for accurate diagnosis and treatment.

Keywords:
Acute disseminated encephalomyelitisdemyelinationdifferentialmultiple sclerosistumefactive

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Area of Science:

  • Neuroscience
  • Neuropathology
  • Radiology

Background:

  • Tumefactive demyelination presents as central nervous system lesions resembling neoplasms radiographically.
  • While known in multiple sclerosis, tumefactive demyelination is increasingly reported in acute disseminated encephalomyelitis (ADEM).

Purpose of the Study:

  • To review literature on tumefactive ADEM.
  • To discuss clinical, imaging, and histopathologic features differentiating tumefactive ADEM from other conditions.

Main Methods:

  • Literature review of tumefactive ADEM cases.
  • Analysis of clinical manifestations, imaging characteristics, and histopathologic findings.
  • Comparison with neoplastic disease and multiple sclerosis diagnostic criteria.

Main Results:

  • Clinical and radiological data alone can be challenging for diagnosing tumefactive ADEM.
  • Biopsy is definitive: ADEM shows perivenular demyelination and mononuclear infiltration; multiple sclerosis shows confluent plaques.
  • Neoplastic disease exhibits nuclear atypia, hyperproliferation, necrosis, and vascular changes.

Conclusions:

  • Tumefactive ADEM requires careful differentiation from neoplasms and multiple sclerosis.
  • Histopathology is crucial for definitive diagnosis.
  • ADEM responds well to corticosteroids and is typically monophasic.