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"Gliomatosis cerebri" simulating an acute diffuse encephalomyelitis. Case report

C L Lancellotti1, M F Amary, A M Barbastefano

  • 1Departamento de Ciências Patológicas, Faculdade de Ciências, Médicas (FCM) da Santa Casa de São Paulo.

Arquivos De Neuro-Psiquiatria
|June 18, 1998
PubMed
Summary

This case study details a fatal central nervous system disorder in a young male, initially mimicking acute diffuse encephalomyelitis. Autopsy revealed gliomatosis cerebri, a diffuse brain tumor, as the underlying cause.

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Area of Science:

  • Neuropathology
  • Neuroradiology
  • Neuro-oncology

Background:

  • A 17-year-old male presented with progressive neurological deficits including hemiparesis and cranial nerve impairment.
  • Initial symptoms mimicked acute diffuse encephalomyelitis, prompting neuroradiologic investigation.

Observation:

  • Magnetic resonance imaging revealed multiple, non-mass-effect lesions suggestive of myelin loss.
  • Neuropathologic examination showed a heavy brain with herniations and diffuse blastomatous proliferation.
  • Histopathology identified tumor cells with glial phenotypes (astrocytic, oligodendrocytic, spongioblastic) and focal anaplastic changes.

Findings:

  • The diffuse blastomatous proliferation infiltrated brain tissue without complete destruction.
  • Tumor cells exhibited glial differentiation and GFAP-positive reactivity.

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  • The final diagnosis of gliomatosis cerebri was established post-mortem.

    • Implications:

    • This case highlights the diagnostic challenges of gliomatosis cerebri, particularly when presenting with symptoms mimicking inflammatory demyelinating diseases.
    • Early recognition and accurate diagnosis are crucial for potential therapeutic interventions in gliomatosis cerebri.
    • The autopsy confirmed gliomatosis cerebri as the cause of the patient's rapid neurological decline and death.