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Prolonged progressive multifocal leukoencephalopathy without immunosuppression.

D W Zochodne1, J C Kaufmann

  • 1Department of Clinical Neurological Sciences, University Hospital, University of Western Ontario, London, Canada.

The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques
|November 1, 1987
PubMed
Summary
This summary is machine-generated.

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Atypical progressive multifocal leukoencephalopathy (PML) can mimic other diseases. This case highlights a rare, prolonged PML presentation without evident immunosuppression, emphasizing diagnostic challenges and varied disease courses.

Area of Science:

  • Neurology
  • Neurovirology
  • Pathology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease.
  • PML is typically associated with immunosuppression and caused by the JC virus.

Observation:

  • A 70-year-old female presented with progressive neurological decline over 43 months, including gait instability, dysarthria, and dementia.
  • The patient's condition mimicked a primary degenerative neurological disorder.
  • PML was not suspected clinically before death.

Findings:

  • Neuropathological examination revealed characteristic PML lesions in the deep cerebral white matter.
  • Microscopic findings included gemistocytic astrocytes and oligodendrocytes with intranuclear inclusions.
  • Electron microscopy confirmed the presence of papovavirus particles within the inclusions.

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  • No underlying immunosuppressive condition was identified pre- or post-mortem.

    • Implications:

    • This case underscores the potential for atypical PML presentations that can evade clinical suspicion.
    • The prolonged course and lack of apparent immunosuppression challenge typical PML diagnostic paradigms.
    • It highlights the importance of considering PML in the differential diagnosis of unexplained neurological deterioration, even in the absence of known risk factors.