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Atypical radiological presentation of progressive multifocal leukoencephalopathy following liver transplantation

Marco A Lima1, Douglas W Hanto, Michael P Curry

  • 1Department of Neurology, Division of Viral Pathogenesis, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA.

Journal of Neurovirology
|April 5, 2005
PubMed

Insights

Progressive multifocal leukoencephalopathy (PML) is a brain disease caused by JC virus (JCV). An atypical PML presentation in a liver transplant recipient showed contrast enhancement and mass effect, leading to a fatal outcome.

Area of Science:

  • Neuroscience
  • Immunology
  • Infectious Diseases

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare, fatal demyelinating disease of the central nervous system.
  • PML is caused by the ubiquitous JC virus (JCV) and typically affects immunocompromised individuals, such as organ transplant recipients.
  • Standard magnetic resonance imaging (MRI) findings include non-enhancing T2/FLAIR hyperintensities in the subcortical white matter.

Observation:

  • This report details an unusual case of PML in a liver transplant recipient.
  • The patient presented with atypical MRI findings, including contrast enhancement and significant mass effect.
  • These features are not characteristic of typical PML lesions.

Findings:

  • The atypical PML presentation was associated with a progressive clinical course.
  • Despite the presence of inflammatory signs (contrast enhancement), the outcome was fatal.
  • This contrasts with some reports suggesting better prognosis in inflammatory PML variants.

Implications:

  • This case highlights the importance of considering atypical PML presentations in immunosuppressed patients, even with concerning MRI features.
  • It underscores the variable clinical and radiological spectrum of PML.
  • Further research into the immunopathogenesis of PML may reveal novel therapeutic targets.

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