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Infratentorial progressive multifocal leukoencephalopathy in a patient treated with fludarabine and rituximab

Simona Bonavita1, Renata Conforti, Antonio Russo

  • 1Department of Neurological Sciences, II University of Naples, Piazza Miraglia 2, 80138 Naples, Italy. simona.bonavita@unina2.it

Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
|April 2, 2008
PubMed

Insights

Progressive multifocal leukoencephalopathy (PML) can occur late after chemotherapy in patients with lymphoproliferative diseases (LPDs). Atypical MRI findings and delayed onset warrant suspicion for PML in LPD patients.

Area of Science:

  • Neurology
  • Oncology
  • Infectious Diseases

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease.
  • PML is a known complication in patients with lymphoproliferative diseases (LPDs) undergoing chemotherapy.
  • JC papovavirus is the causative agent of PML.

Purpose of the Study:

  • To report an atypical case of PML in a patient with B-cell LPD.
  • To highlight unusual clinical and radiological features of PML.
  • To emphasize the importance of considering PML in LPD patients with neurological deficits.

Main Methods:

  • Case report of a 59-year-old woman with B-cell LPD.
  • Patient received three cycles of fludarabine and rituximab chemotherapy.
  • Analysis of clinical presentation, MRI findings, and patient outcome.

Main Results:

  • Atypical PML developed six months after the last chemotherapy cycle.
  • Chemotherapy regimen was not heavy or prolonged.
  • Neurological symptoms onset was unexpectedly late.
  • MRI showed a monofocal, infratentorial lesion with early gadolinium enhancement and mass effect.
  • Prolonged survival was observed despite no specific PML treatment.

Conclusions:

  • Late-onset PML should be suspected in LPD patients with progressive neurological deficits.
  • Atypical MRI findings (monofocal, infratentorial, with enhancement and mass effect) can occur in non-HIV-related PML.
  • These findings suggest a need for heightened vigilance for PML in LPD patients, even with delayed presentation.

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