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Progressive multifocal leukoencephalopathy.

J R Berger1, E O Major

  • 1Department of Neurology, University of Kentucky College of Medicine, Lexington 40536-0284, USA.

Seminars in Neurology
|March 16, 2000
PubMed
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Progressive multifocal leukoencephalopathy (PML) affects up to 5% of AIDS patients, caused by JC virus. While survival is poor, highly active antiretroviral therapy (HAART) shows a positive impact.

Area of Science:

  • Neurology
  • Virology
  • Immunology

Background:

  • Progressive multifocal leukoencephalopathy (PML) was historically rare, associated with leukemia/lymphoma.
  • PML now affects up to 5% of patients with acquired immunodeficiency syndrome (AIDS).
  • The disease is caused by the JC virus (JCV), a papovavirus prevalent in the general population.

Purpose of the Study:

  • To describe the characteristics and outcomes of PML in the context of the AIDS epidemic.
  • To highlight diagnostic criteria and the impact of treatment on survival.

Main Methods:

  • Review of clinical presentation, diagnostic methods (MRI, CSF PCR), and outcomes.
  • Analysis of survival data in relation to CD4 counts and treatment with highly active antiretroviral therapy (HAART).

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Main Results:

  • PML can occur even with CD4 counts above 200 cells/mm3.
  • Focal neurological deficits and white matter abnormalities on MRI are key indicators.
  • Positive CSF PCR for JCV DNA is a reliable diagnostic marker, often negating the need for brain biopsy.
  • Median survival for AIDS-associated PML is approximately 6 months, with some patients experiencing prolonged survival (>12 months).

Conclusions:

  • AIDS-associated PML is a significant opportunistic infection with poor prognosis.
  • Early diagnosis via MRI and CSF PCR is crucial.
  • HAART has demonstrated a beneficial effect on survival for patients with AIDS-associated PML.