Progressive multifocal leukoencephalopathy (PML)

Lisa A. Spacek, M.D., Ph.D.
Progressive multifocal leukoencephalopathy (PML) is a topic covered in the Johns Hopkins HIV Guide.

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PATHOGENS

  • JC virus, a ubiquitous, neurotropic ds-DNA polyomavirus, infects primarily oligodendrocytes, as well as astrocytes and neurons.[2]
  • Primarily affects deep and subcortical white matter of brain and can expand into cortical gray matter. Rarely involves spinal cord. Spares optic nerve.[7]
  • Virus resides in extraneural reservoirs, i.e., lymphoid tissue, kidney, and bone marrow. Excreted in urine of healthy individuals and those with PML.[10]
  • Seroprevalence of 70-80%, antibody not protective, those with deficient cellular response, e.g. HIV/AIDS, organ transplantation, hematologic malignancies, and those receiving immunosuppressive therapies, are at risk for PML.[1]

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PATHOGENS

  • JC virus, a ubiquitous, neurotropic ds-DNA polyomavirus, infects primarily oligodendrocytes, as well as astrocytes and neurons.[2]
  • Primarily affects deep and subcortical white matter of brain and can expand into cortical gray matter. Rarely involves spinal cord. Spares optic nerve.[7]
  • Virus resides in extraneural reservoirs, i.e., lymphoid tissue, kidney, and bone marrow. Excreted in urine of healthy individuals and those with PML.[10]
  • Seroprevalence of 70-80%, antibody not protective, those with deficient cellular response, e.g. HIV/AIDS, organ transplantation, hematologic malignancies, and those receiving immunosuppressive therapies, are at risk for PML.[1]

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Last updated: August 24, 2021