Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2015 Dec 10:4:F1000 Faculty Rev-1424.
doi: 10.12688/f1000research.7071.1. eCollection 2015.

Progressive Multifocal Leukoencephalopathy

Laura Adang  1 , Joseph Berger  2
Affiliations
Review

Progressive Multifocal Leukoencephalopathy

Laura Adang et al. F1000Res. .

Abstract

Progressive multifocal leukoencephalopathy (PML) is a devastating demyelinating disease with significant morbidity and mortality and no effective, targeted therapies. It is most often observed in association with abnormalities of cell-mediated immunity, in particular human immunodeficiency virus (HIV) infection, but also occurs in association with lymphoproliferative diseases, certain immunosuppressive and immunomodulatory regimens, and other conditions. The etiologic agent of PML is a small, ubiquitous polyomavirus, the JC virus (JCV, also known as JCPyV), for which at least 50% of the adult general population is seropositive. PML results when JCV replicates within cerebral oligodendrocytes and astrocytes, leading to oligodendrocyte death and demyelination. Unfortunately, no treatments have been convincingly demonstrated to be effective, though some have been employed in desperation; treatment otherwise includes attempts to restore any immune system defect, such as the withdrawal of the causative agent if possible, and general supportive care.

Keywords: JC virus; Progressive multifocal leukoencephalopathy; demyelination; highly active antiretroviral therapy; immunocompromised.

PubMed Disclaimer

Conflict of interest statement

Competing interests: Laura Adang declares that she has no competing interests.

Joseph R. Berger is or has been a consultant to the following companies: Genentech, Genzyme, Incyte, Inhibikase, Johnson & Johnson, and Novartis. He serves or has served on the PML Adjudication Committees of the following companies: Amgen, AstraZeneca, Bristol-Myers Squibb, Eisai, Janssen, Millennium, PAREXEL, Pfizer, Roche, and Takeda.

No competing interests were disclosed.

References

    1. Astrom KE, Mancall EL, Richardson EP, Jr: Progressive multifocal leuko-encephalopathy; a hitherto unrecognized complication of chronic lymphatic leukaemia and Hodgkin's disease. Brain. 1958;81(1):93–111. 10.1093/brain/81.1.93 - DOI - PubMed
    1. Padgett BL, Walker DL, ZuRhein GM, et al. : Cultivation of papova-like virus from human brain with progressive multifocal leucoencephalopathy. Lancet. 1971;1(7712):1257–60. 10.1016/S0140-6736(71)91777-6 - DOI - PubMed
    1. White MK, Khalili K: Pathogenesis of progressive multifocal leukoencephalopathy--revisited. J Infect Dis. 2011;203(5):578–86. 10.1093/infdis/jiq097 - DOI - PMC - PubMed
    1. Engsig FN, Hansen AB, Omland LH, et al. : Incidence, clinical presentation, and outcome of progressive multifocal leukoencephalopathy in HIV-infected patients during the highly active antiretroviral therapy era: a nationwide cohort study. J Infect Dis. 2009;199(1):77–83. 10.1086/595299 - DOI - PubMed
    1. Berger JR, Aksamit AJ, Clifford DB, et al. : PML diagnostic criteria: consensus statement from the AAN Neuroinfectious Disease Section. Neurology. 2013;80(15):1430–8. 10.1212/WNL.0b013e31828c2fa1 - DOI - PMC - PubMed

LinkOut - more resources