National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Progressive multifocal leukoencephalopathy


Other Names:
Leukoencephalopathy, progressive multifocal; PML
Categories:
Progressive multifocal leukoencephalopathy (PML) is a neurological disorder that damages the myelin that covers and protects nerves in the white matter of the brain. It is caused by the JC virus (JCV). By age 10, most people have been infected with this virus, but it rarely causes symptoms unless the immune system becomes severely weakened.[1][2] The disease occurs, rarely, in organ transplant patients; people undergoing chronic corticosteroid or immunosuppressive therapy; and individuals with cancer, such as Hodgkin’s disease, lymphoma, and sarcoidosis.  PML is most common among individuals with acquired immune deficiency syndrome (AIDS).[3]
Last updated: 4/1/2011
Currently, the best available therapy is reversal of the immune-deficient state. This can sometimes be accomplished by alteration of chemotherapy or immunosuppression. In the case of HIV-associated PML, immediately beginning anti-retroviral therapy will benefit most individuals.[3]
Last updated: 3/3/2011
The mortality rates for those with HIV-PML have fallen dramatically from approximately 90 percent to around 50 percent according to most reports. For non-AIDS individuals with PML, the prognosis remains grim; the disease usually lasts for months and 80 percent die within the first 6 months, although spontaneous improvement has been reported.  Those who survive PML can be left with severe neurological disabilities.[3]
Last updated: 3/3/2011

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Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Progressive multifocal leukoencephalopathy. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

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Financial Resources

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.
  • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Progressive multifocal leukoencephalopathy. Click on the link to view a sample search on this topic.

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  1. Dugdale DC, Jasmin L. Progressive multifocal leukoencephalopathy. MedlinePlus. 2010; http://www.nlm.nih.gov/medlineplus/ency/article/000674.htm. Accessed 3/3/2011.
  2. Koralnik IJ. Progressive Multifocal Leukoencephalopathy. National Organization for Rare Disorders (NORD). 2010; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Progressive%20Multifocal%20Leukoencephalopathy. Accessed 3/3/2011.
  3. NINDS Progressive Multifocal Leukoencephalopathy Information Page. National Institute of Neurological Disorders and Stroke (NINDS). 2010; http://www.ninds.nih.gov/disorders/pml/pml.htm. Accessed 3/3/2011.