Progressive Multifocal Leukoencephalopathy
Pronounced: LOO-koh-en-sef-ah-LOP-ah-theeEn Español (Spanish Version)
Progressive multifocal leukoencephalopathy is a rare, slowly progressive disease of the nervous system. It is caused by a viral infection of oligodendrocytes, the cells that produce myelin.
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PML is caused by a papovavirus, usually the JC strain. Most people acquire this viral infection in childhood and it produces no illness. It reactivates later in life in immunocompromised patients, most often in the setting of HIV/AIDS , producing progressive multifocal leukoencephalopathy.
Prior to the onset of HIV and AIDS in the 1980s, PML was extremely rare. The most common risk factor is HIV or AIDS, with up to 4% of AIDS patients developing PML.
Reactivation of the JC virus and development of PML has rarely been associated with leukemia and lymphoma, tuberculosis , sarcoidosis , prolonged immunosuppression (organ transplant or cancer patients), chronic steroid therapy, and rare inherited immunodeficiencies.
Most recently, the use of natalizimab has been reported to increase the risk of developing PML, but this appears to occur when the drug is used in combination with other immunosuppressants.
If you experience any of these symptoms do not assume it is due to PML. These symptoms may be caused by other health conditions. Symptoms progress over weeks and include:
Your doctor will ask about your symptoms and medical history, and perform a physical exam. Tests may include the following:
While there is no cure for PML, talk with your doctor about the best supportive treatment plan for you.
National Organization for Rare Disorders (NORD)
Canadian AIDS Society
Canadian Organization for Rare Disorders
NINDS progressive multifocal leukoencephalopathy information page. National Institute of Neurological Disorders and Stroke website. Available at http://www.ninds.nih.gov/disorders/pml/pml.htm . Accessed September 30, 2005.
Progressive multifocal leukoencephalopathy. EBSCO Publishing Dynamed website. Available from DynaMed: http://www.ebscohost.com/dynamed . Updated January 10, 2008. Accessed February 23, 2008.
Progressive multifocal leukoencephalopathy. Medline Plus Medical Encyclopedia website. Available at: http://www.nlm.nih.gov/medlineplus/ency/article/000674.htm . Accessed September 30, 2005.
Last reviewed February 2008 by J. Thomas Megerian, MD, PhD, FAAP
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