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Definition:
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Progressive multifocal leukoencephalopathy (PML) is a rare disorder that damages the material (myelin) that covers and protects nerves in the white matter of the brain.
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Causes, incidence, and risk factors:
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The JC virus (JCV) causes PML. By age 10, most people have been infected with this virus. But it hardly ever causes symptoms. Persons with a weakened immune system, though, are at risk of developing PML. Causes of a weakened immune system include:
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AIDS (less common now because of better AIDS treatments)
- Certain medicines used to treat multiple sclerosis, rheumatoid arthritis, and related conditions
- Leukemia and lymphoma
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Symptoms:
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- Headaches
- Loss of coordination, clumsiness
- Loss of language ability (aphasia)
- Memory loss
- Vision problems
- Weakness of the legs and arms that gets worse
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Signs and tests:
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Tests may include:
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Treatment:
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In people with AIDS, treatment to strengthen the immune system can lead to recovery from the symptoms of PML. No other treatments have proved effective for PML.
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Expectations (prognosis):
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PML is a life-threatening condition. Talk to your doctor about care decisions.
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References:
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Tan CS, Koralnik IJ. JC, BK, and other polyomaviruses: progressive multifocal leukoencephalopathy. In: Mandell GL, Bennett JE, Dolin R. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 7th ed. Philadelphia, PA: Elsevier Churchill-Livingstone; 2009:chap 145.
Weissert R. Progressive multifocal leukoencephalopathy. Journal of Neuroimmunology. 2011; 231 (1): 73-77.
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