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Progressive multifocal leukoencephalopathy

Definition

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.

Alternative Names

PML

Causes, incidence, and risk factors

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:

  • AIDS (less common now because of better AIDS treatments)
  • Certain medicines used to treat multiple sclerosis, rheumatoid arthritis, and related conditions
  • Leukemia and lymphoma

Symptoms

  • Headaches
  • Loss of coordination, clumsiness
  • Loss of language ability (aphasia)
  • Memory loss
  • Vision problems
  • Weakness of the legs and arms that gets worse

Signs and tests

Tests may include:

Treatment

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.

Expectations (prognosis)

PML is a life-threatening condition. Talk to your doctor about care decisions.

References

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.

Review Date: 
2/27/2013
Reviewed By: 
Luc Jasmin, MD, PhD, Department of Neurosurgery, Cedars Sinai Medical Center, Los Angeles and Department of Anatomy, University of California, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.