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.
Anyone with a weakened immune system, however, are at greater risk of developing PML. Causes of a weakened immune system include:
- AIDS (less common now because of better AIDS treatments)
- Certain medications 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:
- Brain biopsy (rare)
- Cerebrospinal fluid test for the JCV
- CT scan of the brain
- Electroencephalogram (EEG)
- MRI of the brain
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
Boren EJ, Cheema GS, et al. The emergence of progressive multifocal leukoencephaloparhy (PML) in rheumatic diseases. J Autoimmun. 2008;30(1-2):90-98.
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Luc Jasmin, MD, PhD, Department of Neurosurgery at Cedars-Sinai Medical Center, Los Angeles, CA, and Department of Anatomy at UCSF, San Francisco, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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