Progressive multifocal leukoencephalopathy
PML; John Cunningham virus; JCV
Progressive multifocal leukoencephalopathy (PML) is a rare infection that damages the material ( myelin ) that covers and protects nerves in the white matter of the brain .
Myelin
Myelin is an insulating layer, or sheath that forms around nerves, including those in the brain and spinal cord. It is made up of protein and fatty ...
White matter of the brain
White matter is found in the deeper tissues of the brain (subcortical). It contains nerve fibers (axons), which are extensions of nerve cells (neuro...
Causes
The John Cunningham virus, or JC virus (JCV) causes PML. By age 10, most people have been infected with this virus. But it hardly ever causes symptoms. But, people with a weakened immune system are at risk of developing PML. Causes of a weakened immune system include:
-
HIV/
AIDS
(less common now because of better AIDS treatments).
AIDS
Human immunodeficiency virus (HIV) is the virus that causes AIDS. When a person becomes infected with HIV, the virus attacks and weakens the immune ...
- Certain medicines that suppress the immune system. Such medicines may be used to prevent organ transplant rejection or to treat multiple sclerosis, rheumatoid arthritis, and related conditions.
- Cancers, such as leukemia and Hodgkin lymphoma.
Symptoms
Symptoms may include any of the following:
- Loss of coordination, clumsiness
- Loss of language ability (aphasia)
- Memory loss
- Vision problems
- Weakness of the legs and arms that gets worse
- Personality changes
Exams and Tests
The health care provider will perform a physical exam and ask about symptoms.
Tests may include:
- Brain biopsy (in rare cases)
- Cerebrospinal fluid test for the JCV
-
CT scan of the brain
CT scan of the brain
A computed tomography (CT) scan is an imaging method that uses x-rays to create pictures of cross-sections of the body. Related tests include:Abdomin...
-
Electroencephalogram
(EEG)
Electroencephalogram
An electroencephalogram is a test to measure the electrical activity of the brain.
-
MRI
of the brain
MRI
A magnetic resonance imaging (MRI) scan is an imaging test that uses powerful magnets and radio waves to create pictures of the body. It does not us...
Treatment
In people with HIV/AIDS, treatment to strengthen the immune system can lead to recovery from the symptoms of PML. No other treatments have proved effective for PML.
Outlook (Prognosis)
PML is a life-threatening condition. Depending on how severe the infection is, up to one half of people diagnosed with PML die within the first few months.Talk to your provider about care decisions.
References
Berger JR, Nath A. Cytomegalovirus, Epstein-Barr virus, and slow virus infections of the central nervous system. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 370.
Tan CS, Koralnik IJ. JC, BK, and other polyomaviruses: progressive multifocal leukoencephalopathy (PML). In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases . 8th ed. Philadelphia, PA: Elsevier Saunders; 2015:chap 147.
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Gray and white matter of the brain - illustration
The tissue called "gray matter" in the brain and spinal cord is also known as substantia grisea, and is made up of cell bodies. "White matter", or substantia alba, is composed of nerve fibers.
Gray and white matter of the brain
illustration
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Leukoencephalopathy - illustration
Progressive multifocal leukoencephalopathy is an advancing viral inflammation of the white matter of the brain. Immunosuppressed people are more susceptible to this disorder than the general population. Evidence of the disease may be a person's recent loss of coordination and weakness, progressing to a loss of language, visual problems and headaches.
Leukoencephalopathy
illustration
-
Gray and white matter of the brain - illustration
The tissue called "gray matter" in the brain and spinal cord is also known as substantia grisea, and is made up of cell bodies. "White matter", or substantia alba, is composed of nerve fibers.
Gray and white matter of the brain
illustration
-
Leukoencephalopathy - illustration
Progressive multifocal leukoencephalopathy is an advancing viral inflammation of the white matter of the brain. Immunosuppressed people are more susceptible to this disorder than the general population. Evidence of the disease may be a person's recent loss of coordination and weakness, progressing to a loss of language, visual problems and headaches.
Leukoencephalopathy
illustration
Review Date: 2/27/2016
Reviewed By: Amit M. Shelat, DO, FACP, Attending Neurologist and Assistant Professor of Clinical Neurology, SUNY Stony Brook, School of Medicine, Stony Brook, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.