Progressive Multifocal Leukoencephalopathy

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Progressive Multifocal Leukoencephalopathy
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Specialty scores for Progressive Multifocal Leukoencephalopathy

Overview

Source: In partnership with Genetic and Rare Diseases Information Center, funded by the National Center for Advancing Translational Sciences, and the National Human Genome Research Institute https://rarediseases.info.nih.gov/gard.

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Progressive multifocal leukoencephalopathy

overview Question

What is progressive multifocal leukoencephalopathy?

Progressive multifocal leukoencephalopathy (PML) is a neurological disorder that damages the myelin that covers and protects nerves in the white matter of the brain. It is caused by the JC virus (JCV). By age 10, most people have been infected with this virus, but it rarely causes symptoms unless the immune system becomes severely weakened. The disease occurs, rarely, in organ transplant patients; people undergoing chronic corticosteroid or immunosuppressive therapy; and individuals with cancer, such as Hodgkin’s disease, lymphoma, and sarcoidosis.  PML is most common among individuals with acquired immune deficiency syndrome (AIDS).

Date Modified: 2011-04-01T13:43:00

basic Questions

What is the prognosis for individuals diagnosed with progressive multifocal leukoencephalopathy (PML)?

The mortality rates for those with HIV-PML have fallen dramatically from approximately 90 percent to around 50 percent according to most reports. For non-AIDS individuals with PML, the prognosis remains grim; the disease usually lasts for months and 80 percent die within the first 6 months, although spontaneous improvement has been reported.  Those who survive PML can be left with severe neurological disabilities.

Date Modified: 2011-03-03T22:36:00

How might progressive multifocal leukoencephalopathy (PML) be treated?

Currently, the best available therapy is reversal of the immune-deficient state. This can sometimes be accomplished by alteration of chemotherapy or immunosuppression. In the case of HIV-associated PML, immediately beginning anti-retroviral therapy will benefit most individuals.

Date Modified: 2011-03-03T22:36:00