Progressive multifocal leukoencephalopathy (PML) is a disease of the white matter of the brain caused by a Polyomavirus JC virus infection, which targets cells that make myelin.
Polyomavirus JC is carried by a majority of people and is harmless except among those with lowered immune defenses.
The disease is rare and occurs in patients undergoing chronic corticosteroid or immunosuppressive therapy for organ transplant, or individuals with cancer. Individuals with autoimmune conditions, such as multiple sclerosis, rheumatoid arthritis, and systemic lupus erythematosis — some of whom are treated with biological therapies that allow JC virus reactivation — are at risk for PML as well.
PML is most common among individuals with HIV-1 infection/AIDS. Current HIV therapy using antiretroviral drugs, which effectively restores immune system function, allows as many as half of all HIV-PML patients to survive, although they may sometimes have an inflammatory reaction in the regions of the brain affected by PML.
The symptoms of PML are diverse, since they are related to the location and amount of damage in the brain, and may evolve over the course of several weeks to months. The most prominent symptoms are clumsiness; progressive weakness; and visual, speech, and sometimes personality changes. The progression of deficits leads to life-threatening disability and (frequently) death. A diagnosis of PML can be made following brain biopsy or by combining observations of a progressive course of the disease, consistent white matter lesions visible on a magnetic resonance imaging (MRI) scan, and the detection of the JC virus in spinal fluid.
In general, PML has a mortality rate of 30-50% in the first few months following diagnosis, but depends on the severity of the underlying disease and treatment received. Those who survive PML can be left with severe neurological disabilities.
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Adapted from Progressive multifocal leukoencephalopathy information page. National Institute of Neurological Disorders and Stroke. Internet. Accessed on October 5, 2017.