Progressive multifocal leukoencephalopathy (PML) is a disease that affects the brain's white matter. Its cause is a virus infection that targets nerve cells (neurons) insulating material, myelin. A majority of people are affected by Polyomavirus JC (also referred to as JC virus). It is, however, harmless apart from the few who have low immune defenses.
It is a rare disease and only affects patients who go through chronic corticosteroid or, otherwise, immunosuppressive therapy for transplantation of organs, or those suffering from cancer (Hodgkin’s disease, lymphoma, etc.). Persons who suffer from autoimmune conditions like multiple sclerosis, rheumatoid arthritis, and systemic lupus erythematosis -- few of which are treated with biological therapies -- also run a risk for PML. Individuals suffering from HIV-1 infection / acquired immune deficiency syndrome (AIDS) have a higher chance of being subject to PML.
According to research before effective antiretroviral therapy, about 5 percent of those infected with HIV-1 in due course develop the AIDS-defining illness, PML. However, as many as half of all HIV-PML patients survive due to the existent HIV therapy which makes use of antiretroviral drugs (ART), which has the ability to restore immune system function effectively. These survivors may, sometimes, experience inflammation in the regions of the brain which were affected by PML.
There are diverse symptoms to PML as they are directly related to the location of the disease and the amount of damage it has caused. Clumsiness; progressive weakness; and visual, speech, and sometimes personality changes are some of the prominent symptoms.
Presently, reversal of the immune-deficient state is the best therapy possible for Progressive multifocal leukoencephalopathy. There are no drugs available that effectively block the virus infection without giving way to toxicity. Plasma exchange Can be used to speed up the elimination of the therapeutic agents that put patients at risk for PML in order to facilitate reversal. As far as HIV-associated PML is concerned, anti-retroviral therapy should be effected immediately.
In addition, laboratory tests have found several drugs effective against the infection to cure PML patients. special permission needs to be procured from the U.S. Food and Drug Administration for use of these drugs. Another option that is currently under research as a treatment option for JVC is Hexadecyloxypropyl-Cidofovir (CMX001). It suppresses JVC by restraining replication of viral DNA.
Image courtesy: Getty Images
Read more on Progressive Multifocal Leukoencephalopathy Treatment.