Knowhow-Now Article

Progressive multifocal leukoencephalopathy is a rare, usually fatal demyelinating disease of the central nervous system. It is caused by the JC virus which is present in more than 80% of healthy adults. The virus is harmless unless a weakened immune system allows it to activate.

Characteristics of the disease

PML is a deadly disease but fortunately it only appears among individuals with compromised immune system. 82% of the affected patients are infected with HIV. But other groups of patients can also experience the disorder. People receiving strong immunosuppressive agents, like those suffering from multiple sclerosis, rheumatologic diseases, leukemia and other types of cancer, or who underwent an organ transplant can also develop the disorder.

The virus is present in latent state in the kidney or the gastrointestinal tract. If a person’s immune system fails the virus activates, enters the blood stream and eventually reaches the brain. It starts destroying the white matter of the brain, which covers and protects nerve cells, allowing them to communicate with the rest of the body. If that communication is interrupted the organs will start to fail.

Affected patients don’t even realize the dangers they are facing. The symptoms of progressive multifocal leukoencephalopathy are mild in the first stages of the disease, and they can easily be confused with signs of other medical problems. They also vary considerably from one patient to another, depending on which areas of the brain have been affected. The first symptoms include headaches, clumsiness, weakness of the arms and legs and vision problems. By the time the symptoms develop to memory loss, loss of cognitive functions, speech impairments and paralysis it will already be too late to treat the disease.

Treatment possibilities

The first step in treating the problem is to get a correct and early diagnosis. That can be done with the help of an MRI which will show the lesions of the white matter. The presence of JC virus in the spinal cord fluid or blood can also be established through medical tests.

Unfortunately diagnosing the disease might not be enough, considering that there is no effective treatment against PML. Reactivating a patient’s immune system is the best way to fight the disease. Highly effective antiretroviral therapy is able to help AIDS patients, increasing their survival rate up to 50%. However these patients will still have to face serious neurological disabilities after the treatment, because medication cannot regenerate the damages done to the brain.

So the best way of fighting PML is to be informed and alert. If a patient who could be at risk notices any suspicious signs he is advised to contact a doctor immediately in order to increase his survival chances and to limit the damages to the brain as much as possible.

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