Progressive Multifocal Leukoencephalopathy

November 12, 2012

Progressive Multifocal Leukoencephalopathy

What is Progressive Multifocal Leukoencephalopathy?

The white matter within the brain contains some cells that make a substance known as myelin. Myelin is essential for insulating the nerve cells. Progressive Multifocal Leukoencephalopathy is a disease that targets these myelin-producing cells and can therefore be defined as a disease of the white matter of the brain. There is a virus known as JC virus, which is present in most people and remains harmless unless someone has lowered immune defences. Individuals being treated for some forms of cancer or those suffering form autoimmune conditions including multiple sclerosis and rheumatoid arthritis are at a high risk or JV virus becoming activated, which in turn increases the risk of developing progressive Multifocal Leukoencephalopathy, PML for short. Those who most commonly suffer form PML include those HIV/AIDS. As many as 5% of those infected with HIV will as some later stage go on to develop PML. However the treatment that is currently being used to fight aids, known as antiretroviral drugs target the immune system function and is it currently thought that thanks to this drug over half of these cases survive.

Symptoms of Progressive Multifocal Leukoencephalopathy

The symptoms of this disease vary greatly and depend primarily on the area and extent of the damage to the brain. Symptoms may get progressively worse over the following weeks or months. Some of the more common symptoms include clumsiness, weakness and changes to the speech, vision and sometimes personality. As these issues continue to get worse, the individual may suffer from life-threatening disability and sometimes death. In order to diagnose progressive multifocal leukoencephalopathy, a brain biopsy may be required. Alternatively in some cases a diagnosis can be made through observation of the progression of symptoms and behaviour along with an MRI scan or presence of the JC virus.

Treatments for Progressive Multifocal Leukoencephalopathy

Treatment is difficult but the most effective is currently believed to be treatment aimed at the reversal of the immune-deficient state. Plasma exchange can be used to reduce the quantities of agents that put the individual at risk of the disease. If the individual has developed PML linked to HIV then the most effective treatment will probably be anti-retroviral therapy. Further studies and research continue to be done looking into treatments and prevention of this disease. Currently the mortality rate is between 30-50% in the first few months following diagnosis.


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