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PML (Progressive Multifocal Leukoencephalopathy)
Progressive Multifocal Leukoencephalopathy, more commonly known
as PML, is a severe brain infection that usually results in death.
It is a disease of the white matter of the brain that can develop
simultaneously in several areas. PML is often referred to as
an opportunistic infection, meaning the infection attacks only
when the body’s immune system is too weak to defend against
disease. PML is caused by the JC virus, which attacks the body
when the immune system is weakened. The JC virus is often attained
during childhood and remains in the person’s body as they
enter adulthood. Even as adults, most people with the JC virus
still do not develop PML. However, if a person’s immune
system is weakened or suppressed, the JC virus activates. This
results in PML attacking the brain and central nervous system.
There are many symptoms of PML (Progressive Multifocal Leukoencephalopathy).
Early symptoms include: weakness in extremities (hands or legs),
trouble concentrating or developing thoughts, impaired coordination.
As PML progresses, there are more serious symptoms, including:
- Impaired
Speech
- Loss of vision
- Breakdown of Muscles
- Paralysis
- Memory Failure
- Mental Declination
- Cognition Failure
- Coma
- Seizures
- Permanent disability
- Death
Diagnosing PML can be done in a few different ways. First, physicians
sometimes diagnose PML through MRIs (Magnetic Resonance Imaging).
Second, performing a spinal tap (insertion of a needle into
the spine to collect spinal fluid for examination) is another
method
by which PML can be diagnosed. The third and final way to diagnose
PML is the most invasive, a brain biopsy. Although there are
multiple ways to diagnose patients with PML, diagnosis is still
a challenging task because of how rapidly the infection progresses.
Once the brain infection starts to develop, lesions form on
the brain. This results in every patient experiencing different
symptoms
because the lesions can form on different parts of the brain.
Therefore, there is no usual or common progression of the disease.
The prognosis for this brain infection is often very serious.
According to the National Institute of Health, patients usually
suffer from PML for several months. Sadly, over 80% of PML patients
die within the first six months. For the remainder of their lives,
patients suffering from PML continue to experience irreversible
neurological problems, including loss of coordination/movement,
memory loss, and vision problems.
Most often, people suffering from AIDS develop PML because their
immune system is weakened. However, drugs that suppress the immune
system have also been linked to PML. Most notably, an anti-psoriasis
drug called Raptiva has been linked to PML. After the death of
some patients and countless others suffered from PML, Genentech,
manufacturer of Raptiva, removed the drug from the United States
market. The removal of Raptiva (Efalizumab) from the market came
mainly as a reaction to its link to a number of reported cases
of progressive multifocal leukoencephalopathy (PML) in Raptiva
users. A statement released by Genentech said they anticipated
Raptiva being unavailable in the United States by June 8, 2009.
As a result of the recall, physicians and healthcare professionals
were asked to stop prescribing Raptiva and begin alternative
treatment methods for patients already using Raptiva.
Raptiva is not the only drug linked to PML. Other drugs linked
to PML risk are Rituxan, Tysabri, Campath, and Cellcept. In September
2008, a patient with rheumatoid arthritis was prescribed Rituxan.
He later died from PML. Following his death, the manufacturer
of Rituxan (Genentech) added a warning to Rituxan labels that
PML was a possible side effect. The FDA has also indicated that
PML is linked to Tysabri, a drug used to treat multiple sclerosis.
This FDA warning followed reports of two patients developing
PML. In 2005, Tysabri was removed from the market after three
more patients developed PML. However, the drug regained approval
in 2006.
Treating this brain infection is very difficult because most
antibiotics and medications cannot successfully enter the brain.
Unfortunately, there is currently no proven treatment for PML.
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