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Mysterious And Incurable Brain Disease Now Linked To Mad Cow

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Scientists from the University of California San Francisco (UCSF) identified a type of misfolded brain protein known as prion as the potential cause of a rare and incurable brain disorder with strong similarities to Parkinson's disease.

The debilitating disease, called multiple system atrophy (MSA), is known to affect three in every 100,000 individuals beyond 50 years old.

According to researchers, the rare disease is likely triggered by infectious proteins that share similarities to the prions that cause the human equivalent of mad cow disease called Creutzfeld-Jakob disease.

The National Institutes of Health (NIH) said that people with MSA typically show symptoms when they reach their 50s. This is marked by a rapid decline in health as well the progressive loss of the sufferer's motor function within the next five to 10 years.

In a study featured in the journal Proceedings of National Academy of Sciences, UCSF researcher Dr. Stanley Prusiner and his colleagues discovered that multiple system atrophy is likely caused by misfolded brain proteins known as prions.

Prions are considered to be highly destructive as they can cause other proteins to fold abnormally, resulting in devastating consequences to the brain of an individual.

Prusiner and his team studied the brain data collected from 14 individuals diagnosed with multiple system atrophy.

They made of brain specimens gathered from the participants to infect laboratory mice and other healthy cells with the life-threatening disease. The prion that they identified, called alpha-aynuclein, is considered to be the first of its kind to be discovered in the last 50 years.

Prion research expert Mark Zabel, of Colorado State University, pointed out that while MSA is transmissible, the disease itself cannot trigger an epidemic.

He explained that the way they transmit MSA in laboratory animals is by injecting them with a disease-laden needle. This does not occur too often in wildlife or in human life.

Zabel, who was not involved in the UCSF study, added that clinics and operating rooms are the most likely sources of MSA infection.

Despite subjects developing symptoms of MSA spontaneously, Prusiner and his colleagues caution doctors regarding the application of deep brain stimulation as this could cause others to be infected by the disease.

The researchers note that people suffering from multiple system atrophy are often mistaken for patients with Parkinson's disease. They believe that if these patients are provided with deep brain stimulation treatment, they could place other individuals at risk if the tools used in the surgery are not thoroughly decontaminated.

Photo: NIAID | Flickr 

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