Scientists believe they have discovered the cause of a rare and mysterious disease in the brain that leaves patients with manifestations similar to Parkinson's disease. This incurable and fatal disease is caused by an abnormally folded brain protein called prion that is also linked to mad cow disease in a report released on Monday.
The disease called multiple system atrophy or MSA affects around 15,000 to 50,000 people in the United States and it can cause devastating neurological damage to victims. Researchers are now recommending anyone doing brain surgery to be more careful. This protein is also the cause of Creutzfeld-Jakob Disease, the human form of mad cow disease.
According to ABC News, the people infected with the disease would manifest the symptoms when they reach their 50s and their health would then decline rapidly in the next five to 10 years.
"People with MSA often develop pneumonia in the later stages of the disease and may suddenly die from cardiac or respiratory issues," the National Institute for Neurological Disorders and Stroke, part of the National Institutes of Health, said in a statement.
The study was published in the Proceedings of National Academy of Sciences and the scientists pointed out that these prions are most likely the cause of MSA. These proteins are abnormally folded and can create a domino effect to other proteins. If all proteins have folded, these can cause serious consequences in both motor and neurological function.
The study was led by Dr. Stanley Prusiner, director of the Institute for Neurodegenerative Diseases at University of California San Francisco. He and his colleagues studied the brains of 14 people who had died because of MSA. They discovered that their brains had an abnormal buildup of a protein called alpha synuclein.
They used the specimen from the brains and tested it on mice. They found out that mice got infected and the other cells in the brain also acquired the disease. According to the scientists, this discovery of alpha-synuclein is the first in 50 years.
"Now we've conclusively shown that a new type of prion causes MSA," said Kurt Giles, a neurology expert at UCSF who is part of the study told NBC News.
