New answers have been found after researchers investigate a rare and incurable brain disease that unknowingly affects 3 out of every 100,000 people over the age of 50 years old. In fact, due to a lack of proper understanding of the disease, it has been deemed as near undetectable until the autopsy report confirms it.
However, new studies have found the possible blame for what is called Multiple System Atrophy (MSA), which is an unfortunate condition that is as dreadful as it sounds. Similar to Parkinson’s Disease, MAS causes tremors, rigid muscles, and messes with the body’s involuntary processes, such as blood pressure, digestion or bladder function.
According to the National Institutes of Health, it rapidly progresses in people between 50 and 60 years old, as their condition declines between the next 5 and 10 years with progressive loss of motor functions. The crippling and, ultimately, fatal condition affects thousands of people across the United States, and it currently stands with no cure to battle it.
It has been found that MSA is likely caused by infectious proteins called prions. Dr. Stanley Prusiner of University of California San Francisco (UCSF), who won the Nobel Prize in 1997 for discovering prions and their ability to spread, gathered a team and a number of 14 brains of patients who died of MSA.
They have found that the disease is one of the few existent disorders that are linked to malfunctioning proteins, similar to the ones causing Creutzfeldt Jakob Disease, or more commonly known as the human form of “Mad Cow Disease”. The proteins in the brain fold abnormally, and then spread the same disease-inducing dysfunction to other proteins.
According to Kurt Giles, a neurology expert part of the team of researchers at UCSF, they have “conclusively” proven that a new type of prion is the cause of MSA, but warn that while there are similarities to Mad Cow Disease, it may not necessarily spread the same way.
Mark Zabel, an associate director of a Prion Research Center who was not involved in the study, has stated that the disease is indeed transmissible, but unlikely to cause an epidemic. The most common practice through which it can spread would be due to improperly decontaminated utensils in the operating room.
Thus, it cannot cause an epidemic, but it can be transmitted from one patient to the other through tools often used during brain surgery or therapies. The harmful prions have a strong ability to cling hard to stainless steel wires, which might led to contamination as it’s impossible to clean or kill a protein, according to Giles.
However, Dr. Valeries Sim, an associate professor in the Neurology Division at a University in Alberta, stated that there is so far no proof of human-to-human transmission, and people should not become afraid of contracting MSA just yet.
The research has only proven its ability to remain on tools within the laboratory, and the rat subjects were infected through direct injection into the brain.
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