Minor Head Injury and Prion Disease

[Wise Young]

As some people here know, prion disease causes progressive brain degeneration. Discovered by Stan Prusiner in the 1980's, prions are proteins that can become abnormal and propagate themselves, causing degeneration of neurons. Examples of prion disease include bovine spongiform encephalopathy (BSE or mad cow's disease), Creutzfeldt-Jakob disease (CJD, a disease that is sometimes called Kuru and can pass from infectious brains that are handled or eaten by cannibals or neurosurgeons).

Stan Prusiner recently gave a Friday Evening Lecture at the Marine Biological Laboratory in Woods Hole (Source), where he presented strong evidence that taopathies are essentially prion disease. Tao is a kind of protein that accumulate in the brain cells of many neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, dementia pugilistica (punch-drunk boxers), frontotemporal neurodegenerative disease that is frequently associated with minor head injuries caused by IED blasts in Iraq and Afghanistan.

It was quite a lecture. Prusiner pulled no punches with the audience, which included many young scientists and older white-haired scientists and their spouses, as well as members of the Woods Hole Community in Cape Cod. He pointed out that the incidence of neurodegenerative disease increases as one gets older and that 1 in 5 people over the age 65 have dementia and the number approaches 50% in people over 85 (I am quoting approximate numbers from memory).

He then showed a picture of George Balanchine, probably one of the most famed and celebrated ballet choreographer of the Twentieth Century. The audience gasped shen Prusiner pointed out that he recently analyzed Balanchine's brain and found evidence of prion disease. As described in the following article http://ind.ucsf.edu/truestories/individual/cjd

Then, in the fall of 1982, he was stricken with a mysterious illness. The first symptoms were relatively mild: blurred vision, bouts of dizziness, periods of sudden anger. In a matter of weeks his balance became so poor that he needed to grasp the curtain to avoid falling as he bowed. By winter Balanchine had lost the powers of sight and speech. In a period of weeks the great dance maker had become a husk of his former self.

Prusiner went on to suggest that frontotemporal dementia (FTD), a common condition that occurs after brain concussions such as those suffered by soldiers in blast injuries, is a prion disease. He showed graph after graph, as well as clinical cases, of people who developed FTD after repeated mild head injuries, how this led to symptoms similar to post-traumatic stress syndrome, drug use and addiction, emotional lability, depression, and progressive loss of intellectual and motor function.

Stan Prusiner received the Nobel Prize and the National Medal of Science for his seminal work on prion disease. I have known him for a long time and have not seen him arguing so passionately for more research on this subject. He pointed out that dementia affects hundreds of millions of people in the United States and around the world, that the annual cost of caring for these people will reach $600 billion by 2020 and yet we are spending less than $400 million on research to prevent, stop, or reverse this condition.

Wise.

[zagam]

Prions are interesting. Like a virus with no DNA or RNA. Though the goal of research may be know more about very serious debilitating and costly disease, but pure science will also add to our knowledge.

They are used to fairly alien sulphur eating stuff at the bottom of the ocean so the right place to talk about prions. What about other places in this universe?

I have often wondered how early life could involve nucleic acids and cells which is the chicken-egg problem. Did metabolism and something else involving amino-acids come first?

Though only known as a pure parasite, prions are non-nucleic acid life.

[Sue Pendleton]

I'm a member of a rather large group of folks turned away from volunteering to donate blood because we lived in western Europe for too long in the 1980s and 1990s. What gives? I understood that prion disease showed up within 9 years of exposure as far as mad cow and CJ disease goes. Just recently another friend was turned down solely for living in Europe from 1984 to 1989 with a short visit back in 1990.

As far as the Iraq 1991 soldiers that makes sense because several experimental meds and insecticides were given to many. I know one guy with no history of cancers of any kind in his family and has had two unrelated cancers show up soon after his time in again, he was prior service and went back in during Desert Storm, that he was only vaguely warned about. And Vaccine-A is a very scarey anti-anthrax vaccine that has been pretty much proved to cause a lot of auto-immune diseases during OIF and OEF.