Science broadcaster calls chronic wasting disease “a slow-motion epidemic”

MEDICAL MYSTERIES Jay Ingram reprises popular lecture tour on prion diseases, 
which include CWD, BSE and scrapie, as well as Creutzfeldt-Jakob disease

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Albertans will get a chance to learn more about the “fascinating” world of prions when well-known author and broadcaster Jay Ingram hits the road for a second lecture tour on prion diseases.

“Last time we went to Medicine Hat, Grand Prairie and Red Deer and there were good turnouts, and very interested audiences with good questions,” said Kevin Keough, executive director of the Alberta Prion Research Institute, which is sponsoring the three-city speaking tour along with Alberta Innovates Bio Solutions.

Prion diseases, which are caused by “misfolded” protein molecules, are a source of fascination for scientists and a scourge of livestock producers. Among the prion diseases are bovine spongiform encephalopathy (BSE), chronic wasting disease (CWD) and scrapie, as well as Creutzfeldt-Jakob disease (CJD) in humans. All are fatal.

“The thing about prions is that they’re interesting on many different levels,” said Ingram, former host of Discovery Channel’s science program “Daily Planet.”

“They were fascinating biologically because they seem to be a simple protein molecule that could actually reproduce, and that was sort of unheard of. And the fact that while mad cow disease is fair to say is largely under control, you have CWD in Alberta and Saskatchewan and in 18 states, and that’s definitely not in control.”

First noted in the 1920s, prion diseases gained attention in the 1950s when Kuru disease was discovered in a tribe in Papua New Guinea that practised ritualistic cannibalism to honour their dead.

“Sadly, if you’re going eat somebody’s brain that has a prion disease in it, you are doing the absolute worst thing you could possibly do,” said Ingram, whose latest book, Fatal Flaws, tells the story of prion diseases, clinically known as transmissible spongiform encephalopathies.

“Kuru is incredibly interesting, partially because it triggered interest in the wider medical community around the world, and it actually led to the understanding that CJD and scrapie were all in the same family.”

More attention needed

While the BSE crisis has faded, Ingram warned that CWD among cervids is not getting the attention it deserves.

“It is an epidemic, but because it is so slow running I don’t think people can get their heads around the fact that this is spreading out of control,” said Ingram.

“In 1967, it starts in a deer enclosure in Colorado and now there’s 18 states and two provinces? Sure, the time frame is decades, but the incubation period of these diseases is incredibly long, and maybe this is just a slow-motion epidemic.”

Although prion diseases are very rare in both humans and wildlife, they may have connections to some very significant human diseases such as Alzheimer’s, Parkinson’s, ALS, juvenile diabetes and chronic traumatic encephalopathy.

“These are all chronic neurodegenerative diseases, but they all are linked to the prion diseases because they all feature the accumulation in the brain of misfolded proteins,” said Ingram. “So as the issue of BSE declines in apparent importance, the possibility of clues to these hugely important human neurodegenerative diseases has become much more important.”

Ingram will speak at Lethbridge College on November 13, at Lakeland College, Vermilion Campus on November 14, and at Olds College on November 15. His “Prion Diaries” presentations will be followed by question-and-answer sessions with two researchers from the Alberta Prion Research Institute (see www.prioninstitute.ca for more information).

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