Prions PT2: Kuru, BSE/"Mad Cow", CJD, and Chronic Wasting Disease

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Hello Poison Friends! We are back with part two of Prion Diseases or Transmissible Spongiform Encephalopathies.

Kuru is a TSE/Prion disease that has affected the native people's of Papua New Guinea during the last century or so. I don't want to spoil just how it came about, but it does involve some taboo--and thankfully no longer practiced--funerary rites. The Australians who were observing and studying them just after WWI discovered the Fore people there were dying out quickly (the women and children especially) of some strange illness that led them to shake and tremble violently, become confused, and even laugh deliriously before dying. There native peoples had long believed it to come from sorcery, but the Australians and an American physician with many a flaw to discuss here were not content with such an answer.

Creutzfeldt-Jakob Disease (CJD) was identified in patients in the early 20th century and the symptoms were similar to other diseases now known to be prion diseases: dementia, hallucinations, stumbling, falling, loss of coordination, death. This seemed to be spontaneous and that was that. Then Europe's cattle began to show signs and symptoms that were alarming. They too loss their coordination, became agitated, held their heads strangely, fell over, and died.

Cows who were ill were slaughtered and their meat and tissues made it into the food chain. In Cows, the illness was identified as Bovine Spongiform Encephalopathy (BSE), though some journalists referred to it as "mad cow" disease. While the government agencies tried to keep it a secret to avoid the meat economy from going under, humans became infected via the ingestion of BSE infected meat. This illness in humans was identified as variant Creutzfeldt-Jacob Disease and it caused chaos and panic among those who had been buying and eating beef.

America would face a similar battle with similar government agency actions and there are some (scientists included) who have begun to believe that CJD is never sporadic but likely always related in some way to infected meat. Let's see why they think as much.

Finally, we need to discuss what is happening to deer, especially here in North America. Chronic Wasting Disease showed up in Colorado in deer in the late 1900s (1970 onward) and it has since spread to elk, moose, reindeer and other such animals throughout the US and Canada. These animals are dying of a prion disease that likely jumped from sheep as Scrapie into deer as CWD. How could this have happened and what could it mean for the future?

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