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Comment by adventured | Hacker News Reader

The article mentions 65 CJD deaths per year in Canada. I was surprised it was that high, I had thought CJD was far more rare than that (an order of magnitude more rare). It more or less matches the present US figures however, at the typical 10-1 ratio between the two nations. So the near doubling of CJD deaths in the US from 2006 to 2019 (from 290 to 560), would that likely imply a lot of CJD infections ocurred many decades ago (1970s-1990s?) and they're now manifesting in older persons (resulting of course in a rapid death)?

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An interesting video about human prion diseases that, among other things, discusses epidemiology ("1 in 7000 is, actually, a lifetime risk" [in the U.S.]):

"Creutzfeldt-Jakob Disease and Other Prion Diseases - Brian Appleby, M.D."

https://www.youtube.com/watch?v=4vyuby6gibs&t=328s

Also, some links from my older comment to "Oregon has legalized human composting" article:

https://news.ycombinator.com/item?id=27555105

"Grass plants bind, retain, uptake and transport infectious prions"

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4449294/

"Texas CWD Symposium: Pre-symptomatic prion detection"

https://www.youtube.com/watch?v=Q8ZX8sHAPUw

Also, a good article about potential medication for CJD:

"The rise and fall of pentosan polysulfate in prion disease" (Dec 13, 2012)

https://www.cureffi.org/2012/12/13/the-rise-and-fall-of-pent...

Also, somewhat old (2010) but interesting video about vCJD in the UK by Prof. James Ironside:

"Prof. James Ironside - Prions: The serial killers that attack the brain"

https://www.youtube.com/watch?v=nlIYGYA5q0s?t=2486s

At 41:26:

"...and we looked at thousands of appendices... and we looked at thousands of them [appendices] and of these 12,500 we find that 3 were positive... subsequent studies on tonsils come out with broadly similar figures so our best guess is that that currently [2010] there may be 1 in 10,000 or around 4 in 10,000 are infected with Variant CJD in the UK..."

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Variant Creutzfeldt–Jakob disease (vCJD), commonly referred to as "mad cow disease"... It is caused by prions, which are misfolded proteins. Spread is believed to be primarily due to eating bovine spongiform encephalopathy (BSE)-infected beef. -- https://en.wikipedia.org/wiki/VCJD

Most CJD is not caused by ingesting meat, but rather are spontaneous. You can develop CJD spontaneously.

Sporadic CJD is diagnosis of exclusion. That it happens spontaneously is a hypothesis, there might be root cause(s) that we simply don't know yet.

citation ?

The article we're discussing mentions that.

Without citation, sure. The citations here are great, thanks.

https://en.wikipedia.org/wiki/Creutzfeldt%E2%80%93Jakob_dise...

Classification section, quote:

Sporadic (sCJD), caused by the spontaneous misfolding of prion-protein in an individual.[20] This accounts for 85% of cases of CJD.[56] Familial (fCJD), caused by an inherited mutation in the prion-protein gene.[55] This accounts for the majority of the other 15% of cases of CJD.[56] Acquired CJD, caused by contamination with tissue from an infected person, usually as the result of a medical procedure (iatrogenic CJD). Medical procedures that are associated with the spread of this form of CJD include blood transfusion from the infected person, use of human-derived pituitary growth hormones, gonadotropin hormone therapy, and corneal and meningeal transplants.[55][56][57] Variant Creutzfeldt–Jakob disease (vCJD) is a type of acquired CJD potentially acquired from bovine spongiform encephalopathy or caused by consuming food contaminated with prions.[55][58]

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