Prions are both 100% fatal and virtually indestructible. It's like some sort of glitch universal kill-switch biology happened upon encoded by hell itself.
Proving once again, that true Eldritch horrors are not out there, but right here; not in the cosmos, but in microcosm. Nature is the ancient mad god, and it's not the telescope but the microscope that will take away your sanity.
I've heard this before but the last I tried looking into it I could only find a single study with these findings. Do you know if this was ever replicated? If so, I'd be interested in reading it -- I have to wonder how careful the researchers were to isolate the raw plant material from the contaminated soil, which may have non-plant matter transmitting the prion. Plants transmitting a prion is just hard for me to believe.
It'll probably be a year or two until an attempt at replication is published.
Anyway, the details are all there. It's an open-access paper. (I'm enjoying this new trend where almost everything worth reading is published in open-access journals.)
I'd bet they aren't. Fatal once visible symptoms are present, probably. COVID had a CFR that was beyond horrible while the only cases that they were tracking were the ones that had made it to hospital. 100% CFR is certainly possible, but unless we have cheap ways of measuring prevalence it seems improbable. There could be common prion diseases that have managed to fly under the radar so far.
1) You didn't check the source for the CFR. Wikipedia links an article that doesn't mention CFR or even use the word fatal. And as recently as '08 they were discovering that what they thought was one disease is actually 2.
2) I just wrote a comment explaining why a 100% CFR would be precise but likely not accurate. In case you thought it was, take a moment to reflect that linking to a table where the CFR is 100% isn't a refutation. That number is probably precise but likely not accurate. We don't seem to be good at detecting "mild" prion diseases.
3) Even if by some miracle that table is accurate and precise ... that is one disease. If you want to bet that prions only cause one disease then you're welcome to - but I'm still willing to bet that there are other prion diseases to be discovered that are less lethal.
If the treatment successfully turns off production of endogenous prion protein, then there won’t be any there to convert, and the disease (theoretically) won’t progress, and there’s some reason to believe that it might even reverse itself to at least some extent.
Yeah that's true, but given how ubiquitous PRNP is in mammals, this could also produce some form of memory, cognitive, or other deficit.