: Why do prion diseases have 100% fatality rate ?

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I recently found out about the so-called prion diseases, which are incurable and fatal diseases that affect (a euphemism, the real word is destroy) the CNS and cause a rapid deterioration of mental and physical abilities.

There are many prion diseases, the two most famous are probably the mad cow disease (non-human mammals), and the Creutzfeldt–Jakob disease (CJD) (for human mammals). Both are 100% fatal, and *no one* is known to have survived longer than 2.5 years after a CJD diagnosis. That’s the kind of stuff you wouldn’t wish on your worst enemy.

Why are these diseases so deadly? I read that it has to do with abnormal proteins but that was way over my head.

EDIT : I have another question, can prion diseases be rightly called the most dangerous diseases known to man ?

Thanks;

In: Biology

18 Answers

Anonymous 0 Comments

There are a lot of correct answers about the prion diseases but it’s not the real answer to your question.

The real answer to your question is that we don’t have early diagnosis for prion diseases. We simply don’t know if there’s early stage or weak prion diseases that are somehow contained by the body or even reversed, because we don’t know them happening.

So the answer why people live only short post-diagnosis is that because we have only late diagnosis at the moment. And the reason for that is basically it’s a painless disease (no pain receptors in brain), and a little brain damage is usually shadowed by the brain plasticity (so an early damage is not seen because other brain parts can take over functions). On top of that, there’s no molecular diagnosis, no inflammation no any sign of an early and incomplete prion disease.

And that’s why we only see a prion disease when it’s severe enough to be deadly soon.

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