: 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

In regards to your follow-up question:

I personally wouldn’t call it the most dangerous diseases – but it obviously depends fully on how you define dangerous. If you look solely at the fatality rate, it’s definitely up there though. If you factor in how many people are affected yearly, then it’s quite far down the list.

Pros for the title of most dangerous disease:
– fatality rate
– no cure, and no treatment to slow it down
– can occur sporatic, ie. this type can’t be avoided. You’re just unlucky.

Cons:
– very slow progression compared to other fatal diseases
– very rare

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