PRIONS

Infectious proteins 

Prions are “infectious” misfolded proteins in the CNS. Whenever a prion touches a healthy protein, it misfolds it. Before long, the whole brain turns into a goop of misfolded protein. The histological pattern of damage is referred to as spongiform, aka there are lots of little holes. 

Which proteins can become prions?

As far as I can tell, the only protein in the human body that can spontaneously become a prion is the aptly named “prion protein” (PrP). We don’t know what role PrP plays in the brain. PrPC is the normal form. PrPSC is the diseased form. 

How do you “catch” a prion?

There are four ways to contract a prion. 

1. Creutzfeldt-Jakob Disease - this is a sporadic genetic mutation. Bad luck really.

2. Mad Cow Disease - you contract this by eating the brains of a cow who had prions. When ground beef is made, sometimes bits of brain tissue gets into the grinder. Unlike bacteria, prions can’t be killed with heat, so the burger doesn’t have to necessarily be raw to catch this.

3. Kuru - you can contract this by eating the brains of a human. That’s right folks, cannibalism. 

4. Iatrogenic - neurosurgeons can contract prions from their own surgical instruments after operating on an “infected” brain. Prions are resistant to autoclaves. 

Symptoms?

Rapid onset dementia, as well as myoclonus. This is the only cause of rapid onset dementia that I can find. The patient will often be in their 30’s or 40’s. There is no treatment. Most patients die within 1 year.