Prions

A prion is a type of protein that can cause normal proteins in the nervous system (mostly brain) to fold abnormally, and can affect anything with neurons. 

How are they spread?

They can be spread by anything, as long as they get inside your body. The source of the abnormal protein is unknown in most cases of prion disease. Prion diseases are rare and only 300 cases are REPORTED in the US per year.

What types are there?

CJD: This condition can be inherited, called familial CJD and Sporadic CJD develops more suddenly. Most CJD cases are sporadic and tend to strike around age 60 (in humans). Acquired CJD is caused by exposure to infected tissue (mostly during surgeries).

Variant CJD: This is an infections type of CJD disease and is related to "mad cow disease." Eating infected meat may cause this.

VPSPr (Variably protease-sensitive prionopathy): A very rare type of prion disease and is like CJD, but the protein is less sensitive to digestion. Likely to strike people around age 70.

Gerstmann-Straussler-Scheinker disease: Occurs at around age 40.

Kuru: Mostly seen in New Guinea, caused by eating human brain tissue (ew) contaminated with infectious prions. It is now rare because of increased awareness and the fact that no one really likes to eat brains.

Fatal insomnia: Very rare hereditary disorder. Causes trouble with sleeping, but deadly.

What causes prion diseases?

Normal prion protein can be found on the surface of many cells and prion diseases occur when this protein changes and clumps in the brain.