- The disease is caused by a mysterious class of proteins called prions that destroy nervous system tissue in the brain, causing loss of motor control and eventually death.
- The means of transmission is of particular concern because the protein nature of prions makes them extremely resistant to conventional means of disinfection and sterilization.
- The causative prions are resistant to steam sterilization, dry heat, ethylene oxide gas, and chemical disinfection with either formaldehyde or glutaraldehyde, as normally used in the health care environment.
- For many years after their discovery as the agents of some rare neurodegenerative diseases in mammals, such as scrapie in sheep and human Creutzfeldt-Jakob disease and kuru, prions have remained a truly esoteric research topic.
- Twisted forms of brain proteins called prions spread the disease, making normal proteins misfold.