Highly Efficient Transmission of Prions by Transfusions

Bovine spongiform encephalopathy, commonly known as “mad cow diseaseâ€?, is a fatal neurodegenerative disease in cattle, with a long period of incubation that causes a spongy degeneration in the brain and spinal cord. The disease may be transmitted to human beings by eating food contaminated with the brain, spinal cord, or digestive tract of infected carcasses. In 1996, a new variant of the Creutzfeldt-Jakob disease (vCJD or nvCJD) that causes an incurable and fatal degenerative neurological disorder was identified in humans. Prions are the infectious agents for the transmissible spongiform encephalopathies (TSE) and vCJD disease.