Bovine Spongiform Encephalopathy (BSE) is a fatal neurological disease of cattle. It is caused by a prion, an infectious agent that is resistant to inactivation by conventional sterilization procedures. BSE was first recognized in the United Kingdom in 1986, and since then has spread to other countries, including the United States. More than 200 cases have been diagnosed in cattle worldwide, with the majority occurring in the UK.
BSE affects adult cattle, causing them to become lethargic and lose weight. Ultimately, the animals become paralyzed and die. There is no treatment for BSE once it has begun to develop. The incubation period for BSE can be up to eight years or longer, meaning that infected animals may not show signs of disease until long after they have been exposed to the prion.
There is evidence that BSE can be transmitted to humans who consume infected beef products. This transmission results in a human form of dementia known as Variant Creutzfeldt-Jakob Disease (vCJD). To date, there have been more than 200 confirmed cases of vCJD worldwide, with most occurring in the UK and France. While the risk of contracting vCJD from eating beef products contaminated with BSE prions is considered low, it is nonetheless important for consumers to be aware of this potential hazard when making decisions about what foods to eat.