Prion Diseases

Prion diseases are a group of rare, fatal brain diseases that can affect both humans and animals. They are caused by an abnormal form of a naturally occurring protein, called prion protein, which accumulates in the brain, leading to brain damage and eventually death. Prion diseases are caused by genetic mutations or the transmission of prions from one organism to another. The most common human prion disease is Creutzfeldt-Jakob disease. It typically affects adults and is caused by genetic mutations or by exposure to tissues or organs from an infected person or animal. Symptoms typically appear gradually and include memory loss, behavioral changes, vision problems, difficulties speaking, and difficulty walking. As the disease progresses, it can cause dementia, loss of muscle coordination, and often death within a year of diagnosis. Other types of prion diseases can affect animals, such as scrapie in sheep and goats, bovine spongiform encephalopathy (commonly called “mad cow disease”) in cattle, and chronic wasting disease in deer and elk. These diseases are not contagious to humans, but they can present a risk if a person consumes meat from an infected animal. To help reduce the risk of transmitting a prion disease, the World Health Organization recommends that people avoid consuming meat from an animal suspected of having the disease, as well as the use of glutamate treatment for all animal products. Additionally, regular testing and surveillance of animals in high-risk areas is needed to help reduce the spread of the disease. No effective treatments for prion diseases exist, but research is underway to better understand the disease, its transmission, and how it can be prevented. By understanding prion diseases better, researchers hope to one day find a treatment and a way to reduce the risk of transmission.