The Bizarre Story of Prion Disease

Catalog description

Prions are the world's deadliest infectious agents with a mortality of 100%. They are caused by rogue proteins produced by the victim's own body. These proteins begin to crystallize destroying brain tissue. Prions may be acquired by exposure to someone else with the disease. One tragic example was the early use of human derived growth hormone to treat short stature. Another bizarre example is transmission due to cannibalism in the case of Kuru on the island country of Papua New Guinea. Prions can also be acquired by exposure to infected animals as in the case of Mad Cow Disease. They can be genetically inherited as the case of Fatal Familial Insomnia. Or they can arise spontaneously as in the case of Creutzfeldt Jakob disease. Despite the bizarre nature of these proteins, they can provide key insights into many other neurological and nonneurological diseases as diverse as Alzheimer's disease and sickle cell anemia. Two Nobel Prizes have been awarded to researchers for their work on prions. This class will take a deep dive into the history, epidemiology, clinical features, pathology, patients, politics, prevention, and researchers of prion disease. I will also share some of his personal stories related to prions both here at Stanford and in distant corners of the globe.