ABSTRACT
Creutzfeldt-Jakob disease, also known as transmissible spongiform encephalopathy, is characterized by a fairly rapidly progressive dementia accompanied, in most cases, by myoclonus. In the past, this disease was also termed a ‘slow virus’ infection; however, it is now known that it is caused not by a virus but by a unique agent known as a prion. This is a rare disorder, occurring at a yearly rate of 1-2 cases per 1 000 000, and is equally common in males and females. About 85 percent of cases occur on a sporadic basis, 10 percent are inherited on an autosomal dominant basis, and the remainder represent iatrogenic infections, as has occurred with dura mater grafts for example.
