Incidence of this disease has declined dramatically with the demise of ritualistic cannibalism. Characterized by progressive dementia, incoordination, loss of body functions, and death. Similar to CJD, but is a distinct hereditary disease. The disease progresses very slowly. There is a definite genetic component for susceptibility to scrapie.
Transmission is from dam to offspring and closely associated susceptible lambs.
The epidemiology and source of this disease is problematical and controversial. At the present time, it appears to affect substantial numbers of wild deer and elk in northeastern Colorado and southeastern Wyoming. The health implications for hunters who consume affected elk and deer or for transmission to livestock are unknown. Certainly no animals that show clinical signs of severe weight loss or other symptoms of disease should be consumed by humans or livestock. BSE was first diagnosed in the United Kingdom in October, 1986.