Scrapie

scrapie, also called rida or tremblante du mouton, fatal neurodegenerative disease of sheep and goats. Scrapie has been endemic in British sheep, particularly the Suffolk breed, since the early 18th century. Since that time the disease has been detected in countries worldwide, with the exception of Australia and New Zealand, as well as in other breeds of sheep.

Scrapie is one of a group of diseases classified as spongiform encephalopathies, so named because the resultant deterioration of neurons causes a spongelike pattern to develop in the brain tissue. The agent responsible for those diseases is an abnormal prion, a deviant form of a benign protein normally found in the brain. In susceptible animals, the modified prion protein is thought to be able to convert the normal protein molecule into its own shape, thereby replicating itself within neurons, damaging them and causing the characteristic neurodegeneration.

Scrapie has a long incubation time, typically between about 18 months and five years following transmission. The first signs to arise are usually behavioral changes such as general apprehensiveness and nervousness. As the disease progresses, the animal loses weight and weakens, develops head and neck tremors, loses muscular coordination, and begins to rub or scrape its body against objects, wearing away its fleece or hair—hence the name “scrapie”. The disease inevitably causes death within one to six months. No treatment or palliative measures are known.

The scrapie-causing prion can be spread from sheep to sheep. The primary route of transmission is through the ingestion of placenta or allantoic fluids from an infected female. Hence, newborns are at high risk of infection. There also is evidence that the prion can persist in soil and infect healthy animals that ingest contaminated soil particles. Some sheep carry genetic variants that render the normal prion protein resistant to conversion into the abnormal form that causes scrapie. Scrapie does not appear to be transmissible to humans.