Bovine Spongiform Encephalopathy: A Disease of the 20th Century

Spongiform Encephalopathies are a rare family of degenerative brain diseases characterized by the accumulation of plaques and formation of tiny holes in the brain tissue making it look "spongy". Spongiform Encephalopathies have a relatively short history but their origins date back to a time long before they were recognized as a disease. It was not until the 1700s that the first record of their existence was made. In 1732 a shepherd in England noticed that some sheep in his flock had become itchy and were "scraping" themselves on nearby trees and fence posts; he reported it to the agricultural authorities of the time. As the symptoms seen in his sheep progressed they also developed problems walking and began to have seizures. Eventually their neurological symptoms progressed to an unmanageable level and they died. In 1794, over 50 years later, the Board of Agriculture in the UK termed this illness in sheep "the Rubbers". In the following years while coming in and out of mention in many flocks of sheep "the Rubbers" remained a disease of minimal consequence showing negligible ability to spread among sheep and having no precedence for jumping the species barrier and affecting humans. The first mention of "the Rubbers" as Scrapie was in 1853, and it is still the designation of the disease in sheep today.