Mad Cow (BSE) Bovine Spongiform Encephalopathy
Encephalopathies Information Page
Transmissible spongiform
encephalopathies (TSEs), also known as prion diseases, are a group of rare degenerative brain disorders characterized by tiny holes that give the grain a “spongy” appearance. These holes can be seen when brain tissue is viewed under a microscope.
Creutzfeldt-Jakob disease (CJD) is the most well-known of the human TSEs. It is a rare type of dementia that affects about one in every one million people each year. Other human TSEs include kuru, fata familial insomnia (FFI), and the Gerstmann-Straussler-Scheinker (GSS disease. Kuru was identified in people of an isolated tribe of Papua New Guinea and has now almost disappeared. FFI and GSS are extremely rare hereditary diseases, found in just a few families around the world. A new type of CJD, called variant CJD (vCJD), was first described in 1996 and has been found in Great Britain and several other European countries. The initial symptoms of cCJD are different from those of classic CJD and the disorder typically occurs in younger patients. Research suggests that vCJD may have resulted from human consumption of beef from cattle with a TSE disease called bovine spongiform encephalopathy (BSE), also known as “mad cow disease.”
