transmissible spongiform encephalopathy

Term used for a group of human and animal diseases characterized by the development of spongy changes in the brain. All fatal, they can be passed to other individuals in contaminated tissue. They are caused by prions, and about 10% of the human diseases seem to be inherited. The group includes: bovine spongiform encephalopathy, scrapie in sheep, and Creutzfeldt-Jakob disease and kuru in humans.

Other human transmissible spongiform encephalopathies include Gerstmann-Sträussler-Scheinker disease (loss of coordination often followed by dementia) and fatal familial insomnia (insomnia and dementia).