Definition of Transmissible spongiform encephalopathy

Transmissible spongiform encephalopathy: One of a number of progressive neurodegenerative disorders in animals and humans caused by similar uncharacterized agents that produce spongiform changes in the brain.

Specific examples of transmissible spongiform encephalopathies include:

• In animals:
  • scrapie, which affects sheep and goats;
  • bovine spongiform encephalopathy (BSE), also called mad cow disease, which affects cattle;
  • transmissible mink encephalopathy;
  • feline spongiform encephalopathy;
  • chronic wasting disease (CWD) of mule deer, white-tailed deer, black-tailed deer, and elk.
• In humans:
  • kuru;
  • Creutzfeldt-Jakob disease;
  • Gerstmann-Straussler syndrome;
  • fatal familial insomnia;
  • variant Creutzfeldt-Jakob disease (vCJD).

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