- Transmissible spongiform encephalopathies (TSEs) are a family of diseases of humans and animals characterized by spongy degeneration of the brain with severe and fatal neurological signs and symptoms
- Transmissible spongiform encephalopathies (TSEs) are a class of fatal neurodegenerative diseases caused by an infectious agent which has to date been identified as an abnormal proteinaceous particle termed a “prion.” TSEs can be hereditary, occur spontaneously, or can be transmitted through direct inoculation of infectious tissue.
- TSEs have the characteristic symptoms of progressive neurodegeneration that include lack of muscular coordination, tremors, increasing mental confusion, and progressive muscular weakness that ultimately overcomes the entire body. The disease is always fatal, typically in a matter of months once the onset of symptoms appears.
- Although there is some variation in how the different TSEs manifest themselves, all result in symptoms of progressive neurological deterioration that is caused by the development of “spongy” holes in the brain, resulting in the characteristic spongiform appearance of brain tissue when examined upon autopsy.