Transmissible Spongiform Encephalopathies (TSEs) are a group of rare and progressive neurological disorders that affect humans and animals. They are caused by the presence of abnormal proteins called prions, which accumulate and cause damage to the brain tissue over time.
TSEs are called "transmissible" because they can be transmitted from one individual to another through contact with infected tissues or consumption of contaminated material, although the specific mechanisms of transmission can vary depending on the specific disease. Examples of TSEs in humans include variant Creutzfeldt-Jakob disease (vCJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), and fatal familial insomnia (FFI). In animals, notable TSEs include bovine spongiform encephalopathy (BSE) in cattle, scrapie in sheep, and chronic wasting disease (CWD) in deer and elk.
One of the characteristic features of TSEs is the presence of sponge-like holes or vacuoles in the brain tissue, giving the diseases their name. These vacuoles result from the degeneration and death of nerve cells, leading to a range of symptoms such as dementia, loss of coordination, muscle stiffness, and behavioral changes. TSEs have a long incubation period, with symptoms typically appearing months or even years after exposure. The diseases are progressive and eventually fatal, with no known cure or effective treatment currently available.
Due to the potential for TSEs to spread through the food chain or medical procedures, strict measures have been put in place to prevent and control their transmission. These include regulations regarding the slaughter and disposal of infected animals, as well as procedures to minimize the risk of transmission in medical settings. Public health organizations and agencies continuously monitor and assess
