Transmissible Spongiform Encephalopathy (TSE) refers to a group of rare and fatal neurodegenerative diseases that affect both humans and animals. This group of diseases is characterized by the accumulation of an abnormal isoform of a protein called prion in the brain, leading to the development of sponge-like holes and a progressive loss of brain function.
TSEs are highly unusual as they can be transmitted from one individual to another through contact with infected tissues, ingestion of contaminated materials, or exposure to infectious prions. There are several forms of TSEs, including Creutzfeldt-Jakob disease (CJD), variant CJD (vCJD), kuru, fatal familial insomnia, and Gerstmann-Sträussler-Scheinker syndrome.
Symptoms of TSEs typically include rapidly progressing dementia, memory loss, personality changes, impaired coordination and motor abilities, and eventually severe neurological impairment. The abnormal prion protein has the ability to convert normal prion proteins into the abnormal form, leading to the spreading of the disease within the brain and subsequent tissue damage.
Diagnosis of TSEs often involves conducting neurological examinations, analyzing cerebrospinal fluid, performing brain imaging, and sometimes performing a brain biopsy. Unfortunately, there is currently no cure for TSEs, and treatment mainly focuses on managing symptoms and improving the quality of life for affected individuals.
Prevention of TSEs involves strict implementation of control measures, such as the removal of high-risk tissues from the food chain, proper sterilization of medical equipment, and surveillance of the spread of the disease.
