Transmissible spongiform encephalopathies, or TSE, are a group of progressive, incurable, and fatal diseases associated with the prion protein hypothesis that affect the brains and nervous systems of many animals, including humans, cattle, and sheep . The spread of this disease is primarily attributed to the presence of prion proteins, although there is some data showing an association with minimal meningeal bacterial infections.
"Transmissible spongiform encephalopathy is a unique disease that may be genetic, sporadic, or transmitted through ingestion of infected food."
Under the influence of TSE, brain function gradually deteriorates, resulting in memory loss, personality changes, and abnormal or impaired motor abilities. The main forms of TSE include Creutzfeldt-Jakob disease (CJD), Geltmann-Straussler-shank syndrome (GSS), fatal familial insomnia, and Gulu disease.
CJD comes in many forms, including sporadic CJD, hereditary CJD, iatrogenic CJD, and variant CJD. Symptoms have overlapping characteristics.
"Persistent neuronal death and spongiform changes are the main features of this disease."
In addition to humans, TSEs in non-human animals include velvet disease in sheep, mad cow disease (BSE) in cattle, and chronic attenuating disease in deer and deer. Variant Creutzfeldt-Jakob disease in cattle is directly related to the spread of BSE, and this high-risk cause has attracted global attention.
Different from other infectious diseases, the infectious agent of TSE is prion protein, which is composed only of proteins. This misfolded protein spreads between individuals by causing deformations in other proteins through contact. Most cases of TSE are sporadic, and only a few cases are related to genetic mutations.
"Most of the spread of tamarinosis occurred in the 1980s and 1990s because cattle were fed the processing residues of other cattle."
This inappropriate feeding practice has been banned in many countries to prevent its spread, but it has caused a health threat to humans for decades. Human consumption of contaminated beef has led to a wave of outbreaks of variant Creutzfeldt-Jakob disease, further revealing the dangers of TSE to public health.
Clinically, symptoms of affected humans include personality changes, mental illness, poor coordination and unsteady gait. In the later stages, patients often exhibit severe mental impairment (dementia) and eventually lose the ability to move or communicate.
"Transmissible spongiform encephalopathies in mammals have shown links to human diseases, such as the spread of gulu disease and the practice of cannibalism."
Diagnosing diseases such as TSE is challenging because they have incubation periods of years or even decades and cannot be detected before symptoms appear. However, scientists are working to develop new detection methods to try to identify affected individuals in advance.
Currently, there is no treatment that can cure or prevent this disease. Existing treatments are mainly supportive care. Understanding the neuropathology caused by these proteins may change our understanding of and response to these deadly diseases in the future.
As science continues to advance, our knowledge of TSEs and the contagiousness that ripples out from them continues to be updated. What we need to understand is how will these mysterious lesions continue to challenge our medical system?