What are TSEs?

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Transmissible spongiform encephalopathies (TSEs) are neurological diseases characterized by the degeneration of brain tissue, which causes the formation of numerous plaques and small holes. When the brain is examined during an autopsy, it looks a bit like a sponge, which explains the term “spongiform.” The cause of TSEs is generally thought to be an abnormal protein called a prion, and these diseases currently have no cure.

Prions are proteins that contain no genetic material, instead using unique folding behaviors to replicate, infecting healthy proteins along the way. Prions can survive extreme temperature changes and a variety of antiseptics, making them very difficult to get rid of. Prion diseases are a concern in many communities because the diagnosis is often missed, as symptoms can resemble other conditions, such as dementia. Generally, humans are only diagnosed with TSE when they display traits associated with a rare genetic version or when they experience dementia at an unusually young age, and a definitive diagnosis can only be obtained at autopsy, as it requires inspection of brain tissue .

Symptoms of TSEs typically begin with subtle personality changes and get progressively worse as nervous system function is disrupted. Patients may have difficulty walking or talking and often experience violent dementia as the infection literally punctures the brain. After a certain point, the nervous system simply fails, causing the patient to fall into a coma and die.

TSEs can be found in a number of animals. In humans, the best-known example is probably Creutzfeldt-Jakob disease, but other human TSEs include Gerstmann-Straussler-Scheinker disease, kuru, and fatal familial insomnia. Sheep and goats can get scrapie, while cows get bovine spongiform encephalitis (BSE), also known as mad cow disease. TSEs have also been diagnosed in deer and elk (chronic wasting disease), cats (feline spongiform encephalitis), and mink (transmissible mink encephalopathy).

In all cases, the animals appear to contract TSE by ingesting infected neurological tissue. In animals, the most common cause is contaminated feed, as the practice of including processed animal parts in feed for additional protein is quite common. TSE can also be contracted from brain surgery, although this is rare, as the prions responsible for TSE are able to survive autoclaving.

Since these diseases cannot be cured, most treatment focuses on keeping the patient comfortable. When a TSE is suspected, patients may be offered muscle relaxants to help them cope with the twitching and other physical symptoms associated with late-stage TSE, as the body begins to spin out of control. These drugs can also keep patients with extreme dementia more relaxed, making them easier to treat.

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