What is Prion Disease? - Symptoms & Treatment

Instructor: Adrianne Baron

Adrianne has taught high school and college biology and has a master's degree in cancer biology.

Learn about prion disease and the symptoms that are associated with the disease. We will also look at the treatments that are used to address prion disease. After this lesson, you can take the short quiz to test your knowledge.

What is Prion Disease?

You may recall several years ago that there was a scare in regards to beef. The concern was over a disease that was showing up in cows called mad cow disease. Mad cow disease, formally called Bovine Spongiform Encephalitis or BSE, is a nervous system disease characterized as a prion disease, which causes the brain and spinal cord to degenerate into a spongy-like material.

BSE is prion disease in cows
Clipart of cow

Prions are small proteins made from prion proteins or PrP that are not in their normal formation. They aren't able to carry-out their normal functions due to this abnormal formation. Prions send signals out to neighboring proteins, which cause them to change their formation to the abnormal formation exhibited by prions. This essentially creates areas that look like sponges and are not able to function.

This slide shows the spongy appearance of the brain in prion disease
Slide of TSE brain tissue

It was determined that this disease could also be passed on to humans if they ate the beef that came from a cow with the disease. When the disease occurs in humans from consuming contaminated beef, it is known as variant Creutzfeldt-Jakob disease. There are other ways of developing prion disease.

It can come from receiving corneal transplants from a person infected with prion disease. There are instances where a person inherits the disease or it can occur spontaneously for no known reason at all, in which case it is referred to as sporadic Creutzfeldt-Jakob disease. Another form of prion disease occurs from consuming human brain tissue that is contaminated with prion disease. This prion disease is called Kuru and was pretty much only seen in New Guinea. All prion diseases in humans are collectively called Transmissible Spongiform Encephalopathies or TSE.

Symptoms of Prion Disease

Regardless of how prion disease is obtained or when it develops, the symptoms are the same and the end result is almost always fatal. Prions cause abnormal clumping of proteins in the brain and this can lead to difficulty with moving and speaking due to it interfering with the coordination centers in the brain. It can cause quickly developing dementia, which is memory loss due to loss of memory center function. Other symptoms can include hallucinations, fatigue, muscle stiffness, confusion and personality changes.

TSE causes confusion
Confused Smiley

Treatment of Prion Disease

Prion disease is almost always deadly, and this is because there isn't a cure for it at this time. It is also considered to be untreatable. There is some research being done in order to come up with a treatment plan or cure. The thing that physicians try to do at this point is to slow down how quickly the disease spreads through the brain. This is mostly carried out by decreasing other medications or activities that may increase confusion or decrease memory.

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