Prion disease
Prion Disease
Prion disease (/priːɒn/), also known as transmissible spongiform encephalopathies (TSEs), is a group of progressive conditions that affect the nervous system in humans and animals. The term "prion" (/priːɒn/) is derived from "proteinaceous infectious particle", a name that signifies its unique nature as a pathogen.
Etymology
The term "prion" was first used in 1982 by Stanley B. Prusiner, an American neurologist who won the Nobel Prize in Physiology or Medicine in 1997 for his research on prions. The term is a blend of the words "protein" and "infection".
Types of Prion Diseases
In humans, prion diseases can be inherited, acquired through infection, or appear spontaneously. The most common forms of prion diseases in humans include:
- Creutzfeldt-Jakob disease (CJD)
- Variant Creutzfeldt-Jakob disease (vCJD)
- Kuru
- Fatal familial insomnia (FFI)
- Gerstmann-Sträussler-Scheinker syndrome (GSS)
In animals, prion diseases include:
- Scrapie in sheep and goats
- Bovine spongiform encephalopathy (BSE), also known as "mad cow disease", in cattle
- Chronic wasting disease (CWD) in deer and elk
Symptoms
Symptoms of prion diseases are initially subtle and include minor issues with movement or coordination, personality changes, and memory problems. As the disease progresses, these symptoms become more severe, leading to physical and mental disabilities.
Diagnosis and Treatment
Diagnosis of prion diseases is challenging due to their rarity and the lack of specific tests. The definitive diagnosis can only be confirmed by examining the brain tissue during an autopsy.
There is currently no cure for prion diseases. Treatment is focused on relieving symptoms and making the patient as comfortable as possible.
See Also
External links
- Medical encyclopedia article on Prion disease
- Wikipedia's article - Prion disease
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