Prion diseases are a group of rare neurodegenerative disorders that can affect both humans and animals. Show
They’re caused byabnormally folded proteinsin the brain, particularly the misfolding of prion proteins (PrP). This leads to a progressive decline in brain function, involving changes in memory, behavior, and movement. Eventually, prion diseases are fatal. Around 300 new cases of prion disease are reported each year in the United States. They can be:
In people with prion disease, the misfolded PrP can bind to healthy PrP, which causes the healthy protein to also fold abnormally. Misfolded PrP begins to accumulate and form clumps within the brain, damaging and killing nerve cells. This damage causes tiny holes to form in brain tissue, making it appear sponge-like under a microscope. (That’s why you may see prion diseases referred to as “spongiform encephalopathies.”) Researchers are still working to understand more about prion diseases and find an effective treatment. But they do know some things. Read on to find out about the different types of prion disease, if there are any ways to prevent them, and more. Types of prion diseasesPrion disease can occur in both humans and animals. Below are some different types of prion diseases. More information about each disease follows the table. Human prion diseasesAnimal prion diseasesCreutzfeldt-Jakob disease (CJD)Bovine spongiform encephalopathy (BSE)Variant Creutzfeldt-Jakob disease (vCJD)Chronic wasting disease (CWD)Fatal familial insomnia (FFI)ScrapieGerstmann-Straussler-Scheinker syndrome (GSS)Feline spongiform encephalopathy (FSE)KuruTransmissible mink encephalopathy (TME)Ungulate spongiform encephalopathy Human prion diseases
Risk factors for these diseases include:
Animal prion diseases
Diseases caused by prionsThe prion diseases highlighted above aren’t the only diseases linked to prions. Other neurodegenerative diseases, like Alzheimer’s and Parkinson’s, are also associated with misfolded proteins in the central nervous system. And research has found that some of these misfolded proteins may be prions. But some scientists believe that these proteinsonly act in a prion-like way. They argue that they can’t be prions as the diseases they cause, like Alzheimer’s, aren’t thought to be infectious. What are the symptoms of prion disease?Prion diseases have very long incubation periods, often on the order of many years. When symptoms develop, they progressively worsen, sometimes rapidly. Common symptoms of prion disease include:
How is prion disease treated? (adsbygoogle = window.adsbygoogle || []).push({});There’s currently no cure for prion disease. But treatment focuses on providing supportive care. Examples of this type of care include:
Scientists continue to work to find an effective treatment for prion diseases. Some of the potential therapies being investigated include the use of anti-prion antibodies and “anti-prions” that inhibit the replication of abnormal PrP. How are prion diseases diagnosed?Since prion diseases can present similar symptoms to other neurodegenerative disorders, they can be difficult to diagnose. The only way to confirm a diagnosis of prion disease is through a brain biopsy performed after death. But a healthcare professional can use your symptoms, medical history, and several tests to help diagnose prion disease. The tests they may use include:
Can prion disease be prevented? (adsbygoogle = window.adsbygoogle || []).push({});Several measures have been taken to prevent the transmission of acquired prion diseases. Because of these proactive steps, acquiring a prion disease from food or a medical setting is now extremely rare. Some of the preventive steps taken include:
There’s currently no way to prevent inherited or sporadic forms of prion disease. If someone in your family has an inherited prion disease, you may consider consulting with a genetic counselor to discuss your risk of developing the disease. Key takeawaysPrion diseases are a rare group of neurodegenerative disorders caused by abnormally folded proteins in your brain. The misfolded protein forms clumps that damage nerve cells, leading to a progressive decline in brain function. Some prion diseases are genetically transmitted, while others can be acquired through contaminated food or medical equipment. Other prion diseases develop without any known cause. There’s currently no cure for prion diseases. Instead, treatment focuses on providing supportive care and easing symptoms. Researchers continue to work to discover more about these diseases and to develop potential treatments. What are 5 diseases caused by prions?Identified Prion Diseases. Creutzfeldt-Jakob Disease (CJD). Variant Creutzfeldt-Jakob Disease (vCJD). Gerstmann-Straussler-Scheinker Syndrome.. Fatal Familial Insomnia.. How many types of prion diseases are there?There are 3 major prion diseases in animals: scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and chronic wasting disease (CWD) in deer, elk and other cervids. NIAID scientists use various experimental models to study how prions from one animal species can infect different animal species.
What are prion type diseases?Prion diseases are rare, fatal, degenerative brain disorders that are thought to occur worldwide in both humans and animals. They belong to the general category of brain diseases called proteinopathies, which also includes Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis (ALS).
What are the three categories of prion diseases?The three main forms are: Genetic, Sporadic and Acquired. Genetic disease is caused by an abnormality in a gene (specifically a mutation in the prion protein gene, PRNP). Sporadic disease is of unknown cause.
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