What Are Prion Diseases?

Prion diseases are a rare group of brain disorders that affect both humans and animals. Learn more about prion diseases, including traits, diagnosis, and when to seek treatment.

January 17, 2025 · 6 min · 1276 words · Katelyn Harper

Table of Contents

Table of ContentsView AllTable of ContentsWhat Are Prion Diseases?SymptomsIdentifying Prion DiseasesCausesTypesTreatmentPreventionCoping

Table of ContentsView All

View All

Table of Contents

What Are Prion Diseases?

Symptoms

Identifying Prion Diseases

Causes

Types

Treatment

Prevention

Coping

Close

Prion disease is an umbrella term used to refer to a rare group ofbrain disordersthat affect both humans and animals.This makes it possible for the disease to spread from animal to people and, in some instances, from human to human.

Researchers don’t completely understand why abnormal prion proteins form. Prion diseases are also known astransmissible spongiform encephalopathies(TSEs). These diseases are incredibly rare, and only about 300 cases are diagnosed in the United States every year. Abnormal prion proteins are capable of infecting normal prion proteins—this can turn them into defective proteins that cause disease.

Symptoms of Prion Diseases

Symptoms of prion diseases vary depending on what type of prion disease you have. However, in general, they tend to be fatal and, in many cases, have high mortality rates. Some of the most common symptoms that many prion diseases share include:

What Are the Early Signs of Dementia?

Doctors and healthcare providers typically run several tests to rule out any other conditions in suspected cases of prion diseases. When peoplesuddenly develop dementia, and it progresses rapidly (sometimes over the course of days or weeks), prion diseases are typically considered a likely culprit. Some of the tests done to help identify if you have a prion disease include:

Causes of Prion Diseases

Certain factors put people at a higher risk of developing prion diseases than others. They include:

Types of Prion Diseases

Prion diseases affect both humans and animals. In some cases, prion diseases that affect animals could be transmitted to humans too.

Human Prion Diseases

The two most common forms of human prion diseases include:

Other rare human prion diseases are:

Is Alzheimer’s Disease a Prion Disease?There is a common misconception that Alzheimer’s is a prion disease. This is because they are both neurodegenerative disorders that are linked to the build-up of abnormal proteins on the brain which causes damage. However, while prion disease is linked to the abnormal build-up of prion proteins, Alzheimer’s is linked to the abnormal build-up of proteins called amyloid plaques.

Is Alzheimer’s Disease a Prion Disease?

There is a common misconception that Alzheimer’s is a prion disease. This is because they are both neurodegenerative disorders that are linked to the build-up of abnormal proteins on the brain which causes damage. However, while prion disease is linked to the abnormal build-up of prion proteins, Alzheimer’s is linked to the abnormal build-up of proteins called amyloid plaques.

Animal Prion Diseases

The most common animal prion diseases include:

Other less common animal prion diseases include:

Treatment for Prion Diseases

There’s currently no cure for prion diseases. However, there is ongoing research into developing effective treatments for the diseases. The focus of treatment is to make a person who has the condition as comfortable as possible as they live with the disease’s debilitating symptoms.

Slowing Disease Progression and Alleviating Symptoms

Treatment also aims to slow the rate at which the disease progresses. In specific scenarios,opiatedrugs might be prescribed to relieve pain. A medication likeKlonopin (clonazepam)may also be prescribed to help with symptoms like involuntary muscle spasms.

Preventing Prion Diseases

It’s also possible to prevent prion diseases. Making sure medical pieces of equipment are properly cleaned and sterilized is an excellent first step.

Also, being careful of the kinds of animal meat you consume and where you get it from is essential. Regulatory bodies are also closely monitoring how cows are fed to ensure that they don’t get infected with prion diseases that can be spread to humans.

Coping With Prion Diseases

Prion diseases are fatal and deadly. Most of its treatment features support for as long as the person has the condition. If you have just been diagnosed with a prion disease, it’s essential to start thinking about end-of-life care.

It’s advisable to give directives to your close family and friends on what to do when you can no longer support yourself and in the event of your passing.

A Word From Verywell

14 SourcesVerywell Mind uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Onodera T, Sakudo A.Introduction to current progress in advanced research on prions. Current Issues in Molecular Biology. Published online 2020:63-66.National Institute of Allergy and Infectious Diseases.Prion Diseases.John Hopkins Medicine.Prion Diseases.Kübler E, Oesch B, Raeber AJ.Diagnosis of prion diseases.British Medical Bulletin. 2003;66(1):267-279.Stanford Healthcare.Diagnosis of Prion Diseases.MSD Manual Professional Edition.Overview of prion diseases - neurologic disorders. July 2020Creutzfeldt-Jakob Disease Foundation.Types of prion diseases.US Food Drug and Administration (FDA).Bovine spongiform encephalopathy (Bse) questions and answers.Kellett KAB, Hooper NM.Prion protein and Alzheimer’s disease.Prion. 2009;3(4):190-194.MSD Veterinary Manual.Overview of scrapie - nervous system.Wyoming Department of Health.Prion diseases.Oregon Health & Science University (OHSU).Prion Diseases.National Institute of Neurological Disorders and Stroke.Creutzfeldt-jakob disease fact sheet.Stanford Healthcare.Prevention of Prion Diseases.

14 Sources

Verywell Mind uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Onodera T, Sakudo A.Introduction to current progress in advanced research on prions. Current Issues in Molecular Biology. Published online 2020:63-66.National Institute of Allergy and Infectious Diseases.Prion Diseases.John Hopkins Medicine.Prion Diseases.Kübler E, Oesch B, Raeber AJ.Diagnosis of prion diseases.British Medical Bulletin. 2003;66(1):267-279.Stanford Healthcare.Diagnosis of Prion Diseases.MSD Manual Professional Edition.Overview of prion diseases - neurologic disorders. July 2020Creutzfeldt-Jakob Disease Foundation.Types of prion diseases.US Food Drug and Administration (FDA).Bovine spongiform encephalopathy (Bse) questions and answers.Kellett KAB, Hooper NM.Prion protein and Alzheimer’s disease.Prion. 2009;3(4):190-194.MSD Veterinary Manual.Overview of scrapie - nervous system.Wyoming Department of Health.Prion diseases.Oregon Health & Science University (OHSU).Prion Diseases.National Institute of Neurological Disorders and Stroke.Creutzfeldt-jakob disease fact sheet.Stanford Healthcare.Prevention of Prion Diseases.

Verywell Mind uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.

Onodera T, Sakudo A.Introduction to current progress in advanced research on prions. Current Issues in Molecular Biology. Published online 2020:63-66.National Institute of Allergy and Infectious Diseases.Prion Diseases.John Hopkins Medicine.Prion Diseases.Kübler E, Oesch B, Raeber AJ.Diagnosis of prion diseases.British Medical Bulletin. 2003;66(1):267-279.Stanford Healthcare.Diagnosis of Prion Diseases.MSD Manual Professional Edition.Overview of prion diseases - neurologic disorders. July 2020Creutzfeldt-Jakob Disease Foundation.Types of prion diseases.US Food Drug and Administration (FDA).Bovine spongiform encephalopathy (Bse) questions and answers.Kellett KAB, Hooper NM.Prion protein and Alzheimer’s disease.Prion. 2009;3(4):190-194.MSD Veterinary Manual.Overview of scrapie - nervous system.Wyoming Department of Health.Prion diseases.Oregon Health & Science University (OHSU).Prion Diseases.National Institute of Neurological Disorders and Stroke.Creutzfeldt-jakob disease fact sheet.Stanford Healthcare.Prevention of Prion Diseases.

Onodera T, Sakudo A.Introduction to current progress in advanced research on prions. Current Issues in Molecular Biology. Published online 2020:63-66.

National Institute of Allergy and Infectious Diseases.Prion Diseases.

John Hopkins Medicine.Prion Diseases.

Kübler E, Oesch B, Raeber AJ.Diagnosis of prion diseases.British Medical Bulletin. 2003;66(1):267-279.

Stanford Healthcare.Diagnosis of Prion Diseases.

MSD Manual Professional Edition.Overview of prion diseases - neurologic disorders. July 2020

Creutzfeldt-Jakob Disease Foundation.Types of prion diseases.

US Food Drug and Administration (FDA).Bovine spongiform encephalopathy (Bse) questions and answers.

Kellett KAB, Hooper NM.Prion protein and Alzheimer’s disease.Prion. 2009;3(4):190-194.

MSD Veterinary Manual.Overview of scrapie - nervous system.

Wyoming Department of Health.Prion diseases.

Oregon Health & Science University (OHSU).Prion Diseases.

National Institute of Neurological Disorders and Stroke.Creutzfeldt-jakob disease fact sheet.

Stanford Healthcare.Prevention of Prion Diseases.

Meet Our Review Board

Share Feedback

Was this page helpful?Thanks for your feedback!What is your feedback?HelpfulReport an ErrorOtherSubmit

Was this page helpful?

Thanks for your feedback!

What is your feedback?HelpfulReport an ErrorOtherSubmit

What is your feedback?