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Prion Diseases

What are prion diseases?

Prion diseases comprise several conditions. A prion is a type of protein that can trigger normal proteins in the brain to fold abnormally. Prion diseases can affect both humans and animals. They are sometimes spread to humans by infected meat products. In many cases, the source of the abnormal protein is unknown. The most common form of prion disease that affects humans is Creutzfeldt-Jakob disease (CJD).

Prion diseases are rare. About 300 cases are reported each year in the U.S.

Types of prion diseases include:

What causes prion disease?

Normal prion protein is found on the surface of many cells. Prion diseases occur when this protein becomes abnormal and clumps in the brain. It then causes brain damage. This abnormal buildup of protein in the brain can lead to memory problems, personality changes, and trouble with movement. Experts still don't know a lot about prion diseases. But unfortunately, these disorders are often fatal.

Who is at risk for prion diseases?

You may be at risk for prion diseases if you:

What are the symptoms of prion diseases?

Symptoms of prion diseases include:

How are prion diseases diagnosed?

Prion diseases are confirmed by taking a sample of brain tissue during a biopsy or after death. But given the risks of a brain biopsy, a number of other tests are often done instead. These can help diagnose prion diseases such as CJD or rule out other diseases with similar symptoms. Prion diseases should be considered in all people who have dementia that is quickly getting worse.

The tests include:


How are prion diseases treated?

Prion diseases can't be cured. But certain medicines may help slow their progress. Medical care focuses on keeping people with these diseases as safe and comfortable as possible, despite worsening and debilitating symptoms.

Can prion diseases be prevented?

Properly cleaning and sterilizing medical equipment may prevent the spread of the disease. If you have or may have CJD, don't donate organs or tissue, including corneal tissue. Newer regulations that govern the handling and feeding of cows may help prevent the spread of prion diseases.

Living with prion diseases

As prion diseases progress, people with these diseases generally need help taking care of themselves. In some cases, they may be able to stay in their homes. But they eventually may need to move to a care facility.

Key points about prion diseases

Next steps

Tips to help you get the most from a visit to your healthcare provider: