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:
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.
You may be at risk for prion diseases if you:
Symptoms of prion diseases include:
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
The tests include:
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
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.
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
Tips to help you get the most from a visit to your healthcare provider:
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