Some facts regarding Prion Disease

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Several conditions fall under the term prion diseases. 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 and are sometimes transmitted to humans by infected meat products. The most common form of prion disease that affects humans is Creutzfeldt-Jakob disease (CJD). Prion diseases lead to brain damage when prion proteins cause abnormal clumping in the brain. This abnormal accumulation of protein in the brain can cause memory impairment, personality changes, and difficulties with movement. Experts still don't know a lot about prion diseases, but unfortunately, these disorders are generally fatal.

George has been diagnosed with CJD. This condition can be inherited, in which case it's called familial CJD. Sporadic CJD, on the other hand, develops suddenly without any known risk factors. Most cases of CJD are sporadic and tend to strike people around age 60. Symptoms of CJD quickly lead to severe disability and death. In most cases, death occurs within a year.

Prion diseases can't be cured, but certain medications may help slow their progress. Medical management focuses on keeping people with these diseases as safe and comfortable as possible despite progressive and debilitating symptoms. As prion diseases progress, people with these diseases generally need help taking care of themselves and their daily needs. In some cases they may be able to stay in their homes, but they eventually may need to move to a care facility.

From the Johns Hopkins Medical Library.