are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce an inflammatory response. 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.

Causes

Prion diseases occur when normal prion protein, found on the surface of many cells, becomes abnormal and clump in the brain, causing brain damage. 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.

Symptoms

• Rapidly developing dementia
• Difficulty walking and changes in gait
• Hallucinations
• Muscle stiffness
• Confusion
• Fatigue
• Difficulty speaking

Prevention

Properly cleaning and sterilizing medical equipment may prevent the spread of the disease. If you have or may have CJD, do not 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.

Treatment

Prion diseases can’t be cured, but certain medicines 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. Examples of this type of care include:

• Assistance. As the disease advances, many people need help taking care of themselves and performing daily activities.
• Providing hydration and nutrients. In advanced stages of the disease, IV fluids or a feeding tube may be required.

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 (www.hopkinsmedicine.org)