Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative and fatal brain disorder caused by an infectious agent known as a "prion." Typically, the disease occurs at about age 60 and 90 percent of patients diagnosed with CJD die within a year.

In the early stages, symptoms may include failing memory, behavioral changes, lack of coordination and vision problems.

As the illness progresses, mental deterioration becomes more severe and involuntary movements, blindness, weakness in the arms and legs and then a coma may occur.

CJD may occur in three ways:

• Inherited, when there's a family history or a genetic mutation associated with the disease. About 5 to 10 percent of cases in the United States are hereditary.
• Sporadically without apparent cause and no known risk factors. This is the common form of Creutzfeldt-Jakob disease and accounts for about 85 percent of cases.
• Transmitted through infection when exposed to brain or nervous system tissue, usually through medical procedures.

There is no evidence that Creutzfeldt-Jakob disease is contagious or that it can be spread through casual contact with a patient. Since 1920, less than 1 percent of the reported cases were acquired through infection.

The disease is part of a family of diseases known as transmissible spongiform encephalopathies (TSE). Spongiform refers to the appearance of the infected brain, which becomes filled with holes until it resembles a sponge under a microscope. CJD is the most common of these diseases. Other forms occur in animals such as bovine spongiform encephalopathy (BSE), which develops in cows and often is referred to as "mad cow" disease. Similar diseases occur in cats, elk, deer, goats, sheep and exotic zoo animals.

Our Approach to Creutzfeldt-Jakob Disease

As international leaders in the field of dementia disorders, UCSF specialists are working to find new treatments and a cure for Creutzfeldt-Jakob disease. We offer the full scope of diagnostic tests and therapies to control symptoms and keep patients as comfortable as possible.