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Diseases & Topics

Creutzfeldt-Jakob Disease (CJD)

Classic Creutzfeldt-Jakob Disease (CJD) is a rare human prion disease that affects one to two persons per million per year worldwide. Part of a family of rare brain diseases called transmissible spongiform encephalopathies (TSEs), prion diseases occur in both humans and animals. The causative agent is believed to be a prion — not a bacteria or virus, but an abnormal protein that is infectious and able to induce abnormal folding of normal cellular prion proteins. These abnormal prions accumulate in brain cells and lead to a rapidly progressive neurodegenerative disease of the brain that is always fatal, usually within one year. The average age at death of CJD patients in the United States is 68 years.

The United States has reported fewer than 300 cases of CJD a year in recent years. CJD is not transmissible from person-to-person by normal contact. Most (about 85%) of CJD cases develop spontaneously (no known cause) and are called Sporadic CJD (sCJD). Genetic or familial CJD, represents about 5-15 percent of cases and is associated with inherited mutations of the prion protein gene (examples are Gerstmann-Straussler-Scheinker Syndrome and fatal familial insomnia). Less than one percent of cases occur during invasive medical procedures involving the central nervous system and are due to exposure to contaminated brain tissue.

Individuals with CJD experience a rapid onset of dementia and a range of neurological symptoms including walking difficulties, sudden jerky movements, and sometimes, visual disturbances. An autopsy is very important in the diagnosis of CJD because it is the best way to confirm presence of the disease.

Classic CJD is not related to "mad cow" disease, or Bovine Spongiform Encephalopathy (BSE). Classic CJD also is distinct from "variant CJD," another prion disease that can affect humans; variant CJD is related to BSE.

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