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Creutzfeldt-Jakob disease (CJD) Print E-mail

Dr. Kennedy Creutzfeldt-Jakob disease (CJD) is a degenerative, invariably fatal brain disorder affecting about one person in every one million each year worldwide. In the U,S. there are 200+ cases per year. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90% of patients die within one year. Early stages involve failing memory, behavioral changes, lack of coordination and visual disturbances. With progression, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur. There are three major categories of CJD. Sporadic CJD appears even though the person has no known risk factors for the disease and is the most common type of CJD accounting for at least 85% of cases. Hereditary CJD involves a family history of the disease and/or tests positive for a genetic mutation associated with CJD. About 5 to 10% of cases of CJD in the U.S. are hereditary. Acquired CJD is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures. There is no evidence that CJD is contagious through casual contact with a CJD patient. Since CJD was first described in 1920, fewer than 1% of cases have been acquired CJD. CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains which become filled with holes until they resemble sponges under a microscope. CJD is the most common of the known human TSEs. Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS).



The information in this article is not meant to be medical advice.�Treatment for a medical condition should come at the recommendation of your personal physician.

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