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Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, invariably fatal brain disorder. It affects about one person in every one million people per year worldwide. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 percent of patients die within 1 year.
Major categories of CJD:
the disease appears even though the person has no known risk factors for the disease. This is by far the most common type of CJD.
the person has a family history of the disease and/or tests positive for a genetic mutation associated with CJD.
the disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures.
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.
In the early stages of disease, patients may have failing memory, behavioral changes, lack of coordination and visual disturbances. As the illness progresses, mental deterioration becomes pronounced and involuntary movements, blindness, weakness of extremities, and coma may occur.
Brain shrinkage and deterioration occurs rapidly
Source: U.S. Department of Health and Human Services, National Institutes of Health, National Institute of Neurological Disorders and Stroke. www.ninds.nih.gov
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