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Creutzfeldt-Jakob disease (CJD)
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Creutzfeldt-Jakob disease (CJD)
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CJD is a rare, fatal brain disorder in humans, which causes a rapid, progressive dementia and associated neuromuscular disturbances. The disease is often referred to as a subacute spongiform encephalopathy because it usually produces microscopic vacuoles, or holes, in neurons that appear "sponge-like." CDJ affects about one person in every one million people per year worldwide. In the United States there are about 200 cases per year. CJD usually appears in later life and runs a rapid course. CJD occurs in a form associated with a hereditary predisposition (approximately 5–10% of all cases) and in a more common (or sporadic) form that accounts for 85–90% of cases.
A newly recognized form of CJD, variant Creutzfeldt-Jakob disease (vCJD), was first reported in March 1996 in the UK. In contrast to the classical forms of CJD, vCJD has affected younger patients (average age 29 years, as opposed to 65 years), has a relatively longer duration of illness (median of 14 months as opposed to 4.5 months) and is strongly linked to exposure to BSE (probably through food). In the United Kingdom, where over 1 million cattle may have been infected with BSE, a substantial species barrier appears to protect humans from widespread illness. As of December 1, 2003, a total of 153 vCJD cases had been reported worldwide; of these, 143 cases had occurred in the United Kingdom. The risk to human health from BSE in the United States is extremely low.
Additional CJD Information
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