Mad Cow Disease and Variant Creutzfeldt-Jakob (cont.)
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Prognosis of Variant Creutzfeldt-Jakob Disease
Human prion diseases are devastating and incurable but extremely rare. Fears that an epidemic of the animal disease (BSE) would lead to an epidemic of the human form (vCJD) have not occurred, even in the U.K. where entire herds of diseased cattle were destroyed and over 184,000 animals were known to develop the disease.
According to the CDC, over 200 cases of vCJD have been reported in the world, with about 170 having been reported from the United Kingdom. Other countries, including France, Canada, Ireland, Italy, and the United States, have reported cases, although some of the affected individuals in other countries were known to have resided the U.K. during a key exposure period of the U.K. population to the BSE agent.
The U.S. government has implemented a number of measures to prevent BSE from entering the U.S. and to prevent the spread of the disease, as outlined by the Food Safety and Inspection Service (FSIS). Some of these measures include the following:
Live animal tests may provide valuable information about the level of BSE in all animals, including downer cattle and cattle aged 24 months or older (those most at risk and in whom BSE is found). However, currently there is no sensitive and reliable live animal test for BSE and the only definitive test can be made on the brain and other tissues after slaughter.
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Bovine spongiform encephalopathy (BSE), also known as mad cow disease, and variant Creutzfeldt-Jakob disease (CJD) are related disorders.