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Mad Cow Disease and Variant Creutzfeldt-Jakob (cont.)

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:

  • The USDA's Animal and Plant Health Inspection Service (APHIS) has prohibited the importation of live cattle and certain cattle products, including rendered protein products, from countries where BSE is known to exist. In 1997, due to concerns about widespread risk factors and inadequate surveillance for BSE in many European countries, these importation restrictions were extended to include all of the countries in Europe.
  • APHIS has prohibited all imports of rendered animal protein products, regardless of species, from BSE-restricted countries because of concern that feed intended for cattle may have been cross-contaminated with the BSE agent.
  • In 1997, the FDA prohibited the use of most animal protein in the manufacture of animal feeds given to cattle and other hoofed animals (known as ruminants).
  • The Centers for Disease Control and Prevention (CDC) leads a surveillance system for vCJD and performs random testing (screening) on U.S. cattle slaughtered for human consumption.
  • Additional safeguards include keeping downer animals out of the human food chain because these are at highest risk for being ill with BSE, tracking cattle from birth to slaughter in case an infected animal is found and its meat can be destroyed, and using less aggressive ways to harvest meat from carcasses to avoid brain and spinal tissues. An additional feed-control ban was implemented in the U.S. in 2009 to align U.S. practices with a similar ban enacted in Canada in 2007. These regulations prohibit the use of high-risk parts of cattle in the generation of any animal feed.

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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Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy »

Bovine spongiform encephalopathy (BSE), also known as mad cow disease, and variant Creutzfeldt-Jakob disease (CJD) are related disorders.

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