brain, spinal cord, and nerve disorders
Creutzfeldt Jakob Disease (CJD)
Variant CJD
Variant CJD (vCJD) is acquired by eating beef or beef products from cattle who have bovine spongiform encephalopathy (mad cow disease). vCJD usually begins around age 30 or younger, in contrast to sporadic CJD, which usually begins around age 65. As of May 2022, the following number of cases of vCJD have occurred: Worldwide: 233 United Kingdom: 178 Elsewhere: 55 Considering that for years, people in the United Kingdom ate contaminated beef without knowing it, the number of vCJD cases is surprising small. Spread of the disease has been controlled by massive slaughter of cattle and changes in how beef is processed in the United Kingdom. Widespread surveillance for the disease in cattle has resulted in a progressively lower number of new cases of vCJD in the United Kingdom, with only two cases after 2011. Four cases of vCJD have been diagnosed in the United States, and two have been diagnosed in Canada, but none of them originated in North America. An estimated 1 in 2,000 people in the United Kingdom have the abnormal prion protein that occurs in vCJD, but they have no symptoms. There is some concern that if these people donate blood or have a surgical procedure, other people may become infected. However, criteria for screening blood donors, which are specifically related to vCJD, may further reduce the risk of vCJD transmission by infected people. This risk is already very low outside of France and the United Kingdom. Mad cow disease has been reported in North American cattle (6 in the United States and 20 in Canada). The human form of mad cow disease (variant Creutzfeldt-Jakob disease), which is acquired from eating contaminated meat, has occurred in only 233 people total. 