Wisconsin Department of Agriculture, Trade and Consumer Protection

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Animal Diseases

New-Variant Creutzfeldt-Jakob Disease

New-variant Creutzfeldt-Jakob disease (nv-CJD) is a human disease of the central nervous system that was first diagnosed in the United Kingdom in 1995. Scientists have linked the disease to eating meat products from cattle infected with bovine spongiform encephalopathy, commonly called mad cow disease.

It's important to understand why it's called "new variant," as opposed to "classic" Creutzfeldt-Jakob.

Classic CJD has always been with us. It's very rare, striking about 1 person in a million every year, worldwide. Mostly these cases are "sporadic," meaning they have no known cause. A few have been linked to organ transplants from infected people, or to genetics. Americans die from classic CJD, but they do not contract it from contaminated meat. Classic CJD strikes people 50 or older, progresses rapidly, and has only physical symptoms.

Except for a few cases in other European nations with BSE, nv-CJD has been found almost exclusively in the United Kingdom. It strikes younger people, often begins with psychiatric symptoms, and progresses slowly. Unlike classic CJD, nv-CJD has been linked to eating meat products from cattle that had BSE. Also unlike classic CJD, nv-CJD cases increased for a number of years, although the number per year now appears to be dropping off in the United Kingdom as the result of preventive action. From the original 3 cases in 1995, numbers per year grew to 27 in 2000, but only 7 cases have been confirmed in the first half of 2001. As of June 29, 2001, authorities had counted 102 definite and probable cases of nv-CJD in the United Kingdom. In the United States, there has been only one known case of nv-CJD. A Florida woman died in June 2004 after eating BSE-contaminated beef more than a decade ago in England.

Both diseases eventually destroy victims' motor skills and coordination, and both show the characteristic spongelike appearance in their victims' brains when autopsied. Both are fatal and transmissible spongiform encephalopathies.

Read more about CJD in the United Kingdom and how this disease could affect travel.