“Mad Cow” Disease and Creutzfeldt-Jakob disease (CJD)

There appear to be two forms of Creutzfeldt-Jakob disease (CJD). Rare but well-known for years, classic CJD is a fatal brain disorder that usually occurs in people over the age of 60. Every year about one person out of every million is diagnosed with CJD in the United States, and an average of six North Carolinians die each year from classic CJD. Sometimes called a "spongiform" disease because the brain may develop holes in it like a sponge, CJD is caused by small proteins called prions, which are smaller than bacteria or viruses. There is no known treatment. It is not known how most people get this rare disease, although people can not get it from being around someone with CJD. Approximately 10-15 percent of cases are inherited; a few cases have been related to transplants of tissues from the nervous system.

In 1995 a new kind of CJD, called “new variant CJD” (vCJD), was recognized in Britain. There is no known treatment of vCJD and, like CJD, it is invariably fatal. Persons who have developed vCJD are believed to have become infected through their consumption of meat from cattle that had “mad cow disease” (bovine spongiform encephalopathy, or BSE). As of December 1, 2003, a total of 153 vCJD cases had been reported worldwide, 143 of them in the United Kingdom, where where over 1 million cattle may have been infected with BSE. However, the risk to human health from BSE in the United States is extremely low.

The first likely case of vCJD was identified in the United States in Florida in 2002, in a 22-year-old United Kingdom (UK) citizen residing in the United States. Investigators concluded that the disease was contracted in the UK (see Morbidity and Mortality Weekly Report, October 18, 2002). The first case of BSE in the United States was identified in December 2003 in an adult Holstein cow from Washington state that had been imported into the United States from Canada. The case is under investigation by the Centers for Disease Control and Prevention and other agencies.

For more information:
Bovine spongiform encephalopathy and Creutzfeldt-Jakob disease - Centers for Disease Control and Prevention (CDC)

Consumer Questions and Answers about BSE – N.C. Department of Agriculture and Consumer Services

How North Carolina is working to prevent Mad Cow Disease – N.C. Department of Agriculture and Consumer Services

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