Mad Cow Disease

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What is mad cow disease, and does it infect people?

Mad cow disease is a fatal disease that slowly destroys the brain and spinal cord (central nervous system) in cattle. It also is known as bovine spongiform encephalopathy, or BSE.

People cannot get mad cow disease. But in rare cases they may get a human form of mad cow disease called variant Creutzfeldt-Jakob disease (vCJD), which is fatal.

This can happen if you eat nerve tissue (the brain and spinal cord) of cattle that were infected with mad cow disease. Over time, vCJD destroys the brain and spinal cord.

There is no evidence that people can get mad cow disease or vCJD from eating muscle meat—which is used for ground beef, roasts, and steaks—or from consuming milk or milk products.

People with vCJD cannot spread it to others through casual contact.

People who have spent 3 months or more in places such as the United Kingdom or France between 1980 and 1996 are not allowed to give blood in the United States or Canada.^{1, 2} This is to help prevent vCJD from spreading.

What causes mad cow disease and variant Creutzfeldt-Jakob disease (vCJD)?

Experts are not sure what causes mad cow disease or vCJD.

One theory is that the disease is caused by changes to proteins found in animal cells. In affected cows, the changed proteins are found in the brain, spinal cord, and small intestine. There is no proof that these changed proteins are found in muscle meat (such as steak) or in milk.

Another theory is that mad cow disease is caused by a virus that causes the proteins to change.³

When a cow is slaughtered, parts of it are used for human food and other parts are used in animal feed. If an infected cow is slaughtered and its nerve tissue is used in cattle feed, other cows can become infected.

People can get vCJD if they eat the brain or spinal cord tissue of infected cattle.

How common are mad cow disease and vCJD?

The first case of vCJD was reported in 1996. Since then, there have been few cases of vCJD reported in the world. Most of the cases have been in countries that are part of the United Kingdom (England, Scotland, Wales, and Northern Ireland).

In December 2003, mad cow disease was discovered in one cow in the United States. Before this cow was found to have the disease, the cow was slaughtered and its muscle meat was sent to be sold in grocery stores. But its organs and nerve tissue were not used for human food. Although mad cow disease cannot be spread through muscle meat, the United States Department of Agriculture (USDA) quickly traced the meat and removed it from grocery stores.

Between 2004 and 2006, only two more cows in the United States were found to have mad cow disease. When tested, these cases were found to be different from the first case found in the United States. There is some disagreement about whether these two cases were mad cow disease.

What are the symptoms of vCJD?

Variant Creutzfeldt-Jakob disease (vCJD) causes the brain to become damaged over time. It is fatal. Symptoms include:

• Tingling, burning, or prickling in your face, hands, feet, and legs. But there are much more common illnesses that cause these same symptoms. Having tingling in parts of your body does not mean you have vCJD.
• Dementia.
• Psychotic behavior.
• Problems moving parts of the body. As the disease gets worse, a person is no longer able to walk.
• Coma.

If a person does eat nerve tissue from an infected cow, he or she may not feel sick right away. The time it takes for symptoms to occur after you're exposed to the disease is not known for sure, but experts think it is years.

How is vCJD diagnosed?

There is no single test to diagnose vCJD. Doctors may think that a person has vCJD based on where the person has lived and the person’s symptoms and past health. Imaging tests, such as an MRI, may be done to check for brain changes caused by vCJD.

Researchers are now trying to develop a blood test that looks for vCJD. But no blood test is available at this time.

A brain biopsy is the only way to confirm a diagnosis of vCJD.

How is vCJD treated?

There is no cure for vCJD. Treatment includes managing the symptoms that occur as the disease gets worse.

The following health organizations are tracking and studying mad cow disease and variant Creutzfeldt-Jakob disease (vCJD). Their Web sites contain the most up-to-date information about these diseases.

• U.S. Centers for Disease Control and Prevention (CDC) provides up-to-date information about mad cow disease and variant Creutzfeldt-Jakob disease (vCJD), including tracking, prevention, travel precautions, and food inspection. You can find information at www.cdc.gov/ncidod/diseases/submenus/sub_bse.htm.
• U.S. Department of Agriculture (USDA) provides information about mad cow disease, the safety of the meat supply in the United States, and infection control guidelines. You can find information at www.fsis.usda.gov.
• Health Canada answers frequently asked questions about mad cow disease and vCJD and provides information about infection control and food inspection. You can find information at www.phac-aspc.gc.ca/cjd-mcj/index.html.
• The World Health Organization (WHO) Web site offers information about mad cow disease and vCJD cases around the world and provides infection control guidelines. You can find information at www.who.int/csr/disease/bse/en.

Organizations

References

Citations

1. American Red Cross (2005). Blood donation eligibility guidelines. Available online: http://www.redcross.org/services/biomed/0,1082,0_557_,00.html#tra.

2. Canadian Blood Services (2005). Deferral policies for vCJD. Available online: http://www.bloodservices.ca/CentreApps/Internet/UW_V502_MainEngine.nsf/page/Deferral+Policies+for+vCJD?OpenDocument.

3. Manuelidis L, et al. (2007). Cells infected with scrapie and Creutzfeldt-Jakob disease agents produce intracellular 25-nm virus-like particles. Proceedings of the National Academy of Science, 104(6): 1965–1970.

Other Works Consulted

• González-Scarano F (2008). Transmissible spongiform encephalopathies section of Central nervous system diseases due to slow viruses and prions. In DC Dale, DD Federman, eds., ACP Medicine, section 11, chap. 17. Hamilton, ON: BC Decker.

• U.S. Centers for Disease Control (2007). Fact sheet: Variant Creutzfeldt-Jakob disease. Available online: http://www.cdc.gov/ncidod/dvrd/vcjd/factsheet_nvcjd.htm.