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What is Mad Cow Disease and how does it affect humans?

What is "Mad Cow Disease" (Bovine Spongiform Encephalopathy)?

Mad Cow Disease is the layperson's name for Bovine Spongiform Encephalopathy (BSE), a transmissible, slowly progressive, degenerative, fatal disease affecting the central nervous system of adult cattle. 

Does Mad Cow Disease affect humans?

Bovine spongiform encephalopathy  is a disease that affects cattle. However, there is a disease similar to BSE called variant Creutzfeldt-Jakob Disease, which is found in humans.  There have been a small number of cases of  variant Creutzfeldt-Jakob Disease reported, primarily in the United Kingdom, occurring in people who consumed beef that may have been contaminated. There is strong scientific evidence that the agent that causes BSE in cattle is the agent that causes variant Creutzfeldt-Jakob Disease in people. The one reported case of variant Creutzfeldt-Jakob Disease in the United States was from a young women that contracted the disease while residing in the UK. The symptoms appeared years later after the young woman moved to the U.S.

The disease,variant Creutzfeldt-Jakob Disease, which primarily affects younger persons, is very hard to diagnose until the disease has nearly run its course. In its early stages, the disease may manifest itself through neurologic symptoms, but it is not until the latter stages of the disease that brain abnormalities detectable by x-ray or MRI can be seen.

The incubation period of variant CJD appears to be several years or longer. Initial symptoms may be psychiatric, similar to depression or (less often) psychosis. Patients may complain of unusual sensory symptoms, such as "stickiness" of the skin. Later symptoms include unsteady gait, involuntary movements, dementia, and mutism, progressing inevitably to death within 1-2 years. There is no treatment.

Variant CJD can only be acquired by eating beef or beef products; i.e if you do not consume beef or beef products while visiting a country which has reported "mad cow disease", you cannot become infected. There is no evidence of any risk from pork, lamb, milk or milk products. The Centers for Disease Control suggests that people can reduce their risk by either abstaining from beef completely or eating only solid pieces of muscle meat, such as steak, rather than products like sausage or chopped meat that might be contaminated during processing. Cooking, drying, or freezing does not inactivate the agent that causes new variant CJD or prevent its transmission.

When and how did BSE in cattle occur?

BSE has been of great concern since 1986, when it was first reported among cattle in the United Kingdom.  At its peak, in January 1993, almost 1,000 new cases per week were identified. The outbreak in the United Kingdom may have started from the feeding of contaminated sheep meat-and-bone meal to cattle. The nature of the transmissible agent in BSE is not known.Why or how this substance changes to become disease-producing is still unknown. 

Milk and milk products from cows are not believed to pose any risk for transmitting the BSE agent because experiments have shown that milk from BSE-infected cows has not caused BSE in cows or other test animals

Is Mad Cow Disease  occurring in the United States?
On December 23, 2003, the U.S. Department of Agriculture announced a diagnosis of Mad Cow Disease in an adult Holstein cow from Washington State. Samples were taken from the cow on December 9 as part of USDA's Mad Cow Disease surveillance program.

posted 12-30-03 on


As a reminder, this information should not be relied on as medical advice and is not intended to replace the advice of your child’s pediatrician. Please read our full disclaimer.

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