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Understanding Mad Cow Disease: Causes, Symptoms, and Prevention

Abhijit Bhattacharyya | MD, PhD, MBA

Medically reviewed by Abhijit Bhattacharyya | MD, PhD, MBA, Tufts University School of Medicine - Miami, Florida on January 16th, 2024.

Key Takeaways

  • Bovine spongiform encephalopathy (BSE) and its human variant Creutzfeldt-Jakob disease (vCJD) are caused by misfolded prion proteins that create sponge-like holes in brain tissue over months to years.

  • Human cases peaked during the UK outbreak in the 1990s with 178 deaths, but only 4 cases have been reported in the US, all linked to travel or blood transfusions from affected regions.

  • Symptoms typically appear 10-15 years after exposure and progress rapidly, including psychiatric changes, painful sensations, loss of coordination, and dementia leading to death within 13 months of onset.

  • Ground beef and processed meats carry higher risk than whole muscle cuts because they may contain central nervous system tissue where prions concentrate at levels 100,000 times higher than in muscle.

  • Countries now ban feeding cattle bone meal from ruminants and remove high-risk tissues (brain, spinal cord, eyes) from the food supply, reducing transmission risk by over 99% since implementation.

Mad cow disease, also known as bovine spongiform encephalopathy (BSE), is a neurological disease that affects cattle. It is caused by an abnormal version of a protein called a prion, which destroys nervous system tissue in the brain and spinal cord. The disease gets worse over time and is ultimately fatal.

The first cases of BSE were reported in the United Kingdom in the 1970s, with a major outbreak peaking in 1993. Since then, strict control measures have been put in place to prevent the spread of the disease, and cases have significantly decreased worldwide.

How Does Mad Cow Disease Affect Humans?

In humans, the disease caused by consuming BSE-infected beef is called variant Creutzfeldt-Jakob disease (vCJD). People can contract vCJD by eating beef products containing central nervous system tissue, such as brain and spinal cord, from infected cattle. However, the risk of getting vCJD from eating beef is extremely low, especially in countries with strict control measures.

Understanding Mad Cow Disease: Causes, Symptoms, and Prevention

It is important to note that vCJD is different from classic or sporadic CJD, which has no known cause and occurs worldwide at a rate of one to two cases per million people annually. Classic CJD is not linked to consuming BSE-infected cattle.

Symptoms of vCJD

The symptoms of vCJD can be divided into early and advanced stages:

Early symptoms:

  • Severe depression

  • Withdrawing from family and friends

  • Anxiety

  • Irritability

  • Insomnia

Advanced symptoms:

  • Loss of coordination (trouble walking, speaking, and balance)

  • Muscle twitches or spasms

  • Incontinence and loss of bowel control

  • Vision loss or blindness

  • Memory loss and confusion

  • Loss of appetite

vCJD is fatal, usually within 13 months of the onset of symptoms. In the final stages, patients require constant care and often die from complications such as pneumonia or respiratory failure.

Diagnosing and Treating vCJD

Diagnosing vCJD is challenging, as the only definitive way to confirm the disease is by examining brain tissue, usually after death. However, doctors may suspect vCJD based on a combination of symptoms, medical history, and various tests, including:

  • MRI scans of the brain

  • EEG recordings of brain activity

  • Lumbar puncture to test cerebrospinal fluid

  • Blood tests

  • Tonsil biopsy to look for prions found in vCJD

  • Genetic tests to rule out hereditary prion diseases

Currently, there is no cure or specific treatment for vCJD. Treatment focuses on managing symptoms and providing comfort to the patient. Medications such as antidepressants, sedatives, and painkillers may be used to alleviate vCJD-related symptoms.

Preventing Mad Cow Disease and vCJD

The most effective way to prevent vCJD is by controlling and preventing BSE in cattle. Countries have implemented various measures to minimize the risk of BSE transmission, including:

  • Testing cattle for BSE

  • Culling sick animals

  • Banning high-risk materials in animal and human food products

To further reduce the risk of contracting vCJD, individuals can:

  • Avoid consuming beef in countries where BSE is present

  • Choose solid cuts of beef, such as steaks, over ground meat products like burgers or sausages

  • Be aware of potential risks associated with receiving blood transfusions from donors who have spent significant time in countries with higher BSE prevalence

It is important to note that milk and milk products are not believed to pose any risk for transmitting vCJD.

Conclusion

Although vCJD is a serious condition, the risk of contracting vCJD from consuming beef is extremely low, especially in countries with strict control measures in place. By understanding the causes, symptoms, and prevention methods, individuals can make informed decisions about their health and minimize their risk of exposure to these diseases.

For more information on mad cow disease and vCJD, please visit:

The Bottom Line

This extremely rare but fatal condition is now well-controlled through strict food safety regulations, with your risk of exposure being virtually zero when choosing quality beef from regulated sources. Focus on whole cuts over processed meats if you want maximum protection. If you're experiencing unexplained neurological symptoms or have concerns about past exposure, Doctronic can help connect you with appropriate medical evaluation quickly.

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