Mad Cow Disease: Where Did it Come From and Am I At Risk?

· Mad Cow Disease, also know as Prion disease or acquired Creutzfeldt-Jakob Disease, results after eating meat infected with a protein called the Prion protein. Mad Cow Disease originated in England where a similar Prion protein that had been found in sheep, infected cattle.

· The cow brain is similar to the human brain and when this mutation took place in cattle it allowed the protein the ability to infect humans and cause disease.

· Most people believe that Mad Cow Disease originated in England where sheep carcasses where part of the cattle diet. In order to save money during the energy crisis of the 1970s, ranchers stopped cooking the sheep and were giving their cattle raw sheep carcasses and brain matter. Once the cattle had eaten the raw meat, the mutation took place.
Therefore, most cases of Mad Cow have occurred in England, although it has spread to cattle in the United States with domestic cases being reported here.

· After ingestion of contaminated meat, the infecting Prior protein enters the brain and changes the shape of a similar, naturally occurring protein in the human brain cells and causes irreversible damage and distraction to brain cells.

· Infected individuals deteriorate rapidly, typically first developing psychiatric symptoms followed by neurologic impairment and rapidly progressive muscle dysfunction, tremor, loss of the ability to speak and ultimately death.

· There is currently no cure. A potential cure would have to result from a completely novel approach to fighting infections in that this is an infection of a protein and very different from the traditional infections we see from bacteria, virus, fungus or parasites.

· Almost all cases have occurred in England but there is a concern that we may see more cases in the United States with recent diagnosis of infected domestic cattle. We may all be at risk, although arguably a very low risk compared to other illnesses.

November 24, 2008

Posted on November 24, 2008

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