Mad cow disease is still here, it turns out.  But is it really this rare?  Is it possible that some cases have been misdiagnosed?

CDC confirms 4th U.S. case of mad cow disease after Texas man dies

by Ryan Parker
Los Angeles Times
June 6, 2014

Mad cow disease -- the fourth confirmed case in the U.S. -- is responsible for the death of a Texas man, the Centers for Disease Control and Prevention said Friday.

The variant CJD, as it’s medically known, was confirmed by experts after a sample of the man’s brain tissue was analyzed.

No specifics on the victim or when he died were released.

“The history of this fourth patient, including extensive travel to Europe and the Middle East, supports the likelihood that infection occurred outside the United States,” the CDC said in a statement.

The disease is a rare, degenerative fatal brain disorder in humans that is believed to be caused by eating the meat of cows with the disease bovine spongiform encephalopathy, according to the CDC.
The disease in humans is more prevalent in Europe. The majority are in Britain, which has had 177 confirmed cases since the disease was discovered there in 1996, and France, with 27, the CDC reports.