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Human 'mad cow' case found in U.S.

April 19, 2002 Atlanta Journal and Constitution by M.A.J. Mckenna
A Florida woman has developed the first case in the United States of the human form of "mad cow" disease, the Centers for Disease Control and Prevention said Thursday.

The 22-year-old, whom the CDC would not identify, lives in Florida with her family, but was born and reared in England, where she presumably contracted the disease.

She was diagnosed by British health authorities as having a probable case of "new-variant Creutzfeldt-Jakob disease," the human illness linked to mad cow disease. There have been only 125 cases of the new disease worldwide, 117 of them in the United Kingdom.

"She resided and was raised in the United Kingdom during the period when the [mad cow] epidemic was at its peak," said Dr. Stephen Ostroff of the Atlanta-based CDC. "There is every reason to believe that her exposure and her illness occurred in the United Kingdom and not in the United States."

Since 1987, when authorities began to raise the alarm about mad cow disease, almost 182,000 cases of the brain-destroying disease have been found in British cattle. The worst years of the epidemic were 1990-95. The human form of the disease first emerged in 1994.

Unlike classic Creutzfeldt-Jakob disease, which occurs about 300 times a year among the elderly in the United States, the new disease attacked people in their 20s. It begins with psychiatric symptoms, and then causes movement disorders and dementia that grow worse over six months or more.

It is always fatal. There is still no known treatment. Final diagnosis can be confirmed only by autopsy.

To date, authorities said Thursday, all cases of new-variant Creutzfeldt-Jakob disease have originated in countries where there were livestock cases. There has never been a confirmed case of mad cow disease in livestock in the United States. Since the epidemic began in England, federal agencies have aggressively policed imports of cattle and cattle feed from countries linked to the disease.

The disease is believed to pass to humans via a little-understood "prion" organism when they consume beef or cattle byproducts. Its incubation period, the time lag betwen infection and symptoms, may be a decade or more.

In an extraordinary evening teleconference called Thursday by the CDC and featuring representatives of the departments of Health and Human Services, Agriculture, and the Food and Drug Administration, authorities emphasized that the woman posed no threat to anyone else.

So far as is known, the new-variant is not transmissible person to person. The woman had not been a blood donor, and her family is taking no special precautions, Ostroff said.

The CDC declined to provide any identifying details about the woman, including her current condition. She began showing symptoms in Florida, returned to England to be diagnosed, and is now back in Florida, Ostroff said, adding that British health authorities notified the CDC of her case on Wednesday.

"We have been anticipating for quite some time we would find such a case: There are large numbers of persons who were born and raised in Great Britain who live in the United States, and there are large numbers of Americans who have lived there for extended periods of time," Ostroff said. "This is not an unexpected finding."

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