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June 14, 2013 at 5:42 PM

Cluster of illness similar to mad cow disease suspected in B.C.

Authorities in the Fraser Valley area of British Columbia are investigating several suspected cases of Creutzfeldt-Jakob disease, a rare brain disorder that can be linked to mad cow disease — but officials say is not in this case.

The disease has apparently killed one person and hospitalized two others, according to the Associated Press. All three live in different cities.

Washington state officials said they had not been notified of the suspected cases.

“It would certainly be a concern, and we certainly do have measures to try to protect our livestock from things like that, but at the moment we haven’t received any official notification and typically we would,” said Hector Castro, a spokesman for the Department of Agriculture, adding that while Washington does import cattle from Canada, there are safeguards to ensure they’re healthy.

State Health Department spokesman Donn Moyer cautioned that early reports appeared to be very preliminary.

“There’s a lot of qualifiers in there,” he said.

Creutzfeldt-Jakob disease is the most common human prion disease, a rare condition that affects the brain and nervous system.

No Washington residents have ever been found to have the type of Creutzfeldt-Jakob disease (CJD) linked to cattle, Moyer said. However, seven to nine people a year in Washington are found to have the type of CJD known as “sporadic,” which is not linked to cattle, but occasionally does have genetic links.

Very rarely, Moyer said, CJD has been spread through contaminated neurosurgical instruments or human-derived grafts.

Both the sporadic type of CJD and the type linked to cattle, known as variant CJD and sometimes referred to as “mad cow disease,” can only be definitively diagnosed at autopsy through testing of brain tissue.

Fraser Health Authority chief medical officer Paul Van Buynder told the AP that in this case, “it’s not mad cow disease. It has nothing to do with the food chain.”

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