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Idaho confirms rare Creutzfeldt-Jakob case

Idaho officials confirmed one case of naturally occurring Creutzfeldt-Jakob disease and are investigating five other suspected cases, but said none are believed to be caused by eating infected animals.
/ Source: Reuters

Idaho officials said Friday an initial test has indicated one case of naturally occurring Creutzfeldt-Jakob disease and they are investigating five other suspected cases, but said none are believed to be caused by eating infected animals.

Tom Shanahan, a spokesman for the Idaho Department of Health and Welfare, said five of the cases involve people who have already died, lived in neighboring counties and were over the age of 60. The sixth case centered on a man, also over the age of 60, who lived 90 miles away and was still alive.

He said officials expected to receive the results of a more in-depth second test by the end of next week that would rule out any possibility the disease could be anything but the kind that occurs naturally.

CJD is a rare brain-wasting disease in humans that usually affects older people in their 60s or 70s. It is not the same as the human form of mad cow disease, which is known as variant Creutzfeldt-Jakob Disease and is linked to eating beef from infected cattle.

“There are no indicators that it was anything but classic CJD,” Shanahan said. “There were no signs whatsoever to indicate it was variant.”

Naturally occurring CJD is found at a rate of about one case per 1 million population annually, according to the U.S. Centers for Disease Control and Prevention.

Yet in a state with only 1.4 million people the fact that Idaho has so many suspected cases of the rare disease has sparked concern.

Shanahan said researchers at Case Western Reserve confirmed that brain tissue from one woman showed CJD caused her death and that the state was waiting for results from two other tests. All the deaths occurred this year, beginning in February, he said.

“We actually are real concerned because we have never had more than three cases in a year and they are in one geographic area,” Shanahan said.

The sixth suspected case involves an elderly man who has not yet died, he said. The disease cannot be confirmed until a brain sample is collected and analyzed after death.

Searching for a cause
Investigators so far have not been able to pinpoint anything that may have caused the disease and are looking at such factors as the victims’ diets.

The CDC is monitoring the Idaho situation but has not been asked by the state to participate in a formal probe, said agency spokesman David Daigle.

The U.S. meat industry also said the outbreak of cases had no connection to mad cow disease or bovine spongiform encephalopathy and reassured consumers that meat was safe to eat.

“We are confident in the accuracy of the assessment of the Idaho Health Department that these cases of CJD have absolutely nothing to do with BSE,” said American Meat Institute President J. Patrick Boyle. “Beef has been, and remains, safe to eat.

In recent years, the United States has reported fewer than 300 cases of CJD each year, according to the CDC.

“It is not related as far as we know to eating any infected beef,” Shanahan said.

Last year, health officials found a cluster of 13 deaths due to naturally occurring CJD in New Jersey between 1988 and 1992. All of those people either attended or worked at a race track in Cherry Hill, New Jersey, according to investigators.

The variant form of CJD is the human equivalent of bovine spongiform encephalopathy, or mad cow disease, and is contracted by people who eat beef from infected cattle.

The naturally occurring strain of CJD has no known cause. However, both types are incurable diseases involving a malformed protein, or prion, that kills brain cells.

The United States has confirmed two cases of mad cow disease, one in a Washington state dairy animal in 2003 and the other in a Texas beef cow this year.