10/17/2005

9 Cases of Brain-Wasting Disease in Idaho - Yahoo! News

BOISE, Idaho - From the moment Joan Kingsford first saw her husband stagger in his welding shop, she wanted two things: His recovery and to know what made him sick.
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She got neither. Alvin Kingsford, 72, died recently of suspected sporadic
Creutzfeldt-Jakob disease, the fatal brain-wasting illness. The disease can be conclusively diagnosed only with an autopsy, which did not take place.

State and federal health officials are trying to get to the bottom of nine reported cases of suspected sporadic CJD in Idaho this year. Sporadic, or naturally occurring, CJD differs from the permutation dubbed variant CJD, which is caused by eating mad-cow-tainted beef and has killed at least 180 people in the United Kingdom and continental Europe since the 1990s.

"One thing is very clear in Idaho — the number seems to be higher than the number reported in previous years," said Dr. Ermias Belay, a CJD expert with the federal Centers for Disease Control and Prevention. "So far, the investigations have not found any evidence of any exposure that might be common among the cases."

Normally, sporadic CJD only strikes about one person in a million each year, with an average of just 300 cases per year in the United States, or just over one case a year in Idaho. Over the past two decades, the most cases reported in Idaho in a single year has been three.

Until this year.

Of the nine suspected cases reported so far in 2005, three tested positive for an infectious disease of the nervous system, though more tests are pending to determine if the fatal illness was in fact sporadic CJD. Four apparent victims were buried without autopsies. Two suspected cases tested negative.

Still, federal and state health officials are stopping just short of calling the Idaho cases a "cluster," waiting for final test results from the victims who got autopsies.

The best tool of investigators to pin down the diagnosis — the autopsy — is sometimes hard to get, said Tom Shanahan with the Idaho Department of Health and Welfare.

Pathologists are often reluctant to perform the procedures, the cost of an autopsy can be high and some families are reluctant to give their consent, officials say.

Joan Kingsford wanted an autopsy done on her husband, but no mortician in the area would agree to handle Alvin's body after his brain cavity had been opened. They feared they would catch the rare disease, Kingsford said.

Ultimately, she opted to skip the autopsy and have a traditional funeral service.

CJD is transmitted through a malformed prion found primarily in the brain and spinal fluid of those infected, Belay said. Standard sterilization procedures don't eliminate the risk of infection; instead equipment must be soaked in a chemical solution for more than an hour and then heated, according to the
World Health Organization.

9 Cases of Brain-Wasting Disease in Idaho - Yahoo! News

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