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From: TSS ()
Subject: Elmore County man tests negative for CJD, Belay says Idaho cases could be a 'statistical fluke'
Date: October 14, 2005 at 12:55 pm PST

Elmore County man tests negative for CJD

Associated Press writer Friday, October 14, 2005

BOISE, Idaho -- Tests show that an Elmore County man, presumed to have died from classic Creutzfeldt-Jakob Disease, did not have the illness, state health officials said Thursday. The results reduce to seven the possible CJD cases in Idaho this year.

The man, whose name has not been released by authorities, had been believed to be the seventh Idaho resident to contract the rare brain-wasting disease this year.

But tests of brain tissue, conducted after he died last month, show he did not have CJD nor any other disease related to prions, or malformed proteins, Idaho Department of Health and Welfare spokesman Tom Shanahan said.

"The tests don't show what he did have -- they just test for prion diseases," Shanahan said. "But it could have been a series of small strokes, could be dementia. There's just no way for us to know."

State and federal health officials have been paying close attention to Idaho's multiple cases of suspected CJD this year -- the first full year of mandatory reporting.

Authorities have investigated nine suspected cases in the state, which had previously averaged slightly more than one reported case a year. The only way to conclusively diagnose CJD is through autopsy, according to the federal Centers for Disease Control and Prevention and the World Health Organization.

All nine of Idaho's suspected CJD victims are dead. Three tested positive for prion disease and two tested negative. Autopsies were not performed on four suspected victims -- due to delayed reporting or to family objections.

Classic CJD, also known as sporadic CJD, has no known cause or cure, but is not believed to be linked to consumption of mad-cow-tainted beef. Beef-related cases are classified as variant CJD, which has killed at least 180 people in the United Kingdom and continental Europe since the 1990s.

CJD is not often confused with other diseases, said Dr. Ermias Belay, a CJD expert with the federal Centers for Disease Control and Prevention.

"Most of the time Alzheimer's patients have memory loss and dementia but they walk around. They get lost, and the disease lasts for years," Belay said.

"That's not true of CJD, where in addition to the dementia they have all kinds of deficits. They're not able to stand up and walk properly. Very soon they become bedridden, and usually die within six months."

Occasionally patients are misdiagnosed with CJD when they suffer fast-progressing dementia or a neurological deficit disease.

"In experienced hands, this could be diagnosed with some confidence. But obviously a definitive diagnosis requires an autopsy," Belay said.

The CJD diseases share similar characteristics: One malformed prion triggers another prion to distort, causing a progressive Swiss-cheese effect in the brain.

Ultimately, the destruction caused by classic CJD hijacks the body's ability to control movement and causes dementia. There is no treatment, and the disease is always fatal.

State health officials say investigations into suspected clusters of the disease may one day offer clues about what causes classic CJD.

But Idaho's cases may turn out not to be a cluster at all, said Belay.

"There have been other clusters reported around the nation, but those clusters did not pan out most of the time," he said.

Idaho doctors first were required to report cases of the disease in mid-2004, and that may be a factor in the increase, Belay said, though similar increases have not shown up as other states go to mandatory CJD reporting.

"The one thing that I found interesting is Idaho, because they have an increased number of CJD cases in 2005 compared to the past. But whether or not that is something isolated to Idaho would have to be addressed by the investigation," Belay said.

The increased number of cases here may also simply be a matter of chance.

"Statistically speaking, if you have 280 cases in the United States in a given year, you would not expect a uniform distribution," Belay said. "So this could potentially be a statistical fluke."


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