State health officials are looking into a report of a North Idaho woman who may be suffering from Creutzfeldt-Jacob disease.
It’s one of eight such reports the Idaho Department of Health and Welfare has investigated this year, but none of them has been confirmed to be the “mad cow” form of the disease.
In fact, just one person in the United States ever has been confirmed to have the variant form of the disease, which is the kind acquired from cattle with spongiform encephalopathy, or “mad cow” disease.
The case was recorded in 2002 and involved a Florida woman who had moved to the U.S. from the United Kingdom, where cattle suffered an epidemic of mad cow disease in the 1980s. The first cases of humans contracting the disease from cows were reported in the UK in 1996.
The last confirmed case of Creutzfelt-Jacob disease, also known as CJD, in North Idaho was in 2003. The patient, Peter Putnam, of Twin Lakes, Idaho, died that autumn and an autopsy showed he suffered from the more common form of the disease.
An autopsy is the only way to confirm a diagnosis of CJD.
“It’s a difficult one for doctors to diagnose,” said Tom Shanahan, spokesman for the Idaho Department of Health and Welfare. “There’s no definitive test to tell you that this is CJD.”
The most recent report came from a doctor in North Idaho, and the state epidemiologist is in the initial stages of determining if it even qualifies as a suspected case.
“This is the only report that’s being investigated in our area at this time,” said Susan Cuff, Panhandle Health District spokeswoman. “The report hasn’t even been classified as a suspected case.”
Other possible diagnoses still must be ruled out, such as Alzheimer’s, stroke and other conditions that affect the brain.
CJD occurs when proteins in the brain, known as prions, change shape and attack the brain. The illness is incurable and degeneration is relatively rapid, followed by death, health officials said.
Very few Idahoans have died from CJD, however. The state had one death attributed to CJD in 2004, one in 2003 and none in 2002, according to Tom Shanahan, Idaho Department of Health and Welfare spokesman.
But due to a new state law that requires health professionals to report suspected cases of the disease, the agency has found itself investigating eight cases this year.
The recent North Idaho report and another report in southeast Idaho are in the very preliminary stages of investigation. Six other cases were considered “suspected” cases of CJD, Shanahan said.
Of those, five of the patients have died, but the state did not find out about two of the deaths in time to request an autopsy.
Of the remaining three, two autopsies are complete and the results showed that one had CJD and one did not. The state is still awaiting final test results in the confirmed case to see what form of CJD the patient had, Shanahan said.
Of the mad cow-related cases in Great Britain, the patients tended to be younger, in their late 20s. But the more common form tends to attack older adults, Shanahan said. The possible case in North Idaho involves a woman older than 50, he said.
CJD is not contagious, and is only of public health concern if the patient is believed to have eaten contaminated beef. So far, only two cattle have been found to have the fatal brain wasting disease in the United States, including one in central Washington.
The fact that the state is looking into reports of CJD is nothing to be alarmed about, Cuff said.
“This is not a public health threat,” she said.
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