Few links found in suspected CJD cases

BOISE – State health officials say another person — an Elmore County man — has died of suspected Creutzfeldt-Jakob disease.

Though health investigators continue to sift through a lifetime’s worth of details on the five people who have died of suspected Creutzfeldt-Jakob disease, so far they’ve found few links.

“My gut feeling is that we will probably not find a link,” said Dr. Christine Hahn, the state epidemiologist for the Idaho Department of Health and Welfare.

National attention has been focused on the department’s investigation since five women — all living in Twin Falls County or nearby Minidoka County — were suspected of dying of the rare but fatal brain-wasting illness this year. Tests have since shown that at least one of those cases was not CJD, but the state is now also investigating the case of the Elmore County man. Authorities are saying little about him to protect his family’s privacy.

None of the cases has been confirmed as CJD so far, partly because the only way to confirm a case is by examining brain tissue and two of the original four women were buried without autopsies. Authorities are still waiting for test results on the others.

Besides those five deaths, a doctor in Bear Lake County and a doctor in the Coeur d’Alene region have reported two more possible CJD cases. State health officials are reviewing those cases and have yet to say whether they consider them to be suspected CJD.

Still, even if only half the cases prove to be CJD, it would mark a local uptick in a disease that normally strikes about one in one million people worldwide.

“Normally, Idaho would have between zero and three cases in a year, so it’s possible that when the dust settles we’ll learn we didn’t have a real cluster,” Hahn said. “But we would like to know more, so we’re investigating.”

Creutzfeldt-Jakob kills by causing normal brain prions — or proteins — to fold, leading to brain damage. The Idaho cases are believed to be from the sporadic form of the disease, not the variant type that can result from exposure to mad cow disease, or the familial type that can run in families.

Scientists don’t know what causes sporadic CJD, Hahn said. The state’s investigation could eventually prove useful in discovering a cause and a cure, she said. The results will be forwarded to the U.S. Centers for Disease Control and Prevention for analysis.

“You could take two strangers and sit them side by side and by asking random questions, find 100 things that they have in common,” Hahn said. “So we have to focus on asking questions for a reason. We’ve interviewed the families of victims about meat consumption, travel habits, unusual animal exposures.”

What they found, Hahn said, was fairly typical of most Idaho residents.

All four women originally suspected of having the disease commonly ate beef, chicken and pork, and two ate organ meat such as liver. None of the suspected victims was known to eat animal brains — brain matter can carry CJD-infected prions — and none had any odd dietary habits. Nor had any of the victims undergone neurological or brain surgery, which has also been identified as a possible source of CJD infection.

“The investigation will continue until we have final results from the lab on the remaining tests, and that will probably be two or three months,” Hahn said. “At that point, we might close the investigation.”

This is the first year Idaho doctors have been required to report suspected CJD deaths to the state and that, combined with increasing public awareness about the disease, may be partly responsible for the apparent upswing in cases.

“This is a disease where a physician has no way of knowing if it’s multiple strokes, Alzheimer’s, dementia or something else,” Hahn said. “The best scenario is if doctors do begin requesting more autopsies for suspected CJD, because that may further the science of the disease.”