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From: TSS ()
Subject: Health officials waiting for test results on possible CJD cases
Date: September 16, 2005 at 6:39 am PST

The waiting game
Health officials waiting for test results on possible CJD cases
By Sandy Miller
Times-News writer

TWIN FALLS -- When it comes to the investigation into a possible cluster of people diagnosed with Creutzfeldt-Jakob Disease (CJD), it's now a waiting game.

State and local officials are waiting for test results on victims' brain tissue to come back from National Prion Disease Pathology Surveillance Center at Cleveland's Case Western Reserve University. The only way to absolutely confirm a case of CJD and its type is by examining brain tissue.

"We're just waiting for test results," said Cheryle Becker, an epidemiologist with South Central District Health on Thursday. "I really do believe it will take until December to get that last test result."

CJD is a fatal brain wasting disease carried by prions, an abnormal form of protein in the bloodstream. Prions cause folding of normal protein in the brain, leading to brain damage. Symptoms include dementia and other neurological signs. Its victims usually die within four or five months after onset of the disease, according to the Centers for Disease Control and Prevention.

Here's what we know so far:

* There have been eight cases of possible CJD reported since January. All of the victims have died. Brain tissue studies on one woman from Twin Falls County showed she didn't die of the disease. Normally, there is only one case of CJD per million. Between 1984 and 2004, Idaho averaged 1.2 cases a year, said Tom Shanahan, spokesman for the Idaho Department of Health and Welfare. There was one year during that period when they had three cases, he said.

* Of the remaining seven cases in the current cluster, three of the victims were women from Twin Falls County, one woman was from Minidoka County, one victim was a man from Elmore County, one victim was a woman from Benewah County in northern Idaho and one victim was a woman from Bear Lake County located in the southern corner of Idaho on the Utah border.

* All the victims were over the age of 60, except for Kathy Isenberg, 53, of Benewah County, who died Sept. 5 at Kootenai Medical Center in Coeur d'Alene.

* Initial test results on one Twin Falls County woman showed she did indeed die of a prion disease, but officials are still waiting for the final results to tell them which one. Officials are waiting on the test results from another south-central Idaho woman, the man from Elmore County and the woman from Benewah County

* Autopsies were not done on three of the possible victims -- two women from Twin Falls County and the woman from Bear Lake County. The two local women had already died before their doctors reported they'd suspected CJD as the possible cause. Health officials are now in the process of getting medical records from the neurologist who treated the Bear Lake County woman, Shanahan said. Health officials have pondered the possibility of exhuming the bodies, but it might be too late to find any useable tissue samples, Shanahan said. He added that they would also have to get permission from the women's families.

Becker, state epidemiologist Dr. Christine Hahn, and Chris Carter of the federal public health office in Idaho examined medical records and Becker conducted surveys with the local victims' families. The information was entered into a computer program to help them determine if they had anything in common.

"We did surveys on the five in this area and didn't really find any commonalities," Shanahan said. "We looked at diets, how long they've lived here, surgeries, work history."

All four local women still suspected of having had the disease commonly ate beef, chicken and pork, and two ate organ meat such as liver, Hahn told The Associated Press. 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.

The reason meat eating is a concern is that the variant of CJD is caused by eating meat from a cow with bovine spongiform encephalopathy -- commonly known as mad cow disease. So far, only two cattle in the United States have been confirmed to have had "mad cow" disease. The first case in 2003 was found in a dairy cow in Washington state that had been purchased from a farm in Canada. The second case was identified in a cow in Texas in 2004 and confirmed this year, according to the Federal Food and Drug Administration.

Health officials suspect the Idaho victims had the sporadic form of CJD and not the variant form. The average age of people who've died from sporadic CJD is 68. The average age of people who've died from the variant form of CJD is 28, according to the CDC. However, health officials won't know for sure if the victims died from CJD or what kind it was until the brain tissue tests are completed.

Shanahan said they'll wait until getting the test results back before doing surveys with the other families.

"Then we'll probably be looking at more medical records and looking to the CDC for more guidance because they have more experience at this than we do."

State and local officials are in charge of the investigation and the CDC does not plan to send anyone to Idaho at this time. However, Dr. Ermias Belay, an epidemiologist with the CDC, is assisting the investigation from the CDC's offices in Atlanta.

Two clusters of sporadic CJD deaths in recent years occurred in New Jersey, involving 12 deaths; and in Oregon, involving 14 deaths. Those clusters, however, unfolded over a period of 36 months and 24 months, and in population areas of 1.7 million and 3.4 million, respectively.

Times-News writer Sandy Miller can be reached at 735-3264 or by e-mail at

About prion diseases

* Prion diseases, or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect humans and animals.

* TSEs are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss and a failure to induce inflammatory response.

* The causative agent of TSEs is believe to be a prion. A prion is an abnormal, transmissible agent that is able to induce abnormal folding of normal cellular prion proteins in the brain, leading to brain damage and characteristic signs and symptoms of the disease.

* Prion diseases are usually rapidly progressive and always fatal.

* Human prion diseases: Creutzfeldt-Jakob Disease, Variant Creutzfeldt-Jakob Disease, Gerstmann-Straussler-Scheinker Syndrome, Fatal Familial Insomnia and Kuru.

* Animal prion diseases: Bovine Spongiform Encephalopathy, Scrapie, Chronic Wasting Disease, Transmissible Mink Encephalopathy, Feline Spongiform Encephalopathy and Ungulate Spongiform Encephalopathy.

Source: Centers for Disease Control and Prevention

Story published at on Friday, September 16, 2005


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