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From: TSS ()
Date: August 18, 2005 at 6:45 am PST

TSS 2005

Officials investigate sixth possible case of CJD
Elmore County man receiving treatment
By Sandy Miller
Times-News writer

TWIN FALLS -- Idaho health officials are investigating a sixth possible case of Creutzfeldt-Jakob disease, this time a man in Elmore County, according to the Idaho Department of Health and Welfare.

The man is over the age of 60 and is currently being treated for the neurological disease. He is the sixth person to be diagnosed with CJD since January in southern Idaho.

"We are monitoring it and he is receiving treatment," said Tom Shanahan, a spokesman for the Idaho Department of Health and Welfare on Wednesday.

Shanahan said that if the man dies, health officials will request an autopsy.

Five women, four from Twin Falls County and one from Minidoka County, have died after being diagnosed with CJD. The fifth woman, Marjorie Skinner of Buhl, died almost two weeks ago.

All five of the women were between the ages of 60 and 83 and given their ages, Idaho health officials suspect the women had sporadic CJD and not the variant form of CJD that people get when they eat meat from a cow with bovine spongiform encephalopathy -- commonly known as mad cow disease. 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 Centers for Disease Control and Prevention.

However, the only way to determine the type of CJD is by examining brain tissue. Autopsies were performed on three of the women and their brain tissue has been sent to the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland to be studied, Shanahan said. Two of the women died before their CJD was reported by their physicians and autopsies were not performed, Shanahan said.

"We learned about two of them after death had occurred," he said.

Temporary rules were in effect last year, but it was only this year that CJD officially became a reportable disease in Idaho.

Health officials have already received the preliminary test results on brain tissue of one of the women, which revealed that she did indeed die of a prion disease. Officials will have to wait another week for the final results on the woman, Shanahan said.

"Initially, it looks like sporadic, but we won't know for sure for another week or so," he said. "There's always a possibility of a variant."

CJD is a fatal 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 CDC.

The unusually high number of cases has drawn attention from state health officials as well as the CDC. Normally, the disease infects just one person per 1 million people worldwide a year. In Idaho, there are about three cases of CJD annually, and in recent years, the United States has reported fewer than 300 cases of CJD a year, according to the CDC.

State epidemiologist Dr. Christine Hahn, along with Chris Carter of the federal public health office in Idaho and Cheryle Becker, an epidemiologist with South Central District Health, are involved in the investigation. They'll look at medical records and Becker is conducting surveys with the victims' families, looking for anything the six people might have had in common.

"I have completed all but one so we're close to being done," Becker said. "At this point, we haven't found anything that would give us clues. We're waiting to get the final interview and then we'll be comparing all the information we have."

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.

"They are a great resource," Shanahan said. "The disease has been around a while and we're still learning."

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 believed 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

The investigation

Health officials are investigating six cases of people in southern Idaho who have been diagnosed with Creutzfeldt-Jakob disease since January. Five women -- four from Twin Falls County and one from Minidoka County -- have died, and an Elmore County man is now being treated for the neurological disease. In addition to studying the six victims' medical histories, health officials are conducting surveys with the families to see if there is anything the six people had in common, said Tom Shanahan, spokesman for the Idaho Department of Health and Welfare. The surveys include a wide range of questions.

"It would be nice if we had a path to go down, but we don't, so we have to explore all the possibilities," Shanahan said. "We're looking at pretty much everything."

Shanahan said the surveys include questions about:

* Ethnicity

* Place of birth

* Travel history

* Family history of neurological disorders

* Medical history, including surgeries, childbirth, blood transfusions, whether tissue was ever typed, radiation -- particularly radiation to the head or spinal cord -- and any other medical events.

* Dietary habits -- if they ate beef, wild game, pork, chicken and the frequency in which they ate those foods.

* Occupational history, type of work and location.

* Exposure to animal fluids. For instance, a butcher or hunter would have exposure to animal fluids.

* Hobbies.

* Families also are asked if they have any other exposure concerns.

"A lot of the families have thoughts or other avenues that we haven't thought about and should be exploring," Shanahan said.

Source: Idaho Department of Health and Welfare


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