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From: TSS ()
Subject: Idaho Investigates Reports of Creutzfeldt-Jakob Disease
Date: October 26, 2005 at 3:54 pm PST

October 26, 2005

Idaho Investigates Reports of Creutzfeldt-Jakob Disease

The Idaho Department of Health and Welfare and South Central District Health are investigating four possible cases of Creutzfeldt-Jakob Disease (CJD) in southcentral Idaho. CJD is a degenerative brain disease caused by malformed proteins, called prions. All four of the possible cases are women ages 60 years or older who have died.

An autopsy and testing of brain tissue is the only method to diagnose CJD. Of the four victims in south-central Idaho, two have received autopsies. Test results from one of the two show the person died from a prion disease. Test results from the other autopsied person are pending. Originally, the Department and South Central District Health were investigating a fifth possible case, but autopsy results showed no prion disease in that person.

Over the last 20 years, Idaho has experienced an average of 1.2 deaths a year due to CJD. The Office of Epidemiology and Food Protection has been working with South Central District Health, healthcare providers and families to determine if any common links existed between the women.

Creutzfeldt-Jakob Disease Information 10/4/2005

Creutzfeldt-Jakob Disease is a degenerative brain disease that has been recognized since the 1920s. It is a human prion (infectious protein) disease that progresses rapidly and is always fatal, usually within one year from onset of illness.

CJD is not “Mad Cow disease” in people, which is caused by a different prion and is technically called variant CJD or vCJD. CJD is not transmissible from person-to-person through ordinary contact, but it can be transmitted from one person to another by contaminated instruments used for brain surgery or by transplant of infected tissue. There is no known environmental trigger for development of CJD. For more information on CJD, see and the links under “Where can I get more information about CJD?”

Creutzfeldt-Jakob Disease (CJD) in Idaho: Historical
CJD was not reportable in Idaho until 2004. Prior to that, information on the number of cases of CJD was based on death certificates. Among Idaho residents in the last 20 years, there have been 25 deaths reported on death certificates listing CJD as the cause.

The annual number of reported CJD deaths in Idaho during the last 20 years ranged from zero to three. CJD deaths were reported from 17 Idaho counties. From 1985 to 2004, only 10 of 25 (40%) of persons with CJD on their death certificate had received an autopsy. Autopsy is the only way to confirm the diagnosis of CJD.

Creutzfeldt-Jakob Disease (CJD) Deaths among Idaho Residents:
1985-2003, and 2004 Preliminary
Year Number of Deaths Counties
1985 0
1986 3 Bonneville, Gem, Washington
1987 0
1988 3 Ada, Canyon, Twin Falls
1989 2 Minidoka, Nez Perce
1990 0
1991 0
1992 0
1993 3 Bonner, Canyon, Minidoka
1994 2 Ada, Gooding
1995 2 Ada, Twin Falls
1996 2 Bannock, Canyon
1997 1 Payette
1998 1 Idaho
1999 1 Ada
2000 2 Canyon, Owyhee
2001 1 Elmore
2002 0
2003 1 Kootenai
2004 Preliminary 1
Total 1985-2003, and 2004 Preliminary


*Number of deaths reported on death certificate as CJD, 1985-2004 (2004 preliminary). Idaho resident deaths may have occurred in Idaho or out of state. ICD-9 code used from 1985-1998: 046.1; ICD-10 code used from 1999-2003: A81.0 (Creutzfeldt-Jakob Disease) and B94.8 (Sequelae of other specified infectious and parasitic diseases and Creutzfeldt-Jakob Disease is specified). Data for 2004 are preliminary and are based on records filed as of August 12, 2005.
Source: Bureau of Health Policy and Vital Statistics, Idaho Department of Health and Welfare (8/2005).

CJD in Idaho: 2005
South Central Idaho. In late July 2005, South Central District Health began investigating reports of possible CJD cases. Five possible cases reported between February and July 2005 have been under investigation by South Central District Health and the Idaho Department of Health and Welfare, Office of Epidemiology and Food Protection. All five patients are deceased. Autopsy results on one of the patients were not consistent with CJD and that patient is no longer considered a possible case; the other four cases are still being investigated.

The investigation includes a review of medical records and interviews with treating physicians. A survey for information on dietary habits, residence, travel, occupation, surgeries, and other life experiences also was conducted through interviews with family members.

South Central Idaho Investigation Findings
Of the four possible CJD cases still under investigation, all patients were White females, over 55 years of age (average 70.8 years). No common ethnic population of origin could be identified. The estimated time from onset of illness to death ranged from 1.4 to 10.6 months (average 4.3 months); however, in some cases, it was difficult for the family to remember exactly when the patient started having symptoms.

All patients lived in one of two counties in the South Central health district at the time of death; one patient also lived in a third Idaho county. The number of years of continuous residence in Idaho prior to death ranged from less than one year to 72 years (average 49 years). One patient lived for 18 months in Great Britain prior to 1980. No other travel to Europe was reported for any patient, and no travel in common to all four patients was found. Patients had no occupation or place of employment in common.

Eating habits of all the patients were reviewed. All four patients ate beef (four ate meat, two also ate organs), chicken, and pork. One had a diet that included deer meat, one ate sheep meat, and one ate goat meat. Family members did not believe any of the patients consumed elk, or the brain of any animal. No unusual foods reported to have been consumed were found in common. Patients had no other reported animal exposures in common.

No reported surgical procedures were common to all four patients, and no patients had neurologic surgery or transplants. The number of surgeries over a patient’s lifetime ranged from 1-to-3 (average two). No medications or supplements were found in common to all four patients. No patients had been injected with a growth hormone.

Autopsies are the only method to verify CJD as the cause of death. Of the five people who died from possible CJD, autopsies were conducted on three. Laboratory results provide an initial test result on whether the disease was a prion disease, which includes CJD, and may be followed by additional testing for more in-depth information on the type of prion disease. To date, initial autopsy results show a prion disease in two people, with tests indicating one person was not infected with a prion disease. Final lab results are pending on the two positive cases.

Additional reports of possible CJD
The Idaho local public health districts and State health department are monitoring additional reports of possible cases of CJD. With each report, medical records will be examined and interviews conducted with the physicians and families, if necessary. Local and state health officials investigate and evaluate each report. Four reports are currently under investigation. These include:
A possible case of CJD in an Elmore County man was reported to the Central District Health Department in August, but autopsy results indicate the person did not have a prion disease.
A woman in her 50s from Benewah who died also is being investigated by the Panhandle Health District. An autopsy was performed and initial lab tests indicate she had a prion disease. Additional tests are being conducted.
A report from Bear Lake County in southeastern Idaho of a woman over the age of 55 who is deceased. The case is being investigated by the Southeastern District Health Department. An autopsy was not performed on this person, and
A man over the age of 55 from Caribou County who died, but no autopsy was performed. Southeastern District Health Department is helping with this investigation, too.

Idaho probes series of suspected CJD cases
Amy L. Becker Staff Writer

Oct 25, 2005 (CIDRAP News) – The deaths of nine people in Idaho this year are being investigated as possible cases of Creutzfeldt-Jakob Disease (CJD), a rare, fatal brain-wasting illness.

The unusual number of possible cases has prompted widespread interest and concern. CJD, a disease involving infectious proteins called prions, progresses rapidly and is usually fatal within a year of onset. It differs from the human equivalent of mad cow disease, which is known as variant CJD (vCJD). CJD does not spread among people through ordinary contact, but it may be spread through contaminated surgical instruments or implants of infected tissue.

The usual incidence of CJD is about one case per million people annually. Idaho, with its 1.3 million people, typically has zero to three deaths a year, said Tom Machala, RN, MPH, division director of Communicable Disease and Prevention in Idaho's South Central District Health Department in Twin Falls.

The cases were suspected initially on the basis of signs and symptoms. In the first suspected case, there was no autopsy to confirm CJD, Machala said today. Three more probable CJD deaths were found within 3 weeks after that one.

"That's what prompted us to start an investigation," he said. A review of death records revealed another probable case, for a total of five cases from January through June, he added. All of these were in two counties in south-central Idaho.

Of those five cases, only two triggered full-blown autopsies, which confirmed CJD.

Four other possible CJD cases have occurred: two in eastern Idaho, one in northern Idaho, and one near Boise, Machala said.

An epidemiologist in Idaho is working with a physician from the Centers for Disease Control and Prevention (CDC) in Atlanta, Machala said. Their research includes a retrospective review of medical records and open-ended questionnaires for families based on World Health Organization (WHO) documents. A preliminary report of their findings should be done in December.

Not all of the cases will turn out to be CJD, and not all of the probable cases can be confirmed, Machala said. Autopsies must be done before embalming, which didn't happen in all the cases. Some pathologists were unwilling to conduct autopsies because they feared exposure to the disease agent, according to an Oct 17 Associated Press (AP) report.

Machala said health professionals have become more aware of signs and symptoms of CJD and are in a better position to draw on resources like the National Prion Disease Pathology Surveillance Center at Case Western Reserve University, which works with the CDC and can conduct autopsies appropriate for diagnosis of CJD.

The investigation under way in Idaho, though, won't answer some of the questions people may struggle with the most.

"It's a devastating disease," Machala said. "People are looking for ways to avoid it. That's the trouble: They haven't even figured out what causes it. What triggers it? Is it environmental factors? Is it genetic?"

The lengthy family interviews have included questions about eating habits, occupational exposures, surgeries, travel, and the like, but no smoking gun has emerged.

"One thing is very clear in Idaho—the number seems to be higher than the number reported in previous years," Dr. Ermias Belay, a CJD expert at the CDC, told the AP. "So far, the investigations have not found any evidence of any exposure that might be common among the cases."

Both CJD and vCJD are in the group of diseases called transmissible spongiform encephalopathies (TSEs), which can occur in cattle, sheep, buffalo, antelope, and goats, as well as humans.

See also:

Idaho Department of Health and Welfare news release

Idaho Department of Health CJD information

National Prion Disease Pathology Surveillance Center home page


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