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From: TSS ()
Subject: CJD USA Information (CJD) in Idaho: Historical 9/27/2005
Date: September 29, 2005 at 12:45 pm PST

----- Original Message -----
From: "Terry S. Singeltary Sr."
Sent: Thursday, September 29, 2005 2:46 PM
Subject: CJD USA Information (CJD) in Idaho: Historical 9/27/2005

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CJD USA Information (CJD) in Idaho: Historical 9/27/2005
Thu Sep 29, 2005 14:43

September 29, 2005

Creutzfeldt-Jakob Disease Information 9/27/2005

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 transmissilbe 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. 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 Dept. 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 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 earlier in life. 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. Three reports are currently under investigation. A possible case of CJD was reported to the Central District Health Department in August. The Central District Health Department is investigating this report. The patient is from Elmore County, male, over 55 years old, and is deceased. An autopsy was performed and we are waiting for lab results.

A report of 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. In addition, a report from southeastern Idaho about a deceased patient is being investigated by the Southeastern District Health Department. An autopsy was not performed on this person.

For frequently asked questions about CJD, please click here.
For additional resources about CJD, click here.
For the Idaho Disease Bulletin about CJD, click here.


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