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 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 http://www.cdc.gov/ncidod/dvrd/cjd/ and the links under “Where can I get more information about CJD?”
Creutzfeldt-Jakob Disease (CJD) in Idaho: 1985–2008
CJD was not added to the list of legally reportable diseases in Idaho until 2004. (For more information about reportable diseases please see the Idaho Reportable Disease Summary website.) Prior to that, information on the number of cases of CJD was based only on information reported on death certificates. The annual number of reported CJD deaths in Idaho in 1985—2008 ranged from zero to seven. CJD deaths were reported from 21 Idaho counties. From 1985 to 2008, only 15 (44%) of 34 of persons with CJD on their death certificate had received an autopsy. Although a clinical diagnosis of CJD based on symptoms and physical examination is reportable, autopsy is the only way to confirm the diagnosis.
Creutzfeldt-Jakob Disease (CJD) Deaths among Idaho Residents:
1985—2007, and 2008 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
|
1
|
Franklin
|
|
2005
|
7
|
Bear Lake, Benewah, Caribou, Minidoka, Twin Falls (3)
|
|
2006
|
0
|
|
|
2007
|
0
|
|
|
2008 (Preliminary)
|
2
|
Ada, Lemhi
|
|
Total 1995-2007 and 2008 Preliminary
|
34
|
|
*Number of deaths reported on death certificate as CJD, 1985—2008 (2008 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—2008: A81.0 (Creutzfeldt-Jakob Disease) and B94.8 (Sequelae of other specified infectious and parasitic diseases and Creutzfeldt-Jakob Disease is specified). Data for 2008 are preliminary and are based on records filed as of January 21, 2009.
Source: Bureau of Vital Records and Health Statistics, Idaho Department of Health and Welfare (2/2009).
CJD in Idaho: 2005
In late July 2005, South Central District Health and the Idaho Dept. of Health and Welfare, Office of Epidemiology and Food Protection, began investigating reports of possible CJD cases due to a concern about an increase in this disease in southcentral Idaho. All five patients were over 55 years of age and all are deceased. The investigation included 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. No common travel, occupation, place of employment, surgical procedures, medications, supplements, or unusual food history were identified. Autopsies were done for three of the five patients. A diagnosis of CJD (not variant CJD) was confirmed in two autopsied patients and the autopsy results for the third patient were not consistent with CJD. The illness in the two patients who were not autopsied did not meet the case definition for probable or possible CJD. No explanation for the increase in reports was found, but in 2006 and 2007, no cases of CJD were reported from the area, suggesting the cases may have coincidentally occurred in the same time frame.
For a checklist for healthcare providers regarding suspected CJD cases, click here.
For frequently asked questions about CJD and additional resources concerning CJD, click here.
For the Idaho Disease Bulletin concerning CJD, dated August 2004, click here.
For the Idaho Disease Bulletin concerning CJD, dated October 2005, click here.