BOISE, Idaho – Preliminary tests on the remains of two Idaho women show they died of the brain-wasting illness Creutzfeldt-Jakob disease, but additional tests are needed to determine whether it was the naturally occurring form or the variant related to mad cow disease.
Idaho Department of Health and Welfare officials announced the findings Wednesday after notifying the families of the women, one of whom was in her 60s and lived in Twin Falls County and the other who was previously identified by her family as 53-year-old Kathy Isenberg of St. Maries.
Because of privacy restrictions, state health officials do not release names of individuals suspected of dying from the disease, which can only be conclusively diagnosed post-mortem.
The results bring to three the number of confirmed deaths this year in Idaho caused by diseases related to “prions,” or malformed proteins. Earlier this year, tests by the National Prion Disease Pathology Surveillance Center at Case Western University in Ohio determined that another Twin Falls County woman had died from a prion-related disease believed to be Creutzfeldt-Jakob disease.
Additional tests are under way at the lab to determine what form of CJD was responsible in the three confirmed cases.
“Generally, 85 percent of the tests come back as the sporadic, or naturally occurring form, 14 percent come back as the familial form that is passed down through generations and less than 1 percent come back as the variant form,” said Tom Shanahan, spokesman for the Idaho agency. “There’s never been a variant case acquired in the United States.”
The incurable illness causes normal brain proteins to fold in half, resulting in brain damage and rapid deterioration of body movement and speaking ability. Scientists don’t know what causes the sporadic form of CJD.
The most well-known prion disease in animals is bovine spongiform encephalopathy, or mad cow disease, which humans can get if they eat meat from an infected cow.
There have been eight suspected cases of CJD in Idaho since January. Of the four cases from Twin Falls County, one has been ruled out, an autopsy was not conducted on another and two are now confirmed, along with the confirmation of Isenberg’s case in Benewah County.
Autopsies also were not conducted on reported CJD victims in Bear Lake and Minidoka counties. Preliminary tests have not been received for an Elmore County man in his 60s who died last month and whose doctor suspected CJD as the cause.
This is the first year a new state law requires doctors to report possible cases of the fatal neurodegenerative disease. Idaho officials have never recorded more than three cases in a single year and the disease usually infects only one out of every 1 million people worldwide.
In 2002, one case of variant CJD was diagnosed in a Florida resident who had lived in the United Kingdom.
Idaho officials acknowledge the unusually high number of CJD cases may be attributed to the new reporting requirement, but are working with the federal Centers for Disease Control and Prevention to determine whether the reports represent a cluster and whether there is anything in common among the victims.
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