Idaho investigates brain-wasting disease

2005-08-19T00:00:00Z Idaho investigates brain-wasting disease Casper Star-Tribune Online
August 19, 2005 12:00 am

TWIN FALLS, Idaho (AP) - The Idaho Department of Health and Welfare says authorities are investigating a possible sixth case of Creutzfeldt-Jakob disease after five elderly southern Idaho women died of the brain-wasting illness this year.

The latest suspected case is of a man over the age of 60 in Elmore County, approximately 20 miles southeast of Boise. The county is adjacent to Twin Falls County where four women have died from the disease, the most recent two weeks ago. The fifth death related to the disease was in Minidoka County, which is just east of Twin Falls County.

Creutzfeldt-Jakob disease is an incurable illness that kills brain cells due to a malformed protein known as a prion. State officials are working with federal experts to determine if the Idaho victims had the sporadic, naturally occurring form of CJD and not the variant form of CJD that people can contract when they eat meat from a cow infected with bovine spongiform encephalopathy - commonly known as mad cow disease.

The five women victims were between 60 and 83 years old. Autopsies were performed on three of them. The other two victims died before their disease was reported by a physician and autopsies were not performed. This is the first year that physicians are required to report CJD cases in Idaho.

The unidentified Elmore County man suspected of having the disease is undergoing treatment and state officials say they will ask for an autopsy if he dies.

Brain tissue samples from the three previous autopsies were sent to the National Prion Disease Pathology Surveillance Center at Case Western University in Cleveland. State officials say preliminary results on one of the women showed she did die of a prion disease but they won't know until later this month what form of the disease she had.

"Initially, it looks like sporadic, but we won't know for sure for another week or so," Tom Shanahan of the Idaho Department of Health and Welfare told The Times-News newspaper. "There's always a possibility of a variant."

Normally, the naturally occurring variant of the disease infects just one person per 1 million worldwide annually. Idaho averages three cases a year.

In cooperation with the national Centers for Disease Control and Prevention, state epidemiologist Dr. Christine Hahn is leading an investigation of medical records of the victims and conducting interviews and surveys with victims' families to find any potential link between the cases.

"At this point, we haven't found anything that would give us clues," said Cheryle Becker, an epidemiologist with the South Central District Health office in Twin Falls who is part of the investigative team. We're waiting to get the final interview and then we'll be comparing all the information we have."

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