September 28, 2005

Idaho confirms 3rd death from rare brain disease

By Laura Zuckerman

SALMON, Idaho (Reuters) - Test results confirm a rare brain-wasting illness similar to mad cow disease claimed the life of a 53 year-old northern Idaho woman earlier this month, state health officials said on Wednesday.

The results bring to three the number of confirmed cases this year in Idaho of Creutzfeldt-Jakob Disease, an incurable illness involving a malformed protein that kills brain cells.

Idaho officials believe a naturally occurring form of the disease is responsible for the three cases and may be involved in an additional four deaths this year.

Further testing is under way to rule out variant Creutzfeldt-Jakob Disease, considered the human form of mad cow disease and linked to eating beef from infected cattle.

The naturally occurring form of Creutzfeldt-Jakob Disease, whose cause is unknown and which is not contagious, typically is found at an annual rate of one case per million Americans.

Idaho, with a population of 1.4 million, already has exceeded the expected number of cases in a year, prompting concern among medical professionals.

"Any time you have more cases of a disease than you expect, you want to know why," said Cheryle Becker, epidemiology manager for Idaho's South Central District Health.

Becker's office has overseen the probe into two confirmed cases of Creutzfeldt-Jakob Disease and is investigating two more suspected cases.

"For that part of the country, it seems to be an unusual number of cases," said Ermias Belay, a Creutzfeldt-Jakob Disease expert with the U.S. Centers for Disease Control and Prevention.

The specter of mad cow disease has sparked alarm among some residents of a state whose industries include cattle production. Becker and others are cautioning the public not to overreact.

"We don't want to panic people about this," she said. "We don't have information that would cause people to change their lives in any manner."

Tom Shanahan, a spokesman for the Idaho Department of Health and Welfare, said an analysis of data the agency has collected on confirmed and suspected Creutzfeldt-Jakob Disease victims shows few common denominators other than all were at least 50 years old and lived in Idaho.