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Idaho probes rare brain disease deaths

In late May Marjorie Skinner played golf well enough to place fourth in a Memorial Day weekend golf tournament. Yet within weeks, the previously vibrant retiree suddenly started losing her ability to speak.
/ Source: Reuters

In late May Marjorie Skinner played golf well enough to place fourth in a Memorial Day weekend golf tournament. Yet within weeks, the previously vibrant retiree suddenly started losing her ability to speak.

By the time her family buried her Friday, she was the fifth suspected victim in the same sparsely populated area of Idaho of Creutzfeldt-Jakob Disease (CJD), a rare brain-wasting disease that typically afflicts only one in a million people.

As word of this latest death spread Monday, local and federal health experts sifted through clues about an illness different from variant Creutzfeldt-Jakob Disease, the human form of mad cow disease.

“Five (cases) in one valley is pretty serious,” Sue Skinner, Marjorie’s daughter in law, said in an interview. ”It’s a grave concern in our family.”

The mystery has deepened in recent weeks. Only at the end of May did local health officials see a second elderly woman die of the incurable disease involving a malformed protein, or prion, that kills brain cells. After that, they learned of three other suspected cases, including a CJD death in February that was reported only last month.

“Is what is happening in Idaho an anomaly, a statistical fluke? That is possible,” said Ermias Belay, a top CJD expert with the Centers for Disease Control and Prevention in Atlanta who is helping advise officials in Idaho. “But once it exceeds 1.5 or 2 per million, you start asking questions.”

“If they are all confirmed, it could be odd.”

In a year, the United States typically sees fewer than 300 CJD cases, which mete out rapid death to the elderly, according to the Centers for Disease Control.

Any unusual hobbies?
In Twin Falls, Cheryle Becker, epidemiology manager for Idaho’s South Central District Health, is going to families with detailed questionnaires aimed at finding the roots of a disease that could date back 30 years.

She asks about past travels, unusual hobbies and dietary habits, including of organ meats, brain and venison.

“We’re asking them if they’ve consumed elk,” Becker said, adding that many hunt venison in this region of the country. ”We’re not having many people say that they have.”

Experts say they do not expect to find a link to eating meat, although locals are asking if there is any connection to the human variant of mad cow disease. “It’s very frightening to the community.” said Cheryl Juntunen, director of the South Central District Health.

Two confirmed U.S. cases of mad cow disease, one in a Washington state dairy animal in 2003 and the other in a Texas beef cow this year have further heightened concern.

To date, health experts have found few parallels between the women, all of European heritage. Four were Idaho natives, all had children, none had experienced neurological disease.

One had spent time in Britain prior to the outbreak of mad cow disease there, officials said. Several husbands were involved in farming, as is commonplace in a rural farmland region where locals still talk about stuntman Evel Knievel’s 1974 attempted jump over the Snake River.

“There are things that lead you to believe this is not variant CJD,” Becker said, pointing to the advanced age of the victims and faster death than in mad cow-related cases.

The Centers for Disease Control estimate that spontaneous flaws in cell proteins result in 85 percent of CJD cases. Another 5-15 percent comes from genetic inheritance, leaving just a small percentage of other unexplained cases.

Yet experts say studies of a few past clusters of CJD cases are inconclusive; some say better records and ability to recognize the illness could account for the Idaho mystery.

“I think in the end this may be a statistical fluke,” said Christine Hahn, chief epidemiologist for the state of Idaho. ”But there is so little known and there have been very few published reports on these clusters.”

Families for three out of the five Idaho victims have agreed to autopsies, officials say, and results from those tests may provide essential clues in the coming weeks.