Local man identified with rare brain disease

An unusual cluster of cases in southern Idaho of a rare brain disease, including an elderly man from Elmore County, is being investigated by the state health department.

Five elderly women in the Twin Falls and Buhl area are suspected to have died from the disease, known as Creutzfeldt-Jakob Disease (CJD). All of the cases, including the man from Elmore County who is suspected of having it at this time, involved individuals over the age of 60. The Elmore County man, who has not been identified, is being treated at the VA hospital in Boise.

The disease is almost always fatal within 6 months after the first onset of symptoms, but cannot be conclusively confirmed until autopsies are conducted. Brain tissue typically is sent to Case Western Reserve University in Ohio, which has a lab specializing in the rare disease, to confirm the diagnosis, but tests often take weeks to be completed.

So far, all of the cases of the always fatal brain-wasting illness are only suspected at this point. None have been confirmed.

Researchers believe the disease occurs when misshapen brain proteins (prions) attack brain cells, creating sponge-like holes in brain tissue. But what triggers that isn't known.

The disease causes profound mental and physical deterioration, appears to have long incubation periods before signs and symptoms appear.

Once a person becomes sick, the course of the disease is swift. People usually die from complications of CJD just months after developing symptoms. No treatment exists and nothing slows the progression of the disease. CJD occurs almost always in people over the age of 60.

Worldwide, the disease occurs naturally in about one in every one million people, which means Idaho should have about 1.2 cases per year. In fact, however, the state typically sees three to four cases a year, but no one knows why. This is the first year the disease has been on the list of diseases that doctors must report, but state health officials believe few cases weren't reported in the past.

Because so little is known about the cause of the disease, the existence of a "cluster," such as the one suspected in southern Idaho, could help researchers identify a potential cause, if any common elements can be found. Or, state health officials said, it may just be a statistical anomoly.

The national Center for Disease Control is monitoring the cases, but the state is serving as the lead investigative agency.

All the suspected cases at present involve the "standard" form of CJD, which appears to be naturally occurring. A variant form of the disease (vCJD), can be triggered in rare cases if a person eats meat infected with bovine encephalitis, known as "mad cow" disease.

State health officials stressed, however, that the form of the diseases currently under investigation by state epidemiologists are not related to mad cow disease in any way, and there is no link or threat to the beef supply or to anyone eating beef.