Brain disease suspected in 9 cases

BOISE, Idaho (AP) — State and federal health officials are trying to get to the bottom of nine reported cases of suspected sporadic Creutzfeldt-Jakob disease, a naturally occurring form of the fatal brain-wasting illness.

“One thing is very clear in Idaho — the number seems to be higher than the number reported in previous years,” said Dr. Ermias Belay, a CJD specialist with the federal Centers for Disease Control and Prevention (CDC). “So far, the investigations have not found any evidence of any exposure that might be common among the cases.”

Normally, sporadic CJD strikes about one person in a million each year, with an average of 300 cases per year in the United States, or slightly more than one case a year in Idaho. In the past two decades, the most cases reported in the state in a year have been three.

Sporadic CJD differs from the permutation dubbed variant CJD, which is caused by eating mad cow-tainted beef and has killed at least 180 persons in the United Kingdom and continental Europe since the 1990s.

Of the nine suspected cases reported this year, three tested positive for an infectious disease of the nervous system, though more tests are pending to determine whether the fatal illness was sporadic CJD. Four persons were buried without autopsies. Two suspected cases tested negative.

Still, federal and state health officials are stopping just short of calling the Idaho cases a “cluster,” waiting for final test results from those who underwent autopsies.

The best tool for investigators to pin down the diagnosis — the autopsy — is sometimes difficult to get, said Tom Shanahan of the Idaho Department of Health and Welfare.

Pathologists often are reluctant to perform the procedure, the cost of an autopsy can be high, and some families are reluctant to give consent, officials said.

Joan Kingsford wanted an autopsy done on her husband, Alvin, who died of suspected sporadic CJD, but no mortician in the area would agree to handle his body after his brain cavity had been opened. They feared they would catch the rare disease, Mrs. Kingsford said.

CJD is transmitted through a malformed prion protein found primarily in the brain and spinal fluid of those infected, Dr. Belay said. Standard sterilization procedures do not eliminate the risk of infection; instead, equipment must be soaked in a chemical solution for more than an hour and then heated, according to the World Health Organization.