Two Creutzfeldt-Jakob cases confirmed

September 14, 2001 Kentucky Post by Cincy Starr and Craig Garretson
The incidence of Creutzfeldt-Jakob disease is one in a million. But if the Campbell County coroner is correct, the Northern Kentucky area may be defying the odds.

Since March, Northern Kentucky has had two confirmed cases of CJD, a fatal brain disease associated with tiny protein called prions. Tests are being performed on a third local person wh o died in August to determine if she succumbed to the disease also.

The two confirmed victims are Kenneth Johnstone, 62, the former Boone County superintendent a nd principal who died July 31, and Helen Davis, 76, a nursing home resident who died in March.

The disease was confirmed in Johnstone's case by a brain biopsy, said his widow, Jo Ann Johnstone. Campbell County Coroner Dr. Mark Schweitzer said Ms. Davis, who died of pneumonia, al so had CJD.

Schweitzer, a chiropractor, also believes Sandy Reaves-Haas of Dayton may have died of CJD, although the woman's primary care physician disagrees.

Dr. Robert Tracy, an internal medicine physician from Cold Spring, oversaw Ms. Reaves-Haas' treatment through a mysterious illness that struck suddenly and left her incapacitated and uncommunicative for several months. Tracy said samples of the woman's blood and spinal fluid tested negative for CJD during her illness.

A definitive diagnosis of CJD can be achieved only with a brain biopsy, which Schweitzer requested. But Tracy said Ms. Reaves-Haas' symptoms were not those of a patient with CJD.

In animals, prion disease can take the form of mad cow disease. Mad cow disease has not been detected in the United States, despite active surveillance efforts since May 1990.

Mrs. Johnstone said it's a mystery as to how her husband acquired the rare illness. They h ad never been to England, she said. During a trip to Spain, Italy and Morocco 11 years ago, they ate very little beef. They also rarely ate beef at home.

She said doctors at University Hospital had ruled out the hereditary form of the disease.

The source of Johnson's disease, as with most cases of CJD, may never be known. The disease can lie dormant for many years, further complicating efforts to pin down i ts cause.

Mrs. Johnstone said her husband began showing symptoms of the disease in late June while on vacation. He started tiring easily. When they returned home, that escalated into memory problems .

A former teacher, Johnstone was ''a brilliant man'' until the disease set in, his wifesaid.< p> They saw a neurologist and CJD was suspected; it was later confirmed with a brain biopsy, Mrs . Johnstone said. A few days later -- about six weeks after the first sign of any symptoms -- her husband died.

Schweitzer said the cases appear to be unrelated.

''At this stage in the game, this is not something that caregivers, or the community at-large , needs to be concerned about,'' he said. ''This is a very rare, very hard-to-understand disease, and it can be inflammatory when people see 'mad cow disease.

''It is not a public health risk and should not be a cause for alarm.''

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