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Possible case of CJD investigated.

Byline: Elizabeth Cooney

WORCESTER - Public health specialists are investigating a possible case of Creutzfeldt-Jakob disease, a rare degenerative brain disorder that may have led to the death of a Shrewsbury woman.

The 57-year-old woman died last week at St. Vincent Hospital in Worcester of "organic brain disease," according to her death certificate. After Worcester public health commissioner Dr. Leonard J. Morse pressed for more information, the certificate was amended to "Creutzfeldt-Jakob disease (presumed)," he said Thursday.

The patient's identity is not being released.

Six or seven cases of the disease occur every year in Massachusetts, Dr. Alfred DeMaria of the state Department of Public Health, said yesterday that level has been consistent since the state started tracking incidents

in 1992.

The disease is usually confirmed by a brain biopsy, but one was not performed in this recent case. A laboratory test to detect a protein found in the spinal fluid of CJD patients is not yet complete.

Without a brain tissue test, the diagnosis will still be presumed even if the test result is positive, Dr. DeMaria said.

Creutzfeldt-Jakob disease gained wide attention in the 1980s when a strain of the disease sickened cows in Great Britain and people became ill after eating beef contaminated with that strain.

Only three people in the United States have contracted that variant form of CJD, commonly called mad-cow disease; they all were exposed in Great Britain.

"There's no reason to think this had anything to do with the variant," Dr. DeMaria said. "It's such a different form of the disease. Variant CJD is a different clinical entity and has a different type of spread."

Because CJD is both rare and serious, an epidemiologic study is appropriate when it is suspected, both doctors said.

About 85 percent of cases are classified as sporadic, with no known cause, while the other 15 percent come from eating infected beef, being contaminated with infected tissue through a medical procedure, or carrying a genetic mutation that increases susceptibility.

"Our public health nurses will do an epidemiological study of where she lived, where she traveled, where she worked," Dr. Morse said. "They are already working on it."

People should not be alarmed, both doctors said.

"The public should feel reassured this is not contagious," Dr. Morse said.
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Publication:Telegram & Gazette (Worcester, MA)
Date:Sep 27, 2008
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