DOH Investigating Cases Of Rare Disease Near Knoxville

By: TriCities Staff | TriCities.com
Published: March 23, 2009

KNOXVILLE, Tenn. - Tennessee Department of Health Officials say they are investigating a confirmed case of Classic Creutzfeldt-Jacob Disease (CJD) in Roane County but that the disease is not "mad cow disease." There is also a second preliminary diagnosis of CJD in another person in Roane County.

“We have received calls from concerned residents of the area, and based on preliminary results of our investigation, there is no cause for concern about transmission of this very rare illness,” said Paula Campbell, East Regional assistant director. “Classic CJD is a reportable illness, which means state public health experts are experienced with investigating diseases like this to ensure there is no risk to others in the community.”

Classic CJD is a degenerative brain disease caused by a spontaneous change in certain proteins in a person. It is only passed from person to person in very rare medical circumstances and settings. Classic CJD is not related to "mad cow" disease and is not passed from the food we eat.
Rare forms are passed genetically within a family. Final diagnosis of the disease requires testing of brain tissue and interpretation of results at a national reference laboratory, a process which can take several weeks.

Classic CJD occurs worldwide at a rate of approximately one case per one million people per year, although rates of up to two cases per million are not unusual. The risk increases with age, and in persons aged over 50 years of age, the annual rate is approximately 3.4 cases per million. In recent years, the United States has reported about 300 cases of CJD a year. For 2008, there were four cases of CJD in Tennessee, fewer than the expected number for our population.

Another condition with a similar name, Variant CJD (vCJD) is even rarer than Classic CJD. No person has ever contracted vCJD in the U.S. It has occurred in other countries in susceptible people who consumed beef from cattle that were infected with the disease bovine spongiform encephalopathy (BSE), sometimes called “mad cow disease.” Only three cases of vCJD have ever been reported in the United States; all three occurred in people who were born overseas and spent substantial amounts of time in the United Kingdom or the Middle East.

To learn more about CJD, visit the Centers for Disease Control and Prevention Web site.