Brain disease cases investigated in Roane County, TN

By Bob Fowler (Contact)
Originally published 06:36 p.m., March 23, 2009
Updated 06:36 p.m., March 23, 2009

The Department of Health is investigating a confirmed case in Roane County of an always fatal, very rare brain disease and a second suspected case.

Only four cases of the baffling Creutzfeldt-Jakob Disease or CJD were recorded in Tennessee last year.

Relatives of the recent victims say the odds of two cases within the same county diagnosed at about the same time are astronomical.

They are questioning whether their loved ones died of a variant of CJD linked to eating cattle infected with “mad cow disease.”

But Health Department officials in a news release Monday state that no one in the U.S. has ever contracted the variant of CJD.

And Dr. Jiri Safar, an expert in the disease, called the chances of the deaths being linked to mad cow disease “extremely remote or nil.”

Kingston resident Mary Lee McGill, 69, died of classic CJD on Feb. 4.

A Rockwood man, Brad Smith, 54, died March 18, also of suspected CJD.

It takes extensive autopsies and weeks of tests to obtain final diagnoses, according to the Health Department.

“The best we can determine, Brad and my mom started getting sick around August,” said McGill’s daughter, Teresa Melton.

“As rare as it is, it just seems too much of a coincidence,” said Smith’s widow, Carol Smith, who said she is a registered nurse.

“I’m very concerned there’s some bad meat around here somewhere,” Melton said.

CJD is a degenerative disease that strikes the nervous system.

Health Department spokeswoman Shelley Walker said the classic form of the ailment occurs sporadically and is not contagious.

Walker said there is also an inherited form of the disease, but it only affects a small percentage of patients.

There are about six to eight cases a year of CJD in Tennessee, according to the state Department of Health.

Classic CJD develops randomly, said Safar, associate director of the National Prion Disease Pathology Surveillance Center.

The disease largely remains a mystery, he said. “We don’t understand exactly how it’s triggered in patients,” he said.

Almost all those cases who contracted the variant of CJD linked to mad cow disease involved people from 13 to 40 years of age who lived for a significant amount of time in Europe or the United Kingdom, Safar said.

The outbreak of mad cow disease was far more widespread overseas than in the U.S. or Canada, Safar said.

Still, all it would have taken was one sickened cow to slip through inspection in the U.S., Carol Smith said.

“They’re trying to prevent people from panicking, which is what we want to avoid,” she said. “Still, a possibility exists, however rare it might be.”

More details as they develop online and in Tuesday's News Sentinel.


http://www.knoxnews.com/news/2009/mar/23/brain-disease-cases-investigated-roane-county/