January 19, 2004 Republican-American By Jennifer Clement

Today, people associate CJD with the mad cow epidemic that gripped Europe in the 1990s and the disease that infected a Holstein cow in Washington state last month. But Brinton didn't get CJD from eating bad meat.

At least not that doctors know of.

And that's the problem.

"There's just so much unknown," said Barbara Brinton, who shuttled her husband from one doctor to the next for 18 months, desperately seeking answers while he slipped away, mentally and physically.

"Every day I was losing him, piece by piece," she said.

Though CJD and the human form of mad cow disease - known as variant CJD - share nearly identical names and symptoms, and are both fatal, they are not the same disease, said Dr. Ermias Belay, an epidemiologist at the Centers for Disease Control and Prevention.

The diseases became confused during the European mad-cow epidemic, when bovine spongiform encephalopathy, BSE, jumped from cows to humans. Doctors noticed the resulting human disease, with a rapid onset of dementia and progressive loss of coordination, looked a lot like classic CJD, which has been documented since the 1920s. So they named it new variant or vCJD.

Classic or "sporadic" CJD, which killed Brinton, strikes 250 to 300 Americans each year for no known reason. Variant CJD, which is rarer, has been diagnosed in 153 people worldwide, all of who are thought to have eaten tainted beef in Europe.

Diagnosing the disease can be difficult. Researchers believe both CJD and its variant are "prion" diseases, characterized by malformed proteins in the brain. These diseases can be inherited or transmitted. If the symptoms are recognized early, a spinal tap or MRI may provide further evidence of CJD. However, a 1989 study at Yale University found in 13 percent of cases, CJD was mistaken for Alzheimer's, another disease that can't be verified absent an autopsy. Since not all families request an autopsy, they may never know whether CJD was the cause of death.

Worse, a person can be sick with CJD without showing any symptoms, up to 30 years in some cases.

"They're walking around performing normally and not showing the disease for eight to 30 years and we have no way of testing for an early infection that's easy or that's simple," said Dr. Laura Manuelidis, a neuropathologist and the lead researcher in the Yale University study.

Manuelidis believes CJD and its variant form are closely related, and pointed to research showing that mice infected with mad cow disease presented symptoms that looked like classic CJD, not its variant.

Watching Brinton stumble as he walked down the driveway, his wife was reminded of the TV images she had seen of stumbling cattle that had mad cow disease.

"All I could think of was he looked like that cow," she recalled.

But doctors dismissed her suggestion that Brinton might have CJD. She says a neurologist at UConn Health Center thought he was suffering from lack of sleep. She says another, in New Milford, diagnosed small vessel disease, which is caused by an obstruction in the flow of blood.

CJD was simply too rare, she was told. Brinton's chances of having it were literally 1 in 1 million.

Of the new cases that occur each year in the U.S., the vast majority - 85 percent - are diagnosed as classic or "sporadic" CJD.

Another 15 percent of CJD cases are familial, caused by a genetic mutation. A few cases also have been transmitted during medical procedures, for example, when brain surgery was performed using instruments previously used on a CJD patient. The infectious tissues that can transmit CJD remain infectious after treatment with both heat, which destroys most bacteria, and ultraviolet light, which should inactivate viruses, according to the nonprofit CJD Foundation.

Brinton's first symptoms, which appeared when he was 64, were similar to those of Alzheimer's. He began saying things that made him seem paranoid, withdrew from social situations and experienced memory lapses. Though he had driven neighbors to and from the major airports for years, he suddenly began giving his wife wrong directions.

Like those suffering from Alzheimer's, Brinton also demonstrated a tendency to wander off. That was one of the earliest signs that something was wrong. From home, he would wander to the service station across the street. "They would call me from the garage and say, 'Hey, Barbara. Kenny's over here and he doesn't have a jacket,'" his wife recalled.

A psychiatrist in New Milford diagnosed dementia. Physical symptoms were quick to follow. Brinton became unsteady on his feet. He lost his sense of coordination. Barbara Brinton poignantly recalled how difficult it was to help him in and out of the shower. He had difficulty speaking.

Since Brinton had been treated for an eye condition called uveitis, his wife was concerned that he might have lymphoma of the central nervous system, a rare cancer that can mimic uveitis. She took him to a neuro-oncologist at Massachusetts General Hospital, where doctors performed two spinal taps but, she said, did not examine his spinal fluid for three "marker" proteins that would have suggested CJD.

In May 2001, Brinton was admitted to New Milford Hospital and then moved to a nursing home. As the weeks passed, he lost his ability to speak or move on his own. Eight months later, he died at the age of 65.

Barbara Brinton recalled how, just before his death, a nurse had lifted her husband in her arms to move him to one side of the bed. By then he had lost more than 100 pounds and seemed only a shadow of the man she had known, the one with the hearty laugh and big tummy who dreamed of building a timber frame home for their retirement.

Experts say late-stage CJD patients may seem to be following what is going on around them, but in fact, they are not aware. Barbara Brinton is still not sure. "Everyday I think of it - that he knew," she said. "He was very angry the first night we were at (the nursing home), very, very angry. But he couldn't talk."

She also wonders about what made her husband sick. A form of mad cow disease has been found in deer and elk. Could it have been something he ate at one of the game dinners he went to years ago?

"We were in England two years before the onset," she said, pointing out that she ate the same things he did on that trip. Does that mean she might become sick?

Since the discovery of the first U.S. case of mad cow disease, several groups have called for more widespread testing of cattle. Last week, the Physicians Committee for Responsible Medicine, a nonprofit organization that promotes preventive medicine, sent a letter to U.S. Secretary of Agriculture Ann Veneman, calling for stronger measures to prevent the spread of the disease to other cattle and people.

"It is still possible - even likely - that further cases of mad cow disease will emerge, with human cases to follow," Dr. Amy Joy Lanou, the committee's nutrition director, said in a statement. She further alleged: "The USDA cares far less about public safety than about cattle industry profits and has failed to enact the safety measures we have been recommending for years."

The group described current monitoring of mad cow disease and CJD as "haphazard."

"Although Japan tests every cow at slaughter, the United States tests far less than 1 percent of the 36 million cows slaughtered per year," the committee said.

U.S. Rep. Rosa L. DeLauro, D-3d District, is also calling for more frequent testing of cattle.

"What we need is a comprehensive approach that will finally give us the same ability countries like England have to respond quickly and effectively when health concerns arise. It comes down to a matter of political will. There is no reason in the world our food safety system should not be the envy of the world," she said in a statement last week.

The CDC maintains the risk to human health from mad cow disease in the U.S. is very low. Cases are monitored by analyzing death certificate information compiled by the national Center for Health Statistics. In addition, the National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Ohio tracks CJD cases around the country. Eileen Campbell, who manages the center, said family members or physicians may request post-mortem testing to confirm CJD.

However, the system isn't foolproof. According to the grassroots organization CJD Voice, doctors are only required to report CJD in 24 states, including Connecticut, where any case involving a patient 55 or younger must be reported within 12 hours.

The age restriction may stem from the fact that classic CJD typically strikes patients between the age of 50 and 75, while the variant form usually appears in people younger than 30.

Since few autopsies are performed, the number of CJD cases also may be higher than people think, said Yale's Manuelidis. "If we don't track it we don't know," she said.

To ensure her husband's case was counted, Barbara Brinton sent his autopsy slides to the national surveillance center and requested the cause of death on his death certificate be changed to indicate CJD.

She hopes other survivors are as persistent. "I can't be the only one," she said.