CJD gaining attention in United States

CJD gaining attention in United States

June 5, 2001 Beacon Journal (Akron) by Tracy Wheeler

In the middle of August, June Burdick simply stopped feeling well.

By Labor Day, her walk had taken on a noticeable wobble. And she was talking nonstop -- something out of character for this reserved 73-year-old resident of rural Nebraska.

Within days, her speech became slurred, her walk grew clumsier and her left arm turned uncontrollable, flinging itself into the air as if shooing away flies.

The CAT scans, bone scans, MRIs, vital signs and spinal taps showed nothing unusual.

By mid-September, doctors diagnosed Burdick as having a stroke, ``simply because they couldn't find anything else wrong with her,'' said her daughter, Marianne Fisher. ``They did a test for everything they could think of, then redid them again. The doctors were as frustrated as we were. They were running out of tests to do. They even tested her for Lyme disease.''

Burdick's behavior grew increasingly bizarre over the next two weeks. She laughed at everything, as if drunk. She sat in her bed, pretending to conduct an orchestra. She feared the dark, along with the colors blue and green.

By Oct. 8 -- less than six weeks after the symptoms began to show -- Burdick was dead. It wasn't until four days before her death, after a four-hour trip to see a doctor in Fort Collins, Colo., that her family finally began to learn what was wrong.

``The doctor walked in . . . and within five minutes, we had a probable diagnosis of CJD,'' Fisher recalled. ``Of course, we'd never heard of it.''

Mad cow gets attention

Many people are unfamiliar with Creutzfeldt-Jakob disease -- CJD for short. But it's gaining attention in the United States as a new version -- variant Creutzfeldt-Jakob (vCJD) -- spreads through Europe, leaving a rising death toll in its wake.

This variant was discovered in Great Britain in 1996. Scientists concluded it was caused by the consumption of beef tainted by mad cow disease.

Both mad cow and Creutzfeldt-Jakob are part of a family of neurological diseases called transmissible spongiform encephalopathy. Scrapie in sheep and chronic wasting disease in deer and elk also are part of this disease family.

Scrapie has never passed CJD to humans, but three CJD deaths -- two hunters and a person who ate venison -- have been linked to infected deer and elk in Colorado, Wyoming, Nebraska, Montana, South Dakota and Oklahoma.

In all these diseases, abnormally shaped proteins called prions ravage the central nervous system. Prions are virtually indestructible and invisible to the body's immune system.

In some cases, prions are believed to enter the body through tainted meat or surgical equipment, slowly latching onto and altering healthy proteins. In other cases, prions form on their own.

``Through the course of your lifetime, you make bad proteins and your cells know how to degrade them,'' said Robert B. Petersen, an associate professor of neuropathology at Case Western Reserve University. ``They identify them, shunt them off, degrade them, and start over without (allowing) any type of pathological process. When you age, apparently these mechanisms that do the surveillance -- the cellular cops -- break down. They're just not as efficient.''

When that system fails and prions thrive, disaster follows. Though the prions can be dormant for decades, they will eventually gnaw away at the nervous system, drilling microscopic holes throughout the brain.

The first sign of both CJD and vCJD is blurred vision or a loud ringing in the ears, followed by a loss of balance, arm and hand tremors and dementia. Death is a certainty.

The classic form of Creutzfeldt-Jakob typically strikes between the ages of 50 and 75, after decades of lying dormant in the body. In 85 percent of the cases, the cause is unknown. About 10 percent to 15 percent of the cases have a genetic origin and about 1 percent are contracted through medical procedures. Death occurs quickly once symptoms appear, often within three to six months.

The victims of vCJD are much younger, with 28 being the average age of onset. And the symptoms, while similar, last longer, up to 13 months until death.

``It's just such a horrendous thing to see somebody's mind go so fast,'' Fisher said of her mother. ``Thank God she didn't linger.''

On the lookout

If variant Creutzfeldt-Jakob ever reaches the United States, Dr. Pierluigi Gambetti will be one of the first to know.

Gambetti is director of the National Prion Disease Pathology Surveillance Center, housed at Case Western Reserve University. The center tracks and investigates suspected cases of CJD and vCJD in the United States.

Since the center opened in 1997, 480 cases have been investigated, with 294 diagnosed as CJD. None was identified as vCJD.

However, Gambetti said not every suspected case of CJD or vCJD makes its way to the center. In some cases, the doctors simply don't recognize the symptoms and misdiagnose the death as a stroke or Alzheimer's. In others, doctors suspect CJD, but the family refuses to permit an autopsy for personal, religious or financial reasons. And in still other cases, pathologists are too scared to perform autopsies, fearing exposure to the deadly prions.

It's estimated that CJD is the cause of one to two deaths out of a million. Based on a U.S. population of 284 million, Gambetti expects to find a little more than 400 cases of CJD each year. Instead, he saw only 111 in 2000, about a fourth of what was expected.

``The chance of losing an important case is high,'' he said. ``I won't be completely happy until we reach 80 percent, at least.''

Two studies have found that doctors often don't recognize CJD. One by Yale University found that 13 percent of suspected Alzheimer's patients had actually died of CJD. Another at the University of Pittsburgh showed that 5 percent of Alzheimer's patients were CJD victims.

Devastating illness

Like Burdick's family, Kate Foster, of Killeen, Texas, had to wait weeks for doctors to recognize CJD when her 62-year-old husband became ill. Marvin Foster's symptoms surfaced last May, shortly after shoulder surgery. A diagnosis didn't come for two months.

A former paratrooper, Marvin Foster was a strong, vibrant man. But for five months before he died on Jan. 24, he couldn't walk, talk or swallow.

``Those were the hardest months,'' Kay Foster said. ``Myself and my children were in denial. We couldn't accept the fact that he had this and there was no cure. One doctor along the way said he had encephalitis and give it a year and he would get better. We hung on to that. . . . ''

``We had never heard of this before he was diagnosed.''

And those who have heard of it are often confused by a diagnosis of CJD, thinking that it means a loved one has mad cow disease. For a death to be linked to mad cow, however, an autopsy would have to find vCJD.

Epidemiologists can quickly tell the difference between the two.

Under a microscope, classic CJD would show up in brain tissue as tiny holes. In vCJD, the holes would be surrounded by blotchy white patches of protein referred to as plaques.

If such a sample were to arrive at Case Western Reserve University, ``it would jump out at me,'' Gambetti said.

Believing in cover-up

Sandy Allen, who lives on a small farm in Midland, Texas, isn't reassured that scientists have not found a case of vCJD in the United States.

She believes that both vCJD and mad cow are already here -- and that the government is covering it up.

``I don't know if the government will ever fess up to it,'' she said. ``I like to say, `They'll do it when the mad cows come home.' ''

Her concern about mad cow disease is understandable. Her mother died of CJD 11 years ago, and every time she hears a ringing in her ear or stumbles over her feet, she worries that she has been stricken, too.

``Oh, I think about it all the time,'' she said. ``I'm not worried about cancer. I'm not worried about heart disease. I'm deathly worried about CJD. I'll do myself in. I'm not going to go through that.

``How do I know I didn't eat what my mother ate? You don't know what's in sausage anymore. You don't know what parts of the animal are used for what.''

On the other hand, Fisher doesn't think it really matters whether vCJD is in the United States. CJD is here, killing people.

``When you say there's no (mad cow) in the United States,'' Fisher said, ``I don't think people understand that's not the same as saying there's no CJD in the United States.''

Tracy Wheeler can be reached at 330-996-3721 or tawheeler@thebeaconjournal.com

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