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Mad Cow Case Stirs Confusion Over Two Diseases Linked by Name

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The Seattle Times


SEATTLE - The footage of British cattle stumbling, collapsing and writhing - aired repeatedly these days on TV news reports - is a stinging reminder for Dee Dee Vogt. The cattle's wildly off-balance gait and panicky movements closely mirror her mother's degeneration from Creutzfeldt-Jakob disease, a cousin of mad cow disease.

On May 3, 2003, Vogt and her mother, Carol Lapham, celebrated their shared birthday. Lapham, 67, a homemaker in Washington's Skagit Valley, was her chatty self, atwitter with news of Vogt's upcoming college graduation. But six days later, on Mother's Day, Lapham couldn't figure out how to operate the telephone or salt shaker.

Within two weeks, her flailing legs could no longer walk and her speech went from nonsensical to nonexistent. She died June 23.

Doctors at the University of Washington Medical Center diagnosed Lapham with Creutzfeldt-Jakob disease (CJD), an incurable and always fatal brain-destroying disorder that's even harder to comprehend than to pronounce (KROITS-FELT-YAW-KOBE).

Vogt, who lives in Fairview, Tenn., had heard the name and thought she remembered that people in England had gotten it from eating beef from animals with mad cow disease. But doctors assured her that her mother's case had nothing to do with anything she ate.

Sometimes, they said, CJD, which is thought to be caused by a mysterious rogue protein known as a prion, can pop up with no apparent explanation.

But after the U.S. Department of Agriculture (USDA) announced 12 days ago that a Washington cow showed up with the nation's first case of mad cow disease, the question lingering in Vogt's mind has taken on a new urgency. "How do they really know my mother's case wasn't caused by eating beef?"

It's a question Florence Kranitz, head of the nonprofit Creutzfeldt-Jakob Foundation in Ohio, has heard over and over since news of the mad cow case broke. "I've been getting lots of calls from families whose loved ones died of CJD years ago, and now they want to know if this finally explains it," she said.

Experts said no. Though CJD and the human form of mad cow disease - known as variant CJD - share nearly identical names and similar symptoms, and both are fatal, they are not the same disease, said Dr. Ermias Belray, an epidemiologist at the Centers for Disease Control and Prevention (CDC).

Each year, about one in a million people worldwide gets what's known as classic CJD, a naturally occurring brain disease identified in the 1920s, long before mad cows entered the picture.

The two diseases became confused in the mid-1990s, when mad cow disease, or bovine spongiform encephalopathy (BSE), jumped from cows to humans in England. Doctors noticed the resulting human disease - with rapid onset of dementia and progressive loss of coordination - looked a lot like classic CJD, so they named it new variant CJD.

Classic CJD strikes 250 to 300 Americans each year for no known reason. Variant CJD, which is rarer, has been reported in 153 people worldwide, all of whom are thought to have eaten tainted beef in Europe during the mad cow epidemic.

If you know what to look for, the diseases are quite distinct, said Dr. Joe Zunt, an assistant professor of neurology at the UW. Early symptoms differ and the classic form usually strikes people between 50 and 75, while the variant form usually appears in people younger than 30.

The United States has a surveillance system designed to pinpoint suspicious cases of CJD so that alarms would be raised if the variant form were to enter the country. Only one variant case has been found in the United States: a woman who lives in Florida but grew up in England during the peak of mad cow disease there.

But the system isn't foolproof. Half the states - including Washington - do not require doctors to report cases of CJD. In fact, in Washington, only about one-third of the 102 patients who died from CJD in the past 25 years were autopsied.

That's a big concern, said Shu Chen, a protein expert at the National Prion Disease Pathology Surveillance Center in Cleveland, because an autopsy is the only way to definitively diagnose CJD or to distinguish between the classic and the variant form. "Without an autopsy, you don't know for sure; it could be a strange-looking case of early-onset Alzheimer's. Or an Alzheimer's case could really be CJD," he said.

Washington is working on ramping up its surveillance, said Jo Hofmann, a state epidemiologist for communicable diseases. The state Department of Health will soon send a letter to neurologists, neuropathologists, medical examiners and local health departments requesting that doctors report any suspected cases of CJD to the state and encourage families to have the bodies autopsied. The prion-surveillance center, funded by the CDC, will pay for the autopsies.

As a hospice worker, Sandy Skrinjaric, of Shoreline, Wash., witnessed numerous heart-wrenching deaths - from cancer to the slow decline of Alzheimer's - but she said her mother's death from classic CJD in 2001 was by far the most horrifying she's ever seen. "It was just terrifying to watch her grow crazy by the hour," she said.

Frances Skrinjaric's first complaint was blurry vision, which her daughter chalked up to macular degeneration catching up to her 78-year-old mother, who lived outside Pittsburgh. But within two months, her mother was ripping off her nightgown in the hospital and singing at the top of her lungs. Her arms would shoot into the air and lock in a contorted position.

"From one afternoon to the next, you could tell more and more parts of her brain were being eaten away," she said.

Frances Skrinjaric was diagnosed with classic CJD based on her symptoms and a spinal-fluid test that showed suspicious proteins. But her family couldn't afford an autopsy and didn't know about the surveillance center that would have done it for free.

"Since my mother was never autopsied, they assume it's a classic case," she said. "That strikes me as an extremely big assumption. "

But as long as an autopsy is done, Chen said there's no confusing the diseases. Under the microscope, brains with either disease have an unmistakable spongy appearance. But in new variant cases, the holes surround deposits of plaque, absent in the classic disease.

"It's very clear-cut; people whose family members had the disease and were autopsied here can be assured their loved ones had classic CJD," Chen said.

Public-health officials hope alarm over Washington's single reported case of a stricken cow will help boost surveillance of the human diseases.

"Now that doctors will be thinking about it as possible cases of mad cow disease, they may be more likely to report suspicious neurological conditions, and that could help us solve the mysteries of both diseases," said Belray, of the CDC.



Though classic Creutzfeldt-Jakob disease (CJD) and new variant Creutzfeldt-Jakob disease share a name, they are not the same condition. Here are the key differences.


Who gets it: People between 50 and 75, with an average age of 68

How common: About one case per million people worldwide. In the United States, 250 to 300 cases a year; 102 cases reported in Washington since 1977.

Cause: 85 percent of cases are considered sporadic, meaning there's no known cause. The others are thought to be caused by a genetic mutation or contaminated surgical equipment.

Symptoms: Blurred vision, memory loss, confusion and loss of coordination followed by progressive dementia, uncontrollable jerky movements, coma and death.

Prognosis: Patients usually die within four to six months of first symptoms.

Diagnosis: Abnormal brain waves on electroencephalogram (EEG). Spinal fluid has elevated levels of protein. In autopsy, the brain appears spongelike.


Who gets it: Average age is 28

How common: 153 cases reported worldwide (137 in the United Kingdom).

Cause: All cases have been linked to eating British beef during mad cow disease outbreak in the 1980s and 1990s.

Symptoms: Psychiatric problems such as depression, anxiety, withdrawal and paranoia appear first. Some patients report severe pain in arms and legs. Then disease takes similar course as classic CJD.

Prognosis: Patients can live for two or more years after onset.

Diagnosis: Magnetic resonance imaging (MRI) scan shows distinct pattern. In autopsy, brain shows hallmark plaque deposits surrounded by spongy patches; chemical test can isolate the prion.

Sources: Dr. Joe Zunt, University of Washington; Centers for Disease Control and Prevention; National Prion Disease Pathology Surveillance Center


(c) 2004, The Seattle Times. Distributed by Knight Ridder/Tribune News Service.


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