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MC_USA

Wayne Fugitt

Tue, 06 Jan 2004 23:35:35 -0600

[MC_USA] Classic or Variant

 

Today: January 06, 2004 at 14:30:28 PST

Experts Seek Analysis of Human Mad Cow

By LINDA A. JOHNSON

 

ASSOCIATED PRESS

TRENTON, N.J. (AP) -

 

Scientists have yet to document a single U.S. case of someone getting the human

version of mad cow disease from contaminated beef. Then again, they might not be

looking hard enough.

 

Some experts say scientists should be looking more closely at cases of

Creutzfeldt-Jakob disease - a brain-destroying disorder that kills hundreds of

Americans each year - to see whether some of these deaths were, in fact, caused

by beef from cattle infected with mad cow disease.

" Could there be one (missed) case in there? Maybe, " said Lawrence Schonberger, a

Centers for Disease Control and Prevention epidemiologist who has studied CJD

for more than a decade. " In this game, we never say something's impossible. "

CJD comes in two known varieties: variant CJD, which is caused by eating tainted

beef, and classic CJD. In classic CJD, the source is unknown in about 85 percent

of cases, but doctors generally believe beef is not the cause. The other classic

CJD cases are blamed on an inherited genetic mutation or use of contaminated

instruments or tissue in surgery.

According to government estimates, CJD accounts for about 300 deaths a year in

the United States - all of them believed to be of the classic variety.

But autopsies are performed in only about half those cases. So it is possible

that cases of CJD from eating tainted beef have been missed, experts say.

 

Schonberger and Dr. Pierluigi Gambetti, head of the top U.S. lab studying CJD

and similar diseases, said they hope the mad cow scare that broke out late last

month with the discovery of an infected cow in Washington state will lead to

more autopsies on people with CJD.

 

" I think everyone needs to make a better effort to really gauge the incidence in

the United States and not to miss variant or any other form, " said Gambetti,

director of the National Prion Disease Pathology Surveillance Center at Case

Western Reserve University in Ohio.

 

In both forms of CJD, mutant proteins called prions eat holes in the brain, but

microscopic examination of brain tissue shows distinct differences between the

two.

 

Classic CJD normally strikes people 55 or older, while variant CJD typically

kills people in their 20s and 30s.

Classic CJD incubates silently for years, then usually kills within six months

of first symptoms: trouble standing or walking, speech abnormalities and

declining mental ability.

 

In variant CJD, death typically comes about 14 months after the first symptoms.

Early symptoms include depression, withdrawal and anxiety, and pain, tingling or

numbness. Eventually, patients cannot move or speak.

Only about 150 deaths from variant CJD - most of them in Britain - have been

counted worldwide since 1996.

In New Jersey, one woman is pressing health authorities to take a closer look at

as many as seven CJD deaths among people who ate at the Garden State Park

racetrack in Cherry Hill in the late 1980s and 1990s. Janet Skarbek, a

Cinnaminson accountant, said she is convinced the deaths must be linked to food,

even though six of those cases were ruled classic CJD and an autopsy on the

seventh victim was inconclusive.

Skarbek began following such cases after a woman in the accounting department at

the track where her mother worked died of CJD in 2000 at just 29.

 

Five months later, Skarbek spotted the obituary of an 83-year-old season pass

holder who ate at the track restaurant frequently. Then Skarbek learned a track

patron died of classic CJD in 1997. Through October 2003, Skarbek said, four

more people died of CJD: three former patrons and a 56-year-old woman from the

marketing department at the track, which closed in 2001.

 

Skarbek said she is not disputing that the seven cases are classic CJD,

sometimes called sporadic CJD.

" I'm arguing with the finding that sporadic is not caused by eating tainted

beef, " Skarbek said Wednesday, noting British scientists initially said that mad

cow disease could not be transmitted to humans.

The CDC's Schonberger said Skarbek's theory is unproven but not impossible. One

British study in mice suggested mad cow disease might cause classic CJD.

 

Dr. Omar Bagasra, professor of biology at Claflin University in Orangeburg,

S.C., studied brain tissue from the 29-year-old track employee and said the type

of damage indicates she might have died of yet a new variant of CJD - perhaps

one caused by food.

" CDC needs to investigate this " further, Bagasra said.

---

 

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