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Here's the story for those that don't have web access.

Liz

Form of mad cow disease strikes Oklahoman

By LAURIE WINSLOW World Staff Writer

3/7/99

MIAMI, Okla. -- A 27-year-old Miami man is being treated for a rare and

deadly disease that is a human variation of " mad cow disease. "

Creutzfeldt-Jakob disease is a progressive disease of the nervous system

that causes muscle spasms and loss of mental function. Symptoms may not

occur for months or years after exposure.

Meanwhile, a 30-year-old deer hunter di agnosed with the same condition

is fighting for his life near Salt Lake City. His illness has raised

questions about whether it is possible to contract the illness from

eating deer meat.

The Miami man also was a deer hunter, his relatives said.

Death in the United States among people younger than 30 is extremely

rare, causing fewer than five deaths per billion per year, according to

experts.

Among all ages, the number of cases occurring in Oklahoma ranges from

one to five cases annually, according to the state Health Department.

" This is a tragic case and a very severe disease in a young person, but

it is not communicable person to person, so we don't see a single case

as a public health emergency, " said Dr. Mike Crutcher, state

epidemiologist with the Oklahoma State Department of Health.

At first, symptoms of the disease may resemble those of other dementias

with neglect of personal hygiene, apathy, irritability, forgetfulness

and confusion, accord ing to the Merck Manual of Medical Information.

The symptoms accelerate usually much more rapidly than in Alzheimer's

disease.

Muscle twitching usually appears in the first six months after symptoms

begin. Trembling, clumsiness and peculiar body movements also may

develop, according to the Merck Manual.

No cure exists for the disease, and its progress cannot be slowed. Death

usually occurs about three months to a year after the onset of illness.

The Miami man first showed symptoms of the disease last fall when he

started becoming forgetful, said a family member who did not want her

name used.

He would go out on business but couldn't find his way home, or he would

forget where he had placed things just five to 10 minutes after setting

them down, the Miami woman said. Some of his other symptoms have

included weight loss, incontinence and not feeling pain, she added. He

is be ing treated for the disease at a St. Louis hospital.

" Sometimes he can make up conversations with you, and sometimes there's

a blank that comes over him, and he's spaced out . . . He's not coherent

at all, " she said.

Creutzfeldt-Jakob disease, or CJD, primarily affects adults,

particularly those in their late 50s. A disease similar to

Creutzfeldt-Jakob disease occurs in sheep and goats (scrapie), elk and

deer (chronic wasting disease) and cattle (mad cow disease).

In recent years, breakouts of mad cow disease in Britain have forced the

slaughter of vast numbers of cattle. The disease leaves the brains of

infected animals spongy and riddled with gum-like strings.

Though several young people in Britain contracted a human form of the

fatal disease amid breakouts of mad cow disease, to date there has been

no conclusive evidence linking their disease to eating beef, according

to reports.

No case of mad cow disease (bovine spongiform encephalopathy) has ever

been identified in cattle in the United Sates, despite active

surveillance efforts for several years.

Though the Miami man reportedly was a deer hunter, it is unknown how he

contracted the disease.

The disease is thought to be caused by a subvirus infectious protein

particle called a prion. It is unclear how these protein particles are

transmitted or cause neurologic disease.

" This is an organism that has a predilection for the central nervous

system. So people who are exposed to body tissues of the central nervous

system of something else that is infected are at increased risk of

contracting it, " said Crutcher.

The progressive disease generally has a long incubation period of months

or years.

The majority of cases in humans are sporadic and no one knows what

causes the disease.

About five to 10 percent of the cases are inherited.

A few people have acquired the disease from receiving contaminated

corneal implants or possibly other tissue transplants from infected

donors, according to the Merck Manual.

Also, in the past, the disease has been associated with people who have

received growth hormones prepared from a cadaver's pituitary gland.

Synthetic growth hormone, however, is now available.

end98 This report includes some information from the New York Times News

Service.

Laurie Winslow can be reached at 581-8466.

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