Victims of Human Variant of Mad Cow Disease Suffer Slow Death

When 17-year-old Arnaud Eboli began smashing chairs and dishes in fits of rage two years ago, doctors told his parents it was only adolescent frustration.

The hysteria and mood swings subsided a year later. But then, Arnaud lost the ability to walk and speak. Today, the once-vibrant teen lies paralyzed, barely conscious and kept alive through a feeding tube.

Doctors say he suffers from a variant of Creutzfeldt-Jakob Disease, the human version of mad cow disease. The ailment caused panic across France when it became known last month that potentially contaminated beef had reached supermarket shelves. The fear was fueled by a television special that graphically showed the deterioration of human victims, notably Arnaud.

Creutzfeldt-Jakob is commonly described as a "brain-wasting" illness. Families of two victims told The Associated Press the frightening reality of what that means.

In Arnaud's case, the disease transformed a soft-spoken, handsome athlete who excelled at skiing and martial arts into a limp bag of bones. It started in September 1998 with hysteria.

"We couldn't control him, he would break things all over the house. He fought with us all the time," said his mother, Dominique.

Anger, agitation and depression lasted nearly a year symptoms doctors identified as "normal adolescent behavior," said Mrs. Eboli, 43. "I knew that was wrong."

By September 1999, Arnaud stumbled when he walked, his memory was impaired and speaking took great effort.

"It was as if his mouth was full of food and he couldn't push the words out," his mother said. Arnaud could no longer bathe or feed himself. Sometimes his eyes bulged; sometimes one eye stayed shut.

New doctors called it "irreversible and premature dementia," his mother recalled.

A month later, Arnaud was hospitalized for tests.

Doctors delivered their diagnosis last Christmas Eve, after a biopsy of Arnaud's tonsil detected traces of an infectious protein, prion, often found in people with variant CJD. The disease can only be confirmed by a brain biopsy, usually after death, but studies have shown the illness can be detected in tonsil samples.

"They told us there was no treatment. No medicine. They told us he had 18 months," his mother said.

The Eboli family ate supermarket-bought beef once a week and said they never ate offal an animal's entrails, considered gourmet fare in France. Arnaud ate fast-food hamburgers roughly twice a week.

To calm public fears, France has pulled T-bone steaks and other potentially risky cuts of beef from the nation's markets. The marrow of infected animals can transmit the malady to humans and other animals.

France also banned the use of animal feed containing meat and bone meal from ground-up cow carcasses a suspected source of mad cow disease, or bovine spongiform encephalopathy.

Photographs of Arnaud taken this year show his dramatic decline.

In a snapshot taken in February he is frail, sitting knock-kneed in a wheelchair.

By summer, he had trouble holding his head up. Walking and talking were almost impossible.

"That's June," Arnaud's father, Eric, said softly of a picture of the two in the family's swimming pool. Arnaud, now 19, is curled up like a baby in his father's arms.

"He could only speak a few words, but do you remember what he said to you?" Mrs. Eboli whispered to her husband. "When we put him in the water, he liked it so much. He said, 'Thank you dad. Thank you mom."'

In the final stages of the illness, Arnaud sleeps constantly, though he is not clinically comatose. His once 165-pound frame has shriveled by half.

Not all victims have identical symptoms.

Laurence Duhamel died in February at age 36 after battling variant CJD for just over a year. She initially was sullen, her brother Jean recalled, then reclusive not wanting to leave the house she shared with her mother and sister in a Paris suburb.

She became paranoid, cried constantly and begged for her mother's help with bathing and other personal chores. But on other days she allowed no one to touch her. Then came the delusions.

"She thought she was pregnant. She told me she'd traveled to India, when I knew she had never left Paris," her brother said.

In May 1999, Duhamel's family admitted her to a psychiatric hospital. Three months later, after she lost control of her limbs, she was transferred to a general hospital, where doctors tested for brain disorders.

Duhamel stopped speaking and could no longer move. Suspecting variant CJD, doctors took a brain biopsy.

"In the last few months, I don't think there was any suffering," her brother said. "It was as if her body had already left her or her brain had already left her body." She died Feb. 4.

The families of both victims filed a lawsuit this month, accusing France, Britain and the European Union of not acting fast enough to stamp out mad cow disease. The family of France's only other known CJD fatality, who died in 1997, intends to join the lawsuit.

Duhamel never knew her diagnosis, but her brother said she suspected. "In the hospital, her hands would creep up her body until they reached her head. She would hold her head," Duhamel's brother recalled.

Arnaud, his mother said, had a premonition shortly after 1996, when the tainted beef scare first alarmed Europe.

"He said to me, 'Mom, we're all going to die of this one day."