'Mad cow' disease claims a third victim in france

April 30, 2001 The Toronto Star

PARIS (AP-Reuters) - A French teenager believed to have been suffering from the human variant of "mad cow" disease has died after slowly losing the ability to walk, speak and breathe.

Arnaud Eboli, 19, died late Tuesday after fighting the brain-wasting ailment for more than two years, his family said.

His death marks France's third fatality from new-variant Creutzfeldt-Jakob disease (nvCJD), which is linked to the consumption of tainted beef.

In Britain, where "mad cow" was identified in 1995, 90 people have died of the disease.

Eboli, once an athlete who excelled at skiing and martial arts, lost the ability to bathe or feed himself. Before he died, he was paralyzed and kept alive through a feeding tube.

"He died in appalling conditions. He looked like an old man," his mother Dominique Eboli said yesterday.

Doctors diagnosed Eboli in December, 1999, after a biopsy of his tonsils detected traces of an infectious protein, prion, often found in people suffering from the disease.

The disease can only be confirmed by a brain biopsy, usually after death, and Eboli's body has been transferred to the Pitie-Salpetriere hospital in Paris for an autopsy.

The Ebolis were one of two French families that filed a lawsuit in November charging that French, British and European Union authorities did not act quickly enough to wipe out "mad cow" disease, or bovine spongiform encephalopathy (BSE).

The suit alleges that Eboli and Laurence Duhamel, who died in 1999 at age 36, were victims of poisoning and manslaughter.

The Eboli family has also campaigned for health authorities to provide guidance and assistance to families struggling to come to grips with the little-understood illness.

"We insisted on keeping him at home, because this disease is too atrocious," Dominique Eboli said.

"We held his hand until the end. We knew he was going to die, but today it's very hard. It's emptiness."

Arnaud's illness started in September of 1998 with hysteria. He began smashing chairs and dishes in fits of rage, behaviour that doctors told his parents was only adolescent frustration.

"He would break things all over the house. He fought with us all the time," said Dominique Eboli.

The hysteria and mood swings subsided a year later.

Then, Arnaud lost the ability to walk and speak.

He could no longer bathe or feed himself. Sometimes his eyes bulged; sometimes one eye stayed shut. Doctors called it "irreversible and premature dementia."

Doctors delivered their diagnosis on Christmas Eve, 1999, after the tonsil biopsy detected the infectious protein prion.

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

Six months ago, the once-vibrant and handsome teen lay paralyzed, barely conscious and kept alive through a feeding tube.

News of Eboli's death came as the French government urged town councils to put beef back on the menu in schools and communal canteens, insisting that it was safe to eat.

France has taken drastic measures - such as outlawing certain at-risk cuts of beef, like the T-bone steak - to try to safeguard public health.

"All scientific studies to date have shown that the BSE agent is not present in the muscle of the animal," a letter to mayors signed by five ministers said. "Since commercialized beef consists of muscle, it can be eaten without fear."

New cases of the animal ailment are expected to break out in France until 2002 - five years after agriculture authorities took rigorous measures to prevent more outbreaks.

About 150 cows were discovered with the disease in France last year, compared to 31 the year before.

Meanwhile, medical experts are predicting more elderly people will fall victim to the disease after a 74-year-old man in Scotland became the oldest victim of the fatal brain affliction.

New-variant Creutzfeldt-Jakob disease was diagnosed after an autopsy was requested for the man on the basis that some of his symptoms were not associated with dementia and he died just seven months after they began.

Professor James Ironside of the CJD Surveillance Unit at Western General Hospital in Edinburgh said the man's death is unlikely to be an isolated event, and predicted that more people in their 50s, 60s and 70s would also succumb.

Most of the 95 cases of the brain-wasting illness reported in Britain have been in people decades younger than the oldest victim.

The Edinburgh doctors said in a letter to The Lancet medical journal that the latest case has important implications for the surveillance of nvCJD, and raises the possibility that cases in the elderly might be missed.

Symptoms of the illness, which include loss of co-ordination, confusion and personality changes, can be mistaken for dementia in older people.

The elderly man, a retired electrician, had no family history of brain disease and was healthy until he complained of pains in his hands. He then became forgetful and started having hallucinations and paranoid delusions.

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