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Mad Cow Variant That Hits Humans Is a Puzzle

January 3, 2004 Washington Post by Rob Stein
She had just graduated from college when the frightening symptoms started to surface. The vivacious, easygoing 22-year-old suddenly became inexplicably irritable and short-tempered. Then her memory started failing. Her right hand began to tremble. Her leg started to shake, and her balance gave way.

"She just cried and cried and cried," said the Miami woman's father, who asked that his daughter be identified only by her first name, Charlene. "She knew she was sick, but she didn't know what it was."

Within six months, Charlene was incoherent and helpless, unable to speak or control her bodily functions. Today, two years after the first symptoms appeared, she lies bedridden, her brain ravaged. She is fed through a tube in her stomach and has no hope of surviving.

"She went down very quickly. She lost awareness. She doesn't know where she is and who anyone is," said her father, Patrick. "Every day is hard."

Charlene is the only person in the United States confirmed to have the human form of mad cow disease, in which a misshapen infectious protein inexorably erodes the brain. She is believed to have contracted the insidious disease by eating infected beef during her first 13 years of life, when her family was living in Britain.

Charlene's story is the kind of tragedy that the United States is desperately trying to avoid after the discovery of the first U.S. cow carrying the infection.

"I'm terrified that this is going to happen again. It's like being in the U.K. again, watching this all over again," Patrick said. "I'm worried that people may be eating beef that is contaminated and that down the road people are going to start to die from this disease."

Government officials and many independent experts stress that even with the long-feared detection of an infected cow in this country, existing safeguards mean the risk that anyone eating U.S. beef will face Charlene's fate remains extremely remote. Agriculture officials announced drastic new measures this week in the hopes of erasing any possibility of danger.

But as experts debate the effectiveness of the new protections, try to figure out how the Holstein became infected and search for other contaminated cows that may be in the food supply, the discovery of the infected animal has focused attention on the many uncertainties about the illness and on how well the government monitors for homegrown cases.

"I think the occurrence of mad cow disease is something of a wake-up call that no matter how we insulate ourselves, we need to be increasingly vigilant and fill in whatever gaps there might be," said Raymond Roos of the University of Chicago, who studies the disease.

Worldwide, 153 cases of the human form of mad cow disease have been reported. The overwhelming majority -- 143 -- occurred in England, where the disease was first detected in 1996. Six cases have been documented in France, and one each has been reported in Italy, Ireland, Canada and the United States. All have been linked to the mad cow epidemic in England in the 1980s.

In cows, the disease is officially known as bovine spongiform encephalopathy (BSE) -- "spongiform" because the disease makes the brain look like a hole-riddled sponge. In humans, the disease is called variant Creutzfeldt-Jakob disease (vCJD). It is named after Creutzfeldt-Jakob disease (CJD), a similar but distinct brain malady first identified in Germany in 1920.

CJD is one of a few diseases caused by prions. Prions are strange, normally harmless proteins found in the brain that contort into a shape that makes them lethal to nerve cells. In CJD, which occurs in about 1 out of every 1 million people, the malformation occurs spontaneously for reasons that remain a mystery. Victims quickly and progressively lose their ability to think and control their bodies. It is untreatable and invariably fatal.

The disease almost always hits people in their fifties, sixties and seventies. In the late 1990s, however, British doctors noticed that unusual cases appeared to be striking people in their twenties and thirties. Scientists traced the baffling cases to meat from cows stricken by mad cow disease -- which is also caused by prions.


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