The Human Face of Mad Cow Cases

January 6, 2004

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, fed through a tube in her stomach, with no hope of surviving.

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 known as a prion inexorably erodes the brain. She is believed to have caught the 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.

Government officials and many independent experts stress that even with the detection of an infected cow, existing safeguards and new measures mean the risk that anyone eating U.S. beef will face Charlene's fate remains extremely remote.

"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.

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. All have been linked to the mad cow epidemic that swept England in the 1980s.

The human form of mad cow disease is called variant Creutzfeldt-Jakob disease (vCJD), named after Creutzfeldt-Jakob disease (CJD), a similar but distinct malady identified in Germany in 1920.

The disease almost always hits people in their 50s, 60s and 70s. In the late 1990s, however, British doctors noticed that unusual cases appeared to be striking people in their 20s and 30s. Scientists traced the baffling cases to meat from cows stricken by mad cow disease.

In 1996, Britain faced the possibility of a devastating epidemic of the first prion disease known to be spread by eating infected animals. U.S. officials began monitoring the incidence of CJD in this country and started investigating unusual cases. The key was to differentiate between CJD and vCJD.

The first symptoms of CJD are usually memory lapses and difficulty standing, walking and talking. Victims deteriorate rapidly, becoming bedridden in weeks or months and dying, usually within a year. In contrast, vCJD patients tend first to develop psychiatric symptoms, depression, anxiety, withdrawal, and severe pain or tingling and burning sensations in the legs before they begin to lose the ability to stand, walk and talk. It often stretches two to four years before death.

Magnetic resonance imaging can frequently distinguish vCJD from CJD. Sometimes the prions can be found in the tonsils of vCJD victims, as was the case with Charlene. An electroencephalogram can sometimes spot a distinctive brain wave pattern.

But the only definitive diagnosis is done by a detailed examination of the brain after death. In the past six years, doctors have shipped samples from the brains of 732 patients who have died from suspicious diseases to the National Prion Disease Pathology Surveillance Center set up at Case Western Reserve University in Cleveland by the Centers for Disease Control and Prevention.

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