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Woman first to die in Italy of mad cow disease

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Mayo Clinic
  • Mad cow disease was first reported in the United Kingdom in 1986, peaking in 1993 with almost 1,000 new cases per week. 
  • In 1996, variant Creutzfeldt-Jakob Disease (vCJD) was detected in humans and linked to the mad cow epidemic. Eating contaminated meat and cattle products is presumed to be the cause.
  • Both are fatal brain diseases with unusually long incubation periods, often lasting years.
  • To date, no case of mad cow disease has been identified in the United States.
  • As of April 2, 2002, a total of 125 cases of vCJD had been reported in the world: 117 from the United Kingdom, six from France, and one each from Ireland and Italy.
    Source: CDC
  • ROME, Italy (CNN) -- A 27-year-old woman died Wednesday of the human form of mad cow disease, marking the first Italian death blamed on the illness that has claimed more than 100 lives in other parts of Europe.

    Dr. Enrica Alessi, spokeswoman for the Besta Hospital in Milan, Italy, said the woman had been sick for a year and a half, and the illness may have incubated in her body for years.

    Tests showed she got the disease from eating contaminated beef, Alessi said, but it was not clear whether the woman ate the contaminated beef inside Italy or during her travels outside the country.

    An autopsy to be conducted in the next few days may provide more information, she said.

    Doctors have suspected that previous deaths in Italy were caused by variant Creutzfeld-Jacob Disease, the human form of bovine spongiform encephalopathy, often called mad cow disease. Those suspicions were never proved true.

    In January 2001, Italy reported the first case of mad cow disease in Italian cattle.

    The devastating illness often starts with a bout of depression, progressively cripples the brain and always results in death.

    The World Health Organization says human sufferers of the disease usually experience psychiatric symptoms early in the illness, which most commonly take the form of depression and anxiety or less often, a schizophrenia-like psychosis.

    Neurological problems like unsteadiness and involuntary movements usually follow and shortly before death, patients become completely immobile and mute.

    CNN Producer Sherri Jennings contributed to this report.

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