Mad Cow Disease in Spain "Kills 2 People"

Two people have died of mad cow disease in Spain, reports say.

The death of two youngsters in Castilla y León has been confirmed today to have been caused by Creutzfeld-Jakob, Mad Cow disease. Experts in Spain now consider that more cases are likely to be detected here over the next few months.

Juan José Badiola director of the centre which investigates the disease in Spain has called for calm. He said that both the victim probably ate the infected meat more than eight years ago.

Bovine spongiform encephalopathy (BSE), commonly known as mad-cow disease, is a fatal, neurodegenerative disease in cattle, that causes a spongy degeneration in the brain and spinal cord. BSE has a long incubation period, about 4 years, usually affecting adult cattle at a peak age onset of four to five years, all breeds being equally susceptible.  In the United Kingdom, the country worst affected, 179,000 cattle were infected and 4.4 million killed as a precaution.

It is believed, but not proven, that the disease may be transmitted to human beings who eat infected carcasses. In humans, it is known as new variant Creutzfeldt-Jakob disease (vCJD or nvCJD), and by June 2007, it had killed 165 people in Britain, and six elsewhere with the number expected to rise because of the disease's long incubation period. Between 460,000 and 482,000 BSE-infected animals had entered the human food chain before controls on high-risk offal were introduced in 1989.