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'Mad cow' suspected in death of 46-yr-old Sicilian man

Body sent to Ragusa for analysis

05 February, 14:31
'Mad cow' suspected in death of 46-yr-old Sicilian man (ANSA) - Caltanissetta, February 5 - The human variant of mad cow disease is believed to have killed a 46-year-old man in a hospital in central Sicily, a Sicilian newspaper reported on Wednesday.

If doctors' suspicions are confirmed, it will mark Italy's third documented death from variant Creutzfeldt-Jacob Disease (vCJD). Italy's first was a Sicilian woman who died in 2003, and its second was a 44-year-old woman in Livorno who died in January 2011. "The age of the patient and the rapid evolution of symptoms led us to investigate this case," said Michele Vecchio, chief of the neurological department of the Caltanissetta hospital, where the man was treated during the last weeks of his life.

The man's symptoms of memory loss and impaired motor control became evident just one month ago, reported Giornale di Sicilia.

Vecchio sent the patient's body to Ragusa for an autopsy at the request of the Italian National Institute of Health, where samples of brain tissue and other nervous system tissue will be taken and analyzed. The variant of CJD, for which there is no known cure, is believed to be caused by eating meat infected with bovine spongiform encephalopathy (BSE).

"This could be the variant related to bovine spongiform encephalopathy," Vecchio confirmed.

Both vCJD and BSE are caused by an infectious agent called prions. Prions are misfolded proteins that reproduce by converting properly folded proteins in their host to their own misshapen structure. The disorder causes the brain to develop holes and a sponge-like texture. There have been at least 275 cases of vCJD worldwide over the last thirteen years, with Britain being the worst-affected country.

Almost 190,000 cattle have died from the bovine form in the same period, the vast majority in the UK.

A massive cull in Britain prevented the spread of the disease but isolated cases are still reported.

EU restrictions on people eating beef-off-the-bone and offal were lifted in 2006 as the 'mad cow' crisis was declared officially over.