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New form of brain-wasting disease identified

14.07.2008 12:00 Health - Source: cbc.ca

Researchers in the U.S. have identified the emergence of a new type of brain-wasting disease that resembles Creutzfeld-Jakob, the human form of mad cow disease.

Similarly to Creutzfeld-Jakob disease (CJD), the human variant of bovine spongiform encephalopathy, the new disease causes the brains of sufferers to fill with tiny holes, robbing them of the ability to think, speak and move.

The disease has not been given a name.

In the U.S., it has been found in 16 people since 2002, 10 of whom have died of it.

Cases of the disease were first described in the Annals of Neurology in 2006 and are discussed in an article in the June 20 issue of the journal and in a July 9 article in New Scientist.

"I believe the disease has been around for many years, unnoticed," Pierluigi Gambetti, director of the U.S. National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio, said in a news release Wednesday.

It's believed that excessive amounts of prions, misfolded forms of a brain protein, lead to breakdown of brain tissue in both types of brain-wasting diseases. In the case of CJD, prions are not broken down by enzymes, but in the new disease, they are.

Researchers aren't sure what causes the disease.

"I believe the disease occurs naturally, and is not due to environmental causes," says Gambetti. He plans to further study possible causes using lab mice.

It's not clear what causes classic CJD. Humans can contract a variant of CJD, called acquired CJD, by consuming brain material of an animal with mad cow disease or through exposure to medical instruments that have been improperly sterilized.

About 30 cases of classic CJD turn up in Canada per year.

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