Pin to desktop Australian gets fatal ‘one-in-a-million’ brain disease

An Australian has been diagnosed with a deadly “one-in-a-million” degenerative brain condition, but authorities Wednesday stressed it was unrelated to mad cow disease and not contagious.

The man, named by the media as 63-year-old Frank Burton, is in a serious condition in hospital with a likely case of “classical Creutzfeldt-Jakob Disease (CJD)”, a spokesman for Sydney Local Health District told AFP.

Burton — a former chief financial officer for Aussie Rules football team Sydney Swans — was told on Friday he had three months to live but this has now been reduced to two or three weeks, his friend Peter Kogoy said.

“(He has gone) from having a head of dark, straight (hair) and black beard, to totally white, totally white,” Kogoy told the Australian Broadcasting Corporation.

“A total loss of speech and total loss of movement in his limbs in a matter of weeks and days.”

“Variant CJD” emerged in Britain in the late 1980s and 1990s and was linked to mad cow disease, officially known as bovine spongiform encephalopathy (BSE), which causes a brain-destroying disorder in humans.

But the Sydney health district said “classical CJD” was not linked to mad cow and there have been no cases of BSE in Australia.

It added that visitors and patients at the Sydney hospital were not at risk.

“Classical CJD is an extremely rare disease. The chance of someone in the community having the disease is around one in every one million people,” the health district said in a statement.

“Most cases of CJD occur because of mutations with a person’s brain and are not spread from other people.

The causes of death are usually infection, heart failure, or respiratory failure.