A former chief financial officer of the Sydney Swans AFL club, Frank Burton, has been told he has weeks to live after being diagnosed with the rare and fatal brain disorder Creutzfeldt-Jakob disease (CJD).
Doctors say the the rare and degenerative disorder affects about 20 Australians each year.
The veteran sports journalist Peter Kogoy, a friend of Burton’s, told the ABC the diagnosis came after Burton suffered from a sudden difficulty with speech, which progressed to a total loss of movement in his limbs in a matter of weeks.
“He’s fighting this to the bitter end,” Kogoy told the ABC, saying that friends and family were keeping vigil by his bedside at Sydney’s Royal Prince Alfred hospital.
Creutzfeldt-Jakob disease usually appears later in life and kills 90% of people within one year.
Kogoy has told the media that Burton had been diagnosed with the sporadic form of the disease, thought to be caused by a gene mutation, which suddenly appears and accounts for 85% of cases.
About 5-10% of cases are a rarer, hereditary form of CJD, while a small percentage of people get what is known as acquired CJD, which is transmitted through exposure to brain or nervous system tissue.
Variant CJD, sometimes called “mad cow disease”, is a different disease from the other three forms of CJD.
Professor Colin Masters, one of Australia’s leading experts in CJD, said it was important to recognise that mad cow disease was a separate class of CJD to that which Burton had been diagnosed with.
While doctors had learned a lot about the molecular changes that occurred in the brain as a result of sporadic CJD, he said, “why they start, and occur at a rate of one in a million in the population, is still pretty much unknown”.
This made it difficult to treat, he said.
A professor of infectious diseases with the Australian National University, Peter Collignon, said there was no evidence CJD could be transmitted to other people by casual contact.
“It needs exposure to neurological tissue to be transmitted, for example via neurosurgery or tissue transplantation, or pituitary hormone extracts,” he said.
Mad cow variety could be transmitted by oral route from some foods, he said.
