Mad cow disease concerns allayed after rare diagnosis

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   Frank Burton

Health authorities have urged the New South Wales public not to be concerned after a man was diagnosed with an extremely rare and fatal condition similar to mad cow disease.

Frank Burton, a former financial officer of the Sydney Swans, has been given only weeks to live after being diagnosed with Creutzfeldt-Jakob Disease (CJD).

Health Protection NSW director Dr Jeremy McAnulty said CJD was caused by an abnormality in the brain and was not mad cow disease, which can be caught from eating infected meat.

“CJD [is] a rare disease that occurs around a world. In Australia we expect to see 20 to 30 cases a year,” he said.

“It’s normally caused by an abnormality, a mutation that develops in a person’s brain that causes changes in the protein that leads to a disease that’s quite debilitating and fatal.”

He stressed that there were no recorded cases of mad cow disease in Australia.

“I should also say that we’re not talking about mad cow disease [which was] an outbreak of disease in cattle in UK,” he said.

“Some decades ago in the 80s and 90s people who ate those animals very rarely developed a human form of the disease known as variant CJD.

“We’ve never seen any cases of that in Australia, in animals or people so there’s no public health implications from this particular case and we wish our best to the family and the patient.”

Dr McNaulty also reassured NSW residents that CJD was not infectious.

“There are various forms of CJD. The most common one is called sporadic or classical,” he said.

“It’s not transmitted, people don’t acquire it usually from other people. It’s not in the course of usual events, transmissible from person to person.”

Peter Kogoy said his close friend Mr Burton was told on Friday he had three months to live, but now that timeframe has been accelerated to two or three weeks.

He said Mr Burton was diagnosed “with something unusual” by his doctor just over five weeks ago and that there was a rapid progression of the disease and subsequent tests revealed it was sporadic CJD.

“[He has gone] from having a head of dark, straight [hair] and black beard, to totally white, totally white,” Mr Kogoy said.

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

What is Creutzfeldt-Jakob Disease?

  • Creutzfeldt-Jakob Disease (CJD) is a rare, degenerative, fatal brain disorder. Sporadic CJD, the most common variety, accounts for 85 per cent of cases
  • CJD causes rapidly progressive dementia. Symptoms include impaired muscular coordination, memory and vision and personality changes
  • Sufferers lose the ability to move or speak and enter a coma before death
  • Life expectancy is usually less than a year
  • It can also be hereditary or caused by infection
  • Variant Creutzfeldt-Jakob Disease (vCJD) emerged in the UK in 1980s and is linked with the consumption of beef contaminated with the Bovine Spongiform Encephalogpathy, or mad cow disease
  • vCJD appears in people younger than typical CJD patients and life expectancy is usually 12 to 14 months after diagnosis
  • Both CJD and vCJD belong to a family of diseases known as transmissible spongiform encephalopathies, meaning the brain becomes filled with holes, resembling a sponge

Sources: National Institute of Health and World Health Organisation