The Florey Institute has been armed with a further $3 million in Australian Federal Government funding to combat the bogeyman of neurological disease – misfolded proteins called prions.
Creutzfeldt-Jakob disease (CJD) is a fatal but rare neurodegenerative disease caused by misfolded proteins in the brain. It results in the deaths of about 50-60 people in Australia every year.
The disease is difficult to monitor and detect, as it can remain dormant within an affected individual for years to decades before activating to devastating and always fatal effect.
In search of a cure
The Florey Institute’s Australian National CJD Registry (ANCJDR) provides comprehensive diagnostic, surveillance, research and epidemiological services for all forms of human prion disease.
The registry’s co-director neurologist Professor Steve Collins said the $3 million in funding was a substantial boost that would enable the registry to meet growing demand for diagnostic and other services while searching for a cure.
“These are devastating diseases,” Professor Collins said, “There is no cure and sadly patients grow steadily more ill until death. About 15% of cases are genetic and the rest are without apparent cause often occurring like a bolt from the blue.”
In 2022, The Florey Institute received 599 diagnostic referrals. In 2023, that number rose to 651 cerebrospinal fluid specimens from around Australia, reflecting the increased awareness of prion disease and CJD in Australia.
Vitally important monitoring, advice and care
Registry coordinator Dr Christiane Stehmann said the ANCJDR was launched in 1993 when four young women died after receiving CJD-contaminated hormone fertility treatments that potentially put many more lives at risk.
“The Australian National CJD Registry has become a valuable resource for doctors and health departments,” Dr Stehmann said. “We have also formed close ties within the global CJD surveillance and research community and Australia’s CJD Support Group Network.”
She said the registry’s role was about much more than just monitoring and studying a disease.
“It’s about helping people and making a difference. Our team of nine dedicated scientists and doctors is proud that we contribute to the knowledge of this rare disease.”
Minister for Health and Aged Care Mark Butler said the Government was proud to support the important work of Florey researchers.
“The Albanese Government is investing $3 million with The Florey as part of our commitment to championing Australia’s best and brightest minds in our health and medical research sector, to conduct vital public health surveillance and epidemiology research for CJD, ensuring timely and effective infection control, surveillance, and response to prion disease in Australia,” he said.