NSW health authorities are assuring the public that the variation of mad cow disease that has left a former Sydney Swans official clinging to life is not contagious.
Frank Burton, 63, is in isolation at the Royal Prince Alfred Hospital after being diagnosed with sporadic Creutzfeldt-Jakob disease (CJD), which belongs to a group of diseases that also includes mad cow disease.
Mr Burton was diagnosed with the rare, degenerative disease when he presented at RPA after collapsing on a footpath five weeks ago. His condition was made public on Tuesday.
Sporadic CJD is not caused by eating animals, Dr Jeremy McAnulty, the director of health protection at NSW Health, told Southern Cross Austereo on Wednesday.
What is Creutzfeld-Jakob disease?
– A rare, degenerative and fatal brain disorder.
– Usually kills within a year.
– Sporadic CJD is the most common type.
– Affects one in 1 million people a year, worldwide.
– About 20 cases a year in Australia.
Types of CJD
Until the 1990s, three forms of CJD were recognised:
– Sporadic: cause unknown; accounts for 85 to 90 per cent of all cases; mainly affects those aged 50 to 70.
– Inherited: associated with a gene mutation.
– Health-care acquired: caused by accidental transmission via contaminated instruments or certain transplants.
In the 1990s, a fourth form was recognised:
– Variant: vCJD develops when people eat meat from cattle infected with bovine spongiform encephalopathy, or mad cow disease; longer duration but symptoms very similar to classical CJD
Symptoms:
– Personality/behavioural changes.
– Vision problems.
– Confusion, which may advance to dementia.
– Loss of balance/muscle co-ordination.
– Difficulty walking.
– Muscle spasms.
– Symptoms are severe and progress rapidly.
Diagnosis:
– Brain biopsy or autopsy is required to confirm CJD
– Brain biopsies are not normally conducted, due to the risk to the patient
– Doctors rely on a range of scans to check electrical patterns in the brain and patterns of brain degeneration
Treatment:
– There is no treatment to cure or control CJD.
– Many drugs have been tested but none has shown any consistent benefit.
– Current treatment is aimed at alleviating symptoms and keeping a patient comfortable.
(Sources: World Health Organisation/CJD Support Group Network/National Institute of Neurological Disorders and Stroke)