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 every one million people per year, worldwide
- About 20 cases a year in Australia
TYPES OF CJD: four
Until the 1990s, three forms of CJD were recognised:
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- 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 coordination
- 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)
AAP
What is Creutzfeld-Jakob Disease?
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