What is Creutzfeld-Jakob Disease?

WHAT IS CREUTZFELD-JAKOB DISEASE (CJD)?

* 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:

- 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)