What is Creutzfeld-Jakob disease?

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. Sporadic CJD is not caused by eating animals, Dr Jeremy McAnulty, the director of health protection at NSW Health, told Southern Cross Austereo.

 

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.

The Age, 8 July ;http://www.theage.com.au ;