Diseases and surveillance

Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease (CJD) is a rare degenerative brain disease. It is always serious. Healthcare facilities follow strict procedures to prevent its spread.

At a glance

About Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative brain disease.

It is caused by infectious proteins – called prions – that cause the brain to appear ‘spongy’.

There are different types of CJD, including:

  • familial (genetic) – when it runs in families
  • medically acquired – when it is spread during a medical procedure
  • sporadic – when the disease appears without an identified cause or familial link.

There is another type of CJD, called variant Creutzfeldt-Jakob disease, which is very rare and has never been detected in Australia.

Why it matters to public health

CJD is rare – over the past 10 years, an average of 61 cases per year were reported in Australia. It is a fatal disease.

Most cases of CJD are sporadic with no known cause. About 1 in 10 cases is genetic.

Medically acquired CJD is very rare. Only 9 cases have ever been reported in Australia – the most recent in 2000. Healthcare facilities follow strict guidelines to prevent medically acquired CJD.

Symptoms

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Symptoms of CJD progress rapidly to dementia.

Early symptoms include:

  • changes in mood and behaviour
  • poor concentration and memory
  • confusion.

Later symptoms include:

  • blindness
  • unsteadiness
  • muscle stiffness and jerking movements
  • unclear speech
  • hallucinations (seeing or hearing things that don't exist)
  • inability to move or speak.

Once symptoms begin, CJD progresses rapidly and usually leads to death within months.

Read more about the symptoms, diagnosis and treatment of CJD.

Onset of symptoms

The time it takes for symptoms of sporadic CJD to develop is not well understood, but it could be up to several decades.

For medically acquired CJD, the time to symptom onset depends on the exposure. It can range from about 16 months to 30 years.

How it spreads

Familial CJD is genetically passed from parents to children.

Sporadic CJD happens without an identified cause or familial link.

Medically acquired CJD happens during a medical procedure when:

  • contaminated instruments are used during brain surgery
  • someone receives affected human-derived tissues or hormones.

The disease can only spread via certain human tissues or hormones, including:

CJD cannot spread through household, social or sexual contact.

Infectious period

The brain and spinal cord tissues, optic nerve and retinal tissues, and pituitary hormones of a person with CJD are infectious when the person has symptoms.

It is unknown if tissues are infectious before symptoms occur, but some evidence suggests certain tissues may be.

Prevention

Medically acquired CJD is prevented in Australia through strict controls and guidelines, including:

  • infection control steps in hospitals and clinics
  • careful handling of medical tools and treatments so contaminated ones are not used.
  • manufacturing many medical products that were once made from human tissues (such as hormones) in laboratories.

Because of this, the risk of medically acquired CJD in Australia is very low.

Priority groups and settings

People at greater risk of severe disease

CJD is always fatal.

Most people who develop CJD are aged 50 to 70 years.

People at greater risk of exposure

Most cases of CJD happen without an identified cause or familial link.

Medically acquired CJD is very rare. But people might be at greater risk if they have been exposed to certain human tissues or fluids.

Diagnosis and treatment

A CJD diagnosis can only be confirmed by testing a sample of brain tissue after death.

During the illness, people might have other tests to help make a diagnosis of probable CJD including:

These tests help to exclude other diseases which may cause similar symptoms to CJD.

There is no cure for CJD. People with CJD need supportive end-of-life care.

Read more about how CJD is managed.

Surveillance and reporting

CJD is a nationally notifiable disease – these are diseases that present a risk to public health.

The Australian CJD registry, managed by the Florey Institute, monitors CJD which complements the NNDSS data.

Health authorities in each state and territory report new cases to the Florey Institute to monitor case numbers and disease patterns.

Support

For more information about CJD, see:

For information about CJD in your state or territory, see:

If you need mental health support, see a list of organisations, websites and services that offer support, counselling and information.

Last updated:
Disease groups:
  • New or rare threats