Healthdirect Free Australian health advice you can count on.

Medical problem? Call 1800 022 222. If you need urgent medical help, call triple zero immediately

healthdirect Australia is a free service where you can talk to a nurse or doctor who can help you know what to do.

beginning of content

Creutzfeldt-Jakob disease

4-minute read

What is Creutzfeldt-Jakob disease?

Creutzfeldt-Jakob disease (CJD) is a very rare and serious disease. It causes the brain to degenerate and become spongy, leading to dementia and death. About 40 to 50 people die of CJD each year in Australia.

It happens when infectious particles called prions damage brain cells.

CJD belongs to a group of rare diseases known as transmissible spongiform encephalopathies (TSE) or prion diseases. These diseases can affect humans and animals. The best-known form of TSE is bovine spongiform encephalopathy (BSE), or ‘Mad Cow’ disease.

What are the symptoms of Creutzfeldt-Jakob disease?

CJD takes years to show up, and the first symptoms are vague, often not appearing till the age of 60. Early signs and symptoms of CJD include:

  • changes in personality and behaviour
  • problems with memory and concentration
  • problems with thinking
  • confusion
  • problems with speaking and swallowing
  • double vision or blindness
  • deafness
  • depression
  • muscle spasms
  • seizures
  • balance and mobility problems
  • weakness
  • headaches
  • constipation
  • incontinence
  • difficulty recognising people

In people who have variant CJD, changes in personality seem to be more prominent in the beginning, with dementia developing later in the illness.

Most people with CJD decline quickly towards the end of their life.

What causes Creutzfeldt-Jakob disease?

There are a few different ways to develop CJD:

  • Sporadic CJD occurs for no obvious cause. This is very rare, estimated to affect one in 1 million people per year.
  • Genetic or familial CJD is inherited.
  • Medically acquired CJD is spread accidentally during brain or cornea surgery if medical staff use contaminated instruments. Some people have contracted CJD after receiving transplants of diseased human growth hormone or tissue or after blood tranfusions.
  • Variant CJD is caused by eating meat from cattle that had mad cow disease. It can also be transmitted through blood and blood products. Most cases of variant CJD occurred in the UK in the 1990s. Neither mad cow disease nor variant CJD have been found in Australia.

Creutzfeldt-Jakob disease does not spread from person to person through kissing, sneezing, coughing or body contact.

When should I see my doctor?

The risk of getting CJD is very low in Australia. It is not an infectious disease.

You should see a doctor if you have a lost a family member to CJD. If you have been told you are at increased risk of developing CJD, always tell your doctor or dentist before you have a procedure as they will need to follow infection control guidelines.

How is Creutzfeldt-Jakob disease diagnosed?

Creutzfeldt-Jakob disease can be confirmed only by doing special tests on brain tissue. Often these tests are done during an autopsy, which is performed after the person affected dies.

If someone is showing signs of CJD such as rapid dementia and neurological symptoms, they may have an electroencephalogram (EEG), magnetic resonance imaging (MRI) or a lumbar puncture to rule out another cause of their symptoms.

How is Creutzfeldt-Jakob disease managed?

There is no treatment or cure for CJD. However, research for a cure is continuing. Treatment is aimed at relieving symptoms.

Can Creutzfeldt-Jakob disease be prevented?

There is no way to prevent sporadic CJD. To assess your risk of genetic CJD, you could talk to a genetics counsellor, especially if there are people in your family with diseases that affect the brain.

Medically acquired CJD is very rare in Australia. People who lived in the UK for more than 6 months between 1980 and 1996, or who received a transfusion or injection of blood products in the UK since 1980, aren't allowed to donate blood, because of the slight risk that they might have picked up the infection. Blood banks also don’t accept blood from anyone who is at risk of CJD, for example if someone in their family had inherited CJD.

Complications of Creutzfeldt-Jakob disease

CJD is always fatal. Most patients die within a year of the symptoms starting, and only a small number survive for more than 2 years.

Resources and support

For more information, you can contact the CJD Support Group Network on 1800 052466.

Learn more here about the development and quality assurance of healthdirect content.

Last reviewed: March 2020

Back To Top

Need more information?

These trusted information partners have more on this topic.

Top results

Creutzfeldt - Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a rare degenerative disease that affects brain tissue in humans.

Read more on Queensland Health website

Creutzfeldt-Jakob disease -

Creutzfeldt–Jakob disease (CJD) is a fatal, degenerative brain disorder that causes rapidly progressive dementia and loss of muscle control. It is a rare disease, affecting 1–2 people in every million in Australia.

Read more on myDr website

Creutzfeldt-Jakob Disease - Brain Foundation

Creutzfeldt-Jakob Disease Read more at Virtual Medical Centre Description Creutzfeldt-Jakob disease (CJD) is a rare brain disorder

Read more on Brain Foundation website

Creutzfeldt-Jakob disease (CJD) factsheet - Fact sheets

A factsheet about Creutzfeldt-Jakob disease which is a rare brain disease that is caused by abnormal brain proteins called prions.

Read more on NSW Health website

Support organisations for rare diseases including genetic disorders

An A-Z Support Directory of support organisations for rare diseases including genetic disorders. Search for a rare disease.

Read more on Rare Voices Australia website

Dementia (Non-Alzheimer type) - Brain Foundation

Dementia (Non-Alzheimer type) (See also Alzheimers Disease) Description The term dementia is used to describe the pattern of deteriorating intellectual function particularly (although not exclusively) in the elderly that can occur as a result of various neurological disorders

Read more on Brain Foundation website

How pathology keeps our blood supply pumping | Know Pathology Know Healthcare

How pathology keeps our blood supply pumping Aug 21, 2015 0 Comment Post By:Annette Stenhouse No one wants to be in a situation where they need a blood transfusion but unfortunately many people need donated blood in Australia every day

Read more on Know Pathology Know Healthcare website

Growth hormone - Better Health Channel

Read more on Better Health Channel website

Organ donation -

Organ and tissue donation saves the lives of hundreds of Australians every year. Find out who can donate organs and how to register your decision on the Australian Organ Donor Register

Read more on myDr website

'Mad cow disease' - why you can't donate blood, breast milk and tissues | Queensland Health

People who lived in the UK for six months or more between 1980 and 1996 are ineligible to donate blood, breast milk and tissues in Australia.

Read more on Queensland Health website

Healthdirect 24hr 7 days a week hotline

24 hour health advice you can count on

1800 022 222

Government Accredited with over 140 information partners

We are a government-funded service, providing quality, approved health information and advice

Australian Government, health department logo ACT Government logo New South Wales government, health department logo Northen Territory Government logo Government of South Australia, health department logo Tasmanian government logo Victorian government logo Government of Western Australia, health department logo

Healthdirect Australia acknowledges the Traditional Owners of Country throughout Australia and their continuing connection to land, sea and community. We pay our respects to the Traditional Owners and to Elders both past and present.