Variant Creutzfeldt-Jakob disease

About variant Creutzfeldt-Jakob disease

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

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

In vCJD, prions spread to people through eating meat from cows with bovine spongiform encephalopathy (BSE), also called mad cow disease.

There are other types of Creutzfeldt-Jakob disease (CJD) that are not spread by eating beef.

Why it matters to public health

vCJD is a very rare but fatal disease.

vCJD and BSE have never been detected in Australia.

Australia’s strict biosecurity and food standards mean that beef sold in Australia is free from BSE.

Most cases of vCJD reported worldwide were linked to an outbreak of BSE in the United Kingdom in the 1990s. Since then, the UK has introduced strict controls to stop BSE spreading in cows and to prevent people eating affected beef.

The last reported cases of vCJD occurred in 2016.

Symptoms

Common early symptoms include:

depression
anxiety
numbness, tingling or painful sensations.

The disease progresses to include:

problems with walking
loss of control of movements
memory problems and confusion
dementia
inability to move or speak.

People usually die about a year after symptoms start.

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

Onset of symptoms

The time it takes for symptoms to start is not well understood, but it could be 10 years or more after eating contaminated beef.

How it spreads

Almost all people with vCJD were infected after eating beef from cows with BSE.

Although extremely rare, it has spread from one person to another through blood transfusion.

vCJD does not spread through household, social or sexual contact.

Infectious period

Blood and certain tissues are considered infectious when a person has symptoms, and sometimes before.

Prevention

Australia’s strong monitoring and control programs help keep cattle and beef free from BSE.

Healthcare facilities follow strict infection prevention and control guidelines to prevent potential spread.

Priority groups and settings

People at greater risk of severe disease

vCJD is always a serious disease.

It affects younger adults more often than older adults.

People at greater risk of exposure

People might be at greater risk of vCJD if they:

ate beef in the UK before 1996
received a blood transfusion before 2000
work with BSE or vCJD in a laboratory without appropriate protective measures.

Diagnosis and treatment

A vCJD 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 vCJD including:

collection of spinal fluid
an electroencephalogram (EEG)
magnetic resonance imaging (MRI) scan of the brain

In some situations, specialist testing might be required, including testing other body sites.

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

There is no cure for vCJD. People with vCJD receive supportive end-of-life care.

Surveillance and reporting

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

Data is collected through the Australian National CJD Registry.

This is part of our national surveillance to monitor for cases and disease patterns.

BSE is a nationally notifiable disease of animals. If it was detected, health and food safety authorities would investigate the risk to people.

Support

For more information about vCJD, see:

For information about vCJD 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.