Creutzfeldt-Jakob disease and variant Creutzfeldt-Jakob disease - including symptoms, treatment and prevention
These are progressive fatal infections of the brain caused by an infectious protein particle called a prion. These diseases are some of a group of brain infections known as Transmissible Spongiform Encephalopathies (TSE).
Creutzfeldt-Jakob disease and variant Creutzfeldt-Jakob disease are notifiable conditions1
How Creutzfeldt-Jakob disease is spread
Creutzfeldt-Jakob disease (CJD) is a very rare illness, affecting about one person per million population worldwide, and has been recognised for about 80 years. In almost all cases the source of infection is not apparent – these are called sporadic cases.
Some cases (a small number) are the result of a genetic abnormality and run in families.
An even smaller number have been linked to medical treatment, such as use of human pituitary hormones, corneal transplants or neurosurgery.
Since 1995 a new disease similar to classic CJD has been recognised, known as variant Creutzfeldt-Jakob disease (vCJD). To date there have been about 150 probable or proven cases, most of which have occurred in the United Kingdom. It is believed almost all of these cases are related to a massive epidemic of Bovine Spongiform Encephalopathy (BSE), commonly known as 'mad cow disease', in the United Kingdom which peaked in 1992 to 1993. People became infected by eating cattle products contaminated with the BSE prion.
There have been recent reports of transmission of vCJD by blood transfusion.
Signs and symptoms of Creutzfeldt-Jakob disease
Most cases of classic CJD occur in people over 50 years of age. Symptoms include:
rapidly progressive dementia
vCJD has several important differences from CJD:
It affects much younger people. The average age of death of people with classic Creutzfeldt-Jakob disease is 68 years, but for vCJD it is 28 years. We do not yet know the reason for this.
It generally begins with different symptoms from Creutzfeldt-Jakob disease, with psychiatric and sensory disturbances being prominent.
Diagnosis of Creutzfeldt-Jakob disease
The diagnosis is suggested by:
electroencephalogram (EEG) - recording of the electrical activity of the brain
brain scans - such as magnetic resonance imaging (MRI).
Currently, diagnosis of CJD can only be confirmed by laboratory examination of brain material (usually after death).
Diagnosis of vCJD can also be made by biopsy of the tonsils.
(time between becoming infected and developing symptoms)
For CJD, from 15 months to more than 30 years.
Unknown for vCJD because it is a new disease, but thought to be also from years to decades.
(time during which an infected person can infect others)
Routine social or community contact with a person with CJD or vCJD carries no risk of spread. Infected brain tissue is infectious before and after onset of symptoms.
Treatment for Creutzfeldt-Jakob disease
Currently there is no available vaccine or treatment and these conditions appear to be always fatal.
Prevention of Creutzfeldt-Jakob disease
Strict adherence by health care institutions to infection control guidelines for the management of surgical equipment.
Body tissues or blood from people with symptoms suggestive of CJD or vCJD must not be used for transplantation or transfusion
Human-derived pituitary hormone products are no longer used for treatment and people who have received them must not donate blood. These people should also notify hospitals that they have been treated with human pituitary hormones if they are going to have head or spine surgery.
Livestock herds infected with BSE are slaughtered and incinerated, and animals with symptoms suggestive of BSE are not permitted to enter the food chain.
Beef from countries where BSE is known to occur is subject to quarantine.
Rules on the composition of livestock feeds must be adhered to.
Use of the information and data contained within this site or these pages is at your sole risk.
If you rely on the information on this site you are responsible for ensuring by independent verification its accuracy, currency or completeness.
This site includes links to other websites operated by community, business and government.
These linked websites will have their own terms and conditions of use and you should familiarise yourself with these.
All linked websites are linked 'as is' and the Government of South Australia:
does not sponsor, endorse or necessarily approve of any material on websites linked from or to this Site;
does not make any warranties or representations regarding the quality, accuracy, merchantability or fitness for purpose of any material on websites linked from or to this Site;
does not make any warranties or representations that material on other websites to which this site is linked does not infringe the intellectual property rights of any person anywhere in the world; and
does not authorise the infringement of any intellectual property rights contained in material in other websites by linking this site to those other websites.
If you use automatic language translation services in connection with this site you do so at your own risk.
The information and data on this site is subject to change without notice. The Government of South Australia may revise this disclaimer at any time by updating this posting.
The Government of South Australia, its agents, instrumentalities, officers and employees:
make no representations, express or implied, as to the accuracy of the information and data contained on this site
make no representations, express or implied, as to the accuracy or usefulness of any translation of the information on this site or any linked website into another language
make no representations as to the availability of the site and the availability of websites linked from or to the site
accept no liability however arising for any loss resulting from the use of the site and any information and data or reliance placed on it (including translated information and data)
make no representations, either expressed or implied, as to the suitability of the said information and data for any particular purpose
accepts no liability for any interference with or damage to a user's computer, software or data occurring in connection with or relating to this Site or its use or any website linked to this site
do not represent or warrant that applications or payments initiated through this site will in fact be received or made to the intended recipient. Users are advised to confirm the application or payment by other means.