Alternative Names Jakob-Creutzfeldt disease, spastic pseudoparalysis, subacute spongiform encephalopathy
Definition Creutzfeldt-Jakob disease (CJD) is a rare and fatal infectious disease of the brain.
What is going on in the body? Creutzfeldt-Jakob Disease (CJD) affects about one person in a million. Experts do not know how most people get this disease, although sometimes CJD is inherited and called familial CJD. CJD usually occurs in people who are at least 60 years old. The disease is characterised by rapid progressive dementia. Usually, the person dies within 6 months after symptoms appear. The incubation period, which is the time between exposure to the organism and development of symptoms, is thought to range between 3 and 20 years.
What are the signs and symptoms of the disease? Symptoms include:
dementia, as shown by confusion, disorientation, and memory loss
sight problems
abnormal behaviour
movement abnormalities
muscle spasms
What are the causes and risks of the disease? Experts are not sure what causes CJD. The disease can be transmitted in several ways.
Hormones used to treat certain endocrine problems, such as hormone disorders, are originally taken from an infected person and passed to the recipient.
Donors of certain human transplant tissues, such as corneas, are infected and so the recipient gets infected.
The disease is inherited from another family member.
An individual, such as a surgeon or healthcare worker, is exposed to infected tissue, for example, while operating on an infected patient's brain. This infection is then passed to other patients through contact with the surgeon or healthcare worker.
How is the disease diagnosed? The best way to diagnose CJD is to biopsy the brain by taking tissue and examining it under a microscope. Sometimes brain imaging is helpful using computerised tomography scan (CAT), a special three-dimensional x-ray, or magnetic resonance imaging, a special three-dimensional image using magnets, and an electroencephalogram (EEG),a recording of brain waves.
What are the long-term effects of the disease? CJD causes progressive mental deterioration that results in death in about 6 months.
What are the risks to others? CJD cannot be spread from person to person unless there is contact with infected brain tissue.
What are the treatments for the disease? There are no treatments for CJD.
Author: Danielle Zerr, MD Reviewer: HealthAnswers Australia Medical Review Panel Editor: Dr David Taylor, Chief Medical Officer HealthAnswers Australia Last Updated: 1/10/2001 Contributors Potential conflict of interest information for reviewers available on request
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