What is 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.

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.

Causes of 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.
• 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 the human form of a disease called bovine spongiform encephalopathy (BSE) that occurs in cattle and is often referred to as ‘mad cow disease’. People who ate meat infected with BSE contracted 'variant CJD' in the United Kingdom during the 1980s and 1990s. Neither form of the disease has been found in Australia. Although variant CJD occurs in humans and BSE occurs in cattle, there is often confusion between variant CJD and mad cow disease.

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

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
• problems with thinking
• problems with speaking and swallowing
• double vision or blindness
• depression
• muscle spasms
• seizures

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.

Diagnosis of Creutzfeldt-Jakob disease

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.

Treatment of Creutzfeldt-Jakob disease

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

Prevention of Creutzfeldt-Jakob disease

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 aren't allowed to donate blood, because of the slight risk that they might have picked up the infection.