Overview

Creutzfeldt-Jakob disease (CJD) is a rare and fatal condition that affects the brain. It causes brain damage that worsens rapidly over time.

Symptoms of CJD

Symptoms of CJD include:

• loss of intellect and memory
• change in personality
• loss of balance and co-ordination
• slurred speech
• vision problems and blindness
• abnormal jerking movements
• progressive loss of brain function and mobility

Most people with CJD will die within a year of the symptoms starting, usually from infection. This is because the immobility caused by CJD can make people with the condition vulnerable to infection.

Read more about the symptoms of Creutzfeldt-Jakob disease and diagnosing Creutzfeldt-Jakob disease.

What causes CJD?

CJD appears to be caused by an abnormal infectious protein called a prion. These prions accumulate at high levels in the brain and cause irreversible damage to nerve cells, resulting in the symptoms described above.

While the abnormal prions are technically infectious, they're very different to viruses and bacteria. For example, prions aren't destroyed by the extremes of heat and radiation used to kill bacteria and viruses, and antibiotics or antiviral medicines have no effect on them.

Read more about the causes of Creutzfeldt-Jakob disease.

Types of CJD

There are four main types of CJD, which are described below.

Sporadic CJD

Sporadic CJD is the most common type.

The precise cause of sporadic CJD is unclear, but it's been suggested that a normal brain protein changes abnormally ('misfolds') and turns into a prion.

Most cases of sporadic CJD occur in adults aged between 45 and 75. On average, symptoms develop at age 60-65 years.

Despite being the most common type of CJD, sporadic CJD is still very rare, affecting only one or two people in every million each year in the UK.

In the UK during 2014, there were 90 recorded deaths from sporadic CJD.

Variant CJD

Variant CJD is likely to be caused by consuming meat from a cow that had bovine spongiform encephalopathy (BSE or 'mad cow disease'), a similar prion disease to CJD.

Since the link between variant CJD and BSE was discovered in 1996, strict controls have proved very effective in preventing meat from infected cattle from entering the food chain (see preventing Creutzfeldt-Jakob disease for more information).

However, the average time it takes for the symptoms of variant CJD to occur after initial infection (the incubation period) is still unclear. The incubation period could be very long (over 10 years) in some people, so those exposed to infected meat before the food controls were introduced can still develop variant CJD.

The prion that causes variant CJD can also be transmitted by blood transfusion, although this has only happened four times in the UK.

In the UK during 2014, there were no recorded deaths from variant CJD.

Familial or inherited CJD

Familial CJD is a very rare genetic condition where one of the genes a person inherits from their parent (the prion protein gene) carries a mutation that causes prions to form in their brain during adulthood, triggering the symptoms of CJD. It affects about 1 in every 9 million people in the UK.

The symptoms of familial CJD usually first develop in the early 50s.

In the UK during 2014, there were 10 deaths from familial CJD and similar inherited prion diseases.

Iatrogenic CJD

Iatrogenic CJD is where the infection is accidentally spread from someone with CJD through medical or surgical treatment.

For example, a common cause of iatrogenic CJD in the past was growth hormone treatment using human pituitary growth hormones extracted from deceased individuals, some of whom were infected with CJD. Synthetic versions of human growth hormone have been used since 1985, so this is no longer a risk.

Iatrogenic CJD can also occur if instruments used during brain surgery on a person with CJD aren't properly cleaned between each surgical procedure and are re-used on another person. However, increased awareness of these risks means iatrogenic CJD is now very rare.

In the UK during 2014, there were three deaths from iatrogenic CJD (caused by receiving human growth hormone before 1985).

How CJD is treated

There's currently no cure for CJD, so treatment aims to relieve symptoms and make the affected person feel as comfortable as possible.

This can include using medication such as antidepressants to help with anxiety and depression, and painkillers to relieve pain. Some people will need nursing care and assistance with feeding.

Read more about treating Creutzfeldt-Jakob disease.

Variant CJD compensation scheme

In October 2001, the government announced a compensation scheme for UK victims of variant CJD. A trust fund was set up in April 2001 and payments of £25,000 were made available to most affected families.

National Congenital Anomaly and Rare Diseases Registration Service

If you have CJD, your clinical team will pass information about you on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS).

This helps scientists look for better ways to prevent and treat CJD. You can opt out of the register at any time.