Creutzfeldt-Jakob (Prion) disease
Creutzfeldt-Jakob disease (CJD) is a rare and fatal condition that affects the brain. It causes brain damage that worsens rapidly over time.
This page covers:
Types of CJD
Variant CJD compensation scheme
Symptoms of CJD include:
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 makepeople with the conditionvulnerable to infection.
These prions accumulate at high levels in the brain and cause irreversible damage to nerve cells, resulting in thesymptoms described above.
While the abnormal prions are technically infectious, they're very different to viruses and bacteria. For example, prionsaren't destroyed by the extremes of heat and radiation used tokill bacteria and viruses, and antibiotics or antiviral medicines have no effect on them.
Variant CJD is likely to be caused by consuming meat from a cow that hadbovine 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 CJD is a very rare genetic condition where one of the genes a person inherits fromtheir parent (the prion protein gene) carries a mutation that causes prions to form in their brainduring adulthood, triggering the symptoms of CJD. It affects about 1 in every 9 million people in the UK.
The symptoms offamilial CJD usually first develop inthe early 50s.
Inthe UK during 2014, therewere 10 deathsfrom familial CJD and similar inherited prion diseases.
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 byreceiving human growth hormone before 1985).
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 andassistance with feeding.
A trust fund was set up in April 2001 and payments of 25,000 were made available to most affected families.
Find out about the different types of Creutzfeldt-Jakob disease (CJD), and what causes the condition and how it's treated.
Read about the symptoms of Creutzfeldt-Jakob disease (CJD) which vary depending on the type. In sporadic CJD they are neurological and in variant CJD they are psychological.
Find out what causes Creutzfeldt-Jakob disease (CJD). The main cause of all types of CJD is a build-up of an abnormal infectious protein in the brain called a prion.
Read about the tests used to investigate Creutzfeldt-Jakob disease (CJD) including MRI, EEG and lumbar puncture. A brain biopsy is the only way to confirm a diagnosis of CJD.
Find out what can be done for a person with Creutzfeldt-Jakob disease (CJD) to reduce their symptoms.
Read about the strict controls that are in place to prevent variant Creutzfeldt-Jakob disease (CJD) spreading through the food supply and blood transfusions.