Prion diseases are not infectious in the usual way. For example, they are not spread by airborne droplets like colds and flu, or sexual contact like HIV.

The evidence suggests there is no increased risk of developing CJD from contact with someone with the condition. No special precautions are required. However, it is sensible for anyone who might be exposed to the blood of a CJD patient (usually medical staff) to wear gloves.

How can we be sure that the diagnosis of CJD is a correct one?

There are four types of CJD - sporadic, familial, iatrogenic and variant. The accuracy of a diagnosis varies depending on the form of the disease. A correct diagnosis may only be possible after death when a post mortem has been performed.

In familial CJD, diagnosis depends on an analysis of symptoms and by genetic testing. Iatrogenic CJD is diagnosed on the basis of symptoms developing in someone with a relevant exposure, for example, in those given human growth hormone. The diagnosis of variant CJD is very difficult, but brain scanning, using magnetic resonance imaging (MRI), and tonsil biopsy may prove to be useful.

Is the blood supply safe from CJD?

The Health Minister, John Reid, announced in December 2003 that a patient had died of CJD and it was thought, although not proven, that this was the first case of CJD where transmission had been as a result of a blood transfusion.

In August 2004 it was announced that a second patient had died after a blood transfusion from a donor who had subsequently died of vCJD. The second patient had died of an unrelated cause, but at post mortem showed that prion was present in the spleen and cervical lymph nodes. As all the patients who have died of vCJD had been from the methionine homozygous PRNP genotype (mm) this case showed that vCJD is not confined to this group as the patient was a heterozygote at codon 129 of PRNP (mv).

In September 2004 John Reid announced that selected groups of patients were being notifield that, as they had received certain batches of plasma products in the past, they could be at a small increased risk of carrying the vCJD agent.

Since these announcements people who have received a blood transfusion since 1980 would not now be allowed to donate blood as a further precautionary measure against the possible risk of vCJD being transmitted by blood and blood products.

Is there a risk of contracting CJD from organ transplant surgery?

The risk of contracting CJD from organ transplants is uncertain. A woman later shown to have been suffering from CJD did recently provide material for three eye operations. Unfortunately, a transplant usually has to be done before a full post mortem has taken place, so this risk cannot be completely eliminated. In the vast majority of cases, the benefit of having the transplant far outweighs the risk of contracting CJD from a donor.

Is the person with CJD in pain?

Neurological examination and electroencephalogram (EEG) testing of people in the later stages of CJD indicate that they lose awareness of their condition as the disease progresses. Obviously this saves them - but not their families - much mental suffering.

In the early stages, however, patients with CJD can develop marked fear, which can be very distressing and is probably associated with visual hallucinations.

Is a post mortem necessary in CJD?

Post mortem examination is not compulsory when CJD is suspected - the doctor will need the permission of the next of kin.

It often helps the family if they can have a definite cause of death, which at present is only possible after post mortem. The findings may also help research into the disease.

Is sporadic CJD increasing in the UK?

In the UK, all cases of suspected CJD are reported to the National CJD Surveillance Unit in Edinburgh. The number of cases of sporadic CJD in the UK has increased since 1970, when figures first started being kept. In 1970-71 there were 21 deaths from sporadic CJD and in 2002 there were 67 deaths from sporadic CJD. Most of the increase has occurred since 1990 and in the over 70 age group.

It is not clear whether this is due to greater awareness of CJD among the medical profession, or whether it represents a genuine increase in the incidence of the disease.

Will there be many more cases of variant CJD?

No one knows. There are currently 155 cases of variant CJD (May 2005).  There is evidence that the number of cases are on the decline.

If the disease comes from exposure to infected beef products prior to the ban on specified offal in human food in 1989, as is now widely accepted, then there could be more cases if the incubation period is very long. However, without knowing the exact route of the infection, or who is most at risk and why, it is currently impossible to predict how many more cases of variant CJD there will be.

Can CJD be confused with Alzheimer's disease?

About 10 per cent of patients with CJD have symptoms which are similar to Alzheimer's disease. However, an accurate diagnosis is usually made following electroencephalogram (EEG) testing and an analysis of cerebrospinal fluid.

What is being done to protect us from CJD?

At present there is no way of protecting people from the sporadic or familial forms of CJD. Iatrogenic CJD is guarded against by destroying surgical instruments that have been used on people with CJD, and by not using their organs for transplant.

There is however, a concern that there could be an increase of cases through secondary transmissions. ie through blood transfusions

A number of measures have been used by the government to minimise the risk of contracting variant CJD from BSE infected meat and meat products. These include a temporary ban on specified offal in human food.

As there is no test to screen blood for CJD, a method of filtering the blood, called leucodepletion, was announced in July 1998 and implemented Autumn 1999.

For further blood information, there is a question and answer article, written by the National Blood Service, in issue 13 of the CJD Support Network newsletter.  See the newsletter downloads.

Can a mother pass CJD to a child?

Scientists are aware that it is theoretically possible to pass variant CJD from mother to child. This has happened in some of the animal forms of prion disease. In sheep scrapie, for example, a prion disease similar to BSE, the disease has been known to pass from ewes to their lambs. However, to date there is no firm evidence that this has occurred in humans.

Is there any treatment for CJD?

There is no cure, at present, for CJD, although scientists are researching into the causes of and potential treatments for the disease. However, there are a number of drugs already available which can relieve symptoms and make the patient more comfortable - for instance, valproate and clonazepam for jerking movements.

There is now an official clinical trial using the drug Quinacrine for CJD.  For further information on the trial, an article on the Prion 1 clinical trial of an anti-prion drug can be seen in issue 14 of the CJD Support Network newsletter.  Or you can contact the National Prion Clinic at Prion.help@hclh.org or view their website at www.nationalprionclinic.org

Some patients have also chosen to use Pentosan polysulphate. 

Equally important are general support and care for both the person and their family. Social services should be involved at an early stage to advise on benefits, day centres, respite and long term care. Speech and occupational therapists can help with specific problems, while the district nurse may provide more general nursing care and advice.

What happens on admission to hospital varies. Much depends on the type of hospital, the patient and, of course, the views of the family. Following admission there will be an investigation and tests to establish the diagnosis and general supportive nursing care.