WRITTEN QUESTION E-1984/96 by Hiltrud Breyer (V) to the Commission (17 July 1996)

Subject: Spongiform encephalopathies in humans

1. What basic research is being carried out in the EU above and beyond epidemiological studies of Creutzfeldt-Jakob disease?

2. Are there any BSE or similar infections in non-EU countries such as the USA, Argentina or Japan? If so, what measures are being taken?

3. In mice, the gene for the production of the normal prion protein has allegedly been blocked/removed without side effects. What is the function of the normal prion protein? What help can victims expect from this?

4. What is the cost of treating a CJD case?

5. According to a press agency report (dpa) of 15 April 1996, American scientists have registered a patent for a BSE early recognition test. The background is that ten years ago, in the spinal cord of CJD victims, two proteins were found which the body produces in reaction to this disease. What value do these tests have, e.g. in comparison to the radical solution of destroying animal stocks?

6. Which specific CJD cases caused the British to go public? Why did they do so in March 1996 in particular?

7. Does the Commission know anything about the latest CJD cases in the UK and Germany? Have there been any further cases of CJD in the UK since the eleven cases announced in March?

Answer given by Mr Flynn on behalf of the Commission (4 October 1996)

1. At European level, research into spongiform encephalopathies is proceeding along the following lines:

* extension of the epidemiological surveillance of Creutzfeldt Jakob disease (CJD) to all Member States;

* research into the establishment of neuropathological criteria for diagnosing human spongiform encephalopathies, including the early detection of these diseases;

* characterisation of the agent responsible for transmissible encephalopathies and study of the mechanisms involved in the pathological process, including its spread and the breaching of the barrier between species;

* research into methods for the prevention and treatment of spongiform encephalopathies.

2. Bovine spongiform encephalopathy (BSE) is one of the transmissible spongiform encephalopathies (TSEs). The following TSEs are known to occur naturally:

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Many countries have criteria for trade in live animals, semen and embryos as regards freedom from scrapie and BSE. Some, notably Iceland and the USA, have attempted to eradicate scrapie with limited success.

Also, other countries outside the Community have already notified cases of BSE (Switzerland, Canada, Falkland Islands, Oman).

3. The normal function of the PrP gene which is found in all animal species has not been established. Recent studies on mice published in Nature have shown that mice without this gene have disturbed circadian rhythms. Genetic studies are, by their nature, aimed at a fundamental understanding of the disease, and their results cannot be predicted. However, it could be expected that they could lead to methods of treatment, prevention or diagnosis.

4. The Commission does not have any information for assessing the cost of caring for a patient with Creutzfeldt-Jakob disease.

Moreover, as things stand at present the treatment of cases is exclusively symptomatic, and the treatment costs vary from one Member State to another.

5. The test mentioned is only capable of confirming disease once clinical signs develop. Furthermore, the extraction of cerebro-spinal fluid is not a simple task in farm animals. For an eradication programme a test which shows the presence of infection before signs is needed, using material which is simple to obtain in field conditions eg. blood, urine. There are some tests under development which show promise but none are practical at present. The specificity and sensitivity of these tests must be assessed in animals in order to draw any conclusions regarding the possibility of using them in BSE eradication programs.

6. The British authorities announced ten cases of a variant of Creutzfeldt-Jakob disease on 20 March 1996. Shortly afterwards, an article in a scientific journal ((The Lancet: 1996; 347:921-25. )) gave details of the cases identified in the United Kingdom. The identification and analysis of these cases occurred over several months, via the CJD surveillance system set up in five Member States with the support of the Commission.

7. Following on from the ten cases published in the above-mentioned article, another case has been confirmed in the United Kingdom, and one in France. The Commission does not have any information on other cases of CJD variants declared in other Member States.