§ Mr. MartlewTo ask the Secretary of State for Health (1) what plans he has to make CJD a notifiable disease; and if he will make a statement; [12670]
(2) what instructions have been issued by his Department to (a) neurologists and (b) other medical specialists treating people with CJD or suspected CJD to ensure such cases are brought to the attention of the CJD surveillance unit; [12669]
(3) what plans his Department have to make compulsory post-mortems on people who are suspected of dying from CJD; and if he will make a statement; [12668]
(4) what steps his Department has taken to ensure that relatives of people suspected of having died from CJD are notified of the need for a post-mortem to confirm CJD as the cause of death; [12695]
(5) how many of the CJD cases confirmed by his Department in 1996 fell into the age ranges (a) 0 to 20, (b) 21 to 30, (c) 31 to 40, (d) 41 to 50, (e) 51 to 60, (f) 61 to 70 and (g) over 70 years at the time of death; [12696]
111W(6) how many cases have been brought to the attention of the CJD surveillance unit where people have died under the age of 60 years from suspected CJD but have not had a post-mortem; [12694]
(7) pursuant to his Department's press release of 6 January, by what means his Department's scientists have been able to confirm that two living people have CJD. [12697]
§ Mr. HoramThe national Creutzfeldt-Jakob disease surveillance unit was established in 1990 to monitor the incidence of CJD and investigate the epidemiology of the disease, paying particular attention to occupation and eating habits so that any change in the pattern of CJD following the advent of BSE could be detected.
Regional neurologists were notified when the unit was set up. The unit has established good informal contacts with neurologists throughout the United Kingdom, which allow thorough investigation of a wide range of individual cases during the course of the illness. The clinical indications of classic CJD are well documented and understood by neurologists. The director of the national CJD surveillance unit wrote to all members of the Association of British Neurologists on 21 March 1996 describing the clinical and neuropathological features of the new variant CJD, and requesting that any case with this clinical or neurological profile be referred to the unit. A copy of the letter has been placed in the Library.
Cases of suspect CJD are mainly referred to the CJD surveillance unit directly from professional groups including neurologists, neuropathologists and neurophysiologists, and occasionally other sources. Given the present level of awareness of this disease we think it unlikely that any cases are missed. As a safety net, details are sought from all death certificates coded under the specific rubrics for CJD. There is no evidence to suggest that ascertainment would be improved by making CJD notifiable; indeed, because of the difficult nature of clinical diagnosis of CJD and the consequent difficulty of defining what should be notified, an attempt to make the disease notifiable might lead to fewer suspected cases being referred to the unit. The Government therefore have no plans to make CJD a notifiable disease.
Definite confirmation of CJD is possible only by microscopic examination of brain tissue. Normally this is conducted post-mortem. The team from the unit discusses the importance of the post-mortem with the clinician concerned, and also with the family if they raise the issue during epidemiological investigations. However, the decision of whether a post-mortem is carried out is a matter between the clinician and the relatives. Post-mortem examination has been carried out in 85 per cent. of cases where the patient is aged under 60. Of the 124 patients involved since 1990, only 18 have not had a post-mortem. Of these, seven, including one new variant case, were classified as "probable" according to clinical criteria and so are included in the published statistics.
The Spongiform Encephalopathy Advisory Committee has considered the issue of the role of the coroner in relation to CJD deaths, and recommended that:
it was appropriate to report deaths from iatrogenic CJD to the coroner;there was no need to report deaths from classical sporadic or familial cases;112Wdeaths from new variant CJD should not normally be reported. However, the reporting officer (either the medical consultant or the Registrar of Deaths) would need to consider whether the information available suggest the death could be "unnatural"; the coroner may decide to hold an inquest where there appeared to be doubt or dispute as to whether CJD, in any form, was the actual cause of death.Post-mortems are not compulsory for any other clinical condition, and the Government have no plans to make them so for people who are suspected of having died from CJD. This is a matter in which the wishes of relatives must be paramount.
Diagnosis of CJD can occasionally be confirmed by positive brain biopsy while the patient is still alive. Biopsy is an invasive procedure which is potentially distressing for relatives, carries some risk of complication for the patient, may not always provide evidence of the diagnosis—a negative biopsy does not rule out the disease—and necessitates special precautions to be taken by the staff involved. Whether a biopsy is carried out is a clinical decision and we have no plans to change this.
A breakdown of deaths from CJD in 1996 by age at time of death is set out in the table.
Breakdown of definite and probable CJD deaths in 1996 by age at time of death Age at time of death Number of cases 0–20 2 21–30 4 31–40 3 41–50 7 51–60 4 61–70 10 Over 70 10