HL Deb 11 April 2000 vol 612 cc32-3WA
Lord Lucas

asked Her Majesty's Government:

Further to the Written Answer by Lord Hunt of Kings Heath on 27 March (WA 55–6), whether they will provide data on those who have died from, or been diagnosed with, new-variant CJD in the United Kingdom as to:

  1. (a) the status of nucleotide-21 preceding the prion ATG start codon;
  2. (b) the status of codons 26, 56 and 174 of doppel;
  3. (c) the dates of onset and confirmation for those patients diagnosed with new-variant CJD but still living;
  4. (d) the definition of "onset"; and
  5. (e) the age of the patients at onset to the nearest month. [HL1807]

Lord Hunt of Kings Heath

The genetic information requested is not available. However, extensive studies of polymorphisms in and around the prion protein and doppel genes have been under way for some time at the St Mary's Prion Unit, London. The results of these investigations will be published in the scientific literature, subject to peer review, in due course.

Confirmation of a diagnosis of vCJD is currently obtained by postmortem neuropathology. There are therefore no "confirmed" patients still living. The dates of onset for patients still living and defined as "probable" to the nearest month are as follows:

  • November 1996
  • February 1998
    • WA 33
  • June 1998
  • July 1998
  • July 1998
  • October 1998
  • February 1999
  • March 1999
  • April 1999
  • May 1999
  • May 1999
  • June 1999

"Onset" is defined by the National CJD Surveillance Unit as the timing and nature of the first symptoms reported by the patient's relatives and obtained from the patient's medical, psychiatric and general practitioner case notes. On average, some eight months elapse between date of onset and a patient being diagnosed "probable".

Age at onset for the 12 probable vCJD patients who are still alive (years/months) is as follows:

  • 12 years 8 months
  • 15 years 10 months
  • 18 years 2 months
  • 19 years
  • 21 years 11 months
  • 22 years 9 months
  • 24 years 1 month
  • 25 years 1 month
  • 29 years 10 months
  • 30 years 4 months
  • 33 years 4 months
  • 36 years 7 months

Lord Lucas

asked Her Majesty's Government:

How many cases of new-variant CJD in humans have been strain-typed; and what were the results of these experiments. [HL1867]

Lord Hunt of Kings Heath

The following two published papers address these questions:

  1. 1. Transmissions to mice indicate that 'new variant' CJD is caused by the BSE agent (Bruce et al); and
  2. 2. The same prion strain causes vCJD and BSE (Hill et al)

(Nature, Vol 389, 2 October 1997).

Further research is under way.

Lord Lucas

asked Her Majesty's Government:

Whether there are any indications that there might be more than one strain of new-variant CJD in humans. [HL1868]

Lord Hunt of Kings Heath

New cases of all forms of Creutzfeldt Jakob Disease are subject to close scrutiny by the National CJD Surveillance Unit in Edinburgh. There is currently no evidence of the existence of more than one strain of variant CJD in humans.