Sources: CJDSU's report in Lancet, matched "Age at onset", "Sex", "Year at onset" and "Year of death" (Table 2) with detailed information from British press. There are 28 cases now, at various stages of definite diagnosis, with this age distribution:15, 17, 18, 19, 20, 27, 27, 29, 29, 29, 29, 30, 30, 31, 34, 36, 36, 38, 41, 42, 59.
1. Victoria Rimmer, 18, kennel worker, Connays Quay. In coma since 1994.
2. Stephen Churchill, 19, Wiltshire. Died in May 1995.
3. Peter Hall, 20, student, Co Durham. Died in February 1996.
4. Anonymous, 29-year-old femnale, accountancy student, Canterbury,Kent.
The victim had been ill for two years and died on February 9 1996.
5. Anna Pearson, 29, solicitor from Canterbury, Kent. Died in Feb 1996
6. Michelle Bowen, 29, mother of three children, Manchester. Died in November 1995.
7. Alison Williams, 30, Wales. Died in February 1996.
8. Maurice Callaghan, 30, Belfast. Died in August 1995. (Ann Neurology, 1996;40, T132, p.523-524)
The first Irish case of V-CJD, second case outside of Britain. Died in December 1995, after being ill for
seven months and in a coma for several weeks.
9. Andrew Haig, 31, process engineer, Glenrothes, Fife. Died on May 25 1996.
10. Ann Richardson, 41, health care assistant and mother of one, Liverpool. Died in January 1996.
11. Barry Baker, 29, woodcutter from High Halden, near Ashford, Kent. Died June 2 1996.
12. Henri Rodriguez, 27, Rue Andre Chenier, France. Died on Jan 6 '96
13. A 16--year-old London girl of Turkish-Cypriot origins.
14. A 51-year-old man from the Birmingham area. Died several weeks ago [about Oct 1, 1996).
*** Up to three additional cases, all thought to be women under the age of 30, are also likely to be confirmed soon, according to British press.
*** Further suspected V-CJD cases:
* Jean Wake, 38, meat pie maker from Tyne. Died in November 1995.
* Ken Sharp, 42, businessman from Childwall, Liverpool. CJD symptoms started
in April 1995. Died in March 1996, after many months in coma.
* Janice Stuart, 34, a mother of two young children, Glasgow.
Died on September 13, 1996. Treated for depression at a psychiatric
hospital, for nearly 10 months.
* Graham Brown, 36, fireman from Ashford, Kent. Diagnosed as"possible V-CJD case", has been ill for more than a year.
* Elizabeth Bottle, 59, from Ashford, Kent. Suspected to be the first
V-CJD case in elderly. Died on September 25 1996, after long illness.
* Helen Rutherford, 15, from the Glasgow area. Confirmed as having CJD in
US test on spinal fluid.
* Unnamed 27-year-old, still alive.
* Unnamed 36-year-old, still alive.
* Unnamed 42-year-old.
* Middle-aged man in North Yorkshire, still alive.
Additional suspected cases reported either by CJDSU or victims' support groups (may duplicate some cases above):
[Source: The Irish Times, Friday, October 25 1996]
* Two women aged between 20 and 30.
* A woman in her late 30s.
* A man aged 35, with a condition that is said to be deteriorating rapidly.
Other potential but unconfirmed victims:
* A woman who recently gave birth.
* A women of 42, from the London area. She died recently.
"Sheila Gore, of the Medical Research Council's biostatistics unit, called yesterday for the CJD unit to issue more information about the number of suspected 'new-strain' cases referred to it, rather than issuing figures only for confirmed cases. 'Though only a certain proportion are confirmed, we need to know what proportion that is,' said Dr Gore. 'Once we know that, we can work back from the number of confirmed cases at any stage to how many suspect cases there are which will be confirmed.'
The Department of Health intends to publish its latest quarterly report on CJD cases later this month, covering the three months to the end of September. However, there are no plans at present to include suspected cases in those under 40.
Dr Gore said, 'It would be useful if they did, because if it turned out that eventually 90 per cent of those suspected cases are confirmed as the new strain, we would have something to work with. If it was only 10 per cent, at least we would be able to decide not to take much notice'.
In Orava, a sheep-rearing region of the Slovakian republic, which has seen a remarkably high incidence of CJD since 1976. Studies have shown that the victims share a common gene, which has led most scientists to conclude that these CJD clusters are genetic rather than infectious in origin. (The scrapie-like diseases are unique in that they can be either infectious or genetic.) On the other hand, Eva Mitrova (accent on the "a"), an epidemiologist at the Research Institute for Preventive Medicine in Bratislava, Czechoslovakia, is still unsatisfied with this conclusion. She's the main researcher on the ground looking at the Slovakian cases, and has published some papers that you can look up to see her thinking on the subject. As far as I know, no one has ruled out the possibility that the CJD cluster in Slovakia is actually the result of an intersection of (1) high exposure to infected sheep and (2) rare genetic SUSCEPTIBILITY. If this is the case, scrapie may be transmissible to humans under rare circumstances. In sum, though, it seems clear that sheep scrapie presents negligible direct risk to the human population.
Mitrova, E. et al, "'Clusters' of CJD in Slovakia: the first laboratory evidence of scrapie," European Journal of Epidemiology, Sept. 1991, 7 (5), pp. 520-3. Mitrova, E. et al, "Focal accumulation of CJD in Slovakia: Retrospective investigation of a new rural family cluster," European Journal of Epidemiology, Sept. 1991, 7 (5), pp. 487-9. Mitrova, E. et al, "Some new aspects of CJD epidemiology in Slovakia," European Journal of Epidemiology, Sept. 1991, 7 (5), pp. 439-49.
1. With regard to declaring oneself on probabilities.. at a summer meeting at the House of Commons, Sheila Gore (a statistician) asked the people there (mixture of MPs and scientists) to write, anonymously, their feeling on whether v-CJD was caused by CJD. 0 = definitely not, 10 = certain, and values in between to indicate degrees of uncertainty.
For the record, I put 9. But I asked her later: she said that there tends to be a mixture, with peaks at either end (8/9s and 1/2s), with few 5s. Ralph B would seem to be in a minority (hi Ralph). I expect it's because uncertainty is a difficult thing to build a system of thought around.
I would suspect that many of the MPs there - many of whom had farming interests in their consituencies - put low numbers. But they also demonstrated a rather desperate wish not to listen to the science, I thought.
That is, I don't think that the extent can be reliable deduced from the numbers coming in during the earlier years because of the vast range and form of doses and ambient genetic and somatic backgrounds.
I would definitely go with cumulative dose, as expressed by the integral over incubation time of an infectious titre growth function, modulated by saturation considerations.
I would go with (UK alone) tens of millions seriously exposed and already at various stages of the disease, with deaths over the next twenty years in the low millions, unless more is put into research -- the sooner the better. Treament may well mitigate the toll, with early therapies delaying onset of full-blown illness past the normal life span. Rather like with prostrate cancer, quite a few could then die with it but not from it. Billions are spent on the cull, whereas only tuppence seems available for research, which could be characterized as locking the barn door after the horse is gone, and more denial, respectively.
At this point, we are all just reading the tea leaves.