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From: TSS (
Date: August 11, 2004 at 2:44 pm PST

In Reply to: CJD FARMERS WIFE 1989 AND CJD FARMERS posted by TSS on August 10, 2004 at 1:11 pm:

Update on CJD Situation (Agenda item 8)

19. Dr Will reaffirmed that the incidence of CJD in dairy farmers in
Europe showed an excess over the incidence for the population as a
whole except in the Netherlands. There was no excess if data for all
farmers were used.

20. He confirmed that there is now a CJD suspect in a 52 year old
from York who had a history of having been an abattoir worker. The
patient was exhibiting progressive cognitive symptoms simultaneously
with ataxia- Dr Will's current view was that the patient was "no more
than a suspect" at this stage and the EEG was non-specific. The patient
worked in a mixed abattoir for 18 months in 1989. largely in me lairage,
occasionally stunning cows, where he would also wash out the stunning
pen and would not normally have worn gloves. He occasionally pithed
animals but had much less exposure than other abattoir workers and was
essentially a stockman. The son-in-law was not aware of any injury
received in the abattoir that required medical attention. Most of this
information was provided by the son-in-law who is also an abattoir
worker, and would therefore have a very good grasp on abattoir
practices and his father-in-law's job.

21. Professor Smith said that, with the figures provided by MAFF of
11,500 workers in the red meat slaughterhouse industry 30 per cent
annual turnover, and a potential exposed population of 60,000 over ten
years, one would expect 0.2 cases over a five year period. He felt that it
was not possible to. come to any conclusions on the basis of this case
alone even if CJD was confirmed. Nevertheless, taking into
consideration the affected farmers as well, and even though the abattoir
worker was in an apparently relatively low risk category, the "box" of
"at risk" occupations was getting full compared to expectation on pure
chance, and could not be dismissed. The Chairman agreed but reminded
people that the case was only a suspect at this stage.

22. Dr Will reviewed the age distribution of cases. He continued to
have no concern about the incidence of disease in those aged over 30 but
the number of cases under 30 was worrying. Between 1970 and 1989
Acre had been no cases under 30 except for those due to growth
hormone treatment. Since 1990 there have been four definites and one
possible. These comprised Ac 17 and 19 year olds which were already
published, a 29 year old diagnosed on Ae basis of cerebral biopsy, a 29
year old diagnosed post mortem and a 29 year old who was still alive
and classified as no more than "possible". There were also a 30 and 38
year old confirmed and a 35 year old suspect, who now looks unlike
CJD. In one of the 29 year olds and me 30 year old the pathology is
unique with very extensive plaques in both the cerebellum and cerebral
cortex and Dr Will considered there is a very high chance that these two
are genetic.

23. Dr Kimberlin asked whether the lack of previous cases had been
due to misdiagnosis but Dr Will thought this unlikely because the
disease was unusual in the young. He had checked through 35 cases of
suspect but negative SSPE and had found none which looked like CJD
and no evidence to suggest Aat SSPE was being misdiagnosed. Other
members were if the view that CJD in patients under 30 would not have
been misdiagnosed because of the rarity of symptoms in this age
category. When asked if he knew of a second case in an abattoir works
as mentioned in the press Dr Will stated that the 30 year old case had
visited an abattoir for two days whilst a 38 year old case had worked in
a butcher's shop for a year in 1975 and a 29 year old case had a husband
who worked in an abattoir. He was unaware of any other cases
suspected in abattoir workers';

Dr Will reminded the
Committee that if two of the cases in young adults were genetic then the
figures were perhaps not so worrying at this stage, given the fact that
there had been two in France although over a 15 year period, and two in
Japan and other cases reported in the Netherlands and Australia under


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