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From: TSS ()
Subject: Re: SEAC ANNUAL REPORT 2006
Date: March 19, 2007 at 9:58 am PST

SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE
Draft minutes of the 96th meeting held on 20th February 2007

snip...

ITEM 5 – CONSIDERATION OF FUTURE DISCUSSION OF
UNUSUAL CASES OF SPONGIFORM ENCEPHALOPATHY IN
CATTLE (SEAC 96/2)
14. The Chair explained that the purpose of this item was to consider
the scope of a future discussion of unusual cases of BSE in cattle.
Such cases had been termed ‘atypical BSE’ by some researchers,
however, as had been agreed at SEAC 93, this was a misleading
term and should be avoided. SEAC was asked to identify relevant
data not highlighted in SEAC paper 96/2, key researchers that
could be invited to present data and contribute to discussions and
questions for the future discussion.
15. A member noted that most unusual BSE cases had been
characterised on the basis of PrPres banding patterns on western
6
© SEAC 2007
blot. Cases described as H-type gave a normal distribution of
PrPres glycoforms with a non-glycosylated band of higher molecular
weight than normally observed. Cases described as L-type gave
an unusual distribution of PrPres glycoforms and a non-glycosylated
band of lower molecular weight than normally observed. H-type
cases had been identified in France and the Netherlands, L-type
cases in Italy and both types had been found in Germany.
Transmission experiments using bovinised mice had been
conducted and, whilst the L-type was transmitted with a shorter
incubation period compared with BSE, the transmissibility or
incubation period of the H-type was unclear from the information
available. L-type had also been transmitted to macaques with a
shorter incubation period compared with BSE. Transmission
studies with material from H- and L-type cases into cattle by
intracerebral (ic) inoculation are underway but no data are
available at present. Two unusual BSE cases had also been
identified in Japan, one of L-type and the other with a low content
of diglycosylated PrPres and a non-glycosylated band of lower than
usual molecular weight. Data on unusual BSE cases reported in
the United States of America (USA) is very limited. In summary,
the information suggested that there appeared to be at least two
strains giving rise to the unusual cases of BSE. The data raised a
number of questions that could be considered at a future
discussion:
• Are all the cases in Europe, USA and Japan of the same two
strains or are there further strains?
• What is the origin of these unusual forms of BSE and how are
they related to BSE?
• Are these unusual cases examples of sporadic or
spontaneous BSE in cattle and if so, how can the same
strains appear in a number of countries?
• Was the BSE epidemic in the UK initiated by intra-species
transmission from such a sporadic BSE case?
• What is the tissue distribution of PrPSc and infectivity in these
animals?
• Are all the cases of unusual BSE transmissible to mice?
• Can these unusual BSE cases be transmitted to humanised
mice?
• Are these unusual BSEs related to cases of sporadic CJD in
humans as there are similarities in glycoform profile?
16. The committee agreed that it would be important to discuss the
available data on unusual BSE cases at the next SEAC meeting.
7
© SEAC 2007
17. Members noted that the transmission of Bovine Amyloidotic
Spongiform Encephalopathy to macaques indicated that the clinical
signs differed from signs arising from transmission of BSE. This
was important as it indicated it may be possible to identify and
distinguish infections that occur naturally. It is also important to
understand the tissue distribution of the agent giving rise to
unusual cases of BSE. Dr Matthews noted that transmission
studies had begun in cattle, however interpretation of these data
would be complicated as the ic route of inoculation had been used.
It is not known if studies using the oral route are underway.
Members asked about information available from necropsy of
unusual BSE cases. Dr Matthews explained that detailed post
mortem examinations of the brains of the Italian unusual BSE
cases had been conducted, however most of the other cases were
characterised only by western blot analysis. The two USA cases
had also been characterised by immunohistochemistry (IHC).
18. Members asked if it was known whether a relationship between the
unusual BSE cases and their genotype had been found. Dr
Matthews responded that there was very little information, although
known polymorphisms had been identified in one USA case and
one French case.
19. Members noted that no unusual BSE cases had been found in the
UK and asked whether UK surveillance was sufficient to detect
unusual BSE cases. Dr Matthews explained that UK and French
surveillance was comparable and, although methods used to
detect BSE differed between laboratories, methods used in the UK
should be capable of detecting unusual BSE cases. Projects are
underway to look for evidence of unusual cases of BSE in archived
samples of historic BSE cases. Mr Burke noted that since 2003,
around 570 BSE-positive animals had been screened by VLA
using a discriminatory test with no unusual BSE cases identified. A
member noted the older age of many of the unusual BSE cases
and asked if old cattle would be tested in the UK. Mr Burke
explained that all fallen stock over 24 months of age are tested in
the UK. Mr Burke suggested that the committee might include
consideration of the effect of current BSE controls on unusual BSE
cases entering the food chain in the future discussion of unusual
cases of BSE. Dr Matthews noted that data on cattle surveillance
could be presented by age to highlight the proportion of older
animals tested as part of the future discussion.
20. Members asked whether other animals in the same cohort as
unusual BSE cases had been tested. Dr Matthews responded that
8
© SEAC 2007
such studies may not have been conducted and, even if they had
been, the likelihood of finding cohort cases may be very low.
21. The committee agreed that it would be important to hold as much
of the future discussion of unusual BSE cases in open session as
possible, although noting that some researchers may wish to
present data from incomplete studies in a reserved business
session. It was agreed that the following researchers should be
invited to present data and take part in discussions, following
provision of a list of key questions identified by SEAC:
• Dr Mark Hall (National Veterinary Surveillance Laboratory,
USA) and Dr Juergen Richt (National Animal Disease Centre,
USA) to present data on USA cases.
• Dr Thierry Baron (Unite Agents Transmissibles Non
Conventionnels, France) to present data on French cases.
• Dr Cristina Casalone (Centro di Referenza Nazionale per le
Encefalopatie Animali, Italy) and Dr Fabrizio Tagliavini
(Instituto Nazionale Neurologico, Italy) to present data on
Italian cases.
• Dr Anne Buschmann or Dr Martin Groschup (Friedrich-
Loeffler-Institut, Germany) to present data on German cases.
22. In addition, Professor Lasmézas (SEAC member) would be invited
to present her research data and Japanese researchers would be
invited to provide written material on Japanese cases.
23. The Chair explained that a position statement for publication on the
SEAC website would be prepared based on discussions at the next
meeting. Although there is likely to be much that is unknown, it
would be important to consider the possible public health
implications of these cases and identify knowledge gaps.


snip...


http://www.seac.gov.uk/minutes/96.pdf


TSS

----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Sent: Saturday, February 24, 2007 9:37 PM
Subject: Re: SEAC ANNUAL REPORT 2006


> ----- Original Message -----
> From: XXXXXXXX XXXXXXXXXXXX(SEAC)
> To: 'flounder9@verizon.net'
> Sent: Friday, February 23, 2007 8:13 AM
> Subject: RE: SEAC ANNUAL REPORT 2006
>
>
> Dear Mr Singeltary
>
> Thank you for your email about a possible relationship between BASE and
> sCJD.
>
> SEAC is continually informed about and considers the emerging science on
> both animal and human TSEs. The committee regularly receives updates from
> the UK's National CJD Surveillance Centre Unit on the epidemiology of vCJD
> and sCJD and is also aware of emerging research on BASE. It is envisaged
> that SEAC will conduct a detailed consideration of the available science and
> the possible animal and human health implications of BASE at its meeting on
> 10th May 2007.
>
> Yours sincerely
>
> XXXXXXXXXXXXXX
>
>
>
> ----------------------------------------------------------------------------
> ----
> From: Terry S. Singeltary Sr. [mailto:flounder9@verizon.net]
> Sent: 02 February 2007 16:44
> To: Bovine Spongiform Encephalopathy
> Cc: SEACsecretariat (AHEG); cjdvoice@yahoogroups.com;
> BLOODCJD@YAHOOGROUPS.COM; madcow@lists.iatp.org; Sustainable Agriculture
> Network Discussion Group
> Subject: SEAC ANNUAL REPORT 2006
>
>
> Date: February 2, 2007 at 8:25 am PST
> SEAC ANNUAL REPORT 2006 (ignoring the obvious)
>
>
> http://www.seac.gov.uk/publicats/annualreport2006.pdf
>
>
> i guess the sporadic CJD cases have all be resolved, and the route and cause
> classified.
> to this day, i cannot figure this out. if BSE farmers died from sporadic
> CJD, and now another study seems to point
> that BSE and BASE may be the same thing, and that BASE does not produce vCJD
> like pathology, but pathology resembling that of sporadic CJD, then why does
> SEAC still seem to put sporadic CJD on the back burners ??? IN fact, SEAC
> could not even speak about it in there 2006 report. would it not seem
> prudent to mention these findings in this 2006 SEAC report ;
>
>
> Other work presented suggested that BSE and bovine amyloidotic spongiform
> encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the
> prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A
> MUTATION FOUND IN CASES OF SPORADIC CJD.
>
>
> snip...
>
>
> http://www.seac.gov.uk/minutes/95.pdf
>
>
> ***These results indicate that BASE is transmissible to humans and suggest
> that BASE is more virulent than
> classical BSE in humans.***
>
>
> 6:30 Close of Day One
>
>
> http://www.healthtech.com/2007/tse/day1.asp
>
>
>
> 64. A member noted that at the recent Neuroprion meeting, a study was
> presented showing that in transgenic mice BSE passaged in sheep may be more
> virulent and infectious to a wider range of species than bovine derived BSE.
>
>
> http://www.seac.gov.uk/minutes/95.pdf
>
>
>
> Asante/Collinge et al, that BSE transmission to the 129-methionine
>
> genotype can lead to an alternate phenotype that is indistinguishable
>
> from type 2 PrPSc, the commonest _sporadic_ CJD;
>
>
> http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm
>
>
>
> I STILL think to ignore these findings below is not only rediculous, but
> indeed very suspicious ;
>
>
> CJD FARMERS WIFE 1989
>
>
> http://www.bseinquiry.gov.uk/files/yb/1989/10/13007001.pdf
>
>
> http://www.bseinquiry.gov.uk/files/yb/1989/10/13003001.pdf
>
>
>
>
> cover-up of 4th farm worker ???
>
>
> http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf
>
>
> http://www.bseinquiry.gov.uk/files/yb/1995/10/20006001.pdf
>
>
> CONFIRMATION OF CJD IN FOURTH FARMER
>
>
> http://www.bseinquiry.gov.uk/files/yb/1995/11/03008001.pdf
>
>
> now story changes from;
>
> SEAC concluded that, if the fourth case were confirmed, it would be
> worrying, especially as all four farmers with CJD would have had BSE
> cases on their farms.
>
> to;
>
> This is not unexpected...
>
> was another farmer expected?
>
> http://www.bseinquiry.gov.uk/files/yb/1995/11/13010001.pdf
>
>
> 4th farmer, and 1st teenager
>
> http://www.bseinquiry.gov.uk/files/yb/1996/02/27003001.pdf
>
>
> 2. snip...
>
>
> Over a 5 year period, which is the time period on which the advice
> from Professor Smith and Dr. Gore was based, and assuming a
> population of 120,000 dairy farm workers, and an annual incidence
> of 1 per million cases of CJD in the general population, a
> DAIRY FARM WORKER IS 5 TIMES MORE LIKELY THAN
> an individual in the general population to develop CJD. Using the
> actual current annual incidence of CJD in the UK of 0.7 per
> million, this figure becomes 7.5 TIMES.
>
> 3. You will recall that the advice provided by Professor Smith in
> 1993 and by Dr. Gore this month used the sub-population of dairy
> farm workers who had had a case of BSE on their farms -
> 63,000, which is approximately half the number of dairy farm
> workers - as a denominator. If the above sums are repeated using
> this denominator population, taking an annual incidence in the general
> population of 1 per million the observed rate in this sub-population
> is 10 TIMES, and taking an annual incidence of 0.7 per million,
> IT IS 15 TIMES (THE ''WORST CASE'' SCENARIO) than
> that in the general population...
>
>
> http://www.bseinquiry.gov.uk/files/yb/1995/01/31004001.pdf
>
>
>
> CJD YOUNG PEOPLE
>
> in the USA, a 16 year old in 1978;
>
> ALSO IN USA;
>
> (20 year old died from sCJD in USA in 1980 and a 16 year
> old in 1981. see second url below)
>
> in France, a 19 year old in 1982;
>
> in Canada, a 14 year old of UK origin in 1988;
>
> in Poland, cases in people aged 19, 23, and 27 were identified in
> a retrospective study (published 1991), having been originally
> misdiagnosed with a viral encephalitis;
>
> Creutzfeldt's first patient in 1923 was aged 23.
>
> http://www.bseinquiry.gov.uk/files/yb/1995/10/27013001.pdf
>
>
> 20 year old died from sCJD in USA in 1980 and a 16 year
> old in 1981. A 19 year old died from sCJD in
> France in 1985. There is no evidence of an iatrogenic
> cause for those cases....
>
> http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf
>
>
>
>
> FOR SEAC TO continue to flounder with this ukbsenvcjd only theory, when we
> know that sporadic CJD's were proven long ago to transmit via the medical
> and surgical arena, will only continue to spread this agent around the globe
> via a multitude of proven routes and sources.
>
> IN my opinion, for SEAC to continue to go down this same road, year after
> year, and continue to ignore sporadic CJD's, and continue to say that "BSE
> in the UK sheep population is most likely to be zero, or very low if present
> at all." "Consequently, any impact of the schemes on human health from
> removing BSE from sheep is likely to be negligible." when in fact they do
> not know. and in fact science is showing that in "transgenic mice BSE
> passaged in sheep may be more virulent and infectious to a wider range of
> species than bovine derived BSE." IN my opinion this continued neglect of
> other TSEs in human and animals, the continued belief in this ukbsenvcjd
> only theory, and the risk thereof, is reckless, dangerous, and in my mind,
> criminal. SEAC has failed the public terribly in this regard. ...TSS
>
>
>
>
> ----- Original Message -----
> From: "Terry S. Singeltary Sr."
> To:
> Sent: Friday, February 02, 2007 10:43 AM
> Subject: SEAC ANNUAL REPORT 2006
>
>
> Date: February 2, 2007 at 8:25 am PST
> SEAC ANNUAL REPORT 2006 (ignoring the obvious)
>
>
> http://www.seac.gov.uk/publicats/annualreport2006.pdf
>
>
> i guess the sporadic CJD cases have all be resolved, and the route and cause
> classified.
> to this day, i cannot figure this out. if BSE farmers died from sporadic
> CJD, and now another study seems to point
> that BSE and BASE may be the same thing, and that BASE does not produce vCJD
> like pathology, but pathology resembling that of sporadic CJD, then why does
> SEAC still seem to put sporadic CJD on the back burners ??? IN fact, SEAC
> could not even speak about it in there 2006 report. would it not seem
> prudent to mention these findings in this 2006 SEAC report ;
> ........SNIP.........END............TSS
>



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