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UK's first case of H-type BSE confirmed by DEFRA

flounder

Well-known member
Subject: UK's first case of H-type BSE confirmed by DEFRA
Date: March 9, 2007 at 11:03 am PST
UK's first case of H-type BSE confirmed by DEFRA

09/03/2007 16:28:00
FWi
DEFRA has reported the UK’s first case of H-type Bovine Spongiform Encephalopathy. During routine research carried out by the Veterinary Laboratories Agency, test results revealed a single case of H-type BSE in 2005.

News of this form of BSE, which produces no clinical signs and has only been found in older animals, was announced at a VLA symposium on prion diseases of domestic livestock in June 2006.


To date 18 cases of the H-type strain have been identified in 12 countries worldwide including several other European countries, Japan, Canada and the United States of America.

According to statement from DEFRA the cow died on a farm in Dumfries and Galloway at 13 years of age. The carcass was then sampled and tested for BSE in accordance with EU requirements for testing all fallen stock aged over 24 months.

Test results confirmed the presence of BSE. The carcass was incinerated and the offspring from this cow were culled in line with current BSE regulations.

In August 2006 the VLA began a retrospective examination of brain samples taken from previous BSE cases as part of a research programme designed to analyse different types of BSE in the national herd.


It was this exercise that identified the presence of a H-type case. All forms of BSE are treated identically therefore no additional action would have been required in this case.

The Spongiform Encephalopathy Advisory Committee will examine all the available data on this case during a discussion of different forms of BSE worldwide at their next meeting on 10 May 2007.

by Andrew Watts (About this Author)


http://www.fwi.co.uk/Articles/2007/03/09/102241/uks-first-case-of-h-type-bse-confirmed-by-defra.html




U.K. TSE RESEARCH 2005-2008

http://www.defra.gov.uk/animalh/Bse/pdf/tse-strategy0606.pdf


BSE cases in older cattle which resemble the two different atypical. BSE phenotypes that have recently been described in France. (designated H-type) and ...


http://www.seac.gov.uk/papers/96-2.pdf




----- Original Message -----
From: XXXXXXXX XXXXXXXXXXXX(SEAC)
To: '[email protected]'
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:[email protected]]
Sent: 02 February 2007 16:44
To: Bovine Spongiform Encephalopathy
Cc: SEACsecretariat (AHEG); [email protected];
[email protected]; [email protected]; 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




SOME HISTORY ON THE USA CJD QUESTIONNAIRE




http://www.ask.com/web?q=cjd+questionnaire+tss&qsrc=1&o=0&l=dir



http://www.google.com/search?num=30&hl=en&lr=&as_qdr=d&edition=us&q=cjd+questionnaire+TSS&btnG=Search




One reason for this was the _inaccuracy_ in coding of cases correctly
certified as CJD Coding is carried out by staff who are not medically
qualified and it is not surprising that coding errors occur in the
processing of large numbers of certificates. In 1982, 12,000
certificates per week were processed at the office of population
censuses and surveys bu 15 coders and 6 checkers (Alderson et al., 1983).
The occurrence of both inter- and intra-observer coding errors has been
described (Curb et al., 1983) and the _inaccuracies_ of BOTH
certification and coding discovered in this study _support_ the
introduction of a more accurate system of death certificates and
a more detailed and specific coding system...

snip...

http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf




''Answering critics like Terry Singeltary, who feels that the US
undercounts CJD, Schonberger _conceded_ that the current surveillance
system has errors but stated that most of the errors will be confined to
the older population''...



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SAD THAT OLDER FOLKS SEEM TO BE EXPENDABLE IN RELATIONS TO TSEs.

IF YOUR NOT YOUNG, IF IT's NOT THE UKBSENVCJD ONLY STRAIN, YOUR OUTA LUCK.

YOUR SPORADIC, YOUR SPONTANEOUS, in short, your expendable. ...




> Number of BSE cases in the EU-25 down again by more than 40 percent in 2006.

> This decreasing trend now seems to occur throughout the EU-25




http://www.fas.usda.gov/gainfiles/200703/146280315.pdf




SEEMS the drastic reduction in BSE cases in the EU due to feed ban would dispute the spontaneous event for BSE, so what do they try and do, convince everyone there is a new strain that happens spontaneously, what a hoot. why not a new strain via the same damn route and source i.e. tainted feed just different strain of agent in feed, or maybe vertical and or horizontal route and or source. but to just throw our hands up in the air and say "OH, it just happens on it's on due to some whaco event of a spontanoeus good protein misfolding into a bad one'' and then replicating themselves into more bad apples, just because we find a new strain of mad cow in the bovine that IS related to sporadic CJDs, flies in the face of past science. it does not compute. and if you go back to those mad cow farmers and one wife, with BSE that died from sporadic CJD, now that does compute.

lets do some pondering here, lets say just for BSe, lets just say this BASE is spontaneous, would this not be the final gold straw in prusiners pie. would every country out there then be subject to 100% testing if say this spontaneous theory was proven, and shoot his cdi testing up big time, and others $$$ holy mad cow, just think about it, over 20+ strains of scrapie, atypical scrapies, BSE in sheep and goat, holy smokes, then you have the multiple strains of CWD they have just about finally confirmed of deer and elk, but how many strains there, alot of testing would have to be done if ever proven to be a spontaneous event. but how can it be spontaneous in one and not the others $$$

i have ask prusiner this very thing, with no answer. how many of the 85%+ of all human TSE i.e. sporadic CJD, how many do you believe to be a spontaneous event, from no route and source, knowing that we have to date 6 different phenotypes of sporadic CJD ??? what, 2%, 10%, 25%, 50%, 75%, or all of the sporadic CJDs spontaneous ??? no explanation yet from him, but i'm still waiting. ...

see Prusiner ;

http://www.agobservatory.org/library.cfm?refID=30405




see singeltary question to prusiner ;

Greetings,as a lay person;-) i am thankful for Dr. Prusiners report below. I only wish that he would elaborate on the spontaneous aspect of sporadic CJD and how many of the 85%+ of all CJDs does he think happens spontaneously without route and source of the agent? I am concerned that people who read this, will come to the conclusion that all sporadic CJDs are a spontaneousmutation, when in reality all sporadic CJD is, is CJD from unknown route and source and they could be many. in fact, there could be many phenotypes of CJD that are now called sporadic CJD...


http://www.agobservatory.org/library.cfm?refID=30406




THE USDA JUNE 2004 ENHANCED BSE SURVEILLANCE PROGRAM WAS TERRIBLY FLAWED ;


CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006


The U.S. Department of Agriculture was quick to assure the public earlier
this week that the third case of mad cow disease did not pose a risk to
them, but what federal officials have not acknowledged is that this latest
case indicates the deadly disease has been circulating in U.S. herds for at
least a decade.

The second case, which was detected last year in a Texas cow and which USDA
officials were reluctant to verify, was approximately 12 years old.

These two cases (the latest was detected in an Alabama cow) present a
picture of the disease having been here for 10 years or so, since it is
thought that cows usually contract the disease from contaminated feed they
consume as calves. The concern is that humans can contract a fatal,
incurable, brain-wasting illness from consuming beef products contaminated
with the mad cow pathogen.

"The fact the Texas cow showed up fairly clearly implied the existence of
other undetected cases," Dr. Paul Brown, former medical director of the
National Institutes of Health's Laboratory for Central Nervous System
Studies and an expert on mad cow-like diseases, told United Press
International. "The question was, 'How many?' and we still can't answer
that."

Brown, who is preparing a scientific paper based on the latest two mad cow
cases to estimate the maximum number of infected cows that occurred in the
United States, said he has "absolutely no confidence in USDA tests before
one year ago" because of the agency's reluctance to retest the Texas cow
that initially tested positive.

USDA officials finally retested the cow and confirmed it was infected seven
months later, but only at the insistence of the agency's inspector general.

"Everything they did on the Texas cow makes everything USDA did before 2005
suspect," Brown said. ...snip...end


http://www.upi.com/ConsumerHealthDaily/view.php?StoryID=20060315-055557-1284r


CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ...
Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central
Nervous System ... Address for correspondence: Paul Brown, Building 36, Room
4A-05, ...


http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm





PAUL BROWN COMMENT TO ME ON THIS ISSUE

Tuesday, September 12, 2006 11:10 AM


"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years,
and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the
USDA and the Canadian Food Agency."


OR, what the Honorable Phyllis Fong of the OIG found ;


Audit Report

Animal and Plant Health Inspection Service

Bovine Spongiform Encephalopathy (BSE) Surveillance Program – Phase II

and

Food Safety and Inspection Service

Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat Recovery Products - Phase III

Report No. 50601-10-KC January 2006

Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle Still Remain


http://www.usda.gov/oig/webdocs/50601-10-KC.pdf



THE SEVEN SCIENTIST REPORT ***


http://www.fda.gov/ohrms/dockets/dockets/02n0273/02n-0273-EC244-Attach-1.pdf


Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
Spongiform Encephalopathy (BSE)


http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf




[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk
Materials for Human Food and Requirement for the Disposition of
Non-Ambulatory Disabled Cattle

9/13/2005

http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf




Docket No. 03-080-1 -- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL IMPORTS FROM CANADA 11/03/2003 01:19 PM

https://web01.aphis.usda.gov/BSEcom.nsf/0/b78ba677e2b0c12185256dd300649f9d?OpenDocument&AutoFramed




Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW BSE SAFEGUARDS 07/11/2004 09:34 PM

https://web01.aphis.usda.gov/regpublic.nsf/168556f5aa7a82ba85256ed00044eb1f/eff9eff1f7c5cf2b87256ecf000df08d?OpenDocument




Importation of Whole Cuts of Boneless Beef From Japan

[Docket No. 05-004-2] RIN 0579-AB93


snip...


Peripheral Nerves

Issue: Two commenters stated that the underlying assumption of the
proposed rule, that whole cuts of boneless beef from Japan will not
contain tissues that may carry the BSE agent, is no longer valid
because researchers have found peripheral nervous system tissues,
including facial and sciatic nerves, that contain BSE infectivity.\2\
One of these commenters requested APHIS to explain whether and what
additional mitigation measures are needed to reduce the risks that
these tissues may be present in Japanese beef. This commenter further
requested an additional comment period to obtain public comment
regarding the manner by which APHIS intends to treat this new
scientific finding.
---------------------------------------------------------------------------

\2\ Bushmann, A., and Gruschup, M.; Highly Bovine Spongiform
Encephalopathy-Sensitive Transgenic Mice Confirm the Essential
Restriction of Infectivity to the Nervous System in Clinically
Diseased Cattle. The Journal of Infectious Diseases, 192: 934-42,
September 1, 2005.
---------------------------------------------------------------------------

Response: APHIS is familiar with the results of the study mentioned
by the commenters in which mice, genetically engineered to be highly
susceptible to BSE and to overexpress the bovine prion protein, were
inoculated with tissues from a BSE-infected cow. This study
demonstrated low levels of infectivity in the mouse assay in the facial
and sciatic nerves of the peripheral nervous system. APHIS has
evaluated these findings in the context of the potential occurrence of
infectivity in the peripheral nerves of cattle and the corresponding
risks of the presence of infectivity in such tissues resulting in
cattle or human exposure to the BSE agent. The results from these
experiments in genetically engineered mice should be interpreted with
caution, as the findings may be influenced by the overexpression of
prion proteins and may not accurately predict the natural distribution
of BSE infectivity in cattle. Further, the overexpression of prion
proteins in transgenic mice may not accurately mimic the natural
disease process because the transgenic overexpressing mice have been
shown to develop spontaneous lethal neurological disease involving
spongiform changes in the brain and muscle degeneration.\3\ In
addition, the route of administration to the mice was both
intraperitoneal and intracerebral, which are two very efficient routes
of infection as compared to oral consumption. Given these factors,
APHIS has determined that the finding of BSE infectivity in facial and
sciatic nerves of the transgenic mice is not directly applicable to
cattle naturally infected with BSE. Therefore, we do not consider it
necessary to make any adjustments to the risk analysis for this
rulemaking or to extend the comment period to solicit additional public
comment on this issue.
---------------------------------------------------------------------------

\3\ Westaway, D., et al.; (1994) Degeneration of Skeletal
Muscle, Peripheral Nerves, and the Central Nervous System in
Transgenic Mice Overexpressing Wild-type Prion Proteins. Cell 76, 117-129.
---------------------------------------------------------------------------

Blood

Issue: Two commenters expressed concern that there has been a
limited amount of research conducted on BSE infectivity in blood. One
of these commenters cited a report that discussed, among other things,
the detection of infectivity in sheep experimentally infected with BSE
via blood transfusions.\4\ This commenter also stated that the agent
that causes Creutzfeldt-Jakob disease (CJD), a chronic and fatal
neurodegenerative disease of humans, was detected in blood, and
questioned whether the BSE agent could be detected in blood as well.
The other commenter cited a study that detected infectivity in hamsters
experimentally infected with scrapie.\5\ This commenter requested that
APHIS ban the use of blood in cattle feed.
---------------------------------------------------------------------------

\4\ Pattison, J., et al.; UK Strategy for Research and
Development on Human and Animal Health Aspects of Transmissible
Spongiform Encephalopathies, 2005-2008. Available at
http://www.mrc.ac.uk/pdf-about-tse_uk_strategy_june2005.pdf.
\5\ Castilla, J., et al.; Detection of Prions in Blood. Nature
Medicine, doi: 10.1038/nm1286, August 28, 2005, at 3.
---------------------------------------------------------------------------

Response: As stated in our risk analysis, the pathogenesis studies
of naturally and experimentally infected cattle have not detected BSE
infectivity in blood.
The first study mentioned by the commenter above demonstrated
transmission of disease from sheep experimentally infected with BSE to
another sheep via blood transfusions. We note that there are widely
acknowledged differences between the distribution of BSE infectivity in
the tissues of cattle and sheep. In addition, there is a significant
difference in susceptibility to infection based on the route of
transmission. Infection via oral consumption may be 10,000 times less
efficient than infection via intravenous injection, such as a blood
transfusion.
Both the United Kingdom's Department for Environment, Food and
Rural Affairs' Spongiform Encephalopathy Advisory Committee (SEAC) and
the European Commission's Scientific Steering Committee (SSC), which
are scientific advisory committees, evaluated the findings of
transmission of infectivity via blood transfusions in sheep
experimentally infected with BSE and concluded that

[[Page 73907]]

these findings did not indicate that additional mitigation measures
were necessary to protect public health.\6\ Therefore, based on
currently available information, APHIS considers it unlikely that the
experimental observations in sheep reflect a biologically significant
event for cattle or affect the safety of whole cuts of boneless beef
derived from cattle born, raised, and slaughtered in Japan.
---------------------------------------------------------------------------

\6\ Spongiform Encephalopathy Advisory Committee, Oct. 19, 2000,
Summary of SEAC Committee Meeting 29 September 2000. Available at
http://www.defra.gov.uk/news/seac/seac500.htm.
European Commission Scientific Steering Committee; The
Implications of the Recent Papers on Transmission of BSE by Blood
Transfusion in Sheep (Houston et al., 2000; Hunter et al., 2002),
Adopted by the SSC at its Meeting of 12-13 September. Available at
http://europa.eu.int/comm/food/fs/sc/ssc/out280_en.pdf.

---------------------------------------------------------------------------

The study on scrapie-infected hamsters noted by the commenter
describes a process by which the abnormal prion protein can be
amplified and detected using current testing methods, such as a Western
blot. In this study, blood from hamsters experimentally infected with a
scrapie strain was collected when the animals demonstrated clinical
signs of disease. These blood samples were incubated with excess normal
prion protein from brain tissue for multiple cycles. If abnormal
protein is present in blood, it will convert the normal brain prion to
abnormal prion, yielding an increased amount of abnormal prion that can
be more easily detected. In this manner, the presence of abnormal prion
protein in the initial blood samples, which was present in levels too
low to detect using routine test methods, was demonstrated. While this
finding has many possibilities related to the development of diagnostic
tests, it does not demonstrate BSE infectivity in blood. We also note
that the international community largely considers that studies using
transmissible spongiform encephalopathies (TSEs) other than BSE in non-
bovine animals cannot be directly extrapolated to BSE in cattle because
of the significant interactions between the host species and the prion
strain involved.
Feed regulations in the United States are under the authority of
the Food and Drug Administration (FDA), not APHIS. Therefore, the
commenter's request that APHIS ban the use of blood in cattle feed
falls outside the scope of this rulemaking. For these reasons, we are
not making any changes to the rule based on these comments.


snip...


Issue: Two commenters raised questions regarding the origin of CJD
in humans. One commenter noted that there are different strains of TSEs
being discovered in ruminants, and that new atypical strains of TSE in
cattle look similar to sporadic CJD in humans. Another commenter asked
if APHIS has considered whether sporadic CJD in humans might be caused
by atypical cases of TSEs that have been found in animals. This
commenter further questioned whether blood and other tissues may carry
BSE infectivity in cattle infected with atypical strains of the BSE
agent or other TSE agents.
Response: Sporadic CJD is the most common form of CJD. It has been
found in every country in the world where it has been looked for
including countries that are generally considered by the international
scientific community to be free of BSE and other TSEs (for example,
Australia and New Zealand). In general, it affects about one person per
million. No association between sporadic CJD and consumption of animal
products in general and/or infected or contaminated bovine products has
ever been documented. It is currently believed that sporadic CJD arises
through the spontaneous conversion of PrPC (normal cellular
prion protein) to PrPSC in an individual.\13\ In contrast,
atypical cases of BSE in cattle are rare and have been reported in only
few countries that experience BSE, such as Italy, Belgium, Japan, and
France. It has been speculated that the spontaneous or sporadic form of
BSE could exist in cattle, as well as humans.\14\
---------------------------------------------------------------------------

\13\ Stahl, N. and Prusiner, S.B.; (1991) FASEB-J. 5: 2799-807.
\14\ Biacabe; 2004 EMBO reports, Vol. 5, No. 1.

---------------------------------------------------------------------------

[[Page 73916]]

APHIS agrees with the commenter that reports indicate that some of
the atypical BSE cases, in particular the bovine amyloidotic spongiform
encephalopathy (BASE), and sporadic CJD have similar PrPSC
patterns. APHIS evaluated the findings in the context of risk of
exposure to cattle and humans. Currently, the relevance of the atypical
cases is unknown, but at this time there is no indication that any
control measures--such as feed bans or SRM requirements--should be
modified based on these cases. Additionally, although atypical cases of
BSE and sporadic CJD share similarities at this point, there is no
evidence that they are linked.
Issue: One commenter expressed concern over the number of citations
issued for various SRM violations during the June 2004 enhanced BSE
surveillance program in the United States. This commenter questioned
whether these incidents of noncompliance may have led to infective
materials entering the human or animal food chains. This commenter
cited the case of BSE detected in a 12-year-old cow in Texas as
evidence that infective materials may have entered the food chain. The
commenter suggested that noncompliance reports should be made more
easily available to the public in the future.
Response: FSIS inspectors are responsible for verifying the
effectiveness of an establishment's procedures. If FSIS personnel
determine that an establishment's procedures are ineffective in
preventing cross-contamination, the inspectors will take appropriate
action. We note that none of the meat from the 12-year-old BSE-infected
cow in Texas mentioned by the commenter entered the human food or
animal feed chains.


snip...full text ;



http://www.epa.gov/fedrgstr/EPA-IMPACT/2005/December/Day-14/i24057.htm




Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION


http://docket.epa.gov/edkfed/do/EDKStaffItemDetailView?objectId=090007d480993808




http://docket.epa.gov/edkfed/do/EDKStaffAttachDownloadPDF?objectId=090007d480993808




http://docket.epa.gov/edkfed/do/EDKStaffCollectionDetailView?objectId=0b0007d48096b40d





Docket No. 05-004-1 RIN 0579-AB93 BSE TSS was Received


I would kindly like to comment on [Docket No. 05-004-1] RIN 0579-AB93 ;

PROPOSED RULES
Exportation and importation of animals and animal products:
Whole cuts of boneless beef from-
Japan,
48494-48500 [05-16422]


[Federal Register: August 18, 2005 (Volume 70, Number 159)]
[Proposed Rules]
[Page 48494-48500]
From the Federal Register Online via GPO Access [wais.access.gpo.gov]
[DOCID:fr18au05-7]

========================================================================
Proposed Rules
Federal Register
________________________________________________________________________

This section of the FEDERAL REGISTER contains notices to the public of
the proposed issuance of rules and regulations. The purpose of these
notices is to give interested persons an opportunity to participate in
the rule making prior to the adoption of the final rules.

========================================================================


[[Page 48494]]


DEPARTMENT OF AGRICULTURE

Animal and Plant Health Inspection Service

9 CFR Part 94

[Docket No. 05-004-1]
RIN 0579-AB93


Importation of Whole Cuts of Boneless Beef from Japan

AGENCY: Animal and Plant Health Inspection Service, USDA.

ACTION: Proposed rule.

-----------------------------------------------------------------------

SUMMARY: We are proposing to amend the regulations governing the
importation of meat and other edible animal products by allowing, under
certain conditions, the importation of whole cuts of boneless beef from
Japan. We are proposing this action in response to a request from the
Government of Japan and after conducting an analysis of the risk that
indicates that such beef can be safely imported from Japan under the
conditions described in this proposal.

DATES: We will consider all comments that we receive on or before
September 19, 2005.

ADDRESSES: You may submit comments by any of the following methods:
EDOCKET: Go to http://www.epa.gov/feddocket to submit or


snip...


BSE infectivity has never been demonstrated in the muscle tissue of
cattle experimentally or naturally infected with BSE at any stage of
the disease. Studies performed using TSEs other than BSE in non-bovine
animals have detected prions in muscle tissue. However, the
international scientific community largely considers that these studies
cannot be directly extrapolated to BSE in cattle because of the
significant interactions between the host species and the prion strain
involved.
Pathogenesis studies of naturally and experimentally infected
cattle have not detected BSE infectivity in blood. However,
transmission of BSE was demonstrated in sheep that received a
transfusion of a large volume of blood drawn from other sheep that were
experimentally infected with the BSE agent. The United Kingdom's
Department for Environment, Food and Rural Affairs' Spongiform
Encephalopathy Advisory Committee (SEAC) and the European Commission's
Scientific Steering Committee (SSC), which are scientific advisory
committees, evaluated the implication of this finding in relation to
food safety.\5\ The SEAC concluded that the finding did not represent
grounds for recommending any changes to the current control measures
for BSE. The SSC determined that the research results do not support
the hypothesis that bovine blood or muscle meat constitute a risk to
human health.\6\


snip...


BSE Risk Factors for Whole Cuts of Boneless Beef


The most significant risk management strategy for ensuring the
safety of whole cuts of boneless beef is the prevention of cross-
contamination of the beef with SRMs during stunning and slaughter of
the animal. Control measures that prevent contamination of such beef
involve the establishment of procedures for the removal of SRMs,
prohibitions on air-injection stunning and pithing, and splitting of
carcasses. These potential pathways for contamination and the control
measures that prevent contamination are described in detail in the risk
analysis for this rulemaking.
SRM Removal. Research has demonstrated that SRMs from infected
cattle may contain BSE infectivity. Because infectivity has not been
demonstrated in muscle tissue, the most important mitigation measure
for whole cuts of boneless beef is the careful removal and segregation
of SRMs. Removal of SRMs in a manner that avoids contamination of the
beef with SRMs minimizes the risk of exposure to materials that have
been demonstrated to contain the BSE agent in cattle.

snip...


Variant Creutzfeldt-Jakob disease (vCJD), a chronic and fatal
neurodegenerative disease of humans, has been linked since 1996 through
epidemiological, neuropathological, and experimental data to exposure
to the BSE agent, most likely through consumption of cattle products
contaminated with the agent before BSE control measures were in place.
To date, approximately 170 probable and confirmed cases of vCJD have
been identified worldwide. The majority of these cases have either been
identified in the United Kingdom or were linked to exposure that
occurred in the United Kingdom, and all cases have been linked to
exposure in countries with native cases of BSE. Some studies estimate
that more than 1 million cattle may have been infected with BSE
throughout the epidemic in the United Kingdom. This number of infected
cattle could have introduced a significant amount of infectivity into
the human food supply. Yet, the low number of cases of vCJD identified
to date indicates that there is a substantial species barrier that
protects humans from widespread illness due to exposure to the BSE
agent.


snip...


International Guidelines on BSE

International guidelines for trade in animal and animal products
are developed by the World Organization for Animal Health (formerly
known as the Office International des Epizooties (OIE)), which is
recognized by the World Trade Organization (WTO) as the international
organization responsible for the development of standards, guidelines,
and recommendations with respect to animal health and zoonoses
(diseases that are transmissible from animals to humans). The OIE
guidelines for trade in terrestrial animals (mammals, birds, and bees)
are detailed in the Terrestrial Animal Health Code (available on the
internet at http://www.oie.int). The guidelines on BSE are contained in

Chapter 2.3.13 of the Code and supplemented by Appendix 3.8.4 of the
Code.


snip...end


http://a257.g.akamaitech.net/7/257/2422/01jan20051800/edocket.access.gpo.gov/2005/05-16422.htm

http://a257.g.akamaitech.net/7/257/2422/01jan20051800/edocket.access.gpo.gov/2005/pdf/05-16422.pdf



Greetings again APHIS ET AL,


THIS is not correct. IN fact, there are several factors i would like to kindly address. .......


SNIP...


WE MUST ADHERE TO THE BSE GBR RISK ASSESSMENTS, WE MUST WORK TO ENHANCE THOSE BSE GBR RISK ASSESSMENTS TO INCLUDE ALL ANIMAL TSEs, USDA/APHIS/GW ET ALs BSE MRR (Minimal Risk Region) should be REPEALED/DISBANDED/TRASHED/NADA and done away with for good. The BSE MRR policy is nothing more than a legal tool to trade all strains of TSEs globally...


Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518


Your Comment with Title "[Docket No. 05-004-1] RIN 0579-AB93 BSE TSS " was Received.
The Identifier Assigned is "APHIS-2005-0073-0009".
An Electronic File was Attached to this Submission.

Please note that it may take between 24 and 72 hours for the EDOCKET staff to process your comment before it is available publicly through EDOCKET. You can use the identifier noted above to find your comment through the Quick or Advanced Search pages when it is available. ...........


http://www.aphis.usda.gov/lpa/news/2005/08/japanbeef_vs.html




EPA: Federal Register: Importation of Whole Cuts of Boneless Beef ...Importation of Whole Cuts of Boneless Beef From Japan , Federal Register document. ... we published in the Federal Register (70 FR 48494-48500, Docket No. ...


http://www.epa.gov/fedrgstr/EPA-IMPACT/2005/December/Day-14/i24057.htm




Importation of Whole Cuts of Boneless Beef from Japan [Docket No. 05-004-1] RIN 0579-AB93 TSS SUBMISSION



next 3 urls now dead ;


http://docket.epa.gov/edkfed/do/EDKStaffItemDetailView?objectId=090007d480993808




http://docket.epa.gov/edkfed/do/EDKStaffAttachDownloadPDF?objectId=090007d480993808




http://docket.epa.gov/edkfed/do/EDKStaffCollectionDetailView?objectId=0b0007d48096b40d




"[Docket No. 05-004-1] RIN 0579-AB93 BSE TSS " was Received



BSE; MRR; IMPORTATION OF LIVE BOVINES AND PRODUCTS DERIVED FROM BOVINES [Docket No. APHIS-2006-0041] RIN 0579-AC01





APHIS-2006-0041-0006 Comment from Terry S Singletary Sr 01/09/2007 PUBLIC SUBMISSIONS





http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3412&disposition=attachment&contentType=crtext




APHIS-2006-0041-0006.1 Attachment to Singletary comment 01/09/2007 PUBLIC SUBMISSIONS



http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8






TSS
 

PORKER

Well-known member
***These results indicate that BASE is transmissible to humans and suggest
that BASE is more virulent than
classical BSE in humans.***
 

flounder

Well-known member
Subject: Re: UK's first case of H-type BSE confirmed by DEFRA
Date: March 12, 2007 at 10:46 am PST

INFORMATION BULLETIN

Nobel House, 17 Smith Square, London SW1P 3JR
Out of hours telephone 020 7270 8960 Ref: 71/07
Date: 9 March 2007


2005 case of H-type BSE in a cow

During routine research carried out by the Veterinary Laboratories Agency
(VLA), test results have revealed a single case of H-type Bovine Spongiform
Encephalopathy (BSE) in the UK in 2005. This form of BSE has already been
identified in several other European countries in addition to Japan, Canada
and the United States of America.

The cow died on a farm in Dumfries and Galloway at 13 years of age. The
carcass was sampled and tested for BSE in accordance with EU requirements
for testing all fallen stock aged over 24 months. Test results confirmed the
presence of BSE. The carcass was incinerated and the offspring from this cow
were culled in line with current BSE regulations.

In August 2006 the VLA began a retrospective examination of brain samples
taken from previous BSE cases as part of a research programme designed to
analyse different types of BSE in the national herd. The aim of the study is
to determine whether unusual cases of BSE, had in fact occurred in the UK in
the past. The studies are ongoing however this case has been identified as
H-type. All forms of BSE are treated identically therefore no additional
action would have been required in this case.

The Spongiform Encephalopathy Advisory Committee (SEAC) will examine all the
available data on this case during a discussion of different forms of BSE
worldwide at their next meeting on 10 th May 2007.


Notes to editors
1) SEAC briefly discussed information on the different forms of BSE
worldwide, at their February 2007 meeting. The background papers for that
meeting are available at http://www.seac.gov.uk/agenda/agen200207.htm

2) Japan, the USA, Canada and several European countries (including France,
the Netherlands, Sweden, Switzerland, and Germany) have reported H-type BSE.
Japan and several European countries (including Italy, Denmark, Poland,
Belgium and Germany) have reported L-type BSE. Both types have been reported
predominantly in cattle over 10 years old. In both types, the abnormal prion
protein (PrP Sc ) in these animals has different biochemical characteristics
to that seen in BSE. Mice experimentally infected with the brains of these
animals develop a disease which differs from that seen in BSE infected mice.
In two L-type cases where the intact whole brain was studied, the pattern of
deposition of PrP Sc in the brain differed from that in BSE.


http://www.defra.gov.uk/news/2007/070309b.htm


QUESTIONs FIRST PLEASE, maybe someone can answer this for me.


DOES H-type BSE found now in the U.K., and other EU countries, Japan, Canada
and the USA, does this pathology look like sporadic CJD also, like the
L-type BSE found in Japan and several European countries (including Italy,
Denmark, Poland, Belgium and Germany), as opposed to the nvCJD pathology
being like that of typical BSE ???

IN other words, does both H-type and L-type BSE differ from the BSE in
relations to the nvCJD pathology in humans, and does both H-type and L-type
BSE pathology look like some sporadic CJD phenotypes ???


FINALLY, in the paper submitted here, i found this very interesting ;


9. Atypical Bovine Spongiform Encephalopathy case associated with a prion
protein ?gent mutation

snip...

HERE we report a bovine BSE case associated with a novel prion protein gene
(Prnp) polymorphism resulting in an amino acid substitution. The animal
carrying this mutation was positive for the abnormal prion protein, PrPBSE,
in brainstem tissue. Western blot characterization of the PrPBSE of this
animal revealed a high molecular weight phenotype of BSE when compared to
PrPBSE from typical BSE isolates. AN IDENTICAL MUTATION IN THE HUMAN PRION
PROTEIN SEQUENCE HOMOLOGUE HAS BEEN PREVIOUSLY DESCRIBED AS THE MOST COMMON
CAUSE OF THE GENETIC HUMAN PRION DISEASES (gCJD, FFI and GSS). ...page
8...end


confusious is a bit confused here.....


thank you, kind regards,
terry


CONSIDERATION OF FUTURE DISCUSSION OF UNUSUAL CASES OF SPONGIFORM
ENCEPHALOPATHY IN CATTLE


10. TRANSMISSION OF BOVINE AMYLOIDOTIC SPONGIFORM ENCEPHALOPATHY (BASE) TO
THE PRIMATE


snip...


molecular similarities have been described between two case of BASE found in
Italy and a subtype of sporadic CJD...

snip...

Our observations underline the high susceptibility of a primate species to
the BASE prion strain and provide a biochemical basis for the identification
of a potential occurence in man. ...page 9...end


14. STUDIES ON THE SECOND ATYIPCAL BSE CASE IN A JAPANESE BLACK COW


snip...

SUCH a glycosylation-ratio is distinct from that of the typical BSE agent in
which the di-glycosylated form is dominant (approx. 70%) but, INTRIGUINGLY,
SIMILAR TO THAT OF THE TYPE-2 SPORADIC CJD AGENT.

snip...


JUDGING from the glycosylation-ration, BSE prion herein is different from
the typical BSE prion, and the atypical BSE prion found previously in a
Holstein steer in Japan. INSTEAD, its molecular feature is close, IF NOT
IDENTICAL, to PrPSc found in the cattle succumbed to bovine amyloidtic
spongiform encephalopathy, and to the sporadic CJD-like PrPSc in the mice
inoculated with BSE agent. ...page 12...end


http://www.seac.gov.uk/papers/96-2.pdf



Research Project: Study of Atypical Bse
Location: Virus and Prion Diseases of Livestock

2006 Annual Report

4d.Progress report.
This report serves to document research conducted under a specific
cooperative agreement between ARS and the Italian Reference Centre for
Animal TSE (CEA) at the Istituto Zooprofilattico Sperimentale, Turin, Italy.
Additional details of research can be found in the report for the parent
project 3625-32000-073-00D, Transmission, Differentiation, and Pathobiology
of Transmissible Spongiform Encephalopathies (TSEs).

The aim of the cooperative research project is (i) to compare the U.S.
Bovine Spongiform Encephalopathy (BSE) isolates and the atypical BSE
isolates identified in Italy and (ii) to determine whether diagnostic
methods routinely used at the USDA are able to identify atypical BSE cases.

Within FY06, formalin fixed and frozen brain materials from animals with
typical and atypical Italian (BASE) BSE have been sent by CEA to the
USDA-ARS-NADC laboratory in Ames, IA. The serial brain material sections of
BSE and BASE (consecutively numbered) will be analyzed in Ames using the
USDA immunohistochemistry (IHC) protocol. To evaluate its reproducibility in
Italian laboratories and to standardize the method, the USDA IHC protocol is
being established at the CEA neuropathology laboratory. As soon as the
Ventana NexES IHC Staining System is available to the CEA, the same samples
(different cut numbers) will be examined by the CEA using the CEA in-house
and the USDA IHC protocol.

In order to evaluate the USDA Western blot method, about 2 gram of typical
Italian BSE and about 2 gram of atypical BASE brain tissue have been sent to
the USDA-ARS-NADC laboratory. These samples and U.S. typical and atypical
BSE samples have been analyzed in parallel using both the USDA and CEA
Western blot methods and three different monoclonal antibodies (6H4, P4, SAF
84). These studies were performed during the visit by a CEA collaborator to
the USDA-ARS-NADC. The latter studies revealed that both the Italian and
USDA extraction and Western blot methods allowed the identification of the
typical and atypical BSE samples tested.


http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=408490&showpars=true&fy=2006


ATYPICAL BSE BACKGROUND HISTORY USA


Research Project: Study of Atypical Bse

Location: Virus and Prion Diseases of Livestock

Project Number: 3625-32000-073-07
Project Type: Specific C/A


Start Date: Sep 15, 2004
End Date: Sep 14, 2007


Objective:
The objective of this cooperative research project with Dr. Maria Caramelli
from the Italian BSE Reference Laboratory in Turin, Italy, is to conduct
comparative studies with the U.S. bovine spongiform encephalopathy (BSE)
isolate and the atypical BSE isolates identified in Italy. The studies will
cover the following areas: 1. Evaluation of present diagnostics tools used
in the U.S. for the detection of atypical BSE cases. 2. Molecular comparison
of the U.S. BSE isolate and other typical BSE isolates with atypical BSE
cases. 3. Studies on transmissibility and tissue distribution of atypical
BSE isolates in cattle and other species.

Approach:
This project will be done as a Specific Cooperative Agreement with the
Italian BSE Reference Laboratory, Istituto Zooprofilattico Sperimentale del
Piemonte, in Turin, Italy. It is essential for the U.S. BSE surveillance
program to analyze the effectiveness of the U.S diagnostic tools for
detection of atypical cases of BSE. Molecular comparisons of the U.S. BSE
isolate with atypical BSE isolates will provide further characterization of
the U.S. BSE isolate. Transmission studies are already underway using brain
homogenates from atypical BSE cases into mice, cattle and sheep. It will be
critical to see whether the atypical BSE isolates behave similarly to
typical BSE isolates in terms of transmissibility and disease pathogenesis.
If transmission occurs, tissue distribution comparisons will be made between
cattle infected with the atypical BSE isolate and the U.S. BSE isolate.
Differences in tissue distribution could require new regulations regarding
specific risk material (SRM) removal.


http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=408490


2005 Annual Report


4d.Progress report.
This report serves to document research conducted under a specific
cooperative agreement between ARS and the Italian Reference Centre for
Animal TSE (CEA) at the Istituto Zooprofilattico Sperimentale, Turin, Italy.
Additional details of research can be found in then report for the parent
project 3625-32000-073-00D, Transmission, Differentiation, and Pathobiology
of Transmissible Spongiform Encephalopathies. The aim of the cooperative
research project conducted by the CEA and ARS is to compare the U.S. bovine
spongiform encephalopathy (BSE) isolate and the bovine amyloidotic
spongiform encephalopathy isolates (BASE) identified in Italy. The first
objective was to determine whether diagnostic methods routinely used by USDA
are able to identify the Italian BASE cases. For this purpose, CEA received
the immunohistochemistry (IHC) protocol developed by APHIS-USDA. The IHC
protocol was reproduced and standardized in the CEA laboratory and will be
applied to the Italian BSE and BASE cases. Furthermore, fixed brainstem
sections and frozen brainstem material from Italian BSE and BASE cases will
be sent to ARS for analysis using USDA IHC and Western blot (WB) methods.
These studies will enable us to determine whether the present diagnostic
tools (IHC and WB) employed at the USDA will be able to detect the Italian
BASE cases and also enable us to compare Italian BSE and BASE with the U.S.
BSE cases.


http://www.ars.usda.gov/research/projects/projects.htm?ACCN_NO=408490&showpars=true&fy=2005


NEW findings of BASE in cattle in Italy of Identification of a
second bovine amyloidotic spongiform encephalopathy: Molecular
similarities with sporadic

Creutzfeldt-Jakob disease


http://www.pnas.org/cgi/content/abstract/0305777101v1


Atypical BSE
Because of its contemporary nature, the study of atypical BSE is very much a
work in progress, with comparatively little published data and many
unknowns. The first 2 cases to be identified were a serendipitous discovery
made in the course of an unrelated experimental study that required a
detailed neuropathologic and immunochemical examination of the entire brain
(5). The absence of clinical signs in these older animals, the unusual
distribution of PrPTSE, together with amyloid plaques, and a Western blot
pattern that differed from the stereotypic pattern seen in typical BSE left
little doubt about the probability that a new "atypical strain" had been
identified (bovine amyloidotic spongiform encephalopathy[BASE]).

Although no further cases were found in over 100 cattle examined in Italy,
the initiation of Western blot studies of animals in other countries with
screening test programs began to yield additional atypical patterns (Table
2, Figure 3) (6–14; P. Lind, pers. comm.). Two major patterns have been
described, named L (resembling the original Italian case pattern with a
lower molecular weight than typical BSE) and H (for a distinct pattern first
seen in France with a higher molecular weight than typical BSE). It is not
yet clear whether other mixed patterns result from technical procedures in
different laboratories or whether a more complicated scheme of
classification will evolve as more atypical patterns are discovered.

In addition, Western blots of PrPTSE are a fragile basis on which to define
a BSE phenotype. Little or no information is available about either the
clinical status or neuropathologic features of these animals. We know that
cases have occurred in different breeds and PrP genotypes, and we also know
that very few of the animals have had the typical clinical picture of BSE
(behavioral disturbances, sensory signs, ataxia, and tremors), but a cloud
of ambiguity surrounds the clinical picture even in those animals for which
an extensive post-hoc investigation was undertaken. The fact that few
detailed neuropathologic results are available is explained by the need to
preserve at least a full half brain for examination, which is presently not
done in any of the various countries that have screening test programs. In
the future, the brain as well as the carcass must be retained in cold
storage until the test results are known.

The frequency of atypical cases is another unknown. Published (7,12) and
unpublished (11,13) observations indicate that in some countries it might be
as high as 5%–10% of the total number of older animals diagnosed by rapid
screening tests (e.g., 2/27 in Germany, and 1/9 in Canada), which would seem
to be a surprisingly high proportion of spontaneously occurring cases.
However, data are not yet sufficient to estimate the overall prevalence of
atypical BSE, i.e., cases per million tested animals of all ages.

In this context, a word is in order about the US testing program. After the
discovery of the first (imported) cow in 2003, the magnitude of testing was
much increased, reaching a level of >400,000 tests in 2005 (Figure 4).
Neither of the 2 more recently indigenously infected older animals with
nonspecific clinical features would have been detected without such testing,
and neither would have been identified as atypical without confirmatory
Western blots. Despite these facts, surveillance has now been decimated to
40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and
invites the accusation that the United States will never know the true
status of its involvement with BSE.

In short, a great deal of further work will need to be done before the
phenotypic features and prevalence of atypical BSE are understood. More than
a single strain may have been present from the beginning of the epidemic,
but this possibility has been overlooked by virtue of the absence of
widespread Western blot confirmatory testing of positive screening test
results; or these new phenotypes may be found, at least in part, to result
from infections at an older age by a typical BSE agent, rather than neonatal
infections with new "strains" of BSE. Neither alternative has yet been
investigated.

http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm


mad cow in TEXAS they rendered without any test at ALL.


http://www.fda.gov/bbs/topics/news/2004/NEW01061.html


REMINDER, CATTLE ON FEED IN TEXAS


IN TEXAS, cattle on feed for decades, fda says 5.5 grams ruminant protein,
if tainted with TSE, is not enough to kill a cow. actually, it's enough to
kill 100+ cows ;-)

http://www.fda.gov/bbs/topics/NEWS/2001/NEW00752.html


and add these in ;


UPI previously reported that from 2001 to 2003 the USDA collected the wrong
part of the brain in more than 200 cows that were being screened as part of
its BSE surveillance program.

The USDA documents also indicate the agency never was able to identify or
test 52 cows that came into the United States in 2001 along with the
Washington cow that tested positive in 2003. Of these, 11 were considered to
be "high risk" because they were born within a year and on the same premises
as the infected cow.

These cows may have gone into the food supply and been consumed by people.
The concern is humans can contract a fatal brain disease from eating beef
products contaminated with the mad cow pathogen.


http://www.terradaily.com/upi/2005/WWN-UPI-20050721-17422400-bc-us-madcow.html


NOT to forget what Paul Brown TSE expert at CDC said ;


THE USDA JUNE 2004 ENHANCED BSE SURVEILLANCE PROGRAM WAS TERRIBLY FLAWED ;


CDC DR. PAUL BROWN TSE EXPERT COMMENTS 2006


The U.S. Department of Agriculture was quick to assure the public earlier
this week that the third case of mad cow disease did not pose a risk to
them, but what federal officials have not acknowledged is that this latest
case indicates the deadly disease has been circulating in U.S. herds for at
least a decade.

The second case, which was detected last year in a Texas cow and which USDA
officials were reluctant to verify, was approximately 12 years old.

These two cases (the latest was detected in an Alabama cow) present a
picture of the disease having been here for 10 years or so, since it is
thought that cows usually contract the disease from contaminated feed they
consume as calves. The concern is that humans can contract a fatal,
incurable, brain-wasting illness from consuming beef products contaminated
with the mad cow pathogen.

"The fact the Texas cow showed up fairly clearly implied the existence of
other undetected cases," Dr. Paul Brown, former medical director of the
National Institutes of Health's Laboratory for Central Nervous System
Studies and an expert on mad cow-like diseases, told United Press
International. "The question was, 'How many?' and we still can't answer
that."

Brown, who is preparing a scientific paper based on the latest two mad cow
cases to estimate the maximum number of infected cows that occurred in the
United States, said he has "absolutely no confidence in USDA tests before
one year ago" because of the agency's reluctance to retest the Texas cow
that initially tested positive.

USDA officials finally retested the cow and confirmed it was infected seven
months later, but only at the insistence of the agency's inspector general.

"Everything they did on the Texas cow makes everything USDA did before 2005
suspect," Brown said. ...snip...end


http://www.upi.com/ConsumerHealthDaily/view.php?StoryID=20060315-055557-1284r


CDC - Bovine Spongiform Encephalopathy and Variant Creutzfeldt ...
Dr. Paul Brown is Senior Research Scientist in the Laboratory of Central
Nervous System ... Address for correspondence: Paul Brown, Building 36, Room
4A-05, ...


http://www.cdc.gov/ncidod/eid/vol7no1/brown.htm


PAUL BROWN COMMENT TO ME ON THIS ISSUE

Tuesday, September 12, 2006 11:10 AM


"Actually, Terry, I have been critical of the USDA handling of the mad cow
issue for some years,
and with Linda Detwiler and others sent lengthy detailed critiques and
recommendations to both the
USDA and the Canadian Food Agency."


OR, what the Honorable Phyllis Fong of the OIG found ;


Audit Report

Animal and Plant Health Inspection Service

Bovine Spongiform Encephalopathy (BSE) Surveillance Program – Phase II

and

Food Safety and Inspection Service

Controls Over BSE Sampling, Specified Risk Materials, and Advanced Meat
Recovery Products - Phase III

Report No. 50601-10-KC January 2006

Finding 2 Inherent Challenges in Identifying and Testing High-Risk Cattle
Still Remain


http://www.usda.gov/oig/webdocs/50601-10-KC.pdf



USA BSE OIG 2006


http://www.usda.gov/oig/webdocs/50601-10-KC.pdf


TEXAS ATYPICAL BSE IN BOVINE FINALLY CONFIRMED ALMOST ONE YEAR AFTER FACT BY
OIG DOING END AROUND JOHANNS ET AL, or that cow would have never been
confirmed. 48 HOUR turnaround for confirmation of suspect BSE case by BSE
guidelines flaunted until OIG finally stepped in. ...tss


USDA ANNOUNCES BSE TEST RESULTS AND NEW BSE CONFIRMATORY TESTING PROTOCOL

WASHINGTON, June 24, 2005 -- Agriculture Secretary Mike Johanns today
announced that the U.S. Department of Agriculture has received final test
results from The Veterinary Laboratories Agency in Weybridge, England,
confirming that a sample from an animal that was blocked from the food
supply in November 2004 has tested positive for bovine spongiform
encephalopathy (BSE). Johanns also directed USDA scientists to work with
international experts to thoughtfully develop a new protocol that includes
performing dual confirmatory tests in the event of another "inconclusive"
BSE screening test.

snip...

The animal was selected for testing because, as a non-ambulatory animal, it
was considered to be at higher risk for BSE. An initial screening test on
the animal in November 2004 was inconclusive, triggering USDA to conduct the
internationally accepted confirmatory IHC tests. Those test results were
negative. Earlier this month, USDA's Office of the Inspector General
recommended further testing of the seven-month-old sample using another
internationally recognized confirmatory test, the Western blot. Unlike the
IHC, the Western blot was reactive, prompting USDA to send samples from the
animal to the Weybridge laboratory for further analysis.



http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB/.cmd/ad/.ar/sa.retrievecontent/.c/6_2_1UH/.ce/7_2_5JM/.p/5_2_4TQ/.d/1/_th/J_2_9D/_s.7_0_A/7_0_1OB?PC_7_2_5JM_contentid=2005/06/0232.xml&PC_7_2_5JM_navtype=RT&PC_7_2_5JM_parentnav=LATEST_RELEASES&PC_7_2_5JM_navid=NEWS_RELEASE#7_2_5JM


http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB?contentidonly=true&contentid=2006/03/0084.xml


http://www.usda.gov/wps/portal/usdahome?contentidonly=true&contentid=2005/08/0339.xml


The investigation identified one feed which contained an animal protein
source that could not be identified. The investigation also found one feed
mill that supplied feed to the farm that had used ruminant MBM in feed
formulations for non-ruminant species after the BSE/Ruminant Feed rule went
into effect, which is permitted under the rule, and that several feed mills
had used ruminant MBM in feeds prior to the feed ban. Although the
investigation did not identify a specific feed source as the likely cause of
this animal’s infection, it is probable that the most likely route of
exposure for this animal was consumption of an animal feed containing
mammalian protein prior to the implementation of the BSE/Ruminant Feed rule
in 1997.


http://www.fda.gov/cvm/texasfeedrpt.htm


Texas BSE Investigation
Final Epidemiology Report
August 2005


snip...


Progeny
The owner did keep some replacement heifers and, although he was relatively
sure that he
had not kept any offspring from the yellow cow because of her excitable
demeanor, DNA
analysis of the herd revealed several animals in the herd that may have been
older offspring
of the index cow. While the owner sold 12 calves at the sale with the index
cow on
11/11/04, her last calf was not in that group. According to the owner, the
index cow’s last
calf was born either in Fall 2003 or Spring 2004, weaned early, and sold
through the
livestock market some time between February and October 2004. The calf prior
to that
would have been born either in Fall 2002 or Spring 2003 and was sold at the
livestock
market sometime between January and December 2003.
Birth Cohort
The owner of Farm A kept very few herd records; this made finding
documentation on this
cow’s birth cohort difficult. The birth cohort, by definition, included all
cattle born on the
positive animal’s birth premises within 1 year, before or after, the
positive animal’s date of
birth. The index cow was approximately 12 years of age in November 2004, but
there was
no exact birth date in the herd records. A potential age range of 11 to 13
years was used to
sufficiently cover the animal’s most likely age. Using this range, all
cattle born on the
index premises between 1990 and 1995 were considered part of the birth
cohort.


snip...


Trace Herd 1
The owner of Trace Herd 1 was identified as having received one of the adult
COI from the
index herd. Trace Herd 1 contained 909 head of cattle in multiple pastures
and was placed
under hold order on 7/21/05. Upon completion of herd inventory, the animal
of interest
was not found within the herd. A GDB search of all recorded herd tests
conducted on
Trace Herd 1 and all market sales by the owner failed to locate the
identification tag of the
animal of interest and she was subsequently classified as untraceable. The
hold order on
Trace Herd 1 was released on 8/8/05.
Trace Herd 2
Trace Herd 2 was identified as having received one of the adult COI from the
index herd.
Trace Herd 2 contained 19 head of cattle on one pasture and was placed under
hold order
on 7/25/05. The owner of Trace Herd 2 identified the animal of interest by
her eartag while
he was feeding his cattle out of a bucket and individually penned her for
inspection by field
personnel. While the cow was identified as one of the animals that had left
the index farm,
her age by dentition was estimated to be only 5 years old, which was too
young to have
placed her as part of the birth or feed cohort of the index animal. She was
classified as
found alive but determined not to be one of the COI; the hold order on Trace
Herd 2 was
released on 7/26/05.
11
Trace Herd 3
The owner of Trace Herd 3 was identified as possibly having received an
animal of
interest. The herd was placed under hold order on 7/27/05. The herd
inventory was
conducted on 7/28/05. The animal of interest was not present within the
herd, and the hold
order was released on 7/28/05. The person who thought he sold the animal to
the owner of
Trace Herd 3 had no records and could not remember who else he might have
sold the cow
to. Additionally, a search of GDB for all cattle sold through the markets by
that individual
did not result in a match to the animal of interest. The animal of interest
traced to this herd
was classified as untraceable because all leads were exhausted.
Trace Herd 4
The owner of Trace Herd 4 was identified as having received one of the COI
through an
order buyer. Trace Herd 4 was placed under hold order on 7/29/05. A complete
herd
inventory was conducted on 8/22/05 and 8/23/05. There were 233 head of
cattle that were
examined individually by both State and Federal personnel for all man-made
identification
and brands. The animal of interest was not present within the herd. Several
animals were
reported to have died in the herd sometime after they arrived on the
premises in April 2005.
A final search of GDB records yielded no further results on the eartag of
interest at either
subsequent market sale or slaughter. With all leads having been exhausted,
this animal of
interest has been classified as untraceable. The hold order on Trace Herd 4
was released on
8/23/05.
Trace Herd 5
The owner of Trace Herd 5 was identified as having received two COI and was
placed
under hold order on 8/1/05. Trace Herd 5 is made up of 67 head of cattle in
multiple
pastures. During the course of the herd inventory, the owner located records
that indicated
that one of the COI, a known birth cohort, had been sold to Trace Herd 8
where she was
subsequently found alive. Upon completion of the herd inventory, the other
animal of
interest was not found within the herd. A GDB search of all recorded herd
tests conducted
on Trace Herd 5 and all market sales by the owner failed to locate the
identification tag of
the animal of interest and she was subsequently classified as untraceable
due to all leads
having been exhausted. The hold order on Trace Herd 5 was released on
8/8/05.
Trace Herd 6
The owner of Trace Herd 6 was identified as possibly having received an
animal of interest
and was placed under hold order on 8/1/05. This herd is made up of 58 head
of cattle on
two pastures. A herd inventory was conducted and the animal of interest was
not present
within the herd. The owner of Trace Herd 6 had very limited records and was
unable to
provide further information on where the cow might have gone after he
purchased her from
the livestock market. A search of GDB for all cattle sold through the
markets by that
individual did not result in a match to the animal of interest.
Additionally, many of the
animals presented for sale by the owner of the herd had been re-tagged at
the market
effectually losing the traceability of the history of that animal prior to
re-tagging. The
animal of interest traced to this herd was classified as untraceable due to
all leads having
been exhausted. The hold order on Trace Herd 6 was released on 8/3/05.
12
Trace Herd 7
The owner of Trace Herd 7 was identified as having received an animal of
interest and was
placed under hold order on 8/1/05. Trace Herd 7 contains 487 head of cattle
on multiple
pastures in multiple parts of the State, including a unit kept on an island.
The island
location is a particularly rough place to keep cattle and the owner claimed
to have lost 22
head on the island in 2004 due to liver flukes. Upon completion of the herd
inventory, the
animal of interest was not found present within Trace Herd 7. A GDB search
of all
recorded herd tests conducted on Trace Herd 7 and all market sales by the
owner failed to
locate the identification tag of the animal of interest. The cow was
subsequently classified
as untraceable. It is quite possible though that she may have died within
the herd,
especially if she belonged to the island unit. The hold order on Trace Herd
7 was released
on 8/8/05.
Trace Herd 8
Trace Herd 8 received an animal of interest, which happened to be a known
birth cohort of
the index cow, from Trace Herd 5. Trace Herd 8 consists of 146 head of
cattle that were
placed under hold order on 8/4/05. A herd inventory was conducted, the birth
cohort was
found alive in the herd, and she was purchased and euthanized. The hold
order on Trace
Herd 8 was released on 8/4/05. The cow was sampled on 8/5/05 and BSE tested
by ELISA
at NVSL. Results were negative (as reported on 8/6/05); carcass disposal was
completed
by alkaline digestion.


snip...end


http://www.aphis.usda.gov/lpa/issues/bse/epi-updates/bse_final_epidemiology_report.pdf


ALABAMA ATYPICAL BSE IN BOVINE


Jim Rogers (202) 690-4755
USDA Press Office (202) 720-4723

Statement by USDA Chief Veterinary Officer John Clifford (DVM) Regarding
Positive BSE Test Results
March 13, 2006

“We received a positive result on a Western blot confirmatory test conducted
at the USDA laboratories in Ames, Iowa, on samples from an animal that had
tested “inconclusive” on a rapid screening test performed on Friday, March
10.

“The samples were taken from a non-ambulatory animal on a farm in Alabama. A
local private veterinarian euthanized and sampled the animal and sent the
samples for further testing, which was conducted at one of our contract
diagnostic laboratories at the University of Georgia. The animal was buried
on the farm and it did not enter the animal or human food chains.


snip...

http://www.aphis.usda.gov/newsroom/content/2006/03/bsestatement3-13-06_vs.shtml

http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB?contentidonly=true&contentid=2006/03/0084.xml


Alabama BSE Investigation
Final Epidemiology Report
May 2, 2006


snip...


Summary:
Despite a thorough investigation of two farms that were known to contain the
index cow,
and 35 other farms that might have supplied the index cow to the farms where
the index
case was known to have resided, the investigators were unable to locate the
herd of
origin. The index case did not have unique or permanent identification,
plus, the size and
color of the cow being traced is very common in the Southern United States.
Due to the
unremarkable appearance of solid red cows, it is not easy for owners to
remember
individual animals. In the Southern United States, it is common business
practice to buy
breeding age cows and keep them for several years while they produce calves.
Most
calves produced are sold the year they are born, whereas breeding cows are
sold when
there is a lapse in breeding, which can occur multiple times in cows’ lives.
For all of
these reasons, USDA was unable to locate the herd of origin.


snip...


http://www.aphis.usda.gov/newsroom/hot_issues/bse/content/printable_version/EPI_Final.pdf


AFTER this, it was just to much risk for USDA/APHIS/FSIS et al to do anymore
extensive TSE testing of any sort in the USA bovine. so they reduced BSE
testing drastically ;


USDA ANNOUNCES NEW BSE SURVEILLANCE PROGRAM

WASHINGTON, July 20, 2006-Agriculture Secretary Mike Johanns announced today
that the U.S. Department of Agriculture will soon begin transitioning to an
ongoing Bovine Spongiform Encephalopathy (BSE) surveillance program that
corresponds to the extremely low prevalence of the disease in the U.S.

"It's time that our surveillance efforts reflect what we now know is a very,
very low level of BSE in the United States," said Johanns. "This ongoing
surveillance program will maintain our ability to detect BSE, provide
assurance that our interlocking safeguards are successfully preventing BSE,
while continuing to exceed science-based international guidelines."

The ongoing BSE surveillance program will sample approximately 40,000
animals each year. Under the program, USDA will continue to collect samples
from a variety of sites and from the cattle populations where the disease is
most likely to be detected, similar to the enhanced surveillance program
procedures.


snip...


http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB?contentidonly=true&contentid=2006/07/0255.xml

http://www.usda.gov/wps/portal/!ut/p/_s.7_0_A/7_0_1OB/.cmd/ad/.ar/sa.retrievecontent/.c/6_2_1UH/.ce/7_2_5JM/.p/5_2_4TQ/.d/1/_th/J_2_9D/_s.7_0_A/7_0_1OB?
PC_7_2_5JM_contentid=2006/07/0256.xml&PC_7_2_5JM_parentnav=TRANSCRIPTS_SPEEC
HES&PC_7_2_5JM_navid=TR


Title: Bovine Spongiform Encephalopathy: what is "Atypical BSE" and can we
detect it?


Author

Richt, Juergen


Submitted to: Animal Health Research Reviews
Publication Type: Abstract
Publication Acceptance Date: October 17, 2006
Publication Date: N/A


Technical Abstract: Transmissible spongiform encephalopathy (TSE) agents
induce fatal neurodegenerative diseases in humans and in some other
mammalian species. Human TSEs include Creutzfeldt¿Jakob disease (CJD),
Gerstmann-Sträussler-Scheinker (GSS) syndrome, Kuru and Fatal Familial
Insomnia (FFI). In animals, several distinct TSE diseases are recognized:
scrapie in sheep and goats, transmissible mink encephalopathy (TME) in mink,
chronic wasting disease (CWD) in cervids, and bovine spongiform
encephalopathy (BSE) in cattle. BSE was first detected in 1986 in the United
Kingdom and is the most likely cause of variant CJD in humans. BSE in cattle
is a neurological disease with a characteristic molecular pattern of the
protease-resistant prion protein, PrP**res. This BSE 'signature' has also
been identified in BSE-induced TSEs of both domestic cats and exotic
ruminant species. Since 2004, some cases of prion diseases in cattle have
been described which show unusual or atypical features as assessed by the
molecular characterization of PrP**res and/or histopathology, when compared
to the unique features of previously described BSE. These atypical BSE cases
have been characterized by Western blot and have been referred to as H-
(i.e., high molecular weight) or L-type (i.e., low molecular weight type).
These atypical BSE cases have been found mainly in cattle older than 8
years. In the U.S., three cases of BSE have been diagnosed so far. Case 1
represented a typical BSE isolate, identified in an animal imported from
Canada. Cases 2 and 3 were identified in animals raised in the U.S. and
revealed an unusual molecular PrP**res pattern, consistent with atypical BSE
cases described as H-type in Europe. It should be noted that the Western
Blot method applied for BSE confirmatory tests in the U.S. has been able to
detect both H-type and L-type BSE cases when using known positive European
samples.


http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=202722


Title: Identification and Characterization of U.S. Bse Cases


Authors

Hall, S - USDA, APHIS, VS, NVSL
Richt, Juergen
Davis, A - USDA, APHIS, VS, NVSL
Kluge, J - USDA, APHIS, VS, NVSL
Simmons, M - VETERINARY LABS AGENCY,UK
Stack, M - VETERINARY LABS AGENCY,UK
Spencer, Y - VETERINARY LABS AGENCY,UK


Submitted to: Meeting Abstract
Publication Type: Abstract
Publication Acceptance Date: April 25, 2006
Publication Date: May 28, 2006
Citation: Hall, S.M., Richt, J., Davis, A., Kluge, J., Simmons, M., Stack,
M., Spencer, Y. 2006. Identification and characterization of U.S. BSE cases
[abstract]. Prion Diseases of Domestic Livestock. p. 25.

Technical Abstract: Bovine Spongiform Encephalopathy (BSE) surveillance has
been ongoing in the USA since the early 1990¿s and initial testing was done
at the USDA, National Veterinary Services Laboratories (NVSL) utilizing
routine histopathology exclusively. In 1995, the immunohistochemistry (IHC)
test was incorporated into surveillance testing in addition to routine
histopathology. By 1999 virtually all BSE screening was performed by IHC and
by 2001 the NVSL had switched to an automated IHC procedure. In 2002 and
2003 the NVSL tested about 20,000 high risk animals each year by IHC. In
December, 2003 an animal was identified by IHC as positive for BSE (Case 1);
this animal was determined to be imported from Canada. After this animal was
identified, in June 2004 the USDA began its enhanced surveillance program as
a shared effort between selected state veterinary diagnostic laboratories
and NVSL, as part of the National Animal Health Laboratory Network. The plan
called for testing as many targeted high risk animals as possible in a 12-18
month period. From June 1, 2004 through March 21, 2006, over 650,000 animals
have been tested (Bio-Rad ELISA). Of those tested, two animals (Cases 2 and
3) have been identified as positive for BSE. While all three cases were
strongly positive by Bio-Rad ELISA, Cases 2 and 3 have common features which
are distinct from Case 1. Definitive spongiform changes in the obex, strong
immunohistochemical reactions, and Western blot patterns similar to European
BSE cases were observed in Case 1. In contrast, Cases 2 and 3 did not
contain definitive histological lesions of BSE and the IHC staining was less
intense than Case 1. In addition, Cases 2 (approximately 12 years) and Case
3 (approximately ten years) were older animals while Case 1 was 6.5 years
old. Western blot analysis, PrP**Sc from Case 1 showed molecular features
similar to typical BSE isolates, whereas PrP**Sc from Cases 2 and 3 revealed
an unusual molecular PrP**Sc pattern: molecular mass of the unglycosylated
and monoglycosylated isoform was higher than that of typical BSE isolates.
Case 1 contained more PrP**Sc per brain tissue mg equivalent compared with
Cases 2 and 3 using antibody 6H4. In Western Blot analysis, Case 2 and Case
3 were strongly positive with antibody P4, while Case 1 was negative or
weakly positive with P4.


http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=194279


3:00 Afternoon Refreshment Break, Poster and Exhibit Viewing in the Exhibit
Hall


3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western
Reserve
University

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years. ***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


SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...


http://www.cjdsurveillance.com/resources-casereport.html


There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
collection.

He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf


JOURNAL OF NEUROLOGY

MARCH 26, 2003


RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States


Email Terry S. Singeltary:


[email protected]


I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported 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. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?


http://www.neurology.org/cgi/eletters/60/2/176#535


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

Singeltary, Sr et al. JAMA.2001; 285: 733-734.

http://jama.ama-assn.org/


BRITISH MEDICAL JOURNAL


BMJ


http://www.bmj.com/cgi/eletters/319/7220/1312/b#EL2


BMJ


http://www.bmj.com/cgi/eletters/320/7226/8/b#EL1


THE rest is history, GWs and the OIE BSE MRR policy that will spread these
different strains of TSE around the globe,
all for a buck, cattle futures and commodities rule, to hell with human
health. OF which due to the long incubation period,
ramifications of this blunder will not be felt for a decade or more later
down the road. ...TSS



----- Original Message -----
From: "Terry S. Singeltary Sr." <[email protected]>
To: <[email protected]>
Sent: Friday, March 09, 2007 1:34 PM
Subject: UK's first case of H-type BSE confirmed by DEFRA


snip...end...TSS
 

flounder

Well-known member
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...

ITEM 9 – ANY OTHER BUSINESS

54. The Chair noted that a paper had been provided to update the

committee on an ongoing study on the susceptibility of red deer to

BSE. To date, BSE had transmitted to four ic challenged deer,

however no orally challenged deer had succumbed to the disease.


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



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