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From: TSS ()
Subject: Chronic Wasting Disease Stakeholder Advisory Group August 18th meeting
Date: September 11, 2007 at 11:39 am PST

Chronic Wasting Disease Stakeholder Advisory Group
Group Member Biographies and Contact Information [PDF, 41KB]

Advisory Group Handbook [PDF, 957KB]

Decision Making Process [PDF, 50KB]

Documents from the group’s first meeting, held July 21st at the DNR’s South
Central Region Headquarters in Fitchburg, WI:

7-21 Meeting Agenda [PDF, 22KB]


http://www.dnr.state.wi.us/org/land/wildlife/whealth/issues/cwd/doc/Agenda07_21_07.pdf


7-21 Meeting Minutes [PDF, 47KB]


http://www.dnr.state.wi.us/org/land/wildlife/whealth/issues/cwd/doc/Notes07_21_07.pdf

The CWD Stakeholder Advisory Group met on August 18 at the Lowell Center Inn
and Conference Center in Madison. At this meeting, several presenters from
state and federal agencies and the University of Wisconsin summarized the
state of scientific knowledge about chronic wasting disease, covering topics
from human health risk and risks to other species, to the role of
environmental contamination and the social constraints of managing CWD in
Wisconsin.

8-18 Meeting Agenda [PDF, 13KB]


http://www.dnr.state.wi.us/org/land/wildlife/whealth/issues/cwd/doc/Agenda08_18_07.pdf

8-18 Meeting Minutes [PDF, 53KB]


http://www.dnr.state.wi.us/org/land/wildlife/whealth/issues/cwd/doc/Notes08_18_07.pdf

The following videos were recorded at the August 18th meeting. They feature
the presentations described above and questions asked by members of the CWD
Stakeholder Advisory Group.


(paste full javascript:....... URL in address window and go, video should
play. ...TSS)


CWD: Why Care? [VIDEO Length 20:10]


javascript:mwi('http://media2.wi.gov/DNR/Viewer?peid=4aa21a52-081a-4428-bc51-d7bfd3d8bce1',660,435,0,0)

Human Health Risks [VIDEO Length 47:31]


javascript:mwi('http://media2.wi.gov/DNR/Viewer?peid=563afb70-13b9-45ab-a7ee-3cf4a30e8c09',800,500,0,0)

CWD Risks to Other Species [VIDEO Length 62:43]


javascript:mwi('http://media2.wi.gov/DNR/Viewer?peid=b3365670-6b00-4644-99be-7b1584b22a8c',800,500,0,0)

The Economic Impacts of CWD [VIDEO Length 41:10]


javascript:mwi('http://media2.wi.gov/DNR/Viewer?peid=a1794185-8017-4c0d-a916-4048dd1a748a',800,500,0,0)

CWD Disease Ecology, Part 1 [VIDEO Length 50:48]


javascript:mwi('http://media2.wi.gov/DNR/Viewer?peid=1a711732-773d-47cd-a649-47325167a145',800,500,0,0)

CWD Disease Ecology, Part 2 [VIDEO Length 42:47]


javascript:mwi('http://media2.wi.gov/DNR/Viewer?peid=93d0e2e4-0be6-48d5-8476-2d2df38e024b',800,500,0,0)

What is the Role of Environmental Contamination [VIDEO Length 50:21]


javascript:mwi('http://media2.wi.gov/DNR/Viewer?peid=8f8473c0-8145-4f83-9562-83f019f54bdb',800,500,0,0)

The Role of PrP Genetics in CWD [VIDEO Length 29:20]


javascript:mwi('http://media2.wi.gov/DNR/Viewer?peid=608b7155-afa3-4714-88ae-69c8d60f8f22',800,500,0,0)


The next meeting of the Chronic Wasting Disease Stakeholder Advisory group
will be September 22nd from 9a.m. - 4p.m. in the Gathering Waters and
Glacier's Edge conference rooms at the DNR South Central Region
Headquarters, 3911 Fish Hatchery Road, Fitchburg. At this meeting, the group
will hear presentations from several researchers in the morning. During the
afternoon, the group will begin to discuss the potential impacts of CWD in
Wisconsin. For more information, contact Alan Crossley, DNR CWD project
manager, at (608) 266-5463 or Alan.Crossley@Wisconsin.gov.

Future meetings for the Stakeholder Advisory Group will be held:

October 20, 2007;
November 10, 2007;
December 15, 2007; and
January 12, 2008.
Meeting locations are yet to be determined.

To follow the progress of the Stakeholder Advisory Group and learn about
opportunities for you to participate, please return to this webpage for
updates. Or, for more information, call Alan Crossley at (608)266-5463.

Contact Information
For answers to questions relating to CWD in Wisconsin,
call the DNR operated toll free CWD information line 1-877-WISC CWD or email
Wildlife Health.

http://www.dnr.state.wi.us/org/land/wildlife/whealth/issues/CWD/advisory.htm

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

snip.....end

cwd human health risk Jim Kazmierczak presentation

i only listened to this so far. thought i might comment.

im in a hurry today, sorry. to the point;


HE STATES ''TO DATE NO EVIDENCE THAT SHOWS CWD TRANSMITS TO HUMANS''...

I DISAGREE, there is more evidence showing it could, than it cannot, and
there has never been
human transmission studies. but look at primates and other species i.e.
cattle and sheep for example.
in the lab, both have contracted cwd.


oh my God, the old spontaneous CJD, all 90%, just happens without cause. you
got to be kidding me. this human health aspect of this CWD presentation
sounds like it was rubber stamped by the USDA et al. and speaking of that
test tube conversion of prion, if i remember correctly, exactly what that
study showed was that the conversion of the CWD to human is just as
effeciant or as non-effeciant, depending whom you have watched die from it,
but that study showed that BSE, and even SCRAPIE was just as limited or not
as CWD. let's take a look, this gentleman did not show you this. he claimed
he had the study, he should have taken the time to explain this. he says it
does not happen in the real world, i.e. scrapie, i beg to differ on that one
too.


CWD to CJD in humans (why not?), as easy as BSE/Scrapie;

The EMBO Journal, Vol. 19, No. 17 pp. 4425-4430, 2000
© European Molecular Biology Organization

Evidence of a molecular barrier limiting
susceptibility of humans, cattle and sheep to
chronic wasting disease

G.J. Raymond1, A. Bossers2, L.D. Raymond1, K.I. O?Rourke3,
L.E. McHolland4, P.K. Bryant III4, M.W. Miller5, E.S. Williams6, M.
Smits2
and B. Caughey1,7

1NIAID/NIH Rocky Mountain Laboratories, Hamilton, MT 59840,
3USDA/ARS/ADRU, Pullman, WA 99164-7030, 4USDA/ARS/ABADRL,
Laramie, WY 82071, 5Colorado Division of Wildlife, Wildlife Research
Center, Fort Collins, CO 80526-2097, 6Department of Veterinary Sciences,
University of Wyoming, Laramie, WY 82070, USA and 2ID-Lelystad,
Institute for Animal Science and Health, Lelystad, The Netherlands
7Corresponding author e-mail: bcaughey@nih.gov Received June 7, 2000;
revised July 3, 2000; accepted July 5, 2000.

Abstract

Chronic wasting disease (CWD) is a transmissible
spongiform encephalopathy (TSE) of deer and elk,
and little is known about its transmissibility to other
species. An important factor controlling
interspecies TSE susceptibility is prion protein (PrP)
homology between the source and recipient
species/genotypes. Furthermore, the efficiency with which
the protease-resistant PrP (PrP-res) of one
species induces the in vitro conversion of the normal PrP
(PrP-sen) of another species to the
protease-resistant state correlates with the cross-species
transmissibility of TSE agents. Here we
show that the CWD-associated PrP-res (PrPCWD) of cervids
readily induces the conversion of recombinant cervid PrP-sen
molecules to the protease-resistant state in accordance
with the known transmissibility of CWD between cervids. In contrast,
PrPCWD-induced conversions of human and bovine PrP-sen were
much less efficient, and conversion of ovine PrP-sen was
intermediate. These results demonstrate a barrier at the
molecular level that should limit the susceptibility of these non-cervid
species to CWD.

snip...

Clearly, it is premature to draw firm conclusions about CWD
passing naturally into humans, cattle and sheep, but the present
results suggest that CWD transmissions to humans would be as
limited by PrP incompatibility as transmissions of BSE or sheep
scrapie to humans. Although there is no evidence that sheep
scrapie has affected humans, it is likely that BSE has caused variant
CJD in 74 people (definite and probable variant CJD cases to
date according to the UK CJD Surveillance Unit). Given the
presumably large number of people exposed to BSE infectivity,
the susceptibility of humans may still be very low compared with
cattle, which would be consistent with the relatively inefficient
conversion of human PrP-sen by PrPBSE. Nonetheless, since
humans have apparently been infected by BSE, it would seem prudent
to take reasonable measures to limit exposure of humans
(as well as sheep and cattle) to CWD infectivity as has been
recommended for other animal TSEs.

snip...

http://www.emboj.org/current.shtml


disease specific my hind end...............tss


Scrapie to Humans?

http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=3915057&dopt=Abstract


please note, scrapie transmit to primate by there NON-FORCED ORAL
CONSUMPTION OF TAINTED MATERIAL;

1: J Infect Dis 1980 Aug;142(2):205-8

Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to
nonhuman primates.

Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.

Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep
and goats were transmitted to squirrel monkeys (Saimiri sciureus) that were
exposed to the infectious agents only by their nonforced consumption of
known infectious tissues. The asymptomatic incubation period in the one
monkey exposed to the virus of kuru was 36 months; that in the two monkeys
exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months,
respectively; and that in the two monkeys exposed to the virus of scrapie
was 25 and 32 months, respectively. Careful physical examination of the
buccal cavities of all of the monkeys failed to reveal signs or oral
lesions. One additional monkey similarly exposed to kuru has remained
asymptomatic during the 39 months that it has been under observation.

PMID: 6997404
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract


12/10/76
AGRICULTURAL RESEARCH COUNCIL
REPORT OF THE ADVISORY COMMITTE ON SCRAPIE
Office Note
CHAIRMAN: PROFESSOR PETER WILDY

snip...

A The Present Position with respect to Scrapie
A] The Problem

Scrapie is a natural disease of sheep and goats. It is a slow
and inexorably progressive degenerative disorder of the nervous system
and it ia fatal. It is enzootic in the United Kingdom but not in all
countries.

The field problem has been reviewed by a MAFF working group
(ARC 35/77). It is difficult to assess the incidence in Britain for
a variety of reasons but the disease causes serious financial loss;
it is estimated that it cost Swaledale breeders alone $l.7 M during
the five years 1971-1975. A further inestimable loss arises from the
closure of certain export markets, in particular those of the United
States, to British sheep.

It is clear that scrapie in sheep is important commercially and
for that reason alone effective measures to control it should be
devised as quickly as possible.

Recently the question has again been brought up as to whether
scrapie is transmissible to man. This has followed reports that the
disease has been transmitted to primates. One particularly lurid
speculation (Gajdusek 1977) conjectures that the agents of scrapie,
kuru, Creutzfeldt-Jakob disease and transmissible encephalopathy of
mink are varieties of a single "virus". The U.S. Department of
Agriculture concluded that it could "no longer justify or permit
scrapie-blood line and scrapie-exposed sheep and goats to be processed
for human or animal food at slaughter or rendering plants" (ARC 84/77)"
The problem is emphasised by the finding that some strains of scrapie
produce lesions identical to the once which characterise the human
dementias"

Whether true or not. the hypothesis that these agents might be
transmissible to man raises two considerations. First, the safety
of laboratory personnel requires prompt attention. Second, action
such as the "scorched meat" policy of USDA makes the solution of the
acrapie problem urgent if the sheep industry is not to suffer
grievously.

snip...

76/10.12/4.6

http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf


WHAT ABOUT ATYPICAL SCRAPIE I.E. NOR-98, even Prusiner is worried about
human health implications ;


Infected and Source Flocks

As of June 30, 2007, there were .....

snip...

One field case and one validation case were consistent with Nor-98 scrapie.

http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps


IN the February 2007 Scrapie report it only mentions ;

''One case was consistent with Nor98 scrapie.''

http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/


(please note flocks of origin were in WY, CO, AND CA. PERSONAL COMMUNCATIONS
USDA, APHIS, VS ET AL. ...TSS)

NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=14553


An evaluation of scrapie surveillance in the United States


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=3427

FOIA REQUEST FOR ATYPICAL TSE INFORMATION ON VERMONT SHEEP


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=10451

NOW, this gentleman did not speak of the atypical BSE i.e. BASE, he seems to
oblivious to this as well, and transmission studies show it is more virulent
to humans than the UK BSE strain..........


Subject: Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease
creates a new prion strain

Date: August 25, 2007 at 12:42 pm PST

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=21267

Subject: MAD COW BASE H-TYPE AND L-TYPE

Date: August 23, 2007 at 11:30 am PST


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=19779


yes butts???

indeed there are many yes butts, more so than no butts. .......again, this
science is ignored.

this presentation seemed to be aimed at playing DOWN the very real potential
transmission of CWD to humans.
and there is data even showing that probably more than one strain of CWD
exists. and why not? there are over 20 strains of typical scrapie, with
atypical scrapie cases showing up. oh, scrapie has been around for 200 years
or so, and CJD has not become reportable in most states until just recently
for many, and still there are states where cjd is not reportable. so show me
the transmission studies proving that scrapie does not transmit to man.
there are none. however, just the opposite exist, that it is just as likely
that CWD does transmit to humans. and as far as gummer goes, bush and the
canadian prime minister gave the same false hope. all in all, this
presentation was aimed, in my opinion, at calming fears of the very likely
transmission of CWD to humans, however rare it might be, but the threat is
real. and as far as eating, i would be more fearful of gutting a deer with
CWD as opposed to the oral route. a simple nick on the finger gutting a deer
is nothing more than a crude inoculation. the most efficient mode of
transmission. and this guy is oblivious to the recent data on Alzheimer's
and CJD. he is simply living in the UKBSEnvCJD days, some 3 decades ago. and
i heard him state the old one to two in a million myth. he did not tell you
that at over 50, the statistics jump drastically to one in 9,000. the media
has repeated the 1 in a million ad nauseum. that's a very deceptive figure
for any slow onset disease. then throw in the cases they miss and it is 5
per 9000 or 1 per 1800 which to my way of thinking is a pretty substantial
epidemic for a disease no one had ever heard of in 1920. also, atypical CJD
has been on the rise in the USA, he did not tell you this either ;

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

this gentleman states that there is ''NO CLUSTERING OF SPONTANEOUS/SPORADIC CJD''.

i beg to differ ;


Subject: cjd cluster IDAHO, another 'fluke', another mad cow cjd cover up in
the USA
Date: March 11, 2006 at 8:30 pm PST

In Reply to: MAD COW USA JUNK SCIENCE PR COMING OUT BEFORE RECENT SUSPECT
BSE CASE EVEN CONFIRMED i.e. preparing for the storm posted by TSS on March
11, 2006 at 7:31 pm:


Subject: another cjd cluster, another 'fluke', another mad cow cjd cover up
in the USA
Date: August 16, 2005 at 6:23 am PST


Brain-disease deaths investigated
Idaho seeks link in 5 rare cases
By Adam Tanner, Reuters | August 16, 2005

TWIN FALLS, Idaho -- In late May, Marjorie Skinner played golf well enough
to place fourth in a Memorial Day weekend tournament. Yet within weeks, the
previously vibrant retiree started losing her ability to speak.


By the time her family buried her Friday, she was the fifth suspected victim
in the same sparsely populated area of Idaho of Creutzfeldt-Jakob disease, a
rare brain-wasting disease that typically afflicts only one in a million
people.

As word of the latest death spread yesterday, local and federal health
specialists sifted through clues about an illness different from variant
Creutzfeldt-Jakob disease, the human form of mad cow disease.

''Five [cases] in one valley is pretty serious," said Sue Skinner,
Marjorie's daughter-in-law. ''It's a grave concern in our family."

The mystery has deepened in recent weeks. At the end of May, another elderly
woman died of the incurable disease involving a malformed protein, or prion,
that kills brain cells. After that, health officials learned of three other
suspected cases, including one CJD death in February that was reported only
last month.

''Is what is happening in Idaho an anomaly, a statistical fluke? That is
possible," said Ermias Belay, a top CJD expert with the Centers for Disease
Control and Prevention in Atlanta who is helping advise officials in Idaho.
''But once it exceeds 1.5 or 2 per million, you start asking questions."

''If they are all confirmed, it could be odd," he said.

In a year, the United States typically has fewer than 300 CJD cases, which
mete out rapid death to the elderly, according to the Centers for Disease
Control.

In Twin Falls, Cheryle Becker, epidemiology manager for Idaho's South
Central District Health, is going to families with detailed questionnaires
aimed at finding the roots of a disease that could date back 30 years.

She asks about past travels, unusual hobbies, and dietary habits, including
eating organ meats, brain, and venison.

''We're asking them if they've consumed elk," Becker said, adding that many
people hunt venison in Idaho. ''We're not having many people say that they
have."

Specialists said they do not expect to find a link to eating meat, although
locals are asking whether there is any connection to the human variant of
mad cow disease. ''It's very frightening to the community," said Cheryl
Juntunen, director of the South Central District Health.

Two confirmed US cases of mad cow disease, one in a Washington state dairy
animal in 2003 and the other in a Texas beef cow this year, have further
heightened concern.

Health specialists have found few parallels among the women, all of European
heritage. Four were Idaho natives, all had children, and none had
experienced neurological disease.

One had spent time in Britain before the outbreak of mad cow disease there,
officials said. Several husbands were involved in farming, as is commonplace
in a rural farmland region.

''There are things that lead you to believe this is not variant CJD," Becker
said.

© Copyright 2005 Globe Newspaper Company.


http://www.boston.com/news/nation/articles/2005/08/16/brain_disease_deaths_investigated/?rss_id=Boston+Globe+--+National+News


ERMIAS is at it again. just another fluke. 5 possible 6 cases cjd in one
state in one year, with a population of 1.4 million, and it's just another
fluke. r i g h t , just like those mad cows they tried but failed to cover
up in Texas were. just like the other cow that was suspect but they forgot
about that too and left lie up on a shelf in preservative as to stop all
possible WB testing for confirmation. let the spin begin, all cjd clusters
in the USA are a fluke. a fluke of lies, cover-up and deciet. ...


POLITICS and SAVE THE INDUSTRY AT ALL COST,
including the death of maybe YOUR MOTHER, FATHER,
BROTHER, SISTER, AUNT, UNCLE or maybe just a friend...

Date: Fri, 2 Mar 2001 23:27:10 +0000 (GMT)
From:
Subject: confidential
To: "Terry S. Singeltary Sr."


Okay, you need to know. You don't need to pass it on
as nothing will come of it and there is not a damned
thing anyone can do about it. Don't even hint at it
as it will be denied and laughed at..........
USDA is gonna do as little as possible until there is
actually a human case in the USA of the
nvcjd........if you want to move this thing along and
shake the earth....then we gotta get the victims
families to make sure whoever is doing the autopsy is
credible, trustworthy, and a saint with the courage of
Joan of Arc........I am not kidding!!!!
so, unless we get a human death from EXACTLY the same
form with EXACTLY the same histopath lesions as seen
in the UK nvcjd........forget any action........it is
ALL gonna be sporadic!!!

And, if there is a case.......there is gonna be every
effort to link it to international travel,
international food, etc. etc. etc. etc. etc. They
will go so far as to find out if a sex partner had
ever traveled to the UK/europe, etc. etc. ....
It is gonna be a long, lonely, dangerous twisted
journey to the truth. They have all the cards, all
the money, and are willing to threaten and carry out
those threats....and this may be their biggest
downfall.


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

CJD WATCH MESSAGE BOARD
TSS
Power to the paranoid people
Sat Oct 15, 2005 09:29
68.238.101.188


Power to the paranoid people
15 October 2005
NewScientist.com news service
Debora MacKenzie
CJD is a horrific disease. Whether it's the variant CJD that is caused by
eating animals with BSE, or the sporadic variety that seems to come out of
nowhere, it's a bad way to die. Sporadic CJD happens all over the world. But
fortunately it is rare, striking on average about one person in a million
per year. So when it was revealed that the state of Idaho, population not
much more than 1 million, has had no fewer than seven cases of sporadic CJD
since February, you can't blame people there for being a bit rattled.

No one is known to have contracted variant CJD in the US. But last year a
US-born cow was found to have BSE, and a related disease called CWD afflicts
elk and deer in states close to Idaho. So not surprisingly, campaigners and
conspiracy theorists who specialise in CJD latched onto Idaho's cluster of
CJD cases as evidence that the authorities are covering things up: that
there are way more mad cows, or mad deer, in the US diet than they admit.

Scientists hate this kind of thing, not least because they are often
included among those accused of the cover-up. It is also true that many
scare stories of this kind rely on people's ignorance of statistics.

Take Idaho. The cluster of CJD cases there may be the result of nothing more
sinister than the workings of chance, says Tim Sly, a public health expert
at Ryerson University in Toronto. If a disease is expected to occur at 1 in
a million overall, and we imagine the US's population split into subgroups
of 1 million each, then the chances of exactly one case occurring in every
subgroup in any one year is around 10 ^ -19 That's 1 in 10 billion billion.
It is virtually certain that some of the hypothetical subgroups will have
several cases and some none.

Still, as Sly points out, you don't get news stories saying, "Our state
didn't have any CJD this year, and we should have had at least one case." In
fact, over the last 20 years, Idaho has had on average about 1.3 cases per
million per year. That's close to the predicted incidence. What's more, the
1-in-a-million average is probably lower than the true incidence.
Surveillance for CJD is notoriously patchy, and the number of cases
typically rises when it improves. The recent flurry of diagnoses in Idaho
happened after the state decided that, with all the worries over BSE,
doctors should report every case of CJD.

But even if there is nothing amiss in Idaho, does that mean we should be
telling the conspiracy theorists to shut up and leave public discourse and
policy to the select few who understand statistics? Internet commentator
Henry Niman clearly doesn't think so. He has tracked the unfolding saga of
bird flu, and has posted on his website his own worst-case interpretations
for every twist. These have occasionally been uncritically quoted by the
press, to the annoyance of some scientists, leading one prominent public
health expert to ask if there wasn't some legal way to shut Niman down.

But annoying as these conspiracy theorists can sometimes be, suppressing
them would be a bad idea. Part of the reason the authorities are paying
close attention to CJD in Idaho is because of the spotlight that internet
campaigners have brought to bear. Without that pressure, they would not have
gone back and looked at the incidence over the last 20 years, and they would
not have improved surveillance. The Idaho CJD cluster may just be bad luck.
But if ever something does go wrong it will show up as just such a cluster
of cases. Can we trust the authorities to tell us when that happens? Their
track record is not reassuring.

The campaigners' attention to the smallest of rumours has at times helped
defeat efforts to suppress important news. The heroic reporters who told a
Chinese-language website about the large numbers of wild birds that had died
from flu at Qinghai Lake in central China, and suspicions of human cases
nearby, would probably have been overlooked by world media had it not been
for Niman, who translated and posted their reports.

Would it be better if we just let experts gather together in exclusive chat
rooms, decide what was happening, and then tell us all? Possibly. Except
that scientists, like any other group, have their own agendas, and these may
not necessarily serve the greater good. The recreation of the deadly 1918
flu virus this month was an exciting piece of science, but was it worth the
risk it would pose to public health if it escaped? There are questions: the
review process was far from transparent. How do we know this? From internet
campaigners and conspiracy theorists.

Scientists have usually had to work hard to achieve their expertise, and
with this can come a nasty streak of elitism. Some would prefer not to let
those less expert than themselves have their say. True, uninformed
commentators - especially those who fail to grasp the basics of statistics -
can be a distraction from what experts believe to be the job in hand. But
the same could be said for any opposition to the powers that be in a
democracy. The conspiracy theorists may be monomaniacs, but they keep a
sterner, more unyielding eye on officialdom and its scientists than we poor
journalists ever can. We need them.

http://www.newscientist.com/channel/opinion/mg18825212.900


http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf


http://www.bseinquiry.gov.uk/files/yb/1976/10/12004001.pdf


http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf


http://www.bseinquiry.gov.uk/files/sc/seac17/tab03.pdf


http://www.bseinquiry.gov.uk/files/mb/m11b/tab01.pdf


http://www.bseinquiry.gov.uk/files/yb/1988/10/00001001.pdf


SCRAPIE USA JULY 2005 UPDATE

AS of July 31, 2005, there were 120 scrapie infected soure flocks (figure
3). There were 16 new infected and source flocks reorted in July (Figure 4)
with a total of 143 flocks reported for FY 2005 (Figure 5). The total
infected and source flocks that have been released in FY 2005 are 89 (Figure
6), with 8 flocks released in July. The ratio of infected and source flocks
released to newly infected and source flocks for FY = 0.62 : 1. IN addition,
as of July 31, 2005, 524 scrapie cases have been confirmed and reported by
the National Veterinary Services Laboratories (NVSL), of which 116 were RSSS
cases (Figure 7). This includes 76 newly confirmed cases in July 2005
(Figure 8). Fifteen cases of scrapie in goats have been reported since 1990
(Figure 9). The last goat case was reported in May 2005. ...........

snip...

http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html


SCRAPIE USA JUNE 2005 UPDATE


AS of June 30, 2005, there were 114 scrapie infected and source flocks
(Figure 3). There were 14 new infected and source flocks reported in June
(Figure 4) with a total of 123 flocks reported for FY 2005 (Figure 5).


snip...


In addition, as of June 30, 2005, 448 scrapie cases have been confirmed and
reported by the National Veterinary Services Laboratories (NVSL), of which
106 were RSSS cases (Figure 7). This includes 81 newly confirmed cases in
June 2005 (Figure 8). Fifteen cases of scrapie in goats have been reported
since 1990 (Figure 9). The last goat case was reported in May 2005.


snip...end


http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html


From: TSS ()
Subject: SCRAPIE USA UPDATE MARCH - JUNE 2005
Date: August 24, 2005 at 7:03 pm PST

SCRAPIE USA MONTHLY REPORT 2005

AS of March 31, 2005, there were 70 scrapie infected source flocks (Figure
3). There were 11 new infected and source flocks reported in March (Figure
4) with a total of 51 flocks reported for FY 2005 (Figure 5). The total
infected and source flocks that have been released in FY 2005 are 39 (Figure
6), with 1 flock released in March. The ratio of infected and source flocks
released to newly infected and source flocks for FY 2005 = 0.76 : 1. IN
addition, as of March 31, 2005, 225 scrapie cases have been confirmed and
reported by the National Veterinary Services Laboratories (NVSL), of which
53 were RSSS cases (Figure 7). This includes 57 newly confirmed cases in
March 2005 (Figure 8). Fourteen cases of scrapie in goats have been reported
since 1990 (Figure 9). The last goat cases was reported in January 2005. New
infected flocks, source flocks, and flocks released or put on clean-up plans
for FY 2005 are depicted in Figure 10. ...

FULL TEXT ;

http://www.aphis.usda.gov/vs/nahps/scrapie/monthly_report/monthly-report.html


Mom DOD 12-14-97 Heidenhain Variant Creutzfeldt Jakob Disease CONFIRMED


TSS


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

ANOTHER CJD CONFIRMED IN IDAHO CLUSTER (Scjd)
Wed Feb 1, 2006 08:07
71.248.143.249


##################### Bovine Spongiform Encephalopathy #####################

TSS
Final CJD test results in Woman died from Scjd
Wed Feb 1, 2006 09:09
71.248.143.249


Final CJD test results in
Woman died from classic form of brain disease
By Sandy Miller
Times-News writer

TWIN FALLS -- Final test results on brain tissue have confirmed another
Idaho woman died from the classic form of Creutzfeldt-Jakob disease.

"Test results showed it was not the variant form of CJD," said Tom Shanahan,
spokesman for the Idaho Department of Health and Welfare, on Tuesday. The
variant form of CJD is caused by eating meat from a cow with bovine
spongiform encephalopathy, commonly known as mad cow disease.

Since January 2005, the Idaho Department of Health and Welfare has received
nine reports of people -- seven women and two men -- diagnosed with
Creutzfeldt-Jakob disease -- or CJD -- a fatal brain-wasting disease carried
by prions, an abnormal form of protein in the bloodstream. Prions cause
folding of normal protein in the brain, leading to brain damage. Symptoms
include dementia and other neurological signs. Its victims usually die
within four or five months after onset of the disease, according to the
Centers for Disease Control and Prevention.

The cases included four women from Twin Falls County, a woman from Minidoka
County, a woman from Benewah County in northern Idaho, a woman from Bear
Lake County in the southern corner of Idaho on the Utah border, a man from
Elmore County and a man from Caribou County in southeastern Idaho.

Of the nine people in Idaho who have died, five had autopsies and their
brain tissue was sent to the National Prion Disease Pathology Surveillance
Center at Cleveland's Case Western Reserve University.

Of those five, three women -- two women from Twin Falls County and the woman
from Benewah County -- tested positive for a prion disease, and final
results on all three of them have now shown they died of classic CJD and not
the variant form.

Two people, including the Elmore County man and a Twin Falls woman, tested
negative for a prion disease.

Autopsies were not performed on the other four suspected CJD victims.
However, a CDC neurologist has reviewed their medical records, Shanahan
said.

The number of cases is highly unusual. Normally, there is one case of CJD
per million people a year. Between 1984 and 2004, Idaho averaged 1.2 cases a
year, Shanahan said. He said there was one year during that period when
Idaho had three cases.

Because of their ages -- all of the victims except one were over the age of
60 -- health officials suspected they died of classic CJD and not the
variant. However, the only way to confirm CJD is by testing brain tissue,
according to the National Prion Disease Pathology Surveillance Center.

Times-News writer Sandy Miller can be reached at 735-3264 or by e-mail at
smiller@magicvalley.com.

Story published at magicvalley.com on Wednesday, February 01, 2006


http://www.magicvalley.com/articles/2006/02/01/news_localstate/news_local_state.3.txt

Creutzfeldt-Jakob Disease in Northeast Texas, J.A. Rawlings,*1 K.A.
Hendricks1, O.M. Nuno1, D.A. Brown1, D.A. Evans2, Texas Department of
Health, 1Austin and 2Tyler, Texas
Creutzfeldt-Jacob Disease (CJD), a transmissible spongiform
encephalopathy, is caused by prions composed of proteinaceous material
devoid of nucleic acid. CJD occurs sporadically (generally 1
case/1,000,000 population per year) in older patients (average age of
65) and is characterized by rapidly progressive dementia, accompanied by
severe muscle spasms and incoordination. Death usually occurs within 3
to 12 months (average 7 months). CJD activity in Texas, which has a
population of nearly 19 million, appeared to be typical. The statewide
death rate for 1995 and 1996 was just under 1/1,000,000. In April of
1997, the Texas Department of Health became aware of an increased number
of possible CJD cases in a 23-county area of NE Texas with a population
of just over one million. After review of medical and pathology records,
four patients were identified with definite classic CJD and three were
identified with probable CJD. Dates of death for the eight patients were
from April, 1996 through mid-July 1997. The patients were from 46
through 65 years of age; four were male and three were female. A
case-control study to identify risks for CJD in NE Texas has been initiated.

http://www.jifsan.umd.edu/tse/Rawlings.htm

what i considered a cluster of CJD victims in southeast Texas,
never got the attention of the TDH or CDC. it was just all
sporadic CJDs of no importance...tss

1: Ann Clin Lab Sci 2001 Apr;31(2):211-2

A cluster of Creutzfeldt-Jacob disease patients from Nassau County,
New York, USA.

Adikari D, Farmer P.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11337912&dopt=Abstract

A Cluster of Creutzfeldt-Jacob Disease Patients from Nassau County, New
York, USA

February, 2001 Annals of Clinical and Laboratory Science Vol. 31
no. 2 p. 211-2

Creutzfeldt-Jacob Disease (CJD) is a rare and transmissible neurological
disorder, which has been increasing in frequency in the United States
over the past two decades. [...]

We report 7 cases of CJD from North Shore University Hospital, a
community-based teaching institution that serves Nassau County on Long
Island, NY. These cases were diagnosed and died during the 12-mo period
between mid-June 1999 to midJune 2000... These data suggest a CJD death
rate in Nassau County... approximately 6 times the national rate. During
the previous 1-yr period, no case of CJD was diagnosed in our laboratory

The authors are concerned that the number of CJD cases in our catchment
area appears to have increased dramatically during the 12-mo period.

http://www.organicconsumers.org/madcow/cluster501.cfm

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

Farmer PM, Kane WC, Hollenberg-Sher J.

Incidence of Creutzfeldt-Jakob disease in Brooklyn and Staten Island.

N Engl J Med. 1978 Feb 2;298(5):283-4. No abstract available.
PMID: 339092
=============

2: Nosal R, Kapoor A, Shanin R.

Cluster of cases of Creutzfeldt-Jakob disease--Ontario.
Can Dis Wkly Rep. 1991 Jan 19;17(3):12. No abstract available.
PMID: 2001596
==============

There is one report of a possible cluster of CJD cases in Canada;
between April 1989 and October 1990, six cases were reported in the
province of Ontario, from a population of 9.5 million (1986 census
figure). Two of the patients had come from areas of Czechoslovakia with
a high incidence of familial-type disease, but no other risk factors
were associated with these cases (7).

http://www.cdc.gov/ncidod/eid/vol3no1/stratton.htm


An Evaluation of a Suspected Cluster

of Creutzfeldt-Jakob Disease (CJD) in

New Jersey

May 2004

Division of Epidemiology, Environmental and

Occupational Health


snip...


17

5. Evaluation of suspected cluster of CJD

The evidence does not support the existence of an outbreak of CJD among
attendees at

the GSRT, nor does it suggest that case-patients with CJD were exposed to
BSEcontaminated

beef in the period 1988 to 1992 or at any other time at the Garden State

Racetrack in New Jersey.

a. The largest and earliest cattle outbreak of BSE in the world occurred in
the UK and to

date the number of infected cattle has been many orders of magnitudes
greater than the

number of reported probable and definite vCJD cases in the UK. Thus, an
outbreak of

either variant or sporadic CJD in the US without more evidence of BSE in
this country is

extremely unlikely.

b. All persons throughout the world with BSE-related vCJD tested as of
January 2004

have had a specific genetic make-up characteristic of about 40% of the
general

population (Will et al, 2004). None of the three pathologically confirmed
CJD casepatients

who were tested for this marker were documented to have it.

c. The UK’s National CJD Surveillance Unit reports no evidence that sporadic
CJD has

been caused by BSE. There was an increase in sporadic CJD in the UK from
1990-1996

due to improved case ascertainment (SEAC, 2004). This is to be expected for
any disease

18

following the institution of a new and active surveillance system. However,
there has

been no unusually high incidence of sporadic forms of CJD from 1997-2003 in
the UK,

despite the much heavier exposure of the UK population to the BSE agent
between 1980

and 1996 compared to the US population (UK Department of Health).

d. Ongoing comparisons of exposures of sporadic CJD case-patients with
controls in

Europe, as conducted and interpreted by experts in the UK, have been
reported to provide

no convincing evidence of an increased risk of sporadic forms of CJD through
dietary

habits (Will et al., 2004).

e. As illustrated in Figure 1, all but two of the presumed food-related
deaths due to the

BSE agent in the UK occurred in person younger than 55 years of age. In
contrast, based

on the information available at NJDHSS through April 2004, 10 of the 11
decedents with

definite or probable CJD between 1995-2003 about whom JS has raised concerns
were

>55 at the time of death.

f. None of the six patients in this report who had brain tissue submitted
for examination at

the NPDPSC were documented to have vCJD, the form associated with BSE
exposure.

Rather, they each had either a non-prion disease or form of sporadic CJD
that differed

from each other. These laboratory data provide scientific evidence against a
theory that

any of these illnesses were related to each other or to BSE.

h. There is no evidence of an increased number of CJD case-patients in the
region closest

to the GSRT, in adjacent Philadelphia County, or in New York City.

F. Future CJD surveillance plans in New Jersey

1. General

The key to investigating potential causes of CJD in the US is to encourage
brain

autopsies of all patients who die of suspected CJD. New Jersey is among the
states that

require reporting by medical providers of suspected case-patients with CJD.
Because

physicians nationally and in NJ generally do an inadequate job of reporting
all reportable

diseases, states use additional methods to ensure as complete ascertainment
of cases of

interest as possible. The NJDHSS currently also reviews NJ hospital billing
data and

death certificate data to detect case-patients with CJD that were not
reported by

physicians, making it more likely to have complete ascertainment of
case-patients than

other states. The CDC also reviews national mortality data from the National
Center for

Health Statistics. When a CJD case-patient under 55 years of age is
reported, the CDC

contacts the state health department to review the details of this
case-patient.

The NJDHSS periodically sends out to all physicians in the state, and to
every newly

licensed physician, information on reporting requirements for all diseases
and conditions

listed in the Administrative Code regulations (N.J.A.C. 8:57), including
CJD. The

NJDHSS plans to notify physicians in the next few months about the recently
adopted

revised regulations pertaining to all reportable diseases, including
materials such as

pocket cards and magnets to remind physicians and other health care
providers about

their reporting responsibilities.

In addition, the NJDHSS will be sending a specific letter prepared by the
NPDPSC to

neurologists and pathologists, informing them of the need to consider CJD in
the

differential diagnosis of a patient with a rapidly progressive neurological
disorder with

dementia, and the free services provided by the NPDPSC. Furthermore, the
NPDPSC is

prepared to make arrangements and provide for the costs of the autopsy.

The NJDHSS will continue to review all suspected cases-patients with CJD
detected

through passive reporting by health providers, and by reviewing hospital
billing data and

death certificate data. It will encourage physicians to obtain permission
from family

members for autopsies of suspected case-patients at the time of death and
submit brain

tissue and other laboratory data to the NPDPSC for expert analysis.

The CJD program at the CDC also has notified the NJDHSS that it intends to
provide

limited funds, as it does to several other states, to enhance CJD
surveillance activities in

the state. The NJDHSS plans to enhance education on prion disease, targeted
to

neurologists and pathologists, the physicians most likely to be in a
position to diagnose a

potential case of CJD. Unfortunately, autopsies in general are now only
rarely done and

this is especially true for suspected CJD case-patients where pathologists
are reluctant to

perform autopsies (Louie at al, 2004). However, as a result of the effort
made by the

autopsy program of the NPDPSC, the number of autopsies performed in CJD
casepatients

is currently close to 60% of the number of expected cases.

2. Continued evaluation of the suspected CJD cluster at the GSRT

The NJDHSS will update its report on the suspected cluster of CJD
case-patients at the

GSRT once it receives the information from the pending case-patients. If
there are

additional reported case-patients that need to be assessed, the NJDHSS will
evaluate

those that were NJ residents, and will refer the non-NJ resident
case-patients to other

states for evaluation, where appropriate.

Given the epidemiologic and pathologic evidence described above, the NJDHSS
believes

that conducting a case-control study by interviewing next-of-kin or friends
of the definite

and probable cases is unwarranted at this point for assessing the proposed
hypothesis that

BSE-contaminated beef in the US caused an increased incidence or cluster of
CJD cases

in New Jersey. The proposed hypothesis is not supported by the weight of the
evidence

presented in this report and the CDC report (CDC, 2004a) to justify
conducting such a

research project that is very unlikely to yield meaningful information.

http://www.state.nj.us/health/eoh/cjd2004.pdf


Weekly


May 7, 2004 / 53(Early Release);1-4

Creutzfeldt-Jakob Disease Not Related to a Common Venue --- New Jersey,
1995--2004

Beginning in June 2003, the New Jersey Department of Health and Senior
Services (NJDHSS) and CDC were notified of a suspected cluster of deaths
caused by Creutzfeldt-Jakob disease (CJD) in persons reportedly linked to
Garden State Racetrack in Cherry Hill, New Jersey. Concerns were raised that
these deaths might have resulted from consumption of meat contaminated with
the agent causing bovine spongiform encephalopathy (BSE, commonly called
"mad cow disease") served at racetrack restaurants during 1988--1992.
Consumption of BSE-contaminated cattle products has been linked to a new
variant form of CJD (vCJD) in humans. This report summarizes the results of
an investigation that determined the deaths were not linked causally to a
common source of infection. The findings underscore the need for physicians
to arrange for brain autopsies of all patients with clinically suspected or
diagnosed CJD.

Available clinical and neuropathologic findings were reviewed for 17
suspected CJD deaths referred to NJDHSS and CDC. To investigate the deaths
of these 17 persons, all of whom were reportedly linked to Garden State
Racetrack, health-care providers were contacted and relevant medical records
obtained by NJDHSS, other state health departments, and CDC. Providers were
asked to submit available brain autopsy tissue to the National Prion Disease
Pathology Surveillance Center (NPDPSC), a national prion disease diagnostic
referral laboratory established by CDC and the American Association of
Neuropathologists.

Sufficient demographic and clinical information was available to classify 11
of the 17 deaths as resulting from a definite or probable case of a classic
form of CJD*, on the basis of World Health Organization criteria (1). Of the
remaining six decedents, neuropathologic analyses documented that three
deaths resulted from causes unrelated to either vCJD or classic CJD (Table
1). Three deaths reported as resulting from CJD remain under investigation.
Excluding the three deaths for which CJD was ruled out, the 14 remaining
deaths occurred over a period of approximately 9.25 years (1995--2004); the
average number of cases per complete year (i.e., excluding 2004) was 1.44
(range: zero to three cases). Eleven of the 14 decedents were male; median
age was 69.5 years (range: 50--83 years). Six of the decedents resided in
New Jersey, four in Pennsylvania, and one each in Connecticut, Delaware,
Maryland, and Virginia.

Neuropathologic analysis in the five definite cases with available brain
tissue specimens was diagnostic of classic CJD; none had the characteristic
pathologic findings of vCJD. A genotype at codon 129 of the prion protein
gene (a genetic marker associated with specific subtypes of CJD) was
determined for three of the five CJD deaths confirmed pathologically (Table
1). None of the decedents had the methionine homozyogosity or the
characteristic Western blot pattern present for persons with vCJD. In
addition, the reported CJD subtypes differed from one another. For the six
deaths without tissue diagnosis, available clinical and diagnostic evidence,
including illness duration, electroencephalographic patterns, and presence
of protein 14-3-3 (a marker for classic CJD) in cerebrospinal fluid was
consistent with a probable diagnosis of classic CJD (Tables 1 and 2). None
of the decedents had a diagnosis of vCJD.

For 1995--2002, using CDC's national multiple cause-of-death file (2002 data
are preliminary) compiled annually by the National Center for Health
Statistics, the annual death rate from CJD in the United States has been
stable at approximately one case per 1 million persons per year (Figure 1).
The CJD death rate for New Jersey during the same period was similar.

In 2001, Garden State Racetrack was closed permanently. The number and ages
of all persons visiting or dining at the racetrack is unknown. However,
according to New Jersey Racing Commission records, attendance at the
racetrack during 1988--1992 was approximately 4.1 million. Based on an
annual CJD rate of 3.4 cases per 1 million persons (CDC, unpublished data,
2004) and an overall death rate from all causes of 2.9% for persons aged >50
years, the occurrence over approximately 9.25 years (1995--2004) of at least
14 CJD-related deaths among as few as 300,000 persons aged >50 years would
not be unusual. This number is within the estimated range of the number of
persons attending and dining at the racetrack, given the known attendance.

Reported by: P Gambetti, MD, National Prion Disease Pathology Surveillance
Center, Case Western Reserve Univ, Cleveland, Ohio. J Hadler, MD,
Connecticut Dept of Public Health. A Hathcock, PhD, M Drees, MD, Delaware
Health and Social Svcs. D Blythe, MD, Maryland Dept of Health and Mental
Hygiene. E Bresnitz, MD, M Gerwel, MD, New Jersey Dept of Health and Senior
Svcs. M Hawkins, MD, Philadelphia Dept of Public Health; A Weltman, MD,
Pennsylvania Dept of Health. J Marr, MD, A Buckler, MD, C Novak, MD,
Virginia Health Dept. C Rothwell, MS, K Kochanek, MA, R Anderson, PhD, Div
of Vital Statistics, National Center for Health Statistics; J Sejvar, MD, E
Belay, MD, R Maddox, MPH, A Curns, MPH, R Holman, MS, L Schonberger, MD, Div
of Viral and Rickettsial Diseases, National Center for Infectious Diseases,
CDC.

Editorial Note:

CJD is a neurodegenerative disease characterized by rapidly progressive
dementia associated with brain pathology marked by diffuse spongiform
degeneration; the disease is invariably fatal (2). According to the leading
hypothesis, CJD is caused by an unconventional transmissible agent, an
abnormal protein (i.e., prion) that is able to induce abnormal folding of
normal cellular proteins, leading to neuronal death. Prions are believed to
cause transmissible spongiform encephalopathies (TSEs) that include scrapie
in sheep, BSE in cattle, chronic wasting disease (CWD) in deer and elk, and
CJD in humans.

Two major forms of CJD have been recognized, classic and variant (3).
Classic CJD has been recognized since the early 1920s and is characterized
by certain distinct clinical and diagnostic features (Table 2). The most
common form of classic CJD is believed to occur sporadically, caused by the
spontaneous transformation of normal prion proteins into abnormal prions.
This sporadic disease occurs worldwide at a rate of approximately one case
per 1 million population per year, although rates of up to two cases per
million are not unusual (4). Risk increases with age, and in persons aged
>50 years, the annual rate is approximately 3.4 cases per million.

Variant CJD was first described in 1996 in the United Kingdom and has
different clinical characteristics than classic CJD (Table 2) (2,3). The
median age at death for vCJD patients is 28 years, compared with 68 years
for patients with classic CJD (Figure 2). In addition, all vCJD cases have
neuropathologic findings distinctly different from those of classic CJD (5),
and all have had a particular genetic profile (i.e., homozygosity for
methionine) at codon 129 of the prion protein gene (4). Thus, cases of vCJD
can be distinguished from classic CJD on the basis of clinical and
pathologic data. Epidemiologic and laboratory evidence indicate that the
agent causing BSE in cattle can be transmitted to humans via consumption of
BSE-contaminated cattle products, causing vCJD (2,3). However, this evidence
also suggests that the risk is low for having vCJD, even after consumption
of contaminated product. In 1996, because of the emergence of vCJD in the
United Kingdom, CDC enhanced its surveillance for CJD in the United States
(6).

No evidence has indicated that any of the 17 reported deaths resulted from
vCJD. The CJD subtypes were determined in four decedents, and the subtype in
each differed from the others; this heterogeneity provides scientific
evidence against a common etiology for these cases. Although one study
reported that BSE-infected mice expressing methionine homozygosity at codon
129 produced prions with a molecular phenotype consistent with a subtype of
classic CJD (7), these animal data cannot be reliably extrapolated to humans
in the absence of other supporting evidence. In 2003, the Spongiform
Encephalopathy Advisory Committee of the United Kingdom concluded that these
data did "not provide strong evidence to support" the hypothesis that
exposure to BSE can produce a sporadic CJD-like phenotype in humans (8). In
the United Kingdom, where the largest epidemic of BSE has occurred and an
unusually large proportion of the population has been exposed to the BSE
agent, the absence of an unusually high incidence of classic CJD patients or
an elevated proportion of CJD patients with methionine homozygosity at codon
129 (9) supports the lack of association between BSE and sporadic CJD. In
the United Kingdom, prion disease experts have looked specifically for
evidence of BSE-related disease other than vCJD among classic CJD cases. No
evidence of a new phenotype has been uncovered (R.G. Will, M.D., National
CJD Surveillance Unit, Western General Hospital, Edinburgh, Scotland,
personal communication, 2004).

Neuropathologic evaluation, particularly by immunohistochemistry or Western
blot, is the most definitive method to 1) diagnose human prion diseases, 2)
monitor for vCJD and various subtypes of CJD, and 3) detect the possible
emergence of new prion diseases in the United States. Although not all
decedents in this investigation had pathologic specimens available for
review, demonstration of the absence of a classic CJD or vCJD diagnosis in
certain patients and diagnosis of classic CJD in others indicated these
patients did not die from BSE-related disease. This investigation
underscores the need for physicians to pursue autopsies of all decedents
with clinically suspected and diagnosed CJD and to use the TSE diagnostic
services provided free of charge by NPDPSC. Information regarding this
surveillance center is available at http://www.cjdsurveillance.com or by
telephone, 404-639-3091.

CDC will continue to work with and support state health officials in New
Jersey and nationally to conduct surveillance for CJD. Better defining the
normal occurrence of subtypes of sporadic CJD and other TSEs will facilitate
earlier recognition of vCJD or any other human prion disease that might
emerge in the United States.

* Those types of CJD that differ from vCJD and usually indicate sporadic
CJD.

References

World Health Organization. Global surveillance, diagnosis, and therapy of
human transmissible spongiform encephalopathies: report of a WHO
consultation, 1998. WHO/EMC/ZDI/98.9. Available at http://www.
who.int/emcdocuments/tse/docs/whoemczdi989.pdf.
Belay E, Schonberger L. Variant Creutzfeldt-Jakob disease and bovine
spongiform encephalopathy. Clin Lab Med 2002;22:849--62.
Brown P, Will RG, Bradley R, Asher DM, Detwiler L. Bovine spongiform
encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution,
and current concerns. Emerg Infect Dis 2001;7:6--16.
Will RG, Alpers MP, Dormont D, Schonberger LB. Infectious and sporadic prion
diseases. In: Prusiner SB, ed. Prion Biology and Diseases. New York, New
York: Cold Spring Harbor Laboratory Press, 2004:629--71.
Ironside JW. Neuropathologic findings in new variant CJD and experimental
transmission of BSE. FEMS Immunol Med Microbiol 1998; 21:91--5.
Belay ED, Maddox RA, Gambetti P, Schonberger LB. Monitoring the occurrence
of emerging forms of Creutzfeldt-Jakob disease in the United States.
Neurology 2003;60:176--81.
Asante EA, Linehan JM, Desbruslais M, et al. BSE prions propogate as either
variant CJD-like or sporadic CJD-like prion strains in transgenic mice
expressing human prion protein. EMBO J 2002;23:6358--66.
European Spongiform Encephalopathy Advisory Committee. Final minutes of the
77th annual meeting, February 11, 2003. Available at
http://www.seac.gov.uk/minutes/final77.pdf.
Maddox RA, Belay ED, Schonberger LB. Reply to Singletary. Re: Monitoring the
occurrence of emerging forms of Creutzfeldt-Jakob disease in the United
States (Letter). 2003. Available at
http://www.neurology.org/cgi/eletters/60/2/176.

Table 1


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Figure 1


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Table 2


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Figure 2


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This page last reviewed 5/7/2004


http://www.cdc.gov/mmwr/preview/mmwrhtml/mm53e507a1.htm

The Queniborough CJD cluster

7/14/00
The Times - front page
Alert on village's cluster of CJD deaths
BY ELIZABETH JUDGE AND VALERIE ELLIOTT
AN URGENT government investigation was underway last night into a
cluster of cases of the human form of "mad cow" disease in
Leicestershire, where four people have died.
The investigation follows advice that the four deaths and another
probable case - out of only 75 nationwide - were unlikely to have
occurred by chance. And experts are pointing to the village of
Queniborough as a possible link between them.
Glenn Day, 35, lived there, Stacey Robinson, 18, was a former resident,
and Pamela Beyless, 24, often visited. All three died in 1998
A 19-year-old man who died in May and a 24-year-old who is thought to
have CJD also come from the same part of Leicestershire. It was the
fifth diagnosis in the area that triggered the investigation.

snip...

http://www.mad-cow.org/UKCJD/CJD_news9.html


and how far does the Queniborough CJD cluster really reach ?

I was just looking at this email you sent me. Thurcaston
is about 6 miles from Queniborough - near enough. Is there
any way of contacting this family?

snip...

I've just been talking to professor bob will who runs the CJD
surveillance unit. He is very keen to learn more about the US case you
mentioned to me - the woman who lived in Thurmaston. So am I.

Could you contact them asap and ask if they will speak to us?

snip...

Sadly, everything points to Mrs Soukup being a victim of sporadic CJD.
In particular, she died aged 74 - whereas the oldest victim to date has
been 54. Also she and her husband were in britain in the 1970s - before
any cattle had come down with BSE. IN fact before any of the cattle
destined to get BSE had even been born. Despite this Professor Bob Will
does want to speak to the Soukups to find out more about the case.

snip...

personal email tss

since then, we have a documented case of nv/v CJD in 74 year old.


1: Cent Eur J Public Health 2003 Mar;11(1):19-22

Analysis of unusual accumulation of Creutzfeldt-Jakob disease cases
in Orava and Liptov regions (northern Slovak focus) 1983-2000.

Mad'ar R, Maslenova D, Ranostajova K, Straka S, Baska T.

Institute of Epidemiology, Jessenius Faculty of Medicine, Comenius
University, Sklabinska 26, Martin, 037 53 Slovakia. MADAR@jfmed.uniba.sk

While familial cases of Creutzfeldt-Jakob disease are extremely rare
all over the world, 3 familial clusters were observed between
1983-2000 in a relatively small area situated in the North of
Slovakia. Prevalence of CJD in this area exceeded the overall
prevalence in Slovakia more than 8 times. The majority of CJD
patients admitted consuming sheep brain. Most patients lived in
small secluded villages with rather common familial intermarriage.
CJD affected both sexes equally. All patients were prior to the
disease mentally normal individuals. Shortly after the onset of CJD
their mental status deteriorated remarkably with an average survival
rate of 3.6 months.

PMID: 12690798

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12690798&dopt=Abstract

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

1: Eur J Epidemiol 1991 Sep;7(5):520-3

"Clusters" of CJD in Slovakia: the first laboratory evidence of scrapie.

Mitrova E, Huncaga S, Hocman G, Nyitrayova O, Tatara M.

Institute of Preventive and Clinical Medicine, Bratislava.

Epidemic-like occurrence of Creutzfeldt-Jakob disease was observed in
1987 in Slovakia (Orava). Search for the cause of CJD focus indicated a
coincidence of genetic and environmental risks in clustering patients.
Since Spongiform Encephalopathies might be transmitted orally, (Bovine
Spongiform Encephalopathy), the possibility of zoonotic source of CJD
cases in Orava was also considered. A deficient knowledge about the
occurrence of scrapie in Slovakia stimulated an examination of sheep
with signs of CNS disorders in two flocks of Valasky breed in Orava. In
one flock, neurohistopathological examination revealed in sheep brains
lesions characteristic for scrapie. Frozen brain tissue of these animals
were used for the detection of scrapie associated fibrils. They were
found in 2 animals from the same flock. This is the first laboratory
confirmation of scrapie in Czecho-Slovakia. The possible epidemiological
and economical implications are emphasized.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1761109&dopt=Abstract


Morbidity and Mortality Weekly Report

Weekly February 21, 2003 / Vol. 52 / No. 7

Fatal Degenerative Neurologic Illnesses in Men Who Participated

in Wild Game Feasts — Wisconsin, 2002


depar department tment of health and human ser services vices

Centers for Disease Control and Prevention

INSIDE

128 Atrial Fibrillation as a Contributing Cause of Death and

Medicare Hospitalization — United States, 1999

131 Potential Exposures to Airborne and Settled Surface Dust

in Residential Areas of Lower Manhattan Following the

Collapse of the World Trade Center — New York City,

November 4–December 11, 2001

136 Smallpox Vaccine Adverse Events Among Civilians —

United States, January 24–February 18, 2003

136 Notice to Readers

Creutzfeldt-Jakob disease (CJD) is a fatal neurologic disorder

in humans. CJD is one of a group of conditions known as

transmissible spongiform encephalopathies (TSEs), or prion

diseases, that are believed to be caused by abnormally configured,

host-encoded prion proteins that accumulate in the central

nervous tissue (1). CJD has an annual incidence of

approximately 1 case per million population in the United

States (1) and occurs in three forms: sporadic, genetically

determined, and acquired by infection. In the latter form, the

incubation period is measured typically in years. Recent evidence

that prion infection can cross the species barrier

between humans and cattle has raised increasing public health

concerns about the possible transmission to humans of a TSE

among deer and elk known as chronic wasting disease (CWD)

(2). During 1993–1999, three men who participated in wild

game feasts in northern Wisconsin died of degenerative neurologic

illnesses. This report documents the investigation of

these deaths, which was initiated in August 2002 and which

confirmed the death of only one person from CJD. Although

no association between CWD and CJD was found, continued

surveillance of both diseases remains important to assess

the possible risk for CWD transmission to humans.

Case Reports

Case 1. In December 1992, a Wisconsin man aged 66 years

with a history of seizures since 1969 sought treatment for

recurring seizures, increasing forgetfulness, and worsening

hand tremors. Electroencephalographic (EEG) examination

demonstrated focal epileptiform activity and nonspecific diffuse

abnormalities, but no specific diagnosis was made. In

February 1993, he was hospitalized for increasing confusion,

ataxia, and movement tremors of his extremities. A magnetic

resonance image (MRI) demonstrated mild, nonspecific

enhancement along the inferior parasagittal occipital lobe.

A repeat EEG showed bifrontal intermittent, short-interval,

periodic sharp waves, suggesting a progressive encephalopathy;

a diagnosis of CJD was suspected. The man died later

that month; neuropathologic examination of brain tissue during

autopsy indicated subacute spongiform encephalopathy,

compatible with CJD.

The man was a lifelong hunter who ate venison frequently.

He hunted primarily in northern Wisconsin but also at least

once in Montana. He hosted wild game feasts at his cabin in

northern Wisconsin from 1976 until shortly before his death.

Fixed brain tissue obtained during the autopsy was sent for

analysis to the National Prion Disease Pathology Surveillance

Center (NPDPSC) and reexamined at the institution where

the autopsy was conducted. Histopathologic examination did

not substantiate the diagnosis of prion disease. In addition,

27 brain tissue sections were negative for prions by

immunostaining despite positive antibody reactions against

other proteins (controls), which indicated that other epitopes

in the tissue samples were preserved.

Case 2. In May 1999, a Minnesota man aged 55 years with

no previous history of a neurologic disease sought evaluation

and treatment following a 3-month history of progressive difficulty

in writing and unsteadiness of gait. A computerized

126 MMWR February 21, 2003

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Title]. MMWR 2003;52:[inclusive page numbers].

Centers for Disease Control and Prevention

Julie L. Gerberding, M.D., M.P.H.

Director

David W. Fleming, M.D.

Deputy Director for Public Health Science

Dixie E. Snider, Jr., M.D., M.P.H.

Associate Director for Science

Epidemiology Program Office

Stephen B. Thacker, M.D., M.Sc.

Director

Office of Scientific and Health Communications

John W. Ward, M.D.

Director

Editor, MMWR Series

Suzanne M. Hewitt, M.P.A.

Managing Editor, MMWR Series

David C. Johnson

(Acting) Lead Technical Writer/Editor

Jude C. Rutledge

Teresa F. Rutledge

Jeffrey D. Sokolow, M.A.

Writers/Editors

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Visual Information Specialists

Quang M. Doan

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Information Technology Specialists

Division of Public Health Surveillance

and Informatics

Notifiable Disease Morbidity and 122 Cities Mortality Data

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The MMWR series of publications is published by the

Epidemiology Program Office, Centers for Disease Control

and Prevention (CDC), U.S. Department of Health and

Human Services, Atlanta, GA 30333.

tomography (CT) scan and MRI examination of his head did

not indicate any abnormality. In June 1999, he was hospitalized

following onset of dementia, speech abnormalities, and

myoclonic jerking. An EEG indicated left-hemispheric periodic

sharp waves and moderate generalized background slowing;

CJD was diagnosed clinically. In July 1999, following

worsening symptoms and development of right upper extremity

dystonia, the patient died. Neuropathologic evaluation of

brain tissue during autopsy demonstrated widespread subcortical

spongiform lesions, consistent with CJD.

The man was not a hunter but had a history of eating venison.

He made an estimated 12 visits to the cabin where the

wild game feasts were held, but he participated in only one

feast during the mid-1980s. Sections of fixed and frozen brain

tissue obtained during autopsy were analyzed at NPDPSC,

and prion disease was confirmed by immunohistochemical

and Western blot testing. The Western blot characteristics and

prion disease phenotype in this patient were consistent with

the most common form of sporadic CJD, classified as M/M

(M/V) 1 (3). Subsequent genetic typing confirmed the presence

of methionine homozygosity (M/M) at codon 129 of

the patient’s prion protein gene.

Case 3. In June 1992, a Wisconsin man aged 65 years sought

treatment for progressive slowing of speech, worsening

memory, and personality changes. By January 1993, his speech

was reduced to one-word utterances. Neurologic examination

showed a flat affect, decreased reflexes, and apraxia. A CT

head scan showed mild atrophy, and an EEG was normal.

Pick’s disease was diagnosed. By May, he was unable to perform

any daily living activities; he died in August 1993. Neuropathologic

evaluation of brain tissue during autopsy showed

symmetrical frontal lobe cerebral cortical atrophy and mild

temporal lobe atrophy. No Pick’s bodies or spongiform

lesions were observed.

The man had a history of eating venison and participated

regularly in wild game feasts held at the cabin owned by

patient 1. He was a lifelong hunter and hunted mostly in

Wisconsin but also in Wyoming and British Columbia. No

game was brought to the wild game feasts from his hunting

trips outside of Wisconsin. Examination of fixed brain tissue

sent to NPDPSC demonstrated no lesions indicative of CJD,

and immunohistochemical testing with antibody to the prion

protein did not demonstrate the granular deposits seen in prion

diseases.

Epidemiologic Investigation

Wild game feasts consisting of elk, deer, antelope, and other

game that occurred at a cabin in northern Wisconsin owned

by patient 1 began in 1976 and continued through 2002.

Vol. 52 / No. 7 MMWR 127

These feasts typically involved 10–15 participants and usually

occurred on weekends before or during hunting seasons

in the fall and occasionally in the spring. Wild game brought

to these feasts usually were harvested in Wisconsin, but three

men who attended these feasts reported hunting in the western

United States and bringing game back to Wisconsin. These

activities took place in Colorado (near the towns of Cortez,

Trinidad, Collbran, Durango, and Meeker), Wyoming (near

the towns of Gilette and Cody), and Montana (near the town

of Malta). CWD was not known to be endemic in these areas

at the time that these hunting activities took place.

Information was obtained for 45 (85%) of 53 persons who

were identified as possibly participating in the wild game feasts;

all were male. Information was obtained by direct interview

or from family members of decedents. Of the 45 persons, for

whom information was obtained, 34 were reported to have

attended wild game feasts. Seven of the 34 feast attendees

were deceased, including the three patients. None of the four

other decedents had a cause of death attributed to or associated

with a degenerative neurologic disorder. None of the living

participants had any signs or symptoms consistent with a

degenerative neurologic disorder.

Reported by: JP Davis, MD, J Kazmierczak, DVM, M Wegner, MD,

R Wierzba, Div of Public Health, State of Wisconsin Dept of Health

and Family Svcs. P Gambetti, National Prion Disease Pathology

Surveillance Center, Case Western Reserve University, Cleveland, Ohio.

L Schonberger, MD, R Maddox, MPH, E Belay, MD, Div of Viral and

Rickettsial Diseases, National Center for Infectious Diseases; V Hsu,

MD, EIS Officer, CDC.

Editorial Note: CWD was first described in the United States

in the 1960s and classified as a TSE in 1978. Previously localized

to a contiguous endemic area in northeastern Colorado

and southeast Wyoming, since 2000, CWD has been found

in free-ranging deer or elk in Illinois, Nebraska, New Mexico,

South Dakota, Wisconsin, and outside the previously known

endemic areas of Colorado and Wyoming. CWD has been

identified also in captive deer or elk in Colorado, Kansas,

Minnesota, Montana, Nebraska, Oklahoma, South Dakota,

and Wisconsin (4). Because a variant form of CJD, with specific

neuropathologic and molecular characteristics that distinguish

it from sporadic CJD, has been associated with eating

cattle products infected with a prion that causes bovine

spongiform encephalopathy (5), concern has been raised about

the possibility that the prion associated with CWD might be

transmitted to humans in a similar way.

In this investigation, because only one of the three cases in

Wisconsin had neuropathologic confirmation of a prion disease,

no association could be made between case participation

in the wild game feasts and the development of CJD.

Although patient 2 had confirmed CJD, he was unlikely to

have eaten CWD-infected venison at these feasts because venison

and other game from outside Wisconsin that was served

at these feasts did not originate from known CWD-endemic

areas, and the man participated in the feasts only once. In

addition, the prion disease in this case was consistent with

the most common form of sporadic CJD, without apparent

unusual neuropathologic or molecular characteristics that

might occur if the prion related to CWD had been responsible

for the disease.

The findings in this report are subject to at least two limitations.

First, not all members participating in wild game feasts

could be identified, and not all persons listed as participating

could be contacted for interviews. Second, interviews that were

conducted required recall of events that occurred up to 25

years ago, limiting the detail or accuracy of events. However,

the similar responses obtained from different sources support

the accuracy of the investigation findings.

A previous investigation of unusually young CJD patients

in whom the transmission of CWD was suspected also did

not provide convincing evidence for a causal relationship

between CWD and CJD (2). However, limited epidemiologic

investigations cannot rule out the possibility that CWD

might play a role in causing human illness. Ongoing surveillance

of CJD, particularly in states with CWD, is important

to assess the risk, if any, for CWD transmission to humans.

Because the confirmation of CJD and the detection of a new

prion disease require neuropathologic study of brain tissue,

physicians are encouraged to contact NPDPSC (http://

www.cjdsurveillance.com; telephone, 216-368-0587) to confirm

diagnoses of CJD and to distinguish its various subtypes.

Because of the known severity of TSEs in humans and the

possibility that the CWD prion can affect humans, animals

with evidence of CWD should be excluded from the human

food or animal feed chains. Hunters and wild venison consumers

should follow precautionary guidelines available from

the Wisconsin Department of Agriculture, Trade, and Consumer

Protection (http://datcp.state.wi.us/core/consumerinfo)

to prevent potential exposures to the CWD agent.

References

1. Belay E. Transmissible spongiform encephalopathies in humans. Annu

Rev Microbiol 1999;53:283–314.

2. Belay E, Gambetti P, Schonberger L, et al. Creutzfeldt-Jakob disease in

unusually young patients who consumed venison. Arch Neurol

2001;58:1673–8.

3. Parchi P, Giese A, Capellari S, et al. Classification of sporadic
Creutzfeldt-

Jakob disease based on molecular and phenotypic analysis of 300 subjects.

Ann Neurol 1999;46:224–33.

4. U.S. Department of Agriculture. Positive CWD cases: cumulative

through Dec 2002 (including farm herds already depopulated). Available

at http://aphisweb.aphis.usda.gov/vs/nahps//cwd/USAMapOf

InfectedHerds.jpg.

5.Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-

Jakob disease in the UK. Lancet 1996;347:921–5.


http://www.cdc.gov/mmwr/PDF/wk/mm5207.pdf


I urge you to go over and read all this, especially what belay et al of NIH
said in study, and in private email ;


snip...

Conclusions

The lack of evidence of a link between CWD transmission and unusual cases of
CJD, despite several epidemiologic investigations, and the absence of an
increase in CJD incidence in Colorado and Wyoming suggest that the risk, if
any, of transmission of CWD to humans is low. Although the in vitro studies
indicating inefficient conversion of human prion protein by CWD-associated
prions raise the possibility of low-level transmission of CWD to humans, no
human cases of prion disease with strong evidence of a link with CWD have
been identified. However, the transmission of BSE to humans and the
resulting vCJD indicate that, provided sufficient exposure, the species
barrier may not completely protect humans from animal prion diseases.
Because CWD has occurred in a limited geographic area for decades, an
adequate number of people may not have been exposed to the CWD agent to
result in a clinically recognizable human disease. The level and frequency
of human exposure to the CWD agent may increase with the spread of CWD in
the United States. Because the number of studies seeking evidence for CWD
transmission to humans is limited, more epidemiologic and laboratory studies
should be conducted to monitor the possibility of such transmissions.
Studies involving transgenic mice expressing human and cervid prion protein
are in progress to further assess the potential for the CWD agent to cause
human disease. Epidemiologic studies have also been initiated to identify
human cases of prion disease among persons with an increased risk for
exposure to potentially CWD-infected deer or elk meat (47). If such cases
are identified, laboratory data showing similarities of the etiologic agent
to that of the CWD agent would strengthen the conclusion for a causal link.
Surveillance for human prion diseases, particularly in areas where CWD has
been detected, remains important to effectively monitor the possible
transmission of CWD to humans. Because of the long incubation period
associated with prion diseases, convincing negative results from
epidemiologic and experimental laboratory studies would likely require years
of follow-up. In the meantime, to minimize the risk for exposure to the CWD
agent, hunters should consult with their state wildlife agencies to identify
areas where CWD occurs and continue to follow advice provided by public
health and wildlife agencies. Hunters should avoid eating meat from deer and
elk that look sick or test positive for CWD. They should wear gloves when
field-dressing carcasses, bone-out the meat from the animal, and minimize
handling of brain and spinal cord tissues. As a precaution, hunters should
avoid eating deer and elk tissues known to harbor the CWD agent (e.g.,
brain, spinal cord, eyes, spleen, tonsils, lymph nodes) from areas where CWD
has been identified.


http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

From: TSS (216-119-163-189.ipset45.wt.net)
Subject: CWD aka MAD DEER/ELK TO HUMANS ???
Date: September 30, 2002 at 7:06 am PST

From: "Belay, Ermias"
To:
Cc: "Race, Richard (NIH)" ; ; "Belay,
Ermias"
Sent: Monday, September 30, 2002 9:22 AM
Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS

Dear Sir/Madam,
In the Archives of Neurology you quoted (the abstract of which was
attached to your email), we did not say CWD in humans will present like
variant CJD.

That assumption would be wrong. I encourage you to read the whole
article and call me if you have questions or need more clarification
(phone: 404-639-3091). Also, we do not claim that "no-one has ever been
infected with prion disease from eating venison." Our conclusion stating
that we found no strong evidence of CWD transmission to humans in the
article you quoted or in any other forum is limited to the patients we
investigated.

Ermias Belay, M.D.
Centers for Disease Control and Prevention

> > -----Original Message-----
> > From:
> > Sent: Sunday, September 29, 2002 10:15 AM
> > To: rr26k@nih.gov; rrace@niaid.nih.gov; ebb8@CDC.GOV
> > Subject: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG
> > HUNTERS


Sunday, November 10, 2002 6:26 PM ......snip........end..............TSS

see;

FURTHER into this case study, Colorado Surveillance Program for Chronic
Wasting Disease Transmission to Humans
(TWO SUSPECT CASES) a look at case 1 and case 2 ;


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0704&L=sanet-mg&T=0&P=1165

From: "Terry S. Singeltary Sr."
Subject: CWD UPDATE 88 AUGUST 31, 2007


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&P=450


Date: Wed, 29 Aug 2007 21:13:08 -0500
From: "Terry S. Singeltary Sr."
Subject: CWD NEW MEXICO RECORDS IT'S 19 CASE (near Texas border again)


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=26079

Monitoring the Potential Transmission of Chronic Wasting Disease to Humans
Using a Hunter Registry Database in Wyoming (405 lines)
From: Terry S. Singeltary Sr. <[log in to unmask]>
Date: Thu, 30 Aug 2007 21:23:42 -0500


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&F=&S=&P=27654


Subject: Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease
creates a new prion strain

Date: August 25, 2007 at 12:42 pm PST


Subject: Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease
creates a new prion strain
Date: August 25, 2007 at 12:42 pm PST

J Biol Chem. 2007 Aug 20; : 17709374

Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a
new prion strain.

[My paper] Atsushi Kobayashi , Masahiro Asano , Shirou Mohri , Tetsuyuki
Kitamoto

The genotype (methionine or valine) at polymorphic codon 129 of the human
prion protein (PrP) gene and the type (type 1 or type 2) of abnormal isoform
of PrP (PrP(Sc)) are major determinants of the clinicopathological
phenotypes of sporadic Creutzfeldt-Jakob disease (sCJD). Here we found that
transmission of sCJD prions from a patient with valine homozygosity (129V/V)
and type 2 PrP(Sc) (sCJD-VV2 prions) to mice expressing human PrP with
methionine homozygosity (129M/M) generated unusual PrP(Sc) intermediate in
size between type 1 and type 2. The intermediate type PrP(Sc) was seen in
all examined dura mater graft-associated CJD cases with 129M/M and
plaque-type PrP deposits (p-dCJD). p-dCJD prions and sCJD-VV2 prions
exhibited similar transmissibility and neuropathology, and the identical
type of PrP(Sc) when inoculated into PrP-humanized mice with 129M/M or
129V/V. These findings suggest that p-dCJD could be caused by cross-sequence
transmission of sCJD-VV2 prions.


snip...


In this study, the strain-dependent traits of sCJDMM1
prions were inherited through cross-sequence
transmission without any modification. The
humanized mice with 129V/V produced type 1 PrPres
after inoculation with sCJD-MM1 prions. Because
sCJD-VV1 cases are extremely rare (at most 1-2%
of the total number of sCJD cases) and characterized
by early onset (mean age at onset: 39.3 years) (5),

####################################

our results raise the possibility that CJD cases
classified as VV1 may include cases caused by
iatrogenic transmission of sCJD-MM1 prions or
food-borne infection by type 1 prions from animals,
e.g., chronic wasting disease prions in cervid. In fact,
two CJD-VV1 patients who hunted deer or
consumed venison have been reported (40, 41). The
results of the present study emphasize the need for
traceback studies and careful re-examination of the
biochemical properties of sCJD-VV1 prions.

###################################

In conclusion, cross-sequence transmission of
sCJD-VV2 prions generates a new prion strain with
altered conformational properties and disease
phenotypes as p-dCJD prions. Furthermore, the
newly generated prions have unique transmissibility
including the traceback phenomenon. In the future, if
atypical prion strains emerge through cross-sequence
transmission, especially from animals, traceback
studies will enable us to identify the origin of the
prions.

REFERENCES...snip...end

FULL TEXT ;


http://www.jbc.org/cgi/content/abstract/M704597200v1?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=Cross-sequence+transmission+of+sporadic+Creutzfeldt-Jakob+disease+creates+a+new+&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=21267

OH, and about that CWD to cattle, yep.......


Susceptibility of Cattle to First-passage Intracerebral Inoculation with
Chronic Wasting Disease Agent from White-tailed Deer
A. N. Hamir, J. M. Miller, R. A. Kunkle, S. M. Hall and J. A. Richt
National Animal Disease Center, ARS, USDA, Ames, IA (ANH, JMM, RAK, JAR),
Pathobiology Laboratory, National Veterinary Services Laboratories, Ames, IA
(SMH)

Fourteen, 3-month-old calves were intracerebrally inoculated with the agent
of chronic wasting disease (CWD) from white-tailed deer (CWDwtd) to compare
the clinical signs and neuropathologic findings with those of certain other
transmissible spongiform encephalopathies (TSE, prion diseases) that have
been shown to be experimentally transmissible to cattle (sheep scrapie, CWD
of mule deer [CWDmd], bovine spongiform encephalopathy [BSE], and
transmissible mink encephalopathy). Two uninoculated calves served as
controls. Within 26 months postinoculation (MPI), 12 inoculated calves had
lost considerable weight and eventually became recumbent. Of the 12
inoculated calves, 11 (92%) developed clinical signs. Although spongiform
encephalopathy (SE) was not observed, abnormal prion protein (PrPd) was
detected by immunohistochemistry (IHC) and Western blot (WB) in central
nervous system tissues. The absence of SE with presence of PrPd has also
been observed when other TSE agents (scrapie and CWDmd) were similarly
inoculated into cattle. The IHC and WB findings suggest that the diagnostic
techniques currently used to confirm BSE would detect CWDwtd in cattle,
should it occur naturally. Also, the absence of SE and a distinctive IHC
pattern of CWDwtd and CWDmd in cattle suggests that it should be possible to
distinguish these conditions from other TSEs that have been experimentally
transmitted to cattle.


http://www.vetpathology.org/cgi/content/abstract/44/4/487


yep, they said BSE would not transmit to humans, until it did. blood would
not transmit this agent, until it did. in fact CWD has now transmitted to
cattle AND TO SHEEP in the lab. oral route takes the longest. we will have
to wait and see. ...


now, what about TSE and Alzheimer's ??? i heard that question come up ;


Alzheimer-type neuropathology 28 year old patient with idCJD
Sun Feb 19, 2006 11:14
71.248.144.164


SHORT REPORT
Alzheimer-type neuropathology in a 28 year old patient with iatrogenic
Creutzfeldt-Jakob disease after dural grafting
M Preusser1, T Ströbel1, E Gelpi1,2, M Eiler3, G Broessner4, E Schmutzhard4
and H Budka1,2
1 Institute of Neurology, Medical University Vienna, Austria
2 Austrian Reference Centre for Human Prion Diseases (OERPE), General
Hospital Vienna, Austria
3 Department of Neurology, LKH Rankweil, Austria
4 Department of Neurology, Medical University Innsbruck, Innsbruck, Austria


Correspondence to:
Dr H Budka
Institute of Neurology, Medical University of Vienna, Waehringer Guertel
18-20, 4J, 1097 Vienna, Austria; herbert.budka@kin.at


ABSTRACT
We report the case of a 28 year old man who had received a cadaverous dura
mater graft after a traumatic open skull fracture with tearing of the dura
at the age of 5 years. A clinical suspicion of Creutzfeldt-Jakob disease
(CJD) was confirmed by a brain biopsy 5 months prior to death and by
autopsy, thus warranting the diagnosis of iatrogenic CJD (iCJD) according to
WHO criteria. Immunohistochemistry showed widespread cortical depositions of
disease associated prion protein (PrPsc) in a synaptic pattern, and western
blot analysis identified PrPsc of type 2A according to Parchi et al.
Surprisingly, we found Alzheimer-type senile plaques and cerebral amyloid
angiopathy in widespread areas of the brain. Plaque-type and vascular
amyloid was immunohistochemically identified as deposits of beta-A4 peptide.
CERAD criteria for diagnosis of definite Alzheimer’s disease (AD) were met
in the absence of neurofibrillar tangles or alpha-synuclein immunoreactive
inclusions. There was no family history of AD, CJD, or any other
neurological disease, and genetic analysis showed no disease specific
mutations of the prion protein, presenilin 1 and 2, or amyloid precursor
protein genes. This case represents (a) the iCJD case with the longest
incubation time after dural grafting reported so far, (b) the youngest
documented patient with concomitant CJD and Alzheimer-type neuropathology to
date, (c) the first description of Alzheimer-type changes in iCJD, and (d)
the second case of iCJD in Austria. Despite the young patient age, the
Alzheimer-type changes may be an incidental finding, possibly related to the
childhood trauma.


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

http://jnnp.bmjjournals.com/


CJD1/9 0185


Ref: 1M51A

IN STRICT CONFIDENCE


Dr McGovern From: Dr A Wight

Date: 5 January 1993

Copies: Dr Metters

Dr Skinner

Dr Pickles

Dr Morris

Mr Murray


TRANSMISSION OF ALZHEIMER-TYPE PLAQUES TO PRIMATES


1. CMO will wish to be aware that a meeting was held at DH yesterday,
4 January, to discuss the above findings. It was chaired by Professor
Murray (Chairman of the MRC Co-ordinating Committee on Research in
the Spongiform Encephalopathies in Man), and attended by relevant
experts in the fields of Neurology, Neuropathology, molecular biology,
amyloid biochemistry, and the spongiform encephalopathies, and by
representatives of the MRC and AFRC.

2. Briefly, the meeting agreed that:

i) Dr Ridley et als findings of experimental induction of p amyloid
in primates were valid, interesting and a significant advance in the
understanding of neurodegeneradve disorders;

ii) there were no immediate implications for the public health, and no
further safeguards were thought to be necessary at present; and

iii) additional research was desirable, both epidemiological and at the
molecular level. Possible avenues are being followed up by DH
and the MRC, but the details will require further discussion.

93/01.05/4.1tss


http://www.bseinquiry.gov.uk/files/yb/1993/01/05004001.pdf

BSE101/1 0136


IN CONFIDENCE

5 NOV 1992

CMO From: Dr J S Metters DCMO 4 November 1992


TRANSMISSION OF ALZHEIMER TYPE PLAQUES TO PRIMATES


1. Thank you for showing me Diana Dunstan's letter. I am glad that MRC have
recognised the public sensitivity of these findings and intend to report
them in their
proper context. This hopefully will avoid misunderstanding and possible
distortion by
the media to portray the results as having more greater significance than
the findings
so far justify.


2. Using a highly unusual route of transmission (intra-cerebral injection)
the
researchers have demonstrated the transmission of a pathological process
from two
cases one of severe Alzheimer's disease the other of Gerstmann-Straussler
disease to
marmosets. However they have not demonstrated the transmission of either
clinical
condition as the "animals were behaving normally when killed'. As the report
emphasises the unanswered question is whether the disease condition would
have
revealed itself if the marmosets had lived longer. They are planning funher
research
to sec if the conditions, as opposed to the partial pathological process, is
transmissible.


What are the implications for public health?


3. . The route of transmission is very specific and in the natural state of
things
highly unusual. However it could be argued that the results reveal a
potential risk,
in that brain tissue from these two patients has been shown to transmit a
pathological
process. Should therefore brain tissue from such cases be regarded as
potentially
infective? Pathologists, morticians, neuro surgeons and those assisting at
neuro
surgical procedures and others coming into contact with "raw" human brain
tissue
could in theory be at risk. However, on a priori grounds given the highly
specific
route of transmission in these experiments that risk must be negligible if
the usual
precautions for handling brain tissue are observed.


92/11.4/1-1


BSE101/1 0137


4. The other dimension to consider is the public reaction. To some extent
the GSS
case demonstrates little more than the transmission of BSE to a pig by
intra-cerebral
injection. If other prion diseases can be transmitted in this way it is
little surprise that
some pathological findings observed m GSS were also transmissible to a
marmoset.
But the transmission of features of Alzheimer's pathology is a different
matter, given
the much greater frequency of this disease and raises the unanswered
question whether
some cases are the result of a transmissible prion. The only tenable public
line will
be that "more research is required" before that hypothesis could be
evaluated. The
possibility on a transmissible prion remains open. In the meantime MRC needs
carefully to consider the range and sequence of studies needed to follow
through from
the preliminary observations in these two cases. Not a particularly
comfortable
message, but until we know more about the causation of Alzheimer's disease
the total
reassurance is not practical.


JS METTERS
Room 509
Richmond House
Pager No: 081-884 3344
Callsign: DOH 832

121/YdeStss

92/11.4/1.2

http://www.bseinquiry.gov.uk/files/yb/1992/11/04001001.pdf

also, see the increase of Alzheimer's from 1981 to 1986


http://www.bseinquiry.gov.uk/files/yb/1988/07/08014001.pdf

again, BSE and mice, and this disease specific statement. BSE has
transmitted to many species;

lots of previous fse cheetas

the new one could simply be an export from the UK that is making the rounds,
like the others. for sure the netherlands knows the exact history as all
zoos maintain precise records on rare species.

the UK fed split skulls of cattle to their large zoo cats ... duh

http://www.mad-cow.org/zoo_cites_annotated.html

The 82 zoo animals with BSE as of 22 April 1999.:

Id TSE Genus Species Subsp Birth Origin Death Place of Death
1 + Actinonyx jubatus cheetah 1986 Marwell zoo 1991 Pearle Coast AU
Duke + Actinonyx jubatus cheetah 1984 Marwell zoo 1992 Colchester zoo? UK
Saki + Actinonyx jubatus cheetah 1986 Marwell zoo 1993 unknown UK
Mich + Actinonyx jubatus cheetah 1986 Whipsnade 1993 Whipsnade UK
Fr1 + Actinonyx jubatus cheetah 1987 Whipsnade 1997 Safari de Peaugres FR
Fr2 + Actinonyx jubatus cheetah 1991 Marwell zoo 1997 Safari de Peaugres Fr
xx + Actinonyx jubatus cheetah 19xx xxx zoo 199x Fota zoo IR
yy + Actinonyx jubatus cheetah 19xx yyy zoo 1996+ yyyy zoo UK
zz + Actinonyx jubatus cheetah 19xx zzz zoo 1996+ yyyy zoo UK

aaa + Felis concolor puma 1986 Chester zoo 1991 Chester zoo UK
yy + Felis concolor puma 1980 yyy zoo 1995 yyyy zoo UK
zz + Felis concolor puma 1978 zzz zoo 1995 zzzz zoo UK

xxx + Felis pardalis ocelot 1987 xxx 1994 Chester zoo UK
zzz + Felis pardalis ocelot 1980 zzz 1995 zzzz zoo UK

85 + Felis catus cat 1990+ various 1999+ various UK LI NO
19 + Canis familia. dog 1992+ various 1999+ various UK

Fota + Panthera tigris tiger 1981 xxx zoo 1995 xxxx zoo UK
yy + Panthera tigris tiger 1983 yyy zoo 1998 yyyy zoo UK

Lump + Panthera leo lion 1986 Woburn SP 1998 Edinburgh zoo UK [since 1994]


or see the links under Zoo BSE at
http://www.mad-cow.org/00/sci_archive_frame.html

Zoo Prion Disease: review of scientific literature
Database of 82 zoo animals with prion disease
Switcheroo -- mysteries at the MAFF home page
More cheetah TSE 9 years after 'ban'
The case of the missing pumas, tigers, ocelots, and lion
How many house cats really had TSE?
The Bristol Zoo tigers: 1970-77
Horizontal transmission demonstrated in kudu
Eland, nyala, bison, ankole, arabianory, and scimitar-horned oryx
French primate disaster
Primate BSE quarantine recommendations
TSEs in primates: brief literature survey
Lemur prion sequences predicted
Prosimians: which animals are involved so far?
Earlier BSE problems at French, Australian, and Irish zoos
Further blow aimed at surviving primates
Which zoos, which primates, how many animals?


tss


----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Sent: Friday, September 07, 2007 10:57 AM
Subject: [BSE-L] CHEETAH AT ZOO IN NUREMBERG CONTRACTS FELINE SPONGIFORM
ENCEPHALOPATHY FSE


> Subject: CHEETAH AT ZOO IN NUREMBERG CONTRACTS FELINE SPONGIFORM
> ENCEPHALOPATHY FSE
> Date: September 7, 2007 at 8:26 am PST
>
> New BSE-like disease found in cheetah
> 06/09/2007 - 17:52:28
>
> A cheetah at a zoo in Nuremberg has died after contracting an illness
> similar to mad cow disease, becoming the first confirmed case in Germany
of
> feline spongiform encephalopathy (FSE), city authorities said today.
>
> Lulu, a female cheetah born in 1998, had suffered for six weeks from
> problems that included trouble balancing and weakness in her hind legs,
the
> Nuremberg city government said in a statement.
>
> The animal eventually was put to sleep, and tests by Bavarian and federal
> labs were positive for FSE, it added.
>
> It was unclear how and when Lulu became infected with the disease, which
has
> a several-year incubation period, but Nuremberg authorities said it likely
> happened in the Netherlands, where she was born.
>
> Lulu moved to Germany at the age of 15 months, returned to the Netherlands
> five years later and arrived at the Nuremberg zoo in March 2006.
>
>
> http://www.breakingnews.ie/world/mhcwgbqlkfcw/
>
>
>
http://www.eveningecho.ie/news/bstory.asp?j=230658672&p=z3x659378&n=230659432
>
>
>
http://www.live-pr.com/en/bse-like-disease-detected-in-german-zoo-s-r1048136284.htm


gotta go............terry

----- Original Message -----
From: "Terry S. Singeltary Sr."
To:
Cc: ; ;
; ; ;
; ;
; ;
; ; ;
;
Sent: Thursday, August 16, 2007 8:30 PM
Subject: Pathobiology and diagnosis of animal transmissible spongiform
encephalopathies: current knowledge, research gaps, and opportunities


> Hello CWD Stakeholder Advisory Group,
>
> A kind greetings from Bacliff, Texas!
>
> i have wasted some decade it seems on human and animal transmissible
> spongiform encephalopathies of humans and animals.
>
> before your upcoming meeting, i urge you to read some data on CWD, you may
> not have been aware of.
>
> I wish to send you the following data on CWD. Please use as you wish. i
say
> now, and i say again almost a decade later,
> cwd is but a small part, of a much larger problem, one that is not going
> away anytime soon. ...
>
>
> ........good luck!
>
>
> kindest regards,
> terry
>
>
> ----- Original Message -----
> From: "Terry S. Singeltary Sr."
> To:
> Sent: Saturday, August 11, 2007 3:36 PM
> Subject: [BSE-L] Pathobiology and diagnosis of animal transmissible
> spongiform encephalopathies: current knowledge, research gaps, and
> opportunities
>
>
> Research Project: Transmission, Differentiation, and Pathobiology of
> Transmissible Spongiform Encephalopathies
> Location: Virus and Prion Diseases of Livestock
>
> Title: Pathobiology and diagnosis of animal transmissible spongiform
> encephalopathies: current knowledge, research gaps, and opportunities
>
>
> Authors
>
> Kehrli, Marcus
> O`rourke, Katherine
> Hamir, Amirali
> Richt, Juergen
> Nicholson, Eric
> Silva, Christopher
> Edelman, Daniel - FOOD AND DRUG ADMINISTRAT
> Gay, Cyril
>
>
> Submitted to: Government Publication/Report
> Publication Type: Government Publication
> Publication Acceptance Date: May 1, 2007
> Publication Date: July 1, 2007
>
> Citation: Kehrli, Jr., M.E., O'Rourke, K.I., Hamir, A.N., Richt, J.A.,
> Nicholson, E.M., Silva, C.J., Edelman, D., Gay, C.G. 2007.
>
>
> Pathobiology and diagnosis of animal transmissible spongiform
> encephalopathies: current knowledge, research gaps, and opportunities
> [government white paper]. Beltsville, MD: Interagency Working Group on
Prion
> Science, Subcommittee on Pathobiology and Diagnostics. USDA, Agriculture
> Research Service. 33 p.
>
> Technical Abstract:
>
>
> Transmissible spongiform encephalopathies (TSEs) are fatal neurologic
> diseases that can affect several animal species and human beings. There
are
> four animal TSE agents found in the United States: scrapie of sheep and
> goats; chronic wasting disease (CWD) of deer, elk, and moose;
transmissible
> mink encephalopathy (TME) and bovine spongiform encephalopathy (BSE).
> Although the animal TSEs do not cause major death losses among US
livestock
> populations, they are important because of international trade issues. The
> experience of the United Kingdom and Europe in dealing with the vast
> majority of the world's BSE cases, serves as a reminder of the need for
> continuing vigilance in monitoring risks for public health and research to
> answer remaining questions around the pathogenesis and transmission of
these
> diseases. There remain questions on 1) cross-species transmissibility of
> TSEs in livestock and wildlife; 2) the pathobiology of TSEs in natural and
> secondary hosts; pathogenesis and transmission of CWD; and 4) pathogenesis
> and ante mortem detection of typical and atypical BSE. Our understanding
of
> the pathogenesis and transmission of these diseases continues to evolve as
> ongoing, global TSE research efforts focus on defining tissue sites of
> abnormal prion accumulation, routes of infection, methods of strain
> differentiation, genetics of susceptibility and ante-mortem diagnostics.
In
> this paper, a Subcommittee on Pathobiology and Diagnostics of TSEs for an
> Interagency Working Group on Prion Science summarizes the science of
animal
> TSEs in order to identify knowledge gaps for the purpose of prioritizing
> animal prion research needs. Because of substantial losses involving
> international trade and potential risk for interspecies transmission to
> susceptible livestock and possibly humans, the presence of BSE, CWD,
scrapie
> and TME in the United States presents a liability to U.S. domestic and
> alternative livestock industries. In addition, the proven risk of BSE to
> agriculture and public health from subclinical or clinically sick animals
> requires science-based surveillance for any silent, unrecognized epizootic
> expansions of these diseases in populations of animals that could either
> directly or indirectly affect food animals. CWD is an example of an
> uncontrolled expanding epidemic that threatens not only cervids but
possibly
> other livestock. CWD also has elicited public health surveillance programs
> to monitor for scientific evidence of a prion disease in humans that
consume
> venison. Therefore, some of the research needs are precautionary, but the
> risks to animal and human health from being caught unaware are high.
Efforts
> are being made by both federal and state regulatory agencies to eradicate
> scrapie and CWD, and to determine the prevalence of BSE. The effectiveness
> of these programs will depend heavily on having accurate information about
> the nature of these diseases, not only in the original hosts, but also in
> other species that may be in contact with infected animals.
>
>
>
http://arsserv0.tamu.edu/research/publications/Publications.htm?seq_no_115=212488
>
>
> Summary of Selected Disease Events January—March 2007
>
>
> Scrapie: Nor98-like—Wyoming On March 16, 2007, the USDA Animal and Plant
> Health Inspection Service (APHIS) notified stakeholders that an aged
female
> sheep had tested positive for scrapie, consistent with the Nor98 type. The
> ewe was slaughtered in Michigan, where it was tested as part of USDA’s
> ongoing regulatory scrapie slaughter surveillance program. The ewe was
> traced back to a flock in Wyoming. This is the first time this particular
> type of scrapie has been found in the United States. The Nor98 type of
> scrapie is uncommon even in Europe, with fewer than 300 similar cases
> diagnosed since it was identified in 1998.
>
>
>
http://www.aphis.usda.gov/vs/ceah/cei/taf/iw_2007_files/Summary/quarterly_report_qtr_1_07.pdf
>
>
>
>
> ORAL-04
> EPIDEMIOLOGY OF CHRONIC WASTING DISEASE IN NORTH AMERICAN CERVIDS
> M. W. Miller
> Colorado Division of Wildlife, Wildlife Research Center, Fort Collins,
> Colorado, USA.
> Chronic wasting disease (CWD) occurs naturally in North American deer
> (Odocoileus spp.), wapiti,
> and moose (collectively called “cervids”). CWD presently occurs in
scattered
> foci throughout North
> America, both in the wild and in commercial facilities. CWD is contagious
> among its natural hosts,
> and epidemics can persist under both captive and free-ranging conditions,
> resulting in remarkably
> high infection rates. The precise mechanism of contagion remains unclear,
> although accumulations of
> disease-associated prion protein (PrPCWD) in lymphatic tissues associated
> with the gastrointestinal
> tract suggest shedding via feces and perhaps saliva. Analyses of epidemic
> data suggest that indirect
> (animal-environment-animal) transmission may be the dominant force in
> epidemic dynamics, and the
> CWD agent has been shown to persist in environments contaminated by
excreta
> or carcass remains
> for years. Variation in cellular prion protein appears to influence CWD
> pathogenesis, and may provide
> a biological mechanism for emergence of variant strains within and among
the
> four naturally
> susceptible species. The long-term implications of CWD for public,
> livestock, and wildlife health
> remain uncertain. Unfortunately, limitations of existing technology
> available to combat prion diseases
> make control of CWD ineffective or infeasible under most conditions.
> 23
>
>
> ORAL-19
> IINTERSPECIES PRION TRANSMISSION IS CONTROLLED BY CONFORMATIONAL
> COMPATIBILITY BETWEEN PRPSC AND HETEROTYPIC PRPC
> K.M. Green*1, S.R. Browning*1,6, T.S. Seward2, M. Green4, E.A. Hoover5,
G.C.
> Telling1,2,3,7
> 1Department of Microbiology, Immunology and Molecular Genetics, 2Sanders
> Brown Center on
> Aging,3Department of Neurology, 4UK Transgenic Facility University of
> Kentucky, Lexington, Ky USA.
> 5Department of Microbiology, Immunology and Pathology, Colorado State
> University, Fort Collins, Co, USA
> 6Present address: Department of Infectology, Scripps Research Institute,
> Jupiter, Florida, USA
> 7To whom correspondence should be addressed:gtell2@uky.edu
> * These authors contributed equally
> The threats to humans and livestock from interspecies prion transmission
are
> difficult to assess
> because the factors controlling this process remain uncertain. To address
> this we have used
> transgenic mouse models to understand the roles played by PrP primary
> structure, prion strains and
> the species specificity of protein X in controlling interspecies prion
> infection in the context of cervid
> transmission barriers. Cervid prions are of particular concern because
> chronic wasting disease
> (CWD) of North American and South Korean cervids is the only recognized
> prion disease of wild
> animals and its increasing geographic range, contagious nature, and
> environmental persistence have
> raised concerns about prion dissemination and the potential for further
> interspecies transmission. We
> show that conformational compatibility of PrPSc in a prion strain and PrP
> primary structure in a new
> host is the most important determinant of interspecies prion transmission
> barriers. Although prion
> strains can acquire totally new host range properties following
heterologous
> conversion of PrPP
> C in a
> new host, the strain-related biochemical properties of PrPSc may remain
> relatively stable. We also
> show that the cervid PrP polymorphism at residue 132, which is equivalent
to
> the human PrP 129
> polymorphism, is a crucial determinant of cervid prion transmission and
has
> a profound controlling
> effect on PrPSc-related prion strain properties. Our transgenic approaches
> modeling trans-species
> prion susceptibility in cervids also speak to the possible origins of CWD
> since cervid transgenic mice
> are also vulnerable, to varying degrees, to sheep scrapie prions, the
degree
> of susceptibility being
> strain related. One particularly well-characterized sheep scrapie isolate,
> SSBP/1, caused disease as
> efficiently as CWD prions from diseased deer or elk. Finally, while
> transmissions in transgenic mice
> based on the protein X model of prion propagation produced chimeric
prions,
> passage of which
> resulted in novel cervid prions with an extended host range compared to
> CWD-cervid prions, the
> unexpected susceptibilities of such mice to CWD and mouse prions are
> inconsistent with the
> previously hypothesized role of protein X in prion propagation.
>
>
> GEN-13
> PRION PROTEIN GENES AFFECT SUSCEPTIBILITY OF CERVIDS TO CHRONIC WASTING
> DISEASE
> C. Johnson1, J. Johnson1, J.P. Vanderloo1, D. Keane2, P. Bochsler2, J.M.
> Aiken1, D. McKenzie1
> 1Department of Animal Health and Biomedical Sciences and 2Wisconsin
> Veterinary Diagnostic Laboratory,
> University of Wisconsin, Madison, WI, USA mckenzie@svm.vetmed.wisc.edu
> The primary sequence of the prion protein affects susceptibility to
> transmissible spongiform
> encephalopathies (TSE; prion disease) in mice, sheep and humans. The Prnp
> sequence of freeranging,
> Wisconsin white-tailed deer was determined and the Prnp genotypes of
> CWD-positive and -
> negative deer compared. Six amino acid (AA) changes were identified; two
of
> which were located in
> pseudogenes. Two alleles, a glutamine to lysine polymorphism at codon 226
> and a single
> octapeptide repeat insertion into the pseudogene, have not been previously
> reported. The
> predominant alleles, wild-type (glutamine at AA95, glycine at AA96 and
> glutamine at AA226) and a
> glycine to serine polymorphism at AA96 (G96S), comprise almost 98% of the
> Prnp alleles in the
> Wisconsin white-tailed deer population. Comparison of the allelic
> frequencies in the CWD-positive
> and -negative deer suggests that G96S and a glutamine to histidine
> polymorphism at AA 95 (Q95H)
> are linked to a reduced susceptibility to CWD. The G96S allele does not,
> however, provide complete
> resistance, as a CWD-positive G96S/G96S deer was identified. The G96S
allele
> is also linked to
> slower progression of disease in CWD-positive deer based on the deposition
> of PrPCWD in the obex
> region of the medulla oblongata. To further determine the effect of
> variations of the cervid Prnp alleles
> on susceptibility, deer with known Prnp genotypes were orally dosed with
CWD
> inocula prepared from
> wild-type/wild-type homozygous animals. The experimentally infected
> wild-type/wild-type animals
> have succumbed to disease, animals heterozygous for Prnp alleles have not.
>
>
>
> PA-03
> PRIONS IN SKELETEL MUSCLE OF CWD INFECTED DEER
> R.C. Angers*1, S.R. Browning*1,6, T.S. Seward2, C.J. Sigurdson4, 7, M.W.
> Miller5, E.A. Hoover4, G.C. Telling1,
> 2, 3, 8
> 1Department of Microbiology, Immunology and Molecular Genetics, University
> of Kentucky, Lexington, KY 40536, 2Sanders
> Brown Center on Aging, University of Kentucky, 3Department of Neurology,
> University of Kentucky, 4Department of
> Microbiology, Immunology and Pathology, Colorado State University,
5Colorado
> Division of Wildlife, Wildlife Research Center,
> Fort Collins, CO 80526; 6 Present address: Department of Infectology,
> Scripps Research Institute, 5353 Parkside Drive, RF-2,
> Jupiter, Florida, 33458; 7 Present address: Institute of Neuropathology,
> University of Zurich, Schmelzbergstr 12, 8091 Zurich,
> Switzerland; 8 To whom correspondence should be addressed: e-mail: gtell2@
> uky.edu; * These authors contributed equally to
> this work
> The zoonotic potential of chronic wasting disease (CWD) has become a
public
> health concern since the
> transmission of bovine spongiform encephalopathy (BSE) prions to humans
> resulting in variant Creutzfeldt-
> Jakob disease (vCJD). Studies in mice, sheep and humans indicated that
PrPSc
> could be detected in the skeletal
> muscles. Since the most probable route of human exposure to CWD is through
> consumption or handling of
> meat from infected animals, it is important to assess whether skeletal
> muscle from affected cervids harbors
> prions. CWD-susceptible Tg(CerPrP) mice were intracranially inoculated
with
> brain and matched skeletal
> muscle homogenates from moribund as well as non-infected control deer. Tg
> mice inoculated with either brain
> or muscle homogenates from CWD-infected deer developed clinical illness
with
> characteristic prion disease
> symptoms and the brains of recipients accumulated cervid PrPSc. The mean
> incubation times for animals
> inoculated with brain material ranged between 231 and 283 days, whereas
mice
> receiving muscle tissue had
> average incubation periods between 360 and 492 days. Tg mice inoculated
with
> material from CWD-negative
> deer did not develop prion disease or accumulate PrPSc. Brain and muscle
> samples used to inoculate Tg(CerPrP)
> mice were analyzed for the presence of PrPSc. Brain samples producing the
> shortest incubation times had levels
> of PrPP
> Sc detectable by Western blotting in 25 µg total protein, whereas PrPSc
> P was detectable only after sodium
> phosphotungstate (NaPTA) precipitation of 0.5 mg for isolates with the
> longest incubation periods. No
> protease-resistant material was detected in muscle when 50 mg total
protein
> was precipitated with NaPTA and
> analyzed by Western blot. Although a possible role of prion strain
> variability cannot currently be dismissed,
> these results suggest variable prion titers in the CNS and skeletal muscle
> from different CWD-infected deer in
> the same phase of disease.
>
>
>
>
> ***PLEASE SEE FULL TEXT 234 PAGES *** ;
>
>
>
> http://www.neuroprion.com/pdf_docs/conferences/prion2006/abstract_book.pdf
>
>
>
> sporadic cjd
>
>
> http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&P=25276
>
>
> http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276
>
>
> Colorado Surveillance Program for Chronic Wasting Disease
> Transmission to Humans (TWO SUSPECT CASES, see GSS)
>
> http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0704&L=sanet-mg&T=0&P=1165
>
>
>
>
> 1
> © SEAC 2007
> NINETY SEVENTH MEETING OF THE SPONGIFORM
> ENCEPHALOPATHY ADVISORY COMMITTEE
>
> The Spongiform Encephalopathy Advisory Committee held its 97th
> meeting in London on 10th May 2007, and discussed the following:
> CURRENT ISSUES
>
> SEAC was informed about the following issues, both of which were
> discussed later in the agenda:
>
> • United Kingdom Chief Dental Officers recently issued
> guidance to dentists to limit certain dental instruments to
> single use as a precautionary measure to reduce the potential
> risk of variant Creutzfeldt-Jakob Disease (CJD) transmission1.
> • The first unusual case of Bovine Spongiform Encephalopathy
> (BSE) in the UK.
> ATYPICAL SCRAPIE CASE AUDIT
> SEAC was updated on investigations of an atypical scrapie case in
> a sheep flock previously considered free of transmissible
> spongiform encephalopathies (TSEs). An independent audit of the
> processes used to manage the flock found no evidence of errors in
> the collection of the samples for testing, or any major breaches in
> biosecurity in the importation and subsequent management of the
> flock. SEAC concluded that there was insufficient evidence to
> determine the origin of the case and that it was unlikely the origin
> would ever be unambiguously determined. There was no
> evidence for importation of the disease from New Zealand,
> although this could not be definitively excluded. It was noted that
> an investigation, by the Veterinary Laboratories Agency, of the
> 1
>
>
http://www.gnn.gov.uk/environment/fullDetail.asp?ReleaseID=279256&NewsAreaID=2&NavigatedFromDepartment=False
>
> 2
> © SEAC 2007
> flock had not identified other confirmed atypical scrapie cases,
> although further analyses are continuing.
>
> ATYPICAL SCRAPIE RESEARCH CONTINGENCY PLANNING
>
> To inform contingency planning, the Food Standards Agency
> (FSA) asked SEAC to consider possible scenarios, which might
> arise from research underway on atypical scrapie, and how these
> might change the understanding of the risk to human health.
> SEAC stated it was difficult to assess changes to understanding of
> the risk in the absence of hard scientific data, and that no one data
> set on its own was likely to be definitive. Studies comparing the
> properties of atypical scrapie and other TSE agents using the
> same animal model, especially humanised mice or non-human
> primates, would be most informative in the short term.
> Surveillance data to assess any association between CJDs and
> atypical scrapie prevalence would be most persuasive but are
> unlikely to become available in the short term. In assessing the
> risk it would be important to consider all the information available,
> rather than data from single studies considered in isolation.
>
> FATEPRIDE
>
> SEAC was updated about FATEPriDE, a recently completed multicentre
> European Union funded project, examining the possible
> influence of environmental trace elements on the occurrence of
> TSEs. The committee noted that interactions between trace
> elements and other environmental factors and the occurrence of
> TSEs are likely to be complex. No link between the vast majority
> of environmental factors studied, including organophosphates, and
> TSEs was found. The only environmental factor found to influence
> TSEs was manganese, at a molecular and cellular level. It is
> possible, but hard to prove in the field, that environmental
> manganese may influence susceptibility to, or spread of, some
> TSEs, possibly by increasing retention in the soil.
> UPDATE ON PREVALENCE STUDIES
> SEAC was updated about progress on approaches to obtain better
> estimates of the prevalence of subclinical vCJD:
> 3
> © SEAC 2007
> • The National Anonymous Tonsil Archive was on track to
> report to the SEAC Epidemiology Subgroup in June 2007 the
> analysis of tonsils collected to date from the age group likely
> to be at most risk of BSE infection.
> • A report from an expert group considering the feasibility of
> collecting tissues from autopsies for testing would be
> published in the near future. The recommendations of the
> report would then be considered by the Department of Health
> (DH).
> • Tenders received from manufacturers of prototype blood tests
> for the analysis of a large number of anonymous blood
> samples would be considered in late May 2007 by an expert
> group convened by the HPA.
>
> FUTURE ASSESSMENT OF LIKELY vCJD INFECTIVITY
> ASSOCIATED WITH PLASMA PRODUCTS
>
> SEAC agreed to a DH request to reassess, at SEAC 98, the likely
> removal of infectivity from blood contaminated with the vCJD agent
> during plasma product production. In addition, to estimate the
> timing of the time window for possible infections arising from use of
> plasma products prior to the sourcing plasma from countries
> considered free from BSE.
>
> UNUSUAL CASES OF SPONGIFORM ENCEPHALOPATHY IN
> CATTLE
>
> SEAC received presentations from international experts on
> unusual cases of BSE. This item was discussed in the reserved
> business session as unpublished research was considered, which
> is consistent with the SEAC Code of Practice.
> SEAC noted such cases are very rare. They had been identified
> predominantly by active surveillance and predominantly in older
> animals in a number of European countries and elsewhere. It now
> appears that the cases can be categorised into two types, H-type
> or L-type, on the basis of their biochemical characteristics which
> differ from those of classical BSE. The neuropathological and
> transmission properties of unusual BSE types also differed from
> classical BSE. It is not yet clear whether or not conversion to
> classical BSE may occur on secondary transmissions. The origin
> 4
> © SEAC 2007
> of unusual BSE is not clear and it might possibly be spontaneous.
> SEAC agreed that, given current knowledge, procedures appeared
> to be in place to prevent an epidemic of unusual BSE. Further
> data, particularly on tissue distribution and transmissibility via the
> oral route, is needed. SEAC agreed to publish a position
> statement.
>
> UPDATE ON DENTAL RESEARCH
>
> SEAC was updated on preliminary findings from research to
> estimate the potential risk of vCJD transmission via dental
> procedures. This item was discussed in the reserved business
> session as unpublished research was considered, which is
> consistent with the SEAC Code of Practice.
> SEAC agreed that the data and conclusions appeared robust,
> while recognising they are preliminary data from an animal model
> and that the studies are not yet complete. The research
> suggested that there may be a significant risk that vCJD can be
> transmitted via some dental procedures in man, although the level
> of risk remains difficult to quantify. The committee welcomed the
> Chief Dental Officers’ recent guidance to dentists who were
> advised to ensure that all dental files and reamers were treated as
> single use, but sought reassurance that compliance in both private
> and NHS practice would be closely monitored. SEAC considered
> it important that a comprehensive assessment of the potential risks
> of vCJD transmission from all dental procedures be conducted as
> a priority. This could allow possible additional precautionary
> measures to be identified. SEAC agreed to publish a position
> statement in the near future.
>
> http://www.seac.gov.uk/summaries/seac97_summary.pdf
>
> SUMMARY
>
> http://www.seac.gov.uk/minutes/97.pdf
>
>
> MAY SCRAPIE REPORT 2007 (atypical NOR-98 like disease documented Wyoming,
> USA)
>
>
http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps
>
>
> Atypical scrapie in a swiss goat and implications for transmissible
> spongiform encephalopathy surveillance
> Torsten Seuberlich1, Catherine Botteron, Sylvie L. Benestad, Hervé
> Brünisholz, Reto Wyss, Ulrich Kihm, Heinzpeter Schwermer, Martina Friess,
> Alexandra Nicolier, Dagmar Heim and Andreas Zurbriggen
> Correspondence: 1 Corresponding Author: Torsten Seuberlich, NeuroCenter,
> Department of Clinical Veterinary Medicine, Vetsuisse Faculty,
> Bremgartenstrasse 109a, CH-3001 Berne, Switzerland, e-mail:
> torsten.seuberlich@itn.unibe.ch
>
>
> Different types of transmissible spongiform encephalopathies (TSEs) affect
> sheep and goats. In addition to the classical form of scrapie, both
species
> are susceptible to experimental infections with the bovine spongiform
> encephalopathy (BSE) agent, and in recent years atypical scrapie cases
have
> been reported in sheep from different European countries. Atypical scrapie
> in sheep is characterized by distinct histopathologic lesions and
molecular
> characteristics of the abnormal scrapie prion protein (PrPsc).
> Characteristics of atypical scrapie have not yet been described in detail
in
> goats. A goat presenting features of atypical scrapie was identified in
> Switzerland. Although there was no difference between the molecular
> characteristics of PrPsc in this animal and those of atypical scrapie in
> sheep, differences in the distribution of histopathologic lesions and
PrPsc
> deposition were observed. In particular the cerebellar cortex, a major
site
> of PrPsc deposition in atypical scrapie in sheep, was found to be
virtually
> unaffected in this goat. In contrast, severe lesions and PrPsc deposition
> were detected in more rostral brain structures, such as thalamus and
> midbrain. Two TSE screening tests and PrPsc immunohistochemistry were
either
> negative or barely positive when applied to cerebellum and obex tissues,
the
> target samples for TSE surveillance in sheep and goats. These findings
> suggest that such cases may have been missed in the past and could be
> overlooked in the future if sampling and testing procedures are not
adapted.
> The epidemiological and veterinary public health implications of these
> atypical cases, however, are not yet known.
>
>
> http://www.jvdi.org/cgi/content/abstract/19/1/2
>
>
> 1: Vet J. 2007 Jun 14; [Epub ahead of print]Lack of PrP(sc) immunostaining
> in intracranial ectopic lymphoid follicles in a sheep with concomitant
> non-suppurative encephalitis and Nor98-like atypical scrapie: A case
> report.Vidal E, Tortosa R, Costa C, Benavides J, Francino O,
Sánchez-Robert
> E, Pérez V, Pumarola M.
> Priocat Laboratory, CReSA, Autonomous University of Barcelona, 08193
> Bellaterra (Cerdanyola del Vallès), Spain.
>
> During active surveillance for transmissible spongiform encephalopathies
> (TSEs) in sheep, an initial reactor was detected using a rapid test on a
> brain sample. Immunohistochemistry confirmed an atypical TSE presentation
> that closely resembled the previously described Nor98 cases. Sequencing of
> the prnp gene confirmed the ARQ/AHQ genotype with the L141F mutation at
> codon 141 associated with this phenotype. The head, including the brain
and
> cranial lymphoid tissues, was sampled and examined thoroughly.
Non-purulent
> encephalitis, with ectopic lymphoid follicle formation within the brain,
was
> diagnosed concomitant to the TSE. When scrapie-associated prion protein
> (PrP(sc)) deposition was studied by immunohistochemistry there was a
> noticeable lack of lymphotropism. The distribution of PrP(sc) in the brain
> differed considerably from that of classical scrapie cases. Astrogliosis
and
> microgliosis were demonstrated by histochemical procedures.
>
>
http://www.ncbi.nlm.nih.gov/sites/entrez?Db=pubmed&Cmd=ShowDetailView&TermToSearch=17574883&ordinalpos=1&itool=EntrezSystem2.PEntrez.Pubmed.Pubmed_ResultsPanel.Pubmed_RVDocSum
>
>
>
> Published online before print October 20, 2005
>
> Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102
> Medical Sciences
>
> A newly identified type of scrapie agent can naturally infect sheep with
> resistant PrP genotypes
>
> ( sheep prion | transgenic mice )
>
> Annick Le Dur *, Vincent Béringue *, Olivier Andréoletti , Fabienne Reine
*,
> Thanh Lan Laï *, Thierry Baron , Bjørn Bratberg ¶, Jean-Luc Vilotte ||,
> Pierre Sarradin **, Sylvie L. Benestad ¶, and Hubert Laude *
> *Virologie Immunologie Moléculaires and ||Génétique Biochimique et
> Cytogénétique, Institut National de la Recherche Agronomique, 78350
> Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de la
> Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse,
Interactions
> Hôte Agent Pathogène, 31066 Toulouse, France; Agence Française de Sécurité
> Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels,
> 69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut
> National de la Recherche Agronomique, 37380 Nouzilly, France; and
> ¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway
>
>
> Edited by Stanley B. Prusiner, University of California, San Francisco,
CA,
> and approved September 12, 2005 (received for review March 21, 2005)
>
> Scrapie in small ruminants belongs to transmissible spongiform
> encephalopathies (TSEs), or prion diseases, a family of fatal
> neurodegenerative disorders that affect humans and animals and can
transmit
> within and between species by ingestion or inoculation. Conversion of the
> host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a
> misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission
> and pathogenesis. The intensified surveillance of scrapie in the European
> Union, together with the improvement of PrPSc detection techniques, has
led
> to the discovery of a growing number of so-called atypical scrapie cases.
> These include clinical Nor98 cases first identified in Norwegian sheep on
> the basis of unusual pathological and PrPSc molecular features and "cases"
> that produced discordant responses in the rapid tests currently applied to
> the large-scale random screening of slaughtered or fallen animals.
> Worryingly, a substantial proportion of such cases involved sheep with PrP
> genotypes known until now to confer natural resistance to conventional
> scrapie. Here we report that both Nor98 and discordant cases, including
> three sheep homozygous for the resistant PrPARR allele (A136R154R171),
> efficiently transmitted the disease to transgenic mice expressing ovine
PrP,
> and that they shared unique biological and biochemical features upon
> propagation in mice. These observations support the view that a truly
> infectious TSE agent, unrecognized until recently, infects sheep and goat
> flocks and may have important implications in terms of scrapie control and
> public health.
>
>
> --------------------------------------------------------------------------
--
> ----
>
> Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R.,
> T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B.
> contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed
data;
> and H.L. wrote the paper.
>
> A.L.D. and V.B. contributed equally to this work.
>
> To whom correspondence should be addressed.
>
> Hubert Laude, E-mail: laude@jouy.inra.fr
>
> www.pnas.org/cgi/doi/10.1073/pnas.0502296102
>
>
> http://www.pnas.org/cgi/content/abstract/0502296102v1
>
>
>
> An evaluation of scrapie surveillance in the United States
> From: Terry S. Singeltary Sr.
> Date: Sun, 5 Aug 2007 13:05
>
> http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=3427
>
>
> SEAC New forms of Bovine Spongiform Encephalopathy 1 August 2007
> From: Terry S. Singeltary Sr.
> Date: Sun, 5 Aug 2007 13:09:38 -0500
>
> http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=3573
>
>
> FSE: FIRST CONFIRMED CASE REPORTED IN PORTUGAL AND POTENTIAL MAD CAT
ESCAPES
> LAB IN USA (204 lines)
> From: Terry S. Singeltary Sr. <[log in to unmask]>
> Date: Thu, 9 Aug 2007 16:58:42 -0500
>
>
> http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=7062
>
>
>
>
> Owens, Julie
> From: Terry S. Singeltary Sr. [flounder9@verizon.net]
> Sent: Monday, July 24, 2006 1:09 PM
> To: FSIS RegulationsComments
> Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of
Bovine
> Spongiform Encephalopathy (BSE)
> Page 1 of 98
> 8/3/2006
>
> Greetings FSIS,
>
> I would kindly like to comment on the following ;
>
> [Federal Register: July 12, 2006 (Volume 71, Number 133)]
> [Notices]
> [Page 39282-39283]
> From the Federal Register Online via GPO Access [wais.access.gpo.gov]
> [DOCID:fr12jy06-35]
> -----------------------------------------------------------------------
> DEPARTMENT OF AGRICULTURE
> Food Safety and Inspection Service
> [Docket No. FSIS-2006-0011]
> Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
> Update; Notice of Availability and Technical Meeting
>
>
> snip...
>
>
>
http://a257.g.akamaitech.net/7/257/2422/01jan20061800/edocket.access.gpo.gov/2006/E6-10928.htm
>
>
> MY comments/questions are as follows ;
>
> 1. SINCE the first Harvard BSE Risk Assessment was so flawed and fraught
> with error after the PEER REVIEW
> assessment assessed this fact, how do you plan on stopping this from
> happening again, will there be another peer
> review with top TSE Scientist, an impartial jury so-to-speak, to assess
this
> new and updated Harvard BSE/TSE risk
> assessment and will this assessment include the Atypical TSE and SRM
issues
> ?
>
>
> *** Suppressed peer review of Harvard study October 31, 2002 ***
>
> http://www.fsis.usda.gov/oa/topics/BSE_Peer_Review.pdf
>
>
> 2. WITH A RECENT NATION WIDE MAD COW FEED BAN RECALL in the past few
months
> that consisted of
> some 10,878.06 TONS, then another Mad Cow feed ban warning letter in May,
IT
> should seem prudent to ask why our
> feed bans continue to fail in 2006, and continue to fail today ?
>
>
> snip...
>
> full text 98 pages ;
>
> 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
>
> 03-025IFA
> 03-025IFA-2
> Terry S. Singeltary
>
>
> Page 1 of 17
>
> From: Terry S. Singeltary Sr. [flounder9@verizon.net]
>
> Sent: Thursday, September 08, 2005 6:17 PM
>
> To: fsis.regulationscomments@fsis.usda.gov
>
> Subject: [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified
> Risk Materials for
> Human Food and Requirements for the Disposition of Non-Ambulatory Disabled
> Cattle
>
>
> Greetings FSIS,
>
> I would kindly like to submit the following to [Docket No. 03-025IFA] FSIS
> Prohibition of the Use of Specified
> Risk Materials for Human Food and Requirements for the Disposition of
> Non-Ambulatory Disabled Cattle
> THE BSE/TSE SUB CLINICAL Non-Ambulatory Disabled Cattle Broken bones and
> such may be the first
> signs of a sub clinical BSE/TSE Non-Ambulatory Disabled Cattle ;
>
> SUB CLINICAL PRION INFECTION
>
> MRC-43-00
>
> Issued: Monday, 28 August 2000
>
> NEW EVIDENCE OF SUB-CLINICAL PRION INFECTION: IMPORTANT RESEARCH
>
> FINDINGS RELEVANT TO CJD AND BSE
>
> A team of researchers led by Professor John Collinge at the Medical
>
> Research Council Prion Unit1 report today in the Proceedings of the
>
> National Academy of Sciences, on new evidence for the existence of a
>
> "sub-clinical" form of BSE in mice which was unknown until now....
>
> full text 17 pages ;
>
>
>
https://web01.aphis.usda.gov/regpublic.nsf/0/eff9eff1f7c5cf2b87256ecf000df08d?OpenDocument
>
>
> Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW BSE
> SAFEGUARDS
>
>
> Docket
>
> No. 04-047-l
>
> No. 04-021ANPR
>
> No. 2004N-0264
>
> NEW BSE SAFEGUARDS
>
> Federal Measures to Mitigate BSE Risks: Considerations for Further Action
>
> http://www.fda.gov/cvm/index/updates/bseanprm.htm
>
>
> Greetings FDA, USDA and APHIS et al,
>
> I would kindly like to comment on the continued delay of the regulations
> that have been proposed for years to reduce the risk of BSE/TSE in the
> USA. Each day that is wasted debating this issue allows this agent to
> spread,
> and many many more humans and animals become needlessly exposed to
> this agent via a multitude of potential routes and sources right here in
the
> USA. TO continue to ignore the new findings from several scientists
> about the fact that BSE is not the only strain of TSE in cattle, the fact
> that
> new atypical strains of TSE are showing up in not only cattle, but
> sheep and the fact that the new strain of TSE in cattle seems to be
> more similar to sporadic CJD as opposed to the nv/v CJD, to continue
> to ignore these findings will only further spread this agent.
..............
>
> full text ;
>
>
>
https://web01.aphis.usda.gov/regpublic.nsf/168556f5aa7a82ba85256ed00044eb1f/eff9eff1f7c5cf2b87256ecf000df08d
>
>
> Docket Management Docket: 02N-0273 - Substances Prohibited From Use in
>
> Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed
>
> Comment Number: EC -10
>
> Accepted - Volume 2
>
>
> http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be07.html
>
>
> PART 2
>
>
> http://www.fda.gov/ohrms/dockets/dailys/03/Jan03/012403/8004be09.html
>
>
> 2003D-0186
> Guidance for Industry: Use of Material From Deer and Elk In Animal Feed
>
>
> EMC 7
> Terry S. Singeltary Sr.
> Vol #:
> 1
>
>
> http://www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm
>
>
> Docket Management Docket: 02N-0276 - Bioterrorism Preparedness ...
> General Comments, Subject: Docket No: 02-088-1 RE-Agricultural ...
> From: Terry S. Singeltary Sr.
>
> To: regulations@aphis.usda.gov
>
> Docket No: 02-088-1 Title: ...
>
>
> Greetings FDA and public,
>
>
> if you go to the below site, and search all BSE known countries and check
> out their air traffic illegal meat they have confiscated, and check out
the
> low number checked, compared to actual passenger traffic, would not take
too
> much for some nut to bring in FMD/TSEs into the USA as a 'suitcase bomb'.
>
>
> [[Under APHIS-PPQ's agricultural quarantine inspection monitoring, 284 air
> passengers from Israel were sampled for items of agricultural interest in
> fiscal year 2001. Seven of these passengers, or 2 percent, carried a total
> of 11 kg of meat items that could potentially harbor the pathogen that
> causes BSE. None of these passengers from whom meat items were confiscated
> reported plans to visit or work on a ranch or farm during their visit to
the
> U.S.]]
>
>
> if they were to have questioned the terrorist that bombed the Twin Towers
> with jets, if they were to have questioned them at flight school in the
USA,
> i am sure that they would have said they did not intend to visit the Twin
> Towers as a flying bomb either. what am i thinking, they probably did ask
> this? stupid me. ...
>
>
> full text ;
>
>
> http://www.fda.gov/ohrms/DOCKETS/dockets/02n0276/02N-0276-EC-254.htm
>
>
> PDF]Freas, William TSS SUBMISSION
>
> File Format: PDF/Adobe Acrobat -
>
> Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary
>
> Sr. [flounder@wt.net] Monday, January 08, 200l 3:03 PM freas ...
>
>
> Greetings again Dr. Freas and Committee Members,
>
> I wish to submit the following information to the
> Scientific Advisors and Consultants Staff
> 2001 Advisory Committee (short version).
>
> I understand the reason of having to shorten my submission,
> but only hope that you add it to a copy of the long version,
> for members to take and read at their pleasure,
> (if cost is problem, bill me, address below).
> So when they realize some time in the near future
> of the 'real' risks i speak of from human/animal TSEs and
> blood/surgical products. I cannot explain the 'real' risk
> of this in 5 or 10 minutes at some meeting,
> or on 2 or 3 pages, but will attempt here:
>
> remember AIDS/HIV, 'no problem to heterosexuals in the U.S.?
> no need to go into that, you know of this blunder.
>
> DO NOT make these same stupid mistakes again with
> human/animal TSE's aka MADCOW DISEASE. I lost my Mom to hvCJD,
> and my neighbor lost his Mother to sCJD as well (both cases
> confirmed). I have seen many deaths, from many diseases.
> I have never seen anything as CJD, I still see my Mom laying helpless,
> jerking tremendously, and screaming "God, what's wrong
> with me, why can't I stop this". I still see this, and will
> never forget. Approximately 10 weeks from 1st of symptoms to death.
> This is what drives me. I have learned more in 3 years about not only
> human/animal TSE's but the cattle/rendering/feeding industry/government
> than i ever wished to.
>
> I think you are all aware of CJD vs vCJD, but i don't think
> you all know the facts of human/animal TSE's as a whole,
> they are all very very similar, and are all tied to the
> same thing, GREED and MAN.
>
> I am beginning to think that the endless attempt to track
> down and ban, potential victims from known BSE Countries
> from giving blood will be futile. You would have to ban
> everyone on the Globe eventually? AS well, I think we
> MUST ACT SWIFTLY to find blood test for TSE's,
> whether it be blood test, urine test, eyelid test,
> anything at whatever cost, we need a test FAST.
>
> DO NOT let the incubation time period of these TSEs fool you.............
>
>
> full text 6 pages ;
>
>
> http://www.fda.gov/ohrms/dockets/ac/01/slides/3681s2_09.pdf
>
>
> 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
>
>
> Diagnosis and Reporting of Creutzfeldt-Jakob Disease
>
> Singeltary, Sr et al. JAMA.2001; 285: 733-734.
>
>
http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535
>
>
> BRITISH MEDICAL JOURNAL
>
> BMJ
>
> http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
>
>
> BMJ
>
> http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
>
>
> 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:
>
> flounder@wt.net
>
> 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
>
>
> THE PATHOLOGICAL PROTEIN
> Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
>
> June 2003
>
> BY Philip Yam
>
>
> CHAPTER 14 LAYING ODDS
>
> Answering critics like Terry Singeltary, who feels that the U.S. under-
> counts CJD, Schonberger conceded that the current surveillance system
> has errors but stated that most of the errors will be confined to the
older
> population.
>
> http://www.thepathologicalprotein.com/
>
>
> doi:10.1016/S1473-3099(03)00715-1
> Copyright © 2003 Published by Elsevier Ltd.
> Newsdesk
>
> Tracking spongiform encephalopathies in North America
>
> Xavier Bosch
>
> Available online 29 July 2003.
>
>
> Volume 3, Issue 8, August 2003, Page 463
>
>
> “My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
> mom to hvCJD (Heidenhain variant CJD)
> and have been searching for answers ever since. What I have found is that
we
> have not been told the truth. CWD
> in deer and elk is a small portion of a much bigger problem.”
>
> ............................
>
>
>
http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext
>
>
http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf
>
>
>
> 1: J Infect Dis 1980 Aug;142(2):205-8
>
>
>
> Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to
> nonhuman primates.
>
> Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
>
> Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of sheep
> and goats were transmitted to squirrel monkeys (Saimiri sciureus) that
were
> exposed to the infectious agents only by their nonforced consumption of
> known infectious tissues. The asymptomatic incubation period in the one
> monkey exposed to the virus of kuru was 36 months; that in the two monkeys
> exposed to the virus of Creutzfeldt-Jakob disease was 23 and 27 months,
> respectively; and that in the two monkeys exposed to the virus of scrapie
> was 25 and 32 months, respectively. Careful physical examination of the
> buccal cavities of all of the monkeys failed to reveal signs or oral
> lesions. One additional monkey similarly exposed to kuru has remained
> asymptomatic during the 39 months that it has been under observation.
>
> PMID: 6997404
>
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
>
>
>
> look at the table and you'll see that as little as 1 mg (or 0.001 gm)
caused
> 7% (1 of 14) of the cows to come down with BSE;
>
> Risk of oral infection with bovine spongiform encephalopathy agent in
> primates
>
> Corinne Ida Lasmézas, Emmanuel Comoy, Stephen Hawkins, Christian Herzog,
> Franck Mouthon, Timm Konold, Frédéric Auvré, Evelyne Correia, Nathalie
> Lescoutra-Etchegaray, Nicole Salès, Gerald Wells, Paul Brown,
Jean-Philippe
> Deslys
> Summary The uncertain extent of human exposure to bovine spongiform
> encephalopathy (BSE)--which can lead to variant Creutzfeldt-Jakob disease
> (vCJD)--is compounded by incomplete knowledge about the efficiency of oral
> infection and the magnitude of any bovine-to-human biological barrier to
> transmission. We therefore investigated oral transmission of BSE to
> non-human primates. We gave two macaques a 5 g oral dose of brain
homogenate
> from a BSE-infected cow. One macaque developed vCJD-like neurological
> disease 60 months after exposure, whereas the other remained free of
disease
> at 76 months. On the basis of these findings and data from other studies,
we
> made a preliminary estimate of the food exposure risk for man, which
> provides additional assurance that existing public health measures can
> prevent transmission of BSE to man.
>
>
> snip...
>
>
> BSE bovine brain inoculum
>
> 100 g 10 g 5 g 1 g 100 mg 10 mg 1 mg 0·1 mg 0·01 mg
>
> Primate (oral route)* 1/2 (50%)
>
> Cattle (oral route)* 10/10 (100%) 7/9 (78%) 7/10 (70%) 3/15 (20%) 1/15
(7%)
> 1/15 (7%)
>
> RIII mice (ic ip route)* 17/18 (94%) 15/17 (88%) 1/14 (7%)
>
> PrPres biochemical detection
>
> The comparison is made on the basis of calibration of the bovine inoculum
> used in our study with primates against a bovine brain inoculum with a
> similar PrPres concentration that was
>
> inoculated into mice and cattle.8 *Data are number of animals
> positive/number of animals surviving at the time of clinical onset of
> disease in the first positive animal (%). The accuracy of
>
> bioassays is generally judged to be about plus or minus 1 log. ic
> ip=intracerebral and intraperitoneal.
>
> Table 1: Comparison of transmission rates in primates and cattle infected
> orally with similar BSE brain inocula
>
>
> Published online January 27, 2005
>
> http://www.thelancet.com/journal/journal.isa
>
>
> It is clear that the designing scientists must also have shared Mr Bradley
’s
> surprise at the results because all the dose levels right down to 1 gram
> triggered infection.
>
>
> http://www.bseinquiry.gov.uk/files/ws/s145d.pdf
>
>
>
>
> 2
>
> 6. It also appears to me that Mr Bradley’s answer (that it would take less
> than say 100
>
> grams) was probably given with the benefit of hindsight; particularly if
one
>
> considers that later in the same answer Mr Bradley expresses his surprise
> that it
>
> could take as little of 1 gram of brain to cause BSE by the oral route
> within the
>
> same species. This information did not become available until the "attack
> rate"
>
> experiment had been completed in 1995/96. This was a titration experiment
>
> designed to ascertain the infective dose. A range of dosages was used to
> ensure
>
> that the actual result was within both a lower and an upper limit within
the
> study
>
> and the designing scientists would not have expected all the dose levels
to
> trigger
>
> infection. The dose ranges chosen by the most informed scientists at that
> time
>
> ranged from 1 gram to three times one hundred grams. It is clear that the
> designing
>
> scientists must have also shared Mr Bradley’s surprise at the results
> because all the
>
> dose levels right down to 1 gram triggered infection.
>
>
> http://www.bseinquiry.gov.uk/files/ws/s147f.pdf
>
>
>
>
>
> 2) Infectious dose:
>
> To cattle: 1 gram of infected brain material (by oral ingestion)
>
>
> http://www.inspection.gc.ca/english/sci/bio/bseesbe.shtml
>
>
>
> SHORT COMMUNICATION
>
> Oral Transmission of Chronic Wasting Disease in Captive Shira’s Moose
>
> Terry J. Kreeger1,3, D. L. Montgomery2, Jean E. Jewell2, Will Schultz1 and
> Elizabeth S. Williams2
>
> 1 Wyoming Game and Fish Department, 2362 Highway 34, Wheatland, Wyoming
> 82201, USA;
> 2 Department of Veterinary Sciences, University of Wyoming, Laramie,
Wyoming
> 82071, USA
> 3 Corresponding author (email: tkreeger@wildblue.net )
>
> ABSTRACT: Three captive Shira’s moose (Alces alces shirasi) were orally
> inoculated with a single dose (5 g) of whole-brain homogenate prepared
from
> chronic wasting disease (CWD)–affected mule deer (Odocoileus hemionus).
All
> moose died of causes thought to be other than CWD. Histologic examination
of
> one female moose dying 465 days postinoculation revealed spongiform change
> in the neuropil, typical of transmissible spongiform encephalopathy.
> Immunohistochemistry staining for the proteinase-resistant isoform of the
> prion protein was observed in multiple lymphoid and nervous tissues.
Western
> blot and enzyme-linked immunosorbent assays provided additional
confirmation
> of CWD. These results represent the first report of experimental CWD in
> moose.
> Key words: Alces alces shirasi, chronic wasting disease, enzyme-linked
> immunosorbent assay, immunohistochemistry, moose, oral inoculation, prion,
> PrPCWD.
>
>
> http://www.jwildlifedis.org/cgi/content/abstract/42/3/640
>
>
>
>
> http://www.usaha.org/committees/reports/2005/report-wd-2005.pdf
>
>
>
> -------- Original Message --------
>
> Subject: DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material
> From Deer and Elk in Animal Feed; Availability
> Date: Fri, 16 May 2003 11:47:37 -0500
> From: "Terry S. Singeltary Sr."
> To: fdadockets@oc.fda.gov
>
>
>
> Greetings FDA,
>
> i would kindly like to comment on;
>
> Docket 03D-0186
>
> FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal
> Feed; Availability
>
> Several factors on this apparent voluntary proposal disturbs me greatly,
> please allow me to point them out;
>
> 1. MY first point is the failure of the partial ruminant-to-ruminant feed
> ban of 8/4/97. this partial and voluntary feed ban of some ruminant
> materials being fed back to cattle is terribly flawed. without the
> _total_ and _mandatory_ ban of all ruminant materials being fed
> back to ruminants including cattle, sheep, goat, deer, elk and mink,
> chickens, fish (all farmed animals for human/animal consumption),
> this half ass measure will fail terribly, as in the past decades...
>
> 2. WHAT about sub-clinical TSE in deer and elk? with the recent
> findings of deer fawns being infected with CWD, how many could
> possibly be sub-clinically infected. until we have a rapid TSE test to
> assure us that all deer/elk are free of disease (clinical and
sub-clinical),
> we must ban not only documented CWD infected deer/elk, but healthy
> ones as well. it this is not done, they system will fail...
>
> 3. WE must ban not only CNS (SRMs specified risk materials),
> but ALL tissues. recent new and old findings support infectivity
> in the rump or ass muscle. wether it be low or high, accumulation
> will play a crucial role in TSEs.
>
> 4. THERE are and have been for some time many TSEs in the
> USA. TME in mink, Scrapie in Sheep and Goats, and unidentified
> TSE in USA cattle. all this has been proven, but the TSE in USA
> cattle has been totally ignored for decades. i will document this
> data below in my references.
>
> 5. UNTIL we ban all ruminant by-products from being fed back
> to ALL ruminants, until we rapid TSE test (not only deer/elk) but
> cattle in sufficient numbers to find (1 million rapid TSE test in
> USA cattle annually for 5 years), any partial measures such as the
> ones proposed while ignoring sub-clinical TSEs and not rapid TSE
> testing cattle, not closing down feed mills that continue to violate the
> FDA's BSE feed regulation (21 CFR 589.2000) and not making
> freely available those violations, will only continue to spread these
> TSE mad cow agents in the USA. I am curious what we will
> call a phenotype in a species that is mixed with who knows
> how many strains of scrapie, who knows what strain or how many
> strains of TSE in USA cattle, and the CWD in deer and elk (no
> telling how many strains there), but all of this has been rendered
> for animal feeds in the USA for decades. it will get interesting once
> someone starts looking in all species, including humans here in the
> USA, but this has yet to happen...
>
> 6. IT is paramount that CJD be made reportable in every state
> (especially ''sporadic'' cjd), and that a CJD Questionnaire must
> be issued to every family of a victim of TSE. only checking death
> certificates will not be sufficient. this has been proven as well
> (see below HISTORY OF CJD -- CJD QUESTIONNAIRE)
>
> 7. WE must learn from our past mistakes, not continue to make
> the same mistakes...
>
> REFERENCES
>
>
> Oral transmission and early lymphoid tropism of chronic wasting disease
> PrPres in mule deer fawns (Odocoileus hemionus )
> Christina J. Sigurdson1, Elizabeth S. Williams2, Michael W. Miller3,
> Terry R. Spraker1,4, Katherine I. O'Rourke5 and Edward A. Hoover1
>
> Department of Pathology, College of Veterinary Medicine and Biomedical
> Sciences, Colorado State University, Fort Collins, CO 80523- 1671, USA1
> Department of Veterinary Sciences, University of Wyoming, 1174 Snowy
> Range Road, University of Wyoming, Laramie, WY 82070, USA 2
> Colorado Division of Wildlife, Wildlife Research Center, 317 West
> Prospect Road, Fort Collins, CO 80526-2097, USA3
> Colorado State University Veterinary Diagnostic Laboratory, 300 West
> Drake Road, Fort Collins, CO 80523-1671, USA4
> Animal Disease Research Unit, Agricultural Research Service, US
> Department of Agriculture, 337 Bustad Hall, Washington State University,
> Pullman, WA 99164-7030, USA5
>
> Author for correspondence: Edward Hoover.Fax +1 970 491 0523. e-mail
> ehoover@lamar.colostate.edu
>
> Mule deer fawns (Odocoileus hemionus) were inoculated orally with a
> brain homogenate prepared from mule deer with naturally occurring
> chronic wasting disease (CWD), a prion-induced transmissible spongiform
> encephalopathy. Fawns were necropsied and examined for PrP res, the
> abnormal prion protein isoform, at 10, 42, 53, 77, 78 and 80 days
> post-inoculation (p.i.) using an immunohistochemistry assay modified to
> enhance sensitivity. PrPres was detected in alimentary-tract-associated
> lymphoid tissues (one or more of the following: retropharyngeal lymph
> node, tonsil, Peyer's patch and ileocaecal lymph node) as early as 42
> days p.i. and in all fawns examined thereafter (53 to 80 days p.i.). No
> PrPres staining was detected in lymphoid tissue of three control fawns
> receiving a control brain inoculum, nor was PrPres detectable in neural
> tissue of any fawn. PrPres-specific staining was markedly enhanced by
> sequential tissue treatment with formic acid, proteinase K and hydrated
> autoclaving prior to immunohistochemical staining with monoclonal
> antibody F89/160.1.5. These results indicate that CWD PrP res can be
> detected in lymphoid tissues draining the alimentary tract within a few
> weeks after oral exposure to infectious prions and may reflect the
> initial pathway of CWD infection in deer. The rapid infection of deer
> fawns following exposure by the most plausible natural route is
> consistent with the efficient horizontal transmission of CWD in nature
> and enables accelerated studies of transmission and pathogenesis in the
> native species.
>
> snip...
>
> These results indicate that mule deer fawns develop detectable PrP res
> after oral exposure to an inoculum containing CWD prions. In the
> earliest post-exposure period, CWD PrPres was traced to the lymphoid
> tissues draining the oral and intestinal mucosa (i.e. the
> retropharyngeal lymph nodes, tonsil, ileal Peyer's patches and
> ileocaecal lymph nodes), which probably received the highest initial
> exposure to the inoculum. Hadlow et al. (1982) demonstrated scrapie
> agent in the tonsil, retropharyngeal and mesenteric lymph nodes, ileum
> and spleen in a 10-month-old naturally infected lamb by mouse bioassay.
> Eight of nine sheep had infectivity in the retropharyngeal lymph node.
> He concluded that the tissue distribution suggested primary infection
> via the gastrointestinal tract. The tissue distribution of PrPres in the
> early stages of infection in the fawns is strikingly similar to that
> seen in naturally infected sheep with scrapie. These findings support
> oral exposure as a natural route of CWD infection in deer and support
> oral inoculation as a reasonable exposure route for experimental studies
> of CWD.
>
> snip...
>
> http://vir.sgmjournals.org/cgi/content/full/80/10/2757
>
>
>
> Subject: MAD DEER/ELK DISEASE AND POTENTIAL SOURCES
> Date: Sat, 25 May 2002 18:41:46 -0700
> From: "Terry S. Singeltary Sr."
> Reply-To: BSE-L
> To: BSE-L
>
> 8420-20.5% Antler Developer
> For Deer and Game in the wild
> Guaranteed Analysis Ingredients / Products Feeding Directions
>
> snip...
>
> _animal protein_
>
> http://www.surefed.com/deer.htm
>
> BODE'S GAME FEED SUPPLEMENT #400
> A RATION FOR DEER
> NET WEIGHT 50 POUNDS
> 22.6 KG.
>
> snip...
>
> _animal protein_
>
> http://www.bodefeed.com/prod7.htm
>
> Ingredients
>
> Grain Products, Plant Protein Products, Processed Grain By-Products,
> Forage Products, Roughage Products 15%, Molasses Products,
> __Animal Protein Products__,
> Monocalcium Phosphate, Dicalcium Pyosphate, Salt,
> Calcium Carbonate, Vitamin A Acetate with D-activated Animal Sterol
> (source of Vitamin D3), Vitamin E Supplement, Vitamin B12 Supplement,
> Riboflavin Supplement, Niacin Supplement, Calcium Panothenate, Choline
> Chloride, Folic Acid, Menadione Soduim Bisulfite Complex, Pyridoxine
> Hydorchloride, Thiamine Mononitrate, d-Biotin, Manganous Oxide, Zinc
> Oxide, Ferrous Carbonate, Calcium Iodate, Cobalt Carbonate, Dried
> Sacchoromyces Berevisiae Fermentation Solubles, Cellulose gum,
> Artificial Flavors added.
>
> http://www.bodefeed.com/prod6.htm
> ===================================
>
> MORE ANIMAL PROTEIN PRODUCTS FOR DEER
>
> Bode's #1 Game Pellets
> A RATION FOR DEER
> F3153
>
> GUARANTEED ANALYSIS
> Crude Protein (Min) 16%
> Crude Fat (Min) 2.0%
> Crude Fiber (Max) 19%
> Calcium (Ca) (Min) 1.25%
> Calcium (Ca) (Max) 1.75%
> Phosphorus (P) (Min) 1.0%
> Salt (Min) .30%
> Salt (Max) .70%
>
>
> Ingredients
>
> Grain Products, Plant Protein Products, Processed Grain By-Products,
> Forage Products, Roughage Products, 15% Molasses Products,
> __Animal Protein Products__,
> Monocalcium Phosphate, Dicalcium Phosphate, Salt,
> Calcium Carbonate, Vitamin A Acetate with D-activated Animal Sterol
> (source of Vitamin D3) Vitamin E Supplement, Vitamin B12 Supplement,
> Roboflavin Supplement, Niacin Supplement, Calcium Pantothenate, Choline
> Chloride, Folic Acid, Menadione Sodium Bisulfite Complex, Pyridoxine
> Hydrochloride, Thiamine Mononitrate, e - Biotin, Manganous Oxide, Zinc
> Oxide, Ferrous Carbonate, Calcium Iodate, Cobalt Carbonate, Dried
> Saccharyomyces Cerevisiae Fermentation Solubles, Cellulose gum,
> Artificial Flavors added.
>
> FEEDING DIRECTIONS
> Feed as Creep Feed with Normal Diet
>
> http://www.bodefeed.com/prod8.htm
>
> INGREDIENTS
>
> Grain Products, Roughage Products (not more than 35%), Processed Grain
> By-Products, Plant Protein Products, Forage Products,
> __Animal Protein Products__,
> L-Lysine, Calcium Carbonate, Salt, Monocalcium/Dicalcium
> Phosphate, Yeast Culture, Magnesium Oxide, Cobalt Carbonate, Basic
> Copper Chloride, Manganese Sulfate, Manganous Oxide, Sodium Selenite,
> Zinc Sulfate, Zinc Oxide, Sodium Selenite, Potassium Iodide,
> Ethylenediamine Dihydriodide, Vitamin E Supplement, Vitamin A
> Supplement, Vitamin D3 Supplement, Mineral Oil, Mold Inhibitor, Calcium
> Lignin Sulfonate, Vitamin B12 Supplement, Menadione Sodium Bisulfite
> Complex, Calcium Pantothenate, Riboflavin, Niacin, Biotin, Folic Acid,
> Pyridoxine Hydrochloride, Mineral Oil, Chromium Tripicolinate
>
> DIRECTIONS FOR USE
>
> Deer Builder Pellets is designed to be fed to deer under range
> conditions or deer that require higher levels of protein. Feed to deer
> during gestation, fawning, lactation, antler growth and pre-rut, all
> phases which require a higher level of nutrition. Provide adequate
> amounts of good quality roughage and fresh water at all times.
>
>
http://www.profilenutrition.com/Products/Specialty/deer_builder_pellets.html
> ===================================================
>
> DEPARTMENT OF HEALTH & HUMAN SERVICES
> PUBLIC HEALTH SERVICE
> FOOD AND DRUG ADMINISTRATION
>
> April 9, 2001 WARNING LETTER
>
> 01-PHI-12
> CERTIFIED MAIL
> RETURN RECEIPT REQUESTED
>
> Brian J. Raymond, Owner
> Sandy Lake Mills
> 26 Mill Street
> P.O. Box 117
> Sandy Lake, PA 16145
> PHILADELPHIA DISTRICT
>
> Tel: 215-597-4390
>
> Dear Mr. Raymond:
>
> Food and Drug Administration Investigator Gregory E. Beichner conducted
> an inspection of your animal feed manufacturing operation, located in
> Sandy Lake, Pennsylvania, on March 23,
> 2001, and determined that your firm manufactures animal feeds including
> feeds containing prohibited materials. The inspection found significant
> deviations from the requirements set forth in
> Title 21, code of Federal Regulations, part 589.2000 - Animal Proteins
> Prohibited in Ruminant Feed. The regulation is intended to prevent the
> establishment and amplification of Bovine Spongiform Encephalopathy
> (BSE) . Such deviations cause products being manufactured at this
> facility to be misbranded within the meaning of Section 403(f), of the
> Federal Food, Drug, and Cosmetic
> Act (the Act).
>
> Our investigation found failure to label your
> swine feed with the required cautionary statement "Do Not Feed to cattle
> or other Ruminants" The FDA suggests that the statement be
> distinguished
> by different type-size or color or other means of highlighting the
> statement so that it is easily noticed by a purchaser.
>
> In addition, we note that you are using approximately 140 pounds of
> cracked corn to flush your mixer used in the manufacture of animal
> feeds containing prohibited material. This
> flushed material is fed to wild game including deer, a ruminant animal.
> Feed material which may potentially contain prohibited material should
> not be fed to ruminant animals which may become part of the food chain.
>
> The above is not intended to be an all-inclusive list of deviations from
> the regulations. As a manufacturer of materials intended for animal
> feed use, you are responsible for assuring that your overall operation
> and the products you manufacture and distribute are in compliance with
> the law. We have enclosed a copy of FDA's Small Entity Compliance Guide
> to assist you with complying with the regulation... blah, blah, blah...tss
>
> http://www.fda.gov/foi/warning_letters/g1115d.pdf
>
>
> SNIP...FULL TEXT ;
>
>
> http://www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm
>
>
> TONS Products manufactured from 02/01/2005 until 06/06/2006
> Date: August 6, 2006 at 6:16 pm PST
> PRODUCT
> a) CO-OP 32% Sinking Catfish, Recall # V-100-6;
> b) Performance Sheep Pell W/Decox/A/N, medicated,
> net wt. 50 lbs, Recall # V-101-6;
> c) Pro 40% Swine Conc Meal -- 50 lb, Recall # V-102-6;
> d) CO-OP 32% Sinking Catfish Food Medicated,
> Recall # V-103-6;
>
> *********************************
>
> e) "Big Jim's" BBB Deer Ration, Big Buck Blend,
> Recall # V-104-6;
>
> *********************************
>
> f) CO-OP 40% Hog Supplement Medicated Pelleted,
> Tylosin 100 grams/ton, 50 lb. bag, Recall # V-105-6;
> g) Pig Starter Pell II, 18% W/MCDX Medicated 282020,
> Carbadox -- 0.0055%, Recall # V-106-6;
> h) CO-OP STARTER-GROWER CRUMBLES, Complete
> Feed for Chickens from Hatch to 20 Weeks, Medicated,
> Bacitracin Methylene Disalicylate, 25 and 50 Lbs,
> Recall # V-107-6;
> i) CO-OP LAYING PELLETS, Complete Feed for Laying
> Chickens, Recall # 108-6;
> j) CO-OP LAYING CRUMBLES, Recall # V-109-6;
> k) CO-OP QUAIL FLIGHT CONDITIONER MEDICATED,
> net wt 50 Lbs, Recall # V-110-6;
> l) CO-OP QUAIL STARTER MEDICATED, Net Wt. 50 Lbs,
> Recall # V-111-6;
> m) CO-OP QUAIL GROWER MEDICATED, 50 Lbs,
> Recall # V-112-6
> CODE
> Product manufactured from 02/01/2005 until 06/06/2006
> RECALLING FIRM/MANUFACTURER
> Alabama Farmers Cooperative, Inc., Decatur, AL, by telephone, fax, email
and
> visit on June 9, 2006. FDA initiated recall is complete.
> REASON
> Animal and fish feeds which were possibly contaminated with ruminant based
> protein not labeled as "Do not feed to ruminants".
> VOLUME OF PRODUCT IN COMMERCE
> 125 tons
> DISTRIBUTION
> AL and FL
>
>
> END OF ENFORCEMENT REPORT FOR AUGUST 2, 2006
>
> ###
>
>
> http://www.fda.gov/bbs/topics/enforce/2006/ENF00963.html
>
> snip...end...tss
>
>
>
> NOW, please note what the FDA claims was a safe level ;
>
>
>
> FDA has determined that each animal could have consumed, at most and in
> total, five-and-one-half grams - approximately a quarter ounce -- of
> prohibited material. These animals weigh approximately 600 pounds.
>
> http://www.fda.gov/bbs/topics/NEWS/2001/NEW00752.html
>
>
>
> thats enough to expose and kill 100 cows.....tss
>
>
>
> see history of cjd questionnaire
>
> http://brain.hastypastry.net/forums/showthread.php?t=2408
>
>
>
> Posted July 20, 2007
>
> Hassett resigns DNR post; Doyle picks Frank for job
> By Todd Richmond
> Associated Press writer
> MADISON — After years of battling chronic wasting disease, navigating
> partisan politics and trying to win back outdoorsmen’s trust, Wisconsin
> Department of
> Natural Resources Secretary Scott Hassett has resigned.
> http://www.rhinelanderdailynews.com/articles/2007/07/21/news/news02.txt
>
> ==========
>
>
> cant blame the head of wisconsin DNR. look what happened to the funding
for
> CWD. perhaps this person saw the writing on the wall. ...tss
>
>
>
> wdin@usgs.gov
>
>
> Greetings,
>
> IN reply to ;
>
> Cuts would reduce CWD budget by 60 percent
>
> Published Monday, July 23, 2007 11:59:09 AM Central Time
> By Lee Fahrney Times Outdoors Writer
>
>
> http://www.themonroetimes.com/m0723cwd.htm
>
>
>
> THIS disturbs me greatly, and should disturb every hunter in Wisconsin,
and
> neighboring states. With CWD spreading like it is, we should not be
> cutting any funding for CWD by 60 %. It makes no sense, with CWD
amplifying
> and spreading still.
>
>
> 193 CONFIRMED POSITIVE CWD samples from 19,953 samples collected last time
> in Wisconsin, and they want to reduce funding by 60% for CWD management
> activities ???
>
> this is not logical ???
>
> do they now think that CWD is under control in Wisconsin ???
>
> do they think that CWD is now NOT a potential threat to animal and or
human
> health, and or the environment ???
>
> what is the logistics behind this decision ???
>
>
> 2006-2007 Harvest and CWD Testing
> CWD Public Dialogue to Begin
>
> Preliminary numbers show that during the
> 2006-07 deer seasons, 56,593 deer were
> harvested from the CWD zones. This is a
> decline of more than 15 percent from the 2005-
> 06 season. Antlerless harvest accounts for
> this drop, having decreased 31 percent from
> last year. Buck harvest, however, increased
> by five percent.
> Of the 19,953 samples collected last
> season, 193 tested CWD-positive. Thirty-five
> of these deer were harvested in the herd
> reduction zone. The rest were harvested in
> the disease eradication zones.
> This brings the total number of free ranging
> CWD-positive deer to 844 for all years. Fiftyfour
> of these deer were harvested in the herd
> reduction zone.
> As part of the effort to confirm that CWD
> has not spread out of the CWD zones of southern
> Wisconsin, 9,308 samples were also
> collected in west-central Wisconsin and areas
> of south-central and south-east Wisconsin not
> in a CWD zone. None of these samples tested
> positive for the disease.
>
>
> http://dnr.wi.gov/org/land/wildlife/whealth/issues/cwd/doc/07_05_news.pdf
>
>
> CWD Update 87
>
> July 6, 2007
>
> State and Provincial Updates
>
> Illinois:
>
> Paul Shelton, Illinois Department of Natural Resources provides the
> following: Illinois
>
>
> Department of Natural Resources staff collected 6,733 usable CWD
> surveillance samples during the period beginning July 1, 2006.
> A few additional samples from suspect deer remain untested at this time.
> Testing yielded 41 CWD-positive deer and 6,692 ‘not detected’.
> Forty-one positives were detected in four counties in northern Illinois:
> Winnebago (18), Boone (13), DeKalb (6), and McHenry (4). No
> new counties were identified as having CWD, and no disease was detected in
> Ogle County, in which CWD was first identified during the
> 2005-2006 sampling season. However, CWD was identified in southeastern
> DeKalb County, more than 20 miles southeast of
> previously-identified locations. Samples were collected from a variety of
> sources, including deer check stations in high-risk counties (3,097),
> sample drop-off locations for archery hunters (175), cooperating meat
> processors (1,778), suspect deer (25), roadkills in CWD counties
> (16), and culling efforts (1,642). Cooperating meat lockers were added as
a
> sampling source this year to provide a statewide sampling base
> outside the identified CWD area. Hunter-harvested deer accounted for 17
> (41%) of the positives identified, with the remainder coming from
> suspect deer (4; 10%); roadkills (1; 2%); and sharpshooting (19; 46%).
> Illinois DNR CWD information is available at:
>
> http://dnr.state.il.us/cwd.
>
>
> http://wildlifedisease.nbii.gov/documents/CWD%20Updates/Update%2087.pdf
>
>
>
> Title: Susceptibility of cattle to first-passage intracerebral inoculation
> with chronic wasting disease agent from white-tailed deer
>
>
>
http://www.ars.usda.gov/research/publications/Publications.htm?seq_no_115=194089
>
>
>
> Title: Transmission of chronic wasting disease of mule deer to Suffolk
sheep
> following intracerebral inoculation
>
> Submitted to: Journal of Veterinary Diagnostic Investigation
> Publication Type: Peer Reviewed Journal
> Publication Acceptance Date: June 20, 2006
> Publication Date: November 1, 2006
> Citation: Hamir, A.N., Kunkle, R.A., Cutlip, R.C., Miller, J.M., Williams,
> E.S., Richt, J.A. 2006. Transmission of chronic wasting disease of mule
deer
> to Suffolk sheep following intracerebral inoculation. Journal of
Veterinary
> Diagnostic Investigation. 18(6):558-565.
>
> Interpretive Summary: Chronic wasting disease (CWD) has been identified in
> captive and free ranging deer and elk since 1967. To determine the
> transmissibility of CWD to sheep and to provide information about the
> disease and tests for detection of CWD in sheep, 8 lambs were inoculated
> with brain suspension from mule deer naturally affected with CWD. Two
other
> lambs were kept as controls. Only 1 sheep developed clinical disease at 35
> months after inoculation. The study was terminated at 72 months after the
> inoculation. At that time one other sheep was found to be positive for the
> disease. It is proposed that the host's genetic makeup may play a role in
> transmission of the disease to domestic sheep. Impact. This is the first
> study which shows that it is possible to transmit CWD to a small number of
> sheep.
> Technical Abstract
>
>
> snip...end
>
>
http://www.ars.usda.gov/research/publications/publications.htm?SEQ_NO_115=191571
>
>
>
> 0022-538X/05/$08.00+0 doi:10.1128/JVI.79.21.13794-13796.2005
> Copyright © 2005, American Society for Microbiology. All Rights Reserved.
>
> Interspecies Transmission of Chronic Wasting Disease Prions to Squirrel
> Monkeys (Saimiri sciureus)
> Richard F. Marsh,1, Anthony E. Kincaid,2 Richard A. Bessen,3 and Jason C.
> Bartz4*
> Department of Animal Health and Biomedical Sciences, University of
> Wisconsin, Madison 53706,1 Department of Physical Therapy,2 Department of
> Medical Microbiology and Immunology, Creighton University, Omaha, Nebraska
> 68178,4 Department of Veterinary Molecular Biology, Montana State
> University, Bozeman, Montana 597183
>
> Received 3 May 2005/ Accepted 10 August 2005
>
> Chronic wasting disease (CWD) is an emerging prion disease of deer and
elk.
> The risk of CWD transmission to humans following exposure to CWD-infected
> tissues is unknown. To assess the susceptibility of nonhuman primates to
> CWD, two squirrel monkeys were inoculated with brain tissue from a
> CWD-infected mule deer. The CWD-inoculated squirrel monkeys developed a
> progressive neurodegenerative disease and were euthanized at 31 and 34
> months postinfection. Brain tissue from the CWD-infected squirrel monkeys
> contained the abnormal isoform of the prion protein, PrP-res, and
displayed
> spongiform degeneration. This is the first reported transmission of CWD to
> primates.
>
>
> --------------------------------------------------------------------------
--
> ----
>
> * Corresponding author. Mailing address: Department of Medical
Microbiology
> and Immunology, Creighton University, 2500 California Plaza, Omaha, NE
> 68178. Phone: (402) 280-1811. Fax: (402) 280-1875. E-mail:
> jbartz@creighton.edu .
>
> Deceased.
>
>
> --------------------------------------------------------------------------
--
> ----
>
> Journal of Virology, November 2005, p. 13794-13796, Vol. 79, No. 21
> 0022-538X/05/$08.00+0 doi:10.1128/JVI.79.21.13794-13796.2005
> Copyright © 2005, American Society for Microbiology. All Rights Reserved.
>
>
>
http://jvi.asm.org/cgi/content/abstract/79/21/13794?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=cwd&searchid=1129736446553_4280&stored_search=&FIRSTINDEX=0&volume=79&issue=21&journalcode=jvi
>
>
>
> Chronic Wasting Disease and Potential Transmission to Humans
> Ermias D. Belay,* Ryan A. Maddox,* Elizabeth S. Williams,† Michael W.
> Miller,‡ Pierluigi Gambetti,§ and Lawrence B. Schonberger*
> *Centers for Disease Control and Prevention, Atlanta, Georgia, USA;
> †University of Wyoming, Laramie, Wyoming, USA; ‡Colorado Division of
> Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve
University,
> Cleveland, Ohio, USA
>
> Suggested citation for this article: Belay ED, Maddox RA, Williams ES,
> Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and
potential
> transmission to humans. Emerg Infect Dis [serial on the Internet]. 2004
Jun
> [date cited]. Available from:
> http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm
>
>
> --------------------------------------------------------------------------
--
> ----
>
> Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner
> area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been
> detected in other parts of the United States. Although detection in some
> areas may be related to increased surveillance, introduction of CWD due to
> translocation or natural migration of animals may account for some new
foci
> of infection. Increasing spread of CWD has raised concerns about the
> potential for increasing human exposure to the CWD agent. The foodborne
> transmission of bovine spongiform encephalopathy to humans indicates that
> the species barrier may not completely protect humans from animal prion
> diseases. Conversion of human prion protein by CWD-associated prions has
> been demonstrated in an in vitro cell-free experiment, but limited
> investigations have not identified strong evidence for CWD transmission to
> humans. More epidemiologic and laboratory studies are needed to monitor
the
> possibility of such transmissions.
>
>
> http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm
>
>
>
> May 2007
>
> NIAID Research on Prion Diseases
> OVERVIEW
>
> snip...
>
> More research is necessary to determine whether CWD poses any risk to
> humans, particularly because it is spreading over a wider geographical
area
> in the United States. There have been several reported cases of CJD in
> individuals who have consumed venison, most much younger than the typical
> age associated with CJD. In each of these instances, careful
investigations
> by CDC have shown no causal link between CJD and CWD in deer and elk
> populations. Continued surveillance is important, however, to assess any
> possible risk of CWD transmission to humans.
>
>
>
> http://www.niaid.nih.gov/factsheets/priondis.htm
>
>
>
> > In each of these instances, careful investigations by CDC have shown no
> _causal_ link between CJD and CWD in deer and elk populations.
>
>
> WRONG !
>
> BELAY ET AL STATES ;
>
> ''Our conclusion stating that we found no _strong_ evidence of CWD
> transmission to humans''
>
>
> THERE'S a big difference between ''casual'' and ''strong''. ...TSS
>
>
> Colorado Surveillance Program for Chronic Wasting Disease
> Transmission to Humans (TWO SUSPECT CASES)
>
>
>
> From: TSS (216-119-163-189.ipset45.wt.net)
> Subject: CWD aka MAD DEER/ELK TO HUMANS ???
> Date: September 30, 2002 at 7:06 am PST
>
> From: "Belay, Ermias"
> To:
> Cc: "Race, Richard (NIH)" ; ; "Belay,
> Ermias"
> Sent: Monday, September 30, 2002 9:22 AM
> Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE DEATHS - CWD - YOUNG HUNTERS
>
> Dear Sir/Madam,
> In the Archives of Neurology you quoted (the abstract of which was
> attached to your email), we did not say CWD in humans will present like
> variant CJD.
>
> That assumption would be wrong. I encourage you to read the whole
> article and call me if you have questions or need more clarification
> (phone: 404-639-3091). Also, we do not claim that "no-one has ever been
> infected with prion disease from eating venison." Our conclusion stating
> that we found no strong evidence of CWD transmission to humans in the
> article you quoted or in any other forum is limited to the patients we
> investigated.
>
> Ermias Belay, M.D.
> Centers for Disease Control and Prevention
>
>
> full text ;
>
> http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0704&L=sanet-mg&T=0&P=1165
>
>
> 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
>
>
> Diagnosis and Reporting of Creutzfeldt-Jakob Disease
>
> Singeltary, Sr et al. JAMA.2001; 285: 733-734.
>
>
http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535
>
>
> BRITISH MEDICAL JOURNAL
>
> BMJ
>
> http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
>
>
> BMJ
>
> http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
>
>
> 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:
>
> flounder@wt.net
>
> 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
>
>
> doi:10.1016/S1473-3099(03)00715-1
> Copyright © 2003 Published by Elsevier Ltd.
> Newsdesk
>
> Tracking spongiform encephalopathies in North America
>
> Xavier Bosch
>
> Available online 29 July 2003.
>
>
> Volume 3, Issue 8, August 2003, Page 463
>
>
> “My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
> mom to hvCJD (Heidenhain variant CJD)
> and have been searching for answers ever since. What I have found is that
we
> have not been told the truth. CWD
> in deer and elk is a small portion of a much bigger problem.”
> ............................
>
>
>
http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext
>
>
http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf
>
>
>
> thank you,
>
> kindest regards,
>
>
> Terry S. Singeltary Sr.
> P.O. Box 42
> Bacliff, Texas USA 77518
>
> Sent: Monday, September 10, 2007 3:59 PM
> Subject: re-Pathobiology and diagnosis of animal transmissible spongiform encephalopathies: current knowledge, research gaps, and opportunities


> Greetings again CWD working group.
>
> I thought I might forward this study by Aguzzi et al on TSE. some may have
> it, but for those that don't, it is a good synopsis of the overall picture.
> use as you wish. I am not here as an anti-hunter, anti-meat, extremist. I am
> a meat eater. I am here to hopefully shed a bit of light on the topic, from
> my 10 years daily of researching and staying on top of this the best I can,
> as a lay person, thrown into the world of TSE through unforeseen
> circumstances i.e. my mom dod 12-14-97 hvCJD 'confirmed'. I just would not
> except ''oh, it just happens 1 in a million, sporadically spontaneously,
> without cause. that only the UK people dieing from only UK mad cow, were
> dieing from new variant creutzfeldt jakob disease, only in one geographical
> part of the world. nope, did not buy it then, do not buy it now. now think
> about this too. all those farmers with BSE herds that died from SPORADIC
> CJD, and A WIFE, all died of sporadic cjd, and had BSE herds??? something is
> not right with the diagnostic criteria, of diagnosing and differentiating
> between the different strains. the UKBSEnvCJD only theory is bogus, and
> always has been;
>
> see history of cjd questionnaire
>
> http://brain.hastypastry.net/forums/showthread.php?t=2408
>
>
> sporadic CJD, the big lie
>
> see full text ;
>
>
> http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276
>
>

snip......end


> greetings again,
>
>
> i forgot to pass this along. i wrote it in 2000. the mbm recipe in the USA
> is unique, it has not only BSE, BASE, multiple strains of scrapie typical
> and atypical, but also CWD, maybe even a few TME cases from what small
> number of mink are rendered (it does happen), but it's a smorgasbord or
> petri dish for TSE, and our rendering techniques mirrored that of the UK, in
> fact we sent them the technology that brewed these TSE up, through the feed
> mode of transmission, and deer, elk, and i think moose readily contract CWD
> via the oral route. you ever read what some of these folks feed there
> cervids. i wrote about that too many moons ago. but not only commingling at
> feed sites increases risk i.e. environmental, casual contact nose to nose,
> but also the feed itself. ...
>
>
>
> U.S. Scientist should be concerned with a CJD epidemic in the U.S., as
> well... 2 January 2000
>
> Terry S Singeltary
> retired
> Send response to journal:
> Re: U.S. Scientist should be concerned with a CJD epidemic in the U.S., as
> well...
>
>
>
> In reading your short article about 'Scientist warn of CJD epidemic' news
> in brief Jan. 1, 2000. I find the findings in the PNAS old news, made famous
> again. Why is the U.S. still sitting on their butts, ignoring the facts? We
> have the beginning of a CJD epidemic in the U.S., and the U.S. Gov. is doing
> everything in it's power to conceal it.
>
> The exact same recipe for B.S.E. existed in the U.S. for years and years. In
> reading over the Qualitative Analysis of BSE Risk Factors-1, this is a 25
> page report by the USDA:APHIS:VS. It could have been done in one page. The
> first page, fourth paragraph says it all;
>
> "Similarities exist in the two countries usage of continuous rendering
> technology and the lack of usage of solvents, however, large differences
> still remain with other risk factors which greatly reduce the potential risk
> at the national level."
>
> Then, the next 24 pages tries to down-play the high risks of B.S.E. in the
> U.S., with nothing more than the cattle to sheep ratio count, and the
> geographical locations of herds and flocks. That's all the evidence they can
> come up with, in the next 24 pages.
>
> Something else I find odd, page 16;
>
> "In the United Kingdom there is much concern for a specific continuous
> rendering technology which uses lower temperatures and accounts for 25
> percent of total output. This technology was _originally_ designed and
> imported from the United States. However, the specific application in the
> production process is _believed_ to be different in the two countries."
>
> A few more factors to consider, page 15;
>
> "Figure 26 compares animal protein production for the two countries. The
> calculations are based on slaughter numbers, fallen stock estimates, and
> product yield coefficients. This approach is used due to variation of up to
> 80 percent from different reported sources. At 3.6 million tons, the United
> States produces 8 times more animal rendered product than the United
> Kingdom."
>
> "The risk of introducing the BSE agent through sheep meat and bone meal is
> more acute in both relative and absolute terms in the United Kingdom
> (Figures 27 and 28). Note that sheep meat and bone meal accounts for 14
> percent, or 61 thousand tons, in the United Kingdom versus 0.6 percent or 22
> thousand tons in the United States. For sheep greater than 1 year, this is
> less than one-tenth of one percent of the United States supply."
>
> "The potential risk of amplification of the BSE agent through cattle meat
> and bone meal is much greater in the United States where it accounts for 59
> percent of total product or almost 5 times more than the total amount of
> rendered product in the United Kingdom."
>
> Considering, it would only take _one_ scrapie infected sheep to contaminate
> the feed. Considering Scrapie has run rampant in the U.S. for years, as of
> Aug. 1999, 950 scrapie infected flocks. Also, Considering only one quarter
> spoonful of scrapie infected material is lethal to a cow. Considering all
> this, the sheep to cow ration is meaningless. As I said, it's 24 pages of
> B.S.e.
>
> To be continued...
>
> Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA
>
>
>
> http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
>
>
>
> -------- Original Message --------
> Subject: DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material
> From Deer and Elk in Animal Feed; Availability
> Date: Fri, 16 May 2003 11:47:37 -0500
> From: "Terry S. Singeltary Sr."
> To: fdadockets@oc.fda.gov
>
>
> Greetings FDA,
>
> i would kindly like to comment on;
>
> Docket 03D-0186
>
> FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal
> Feed; Availability
>
> Several factors on this apparent voluntary proposal disturbs me greatly,
> please allow me to point them out;
>
> 1. MY first point is the failure of the partial ruminant-to-ruminant feed
> ban of 8/4/97. this partial and voluntary feed ban of some ruminant
> materials being fed back to cattle is terribly flawed. without the
> _total_ and _mandatory_ ban of all ruminant materials being fed
> back to ruminants including cattle, sheep, goat, deer, elk and mink,
> chickens, fish (all farmed animals for human/animal consumption),
> this half ass measure will fail terribly, as in the past decades...
>
> 2. WHAT about sub-clinical TSE in deer and elk? with the recent
> findings of deer fawns being infected with CWD, how many could
> possibly be sub-clinically infected. until we have a rapid TSE test to
> assure us that all deer/elk are free of disease (clinical and sub-clinical),
> we must ban not only documented CWD infected deer/elk, but healthy
> ones as well. it this is not done, they system will fail...
>
> 3. WE must ban not only CNS (SRMs specified risk materials),
> but ALL tissues. recent new and old findings support infectivity
> in the rump or ass muscle. wether it be low or high, accumulation
> will play a crucial role in TSEs.
>
> 4. THERE are and have been for some time many TSEs in the
> USA. TME in mink, Scrapie in Sheep and Goats, and unidentified
> TSE in USA cattle. all this has been proven, but the TSE in USA
> cattle has been totally ignored for decades. i will document this
> data below in my references.
>
> 5. UNTIL we ban all ruminant by-products from being fed back
> to ALL ruminants, until we rapid TSE test (not only deer/elk) but
> cattle in sufficient numbers to find (1 million rapid TSE test in
> USA cattle annually for 5 years), any partial measures such as the
> ones proposed while ignoring sub-clinical TSEs and not rapid TSE
> testing cattle, not closing down feed mills that continue to violate the
> FDA's BSE feed regulation (21 CFR 589.2000) and not making
> freely available those violations, will only continue to spread these
> TSE mad cow agents in the USA. I am curious what we will
> call a phenotype in a species that is mixed with who knows
> how many strains of scrapie, who knows what strain or how many
> strains of TSE in USA cattle, and the CWD in deer and elk (no
> telling how many strains there), but all of this has been rendered
> for animal feeds in the USA for decades. it will get interesting once
> someone starts looking in all species, including humans here in the
> USA, but this has yet to happen...
>
> 6. IT is paramount that CJD be made reportable in every state
> (especially ''sporadic'' cjd), and that a CJD Questionnaire must
> be issued to every family of a victim of TSE. only checking death
> certificates will not be sufficient. this has been proven as well
> (see below HISTORY OF CJD -- CJD QUESTIONNAIRE)
>
> 7. WE must learn from our past mistakes, not continue to make
> the same mistakes...
>
> REFERENCES
>
> Six white-tailed deer fawns test positive for CWD
>
> MADISON -- Six fawns in the area of south central Wisconsin where
> chronic wasting disease has been found in white-tailed deer have tested
> positive for the disease, according to Department of Natural Resources
> wildlife health officials. These are the youngest wild white-tailed deer
> detected with chronic wasting disease (CWD) to date.
>
> Approximately 4,200 fawns, defined as deer under 1 year of age, were
> sampled from the eradication zone over the last year. The majority of
> fawns sampled were between the ages of 5 to 9 months, though some were
> as young as 1 month. Two of the six fawns with CWD detected were 5 to 6
> months old. All six of the positive fawns were taken from the core area
> of the CWD eradication zone where the highest numbers of positive deer
> have been identified.
>
> snip...
>
> http://www.dnr.state.wi.us/org/caer/ce/news/on/2003/on20030513.htm#art4
>
> ===================================================
>
> Issued: Monday, 28 August 2000
> NEW EVIDENCE OF SUB-CLINICAL PRION INFECTION: IMPORTANT RESEARCH
> FINDINGS RELEVANT TO CJD AND BSE
>
> A team of researchers led by Professor John Collinge at the Medical
> Research Council Prion Unit1 report today in the Proceedings of the
> National Academy of Sciences, on new evidence for the existence of a
> 'sub-clinical' form of BSE in mice which was unknown until now.
>
> The scientists took a closer look at what is known as the 'species
> barrier' - the main protective factor which limits the ability of
> prions2 to jump from one species to infect another. They found the mice
> had a 'sub-clinical' form of disease where they carried high levels of
> infectivity but did not develop the clinical disease during their normal
> lifespan. The idea that individuals can carry a disease and show no
> clinical symptoms is not new. It is commonly seen in conventional
> infectious diseases.
>
> Researchers tried to infect laboratory mice with hamster prions3 called
> Sc237 and found that the mice showed no apparent signs of disease.
> However, on closer inspection they found that the mice had high levels
> of mouse prions in their brains. This was surprising because it has
> always been assumed that hamster prions could not cause the disease in
> mice, even when injected directly into the brain.
>
> In addition the researchers showed that this new sub-clinical infection
> could be easily passed on when injected into healthy mice and hamsters.
>
> The height of the species barrier varies widely between different
> combinations of animals and also varies with the type or strain of
> prions. While some barriers are quite small (for instance BSE easily
> infects mice), other combinations of strain and species show a seemingly
> impenetrable barrier. Traditionally, the particular barrier studied here
> was assumed to be robust.
>
> Professor John Collinge said: "These results have a number of important
> implications. They suggest that we should re-think how we measure
> species barriers in the laboratory, and that we should not assume that
> just because one species appears resistant to a strain of prions they
> have been exposed to, that they do not silently carry the infection.
> This research raises the possibility, which has been mentioned before,
> that apparently healthy cattle could harbour, but never show signs of, BSE.
>
> "This is a timely and unexpected result, increasing what we know about
> prion disease. These new findings have important implications for those
> researching prion disease, those responsible for preventing infected
> material getting into the food chain and for those considering how best
> to safeguard health and reduce the risk that theoretically, prion
> disease could be contracted through medical and surgical procedures."
>
> ISSUED FRIDAY 25 AUGUST UNDER EMBARGO. PLEASE NOTE THAT THE EMBARGO IS
> SET BY THE JOURNAL.
>
> FOR FURTHER INFORMATION CONTACT THE MRC PRESS OFFICE ON 020 7637 6011
> (OFFICE HOURS) OR 07818 428297 OR 0385 774357 (OUT-OF-OFFICE-HOURS) OR
> PROFESSOR JOHN COLLINGE ON 020 7594 3760. PLEASE NOTE THAT OWING TO
> TRAVEL COMMITMENTS PROFESSOR COLLINGE WILL ONLY BE AVAILABLE UNTIL 16.30
> ON FRIDAY 25 AUGUST AND CONTACTABLE AGAIN ON MONDAY 28 AUGUST VIA THE
> MRC PRESS OFFICE. DR ANDREW HILL (A CO-AUTHOR ON THE PAPER) FROM THE
> DEPARTMENT OF PATHOLOGY AT THE UNIVERSITY OF MELBOURNE WILL BE AVAILABLE
> ON 00 61 3 8344 3995 (DURING OFFICE HOURS) OR 00 61 3 9443 0009
> (OUT-OF-OFFICE HOURS). PLEASE NOTE THAT AUSTRALIA IS TEN HOURS AHEAD OF
> UK TIME.
>
> NOTES FOR EDITORS
>
> Professor Collinge is a consultant neurologist and Director of the newly
> formed MRC Prion Unit based at The Imperial College School of Medicine
> at St Mary's Hospital. He is also a member of the UK Government's
> Spongiform Encephalopathy Advisory Committee (SEAC). The MRC prion unit
> is was set up in 1999, and its work includes molecular genetic studies
> of human prion disease and transgenic modelling of human prion diseases.
>
> Prions are unique infectious agents that cause fatal brain diseases such
> as Creutzfeldt-Jakob disease (CJD) in humans and scrapie and BSE (mad
> cow disease) in animals. In some circumstances prions from one species
> of animals can infect another and it is clear that BSE has done this to
> cause the disease variant CJD in the UK and France. It remains unclear
> how large an epidemic of variant CJD will occur over the years ahead.
>
> The strain of prion used here to infect the mice is the Sc237 strain
> (also known as 263K) which infects hamsters, and until now was assumed
> not to infect mice.
>
> This research was funded by the Medical Research Council and Wellcome Trust.
>
> The Medical Research Council (MRC) is a national organisation funded by
> the UK tax-payer. Its business is medical research aimed at improving
> human health; everyone stands to benefit from the outputs. The research
> it supports and the scientists it trains meet the needs of the health
> services, the pharmaceutical and other health-related industries and the
> academic world. MRC has funded work which has led to some of the most
> significant discoveries and achievements in medicine in the UK. About
> half of the MRC's expenditure of £345 million is invested in over 50 of
> its Institutes and Units, where it employs its own research staff. The
> remaining half goes in the form of grant support and training awards to
> individuals and teams in universities and medical schools.
>
> The Wellcome Trust is the world's largest medical research charity with
> a spend of some £600 million in the current financial year 1999/2000.
> The Wellcome Trust supports more than 5,000 researchers, at 400
> locations, in 42 different countries to promote and foster research with
> the aim of improving human and animal health. As well as funding major
> initiatives in the public understanding of science, the Wellcome Trust
> is the country's leading supporter of research into the history of medicine.
>
> ©2002 Medical Research Council
> Data Protection policy | Contact the MRC
>
> http://www.mrc.ac.uk/index/public_interest/public-press_office/public-press_releases_2000/public-mrc-43-00.htm
>
> ======================================
>
> Oral transmission and early lymphoid tropism of chronic wasting disease
> PrPres in mule deer fawns (Odocoileus hemionus )
> Christina J. Sigurdson1, Elizabeth S. Williams2, Michael W. Miller3,
> Terry R. Spraker1,4, Katherine I. O'Rourke5 and Edward A. Hoover1
>
> Department of Pathology, College of Veterinary Medicine and Biomedical
> Sciences, Colorado State University, Fort Collins, CO 80523- 1671, USA1
> Department of Veterinary Sciences, University of Wyoming, 1174 Snowy
> Range Road, University of Wyoming, Laramie, WY 82070, USA 2
> Colorado Division of Wildlife, Wildlife Research Center, 317 West
> Prospect Road, Fort Collins, CO 80526-2097, USA3
> Colorado State University Veterinary Diagnostic Laboratory, 300 West
> Drake Road, Fort Collins, CO 80523-1671, USA4
> Animal Disease Research Unit, Agricultural Research Service, US
> Department of Agriculture, 337 Bustad Hall, Washington State University,
> Pullman, WA 99164-7030, USA5
>
> Author for correspondence: Edward Hoover.Fax +1 970 491 0523. e-mail
> ehoover@lamar.colostate.edu
>
> Mule deer fawns (Odocoileus hemionus) were inoculated orally with a
> brain homogenate prepared from mule deer with naturally occurring
> chronic wasting disease (CWD), a prion-induced transmissible spongiform
> encephalopathy. Fawns were necropsied and examined for PrP res, the
> abnormal prion protein isoform, at 10, 42, 53, 77, 78 and 80 days
> post-inoculation (p.i.) using an immunohistochemistry assay modified to
> enhance sensitivity. PrPres was detected in alimentary-tract-associated
> lymphoid tissues (one or more of the following: retropharyngeal lymph
> node, tonsil, Peyer's patch and ileocaecal lymph node) as early as 42
> days p.i. and in all fawns examined thereafter (53 to 80 days p.i.). No
> PrPres staining was detected in lymphoid tissue of three control fawns
> receiving a control brain inoculum, nor was PrPres detectable in neural
> tissue of any fawn. PrPres-specific staining was markedly enhanced by
> sequential tissue treatment with formic acid, proteinase K and hydrated
> autoclaving prior to immunohistochemical staining with monoclonal
> antibody F89/160.1.5. These results indicate that CWD PrP res can be
> detected in lymphoid tissues draining the alimentary tract within a few
> weeks after oral exposure to infectious prions and may reflect the
> initial pathway of CWD infection in deer. The rapid infection of deer
> fawns following exposure by the most plausible natural route is
> consistent with the efficient horizontal transmission of CWD in nature
> and enables accelerated studies of transmission and pathogenesis in the
> native species.
>
> snip...
>
> These results indicate that mule deer fawns develop detectable PrP res
> after oral exposure to an inoculum containing CWD prions. In the
> earliest post-exposure period, CWD PrPres was traced to the lymphoid
> tissues draining the oral and intestinal mucosa (i.e. the
> retropharyngeal lymph nodes, tonsil, ileal Peyer's patches and
> ileocaecal lymph nodes), which probably received the highest initial
> exposure to the inoculum. Hadlow et al. (1982) demonstrated scrapie
> agent in the tonsil, retropharyngeal and mesenteric lymph nodes, ileum
> and spleen in a 10-month-old naturally infected lamb by mouse bioassay.
> Eight of nine sheep had infectivity in the retropharyngeal lymph node.
> He concluded that the tissue distribution suggested primary infection
> via the gastrointestinal tract. The tissue distribution of PrPres in the
> early stages of infection in the fawns is strikingly similar to that
> seen in naturally infected sheep with scrapie. These findings support
> oral exposure as a natural route of CWD infection in deer and support
> oral inoculation as a reasonable exposure route for experimental studies
> of CWD.
>
> snip...
>
> http://vir.sgmjournals.org/cgi/content/full/80/10/2757
> ===================================
>
> now, just what is in that deer feed? _ANIMAL PROTEIN_
>
> Subject: MAD DEER/ELK DISEASE AND POTENTIAL SOURCES
> Date: Sat, 25 May 2002 18:41:46 -0700
> From: "Terry S. Singeltary Sr."
> Reply-To: BSE-L
> To: BSE-L
>
> 8420-20.5% Antler Developer
> For Deer and Game in the wild
> Guaranteed Analysis Ingredients / Products Feeding Directions
>
> snip...
>
> _animal protein_
>
> http://www.surefed.com/deer.htm
>
> BODE'S GAME FEED SUPPLEMENT #400
> A RATION FOR DEER
> NET WEIGHT 50 POUNDS
> 22.6 KG.
>
> snip...
>
> _animal protein_
>
> http://www.bodefeed.com/prod7.htm
>
> Ingredients
>
> Grain Products, Plant Protein Products, Processed Grain By-Products,
> Forage Products, Roughage Products 15%, Molasses Products,
> __Animal Protein Products__,
> Monocalcium Phosphate, Dicalcium Pyosphate, Salt,
> Calcium Carbonate, Vitamin A Acetate with D-activated Animal Sterol
> (source of Vitamin D3), Vitamin E Supplement, Vitamin B12 Supplement,
> Riboflavin Supplement, Niacin Supplement, Calcium Panothenate, Choline
> Chloride, Folic Acid, Menadione Soduim Bisulfite Complex, Pyridoxine
> Hydorchloride, Thiamine Mononitrate, d-Biotin, Manganous Oxide, Zinc
> Oxide, Ferrous Carbonate, Calcium Iodate, Cobalt Carbonate, Dried
> Sacchoromyces Berevisiae Fermentation Solubles, Cellulose gum,
> Artificial Flavors added.
>
> http://www.bodefeed.com/prod6.htm
> ===================================
>
> MORE ANIMAL PROTEIN PRODUCTS FOR DEER
>
> Bode's #1 Game Pellets
> A RATION FOR DEER
> F3153
>
> GUARANTEED ANALYSIS
> Crude Protein (Min) 16%
> Crude Fat (Min) 2.0%
> Crude Fiber (Max) 19%
> Calcium (Ca) (Min) 1.25%
> Calcium (Ca) (Max) 1.75%
> Phosphorus (P) (Min) 1.0%
> Salt (Min) .30%
> Salt (Max) .70%
>
>
> Ingredients
>
> Grain Products, Plant Protein Products, Processed Grain By-Products,
> Forage Products, Roughage Products, 15% Molasses Products,
> __Animal Protein Products__,
> Monocalcium Phosphate, Dicalcium Phosphate, Salt,
> Calcium Carbonate, Vitamin A Acetate with D-activated Animal Sterol
> (source of Vitamin D3) Vitamin E Supplement, Vitamin B12 Supplement,
> Roboflavin Supplement, Niacin Supplement, Calcium Pantothenate, Choline
> Chloride, Folic Acid, Menadione Sodium Bisulfite Complex, Pyridoxine
> Hydrochloride, Thiamine Mononitrate, e - Biotin, Manganous Oxide, Zinc
> Oxide, Ferrous Carbonate, Calcium Iodate, Cobalt Carbonate, Dried
> Saccharyomyces Cerevisiae Fermentation Solubles, Cellulose gum,
> Artificial Flavors added.
>
> FEEDING DIRECTIONS
> Feed as Creep Feed with Normal Diet
>
> http://www.bodefeed.com/prod8.htm
>
> INGREDIENTS
>
> Grain Products, Roughage Products (not more than 35%), Processed Grain
> By-Products, Plant Protein Products, Forage Products,
> __Animal Protein Products__,
> L-Lysine, Calcium Carbonate, Salt, Monocalcium/Dicalcium
> Phosphate, Yeast Culture, Magnesium Oxide, Cobalt Carbonate, Basic
> Copper Chloride, Manganese Sulfate, Manganous Oxide, Sodium Selenite,
> Zinc Sulfate, Zinc Oxide, Sodium Selenite, Potassium Iodide,
> Ethylenediamine Dihydriodide, Vitamin E Supplement, Vitamin A
> Supplement, Vitamin D3 Supplement, Mineral Oil, Mold Inhibitor, Calcium
> Lignin Sulfonate, Vitamin B12 Supplement, Menadione Sodium Bisulfite
> Complex, Calcium Pantothenate, Riboflavin, Niacin, Biotin, Folic Acid,
> Pyridoxine Hydrochloride, Mineral Oil, Chromium Tripicolinate
>
> DIRECTIONS FOR USE
>
> Deer Builder Pellets is designed to be fed to deer under range
> conditions or deer that require higher levels of protein. Feed to deer
> during gestation, fawning, lactation, antler growth and pre-rut, all
> phases which require a higher level of nutrition. Provide adequate
> amounts of good quality roughage and fresh water at all times.
>
> http://www.profilenutrition.com/Products/Specialty/deer_builder_pellets.html
> ===================================================
>
> DEPARTMENT OF HEALTH & HUMAN SERVICES
> PUBLIC HEALTH SERVICE
> FOOD AND DRUG ADMINISTRATION
>
> April 9, 2001 WARNING LETTER
>
> 01-PHI-12
> CERTIFIED MAIL
> RETURN RECEIPT REQUESTED
>
> Brian J. Raymond, Owner
> Sandy Lake Mills
> 26 Mill Street
> P.O. Box 117
> Sandy Lake, PA 16145
> PHILADELPHIA DISTRICT
>
> Tel: 215-597-4390
>
> Dear Mr. Raymond:
>
> Food and Drug Administration Investigator Gregory E. Beichner conducted
> an inspection of your animal feed manufacturing operation, located in
> Sandy Lake, Pennsylvania, on March 23,
> 2001, and determined that your firm manufactures animal feeds including
> feeds containing prohibited materials. The inspection found significant
> deviations from the requirements set forth in
> Title 21, code of Federal Regulations, part 589.2000 - Animal Proteins
> Prohibited in Ruminant Feed. The regulation is intended to prevent the
> establishment and amplification of Bovine Spongiform Encephalopathy
> (BSE) . Such deviations cause products being manufactured at this
> facility to be misbranded within the meaning of Section 403(f), of the
> Federal Food, Drug, and Cosmetic
> Act (the Act).
>
> Our investigation found failure to label your
> swine feed with the required cautionary statement "Do Not Feed to cattle
> or other Ruminants" The FDA suggests that the statement be
> distinguished
> by different type-size or color or other means of highlighting the
> statement so that it is easily noticed by a purchaser.
>
> In addition, we note that you are using approximately 140 pounds of
> cracked corn to flush your mixer used in the manufacture of animal
> feeds containing prohibited material. This
> flushed material is fed to wild game including deer, a ruminant animal.
> Feed material which may potentially contain prohibited material should
> not be fed to ruminant animals which may become part of the food chain.
>
> The above is not intended to be an all-inclusive list of deviations from
> the regulations. As a manufacturer of materials intended for animal
> feed use, you are responsible for assuring that your overall operation
> and the products you manufacture and distribute are in compliance with
> the law. We have enclosed a copy of FDA's Small Entity Compliance Guide
> to assist you with complying with the regulation... blah, blah, blah...
>
> http://www.fda.gov/foi/warning_letters/g1115d.pdf
> ==================================
>
>
>
> -------- Original Message --------
> Subject: ON THE ORIGIN OF MINK TME MARSH/HANSON (Scrapie in USA sheep,
> to TSE in USA cattle, or BOTH)
> Date: Thu, 15 May 2003 15:23:46 -0500
> From: "Terry S. Singeltary Sr."
> Reply-To: Bovine Spongiform Encephalopathy
> To: BSE-L@uni-karlsruhe.de
>
>
>
> ######## Bovine Spongiform Encephalopathy #########
>
> ABSTRACT--studies on mink susceptibility to sources of scrapie
> from the United States, but not from the United Kingdom, indicate that
> transmissible mink encephalopathy (TME) most likely originates from
> mink fed scrapie-infected sheep or goat tissues.
> Experiments further suggest that the shortest natural route of infection is
> via bite wounds inflicted by littermates rather than by the oral route
> per se.
> Other studies, on the biologic characterization of TME agent from Sawyer
> County, Wisconsin, indicate that this particular source of TME is composed
> of a mixture of subpopulations which include a hamster pathogen and a
> mink-monkey pathogen...
>
> snip...
>
> with so many disease features in common, it would seem a simple matter
> to demonstrate that TME results from feeding scrapie-infected tissue to
> mink.
> BUT such has not been the case. Epizootiologic studies of the 14 worldwide
> occurrences of TME have revealed probably exposure to scrapie in only one
> instance, a 1965 incidence in Finland in which the affected farm was the
> only
> one in the area feeding sheep heads (Kangas, personal communication).
> Experimentally, mink have been found to be susceptible to some sources of
> scrapie and the disease produces was indistinguishable from TME (6)...
>
> snip...
>
> The purpose of these present studies was to attempt to explain differences
> between field and experimental observations, and to further characterize
> the
> biologic properties of the Sawyer County, Wisconsin, isolate of TME.
> Our results indicate that mink are more susceptible to sources of scrapie
> present in the UNITED STATES that those found in the UK, and that
> BITE WOUNDS from littermates may represent a significant route of
> natural exposure...
>
> snip...
>
> This Nubian X Toggenburg buck was naturally infected via exposure to
> scrapie-contaminated pasture at Mission, TEXAS; the pasture being
> previously occupied by a flock of scrapie-affected Suffolk sheep. At 6
> months
> of age, animal B-834 was removed from exposure and placed in a pen where
> he subsequently developed signs of scrapie at 40 months of age...
>
> snip...
>
> Therefore, it should be expected that the pathology of natural TME will vary
> depending on the source of scrapie to which mink are exposed.
> Johannsen and Hartung have reported an incidence of TME occuring
> in East Germany in 1967 in which affected mink had diffuse cerebral
> ''edema'' and widespread lesions in the spinal cord (10)...
>
> snip...
>
> Even though B-834 produced short incubation periods when inoculated
> intracerebrally, exposure by the oral route was ineffective during an
> observation period of two years. Thus, we once again seem to have a
> conflict between field and experimental data. However, Gajdusek
> has suggested that the main route of entry for these transmissible agents
> is not the oral route per se, but rather via breaks or abrasions of skin and
> mucosal surfaces (11).
>
> full text;
>
> http://www.bseinquiry.gov.uk/files/mb/m08/tab016.pdf
>
> years later Marsh finds out;
>
> Part of the Proceedings of an International Roundtable on Bovine
> Spongiform Encephalopathy, Bethesda, Maryland, USA, June 27-28, 1989.
>
> The possibility of infection with BSE in the United States, as defined
> by studies on the disease in Great Britain, is judged to be low on the
> basis of the following: (1) meat and bonemeals imported into the United
> States from Great Britain between 1980 and 1988 were used mainly in
> poultry, not ruminant feed; (2) the Scrapie Eradication Program had
> reduced the prevalence of scrapie in the United States compared with
> that in Great Britain; and (3) little, if any, rendered animal products
> are used for protein supplements in cattle feed in the United States.
> However, there is some evidence that there may already be a scrapie-like
> disease in cattle in the United States. This evidence comes from
> epidemiologic studies on an incident of transmissible mink
> encephalopathy (TME) in Stetsonville, Wis, in 1985. This mink farmer
> used no commercially available animal by-product mixtures in his feed,
> but instead slaughtered all animals going into the mink diet, which
> included mostly (>95%) "downer" dairy cows, a few horses, but never
> sheep. To examine the possibility that cattle may have been the source
> of this incident of TME, two 6-week-old Holstein bull calves were
> inoculated intracerebrally with mink brain from the affected farm. The
> bulls developed neurologic disease 18 and 19 months after inoculation.
> Both brains had spongiform degeneration at necropsy and both were
> transmissible back to mink by either intracerebral (incubation period of
> 4 months) or oral (incubation period of 7 months) inoculation
> Whereas TME has been thought to be caused by feeding scrapie-infected
> sheep to mink, this theory has no conclusive evidence. Experimental oral
> inoculation of mink with several different sources of sheep scrapie has
> never been successful, and an incubation period of less than 12 months
> has never (sic) produced by intracerebral inoculation. Transmissible
> mink encephalopathy can develop naturally by infection with incubation
> periods of less than 12 months.
> There is reason to believe that scrapie has not been transmitted in the
> United States from sheep to cattle by rendered protein concentrates as
> it was in Great Britain. However, some circumstantial evidence exists
> that cattle may be a source of some TME infections. It is recommended
> that we increase our surveillance for a BSE-like disease in American
> cattle by encouraging state diagnostic laboratories to formalin-fix
> specimens of midbrain and brain stem from bovine brains submitted for
> rabies testing. If results of these tests are negative, these fixed
> tissues can then be examined for evidence of spongiform degeneration of
> the gray matter.
>
> Letter to the Editor, Journal of the American Veterinary Medical
> Association, August 15, 1990
> In my article, "Bovine spongiform encephalopathy in the United States"
> (JAVMA, May 15, 1990, p 1677), I stated that "little, if any, rendered
> animal products are used for protein supplements in cattle feed in the
> United States." I have since learned that this is incorrect, because of
> the recent trend of using less assimilated "by-pass" proteins in cattle
> feed. A large amount of meat-and-bone meal is being fed to American
> cattle, and this change in feeding practice has greatly increased the
> risk of bovine spongiform encephalopathy (BSE) developing in the United
> States.
> Epidemiologic studies on BSE in Great Britain have indicated that the
> disease originated in cattle by exposure to the heat-resistant
> transmissible agent in compounded feed containing rendered animal
> protein. The most likely source of infection was assumed to be
> meat-and-bone meal prepared from scrapie-infected sheep, but it is also
> possible that a heretofore unrecognized scrapie-like infection of cattle
> could have been spread in the same manner.
> Because of concern for the possible development of BSE in the United
> States, the American rendering industry discontinued the processing of
> fallen and sick sheep last December. In my opinion, this was a prudent
> policy, but one that will not prevent the possible transmission of BSE
> from cattle to cattle. As emphasized in my article, there is some
> evidence that BSE-like infection may already exist in American cattle.
> The current practice of feeding meat-and-bone meal to cattle solidifies
> the most important means to perpetuate and amplify the disease cycle.
> In Great Britain, BSE has produced a great economic and emotional
> burden. We must take all reasonable measures to prevent BSE from
> developing in the United States. Therefore, the practice of using animal
> protein in cattle feed should be discontinued as soon as possible.
> Waiting until the first case of BSE is diagnosed in the United States
> will certainly be "closing the barn door after the horse is gone." With
> a disease having a 3- to 6-year incubation period, thousands of animals
> would be exposed before we recognize the problem and, if that happens,
> we would be in for a decade of turmoil.
> R. F. Marsh, DVM, PhD
> Madison, Wis
>
> To be published in the Proceedings of the
> Fourth International Scientific Congress in
> Fur Animal Production. Toronto, Canada,
> August 21-28, 1988
>
> Evidence That Transmissible Mink Encephalopathy
> Results from Feeding Infected Cattle
>
> _ - R.F. Marsh* and G.R. Hartsough
>
> "Department of Veterinary Science, University of Wisconsin-Madison, Madison,
> Wisconsin 53706; and ^Emba/Creat Lakes Ranch Service, Thiensville,
> Wisconsin 53092
>
> ABSTRACT
> Epidemiologic investigation of a new incidence of
> transmissible mink encephalopathy (TME) in Stetsonville, Wisconsin
> suggests that the disease may have resulted from feeding infected
> cattle to mink. This observation is supported by the transmission of
> a TME-like disease to experimentally inoculated cattle, and by the
> recent report of a new bovine spongiform encephalopathy in
> England.
>
> INTRODUCTION
>
> Transmissible mink encephalopathy (TME) was first reported in 1965
> by Hartsough and Burger who demonstrated that the disease was
> transmissible with a long incubation period, and that affected mink
> had a spongiform encephalopathy similar to that found in scrapie-affecied
> sheep (Hartsough and Burger, 1965; Burger and Hartsough, 1965).
> Because of the similarity between TME and scrapie, and the subsequent
> finding that the two transmissible agents were indistinguishable
> (Marsh and Hanson, 1969), it was concluded that TME most likely resulted
> from feeding mink scrapie-infecied sheep.
> The experimental transmission of sheep scrapie to mink (Hanson et al.,
> 1971) confirmed the close association of TME and scrapie, but at the
> same time provided evidence that they may be different. Epidemiologic
> studies on previous incidences of TME indicated that the incubation periods
> in field cases were between six months and one year in length (Harxsough
> and Burger, 1965). Experimentally, scrapie could not be transmitted to mink
> in less than one year.
> To investigate the possibility that TME may be caused by a (particular
> strain of scrapie which might be highly pathogenic for mink, 21 different
> strains of the scrapie agent, including their sheep or goat sources, were
> inoculated into a total of 61 mink. Only one mink developed a progressive
> neurologic disease after an incubation period of 22 mon..s (Marsh and
> Hanson,
> 1979). These results indicated that TME was either caused by a strain of
> sheep scrapie not yet tested, or was due to exposure to a scrapie-like
> agent from an unidentified source.
>
> OBSERVATIONS AND RESULTS
>
> A New Incidence of TME. In April of 1985, a mink rancher in Stetsonville,
> Wisconsin reported that many of his mink were "acting funny", and some had
> died. At this time, we visited the farm and found that approximately 10% of
> all adult mink were showing typical signs of TME: insidious onset
> characterized
> by subtle behavioral changes, loss of formal habits of cleanliness,
> deposition
> of droppings throughout the pen rather than in a single area,
> hyperexcitability,
> difficulty in chewing and swallowing, and tails arched over their _backs
> like
> squirrels. These signs were followed by progressive deterioration of
> neurologic
> function beginning with locomoior incoordination, long periods of
> somnolence in which the affected mink would stand motionless with its head
> in the corner of the cage, complete debilitation, and death. Over the next
> 8-10 weeks, approximately 40% of all the adult mink on the farm died from
> TME. Since previous incidences of TME were associated with common or shared
> feeding practices, we obtained a careful history of feed ingredients used
> over the past 12-18 months. The rancher was a "dead stock" feeder using
> mostly
> (>95%) downer or dead dairy cattle and a few horses. Sheep had never been
> fed.
>
> Experimental Transmission. The clinical diagnosis of TME was confirmed by
> histopaihologic examination and by experimental transmission to mink
> after incubation periods of four months. To investigate the possible
> involvement
> of cattle in this disease cycle, two six-week old castrated Holstein bull
> calves
> were inoculated intracerebrally with a brain suspension from affected mink.
> Each developed a fatal spongiform encephalopathy after incubation periods of
> 18 and 19 months.
>
> DISCUSSION
>
> These findings suggest that TME may result from feeding mink infected
> cattle and we have alerted bovine practitioners that there may exist an
> as yet unrecognized scrapie-like disease of cattle in the United States
> (Marsh and Hartsough, 1986). A new bovine spongiform encephalopathy has
> recently been reported in England (Wells et al., 1987), and investigators
> are presently studying its transmissibility and possible relationship to
> scrapie. Because this new bovine disease in England is characterized by
> behavioral changes, hyperexcitability, and agressiveness, it is very
> likely it would be confused with rabies in the United Stales and not be
> diagnosed. Presently, brains from cattle in the United States which are
> suspected of rabies infection are only tested with anti-rabies virus
> antibody
> and are not examined histopathologically for lesions of spongiform
> encephalopathy. We are presently pursuing additional studies to further
> examine the possible involvement of cattle in the epidemiology of TME.
> One of these is the backpassage of our experimental bovine encephalopathy
> to mink. Because (here are as yet no agent-specific proteins or nucleic
> acids identified for these transmissible neuropathogens, one means of
> distinguishing them is by animal passage and selection of the biotype
> which grows best in a particular host. This procedure has been used to
> separate hamster-adapted and mink-udapted TME agents (Marsh and Hanson,
> 1979). The intracerebral backpassage of the experimental bovine agent
> resulted in incubations of only four months indicating no de-adaptation
> of the Stetsonville agent for mink after bovine passage. Mink fed infected
> bovine brain remain normal after six months. It will be essential to
> demonstrate oral transmission from bovine to mink if this proposed
> epidemiologic association is to be confirmed.
>
> ACKNOWLEDGEMENTS
> These studies were supported by the College of Agricultural and Life
> Sciences, University of Wisconsin-Madison and by a grant (85-CRCR-1-1812)
> from the United States Department of Agriculture. The authors also wish
> to acknowledge the help and encouragement of Robert Hanson who died during
> the course of these investigations.
>
> REFERENCES
> Burger, D. and Hartsough, G.R. 1965. Encephalopathy of mink. II.
> Experimental and natural transmission. J. Infec. Dis. 115:393-399.
> Hanson, R.P., Eckroade, R.3., Marsh, R.F., ZuRhein, C.M., Kanitz, C.L.
> and Gustatson, D.P. 1971. Susceptibility of mink to sheep scrapie. Science
> 172:859-861.
> Hansough, G.R. and Burger, D. 1965. Encephalopathy of mink. I.
> Epizoociologic and clinical observations. 3. Infec. Dis. 115:387-392.
> Marsh, R.F. and Hanson, R.P. 1969. Physical and chemical properties of the
> transmissible mink encephalopathy agent. 3. ViroL 3:176-180.
> Marsh, R.F. and Hanson, R.P. 1979. On the origin of transmissible mink
> encephalopathy. In Hadlow, W.J. and Prusiner, S.P. (eds.) Slow transmissible
> diseases of the nervous system. Vol. 1, Academic Press, New York, pp
> 451-460.
> Marsh, R.F. and Hartsough, G.R. 1986. Is there a scrapie-like disease in
> cattle?
> Proceedings of the Seventh Annual Western Conference for Food Animal
> Veterinary Medicine. University of Arizona, pp 20.
> Wells, G.A.H., Scott, A.C., Johnson, C.T., Cunning, R.F., Hancock, R.D.,
> Jeffrey, M., Dawson, M. and Bradley, R. 1987. A novel progressive spongiform
> encephalopathy in cattle. Vet. Rec. 121:419-420.
>
> http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
>
> Is there a Scrapie-like disease in cattle in USA
>
> http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf
>
>
> SEWING THE SEEDS OF CWD THROUGH ANIMAL PROTEIN?
>
> http://www.tx-outdoors.com/hunting_issues/_disc11/00000084.htm
>
> re-vCJD/blood and meeting of Feb. 20, 2003
>
> http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.pdf
>
> Subject: SCRAPIE 'USA' ANNUAL REPORT (105 __newly__ infected flocks
> 2002) & CWD IN USA Date: Tue, 10 Dec 2002 08:17:17 -0600 From: "Terry S.
> Singeltary Sr." To: flounder@wt.net Date: Mon, 9 Dec
> 2002 21:21:10 -0600 Reply-To: Bovine Spongiform Encephalopathy Sender:
> Bovine Spongiform Encephalopathy From: "Terry S. Singeltary Sr."
> Subject: SCRAPIE 'USA' ANNUAL REPORT (105 newly
> infected flocks 2002) & CWD IN USA As of September 30, 2002, there were
> 45 scrapie infected and source flocks (figure 3). There were 105 newly
> infected flocks, reported in FY2002 (figure 4). In addition, 379 scrapie
> cases were confirmed and reported by the National Veterinary Services
> Laboratories (NVSL) in FY 2002 (figure 5) and (figure 6). Five cases of
> scrapie in goats were reported in FY 2002 (figure 7), the last of which
> was confirmed in August 2002. New infected and source flocks numbers and
> the number of these flocks released in FY 2002 are depicted in chart 4.
> One hundred (100) flocks which is 67 percent of the scrapie infected and
> source flocks present in FY 2002 were released or put on clean-up plans
> in FY2002. Slaughter Surveillance Slaughter Surveillance is currently in
> Phase II which is intended to determine the prevalence of scrapie in the
> US culled sheep population. Through September 2002 samples from 3,269
> sheep were submitted to NVSL for testing. Samples from a total of 6,795
> sheep have been submitted since the beginning of Phase II on April 1,
> 2002. Surveillance regions are depicted in (figure 8). Scrapie Testing
> During FY 2002 11,751 animals have been tested for scrapie which
> includes: 2,711 regular necropsy cases, 1,343 third eyelid biopsies for
> the test validation project, 546 third eyelid biopsies for the
> regulatory program, and approximately 7,151 animals for Phase I & II of
> SOSS (chart 5). Laboratory testing has been taking 10 - 11 days on
> average with a range of 3 - 34 days. Ear Tag Orders During FY 2002 9.9
> million plastic and 6.0 million metal tags were distributed by APHIS
> (chart 6).
> http://www.aphis.usda.gov/vs/nahps/scrapie/annual_report/annual-report.html
> NEW SCRAPIE INFECTED AND SOURCE FLOCKS
> http://www.aphis.usda.gov/vs/nahps/scrapie/annual_report/figure04.gif
> DISTRIBUTION OF CHRONIC WASTING DISEASE THROUGHOUT THE STATES (as of
> Oct. 2002) http://www.aphis.usda.gov/vs/nahps/cwd/cwd-distribution.html
> CWD USA surveillance http://www.aphis.usda.gov/vs/nahps/cwd/cwd-state.html
>
> Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease
> in the United States [FULL TEXT] Date: February 22, 2003 at 7:38 am PST
> plus TSS rebuttal and submission to Neurology
>
> http://www.vegsource.com/talk/madcow/messages/9912538.html
>
> or short version;
>
> http://www.neurology.org/cgi/eletters/60/2/176#535
>
> TSS
>
> ########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html
> ############
>
> =====================================================
>
>
> USA 8/4/97 RUMINANT-TO-RUMINANT FEED BAN that never was...
>
> 'ANIMAL PROTEIN' SEARCH 9/9/02
> ==============================
>
> Darling International, Inc.
> 5/07/02
> Seattle District Office Animal Proteins Prohibited in Ruminant
> Feed/Misbranded [PDF]
> [HTML] All American Feed & Tractor
> 4/01/02
> Seattle District Office Animal Proteins Prohibited in Ruminant
> Feed/Adulterated [PDF]
> [HTML] Tyson Foods
> 2/12/02
> Seattle District Office Animal Proteins Prohibited in Ruminant
> Feed/Misbranded [PDF]
> [HTML] The Feed Bucket
> 12/11/01
> Atlanta District Office Animal Proteins Prohibited in Ruminant
> Feed/Adulterated/Misbranded [PDF]
> [HTML] Finlayson Ag Center
> 11/08/01
> Minneapolis District Office Animal Proteins Prohibited in Ruminant
> Feed/Adulterated [PDF]
> [HTML] Dixon Feeds, Inc.
> 10/24/01
> Seattle District Office Animal Proteins Prohibited in Ruminant
> Feed/Adulterated [PDF]
> [HTML] Buckeye Feed Mills, Inc.
> 9/20/01
> Cincinnati District Office Animal Proteins Prohibited in Ruminant
> Feed/Adulterated/Misbranded [PDF]
> [HTML] Wilcox Farms, Inc.
> 9/14/01
> Seattle District Office Animal Proteins Prohibited in Ruminant Feed [PDF]
> [HTML]
>
> http://www.accessdata.fda.gov/scripts/wlcfm/full_text.cfm?full_text=animal+protein&Search=Search
>
> now, compare search on 8/8/01...tss
> ===================================
>
> 'ANIMAL PROTEIN' SEARCH 8/8/01
> ==============================
>
> Date: Tue, 28 Aug 2001 11:13:43 -0700
> Reply-To: BSE-L
> Sender: Bovine Spongiform Encephalopathy BSE-L
> From: "Terry S. Singeltary Sr."
> Subject: MAD COW FEED BAN WARNING LETTERS U.S.A. AUGUST 8, 2001
>
> DEPARTMENT OF HEALTH AND HUMAN SERVICES
>
> Food and Drug Administration
>
> Seattle District Pacific Region 22201 23rd Drive SE Bothell, WA 98021-4421
>
> Telephone: 426-486-8788 FAX: 426-483-4996
>
> August 8, 2001
>
> VIA CERTIFIED MAIL RETURN RECEIPT REQUESTED
>
> In reply refer to Warning Letter SEA 01-75
>
> William W. Himmelspach, Owner 22195 S.W. 78th Tualatin, Oregon 97062
>
> WARNING LETTER
>
> Dear Mr. Himmelspach:
>
> An investigation at your animal feed manufacturing operation located at
> 22195 S.W. 78th Tualatin, Oregon 97062, conducted by a Food and Drug
> Administration investigator on July 12, 2001, found significant
> deviations from the requirements set forth in Title 21, Code of Federal
> Regulations, Part 589.2000 - Animal Proteins Prohibited in Ruminant
> Feed. The regulation is intended to prevent the establishment and
> amplification of Bovine Spongiform Encephalopathy (BSE). Such deviations
> cause products being manufactured at this facility to be adulterated
> within the meaning of Section 402(a)(2)(C), and 402(a)(4) of the Federal
> Food, Drug and Cosmetic Act (the Act).
>
> Our investigation found a failure to separate the receipt, processing,
> and storage of the product containing prohibited material from
> non-prohibited material; failure to establish a written system,
> including clean-out and flushing procedures, to avoid commingling and
> cross-contamination of common equipment; and failure to maintain records
> sufficient to track the materials throughout the receipt, processing,
> and distribution of your products.
>
> In addition, our investigation found a failure to label your products
> with the required cautionary, statement "Do Not Feed to Cattle or Other
> Ruminants," Your pig feeds, containing prohibited materials, were not
> labeled with the cautionary statement, and you reuse poly-tote bags for
> ruminant feed and pig feed, where the bags could become contaminated
> with prohibited material. The FDA suggests the statement be
> distinguished by different type size or color or other means of
> highlighting the statement so that it is easily noticed by a purchaser.
>
> The above is not intended to be an all-inclusive list of deviations from
> the regulations. As a manufacturer of materials intended for animal feed
> use, you are responsible for assuring that your overall operation and
> the products you manufacture and distribute are in compliance with
>
> William W. Himmelspach Tualatin, Oregon Re: Warning Letter SEA 01-75 Page 2
>
> your overall operation and the products you manufacture and distribute
> are in compliance with the law. We have enclosed a copy of the FDA's
> Small Entity Compliance Guide to assist you with complying with the
> regulation.
>
> You should take prompt action to correct these violations, and you
> should establish a system whereby such violations do not recur. Failure
> to promptly correct these violations may result in regulatory action
> without further notice, such as seizure and/or injunction.
>
> You should notify this office in writing within 15 working days of
> receipt of this letter, of the steps you have taken to bring your firm
> into compliance with the law. Your response should include an
> explanation of each step being taken to correct the violations, and
> prevent their recurrence. If corrective action cannot be completed in 15
> working days, state the reason for the delay and the date by which the
> corrections will be completed. Include copies of any available
> documentation demonstrating that corrections have been made.
>
> Your reply should be directed to the Food and Drug Administration,
> Attention: Bruce Williamson, Compliance Officer. If you have any
> questions please contact Mr. Williamson at (425) 483-4976.
>
> Sincerely,
>
> Charles M. Breen District Director
>
> Enclosure; Form FDA 483 Small Entity Compliance Guide
>
> http://www.fda.gov/foi/warning_letters/g1619d.pdf
>
> Warning Letters Index - Search Form Results Company Name Date Issued
> Issuing Office
>
> Subject
>
> File Adrian Elevator, Inc. 5/03/01 Minneapolis District Office Animal
> Proteins Prohibited in Ruminant Feed
>
> View File Alaska Garden and Pet Supply, Inc. 4/27/01 Seattle District
> Office Animal Proteins Prohibited in Ruminant Feed
>
> View File Bryan Enterprises 2/20/01 Cincinnati District Office Feed
> Mill/Animal Proteins Prohibited in Ruminant Feed/Adulterated
>
> View File Carrollton Farmers Exchange 7/12/01 Cincinnati District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File Centerburg Mill and General Store, Inc 3/23/01 Cincinnati
> District Office Animal Proteins Prohibited in Ruminant Feed
>
> View File Centerburg Mill and General Store, Inc. 5/23/01 Cincinnati
> District Office Animal Proteins Prohibited in Ruminant Feed
>
> View File Central Ohio Farmers Cooperative, Inc. 5/24/01 Cincinnati
> District Office Animal Protein Prohibited in Ruminant Feed
>
> View File Champaign Landmark, Inc. 3/05/01 Cincinnati District Office
> Animal Proteins Prohibited in Ruminant Feed/Misbranded
>
> View File Countryline Co-Op, Inc. 5/14/01 Cincinnati District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File Dorset Milling 4/16/01 Cincinnati District Office Animal
> Proteins Prohibited in Ruminant Feed
>
> View File Earl B. Olson Feed Mill 4/23/01 Minneapolis District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File Faler Feed Store, Inc. 3/21/01 Cincinnati District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File Farmers Mill & Elevator Company 3/30/01 Atlanta District
> Office Animal Proteins Prohibited in Ruminant Feed
>
> View File Farnam Companies, Inc. 7/20/01 Kansas City District Office
> Animal Proteins Prohibited in Ruminant Feed/Adulterated
>
> View File Greeley Elevator Company 4/04/01 Denver District Office Animal
> Proteins Prohibited in Ruminant Feed
>
> View File Hartville Elevator Company, Inc. 2/22/01 Cincinnati District
> Office Feed Mill/Animal Proteins Prohibited in Ruminant Feed/Adulterated
>
> View File Himmelspach, William W. 8/08/01 Seattle District Office Animal
> Proteins Prohibited in Ruminant Feed
>
> View File Integral Fish Foods, Inc. 6/12/01 Denver District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File Jefferson Milling Company 4/16/01 Cincinnati District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File Lime Creek Ag Services, Inc. 4/25/01 Minneapolis District
> Office Animal Proteins Prohibited in Ruminant Feed
>
> View File Material Resources LLC 5/04/01 Chicago District Office Animal
> Proteins Prohibited in Ruminant Feed
>
> View File Material Resources, LLC 5/04/01 Chicago District Office Animal
> Protein Prohibited in Ruminant Feed
>
> View File Medina Landmark, Inc. 3/23/01 Cincinnati District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File Minister Farmers Cooperative Exchange, Inc. 4/10/01 Cincinnati
> District Office Animal Proteins Prohibited in Ruminant Feed/Feed Mill
>
> View File Peco Foods, Inc. 2/23/01 New Orleans District Office CGMP
> Requirements for Medicated Feeds/Animal Proteins Prohibited in Ruminant Feed
>
> View File Perry Coal and Feed Company 4/16/01 Cincinnati District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File Rietdyk's Milling Company 3/05/01 Seattle District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File River Valley Co-Op 3/22/01 Cincinnati District Office Animal
> Proteins Prohibeted in Ruminant Feed
>
> View File River Valley Co-Op 5/22/01 Cincinnati District Office Animal
> Proteins Prohibited in Ruminant Feed
>
> View File Round Lake Farmers Coop. 5/30/01 Minneapolis District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File Rudy, Inc. 3/22/01 Cincinnati District Office Animal Proteins
> Prohibited in Ruminant Feed
>
> View File Rudy, Inc. 5/22/01 Cincinnati District Office Animal Proteins
> Prohibited in Ruminant Feed
>
> View File Sandy Lake Mills 4/09/01 Philadelphia District Office Animal
> Proteins Prohibited in Ruminant Feed
>
> View File Shields Feed and Supply Company 3/07/01 New Orleans District
> Office Animal Proteins Prohibited in Ruminant Feed
>
> View File Stewart's Farm Supply 3/21/01 Cincinnati District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File Superior Feeds 6/06/01 Seattle District Office Animal Proteins
> Prohibited in Ruminant Feed
>
> View File The Scoular Company 5/30/01 Minneapolis District Office Animal
> Proteins Prohibited in Ruminant Feed
>
> View File University of Minnesota 5/10/01 Minneapolis District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File Valley Feed Mill, Inc. 5/22/01 Cincinnati District Office
> Animal Proteins Prohibited in Ruminant Feed
>
> View File Wallowa County Grain Growers, Inc. 5/17/01 Seattle District
> Office Animal Proteins Prohibited in Ruminant Feed
>
> View File Wallowa County Grain Growers, Inc. 5/17/01 Seattle District
> Office Animal Proteins Prohibited in Ruminant Feed
>
> View File Western Reserve Farm Cooperative 3/21/01 Cincinnati District
> Office Animal Protein Prohibited in Ruminant Feed
>
> View File Yachere Feed, Inc. 4/09/01 Philadelphia District Office Animal
> Proteins Prohibited in Ruminant Feed
>
> View File Z & W Mill, Inc. 3/27/01 Denver District Office Animal
> Proteins Prohibited in Ruminant Feed
>
> View File
>
> http://63.75.126.221/scripts/wlcfm/resultswl.cfm
>
> (TYPE IN 'ANIMAL PROTEIN')
>
> In Reply to: Docket No. 01-068-1 Risk Reduction Strategies for Potential
> BSE Pathways Involving Downer Cattle and Dead Stock of Cattle and Other
> Species [TSS SUBMISSION] January 21, 2003
>
> http://www.vegsource.com/talk/madcow/messages/9912358.html
>
> =================================================
>
> Date: Sun, 13 Apr 2003 11:14:20 -0500
> Reply-To: Bovine Spongiform Encephalopathy
> Sender: Bovine Spongiform Encephalopathy
> From: "Terry S. Singeltary Sr."
> Subject: SCRAPIE AGENT IN MUSCLE - PATTISON I A (1990) & Prusiner et
> al
> 2001
>
> ######## Bovine Spongiform Encephalopathy
> #########
>
> Greetings List Members,
>
> in response to;
>
> EMBO reports AOP Published online: 11 April 2003 Widespread PrPSc
> accumulation in muscles of hamsters orally infected with scrapie Achim
> Thomzig, Christine Kratzel, Gudrun Lenz, Dominique Krüger & Michael
> Beekes Robert Koch-Institut, P26, Nordufer 20, D-13353 Berlin, Germany
>
> Received 13 February 2003; Accepted 13 March 2003; Published online 11
> April 2003.
>
> Abstract :
>
> Scrapie, bovine spongiform encephalopathy and chronic wasting disease
> are orally communicable, transmissible spongiform encephalopathies
> (TSEs). As zoonotic transmissions of TSE agents may pose a risk to human
> health, the identification of reservoirs for infectivity in animal
> tissues and their exclusion from human consumption has become a matter
> of great importance for consumer protection. In this study, a variety of
> muscles from hamsters that were orally challenged with scrapie was
> screened for the presence of a molecular marker for TSE infection, PrPSc
> (the pathological isoform of the prion protein PrP). Sensitive western
> blotting revealed consistent PrPSc accumulation in skeletal muscles from
> forelimb and hindlimb, head, back and shoulder, and in tongue.
> Previously, our animal model has provided substantial baseline
> information about the peripheral routing of infection in naturally
> occurring and orally acquired ruminant TSEs. Therefore, the findings
> described here highlight further the necessity to investigate thoroughly
> whether muscles of TSE-infected sheep, cattle, elk and deer contain
> infectious agents.
>
> http://www.emboreports.org/
>
> some previous data on TSE in muscle;
>
> J69
>
> CVO BSE 1 5
>
> SCRAPIE AGENT IN MUSCLE - PATTISON I A (1990) VETERINARY RECORD, 20
> JANUARY 1990, p.68
>
> Background
>
> 1 Dr Pattison, a retired but eminent worker on scrapie for many years in
> the AFRC, has pointed out that in one of his experimental studies of
> scrapie in goats he found scrapie agent in the biceps femoris (rump)
> muscle of one animal with clinical disease but not in 2 others with
> clinical disease and in none with pre-clinical disease. MAFF have based
> their policy on BSE in regard to meat (beef) on the results of studies
> of natural scrapie (ie disease occurring under farm conditions) in both
> sheep and goats by Hadlow 1979, 80, 81.
>
> Other Infectivity Studies
>
> 2. These studies on 52 animals by equally eminent scrapie workers
> (Hadlow et al) revealed no evidence whatever of infectivity in skeletal
> muscle from these natural cases either in the pre-clinical or even
> clinical stages of disease.
>
> It is clear that the pathogenesis of experimental (Pattison) and natural
> (Hadlow) scrapie may be different and it was therefore considered wise
> to base present policy on knowledge of the natural disease.
>
> 3. Pattison exposed his 14 goats to intracerebral inoculation of thrice
> passaged scrapie virus (in goats). This may have resulted in strain
> selection and/or mutation of the natural agent. In contrast Hadlow's
> study involved natural strains (probably multiple) in a flock with a
> high incidence of disease in which exposure would almost certainly have
> been by the mouth.
>
> 4. The fact that Hadlow identified no infectivity in muscle by mouse
> inoculation (whereas some other tissues not normally consumed had
> detectable infectivifcy) shows that cross contamination of his tissues
> did not occur. Pattison's experiments were reported about 20 years
> earlier when much less was known about Scrapie. In the intervening
> period the knowledge available to Hadlow on the insensitivity of scrapie
> agent to heat became available. There is therefore at least the
> possibility that Pattison's instruments were not sterilised effectively,
> thus possibly giving the false positive result for muscle.
>
> 5. Pattison used a more sensitive model for the detection of
> infectivifcy, namely goats, whereas Hadlow used mice ie necessitating
> crossing the species barrier and possibly reducing the test sensitivity.
>
> 90/1.19/9.1
>
> CVO BSE 1 5
>
> 6. In regard to the choice of species for agent assay, mice (Hadlow),
> these would be guaranteed free of pre-existing Scrapie infection.
> Pattison could offer no such guarantee that this was the case in the
> animal to which muscle was passaged and disease could have developed
> from exposure from a source other than muscle.
>
> 7. Pattison did not report that his recipient animals, including the one
> inoculated with muscle, were examined by histopathology to confirm the
> presence of disease. This is a significant deficit. Clinical diagnosis
> alone is not acceptable as adequate evidence for the existence of scrapie.
>
> 8. Even in Pattison's studies only in 1 out of 14 goats was infectivity
> detected in muscle and that was in a CLINICAL case. In BSE all clinical
> cases are notified and do not enter any food chain.
>
> 9. The last paragraph of Pattison's letter is illogical. Furthermore,
> this is no evidence whatsoever that scrapie or BSE is a danger to man.
>
> W A WATSON 19 January 1990
>
> Private Offices Mr K C Meldrum Mrs E Attridge Mr R Lowson Ms L Austin Mr
> R Bradley
>
> 90/1.19/9.2
>
> http://www.bseinquiry.gov.uk/files/yb/1990/01/19009001.pdf
>
> Prions in skeletal muscle
>
> Patrick J. Bosque*,dagger ,Dagger , Chongsuk Ryou*, Glenn Telling*,§,
> David Peretz*,dagger , Giuseppe Legname*,dagger , Stephen J.
> DeArmond*,dagger ,¶, and Stanley B. Prusiner*,dagger ,||,**
>
> * Institute for Neurodegenerative Diseases and Departments of dagger
> Neurology, ¶ Pathology, and || Biochemistry and Biophysics, University
> of California, San Francisco, CA 94143
>
> Contributed by Stanley B. Prusiner, December 28, 2001
>
> Considerable evidence argues that consumption of beef products from
> cattle infected with bovine spongiform encephalopathy (BSE) prions
> causes new variant Creutzfeldt-Jakob disease. In an effort to prevent
> new variant Creutzfeldt-Jakob disease, certain "specified offals,"
> including neural and lymphatic tissues, thought to contain high titers
> of prions have been excluded from foods destined for human consumption
> [Phillips, N. A., Bridgeman, J. & Ferguson-Smith, M. (2000) in The BSE
> Inquiry (Stationery Office, London), Vol. 6, pp. 413-451]. Here we
> report that mouse skeletal muscle can propagate prions and accumulate
> substantial titers of these pathogens. We found both high prion titers
> and the disease-causing isoform of the prion protein (PrPSc) in the
> skeletal muscle of wild-type mice inoculated with either the Me7 or
> Rocky Mountain Laboratory strain of murine prions. Particular muscles
> accumulated distinct levels of PrPSc, with the highest levels observed
> in muscle from the hind limb. To determine whether prions are produced
> or merely accumulate intramuscularly, we established transgenic mice
> expressing either mouse or Syrian hamster PrP exclusively in muscle.
> Inoculating these mice intramuscularly with prions resulted in the
> formation of high titers of nascent prions in muscle. In contrast,
> inoculating mice in which PrP expression was targeted to hepatocytes
> resulted in low prion titers. Our data demonstrate that factors in
> addition to the amount of PrP expressed determine the tropism of prions
> for certain tissues. That some muscles are intrinsically capable of
> accumulating substantial titers of prions is of particular concern.
> Because significant dietary exposure to prions might occur through the
> consumption of meat, even if it is largely free of neural and lymphatic
> tissue, a comprehensive effort to map the distribution of prions in the
> muscle of infected livestock is needed. Furthermore, muscle may provide
> a readily biopsied tissue from which to diagnose prion disease in
> asymptomatic animals and even humans. Dagger Present address: Department
> of Medicine, Denver Health Medical Center, Denver, CO 80204.
>
> § Present address: Department of Microbiology and Immunology, University
> of Kentucky, Lexington, KY 40536-0230.
>
> ** To whom reprint requests should be addressed. E-mail: vann@cgl.ucsf.edu.
>
> www.pnas.org/cgi/doi/10.1073/pnas.052707499
>
> http://www.pnas.org/cgi/content/abstract/99/6/3812?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&author1=prusiner&author2=prusiner&titleabstract=prions+meat+tissue+mice&fulltext=prions+meat+tissue+mice&searchid=1024346978866_6016&stored_search=&FIRSTINDEX=0&fdate=1/1/2002
>
>
>
> FULL TEXT;
>
> http://www.pnas.org/cgi/content/full/99/6/3812?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&author1=prusiner&author2=prusiner&titleabstract=prions+meat+tissue+mice&fulltext=prions+meat+tissue+mice&searchid=1050249844061_1953&stored_search=&FIRSTINDEX=0&fdate=1/1/2002
>
>
>
> Greetings,
>
> seems they just will not accept that BSE and Scrapie can transmit to
> primates and humans. how many times do we have to do the same studies
> over and over again, before action is taken on all human/animal TSEs? if
> they would have just listened. they were so wrong about BSE, how can
> they be so right about Scrapie, especially since it too transmits to
> primates...
>
> COMMERCIAL IN CONFIDENCE NOT FOR PUBLICATION SUB COMMITTEE ON
> BIOLOGICALS. COMMITTEE ON SAFETY OF MEDICINES
>
> CSM/SEAR/88 10TH MEETING. BIOLS/88/6TH MEETING
>
> This paper was discussed by the Biological Sub-Committee on 2 November
> 1988, when the following recommendations were made;
>
> 1. No immediate licensing action should be taken against oral products,
> in which bovine material has been used.
>
> 2. All bovine materials should come from cattle from appropriately
> certified healthy herds, which have not been given food supplements
> containing material of animal origin. No brain or lymphoid tissue should
> be used in parenteral products.'
>
> 3. Manufacturers of parenteral products should show that their
> manufacturing processes are capable of eliminating scrapie-like agents.
>
> 4. All licences for new products from bovine materials should comply
> with the above.
>
> 5. There should be an article in MAIL requesting manufacturers to
> identify products in which bovine materials have been used. Bovine
> albumin and foetal calf serum should come from appropriately certified
> healthy herds.
>
> 6. The above should be drawn to the attention of the review/CDSM
> sections along with the need to search for preparations containing
> bovine material.
>
> 7. The above should be drawn to the attention of the ADR Section and
> SEAR along with the need to search the ADR database for reactions to
> bovine products.
>
> REMARK.
>
> 1. The Licensing Authority's attention was drawn to the need to give
> ongoing consideration to whether action was required on bovine insulin
> and heparin products.
>
> 88/11.02/5.1
>
> http://www.bseinquiry.gov.uk/files/yb/1988/11/02005001.pdf
>
> Adaptation of the bovine spongiform encephalopathy agent to primates and
> comparison with Creutzfeldt- Jakob disease: Implications for human health
>
> snip...
>
> We found that the BSE agent in nonhuman primates is similar to that
> causing vCJD in humans and tends to evolve rapidly toward a
> primate-adapted variant. Furthermore, we showed that the strain
> responsible for iCJD is closely related to that of one patient with
> sCJD, and, more unexpectedly, that these agents were similar to the
> French scrapie strain studied (but different from the U.S. scrapie strain).
>
> snip...
>
> http://www.pnas.org/cgi/content/full/041490898v1
>
> BSE Inquiry VACCINES & SUTURES
>
> http://www.bseinquiry.gov.uk/files/yb/1989/02/14010001.pdf
>
> http://www.bseinquiry.gov.uk/files/yb/1989/02/14011001.pdf
>
> http://www.bseinquiry.gov.uk/files/yb/1990/01/10009001.pdf
>
> http://www.bseinquiry.gov.uk/files/ws/s422.pdf
>
> http://www.bseinquiry.gov.uk/files/yb/1989/07/03004001.pdf
>
> 1: J Infect Dis 1980 Aug;142(2):205-8 Related Articles, Links
>
> Oral transmission of kuru, Creutzfeldt-Jakob disease, and scrapie to
> nonhuman primates.
>
> Gibbs CJ Jr, Amyx HL, Bacote A, Masters CL, Gajdusek DC.
>
> Kuru and Creutzfeldt-Jakob disease of humans and scrapie disease of
> sheep and goats were transmitted to squirrel monkeys (Saimiri sciureus)
> that were exposed to the infectious agents only by their nonforced
> consumption of known infectious tissues. The asymptomatic incubation
> period in the one monkey exposed to the virus of kuru was 36 months;
> that in the two monkeys exposed to the virus of Creutzfeldt-Jakob
> disease was 23 and 27 months, respectively; and that in the two monkeys
> exposed to the virus of scrapie was 25 and 32 months, respectively.
> Careful physical examination of the buccal cavities of all of the
> monkeys failed to reveal signs or oral lesions. One additional monkey
> similarly exposed to kuru has remained asymptomatic during the 39 months
> that it has been under observation.
>
> http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=6997404&dopt=Abstract
>
>
>
> TSS
>
> ########### http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html
> ############
>
> ===================================================
>
> -------- Original Message --------
>
> Subject: HISTORY OF CJD -- CJD QUESTIONNAIRE
> Date: Thu, 15 May 2003 10:59:54 -0500
> From: "Terry S. Singeltary Sr."
> Reply-To: Bovine Spongiform Encephalopathy
> To: BSE-L@uni-karlsruhe.de
>
>
>
> ######## Bovine Spongiform Encephalopathy #########
>
> CJD QUESTIONNAIRE
>
> http://www.bseinquiry.gov.uk/files/mb/m26/tab04.pdf
>
> THE EPIDEMIOLOGY OF CJD
> R.G. WILL 1984 (182 PAGES)
>
> snip...
>
> Table I: Synonyms for CJD
>
> Creutzfeldt-Jakob Disease
> Spastic Pseudosclerosis
> Cortico-Striatal-spinal Degeneration
> Brownell-Oppenheimer Syndrome
> Jakob Type of Spastic Pseudosclerosis
> with muscular atropy
> Disseminated Encephalomyelopathy
> Transmissible Virus Dementia
> Subacute Spongiform Encephalopathy
> Heidenhain's Syndrome
> Jakob's Syndrome
> Subacute Progressive Encephalopthy
> with Bulbar Myoclonus
> [and now vCJD...how many names are we going to name the same disease?
> i call this disease ''nobelitice'' $$$ TSS]
>
> snip...
>
> Scrapie infected meat or sheep products are an obvious potential
> environmental source of infection. Oral transmission of Scrapie
> and TME in the laboratory is well established (Burger and Hartsourgh,
> 1965; Pattison et al., 1972) and more recently CJD has been transmitted
> to primates by the oral consuption of brain, kidney or spleen
> (Gibbs et al., 1980). TME is __thought__ to have been transmitted
> to mink by the consumption of scrapie affected sheep carcases (Marsh
> and Hanson, 1979) [but that theory has changed, please see url...TSS]
>
> MARSH
>
> To be published in the Proceedings of the
> Fourth International Scientific Congress in
> Fur Animal Production. Toronto, Canada,
> August 21-28, 1988
>
> Evidence That Transmissible Mink Encephalopathy
> Results from Feeding Infected Cattle
>
> _ - R.F. Marsh* and G.R. Hartsough
>
> "Department of Veterinary Science, University of Wisconsin-Madison,
> Madison, Wisconsin 53706; and ^Emba/Creat Lakes Ranch Service,
> Thiensville, Wisconsin 53092
>
> ABSTRACT
> Epidemiologic investigation of a new incidence of
> transmissible mink encephalopathy (TME) in Stetsonville, Wisconsin
> suggests that the disease may have resulted from feeding infected
> cattle to mink. This observation is supported by the transmission of
> a TME-like disease to experimentally inoculated cattle, and by the
> recent report of a new bovine spongiform encephalopathy in
> England.
>
> INTRODUCTION
>
> Transmissible mink encephalopathy (TME) was first reported in 1965 by
> Hartsough and Burger who demonstrated that the disease was transmissible
> with a long incubation period, and that affected mink had a spongiform
> encephalopathy similar to that found in scrapie-affecied sheep (Hartsough
> and Burger, 1965; Burger and Hartsough, 1965). Because of the similarity
> between TME and scrapie, and the subsequent finding that the two
> transmissible agents were indistinguishable (Marsh and Hanson,
> 1969), it was concluded that TME most likely resulted from feeding
> mink scrapie-infecied sheep.
> The experimental transmission of sheep scrapie to mink (Hanson et al., 1971)
> confirmed the close association of TME and scrapie, but at the same time
> provided evidence that they may be different. Epidemiologic studies on
> previous incidences of TME indicated that the incubation periods in field
> cases were between six months and one year in length (Harxsough and Burger,
> 1965). Experimentally, scrapie could not be transmitted to mink in less than
> one year.
> To investigate the possibility that TME may be caused by a (particular
> strain of scrapie which might be highly pathogenic for mink, 21 different
> strains of the scrapie agent, including their sheep or goat sources, were
> inoculated into a total of 61 mink. Only one mink developed a progressive
> neurologic disease after an incubation period of 22 mon..s (Marsh and
> Hanson,
> 1979). These results indicated that TME was either caused by a strain of
> sheep
> scrapie not yet tested, or was due to exposure to a scrapie-like agent
> from an unidentified source.
>
> OBSERVATIONS AND RESULTS
>
> A New Incidence of TME. In April of 1985, a mink rancher in
> Stetsonville, Wisconsin reported that many of his mink were
> "acting funny", and some had died. At this time, we visited
> the farm and found that approximately 10% of all adult mink
> were showing typical signs of TME: insidious onset characterized
> by subtle behavioral changes, loss of normal habits of cleanliness,
> deposition of droppings throughout the pen rather than in a single
> area, hyperexcitability, difficulty in chewing and swallowing, and
> tails arched over their _backs like squirrels. These signs were
> followed by progressive deterioration of neurologic function
> beginning with locomoior incoordination, long periods of somnolence
> in which the affected mink would stand motionless with its head in
> the corner of the cage, complete debilitation, and death. Over the
> next 8-10 weeks, approximately 40% of alt the adult mink on the farm
> died from TME.
> Since previous incidences of TME were associated with common or shared
> feeding practices, we obtained a careful history of feed ingredients used
> over the past 12-18 months. The rancher was a "dead stock" feeder using
> mostly (>95%) downer or dead dairy cattle and a few horses.
> Sheep had never been fed.
>
> Experimental Transmission. The clinical diagnosis of TME was confirmed
> by histopaihologic examination and by experimental transmission to mink
> after incubation periods of four months. To investigate the possible
> involvement of cattle in this disease cycle, two six-week old castrated
> Holstein bull calves were inoculated intracerebrally with a brain suspension
> from affected mink. Each developed a fatal spongiform encephalopathy after
> incubation periods of 18 and 19 months.
>
> DISCUSSION
>
> These findings suggest that TME may result from feeding mink infected
> cattle and we have alerted bovine practitioners that there may exist
> an as yet unrecognized scrapie-like disease of cattle in the United States
> (Marsh and Hartsough, 1986). A new bovine spongiform encephalopathy has
> recently been reported in England (Wells et al., 1987), and investigators
> are presently studying its transmissibility and possible relationship to
> scrapie. Because this new bovine disease in England is characterized by
> behavioral changes, hyperexcitability, and agressiveness, it is very
> likely it would be confused with rabies in the United Stales and not be
> diagnosed. Presently, brains from cattle in the United States which are
> suspected of rabies infection are only tested with anti-rabies virus
> antibody
> and are not examined histopathologically for lesions of spongiform
> encephalopathy.
> We are presently pursuing additional studies to further examine the possible
> involvement of cattle in the epidemiology of TME. One of these is the
> backpassage of our experimental bovine encephalopathy to mink. Because
> (here are as yet no agent-specific proteins or nucleic acids identified
> for these transmissible neuropathogens, one means of distinguishing them
> is by animal passage and selection of the biotype which grows best in a
> particular host. This procedure has been used to separate hamster-
> adapted and mink-udapted TME agents (Marsh and Hanson, 1979). The
> intracerebral backpassage of the experimental bovine agent resulted in
> incubations of only four months indicating no de-adaptation of the
> Stetsonville agent for mink after bovine passage. Mink fed infected bovine
> brain remain normal after six months. It will be essential to demonstrate
> oral transmission fiom bovine to mink it this proposed epidemiologic
> association is to be confirmed.
>
> ACKNOWLEDGEMENTS
> These studies were supported by the College of Agricultural and Life
> Sciences, University of Wisconsin-Madison and by a grant (85-CRCR-1-1812)
> from the
> United States Department of Agriculture. The authors also wish to
> acknowledge
> the help and encouragement of Robert Hanson who died during the course of
> these
> investigations.
>
> REFERENCES
>
> Burger, D. and Hartsough, G.R. 1965. Encephalopathy of mink. II.
> Experimental and
> natural transmission. J. Infec. Dis. 115:393-399.
> Hanson, R.P., Eckroade, R.3., Marsh, R.F., ZuRhein, C.M., Kanitz, C.L.
> and Gustatson,
> D.P. 1971. Susceptibility of mink to sheep scrapie. Science 172:859-861.
> Hansough, G.R. and Burger, D. 1965. Encephalopathy of mink. I.
> Epizoociologic and
> clinical observations. 3. Infec. Dis. 115:387-392.
> Marsh, R.F. and Hanson, R.P. 1969. Physical and chemical properties of the
> transmissible mink encephalopathy agent. 3. ViroL 3:176-180.
> Marsh, R.F. and Hanson, R.P. 1979. On the origin of transmissible mink
> encephalopathy. In Hadlow, W.J. and Prusiner, S.P. (eds.) Slow transmissible
> diseases of the nervous system. Vol. 1, Academic Press, New York, pp
> 451-460.
> Marsh, R.F. and Hartsough, G.R. 1986. Is there a scrapie-like disease in
> cattle?
> Proceedings of the Seventh Annual Western Conference for Food Animal
> Veterinary
> Medicine. University of Arizona, pp 20.
> Wells, G.A.H., Scott, A.C., Johnson, C.T., Cunning, R.F., Hancock, R.D.,
> Jeffrey, M.,
> Dawson, M. and Bradley, R. 1987. A novel progressive spongiform
> encephalopathy
> in cattle. Vet. Rec. 121:419-420.
>
> http://www.bseinquiry.gov.uk/
>
> Is there a Scrapie-like disease in cattle in USA
>
> http://www.bseinquiry.gov.uk/files/mb/m09a/tab01.pdf
>
> http://www.bseinquiry.gov.uk/files/mb/m09/tab05.pdf
>
> and kuru was almost certainly spread by ritual cannibalism (Alpers, 1979)...
>
> snip...
>
> the conclusion must be that there is no _firm_ evidence linking the
> development of CJD to environmental exposure to the scrapie agent...
>
> snip...
>
> Localised areas with high incidence of CJD have been described in England,
> Hungary, Czechoslovakia, ''U.S.A.'', and Italy, but in the absence of a
> comparison with the national incidence, these ''CLUSTERS'' may well have
> been
> discovered by chance...[hense, the reason USA still refuses to have _NO_ CJD
> Questionnaire...TSS]
>
> snip...
>
> HOWEVER, detailed _questioning of patients, relatives and subsequent
> investigation_ (which the USA still refused to do in 2003...TSS)
> revealed some remarkable coincidences. (or where they that coincidental?tss)
> One patient, a dentist, had for many years daily passed the residence of
> another patient, the husband of a hairdresser who worked in the family home.
> The
> dentist's wife used the hairdresser on occasions but the patient himself had
> never
> entered the salon. Interestingly the dentist himself had possible contact
> with
> other cases (vide infra) and a close acquaintance, another dentist, worked
> in a
> small town in Essex in which two further cases of CJD had occurred. In a
> different
> part of the country a district nurse who died of possible CJD may have
> tended a patient dying of CJD seven years before she herself died of the
> condition.
> The nurse's daughter lived in a nearby city within 30 yards of the house of
> another
> patient. Detailed investigation of other cases revealed close proximity to
> other
> cases but no definite contact. Intensive questioning of surviving relatives
> and
> other witnesses was limited for FEAR OF CAUSING UNNECESSARY ALARM OR
> DISTRESS...
>
> snip...
>
> One patient who had previously worked on a farm died of CJD, but he had
> workded with cattle, a species not known to be susceptible to natural
> scrapie, and had no contact with sheep. [wrong they did know at this
> time ...tss]
>
> snip...
>
> (Masters et al., 1979b) concluded that 'in familial clusters of CJD, the
> virus is more likely to have gained access to the host through some form
> of common environmental contamination than by vertical transmission'.
>
> snip...
>
> 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
> by 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
>
> snip...........end
>
>
> full text ;
>
> 2003D-0186
> Guidance for Industry: Use of Material From Deer and Elk In Animal Feed
>
>
> EMC 7
> Terry S. Singeltary Sr.
> Vol #:
> 1
>
>
> http://www.fda.gov/ohrms/dockets/dailys/03/oct03/100203/100203.htm
>
>
> thank you,
> I am sincerely,
>
> Terry S. Singeltary SR.
> P.O. Box 42
> Bacliff, TEXAS USA 77518
>
>






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