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Nor-98 and CJD IN USA

flounder

Well-known member
Subject: Aspects of the Cerebellar Neuropathology in Nor98
Date: September 26, 2007 at 4:06 pm PST

P03.141

Aspects of the Cerebellar Neuropathology in Nor98


Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1
1National Veterinary Insitute, Sweden; 2National Veterinary Institute,
Norway


Nor98 is a prion disease of old sheep and goats. This atypical form of
scrapie was first
described in Norway in 1998. Several features of Nor98 were shown to be
different from classical scrapie including the distribution of disease
associated
prion protein (PrPd) accumulation in the brain. The cerebellum is generally
the most
affected brain area in Nor98. The study here presented aimed at adding
information on the
neuropathology in the cerebellum of Nor98 naturally affected sheep of
various
genotypes in Sweden and Norway. A panel of histochemical and
immunohistochemical (IHC) stainings such as IHC for PrPd, synaptophysin,
glial fibrillary acidic
protein, amyloid, and cell markers for phagocytic cells were conducted. The
type of
histological lesions and tissue reactions were evaluated. The types of PrPd
deposition
were characterized. The cerebellar cortex was regularly affected, even
though
there was a variation in the severity of the lesions from case to case.
Neuropil
vacuolation was more marked in the molecular layer, but affected also the
granular cell
layer. There was a loss of granule cells. Punctate deposition of PrPd was
characteristic. It
was morphologically and in distribution identical with that of
synaptophysin,
suggesting that PrPd accumulates in the synaptic structures. PrPd was also
observed in
the granule cell layer and in the white matter. *** The pathology features
of Nor98
in the cerebellum of the affected sheep showed similarities with those of
sporadic
Creutzfeldt-Jakob disease in humans.


http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf


From: "Terry S. Singeltary Sr."
Sent: Tuesday, August 21, 2007 9:50 AM
Subject: TWO MORE Nor98 atypical Scrapie cases detected in USA bringing
total to 3 cases to date


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



NEW SCIENTIST MAGAZINE 4/02/01

NEW SCIENTIST EDITORIAL PAGE 3

MAD SHEEP DISEASE?

IF THERE is one categorical pronouncement you
can safely make about prion diseases like BSE
or CJD, it is that one should not make
categorical pronouncements. "British beef is
safe" and "there is no BSE in Germany" come
to mind. Now there are two more: "scrapie is
safe", and "people don't catch sporadic CJD".
Scrapie is the most widespread prion
disease, infecting untold numbers of
sheep worldwide. Sporadic CJD is the
old-fashioned pre-BSE kind that is supposed
to happen spontaneously in unlucky people.
But a surprise observation in France suggests
some sCJD cases--though by no means all--may
be linked to scrapie after all (see p 4).

For years, British authorities asserted that
BSE was harmless because it was a form of
scrapie. In fact, the only evidence scrapie
is safe is some broad-brush epidemiology, good
as far as it goes but unable to reveal
occasional risks for some people from some
sheep. Alarm bells should have rung in 1980
when researchers gave monkeys scrapie by
feeding them infected brains. But that
research, like so much other work on
prion diseases, was never followed up.
We still have little idea what BSE does
in pigs and chickens. The Queniborough
vCJD outbreak (see p 5) would be easier
to understand if we knew how much brain
we must eat to be infected. As for scrapie,
it shouldn't take a chance finding to
tell us that there may be dangerous sheep
out there.

Suspect symptoms

What if you can catch old-fashioned CJD by
eating meat from a sheep infected with
scrapie?

Exclusive from New Scientist magazine

Four years ago, Terry Singeltary watched his
mother die horribly from a degenerative brain disease.................

full text url follows
By Debora MacKenzie

Suspect Symptoms

http://www.newscientist.com/channel/health/bse/mg16922840.300

if url dead, go here for 'SUSPECT SYMPTOMS'

you can access article here also;

http://www.mad-cow.org/UKCJD/CJD_news52.html#29%20Mar%2001%20-%20CJD%20-%20Suspect%20symptoms

http://www.organicconsumers.org/meat/scrapiecjd.cfm


Then follow up with PNAS studies from which
new scientist article written from;

Published online before print March 20, 2001
Proc. Natl. Acad. Sci. USA, 10.1073/pnas.041490898
Abstract of this Article
Reprint (PDF) Version of this Article
Similar articles found in:
PNAS Online
PubMed
PubMed Citation
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Lasmézas, C. I. || Deslys, J.-P.
Alert me when:
new articles cite this article
Download to Citation Manager
Neurobiology
Adaptation of the bovine spongiform encephalopathy agent to primates and
comparison with Creutzfeldt- Jakob disease: Implications for human health
Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*, Virginie Nouvel*,
Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger
] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique
Dormont*, and Jean-Philippe Deslys*

* Commissariat à l'Energie Atomique, Service de Neurovirologie,
Direction des Sciences du Vivant/Département de Recherche Medicale,
Centre de Recherches du Service de Santé des Armées 60-68, Avenue du
Général Leclerc, BP 6, 92 265 Fontenay-aux-Roses Cedex, France; [Dagger
] Hôpital Neurologique Pierre Wertheimer, 59, Boulevard Pinel, 69003
Lyon, France; § Laboratoire de Neuropathologie, Hôpital de la
Salpêtrière, 83, Boulevard de l'Hôpital, 75013 Paris, France; ¶
Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital,
Crewe Road, Edinburgh EH4 2XU, United Kingdom; and [||] Institute for
Animal Health, Neuropathogenesis Unit, West Mains Road, Edinburgh EH9
3JF, United Kingdom

Edited by D. Carleton Gajdusek, Centre National de la Recherche
Scientifique, Gif-sur-Yvette, France, and approved December 7, 2000
(received for review October 16, 2000)

Abstract

There is substantial scientific evidence to support the notion that
bovine spongiform encephalopathy (BSE) has contaminated human beings,
causing variant Creutzfeldt-Jakob disease (vCJD). This disease has
raised concerns about the possibility of an iatrogenic secondary
transmission to humans, because the biological properties of the
primate-adapted BSE agent are unknown. We show that (i) BSE can be
transmitted from primate to primate by intravenous route in 25 months,
and (ii) an iatrogenic transmission of vCJD to humans could be readily
recognized pathologically, whether it occurs by the central or
peripheral route. Strain typing in mice demonstrates that the BSE agent
adapts to macaques in the same way as it does to humans and confirms
that the BSE agent is responsible for vCJD not only in the United
Kingdom but also in France. The agent responsible for French iatrogenic
growth hormone-linked CJD taken as a control is very different from vCJD
but is similar to that found in one case of sporadic CJD and one sheep
scrapie isolate. These data will be key in identifying the origin of
human cases of prion disease, including accidental vCJD transmission,
and could provide bases for vCJD risk assessment.

Introduction

The recognition of a variant of the human transmissible spongiform
encephalopathy (TSE) Creutzfeldt-Jakob Disease (vCJD) in the U.K. in
1996 raised the major concern that it would correspond to human
infection with the agent responsible for bovine spongiform
encephalopathy (BSE; ref. 1). Transmission of BSE to macaques provided
the first experimental evidence as it produced a disease close to vCJD
in humans (2). Strain typing in inbred mice (consisting of measuring the
incubation period and establishing lesion profiles corresponding to the
strain-specific distribution of brain vacuolation) allows reliable
identification of TSE strains (3). This method, together with
biochemical methods, has revealed a single phenotype for the agents of
BSE and the British cases of vCJD (4-6). Mice expressing only the bovine
prion protein (PrP) were highly susceptible to vCJD and BSE, which
induced the same disease (7). Thus, it is now well established that BSE
has caused vCJD, probably by alimentary contamination. In this respect,
the finding of abnormal PrP labeling in the gastrointestinal tract and
lymphatic tissues of orally BSE-contaminated lemurs shows that the BSE
agent can infect primates by the oral route (8). About 1 million
contaminated cattle may have entered the human food chain, and the
future number of vCJD cases could range from 63 to 136,000 depending on
the incubation period of BSE in humans (9). Unlike sporadic CJD (sCJD)
and iatrogenic CJD (iCJD) linked to the administration of contaminated
growth hormone extracted from human hypophyses, in vCJD, the infectious
agent seems to be widely distributed in lymphoid organs, as pathological
PrP (PrPres) can be detected in tonsils, lymph nodes, spleen, and
appendix even in the preclinical phase of the disease (10, 11). This
raises a public health issue with regard to the risk of iatrogenic
transmission of vCJD through surgical instruments, grafts, blood
transfusion, or parenteral administration of biological products of
human origin. However, this risk is difficult to assess, because it
largely depends on factors such as the virulence of the BSE agent
adapted to primates and the efficiency of secondary transmission to
humans by a peripheral route such as the i.v. one. A further issue is
whether vCJD accidentally acquired from humans would be recognized. The
latter poses the question of a phenotypic variation of the BSE agent
after successive transmissions in humans: does it retain its strain
characteristics, and does it induce a pathology similar to that observed
in the previous host? A 9-year history of transmission of BSE to
primates and mice enables us today to clarify a number of these
important points.

Although BSE has mainly affected the U.K., two definite cases and one
probable case of vCJD have now been reported in France in people who
have never resided in the U.K. (12, 13). We strain-typed the first of
these cases to establish its origin. Strain typing in C57BL/6 mice of
BSE, French, and British vCJD was compared with that of BSE passaged in
nonhuman primates, thus allowing us to study the effect of serial
passages in primates. Comparisons were also made with French cases of
sCJD and iCJD and two strains of scrapie (one of French and one of U.S.
origin). Our findings provide experimental demonstration that the same
agent, namely that responsible for the cattle disease BSE, has caused
vCJD both in France and in the U.K., in line with biochemical data and
with the fact that, until 1996, about 10% of the beef consumed in France
was imported from the U.K. 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). This finding requires a cautious interpretation
for several reasons, not least because of the inevitably limited number
of TSE strains that can be studied by such a cumbersome method as strain
typing. Nonetheless, it also prompts reconsideration of the possibility
that, in some instances, sheep and human TSEs can share a common origin.

snip...

http://www.pnas.org/cgi/content/full/041490898v1


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


TSS

---------- https://lists.aegee.org/cjd-l.html ----------
 

flounder

Well-known member
The dormant but ongoing threat of vCJD
LYNDSAY MOSS
ACROSS Edinburgh, teams of scientists are searching for answers to one of the most deadly and frightening diseases to hit the UK in recent years - variant Creutzfeldt-Jakob disease (vCJD).

The National CJD Surveillance Unit at Edinburgh University is not only counting cases of the disease, thought to be caused by having eaten Bovine Spongiform Encephalopathy (BSE)-contaminated meat, but is also looking for clues as to how it can be stopped. Now experts from around the world are meeting in Edinburgh at Prion2007 to discuss vCJD and related diseases, and how they might be tackled.

Professor James Ironside, a professor of clinical neuropathology at the Edinburgh unit, is one of those investigating prion diseases - those caused by abnormal proteins in the brain. He says that after the crisis of "mad cow disease" and subsequent vCJD infections in humans, the UK had a "special relationship" with such diseases.

Ironside says this means we should remain wary of new threats to humans from animals, including from scrapie - a prion disease mainly affecting sheep.

"There's a feeling in many areas that BSE is over and it's extinct," says Ironside. "It's not quite extinct in the UK, but there is a feeling we don't need to bother about these things any more. But, as with any other diseases, the more you look, the more you find, and recently there have been new forms of BSE and new forms of scrapie identified in animals in the UK and Europe. They have found a form of atypical scrapie. It actually turns out to be probably more widely distributed than the typical form of scrapie.

"The idea that this problem is over is not completely true. There are still unresolved questions that we need to find out about."

Ironside says a lot of time and effort has been put into scrapie in sheep, and trying to reduce its incidence, and that these efforts have worked. But, he adds: "That was all set in place before these atypical forms were identified, so in a way it's a completely different disease and we have to bear that in mind without being scaremongering."

Ironside says there is still debate about whether BSE came as the spontaneous result of cows eating feed made from animal remains, or if it originated from a type of scrapie. He says that while there is no evidence of it so far, atypical scrapie has the potential to be transmissible to humans and this prospect is currently being studied.

"You can study its transmission characteristics in mice," Ironside says. "We will have to see how these diseases go. One problem with these diseases is that although we do have models of infection, the incubation periods tend to be lengthy. Even in mice, incubation periods can be a year or two years and that is not uncommon."

Ironside says he is still stunned that cattle were fed the remains of other animals, thus allowing BSE to pass to humans in the form of vCJD. And he adds there are still good reasons to keep BSE prevention measures high to stop another widespread outbreak.

"I don't want to be alarmist about this, but with the relaxation of measures for BSE, and this occurring Europe-wide, what we don't want to happen is another outbreak of BSE or some other form of these diseases in Britain or anywhere else," he says. "We need to make sure that the steps that halted the infection of cattle with BSE continue. It basically boiled down to a form of cannibalism, feeding animal remains to animals. I had no idea that was going on, and I think most people didn't. It just seems wrong.

"Also, the contaminated animal feed from the UK was exported to other countries, so BSE has popped up not just in Europe but in Japan and places like that. We know the animal feed also went to other countries that don't have the resources to test for BSE, in Asia for example.

"What I wouldn't like to see is BSE from these other countries somehow, with globalisation, causing a boomerang effect so it comes back."

The threat from the previous outbreak is also far from over. While cases of vCJD are declining, Ironside and others have raised fears that many people may be harbouring the infection without developing symptoms.

This means they might pass it on through blood donations or on contaminated surgical equipment. Long incubation periods for the disease may mean that due to genetic influences, some people may not develop vCJD symptoms for many years to come. It is hoped that a rapid screening test could help prevent the disease becoming endemic through blood transfusions and surgery. But there are also ethical concerns over what you tell someone who is found to be infected but might never develop clinical disease, with no cure or treatment currently available.

Ironside says this made the search for vaccines and treatments all the more important, but this held challenges.

"The problem with treating any type of brain disease is a thing called the blood-brain barrier, which stops certain types of drugs, including some really useful drugs, from getting into the brain," he says. "That is why some of these drugs have to be injected through the spinal cord.

"There are some studies of compounds in animals and cell cultures which have been shown to slow down the progression of these diseases. A lot only seem effective if given before or in the early stages of disease. I'm more optimistic about treatments, but I don't think it's going to be a quick win."

. Prion2007 begins tomorrow at the Edinburgh International Conference Centre.

This article: http://news.scotsman.com/health.cfm?id=1529872007


TSS
 
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