Chronic wasting disease of deer – is the battle to keep Europe free already lost?
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Chronic wasting disease of deer – is the battle to keep Europe free already
lost?
Veterinary Record 2016;179:121-123 doi:10.1136/vr.i4165
Research Editorial
Chronic wasting disease of deer – is the battle to keep Europe free already
lost?
Mark P. Dagleish, BVM & S, PhD, MRCVS, FRCPath + Author
Affiliations
President Veterinary Deer Society, Moredun Research Institute, Pentlands
Science Park, Bush Loan, Penicuik, Edinburgh EH26 0PZ, UK, e-mail:
mark.dagleish@moredun.ac.uk CHRONIC wasting disease (CWD) is a prion disease
affecting many species of deer, which was, until recently, confined to North
America. However, confirmed diagnoses in Norway in both reindeer (Rangifer
tarandus) and European elk/moose (Alces alces) in two geographically widely
separated regions (Defra 2016a) is particularly disturbing.
The origin of CWD in Norway is obscure. It is possible that introduction
from North America in contaminated deer material occurred as a single episode
and the infection has spread or there have been two independent introductions.
Alternatively, abnormal prions may have been generated anew in a Norwegian deer
with subsequent dissemination. Establishing the most probable route will
facilitate the implementation of appropriate control strategies to limit further
incursion of CWD into Europe. However, if the North American experience is
anything to go by, this incursion will have far-reaching implications throughout
Europe and Asia since it has severely damaged deer farming and hunting
industries in affected regions of Canada and the USA.
Like all prion diseases, such as scrapie and bovine spongiform
encephalopathy (BSE), CWD is a member of the transmissible spongiform
encephalopathies (TSE) identified by the typical histological lesions found in
the brain. As such, it is an invariably fatal neurodegenerative disease with
pathological changes associated with the conversion of the normal host-encoded
membrane-associated prion protein (PrPC) to abnormal disease-associated isoforms
(PrPd) which accumulate, principally in nervous tissue, due to their resistance
to enzymatic breakdown; it is also referred to as PrPres (Collinge 2001). In
CWD, PrPd also accumulates peripherally in lymphoid tissue, similar to scrapie
in sheep, and detection of PrPd is the current method of definitive diagnosis
(Gavier-Widen and others 2005).⇓
snip...
CWD is known to infect many different species of deer in addition to those
mentioned previously, including white tailed deer (Odocoileus virginianus), mule
deer (Odocoileus hemionus), black tailed deer (Odocoileus hemionus columbianus),
North American elk (Cervus canadensis) and, critically for those of us in
Europe, European red deer (Cervus elaphus elaphus) (Balachandran and others
2010). Roe deer (Capreolus capreolus), the most numerous and widely distributed
species of deer in Europe, have never been experimentally challenged to assess
their susceptibility to CWD. However, their close phylogenetic relationship to
Odocoileus species means susceptibility should be assumed and their potential to
act as vectors/amplifiers recognised.
CWD is unique among prion diseases, being the only one maintained in wild,
free-ranging populations, often at low densities (two to three deer per square
mile). The infectious CWD prion protein is shed in the saliva, urine, faeces and
antler velvet of infected animals in both the clinical and preclinical stages of
the disease. This is in addition to the contribution decomposing infected
carcases make to environmental contamination. Infective CWD PrPd adheres firmly
to soil surface particles, has been found in plant material (including livestock
forage) and ground water and has been shown to remain viable after passing
through the digestive tract of scavengers of deer carcases such as coyotes
(Canis latrans) and American crows (Corvus brachyrhynchos) (Vercauteren and
others 2012), all of which further aid spread of the disease.
CWD was originally described in 1967 in a wildlife research facility in
northern Colorado, USA, but it took 10 years to be classified as a TSE. From
this localised occurrence CWD has spread relentlessly via captive and
free-ranging populations of several species of deer across the USA and into
Canada (Williams 2005). Unfortunately, attempts to contain and eradicate it
failed despite large amounts of financial and other resources. Presently, most
research in North America is focused on rapid and accurate diagnosis to allow
culling of affected individuals (Haley and Hoover 2015) as, unlike scrapie in
sheep, there appears to be no prion protein genotypes that confer complete
disease resistance to CWD in the deer species studied extensively to date. Some
genotypes of the prion protein do prolong the incubation period but as animals
shed infective prions in the preclinical phase this enhances disease spread.
There is the possibility of a diseaseresistant genotype in reindeer (Mitchell
and others 2012), and fallow deer (Dama dama) may be resistant to natural
infection (Hamir and others 2011), but these possibilities have yet to be
confirmed.
Clinically, CWD is an invariably fatal and progressive neurological
disease. The duration of the clinical disease is highly variable and death can
occur within four weeks but some clinically affected animals may survive as long
as a year. The incubation period is typically between two and four years
(Williams 2005) but shorter incubation periods are possible and during the
preclinical period the animal is infectious. Clinical signs are typically
locomotor abnormalities progressing to chronic weight loss, decreased/altered
interaction with conspecifics, fine head tremors, stereotypic behaviour,
polydipsia, polyuria, salivation and bruxism. Aspiration pneumonia is a common
proximate cause of death, presumably due to laryngeal/ pharyngeal paralysis.
There is also a loss of fear of people and anthropogenic activity, which can
result in increased incidences of road traffic accidents and consequent
fatalities in people.
There is a considerable body of scientific knowledge relating to CWD due to
the large amount of funding provided for many years by the USA and Canadian
governments. Most importantly, CWD has been shown not to be zoonotic and under
natural transmission conditions appears confined to deer species only with no
record of spread to cograzing cattle, sheep or any other genus. This is a
critical point as CWD is clinically indistinguishable from experimental
infection of deer with BSE (Dagleish and others 2008, 2015). Fortunately,
methods for clearly distinguishing CWD from BSE infection in deer have been
developed (Martin and others 2009).
In the UK, the national deer herd comprises up to 2,000,000 wild deer with
up to 300,000 animals culled every year (Parliamentary Office of Science and
Technology 2009). While it is challenging to accurately estimate the size of
deer populations it is generally accepted that in recent years the national herd
has increased in size (Ward 2005). The UK Government has a remit for raising
awareness and monitoring diseases of deer and carrying out some surveillance
through the Action Plans for England, Scotland and Wales (Deer Action Plans
2011, Llywodraeth Cymru Welsh Government 2016, Wild Deer National Approach
2016). It would be hoped that stalkers would alert the relevant authorities if
they were to suspect CWD in British deer. If CWD were to reach the UK it would
pose a risk to the national wild herd and to the farmed deer industry, which
comprises over 31,000, mainly red, deer (Defra 2015). Furthermore, there are
approximately 260 enclosed deer parks, mainly red and fallow deer but also other
species, comprising up to 47,000 animals (The Deer Initiative 2015). While
farmed and park deer may be a small proportion of the wild deer population they
have relatively close contact with people and are of high economic and aesthetic
value. The Animal and Plant Health Agency (APHA) risk assessment document on CWD
states that ‘farmed and park deer may have a higher probability of exposure to
CWD transferred to the environment than wild deer given the restricted habitat
range and higher frequency of contact with tourists and returning GB residents’
(Defra 2016b). Therefore, it may be prudent to prioritise surveillance of park
and farmed deer to increase the sensitivity of detecting CWD incursion into the
UK.
Disease surveillance of wild, free-living populations is inevitably
inefficient, thus CWD could have gone undetected for years if not decades, in
Norway. However, despite the paucity of data at this stage it would appear
prudent that the APHA, as a matter of urgency, reviews the situation and
initiates policies to reduce the threat of CWD incursion to the UK.
Key points
Chronic wasting disease (CWD) is a transmissible spongiform encephalopathy
that causes an invariably fatal disease in many species of deer and has, until
recently, been restricted to North America.
The long incubation period, usually over a year, combined with the
infectious CWD prion protein being extremely environmentally persistent and its
diagnosis in two wild, free-ranging species of deer in Norway, means CWD is
probably well established in the environment already and spread to other
European countries is inevitable.
CWD has been shown not to be zoonotic despite extensive investigations but
it is clinically indistinguishable from experimental infection of deer with
bovine spongiform encephalopathy (BSE).
No case of natural infection with BSE in deer has been recorded.
Establishment of CWD as an endemic disease in the UK would result in
significant negative effects on deer farming and stalking industries as well as
wildlife populations.
References
BMC Vet Res. 2008; 4: 17.
Published online 2008 May 28. doi: 10.1186/1746-6148-4-17
PMCID: PMC2423184
Experimental transmission of bovine spongiform encephalopathy to European
red deer (Cervus elaphus elaphus)
Mark P Dagleish,corresponding author1 Stuart Martin,2 Philip Steele,1
Jeanie Finlayson,1 Sílvia Sisó,2 Scott Hamilton,1 Francesca Chianini,1 Hugh W
Reid,1 Lorenzo González,2 and Martin Jeffrey2
corresponding author
Author information ► Article notes ► Copyright and License information ►
This article has been cited by other articles in PMC.
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Abstract
Background
Bovine spongiform encephalopathy (BSE), a member of the transmissible
spongiform encephalopathies (TSE), primarily affects cattle. Transmission is via
concentrate feed rations contaminated with infected meat and bone meal (MBM). In
addition to cattle, other food animal species are susceptible to BSE and also
pose a potential threat to human health as consumption of infected meat products
is the cause of variant Creutzfeldt-Jakob disease in humans, which is invariably
fatal. In the UK, farmed and free ranging deer were almost certainly exposed to
BSE infected MBM in proprietary feeds prior to legislation banning its
inclusion. Therefore, although BSE has never been diagnosed in any deer species,
a possible risk to human health remains via ingestion of cervine products.
Chronic wasting disease (CWD), also a TSE, naturally infects several cervid
species in North America and is spreading rapidly in both captive and
free-ranging populations.
Results
Here we show that European red deer (Cervus elaphus elaphus) are
susceptible to intra-cerebral (i/c) challenge with BSE positive cattle brain
pool material resulting in clinical neurological disease and weight loss by
794–1290 days and the clinical signs are indistinguishable to those reported in
deer with CWD. Spongiform changes typical of TSE infections were present in
brain and accumulation of the disease-associated abnormal prion protein (PrPd)
was present in the central and peripheral nervous systems, but not in lymphoid
or other tissues. Western immunoblot analysis of brain material showed a similar
glycosylation pattern to that of BSE derived from infected cattle and
experimentally infected sheep with respect to protease-resistant PrP isoforms.
However, the di-, mono- and unglycosylated bands migrated significantly (p <
0.001) further in the samples from the clinically affected deer when compared to
BSE infected brains of cattle and sheep.
Conclusion
This study shows that deer are susceptible to BSE by intra-cerebral
inoculation and display clinical signs and vacuolar pathology that are similar
to those of CWD. These findings highlight the importance of preventing the
spread to Europe of CWD from North America as this may necessitate even more
extensive testing of animal tissues destined for human consumption within the
EU. Although the absence of PrPd in lymphoid and other non-neurological tissues
potentially limits the risk of transmission to humans, the replication of TSE
agents in peripheral tissues following intra-cerebral challenge is often
limited. Thus the assessment of risk posed by cervine BSE as a human pathogen or
for environmental contamination should await the outcome of ongoing oral
challenge experiments.
snip...
Conclusion
This study shows that European red deer are susceptible to i/c challenge
with the BSE agent resulting in a disease that is clinically indistinguishable
by routine clinical examination from that reported for CWD [14]. Thus strong
measures to prevent the spread of CWD to Europe are essential as even small
numbers of CWD cases could result in the need for extensive testing of deer
tissues destined for human consumption.
The susceptibility of UK red deer to natural, presumably oral, exposure is
still uncertain. The absence of PrPd in lymphoid tissues in the present work
might appear to limit the risk to humans of infection from venison and other
non-neuronal edible deer tissues and also limits the maintenance of natural
infection in the environment. However, the susceptibility of peripheral tissues
to infection cannot be ascertained from i/c challenge and must await the outcome
of parallel oral challenge experiments.
please see below;
> This investigation resulted in the first and only known case, to date,
of clinical disease or accumulation of abnormal PrPd in any cervid species due
to oral challenge with BSE.
Susceptibility of European Red Deer (Cervus elaphus elaphus) to Alimentary
Challenge with Bovine Spongiform Encephalopathy
Mark P. Dagleish , Stuart Martin, Philip Steele, Jeanie Finlayson, Samantha
L. Eaton, Sílvia Sisó, Paula Stewart, Natalia Fernández-Borges, Scott Hamilton,
Yvonne Pang, Francesca Chianini, Hugh W. Reid, Wilfred Goldmann,
[ ... ], Martin Jeffrey
PLOS
Published: January 23, 2015 • http://dx.doi.org/10.1371/journal.pone.0116094
Abstract
European red deer (Cervus elaphus elaphus) are susceptible to the agent of
bovine spongiform encephalopathy, one of the transmissible spongiform
encephalopathies, when challenged intracerebrally but their susceptibility to
alimentary challenge, the presumed natural route of transmission, is unknown. To
determine this, eighteen deer were challenged via stomach tube with a large dose
of the bovine spongiform encephalopathy agent and clinical signs, gross and
histological lesions, presence and distribution of abnormal prion protein and
the attack rate recorded. Only a single animal developed clinical disease, and
this was acute with both neurological and respiratory signs, at 1726 days post
challenge although there was significant (27.6%) weight loss in the preceding
141 days. The clinically affected animal had histological lesions of vacuolation
in the neuronal perikaryon and neuropil, typical of transmissible spongiform
encephalopathies. Abnormal prion protein, the diagnostic marker of transmissible
encephalopathies, was primarily restricted to the central and peripheral nervous
systems although a very small amount was present in tingible body macrophages in
the lymphoid patches of the caecum and colon. Serial protein misfolding cyclical
amplification, an in vitro ultra-sensitive diagnostic technique, was positive
for neurological tissue from the single clinically diseased deer. All other
alimentary challenged deer failed to develop clinical disease and were negative
for all other investigations. These findings show that transmission of bovine
spongiform encephalopathy to European red deer via the alimentary route is
possible but the transmission rate is low. Additionally, when deer carcases are
subjected to the same regulations that ruminants in Europe with respect to the
removal of specified offal from the human food chain, the zoonotic risk of
bovine spongiform encephalopathy, the cause of variant Creutzfeldt-Jakob
disease, from consumption of venison is probably very low.
snip...
Discussion This investigation resulted in the first and only known case, to
date, of clinical disease or accumulation of abnormal PrPd in any cervid species
due to oral challenge with BSE. The increase in incubation period compared to
European red deer challenged with BSE intra-cerebrally (1060 days) [33] compared
to oral challenge (1727 days) is approximately 60% and similar to the
differences observed in incubation periods for sheep or goats when challenged
with TSE agents by these two routes [40,41]. The neurological clinical signs
observed could be broadly related to the spongiform encephalopathy and the
accumulation of PrPd in that the restlessness, stereotypic head movements and
pacing may be due to compromise of the nucleus accumbens [42], found in the
striatum, and the laboured breathing due to the lesions in the medulla, where
the respiratory centre is located [43]. Alternatively, the laboured and audible
mouth breathing may have been due to, or contributed to by, compromise of either
of the recurrent laryngeal nerves resulting in some degree of laryngeal
paralysis but we were unable to determine this. Apart from the gradual loss of
body weight, the speed of onset of clinical signs and progression was very rapid
but animal welfare requirements precluded any further longitudinal study of
these. The clinical signs described for this animal are broadly similar to those
reported for clinical BSE in European red deer challenged via the intracerebral
route [33], clinical cases of CWD in deer [44] and clinical cases of BSE in
cattle [45].
The predominant hindbrain vacuolation pattern in this clinically affected
European red deer challenged orally with BSE was similar to the same species
challenged intra-cerebrally with the same BSE inoculum [33], cattle naturally
infected with BSE [46], sheep challenged orally with the BSE agent [41] and deer
and elk naturally infected with CWD [47]. Additionally, the tissue distribution
of PrPd in the clinically affected European red deer, which was restricted
primarily to the central and peripheral nervous systems, was similar to that
seen in the same species challenged intracerebrally with the same BSE inoculum
[33]. However, this was in contrast to deer and elk naturally infected with CWD
[44] and sheep naturally [48] or experimentally challenged via the oral route
with scrapie [5] or BSE [41] which all have extensive peripheral accumulation of
PrPd in the lymphoid tissues and supports the body of work that states that both
the host species and the TSE agent both play roles in determining the pattern of
accumulation of PrPd [4,5]. The PrPd labelling in the brain of the clinically
affected European red deer was predominantly punctate and granular and this
pattern, along with the sites listed, is typical of accumulations in the brains
of other ruminant sources of BSE infection [41].
The presence of PrPd in a small number of tingible body macrophages in
gut-associated lymphoid follicles in the clinically affected European red deer
was not present when the same species was challenged by the intracerebral route
with the same inoculum [33]. The mechanism of spread of accumulation of PrPd in
the intracerebrally challenged European red deer was probably incubation-period
related centrifugal spread from the brain to the spinal column, cranial and
other peripheral nerves [33]. This is thought also to be the major mechanism of
dissemination in cattle infected by the oral route with the BSE agent after
initial haematogenous spread from the intestine to the brain [49]. Studies have
examined, in detail, the anatomy of initial translocation of both the BSE [50]
and scrapie [10] agents across the intestinal mucosal barrier of sheep and
concluded that the villous lacteals of the lamina propria and submucosal
lymphatics are the key route in the first two to three and a half hours of
exposure. PrPd was not found in draining lymph nodes until 24 hours
post-challenge and not before 30 days in Peyer’s patches [10] suggesting that
the lymph node accumulation was probably residual inoculum and that in the
Peyer’s patches was probably de novo PrPd. As PrPd was detected only in the
lymphoid tissue of the clinically affected European red deer challenged orally
with BSE after an incubation period of 1727 days, and then only in very small
amounts, and not those examined at 180 and 360 dpc nor those challenged
intracerebrally which were sampled at 794–1290 dpc [33] it would appear that
this is de novo PrPd rather than residual inoculum. This being the case,
European red deer challenged orally with BSE appear very similar to cattle with
respect to organ distribution of PrPd [51,52]. These findings further support
the belief that the accumulation and distribution of PrPd are influenced by a
combination of the TSE agent and the host species [4,5].
The only animal to develop clinical disease in this study was homozygous
for glutamine at PRNP codon 226 and, unfortunately, this was the only animal of
this genotype in the group allowed to progress to clinical disease or
termination of the experiment (Table 1). At the commencement of this study the
only known genetic variation in the PRNP of cervid species closely related to
European red deer and known to influence TSE susceptibility was codon 132 in elk
where heterozygosity (methionine/leucine) was thought to confer a degree of
resistance/lengthening of the incubation period in CWD infections [25–27]. This
was supported by codon 132 in elk being the equivalent of human PRNP codon 129
which is highly influential in susceptibility to both sporadic and variant CJD
[18,53]. Therefore, the European red deer in this study were initially subjected
to evaluation of PRNP codon 132 only and as all were homozygous for methionine
they were randomly assigned to the four experimental groups (6 month cull, 12
month cull, progression to clinical signs/termination of experiment and
unchallenged negative control). At a later date it was shown that codon 226 of
deer PRNP contained a polymorphism [28] that may be associated with TSE
susceptibility [29] hence this was determined retrospectively after the animals
had been assigned to their groups and already challenged with the BSE agent.
Although it is tempting to speculate that European red deer homozygous for
glutamine at PRNP codon 226 may be genetically more susceptible to BSE by the
oral route than deer carrying glutamate at codon 226, one clinical animal cannot
be statistically sufficient to make this association and further trials are
required. Similar problems have been encountered in other long-term TSE studies
where variations in previously unexamined PRNP codons have subsequently been
shown to significantly affect the clinical disease outcome [11]. The only
published assessments of the frequency of the various genotypes of PRNP codon
226 for European red deer were from those in Scotland were the QQ genotype was
found in only 12.9% of animals over the whole country compared to 50% which were
EE and 37.1% EQ [28]. However, one well studied island population within
Scotland, which has no exchange with the mainland, had a prevalence for the QQ
genotype of only 6.0% and the whole study examined 132 animals only [28]. If
this study is representative of the European red deer population then the
prevalence of the PRNP codon 226 QQ genotype is the lowest.
The presence of possible infectivity in both the single clinically affected
animal and the non-clinically affected animals would have ideally been assessed
by subjecting the resultant European red deer brain material to in vivo
infectivity studies in a rodent model of shorter incubation period and/or
further passage in European red deer but this was out-with the scope of this
study. Fortunately, sPMCA was available and due to its exceptionally high
sensitivity it has been proposed as a credible replacement for bioassay [54].
Our initial trials using bovine brain tissue as a substrate for detecting BSE by
sPMCA showed that it was highly suitable for testing tissue from European red
deer being able to detect a positive result to a sample dilution of 10−11 and,
therefore, would probably be suitable for testing other closely related deer
species, if not all cervid species. The consistent presence of PrPres in 4/4
tubes at all rounds in both experiments of sPMCA is strongly suggestive that
infectivity was present in the single clinically affected European red deer. The
single positive tube, one of four replicates, from a negative control animal
(039) in round three of the first experiment of sPMCA was considered negative
and to be due to contamination of the sample at some point as all subsequent
rounds from this animal in experiment two were negative for PrPres.
Additionally, as all replicates at all rounds of the unseeded BSE negative
bovine brain homogenate were negative it is unlikely this was a stochastic
event.
The results of this study show that alimentary transmission of BSE to
European red deer is possible but the transmission rate is low. However, culling
of red deer in the UK, particularly males, occurs mostly when they are 8–10
years (2920–3650 days) old and therefore well within the possible incubation
period for BSE [55]. Altogether our data suggest that when deer carcases for
human consumption are subjected to the same regulations as other ruminant
carcases (The Specified Bovine Offal Order 1990 [56] and its amendments) the
zoonotic risk of BSE from consuming muscle from European red deer is probably
very low.
Greetings Dr. Dagleish and Veterinary Record et al,
we can only pray that Europe is not lost to cwd tse prion.
I read with great interest, but only could read the short abstract, that is
all I have access to as lay person. if you could please send me full text pdf
that would be great. regardless, I wish to forward the latest from the USA, and
some thoughts on where and how cwd was brought to Norway. I do know that there
are no export papers needed for hay and such to be exported to Norway, and we
now know there is a potential risk factor for TSE Prion and plants.
please see ;
***Recently, we have been using PMCA to study the role of environmental
prion contamination on the horizontal spreading of TSEs. These experiments have
focused on the study of the interaction of prions with plants and
environmentally relevant surfaces. Our results show that plants (both leaves and
roots) bind tightly to prions present in brain extracts and excreta (urine and
feces) and retain even small quantities of PrPSc for long periods of time.
Strikingly, ingestion of prioncontaminated leaves and roots produced disease
with a 100% attack rate and an incubation period not substantially longer than
feeding animals directly with scrapie brain homogenate. Furthermore, plants can
uptake prions from contaminated soil and transport them to different parts of
the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety
of environmentally relevant surfaces, including stones, wood, metals, plastic,
glass, cement, etc. Prion contaminated surfaces efficiently transmit prion
disease when these materials were directly injected into the brain of animals
and strikingly when the contaminated surfaces were just placed in the animal
cage. These findings demonstrate that environmental materials can efficiently
bind infectious prions and act as carriers of infectivity, suggesting that they
may play an important role in the horizontal transmission of the disease.
Since its invention 13 years ago, PMCA has helped to answer fundamental
questions of prion propagation and has broad applications in research areas
including the food industry, blood bank safety and human and veterinary disease
diagnosis.
snip...
CELL REPORTS
Report
Grass Plants Bind, Retain, Uptake, and Transport Infectious Prions
PRION UPDATE VIA VEGETABLE PLANTS FROM THE SOIL
56. Members considered that there is no evidence that crops grown on the
land which received composted excreta from BSE-challenged animals pose a TSE
risk to humans or animals. One member suggested that, as some of these animals
are orally challenged with high doses of BSE-infected materials, and the
distribution of infectivity in the digestive system is not completely
understood, it might be premature to conclude that there is no infective agent
in the manure.
Furthermore, an unpublished study had indicated low level absorption of PrP
from soil by tomato plants although it should be noted that this study had not
been repeated. Details of this work would be sent to the SEAC Secretary. Dr
Matthews explained that most of the manure from animals challenged with high
doses of BSE had already been composted and used for coppicing. Members agreed
that the risks from disposal of residual manure from experimental animals would
be much less than historic risks of on farm contamination from naturally
infected animals at the height of the BSE epidemic. ...SNIP...END
SRM are certain cattle tissues capable of transmitting BSE. There is no
human health risk assessment to indicate the absence of human health concerns
associated with use of composted SRM domestically. To date, scientific evidence
has not been able to demonstrate that composting destroys prions. Although
domestic use would pose a negligible risk to livestock, there is a potential
risk to humans via direct ingestion of the compost or of compost particles
adhered to skin or plant material (e.g. carrots). Another potential route of
exposure is by ingestion of prions that have been taken up by plants. It has
been proven that bacteria are readily taken up by some plants (e.g. E. coli in
lettuce) thus the uptake of prions by plants cannot be precluded or dismissed at
this time. As a science-based regulator, the CFIA cannot change the policy on
this issue without a risk assessment demonstrating that the use of composted SRM
poses an acceptable risk to humans.
The BSE Inquiry / Statement No 19B (supplementary) Dr Alan Colchester
Issued 06/08/1999 (not scheduled to give oral evidence) SECOND STATEMENT TO THE
BSE INQUIRY Dr A Colchester BA BM BCh PhD FRCP Reader in Neurosciences &
Computing, University of Kent at Canterbury; Consultant Neurologist, Guy’s
Hospital London and William Harvey Hospital Ashford April 1999
snip...
88. Natural decay: Infectivity persists for a long time in the environment.
A study by Palsson in 1979 showed how scrapie was contracted by healthy sheep,
after they had grazed on land which had previously been grazed by
scrapie-infected sheep, even though the land had lain fallow for three years
before the healthy sheep were introduced. Brown also quoted an early experiment
of his own (1991), where he had buried scrapie-infected hamster brain and found
that he could still detect substantial infectivity three years later near where
the material had been placed. 89. Potential environmental routes of infection:
Brown discusses the various possible scenarios, including surface or subsurface
deposits of TSE-contaminated material, which would lead to a build-up of
long-lasting infectivity. Birds feeding on animal remains (such as gulls
visiting landfill sites) could disperse infectivity. Other animals could become
vectors if they later grazed on contaminated land. "A further question concerns
the risk of contamination of the surrounding water table or even surface water
channels, by effluents and discarded solid wastes from treatment plants. A
reasonable conclusion is that there is a potential for human infection to result
from environmental contamination by BSE-infected tissue residues. The potential
cannot be quantified because of the huge numbers of uncertainties and
assumptions that attend each stage of the disposal process". These comments,
from a long established authority on TSEs, closely echo my own statements which
were based on a recent examination of all the evidence. 90. Susceptibility: It
is likely that transmissibility of the disease to humans in vivo is probably
low, because sheep that die from scrapie and cattle that die from BSE are
probably a small fraction of the exposed population. However, no definitive data
are available.
91. Recommendations for disposal procedures: Brown recommends that material
which is actually or potentially contaminated by BSE should be: 1) exposed to
caustic soda; 2) thoroughly incinerated under carefully inspected conditions;
and 3) that any residue should be buried in landfill, to a depth which would
minimise any subsequent animal or human exposure, in areas that would not
intersect with any potable water-table source.
92. This review and recommendations from Brown have particular importance.
Brown is one of the world's foremost authorities on TSEs and is a senior
researcher in the US National Institutes of Health (NIH). It is notable that
such a respected authority is forthright in acknowledging the existence of
potential risks, and in identifying the appropriate measures necessary to
safeguard public health. Paper by SM Cousens, L Linsell, PG Smith, Dr M
Chandrakumar, JW Wilesmith, RSG Knight, M Zeidler, G Stewart, RG Will,
"Geographical distribution of variant CJD in the UK (excluding Northern
Ireland)". Lancet 353:18-21, 2 nd January 1999 93. The above paper {Appendix 41
(02/01/99)} (J/L/353/18) examined the possibility that patients with vCJD
(variant CJD) might live closer to rendering factories than would be expected by
chance. All 26 cases of vCJD in the UK with onset up to 31 st August 1998 were
studied. The incubation period of vCJD is not known but by analogy with other
human TSEs could lie within the range 5-25 years. If vCJD had arisen by exposure
to rendering products, such exposure might plausibly have occurred 8-10 years
before the onset of symptoms. The authors were able to obtain the addresses of
all rendering plants in the UK which were in production in 1988. For each case
of vCJD, the distance from the place of residence on 1st January 1998 to the
nearest rendering plant was calculated
snip...
Friday, February 08, 2013
*** Behavior of Prions in the Environment: Implications for Prion Biology
snip...
***SO, my question, why is it the OIE and other trade officials and policy
making there from, knowing that the USA and Canada, with not a clue about
Mexico, why is it that nobody has Declared an EXTRAORDINARY EMERGENCY DUE TO A
FOREIGN ANIMAL DISEASE TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION CHRONIC
WASTING DISEASE CWD IN THE UNITED STATES AND NORTH AMERICA ?
well, since no one else will, than I must.
Terry S. Singeltary Sr. Declares a DECLARATION OF EXTRAORDINARY EMERGENCY
DUE TO A FOREIGN ANIMAL DISEASE TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE
PRION CHRONIC WASTING DISEASE CWD IN THE UNITED STATES AND NORTH AMERICA.
The elephant in the room I was speaking of that we all have missed was the
feed, yes we all know of ruminant and non ruminant protein and risk factors
there from with TSE Prion disease, but we missed the rest of the feed i.e. FEED
GRAINS. YES, science has shown in the past, and now recently, the shedding of
the CWD TSE Prion into the environment is indeed a risk factor, and for all the
grains and such that goes into feed, even hay, hell, Norway does not require any
APHIS-Veterinary Services certification for the import of hay/straw. see for
yourself ;
Hay/Straw
Norway does not require any APHIS-Veterinary Services certification for the
import of hay/straw.
you add up all the other grains in feed, and then wonder about exposure to
the CWD TSE PRION from cervid and risk factor from the CWD there from via
shedding or right down to the soil these grains were grown in, and you have a
world of problems. see ;
Feed Grains Data: Yearbook Tables Created March 10, 2016 Updates of this
data, and data covering more years and countries, can be found at http://www.ers.usda.gov/data-products/feed-grains-database/feed-grains-yearbook-tables.aspx
U.S. Acreage, Production, Yield, and Farm Price Table 1--Corn, sorghum, barley,
and oats: Planted acreage, harvested acreage, production, yield, and farm price
World Production, Supply, and Disappearance Table 2--Foreign coarse grains:
Supply and disappearance Table 3--Feed grains (corn, sorghum, barley, and oats):
Supply and disappearance U.S. Supply and Disappearance Table 4--Corn: Supply and
disappearance Table 5--Sorghum: Supply and disappearance Table 6--Barley: Supply
and disappearance Table 7--Oats: Supply and disappearance U.S. Production,
Yield, and Stocks Table 8--Hay: Production, harvested acreage, yield, and stocks
Domestic and International Prices Table 9--Corn and sorghum: Average prices
received by farmers, United States Table 10--Barley and oats: Average prices
received by farmers, United States Table 11--Hay: Average prices received by
farmers, United States Table 12--Corn: Cash prices at principal markets Table
13--Sorghum: Cash prices at principal markets Table 14--Barley and oats: Cash
prices at principal markets Table 15--Feed-price ratios for livestock, poultry,
and milk Table 16--Byproduct feeds: Average wholesale price, bulk, specified
markets Table 17--Processed corn products: Quoted market prices Exports and
Imports Table 18--U.S. corn and sorghum exports Table 19--U.S. barley and oats
exports Table 20--U.S. corn and sorghum imports Table 21--U.S. barley and oats
imports Table 22--U.S. corn and sorghum exports by selected destinations Table
23--U.S. barley and oats exports by selected destinations Table 24--U.S. corn
and sorghum imports by selected sources Table 25--U.S. barley and oats imports
by selected sources Table 26--U.S. white corn exports by selected destinations
Table 27--World coarse grain trade: Selected exporters and importers by
commodity Rail rates and shipments Table 28--Rail rates and grain shipments
Processed feeds and animal unit indexes Table 29--Processed feeds: Quantities
fed and feed per grain-consuming animal unit Table 30--Indexes of feed consuming
animal units Feed, seed, and industrial uses Table 31—Corn: Feed, seed, and
industrial uses Exports and imports for ethyl alcohol and brewers’ and
distillers’ dregs and waste Table 32—U.S. exports of ethyl alcohol by selected
destinations Table 33—U.S. imports of ethyl alcohol by selected sources Table
34—U.S. exports of brewers’ and distillers’ dregs and waste by selected
commodities Table 35—U.S. imports of brewers’ and distillers’ dregs and waste by
selected sources Contact: Thomas Capehart at tcapehart+A25@ers.usda.gov
‘’The statement you were concerned about was corrected to "One sorghum DDGS
out of 168 DG samples was contaminated with animal protein prohibited for use in
ruminant feed and was channeled to poultry feed."
Subject: Re: TEXAS CONFIRMATION OF BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE
PRION IN ONE SAMPLE OF SORGHUM DDGS OUT OF 168 DG SAMPLES
***UDATED CORRECTION BY AUTHOR...SEE EMAIL TO ME...terry
From: Kyung-Min Lee Sent: Thursday, October 01, 2015 1:39 PM To: Terry S.
Singeltary Sr. ; BSE-L@LISTS.AEGEE.ORG Cc: CJD-L@LISTS.AEGEE.ORG ;
cjdvoice@yahoogroups.com ; bloodcjd@yahoogroups.com ;
jcattanach@foodprotection.org ; cnc3@psu.edu ; dloynachan@foodprotection.org ;
lhovey@foodprotection.org ; Timothy J. Herrman Subject: RE: TEXAS CONFIRMATION
OF BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION IN ONE SAMPLE OF SORGHUM DDGS
OUT OF 168 DG SAMPLES
Dear Terry S. Singeltary Sr.
Thank for your interest and concern about our published article entitled
“Evaluation of Selected Nutrients and Contaminants in Distillers Grains from
Ethanol Production in Texas”. I should apologize you and others that there were
some errors and misleading statements in this article due to inappropriate
terminology. The statement you were concerned about was corrected to "One
sorghum DDGS out of 168 DG samples was contaminated with animal protein
prohibited for use in ruminant feed and was channeled to poultry feed." We
requested the journal editor to correct some errors and the relevant statements,
or to withdraw the article from the journal.
Again I sincerely apologize for any confusion and inconvenience this may
cause. Thanks.
best wishes,
Kyung-Min
Kyung-Min Lee, Ph. D. Research Scientist Office of the Texas State Chemist
Texas A&M AgriLife Research P.O. Box 3160, College Station, TX
77841-3160 Phone: 979-845-4113 (ext 132) Email:kml@otsc.tamu.edu Fax:
979-845-1389
snip...end...tss
my link corrected
Sunday, September 27, 2015
TEXAS CONFIRMATION OF BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION IN ONE
SAMPLE OF SORGHUM DDGS OUT OF 168 DG SAMPLES
kind regards, terry
Subject: Fw: TEXAS CONFIRMATION OF BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE
PRION IN ONE SAMPLE OF SORGHUM DDGS OUT OF 168 DG SAMPLES
From: Terry S. Singeltary Sr. Sent: Sunday, September 27, 2015 4:39 PM To:
BSE-L@LISTS.AEGEE.ORG Cc: CJD-L@LISTS.AEGEE.ORG ; cjdvoice@yahoogroups.com ;
bloodcjd@yahoogroups.com ; jcattanach@foodprotection.org ; cnc3@psu.edu ;
dloynachan@foodprotection.org ; lhovey@foodprotection.org ; kml@otsc.tamu.edu
Subject: TEXAS CONFIRMATION OF BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION IN
ONE SAMPLE OF SORGHUM DDGS OUT OF 168 DG SAMPLES
TEXAS One sorghum DDGS sample out of 168 DG samples was contaminated with
bovine spongiform encephalopathy, but the transmission route of the bovine
spongiform encephalopathy agent could not be clearly defined.
J Food Prot. 2015 Oct;78(10):1861-9. doi: 10.4315/0362-028X.JFP-15-157.
Evaluation of Selected Nutrients and Contaminants in Distillers Grains from
Ethanol Production in Texas.
Lee KM1, Herrman TJ2. Author information 1Office of the Texas State
Chemist, Texas A&M AgriLife Research, Texas A&M University System,
College Station, Texas 77841, USA. kml@otsc.tamu.edu. 2Office of the Texas State
Chemist, Texas A&M AgriLife Research, Texas A&M University System,
College Station, Texas 77841, USA.
Abstract
This study evaluated distillers grain (DG) by-products produced in
different ethanol plants and supplemented in animal diets in Texas, based on
samples analyzed from 2008 to 2014. The samples were assessed for concentration,
occurrence, and prevalence of selected nutrients and contaminants. Protein and
sulfur contents of DG were largely different between corn and sorghum
by-products as well as wet distillers grain with solubles and dry distillers
grain with solubles (DDGS), indicating a significant effect of grain feedstock
and dry-grind process stream on DG composition and quality. Salmonella was
isolated in 4 DDGS samples out of a total of 157 DG samples, a percentage (2.5%)
that is much lower than the percentage of Salmonella-positive samples found in
other feed samples analyzed during the same period. A small amount of
virginiamycin residue was found in 24 corn DDGS, 1 corn wet distillers grain
with solubles, and 2 sorghum DDGS samples out of 242 samples in total. One
sorghum DDGS sample out of 168 DG samples was contaminated with bovine
spongiform encephalopathy, but the transmission route of the bovine spongiform
encephalopathy agent could not be clearly defined. The concentrations of
aflatoxin and fumonisin DG by-products averaged 3.4 μg/kg and 0.7 mg/kg,
respectively. Among contaminated corn DG samples, five DDGS samples for
aflatoxin contained a higher concentration than the U.S. Food and Drug
Administration action level for use in animal feed, whereas no sample for
fumonisin was found above the action level. The study results raised some
important issues associated with the quality and use of DG by-products,
suggesting several approaches and strategies for their effective and safe use as
a feed ingredient to promote animal and human health and welfare.
PMID: 26408135 [PubMed - in process]
Terry S. Singeltary Sr. Declares a DECLARATION OF EXTRAORDINARY EMERGENCY
DUE TO A FOREIGN ANIMAL DISEASE TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE
PRION CHRONIC WASTING DISEASE CWD IN THE UNITED STATES AND NORTH AMERICA.
it’s time someone steps up to the plate (OIE ...LMAO!), and declare an
extraordinary emergency for foreign animal disease due to Chronic Wasting
Disease or Cervid Spongiform Encephalopathy TSE Prion disease from the United
States of America, Canada, and Mexico i.e. North America, before this damn
disease is spread to hell and back, and you can just throw in there BSE and
Scrapie just for grins. ...and I ain’t grinning Sad smile OIE, you have
floundered too long with mad cow type TSE Prion disease...
snip...see full text ;
Sunday, July 17, 2016
*** CHRONIC WASTING DISEASE CWD TSE PRION GLOBAL REPORT UPDATE JULY 17 2016
***
Tuesday, April 12, 2016
The first detection of Chronic Wasting Disease (CWD) in Europe free-ranging
reindeer from the Nordfjella population in South-Norway.
Tuesday, June 14, 2016
*** Chronic Wasting Disease (CWD) in a moose from Selbu in Sør-Trøndelag
Norway ***
Thursday, July 07, 2016
Norway reports a third case Chronic Wasting Disease CWD TSE Prion in 2nd
Norwegian moose
14/06/2016 - Norway reports a third case
Saturday, July 16, 2016
Chronic wasting Disease in Deer (CWD or Spongiform Encephalopathy) The
British Deer Society 07/04/2016
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics
of BSE in Canada Singeltary reply ;
Saturday, January 31, 2015
European red deer (Cervus elaphus elaphus) are susceptible to Bovine
Spongiform Encephalopathy BSE by Oral Alimentary route
I strenuously once again urge the FDA and its industry constituents, to
make it MANDATORY that all ruminant feed be banned to all ruminants, and this
should include all cervids as soon as possible for the following
reasons...
======
In the USA, under the Food and Drug Administrations BSE Feed Regulation (21
CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from
deer and elk is prohibited for use in feed for ruminant animals. With regards to
feed for non-ruminant animals, under FDA law, CWD positive deer may not be used
for any animal feed or feed ingredients. For elk and deer considered at high
risk for CWD, the FDA recommends that these animals do not enter the animal feed
system.
***However, this recommendation is guidance and not a requirement by law.
======
31 Jan 2015 at 20:14 GMT
*** Ruminant feed ban for cervids in the United States? ***
31 Jan 2015 at 20:14 GMT
see Singeltary comment ;
Docket No. FDA-2003-D-0432 (formerly 03D-0186) Use of Material from Deer
and Elk in Animal Feed Singeltary Submission
*** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary
Sr. Submission ***
Monday, November 16, 2015
*** Docket No. APHIS-2007-0127 Scrapie in Sheep and Goats Terry Singeltary
Sr. Submission ***
*** Transmissible Spongiform Encephalopathy TSE PRION UPDATE USA update
2016 ***
Saturday, July 23, 2016
*** BOVINE SPONGIFORM ENCEPHALOPATHY BSE TSE PRION SURVEILLANCE, TESTING,
AND SRM REMOVAL UNITED STATE OF AMERICA UPDATE JULY 2016
Tuesday, July 26, 2016
*** Atypical Bovine Spongiform Encephalopathy BSE TSE Prion UPDATE JULY
2016
Monday, June 20, 2016
*** Specified Risk Materials SRMs BSE TSE Prion Program
Saturday, July 16, 2016
*** Importation of Sheep, Goats, and Certain Other Ruminants [Docket No.
APHIS-2009-0095]RIN 0579-AD10
*** WITH great disgust and concern, I report to you that the OIE, USDA,
APHIS, are working to further legalize the trading of Transmissible Spongiform
Encephalopathy TSE Pion disease around the globe.
THIS is absolutely insane. it’s USDA INC.
Wednesday, May 25, 2016
USDA APHIS National Scrapie TSE Prion Eradication Program April 2016
Monthly Report Prion 2016 Tokyo Update
*** Transmission data also revealed that several scrapie prions propagate
in HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the
efficiency of transmission at primary passage was low, subsequent passages
resulted in a highly virulent prion disease in both Met129 and Val129 mice.
Transmission of the different scrapie isolates in these mice leads to the
emergence of prion strain phenotypes that showed similar characteristics to
those displayed by MM1 or VV2 sCJD prion. These results demonstrate that scrapie
prions have a zoonotic potential and raise new questions about the possible link
between animal and human prions.
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
TOKYO
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X
Monday, May 02, 2016
*** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo ***
Friday, July 29, 2016
IOWA CHRONIC WASTING DISEASE CWD TSE PRION TOTAL TO DATE 304 CASES WILD AND
CAPTIVE REPORT UPDATE JULY 2016
Wednesday, July 27, 2016
Arkansas CWD 101 positive cases documented to date, Biologists to take
additional samples in in southern Pope County, Aug. 1-5
Friday, July 01, 2016
TEXAS Thirteen new cases of chronic wasting disease (CWD) were confirmed at
a Medina County captive white-tailed deer breeding facility on June 29,
2016
*** How Did CWD Get Way Down In Medina County, Texas?
DISCUSSION Observations of natural outbreaks of scrapie indicated that the
disease spread from flock to flock by the movement of infected, but apparently
normal, sheep which were incubating the disease.
There was no evidence that the disease spread to adjacent flocks in the
absent of such movements or that vectors or other host species were involved in
the spread of scrapie to sheep or goats; however, these possibilities should be
kept open...
Tuesday, June 07, 2016
*** Comparison of two US sheep scrapie isolates supports identification as
separate strains ***
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Monday, July 18, 2016
Texas Parks Wildlife Dept TPWD HIDING TSE (CWD) in Deer Herds, Farmers
Sampling Own Herds, Rapid Testing, False Negatives, a Recipe for Disaster
Sunday, July 17, 2016
*** CHRONIC WASTING DISEASE CWD TSE PRION GLOBAL REPORT UPDATE JULY 17 2016
Saturday, May 28, 2016
*** Infection and detection of PrPCWD in soil from CWD infected farm in
Korea Prion 2016 Tokyo ***
*** NIH awards $11 million to UTHealth researchers to study deadly CWD
prion diseases Claudio Soto, Ph.D. ***
Public Release: 29-Jun-2016
Tuesday, July 12, 2016
Chronic Wasting Disease CWD, Scrapie, Bovine Spongiform Encephalopathy BSE,
TSE, Prion Zoonosis Science History
see history of NIH may destroy human brain collection
Monday, August 1, 2016
USDA Announces Reopening of Brazilian Market to U.S. Beef Exports and the
Potential for Transmissible Spongiform Encephalopathy TSE prion disease
Release No. 0175.16
Monday, April 11, 2016
*** DECLARATION OF EXTRAORDINARY EMERGENCY DUE TO A FOREIGN ANIMAL DISEASE
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION CHRONIC WASTING DISEASE CWD IN
THE UNITED STATES AND NORTH AMERICA ?
or, how the USDA et al employees out BSE TSE Prion testing, and this is not
the first time either ;
Wednesday, March 2, 2016
*** RANCHO He did not know that they were placing healthy cow heads next to
suspect carcasses BSE TSE Prion ***
or what Dr. Paul Brown of NIH said;
"The fact the Texas cow showed up fairly clearly implied the existence of
other undetected cases," Dr. Paul Brown, former medical director of the National
Institutes of Health's Laboratory for Central Nervous System Studies and an
expert on mad cow-like diseases, told United Press International. "The question
was, 'How many?' and we still can't answer that." Brown, who is preparing a
scientific paper based on the latest two mad cow cases to estimate the maximum
number of infected cows that occurred in the United States, said he has
"absolutely no confidence in USDA tests before one year ago" because of the
agency's reluctance to retest the Texas cow that initially tested positive.
USDA officials finally retested the cow and confirmed it was infected seven
months later, but only at the insistence of the agency's inspector
general.
"Everything they did on the Texas cow makes everything they did before 2005
suspect," Brown said.
or former Ag Secretary Ann Veneman;
Thursday, October 22, 2015
*** Former Ag Secretary Ann Veneman talks women in agriculture and we talk
mad cow disease USDA and what really happened ***
Thursday, January 14, 2016
*** EMERGING ANIMAL DISEASES Actions Needed to Better Position USDA to
Address Future Risks Report to the Chairman, Committee on Energy and Commerce,
House of Representatives December 2015 GAO-16-132
GAO
I remember back in 2012 that the OIE was recommended that Scrapie be
delisted. see ;
OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED AND SAME OLD BSe
WITH BOVINE MAD COW DISEASE
A_Annex_VII_A_AHG_Report_Meeting_Final
Scrapie – The disease does not show significant morbidity (2-30%
within-flock morbidity) or mortality and is not zoonotic. However, the Group
noted the difficulty in evaluating the level of morbidity for diseases with a
long incubation period such as scrapie. The Group recommended that the disease
be delisted.
Chapter 1.6.
PROCEDURES FOR SELF DECLARATION AND FOR OFFICIAL RECOGNITION BY the OIE
Article 1.6.1.
[No change]
Article 1.6.2.
[No change]
Article 1.6.3.
Questionnaire on bovine spongiform encephalopathy
SNIP...
Article 11.5.29.
Conclusions of the risk assessment
The overall risk of BSE in the cattle population of a country or zone is
proportional to the level of known or potential exposure to BSE infectivity and
the potential for recycling and amplification of the infectivity through
livestock feeding practices. For the risk assessment to conclude that the cattle
population of a country or zone is free from BSE risk, it should have
demonstrated that appropriate measures have been taken to manage any risks
identified.
Thursday, December 20, 2012
*** OIE GROUP RECOMMENDS THAT SCRAPE PRION DISEASE BE DELISTED, WISHES TO
CONTINUE SPREADING IT AROUND THE GLOBE
Monday, November 30, 2009
*** USDA AND OIE COLLABORATE TO EXCLUDE ATYPICAL SCRAPIE NOR-98 ANIMAL
HEALTH CODE, DOES NOT SURPRISE ME $
there is great concern now for risk to humans from Scrapie, course, there
always was science showing these risk factors, just that governments chose to
ignore the science ;
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
Also, I have tried for over a decade or more to get the OIE to recognize
CWD as serious risk factor to humans and animals ;
From: Stuart MacDiarmid (Stuart)
Sent: Monday, May 12, 2014 5:12 PM
To: Terry S. Singeltary Sr. Cc: Stuart MacDiarmid (Stuart) ; Alex Thiermann
Subject: RE: Member Country’s detailed justification for listing of chronic
wasting disease of cervids (CWD) against the criteria of Article 1.2.2., the
Code Commission _recommended_ this disease be reconsidered for listing.
Dear Mr Singeltary, I apologise for the lengthy delay in replying to your
email; I have been unwell. I assume that you are writing to me in my capacity as
an elected member of the OIE's Terrestrial Animal Health Standards Commission.
On more than one occasion our Commission has received a request from a Member
Country to list CWD as a disease notifiable to the OIE. However, it is not our
practice to specify which Member Countries make specific requests to us. All
countries which submit national comments to us at our February and September
meetings are listed in the reports of our meetings. However, the country names
are not linked to specific comments or requests. The most recent submissions on
CWD, in February 2013 and September 2013, were in response to the work of an ad
hoc group which met in 2012 to examine all currently listed diseases against the
recently-adopted criteria for listing published in the Terrestrial Animal Health
Code. The report of that ad hoc group and its recommendations were circulated
for Member Country comments in the report of the Terrestrial Animal Health
Standards Commission. The ad hoc group had recommended the delisting of certain
diseases because they do not meet the criteria for listing. However, in
commenting on the recommendations, a Member Country made a submission proposing
the listing of CWD. That was in February 2013. The same country made the same
proposal to the September meeting of the Terrestrial Animal Health Standards
Commission (TAHSC). The TAHSC passed the recommendation to the Director General
of the OIE with a request that next time an ad hoc expert group is convened to
consider issues of listing or delisting they may also evaluate CWD against the
OIE's criteria. That is where the situation stands at present. Next time an ad
hoc group is convened to consider issues of listing and delisting, CWD will be
evaluated. I have no idea of time frames. Because I am off work I do not have
access to my usual resources, otherwise I could have given you URLs to the
various reports and also the Terrestrial Animal Health Code chapter which gives
the criteria for listing a disease as notifiable to the OIE. However, I think if
you search the OIE web site you should be able to find everything. Regards,
Stuart C MacDiarmid Terrestrial Animal Health Standards Commission
--------------------------------------------------------------------------------
From: Terry S. Singeltary Sr. [flounder9@verizon.net]
Sent: Thursday, 8 May 2014 10:07 a.m.
To: Stuart MacDiarmid (Stuart)
Subject: Member Country’s detailed justification for listing of chronic
wasting disease of cervids (CWD) against the criteria of Article 1.2.2., the
Code Commission _recommended_ this disease be reconsidered for listing.
Subject: Member Country’s detailed justification for listing of chronic
wasting disease of cervids (CWD) against the criteria of Article 1.2.2., the
Code Commission _recommended_ this disease be reconsidered for listing.
> In response to a _Member Country’s_ detailed justification for listing
of chronic wasting disease of cervids (CWD) against the criteria of Article
1.2.2., the Code Commission _recommended_ this disease be reconsidered for
listing.
Annual report of the Scientific Network on BSE-TSE EFSA, Question No
EFSA-Q-2013-01004, approved on 11 December 2013 *** Further, it was addressed
that recently discussions have being held at OIE level on Chronic Wasting
Disease of cervids. page 6; http://www.efsa.europa.eu/en/supporting/doc/532e.pdf
Greetings Prof. Stuart MacDiarmid,
I kindly wish to ask a question please. as you know, I have been very
concerned since 1997, of the TSE prion disease, since the death of my mother to
the hvCJD. I have watched keenly the spread of cwd in North America and Korea.
In the links above, it states that there was a possibility that CWD Chronic
Wasting Disease of cervids, was to be brought to the table for possible listing
by OIE, and that some Country had brought this to the table for reasons I am not
sure about. wisely so though.
could you please tell me which Country brought this to the table and for
what reasons ?
and since the code commission recommended this, what happened since then
?
was it finally listed ?
if not, what were the reasons for not listing it ?
Thank You Kindly,
terry
Dear Mr Singeltary,
Further to Professor MacDiarmid’s reply, and in acknowledgement of the same
email sent to a variety of OIE recipients, including Dr Vallat, the urls to the
Terrestrial Animal Health Standards Commission reports that Professor MacDiarmid
refers to are:
February 2013 (Item 5 Criteria for listing diseases):
September 2013 (Item 5 Criteria for listing diseases):
Thank you for your interest and support of OIE’s work.
Yours sincerely
OIE International Trade Department
Thank you for the links, and Thank you for reconsidering CWD as a listed
disease.
I am writing up a report on cwd TSE prion disease, in North America and the
USA, risk factors there from, and game farms, I will be submitting it to you at
a later date for consideration or just for your files later...thanks again,
terry
snip...end...tss
From: Terry S. Singeltary Sr. [mailto:flounder9@verizon.net]
Sent: 23 November 2013 03:39
To: OfficialReport
Cc: Public Information
Subject: Fw: Wasting disease is threat to the entire UK deer
population
snip...end...tss
======================================
Monday, May 05, 2014
Member Country details for listing OIE CWD 2013 against the criteria of
Article 1.2.2., the Code Commission recommends consideration for listing
Tuesday, July 17, 2012
O.I.E. BSE, CWD, SCRAPIE, TSE PRION DISEASE Final Report of the 80th
General Session, 20 - 25 May 2012
*** URGENT CWD UPDATE Friday, January 17, 2014
FINALLY, 12 years later, the OIE becomes concerned with CWD to humans, not
that I did not try and warn them 12 years ago. ...kind regards, terry
Friday, January 17, 2014
Annual report of the Scientific Network on BSE-TSE EFSA, Question No
EFSA-Q-2013-01004, approved on 11 December 2013
*** Further, it was addressed that recently discussions have being held at
OIE level on Chronic Wasting Disease of cervids.
2002 Singeltary vs O.I.E. on CWD to human risk factor ;
Subject: Re: CWD AMERICA ???
Date: Fri, 12 Jul 2002 19:10:18 +0200
From: "INFORMATION DEPT"
Organization: O.I.E
To: "Terry S. Singeltary Sr."
References: <3d2f0169 .3="" wt.net=""> < 012901c229b2 ad43bb90=""
f00000a=""> 3D2F2358.5010700@wt.net
I agree with you Dr Terry. The OIE, namely the International Animal Health
Code Commission is working on making proposals to Member Countries to change the
OIE lists so to avoid some the problems mentioned in you e-mail. This will take
at least two years before adoption by the International Committee. For BSE,
countries asked the OIE to post information on BSE on the OIE web site.
Personally, I am interested in Chronic Wasting Disease and I follow what is
distributed through ProMed. Delegates of OIE Member Countries can propose
diseases to be added to the list.
Kind regards.
Karim Ben Jebara
----- Original Message -----
From: "Terry S. Singeltary Sr."
To: "INFORMATION DEPT"
Sent: Friday, July 12, 2002 8:43 PM
Subject: Re: CWD AMERICA ???
>>> *** Further, it was addressed that recently discussions have
being held at OIE level on Chronic Wasting Disease of cervids. <<<
> hello Dr. Jebara,
> > many thanks for your swift and kind reply.
> > if i am not mistaken, it was the same email address.
> it was 3 or 4 weeks ago i wrote, as it is, i don't
> save 'sent' emails anymore, unless very important.
> > my main concern (besides the fact that a potential TSE
> has been in the USA cattle for some time, but the APHIS
> do not test to find), is that the CWD could very well be
> transmitting to humans, and i just did not see to much
> posted about it on OIE site.
> > > Coming back to your question, Chronic Wasting Disease is not
an OIE
> > > listed disease. Please see OIE disease lists at
> > why is this TSE (CWD) not listed and followed as with BSE ?
> > Article 1.1.3.2.
> 1. Countries shall make available to other countries, through the
> OIE, whatever information is necessary to minimise the spread of
> important animal diseases and to assist in achieving better worldwide
> control of these diseases.
> > The USA CWD is an important animal disease.
> > why is it not followed?
> > > The decision to add or delete a disease from the OIE lists,
come
> > > through proposals made by Member Countries and it has to be
adopted by
> > > the International Committee.
> > i _urgently_ suggest a proposal to the OIE to follow this disease
very
> closely, and to propose _more_ testing in the USA for TSEs in the USA
> cattle...
> > kindest regards,
> terry
> > INFORMATION DEPT wrote:
> > > Dear Sir,
> > > > This is the first time that I receive your e-mail. To
whom have you written
> > in the OIE or to which address?
> > > > Coming back to your question, Chronic Wasting Disease
is not an OIE listed
> > disease. Please see OIE disease lists at
> > > > Countries should report to the OIE any disease even is
not listed in the
> > OIE's lists in some conditions (example: an exceptional
epidemiological
> > event). Please read Chapter 1.1.3 of the International animal
health code to
> > have more information on disease notification and epidemiological
> > information agreed by OIE Member Countries at :
> > > > The decision to add or delete a disease from the OIE
lists, come through
> > proposals made by Member Countries and it has to be adopted by
the
> > International Committee.
> > > > Hope that I answered to your question.
> > > > Best regards.
> > > > Dr Karim Ben Jebara
> > Head
> > Animal Health Information Department
> > OIE
> > > > > > > > ----- Original Message -----
> > From: "Terry S. Singeltary Sr."
> > To:
> > Sent: Friday, July 12, 2002 6:18 PM
> > Subject: CWD AMERICA ???
> > > > > > > >>I WROTE TO OIE RECENTLY ASKING
'WHY OIE DOES NOT FOLLOW CWD IN
> >>AMERICA' ? with no reply ? i am still seeking an answer ?
> >> > >>many thanks,
> >>and kind regards,
> >>terry =====================
SNIP...SEE FULL REPORT HERE ;
Friday, January 17, 2014
Annual report of the Scientific Network on BSE-TSE EFSA, Question No
EFSA-Q-2013-01004, approved on 11 December 2013
*** CHRONIC WASTING DISEASE TSE PRION ZOONOSIS 2016 ***
PRION 2016 TOKYO
Zoonotic Potential of CWD Prions: An Update
Ignazio Cali1, Liuting Qing1, Jue Yuan1, Shenghai Huang2, Diane Kofskey1,3,
Nicholas Maurer1, Debbie McKenzie4, Jiri Safar1,3,5, Wenquan Zou1,3,5,6,
Pierluigi Gambetti1, Qingzhong Kong1,5,6
1Department of Pathology, 3National Prion Disease Pathology Surveillance
Center, 5Department of Neurology, 6National Center for Regenerative Medicine,
Case Western Reserve University, Cleveland, OH 44106, USA.
4Department of Biological Sciences and Center for Prions and Protein
Folding Diseases, University of Alberta, Edmonton, Alberta, Canada,
2Encore Health Resources, 1331 Lamar St, Houston, TX 77010
Chronic wasting disease (CWD) is a widespread and highly transmissible
prion disease in free-ranging and captive cervid species in North America. The
zoonotic potential of CWD prions is a serious public health concern, but the
susceptibility of human CNS and peripheral organs to CWD prions remains largely
unresolved. We reported earlier that peripheral and CNS infections were detected
in transgenic mice expressing human PrP129M or PrP129V. Here we will present an
update on this project, including evidence for strain dependence and influence
of cervid PrP polymorphisms on CWD zoonosis as well as the characteristics of
experimental human CWD prions.
PRION 2016 TOKYO
In Conjunction with Asia Pacific Prion Symposium 2016
PRION 2016 Tokyo
Prion 2016
Prion 2016
Purchase options Price * Issue Purchase USD 198.00
Cervid to human prion transmission
Kong, Qingzhong
Case Western Reserve University, Cleveland, OH, United States
Abstract
Prion disease is transmissible and invariably fatal. Chronic wasting
disease (CWD) is the prion disease affecting deer, elk and moose, and it is a
widespread and expanding epidemic affecting 22 US States and 2 Canadian
provinces so far. CWD poses the most serious zoonotic prion transmission risks
in North America because of huge venison consumption (>6 million deer/elk
hunted and consumed annually in the USA alone), significant prion infectivity in
muscles and other tissues/fluids from CWD-affected cervids, and usually high
levels of individual exposure to CWD resulting from consumption of the affected
animal among often just family and friends. However, we still do not know
whether CWD prions can infect humans in the brain or peripheral tissues or
whether clinical/asymptomatic CWD zoonosis has already occurred, and we have no
essays to reliably detect CWD infection in humans. We hypothesize that:
(1) The classic CWD prion strain can infect humans at low levels in the
brain and peripheral lymphoid tissues;
(2) The cervid-to-human transmission barrier is dependent on the cervid
prion strain and influenced by the host (human) prion protein (PrP) primary
sequence;
(3) Reliable essays can be established to detect CWD infection in
humans;and
(4) CWD transmission to humans has already occurred. We will test these
hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary in
vitro approaches.
Aim 1 will prove that the classical CWD strain may infect humans in brain
or peripheral lymphoid tissues at low levels by conducting systemic bioassays in
a set of "humanized" Tg mouse lines expressing common human PrP variants using a
number of CWD isolates at varying doses and routes. Experimental "human CWD"
samples will also be generated for Aim 3.
Aim 2 will test the hypothesis that the cervid-to-human prion transmission
barrier is dependent on prion strain and influenced by the host (human) PrP
sequence by examining and comparing the transmission efficiency and phenotypes
of several atypical/unusual CWD isolates/strains as well as a few prion strains
from other species that have adapted to cervid PrP sequence, utilizing the same
panel of humanized Tg mouse lines as in Aim 1.
Aim 3 will establish reliable essays for detection and surveillance of CWD
infection in humans by examining in details the clinical, pathological,
biochemical and in vitro seeding properties of existing and future experimental
"human CWD" samples generated from Aims 1-2 and compare them with those of
common sporadic human Creutzfeldt-Jakob disease (sCJD) prions.
Aim 4 will attempt to detect clinical CWD-affected human cases by examining
a significant number of brain samples from prion-affected human subjects in the
USA and Canada who have consumed venison from CWD-endemic areas utilizing the
criteria and essays established in Aim 3. The findings from this proposal will
greatly advance our understandings on the potential and characteristics of
cervid prion transmission in humans, establish reliable essays for CWD zoonosis
and potentially discover the first case(s) of CWD infection in humans.
Public Health Relevance There are significant and increasing human exposure
to cervid prions because chronic wasting disease (CWD, a widespread and highly
infectious prion disease among deer and elk in North America) continues
spreading and consumption of venison remains popular, but our understanding on
cervid-to-human prion transmission is still very limited, raising public health
concerns. This proposal aims to define the zoonotic risks of cervid prions and
set up and apply essays to detect CWD zoonosis using mouse models and in vitro
methods. The findings will greatly expand our knowledge on the potentials and
characteristics of cervid prion transmission in humans, establish reliable
essays for such infections and may discover the first case(s) of CWD infection
in humans.
Funding Agency Agency National Institute of Health (NIH)
Institute National Institute of Neurological Disorders and Stroke (NINDS)
Type Research Project (R01)
Project # 1R01NS088604-01A1
Application # 9037884
Study Section Cellular and Molecular Biology of Neurodegeneration Study
Section (CMND)
Program Officer Wong, May
Project Start 2015-09-30
Project End 2019-07-31
Budget Start 2015-09-30
Budget End 2016-07-31
Support Year 1
Fiscal Year 2015
Total Cost $337,507
Indirect Cost $118,756
Institution
Name Case Western Reserve University
Department Pathology
Type Schools of Medicine
DUNS # 077758407
City Cleveland
State OH
Country United States
Zip Code 44106
===========================================================
We hypothesize that:
(1) The classic CWD prion strain can infect humans at low levels in the
brain and peripheral lymphoid tissues;
(2) The cervid-to-human transmission barrier is dependent on the cervid
prion strain and influenced by the host (human) prion protein (PrP) primary
sequence;
(3) Reliable essays can be established to detect CWD infection in
humans;and
(4) *** CWD transmission to humans has already occurred. *** We will test
these hypotheses in 4 Aims using transgenic (Tg) mouse models and complementary
in vitro approaches.
============================================================
Key Molecular Mechanisms of TSEs
Zabel, Mark D.
Colorado State University-Fort Collins, Fort Collins, CO, United States
Abstract Prion diseases, or transmissible spongiform encephalopathies (TSEs),
are fatal neurodegenerative diseases affecting humans, cervids, bovids, and
ovids. The absolute requirement of PrPC expression to generate prion diseases
and the lack of instructional nucleic acid define prions as unique infectious
agents. Prions exhibit species-specific tropism, inferring that unique prion
strains exist that preferentially infct certain host species and confront
transmission barriers to heterologous host species. However, transmission
barriers are not absolute. Scientific consensus agrees that the sheep TSE
scrapie probably breached the transmission barrier to cattle causing bovine
spongiform encephalopathy that subsequently breached the human transmission
barrier and likely caused several hundred deaths by a new-variant form of the
human TSE Creutzfeldt-Jakob disease in the UK and Europe. The impact to human
health, emotion and economies can still be felt in areas like farming, blood and
organ donations and the threat of a latent TSE epidemic. This precedent raises
the real possibility of other TSEs, like chronic wasting disease of cervids,
overcoming similar human transmission barriers. A groundbreaking discovery made
last year revealed that mice infected with heterologous prion strains facing
significant transmission barriers replicated prions far more readily in spleens
than brains6. Furthermore, these splenic prions exhibited weakened transmission
barriers and expanded host ranges compared to neurogenic prions. These data
question conventional wisdom of avoiding neural tissue to avoid prion
xenotransmission, when more promiscuous prions may lurk in extraneural tissues.
Data derived from work previously funded by NIH demonstrate that Complement
receptors CD21/35 bind prions and high density PrPC and differentially impact
prion disease depending on the prion isolate or strain used. Recent advances in
live animal and whole organ imaging have led us to generate preliminary data to
support novel, innovative approaches to assessing prion capture and transport.
We plan to test our unifying hypothesis for this proposal that CD21/35 control
the processes of peripheral prion capture, transport, strain selection and
xenotransmission in the following specific aims.
1. Assess the role of CD21/35 in splenic prion strain selection and host
range expansion.
2. Determine whether CD21/35 and C1q differentially bind distinct prion
strains
3. Monitor the effects of CD21/35 on prion trafficking in real time and
space
4. Assess the role of CD21/35 in incunabular prion trafficking
Public Health Relevance Transmissible spongiform encephalopathies, or prion
diseases, are devastating illnesses that greatly impact public health,
agriculture and wildlife in North America and around the world. The impact to
human health, emotion and economies can still be felt in areas like farming,
blood and organ donations and the threat of a latent TSE epidemic. This
precedent raises the real possibility of other TSEs, like chronic wasting
disease (CWD) of cervids, overcoming similar human transmission barriers. Early
this year Canada reported its first case of BSE in over a decade audits first
case of CWD in farmed elk in three years, underscoring the need for continued
vigilance and research. Identifying mechanisms of transmission and zoonoses
remains an extremely important and intense area of research that will benefit
human and other animal populations.
Funding Agency Agency National Institute of Health (NIH)
Institute National Institute of Allergy and Infectious Diseases (NIAID)
Type High Priority, Short Term Project Award (R56)
Project # 1R56AI122273-01A1
Application # 9211114
Study Section Cellular and Molecular Biology of Neurodegeneration Study
Section (CMND)
Program Officer Beisel, Christopher E
Project Start 2016-02-16
Project End 2017-01-31
Budget Start 2016-02-16
Budget End 2017-01-31
Support Year 1
Fiscal Year 2016
Total Cost
Indirect Cost Institution Name Colorado State University-Fort Collins
Department Microbiology/Immun/Virology
Type Schools of Veterinary Medicine
DUNS # 785979618 City Fort Collins
State CO
Country United States
Zip Code 80523
PMCA Detection of CWD Infection in Cervid and Non-Cervid Species
Hoover, Edward Arthur
Colorado State University-Fort Collins, Fort Collins, CO, United States
Abstract Chronic wasting disease (CWD) of deer and elk is an emerging highly
transmissible prion disease now recognized in 18 States, 2 Canadian provinces,
and Korea. We have shown that Infected deer harbor and shed high levels of
infectious prions in saliva, blood, urine, and feces, and in the tissues
generating those body fluids and excreta, thereby leading to facile transmission
by direct contact and environmental contamination. We have also shown that CWD
can infect some non-cervid species, thus the potential risk CWD represents to
domestic animal species and to humans remains unknown. Whether prions borne in
blood, saliva, nasal fluids, milk, or excreta are generated or modified in the
proximate peripheral tissue sites, may differ in subtle ways from those
generated in brain, or may be adapted for mucosal infection remain open
questions. The increasing parallels in the pathogenesis between prion diseases
and human neurodegenerative conditions, such as Alzheimer's and Parkinson's
diseases, add relevance to CWD as a transmissible protein misfolding disease.
The overall goal of this work is to elucidate the process of CWD prion
transmission from mucosal secretory and excretory tissue sites by addressing
these questions: (a) What are the kinetics and magnitude of CWD prion shedding
post-exposure? (b) Are excreted prions biochemically distinct, or not, from
those in the CNS? (c) Are peripheral epithelial or CNS tissues, or both, the
source of excreted prions? and (d) Are excreted prions adapted for horizontal
transmission via natural/trans-mucosal routes? The specific aims of this
proposal are: (1) To determine the onset and consistency of CWD prion shedding
in deer and cervidized mice; (2); To compare the biochemical and biophysical
properties of excretory vs. CNS prions; (3) To determine the capacity of
peripheral tissues to support replication of CWD prions; (4) To determine the
protease- sensitive infectious fraction of excreted vs. CNS prions; and (5) To
compare the mucosal infectivity of excretory vs. CNS prions. Understanding the
mechanisms that enable efficient prion dissemination and shedding will help
elucidate how horizontally transmissible prions evolve and succeed, and is the
basis of this proposal. Understanding how infectious misfolded proteins (prions)
are generated, trafficked, shed, and transmitted will aid in preventing,
treating, and managing the risks associated with these agents and the diseases
they cause.
Public Health Relevance Chronic wasting disease (CWD) of deer and elk is an
emergent highly transmissible prion disease now recognized throughout the USA as
well as in Canada and Korea. We have shown that infected deer harbor and shed
high levels of infectious prions in saliva, blood, urine, and feces thereby
leading to transmission by direct contact and environmental contamination. In
that our studies have also shown that CWD can infect some non-cervid species,
the potential risk CWD may represents to domestic animal species and humans
remains unknown. The increasing parallels in the development of major human
neurodegenerative conditions, such as Alzheimer's and Parkinson's diseases, and
prion diseases add relevance to CWD as a model of a transmissible protein
misfolding disease. Understanding how infectious misfolded proteins (prions) are
generated and transmitted will aid in interrupting, treating, and managing the
risks associated with these agents and the diseases they cause.
Funding Agency Agency National Institute of Health (NIH)
Institute National Institute of Neurological Disorders and Stroke (NINDS)
Type Research Project (R01)
Project # 4R01NS061902-07
Application # 9010980
Study Section Cellular and Molecular Biology of Neurodegeneration Study
Section (CMND)
Program Officer Wong, May Project Start 2009-09-30
Project End 2018-02-28
Budget Start 2016-03-01
Budget End 2017-02-28
Support Year 7
Fiscal Year 2016
Total Cost $409,868
Indirect Cost $134,234 Institution Name Colorado State University-Fort
Collins
Department Microbiology/Immun/Virology
Type Schools of Veterinary Medicine
DUNS # 785979618 City Fort Collins
State CO
Country United States
Zip Code 80523
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
Taylor & Francis
Prion 2016 Animal Prion Disease Workshop Abstracts
WS-01: Prion diseases in animals and zoonotic potential
Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a.
Vincent Beringue c. Patricia Aguilar a,
Natalia Fernandez-Borges a. and Alba Marin-Moreno a
"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos,
Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT.
Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas.
France
Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated
bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD)
disease in human. To date, BSE agent is the only recognized zoonotic prion.
Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that
have been circulating for centuries in farmed ruminants there is no apparent
epidemiological link between exposure to ruminant products and the occurrence of
other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD).
However, the zoonotic potential of the diversity of circulating TSE agents has
never been systematically assessed. The major issue in experimental assessment
of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the
biological phenomenon that limits TSE agents’ propagation from a species to
another. In the last decade, mice genetically engineered to express normal forms
of the human prion protein has proved essential in studying human prions
pathogenesis and modeling the capacity of TSEs to cross the human species
barrier.
To assess the zoonotic potential of prions circulating in farmed ruminants,
we study their transmission ability in transgenic mice expressing human PrPC
(HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC
(129Met or 129Val) are used to determine the role of the Met129Val dimorphism in
susceptibility/resistance to the different agents.
These transmission experiments confirm the ability of BSE prions to
propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be
susceptible to BSE in sheep or goat to a greater degree than the BSE agent in
cattle and that these agents can convey molecular properties and
neuropathological indistinguishable from vCJD. However homozygous 129V mice are
resistant to all tested BSE derived prions independently of the originating
species suggesting a higher transmission barrier for 129V-PrP variant.
Transmission data also revealed that several scrapie prions propagate in
HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the
efficiency of transmission at primary passage was low, subsequent passages
resulted in a highly virulent prion disease in both Met129 and Val129 mice.
Transmission of the different scrapie isolates in these mice leads to the
emergence of prion strain phenotypes that showed similar characteristics to
those displayed by MM1 or VV2 sCJD prion. These results demonstrate that scrapie
prions have a zoonotic potential and raise new questions about the possible link
between animal and human prions.
LOOKING FOR CWD IN HUMANS AS nvCJD or as an ATYPICAL CJD, LOOKING IN ALL
THE WRONG PLACES $$$
*** These results would seem to suggest that CWD does indeed have zoonotic
potential, at least as judged by the compatibility of CWD prions and their human
PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests
that if zoonotic CWD occurred, it would most likely effect those of the PRNP
codon 129-MM genotype and that the PrPres type would be similar to that found in
the most common subtype of sCJD (MM1).***
PRION 2015 CONFERENCE FT. COLLINS CWD RISK FACTORS TO HUMANS
*** LATE-BREAKING ABSTRACTS PRION 2015 CONFERENCE ***
O18
Zoonotic Potential of CWD Prions
Liuting Qing1, Ignazio Cali1,2, Jue Yuan1, Shenghai Huang3, Diane Kofskey1,
Pierluigi Gambetti1, Wenquan Zou1, Qingzhong Kong1 1Case Western Reserve
University, Cleveland, Ohio, USA, 2Second University of Naples, Naples, Italy,
3Encore Health Resources, Houston, Texas, USA
*** These results indicate that the CWD prion has the potential to infect
human CNS and peripheral lymphoid tissues and that there might be asymptomatic
human carriers of CWD infection.
==================
***These results indicate that the CWD prion has the potential to infect
human CNS and peripheral lymphoid tissues and that there might be asymptomatic
human carriers of CWD infection.***
==================
P.105: RT-QuIC models trans-species prion transmission
Kristen Davenport, Davin Henderson, Candace Mathiason, and Edward Hoover
Prion Research Center; Colorado State University; Fort Collins, CO USA
Conversely, FSE maintained sufficient BSE characteristics to more
efficiently convert bovine rPrP than feline rPrP. Additionally, human rPrP was
competent for conversion by CWD and fCWD.
***This insinuates that, at the level of protein:protein interactions, the
barrier preventing transmission of CWD to humans is less robust than previously
estimated.
================
***This insinuates that, at the level of protein:protein interactions, the
barrier preventing transmission of CWD to humans is less robust than previously
estimated.***
================
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014
*** chronic wasting disease, there was no absolute barrier to conversion of
the human prion protein.
*** Furthermore, the form of human PrPres produced in this in vitro assay
when seeded with CWD, resembles that found in the most common human prion
disease, namely sCJD of the MM1 subtype.
*** These results would seem to suggest that CWD does indeed have zoonotic
potential, at least as judged by the compatibility of CWD prions and their human
PrPC target. Furthermore, extrapolation from this simple in vitro assay suggests
that if zoonotic CWD occurred, it would most likely effect those of the PRNP
codon 129-MM genotype and that the PrPres type would be similar to that found in
the most common subtype of sCJD (MM1).***
*** The potential impact of prion diseases on human health was greatly
magnified by the recognition that interspecies transfer of BSE to humans by beef
ingestion resulted in vCJD. While changes in animal feed constituents and
slaughter practices appear to have curtailed vCJD, there is concern that CWD of
free-ranging deer and elk in the U.S. might also cross the species barrier.
Thus, consuming venison could be a source of human prion disease. Whether BSE
and CWD represent interspecies scrapie transfer or are newly arisen prion
diseases is unknown. Therefore, the possibility of transmission of prion disease
through other food animals cannot be ruled out. There is evidence that vCJD can
be transmitted through blood transfusion. There is likely a pool of unknown size
of asymptomatic individuals infected with vCJD, and there may be asymptomatic
individuals infected with the CWD equivalent. These circumstances represent a
potential threat to blood, blood products, and plasma supplies.
***********CJD REPORT 1994 increased risk for consumption of veal and
venison and lamb***********
CREUTZFELDT JAKOB DISEASE SURVEILLANCE IN THE UNITED KINGDOM THIRD ANNUAL
REPORT AUGUST 1994
Consumption of venison and veal was much less widespread among both cases
and controls. For both of these meats there was evidence of a trend with
increasing frequency of consumption being associated with increasing risk of
CJD. (not nvCJD, but sporadic CJD...tss)
These associations were largely unchanged when attention was restricted to
pairs with data obtained from relatives. ...
Table 9 presents the results of an analysis of these data.
There is STRONG evidence of an association between ‘’regular’’ veal eating
and risk of CJD (p = .0.01).
Individuals reported to eat veal on average at least once a year appear to
be at 13 TIMES THE RISK of individuals who have never eaten veal.
There is, however, a very wide confidence interval around this estimate.
There is no strong evidence that eating veal less than once per year is
associated with increased risk of CJD (p = 0.51).
The association between venison eating and risk of CJD shows similar
pattern, with regular venison eating associated with a 9 FOLD INCREASE IN RISK
OF CJD (p = 0.04).
There is some evidence that risk of CJD INCREASES WITH INCREASING FREQUENCY
OF LAMB EATING (p = 0.02).
The evidence for such an association between beef eating and CJD is weaker
(p = 0.14). When only controls for whom a relative was interviewed are included,
this evidence becomes a little STRONGER (p = 0.08).
snip...
It was found that when veal was included in the model with another
exposure, the association between veal and CJD remained statistically
significant (p = < 0.05 for all exposures), while the other exposures ceased
to be statistically significant (p = > 0.05).
snip...
In conclusion, an analysis of dietary histories revealed statistical
associations between various meats/animal products and INCREASED RISK OF CJD.
When some account was taken of possible confounding, the association between
VEAL EATING AND RISK OF CJD EMERGED AS THE STRONGEST OF THESE ASSOCIATIONS
STATISTICALLY. ...
snip...
In the study in the USA, a range of foodstuffs were associated with an
increased risk of CJD, including liver consumption which was associated with an
apparent SIX-FOLD INCREASE IN THE RISK OF CJD. By comparing the data from 3
studies in relation to this particular dietary factor, the risk of liver
consumption became non-significant with an odds ratio of 1.2 (PERSONAL
COMMUNICATION, PROFESSOR A. HOFMAN. ERASMUS UNIVERSITY, ROTTERDAM). (???...TSS)
snip...see full report ;
CJD9/10022
October 1994
Mr R.N. Elmhirst Chairman British Deer Farmers Association Holly Lodge
Spencers Lane BerksWell Coventry CV7 7BZ
Dear Mr Elmhirst,
CREUTZFELDT-JAKOB DISEASE (CJD) SURVEILLANCE UNIT REPORT
Thank you for your recent letter concerning the publication of the third
annual report from the CJD Surveillance Unit. I am sorry that you are
dissatisfied with the way in which this report was published.
The Surveillance Unit is a completely independant outside body and the
Department of Health is committed to publishing their reports as soon as they
become available. In the circumstances it is not the practice to circulate the
report for comment since the findings of the report would not be amended. In
future we can ensure that the British Deer Farmers Association receives a copy
of the report in advance of publication.
The Chief Medical Officer has undertaken to keep the public fully informed
of the results of any research in respect of CJD. This report was entirely the
work of the unit and was produced completely independantly of the the
Department.
The statistical results reqarding the consumption of venison was put into
perspective in the body of the report and was not mentioned at all in the press
release. Media attention regarding this report was low key but gave a realistic
presentation of the statistical findings of the Unit. This approach to
publication was successful in that consumption of venison was highlighted only
once by the media ie. in the News at one television proqramme.
I believe that a further statement about the report, or indeed statistical
links between CJD and consumption of venison, would increase, and quite possibly
give damaging credence, to the whole issue. From the low key media reports of
which I am aware it seems unlikely that venison consumption will suffer
adversely, if at all.
http://web.archive.org/web/20030511010117/http://www.bseinquiry.gov.uk/files/yb/1994/10/00003001.pdf
Monday, May 02, 2016
*** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo ***
*** PRION 2014 CONFERENCE CHRONIC WASTING DISEASE CWD
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014
Wednesday, January 01, 2014
Molecular Barriers to Zoonotic Transmission of Prions
*** chronic wasting disease, there was no absolute barrier to conversion of
the human prion protein.
*** Furthermore, the form of human PrPres produced in this in vitro assay
when seeded with CWD, resembles that found in the most common human prion
disease, namely sCJD of the MM1 subtype.
*** now, let’s see what the authors said about this casual link, personal
communications years ago, and then the latest on the zoonotic potential from CWD
to humans from the TOKYO PRION 2016 CONFERENCE.
see where it is stated NO STRONG evidence. so, does this mean there IS
casual evidence ???? “Our conclusion stating that we found no strong evidence of
CWD transmission to humans”
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
Thursday, April 03, 2008
A prion disease of cervids: Chronic wasting disease 2008 1: Vet Res. 2008
Apr 3;39(4):41 A prion disease of cervids: Chronic wasting disease Sigurdson CJ.
snip...
*** twenty-seven CJD patients who regularly consumed venison were reported
to the Surveillance Center***,
snip... full text ;
Monday, May 02, 2016
*** Zoonotic Potential of CWD Prions: An Update Prion 2016 Tokyo ***
*** NIH awards $11 million to UTHealth researchers to study deadly CWD
prion diseases Claudio Soto, Ph.D. ***
Public Release: 29-Jun-2016
I urge everyone to watch this video closely...terry
*** you can see video here and interview with Jeff's Mom, and scientist
telling you to test everything and potential risk factors for humans ***
Tuesday, July 12, 2016
Chronic Wasting Disease CWD, Scrapie, Bovine Spongiform Encephalopathy BSE,
TSE, Prion Zoonosis Science History
see history of NIH may destroy human brain collection
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
Taylor & Francis
Prion 2016 Animal Prion Disease Workshop Abstracts
WS-01: Prion diseases in animals and zoonotic potential
Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a.
Vincent Beringue c. Patricia Aguilar a,
Natalia Fernandez-Borges a. and Alba Marin-Moreno a
"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos,
Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT.
Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas.
France
Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated
bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD)
disease in human. To date, BSE agent is the only recognized zoonotic prion.
Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that
have been circulating for centuries in farmed ruminants there is no apparent
epidemiological link between exposure to ruminant products and the occurrence of
other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD).
However, the zoonotic potential of the diversity of circulating TSE agents has
never been systematically assessed. The major issue in experimental assessment
of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the
biological phenomenon that limits TSE agents’ propagation from a species to
another. In the last decade, mice genetically engineered to express normal forms
of the human prion protein has proved essential in studying human prions
pathogenesis and modeling the capacity of TSEs to cross the human species
barrier.
To assess the zoonotic potential of prions circulating in farmed ruminants,
we study their transmission ability in transgenic mice expressing human PrPC
(HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC
(129Met or 129Val) are used to determine the role of the Met129Val dimorphism in
susceptibility/resistance to the different agents.
These transmission experiments confirm the ability of BSE prions to
propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be
susceptible to BSE in sheep or goat to a greater degree than the BSE agent in
cattle and that these agents can convey molecular properties and
neuropathological indistinguishable from vCJD. However homozygous 129V mice are
resistant to all tested BSE derived prions independently of the originating
species suggesting a higher transmission barrier for 129V-PrP variant.
Transmission data also revealed that several scrapie prions propagate in
HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the
efficiency of transmission at primary passage was low, subsequent passages
resulted in a highly virulent prion disease in both Met129 and Val129 mice.
Transmission of the different scrapie isolates in these mice leads to the
emergence of prion strain phenotypes that showed similar characteristics to
those displayed by MM1 or VV2 sCJD prion. These results demonstrate that scrapie
prions have a zoonotic potential and raise new questions about the possible link
between animal and human prions.
SCRAPIE AND CWD ZOONOSIS
PRION 2016 CONFERENCE TOKYO
Saturday, April 23, 2016
*** SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
***
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X
Transmission of scrapie prions to primate after an extended silent
incubation period
***Moreover, sporadic disease has never been observed in breeding colonies
or primate research laboratories, most notably among hundreds of animals over
several decades of study at the National Institutes of Health25, and in nearly
twenty older animals continuously housed in our own facility.***
Wednesday, June 29, 2016
CWD, SCRAPIE, ZOONOSIS, it’s for real folks, the risk factors have
increased greatly, and science has spoken, cwd and scrapie to humans as sporadic
cjd may have already happened.
Transmission of scrapie prions to primate after an extended silent
incubation period
Emmanuel E. Comoy , Jacqueline Mikol , Sophie Luccantoni-Freire , Evelyne
Correia , Nathalie Lescoutra-Etchegaray , Valérie Durand , Capucine Dehen ,
Olivier Andreoletti , Cristina Casalone , Juergen A. Richt , Justin J. Greenlee
, Thierry Baron , Sylvie L. Benestad , Paul Brown & Jean-Philippe Deslys
Abstract
Classical bovine spongiform encephalopathy (c-BSE) is the only animal prion
disease reputed to be zoonotic, causing variant Creutzfeldt-Jakob disease (vCJD)
in humans and having guided protective measures for animal and human health
against animal prion diseases. Recently, partial transmissions to humanized mice
showed that the zoonotic potential of scrapie might be similar to c-BSE. We here
report the direct transmission of a natural classical scrapie isolate to
cynomolgus macaque, a highly relevant model for human prion diseases, after a
10-year silent incubation period, with features similar to those reported for
human cases of sporadic CJD. Scrapie is thus actually transmissible to primates
with incubation periods compatible with their life expectancy, although fourfold
longer than BSE. Long-term experimental transmission studies are necessary to
better assess the zoonotic potential of other prion diseases with high
prevalence, notably Chronic Wasting Disease of deer and elk and atypical/Nor98
scrapie.
snip...
In addition to previous studies on scrapie transmission to primate1,8,9 and
the recently published study on transgenic humanized mice13, our results
constitute new evidence for recommending that the potential risk of scrapie for
human health should not be dismissed. Indeed, human PrP transgenic mice and
primates are the most relevant models for investigating the human transmission
barrier. To what extent such models are informative for measuring the zoonotic
potential of an animal TSE under field exposure conditions is unknown. During
the past decades, many protective measures have been successfully implemented to
protect cattle from the spread of c-BSE, and some of these measures have been
extended to sheep and goats to protect from scrapie according to the principle
of precaution. Since cases of c-BSE have greatly reduced in number, those
protective measures are currently being challenged and relaxed in the absence of
other known zoonotic animal prion disease. We recommend that risk managers
should be aware of the long term potential risk to human health of at least
certain scrapie isolates, notably for lymphotropic strains like the classical
scrapie strain used in the current study. Relatively high amounts of infectivity
in peripheral lymphoid organs in animals infected with these strains could lead
to contamination of food products produced for human consumption. Efforts should
also be maintained to further assess the zoonotic potential of other animal
prion strains in long-term studies, notably lymphotropic strains with high
prevalence like CWD, which is spreading across North America, and atypical/Nor98
scrapie (Nor98)50 that was first detected in the past two decades and now
represents approximately half of all reported cases of prion diseases in small
ruminants worldwide, including territories previously considered as scrapie
free. Even if the prevailing view is that sporadic CJD is due to the spontaneous
formation of CJD prions, it remains possible that its apparent sporadic nature
may, at least in part, result from our limited capacity to identify an
environmental origin.
***Moreover, sporadic disease has never been observed in breeding colonies
or primate research laboratories, most notably among hundreds of animals over
several decades of study at the National Institutes of Health25, and in nearly
twenty older animals continuously housed in our own facility.***
2015
O.05: Transmission of prions to primates after extended silent incubation
periods: Implications for BSE and scrapie risk assessment in human populations
Emmanuel Comoy, Jacqueline Mikol, Valerie Durand, Sophie Luccantoni,
Evelyne Correia, Nathalie Lescoutra, Capucine Dehen, and Jean-Philippe Deslys
Atomic Energy Commission; Fontenay-aux-Roses, France
Prion diseases (PD) are the unique neurodegenerative proteinopathies
reputed to be transmissible under field conditions since decades. The
transmission of Bovine Spongiform Encephalopathy (BSE) to humans evidenced that
an animal PD might be zoonotic under appropriate conditions. Contrarily, in the
absence of obvious (epidemiological or experimental) elements supporting a
transmission or genetic predispositions, PD, like the other proteinopathies, are
reputed to occur spontaneously (atpical animal prion strains, sporadic CJD
summing 80% of human prion cases). Non-human primate models provided the first
evidences supporting the transmissibiity of human prion strains and the zoonotic
potential of BSE. Among them, cynomolgus macaques brought major information for
BSE risk assessment for human health (Chen, 2014), according to their
phylogenetic proximity to humans and extended lifetime. We used this model to
assess the zoonotic potential of other animal PD from bovine, ovine and cervid
origins even after very long silent incubation periods.
*** We recently observed the direct transmission of a natural classical
scrapie isolate to macaque after a 10-year silent incubation period,
***with features similar to some reported for human cases of sporadic CJD,
albeit requiring fourfold long incubation than BSE. Scrapie, as recently evoked
in humanized mice (Cassard, 2014),
***is the third potentially zoonotic PD (with BSE and L-type BSE),
***thus questioning the origin of human sporadic cases. We will present an
updated panorama of our different transmission studies and discuss the
implications of such extended incubation periods on risk assessment of animal PD
for human health.
===============
***thus questioning the origin of human sporadic cases***
===============
***our findings suggest that possible transmission risk of H-type BSE to
sheep and human. Bioassay will be required to determine whether the PMCA
products are infectious to these animals.
==============
Saturday, July 16, 2016
Importation of Sheep, Goats, and Certain Other Ruminants [Docket No.
APHIS-2009-0095]RIN 0579-AD10
WITH great disgust and concern, I report to you that the OIE, USDA, APHIS,
are working to further legalize the trading of Transmissible Spongiform
Encephalopathy TSE Pion disease around the globe.
THIS is absolutely insane. it’s USDA INC.
*** Infectious agent of sheep scrapie may persist in the environment for at
least 16 years ***
Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3
Using in vitro prion replication for high sensitive detection of prions and
prionlike proteins and for understanding mechanisms of transmission.
Claudio Soto
Mitchell Center for Alzheimer's diseases and related Brain disorders,
Department of Neurology, University of Texas Medical School at Houston.
Prion and prion-like proteins are misfolded protein aggregates with the
ability to selfpropagate to spread disease between cells, organs and in some
cases across individuals. I n T r a n s m i s s i b l e s p o n g i f o r m
encephalopathies (TSEs), prions are mostly composed by a misfolded form of the
prion protein (PrPSc), which propagates by transmitting its misfolding to the
normal prion protein (PrPC). The availability of a procedure to replicate prions
in the laboratory may be important to study the mechanism of prion and
prion-like spreading and to develop high sensitive detection of small quantities
of misfolded proteins in biological fluids, tissues and environmental samples.
Protein Misfolding Cyclic Amplification (PMCA) is a simple, fast and efficient
methodology to mimic prion replication in the test tube. PMCA is a platform
technology that may enable amplification of any prion-like misfolded protein
aggregating through a seeding/nucleation process. In TSEs, PMCA is able to
detect the equivalent of one single molecule of infectious PrPSc and propagate
prions that maintain high infectivity, strain properties and species
specificity. Using PMCA we have been able to detect PrPSc in blood and urine of
experimentally infected animals and humans affected by vCJD with high
sensitivity and specificity. Recently, we have expanded the principles of PMCA
to amplify amyloid-beta (Aβ) and alphasynuclein (α-syn) aggregates implicated in
Alzheimer's and Parkinson's diseases, respectively. Experiments are ongoing to
study the utility of this technology to detect Aβ and α-syn aggregates in
samples of CSF and blood from patients affected by these diseases.
=========================
***Recently, we have been using PMCA to study the role of environmental
prion contamination on the horizontal spreading of TSEs. These experiments have
focused on the study of the interaction of prions with plants and
environmentally relevant surfaces. Our results show that plants (both leaves and
roots) bind tightly to prions present in brain extracts and excreta (urine and
feces) and retain even small quantities of PrPSc for long periods of time.
Strikingly, ingestion of prioncontaminated leaves and roots produced disease
with a 100% attack rate and an incubation period not substantially longer than
feeding animals directly with scrapie brain homogenate. Furthermore, plants can
uptake prions from contaminated soil and transport them to different parts of
the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety
of environmentally relevant surfaces, including stones, wood, metals, plastic,
glass, cement, etc. Prion contaminated surfaces efficiently transmit prion
disease when these materials were directly injected into the brain of animals
and strikingly when the contaminated surfaces were just placed in the animal
cage. These findings demonstrate that environmental materials can efficiently
bind infectious prions and act as carriers of infectivity, suggesting that they
may play an important role in the horizontal transmission of the disease.
========================
Since its invention 13 years ago, PMCA has helped to answer fundamental
questions of prion propagation and has broad applications in research areas
including the food industry, blood bank safety and human and veterinary disease
diagnosis.
see ;
with CWD TSE Prions, I am not sure there is any absolute yet, other than
what we know with transmission studies, and we know tse prion kill, and tse
prion are bad. science shows to date, that indeed soil, dirt, some better than
others, can act as a carrier. same with objects, farm furniture. take it with
how ever many grains of salt you wish, or not. if load factor plays a role in
the end formula, then everything should be on the table, in my opinion. see
;
***Recently, we have been using PMCA to study the role of environmental
prion contamination on the horizontal spreading of TSEs. These experiments have
focused on the study of the interaction of prions with plants and
environmentally relevant surfaces. Our results show that plants (both leaves and
roots) bind tightly to prions present in brain extracts and excreta (urine and
feces) and retain even small quantities of PrPSc for long periods of time.
Strikingly, ingestion of prioncontaminated leaves and roots produced disease
with a 100% attack rate and an incubation period not substantially longer than
feeding animals directly with scrapie brain homogenate. Furthermore, plants can
uptake prions from contaminated soil and transport them to different parts of
the plant tissue (stem and leaves). Similarly, prions bind tightly to a variety
of environmentally relevant surfaces, including stones, wood, metals, plastic,
glass, cement, etc. Prion contaminated surfaces efficiently transmit prion
disease when these materials were directly injected into the brain of animals
and strikingly when the contaminated surfaces were just placed in the animal
cage. These findings demonstrate that environmental materials can efficiently
bind infectious prions and act as carriers of infectivity, suggesting that they
may play an important role in the horizontal transmission of the disease.
Since its invention 13 years ago, PMCA has helped to answer fundamental
questions of prion propagation and has broad applications in research areas
including the food industry, blood bank safety and human and veterinary disease
diagnosis.
see ;
Oral Transmissibility of Prion Disease Is Enhanced by Binding to Soil
Particles
Author Summary
Transmissible spongiform encephalopathies (TSEs) are a group of incurable
neurological diseases likely caused by a misfolded form of the prion protein.
TSEs include scrapie in sheep, bovine spongiform encephalopathy (‘‘mad cow’’
disease) in cattle, chronic wasting disease in deer and elk, and
Creutzfeldt-Jakob disease in humans. Scrapie and chronic wasting disease are
unique among TSEs because they can be transmitted between animals, and the
disease agents appear to persist in environments previously inhabited by
infected animals. Soil has been hypothesized to act as a reservoir of
infectivity and to bind the infectious agent. In the current study, we orally
dosed experimental animals with a common clay mineral, montmorillonite, or whole
soils laden with infectious prions, and compared the transmissibility to unbound
agent. We found that prions bound to montmorillonite and whole soils remained
orally infectious, and, in most cases, increased the oral transmission of
disease compared to the unbound agent. The results presented in this study
suggest that soil may contribute to environmental spread of TSEs by increasing
the transmissibility of small amounts of infectious agent in the
environment.
tse prion soil
Wednesday, December 16, 2015
Objects in contact with classical scrapie sheep act as a reservoir for
scrapie transmission
The sources of dust borne prions are unknown but it seems reasonable to
assume that faecal, urine, skin, parturient material and saliva-derived prions
may contribute to this mobile environmental reservoir of infectivity. This work
highlights a possible transmission route for scrapie within the farm
environment, and this is likely to be paralleled in CWD which shows strong
similarities with scrapie in terms of prion dissemination and disease
transmission. The data indicate that the presence of scrapie prions in dust is
likely to make the control of these diseases a considerable challenge.
>>>Particle-associated PrPTSE molecules may migrate from locations
of deposition via transport processes affecting soil particles, including
entrainment in and movement with air and overland flow. <<<
Fate of Prions in Soil: A Review
Christen B. Smith, Clarissa J. Booth, and Joel A. Pedersen*
Several reports have shown that prions can persist in soil for several
years. Significant interest remains in developing methods that could be applied
to degrade PrPTSE in naturally contaminated soils. Preliminary research suggests
that serine proteases and the microbial consortia in stimulated soils and
compost may partially degrade PrPTSE. Transition metal oxides in soil (viz.
manganese oxide) may also mediate prion inactivation. Overall, the effect of
prion attachment to soil particles on its persistence in the environment is not
well understood, and additional study is needed to determine its implications on
the environmental transmission of scrapie and CWD.
P.161: Prion soil binding may explain efficient horizontal CWD transmission
Conclusion. Silty clay loam exhibits highly efficient prion binding,
inferring a durable environmental reservoir, and an efficient mechanism for
indirect horizontal CWD transmission.
>>>Another alternative would be an absolute prohibition on the
movement of deer within the state for any purpose. While this alternative would
significantly reduce the potential spread of CWD, it would also have the
simultaneous effect of preventing landowners and land managers from implementing
popular management strategies involving the movement of deer, and would deprive
deer breeders of the ability to engage in the business of buying and selling
breeder deer. Therefore, this alternative was rejected because the department
determined that it placed an avoidable burden on the regulated
community.<<<
Wednesday, December 16, 2015
Objects in contact with classical scrapie sheep act as a reservoir for
scrapie transmission
Objects in contact with classical scrapie sheep act as a reservoir for
scrapie transmission
Timm Konold1*, Stephen A. C. Hawkins2, Lisa C. Thurston3, Ben C. Maddison4,
Kevin C. Gough5, Anthony Duarte1 and Hugh A. Simmons1
1 Animal Sciences Unit, Animal and Plant Health Agency Weybridge,
Addlestone, UK, 2 Pathology Department, Animal and Plant Health Agency
Weybridge, Addlestone, UK, 3 Surveillance and Laboratory Services, Animal and
Plant Health Agency Penrith, Penrith, UK, 4 ADAS UK, School of Veterinary
Medicine and Science, University of Nottingham, Sutton Bonington, UK, 5 School
of Veterinary Medicine and Science, University of Nottingham, Sutton Bonington,
UK
Classical scrapie is an environmentally transmissible prion disease of
sheep and goats. Prions can persist and remain potentially infectious in the
environment for many years and thus pose a risk of infecting animals after
re-stocking. In vitro studies using serial protein misfolding cyclic
amplification (sPMCA) have suggested that objects on a scrapie affected sheep
farm could contribute to disease transmission. This in vivo study aimed to
determine the role of field furniture (water troughs, feeding troughs, fencing,
and other objects that sheep may rub against) used by a scrapie-infected sheep
flock as a vector for disease transmission to scrapie-free lambs with the prion
protein genotype VRQ/VRQ, which is associated with high susceptibility to
classical scrapie. When the field furniture was placed in clean accommodation,
sheep became infected when exposed to either a water trough (four out of five)
or to objects used for rubbing (four out of seven). This field furniture had
been used by the scrapie-infected flock 8 weeks earlier and had previously been
shown to harbor scrapie prions by sPMCA. Sheep also became infected (20 out of
23) through exposure to contaminated field furniture placed within pasture not
used by scrapie-infected sheep for 40 months, even though swabs from this
furniture tested negative by PMCA. This infection rate decreased (1 out of 12)
on the same paddock after replacement with clean field furniture. Twelve grazing
sheep exposed to field furniture not in contact with scrapie-infected sheep for
18 months remained scrapie free. The findings of this study highlight the role
of field furniture used by scrapie-infected sheep to act as a reservoir for
disease re-introduction although infectivity declines considerably if the field
furniture has not been in contact with scrapie-infected sheep for several
months. PMCA may not be as sensitive as VRQ/VRQ sheep to test for environmental
contamination.
snip...
Discussion
Classical scrapie is an environmentally transmissible disease because it
has been reported in naïve, supposedly previously unexposed sheep placed in
pastures formerly occupied by scrapie-infected sheep (4, 19, 20). Although the
vector for disease transmission is not known, soil is likely to be an important
reservoir for prions (2) where – based on studies in rodents – prions can adhere
to minerals as a biologically active form (21) and remain infectious for more
than 2 years (22). Similarly, chronic wasting disease (CWD) has re-occurred in
mule deer housed in paddocks used by infected deer 2 years earlier, which was
assumed to be through foraging and soil consumption (23).
Our study suggested that the risk of acquiring scrapie infection was
greater through exposure to contaminated wooden, plastic, and metal surfaces via
water or food troughs, fencing, and hurdles than through grazing. Drinking from
a water trough used by the scrapie flock was sufficient to cause infection in
sheep in a clean building. Exposure to fences and other objects used for rubbing
also led to infection, which supported the hypothesis that skin may be a vector
for disease transmission (9). The risk of these objects to cause infection was
further demonstrated when 87% of 23 sheep presented with PrPSc in lymphoid
tissue after grazing on one of the paddocks, which contained metal hurdles, a
metal lamb creep and a water trough in contact with the scrapie flock up to 8
weeks earlier, whereas no infection had been demonstrated previously in sheep
grazing on this paddock, when equipped with new fencing and field furniture.
When the contaminated furniture and fencing were removed, the infection rate
dropped significantly to 8% of 12 sheep, with soil of the paddock as the most
likely source of infection caused by shedding of prions from the
scrapie-infected sheep in this paddock up to a week earlier.
This study also indicated that the level of contamination of field
furniture sufficient to cause infection was dependent on two factors: stage of
incubation period and time of last use by scrapie-infected sheep. Drinking from
a water trough that had been used by scrapie sheep in the predominantly
pre-clinical phase did not appear to cause infection, whereas infection was
shown in sheep drinking from the water trough used by scrapie sheep in the later
stage of the disease. It is possible that contamination occurred through
shedding of prions in saliva, which may have contaminated the surface of the
water trough and subsequently the water when it was refilled. Contamination
appeared to be sufficient to cause infection only if the trough was in contact
with sheep that included clinical cases. Indeed, there is an increased risk of
bodily fluid infectivity with disease progression in scrapie (24) and CWD (25)
based on PrPSc detection by sPMCA. Although ultraviolet light and heat under
natural conditions do not inactivate prions (26), furniture in contact with the
scrapie flock, which was assumed to be sufficiently contaminated to cause
infection, did not act as vector for disease if not used for 18 months, which
suggest that the weathering process alone was sufficient to inactivate prions.
PrPSc detection by sPMCA is increasingly used as a surrogate for
infectivity measurements by bioassay in sheep or mice. In this reported study,
however, the levels of PrPSc present in the environment were below the limit of
detection of the sPMCA method, yet were still sufficient to cause infection of
in-contact animals. In the present study, the outdoor objects were removed from
the infected flock 8 weeks prior to sampling and were positive by sPMCA at very
low levels (2 out of 37 reactions). As this sPMCA assay also yielded 2 positive
reactions out of 139 in samples from the scrapie-free farm, the sPMCA assay
could not detect PrPSc on any of the objects above the background of the assay.
False positive reactions with sPMCA at a low frequency associated with de novo
formation of infectious prions have been reported (27, 28). This is in contrast
to our previous study where we demonstrated that outdoor objects that had been
in contact with the scrapie-infected flock up to 20 days prior to sampling
harbored PrPSc that was detectable by sPMCA analysis [4 out of 15 reactions
(12)] and was significantly more positive by the assay compared to analogous
samples from the scrapie-free farm. This discrepancy could be due to the use of
a different sPMCA substrate between the studies that may alter the efficiency of
amplification of the environmental PrPSc. In addition, the present study had a
longer timeframe between the objects being in contact with the infected flock
and sampling, which may affect the levels of extractable PrPSc. Alternatively,
there may be potentially patchy contamination of this furniture with PrPSc,
which may have been missed by swabbing. The failure of sPMCA to detect
CWD-associated PrP in saliva from clinically affected deer despite confirmation
of infectivity in saliva-inoculated transgenic mice was associated with as yet
unidentified inhibitors in saliva (29), and it is possible that the sensitivity
of sPMCA is affected by other substances in the tested material. In addition,
sampling of amplifiable PrPSc and subsequent detection by sPMCA may be more
difficult from furniture exposed to weather, which is supported by the
observation that PrPSc was detected by sPMCA more frequently in indoor than
outdoor furniture (12). A recent experimental study has demonstrated that
repeated cycles of drying and wetting of prion-contaminated soil, equivalent to
what is expected under natural weathering conditions, could reduce PMCA
amplification efficiency and extend the incubation period in hamsters inoculated
with soil samples (30). This seems to apply also to this study even though the
reduction in infectivity was more dramatic in the sPMCA assays than in the sheep
model. Sheep were not kept until clinical end-point, which would have enabled us
to compare incubation periods, but the lack of infection in sheep exposed to
furniture that had not been in contact with scrapie sheep for a longer time
period supports the hypothesis that prion degradation and subsequent loss of
infectivity occurs even under natural conditions.
In conclusion, the results in the current study indicate that removal of
furniture that had been in contact with scrapie-infected animals should be
recommended, particularly since cleaning and decontamination may not effectively
remove scrapie infectivity (31), even though infectivity declines considerably
if the pasture and the field furniture have not been in contact with
scrapie-infected sheep for several months. As sPMCA failed to detect PrPSc in
furniture that was subjected to weathering, even though exposure led to
infection in sheep, this method may not always be reliable in predicting the
risk of scrapie infection through environmental contamination. These results
suggest that the VRQ/VRQ sheep model may be more sensitive than sPMCA for the
detection of environmentally associated scrapie, and suggest that extremely low
levels of scrapie contamination are able to cause infection in susceptible sheep
genotypes.
Keywords: classical scrapie, prion, transmissible spongiform
encephalopathy, sheep, field furniture, reservoir, serial protein misfolding
cyclic amplification
Wednesday, December 16, 2015
*** Objects in contact with classical scrapie sheep act as a reservoir for
scrapie transmission ***
*** Infectious agent of sheep scrapie may persist in the environment for at
least 16 years ***
Gudmundur Georgsson1, Sigurdur Sigurdarson2 and Paul Brown3
>>>Another alternative would be an absolute prohibition on the
movement of deer within the state for any purpose. While this alternative would
significantly reduce the potential spread of CWD, it would also have the
simultaneous effect of preventing landowners and land managers from implementing
popular management strategies involving the movement of deer, and would deprive
deer breeders of the ability to engage in the business of buying and selling
breeder deer. Therefore, this alternative was rejected because the department
determined that it placed an avoidable burden on the regulated
community.<<<
Circulation of prions within dust on a scrapie affected farm
Kevin C Gough1, Claire A Baker2, Hugh A Simmons3, Steve A Hawkins3 and Ben
C Maddison2*
Abstract
Prion diseases are fatal neurological disorders that affect humans and
animals. Scrapie of sheep/goats and Chronic Wasting Disease (CWD) of deer/elk
are contagious prion diseases where environmental reservoirs have a direct link
to the transmission of disease. Using protein misfolding cyclic amplification we
demonstrate that scrapie PrPSc can be detected within circulating dusts that are
present on a farm that is naturally contaminated with sheep scrapie. The
presence of infectious scrapie within airborne dusts may represent a possible
route of infection and illustrates the difficulties that may be associated with
the effective decontamination of such scrapie affected premises.
snip...
Discussion
We present biochemical data illustrating the airborne movement of scrapie
containing material within a contaminated farm environment. We were able to
detect scrapie PrPSc within extracts from dusts collected over a 70 day period,
in the absence of any sheep activity. We were also able to detect scrapie PrPSc
within dusts collected within pasture at 30 m but not at 60 m distance away from
the scrapie contaminated buildings, suggesting that the chance of contamination
of pasture by scrapie contaminated dusts decreases with distance from
contaminated farm buildings. PrPSc amplification by sPMCA has been shown to
correlate with infectivity and amplified products have been shown to be
infectious [14,15]. These experiments illustrate the potential for low dose
scrapie infectivity to be present within such samples. We estimate low ng levels
of scrapie positive brain equivalent were deposited per m2 over 70 days, in a
barn previously occupied by sheep affected with scrapie. This movement of dusts
and the accumulation of low levels of scrapie infectivity within this
environment may in part explain previous observations where despite stringent
pen decontamination regimens healthy lambs still became scrapie infected after
apparent exposure from their environment alone [16]. The presence of sPMCA
seeding activity and by inference, infectious prions within dusts, and their
potential for airborne dissemination is highly novel and may have implications
for the spread of scrapie within infected premises. The low level circulation
and accumulation of scrapie prion containing dust material within the farm
environment will likely impede the efficient decontamination of such scrapie
contaminated buildings unless all possible reservoirs of dust are removed.
Scrapie containing dusts could possibly infect animals during feeding and
drinking, and respiratory and conjunctival routes may also be involved. It has
been demonstrated that scrapie can be efficiently transmitted via the nasal
route in sheep [17], as is also the case for CWD in both murine models and in
white tailed deer [18-20].
The sources of dust borne prions are unknown but it seems reasonable to
assume that faecal, urine, skin, parturient material and saliva-derived prions
may contribute to this mobile environmental reservoir of infectivity. This work
highlights a possible transmission route for scrapie within the farm
environment, and this is likely to be paralleled in CWD which shows strong
similarities with scrapie in terms of prion dissemination and disease
transmission. The data indicate that the presence of scrapie prions in dust is
likely to make the control of these diseases a considerable challenge.
***Moreover, sporadic disease has never been observed in breeding colonies
or primate research laboratories, most notably among hundreds of animals over
several decades of study at the National Institutes of Health25, and in nearly
twenty older animals continuously housed in our own facility.***
Saturday, April 16, 2016
APHIS [Docket No. APHIS-2016-0029] Secretary's Advisory Committee on Animal
Health; Meeting May 2, 2016, and June 16, 2016 Singeltary Submission
Evidence That Transmissible Mink Encephalopathy Results from Feeding
Infected Cattle
Over the next 8-10 weeks, approximately 40% of all the adult mink on the
farm died from TME.
snip...
The rancher was a ''dead stock'' feeder using mostly (>95%) downer or
dead dairy cattle...
In Confidence - Perceptions of unconventional slow virus diseases of
animals in the USA - APRIL-MAY 1989 - G A H Wells
3. Prof. A. Robertson gave a brief account of BSE. The US approach was to
accord it a very low profile indeed. Dr. A Thiermann showed the picture in the
''Independent'' with cattle being incinerated and thought this was a fanatical
incident to be avoided in the US at all costs. ...
”The occurrence of CWD must be viewed against the contest of the locations
in which it occurred. It was an incidental and unwelcome complication of the
respective wildlife research programmes. Despite it’s subsequent recognition as
a new disease of cervids, therefore justifying direct investigation, no specific
research funding was forthcoming. The USDA veiwed it as a wildlife problem and
consequently not their province!” ...page 26.
*** Spraker suggested an interesting explanation for the occurrence of CWD.
The deer pens at the Foot Hills Campus were built some 30-40 years ago by a Dr.
Bob Davis. At or abut that time, allegedly, some scrapie work was conducted at
this site. When deer were introduced to the pens they occupied ground that had
previously been occupied by sheep.
Spongiform Encephalopathy in Captive Wild ZOO BSE INQUIRY
Friday, August 14, 2015
*** Susceptibility of cattle to the agent of chronic wasting disease from
elk after intracranial inoculation ***
Saturday, May 28, 2016
*** Infection and detection of PrPCWD in soil from CWD infected farm in
Korea Prion 2016 Tokyo ***
I urge everyone to watch this video closely...terry
*** you can see video here and interview with Jeff's Mom, and scientist
telling you to test everything and potential risk factors for humans ***
Monday, May 09, 2016
A comparison of classical and H-type bovine spongiform encephalopathy
associated with E211K prion protein polymorphism in wild type and EK211 cattle
following intracranial inoculation
Wednesday, July 15, 2015
Additional BSE TSE prion testing detects pathologic lesion in unusual brain
location and PrPsc by PMCA only, how many cases have we missed?
***however in 1 C-type challenged animal, Prion 2015 Poster Abstracts S67
PrPsc was not detected using rapid tests for BSE.
***Subsequent testing resulted in the detection of pathologic lesion in
unusual brain location and PrPsc detection by PMCA only.
IBNC Tauopathy or TSE Prion disease, it appears, no one is sure
Posted by flounder on 03 Jul 2015 at 16:53 GMT
Tuesday, April 19, 2016
Docket No. FDA-2013-N-0764 for Animal Feed Regulatory Program Standards
Singeltary Comment Submission
I strenuously once again urge the FDA and its industry constituents, to
make it MANDATORY that all ruminant feed be banned to all ruminants, and this
should include all cervids as soon as possible for the following
reasons...
======
In the USA, under the Food and Drug Administrations BSE Feed Regulation (21
CFR 589.2000) most material (exceptions include milk, tallow, and gelatin) from
deer and elk is prohibited for use in feed for ruminant animals. With regards to
feed for non-ruminant animals, under FDA law, CWD positive deer may not be used
for any animal feed or feed ingredients. For elk and deer considered at high
risk for CWD, the FDA recommends that these animals do not enter the animal feed
system.
***However, this recommendation is guidance and not a requirement by law.
======
31 Jan 2015 at 20:14 GMT
*** Ruminant feed ban for cervids in the United States? ***
31 Jan 2015 at 20:14 GMT
see Singeltary comment ;
*** Singeltary reply ; Molecular, Biochemical and Genetic Characteristics
of BSE in Canada Singeltary reply ;
>>> It is distinct from atypical BSE, which may develop
spontaneously, according to information from the U.S. Centers for Disease
Control and Prevention.
THIS IS A MYTH $$$
***atypical spontaneous BSE in France LOL***
FRANCE STOPS TESTING FOR MAD COW DISEASE BSE, and here’s why, to many
spontaneous events of mad cow disease $$$
***so 20 cases of atypical BSE in France, compared to the remaining 40
cases in the remaining 12 Countries, divided by the remaining 12 Countries,
about 3+ cases per country, besides Frances 20 cases. you cannot explain this
away with any spontaneous BSe. ...TSS
Sunday, October 5, 2014
France stops BSE testing for Mad Cow Disease
Thursday, March 24, 2016
FRANCE CONFIRMS BOVINE SPONGIFORM ENCEPHALOPATHY BSE MAD COW (ESB) chez une
vache dans les Ardennes
***atypical spontaneous BSE in France LOL***
FRANCE STOPS TESTING FOR MAD COW DISEASE BSE, and here’s why, to many
spontaneous events of mad cow disease $$$
If you Compare France to other Countries with atypical BSE, in my opinion,
you cannot explain this with ‘spontaneous’.
Table 1: Number of Atypical BSE cases reported by EU Member States in the
period 2001–2014 by country and by type (L- and H-BSE) (extracted from EU BSE
databases on 1 July 2014). By 2015, these data might be more comprehensive
following a request from the European Commission to Member States for re-testing
and retrospective classification of all positive bovine isolates in the EU in
the years 2003–2009
BSE type
Country 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 2011 2012 2013(a)
2014(a) Total
H-BSE Austria 1 1
France(b) 1 2 3 1 2 2 2 2 15
Germany 1 1 2
Ireland 1 1 2 1 5
The Netherlands 1 1
Poland 1 1 2
Portugal 1 1
Spain 1 1 2
Sweden 1 1
United Kingdom 1 1 1 1 1 5
Total 2 3 3 1 1 2 2 2 4 4 5 1 4 1 35
L-BSE Austria 1 1 2
Denmark 1 1
France(b) 1 1 1 1 2 1 3 2 1 1 14
Germany 1 1 2
Italy 1 1 1 1 1 5
The Netherlands 1 1 1 3
Poland 1 2 2 1 2 1 2 1 12
Spain 2 2
United Kingdom 1 1 1 1 4
Total 0 5 3 4 3 3 6 3 3 4 3 6 1 1 45
Total Atypical cases (H + L)
2 8 6 5 4 5 8 5 7 8 8 7 5 2 80
(a): Data for 2013-2014 are incomplete and may not include all
cases/countries reported.
(b): France has performed extensive retrospective testing to classify BSE
cases, which is probably the explanation for the higher number of Atypical BSE
cases reported in this country.
The number of Atypical BSE cases detected in countries that have already
identified them seems to be similar from year to year. In France, a
retrospective study of all TSE-positive cattle identified through the compulsory
EU surveillance between 2001 and 2007 indicated that the prevalence of H-BSE and
L-BSE was 0.35 and 0.41 cases per million adult cattle tested, respectively,
which increased to 1.9 and 1.7 cases per million, respectively, in tested
animals over eight years old (Biacabe et al., 2008). No comprehensive study on
the prevalence of Atypical BSE cases has yet been carried out in other EU Member
States. All cases of Atypical BSE reported in the EU BSE databases have been
identified by active surveillance testing (59 % in fallen stock, 38 % in healthy
slaughtered cattle and 4 % in emergency slaughtered cattle). Cases were reported
in animals over eight years of age, with the exception of two cases (one H-BSE
and one L-BSE) detected in Spain in 2011/2012. One additional case of H-BSE was
detected in Switzerland in 2012 in a cow born in Germany in 2005 (Guldimann et
al., 2012).
SPONTANEOUS TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY TSE PRION AKA MAD COW
TYPE DISEASE ???
*** We describe the transmission of spongiform encephalopathy in a
non-human primate inoculated 10 years earlier with a strain of sheep c-scrapie.
Because of this extended incubation period in a facility in which other prion
diseases are under study, we are obliged to consider two alternative
possibilities that might explain its occurrence. We first considered the
possibility of a sporadic origin (like CJD in humans). Such an event is
extremely improbable because the inoculated animal was 14 years old when the
clinical signs appeared, i.e. about 40% through the expected natural lifetime of
this species, compared to a peak age incidence of 60–65 years in human sporadic
CJD, or about 80% through their expected lifetimes.
***Moreover, sporadic disease has never been observed in breeding colonies
or primate research laboratories, most notably among hundreds of animals over
several decades of study at the National Institutes of Health25, and in nearly
twenty older animals continuously housed in our own facility.***
>>> Moreover, sporadic disease has never been observed in breeding
colonies or primate research laboratories, most notably among hundreds of
animals over several decades of study at the National Institutes of Health25,
and in nearly twenty older animals continuously housed in our own facility.
<<<
besides France’s HUGE increase of atypical BSE, see Poland’s HUGE increase
in atypical L-type BASE BSE. another spontaneous event and not feed ???
really???
see these two happenings of spontaneous BSeee...tss
Tuesday, July 26, 2016
Atypical Bovine Spongiform Encephalopathy BSE TSE Prion UPDATE JULY 2016
Monday, May 09, 2016
A comparison of classical and H-type bovine spongiform encephalopathy
associated with E211K prion protein polymorphism in wild type and EK211 cattle
following intracranial inoculation
*** Singeltary reply ; Molecular, Biochemical and Genetic
Characteristics of BSE in Canada Singeltary reply ;
*** It also suggests a similar cause or source for atypical BSE in these
countries. ***
Discussion: The C, L and H type BSE cases in Canada exhibit molecular
characteristics similar to those described for classical and atypical BSE cases
from Europe and Japan.
*** This supports the theory that the importation of BSE contaminated
feedstuff is the source of C-type BSE in Canada.
*** It also suggests a similar cause or source for atypical BSE in these
countries. ***
see page 17 6 of 201 pages...tss
Monday, June 20, 2016
*** Specified Risk Materials SRMs BSE TSE Prion Program
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14,
2001 JAMA
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
To the Editor: In their Research Letter, Dr Gibbons and colleagues1
reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD)
has been stable since 1985. These estimates, however, are based only on reported
cases, and do not include misdiagnosed or preclinical cases. It seems to me that
misdiagnosis alone would drastically change these figures. An unknown number of
persons with a diagnosis of Alzheimer disease in fact may have CJD, although
only a small number of these patients receive the postmortem examination
necessary to make this diagnosis. Furthermore, only a few states have made CJD
reportable. Human and animal transmissible spongiform encephalopathies should be
reportable nationwide and internationally.
Terry S. Singeltary, Sr Bacliff, Tex
1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob
disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323.
26 March 2003
Terry S. Singeltary, retired (medically) CJD WATCH
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?
The Lancet Infectious Diseases, Volume 3, Issue 8, Page 463, August 2003
doi:10.1016/S1473-3099(03)00715-1Cite or Link Using DOI
Tracking spongiform encephalopathies in North America
Original
Xavier Bosch
“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.” 49-year—old Singeltary is
one of a number of people who have remained largely unsatisfied after being told
that a close relative died from a rapidly progressive dementia compatible with
spontaneous Creutzfeldt—Jakob ...
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
Suspect symptoms
What if you can catch old-fashioned CJD by eating meat from a sheep
infected with scrapie?
28 Mar 01
Most doctors believe that sCJD is caused by a prion protein deforming by
chance into a killer. But Singeltary thinks otherwise. He is one of a number of
campaigners who say that some sCJD, like the variant CJD related to BSE, is
caused by eating meat from infected animals. Their suspicions have focused on
sheep carrying scrapie, a BSE-like disease that is widespread in flocks across
Europe and North America. Now scientists in France have stumbled across new
evidence that adds weight to the campaigners' fears. To their complete surprise,
the researchers found that one strain of scrapie causes the same brain damage in
mice as sCJD.
"This means we cannot rule out that at least some sCJD may be caused by
some strains of scrapie," says team member Jean-Philippe Deslys of the French
Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses,
south-west of Paris. Hans Kretschmar of the University of Göttingen, who
coordinates CJD surveillance in Germany, is so concerned by the findings that he
now wants to trawl back through past sCJD cases to see if any might have been
caused by eating infected mutton or lamb...
Tuesday, December 16, 2014
Evidence for zoonotic potential of ovine scrapie prions
Hervé Cassard,1, n1 Juan-Maria Torres,2, n1 Caroline Lacroux,1, Jean-Yves
Douet,1, Sylvie L. Benestad,3, Frédéric Lantier,4, Séverine Lugan,1, Isabelle
Lantier,4, Pierrette Costes,1, Naima Aron,1, Fabienne Reine,5, Laetitia
Herzog,5, Juan-Carlos Espinosa,2, Vincent Beringue5, & Olivier
Andréoletti1, Affiliations Contributions Corresponding author Journal name:
Nature Communications Volume: 5, Article number: 5821 DOI:
doi:10.1038/ncomms6821 Received 07 August 2014 Accepted 10 November 2014
Published 16 December 2014 Article tools Citation Reprints Rights &
permissions Article metrics
Abstract
Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant
Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie
prions remains unknown. Mice genetically engineered to overexpress the human
prion protein (tgHu) have emerged as highly relevant models for gauging the
capacity of prions to transmit to humans. These models can propagate human
prions without any apparent transmission barrier and have been used used to
confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie
prions transmit to several tgHu mice models with an efficiency comparable to
that of cattle BSE. The serial transmission of different scrapie isolates in
these mice led to the propagation of prions that are phenotypically identical to
those causing sporadic CJD (sCJD) in humans. These results demonstrate that
scrapie prions have a zoonotic potential and raise new questions about the
possible link between animal and human prions.
Subject terms: Biological sciences• Medical research At a glance
why do we not want to do TSE transmission studies on chimpanzees $
5. A positive result from a chimpanzee challenged severly would likely
create alarm in some circles even if the result could not be interpreted for
man. I have a view that all these agents could be transmitted provided a large
enough dose by appropriate routes was given and the animals kept long enough.
Until the mechanisms of the species barrier are more clearly understood it might
be best to retain that hypothesis.
snip...
R. BRADLEY
*** In complement to the recent demonstration that humanized mice are
susceptible to scrapie, we report here the first observation of direct
transmission of a natural classical scrapie isolate to a macaque after a 10-year
incubation period. Neuropathologic examination revealed all of the features of a
prion disease: spongiform change, neuronal loss, and accumulation of PrPres
throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of
scrapie to humans, at a time when protective measures for human and animal
health are being dismantled and reduced as c-BSE is considered controlled and
being eradicated.
*** Our results underscore the importance of precautionary and protective
measures and the necessity for long-term experimental transmission studies to
assess the zoonotic potential of other animal prion strains.
Saturday, April 23, 2016
SCRAPIE WS-01: Prion diseases in animals and zoonotic potential 2016
Prion. 10:S15-S21. 2016 ISSN: 1933-6896 printl 1933-690X online
Taylor & Francis
Prion 2016 Animal Prion Disease Workshop Abstracts
WS-01: Prion diseases in animals and zoonotic potential
Juan Maria Torres a, Olivier Andreoletti b, J uan-Carlos Espinosa a.
Vincent Beringue c. Patricia Aguilar a,
Natalia Fernandez-Borges a. and Alba Marin-Moreno a
"Centro de Investigacion en Sanidad Animal ( CISA-INIA ). Valdeolmos,
Madrid. Spain; b UMR INRA -ENVT 1225 Interactions Holes Agents Pathogenes. ENVT.
Toulouse. France: "UR892. Virologie lmmunologie MolécuIaires, Jouy-en-Josas.
France
Dietary exposure to bovine spongiform encephalopathy (BSE) contaminated
bovine tissues is considered as the origin of variant Creutzfeldt Jakob (vCJD)
disease in human. To date, BSE agent is the only recognized zoonotic prion.
Despite the variety of Transmissible Spongiform Encephalopathy (TSE) agents that
have been circulating for centuries in farmed ruminants there is no apparent
epidemiological link between exposure to ruminant products and the occurrence of
other form of TSE in human like sporadic Creutzfeldt Jakob Disease (sCJD).
However, the zoonotic potential of the diversity of circulating TSE agents has
never been systematically assessed. The major issue in experimental assessment
of TSEs zoonotic potential lies in the modeling of the ‘species barrier‘, the
biological phenomenon that limits TSE agents’ propagation from a species to
another. In the last decade, mice genetically engineered to express normal forms
of the human prion protein has proved essential in studying human prions
pathogenesis and modeling the capacity of TSEs to cross the human species
barrier.
To assess the zoonotic potential of prions circulating in farmed ruminants,
we study their transmission ability in transgenic mice expressing human PrPC
(HuPrP-Tg). Two lines of mice expressing different forms of the human PrPC
(129Met or 129Val) are used to determine the role of the Met129Val dimorphism in
susceptibility/resistance to the different agents.
These transmission experiments confirm the ability of BSE prions to
propagate in 129M- HuPrP-Tg mice and demonstrate that Met129 homozygotes may be
susceptible to BSE in sheep or goat to a greater degree than the BSE agent in
cattle and that these agents can convey molecular properties and
neuropathological indistinguishable from vCJD. However homozygous 129V mice are
resistant to all tested BSE derived prions independently of the originating
species suggesting a higher transmission barrier for 129V-PrP variant.
Transmission data also revealed that several scrapie prions propagate in
HuPrP-Tg mice with efficiency comparable to that of cattle BSE. While the
efficiency of transmission at primary passage was low, subsequent passages
resulted in a highly virulent prion disease in both Met129 and Val129 mice.
Transmission of the different scrapie isolates in these mice leads to the
emergence of prion strain phenotypes that showed similar characteristics to
those displayed by MM1 or VV2 sCJD prion. These results demonstrate that scrapie
prions have a zoonotic potential and raise new questions about the possible link
between animal and human prions.
Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Title: Transmission of scrapie prions to primate after an extended silent
incubation period
Authors
item Comoy, Emmanuel - item Mikol, Jacqueline - item Luccantoni-Freire,
Sophie - item Correia, Evelyne - item Lescoutra-Etchegaray, Nathalie - item
Durand, Valérie - item Dehen, Capucine - item Andreoletti, Olivier - item
Casalone, Cristina - item Richt, Juergen item Greenlee, Justin item Baron,
Thierry - item Benestad, Sylvie - item Hills, Bob - item Brown, Paul - item
Deslys, Jean-Philippe -
Submitted to: Scientific Reports Publication Type: Peer Reviewed Journal
Publication Acceptance Date: May 28, 2015 Publication Date: June 30, 2015
Citation: Comoy, E.E., Mikol, J., Luccantoni-Freire, S., Correia, E.,
Lescoutra-Etchegaray, N., Durand, V., Dehen, C., Andreoletti, O., Casalone, C.,
Richt, J.A., Greenlee, J.J., Baron, T., Benestad, S., Brown, P., Deslys, J.
2015. Transmission of scrapie prions to primate after an extended silent
incubation period. Scientific Reports. 5:11573.
Interpretive Summary: The transmissible spongiform encephalopathies (also
called prion diseases) are fatal neurodegenerative diseases that affect animals
and humans. The agent of prion diseases is a misfolded form of the prion protein
that is resistant to breakdown by the host cells. Since all mammals express
prion protein on the surface of various cells such as neurons, all mammals are,
in theory, capable of replicating prion diseases. One example of a prion
disease, bovine spongiform encephalopathy (BSE; also called mad cow disease),
has been shown to infect cattle, sheep, exotic undulates, cats, non-human
primates, and humans when the new host is exposed to feeds or foods contaminated
with the disease agent. The purpose of this study was to test whether non-human
primates (cynomologous macaque) are susceptible to the agent of sheep scrapie.
After an incubation period of approximately 10 years a macaque developed
progressive clinical signs suggestive of neurologic disease. Upon postmortem
examination and microscopic examination of tissues, there was a widespread
distribution of lesions consistent with a transmissible spongiform
encephalopathy. This information will have a scientific impact since it is the
first study that demonstrates the transmission of scrapie to a non-human primate
with a close genetic relationship to humans. This information is especially
useful to regulatory officials and those involved with risk assessment of the
potential transmission of animal prion diseases to humans. Technical Abstract:
Classical bovine spongiform encephalopathy (c-BSE) is an animal prion disease
that also causes variant Creutzfeldt-Jakob disease in humans. Over the past
decades, c-BSE's zoonotic potential has been the driving force in establishing
extensive protective measures for animal and human health.
*** In complement to the recent demonstration that humanized mice are
susceptible to scrapie, we report here the first observation of direct
transmission of a natural classical scrapie isolate to a macaque after a 10-year
incubation period. Neuropathologic examination revealed all of the features of a
prion disease: spongiform change, neuronal loss, and accumulation of PrPres
throughout the CNS.
*** This observation strengthens the questioning of the harmlessness of
scrapie to humans, at a time when protective measures for human and animal
health are being dismantled and reduced as c-BSE is considered controlled and
being eradicated.
*** Our results underscore the importance of precautionary and protective
measures and the necessity for long-term experimental transmission studies to
assess the zoonotic potential of other animal prion strains.
Wednesday, March 02, 2016
Endoscope Maker Olympus Agrees To $646 Million Settlement Over Kickbacks,
while still ignoring the elephant in the room, CJD TSE PRIONS Health Inc.
*** Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary
to Bramble et al ***
Evidence For CJD/TSE Transmission Via Endoscopes
From Terry S. Singletary, Sr flounder@wt.net 1-24-3
Thursday, April 28, 2016
Persistent residual contamination in endoscope channels; a fluorescence
epimicroscopy study
this must be on the forefront of research i.e. ‘iatrogenic’ transmission.
Alzheimer’s disease, iatrogenic, and Transmissible Spongiform
Encephalopathy TSE Prion disease, that is the question ???
>>> The only tenable public line will be that "more research is
required’’ <<<
>>> possibility on a transmissible prion remains
open<<<
O.K., so it’s about 23 years later, so somebody please tell me, when is
"more research is required’’ enough time for evaluation ?
http://web.archive.org/web/20040315075058/http://www.bseinquiry.gov.uk/files/yb/1992/12/16005001.pdf
SWISS MEDICAL WEEKLY
Alzheimer-type brain pathology may be transmitted by grafts of dura mater
26/01/2016 Singeltary comment ;
re-Evidence for human transmission of amyloid-β pathology and cerebral
amyloid angiopathy
Nature 525, 247?250 (10 September 2015) doi:10.1038/nature15369 Received 26
April 2015 Accepted 14 August 2015 Published online 09 September 2015 Updated
online 11 September 2015 Erratum (October, 2015)
snip...see full Singeltary Nature comment here;
Self-Propagative Replication of Ab Oligomers Suggests Potential
Transmissibility in Alzheimer Disease
*** Singeltary comment PLoS ***
Alzheimer’s disease and Transmissible Spongiform Encephalopathy prion
disease, Iatrogenic, what if ?
Posted by flounder on 05 Nov 2014 at 21:27 GMT
Sunday, November 22, 2015
*** Effect of heating on the stability of amyloid A (AA) fibrils and the
intra- and cross-species transmission of AA amyloidosis Abstract
Amyloid A (AA) amyloidosis is a protein misfolding disease characterized by
extracellular deposition of AA fibrils. AA fibrils are found in several tissues
from food animals with AA amyloidosis. For hygienic purposes, heating is widely
used to inactivate microbes in food, but it is uncertain whether heating is
sufficient to inactivate AA fibrils and prevent intra- or cross-species
transmission. We examined the effect of heating (at 60 °C or 100 °C) and
autoclaving (at 121 °C or 135 °C) on murine and bovine AA fibrils using Western
blot analysis, transmission electron microscopy (TEM), and mouse model
transmission experiments. TEM revealed that a mixture of AA fibrils and
amorphous aggregates appeared after heating at 100 °C, whereas autoclaving at
135 °C produced large amorphous aggregates. AA fibrils retained antigen
specificity in Western blot analysis when heated at 100 °C or autoclaved at 121
°C, but not when autoclaved at 135 °C. Transmissible pathogenicity of murine and
bovine AA fibrils subjected to heating (at 60 °C or 100 °C) was significantly
stimulated and resulted in amyloid deposition in mice. Autoclaving of murine AA
fibrils at 121 °C or 135 °C significantly decreased amyloid deposition.
Moreover, amyloid deposition in mice injected with murine AA fibrils was more
severe than that in mice injected with bovine AA fibrils. Bovine AA fibrils
autoclaved at 121 °C or 135 °C did not induce amyloid deposition in mice. These
results suggest that AA fibrils are relatively heat stable and that similar to
prions, autoclaving at 135 °C is required to destroy the pathogenicity of AA
fibrils. These findings may contribute to the prevention of AA fibril
transmission through food materials to different animals and especially to
humans.
Purchase options Price * Issue Purchase USD 511.00 Article Purchase USD
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*** Transmission of Creutzfeldt-Jakob disease to a chimpanzee by electrodes
contaminated during neurosurgery ***
Gibbs CJ Jr, Asher DM, Kobrine A, Amyx HL, Sulima MP, Gajdusek DC.
Laboratory of Central Nervous System Studies, National Institute of Neurological
Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892.
Stereotactic multicontact electrodes used to probe the cerebral cortex of a
middle aged woman with progressive dementia were previously implicated in the
accidental transmission of Creutzfeldt-Jakob disease (CJD) to two younger
patients. The diagnoses of CJD have been confirmed for all three cases. More
than two years after their last use in humans, after three cleanings and
repeated sterilisation in ethanol and formaldehyde vapour, the electrodes were
implanted in the cortex of a chimpanzee. Eighteen months later the animal became
ill with CJD. This finding serves to re-emphasise the potential danger posed by
reuse of instruments contaminated with the agents of spongiform
encephalopathies, even after scrupulous attempts to clean them.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8006664&dopt=Abstract
Sent: Monday, January 08,2001 3:03 PM
TO: freas@CBS5055530.CBER.FDA.GOV
FDA CJD BSE TSE Prion Scientific Advisors and Consultants Staff January
2001 Meeting Singeltary Submission
2001 FDA CJD TSE Prion Singeltary Submission
Thursday, April 14, 2016
Arizona 22 year old diagnosed with Creutzfeldt Jakob Disease CJD
just made a promise to mom, never forget, and never let them forget, before
we all do...
Heidenhain Variant Creutzfeldt Jakob Disease autopsy case report 'MOM'
DIVISION OF NEUROPATHOLOGY University of Texas Medical Branch 114
McCullough Bldg. Galveston, Texas 77555-0785
FAX COVER SHEET
DATE: 4-23-98
TO: Mr. Terry Singeltary @ -------
FROM: Gerald Campbell
FAX: (409) 772-5315 PHONE: (409) 772-2881
Number of Pages (including cover sheet):
Message:
*CONFIDENTIALITY NOTICE*
This document accompanying this transmission contains confidential
information belonging to the sender that is legally privileged. This information
is intended only for the use of the individual or entry names above. If you are
not the intended recipient, you are hereby notified that any disclosure, copying
distribution, or the taking of any action in reliances on the contents of this
telefaxed information is strictly prohibited. If you received this telefax in
error, please notify us by telephone immediately to arrange for return of the
original documents.
--------------------------
Patient Account: 90000014-518 Med. Rec. No.: (0160)118511Q Patient Name:
POULTER, BARBARA Age: 63 YRS DOB: 10/17/34 Sex: F Admitting Race: C
Attending Dr.: Date / Time Admitted : 12/14/97 1228 Copies to:
UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409)
772-1238 Fax (409) 772-5683 Pathology Report
FINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858
Autopsy NO.: AU-97-00435
AUTOPSY INFORMATION: Occupation: Unknown Birthplace: Unknown Residence:
Crystal Beach Date/Time of Death: 12/14/97 13:30 Date/Time of Autopsy: 12/15/97
15:00
Pathologist/Resident: Pencil/Fernandez Service: Private Restriction: Brain
only
FINAL AUTOPSY DIAGNOSIS
I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.
***TYPE: Anatomic(A) or Clinical(C) Diagnosis. IMPORTANCE: 1-immediate
cause of death (COD); 2.ureterlying COD; 3-contributory COD: 4.concomitant,
significant; 5-incidental ***
Patient Name: POULTER, BARBARA Patient Location: AUTOPSY Room/Bed: Printed
Date; Time: 01/30/98 - 0832
Page: 1 Continued ....
--------------
UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409)
772-1238 Fax (409) 772-5683
Pathology Report
Autopsy NO,: AU-97-00435
MICROSCOPIC DESCRIPTION: The spongiform change is evident in all areas of
neocortex, varying from mild to moderate in severity with only very mild
neuronal loss and gliosis. In the bilateral occipital lobes, there is severe
loss cortical neurons and gliosis, with a corresponding pallor of the underlying
white matter. There is only minimal, focal spongiform change in corpus striatum,
lentiform nuclei, thalamus, hippocampus, brainstem and cerebellum. There is no
significant loss of neurons from the lateral geniculate nucleus, and the optic
chiasm and tracts are well-myelinated.
SECTIONS TAKEN: N-l) Pituitary, N-2) Right frontal, N-3) Right inferior
frontal, N-4) Right caudate putamen. N-5) Right lentiform nuclei, N-6) Right
hippocampus, N-7) optic chiasm. N-8) Left inferior temporal lobe, N-9) Right
inferior occipital, N-10} Cerabellum. N-l1) Midbrain, N-12) Pons, N-13) Medulla.
FINAL DIAGNOSES: BRAIN: 1. Clinical history of rapidly progressive
dementia, clinically consistent with Creutzfeldt-Jakob Disease.
a. spongiform encephalopathy, most Severe in occipital lobes, consistent
with Heidenhain variant of Creutzfeldt-Jakob disease.
b. Ventriculer enlargement, moderate, consistent with atrophy. 1.
Communicating spherical enlargement of occipital horn of left lateral ventricle
(possible incidental congenital anomaly).
DURA; Left subdural hemorrhage, recent, minimal.
PITUITARY: Severe capillary congestion.
COMMENTS; See also western blot report from Dr. Gambetti's lab Amyloid
stains are not completed for this case as of this date. The results, which are
not essential for the diagnosis, will be reported separately in an addendum.
(this was hand written notes) no amyloid evident in the special stains. no
evidence of plaques.GAE
Gerald A. Campbell, M.D., Pathologist Division of Neuropathology
(Electronic Signature}. (Gross: 01/16/98 Final: 02/08/98
Patient Name: POULTER, BARBARA Patient Location: AUTOPSY Room/Bed: Printed
Date: Time: 02/09/98 - 1120
Page 2 END OF REPORT -------
UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409)
772-1238 fax (409) 772-5683 Pathology Report
Date/Time of Death: 12/14/97 Autopsy No.: AU-97-00435
NEUROPATHOLOGY CONSULTATION
CLINICAL HISTORY This patient was a 63-year-old white female with recent
onset of progressive dementia. She was well until September of this year, when
she noted a decrease in her visual activity and was found to have visual field
defects as well. MRI revealed no lesions in the orbits or optic pathways. She
was admitted to the hospital with the working diagnosis of bilateral optic
neuropathy for a course of intravenous methylprednisolone, but her vision
continued to deteriorate. She developed increasing memory and speech impairment,
weakness and myoclonus. She died on 12/14/97, approximately three and one-half
months after her symptoms started.
Date/Time of Death: 12/14/97 13:30 Date/Time Autopsy: 12/15/97 15:00
Pathologist Resident: PENCIL/FERNANDEZ
GROSS DESCRIPTION: Submitted are the brain, convexity dura and pituitary
gland.
The pituitary gland is very dark and almost hemorrhagic in appearance, but
has no obvious hematoma. It is submitted totally for histology.
The right convexity dura has diffuse but minimal subdura hemorrhage, and
the dura is otherwise unremarkable.
The brain is normally developed with normal size for an adult and is
symmetric externally. It does not have apparent sulcal widening. There is mild
congestion of the leptomeninges, which are transparent. There is no evidence of
inflammatory exudete. There is no evidence of internal softenings or other
lesions externally. The cerebral arteries have focal atherosclerosis, but are
without significant compromise of the vessels lumens. There is no evidence of
aneurysms or malformations.
The hemispheres are sliced coronally revealing, a ventricular system which
is mildly enlarged. The cortical ribbon is normal in thickness throughout most
of the brain, except for the inferior and medial occipital lobes bilaterally,
where the cortex is firm, thin and has a brownish discoloration, more severely
so on the left than the right. In addition there is a spherical enlargement of
the left occipital horn of the lateral ventricle which communicates with the
remainder of the lateral ventricle. The tissue of the white matter around this
enlargement is somewhat softer then in other areas. Other areas of the brain are
grossly unremarkable. The brainstem and cerebellum are sliced transversely,
revealing normal development and no evidence of gross changes or lesions.
DICTATED BY: GERALD A. CAMPBELL, M.D., PATHOLOGIST 01/16/98
Page 1 Continued ....
---------------
Patient Account: 90000014-518 Med. Rec. No,: (0160)118511Q
Patient Name: POULTER, BARBARA Age: 63 YRS DOB: 10/17/34 Sex:F Race:C
Admitting Dr.: Attending Dr: Date/Time Admitted: 12/14/97 1228
UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409)
772-1238 Fax (409) 772-5683 Pathology Report
FINAL AUTOPSY REPORT Autopsy Office (409)772-2858 Autopsy No.: AU-97-00435
CLINICAL SUMMARY:
This is a 63-year-old white female with a recent onset of progressive
dementia. Her past medical history is significant for hypothyroidism. She was
well until September of this year, when she noted visual difficulty. By
mid-October, she could not read the newspaper. She was found to have a decrease
in visual acuity and visual field defects. One week after her initial
evaluation, a panel of blood tests showed no significant abnormalities and a MRI
revealed some periventricular white matter "plaque-like" areas but no lesions in
the orbits or optic pathways.
The patient had continued deterioration and distortion of her vision. The
visual field defects increased, and she was found to have paracentral scotomas
which were thought to be consistent with bilateral optic neuropathy. Early in
November, she was admitted to the hospital for a course of intravenous methyl
prednisolone.
During her hospital stay, she was noted to have short term memory and
speech impairment; her vision did not improve. She was discharged with the
diagnosis of Creutzfeldt-Jakob disease.
Later, the patient developed progressive dementia with marked impairment of
speech and memory. She had complete visual loss, increased weakness and
myoclonus. She died on December 14, 1997.
MF /AV 12/16/97
Patient Name: POULTER, BARBARA Patient Location: AUTOPSY Room/Bed: Printed
Date / Time: 01//30/98 - 0832 Page: 2 Continued .... --------------
Patient Account: 90000014-518 Med. Rec. No.: (0160)118511Q Patient Name:
POULTER, BARBARA Age: 63 YRS DOB: 10/17/34 Sex: F Race: C Admitting Dr.:
Attending Dr.: Date / Time Admitted : 12/14/97 1228
UTMB University of Texas Medical Branch Galveston. Texas 77555-0543 (409)
772-1238 Fax (409) 772-5683 Pathology Report
AU-97-00435
GROSS DESCRIPTION:
EXTERNAL EXAMINATION: The body is that of a 63-year-old well-nourished,
well-developed white female. There is no rigor mortis present, and there is
unfixed dependent lividity on the posterior surface. The head is normocephalic
with a moderate amount of gray, medium length scalp hair. The irides are blue
with equal pupils measuring 0.4 mm in diameter. The nares are patent with no
exudate. Dentition is fair. Buccal membranes are normal. There is normal female
hair distribution. The chest does not have increased anterior-posterior
diameter. The abdomen is slightly protuberant. Lymph node enlargement is not
present. The extremities are unremarkable. The genitalia are those of a normal
female. Two well-healed remote scars are identified in the abdomen: one in the
right upper quadrant and another in the superpubic area.
BRAIN: The brain weighs 1450 gm. The gyri and sulci display a normal
pattern without edema or atrophy. The meninges show no abnormalities. The circle
of Willis, basilar and vertebral arteries show no significant atherosclerosis.
The brain is fixed in formalin for later examination by a neuropathologist (see
neuropathology report). No indentation of the cingulate gyri, unci or molding of
the cerebellar tonsils are noted.
SPINAL CORD: The spinal cord is not removed.
PITUITARY GLAND: The pituitary gland is removed and is fixed in formalin
for subsequent examination by a neuropathologist.
MF /AV 12/16/97
Patient Name: POULTER, BARBARA Patient Location: AUTOPSY Room/Bed: Printed
Date / Time: 01/30/98 - 0832
Page 3 Continued ....
--------------
Patient Account : 90000014-518 Med. Rec. No.: (0160)118511Q patient Name:
POULTER, BARBARA Age: 63 YRS DOB: 10/17/34 Sex: F Race: C Admitting Dr.:
Attending Dr,: Date/Time Admitted: 12/14/97 1228
UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409)
772-1238 Fax (409) 772-5683
Pathology Report AU-97-00435
MICROSCOPIC DESCRIPTION:
BRAIN: Histologic examination of multiple sampled areas of the brain showed
the characteristic features of Creutzfetdt-Jakob disease. These were present in
most sections, but were particularly prominent in the occipital cortex. The
spongiform degeneration was seen in the neuropil of the gray matter as multiple
vacuoles amoung numerous reactive astrocytes and occasional neuronal cell
bodies. These changes were most notable in the basal layer of the cortex. PAS
and amyloid stains will be performed on selected sections to asses the presence
of plaques.
MF /MF 01/28/98
Patient Name: POULTER, BARBARA Patient Location: AUTOPSY Room/Bed: Printed
Date / Time: 01/30/98 - 0832
Page: 4 Continued .... --------------
Patient Account: 90000014-518 Med. Rec. No.: (0160}118511Q Patient Name:
POULTER, BARBARA Age: 63 YRS DOB: 10/17/34 Sex: F Race: C Admitting Dr.:
Attending Dr.: Date / Time Admitted : 12/14/97 1228
UTMB University of Texas Medical Branch Galveston, Texas 775550-0543 (409)
772-1238 Fax (409) 772-5683 Pathology Report
Autopsy office (409)772-2858 Autopsy No.: AU-97-00435
FINAL AUTOPSY REPORT
CLINICOPATHOLOGIC CORRELATION:
The clinical findings in this case strongly suggest the diagnosis of
Creutzfeldt-Jakob disease: progressive dementia, typical EEG changes, visual
disturbances and myoclonus. These characteristics indicate this is a "probable
case of CJD", according the criteria set by the EC Surveillance Group of
Creutzfeldt-Jakob Disease in Europe (1).
The definitive diagnosis of Creutzfeldt-Jakob disease, however, is
established by neuropathologic findings. There are three changes that are
classically described and considered diagnostic: spongiform change, neuronal
loss and astrocytic gliosis. The presence of these can vary significantly in
proportion and distribution and often correlate with clinical symptoms. This
permits classification of the disease into several variants.
Three variants of Creutzfeldt-Jakob disease have been proposed by Roos and
Gajdusek (2): frontopyramidal, with pyramidal or lower motor neuron involvement;
occipitoparietal {Heidenhain), characterized by disorders in higher cortical
function and vision; and diffuse, with cerebral, cortical, basal ganglia,
thalamic, cerebellar, midbrain and spinal cord involvement.
Histological examination from multiple samples of the brain in this case
revealed astrocytic gliosis, spongiform degeneration and neuronal loss. Although
these changes were seen in most sections, they were most prominent in the
occipital cortex. This correlates very well with the clinical history of visual
disturbances. Based on this finding, the present case corresponds to the
Heidenhain variant. It is not uncommon for Creutzfeldt-Jakob disease to present
with visual symptoms as the initial manifestation of the disease. Vargas et al
(3) has reported three cases with these characteristics.
There have been numerous and significant advances in our understanding of
Creutzfeldt-Jakob disease and prion diseases in general. These have been
reviewed in several papers written recently, including one by Horowich and
Weissman (4).
In summary, this 63 year old female with a history of visual disturbances
and dementia of rapid progression was found to have the neuropathologic changes
characteristic of Creutzfeldt-Jakob disease, predominantly in the occipital
cortex. The occipital tropism and consequent visual symptoms indicate this case
corresponds to the Heidenhain variant.
REFERENCES:
Patient Name: POULTER, BARBARA Patient location: AUTOPSY Room/Bed: Printed
Date / Time: 01/30/98 * 0832
Page: 5 Continued ....
--------------
Patient Account: 90000014-518 Med. Rec. No.: (0160)118511Q Patient Name:
POULTER, BARBARA Age: 63 YRS DOB: 10/17/34 Sex: F Race: C Admitting Dr.:
Attending Dr.: Date / Time Admitted : 12/14/97 1228
UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409)
772-1238 Fax (409} 772-5683 Pathology Report
Autopsy No.: AU-97-00435
FINAL AUTOPSY REPORT
CLINICOPATHOLOGIC CORRELATION:
1. Budka H, et al: Tissue handling in suspected Creutzfeldt-Jakob disease
(CJD) and other human spongiform encephalopathies (prion diseases), Brain
Pathology. 5:319-322,1995.
2. Roos R, Gajdusek DC, Gibbs CJ Jr: The clinical characteristics of
transmissible Creutzfeldt-Jakob disease. Brain 96: 1-20, 1973.
3. Vargas ME, et al: Homonymous field defect as the first Manifestation of
Creutzfeldt-Jakob disease. American Journal of Ophthalmology. 119:497-504, 1995.
4. Horowich AL, Weissman JS: Deadly conformations - protein misfolding in
prion disease. Cell Vol.89, 499-510, 1997.
MF /MF 01/28/98
SCOT D. PENCIL, M.D., PATHOLOGIST MARTIN FERNANDEZ, M.D. 01/29/98
(Electronic Signature)
Patient Name: POULTER, BARBARA Patient Location: AUTOPSY Room/Bed: Printed
Date / Time: 01/30/98 - 0832
Page: 6 END OF REPORT
--------------
The University of Texas Medical Branch at Galveston
Gerald A, Campbell, Ph.D., M.D, Associate Professor and Director Division
of Neuropathology Department of Pathology
February 26, 1998
Pierluigi Gambetti, M.D. Professor Institute of Pathology Case Western
Reserve University 2085 Adelbert Road Cleveland Ohio 44106
Dear Dr, Gambetti:
Enclosed please find the microscopic slides and autopsy report from our
patient, Barbara Poulter (Hosp.# 11851lQ, Autopsy # AU97-435). These slides are
being sent for consultation at the request of Mr. Singletary, Ms. Poulter's son
and next of kin. We will also send frozen tissue from the brain on dry ice next
week, and someone will call you on the day the tissue is shipped. Please return
the slides when you have finished with your examination. If you need any further
information, please do not hesitate to call me. Thanks for your assistance with
this case.
Sincerely, Gerald A. Campbell
------------------
CASE WESTERN RESERVE UNIVERSITY
February 26, 1988
Gerald Campbell, M.D,, PhD. Division of Neuropathology, G85 University TX
Medical Branch Galveston, TX 77555-0785
Dear Dr. Campbell,
As per our telephone conversation concerning a recent case of CJD, I Will
be willing to examine slides and the frozen tissue on western blotting, I will
issue a report to you about our conclusions. Below is my address, Our Fed Ex
number is XXXXXXXXXXXXXXX.
Thank your for your assistance in this matter,
Best personal regards,
Pierluigi Gambetti, M.D.
PG:In
Division of Neuropathology Pierluigi Gambetti, M.D. Director Institute Of
Neuropathology 2085 Adelbert Road Cleveland, Ohio 44106
Phone 216-368-0587 Fax 216-368-2546
------------------
CASE WESTERN RESERVE UNIVERSITY
February 27, 1998
Dr. Gerald A. Campbell The University of Texas Medical Branch at Galveston
Division of Neuropathology, G85 Galveston. TX 77555-0785
Dear Dr. Campbell,
We are in receipt of the slides you sent on Mrs. Barbara Poulter (your #:
AU97-435;our#098-28).
Best personal regards, Pierluigi Gambetti, M.D.
PG:sb
Division of Neuropathology Pierluigi Gambetti, M.D., Director
-----------------------------------
CASE WESTERN RESERVE UNIVERSITY
March 30, 1998
Dr. Gerald A, Campbell The University of Texas Medical Branch at Galveston
Division of Neuropathology Department of Pathology Galveston, Texas
Dear Dr Campbell,
We performed Western immunoblot analysis on the frozen tissue from your
case #AU97-435 (our #098-28). The Immunoblot reveals the presence of
protease-resistant prion protein (PrPres) confirming the diagnosis of prion
disease. The immunoblot pattern of PrPres is consistent with the diagnosis of
Creutzfeldt-Jakob disease.
Thank you for referring to us this interesting case.
Sincerely,
Piero Parchi, M.D.
Pierluigi Gambetti, M.D.
PP:sb
Division of Neuropathology Pierluigi Gambetti, M.D., Director Case Western
Reserve University
This Autopsy report is for the use of anyone, who is trying to understand
this hideous disease CJD. I hope it can be beneficial for some in researching
human TSE. Please remember, this was my Mom, and to use this with great respect.
thank you, kind regards,
Terry S. Singeltary Sr., Bacliff, Texas USA
-------------------------------
Sunday, January 17, 2016
Of Grave Concern Heidenhain Variant Creutzfeldt Jakob Disease
sorry, this email started out to be short. I got a bit long winded. could
have been much longer though...
kindest regards, terry
I’m still here damn’t....
Terry S. Singeltary Sr. Bacliff, Texas USA 77518 flounder9@verizon.net
posted by Terry S. Singeltary Sr. at
2:25 PM
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