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From: TSS ()
Subject: Geographical BSE Risk: EFSA consults on revision of assessment methodology
Date: November 21, 2006 at 8:12 am PST

Geographical BSE Risk: EFSA consults on revision of assessment methodology

Last updated: 21 November 2006 Publication Date: 21 November 2006

Today, EFSA’s Panel on Biological Hazards (BIOHAZ) launched a public consultation on a revision of the methodology for Geographical BSE-Risk (GBR) assessment. The European Commission uses this scientific advice as the basis for attributing BSE risk status to countries worldwide.

Press release
Today, EFSA’s Panel on Biological Hazards (BIOHAZ) launched a public consultation on a revision of the methodology for Geographical BSE-Risk (GBR) assessment. The European Commission uses this scientific advice as the basis for attributing BSE risk status to countries worldwide. The update takes account of new scientific knowledge on BSE and recent trends in BSE prevalence based on the most recent surveillance data. By allowing a more accurate assessment of geographical BSE risk, the revised methodology will assist risk managers in taking decisions to protect consumers which are commensurate with the risk identified.

Since 2003, EFSA is responsible for assessing Geographical BSE Risk, by which countries are classified according to their level of risk with respect to BSE. This work is fundamental for consumer protection and also has implications for global trade. EFSA has so far carried out GBRs for 19[1] countries, applying a methodology originally developed in 1998[2].

EFSA’s proposal to revise the methodology for assessing Geographical BSE risk includes innovations and changes at different levels. In particular, it takes account of:
predicted development of BSE risk over time (including a possible reduction of such risk);
steps taken by countries to control and reduce the risk of BSE;
data now available on the epidemiological surveillance of BSE in cattle since 2001 in all EU Member States:
the need not to overstate risk in countries with a low BSE prevalence but large cattle populations;
harmonisation with the guidelines of the World Organisation for Animal Health (OIE).

EFSA is consulting widely on its proposed approach to ensure the update is done openly and transparently and to allow input from all interested parties. Contributions can be made on the draft opinion until 14th January 2007, by visiting the EFSA website at:

Notes for editors:
Geographical BSE-Risk (GBR) is a qualitative indicator of the likelihood of the presence of one or more cattle being infected with BSE, pre-clinically as well as clinically, at a given point in time, in a country. Where its presence is confirmed, the GBR gives an indication of the level of infection.

For media enquiries, please contact:

Alun Jones, press officer,
Tel: +39 0521 036 487
Anne-Laure Gassin, EFSA Communications Director,
Tel : +39 0521 036 248
Mobile : +39 348 640 3434


[1] Argentina, Australia, Botswana, Brazil, Canada, Chile, Costa Rica, El Salvador, Namibia, Nicaragua, Norway, Mexico, Panama, Paraguay, South Africa (EFSA self task), Swaziland, Sweden, United States of America, and Uruguay.
[2] The methodology was originally developed by the Scientific Steering Committee (SSC) of the European Commission, and was subsequently revised in the following opinions:
- Preliminary-opinion on a method to assess the geographical BSE-Risk of Countries or Regions (adopted on 10 December 1998).
- Final opinion on the Geographical Risk of Bovine Spongiform Encephalopathy (GBR) (adopted on 6 July 2000).
- Updated opinion on the Geographical Risk of Bovine Spongiform Encephalopathy (GBR) (adopted on 11 January 2002).


On the Question of Sporadic

or Atypical Bovine SpongiformEncephalopathy and

Creutzfeldt-Jakob Disease

Paul Brown,* Lisa M. McShane,† Gianluigi Zanusso,‡ and Linda Detwiler§

Strategies to investigate the possible existence of sporadic

bovine spongiform encephalopathy (BSE) require

systematic testing programs to identify cases in countries

considered to have little or no risk for orally acquired disease,

or to detect a stable occurrence of atypical cases in

countries in which orally acquired disease is disappearing.

To achieve 95% statistical confidence that the prevalence

of sporadic BSE is no greater than 1 per million (i.e., the

annual incidence of sporadic Creutzfeldt-Jakob disease

[CJD] in humans) would require negative tests in 3 million

randomly selected older cattle. A link between BSE and

sporadic CJD has been suggested on the basis of laboratory

studies but is unsupported by epidemiologic observation.

Such a link might yet be established by the discovery

of a specific molecular marker or of particular combinations

of trends over time of typical and atypical BSE and various

subtypes of sporadic CJD, as their numbers are influenced

by a continuation of current public health measures that

exclude high-risk bovine tissues from the animal and

human food chains. ......


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

3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse
Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western Reserve
Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years. These results indicate that BASE is
transmissible to humans and suggest that BASE is more virulent than
classical BSE in humans.

6:30 Close of Day One

but i think science has pretty much proven the USA has a serious problem
from all TSEs ;


This study reveals remarkable international differences in the HTSE panorama

change with time, as seen from deaths in eleven countries in the period

Knowledge of possible biases in the study cohort is vital for future
applications of this

dataset, both in clinical/epidemiological research and in public health



1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...

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

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

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


Research Project: Study of Atypical Bse

Location: Virus and Prion Diseases of Livestock

Project Number: 3625-32000-073-07

Project Type: Specific C/A

Start Date: Sep 15, 2004

End Date: Sep 14, 2007


The objective of this cooperative research project with Dr. Maria Caramelli
from the Italian BSE Reference Laboratory in Turin, Italy, is to

conduct comparative studies with the U.S. bovine spongiform encephalopathy
(BSE) isolate and the atypical BSE isolates identified in Italy.

The studies will cover the following areas: 1. Evaluation of present
diagnostics tools used in the U.S. for the detection of atypical BSE cases.

Molecular comparison of the U.S. BSE isolate and other typical BSE isolates
with atypical BSE cases. 3. Studies on transmissibility and tissue

distribution of atypical BSE isolates in cattle and other species.


This project will be done as a Specific Cooperative Agreement with the
Italian BSE Reference Laboratory, Istituto

Zooprofilattico Sperimentale del Piemonte, in Turin, Italy. It is essential
for the U.S. BSE surveillance program to

analyze the effectiveness of the U.S diagnostic tools for detection of
atypical cases of BSE. Molecular comparisons of

the U.S. BSE isolate with atypical BSE isolates will provide further
characterization of the U.S. BSE isolate.

Transmission studies are already underway using brain homogenates from
atypical BSE cases into mice, cattle and

sheep. It will be critical to see whether the atypical BSE isolates behave
similarly to typical BSE isolates in terms of

transmissibility and disease pathogenesis. If transmission occurs, tissue
distribution comparisons will be made between

cattle infected with the atypical BSE isolate and the U.S. BSE isolate.
Differences in tissue distribution could require

new regulations regarding specific risk material (SRM) removal.

Page 5 of 98



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




>> Differences in tissue distribution could require new regulations >>
regarding specific risk material (SRM) removal.


full text 33 PAGES ;

It was, however, performed in the USA in 1979, when it was shown that cattle
inoculated with the scrapie agent endemic in the flock of Suffolk sheep at
the United States Department of Agriculture in Mission, Texas, developed a
TSE quite unlike BSE. 32 The findings of the initial transmission, though
not of the clinical or neurohistological examination, were communicated in
October 1988 to Dr Watson, Director of the CVL, following a visit by Dr
Wrathall, one of the project leaders in the Pathology Department of the CVL,
to the United States Department of Agriculture. 33 The results were not
published at this point, since the attempted transmission to mice from the
experimental cow brain had been inconclusive. The results of the clinical
and histological differences between scrapie-affected sheep and cattle were
published in 1995. Similar studies in which cattle were inoculated
intracerebrally with scrapie inocula derived from a number of
scrapie-affected sheep of different breeds and from different States, were
carried out at the US National Animal Disease Centre. 34 The results,
published in 1994, showed that this source of scrapie agent, though
pathogenic for cattle, did not produce the same clinical signs of brain
lesions characteristic of BSE.

1: J Infect Dis. 1994 Apr;169(4):814-20.

Intracerebral transmission of scrapie to cattle.

Cutlip RC, Miller JM, Race RE, Jenny AL, Katz JB, Lehmkuhl HD, DeBey BM,
Robinson MM.

USDA, Agriculture Research Service, National Animal Disease Center, Ames, IA

To determine if sheep scrapie agent(s) in the United States would induce a
disease in cattle resembling bovine spongiform encephalopathy, 18 newborn
calves were inoculated intracerebrally with a pooled suspension of brain
from 9 sheep with scrapie. Half of the calves were euthanatized 1 year after
inoculation. All calves kept longer than 1 year became severely lethargic
and demonstrated clinical signs of motor neuron dysfunction that were
manifest as progressive stiffness, posterior paresis, general weakness, and
permanent recumbency. The incubation period was 14-18 months, and the
clinical course was 1-5 months. The brain from each calf was examined for
lesions and for protease-resistant prion protein. Lesions were subtle, but a
disease-specific isoform of the prion protein was present in the brain of
all calves. Neither signs nor lesions were characteristic of those for
bovine spongiform encephalopathy.

MeSH Terms: Animals Brain/microbiology* Brain/pathology Cattle Cattle
Diseases/etiology* Cattle Diseases/pathology Encephalopathy, Bovine
Spongiform/etiology* Encephalopathy, Bovine Spongiform/pathology
Immunoblotting/veterinary Immunohistochemistry Male Motor Neurons/physiology
Prions/analysis Scrapie/pathology Scrapie/transmission* Sheep Sleep Stages
Time Factors

Substances: Prions


Page 16 of 17

Intracerebral transmission of scrapie to cattle FULL TEXT PDF;



WE conclude that American sources of sheep scrapie are transmissible to
cattle by direct intracerebral inoculation but the disease induced is NOT
identical to BSE as seen in the United Kingdom. While there were
similarities in clinical signs between this experimental disease and BSE,
there was no evidence of aggressiveness, hyperexcitability, hyperesthesia
(tactile or auditory), or hyperemetria of limbs as has been reported for BSE
(9). Neither were there extensive neurologic lesions, which are primary for
BSE, such as severe vacuolation of neurons and neuropil or neuronal necrosis
and gliosis. Although some vacuolation of neuropil, chromotolysis in
neurons, and gliosis were seen in the brains of some affected calves, these
were industinguishable from those of controls. Vacuolated neurons in the red
nucleus of both challenged and normal calves were considered normal for the
bovines as previously described (50).

the amount of PrP-res positively related to the length of the incubation.


WE also conclude from these studies that scrapie in cattle MIGHT NOT BE
THAT DETECTION OF PrP-res by immunohistochemistry or immunoblotting is
necessary to make a definitive diagnosis. THUS, undiagnosed scrapie
infection could contribute to the ''DOWNER-COW'' syndrome and could be
responsible for some outbreaks of transmissible mink encephalopathy proposed
by Burger and Hartsough

(8) and Marsh and harsough (52). ...


Multiple sources of sheep affected with scrapie and two breeds of cattle
from several sources were used inthe current study in an effort to avoid a
single strain of either agent or host. Preliminary results from mouse
inoculations indicate multiple strains of the agent were present in the
pooled inoculum (unpublished data). ...

Transmission of the sheep scrapie to cattle was attempted in 1979 by using
intracerebral, intramuscular, subcutaneous, and oral routes of inoculation
of 5, 8- to 11-month old cattlw with a homologous mixture of brain from 1
affected sheep (61, 62). ONE of the 5 cattle develped neurologic signs 48
months after inoculation. Signs were disorientation, incoordination, a
stiff-legged stilted gait, progressive difficulty in rising, and finally in
terminal recumbency. The clinical course was 2.5 months. TWO of the 5 cattle
similarly inoculated with brain tissue from a goat with scrapie exhibited
similar signs 27 and 36 months after incoluation. Clinical courses were 43
an 44 days. Brain lesions of mild gliosis and vacuolation and mouse
inoculation data were insufficient to confirm a diagnosis of scrapie. This
work remained controversial until recent examination of the brains detected
PrP-res in all 3 cattle with neurologic disease but in none of the
unaffected cattle (62). Results of these studies are similar to ours and
underscore the necessity of methods other than histopathology to diagnose
scrapie infection in cattle. We believe that immunologic techniques for
detecting PrP-res currently provide the most sensitive and reliable way to
make a definitive diagnosis...

Visit to USA ... info on BSE and Scrapie;f=12;t=000385



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

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

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


Page 17 of 17

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

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

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



THE infamous USA SPORADIC CJDs, something to ponder;

IF the USA TSE in cattle all does not look like UK BSE, why would all USA
human TSE look like UK nvCJD???

over 20 strains of scrapie documented to date with new atypical strains now
being documented in sheep and goat i.e. BSE.

atypical strains of BSE/TSE showing up in cattle in different countries?

ALL animals for human/animal consumption must be tested for TSE.

ALL human TSEs must be made reportable Nationally and Internationally, OF

IN a time when FSIS/APHIS/USDA/FDA et al should be strengthening the TSE
regulations, it seems corporate interest has won out again over sound
science and consumer protection from an agent that is 100% fatal for the
ones that go clinical. With the many different atypical TSEs showing up in
different parts of the world, and with GWs BSE MRR policy (the legal policy
of trading all strains of TSEs), the battle that has waged for the last 25
years to eradicate this agent from this planet will be set back decades, if
not lost for good. ...

Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518



Full Text

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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




Terry S. Singeltary SR.
P.O. Box 42
Bacliff, Texas USA 77518

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