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

**Date:** August 24, 2005 at 2:47 pm PST

August 24, 2005

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

Greetings APHIS ET AL,

My name is Terry S. Singeltary Sr.

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

PROPOSED RULES

Exportation and importation of animals and animal products:

Whole cuts of boneless beef from-

Japan,

48494-48500 \[05-16422\]

\[Federal Register: August 18, 2005 (Volume 70, Number 159)\]

\[Proposed Rules\]

\[Page 48494-48500\]

From the Federal Register Online via GPO Access \[wais.access.gpo.gov\]

\[DOCID:fr18au05-7\]

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

Proposed Rules

Federal Register

\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

This section of the FEDERAL REGISTER contains notices to the public of

the proposed issuance of rules and regulations. The purpose of these

notices is to give interested persons an opportunity to participate in

the rule making prior to the adoption of the final rules.

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

\[\[Page 48494\]\]

DEPARTMENT OF AGRICULTURE

Animal and Plant Health Inspection Service

9 CFR Part 94

\[Docket No. 05-004-1\]

RIN 0579-AB93

Importation of Whole Cuts of Boneless Beef from Japan

AGENCY: Animal and Plant Health Inspection Service, USDA.

ACTION: Proposed rule.

\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\--

SUMMARY: We are proposing to amend the regulations governing the

importation of meat and other edible animal products by allowing, under

certain conditions, the importation of whole cuts of boneless beef from

Japan. We are proposing this action in response to a request from the

Government of Japan and after conducting an analysis of the risk that

indicates that such beef can be safely imported from Japan under the

conditions described in this proposal.

DATES: We will consider all comments that we receive on or before

September 19, 2005.

ADDRESSES: You may submit comments by any of the following methods:

EDOCKET: Go to http://www.epa.gov/feddocket to submit or

snip\...

BSE infectivity has never been demonstrated in the muscle tissue of

cattle experimentally or naturally infected with BSE at any stage of

the disease. Studies performed using TSEs other than BSE in non-bovine

animals have detected prions in muscle tissue. However, the

international scientific community largely considers that these studies

cannot be directly extrapolated to BSE in cattle because of the

significant interactions between the host species and the prion strain

involved.

Pathogenesis studies of naturally and experimentally infected

cattle have not detected BSE infectivity in blood. However,

transmission of BSE was demonstrated in sheep that received a

transfusion of a large volume of blood drawn from other sheep that were

experimentally infected with the BSE agent. The United Kingdom\'s

Department for Environment, Food and Rural Affairs\' Spongiform

Encephalopathy Advisory Committee (SEAC) and the European Commission\'s

Scientific Steering Committee (SSC), which are scientific advisory

committees, evaluated the implication of this finding in relation to

food safety.\\5\\ The SEAC concluded that the finding did not represent

grounds for recommending any changes to the current control measures

for BSE. The SSC determined that the research results do not support

the hypothesis that bovine blood or muscle meat constitute a risk to

human health.\\6\\

snip\...

BSE Risk Factors for Whole Cuts of Boneless Beef

The most significant risk management strategy for ensuring the

safety of whole cuts of boneless beef is the prevention of cross-

contamination of the beef with SRMs during stunning and slaughter of

the animal. Control measures that prevent contamination of such beef

involve the establishment of procedures for the removal of SRMs,

prohibitions on air-injection stunning and pithing, and splitting of

carcasses. These potential pathways for contamination and the control

measures that prevent contamination are described in detail in the risk

analysis for this rulemaking.

SRM Removal. Research has demonstrated that SRMs from infected

cattle may contain BSE infectivity. Because infectivity has not been

demonstrated in muscle tissue, the most important mitigation measure

for whole cuts of boneless beef is the careful removal and segregation

of SRMs. Removal of SRMs in a manner that avoids contamination of the

beef with SRMs minimizes the risk of exposure to materials that have

been demonstrated to contain the BSE agent in cattle.

snip\...

Variant Creutzfeldt-Jakob disease (vCJD), a chronic and fatal

neurodegenerative disease of humans, has been linked since 1996 through

epidemiological, neuropathological, and experimental data to exposure

to the BSE agent, most likely through consumption of cattle products

contaminated with the agent before BSE control measures were in place.

To date, approximately 170 probable and confirmed cases of vCJD have

been identified worldwide. The majority of these cases have either been

identified in the United Kingdom or were linked to exposure that

occurred in the United Kingdom, and all cases have been linked to

exposure in countries with native cases of BSE. Some studies estimate

that more than 1 million cattle may have been infected with BSE

throughout the epidemic in the United Kingdom. This number of infected

cattle could have introduced a significant amount of infectivity into

the human food supply. Yet, the low number of cases of vCJD identified

to date indicates that there is a substantial species barrier that

protects humans from widespread illness due to exposure to the BSE

agent.

snip\...

International Guidelines on BSE

International guidelines for trade in animal and animal products

are developed by the World Organization for Animal Health (formerly

known as the Office International des Epizooties (OIE)), which is

recognized by the World Trade Organization (WTO) as the international

organization responsible for the development of standards, guidelines,

and recommendations with respect to animal health and zoonoses

(diseases that are transmissible from animals to humans). The OIE

guidelines for trade in terrestrial animals (mammals, birds, and bees)

are detailed in the Terrestrial Animal Health Code (available on the

internet at http://www.oie.int). The guidelines on BSE are contained in

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

Code.

snip\...end

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

Greetings again APHIS ET AL,

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

Muscle tissue has recently been detected with PrPSc

in the peripheral nerves (sciatic nerve, tibial nerve, vagus nerve) of
the 11th BSE

cow in Japan (Yoshifumi Iwamaru et al). also recently, Aguzzi et al
Letter to the Editor

Vet Pathol 42:107-108 (2005), Prusiner et al CDI test is another example
of detection

of the TSE agent in muscle in sCJD, Herbert Budka et al CJD and
inclusion body myositis:

Abundant Disease-Associated Prion Protein in Muscle, and older studies
from Watson

Meldrum et al Scrapie agent in muscle - Pattison I A (1990), references
as follow ;

PrPSc distribution of a natural case of bovine

spongiform encephalopathy

Yoshifumi Iwamaru, Yuka Okubo, Tamako Ikeda, Hiroko Hayashi, Mori-

kazu Imamura, Takashi Yokoyama and Morikazu Shinagawa

Priori Disease Research Center, National Institute of Animal Health,
3-1-5

Kannondai, Tsukuba 305-0856 Japan gan@affrc.go.jp

Abstract

Bovine spongiform encephalopathy (BSE) is a disease of cattle that
causes

progressive neurodegeneration of the central nervous system. Infectivity

of BSE agent is accompanied with an abnormal isoform of prion protein

(PrPSc).

The specified risk materials (SRM) are tissues potentially carrying BSE

infectivity. The following tissues are designated as SRM in Japan: the

skull including the brain and eyes but excluding the glossa and the
masse-

ter muscle, the vertebral column excluding the vertebrae of the tail,
spinal

cord, distal illeum. For a risk management step, the use of SRM in both

animal feed or human food has been prohibited. However, detailed

PrPSc distribution remains obscure in BSE cattle and it has caused con-

troversies about definitions of SRM. Therefore we have examined PrPSc

distribution in a BSE cattle by Western blotting to reassess definitions
of

SRM.

The 11th BSE case in Japan was detected in fallen stock surveillance.

The carcass was stocked in the refrigerator. For the detection of PrPSc,

200 mg of tissue samples were homogenized. Following collagenase

treatment, samples were digested with proteinase K. After digestion,

PrPSc was precipitated by sodium phosphotungstate (PTA). The pellets

were subjected to Western blotting using the standard procedure.

Anti-prion protein monoclonal antibody (mAb) T2 conjugated horseradish

peroxidase was used for the detection of PrPSc.

PrPSc was detected in brain, spinal cord, dorsal root ganglia,
trigeminal

ganglia, sublingual ganglion, retina. In addition, PrPSc was also
detected

in the peripheral nerves (sciatic nerve, tibial nerve, vagus nerve).

Our results suggest that the currently accepted definitions of SRM in

BSE cattle may need to be reexamined. \...

179

T. Kitamoto (Ed.)

PRIONS

Food and Drug Safety

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

ALSO from the International Symposium of Prion Diseases held in Sendai,
October 31, to

November 2, 2004;

Bovine spongiform encephalopathy (BSE) in Japan

snip\...

\"Furthermore, current studies into transmission of cases of BSE that
are

atypical or that develop in young cattle are expected to amplify the BSE

prion\"

NO. Date conf. Farm Birth place and Date Age at diagnosis

8\. 2003.10.6. Fukushima Tochigi 2001.10.13. 23

9\. 2003.11.4. Hiroshima Hyogo 2002.1.13. 21

Test results

\# 8b, 9c cows Elisa Positive, WB Positive, IHC negative, histopathology

negative

b = atypical BSE case

c = case of BSE in a young animal

b,c, No PrPSc on IHC, and no spongiform change on histology

International Symposium of Prion Diseases held in Sendai, October 31, to

November 2, 2004.

The hardback book title is \'PRIONS\' Food and Drug Safety

T. Kitamoto (Ed.)

Tetsuyuki Kitamoto

Professor and Chairman

Department of Prion Research

Tohoku University School of Medicine

2-1 SeiryoAoba-ku, Sendai 980-8575, JAPAN

TEL +81-22-717-8147 FAX +81-22-717-8148

e-mail; kitamoto@mail.tains.tohoku.ac.jp

Symposium Secretariat

Kyomi Sasaki

TEL +81-22-717-8233 FAX +81-22-717-7656

e-mail: kvomi-sasaki@mail.tains.tohoku.ac.ip

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

107

Vet Pathol 42:107--108 (2005)

Letters to the Editor

Editor:

Absence of evidence is not always evidence of absence.

In the article ''Failure to detect prion protein (PrPres) by

immunohistochemistry in striated muscle tissues of animals

experimentally inoculated with agents of transmissible spongiform

encephalopathy,'' recently published in Veterinary

Pathology (41:78--81, 2004), PrPres was not detected in striated

muscle of experimentally infected elk, cattle, sheep, and

raccoons by immunohistochemistry (IHC). Negative IHC,

however, does not exclude the presence of PrPSc. For example,

PrPres was detected in skeletal muscle in 8 of 32

humans with the prion disease, sporadic Creutzfeldt-Jakob

disease (CJD), using sodium phosphotungstic acid (NaPTA)

precipitation and western blot.1 The NaPTA precipitation,

described by Wadsworth et al.,3 concentrates the abnormal

isoform of the prion, PrPres, from a large tissue homogenate

volume before western blotting. This technique has increased

the sensitivity of the western blot up to three orders

of magnitude and could be included in assays to detect

PrPres. Extremely conspicuous deposits of PrPres in muscle

were detected by IHC in a recent case report of an individual

with inclusion body myositis and CJD.2 Here, PrPres was

detected in the muscle by immunoblotting, IHC, and paraf-

fin-embedded tissue blot. We would therefore caution that,

in addition to IHC, highly sensitive biochemical assays and

bioassays of muscle are needed to assess the presence or

absence of prions from muscle in experimental and natural

TSE cases.

Christina Sigurdson, Markus Glatzel, and Adriano Aguzzi

Institute of Neuropathology

University Hospital of Zurich

Zurich, Switzerland

References

1 Glatzel M, Abela E, et al: Extraneural pathologic prion

protein in sporadic Creutzfeldt-Jakob disease. N Engl J

Med 349(19):1812--1820, 2003

2 Kovacs GG, Lindeck-Pozza E, et al: Creutzfeldt-Jakob

disease and inclusion body myositis: abundant diseaseassociated

prion protein in muscle. Ann Neurol 55(1):

121--125, 2004

3 Wadsworth JDF, Joiner S, et al: Tissue distribution of protease

resistant prion protein in variant CJD using a highly

sensitive immuno-blotting assay. Lancet 358:171--180,

2001

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

Corinna Kaarlela, News Director

Source: Jennifer O\'Brien

jobrien@pubaff.ucsf.edu

415-476-2557

14 February 2005

Diagnosis of prions in patients should utilize novel strategy, team says

A technique for detecting prions in tissue, developed in recent years by

UCSF scientists, is significantly more sensitive than the diagnostic

procedures currently used to detect the lethal particles in samples of

brain tissue from patients, according to a study performed by a UCSF
team.

The finding indicates that the diagnostic technique, known as the

conformation-dependent immunoassay (CDI), should be established as the

standard approach for brain biopsies of patients suspected of having the

disease, they say. The team is exploring whether the CDI might be

adapted to detect prions in blood and muscle.

The finding suggests that reliance on the current methods for detecting

prions in human brain tissue \-- microscopic examination of tissue for

the telltale vacuoles that form in brain cells and immunohistochemistry

(IHC), which involves detecting prions in brain sections using prion

protein-specific antibodies \-- may have led to an under diagnosis of
the

disease in patients in recent years, they say. (A definitive diagnosis

of the disease in humans is made only on autopsy, when a

neuropathologist can analyze multiple brain regions for vacuoles and

evidence of prions by IHC, and it is estimated that only 50 percent of

human cases are autopsied, in part because many pathologists do not want

to risk infection during the autopsy.)

In the study, the team compared the ability of the CDI and the two

traditional diagnostic techniques to detect prions in various brain

samples from 28 patients diagnosed on autopsy as having one of several

human forms of the disease \-- sporadic, familial or iatrogenic

Creutzfeldt-Jakob disease (CJD). While the CDI detected the biochemical

signal for prions in 100 percent of the samples studied, the traditional

tests failed to detect the prion in a high proportion of cases. For

example, in an experiment that focused on 18 brain regions from eight

patients with sporadic CJD, the CDI detected prions in 100 percent of

the samples, while IHC detected them in 22 percent and routine tissue

examination in 17 percent.

\"In about 80 percent of the different brain regions examined, prions

were not consistently detected by either IHC or routine histology that

measure vacuolation. In contrast, the CDI was always positive in all

regions of the brain,\" says the lead author of the study, Jiri Safar,

MD, associate adjunct professor of neurology and a member of the UCSF

Institute for Neurodegenerative Diseases, which is directed by senior

author Stanley B. Prusiner, MD, UCSF professor of neurology and

biochemistry.

\"These findings indicate that histology and immunohistochemistry should

no longer be used to rule out prion disease in single-site biopsy

samples,\" says Safar. \"The superior performance of the CDI in
diagnosing

prion disease suggests that the CDI be used in future diagnostic

evaluations of prion disease, particularly for single-site brain

biopsies during life\"

\"If the traditional techniques are used at autopsy, they must be
applied

to many cortical and subcortical samples,\" says co-author Stephen J.

DeArmond, MD, PhD, UCSF professor of neuropathology.

Moreover, while the study examined the efficacy of the CDI in comparison

to the two techniques routinely used by neuropathologists to detect

prions in human brain tissue, previous studies at UCSF indicate that the

CDI is also significantly more sensitive than Western blot analysis, the

technology used with IHC to detect prions in brain tissue from cattle

suspected of having bovine spongiform encephalopathy (BSE). That IHC and

Western blot analysis are relatively insensitive methods, the

researchers say, supports their ongoing assertion that the CDI should

also be used to evaluate the brain tissue of cattle.

\"The studies reported here are likely to change profoundly the approach

to the diagnosis of prion disease in both humans and livestock,\" says
Safar.

More broadly, the scientists say, the high sensitivity of the CDI

suggests that CDI-like tests could also prove useful for diagnosing

other neurodegenerative diseases, such as Alzheimer\'s disease,

Parkinsons\'s disease and fronto-temporal dementias, all of which, like

prion diseases, involve various forms of protein misprocessing. These

diseases currently are diagnosed by neuropathological analysis and

immunohistochemistry.

\"Whether immunohistochemistry underestimates the incidence of one or

more of these common neurodegenerative diseases is unknown, but the CDI

could shed light on these diseases,\" says co-author Bruce Miller, MD,

UCSF A.W. and Mary Margaret Clausen Distinguished Professor of Neurology

and director of the UCSF Memory and Aging Center.

The finding will be printed on-line and in print on March 1, 2005 in

Proceedings of the National Academy of Sciences.

The study brings into high relief the different detection strategies of

immunohistochemistry and the CDI, both of which involve revealing the

presence of prions, known as PrPsc, by applying antibodies to brain
tissue.

Standard immunohistochemistry, developed in the DeArmond lab 20 years

ago, involves using an enzyme known as a protease, or a combination of

harsh acid and high temperature treatment, to destroy normal prion

protein (PrPC), which is ubiquitous in brain tissue. Once this occurs,

scientists apply fluorescently lit antibodies that react with residues

of the relatively resistant abnormal prion protein (PrPSc), thereby

highlighting it.

The limitation of this technique is that scientists have since learned

that there is a large part of the abnormal prion protein that is

protease sensitive, and that portion escapes detection by the standard

technique. Thus, this traditional method underestimates the level of

PrPSc in tissue.

The CDI addresses this limitation by revealing the region of PrPSc that

is exposed in the normal PrPC but is buried in infectious PrPSc, using

high affinity, newly generated antibodies that identify PrPSc through

the distinct shape of the molecule, independent of proteolytic

treatments. This makes it possible to detect potentially large

concentrations of protease sensitive PrPSc molecules.

Detractors would say that it is not necessary to detect the minute level

of infectious agent that the CDI is capable of revealing, as it would be

unlikely to be lethal, says Safar. But Prusiner and his colleagues

maintain that any risk is too great when it comes to having prions in

the food supply. In addition, because even low levels of prions are

extremely resistant to inactivation, they may contaminate the

environment for many years.

Prusiner won the 1997 Nobel Prize in Physiology or Medicine for

discovering that a class of neurodegenerative diseases known as

spongiform encephalopathies was caused by prions. Prion diseases develop

in humans, cattle, sheep, deer, elk and mink.

The CDI was developed by members of the Prusiner lab. The CDI

methodology has been licensed to InPro Biotechnology, Inc.

Prusiner, Safar, DeArmond and other members of the Institute for

Neurodegenerative Diseases are scientific advisors to, or own stock in,

InPro.

Other co-authors of the study were Michael D. Geschwind, Camille

Deering, Svetlana Didorenko, Mamta Sattavat, Henry Sanchesz, Ana Serban,

Kurt Giles, of UCSF, and Martin Vey, of Behring, Marburg, Germany, and

Henry Baron, of Behring, Paris.

The study was funded by the National Institutes of Health, the John

Douglas French Foundation for Alzheimer\'s research, the McBean

Foundation, the State of California, Alzheimer\'s Disease Research
Center

of California and the RR00079 General Clinical Research Center.

The UCSF Institute for Neurodegenerative Diseases:

http://ind.medschool.ucsf.edu/.

FURTHER COMPARISON OF THE CDI TO THE STANDARD DIAGNOSTIC PROCEDURES,

PROVIDED BY STEPHEN J. DEARMOND, MD, PHD, UCSF PROFESSORS OF
NEUROPATHOLOGY:

Explanation as to why the CDI is more sensitive than Western blot

analysis: Studies at UCSF during development of the CDI showed that CDI

could detect prions in brain homogenates at levels that fail to produce

disease in animals (bioassay for prions). Therefore, the CDI is more

sensitive than the bioassay method, which was considered to be the most

sensitive technique for detecting prions. In contrast, Western blot

analysis for prions is significantly less sensitive than the bioassay

and is, therefore, significantly less sensitive than the CDI. Currently,

the USDA uses a combination of Western blot analysis of brainstem

homogenates and immunohistochemistry of the medulla to test cattle

suspected of having bovine spongiform encephalopathy (\"mad cow

disease\"). The relative insensitivity of IHC and Western blot analysis,

says DeArmond, supports the UCSF scientists\' ongoing assertion that the

CDI should also be used to evaluate the brain tissue of cattle.

DeArmond cites additional evidence about Western blot analysis from a

World Health Organization (WHO) study group, which compared the CDI

method with Western blots for detection of prions in sporadic and

variant CJD brains. Based on the smallest amount of prions that could

detected by the two techniques, they found that the CDI was from 1000-

to 100,000-fold more sensitive than Western blot analysis performed in

six different research laboratories (Minor et al. Standards for the

assay of Creutzfeldt-Jakob disease specimens. J. Gen. Virol. 85:

1777-1784, 2004).

Explanation as to why IHC for prions is less sensitive than the CDI: IHC

is routinely performed on formalin-fixed, paraffin-embedded samples of

brain. Formalin fixation markedly decreases the ability of antibodies to

bind to proteins in general, which greatly weakens the IHC signal for

prions (PrPSc). In contrast, homogenates for the CDI are not treated

with reagents that decrease prion antigenicity. Moreover, to concentrate

the PrPSc for measurement by the CDI, the homogenates are exposed to

phosphotungstic acid, which selectively precipitates both

protease-sensitive and protease-resistant PrPSc that comprise prions,

but not the normal prion protein conformer found in uninfected animals,

PrPC. This step results in a higher concentration of PrPSc for detection

by the CDI. Because the PrPSc was not exposed to proteases, the CDI

measures all forms of abnormally folded PrPSc molecules.

Protease-sensitive PrPSc can account for 50 percent of the total PrPSc.

For Western analysis, homogenates of brain are treated with protease to

eliminate PrPC; however, this step also eliminates protease-sensitive

PrPSc leaving only protease-resistant PrPSc for Western blot detection

and decreasing the PrPSc signal at least in half.

\###

http://pub.ucsf.edu/newsservices/releases/200502147/

PNAS \| March 1, 2005 \| vol. 102 \| no. 9 \| 3501-3506

NEUROSCIENCE

Diagnosis of human prion disease

Jiri G. Safar \*, , Michael D. Geschwind , , Camille Deering

\*, Svetlana Didorenko \*, Mamta Sattavat ¶, Henry Sanchez ¶,

Ana Serban \* , Martin Vey \|\|, Henry Baron \*\*, Kurt Giles \*,

, Bruce L. Miller , , Stephen J. DeArmond \* , ¶ and Stanley

B. Prusiner \*, , ,

\*Institute for Neurodegenerative Diseases, Memory and Aging

Center, and Departments of Neurology, ¶Pathology, and

Biochemistry and Biophysics, University of California, San

Francisco, CA 94143; \|\|ZLB Behring, 35041 Marburg, Germany;

and \*\*ZLB Behring, 75601 Paris, France

Contributed by Stanley B. Prusiner, December 22, 2004

Abstract

With the discovery of the prion protein (PrP),

immunodiagnostic procedures were applied to diagnose

Creutzfeldt--Jakob disease (CJD). Before development of the

conformation-dependent immunoassay (CDI), all immunoassays

for the disease-causing PrP isoform (PrPSc) used limited

proteolysis to digest the precursor cellular PrP (PrPC).

Because the CDI is the only immunoassay that measures both

the protease-resistant and protease-sensitive forms of

PrPSc, we used the CDI to diagnose human prion disease. The

CDI gave a positive signal for PrPSc in all 10--24 brain

regions (100%) examined from 28 CJD patients. A subset of 18

brain regions from 8 patients with sporadic CJD (sCJD) was

examined by histology, immunohistochemistry (IHC), and the

CDI\. Three of the 18 regions (17%) were consistently

positive by histology and 4 of 18 (22%) by IHC for the 8

sCJD patients. In contrast, the CDI was positive in all 18

regions (100%) for all 8 sCJD patients. In both gray and

white matter, 90% of the total PrPSc was protease-sensitive

and, thus, would have been degraded by procedures using

proteases to eliminate PrPC. Our findings argue that the CDI

should be used to establish or rule out the diagnosis of

prion disease when a small number of samples is available as

is the case with brain biopsy. Moreover, IHC should not be

used as the standard against which all other

immunodiagnostic techniques are compared because an

immunoassay, such as the CDI, is substantially more

sensitive.

snip\...

Discussion

The clinical diagnosis of human prion disease is often

difficult until the patient shows profound signs of

neurologic dysfunction. It is widely accepted that the

clinical diagnosis must be provisional until a tissue

diagnosis either confirms or rules out the clinical

assessment. Before the availability of Abs to PrP, a tissue

diagnosis was generally made by histologic evaluation of

neuropil vacuolation. IHC with

anti-glial-fibrillary-acidic-protein Abs in combination with

H&E staining preceded the use of anti-PrP Ab staining.

Recently, the role of IHC in the diagnosis of scrapie in the

brains of eight clinically affected goats inoculated with

the SSBP1 prion isolate has been challenged (14). Thalamic

samples taken from seven of eight goats with scrapie were

positive for PrPSc by Western blotting but negative by IHC.

The eighth goat was negative by Western blotting and IHC.

Consistent with these findings in goats are the data

reported here, in which IHC of formalin-fixed,

paraffin-embedded human brain samples was substantially less

sensitive than the CDI.

The CDI was developed to quantify PrPSc in tissue samples

from mammals producing prions. Concerned that limited PK

digestion was hydrolyzing some or even most of the PrPSc, we

developed a CDI that does not require PK digestion. The CDI

revealed that as much as 90% of PrPSc is sPrPSc; thus, it

was being destroyed during limited proteolytic digestion

used to hydrolyze PrPC. sPrPSc comprises 80% of PrPSc in the

frontal lobe and in the white matter (Fig. 4).

The CDI detected HuPrPSc with a sensitivity comparable to

the bioassay for prion infectivity in Tg(MHu2M) mice (Fig.

1). The high sensitivity achieved by the CDI is due to

several factors (8, 10, 11, 15). First, both sPrPSc and

rPrPSc conformers are specifically precipitated by PTA

(Table 5) (8, 9). PTA has also been used to increase the

sensitivity of Western blots enabling the detection of

rPrPSc in human muscle and other peripheral tissues (16,

17). Second, a sandwich protocol was used with the

high-affinity MAR1 mAb (11) to capture HuPrPSc and

Eu-labeled 3F4 mAb to detect HuPrPSc (12). Third, the CDI

detects PrPSc by Ab-binding to native and denatured forms of

the protein and, therefore, does not depend on proteolytic

degradation of PrPC. We chose not to perform Western blots

on most of the samples used in this study because such

immunoblots require denaturation of the sample, which

eliminates measurement of the native signal corresponding to

PrPC (Table 5). Moreover, a comparison between the CDI and

Western blotting on brain samples from sCJD and variant CJD

patients showed that the CDI was 50- to 100-fold more

sensitive (15). Additionally, Western blots combined with

densitometry are linear over a 10- to 100-fold range of

concentrations, whereas the CDI is linear over a \>104-fold

range. The CDI has been automated, which not only improves

accuracy and reproducibility (10) but also allows numerous

samples to be analyzed, as reported here. Western blots are

difficult to automate and are labor intensive.

Our studies show that only the CDI detected PrPSc in all

regions examined in 24 sCJD and 3 fCJD(E200K) brains (Figs.

2 and 6). Comparative analyses demonstrated that the CDI was

vastly superior to histology and IHC. When 18 regions of 8

sCJD and 2 fCJD(E200K) brains were compared, we discovered

that histology and IHC were unreliable diagnostic tools

except for samples from a few brain regions. In contrast,

the CDI was a superb diagnostic procedure because it

detected PrPSc in all 18 regions in 8 of 8 sCJD and 2 of 2

fCJD(E200K) cases (Tables 1 and 2).

Histologic changes in prion disease have been shown to

follow the accumulation of prions as measured by bioassay of

infectivity and by PrPSc accumulation (18--22). Because low

levels of PrPSc are not associated with neuropathologic

changes, some discrepancy between vacuolation and PrPSc was

expected. In contrast to histology, IHC measures PrP

immunostaining after autoclaving tissue sections exposed to

formic acid. Because IHC measures PrP, we expected the

sensitivity of this procedure might be similar to the CDI,

but that proved not to be the case. Whether exposure of

formic acid-treated tissue sections to elevated temperature

destroys not only PrPC but also sPrPSc and only denatures

rPrPSc remains to be determined. Such a scenario could

account for the lower sensitivity of IHC compared with CDI

or bioassay (Tables 1 and 2).

Studies of the white matter in CJD brains were particularly

informative with respect to the sensitivity of the CDI,

where PrPSc levels were low but readily detectable, 10- to

100-fold above the threshold value (Fig. 4). Because animal

studies have shown that PrPSc and infectivity are

transported anterogradely from one brain region to another

along neuroanatomical pathways (23--25), we expected to find

PrPSc in white matter as demonstrated by the CDI but not

IHC. Axonal transport of PrPSc is also suggested by

diffusion-weighted MRI scans of CJD cases, which show

high-intensity signals in analogous neocortical regions of

the right and left cerebral hemispheres (26). This symmetry

of neuroradiological abnormalities is consistent with spread

of PrPSc to the contralateral cortex by means of callosal

commissural pathways.

Most immunoassays that detect HuPrPSc do so only after

subjecting the sample to limited proteolysis to form PrP

27--30, followed by denaturation. Because the CDI measures

the immunoreactivity before and after denaturation to an

epitope that is exposed in native PrPC but buried in PrPSc,

limited proteolysis to eliminate PrPC is unnecessary. Assays

based on limited proteolysis underestimate the level of

PrPSc because they digest sPrPSc, which represents 80--90% of

PrPSc in CJD and scrapie brains (Fig. 4 and Table 5).

Gerstmann--Sträussler--Scheinker, an inherited human prion

disease, is caused by the P102L mutation in the PRNP gene.

In mice expressing the Gerstmann--Sträussler--Scheinker mutant

PrP transgene, the CDI detected high levels of sPrPSc(P101L)

as well as low levels of rPrPSc(P101L) long before

neurodegeneration and clinical symptoms occurred (9).

sPrPSc(P101L) as well as low concentrations of rPrPSc(P101L)

previously escaped detection (27). Whether a similar

situation applies in other genetic forms of prion disease,

sCJD, or variant CJD remains to be determined. Because most

of the PrPSc in the brains of sCJD patients is

protease-sensitive (Fig. 4), it is likely that the lower

sensitivity of IHC is due to its inability to detect sPrPSc.

Presently, we have no information about the kinetics of

either sPrPSc or rPrPSc accumulation in human brain. Limited

information on the kinetics of PrPSc accumulation in

livestock comes from studies of cattle, sheep, and goats

inoculated orally, but most of the bioassays were performed

in non-Tg mice (28--30) in which prion titers were

underestimated by as much as a factor of 104 (10).

The studies reported here are likely to change profoundly

the approach to the diagnosis of prion disease in both

humans and livestock (31--33). The superior performance of

the CDI in diagnosing prion disease compared to routine

neuropathologic examination and IHC demands that the CDI be

used in future diagnostic evaluations of prion disease.

Prion disease can no longer be ruled out by routine

histology or IHC. Moreover, the use of IHC to confirm cases

of bovine spongiform encephalopathy after detection of

bovine PrPSc by the CDI (10) seems an untenable approach in

the future. Clearly, the CDI for HuPrPSc is as sensitive or

more sensitive than bioassays in Tg(MHu2M) mice (Fig. 1).

Our results suggest that using the CDI to test large numbers

of samples for human prions might alter the epidemiology of

prion diseases. At present, there is limited data on the

frequency of subclinical variant CJD infections in the U.K.

population (34). Because appendixes and tonsils were

evaluated only by IHC, many cases might have escaped

detection (Tables 1 and 2). Equally important may be the use

of CDI-like tests to diagnose other neurodegenerative

disorders, such as Alzheimer\'s disease, Parkinson\'s disease,

and the frontotemporal dementias. Whether IHC underestimates

the incidence of one or more of these common degenerative

diseases is unknown. Moreover, CDI-like tests may help

determine the frequency with which these disorders and the

prion diseases occurs concomitantly in a single patient (35,

36).

Acknowledgements

snip\...END

http://www.pnas.org/

Volume 349:1812-1820 November 6, 2003 Number 19

Extraneural Pathologic Prion Protein in Sporadic Creutzfeldt-Jakob
Disease

Background In patients with sporadic Creutzfeldt--Jakob disease,
pathologic disease-associated prion protein (PrPSc) has been identified
only in the central nervous system and olfactory-nerve tissue.
Understanding the distribution of PrPSc in Creutzfeldt--Jakob disease is
important for classification and diagnosis and perhaps even for
prevention.

Methods We used a highly sensitive method of detection --- involving the
concentration of PrPSc by differential precipitation with sodium
phosphotungstic acid, which increased the sensitivity of Western blot
analysis by up to three orders of magnitude --- to search for PrPSc in
extraneural organs of 36 patients with sporadic Creutzfeldt--Jakob
disease who died between 1996 and 2002.

Results PrPSc was present in the brain tissue of all patients. In
addition, we found PrPSc in 10 of 28 spleen specimens and in 8 of 32
skeletal-muscle samples. Three patients had PrPSc in both spleen and
muscle specimens. Patients with extraneural PrPSc had a significantly
longer duration of disease and were more likely to have uncommon
molecular variants of sporadic Creutzfeldt--Jakob disease than were
patients without extraneural PrPSc.

Conclusions Using sensitive techniques, we identified extraneural
deposition of PrPSc in spleen and muscle samples from approximately one
third of patients who died with sporadic Creutzfeldt--Jakob disease.
Extraneural PrPSc appears to correlate with a long duration of disease.

Source Information

From the Institute of Neuropathology and National Reference Center for
Prion Diseases, University Hospital of Zurich, Zurich, Switzerland.

Dr. Glatzel and Mr. Abela contributed equally to the article.

Address reprint requests to Dr. Aguzzi at the Institute of
Neuropathology, University Hospital of Zurich, Schmelzbergstr. 12,
CH-8091 Zurich, Switzerland, or at adriano@pathol.unizh.ch .

http://content.nejm.org/cgi/

Creutzfeldt-Jakob disease and inclusion body myositis: Abundant
disease-associated prion protein in muscle

Gabor G. Kovacs, MD PhD 1 2, Elisabeth Lindeck-Pozza, MD 1, Leila
Chimelli, MD, PhD 3, Abelardo Q. C. Araújo, MD, PhD 4, Alberto A.
Gabbai, MD, PhD 5, Thomas Ströbel, PhD 1, Markus Glatzel, MD 6, Adriano
Aguzzi, MD, PhD 6, Herbert Budka, MD 1 \*

1Institute of Neurology, University of Vienna, and Austrian Reference
Centre for Human Prion Diseases, Vienna, Austria

2National Institute of Psychiatry and Neurology, Budapest, Hungary

3Department of Pathology, School of Medicine, Federal University of Rio
de Janeiro

4Department of Neurology, School of Medicine, Federal University of Rio
de Janeiro

5Department of Neurology, School of Medicine, Federal University of Sao
Paulo, Brazil

6Institute of Neuropathology, University Hospital of Zürich, Zürich,
Switzerland

email: Herbert Budka (h.budka@akh-wien.ac.at )

\*Correspondence to Herbert Budka, Institute of Neurology, AKH 4J,
Wühringer Gürtel 18-20, POB 48, A-1097 Vienna, Austria

Funded by:

European Union (EU) Project; Grant Number: TSELAB QLK2-CT-2002-81523

EU Concerted Action PRIONET; Grant Number: QLK2-2000-CT-00837

Abstract

Pathologicalprion protein (PrPSc) is the hallmark of prion diseases
affecting primarily the central nervous system. Using
immunohistochemistry, paraffin-embedded tissue blot, and Western blot,
we demonstrated abundant PrPSc in the muscle of a patient with sporadic
Creutzfeldt-Jakob disease and inclusion body myositis. Extraneural
PrPC-PrPSc conversion in Creutzfeldt-Jakob disease appears to become
prominent when PrPC is abundantly available as substrate, as in
inclusion body myositis muscle.

\-\-\-\-\-\-\-\-\-\-\-\-\--

Received: 16 June 2003; Revised: 11 September 2003; Accepted: 11
September 2003

Digital Object Identifier (DOI)

10.1002/ana.10813 About DOI

http://www3.interscience.wiley.com/

AS Professor Aguzzi kindly put it most recently ;

107

Vet Pathol 42:107 108 (2005)

Letters to the Editor

Editor:

Absence of evidence is not always evidence of absence. In the article
Failure to detect prion protein (PrPres) by immunohistochemistry in
striated muscle tissues of animals experimentally inoculated with agents
of transmissible spongiform encephalopathy, recently published in
Veterinary Pathology (41:78 81, 2004), PrPres was not detected in
striated muscle of experimentally infected elk, cattle, sheep, and
raccoons by immunohistochemistry (IHC). Negative IHC, however, does not
exclude the presence of PrPSc. For example, PrPres was detected in
skeletal muscle in 8 of 32 humans with the prion disease, sporadic
Creutzfeldt-Jakob disease (CJD), using sodium phosphotungstic acid
(NaPTA) precipitation and western blot.1 The NaPTA precipitation,
described by Wadsworth et al.,3 concentrates the abnormal isoform of the
prion, PrPres, from a large tissue homogenate volume before western
blotting. This technique has increased the sensitivity of the western
blot up to three orders of magnitude and could be included in assays to
detect PrPres. Extremely conspicuous deposits of PrPres in muscle were
detected by IHC in a recent case report of an individual with inclusion
body myositis and CJD.2 Here, PrPres was detected in the muscle by
immunoblotting, IHC, and paraf- fin-embedded tissue blot. We would
therefore caution that, in addition to IHC, highly sensitive biochemical
assays and bioassays of muscle are needed to assess the presence or
absence of prions from muscle in experimental and natural TSE cases.

Christina Sigurdson, Markus Glatzel, and Adriano Aguzzi

Institute of Neuropathology

University Hospital of Zurich

Zurich, Switzerland

References

1 Glatzel M, Abela E, et al: Extraneural pathologic prion

protein in sporadic Creutzfeldt-Jakob disease. N Engl J

Med 349(19):1812 1820, 2003

2 Kovacs GG, Lindeck-Pozza E, et al: Creutzfeldt-Jakob

disease and inclusion body myositis: abundant diseaseassociated

prion protein in muscle. Ann Neurol 55(1):

121 125, 2004

3 Wadsworth JDF, Joiner S, et al: Tissue distribution of protease

resistant prion protein in variant CJD using a highly

sensitive immuno-blotting assay. Lancet 358:171 180,

2001\...///

EMBO reports AOP Published online: 11 April 2003

Widespread PrPSc accumulation in muscles of hamsters orally infected
with scrapie

http://www.emboreports.org/

Watson Meldrum et al Scrapie agent in muscle - Pattison I A (1990)

Veterinary record, 20 January 1990. p.68

http://www.bseinquiry.gov.uk/files/yb/1990/01/19009001.pdf

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

GREETINGS AGAIN APHIS ET AL,

FURTHERMORE, WE HAVE FAILED TO EVEN STOP THE SRMs FROM WHOLE

CUTS OF BONELESS BEEF IMPORTED FROM CANADA IN THE VERY ONSET OF

THE NEW BSE MRR (MINIMAL RISK REGION). THIS IS THE VERY REASON I HAVE

SAID TIME AND TIME AGAIN THAT BY THIS ADMINISTRATION ABANDONING THE

BSE GBR RISK ASSESSMENTS BECAUSE THEY DID NOT LIKE THE ASSESSMENT

OF BSE GBR III, AND ADHERING TO A NEW BSE MRR POLICY THAT WAS DESIGNED

NOT FOR HUMAN HEALTH, BUT ONLY FOR COMMODITIES AND FUTURES, WILL FURTHER

EXPOSE NEEDLESSLY MILLIONS AND MILLIONS OF HUMANS AND ANIMALS VIA THE
FREE

TRADING OF ALL STRAINS OF TSE GLOBALLY. references as follow ;

Wisconsin Firm Recalls Beef Products

Recall Release CLASS II RECALL

FSIS-RC-032-2005 HEALTH RISK: LOW

Congressional and Public Affairs

\(202\) 720-9113

Steven Cohen

WASHINGTON, Aug. 19, 2005 - Green Bay Dressed Beef, a Green Bay, Wis.,
establishment, is voluntarily recalling approximately 1,856 pounds of
beef products that may contain portions of the backbone from a cow just
over 30 months old, the U.S. Department of Agriculture\'s Food Safety
and Inspection Service announced today. The product was from a cow
imported directly for slaughter from Canada.

Based on information provided by Canada, the products subject to this
Class II recall are from a cow that is approximately one month older
than the 30-month age limit. Both ante-mortem and post-mortem inspection
were done on the cow in question. FSIS inspection program personnel
determined the cow to be healthy and fit for human food. FSIS\'
designation of this recall as Class II is because it is a situation
where there is a remote probability of adverse health consequences from
the use of the product.

FSIS learned about this as a result of a Canadian audit of their health
certificate that accompanied the imported cow. Prior to slaughter, the
health certificate accompanying the cow was presented to the
establishment, and it appeared complete and accurate. However, a
subsequent audit of information related to the health certificate by
Canadian officials found that it was not accurate. Action has been taken
by Canadian Food Inspection Agency officials in response to findings
from the audit.

The products subject to recall are:

Five boxes of 243 lb. vacuum pouched packages of \"American Foods Group,
NECKBONE UNTRIM\'D, USDA CHOICE OR HIGHER\" with the case code of 77333;

One box of 50 lb. vacuum pouched package of \"American Foods Group,
SHORTLOIN 2X2, USDA SELECT OR HIGHER\" with the case code of 75231;

One box of 60 lb. vacuum pouched package of \"American Foods Group,
SHORTLOIN 2X2, USDA CHOICE OR HIGHER\" with the case code of 75060;

Five boxes of 258 lb. vacuum pouched packages of \"Dakota Supreme Beef,
SHORTLOIN 0X11/4, USDA SELECT OR HIGHER\" with the case code of 75442;

Sixteen boxes of 811 lb. vacuum pouched packages of \"American Foods
Group, BLADE BI N/O CHUCK, USDA CHOICE OR HIGHER\" with the case code of
75955;

Nine boxes of 435 lb. vacuum pouched packages of \"American Foods Group,
BLADE BI N/O CHUCK, USDA SELECT OR HIGHER\" with the case code of 75952.

Each box bears the establishment number \"410\" inside the USDA seal of
inspection. The products were produced on August 4, and were distributed
to wholesale distributors in Pennsylvania, Florida, Illinois, Maryland,
Minnesota and Wisconsin.

Under the interim final rules FSIS implemented on January 12, 2004,
certain specified risk materials must be removed from all cattle
depending on the age of the animal. On this animal all specified risk
materials for cattle 30 months and over were removed, with the exception
of the vertebral column. At the time of slaughter, the animal was
certified to be under 30 months of age and removal of the vertebral
column was not required. A subsequent audit determined the animal was
just over 30 months of age; therefore, the vertebral column is required
to be removed. This is the reason for the recall of the selected
products.

Consumers with questions about the recall may contact Sally VandeHei,
Executive Assistant at 1-877-894-3927. National media with questions may
contact Jim Mulhern at (202) 496-2468. Local media with questions may
contact Susan Finco at (920) 965-7750 ext.158.

Consumers with other food safety questions can phone the toll-free USDA
Meat and Poultry Hotline at 1-888-MPHotline (1-888-674-6854). The
hotline is available in English and Spanish and can be reached from 10
a.m. to 4 p.m. (Eastern Time), Monday through Friday. Recorded food
safety messages are available 24 hours a day.

Sample Product Labels: These are similar to, but not identical to,
labels on the recalled product.

\#

USDA Recall Classifications

Class I This is a health hazard situation where there is a reasonable
probability that the use of the product will cause serious, adverse
health consequences or death.

Class II This is a health hazard situation where there is a remote
probability of adverse health consequences from the use of the product.

Class III This is a situation where the use of the product will not
cause adverse health consequences.

http://www.fsis.usda.gov/News\_&\_Events/Recall_032_2005_Release/index.asp

:

Docket No. 03-080-1 \-- USDA ISSUES PROPOSED RULE TO ALLOW LIVE ANIMAL
IMPORTS FROM CANADA

\[TSS SUBMISSION 11/03/2003 01:19 PM To: regulations@aphis.usda.gov \]

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

THE BSE MRR POLICY SHOULD BE ABOLISHED/REPEALED IMMEDIATELY AND THE BSE
GBR RISK ASSESSMENTS AND POLICY SHOULD BE STRICTLY ENFORCED AND FURTHER
ENHANCED TO INCLUDE CWD AND ALL TSEs\...TSS

EFSA Scientific Report on the Assessment of the Geographical BSE-Risk
(GBR) of the United States of America (USA)

Publication date: 20 August 2004

Adopted July 2004 (Question N° EFSA-Q-2003-083)

Report

Summary

Summary of the Scientific Report

The European Food Safety Authority and its Scientific Expert Working
Group on the Assessment of the Geographical Bovine Spongiform
Encephalopathy (BSE) Risk (GBR) were asked by the European Commission
(EC) to provide an up-to-date scientific report on the GBR in the United
States of America, i.e. the likelihood of the presence of one or more
cattle being infected with BSE, pre-clinically as well as clinically, in
USA. This scientific report addresses the GBR of USA as assessed in 2004
based on data covering the period 1980-2003.

The BSE agent was probably imported into USA and could have reached
domestic cattle in the middle of the eighties. These cattle imported in
the mid eighties could have been rendered in the late eighties and
therefore led to an internal challenge in the early nineties. It is
possible that imported meat and bone meal (MBM) into the USA reached
domestic cattle and leads to an internal challenge in the early
nineties.

A processing risk developed in the late 80s/early 90s when cattle
imports from BSE risk countries were slaughtered or died and were
processed (partly) into feed, together with some imports of MBM. This
risk continued to exist, and grew significantly in the mid 90's when
domestic cattle, infected by imported MBM, reached processing. Given the
low stability of the system, the risk increased over the years with
continued imports of cattle and MBM from BSE risk countries.

EFSA concludes that the current GBR level of USA is III, i.e. it is
likely but not confirmed that domestic cattle are (clinically or
pre-clinically) infected with the BSE-agent. As long as there are no
significant changes in rendering or feeding, the stability remains
extremely/very unstable. Thus, the probability of cattle to be
(pre-clinically or clinically) infected with the BSE-agent persistently
increases.

http://www.efsa.eu.int/science/efsa_scientific_reports/gbr_assessments/573_en.html

From: Terry S. Singeltary Sr. \[flounder@wt.net\]

Sent: Tuesday, July 29, 2003 1:03 PM

To: fdadockets@oc.fda.gov

Cc: ggraber@cvm.fda.gov; Linda.Grassie@fda.gov; BSE-L

Subject: Docket No. 2003N-0312 Animal Feed Safety System \[TSS
SUBMISSION

TO DOCKET 2003N-0312\]

Greetings FDA,

snip\...

PLUS, if the USA continues to flagrantly ignore the \_documented\_
science to date about the known TSEs in the USA (let alone the
undocumented TSEs in cattle), it is my opinion, every other Country that
is dealing with BSE/TSE should boycott the USA and demand that the SSC
reclassify the USA BSE GBR II risk assessment to BSE/TSE GBR III
\'IMMEDIATELY\'. for the SSC to \_flounder\_ any longer on this issue,
should also be regarded with great suspicion as well. NOT to leave out
the OIE and it\'s terribly flawed system of disease surveillance. the
OIE should make a move on CWD in the USA, and make a risk assessment on
this as a threat to human health. the OIE should also change the
mathematical formula for testing of disease. this (in my opinion and
others) is terribly flawed as well. to think that a sample survey of 400
or so cattle in a population of 100 million, to think this will find
anything, especially after seeing how many TSE tests it took Italy and
other Countries to find 1 case of BSE (1 million rapid TSE test in less
than 2 years, to find 102 BSE cases), should be proof enough to make
drastic changes of this system. the OIE criteria for BSE Country
classification and it\'s interpretation is very problematic. a text that
is suppose to give guidelines, but is not understandable, cannot be
considered satisfactory. the OIE told me 2 years ago that they were
concerned with CWD, but said any changes might take years. well, two
years have come and gone, and no change in relations with CWD as a human
health risk. if we wait for politics and science to finally make this
connection, we very well may die before any decisions

or changes are made. this is not acceptable. we must take the politics
and the industry out of any final decisions of the Scientific community.
this has been the problem from day one with this environmental man made
death sentence. some of you may think i am exaggerating, but you only
have to see it once, you only have to watch a loved one die from this
one time, and you will never forget, OR forgive\...yes, i am still very
angry\... but the transmission studies DO NOT lie, only the politicians
and the industry do\... and they are still lying to this day\...TSS

http://www.fda.gov/ohrms/dockets/dockets/03n0312/03N-0312_emc-000001.txt

GREETINGS AGAIN APHIS ET AL,

Moving on to the theory that BSE agent is not in blood. THIS is what
they use to think with nvCJD. However

the nvCJD agent has now been detected and transmitted the TSE agent by
blood. nvCJD is the BSE agent

that has transmitted to humans. nvCJD is human BSE. so if nvCJD
transmits by blood, why not BSE? with

the limited testing to date, the limited sensitivity of the detection of
the BSE/TSE agent blood to date, i would

not be so sure that the BSE/TSE agent does not transmit by blood. just
recent Ag. Comm. Johanns stated

that they would not address the blood issue being fed to cattle. a
foolish and careless mistake. but typical.

we now have detected new atypical strains of the BSE/TSE agent in cattle
in many countries i.e. Japan, France,

Belgium, Germany, and Italy. In the Italian study of BASE, a new? TSE in
cattle they have discovered that is

not like the nvCJD, but very similar to sporadic CJD. They have detected
2 such cattle at printing of this study

March 2, 2004. Identification of a second bovine amyloidotic spongiform
encephalopathy: Molecular similarities

with sporadic Creutzfeldt-Jakob disease PNAS. MY point is that with
these new atypical TSEs showing up in

cattle, sheep and goats, we don\'t know if blood and other tissues
transmits the disease. THE SRM list may

and should be reevaluated. WE know BSE is in the USA, but we also know
that in the studies of Mission Texas,

where USA suffolk scrapie sheep were inoculated into USA cattle, the
PRION agent that was produced did not

look like the UK BSE strain. so why would all CJD cases in the USA look
like the UK human BSE i.e. nv/v CJD?

WHY wait and expose millions and millions needlessly as in the past with
previous TSE blunders, why wait to

act. why not act first with what we know, which is very concerning, then
as science evolves, go from there.

references as follow ;

UK Strategy for Research and

Development on Human and Animal

Health Aspects of Transmissible

Spongiform Encephalopathies

2005-2008

snip\...

3.2 Tackling the spread of infection

3.2.1 The species barrier and the carrier state

3.2.1.1 The possibility of 'carrier' states in animals and humans, and
our present inability

to identify them, pose a potential threat to public and animal health.
The susceptibility of

humans to BSE infection, and the ability of the disease to remain
clinically silent for

many years, is of major concern to DH. Although the death of a UK blood
donor from

vCJD in 1999 three years after making the donation and the subsequent
death from vCJD

of the recipient in 2003 have not been causally linked, transmission of
infection through

blood transfusion is the most likely explanation327. The case heightens
concerns that

'carriers' could be transmitting the disease through blood, tissue and
organ donation or

by contaminating surgical instruments when undergoing surgery. DH will
continue to

support research to detect infectious prions in human tissue, to
investigate the

decontamination of surgical instruments and to develop measures to
protect blood

supplies.

3.2.1.2 Animal models of some TSEs have detected infectivity in blood.
Experiments,

which have involved transfusing large volumes of blood from infected
sheep to healthy

recipient sheep, have demonstrated that infectivity can be transmitted
by blood

transfusion. A central part of DH policy in this area has been the
leucodepletion of blood

donations and the efficacy of this technology can now be tested in
sheep.

snip\...

4.5.4 In 1987, epidemiological studies of BSE cases identified meat and
bone meal as the

probable means by which the disease was being spread. In an attempt to
prevent

further infections a ban on incorporating ruminant protein in ruminant
feed was

introduced in July 1988. Due to the long incubation period associated
with this disease

the efficacy of this control measure was not immediately apparent. As
time passed it

became clear from the number of cases born after the ban that it was not
wholly

effective.

4.5.5 Epidemiological analysis of these cases showed that a high
proportion of them

occurred in areas where the pig population was high. This observation,
coupled with

research data that showed that only a very small dose of the infective
material was

needed to cause disease in cattle, led to the conclusion that
cross-contamination of feed

was occurring.

4.5.6 Since 1988, increasingly stringent feed controls have been put in
place. Key

amongst these have been:

• the ban on the use of specified bovine offal in all animal feed
(September 1990);

• the ban on feeding any farmed animal, including horses and fish, with
mammalian

meat and bone meal. (This began in March 1996 but following this ban
there was

a recall scheme and the date from which the ban was considered to be
fully

effective is regarded as being 1 August 1996);

62

v6.1

• EU-wide controls on feed which extended the ban to include all
processed animal

protein, including that derived from birds and fish (implemented in the
UK from 1

August 2001).

4.5.7 As illustrated in fig. \[ \] these later measures have reduced the
spread of BSE.

However, they have not been one hundred per cent effective. As at 31st
December 2003

there had been 81 cases of BSE in animals born since 1 August 1996 in
the UK.

full text 91 pages;

http://www.mrc.ac.uk/pdf-about-tse_uk_strategy_june2005.pdf

GREETINGS AGAIN APHIS ET AL,

JUST what about those old studies at Mission Texas and the atypical TSE
in cattle?

would it not be prudent for human health purpose, the question that,
with all the atypical

TSEs showing up in animal and man in different countries, the fact that
most all of these

TSEs transmit as freely or not as freely as BSE (depending whom you have
witnessed die from

this agent either directly or indirectly via a multitude of potential
routes and sources) to primates.

would it not be prudent to ask yourself if some if not all of these
sporadic CJDs might be a

by-product of these TSEs either directly or indirectly via a multitude
of proven routes and sources

in animal studies? it is unethical for human transmission studies
considering the fact that the agent

is 100% fatal, slow, but fatal. there has been no sound science in any
of the recent decisions in the

USA in regards to BSE/TSE human or animal, all one has to do is look at
TEXAS, the mad cow that

got away, the stumbling and staggering one that NO TSE TEST AT ALL was
done, ordered rendered,

and then the infamous positive, positve, secret positive, inconclusive
(NO WB), negative, 8 month delay,

then the \'Fong Effect\' took place, THEN FINALLY CONFIRMED SOME 8
MONTHS LATER IN WEYBRIDGE.

Finally recently, another inconclusive that took place that sat untested
on some shelf for about 4 months while

the Texas mad cow blunder was going on. the tissues of this cow this
time were preserved in preservative as

to render any WB for further confirmation, what i called the \'FONG
SYNDROME\' or the \'end around\' the WB

ordered previously by the Honorable Phyllis Fong of the OIG. Politics at
its finest, to hell with human health.

WE find now that 9,200 USA POTENTIAL MAD COWS IN JUNE 2004 ENHANCED
COVER-UP

SURVEILLANCE PROGRAM WENT UNTESTED WITH NO RAPID TEST OR WB, ONLY IHC,
the test

that fails the most, that is very unreliable as noted above in my
submission by Prusiner et al and other scientist.

NOW, back to Mission, Texas ;

\>\> Differences in tissue distribution could require new regulations

\>\> regarding specific risk material (SRM) removal.

snip\...end

full text 33 PAGES ;

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

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

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.

http://www.bseinquiry.gov.uk/

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

50010\.

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

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8133096&dopt=Citation

Intracerebral transmission of scrapie to cattle FULL TEXT PDF;

SNIP\...

Discussion

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).

PrP-res was found in ALL CHALLENGED CALVES REGARDLESS OF CLINCIAL SIGNS,
and

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

\...

snip\...

WE also conclude from these studies that scrapie in cattle MIGHT NOT BE

RECOGNIZED BY ROUTINE HISTOPATHOLOGICAL EXAMINATION OF THE BRAIN AND
SUGGEST

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). \...

snip\...

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\...

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

Visit to USA \... info on BSE and Scrapie

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

GREETINGS AGAIN APHIS ET AL,

COMMENTING ON THE SRMs AND THE REMOVAL OF SRMs. I FIND IT DISTURBING
THAT RECENTLY,

AFTER A LONG BATTLE FOR DOCUMENTS VIA THE FOIA THAT THE MEDIA AND
CONSUMERS UNION REQUESTED,

THAT DURING THE JUNE 2004 ENHANCED BSE SURVIELLANCE PROGRAM, THAT OVER
1,000 CITATIONS WERE

ISSUED FOR VARIOUS SRM VIOLATIONS, BUT YET IT TOOK THE FOIA TO GET THIS
TO THE PUBLIC.

WOULD it not be likely that from some of these noncompliance reports
that

indeed some breaches led to some potential tainted materials to enter
the

animal/human feed chain?

ALL we have heard about in the last 7 years or better, well, since the

12/14/97 partial and voluntary ruminant-to-ruminant feed ban is that the

feed ban is working, tripple fire walls, no ruminant protein entering
the

animal feed chain. along with this was a constant barage of \'no mad cow

disease in the USA\'. then we find this 12 year old TEXAS cow that was

infected from tainted feed some time in that 12 year period. so, the NE

TEXAS CJD cluster, where it was stated that NO mad cow was in the USA or

TEXAS at that time frame, was in fact not true.

SINCE some 460 of these occurred because slaughter plants did not have
an

adequate plan for dealing with BSE in their plant\'s food safety plan,
as

required by the USDA, the analysis showed, and of those 460 violations,
60

percent described plans that contained no mention of BSE at all. then
again,

would it not be very possible that indeed some potentially tainted
material

of a BSE or atypical TSE DID enter the animal feed chain, thus later
some of

those animals entering the human food chain.

WHAT about the SRM violations? Violations of rules about the removal and

handling of specified risk material (SRMs) occurred at 131 plants in at

least 35 states. SRMs are the high-risk materials, such as brains and
spinal

cords, most likely to be infectious. More than 30 percent of the NRs

analyzed were due to either improperly handling or removing SRMs. Could
this

not have also led to potentially BSE/TSE tainted materials entering the

animal/human food chain?

In 10 percent of the NRs analyzed, plants incorrectly identified the age
of

cattle. THIS also could have led to tainted BSE/TSE SRM materials
entering

the animal/human food chain.

IN my opinion, this could have led to many feed discrepancies and should

HAVE been reported to the public, without the media having to request
this

data via FOIA. I think in the future it would be best if the NRs

(non-compliance reports) were made easily available to the public in
there

feed enforement reports. \...

FOR IMMEDIATE RELEASE

AUGUST 18, 2005

5:25 PM CONTACT: Public Citizen

\(202\) 588-1000

Evidence of Weak Meat Inspection Program Found in Nearly a Thousand

Violations of Mad Cow Rules at Slaughter Plants

Noncompliance Records Show Plants Failed to Follow Regulations

WASHINGTON - August 18 - In stark contrast to the public relations
message

touted by the U.S. Department of Agriculture (USDA) and the beef
industry

that the U.S. regulatory system is adequate to prevent the spread of mad
cow

disease, an analysis released today by the consumer group Public Citizen

found significant lapses in the industry\'s compliance with federal
rules.

The analysis stems from a December 2004 Freedom of Information Act
(FOIA)

request from Public Citizen to the USDA for all \"noncompliance
records\"

(NRs) related to bovine spongiform encephalopathy (BSE). Public Citizen

received copies of 829 records on Aug. 15.

More than half the violations (460) occurred because slaughter plants
did

not have an adequate plan for dealing with BSE in their plant\'s food
safety

plan, as required by the USDA, the analysis shows. Of those 460
violations,

60 percent described plans that contained no mention of BSE at all.

\"The fact that 60 percent of the violations were due to a failure to
even

mention BSE or risk materials such as brains and spinal cords is

significant,\" said Patty Lovera, deputy director of Public Citizen\'s
food

program. \"If officials running a meat plant cannot be bothered to
recognize

the risk of BSE when writing their safety plan, how much of a priority
is it

in daily operations and training of staff?\"

The analysis also found that:

Violations of rules about the removal and handling of specified risk

material (SRMs) occurred at 131 plants in at least 35 states. SRMs are
the

high-risk materials, such as brains and spinal cords, most likely to be

infectious. More than 30 percent of the NRs analyzed were due to either

improperly handling or removing SRMs. The SRM ban is considered a
critical

firewall in protecting the food supply from BSE.

The violations described in the NRs occurred from January 2004 through
March

2005\. This shows that the problems in the plants persisted long after
plants

should have adapted to new rules issued in January 2004 after the
discovery

of the first case of BSE in the United States.

In 10 percent of the NRs analyzed, plants incorrectly identified the age
of

cattle. Properly determining the age of cattle is a crucial step in
proper

SRM removal because the definition of SRMs is dependent on age; in
cattle

older than 30 months, there is a greater likelihood that SRM will carry
BSE

and therefore must be removed. Accurately identifying the head, spine
and

carcass of cattle by age is necessary to ensure that all SRMs are
removed as

the carcass moves down the slaughter line.

\"These enforcement records only increase our concerns about how easily

potentially infected cattle are bypassing inspection points at

slaughterhouses, creating one more opportunity for infected meat to slip

through the system,\" said Tony Corbo, legislative representative of
Public

Citizen\'s food program. \"We\'re approaching the two-year mark of our
first

case of mad cow in the United States, yet the government is still
lagging

behind on protecting consumers.\"

Public Citizen sent the FOIA request to the USDA in December 2004 after
the

chairman of the USDA meat inspectors union, Stan Painter, raised
concerns

about the agency\'s policy for ensuring that cattle age is properly

determined. Instead of investigating whether the policy was adequate,
the

agency opened a misconduct investigation on Painter. The investigation
was

closed this week, shortly after Public Citizen received the
documentation,

which contained more than 80 records of plants improperly identifying
cattle

age.

\###

http://www.commondreams.org/news2005/0819-02.htm

Daily Update

On August 19, 2005, no inconclusive test results were reported.

National Veterinary Services Laboratory (NVSL) Immunohistochemistry
(IHC) Testing Summary

The BSE enhanced surveillance program involves the use of a rapid
screening test, followed by confirmatory testing for any samples that
come back \"inconclusive.\" The weekly summary below captures all rapid
tests conducted as part of the enhanced surveillance effort. It should
be noted that since the enhanced surveillance program began, USDA has
also conducted approximately 9,200 routine IHC tests on samples that did
not first undergo rapid testing. This was done to ensure that samples
inappropriate for the rapid screen test were still tested, and also to
monitor and improve upon IHC testing protocols. Of those 9,200 routine
tests, one test returned a non-definitive result on July 27, 2005. That
sample underwent additional testing at NVSL, as well as at the
Veterinary Laboratories Agency in Weybridge, England, and results were
negative.

To view the IHC testing numbers from 1990 through 2004, click on the
following link:
http://www.aphis.usda.gov/lpa/issues/bse/surveillance/figure2f.html

GREETINGS AGAIN,

THE APHIS/USDA/FSIS et al has failed the public terribly with there
industry friendly approach to erradicate BSE/TSE aka mad cow disease in
the USA. the 8/4/97 partial and voluntary ruminant to rumiant feed ban
program and there cealed borders were, have been and still are a joke.
by sleeping with the industry, this administration has needlessly
exposed millions and millions to the TSE agent at home, and abroad. THE
fact that the first documented home grown mad cow came from TEXAS, the
TAHC/USDA et al had already covered up one suspect mad cow and rendered
with no test at all, but for the Honorable Phyllis Fong of the OIG to
have the courage to go around the Agriculture Secretary Johann to have
it confirmed in Weybridge, England (it would have never been confirmed
any other way), but to have her do this, was truely a coup of sorts, one
of the most couragous I have seen in a while by any person of our
Government. BUT to finally have this mad cow confirmed, and then have
the Secretary of Agriculture Johann not give praise to her for finally
confirming this deadly disease in a home grown case, but to have him
want her head for it raises serious serious doubts of the over June 2004
Enhanced BSE program where they claim some 500,000 cows have now been
tested. NOW, we find indeed this was the case. WE find now that of those
500,000+ cows, 9,200 of them did not have rapid testing at all, no WB,
only IHC. The least likely to find a case of BSE/TSE, the one that fails
the most. a proven hit and miss test. Dr. Detwiler tried to tell them
this in 2003 ;

USDA 2003

We have to be careful that we don\'t get so set in the way we do things
that

we forget to look for different emerging variations of disease. We\'ve
gotten

away from collecting the whole brain in our systems. We\'re using the
brain

stem and we\'re looking in only one area. In Norway, they were doing a

project and looking at cases of Scrapie, and they found this where they
did

not find lesions or PRP in the area of the obex. They found it in the

cerebellum and the cerebrum. It\'s a good lesson for us. Ames had to go

back and change the procedure for looking at Scrapie samples. In the
USDA,

we had routinely looked at all the sections of the brain, and then we
got

away from it. They\'ve recently gone back.

Dr. Keller: Tissues are routinely tested, based on which tissue provides
an

\'official\' test result as recognized by APHIS

.

Dr. Detwiler: That\'s on the slaughter. But on the clinical cases,
aren\'t

they still asking for the brain? But even on the slaughter, they\'re
looking

only at the brainstem. We may be missing certain things if we confine

ourselves to one area.

snip\...\...\...\....

Dr. Detwiler: It seems a good idea, but I\'m not aware of it.

Another important thing to get across to the public is that the
negatives

do not guarantee absence of infectivity. The animal could be early in
the

disease and the incubation period. Even sample collection is so
important.

If you\'re not collecting the right area of the brain in sheep, or if

collecting lymphoreticular tissue, and you don\'t get a good biopsy, you

could miss the area with the PRP in it and come up with a negative test.

There\'s a new, unusual form of Scrapie that\'s been detected in Norway.
We

have to be careful that we don\'t get so set in the way we do things
that we

forget to look for different emerging variations of disease. We\'ve
gotten

away from collecting the whole brain in our systems. We\'re using the
brain

stem and we\'re looking in only one area. In Norway, they were doing a

project and looking at cases of Scrapie, and they found this where they
did

not find lesions or PRP in the area of the obex. They found it in the

cerebellum and the cerebrum. It\'s a good lesson for us. Ames had to go

back and change the procedure for looking at Scrapie samples. In the
USDA,

we had routinely looked at all the sections of the brain, and then we
got

away from it. They\'ve recently gone back.

Dr. Keller: Tissues are routinely tested, based on which tissue provides
an

\'official\' test result as recognized by APHIS

.

Dr. Detwiler: That\'s on the slaughter. But on the clinical cases,
aren\'t

they still asking for the brain? But even on the slaughter, they\'re
looking

only at the brainstem. We may be missing certain things if we confine

ourselves to one area.

snip\...

FULL TEXT;

Completely Edited Version

PRION ROUNDTABLE

Accomplished this day, Wednesday, December 11, 2003, Denver, Colorado

Greetings again APHIS et al,

THE June 2004 Enhanced BSE/TSE surveillance program was a terrible
failure, other than to prove just how bad the situation is in the USA,
and how out of control the Federal Government is in trying to cover it
up. THE OIG should hold an inquiry into this program. THE BSE MRR policy
should be dismantled, and the USA BSE GBR risk assessment should be
immediately raised to BSE GBR IV.

THE International guidelines for trade in animal and animal products
which are developed by the World Organization for Animal Health
(formerly known as the Office International des Epizooties (OIE)), is
and has been terrible flawed. ALL one has to do is to look at the
countries that have gone by there very minimal guidelines, most all went
on to develop BSE. IT would be nice if the OIE et al would define
"controlled BSE-risk country" or "effectively enforced ban". The USA and
North America have neither. THIS has been proven time and time again via
the GAO, OIG and the European EFSA BSE-risk assessments of North
American countries. Many Countries have not even reported there first
case of BSE yet, and many countries have not even produced a risk
analysis for BSE. A fine example is Mexico and Canada. I pointed out
about Canada above, but now lets look at Mexico, which is also a BSE GBR
III country. IN Mexico, they are NOT even required to remove SRM;

Working Group Report on

the Assessment of the Geographical BSE-Risk (GBR) of

MEXICO

2004

Specified Risk Material (SRM) and fallen stock

There is no SRM-ban. SRM is normally destined for human consumption.
According

to the CD, fallen stock from pasture and diseased animals are
incinerated and not

rendered.

Conclusion on the ability to avoid recycling

In light of the above information, it has to be assumed that the BSE
agent, should it

have entered Mexico, could have been recycled and potentially amplified.

snip\...

In view of the above - described consideration the combination of the
very / extremely

high external challenges with a very unstable system makes the
occurrence of an

internal challenge likely in Mexico from approximately 1993 onwards.

4.2 Risk that BSE infectivity entered processing

It is likely that BSE infectivity entered processing at the time of
imported 'at - risk'

MBM (1993) and at the time of slaughter of imported live 'at - risk'
cattle (mid to late

1990's). The high level of external challenge is maintained throughout
the reference

period, and the system has not been made stable, leading to increased
internal

4.3 Risk that BSE infectivity was recycled and propagated

It is likely that BSE infectivity was recycled and propagated from
approximately

1993\. The risk has since grown consistently due to a maintained
internal and external

challenge and lack of a stable system.

5\. CONCLUSION ON THE GEOGRAPHICAL BSE - RISK

5.1 The current GBR as function of the past stability and challenge

The current geographical BSE risk (GBR) level is III, i.e. it is likely
but not confirmed

that domestic cattle are (clinically or pre-clinically) infected with
the BSE-agent.

snip\...end

http://www.efsa.eu.int/science/efsa_scientific_reports/gbr_assessments/scr_annexes/566/sr04_biohaz02_mexico_report_annex_en1.pdf

MORE ON THOSE USA BSE/TSE SEALED BORDERS

Aug. 22, 2005, 12:35AM

Mexican cattle business linked to drug cartels

Animals sold to Texas ranchers by 2 companies can be seized

By MICHAEL HEDGES

Copyright 2005 Houston Chronicle Washington Bureau

WASHINGTON - The Treasury Department, trying to block an elaborate
money-laundering scheme, has announced that two Mexican cattle companies
are fronts for drug-trafficking cartels.

The action means that cattle sold by the companies to Texas ranchers
after Friday\'s announcement are subject to seizure by the federal
government, said a high-ranking Treasury official who asked not to be
named.

\"Cattle already purchased and owned before the companies were
identified as tied to the drug cartels are not going to suddenly be
blocked,\" the official said.

The Treasury Department plans to inform cattle associations and other
groups later this week of the action taken against the Mexican
companies, officials said. The Treasury also will provide other
information, such as the brands used by the cattle companies linked to
the drug cartels.

For now, buyers are expected to practice due diligence when purchasing
cattle.

Two Mexican drug cartels were named in the Treasury Department\'s
statement, the Arriola Marquez organization and the Arellano Felix
cartel based in Tijuana. The Arriola Marquez group, based in Mexico\'s
Chihuahua state, is linked to Mexican drug kingpin Joaquin \"El Chapo\"
Guzman, the department said. Guzman leads one of the factions fighting
for control of Nuevo Laredo and its smuggling routes into Texas,
officials have said.

snip\...

It is not clear how many Mexican cattle owned by companies linked to
drug cartels had been sold in Texas, said government officials and Texas
cattle raisers.

snip\...end

http://www.chron.com/cs/CDA/ssistory.mpl/topstory/3319609

I do not think that USDA et al used rapid test, WB or IHC on any of
these cattle for BSE/TSE.

TRIPLE firewalls and SEALED borders, I dont think so.

FINALLY COMMENTING ON THE UK BSE/nv/vCJD ONLY THEORY. there are
different strains of TSE showing up in cattle, sheep, and goats. nvCJD
has been documented in a 74 year old and the young are dying of sporadic
CJD. one of the new atypical strains of TSE in cattle \'BASE\' does not
look like nvCJD in humans, but it looks very similar to sporadic CJD.
also, Asante/Collinge et al have shown that BSE can propagate as nvCJD
AND sporadic CJD. to continue to flounder and ignore all of this as in
the past, to continue to cater to big beef, big feed will only allow
this agent to further amplify and spread. there is more to this agent
than the mad cow hamburger. AS long as the BSE MRR policy is in effect,
the agent will continue to spread globally. references as follow ;

Characterization of two distinct prion strains derived from bovine
spongiform encephalopathy transmissions to inbred mice

Sarah E. Lloyd, Jacqueline M. Linehan, Melanie Desbruslais, Susan
Joiner, Jennifer Buckell, Sebastian Brandner, Jonathan D. F. Wadsworth
and John Collinge

MRC Prion Unit and Department of Neurodegenerative Disease, Institute of
Neurology, University College, London WC1N 3BG, UK

Correspondence

John Collinge

j.collinge@prion.ucl.ac.uk

Distinct prion strains can be distinguished by differences in incubation
period, neuropathology and biochemical properties of disease-associated
prion protein (PrPSc) in inoculated mice. Reliable comparisons of mouse
prion strain properties can only be achieved after passage in
genetically identical mice, as host prion protein sequence and genetic
background are known to modulate prion disease phenotypes. While
multiple prion strains have been identified in sheep scrapie and
Creutzfeldt-Jakob disease, bovine spongiform encephalopathy (BSE) is
thought to be caused by a single prion strain. Primary passage of BSE
prions to different lines of inbred mice resulted in the propagation of
two distinct PrPSc types, suggesting that two prion strains may have
been isolated. To investigate this further, these isolates were
subpassaged in a single line of inbred mice (SJL) and it was confirmed
that two distinct prion strains had been identified. MRC1 was
characterized by a short incubation time (110±3 days), a
mono-glycosylated-dominant PrPSc type and a generalized diffuse pattern
of PrP-immunoreactive deposits, while MRC2 displayed a much longer
incubation time (155±1 days), a di-glycosylated-dominant PrPSc type and
a distinct pattern of PrP-immunoreactive deposits and neuronal loss.
These data indicate a crucial involvement of the host genome in
modulating prion strain selection and propagation in mice. It is
possible that multiple disease phenotypes may also be possible in BSE
prion infection in humans and other animals.

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

Identification of a second bovine amyloidotic spongiform encephalopathy:
Molecular similarities with sporadic Creutzfeldt-Jakob disease

Cristina Casalone \*, Gianluigi Zanusso , Pierluigi Acutis \*, Sergio
Ferrari , Lorenzo Capucci , Fabrizio Tagliavini ¶, Salvatore Monaco
\|\|, and Maria Caramelli \*

\*Centro di Referenza Nazionale per le Encefalopatie Animali, Istituto
Zooprofilattico Sperimentale del Piemonte, Liguria e Valle d\'Aosta, Via
Bologna, 148, 10195 Turin, Italy; Department of Neurological and Visual
Science, Section of Clinical Neurology, Policlinico G.B. Rossi, Piazzale
L.A. Scuro, 10, 37134 Verona, Italy; Istituto Zooprofilattico
Sperimentale della Lombardia ed Emilia Romagna, Via Bianchi, 9, 25124
Brescia, Italy; and ¶Istituto Nazionale Neurologico \"Carlo Besta,\" Via
Celoria 11, 20133 Milan, Italy

Edited by Stanley B. Prusiner, University of California, San Francisco,
CA, and approved December 23, 2003 (received for review September 9,
2003)

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are
mammalian neurodegenerative disorders characterized by a
posttranslational conversion and brain accumulation of an insoluble,
protease-resistant isoform (PrPSc) of the host-encoded cellular prion
protein (PrPC). Human and animal TSE agents exist as different
phenotypes that can be biochemically differentiated on the basis of the
molecular mass of the protease-resistant PrPSc fragments and the degree
of glycosylation. Epidemiological, molecular, and transmission studies
strongly suggest that the single strain of agent responsible for bovine
spongiform encephalopathy (BSE) has infected humans, causing variant
Creutzfeldt-Jakob disease. The unprecedented biological properties of
the BSE agent, which circumvents the so-called \"species barrier\"
between cattle and humans and adapts to different mammalian species, has
raised considerable concern for human health. To date, it is unknown
whether more than one strain might be responsible for cattle TSE or
whether the BSE agent undergoes phenotypic variation after natural
transmission. Here we provide evidence of a second cattle TSE. The
disorder was pathologically characterized by the presence of
PrP-immunopositive amyloid plaques, as opposed to the lack of amyloid
deposition in typical BSE cases, and by a different pattern of regional
distribution and topology of brain PrPSc accumulation. In addition,
Western blot analysis showed a PrPSc type with predominance of the low
molecular mass glycoform and a protease-resistant fragment of lower
molecular mass than BSE-PrPSc. Strikingly, the molecular signature of
this previously undescribed bovine PrPSc was similar to that encountered
in a distinct subtype of sporadic Creutzfeldt-Jakob disease.

\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\--

C.C. and G.Z. contributed equally to this work.

\|\|To whom correspondence should be addressed.

E-mail: salvatore.monaco@mail.univr.it.

www.pnas.org/cgi/doi/10.1073/pnas.0305777101

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

eurobiology

Adaptation of the bovine spongiform encephalopathy agent to primates and
comparison with Creutzfeldt- Jakob disease: Implications for human
health

Corinne Ida Lasmézas\*,, Jean-Guy Fournier\*, Virginie Nouvel\*, Hermann
Boe\*, Domíníque Marcé\*, François Lamoury\*, Nicolas Kopp, Jean-Jacques
Hauw§, James Ironside¶, Moira Bruce, Dominique Dormont\*, and
Jean-Philippe Deslys\*

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

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

Abstract

Top

Abstract

Introduction

Materials and Methods

Results

Discussion

Conclusions

References

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

Introduction

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

IN light of Asante/Collinge et al findings 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;

\-\-\-\-\-\-\-- Original Message \-\-\-\-\-\-\-- Subject: re-BSE prions
propagate as

either variant CJD-like or sporadic CJD Date: Thu, 28 Nov 2002 10:23:43

-0000 From: \"Asante, Emmanuel A\" To:

\"\'flounder@wt.net\'\"

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am

a Senior Scientist in the MRC Prion Unit and the lead author on the

paper. I have attached a pdf copy of the paper for your attention. Thank

you for your interest in the paper.

In respect of your first question, the simple answer is, yes. As you

will find in the paper, we have managed to associate the alternate

phenotype to type 2 PrPSc, the commonest sporadic CJD.

It is too early to be able to claim any further sub-classification in

respect of Heidenhain variant CJD or Vicky Rimmer\'s version. It will

take further studies, which are on-going, to establish if there are

sub-types to our initial finding which we are now reporting. The main

point of the paper is that, as well as leading to the expected new

variant CJD phenotype, BSE transmission to the 129-methionine genotype

can lead to an alternate phenotype which is indistinguishable from type

2 PrPSc.

I hope reading the paper will enlighten you more on the subject. If I

can be of any further assistance please to not hesitate to ask. Best
wishes.

Emmanuel Asante

\<\>
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial

College School of Medicine (St. Mary\'s) Norfolk Place, LONDON W2 1PG

Tel: +44 (0)20 7594 3794 Fax: +44 (0)20 7706 3272 email:

e.asante@ic.ac.uk (until 9/12/02)

New e-mail: e.asante@prion.ucl.ac.uk (active from now)

\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

snip\...

full text ;

http://www.fda.gov/ohrms/dockets/ac/03/slides/3923s1_OPH.htm

SCRAPIE USA MONTHLY REPORT 2005

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

FULL TEXT ;

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

SCRAPIE USA JUNE 2005 UPDATE

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

snip\...

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

snip\...end

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

USA CWD MAP

http://www.aphis.usda.gov/vs/nahps/cwd/labmap.html

Perspective

Chronic Wasting Disease and Potential Transmission to Humans

Ermias D. Belay,\* Ryan A. Maddox,\* Elizabeth S. Williams,† Michael W.
Miller,‡ Pierluigi Gambetti,§ and Lawrence B. Schonberger\*

\*Centers for Disease Control and Prevention, Atlanta, Georgia, USA;
†University of Wyoming, Laramie, Wyoming, USA; ‡Colorado Division of
Wildlife, Fort Collins, Colorado, USA; and §Case Western Reserve
University, Cleveland, Ohio, USA

Suggested citation for this article: Belay ED, Maddox RA, Williams ES,
Miller MW, Gambetti P, Schonberger LB. Chronic wasting disease and
potential transmission to humans. Emerg Infect Dis \[serial on the
Internet\]. 2004 Jun \[date cited\]. Available from:
http://www.cdc.gov/ncidod/EID/vol10no6/03-1082.htm

\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\--

Chronic wasting disease (CWD) of deer and elk is endemic in a tri-corner
area of Colorado, Wyoming, and Nebraska, and new foci of CWD have been
detected in other parts of the United States. Although detection in some
areas may be related to increased surveillance, introduction of CWD due
to translocation or natural migration of animals may account for some
new foci of infection. Increasing spread of CWD has raised concerns
about the potential for increasing human exposure to the CWD agent. The
foodborne transmission of bovine spongiform encephalopathy to humans
indicates that the species barrier may not completely protect humans
from animal prion diseases. Conversion of human prion protein by
CWD-associated prions has been demonstrated in an in vitro cell-free
experiment, but limited investigations have not identified strong
evidence for CWD transmission to humans. More epidemiologic and
laboratory studies are needed to monitor the possibility of such
transmissions.

SNIP\...

Conclusions

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

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

Research

Environmental Sources of Prion Transmission in Mule Deer

Michael W. Miller,\* Elizabeth S. Williams,† N. Thompson Hobbs,‡ and
Lisa L. Wolfe\*

\*Colorado Division of Wildlife, Fort Collins, Colorado, USA;
†University of Wyoming, Laramie, Wyoming, USA; and ‡Colorado State
University, Fort Collins, Colorado, USA

Suggested citation for this article: Miller MW, Williams ES, Hobbs NT,
Wolfe LL. Environmental sources of prion transmission in mule deer.
Emerg Infect Dis \[serial on the Internet\]. 2004 Jun \[date cited\].
Available from: http://www.cdc.gov/ncidod/EID/vol10no6/04-0010.htm

\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\-\--

Whether transmission of the chronic wasting disease (CWD) prion among
cervids requires direct interaction with infected animals has been
unclear. We report that CWD can be transmitted to susceptible animals
indirectly, from environments contaminated by excreta or decomposed
carcasses. Under experimental conditions, mule deer (Odocoileus
hemionus) became infected in two of three paddocks containing naturally
infected deer, in two of three paddocks where infected deer carcasses
had decomposed in situ ˜1.8 years earlier, and in one of three paddocks
where infected deer had last resided 2.2 years earlier. Indirect
transmission and environmental persistence of infectious prions will
complicate efforts to control CWD and perhaps other animal prion
diseases.

snip\...

Discussion

Prions cannot be directly demonstrated in excreta or soil. However, CWD
infection--specific protease-resistant prion protein (PrPCWD)
accumulates in gut-associated lymphoid tissues (e.g., tonsils, Peyer
patches, and mesenteric lymph nodes) of infected mule deer (11,17,22),
which implicates alimentary shedding of the CWD agent in both feces and
saliva (10,11,17). Because PrPCWD becomes progressively abundant in
nervous system and lymphoid tissues through the disease course (11),
carcasses of deer succumbing to CWD also likely harbor considerable
infectivity and thus serve as foci of infection. We could not determine
the precise mechanism for CWD transmission in excreta-contaminated
paddocks, but foraging and soil consumption seemed most plausible. Deer
did not actively consume decomposed carcass remains, but they did forage
in the immediate vicinity of carcass sites where a likely nutrient flush
(23) produced lush vegetation (Figure).

Our findings show that environmental sources of infectivity may
contribute to CWD epidemics and illustrate the potential complexity of
such epidemics in natural populations. The relative importance of
different routes of infection from the environment cannot be discerned
from our experiment, but each could play a role in sustaining natural
epidemics. Although confinement likely exaggerated transmission
probabilities, conditions simulated by this experiment do arise in the
wild. Mule deer live in established home ranges and show strong fidelity
to historic home ranges (24-26). As a result of such behavior,
encounters with contaminated environments will occur more frequently
than if deer movements were random. Feces and carcass remains are
routinely encountered on native ranges, thus representing natural
opportunities for exposure. Social behavior of deer, particularly their
tendency to concentrate and become sedentary on their winter range, also
may increase the probability of coming into contact with sources of
infection in their environment.

The ability of the CWD agent to persist in contaminated environments for
\>2 years may further increase the probability of transmission and
protract epidemic dynamics (8). Because infectivity in contaminated
paddocks could not be measured, neither the initial levels nor
degradation rate of the CWD agent in the environment was estimable.
However, the observed persistence of the CWD agent was comparable to
that of the scrapie agent, which persisted in paddocks for ˜1 to 3 years
after removal of naturally infected sheep (7). Similarities between the
CWD and scrapie agents suggest that environmental persistence may be a
common trait of prions. Whether persistence of the BSE prion in
contaminated feed production facilities or in environments where cattle
reside contributed to BSE cases in the United Kingdom after feed bans
were enacted (27) remains uncertain but merits further consideration.

Indirect transmission and environmental persistence of prions will
complicate efforts to control CWD and perhaps other animal prion
diseases. Historically, control strategies for animal prion diseases
have focused on infected live animals as the primary source of
infection. Although live deer and elk represent the most plausible
mechanism for geographic spread of CWD, our data show that environmental
sources could contribute to maintaining and prolonging local epidemics,
even when all infected animals are eliminated. Moreover, the efficacy of
various culling strategies as control measures depends in part on the
rates at which the CWD agent is added to and lost from the environment.
Consequently, these dynamics and their implications for disease
management need to be more completely understood.

snip\...

http://www.cdc.gov/ncidod/EID/vol10no6/04-0010.htm

For Release: Wednesday, May 4, 2005

Contact: Michael Fraser (518) 402-8000

DEC Announces Sampling Results for Chronic Wasting Disease

The New York State Department of Environmental Conservation today
announced that it has received the remainder of test results for chronic
wasting disease (CWD) that were part of intensive sampling efforts in
central New York. DEC has received two positive results for the disease
out of 292 wild deer sampled.

The first positive result in a wild deer was announced on April 27, 2005
and came from a yearling white-tailed deer sampled from the Town of
Verona, Oneida County. The second positive result is from a 3-year-old
doe, located within a mile of the location where the initial positive
result was detected. The sample tissues were tested at the State\'s
Veterinary Diagnostic Laboratory at Cornell University. These are the
first known occurrences of CWD in wild deer in New York State.

DEC implemented intensive monitoring efforts after CWD was found in two
captive white-tailed deer herds in Oneida County -- the first incidents
of CWD in New York State. On April 8, 2005, the State Department of
Agriculture and Markets (DAM) completed testing of the captive deer and
found a total of five positive results for CWD in the two captive herds.

DEC, along with the U.S. Department of Agriculture\'s Wildlife Services
program, completed intensive monitoring on April 30, 2005. The effort
resulted in 290 samples of wild deer from Oneida County, two from
neighboring Madison County, and 25 wild deer from the Town of Arietta,
Hamilton County. Since 2002, DEC has conducted statewide sampling of
wild deer for CWD. When combined with sampling efforts in Oneida and
Hamilton Counties, DEC has collected more than 3,700 samples from wild
white-tailed deer.

DEC and DAM will continue public outreach to interested parties in
central New York to help educate citizens on CWD and to discuss next
steps to be taken. The agencies will hold a public meeting on Thursday,
May 12, 2005, at 7 p.m. in the Vernon-Verona-Sherrill High School
Auditorium, located on State Highway 31 in the Town of Verona. In
addition, DEC and DAM will conduct additional outreach and continue to
aggressively pursue inspection and enforcement across the State.

DAM continues to investigate, sample and test white-tailed deer from two
captive herds directly associated with the two herds that were confirmed
positive for CWD in Oneida County. Results for these sampling efforts
will be announced when available.

Statewide sampling for CWD - which has resulted in more than 1,000 tests
each year - will be increased to closely monitor the distribution and
prevalence of CWD in wild deer. In addition, DEC has implemented
emergency regulations regarding the handling, transport and management
of deer in the State. The emergency regulations are currently in effect
and represent an aggressive response to the recent discovery of chronic
wasting disease (CWD) in Oneida County.

DEC\'s emergency regulations are designed to ensure the proper handling
of deer and prevent further spread of CWD in the wild herd. The
emergency regulations are effective for 90 days. In addition, DEC will
begin the process of developing permanent regulations, which will appear
in the State Register and include a 45-day public comment period.

CWD is a transmissible disease that affects the brain and central
nervous system of certain deer and elk. There is no evidence that CWD is
linked to disease in humans or domestic livestock other than deer and
elk. More information on CWD can be found at DEC\'s website at
www.dec.state.ny.us/website/dfwmr/wildlife/deer/currentcwd.html

05-48

http://www.dec.state.ny.us/website/press/pressrel/2005/200548.html

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

Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518