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From: TSS ()
Subject: Pathobiology and diagnosis of animal transmissible spongiform encephalopathies: current knowledge, research gaps, and opportunities
Date: August 11, 2007 at 1:04 pm PST

Research Project: Transmission, Differentiation, and Pathobiology of Transmissible Spongiform Encephalopathies
Location: Virus and Prion Diseases of Livestock

Title: Pathobiology and diagnosis of animal transmissible spongiform encephalopathies: current knowledge, research gaps, and opportunities


Kehrli, Marcus
O`rourke, Katherine
Hamir, Amirali
Richt, Juergen
Nicholson, Eric
Silva, Christopher
Gay, Cyril

Submitted to: Government Publication/Report
Publication Type: Government Publication
Publication Acceptance Date: May 1, 2007
Publication Date: July 1, 2007

Citation: Kehrli, Jr., M.E., O'Rourke, K.I., Hamir, A.N., Richt, J.A., Nicholson, E.M., Silva, C.J., Edelman, D., Gay, C.G. 2007.

Pathobiology and diagnosis of animal transmissible spongiform encephalopathies: current knowledge, research gaps, and opportunities [government white paper]. Beltsville, MD: Interagency Working Group on Prion Science, Subcommittee on Pathobiology and Diagnostics. USDA, Agriculture Research Service. 33 p.

Technical Abstract:

Transmissible spongiform encephalopathies (TSEs) are fatal neurologic diseases that can affect several animal species and human beings. There are four animal TSE agents found in the United States: scrapie of sheep and goats; chronic wasting disease (CWD) of deer, elk, and moose; transmissible mink encephalopathy (TME) and bovine spongiform encephalopathy (BSE). Although the animal TSEs do not cause major death losses among US livestock populations, they are important because of international trade issues. The experience of the United Kingdom and Europe in dealing with the vast majority of the world's BSE cases, serves as a reminder of the need for continuing vigilance in monitoring risks for public health and research to answer remaining questions around the pathogenesis and transmission of these diseases. There remain questions on 1) cross-species transmissibility of TSEs in livestock and wildlife; 2) the pathobiology of TSEs in natural and secondary hosts; pathogenesis and transmission of CWD; and 4) pathogenesis and ante mortem detection of typical and atypical BSE. Our understanding of the pathogenesis and transmission of these diseases continues to evolve as ongoing, global TSE research efforts focus on defining tissue sites of abnormal prion accumulation, routes of infection, methods of strain differentiation, genetics of susceptibility and ante-mortem diagnostics. In this paper, a Subcommittee on Pathobiology and Diagnostics of TSEs for an Interagency Working Group on Prion Science summarizes the science of animal TSEs in order to identify knowledge gaps for the purpose of prioritizing animal prion research needs. Because of substantial losses involving international trade and potential risk for interspecies transmission to susceptible livestock and possibly humans, the presence of BSE, CWD, scrapie and TME in the United States presents a liability to U.S. domestic and alternative livestock industries. In addition, the proven risk of BSE to agriculture and public health from subclinical or clinically sick animals requires science-based surveillance for any silent, unrecognized epizootic expansions of these diseases in populations of animals that could either directly or indirectly affect food animals. CWD is an example of an uncontrolled expanding epidemic that threatens not only cervids but possibly other livestock. CWD also has elicited public health surveillance programs to monitor for scientific evidence of a prion disease in humans that consume venison. Therefore, some of the research needs are precautionary, but the risks to animal and human health from being caught unaware are high. Efforts are being made by both federal and state regulatory agencies to eradicate scrapie and CWD, and to determine the prevalence of BSE. The effectiveness of these programs will depend heavily on having accurate information about the nature of these diseases, not only in the original hosts, but also in other species that may be in contact with infected animals.

sporadic cjd

Colorado Surveillance Program for Chronic Wasting Disease
Transmission to Humans (TWO SUSPECT CASES)

An evaluation of scrapie surveillance in the United States
From: Terry S. Singeltary Sr.
Date: Sun, 5 Aug 2007 13:05

SEAC New forms of Bovine Spongiform Encephalopathy 1 August 2007
From: Terry S. Singeltary Sr.
Date: Sun, 5 Aug 2007 13:09:38 -0500

Owens, Julie
From: Terry S. Singeltary Sr. []
Sent: Monday, July 24, 2006 1:09 PM
To: FSIS RegulationsComments
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
Page 1 of 98

Greetings FSIS,

I would kindly like to comment on the following ;

[Federal Register: July 12, 2006 (Volume 71, Number 133)]
[Page 39282-39283]
From the Federal Register Online via GPO Access []
Food Safety and Inspection Service
[Docket No. FSIS-2006-0011]
Harvard Risk Assessment of Bovine Spongiform Encephalopathy (BSE)
Update; Notice of Availability and Technical Meeting


MY comments/questions are as follows ;

1. SINCE the first Harvard BSE Risk Assessment was so flawed and fraught with error after the PEER REVIEW
assessment assessed this fact, how do you plan on stopping this from happening again, will there be another peer
review with top TSE Scientist, an impartial jury so-to-speak, to assess this new and updated Harvard BSE/TSE risk
assessment and will this assessment include the Atypical TSE and SRM issues ?

*** Suppressed peer review of Harvard study October 31, 2002 ***

2. WITH A RECENT NATION WIDE MAD COW FEED BAN RECALL in the past few months that consisted of
some 10,878.06 TONS, then another Mad Cow feed ban warning letter in May, IT should seem prudent to ask why our
feed bans continue to fail in 2006, and continue to fail today ?


full text 98 pages ;

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

Terry S. Singeltary

Page 1 of 17

From: Terry S. Singeltary Sr. []

Sent: Thursday, September 08, 2005 6:17 PM


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

Greetings FSIS,

I would kindly like to submit the following to [Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified
Risk Materials for Human Food and Requirements for the Disposition of Non-Ambulatory Disabled Cattle
THE BSE/TSE SUB CLINICAL Non-Ambulatory Disabled Cattle Broken bones and such may be the first
signs of a sub clinical BSE/TSE Non-Ambulatory Disabled Cattle ;



Issued: Monday, 28 August 2000



A team of researchers led by Professor John Collinge at the Medical

Research Council Prion Unit1 report today in the Proceedings of the

National Academy of Sciences, on new evidence for the existence of a

"sub-clinical" form of BSE in mice which was unknown until now....

full text 17 pages ;

Docket No, 04-047-l Regulatory Identification No. (RIN) 091O-AF46 NEW BSE SAFEGUARDS


No. 04-047-l

No. 04-021ANPR

No. 2004N-0264


Federal Measures to Mitigate BSE Risks: Considerations for Further Action

Greetings FDA, USDA and APHIS et al,

I would kindly like to comment on the continued delay of the regulations
that have been proposed for years to reduce the risk of BSE/TSE in the
USA. Each day that is wasted debating this issue allows this agent to spread,
and many many more humans and animals become needlessly exposed to
this agent via a multitude of potential routes and sources right here in the
USA. TO continue to ignore the new findings from several scientists
about the fact that BSE is not the only strain of TSE in cattle, the fact that
new atypical strains of TSE are showing up in not only cattle, but
sheep and the fact that the new strain of TSE in cattle seems to be
more similar to sporadic CJD as opposed to the nv/v CJD, to continue
to ignore these findings will only further spread this agent. ..............

full text ;

Docket Management Docket: 02N-0273 - Substances Prohibited From Use in

Animal Food or Feed; Animal Proteins Prohibited in Ruminant Feed

Comment Number: EC -10

Accepted - Volume 2


Guidance for Industry: Use of Material From Deer and Elk In Animal Feed

Terry S. Singeltary Sr.
Vol #:

Docket Management Docket: 02N-0276 - Bioterrorism Preparedness ...
General Comments, Subject: Docket No: 02-088-1 RE-Agricultural ...
From: Terry S. Singeltary Sr.


Docket No: 02-088-1 Title: ...

Greetings FDA and public,

if you go to the below site, and search all BSE known countries and check out their air traffic illegal meat they have confiscated, and check out the low number checked, compared to actual passenger traffic, would not take too much for some nut to bring in FMD/TSEs into the USA as a 'suitcase bomb'.

[[Under APHIS-PPQ's agricultural quarantine inspection monitoring, 284 air passengers from Israel were sampled for items of agricultural interest in fiscal year 2001. Seven of these passengers, or 2 percent, carried a total of 11 kg of meat items that could potentially harbor the pathogen that causes BSE. None of these passengers from whom meat items were confiscated reported plans to visit or work on a ranch or farm during their visit to the U.S.]]

if they were to have questioned the terrorist that bombed the Twin Towers with jets, if they were to have questioned them at flight school in the USA, i am sure that they would have said they did not intend to visit the Twin Towers as a flying bomb either. what am i thinking, they probably did ask this? stupid me. ...

full text ;


File Format: PDF/Adobe Acrobat -

Page 1. J Freas, William From: Sent: To: Subject: Terry S. Singeltary

Sr. [] Monday, January 08, 200l 3:03 PM freas ...

Greetings again Dr. Freas and Committee Members,

I wish to submit the following information to the
Scientific Advisors and Consultants Staff
2001 Advisory Committee (short version).

I understand the reason of having to shorten my submission,
but only hope that you add it to a copy of the long version,
for members to take and read at their pleasure,
(if cost is problem, bill me, address below).
So when they realize some time in the near future
of the 'real' risks i speak of from human/animal TSEs and
blood/surgical products. I cannot explain the 'real' risk
of this in 5 or 10 minutes at some meeting,
or on 2 or 3 pages, but will attempt here:

remember AIDS/HIV, 'no problem to heterosexuals in the U.S.?
no need to go into that, you know of this blunder.

DO NOT make these same stupid mistakes again with
human/animal TSE's aka MADCOW DISEASE. I lost my Mom to hvCJD,
and my neighbor lost his Mother to sCJD as well (both cases
confirmed). I have seen many deaths, from many diseases.
I have never seen anything as CJD, I still see my Mom laying helpless,
jerking tremendously, and screaming "God, what's wrong
with me, why can't I stop this". I still see this, and will
never forget. Approximately 10 weeks from 1st of symptoms to death.
This is what drives me. I have learned more in 3 years about not only
human/animal TSE's but the cattle/rendering/feeding industry/government
than i ever wished to.

I think you are all aware of CJD vs vCJD, but i don't think
you all know the facts of human/animal TSE's as a whole,
they are all very very similar, and are all tied to the
same thing, GREED and MAN.

I am beginning to think that the endless attempt to track
down and ban, potential victims from known BSE Countries
from giving blood will be futile. You would have to ban
everyone on the Globe eventually? AS well, I think we
MUST ACT SWIFTLY to find blood test for TSE's,
whether it be blood test, urine test, eyelid test,
anything at whatever cost, we need a test FAST.

DO NOT let the incubation time period of these TSEs fool you.............

full text 6 pages ;

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

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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





MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam


Answering critics like Terry Singeltary, who feels that the U.S. under-
counts CJD, Schonberger conceded that the current surveillance system
has errors but stated that most of the errors will be confined to the older

Copyright © 2003 Published by Elsevier Ltd.

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.

Volume 3, Issue 8, August 2003, Page 463

“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD)
and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD
in deer and elk is a small portion of a much bigger problem.”


see history of cjd questionnaire

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

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