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From: TSS (
Date: October 11, 2004 at 7:51 am PST

-------- Original Message --------
Date: Mon, 11 Oct 2004 09:51:44 -0500
From: "Terry S. Singeltary Sr."
To: Bovine Spongiform Encephalopathy

Investigation into testing failures report published
Monday, 11 October 2004
The findings of an independent inquiry into the failure of the Meat
Hygiene Service (MHS), an Executive Agency of the Food Standards Agency
(FSA), to BSE test an estimated 261 casualty cattle are published today.
The inquiry was initiated by the FSA Board in June, following an MHS
audit that uncovered a number of failures to test casualty cattle1 aged
24-30 months.

The independent inquiry2 concluded that, on the basis of existing advice
from the Government's Spongiform Encephalopathy Advisory Committee
(SEAC)3, the risk to human health from all casualty cattle entering the
food chain was very low. This is because there have been no confirmed
cases of BSE in cattle under 30 months in the UK since 1996, there have
been no BSE positives detected in the more than 2,800 casualty cattle
aged 24-30 months tested to date, and that Specified Risk Material (SRM)
controls (which remove more than 99% of any infectivity that may be
present) are applied.

A steering group, led by Professor Patrick Wall, Professor of Food
Safety at University College Dublin and former Chief Executive of the
Food Safety Authority of Ireland, was set up to oversee the inquiry. Its
report and recommendations will be considered by the Board of the FSA at
its open meeting this Thursday, after which an action plan will be
developed for further consideration by the Agency's Board at its meeting
in December.

Professor Wall said:

'We have concluded that there were a number of reasons for the testing
failures and that all organisations involved – FSA, Defra, MHS and their
contractors – contributed to one degree or another. It is our view that
these failures occurred principally because the requirements and the
objectives of testing were not clearly agreed nor communicated
effectively and not properly monitored.

'The Steering Group considers that these failures are in contrast to the
MHS delivery of the requirement to remove specified risk material (SRM)
– the main BSE control. Here the instructions were clear and
unambiguous, with the MHS giving the requirement high priority,
providing good training and supervision, and rigorous monitoring.

'I would also stress that during our investigations we did not uncover
any problems that cannot be remedied without too much difficulty. I hope
that our recommendations will be considered carefully by all the parties
involved and would like to thank the members of the independent group
for their contributions.'

Sir John Krebs, Chairman of the FSA, said:

'When these failures were first reported the Food Standards Agency
ordered the MHS to take immediate action to ensure that BSE testing of
casualty cattle was being applied. While it is reassuring that the risks
from these failings is very low, the Agency expects the proper
implementation of all BSE controls and surveillance measures.

'I am very grateful to Professor Wall and his colleagues for their
report and such a thorough independent inquiry. The Board will consider
their findings very carefully and commission an action plan that
addresses these recommendations in a robust and thorough way.'

Along with Professor Wall, the independent Steering Group also included
former president of the British Veterinary Association and member of
SEAC, Peter Jinman, as well as Barbara Saunders, consumer affairs
consultant and former member of the FSA's Food Advisory Committee. The
investigation, overseen by the Steering Group, was carried out on their
behalf by expert auditors PKF. Its investigation involved a
comprehensive review of the relevant arrangements within MHS, FSA and
DEFRA, along with visits to plants and interviews and surveys of
contractors and Official Veterinary Surgeons (OVSs).

Notes to editors
(1) Casualty cattle are animals subject to special emergency slaughter,
because of an accident or other serious condition, or because they show
signs of any disease, injury or abnormality at ante mortem inspection.

(2) The Inquiry was set up by the FSA to find out the reasons for the
failings by the MHS and why they were not identified earlier; as well as
to make recommendations to improve the robustness of the testing
arrangements. It was supported in its work by investigations undertaken
by independent auditors PKF.

(3) SEAC – the Spongiform Encephalopathy Advisory Committee's role is to
provide independent expert scientific advice to the Government on
spongiform encephalopathies such as BSE, CJD and scrapie. Its remit is
wide-ranging, and covers public health, food safety and animal health

Contact: Shaun Whelan at FSA Press Office on 020 7276 8888

* Inquiry into the failure to comply with the requirements to test all
relevant 24-30 month old casualty animals for BSE

Read the full report (269 kb)


* Inquiry into the failure to comply with the requirements to test all
relevant 24-30 month old casualty animals for BSE


The scale of the problem
5. Following the reporting by the MHS in May 2004 of four incidents
where cattle were
not tested in accordance with instructions set out in the MHS Operations
Manual, the
MHS senior management instigated a full investigation to determine if
other cattle
should have been tested. The investigation revealed that in GB there
were 128
confirmed cases (representing 6.2% of recorded tests shown to be
required by MHS
records) that had not been tested according to the instructions and 133
further cases.


Reasons for the failures
18. The Steering Group has considered the findings of the PKF
investigation and
concludes that there were a number of reasons for the GB testing
failures and that all
organisations involved – FSA, Defra, MHS, and their Contractors
contributed to the
failure to one degree or another.
19. It is the view of the Group that the failures occurred principally
because the
requirements and the objectives of testing were not clearly agreed nor
effectively, and not properly monitored by Contractors or the MHS.
20. Where the cattle are known or suspected to be diseased or injured,
the farmer is
required to prepare a Schedule 18 Certificate to accompany the animal to
This is to give adequate warning of the animal’s condition to the
operator. Sixty-three per cent of the failures were accompanied by
Schedule 18
Declarations that should have been seen by the OVSs and led to testing.
21. Where cattle are killed on farm, the farmer is required to ensure
that a Schedule 19
Certificate is signed by a veterinary surgeon and accompanies the
carcase to a
slaughterhouse. Nine of the failures concerned were animals slaughtered
on the farm.
The Group consider that there can be no excuse for Schedule 18 and 19
animals not
having been tested.
22. The Group has concluded that there were a number of factors that led
to a systems
failure of the testing requirement. These were:
• The lack of specific measures of technical performance or standards in the
Defra/MHS Service Level Agreement (SLA).
• That the instruction was not communicated effectively. The instruction was
changed on five occasions and the most significant change, which removed
flexibility in interpretation of the instruction by withdrawing the
right of the
OVS to exercise professional judgement, was not flagged as a key change. The
arrangements for providing guidance to OVSs were inconsistent and did not
recognise the importance of the instruction.
• That where OVSs sought further guidance the response was often
inconsistent and
this contributed to variations in the implementation of the instruction.
• That the instruction was not practical to implement in all
circumstances. The
instruction required, in its final form, the detection and testing of
all 24–30 month
old cattle that during ante-mortem inspection are identified as showing
signs of any
disease, injury or abnormality, no matter how insignificant. This
appears to exceed
the requirement of the EC Regulation.
• That there was an inadequate supervisory structure for OVSs, who often
monitoring and support and consequently were often isolated. Some of the
involved, particularly those trained abroad, were clinically
inexperienced and not
familiar with GB slaughterhouse practices. They needed a higher level of
guidance and support than was available whether from the MHS (in the case of
directly employed OVSs), or from their employer (where this was a
• That there was insufficient monitoring of performance against the
requirements of
the SLA by either Defra or by the MHS, with no agreed performance
indicators to
establish the quality of the service to be provided. The SLA did not
identify a key
performance indicator against which the standards of testing being
delivered could
be checked.
• That there was insufficient training of OVSs by the Contractors in the
case of OVSs
supplied by them or the MHS in the case of directly employed OVSs.
• That there was no formal cross-checking arrangements between the
results of
ante-mortem and post-mortem inspections, as identified by PKF. The
examination of all animals could have acted as a means of picking up animals
which should have been identified for testing when inspected in the
lairage, but
which were missed.
• That there was no means by which an OVS would know when a Schedule 18 had
been submitted to the plant operator but not passed on to them.


Causes of testing omissions
Specifically the testing omissions occurred as a result of OVSs not
identifying all relevant
animals for testing, either by the inappropriate exercise of
professional judgement or by
matters not spotted at the ante-mortem stage.
The failures to test arose as a result of a combination of the following
• The requirement was seen by OVSs, MHS, FSA and Defra as being
primarily for
disease surveillance and not food safety. This meant that it was
perceived as a
lower priority for ensuring compliance and the arrangements for
implementing it
were designed accordingly.
• There was a lack of clarity as to what was required. The instruction
was changed
on 5 occasions and the most significant change, which removed professional
judgement, was flagged as a reminder rather than a key change. The
for communicating the instruction were ineffective and the arrangements for
providing guidance to OVSs were inconsistent and did not recognise the
importance of the instruction.
• The final version of the instruction conflicted with OVS views as to
what reflected
a BSE risk and implied a detail of ante-mortem inspection that was much
than previously carried out in order to identify sign of any disease,
injury or
abnormality – this was not practical at the larger plants as the design and
operational arrangements limit the visibility of individual animals and the
throughput demands limit the time available.
• There was inadequate supervision and monitoring of OVS records or
activity to
identify that the instruction had not been met. When the POVS role was
the level of alternative supervision was not increased and this remains
an issue.
• There is no cross check between the results of the ante-mortem and
inspections. This could have identified further animals with injuries and
abnormalities that were not being identified at the ante-mortem stage.
The lack of
a formal check was exacerbated by the OVS and MHIs not working as a team.
• The Service Level Agreement did not identify testing activity as a key
indicator. MHS monitoring was driven by the key performance measures.
• There was no expectation of testing levels by MHS or Defra and
therefore no
mechanism to identify that tests were being missed.
• Many of the OVSs involved were inexperienced and in need of a higher
level of
training, support and guidance than was available. In some cases this
may have
been exacerbated by unfamiliarity with the GB system and language
The designation training for OVSs is considered to be insufficient by MHS,
Contractors and OVSs and many also feel that subsequent training is not
enough to
assist them to carry out their role.
Delay in identifying testing omissions
Identification of the failures was delayed as a result of a lack of
effective technical
supervision and monitoring by Contractors and MHS. Initially reliance
was placed on the
POVS, but this was ineffective as POVSs did not understand the
requirements themselves
and varied in their effectiveness as supervisors. Since the removal of
the POVS role in 2002,
responsibility for supervision has been delegated to Contractors without
technical MHS
check. Again the structure has failed on this issue as the Contractors
did not understand the
FSA and Defra relied on FSA audit of the application of the Manual by
the MHS. However
this particular testing requirement was not included in the audit work
specified or carried
out as it was not identified as a high priority for check.
Records of ante-mortem and testing activity are paper based and held
locally. They do not
facilitate easy checking of completeness of tests nor easy analysis of
data. Reporting within
MHS has therefore tended to focus on exceptions rather than giving
fuller information. The
matter was eventually identified when MHS introduced its own internal
audit function. The
testing failures identified are likely to have been only part of those
missed as OVSs
interviewed indicated that they do not always record minor injuries on
the ante-mortem
The Key Performance Indicators (KPIs) focus upon administrative matters
and there was no
effective measurement or monitoring of technical standards, or of
technical performance.
Measurement of such qualitative matters is limited – the performance of
MHS was not
monitored by Defra, the Contractors performance was not monitored by MHS.
Current areas of risk of BSE testing omissions
Action taken since the discovery of the failures has heightened
awareness of the issue and
improved the specific skills, thereby reducing the likelihood of errors
on Schedule 18 and 19
cattle. However, human error will mean that without an effective check
mechanism some
missed tests may still arise. Of more concern is the continuing
vulnerability to delayed or
lost Schedule 18 certificates and the difficulty for the ante-mortem
inspection process in
identifying all the animals meeting the requirement for testing.
The GB testing requirement appears to exceed that required by the EU
regulation, as the last
change to it was introduced in order to increase the numbers tested
rather than to meet a
technical requirement of the regulation. It also appears to be
impractical to achieve at some
plants without increasing the resource available to carry out the
ante-mortem inspection.
This needs to be considered in the arrangements going forward.





July 13, 2004

IG Audit Finds Multiple Flaws in Mad Cow Surveillance Plan
Rep. Waxman raises questions about the effectiveness and credibility of
USDA's response to mad cow disease, citing an audit by the USDA
Inspector General that finds systemic deficiencies in the Department's
surveillance plan and new evidence that USDA misled the public in the
wake of the detection of an infected cow in Washington State.

- Letter to USDA

IG Draft Audit

May 13, 2004

Failure To Test Staggering Cow May Reflect Wider Problems
Rep. Waxman raises concerns that the recent failure of USDA to test an
impaired cow for BSE may not be an isolated incident, citing the failure
of USDA to monitor whether cows condemned for central nervous system
symptoms are actually tested for mad cow disease.

- Letter to USDA



No mad cow results for nearly 500 cows

By Steve Mitchell
United Press International
Published 8/11/2004 11:23 AM

WASHINGTON, Aug. 11 (UPI) -- The U.S. Department of Agriculture failed
to test for mad cow disease or collect the correct portion of the brain
on nearly 500 suspect cows over the past two years -- including some in
categories considered most likely to be infected -- according to agency
records obtained by United Press International.

The testing problems mean it may never be known with certainty whether
these animals were infected with the deadly disease. Department
officials said these animals were not included in the agency's final
tally of mad cow tests, but the records, obtained by UPI under the
Freedom of Information Act, indicate at least some of them were counted...



Steve Mitchell is UPI's Medical Correspondent. E-mail
Copyright © 2001-2004 United Press International

From: Terry S. Singeltary Sr. []
Sent: Tuesday, July 29, 2003 1:03 PM
Cc:;; BSE-L
Subject: Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION
TO DOCKET 2003N-0312]

Greetings FDA,


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

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)

* 167 kB Report
* 105 kB 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.

IMPORTS FROM CANADA [takes a few minutes to load]

Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION]

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



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 ...

Asante/Collinge et al, 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;

Docket Management Docket: 96N-0417 - Current Good Manufacturing Practice
in Manufacturing, Packing, or Holding Dietary Ingredients a
Comment Number: EC -2
Accepted - Volume 7

[PDF] Appendices to PL107-9 Inter-agency Working Group Final Report 1-1
File Format: PDF/Adobe Acrobat - View as HTML
Agent, Weapons of Mass Destruction Operations Unit Federal Bureau of
those who provided comments in response to Docket No. ...
Meager 8/18/01 Terry S. Singeltary Sr ...

Docket No. 2003N-0312 Animal Feed Safety System [TSS SUBMISSION
TO DOCKET 2003N-0312]

# Docket No: 02-088-1 RE-Agricultural Bioterrorism Protection Act of
TSS 1/27/03 (0)

Docket Management

Docket: 02N-0276 - Bioterrorism Preparedness; Registration of Food Facilities, Section 305
Comment Number: EC-254 [TSS SUBMISSION]

Dockets Entered On October 2, 2003 Table of Contents, Docket #,
Title, 1978N-0301,

OTC External Analgesic Drug Products, ... EMC 7, Terry S. Singeltary Sr.
Vol #: 1, ...

Daily Dockets Entered on 02/05/03

DOCKETS ENTERED on 2/5/03. ... EMC 4 Terry S. Singeltary Sr. Vol#: 2.
... Vol#: 1.

03N-0009 Federal Preemption of State & Local Medical Device Requireme. ...

Docket Management

Docket: 02N-0370 - Neurological Devices; Classification of Human Dura Mater

Comment Number: EC -1

Accepted - Volume 1

Daily Dockets - 04/10/03
... 00D-1662 Use of Xenotransplantation Products in Humans.
EMC 98 Terry S. Singeltary Sr. Vol#: 3. 01F ... - 05-20-2003 - Cached

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

Terry S. Singeltary Sr.
Vol #:

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

Terry S. Singeltary Sr.
Vol #:

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

Terry S. Singeltary Sr.
Vol #:

01N-0423 Substances Prohibited from use in animal food/Feed Ruminant

APE 5 National Renderers Association, Inc. Vol#: 2

APE 6 Animal Protein Producers Industry Vol#: 2

APE 7 Darling International Inc. Vol#: 2

EMC 1 Terry S. Singeltary Sr. Vol#: 3

Send Post-Publication Peer Review to journal:

Re: 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?


Volume 3, Number 8 01 August 2003


Tracking spongiform encephalopathies in North America

Xavier Bosch

My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost

my mom to hvCJD (Heidenhain variant CJD) and have been searching for

answers ever since. What I have found is that we have not been told the

truth. CWD in deer and elk is a small portion of a much bigger problem.

49-year-old Singeltary is one of a number of people who have remained

largely unsatisfied after being told that a close relative died from a

rapidly progressive dementia compatible with spontaneous

Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of

documents on transmissible spongiform encephalopathies (TSE) and

realised that if Britons could get variant CJD from bovine spongiform

encephalopathy (BSE), Americans might get a similar disorder from

chronic wasting disease (CWD)the relative of mad cow disease seen among

deer and elk in the USA. Although his feverish search did not lead him

to the smoking gun linking CWD to a similar disease in North American

people, it did uncover a largely disappointing situation.

Singeltary was greatly demoralised at the few attempts to monitor the

occurrence of CJD and CWD in the USA. Only a few states have made CJD

reportable. Human and animal TSEs should be reportable nationwide and

internationally, he complained in a letter to the Journal of the

American Medical Association (JAMA 2003; 285: 733). I hope that the CDC

does not continue to expect us to still believe that the 85% plus of all

CJD cases which are sporadic are all spontaneous, without route or source.

Until recently, CWD was thought to be confined to the wild in a small

region in Colorado. But since early 2002, it has been reported in other

areas, including Wisconsin, South Dakota, and the Canadian province of

Saskatchewan. Indeed, the occurrence of CWD in states that were not

endemic previously increased concern about a widespread outbreak and

possible transmission to people and cattle.

To date, experimental studies have proven that the CWD agent can be

transmitted to cattle by intracerebral inoculation and that it can cross

the mucous membranes of the digestive tract to initiate infection in

lymphoid tissue before invasion of the central nervous system. Yet the

plausibility of CWD spreading to people has remained elusive.

Part of the problem seems to stem from the US surveillance system. CJD

is only reported in those areas known to be endemic foci of CWD.

Moreover, US authorities have been criticised for not having performed

enough prionic tests in farm deer and elk.

Although in November last year the US Food and Drug Administration

issued a directive to state public-health and agriculture officials

prohibiting material from CWD-positive animals from being used as an

ingredient in feed for any animal species, epidemiological control and

research in the USA has been quite different from the situation in the

UK and Europe regarding BSE.

Getting data on TSEs in the USA from the government is like pulling

teeth, Singeltary argues. You get it when they want you to have it,

and only what they want you to have.

Norman Foster, director of the Cognitive Disorders Clinic at the

University of Michigan (Ann Arbor, MI, USA), says that current

surveillance of prion disease in people in the USA is inadequate to

detect whether CWD is occurring in human beings; adding that, the

cases that we know about are reassuring, because they do not suggest the

appearance of a new variant of CJD in the USA or atypical features in

patients that might be exposed to CWD. However, until we establish a

system that identifies and analyses a high proportion of suspected prion

disease cases we will not know for sure. The USA should develop a

system modelled on that established in the UK, he points out.

Ali Samii, a neurologist at Seattle VA Medical Center who recently

reported the cases of three hunterstwo of whom were friendswho died

from pathologically confirmed CJD, says that at present there are

insufficient data to claim transmission of CWD into humans; adding that

[only] by asking [the questions of venison consumption and deer/elk

hunting] in every case can we collect suspect cases and look into the

plausibility of transmission further. Samii argues that by making both

doctors and hunters more aware of the possibility of prions spreading

through eating venison, doctors treating hunters with dementia can

consider a possible prion disease, and doctors treating CJD patients

will know to ask whether they ate venison.

CDC spokesman Ermias Belay says that the CDC will not be investigating

the [Samii] cases because there is no evidence that the men ate

CWD-infected meat. He notes that although the likelihood of CWD

jumping the species barrier to infect humans cannot be ruled out 100%

and that [we] cannot be 100% sure that CWD does not exist in humans&

the data seeking evidence of CWD transmission to humans have been very


he complained in a letter to the Journal of the American Medical

Association (JAMA 2003; 285: 733). I hope that the CDC does not

continue to expect us to still believe that the 85% plus of all CJD

cases which are sporadic are all spontaneous, without route or source.<<<

actually, that quote was from a more recent article in the Journal of

Neurology (see below), not the JAMA article...

Full Text

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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






BY Philip Yam

Yam Philip Yam News Editor Scientific American

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:

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: (until 9/12/02)

New e-mail: (active from now)



full text ;

AND the new findings of BASE in cattle in Italy of Identification of a
second bovine amyloidotic spongiform encephalopathy: Molecular
similarities with sporadic

Creutzfeldt-Jakob disease

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

THE findings from Corinne Ida Lasmézas*, [dagger] , Jean-Guy Fournier*,
Virginie Nouvel*,

Hermann Boe*, Domíníque Marcé*, François Lamoury*, Nicolas Kopp [Dagger

] , Jean-Jacques Hauw§, James Ironside¶, Moira Bruce [||] , Dominique

Dormont*, and Jean-Philippe Deslys* et al, that 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;

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

ALL human TSEs must be made reportable Nationally and Internationally...



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