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From: TSS (
Subject: Re: TSE ADVISORY COMMITTEE; ACTION: Notice. [TSS SUBMISSION FR Doc. 04-21282 AND FR Doc. 04-21283
Date: September 22, 2004 at 1:31 pm PST

In Reply to: TSE ADVISORY COMMITTEE; ACTION: Notice. posted by TSS on September 22, 2004 at 7:08 am:

-------- Original Message --------
Subject: Transmissible Spongiform Encephalopathies Advisory Committee; [FR Doc. 04-21282 Filed 9-21-04; 8:45 am] TSS SUBMISSION
Date: Wed, 22 Sep 2004 15:31:06 -0500
From: "Terry S. Singeltary Sr."


Transmissible Spongiform Encephalopathies Advisory Committee;
[FR Doc. 04-21282 Filed 9-21-04; 8:45 am]

Greetings Dr. Freas, Dr. Langford and all FDA,


-------- Original Message --------

Subject: Blood Products Advisory Committee [FR Doc. 04-21283 Filed 9-21-04; 8:45 am] TSS SUBMISSION
Date: Wed, 22 Sep 2004 15:31:55 -0500
From: "Terry S. Singeltary Sr."


Blood Products Advisory Committee
[FR Doc. 04-21283 Filed 9-21-04; 8:45 am]

Greetings Dr. Smallwood and all FDA,

I kindly wish to submit the following information and
comments to the Blood Products Advisory Committee.

I kindly wish to submit the following information and
comments to the TSE Advisory Committee on blood products.

IF you remember correctly, in my submission of;

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 ...


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.


NOW, some 3+ years later, seems we are faced with just this
nightmare scenario. Seems we have floundered too long, but my
fear is that we are still missing the bigger picture, sporadic CJD.
I have made several submissions to the FDA about my concern
with the transmission of the TSE agent from sporadic CJD via
the medical surgical arena and tissue transplantation;

Docket Management

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

Comment Number: EC -1
Accepted - Volume 1

I WISH to make the following submission and comments about all
topics of this meeting about human/animal TSEs. My comments and
submissions as follows please;

>USDA-licensed tests for the diagnosis of bovine
>spongiform encephalopathy (BSE) and other transmissible spongiform
>encephalopathies (TSE),
IN the past all we have heard is the fact that the present BSEs test do not
guarantee public health safety. I request that the USA implement a
BSE/TSE test that DOES guarantee public health safety, one approved
for consumer protection, a test for live cattle, one that would detect all
TSEs in the bovine, one that would detect sub clinical TSEs;

EFSA Scientific Report on the Design of a Field Trial Protocol for the
Evaluation of BSE Tests for Live Cattle

Publication date: 17 September 2004

Adopted on 1 July 2004 (Question N° EFSA-Q-2003-084)

* 146 kB Report

* 90 kB Summary

Summary of the Report

The European Food Safety Authority and its Scientific Expert Working
Group on Transmissible Spongiform Encephalopathy (TSE) Testing were
asked by the European Commission (EC) to take over the mandate of the
former Scientific Steering Committee (SSC) for the scientific evaluation
of rapid TSE/BSE (Bovine Spongiform Encephalopathy) tests. At present 5
rapid BSE test kits are approved by the EC for the routine post mortem
testing of slaughtered cattle over 30 months of age in accordance with
the TSE Regulation (EC) No 999/2001. Following a call for expression of
interest in the Official Journal of the European Union (No C15) on 22
January 2003, several parties indicated their interest in participating
in an EC evaluation exercise of their newly developed rapid BSE post
mortem and live animal tests.

It has been recognized that the availability of a rapid test for live
cattle would be a major advance in dealing with the problem of BSE and
TSE in general, but particularly with regard to epidemiological
screening. In the long term an accurate live animal test might offer the
possibility to reduce the number of culled animals after the detection
of one positive animal.

A rapid BSE test for live cattle could be approved for the purpose of
consumer protection, for epidemiological screening or for both. For the
purpose of consumer protection any new rapid BSE test including tests
for live animals should not be statistically inferior to that of the
currently approved post mortem tests.

This report provides a protocol for the design of a field trial protocol
for the evaluation of BSE tests for live cattle for the purpose of
consumer protection only.


>review of the worldwide BSE situation,

THE most disturbing factor of this topic are the new atypical TSEs
showing up in not only cattle, but also sheep, and no one know's yet
about how many different strains of cwdTSE in deer/elk. WITH evidence of
sporadic CJD being very similar to these atypical TSEs in cattle and sheep,
and the findings from Asante, Collinge et al that BSE prions propagate
as either nvCJD or sporadic CJD, the ramifications of these findings are
very worrysome and should not go ignored any further. WITH
the fact that there are over 20 documented strains of scrapie, and the
most logical hypothisis is scrapie to BSE, why would one believe in
only one phenotype of TSE in the bovine;

BSE prions propagate as either variant CJD-like or sporadic CJD-like
prion strains in transgenic mice expressing human prion protein

Emmanuel A. Asante, Jacqueline M. Linehan, Melanie Desbruslais, Susan
Joiner, Ian Gowland, Andrew L. Wood, Julie Welch, Andrew F. Hill, Sarah
E. Lloyd, Jonathan D.F. Wadsworth and John Collinge1

MRC Prion Unit and Department of Neurodegenerative Disease, Institute of
Neurology, University College, Queen Square, London WC1N 3BG, UK 1
Corresponding author e-mail:

Received August 1, 2002; revised September 24, 2002; accepted October
17, 2002


Variant CreutzfeldtJakob disease (vCJD) has been recognized to date
only in individuals homozygous for methionine at PRNP codon 129. Here we
show that transgenic mice expressing human PrP methionine 129,
inoculated with either bovine spongiform encephalopathy (BSE) or variant
CJD prions, may develop the neuropathological and molecular phenotype of
vCJD, consistent with these diseases being caused by the same prion
strain. Surprisingly, however, BSE transmission to these transgenic
mice, in addition to producing a vCJD-like phenotype, can also result in
a distinct molecular phenotype that is indistinguishable from that of
sporadic CJD with PrPSc type 2. These data suggest that more than one
BSE-derived prion strain might infect humans; it is therefore possible
that some patients with a phenotype consistent with sporadic CJD may
have a disease arising from BSE exposure...

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

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

John Collinge

MRC Prion Unit and Department of Neurodegenerative Disease, Institute of
University College, London WC1N 3BG, UK
Received 9 December 2003
Accepted 27 April 2004

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 CreutzfeldtJakob 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.

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;

THE recent discoveries of previously unidentified strains of
Scrapie such as 221C44 and the Nor9845;

2.5.2 The existence of distinct strains was recognised when sources of
sheep scrapie were serially transmitted to mice. A single inbred line of
mice, in which all
individuals make the same PrPC, can propagate several different strains
of scrapie, each with
its own distinctive incubation period, pattern of damage in the brain
and PrPSc
properties42, 43. Strain variation also occurs in natural scrapie, and
the recent discoveries of
previously unidentified strains, such as 221C44 and Nor9845, suggests
that the
spectrum of different strains may change with time. Since different
strains can have very
different patterns of pathology and distribution of PrPSc, their
presence may be missed by the
application of standard sampling and testing protocols used for
Although a single strain of BSE appears to be responsible for the vast
majority of cases around the
world46, 47, evidence is emerging that suggests that there may be other
strains which
have different properties when transmitted to mice48 or result in
different pathology
or properties of PrPSc in infected cattle49, 50.


UK Strategy for Research and
Development on Human and Animal
Health Aspects of Transmissible
Spongiform Encephalopathies


WHEN in fact, the findings from Marsh and the findings at
MISSION, TEXAS support even further evidence that there
are furthers strains of TSE in the USA besides that one
accidently documented BSE case in Washington on
Dec. 23, 2003;

In Confidence - Perceptions of unconventional slow virus diseases of
animals in the USA - Report of a visit to the USA - April-May 1989 - G A H Wells
[head of England's main veterinary lab]

2. Meeting with USDA, BSE Task Force

This group comprises Alex Thierman (USDA-APHIS, International Programs)
(Chairman), Roger Breeze (USDA-ARS Director, Plum Island), Bill Hadlow
(Neuropathologist - retired, formerly of NIH Rocky Mountain Laboratory,
Hamilton, Montana), John Gorhan and Mark Robinson (USDA-ARS, Pullman,
Washington) and Dick Marsh (Dept. Vet. Science, Univ Wisconsin -

The objectives of the group are to assess the implications of
the occurrence of BSE for US cattle particularly the risk
of BSE occurrence in the US in relation to endemic scrapie agent.
The purpose of my invitation to this meeting was to discuss aspects of
research which are of common interest and to identify a tentative USDA
research programme including any potential collaborative projects. The
discussions were informal and there was no agenda.

The general opinion
of those present was that BSE, as an overt disease phenomenon, could
exist in the USA but if it did it was very rare. The need for improved
and specific surveillance methods to detect it was recognised. Clinical
similarities between BSE and rabies suggested one means of sampling the
cattle population which might increase the probability of detection of
BSE. It was clear that the bovine rabies negative rate would vary
greatly between States but initially this should be determined and as it
would inevitably be high relative to positive cases, some differential
diagnosis carried out.

The work of Wilbur Clarke at Mission, Texas was discussed briefly. the
results of this study in which 10 calves were inoculated with scrapie
has not been published and perhaps would not be published. USDA are
sensitive regarding publicity of the results of the study which remain
far from conclusive. Apparently only 3 of the inoculated animals
developed neurological signs. The neuropathology of the affected cattle
has not been examined in any depth but Hadlow has the material. Marsh
indicated the requirement to obtain the fresh brain material from this
study in order to perform PrP extractions.

Because of the successful transmission of the Brecke (Stetsonville)
isolate of TME to cattle and the subsequent passage history in mink it
was generally considered important that comparisons be made with BSE
isolates in mink. Is BSE like scrapie in mink? Is BSE like the Brecke
isolate of TME?

Very little was said about CWD but some present considered that its
occurrence may indicate a sylvatic origin of agent. It was also agreed
that the role of possible subclinical infection in the
epidemiology of transmissible spongiform encephalopathies could well
be important but was unknown.

Marsh remarked on the possibility that
BSE was due to an extremely thermostable strain of agent. His
experience in the past with one particular Wisconsin isolate of TME
(Hayward strain) suggested that i/c biopsy needles could not be
effectively "scrapie sterilised", even employing an experimental
autoclave system capable of 60 psi and 300"C+ for 5 hours. This
experience led him to the policy that in scrapie or TME transmission
studies re-use of instruments or glassware that had contained agent was
an unacceptable protocol.

I was given confidential access to sections from the Clarke scrapie-
cattle transmission experiment. Details of the experimental design were
as supplied previously by Dr Wrathall (copy of relevant information
appended). Only 3 animals (2 inoculated with 2nd pass Suffolk Scrapie
and 1 inoculated with Angora goat passaged scrapie) show clinical signs.

Clinical signs were characterised by weakness,
"a stilted hindlimb gait", disorientation, ataxia and, terminally,
lateral recumbency. The two cattle from which I examined material were
inoculated at 8 months of age and developed signs 36 months pi (goat
scrapie inoculum) and 49 months pi (one of the Suffolk scrapie
inoculated) respectively.

This latter animal was killed at 58 months
of age and so the clinical duration was only 1 month. The neuropathology
was somewhat different from BSE or the Stetsonville TME in cattle.
Vacuolar changes were minimal, to the extent that detection required
careful searching. Conversely astrocyte hypertrophy was a widespread and
prominent feature. The material requires detailed neuropathological
assessment but whether or not this will be done remains in question.

Appendix I


1. Dr Clark lately of the Scrapie Research Unit, Mission Texas has
successfully transmitted ovine and caprine scrapie to cattle. The
experimental results have not been published but there are plans to do
this. This work was initiated in 1978. A summary of it is:-

Expt A 6 Her x Jer calves born in 1978 were inoculated as follows with a
2nd Suffolk scrapie passage:-

i/c 1ml i/m, 5ml; s/c 5ml; oral 30ml.

1/6 went down after 48 months with a scrapie/BSE-like disease.

Expt B 6 Her or Jer or HxJ calves were inoculated with angora Goat virus
2/6 went down similarly after 36 months.

Expt C Mice inoculated from brains of calves/cattle in expts A & B
were resistant, only 1/20 going down with scrapie and this was the
reason given for not publishing.

Diagnosis in A, B, C was by histopath. No reports on SAF were given.

Dr Warren Foote indicated success so far in eliminating scrapie in
offspring from experimentally (and naturally) infected sheep by ET. He
had found difficulty in obtaining emhryos from naturally infected sheep (cf SPA).

3. Prof. A Robertson gave a brief account of BSE. The US approach was to
accord it a very low profile indeed. Dr A Thiermann showed the picture
in the "Independent" with cattle being incinerated and thought this was
a fanatical incident to be avoided in the US at all costs. BSE was not
reported in USA.

4. Scrapie incidents (ie affected flocks) have shown a dramatic increase
since 1978. In 1953 when the National Control Scheme was started
there were 10-14 incidents, in 1978 - 1 and in 1988 so far 60.

5. Scrapie agent was reported to have been isolated from a solitary fetus.

6. A western blotting diagnostic technique (? on PrP) shows some promise.

7. Results of a questionnaire sent to 33 states on the subject of
the national sheep scrapie programme survey indicated;

17/33 wished to drop it
6/33 wished to develop it
8/33 had few sheep and were neutral

Information obtained from Dr Wrathall's notes of a meeting of the U.S.
Animal Health Association at Little Rock, Arkansas Nov. 1988.


6.1 BSE to pigs


full text ;

To be published in the Proceedings of the
Fourth International Scientific Congress in
Fur Animal Production. Toronto, Canada,
August 21-28, 1988

Evidence That Transmissible Mink Encephalopathy
Results from Feeding Infected Cattle

R.F. Marsh* and G.R. Hartsough

"Department of Veterinary Science, University of Wisconsin-Madison, Madison,
Wisconsin 53706; and ^Emba/Creat Lakes Ranch Service, Thiensville,
Wisconsin 53092


Epidemiologic investigation of a new incidence of
transmissible mink encephalopathy (TME) in Stetsonville, Wisconsin
suggests that the disease may have resulted from feeding infected
cattle to mink. This observation is supported by the transmission of
a TME-like disease to experimentally inoculated cattle, and by the
recent report of a new bovine spongiform encephalopathy in



Over the next 8-10 weeks, approximately 40% of all the adult mink on the
farm died from

Since previous incidences of TME were associated with common or shared
practices, we obtained a careful history of feed ingredients used over
the past 12-18
months. The rancher was a "dead stock" feeder using mostly (>95%) downer
or dead dairy
cattle and a few horses. Sheep had never been fed.

Experimental Transmission. The clinical diagnosis of TME was confirmed by
histopaihologic examination and by experimental transmission to mink
after incubation
periods of four months. To investigate the possible involvement of
cattle in this disease
cycle, two six-week old castrated Holstein bull calves were inoculated
with a brain suspension from affected mink. Each developed a fatal
encephalopathy after incubation periods of 18 and 19 months.


These findings suggest that TME may result from feeding mink infected
cattle and
we have alerted bovine practitioners that there may exist an as yet
scrapie-like disease of cattle in the United States (Marsh and
Hartsough, 1986).


full text;

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

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




The neuropathology of experimental bovine spongiform encephalopathy
in the pig.

Ryder SJ, Hawkins SA, Dawson M, Wells GA.

Veterinary Laboratories Agency Weybridge, Woodham Lane, New Haw,
Addlestone, Surrey, KT15 3NB, UK.

In an experimental study of the transmissibility of BSE to the pig,
seven of 10 pigs, infected at 1-2 weeks of age by multiple-route
parenteral inoculation with a homogenate of bovine brain from
natural BSE cases developed lesions typical of spongiform
encephalopathy. The lesions consisted principally of severe neuropil
vacuolation affecting most areas of the brain, but mainly the
forebrain. In addition, some vacuolar change was identified in the
rostral colliculi and hypothalamic areas of normal control pigs. PrP
accumulations were detected immunocytochemically in the brains of
BSE-infected animals. PrP accumulation was sparse in many areas and
its density was not obviously related to the degree of vacuolation.
The patterns of PrP immunolabelling in control pigs differed
strikingly from those in the infected animals.

PMID: 10684682 [PubMed - indexed for MEDLINE]

>new FDA/Center for Food Safety and Applied Nutrition BSE-food safety rules,

FDA again is missing the bigger picture, ALL TSEs! THE BSE/nvCJD only
theory should be trashed
once and for all. the name should read;

new FDA/Center for Food Safety and Applied Nutrition TSE-food safety rules

>labeling claims for TSE clearance studies for plasma derivative

>The committee will then discuss and make recommendations
>regarding presumptive transfusion transmissions of variant Creutzfeldt
>Jakob Disease (vCJD) and current FDA-recommended safeguards.

ONE of the things i have been most worried about and will continue to
bring to the attention of the
TSE advisory committee is the continued denial of the potential of
transmission of sporadic CJD
via blood and blood products. THERE are studies showing infectivity in
the blood of sporadic CJD,
and just how many phenotypes there are of sporadic CJD is anyone's guess
at this point in time.
WE have already documented transmission of sporadic CJD via tissues and
TO continue the BSE/nvCJD only theory will only continue to spread this
agent. WE must move
away from this mentality and move on with all TSEs and treat all TSEs as
one, until proven otherwise,
not the other way around. with 6,000 'DEAR nvCJD LETTERS' due to blood
and blood products so far,
i can only imagine the number, once sporadic CJD is documented to have
transmitted via blood, what that
number will be when those 'DEAR SPORADIC CJD LETTERS' go out and how
many will become
clinical once exposed, a frightening thought;

It is possible to transmit BSE to a sheep
by transfusion with whole blood taken from
another sheep during the symptom-free phase
of an experimental BSE infection'

It is well known that variant Creutzfeldt-Jakob
disease (vCJD) is caused by the same strain of
agent that causes bovine spongiform encephalopathy
(BSE) in cattle. F Houston and colleagues
report the preliminary findings of transfusing
blood from 19 UK Cheviot sheep fed with 5 g
BSE-affected cattle brain into Cheviot sheep from
scrapie-free flock of New Zealand-derived
animals. The investigators found BSE clinical
signs and pathology in one recipient of blood taken
from a BSE infected animal. Immunocytochemistry on
tissues taken from the transfused sheep
showed widespread PrPSC deposition throughout the
brain and the periphery. This finding
suggests that blood donated by symptom-free
vCJD-infected human beings could transmit
infection to recipients of blood transfusions.
In a Commentary, Paul Brown states that these
observations are consistent with previous reports
in experimentally infected rodents.


Research letters
Volume 356, Number 9234 16 September 2000

Transmission of BSE by blood transfusion in sheep

Lancet 2000; 356: 999 - 1000
Download PDF (1 Mb)

F Houston, J D Foster, Angela Chong, N Hunter, C J Bostock

See Commentary

We have shown that it is possible to transmit
bovine spongiform encephalopathy (BSE)
to a sheep by transfusion with whole blood
taken from another sheep during the
symptom-free phase of an experimental BSE
infection. BSE and variant
Creutzfeldt-Jakob disease (vCJD) in human
beings are caused by the same infectious
agent, and the sheep-BSE experimental model
has a similar pathogenesis to that of
human vCJD. Although UK blood transfusions
are leucodepleted--a possible protective
measure against any risk from blood
transmission--this report suggests that blood
donated by symptom-free vCJD-infected human
beings may represent a risk of spread
of vCJD infection among the human population
of the UK.

The demonstration that the new variant of
Creutzfeldt-Jakob disease (vCJD) is caused by the
same agent that causes bovine spongiform
encephalopathy (BSE) in cattle1 has raised concerns
that blood from human beings in the symptom-free
stages of vCJD could transmit infection to
recipients of blood transfusions. There is no
evidence that iatrogenic CJD has ever occurred as a
result of the use of blood or blood products,
but vCJD has a different pathogenesis and could
present different risks. CJD is one of the
transmissible spongiform encephalopathies (TSEs)
characterised by the deposition of an abnormal
form of a host protein, PrPSc; the normal
isoform (PrPC) is expressed in many body tissues.
Available evidence, based on detection of
infectivity in blood in rodent models, and absence
of infectivity in naturally occurring TSEs, adds
to the uncertainty in risk assessments of the
safety of human blood. PrPSc has been reported in
blood taken from preclinical TSE-infected sheep,2
but it does not follow that blood is infectious.
Bioassays of human blood can only be carried out
in non-human species, limiting the sensitivity
of the test. One way of avoiding such a species
barrier is to transfer blood by transfusion in an
appropriate animal TSE model. BSE-infected sheep
harbour infection in peripheral tissues3 and
are thus similar to humans infected with vCJD.4
BSE infectivity in cattle does not have
widespread tissue distribution.

We report preliminary data from a study
involving blood taken from UK Cheviot sheep
challenged orally with 5 g BSE-affected cattle
brain and transfused into Cheviot sheep from a
scrapie-free flock of New Zealand-derived animals
(MAFF/SF flock). MAFF/SF sheep do not
develop spontaneous TSE and the transfused
animals are housed separately from other sheep.
All sheep in the study have the PrP genotype
AA136QQ171 which has the shortest incubation
period of experimental BSE in sheep.5 19 transfusions from
BSE-challenged sheep have been
done, mostly with whole blood. Sheep have
complex blood groups and only simple
cross-matching can be done by mixing recipient
serum and donor erythrocytes and vice versa.
Therefore single transfusions only were made
between sedated cross-matched animals to
minimise the risk of severe reactions. Negative
controls were MAFF/SF sheep transfused with
blood from uninfected UK Cheviot sheep. As a
positive control, MAFF/SF sheep were
intravenously injected with homogenised BSE-affected
cattle brain.

We have seen BSE clinical signs and pathological
changes in one recipient of blood from a
BSE-infected animal, and we regard this finding
as sufficiently important to report now rather
than after the study is completed, several years
hence. The blood donation resulting in
transmission of BSE to the recipient was 400 mL
of whole blood taken from a healthy sheep
318 days after oral challenge with BSE. BSE subsequently
developed in this donor animal 629
days after challenge, indicating that blood was taken
roughly half way through the incubation
period. 610 days after transfusion, the transfused
sheep (D505) itself developed typical TSE
signs: weight loss, moderate pruritus, trembling
and licking of the lips, hind-limb ataxia, and
proprioceptive abnormalities. This is the first
experimental transmission of BSE from sheep to
sheep and so we have nothing with which to compare
this incubation period directly. In
cross-species transmissions, bovine BSE injected
intracerebrally gives incubation periods of
about 450 days in these sheep,5 and the donor animal
had an oral BSE incubation period of 629
days (see above). There are no similar data
available on other infection routes.
Immunocytochemistry with the antibody BG4 on tissues
taken from sheep D505 showed
widespread PrPSc deposition throughout the brain and
periphery. Western blot analysis of brain
tissue with the antibody 6H4 showed that the PrPSc
protein had a glycoform pattern similar to
that of experimental BSE in sheep and unlike that
of UK natural scrapie (figure), indicating that
the TSE signs resulted from transmission
of the BSE agent. All other recipients of transfusions
and positive and negative controls are
alive and healthy. The positive controls, which involve a
species barrier, are expected to have
lengthy incubation periods. With one exception, all
transfused animals are at earlier stages
post-transfusion than was D505. The exception is a
sheep which is healthy 635 days after transfusion
with BSE-blood donated at less than 30% of
the BSE incubation period of the donor sheep.

PrPSc (proteinase K treated) analysed by SDS-PAGE,
immunoblotted with 6H4, and
visualised with a chemiluminescent substrate

All lanes are from the same gel with different
exposure times. Size markers are to the left of
lane 1. Lane1: natural scrapie sheep brain,
3 min exposure. Lane 2: as lane 1, 10 min exposure.
Lane 3: sheep D505, blood-transfusion
recipient, 10 min exposure. Lane 4: experimental
BSE-affected sheep brain, 30 s exposure.
Lane 5: as lane 4, 10 min exposure. Each lane
loaded with amount of protein extracted from
0·1 g wet weight of brain, except lane 3 which
was extracted from 0·2 g brain.

Although this result was in only one animal, it
indicates that BSE can be transmitted between
individuals of the same species by whole-blood
transfusion. We have no data on blood fractions
or on levels of infectivity in blood of preclinical
vCJD cases, but whole blood is not now used in
UK transfusions. The presence of BSE infectivity
in sheep blood at an early stage in the
incubation period suggests that it should be
possible to identify which cells are infected, to test
the effectiveness of leucodepletion, and to
develop a diagnostic test based on a blood sample.

We thank Karen Brown, Moira Bruce, Calum
McKenzie, David Parnham, Diane Ritchie, and
the Scottish Blood Transfusion Service. The
project is funded by the Department of Health.

1 Bruce ME, Will RG, Ironside JW, et al.
Transmissions to mice indicate that 'new variant' CJD
is caused by the BSE agent. Nature 1997;
389: 488-501 [PubMed].

2 Schmerr MJ, Jenny A, Cutlip RC. Use of
capillary sodium dodecyl sulfate gel electrophoresis
to detect the prion protein extracted from
scrapie-infected sheep. J Chromatogr B Biomed
Appl 1997; 697: 223-29 [PubMed].

3 Foster JD, Bruce M, McConnell I, Chree A,
Fraser H. Detection of BSE infectivity in brain
and spleen of experimentally infected sheep.
Vet Rec 1996; 138: 546-48 [PubMed].

4 Hill AF, Zeidler M, Ironside J, Collinge J.
Diagnosis of new variant Creutzfeldt-Jakob disease
by tonsil biopsy. Lancet 1997; 349: 99-100.

5 Goldmann W, Hunter N, Smith G, Foster J,
Hope J. PrP genotype and agent effects in
scrapie: change in allelic interaction with
different isolates of agent in sheep, a natural host of
scrapie. J Gen Virol 1994; 75: 989-95 [PubMed].

Institute for Animal Health, Compton, Newbury,
UK (F Houston PhD, CJ Bostock
PhD); and Institute for Animal Health, Neuropathogenesis Unit,
Edinburgh, EH9
3JF, UK (N Hunter PhD, JD Foster BSc, Angela Chong BSc)

Correspondence to: Dr N Hunter


Transmission of prion diseases by blood transfusion

Nora Hunter,1 James Foster,1 Angela Chong,1 Sandra McCutcheon,2 David
Parnham,1 Samantha Eaton,1 Calum MacKenzie1 and Fiona Houston2

see full text;

Volume 356, Number 9234 16 September 2000

BSE and transmission through blood

Lancet 2000; 356: 955 - 956
Download PDF (55 Kb)


Blood from four of 37 human beings with
clinically evident sporadic CJD has been reported
to transmit the disease after intracerebral inoculation into guineapigs,
mice, or hamsters. But each
success has been questioned on technical grounds
and has not been reproducible;


Is it appropriate to publish an experimental
result from a single animal in a study that is not far
enough along even to have validated its positive
controls? Especially a result that does not in any
fundamental way change our current thinking about
BSE and vCJD and which would not seem
to have any practical consequences for public
health? The UK National Blood Transfusion
Service has already implemented leucodepletion
of donated blood, and imports all plasma and
plasma derivatives from BSE-free countries. No
further measures would seem possible--short
of a draconian decision to shut down the whole
UK blood-donor system. What, therefore, is the
rationale for this publishing urgency? The
answer, evidently, is a perceived need to "defuse", by
an immediate and accurate scientific report,
public reaction to possibly inaccurate media
accounts. The full study, when it appears, will
be an important addition to our knowledge of
TSEs, but science should not be driven to what
in certain medical quarters might be termed a
premature emission through fear of media misrepresentation.

Paul Brown

Laboratory of Central Nervous System Studies,
National Institutes of Health, Bethesda,
MD 20892, USA

> What, therefore, is the
> rationale for this publishing urgency?

Wonder what Dr. Paul Brown would think of this stupid statement now?
SEEMS the urgency was and is a long time coming. HOW many more
do we expose with 'rationale' such as Paul Browns?

Transmission of Creutzfeldt-Jakob Disease from Blood and Urine Into Mice

The Lancet, November 9, 1985

Sir,--Professor Manuelidis and his colleagues (Oct 19, p896) report
transmission to animals of Creutzfeldt-Jakob disease (CJD) from the
buffy coat from two patients. We also transmitted the disease from
whole blood samples of a patient (and of mice) infected with CJD.1
Brain, Cornea, and urine from this patient were also infectious, and
the clinicopathological findings2 are summarised as follows.

A 70-year-old man was noted to have a slowing of speech and writing
and some disorientation, all of which progressed rapidly. Decorticate
rigidity, forced grasping, positive snout reflex, and myoclonus
appeared within 2 months. Electroencephalogram revealed typical
periodic synchronous discharge, and he died of pneumonia and upper
gastrointestinal haemorrhage, about 3 months after onset of the
symptoms. The Brain weighed 1290g and showed severe histological
changes diagnostic of CJD, including spongiform change, loss of
nerve cells, and diffuse proliferation of astrocytes. There were no
inflammatory cells, microglia, neurofibrillary tangles, and
amyloid plaques, although virus-like particles were detected by
electron microscopy.

Results of innoculation in Mice

Inocula NO* Incubation period (days)+
Brain 7/10 (4) 789 (+ or - 112)
Cornea 1/6 (0) 1037
Blood 2/13 (0) 1080 (+ or - 69)
Urine 5/10 (1) 880 (+ or - 55)
CSF 0/10

* Number of mice with CJD change/number examined histologically.
Number with amyloid plaques shown in parentheses.

+ means + or - SD

Samples were taken aseptically at necropsy. 10% crude homogenates
of brain and cornea in saline, whole blood (after crushing a clot),
and untreated CSF and urine were innoculated intracerebrally into
CF1 strain mice (20 ul per animal). Some mice showed emaciation,
bradykinesia, rigidity of the body and tail, and sometimes tremor
after long incubation periods. Tissues obtained after the animal
died (or was killed) were studied histologically (table). Animals
infected by various inocula showed common pathological changes,
consisting of severe spongiform changes, glial proliferation, and
a moderate loss of nerve cells. A few mice inoculated with brain
tissue or urine had the same amyloid plaques found in patients and
animals with CJD.3

In our long-term experiments, inoculating materials taken from
twenty patients with CJD or Gerstmann-Straussler-Scheinker's
disease (GSS) into rodents, positive results were obtained in
seventeen cases, including this patient. Brain tissue transmitted
the disease most frequently within the shortes incubation period,
except for one case where the lymph node was the most infectious.
Transmission through the cornea has been noted in man4 and in
guineapigs.5 Whole blood samples taken from three patients were
inoculated and a positive transmission occured only in the case
recorded here. Mouse-to-mouse transmission through blood
inoculation was successful after a mean incubation period of 365
days.1 Transmission through urine was positive in this patient
only, and negative in one other patient and in many infected animals.
Transmission through the CSF from eight patients was negative, yet
transmission via the CSF of infected rats was positive.1

As viraemia has been proved in guineapigs,6 mice,1,7 and lately
in patients with CJD, blood for transfusion or blood products for
medical use must be tested for unconventional pathogens. For this
purpose, we inoculated blood products inot rodents.8 The CJD
pathogen was not found in the products examined. However, this
approach takes too long to be of practical value. More efficient
methods must be developed to detect pathogens and to eliminate
them from blood. One proposal9 is to apply membrane filtration to
the pruification protocol of human growth hormone suspected of
being contaminated with CJD. Similar methods are needed for blood

Department of Neuropathology,
Neurological Institute,
Faculty of Medicine,
Kyushu University,
Fukuoka812, Japan


1. Tateishi J, Sato Y, Kaga M. Doi H, Ohta M. Experimental transmission
of human subacute spongiform encephalopathy to small
rodents 1: Clinical and histological observations.
Acta Neuropathol (Berl) 1980; 51: 127.

2. Shibayama Y, Sakaguchi Y, Nakata K, et al, Creutzfeldt-Jakob
disease with demonstration of virus-like particles.
Acta pathol Jpn 1982;32: 695.

3. Tateishi J, Nagara H, Hikita K, Sato Y. Amyloid plaques in the
brains of mice with Creutzfeldt-Jakob disease.
Ann Neurol 1984; 15: 278.

4. Duffy P, Wolf J, Colings G, DeVoe AG, Streeten B, Cowen D.
Possible person-to-person transmission of Creutzfeldt-Jakob disease.
N Engl J Med 1974; 290: 692.

5. Manuelidis EE, Angelo JN, Gorgacz EJ, Kim JH, Manuelidis L.
Experimental Creutzfeldt-Jakob disease transmitted via the eye
with infected cornea. N Engl J Med 1977; 296: 1334.

6. Manuelidis EE, Gorgacz EJ, Manuelidis L. Viremia in experimental
Creutzfeldt-Jakob disease. Science 1978: 200: 1069.

7. Kuroda Y, Gibbs CJ Jr, Amyx HL, Gajdusek DC.
Creutzfeldt-Jakob disease in mice. Persistent viremiam and
preferential replication of virus in low-density lymphocytes.
Infect Immun 1983; 41: 154.

8. Tateishi J, Tsuji S. Unconventional pathogens causing spongiform
encephalopathis absent in blood products. J Med Virol 1985; 15: 11.

9. Tateishi J, Kitamoto T, Hiratani H. Creutzfeldt-Jakob disease
pathogen in growth hormone preparations is eliminatable.
Lancet (in press).

STUDY DESIGN AND METHODS: BSE was passaged through macaque monkeys and
then adapted to the prosimian microcebe (Microcebus murinus ). Brain
homogenate and buffy coat from an affected microcebe were separately
inoculated intracerebrally into three healthy microcebes (two animals
received brain and one received buffy coat).

RESULTS: All three inoculated microcebes became ill after incubation
periods of 16 to 18 months. Clinical, histopathologic, and
immunocytologic features were similar in each of the recipients.

CONCLUSION: Buffy coat from a symptomatic microcebe infected 17 months
earlier with BSE contained the infectious agent. This observation
represents the first documented transmission of BSE from the blood of an
experimentally infected primate, which in view of rodent buffy coat
infectivity precedents and the known host range of BSE is neither
unexpected nor cause for alarm.

Prions in skeletal muscle

Patrick J. Bosque*,dagger ,Dagger , Chongsuk Ryou*, Glenn Telling*,§,
David Peretz*,dagger , Giuseppe Legname*,dagger , Stephen J.
DeArmond*,dagger ,¶, and Stanley B. Prusiner*,dagger ,||,**

* Institute for Neurodegenerative Diseases and Departments of dagger
Neurology, ¶ Pathology, and || Biochemistry and Biophysics, University
of California, San Francisco, CA 94143

Contributed by Stanley B. Prusiner, December 28, 2001

Considerable evidence argues that consumption of beef products from
cattle infected with bovine spongiform encephalopathy (BSE) prions
causes new variant Creutzfeldt-Jakob disease. In an effort to prevent
new variant Creutzfeldt-Jakob disease, certain "specified offals,"
including neural and lymphatic tissues, thought to contain high titers
of prions have been excluded from foods destined for human consumption
[Phillips, N. A., Bridgeman, J. & Ferguson-Smith, M. (2000) in The BSE
Inquiry (Stationery Office, London), Vol. 6, pp. 413-451]. Here we
report that mouse skeletal muscle can propagate prions and accumulate
substantial titers of these pathogens. We found both high prion titers
and the disease-causing isoform of the prion protein (PrPSc) in the
skeletal muscle of wild-type mice inoculated with either the Me7 or
Rocky Mountain Laboratory strain of murine prions. Particular muscles
accumulated distinct levels of PrPSc, with the highest levels observed
in muscle from the hind limb. To determine whether prions are produced
or merely accumulate intramuscularly, we established transgenic mice
expressing either mouse or Syrian hamster PrP exclusively in muscle.
Inoculating these mice intramuscularly with prions resulted in the
formation of high titers of nascent prions in muscle. In contrast,
inoculating mice in which PrP expression was targeted to hepatocytes
resulted in low prion titers. Our data demonstrate that factors in
addition to the amount of PrP expressed determine the tropism of prions
for certain tissues. That some muscles are intrinsically capable of
accumulating substantial titers of prions is of particular concern.
Because significant dietary exposure to prions might occur through the
consumption of meat, even if it is largely free of neural and lymphatic
tissue, a comprehensive effort to map the distribution of prions in the
muscle of infected livestock is needed. Furthermore, muscle may provide
a readily biopsied tissue from which to diagnose prion disease in
asymptomatic animals and even humans. Dagger Present address: Department
of Medicine, Denver Health Medical Center, Denver, CO 80204.

§ Present address: Department of Microbiology and Immunology, University
of Kentucky, Lexington, KY 40536-0230.

** To whom reprint requests should be addressed. E-mail:


Extraneural Pathologic Prion Protein in Sporadic Creutzfeldt-Jakob Disease

Markus Glatzel, M.D., Eugenio Abela, Manuela Maissen, M.S., and Adriano
Aguzzi, M.D., Ph.D.


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

EMBO reports AOP Published online: 11 April 2003

Widespread PrPSc accumulation in muscles of hamsters orally infected
with scrapie

Achim Thomzig, Christine Kratzel, Gudrun Lenz, Dominique KrÒ¼ger &
Beekes Robert Koch-Institut, P26, Nordufer 20, D-13353 Berlin, Germany

Received 13 February 2003; Accepted 13 March 2003; Published online 11
April 2003.

Abstract :

Scrapie, bovine spongiform encephalopathy and chronic wasting disease
are orally communicable, transmissible spongiform encephalopathies
(TSEs). As zoonotic transmissions of TSE agents may pose a risk to human
health, the identification of reservoirs for infectivity in animal
tissues and their exclusion from human consumption has become a matter
of great importance for consumer protection. In this study, a variety of
muscles from hamsters that were orally challenged with scrapie was
screened for the presence of a molecular marker for TSE infection, PrPSc
(the pathological isoform of the prion protein PrP). Sensitive western
blotting revealed consistent PrPSc accumulation in skeletal muscles from
forelimb and hindlimb, head, back and shoulder, and in tongue.
Previously, our animal model has provided substantial baseline
information about the peripheral routing of infection in naturally
occurring and orally acquired ruminant TSEs. Therefore, the findings
described here highlight further the necessity to investigate thoroughly
whether muscles of TSE-infected sheep, cattle, elk and deer contain
infectious agents.

some previous data on TSE in muscle;



JANUARY 1990, p.68


1 Dr Pattison, a retired but eminent worker on scrapie for many years in
the AFRC, has pointed out that in one of his experimental studies of
scrapie in goats he found scrapie agent in the biceps femoris (rump)
muscle of one animal with clinical disease but not in 2 others with
clinical disease and in none with pre-clinical disease. MAFF have based
their policy on BSE in regard to meat (beef) on the results of studies
of natural scrapie (ie disease occurring under farm conditions) in both
sheep and goats by Hadlow 1979, 80, 81.

Other Infectivity Studies

2. These studies on 52 animals by equally eminent scrapie workers
(Hadlow et al) revealed no evidence whatever of infectivity in skeletal
muscle from these natural cases either in the pre-clinical or even
clinical stages of disease.

It is clear that the pathogenesis of experimental (Pattison) and natural
(Hadlow) scrapie may be different and it was therefore considered wise
to base present policy on knowledge of the natural disease.

3. Pattison exposed his 14 goats to intracerebral inoculation of thrice
passaged scrapie virus (in goats). This may have resulted in strain
selection and/or mutation of the natural agent. In contrast Hadlow's
study involved natural strains (probably multiple) in a flock with a
high incidence of disease in which exposure would almost certainly have
been by the mouth.

4. The fact that Hadlow identified no infectivity in muscle by mouse
inoculation (whereas some other tissues not normally consumed had
detectable infectivifcy) shows that cross contamination of his tissues
did not occur. Pattison's experiments were reported about 20 years
earlier when much less was known about Scrapie. In the intervening
period the knowledge available to Hadlow on the insensitivity of scrapie
agent to heat became available. There is therefore at least the
possibility that Pattison's instruments were not sterilised effectively,
thus possibly giving the false positive result for muscle.

5. Pattison used a more sensitive model for the detection of
infectivifcy, namely goats, whereas Hadlow used mice ie necessitating
crossing the species barrier and possibly reducing the test sensitivity.



6. In regard to the choice of species for agent assay, mice (Hadlow),
these would be guaranteed free of pre-existing Scrapie infection.
Pattison could offer no such guarantee that this was the case in the
animal to which muscle was passaged and disease could have developed
from exposure from a source other than muscle.

7. Pattison did not report that his recipient animals, including the one
inoculated with muscle, were examined by histopathology to confirm the
presence of disease. This is a significant deficit. Clinical diagnosis
alone is not acceptable as adequate evidence for the existence of scrapie.

8. Even in Pattison's studies only in 1 out of 14 goats was infectivity
detected in muscle and that was in a CLINICAL case. In BSE all clinical
cases are notified and do not enter any food chain.

9. The last paragraph of Pattison's letter is illogical. Furthermore,
this is no evidence whatsoever that scrapie or BSE is a danger to man.

W A WATSON 19 January 1990

Private Offices Mr K C Meldrum Mrs E Attridge Mr R Lowson Ms L Austin Mr
R Bradley


CONSIDER the fact non-documented TSEs have been circulating in the US
bovine for decades.
CWD in deer and elk for decades and spreading.
Scrapie in sheep and goats for decades and spreading.
TME in the USA as well.
ALL rendered for human/animal consumption for decades in the USA
(don't think too much mink have been rendered due to musk gland?)
THE lack of TSE surveillance in both animals and MAN.

4.5 MILLION ALZHIEMER'S a year with a projected 20 million by 2050.
4 studies showing from 3 to 33 percent of those diagnosed with Alzheimer's
after autopsy, actually had CJD (Yale, Duke, PA and a Mexican study).
amplfication via medical/surgical arena?

NOW, the important question would be, how many of those new atypical TSE
showing up in cattle and sheep
that are very similar to sporadic CJD, how those tissues, blood and
blood products infectivity studies might come
out and IF BSE propagates as both nvCJD and sporadic CJDs, then the
blood ban is not complete. either way,
the public looses. FOR this reason the damn BSE/nvCJD only theory is
more dangerous than the agent itself.
YOUR missing 85%+ of all cases, and the agent (however many different
phenotypes there maybe)
continues to spread and expose. NOW, for a final thought;

-------- Original Message --------
Subject: TSE REPORT USA September 14, 2004
Date: Tue, 14 Sep 2004 14:54:52 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

Prepared by National Center for Animal Health Programs Eradication and
Surveillance Team July 31, 2004 SCRAPIE INFECTED AND SOURCE FLOCKS AS of
July 31, 2004, there were 71 scrapie infected and source flocks (figure
3). There were 4 new infected and source flocks reported in July (figure
4) with a total of 90 flocks reported for FY 2004 (figure 5). The total
infected and source flocks that have been released in FY 2004 are 62
(figure 6), with 15 flocks released in July. The ratio of infected and
source flocks released to newly infected and source flocks for FY 2004 =
CONFIRMED and reported by the National Veterinary Services Laboratories
(NVSL) in FY 2004, OF WHICH 42 WERE RSSS CASES (figure 7). This includes
60 NEWLY CONFIRMED CASES IN JULY 2004 (figure 8). THIRTEEN cases of
scrapie in GOATS have been reported since 1990 (figure 9). ONE NEW GOAT
CASE WAS REPORTED IN FY 2004. New infected flocks, source flocks, and
flocks released or put on clean-up plans for FY 2004 are depicted in
figure 10... snip...
Emergency Programs * Imported Belgium/Netherlands Sheep Test Results
APRIL 2002 snip... The Western-blot test however cannot differentiate
between scrapie and BSE. The only known validated method to
differentiate between these two diseases requires a series of mouse
bioassay systems, which take at least 23 years for completion. snip...
-------- Original Message -------- Subject: Re: AW: [BSE-L] USDA did not
test possible mad cows - Dr. Detwiler, what about those sheep? Date:
Sun, 13 Jun 2004 11:27:24 -0500 From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy Greetings list members,
Thought I should let the list know that Dr. Detwiler kindly replied to
my question about the delayed 'atypical' TSE testing in the Vermont
sheep and tried to explain what caused the delay. If I interpreted it
correctly, seems it was the fault of the U.K. ; -------- Original
Message -------- Subject: Sheep Date: Sat, 12 Jun 2004 14:26:04 EDT
From: To: Mr. Singeltary. I hope this
finds you well. As you are aware I left the USDA last year. I can only
update you on the sheep before that time. Contact was established with
the UK on doing the bioassay studies. They agreed. However, we were
prioritized after their own needs, hence the delay. I am aware that
there are now additional labs in Europe running the mouse bioassay
strain typing. You will have to contact USDA for further word. Linda
Detwiler ========= My reply to Dr. Detwiler; -------- Original Message
-------- Subject: Re: Sheep Date: Sat, 12 Jun 2004 13:53:57 -0500 From:
"Terry S. Singeltary Sr." To: References:
<> hello Dr. Detwiler, thanks for your kind
reply. > However, we were prioritized after their own needs, hence the
delay. not sure i understand that? > You will have to contact USDA for
further word. already done that, and there answer was; >5/20/04 > >Dear
Mr. Singeltary, > >The Western blot tests on these animals were
completed in April of this >year. That means that we can begin the mouse
inoculations. To get the >results of the Western blot tests, you will
need to submit a Freedom of >Information Act request through our FOIA
office. The FAX number there is >301-734-5941. > >Have a nice day, >
>Jim Rogers >APHIS LPA > and with my previous attempts for information
via the FOIA through this administration (as you are probably very well
aware of) they have all been ignored/refused. so any further attempts
would be fruitless i am sure. thanks anyway... kindest regards, Terry wrote: > Mr. Singeltary. snip... TSS
============================================= 12/10/76 AGRICULTURAL
CHAIRMAN: PROFESSOR PETER WILDY snip... A The Present Position with
respect to Scrapie A] The Problem Scrapie is a natural disease of sheep
and goats. It is a slow and inexorably progressive degenerative disorder
of the nervous system and it ia fatal. It is enzootic in the United
Kingdom but not in all countries. The field problem has been reviewed by
a MAFF working group (ARC 35/77). It is difficult to assess the
incidence in Britain for a variety of reasons but the disease causes
serious financial loss; it is estimated that it cost Swaledale breeders
alone $l.7 M during the five years 1971-1975. A further inestimable loss
arises from the closure of certain export markets, in particular those
of the United States, to British sheep. It is clear that scrapie in
sheep is important commercially and for that reason alone effective
measures to control it should be devised as quickly as possible.
Recently the question has again been brought up as to whether scrapie is
transmissible to man. This has followed reports that the disease has
been transmitted to primates. One particularly lurid speculation
(Gajdusek 1977) conjectures that the agents of scrapie, kuru,
Creutzfeldt-Jakob disease and transmissible encephalopathy of mink are
varieties of a single "virus". The U.S. Department of Agriculture
concluded that it could "no longer justify or permit scrapie-blood line
and scrapie-exposed sheep and goats to be processed for human or animal
food at slaughter or rendering plants" (ARC 84/77)" The problem is
emphasised by the finding that some strains of scrapie produce lesions
identical to the once which characterise the human dementias" Whether
true or not. the hypothesis that these agents might be transmissible to
man raises two considerations. First, the safety of laboratory personnel
requires prompt attention. Second, action such as the "scorched meat"
policy of USDA makes the solution of the acrapie problem urgent if the
sheep industry is not to suffer grievously. snip... 76/10.12/4.6 CHRONIC
JOURNAL Volume 3, Number 8 01 August 2003 Newsdesk Tracking spongiform
encephalopathies in North America Xavier Bosch snip... 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 limited. snip... From: "Belay, Ermias"
To: Cc: "Race, Richard (NIH)" ; ; "Belay, Ermias" Sent: Monday,
September 30, 2002 9:22 AM Subject: RE: TO CDC AND NIH - PUB MED- 3 MORE
DEATHS - CWD - YOUNG HUNTERS Dear Sir/Madam, In the Archives of
Neurology you quoted (the abstract of which was attached to your email),
we did not say CWD in humans will present like variant CJD. That
assumption would be wrong. I encourage you to read the whole article and
call me if you have questions or need more clarification (phone:
404-639-3091). Also, we do not claim that "no-one has ever been infected
with prion disease from eating venison." Our conclusion stating that we
found no strong evidence of CWD transmission to humans in the article
you quoted or in any other forum is limited to the patients we
investigated. Ermias Belay, M.D. Centers for Disease Control and
Prevention > > -----Original Message----- > > From: > > Sent: Sunday,
September 29, 2002 10:15 AM > > To:;;
- YOUNG > > HUNTERS continued...tss Chronic Wasting Disease and
Potential Transmission to Humans Ermias D. Belay,*Comments 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 Environmental Sources
of Prion Transmission in Mule Deer Michael W. Miller,*Comments 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: PAGE 25 Transmission
Studies Mule deer transmissions of CWD were by intracerebral inoculation
and compared with natural cases resulted in a more rapidly progressive
clinical disease with repeated episodes of synocopy ending in coma. One
control animal became affected, it is believed through contamination of
inoculam (?saline). Further CWD transmissions were carried out by Dick
Marsh into ferret, mink and squirrel monkey. Transmission occurred in
all of these species with the shortest incubation period in the ferret.
snip... The occurrence of CWD must be viewed against the context of the
locations in which it occurred. It was an incidental and unwelcome
complication of the respective wildlife research programmes. Despite its
subsequent recognition as a new disease of cervids, therefore justifying
direct investigation, no specific research funding was forthcoming. The
USDA viewed it as a wildlife problem and consequently not their
province! [figures...TSS] snip... VISIT TO USA - DR A E WRATHALL - INFO
ON BSE AND SCRAPIE 1. Dr Clark lately of the Scrapie Research Unit,
Mission Texas has successfully transmitted ovine and caprine scrapie to
cattle. The experimental results have not been published but there are
plans to do this. This work was initiated in 1978. A summary of it is:-
snip... Gerald Wells: Report of the Visit to USA, April-May 1989 Aguzzi warns of CWD
danger The TSE family of diseases also includes chronic wasting disease
(CWD) in deer, a condition that has spread in the US in recent years
(Nature 416, 569; 2002). Speaking at the Days of Molecular Medicine
conference in La Jolla in March, prion expert Adriano Aguzzi issued a
strong warning against underestimating this form of TSE. "For more than
a decade, the US has by-and-large considered mad cows to be an
exquisitely European problem. The perceived need to protect US citizens
from this alien threat has even prompted the deferral of blood donors
from Europe," he said. "Yet the threat-from-within posed by CWD needs
careful consideration, since the evidence that CWD is less dangerous to
humans than BSE is less-than-complete. Aguzzi went on to point out that
CWD is arguably the most mysterious of all prion diseases. "Its
horizontal spread among the wild population is exceedingly efficient,
and appears to have reached a prevalence unprecedented even by BSE in
the UK at its peak. The pathogenesis of CWD, therefore, deserves a
vigorous research effort. Europeans also need to think about this
problem, and it would be timely and appropriate to increase CWD
surveillance in Europe too." Aguzzi has secured funding from the
National Institutes of Health to investigate CWD, and the effort will be
lead by Christina Sigurdson in his department at the University of
Zurich. KAREN BIRMINGHAM, LONDON This quote from Dr. Gambetti is
especially significant since he is the rather cautious TSE researcher
under contract with the Centers for Disease Control to examine the
brains of individuals who have died of CJD. ----------------- Pierluigi
Gambetti, director of the National Prion Disease Pathology Surveillance
Center at Case Western Reserve University in Cleveland, said all deer
should be tested for chronic wasting disease before any processing is
done. "There is no way around it," he said. "Nobody should touch that
meat unless it has been tested." -------------------------------------- TRANSMISSIBLE MINK ENCEPHALOPATHY
Transmissible Mink Encephalopathy Transmissible mink encephalopathy
(TME) is a rare illness that affects the central nervous system of
ranch-raised mink. It was first detected in the United States in 1947.
Since then, TME outbreaks have been reported in numerous locations
worldwide, including the United States, Canada, Finland, Germany, and
the republics of the former Soviet Union. snip... The first documented
TME outbreak in the United States occurred in 1947 on one ranch in
Wisconsin and then on a ranch in Minnesota that had received mink from
the Wisconsin ranch. In 1961, TME outbreaks occurred on five ranches in
Wisconsin. In 1963, outbreaks occurred in Idaho, Minnesota, and
Wisconsin. Epidemiologic data from the Minnesota and Wisconsin outbreaks
trace the cases in those States to one common purchased food source. The
1985 Stetsonville Outbreak The most recent TME outbreak occurred on one
mink ranch in Stetsonville, WI, in 1985. In the herd of 7,300 adult
mink, 60 percent of the animals died. Clinical signs included tail
arching, incoordination, and hyperexcitability. At the most advanced
stages of the disease, the animals were in trancelike states and
eventually died. The outbreak lasted 5 months. Microscopic examination
of sections of the brain confirmed the spongelike changes characteristic
of TME. Diagnostic tests identified the prion protein. The following
year, mink born during the outbreak showed no signs of TME. The late
Richard Marsh, a veterinary virologist at the University of Wisconsin
who studied the transmission of TME and other TSEs, investigated this
outbreak. Marsh learned that the mink were fed a diet composed of fresh
meat products from downer cattle and commercial sources of fish,
poultry, and cereal. Downer cattle are nonambulatory and cannot rise
because they are affected with a condition such as a metabolic disease,
broken limbs, or a central nervous system disorder. Marsh theorized that
the meat from these downer cattle introduced a TSE agent to the mink in
which TME resulted. Although Marshs hypothesis is based on speculation
and anecdotal evidence, in 1993 APHIS adjusted its national BSE
surveillance program to include testing downer cattle for evidence of a
TSE. The brains of more than 20,141 cattle have been examined at APHIS
National Veterinary Services Laboratories and other State diagnostic
laboratories. Not a single tissue sample has revealed evidence of BSE or
another TSE in cattle... snip... BOVINE SPONGIFORM
ENCEPHALOPATHY 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... snip... -- Steve Mitchell is UPI's Medical
Correspondent. E-mail Copyright 2001-2004 United
Press International 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
EFSA Scientific Report on the Assessment of the Geographical BSE-Risk
(GBR) of the United States of America (USA) Adopted July 2004 (Question
N EFSA-Q-2003-083) [20 August 2004]
From: Terry S. Singeltary Sr. [] Sent: Tuesday, July 29,
2003 1:03 PM To: Cc:;; 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
EFSA Scientific Report on the Assessment of the Geographical BSE-Risk
(GBR) III of the United States of America (USA) Adopted July 2004
(Question N EFSA-Q-2003-083) [20 August 2004]
THE MAD COW DOWNER THAT WALKED Dave Louthan - Killed the Mad Cow Stanley Prusiner ''nobody
has ever ask us to comment'' ''they don't want us to comment'' ''they
never ask'' i tried to see Venemon, after Candian cow was discovered
with BSE. went to see lyle. after talking with him... absolute
ignorance... then thought i should see Venemon... it was clear his
entire policy was to get cattle boneless beef prods across the border...
nothing else mattered... his aids confirmed this... 5 times i tried to
see Venemon, never worked... eventually met with carl rove the
political... he is the one that arranged meeting with Venemon... just
trying to give you a sense of the distance... healh public safety... was
never contacted... yes i believe that prions are bad to eat and you can
die from them...END Dr. Stan bashing Ann Veneman - 3 minutes On September 13, 2004, no
positive or inconclusive test results were reported. Weekly Summary
Cumulative Total from June 1, 2004: 57,064
Research Project: Bovine Spongiform Encephalopathy and Other
Transmissible Spongiform Encephalopathies Location: Virus and Prion
Diseases of Livestock Title: Experimental Inoculation of Tme, Scrapie,
and Cwd to Raccoons (Procyon Lotor) and the Utilization of Raccoons for
Strain-Typing of Unknown Tses in the United States Authors item Hamir,
Amirali item Miller, Janice item Cutlip, Randall item Stack, M - VSA,
WEYBRIDGE, UK item Chaplin, M - VSA, WEYBRIDGE, UK item Bartz, J -
CREIGHTON UNIVERSITY item Jenny, A - USDA, APHIS item Williams, E -
Submitted to: American Association Of Veterinary Laboratory
Diagnosticians Publication Acceptance Date: April 29, 2002 Publication
Date: October 17, 2002 Abstract only Technical Abstract: Raccoons
(Procyon lotor) are omnivorous and their diet may include carrion. It
is, therefore, possible that in the wild they may get exposed to
carcasses of animals with transmissible spongiform encephalopathies
(TSEs). To determine the susceptibility of raccoons to transmissible
mink encephalopathy (TME), scrapie, and chronic wasting disease (CWD),
each of these agents was inoculated intracerebrally into a group of 4
kits. Three uninoculated kits served as controls. All raccoons in the
TME-inoculated group developed neurologic signs and were euthanized
within 6 months post inoculation (PI). In the scrapie-inoculated group,
3 animals became sick and were euthanized between 18 and 22 PI. Although
the fourth raccoon in this group did not show any clinical signs, it was
euthanized at 24 months PI. At present, 3 years PI, all CWD-infected
raccoons are alive and apparently healthy. At necropsy all clinically
affected raccoons had extensive microscopic lesions of spongiform
encephalopathy and protease-resistant prion protein (PrPres) was
detected in the CNS by immunohistochemistry and Western blot. These
preliminary findings demonstrate that TME and scrapie can be transmitted
to raccoons within 6 months and 2 years, respectively, whereas CWD
cannot. Based on these incubation periods, it may be possible to
differentiate these 3 TSEs. Such a laboratory model would be relatively
simple, fast and inexpensive for strain-typing of unknown TSEs in the
CHAPTER 14 LAYING ODDS 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 population. INTRODUCTION CJD screening may
miss thousands of cases By Steve Mitchell UPI Medical Correspondent
Published 7/21/2003 3:00 PM View printer-friendly version WASHINGTON,
July 21 (UPI) -- The federal government's monitoring system for cases of
Creutzfeldt-Jakob disease, a fatal human brain illness, could be missing
tens of thousands of victims, scientists and consumer advocates have
told United Press International. Creutzfeldt-Jakob disease or CJD can be
caused by eating beef contaminated with mad cow disease, but the critics
assert without a better tracking system it might be impossible to
determine whether any CJD cases are due to mad cow or obtain an accurate
picture of the prevalence of the disorder in the United States.
Beginning in the late 1990s, more than 100 people contracted CJD in the
United Kingdom and several European countries after eating beef infected
with bovine spongiform encephalopathy -- the clinical name for mad cow
disease. No case of mad cow has ever been detected in U.S. cattle and
the Centers for Disease Control and Prevention's monitoring system has
never detected a case of CJD due to eating contaminated American beef.
Nevertheless, critics say, the CDC's system misses many cases of the
disease, which currently is untreatable and is always fatal. The first
symptoms of CJD typically include memory loss and difficulty keeping
balance and walking. As the disease destroys the brain, patients rapidly
progress in a matter of months to difficulty with movement, an inability
to talk and swallow and, finally, death. Spontaneously-occurring or
sporadic CJD is a rare disorder. Only about 300 cases appear nationwide
each year, but several studies have suggested the disorder might be more
common than thought and as many as tens of thousands of cases might be
going unrecognized. Clusters of CJD have been reported in various areas
of the United States -- Pennsylvania in 1993, Florida in 1994, Oregon in
1996, New York in 1999-2000 and Texas in 1996. In addition, several
people in New Jersey developed CJD in recent years, including a 56 year
old woman who died on May 31, 2003. Although in some instances, a mad
cow link was suspected, all of the cases ultimately were classified as
sporadic. People who develop CJD from eating mad-cow-contaminated beef
have been thought to develop a specific form of the disorder called
variant CJD. But new research, released last December, indicates the mad
cow pathogen can cause both sporadic CJD and the variant form. "Now
people are beginning to realize that because something looks like
sporadic CJD they can't necessarily conclude that it's not linked to
(mad cow disease)," said Laura Manuelidis, section chief of surgery in
the neuropathology department at Yale University, who conducted a 1989
study that found 13 percent of Alzheimer's patients actually had CJD.
Several studies, including Manuelidis', have found that autopsies reveal
3 percent to 13 percent of patients diagnosed with Alzheimer's or
dementia actually suffered from CJD. Those numbers might sound low, but
there are 4 million Alzheimer's cases and hundreds of thousands of
dementia cases in the United States. A small percentage of those cases
could add up to 120,000 or more CJD victims going undetected and not
included in official statistics. Experiences in England and Switzerland
-- two countries that discovered mad cow disease in their cattle -- have
heightened concerns about the possibility some cases of sporadic CJD are
due to consuming mad-cow-tainted beef. Both countries have reported
increases in sporadic CJD since mad cow was first detected in British
herds in 1986. Switzerland discovered last year its CJD rate was twice
that of any other country in the world. Switzerland had been seeing
about eight to 11 cases per year from 1997 to 2000. Then the incidence
more than doubled, to 19 cases in 2001 and 18 cases in 2002. The CDC
says the annual rate of CJD in the United States is one case per million
people, but the above studies suggest the true prevalence of CJD is not
known, Manuelidis told UPI. Diagnosing CJD or Alzheimer's is difficult
because no test exists that can identify either disease in a living
patient with certainty. So physicians must rely on the patient's
symptoms to determine which illness might be present. Sometimes,
however, the symptoms of one disease can appear similar to the other
disorder. The only way to determine the disease conclusively is to
perform an autopsy on the brain after death. Unfortunately, although
autopsies once were performed on approximately half of all corpses, the
frequency has dropped to 15 percent or less in the United States. The
National Center for Health Statistics -- a branch of the CDC -- stopped
collecting autopsy data in 1995. "If we don't do autopsies and we don't
look at people's brains ... we have no idea about what is the general
prevalence of these kinds of infections and (whether) it is changing,"
Manuelidis said. At the same time autopsies have been declining, the
number of deaths attributed to Alzheimer's has increased more than
50-fold since 1979, going from 857 deaths then to nearly 50,000 in 2000.
Though it is unlikely the dramatic increase in Alzheimer's is due
entirely to misdiagnosed CJD cases, it "could explain some of the
increase we've seen," Manuelidis said. "Neurodegenerative disease and
Alzheimer's disease have become a wastebasket" for mental illness in the
elderly that is difficult to diagnose conclusively, she said. "In other
words, what people call Alzheimer's now is more broad than what people
used to call it, and that has the possibility of encompassing more
diseases -- including CJD." The autopsy studies that found undiagnosed
CJD cases raise the question of whether the United States "already has
an undetected epidemic here," Jeff Nelson, director of, a
vegetarian advocacy Web site, told UPI. "What's the source of that?"
Nelson asked. "Could it be the same source of encephalitis we saw in
minks?" Nelson referred to an outbreak of a mad-cow-type disorder in
minks in Wisconsin in the 1980s. The origin was traced back to the
animals' diet, which included parts of so-called downer cattle -- sick
cows that are unable to stand, which often indicates a neurological
disease, including mad cow. The mink disease raised concerns about
whether U.S. cattle were carrying a mad-cow-like pathogen even prior to
the U.K. epidemic that began in 1986. Andrew Monjan, chief of the
neuropsychology of aging program at the National Institute of Aging --
part of the National Institutes of Health in Bethesda, Md. --
acknowledged there has been an increase in U.S. Alzheimer's cases.
However, he told UPI, this probably is due to the aging of the
population -- as people grow older, they develop a higher risk of
developing Alzheimer's. "There's been no change in the number of CJD
cases in the country and there has been clearly a tracking of the
unusual cases of CJD" that could be due to mad cow disease, Monjan said.
However, Terry Singletary, coordinator of CJD Watch -- an organization
founded to track CJD cases -- says efforts to track the disease have
been close to nonexistent. For example, only 12 states require such
reports. Therefore, many cases might be going undetected, unreported or
misdiagnosed. If more states made CJD a reportable illness, there would
be more clusters detected across the United States, said Singletary, who
became involved with CJD advocacy after his mother died from a form of
CJD known as Heidenhain variant. In the 18-year period between 1979 and
1996, he noted, the country saw a jump from one case of sporadic CJD in
people under the age of 30 -- a warning sign for a link to mad cow
because nearly all of the U.K. victims were 30 years of age or younger
-- to five cases in five years between 1997 and 2001. "That represents a
substantial blip," he told UPI. Singletary also said there have been
increases in sporadic CJD in France, Germany and Italy, all of which
have detected mad cow disease in their cattle. So far, the CDC has
refused to impose a national requirement that physicians and hospitals
report cases of the disease. The agency has not chosen to make CJD a
reportable disease because "making it reportable is not necessarily
directly helpful in surveillance because in some states where it's
reportable you may not get the physician to report it," said Dr. Ermias
Belay, CDC's medical epidemiologist working on CJD. Instead, the agency
relies on other methods, including death certificates and urging
physicians to send suspicious cases to the National Prion Disease
Pathology Surveillance Center at Case Western Reserve University in
Cleveland, which is funded by the CDC. However, because autopsies
generally are not done, if a CJD case is misdiagnosed as Alzheimer's or
dementia, a correct diagnosis might never be determined and therefore
the cause of death listed on a death certificate might be inaccurate.
Belay told UPI he discounted this possibility. It is unlikely to happen,
he said, because it is easy to distinguish CJD from Alzheimer's -- the
two conditions display different symptoms. Manuelidis disagreed. It can
be quite difficult to determine accurately if a patient has CJD, as
evidenced by her study, in which respected and competent neurologists
and psychiatrists at Yale originally diagnosed patients with
Alzheimer's, yet were wrong at least 13 percent of the time. Another
study conducted at the University of Pennsylvania, which found 6 percent
of dementia patients actually were suffering from CJD, supports the
difficulty in distinguishing the illnesses correctly. The U. Penn.
researchers concluded: "These results show that in patients with a
clinical diagnosis of dementia, the etiology (cause) cannot be
accurately predicted during life." In addition, the NPDPSC sees less
than half of all the CJD cases each year, so the CDC's investigational
system not only is missing many of the misdiagnosed CJD cases, it also
is not conducting autopsies on most of the detected cases. Belay said
the CDC follows up on all cases of CJD that occur in people under age
55, as these could be linked to variant -- mad-cow-related -- CJD. But
so far, all have turned out to be sporadic forms of the disease. About
30 cases of the disorder occur each year in the United States in this
age group, while the remaining 270 or so are older. The case of Carrie
Mahan -- a Philadelphia woman who developed a brain disorder that
appeared to be CJD and died from it in 2000 at the age of 29 --
illustrates just how difficult it can be to diagnose the disease.
Mahan's physician, Dr. Peter Crinos of the University of Pennsylvania
Medical Center, ruled out other disorders and felt certain the young
woman had died of CJD, a concern that raised the possibility of a link
to mad cow disease because of her young age. When neuropathologist
Nicholas Gonatas, who had seen CJD before, examined Mahan's brain after
her death, he, likewise, was confident he detected the microscopic,
sponge-like holes caused by the disease. But when he sent brain samples
to the NPDPSC, the results came back negative. Gonatas, convinced the
surveillance center's finding was erroneous, sent off two more samples,
only to have them both come back negative. Subsequent research, however,
has shown the test used by the surveillance center cannot rule out CJD,
said Crinos, an assistant professor of neurology. "There's no question
that Carrie had a spongiform encephalopathy," Crinos said, but added
although it appeared to be CJD, it is difficult if not impossible to say
if it was due to mad cow disease. Crinos told UPI until the CDC
implements a better tracking system, a lot of questions will remain
about CJD and cases like Carrie Mahan's. One central question: Why are
cases of what is presumed to be a rare disease popping up in clusters in
certain areas of the country? Crinos said the clustering suggests an
environmental or food-borne cause, but so far, "No one knows the answer
to that." Copyright © 2001-2003 United Press International THE
EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES) snip... One reason for this
was the _inaccuracy_ in coding of cases correctly certified as CJD
Coding is carried out by staff who are not medically qualified and it is
not surprising that coding errors occur in the processing of large
numbers of certificates. In 1982, 12,000 certificates per week were
processed at the office of population censuses and surveys bu 15 coders
and 6 checkers (Alderson et al., 1983). The occurrence of both inter-
and intra-observer coding errors has been described (Curb et al., 1983)
and the _inaccuracies_ of BOTH certification and coding discovered in
this study _support_ the introduction of a more accurate system of death
certificates and a more detailed and specific coding system... snip... 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? WHO is to say how
much infectivity are in some of these variants of sporadic CJDs, without
confirming this? if we look at the 6 different variants of sporadic
CJDs, has the infective dose for all 6 _documented_ variants been
quantified, and documented as being 'measurable'? WILL there be more
variants of sporadic CJDs, and what of the ramifications from them? WHAT
of other strains/variants of TSE in cattle, BSE in sheep, CWD in deer
and elk, or any of the 20+ strains of Scrapies in sheep or goats? i get
dizzy thinking of the different scenerio's. what would the human TSEs
from these species look like?
HOUND STUDY AS implied in the Inset 25 we must not _ASSUME_ that
transmission of BSE to other species will invariably present pathology
typical of a scrapie-like disease. snip...

ONE FINAL important factor, the recent finding of the incubation period
of 38 years from a _small_ dose of human growth hormone; snip... We
describe the second patient with hGH related CJD in the Netherlands. The
patient developed the disease 38 years after hGH injections. To our
knowledge, this is the longest incubation period described for any form
of iatrogentic CJD. Furthermore, our patient was _not_ treated with hGH,
but only received a _low_ dose as part of a diagnostic procedure. (see
full text below). snip...

Creutzfeldt-Jakob disease 38 years after diagnostic

use of human growth hormone SO my quesion is,
how low is 'low' in quantifing the infectious dose in vCJD, comparing to
_all_ sporadic CJDs, from the many different potential routes, sources,
and infectivity dose? HOW many more do we expose while waiting for

Thank You,

I am sincerely,

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

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