Follow Ups | Post Followup | Back to Discussion Board | VegSource
See spam or
inappropriate posts?
Please let us know.

From: TSS ()
Subject: BSE Agriculture Secretary Mike Johanns ''SOUND SCIENCE'' he does not have a clue
Date: March 3, 2005 at 7:26 pm PST

-------- Original Message --------
Subject: Re: Consumers Union asks feds to retest suspect mad cow after crucial test omitted
Date: Thu, 03 Mar 2005 21:18:51 -0600
From: "Terry S. Singeltary Sr."
To: Bovine Spongiform Encephalopathy
References: <> <>


I am listening to this hearing on Tuesday, and they keep
repeating 'sound science' time and time again, but never
mention any. I still want to know why my Texas Mad Cows
were either rendered or tested postive twice but ruled inconclusive
without WB? This is NOT 'sound science'.

Dr. Prusiner won the Nobel Peace Prize for his work on
TSEs and they flub his warnings like HE is a high school drop
out, and they call this 'sound science' ?

Dr. Prusiner et al says ;

>PNAS | March 1, 2005 | vol. 102 | no. 9 | 3501-3506
>Diagnosis of human prion disease
>Jiri G. Safar *, , Michael D. Geschwind , , Camille Deering
>*, Svetlana Didorenko *, Mamta Sattavat ¶, Henry Sanchez ¶,
>Ana Serban * , Martin Vey ||, Henry Baron **, Kurt Giles *,
>, Bruce L. Miller , , Stephen J. DeArmond * , ¶ and Stanley
>B. Prusiner *, , ,
>*Institute for Neurodegenerative Diseases, Memory and Aging
>Center, and Departments of Neurology, ¶Pathology, and
>Biochemistry and Biophysics, University of California, San
>Francisco, CA 94143; ||ZLB Behring, 35041 Marburg, Germany;
>and **ZLB Behring, 75601 Paris, France
>Contributed by Stanley B. Prusiner, December 22, 2004


>The studies reported here are likely to change profoundly
>the approach to the diagnosis of prion disease in both
>humans and livestock (31–33). The superior performance of
>the CDI in diagnosing prion disease compared to routine
>neuropathologic examination and IHC demands that the CDI be
>used in future diagnostic evaluations of prion disease.
>Prion disease can no longer be ruled out by routine
>histology or IHC. Moreover, the use of IHC to confirm cases
>of bovine spongiform encephalopathy after detection of
>bovine PrPSc by the CDI (10) seems an untenable approach in
>the future. Clearly, the CDI for HuPrPSc is as sensitive or
>more sensitive than bioassays in Tg(MHu2M) mice (Fig. 1).
>Our results suggest that using the CDI to test large numbers
>of samples for human prions might alter the epidemiology of
>prion diseases. At present, there is limited data on the
>frequency of subclinical variant CJD infections in the U.K.
>population (34). Because appendixes and tonsils were
>evaluated only by IHC, many cases might have escaped
>detection (Tables 1 and 2). Equally important may be the use
>of CDI-like tests to diagnose other neurodegenerative
>disorders, such as Alzheimer's disease, Parkinson's disease,
>and the frontotemporal dementias. Whether IHC underestimates
>the incidence of one or more of these common degenerative
>diseases is unknown. Moreover, CDI-like tests may help
>determine the frequency with which these disorders and the
>prion diseases occurs concomitantly in a single patient (35,


AND then they sit up there and say they are using 'sound science' $


OR what about the 'sound science' of the 30 month rule $

"Terry S. Singeltary Sr."

11/03/2003 01:19 PM






I would like to kindly comment on Docket No. 03-080-1


>Under this proposal, ruminant and ruminant products eligible for entry into
>the United States from a BSE minimal risk region would include:
>1) bovine
>animals less than 30 months of age for immediate slaughter;
>2) bovine
>animals for feeding to be moved to a designated feedlot and then to
>slaughter at less than 30 months of age;

>6) fresh (chilled or frozen)
>meat from bovines less than 30 months of age; 7) fresh (chilled or frozen)
>whole or half carcasses of bovines less than 30 months of age; 8) fresh
>(chilled or frozen) bovine liver; 9) fresh (chilled or frozen) bovine

the myth that cattle under 30 months of age are free from BSE/TSE is
just that, a myth,
and it's a false myth !

the youngest age of BSE case to date is 20 months old; As at: 31 May
2003 Year of onset Age youngest case (mnths) Age 2nd youngest case
(mnths) Age 2nd oldest case (yrs.mnths) Age oldest case (yrs.mnths) 1986
30 33 5.03 5.07 1987 30 31 9.09 10.00 1988 24 27 10.02 11.01(2) 1989 21
24(4) 12.00(2) 15.04 1990 24(2) 26 13.03 14.00 1991 24 26(3) 14.02 17.05
1992 20 26 15.02 16.02 1993 29 30(3) 14.10 18.10 1994 30(2) 31(2) 14.05
16.07 1995 24 32 14.09 15.05 1996 29 30 15.07 17.02 1997 37(7) 38(3)
14.09 15.01 1998 34 36 14.07 15.05 1999 39(2) 41 13.07 13.10 2000 40 42
17.08 19.09 2001 48(2) 56 14.10 14.11 2002 51 52 15.08 15.09(2) 2003 50
62 11.11 14.11

The implications of the Swiss result for Britain, which has had the most
BSE, are complex. Only cattle aged 30 months or younger are eaten in
Britain, on the assumption, based on feeding trials, that cattle of that
age, even if they were infected as calves, have not yet accumulated
enough prions to be infectious. But the youngest cow to develop BSE on
record in Britain was 20 months old, showing some are fast incubators.
Models predict that 200-300 cattle under 30 months per year are infected
with BSE and enter the food chain currently in Britain. Of these 3-5
could be fast incubators and carrying detectable quantities of prion.


"Terry S. Singeltary Sr."

07/11/2004 09:34 PM





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


No. 04-047-l

No. 04-021ANPR

No. 2004N-0264


Federal Measures to Mitigate BSE Risks: Considerations for Further Action

Greetings FDA, USDA and APHIS et al,

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

CWD and Scrapie have been running rampant in the USA for decades.
BOTH of which have been rendered and fed back to animals for
human/animal consumption for decades. All of which transmits to primates
by the natural and non-forced
oral consumption of TSE scrapie, CJD, Kuru agent (and CWD by inoculation).

Strong Scientific evidence discovered
back in the 80s support the fact that a TSE has been prevalent in the
USA bovine for decades, either undetected or ignored. IF you consider
the recent
stumbling and staggering TEXAS cow that was showing all signs of a CNS/TSE
disorder that was ordered to be rendered without BSE/TSE test, brains,
spinal cord, head and all (as to no possible evidence left of TSE), I
would think the 'ignored'
or 'covered up' to be the better terminology. Then you have the Downer
in Washington state that was actually a good walker and then all the
banned Canadian products that some how found it's way across the
border into the USA, considering all this, it is very difficult for me to
believe that the FDA/USDA/APHIS et al are doing everything
possible to protect the 'consumer'. Hardly the case;

Congressman Henry Waxmans Letter to the Honorable Ann Veneman

-------- Original Message --------
Subject: Re: Congressman Henry Waxmans Letter to the Honorable Ann
Veneman on failure by USDA/APHIS TO TEST TEXAS MAD COW
Date: Wed, 9 Jun 2004 16:48:31 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
References: <>

######## Bovine Spongiform Encephalopathy #########


> October 1998
> BSE Red Book 2.1-36

> Laboratory Coordination--The Laboratory Coordination Officer
> will advise the READE(3 Director concerning laboratory capabilities and
> appropriate laboratory examinations to be conducted to provide needed
> results as rapidly as possible. This individual will assist with
> interpretation of results.

seems that if the 'enhanced BSE/TSE testing program' is to test some
400,000+ animals in 1 1/2 years, they better hurry up, times a wasting.

> BSE Red Book 2.1-39
> 7.6 Depopulation Procedures
> Under no
> circumstances may BSE suspects be sent fo slaughhter or rendering.


> BSE Red Book 2.1-40
> 7.7 Disposal
> Under no circumstances may BSE suspects be sent to slaughter or
> rendering. Notify FDA, CVM if you suspect that the carcass of a
> BSE-confirmed animal has moved to rendering or animal feed
> manufacturing. Field personel should arrange for the carcass to be
> transported to and examined by a qualified veterinary pathologist or
> field veterinary medical officer. After the pathologic examination has
> been completed and the necessary diagnostic specimens have been
> obtained, field personnel should arrange for disposal of the carcass.
> Before a method of disposal is selected, there are many factors that
> must be considered, and often other State and Federal agencies must be
> consulted. The environmental and legal impacts of the operation must be
> considered. Upon recommendation of the State or Federal agencies, VS may
> consider other disposal methods.

> 7.7.3 Rendering
> Because BSE is spread by rendered animal protein, BSE-suspect and
> confirmed carcasses must not be rendered, unless the rendered material
> is incinerated. Notify FDA, CVM if you suspect that dead BSE animals or
> carcasses have moved to rendering or animal feed manufacturing.


> 7.10.11 Prevention--Suspects and animals confirmed to have BSE must not
> be rendered. Producers, feed mills, and rendering establishments should
> adhere to U.S. State and local rendering policies and FDA regulations
> concerning the feeding of rendered animal protein to ruminants...



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

From: Terry S. Singeltary Sr. []

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



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

SUPPRESSED peer review of Harvard study October 31, 2002

COURSE, why would they pay attention to anyone else,
if they are not going to pay attention to Prusiner et al;

Vet Pathol 42:107–108 (2005)
Letters to the Editor
Absence of evidence is not always evidence of absence.
In the article ‘‘Failure to detect prion protein (PrPres) by
immunohistochemistry in striated muscle tissues of animals
experimentally inoculated with agents of transmissible spongiform
encephalopathy,’’ recently published in Veterinary
Pathology (41:78–81, 2004), PrPres was not detected in striated
muscle of experimentally infected elk, cattle, sheep, and
raccoons by immunohistochemistry (IHC). Negative IHC,
however, does not exclude the presence of PrPSc. For example,
PrPres was detected in skeletal muscle in 8 of 32
humans with the prion disease, sporadic Creutzfeldt-Jakob
disease (CJD), using sodium phosphotungstic acid (NaPTA)
precipitation and western blot.1 The NaPTA precipitation,
described by Wadsworth et al.,3 concentrates the abnormal
isoform of the prion, PrPres, from a large tissue homogenate
volume before western blotting. This technique has increased
the sensitivity of the western blot up to three orders
of magnitude and could be included in assays to detect
PrPres. Extremely conspicuous deposits of PrPres in muscle
were detected by IHC in a recent case report of an individual
with inclusion body myositis and CJD.2 Here, PrPres was
detected in the muscle by immunoblotting, IHC, and paraf-
fin-embedded tissue blot. We would therefore caution that,
in addition to IHC, highly sensitive biochemical assays and
bioassays of muscle are needed to assess the presence or
absence of prions from muscle in experimental and natural
TSE cases.
Christina Sigurdson, Markus Glatzel, and Adriano Aguzzi
Institute of Neuropathology
University Hospital of Zurich
Zurich, Switzerland
1 Glatzel M, Abela E, et al: Extraneural pathologic prion
protein in sporadic Creutzfeldt-Jakob disease. N Engl J
Med 349(19):1812–1820, 2003
2 Kovacs GG, Lindeck-Pozza E, et al: Creutzfeldt-Jakob
disease and inclusion body myositis: abundant diseaseassociated
prion protein in muscle. Ann Neurol 55(1):
121–125, 2004
3 Wadsworth JDF, Joiner S, et al: Tissue distribution of protease
resistant prion protein in variant CJD using a highly
sensitive immuno-blotting assay. Lancet 358:171–180,

still very disgusted,

Terry S. Singeltary Sr. P.O. BOX 42 Bacliff, TEXAS USA

Terry S. Singeltary Sr. wrote:

> ##################### Bovine Spongiform Encephalopathy
> #####################
> United Press International: USDA vet: Texas mad cow breach not unique...
> Published 5/4/2004 5:01 PM WASHINGTON
> United Press International: USDA orders silence on mad cow in ...
> Published 5/11/2004 10:16 PM WASHINGTON
> Feds reviewing Texas mad cow breach - (United Press International)
> By Steve Mitchell United Press International.
> Washington, DC, May. 5 (UPI) -- The US Department ...
> No mad cow tests at Texas firm in 2004
> By Steve Mitchell
> United Press International
> Published 5/14/2004 11:06 AM
> WASHINGTON, May 14 (UPI) -- The U.S. Department of Agriculture did not
> test any cows for mad cow disease in the past seven months at the same
> Texas facility where federal testing policies for the deadly disorder
> were violated last month, United Press International has learned.
> USDA Ordered that Suspected Mad Cow in Texas Not Be Tested.
> USDA's San Angelo vets and techs ordered not to test suspect cow.
> by Daniel ...
> USDA Ordered that Suspected Mad Cow in Texas Not Be Tested
> USDA's San Angelo vets and techs ordered not to test suspect cow
> by Daniel Yovich on 5/5/04 for
> It was a trio of Agriculture Department staff < two veterinarians and one
> technician < who were supposed to follow agency protocol by testing what
> they determined was an older cow that likely had a central nervous system
> disorder when it arrived April 27 at the Lone Star Beef plant in San
> Angelo,
> Texas.
> One government source and another within the industry, both of whom
> say they
> have firsthand knowledge of events that day, said the final call on
> not to
> test the animal was made by an APHIS supervisor in Austin, Texas,
> after an
> APHIS technician at the plant advised her supervisor she was preparing to
> take a tissue sample from the culled animal for BSE testing. Both sources
> spoke to on condition of anonymity, and USDA
> officials did
> not return telephone calls Tuesday seeking comment and confirmation of
> the
> allegations.
> What USDA has confirmed is that the agency's standard operating
> procedures
> call for animals condemned due to a possible CNS disorder be kept until
> APHIS officials can collect samples for testing. That clearly was done in
> this case. The animal sat for more than 90 minutes and less than two
> hours
> after it was condemned, stunned and killed before the APHIS tech told
> Lone
> Star Beef management to dispose of the animal "in a routine manner."
> As a condemned cow, there was never any chance that the meat from the
> animal
> would enter the food chain. What is less clear is what went wrong at USDA
> and why.
> USDA spokesman Ed Loyd said the agency was conducting an investigation
> into
> the issue < attempting to establish a timeline and chronology of who was
> involved and who made the decisions last week in San Angelo.
> What is clear, in the mind of the two sources who spoke to
> , is that all three of USDA's key decision makers on the ground at the
> San
> Angelo plant were overruled by a staffer with more authority in Austin.
> "Everybody expected a test, and then the word came that there wasn't
> going
> to be any test," one source said. "I'm not sure why that decision was
> made,
> and I'm not going to speculate about the reasons for it. But I think what
> USDA is going to find is that the final decision was made up the food
> chain,
> and I think a lot of people will be interested in why that decision was
> made."
> Forums - Congressman Waxman's Letter and Texas Mad Cow
> June 12th, 2004, 01:59 PM, #1. Terry. Registered User. Join Date: Oct
> 2002. Location:
> Bacliff, Texas. Posts: 408. Congressman Waxman's Letter and Texas Mad
> Cow. ...
> Assigned vet wanted it tested.
> Gov. insp. over rided and decided not to test.
> SYSTEM broken around the Country.
> APHIS inspectors do not follow through.
> Statement
> May 4, 2004
> Media Inquiries: 301-827-6242
> Consumer Inquiries: 888-INFO-FDA
> Statement on Texas Cow With Central Nervous System Symptoms
> On Friday, April 30 th , the Food and Drug Administration learned that
> a cow with central nervous system symptoms had been killed and shipped
> to a processor for rendering into animal protein for use in animal feed.
> FDA's investigation showed that the animal in question had already
> been rendered into "meat and bone meal" (a type of protein animal
> feed). Over the weekend FDA was able to track down all the implicated
> material. That material is being held by the firm, which is
> cooperating fully with FDA.
> OOPS...
> Letter and Texas Mad Cow
> Forums > Books by PR Watch Staff > Mad Cow USA > Congressman Waxman's
> Letter
> and Texas Mad Cow. View Full Version : Congressman Waxman's ...
> -------- Original Message --------
> Date: Mon, 22 Nov 2004 17:16:23 -0600
> From: "Terry S. Singeltary Sr."
> Reply-To: BSE-L
> BSE ( test results ;-) Texas Animal Health Commission News Release ...
> Counting that one and the other positive, positive, inconclusive, and
> finally
> declared and documented as negative cows (NO WB), the USA in my ...
> Re: ''INCONCLUSIVE'' IS NEGATIVE or so they claim...OFFICIAL ...
> would not be telling us of any 'inconclusive', but > they ... because
> the likelihood >
> of it > being positive was very ... tell you why, they wanted a
> negative so bad ...
> Forums - Mad Cow USA
> ... 2nd Positive Inconclusive Negative For Bse Usa; 1st Positive
> Inconclusive Is Negative; ...
> ... Dec 2, that IHC- DOES NOT MEAN IT IS NEGATIVE. ... so many
> errors (i am assuming X meant
> inconclusive), why are ... at the sheep that tested IHC- but were
> positive''. ...
> CJD WATCH... positive and histopathology and immunohistochemistry
> negative) with the ... 2005 positive
> The case was confirmed on ... Origin of infection: unknown or
> inconclusive. ...
> ... Date: February 1, 2005 at 3:08 pm PST. In Reply to: Re: BSE
> FROM TEXAS ??? posted by TSS on November 19, 2004 at 9:41 am: ...
> Forums - View Single Post - Re: MAD COW CONFIRMED TEXAS COW (rumor ......
> Harrison >> November 22, 2004 >> >> Test results for the BSE
> inconclusive are not ...
> was just this...damn, >> i will not sleep tonight/// >> TSS >> >>
> Forums - Bse Usa 'inconclusive' Test Reported Nov. 18, 2004
> ... Because this test is only an inconclusive test result, we ...
> animal presented for slaughter is sampled for BSE, holding the ...
> tss USDA News
> Nebraska Outdoor Forum: Study of Atypical Bse Project Number: 3625
> .. TSS Terry S ... mice from the
> experimental cow brain
> had been inconclusive. ... clinical signs of brain lesions
> characteristic of BSE. ...
> ... that the USA is now facing, an epidemic of > ''INCONCLUSIVE''
> TSEs...TSS > > Terry
> S ... 5:15 this evening, we were notified that an >> inconclusive BSE
> test result ...
> -------- Original Message --------
> Date: Wed, 24 Mar 2004 16:12:06 -0600
> From: "Terry S. Singeltary Sr."
> Reply-To: BSE-L
> The US plans to measure the incidence
> of mad cow disease in its cattle with a
> test that its own officials have said gives
> too many false positives. Some experts
> fear the choice reflects an official desire
> to downplay the impact of the first
> positive BSE tests that emerge, when
> they turn out not to be confirmed.
> Last week the US Department of
> Agriculture (USDA) approved two tests,
> including one made by the Californian
> firm BioRad, for screening up to 300,000
> cattle for BSE, starting in July. No more
> tests will be licensed for months.
> Announcing the testing plan, chief
> veterinary officer Ron DeHaven cautioned
> that "there will be positive results",
> many of them false.
> BioRad's antibody-based test for the
> prion protein that causes BSE has given
> numerous false positives in Belgium and
> Germany. And in Japan only 8 of 113 cattle
> that repeatedly tested positive with
> BioRad were confirmed by slower tests
> that do not give false positives.
> The USDA even wrote last May that
> "it is well known" that tests like
> BioRad's give false positives. It states
> that other kinds of quick tests are more
> suitable for testing for very low levels of
> BSE, which are expected in the US.
> The second quick test approved by
> the USDA, made by Maine-based IDEXX,
> could also in theory give false positives.
> It remains unclear how reliable it is,
> because there has been little practical
> experience with the test so far. It is not
> yet approved for use in Europe, where
> the vast majority of BSE tests are done.
> Debora MacKenzie,
> Brussels correspondent,
> New Scientist.
> tel +32-2-245-0412
> fax +32-2-245-0552
> mobile +32-49-754-0444
> =======================
> Greetings,
> odd that the USDA et al approves two US-OWNED tests that are
> _known_ to give false positives, when they know other rapid
> TSE test are much more reliable. IT's like they purposely do
> not want to find any TSE in the USA bovine, so they pick the
> worst test available. The USDA own experts think BioRad is
> not suitable for supposedly BSE/TSE free and low incidence
> areas, so why did they choose this test and or the IDEXX,
> which i dont think has even been submitted to the EU for evaluation
> and has no commercial experiance to my knowledge. You could
> almost get the feeling they are deliberately skipping over
> Prionics for the least supperior TSE rapid test. I believe
> the Canadians finally did choose prionics. maybe paul or marcus
> might comment? seems if North America is going to be a
> consolidated BEEF trading market amongst themselves and expect
> to export there tainted products everywhere, they could at least
> come up with the same TSE rapid Test. how can one use a less
> reliable test and the other use a more reliable test, and it
> all be the same? i know there is a word Dehaven used, but it
> slips my mind now, (consolidated markets) that's not it,
> but you get the just of my thoughts, i think;-)...TSS
> ----- Original Message -----
> From: "Terry S. Singeltary Sr."
> To: BSE-L
> Sent: Wednesday, June 30, 2004 6:57 PM
> > greetings list members,
> >
> > > -------- Original Message --------
> > > Date: Sat, 26 Jun 2004 13:55:42 -0500
> > > From: "Terry S. Singeltary Sr."
> > > Reply-To: BSE-L
> > > To: BSE-L
> > > References:
> > >
> > >
> > snip...
> >
> > > on the other, i wonder if this is another faked incident like the
> feed
> > > bag
> > > event in texas a couple of years back ("the system worked!").
> surprise
> > > surprise this one won't be confirmed. in essence, a drill to train
> > > trading
> > > partners not to respond to a positive test kit result. dull the
> > > response of
> > > media and public. a steady drumbeat of "inconclusive" positives and
> > > anticlimatic followups 4-7 days later (say friday pm before 4th of
> july
> > > weekend) of which occasionally one will be positive as "expected" so
> > > as not
> > > to be newsworthy.
> > >
> > > the lack of detail makes it impossible for the press to follow up,
> > > "refused
> > > to identify if the
> > > suspect animal was a cow or a steer, its age, location or any other
> > > information. not going to be any tv crews swarming around a
> > > slaughterhouse
> > > or interviewing another dave lothan. total control. just a statistic.
> > >
> > > problem solved...
> > >
> >
> >
> > snip...
> >
> >
> >
> > >i don't share your view (patty hearst syndrome?) that usda has been
> > >transparent or honest. how could they be unaware, during the long
> selection
> > >process leading to BioRad, of the very low false positive rates
> observed in
> > >Europe, yet the chief guy at usda has repeatedly turned the rates
> > >completely upside down, from 1 in 1000 to 999 in a 1000 for a biorad
> > >positive being confirmed positive.
> > >
> > >while i don't know how many false positive or total tests japan has
> done,
> > >the rates you cite from japan are not consistent with europe or
> usda. even
> > >at face value, you are quoting a 1 in 5 chance of confirmation. with 2
> > >cows, that is 16/25 of both being negative or 9/25 of one or more true
> > >positives, that's 36%, making a liar out of the usda guy (who is
> not a pr
> > >person but way up in the professional staff).
> > >
> > >for a $20 rapid test kit it makes sense to run a presumptive positive
> > >another couple of times the same day. this lowers the rate to 1 in
> 100,000
> > >without the ridiculous 4-7 day delay which in my opinion is solely
> intended
> > >to make yet another Friday pm announcement on the biggest meat buying
> > >weekend of the year (since they can't stall until christmas eve
> this time)
> > >plus give them 3-6 days to ramp up their pr engine plus tip off
> friends in
> > >the commodities pit again.
> > >
> > >i think it is a little manipulative not to disclose the ages of the
> cow and
> > >whether they are from the same test lab. like the market is not making
> > >speculation now?
> > >
> > >it is very very clear to me that they do not want to test large
> numbers of
> > >cows in the manner of japan and europe. this is not because of kit
> > >economics but because every last country that has done so, has
> found higher
> > >numbers than their ag agencies had ever indicated possible.
> > >
> > >while we can wait for their next announcement, the truth is we have
> no idea
> > >whether a non-confirmation will be the truth because testing is a
> totally
> > >closed agency shop, eg Creekstone.
> > >
> > > they would never never never allow a university lab like
> prusiner's to get
> > >their hands on this sample. why don't you throw your weight behind
> getting
> > >some sample retested in europe with biorad and prionics and by
> prusiner,
> > >just to restore confidence in usda?
> > >
> > >i do feel it is possible for there to be glitches in start-up with
> so new
> > >many labs getting going, though i am not aware of anything technically
> > >groudnbreaking, quite the contrary, about the biorad tests
> > >
> > >
> > >
> > >
> > >
> >
> > NOW, why are we using the BIO-RAD _if_ PRIONICS is better?
> >
> > OR maybe PRIONICS is not as complicated as BIO-RAD?
> >
> > either way, we have some 8,585 (BSE-expanded) test so far and the
> > 1st of 2 positive ''inconclusives'' in the 1st month is negative.
> OH, don't
> > forget about the mad cow in TEXAS, that don't count though?
> > something seems terribly wrong here.
> >
> > TSS
> >
> >
> > Terry S. Singeltary Sr. wrote:
> >
> > >
> > >
> > > Release No. 0272.04
> > >
> > > Contact:
> > > USDA Press Office (202) 720-4623
> > >
> > >
> > >
> > > Statement By Deputy Administrator Dr. John Clifford For The Animal
> And
> > > Plant Health Inspection Service
> > >
> > > June 30, 2004
> > >
> > > At approximately, 3:45 p.m. today, we were notified by the USDA
> > > National Veterinary Services Laboratories (NVSL) in Ames, Iowa
> that the
> > > inconclusive screening test sample reported on June 25, tested
> negative
> > > for BSE upon confirmatory testing.
> > >
> > > NVSL used the world-recognized gold-standard test for BSE, the
> > > immunohistochemistry test to confirm this finding.
> > >
> > >
> > >
> > >
> > >
> simply amazing that countries are willing to expose there people to
> all this TSE...
> Terry S. Singeltary Sr. wrote:
>> ##################### Bovine Spongiform Encephalopathy
>> #####################
>> EMBARGOED for release
>> 9 a.m. (EST) Thursday, Feb. 24, 2005
>> Consumers Union asks feds to retest suspect mad cow after crucial
>> test omitted
>> USDA urged to follow internationally recognized procedures
>> WASHINGTON, DC. -- Consumers Union, publisher of Consumer Reports,
>> today asked the U.S. Department of Agriculture to retest a cow
>> suspected in November 2004 of having mad cow disease, using a
>> critical, internationally recognized test that the agency failed to
>> use. The test, called the Western blot, is used by authorities in
>> Japan and Europe when making a final determination as to whether a
>> suspect cow has the fatal brain-wasting affliction, which can be
>> passed on to humans.
>> A Consumers Union delegation met earlier this month with USDA
>> officials and today issued a letter to USDA Secretary Mike Johanns
>> urging the agency to revise its testing methods. CU is asking the
>> agency to retest the November cow using the Western blot and to send
>> samples from the cow to the United Kingdom for an independent
>> evaluation.
>> Given the potential consequences to both public health and the
>> cattle industry if this brain-wasting disease were to become
>> established here, it is extremely important that every scientifically
>> justifiable step be taken to prevent it, said the letter signed by
>> Michael Hansen, PhD., a biologist with Consumers Union and
>> spokesperson for its campaign and Jean Halloran,
>> director of CUs Consumer Policy Institute.
>> The USDA limited its confirmatory testing in November 2004 to the
>> immunohistochemistry (IHC) test, which it describes as the gold
>> standard. The result of the IHC test was negative. USDA did not
>> perform the Western blot test, even though it had previously used
>> both IHC and the Western blot test in confirming the first U.S. case
>> of mad cow disease, from Washington State in December, 2003. The USDA
>> also sent material from the 2003 Washington State cow to the United
>> Kingdom for further review of its results.
>> Scientists in Japan and Belgium have reported that suspect cows may
>> be negative on the IHC and still register as positive on the Western
>> blot. Such cows are universally regarded as infected.
>> The IHC test is more subjective than the Western blot test, relying
>> on the judgment of a skilled scientist is assessing the appearance of
>> thin slices of brain material under a microscope, Hansen said. The
>> Western blot test is more objective, with results that can be read by
>> any technician. In the U.S., the IHC test is performed by a USDA
>> scientist at a USDA laboratory in Ames, Iowa.
>> The cow USDA assessed in November 2004 had come up as suspect for
>> mad cow disease in two runs of the Biorad quick test. The Biorad
>> test has been used to screen over 200,000 cows for mad cow disease
>> since USDA began a new testing initiative in July 2004. However, all
>> international authorities agree that the Biorad screening test can
>> give a false positive result. Thus it must be confirmed by other
>> tests. CU urges USDA to use both Western blot and IHC for confirmation.
>> The USDA should operate out of an abundance of caution in its
>> efforts to keep the U.S. food supply safe from (mad cow disease),
>> the letter to Johanns stated. The experience of the United Kingdom,
>> where millions of cattle have been destroyed, beef exports blocked
>> for many years and 147 people have died, painfully demonstrates the
>> consequences of insufficient action to prevent the spread of mad
>> cow disease.
>> # # #
>> Consumers Union, publisher of Consumer Reports, is an independent,
>> nonprofit testing and information organization serving only the
>> consumer. We are a comprehensive source of unbiased advice about
>> products and services, personal finance, health nutrition, and other
>> consumer concerns. Since 1936, our mission has been to test products,
>> inform the public, and protect consumers.
>> ===================================
>> February 24, 2005
>> Hon. Mike Johanns
>> Secretary of Agriculture
>> US Department of Agriculture
>> 1400 Independence Avenue, SW
>> Washington, DC 20250
>> Dear Secretary Johanns:
>> We appreciate having had the opportunity to meet with you on February
>> 9, 2005 with the Food Safety Coalition. Because we had limited time
>> on that occasion, we are following up with a letter explaining our
>> concerns on one issue that is especially important to Consumers
>> Union: USDAs bovine spongiform encephalopathy (BSE), commonly known
>> as mad cow disease, testing procedures.
>> For reasons we explain below, we urge USDA to expand its testing
>> protocol to bring it in line with those of Europe and Japan, by
>> including a test called the Western blot when evaluating cows
>> suspected of mad cow disease, such as the suspect cow identified in
>> November, 2004. Under current USDA testing protocols, it is possible
>> that USDA will miss cases of mad cow disease that could be confirmed
>> through additional testing.
>> As Secretary of Agriculture, you face the important and very
>> difficult responsibility of preventing mad cow disease in the United
>> States. Given the potential consequences to both public health and
>> the cattle industry if this brain-wasting disease were to become
>> established here, it is extremely important that every scientifically
>> justifiable step be taken to prevent it. It is especially critical to
>> understand to what extent the disease may already be present in the
>> United States, now that one case was discovered in Washington State
>> in December, 2003.
>> As you know, the USDA has tested some 230,000 cattle since June,
>> 2004, for mad cow disease. Although this seems like a large number,
>> it is still less than 1% of the 35 million cattle slaughtered
>> annually in the US. The number of cows tested should be increased.
>> Consumers Union also believes that USDAs testing protocol should be
>> expanded. USDAs testing protocol specifically does not include the
>> Western blot test (accompanied by a sodium phosphotungstinic acid
>> [NaPTA] precipitation step), a test used by all European Union
>> countries and Japan. When a cow in the USDA testing program is
>> considered suspect as a result of positives in two runs of the Biorad
>> quick test, as happened in November, 2004, it is sent to the USDA
>> Ames Iowa laboratory for further evaluation. That evaluation includes
>> only an immunohistochemistry test (IHC), which USDA refers to as
>> the gold standard. We disagree with that characterization. Recent
>> studies in Belgium and Japan have shown that the IHC test misses some
>> cases of mad cow disease. A letter in last months Veterinary
>> Pathology, from one of the worlds leading authorities on mad cow
>> disease testing, pointed out that the Western blot, when accompanied
>> by the NaPTA step, is far more sensitive than IHC in detecting the
>> mad cow disease infectious agent .
>> In fact, USDA used both the IHC and Western blot tests to confirm its
>> first case of mad cow disease, in December 2003. According to a USDA
>> publication, the Western blot test was crucial to identifying that
>> case. It is thus difficult to understand why USDA did not again use
>> the Western blot test along with IHC on its second suspect cow.
>> We therefore urge you to go back and retest--using the Western blot
>> test with the NaPTA step--the suspect cow that was identified in
>> November, 2004, and to send appropriate material from that cow to the
>> United Kingdom laboratories for an independent evaluation. We further
>> urge you to revise USDA policy and routinely use the Western blot
>> test with the NAPTA step, as well as the immunohistochemistry (IHC)
>> test, for confirmation of suspect mad cow cases.
>> The USDA should operate out of an abundance of caution in its
>> efforts to keep the US food supply safe from BSE. The experience of
>> the United Kingdom, where millions of cattle had to be destroyed,
>> beef exports were blocked for many years, and 147 people have died,
>> painfully demonstrates the consequences of insufficient action to
>> prevent the spread of mad cow disease.
>> The trust of American consumers, and of foreign markets, in the
>> safety of American beef, rests on having confidence that USDA is
>> utilizing the best science available, comparable to that used in
>> other scientifically advanced countries.
>> A copy of a prior letter on this subject, which we sent to Dr. John
>> R. Clifford, Deputy Administrator of the Animal Plant Health
>> Inspection Service, is attached and we understand is in the process
>> of being answered.
>> We look forward to hearing from you about whether you will direct
>> USDA to retest the November 2004 suspect cow and revise USDA policy
>> to routinely use the Western blot as well as IHC on all suspect animals.
>> Sincerely,
>> Jean Halloran, Director Michael K. Hansen, Ph.D.
>> Consumer Policy Institute Senior Research Associate
>> Cc: Dr. John R. Clifford
>> =====================================
>> ooops...
>> #########
>> ##########
> #########
> ##########


Variant Creutzfeldt-Jakob Disease

Ironside JW, Head MW, Knight R, Ward H
National CJD Surveillance Unit, University of Edinburgh, Edinburgh, UK

Variant Creutzfeldt-Jakob disease (vCJD) is a novel human prion disorder
which on the basis of experimental
strain typing appears to result from human exposure to the bovine
spongiform encephalopathy (BSE) agent,
probably by the consumption of BSE-contaminated meat products. This
disorder tends to present in young
adults (median age 28 y) as a psychiatric disorder often accompanied by
sensory abnormalities and later
followed by ataxia, myoclonus and other movement disorders. Death occurs
after a median duration of illness
of 13 months. MRI brain scans have shown that abnormal areas of high
signal occur in T2 and proton density
images in the posterior thalamus. All patients with vCJD have been
homozygous for methionine at codon 129
in the PrP gene. Pathologically, vCJD is characterised by florid plaques
in the cerebrum and cerebellum, and
by the presence of a PrP isotype (2B), which is distinct from other
human prion diseases. vCJD also differs
from other human prion disorders in that abnormal PrP is widely
distributed outside the nervous system in
lymphoid tissues. This has given rise to concerns that vCJD may be
transmitted by surgical instruments used
on these tissues, even if infectivity appears to be at lower levels than
in the brain. 2 cases of iatrogenic vCJD
infection have recently been reported following blood transfusion from
donors who subsequently developed
vCJD, one of whom was a heterozygote at codon 129 in the prion protein
gene and died before the onset of
clinical neurological disease. There are considerable uncertainties over
future numbers of vCJD cases in the
UK, since clinical cases appear to be declining in frequency, but
retrospective studies of PrP accumulation in
tonsils and appendix tissue have suggested that a few thousand
individuals may be incubating the disease in
the UK. Continuing surveillance is required in the UK and in other
countries where BSE has been identified.



Richard Knight
Clinical Neurologist, National CJD Surveillance Unit, UK

160 cases of variant CJD have been identified (149 in the UK). The
clinico-pathological profile of human prion
diseases is influenced by PRNP codon-129 genotype, prion ‘agent strain’
(or prion protein type) and mode of
acquisition. All tested cases of variant CJD have occurred in one
genotype (codon 129 MM), with one
presumed agent strain/one protein type and one probable cause: BSE
dietary contamination (but for one
instance of possible blood transmission). Therefore, it is perhaps
unsurprising that variant CJD has a relatively
uniform clinical picture, unlike the significant clinico-pathological
heterogeneity of sporadic CJD. Also unlike
sporadic CJD, it is predominantly affects the young with a relatively
long duration (median ages at onset:
vCJD 26 years, sCJD 66 years; median durations: vCJD14 months, sCJD 4
months in sporadic CJD).
Sporadic CJD typically presents in a clearly neurological way, most
often with a rapidly progressive dementia.
Variant CJD typically presents with a psychiatric or behavioural
disturbance; its essentially neurological nature
may not be apparent for some months.
Social withdrawal, loss of interest, dysphoria, anxiety, irritability
and insomnia are common early symptoms;
depression being a frequent initial diagnosis.
Sensory symptoms (typically unpleasant) are reported in around two
thirds of cases. Specifically neurological
signs appear at a median of around 6 months; memory/cognitive
dysfunction and cerebellar ataxia being the
commonest features.
Other neurological features include: pyramidal signs, and involuntary
movements (chorea, dystonia, tremor,
Definite diagnosis requires neuropathology. The clinical diagnosis of
variant CJD rests on: a clinical suspicion
of the condition, the exclusion of other possible diagnoses and the
appropriate use of supportive diagnostic
tests. The most useful non-invasive investigation is the cerebral MRI,
showing the so-called ‘Pulvinar sign’ in
most cases. Tonsil biopsy is useful in some.



Nikolai G Rainov 1,2
1 Department of Neurological Science, The University of Liverpool, and
2The Walton Centre for Neurology
and Neurosurgery NHS Trust, Liverpool, UK

Transmissible spongiform encephalopathies (TSE) are diseases believed to
be caused by accumulation of an abnormal
isoform of the prion protein (PrPsc) in the central nervous system.
There are sporadic, aquired and hereditary TSE.
Creutzfeld-Jacob disease (CJD) in its sporadic and variant form is the
most frequent and clinically important TSE. At
present there is no proven specific or effective treatment available for
any form of CJD, although drugs such as
quinacrine are being investigated in early clinical trials.
Pentosan polysulphate (PPS), a large polyglycoside molecule with weak
heparin-like activity, has been shown to prolong
the incubation period of PrPsc infection when administered to the
cerebral ventricles in a rodent scrapie model. PPS also
prevents the production of further PrPsc in cell culture models.
However, PPS penetrates poorly the blood-brain barrier
and only a minor fraction of orally administered drug may reach the CNS.
These properties of PPS prompted its cerebroventricular administration
in a total of 8 patients with vCJD and other TSE,
such as iatrogenic CJD and Gerstmann-Sträussler-Scheinker syndrome
(GSS). Long-term continuous infusion of PPS at
doses from 11 µg/kg/day to 110 µg/kg/d did not cause any drug-related
side effects. Follow-up CT and MRI imaging
demonstrated that brain atrophy may progress during PPS administration.
Proof of clinical efficacy has not been the aim
of these early studies, however one patient with vCJD survived for 37
months after initial symptoms and 30 months after
diagnosis, while the median duration of illness with vCJD is 13 months
(range 6-39).
Some lessons have been learned from the first cases. Surgery in a brain
affected by TSE may result in a higher rate of
surgical complications than might be expected in analogous cases without
TSE. Secondly, if clinically significant benefits
are to be expected, PPS administration should start as early as possible
in the course of the respective disease and
before irreversible loss of neurological function has occurred.
Further clinical, neuroradiological and laboratory investigations in the
setting of a prospective clinical study with
standardised follow-up protocol and data collection are essential in
order to assess the efficacy of PPS administration in
vCJD and in other TSE.


Peripheral Pathogenesis of Human Prion Diseases

Markus Glatzel1, Eugenio Abela1, Nicolas Genoud1, James Ironside2, and
Adriano Aguzzi
1Swiss National Reference Center for Prion Diseases, University Hospital
of Zürich, CH-8091 Zürich, Switzerland
2The National Creutzfeldt-Jakob Disease Surveillance Unit, Western
General Hospital, Edinburgh EH4 2XU, UK

Ante-mortem diagnosis of human prion diseases has been attempted by
brain biopsy, but is complicated by
insurmountable biosafety problems, is highly invasive, and is thus
generally considered obsolete in the
diagnosis of a human prion disease. As a consequence, there is currently
no minimally invasive ante-mortem
test available to confirm the clinical suspicion of a human prion
disease or to discriminate prion strains. By
studying the precise distribution of disease-associated prion protein
(PrPSc) in sporadic Creutzfeldt-Jakob
disease we have identified muscle as a putative target for the diagnosis
of Creutzfeldt-Jakob disease. These
investigations are supplemented by studies employing genetically
modified mice aimed unraveling the
molecular basis of muscular prion replication.


Further advances in the molecular and pathological diagnosis of
sporadic Creutzfeldt-Jakob disease subtypes.

Piero Parchi, Silvio Notari, Rosaria Strammiello, Sabina Capellari.
Laboratory of Neuropathology, Department of Neurological Sciences,
University of Bologna, Italy.

The characterization of at least six clinico-pathological phenotypes of
sporadic Creutzfeldt-Jakob disease
(sCJD) which largely correlate at the molecular level with the genotype
at codon 129 (MM, MV, VV) and either
one of two major types of PrPSc 27-30 with distinct physicochemical
properties (i.e. type 1 and type 2) has
provided the basis for a molecular classification of sCJD, and a
potentially powerful method for strain typing.
Despite the significant advances, however, additional work needs to be
to done to fully explore the whole
spectrum of sCJD variants and the issue of the molecular basis of
phenotypic variability in sCJD. The
discovery of subjects with the co-occurrence of PrPSc type 1 and type 2
constitutes a potential drawback for
the widespread application of this classification to CJD diagnostics and
epidemiology. Furthermore, there is
still some disparity among laboratories regarding the understanding and
nomenclature of PrPSc types and it is
still unknown whether a specific PrPSc type is associated with each sCJD
phenotypic variant. Lastly, the recent
identification of novel C-terminal fragments of PrP (PrP-CTF) has
provided a potential novel molecular marker,
but it is currently unclear to what extent PrP-CTF properties correlate
with the sCJD phenotype. We report on
our recent results on the above issues. I) Studying in detail a large
series of cases we found that the
co-occurrence of types 1 and 2 is not random and characterizes about 30%
of MM cases and 20-25% of the
sCJD population as a whole. Our data show that a correct molecular
classification can be reached in the
absolute majority of sCJD cases, but requires pathological and
biochemical analyses of 6-8 samples from the
cerebral cortex, thalamus and cerebellum. II) We further analyzed PrPSc
27-30 properties using a high
resolution gel electrophoresis system and varying experimental
conditions and found that pH varies among
CJD brain homogenates in standard buffers thereby influencing the
characteristics of PrPSc 27-30. We also
found that PrPSc 27-30 type 1 and type 2 are heterogeneous species,
which can be further distinguished into
molecular subtypes that fit the current histopathological classification
of sCJD variants. III) Finally, our latest
results indicate that PrP-CTF characterization is also useful for the
molecular diagnostics of some sCJD
subtypes. Supported by the Italian Ministry of Health (Ricerca
Finalizzata 1%/2001), the EU contract
QLK3-CT-2001-02345, and the G. Galletti Foundation.


Chronic Wasting Disease in Cervids in North America

Elizabeth S. Williams
University of Wyoming

Chronic wasting disease (CWD) is a transmissible spongiform
encephalopathy of free-ranging and farmed
cervids in North America that is distinct from scrapie of domestic
sheep, bovine spongiform encephalopathy,
and Creutzfeldt-Jacob disease of humans. The purpose of this paper is to
review the current status of CWD in
North America. The natural host range of CWD includes mule deer
(Odocoileus hemionus), white-tailed deer
(Odocoileus virginianus), and Rocky Mountain elk (Cervus elaphus
nelsoni). Experimentally, by intracerebral
or oral exposure, the host range is wider, but there appears to be a
significant barrier to infection of cattle and
humans. The exact mechanism of CWD transmission is not known but recent
studies indicate that direct
transmission, indirect transmission via environmental contamination, and
transmission associated with
carcasses are possible. Maternal transmission does not appear to play a
significant role in CWD. Although still
under investigation, polymorphisms in the prion protein influence CWD
pathogenesis in mule deer and elk.
Studies of CWD pathogenesis following oral exposure demonstrate early
widespread distribution of abnormal
prion protein in the lymphoid tissue prior to invasion of the central
nervous system. These data have lead to
techniques for CWD surveillance in deer based on testing retropharyngeal
lymph node. However, both brain
and lymph node must be tested in elk for highest sensitivity due to
differences in pathogenesis in this species
compared to deer. The unique nature of a transmissible spongiform
encephalopathy occurring in free-ranging
cervids is a serious challenge to wildlife managers and animal health
agencies in North America.


Bovine spongiform encephalopathy (BSE) in Japan

Takashi Yokoyama, Kumiko M. Kimura, Morikazu Shinagawa
Prion Disease Research Center, National Institute of Animal Health, Japan

Bovine spongiform encephalopathy (BSE) has become an important problem
not only for animal industry, but
also for public health. In Japan, BSE was first recognized in September
2001 by fallen stock surveillance.
Since October 2001, BSE examination for all cattle slaughtered at
abattoirs has started. In April 2004, all dead
cattle examination (over 24 months) has been conducted at livestock
hygiene service center. Samples positive
in enzyme linked immunosorbent assay (ELISA) are further subjected to
western blot (WB) and
immunohistochemistry (IHC). Thirteen BSE cases have been reported by
September 2004. Twelve cases
were classified as typical BSE, and the remained one was an atypical
BSE. Variant forms of BSE with atypical
histopathological and/or biochemical phenotype were reported in Italy
and France. Further study is required
for BSE prion characteristics.
To characterize BSE prion properties, brain homogenates of Japanese BSE
cases were intracerebrally
inoculated into wild-type mice. The first case (BSE/Chiba) was
successfully transmitted to rodents. The mean
incubation periods (409.0 days) in this experiment was preferably longer
than that of previously reported.
PrPSc distribution, prion titer, mice susceptibility and/or storage
condition of sample might be influenced the
result. Recently, we introduced transgenic mice that overexpress a
bovine PrP gene to overcome the species
barrier problem. These mice are expected to accelerate the transmission
experiment of BSE prion.
Transmission of atypical BSE case is undergoing by using these
transgenic mice.


How does host PrP control TSE disease?

Jean Manson1, R Barron1, N Tuzi1, H Baybutt1, Enrico Cancellotti1, P
Hart1, L Aitchison1, B.Bradford1, D King1 , R Moore2, D
Melton2, M Bishop3, J Ironside3, R Will3
1Institute for Animal Health, Neuropathogenesis Unit, Edinburgh, UK,
2University of Edinburgh, 3National CJD Surveillance
Unit, Edinburgh

PrP is central to the TSE disease process and has been hypothesised to
be the infectious agent.
Polymorphisms in the PrP gene of a number of species are associated with
different incubation times of
disease following exposure to an infectious agent and mutations in the
human PrP gene can apparently lead
to spontaneous genetic disease. Strains of TSE agent are proposed to be
generated and maintained through
differences in glycosylation or conformation of PrP and the barrier to
infection between species is thought to
be due to the differences in the sequence of PrP between different species.
In order to test these hypotheses, we have introduced specific
modifications into the endogenous mouse
Prnp gene by gene targeting. The mutated PrP gene is in the correct
location under the control of the
endogenous Prnp regulatory sequences and thus expressed in the same
tissues and amounts as the wild type
Prnp gene. This strategy therefore allows the effect of specific
mutations in the PrP gene to be assessed.
By altering the murine PrP coding region to that of another species we
have established that increasing
overall identity between host and donor PrP can lead to either an
increase or a decrease in incubation time of
disease in a strain dependent manner. We have introduced a point
mutation (101L) into the N-terminus of the
host PrP and shown that it dramatically changes the susceptibility of
the host to infection from different
species. We have in addition demonstrated that polymorphisms in the N
terminus (L108T) and C-terminus
(F189V) of host PrP both alter the incubation time of disease but each
operates by a different mechanism.
We have introduced mutations into the Prnp gene which prevent
glycosylation at each or both of the two
N-linked glycosylation sites of PrP. Inoculation of these mice with
infectivity has established that glycosylation
of host PrP can influence incubation time of disease, vacuolar pathology
and strain determination. We have
during these studies produced a model of TSE disease which contains high
levels of infectivity in the apparent
absence of PrPSc and we are using this model to define the nature of the
infectious agent.
We have thus established that the gene targeting approach can produce
models for TSE disease which
address fundamental questions associated with these diseases. We aim to
use these models to address
central issues including the origin of strains, the species barrier and
the nature of the infectious agent.


Prion interactions with the immune system
Neil A Mabbott
Institute for Animal Health, Edinburgh, UK

Many natural prion infections are likely to be acquired peripherally for
example, following ingestion of
prion-contaminated feed. Following peripheral exposure prions accumulate
in lymphoid tissues before
spreading to the brain. Using mice experimentally infected with scrapie
prions we have shown that mature
follicular dendritic cells (FDCs), expressing the host prion protein
(PrPc), are critical for replication of infection
in lymphoid tissues. Prion neuroinvasion is also dependent on FDCs as in
their absence disease
susceptibility is reduced. For example, temporary depletion of FDCs
before oral inoculation with prions
blocks the accumulation of disease-specific PrP in Peyer’s patches and
mesenteric lymph nodes, and
prevents neuroinvasion.
Studies in mice have shown that skin scarification is also an effective
means of prion transmission.
Following inoculation via the skin scrapie prions accumulate in the
draining lymph node in association with
FDCs. The accumulation of prions in association with FDCs is also
critical for the transmission of disease
from the skin to the brain, as disease susceptibility is reduced in
their absence. The mechanisms through
which prions are transported from the skin to lymphoid tissues are not
known. Langerhans cells (LCs) reside
in the epidermis and migrate to the draining lymph node after
encountering antigen. Our studies show that
LCs have the potential to acquire and degrade PrPSc following in vitro
exposure. To investigate the potential
role of LCs in prion transportation from the skin, we utilized mouse
models in which their migration was
blocked. We show that the early accumulation of prions in the draining
lymph node and subsequent
neuroinvasion was not impaired in mice with blocked LC migration. These
data therefore demonstrate that
although LCs have the potential to acquire prions they are not involved
in their transportation to draining
lymphoid tissues.
Thorough analysis of the early events in prion pathogenesis in lymphoid
tissues may identify potential
targets for therapeutic intervention.


Scrapie infection of SN56 cells: greater efficiency by
membrane-associated versus purified PrP-res
Gerald S. Baron1, Ana C. Magalhães2, Marco A.M. Prado2, and Byron Caughey1
1Rocky Mountain Laboratories, Laboratory of Persistent Viral Diseases,
NIAID, NIH, 903 S. 4th St., Hamilton, MT 59840

2Program of Molecular and Biochemical Pharmacology, Department of
Pharmacology, ICB, Universidade Federal de Minas
Gerais, Av. Antonio Carlos 6627, 31270-901, Brazil
The process by which transmissible spongiform encephalopathy (TSE)
agents, or prions, infect cells is
unknown. We employed a new highly susceptible cell line (SN56) and a
previously described cell line (N2a)
to gain insight into the mechanism of infection. The effect of
disease-associated PrP (PrP-res) association
with membranes on infection efficiency was examined by comparing
sustained PrP-res production in cells
treated with either scrapie brain microsomes or purified,
detergent-extracted PrP-res. When normalized for
quantity of input PrP-res, scrapie brain microsomes induced dramatically
enhanced persistent PrP-res
formation compared to purified PrP-res. Infected SN56 cells released low
levels of PrP-res into the culture
supernatant, which also efficiently initiated infection in recipient
cells. Interestingly, microsomes labeled with
a fluorescent marker were internalized by SN56 cells in small vesicles,
which were subsequently found in
neuritic processes. When bound to culture wells to reduce
internalization during the infection process,
scrapie microsomes induced less long-term PrP-res production than
suspended microsomes. Our
observations suggest that efficient infection of cells may involve a
transfer and/or internalization of membranes
containing PrP-res.


Slow dynamics of prion protein
Kazuo Kuwata
Division of Prion Research, Center for Emerging Infectious Deseases
(CEID), Gifu University

Although the conformational conversion mechanism from cellular (PrPC) to
scrapie (PrPSc) form of animal
prion proteins has not yet been elucidated, much evidence is
accumulating that potentially provides insights
into the conversion process at atomic resolution. We characterized the
critical aspects of the slow fluctuation
dynamics of the recombinant hamster prion protein, rPrP(90-231), based
on NMR relaxation analysis using
Carr-Purcell-Meiboom-Gill (CPMG) experiments, and compare them in detail
with the results from
high-pressure NMR. Residues exhibiting slow fluctuations on the time
scale of microseconds to milliseconds
are mainly localized on helices B and C (172-193 and 200-227), which
include locally disordered regions in an
intermediate conformer, PrP*, identified previously by high pressure NMR
(Kuwata et al., Biochemistry
12277-12283 (2002)). Moreover, chemical shift differences between two
putative exchanging conformers
obtained by the CPMG relaxation analysis and the linear component of the
pressure-induced chemical shift
changes are well correlated at individual residue sites. These
observations apparently support the notion that
both the CMPG relaxation and the pressure shifts represent slow
conformational fluctuations, and that these
slow motions in PrPC are fundamentally on the trajectories leading to
the transition to PrP*. Furthermore the
slow conformational exchange rates were not in proportion to the square
of the static magnetic field, indicating
that the slow dynamical trajectory of the prion protein is not simply
described by the semiclassical periodic
approximation, but includes bifurcation and/or singularities. Those
abnormal dynamical characterstics of prion
may be related to its pathogenicity.

Department of Prion Research
Tohoku University School of Medicine
2-1 Seiryo-cho Aoba-ku, Sendai 980-8575, JAPAN
Tel: +81-22-717-8233
Fax: +81-22-717-8148

Japan Consumer Press online
Nippon shouhisha shinbun
Last modified, 11/09/2004 13:42:49
BSE death cow's anomalous prion detected from peripheral nerve tissue,
suprarenal gland
First time from non-Specified Risk Material, or SRM
National Institute of Animal Health Animal announced on November 1 that
it had detected the anomalous prion protein that was the etiologic agent
of the mad cow disease, or BSE, or bovine spongiform encephaalopathy,
from the peripheral nerve tissue and the suprarenal gland of the cow of
the age in the mad cow disease for the dying infection 94 months on
March 9 this year.
Japan is obligating the removal of the Specified Risk Material, or SRM
such as the head, the spinal cord, the vertebral columns, and the small
intestines that accumulate the anomalous prion protein easily as a BSE
(bovine spongiform encephaalopathy) measures.
Because the mad cow disease etiologic agent was detected from a tissue
different from the Specified Risk Material, or SRM, the review of the
Specified Risk Material, or SRM might be urged on the Japanese Government.
International Symposium of PRION DISEASES for food and drug safety
national institute of animal health(only in Japanese)
The statement of the Ministry of Health, Labour and Welfare
(only in Japanese)
Yomiuri on line (only in Japanese)
Asahi on line(only in Japanese)
Mainichi on line(only in Japanese)

Working Group Report on
the Assessment of the Geographical BSE-Risk (GBR) of


Annex to the EFSA Scientific Report (2004) 2, 1-15 on the Assessment of the
Geographical BSE Risk of Canada
- 11 -


- 2 -
2.1 Import of cattle from BSE-Risk2 countries
An overview of the data on live cattle imports is presented in table 1 and is based on
data as provided in the country dossier (CD) and corresponding data on relevant exports
as available from BSE risk countries that exported to Canada. Only data from risk
periods are indicated, i.e. those periods when exports from a BSE risk country already
represented an external challenge, according to the SSC opinion on the GBR (SSC July
2000 and updated January 2002).
• According to the CD, 231 cattle were imported from UK during the years 1980 to
1990 and no cattle imports from UK were recorded after 1990.
• According to Eurostat, altogether 198 cattle have been imported from the UK during
the years 1980 to 1990, Additionally 500 were recorded in 1993; this import is
1 For the purpose of the GBR assessment the abbreviation “MBM” refers to rendering products, in particular
the commodities Meat and Bone Meal as such; Meat Meal; Bone Meal; and Greaves. With regard to imports
it refers to the customs code 230110 “flours, meals and pellets, made from meat or offal, not fit for human
2 BSE-Risk countries are all countries already assessed as GBR III or IV or with at least one confirmed
Annex to the EFSA Scientific Report (2004) 2, 1-14 on the Assessment of the
Geographical BSE Risk of Canada
- 3 -
mentioned in Eurostat and the updated UK export statistic as male calves, but not
mentioned in the original UK export statistics. According to the CD, detailed
investigations were carried out and it is very unlikely that the 500 calves have been
imported. Therefore, they were not taken into account.
• According to the CD, in 1990 all cattle imported from UK and Ireland since 1982
were placed in a monitoring program.
• Following the occurrence of the BSE index case in 1993 (imported from UK in 1987
at the age of 6 months), an attempt was made to trace all other cattle imported from
UK between 1982 and 1990.
• Of the 231 cattle imported from the UK between 1980 and 1990, 108 animals had
been slaughtered and 9 had died. From the remaining, 37 were exported, 76 were
sent to incineration and one was buried; these were not entering the rendering system
and therefore not taken into account.
• According to the CD, 16 cattle were imported from Ireland (according to Eurostat
20), of which 9 were slaughtered, 3 died. The remaining 4 were incinerated and did
therefore not enter the rendering system. According to the CD, the 6 animals which
were imported in 1990 according to Eurostat, were never imported.
• Moreover 22 cattle have been imported from Japan (through USA), of which 4 were
exported (excluded from the table) and 14 were destroyed and therefore not entering
the rendering system, 4 were slaughtered.
• Of 28 imported bovines from Denmark, 1 was destroyed and 1 was exported. Of the
19 buffalos imported in 2000, 1 was incinerated and the others were ordered to be
• Additionally in total 264 cattle according to the CD (276 according to other sources)
were imported from Austria, France, Germany, Hungary, Italy, The Netherlands and
• The numbers imported according to the CD and Eurostat are very similar. Some
discrepancies in the year of import can be explained by an extended quarantine;
therefore it is likely that imports according to Eurostat in 1980 and imports
according to the CD in 1981 are referring to the same animals.
• Additionally, between 16.000 and 340.000 bovines have annually been imported
from US, almost all are steers and heifers. In total, between 1981 and 2003,
according to the CD more than 2.3 million, according to other sources 1.5 million
cattle have been imported.
• According to the CD, feeder/slaughter cattle represent typically more than 90% of
the imported cattle from the USA; therefore, only 10% of the imported cattle have
been taken into account.


Annex to the EFSA Scientific Report (2004) 2, 1-15 on the Assessment of the
Geographical BSE Risk of Canada
- 5 -
2.2 Import of MBM or MBM-containing feedstuffs from BSE-Risk
An overview of the data on MBM imports is presented in table 2 and is based on data
provided in the country dossier (CD) and corresponding data on relevant exports as
available from BSE risk countries that exported to Canada. Only data from risk periods
are indicated, i.e. those periods when exports from a BSE risk country already
represented an external challenge, according to the SSC opinion on the GBR (SSC, July
2000 and updated January 2002).
According to the CD, no imports of MBM took place from UK since 1978 (initially
because of FMD regulations).
• According to Eurostat data, Canada imported 149 tons MBM from the UK in the
period of 1993 to 2001. According to up-dated MBM statistics from UK (August
2001) no mammalian MBM was exported to Canada from 1993 – 1996. As it was
illegal to export mammalian meat meal, bone meal and MBM from UK since
27/03/1996, exports indicated after that date should only have included nonmammalian
MBM. Therefore, these imports were not taken into account.
• According to the CD, imports of MBM have taken place from Denmark, Germany,
France, Japan and US.
• According to Eurostat Canada imported MBM from Denmark, Belgium, France and
• According to the CD further investigations concluded that all imported MBM from
Denmark consisted of pork and poultry origin and was directly imported for
aquaculture, the imported MBM from France was feather meal, the imported MBM
from Germany was poultry meal for aquaculture and the imported MBM from
Belgium was haemoglobin; therefore these imports were not taken into account.
• The main imports of MBM were of US origin, according to the CD around 250.000
tons, according to other sources around 310.000 tons between 1988 and 2003.


Annex to the EFSA Scientific Report (2004) 2, 1-15 on the Assessment of the
Geographical BSE Risk of Canada
- 7 -
2.3 Overall assessment of the external challenge
The level of the external challenge that has to be met by the BSE/cattle system is
estimated according to the guidance given by the SSC in its final opinion on the GBR of
July 2000 (as updated in January 2002).
Live cattle imports:
In total the country imported according to the CD more than 2.3 million, according to
other data 1.5 million live cattle from BSE risk countries, of which 231 (CD)
respectively 698 (other sources) came from the UK. The numbers shown in table 1 are
the raw import figures and are not reflecting the adjusted imports for the assessment of
the external challenge. Broken down to 5 year periods the resulting external challenge is
as given in table 3. This assessment takes into account the different aspects discussed
above that allow to assume that certain imported cattle did not enter the domestic
BSE/cattle system, i.e. were not rendered into feed. In the case of Canada, the 500 cattle
imported from UK according to Eurostat were not taken into account and it is assumed
that all incinerated, buried, exported animals and the animals still alive did not enter the
rendering system and were therefore excluded from the external challenge.
MBM imports:
In total the country imported according to the CD around 300.000 tons, according to
other sources nearly 360.000 tons of MBM from BSE risk countries, of which 149 tons
came from the UK. The majority consisted of MBM imported from the US. The
numbers shown in table 2 are the raw import figures and are not reflecting the adjusted
imports for the assessment of the external challenge. Broken down to 5 year periods the
resulting external challenge is as given in table 3. This assessment takes into account
the different aspects discussed above that allow to assume that certain imported MBM
did not enter the domestic BSE/cattle system or did not represent an external challenge
for other reasons. As it was illegal to export mammalian meat meal, bone meal and
MBM from UK since 27/03/1996, exports indicated after that date should only have
included non-mammalian MBM. In the case of Canada all imported MBM from UK,
Germany, Belgium, Denmark and France was not taken into account.
On the basis of the available information, the overall assessment of the external
challenge is as given in table 3 below.
Annex to the EFSA Scientific Report (2004) 2, 1-15 on the Assessment of the
Geographical BSE Risk of Canada
- 8 -
External Challenge experienced by CANADA
External challenge Reason for this external challenge
Period Overall Level Cattle
1980 to 1990 Low Low Negligible
1991 to 1995 High Moderate High
1996 to 2000 Extremely
high High Extremely
2001 to 2003 Very high High Very high
Table 3: External challenge resulting from live cattle and/or MBM imports from the UK and other BSE risk
countries. The challenge level is determined according to the SSC-opinion on the GBR of July 2000 (as
updated in January 2002).
3.1 Overall appreciation of the ability to avoid recycling of BSE
infectivity, should it enter processing
The annual Canadian production of MBM is approximately 575,000 tons of which
approx. 40,000 tons are exported each year, mainly to USA.
Use of MBM in cattle feed
• Before the feed ban, dairy cattle received supplementary feed containing MBM
during their productive life (maximum 200-400 g MBM per day). Beef cattle in the
western part of the country do not usually receive complementary feed. Beef cattle
in the eastern part receive normally no supplement protein but the calves could have
access to creep feeds containing MBM, after weaning the ratios may have contained
supplemental protein containing MBM (100-400 g per day).
• According to the CD, MBM is mainly fed to pigs and poultry and included in pet
• According to the CD, only a proportion of dairy cattle may have received MBM.
Feed bans
• Before 1997, there was no legal restriction to include MBM into cattle feed.
• An MBM-ban was introduced in August 1997; it is forbidden since to feed
mammalian MBM to ruminants except if of pure porcine, equine and non
mammalian origin, i.e. in practice a ruminant-to-ruminant ban (RMBM-ban).
Annex to the EFSA Scientific Report (2004) 2, 1-15 on the Assessment of the
Geographical BSE Risk of Canada
- 9 -
Potential for cross-contamination and measures taken against
• Cross-contamination in the about 600 feed mills is assumed to be possible as long as
cattle and pig feed is produced in the same production lines, and premises.
• Cross-contamination during transport is possible, particularly if the same trucks are
used for transporting ruminant MBM (RMBM) and non-ruminant MBM (porcine or
poultry MBM which still might be included into cattle feed) or for transporting
pig/poultry feed and cattle feed.
• On-farm cross-contamination is regarded to be possible.
• Cross-contamination of cattle feed with RMBM can not be excluded. Hence, as
reasonable worst case scenario, it has to be assumed that cattle, in particular dairy
cattle, can still be exposed to RMBM and hence to BSE-infectivity, should it enter
the feed chain.
Control of Feed bans and cross-contamination
• With the introduction of the RMBM ban (1997) the feed mills (approximately 600)
were checked for compliance with the ban, including good manufacturing practices
(GMP) and record keeping, i.e. the separation in production of MBM containing
ruminant material (RMBM) from non-ruminant MBM.
• The feed mills had previously – since 1983 – been regularly checked in relation to
production of medicated feed.
• No examinations are performed to assess cross-contamination with RMBM of the
protein (e.g. non ruminant MBM) that enters cattle feed. Differentiation would
anyway be difficult.
Raw material used for rendering
• Ruminant material is rendered together with material from other species, but
according to the CD only in the production of MBM prohibited for use in ruminant
• Slaughter by-products, including specified risk material (SRM) and fallen stock are
• The country expert estimated that 20% of the rendering plants, processing 20% of
the total amount of raw material, are connected to slaughterhouses. Their raw
material is more than 98 % animal waste from these slaughterhouses while less than
2 % is fallen stock. No estimation was given for the remaining 80% of the rendering
• There are 32 rendering plants of which 3 are processing blood exclusively.
Rendering processes
• The rendering systems (parameters) were specified for 6 plants producing mixed
MBM, none of these fulfilled the 133/20/3 standard. Of these, 5 have dedicated
facilities to produce products for use in ruminant feed and products not permitted for
use in ruminant feed.
• The remaining plants process porcine or poultry material exclusively.
SRM and fallen stock
• There is an SRM ban for human food in place since 2003.
Annex to the EFSA Scientific Report (2004) 2, 1-15 on the Assessment of the
Geographical BSE Risk of Canada
• However, SRM are rendered together with other slaughter waste and fallen stock.
However, according to the CD, MBM with SRM is not permitted to be fed to
Conclusion on the ability to avoid recycling
• Between 1980 and 1997 the Canadian system would not have been able to avoid
recycling of the BSE-agent to any measurable extent. If the BSE-agent was
introduced into the feed chain, it could have reached cattle.
• Since 1997 this ability gradually improved with the introduction of the ruminant
MBM ban and its implementation.
• Since cross-contamination cannot be excluded, and as SRM is still rendered by
processes unable to significantly reduce BSE-infectivity, the system is still unable to
avoid recycling of BSE-infectivity already present in the system or incoming.
BSE surveillance
laboratory tests).
i.e. formalin fixation.


In 1990, when BSE was made notifiable, this awareness was extended to
suspicions of BSE.
" Since 1993 the number of brains examined per year did exceed the number
recommended by OIE (300 - 336 for countries with a cattle population over 24
months of age of 5.0 to 7.0 Million)


in all years, except in 1995 (table 4).
year 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003
samples 225 645 426 269 454 759 940 895 1´020 1´581 3´377 3´361
Table 4: Number of bovine brains annually examined for CNS diseases,
including BSE.
" According to the CD approx. 98% of the examined cattle were older than
24 months
and approx. 90% exhibited neurological symptoms. Although the identification
system of Canada does not document the birth date or age of the animals,
to the CD, examination of the dentition is used to ascertain the
maturity of the
" The list of neurological differential diagnoses for the 754 brains
examined in 1997
included encephalitis (70 cases), encephalomalacia (19), hemophilus (7),
hemorrhage (2), listeriosis (38), meningoencephalitis (36), rabies (22),
tumors (2),
other conditions (135) and no significant findings (423).
" Compensation is paid for suspect BSE cases as well as for animals
ordered to be
destroyed (90-95% of market value with a maximum of 2,500 Can$ per cow).
" Diagnostic criteria developed in the United Kingdom are followed at ADRI,
Nepean. According to the very detailed protocol for the collection,
fixation and
submission of Bovine Spongiform Encephalopathy (BSE) specimens at abattoirs
under inspection by the Canadian Food Inspection Agency, the specimen
shall be
shipped to National Center for Foreign Animal Disease, Winnipeg, Manitoba.
" In 2003, around 3000 animals from risk populations have been tested.
" According to the CD, it is aimed to test a minimum of 8000 risk
animals (animals
with clinical signs consistent with BSE, downer cows, animals died on
farm animals
diseased or euthanized because of serious illness) in 2004 and then
continue to
progressively increase the level of testing to 30,000.
" In May 2003, Canada reported its first case of domestic BSE. A second
case was
detected in the US on 23 December 2003 and traced back to Canadian
origin. Both
were born before the feed ban and originated from Western Canada.
3.3 Overall assessment of the stability
For the overall assessment of the stability, the impact of the three
main stability factors
(i.e. feeding, rendering and SRM-removal) and of the additional
stability factor,
surveillance, has to be estimated. Again, the guidance provided by the
SSC in its
opinion on the GBR of July 2000 (as updated January 2002) is applied.
Until 1997, it was legally possible to feed ruminant MBM to cattle and a
certain fraction of
cattle feed (for calves and dairy cattle) is assumed to have contained
MBM. Therefore
feeding was Not OK. In August 1997 a ruminant MBM ban was introduced
but feeding
of non-ruminant MBM to cattle remained legal as well as feeding of
ruminant MBM to
non-ruminant animals. This makes control of the feed ban very difficult
because laboratory
differentiation between ruminant and non ruminant MBM is difficult if
not impossible.
Annex to the EFSA Scientific Report (2004) 2, 1-15 on the Assessment of the
Geographical BSE Risk of Canada
- 12 -
Due to the highly specialised production system in Canada, various
mammalian MBM
streams can be separated. Such a feed ban would therefore be assessed as
OK", for all regions where this highly specialised system exists.
However, several areas
in Canada do have mixed farming and mixed feed mills, and in such
regions, an RMBM
ban would not suffice. Additionally, official controls for cattle feeds
to control for the
compliance with the ban were not started until the end of 2003. Thus,
for the whole
country, the assessment of the feeding after 1997 remains "Not OK".
The rendering industry is operating with processes that are not known to
reduce infectivity.
It is therefore concluded that the rendering was and is Not OK.
SRM and fallen stock were and are rendered for feed. Therefore
SRM-removal is assessed
as Not OK
BSE surveillance
Before 1989, the ability of the system to identify (and eliminate)
BSE-cases was limited.
Since 1990 this ability is improved, thanks to a specific (passive) BSE
Today the surveillance should be able to detect clinical BSE-cases
within the limits set
by an essentially passive surveillance system.
" Passive surveillance has been carried out since 1990. In 1993
surveillance was
intensified and has considerably improved with mandatory reporting and basic
compensation ensured, awareness raising measures and education of
veterinarians, and
a specific BSE-surveillance programme targeting cattle showing clinical
signs that
could be compatible with BSE.
" The initiated introduction of active surveillance should improve the
Stability of the BSE/cattle system in CANADA over time
Stability Reasons
Period Level Feeding Rendering SRM
1980 to 2000 Mainly
2001 to 2003
unstable Not OK Not OK Not OK
with some
testing of
risk groups
Table 5: Stability resulting from the interaction of the three main
stability factors and the BSE
surveillance. The stability level is determined according to the
SSC-opinion on the GBR of July 2000 (as
updated in 2002).
Annex to the EFSA Scientific Report (2004) 2, 1-15 on the Assessment of the
Geographical BSE Risk of Canada
- 13 -
On the basis of the available information, it has to be concluded that
the country's
BSE/cattle system was extremely unstable until today, i.e., it would
have recycled and
amplified BSE-infectivity very fast, should it have entered the system.
The stability of the
BSE/cattle system in Canada overtime is as given in table 5 above.
4.1 Interaction of stability and challenges
In conclusion, the stability of the Canada BSE/cattle system in the past
and the external
challenges the system has coped with are summarised in the table 6.
Period Stability External Challenge Internal challenge
1980 to 1990 Low Unlikely but not excluded
1991 to 1995 High
1996 to 2000 Extremely high
Likely and rapidly growing
2001 to 2003
Very high Confirmed at a lower level
Table 6: Internal challenge resulting from the interaction of the
external challenge and stability. The
internal challenge level is determined according to guidance given in
the SSC-opinion on the GBR of
July 2000 (as updated in 2002).

From the interaction of the two parameters stability and external
challenge a

conclusion is drawn on the level of internal challenge that emerged
and had to be met
by the system, in addition to external challenges that occurred.
An external challenge resulting from cattle import could only lead to an
challenge once imported infected cattle were rendered for feed and this
feed reached domestic cattle. Cattle imported for slaughter would
normally be
slaughtered at an age too young to harbour plenty of BSE infectivity or
to show signs,
even if infected prior to import. Breeding cattle, however, would
normally live much
longer and only animals having problems would be slaughtered younger. If
being 4-6
years old when slaughtered, they could suffer from early signs of BSE, being
approaching the end of the BSE-incubation period. In that case, they
would harbour,
while being pre-clinical, as much infectivity as a clinical BSE case.
Hence cattle imports
could have led to an internal challenge about 3 years after the import
of breeding cattle
(that are normally imported at 20-24 months of age) that could have been
infected prior
to import. In case of Canada this implies that cattle imported in the
mid eighties could
have been rendered in the late eighties and therefore led to an internal
challenge in the
early 90s.
On the other hand imports of contaminated MBM would lead to an internal
challenge in
the year of import, if fed to cattle. The feeding system is of utmost
importance in this
context. If it could be excluded that imported, potentially contaminated
feed stuffs
reached cattle, such imports might not lead to an internal challenge at
all. In case of
Annex to the EFSA Scientific Report (2004) 2, 1-15 on the Assessment of the
Geographical BSE Risk of Canada
- 14 -
Canada this implies that it was possible that imported MBM reached
domestic cattle and
lead to an internal challenge in the early 90s.
4.2 Risk that BSE infectivity entered processing
A certain risk that BSE-infected cattle entered processing in Canada,
and were at least
partly rendered for feed, occurred in the early 1990s when cattle
imported from UK in
the mid 80s could have been slaughtered. This risk continued to exist,
and grew
significantly in the mid 90s 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.
4.3 Risk that BSE infectivity was recycled and propagated
A risk that BSE-infectivity was recycled and propagated exists since a
processing risk
first appeared; i.e. in the early 90s. Until today this risk persists
and increases fast
because of the extremely unstable BSE/cattle system in Canada.
5.1 The current GBR as function of the past stability and challenge
The current geographical BSE-risk (GBR) level is III, i.e. it is
confirmed at a lower level
that domestic cattle are (clinically or pre-clinically) infected with
the BSE-agent.
This assessment deviates from the previous assessment (SSC opinion,
2000) because at
that time several exporting countries were not considered a potential risk.
5.2 The expected development of the GBR as a function of the past and
present stability and challenge
" As long as the system remains unstable, it is expected that the GBR
continues to
grow, even if no additional external challenges occur.
" Since recent improvements in the safety of MBM production in many
countries or
significant recent reductions in the incidence of BSE are not taken into
account for
the assessment of the external challenge, the external challenge
assessed after 2001
could be overestimated and is the worst case assumption. However all
current GBR
conclusions are not dependent on these assumptions in any of the
countries assessed.
For future assessments and when the impact of the production,
surveillance and true
incidence changes has been fully quantified, these developments should
be taken
into account.
5.3 Recommendations for influencing the future GBR
" Enhancing the stability of the system, in particular by ensuring that
cattle have no
access to mammalian MBM in combination with appropriate rendering and
exclusion of
SRM and fallen stock from any feed chain could lead, over time, to a
reduction of the
" Improved passive and active surveillance, i.e. sampling of animals not
signs compatible with BSE from at-risk cattle populations, such as
adult cattle in
Annex to the EFSA Scientific Report (2004) 2, 1-15 on the Assessment of the
Geographical BSE Risk of Canada
- 15 -
fallen stock and emergency slaughter, by means of rapid screening, would
monitoring the efficiency of stability enhancing measures.
Documentation provided to EFSA
" Letter with the ref D(2003)KVD/ip/420722 from the European Commission
requesting a geographical risk assessment for the appearance of BSE in a
" Country Dossier as prepared by the country in response to the EC and EFSA
data collection request.
" Other sources of data information i.e. exports from third countries and
Eurostat data.
" SSC, July 2000. Final opinion on the Geographical Risk of Bovine
Spongiform Encephalopathy (GBR).
" SSC, January 2002. Updated opinion on the Geographical Risk of Bovine
Spongiform Encephalopathy (GBR).
Members of the EFSA Scientific Expert Working Group on GBR are
acknowledged for
their valuable contribution to this mandate. The members are: Didier
Calavas, Aline De
Koeijer, Michael Gravenor, John Griffin, Dagmar Heim, Matthias Kramer,
Maijala, Mo Salman, Vittorio Silano, Emmanuel Vanopdenbosch, and Stig


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;

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

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

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

Follow Ups:

Post a Followup

E-mail: (optional)


Optional Link URL:
Link Title:
Optional Image URL: