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From: TSS (216-119-144-28.ipset24.wt.net)
Subject: TSE (England) (Amendment) Regulations 2004
Date: September 9, 2004 at 7:32 am PST

-------- Original Message --------
Subject: TSE (England) (Amendment) Regulations 2004
Date: Thu, 09 Sep 2004 09:40:37 -0500
From: "Terry S. Singeltary Sr."
To: Bovine Spongiform Encephalopathy


TSE (England) (Amendment) Regulations 2004


9.17 p.m.

Baroness Byford rose to move, That an humble Address be presented to Her
Majesty praying that the regulations, laid before the House on 18 June,
be annulled (S.I. 2004/1518) [15th Report from the Merits Committee].

The noble Baroness said: My Lords, the regulations will have a great
impact on farmers who breed sheep or keep goats. Concerns have been
expressed to me over the scientific certainty, or lack of it, on which
the regulations are based.

In its response to Defra's consultation on the implementation of the new
EU-wide controls on scrapie- affected farms, the National Sheep
Association has stated:


"The National Sheep Association welcomes the opportunity to
respond to the [Defra] consultation on the implementation of
EU-wide controls on scrapie-affected farms".

The NSA is the leading specialist representative organisation for the
United Kingdom sheep sector. Its members come from those involved with
every pedigree sheep bred in this country; indeed, from all the sheep
breed societies. As the pedigree sector has been the one most affected
by the various regulations concerned with the eradication of scrapie so
far, we feel that this makes the NSA response particularly significant
in the development of the resulting policy. This is in addition to a
majority of members of the commercial sheep sector. We can therefore say
with

8 Sept 2004 : Column 669

confidence that our views are truly representative of the entire UK
sheep sector. I am sure that the Minister will wish to congratulate the
NSA on the work it has done to date in the eradication of scrapie.

The NSA appreciates the fact that Defra is consulting on how best to
implement the new EU regulation and not the regulation itself. However,
it feels that it is important to reiterate that it has grave concerns
over the EU regulation that Defra is being obliged to implement. The NSA
feels that it is an ill-conceived regulation, in general, with measures
and provisions that are draconian in nature and totally disproportionate
to the scale of the issue to be dealt with.

Indeed, the regulation is best described by the commonly used phrase
"bad law" and, as such, it runs the risk of being regarded as a step too
far by the many sectors of the sheep industry that may be affected by
its introduction, as outlined in the consultation. The National Sheep
Association feels particularly strongly about the measures proposed
under the restrictions section. The proposals as outlined are very
severe and, for some businesses which are reliant on the sale of store
and breeding sheep, it may be that their imposition will result in the
collapse of such businesses. In addition, the proposals could seriously
affect the asset value of a farm should the farmer wish to sell his
property while under restriction.

However, as previously stated, the National Sheep Association
appreciates that Defra is obliged to implement the regulation given that
it has been agreed within the EU and, as such, cannot legally be
ignored. On this point, the NSA will be constructive in its response, as
I am sure the Minister will acknowledge that it has been in the past.
But this is set against the backdrop of its concerns over the regulation
itself.

With this in mind, it is worth mentioning that it is not strictly
correct for Defra to sayas it does in the letter sent out with the
consultation documentthat key industry representatives are content with
the proposals. As the NSA, for one, most definitely is not, I ask the
Minister to comment on who Defra is referring to as "key industry
representatives" if they do not include the National Sheep Association.

When the Merits of Statutory Instruments Committee looked at the
regulations it raised two specific points, on which it wrote to Margaret
Beckett, the Secretary of State for the Environment, Food and Rural
Affairs. Under the heading "Adequacy of measures proposed", paragraph 2
of the letter states:


"While the committee accepted the need to ensure implementation of
European requirements, we were concerned to establish whether the
Regulations and the controls introduced by them are an adequate
response to the scale of the problem, given that scrapie has
occurred for so many years and so widely in sheep in this country.
We would be grateful if you could comment on your assessment of
the scale of the occurrence of scrapie in sheep in this country,
and on how quickly and effectively the approach being followed in
the Regulations will succeed in eliminating the risks posed by
scrapie".

8 Sept 2004 : Column 670

The letter then goes on to refer to one or two other matters that I
should like to highlight. It pointed out that the RIA of 17 June stated
that 20 responses were received to Defra's consultation on the partial
RIA and that,


"most thought that Option 2 as set out in the RIA was the best
approach; and that a number of concerns were raised about the EU
regulation which DEFRA continued to pursue with the European
Commission".

The letter continues:


"In addition, your Department provided us with a copy of DEFRA's
summary of responses to the consultation exercise. We were struck
by the difference in tone, if not the substance, of that summary
from the statement in the RIA".

The summary referred to a universal feeling that the regulation is
draconian and disproportionate in its effect compared with the risk with
which it deals. It acknowledges that the science on which it is based is
questioned. It states that,


"the introduction of the compulsory [scheme] is resented".

The chairman of the committee said that it felt that the RIA statement
on the consultation responses appeared to understate significantly the
strength of concern among respondents.

I shall not read the rest of the letter, but it is important that your
Lordships are aware of the serious concerns felt in the farming community.

In her response, Margaret Beckett stated that they realised that most
scrapie cases are not reported so that the scale of the problem is
significantly larger than the 230 cases confirmed in sheep in England in
2003. If this is the case, how many flocks does the department estimate
to be infected, or is this still a totally unknown number?

In 1999, SEAC recommended the introduction of a breeding programme to
eradicate scrapie from the sheep population in view of the theoretical
possibility that sheep could have contracted BSE which was being masked
by scrapie. How many projects have begun since 1999 to prove this
theoretical possibility, how much has that cost to date and, more
importantly, has any evidence been established to confirm this possible
theory?

Will the Minister accept that the longer this theoretical possibility
continues to be unproven, it is reasonable to conclude that a link is
not established. In that case, should we really be approving a
regulation which has measures and provisions that are draconian in
nature and disproportionate?

This morning the Prime Minister again reinforced his Government's
commitment to legislating on the basis of scientific proof. But these
regulations are based on scientific uncertainty. Can the Minister
explain the discrepancy? Furthermore, when the regulations were
considered at drafting stage, were these issues raised, and did Her
Majesty's Government agree with the drafting?

I understand that over the past few years, Defra has carried out a large
survey of sheep testing. This year, for the first time, the number of
animals tested has decreased from 60,000 a year to 10,000. Can the
Minister tell me why?

8 Sept 2004 : Column 671

Does the Minister recognise the success of the voluntary scheme
established under the voluntary scrapie flocks scheme, which has more
than 100 applications already? Does she care that these restrictive
regulations may well result in a fall in the number of scrapie cases
reported? I understand that the Government will monitor the situation
closely and if they find that reporting decreases significantly, they
will take it up with the European Commission. Over what time period will
that monitoring take place? What response do the Government expect from
the commission?

I raise these concerns because this will be very costly for the
Government. I noticed that in yesterday's Hansard, a Written Answer in
response to my noble friend Lord Marlesford showed that bovine TB has
cost the taxpayer some £38 million this year alone. These are huge
figures and we need to be very sure of our ground when we are committing
ourselves to additional costs. At the moment, we have no idea what the
costs might be. What is the estimated cost of slaughter and how much
will the implementation, controls and monitoring cost? Is the true cost
proportional to the risk when one considers that the Government refuse
to ban smoking? That is not a light, throwaway question. It puts into
context the whole question of this regulation. I beg to move.

Moved, That an humble Address be presented to Her Majesty praying that
the regulations, laid before the House on 18 June, be annulled (S.I.
2004/1518) [15th Report from the Merits Committee].(Baroness Byford.)

The Countess of Mar: My Lords, I am very sorry that the noble Lord, Lord
Whitty, is not able to be with us tonight and I hope that we shall soon
hear him in good voice before the House. I am sure that I carry the
wishes of other noble Lords when I say that.

I am sure that the Minister and other noble Lords taking part in this
debate will recall my objections to the TSE (England) Regulations 2002,
which we debated in May 2002. They will not be surprised that I am very
grateful to the noble Baroness, Lady Byford, for allowing me to return
to the fray this evening. I declare my interest as the wife of a sheep
and goat farmer. As well as being a goat cheese-maker, I take an active
part in our livestock management.

I was astonished to read in the regulations that goats are to be dealt
with far more harshly than sheep. In the Explanatory Memorandum is the
statement:


"Goats are also susceptible to scrapie and scientific advice is
that goat herds would also pose a public health risk if BSE were
to be found in sheep".

May I ask the Minister exactly what is the scientific evidence upon
which this scientific advice is based? Who is providing the scientific
advice upon which these statements are made? Poor old goatswith no
specific genotypes and little information available, it seems easier to
kill them all. What an odd approach. Was it dreamt up because scrapie is
so much rarer in goats than sheep, so slaughter should eliminate the

8 Sept 2004 : Column 672

disease, or was it that, because it is so rare, the possibility that a
whole herd would have to be despatched is remote? I would hope that it
is the latter.

It has already been noted that the incidence of scrapie in the sheep
flock is probably under-reported. I suspect that this is simply because
it is accepted that sheep out on the moors and hills die from a number
of causes. Their carcasses are often eaten by foxes and other scavengers
before the shepherd finds them. It is the nature of modern shepherding
that the flocks are not constantly watched over. Such is the
relationship of most of the goat keepers that I know with their animals
that a vet would be called to attend any really sick animal. Three
reported cases in eight years is likely to be an accurate reflection of
the incidence in the national goat herd. It would also appear to
indicate that the goat to goat transmission rate is low. If this is the
case, what on earth would be achieved by slaughtering the whole herd?

Although I recognise that the Minister will say that this amendment to
the 2002 regulations is to provide,


"powers for the enforcement of EU legislation introducing controls
on farms that have had a confirmed case of scrapie",

it still seems extraordinary that all the plans and regulations relating
to TSEs and particularly to scrapie are based upon an hypothesis that
has become an assumption that it is a rogue isoform of PrP that causes
TSEs.

Humans have lived with scrapie for at least 250 years and there has been
no evidence that scrapie has ever been transmitted to humans, although I
accept that absence of evidence is not evidence of absence. The
conclusion that a single strain of the agent which causes BSE is more
easily transmissible between species than previously known strains and
may therefore be present in sheep still remains unsupported by critical
scientific evidence.

I will repeat my concerns that, by requiring the slaughter of sheep of
particular genotypes we will be depleting the gene pool in our sheep
flock. We may well be laying the animals open to epidemics of TSEs other
than those of which we have current knowledge, or, worse still, to some
other highly infectious diseases to which the national flock is at
present resistant. Why are not the sensible measures already in force
sufficient? We are already required to minimise the routine spread of
these not particularly infectious agents.

I note that the Explanatory Memorandum to this regulation frequently
uses the terms "TSE resistant" and "resistant genotypes". Unless and
until there is an adequately comprehensive list of the TSE strains
tested and it can be shown that they can survive passage between
animals, these terms are not appropriate. When the real molecular nature
of the agent that causes TSEs is known, it may be possible to employ
these terms.

Should we not be dealing with facts rather than the assumptions? I may
be criticised for suggesting that we fly in the face of the
precautionary principle in this instance, when I have fought long and
hard for it to be exercised in the case of exposure of humans to toxic

8 Sept 2004 : Column 673

chemicals. I would respond by saying that there is a clear correlation
between, for example, organophosphate exposure and ill health, while
there is not one between naturally occurring BSE in sheep and goats and
variant CJD in humans. I would argue that there is a place for
proportionality.

The statement that TSEs are caused by a so-called "rogue protein"a
misshapen version of a host protein known as PrPhas never been proven.
The infective PrP has never been synthesised de novo. There is, I
understand, substantial evidence to show that this protein becomes
misshapen by infection, rather than that it is the cause of the
infection, with the causal molecule not proven. That means that the
majority of the current laboratory work is uncertain in its direct
relevance to the infective nature of TSEs.

There has been very little research to establish the reason why BSE was
able to infect some humans across the species barrier from cattle. We
need to know whether the pressure was exerted by the massive scale of
the human exposure to infected beef products during the BSE epidemic or
whether the cattle strain of BSE, dose for dose, is more easily
transmitted to humans than current strains of scrapie or other TSEs. If
the former is the case, the risk is well controlled. It is only if the
latter is correct that the rigorous precautionary principles now being
implemented are needed, as there is a much more serious risk when there
is no species barrier, such as in human blood transfusion. May I ask the
Minister whether it is the Government's intention that such research
should be conducted? If she says no, then I would suggest that we should
leave our sheep and goats to get on with their lives and save the
taxpayer a small fortune.

I note that the Commission Regulation (EC) No. 1915/2003 requires
thorough cleaning and disinfection of all animal housing on the premises
following destocking and before restocking. In view of the fact that we
know that total destruction of the infected material in a hospital
setting is almost impossible, will the Minister please tell the House
how effective disinfection of rambling farm buildings will be achieved?
What about grazing land upon which infected animals may have defecated
or left foetal remains? What advice has she received on these matters?

It really is time that we seriously questioned the quality of scientific
advice that Ministers are receiving. I have said this from time to time
over a great many years. We are repeatedly told that the scientific
committees are independent. Experience has taught me that it might be
true that they are independent of government but, I would suggest, they
are not independent of Whitehall or some of the other organisations with
whom the mandarins have close association. Too often, busy experts from
fields unconnected with the subject under consideration have been
expected to make recommendations which are outside their competence. It
is not their fault; they do their best within their limitations.
Unfortunately, I believe that this system has led to an intellectual
corruption of science.

8 Sept 2004 : Column 674

TSE, and particularly BSE, research has been corrupted by the publicity
that has been generated, first by those dreadful pictures of Daisy the
cow, and then the discovery that humans could suffer from a similar
awful disease. Instead of going to the centres of existing knowledgeto
the people who had some understanding of the subject after working for
years on scrapiethe MAFF and Defra career civil servants, through the
funding agencies and the advisory committees, diverted research funding
to organisations that subsequently proved their incompetence very publicly.

Progress has consistently been hindered by what I can only describe as
wild goose chases. Government departments should not control basic
scientific research, especially when it is in the vanguard of scientific
progress. Perhaps it would be better if they were to admit that they did
not know the answers and make a point of finding a scientist who does,
or at least has the expertise to do so.

Will the Minister kindly tell the House what means are used to test the
competence of the expert advice that Ministers receive? May I suggest,
as it seems unlikely that anyone in government will take an executive
decision to review the quality of scientific advice offered to all
Ministers, that there should be a committee set up to discuss the
matter? Defra is very good at setting up committees and discussing
matters. It does not get anywhere but it does discuss them.

At the risk of being boringly repetitive the facts are that there is no
historical evidence of BSE in sheep. There is no current evidence of BSE
in sheep. If sheep ever were infected from eating feed contaminated by
BSE-infected meat and bone meal taken out of the food chain before any
current commercial sheep were born, the possibility of ever finding BSE
in sheep must surely be diminishing rather than increasing. What are we
doing implementing this regulation?

http://www.publications.parliament.uk/pa/ld199697/ldhansrd/pdvn/lds04/text/40908-30.htm#40908-30_dl0

> Humans have lived with scrapie for at least 250 years and there has
> been no evidence that scrapie has ever been transmitted to humans,
> although I accept that absence of evidence is not evidence of absence.
> The conclusion that a single strain of the agent which causes BSE is
> more easily transmissible between species than previously known
> strains and may therefore be present in sheep still remains
> unsupported by critical scientific evidence.


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_;

http://www.pnas.org/cgi/content/full/041490898v1

SUSPECT SYMPTOMS

http://www.organicconsumers.org/meat/scrapiecjd.cfm

STATEMENT OF DR HELEN GRANT MD FRCP
ISSUED 13/05/1999

BSE INQUIRY

http://www.bseinquiry.gov.uk/files/ws/s410.pdf
http://www.bseinquiry.gov.uk/files/ws/s410x.pdf

http://www.bseinquiry.gov.uk/evidence/ws/ws8.htm

CWD to CJD in humans (why not?), as easy as BSE/Scrapie;

The EMBO Journal, Vol. 19, No. 17 pp. 4425-4430, 2000
© European Molecular Biology Organization

Evidence of a molecular barrier limiting
susceptibility of humans, cattle and sheep to
chronic wasting disease

G.J. Raymond1, A. Bossers2, L.D. Raymond1, K.I. O?Rourke3,
L.E. McHolland4, P.K. Bryant III4, M.W. Miller5, E.S. Williams6, M.
Smits2
and B. Caughey1,7

1NIAID/NIH Rocky Mountain Laboratories, Hamilton, MT 59840,
3USDA/ARS/ADRU, Pullman, WA 99164-7030, 4USDA/ARS/ABADRL,
Laramie, WY 82071, 5Colorado Division of Wildlife, Wildlife Research
Center, Fort Collins, CO 80526-2097, 6Department of Veterinary Sciences,
University of Wyoming, Laramie, WY 82070, USA and 2ID-Lelystad,
Institute for Animal Science and Health, Lelystad, The Netherlands
7Corresponding author e-mail: bcaughey@nih.gov Received June 7, 2000;
revised July 3, 2000; accepted July 5, 2000.

Abstract

Chronic wasting disease (CWD) is a transmissible
spongiform encephalopathy (TSE) of deer and elk,
and little is known about its transmissibility to other
species. An important factor controlling
interspecies TSE susceptibility is prion protein (PrP)
homology between the source and recipient
species/genotypes. Furthermore, the efficiency with which
the protease-resistant PrP (PrP-res) of one
species induces the in vitro conversion of the normal PrP
(PrP-sen) of another species to the
protease-resistant state correlates with the cross-species
transmissibility of TSE agents. Here we
show that the CWD-associated PrP-res (PrPCWD) of cervids
readily induces the conversion of recombinant cervid PrP-sen
molecules to the protease-resistant state in accordance
with the known transmissibility of CWD between cervids. In contrast,
PrPCWD-induced conversions of human and bovine PrP-sen were
much less efficient, and conversion of ovine PrP-sen was
intermediate. These results demonstrate a barrier at the
molecular level that should limit the susceptibility of these non-cervid
species to CWD.

snip...

Clearly, it is premature to draw firm conclusions about CWD
passing naturally into humans, cattle and sheep, but the present
results suggest that CWD transmissions to humans would be as
limited by PrP incompatibility as transmissions of BSE or sheep
scrapie to humans. Although there is no evidence that sheep
scrapie has affected humans, it is likely that BSE has caused variant
CJD in 74 people (definite and probable variant CJD cases to
date according to the UK CJD Surveillance Unit). Given the
presumably large number of people exposed to BSE infectivity,
the susceptibility of humans may still be very low compared with
cattle, which would be consistent with the relatively inefficient
conversion of human PrP-sen by PrPBSE. Nonetheless, since
humans have apparently been infected by BSE, it would seem prudent
to take reasonable measures to limit exposure of humans
(as well as sheep and cattle) to CWD infectivity as has been
recommended for other animal TSEs.

snip...

http://www.emboj.org/current.shtml

Scrapie to Humans USA?

http://www.ncbi.nlm.nih.gov:80/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=3915057&dopt=Abstract


Transmission of prion diseases by blood transfusion

Nora Hunter,1 James Foster,1 Angela Chong,1 Sandra McCutcheon,2 David

Parnham,1 Samantha Eaton,1 Calum MacKenzie1 and Fiona Houston2

http://www.socgenmicrobiol.org.uk/JGVDirect/18580/18580ft.pdf

Also, at paragraph 17, it is noted that BSE had transmitted to the NPU
negative line sheep (please not that as at January 1996, only one of six
challenged sheep was clinically affected after oral challenge, four
others have since died, and one remains alive. Following intracerebral
challenge, three out of six were clinically affected, two confirmed only
on pathology, while one was negative.)

4. Meeting 16, on 26/1/94 - the update on research (16/5) confirmed that
BSE had been transmitted to sheep, and that there was clinical evidence
of transmission to mice from the spleen of the affected sheep.

snip...

IN CONFIDENCE

A STUDY AIMED AT DETERMINING WHETHER OR NOT THERE HAVE BEEN SIGNIFICANT
CHANGES IN THE NEUROPATHOLOGY OF SCRAPIE IN SHEEP AND GOATS DURING THE
LAST TWO DECADES IN MATERIAL SUBMITTED TO CVL PATHOLOGY DEPARTMENT

http://www.bseinquiry.gov.uk/files/sc/seac24/tab03.pdf

EXPERIMENTAL TRANSMISSION OF BSE TO SHEEP

http://www.bseinquiry.gov.uk/files/sc/seac25/tab05.pdf

THE RISK OF TRANSMISSION OF BSE TO SHEEP VIA FEED

http://www.bseinquiry.gov.uk/files/sc/seac31/tab01.pdf

Personal $ Confidential -- Addressee only TO ALL MEMBERS OF SEAC

THE EXPERIMENTAL TRANSMISSION OF BSE TO SHEEP

http://www.bseinquiry.gov.uk/files/sc/seac33/tab02.pdf

a) Summary of transmission studies. b) Update

http://www.bseinquiry.gov.uk/files/sc/seac33/tab03.pdf

The only circumstance in which infection with the natural isolate
produces an higher incidence of disease compared to BSE, is in
intracerebrally (and possibly orally) challenged ''positive'' line
sheep. Notwithstanding the possibility of indigenous natural scrapie in
some of these sheep, there are still sufficient numbers of transmission
cases with PrP genotypes which preclude the natural disease developing
i.e. those typed as VA136/RR154/QR171.

As an extension to this study, it has been possible to recover BSE by
passage in mice from brain and spleen taken from ''negative'' line sheep
infected with BSAE by ic and oral challenge (Foster and others 1996).
The close similarity of incubation periods and pathology from the
passage of these tissues in mice to those seen in direct BSE
transmission studies from cattle to mice suggests that passaging BSE in
sheep does not alter its bilogical properties (Bruce and others 1994).
IN FACT, because it has been possible to isolate BSE infectivity from
ovine spleens, when this proved impossible from the spleens of naturally
infected BSE cows (Fraser and Foster 1993), experimentally-induced BSE
in sheep appears to behave more like the natural disease of
scrapie.Whether this putative similarity to natural scrapie extends to
the possibility of maternal transmission of experimentally-induced BSE
in sheep, has till to be elucidated...

http://www.bseinquiry.gov.uk/files/mb/m09/tab01.pdf

we have found a link between BSE and CH1641, a C-group of scrapie.
Disease susceptibility of sheep to these isolates is associated with
different PrP genotypes compared to SSBP/1 scrapie...

Transmission of BSE in sheep, goats and mice.

snip...

BSE has been transmitted in two lines of genetically selected sheep
(differeing in their susceptibilities to the SSBP/1 source of scrapie),
and to goats by intracerebral injection AND BY ORAL DOSING.

snip...

Also, intermediate passage of BSE in sheep or goats did not alter these
primary transmission properties. Hamsters were susceptible to BSE only
after intervening passage through mice...

http://www.bseinquiry.gov.uk/files/mb/m09/tab11.pdf

IN CONFIDENCE

Perceptions of unconventional slow virus in the USA

3. Prof. A Robertson gave a brief account of BSE. The US approach was to
accord it a very low profile indeed. Dr. A Thiermann showed the picture
in the ''Independent'' with cattle being incinerated and thought this
was a fantical incident to be avoided in the USA AT ALL COSTS. BSE was
not reported in the USA...........(some good data on CWD)

> avoided in the USA AT ALL COSTS

and indeed they have and it continues today...TSS

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

BSE TRANSMISSION STUDIES

http://www.bseinquiry.gov.uk/files/sc/seac18/tab02b.pdf

1: Cent Eur J Public Health 2003 Mar;11(1):19-22

Analysis of unusual accumulation of Creutzfeldt-Jakob disease cases
in Orava and Liptov regions (northern Slovak focus) 1983-2000.

Mad'ar R, Maslenova D, Ranostajova K, Straka S, Baska T.

Institute of Epidemiology, Jessenius Faculty of Medicine, Comenius
University, Sklabinska 26, Martin, 037 53 Slovakia. MADAR@jfmed.uniba.sk

While familial cases of Creutzfeldt-Jakob disease are extremely rare
all over the world, 3 familial clusters were observed between
1983-2000 in a relatively small area situated in the North of
Slovakia. Prevalence of CJD in this area exceeded the overall
prevalence in Slovakia more than 8 times. The majority of CJD
patients admitted consuming sheep brain. Most patients lived in
small secluded villages with rather common familial intermarriage.
CJD affected both sexes equally. All patients were prior to the
disease mentally normal individuals. Shortly after the onset of CJD
their mental status deteriorated remarkably with an average survival
rate of 3.6 months.

PMID: 12690798

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12690798&dopt=Abstract


------------------------------------------------------------------------

1: Eur J Epidemiol 1991 Sep;7(5):520-3

"Clusters" of CJD in Slovakia: the first laboratory evidence of scrapie.

Mitrova E, Huncaga S, Hocman G, Nyitrayova O, Tatara M.

Institute of Preventive and Clinical Medicine, Bratislava.

Epidemic-like occurrence of Creutzfeldt-Jakob disease was observed in
1987 in Slovakia (Orava). Search for the cause of CJD focus indicated a
coincidence of genetic and environmental risks in clustering patients.
Since Spongiform Encephalopathies might be transmitted orally, (Bovine
Spongiform Encephalopathy), the possibility of zoonotic source of CJD
cases in Orava was also considered. A deficient knowledge about the
occurrence of scrapie in Slovakia stimulated an examination of sheep
with signs of CNS disorders in two flocks of Valasky breed in Orava. In
one flock, neurohistopathological examination revealed in sheep brains
lesions characteristic for scrapie. Frozen brain tissue of these animals
were used for the detection of scrapie associated fibrils. They were
found in 2 animals from the same flock. This is the first laboratory
confirmation of scrapie in Czecho-Slovakia. The possible epidemiological
and economical implications are emphasized.

http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1761109&dopt=Abstract


TSE in Sheep Contingency Planning Assessment of Risk due to BSE
Infectivity from Disposal of Sheep A report for DEFRA November 2001

http://www.defra.gov.uk/animalh/bse/bse-publications/seac/DNVReport.pdf


TSS





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