For the first time since 2007, a case of atypical Bovine spongiform
Encephalitis (BSE) was identified in a beef cow in the German state of
Brandenburg. The Brandenburg Ministry of Health released a report stating that
the cow had not shown symptoms of the disease and thus had been slaughtered to
prepare the meat for consumption. However, since the animal was more than 10
years old, it underwent an obligatory BSE-test, which came back positive.
“This [atypical] form occurs rarely, and differentiates from classical BSE
by developing spontaneously and only in older animals,” explains veterinarian
Klaus Reimer. HealthMap reported on a similar case in 2012, when a case of
atypical BSE was found in a dairy cow in California.
The slaughtered animal was discarded according to the proper regulations
and the slaughterhouse has been disinfected. Although it is unlikely that BSE
can be transmitted through animal-to-animal contact, the herd from which the
affected animal came is currently under quarantine and the necessary
epidemiological investigations are underway.
To reduce the risk of BSE transmission to humans, Germany has strict
regulations in place on how to handle slaughtered beef. Animal by-products that
are not fit for human consumption, such as the bowel, the skull and the spine,
have to be removed and discarded properly. All beef cows eight years and older
undergo a BSE “rapid test” that quickly screens cattle for BSE. “Due to these
protective measures, no health risk exists for consumers,” said a spokesperson
for the German Federal Department of Agriculture.
BSE is a progressive, often fatal, neurological disease in cattle that
develops due to infection by a prion. Prions are not well understood, yet the
CDC explains that the most widely accepted theory is that prions are modified
proteins, or prion proteins, that can damage the central nervous system of
cattle. Researchers believe the disease may have originated in animals that were
fed animal by-products infected with BSE. A similar disease exists in humans
known as Creutzfeldt-Jakob Disease (CJD).
Since BSE develops over long periods of time, cattle may not show any signs
of infection for several years. With time, symptoms may include nervous or
aggressive behavior, abnormal posture, lack of coordination, decreased milk
production and weight loss. BSE is not contagious and cannot be transmitted
between animals, nor can the disease be spread through dairy products.
13.01.2014BSE: Erste Kontakttiere ermittelt Potsdam – Im Zusammenhang mit
der bei einem Rind im Landkreis Oder-Spree festgestellten atypischen BSE (Bovine
Spongiforme Encephalopathie) konnten jetzt erste Kontakttiere ermittelt werden.
„Nach ersten Ermittlungen konnten bisher sieben Nachkommen ermittelt werden, von
denen sich noch zwei Tiere im Bestand befinden“, so Landestierarzt Dr. Klaus
Reimer. Er verweist darauf, dass die Nachforschungen zur sogenannten
Geburtskohorte intensiv weitergeführt werden.
Bisher haben die epidemiologischen Ermittlungen ergeben, dass das
betroffene Tier insgesamt 7 Nachkommen hatte, von denen derzeit noch 2 Tiere
leben. Die Ermittlung der Geburtskohorte gestaltet sich schwieriger und dauert
derzeit noch an. Dazu müssen die Bestandsbücher des Betriebes und die Datenbank
des Herkunftssicherungs- und Informationssystems Tiere (HIT), insbesondere für
den zurückliegenden geburtsnahen Zeitraum (1 Jahr vor und 1 Jahr nach der Geburt
des betroffenen Tieres), analysiert werden. Auf jeden Fall wird sichergestellt,
dass die ermittelten Nachkommen und die Tiere der Geburtskohorte nicht in den
Nahrungskreislauf gelangen.
Die festgestellte atypische BSE ist im Rahmen der routinemäßigen
Schlachtuntersuchung im Schlachthof festgestellt worden und ein Zeichen dafür,
dass das System der vorbeugenden und vorsorglichen Kontrolle in Bezug auf BSE
funktioniert. Reimer geht davon aus, dass es sich bei der festgestellten
Erkrankung um einen Einzelfall handelt. „Wir führen jährlich etwa 8000 bis
10.000 BSE-Schnelltests bei Schlachttieren durch. Von 2007 bis 2013 gab es
keinen positiven Befund“, so der Landstierarzt.
Bis zum Abschluss der epidemiologischen Ermittlungen bleibt die Herde
gesperrt. Der Landkreis hat alle notwendigen Maßnahmen ergriffen und wird durch
die Task Force des Landes unterstützt.
Weitere Informationen:
weitere Informationen Atypische BSE (Fragen und Antworten)
SEE OIE REPORT HERE;
Saturday, January 18, 2014
Bovine spongiform encephalopathy ,Germany Information received on
17/01/2014
Monday, October 10, 2011
EFSA Journal 2011 The European Response to BSE: A Success Story
***Oral Transmission of L-type Bovine Spongiform Encephalopathy in Primate
Model
***Infectivity in skeletal muscle of BASE-infected cattle
***feedstuffs- It also suggests a similar cause or source for atypical BSE
in these countries.
http://www.neuroprion.org/resources/pdf_docs/conferences/prion2009/prion2009_bookofabstracts.pdf
***Also, a link is suspected between atypical BSE and some apparently
sporadic cases of Creutzfeldt-Jakob disease in humans.
full text ;
atypical L-type BASE BSE
Tuesday, May 1, 2012
BSE MAD COW LETTERS TO USDA (Tom Vilsack, Secretary of Agriculture) and FDA
(Magaret Hamburg, Commissioner of FDA) May 1, 2012
Sunday, December 15, 2013
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED
VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
Friday, January 17, 2014
*** Annual report of the Scientific Network on BSE-TSE EFSA, Question No
EFSA-Q-2013-01004, approved on 11 December 2013 TECHNICAL REPORT
P04.40 A Case Study of Bovine Spongiform Encephalopathy and Variant
Creutzfeldt-Jakob Disease in Germany
Lewis, R; Krewski, D; Tyshenko, MG. University of Ottawa, McLaughlin
Centre, Canada
Despite the emergence of Bovine Spongiform Encephalopathy (BSE) throughout
Europe, Germany considered itself an oasis that was free of the disease.
However, on November 26, 2000 the first domestic case of BSE appeared in the
country and public concerns over food and health safety ensued. Germany
traditionally is known for its large agriculture and farming sectors with health
and environmentally conscientious citizens. The occurrence of BSE within the
country borders had dramatic economic and social effects. The response of
Germany’s public and politicians far surpassed that of most other countries
affected by the disease. In Germany the reaction to BSE and perception of risk
was even more intense than that of the United Kingdom, which to date is still
the region that has been most affected by BSE and variant Creutzfeldt-Jakob
disease (vCJD). Response to BSE was rapid with new policies and high percentage
testing of cattle implemented within months. To date, Germany has not had a
single case of vCJD.
MISSING the bigger picture i.e. sporadic CJD and BSE/BASE, both of which
have been related to the sporadic CJD as well, BOTH of which increased in
numbers, and decreased, at or about the same time period of the increase and
decrease of BSE cases (BASE however, seems to be increasing). ...tss
Subject: CJD DOUBLES IN GERMANY FROM 1993 TO 2005 Date: May 1, 2007 at 8:47
am PST
© The Author (2007). Published by Oxford University Press on behalf of the
Guarantors of Brain. All rights reserved. For Permissions, please email:
journals.permissions@oxfordjournals.org
Creutzfeldt–Jakob disease in Germany: a prospective 12-year surveillance U.
Heinemann1, A. Krasnianski1, B. Meissner1, D. Varges1, K. Kallenberg2, W. J.
Schulz-Schaeffer3, B. J. Steinhoff4, E. M. Grasbon-Frodl5, H. A. Kretzschmar5
and I. Zerr1 1National TSE Reference Center at Department of Neurology,
Georg-August University Göttingen, Germany, 2Department of Neuroradiology,
Georg-August University Göttingen, Germany, 3Department of Neuropathology,
Georg-August University Göttingen, Germany, 4Epilepsy Center Kork, Diakonie
Kork, Germany and 5Department of Neuropathology, Ludwig-Maximilian University
Munich, Germany
Correspondence to: Inga Zerr, MD, National Reference Center for TSE,
Department of Neurology, Georg-August University Göttingen, Robert-Koch-Strasse
40, 37075 Göttingen, Germany E-mail: IngaZerr@med.uni-goettingen.de
Creutzfeldt–Jakob disease (CJD) is a rare and fatal neurodegenerative
disorder with a worldwide incidence of 1–1.5 per million. As in other countries,
a CJD surveillance unit with a clinical and neuropathological approach was
established in Goettingen (Germany) in 1993. Here we report the epidemiological
data from a prospective 12-year surveillance. Since 1993, there has been an
increasing incidence of CJD, from 0.7 in 1993 to 1.6 in 2005 with a quite stable
level since 1998. During this period, the proportion of patients with MV and VV
codon 129 genotype rose, possibly because of better identification of atypical
subtypes. Six percent of all patients had a PRNP mutation, mainly D178N-129M
(FFI), E200K and V210I. Iatrogenic CJD was a rare phenomenon. No patient
infected by cadaveric growth hormone extracts was reported. Furthermore, no
variant CJD patient has yet been identified in Germany. Differential diagnoses
revealed a variety of neurodegenerative diseases, with Alzheimer's disease in
the lead. One-third of the non-CJD patients included in this study suffered from
a potentially treatable disorder such as metabolic or inflammatory diseases. The
incidence and mortality rates in Germany are similar to those in other European
countries. In contrast, however, acquired forms, such as iatrogenic and variant
CJD are still rare in Germany or have not yet been identified.
Key Words: CJD; dementia; epidemiology; diagnosis; CSF; MRI; codon 129
genotype; genetic CJD; reversible/treatable dementia
Copyright © 2006 Elsevier B.V. All rights reserved.
Atypical BSE in Germany—Proof of transmissibility and biochemical
characterization
A. Buschmanna, A. Gretzschela, A.-G. Biacabeb, K. Schiebelc, C. Coronad, C.
Hoffmanna, M. Eidena, T. Baronb, C. Casaloned and Martin H. Groschupa, ,
aFriedrich-Loeffler-Institut (FLI), Institute for Novel and Emerging
Infectious Diseases, Boddenblick 5a, 17493 Greifswald, Insel Riems, Germany
bAFSSA-Lyon, Unite ATNC, Lyon, France cInstitut für Biochemie, Universitity
Erlangen-Nürnberg, Germany dCEA, Instituto Zooprofilattico di Turino, Turin,
Italy
Received 11 January 2006; revised 23 May 2006; accepted 2 June 2006.
Available online 17 August 2006.
Abstract Intensive active surveillance has uncovered two atypical German
BSE cases in older cattle which resemble the two different atypical BSE
phenotypes that have recently been described in France (designated H-type) and
Italy (designated L-type or BASE). The H-type is characterized by a
significantly higher molecular size, but a conventional glycopattern of the
proteinase K treated abnormal prion protein (PrPSc), while the L-type PrPSc has
only a slightly lower molecular size and a distinctly different glycopattern. In
this paper we describe the successful transmission of both German atypical BSE
cases to transgenic mice overexpressing bovine PrPC. Upon challenge with the
L-type, these mice developed BSE after a substantially shorter incubation period
than any classical BSE transmission using these mice to date. In contrast, the
incubation period was distinctly prolonged when these mice were challenged with
the H-type. PrPSc accumulated in the brains of these mice were of the same
atypical BSE type that had been used for the transmission. These atypical cases
suggest the possible existence of sporadic BSE cases in bovines. It is thus
feasible that the BSE epidemic in the UK could have also been initiated by an
intraspecies transmission from a sporadic BSE case.
Keywords: BSE; Cattle; PrPSc; Biochemical differentiation
Subject: BSE in Germany - Update Covering 2006 compared to USA Date:
February 1, 2007 at 3:09 pm PST
Released: Feb 1 2007 Germany | BSE in Germany - Update Covering 2006 GM7004
Highlight: In 2006, 16 cases of BSE were confirmed in Germany, compared to 32 in
2005. This brings the total number of BSE cases to 405, since it was first
detected in Germany in November 2000. In June 2006, Germany abolished its
stricter BSE testing requirements and replaced it with the standard EU testing
regime. Beef consumption is still below the pre-BSE level, primarily because of
healthier consumer eating habits rather than fears of BSE.
snip...
BSE tests
In 2006, a total of 1,888,053 animals were tested for BSE in Germany, of
which 16 BSE
cases were confirmed. Of the total, eight cases were discovered through
routine testing at
slaughter.
GM6020 06/19/2006 Germany Raises BSE Testing Age to EU Level
GM6004 02/16/2006 Germany plans to adjust BSE testing age to EU level
GM6003 01/27/2006 BSE in Germany - Update Covering 2005
GM3006 02/27/2003 German Cattle Identification and Beef Labeling
GM1033 11/27/2001 One year after the detection of BSE in Germany
(Includes a detailed outline of the German risk management
system)
DID THE U.K. START THE BASE EPIDEMIC AS WELL ???
STRICTLY CONFIDENTIAL
SEE;
BSE FRENCH AND GERMANS
SEE;
URL DOES NOT WORK AND NO RECORD I CAN FIND...TSS
In your letter, you accurately state that I am being pressed to take
political decisions in this area. This is due to the fact that, in the opinion
of experts, BSE may also pose a risk to humans, although the extent of this risk
and the conclusions to be drawn are a matter of contention.
Great political pressure has built up here in Germany and I have to take
account of it. This pressure comes from the field of scientific debate and the
media, but above all from Bundestag and the Bundescrat, which has a strong
position in the legislative process in Germany. ...94/06.16/11.1
British Beef Exports
see;
BSE-GERMANY PROPOSAL FOR COSMETICS PRODUCTS
SEE ;
EXPORT of bone-in beef from UK, to other member state BSE
see;
e) one way for the industry to take the damages action would be for it to
send a consignment of beef to Germany after the regulation came into affect, and
to have it turned back at the German border.
see ;
CONFIDENTIAL - POLICY AND COMMERCIAL
BSE: LEGAL ACTIONS AGAINST GERMANY
see ;
it's o.k. to poison 3rd world countries with BSE
CONFIDENTIAL POLICY
‘’WE WOULD NOT RECOMMEND AGREEING TO A BAN ON OUR EXPORTS TO THIRD
COUNTRIES AND THIS HAS NOT FEATURED IN THE RECENT DISCUSSIONS.’’
see ;
Subject: Re: no further BSE-tests in NRW (Germany) From: "Roland Heynkes @
T-Online" Reply-To: Bovine Spongiform Encephalopathy Date: Tue, 29 Jun 1999
00:20:09 +0200 Content-Type: text/plain
Dear Terry,
> Roland, that's rather scarey..............
> But, what I find most interesting, is that a country as small as
Germany, can
> test 5,029 cattle for B.S.E., in about 4 months. But, it takes the
U.S.A. 9
> years to test 7,749 cattle for B.S.E. With the cattle population in
the U.S.,
> compared to that of Germany, it would look as if though, they really
were not > looking very hard, for B.S.E. in the United States..............
> Germany is not so small and about 140,000 dollars for this 5000 tests
are peanuts even for Nordrhein-Westfalen which is only a relatively small part
of Germany. This are less than 28 dollar for each animal or a few cent per
steak. This is essentially nothing and of course much less than the decrease of
the prize for cattle as a consequence of the erosion of trust. I am sure we
agree that the costs are not the reason for the test stop in NRW or the refusal
to test in other countries. The real reason not to test for BSE is of cause the
fear to find something.
The 5029 Prionics tests in NRW are not comparable with the 7749 tests in
the USA, because the Prionics test is much faster and cheaper.
best regards
Roland Heynkes
FURTHER DISCUSSION on BSE-L about BSE risk in Germany (these are old
discussions). ...TSS
Atypical scrapie cases in Germany and France are identified by discrepant
reaction patterns in BSE rapid tests.Buschmann A, Biacabe AG, Ziegler U, Bencsik
A, Madec JY, Erhardt G, Lühken G, Baron T, Groschup MH. Federal Research Centre
for Virus Diseases of Animals, Institute for Novel and Emerging Infectious
Diseases, Boddenblick 5a, 17493 Greifswald-Insel Riems, Germany.
The intensified surveillance of scrapie in small ruminants in the European
Union (EU) has resulted in a substantial increase of the number of diagnosed
cases. Four rapid tests which have passed the EU evaluation for BSE testing of
cattle are also recommended currently and used for the testing of small
ruminants by the EU authorities. These tests include an indirect ELISA (cELISA),
a colorimetric sandwich ELISA (sELISA I), a chemiluminescent sandwich ELISA
(sELISA II), and a Western blot (WB). To this point, the majority of samples
have been screened by using either sELISA I (predominantly in Germany) or WB
(predominantly in France). In this study, it is shown that a number of the
German and French scrapie cases show inconsistent results using rapid and
confirmatory test methods. Forty-eight German sheep, 209 French sheep and 19
French goat transmissible spongiform encephalopathy (TSE) cases were tested. All
cases were recognised by the sELISA I and either one of the confirmatory methods
(scrapie-associated fibrils (SAF)-immunoblot or immunohistochemistry).
Surprisingly, three rapid tests failed to detect a significant number of scrapie
cases (29 in France and 24 in Germany). The possible reasons for these
inconsistent reaction patterns of scrapie cases are discussed. Similar
discrepancies have not been observed during rapid testing of cattle for BSE, the
disease for which all diagnostic methods applied have been evaluated.
PMID: 15019257 [PubMed - indexed for MEDLINE]
P03.141 Aspects of the Cerebellar Neuropathology in Nor98
Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E1 1National
Veterinary Insitute, Sweden; 2National Veterinary Institute,
Norway Nor98 is a prion disease of old sheep and goats. This atypical form
of scrapie was first described in Norway in 1998. Several features of Nor98 were
shown to be different from classical scrapie including the distribution of
disease associated prion protein (PrPd) accumulation in the brain. The
cerebellum is generally the most affected brain area in Nor98. The study here
presented aimed at adding information on the neuropathology in the cerebellum of
Nor98 naturally affected sheep of various genotypes in Sweden and Norway. A
panel of histochemical and immunohistochemical (IHC) stainings such as IHC for
PrPd, synaptophysin, glial fibrillary acidic protein, amyloid, and cell markers
for phagocytic cells were conducted. The type of histological lesions and tissue
reactions were evaluated. The types of PrPd deposition were characterized. The
cerebellar cortex was regularly affected, even though there was a variation in
the severity of the lesions from case to case. Neuropil vacuolation was more
marked in the molecular layer, but affected also the granular cell layer. There
was a loss of granule cells. Punctate deposition of PrPd was characteristic. It
was morphologically and in distribution identical with that of synaptophysin,
suggesting that PrPd accumulates in the synaptic structures. PrPd was also
observed in the granule cell layer and in the white matter. The pathology
features of Nor98 in the cerebellum of the affected sheep showed similarities
with those of sporadic Creutzfeldt-Jakob disease in humans.
----- Original Message
-----
From: Terry S. Singeltary Sr.
To: TERRY SINGELTARY
Sent: Tuesday,
October 27, 2009 12:36 PM
Subject: CJD GERMANY
CJK in
Deutschland
Stand 06.10.2009
Jahr sicher wahrscheinlich
möglich GSS FFI genetische
CJD
Iatrogen vCJK
Inzidenz
1993 24 8 4 1 0 0 0 - 0,7
1994 45 27 26 0 2 4 1 -
0,9
1995 64 23 15 1 2 2 0 - 1,1
1996 55 34 22 1 5 5 1 - 1,1
1997 73 34
31 1 1 6 1 - 1,3
1998 63 54 11 1 3 7 0 - 1,4
1999 68 35 5 0 1 9 1 -
1,3
2000 53 55 4 2 1 7 1 - 1,3
2001 69 55 11 0 3 8 0 - 1,5
2002 52 46 6
0 2 7 0 - 1,2
2003 52 63 8 1 1 3 3 - 1,4
2004 80 60 5 1 4 4 0 -
1,7
2005 62 82 9 0 5 9 2 - 1,8
2006 61 80 8 0 4 5 1 - 1,7
2007 48 83 14
0 3 1 0 - 1,6
2008 57 78 9 0 3 0 0 - 1,6
2009 16 70 8 1 4 1 0 -
1,4*
suspected
CJD cases ≤50 years
|
|
Year |
suspected
cases≤50
|
definite & probable
cases ≤50
|
1993 |
0
|
0
|
1994 |
20
|
3
|
1995 |
14
|
3
|
1996 |
32
|
5
|
1997 |
13
|
6
|
1998 |
20
|
7
|
1999 |
12
|
7
|
2000 |
9
|
4
|
2001 |
15
|
5
|
2002 |
15
|
4
|
2003 |
19
|
9
|
2004 |
10
|
4
|
2005 |
18
|
8
|
2006 |
26
|
5
|
2007 |
28
|
7
|
2008 |
40
|
4
|
2009 |
27
|
4
|
2010 |
20
|
7
|
2011 |
14
|
4
|
2012 |
8
|
4
|
2013 |
7
|
3
|
2014 |
-
|
-
|
Gesamt |
367
|
103
|
|
However, a BSE expert said that consumption of infected material is the
only known way that cattle get the disease under natural conditons.
*** “In view of what we know about BSE after almost 20 years experience,
contaminated feed has been the source of the epidemic,” said Paul Brown, a
scientist retired from the National Institute of Neurological Diseases and
Stroke. BSE is not caused by a microbe. It is caused by the misfolding of the
so-called “prion protein” that is a normal constituent of brain and other
tissues. If a diseased version of the protein enters the brain somehow, it can
slowly cause all the normal versions to become misfolded. It is possible the
disease could arise spontaneously, though such an event has never been recorded,
Brown said.
*** What irks many scientists is the USDA’s April 25 statement that the
rare disease is “not generally associated with an animal consuming infected
feed.” The USDA’s conclusion is a “gross oversimplification,” said Dr. Paul
Brown, one of the world’s experts on this type of disease who retired recently
from the National Institutes of Health. "(The agency) has no foundation on which
to base that statement.”
2012 ATYPICAL L-TYPE BSE BASE CALIFORNIA ‘confirmed’ Saturday, August 4,
2012
*** Final Feed Investigation Summary - California BSE Case - July 2012
Saturday, August 14, 2010
BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and
VPSPr PRIONPATHY
(see mad cow feed in COMMERCE IN ALABAMA...TSS)
Friday, January 17, 2014
*** Annual report of the Scientific Network on BSE-TSE EFSA, Question No
EFSA-Q-2013-01004, approved on 11 December 2013
Sunday, December 15, 2013
*** FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED
VIOLATIONS OFFICIAL ACTION INDICATED OAI UPDATE DECEMBER 2013 UPDATE
Saturday, December 21, 2013
**** Complementary studies detecting classical bovine spongiform
encephalopathy infectivity in jejunum, ileum and ileocaecal junction in
incubating cattle ****
Wednesday, December 4, 2013
*** Bovine Spongiform Encephalopathy; Importation of Bovines and Bovine
Products; Final Rule Federal Register / Vol. 78 , No. 233 / Wednesday, December
4, 2013
Saturday, November 2, 2013
*** APHIS Finalizes Bovine Import Regulations in Line with International
Animal Health Standards while enhancing the spread of BSE TSE prion mad cow type
disease around the Globe
Tuesday, October 29, 2013
VARIANT CJD PRESENTS DIFFERENTLY IN OLDER PATIENTS
Wednesday, October 09, 2013
*** WHY THE UKBSEnvCJD ONLY THEORY IS SO POPULAR IN IT'S FALLACY,
£41,078,281 in compensation REVISED
Thursday, October 10, 2013
CJD REPORT 1994 increased risk for consumption of veal and venison and lamb
Friday, August 16, 2013
*** Creutzfeldt-Jakob disease (CJD) biannual update August 2013 U.K. and
Contaminated blood products induce a highly atypical prion disease devoid of
PrPres in primates
WHAT about the sporadic CJD TSE proteins ?
WE now know that some cases of sporadic CJD are linked to atypical BSE and
atypical Scrapie, so why are not MORE concerned about the sporadic CJD, and all
it’s sub-types $$$
Creutzfeldt-Jakob Disease CJD cases rising North America updated report
August 2013
*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada
seeing an extreme increase of 48% between 2008 and 2010 ***
Sunday, October 13, 2013
*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012
TSS