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From: TSS ()
Subject: A Case Study of Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease in Germany
Date: September 26, 2007 at 1:11 pm PST

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

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:

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:

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.

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.


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


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




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.



it's o.k. to poison 3rd world countries with BSE

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
> 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

Erkwiesenstr. 19
D-52072 Aachen (Germany)
Tel.: +49 (0)241/932070
Fax: +49 (0)241/932071

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]

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.

From: "Terry S. Singeltary Sr."
Sent: Tuesday, August 21, 2007 9:50 AM
Subject: TWO MORE Nor98 atypical Scrapie cases detected in USA bringing
total to 3 cases to date

Infected and Source Flocks

As of June 30, 2007, there were .....


One field case and one validation case were consistent with Nor-98 scrapie.

IN the February 2007 Scrapie report it only mentions ;

''One case was consistent with Nor98 scrapie.''

(please note flocks of origin were in WY, CO, AND CA. PERSONAL COMMUNCATIONS


An evaluation of scrapie surveillance in the United States



Date: Mon, 24 Sep 2007 21:31:55 -0500

I suggest that you all read the data out about h-BASE and sporadic CJD, GSS,
blood, and some of the other abstracts from the PRION2007. ...

USA BASE CASE, (ATYPICAL BSE), AND OR TSE (whatever they are calling it
today), please note that both the ALABAMA COW, AND THE TEXAS COW, both were
''H-TYPE'', personal communication Detwiler et al Wednesday, August 22, 2007
11:52 PM. ...TSS

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 ;

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

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

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

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


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

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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


MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to

comment on the CDC's attempts to monitor the occurrence of emerging

forms of CJD. Asante, Collinge et al [1] have reported that BSE

transmission to the 129-methionine genotype can lead to an alternate

phenotype that is indistinguishable from type 2 PrPSc, the commonest

sporadic CJD. However, CJD and all human TSEs are not reportable

nationally. CJD and all human TSEs must be made reportable in every

state and internationally. I hope that the CDC does not continue to

expect us to still believe that the 85%+ of all CJD cases which are

sporadic are all spontaneous, without route/source. We have many TSEs in

the USA in both animal and man. CWD in deer/elk is spreading rapidly and

CWD does transmit to mink, ferret, cattle, and squirrel monkey by

intracerebral inoculation. With the known incubation periods in other

TSEs, oral transmission studies of CWD may take much longer. Every

victim/family of CJD/TSEs should be asked about route and source of this

agent. To prolong this will only spread the agent and needlessly expose

others. In light of the findings of Asante and Collinge et al, there

should be drastic measures to safeguard the medical and surgical arena

from sporadic CJDs and all human TSEs. I only ponder how many sporadic

CJDs in the USA are type 2 PrPSc?

Copyright © 2003 Published by Elsevier Ltd.

Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.

Volume 3, Issue 8, August 2003, Page 463

“My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my
mom to hvCJD (Heidenhain variant CJD)
and have been searching for answers ever since. What I have found is that we
have not been told the truth. CWD
in deer and elk is a small portion of a much bigger problem.”

see history of USA cjd questionnaire

Sent: Monday May 28, 2007



CJD VOICE (voice for _all_ victims of human TSE)

Terry S. Singeltary Sr, Bacliff, Texas

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