Draft minutes of the 99th meeting held on 14th December 2007
snip...
• A member explained that a confirmed case of atypical scrapie
had been recently identified in a research project at the
Institute of Animal Health (IAH). The animal had been born in
1997 in New Zealand, imported into the UK in 1998, spending
the first six months at the Arthur Rickwood Sheep Unit
(ARSU) before transfer to the IAH site at Compton. Thus, it is
possible it may have become infected either in New Zealand,
whilst at ARSU, where two other cases have now been
confirmed, or at Compton, perhaps as a result of the
experiments conducted during the research study involving
transfusion of blood between sheep. The case is under
further investigation.
• Members considered a report describing a case of
Creutzfeldt-Jakob Disease (CJD) of prion protein gene codon
129 VV genotype who had died in 2000 at the age of 39 years
old. The case had unusual neuropathological features and
abnormal prion protein (PrPSc) western blot banding pattern1.
One possible interpretation of these data was that this could
represent the first case of vCJD in an individual of VV
genotype. However, members noted that the clinical, cerebral
Magnetic Resonance Imaging and neuropathological features
were within the range previously observed with sporadic CJD
(sCJD). Although there were similarities between the
molecular features of PrPSc in the case and those of cases of
vCJD, they were not identical and only strain typing mouse
bioassays could provide conclusive evidence about the
causative transmissible spongiform encephalopathy (TSE)
agent. The paper stated that transmission studies of this
case, in transgenic mice, were being undertaken but there
was no information about the current status of these
experiments. The Chair asked the Acting Secretary to contact
1 Mead et al. (2007) Creutzfeldt-Jakob disease, prion protein gene codon
129VV, and
a novel PrPSc type in a young British women. Arch. Neurol. 64, 1780-1784.
5
© SEAC 2007
the research group to ask about such transmission
experiments.
• A member informed SEAC that the Department for
Environment, Food and Rural Affairs (Defra) was consulting
on cost and responsibility sharing for animal health and
welfare2 and that this included specific proposals for TSE
controls between government and industry.
snip...
11. Dr Danny Matthews (Veterinary Laboratories Agency [VLA]) noted
that all three of the atypical scrapie cases associated with ARSU
are of the Cheviot breed and two were homozygous and one was
heterozygous for the AFRQ allele. It was possible that the sheep
carrying this allele may be susceptible to atypical scrapie that
arises spontaneously.
2 Defra consultation on sharing costs and responsibility: animal health and
welfare.
http://www.defra.gov.uk/news/latest/2007/animal-1211.htm
6
© SEAC 2007
12. Members asked what measures might be taken to minimise the
spread of atypical scrapie at the site referred to in paragraph 15 of
the report and were informed that manure from the Unit had been
spread on adjacent farmland, which provided a source of straw of
the Unit. It had been suggested this practice might represent a
potential route for recycling of the atypical scrapie agent, and may
be inadvisable.
13. In relation to (ii), members noted there were four hypotheses for
the interpretation of the findings from passage of two sheep TSE
cases in mice: an experimental error had occurred, the features
observed may be a normal consequence of passage of classical
scrapie isolates in the breed and genotype of sheep, a strain
conversion may have occurred or the features observed may
reflect a mixed BSE-classical scrapie infection in sheep.
14. Dr Matthews explained that an internal audit had found no
evidence of experimental error but an independent audit was
planned with Professor Alun Williams as the scientific advisor. Dr
Jim Hope (VLA) explained that the suggestion that the features
observed on strain typing of two sheep TSE cases may be a
normal consequence of passage of classical scrapie isolates from
the particular breed and genotype of sheep, had arisen as one of
the cases was an ARQ/ARQ Swaledale. Sheep of this genotype
and breed rarely succumb to classical scrapie. Only one other
case of classical scrapie in an animal of this genotype and breed
had been strain typed by mouse bioassay with the features on
passage consistent with classical scrapie.
15. The Chair considered that of the four possibilities, the first two
appear to be the least likely, the third possibility may be the most
likely but is unproven and the fourth possibility cannot be excluded.
It is important to note that the features observed on mouse
bioassay are not consistent with features observed when BSE is
passaged in mice. If the findings did reflect a mixed BSE-classical
scrapie infection, the isolates were from historic sheep TSE cases
and the probability of mixed infections was low. Thus, there is no
indication from these data of a current significant risk to human
health from BSE in sheep.
16. A member asked why the report only considered mixed infections
of classical scrapie and BSE rather than atypical scrapie as it is
known that classical and atypical scrapie can occur together. The
Chair explained that as the report described the analysis of two
sheep TSE cases that discriminatory testing indicated were cases
7
© SEAC 2007
of classical scrapie, only mixed infections of classical scrapie and
BSE had been considered.
17. A member suggested that less certainty should be reflected in the
estimate of the probability of mixed infection arising (footnote 8 of
the draft report) as the estimates relied on an assumption that BSE
and classical scrapie would occur independently. In addition, it
was incorrect to state that the most likely prevalence of BSE in
sheep was zero (paragraphs 32 and 39 of the draft report); the
sample gives an estimate of the maximum prevalence that is
consistent with the results of the survey.
snip...
ITEM 5 – SCIENTIFIC BASIS OF CLASSICAL SCRAPIE CONTROLS
(SEAC 99/3)
21. Mr Andrew Gresham (Defra) gave an overview of the background
and policy context of the issue. The European Court of First
Instance had, following an application by the French Government
and, pending a full hearing, suspended clauses in new European
Commission legislation to allow sheep from classical scrapie
affected flocks to enter the human food chain if testing negative for
TSE. The UK intended to support the Commission at the full
hearing but wished to seek SEAC’s advice in relation to possible
links between classical scrapie and human TSEs and the
performance characteristics of discriminatory tests for sheep TSEs.
SEAC had been provided with the opinions of the French Food
Safety Authority (AFSSA), the European Food Safety Authority
(EFSA) and the German TSE advisory committee (KOM AG TSE)
8
© SEAC 2007
that had considered these issues. Advice from SEAC could be
incorporated into a UK submission to the Court.
22. A member noted that the AFSSA opinion reflected concerns that
as a consequence of the release of animals from classical scrapie
affected sheep flocks into the human chain, cases of undiagnosed
BSE may also be inadvertently released into the food chain.
Furthermore, a greater number of classical scrapie infected sheep
may enter the food chain even though it is not possible to exclude
a risk to human health from classical scrapie. Three key
uncertainties had been identified by AFSSA, EFSA and KOM AG
TSE, although there were some differences in emphasis about the
uncertainties in the opinions. The uncertainties related to (i) the
capability of tests to detect TSEs in sheep during the stage when
PrPSc is accumulating in the periphery only, (ii) the ability of the
tests to detect BSE when another TSE is present and (iii) the
evidence suggesting a lack of link between human and animal
TSEs other than BSE. In relation to (iii), observations that classical
scrapie has been an endemic disease in sheep for more than 200
years without any apparent association with human disease, and
that sporadic Creutzfeldt-Jakob Disease (sCJD) exists in countries
such as Australia and New Zealand with no reported cases of
classical scrapie, are incontrovertible. However, it should be noted
that it would be very difficult to demonstrate an epidemiological link
between such relatively rare diseases in animals and humans.
Authors of two epidemiological studies3,4 that had examined risk
factors for sporadic Creutzfeldt-Jakob Disease (sCJD) dismissed a
link between classical scrapie and sCJD. However, these data
could be interpreted differently to suggest a potential link, this
could be a chance association arising from biases inherent in the
design of these retrospective studies. It was therefore important
not to be completely dismissive of a lack of a link as it would be
very difficult to prove an epidemiological link between such rare
diseases.
23. Members noted that, although there is no evidence for a risk to
human health from classical scrapie, a risk could never be ruled
out. However, even if there is a risk, the risk must be very small
indeed as the observed prevalence of sCJD is very low.
snip...
ITEM 8 – PUBLIC QUESTION AND ANSWER SESSION
40. The Chair explained that the purpose of the question and answer
session was to give members of the public an opportunity to ask
questions related to the work of SEAC. Mr Terry Singeltary
(Texas, USA) had submitted a question prior to the meeting,
asking: “With the Nor-98 now documented in five different states so
far in the USA in 2007, and with the two atypical BSE H-base
13
© SEAC 2007
cases in Texas and Alabama, with both scrapie and chronic
wasting disease (CWD) running rampant in the USA, is there any
concern from SEAC with the rise of sporadic CJD in the USA from
''unknown phenotype'', and what concerns if any, in relations to
blood donations, surgery, optical, and dental treatment, do you
have with these unknown atypical phenotypes in both humans and
animals in the USA? Does it concern SEAC, or is it of no concern
to SEAC? Should it concern USA animal and human health
officials?”
41. A member considered that this question appeared to be primarily
related to possible links between animal and human TSEs in the
USA. There is no evidence that sCJD is increasing in the USA and
no evidence of any direct link between TSEs and CJD in the USA.
Current evidence does not suggest that CWD is a significant risk to
human health. There are unpublished data from a case of human
TSE in the USA that are suggestive of an apparently novel form of
prion disease with distinct molecular characteristics. However, it is
unclear whether the case had been further characterised, if it could
be linked to animal TSEs or if other similar cases had been found
in the USA or elsewhere. In relation to the possible public health
implications of atypical scrapie, H-type BSE and CWD, research
was being conducted to investigate possible links and surveillance
was in place to detect any changes in human prion diseases.
Although possible links between these diseases and human TSEs
are of concern and require research, there is no evidence to
suggest immediate public health action is warranted. The possible
human health risks from classical scrapie had been discussed
earlier in the meeting. Members noted that there are effective
channels of discussion and collaboration on research between
USA and European groups. Members agreed it is important that to
keep a watching brief on new developments on TSEs.
ITEM 9 – UPDATE ON vCJD AND sCJD EPIDEMIOLOGY
42. Dr Richard Knight (NCJDSU) presented an update on the
epidemiology of cases of sCJD and vCJD in the UK and
elsewhere. Between May 1990 and October 2007, 944 cases of
sCJD had been identified in the UK with a mean age at onset of 66
(range 15-94) years and mean age of death of 67 (range 20-95)
years. There is no significant gender difference in sCJD incidence.
There had been a trend towards an increasing number of cases
over time to almost 80 cases per year in 2003; this increased trend
had also been observed in other countries and was considered to
be a result of better surveillance and diagnosis of disease. There
has been a decline in number since 2003, but this may not be of
15
© SEAC 2007
significance. The post mortem rate for sCJD referral is about 60%.
The genotype distribution of sCJD cases was 64% MM, 18% MV
and 18% VV at codon 129 of the prion protein gene.
43. Dr Knight explained that the total number of definite and probable
vCJD cases in the UK up to November 2007 was 166, with four
cases still alive. Three of out of four vCJD cases treated with
pentosan polysulphate (PPS) had appreciably longer survival
times, but it is not proven that this is the result of treatment. No
statistically significant gender difference had been observed in
vCJD cases. The age distribution of vCJD had not altered over the
course of the UK epidemic, with the median age of death of 30
(range 14-75) years. Statistical analysis of the UK incidence of
deaths from vCJD suggested the epidemic had peaked in 2000
with 28 deaths. There are three cases identified with onset in 2006
and four deaths in 2007. Geographical distribution of vCJD cases
in the UK shows higher incidence in the North than South. All 146
vCJD cases tested to date are of the MM genotype.
44. Dr Knight explained that elsewhere in the world up to November
2007, 39 vCJD cases have been reported with 23 in France, four in
the Republic of Ireland (RoI), three in the USA, two in the
Netherlands, two in Portugal and single cases in Italy, Canada,
Japan, Saudi Arabia and Spain. Infection is considered likely to
have occurred in the UK in two RoI cases, two USA cases, one
French case, the Japanese, and Canadian cases. One of the
French cases had a history of possibly significant residence in the
UK. One USA case is thought likely to have been exposed to
infection in Saudi Arabia, rather than the USA.
45. Dr Knight explained that the Transfusion Medicine Epidemiology
Review study had identified four instances of vCJD infection
resulting from receipt of non-leucodepleted red blood cells donated
by individuals who had subsequently developed vCJD. The donors
developed clinical vCJD ranging from 17 months to 3.5 years after
blood donation and this indicates that blood can be infective 3.5
years before the development of clinical disease. Clinical vCJD
was identified in three recipients (all of MM genotype) between 6.5
and 8.3 years after receipt of blood. The fourth recipient, who died
of non-neurological disease, with only lymphoreticular evidence of
vCJD infection was of MV genotype.
46. In response to a question about the neuropathology of the vCJD
case that died after receiving PPS, Dr Knight explained that no
autopsy was undertaken.
16
© SEAC 2007
47. A member asked about the reason for the increase in sCJD
detection in the year up to 2003. Dr Knight replied that it was
probably due to better awareness of the disease and the
availability of better diagnostic methods such as cerebrospinal fluid
testing and magnetic resonance imaging.
48. Mr Mark Noterman (Department of Health [DH]) asked whether the
neuropathological referrals rate had increased after the Chief
Medical Officer’s letter to clinicians earlier in the year to remain
vigilant about cases of neurological disease that could be related to
prion disease. Dr Knight replied that there had been no
subsequent significant increase in referral rate.
snip...
ITEM 11 – RE-ASSESSMENT OF THE POTENTIAL RISK OF vCJD
TRANSMISSION VIA DENTISTRY (SEAC 99/7)
59. Dr Bennett and Dr Peter Grove (DH) presented findings of an
interim assessment examining the risk that vCJD may be
transmitted via dental procedures. As there is a lack of substantial
19
© SEAC 2007
data with which to accurately quantify many of the key parameters
in the risk assessment, plausible ranges for parameters were
established to take account of the often large uncertainties in the
data. The key areas of uncertainty are infectivity in dental and oral
tissues of patients incubating vCJD, the level of protein residues on
dental instruments following decontamination, the efficacy of
autoclaving, the current prevalence of vCJD infection in the
population, and the epidemiology of vCJD. These uncertainties
strongly influence the quantification of the risk.
60. It was explained that many plausible scenarios built up using
ranges for each of these factors suggest that dental transmission
may have no detectable effect on the course of the vCJD epidemic.
However, there are some scenarios which include a combination of
pessimistic assumptions as regards the infectivity of dental/oral
tissues and the effects of instrument decontamination which
suggest that there could be some hundreds of vCJD transmissions
per annum via dentistry, albeit against a background of many
thousand existing subclinical vCJD infections, or where dental
transmission could generate a self-sustaining reservoir of vCJD
infection within the population. Should a large proportion of
secondary transmissions result in subclinical infections, either
never developing into clinical disease or doing so over an extended
time-scale, and such infections are infectious, the likelihood of a
self-sustaining epidemic increases. The proportion of individuals
that may be infected from having consumed BSE contaminated
food or from human to human transmission of vCJD that may enter
such a subclinical carrier state is unknown. Research to address
the key uncertainties is on-going and new data would enable some
of the assumptions underpinning these scenarios to be revised.
61. The committee welcomed the risk assessment, acknowledging it
had been developed in collaboration with a scientific reference
group of independent experts. Studies to address the scientific
uncertainties were considered important, particularly infectivity
studies on human oral and dental tissues from vCJD patients.
62. A member suggested that following secondary transmission, the
agent may adapt to become infectious to all prion protein
genotypes. Dr Grove noted that since the risk assessment
considers four scenarios ranging from one in which no secondary
infection develops into clinical disease to one in which everyone
who is infected develops clinical disease, this possibility is
considered.
20
© SEAC 2007
63. Members noted that there were two obvious precautionary
measures that could be put in place to dramatically reduce the
potential risk of vCJD transmission via dental procedures: making
endodontic files and reamers single use, which was implemented
in April 2007 and improving instrument decontamination using
current technologies. A 0.5 – 1.0 log reduction in infectivity from
improved decontamination practice could remove the risk of a self
sustaining epidemic. It is very important, therefore, that DH
ensures dentists do adopt good practice throughout the profession
and that this is audited. Introduction of consistent decontamination
practices would also reduce the observed variability of instrument
contamination, and thus reduce the risk of local outbreaks of
transmission.
64. Mr Barry Cockcroft (Chief Dental Officer, DH) noted that the effect
of the guidance on making endodontic files and reamers single use
had been included in the risk assessment. There is good evidence
that dentists are adhering to the guidance. A survey by DH
Regional Directors of Public Health could not find any dentists who
are unaware of the Chief Dental Officer’s Professional Letter
advising that files and reamers should be treated as single use
only, and dental instrument suppliers have reported that sales of
files and reamers have increased dramatically since the guidance
was issued. A draft Health Technical Memorandum would be
issued for consultation in early 2008 designed specifically for
dental practitioners and their staff as a comprehensive guide to
best practice. An audit tool will be available to help dentists assess
their own compliance with the guidance and to enable DH to
assess whether new guidance is working in practice. Dental
nurses will now also be regulated and registered with the General
Dental Council.
snip...end...full text ;
http://www.seac.gov.uk/minutes/99.pdf
SEAC stated ;
> There is no evidence that sCJD is increasing in the USA
i respectfully beg to differ. from 26 documented sCJD cases in 1996 rising to 157 documented sCJD cases in 2004, with 152 cases in 2005, to 143 in 2006, this would seem to be a rise in documented sporadic CJD cases from 1996 to me. ...TSS
CJD Cases examined
----------------------
Year / Referrals / Prion disease / Sporadic / Familial / Iatrogenic / vCJD
> 1996 / 42 / 32 / 26 / 4 / 0 / 0
> 1997 / 115 / 68 / 57 / 9 / 0 / 0
> 1998 / 93 / 53 / 45 / 7 / 1 / 0
> 1999 / 114 / 69 / 61 / 8 / 0 / 0
> 2000 / 151 / 103 / 89 / 14 / 0 / 0
> 2001 / 208 / 116 / 106 / 9 / 0 / 0
> 2002 / 255 / 143 / 118 / 23 / 2 / 0
> 2003 / 272 / 174 / 132 / 41 / 0 / 0
> 2004 / 334 / 183 / 157 / 21 / 0 / 1*
> 2005 / 352 / 195 / 152 / 37 / 1 / 0
> 2006 / 372 / 186 / 143 / 30 / 0 / 1**
> 2007 / 120 / 68 / 35 / 7 / 0 / 0
A ProMED-mail post
snip...
[2] USA: National Prion Disease Pathology Surveillance Center
Date: June 2007
Source: National Prion Disease Pathology Surveillance Center (USA) [edited]
CJD Cases examined
----------------------
Year / Referrals / Prion disease / Sporadic / Familial / Iatrogenic / vCJD
1996 / 42 / 32 / 26 / 4 / 0 / 0
1997 / 115 / 68 / 57 / 9 / 0 / 0
1998 / 93 / 53 / 45 / 7 / 1 / 0
1999 / 114 / 69 / 61 / 8 / 0 / 0
2000 / 151 / 103 / 89 / 14 / 0 / 0
2001 / 208 / 116 / 106 / 9 / 0 / 0
2002 / 255 / 143 / 118 / 23 / 2 / 0
2003 / 272 / 174 / 132 / 41 / 0 / 0
2004 / 334 / 183 / 157 / 21 / 0 / 1*
2005 / 352 / 195 / 152 / 37 / 1 / 0
2006 / 372 / 186 / 143 / 30 / 0 / 1**
2007 / 120 / 68 / 35 / 7 / 0 / 0
TOTAL / 2428*** / 1390**** / 1121 / 210 / 4 / 2
*Acquired in UK
** Acquired in Saudi Arabia
*** Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007.
**** Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1
from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36
type pending (2 from 2005, 8 from 2006, 26 from 2007).
Notes:
-- Cases are listed based on the year of death when available. If the
year of death is not available, the year of sample receipt is used.
-- Referrals: Cases with possible or probable prion disease from
which brain tissue or blood in the case of familial disease were submitted.
-- Inconclusive: Cases in which the samples were not sufficient to
make a diagnosis.
-- Non-vCJD type unknown are cases in which the tissue submitted was
adequate to establish the presence but not the type; in all cases,
vCJD could be excluded.
--
Communicated by:
Terry S. Singeltary Sr.
[In submitting these data, Terry S. Singeltary Sr. draws attention to
the steady increase in the "type unknown" category, which, according
to their definition, comprises cases in which vCJD could be excluded.
The total of 26 cases for the current year (2007) is disturbing,
possibly symptomatic of the circulation of novel agents.
Characterization of these agents should be given a high priority. - Mod.CP]
http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963
There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance
collection.
He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm
http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf
PLEASE NOTE SEAC COMMENT ;
16. A member asked why the report only considered mixed infections
of classical scrapie and BSE rather than atypical scrapie as it is
known that classical and atypical scrapie can occur together. The
Chair explained that as the report described the analysis of two
sheep TSE cases that discriminatory testing indicated were cases
7
© SEAC 2007
of classical scrapie, only mixed infections of classical scrapie and
BSE had been considered. ...END
PLEASE SEE THE FOLLOWING ;
Coexistence of multiple PrPSc types in individuals with Creutzfeldt-Jakob disease
Magdalini Polymenidou, Katharina Stoeck, Markus
Glatzel, Martin Vey, Anne Bellon, and Adriano Aguzzi
Interpretation
The regular coexistence of multiple PrPSc types in patients with CJD casts doubts on the validity of electrophoretic PrPSc mobilities as surrogates for prion strains, and questions the rational basis of current CJD classifications.
snip...
The above results set the existing CJD classifications into debate and introduce interesting questions about human CJD types. For example, do human prion types exist in a dynamic equilibrium in the brains of affected individuals? Do they coexist in most or even all CJD cases? Is the biochemically identified PrPSc type simply the dominant type, and not the only PrPSc species?
Published online October 31, 2005
http://neurology.thelancet.com
Detection of Type 1 Prion Protein in Variant Creutzfeldt-Jakob Disease
Helen M. Yull,* Diane L. Ritchie,*
Jan P.M. Langeveld,? Fred G. van Zijderveld,?
Moira E. Bruce,? James W. Ironside,* and
Mark W. Head*
From the National CJD Surveillance Unit,* School of Molecular
and Clinical Medicine, University of Edinburgh, Edinburgh,
United Kingdom; Central Institute for Animal Disease Control
(CIDC)-Lelystad, ? Lelystad, The Netherlands; Institute for Animal
Health, Neuropathogenesis Unit, ? Edinburgh, United Kingdom
Molecular typing of the abnormal form of the prion protein (PrPSc) has come to be regarded as a powerful tool in the investigation of the prion diseases. All evidence thus far presented indicates a single PrPSc molecular type in variant Creutzfeldt-Jakob disease (termed type 2B), presumably resulting from infection with a single strain of the agent (bovine spongiform encephalopathy). Here we show for the first time that the PrPSc that accumulates in the brain in variant Creutzfeldt-Jakob disease also contains a minority type 1 component. This minority type 1 PrPSc was found in all 21 cases of variant Creutzfeldt-Jakob disease tested, irrespective
of brain region examined, and was also present in the variant Creutzfeldt-Jakob disease tonsil.
The quantitative balance between PrPSc types was maintained when variant Creutzfeldt-Jakob disease was transmitted to wild-type mice and was also found in bovine spongiform encephalopathy cattle brain, indicating that the agent rather than the host specifies their relative representation. These results indicate that PrPSc molecular typing is based on quantitative rather than qualitative phenomena and point to a complex relationship between prion protein biochemistry, disease phenotype and agent strain.
(Am J Pathol 2006, 168:151-157;
DOI: 10.2353/ajpath.2006.050766)
snip...END
http://ajp.amjpathol.org/
JOURNAL OF NEUROLOGY
MARCH 26, 2003
RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
disease in the United States
Email Terry S. Singeltary:
flounder@wt.net
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?
http://www.neurology.org/cgi/eletters/60/2/176#535
THE PATHOLOGICAL PROTEIN
Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9
June 2003
BY Philip Yam
CHAPTER 14 LAYING ODDS
Answering critics like Terry Singeltary, who feels that the U.S. under-
counts CJD, Schonberger conceded that the current surveillance system
has errors but stated that most of the errors will be confined to the older
population.
http://www.thepathologicalprotein.com/
doi:10.1016/S1473-3099(03)00715-1
Copyright © 2003 Published by Elsevier Ltd.
Newsdesk
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." ...
http://www.thelancet.com/journals/laninf/article/PIIS1473309903007151/fulltext
http://download.thelancet.com/pdfs/journals/1473-3099/PIIS1473309903007151.pdf
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Singeltary, Sr et al. JAMA.2001; 285: 733-734.
http://jama.ama-assn.org/http://www.neurology.org/cgi/eletters/60/2/176#535
APHIS-2006-0041-0006 TSE advisory committee for the meeting December 15,
2006
http://www.regulations.gov/fdmspublic/ContentViewer?objectId=09000064801f3413&disposition=attachment&contentType=msw8
Subject: [Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
Spongiform Encephalopathy (BSE)
http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf
[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk
Materials for Human Food and Requirement for the Disposition of
Non-Ambulatory Disabled Cattle
9/13/2005
http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf
Like lambs to the slaughter
31 March 2001
Debora MacKenzie
Magazine issue 2284
FOUR years ago, Terry Singeltary watched his mother die horribly from a
degenerative brain disease. Doctors told him it was Alzheimer's, but
Singeltary was suspicious. The diagnosis didn't fit her violent symptoms,
and he demanded an autopsy. It showed she had died of sporadic
Creutzfeldt-Jakob disease.
Most doctors believe that sCJD is caused by a prion protein deforming by
chance into a killer. But Singeltary thinks otherwise. He is one of a number
of campaigners who say that some sCJD, like the variant CJD related to BSE,
is caused by eating meat from infected animals. Their suspicions have
focused on sheep carrying scrapie, a BSE-like disease that is widespread in
flocks across Europe and North America.
Now scientists in France have stumbled across new evidence that adds weight
to the campaigners' fears. To their complete surprise, the researchers found
that one strain of scrapie causes the same brain damage in mice as sCJD.
"This means we cannot rule out that at least some sCJD may be caused by some
strains of scrapie," says team member Jean-Philippe Deslys of the French
Atomic Energy Commission's medical research laboratory in
Fontenay-aux-Roses, south-west of Paris.
Hans Kretschmar of the University of Göttingen, who coordinates CJD
surveillance in Germany, is so concerned by the findings that he now wants
to trawl back through past sCJD cases to see if any might have been caused
by eating infected mutton or lamb. ...
http://www.newscientist.com/article/mg16922840.300-like-lambs-to-the-slaughter.html
DER SPIEGEL (9/2001) - 24.02.2001 (9397 Zeichen)
USA: Loch in der Mauer
Die BSE-Angst erreicht Amerika: Trotz strikter Auflagen gelangte in Texas
verbotenes Tiermehl ins Rinderfutter - die Kontrollen der Aufsichtsbehörden
sind lax.
Link auf diesen Artikel im Archiv:
http://service.spiegel.de/digas/find?DID=18578755
"Its as full of holes as Swiss Cheese" says Terry Singeltary of the FDA
regulations. ...
http://service.spiegel.de/digas/servlet/find/DID=18578755
2 January 2000
British Medical Journal
U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well
http://www.bmj.com/cgi/eletters/320/7226/8/b#6117
15 November 1999
British Medical Journal
vCJD in the USA * BSE in U.S.
http://www.bmj.com/cgi/eletters/319/7220/1312/b#5406
Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in
the United States
http://cjdusa.blogspot.com/
Sunday, December 16, 2007
Creutzfeldt-Jakob Disease Surveillance in Texas 2000-2006
http://cjdtexas.blogspot.com/
CJD QUESTIONNAIRE
http://cjdquestionnaire.blogspot.com/
SCRAPIE USA
http://scrapie-usa.blogspot.com/
NOR-98 ATYPICAL SCRAPIE CASES USA
http://nor-98.blogspot.com/
CREUTZFELDT JAKOB DISEASE MAD COW BASE UPDATE USA
http://cjdmadcowbaseoct2007.blogspot.com/
Transmissible Mink Encephalopathy TME
http://transmissible-mink-encephalopathy.blogspot.com/
CHRONIC WASTING DISEASE
http://chronic-wasting-disease.blogspot.com/
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
