National Prion Disease Pathology Surveillance Center Cases Examined1 as of
January 8, 2014
National Prion Disease Pathology Surveillance Center Cases Examined1
(January 8, 2014)
Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD
1996 & earlier 53 35 30 4 0 0
1997 114 68 59 9 0 0
1998 88 52 44 7 1 0
1999 123 74 65 8 1 0
2000 145 103 89 14 0 0
2001 212 120 110 10 0 0
2002 248 149 126 21 2 0
2003 266 168 137 31 0 0
2004 326 187 164 22 0 13
2005 343 194 157 36 1 0
2006 381 194 164 28 0 24
2007 377 213 185 28 0 0
2008 397 233 208 25 0 0
2009 422 256 213 42 1 0 2010 414 258 217 41 0 0
2011 411 257 215 42 0 0
2012 422 254 217 35 0 0
2013 395 246 167 54 1 0
TOTAL 51375 30616 2567 457 7 3
1 Listed based on the year of death or, if not available, on year of
referral;
2 Cases with suspected prion disease for which brain tissue and/or blood
(in familial cases) were submitted;
3 Disease acquired in the United Kingdom;
4 Disease was acquired in the United Kingdom in one case and in Saudi
Arabia in the other case;
5 Includes 10 cases in which the diagnosis is pending, and 19 inconclusive
cases;
6 Includes 27 (24 from 2013) cases with type determination pending in which
the diagnosis of vCJD has been excluded.
The sporadic cases include 2500 cases of sporadic Creutzfeldt-Jakob disease
(sCJD), 48 cases of Variably Protease-Sensitive Prionopathy (VPSPr) and 19 cases
of sporadic Fatal Insomnia (sFI).
================================================
> The sporadic cases include 2500 cases of sporadic Creutzfeldt-Jakob
disease (sCJD), ***48 cases of Variably Protease-Sensitive Prionopathy (VPSPr)
and *** 19 cases of sporadic Fatal Insomnia (sFI).
================================================
greetings TSE PRION WORLD,
i am reminded of a few things deep throat told me years ago;
*** The most frightening thing I have read all day is the report of
Gambetti's finding of a new strain of sporadic cjd in young people.........
Dear God, what in the name of all that is holy is that!!! If the US has
different strains of scrapie..... why???? than the UK... then would the same
mechanisms that make different strains of scrapie here make different strains of
BSE... if the patterns are different in sheep and mice for scrapie..... could
not the BSE be different in the cattle, in the mink, in the humans....... I
really think the slides or tissues and everything from these young people with
the new strain of sporadic cjd should be put up to be analyzed by many, many
experts in cjd........ bse..... scrapie
Scrape the damn slide and put it into mice..... wait..... chop up the mouse
brain and and spinal cord........ put into some more mice..... dammit amplify
the thing and start the damned research..... This is NOT rocket science... we
need to use what we know and get off our butts and move.... the whining about
how long everything takes..... well it takes a whole lot longer if you whine for
a year and then start the research!!!
Not sure where I read this but it was a recent press release or something
like that: I thought I would fall out of my chair when I read about how there
was no worry about infectivity from a histopath slide or tissues because they
are preserved in formic acid, or formalin or formaldehyde..... for God's
sake........ Ask any pathologist in the UK what the brain tissues in the
formalin looks like after a year....... it is a big fat sponge... the agent
continues to eat the brain ...... you can't make slides anymore because the
agent has never stopped........ and the old slides that are stained with
Hemolysin and Eosin...... they get holier and holier and degenerate and
continue... what you looked at 6 months ago is not there........ Gambetti better
be photographing every damned thing he is looking at.....
***Okay, you need to know. You don't need to pass it on as nothing will
come of it and there is not a damned thing anyone can do about it. Don't even
hint at it as it will be denied and laughed at.......... USDA is gonna do as
little as possible until there is actually a human case in the USA of the
nvcjd........ if you want to move this thing along and shake the earth.... then
we gotta get the victims families to make sure whoever is doing the autopsy is
credible, trustworthy, and a saint with the courage of Joan of Arc........ I am
not kidding!!!! so, unless we get a human death from EXACTLY the same form with
EXACTLY the same histopath lesions as seen in the UK nvcjd........ forget any
action........ it is ALL gonna be sporadic!!! And, if there is a case.......
there is gonna be every effort to link it to international travel, international
food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex
partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a
long, lonely, dangerous twisted journey to the truth. They have all the cards,
all the money, and are willing to threaten and carry out those threats.... and
this may be their biggest downfall...***
Thanks as always for your help. (Recently had a very startling revelation
from a rather senior person in government here.......... knocked me out of my
chair........ you must keep pushing. If I was a power person.... I would be
demanding that there be at least a million bovine tested as soon as possible and
agressively seeking this disease. The big players are coming out of the wood
work as there is money to be made!!!
In short: "FIRE AT WILL"!!! for the very dumb.... who's "will"! "Will be
the burden to bare if there is any coverup!"
again it was said years ago and it should be taken seriously.... BSE will
NEVER be found in the US!
As for the BSE conference call... I think you did agreat service to freedom
of information and making some people feign integrity... I find it scary to see
that most of the "experts" are employed by the federal government or are
supported on the "teat" of federal funds. A scary picture! I hope there is a
confidential panel organized by the new government to really investigate this
thing.
You need to watch your back........ but keep picking at them....... like a
buzzard to the bone... you just may get to the truth!!! (You probably have more
support than you know. Too many people are afraid to show you or let anyone else
know. I have heard a few things myself... you ask the questions that everyone
else is too afraid to ask.)
================================================
greetings again,
then i remind everyone to read this;
'As implied in the Inset 25 we must not assume that transmission of BSE to
other species will invariably present pathology typical of a scrapie-like
disease.'
see new url ;
*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***
Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014
*** chronic wasting disease, there was no absolute barrier to conversion of
the human prion protein.
*** Furthermore, the form of human PrPres produced in this in vitro assay
when seeded with CWD, resembles that found in the most common human prion
disease, namely sCJD of the MM1 subtype.
Wednesday, January 01, 2014
Molecular Barriers to Zoonotic Transmission of Prions
*** chronic wasting disease, there was no absolute barrier to conversion of
the human prion protein.
*** Furthermore, the form of human PrPres produced in this in vitro assay
when seeded with CWD, resembles that found in the most common human prion
disease, namely sCJD of the MM1 subtype.
*** The potential impact of prion diseases on human health was greatly
magnified by the recognition that interspecies transfer of BSE to humans by beef
ingestion resulted in vCJD. While changes in animal feed constituents and
slaughter practices appear to have curtailed vCJD, there is concern that CWD of
free-ranging deer and elk in the U.S. might also cross the species barrier.
Thus, consuming venison could be a source of human prion disease. Whether BSE
and CWD represent interspecies scrapie transfer or are newly arisen prion
diseases is unknown. Therefore, the possibility of transmission of prion disease
through other food animals cannot be ruled out. There is evidence that vCJD can
be transmitted through blood transfusion. There is likely a pool of unknown size
of asymptomatic individuals infected with vCJD, and there may be asymptomatic
individuals infected with the CWD equivalent. These circumstances represent a
potential threat to blood, blood products, and plasma supplies.
Thursday, January 2, 2014
*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant
Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***
From: Terry S. Singeltary Sr.
Sent: Friday, January 10, 2014 10:12 PM
To: BSE-L BSE-L
Cc: journal@neurology.org ; pxg13@case.edu ; CJD-L ; CJDVOICE CJDVOICE ;
bloodcjd bloodcjd Subject: vpspr, sgss, sffi, TSE, an iatrogenic by-product of
gss, ffi, familial type prion disease, what it ???
Greetings Friends, Neighbors, and Colleagues,
vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial type
prion disease, what it ???
Confucius is confused again.
I was just sitting and thinking about why there is no genetic link to some
of these TSE prion sGSS, sFFi, and it’s really been working on my brain, and
then it hit me today.
what if, vpspr, sgss, sffi, TSE prion disease, was a by-product from
iatrogenic gss, ffi, familial type prion disease ???
it could explain the cases of no genetic link to the gss, ffi, familial
type prion disease.
sporadic and familial is a red herring, in my opinion, and underestimation
is spot on, due to the crude prehistoric diagnostic procedures and criteria and
definition of a prion disease.
I say again, what if, iatrogenic, what if, with all these neurological
disorders, with a common denominator that is increasingly showing up in the
picture, called the prion.
I urge all scientist to come together here, with this as the utmost of
importance about all these neurological disease that are increasingly showing up
as a prion mechanism, to put on the front burners, the IATROGENIC aspect and the
potential of transmission there from, with diseases/disease??? in question.
by definition, could they be a Transmissible Spongiform Encephalopathy TSE
prion type disease, and if so, what are the iatrogenic chances of transmission?
this is very important, and should be at the forefront of research, and if
proven, could be a monumental breakthrough in science and battle against the
spreading of these disease/diseases.
the US National Library of Medicine National Institutes of Health pub-med
site, a quick search of the word SPORADIC will give you a hit of 40,747. of
those, there are a plethora of disease listed under sporadic. sporadic simply
means (UNKNOWN).
the US National Library of Medicine National Institutes of Health pub-med
site, a quick search of the word FAMILIAL will give you a hit of 921,815. of
those, there are a plethora of disease listed under familial.
again, sporadic and familial is a red herring, in my opinion.
also, in my opinion, when you start have disease such as sporadic Fatal
Familial Insomnia, (and or sporadic GSS, or the VPSPr type prion disease), and
there is NO familial genetic linkage to the family of the diseased, I have
serious questions there as to a familial type disease, and thus, being defined
as such.
because there is _NO_ genetic link to that particular family, the theory
of a familial disease is out the door, in my opinion. there has to be a source,
route, and mode of transmission, if there is no genetic link to the
family.
SPONTANEOUS HAS NEVER EVER BEEN PROVEN, ESPECIALLY IN 85%+ OF ALL HUMAN TSE
PRION CASES, AND THE FOLLOWING STATEMENT HAS NO SCIENTIFIC PROOF.
WHAT POLITICAL TSE PRION SCIENCE HAS DONE, IS TAKEN THE WORDS OF THEORY AND
HYPOTHESIS FROM SCIENTIST, AND THEM PUT IT IN PRINT, MADE THEM FACT, a terrible
unjust to society.
*** ''Gambetti, who played a key role in the discovery of fatal insomnia,
theorizes that Michael’s illness was random, despite the odds. It may be that,
by chance or some unknown factor, Michael’s brain perfectly bred its own
pathogen.''
I ALSO STRONGLY DISAGREE WITH THE STATEMENT
'OR OUTSIDE CAUSE',
IF THERE IS NO
'WITHOUT AN INHERITED CAUSE',
THEN THERE MUST BE AN OUTSIDE CAUSE, AND I PROPOSED, IN SOME CASES, IT BE
IATROGENIC.
VPSPR IS TRANSMISSIBLE
please see ;
Conclusions. Preliminary results from transmission studies in bank voles
strongly support the notion that VPSPr is a transmissible prion disease.
Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of
voles, with sCJD-like features in BvM109 and GSS-like properties in
BvI109.
GSS IS TRANSMISSIBLE
Acta Neuropathol. 1984;64(1):85-8.
Experimental transmission of human subacute spongiform encephalopathy to
small rodents. IV. Positive transmission from a typical case of
Gerstmann-Sträussler-Scheinker's disease.
Tateishi J, Sato Y, Nagara H, Boellaard JW.
Abstract
Spongiform encephalopathy was transmitted to mice from a patient belonging
to the "Sch" family with Gerstmann-Sträussler-Scheinker's disease (GSS).
Incubation periods in the first passage were much shorter than those in mice
infected with Creutzfeldt-Jakob disease. Clinical and pathologic findings of
mice infected with both diseases were almost identical. This is the first
successful transmission from a typical GSS case without severe spongiform change
which suggests the possible transmissible nature of this disorder.
FFI IS TRANSMISSIBLE
Nature 376, 434 - 435 (03 August 2002); doi:10.1038/376434a0
First experimental transmission of fatal familial insomnia
Jun Tateishi*, Paul Brown‡, Tetsuyuki Kitamoto*, Zahirul M. Hoque*, Raymond
Roos§, Robert Wollman∥, Larisa Cervenà kovà ‡ & D. Carleton Gajdusek‡
* Department of Neuropathology, Neurological Institute, Kyushu University,
Fukuoka 812, Japan ‡ Laboratory of CMS Studies, National Institute of
Neurological Disorders and Stroke, National Institutes of Health, Bethesda,
Mayland 20892, USA Departments of Neurology § and Pathology∥, University of
Chicago Medical Center, Chicago, Illinois 60637, USA
ORIGINALLY described by Lugaresi et al. in 1986 (ref. 1), fatal familial
insomnia (FFI) is a rare inherited neurological disease characterized by the
subacute progression of intractable insomnia and other autonomic abnormalities,
cerebellar and pyramidal signs, myoclonus and dementia; neuropathologically, the
major feature is severe neuronal loss with associated gliosis in the ventral and
mediodorsal thalamic nuclei. The disease has been related to the group of
spongiform encephalopathies by virtue of the presence of low levels of
proteinase-resistant amyloid protein (PrPres) in the brain2á¤-4, and of a
pathogenic single-allele mutation at codon 178 of the PRNP gene that encodes
PrF68 (refs 2, 5). Here we report the successful transmission of the disease to
experimental animals, placing FFI within the group of infectious cerebral
amyloidoses.
Wednesday, November 09, 2011
*** Case report Sporadic fatal insomnia in a young woman: A diagnostic
challenge: Case Report TEXAS
IATROGENIC
all iatrogenic cjd is, is sporadic CJD, until route and source of the
iatrogenic event that took place, is detected, documented, placed in the
academic domain as fact, and recorded, which happens very seldom due to a lot of
factors, besides the incubation period, and that be mainly industry. kind of
like asbestos and tobacco and the industry there from, they knew in the early
1900’s that they both were killing, and they both had long incubation, and
somebody chose not to do anything about if for decades and decades. kind of like
what we have here with the TSE prion disease. $$$
> In 12 of 15 hospitals with neurosurgical incidents, a decision was
made to notify patients of their potential exposure.
SO, X number of patients, from 3 hospitals, where
''exposure to potentially CJD-contaminated instruments ''
took place on these patients, the final decision NOT to tell those folks
about the potential exposure to the CJD TSE prion
insane, thus, the TSE prion agent continues to spread. ...
please see further comments here ;
Saturday, November 16, 2013
Management of neurosurgical instruments and patients exposed to
creutzfeldt-jakob disease 2013 December
Monday, January 14, 2013
Gambetti et al USA Prion Unit change another highly suspect USA mad cow
victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes
along with this BSe
VARIABLY PROTEASE-SENSITVE PRIONOPATHY IS TRANSMISSIBLE ...price of prion
poker goes up again $
OR-10 15:25 - 15:40 VARIABLY PROTEASE-SENSITIVE PRIONOPATHY IS
TRANSMISSIBLE IN BANK VOLES Nonno
OR-10: Variably protease-sensitive prionopathy is transmissible in bank
voles
Romolo Nonno,1 Michele Di Bari,1 Laura Pirisinu,1 Claudia D’Agostino,1
Stefano Marcon,1 Geraldina Riccardi,1 Gabriele Vaccari,1 Piero Parchi,2 Wenquan
Zou,3 Pierluigi Gambetti,3 Umberto Agrimi1 1Istituto Superiore di Sanità ; Rome,
Italy; 2Dipartimento di Scienze Neurologiche, Università di Bologna; Bologna,
Italy; 3Case Western Reserve University; Cleveland, OH USA
Background. Variably protease-sensitive prionopathy (VPSPr) is a recently
described “sporadic”neurodegenerative disease involving prion protein
aggregation, which has clinical similarities with non-Alzheimer dementias, such
as fronto-temporal dementia. Currently, 30 cases of VPSPr have been reported in
Europe and USA, of which 19 cases were homozygous for valine at codon 129 of the
prion protein (VV), 8 were MV and 3 were MM. A distinctive feature of VPSPr is
the electrophoretic pattern of PrPSc after digestion with proteinase K (PK).
After PK-treatment, PrP from VPSPr forms a ladder-like electrophoretic pattern
similar to that described in GSS cases. The clinical and pathological features
of VPSPr raised the question of the correct classification of VPSPr among prion
diseases or other forms of neurodegenerative disorders. Here we report
preliminary data on the transmissibility and pathological features of VPSPr
cases in bank voles.
Materials and Methods. Seven VPSPr cases were inoculated in two genetic
lines of bank voles, carrying either methionine or isoleucine at codon 109 of
the prion protein (named BvM109 and BvI109, respectively). Among the VPSPr cases
selected, 2 were VV at PrP codon 129, 3 were MV and 2 were MM. Clinical
diagnosis in voles was confirmed by brain pathological assessment and western
blot for PK-resistant PrPSc (PrPres) with mAbs SAF32, SAF84, 12B2 and 9A2.
Results. To date, 2 VPSPr cases (1 MV and 1 MM) gave positive transmission
in BvM109. Overall, 3 voles were positive with survival time between 290 and 588
d post inoculation (d.p.i.). All positive voles accumulated PrPres in the form
of the typical PrP27–30, which was indistinguishable to that previously observed
in BvM109 inoculated with sCJDMM1 cases. In BvI109, 3 VPSPr cases (2 VV and 1
MM) showed positive transmission until now. Overall, 5 voles were positive with
survival time between 281 and 596 d.p.i.. In contrast to what observed in
BvM109, all BvI109 showed a GSS-like PrPSc electrophoretic pattern,
characterized by low molecular weight PrPres. These PrPres fragments were
positive with mAb 9A2 and 12B2, while being negative with SAF32 and SAF84,
suggesting that they are cleaved at both the C-terminus and the N-terminus.
Second passages are in progress from these first successful transmissions.
Conclusions. Preliminary results from transmission studies in bank voles
strongly support the notion that VPSPr is a transmissible prion disease.
Interestingly, VPSPr undergoes divergent evolution in the two genetic lines of
voles, with sCJD-like features in BvM109 and GSS-like properties in BvI109. The
discovery of previously unrecognized prion diseases in both humans and animals
(i.e., Nor98 in small ruminants) demonstrates that the range of prion diseases
might be wider than expected and raises crucial questions about the epidemiology
and strain properties of these new forms. We are investigating this latter issue
by molecular and biological comparison of VPSPr, GSS and Nor98.
SOURCE PRION2012
SNIP...
SEE FULL TEXT ;
Friday, January 10, 2014
*** vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial
type prion disease, what it ???
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
PRIONOPATHY OR PRIONOBALONEY $$$
Monday, September 26, 2011
Variably Protease-Sensitive Prionopathy, Prionpathy, Prionopathy, FFI, GSS,
gCJD, hvCJD, sCJD, TSE, PRION, update 2011
Monday, June 27, 2011
Comparison of Sheep Nor98 with Human Variably Protease-Sensitive
Prionopathy and Gerstmann-Sträussler-Scheinker Disease
*** vpspr - None of the subjects had mutations in the PrP gene coding
region.
*** sporadic FFI - Genetic tests identified no prion protein (PrP) gene
mutation
Monday, January 14, 2013
snip...
Gambetti et al USA Prion Unit change another highly suspect USA mad cow
victim to another fake name i.e. sporadic FFI at age 16 CJD Foundation goes
along with this BSe
Only months later would Michael’s family learn the reason for his
degradation and death. An autopsy showed that the teenager died of sporadic
fatal insomnia, a subtype of Creutzfeldt-Jakob Disease.
In fact, Michael may be among the youngest ever to be affected by a
neurodegenerative disease without an inherited or outside cause.
Gambetti, who played a key role in the discovery of fatal insomnia,
theorizes that Michael’s illness was random, despite the odds. It may be that,
by chance or some unknown factor, Michael’s brain perfectly bred its own
pathogen.
snip...
sporadic GSS -
We report here an unusual sporadic case of Creutzfeldt-Jakob disease (CJD)
characterized by an abundance of prion protein (PrP)-immunopositive kuru and
multicentric but not florid plaques. Molecular genetic analysis of the PRNP open
reading frame region spanning codons 8-221 was performed. Neither deletion nor
insertion mutations were detected in the repeat area of the PRNP. No pathogenic
mutation was found in the sequenced region between codon 108-221. Restriction
analysis of the amplified fragment using restriction endonucleases DdeI, PvuII
and AluI did not show any of the previously described pathogenic mutations at
codon 102, 105, and 117 associated with Gerstmann-Sträussler-Scheinker (GSS).
The patient was heterozygous for the methionine/valine coding triplet at
polymorphic codon 129 of the PRNP gene by sequence, restriction endonuclease
analysis and hybridization with allele-specific nucleotides. Furthermore,
hybridization with 32P-labeled allele-specific oligonucleotides confirmed the
absence of pathogenic mutations at codons 102, 200 and 178. Such a case may
present a missing "link" between sporadic CJD and familial GSS.
Nor98 Prions Share Biochemical Features with Human
Gerstmann-Sträussler-Scheinker Disease and Variably Protease-Sensitive
Prionopathy
Tuesday, August 03, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the
prion protein
Tuesday, August 03, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the
prion protein
Monday, August 9, 2010
Variably protease-sensitive prionopathy: A new sporadic disease of the
prion protein or just more PRIONBALONEY ?
snip...see full text ;
Thursday, July 10, 2008
A New Prionopathy update July 10, 2008
Thursday, July 10, 2008
A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease
update July 10, 2008 Friday, June 20, 2008
Sunday, August 10, 2008
A New Prionopathy OR more of the same old BSe and sporadic CJD
Sunday, August 24, 2008
Sporadic Fatal Insomnia with Unusual Biochemical and Neuropathological
Findings
O.K. let's compare some recent cases of this prionpathy in other countries
besides Gambetti's first 10 recently, that he claims is a spontaneous event,
from a genetic disorder, that is not genetic, but sporadic, that is related to
no animal TSE in North America, or the world. ...
Sunday, August 21, 2011
The British disease, or a disease gone global, The TSE Prion Disease
(see video here)
U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ?
(see video at bottom)
Sunday, September 6, 2009
MAD COW USA 1997
(SEE SECRET VIDEO)
Sunday, August 09, 2009
***CJD...Straight talk with...James Ironside...and...Terry Singeltary...
2009
Tuesday, August 18, 2009
BSE-The Untold Story - joe gibbs and singeltary 1999 – 2009 snip... Wed, 29
Nov 2000 14:14:18 -0500
a private email from the late Dr. Gibbs, a true pioneer in the research of
human/animal TSEs and one that never wavered on helping the families and victims
of this horrible disease, and one that helped me many times in trying to seek
out the truth;
Subject: Re: Hello Dr. Gibbs...........
Date: Wed, 29 Nov 2000 14:14:18 –0500
From: "Clarence J. Gibbs, Jr., Ph.D."
To: "Terry S. Singeltary Sr." References:
<3a254430 .9fb97284="" wt.net="">
Hi Terry:
326 E Stret N.E., Washington, D. C. 20002.
Better shrimp and oysters than cards!!!!
Have a happy holiday and thanks for all the information you bring to the
screen.
Joe Gibbs
==========
2011
EFSA Journal 2011 The European Response to BSE: A Success Story
This is an interesting editorial about the Mad Cow Disease debacle, and
it's ramifications that will continue to play out for decades to come ;
Monday, October 10, 2011
EFSA Journal 2011 The European Response to BSE: A Success Story
snip...
EFSA and the European Centre for Disease Prevention and Control (ECDC)
recently delivered a scientific opinion on any possible epidemiological or
molecular association between TSEs in animals and humans (EFSA Panel on
Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical
BSE prions as the only TSE agents demonstrated to be zoonotic so far but the
possibility that a small proportion of human cases so far classified as
"sporadic" CJD are of zoonotic origin could not be excluded. Moreover,
transmission experiments to non-human primates suggest that some TSE agents in
addition to Classical BSE prions in cattle (namely L-type Atypical BSE,
Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic
wasting disease (CWD) agents) might have zoonotic potential.
snip...
see follow-up here about North America BSE Mad Cow TSE prion risk factors,
and the ever emerging strains of Transmissible Spongiform Encephalopathy in many
species here in the USA, including humans ;
Thursday, August 12, 2010
Seven main threats for the future linked to prions First threat The TSE
road map defining the evolution of European policy for protection against prion
diseases is based on a certain numbers of hypotheses some of which may turn out
to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform
Encephalopathy), recently identified by systematic testing in aged cattle
without clinical signs, may be the origin of classical BSE and thus potentially
constitute a reservoir, which may be impossible to eradicate if a sporadic
origin is confirmed.
***Also, a link is suspected between atypical BSE and some apparently
sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases
constitute an unforeseen first threat that could sharply modify the European
approach to prion diseases. Second threat
snip...
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 $$$
Monday, June 27, 2011
*** Comparison of Sheep Nor98 with Human Variably Protease-Sensitive
Prionopathy and Gerstmann-Sträussler-Scheinker Disease
Monday, September 26, 2011
L-BSE BASE prion and atypical sporadic CJD
Thursday, August 4, 2011
Terry Singeltary Sr. on the Creutzfeldt-Jakob Disease Public Health Crisis,
Date aired: 27 Jun 2011
CANADA CJD UPDATE 2011
CJD Deaths Reported by CJDSS1, 1994-20112 As of January 31, 2011
3. Final classification of 49 cases from 2009, 2010, 2011 is pending.
snip...
USA 2011
USA
National Prion Disease Pathology Surveillance Center
Cases Examined1
(November 1, 2010)
Year Total Referrals2 Prion Disease Sporadic Familial Iatrogenic vCJD
1996 & earlier 51 33 28 5 0 0
1997 114 68 59 9 0 0
1998 87 51 43 7 1 0
1999 121 73 65 8 0 0
2000 146 103 89 14 0 0
2001 209 119 109 10 0 0
2002 248 149 125 22 2 0
2003 274 176 137 39 0 0
2004 325 186 164 21 0 13
2005 344 194 157 36 1 0
2006 383 197 166 29 0 24
2007 377 214 187 27 0 0
2008 394 231 205 25 0 0
2009 425 258 215 43 0 0
2010 333 213 158 33 0 0
TOTAL 38315 22656 1907 328 4 3
1 Listed based on the year of death or, if not available, on year of
referral;
2 Cases with suspected prion disease for which brain tissue and/or blood
(in familial cases) were submitted;
3 Disease acquired in the United Kingdom;
4 Disease was acquired in the United Kingdom in one case and in Saudi
Arabia in the other case;
5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive
cases;
6 Includes 23 (22 from 2010) cases with type determination pending in which
the diagnosis of vCJD has been excluded.
Please notice where sporadic CJD cases in 1996 went from 28 cases, to 215
cases in 2009, the highest recorded year to date. sporadic CJD is on a steady
rise, and has been since 1996.
I also urge you to again notice these disturbing factors in lines 5 and 6
;
5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive
cases;
6 Includes 23 (22 from 2010) cases with type determination pending in which
the diagnosis of vCJD has been excluded.
========end=====tss=====
CJD or prion disease 2 CASES McLennan County Texas population 230,213 both
cases in their 40s 2 mysterious cases of disease in McLennan County a rarity,
but no cause for alarm
By Cindy V. Culp Tribune-Herald staff writer
Friday July 9, 2010
Two likely cases of a mysterious, fatal brain disorder have been reported
in McLennan County — a statistical anomaly considering that only one in 1
million people worldwide is affected by the condition in any given year.
Adding to the peculiarity is that the noncontagious disorder belongs to the
same family as Creutzfeldt-Jakob disease.
One of its forms is believed to be triggered by people eating meat from
cattle infected with mad cow disease.
As frightening as that might sound, officials said residents shouldn’t be
alarmed.
One of the local cases definitely is not the type associated with mad cow
disease, and there is no evidence the other one is, either. More importantly,
the disorder cannot be transmitted from person to person, officials said.
“To have potentially two cases this close together is statistically
unusual,” said Dr. Farley Verner, an infectious disease specialist who advises
the Waco-McLennan County Public Health District. “But because of the type of
disorder it is, and because of what we know about how it develops, it’s not a
worrisome coincidence. It’s just a coincidence.”
Because of privacy laws, health officials can release only limited details
about the local cases. Both were reported in May.
The first case involved a 49-year-old man from McGregor, Hammad Akram, the
health district’s epidemiologist, said. The man has since died.
Initial results from an autopsy show he had some type of human prion
disease, a family of diseases involving an abnormal protein.
Creutzfeldt-Jakob disease, or CJD, is the most common type of human prion
disease. The autopsy ruled out CJD, however, Akram said.
The second case involves a Waco woman in her late 40s, Akram said. Her
symptoms point to CJD, but since the only way to confirm the disease is to study
brain tissue after death, that diagnosis is not confirmed, he said.
No apparent link
There is no apparent link between the two local victims, Akram said.
Prion disease usually occurs in people older than age 60.
Doctors give patients a “working diagnosis” of human prion disease based on
certain symptoms, combined with results from a blood test, Farley said.
The symptoms are similar to those of other neurological conditions:
confusion, difficulty remembering recent events, loss of feeling in certain body
parts, balance problems, difficulty walking and muscle jerks and spasms.
If a physician rules out other causes for such symptoms, a blood test can
be done that indicates whether the person has a genetic mutation associated with
human prion disease. The test cannot confirm it, but positive results make the
diagnosis more likely, Verner said.
The name of the disease category comes from a protein called a prion.
People have normal prions, which are concentrated in the brain. But in some
instances, there is abnormal prion protein, which causes normal prions to be
converted to abnormal form.
That destroys brain tissue and is eventually fatal. The process can take
years, but most people die within three months to a year of having
symptoms.
There are three main categories of human prion disease — sporadic, familial
and acquired.
Sporadic cases start spontaneously, without a clearly identifiable cause.
They account for about 85 percent of all human prion disease, according to the
National Prion Disease Pathology Surveillance Center.
Familial cases are inherited and are caused by a defect in the prion
protein gene, the center said.
Acquired cases are transmitted by infection, which can occur if a person
receives a transplant infected with prion disease or undergoes surgery where
contaminated instruments are used, according to the center.
Another avenue of infection is when someone eats contaminated beef, the
center said. That’s where the connection to mad cow disease comes in.
Only three cases linked to contaminated beef have been found in the United
States, according to health officials. In all three cases, the victims are
thought to have been infected while living overseas.
In Texas, about 120 people died from human prion disease between 2000-08,
according to state data.
Last year, there were 19 probable or confirmed cases of sporadic CJD and
two familial CJD cases statewide.
McLennan County has not had any human prion disease cases in the past
decade, according to state records. Verner said he can only recall two or three
cases in the 25 years he has been here.
cculp@wacotrib.com
757-5744
> "It’s just a coincidence.”
r i g h t. $$$
cjd = one-in-a-million ???
McLennan County, Texas population 2008 230,213
Thursday, July 08, 2010
GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010
Thursday, July 08, 2010
Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from
a risk-based assessment of surgical interventions Public release date:
8-Jul-2010
Wednesday, June 16, 2010
Defining sporadic Creutzfeldt-Jakob disease strains and their transmission
properties
CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN
AND SPINAL CORD MATTER
>>> Up until about 6 years ago, the pt worked at Tyson foods where
she worked on the assembly line, slaughtering cattle and preparing them for
packaging. She was exposed to brain and spinal cord matter when she would
euthanize the cattle. <<<
>>> Up until about 6 years ago, the pt worked at Tyson foods where
she worked on the assembly line, slaughtering cattle and preparing them for
packaging. She was exposed to brain and spinal cord matter when she would
euthanize the cattle. <<<
CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN
AND SPINAL CORD MATTER
*** re-mad cow disease, the cow that stole Christmas December 23, 2003 10
years later, USA still feeding cows to cows in 2013.
FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED
VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE
OAI 2012-2013
OAI (Official Action Indicated) when inspectors find significant
objectionable conditions or practices and believe that regulatory sanctions are
warranted to address the establishment’s lack of compliance with the regulation.
An example of an OAI classification would be findings of manufacturing
procedures insufficient to ensure that ruminant feed is not contaminated with
prohibited material. Inspectors will promptly re-inspect facilities classified
OAI after regulatory sanctions have been applied to determine whether the
corrective actions are adequate to address the objectionable conditions.
ATL-DO 1035703 Newberry Feed & Farm Ctr, Inc. 2431 Vincent St.
Newberry SC 29108-0714 OPR DR, FL, FR, TH HP 9/9/2013 OAI Y
DET-DO 1824979 Hubbard Feeds, Inc. 135 Main, P.O. Box 156 Shipshewana IN
46565-0156 OPR DR, FL, OF DP 8/29/2013 OAI Y
ATL-DO 3001460882 Talley Farms Feed Mill Inc 6309 Talley Rd Stanfield NC
28163-7617 OPR FL, TH NP 7/17/2013 OAI N
NYK-DO 3010260624 Sherry Sammons 612 Stoner Trail Rd Fonda NY 12068-5007
OPR FR, OF NP 7/16/2013 OAI Y
DEN-DO 3008575486 Rocky Ford Pet Foods 21693 Highway 50 East Rocky Ford CO
81067 OPR RE, TH HP 2/27/2013 OAI N
CHI-DO 3007091297 Rancho Cantera 2866 N Sunnyside Rd Kent IL 61044-9605 OPR
FR, OF HP 11/26/2012 OAI Y
*** DEN-DO 1713202 Weld County Bi Products, Inc. 1138 N 11th Ave Greeley CO
80631-9501 OPR RE, TH HP 10/12/2012 OAI N
Ruminant Feed Inspections Firms Inventory (excel format)
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
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
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
*** Saturday, November 2, 2013 ***
Exploring the risks of a putative transmission of BSE to new species
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
Monday, December 02, 2013
*** A parliamentary inquiry has been launched today into the safety of
blood, tissue and organ screening following fears that vCJD – the human form of
‘mad cow’ disease – may be being spread by medical procedures
Wednesday, December 11, 2013
*** Detection of Infectivity in Blood of Persons with Variant and Sporadic
Creutzfeldt-Jakob Disease
Thursday, January 2, 2014
*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant
Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ??? ***
Friday, January 10, 2014
*** vpspr, sgss, sffi, TSE, an iatrogenic by-product of gss, ffi, familial
type prion disease, what it ???
Wednesday, January 15, 2014
*** INFECTION PREVENTION AND CONTROL OF CJD, VCJD AND OTHER HUMAN PRION
DISEASES IN HEALTHCARE AND COMMUNITY SETTINGS Variably Protease-Sensitive
Prionopathy (VPSPr) January 15, 2014
U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001
[host Richard Barns] and now a question from Terry S. Singeltary of CJD
Watch.
[TSS] yes, thank you, U.S. cattle, what kind of guarantee can you give for
serum or tissue donor herds?
[no answer, you could hear in the back ground, mumbling and 'we can't. have
him ask the question again.]
[host Richard] could you repeat the question?
[TSS] U.S. cattle, what kind of guarantee can you give for serum or tissue
donor herds?
[not sure whom ask this] what group are you with?
[TSS] CJD Watch, my Mom died from hvCJD and we are tracking CJD
world-wide.
[not sure who is speaking] could you please disconnect Mr. Singeltary
SNIP...SEE FULL TEXT ;
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE
RISE IN NORTH AMERICA
Thursday, February 21, 2013
National Prion Disease Pathology Surveillance Center Cases Examined January
16, 2013
Sunday, February 12, 2012
National Prion Disease Pathology Surveillance Center Cases Examined1
(August 19, 2011) including Texas
CJD QUESTIONNAIRE
CJD VOICE
TSS
