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From: TSS ()
Subject: PRION2007 ABSTRACTS SPORADIC CJD AND H BASE MAD COW ALABAMA AND TEXAS SEPTEMBER 2007
Date: September 24, 2007 at 6:52 pm PST

P02.35
Molecular Features of the Protease-resistant Prion Protein (PrPres) in H-type BSE
Biacabe, A-G1; Jacobs, JG2; Gavier-Widén, D3; Vulin, J1; Langeveld, JPM2; Baron,
TGM1
1AFSSA, France; 2CIDC-Lelystad, Netherlands; 3SVA, Sweden
Western blot analyses of PrPres accumulating in the brain of BSE-infected cattle have
demonstrated 3 different molecular phenotypes regarding to the apparent molecular
masses and glycoform ratios of PrPres bands. We initially described isolates (H-type
BSE) essentially characterized by higher PrPres molecular mass and decreased levels
of the diglycosylated PrPres band, in contrast to the classical type of BSE. This type
is also distinct from another BSE phenotype named L-type BSE, or also BASE (for
Bovine Amyloid Spongiform Encephalopathy), mainly characterized by a low
representation of the diglycosylated PrPres band as well as a lower PrPres molecular
mass. Retrospective molecular studies in France of all available BSE cases older than 8 years
old and of part of the other cases identified since the beginning of the exhaustive
surveillance of the disease in 20001 allowed to identify 7 H-type BSE cases, among
594 BSE cases that could be classified as classical, L- or H-type BSE.
By Western blot analysis of H-type PrPres, we described a remarkable specific feature
with antibodies raised against the C-terminal region of PrP that demonstrated the
existence of a more C-terminal cleaved form of PrPres (named PrPres#2 ), in addition
to the usual PrPres form (PrPres #1). In the unglycosylated form, PrPres #2 migrates at
about 14 kDa, compared to 20 kDa for PrPres #1. The proportion of the PrPres#2 in
cattle seems to by higher compared to the PrPres#1. Furthermore another
PK–resistant fragment at about 7 kDa was detected by some more N-terminal
antibodies and presumed to be the result of cleavages of both N- and C-terminal parts
of PrP. These singular features were maintained after transmission of the disease to
C57Bl/6 mice.
The identification of these two additional PrPres fragments (PrPres #2 and 7kDa band)
reminds features reported respectively in sporadic Creutzfeldt-Jakob disease and in
Gerstmann-Sträussler-Scheinker (GSS) syndrome in humans.

FC5.5.1
BASE Transmitted to Primates and MV2 sCJD Subtype Share PrP27-30 and PrPSc
C-terminal Truncated Fragments

Zanusso, G1; Commoy, E2; Fasoli, E3; Fiorini, M3; Lescoutra, N4; Ruchoux,
MM4; Casalone, C5; Caramelli, M5; Ferrari, S3; Lasmezas, C6; Deslys, J-P4;
Monaco, S3 1University of Verona, of Neurological and Visual Sciences, Italy; 2CEA,
IMETI/SEPIA, France; 3University of Verona, Neurological and Visual Sciences, Italy;
4IMETI/SEPIA, France; 5IZSPLVA, Italy; 6The Scripps Research Insitute, USA


The etiology of sporadic Creutzfeldt-Jakob disease (sCJD), the most frequent human
prion disease, remains still unknown. The marked disease phenotype heterogeneity
observed in sCJD is thought to be influenced by the type of proteinase K-resistant
prion protein, or PrPSc (type 1 or type 2 according to the electrophoretic mobility of
the unglycosylated backbone), and by the host polymorphic Methionine/Valine (M/V)
codon 129 of the PRNP. By using a two-dimensional gel electrophoresis (2D-PAGE)
and imunoblotting we previously showed that in sCJD, in addition to the PrPSc type,
distinct PrPSc C-terminal truncated fragments (CTFs) correlated with different sCJD
subtypes. Based on the combination of CTFs and PrPSc type, we distinguished three
PrPSc patterns: (i) the first was observed in sCJD with PrPSc type 1 of all
genotypes,;

(ii) the second was found in M/M-2 (cortical form); (iii) the third in amyloidogenic M/V-
2 and V/V-2 subtypes (Zanusso et al., JBC 2004) . Recently, we showed that sCJD
subtype M/V-2 shared molecular and pathological features with an atypical form of
BSE, named BASE, thus suggesting a potential link between the two conditions. This
connection was further confirmed after 2D-PAGE analysis, which showed an
identical PrPSc signature, including the biochemical pattern of CTFs. To pursue this
issue, we obtained brain homogenates from Cynomolgus macaques intracerebrally
inoculated with brain homogenates from BASE. Samples were separated by using a
twodimensional electrophoresis (2D-PAGE) followed by immunoblotting. We here show
that the PrPSc pattern obtained in infected primates is identical to BASE and sCJD MV-2
subtype. These data strongly support the link, or at least a common ancestry, between a
sCJD subtype and BASE. This work was supported by Neuroprion (FOOD-CT-2004-506579)


*******************************************************


USA MAD COW CASES IN ALABAMA AND TEXAS


***PLEASE NOTE***


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


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=19779


*******************************************************

FC5.5.2 Transmission of Italian BSE and BASE Isolates in Cattle Results into a
Typical BSE Phenotype and a Muscle Wasting Disease


Zanusso, G1; Lombardi, G2; Casalone, C3; D’Angelo, A4; Gelmetti, D2; Torcoli, G2;
Barbieri, I2; Corona, C3; Fasoli, E1; Farinazzo, A1; Fiorini, M1; Gelati, M1; Iulini, B3;
Tagliavini, F5; Ferrari, S1; Monaco, S1; Caramelli, M3; Capucci, L2
1University of Verona, Neurological and Visual Sciences, Italy; 2IZSLER, Italy; 3IZSPLVA,
Italy; 4University of Turin, Animal Pathology, Italy; 5Isituto Carlo Besta, Italy


The clinical phenotype of bovine spongiform encephalopathy has been extensively
reported in early accounts of the disorder. Following the introduction of statutory active
surveillance, almost all BSE cases have been diagnosed on a pathological/molecular
basis, in a pre-symptomatic clinical stage. In recent years, the active surveillance
system has uncovered atypical BSE cases, which are characterized by distinct
conformers of the PrPSc, named high-type (BSE-H) and low-type (BSE-L), whose
clinicopathological phenotypes remain unknown. We recently reported two
Italian atypical cases with a PrPSc type similar to BSE-L, pathologically
characterized by PrP amyloid plaques. Experimental transmission to TgBov mice has recently
disclosed that BASE is caused by a distinct prion strain which is extremely virulent. A
major limitation of transmission studies to mice is the lack of reliable information on
clinical phenotype of BASE in its natural host. In the present study, we experimentally
infected Fresian/Holstein and Alpine/Brown cattle with Italian BSE and BASE isolates
by i.c. route. BASE infected cattle showed survival times significantly shorter than
BSE, a finding more readily evident in Fresian/Holstein, and in keeping with
previous observations in TgBov mice. Clinically, BSE-infected cattle developed a
disease phenotype highly comparable with that described in field BSE cases and in
experimentally challenged cattle. On the contrary, BASE-inoculated cattle
developed an amyotrophic disorder accompanied by mental dullness.
The molecular and neuropathological profiles, including PrP deposition
pattern, closely matched those observed in the original cases. This study further confirms
that BASE is caused by a distinct prion isolate and discloses a novel disease
phenotype in cattle, closely resembling the phenotype previous reported in scrapie-inoculated
cattle and in some subtypes of inherited and sporadic Creutzfeldt-Jakob disease.
Oral Abstracts
14

FC5.1.1
Transmission Results in Squirrel Monkeys Inoculated with Human sCJD, vCJD,
and GSS Blood Specimens: the Baxter Study

Brown, P1; Gibson, S2; Williams, L3; Ironside, J4; Will, R4; Kreil, T5; Abee, C3
1Fondation Alliance BioSecure, France; 2University of South Alabama, USA;
3University of Texas MD Anderson Cancer Center, USA; 4Western General Hospital, UK;
5Baxter BioSience, Austria


Background: Rodent and sheep models of Transmissible Spongiform Encephalopathy
(TSE) have documented blood infectivity in both the pre-clinical and clinical phases of
disease. Results in a (presumably more appropriate) non-human primate model
have not been reported.
Objective: To determine if blood components (red cells, white cells, platelets, and
plasma) from various forms of human TSE are infectious.
Methods: Blood components were inoculated intra-cerebrally (0.1 ml) and
intravenously (0.5 ml) into squirrel monkeys from 2 patients with sporadic
Creutzfeldt- Jakob disease (sCJD) and 3 patients with variant Creutzfeldt-Jakob disease
(vCJD). Additional monkeys were inoculated with buffy coat or plasma samples from
chimpanzees infected with either sCJD or Gerstmann-Sträussler-Scheinker
disease (GSS). Animals were monitored for a period of 5 years, and all dying or
sacrificed animals had post-mortem neuropathological examinations and Western blots to
determine the presence or absence of the misfolded ‘prion’ protein (PrPTSE).
Results: No transmissions occurred in any of the animals inoculated with
blood components from patients with sporadic or variant CJD. All donor chimpanzees
(sCJD and GSS) became symptomatic within 6 weeks of their pre-clinical phase
plasmapheresis, several months earlier than the expected onset of illness.
One monkey inoculated with purified leukocytes from a pre-clinical GSS
chimpanzee developed disease after 36 months.
Conclusion: No infectivity was found in small volumes of blood components
from 4 patients with sporadic CJD and 3 patients with variant CJD.


***However, a single transmission from a chimpanzee-passaged strain of GSS
shows that infectivity may be present in leukocytes, and the ‘shock’ of general anaesthesia and
plasmspheresis appears to have triggered the onset of illness in pre-clinical donor chimpanzees.


FC5.1.2
Interim Transmission Results in Cynomolgus Macaques Inoculated with BSE and
vCJD Blood Specimens


Lasmezas, C1; Lescoutra, N2; Comoy, E2; Holznagel, E3; Loewer, J3; Motzkus, D4;
Hunsmann, G4; Ingrosso, L5; Bierke, P6; Pocchiari, M5; Ironside, J7; Will,
R7; Deslys, JP2 1Scripps Florida, Infectology, USA; 2CEA, France; 3PEI, Germany; 4DPZ,
Germany; 5Istituto Superiore di Sanita, Italy; 6SMI, Sweden; 7CJD Surveillance Unit, UK


BSE and vCJD transmitted to cynomolgus macaques reproduce many features of
human vCJD, including clinical symptoms, neuropathological hallmarks of vCJD,
PrPres electrophoretical pattern and, most importantly, the wide distribution of
infectivity in peripheral organs. The latter characteristic distinguishes vCJD from sCJD
in both humans and cynomolgus macaques, and prompted us to use this non-human
primate model for further investigations of vCJD and its risk for human health. The
occurrence of four vCJD infections in humans transfused with blood from patients who
later developed vCJD has raised concern about blood transfusion safety in countries
with vCJD.
In this collaborative European study, we investigated the infectivity of
blood components and whole blood administered by intracerebral (ic) and
intravenous (iv) routes. Buffy-coat and whole blood was inoculated by ic and iv route,
respectively, from two vCJD patients and from two clinical vCJD-inoculated macaques.
Transfusions were also performed from whole blood and blood leucodepleted
according to hospital practice standards from two clinical BSE inoculated
macaques.
Blood infectivity during the preclinical phase is being examined in orally infected
macaques. Whole blood was collected and transfused from one such animal two
years after oral challenge, whereas buffy-coat and plasma from two animals at 2
and 4.5 years post-challenge, respectively, have been inoculated by the ic route.
This is an ongoing study in which recipient animals continue to be observed
at various times post-inoculation. So far, we have had one positive transmission in one
animal transfused 65 months earlier with 40 ml of whole blood from a vCJD macaque
(the characteristics of the disease in this animal will be shown in a separate poster by E.
Comoy). This positive transmission reproduces transfusion transmission of vCJD in
humans, with an incubation of 5.5 years compatible with incubation periods
observed in humans.

FC5.3
Assessing the Risk of vCJD Transmission by Dentistry; Distribution of
Infectivity in Oral Tissues of VM Mice after Simulated Oral Feeding of BSE-301V

Sutton, JM1; Kirby, E1; Dickinson, J1; Dennis, M1; Cornwall, M1; Vassey,
MJ1; Smith, A2; Marsh, PD3; Walker, JT1; Raven, NDH1
1Health Protection Agency, Centre for Emergency Preparedness and Response,,
TSE Research group, UK; 2University of Glasgow, Dental School, UK; 3Health
Protection Agency, Centre for Emergency Preparedness and Response,, UK


Background: Ongoing concerns about the prevalence of variant Creutzfeldt Jakob
Disease (vCJD) in the UK population has heightened concerns about the risks
of iatrogenic transmission of the disease. Although there have been no cases to
date of transmission by surgery there have been 4 cases involving blood transfusion.
This study aims to assess the potential of transmission of the disease by dental
procedures. Whilst the risks are undoubtably low the very large numbers of procedures
carried out annually have the potential to amplify the risks considerably and there is
very little data in this area to form the basis for accurate risk assessments.
Aim(s)/Objective(s): To assess the relative levels of infectivity in oral tissues from a
murine model following exposure to BSE-301V through the small intestine.
Methods.
The study uses a BSE-301V, VM mouse model as a clinically relevant model for
assessing iatrogenic vCJD transmission between humans. Infectious mouse brain
homogenate was prepared and inoculated into a loop of the duodenum, to prevent
direct contamination of the oral tissues. Mice were sacrificed at 3-weekly
intervals and at appearance of clinical symptoms. A range of oral tissues, including
dental pulp, gingival margin, salivary gland, saliva, lingual tonsil and trigeminal ganglia,
together with brain and spleen tissues were removed, processed as homogenates and
reinoculated intracranially (ic.) into indicator mice.
Results: The primary challenge proved to be a very efficient route of
infection with a 100% attack rate and a mean incubation to clinical disease of 157 ± 17 days
(compared to 120 days for the same titre inoculum ic.). Infectivity was observed in all
oral and control tissues with varying time-courses and titres estimated from incubation
period.
Discussion: The results throw new light on the potential routes of dissemination and
spread of infectivity from the small intestine to the oral cavity and its implications for
possible iatrogenic transmission of vCJD via dental, endoscopic or other forms of
surgery.
Conclusion: The data generated from the study provides support for ongoing risk
assessments to look at the potential for vCJD transmission via dental procedures
alongside other elements of studies looking at effectiveness of decontamination and
re-use of dental instruments.

P02.15
Beyond the PrPres Type1/ Type2 dichotomy in Creutzfeldt-Jakob Disease

Cassard, H1; Uro-Coste, E2; Simon, S3; Bilheude, JM4; Perret-Liaudet, A5; Ironside, J6;
Haik, S7; Basset-Leobon, C2; Lacroux, C1; Peoch’, K8; Stressenberger, N9; Langeveld,
J10; Head, M11; Hauw, JJ12; Schecher, F1; Delisle, MB13; Andreoletti, O1
1Ecole Natinale Vétérinaire de Toulouse, France; 22Service d’Anatomie Pathologique
and INSERM U466 R, France; 3DRM, CEA/Saclay, France; 4 Bio-Rad, R&D, France; 5
Hôpital Neurologique Service de Neurochimie, France; 6School of Molecular & Clinical
Medicine (Pathology),, National Creutzfeldt-Jakob Disease Surveillance Un, UK; 7Pitié
Salpetriere Universitary Hospital, France; 8Hôpital Lariboisière, Service de Biochimie et
Biologie Moléculaire,, France; 9Hôpital Neurologique -Service de Neurochimie, France;
10CIDC-Lelystad, Netherlands; 11University of Edinburgh, Western General Hospital,
UK; 12Pitié Salpetriere Universitary Hospital,, Laboratoire de Neuropathologie, France;
13Rangueil Universitary Hospital, Service d’Anatomie Pathologique, France


Creutzfeldt-Jakob disease (CJD) cases are currently classified according to
established diagnostic criteria and by the genotype at codon 129 of the PRNP gene
and the Western blotting of the proteinase K digested abnormal prion protein that
distinguishes a type 1 and a type 2 profile. These biochemically distinct PrPres types
have been proposed to represent distinct prion strains. However, since the cooccurence
of type 1 and type 2 PrPres in the same patient is common, the rationale of
this classification and strain concept as applied to CJD are currently under discussion.
Five different brain areas from of 40 sporadic CJD and 11 iatrogenic CJD (both dura
matter-, and growth hormone-associated) cases, originating from UK and France, were
systematically investigated, using Western blotting typing, and by two others
biochemical assays that depend on the behaviour of PrPSc in variable PK digestion
conditions. As described previously, co-occurrence of type 1 and type 2 PrPres was
found in 30% of the CJD patients examined. However, our novel PK concentration
dependent assays identified a single uniform PrP type in cases where both type 1 and
type 2 were present. Moreover, in sCJD four distinct biochemical PrPSc signatures
were identified by the PK concentration dependent assays and these correlated to the
current genotype/clinico-pathological sCJD groups. In iCJD the four similar
biochemical signatures were observed, but were not correlated to particular PRNP 129
polymorphism or Western Blot PrPres patterns. Moreover notable differences were
observed between PrPSc biochemical properties of French and UK GH-CJD cases,
which could reflect, as already suspected, differences in the causative agents.
Identification, in sCJD and iCJD, of four different PrPSc phenotypes irrespective of
patients PRNP polymorphism at codon 129 and Western blot profile provides new
insights into human prion disease aetiology and could reflects an unsuspected
diversity of TSE agents in human disease. Further investigations are currently
underway using animal transmission to correlate agent strain with our new discriminant
biochemical assays.


see full text 143 pages ;


http://www.prion2007.com/pdf/Prion%20Book%20of%20Abstracts.pdf

From: "Terry S. Singeltary Sr."
Subject: CWD UPDATE 88 AUGUST 31, 2007


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0709&L=sanet-mg&T=0&P=450


Date: Wed, 29 Aug 2007 21:13:08 -0500
From: "Terry S. Singeltary Sr."
Subject: CWD NEW MEXICO RECORDS IT'S 19 CASE (near Texas border again)


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=26079

Monitoring the Potential Transmission of Chronic Wasting Disease to Humans
Using a Hunter Registry Database in Wyoming (405 lines)
From: Terry S. Singeltary Sr. <[log in to unmask]>
Date: Thu, 30 Aug 2007 21:23:42 -0500


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&F=&S=&P=27654


Subject: Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease
creates a new prion strain

Date: August 25, 2007 at 12:42 pm PST


Subject: Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease
creates a new prion strain
Date: August 25, 2007 at 12:42 pm PST

J Biol Chem. 2007 Aug 20; : 17709374

Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a
new prion strain.

[My paper] Atsushi Kobayashi , Masahiro Asano , Shirou Mohri , Tetsuyuki
Kitamoto

The genotype (methionine or valine) at polymorphic codon 129 of the human
prion protein (PrP) gene and the type (type 1 or type 2) of abnormal isoform
of PrP (PrP(Sc)) are major determinants of the clinicopathological
phenotypes of sporadic Creutzfeldt-Jakob disease (sCJD). Here we found that
transmission of sCJD prions from a patient with valine homozygosity (129V/V)
and type 2 PrP(Sc) (sCJD-VV2 prions) to mice expressing human PrP with
methionine homozygosity (129M/M) generated unusual PrP(Sc) intermediate in
size between type 1 and type 2. The intermediate type PrP(Sc) was seen in
all examined dura mater graft-associated CJD cases with 129M/M and
plaque-type PrP deposits (p-dCJD). p-dCJD prions and sCJD-VV2 prions
exhibited similar transmissibility and neuropathology, and the identical
type of PrP(Sc) when inoculated into PrP-humanized mice with 129M/M or
129V/V. These findings suggest that p-dCJD could be caused by cross-sequence
transmission of sCJD-VV2 prions.


snip...


In this study, the strain-dependent traits of sCJDMM1
prions were inherited through cross-sequence
transmission without any modification. The
humanized mice with 129V/V produced type 1 PrPres
after inoculation with sCJD-MM1 prions. Because
sCJD-VV1 cases are extremely rare (at most 1-2%
of the total number of sCJD cases) and characterized
by early onset (mean age at onset: 39.3 years) (5),

####################################

our results raise the possibility that CJD cases
classified as VV1 may include cases caused by
iatrogenic transmission of sCJD-MM1 prions or
food-borne infection by type 1 prions from animals,
e.g., chronic wasting disease prions in cervid. In fact,
two CJD-VV1 patients who hunted deer or
consumed venison have been reported (40, 41). The
results of the present study emphasize the need for
traceback studies and careful re-examination of the
biochemical properties of sCJD-VV1 prions.

###################################

In conclusion, cross-sequence transmission of
sCJD-VV2 prions generates a new prion strain with
altered conformational properties and disease
phenotypes as p-dCJD prions. Furthermore, the
newly generated prions have unique transmissibility
including the traceback phenomenon. In the future, if
atypical prion strains emerge through cross-sequence
transmission, especially from animals, traceback
studies will enable us to identify the origin of the
prions.

REFERENCES...snip...end

FULL TEXT ;


http://www.jbc.org/cgi/content/abstract/M704597200v1?maxtoshow=&HITS=10&hits=10&RESULTFORMAT=&fulltext=Cross-sequence+transmission+of+sporadic+Creutzfeldt-Jakob+disease+creates+a+new+&searchid=1&FIRSTINDEX=0&resourcetype=HWCIT

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=21267

Re: Colorado Surveillance Program for Chronic Wasting Disease
Transmission to Humans (TWO SUSPECT CASES)


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0704&L=sanet-mg&T=0&P=1165

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 .....

snip...

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

http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/downloads/monthly_scrapie_rpt.pps


IN the February 2007 Scrapie report it only mentions ;

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

http://www.aphis.usda.gov/animal_health/animal_diseases/scrapie/


(please note flocks of origin were in WY, CO, AND CA. PERSONAL COMMUNCATIONS
USDA, APHIS, VS ET AL. ...TSS)

NOR98 SHOWS MOLECULAR FEATURES REMINISCENT OF GSS


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=14553


An evaluation of scrapie surveillance in the United States


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=3427

FOIA REQUEST FOR ATYPICAL TSE INFORMATION ON VERMONT SHEEP


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0708&L=sanet-mg&T=0&P=10451

Subject: FSIS NOTICE SAMPLE COLLECTION FROM CATTLE UNDER THE BOVINE
SPONGIFORM ENCEPHALOPATHY (BSE)
ONGOING SURVEILLANCE PROGRAM
From: "Terry S. Singeltary Sr."
Reply-To: Sustainable Agriculture Network Discussion Group
Date: Fri, 2 Feb 2007 17:32:58 -0600

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0702&L=sanet-mg&P=720

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...


http://www.cjdsurveillance.com/resources-casereport.html

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


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


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


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


see history of cjd questionnaire

http://brain.hastypastry.net/forums/showthread.php?t=2408


Sent: Monday May 28, 2007

Subject: THE BIG LIE SPORADIC CJD AND MAD COW DISEASEs i.e. TSE


http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0705&L=sanet-mg&T=0&P=25276

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD
only theory


TSEs have been rampant in the USA for decades in many species, and they all
have been rendered and fed back to animals for human/animal consumption.
propose that the current diagnostic criteria for human TSEs only enhances
and helps the spreading of human TSE from the continued belief of the
UKBSEnvCJD only theory in 2007. With all the science to date refuting it, to
continue to validate this myth, will only spread this TSE agent through a
multitude of potential routes and sources i.e. consumption, surgical, blood,
medical, cosmetics etc. I propose as with Aguzzi, Asante, Collinge, Caughey,
Deslys, Dormont, Gibbs, Ironside, Manuelidis, Marsh, et al and many more,
that the world of TSE Transmissible Spongiform Encephalopathy is far from an
exact science, but there is enough proven
science to date that this myth should be put to rest once and for all, and
that we move forward with a new classification for human and animal TSE that
would properly identify the infected species, the source species, and then
the route.


This would further have to be broken down to strain of species and then the
route of transmission would further have to be broken down. Accumulation and
Transmission are key to the threshold from sub-clinical to clinical disease,
and key to all this, is to stop the amplification and transmission of this
agent, the spreading of, no matter what strain. In my opinion, to continue
with this myth that the U.K. strain of BSE (one strain TSE in cows), and the
nv/v CJD (one strain TSE humans) and that all the rest of human TSE are just
one single strain i.e. sporadic CJD (when to date there are 6 different
phenotypes of sCJD, and growing per Gambetti et al), and that no other
animal TSE transmits to humans, to continue with this masquerade will only
continue to spread, expose, and kill, who knows how many more in the years
and decades to come. ONE was enough for me, My Mom, hvCJD i.e. Heidenhain
Variant CJD, DOD 12/14/97 confirmed, which is nothing more than another mans
name added to CJD, like CJD itself, Jakob and Creutzfeldt, or
Gerstmann-Straussler-Scheinker syndrome, just another CJD or human TSE,
named after another human.


WE are only kidding ourselves with the current diagnostic criteria for human
and animal TSE, especially differentiating between the nvCJD vs the sporadic
CJD
strains and then the GSS strains and also the FFI fatal familial insomnia
strains or the ones that mimics one or the other of those TSE? Tissue
infectivity and strain typing of the many variants of the human and animal
TSEs are paramount in all variants of all TSE. There must be a proper
classification that will differentiate between all these human TSE in order
to do this. With the CDI and other more sensitive testing coming about, I
only hope that my proposal will some day be taken seriously. ...

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518
flounder9@verizon.net


CJD NEWS

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CJD VOICE (voice for _all_ victims of human TSE)

http://members.aol.com/larmstr853/cjdvoice/cjdvoice.htm






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