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Ranchers.net

Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Location: Virus and Prion Diseases of Livestock

Title: BSE: description of typical and atypical cases


Author

Richt, Juergen


Submitted to: American College of Veterinary Internal Medicine
Publication Type: Proceedings/Symposium
Publication Acceptance Date: April 1, 2007
Publication Date: June 6, 2007
Citation: Richt, J. 2007. BSE: description of typical and atypical cases.
In: Proceedings of the American College of Veterinary Internal Medicine.
2007 ACVIM Forum, June 6-9, 2007, Seattle, Washington. Paper No. 159.

Technical Abstract: Introduction Transmissible spongiform encephalopathy
(TSE) agents or prions induce fatal neurodegenerative diseases in humans and
in other mammalian species. They are transmissible among their species of
origin, but they can also cross the species barrier and induce infection
and/or disease in other species. Human TSEs include Creutzfeldt¿Jakob
disease (CJD), Gerstmann¿Sträussler¿Scheinker syndrome (GSS), Kuru and fatal
familial insomnia (FFI) (1). In animals, four distinct TSE diseases are
recognized: scrapie in sheep and goats, transmissible mink encephalopathy
(TME) in mink, chronic wasting disease (CWD) in cervids, and bovine
spongiform encephalopathy (BSE) in cattle. Although considerable research
has been undertaken, the precise nature of the causative agent remains
controversial. A number of theories describe the etiology, however the
"protein only" theory has emerged to dominate the literature. Bovine
Spongiform Encephalopathy (BSE) Widely referred to as "mad cow disease", BSE
was first identified as a TSE of cattle in the mid 1980s in the U.K. and
more than 180,000 positive cases have been diagnosed in the U.K. to date.
BSE is also transmissible via BSE-contaminated feed to cats (feline
spongiform encephalopathy, FSE) and exotic ungulates (exotic ungulate
encephalopathy, EUE) (2, 3). BSE is a chronic degenerative disease affecting
the central nervous system of cattle. Affected animals display changes in
temperament, abnormal posture, incoordination and difficulty in rising,
decreased milk production, and/or loss of body weight despite continued
appetite (4). The average incubation period is about 4-6 years and all
affected animals succumb to the disease (5). Following the onset of clinical
signs, the animal's condition deteriorates until it either dies or is
destroyed. This process usually takes from 2 weeks to 6 months. Most cases
in the U.K. occurred in dairy cows between 3 and 6 years of age with the
highest susceptibility to infection being in the first 6 months of life;
adult cattle appear to be at relatively low risk of infection (6). Using
epidemiological surveillance programs, many European and non-European
countries have discovered BSE-positive animals within the last decade (7)
(8). BSE has been reported in native-born cattle in twenty-four countries
with a geographic distribution that includes Europe, the Middle East, North
America, and Asia. These outbreaks, caused by the consumption of infected
meat and bone meal containing a malformed prion protein, have resulted in
the destruction of thousands of cattle and have caused significant economic
losses. All currently validated diagnostic tests for BSE require brain
tissue. There are no validated ante mortem tests for BSE available at
present. "Typical" versus "Atypical" BSE cases Molecular characterization of
the abnormal form of the prion protein, called PrPres, has allowed the
identification of "atypical" cases of BSE cases in cattle. BSE in cattle was
considered to be a disease with unique features (9) and the majority of BSE
cases so far have been defined as "typical" BSE cases. However, unusual or
"atypical" cases of BSE have been reported in the past 3 years by
investigators from several countries. Most of these animals were greater
than 8 years of age and of various breeds. There have been two molecular
types of "atypical" BSE isolates described in the literature so far: (i) a
type with a lower molecular mass of the unglycosylated isoform also called
the L-type and (ii) a type with a higher molecular mass of the
unglycosylated isoform, also called the H-type. The L-type has been found in
cattle in Italy (10), Japan (11), Germany (12) and Belgium (13). So far, the
H-type has been described in cattle from France (14), Germany (12) and the
United States (15). The U.S. cases were animals born and raised in the U.S.
(Texas, Alabama). Unusual cases of BSE are an unexpected finding since it
was previously believed that BSE disease in cattle is caused by a single
strain of infectious agent, which has been shown to be very consistent and
uniform in appearance, even after transmission to other species. The reports
of unusual phenotypes of BSE in cattle suggest that different PrPSc
phenotypes exist in cattle with BSE. There are several hypotheses which can
explain these findings: (i) there are variants of the BSE agent with
different molecular features in cattle; (ii) cattle may have been infected
by another source of an infectious prion agent (e.g. scrapie or CWD); or
(iii) a rare sporadic or genetic form of TSE disease could exist in cattle
as described for humans.


http://arsserv0.tamu.edu/research/publications/publications.htm?seq_no_115=208195



Research Project: TRANSMISSION, DIFFERENTIATION, AND PATHOBIOLOGY OF
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES
Location: Virus and Prion Diseases of Livestock

Title: Experimental transmission of transmissible spongiform
encephalopathies (scrapie, chronic wasting disease, transmissible mink
encephalopathy) to cattle and their differentiation from bovine spongiform
encephalopathy


Authors

Hamir, Amirali
Cutlip, Randall - ARS RETIRED
Miller, Janice - ARS RETIRED
Kunkle, Robert
Richt, Juergen
Greenlee, Justin
Nicholson, Eric
Kehrli, Marcus


Submitted to: World Association of Veterinary Laboratory Diagnosticians
Publication Type: Proceedings/Symposium
Publication Acceptance Date: August 10, 2007
Publication Date: November 11, 2007
Citation: Hamir, A.N., Cutlip, R.C., Miller, J.M., Kunkle, R.A., Richt,
J.A., Greenlee, J.J., Nicholson, E.M., Kehrli, Jr., M.E. 2007. Experimental
transmission of transmissible spongiform encephalopathies (scrapie, chronic
wasting disease, transmissible mink encephalopathy) to cattle and their
differentiation from bovine spongiform encephalopathy. In: Proceedings of
the World Association of Veterinary Laboratory Diagnosticians 13th
International Symposium, November 11-14, 2007, Melbourne, Australia. p. 29.

Technical Abstract: Introduction: Experimental cross-species transmission of
TSE agents provides valuable information for identification of potential
host ranges of known TSEs. This report provides a synopsis of TSE (scrapie,
CWD, TME) transmission studies that have been conducted in cattle and
compares these findings to those seen in animals with BSE. Materials &
Methods: Generally 6-month-old bull calves were obtained and assigned to
inoculated and control groups. Inoculated calves were housed in a Biosafety
Level 2 isolation barn at the National Animal Disease Center (NADC), Ames,
Iowa. Calves were inoculated intracerebrally with 1 ml of a 10% TSE brain
inoculum. Results: Results of various TSE cattle experiments with
intracerebral inoculation of scrapie, CWD and TME are shown in tabular form
(Table 1). Table 1. Comparison of experimental scrapie, chronic wasting
disease (CWD) and transmissible mink encephalopathy (TME) in cattle
inoculated by the intracerebral route during first passage of the inocula.
Abnormal CNS signs: Scrapie. Anorexia, weight loss, leg and back stiffness.
Some showed incoordination and posterior weakness. Eventual severe lethargy.
CWD. Anorexia, weight loss, occasional aimless circling, listlessness and
excited by loud noises. TME. Variable hyperexcitability with occasional
falling to the ground. Some showing circling and aggressive behavior.
Incubation (survival) time: Scrapie. 14 ¿ 18 months. CWD. 23 ¿ 63 months.
TME. 13 ¿ 16 months. Attack rate: Scrapie. 100%. CWD. CWD from mule deer:
38%. CWD from elk: 86%. TME. 100% Histopatholgic lesions: Scrapie. Some
vacuolation and central of chromatolysis of neurons. CWD. Isolated
vacuolated neurons, a few degenerate axons, and a mild astrocytosis. TME.
Extensive vacuolation of neuronal perikarya and neuropil. Presence of mild
multifocal gliosis. Western blot (brainstem): Scrapie. All three isoforms of
PrP**res present. CWD. All three isoforms of PrP**res seen. TME. All three
isoforms of PrP**res seen. Immunohistochemistry: PrP**res in lymphoreticular
tissues: Scrapie. Not present. CWD. Not present. TME. Not present. PrP**res
in CNS: Scrapie. Present within perikaryon and processes of neurons. CWD.
Multifocal distribution with labeling primarily in glial cells (astrocytes).
TME. Diffusely present and usually evenly distributed in neuropil.
Conclusions: 1. All three TSEs agents (scrapie, CWD and TME) are capable of
propagating in cattle tissues when administered intracerebrally. 2. All
three TSEs can be distinguished from each other and from BSE when inoculated
intracerebrally by histopathology, immunohistochemistry and Western blot
techniques.



http://arsserv0.tamu.edu/research/publications/publications.htm?seq_no_115=212439



Research Project: Transmission, Differentiation, and Pathobiology of
Transmissible Spongiform Encephalopathies
Location: Virus and Prion Diseases of Livestock

Title: Pathobiology and diagnosis of animal transmissible spongiform
encephalopathies: current knowledge, research gaps, and opportunities


Authors

Kehrli, Marcus
O`rourke, Katherine
Hamir, Amirali
Richt, Juergen
Nicholson, Eric
Silva, Christopher
Edelman, Daniel - FOOD AND DRUG ADMINISTRAT
Gay, Cyril


Submitted to: Government Publication/Report
Publication Type: Government Publication
Publication Acceptance Date: May 1, 2007
Publication Date: July 1, 2007
Citation: Kehrli, Jr., M.E., O'Rourke, K.I., Hamir, A.N., Richt, J.A.,
Nicholson, E.M., Silva, C.J., Edelman, D., Gay, C.G. 2007. Pathobiology and
diagnosis of animal transmissible spongiform encephalopathies: current
knowledge, research gaps, and opportunities [government white paper].
Beltsville, MD: Interagency Working Group on Prion Science, Subcommittee on
Pathobiology and Diagnostics. USDA, Agriculture Research Service. 33 p.

Technical Abstract: Transmissible spongiform encephalopathies (TSEs) are
fatal neurologic diseases that can affect several animal species and human
beings. There are four animal TSE agents found in the United States: scrapie
of sheep and goats; chronic wasting disease (CWD) of deer, elk, and moose;
transmissible mink encephalopathy (TME) and bovine spongiform encephalopathy
(BSE). Although the animal TSEs do not cause major death losses among US
livestock populations, they are important because of international trade
issues. The experience of the United Kingdom and Europe in dealing with the
vast majority of the world's BSE cases, serves as a reminder of the need for
continuing vigilance in monitoring risks for public health and research to
answer remaining questions around the pathogenesis and transmission of these
diseases. There remain questions on 1) cross-species transmissibility of
TSEs in livestock and wildlife; 2) the pathobiology of TSEs in natural and
secondary hosts; pathogenesis and transmission of CWD; and 4) pathogenesis
and ante mortem detection of typical and atypical BSE. Our understanding of
the pathogenesis and transmission of these diseases continues to evolve as
ongoing, global TSE research efforts focus on defining tissue sites of
abnormal prion accumulation, routes of infection, methods of strain
differentiation, genetics of susceptibility and ante-mortem diagnostics. In
this paper, a Subcommittee on Pathobiology and Diagnostics of TSEs for an
Interagency Working Group on Prion Science summarizes the science of animal
TSEs in order to identify knowledge gaps for the purpose of prioritizing
animal prion research needs. Because of substantial losses involving
international trade and potential risk for interspecies transmission to
susceptible livestock and possibly humans, the presence of BSE, CWD, scrapie
and TME in the United States presents a liability to U.S. domestic and
alternative livestock industries. In addition, the proven risk of BSE to
agriculture and public health from subclinical or clinically sick animals
requires science-based surveillance for any silent, unrecognized epizootic
expansions of these diseases in populations of animals that could either
directly or indirectly affect food animals. CWD is an example of an
uncontrolled expanding epidemic that threatens not only cervids but possibly
other livestock. CWD also has elicited public health surveillance programs
to monitor for scientific evidence of a prion disease in humans that consume
venison. Therefore, some of the research needs are precautionary, but the
risks to animal and human health from being caught unaware are high. Efforts
are being made by both federal and state regulatory agencies to eradicate
scrapie and CWD, and to determine the prevalence of BSE. The effectiveness
of these programs will depend heavily on having accurate information about
the nature of these diseases, not only in the original hosts, but also in
other species that may be in contact with infected animals.


http://www.ars.usda.gov/research/publications/publications.htm?seq_no_115=212488


5. Neuropathological investigations suggest that PrPsc may be more widely
distributed, with a different brain distribution pattern for L- and H-type
BSE, compared with classical BSE. However, these investigations are limited
by the very low number of animals for which a complete brain has been
available for analysis. There are no data on the peripheral distribution of
PrPsc or infectivity of L- and H-type BSE or on the pathogenesis of these
diseases. However, studies to assess the tissue distribution of infectivity
and PrPsc in animals throughout the incubation period following
intracerebral challenge are underway.


http://www.seac.gov.uk/minutes/95.pdf




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


TEXAS AND ALABAMA MAD COW CASES

http://lists.ifas.ufl.edu/cgi-bin/wa.exe?A2=ind0703&L=sanet-mg&T=0&P=8125



18 January 2007 - Draft minutes of the SEAC 95 meeting (426 KB) held on 7
December 2006 are now available.


snip...


64. A member noted that at the recent Neuroprion meeting, a study was
presented showing that in transgenic mice BSE passaged in sheep may be more
virulent and infectious to a wider range of species than bovine derived BSE.


***

Other work presented suggested that BSE and bovine amyloidotic spongiform
encephalopathy (BASE) MAY BE RELATED. A mutation had been identified in the
prion protein gene in an AMERICAN BASE CASE THAT WAS SIMILAR IN NATURE TO A
MUTATION FOUND IN CASES OF SPORADIC CJD.

***


snip...


http://www.seac.gov.uk/minutes/95.pdf


3:30 Transmission of the Italian Atypical BSE (BASE) in Humanized Mouse

Models Qingzhong Kong, Ph.D., Assistant Professor, Pathology, Case Western
Reserve
University

Bovine Amyloid Spongiform Encephalopathy (BASE) is an atypical BSE strain
discovered recently in Italy, and similar or different atypical BSE cases
were also reported in other countries. The infectivity and phenotypes of
these atypical BSE strains in humans are unknown. In collaboration with
Pierluigi Gambetti, as well as Maria Caramelli and her co-workers, we have
inoculated transgenic mice expressing human prion protein with brain
homogenates from BASE or BSE infected cattle. Our data shows that about half
of the BASE-inoculated mice became infected with an average incubation time
of about 19 months; in contrast, none of the BSE-inoculated mice appear to
be infected after more than 2 years.


***

These results indicate that BASE is transmissible to humans and suggest
that BASE is more virulent than classical BSE in humans.

***


6:30 Close of Day One


http://www.healthtech.com/2007/tse/day1.asp



***


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


***

If, on the other hand, atypical BSE continues to occur as typical BSE
disappears, this would be a strong indication that it is indeed sporadic,
and if in addition at least 1 form of what is presently considered as
sporadic CJD (such as the type 2 M/V subtype shown to have a Western blot
signature like BASE) were to increase, this would suggest (although not
prove) a causal relationship (Figure 5).


***


http://www.cdc.gov/ncidod/EID/vol12no12/06-0965.htm



In FY 2007, 331 scrapie cases have been confirmed and reported by the
National Veterinary Services Laboratories (NVSL), including 59* Regulatory
Scrapie Slaughter Surveillance (RSSS) cases (Figure 5 and Slide 16). In FY
2007, two field cases, one validation case, and two RSSS cases were
consistent with Nor-98 scrapie. The Nor98-like cases originated from flocks
in California, Minnesota, Colorado, Wyoming and Indiana respectively.
Nineteen cases of scrapie in goats have been reported since 1990 (Figure 6).
The last goat case was reported in September 2007.


snip...



see full report here ;



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



P03.141

Aspects of the Cerebellar Neuropathology in Nor98

Gavier-Widén, D1; Benestad, SL2; Ottander, L1; Westergren, E11National
Veterinary Insitute, Sweden; 2National Veterinary Institute, Norway

Nor98 is a prion disease of old sheep and goats. This atypical form of
scrapie was firstdescribed in Norway in 1998. Several features of Nor98 were
shown to be differentfrom classical scrapie including the distribution of
disease associated prion protein(PrPd) accumulation in the brain. The
cerebellum is generally the most affected brainarea in Nor98. The study here
presented aimed at adding information on theneuropathology in the cerebellum
of Nor98 naturally affected sheep of variousgenotypes in Sweden and Norway.
A panel of histochemical and immunohistochemical(IHC) stainings such as IHC
for PrPd, synaptophysin, glial fibrillary acidic protein,amyloid, and cell
markers for phagocytic cells were conducted. The type of histologicallesions
and tissue reactions were evaluated. The types of PrPd deposition
werecharacterized. The cerebellar cortex was regularly affected, even though
there was avariation in the severity of the lesions from case to case.
Neuropil vacuolation wasmore marked in the molecular layer, but affected
also the granular cell layer. There wasa loss of granule cells. Punctate
deposition of PrPd was characteristic. It wasmorphologically and in
distribution identical with that of synaptophysin, suggestingthat PrPd
accumulates in the synaptic structures. PrPd was also observed in thegranule
cell layer and in the white matter. The pathology features of Nor98 in
thecerebellum of the affected sheep showed similarities with those of
sporadicCreutzfeldt-Jakob disease in humans.


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


It will be critical to see whether the atypical BSE isolates behave
similarly to typical BSE isolates in terms of transmissibility and disease
pathogenesis. If transmission occurs, tissue distribution comparisons will
be made between cattle infected with the atypical BSE isolate and the U.S.
BSE isolate. Differences in tissue distribution could require new
regulations regarding specific risk material (SRM) removal.

http://www.ars.usda.gov/research/projects/projects.htm?accn_no=408490



In sheep experimentally infected with BSE, the distribution of the
infectious agent (prion) in tissues is wide-spread as the prion can be found
in secondary lymphoid tissue, skeletal muscle and blood. In considering more
recent attempts at quantifying the risk specifically from this experimental
ovine BSE, and in reviewing biochemical approaches to quantify titres in
affected animals, a major stumbling block to quantification was identified
to be the fact that the influence of age and genotype on the distribution of
BSE infectivity in sheep is only defined qualitatively. The BIOHAZ panel
agreed that absolute quantification of prions by biochemical methods was
difficult. However, in the absence of comprehensive infectivity data to
facilitate a QRA, it was concluded that Specified Risk Materials (SRM)
removal alone was unlikely to be sufficient to eliminate the residual BSE
risk to the consumer from a BSE-infected sheep carcass.


http://www.efsa.europa.eu/en/science/biohaz/biohaz_opinions/ej442_qra_bse_sheep.html


Published online before print October 20, 2005

Proc. Natl. Acad. Sci. USA, 10.1073/pnas.0502296102
Medical Sciences

A newly identified type of scrapie agent can naturally infect sheep with
resistant PrP genotypes

( sheep prion | transgenic mice )

Annick Le Dur *, Vincent Béringue *, Olivier Andréoletti , Fabienne Reine *,
Thanh Lan Laï *, Thierry Baron , Bjørn Bratberg ¶, Jean-Luc Vilotte ||,
Pierre Sarradin **, Sylvie L. Benestad ¶, and Hubert Laude *
*Virologie Immunologie Moléculaires and ||Génétique Biochimique et
Cytogénétique, Institut National de la Recherche Agronomique, 78350
Jouy-en-Josas, France; Unité Mixte de Recherche, Institut National de la
Recherche Agronomique-Ecole Nationale Vétérinaire de Toulouse, Interactions
Hôte Agent Pathogène, 31066 Toulouse, France; Agence Française de Sécurité
Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels,
69364 Lyon, France; **Pathologie Infectieuse et Immunologie, Institut
National de la Recherche Agronomique, 37380 Nouzilly, France; and
¶Department of Pathology, National Veterinary Institute, 0033 Oslo, Norway

Edited by Stanley B. Prusiner, University of California, San Francisco, CA,
and approved September 12, 2005 (received for review March 21, 2005)

Scrapie in small ruminants belongs to transmissible spongiform
encephalopathies (TSEs), or prion diseases, a family of fatal
neurodegenerative disorders that affect humans and animals and can transmit
within and between species by ingestion or inoculation. Conversion of the
host-encoded prion protein (PrP), normal cellular PrP (PrPc), into a
misfolded form, abnormal PrP (PrPSc), plays a key role in TSE transmission
and pathogenesis. The intensified surveillance of scrapie in the European
Union, together with the improvement of PrPSc detection techniques, has led
to the discovery of a growing number of so-called atypical scrapie cases.
These include clinical Nor98 cases first identified in Norwegian sheep on
the basis of unusual pathological and PrPSc molecular features and "cases"
that produced discordant responses in the rapid tests currently applied to
the large-scale random screening of slaughtered or fallen animals.
Worryingly, a substantial proportion of such cases involved sheep with PrP
genotypes known until now to confer natural resistance to conventional
scrapie. Here we report that both Nor98 and discordant cases, including
three sheep homozygous for the resistant PrPARR allele (A136R154R171),
efficiently transmitted the disease to transgenic mice expressing ovine PrP,
and that they shared unique biological and biochemical features upon
propagation in mice. These observations support the view that a truly
infectious TSE agent, unrecognized until recently, infects sheep and goat
flocks and may have important implications in terms of scrapie control and
public health.

----------------------------------------------------------------------------
----

Author contributions: H.L. designed research; A.L.D., V.B., O.A., F.R.,
T.L.L., J.-L.V., and H.L. performed research; T.B., B.B., P.S., and S.L.B.
contributed new reagents/analytic tools; V.B., O.A., and H.L. analyzed data;
and H.L. wrote the paper.

A.L.D. and V.B. contributed equally to this work.

To whom correspondence should be addressed.

Hubert Laude, E-mail: [email protected]

www.pnas.org/cgi/doi/10.1073/pnas.0502296102

http://www.pnas.org/cgi/content/abstract/0502296102v1


SEAC 99th meeting on Friday 14th December 2007

snip...


SEAC 99th meeting on Friday 14th December 2007

DECEMBER 14, 2007, 10 year Anniversary of my Moms death 'confirmed' from
Heidenhain Variant Creutzfeldt Jakob Disease


Greetings,


AS one of them _lay_ folks, one must only ponder ;


"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 cases in Texas and Alabama,
with both scrapie and 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, 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?"


snip...


----- Original Message -----
From: xxxxxxxxxx
To: [email protected]
Sent: Thursday, November 22, 2007 5:39 AM
Subject: QUESTION FOR SEAC


Mr Terry S Singeltary Sr.,
Bacliff,
Texas 77518
USA.

Dear Mr Singeltary,


"Thank you for your e-mail of yesterday with the question for SEAC. I can
confirm that this will be asked at the meeting on your behalf and the
question and answer will appear in the minutes of the meeting which will be
published on the SEAC Internet site."


snip...end...TSS

http://www.seac.gov.uk
http://www.seac.gov.uk/agenda/agen141207.htm


Archive Number 20071105.3602
Published Date 05-NOV-2007
Subject PRO/AH/EDR> Prion disease update 2007 (07)

PRION DISEASE UPDATE 2007 (07)
******************************
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



SEAC 99th meeting on Friday 14th December 2007

http://seac992007.blogspot.com/



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/


BSE (Mad Cow) Update:


Do Reports of sCJD Clusters Matter?


snip... see full text ;

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/


BSE BASE MAD COW TESTING TEXAS, USA, AND CANADA, A REVIEW OF SORTS


http://madcowtesting.blogspot.com/


MADCOW USDA the untold story

http://madcowusda.blogspot.com/


USA NVCJD BLOOD RECALLS ONLY ;

http://www.google.com/search?hl=en&q=CJD+BLOOD+RECALLS+TSS&btnG=Search


vCJD case study highlights blood transfusion risk

http://vcjdblood.blogspot.com/



Transmissible Mink Encephalopathy TME

http://transmissible-mink-encephalopathy.blogspot.com/


CHRONIC WASTING DISEASE

http://chronic-wasting-disease.blogspot.com/


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