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From: TSS ()
Subject: Atypical Bovine Spongiform Encephalopathies, France, 2001-2007
Date: January 29, 2008 at 2:22 pm PST

Tuesday, January 29, 2008
Atypical Bovine Spongiform Encephalopathies, France, 2001-2007

Volume 14, Number 2–February 2008


Atypical Bovine Spongiform Encephalopathies, France, 2001–2007

Anne-Gaëlle Biacabe,* Eric Morignat,* Johann Vulin,* Didier Calavas,* and
Thierry G.M. Baron*
*Agence Française de Sécurité Sanitaire des Aliments, Lyon, France

Suggested citation for this article


In France, through exhaustive active surveillance, ˜17.1 million adult
cattle were tested for bovine spongiform encephalopathy from July 2001
through July 2007; ˜3.6 million were >8 years of age. Our retrospective
Western blot study of all 645 confirmed cases found that 7 were H-type and 6
were L-type.

Most cases of bovine spongiform encephalopathy (BSE) have shown strikingly
uniform features. The origin of epidemics, mainly in the United Kingdom but
to a lesser extent in other countries, has been foodborne contamination by a
single major strain of the transmissible spongiform encephalopathy (TSE)
agent (1,2). However, in recent years, 2 distinct forms of the disease have
been described; these forms deviate phenotypically from the previously
identified classic BSE (C-type) (3,4). Western blot studies of the
protease-resistant prion protein (PrPres) showed higher and lower molecular
masses of unglycosylated PrPres in these 2 types, subsequently named H-type
and L-type compared with C-type BSE (5). In L-type BSE, the most
discriminant molecular feature was the lower level of diglycosylated PrPres
(4,5). Such cases have now been identified in a number of different
countries (6–8).

The origin of H-type and L-type BSE cases is unknown, but they may represent
spontaneous or so-called sporadic forms of TSE, reminiscent of most cases of
Creutzfeldt-Jakob disease in humans. Transmission studies in wild-type mice
(9) or in transgenic mice expressing the bovine PrP gene (5,10–12) have
shown that the infectious agents involved in H-type and L-type BSEs differ
from the single strain isolated from C-type BSE.

Epidemiologic data are crucial to our understanding of the origin of such
cases, but precise prevalence needs to be determined. The prevalence in
cattle can now be assessed with accuracy because exhaustive BSE testing of
adult cattle was implemented in 2001 in all European member states (January
1 for abattoirs and July 1 for rendering plants). We report the results of a
retrospective study to determine the frequency of H-type and L-type BSE
identified in France since July 2001.

The Study

A retrospective Western blot study was performed for all confirmed BSE cases
diagnosed in France after July 1, 2001, as previously described (13).
Briefly, PrPres was extracted using the TeSeE Western blot confirmatory
assay (Bio-Rad, Marnes-la-Coquette, France) according to the manufacturer's
instructions. For immunoblotting, antibodies RB1, 6H4 (R-Biopharm, St.
Didier au Mont d'Or, France), Sha31 from the TeSeE Western blot (Bio-Rad),
and SAF84 (kindly provided by J. Grassi, Commissariat Energie Atomique,
Saclay, France) were used; these antibodies recognize the bovine PrP
sequences 110–113, 156–164, 156–163, and 175–180, respectively. Two Western
blot assays were conducted on the brainstem samples and used either an
antibody against the PrP core (RB1, 6H4, or Sha31) or a C-terminal antibody
(SAF84) (13). The characteristics of H-type and L-type BSE (6,13) that were
sought included 1) a higher or lower molecular mass of unglycosylated PrPres
with core antibodies in H-type and L-type BSE compared with C-type BSE, 2) a
lower proportion of diglycosylated PrPres in L-type BSE, and 3) presence of
an additional band at ˜14 kDa with SAF84 in H-type BSE. All cases with
features of H-type or L-type BSE were then subjected to further Western blot
analyses for detailed quantitative analysis of PrPres molecular features
(apparent molecular masses and glycoforms proportions).

Among the 645 BSE cases confirmed between July 1, 2001, and July 1, 2007, 7
H-type and 6 L-type isolates were identified; these had occurred at a
frequency of 0–3 H-type and 1 L-type case per year, compared with 6–219
cases of C-type BSE per year (Table). Molecular typing of 48 of these 645
samples was not possible because low levels of PrPres prevented detailed
molecular characterization or because sample amount was insufficient. All 13
atypical cases were detected in cattle >8 years of age, from fallen stock (9
cases) or abattoir (4 cases); not 1 atypical case was found among the 98 BSE
cases detected by clinical surveillance during this period. These 13
atypical cases were diagnosed by the different rapid BSE tests routinely
used in France: 9 by Prionics-Check Western, LIA, or Priostrip (AES,
Combourg, France), 3 by ELISA Bio-Rad, and 1 by IDEXX HerdChek (IDEXX
Laboratories, Schiphol-Rijk, the Netherlands). During retrospective
interviews, the farmer and veterinarian for 6 of these animals reported
clinical signs consistent with TSE in 3 fallen stock. This series of 13
cases identified since the beginning of exhaustive active surveillance
should be compared with a total of ˜17.1 million adult cattle tested, of
which ˜3.6 million were >8 years of age. In addition to these 13 cases, the
first case of atypical (H-type) BSE was identified in 2000, during an
exhaustive active surveillance program in rendering plants in a limited
region of France.

The distribution of BSE-infected cattle by birth date (Figure 1) shows that
1 or 2 (H- or L-type cases) were eventually found positive by rapid tests of
the brainstem in each annual birth cohort from 1986 through 1997, which
compares with up to 221 cattle infected with C-type BSE born during
1990–2001 for which BSE was diagnosed during 2001–2007. All H-type and
L-type BSE cases showed similar features (Figure 2) regarding the high
apparent molecular masses of unglycosylated PrPres (mean difference of ˜1
kDa using 6H4 antibody) for H-type BSE and lower levels of diglycosylated
PrPres (mean difference of 35% using 6H4 antibody) for L-type BSE, compared
with C-type BSE. For L-type BSE cases, the differences in apparent molecular
masses were more obvious for the diglycosylated band (˜1-kDa difference
using 6H4, compared with 0.3 kDa for the unglycosylated band), as previously
observed (6). The ˜14-kDa band characteristic of H-type BSE was similarly
detected in the 8 isolates with SAF84 antibody but in none of the other
cases classified as L-type or C-type BSE.


This study involved exhaustive molecular typing of BSE cases during a given
test period and in a country in which BSE testing has been mandatory for all
adult slaughtered or fallen cattle. France tests more animals than any other
European country; ˜30% of the animals tested in the European Union are
tested in France. The estimated frequency of H-type and L-type BSE was 0.41
and 0.35 per million adult cattle tested, respectively (1.9 and 1.7 in
cattle >8 years of age). Given the implementation dates of measures to
control BSE and the birth dates of these BSE-infected cattle, foodborne
exposure to an infectious agent cannot be fully excluded for any of these
cattle. However, the distribution of cattle affected by H- and L-type BSE,
by year of birth, differs strikingly from that of cattle affected by C-type
BSE and is consistent with the hypothesis that H-type and L-type BSE might
represent sporadic prion disorders. In comparison with another sporadic
prion disease, the annual frequency of sporadic Creutzfeldt-Jakob disease in
humans, which is estimated only by analyses of reported clinically suspect
cases, is 1–2 cases per million. Similar studies in other countries, instead
of those free of C-type BSE, would be useful. An alternative hypothesis to
foodborne contamination, such as contamination by a scrapie agent, cannot be
fully excluded, as such contamination has been shown to be a risk factor for
scrapie in sheep (14).

This study relied on the identification of BSE cases by examination of the
brainstem only, as derived from our knowledge of C-type BSE (15). We cannot
exclude possible differences in the pathogenesis of atypical BSEs that might
result in underestimation of their frequency, e.g., involvement of the
brainstem at a later stage than with C-type BSE. This possibility is at
least suggested by data available for L-type BSE, which shows a preferential
distribution of abnormal PrP in more rostral brain regions (4,12). Studies
of the pathogenesis of these novel BSE forms are thus important for
understanding of prion disorders of domestic ruminant species.



i guess the infamous 'sporadic' and or 'spontaneous' myth for all other
strains of mad cow disease, whether
it is the h-base and or l-base, or any sub-type thereof, is an accepted
international myth now. only the UK BSE can be transmitted orally to cattle
or humans, and all the rest is spontaneous? no recycled feed from atypical
TSE can transmit a TSE, and this has been proven?

and they call this science$ i call it BSe. Junk and or political science at
its best. ...

once again ;

sporadic CJD is NOT a single strain, phenotype.

sporadic CJD is simply a human TSE, which routes, sources, strains, are

there are 6 different documented phenotypes of the sporadic CJD, with
'UNKNOWN' sub-types growing.

what would 2nd, 3rd, 4th passage iatrogenic i.e. friendly fire look like
from BSE compared to the h-type, compared to l-type ???

spontaneous TSE in the field ??? never has been proven, and highly unlikely
that 85%+ of all human TSE i.e. sporadic CJD, just happen without route and

Volume 12, Number 12–December 2006


On the Question of Sporadic or Atypical Bovine Spongiform Encephalopathy and
Creutzfeldt-Jakob Disease

Paul Brown,* Lisa M. McShane,† Gianluigi Zanusso,‡ and Linda Detwiler§

*Bethesda, Maryland, USA; †National Institutes of Health, Bethesda,
Maryland, USA; ‡University of Verona, Verona, Italy; and §Virginia-Maryland
Regional College of Veterinary Medicine, College Park, Maryland, USA


Sporadic CJD

The possibility that at least some cases of apparently sporadic CJD might be
due to infection by sporadic cases of BSE cannot be dismissed outright.
Screening programs needed to identify sporadic BSE have yet to be
implemented, and we know from already extant testing programs that at least
a proportion of infected animals have no symptoms and thus would never be
identified in the absence of systematic testing. Thus, sporadic BSE (or for
that matter, sporadic disease in any mammalian species) might be occurring
on a regular basis at perhaps the same annual frequency as sporadic CJD in
humans, that is, in the range of 1 case per million animals.

Whether humans might be more susceptible to atypical forms of BSE cannot be
answered at this time. Experimentally transmitted BASE shows shorter
incubation periods than BSE in at least 1 breed of cattle, bovinized
transgenic mice, and Cynomolgus monkeys (12,13). In humanized transgenic
mice, BASE transmitted, whereas typical BSE did not transmit (13).
Paradoxically, the other major phenotype (H) showed an unusually long
incubation period in bovinized transgenic mice (12).

The limited experimental evidence bearing on a possible relationship between
BSE and sporadic CJD is difficult to interpret. The original atypical BASE
strain of BSE had a molecular protein signature very similar to that of 1
subtype (type 2 M/V) of sporadic CJD in humans (5). In another study, a
strain of typical BSE injected into humanized mice encoding valine at codon
129 showed a glycopattern indistinguishable from the same subtype of
sporadic CJD (15). In a third study, the glycopatterns of both the H and L
strains of atypical BSE evidently did not resemble any of the known sporadic
CJD subtypes (12).

To these molecular biology observations can be added the epidemiologic data
accumulated during the past 30 years. The hypothesis that at least some
cases of apparently sporadic CJD are due to unrecognized BSE infections
cannot be formally refuted, but if correct, we might expect by now to have
some epidemiologic evidence linking BSE to at least 1 cluster of apparently
sporadic cases of CJD. Although only a few clusters have been found (and
still fewer published), every proposed cluster that has been investigated
has failed to show any common exposure to bovines. For that matter, no
common exposure has been shown to any environmental vehicles of infection,
including the consumption of foodstuffs from bovine, ovine, and porcine
sources, the 3 livestock species known to be susceptible to transmissible
spongiform encephalopathies. Additional negative evidence comes from several
large case-control studies in which no statistically significant dietary
differences were observed between patients with sporadic CJD and controls

On the other hand, the difficulty of establishing a link between BSE and CJD
may be compounded by our ignorance of the infectious parameters of a
sporadic form of BSE (e.g., host range, tissue distribution of infectivity,
route of transmission, minimum infectious dose for humans, whether single or
multiple). Presumably, these parameters would resemble those of variant CJD;
that is, high infectivity central nervous system and lymphoreticular tissues
of an infected cow find their way into products consumed by humans.
Transmissions that might have occurred in the past would be difficult to
detect because meat products are generally not distributed in a way that
results in detectable geographic clusters.

Barring the discovery of a specific molecular signature (as in variant CJD),
the most convincing clue to an association will come from the observation of
trends over time of the incidence of typical and atypical BSE and of
sporadic and variant CJD. With 4 diseases, each of which could have
increasing, unchanging, or decreasing trends, there could be 81 (34)
possible different combinations. However, it is highly likely that the
trends for typical BSE and variant CJD will both decrease in parallel as
feed bans continue to interrupt recycled contamination. The remaining
combinations are thus reduced to 9 (32), and some of them could be highly

For example, if the incidence of atypical BSE declines in parallel with that
of typical BSE, its candidacy as a sporadic form of disease would be
eliminated (because sporadic disease would not be influenced by current
measures to prevent oral infection). 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).

Recognition of the different forms of BSE and CJD depends upon continuing
systematic testing for both bovines and humans, but bovine testing will be
vulnerable to heavy pressure from industry to dismantle the program as the
commercial impact of declining BSE cases ceases to be an issue. Industry
should be aware, however, of the implications of sporadic BSE. Its
occurrence would necessitate the indefinite retention of all of the public
health measures that exclude high-risk bovine tissues from the animal and
human food chains, whereas its nonoccurrence would permit tissues that are
now destroyed to be used as before, once orally acquired BSE has

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



[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],F2400_P1001_PUB_MAIL_ID:1010,39963

There is a growing number of human CJD cases, and they were presented last
week in San Francisco by Luigi Gambatti(?) from his CJD surveillance

He estimates that it may be up to 14 or 15 persons which display selectively
SPRPSC and practically no detected RPRPSC proteins.


MARCH 26, 2003

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

disease in the United States

Email Terry S. Singeltary:

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

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

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

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

phenotype that is indistinguishable from type 2 PrPSc, the commonest

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

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

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

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

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

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

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

intracerebral inoculation. With the known incubation periods in other

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

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

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

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

should be drastic measures to safeguard the medical and surgical arena

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

CJDs in the USA are type 2 PrPSc?

Hardcover, 304 pages plus photos and illustrations.
ISBN 0-387-95508-9 June 2003
BY Philip Yam


Answering critics like Terry Singeltary, who feels that the U.S.
under-counts CJD, Schonberger conceded that the current surveillance system
has errors but stated that most of the errors will be confined to the older

doi:10.1016/S1473-3099(03)00715-1Copyright ? 2003 Published by Elsevier Ltd.


Tracking spongiform encephalopathies in North America

Xavier Bosch

Available online 29 July 2003.
Volume 3, Issue 8, August 2003, Page 463

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


Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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

please notice Texas 2006 ;

† Confirmed in United Kingdom and reported to Texas Department of State
Health Services
through Centers for Disease Control and Prevention.

Only 1 case of variant
CJD has ever been diagnosed in Texas.
The patient was a former resident of the
United Kingdom, where the exposure
was likely to have occurred. Texas has a
population of 23 million, and since the
national rate of sporadic CJD is about 1
per million, it is expected that
approximately 23 cases of CJD would
occur each year in the state. Therefore,
it is believed that CJD is currently underreported
in Texas. ...END...TSS

also see ;

Creutzfeldt-Jakob Disease in Northeast Texas,

J.A. Rawlings,*1 K.A.
Hendricks1, O.M. Nuno1, D.A. Brown1, D.A. Evans2, Texas Department of
Health, 1Austin and 2Tyler, Texas

Creutzfeldt-Jacob Disease (CJD), a transmissible spongiform
encephalopathy, is caused by prions composed of proteinaceous material
devoid of nucleic acid. CJD occurs sporadically (generally 1
case/1,000,000 population per year) in older patients (average age of
65) and is characterized by rapidly progressive dementia, accompanied by
severe muscle spasms and incoordination. Death usually occurs within 3
to 12 months (average 7 months). CJD activity in Texas, which has a
population of nearly 19 million, appeared to be typical. The statewide
death rate for 1995 and 1996 was just under 1/1,000,000. In April of
1997, the Texas Department of Health became aware of an increased number
of possible CJD cases in a 23-county area of NE Texas with a population
of just over one million. After review of medical and pathology records,
four patients were identified with definite classic CJD and three were
identified with probable CJD. Dates of death for the eight patients were
from April, 1996 through mid-July 1997. The patients were from 46
through 65 years of age; four were male and three were female. A
case-control study to identify risks for CJD in NE Texas has been initiated.

Division of Neuropathology
Pierluigi Gambetti, M.D.,



March 30, 1998

Dr. Gerald A, Campbell

The University of Texas
Medical Branch at Galveston
Division of Neuropathology
Department of Pathology
Galveston, Texas

Dear Dr Campbell,

We performed Western immunoblot analysis on the frozen tissue from your case
#AU97-435 (our #098-28). The Immunoblot reveals the presence of
protease-resistant prion protein (PrPres) confirming the diagnosis of prion
disease. The immunoblot pattern of PrPres is consistent with the diagnosis
of Creutzfeldt-Jakob disease.

Thank you for referring to us this interesting case.


Piero Parchi, M.D.

Pierluigi Gambetti, M.D.

PP:sbDivision of Neuropathology

Pierluigi Gambetti, M.D.,


Case Western Reserve University


-------- Original Message --------

Subject: re-BSE prions propagate as either variant CJD-like or sporadic CJD

Date: Thu, 28 Nov 2002 10:23:43-0000

From: "Asante, Emmanuel A"

To: "'"

Dear Terry,

I have been asked by Professor Collinge to respond to your request. I am a
Senior Scientist in the MRC Prion Unit and the lead author on the paper. I
have attached a pdf copy of the paper for your attention. Thank you for your
interest in the paper. In respect of your first question, the simple answer
is, yes. As you will find in the paper, we have managed to associate the
alternate phenotype to type 2 PrPSc, the commonest sporadic CJD. It is too
early to be able to claim any further sub-classification in respect of
Heidenhain variant CJD or Vicky Rimmer's version. It will take further
studies, which are on-going, to establish if there are sub-types to our
initial finding which we are now reporting. The main point of the paper is
that, as well as leading to the expected new variant CJD phenotype, BSE
transmission to the 129-methionine genotype can lead to an alternate
phenotype which is indistinguishable from type 2 PrPSc. I hope reading the
paper will enlighten you more on the subject. If I can be of any further
assistance please to not hesitate to ask.

Best wishes.

Emmanuel Asante



Dr. Emmanuel A Asante MRC Prion Unit & Neurogenetics Dept. Imperial College
School of Medicine (St. Mary's) Norfolk Place, LONDON W2 1PG Tel: +44 (0)20
7594 3794 Fax: +44 (0)20 7706 3272 email: (until
9/12/02)New e-mail:

(active from now)


CJD, PrP Codon 129VV, Novel PrPSc in a Young British Woman

Wed Jan 2, 2008 16:5071.248.140.49

Creutzfeldt-Jakob Disease, Prion Protein Gene Codon 129VV, and a Novel PrPSc
Type in a Young British Woman

Simon Mead, PhD, MRCP; Susan Joiner, MSc; Melanie Desbruslais, BSc;
JonathanA. Beck, BSc; Michael O’Donoghue, PhD; Peter Lantos, FRCP; Jonathan
D. F.Wadsworth, PhD; John Collinge, FRSArch Neurol. 2007;64(12):1780-1784.


Variant Creutzfeldt-Jakob disease (vCJD) is an acquired prion disease
causally related to bovine spongiform encephalopathy that has occurred
predominantly in young adults. All clinical cases studied have been
methionine homozygotes at codon 129 of the prion protein gene (PRNP) with
distinctive neuropathological findings and molecular strain type (PrPSc
type4). Modeling studies in transgenic mice suggest that other PRNP
genotypes will also be susceptible to infection with bovine spongiform
encephalopathy prions but may develop distinctive phenotypes.


To describe the histopathologic and molecular investigation in ayoung
British woman with atypical sporadic CJD and valine homozygosity at PRNP
codon 129.

Design Case report, autopsy, and molecular analysis.

Setting Specialist neurology referral center, together with the laboratory
services of the MRC [Medical Research Council] Prion Unit.

Subject Single hospitalized patient.

Main Outcome Measures Autopsy findings and molecular investigation results.

Results Autopsy findings were atypical of sporadic CJD, with marked gray and
white matter degeneration and widespread prion protein (PrP) deposition.
Lymphoreticular tissue was not available for analysis. Molecular analysis of
PrPSc (the scrapie isoform of PrP) from cerebellar tissue demonstrated a
novel PrPSc type similar to that seen in vCJD (PrPSc type 4). However, this
could be distinguished from the typical vCJD pattern by an altered protease
cleavage site in the presence of the metal ion chelator EDTA.

Conclusions Further studies will be required to characterize the prion
strain seen in this patient and to investigate its etiologic relationship
with bovine spongiform encephalopathy. This case illustrates the importance
of molecular analysis of prion disease, including the use of EDTA to
investigate the metal dependence of protease cleavage patterns of PrPSc.

Author Affiliations: MRC [Medical Research Council] Prion Unit and
Department of Neurodegenerative Disease, Institute of Neurology, University
College London, National Hospital for Neurology and Neurosurgery, London,
England (Drs Mead, Wadsworth, and Collinge; Mss Joiner and Desbruslais; and
Mr Beck); and Institute of Psychiatry, King's College London (Dr Lantos).
DrO’Donoghue is now with the Department of Clinical Neurology, Nottingham
University Hospitals NHS [National Health Service] Trust, Nottingham,

North American Equity Research

New York

13 January 2004

BSE (Mad Cow) Update:

Do Reports of sCJD Clusters Matter?

· There have been seven cases of human sCJD clusters identified in the
US in the last 15 years, in which people in a specific location were
diagnosed with sCJD, resulting in rates between 2.1 and 8.4 deaths per
million people for that specific location compared with the national
average of one in 1 million. · There is no proven link between sCJD and
BSE, and hence it is considered a different disease from vCJD (which has
been linked to BSE). However, the existence of clusters raises the
question of “contamination" or “infection”, and also raises the
hypothesis that rather than cases of sCJD these might have been cases of
vCJD. · Clusters are not spontaneous, they normally have a source.
Moreover, some cases of sCJD may have been improperly diagnosed as
Alzheimer's.· We continue to believe that as long as no further cases of
BSE-positive cows are found in North America and the industry has
respected the 1997 ban on animal feed for live cattle, beef consumption
in the US will not suffer. · Moreover, due to political pressure we
expect key overseas markets (Japan, South Korea, and Mexico) to open up
to US beef in the next six months – the recent 20% drop in cattle prices
can be attributed mainly to these import bans. · However, two concerns
linger and should be kept in mind by investors, 1) Has the 1997 ban on
animal feed for live cattle been honored by the beef industry? 2) Can
clusters of cases of sporadic CJD (or sCJD) really be a variant of CJD
and indeed be linked to BSE? In this note we focus on the issue of sCJD
clusters, and the potential impact that the growing debate on clusters
could have on beef consumption in the US. United States Foods
Pablo E. Zuanic(1-212) 622-6744pablo.zuanic@jpmorgan.comChristopher M.
Bledsoe(1-212) 622-6386christopher.m.bledsoe@jpmorgan.comDaniel

State of Our Views Regarding BSE in the US


Existence of Clusters of sCJD May Imply They Are Really Cases of vCJD
There have been seven sCJD clusters identified in the US in the last 15
years, in which people in a specific location were diagnosed with sCJD,
resulting in rates between 1.2 and 8.4 deaths per million people for
that specific location compared with the national average of one in 1
million. The existence of clusters raises the question of
“contamination" or “infection”, and also raises the hypothesis that
rather than cases of sCJD these might have been cases of vCJD. Clusters
are not spontaneous, they normally have a source.

A cluster consists of two statistical improbabilities: 1) multiple cases
occurring in a relatively limited geographic area, and 2) multiple cases
occurring within the same time period. The most recent cluster was found
in Cherry Hill, New Jersey. The others have been found in Lehigh,
Pennsylvania (1986-90), Allentown, Pennsylvania (1989-92), Tampa,
Florida (1996-97), Oregon (2001-02), and Nassau County, New York
(1999-2000). Given that sCJD occurs randomly in one out of one million
cases, it is a statistical rarity to find an sCJD cluster – let alone
six. The following tables highlight known clusters in the US.

Table 1:

Clustered sCJD Deaths

Local sCJD Deaths

Time Span State Local Area Pop. (MM) Period (mo.) Total Ann'lized

1986-1990 PA Lehigh Valley 0.5 48 18 4.5

1989-1992 PA Allentown 2.5 36 15 5.0

1996-1997 FL Tampa 2.2 18 13 8.7

1996-1999 TX Denton .01 38 4 1.3

1999-2000 NY Nassau County 1.3 12 7 7.0

2001-2002 OR Entire State 3.4 24 14 7.0

2000-2003* NJ Cherry Hill Area 1.7 36 12 4.0

Source: JPMorgan.

The second table, below, shows what portion of the state's total
expected sCJD cases (as based on a one per million occurrence) were
found in the local cluster, comparing the local cluster's portion of
cases with the local area's portion of the state's total population. The
greater the factor between the former and the latter suggests a higher
statistical improbability that the cluster is spontaneous (sCJD).

Table 2: Clustered sCJD Deaths vs. Expected State Cases

Annual Statewide Local Area (% of Time Span State Local Area sCJD Deaths*
exp. state cases state pop.

1986-1990 PA Lehigh Valley 11.9 37.8% 4.5%

1989-1992 PA Allentown 12.0 41.7% 20.8%

1996-1997 FL Tampa 14.1 61.5% 15.7%

1996-1999 TX Denton 20.9 6.1% .02%

1999-2000 NY Nassau County 18.1 38.7% 7.4%

2001-2002 OR Entire State 3.4 205.9% 100.0%

2000-2003* NJ Cherry Hill Area 8.0 50.0% 21.6%

* *State cases are extrapolated based on state population and the 1 per
million national average. Source: JPMorgan.


Copyright 2003 J.P. Morgan Chase & Co.—All rights reserved.


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see full text ;

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in
the United States

Creutzfeldt Jakob Disease


SEAC 99th meeting on Friday 14th December 2007

please see full text ;

16 January 2008 - The final minutes of the 98th SEAC meeting have been


© SEAC 2007

SEAC considered a question about possible links between CJD cases and animal
TSEs in the United States of America (USA).

full text ;

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA 77518

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