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From: TSS ()
Subject: Re: Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States [FULL TEXT]
Date: March 16, 2005 at 1:17 pm PST

In Reply to: Re: Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States [FULL TEXT] posted by TSS on March 13, 2005 at 6:58 pm:

-------- Original Message --------
Subject: Re: The public health impact of prion diseases (1)
Date: Mon, 14 Mar 2005 16:57:46 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@KALIV.UNI-KARLSRUHE.DE
References: <4234FD63.5040302@wt.net>


##################### Bovine Spongiform Encephalopathy #####################

Greetings List Members,

can someone please explain this phenomenal statement to me ;

> However, in the
> period following the first published description of vCJD in 1996,
> there was no
> increasing trend in the reported annual number of U.K. sporadic CJD
> deaths (52).


the UK went from 40 cases in 1996 to 77 cases in 2003, almost double.
this is an increase.

IN this full text article (some 25 pages) it states ;

Multiple BSE Strains
Unlike scrapie or classic CJD, BSE is likely caused by a single strain
of an infectious
agent that has a strikingly stable molecular property after natural or
experimental
transmission to other species. In 2004, however, researchers in Italy
reported that the brain lesions of two of eight cattle they studied were
distinguished
from BSE by the presence of amyloid plaques and distribution of
protease-resistant
prion protein in the brain (16). OnWestern blot analysis, the prion
fragment in the
two cases had a lower molecular mass than that of the classic form of
BSE. The
researchers suggested that the difference in neuropathology and
molecular mass
indicates the existence of a second strain of BSE. To distinguish the
disease in the
two cows from the classic form of BSE, the researchers coined the name
bovine
amyloidotic spongiform encephalopathy (BASE). Three BSE cases with distinct
molecular phenotype among cattle routinely diagnosed in a BSE
surveillance system
were independently reported in France also (9). Whether the findings
from Italy
and France do or do not represent new strains of widely circulating BSE
should be
confirmed through identification of more cases and additional laboratory
studies.
No scientific evidence exists to causally link any form of BSE with a
sporadic
CJD-like illness in humans. Concerns about BSE causing a sporadic CJD-like
illness have persisted after BSE-infected transgenic mice expressed
prions with a
molecular phenotype consistent with a subtype of sporadic CJD (2). The
transgenic
mice were designed to produce the human prion protein, which is
homozygous for
methionine at codon 129. If BSE causes a sporadic CJD-like illness in
humans, an
increase in sporadic CJD cases would be expected to occur in the United
Kingdom
first, where the vast majority of vCJD cases have been reported.
However, in the
period following the first published description of vCJD in 1996, there
was no
increasing trend in the reported annual number of U.K. sporadic CJD
deaths (52).
Furthermore, surveillance in the United Kingdom has shown no increase in the
proportion of sporadic CJD cases that are homozygous for methionine.

> No scientific evidence exists to causally link any form of BSE with a
> sporadic
> CJD-like illness in humans. Concerns about BSE causing a sporadic CJD-like
> illness have persisted after BSE-infected transgenic mice expressed
> prions with a
> molecular phenotype consistent with a subtype of sporadic CJD (2). The
> transgenic
> mice were designed to produce the human prion protein, which is
> homozygous for
> methionine at codon 129. If BSE causes a sporadic CJD-like illness in
> humans, an
> increase in sporadic CJD cases would be expected to occur in the
> United Kingdom
> first, where the vast majority of vCJD cases have been reported.
> However, in the
> period following the first published description of vCJD in 1996,
> there was no
> increasing trend in the reported annual number of U.K. sporadic CJD
> deaths (52).
> Furthermore, surveillance in the United Kingdom has shown no increase
> in the
> proportion of sporadic CJD cases that are homozygous for methionine.


IF we look at sporadic incidence of CJD in UK from 1993 to 2003,
the incidence rose from 37 in 1993 to 77 in 2003. THIS seems to
show an increase to me? I do not understand the statement ;

> However, in the
> period following the first published description of vCJD in 1996,
> there was no
> increasing trend in the reported annual number of U.K. sporadic CJD
> deaths (52).

IF we go further and look at some of the other documented BSE countries,
you will the increase of sporadic CJD there as well ;

Canada from 2 to 25

France from 35 to 108

Germany 21+ to 96

Italy 27 to 76

http://www.eurocjd.ed.ac.uk/sporadic.htm

and Switzerland sporadic CJD ;

Swiss rise in CJD raises concerns over possible BSE link
[LONDON]
THE LANCET

Plaque attack: Swiss patients have spongiform patterns in the brain
typical of sporadic CJD.
The number of people dying from Creutzfeldt-Jakob disease (CJD) has
risen sharply in Switzerland -- sparking fears of a possible link with
bovine spongiform encephalopathy (BSE).

BSE is thought to be the cause of a distinctive form of the
brain-wasting disease known as variant CJD. The Swiss cases, in
contrast, are standard 'sporadic' CJD. Each year between 1997 and 2000,
no more than 11 Swiss people developed CJD. But 19 cases were reported
in 2001, and seven were recorded in the first quarter of this year. This
is some four times higher than the incidence elsewhere, reports a team
led by Adriano Aguzzi of the University Hospital Zurich (M. Glatzel et
al. Lancet 360, 139-141; 2002).

The increase could be a mere statistical blip, or it may be due to
increased awareness of the disease leading to more diagnoses. More
disturbing is the possibility that the cases are linked to the
consumption of BSE-infected meat products -- which would mean that the
BSE agent can cause two distinct forms of CJD.

Possible links between the Swiss CJD cases and BSE will now be explored
by strain-typing experiments in which the disease is transmitted to
mice. These tests will take at least a year to complete. "It's the best
way to establish or exclude any suspected link," says Moira Bruce of the
UK Institute for Animal Health's Neuropathogenesis Unit in Edinburgh.

======================================

Experiences in England and Switzerland -- two countries that discovered
mad cow disease in their cattle -- have heightened concerns about the
possibility some cases of sporadic CJD are due to consuming
mad-cow-tainted beef. Both countries have reported increases in sporadic
CJD since mad cow was first detected in British herds in 1986.

Switzerland discovered last year its CJD rate was twice that of any
other country in the world. Switzerland had been seeing about eight to
11 cases per year from 1997 to 2000. Then the incidence more than
doubled, to 19 cases in 2001 and 18 cases in 2002.

http://www.upi.com/view.cfm?StoryID=20030721-102924-4786r

Mouse model sheds new light on human prion disease


snip...


Professor John Collinge said We are not saying that all or even most

cases of sporadic CJD are as a result of BSE exposure, but some more

recent cases may be  the incidence of sporadic CJD has shown an upward

trend in the UK over the last decade. While most of this apparent

increase may be because doctors are now more aware of CJD and better at

diagnosing it, serious consideration should be given to a proportion of

this rise being BSE-related. Switzerland, which has had a substantial

BSE epidemic, has noted a sharp recent increase in sporadic CJD.

snip...

http://www.mrc.ac.uk/txt/index/public-interest/public-news-4/public-news_archive/public-news-archive_nov_dec_02/public-bse_and_sporadic_cjd.htm

snip...END...TSS

I still strongly disagree with the statement. THE figures do NOT support
this statement;

> However, in the
> period following the first published description of vCJD in 1996,
> there was no
> increasing trend in the reported annual number of U.K. sporadic CJD
> deaths (52).

BACK TO ARTICLE;

CHRONICWASTING DISEASE
Distribution
CWD (chronic wasting disease), a prion disease of North American deer
and elk,
was first identified as a fatal wasting syndrome of captive mule deer in
the late
1960s in research facilities in Colorado (76). It was first recognized
as a TSE in
1978. The occurrence of CWD among wild cervids was first identified in 1981
when a free-ranging elk from Colorado was diagnosed with the disease.
Subsequent
surveillance studies demonstrated the endemic occurrence of CWD among
free-ranging deer and elk in a contiguous area in northeastern Colorado,
southeastern
Wyoming, and, most recently, in western Nebraska (76). Epidemic modeling
suggested thatCWDmight have been present among free-ranging animals in some
portions of the endemic area several decades before itwas initially
recognized (55).
PRION DISEASES 207
After 2000, new foci of CWD have increasingly been identified in
Illinois, New
Mexico, South Dakota, Utah, Wisconsin, and non-CWD-endemic areas of Colorado
and Wyoming (6, 76). The identification of CWD in these new areas seems
to be related to increased surveillance and spread of the disease as a
result of
natural migration of deer and elk or translocation of infected cervids
by humans.
Currently, two largely independent outbreaks, one in free-ranging deer
and elk
and another in the captive elk and deer industry, are occurring in
Canada and the
United States (6).
Risk to Humans
The increasing spread of CWD in the United States and the zoonotic
transmission
of BSE raised concerns about the possible transmission of CWD to humans
(6). Several CJD cases or apparent CJD clusters with suspect CWD
transmission
have been reported in the United States (4, 6, 23). Epidemiologic and
laboratory
investigations of these isolated cases and clusters did not provide
convincing evidence
for a link between CWD and the patients illnesses. However, the studies
seeking evidence for a possible link between CWD and human illness have been
limited. Additional epidemiologic and laboratory studies should be
conducted before
the CWD agent can be exonerated as a possible human pathogen. Because
persons who hunted deer and elk in the known CWD-endemic areas of Colorado
andWyoming are more likely to have been exposed to the CWD agent over many
years, a follow-up study of these hunters has been initiated to monitor
the possible
zoonotic transmission of CWD. A transgenic mice study, involving humanized
and cervidized mice, is also in progress to determine the susceptibility
of these
mice to the CWD agent (6).
CONCLUSION
Three distinct prion diseaserelated human health risks from
environmental sources
of infection can be identified in the United States. These include the
iatrogenic
transmission of CJD, occurrence of vCJD from exposure to BSE-contaminated
cattle products in the United States or other countries with BSE, and
possible
transmission of CWD to humans. The iatrogenic transmission of CJD appears to
be on the decline following appropriate preventive measures that were
instituted
as the different iatrogenic modes of spread were identified. Additional
iatrogenic
CJD cases, however, can be anticipated primarily because of the long
incubation
period associated with prion diseases.
To date, only one vCJD patient has been identified as a resident of the
United
States (25). This patient is believed to have contracted the disease
while growing
up in her native country of Britain during the height of human exposure to
the BSE outbreak. Although the public health preventive measures
recently instituted
by the USDA should further reduce the risk of BSE exposure to the U.S.
population, the possibility that domestically acquired vCJD may appear
in the
Annu. Rev. Public. Health. 2005.26:191-212. Downloaded from
arjournals.annualreviews.org
by IRMO/Information Center on 03/14/05. For personal use only.
208 BELAY  SCHONBERGER
United States cannot be totally dismissed. However, this possibility is
probably
much smaller than the risk of contracting vCJD as a result of BSE
exposure during
any previous travel or residence in countries where a much higher rate
of BSE
has been documented. Recent reports of vCJD transmission via blood products
obtained from donors who were incubating the disease are of concern because
of a potentially large number of blood donors who might have been exposed to
BSE and are incubating the disease. Theoretically, these persons might
transmit
the vCJD agent if they donate blood while they are clinically
asymptomatic. The
blood donor deferral policy instituted by the FDA is expected to greatly
minimize
this possible risk of bloodborne transmission of vCJD in the United
States. The
findings of vCJD transmission in a patient who was heterozygous at codon 129
may have implications for the eventual size of the vCJD outbreak.
Heterozygous
patients may develop vCJD after a longer incubation period and at an
older age
than methionine homozygous patients, potentially resulting in a more
protracted
course for the vCJD outbreak.
To date, no convincing evidence of CWD transmission to humans has been
reported. Because the decade-long occurrence ofCWDhad been relatively
limited
to a small geographic area, it is possible that not enough human
exposure with
the appropriate latency period has occurred for the agent to overcome
the species
barrier and cause disease in humans. There is a concern that the level
of human
exposure to CWD might increase over time with the increasing spread of CWD
to new areas. Continued surveillance for possible human CWD among high-risk
populations (e.g., persons hunting for many years in the CWD-endemic
areas of
Colorado and Wyoming) and evaluation of the zoonotic potential of the CWD
agent in transgenic animal models should be conducted to monitor the
possibility
that the CWD agent can cause disease in humans.
Suspected cases of iatrogenic CJD, vCJD, or human CWD cases should be
reported to the CDC through local and state health departments. To
facilitate
surveillance for emerging forms of prion diseases such as vCJD and human
CWD,
the CDC, in collaboration with the American Association of
Neuropathologists,
established a National Prion Disease Pathology Surveillance Center. This
pathology
center is located at CaseWestern Reserve University, in Cleveland, Ohio, and
provides state-of-the-art diagnostic support free of charge to U.S.
physicians and
develops laboratory methods to detect emerging human prion diseases.
Autopsies
should be sought in all clinically suspected and diagnosed human prion
disease
cases. Brain tissues from these cases should be sent to the National
Prion Disease
Pathology Surveillance Center to confirm the diagnosis of CJD and
determine the
CJD subtype. Increased testing of brain tissues from suspected
case-patientswould
facilitate detection of the emergence of any new prion diseases, such as
vCJD or
possible human CWD, in the United States.
Annu. Rev. Public. Health. 2005.26:191-212. Downloaded from
arjournals.annualreviews.org
by IRMO/Information Center on 03/14/05. For personal use only.

snip...END

> Autopsies
> should be sought in all clinically suspected and diagnosed human prion
> disease
> cases.


SADLY, in the elderly, i think only about 1 in 25 deaths get autopsied...

kind regards,

Terry S. Singeltary Sr.


Terry S. Singeltary Sr. wrote:

> ##################### Bovine Spongiform Encephalopathy
> #####################
>
>
> Annu Rev Public Health. 2005;26:191-212.
>
>
> The public health impact of prion diseases (1).
>
> Belay ED, Schonberger LB.
>
> Division of Viral and Rickettsial Diseases, National Center for
> Infectious Diseases, Centers for Disease Control and Prevention,
> Atlanta, Georgia 30333; email: EBelay@cdc.gov.
>
> Several prion disease-related human health risks from an exogenous
> source can be identified in the United States, including the
> iatrogenic transmission of Creutzfeldt-Jakob disease (CJD), the
> possible occurrence of variant CJD (vCJD), and potential zoonotic
> transmission of chronic wasting disease (CWD). Although
> cross-species transmission of prion diseases seems to be limited by
> an apparent "species barrier," the occurrence of bovine spongiform
> encephalopathy (BSE) and its transmission to humans indicate that
> animal prion diseases can pose a significant public health risk.
> Recent reports of secondary person-to-person spread of vCJD via
> blood products and detection of vCJD transmission in a patient
> heterozygous at codon 129 further illustrate the potential public
> health impacts of BSE.
>
> PMID: 15760286 [PubMed - in process]
>
> ------------------------------------------------------------------------
>
> http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15760286
>
>
>
> Reply to Singletary 26 March 2003
>
> Previous Correspondence
> Top
>
> Ryan A. Maddox, MPH
> Centers for Disease Control and Prevention Atlanta GA,
> Ermias D. Belay, MD, Lawrence B. Schonberger, MD
>
> Send Correspondence to journal:
> Re: Reply to Singletary
>
>
>
> Email Ryan A.
> Maddox, MPH, et al.
>
> Mr. Singletary raises several issues related to current Creutzfeldt-
> Jakob disease (CJD) surveillance activities. Although CJD is not a
> notifiable disease in most states, its unique characteristics,
> particularly its invariably fatal outcome within usually a year of
> onset, make routine mortality surveillance a useful surrogate for
> ongoing CJD surveillance.[1] In addition, because CJD is least
> accurately diagnosed early in the course of illness,
> notifiable-disease surveillance could be less accurate than, if not
> duplicative of, current mortality surveillance.[1] However, in states
> where making CJD officially notifiable would meaningfully facilitate
> the collection of data to monitor for variant CJD (vCJD) or other
> emerging prion diseases, CDC encourages the designation of CJD as a
> notifiable disease.[1] Moreover, CDC encourages physicians to report
> any diagnosed or suspected CJD cases that may be of special public
> health importance (e.g., vCJD, iatrogenic CJD, unusual CJD clusters).
>
> As noted in our article, strong evidence is lacking for a causal link
> between chronic wasting disease (CWD) of deer and elk and human
> disease,[2] but only limited data seeking such evidence exist.
> Overall, the previously published case-control studies that have
> evaluated environmental sources of infection for sporadic CJD have not
> consistently identified strong evidence for a common risk factor.[3]
> However, the power of a case-control study to detect a rare cause of
> CJD is limited, particularly given the relatively small number of
> subjects generally involved and its long incubation period, which may
> last for decades. Because only a very small proportion of the US
> population has been exposed to CWD, a targeted surveillance and
> investigation of unusual cases or case clusters of prion diseases
> among persons at increased risk of exposure to CWD is a more efficient
> approach to detecting the possible transmission of CWD to humans. In
> collaboration with appropriate local and state health departments and
> the National Prion Disease Pathology Surveillance Center, CDC is
> facilitating or conducting such surveillance and case- investigations,
> including related laboratory studies to characterize CJD and CWD prions.
>
> Mr. Singletary also expresses concern over a recent publication by
> Asante and colleagues indicating the possibility that some sporadic
> CJD cases may be attributable to bovine spongiform encephalopathy
> (BSE).[4] The authors reported that transgenic mice expressing human
> prion protein homozygous for methionine at codon 129, when inoculated
> with BSE prions, developed a molecular phenotype consistent with a
> subtype of sporadic CJD. Although the authors implied that BSE might
> cause a sporadic CJD-like illness among persons homozygous for
> methionine, the results of their research with mice do not necessarily
> directly apply to the transmission of BSE to humans. If BSE causes a
> sporadic CJD-like illness in humans, an increase in sporadic CJD cases
> would be expected to first occur in the United Kingdom, where the vast
> majority of vCJD cases have been reported. In the United Kingdom
> during 1997 through 2002, however, the overall average annual
> mortality rate for sporadic CJD was not elevated; it was about 1 case
> per million population per year. In addition, during this most recent
> 6-year period following the first published description of vCJD in
> 1996, there was no increasing trend in the reported annual number of
> UK sporadic CJD deaths.[3, 5] Furthermore, surveillance in the UK has
> shown no increase in the proportion of sporadic CJD cases that are
> homozygous for methionine (Will RG, National CJD Surveillance Unit,
> United Kingdom, 2003; personal communication).
>
> References
>
> 1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Diagnosis and
> reporting of Creutzfeldt-Jakob disease. JAMA 2001;285:733-734.
>
> 2. Belay ED, Maddox RA, Gambetti P, Schonberger LB. Monitoring the
> occurrence of emerging forms of Creutzfeldt-Jakob disease in the
> United States. Neurology 2003;60:176-181.
>
> 3. Belay ED. Transmissible spongiform encephalopathies in humans. Annu
> Rev Microbiol 1999;53:283-314.
>
> 4. Asante EA, Linehan JM, Desbruslais M, et al. BSE prions propagate
> as either variant CJD-like or sporadic CJD-like prion strains in
> transgenic mice expressing human prion protein. EMBO J 2002;21:6358-6366.
>
> 5. The UK Creutzfeldt-Jakob Disease Surveillance Unit. CJD statistics.
> Available at: http://www.cjd.ed.ac.uk/figures.htm. Accessed February
> 18, 2003.
>
> RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob
> disease in the United States 26 March 2003
>
> Next Correspondence
> Top
>
> Terry S. Singeltary,
> retired (medically)
> CJD WATCH
>
> Send Correspondence to journal:
> Re: 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?
>
>
>
> http://www.neurology.org/cgi/eletters/60/2/176#535
>
> TSS
>
> ######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html
> ##########
>

######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html ##########






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