PDA

View Full Version : CJD (NEW VAR.) UPDATE 2006 (10) ProMED


flatfish
10-06-2006, 01:25 PM
CJD (NEW VAR.) UPDATE 2006 (10)
*******************************
A ProMED-mail post
<http://www.promedmail.org>
ProMED-mail is a program of the
International Society for Infectious Diseases
<http://www.isid.org>

[The definition of the designations deaths, definite cases, probable vCJD
cases, and the case definitions can be found by accessing the Department of
Health website, or by reference to a previous ProMED-mail post in this
thread (for example, CJD (new var.) - UK: update March 2002 20020305.3693).

Data on vCJD cases from other parts of the world are now included in these
updates whenever available.

Also, data on other forms of CJD (sporadic, iatrogenic, familial and GSS)
are now included when they have some relevance to the incidence and
etiology of vCJD. - Mod.CP]

In this update:
[1] UK: Department of Health monthly CJD statistics, Mon 2 Oct 2006
[2] Germany: characterization of atypical BSE: correction

******
[1] UK: vCJD and CJD statistics
Date: Mon 2 Oct 2006
From: ProMED-mail <promed@promedmail.org>
Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease
Statistics, Mon 2 Oct 2006 [edited]
<http://www.gnn.gov.uk/Content/Detail.asp?ReleaseID=231175&NewsAreaID=2>


The Department of Health is today [Mon 2 Oct 2006] issuing the latest
information about the numbers of known cases of Creutzfeldt-Jakob disease.
This includes cases of variant Creutzfeldt-Jakob disease [abbreviated in
ProMED-mail as CJD (new var.) or vCJD], the form of the disease thought to
be linked to BSE (bovine spongiform encephalopathy).

Definite and probable CJD cases in the UK, as at Fri 29 Sep 2006:
-----------------------------------------------------------------
Summary of vCJD cases - deaths
------------------------------
Deaths from definite vCJD (confirmed): 112
Deaths from probable vCJD (without neuropathological confirmation): 44
Deaths from probable vCJD (neuropathological confirmation pending): 0
Number of deaths from definite or probable vCJD (as above): 156

Summary of vCJD cases - alive
-----------------------------
Number of probable vCJD cases still alive: 6

Total
-----
Number of definite or probable vCJD (dead and alive): 162
(The next table will be published on Mon 6 Nov 2006).

Since the previous monthly statistics were released on Mon 4 Sep 2006, the
total number of deaths from definite vCJD remains unchanged at 156, and the
overall total number of definite or probable vCJD cases (dead and alive)
also remains unchanged at 162 for the second month in succession.

These data are consistent with the view that the vCJD outbreak in the UK is
in decline. The total number of deaths due to vCJD in the UK remains 156.
The peak number of deaths was 28 in the year 2000, followed by 20 in 2001,
17 in 2002, 18 in 2003, and 9 in 2004, 5 in 2005. The number of deaths due
to definite or probable vCJD in the UK during the first 9 months of 2006
remains 3.

Totals for all types of CJD cases in the UK in 2005 and 2006
------------------------------------------------------------
As of 29 Sep 2006, in the UK in the year 2005, there were 122 referrals of
suspected CJD, and there were 65 deaths from sporadic CJD, 6 from familial
CJD, 3 from iatrogenic CJD, 6 GSS (Gerstmann-Straussler-Scheinker) syndrome
cases, and 5 deaths from vCJD.

The corresponding figures so far for the 1st 9 months of 2006 are: 81
referrals, 41 deaths from sporadic CJD, 3 from vCJD, 4 from familial CJD, 3
from GSS and one from iatrogenic CJD.

During the period 1995, when vCJD was first diagnosed, up to the present
there have been 937 deaths from all forms of CJD including 156 deaths
attributable to definite or probable vCJD.

[These data are accessible via
<http://www.gnn.gov.uk/Content/Detail.asp?ReleaseID=231175&NewsAreaID=2>. -
Mod.CP]

--
ProMED-mail
<promed@promedmail.org>

******
[2] Correction
Date: Tue 5 Sep 2006
From: "Terry S Singeltary Sr" <flounder9@verizon.net>


Characterization of atypical BSE in Germany: correction
-------------------------------------------------------
[In the Moderator's comment accompanying the abstract of the paper entitled
"Atypical BSE in Germany-Proof of transmissibility and biochemical
characterization'" by A Buschmannaet et al, (see part [2] of CJD (new var.)
update 2006 (09) 20060904.2519) it was wrongly implied that Terry S
Singeltary Sr endorsed the conclusions of the paper, whereas his comments
were intended merely to highlight the conclusions of the paper. Namely that
the atypical cases suggested the possible existence of sporadic BSE cases
in bovines and perhaps the BSE epidemic in the UK could have also been
initiated by an intraspecies transmission from a sporadic BSE case. I
apologize for inadvertently misrepresenting Terry's views. - Mod.CP]

Terry S Singeltary Sr has written the following. "In fact I disagree with
the spontaneous/sporadic BSE/TSE theory, IF this is what the authors of
this paper meant by 'sporadic BSE' to mean. For one thing, it has never
been proven. IF atypical BSE i.e. BASE is so similar to some sporadic CJDs,
then how did they all of a sudden become spontaneous? Could it not be so
simple as an atypical BSE i.e. BASE was transmitted the same way most of
all of the other BSE cattle were i.e. feed of just an atypical source, thus
causing atypical strain? Why would these animals not develop an atypical
BSE i.e. BASE from the same oral route? WHAT about an atypical strain
mutating to become infectious via a lateral or horizontal mode in the
bovine, as with CWD and scrapie? Also, please explain to me how a distinct
synthetic prion, of a strain that is supposedly unlike any other we have
ever seen, how can this explain 6 different documented phenotypes of
sporadic CJD to date?

It's like trying to explain away all the 6 phenotypes of sporadic CJD with
the spontaneous theory, it's just not scientific. OR, if you render an
atypical TSE of what ever phenotype, in what ever species, of the atypical
strain and feed it to another whatever species, nothing happens x 1 x 2 x 3
x 4 etc passage? This all has been proven?

Please show me these transmission studies? What Prusiner and Soto produced
in vitro did not look like any natural field TSE, and as far as this in
vitro TSE being infectious, well this was questionable too. If this was the
case, then why does CWD not spontaneously happen in geographical areas
where it has never been documented, OR with scrapie, as in scrapie free New
Zealand? If TSE were to arise spontaneously, I don't see how the scientific
arena can dictate which animal TSE can arise spontaneously, and which ones
cannot, without any scientific evidence to support this to date, and by
even suggesting this in this study, was not scientific. The words sporadic
and spontaneous are very confusing in the world literature of human and
animal TSE and, in my opinion, should not be used as terminology of any TSE."

--
Terry S Singeltary Sr
<flounder9@verizon.net>

[see also:
CJD (new var.) update 2006 (09) 20060904.2519
snip...
Become a ProMED-mail Premium Subscriber at
<http://www.isid.org/ProMEDMail_Premium.shtml>
************************************************** **********
Visit ProMED-mail's web site at <http://www.promedmail.org>.
Send all items for posting to: promed@promedmail.org


snip...continued

flatfish
10-06-2006, 01:26 PM
UK SPORADIC CJD CASES WENT FROM 28 IN 1990 TO 76 IN 2003 ;


http://www.dh.gov.uk/assetRoot/04/10/07/99/04100799.pdf




SEE STEADY INCREASE IN SPORADIC CJD CASES IN THE USA FROM 54 in
1997 TO 167 IN 2004. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...


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




TSS

#################### https://lists.aegee.org/bse-l.html ####################


4 October 2006 - The final minutes (154 KB) of the 93rd SEAC meeting held
on 6 July 2006 have been published.


snip...


The committee
will hold a reserved business session in the afternoon to allow
discussion of unpublished studies on BSE in cattle. This is in
accordance with the SEAC Code of Practice. Short summaries of
the open and reserved business discussions will be posted on the
SEAC website next week.


snip...


. The Chair noted that recent reports described two cases of
BSE in cattle in the United States of America (USA) as being
similar to atypical cases of BSE found in a number of
European countries. The Chair suggested that the term
"atypical BSE", used in the USA report, is potentially
confusing and that this would be discussed under any other
business. Dr Danny Matthews (Veterinary Laboratories
Agency [VLA]) explained that data from western blots of the
USA cases resembled that of a small number of atypical
cases of BSE in France. A study of the French cases had
shown the condition to be transmissible to mice by
intracerebral (ic) inoculation with the neuropathological
phenotype maintained on transmission3. Claims have been
made about the existence of atypical cases of BSE in other
countries but these have yet to be confirmed. No study has
yet examined the tissue distribution of abnormal prion protein
(PrPSc) or infectivity in such atypical cases of BSE. ...

snip...

http://www.seac.gov.uk/minutes/final93.pdf



[Docket No. FSIS-2006-0011] FSIS Harvard Risk Assessment of Bovine
Spongiform Encephalopathy (BSE)


snip...


3. WHY still now only partial ruminant feed ban, with the fact that now we
seem to have 3 cases of nvCJD to humans
i.e. humanbovineTSE that were responsible from blood, and the fact the last
2 mad cows documented in the USA were
that of an Atypical strain, would it not seem prudent to remove blood as
well from ruminant feed ?

Page 4 of 98

8/3/2006

WOULD it not seem prudent to improve and expand the SRM list now? as per
your own thinking ;

> 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.


FULL text ;


Research Project: Study of Atypical Bse
Location: Virus and Prion Diseases of Livestock
Project Number: 3625-32000-073-07
Project Type: Specific C/A
Start Date: Sep 15, 2004
End Date: Sep 14, 2007

Objective:

The objective of this cooperative research project with Dr. Maria Caramelli
from the Italian BSE Reference Laboratory in Turin, Italy, is to
conduct comparative studies with the U.S. bovine spongiform encephalopathy
(BSE) isolate and the atypical BSE isolates identified in Italy.
The studies will cover the following areas: 1. Evaluation of present
diagnostics tools used in the U.S. for the detection of atypical BSE cases.
2.
Molecular comparison of the U.S. BSE isolate and other typical BSE isolates
with atypical BSE cases. 3. Studies on transmissibility and tissue
distribution of atypical BSE isolates in cattle and other species.

Approach:

This project will be done as a Specific Cooperative Agreement with the
Italian BSE Reference Laboratory, Istituto
Zooprofilattico Sperimentale del Piemonte, in Turin, Italy. It is essential
for the U.S. BSE surveillance program to
analyze the effectiveness of the U.S diagnostic tools for detection of
atypical cases of BSE. Molecular comparisons of
the U.S. BSE isolate with atypical BSE isolates will provide further
characterization of the U.S. BSE isolate.
Transmission studies are already underway using brain homogenates from
atypical BSE cases into mice, cattle and
sheep. 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


Page 5 of 98
8/3/2006

HOWEVER, JAPAN has already shown infectivity in tissues other than CNS in
there atypical TSE in cattle, so why should we wait,
and expose many to this agent needlessly, since the last two mad cows in the
USA were also atypical TSE ?
PrPSc distribution of a natural case of bovine spongiform encephalopathy

Yoshifumi Iwamaru, Yuka Okubo, Tamako Ikeda, Hiroko Hayashi, Mori- kazu
Imamura, Takashi Yokoyama and Morikazu Shinagawa
Priori Disease Research Center, National Institute of Animal Health, 3-1-5
Kannondai, Tsukuba 305-0856 Japan gan@affrc.go.jp

Abstract ... snip...full text 98 pages ;


http://www.fsis.usda.gov/OPPDE/Comments/2006-0011/2006-0011-1.pdf



Published online before print February 17, 2004, 10.1073/pnas.0305777101

Medical Sciences
Identification of a second bovine amyloidotic spongiform encephalopathy:
Molecular similarities with sporadic Creutzfeldt-Jakob disease



http://www.pnas.org/cgi/content/abstract/0305777101v1


J Gen Virol 85 (2004), 2471-2478; DOI 10.1099/vir.0.79889-0
Characterization of two distinct prion strains derived from bovine
spongiform encephalopathy transmissions to inbred mice

http://vir.sgmjournals.org/cgi/content/abstract/85/8/2471



[Docket No. 03-025IFA] FSIS Prohibition of the Use of Specified Risk
Materials for Human Food and Requirement for the Disposition of
Non-Ambulatory Disabled Cattle

03-025IFA
03-025IFA-2


http://www.fsis.usda.gov/OPPDE/Comments/03-025IFA/03-025IFA-2.pdf



SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2004. SPORADIC CJD CASES TRIPLED;

please note 'TYPE UNKNOWN' ???


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



kind regards,

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


P.S., THANKS JOHN FOR GETTING THE SITE UP AND GOING AGAIN HERE AT BRAINTALKS!!!