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From: TSS ()
Subject: CJD (NEW VAR.) UPDATE 2006 (10) ProMED
Date: October 2, 2006 at 10:11 am PST


CJD (NEW VAR.) UPDATE 2006 (10)
*******************************
A ProMED-mail post

ProMED-mail is a program of the
International Society for Infectious Diseases

[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
Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease
Statistics, Mon 2 Oct 2006 [edited]


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
. -
Mod.CP]

--
ProMED-mail

******
[2] Correction
Date: Tue 5 Sep 2006
From: "Terry S Singeltary Sr"


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

[see also:
CJD (new var.) update 2006 (09) 20060904.2519
CJD (new var.) update 2006 (08) 20060807.2207
CJD (new var.) update 2006 (07) 20060703.1831
CJD (new var.) - Netherlands: 2nd case 20060623.1741
CJD (new var.) update 2006 (06) 20060605.1566
CJD (new var.) update 2006 (05) 20060508.1332
CJD (new var.) update 2006 (04) 20060404.1005
CJD (new var.) update 2006 (03) 20060306.0728
CJD (new var.) - UK: 3rd transfusion-related case 20060209.0432
CJD (new var.) update 2006 (02) 20060206.0386
CJD (new var.) update 2006 (01) 20060111.0101
CJD (new var.) update 2006 20060111.0101
2005
---
CJD (new var.) update 2005 (12) 20051209.3547
CJD (new var.) update 2005 (11) 20051108.3270
CJD (new var.) update 2005 (10) 20051006.2916
CJD (new var.) update 2005 (09) 20050905.2627
CJD (new var.) update 2005 (08) 20050801.2237
CJD (new var.) update 2005 (07) 20050703.1889
CJD (new var.) update 2005 (06) 20050607.1584
CJD (new var.) update 2005 (05) 20050505.1243
CJD (new var.) update 2005 (04) 20050405.0982
CJD (new var.) update 2005 (03) 20050308.0687
CJD (new var.) update 2005 (02) 20050211.0467
CJD (new var.) - UK: update 2005 (01) 20050111.0095
2004
---
CJD, genetic susceptibility 20041112.3064
CJD (new var.) - UK: update 2004 (14) 20041206.3242
CJD (new var.) - UK: update 2004 (13) 20041103.2977
CJD (new var.) - UK: update 2004 (12) 20041023.2871
CJD (new var.) - UK: update 2004 (11) 20041008.2758
CJD (new var.) - UK: update 2004 (10) 20040909.2518
CJD (new var.) - UK: update 2004 (09) 20040809.2199
CJD (new var.) - UK: update 2004 (08) 20040806.2150
CJD (new var.) - UK: update 2004 (07) 20040706.1807
CJD (new var.) - UK: update 2004 (06) 20040608.1535
CJD (new var.) - UK: update 2004 (05) 20040510.1262
CJD (new var.) - UK: update 2004 (04) 20040406.0937
CJD (new var.) - UK: update 2004 (03) 20040314.0713
CJD (new var.) - UK: update 2004 (02) 20040202.0400
CJD (new var.) - UK: update 2004 (01) 20040106.0064
CJD (new var.) - France: 8th case 20041022.2864
CJD (new var.) - France: 9th case 20041123.3138
CJD (new var.), blood supply - UK 20040318.0758
CJD (new var.), carrier frequency study - UK 20040521.1365
2003
---
CJD (new var.) - UK: update 2003 (13) 20031216.3072
CJD (new var.) - UK: update 2003 (01) 20030108.0057
2002
---
CJD (new var.) - UK: update Dec 2002 20021207.5997
CJD (new var.) - UK: update Jan 2002 20020111.3223
2001
---
CJD (new var.), incidence & trends - UK (02) 20011124.2875
CJD (new var.), incidence & trends - UK 20011115.2816
CJD (new var.) - UK: reassessment 20011029.2671
CJD (new var.) - UK: update Oct 2001 20011005.2419
CJD (new var.) - UK: regional variation (02) 20010907.2145
CJD (new var.) - UK: update Sep 2001 20010906.2134
CJD (new var.) - UK: update Aug 2001 20010808.1872
CJD (new var.) - UK: 9th Annual Report 20010628.1231
CJD (new var.) - UK: update June 2001 20010622.1188
CJD (new var.) - UK: update 3 Jan 2001 20010104.0025]

................cp/sh


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


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

TSS




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