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From: TSS ()
Subject: PRO/AH/EDR> CJD (new var.) update 2005 (12)
Date: December 9, 2005 at 11:31 am PST

CJD (NEW VAR.) UPDATE 2005 (12)
A ProMED-mail post

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

Sponsored in part by Elsevier, publisher of
Tropical Infectious Diseases, 2nd edition

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

Data on vCJD cases from other parts of the world are now included in these
updates. Also data on other forms of CJD (spradic, iatrogenic, familial and
GSS) are now included where they have some relevance to the incidence and
etiology of vCJD. - Mod.CP]

In this update:

[1] UK: DH vCJD Monthly Statistics - as of Mon 5 Dec 2005
[2] UK: National CJD Surveillance Unit -- 13th Annual Report 2004

[1] UK: DH vCJD Monthly Statistics - as of Mon 5 Dec 2005
Date: Fri 9 Dec 2005
From: ProMED-mail
Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease
Statistics, Press release no. 2005/0438, Mon 5 Dec 2005 [edited]

Monthly Creutzfeldt Jakob Disease Statistics - As of 5 Dec 2005
The Department of Health is today [Mon 5 Dec 2005] 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 of Fri 2 Dec 2005:

Summary of vCJD Cases - Deaths

Deaths from definite vCJD (confirmed): 109

Deaths from probable vCJD (without neuropathological confirmation): 43

Deaths from probable vCJD (neuropathological confirmation pending): 1

Number of deaths from definite or probable vCJD (as above): 153

Summary of vCJD Cases - Alive

Number of probable vCJD cases still alive: 6


Number of definite or probable vCJD (dead and alive): 159

(The next table will be published on Mon 9 Jan 2006)

[Since the previous monthly statistics were released on Mon Nov 7 Nov 2005,
the total number of deaths from definite or probable vCJD has increased by
one. Consequently the overall total number of definite or probable vCJD
cases (dead and alive) is now 159.

These data are consistent with the view that the vCJD outbreak in the UK is
now in decline. The number of deaths due to definite or probable vCJD in
the UK during the 1st 11 months of 2005 is now 5. 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. - Mod.CP]

Totals for All Types of CJD Cases in the UK during 2005
[As of 2 Dec 2005, so far in the UK for the year 2005 there have 109
referrals of suspected CJD; and there have been 55 deaths from sporadic
CJD, 4 from familial CJD, 3 from iatrogenic CJD, 4 GSS
((Gerstmann-Straussler-Scheinker) syndrome cases, and 5 deaths from vCJD.

I am indebted to Terry S. Singeltary Sr. for
drawing my attention to similar data concerning CJD surveillance in the
USA, which are being compiled by the National Prion Disease Pathology
Surveillance Center at Case Western Reserve University beginning from the
year 1997 (. The corresponding figures for
the USA for the year 2005 up to the end of September are: 256 referrals of
suspected CJD, which include 46 cases of sporadic CJD, 22 cases of familial
CJD, none of iatrogenic CJD, and none of vCJD. Another 52 cases have been
diagnosed as prion diseases with precise identification pending.

Since 1997 in the USA there have been 876 confirmed cases of sporadic CJD,
159 cases of familial CJD, 15 cases of iatrogenic CJD, and one case of vCJD
(almost certainly acquired in the UK). No cases of GSS
(Gerstmann-Straussler-Scheinker) syndrome have been diagnosed in the USA
since the inception of surveillance in 1997.

The corresponding totals for confirmed deaths from definite and probable
CJD in the UK since 1990 to the end of November 2005 are 819 sporadic
cases, 48 familial cases, 51 iatrogenic cases, 27 GSS cases and 153 vCJD
cases. The reasons for the discrepancies in the frequencies, vCJD apart,
are unknown. - Mod.CP]


[2] UK: National CJD Surveillance Unit -- 13th Annual Report 2004
Date: Tue 8 Nov 2005
From: Terry S. Singeltary Sr.
Source: The National CJD Surveillance Unit (NCJDS), Department of Health
and the Scottish Executive Health Department, Tue 7 Nov 2005. [edited]


UK National CJD Surveillance Unit - 13th Annual Report 2004
The 13th Annual Report of the National Creutzfeldt-Jakob Disease
Surveillance Unit was published today [7 Nov 2005]. The report looks back
over the period from May 1990 when the Unit was set up to 31 Dec 2004. The
report outlines the Unit's work in the clinical surveillance of sporadic,
variant (vCJD) and iatrogenic CJD.

Also included in the report are details of a study on the potential risk
factors for variant and sporadic CJD and the work of the National Care Team
in arranging care and advice to the families of CJD patients.

The full report is available on the NCJDSU website at
. The following is the Summary.

"The national surveillance programme for Creutzfeldt-Jakob disease (CJD) in
the UK was initiated in May 1990. In 1999, the National CJD Surveillance
Unit (NCJDSU) became a WHO Collaborative Centre for Reference and Research
on the surveillance and epidemiology of human transmissible
encephalopathies (TSEs). In September 2001 the National Care Team was
formed, which currently comprises a care coordinator and a secretary. The
National Care Team is based within the NCJDSU and was formed in response to
concerns regarding the care of CJD patients.

The information provided in this 13th report continues to provide evidence
of a high level of case ascertainment. The decrease in referrals is however
potentially concerning from the point of view of complete case
ascertainment. The reason for this drop is unknown and the numbers will
need careful monitoring over 2005. It is particularly notable that the
number of recorded sporadic CJD deaths in 2004 is lower than in the 3
previous years; however, the death data for 2004 may be incomplete.

Detailed clinical and epidemiological information has been obtained for the
great majority of patients. The case-control study for risk factors of CJD
has continued and initial analysis has been undertaken. The post mortem
rate for patients with suspected CJD is high, although there is ongoing
evidence that this rate continues to decline, in line with general autopsy
rates in the UK. This is reflected in the reduced number of brain specimens
examined in the neuropathology laboratory in 2004. The reduction in
sporadic CJD numbers (32 in 2004, 52 in 2003) is another potential concern
along with the fall in referral numbers and sporadic CJD deaths noted above.

In 1990-2004 mortality rates from sporadic CJD in England, Wales, Scotland
and Northern Ireland were, respectively, 0.86, 1.05, 0.88 and
0.53/million/year. The difference between the rates in each country is not
statistically significant (p>0.2). These rates are comparable to those
observed in other countries in Europe and elsewhere in the world, including
countries which are free of BSE. There was some variation in the observed
mortality rates between the different regions within the UK, but this
variation is not statistically significant (p>0.2). The highest and lowest
mortality rates from sporadic CJD were observed in the South West (SMR=135)
and Northern Ireland (SMR=74).

Up to 31 Dec 2004, there have been 148 deaths from definite or probable
variant CJD (vCJD) in the UK. Of these, 106 were confirmed by
neuropathology. A further 5 probable cases were alive as at 31 Dec 2004.
The clinical, neuropathological and epidemiological features of these cases
of vCJD are remarkably uniform and consistent with our previous
descriptions. Analysis of the incidence of vCJD onsets and deaths from
January 1994 to December 2004 indicates that a peak has been passed. While
this is an encouraging finding, incidence of vCJD may increase again,
particularly if different genetic subgroups are found to be affected. The
identification of disease-related PrP in the spleen of a blood recipient of
PRNP-129 MVgenotype emphasises this point. In addition, this case along
with the report of the appendix study suggests at least a possibility of a
greater number of preclinical or subclinical cases in the population than
might be indicated by the present numbers of confirmed cases.

Risk factors for the development of vCJD include age, residence in the UK
and methionine homozygosity at codon 129 of the prion protein gene - all
131 cases of vCJD with available genetic analysis (86 percent) have been
methionine homozygotes. The incidence of vCJD across the UK continues to
show a "North-South" difference (though slightly less than previously
reported), with a higher incidence being maintained in the North of the UK.
The underlying reason for this finding is not clear. The only statistically
significant geographic cluster of vCJD cases in the UK was in
Leicestershire. All geographically associated cases of vCJD are considered
for investigation according to a protocol which involves the NCJDSU,
colleagues at the HPA, HPS and local public health physicians.

The activities of the NCJDSU are strengthened by collaboration in other
surveillance projects, including the Transfusion Medicine Epidemiology
Review and the study of Progressive Intellectual and Neurological
Deterioration in Children. The collaboration of our colleagues in these
projects is greatly appreciated; the effectiveness of this collaboration
allowed the identification in 2003 of a case of variant CJD associated with
blood transfusion and the identification in 2004 of PrPres in the spleen of
a blood recipient. The success of the National CJD Surveillance Project
continues to depend on the extraordinary level of co-operation from the
neuroscience community and other medical and paramedical staff throughout
the UK. We are particularly grateful to the relatives of patients for their
help with this study."

Terry S. Singeltary Sr.

[see also:
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
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
CJD (new var.) - UK: update 2003 (13) 20031216.3072
CJD (new var.) - UK: update 2003 (01) 20030108.0057
CJD (new var.) - UK: update Dec 2002 20021207.5997

CJD (new var.) - UK: update Jan 2002 20020111.3223
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]

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Coexistence of multiple PrPSc types in individuals with

Creutzfeldt-Jakob disease

Magdalini Polymenidou, Katharina Stoeck, Markus Glatzel, Martin Vey, Anne Bellon, and Adriano Aguzzi


Background The molecular typing of sporadic Creutzfeldt-Jakob disease (CJD) is based on the size and glycoform

ratio of protease-resistant prion protein (PrPSc), and on PRNP haplotype. On digestion with proteinase K, type 1 and

type 2 PrPSc display unglycosylated core fragments of 21 kDa and 19 kDa, resulting from cleavage around amino

acids 82 and 97, respectively.

Methods We generated anti-PrP monoclonal antibodies to epitopes immediately preceding the differential proteinase

K cleavage sites. These antibodies, which were designated POM2 and POM12, recognise type 1, but not type 2, PrPSc.

Findings We studied 114 brain samples from 70 patients with sporadic CJD and three patients with variant CJD.

Every patient classified as CJD type 2, and all variant CJD patients, showed POM2/POM12 reactivity in the

cerebellum and other PrPSc-rich brain areas, with a typical PrPSc type 1 migration pattern.

Interpretation The regular coexistence of multiple PrPSc types in patients with CJD casts doubts on the validity of

electrophoretic PrPSc mobilities as surrogates for prion strains, and questions the rational basis of current CJD



The above results set the existing CJD classifications

into debate and introduce interesting questions about

human CJD types. For example, do human prion types

exist in a dynamic equilibrium in the brains of affected

individuals? Do they coexist in most or even all CJD

cases? Is the biochemically identified PrPSc type simply

the dominant type, and not the only PrPSc species?

Published online October 31, 2005


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