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From: TSS ()
Subject: CJD statistics published As at 3 November 2006
Date: November 7, 2006 at 9:07 am PST

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

CJD statistics published As at 3 November 2006

Mon Nov 6, 2006 10:24


Monthly Creutzfeldt Jakob disease statistics published


The Department of Health is today issuing the latest information about the numbers of known cases of Creutzfeldt Jakob disease. This includes cases of variant Creutzfeldt Jakob disease (vCJD) - the form of the disease thought to be linked to BSE. The position is as follows:

Definite and probable CJD cases in the UK:

As at 3 November 2006

Summary of vCJD cases

Deaths

Deaths from definite vCJD (confirmed): 112

Deaths from probable vCJD (without neuropathological confirmation): 46

Deaths from probable vCJD (neuropathological confirmation pending): 0

Number of deaths from definite or probable vCJD (as above): 158

Alive

Number of probable vCJD cases still alive: 6

Total number of definite or probable vCJD (dead and alive): 164

The next table will be published on Monday 4th December 2006

Referrals: a simple count of all the cases which have been referred to the National CJD Surveillance Unit for further investigation in the year in question. CJD may be no more than suspected; about half the cases referred in the past have turned out not to be CJD. Cases are notified to the Unit from a variety of sources including neurologists, neuropathologists, neurophysiologists, general physicians, psychiatrists, electroencephalogram (EEG) departments etc. As a safety net, death certificates coded under the specific rubrics 046.1 and 331.9 in the 9th ICD Revisions are obtained from the Office for National Statistics in England and Wales, the General Register Office for Scotland and the General Register Office for Northern Ireland.

Deaths: All columns show the number of deaths that have occurred in definite and probable cases of all types of CJD and GSS in the year shown. The figures include both cases referred to the Unit for investigation while the patient was still alive and those where CJD was only discovered post mortem (including a few cases picked up by the Unit from death certificates). There is therefore no read across from these columns to the referrals column. The figures will be subject to retrospective adjustment as diagnoses are confirmed.

Definite cases: this refers to the diagnostic status of cases. In definite cases the diagnosis will have been pathologically confirmed, in most cases by post mortem examination of brain tissue (rarely it may be possible to establish a definite diagnosis by brain biopsy while the patient is still alive).

Probable vCJD cases: are those who fulfil the 'probable' criteria set out in the Annex and are either still alive, or have died and await post mortem pathological confirmation. Those still alive will always be shown within the current year's figures.

Sporadic: Classic CJD cases with typical EEG and brain pathology. Sporadic cases appear to occur spontaneously with no identifiable cause and account for 85% of all cases.

Probable sporadic: Cases with a history of rapidly progressive dementia, typical EEG and at least two of the following clinical features; myoclonus, visual or cerebellar signs, pyramidal/extrapyramidalsigns or akinetic mutism. Iatrogenic: where infection with classic CJD has occurred accidentally as the result of a medical procedure. All UK cases have resulted from treatment with human derived pituitary growth hormones or from grafts using dura mater (a membrane lining the skull).

Familial: cases occurring in families associated with mutations in the PrP gene (10 - 15% of cases).

GSS: Gerstmann-Straussler-Scheinker syndrome - an exceedingly rare inherited autosomal dominant disease, typified by chronic progressive ataxia and terminal dementia. The clinical duration is from 2 to 10 years, much longer than for CJD.

vCJD: Variant CJD, the hitherto unrecognised variant of CJD discovered by the National CJD

Surveillance Unit and reported in The Lancet on 6 April 1996. This is characterised clinically by a progressive neuropsychiatric disorder leading to ataxia, dementia and myoclonus (or chorea) without the typical EEG appearance of CJD. Neuropathology shows marked spongiform change and extensive florid plaques throughout the brain.

Definite vCJD cases still alive: These will be cases where the diagnosis has been pathologically confirmed (by brain biopsy).

Related links

Download cjd annual stats (PDF, 145K)

Notes to editor

ANNEX

DIAGNOSTIC CRITERIA FOR VARIANT CJD

I A) PROGRESSIVE NEUROPSYCHIATRIC DISORDER

B) DURATION OF ILLNESS > 6 MONTHS

C) ROUTINE INVESTIGATIONS DO NOT SUGGEST AN ALTERNATIVE

DIAGNOSIS

D) NO HISTORY OF POTENTIAL IATROGENIC EXPOSURE

II A) EARLY PSYCHIATRIC SYMPTOMS *

B) PERSISTENT PAINFUL SENSORY SYMPTOMS **

C) ATAXIA

D) MYOCLONUS OR CHOREA OR DYSTONIA

E) DEMENTIA

III A) EEG DOES NOT SHOW THE TYPICAL APPEARANCE OF SPORADIC

CJD *** (OR NO EEG PERFORMED)

B) BILATERAL PULVINAR HIGH SIGNAL ON MRI SCAN

IV A) POSITIVE TONSIL BIOPSY

DEFINITE: IA (PROGRESSIVE NEUROPSYCHIATRIC DISORDER) and

NEUROPATHOLOGICAL CONFIRMATION OF vCJD **** PROBABLE: I and 4/5 OF II and III A and III B

or I and IV A

* depression, anxiety, apathy, withdrawal, delusions.

** this includes both frank pain and/ or unpleasant dysaesthesia

*** generalised triphasic periodic complexes at approximately one per second

****spongiform change and extensive PrP deposition with florid plaques, throughout the cerebrum and cerebellum.

Client ref NO REF NUMBER

GNN ref 140143P

https://www.gnn.gov.uk/content/detail.asp?NewsAreaID=2&ReleaseID=239915

SEE SPORADIC CJD CASES EU

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

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


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 collection.

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

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm


http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf

sporadic CJD Canada

CJD occurs spontaneously, usually in the

elderly. The overall incidence of CJD is low,

with some 35 to 40 cases identified in Canada

annually.

http://www.prionetcanada.ca/newsletter/PrioNews_SeptOct2006.pdf

http://www.phac-aspc.gc.ca/hcai-iamss/cjd-mcj/cjdss-ssmcj/pdf/0999_e.pdf

see latest CASE REPORTS

http://www.phac-aspc.gc.ca/bid-bmi/dsd-dsm/ndmr-rmmdo/pdf/2006/jun06.pdf

TSS

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


CJD (NEW VARIANT) UPDATE 2006 (11)
**********************************
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 6 Nov 2006
[2] EUROCJD data as of 31 Oct 2006
[3] France: novel prion strain

******
[1] UK: Department of Health monthly CJD statistics, Mon 6 Nov 2006
Date: Mon 6 Nov 2006
From: ProMED-mail
Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease
Statistics [edited]


The Department of Health is today [Mon 6 Nov 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 of Fri 3 Nov 2006:
-----------------------------------------------
Summary of vCJD cases - deaths
-----------------------------
Deaths from definite vCJD (confirmed): 112
Deaths from probable vCJD (without neuropathological confirmation): 46
Deaths from probable vCJD (neuropathological confirmation pending): 0
Number of deaths from definite or probable vCJD (as above): 158

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

Total
-----
Number of definite or probable vCJD (dead and alive): 164

(The next table will be published on Mon 4 Dec 2006).

Since the previous monthly statistics were released on Mon 6 Nov
2006, the total number of deaths from definite vCJD has increased by
2 and stands at 158, and the overall total number of definite or
probable vCJD cases (dead and alive) has increased by 2, making the
overall total 164.

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 is
now 158. 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 1st 10 months of 2006 has risen to 5.

Totals for all types of CJD cases in the UK in 2005 and 2006
-----------------------------------------------
As of 3 Nov 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 10 months of 2006 are:
87 referrals, 48 deaths from sporadic CJD, 5 from vCJD, 4 from
familial CJD, 3 from GSS and one from iatrogenic CJD.

During the period 1995, when vCJD was 1st diagnosed, up to the
present, there have been 946 deaths from all forms of CJD, including
the 158 deaths attributable to definite or probable vCJD.

[These data are accessible via
.]

--
ProMED-mail

******
[2] EUROCJD data as of 31 Oct 2006
Date: Tue 31 Oct 2006
From: ProMED-mail
Source: EUROCJD [edited]


The European And Allied Countries Collaborative Study Group of CJD (EUCJD)
-----------------------------------------------
This web-site includes information from 2 projects funded by the
European Commission. The EUROCJD project started in 1993 and compares
data from national registries in Australia, Austria, Canada, France,
Germany, Italy, the Netherlands, Slovakia, Spain, Switzerland and the
UK. The NEUROCJD project started in 1998 after the European Union
Council recommended that epidemiological surveillance of CJD should
be extended to all member states. The member states involved in this
project are Belgium, Denmark, Finland, Greece, Iceland, Ireland,
Israel, Norway and Portugal. Both projects are coordinated from the
National CJD Surveillance Unit based in Edinburgh.

Current data as of October 2006
-------------------------------
Country / Total No. of Primary cases (No. alive) / Cumulative
residence in UK (>6 months) / Secondary transmission by blood transfusion

United Kingdom / 162 (6) / 164 / 2 (0)

France / 21 (2) / 1 / 0

Republic of Ireland / 4 (1) / 2 / 0

Italy / 1 (0) / 0 / 0

USA / 2 (0) / 2 / 0

Canada / 1 (0) / 1 / 0

Saudi Arabia / 1 (1) / 0 / 0

Japan / 1* (0) / 0 / 0

Portugal / 1 (1) / 0 / 0

Spain / 1 (0) / 0 / 0

Total / 197 (12) / - / 2

Footnote:
---------
* Residence in the UK for 24 days

--
ProMED-mail

******
[3] France: novel prion strain
Date: Thu 12 Oct 2006
From: Terry Singeltary
Source: PLoS Pathogens 2(10); published ahead of print [edited]


Terry S. Singeltary Sr. has drawn ProMED-mail's attention to the
following paper published ahead of print in PLoS Pathogens, which
although not directly featuring vCJD, he considers is relevant to
understanding the origin of the BSE outbreak in cattle and vCJD in
humans. He comments that this research indicates that different prion
disease phenotypes result from inoculation of cattle with 2
temporally separated sources of sheep scrapie from Great Britain.

The paper is entitled "Isolation from Cattle of a Prion Strain
Distinct from That Causing Bovine Spongiform Encephalopathy" and is
authored by Vincent Beringue and 10 others. The abstract reads as follows:

"To date, bovine spongiform encephalopathy (BSE) and its human
counterpart, variant Creutzfeldt-Jakob disease, have been associated
with a single prion strain. This strain is characterized by a unique
and remarkably stable biochemical profile of abnormal
protease-resistant prion protein (PrP(res)) isolated from brains of
affected animals or humans. However, alternate PrP(res) signatures in
cattle have recently been discovered through large-scale screening.
To test whether these also represent separate prion strains, we
inoculated French cattle isolates characterized by a PrP(res) of
higher apparent molecular mass, called H-type, into transgenic mice
expressing bovine or ovine PrP. All mice developed neurological
symptoms and succumbed to these isolates, showing that these
represent a novel strain of infectious prions. Importantly, this
agent exhibited strain-specific features clearly distinct from that
of BSE agent inoculated to the same mice, which were retained on
further passage. Moreover, it also differed from all sheep scrapie
isolates passaged so far in ovine PrP-expressing mice. Our findings
therefore raise the possibility that either various prion strains may
exist in cattle, or that the BSE agent has undergone divergent
evolution in some animals."

The authors' synopsis of their paper reads as follows: Prions are
unconventional agents of proteic nature that are formed of abnormal
conformations of the host-encoded prion protein (PrP). They cause
fatal neurodegenerative diseases in both animals and humans and can
be transmitted between species, as exemplified in humans by the
emergence of variant Creutzfeldt-Jakob disease following the epidemic
of bovine spongiform encephalopathy (BSE) in the United Kingdom.
Since diagnosis of prion infection is only possible once the central
nervous system has been invaded, brains of slaughtered or fallen
cattle are routinely screened in Europe to protect the consumers from
BSE. This has unexpectedly led to the discovery of unprecedented PrP
conformations that were distinct from the single one associated so
far with BSE or BSE-related diseases. To precisely determine their
etiology, the authors have studied the transmissibility of these new
conformations, termed H-type, to transgenic mice expressing either
bovine or ovine PrP. They show that these cases are highly pathogenic
for these mice. The authors also demonstrate that they are not
directly related to the agent involved in the BSE epidemic,
supporting the view for isolation of a new prion strain from cattle,
whose prevalence and associated zoonotic risk should be carefully
monitored in the future."

--
Terry S. Singeltary Sr

[see also:
CJD (new var.) update 2006 (10) 20061002.2820
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 (05) 20050505.1243
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 (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/msp/jw


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