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PRION DISEASE UPDATE 2007 (07)

******************************

A ProMED-mail post

<http://www.promedmail.org>

ProMED-mail is a program of the

International Society for Infectious Diseases

<http://www.isid.org>


[Note: With continuing decline of the number of cases of variant

Creutzfeldt-Jacob disease (abbreviated previously as vCJD or CJD (new

var.) in ProMED-mail) in the human population, it has been decided to

broaden the scope of the occasional ProMED-mail reports to include

other prion-related diseases. These updates supersede the previous

update thread.


The definitions of the designations deaths, definite cases, probable

vCJD cases, can be found by accessing the Department of Health website

(<http://www.dh.gov.uk/en/index.htm>)

or by reference to a previous ProMED-mail post in the vCJD thread.


Data on vCJD cases from any part of the world are now included in

these updates where appropriate, and other forms of CJD (sporadic,

iatrogenic, familial, and GSS Gerstmann-Straussler-Scheinker disease)

are included also when they have some relevance to the incidence and

etiology of vCJD.


In addition, prion-related diseases of domesticated and free-living

animals may also be included if relevant. - Mod.CP]


In this update:


[1] UK: National CJD Surveillance Unit - Monthly statistics

[2] USA: National Prion Disease Pathology Surveillance Center


******

[1] UK National CJD Surveillance Unit - Monthly statistics

Date: Mon 5 Nov 2007

Source: UK National CJD Surveillance Unit, Monthly Statistics, 2007 [edited]

<http://www.cjd.ed.ac.uk/figures.htm>



Monthly Creutzfeldt-Jakob disease statistics - as of 2 Nov 2007


[N.B. The Department of Health ceased issuing monthly CJD press

notices at the end of September 2007 because the same data are also

published by the National CJD Surveillance Unit in Edinburgh at

<http://www.cjd.ed.ac.uk/figures.htm>.]


These following figures show the number of suspect cases of CJD

referred to the CJD surveillance unit in Edinburgh and the number of

deaths of definite and probable 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 vCJD cases in the UK as of 2 Nov 2007

----------------------


Summary of vCJD cases -- deaths

----------------------

Deaths from definite vCJD (confirmed): 114

Deaths from probable vCJD (without neuropathological confirmation): 47

Deaths from probable vCJD (neuropathological confirmation pending): 1

Number of deaths from definite or probable vCJD (as above): 162


Summary of vCJD cases -- alive

----------------------

Number of probable vCJD cases still alive: 4


Total

-----

Number of definite or probable vCJD (dead and alive): 166


These data indicate that there have been no new cases diagnosed

during the past month, but one patient has died, raising the vCJD

death toll to 162.


These data are still consistent with the view that the vCJD outbreak

in the UK is in decline. The peak number of deaths was 28 in the year

2000, followed by 20 in 2001, 17 in 2002, 18 in 2003, 9 in 2004, 5 in

2005, 5 in 2006, and so far 4 in 2007.


Totals for all types of CJD cases in the year 2007 so far

-----------------------------

As of 2 Nov 2007 in the UK in the year 2007, so far there have been

93 referrals, 38 deaths from sporadic CJD, 2 deaths from iatrogenic

CJD, 2 deaths from familial CJD, one from GSS, and 4 deaths from vCJD.


--

Communicated by:

ProMED-mail


******

[2] USA: National Prion Disease Pathology Surveillance Center

Date: June 2007

Source: National Prion Disease Pathology Surveillance Center (USA) [edited]

<http://www.cjdsurveillance.com/pdf/case-table.pdf>



CJD Cases examined

----------------------

Year / Referrals / Prion disease / Sporadic / Familial / Iatrogenic / vCJD


1996 / 42 / 32 / 26 / 4 / 0 / 0

1997 / 115 / 68 / 57 / 9 / 0 / 0

1998 / 93 / 53 / 45 / 7 / 1 / 0

1999 / 114 / 69 / 61 / 8 / 0 / 0

2000 / 151 / 103 / 89 / 14 / 0 / 0

2001 / 208 / 116 / 106 / 9 / 0 / 0

2002 / 255 / 143 / 118 / 23 / 2 / 0

2003 / 272 / 174 / 132 / 41 / 0 / 0

2004 / 334 / 183 / 157 / 21 / 0 / 1*

2005 / 352 / 195 / 152 / 37 / 1 / 0

2006 / 372 / 186 / 143 / 30 / 0 / 1**

2007 / 120 / 68 / 35 / 7 / 0 / 0

TOTAL / 2428*** / 1390**** / 1121 / 210 / 4 / 2


*Acquired in UK

** Acquired in Saudi Arabia

*** Includes 17 inconclusive and 9 pending (1 from 2006, 8 from 2007.

**** Includes 17 non-vCJD type unknown (2 from 1996, 2 from 1997, 1

from 2001, 1 from 2003, 4 from 2004, 3 from 2005, 4 from 2006) and 36

type pending (2 from 2005, 8 from 2006, 26 from 2007).


Notes:


-- Cases are listed based on the year of death when available. If the

year of death is not available, the year of sample receipt is used.


-- Referrals: Cases with possible or probable prion disease from

which brain tissue or blood in the case of familial disease were submitted.


-- Inconclusive: Cases in which the samples were not sufficient to

make a diagnosis.


-- Non-vCJD type unknown are cases in which the tissue submitted was

adequate to establish the presence but not the type; in all cases,

vCJD could be excluded.


--

Communicated by:

Terry S. Singeltary Sr.


[In submitting these data, Terry S. Singeltary Sr. draws attention to

the steady increase in the "type unknown" category, which, according

to their definition, comprises cases in which vCJD could be excluded.

The total of 26 cases for the current year (2007) is disturbing,

possibly symptomatic of the circulation of novel agents.

Characterization of these agents should be given a high priority. - Mod.CP]


[see also:

Prion disease update 2007 (06) 20071003.3269

Prion disease update 2007 (05) 20070901.2879

Prion disease update 2007 (04) 20070806.2560

Prion disease update 2007 (03) 20070702.2112

Prion disease update 2007 (02) 20070604.1812

Prion disease update 2007 20070514.1542

CJD (new var.) update 2007 (05) 20070403.1130

CJD (new var.) update 2007 (04) 20070305.0780

CJD (new var.) update 2007 (03) 20070205.0455

CJD (new var.) update 2007 (02): South Korea, susp 20070115.0199

2006

----

CJD (new var.), blood transfusion risk 20061208.3468

CJD, transmission risk - Canada (ON) 20061207.3457

CJD (new var.) update 2006 (12) 20061205.3431

CJD (new var.) update 2006 (11) 20061106.3190

CJD (new var.) update 2006 (10) 20061002.2820

CJD (new var.) - Netherlands: 2nd case 20060623.1741

CJD (new var.) - UK: 3rd transfusion-related case 20060209.0432

CJD (new var.) update 2006 (02) 20060206.0386

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 (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 (10) 20040909.2518

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/msp/dk


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