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From: TSS ()
Subject: CJD (NEW VAR.) UPDATE 2006 (12) ProMED
Date: December 5, 2006 at 6:02 pm PST

CJD (NEW VAR.) UPDATE 2006 (12)
*******************************
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 vCJD and CJD statistics, Mon 4 Dec 2006
[2] EUROCJD data as of 31 October 2006
[3] National (US) Prion Disease Pathology Surveillance Center Data: 8 Nov 2006
[4] The Netherlands: 2nd vCJD death
[5] USA: 3rd vCJD case (ex Saudi Arabia)

******
[1] UK: vCJD and CJD statistics
Date: Mon 4 Dec 2006
From: ProMED-mail
Source: UK Department of Health, Monthly Creutzfeldt-Jakob Disease
Statistics, Mon 4 Dec 2006 [edited]


The Department of Health is today [Mon 4 Dec 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 1 Dec 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 8 Jan 2007).

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

These data are 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, and 5 in
2005. The number of deaths due to definite or probable vCJD in the UK
during the first 11 months of 2006 remains at 5.

Totals for all types of CJD cases in the UK in 2005 and 2006
----------------------------------------------------
As of Fri 1 Dec 2006, in the UK in the year 2005, there were 123
referrals of suspected CJD, and there were 65 deaths from sporadic
CJD, 7 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 11 months of 2006 are:
98 referrals, 53 deaths from sporadic CJD, 5 from vCJD, 5 from
familial CJD, 3 from GSS and one from iatrogenic CJD.

During the period from 1995, when vCJD was first diagnosed, up to the
present there have been 953 deaths from all forms of CJD including
the 158 deaths attributable to definite or probable vCJD.

[These data are accessible via
]

--
ProMED-mail

******
[2] EU - EUROCJD Data
Date: Tue 31 Oct 2006
From: ProMED-mail
Source: EUROCJD 31 Oct 2006 [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
U.K. National CJD Surveillance Unit based in Edinburgh.

Current data as at November 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 / 3 (1*) / 2 / 0 / 0
Canada / 1 (0) / 1 / 0
Saudi Arabia / 1 (1) / 0 / 0
Japan / 1** (0) / 0 / 0
Netherlands*** / 2 (0) / 0 / 0
Portugal / 1 (1) / 0 / 0
Spain / 1 (0) / 0 / 0

Total / 197 (11) / - / 2

Footnotes
----------
* One case probably contracted in Saudi Arabia [see part (5) below],
and 2 in U.K.
** Residence in the UK for 24 days
*** Data for the Netherlands case (see part [4] below) was omitted in
error from the corresponding table for October posted on 31 Oct 2006

--
ProMED-mail

******
[3] USA - CJD National Prion Disease Surveillance Data
Date: Wed 8 Nov 2006
From: Terry S. Singeltary Sr
Source: CJD Surveillance, National Prion Disease Pathology
Surveillance Center, 8 Nov 2006 [edited]


Resources/Case Report
---------------------

Year / Referral / Total / Sporadic / Familial / Iatrogenic / vCJD

1997 / 104 / 60 / 54 / 6 / 0 / 0
1998 / 94 / 51 / 44 / 6 / 1 / 0
1999 / 114 / 74 / 65 / 9 / 0 / 0
2000 / 169 / 111 / 97 / 12 / 2 / 0
2001 / 2247 / 154 / 138 / 16 / 0 / 0
2002 / 265 / 151 / 127 / 22 / 1 / 0
2003 / 284 / 191 / 142 / 45 / 1 / 0
2004 / 360 / 202 / 167 / 21 / 0 / 1*
2005 / 357 / 201 / 144 / 39 / 0 / 0
2006 / 208 / 86 / 46 / 8 / 0 /1**

Total / 2202 / 1281 / 1024 / 184 / 5 / 2*

* Acquired in the United Kingdom
** Acquired in Saudi Arabia [see part (5) below]

[The gradual increase in sporadic and other forms of CJD in the USA
throughout this period may merely reflect improvements in
surveillance and diagnosis rather than a real increase in incidence.
For example, according to the tables above, there were approximately
1 per million cases of sporadic CJD in the USA during 2005, compared
with 2 cases per million in the UK during the same period. This
difference could be indicative of a real difference in incidence or a
difference in efficiency of diagnosis. - Mod.CP]

--
Terry S. Singeltary Sr

******
[4] The Netherlands - 2nd death
Date: Thu 16 Nov 2006
From: Mary Marshall
Source: iol.co.za Medical, Reuters report, Thu 16 Nov 2006 [edited]


A 2nd Dutch person has died from the human variant of mad cow disease
following the death of a woman last year, health authorities said on
Thu 16 Nov 2006. The Dutch Institute for Health and Environment
(RIVM) give no details about the victim, but Dutch television
stations said he was a 16-year-old boy.

A spokeswoman for the RIVM said the victim died from the
brain-wasting [variant] Creutzfeldt-Jakob Disease (vCJD), the human
form of Bovine Spongiform Encephalopathy (BSE) about 2 weeks ago. She
did not explain why the RIVM had waited 2 weeks to confirm the death,
which was reported in Dutch media on Thursday. The RIVM diagnosed the
person with the human variant of mad cow [disease] in June and said
at the time that the patient had most probably become infected by
eating contaminated meat products. It was the 2nd Dutch death from
the human variant of mad cow disease after a 26-year-old woman died
in May 2005.

The disease is fatal and incurable. It is thought to be caused by
eating food tainted with material from cattle with BSE, a progressive
neurological disorder. More than 150 cases of vCJD have been reported
around the world, mostly in Britain, but also in France, Ireland,
Italy, Japan, Canada and the United States. Mad cow disease first
emerged in Britain in the 1980s and has forced the destruction of
millions of cattle.

--
ProMED-mail

******
[5]
Date: Wed 29 Nov 2006
From: Terry S. Singeltary Sr.
Source: CDC, National Center for Infectious Diseases, Wed 29 Nov 2006 [edited]


Confirmed Case of Variant Creutzfeldt Jakob Disease (vCJD) in the
United States in a Patient from the Middle East
---------------------------------------------------
The Virginia Department of Health
[] and the Centers
for Disease Control and Prevention announced the recent confirmation
of a vCJD case in a U.S. resident. This is the 3rd vCJD case
identified in a U.S. resident. This latest U.S. case occurred in a
young adult who was born and raised in Saudi Arabia and has lived in
the United States since late 2005. The patient occasionally stayed in
the United States for up to 3 months at a time since 2001 and there
was a shorter visit in 1989. In late November 2006, the Clinical
Prion Research Team at the University of California San Francisco
Memory and Aging Center confirmed the vCJD clinical diagnosis by
pathologic study of adenoid and brain biopsy tissues. The 2
previously reported vCJD case-patients in U.S. residents were each
born and raised in the United Kingdom (U.K.), where they were
believed to have been infected by the agent responsible for their
disease. There is strong scient!
ific evidence that the agent causing vCJD is the same agent that
causes bovine spongiform encephalopathy (BSE, commonly known as mad
cow disease).

Variant CJD is a rare, degenerative, fatal brain disorder that
emerged in the United Kingdom in the mid-1990s. Although experience
with this new disease is limited, evidence to date indicates that
there has never been a case transmitted from person-to-person except
through blood transfusion. Instead, the disease is thought to result
primarily from consumption of cattle products contaminated with the
BSE agent. Although no cases of BSE in cattle have been reported in
Saudi Arabia, potentially contaminated cattle products from the
United Kingdom may have been exported to Saudi Arabia for many years
during the large U.K. BSE outbreak.

The current case-patient has no history of receipt of blood, a past
neurological procedure, or residing in or visiting countries of
Europe. Based on the patient's history, the occurrence of a
previously reported Saudi case of vCJD attributed to likely
consumption of BSE-contaminated cattle products in Saudi Arabia, and
the expected greater than 7 year incubation period for food-related
vCJD, this U.S. case-patient was most likely infected from
contaminated cattle products consumed as a child when living in Saudi
Arabia (1). The current patient has no history of donating blood and
the public health investigation has identified no risk of
transmission to U.S. residents from this patient.

As of November 2006, 200 vCJD patients were reported world-wide,
including 164 patients identified in the United Kingdom, 21 in
France, 4 in the Republic of Ireland, 3 in the United States
(including the present case-patient), 2 in the Netherlands and 1 each
in Canada, Italy, Japan, Portugal, Saudi Arabia and Spain. Of the 200
reported vCJD patients, all except 10 of them (including the present
case-patient) had resided either in the United Kingdom (170 cases)
for over 6 months during the 1980-1996 period of the large UK BSE
outbreak or alternatively in France (20 cases).

As reported in 2005 (1), the U.S. National Prion Disease Pathology
Surveillance Center at Case Western Reserve University confirmed the
diagnosis in the one previously identified case of vCJD in a Saudi
resident. He was hospitalized in Saudi Arabia and his brain biopsy
specimen was shipped to the United States for analysis. This earlier
vCJD case-patient was believed to have contracted his fatal disease
in Saudi Arabia (1).

Reference
---------
(1) Belay ED, Sejvar JJ, Shieh W-J, Wiersma ST, Zou W-Q, Gambetti P,
Hunter S, Maddox RA, Crockett L, Zaki SR, Schonberger LB. Variant
Creutzfeldt-Jakob disease death, United States. Emerg Infect Dis
2005, 11 (9):1351-1354.

--
Terry S. Singeltary Sr.

[There is inconsistency in the number of vCJD cases attributed to the
United States. The Virginia Department of Health/CDC report cited in
the above list 3 cases -- 2 cases probably contracted in the U.K. and
the young adult described above who was born and raised in Saudi
Arabia. The EUROCJD data for October 2006 in part (2) above also
lists 2 U.S. cases, both assumed to have been contracted in the
U.K. The U.S. National Prion Disease Pathology Surveillance Center
Data published on 8 Nov 2006 (part 3 above), on the other hand, list
only a single U.S. case believed to have been contracted in the U.K. - Mod.CP]

[see also:
CJD (new var.) update 2006 (11) 20061106.3190
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 (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/pg/mpp

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