A ProMED-mail post
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International Society for Infectious Diseases
[With the continuing decline of the number of cases in the human population
of variant Creutzfeldt-Jakob disease -- abbreviated previously as vCJD or
CJD (new var.) in ProMED-mail -- it has been decided to broaden the scope
of the occasional ProMED-mail updates to include other prion-related
diseases. In addition to vCJD, data on other forms of CJD: sporadic,
iatrogenic, familial, and GSS (Gerstmann-Straussler-Scheinker disease) are
included also since they may have some relevance to the incidence and
etiology of vCJD. - Mod.CP]
In this update:
[1] UK: National CJD Surveillance Unit -- monthly statistics as of Mon 9
Aug 2010 -- no new vCJD cases
[2] France: Institut de Veille Sanitaire -- monthly statistics as of Mon 26
Jul 2010 -- no new vCJD cases
[3] USA - data not updated since 30 Apr 2010, no indigenous vCJD
[4] vCJD statistical analysis, UK, 1994-2009
[5,6] Italy: 2nd vCJD case confirmed
******
[1] UK: National CJD Surveillance Unit -- monthly statistics as of Mon 9
Aug 2010 -- no new vCJD cases
Date: Mon 9 Aug 2010
Source: UK National CJD Surveillance Unit, monthly statistics [edited]
The number of deaths due to definite or probable vCJD cases remains 169. A
total of 4 definite/probable patients are still alive so that the total
number of definite or probable vCJD cases is still 173. So far, 2
fatalities have been recorded in 2010.
Although 3 new deaths due to vCJD were recorded in 2009 and 2 deaths so far
in 2010, the overall picture is still consistent with the view that the
vCJD outbreak in the UK is in decline [but see (4) below], albeit now with
a pronounced tail. The 1st cases were observed in 1995, and 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, 5 in 2007, one in 2008, 3 in
2009, and now 2 so far in 2010.
Totals for all types of CJD cases in the UK so far in 2010
----------------------------------------------------------
During the 1st 7 months or 2010, there have been 83 referrals, 29 fatal
cases of sporadic CJD, 2 fatal cases of vCJD, one case of iatrogenic CJD,
and none of familial CJD or GSS.
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[2] France: Institut de Veille Sanitaire -- monthly statistics as of 26 Jul
2010 -- no new vCJD cases
Date: Mon 26 Jul 2010
Source: IVS - Maladie de Creutzfeldt-Jakob et maladies apparentees [in
French, trans. & summ. Mod.CP, edited]
During the 1st 7 months of 2010, there were 840 referrals, 41 confirmed
cases of sporadic CJD, one case of familial CJD, and no cases of iatrogenic
CJD or vCJD.
A total of 25 cases of confirmed or probable vCJD has now been recorded in
France since records began in 1992. There was 1 case in 1996, 1 in 2000,
and 1 in 2001, there were 3 in 2002, 2 in 2004, 6 in 2005, 6 in 2006, 3 in
2007, 2 in 2009, and no cases have been recorded so far in 2010.
The 25 confirmed cases comprise 13 females and 12 males. All 25 are now
deceased. Their median age is 37 (between 19 and 58). 7 were resident in
the Ile-de-France and 18 in the provinces. All the identified cases have
been Met-Met homozygotes. No risk factor has been identified. One of the 25
had made frequent visits to the United Kingdom.
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[3] USA -- not updated since 30 Apr 2010, no indigenous vCJD
Date: Fri 30 Apr 2010
Source: US National Prion Disease Pathology Surveillance Center [edited]
During the 4 month period 1 Jan 2010 to 30 Apr 2010, there were 116
referrals, of which 62 were classified as prion disease, comprising 31
cases of sporadic CJD, 7 of familial CJD, and no cases of iatrogenic CJD or
vCJD. Since 1996 when records began there have been 3617 referrals, of
which 2117 were classified as prion disease, comprising 1781 cases of
sporadic CJD, 301 of familial CJD, 4 of iatrogenic CJD, and 3 of vCJD.
The 3 cases of VCJD were considered to have been contracted in the United
Kingdom or Saudi Arabia. The prion disease category includes 28 (24 in
2010) cases with type determination pending in which vCJD has been excluded.
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[4] vCJD statistical analysis, UK 1994-2009
Date 22 Jun 2010
Source: Health Protection Agency, Statistical Unit [edited]
Incidence of vCJD diagnoses and deaths in the UK: Jan 1994 - Dec 2009
----------------------------------------------------------------------
Summary: By the end of 2009 a total of 170 diagnoses of vCJD had been
reported in the UK, 166 of whom had died. An additional death occurred in
2009, which was not diagnosed until 2010. Within 2009 there were 3
diagnoses and 3 deaths (one of whom was diagnosed in 2010).
Results from modelling the underlying incidence of diagnoses and deaths
indicate that the epidemic reached a peak in the year 2000, when there were
27 diagnoses and 28 deaths, and has since declined to a current incidence
of about 1 diagnosis/death per year. Extrapolating the best fitting model
(the cubic model) gives an estimate of 1 death in the next 12 months (95
per cent prediction interval 0 to 3).
An analysis that looked at deaths by birth cohort (pre 1970, 1970s, 1980s)
showed that the shape of the epidemic differs between cohorts, mainly due
to the fact that deaths of individuals born in the 1980s were only seen
from 1999 onwards.
It is important to note that although a peak has been passed, it is
possible that there will be future peaks, possibly in other genetic groups.
There is also the possibility of ongoing person to person spread as seen
with 4 cases of transfusion association vCJD infection to date, who
received blood in 1999 or earlier from donors who were later diagnosed with
clinical vCJD. 3 of these individuals developed vCJD (one diagnosed in 2003
and 2 in 2006), whilst the 4th died from causes unrelated to vCJD, but was
found on postmortem examination to have abnormal prion protein present in
the spleen and a lymph node.
[byline: N J Andrews, Senior Statistician, Statistics Unit, Centre for
Infections, Health Protection Agency]
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[Interested readers should consult the original text, which includes
graphical presentation of the data analysed according to different
statistical models. - Mod.CP]
******
[5] Italy - 2nd case confirmed
Date: Thu 22 Jul 2010
Source: Agence France-Presse [edited]
Another human mad cow disease case reported in Italy
----------------------------------------------------
A 42 year old Italian woman was reported as the 2nd ever case of mad cow
disease in humans in the country and is currently hospitalised in a
desperate condition, the ANSA news agency said on Wednesday [21 Jul 2010].
The woman was diagnosed with a variant of the Creutzfeldt-Jakob disease
(vCJD) at a Milan neurological hospital in the past months and was then
transferred to a hospital in Livorno, in western Tuscany, when she was
already in a coma, ANSA said.
It is still unclear how the woman contracted the disease. In 2009, Italy's
health ministry had reported the woman's disease as a "likely variant of
the CJD". The only previous case of mad cow disease in humans in Italy was
reported in 2002 on the island of Sicily.
On Friday [16 Jul 2010] the European Union said it had nearly wiped out mad
cow disease in animals in Europe. Only 67 positive cases of mad cow disease
were identified last year [2009] and the animals were old cows that could
have been contaminated long ago, the EU said. In the 1990s, panic erupted
after Britain reported a link between mad cow disease and a new form of
Creutzfeldt-Jakob disease in humans.
******
[6] Italy: 2nd case confirmed
Date: Sun 25 Jul 2010
Source: NowItaly.com [edited]
Another Italian case of human "mad cow" disease
-----------------------------------------------
Italy has got its 2nd case of the human variant of mad cow disease, a
Livorno hospice reported Wednesday [21 Jul 2010]. The 42 year old woman
from Livorno was reported to be in the final stages of her battle against
variant Creutzfeldt-Jakob disease (vCJD), the human form of the fatal
brain-wasting illness (CJD). It is not known how the woman contracted the
disease. Italy's 1st case, a Sicilian woman, died in 2003 after struggling
with vCJD for about a year. The variant [form] of CJD, for which there is
no known cure, is believed to be caused by eating meat infected with mad
cow disease or BSE (bovine spongiform encephalopathy).
Since variant CJD was 1st reported in 1996, a total of 217 patients from 11
countries have been identified [for full details see
reported from the following countries: 170 from the United Kingdom, 25 from
France, 5 from Spain, 4 from Ireland, 3 from the United States, 3 in the
Netherlands, 22 in Portugal and Italy, and one each from Canada, Japan, and
Saudi Arabia. 2 of the 3 US cases, 2 of the 4 cases from Ireland and the
single cases from Canada and Japan were likely exposed to the BSE agent
while residing in the United Kingdom. One of the 25 French cases may also
have been infected in the United Kingdom.
There has never been a case of vCJD that did not have a history of exposure
within a country where the cattle disease was occurring. In 3 cases, each
reported in the United Kingdom, contamination is thought to have come
through receipt of blood from an asymptomatic, infected donor.
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[Previously the Italian Health Ministry on Fri 23 Oct 2009 reported a
"likely" case of [variant] Creutzfeldt-Jakob Disease (vCJD), the human form
of mad cow disease (see ProMED-mail report archived as: "vCJD - Italy:
susp. 20091024.3671"). It is presumed that the 42 year old woman described
in the above 2 reports is this previously suspected case. The Ministry of
Health stated at that time that it believed that this suspected case had
been infected before the use of animal and bone meal in animal feed was
banned in Italy December 2000.
The interactive HealthMap/ProMED map for Italy is available at
[see also:
Prion disease update 2010 (06) 20100706.2248
Prion disease update 2010 (05) 2010 20100507.1488
Prion disease update 2010 (04) 2010 20100405.1091
Prion disease update 2010 (03) 20100304.0709
Prion disease update 2010 (02) 2010 20100205.0386
Prion disease update 2010 20100107.0076
2009
---
Prion disease update 2009 (10) 20091103.3784
vCJD - Italy: susp. 20091024.3671
Prion disease update 2009 (09) 20091005.3461
Prion disease update 2009 (08) 20090908.3170
Prion disease update 2009 (07) 20090806.2783
Prion disease update 2009 (06) 20090706.2433
Prion disease update 2009 (05) 20090602.2054
Prion disease update 2009 (04) 20090406.1337
vCJD, 5th death - Spain (Cantabria) 20090307.0953
Prion disease update 2009 (03) 20090305.0918
Prion disease update 2009 (02) 20090202.0463
Prion disease update 2009 (01) 20090108.0076
2008
---
Prion disease update 2008 (14): new vCJD wave imminent? 20081218.3980
Prion disease update 2008 (13) 20081201.3780
Prion disease update 2008 (12) 20081103.345
Prion disease update 2008 (11) 20081006.3159
vCJD, mother & son - Spain: (Leon) 20080926.3051
Prion disease update 2008 (10) 20080902.2742
vCJD - Spain: susp. 20080410.1311
Prion disease update 2008 (05) 20080408.1285
Prion disease update 2008 (01): correction 20080104.0046
Prion disease update 2008 (01) 20080102.0014
2007
---
Prion disease update 2007 (08) 20071205.3923
Prion disease update 2007 (07) 20071105.3602
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]
............sb/cp/ejp/sh
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