December

France - Institut de Veille Sanitaire - as of 30 Dec 2008
30 Dec 2008
IVS - Maladie de Creutzfeldt-Jakob et maladies apparentees [French, trans. & summ. Mod.CP, edited] [Promed]
During the period 1992 to 2008, there were 23 cases of vCJD, all now deceased. They occurred between 1996 and 2007: one case in 1996, one in 2000, one in 2001, 3 in 2002, none in 2003, 2 in 2004, 6 in 2005, 6 in 2006, 3 in 2007, and none so far in 2008. There were 12 male and 11 female patients.
Their ages at time of death ranged from 19 to 58 years (mean 39); 6 of the patients resided in the Ile-de-France [Paris area] and 17 in the provinces. All the cases were met-met homozygotes for codon 129 of the prion protein gene. No special risk factors were evident, which distinguished these patients from those with other forms of CJD (sporadic, genetic, iatrogenic). However, one patient had visited the UK at regular intervals.
Totals for all types of CJD cases in the year 2008
As of 30 Dec 2008 in France, during the course of 2008 there have been 1438 referrals, 76 deaths from sporadic CJD, 3 deaths from iatrogenic CJD, 8 from familial CJD, none from GSS, and none from vCJD.

United Kingdom - Fears raised over new vCJD wave
18 Dec 2008
BBC Newsnight [edited] [Promed]
According to this story, doctors fear that a new wave of the human form of "mad cow disease" is about to hit Britain, as a new case had been diagnosed on a clinical basis as vCJD. It is reported that what is of concern to doctors in the new case is that the individual concerned has a particular genetic make-up and it is the first case to appear of that type.

United Kingdom - National CJD Surveillance Unit
1 Dec 2008 [edited][Promed]
UK National CJD Surveillance Unit, monthly statistics <http://www.cjd.ed.ac.uk/figures.htm>
The number of suspect cases of vCJD 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), remain unchanged since the previous monthly report; that is, the number of definite or probable vCJD cases (dead and alive) remains 167.
This situation is consistent with the view that the vCJD outbreak in the UK is in decline. The first 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, and only one so far (up to the end of November) in 2008.
Totals for all types of CJD cases in the year 2008
As of 1 Dec 2008 in the UK, so far there have been 124 referrals, 71 deaths from sporadic CJD, 5 deaths from iatrogenic CJD, 3 from GSS, 2 from familial CJD, and one from vCJD.

November

United Kingdom - National CJD Surveillance Unit
3 Nov 2008
UK National CJD Surveillance Unit , monthly statistics [edited] [Promed]
The number of suspect cases of vCJD 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), remain unchanged since the previous monthly report; that is, the number of definite or probable vCJD cases (dead and alive) remains 167.
This situation is consistent with the view that the vCJD outbreak in the UK is in decline. The first 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, and only one so far (up to the end of October) in 2008.
Totals for all types of CJD cases in the year 2008 As of 3 Nov 2008 in the UK, so far there have been 114 referrals, 62 deaths from sporadic CJD, 4 deaths from iatrogenic CJD, 3 from GSS, 2 from familial CJD, and one from vCJD.
Related stories
13 Nov 2008 - National CJD Surveillance Unit - 16th Annual Report 2008
UK National CJD surveillance Unit, 16th Annual Report [abbreviated, edited] [Promed]
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 spongiform encephalopathies (TSEs). In September 2001, the National Care Team was formed, which currently comprises 2 care coordinators and a secretary. It is based within the NCJDSU and was formed in response to concerns regarding the care of CJD patients.

October

France - Institut de Veille Sanitaire
30 Oct 2008
IVS - Maladie de Creutzfeldt-Jakob et maladies apparentees [French, trans. & summ. Mod.CP, edited] [Promed]
During the period 1992 to 2008, there were 23 cases of vCJD, all now deceased. They occurred between 1996 and 2007: one case in 1996, one in 2000, one in 2001, 3 in 2002, none in 2003, 2 in 2004, 6 in 2005, 6 in 2006, 3 in 2007, and none so far in 2008. There were 12 male and 11 female patients.
Their ages at time of death ranged from 19 to 58 years (mean 39); 6 of the patients resided in the Ile-de-France [Paris area] and 17 in the provinces.
All the cases were met-met homozygotes for codon 129 of the prion protein gene. No special risk factors were evident, which distinguished these patients from those with other forms of CJD (sporadic, genetic, iatrogenic). However, one patient had visited the UK at regular intervals.
Totals for all types of CJD cases in the year 2008
As of 30 Oct 2008 in France, so far in 2008 there have been 1230 referrals,
56 deaths from sporadic CJD, 3 deaths from iatrogenic CJD, 7 from familial
CJD, none from GSS, and none from vCJD.

United Kingdom - Proposals to reduce BSE testing of cattle slaughtered for food
15 Oct 2008
Food Standards Agency [edited][iFSN]
The Spongiform Encephalopathy Advisory Committee (SEAC) has been estimating the impact to human health of actioning proposals to reduce BSE testing of cattle slaughtered for food. This will be discussed at the FSA Board meeting today.
The following statement was issued by SEAC today, before the FSA Board meeting:
SEAC considered the results from a mathematical model that had been used to estimate the number of infected cattle that may be undetected as result of raising the minimum age at which healthy slaughtered and fallen stock cattle must be tested for BSE. The model itself, produced by the Veterinary Laboratories Agency (VLA), was previously reviewed by SEAC1.
The increased risks to human health estimated by the model from raising the age at which healthy slaughtered cattle are tested for BSE (up to 60 months, the highest age modeled) are very small. The model estimates that much less than one BSE case would be missed annually in the Great Britain herd by increasing the age of testing to 60 months for the healthy slaughter surveillance stream. Although uncertainties are inherent in such modeling, the validation of the model that has been conducted provides assurances about the reliability of the results. Similar results from a different analysis by the European Food Safety Authority (EFSA)2,3 provide additional confidence in the findings. The EFSA analysis estimates that less than two BSE cases would be missed annually in the whole of the EU154 by increasing the age of testing to 60 months for the healthy slaughter surveillance stream. These risk assessments hold provided the incidence of BSE in cattle remains low.
Therefore, regulations should not be modified unless effective surveillance remains in place. Surveillance is the only means of monitoring changes in the incidence and prevalence of BSE, the effectiveness of control measures in preventing an epidemic and the possible emergence of new prion diseases. As control measures to prevent cattle and human infection are modified, continued active and passive surveillance become increasingly important to ensure that the remaining controls are effective in minimising the risk to human and animal health.

United Kingdom - National CJD Surveillance Unit - monthly statistics as of Mon
6 Oct 2008
UK National CJD Surveillance Unit, monthly statistics [edited] [Promed]
The number of suspect cases of vCJD referred to the CJD surveillance unit in Edinburgh and the number of deaths of definite and probable variant Creutzfeldt-Jakob disease, the form of the disease thought to be linked to BSE (bovine spongiform encephalopathy), remain unchanged since the previous monthly report; that is, the number of definite or probable vCJD cases (dead and alive) remains 167.
This situation is consistent with the view that the vCJD outbreak in the UK is in decline. 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, and only one so far in 2008.
Totals for all types of CJD cases in the year 2008
As of 6 Oct 2008 in the UK, so far there have been 99 referrals, 59 deaths from sporadic CJD, 4 deaths from iatrogenic CJD, 3 from GSS, one from familial CJD, and one from vCJD.

September

France - Institut de Veille Sanitaire - monthly report as of 30 Sep 2008
30 Sep 2008
IVS - Maladie de Creutzfeldt-Jakob et maladies apparentees
[French, trans. & summ. Mod.CP, edited] [Promed]
During the period 1992 to 2008, there were 23 cases of vCJD, all now deceased. They occurred between 1996 and 2007: one case in 1996, one in 2000, one in 2001, 3 in 2002, none in 2003, 2 in 2004, 6 in 2005, 6 in 2006, 3 in 2007, and none so far in 2008. There were 12 male and 11 female patients.
Their ages at time of death ranged from 19 to 58 years (mean 39); 6 of the patients resided in the Ile-de-France [Paris area] and 17 in the provinces. All the cases were met-met homozygotes for codon 129 of the prion protein gene. No special risk factors were evident, which distinguished these patients from those with other forms of CJD (sporadic, genetic, iatrogenic). However, one patient had visited the UK at regular intervals.
Totals for all types of CJD cases in the year 2008
As of 30 Sep 2008 in France, so far in 2008 there have been 1077 referrals, 41 deaths from sporadic CJD, 2 deaths from iatrogenic CJD, 5 from familial CJD, none from GSS, and none from vCJD.

Spain gets fourth mad cow disease death
25 Sep 2008
International Business Times [edited][iFSN]
According to this story, a woman in her 60s has died from the human version of mad cow disease, reported Spain's Ministry of Health. It is reported that the health researchers said on Wednesday tests confirm that the woman, 64, who died in August in northern Castilla-Leon region had variant Creutzfeltd-Jakob disease (CJD) linked to the Bovine Spongiform Encephalopathy (BSE) or mad cow disease, following the death of her 41-year-old son in April of the same disease, according to reports. Spain recorded its fourth fatality from the disease since 2005, and marks the first time that two members of the same family died of the disease, government officials said. Spain 2001 began taking preventative measures against the disease in 2001.
Related stories
25 Sep 2008 – Mad cow disease kills mother and son
CNN.com/Health [edited] [Promed]
According to this story, it is believed to be the first case in the world where 2 members of the same family have died from mad cow disease. It is reported that researchers will try to determine whether the mother and son shared a genetic structure that might have been more prone to contracting the illness.

United Kingdom - National CJD Surveillance Unit - monthly statistics as of 1 Sep 2008
1 Sep 2008
UK National CJD Surveillance Unit, monthly statistics [edited] [Promed]
The number of suspect cases of vCJD 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) remain unchanged since the previous monthly report; -- that is, the number of definite or probable vCJD cases (dead and
alive) remains 167.
This situation is consistent with the view that the vCJD outbreak in the UK is in decline. 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, and only one so far in 2008.
Totals for all types of CJD cases in the year 2008
As of 1 Sep 2008 in the UK, so far there have been 88 referrals, 47 deaths
from sporadic CJD, 4 deaths from iatrogenic CJD, 3 from GSS, one from
familial CJD, and one from vCJD.

France: Institut de Veille Sanitaire - monthly report as of 1 Sep 2008
1 Sep 2008
IVS - Maladie de Creutzfeldt-Jakob et maladies apparentees [French, trans./summ. Mod.CP, edited] [Promed]
During the period 1992 to 2008, there were 23 cases of vCJD, all now deceased. They occurred between 1996 and 2007: one case in 1996, one in 2000, one in 2001, 3 in 2002, none in 2003, 2 in 2004, 6 in 2005, 6 in 2006, 3 in 2007, and none so far in 2008. There were 12 male and 11 female patients.
Their ages at time of death ranged from 19 to 58 years (mean 39); 6 of the patients resided in the Ile-de-France [Paris area] and 17 in the provinces. All the cases were met-met homozygotes for codon 129 of the prion protein gene. No special risk factors were evident, which distinguished these patients from those with other forms of CJD (sporadic, genetic, iatrogenic). However, one patient had visited the UK at regular intervals.
Totals for all types of CJD cases in the year 2008
As of 1 Sep 2008 in France, so far in 2008 there have been 940 referrals, 37 deaths from sporadic CJD, 2 deaths from iatrogenic CJD, 5 from familial CJD, none from GSS, and none from vCJD.

August

United Kingdom - National CJD Surveillance Unit -- monthly statistics as of 4 Aug 2008
4 Aug 2008
UK National CJD Surveillance Unit, monthly statistics [edited] [Promed]
The number of suspect cases of vCJ 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) remain unchanged since the previous monthly report; i.e., the number of definite or probable vCJD cases (dead and alive) remains 167.
This situation is consistent with the view that the vCJD outbreak in the UK is in decline. 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, and only one so far in 2008.
Totals for all types of CJD cases in the year 2008
As of 4 Aug 2008 in the UK, so far there have been 78 referrals, 44 deaths from sporadic CJD, 4 deaths from iatrogenic CJD, 3 from GSS, one from familial CJD, and one from vCJD.

June

France - Institut de Veille Sanitaire -- monthly report as of 30 Jun 2008
30 Jun 2008
Institut de Veille Sanitaire -- Maladie de Creutzfeldt-Jakob et maladies apparentees [in French, trans. & summ. Mod.CP, edited] [Promed]
This document has not been updated for the month of July 2008. The data are repeated here to allow comparison with the corresponding data from the UK (above) and the USA (below). During the period 1992 to 2008 there were 23 cases of vCJD, all now deceased. They occurred between 1996 and 2007: one case in 1996, one in 2000, one in 2001, 3 in 2002, none in 2003, 2 in 2004, 6 in 2005, 6 in 2006, 3 in 2007, and none so far in 2008. There were 12 male and 11 female patients.
Their ages at time of death ranged from 19 to 58 years (mean 39); 6 of the patients resided in the Ile-de-France [Paris area] and 17 in the provinces. All the cases were met-met homozygotes for codon 129 of the prion protein gene. No special risk factors were evident, which distinguished these patients from those with other forms of CJD (sporadic, genetic, iatrogenic). However one patient had visited the
UK at regular intervals.
Totals for all types of CJD cases in the year 2008
As of 30 Jun 2008 in France, so far in 2008 there have been 722 referrals, 26 deaths from sporadic CJD, one death from iatrogenic CJD, 3 from familial CJD, none from GSS, and none from vCJD.

May

Netherlands - New case of mad cow disease discovered in Netherlands - 85th case since 1997
21 May 2008
AllHeadlinesNews (AHN) [edited] [Promed]
A fresh case of mad cow disease has been discovered in northern Netherlands in the Drenthe province, it was reported. Radio Netherlands Worldwide in its report said that an 8-year-old cow fell ill in May [2008] in Drenthe and had difficulty in walking. Veterinarians eventually killed the animal and an autopsy was conducted. During examination it was found the cow was infected with BSE.This is the 3rd case of mad cow [disease] in the Netherlands in 2008 and the 85th since 1997.

Finland - Call to minimise BSE testing
12 May 2008
YLE News [edited]
According to this story, the Finnish Food Safety Authority wants to cut down mad cow disease testing in Finland. It is reported that the Director General of the Finnish Food Safety Authority said that testing for bovine spongiform encephalopathy, or BSE, could be scaled back without risks to human health as the only mad cow disease case to date in Finland was in 2001.
Related stories
29 May 2008 - Finland's risk of mad cow disease minuscule
YLE (Finnish Broadcasting Company) [edited] [Promed]
It is reported in this story that the World Organisation for Animal Health (OIE) says Finland is free of bovine spongiform encephalopathy (BSE). The organisation made the announcement at its annual general meeting in Paris on Tuesday [27 May 2008]. The declaration means the risk of mad cow disease in Finland is minuscule. According to the director of the Finnish Food Safety Authority, the announcement offers Finland an edge in exporting meat outside the EU. She adds that Finland may be able to cut back on testing of the disease due to the declaration. One case of mad cow disease was detected in Finland in 2001. After the incident, Finland began widespread testing for the disease. According to thi story, currently, tests are carried out on all slaughtered animals over the age of 2.5, as well as on all cattle less than two years that are ill or butchered due to emergency situations.

United Kingdom - National CJD Surveillance Unit - monthly statistics as of 2 May 2008
2 May 2008
UK National CJD Surveillance Unit, monthly statistics [edited]
Monthly Creutzfeldt-Jakob disease statistics as of 2 May 2008
These following figures show the number of suspect cases of CJ referred to the CJDsurveillance unit in Edinburgh and the number of deaths of definite and probable variant
Creutzfeldt-Jakob disease, the form of the disease thought to be linked to BSE (bovine spongiform encephalopathy).
Definite and probable vCJD cases in the UK as of 7 Apr 2008
Summary of vCJD cases – deaths
Deaths from definite vCJD (confirmed): 115
Deaths from probable vCJD (without neuropathological confirmation): 48
Deaths from probable vCJD (neuropathological confirmation pending): 0
Number of deaths from definite or probable vCJD (as above): 163
Summary of vCJD cases – alive
Number of probable vCJD cases still alive: 3
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, and the number of patients alive is unchanged.
These data are consistent with the view that the vCJD outbreak in the UK is in decline (although the incidence curve may be developing a tail). 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, and so far none in 2008.
Totals for all types of CJD cases in the year 2008
As of 2 May 2008 in the UK, so far there have been 42 referrals, 23 deaths from sporadic CJD, 2 deaths from iatrogenic CJD, none from familial CJD, 2 from GSS, and none from vCJD.

April

France - Institut de veille sanitaire - Monthly report as of 30 Apr 2008
30 Apr 2008
Institut de veille sanitaire - Maladie de Creutzfeldt-Jakob et maladies apparentees [translated, abbreviated and edited - Mod.Promed]
This document lists the numbers of confirmed and probable cases of all forms of CJD recorded in France from 1992 t0 2008. The original text should be consulted for a full breakdown of the figures. During that period there were 23 cases of vCJD, all now deceased. They occurred between 1996 and 2007: one case in 1996, one case in 2000, one case in 2001, 3 cases in 2002, none in 2003, 2 cases in 2004, 6 cases in 2005, 6 cases in 2006, 3 cases 2007 and none so far in 2008. There were 12 male and 11 female patients. Their ages at time of death ranged from 19 to 58 years (mean 39). All the cases were met-met homozygotes for codon 129 of the prion protein gene. No special risk factors were evident which distinguished these patients from those with other forms of CJD (sporadic, genetic, iatrogenic). However one patient had visited the UK at regular intervals.
Totals for all types of CJD cases in the year 2008
As of 30 Apr 2008 in France, so far in 2008 there have been 524 referrals, 10 deaths from sporadic CJD, one death from iatrogenic CJD, one from familial CJD, none from GSS, and none from vCJD.

United Kingdom- National CJD Surveillance Unit -- monthly statistics
07 Apr 2008
UK National CJD Surveillance Unit, monthly statistics [edited]
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 the form of the disease thought to be linked to BSE (bovine spongiform encephalopathy).
Summary of vCJD cases – deaths
Deaths from definite vCJD (confirmed): 115
Deaths from probable vCJD (without neuropathological confirmation): 48
Deaths from probable vCJD (neuropathological confirmation pending): 0
Number of deaths from definite or probable vCJD (as above): 163
Summary of vCJD cases – alive
Number of probable vCJD cases still alive: 3
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, and the number of patients alive is unchanged.
Totals for all types of CJD cases in the year 2008
As of 7 Apr 2008 in the UK, so far there have been 33 referrals, 13 deaths from sporadic CJD, one death from iatrogenic CJD, none from familial CJD, 2 from GSS, and none from vCJD.

January

United Kingdom - National CJD Surveillance Unit -- Monthly statistics & 2007 totals
7 Jan 2008
UK National CJD Surveillance Unit, monthly statistics, 2007 [edited] Monthly Creutzfeldt-Jakob disease statistics -- as of 7 Jan 2008
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 7 Jan 2008 Summary of vCJD cases – deaths Deaths from definite vCJD (confirmed): 114 Deaths from probable vCJD (without neuropathological confirmation): 48
Deaths from probable vCJD (neuropathological confirmation pending): 1
Number of deaths from definite or probable vCJD (as above): 163
Summary of vCJD cases – alive Number of probable vCJD cases still alive: 3
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 the number of patients alive has decreased by one. These data are still consistent with the view that the vCJD outbreak in the UK is in decline (although the incidence curve may be developing a tail).
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 5 in 2007.
Totals for all types of CJD cases in the year 2007
As of 31 Dec 2007 in the UK in the year 2007, there were 111 referrals, 47 deaths from sporadic CJD, 2 deaths from iatrogenic CJD, 4 deaths from familial CJD, one from GSS, and 5 deaths from vCJD.

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