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From: TSS ()
Subject: NATIONAL CJD SURVEILLANCE UNIT PUBLISHES 13TH ANNUAL REPORT
Date: November 7, 2005 at 6:31 am PST

Date: November 07, 2005 Time: 12:15
NATIONAL CJD SURVEILLANCE UNIT PUBLISHES 13TH ANNUAL REPORT

The Thirteenth Annual Report of the National Creutzfeldt-Jakob Disease Surveillance Unit was published today. The report looks back over the period from May 1990 when the Unit was set up to 31 December 2004. The report outlines the Unit's work in the clinical surveillance of sporadic, variant (vCJD) and iatrogenic CJD.

Also included in the report are details of a study on the potential risk factors for variant and sporadic CJD and the work of the National Care Team in arranging care and advice to the families of CJD patients.

The main issues highlighted are:

153 cases of definite or probable vCJD had been identified in the UK, with 148 deaths reported up to 31 December 2004. Analysis of the incidence of vCJD onsets and deaths from January 1994 to December 2004 provides statistically significant evidence that a peak in the incidence of vCJD has been passed.

The Transfusion Medicine Epidemiology Review had identified the first case of vCJD associated with blood transfusion in 2003 in which a blood recipient had developed symptoms of vCJD 6 1/2 years after receiving a transfusion of red cells donated 3 1/2 years before the donor developed symptoms of vCJD.

The incidence of vCJD across the UK continues to show a "North - South" difference, although slightly less than previously reported, with a higher incidence being maintained in the North of the UK. The underlying reason for this finding is not clear.

It remains the case that the only statistically significant geographic cluster of vCJD cases in the UK was in Leicestershire. This cluster was first identified in July 2000. All geographically associated cases of vCJD are considered for investigation according to a protocol which involves the NCJDSU, the Health Protection Agency, The Scottish Centre for Infection and Environmental Health (SCIEH), and local public health physicians.

There is no evidence of any difference between the UK and other European countries in the incidence of sporadic CJD, nor any significant variation in recorded mortality within the UK.

Notes for Editors

The NCJDSU is jointly funded by the Department of Health and the Scottish Executive Health Department.
The report is available on the NCJDSU website at
http://www.cjd.ed.ac.uk/

http://www.wired-gov.net/WGLaunch.aspx?ARTCL=35151&ALERT_TYPE=15

THIRTEENTH ANNUAL REPORT 2004

CREUTZFELDT-JAKOB DISEASE

SURVEILLANCE IN THE UK

The National CJD Surveillance Unit

Western General Hospital, Edinburgh, EH4 2XU

www.cjd.ed.ac.uk

Department of Infectious and Tropical Diseases

London School of Hygiene and Tropical Medicine

Keppel Street, London, WC1E 7HT

Thirteenth Annual Report 2004 1

Table of Contents

SECTION 1

Summary 3

SECTION 2

Clinical Surveillance

2.1 Referrals

2.2 Sporadic CJD

2.3 Variant CJD

2.4 Iatrogenic CJD

2.5 Transfusion Medicine Epidemiology Review

2.6 Study of Progressive Intellectual and Neurological Deterioration (PIND)

5

5

6

13

21

22

24

SECTION 3

Case-Control Study 25

SECTION 4

Laboratory Activities

4.1 Neuropathology – Statement of Progress

4.2 Surveillance and Workload during 2002

4.3 Protein Laboratory

4.4 Brain Banking Activities

4.5 Molecular Genetics

4.6 CSF 14-3-3 and other brain specific proteins

28

28

28

30

31

31

32

SECTION 5

National Care Team 35

SECTION 6

Publications 38

SECTION 7

Staff 41

Thirteenth Annual Report 2004 2

Section 1

SUMMARY

he 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 encephalopathies (TSEs). In September 2001 the National Care Team

was formed, which currently comprises a care coordinator and a secretary. The National Care

Team is based within the NCJDSU and was formed in response to concerns regarding the care of

CJD patients.

T

The information provided in this thirteenth report continues to provide evidence of a high level of

case ascertainment. The decrease in referrals is however potentially concerning from the point of

view of complete case ascertainment. The reason for this drop is unknown and the numbers will

need careful monitoring over 2005. It is particularly notable that the number of recorded sporadic

CJD deaths in 2004 is lower than in the three previous years, however, the death data for 2004

may be incomplete. Detailed clinical and epidemiological information has been obtained for the

great majority of patients. The case-control study for risk factors of CJD has continued

recruitment and initial analysis has been undertaken. The post mortem rate for patients with

suspected CJD is high, although there is ongoing evidence that this rate continues to decline, in

line with general autopsy rates in the UK. This is reflected in the reduced number of brain

specimens examined in the neuropathology laboratory this year. The reduction in sporadic CJD

numbers (32 in 2004, 52 in 2003) is another potential concern along with the fall in referral

numbers and sporadic CJD deaths noted above.

In 1990-2004 mortality rates from sporadic CJD in England, Wales, Scotland and Northern

Ireland were, respectively, 0.86, 1.05, 0.88 and 0.53/million/year. The difference between the rates

in each country is not statistically significant (p>0.2). These rates are comparable to those observed

in other countries in Europe and elsewhere in the world, including countries which are free of

BSE. There was some variation in the observed mortality rates between the different regions

within the UK but this variation is not statistically significant (p>0.2). The highest and lowest

mortality rates from sporadic CJD were observed in the South West (SMR=135) and Northern

Ireland (SMR=74).

Up to 31 December 2004, there have been 148 deaths from definite or probable variant CJD

(vCJD) in the UK. Of these, 106 were confirmed by neuropathology. A further 5 probable cases

were alive as at 31st December 2004. The clinical, neuropathological and epidemiological features

of these cases of vCJD are remarkably uniform and consistent with our previous descriptions.

Analysis of the incidence of vCJD onsets and deaths from January 1994 to December 2004

indicates that a peak has been passed. While this is an encouraging finding, incidence of vCJD

may increase again, particularly if different genetic subgroups are found to be affected. The

identification of disease-related PrP in the spleen of a blood recipient of PRNP-129 MVgenotype

Thirteenth Annual Report 2004 3

emphasises this point. In addition, this case along with the report of the appendix study suggests

at least a possibility of a greater number of preclinical or subclinical cases in the population than

might be indicated by the present numbers of confirmed cases.

Risk factors for the development of vCJD include age, residence in the UK and methionine

homozygosity at codon 129 of the prion protein gene - all 131 cases (86%) of vCJD with available

genetic analysis have been methionine homozygotes. The incidence of vCJD across the UK

continues to show a "North-South" difference (though slightly less than previously reported), with

a higher incidence being maintained in the North of the UK. The underlying reason for this

finding is not clear. The only statistically significant geographic cluster of vCJD cases in the UK

was in Leicestershire. All geographically associated cases of vCJD are considered for investigation

according to a protocol which involves the NCJDSU, colleagues at the HPA, HPS and local public

health physicians.

The activities of the NCJDSU are strengthened by collaboration in other surveillance projects,

including the Transfusion Medicine Epidemiology Review and the study of Progressive

Intellectual and Neurological Deterioration in Children. The collaboration of our colleagues in

these projects is greatly appreciated; the effectiveness of this collaboration allowed the

identification in 2003 of a case of variant CJD associated with blood transfusion and the

identification in 2004 of PrPres in the spleen of a blood recipient. The success of the National CJD

Surveillance Project continues to depend on the extraordinary level of co-operation from the

neuroscience community and other medical and paramedical staff throughout the UK. We are

particularly grateful to the relatives of patients for their help with this study.

Thirteenth Annual Report 2004 4

snip...

http://www.cjd.ed.ac.uk/report13.pdf

Interesting isn't it. when nvCJD goes up along with the sporadic CJDs, they try and downplay it with the old 'it's due to better surveillance', so along with the nvCJD, we will pick up more cases of sporadic CJDs, but it is still one-in-a-million. BUT, when the BSE cases in cattle start to go down, along with the nvCJD cases, and then we see a decline with sporadic CJDs, in countries where BSE regulations have been strictly enforced over decades, and a good human TSE surveillance effort, and then we look at the USA, where the BSE/TSE surveillance in humans and animals have been anything but 'sound science', it's been more like coporate/political TSE science. IN the USA sporadic CJD has trippled since 1997 ;

TSS
Animal Prion Diseases Relevant to Humans (unknown types?)
Thu Oct 27, 2005 12:05
71.248.128.109

About Human Prion Diseases /
Animal Prion Diseases Relevant to Humans

Bovine Spongiform Encephalopathy (BSE) is a prion disease of cattle. Since 1986, when BSE was recognized, over 180,000 cattle in the UK have developed the disease, and approximately one to three million are likely to have been infected with the BSE agent, most of which were slaughtered for human consumption before developing signs of the disease. The origin of the first case of BSE is unknown, but the epidemic was caused by the recycling of processed waste parts of cattle, some of which were infected with the BSE agent and given to other cattle in feed. Control measures have resulted in the consistent decline of the epidemic in the UK since 1992. Infected cattle and feed exported from the UK have resulted in smaller epidemics in other European countries, where control measures were applied later.

Compelling evidence indicates that BSE can be transmitted to humans through the consumption of prion contaminated meat. BSE-infected individuals eventually develop vCJD with an incubation time believed to be on average 10 years. As of November 2004, three cases of BSE have been reported in North America. One had been imported to Canada from the UK, one was grown in Canada, and one discovered in the USA but of Canadian origin. There has been only one case of vCJD reported in the USA, but the patient most likely acquired the disease in the United Kingdom. If current control measures intended to protect public and animal health are well enforced, the cattle epidemic should be largely under control and any remaining risk to humans through beef consumption should be very small. (For more details see Smith et al. British Medical Bulletin, 66: 185. 2003.)

Chronic Wasting Disease (CWD) is a prion disease of elk and deer, both free range and in captivity. CWD is endemic in areas of Colorado, Wyoming, and Nebraska, but new foci of this disease have been detected in Nebraska, South Dakota, New Mexico, Wisconsin, Mississippi Kansas, Oklahoma, Minnesota, Montana, and Canada. Since there are an estimated 22 million elk and deer in the USA and a large number of hunters who consume elk and deer meat, there is the possibility that CWD can be transmitted from elk and deer to humans. As of November 2004, the NPDPSC has examined 26 hunters with a suspected prion disease. However, all of them appeared to have either typical sporadic or familial forms of the disease. The NPDPSC coordinates with the Centers for Disease Control and state health departments to monitor cases from CWD-endemic areas. Furthermore, it is doing experimental research on CWD transmissibility using animal models. (For details see Sigurdson et al. British Medical Bulletin. 66: 199. 2003 and Belay et al. Emerging Infectious Diseases. 10(6): 977. 2004.)

http://www.cjdsurveillance.com/abouthpd-animal.html

SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM 1997 TO 2004. SPORADIC CJD CASES TRIPLED, and that is with a human TSE surveillance system that is terrible flawed. in 1997 cases of the _reported_ cases of cjd were at 54, to 163 _reported_ cases in 2004. see stats here;

p.s. please note the 47 PENDING CASES to Sept. 2005

p.s. please note the 2005 Prion D. total 120(8) 8=includes 51 type pending, 1 TYPE UNKNOWN ???

p.s. please note sporadic CJD 2002(1) 1=3 TYPE UNKNOWN???

p.s. please note 2004 prion disease (6) 6=7 TYPE UNKNOWN???

http://www.cjdsurveillance.com/resources-casereport.html

CWD TO HUMANS = sCJD ???

AS implied in the Inset 25 we must not _ASSUME_ that
transmission of BSE to other species will invariably
present pathology typical of a scrapie-like disease.

snip...

http://www.bseinquiry.gov.uk/files/yb/1991/01/04004001.pdf

ATYPICAL TSEs in USA CATTLE AND SHEEP ?

http://www.bseinquiry.gov.uk/files/sc/seac17/tab03.pdf


all animals for human/animal consumption must be TSE tested,

all human/animal TSE must be made reportable, of all ages and in every state.

IF, the theory of sporadic/spontaneous in about one per million were true, then we would see no drop in sporadic CJDs. something caused an increase, and something caused a decline. it's not a sporadic cause or a spontaneous cause, but a real cause$

TSS




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