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From: TSS ()
Subject: 94th SEAC meeting held on 21 September 2006 Summary
Date: September 26, 2006 at 8:56 am PST

1

© SEAC 2006

NINETY FOURTH MEETING OF THE SPONGIFORM

ENCEPHALOPATHY ADVISORY COMMITTEE

The Spongiform Encephalopathy Advisory Committee held its 94th

meeting in Belfast on 21st September 2006, and discussed the

following:

CURRENT ISSUES

SEAC was informed about the following issues:

• Publication of a report by the Scientific Committee on

Emerging and Newly Identified Health Risks1 following a

public consultation. The report had been revised in light of

comments from SEAC and other committees.

• An interim report from the Implementation Review Group

concluding that the bovine spongiform encephalopathy (BSE)

testing system for cattle aged over thirty months had worked

satisfactorily since its introduction. The report is available on

the Food Standards Agency (FSA) website2.

• The FSA referred attendees to the FSA website concerning an

investigation into an alleged breach of Over Thirty Months

BSE testing controls3.

• The Clinical Governance Advisory Group, convened to advise

the Department of Health (DH) on appropriate arrangements

and care for individuals ‘at risk from variant Creutzfeldt-Jakob

disease (vCJD) for public health purposes’, will meet on the

1European Commission Scientific Committee on Emerging and Newly Identified

Health Risks (2006) Safety of Human-derived Products with regard to vCJD

http://ec.europa.eu/health/ph_risk/committees/04_scenihr/scenihr_cons_02_en.htm

2http://www.food.gov.uk/aboutus/ourboard/boardmeetings/boardmeetings2006/board

meeting130706/agenda13jul06

3http://www.food.gov.uk/news/newsarchive/2006/aug/srmupdate0708

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© SEAC 2006

10th October 2006 to finalise its report. The report is

anticipated to be released in November 2006.

• The membership and draft terms of reference for an expert

group to be convened by the Health Protection Agency (HPA)

to consider the most effective means, including post-mortem

testing, of ascertaining the prevalence, age and genotype

distribution of vCJD infection in the United Kingdom (UK), as

recommended by SEAC4. The committee’s comments on the

scope of expertise on the group and the draft terms of

reference would be referred to the HPA.

UPDATE ON TSE TESTING

SEAC was presented with statistics showing the annual number of

transmissible spongiform encephalopathy (TSE) cases from

surveillance of cattle, sheep and goats in Northern Ireland since

2000. The data showed a decline in the incidence of BSE. Few

or, in some years, no TSE positives in sheep had been found.

CJD UPDATE

SEAC was updated on the latest figures on the number of sporadic

CJD (sCJD) and vCJD cases up to September 2006. From May

1990, 845 cases of sCJD had been identified in the UK with a

mean age at death of 67 years and genotype distribution of 64%

MM, 18% MV and 18% VV at codon 129 of the prion protein gene.

One hundred and sixty two cases of vCJD had been identified in

the UK with a median age of death of 28 years. Statistical analysis

showed the incidence of vCJD peaked with 28 deaths in 2000.

Elsewhere, 34 vCJD cases had been reported with a distribution of

20 in France, four in the Republic of Ireland, two in the United

States of America (USA), two in the Netherlands and single cases

in Italy, Canada, Saudi Arabia, Japan, Spain and Portugal. In two

Irish cases, both USA cases, one French case, the Japanese and

Canadian cases, infection was presumed to have occurred in the

UK.

All vCJD cases that had been genotyped were MM at codon 129 of

the prion protein gene. However, findings from studies of

4 http://www.seac.gov.uk/statements/state260106.htm

3

© SEAC 2006

humanised mice and genotyping of samples testing positive in a

retrospective survey of abnormal prion protein in appendix and

tonsil tissue suggested that cases of vCJD infection, and possibly

clinical vCJD, in genotypes other than MM could be expected.

Epidemiological studies on the relationships between recipients or

donors of blood from or to vCJD cases were reviewed.

REPUBLIC OF IRELAND PERSPECTIVE OF TSEs

SEAC was provided with an overview of the surveillance,

epidemiology and management of BSE and vCJD in the Republic

of Ireland.

HORIZON SCANNING

The committee was informed by representatives from the

Department of Environment, Food and Rural Affairs, FSA, DH and

the National CJD Surveillance Unit about issues that may require

future consideration by SEAC including:

• proposals arising from the European Commission’s TSE

Roadmap such as possible changes to specific risk material

(SRM) controls and tolerance levels for fishmeal in ruminant

feed.

• aspects of the contingency policy if BSE were found in the

national sheep flock.

• ongoing research on the animal and possible human health

implications of atypical scrapie.

• the DH programme to ascertain better the prevalence of vCJD

infection in the UK.

• protocols for the evaluation of diagnostic tests and

decontamination technologies for vCJD.

• possible re-evaluation of risk assessments of secondary vCJD

transmission should they be modified in light of new data.

PUBLIC QUESTION AND ANSWER SESSION

Answers were given to questions from the public about the

implications of historic exposure to bone meal in non-agricultural

fertiliser, the appropriate transport of SRM and the timing of

possible changes to restrictions on bovine vertebral column.

4

© SEAC 2006

EVALUATION CRITERIA FOR ANTE MORTEM DIAGNOSTIC

TESTS FOR SUBCLINICAL vCJD

DH requested SEAC advice on evaluation criteria for rapid ante

mortem diagnostic tests to detect subclinical vCJD.

SEAC noted that current prototype tests were all based on

detection of abnormal prion protein. However, the relationship

between the presence of abnormal prion protein and vCJD

infectivity is not well understood.

SEAC recommended the independent evaluation and validation of

diagnostic tests prior to implementation. Preliminary evaluation of

the specificity and sensitivity of tests could be achieved by using

blood spiked with brain material from vCJD cases. Blood from

animal models may also provide a useful source of test material.

However it is very important that final evaluation also includes

testing of blood from vCJD cases, as spiked blood may not be

closely representative of endogenous infectivity in blood. SEAC

suggested a number of risk assessments need to be carried out in

advance of testing to ascertain what sensitivity and specificity is

needed. It is critical that appropriate arrangements are made for

collecting suitable material, storing and managing it. It is also of

utmost importance that the ethical implications of ante mortem

testing for vCJD are addressed prior to implementation of a blood

test. SEAC agreed to produce a statement of its deliberations.

http://www.seac.gov.uk/summaries/seac94_summary.pdf

TOTAL CASES OF SPORADIC CJD (DEATHS)
DEFINITE AND PROBABLE CASES

http://www.eurocjd.ed.ac.uk/sporadic.htm


TOTAL CASES OF FAMILIAL/GENETIC CJD AND IATROGENIC CJD DEATHS TO 30 JUNE 2006

http://www.eurocjd.ed.ac.uk/genetic.htm


SEE STEADY INCREASE IN SPORADIC CJD IN THE USA FROM
1997 TO 2006. SPORADIC CJD CASES TRIPLED, with phenotype
of 'UNKNOWN' strain growing. ...


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


TSS




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