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SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

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SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Draft minutes of the 100th meeting held on 25th April 2008

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ITEM 3 - CURRENT ISSUES

9.

SEAC was informed about the following issues:

. Three cases of variant CJD (vCJD) had been identified in Spain: one each in 2005, 2007 and 2008, with the last two cases reported from the same geographical region. Media reports in Spain had suggested there could be up to five further cases. One of these five cases is a young individual with clinical symptoms of a relatively long duration that had been classified by the Spanish Registry as possible sporadic CJD (sCJD). Although it is possible that this case may be subsequently confirmed as vCJD there were good reasons, which could not be discussed at the present time, for thinking it was not. Four other cases were not considered to be vCJD by TSE experts in Spain. More information would be available as investigations progress.

http://www.seac.gov.uk/papers/101-1.pdf

3 10:10 Current issues

vCJD cluster in Spain Testing of a goat

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In the afternoon of the 15th of October 2008, SEAC will discuss preliminary research on tissues of the eye from a vCJD case and preliminary research on a new human prion disease in the United States of America (as reported by Gambetti et al. (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann. Neurol. 63, 697-708). This part of the meeting will be held in closed session to allow discussion of preliminary unpublished data. This is in accordance with the SEAC Code of Practice.

* SEAC 101/4 and SEAC 101/5 and Annex 1 of SEAC 101/2 have not been provided as they contain either draft reports or unpublished data. This is in accordance with the SEAC Code of Practice.

http://www.seac.gov.uk/agenda/agen151008.htm

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SEAC 99th meeting on Friday 14th December 2007

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SPONGIFORM ENCEPHALOPATHY ADVISORY COMMITTEE Minutes of the 99th meeting held on 14th December 2007

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ITEM 8 - PUBLIC QUESTION AND ANSWER SESSION 40. The Chair explained that the purpose of the question and answer session was to give members of the public an opportunity to ask questions related to the work of SEAC. Mr Terry Singeltary (Texas, USA) had submitted a question prior to the meeting, asking: "With the Nor-98 now documented in five different states so far in the USA in 2007, and with the two atypical BSE H-base cases in Texas and Alabama, with both scrapie and chronic 14 © SEAC 2007 wasting disease (CWD) running rampant in the USA, is there any concern from SEAC with the rise of sporadic CJD in the USA from ''unknown phenotype'', and what concerns if any, in relations to blood donations, surgery, optical, and dental treatment, do you have with these unknown atypical phenotypes in both humans and animals in the USA? Does it concern SEAC, or is it of no concern to SEAC? Should it concern USA animal and human health officials?" 41.

A member considered that this question appeared to be primarily related to possible links between animal and human TSEs in the USA.

There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA. Current evidence does not suggest that CWD is a significant risk to human health. There are unpublished data from a case of human TSE in the USA that are suggestive of an apparently novel form of prion disease with distinct molecular characteristics. However, it is unclear whether the case had been further characterised, if it could be linked to animal TSEs or if other similar cases had been found in the USA or elsewhere. In relation to the possible public health implications of atypical scrapie, H-type BSE and CWD, research was being conducted to investigate possible links and surveillance was in place to detect any changes in human TSEs. Although possible links between these diseases and human TSEs are of concern and require research, there is no evidence to suggest immediate public health action is warranted. The possible human health risks from classical scrapie had been discussed earlier in the meeting. Members noted that there are effective channels of discussion and collaboration on research between USA and European groups. Members agreed it is important to keep a watching brief on new developments on TSEs.

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http://www.seac.gov.uk/minutes/99.pdf

There is no evidence that sCJD is increasing in the USA and no evidence of any direct link between TSEs and CJD in the USA.

10 people killed by new CJD-like disease

Public release date: 9-Jul-2008 [ Print Article E-mail Article Close Window ]

Contact: Claire Bowles mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:[email protected] 44-207-611-1210 New Scientist

10 people killed by new CJD-like disease A NEW form of fatal dementia has been discovered in 16 Americans, 10 of whom have already died of the condition. It resembles Creutzfeldt-Jakob disease - with patients gradually losing their ability to think, speak and move - but has features that make it distinct from known forms of CJD.

No one yet knows how the disease originates, or under what conditions it might spread. Nor is it clear how many people have the condition. "I believe the disease has been around for many years, unnoticed," says Pierluigi Gambetti, director of the US National Prion Disease Pathology Surveillance Center at Case Western Reserve University in Cleveland, Ohio. Cases may previously have been mistaken for other forms of dementia.

Since Gambetti's team wrote a paper describing an initial 11 cases referred to his centre between 2002 and 2006 (Annals of Neurology, vol 63, p 697), another five have come to light. "So it is possible that it could be just the tip of the iceberg," Gambetti says.

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http://www.eurekalert.org/pub_releases/2008-07/ns-tpk070908.php

Thursday, July 10, 2008 A Novel Human Disease with Abnormal Prion Protein Sensitive to Protease update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/novel-human-disease-with-abnormal-prion.html

Thursday, July 10, 2008 A New Prionopathy update July 10, 2008 http://cjdmadcowbaseoct2007.blogspot.com/2008/07/new-prionopathy-update-july-10-2008.html

Communicated by: Terry S. Singeltary Sr. <mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000076/!x-usc:mailto:[email protected]>

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]

http://pro-med.blogspot.com/2007/11/proahedr-prion-disease-update-2007-07.html

http://www.promedmail.org/pls/askus/f?p=2400:1001:6833194127530602005::NO::F2400_P1001_BACK_PAGE,F2400_P1001_PUB_MAIL_ID:1010,39963

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/1006-4240t1.htm

http://www.fda.gov/ohrms/dockets/ac/06/transcripts/2006-4240t1.pdf


http://seac992007.blogspot.com/

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http://www.seac.gov.uk/minutes/99.pdf

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SEAC 101st meeting on Wednesday 15th October 2008 AGENDA

http://seac992007.blogspot.com/2008/10/seac-101st-meeting-on-wednesday-15th.html


Wednesday, October 08, 2008

Idiopathic Brainstem Neuronal Chromatolysis (IBNC): a novel prion protein related disorder of cattle?

http://bse-atypical.blogspot.com/2008/10/idiopathic-brainstem-neuronal.html



Tuesday, August 19, 2008

Atypical BSE (BASE) Transmitted from Asymptomatic Aging Cattle to a Primate

http://bse-atypical.blogspot.com/2008/08/atypical-bse-base-transmitted-from.html




Wednesday, October 08, 2008

CJD FOUNDATION UPDATE

http://creutzfeldt-jakob-disease.blogspot.com/2008/10/cjd-foundation-update.html



2008

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

http://www.cjdfoundation.org/fact.html



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