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CJD

Volume 478: debated on Monday 23 June 2008

To ask the Secretary of State for Health what research his Department has commissioned or evaluated on (a) the origins, (b) a cure and (c) the potential incubation period of variant Creutzfeldt-Jakob disease. (211954)

In 1990 the Department established the National Surveillance Creutzfeldt-Jakob Disease Unit (NCJDSU), which we continue to fund. The NCJDSU monitors all cases of Creutzfeldt-Jakob disease (CJD) in the United Kingdom in order to investigate each case by clinical examination, clinical investigations, neuropathological examination, genetic analysis, molecular biological studies, collecting basic epidemiological data and carrying out a case-control study to:

provide accurate data on the incidence of CJD, including variant CJD (vCJD);

investigate risk factors for CJD, including vCJD;

identify the mechanism of transmission of bovine spongiform encephalopathy (BSE) to the human population;

provide estimates of short-term and long-term trends in the rate of occurrence of vCJD;

evaluate the potential risks of onward transmission of vCJD, including through iatrogenic routes;

identify any novel forms of human spongiform encephalopathy;

evaluate case definitions of CJD, including vCJD; and

evaluate diagnostic tests for CJD, including vCJD.

The NCJDSU's annual reports which include evaluations of the origin and potential incubation period of vCJD are available at

www.cjd.ed.ac.uk.

The Department also has a ring-fenced budget for commissioning variant Creutzfeldt-Jakob disease research. The funded programme of current studies and projects, including those studies which are complete and await formal closure, includes the following of relevance to origins, cure and incubation period:

Project title

Assessing the Feasibility of accessing dental case records for detailed dental histories in CJD cases and controls

Conditional expression of PrP in the gut of transgenic mice to investigate the uptake of infectivity in the gut in the transmissible spongiform encephalopathies (TSEs)

Examination of the potential for transmission of vCJD through dentistry

Investigating dental treatment as a possible risk factor for vCJD

Investigation into transmissibility of TSEs via blood

National Prion monitoring cohort

Predicting future numbers of cases of vCJD

Retrospective review of CJD and related disorders

Surveillance for asymptomatic Prion infection in primary immunodeficiency patients exposed to UK sourced immunoglobulin

The effect of Leucodepletion on transmission of BSE by transfusion of sheep blood components

To undertake prospective multisource surveillance for all cases of progressive intellectual and neurological deterioration (PIND) occurring in children in the UK

Transfusion medicine epidemiology review (TMER)

vCJD tissue infectivity studies

Investigation of Pentosan Polysulphate as a potential prophylactic agent against the transmission of vCJD by blood products

Prion 1 clinical trial: Quinacrine for human Prion disease

Strategies to inhibit neurodegeneration in Prion disease

The development of an effective treatment for Prion infection of humans.

Past studies commissioned by the Department are included in the Medical Research Council's (MRCs) UK TSE Portfolio which is available at:

www.mrc.ac.uk/OurResearch/ResearchPortfolios/TSE/UKTSEPortfolio/index.htm

which contains details of TSE research supported by the major UK public funders: the Department for Environment, Food and Rural Affairs, the Department of Health, the Food Standards Agency, the MRC and the Biotechnology and Biological Sciences Research Council.

As a joint funder of the Spongiform Encephalopathy Advisory Committee (SEAC) the Department has considered the studies set out in the list of scientific papers supplied to SEAC available at

www.seac.gov.uk/papers/references.htm

To ask the Secretary of State for Health how many cases of variant Creutzfeldt-Jakob disease were diagnosed in (a) humans and (b) animals in each of the last five years; and what the identified cause of infection was in each case. (211956)

Figures provided by the National Creutzfeldt-Jakob Disease Surveillance Unit show the following number of patients received a diagnosis of variant Creutzfeldt-Jakob disease (vCJD) in the last five years:

Number

2003

16

2004

8

2005

6

2006

6

2007

1

2008 (to 16 June)

1

In three cases, one in 2003 and two in 2006, the diagnosed patients are thought to have been infected by blood transfusions from donors who later went on to develop vCJD. In all other cases the presumed route of infection is thought to be dietary.

There are no cases of vCJD in animals.