Title: National CJD Research and Surveillance Unit
Funder: Department of Health and Social Care

Abstract: Background: CJD has an importance greater than its rarity might suggest; being potentially transmissible from person-to-person and with one form, vCJD, being a zoonotic illness (due to BSE in cattle). Particular concerns include: the magnitude of the primary (diet-related) human vCJD epidemic; the magnitude and significance of subclinical BSE/vCJD infection in the human population; the risk of secondary vCJD transmission through blood, blood products, dentistry, surgery or transplantation; the risks associated with (non vCJD) iatrogenic disease (eg related to human growth hormone or neurosurgery); the possible emergence of new clicico-pathological phenotypes of prion disease (eg related to BSE infection of non-MM PRNP individuals). There are many uncertainties surrounding these concerns and accurate human population disease data critically underpin decisions about public health protection. Aims: To continue UK CJD surveillance: identifying cases of CJD and obtaining appropriate clinical, pathological & epidemiological data relevant to the concerns listed above. Research Plan/Methods: The Unit’s methodology is described elsewhere in this document. However, in brief, we investigate suspect cases of CJD, including visits to patients & families throughout the UK and collect relevant clinical & epidemiological data. Pathological confirmation of the diagnosis is undertaken whenever possible. Cases are classified according to established criteria and disease trends analysed in conjunction with our stored historical data. There are a number of separately funded studies that dovetail with this core research-including the TMER study of possible blood transmission of vCJD; the Haemophilia study; the paediatric PIND study and collaborative studies with the Roslin Institute including correlations with their animal transmission studies. Research Team: The Lead Applicant & Co-Applicant details are given elsewhere. Other Team members include: two sub-consultant grade clinical neurologists, an Operations Director, a Study Data Co-ordinator, a Molecular Geneticist, Neuropathology, protein & CSF laboratory staff, administrative & secretarial support. The Unit has been established since 1990, within the University of Edinburgh. Impact/Influence: The Unit’s identification of vCJD and the first instance of blood transfusion-related vCJD have had obvious impacts on public health policy. Our data have underpinned the considerations of various expert advisory committees (including SEAC, ACDP & SaBTO) and various DH risk assessments. Continuing study will have importance in informing future assessments and policy decisions. Both positive and negative findings (such as further or no further cases of blood-related vCJD) will be important for future policy decisions; negative findings have secure meaning only if good surveillance is in place.

Lay abstract: The National CJD Research and Surveillance Unit (established 1990) will continue to collect information on UK CJD (Creutzfeldt-Jakob Disease). We obtain the patient’s clinical records, their diagnostic tests (such as brain scans), and visit them and their families whenever possible. CJD is rare (1-2 deaths/million/ year) with no simple diagnostic test in life. We have a lot of CJD experience and provide advice to local clinicians; we also provide laboratory testing (for spinal fluid samples in life, and autopsy tissues) that aids or confirms diagnosis. With records of CJD over more than 2 decades, we are able to see if there are any changes in how common the illness is or how it affects people. CJD exists in four main forms (variant CJD-related to BSE in cattle, sporadic CJD-a spontaneous disease of middle/late life, genetic CJD-inherited/familial and iatrogenic CJD-accidentally via surgery, blood or certain medical treatments). Because of the concerns relating to BSE infection through food, and iatrogenic CJD (including the risk of blood transfusion transmission), accurate population disease information is vital for public health policy. We are an established Unit with proven methods of identifying this illness in conjunction with UK clinicians and pathologists and well-established links with international CJD surveillance systems and research groups. We have a good track record of disseminating our data and results to the medical/scientific community, the public and UK public heath bodies. We are a WHO (World Health Organisation) CJD Reference Centre, and the variant CJD Reporting Hub for ECDC (European Centre for Disease Control), also chairing the EUROCJD (EU & other countries) CJD collaborative group.

Research activity codes: 2.4 SURVEILLANCE AND DISTRIBUTION
Health categories: NEUROLOGICAL