The National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU)

The incidence of Creutzfeldt-Jakob disease (CJD) is monitored in the UK by the National CJD Surveillance Unit (NCJDSU) based at the Western General Hospital in Edinburgh, Scotland. The Unit brings together a team of clinical neurologists, neuropathologists and scientists specialising in the investigation of this disease. This document is intended to summarise the research in progress at the NCJDSU and also provide some background information about CJD and other human spongiform encephalopathies. We have also provided some links to other resources and contrary points of view available on the Web.

Creutzfeldt-Jakob Disease Surveillance.

• Figures for the number of CJD cases and referrals of suspected cases of CJD to the NCJDSU since 1990.
• NCJDSU protocol for CJD surveillance across the UK.
• National Referral System. From July 2004, a new national reporting system was announced by the Chief Medical Officer.  This is centred on the National CJD Reporting Form to be faxed, by the notifying clinician, to the National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU), the National Prion Clinic (NPC) and the local CCDC. 
• Interim guidance on incidents involving inherited prion disease - this document sets out how inherited prion disease incidents should be reported to the CJD Incidents Panel and complements the guidance on local reporting by clinicians of CJD cases to public health departments (above).
• Text and figures of the latest annual report of the NCJDSU (published 3rd November 2009).   
• Archive of previous NCJDSU annual reports (2001-2007).
• National CJD Surveillance Unit Scientific Report 2007/08 (published 13th November 2008).
• Reporting CJD cases to public health departments - Guidance Document. - (updated November 2006)
• Potential treatments for Creutzfeldt-Jakob disease (updated July 2006).

Information on variant CJD.

• Reproduction of the complete Lancet article published by the NCJDSU in April 1996.
• Text of a letter written by Dr Robert Will to every Neurologist in the UK. This letter describes in some detail the clinical and pathological variants observed between sporadic CJD and the new variant of the disease which has been identified here at the NCJDSU.
• The original statement issued by SEAC, the government's advisory committee on spongiform encephalopathies, about these ten cases of the new variant of CJD.
• Protocol for the investigation of geographically associated cases of variant CJD
• Yearly Report entitled "Incidence of variant Creutzfeldt-Jakob disease deaths in the UK", compiled by N J Andrews at the Statistics Unit, Centre for Infections, Health Protection Agency.(updated 28h January 2009)
• Figures for the number of vCJD cases worldwide [data courtesy of the European and Allied Countries Study Group of CJD (EUROCJD/NEUROCJD)]

Care and Support.

• Reproduction of report entitled Patients with new variant Creutzfeldt-Jakob disease and their families: care and information needs by Dr Margaret Douglas, Dr Harry Campbell and Professor Robert Will.
• About the National Care-Cordinators (updated December 2004).
• CJD Support Network. The CJD Support Network was set up in Autumn 1995 by relatives of people who have died of CJD with backing from the Alzheimer's Disease Society.  Since 2003 the CJD Support Network became an independent charity and offers practical and emotional support to families and professionals affected by all strains of CJD.
• Human BSE Foundation. The Foundation offers support to victims of Human BSE, their families and friends.
• CJD Advice Network
• Guidance for Councils with Social Care Responsibilities (See Section XI)

Practical information about CJD.

• The different types of CJD are described.
• The clinical aspects of this disorder are described. (updated July 2003)
• Possible investigations undertaken in cases of CJD are described here. (Updated 15/10/02)
• The neuropathology of CJD as observed in the CJD surveillance unit laboratory.
• The National CJD Surveillance Unit Brain Bank
• A Glossary of medical and scientific terms relevant to CJD.

Creutzfeldt-Jakob Disease research.

• A review of work on image analysis in the investigation of CJD neuropathology.
• Complete reference list of scientific research articles produced by the CJD surveillance unit since 1990.

Other links

• The European and Allied Countries Collaborative Study Group of CJD (EUROCJD/NEUROCJD). Information and results.
• Transfusion Medicine Epidemiology Review (TMER).  Collaborative project between the UK NCJDSU and the NHS Blood and Transplant.  Information and results from this project whose main purpose is to investigate whether there is any evidence that CJD or vCJD may have been transmitted via the blood supply.  (updated May 2009)

• NEUROPRION is a network of excellence funded by the European Union via the 6th Framework programme in the priority 5 "Food Quality and Safety".

• Scottish TSE Network  The aim of the STN is to provide a forum for the exchange of ideas, results, reagents and expertise in research, diagnosis and clinical studies of TSE diseases in Scotland.
• Transfusion Guidelines Joint UKBTS/NIBSC Professional Advisory Committee Position Statement (Creutzfeldt-Jakob disease) prepared by the vCJD Working Party of the Standing Advisory Committee on Transfusion Transmitted Infections.
• Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection.  Guidance from the Advisory Committe on Dangerous Pathogens and the Spongiform Encephalopathy Advisory Committee.  (updated June 2003 - updates and revisions since June 2003 are clearly indicated in the document index)
• Department of Health CJD Policy Unit
• CJD Incidents Panel containing information about the Panel and relevant documents and links, including the new framework document and the surgical incident reporting form.
• vCJD Trust - website set up by Charles Russell Solicitors containing information on the Compensation Scheme and how to claim
• The Association of British Neurologists
• The National Prion Clinic at the National Hospital for Neurology and Neurosurgery, Queen Square, London.
• PRION-1 trial  this website provides details of a randomised trial of quinacrine in human prion disease.  Further details can be found here.
• MRC New Therapies Scrutiny Group for Prion Disease (NTSG) provides an independent source of advice on research into the development of potential therapeutics or preventative agents for prion disease.
• Food Standards Agency is a new government department set up in April 2000 to protect people's health and the interests of consumers in relation to food.
• The European Food Safety Authority (EFSA)  has been delivering risk assessments and scientific opinions on BSE/TSE since 2003.  Information on BSE/TSE can chiefly be found in the subsections under the headings BIOHAZ Panel and TSE Assessments.
• TSE Reagent Resource Centre. The TSE Resource Centre, directly supported by the BBSRC and MRC, was established in 1999 to collect, store, characterise, produce and distribute a range of reagents, from monoclonal antibodies to teaching aids.
• CJD Resource Centre  provides standard reagents to researchers working on CJD.
• CJD Alliance is an independently run organisation involved in TSE's such as CJD - its key interests are in early diagnosis and treatment issues.
• USA - American CJD Foundation  The Foundation seeks to promote the research, education and awareness of CJD, as well as to reach out to people who have lost loved ones to this illness.
• Information on CJD and BSE in the USA - Centers for Disease Control and Prevention, Atlanta, Georgia
• USA - National Prion Disease Pathology Surveillance Center  (from the Division of Neuropathology, Case Western Reserve University.  CJD surveillance system in collaboration with CDC).
• USA - CDC's website on infection control and TSEs
• USA - FDA website describing the ruminant feed ban introduced in the US as a result of the BSE outbreak in Europe.
• Information on BSE and CJD provided by the British Medical Journal.
• The World Health Organisation have provided information on BSE and the possible risks to humans.
• Research Report on Transmissible Spongiform Encephalopathies from the Biotechnology and Biological Sciences Research Council.
• Blood Recall/Withdrawal - Creutzfeldt-Jakob disease (CJD). An e-mail information/support group for blood product recall/withdrawal notification recipients and people affected by blood donor bans.
• Position statement on BSE from the Institute of Food Science & Technology.
• The Yahoo Index for CJD information on the WWW.

About ourselves

• The people involved in the research being undertaken at the CJD unit.
• The setup, details of the funding of the CJD and the collaborative projects we are involved in.
• Our address should you wish further contact.

For enquiries contact: