National CJD Surveillance Unit
| Abbreviation | NCJDRSU |
|---|---|
| Founded | May 1990 |
| Defunct | 31 March 2025 |
| Type | Research institute and surveillance unit |
| Headquarters | |
| Location |
|
Directors |
|
| Affiliations | UK Department of Health Scottish Executive Department of Health |
| Website | www |
The National CJD Surveillance Unit (later renamed the National Creutzfeldt-Jakob Disease Research and Surveillance Unit) was a specialist clinical research centre in the United Kingdom, founded in May 1990 to monitor changes in Creutzfeldt–Jakob disease (CJD) that could be related to Bovine spongiform encephalopathy (BSE).[1]
The unit's research identified Variant Creutzfeldt–Jakob disease (vCJD) in 1996, and causally linked the variant to BSE. During the United Kingdom BSE outbreak, the unit recorded 178 deaths due to vCJD.[2] Its funding from the National Institute for Health and Care Research and Scottish Executive Department of Health ceased in 2025.[3]
History
[edit]Foundation and identification of vCJD
[edit]From 1980–1984, the Medical Research Council (MRC) funded a Creutzfeldt–Jakob disease (CJD) surveillance project, which covered England and Wales, based at the University of Oxford. It collected data from cases of CJD over a 15 year period from 1970.[3][4] CJD is a generally a sporadic, and invariably fatal, neurological disease thought to be caused by misfolded proteins in the brain (prions), which affects 1–2 per million people in the UK each year.[5]
The National CJD Surveillance Unit was established in May 1990, after the outbreak of BSE in the United Kingdom, following a recommendation in the Southwood Committee Report of the Working Party on Bovine Spongiform Encephalopathy (BSE) which stated that any changes in BSE which could result in human infection should be monitored.[6]
In June 1995, Stephen Churchill, a 19-year old, died of CJD. Later that year, two others died from the disease. These cases were seen as atypical due to the younger age of the patients (CJD typically affects peopled aged 55 years and older).[7]
James Ironside, who worked as a pathologist at the National CJD Surveillance Unit, analysed the brains of these patients and found an atypical form of spongiform change, which he named variant CJD (vCJD).[7][8]
From 1997, the unit also studied the likelihood of vCJD being transferred by blood transfusion, and found 3 confirmed or probable cases in which this had occurred.[9][10] It would later give evidence at the infected blood inquiry in 2022.
The National CJD Surveillance Centre supplied urine samples to the Royal London Hospital in 1998 and 1999, who were funded by the MRC to trial the work of Dr Harash Narang, a controversial microbiologist who claimed to have developed a non-invasive urine test for BSE and CJD.[11][12] Later that year, the National CJD Research & Surveillance Unit became a WHO Collaborative Centre on the surveillance, diagnosis and epidemiology of human transmissible spongiform encephalopathies (TSEs).
Later research
[edit]In January 2002, Ironside replaced Robert G. Will (who had led the unit since its foundation in 1990) as director, as part of a three-year rotation.[13] Richard Knight was appointed deputy director,[13] and would later serve as director until its dissolution in 2025.
In May 2004, research from the centre modelled that up to hundreds of thousands of people could potentially carry the infectious agent that caused vCJD.[14] The unit was later based at University of Edinburgh from 2014 to 2017, where it conducted three studies on the epidemiology of the disease.[15]
In January 2024, Knight confirmed the rise in overall CJD cases; saying: "In the United Kingdom, the annual mortality rate from sporadic CJD rose from approximately 1.1 to 1.9 per million from 2007 to 2020. The current rate in the UK is 2.2 per million."[16]
Funding for the unit ceased on 31 March 2025.[3] A National CJD Diagnostic Advisory Service existing for the following 12 months, providing diagnostic advice and guidance to clinicians about possible CJD cases, until 31 March 2026.[3]
References
[edit]- ↑ "From BSE to CJD and what we were told at the time". The Guardian. 27 October 2000. ISSN 0261-3077. Retrieved 28 August 2026.
- ↑ "Creutzfeldt-Jakob disease in the UK (By Calendar Year)" (PDF). National CJD Research and Surveillance Unit. 8 January 2024. Archived from the original (PDF) on 14 December 2024. Retrieved 10 January 2024.
- 1 2 3 4 "End of an Era for CJD Surveillance, Research and Support in the UK — CJD Support Network". CJD Support Network. 23 March 2026. Retrieved 28 August 2026.
- ↑ Harries-Jones, R.; Knight, R.; Will, R. G.; Cousens, S.; Smith, P. G.; Matthews, W. B. (September 1988). "Creutzfeldt-Jakob disease in England and Wales, 1980-1984: a case-control study of potential risk factors". Journal of Neurology, Neurosurgery, and Psychiatry. 51 (9): 1113–1119. doi:10.1136/jnnp.51.9.1113. PMC 1033012. PMID 3066847.
- ↑ "Creutzfeldt-Jakob disease". nhs.uk. 23 October 2017. Retrieved 28 August 2026.
- ↑ Southwood Committee (1989). Report of the working committee on bovine spongiform encephalopathy (Report). London: Department of Health and Ministry of Agriculture, Fisheries and Food.
- 1 2 Ainsworth, Claire; Carrington, Damian (25 October 2000). "BSE disaster: the history". The New Scientist. Retrieved 28 August 2026.
- ↑ Hawkes, Nigel (6 August 2004). "New vCJD 'epidemic' could be on the way, scientists say". The Times.
- ↑ Hewitt, P. (November 2006). "Variant de la maladie de Creutzfeldt-Jakob et transfusion sanguine au Royaume-Uni" [vCJD and blood transfusion in the United Kingdom]. Transfusion Clinique et Biologique. 13 (5): 312–316. doi:10.1016/j.tracli.2006.11.006.
- ↑ "BTO0008 - Evidence on Blood, tissue and organ screening". committees.parliament.uk. Retrieved 28 August 2026.
- ↑ "Search for BSE test abandoned". The Independent. 11 August 1999. Retrieved 28 August 2026 – via Farmers Weekly.
- ↑ Connor, Steve (10 August 1999). "Fiasco as officials abort bid to prove BSE `wonder test'". The Independent. Retrieved 28 August 2026.
- 1 2 "People: University of Edinburgh announces directorship of the National Creutzfeldt-Jakob Disease surveillance unit:". Med Insights. 24 January 2002. Retrieved 28 August 2026.
- ↑ Hawkes, Nigel (21 May 2004). "Thousands carry ticking variant-CJD timebomb". Irish Independent. London. Retrieved 28 August 2026.
- ↑ "National CJD Research and Surveillance Unit". University of Edinburgh Research Explorer. School of Regeneration and Repair; University of Edinburgh; School of Population Health Sciences. Retrieved 28 August 2026.
- ↑ Burling, Stacey (18 January 2024). "New Research Finds That Creutzfeldt-Jakob Disease Is on the Rise". Neurology Today. 24 (2). Ovid Technologies (Wolters Kluwer Health): 5–6. doi:10.1097/01.NT.0001006340.74111.83.
Sources
[edit]- Ward, Hester JT. (September 2000). "Surveillance of Creutzfeldt Jakob disease in the United Kingdom". Eurosurveillance. 5 (9): 90–94. doi:10.2807/esm.05.09.00009-en.
- Watson, Neil; Kurudzhu, Hatice; Green, Alison; Summers, David; et al. (15 January 2021). "Application of telehealth for comprehensive Creutzfeldt-Jakob disease surveillance in the United Kingdom". Journal of the Neurological Sciences. 420: 117221.