Abstract
BACKGROUND
Creutzfeldt-Jakob disease is a fatal neurological disease caused by abnormal infectious proteins called prions. Prions that are present on surgical instruments cannot be completely deactivated; therefore, patients who are subsequently operated on using these instruments may become infected. This can result in surgically transmitted Creutzfeldt-Jakob disease.
OBJECTIVE
To update literature reviews, consultation with experts and economic modelling published in 2006, and to provide the cost-effectiveness of strategies to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease.
METHODS
Eight systematic reviews were undertaken for clinical parameters. One review of cost-effectiveness was undertaken. Electronic databases including MEDLINE and EMBASE were searched from 2005 to 2017. Expert elicitation sessions were undertaken. An advisory committee, convened by the National Institute for Health and Care Excellence to produce guidance, provided an additional source of information. A mathematical model was updated focusing on brain and posterior eye surgery and neuroendoscopy. The model simulated both patients and instrument sets. Assuming that there were potentially 15 cases of surgically transmitted Creutzfeldt-Jakob disease between 2005 and 2018, approximate Bayesian computation was used to obtain samples from the posterior distribution of the model parameters to generate results. Heuristics were used to improve computational efficiency. The modelling conformed to the National Institute for Health and Care Excellence reference case. The strategies evaluated included neither keeping instruments moist nor prohibiting set migration; ensuring that instruments were kept moist; prohibiting instrument migration between sets; and employing single-use instruments. Threshold analyses were undertaken to establish prices at which single-use sets or completely effective decontamination solutions would be cost-effective.
RESULTS
A total of 169 papers were identified for the clinical review. The evidence from published literature was not deemed sufficiently strong to take precedence over the distributions obtained from expert elicitation. Forty-eight papers were identified in the review of cost-effectiveness. The previous modelling structure was revised to add the possibility of misclassifying surgically transmitted Creutzfeldt-Jakob disease as another neurodegenerative disease, and assuming that all patients were susceptible to infection. Keeping instruments moist was estimated to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease cases and associated costs. Based on probabilistic sensitivity analyses, keeping instruments moist was estimated to on average result in 2.36 (range 0-47) surgically transmitted Creutzfeldt-Jakob disease cases (across England) caused by infection occurring between 2019 and 2023. Prohibiting set migration or employing single-use instruments reduced the estimated risk of surgically transmitted Creutzfeldt-Jakob disease cases further, but at considerable cost. The estimated costs per quality-adjusted life-year gained of these strategies in addition to keeping instruments moist were in excess of £1M. It was estimated that single-use instrument sets (currently £350-500) or completely effective cleaning solutions would need to cost approximately £12 per patient to be cost-effective using a £30,000 per quality-adjusted life-year gained value.
LIMITATIONS
As no direct published evidence to implicate surgery as a cause of Creutzfeldt-Jakob disease has been found since 2005, the estimations of potential cases from elicitation are still speculative. A particular source of uncertainty was in the number of potential surgically transmitted Creutzfeldt-Jakob disease cases that may have occurred between 2005 and 2018.
CONCLUSIONS
Keeping instruments moist is estimated to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease cases and associated costs. Further surgical management strategies can reduce the risks of surgically transmitted Creutzfeldt-Jakob disease but have considerable associated costs.
STUDY REGISTRATION
This study is registered as PROSPERO CRD42017071807.
FUNDING
This project was funded by the National Institute for Health Research (NIHR) Health Technology Assessment programme and will be published in full in Health Technology Assessment; Vol. 24, No. 11. See the NIHR Journals Library website for further project information.
Plain language summary
The aims of this report were to summarise evidence relating to surgically transmitted Creutzfeldt–Jakob disease and to explore the value for money of strategies to reduce the chance of any future surgically transmitted Creutzfeldt–Jakob disease cases. Current recommendations include keeping sets of surgical instruments together for high-risk operations and using separate instruments for people born after 1996. The project involved reviewing published papers, speaking with experts and building a computer model. The literature reviews found that Creutzfeldt–Jakob disease occurs in around 1–2 per million people and that no definite cases of surgically transmitted Creutzfeldt–Jakob disease have been observed since the 1970s. The reviews also looked for information on the possibility of patients being infected with Creutzfeldt–Jakob disease after having surgery on high-risk tissues, such as the brain and the back of the eye. They found that there was a great deal of uncertainty regarding who might have Creutzfeldt–Jakob disease, but not yet have symptoms, as well as the risk of transmission and the ability of strategies to reduce this risk. The computer model aimed to estimate value for money of different strategies to reduce the risks of surgically transmitted Creutzfeldt–Jakob disease. However, the reviews found that some of the numbers needed for the model were not known, so experts were asked to estimate this information instead along with the range of possible values. This information included the effectiveness of different cleaning practices and the chances of infected tissue being transmitted between patients undergoing high-risk surgery. The model found that keeping surgical instruments moist prior to cleaning was likely to save money and reduce the chance of future surgically transmitted Creutzfeldt–Jakob disease cases. However, additional measures, such as using only sets of single-use instruments, ensuring that instruments were kept together in their sets or using separate instruments for those born after 1996, appeared to be poor value for money.
Full text of this article can be found in Bookshelf.
References
- University of Edinburgh. The National CJD Research & Surveilance Unit (NCJDRSU). 2017. URL: www.cjd.ed.ac.uk/ (accessed 11 July 2017).
- National CJD Research & Surveillance Unit. Creutzfeldt–Jakob Disease Surveillance in the UK: 25th Annual Report 2016. Edinburgh: The National CJD Research & Surveillance Unit; 2016.
- Brown P, Brandel JP, Sato T, Nakamura Y, MacKenzie J, Will RG, et al. Iatrogenic Creutzfeldt–Jakob disease, final assessment. Emerging Infect Dis 2012;18:901–7. https://doi.org/10.3201/eid1806.120116 doi: 10.3201/eid1806.120116. [DOI] [PMC free article] [PubMed]
- World Health Organization (WHO). WHO Infection Control Guidelines for Transmissible Spongiform Encephalopathies: Report of a WHO Consultation, Geneva, Switzerland, 23–26 March 1999. Geneva: WHO; 2000.
- World Health Organization (WHO). WHO Tables on Tissue Infectivity Distribution in Transmissible Spongiform Encephalopathies. Geneva: WHO; 2010.
- Brown P, Farrell M. A practical approach to avoiding iatrogenic Creutzfeldt–Jakob disease (CJD) from invasive instruments. Infect Control Hosp Epidemiol 2015;36:844–8. https://doi.org/10.1017/ice.2015.53 doi: 10.1017/ice.2015.53. [DOI] [PubMed]
- Hilton DA, Ghani AC, Conyers L, Edwards P, McCardle L, Ritchie D, et al. Prevalence of lymphoreticular prion protein accumulation in UK tissue samples. J Pathol 2004;203:733–9. https://doi.org/10.1002/path.1580 doi: 10.1002/path.1580. [DOI] [PubMed]
- Ghani AC, Ferguson NM, Donnelly CA, Anderson RM. Factors determining the pattern of the variant Creutzfeldt–Jakob disease (vCJD) epidemic in the UK. Proc R Soc Lond B 2003;270:689–98. https://doi.org/10.1098/rspb.2002.2313 doi: 10.1098/rspb.2002.2313. [DOI] [PMC free article] [PubMed]
- Clarke P, Ghani AC. Projections of the future course of the primary vCJD epidemic in the UK: inclusion of subclinical infection and the possibility of wider genetic susceptibility. J R Soc Interface 2005;2:19–31. https://doi.org/10.1098/rsif.2004.0017 doi: 10.1098/rsif.2004.0017. [DOI] [PMC free article] [PubMed]
- Lloyd Jones M, Stevenson M, Sutton A. Patient Safety and Reduction of Risk of Transmission of Creutzfeldt–Jakob Disease (CJD) via Interventional Procedures. Interventional Procedure Guidance 196. London: National Institute for Health and Care Excellence, University of Sheffield, School of Health and Related Research; 2006.
- Stevenson M, Oakley J, Chick SE. Patient safety and reduction of risk of transmission of Creutzfeldt–Jakob disease (CJD) via interventional procedures—final report. Sheffield: School of Health and Related Research, University of Sheffield; 2006. URL: www.nice.org.uk/guidance/ipg196/documents/ipg196-patient-safety-and-reduction-of-risk-of-transmission-of-creutzfeldtjakob-disease-cjd-via-interventional-procedures-final-report2 (accessed 10 February 2020).
- Stevenson MD, Oakley JE, Chick SE, Chalkidou K. The cost-effectiveness of surgical instrument management policies to reduce the risk of vCJD transmission to humans. J Oper Res Soc 2009;60:506–18. https://doi.org/10.1057/palgrave.jors.2602580 doi: 10.1057/palgrave.jors.2602580. [DOI]
- Bennett P, Hare A, Townshend J. Assessing the risk of vCJD transmission via surgery: models for uncertainty and complexity. J Oper Res Soc 2005;56:202–13. https://doi.org/10.1057/palgrave.jors.2601899 doi: 10.1057/palgrave.jors.2601899. [DOI]
- National Institute for Health and Care Excellence (NICE). NICE Interventional Procedure Guidance 196. Patient Safety and Reduction of Risk of Transmission of Creutzfeldt–Jakob Disease (CJD) Via Interventional Procedures. London: NICE; 2008. www.nice.org.uk/guidance/ipg196/documents/ipg196-patient-safety-and-reduction-of-risk-of-transmission-of-creutzfeldtjakob-disease-cjd-via-interventional-procedures-guidance2 (accessed 2 February 2018).
- Public Health England. Summary Results of the Third National Survey of Abnormal Prion Prevalence in Archived Appendix Specimens. London: Public Health England; 2016.
- Advisory Committee on Dangerous Pathogens TSE Subgroup. Updated Position Statement on Occurrence of vCJD and Prevalence of Infection in the UK. 2016. URL: www.clinicalvirology.org/news/acdp-tse-subgroup-updated-position-statement-on-occurrence-of-vcjd-and-prevalence-of-infection-in-the-uk/ (accessed 8 January 2020).
- Jaunmuktane Z, Quaegebeur A, Taipa R, Viana-Baptista M, Barbosa R, Koriath C, et al. Evidence of amyloid-β cerebral amyloid angiopathy transmission through neurosurgery. Acta Neuropathol 2018;135:671–9. https://doi.org/10.1007/s00401-018-1822-2 doi: 10.1007/s00401-018-1822-2. [DOI] [PMC free article] [PubMed]
- Stevenson M, Jeremy O, Chick S. Patient Safety and Reduction of Risk of Transmission of Creutzfeldt–Jakob Disease (CJD) via Interventional Procedures. 2006. URL: www.nice.org.uk/guidance/ipg196/documents/ipg196-patient-safety-and-reduction-of-risk-of-transmission-of-creutzfeldtjakob-disease-cjd-via-interventional-procedures-final-report2 (accessed 3 October 2019).
- Lloyd Jones M, Stevenson M, Sutton A. Patient Safety and Reduction of Risk of Transmission of Creutzfeldt–Jakob Disease (CJD) via International Procedures. 2006. URL: www.nice.org.uk/guidance/ipg196/documents/ipg196-patient-safety-and-reduction-of-risk-of-transmission-of-creutzfeldtjakob-disease-cjd-via-interventional-procedures-systematic-review2 (accessed 3 October 2019).
- Moher D, Liberati A, Tetzlaff J, Altman DG, PRISMA Group. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement. PLOS Med 2009;6:e1000097. https://doi.org/10.1371/journal.pmed.1000097 doi: 10.1371/journal.pmed.1000097. [DOI] [PMC free article] [PubMed]
- Uttley L, Carroll C, Wong R, Stevenson M. Update review: risk of transmission via surgical interventional procedures of Creutzfeldt–Jakob disease (CJD) and economic modelling of clinical management policies. PROSPERO 2017 CRD42017071807. URL: www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42017071807 (accessed 10 February 2020).
- Creutzfeldt–Jakob Disease International Surveillance Network Formerly EuroCJD. CJD Surveillance Data 1993–2017. 2017. URL: www.eurocjd.ed.ac.uk/surveillance%20data%201.html (accessed 23 January 2018).
- US Centres for Disease Control and Prevention. Creutzfeldt–Jakob Disease, Classic (CJD) Occurrence and Transmission. 2018. www.cdc.gov/prions/cjd/occurrence-transmission.html (accessed 23 January 2018).
- Yamada M, Hamaguchi T, Sakai K, Nozaki I, Noguchi-Shinohara M, Sanjo N, et al. Epidemiological and clinical features of human prion diseases in Japan: prospective 17-year surveillance. Prion 2016;10:S10–S1.
- Klug GM, Boyd A, Sarros S, Stehmann C, Simpson M, McLean CA, et al. Creutzfeldt–Jakob disease surveillance in Australia: update to December 2015. Commun Dis Intell Q Rep 2016;40:E368–E376. [PubMed]
- Begué C, Martinetto H, Schultz M, Rojas E, Romero C, D’Giano C, et al. Creutzfeldt–Jakob disease surveillance in Argentina, 1997–2008. Neuroepidemiology 2011;37:193–202. https://doi.org/10.1159/000331907 doi: 10.1159/000331907. [DOI] [PubMed]
- Lu CJ, Sun Y, Chen SS. Incidence of Creutzfeldt–Jakob disease in Taiwan: a prospective 10-year surveillance. Eur J Epidemiol 2010;25:341–7. https://doi.org/10.1007/s10654-010-9446-4 doi: 10.1007/s10654-010-9446-4. [DOI] [PubMed]
- Jeon BH, Kim J, Kim GK, Park SC, Kim S, Cheong HK. Estimation of the size of the iatrogenic Creutzfeldt–Jakob disease outbreak associated with cadaveric dura mater grafts in Korea. Epidemiol Health 2016;38:e2016059. https://doi.org/10.4178/epih.e2016059 doi: 10.4178/epih.e2016059. [DOI] [PubMed]
- Gao C, Shi Q, Tian C, Chen C, Han J, Zhou W, et al. The epidemiological, clinical, and laboratory features of sporadic Creutzfeldt–Jakob disease patients in China: surveillance data from 2006 to 2010. PLOS ONE 2011;6:e24231. https://doi.org/10.1371/journal.pone.0024231 doi: 10.1371/journal.pone.0024231. [DOI] [PMC free article] [PubMed]
- University of Edinburgh. The National CJD Research & Surveilance Unit (NCJDRSU). Creutzfeldt–Jakob disease in the UK (by calendar year). 2019. URL: www.cjd.ed.ac.uk/sites/default/files/figs.pdf (accessed 26 November 2019).
- Molesworth A, Yates P, Hewitt PE, Mackenzie J, Ironside JW, Galea G, Ward HJT. vCJD associated with organ or tissue transplantation in the UK: a lookback study. Transplantation 2014;98:585–9. doi: 10.1097/TP.0000000000000105. [DOI] [PubMed]
- Isotalo J, Gardberg M, Verkkoniemi-Ahola A, Paetau A, Martikainen MH, Korpela J, et al. [Phenotype and incidence of Creutzfeldt–Jakob disease in Finland in 1997-2013.] Duodecim 2015;131:465–74. [PubMed]
- Chen SS. Surveillance of prion diseases in Taiwan. Prion 2016;10:S11.
- Van Everbroeck B, Michotte A, Sciot R, Godfraind C, Deprez M, Quoilin S, et al. Increased incidence of sporadic Creutzfeldt–Jakob disease in the age groups between 70 and 90 years in Belgium. Eur J Epidemiol 2006;21:443–7. https://doi.org/10.1007/s10654-006-9012-2 doi: 10.1007/s10654-006-9012-2. [DOI] [PubMed]
- Ae R, Nakamura Y, Takumi I, Sanjo N, Kitamoto T, Yamada M, et al. Epidemiologic features of human prion diseases in Japan: a prospective 15-year surveillance study. Prion 2016;10:S103–S4.
- Rus T, Caks–Jager N, Popovic M, Blasko Markic M, Kramberger Gregoric M. High incidence of sporadic Creutzfeldt–Jakob disease in Slovenia in 2015. Eur J Neurol 2016;23:602. doi: 10.1159/000486712. [DOI] [PMC free article] [PubMed]
- Mok T, Jaunmuktane Z, Joiner S, Campbell T, Morgan C, Wakerley B, et al. Variant Creutzfeldt–Jakob disease in a patient with heterozygosity at PRNP codon 129. N Engl J Med 2017;376:292–4. https://doi.org/10.1056/NEJMc1610003 doi: 10.1056/NEJMc1610003. [DOI] [PubMed]
- Urwin P, Mackenzie J, Knight R, Will R, Molesworth A. Is sporadic CJD an acquired disease? A review of the UK CJD cases. Prion 2016;10:S77.
- Shi J, Chen Q, Chen X, Zhang J. Case report: clinical scenarios in Creutzfeldt–Jakob disease (CJD): report of nine cases. Int J Exp Pathol 2016;9:2744–51.
- Baig M, Phillips M. A case of Creutzfeldt–Jakob disease: diagnostic dilemmas of a rapidly fatal disease. Infect Dis Rep 2013;5:e10. https://doi.org/10.4081/idr.2013.e10 doi: 10.4081/idr.2013.e10. [DOI] [PMC free article] [PubMed]
- Maddox RA, Holman RC, Folkema AM, Gambetti P, Zou WQ, Minino AM, et al. Creutzfeldt–Jakob disease among blacks in the United States, 1994–2007. Prion 2010;4:161.
- Holman RC, Belay ED, Christensen KY, Maddox RA, Minino AM, Folkema AM, et al. Human prion diseases in the United States. PLOS ONE 2010;5:e8521. https://doi.org/10.1371/journal.pone.0008521 doi: 10.1371/journal.pone.0008521. [DOI] [PMC free article] [PubMed]
- Maddox RA, Holman RC, Minino AM, Blevins JE, Schonberger LB, Belay ED. Prion disease among Asians and Pacific Islanders in the United States, 2003–9. Prion 2013;7:60.
- Nakatani E, Nishimura T, Zhou B, Kaneda H, Teramukai S, Nagai Y, et al. Temporal and regional variations in sporadic Creutzfeldt–Jakob disease in Japan, 2001–10. Epidemiol Infect 2015;143:1073–8. https://doi.org/10.1017/S0950268814001605 doi: 10.1017/S0950268814001605. [DOI] [PMC free article] [PubMed]
- Klug GM, Wand H, Boyd A, Law M, Whyte S, Kaldor J, et al. Enhanced geographically restricted surveillance simulates sporadic Creutzfeldt–Jakob disease cluster. Brain 2009;132:493–501. https://doi.org/10.1093/brain/awn303 doi: 10.1093/brain/awn303. [DOI] [PubMed]
- Brandel JP, Peckeu L, Haïk S. The French surveillance network of Creutzfeldt–Jakob disease. Epidemiological data in France and worldwide. Transfus Clin Biol 2013;20:395–7. https://doi.org/10.1016/j.tracli.2013.02.029 doi: 10.1016/j.tracli.2013.02.029. [DOI] [PubMed]
- Mitrová E, Kosorinová D, Gajdoš M, Šebeková K, Tomečková I. A pilot study of a genetic CJD risk factor (E200K) in the general Slovak population. Eur J Epidemiol 2014;29:595–7. https://doi.org/10.1007/s10654-014-9937-9 doi: 10.1007/s10654-014-9937-9. [DOI] [PubMed]
- Ladogana A, Puopolo M, Croes EA, Budka H, Jarius C, Collins S, et al. Mortality from Creutzfeldt–Jakob disease and related disorders in Europe, Australia, and Canada. Neurology 2005;64:1586–91. https://doi.org/10.1212/01.WNL.0000160117.56690.B2 doi: 10.1212/01.WNL.0000160117.56690.B2. [DOI] [PubMed]
- Tuskan-Mohar L, Legac M, Prunk DA, Bucuk M, Perkovic O, Antoncic I. The frequency of Creutzfeldt–Jakob disease in Primorsko-Goranska county. Acta Clin Croat Suppl 2012;51:83–4.
- Kosier N. Why won’t she talk? A case of Creutzfeldt–Jakob disease masquerading as psychiatric decompensation. J Am Geriatr Soc 2017;65:S25.
- Litzroth A, Cras P, De Vil B, Quoilin S. Overview and evaluation of 15 years of Creutzfeldt–Jakob disease surveillance in Belgium, 1998-2012. BMC Neurol 2015;15:250. https://doi.org/10.1186/s12883-015-0507-x doi: 10.1186/s12883-015-0507-x. [DOI] [PMC free article] [PubMed]
- Brett FM, Looby S, Chalissery A, Chen D, Heaney C, Heffernan J, et al. Brain biopsies requiring Creutzfeldt–Jakob disease precautions in the Republic of Ireland 2005–2016. Ir J Med Sci 2018;187:5–5–20. https://doi.org/10.1007/s11845-017-1673-1 doi: 10.1007/s11845-017-1673-1. [DOI] [PubMed]
- Loftus T, Chen D, Looby S, Chalissery A, Howley R, Heaney C, et al. CJD surveillance in the Republic of Ireland from 2005 to 2015: a suggested algorithm for referrals. Clin Neuropathol 2017;36:188–94. https://doi.org/10.5414/NP301016 doi: 10.5414/NP301016. [DOI] [PubMed]
- Ali A, Abbas M, Ahmed S, Ejaz K. Creutzfeldt–Jakob Disease (CJD) rare stroke mimic. Cerebrovasc Dis 2015;39:147.
- Hirst CL. Sporadic Creutzfeldt–Jakob disease presenting as a stroke mimic590. Br J Hosp Med 2011;72:590–1. https://doi.org/10.12968/hmed.2011.72.10.590 doi: 10.12968/hmed.2011.72.10.590. [DOI] [PubMed]
- Hanumanthu R, Alchaki A, Nyaboga A, Ghuman H, Chen J, Feinstein E. An unusual case of sporadic Creutzfeld Jacob disease presenting as acute neuropathy [abstract]. Mov Disord 2017;32:563–4.
- Karatas H, Dericioglu N, Kursun O, Saygi S. Creutzfeldt–Jakob disease presenting as hyperparathyroidism and generalized tonic status epilepticus. Clinical EEG Neurosci 2007;38:203–6. https://doi.org/10.1177/155005940703800404 doi: 10.1177/155005940703800404. [DOI] [PubMed]
- Kher M, Rao MY, Acharya PT, Mahadevan A, Shankar SK. Heidenhain variant of Creutzfeldt–Jakob disease: an autopsy study from India. Ann Indian Acad Neurol 2009;12:48–51. doi: 10.4103/0972-2327.48856. [DOI] [PMC free article] [PubMed]
- Neville JL, Fichtenbaum C. ‘Rapidly progressive dementia: Sometimes it is a zebra’. J Gen Intern Med 2012;27:S508.
- Pachalska M, Kurzbauer H, Formińska-Kapuścik M, Urbanik A, Bierzyńska-Macyszyn G, Właszczuk P. Atypical features of dementia in a patient with Creutzfeldt–Jakob disease. Med Sci Monit 2007;13:CS9–19. [PubMed]
- Patrawala S, Soltani M, Zulauf M. A rare case of ataxia and rapidly progressing dementia. J Gen Intern Med 2014;29:S280–S1.
- Krystina C, Abbas A, Hall A, Khadjooi K, Elhag RA, Rostami K. Sporadic CJD presenting with aphasia diagnosed in medical admissions unit. Eur J Intern Med 2011;22:S111. https://doi.org/10.1016/S0953-6205(11)60451-2 doi: 10.1016/S0953-6205(11)60451-2. [DOI]
- Sann AA, Zaw MM, Choie TL. Creutzfeldt–Jakob disease presenting predominantly with movement disorder: a case report. Mov Disord 2016;31:S577–S8.
- Bruton CJ, Bruton RK, Gentleman SM, Roberts GW. Diagnosis and incidence of prion (Creutzfeldt–Jakob) disease: a retrospective archival survey with implications for future research. Neurodegeneration 1995;4:357–68. https://doi.org/10.1006/neur.1995.0043 doi: 10.1006/neur.1995.0043. [DOI] [PubMed]
- Urwin P, Thanigaikumar K, Ironside JW, Molesworth A, Knight RS, Hewitt PE, et al. Sporadic Creutzfeldt–Jakob disease in 2 plasma product recipients, United Kingdom. Emerging Infect Dis 2017;23. https://doi.org/10.3201/eid2306.161884 doi: 10.3201/eid2306.161884. [DOI] [PMC free article] [PubMed]
- Shepherd KJ, Barker BR. A common presentation of a rare disease: sporadic CJD. J Gen Intern Med 2016;1:S500–S1.
- Galeno R, Di Bari MA, Nonno R, Cardone F, Sbriccoli M, Graziano S, et al. Prion strain characterization of a novel subtype of Creutzfeldt–Jakob disease. J Virol 2017;91:e02390–16. https://doi.org/10.1128/JVI.02390-16 doi: 10.1128/JVI.02390-16. [DOI] [PMC free article] [PubMed]
- Nagoshi K, Sadakane A, Nakamura Y, Yamada M, Mizusawa H. Duration of prion disease is longer in Japan than in other countries. J Epidemiol 2011;21:255–62. https://doi.org/10.2188/jea.JE20100085 doi: 10.2188/jea.JE20100085. [DOI] [PMC free article] [PubMed]
- Rudge P, Jaunmuktane Z, Adlard P, Bjurstrom N, Caine D, Lowe J, et al. Iatrogenic CJD due to pituitary-derived growth hormone with genetically determined incubation times of up to 40 years. Brain 2015;138:3386–99. https://doi.org/10.1093/brain/awv235 doi: 10.1093/brain/awv235. [DOI] [PMC free article] [PubMed]
- Sanchez-Juan P, Bishop MT, Croes EA, Knight RS, Will RG, van Duijn CM, Manson JC. A polymorphism in the regulatory region of PRNP is associated with increased risk of sporadic Creutzfeldt–Jakob disease. BMC Med Genet 2011;12:73. https://doi.org/10.1186/1471-2350-12-73 doi: 10.1186/1471-2350-12-73. [DOI] [PMC free article] [PubMed]
- Brandner S, Jaunmuktane Z. Prion disease: experimental models and reality. Acta Neuropathol 2017;133:197–222. https://doi.org/10.1007/s00401-017-1670-5 doi: 10.1007/s00401-017-1670-5. [DOI] [PMC free article] [PubMed]
- Giaccone G, Capellari S, Ingrosso L, Ferrari S, Imperiale D, Taraglio S, et al. An update of the epidemiology of sporadic Creutzfeldt–Jakob disease in Italy based on neuropathologic and molecular typing of a large cohort of patients. Clin Neuropathol 2009;28:229.
- Ironside JW, Bishop MT, Connolly K, Hegazy D, Lowrie S, Le Grice M, et al. Variant Creutzfeldt–Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. BMJ 2006;332:1186–8. https://doi.org/10.1136/bmj.38804.511644.55 doi: 10.1136/bmj.38804.511644.55. [DOI] [PMC free article] [PubMed]
- Gill ON, Spencer Y, Richard-Loendt A, Kelly C, Dabaghian R, Boyes L, et al. Prevalent abnormal prion protein in human appendixes after bovine spongiform encephalopathy epizootic: large scale survey. BMJ 2013;347:f5675. https://doi.org/10.1136/bmj.f5675 doi: 10.1136/bmj.f5675. [DOI] [PMC free article] [PubMed]
- Mead S, Whitfield J, Poulter M, Shah P, Uphill J, Beck J, et al. Genetic susceptibility, evolution and the kuru epidemic. Phil Trans R Soc B 2008;363:3741–6. https://doi.org/10.1098/rstb.2008.0087 doi: 10.1098/rstb.2008.0087. [DOI] [PMC free article] [PubMed]
- Kaski D, Mead S, Hyare H, Cooper S, Jampana R, Overell J, et al. Variant CJD in an individual heterozygous for PRNP codon 129. Lancet 2009;374:2128. https://doi.org/10.1016/S0140-6736(09)61568-3 doi: 10.1016/S0140-6736(09)61568-3. [DOI] [PubMed]
- Peden A, McCardle L, Head MW, Love S, Ward HJ, Cousens SN, et al. Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia. Haemophilia 2010;16:296–304. https://doi.org/10.1111/j.1365-2516.2009.02181.x doi: 10.1111/j.1365-2516.2009.02181.x. [DOI] [PubMed]
- Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004;364:527–9. https://doi.org/10.1016/S0140-6736(04)16811-6 doi: 10.1016/S0140-6736(04)16811-6. [DOI] [PubMed]
- Bishop MT, Hart P, Aitchison L, Baybutt HN, Plinston C, Thomson V, et al. Predicting susceptibility and incubation time of human-to-human transmission of vCJD. Lancet Neurol 2006;5:393–8. https://doi.org/10.1016/S1474-4422(06)70413-6 doi: 10.1016/S1474-4422(06)70413-6. [DOI] [PubMed]
- Pennington C, Knight R. The clinicopathological phenotype of genetic CJD due to the E200K mutation in the UK. Prion 2010;4:197.
- Coulthart MB, Geschwind MD, Qureshi S, Phielipp N, Demarsh A, Abrams JY, et al. A case cluster of variant Creutzfeldt–Jakob disease linked to the Kingdom of Saudi Arabia. Brain 2016;139:2609–16. https://doi.org/10.1093/brain/aww206 doi: 10.1093/brain/aww206. [DOI] [PMC free article] [PubMed]
- Chohan G, Llewelyn C, Mackenzie J, Cousens S, Kennedy A, Will R, Hewitt P. Variant Creutzfeldt–Jakob disease in a transfusion recipient: coincidence or cause? Transfusion 2010;50:1003–6. https://doi.org/10.1111/j.1537-2995.2010.02614.x doi: 10.1111/j.1537-2995.2010.02614.x. [DOI] [PubMed]
- Davidson LR, Llewelyn CA, Mackenzie JM, Hewitt PE, Will RG. Variant CJD and blood transfusion: are there additional cases? Vox Sang 2014;107:220–5. https://doi.org/10.1111/vox.12161 doi: 10.1111/vox.12161. [DOI] [PubMed]
- Molesworth A, Yates P, Hewitt PE, Mackenzie J, Ironside JW, Galea G, Ward HJ. Investigation of variant Creutzfeldt–Jakob disease implicated organ or tissue transplantation in the United Kingdom. Transplantation 2014;98:585–9. https://doi.org/10.1097/TP.0000000000000105 doi: 10.1097/TP.0000000000000105. [DOI] [PubMed]
- Ward HJT, Will RG, Ghani A, Ironside JW. An update on variant CJD (vCJD), secondary transmission and prevalence. Eur J Neurol 2006;13:306–7.
- Urwin PJ, Mackenzie JM, Llewelyn CA, Will RG, Hewitt PE. Creutzfeldt–Jakob disease and blood transfusion: updated results of the UK transfusion medicine epidemiology review study. Vox Sang 2016;110:310–16. https://doi.org/10.1111/vox.12371 doi: 10.1111/vox.12371. [DOI] [PubMed]
- Ward HJ, MacKenzie JM, Llewelyn CA, Knight RS, Hewitt PE, Connor N, et al. Variant Creutzfeldt–Jakob disease and exposure to fractionated plasma products. Vox Sang 2009;97:207–10. https://doi.org/10.1111/j.1423-0410.2009.01205.x doi: 10.1111/j.1423-0410.2009.01205.x. [DOI] [PubMed]
- Diack AB, Will RG, Manson JC. Public health risks from subclinical variant CJD. PLOS Pathog 2017;13:e1006642. https://doi.org/10.1371/journal.ppat.1006642 doi: 10.1371/journal.ppat.1006642. [DOI] [PMC free article] [PubMed]
- Diack AB, Head MW, McCutcheon S, Boyle A, Knight R, Ironside JW, et al. Variant CJD. 18 years of research and surveillance. Prion 2014;8:286–95. https://doi.org/10.4161/pri.29237 doi: 10.4161/pri.29237. [DOI] [PMC free article] [PubMed]
- Ritchie DL, Boyle A, McConnell I, Head MW, Ironside JW, Bruce ME. Transmissions of variant Creutzfeldt–Jakob disease from brain and lymphoreticular tissue show uniform and conserved bovine spongiform encephalopathy-related phenotypic properties on primary and secondary passage in wild-type mice. J Gen Virol 2009;90:3075–82. https://doi.org/10.1099/vir.0.013227-0 doi: 10.1099/vir.0.013227-0. [DOI] [PubMed]
- Diack AB, Boyle A, Ritchie DL, Rabano A, de Pedro-Cuesta J, Brandel JP, et al. Variant CJD: Lessons in public health. Prion 2016;10:S82–S3.
- de Marco MF, Linehan J, Gill ON, Clewley JP, Brandner S. Large-scale immunohistochemical examination for lymphoreticular prion protein in tonsil specimens collected in Britain. J Pathol 2010;222:380–7. https://doi.org/10.1002/path.2767 doi: 10.1002/path.2767. [DOI] [PubMed]
- Clewley JP, Kelly CM, Andrews N, Vogliqi K, Mallinson G, Kaisar M, et al. Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: cross sectional opportunistic survey. BMJ 2009;338:b1442. https://doi.org/10.1136/bmj.b1442 doi: 10.1136/bmj.b1442. [DOI] [PMC free article] [PubMed]
- Olsen SB, Sheikh A, Peck D, Darzi A. Variant Creutzfeldt–Jakob disease: a cause for concern. Review of the evidence for risk of transmission through abdominal lymphoreticular tissue surgery. Surg Endosc 2005;19:747–50. https://doi.org/10.1007/s00464-004-9205-2 doi: 10.1007/s00464-004-9205-2. [DOI] [PubMed]
- McGowan CR, Viens AM. Coroners and the obligation to protect public health: the case of the failed UK vCJD study. Public Health 2011;125:234–7. https://doi.org/10.1016/j.puhe.2010.12.001 doi: 10.1016/j.puhe.2010.12.001. [DOI] [PubMed]
- McGowan CR, Viens AM. Death investigation systems and disease surveillance. Epidemiol Infect 2011;139:986–90. https://doi.org/10.1017/S0950268810002840 doi: 10.1017/S0950268810002840. [DOI] [PubMed]
- Rebello A. Correspondence With the CSEW Concerning Research into Subclinical vCJD. Liverpool: Honorary Secretary of the Coroners’ Society of England and Wales; 2007.
- Kobayashi A, Parchi P, Yamada M, Mohri S, Kitamoto T. Neuropathological and biochemical criteria to identify acquired Creutzfeldt–Jakob disease among presumed sporadic cases. Neuropathology 2016;36:305–10. https://doi.org/10.1111/neup.12270 doi: 10.1111/neup.12270. [DOI] [PubMed]
- Kobayashi A, Parchi P, Yamada M, Brown P, Saverioni D, Matsuura Y, et al. Iatrogenic transmission of Creutzfeldt–Jakob disease. Prion 2016;10:S5.
- Kobayashi A, Parchi P, Yamada M, Brown P, Saverioni D, Matsuura Y, et al. Transmission properties of atypical Creutzfeldt–Jakob disease: a clue to disease etiology? J Virol 2015;89:3939–46. https://doi.org/10.1128/JVI.03183-14 doi: 10.1128/JVI.03183-14. [DOI] [PMC free article] [PubMed]
- Gnanajothy R, Umashanker D, Vega MC, Wu BJ. A case of Creutzfeldt–Jakob disease following cataract surgery: sporadic versus iatrogenic cause. Conn Med 2013;77:335–7. [PubMed]
- Tuck K, Mass M. Sporadic Creutzfeld-Jakob disease in a 33 year old male with prior cerebral instrumentation. Neurology 2013;80:P06.195.
- Moreno MJ, Escriche D, Romero J, Maciñeiras JL, Corredera E, Castro MD, et al. Creutzfeldt–Jakob disease cluster in the health area of Meixoeiro Hospital. Acta Neurol Scand 2013;127:38–45. https://doi.org/10.1111/j.1600-0404.2012.01678.x doi: 10.1111/j.1600-0404.2012.01678.x. [DOI] [PubMed]
- Puopolo M, Ladogana A, Vetrugno V, Pocchiari M. Transmission of sporadic Creutzfeldt–Jakob disease by blood transfusion: risk factor or possible biases. Transfusion 2011;51:1556–66. https://doi.org/10.1111/j.1537-2995.2010.03004.x doi: 10.1111/j.1537-2995.2010.03004.x. [DOI] [PubMed]
- de Pedro-Cuesta J, Mahillo-Fernández I, Rábano A, Calero M, Cruz M, Siden A, et al. Nosocomial transmission of sporadic Creutzfeldt–Jakob disease: results from a risk-based assessment of surgical interventions. J Neurol Neurosurg Psychiatry 2011;82:204–12. https://doi.org/10.1136/jnnp.2009.188425 doi: 10.1136/jnnp.2009.188425. [DOI] [PMC free article] [PubMed]
- Mahillo-Fernandez I, de Pedro-Cuesta J, Bleda MJ, Cruz M, Mølbak K, Laursen H, et al. Surgery and risk of sporadic Creutzfeldt–Jakob disease in Denmark and Sweden: registry-based case-control studies. Neuroepidemiology 2008;31:229–40. https://doi.org/10.1159/000163097 doi: 10.1159/000163097. [DOI] [PMC free article] [PubMed]
- Hamaguchi T, Noguchi-Shinohara M, Nozaki I, Nakamura Y, Sato T, Kitamoto T, et al. The risk of iatrogenic Creutzfeldt–Jakob disease through medical and surgical procedures. Neuropathology 2009;29:625–31. https://doi.org/10.1111/j.1440-1789.2009.01023.x doi: 10.1111/j.1440-1789.2009.01023.x. [DOI] [PubMed]
- Hamaguchi T, Noguchi-Shinohara M, Nozaki I, Nakamura Y, Sato T, Kitamoto T, et al. Medical procedures and risk for sporadic Creutzfeldt–Jakob disease, Japan, 1999–2008. Emerging Infect Dis 2009;15:265–71. https://doi.org/10.3201/eid1502.080749 doi: 10.3201/eid1502.080749. [DOI] [PMC free article] [PubMed]
- Ruegger J, Stoeck K, Amsler L, Blaettler T, Zwahlen M, Aguzzi A, et al. A case-control study of sporadic Creutzfeldt–Jakob disease in Switzerland: analysis of potential risk factors with regard to an increased CJD incidence in the years 2001–2004. BMC Public Health 2009;9:18. https://doi.org/10.1186/1471-2458-9-18 doi: 10.1186/1471-2458-9-18. [DOI] [PMC free article] [PubMed]
- Ward HJ, Everington D, Cousens SN, Smith-Bathgate B, Leitch M, Cooper S, et al. Risk factors for variant Creutzfeldt–Jakob disease: a case-control study. Ann Neurol 2006;59:111–20. https://doi.org/10.1002/ana.20708 doi: 10.1002/ana.20708. [DOI] [PubMed]
- Ward HJ, Everington D, Cousens SN, Smith-Bathgate B, Gillies M, Murray K, et al. Risk factors for sporadic Creutzfeldt–Jakob disease. Ann Neurol 2008;63:347–54. https://doi.org/10.1002/ana.21294 doi: 10.1002/ana.21294. [DOI] [PubMed]
- de Pedro-Cuesta J, Ruiz Tovar M, Ward H, Calero M, Smith A, Verduras CA, et al. Sensitivity to biases of case-control studies on medical procedures, particularly surgery and blood transfusion, and risk of Creutzfeldt–Jakob disease. Neuroepidemiology 2012;39:1–18. https://doi.org/10.1159/000339318 doi: 10.1159/000339318. [DOI] [PubMed]
- Alcalde-Cabero E, Almazan-Isla J, Brandel JP, Breithaupt M, Catarino J, Collins S, et al. Health professions and risk of sporadic Creutzfeldt–Jakob disease, 1965 to 2010. Euro Surveill 2012;17:20144. [PubMed]
- de Pedro-Cuesta J, Mahillo-Fernandez I, Calero M, Rábano A, Cruz M, Siden Å, et al. Towards an age-dependent transmission model of acquired and sporadic Creutzfeldt–Jakob disease. PLOS ONE 2014;9:e109412. https://doi.org/10.1371/journal.pone.0109412 doi: 10.1371/journal.pone.0109412. [DOI] [PMC free article] [PubMed]
- Cruz M, Mahillo-Fernandez I, Rábano A, Siden A, Calero M, Laursen H, et al. Late-in-life surgery associated with Creutzfeldt–Jakob disease: a methodological outline for evidence-based guidance. Emerg Themes Epidemiol 2013;10:5. https://doi.org/10.1186/1742-7622-10-5 doi: 10.1186/1742-7622-10-5. [DOI] [PMC free article] [PubMed]
- Kobayashi A. Mechanisms of transmission of prion diseases. Clin Neurol 2016;56:S84.
- Bryant G, Hewitt P, Hope J, Howard C, Ironside J, Knight R, et al. Minimise Transmission Risk of CJD and vCJD in Healthcare Settings. Report on the Prevention of CJD and vCJD by Advisory Committee on Dangerous Pathogens’ Transmission Spongiform Encephalopathy (ACDP TSE) Subgroup. 2015. URL: www.gov.uk/government/publications/guidance-from-the-acdp-tse-risk-management-subgroup-formerly-tse-working-group (accessed 8 January 2020).
- Hall V, Brookes D, Nacul L, Gill ON, Connor N, CJD Incidents Panel. Managing the risk of iatrogenic transmission of Creutzfeldt–Jakob disease in the UK. J Hosp Infect 2014;88:22–7. https://doi.org/10.1016/j.jhin.2014.06.002 doi: 10.1016/j.jhin.2014.06.002. [DOI] [PubMed]
- Belay ED, Blase J, Sehulster LM, Maddox RA, Schonberger LB. Management of neurosurgical instruments and patients exposed to Creutzfeldt–Jakob disease. Infect Control Hosp Epidemiol 2013;34:1272–80. https://doi.org/10.1086/673986 doi: 10.1086/673986. [DOI] [PMC free article] [PubMed]
- Thomas JG, Chenoweth CE, Sullivan SE. Iatrogenic Creutzfeldt–Jakob disease via surgical instruments. J Clin Neurosci 2013;20:1207–12. https://doi.org/10.1016/j.jocn.2013.01.007 doi: 10.1016/j.jocn.2013.01.007. [DOI] [PubMed]
- Orrú CD, Yuan J, Appleby BS, Li B, Li Y, Winner D, et al. Prion seeding activity and infectivity in skin samples from patients with sporadic Creutzfeldt–Jakob disease. Sci Transl Med 2017;9. https://doi.org/10.1126/scitranslmed.aam7785 doi: 10.1126/scitranslmed.aam7785. [DOI] [PMC free article] [PubMed]
- Zou W, Orru CD, Yuan J, Appleby BS, Li Y, Rarick J, et al. PrPSc in the skin of CJD patients. Prion 2016;10:S29.
- Notari S, Moleres FJ, Hunter SB, Belay ED, Schonberger LB, Cali I, et al. Multiorgan detection and characterization of protease-resistant prion protein in a case of variant CJD examined in the United States. PLOS ONE 2010;5:e8765. https://doi.org/10.1371/journal.pone.0008765 doi: 10.1371/journal.pone.0008765. [DOI] [PMC free article] [PubMed]
- Davanipour Z, Sobel E, Ziogas A, Smoak C, Bohr T, Doram K, Liwnicz B. Ocular tonometry and sporadic Creutzfeldt–Jakob disease (sCJD): a confirmatory case-control study. Br J Med Med Res 2014;4:2322–33. https://doi.org/10.9734/BJMMR/2014/7247 doi: 10.9734/BJMMR/2014/7247. [DOI] [PMC free article] [PubMed]
- Tullo AB, Buckley RJ, Kelly T, Head MW, Bennett P, Armitage WJ, Ironside JW. Transplantation of ocular tissue from a donor with sporadic Creutzfeldt–Jakob disease. Clin Experiment Ophthalmol 2006;34:645–9. https://doi.org/10.1111/j.1442-9071.2006.01308.x doi: 10.1111/j.1442-9071.2006.01308.x. [DOI] [PubMed]
- Jirsova K, Krabcova I, Novakova J, Hnathova I, Koukolik F, Kubesova B, et al. The assessment of pathogenic prions in the brains of eye tissue donors: 2-years experience in the Czech Republic. Cornea 2010;29:996–9. https://doi.org/10.1097/ICO.0b013e3181cc7b37 doi: 10.1097/ICO.0b013e3181cc7b37. [DOI] [PubMed]
- Maddox RA, Belay ED, Curns AT, Zou WQ, Nowicki S, Lembach RG, et al. Creutzfeldt–Jakob disease in recipients of corneal transplants. Cornea 2008;27:851–4. https://doi.org/10.1097/ICO.0b013e31816a628d doi: 10.1097/ICO.0b013e31816a628d. [DOI] [PMC free article] [PubMed]
- Bourvis N, Boelle PY, Cesbron JY, Valleron AJ. Risk assessment of transmission of sporadic Creutzfeldt–Jakob disease in endodontic practice in absence of adequate prion inactivation. PLOS ONE 2007;2:e1330. https://doi.org/10.1371/journal.pone.0001330 doi: 10.1371/journal.pone.0001330. [DOI] [PMC free article] [PubMed]
- Everington D, Smith AJ, Ward HJ, Letters S, Will RG, Bagg J. Dental treatment and risk of variant CJD – a case control study. Br Dent J 2007;202:E19. https://doi.org/10.1038/bdj.2007.126 doi: 10.1038/bdj.2007.126. [DOI] [PubMed]
- Azarpazhooh A, Fillery ED. Prion disease: the implications for dentistry. J Endod 2008;34:1158–66. https://doi.org/10.1016/j.joen.2008.07.008 doi: 10.1016/j.joen.2008.07.008. [DOI] [PubMed]
- Collinge J, Whitfield J, McKintosh E, Frosh A, Mead S, Hill AF, et al. A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea. Phil Trans R Soc B 2008;363:3725–39. https://doi.org/10.1098/rstb.2008.0068 doi: 10.1098/rstb.2008.0068. [DOI] [PMC free article] [PubMed]
- Collinge J. Lessons of kuru research: background to recent studies with some personal reflections. Phil Trans R Soc B 2008;363:3689–96. https://doi.org/10.1098/rstb.2008.0121 doi: 10.1098/rstb.2008.0121. [DOI] [PMC free article] [PubMed]
- Collinge J, Whitfield J, McKintosh E, Beck J, Mead S, Thomas DJ, Alpers MP. Kuru in the 21st century – an acquired human prion disease with very long incubation periods. Lancet 2006;367:2068–74. https://doi.org/10.1016/S0140-6736(06)68930-7 doi: 10.1016/S0140-6736(06)68930-7. [DOI] [PubMed]
- Collinge J, Alpers MP. Incubation period of human prion disease - author’s reply. Lancet 2006;368:914–15. https://doi.org/10.1016/S0140-6736(06)69363-X doi: 10.1016/S0140-6736(06)69363-X. [DOI]
- Haïk S, Brandel JP. Infectious prion diseases in humans: cannibalism, iatrogenicity and zoonoses. Infect Genet Evol 2014;26:303–12. https://doi.org/10.1016/j.meegid.2014.06.010 doi: 10.1016/j.meegid.2014.06.010. [DOI] [PubMed]
- Hamaguchi T. [Clinical manifestations and epidemiology of prion diseases in Japan.] Rinsho Shinkeigaku 2013;23:1246–8. https://doi.org/10.5692/clinicalneurol.53.1246 doi: 10.5692/clinicalneurol.53.1246. [DOI] [PubMed]
- Heath CA, Barker RA, Esmonde TF, Harvey P, Roberts R, Trend P, et al. Dura mater-associated Creutzfeldt–Jakob disease: experience from surveillance in the UK. J Neurol Neurosurg Psychiatry 2006;77:880–2. https://doi.org/10.1136/jnnp.2005.073395 doi: 10.1136/jnnp.2005.073395. [DOI] [PMC free article] [PubMed]
- Hirst C. Iatrogenic Creutzfeldt–Jakob disease presenting 24 years after human growth hormone administration. Br J Hosp Med 2005;66:592–3. https://doi.org/10.12968/hmed.2005.66.10.19901 doi: 10.12968/hmed.2005.66.10.19901. [DOI] [PubMed]
- Meissner B, Kallenberg K, Sanchez-Juan P, Ramljak S, Krasnianski A, Heinemann U, et al. MRI and clinical syndrome in dura mater-related Creutzfeldt–Jakob disease. J Neurol 2009;256:355–63. https://doi.org/10.1007/s00415-009-0026-z doi: 10.1007/s00415-009-0026-z. [DOI] [PubMed]
- Ritchie DL, Barria MA, Peden AH, Yull HM, Kirkpatrick J, Adlard P, et al. UK Iatrogenic Creutzfeldt–Jakob disease: investigating human prion transmission across genotypic barriers using human tissue-based and molecular approaches. Acta Neuropathol 2017;133:579–95. https://doi.org/10.1007/s00401-016-1638-x doi: 10.1007/s00401-016-1638-x. [DOI] [PMC free article] [PubMed]
- Wroe SJ, Pal S, Siddique D, Hyare H, Macfarlane R, Joiner S, et al. Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt–Jakob disease associated with blood transfusion: a case report. Lancet 2006;368:2061–7. https://doi.org/10.1016/S0140-6736(06)69835-8 doi: 10.1016/S0140-6736(06)69835-8. [DOI] [PubMed]
- Cervenova L, Goldfarb LG, Garruto R, Lee HS, Gajdusek DC, Brown P. Phenotype-genotype studies in kuru: implications for new variant Creutzfeldt–Jakob disease. Proc Natl Acad Sci USA 1998;95:13239–41. https://doi.org/10.1073/pnas.95.22.13239 doi: 10.1073/pnas.95.22.13239. [DOI] [PMC free article] [PubMed]
- Klitzman RL, Alpers MP, Gadjusek DC. The natural incubation period of kuru and the episodes of transmission in three clusters of patients. Neuroepidemiology 1984;3:3–20. https://doi.org/10.1159/000110837 doi: 10.1159/000110837. [DOI]
- Kobayashi A, Teruya K, Matsuura Y, Shirai T, Nakamura Y, Yamada M, et al. The influence of PRNP polymorphisms on human prion disease susceptibility: an update. Acta Neuropathol 2015;130:159–70. https://doi.org/10.1007/s00401-015-1447-7 doi: 10.1007/s00401-015-1447-7. [DOI] [PubMed]
- Xiao X, Yuan J, Qing L, Cali I, Mikol J, Delisle MB, et al. Comparative study of prions in iatrogenic and sporadic creutzfeldt–jakob disease. J Clin Cell Immunol 2014;5:240. https://doi.org/10.4172/2155-9899.1000240 doi: 10.4172/2155-9899.1000240. [DOI] [PMC free article] [PubMed]
- Ironside JW. Variant Creutzfeldt–Jakob disease. Haemophilia 2010;16:175–80. https://doi.org/10.1111/j.1365-2516.2010.02317.x doi: 10.1111/j.1365-2516.2010.02317.x. [DOI] [PubMed]
- Hamaguchi T, Sakai K, Noguchi-Shinohara M, Nozaki I, Takumi I, Sanjo N, et al. Insight into the frequent occurrence of dura mater graft-associated Creutzfeldt–Jakob disease in Japan. J Neurol Neurosurg Psychiatry 2013;84:1171–5. https://doi.org/10.1136/jnnp-2012-304850 doi: 10.1136/jnnp-2012-304850. [DOI] [PubMed]
- Ibrahim-Verbaas C, Engelen-Lee JY, Spliet W, Mondria T, Willems S, Van Duijn C, et al. CJD with numerous Abeta plaques in a 58-year old patient 28 years after dura mater grafting. Prion 2012;6:131–2.
- Makarava N, Savtchenko R, Alexeeva I, Rohwer RG, Baskakov IV. Fast and ultrasensitive method for quantitating prion infectivity titre. Nat Commun 2012;3:741. https://doi.org/10.1038/ncomms1730 doi: 10.1038/ncomms1730. [DOI] [PMC free article] [PubMed]
- Halliez S, Reine F, Herzog L, Jaumain E, Haïk S, Rezaei H, et al. Accelerated, spleen-based titration of variant Creutzfeldt–Jakob disease infectivity in transgenic mice expressing human prion protein with sensitivity comparable to that of survival time bioassay. J Virol 2014;88:8678–86. https://doi.org/10.1128/JVI.01118-14 doi: 10.1128/JVI.01118-14. [DOI] [PMC free article] [PubMed]
- Ironside JW. Variant Creutzfeldt–Jakob disease: an update. Folia Neuropathol 2012;50:50–6. [PubMed]
- Bishop MT, Diack AB, Ritchie DL, Ironside JW, Will RG, Manson JC. Prion infectivity in the spleen of a PRNP heterozygous individual with subclinical variant Creutzfeldt–Jakob disease. Brain 2013;136:1139–45. https://doi.org/10.1093/brain/awt032 doi: 10.1093/brain/awt032. [DOI] [PMC free article] [PubMed]
- Cali I, Cohen I, Blevins J, Castellani R, Al-Shekhlee A, Yuan J, et al. The co-existence of PrPSc type 1 and 2 in sporadic Creutzfeldt–Jakob disease affects the phenotype and PrPSc conformation. J Neuropathol Exp Neurol 2009;68:553. https://doi.org/10.1093/brain/awp196 doi: 10.1093/brain/awp196. [DOI]
- Parchi P, Strammiello R, Notari S, Giese A, Langeveld JP, Ladogana A, et al. Incidence and spectrum of sporadic Creutzfeldt–Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification. Acta Neuropathol 2009;118:659–71. https://doi.org/10.1007/s00401-009-0585-1 doi: 10.1007/s00401-009-0585-1. [DOI] [PMC free article] [PubMed]
- Bishop MT, Will RG, Manson JC. Defining sporadic Creutzfeldt–Jakob disease strains and their transmission properties. Proc Natl Acad Sci USA 2010;107:12005–10. https://doi.org/10.1073/pnas.1004688107 doi: 10.1073/pnas.1004688107. [DOI] [PMC free article] [PubMed]
- Uro-Coste E, Cassard H, Simon S, Lugan S, Bilheude JM, Perret-Liaudet A, et al. Beyond PrP9res) type 1/type 2 dichotomy in Creutzfeldt–Jakob disease. PLOS Pathog 2008;4:e1000029. https://doi.org/10.1371/journal.ppat.1000029 doi: 10.1371/journal.ppat.1000029. [DOI] [PMC free article] [PubMed]
- Jansen C, Parchi P, Capellari S, Ibrahim-Verbaas CA, Schuur M, Strammiello R, et al. Human prion diseases in the Netherlands (1998-2009): clinical, genetic and molecular aspects. PLOS ONE 2012;7:e36333. https://doi.org/10.1371/journal.pone.0036333 doi: 10.1371/journal.pone.0036333. [DOI] [PMC free article] [PubMed]
- Mackay G, Yull H, Ironside J, Head M, Knight R. Unravelling the mysteries of sporadic CJD. J Neurol Neurosurg Psychiatry 2013;84:e2. https://doi.org/10.1136/jnnp-2013-306573.18 doi: 10.1136/jnnp-2013-306573.18. [DOI]
- Iwasaki Y, Mimuro M, Yoshida M, Hashizume Y, Kitamoto T, Sobue G. Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt–Jakob disease. Neuropathology 2008;28:51–61. https://doi.org/10.1111/j.1440-1789.2007.00847.x doi: 10.1111/j.1440-1789.2007.00847.x. [DOI] [PubMed]
- Sakai K, Hamaguchi T, Noguchi-Shinohara M, Nozaki I, Takumi I, Sanjo N, et al. Graft-related disease progression in dura mater graft-associated Creutzfeldt–Jakob disease: a cross-sectional study. BMJ Open 2013;3:e003400. https://doi.org/10.1136/bmjopen-2013-003400 doi: 10.1136/bmjopen-2013-003400. [DOI] [PMC free article] [PubMed]
- Béringue V, Le Dur A, Tixador P, Reine F, Lepourry L, Perret-Liaudet A, et al. Prominent and persistent extraneural infection in human PrP transgenic mice infected with variant CJD. PLOS ONE 2008;3:e1419. https://doi.org/10.1371/journal.pone.0001419 doi: 10.1371/journal.pone.0001419. [DOI] [PMC free article] [PubMed]
- Béringue V, Vilotte JL, Laude H. Prion agent diversity and species barrier. Vet Res 2008;39:47. https://doi.org/10.1051/vetres:2008024 doi: 10.1051/vetres:2008024. [DOI] [PubMed]
- Cali I, Miller CJ, Parisi JE, Geschwind MD, Gambetti P, Schonberger LB. Distinct pathological phenotypes of Creutzfeldt–Jakob disease in recipients of prion-contaminated growth hormone. Acta Neuropathol Commun 2015;3:37. https://doi.org/10.1186/s40478-015-0214-2 doi: 10.1186/s40478-015-0214-2. [DOI] [PMC free article] [PubMed]
- Peden AH, Kirkpatrick JRM, Head MW, Ironside JW. Comparison of the in vitro seeding activity of UK iatrogenic and sporadic Creutzfeldt–Jakob disease subtypes by real time quaking induced conversion. Prion 2016;10:S50–S1.
- Bougard D, Brandel JP, Bélondrade M, Béringue V, Segarra C, Fleury H, et al. Detection of prions in the plasma of presymptomatic and symptomatic patients with variant Creutzfeldt–Jakob disease. Sci Transl Med 2016;8:370ra182. https://doi.org/10.1126/scitranslmed.aag1257 doi: 10.1126/scitranslmed.aag1257. [DOI] [PubMed]
- Mead S, Wadsworth JD, Porter MC, Linehan JM, Pietkiewicz W, Jackson GS, et al. Variant Creutzfeldt–Jakob disease with extremely low lymphoreticular deposition of prion protein. JAMA Neurol 2014;71:340–3. https://doi.org/10.1001/jamaneurol.2013.5378 doi: 10.1001/jamaneurol.2013.5378. [DOI] [PMC free article] [PubMed]
- Peden AH, McGuire LI, Appleford NE, Mallinson G, Wilham JM, Orrú CD, et al. Sensitive and specific detection of sporadic Creutzfeldt–Jakob disease brain prion protein using real-time quaking-induced conversion. J Gen Virol 2012;93:438–49. https://doi.org/10.1099/vir.0.033365-0 doi: 10.1099/vir.0.033365-0. [DOI] [PMC free article] [PubMed]
- Douet JY, Lacroux C, Aron N, Head MW, Lugan S, Tillier C, et al. Distribution and quantitative estimates of variant Creutzfeldt–Jakob disease prions in tissues of clinical and asymptomatic patients. Emerging Infect Dis 2017;23:946–56. https://doi.org/10.3201/eid2306.161734 doi: 10.3201/eid2306.161734. [DOI] [PMC free article] [PubMed]
- Bishop MT, Diack A, Cancellotti E, Will R, Manson J. Variant CJD strain remains stable after secondary transmission. Prion 2010;4:143.
- Wadsworth JD, Dalmau-Mena I, Joiner S, Linehan JM, O’Malley C, Powell C, et al. Effect of fixation on brain and lymphoreticular vCJD prions and bioassay of key positive specimens from a retrospective vCJD prevalence study. J Pathol 2011;223:511–18. https://doi.org/10.1002/path.2821 doi: 10.1002/path.2821. [DOI] [PubMed]
- Ritchie DL, Gibson SV, Abee CR, Kreil TR, Ironside JW, Brown P. Blood transmission studies of prion infectivity in the squirrel monkey (Saimiri sciureus): the Baxter study. Transfusion 2016;56:712–21. https://doi.org/10.1111/trf.13422 doi: 10.1111/trf.13422. [DOI] [PubMed]
- Moore RA, Head MW, Ironside JW, Ritchie DL, Zanusso G, Choi YP, et al. The distribution of prion protein allotypes differs between sporadic and iatrogenic Creutzfeldt–Jakob disease patients. PLOS Pathog 2016;12:e1005416. https://doi.org/10.1371/journal.ppat.1005416 doi: 10.1371/journal.ppat.1005416. [DOI] [PMC free article] [PubMed]
- House of Commons, The Science and Technology Committee. After the Storm? UK Blood Safety and the Risk of Variant Creutzfeldt–Jakob Disease. Second Report of Session. 2014. URL: https://publications.parliament.uk/pa/cm201415/cmselect/cmsctech/327/32706.htm#a7 (accessed 15 March 2018).
- Lehmann S, Pastore M, Rogez-Kreuz C, Richard M, Belondrade M, Rauwel G, et al. New hospital disinfection processes for both conventional and prion infectious agents compatible with thermosensitive medical equipment. J Hosp Infect 2009;72:342–50. https://doi.org/10.1016/j.jhin.2009.03.024 doi: 10.1016/j.jhin.2009.03.024. [DOI] [PubMed]
- Lemmer K, Mielke M, Kratzel C, Joncic M, Oezel M, Pauli G, Beekes M. Decontamination of surgical instruments from prions. II. In vivo findings with a model system for testing the removal of scrapie infectivity from steel surfaces. J Gen Virol 2008;89:348–58. https://doi.org/10.1099/vir.0.83396-0 doi: 10.1099/vir.0.83396-0. [DOI] [PubMed]
- Rogez-Kreuz C, Yousfi R, Soufflet C, Quadrio I, Yan ZX, Huyot V, et al. Inactivation of animal and human prions by hydrogen peroxide gas plasma sterilization. Infect Control Hosp Epidemiol 2009;30:769–77. https://doi.org/10.1086/598342 doi: 10.1086/598342. [DOI] [PubMed]
- Belondrade M, Nicot S, Béringue V, Coste J, Lehmann S, Bougard D. Rapid and highly sensitive detection of variant Creutzfeldt–Jakob disease abnormal prion protein on steel surfaces by protein misfolding cyclic amplification: application to prion decontamination studies. PLOS ONE 2016;11:e0146833. https://doi.org/10.1371/journal.pone.0146833 doi: 10.1371/journal.pone.0146833. [DOI] [PMC free article] [PubMed]
- Lawson VA, Stewart JD, Masters CL. Enzymatic detergent treatment protocol that reduces protease-resistant prion protein load and infectivity from surgical-steel monofilaments contaminated with a human-derived prion strain. J Gen Virol 2007;88:2905–14. https://doi.org/10.1099/vir.0.82961-0 doi: 10.1099/vir.0.82961-0. [DOI] [PubMed]
- Beekes M, Lemmer K, Thomzig A, Joncic M, Tintelnot K, Mielke M. Fast, broad-range disinfection of bacteria, fungi, viruses and prions. J Gen Virol 2010;91:580–9. https://doi.org/10.1099/vir.0.016337-0 doi: 10.1099/vir.0.016337-0. [DOI] [PubMed]
- Bellon A, Comoy E, Simoneau S, Mornac S, Dehen C, Perrin A, et al. Decontamination of prions in a plasma product manufacturing environment. Transfusion 2014;54:1028–36. https://doi.org/10.1111/trf.12381 doi: 10.1111/trf.12381. [DOI] [PubMed]
- Fichet G, Antloga K, Comoy E, Deslys JP, McDonnell G. Prion inactivation using a new gaseous hydrogen peroxide sterilisation process. J Hosp Infect 2007;67:278–86. https://doi.org/10.1016/j.jhin.2007.08.020 doi: 10.1016/j.jhin.2007.08.020. [DOI] [PubMed]
- Hervé R, Kong M, Comoy E, Deslys JP, Keevil B. Cold atmospheric plasma for the decontamination of reusable surgical instruments. Prion 2010;4:218.
- Hervé R, Keevil CW. Current limitations about the cleaning of luminal endoscopes. J Hosp Infect 2013;83:22–9. https://doi.org/10.1016/j.jhin.2012.08.008 doi: 10.1016/j.jhin.2012.08.008. [DOI] [PubMed]
- Howlin RP, Khammo N, Secker T, McDonnell G, Keevil CW. Application of a fluorescent dual stain to assess decontamination of tissue protein and prion amyloid from surgical stainless steel during simulated washer-disinfector cycles. J Hosp Infect 2010;75:66–71. https://doi.org/10.1016/j.jhin.2009.12.023 doi: 10.1016/j.jhin.2009.12.023. [DOI] [PubMed]
- Edgeworth JA, Sicilia A, Linehan J, Brandner S, Jackson GS, Collinge J. A standardized comparison of commercially available prion decontamination reagents using the standard steel-binding assay. J Gen Virol 2011;92:718–26. https://doi.org/10.1099/vir.0.027201-0 doi: 10.1099/vir.0.027201-0. [DOI] [PMC free article] [PubMed]
- Jackson GS, McKintosh E, Flechsig E, Prodromidou K, Hirsch P, Linehan J, et al. An enzyme-detergent method for effective prion decontamination of surgical steel. J Gen Virol 2005;86:869–78. https://doi.org/10.1099/vir.0.80484-0 doi: 10.1099/vir.0.80484-0. [DOI] [PubMed]
- Peretz D, Supattapone S, Giles K, Vergara J, Freyman Y, Lessard P, et al. Inactivation of prions by acidic sodium dodecyl sulfate. J Virol 2006;80:322–31. https://doi.org/10.1128/JVI.80.1.322-331.2006 doi: 10.1128/JVI.80.1.322-331.2006. [DOI] [PMC free article] [PubMed]
- Giles K, Supattapone S, Peretz D, Glidden DV, Baron H, Prusiner SB. Disinfection of prions. New Biocides Dev 2007;967:52–74. https://doi.org/10.1021/bk-2007-0967.ch003 doi: 10.1021/bk-2007-0967.ch003. [DOI]
- Baxter HC, Campbell GA, Whittaker AG, Jones AC, Aitken A, Simpson AH, et al. Elimination of transmissible spongiform encephalopathy infectivity and decontamination of surgical instruments by using radio-frequency gas-plasma treatment. J Gen Virol 2005;86:2393–9. https://doi.org/10.1099/vir.0.81016-0 doi: 10.1099/vir.0.81016-0. [DOI] [PubMed]
- Giles K, Glidden DV, Beckwith R, Seoanes R, Peretz D, DeArmond SJ, Prusiner SB. Resistance of bovine spongiform encephalopathy (BSE) prions to inactivation. PLOS Pathog 2008;4:e1000206. https://doi.org/10.1371/journal.ppat.1000206 doi: 10.1371/journal.ppat.1000206. [DOI] [PMC free article] [PubMed]
- Hervé RC, Keevil CW. Persistent residual contamination in endoscope channels; a fluorescence epimicroscopy study. Endoscopy 2016;48:609–16. https://doi.org/10.1055/s-0042-105744 doi: 10.1055/s-0042-105744. [DOI] [PubMed]
- Baxter RL, Baxter HC, Campbell GA, Grant K, Jones A, Richardson P, Whittaker G. Quantitative analysis of residual protein contamination on reprocessed surgical instruments. J Hosp Infect 2006;63:439–44. https://doi.org/10.1016/j.jhin.2006.03.011 doi: 10.1016/j.jhin.2006.03.011. [DOI] [PubMed]
- Murdoch H, Taylor D, Dickinson J, Walker JT, Perrett D, Raven ND, Sutton JM. Surface decontamination of surgical instruments: an ongoing dilemma. J Hosp Infect 2006;63:432–8. https://doi.org/10.1016/j.jhin.2006.02.015 doi: 10.1016/j.jhin.2006.02.015. [DOI] [PubMed]
- Lipscomb IP, Sihota AK, Botham M, Harris KL, Keevil CW. Rapid method for the sensitive detection of protein contamination on surgical instruments. J Hosp Infect 2006;62:141–8. https://doi.org/10.1016/j.jhin.2005.07.008 doi: 10.1016/j.jhin.2005.07.008. [DOI] [PubMed]
- Lipscomb IP, Sihota AK, Keevil CW. Comparative study of surgical instruments from sterile-service departments for presence of residual gram-negative endotoxin and proteinaceous deposits. J Clin Microbiol 2006;44:3728–33. https://doi.org/10.1128/JCM.01280-06 doi: 10.1128/JCM.01280-06. [DOI] [PMC free article] [PubMed]
- Smith A, Winter S, Lappin D, Sherriff A, McIvor I, Philp P, et al. Reducing the risk of iatrogenic CJD by improving the cleaning of neurosurgical instruments. J Hosp Infect 2018;100:e70–e76. https://doi.org/10.1016/j.jhin.2018.03.001 doi: 10.1016/j.jhin.2018.03.001. [DOI] [PubMed]
- Baxter HC, Campbell GA, Richardson PR, Jones AC, Whittle IR, Casey M, et al. Surgical instrument decontamination: efficacy of introducing an argon:oxygen RF gas-plasma cleaning step as part of the cleaning cycle for stainless steel instruments. IEEE Trans Plasma Sci, IEEE Nucl Plasma Sci Soc 2006;34:1337–44. https://doi.org/10.1109/TPS.2006.878387 doi: 10.1109/TPS.2006.878387. [DOI]
- Baxter HC, Jones AC, Baxter RL. Application of epifluorescence scanning for monitoring the efficacy of protein removal by RF gas-plasma decontamination. Prion 2009;4:204–5.
- Lipscomb IP, Pinchin H, Collin R, Keevil CW. Effect of drying time, ambient temperature and pre-soaks on prion-infected tissue contamination levels on surgical stainless steel: concerns over prolonged transportation of instruments from theatre to central sterile service departments. J Hosp Infect 2007;65:72–7. https://doi.org/10.1016/j.jhin.2006.09.025 doi: 10.1016/j.jhin.2006.09.025. [DOI] [PubMed]
- Secker TJ, Pinchin HE, Hervé RC, Keevil CW. Efficacy of humidity retention bags for the reduced adsorption and improved cleaning of tissue proteins including prion-associated amyloid to surgical stainless steel surfaces. Biofouling 2015;31:535–41. https://doi.org/10.1080/08927014.2015.1067686 doi: 10.1080/08927014.2015.1067686. [DOI] [PubMed]
- Secker TJ, Hervé R, Keevil CW. Adsorption of prion and tissue proteins to surgical stainless steel surfaces and the efficacy of decontamination following dry and wet storage conditions. J Hosp Infect 2011;78:251–5. https://doi.org/10.1016/j.jhin.2011.03.021 doi: 10.1016/j.jhin.2011.03.021. [DOI] [PubMed]
- Secker TJ, Hervea R, Keevil CW. Wet versus dry: do environmental conditions have an effect on prion decontamination? Prion 2010;4:213–4.
- Department of Health and Social Care (DHSC). CFPP-01-01: Decontamination of Surgical Instruments (HTM 01-01) Health Technical Memorandum (HTM) 01-01: Management and Decontamination of Surgical Instruments (Medical Devices) Used in Acute Care. Part B: Common Elements. London: DHSC; 2016. URL: www.gov.uk/government/publications/management-and-decontamination-of-surgical-instruments-used-in-acute-care (accessed 8 January 2020).
- Bonda DJ, Manjila S, Mehndiratta P, Khan F, Miller BR, Onwuzulike K, et al. Human prion diseases: surgical lessons learned from iatrogenic prion transmission. Neurosurg Focus 2016;41:E10. https://doi.org/10.3171/2016.5.FOCUS15126 doi: 10.3171/2016.5.FOCUS15126. [DOI] [PMC free article] [PubMed]
- Rutala WA, Weber DJ, Society for Healthcare Epidemiology of America. Guideline for disinfection and sterilization of prion-contaminated medical instruments. Infect Control Hosp Epidemiol 2010;31:107–17. https://doi.org/10.1086/650197 doi: 10.1086/650197. [DOI] [PubMed]
- Dickinson J, Murdoch H, Dennis MJ, Hall GA, Bott R, Crabb WD, et al. Decontamination of prion protein (BSE301V) using a genetically engineered protease. J Hosp Infect 2009;72:65–70. https://doi.org/10.1016/j.jhin.2008.12.007 doi: 10.1016/j.jhin.2008.12.007. [DOI] [PubMed]
- Fichet G, Comoy E, Duval C, Antloga K, Dehen C, Charbonnier A, et al. Novel methods for disinfection of prion-contaminated medical devices. Lancet 2004;364:521–6. https://doi.org/10.1016/S0140-6736(04)16810-4 doi: 10.1016/S0140-6736(04)16810-4. [DOI] [PubMed]
- Rochefort F. [The role of detergents in prevention of transmission of Creutzfeldt–Jakob disease.] Zentralsterilisation 2010;18:395–400.
- Hervé R, Secker TJ, Keevil CW. Current risk of iatrogenic Creutzfeld-Jakob disease in the UK: efficacy of available cleaning chemistries and reusability of neurosurgical instruments. J Hosp Infect 2010;75:309–13. https://doi.org/10.1016/j.jhin.2010.01.024 doi: 10.1016/j.jhin.2010.01.024. [DOI] [PubMed]
- National Institute for Health and Care Excellence. Adoption and Impact Programme. IPG196 Adoption Scoping Report. 2016. (unpublished)
- Department of Health and Social Care. Minimise Transmission Risk of CJD and vCJD in Healthcare Settings. 2012. URL: www.gov.uk/government/publications/guidance-from-the-acdp-tse-risk-management-subgroup-formerly-tse-working-group (accessed 23 January 2018).
- Department of Health and Social Care, Economics and Operational Research Division (EOR4). Risk Assessment for Transmission of vCJD via Surgical Instruments: A Modelling Approach and Numerical Scenarios. London: Department of Health and Social Care, Economics and Operational Research Division; 2001.
- Bird SM, Merrall EL, Ward HJ, Will RG. Survival and re-operation rates after neurosurgical procedures in Scotland: implications for targeted surveillance of sub-clinical variant Creutzfeldt–Jakob disease. Neuroepidemiology 2009;33:1–11. https://doi.org/10.1159/000209281 doi: 10.1159/000209281. [DOI] [PubMed]
- National Institute for Health and Care Excellence (NICE). Patient Safety and Reduction of Risk of Transmission of Creutzfeldt–Jakob (CJD) via Interventional Procedures [IPG196]. London: NICE; 2006.
- Garske T, Ward HJ, Clarke P, Will RG, Ghani AC. Factors determining the potential for onward transmission of variant Creutzfeldt–Jakob disease via surgical instruments. J R Soc Interface 2006;3:757–66. https://doi.org/10.1098/rsif.2006.0142 doi: 10.1098/rsif.2006.0142. [DOI] [PMC free article] [PubMed]
- National Institute for Health and Care Excellence. Guide to the Methods of Technology Appraisal 2013. 2013. URL: www.nice.org.uk/article/pmg9/ (accessed 19 March 2018). [PubMed]
- Office for National Statistics. National Life Tables: United Kingdom. 2017. URL: www.ons.gov.uk/peoplepopulationandcommunity/birthsdeathsandmarriages/lifeexpectancies/datasets/nationallifetablesunitedkingdomreferencetables (accessed 13 January 2019).
- Curtis L, Burns A. Unit Costs of Health and Social Care 2017. Canterbury: PSSRU, University of Kent; 2017.
- Curtis L. Unit Costs of Health and Social Care 2014. Canterbury: PSSRU, University of Kent; 2014.
- Barnett F, McLean G. Care management of Creutzfeldt–Jakob Disease within the United Kingdom. J Nurs Manag 2005;13:111–18. https://doi.org/10.1111/j.1365-2934.2005.00449.x doi: 10.1111/j.1365-2934.2005.00449.x. [DOI] [PubMed]
- National Institute for Health and Care Excellence (NICE). Interim Process and Methods of the Highly Specialised Technologies Programme Updated to Reflect 2017 Changes. London: NICE; 2017. [PubMed]