Hostname: page-component-78c5997874-g7gxr Total loading time: 0 Render date: 2024-11-04T21:23:17.228Z Has data issue: false hasContentIssue false

Creutzfeldt–Jakob disease and ENT

Published online by Cambridge University Press:  22 October 2013

N Sethi*
Affiliation:
Department of Otolaryngology – Head and Neck Surgery, Mid Yorkshire NHS Trust, Pinderfields Hospital, Wakefield, UK
J Kane
Affiliation:
Department of Otolaryngology – Head and Neck Surgery, Mid Yorkshire NHS Trust, Pinderfields Hospital, Wakefield, UK
L Condon
Affiliation:
Department of Otolaryngology – Head and Neck Surgery, Mid Yorkshire NHS Trust, Pinderfields Hospital, Wakefield, UK
*
Address for correspondence: Mr Neeraj Sethi, 3 Victoria St, Leeds LS7 4PA, UK Fax: +44 (0)113 269 8885 E-mail: [email protected]

Abstract

Objective:

This review addresses Creutzfeldt–Jakob disease in the context of ENT, and aims to summarise the relevant history, pathophysiology and implications for contemporary practice.

Overview:

Creutzfeldt–Jakob disease is a rare, fatal, neurodegenerative disorder. It is a prion disease with four different subtypes that can only be definitively diagnosed post-mortem. The main implications for the ENT surgeon lie in the risk of iatrogenic transmission. The three facets of assessing individual patient risk are: patient history; tissue infectivity; and procedure infectivity.

Conclusion:

This is a controversial area in medicine, and ENT in particular. This review highlights a clinically applicable approach for everyday use.

Type
Review Articles
Copyright
Copyright © JLO (1984) Limited 2013 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1Lumley, JSP. The impact of Creutzfeldt-Jakob disease on surgical practice. Ann R Coll Surg Engl 2008;90:91–4CrossRefGoogle ScholarPubMed
2Creutzfeldt, HG. A peculiar focal illness of the central nervous system [in German]. Z Gesamte Neurol Psychiatr 1920;57:118CrossRefGoogle Scholar
3Klatso, I, Gajdusek, DC, Zigas, V. Pathology of kuru. Lab Invest 1959;8:799847Google Scholar
4Hadlow, WJ. Scrapie and kuru. Lancet 1959;ii:289–90CrossRefGoogle Scholar
5Frosh, A. Prions and the ENT surgeon. J Laryngol Otol 1999;113:1064–7CrossRefGoogle ScholarPubMed
6Gajdusek, DC, Gibbs, CJ, Alpers, M. Experimental transmission of kuru-like syndrome to chimpanzees. Nature 1966;209:794–6CrossRefGoogle ScholarPubMed
7Pruisner, SB. Novel proteinaceous particles cause scrapie. Science 1982;216:136–44CrossRefGoogle Scholar
8Mocsny, N. The spongiform encephalopathies: prion diseases. J Neurosci Nurs 1998;30:303–6CrossRefGoogle ScholarPubMed
9Antiloga, K, Meszaros, J, Malchesky, PS, McDonnel, G. Prion disease and medical devices. ASAIO J 2000;46:S69–72Google Scholar
10Weissman, C. Molecular biology of transmissible spongiform encephalopathies. Prog Brain Res 1995;105:1522CrossRefGoogle Scholar
11Doerr, HW, Cinatl, J, Sturmer, M, Rabenau, HF. Prions and orthopedic surgery. Infection 2003;31:163–71CrossRefGoogle ScholarPubMed
12Advisory Committee on Dangerous Pathogens and the Spongiform Encephalopathy Advisory Committee. Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection. London: Department of Health, 2007Google Scholar
13Armitage, WJ, Tullo, AB, Ironside, JW. Risk of Creutzfeldt-Jakob disease transmission by ocular surgery and tissue transplantation. Eye 2009;23:1926–30CrossRefGoogle ScholarPubMed
14Liao, YC, Lebo, RV, Clawson, GA, Smuckler, EA. Human prion protein cDNA: molecular cloning, chromosomal mapping and biological implications. Science 1986;233:365–7CrossRefGoogle ScholarPubMed
15Will, RG, Ironside, JW, Zeidler, M, Cousens, SN, Estibeiro, K, Alperovitch, A et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996;347:921–5CrossRefGoogle ScholarPubMed
16The National Creutzfeldt-Jakob Disease Research and Surveillance Unit figures. In: http://www.cjd.ed.ac.uk/data.html [28 September 2013]Google Scholar
17Hilton, 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;203:733–9CrossRefGoogle ScholarPubMed
18Clewley, JP, Kelly, CM, Andrews, N, Vogliqi, K, Malinson, G, Kaisar, M et al. Prevalence of disease related prion protein in anonymous tonsil specimens in Britain: cross sectional opportunistic survey. BMJ 2009;338:b1442CrossRefGoogle ScholarPubMed
19Brown, P, Brandell, JP, Sato, T, Nakamura, Y, MacKenzie, J, Will, RG et al. Iatrogenic Creutzfeldt-Jakob disease, final assessment. Emerg Infect Dis 2012;18:901–7CrossRefGoogle ScholarPubMed
20World Health Organization. WHO Guidelines on Tissue Infectivity Distribution in Transmissible Encephalopathies. Geneva: World Health Organisation, 2006Google Scholar
21World Health Organization. WHO Infection Control Guidelines for Transmissible Spongiform Encephalopathies. Geneva: World Health Organization, 2000Google Scholar
22Thadani, V, Penar, PL, Partington, J, Kalb, R, Janssen, R, Schonberger, LB et al. Creutzfeldt-Jakob disease probably acquired from a cadaveric dura mater graft. J Neurosurg 1988;69:766–9CrossRefGoogle ScholarPubMed
23Tange, RA, Troost, D, Limburg, M. Progressive fatal dementia in a patient who received homograft tissue for tympanic membrane closure. Eur Arch Otorhinolaryngol 1990;247:199201CrossRefGoogle Scholar
24Lubbe, D, Fagan, JJ. Revisiting the risks involved in using homograft ossicles in otological surgery. J Laryngol Otol 2008;122:111–15CrossRefGoogle ScholarPubMed
25Minatogawa, T, Kumoi, T. Problems in utility and safety of otological allografts. Transplant Proc 1999;31:2036–7CrossRefGoogle ScholarPubMed
26Hotz, MA, Hausler, RH. Ossicle homografts revisited. Laryngoscope 2003;113:1274–5Google ScholarPubMed
27Department of Health. Patient Safety and Reduction of Risk of Transmission of Creutzfeldt-Jakob Disease (CJD) Via Interventional Procedures. NICE Interventional Procedure Guidance 196. London: Department of Health, 2006Google Scholar
28Rabano, A, de Pedro-Cuesta, J, Molbak, K, Siden, A, Calero, M, Laursen, H et al. Tissue classification for the epidemiological assessment of surgical transmission of sporadic Creutzfeldt-Jakob disease: a proposal on hypothetical risk levels. BMC Public Health 2005;5:9CrossRefGoogle ScholarPubMed
29Rutala, WA, Weber, D. Guideline for disinfection and sterilization of prion-contaminated medical instruments. Infect Control Hosp Epidemiol 2010;31:107–16CrossRefGoogle ScholarPubMed
30Axon, ATR, Beilenhoff, MG, Bramble, S, Ghosh, A, Kruse, GE, McDonnell, C et al. Variant Creutzfeldt-Jakob disease (vCJD) and gastrointestinal endoscopy. Endoscopy 2001;33:1070–80CrossRefGoogle ScholarPubMed
31Advisory Committee on Dangerous Pathogens' Transmissible Spongiform Encephalopathy Risk Management Subgroup. Minimise Transmission Risk of CJD and vCJD in Healthcare Setting by Advisory Committee on Dangerous Pathogens' Transmissible Spongiform Encephalopathy (ACDP TSE) Risk Management Subgroup. London: Department of Health, 2013Google Scholar
32Zanusso, G, Ferrari, S, Cardone, F, Zampieri, P, Gelati, M, Fiorini, M et al. Detection of pathologic prion protein in the olfactory epithelium in sporadic Creutzfeldt-Jakob disease. N Engl J Med. 2003;348:711–9CrossRefGoogle ScholarPubMed
33Department of Health. £200 Million for NHS Equipment to Protect Patients Against Possible Variant CJD Risk. London: Department of Health, 2001Google Scholar
34Maini, S, Waine, E, Evans, K. Increased post-tonsillectomy haemorrhage with disposable instruments: an audit cycle. Clin Otolaryngol 2002;27:175–8CrossRefGoogle ScholarPubMed
35Chicken, DW, Sivaji, N, Kanegaonkar, RG. A warning to users of disposable tonsillectomy instruments. J Laryngol Otol 2001;115:686–7Google ScholarPubMed
36Tomkinson, A, Owens, D, Phillips, E, Simmons, M, Davies, E, Harrison, W et al. All Wales Annual Tonsillectomy Surveillance Report. Cardiff: National Public Health Service for Wales, 2005Google Scholar
37O'Flynn, P, Silva, S, Kothari, P, Persaud, R. A multi-centre audit of single-use surgical instruments for tonsillectomy and adenoidectomy. Ann R Coll Surg Engl 2007;89:616–23CrossRefGoogle Scholar
38Scott, A, De, R, Sadek, SA, Garrido, MC, Courtney-Harris, RG. Temporal bone dissection: a potential route for prion transmission? J Laryngol Otol 2001;115:374–5CrossRefGoogle Scholar