Evaluation of the Australian CJD Surveillance System
DOI:
https://doi.org/10.33321/cdi.2002.26.24Keywords:
CJD, Creutzfeldt-Jakob disease, Australian National Creutzfeldt- Jakob Disease Registry, evaluation, surveillanceAbstract
An evaluation of the surveillance capacity of the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) was undertaken. It focused on the ability of the Registry to detect CJD in Australia and, in particular, to identify cases that require public health responses. The Registry relies on a complex reporting system and staff with expertise to accurately identify and classify cases of CJD. The Registry satisfies the criteria of flexibility and acceptability and has a high positive predictive value and representativeness. The sensitivity of the system could not be evaluated, as the rarity of the condition precludes an independent assessment of the incidence of CJD, but the incidence of CJD is comparable to that found in other countries. The time required to establish a definite diagnosis of CJD is approximately 2 months, impacting negatively on the timeliness of the system. In order to maximise the likelihood of detecting all cases of CJD in Australia in a timely fashion, suggestions are made for improving the system's sensitivity and timeliness of reporting as well as for using methods that allow meaningful comparisons of incidence between populations with different age structures. Comm Dis Intell 2002;26:265-272.
Downloads
References
Davanipour Z, Alter M, Sobel E. Creutzfeldt-Jakob disease. Neurology Clinics 1986;4:415-426.
Collins S, Masters CL. Transmissibility of Creutzfeldt-Jakob disease and related disorders. Sci Prog 1995;78:217-227.
Alter M. How is Creutzfeldt-Jakob disease acquired? Neuroepidemiology 2000;19:55-61.
Brown P, Preece M, Brandel J, Sato T, McShane L, Zerr I, et al. Iatrogenic Creutzfeldt-Jakob disease at the Millennium. Neurology 2000;55:1075-1081.
Collins S, Masters CL. Iatrogenic and zoonotic Creutzfeldt-Jakob disease; the Australian perspective. Med J Aust 1996;164:598-602.
Newcombe RL. Neurosurgery and iatrogenic transmission of Creutzfeldt-Jakob disease. Med J Aust 1996;164:603-604.
Hornabrook RW. Kuru. Med J Aust 1968;2:35-36.
Will 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;347:921-925.
Klaucke DN, Buehler JW, Thacker SB, Gibson Parrish R, Trowbridge FL, Berkelman RL. Guidelines for evaluating surveillance systems. Atlanta: US Centers for Disease Control, 1988.
World Health Organization. Protocol for the Assessment of National Communicable Disease Surveillance and Response Systems. WHO/CDS/ISR/2001.2.
Centers for Disease Control and Prevention. Updated guidelines for evaluating public health surveillance systems: recommendations from the guidelines working group. MMWR 2001;50( no RR-13):1-35.
Boyd A, Fletcher A, Lee JS, Lewis V, Masters CL, Collins SJ. Transmissible spongiform encephalopathies in Australia. Commun Dis Intell 2001;25:248-52.
Brandel JP, Delasniere-Laupretre N, Laplanche JL, Hauw JJ, Alperovitch A. Diagnosis of Creutzfeldt-Jakob disease: effect of clinical criteria on incidence estimates. Neurology 2000;322:841-844.
Hauw JJ, Sazdovitch V, Laplanche JL, Peoc'h K, Kopp N, Kemeny J, et al. Neuropathologic variants of sporadic Creutzfeldt-Jakob disease and codon 129 of PrP gene. Neurology 2000;54:1641-1646.
UK Department of Health. Monthly Creutzfeldt-Jakob disease figures. www.doh.gov.UK/cjd/stats, 2001.
Collins S, Boyd A, Fletcher A, Gonzales MF, McLean CA, Masters CL. Recent advances in the pre-mortem diagnosis of Creutzfeldt-Jakob disease. J Clin Neurosci 2000;7:195-202.
Collins S, Boyd A, Fletcher A, Gonzales M, McLean CA, Byron K, Masters CL. Creutzfeldt-Jakob disease: diagnostic utility of 14.3.3 Protein immunodetection in cerebrospinal fluid. J Clin Neurosci 2000;7:203-208.
Chapman T, McKeel DW, Morris JC. Misleading results with the 14-3-3 assay for the diagnosis of Creutzfeldt-Jakob disease. Neurology 2000;57:1058-1063.
Hill AF, Butterworth RJ, Joiner S, Jackson G, Rossor MN, Thomas DJ, et al. Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples. Lancet 1999;353:183-189.
Cohen FE. Protein misfolding and prion diseases. J Mol Biol 1999;293:317-320.
Alperovitch A, Zerr I, Pocchiari M, Mitrova E, Cuesta JD, Hegyi I, et al. Codon 129 prion protein genotype and sporadic Creutzfeldt-Jakob disease. Lancet 1999;353:1673-1674.
Parchi P, Peterson RB, Gambetti P. New topics in familial prion diseases. Seminars in virology 1996;181-187.
Australian Government Publishing Service. Report of the inquiry into the use of pituitary-derived hormones in Australia and Creutzfeldt-Jakob disease. Australian Government Publishing Service, Canberra, June 1994.
D'Aignaux JH, Laplanche JL, Delasnerie-Laupretre N, Brandel JP, Peoc'h K, Salomon D, et al. Trends in mortality from sporadic Creutzfeldt-Jakob disease in France 1992-1997. J Neurol Neurosurg Psychiatry 2000;68:787-789.
Will RG, Alperovitch A, Poser S, Pocchiari M, Hofman A, Mitrova E, et al. Descriptive epidemiology of Creutzfeldt-Jakob disease in six European countries, 1993-1995. Ann Neurol 1998;43:763-767.
Holman RC, Khan AS, Kent J, Strine TW, Schonberger LB. Epidemiology of Creutzfeldt-Jakob disease in the United States, 1979-1990 - analysis of national mortality data. Neuroepidemiology 1995;14:174-181.
Holman RC, Khan AS, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States, 1979-1994 - using national mortality data to assess the possible occurrence of variant cases. Emerg Infect Dis 1996,2:333-337.
Breslow NE, Day NE. Statistical methods in cancer research. Vol. 2. Lyon: WHO, 1987.
Downloads
Published
How to Cite
Issue
Section
Categories
License
Copyright (c) 2002 Communicable Diseases Intelligence

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
