Creutzfeldt-Jakob disease surveillance in Australia: update to 31 December 2020

Authors

  • Christiane Stehmann The Florey Institute, The University of Melbourne, Victoria, 3010, Australia
  • Matteo Senesi Department of Medicine, The University of Melbourne, Victoria, 3010, Australia
  • Shannon Sarros The Florey Institute, The University of Melbourne, Victoria, 3010, Australia
  • Amelia McGlade The Florey Institute, The University of Melbourne, Victoria, 3010, Australia
  • Victoria Lewis Department of Medicine, The University of Melbourne, Victoria, 3010, Australia
  • Marion Simpson The Florey Institute, The University of Melbourne, Victoria, 3010, Australia
  • Genevieve Klug The Florey Institute, The University of Melbourne, Victoria, 3010, Australia
  • Catriona McLean The Florey Institute, The University of Melbourne, Victoria, 3010, Australia; The Alfred Hospital, Department of Anatomical Pathology, 55 Commercial Rd, Melbourne Vic 3004 Australia
  • Colin L Masters The Florey Institute, The University of Melbourne, Victoria, 3010, Australia
  • Steven Collins Department of Medicine, The University of Melbourne, Victoria, 3010, Australia

DOI:

https://doi.org/10.33321/cdi.2021.45.38

Keywords:

Creutzfeldt-Jakob disease, prion disease, transmissible spongiform encephalopathy, disease surveillance

Abstract

NOTE: The details of the number of ‘incomplete’ suspect case notifications, in the second paragraph of the report’s Abstract are incorrect. The corrected Abstract is published as Erratum: Creutzfeldt-Jakob disease surveillance in Australia (https://doi.org/10.33321/cdi.2021.45.40)

Nationwide surveillance of Creutzfeldt-Jakob disease and other human prion diseases is performed by the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR). National surveillance encompasses the period since 1 January 1970, with prospective surveillance occurring from 1 October 1993. Over this prospective surveillance period, considerable developments have occurred in pre-mortem diagnostics; in the delineation of new disease subtypes; and in a heightened awareness of prion diseases in healthcare settings. Surveillance practices of the ANCJDR have evolved and adapted accordingly. This report summarises the activities of the ANCJDR during 2020.
Since the ANCJDR began offering diagnostic cerebrospinal fluid (CSF) 14-3-3 protein testing in Australia in September 1997, the annual number of referrals has steadily increased. In 2020, 510 domestic CSF specimens were referred for 14-3-3 protein testing and 85 persons with suspected human prion disease were formally added to the national register. As of 31 December 2020, just over half (44 cases) of the 85 suspect case notifications remain classified as ‘incomplete’; 27 cases were excluded through either detailed clinical follow-up (9 cases) or neuropathological examination (18 cases); 18 cases were classified as ‘definite’ and eleven as ‘probable’ prion disease. For 2020, sixty percent of all suspected human-prion-disease-related deaths in Australia underwent neuropathological examination. No cases of variant or iatrogenic CJD were identified.
The SARS-CoV-2 pandemic did not affect prion disease surveillance outcomes in Australia.

 

Downloads

Download data is not yet available.

References

Klug GM, Boyd A, Zhao T, Stehmann C, Simpson M, McLean CA et al. Surveillance for Creutzfeldt-Jakob disease in Australia: update to December 2012. Commun Dis Intell Q Rep. 2013;37(2):E115–20.

Allars M. Report of the inquiry into the use of pituitary derived hormones in Australia and Creutzfeldt-Jakob disease. Canberra: AGPS, 1994.

Zerr I, Kallenberg K, Summers DM, Romero C, Taratuto A, Heinemann U et al. Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease. Brain. 2009;132(10):2659–68.

Creutzfeldt-Jakob Disease International Surveillance Network: Surveillance Data. [Internet.] Solna: European Centre for Disease Prevention and Control; 2021. Available from: https://www.eurocjd.ed.ac.uk/data_tables.

Uttley L, Carroll C, Wong R, Hilton DA, Stevenson M. Creutzfeldt-Jakob disease: a systematic review of global incidence, prevalence, infectivity, and incubation. Lancet Infect Dis. 2020;20(1):e2–10.

Klug GM, Wand H, Simpson M, Boyd A, Law M, Masters CL et al. Intensity of human prion disease surveillance predicts observed disease incidence. J Neurol Neurosurg Psychiatry. 2013;84(2):1372–7.

Australian Bureau of Statistics. National, state and territory population - reference period June 2020. [Webpage.] Canberra: Australian Bureau of Statistics; 17 December 2020. [Accessed on 1 February 2021.] Available from: https://www.abs.gov.au/statistics/people/population/national-state-and-territory-population/jun-2020.

Glatzel M, Rogivue C, Ghani A, Streffer JR, Amsler L, Aguzzi A. Incidence of Creutzfeldt-Jakob disease in Switzerland. Lancet. 2002;360(9327):139–41.

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(2);493–501.

Collins S, Boyd A, Fletcher A, Kaldor J, Hill A, Farish S et al. Creutzfeldt-Jakob disease cluster in an Australian rural city. Ann Neurol. 2002;52(1):115–8.

Stevenson M, Uttley L, Oakley JE, Carroll C, Chick SE and Wong,R. Interventions to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease: a cost-effective modelling review. Health Technol Assess. 2020;24(11):1–150.

The Royal College of Pathologists of Australasia Autopsy Working Party. The decline of the hospital autopsy: a safety and quality issue for healthcare in Australia. Med J Aust. 2004;180(6):281–5.

Jackett L, McLean C. Hospital autopsy audit: discordant primary clinical diagnoses are found in 20% of cases in a reducing autopsy case load. Selection bias or significant findings? Pathology. 2015;47(6):499–502.

Panegyres PK, Stehmann C, Klug GM, Masters CL, Collins S. Prion disease in Indigenous Australians. Intern Med J. 2020. doi: https://doi.org10.1111/imj.14835.

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(3):292–4..

Alnakhli SH, Wand H, Law M, Sarros S, Stehmann C, Senesi M et al. Intra-cerebral haemorrhage but not neurodegenerative disease appears over-represented in deaths of Australian cadaveric pituitary hormone recipients. J Clin Neurosci. 2020;81:78–82.

Cali I, Cohen ML, Haїk S, Parchi P, Giaccone G, Collins SJ et al. Iatrogenic Creutzfeldt-Jakob disease with Amyloid-β pathology: an international study. Acta Neuropathol Commun. 2018;6(1):5. doi: https://doi.org/10.1186/s40478-017-0503-z.

Purro SA, Farrow MA, Linehan J, Nazari T, Thomas DX, Chen Z et al. Transmission of amyloid-β protein pathology from cadaveric pituitary growth hormone. Nature. 2018;564(7736):415–9. doi: https://doi.org/10.1038/s41586-018-0790-y.

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(5):671–9. doi: https://doi.org/10.1007/s00401-018-1822-2.

Downloads

Additional Files

Published

20/07/21

How to Cite

Stehmann, Christiane, Matteo Senesi, Shannon Sarros, Amelia McGlade, Victoria Lewis, Marion Simpson, Genevieve Klug, Catriona McLean, Colin L Masters, and Steven Collins. 2021. “Creutzfeldt-Jakob Disease Surveillance in Australia: Update to 31 December 2020 ”. Communicable Diseases Intelligence 45 (July). https://doi.org/10.33321/cdi.2021.45.38.

Issue

Vol. 45 (2021)

Section

Annual report

Categories

License

Copyright (c) 2021 Communicable Diseases Intelligence

Creative Commons License

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

Most read articles by the same author(s)

1 2 3 > >>