Creutzfeldt-Jakob disease surveillance in Australia: update to 31 December 2022

Authors

  • Christiane Stehmann The Florey, The University of Melbourne, Victoria, 3010, Australia
  • Matteo Senesi Department of Medicine, The University of Melbourne, Victoria, 3010, Australia
  • Shannon Sarros Department of Medicine, The University of Melbourne, Victoria, 3010, Australia
  • Amelia McGlade The Florey, The University of Melbourne, Victoria, 3010, Australia
  • Victoria Lewis Department of Medicine, The University of Melbourne, Victoria, 3010, Australia
  • Laura Ellett The Florey, The University of Melbourne, Victoria, 3010, Australia
  • Daniel Barber The Florey, The University of Melbourne, Victoria, 3010, Australia
  • Marion Simpson The Florey, The University of Melbourne, Victoria, 3010, Australia
  • Genevieve Klug The Florey, The University of Melbourne, Victoria, 3010, Australia
  • Catriona A McLean The Florey, 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, The University of Melbourne, Victoria, 3010, Australia
  • Steven J Collins Department of Medicine, The University of Melbourne, Victoria, 3010, Australia

DOI:

https://doi.org/10.33321/cdi.2023.47.37

Keywords:

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

Abstract

Nationwide surveillance of Creutzfeldt-Jakob disease (CJD) 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 2022.

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 2022, a total of 599 domestic CSF specimens were referred for diagnostic testing and 79 persons with suspected human prion disease were formally added to the national register. As of 31 December 2022, just under half of the 79 suspect case notifications (36/79) remain classified as ‘incomplete’; 15 cases were classified as ‘definite’ and 23 as ‘probable’ prion disease; five cases were excluded through neuropathological examination. For 2022, fifty-five 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 during 2022.

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Published

26/06/23

How to Cite

Stehmann , Christiane, Matteo Senesi, Shannon Sarros, Amelia McGlade, Victoria Lewis, Laura Ellett, Daniel Barber, et al. 2023. “Creutzfeldt-Jakob Disease Surveillance in Australia: Update to 31 December 2022”. Communicable Diseases Intelligence 47 (June). https://doi.org/10.33321/cdi.2023.47.37.

Issue

Vol. 47 (2023)

Section

Annual report

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