Creutzfeldt-Jakob disease surveillance in Australia: update to 31 December 2021

Authors

  • Christiane Stehmann Florey Institute, The University of Melbourne, Victoria, 3010, Australia
  • Matteo Senesi Department of Medicine, The University of Melbourne, Victoria, 3010, Australia
  • Shannon Sarros Florey Institute, The University of Melbourne, Victoria, 3010, Australia
  • Amelia McGlade Florey Institute, The University of Melbourne, Victoria, 3010, Australia
  • Victoria Lewis Department of Medicine, The University of Melbourne, Victoria, 3010, Australia
  • Marion Simpson Florey Institute, The University of Melbourne, Victoria, 3010, Australia
  • Genevieve Klug Florey Institute, The University of Melbourne, Victoria, 3010, Australia
  • Catriona McLean Florey Institute, The University of Melbourne, Victoria, 3010, Australia; Alfred Hospital, Department of Anatomical Pathology, 55 Commercial Rd, Melbourne Vic 3004 Australia
  • Colin L Masters 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.2022.46.53

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 2021.
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 2021, a total of 548 domestic CSF specimens were referred for 14-3-3 protein testing; 73 persons with suspected human prion disease were formally added to the national register. As of 31 December 2021, just over half of the 73 suspect case notifications (37/73) remain classified as ‘incomplete’; 17 cases were classified as ‘definite’ and 13 as ‘probable’ prion disease; six cases were excluded through either detailed clinical follow-up (two cases) or neuropathological examination (four cases). For 2021, sixty-four percent of all suspected human-prion-disease-related deaths in Australia underwent neuropathological examination. No cases of variant or iatrogenic CJD were identified.

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Published

18/08/22

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. 2022. “Creutzfeldt-Jakob Disease Surveillance in Australia: Update to 31 December 2021”. Communicable Diseases Intelligence 46 (August). https://doi.org/10.33321/cdi.2022.46.53.

Issue

Vol. 46 (2022)

Section

Annual report

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