Creutzfeldt-Jakob disease surveillance in Australia: update to 31 December 2025

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 improvements have been developed in pre-mortem diagnostics in parallel with the delineation of new disease subtypes and 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 2025. Since the ANCJDR began offering diagnostic cerebrospinal fluid (CSF) 14-3-3 protein testing in Australia in September 1997, the annual number of diagnostic test referrals has steadily increased. In 2025, there were 788 domestic CSF specimens referred for diagnostic testing; 92 persons with suspected human prion disease were formally added to the national register. As of 31 December 2025, more than half of the 84 initial case notifications for 2025 (44/84) remain classified as 'incomplete'; 35 cases have been confirmed as 'definite' or 'probable' prion disease; five cases were excluded. In 2025, most suspected human-prion-disease-related deaths in Australia (56%) underwent neuropathological examination. No cases of variant or iatrogenic CJD were identified in Australia during 2025.