Australia Creutzfeldt-Jakob disease surveillance update to 31 December 2023

 2025 • Volume 49

Communicable Diseases Intelligence

Creutzfeldt-Jakob disease surveillance in Australia: update to 31 December 2023

Christiane Stehmann, Matteo Senesi, Shannon Sarros, Amelia McGlade, Victoria Lewis, Priscilla Agustina, Laura Ellett, Daniel Barber, Genevieve Klug, Catriona McLean, Colin L Masters, Steven Collins

Website: www.health.gov.au/cdi Email: cdi.editor@health.gov.au www.health.gov.au/cdi • Commun Dis Intell (2018) 2025;49 (https://doi.org/10.33321/cdi.2025.49.012) • Epub 25/03/2025 2

Annual report

Creutzfeldt-Jakob disease surveillance in Australia: update to 31 December 2023

Christiane Stehmann, Matteo Senesi, Shannon Sarros, Amelia McGlade, Victoria Lewis, Priscilla Agustina, Laura Ellett, Daniel Barber, Genevieve Klug, Catriona McLean, Colin L Masters, Steven Collins

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 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 2023. 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 2023, a total of 651 domestic CSF specimens were referred for diagnostic testing and 83 persons with suspected human prion disease were formally added to the national register. As of 31 December 2023, just under half of the 83 suspect case notifications (41) remain classified as ‘incomplete’; 10 cases were classified as ‘definite’ and 28 as ‘probable’ prion disease; three cases were excluded through neuropathological examination and one was removed from the register as ‘unlikely CJD’ after clinical evaluation. For 2023, fifty-three percent of all suspected human-prion-disease-related deaths in Australia underwent neuropathological examination. No cases of variant or iatrogenic CJD were identified.

Keywords: Creutzfeldt-Jakob disease; prion disease; transmissible spongiform encephalopathy; disease surveillance

https://www1.health.gov.au/internet/main/publishing.nsf/Content/A005BA6FD1F9ACD4CA258BFC0001FE0A/$File/cdi-2025-49-012.pdf

“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.”

TUESDAY, JANUARY 1, 2019

***> CHILDHOOD EXPOSURE TO CADAVERIC DURA Singeltary et al

https://betaamyloidcjd.blogspot.com/2019/01/childhood-exposure-to-cadaveric-dura.html

THURSDAY, JULY 29, 2021 

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

https://creutzfeldt-jakob-disease.blogspot.com/2021/07/creutzfeldt-jakob-disease-surveillance.html

SATURDAY, JANUARY 12, 2019

Australia Creutzfeldt-Jakob disease surveillance: update to December 2016 Published December 17, 2018

https://creutzfeldt-jakob-disease.blogspot.com/2019/01/australia-creutzfeldt-jakob-disease.html

SATURDAY, FEBRUARY 07, 2015

Annual report Creutzfeldt-Jakob disease surveillance in Australia, 2013

https://creutzfeldt-jakob-disease.blogspot.com/2015/02/annual-report-creutzfeldt-jakob-disease.html

MONDAY, FEBRUARY 24, 2014

UPDATE ON CREUTZFELDT–JAKOB DISEASE Masters et al Australia

https://creutzfeldt-jakob-disease.blogspot.com/2014/02/update-on-creutzfeldtjakob-disease.html

Saturday, November 09, 2013

Surveillance for creutzfeldt-Jakob disease in Australia: update to December 2012

http://creutzfeldt-jakob-disease.blogspot.com/2013/11/surveillance-for-creutzfeldt-jakob.html

SATURDAY, APRIL 14, 2012

Australian Disease Strategy Bovine spongiform encephalopathy Version 3.2 and Creutzfeldt Jakob Disease report 2012

http://transmissiblespongiformencephalopathy.blogspot.com/2012/04/australian-disease-strategy-bovine.html

Thursday, August 05, 2010

Surveillance of Creutzfeldt-Jakob disease in Australia: 2010 update

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/surveillance-of-creutzfeldt-jakob.html

THURSDAY, MAY 15, 2025 

Cadaveric Human Growth Hormone–Associated Creutzfeldt-Jakob Disease with Long Latency Period, United States 

https://creutzfeldt-jakob-disease.blogspot.com/2025/05/cadaveric-human-growth.html

SUNDAY, MARCH 23, 2025

Creutzfeldt Jakob Disease TSE Prion Increasing 2025 Update

https://creutzfeldt-jakob-disease.blogspot.com/2025/03/creutzfeldt-jakob-disease-tse-prion.html

FRIDAY, DECEMBER 13, 2024

Creutzfeldt Jacob Disease CJD, BSE, CWD, TSE Prion, December 14, 2024 Annual Update

https://creutzfeldt-jakob-disease.blogspot.com/2024/12/creutzfeldt-jacob-disease-cjd-bse-cwd.html

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