Creutzfeldt-Jakob disease (CJD) case definition - V1.0

This document contains the case definitions for Creutzfeldt-Jakob disease which is nationally notifiable within Australia. This definition should be used to determine whether a case should be notified.

Page last updated: 11 August 2010

Printable PDF version of Creutztfeldt-Jakob disease surveillance case definition (PDF 18 KB)

Last reviewed
Endorsement date
Implementation date
1.0 Initial CDNA case definition CDWG November 2009 CDNA 16 December 2009 1 July 2010


Confirmed and probable cases should be notified. This includes sporadic, accidental and familial cases. (NB: a “confirmed” case is equivalent to the ANCJDR classification of “definite”)

Confirmed case

A confirmed case requires laboratory definitive evidence

Laboratory definitive evidence

Neuropathological confirmation of CJD supplemented by immunochemical detection of protease-resistant PrP by western blot OR immunocytochemistry.

Probable case

A probable case requires clinical evidence AND either electroencephalogram (EEG) or laboratory suggestive evidence.

Laboratory suggestive evidence

Positive 14-3-3 protein CSF test.

Clinical evidence

1. Progressive dementia of less than two years duration; AND
2. At least 2 of the following clinical features: