Creutzfeldt-Jakob disease surveillance in Australia January 1970 to December 2003.

The Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) was established by the Commonwealth Government in October 1993 in response to the recognition of four probable human pituitary hormone related Creutzfeldt-Jakob disease (CJD) deaths. An inquiry into CJD in Australia and the use of human pituitary hormones under the Australian Human Pituitary Hormone Program suggested the expansion of some activities of the Registry to include retrospective case ascertainment from 1 January 1970. In parallel with monitoring possible medically acquired (iatrogenic) cases of CJD, the ANCJDR prospectively monitors and investigates all suspect cases of transmissible spongiform encephalopathies occurring within the states and territories of Australia, including sporadic and familial, and the potential occurrence of variant CJD. The ANCJDR also actively participates in an international surveillance consortium. This brief report summarises methods of classification and ascertainment as well as current epidemiological findings and new surveillance techniques that are being adopted to improve case ascertainment.