The Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) commenced surveillance in September 1993 as part of the Commonwealth's response to 4 cases of pituitary hormone (gonadotrophin)-associated Creutzfeldt-Jakob disease (CJD). With the passage of time, the Registry has become responsible for ascertaining all human transmissible spongiform encephalopathies (TSE; also known as prion diseases) within Australia since 1970. Included in the spectrum of diseases monitored are classical (sporadic, genetic, and health care acquired) CJD, and variant CJD (vCJD), first reported in 1996 in the United Kingdom. Variant CJD has not yet been diagnosed in Australia. Final classification of persons with suspected human prion disease is based upon all available clinical, investigational and pathological information. Ascertainment methods are diverse and include prompted, half-yearly personal communications from neurologists and neuropathologists, death certificate searches, and morbidity separation coding searches of major hospital, and State and Territory databases. More recently, referral for diagnostic CSF 14-3-3 protein testing (performed by the ANCJDR) has considerably increased prospective notifications of suspect cases. As at September 2001 there were 460 cases on the register; 237 definite cases, 168 probable and 55 incomplete cases awaiting final classification.