摘要：The Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) commenced surveillance in September1993 as part of the Commonwealth's response to 4 cases of pituitary hormone (gonadotrophin)-associatedCreutzfeldt-Jakob disease (CJD). With the passage of time, the Registry has become responsible for ascertaining allhuman transmissible spongiform encephalopathies (TSE; also known as prion diseases) within Australia since1970. 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 beendiagnosed in Australia. Final classification of persons with suspected human prion disease is based upon allavailable clinical, investigational and pathological information. Ascertainment methods are diverse and includeprompted, half-yearly personal communications from neurologists and neuropathologists, death certificatesearches, and morbidity separation coding searches of major hospital, and State and Territory databases. Morerecently, referral for diagnostic CSF 14-3-3 protein testing (performed by the ANCJDR) has considerablyincreased 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
