Creutzfeldt-Jakob disease: Australian surveillance update to December 2005.

Australia-wide prospective surveillance of human transmissible spongiform encephalopathies (TSEs) has been conducted by the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) since October 1993. In addition, the Registry retrospectively ascertained TSE cases within Australia from 1970. Referrals of all suspect cases of human prion diseases or TSEs are investigated by the ANCJDR and include Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia and variant CJD. This semi-annual progressive update presents epidemiological findings of the ANCJDR based on Australian data obtained for the period 1970 to 31 December 2005.