Australia-wide surveillance of all human transmissible spongiform encephalopathies (TSEs) is performed by the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR), since establishment in October 1993. During the surveillance period 1 April 2007 to 31 March 2008, the ANCJDR received 78 new suspect case notifications of TSEs (67 in 2007, 13 in 2008). This level of suspect case notification aligns with the previous 2006/2007 surveillance period, which was elevated in comparison to the previous 5 years. Based on the total number of probable and definite Creutzfeldt-Jakob disease (CJD) cases, encompassing retrospective cases to 1970 and prospectively ascertained cases from 1993 to 31 March 2008, the average age-adjusted mortality rate is 1.16 deaths per million per year. In this short report, we provide updated Australian TSE figures and describe recent changes in surveillance mechanisms and review their impact on case notifications and eventual CJD classification.