Creutzfeldt-Jakob disease: Australian surveillance update to March 2007.

From October 1993, prospective, national surveillance of the rare class of neurodegenerative diseases known as transmissible spongiform encephalopathies (TSEs) has been performed by the Australian National Creutzfeldt-Jakob Disease Registry. Surveillance of TSEs prior to October 1993, involved the retrospective ascertainment of TSE cases from 1970 to 1993. In this report, surveillance data for 2006 are presented in detail and compared to cumulative national TSE ascertainment as well as international experience. The higher incidence of TSEs in 2006 is not without precedent and can be attributed to higher referrals and consequent post-mortem rates.