The Australian National Creutzfelt-Jakob Disease Registry

Discussion at support group meetings and questions asked of coordinators show that many members of the recipient community are keen to know that they are up to date with developments in the area of CJD. Alison Boyd of the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) has agreed to provide a series of short articles for our support group newsletters. Below is the first of Alison’s contributions, an introductory article that we hope will be followed in the future by updates that will continue to provide information that is current, accurate, and we hope interesting.
David Ralston
CJDSGN

The Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) was established in October 1993 in response to the four CJD deaths in recipients of gonadotrophin (hPG). The ANCJDR is headed by co-directors Professors Colin Masters and Steven Collins. The ANCJDR was established to perform surveillance of CJD and prion diseases, with an emphasis on monitoring for medically acquired CJD cases in Australia. The ANCJDR is designated a referral centre for human Transmissible Spongiform Encephalopathies (TSE) by the World Health Organization, offering laboratory based TSE diagnostic services.

Current Australian CJD figures for the period 1993-2004 report an overall incidence rate of 1.15 deaths per million people, per head of population, per year. This rate is consistent with anticipated numbers in a population of the size found in Australia. For this introduction, I will provide numbers on the medically acquired Australian CJD cases. In total 10 cases have occurred between 1987 and 2000; four of these were recipients of hPG (3 cases were confirmed with CJD and one case is classed as clinically probable CJD) and a single possible case is recorded in a recipient of growth hormone (hGH). There was insufficient supporting evidence to elevate this case to a firm diagnosis of probable CJD, consequently it is classed as suspicious for CJD only. A further five people have also died confirmed with CJD, with histories of dura mater grafts during neurosurgery in Australia. The risk for medically acquired CJD generates much interest, but the ANCJDR can report there have been no medically acquired CJD cases arising from corneal transplants or through contaminated neurosurgical instruments in Australia, and no cases of medically acquired CJD have occurred after 2000.