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Creutzfeldt-Jakob disease in Australia 2000

(Based on the report from the Australian CJD Registry, The University of Melbourne - update to January 2000)

The Australian Creutzfeldt-Jakob disease (CJD) Registry was established in October 1993 in response to the recognition of four probable human pituitary hormone related CJD deaths. The Registry is funded by the Commonwealth Department of Health and Ageing and is based in the Department of Pathology at the University of Melbourne. An inquiry into the use of human pituitary hormones under the Australian Human Pituitary Hormone Program suggested the expansion of some activities of the Registry.¹¹² These have been adopted, including retrospective case ascertainment from 1 January 1970. However, in parallel with monitoring possible iatrogenic cases of CJD, the Registry monitors all cases of transmissible spongiform encephalopathies, both sporadic and familial, within Australia. There is no systematic neuropathological or prion protein genetic assessment, but in line with non-domestic programs, evaluation of this type is now attempted in all prospective cases.
As of July 2001, Australia remains free of animal forms of prion disease, such as bovine spongiform encephalopathy and scrapie, and has no confirmed cases of variant CJD. As of the middle of 2001, there were 454 cases on the Registry, which included 237 definite cases, 168 probable cases and 49 incomplete cases (cases positive in an immunoassay but not finally classified).

In Australia, there has been a doubling of the average incidence of spongiform encephalopathies to one case per million since the mid-1980s. This mainly reflects case ascertainment bias due to improved recognition, confirmation and reporting. The composition of cases on the Registry is 90.4 per cent sporadic, 7.4 per cent familial and 2.2 per cent iatrogenic.


This article was published in Communicable Diseases Intelligence Volume 26, No 2, June 2002