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Other communicable disease surveillance

Creutzfeldt-Jakob disease

This section is based on reports for the period from the Australian National Creutzfeldt-Jakob disease (CJD) Registry, the University of Melbourne.

The Australian National CJD Registry was established in 1993, in response to recognition of four probable cases of iatrogenic CJD resulting from the use of human pituitary hormones. It has subsequently undertaken retrospective case ascertainment in addition to its ongoing monitoring and surveillance activities and the Register now includes cases of CJD dating back to 1 January 1970 (Table 25).

Table 25. Cases reported to the Australian National Creutzfeldt-Jakob Disease Registry: 1970 to 2001

Classification	Number of cases as at December 2001	Change in number of cases during 2001*
Definite	243	+ 7
Probable	170	+10
Possible	0	- 1
Incomplete†	66	- 23
Total	479	- 7

* These changes are due to reclassification of previously notified cases, as more definitive data confirm or exclude provisional diagnoses

The average annual incidence of CJD in Australia between 1988 and 2000 is 1.13 cases per million population. International rates are generally around one case per million population per annum. Of the cases recorded on the Register, 90.3 per cent of cases are sporadic, 7.5 per cent of cases are familial and 2.2 per cent of cases are iatrogenic in origin. No new cases of iatrogenic disease were recorded in Australia in 2001.

The average age of death for sporadic CJD cases by sex was 64 years for males and 67 years for females. For familial CJD the average age for death was 52 years for males and 59 years for females, and for people who acquired the disease iatrogenically, the average age of death was 45 years for both males and females.

As at the end of December 2001, Australia remains free of animal forms of transmissible spongiform encephalopathies and no cases of the variant form of CJD have been detected.

In Australia, autopsy rates have been steadily declining, in line with international trends. Lack of autopsy frequently compromises the ability of the Registry to classify cases as definite, pending the development of additional tests. The Registry has seen an increase in notification of suspected cases since the introduction of diagnostic testing of the 14-3-3 protein in cerebrospinal fluid.


This article was published in Communicable Diseases Intelligence Volume 27, No 1, March 2003.