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Transmissible spongiform encephalopathies in Australia.

A Boyd¹, A Fletcher, J S Lee

¹Australian National Creutzfeldt-Jakob Disease Registry, Department of Pathology, The University of Melbourne, Parkville, Vic. aboyd@unimelb.edu.au

Communicable Diseases Intelligence Quarterly Report

|January 25, 2002

Summary

The Australian National Creutzfeldt-Jakob Disease Registry monitors human prion diseases, including Creutzfeldt-Jakob disease (CJD) and variant CJD. Surveillance since 1993 has identified 460 cases by September 2001.

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Area of Science:

Neurology
Epidemiology
Pathology

Background:

Established in 1993, the Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) monitors human prion diseases.
Surveillance expanded to include all human transmissible spongiform encephalopathies (TSEs) in Australia since 1970.

Purpose of the Study:

To ascertain and classify all human prion disease cases in Australia.
To monitor the incidence of classical Creutzfeldt-Jakob disease (CJD) and variant CJD (vCJD).

Main Methods:

Diverse ascertainment strategies including physician communication, death certificate searches, and hospital database reviews.
Diagnostic CSF 14-3-3 protein testing referrals significantly increased prospective case notifications.
Final classification relies on comprehensive clinical, investigational, and pathological data.

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Main Results:

As of September 2001, 460 cases were registered: 237 definite, 168 probable, and 55 incomplete.
Variant CJD (vCJD) has not been diagnosed in Australia.
The registry monitors sporadic, genetic, and healthcare-acquired CJD.

Conclusions:

The ANCJDR plays a crucial role in national surveillance of human prion diseases.
The registry employs multiple methods to ensure comprehensive case ascertainment and classification.
Ongoing surveillance is essential for understanding the epidemiology of CJD and related disorders in Australia.