Creutzfeldt-Jakob disease (CJD) is one of the human forms of the transmissible spongiform encephalopathies (TSE), also referred to as prion diseases. Prion diseases mostly occur as a chance event (sporadic CJD), but can be in rare cases attributed to a genetic mutation (genetic CJD) or be acquired through a transmission event (iatrogenic forms or variant CJD).
The Australian National Creutzfeldt-Jakob Disease Registry (ANCJDR) is under contract to the Commonwealth Department of Health and is responsible for the national surveillance of clinically suspected and diagnosed human prion diseases in Australia. ANCJDR has formal agreements with State Health Departments in Victoria, New South Wales and Queensland to assist in the evaluation of notified cases of “possible CJD”. See the ANCJDR website for further details https://www.florey.edu.au/australian-national-cjd-registry
The ANCJDR is responsible for diagnostic testing of CSF specimens (eg 14-3-3), PrPres testing of tonsil biopsies and molecular sub-typing of sporadic CJD using brain tissue (PRNP codon 129 and prion protein glycotype)
Neuropathological investigation of whole brain tissue after death
Only the investigation of the whole brain after death can provide a definite diagnosis of whether or not the individual had prion disease; it provides the family and clinicians with the highest level of confidence in the diagnosis of the patient. When a differential diagnosis includes CJD full body autopsies cannot be offered due to infection control reasons.
A brain only autopsy, where the whole brain is examined, is voluntary and depends entirely on informed consent from the Senior Available Next of Kin (SANOK). The treating doctor is expected to educate the patient’s family about benefits and disadvantages of the autopsy, as if undertaking any other routine medical procedure. Consent for an elective autopsy is documented by the requesting doctor and SANOK signing an autopsy consent form. An autopsy request form from any hospital is acceptable documentation of consent. All Victorian CJD autopsies are performed by the Alfred Health mortuary. A copy of the Alfred Health autopsy form can be downloaded from the Mortuary page of the Pathology Handbook if required.
As CJD brain only autopsies assist public health surveillance of CJD in Australia and contribute to the knowledge base and research of CJD, there is no additional cost to the family or referring institution for any aspect of the autopsy including transfer of the deceased to the Alfred mortuary.
Contact information
ANCJDR Office hours : Weekdays 9am – 5 pm
ANCJDR is Closed On Victorian Public And University Holidays
Family members can contact the coordinator of the ANCJDR (03 8344 1949) or the CJD Support Group network (1800 052 466) for information, assistance and support.
The CJD Support Group Network provides a 24 hour hotline (1800 052 466) and is able to contact the ANCJDR coordinator in urgent matters.
Arranging Body Transfer
Transfer of a deceased can be arranged through a funeral transfer company at no cost to the family. Contact: Melbourne and Peninsula Funeral Transfers (0475 448 996). Transfer of the body to the Alfred mortuary can also be performed by the Funeral Director appointed by the next of kin.
Coordination of a transfer should be made by contacting the Alfred mortuary.
Alfred Mortuary hours: Weekdays 7am – 4 pm (03 9076 2684).
The Alfred Mortuary is closed weekends and public holidays.
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