Australian National Creutzfeldt-Jakob Disease Registry
Prof
Steven
Collins
The Florey Institute
30 Royal Pde
Parkville
3052
Australia
Prion disease
The detection and surveillance of Cruetzfeldt-Jakob diseaseand associated prion diseases in Australia.
The Registry is unusal as it is tasked with the public health surveillance of prion diseases for local and the Commonwealth Health Departments. The Registry provides specialist advice and diagnostic services, similar to other prion disease units oversease. As a specialist reference centre, the ANCJDR manages the clinical case evaluation and disease classification of individuals prior to cases inclusion in incidence and epidemiology reports. Classification uses WHO surveillance clinical criteria.. The Registry is also unusual as it involves clinically suspect notifications under noifiable diseases, while patients are usually alive and the illness isnt always clearly characerised due to the complexity of diagnosis and the lack of a single specific ante-mortem diagnostic test. Cases are routinely evaluated after death by the ANCJDR, relying either on pathological autopsy findings or ANCJDR evaluation of clinical details and test findings. Medico-demographic information is accessed from medical records and surviving family mmebrs assisting teh ANCJDR.
>15
Collins S. Creutzfeldt-Jakob disease surveillance in Australia: update to December 2013. Commun Dis Intell Q Rep. 2014 Dec 31;38(4):E348-355.| Collins S. Enhanced geographically restricted surveillance simulates /Masters CL. Creutzfeldt-Jakob disease cluster in an Australian rural city. Annals of Neurology. 52(1):115-118, 2002 Jul. sporadic Creutzfeldt-Jakob disease cluster. Brain. 2009 Feb; 132(Pt 2):493-501. |
CDI publications - http://www.health.gov.au/internet/main/publishing.nsf/content/cda-cjdanrep.htm and an extensive list of collaborative peer reviewed publications.
n/a
1
until death
The clinical suspicion of prion disease
Insufficient cliinical evidence to support a CJD diagnosis, the diagnosis of an alternative illness and exclude CJD, or alternatively pathology proven not CJD after brain autopsy.
1001-5000 clinical cases
Clinical review of the illness raising concern for prion diseases, accepted as statistical case hen case criteria meets WHO surveillance criteria for CJD and associated prion diseases.
No
Yes
01/01/1970
Nil
Data collection ongoing|Data analysis ongoing
Funded by the Commonwealth Health Dept.
Yes
Current contract to mid 2017, funding may continue.
0
n/a
0
0
Only collected through the study
No
Data summarised in database
No
No
No
No
English
Data is held as individual records
No
Data held on computer based records
Data held on cards
English
No
Apply to PI or co-ordinator at resource
Data made publicly available after a specified time point
Yes
Post-mortem donors: brain
Primary Samples: Stabilised samples (frozen or fixed)
No
If available for a subset please specify number of patients and % of total cohort
Yes