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Australian national notifiable diseases case definitions

Variant Creutzfeldt-Jakob disease surveillance case definition - V1.0

This document contains the case definitions for variant creutzfeldt Jakob disease, which is nationally notifiable within Australia. This definition should be used to determine whether a case should be notified.

Printable PDF version of variant Creutztfeldt-Jakob disease surveillance case definition (PDF 14 KB)

Version	Status	Last reviewed	Endorsement date	Implementation date
1.0	Initial CDNA case definition	CDWG November 2009	CDNA 16 December 2009	1 July 2010

Reporting

Confirmed and probable cases should be notified. (NB: a “confirmed” case is equivalent to the ANCJDR classification of “definite”)

Confirmed case

A confirmed case requires laboratory definitive evidence AND clinical evidence

Laboratory definitive evidence

Neuropathological confirmation of vCJD

Clinical evidence

Progressive neuropsychiatric disorder

Probable case

A probable case requires clinical definitive evidence

OR
Clinical suggestive evidence AND laboratory suggestive evidence.

Clinical definitive evidence

1. Progressive neuropsychiatric disorder AND duration of illness greater than six months AND routine investigations do not suggest an alternative diagnosis AND no history of potential iatrogenic exposure AND no evidence of a familial form of TSE

AND
2. Four of the following symptoms:

• Early psychiatric symptoms
• Persistent painful sensory symptoms
• Ataxia
• Myoclonus or chorea or dystonia
• Dementia

AND
3. Bilateral pulvinar high signals on magnetic resonance imaging (MRI) scans
AND
4. Electroencephalogram (EEG) which does not exhibit the typical appearance of classic CJD

Clinical suggestive evidence

1. Progressive neuropsychiatric disorder AND duration of illness greater than six months AND routine investigations do not suggest an alternative diagnosis AND no history of potential iatrogenic exposure AND no evidence of a familial form of TSE

Laboratory suggestive evidence

1. A PrP^SC positive tonsil biopsy