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Creutzfeldt-Jakob Disease F005

Last amended 
15 February 2021
Current RMA Instruments
Reasonable Hypothesis SOP
76 of 2014
Balance of Probabilities SOP
77 of 2014
Changes from previous Instruments

SOP Bulletin 177

ICD Coding
  • ICD-9-CM Codes: 046.1
  • ICD-10-AM Codes: A81.0
Brief description

This SOP covers both Creutzfeldt-Jakob disease (CJD), a rapidly progressive degenerative brain disorder, and variant CJD associated with mad cow disease.  CJD is a very rare disease.  No case of variant CJD has ever been reported in Australia (at Feb ‘21).

Confirming the diagnosis

This diagnosis is complex and requires diagnosis by a specialist neurologist or infectious disease physician, or confirmation by autopsy.

Additional diagnoses covered by SOP
  • Classical Creutzfeldt Jakob disease
  • Variant Creutzfeldt Jakob disease
Conditions excluded from SOP
  • Kuru - non SOP
  • Other forms of dementia
Clinical onset

Mean age of onset is around 60.  The typical clinical presentation is with rapid neuropsychiatric decline.

Clinical worsening

There are no clinical worsening factors in the SOP.