Date amended:
Statements of Principles
Current RMA Instruments
Reasonable Hypothesis SOP
80 of 2022
Balance of Probabilities SOP
81 of 2022
Changes from previous Instruments


ICD Coding
  • ICD-10-AM Code: 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 (as at Sep ‘22).

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.