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Huntington Disease P011

Last amended 
8 May 2023
Current RMA Instruments
Reasonable Hypothesis SOP
49 of 2023
Balance of Probabilities SOP
50 of 2023
Changes from previous Instruments

SOP Bulletin 236

ICD Coding

ICD-10-AM Code: G10

Brief description

This is an inherited, progressive, degenerative disorder of the nervous system, manifesting with a movement disorder (chorea), cognitive dysfunction (ending in dementia) and psychiatric problems.

Confirming the diagnosis

Diagnosis is based on the typical clinical features, family history and genetic testing.

The relevant medical specialist is a neurologist.

Additional diagnoses covered by SOP
  • Huntington's chorea
Conditions excluded from SOP
  • Brain and nervous pathology from other causes
  • Dementia from other causes including Alzheimer disease
  • Psychiatric disorders from other causes
Clinical onset

Clinical manifestations commonly begin in midlle life but can appear at any age from childhhod to the eighth decade.  Onset is typically insidious and so diagnosis may be delayed.  There is usually a slow relentless progression of symptoms and signs.

Clinical worsening

The only SOP factor is for inability to obtain appropriate clinical management.  No cure or disease-modifying treatment is currently available for Huntington disease.  Therapy is focused on symptom management and supportive care.