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Huntington Disease P011
Current RMA Instruments
|Reasonable Hypothesis SOP||49 of 2023|
|Balance of Probabilities SOP||50 of 2023|
Changes from previous Instruments
ICD-10-AM Code: G10
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 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.
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.