Synopsis
Recognise chorea as a movement phenotype, identify acquired and treatable causes before attributing it to inheritance, and manage suspected Huntington disease through consented genomic testing, multidisciplinary symptom care and family-centred planning.
- Chorea consists of brief, irregular, non-rhythmic movements that flow unpredictably between body regions and become incorporated into apparently purposeful activity; it differs from rhythmic tremor and stereotyped tics.
- Look for motor impersistence such as an unsustained tongue protrusion or fluctuating milkmaid grip, unpredictable facial movements, fidgeting gait and intrusive distal movements during posture.
- Huntington disease is an autosomal dominant neurodegenerative disorder caused by a CAG repeat expansion in HTT; each child of an affected person has a one-in-two chance of inheriting the expansion.
Key red flags
Abrupt unilateral chorea suggests a contralateral basal ganglia or subthalamic lesion, severe non-ketotic hyperglycaemia or another focal process and needs urgent imaging and metabolic assessment.
Investigation priorities
Confirm chorea, map progression and identify cognitive, psychiatric, medicine and family clues.
Management branches
Irregular involuntary movements are newly observed or rapidly worsening.
- 1. Confirm choreic phenomenology, establish sudden versus progressive onset, examine focal neurology and assess cognition, behaviour, temperature and swallowing safety.
- 2. Reconcile prescription, antiemetic, psychiatric, dopaminergic and recreational exposures with the movement timeline, including recently stopped agents.
Key medicines
TetrabenazineA movement-disorder specialist starts a low daily dose and titrates in small divided increments to the lowest effective regimen, following the current BNF and product maximum rather than a fixed target.
Specialist-selected antipsychoticUse the lowest effective oral dose with slow titration when psychosis, severe irritability or chorea provides a documented indication; medicine and schedule depend on metabolic and motor risk.