Huntington Disease – Chorea Treatment — SCE Neurology MCQ

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ModerateMovement DisordersHuntington Disease – Chorea TreatmentSCE Neurology

A 45-year-old man with Huntington disease (confirmed 44 CAG repeats) has disabling chorea that significantly impairs his function. He has mild cognitive impairment but no depression. What is the recommended first-line pharmacological treatment for chorea in HD?

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Correct answer: B — Tetrabenazine

Tetrabenazine (a vesicular monoamine transporter 2 inhibitor that depletes presynaptic dopamine) is the only medication specifically licensed and recommended for chorea in Huntington disease. It reduces chorea by 25–50% in clinical trials. Key side effects include depression, parkinsonism, akathisia, and somnolence. Depression and suicidality must be monitored closely (as HD patients already have high suicide risk). A: Levodopa would worsen chorea. C: Haloperidol has been used historically but has more side effects and is not first-line. D: Amantadine has some evidence but is not first-line. E: Botulinum toxin is for focal dystonia, not generalised chorea.

Reference: EAN Huntington Disease Guidelines; NICE; BNF – Tetrabenazine