Objective: To develop an evidence-based guideline assessing pharmacologic options for treating Huntington disease (HD) chorea.
Methods: We evaluated available evidence from a structured literature review performed through
Results and recommendations: If HD chorea requires treatment, clinicians should prescribe tetrabenazine (up to 100 mg/day), amantadine (300–400 mg/day), or riluzole (200 mg/day) (Level B)
for varying degrees of expected benefit. Occurrence of adverse events should be discussed and
monitored, particularly depression/suicidality and parkinsonism with tetrabenazine and elevated
liver enzymes with riluzole. Clinicians may also prescribe nabilone for modest decreases (1- to
2-point changes on the Unified Huntington’s Disease Rating Scale [UHDRS] chorea score) in HD
chorea (Level C), but information is insufficient to recommend long-term use, particularly given
abuse potential concerns (Level U). Clinicians should not prescribe riluzole 100 mg/day for moderate (2- to 3-point UHDRS chorea change) short-term benefits (Level B) or for any long-term
(3-year) HD antichoreic goals (Level B). Clinicians may choose not to prescribe ethyl-EPA (Level B),
minocycline (Level B), or creatine (Level C) for very important improvements (3-point UHDRS
chorea change) in HD chorea. Clinicians may choose not to prescribe coenzyme Q10 (Level B) for
moderate improvements in HD chorea. Data are insufficient to make recommendations regarding
the use of neuroleptics or donepezil for HD chorea treatment (Level U).
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