From the FDA Drug Label
The efficacy of tetrabenazine tablets as a treatment for the chorea of Huntington’s disease was established primarily in a randomized, double-blind, placebo-controlled multi-center trial (Study 1) conducted in ambulatory patients with a diagnosis of HD. Tetrabenazine tablets were started at a dose of 12.5 mg per day, followed by upward titration at weekly intervals, in 12.5 mg increments until satisfactory control of chorea was achieved, intolerable side effects occurred, or until a maximal dose of 100 mg per day was reached.
The management of early Huntington's disease (HD) with tetrabenazine tablets involves:
- Starting with a dose of 12.5 mg per day
- Upward titration at weekly intervals in 12.5 mg increments
- Targeting a dose that achieves satisfactory control of chorea, but not exceeding 100 mg per day
- Monitoring for side effects and adjusting the dose as needed 1
From the Research
The management of early Huntington's disease focuses on symptom control and supportive care, with a multidisciplinary approach involving neurologists, psychiatrists, genetic counselors, physical therapists, and social workers providing the most comprehensive care. For chorea, which is often the most visible symptom, tetrabenazine (12.5-25 mg three times daily) or deutetrabenazine (6-12 mg twice daily) are first-line medications, as supported by the most recent study 2. Antipsychotics like risperidone (0.5-2 mg daily) or olanzapine (2.5-10 mg daily) may help control both chorea and psychiatric symptoms. Depression can be treated with SSRIs such as sertraline (50-200 mg daily) or escitalopram (10-20 mg daily). Irritability and aggression might respond to mood stabilizers like valproate (250-500 mg twice daily).
Some key aspects of care include:
- Physical therapy, occupational therapy, and speech therapy to maintain function as long as possible
- Genetic counseling to understand inheritance patterns and reproductive options
- Regular reassessment and adjustment of care plans to address changing symptoms and functional abilities
- Early advance care planning discussions to establish the patient's wishes for future care
Alternative approaches, such as music therapy, video games, yoga, and exercise-based programs, may also have a role in improving symptoms, as discussed in a recent narrative review 2. However, the primary approach should remain focused on evidence-based symptom control and supportive care, with a multidisciplinary team guiding the management of the disease.