Short-term effects of tetrabenazine on chorea associated with Huntington disease

Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA.
Movement Disorders (Impact Factor: 5.68). 01/2007; 22(1):10-3. DOI: 10.1002/mds.21161
Source: PubMed


We sought to assess the short-term clinical effects of tetrabenazine (TBZ) on choreic movements in Huntington's disease patients. A total of 10 patients on stable doses of TBZ were enrolled in this observational study. Patients took their evening dose of TBZ and presented the next day to the Baylor College of Medicine Movement Disorders Clinic without taking the usual morning dose. They were assessed using the Unified Huntington's Disease Rating Scale (UHDRS) motor assessment and Beck Depression Inventory. The usual morning dose of TBZ was then administered and patients were followed with serial UHDRS motor examinations approximately every 2 hours until choreic movements subsided and then returned. TBZ decreased the UHDRS chorea score on average 42.4% +/- 17.8%. The duration of effect varied from a minimum of 3.2 hours to a maximum of 8.1 hours (mean = 5.4 +/- 1.3). No patient experienced an adverse event related to TBZ or its withdrawal. During short-term follow-up after a single dose, TBZ improves chorea for approximately 5 hours.

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Available from: Joseph Jankovic, Oct 13, 2014
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    • "Safety was ensured during the off-TBZ time period by asking a family member to drive them to the testing site and by close monitoring by the investigators or family members. Previous studies have shown no significant adverse effects from the sudden withdrawal of TBZ [16] [17]. All statistical analyses were performed using SPSS Version 21. "
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    ABSTRACT: Chorea may contribute to balance problems and walking difficulties that lead to higher fall rates in individuals with Huntington’s disease (HD). Few studies have examined the effects of tetrabenazine (TBZ), an anti-choreic drug, on function and mobility in HD. The purpose of this study was to compare: 1) gait measures in forward walking, 2) balance and mobility measures, and 3) hand and forearm function measures on and off TBZ. We hypothesized that use of TBZ would improve gait, transfers and hand and forearm function. Eleven individuals with HD on stable doses of TBZ were evaluated while off medication and again following resumption of medication. Significant improvements were found on the Unified Huntington’s Disease Rating Scale (UHDRS) motor scores, Tinetti Mobility Test (TMT) total (t = 4.20, p = 0.002) and balance subscale (t = − 4.61, p = 0.001) scores, and the Five Times Sit-to-Stand test (5TSST, t = 3.20, p = .009) when on-TBZ compared to off-TBZ. Spatiotemporal gait measures, the Six Condition Romberg test, and UHDRS hand and forearm function items were not changed by TBZ use. Improved TMT and 5TSST performance when on drug indicates that TBZ use may improve balance and functional mobility in individuals with HD.
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    • "By reversibly inhibiting the central vesicular monoamine transporter type 2 (VMAT2), TBZ more selectively depletes dopamine than norepinephrine.69,70 The highest binding density for TBZ is in the caudate nucleus, putamen, and nucleus accumbens, areas known to bear the brunt of pathology in HD.71,72 VMAT2 binding and monoamine depletion by TBZ are reversible, last hours, and are not modified by long-term treatment.73,74 These features of the drug differentiate it from the other dopamine-depleting agent, reserpine. "
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    ABSTRACT: Huntington disease (HD) is a dominantly inherited progressive neurological disease characterized by chorea, an involuntary brief movement that tends to flow between body regions. HD is typically diagnosed based on clinical findings in the setting of a family history and may be confirmed with genetic testing. Predictive testing is available to those at risk, but only experienced clinicians should perform the counseling and testing. Multiple areas of the brain degenerate mainly involving the neurotransmitters dopamine, glutamate, and γ-aminobutyric acid. Although pharmacotherapies theoretically target these neurotransmitters, few well-conducted trials for symptomatic or neuroprotective interventions yielded positive results. Tetrabenazine (TBZ) is a dopamine-depleting agent that may be one of the more effective agents for reducing chorea, although it has a risk of potentially serious adverse effects. Some newer antipsychotic agents, such as olanzapine and aripiprazole, may have adequate efficacy with a more favorable adverse-effect profile than older antipsychotic agents for treating chorea and psychosis. This review will address the epidemiology and diagnosis of HD as background for understanding potential pharmacological treatment options. Because TBZ is the only US Food and Drug Administration-approved medication in the United States for HD, the focus of this review will be on its pharmacology, efficacy, safety, and practical uses. There are no current treatments to change the course of HD, but education and symptomatic therapies can be effective tools for clinicians to use with patients and families affected by HD.
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