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

Parkinson's Disease Center and Movement Disorders Clinic, Department of Neurology, Baylor College of Medicine, Houston, Texas 77030, USA.
Movement Disorders (Impact Factor: 5.63). 02/2007; 22(1):10-3. DOI: 10.1002/mds.21161
Source: PubMed

ABSTRACT 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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    ABSTRACT: Huntington's 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 family members 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 interventions have yielded positive results and current treatments have focused on the motor aspects of HD. Tetrabenazine 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 neuroleptic agents, such as olanzapine and aripiprazole, may have adequate efficacy with a more favorable adverse effect profile than older neuroleptic agents for treating chorea and psychosis. 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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    ABSTRACT: Basal ganglia are group of subcortical nuclei which are components of modular circuits involving cerebral cortex, thalamus and brain stem related with cortical functions. Basal ganglia includes four to five distinct loop which is responsible for parallel processing of information and grouped into two distinct direct and indirect pathways. Thus, this dual system provides a motor centre exhibiting both excitatory and inhibitory effect. These circuits are involved in movement disorders categorized as Hyperkinetic and Hypokinetic movement disorder. In recent year, with the upcoming studies it has been clear that functions of basal ganglia not only focuses motor disturbances but also involves cognitive and emotional functions as well.
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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.
    Journal of the Neurological Sciences 10/2014; · 2.26 Impact Factor

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