Riluzole therapy in Huntington's disease (HD)

Authors

  • H. Diana Rosas MD,

    Corresponding author
    1. Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
    2. MGH-NMR Center, Department of Radiology, Massachusetts General Hospital and Harvard Medical School, Charlestown, Massachusetts;
    • Massachusetts General Hospital, Neurology Service Warren 408, 32 Fruit Street, Boston, MA 02114, U.S.A.
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  • Walter J. Koroshetz MD,

    1. Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
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  • Bruce G. Jenkins PhD,

    1. Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
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  • Y. Iris Chen PhD,

    1. MGH-NMR Center, Department of Radiology, Massachusetts General Hospital and Harvard Medical School, Charlestown, Massachusetts;
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  • Douglas L. Hayden,

    1. General Clinical Research Center, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, U.S.A.
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  • M. Flint Beal MD,

    1. Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
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  • Merit E. Cudkowicz MD

    1. Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts
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Abstract

We conducted a 6-week open-label trial of riluzole (50 mg twice a day) in eight subjects with Huntington's disease. Subjects were evaluated before riluzole treatment, on treatment, and off treatment with the chorea, dystonia, and total functional capacity (TFC) scores from the Unified Huntington's Disease Rating Scale and magnetic resonance spectroscopy measurements of occipital cortex and basal ganglia lactate levels. Adverse events and safety blood and urine tests were assessed throughout the study. All subjects completed the study and riluzole was well tolerated. The age was 45 ± 10.2 years (mean ± standard deviation) and the disease duration was 6.1 ± 4.1 years. The chorea rating score improved by 35% on treatment (p = 0.013) and worsened after discontinuation of treatment (p = 0.026). There were no significant treatment effects on the dystonia or TFC scores. The baseline occipital and basal ganglia lactate levels were elevated in all subjects; there was a trend toward lower lactate/creatine ratios during riluzole treatment in the basal ganglia spectra but not in occipital cortex spectra. Additional clinical studies of riluzole for both symptomatic and neuroprotective benefit in Huntington's disease are warranted.

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