Movement Disorders (revue)

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Riluzole therapy in Huntington's disease (HD)

Identifieur interne : 004C50 ( Main/Exploration ); précédent : 004C49; suivant : 004C51

Riluzole therapy in Huntington's disease (HD)

Auteurs : H. Diana Rosas [États-Unis] ; Walter J. Koroshetz [États-Unis] ; Bruce G. Jenkins [États-Unis] ; Y. Iris Chen ; Douglas L. Hayden [États-Unis] ; M. Flint Beal [États-Unis] ; Merit E. Cudkowicz [États-Unis]

Source :

RBID : ISTEX:7A70222DCD70873629959102D42BFCB7987DC0C0

Descripteurs français

English descriptors

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.

Url:
DOI: 10.1002/1531-8257(199903)14:2<326::AID-MDS1019>3.0.CO;2-Q


Affiliations:


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Le document en format XML

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<div type="abstract" xml:lang="en">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.</div>
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