Riluzole therapy in Huntington's disease (HD)

We conducted a 6-week open-label trial of riluzole (50 mg twice a day) in e ight subjects with Huntington's disease. Subjects were evaluated before ril uzole treatment, on treatment, and off treatment with the chorea, dystonia, and total functional capacity (TFC) scores from the Unified Huntington's D isease Rating Scale and magnetic resonance spectroscopy measurements of occ ipital cortex and basal ganglia lactate levels. Adverse events and safety b lood and urine tests were assessed throughout the study. All subjects compl eted the study and riluzole was well tolerated. The age was 45 +/- 10.2 yea rs (mean +/- standard deviation) and the disease duration was 6.1 +/- 4.1 y ears. The chorea rating score improved by 35% on treatment (p = 0.013) and worsened alter discontinuation of treatment (p = 0.026). There were no sign ificant treatment effects on the dystonia or TFC scores. The baseline occip ital 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. Additiona l clinical studies of riluzole for both symptomatic and neuroprotective ben efit in Huntington's disease are warranted.