Riluzole therapy in Huntington's disease (HD)

Mov Disord. 1999 Mar;14(2):326-30. doi: 10.1002/1531-8257(199903)14:2<326::aid-mds1019>;2-q.


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.

Publication types

  • Clinical Trial
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Basal Ganglia / drug effects
  • Basal Ganglia / metabolism
  • Chorea / drug therapy
  • Excitatory Amino Acid Antagonists / adverse effects
  • Excitatory Amino Acid Antagonists / therapeutic use*
  • Female
  • Humans
  • Huntington Disease / drug therapy*
  • Huntington Disease / metabolism
  • Lactic Acid / metabolism
  • Magnetic Resonance Spectroscopy
  • Male
  • Middle Aged
  • Neuroprotective Agents / adverse effects
  • Neuroprotective Agents / therapeutic use*
  • Occipital Lobe / drug effects
  • Occipital Lobe / metabolism
  • Pilot Projects
  • Riluzole / adverse effects
  • Riluzole / therapeutic use*
  • Severity of Illness Index
  • Single-Blind Method
  • Treatment Outcome


  • Excitatory Amino Acid Antagonists
  • Neuroprotective Agents
  • Lactic Acid
  • Riluzole