The objective of this study is to evaluate the tolerability and preliminary efficacy of levetiracetam (LEV) in reducing chorea in Huntington's disease (HD) patients in a prospective open-label pilot study. Nine HD patients with chorea were treated with LEV in doses up to 3,000 mg/day for up to 48 days. The primary endpoint measure was the Unified Huntington's Disease Rating Scale (UHDRS) chorea subscore. The mean dose (+/-SD) of LEV at endpoint was 2,583.3 +/- 1,020.6 mg/day. Mean UHDRS chorea score decreased from 12.6 +/- 3.0 at baseline to 6.7 +/- 4.3 at endpoint (P = 0.01). There was no significant change in UHDRS total motor scores (38.8 +/- 11.4 at baseline and 33.6 +/- 26.7 at endpoint; P = 0.24). Somnolence contributed to a 33% drop-out rate, and 3 patients developed Parkinsonism. Results of this open label study suggest that LEV may be efficacious in reducing chorea in HD patients.