24 adults, between the ages of 18 and 75 years, with cerebellar ataxia will be enrolled in a
12 week trial of BHV-4157 for treatment of ataxia. BHV-4157 is a pro-drug of riluzole (which
is currently FDA-approved for ALS, Lou Gehrig's disease). There will be 5 visits to UCLA
required--Screening when general and neurological examination, blood and urine testing, ECG,
and questionnaires will be administered; Baseline when general and neurological examination
and questionnaires will be administered and study drug dispensed; Week 4 and Week 12 when
general and neurological examination, blood and urine testing, ECG, and questionnaires will
be administered; 2 weeks after finishing study drug when general examination and blood
testing will be completed. There is an option for a 36 week extension of the study drug
trial.