Clinical trial of acetazolamide in SCA6, with assessment using the Ataxia Rating Scale and body stabilometry

Objective– To investigate the effect of acetazolamide on spinocerebellar ataxia type 6 (SCA6). Methods– Acetazolamide (250–500 mg/day) was administered orally for 88 weeks to 6 patients with SCA6, and its effect was quantitatively monitored using the Ataxia Rating Scale (ARS) and body sway analysis by stabilometry. Results– During administration of acetazolamide, the ARS score and the amplitude of body sway were significantly reduced compared with before administration. However, the response became weaker after 1 year of treatment. Conclusion– Although this was an open trial, the results suggested that acetazolamide can temporarily reduce the severity of symptoms during the progression of SCA6.