{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T17:59:27Z","timestamp":1789927167117,"version":"4.0.1"},"reference-count":27,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2011,5,28]],"date-time":"2011-05-28T00:00:00Z","timestamp":1306540800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2011,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We quantitatively investigated the clinical severity and progression of diseases with ataxia, as measured with the Scale for the Assessment and Rating of Ataxia, and examined the potential application of the Scale for the Assessment and Rating of Ataxia for future therapeutic trials. Severity of ataxia was assessed in 238 patients with spinocerebellar ataxia type 2, spinocerebellar ataxia type 3, spinocerebellar ataxia type 6, spinocerebellar ataxia type 17, multiple system atrophy\u2010cerebellar variant, or Gerstman\u2010Str\u00e4ussler\u2010Scheinker disease. Among them, 119 (50%) were longitudinally examined three to seven times, in a period of 8 to 38 months, resulting in a total set of 535 assessments. The differences between spinocerebellar ataxia and multiple system atrophy\u2010cerebellar variant were ascertained cross\u2010sectionally and longitudinally. Gerstman\u2010Str\u00e4ussler\u2010Scheinker disease had the fastest progression, followed by multiple system atrophy\u2010cerebellar variant, spinocerebellar ataxia type 17, spinocerebellar ataxia type 3, spinocerebellar ataxia type 2, and spinocerebellar ataxia type 6. Patients with multiple system atrophy\u2010cerebellar variant had a faster progression in gait, sitting, speech, and total score than patients with spinocerebellar ataxias. For a randomized, case\u2010control trial, a sample size of 47 for spinocerebellar ataxia and 85 for multiple system atrophy\u2010cerebellar variant in the treatment or placebo arms would have a sufficient statistical power to demonstrate the efficacy of a new therapy that would retard ataxia progression by 1 point per year as measured by the Scale for the Assessment and Rating of Ataxia. The results will have a significant impact on the planning and implementation of future therapeutic trials of spinocerebellar ataxia and multiple system atrophy\u2010cerebellar variant. \u00a9 2011 Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.23809","type":"journal-article","created":{"date-parts":[[2011,5,28]],"date-time":"2011-05-28T15:25:30Z","timestamp":1306596330000},"page":"2081-2087","source":"Crossref","is-referenced-by-count":61,"title":["Comparison of cerebellar ataxias: A three\u2010year prospective longitudinal 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