ArticleJournal of neurology2025
Two-year decline in performance on the Cerebellar Cognitive Affective Syndrome Scale in spinocerebellar ataxias.
Article in Journal of neurology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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Who cites it
1 citing paper in PubMed.
- Remote Online-Based Application of the Cerebellar Cognitive Affective Syndrome Scale.Cerebellum (London, England) · 2026Article
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Authors and funding
7 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
objectivesCognitive deficits are observed in a subset of individuals with spinocerebellar ataxias (SCAs); however, there is limited research on the longitudinal trajectory of such deficits. We investigated longitudinal cognitive performance amongst individuals with SCAs relative to controls, and the relationship of change in cognitive performance with change in self-reported function.
methodsIndividuals with symptomatic SCA1, SCA2, SCA3, and SCA6 as well as unaffected controls were administered the Cerebellar Cognitive Affective Syndrome Scale (CCAS-S) at yearly intervals for up to two years. Participants with SCA self-reported day-to-day cognitive and motor function via annual completion of the Patient-Reported Outcome Measure of Ataxia.
resultsSignificant two-year decline in CCAS-S performance was observed in individuals with SCA (n = 25). In a larger cohort of individuals who completed 1-year follow-up (n = 45 SCA), greater decline was observed among individuals with better baseline performance, and, at a trending level, those with milder motor ataxia severity. Greater one-year decline on the CCAS-S was significantly associated with greater decline in self-reported day-to-day cognitive function. In contrast, one-year CCAS-S change was not related to change in self-reported motor function.
conclusionsThe CCAS-S indexes functionally relevant cognitive change over time in SCAs, with this decline apparent in at least a subset of individuals with SCAs. Decline in cognitive performance may be particularly pronounced in early/mild disease stages. Our results motivate larger and longer-term studies of the CCAS-S in SCAs, including investigating cognitive change across the pre-symptomatic and symptomatic disease course, and identifying risk factors for cognitive decline, to inform appropriate clinical support.
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