Two-year decline in performance on the Cerebellar Cognitive Affective Syndrome Scale in spinocerebellar ataxias

The 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.