Clinical cerebellar neuroscience: cognitive functioning in spinocerebellar ataxias: an update and future directions

Abstract

Cognitive deficits are an increasingly well-recognised feature of spinocerebellar ataxias (SCAs). A growing body of research using the Cerebellar Cognitive Affective Syndrome Scale, as well as more targeted neuropsychological assessments, consistently supports the presence of multidomain cognitive impairments in the most common SCAs. However, it is also becoming clear that cognitive changes may impact only a subset of patients. Further research is necessary to understand the risk factors, demographic correlates and neural basis of this heterogeneity. Evidence that cognitive changes precede the onset of motor symptoms is emerging but remains equivocal, with many studies of pre-ataxic individuals limited by small sample sizes. Longitudinal assessments similarly suggest progressive cognitive decline over time, but the cognitive domains and tests that are most sensitive to change remain unclear. Finally, the functional impact of cognitive deficits on daily life in individuals with SCAs remains a significant knowledge gap.