Prevalence, Severity, and Progression of Cerebellar Cognitive-Affective Syndrome in Patients With Spinocerebellar Ataxias

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BACKGROUND AND OBJECTIVES: Cerebellar cognitive-affective syndrome (CCAS) results from cerebellar degeneration, but its prevalence in spinocerebellar ataxias (SCAs) remains underexplored. This study assessed CCAS prevalence, severity, and progression across different SCAs. METHODS: /SCA27B and SPG7, and controls. Cognitive function was assessed with the CCAS scale and ataxia severity with the Scale for the Assessment and Rating of Ataxia (SARA) and Composite Cerebellar Functional Severity (CCFS) score. We correlated CCAS score with ataxia severity, brain MRI, and plasma neurofilament light chain (NfL) levels. Subtest comparisons among genotypes were adjusted for age, education, and SARA score. In PolyQ SCA carriers, we explored CCAS progression. RESULTS: = 0.52). DISCUSSION: We observed early cognitive impairment in PolyQ SCA carriers, correlating with clinical measures, NfL levels, and cerebellum volume. Improvement over 3 years likely reflects a practice effect, potentially limiting the scale's longitudinal utility.

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