Association of Crus I With Cerebellar Cognitive Affective Syndrome in Individuals With Friedreich Ataxia

Friedreich ataxia (FA) is an inherited neurodegenerative disease characterized by progressive cerebellar and systemic dysfunction. In addition to motor impairment, individuals with FA frequently display cognitive and affective disturbances, referred to as cerebellar cognitive affective syndrome (CCAS). The CCAS scale (CCAS-S) is a validated tool to assess these symptoms in clinical and research settings. Previous studies suggest that Crus I, a posterior cerebellar region involved in executive and salience networks, may play a role in the pathophysiology of CCAS. However, the structural and functional basis of CCAS in FA remains incompletely understood. The aim of this study was to assess whether structural atrophy and functional connectivity changes in Crus I are associated with CCAS in individuals with FA.