Work

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

Year: 2025

Type: article

Abstract: 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.

Source: Neurology Open Access

DOI: 10.1212/wn9.0000000000000045

Authors: Virginie Destrebecq, Antonin Rovai, Nicola Trotta, Gilles Naeije

Institutions: Université Libre de Bruxelles, Erasmus Hospital

Language: English

Topic: Genetic Neurodegenerative Diseases

Subfield: Cellular and Molecular Neuroscience

Field: Neuroscience

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: diamond

Funder: Fonds De La Recherche Scientifique - FNRS