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The Role of Verbal Fluency in the Cerebellar Cognitive Affective Syndrome Scale in Friedreich Ataxia. | LitMetric

AI Article Synopsis

  • Cerebellar issues are linked to movement difficulties in Friedreich ataxia (FRDA), but their effect on cognitive functions is not well understood.
  • The study examined the relationship between cognitive performance, as measured by the Cerebellar Cognitive Affective Syndrome Scale (CCAS-S), and disease severity in 57 FRDA patients, noting that speech problems like dysarthria complicate test results.
  • Findings showed a strong connection between clinical metrics and CCAS-S performance, particularly in verbal fluency tasks, but the impact of speech intelligibility on scores indicates that more research is needed before using the CCAS-S as a reliable cognitive assessment for FRDA patients.

Article Abstract

Cerebellar pathology engenders the disturbance of movement that characterizes Friedreich ataxia (FRDA), yet the impact of cerebellar pathology on cognition in FRDA remains unclear. Numerous studies have unequivocally demonstrated the role of the cerebellar pathology in disturbed cognitive, language and affective regulation, referred to as Cerebellar Cognitive Affective Syndrome (CCAS), and quantified by the CCAS-Scale (CCAS-S). The presence of dysarthria in many individuals with ataxia, particularly FRDA, may confound results on some items of the CCAS-S resulting in false-positive scores. This study explored the relationship between performance on the CCAS-S and clinical metrics of disease severity in 57 adults with FRDA. In addition, this study explored the relationship between measures of intelligibility and naturalness of speech and scores on the CCAS-S in a subgroup of 39 individuals with FRDA. We demonstrated a significant relationship between clinical metrics and performance on the CCAS-S. In addition, we confirmed the items that returned the greatest rate of failure were based on Verbal Fluency Tasks, revealing a significant relationship between these items and measures of speech. Measures of speech explained over half of the variance in the CCAS-S score suggesting the role of dysarthria in the performance on the CCAS-S is not clear. Further work is required prior to adopting the CCAS-S as a cognitive screening tool for individuals with FRDA.

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Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11489268PMC
http://dx.doi.org/10.1007/s12311-024-01694-xDOI Listing

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