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Updated: Dec 19, 2025

Cerebellar Regional Dissection for Molecular Analysis
Published on: December 5, 2020
Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia
Gilles Naeije1,2, Myriam Rai3, Nick Allaerts2
1Laboratoire de Cartographie Fonctionnelle du Cerveau, ULB Neuroscience Institute, Université libre de Bruxelles (ULB), Brussels, Belgium.
Abstract:
Dentate nuclei (DN) are involved in cerebellar modulation of motor and cognitive functions, whose impairment causes ataxia and cerebellar cognitive affective syndrome (CCAS). Friedreich ataxia (FRDA) disease progression relates to degeneration of the dentate nucleus and dentato-thalamic pathways, causing cerebellar ataxia. Volumetric MRI also shows mild loss in the cerebellar cortex, brainstem, and motor cortex. Cognitive deficits occur in FRDA, but their relationship with ataxia progression is not fully characterized. We found a significant positive correlation between severity of patients' ataxia and more marked CCAS as assessed with the CCAS-Scale. This relation could be related to progressive DN impairment.
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