Open AccessCerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia
Author(s) -
Naeije Gilles,
Rai Myriam,
Allaerts Nick,
Sjogard Martin,
De Tiège Xavier,
Pandolfo Massimo
Publication year - 2020
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.51079
Subject(s) - ataxia , dentate nucleus , neuroscience , cerebellum , medicine , cerebellar ataxia , brainstem , cognition , motor cortex , cerebellar cortex , psychology , stimulation
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.