Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia

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, ca...

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Autores principales: Naeije, Gilles, Rai, Myriam, Allaerts, Nick, Sjogard, Martin, De Tiège, Xavier, Pandolfo, Massimo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2020
Materias:
Brief Communication
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7317641/
https://www.ncbi.nlm.nih.gov/pubmed/32510804
http://dx.doi.org/10.1002/acn3.51079
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author Naeije, Gilles
Rai, Myriam
Allaerts, Nick
Sjogard, Martin
De Tiège, Xavier
Pandolfo, Massimo
author_facet Naeije, Gilles
Rai, Myriam
Allaerts, Nick
Sjogard, Martin
De Tiège, Xavier
Pandolfo, Massimo
author_sort Naeije, Gilles
collection PubMed
description 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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spelling pubmed-73176412020-06-29 Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia Naeije, Gilles Rai, Myriam Allaerts, Nick Sjogard, Martin De Tiège, Xavier Pandolfo, Massimo Ann Clin Transl Neurol Brief Communication 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. John Wiley and Sons Inc. 2020-06-08 /pmc/articles/PMC7317641/ /pubmed/32510804 http://dx.doi.org/10.1002/acn3.51079 Text en © 2020 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Brief Communication
Naeije, Gilles
Rai, Myriam
Allaerts, Nick
Sjogard, Martin
De Tiège, Xavier
Pandolfo, Massimo
Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia
title Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia
title_full Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia
title_fullStr Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia
title_full_unstemmed Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia
title_short Cerebellar cognitive disorder parallels cerebellar motor symptoms in Friedreich ataxia
title_sort cerebellar cognitive disorder parallels cerebellar motor symptoms in friedreich ataxia
topic Brief Communication
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7317641/
https://www.ncbi.nlm.nih.gov/pubmed/32510804
http://dx.doi.org/10.1002/acn3.51079
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