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Ocular Involvement in Friedreich Ataxia Patients and Its Relationship with Neurological Disability, a Follow-Up Study

Background: This study compared functional and structural visual changes in Friedreich ataxia (FRDA) patients with healthy controls (HC) and correlated these changes with neurological disability. Methods: Eight FRDA Spanish patients and eight HC were selected from 2014 to 2018. Best corrected visual...

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Autores principales: Rojas, Pilar, Ramírez, Ana I., de Hoz, Rosa, Cadena, Manuel, Ferreras, Antonio, Monsalve, Blanca, Salobrar-García, Elena, Muñoz-Blanco, José L., Urcelay-Segura, José L., Salazar, Juan J., Ramírez, José M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7168917/
https://www.ncbi.nlm.nih.gov/pubmed/32013267
http://dx.doi.org/10.3390/diagnostics10020075
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author Rojas, Pilar
Ramírez, Ana I.
de Hoz, Rosa
Cadena, Manuel
Ferreras, Antonio
Monsalve, Blanca
Salobrar-García, Elena
Muñoz-Blanco, José L.
Urcelay-Segura, José L.
Salazar, Juan J.
Ramírez, José M.
author_facet Rojas, Pilar
Ramírez, Ana I.
de Hoz, Rosa
Cadena, Manuel
Ferreras, Antonio
Monsalve, Blanca
Salobrar-García, Elena
Muñoz-Blanco, José L.
Urcelay-Segura, José L.
Salazar, Juan J.
Ramírez, José M.
author_sort Rojas, Pilar
collection PubMed
description Background: This study compared functional and structural visual changes in Friedreich ataxia (FRDA) patients with healthy controls (HC) and correlated these changes with neurological disability. Methods: Eight FRDA Spanish patients and eight HC were selected from 2014 to 2018. Best corrected visual acuity (BCVA), visual field (VF), optic coherence tomography (OCT), and neurological disability measured by “scale for the assessment and rating of ataxia” (SARA) were taken in a basal exploration and repeated after 6 months. A linear mixed analysis and Bonferroni p-value correction were performed. Results: FRDA baseline and follow-up patients showed statistically significant decreases in BCVA, VF, and OCT parameters compared with the HC. Some of the VF measurements and most of the OCT parameters had an inverse mild-to-strong correlation with SARA. Moreover, the analysis of the ROC curve demonstrated that the peripapillary retinal nerve fiber layer (pRNFL) average thickness was the best parameter to discriminate between FRDA patients and HC. Conclusions: The follow-up study showed a progression in OCT parameters. Findings showed a sequential effect in pRNFL, ganglion cell complex (GCC), and macula. The VF and the OCT could be useful biomarkers in FRDA, both for their correlation with neurological disease as well as for their ability to evaluate disease progression.
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spelling pubmed-71689172020-04-20 Ocular Involvement in Friedreich Ataxia Patients and Its Relationship with Neurological Disability, a Follow-Up Study Rojas, Pilar Ramírez, Ana I. de Hoz, Rosa Cadena, Manuel Ferreras, Antonio Monsalve, Blanca Salobrar-García, Elena Muñoz-Blanco, José L. Urcelay-Segura, José L. Salazar, Juan J. Ramírez, José M. Diagnostics (Basel) Article Background: This study compared functional and structural visual changes in Friedreich ataxia (FRDA) patients with healthy controls (HC) and correlated these changes with neurological disability. Methods: Eight FRDA Spanish patients and eight HC were selected from 2014 to 2018. Best corrected visual acuity (BCVA), visual field (VF), optic coherence tomography (OCT), and neurological disability measured by “scale for the assessment and rating of ataxia” (SARA) were taken in a basal exploration and repeated after 6 months. A linear mixed analysis and Bonferroni p-value correction were performed. Results: FRDA baseline and follow-up patients showed statistically significant decreases in BCVA, VF, and OCT parameters compared with the HC. Some of the VF measurements and most of the OCT parameters had an inverse mild-to-strong correlation with SARA. Moreover, the analysis of the ROC curve demonstrated that the peripapillary retinal nerve fiber layer (pRNFL) average thickness was the best parameter to discriminate between FRDA patients and HC. Conclusions: The follow-up study showed a progression in OCT parameters. Findings showed a sequential effect in pRNFL, ganglion cell complex (GCC), and macula. The VF and the OCT could be useful biomarkers in FRDA, both for their correlation with neurological disease as well as for their ability to evaluate disease progression. MDPI 2020-01-29 /pmc/articles/PMC7168917/ /pubmed/32013267 http://dx.doi.org/10.3390/diagnostics10020075 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Rojas, Pilar
Ramírez, Ana I.
de Hoz, Rosa
Cadena, Manuel
Ferreras, Antonio
Monsalve, Blanca
Salobrar-García, Elena
Muñoz-Blanco, José L.
Urcelay-Segura, José L.
Salazar, Juan J.
Ramírez, José M.
Ocular Involvement in Friedreich Ataxia Patients and Its Relationship with Neurological Disability, a Follow-Up Study
title Ocular Involvement in Friedreich Ataxia Patients and Its Relationship with Neurological Disability, a Follow-Up Study
title_full Ocular Involvement in Friedreich Ataxia Patients and Its Relationship with Neurological Disability, a Follow-Up Study
title_fullStr Ocular Involvement in Friedreich Ataxia Patients and Its Relationship with Neurological Disability, a Follow-Up Study
title_full_unstemmed Ocular Involvement in Friedreich Ataxia Patients and Its Relationship with Neurological Disability, a Follow-Up Study
title_short Ocular Involvement in Friedreich Ataxia Patients and Its Relationship with Neurological Disability, a Follow-Up Study
title_sort ocular involvement in friedreich ataxia patients and its relationship with neurological disability, a follow-up study
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7168917/
https://www.ncbi.nlm.nih.gov/pubmed/32013267
http://dx.doi.org/10.3390/diagnostics10020075
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