Cargando…
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...
Autores principales: | , , , , , , , , , , |
---|---|
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 |
_version_ | 1783523742039670784 |
---|---|
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. |
format | Online Article Text |
id | pubmed-7168917 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT rojaspilar ocularinvolvementinfriedreichataxiapatientsanditsrelationshipwithneurologicaldisabilityafollowupstudy AT ramirezanai ocularinvolvementinfriedreichataxiapatientsanditsrelationshipwithneurologicaldisabilityafollowupstudy AT dehozrosa ocularinvolvementinfriedreichataxiapatientsanditsrelationshipwithneurologicaldisabilityafollowupstudy AT cadenamanuel ocularinvolvementinfriedreichataxiapatientsanditsrelationshipwithneurologicaldisabilityafollowupstudy AT ferrerasantonio ocularinvolvementinfriedreichataxiapatientsanditsrelationshipwithneurologicaldisabilityafollowupstudy AT monsalveblanca ocularinvolvementinfriedreichataxiapatientsanditsrelationshipwithneurologicaldisabilityafollowupstudy AT salobrargarciaelena ocularinvolvementinfriedreichataxiapatientsanditsrelationshipwithneurologicaldisabilityafollowupstudy AT munozblancojosel ocularinvolvementinfriedreichataxiapatientsanditsrelationshipwithneurologicaldisabilityafollowupstudy AT urcelaysegurajosel ocularinvolvementinfriedreichataxiapatientsanditsrelationshipwithneurologicaldisabilityafollowupstudy AT salazarjuanj ocularinvolvementinfriedreichataxiapatientsanditsrelationshipwithneurologicaldisabilityafollowupstudy AT ramirezjosem ocularinvolvementinfriedreichataxiapatientsanditsrelationshipwithneurologicaldisabilityafollowupstudy |