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: Rojas Lozano, María Del Pilar, Ramírez Sebastián, Ana Isabel, Hoz Montañana, María Rosa De, Cadena Santoyo, Manuel, Ferreras Amez, Antonio, Monsalve Córdova, Blanca, García Martín, Elena Salobrar, Muñóz Blanco, José L., Urcelay Segura, José Luis, Salazar Corral, Juan José, Ramírez Sebastián, José Manuel
Tipo de recurso: artículo
Fecha de publicación:2020
País:España
Institución:Universidad Complutense de Madrid (UCM)
Repositorio:Docta Complutense
Idioma:inglés
OAI Identifier:oai:docta.ucm.es:20.500.14352/6042
Acceso en línea:https://hdl.handle.net/20.500.14352/6042
Access Level:acceso abierto
Palabra clave:617.731-073
616.8-009.26
OCT
Friedreich ataxia
FRDA
SARA
Neurodegeneration
Neurological disability
Visual field
Neurociencias (Medicina)
Oftalmología
Anatomía ocular
2490 Neurociencias
3201.09 Oftalmología
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oai_identifier_str oai:docta.ucm.es:20.500.14352/6042
network_acronym_str ES
network_name_str España
repository_id_str
spelling Ocular Involvement in Friedreich Ataxia Patients and its Relationship with Neurological Disability, a Follow-up StudyRojas Lozano, María Del PilarRamírez Sebastián, Ana IsabelHoz Montañana, María Rosa DeCadena Santoyo, ManuelFerreras Amez, AntonioMonsalve Córdova, BlancaGarcía Martín, Elena SalobrarMuñóz Blanco, José L.Urcelay Segura, José LuisSalazar Corral, Juan JoséRamírez Sebastián, José Manuel617.731-073616.8-009.26OCTFriedreich ataxiaFRDASARANeurodegenerationNeurological disabilityVisual fieldNeurociencias (Medicina)OftalmologíaAnatomía ocular2490 Neurociencias3201.09 OftalmologíaBackground: 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.MDPIUniversidad Complutense de Madrid20202020-01-2920202020-01-29journal articlehttp://purl.org/coar/resource_type/c_6501info:eu-repo/semantics/articleapplication/pdfhttps://hdl.handle.net/20.500.14352/6042reponame:Docta Complutenseinstname:Universidad Complutense de Madrid (UCM)Inglésengopen accesshttp://purl.org/coar/access_right/c_abf2Atribución 3.0 Españahttps://creativecommons.org/licenses/by/3.0/es/info:eu-repo/semantics/openAccessoai:docta.ucm.es:20.500.14352/60422026-06-02T12:44:21Z
dc.title.none.fl_str_mv 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
spellingShingle Ocular Involvement in Friedreich Ataxia Patients and its Relationship with Neurological Disability, a Follow-up Study
Rojas Lozano, María Del Pilar
617.731-073
616.8-009.26
OCT
Friedreich ataxia
FRDA
SARA
Neurodegeneration
Neurological disability
Visual field
Neurociencias (Medicina)
Oftalmología
Anatomía ocular
2490 Neurociencias
3201.09 Oftalmología
title_short 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_sort Ocular Involvement in Friedreich Ataxia Patients and its Relationship with Neurological Disability, a Follow-up Study
dc.creator.none.fl_str_mv Rojas Lozano, María Del Pilar
Ramírez Sebastián, Ana Isabel
Hoz Montañana, María Rosa De
Cadena Santoyo, Manuel
Ferreras Amez, Antonio
Monsalve Córdova, Blanca
García Martín, Elena Salobrar
Muñóz Blanco, José L.
Urcelay Segura, José Luis
Salazar Corral, Juan José
Ramírez Sebastián, José Manuel
author Rojas Lozano, María Del Pilar
author_facet Rojas Lozano, María Del Pilar
Ramírez Sebastián, Ana Isabel
Hoz Montañana, María Rosa De
Cadena Santoyo, Manuel
Ferreras Amez, Antonio
Monsalve Córdova, Blanca
García Martín, Elena Salobrar
Muñóz Blanco, José L.
Urcelay Segura, José Luis
Salazar Corral, Juan José
Ramírez Sebastián, José Manuel
author_role author
author2 Ramírez Sebastián, Ana Isabel
Hoz Montañana, María Rosa De
Cadena Santoyo, Manuel
Ferreras Amez, Antonio
Monsalve Córdova, Blanca
García Martín, Elena Salobrar
Muñóz Blanco, José L.
Urcelay Segura, José Luis
Salazar Corral, Juan José
Ramírez Sebastián, José Manuel
author2_role author
author
author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Universidad Complutense de Madrid
dc.subject.none.fl_str_mv 617.731-073
616.8-009.26
OCT
Friedreich ataxia
FRDA
SARA
Neurodegeneration
Neurological disability
Visual field
Neurociencias (Medicina)
Oftalmología
Anatomía ocular
2490 Neurociencias
3201.09 Oftalmología
topic 617.731-073
616.8-009.26
OCT
Friedreich ataxia
FRDA
SARA
Neurodegeneration
Neurological disability
Visual field
Neurociencias (Medicina)
Oftalmología
Anatomía ocular
2490 Neurociencias
3201.09 Oftalmología
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.
publishDate 2020
dc.date.none.fl_str_mv 2020
2020-01-29
2020
2020-01-29
dc.type.none.fl_str_mv journal article
http://purl.org/coar/resource_type/c_6501
dc.type.openaire.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://hdl.handle.net/20.500.14352/6042
url https://hdl.handle.net/20.500.14352/6042
dc.language.none.fl_str_mv Inglés
eng
language_invalid_str_mv Inglés
language eng
dc.rights.none.fl_str_mv open access
http://purl.org/coar/access_right/c_abf2
Atribución 3.0 España
https://creativecommons.org/licenses/by/3.0/es/
dc.rights.openaire.fl_str_mv info:eu-repo/semantics/openAccess
rights_invalid_str_mv open access
http://purl.org/coar/access_right/c_abf2
Atribución 3.0 España
https://creativecommons.org/licenses/by/3.0/es/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv MDPI
publisher.none.fl_str_mv MDPI
dc.source.none.fl_str_mv reponame:Docta Complutense
instname:Universidad Complutense de Madrid (UCM)
instname_str Universidad Complutense de Madrid (UCM)
reponame_str Docta Complutense
collection Docta Complutense
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15,300719

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