Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients

Spectral domain optical coherence tomography (SD-OCT) allows noninvasive measurements of retinal neuron layers. Here, we evaluate the relationship between clinical features and anatomical SD-OCT measurements in patients with spinocerebellar ataxia type 3 (SCA3) and how they change with time. A retro...

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Autores: Camós Carreras, Anna, Figueras Roca, Marc, Dotti Boada, Marina, Alcubierre, Rafel, Casaroli Marano, Ricardo Pedro, Muñoz, Esteban, Sánchez Dalmau, Bernardo
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2024
País:España
Recursos:Universidad de Barcelona
Repositorio:Dipòsit Digital de la UB
OAI Identifier:oai:diposit.ub.edu:2445/220547
Acesso em linha:https://hdl.handle.net/2445/220547
Access Level:acceso abierto
Palavra-chave:Retina
Neurologia
Tomografia de coherència òptica
Malalties neurodegeneratives
Axons
Neurology
Optical coherence tomography
Neurodegenerative Diseases
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spelling Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 PatientsCamós Carreras, AnnaFigueras Roca, MarcDotti Boada, MarinaAlcubierre, RafelCasaroli Marano, Ricardo PedroMuñoz, EstebanSánchez Dalmau, BernardoRetinaNeurologiaTomografia de coherència òpticaMalalties neurodegenerativesAxonsRetinaNeurologyOptical coherence tomographyNeurodegenerative DiseasesAxonsSpectral domain optical coherence tomography (SD-OCT) allows noninvasive measurements of retinal neuron layers. Here, we evaluate the relationship between clinical features and anatomical SD-OCT measurements in patients with spinocerebellar ataxia type 3 (SCA3) and how they change with time. A retrospective review was conducted on SCA3 patients. Clinical variables such as disease duration, number of CAG repeats, and the Scale for the Assessment and Rating of Ataxia (SARA) score were correlated with SD-OCT measurements, including retinal nerve fiber layer (RNFL) thickness, ganglion cell complex (GCC) thickness, macular volume (MV), and central macular thickness (CMT). Seventeen SCA3 patients with an average follow-up of 44.9 months were recruited. Clinical features with significant baseline correlations with SD-OCT measurements included disease duration (CMT r = - 0.590; GCC r = - 0.585), SARA score (CMT r = - 0.560; RNFL r = - 0.390), and number of CAG repeats (MV r = - 0.552; RNFL r = - 0.503; GCC r = - 0.493). The annual rate of change of the SARA score during follow-up was associated with that of both the MV (r = - 0.494; p = 0.005) and GCC thickness (r = - 0.454; p = 0.012). High disability (stages 2 and 3) was independently inversely associated with the annual change in MV (ß coefficient - 17.09; p = 0.025). This study provides evidence of an association between clinical features and objective anatomical measurements obtained by SD-OCT in SCA3 patients. MV and GCC thickness could serve as potential biomarkers of disease severity, as their rates of decrease seem to be related to a worsening in the SARA score. These findings highlight the potential of SD-OCT as a noninvasive tool for assessing disease severity and progression in SCA3 patients.<br /><br />Springer Verlag2024info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://hdl.handle.net/2445/220547Articles publicats en revistes (Cirurgia i Especialitats Medicoquirúrgiques)reponame:Dipòsit Digital de la UBinstname:Universidad de BarcelonaInglésReproducció del document publicat a: https://doi.org/10.1007/s12311-023-01634-1The Cerebellum, 2024, vol. 23, num.4, p. 1348-1354https://doi.org/10.1007/s12311-023-01634-1cc by (c) Camós-Carreras, Anna et al., 2024https://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:diposit.ub.edu:2445/2205472026-05-27T06:46:51Z
dc.title.none.fl_str_mv Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients
title Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients
spellingShingle Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients
Camós Carreras, Anna
Retina
Neurologia
Tomografia de coherència òptica
Malalties neurodegeneratives
Axons
Retina
Neurology
Optical coherence tomography
Neurodegenerative Diseases
Axons
title_short Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients
title_full Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients
title_fullStr Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients
title_full_unstemmed Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients
title_sort Progression of Retinal Ganglion Cell and Nerve Fiber Layer Loss in Spinocerebellar Ataxia 3 Patients
dc.creator.none.fl_str_mv Camós Carreras, Anna
Figueras Roca, Marc
Dotti Boada, Marina
Alcubierre, Rafel
Casaroli Marano, Ricardo Pedro
Muñoz, Esteban
Sánchez Dalmau, Bernardo
author Camós Carreras, Anna
author_facet Camós Carreras, Anna
Figueras Roca, Marc
Dotti Boada, Marina
Alcubierre, Rafel
Casaroli Marano, Ricardo Pedro
Muñoz, Esteban
Sánchez Dalmau, Bernardo
author_role author
author2 Figueras Roca, Marc
Dotti Boada, Marina
Alcubierre, Rafel
Casaroli Marano, Ricardo Pedro
Muñoz, Esteban
Sánchez Dalmau, Bernardo
author2_role author
author
author
author
author
author
dc.subject.none.fl_str_mv Retina
Neurologia
Tomografia de coherència òptica
Malalties neurodegeneratives
Axons
Retina
Neurology
Optical coherence tomography
Neurodegenerative Diseases
Axons
topic Retina
Neurologia
Tomografia de coherència òptica
Malalties neurodegeneratives
Axons
Retina
Neurology
Optical coherence tomography
Neurodegenerative Diseases
Axons
description Spectral domain optical coherence tomography (SD-OCT) allows noninvasive measurements of retinal neuron layers. Here, we evaluate the relationship between clinical features and anatomical SD-OCT measurements in patients with spinocerebellar ataxia type 3 (SCA3) and how they change with time. A retrospective review was conducted on SCA3 patients. Clinical variables such as disease duration, number of CAG repeats, and the Scale for the Assessment and Rating of Ataxia (SARA) score were correlated with SD-OCT measurements, including retinal nerve fiber layer (RNFL) thickness, ganglion cell complex (GCC) thickness, macular volume (MV), and central macular thickness (CMT). Seventeen SCA3 patients with an average follow-up of 44.9 months were recruited. Clinical features with significant baseline correlations with SD-OCT measurements included disease duration (CMT r = - 0.590; GCC r = - 0.585), SARA score (CMT r = - 0.560; RNFL r = - 0.390), and number of CAG repeats (MV r = - 0.552; RNFL r = - 0.503; GCC r = - 0.493). The annual rate of change of the SARA score during follow-up was associated with that of both the MV (r = - 0.494; p = 0.005) and GCC thickness (r = - 0.454; p = 0.012). High disability (stages 2 and 3) was independently inversely associated with the annual change in MV (ß coefficient - 17.09; p = 0.025). This study provides evidence of an association between clinical features and objective anatomical measurements obtained by SD-OCT in SCA3 patients. MV and GCC thickness could serve as potential biomarkers of disease severity, as their rates of decrease seem to be related to a worsening in the SARA score. These findings highlight the potential of SD-OCT as a noninvasive tool for assessing disease severity and progression in SCA3 patients.<br /><br />
publishDate 2024
dc.date.none.fl_str_mv 2024
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://hdl.handle.net/2445/220547
url https://hdl.handle.net/2445/220547
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Reproducció del document publicat a: https://doi.org/10.1007/s12311-023-01634-1
The Cerebellum, 2024, vol. 23, num.4, p. 1348-1354
https://doi.org/10.1007/s12311-023-01634-1
dc.rights.none.fl_str_mv cc by (c) Camós-Carreras, Anna et al., 2024
https://creativecommons.org/licenses/by/4.0/
info:eu-repo/semantics/openAccess
rights_invalid_str_mv cc by (c) Camós-Carreras, Anna et al., 2024
https://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Springer Verlag
publisher.none.fl_str_mv Springer Verlag
dc.source.none.fl_str_mv Articles publicats en revistes (Cirurgia i Especialitats Medicoquirúrgiques)
reponame:Dipòsit Digital de la UB
instname:Universidad de Barcelona
instname_str Universidad de Barcelona
reponame_str Dipòsit Digital de la UB
collection Dipòsit Digital de la UB
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15,811543

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