RT Journal Article T1 Ocular Involvement in Friedreich Ataxia Patients and its Relationship with Neurological Disability, a Follow-up Study A1 Rojas Lozano, María Del Pilar A1 Ramírez Sebastián, Ana Isabel A1 Hoz Montañana, María Rosa De A1 Cadena Santoyo, Manuel A1 Ferreras Amez, Antonio A1 Monsalve Córdova, Blanca A1 García Martín, Elena Salobrar A1 Muñóz Blanco, José L. A1 Urcelay Segura, José Luis A1 Salazar Corral, Juan José A1 Ramírez Sebastián, José Manuel AB 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. PB MDPI SN 2075-4418 YR 2020 FD 2020-01-29 LK https://hdl.handle.net/20.500.14352/6042 UL https://hdl.handle.net/20.500.14352/6042 LA eng NO Rojas Lozano, P., Ramírez Sebastián, A. I., Hoz Momtañana, M. R. et al. «Ocular Involvement in Friedreich Ataxia Patients and Its Relationship with Neurological Disability, a Follow-Up Study». Diagnostics, vol. 10, n.o 2, enero de 2020, p. 75. DOI.org (Crossref), https://doi.org/10.3390/diagnostics10020075. NO (This article belongs to the Section Pathology and Molecular Diagnostics)Received: 24 December 2019 / Revised: 28 January 2020 / Accepted: 29 January 2020 / Published: 29 January 2020 DS Docta Complutense RD 22 abr 2025