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Article

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

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Hospital General Universitario Gregorio Marañón, Instituto Oftálmico de Madrid, 28007 Madrid, Spain
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Instituto de Investigaciones Oftalmológicas Ramón Castroviejo, Universidad Complutense de Madrid, 28040 Madrid, Spain
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Departamento de Inmunología, Oftalmología y ORL, Facultad de Óptica y Optometría, Universidad Complutense de Madrid, 28037 Madrid, Spain
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Hospital Universitario Miguel Servet, Instituto Aragonés de Ciencias de la Salud, 50009 Zaragoza, Spain
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Departamento de Inmunología, Oftalmología y ORL, Facultad de Medicina, Universidad Complutense de Madrid, 28040 Madrid, Spain
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Instituto de Investigación Sanitaria Gregorio Marañón, Unidad ALS-Neuromuscular, Departamento de Neurología, 28007 Madrid, Spain
*
Authors to whom correspondence should be addressed.
These authors contributed equally to this work.
Diagnostics 2020, 10(2), 75; https://doi.org/10.3390/diagnostics10020075
Received: 24 December 2019 / Revised: 28 January 2020 / Accepted: 29 January 2020 / Published: 29 January 2020
(This article belongs to the Section Pathology and Molecular Diagnostics)
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. View Full-Text
Keywords: OCT; Friedreich ataxia; FRDA; SARA; neurodegeneration; neurological disability; visual field OCT; Friedreich ataxia; FRDA; SARA; neurodegeneration; neurological disability; visual field
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MDPI and ACS Style

Rojas, P.; Ramírez, A.I.; Hoz, R.d.; Cadena, M.; Ferreras, A.; Monsalve, B.; Salobrar-García, E.; Muñoz-Blanco, J.L.; Urcelay-Segura, J.L.; Salazar, J.J.; Ramírez, J.M. Ocular Involvement in Friedreich Ataxia Patients and Its Relationship with Neurological Disability, a Follow-Up Study. Diagnostics 2020, 10, 75. https://doi.org/10.3390/diagnostics10020075

AMA Style

Rojas P, Ramírez AI, Hoz Rd, Cadena M, Ferreras A, Monsalve B, Salobrar-García E, Muñoz-Blanco JL, Urcelay-Segura JL, Salazar JJ, Ramírez JM. Ocular Involvement in Friedreich Ataxia Patients and Its Relationship with Neurological Disability, a Follow-Up Study. Diagnostics. 2020; 10(2):75. https://doi.org/10.3390/diagnostics10020075

Chicago/Turabian Style

Rojas, Pilar, Ana I. Ramírez, Rosa d. Hoz, Manuel Cadena, Antonio Ferreras, Blanca Monsalve, Elena Salobrar-García, José L. Muñoz-Blanco, José L. Urcelay-Segura, Juan J. Salazar, and José M. Ramírez 2020. "Ocular Involvement in Friedreich Ataxia Patients and Its Relationship with Neurological Disability, a Follow-Up Study" Diagnostics 10, no. 2: 75. https://doi.org/10.3390/diagnostics10020075

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