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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Detalles Bibliográficos
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
Descripción
Sumario: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.

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