Next Article in Journal
Degradation-Aware Deep Learning Framework for Sparse-View CT Reconstruction
Previous Article in Journal
Diastolic Cardiac Function by MRI—Imaging Capabilities and Clinical Applications
 
 
Search for Articles:
Title / Keyword
Author / Affiliation / Email
Journal
Article Type
 
 
Advanced Search
 
Section
Special Issue
Volume
Issue
Number
Page
 
Journals
Tomography
Volume 7
Issue 4
10.3390/tomography7040076
tomography-logo
Submit to this Journal Review for this Journal Propose a Special Issue
► Article Menu

Article Menu

share Share announcement Help format_quote Cite question_answer Discuss in SciProfiles
first_page
settings
Order Article Reprints
Font Type:
Arial Georgia Verdana
Font Size:
Aa Aa Aa
Line Spacing:
Column Width:
Background:
Article

Longitudinal Assessment Using Optical Coherence Tomography in Patients with Friedreich’s Ataxia

by
Petya Bogdanova-Mihaylova
1,*,
Helena Maria Plapp
2,
Hongying Chen
2,
Anne Early
3,
Lorraine Cassidy
3,
Richard A. Walsh
1,4,5,† and
Sinéad M. Murphy
1,5,†
1
National Ataxia Clinic, Department of Neurology, Tallaght University Hospital, Tallaght, Dublin 24, Ireland
2
School of Medicine, Trinity College Dublin, Dublin 2, Ireland
3
Department of Ophthalmology, Tallaght University Hospital, Dublin 24, Ireland
4
Dublin Neurological Institute at the Mater Hospital and University College Dublin, Dublin 7, Ireland
5
Academic Unit of Neurology, Trinity College Dublin, Dublin 2, Ireland
*
Author to whom correspondence should be addressed.
†
These authors contributed equally.
Tomography 2021, 7(4), 915-931; https://doi.org/10.3390/tomography7040076
Submission received: 8 November 2021 / Revised: 1 December 2021 / Accepted: 2 December 2021 / Published: 8 December 2021
(This article belongs to the Section Neuroimaging)
Browse Figures
Versions Notes

Abstract

Ocular abnormalities occur frequently in Friedreich’s ataxia (FRDA), although visual symptoms are not always reported. We evaluated a cohort of patients with FRDA to characterise the clinical phenotype and optic nerve findings as detected with optical coherence tomography (OCT). A total of 48 patients from 42 unrelated families were recruited. Mean age at onset was 13.8 years (range 4–40), mean disease duration 19.5 years (range 5–43), mean disease severity as quantified with the Scale for the Assessment and Rating of Ataxia 22/40 (range 4.5–38). All patients displayed variable ataxia and two-thirds had ocular abnormalities. Statistically significant thinning of average retinal nerve fibre layer (RNFL) and thinning in all but the temporal quadrant compared to controls was demonstrated on OCT. Significant RNFL and macular thinning was documented over time in 20 individuals. Disease severity and visual acuity were correlated with RNFL and macular thickness, but no association was found with disease duration. Our results highlight that FDRA is associated with subclinical optic neuropathy. This is the largest longitudinal study of OCT findings in FRDA to date, demonstrating progressive RNFL thickness decline, suggesting that RNFL thickness as measured by OCT has the potential to become a quantifiable biomarker for the evaluation of disease progression in FRDA.
Keywords: Friedreich’s ataxia; FRDA; optical coherence tomography; OCT; retinal nerve fibre layer; RNFL Friedreich’s ataxia; FRDA; optical coherence tomography; OCT; retinal nerve fibre layer; RNFL

Share and Cite

MDPI and ACS Style

Bogdanova-Mihaylova, P.; Plapp, H.M.; Chen, H.; Early, A.; Cassidy, L.; Walsh, R.A.; Murphy, S.M. Longitudinal Assessment Using Optical Coherence Tomography in Patients with Friedreich’s Ataxia. Tomography 2021, 7, 915-931. https://doi.org/10.3390/tomography7040076

AMA Style

Bogdanova-Mihaylova P, Plapp HM, Chen H, Early A, Cassidy L, Walsh RA, Murphy SM. Longitudinal Assessment Using Optical Coherence Tomography in Patients with Friedreich’s Ataxia. Tomography. 2021; 7(4):915-931. https://doi.org/10.3390/tomography7040076

Chicago/Turabian Style

Bogdanova-Mihaylova, Petya, Helena Maria Plapp, Hongying Chen, Anne Early, Lorraine Cassidy, Richard A. Walsh, and Sinéad M. Murphy. 2021. "Longitudinal Assessment Using Optical Coherence Tomography in Patients with Friedreich’s Ataxia" Tomography 7, no. 4: 915-931. https://doi.org/10.3390/tomography7040076

APA Style

Bogdanova-Mihaylova, P., Plapp, H. M., Chen, H., Early, A., Cassidy, L., Walsh, R. A., & Murphy, S. M. (2021). Longitudinal Assessment Using Optical Coherence Tomography in Patients with Friedreich’s Ataxia. Tomography, 7(4), 915-931. https://doi.org/10.3390/tomography7040076

Article Metrics

Back to TopTop