Optical coherence tomography shows early loss of the inferior temporal quadrant retinal nerve fiber layer in autosomal dominant optic atrophy

Abstract Background Progressive myelopathy is the main cause of disability in adrenoleukodystrophy (ALD). Development of therapies is hampered by a lack of quantitative outcome measures. In this study, we investigated whether myelopathy in ALD is associated with retinal neurodegeneration on optical coherence tomography (OCT), which could serve as a surrogate outcome measure. Methods Sixty-two patients (29 men and 33 women) and 70 age-matched and sex-matched controls (33 men and 37 women) were included in this cross-sectional study. We compared retinal nerve fiber layer (RNFL), ganglion cell layer (GCL) and peripapillary retinal nerve fiber layer (pRNFL) thickness between ALD patients and controls. In addition, we correlated these OCT measurements with clinical parameters of severity of myelopathy. Results Patients had significantly thinner RNFL (male group, p < 0.05) and pRNFL superior and temporal quadrant [both male (p < 0.005) and female (p < 0.05) groups] compared to controls. Comparing three groups (symptomatic patients, asymptomatic patients and controls), there were significant differences in RNFL thickness (total grid and peripheral ring) in the male group (p ≤ 0.002) and in pRNFL thickness (superior and temporal quadrant) in both male (p ≤ 0.02) and the female (p ≤ 0.02) groups. Neuroretinal layer thickness correlated moderately with severity of myelopathy in men (correlation coefficients between 0.29–0.55, p < 0.02), but not in women. Conclusions These results suggest that neurodegeneration of the spinal cord in ALD is reflected in the retina of patients with ALD. Therefore, OCT could be valuable as an outcome measure for the myelopathy of ALD. Additional longitudinal studies are ongoing.

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Measurement of retinal nerve fiber layer thickness in optic atrophy eyes of patients with optic neuritis using optical coherence tomography

Graefe s Archive for Clinical and Experimental Ophthalmology ◽

10.1007/s00417-010-1326-9 ◽

2010 ◽

Vol 248 (7) ◽

pp. 1013-1018 ◽

Cited By ~ 10

Author(s):

Xin-Ling Wang ◽

Tao Yu ◽

De-Zhao Xia ◽

Jin-Song Zhang ◽

Qi-chang Yan ◽

...

Keyword(s):

Optical Coherence Tomography ◽

Optic Neuritis ◽

Nerve Fiber ◽

Layer Thickness ◽

Retinal Nerve Fiber Layer ◽

Optic Atrophy ◽

Optical Coherence ◽

Fiber Layer ◽

Nerve Fiber Layer ◽

Nerve Fiber Layer Thickness

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Faculty Opinions recommendation of Retinal nerve fiber layer thickness in dominant optic atrophy measurements by optical coherence tomography and correlation with age.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.11433956.12457054 ◽

2011 ◽

Author(s):

Prem Subramanian ◽

Zoe Williams

Keyword(s):

Optical Coherence Tomography ◽

Nerve Fiber ◽

Layer Thickness ◽

Retinal Nerve Fiber Layer ◽

Optic Atrophy ◽

Optical Coherence ◽

Fiber Layer ◽

Nerve Fiber Layer ◽

Dominant Optic Atrophy ◽

Nerve Fiber Layer Thickness

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Faculty Opinions recommendation of Peripapillary Retinal Nerve Fiber Layer Vascular Microcirculation in Glaucoma Using Optical Coherence Tomography-Based Microangiography.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.726569944.793536592 ◽

2017 ◽

Author(s):

Christopher Leung

Keyword(s):

Optical Coherence Tomography ◽

Nerve Fiber ◽

Retinal Nerve Fiber Layer ◽

Optical Coherence ◽

Fiber Layer ◽

Nerve Fiber Layer ◽

Retinal Nerve Fiber

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Faculty Opinions recommendation of Automated segmentation errors when using optical coherence tomography to measure retinal nerve fiber layer thickness in glaucoma.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.726937566.793525178 ◽

2016 ◽

Author(s):

Julian Garcia-Feijoo ◽

Federico Saenz-Frances

Keyword(s):

Optical Coherence Tomography ◽

Nerve Fiber ◽

Layer Thickness ◽

Retinal Nerve Fiber Layer ◽

Automated Segmentation ◽

Optical Coherence ◽

Fiber Layer ◽

Nerve Fiber Layer ◽

Nerve Fiber Layer Thickness ◽

Retinal Nerve Fiber

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Retinal Optical Coherence Tomography Metrics Are Unchanged in Verubecestat Alzheimer’s Disease Clinical Trial but Correlate with Baseline Regional Brain Atrophy

Journal of Alzheimer s Disease ◽

10.3233/jad-200735 ◽

2021 ◽

Vol 79 (1) ◽

pp. 275-287

Author(s):

Robert C. Sergott ◽

Annaswamy Raji ◽

James Kost ◽

Cyrille Sur ◽

Saheeda Jackson ◽

...

Keyword(s):

Clinical Trial ◽

Optical Coherence Tomography ◽

Nerve Fiber ◽

Retinal Nerve Fiber Layer ◽

Brain Atrophy ◽

Retinal Thickness ◽

Optical Coherence ◽

Fiber Layer ◽

Nerve Fiber Layer ◽

Retinal Nerve Fiber

Background: We performed exploratory analyses of retinal thickness data from a clinical trial of the AβPP cleaving enzyme (BACE) inhibitor verubecestat in patients with Alzheimer’s disease (AD). Objective: To evaluate: 1) possible retinal thickness changes following BACE inhibition; and 2) possible association between retinal thickness and brain atrophy. Methods: Retinal thickness was measured using spectral-domain optical coherence tomography in a 78-week randomized placebo-controlled trial of verubecestat in 1,785 patients with mild-to-moderate AD. Changes from baseline in retinal pigment epithelium, macular grid retinal nerve fiber layer, central subfield retinal thickness, and macular grid volume were evaluated for verubecestat versus placebo. Correlation analyses were performed to investigate the potential association between macular grid retinal nerve fiber layer and central subfield retinal thickness with brain volumetric magnetic resonance imaging (vMRI) data at baseline, as well as correlations for changes from baseline at Week 78 in patients receiving placebo. Results: Verubecestat did not significantly alter retinal thickness during the trial compared with placebo. At baseline, mean macular grid retinal nerve fiber layer and central subfield retinal thickness were weakly but significantly correlated (Pearson’s r values≤0.23, p-values < 0.01) with vMRI of several brain regions including whole brain, hippocampus, and thalamus. At Week 78, correlations between retinal thickness and brain vMRI changes from baseline in the placebo group were small and mostly not statistically significant. Conclusion: BACE inhibition by verubecestat was not associated with adverse effects on retinal thickness in patients with mild-to-moderate AD. Correlations between retinal thickness and brain volume were observed at baseline. Trial registration: Clinicaltrials.gov NCT01739348 (registered December 3, 2012; https://clinicaltrials.gov/ct2/show/NCT01739348).

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