Macular sub-layer thinning and association with pulmonary function tests in Amyotrophic Lateral Sclerosis

Joseph M Simonett,Sina Farsiu,Nicholas J Volpe,Teepu Siddique,Amani A Fawzi,Nailah Siddique,Russell Huang

doi:10.1038/srep29187

Joseph M Simonett, Sina Farsiu + Show 5 more

Open Access

https://doi.org/10.1038/srep29187

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Journal: Scientific Reports	Publication Date: Jul 7, 2016
Citations: 32	License type: CC BY 4.0

Affiliation: Northwestern University, Duke University

Abstract

Amyotrophic Lateral Sclerosis (ALS) is a complex neurodegenerative disorder that may have anterior visual pathway involvement. In this study, we compare the macular structure of patients with ALS to healthy controls, and examine correlations between macular sub-layer thickness measurements and pulmonary function tests and disease duration. ALS patients underwent optical coherence tomography (OCT) imaging to obtain macular cube scans of the right eye. Macular cube OCT data from age-matched healthy subjects were provided by the OCT reading center. Semi-automated retinal segmentation software was used to quantify macular sub-layers. Pulmonary function tests and time since symptom onset were collected retrospectively from the electronic medical records of ALS patients. Macular retinal nerve fiber layer was significantly thinner in ALS patients compared to healthy controls (P < 0.05). Total macular and other sub-layer thicknesses were not reduced in the ALS cohort. Macular retinal nerve fiber layer thickness positively correlated with forced vital capacity % predicted and forced expiratory volume in 1 second % predicted (P < 0.05). In conclusion, analysis of OCT measurements supports the involvement of the anterior visual pathway in ALS. Subtle structural thinning in the macular retinal nerve fiber layer correlates with pulmonary function tests.

Highlights

Amyotrophic Lateral Sclerosis (ALS) is a complex neurodegenerative disorder that may have anterior visual pathway involvement
We present structural optical coherence tomography (OCT) data that is supportive of subtle anterior visual pathway involvement in ALS
We propose that these structural alterations highlight underlying retinal neuronal degeneration, in ganglion cell axons, that may correlate with the visual function deficits observed in a previous ALS cohort[14]

Summary

Introduction

Amyotrophic Lateral Sclerosis (ALS) is a complex neurodegenerative disorder that may have anterior visual pathway involvement. These findings differed from an earlier study that found no statistical differences in average peripapillary RNFL or macular sub-layer thicknesses between sporadic ALS patients and healthy controls[31].

Results

Conclusion