Subtle retinal pathology in amyotrophic lateral sclerosis

M. Ringelstein,P. Albrecht,M. Südmeyer,Jens Harmel,Ann-Kristin Müller,Nazmiye Keser,D. Finis,S. Ferrea,R. Guthoff,A. Schnitzler,H. Hartung,A. Methner,O. Aktas

Published 2014 in Annals of Clinical and Translational Neurology

ABSTRACT

Amyotrophic lateral sclerosis (ALS) is characterized by neuro‐ophthalmological abnormalities beyond disturbed oculomotor control such as decreased visual acuity and disturbed visual evoked potentials. Here we report retinal alterations in a cohort of 24 patients with clinically definite (n = 20) or probable (n = 4) ALS as compared to matched controls. High‐resolution spectral domain optical coherence tomography with retinal segmentation revealed a subtle reduction in the macular thickness and the retinal nerve fiber layer (RNFL) as well as a marked thinning of the inner nuclear layer (INL). Our data indicate an unprecedented retinal damage pattern and suggest neurodegeneration beyond the motor system in this disease.

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