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Ann Clin Transl Neurol. 2014 Apr;1(4):290-7. doi: 10.1002/acn3.46. Epub 2014 Mar 11.

Subtle retinal pathology in amyotrophic lateral sclerosis.

Author information

1
Department of Neurology, Medical Faculty, Heinrich-Heine University Düsseldorf, Germany.
2
Department of Neurology, Medical Faculty, Heinrich-Heine University Düsseldorf, Germany ; Institute of Clinical Neuroscience and Medical Psychology, Medical Faculty, Heinrich-Heine University Düsseldorf, Germany.
3
Department of Ophthalmology, Medical Faculty, Heinrich-Heine University Düsseldorf, Germany.
4
Department of Neurology, Medical Faculty, Heinrich-Heine University Düsseldorf, Germany ; Department of Neurology, Medical Faculty, Johannes Gutenberg University Mainz, Germany.

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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