Selective and Inverse U-Shaped Curve Alteration of the Retinal Nerve in Amyotrophic Lateral Sclerosis: A Potential Mirror of the Disease

• DOI: 10.3389/fnagi.2021.783431
• Publication Date: 2022-01-06T04:51:45Z
• Authors: Yixuan Zhang; Xiangyi Liu; Jiayu Fu; Yuanjin Zhang; Xue Yang; Shuo Zhang; Dongsheng Fan
• Keywords: U-shaped curve alteration; amyotrophic lateral sclerosis; 03 medical and health sciences; disease progression rate; 0302 clinical medicine; Aging Neuroscience; retinal nerve fiber layer; Neurosciences. Biological psychiatry. Neuropsychiatry; retinal ganglion cell; RC321-571

Introduction: Alterations in the visual pathway involving retina have been reported amyotrophic lateral sclerosis (ALS) but they lack consistency and subgroup analysis. We aimed to assess retinal nerve fiber layer (RNFL) ganglion cells (RGCs) alterations different stages of ALS patients their association with progression parameters. Methods: The study population consisted 70 clinically diagnosed 55 age, sex matched controls. All them underwent ophthalmic assessments optical coherence tomography imaging. Four quadrants peripapillary RNFL cell/inner plexiform complex (GCIP) were observed automatically measured. Early-stage distal motor neuron axon dysfunction was detected by compound muscle action potential (CMAP) limbs within 12 months. disease parameters included ALSFRS-R score rate (ΔFS). Results: Generally compared controls, nasal (p = 0.016) quadrant thicker patients. When controlling for age ΔFS, RNFL(r 0.37, p 0.034) GCIP(r 0.40, 0.021) significantly thickened as progressed months, while declined time after one year (r -0.41, 0.037). subclassified into (T-RNFL, >95th percentile normal), impaired (I-RNFL, <5th normal) normal RNFL. There significant differences GCIP among three groups < 0.001). In T-RNFL group (n 18), negatively correlated abductor pollicis brevis-CMAP amplitude months -0.56, 0.01). Patients this faster than others 0.039). 22), 13 beyond whose ΔFS remarkably lower 0.007). I-RNFL 30), early stage (<12 months) had higher 0.006). One patient SOD1 pathogenic variant (p.A5V). Conclusion: not consistent diverse phenotypes rates. speaking, during first then gradually declined, which is related preceding thickness change RGCs. a thinning may rate. inverse U-shaped curve transformation might be accordance early-stage axonopathy.