Purpose: The purpose of this study is to describe the relationships between full-field stimulus threshold (FST), electroretinography (ERG), and visual field (VF) outcomes in retinitis pigmentosa (RP).
Methods: Data from 47 patients with RP (n = 94 eyes) were evaluated. Patients were submitted to comprehensive ophthalmological examination including measurement of best-corrected visual acuity (BCVA), 30-2 threshold static VF, and microperimetry. Full-field ERG (ISCEV standard) was recorded, and achromatic FST was measured using a Diagnosys Espion system with the ColorDome™ LED full-field stimulator (Diagnosys LLC, Lowell, MA, USA).
Results: BCVA mean ± SD was 0.31 ± 0.03 logMAR, and FST mean ± SD was -18.45 ± 9.53 dB. No significant correlation was found between BCVA and FST. In contrast, statistically significant correlations were found between FST and static 30-2 VF mean deviation (r = -0.389; P < 0.01), microperimetry mean threshold (r = -0.607; P < 0.01). Dark and light-adapted ERGs were detectable in 28 and 48 eyes, respectively. Nevertheless, considering only the eyes with recordable ERG responses, moderate correlations were found between combined dark-adapted a-wave amplitude (r = -0.560; P < 0.01), b-wave amplitude (r = -0.643; P < 0.001), 30-Hz flicker response (r = -0.501; P < 0.01), and FST, and high correlation with FST for cone b-wave amplitude (r = -0.715; P < 0.01).
Conclusions: FST could be successfully determined in RP patients with a wide range of vision loss. FST results showed stronger correlations with full-field ERG amplitude than with sensitivity measured with visual field tests. FST is as an alternative to VF or ERG for assessment of retinal function in patients unable to do visual fields or with non-detectable ERGs.
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http://dx.doi.org/10.1007/s10633-013-9393-y | DOI Listing |
Purpose: To describe progression of best-corrected visual acuity (BCVA), full-field stimulus thresholds (FST), and electroretinography (ERG) over 4 years in the -related Retinal Degeneration study and to assess their suitability as clinical trial endpoints.
Design: Prospective natural history study.
Participants: Participants (n = 105) with biallelic disease-causing sequence variants in USH2A and BCVA letter scores of ≥54 were included.
Can J Ophthalmol
January 2025
Department of Ophthalmology and Vision Sciences, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada. Electronic address:
Objective: Biallelic RPE65 pathogenic variants may cause Leber congenital amaurosis (LCA). Voretigene neparvovec-rzyl (VN, Luxturna) is the only approved subretinal gene therapy that demonstrated benefit and safety. The eligibility criteria are vague and variable between centres.
View Article and Find Full Text PDFJ Neurophysiol
January 2025
Department of Psychological and Brain Sciences, Boston University, Boston, MA, 02215.
Visuocortical responses are regulated by gain control mechanisms, giving rise to fundamental neural and perceptual phenomena such as surround suppression. Suppression strength, determined by the composition and relative properties of stimuli, controls the strength of neural responses in early visual cortex, and in turn, the subjective salience of the visual stimulus. Notably, suppression strength is modulated by feature similarity; for instance, responses to a center-surround stimulus in which the components are collinear to each other are weaker than when they are orthogonal.
View Article and Find Full Text PDFInvest Ophthalmol Vis Sci
December 2024
Scheie Eye Institute, Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States.
Purpose: The purpose of this study was to determine the natural history of the photoreceptor disease in a large group of pediatric patients with RHD12-associated Leber congenital amaurosis (RDH12-LCA), to estimate the changes expected over the duration of a clinical trial, and to define the relationship between the photoreceptor loss and visual dysfunction.
Methods: Forty-six patients representing 36 families were included. The great majority of patients were under the age of 18 years.
Acta Ophthalmol
December 2024
Department of Ophthalmology, Leiden University Medical Center, Leiden, The Netherlands.
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