Correlations Between Dark-Adapted Rod Threshold Elevations and ERG Response Deficits in Duchenne Muscular Dystrophy
| Autor: | Marcelo Fernandes da Costa, Francisco Max Damico, Jan Kremers, Kallene Summer Moreira Vidal, Mirella Telles Salgueiro Barboni, Dora Fix Ventura, Leonardo Aparecido Silva, Balázs Vince Nagy, Sarah Leonardo Dias |
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| Rok vydání: | 2021 |
| Předmět: |
Male
retina medicine.medical_specialty Adolescent genetic structures media_common.quotation_subject Duchenne muscular dystrophy Dark Adaptation Adaptation (eye) Young Adult 03 medical and health sciences 0302 clinical medicine Ophthalmology dystrophin protein Electroretinography Humans Contrast (vision) Medicine human vision Child media_common medicine.diagnostic_test business.industry electroretinogram (ERG) cone medicine.disease Muscular Dystrophy Duchenne Dark-adapted Sensory Thresholds Duchenne muscular dystrophy (DMD) ERG response dark-adaptation Retinal Cone Photoreceptor Cells Visual Perception 030221 ophthalmology & optometry Clinical electrophysiology Female sense organs Visual Neuroscience rod DISTROFIA MUSCULAR business Erg Photic Stimulation 030217 neurology & neurosurgery |
| Zdroj: | Repositório Institucional da USP (Biblioteca Digital da Produção Intelectual) Universidade de São Paulo (USP) instacron:USP Investigative Ophthalmology & Visual Science |
| ISSN: | 1552-5783 |
| DOI: | 10.1167/iovs.62.4.29 |
| Popis: | Purpose The purpose of this study was to characterize changes in the full-field flash electroretinogram (ERG) in association with psychophysical dark-adapted visual thresholds in patients with genetically characterized Duchenne muscular dystrophy (DMD) either lacking Dp427 (Up 30) or at least Dp260 in addition to Dp427 (Down 30). Methods Twenty-one patients with DMD and 27 age-similar controls participated in this study. Dark-adapted (0.01, 3.0, and 10 cd.s/m² flashes) and light-adapted (3.0 cd.s/m² flash) ERGs were recorded following International Society for Clinical Electrophysiology of Vision (ISCEV) standard protocols. Visual detection thresholds to 625-nm (cone function) and 527-nm (rod function) light-emitting diode (LED) flashes (2 degree diameter) were measured during a dark adaptation period after a 1-minute exposure to a bleaching light (3000 cd/m²). Initially, 8 minutes of interleaved 625-nm and 527-nm thresholds were measured. After an additional 5 minutes of dark-adaptation, a second set of threshold measurements to 527-nm stimuli was performed during the subsequent 6 minutes. Results Dark-adapted b-wave amplitude was significantly reduced to all strengths of flash and a-wave in response to the strong flash stimulus was delayed (15.6 vs. 14.7 ms, P < 0.05) in patients with Down 30 compared with controls. Dark-adapted cone thresholds did not differ among the groups (−2.0, −1.8, and −1.7 log cd/m² for Down 30, Up 30, and controls, respectively, P = 0.21). In contrast, dark-adapted rod thresholds were elevated (F(2,36) = 8.537, P = 0.001) in patients with Down 30 (mean = −3.2 ± 1.1 log cd/m²) relative to controls (mean = −4.2 ± 0.3 log cd/m²). Dark-adapted b-wave amplitudes were correlated with dark-adapted rod sensitivity in patients with DMD (Spearman Rho = 0.943, P = 0.005). The changes were much smaller or absent in patients with intact Dp260. Conclusions Dp260 is particularly required for normal rod-system function in dark adaptation. |
| Databáze: | OpenAIRE |
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