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Invest Ophthalmol Vis SciOctober 20214 citations

Luminance Thresholds and Their Correlation With Retinal Structure in X-Linked Retinoschisis.

McAnany J Jason, Park Jason C, Fishman Gerald A, Hyde Robert A


AI Summary

This study found X-linked retinoschisis causes complex, location- and adaptation-dependent vision loss, with macular function resembling normal peripheral vision. This helps understand disease progression and guide treatment.

Abstract

Purpose

To provide a comprehensive analysis of light- and dark-adapted luminance thresholds and their associations with retinal structure in X-linked retinoschisis (XLRS).

Methods

Nine subjects with XLRS and 10 visually-normal individuals participated. Threshold was measured at 15 locations along the horizontal meridian of the visual field at several adaptation levels (5 × 10-5 to 50 cd/m2) after dark-adaptation. The relationship between threshold and adaptation level across the field was described using a standard "threshold-versus-illuminance" model. Optical coherence tomography images were obtained and segmented to quantify outer nuclear layer (ONL+) and outer segment (OS+) thickness. A linear structure-function model was used to describe the relationship between threshold and the product of ONL+ and OS+ thickness.

Results

For peripheral field measurements, thresholds were generally normal for most subjects with XLRS. All subjects had perifoveal and parafoveal threshold elevations under dark-adapted and high illuminance conditions, with thresholds at moderate illuminances being closer to normal. For foveal measurements, seven of nine subjects with XLRS had normal dark-adapted thresholds, and all had abnormally elevated high illuminance thresholds. Threshold-versus-illuminance curves in the fovea, parafovea, and perifovea were abnormally steep for subjects with XLRS, appearing similar to the normal peripheral field shape. Under both dark- and light-adapted conditions, threshold was predicted by ONL+ × OS+ thickness at nearly all field locations.

Conclusions

Threshold elevation in XLRS is complex, depending on both the adaptation level and the visual field location. The pattern of threshold-versus-illuminance suggests that macular function in XLRS is similar to the periphery of controls.


MeSH Terms

AdolescentAdultDark AdaptationElectroretinographyFemaleFovea CentralisHumansMaleMiddle AgedPhotic StimulationRetinoschisisTomography, Optical CoherenceVisual AcuityVisual FieldsYoung Adult

Key Concepts5

For peripheral visual field measurements, luminance thresholds were generally normal for most subjects with X-linked retinoschisis (XLRS) in a study of 9 subjects with XLRS and 10 visually-normal individuals.

DiagnosisCross-sectionalCross-sectional studyn=9 subjects with XLRS, 10 visually-nor…Ch6

All 9 subjects with X-linked retinoschisis (XLRS) had perifoveal and parafoveal luminance threshold elevations under dark-adapted and high illuminance conditions, with thresholds at moderate illuminances being closer to normal, in a study of 9 subjects with XLRS and 10 visually-normal individuals.

DiagnosisCross-sectionalCross-sectional studyn=9 subjects with XLRS, 10 visually-nor…Ch6

Seven of nine subjects with X-linked retinoschisis (XLRS) had normal dark-adapted foveal luminance thresholds, and all 9 subjects had abnormally elevated high illuminance foveal thresholds, in a study of 9 subjects with XLRS and 10 visually-normal individuals.

DiagnosisCross-sectionalCross-sectional studyn=9 subjects with XLRS, 10 visually-nor…Ch6

Luminance threshold-versus-illuminance curves in the fovea, parafovea, and perifovea were abnormally steep for subjects with X-linked retinoschisis (XLRS), appearing similar to the normal peripheral field shape, in a study of 9 subjects with XLRS and 10 visually-normal individuals.

MechanismCross-sectionalCross-sectional studyn=9 subjects with XLRS, 10 visually-nor…Ch6

Under both dark- and light-adapted conditions, luminance threshold was predicted by the product of outer nuclear layer (ONL+) and outer segment (OS+) thickness at nearly all visual field locations in subjects with X-linked retinoschisis (XLRS), in a study of 9 subjects with XLRS and 10 visually-normal individuals.

MechanismCross-sectionalCross-sectional studyn=9 subjects with XLRS, 10 visually-nor…Ch5Ch6

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