Journalartikel

Chromatic Pupillometry Dissects Function of the Three Different Light-Sensitive Retinal Cell Populations in RPE65 Deficiency


AutorenlisteLorenz, Birgit; Strohmayr, Elisabeth; Zahn, Steffen; Friedburg, Christoph; Kramer, Martin; Preising, Markus; Stieger, Knut

Jahr der Veröffentlichung2012

Seiten5641-5652

ZeitschriftInvestigative ophthalmology & visual science

Bandnummer53

Heftnummer9

ISSN0146-0404

eISSN1552-5783

DOI Linkhttps://doi.org/10.1167/iovs.12-9974

VerlagAssociation for Research in Vision and Ophthalmology


Abstract

PURPOSE. The aim of the study was to objectively characterize the function of rods, cones, and intrinsic photosensitive retinal ganglion cells (ipRGCs) in patients with RPE65 mutations by using two published protocols for chromatic pupillometry, and to correlate the data with the clinical phenotype.

METHODS. The study group comprised 11 patients with RPE65 mutations, and for control purposes, 32 healthy probands and 2 achromats. A custom-made binocular chromatic pupillometer (Bino I) connected to a ColorDome Ganzfeld stimulator was used to assess changes in pupil diameter in response to red (640 nm) and blue (462 nm) light stimuli. Light intensities, stimulus duration, and background varied depending on the protocol used. Results were compared to the clinical phenotype, that is, visual field (Goldmann perimetry), best corrected visual acuity, and full-field stimulus testing (FST).

RESULTS. No significant differences in any of the pupil response parameters were observed in intraday or intervisit variability tests. Pupil responses to rod-weighted stimulation were significantly diminished in all RPE65 patients. Pupil responses to cone-weighted stimuli differed among RPE65 patients and did not always correlate with residual visual field and cone sensitivity loss in FST. Pupil responses to ipRGC-weighted answers were slightly but significantly diminished, and the postillumination pupil response was significantly increased.

CONCLUSIONS. Chromatic pupillometry represents a highly sensitive and objective test to quantify the function of rods, cones, and ipRGCs in patients with RPE65 mutations. (Invest Ophthalmol Vis Sci. 2012;53:5641-5652) DOI:10.1167/iovs.12-9974




Zitierstile

Harvard-ZitierstilLorenz, B., Strohmayr, E., Zahn, S., Friedburg, C., Kramer, M., Preising, M., et al. (2012) Chromatic Pupillometry Dissects Function of the Three Different Light-Sensitive Retinal Cell Populations in RPE65 Deficiency, Investigative ophthalmology & visual science, 53(9), pp. 5641-5652. https://doi.org/10.1167/iovs.12-9974

APA-ZitierstilLorenz, B., Strohmayr, E., Zahn, S., Friedburg, C., Kramer, M., Preising, M., & Stieger, K. (2012). Chromatic Pupillometry Dissects Function of the Three Different Light-Sensitive Retinal Cell Populations in RPE65 Deficiency. Investigative ophthalmology & visual science. 53(9), 5641-5652. https://doi.org/10.1167/iovs.12-9974



Schlagwörter


GENE-THERAPYLEBER CONGENITAL AMAUROSISPHOTOPIGMENTTHERAPY RESTORES VISION


Nachhaltigkeitsbezüge


Zuletzt aktualisiert 2025-21-05 um 18:39