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Chromatic pupillometry dissects function of the three different light sensitive retinal cell populations in RPE65 deficiency.
MedLine Citation:
PMID:  22807296     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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 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, AMTech) connected to a Colordome Ganzfeld stimulator (Diagnosys LLC) 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, i.e. visual field (Goldmann perimetry), best corrected visual acuity (BCVA), and full field stimulus testing (FST). Results: No significant differences in any of the pupil response parameters were observed in intra day or inter visit 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 post illumination pupil response (PIPR) was significantly increased.Conclusion: Chromatic pupillometry represents a highly sensitive and objective test to quantify the function of rods, cones, and ipRGCs in patients with RPE65 mutations.
Authors:
Birgit Lorenz; Elisabeth Strohmayr; Steffen Zahn; Christoph Friedburg; Martin Kramer; Markus Preising; Knut Stieger
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-7-17
Journal Detail:
Title:  Investigative ophthalmology & visual science     Volume:  -     ISSN:  1552-5783     ISO Abbreviation:  -     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-7-18     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7703701     Medline TA:  Invest Ophthalmol Vis Sci     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Ophthalmology, Faculty of Medicine, Justus-Liebig-University, Friedrichstr. 18, Giessen, 35392, Germany.
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