Original articleChromatic Pupillometry in Patients with Retinitis Pigmentosa
Section snippets
Patients
The study was conducted according to the tenets of the Declaration of Helsinki and received approval from the University of Iowa Institutional Review Board committee. All patients gave informed consent for study participation.
Thirty-two consecutive patients with a diagnosis of retinitis pigmentosa (RP) underwent pupil testing at the time of their clinic appointment in the Retina Clinic of the University of Iowa (author ES) between 2007 and 2008. Inclusion criteria included abnormal fundus
Results
Thirty-two patients were included in this study (17 women and 15 men). Diagnoses were RP in 30 patients and Stargardt's disease in 2 patients. Two patients with RP demonstrated genetic transmission consistent with an autosomal dominant form of RP, 5 patients had an autosomal recessive form, and 4 patients had an X-linked form. A recordable pupil response was obtained in all patients except for 1 patient with X-linked RP who had no light perception, had a flat ERG, showed no pupil response to
Discussion
This study examined the pupil light reflex using a novel protocol with red and blue light stimuli in patients with rod and cone dysfunction. We had previously developed the stimulus conditions based on light wavelength, intensity, and duration intended to bias the input in the afferent pupillomotor signal to favor rods, cones, or melanopsin activation.7 After testing a group of normal subjects, we applied the protocol to a nonselected group of patients with RP and evaluated their pupil
References (15)
- et al.
Chromatic pupil responses: preferential activation of the melanopsin-mediated versus outer photoreceptor-mediated pupil light reflex
Ophthalmology
(2009) - et al.
Human and macaque pupil responses driven by melanopsin-containing retinal ganglion cells
Vision Res
(2007) - et al.
The influence of intrinsically-photosensitive retinal ganglion cells on the spectral sensitivity and response dynamics of the human pupillary light reflex
Vision Res
(2010) - et al.
Rate of visual field loss and long-term visual outcome in primary open-angle glaucoma
Am J Ophthalmol
(2001) - et al.
Acute photoreceptor degeneration down-regulates melanopsin expression in adult rat retina
Neurosci Lett
(2006) - et al.
Age-dependent effects of RPE65 gene therapy for Leber's congenital amaurosis: a phase 1 dose-escalation trial
Lancet
(2009) - et al.
Melanopsin and rod-cone photoreceptive systems account for all major accessory visual functions in mice
Nature
(2003)
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Manuscript no. 2010-194.
Financial Disclosure(s): The author(s) have made the following disclosure(s):
Dr. Kawasaki is a paid consultant by Bayer SpA, Milano Italy. None of the authors have a proprietary interest in any of the instrumentation, technology, or products mentioned in this manuscript.
Supported by a Merit Review and Rehabilitation Award from the Veterans Administration, Washington, DC (Dr. Kardon, VA Center of Excellence for the Prevention and Treatment of Vision Loss). Dr. Kardon is the recipient of a Lew Wasserman Scholar Award (Research to Prevent Blindness) and the Pomerantz Chair in Ophthalmology. Dr. Kawasaki was supported in part by a grant from the Foundation for Research in Ophthalmology and Loterie Romande Swiss. Unrestricted support was also received from Research to Prevent Blindness (New York, NY). Dr. Stone receives support as an Investigator of the Howard Hughes Medical Institute and from the Carver Family Center and the Center for Macular Degeneration.
This article contains additional online-only material. The following figures should appear online-only: Figures 1 and 3 to 6, and Table 1.