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This Article Full Text (P<P[PDF]) All Versions of this Article: iovs.09-4372v1 51/2/1079    most recent Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Google Scholar Articles by Jacobson, S. Articles by Cideciyan, A. PubMed PubMed Citation Articles by Jacobson, S. Articles by Cideciyan, A.

Normal Central Retinal Function and Structure Preserved in Retinitis Pigmentosa

¹Ophthalmology, Scheie Eye Institute, Philadelphia, United States ²Ophthalmology, University of Pennsylvania, Philadelphia, United States ³Ophthalomololgy, University of Pennsylvania, Philadelphia, United States ⁴Ophthalmology, Scheie Eye Institute, University of Pennsylvania, Philadelphia, United States ⁵Center for Hereditary Retinal Degenerations, University of Pennsylvania. Scheie Eye Institute., Philadelphia, United States ⁶Ophthalmology, University of Pennsylvania, Philadelphia, United States ⁷Ophthalmology, University of Pennsylvania, Philadelphia, United States ⁸Ophthalmology, University of Pennsylvania, Philadelphia, United States ⁹Ophthalmology, University of Pennsylvania, Philadelphia, United States ¹⁰Department of Ophthalmology, University of Pennsylvania, Philadelphia, United States

Samuel Jacobson, Email: jacobsos{at}mail.med.upenn.edu

Abstract

PURPOSE. To determine whether normal function and structure, as recently found in forms of Usher syndrome, also occurs in a population of patients with non-syndromic retinitis pigmentosa (RP).

METHODS. Patients with simplex, multiplex or autosomal recessive RP (n = 238; ages 9-82) were studied with static chromatic perimetry. A subset was evaluated with optical coherence tomography (OCT). Co-localized visual sensitivity and photoreceptor nuclear layer thickness were measured across the central retina to establish the relationship of function and structure. Comparisons were made to patients with Usher syndrome (n = 83, ages 10-69).

RESULTS. Cross-sectional psychophysical data identified RP patients with normal rod- and cone-mediated function in the central retina. There were two other patterns with greater dysfunction and longitudinal data confirmed progression could occur from normal rod and cone function to cone-only central islands. The retinal extent of normal laminar architecture by OCT corresponded to the extent of normal visual function in RP patients. Central retinal preservation of normal function and structure did not show a relationship with age or retained peripheral function. Usher syndrome results were like those in non-syndromic RP.

CONCLUSIONS. Regional disease variation is a well-known finding in RP. Unexpected was the observation that patients with presumed recessive RP can have regions with functionally- and structurally-normal retina. Such patients will require special consideration in future clinical trials of either focal or systemic treatment. Whether there is a common molecular mechanism shared by forms of RP with normal regions of retina warrants further study.

Key Words: retinitis pigmentosa • retinal degeneration • photoreceptor dystrophy • photoreceptor morphology • optical coherence tomography • scotopic sensitivity