Pseudo-fovea formation after gene therapy for RPE65-LCA.

ABSTRACT:

Purpose

The purpose of this study was to evaluate fixation location and oculomotor characteristics of 15 patients with Leber congenital amaurosis (LCA) caused by RPE65 mutations (RPE65-LCA) who underwent retinal gene therapy.

Methods

Eye movements were quantified under infrared imaging of the retina while the subject fixated on a stationary target. In a subset of patients, letter recognition under retinal imaging was performed. Cortical responses to visual stimulation were measured using functional magnetic resonance imaging (fMRI) in two patients before and after therapy.

Results

All patients were able to fixate on a 1° diameter visible target in the dark. The preferred retinal locus of fixation was either at the anatomical fovea or at an extrafoveal locus. There wer

SUBMITTER: Cideciyan AV 

PROVIDER: S-EPMC4303042 | biostudies-literature | 2014 Dec

REPOSITORIES: biostudies-literature