case_images: PMC10601797_01
case_images: A timeline representing the changes in the visual acuity of both patients over the course of following up their progression, sibling 1 (a), sibling 2 (b). Notice the increase in visual acuity after the genetic treatment.
case_images: cop-2023-0014-0001-530086_F01.jpg
case_images: PMC10601797_01_cop-2023-0014-0001-530086_F01.jpg
case_images: F1
case_images: Visual acuity in the left eye started to deteriorate as well (Fig. 1a), and, by 2015, only the ability to detect hand movements remained, and visual field was not detectable. Her visual acuity gradually worsened to 0.01 (right eye) and to 0.02 (left eye) by the age of 23 years (Fig. 1b).
case_images: Flash-evoked visual evoked potentials from the left and right eyes of both siblings before treatment over the follow-up period (a, b) and from the left eyes of both siblings before and 4 months after the Luxturna treatment (c, d).
case_images: cop-2023-0014-0001-530086_F02.jpg
case_images: PMC10601797_03_cop-2023-0014-0001-530086_F02.jpg
case_images: F2
case_images: The improvement was detectable also in the FVEP: the N1-P1 amplitude increased from 0.39 muV to 4.02 muV (Fig. 2d).
case_images: Multifocal ERG of the sibling 2 at her age of 10 years. Top: trace arrays (plots), middle: three-dimensional representation, bottom: ring analysis of the responses.
case_images: cop-2023-0014-0001-530086_F03.jpg
case_images: PMC10601797_01_cop-2023-0014-0001-530086_F03.jpg
case_images: F3
case_images: The mfERG revealed seriously affected central cone function, reflecting a "patchy" pattern of degeneration, which means that there were some rather good kernels (responses), and also completely extinguished ones in the central 30  of the retina (Fig. 3).
case_images: PMC10601797_02
case_images: Flash-evoked visual evoked potentials from the left and right eyes of both siblings before treatment over the follow-up period (a, b) and from the left eyes of both siblings before and 4 months after the Luxturna treatment (c, d).
case_images: cop-2023-0014-0001-530086_F02.jpg
case_images: PMC10601797_03_cop-2023-0014-0001-530086_F02.jpg
case_images: F2
case_images: The improvement was detectable also in the FVEP: the N1-P1 amplitude increased from 0.39 muV to 4.02 muV (Fig. 2d).
case_images: PMC10601797_03
case_images: Flash-evoked visual evoked potentials from the left and right eyes of both siblings before treatment over the follow-up period (a, b) and from the left eyes of both siblings before and 4 months after the Luxturna treatment (c, d).
case_images: cop-2023-0014-0001-530086_F02.jpg
case_images: PMC10601797_03_cop-2023-0014-0001-530086_F02.jpg
case_images: F2
case_images: The improvement was detectable also in the FVEP: the N1-P1 amplitude increased from 0.39 muV to 4.02 muV (Fig. 2d).