Article Figures & Data
Figures
- Figure 1.
Reorganization of visual processing in MD8. A, Column 1, Schematic of the visual field in the left eye showing the large extent of the blind field (scotoma), the PRL (blue star), and the non-PRL (red star), labeled accordingly. MD8 was tested binocularly and the field loss in the right eye was very similar to that shown for the left eye. A, Columns 2 and 3, Statistical parametric maps on the flattened cortex showing activation at occipital pole ROI (white outlines show the occipital pole ROI). The activation maps are displayed on the flattened cortex and show activation in response to visual objects presented at the PRL (column 2) and the non-PRL (column 3). In MD8, the non-PRL was located in the left visual field, and thus the data are shown for the right hemisphere only. In each case, activation was observed not only in parts of cortex corresponding to the retinal location of the PRL and non-PRL (arrows), but also in the occipital pole corresponding to the foveal confluence. A, Column 4, Statistical parametric map on the flattened cortex showing activation for the contrast of PRL versus non-PRL. This activation map shows the expected activation in response to visual objects presented at the PRL (yellow), and the non-PRL (blue), but not at the occipital pole. B, Bar chart showing percentage signal change (from fixation baseline) in the independently defined occipital pole ROI (white outlines on the statistical maps). Stimuli presented at the PRL (blue bar) and the non-PRL (red bar) elicited strong responses in the occipital pole ROI, while stimuli presented at the fovea (green bar) elicited no response. C, Average time course of activation in the occipital pole ROI. There is a strong increase in activation relative to the fixation baseline for stimuli presented at the PRL (blue line) and the non-PRL (red line), but no response to stimuli presented at the fovea (green line) over the course of the blocks.
- Figure 2.
Reorganization of visual processing in MD1. A, Column 1, Schematic of the visual field in the right eye showing the large extent of the blind field (scotoma), the PRL (blue star), and the non-PRL (red star), labeled accordingly. MD1's right eye only was tested because the non-PRL location, if presented binocularly, would have been in the scotoma of the left eye. A, Columns 2 and 3, Statistical parametric maps on the flattened cortex showing activation at occipital pole ROI (white outlines show the occipital pole ROI). The activation maps are displayed on the flattened cortex and show activation in response to visual objects presented at the PRL (column 2) and the non-PRL (column 3). In MD1, the non-PRL was located in the left visual field, and thus the data are shown for the right hemisphere only. In each case, activation was observed not only in parts of cortex corresponding to the retinal location of the PRL and non-PRL (arrows), but also in the occipital pole corresponding to the foveal confluence. A, Column 4, Statistical parametric map on the flattened cortex showing activation for the contrast of PRL versus non-PRL. This activation map shows the expected activation in response to visual objects presented at the PRL (yellow) and the non-PRL (blue), but not at the occipital pole. B, Bar chart showing percentage signal change (from fixation baseline) in the independently defined occipital pole ROI (white outlines on the statistical maps). Stimuli presented at the PRL (blue bar) and the non-PRL (red bar) elicited strong responses in the occipital pole ROI. C, Average time course of activation in the occipital pole ROI. There is a strong increase in activation relative to the fixation baseline for stimuli presented at the PRL (blue line) and the non-PRL (red line) over the course of the blocks.
- Figure 3.
Lack of reorganization of visual processing in matched controls, labeled accordingly. A, Columns 1 and 2, Statistical parametric maps on the flattened cortex showing no activation at the occipital pole (white outlines show the occipital pole ROIs). The activation maps are displayed on the flattened cortex and show activation in response to visual objects presented at the PRL (column 2) and the non-PRL (column 3). In each case, activation was observed only in parts of cortex corresponding to the retinal location of the PRL and non-PRL (arrows), not at the occipital pole corresponding to the foveal confluence. A, Column 3, Statistical parametric map on the flattened cortex showing activation for the contrast of PRL versus non-PRL. This activation map shows the expected activation in response to visual objects presented at the PRL (yellow) and the non-PRL (blue). B, Bar chart showing percentage signal change (from fixation baseline) in the independently defined occipital pole ROI (white outlines on the statistical maps). Stimuli presented at the PRL (blue bar) and the non-PRL (red bar) elicited no discernable responses above baseline in the occipital pole ROI. In contrast, for MD8 Control, stimuli presented at the fovea elicited a strong response (green bar). C, Average time course of activation in the occipital pole ROI. There is no activation relative to the fixation baseline for stimuli presented at the PRL (blue line) and the non-PRL (red line) over the course of the blocks, and the expected activation for stimuli presented at the fovea.
Tables
Participant Gender Age of onset (years) Time since onset (years) Visual acuity Diagnosis Fixation stability from Nidek Stimulus size (degrees: w × h) OD OS OD OS 2° (%) 4° (%) 2° (%) 4° (%) MD8 F 24 26 20/100 20/100 Stargardt's disease 91 100 72 97 4 × 4 MD1 M 37 20 20/330* 20/330 Cone–rod dystrophy 100 100 N.A. N.A. 6 × 6 -
An asterisk denotes the tested eye (no asterisk indicates that the participant was tested binocularly). OD, Right eye; OS, left eye; N.A., not available; w, width; h, height.
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Supplemental Data
Files in this Data Supplement:
- supplemental material - Supplemental Legend
- supplemental material - Supplemental Figure 1
- supplemental material - Supplemental Figure 2
- supplemental material - Supplemental Figure 3
