Visual Cortex Activation in Patients With Stargardt Disease

Melillo, Paolo; Prinster, Anna; Iorio, Valentina Di; Olivo, Gaia; D'Alterio, Francesco Maria; Cocozza, Sirio; Orrico, Ada; Quarantelli, Mario; Testa, Francesco; Brunetti, Arturo; Simonelli, Francesca

doi:10.1167/iovs.17-22900

Cited by 5 publications

(6 citation statements)

References 49 publications

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“…In this study, mfERG was a valuable tool for evaluating the residual function of the retina, and could manifest a correlation with the response of the primary visual cortex in end‐stage glaucoma. This finding was similar to a previous study showing that higher ERG responses were associated with a larger functional cerebral response in subjects with Stargardt disease 43 …”

Section: Discussionsupporting

confidence: 93%

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Activation of lesion projection zone in primary visual cortex is dependent on bilateral central vision loss in patients with end‐stage glaucoma

Zhu

Liu

et al. 2022

Ophthalmic Physiologic Optic

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Purpose: To investigate activation of the lesion projection zone (LPZ) in the primary visual cortex during end-stage glaucoma using functional magnetic resonance imaging (fMRI), as well as the relationship between fMRI responses and clinical data. Methods:Twelve subjects with bilateral end-stage glaucoma (group A), 12 with unilateral end-stage glaucoma (group B) and 12 healthy controls (group C) were enrolled. fMRI was performed under two testing stimuli conditions: passive viewing of a full-field flickering checkerboard and active viewing of a one-back task with scene images. In fMRI analysis, the primary visual cortex was divided into six regions of interest (ROIs). The beta values of the six ROIs were compared across the three groups using one-way analysis of variance under two viewing conditions.Associations between the fMRI beta value and clinical data including multifocal electroretinogram (mfERG), microperimeter-1 and optical coherence tomography were analysed by Spearman correlation. Results:The beta values for ROIs 1-3 representing the LPZ were significantly different between the three groups under active viewing conditions, whereas no significant changes were detected under passive viewing. In group A, there were significant differences between all six ROIs for the two viewing conditions, while no significant differences were found in groups B and C. In group A, the P-wave amplitudes of the mfERG was significantly correlated with the beta values of ROIs 1 and 2 under active viewing. In addition, the P-wave latencies of the mfERG were significantly correlated with the beta values for ROIs 2-5. No associations were found between fMRI beta values and clinical data in groups B and C.Conclusions: Activation of the LPZ in the primary visual cortex was observed in patients with bilateral end-stage glaucoma under active viewing conditions. These changes were correlated with residual retinal function.

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Section: Discussionsupporting

confidence: 93%

“…This finding was similar to a previous study showing that higher ERG responses were associated with a larger functional cerebral response in subjects with Stargardt disease. 43 There were some limitations to this study. Firstly, we did not record the eye movements of the participants.…”

Section: Groupmentioning

confidence: 93%

Activation of lesion projection zone in primary visual cortex is dependent on bilateral central vision loss in patients with end‐stage glaucoma

Zhu

Liu

et al. 2022

Ophthalmic Physiologic Optic

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“…35 This theory is, however, much debated; in fact, other authors have shown that, in patients with late-onset macular degeneration, the persistent activation of foveal corresponding area was due to the activation of close areas (a top-down effect), especially in adults in whom less neuroplasticity phenomena could be expected. 36 To this regard, in our previous study 37 we showed stronger PVC activation in patients with STGD1 with a more preserved retinal function and macular structure, suggesting that the evaluation of neuronal reorganization could be performed only by considering retinal parameters, particularly ERG responses.…”

Section: Discussionmentioning

confidence: 92%

Biofeedback Rehabilitation and Visual Cortex Response in Stargardt's Disease: A Randomized Controlled Trial

Melillo

Prinster

Iorio

et al. 2020

Trans. Vis. Sci. Tech.

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Purpose: to evaluate the effect of biofeedback (BF) rehabilitation on the visual function and on the activity of primary visual cortex (PVC) in patients with Stargardt's disease owing to mutations in the ABCA4 gene (STGD1). Methods: This was a single-center, controlled, randomized study. Twenty-four patients with STGD1 were randomized into two groups: a treatment group (TG) undergoing BF rehabilitation and a control group (CG). Treatment with BF consisted of a 10-minute session per eye performed weekly for 12 weeks. The subjects underwent a baseline and 3-month follow-up visits, including best-corrected visual acuity (BCVA), reading test, microperimetry, and functional magnetic resonance imaging (fMRI). The fMRI studies were acquired sequentially using a passive viewing condition and an active reading task. The primary outcomes were the change in the fMRI activation of primary visual cortex and the change in reading ability. Results: After treatment, the patients in the TG were able to read smaller characters (P = 0.002) with a greater reading speed (P = 0.014) compared with patients in the CG. The fMRI studies showed a significant effect (P < 0.001) of BF on primary visual cortex activation in the TG compared with the CG. Finally, we observed significant (P < 0.05) improvements of best-corrected visual acuity, macular sensitivity, and fixation stability parameters in the TG compared with the CG. Conclusions: Our study showed that visual rehabilitation using BF improved the usage of residual visual function in patients with STGD1.

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“…In the current experiment, we demonstrated that fMRI can detect signals of spared visual processing with higher sensitivity than other objective and quantitative measures used for standard ophthalmological testing. A recent study on patients with Stargardt disease showed that electrophysiological responses were strongly correlated with activation of the primary visual cortex and concluded that evaluation of neural reorganization can be performed considering ERG responses only 26. However, reliable ERG responses might not be detectable in patients affected by retinal dystrophies at the very late stage,9,11 limiting the diagnostic use of this technique.…”

Section: Discussionmentioning

confidence: 99%

Residual Visual Responses in Patients With Retinitis Pigmentosa Revealed by Functional Magnetic Resonance Imaging

Castaldi

Cicchini

Falsini

et al. 2019

Trans. Vis. Sci. Tech.

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PurposeWe evaluated the potential of magnetic resonance imaging in identifying signs of cortical visual processing with greater sensitivity than standard ophthalmological measures in patients with retinitis pigmentosa (RP) at advanced stages.MethodsEight patients affected with RP with only bare light perception and weak or absent visual evoked potential (VEP) or electroretinogram (ERG) responses to flashes of light were tested. Visual impairment was evaluated by means of psychophysical testing, where patients were asked to discriminate the drifting direction of a contrast modulated grating. Patients underwent magnetic resonance imaging scanning, and the behavioral performance was correlated with both blood oxygenation level-dependent (BOLD) signal elicited by flashes of lights and cortical thickness measured in primary visual area.ResultsContrast sensitivity to drifting gratings of very low spatial and temporal frequency was greatly impaired, yet measurable in all patients. Weak luminance flashes elicited significant BOLD responses in the striate and extrastriate cortex, despite that the stimuli were not perceived during scanning. Importantly, patients with less severe impairment of contrast sensitivity showed stronger V1 BOLD responses. Striate cortical thickness did not correlate with visual sensitivity.ConclusionsBOLD responses provide a sensitive and reliable index of visual sparing more than VEPs or ERGs, which are often absent in RP patients. The minimal residual vision can be assessed by optimal visual stimulation in two alternative forced choice discrimination tasks and by BOLD responses. Imaging techniques provide useful information to monitor progressive vision loss.Translational RelevanceFunctional magnetic resonance imaging might be a practical tool for assessing visual sparing, as it is more feasible and sensitive than psychophysical or ophthalmological testing.

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Visual Cortex Activation in Patients With Stargardt Disease

Cited by 5 publications

References 49 publications

Activation of lesion projection zone in primary visual cortex is dependent on bilateral central vision loss in patients with end‐stage glaucoma

Activation of lesion projection zone in primary visual cortex is dependent on bilateral central vision loss in patients with end‐stage glaucoma

Biofeedback Rehabilitation and Visual Cortex Response in Stargardt's Disease: A Randomized Controlled Trial

Residual Visual Responses in Patients With Retinitis Pigmentosa Revealed by Functional Magnetic Resonance Imaging

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