Functional changes at the preferred retinal locus in subjects with bilateral central vision loss.

PurposeTo investigate the effect of disease progression on the monocular preferred retinal locus (PRL) of the better eye (BE) and worse eye (WE) of patients with central vision loss.MethodsFifty-one patients with bilateral macular diseases were included. The monocular PRL was recorded for each eye (N = 102 eyes) with the MP-1 microperimeter in two visits that were 458 ± 249 days apart. For each eye and visit, the PRL distance from the former fovea, polar angle, and scotoma size were measured. The change in PRL location from visit 1 to visit 2 was evaluated with the differential map analysis.ResultsScotoma size increased significantly in both eyes. The PRL distance from the former fovea increased significantly from visit 1 to visit 2 in the BE, but not in the WE. The polar angle was relatively stable in both visits for the BE. The change in PRL location in the BE was predicted only by the PRL distance from the former fovea in visits 1 and 2, but not by polar angle or scotoma size. For the WE, the change in PRL location depended on the change in PRL location in the BE, rather than on measurements made on that eye.ConclusionsDisease progression affects monocular PRL location differently in the 2 eyes. The results suggest a recalibration of the oculomotor system with its reference at the PRL from the BE.Translational RelevanceThese findings are important for deciding the course of treatment and/or for developing rehabilitation techniques focusing on PRL relocation.

Objective: Preferred retinal locus (PRL) training has been applied to low-vision rehabilitation for patients with central vision loss (CVL). This study aimed to explore the characteristics of a natural PRL in eyes with different macular lesions. The data may be useful in customizing training programs. Methods: A total of 72 eyes with CVL were included and assigned into two groups. In group A, 29 eyes diagnosed with macular holes featured relatively sharp borders and small areas of lesions. In group B, 44 eyes showed lesions characterized by irregular borders and large areas. The PRL location relative to a scotoma in the retina, fixation stability, and the average threshold surrounding the PRL were determined and compared between the two groups. Results: In group A, the PRL was located above in 48.28%, below in 27.59%, left in 62.07%, right in 31.03%, and inside in 3.45% of the eyes. In group B, the PRL was located above in 39.53%, below in 4.65%, left in 44.19%, right in 6.98%, and inside in 27.91% of eyes. The amount of retinal displacement occurring within 1° from an initial reference point (P< .05) and the 95% bivariate contour ellipse area (P< .05) in group A were respectively higher and lower than those in group B. However, the average thresholds around the PRLs in the two groups showed no significant difference (P > .05). Conclusions: A PRL to the left of or above a scotoma tends to develop to avoid the right and inferior field defect, regardless of the scale and boundary of lesions. Although light sensitivity around a PRL shows no relation to lesion features, fixation stability is worse in irregular and large lesions.

PurposeFixation stability (FS) of the preferred retinal locus (PRL) may be improved by biofeedback fixation training (BFT) with microperimetry. Such training can be done on the patient’s PRL or in different retinal loci with better functional characteristics. We studied both options and compared the outcomes.MethodsSixty-seven consecutive patients with bilateral central vision loss, poor FS and visual acuity (VA) lower than 0.3 LogMAR were recruited for BFT with microperimeter. Patients were assigned into 2 groups. In group A, BFT was performed on the patient’s spontaneous PRL. In group B, PRL was located between 2 adjacent loci with the highest light sensitivity and the lowest distance from the fovea. Two sets of 12 weekly BFT sessions were performed. Primary outcomes were: FS, VA and reading speed.ResultsOutcomes were statistically significantly better in group B. Mean percentage of FS at therapy end improved from 32 to 35% for group A and from 40 to 55% in group B. Mean VA improved from 1 to 0.86 in group A and from 1 to 0.84 in group B. Reading speed (wpm) improved from 56 to 58 in group A and from 63 to 89 in group B.ConclusionsThis study describes a reliable methodology of improving eccentric fixation stability using BFT in microperimetry, when the fixation training locus is individualized as the retinal area with best functional characteristics. Further studies are needed to validate its value in a larger scale of patients, at different stages of the disease, and its persistence over time.

Purpose: Improvement in visual acuity is often considered the best indicator of the effectiveness of a treatment in age-related maculopathy. However, during the course of the disease, the location of the patients’ preferred retinal locus of fixation may change. This can lead to an unexpected functional improvement, unrelated to treatment.Methods: From a running database of 1,369 retina patients, we identified 116 patients over 60 years of age when age-related maculopathy was diagnosed based on the following inclusion criteria: one study eye with an initial acuity of the logarithm of the minimum angle of resolution (logMAR) 0.7 or worse, a fellow eye with central fixation and a follow-up period of 3 years or more with precise documentation of the preferred retinal locus of fixation and scotoma distribution for both eyes using scanning laser ophthalmoscopy-based microperimetry.Results: We expected an improvement in the visual acuity in one eye (study eye) without the possibility of improvement due to previous or concurrent treatment in that eye. Twelve patients met the selection criteria. Over time, these patients had significant improvements in the visual acuity in the weaker study eye, characteristically accompanied by a concurrent decrease in visual acuity in the other eye, which initially had better visual acuity. Moreover, in all the study eyes, an unstable pseudo-central von Noorden fixation pattern evolved into a more stable eccentric preferred retinal locus.Conclusions: Visual acuity remains the gold standard for assessing visual functioning in age-related maculopathy when interpreted with caution. Improvements in visual acuity can occur solely due to the course of the disease in the other eye and as a result of its impact on binocular fixation characteristics. This finding has significant implications for low-vision rehabilitation and evaluation of various therapies in large long-term clinical studies.

Neural and perceptual adaptations in bilateral macular degeneration: an integrative review.

Older individuals with macular diseases, such as age-related macular degeneration, experience central vision loss (CVL) due to degeneration of their photoreceptors and retinal cells. Patients with CVL may experience various vision impairments, including of visual acuity, fixation stability, contrast sensitivity, and stereoacuity. After CVL, most patients develop a preferred retinal locus outside the affected macular region, which serves as a new visual reference. In this review, we provide an overview of the visual function and impairment in individuals with CVL. In addition, the important role of biofeedback training on the visual function and activity of individuals with CVL is also reviewed. Accordingly, the location and development of the preferred retinal loci are discussed. Finally, this review discusses how to conduct biofeedback training to treat individuals with CVL.

Objective To investigate the distribution of the preferred retinal locus (PRL) of eccentric fixation in eyes with high myopia. Methods A total of 40 highly myopic patients (54 eyes) with eccentric fixation were examined by MP-1 microperimeter to identify the PRL. The position of PRL relative to the fovea was estimated using the 90% confidence ellipse of normal adult foveal fixation. The differences of visual acuity between desirable and PRL were tested by analysis of variance. Results In 54 eyes with high myopia, 24 eyes (44.44%) had PRL of eccentric fixation below the scotoma after loss of central vision; 19 eyes (35.19%) had a left-field PRL; 6 eyes (11.11%) had an upper-field PRL; and 5 eyes (9.26%) had right-field PRL. In 14 patients who had binocular eccentric fixation, 13 had the same fixation pattern in both eyes, including lower-field PRL in 7 (50.00 %), left-field PRL in 5 (35.71 %), and upper-field PRL in 1 patient (7.14%). The difference of visual acuity between lower- and left-field PRL group and right- and upper-field undesirable PRL group was not statistically significant (F=0.144, P > 0.05). Conclusions The eccentric fixation in eyes with high myopia is usually situated as near as possible to the fovea. The optimal PRL is inferior visual field. Key words: Myopia; degenerative/complications; Fixation; ocular/pathophysiology; Perimetry

Many persons with low vision have diseases that damage the retina only in selected areas, which can lead to scotomas (blind spots) in perception. The most frequent of these diseases is age-related macular degeneration (AMD), in which foveal vision is often impaired by a central scotoma that impairs vision of fine detail and causes problems with reading and recognizing faces. One would think that differences between damaged and relatively intact areas should be painfully obvious to those who have AMD. However, as clinical practice and scientific studies have shown, this is often not the case (Safran & Landis, 1996, 1999; Schuchard, 1995). Thus, patients and rehabilitation workers need to know where on the retina or in the visual field vision is still good enough to be usable. Having this information increases the chances for successful rehabilitation, the necessity and feasibility of which have been demonstrated (Backman & Inde, 1979; Nilsson, Frennesson, & Nilsson, 2003; Otto, 1969; Park, 1999). The fact that rehabilitation is possible emphasizes the need for a topographic assessment of remaining vision. In this article, I describe the Macular Mapping Test (MMT) and illustrate with case reports how it can be used in clinical practice to assist in the assessment and rehabilitation of patients with central vision loss. THE IDEAL SOLUTION Topographic assessment is best done by a scanning laser ophthalmoscope (SLO) that shows live images of the retina and the stimulus that is scanned directly onto it. A useful vision assessment in AMD does not need to encompass the entire visual field. It can be restricted to the macula, a central area of 20 degrees diameter, where most preferred retinal loci (PRL) develop (Fletcher & Schuchard, 1997). If small spots of light are flashed onto the retina (microperimetry), the patient indicates which are detected and which are not. For precise placement of stimuli, modern software compensates for involuntary eye movements during testing, so that precise fixation is not necessary (MacKeben & Gofen, 2007). Since SLOs are expensive and rare, they are not available to most vision professionals. WHAT OTHER MEANS ARE AVAILABLE? Conventional perimetry can provide relevant data, but it is time-consuming and requires sophisticated equipment and special expertise. In addition, the resulting intensity thresholds are not needed to find usable remaining vision for rehabilitation. The standard tangent screen test has shortcomings in that it makes it hard to include a discrimination (recognition) paradigm for a better measurement of visual performance. Furthermore, it is difficult to time the appearance and disappearance of a target precisely at defined locations, and the results can vary with the examiner's level of skill and personal habits. The Amsler grid is a subjective test that is sensitive only to drastic changes, but not to those that develop over longer periods. At best, it indicates where damage has been done, but not visual performance. In addition, the normal filling-in mechanism often prevents the detection of scotomas of less than 5 degrees diameter (Ramachandran & Gregory, 1991; Schuchard, 1993). [FIGURE 1 OMITTED] Note that measuring visual acuity alone is insufficient, despite the importance of visual acuity for prescribing optical aids, because it has limited utility in predicting the performance of activities of daily living, like reading (Backman, 2000; Crossland, Culham, & Rubin, 2005) or visually guided mobility (Brown, Brabyn, Welch, Haegerstrom-Portnoy, & Colenbrander, 1986; Hassan, Lovie-Kitchen, & Woods, 2002; Marron & Bailey, 1982) after the loss of central vision. Also note that subsets of the data presented here were published previously (MacKeben & Colenbrander, 2000; MacKeben, Colenbrander, & Gofen, 1999). DESCRIPTION OF THE TEST Procedure Although MMT is not new (MacKeben & Colenbrander, 2000; MacKeben et al. …

Patients with age-related macular degeneration (AMD) or hereditary macular dystrophies (JMD) rely on an efficient use of their peripheral visual field. We trained eight AMD and five JMD patients to perform a texture-discrimination task (TDT) at their preferred retinal locus (PRL) used for fixation. Six training sessions of approximately one hour duration were conducted over a period of approximately 3 weeks. Before, during and after training twelve patients and twelve age-matched controls (the data from two controls had to be discarded later) took part in three functional magnetic resonance imaging (fMRI) sessions to assess training-related changes in the BOLD response in early visual cortex. Patients benefited from the training measurements as indexed by significant decrease (p = 0.001) in the stimulus onset asynchrony (SOA) between the presentation of the texture target on background and the visual mask, and in a significant location specific effect of the PRL with respect to hit rate (p = 0.014). The following trends were observed: (i) improvement in Vernier acuity for an eccentric line-bisection task; (ii) positive correlation between the development of BOLD signals in early visual cortex and initial fixation stability (r = 0.531); (iii) positive correlation between the increase in task performance and initial fixation stability (r = 0.730). The first two trends were non-significant, whereas the third trend was significant at p = 0.014, Bonferroni corrected. Consequently, our exploratory study suggests that training on the TDT can enhance eccentric vision in patients with central vision loss. This enhancement is accompanied by a modest alteration in the BOLD response in early visual cortex.

Reading with simulated scotomas: attending to the right is better than attending to the left.

Following the onset of central vision loss, most patients develop an eccentric retinal location outside the affected macular region, the preferred retinal locus (PRL), as their new reference for visual tasks. The first goal of this article is to present behavioral evidence showing the presence of experience-dependent plasticity in people with central vision loss. The evidence includes the presence of oculomotor re-referencing of fixational saccades to the PRL; the characteristics of the shape of the crowding zone (spatial region within which the presence of other objects affects the recognition of a target) at the PRL are more "foveal-like" instead of resembling those of the normal periphery; and the change in the shape of the crowding zone at a para-PRL location that includes a component referenced to the PRL. These findings suggest that there is a shift in the referencing locus of the oculomotor and the sensory visual system from the fovea to the PRL for people with central vision loss, implying that the visual system for these individuals is still plastic and can be modified through experiences. The second goal of the article is to demonstrate the feasibility of applying perceptual learning, which capitalizes on the presence of plasticity, as a tool to improve functional vision for people with central vision loss. Our finding that visual function could improve with perceptual learning presents an exciting possibility for the development of an alternative rehabilitative strategy for people with central vision loss.

Perifoveal laser photocoagulation has been proposed for the treatment of subfoveal neovascular membranes in age-related macular degeneration. We evaluated residual function in seven eyes of six treated patients by means of transient focal visual potentials evoked with a scanning laser ophthalmoscope. The site of the preferred retinal locus was determined. The modulation of the helium-neon laser beam generated three tests (a homogeneous 6 x 6 degrees square--offset and onset--and two alternating pattern checkerboards 6 x 6 degrees and 2.5 x 2.5 degrees 60', 2 Hz) projected onto the preferred retinal locus. The focal visual evoked potentials were recorded. One eye had an unstable fixation with no discernible focal visual evoked potentials. The other six eyes had a stable fixation located in the superior retina, temporally for the right eyes and nasally for the left eyes. The homogeneous 6 x 6 degrees square evoked discernible responses in all six patients. The two checkerboards evoked discernible responses in five of six patients. These results were compared with those recorded in four controls in whom the three tests were projected onto the same retinal areas as in the patients. Evoked responses were more often recorded in the preferred retinal locus of the treated patients with age-related macular degeneration than in the corresponding retinal areas of the controls. The scanning laser ophthalmoscope allowed us to control the site of stimulation in the patients' and controls' retinas. These preliminary results suggest that there may be a functional plasticity of the visual system after therapeutic laser-induced central scotoma.

The aim of this study is to evaluate the effectiveness of an eccentric viewing training program that combines biofeedback training using micro-perimetry with home exercises on multiple visual function parameters and to explore potential relationships between post-treatment visual function parameters. A retrospective observational review of 27 participants who underwent the training program was performed. Eligible participants were diagnosed with bilateral central scotomas secondary to age-related macular degeneration. All participants undertook up to 15 visual and acoustic biofeedback training sessions and were required to partake in traditional home exercises between sessions. The biofeedback training was conducted in the better eye using the Macular Integrity Assessment microperimeter (MAIA). Distance and near acuity, contrast sensitivity and fixation stability quantified by the P1 and P2 values and the 63% and 95% bivariate contour ellipse area (BCEA) before and after the rehabilitation program were recorded. Significant improvement was noted post-training for distance visual acuity (t(26) = 4938 p = .000), near visual acuity (Z = −4.461 p = .000), contrast sensitivity (Z = −3.647 p = .000) and fixation stability for all measures, including P1 (t(26) = −9.490 p = .000), P2 (t(26) = −7.338 p = 0.000), 63% BCEA (Z = 3.569 p = .000) and 95% BCEA (t(26) = 4.687 p = .000). Significant medium-to-large correlations were also found between most visual function variables. Visual and acoustic biofeedback in conjunction with home exercises has the potential to improve visual function parameters in patients with age-related macular degeneration and irreversible central vision loss.

To compare macular atrophy (MA) secondary to age-related macular degeneration (AMD) and Stargardt disease (STGD) using the choroidal vascularity index (CVI). In this multicentric retrospective study, two distinct cohorts were collected: patients with MA secondary to AMD and MA secondary to STGD. All patients were investigated using a multimodal imaging approach, including CVI in the subfoveal 1000μm area. Of note, the CVI is not influenced by aging, which allows comparisons between different cohorts. Seventy eyes were included: 35 eyes of 35 patients (mean age 78 ± 7years) in the AMD group and 35 eyes of 35 patients (mean age 41 ± 16years, p < 0.001) in the STGD group. Choroidal thickness was significantly lower in the AMD group in comparison to the STGD group (151 ± 80μm vs 353 ± 105μm, p < 0.001). The total choroidal area (TCA) was significantly greater in the STGD group in comparison to the AMD group (1.734 ± 0.958mm2 vs 0.538 ± 0.391mm2, respectively, p < 0.001). Interestingly, the CVI was significantly lower in AMD patients in comparison to STGD patients (27.322 ± 15.320% vs 49.880 ± 7.217%, respectively, p < 0.001), and this difference was confirmed in the subgroup of patients over 50 years old. Our results corroborate the hypothesis that large choroidal vessels were impaired to a greater extent in AMD than in STGD. CVI may help in differentiating AMD from STGD in the presence of MA, better understanding of the pathogenesis, and monitoring of therapeutic response.

For patients with central vision loss and controls with normal vision, we examined the horizontal vestibulo-ocular reflex (VOR) in complete darkness and in the light when enhanced by vision (VVOR). We expected that the visual-vestibular interaction during VVOR would produce an asymmetry in the gain due to the location of the preferred retinal locus (PRL) of the patients. In the dark, we hypothesized that the VOR would not be affected by the loss of central vision. Nine patients (ages 67 to 92 years) and 17 controls (ages 16 to 81 years) were tested in 10-s active VVOR and VOR procedures at a constant frequency of 0.5 Hz while their eyes and head movements were recorded with a video-based binocular eye tracker. We computed the gain by analyzing the eye and head peak velocities produced during the intervals between saccades. In the light and in darkness, a significant proportion of patients showed larger leftward than rightward peak velocities, consistent with a PRL to the left of the scotoma. No asymmetries were found for the controls. These data support the notion that, after central vision loss, the preferred retinal locus (PRL) in eccentric vision becomes the centre of visual direction, even in the dark.