Case Report: An Unusual Presentation of Leber's Hereditary Optic Neuropathy.

SIGNIFICANCE: Leber's hereditary optic neuropathy (LHON) is often associated with onset in the young, adult male demographic. This case report serves as a reminder that it can affect both sexes with onset into middle age. PURPOSE: Leber's hereditary optic neuropathy is a maternally inherited mitochondrial disorder that typically affects men during young adulthood. It presents with a rapid, yet painless loss of vision, with the fellow eye often affected within a few months. The optic neuropathy causes a dense central scotoma with visual acuities reduced to less than 20/400. CASE REPORT: A 60-year-old White woman presented with reports of decreased vision in both eyes for the previous 2 months. She had been followed up for the previous 5 years for glaucoma suspect monitoring, with full fields and normal optical coherence tomography scans. Entering visual acuity was finger counting at 1 m in the right eye and 20/100 in the left eye. Pupil testing revealed a grade 1 relative afferent pupillary defect in the right eye. Dilated fundus examination revealed stable moderate optic nerve cupping and intact neuroretinal rim tissue. Humphrey 24-2 Swedish Interactive Thresholding Algorithm standard visual field testing showed a significant superior altitudinal defect and inferior paracentral defect in the right eye and a partial superior arcuate in the left eye. The result of the MRI with contrast of the head and orbits was normal. A history of alcoholism was elicited, and LHON testing revealed positive 11778 mutation at homoplasmy. CONCLUSIONS: Although still uncommon, presentation of LHON in a middle-aged woman is possible and should be considered a viable differential diagnosis when individuals present with painless vision loss and central/centrocecal scotomas.

Effectiveness of visual and acoustic biofeedback eccentric viewing training in conjunction with home exercises on visual function: a retrospective observational review.

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.

Population-based associations between progression of normal-tension glaucoma and Yang-deficient constitution among Chinese persons.

PURPOSE: To explore the association between constitution types as defined by traditional Chinese medicine (TCM) and risk for normal-tension glaucoma (NTG). DESIGN: Population-based cohort study. METHODS: Persons were identified in a population cohort aged ≥30 years with NTG, defined as having an untreated mean intraocular pressure measurement ≤21 mm Hg over six separate occasions, with no single reading >24 mm Hg (as in the Collaborative Normal Tension Glaucoma Study). The Body Constitution in Traditional Chinese Medicine Questionnaire was used to assess each participant's TCM constitution types. The association between various constitutions and visual field progression according to Early Manifest Glaucoma Trial criteria was assessed using Cox regression HR models. RESULTS: Among 142 participants (245 eyes), 23 persons (17.6%) and 25 eyes (10.2%) progressed, over a mean (SD) follow-up duration of 3.49 (0.99) years. Progression rates were highest in participants with Yang-deficient constitution (n=19, 13.4%), among whom 7 (36.8%) exhibited worsening fields. After adjusting for sex, age, central corneal thickness, retinal nerve fibre layer thickness and mean deviation on visual field testing, Yang-deficient constitution (HR 4.63, 95% CI 1.77 to 12.1, p=0.002) and higher mean intraocular pressure during follow-up (HR 1.25, 95% CI 1.01 to 1.56, p=0.044) were associated with field progression. CONCLUSIONS: Yang-deficient constitution and higher intraocular pressure are risk factors for visual field progression in NTG patients. Yang deficiency is characterised by abnormal vasoregulation, and these results may be consistent with prior studies linking NTG progression to Raynaud's phenomenon and migraine.

Glaucoma suspects referred by general ophthalmologists to a tertiary center in Brazil: outcomes of the glaucoma specialist assessment.

PURPOSE: To characterize patients with suspected glaucoma who were referred to the clinic for suspected glaucoma in a tertiary public hospital in southern Brazil and to evaluate differences in functional and structural damages between patients diagnosed with different types of glaucoma, those with normal eye examination results, and those who remained as glaucoma suspects. METHODS: This is a cohort study of patients referred by general ophthalmologists to the clinic for suspected glaucoma at Hospital Nossa Senhora da Conceição, Porto Alegre, Brazil, between March 2016 and December 2018. The patients were followed up until they had undergone reliable examinations (eye examination, visual field screening, and optic coherence tomography for classification as normal and having a suspected glaucoma, glaucoma with an elevated intraocular pressure, normotensive glaucoma, or ocular hypertension. RESULTS: A total of 135 patients were included in this study. Of the patients, 117 subjects completed all examinations and met the inclusion criteria. Most patients were normal (36.8%), followed by those with suspected glaucoma (25.64%), normal tension glaucoma (18.8%), glaucoma with elevated intraocular pressure (12%), and ocular hypertensive (6%). The main reason for referral was increased optic nerve head cupping. The patients with normal tension glaucoma were older than the other subjects on average (p=0.03). In addition, the normal tension glaucoma group had a significantly worse baseline visual field index and mean deviation of the visual field than the normal, glaucoma suspect, and ocular hypertensive groups. The circumpapillary retinal nerve fiber layer on OCT was thinner on average in the normal tension glaucoma group than in the normal and glaucoma suspect groups (p<0.002) but did not significantly differ between the glaucoma group with elevated intraocular pressure and the other groups. CONCLUSIONS: Patients with normal tension glaucoma tend to be diagnosed later because of their normal intraocular pressures; thus, the optic nerve cupping must be greater to raise the suspicion of glaucoma. In this study, we found that the patients with normal tension glaucoma had worse disease at the time of diagnosis.

Outcomes of transcorneal electrical stimulation therapy in the early stages of retinitis pigmentosa.

BACKGROUND: To investigate the effect of transcorneal electrical stimulation (TES) therapy in patients with retinitis pigmentosa (RP). METHODS: We performed TES therapy in 21 patients with RP in 12 sessions with 1-week intervals. The following parameters obtained before and after the TES therapy were compared statistically; the best corrected visual acuity (BCVA, logMAR), Ishihara color vision level, multifocal electroretinography (mf-ERG) response, automated visual field (VF) outcome, and the 25-item low vision quality-of-life (LVQOL) questionnaire points. RESULTS: The mean age of patients (6 females; 15 males) was 31.67 ± 9.80 years (20-50 years). While increases in BCVA level, color vision level, mf-ERG response in p1 amplitude of ring 1, and LVQOL questionnaire points were statistically significant, changes in VF test and other mf-ERG responses were not. Twenty of the patients (95.24%) stated that they were satisfied with the TES therapy. No considerable side effect was observed in any patient due to the therapy. DISCUSSION: The TES therapy may be an effective and safe treatment modality in slowing the RP progression, especially in the early stages of the disease. Longer-term follow-ups in larger patient populations are warranted.

Influence of implantation of diffractive trifocal intraocular lenses on standard automated perimetry.

BACKGROUND: This prospective comparative study aimed to investigate the influence of diffractive trifocal intraocular lenses (IOLs) implantation on standard automated perimetry. METHODS: Patients with no diseases affecting the visual field had undergone cataract surgery following the implantation of trifocal or monofocal IOLs from July 2019 to August 2020 were recruited. The normality of the anterior and posterior segments and absence of glaucomatous optic nerve cupping were confirmed preoperatively by slit-lamp examination. Standard automated perimetry was performed using Humphrey Visual Field 10-2 testing, 2-3 months after cataract surgery in only one eye per patient. The mean deviation (MD) and foveal sensitivity were compared between IOLs in eyes with acceptable reliability indices and best-corrected visual acuity of 20/25 or better. RESULTS: Among the 83 eyes of the 83 patients included, 39 and 29 eyes eligible for perimetry analysis had trifocal and monofocal IOLs, respectively. The mean MD and foveal sensitivity in eyes with trifocal IOLs were significantly lower than those in eyes with monofocal IOLs (P < 0.021), with mean differences of 0.77 and 1.01 dB, respectively. CONCLUSION: The comparison in nonglaucomatous eyes demonstrated that the influence of trifocal IOLs on standard automated perimetry was greater than that of monofocal IOLs.

Enhanced depth imaging optical coherence tomography of the optic nerve head improves correct diagnosis in glaucoma suspects without glaucomatous optic disc morphology.

An 83-year-old woman with a long history of glaucoma and optic disc drusen (ODD) was referred for neuro-ophthalmological second opinion. The patient had been treated for decades with bilateral intraocular pressure (IOP)-lowering eye drops, laser trabeculoplasty and trabeculectomy and had severe, bilateral loss of visual fields and retinal nerve fibre layer (RNFL) thinning on optical coherence tomography (OCT) despite IOP that never exceeded 24 mm Hg. On ophthalmoscopy, only a single ODD was visible in the left eye and no optic disc cupping was apparent in either eye. Enhanced depth imaging OCT (EDI-OCT) of the optic nerve head revealed bilateral multiple, large, deep ODD, which in itself could easily explain the visual field loss and RNFL thinning of this patient. Optic nerve head examination using EDI-OCT is highly recommended for patients with a history of glaucoma but without optic nerve head cupping to avoid a potential misdiagnosis with consequent unnecessary treatment.

Visual Dysfunction and Structural Correlates in Sorsby Fundus Dystrophy.

PURPOSE: To elucidate morphological determinants of rod and cone dysfunction in Sorsby fundus dystrophy (SFD), and to systematically compare visual function tests for interventional trials. DESIGN: Prospective cross-sectional study. METHODS: Patients with SFD (n = 16) and controls (n = 20) underwent visual function testing (best-corrected visual acuity [BCVA] and low luminance visual acuity [LLVA], contrast sensitivity, mesopic and dark-adapted (DA) fundus-controlled perimetry [FCP], rod-mediated dark adaptation [RMDA]), and multimodal imaging. Vision-related quality of life was evaluated. FCP and RMDA thresholds were analyzed using mixed models and structure-function correlation using machine learning (ML). Longitudinal data of 1 patient with high-dose vitamin A supplementation were available. RESULTS: Although photopic BCVA was normative in SFD, LLVA was impaired (0.30 LogMAR [0.20; 0.45] vs 0.20 LogMAR [0.03; 0.28], P < .05). Scotopic visual function exhibited a delayed rod-intercept time (21 minutes [12.15; 21] vs 4.05 minutes [3.22; 5.36], P < .001), and marked DA cyan mean sensitivity loss (-11.80 dB [-3.47; -19.85]), paralleled by a reduced vision-related quality of life. ML-based structure-function correlation allowed prediction of mesopic, DA cyan, and red sensitivity with high accuracy (cross-validated mean absolute error: 4.36, 7.77, and 5.31 dB, respectively), whereas RMDA could be slowed even in the absence of fundus alterations on multimodal imaging. After high-dose vitamin A supplementation, RMDA and DA thresholds improved markedly. CONCLUSIONS: Patients with SFD exhibit severely impaired scotopic visual function even in the absence of funduscopic alterations on multimodal imaging. In contrast to BCVA, scotopic visual function tests are suitable to quantify dysfunction in the early stages. Improvement of scotopic dysfunction after (off-label) high-dose vitamin A intake, as observed in one patient in our study, is compatible with the hypothesized local deficiency of vitamin A secondary to Bruch's membrane alterations.

Spacetime in the brain: rapid brain network reorganization in visual processing and recovery.

Functional connectivity networks (FCN) are the physiological basis of brain synchronization to integrating neural activity. They are not rigid but can reorganize under pathological conditions or during mental or behavioral states. However, because mental acts can be very fast, like the blink of an eye, we now used the visual system as a model to explore rapid FCN reorganization and its functional impact in normal, abnormal and post treatment vision. EEG-recordings were time-locked to visual stimulus presentation; graph analysis of neurophysiological oscillations were used to characterize millisecond FCN dynamics in healthy subjects and in patients with optic nerve damage before and after neuromodulation with alternating currents stimulation and were correlated with visual performance. We showed that rapid and transient FCN synchronization patterns in humans can evolve and dissolve in millisecond speed during visual processing. This rapid FCN reorganization is functionally relevant because disruption and recovery after treatment in optic nerve patients correlated with impaired and recovered visual performance, respectively. Because FCN hub and node interactions can evolve and dissolve in millisecond speed to manage spatial and temporal neural synchronization during visual processing and recovery, we propose "Brain Spacetime" as a fundamental principle of the human mind not only in visual cognition but also in vision restoration.

Night Vision and Carotenoids (NVC): A Randomized Placebo Controlled Clinical Trial on Effects of Carotenoid Supplementation on Night Vision in Older Adults.

Twilight and low luminance levels are visually challenging environments for the elderly, especially when driving at night. Carotenoid rich diets are known to increase macular pigment optical density (MPOD), which in turn leads to an improvement in visual function. It is not known whether augmenting MPOD can lead to a decrease in vision related night driving difficulties. Additionally, it is unknown if carotenoid supplementation provides additional measurable benefits to one's useful field of view (UFOV) along with a decreased composite crash risk score. The aim of the study was to evaluate changes in night vision function and UFOV in individuals that took carotenoid vitamin supplements for a six-month period compared to a placebo group. METHODS: A prospective, randomized, double-blind, six-month trial of a 14 mg zeaxanthin/7 mg lutein-based supplement was carried out. Participants were randomized into active or placebo group (approx 2:1). RESULTS: n = 33 participants (26 males/7 females) participated with 93% capsule intake compliance in the supplemented group (n = 24) and placebo group (n = 9). MPOD (mean/standard error SE) in the active group increased in the Right eye from 0.35 density units (du)/0.04 SE to 0.41 du/0.05 SE; p < 0.001 and in the Left eye from 0.35 du/0.05 SE to 0.37 du, p > 0.05). The supplemented group showed significant improvements in contrast sensitivity with glare in both eyes with improvements in LogMAR scores of 0.147 and 0.149, respectively (p = 0.02 and 0.01, respectively), monocularly tested glare recovery time improved 2.76 and 2.54 s, respectively, (p = 0.008 and p = 0.02), and we also noted a decreased preferred luminance required to complete visual tasks (p = 0.02 and 0.03). Improvements in UFOV scores of divided attention (p < 0.001) and improved composite crash risk score (p = 0.004) were seen in the supplemented group. The placebo group remained unchanged. CONCLUSIONS: The NVC demonstrates that augmenting MPOD in individuals with difficulty in night vision showed measurable benefits in numerous visual functions that are important for night vision driving in this small sample RCT. Additionally, we observed an improvement in UFOV divided attention test scores and decreased composite risk scores.

Incidence of non-glaucomatous ocular disease in patients with asymmetric optic disc cupping.

PURPOSE: To evaluate the incidence of non-glaucomatous ocular disease in patients with asymmetric optic disc cupping. METHODS: A retrospective case series, including consecutive patients with cup-to-disc ratio (CDR) asymmetry greater than 0.2. All patients underwent a complete neuro-ophthalmological examination, automated perimetry with the Humphrey 24-2 visual fields program. Retinal nerve fibre layer thickness was measured by optical coherence tomography (OCT). The results of neuroimaging, macular OCT and blood tests were recorded as well. Patients were assigned a diagnosis of glaucomatous optic neuropathy (GON) or non-glaucomatous disease (NGD). The main outcome measure was the rate of non-glaucomatous ocular disease. RESULTS: A total of 120 (67 males) patients with a mean age of 71.1 ± 12.5 years met the inclusion criteria and were included in this study. The mean asymmetry in CDR between the eyes was 0.3 ± 0.13 (range, 0.2-0.9). Twenty patients (16.6%) had a visual field defect not typical for glaucoma and positive relative afferent pupillary defect was found in 24 patients (20%). Six patients were found to have newly diagnosed non-glaucomatous ocular disease: maculopathy in three patients, retinopathy in one patient and traumatic optic neuropathy in two patients. Patients with NGD were significantly younger than the patients with GON (59.8 ± 23.3 vs. 71.3 ± 11.5 years, P = 0.001). Optic disc pallor was found in 4/93 patients with glaucoma compared to 3/6 with newly diagnosed non-glaucomatous disease (4.7% vs. 50.0%, P = 0.03). CONCLUSIONS: Asymmetric optic disc cupping can be associated with non-glaucomatous disease and may warrant neuro-ophthalmological evaluation, especially in younger patients or those with optic disc pallor.

Use of computerized campimetry and/or optical coherence tomography for glaucoma diagnosis by non-glaucoma specialists / Uso da campimetria e/ou da tomografia de coerência óptica no diagnóstico do glaucoma por oftalmologistas generalistas

ABSTRACT Purpose: To compare the use of visual field and/or optical coherence tomography (OCT) combined with color retinography by non-glaucoma specialists for differentiating glaucoma from physiological cupping. Methods: Eighty patients with glaucoma or physiological cupping (40 of each) were randomized according to the examination used (GI: color retinography, GII: color retinography + visual field, GIII: color retinography + optical coherence tomography, GIV: color retinography + visual field + optical coherence tomography). Twenty non-specialist ophthalmologists diagnosed glaucoma from PowerPoint slide images, without direct patient examination. Results: Inter-examiner agreement was good for GII (ĸ: 0.63; 95%CI, 0.53-0.72), moderate for GIII (ĸ: 0.58; 95%CI, 0.48-0.68) and GIV (ĸ: 0.41; 95%CI, 0.31-0.51), and low for GI (ĸ: 0.30; 95%CI, 0.20-0.39) (p<0.001). Diagnostic accuracy was higher in GIII (15.8 ± 1.82) than GI (12.95 ± 1.46, p<0.001) and higher in GII (16.25 ± 2.02) than GI and GIV (14.10 ± 2.24) (both p<0.001). For glaucoma patients only, diagnostic accuracy in GII and GIII was superior to that in GI and GIV (both p<0.001). Sensitivity and specificity were 59% and 70.5% in GI; 86.5% and 76% in GII, 86.5% and 71.5% in GIII; and 68.5% and 72.5% in GIV, respectively. Accuracy was highest in GII (81.3% [95%CI, 77.1-84.8]), followed by GIII (79% [95%CI, 74.7-82.7]), GIV (70,5% [95%CI, 65.9-74.8]), and GI (64.8% [95%CI, 60.0-69.3]). Conclusions: Non-glaucoma specialists could not differentiate glaucoma from increased physiological cupping when using color retinography assessment alone. Diagnostic accuracy and inter-rater agreement improved significantly with the addition of visual field or optical coherence tomography. However, the use of both modalities did not improve sensitivity/specificity.(AU)

RESUMO Objetivos: Verificar a influência do campo visual e/ou tomografia de coerência óptica, quando analisados em associação à retinografia colorida, na diferenciação entre indivíduos com glaucoma daqueles com aumento fisiológico de escavação. Métodos: Oitenta pacientes com glaucoma ou aumento fisiológico de escavação (40 cada) foram randomizados de acordo com o exame testado (GI: retinografia colorida, GII: retinografia colorida + campo visual, GIII: retinografia colorida + tomografia de coerência óptica, GIV: retinografia colorida + campo visual + tomografia de coerência óptica). Vinte oftalmologistas não especialistas em glaucoma diagnosticaram glaucoma através de slides do PowerPoint, sem o exame direto do paciente. Resultados: A concordância interexaminador foi boa para o GII (ĸ: 0,63; 95%CI, 0,53-0,72), moderada para GIII (ĸ: 0,58; 95%CI, 0,48-0,68) e GIV (ĸ: 0,41; 95%CI, 0,31-0,51), e baixa para o GI (ĸ: 0,30; 95%CI, 0,20-0,39) (p<0,001). Acurácia diagnostica foi maior no GIII (15,8 ± 1,82) em comparação ao GI (12,95 ± 1,46, p<0,001) e o GII (16,25 ± 2,02) maior em comparação ao GI e GIV (14,10 ± 2,24) (para ambos, p<0,001). Para os pacientes com glaucoma, a acurácia diagnostica nos grupos GII e GIII foi superior do que em GI e GIV (ambos p<0,001). Sensibilidade e especificidade foram 59% e 70,5% no GI; 86,5% e 76% no GII, 86,5% e 71,5% no GIII; 68,5% e 72,5% no GIV, respectivamente. A acurácia foi maior no GII (81,3% [95%CI, 77,1-84,8]), seguido pelo GIII (79% [95%CI, 74,7-82,7]), GIV (70,5% [95%CI, 65,9-74,8]), e GI (64,8% [95%CI, 60,0-69,3]). Conclusões: A avaliação isolada da retinografia colorida por oftalmologistas não especialistas em glaucoma não pode diferenciar pacientes com glaucoma daqueles com aumento fisiológico de escavação. Houve aumento da acurácia diagnóstica e da concordância interobservador com o acréscimo do campo visual ou da tomografia de coerência óptica. Entretanto, o uso de ambas as modalidades não melhorou a sensibilidade/especificadade.(AU)

Glaucoma Mimickers: A major review of causes, diagnostic evaluation, and recommendations.

Background: Glaucoma is characterized by chronic progressive optic neuropathy with corresponding visual field changes, with or without raised intraocular pressure (IOP). When diagnosing glaucoma or monitoring its progression, the examiner has to rely on the appearance of the optic disc, characteristic retinal nerve fiber layer defects, and corresponding visual field defects. However, similar changes and symptoms may be observed in several other disorders of the optic nerve and retina that may mimic glaucoma, often leading to misdiagnosis. Methods and result: The consequences of misdiagnosis not only result in improper treatment that may impact vision but also would negatively affect the overall health, psychological well-being of the patient, and may have considerable economic implications.Conclusion: The current review describes various conditions that mimic glaucoma and the features that help differentiate these conditions from glaucoma.

Use of computerized campimetry and/or optical coherence tomography for glaucoma diagnosis by non-glaucoma specialists.

PURPOSE: To compare the use of visual field and/or optical coherence tomography (OCT) combined with color retinography by non-glaucoma specialists for differentiating glaucoma from physiological cupping. METHODS: Eighty patients with glaucoma or physiological cupping (40 of each) were randomized according to the examination used (GI: color retinography, GII: color retinography + visual field, GIII: color retinography + optical coherence tomography, GIV: color retinography + visual field + optical coherence tomography). Twenty non-specialist ophthalmologists diagnosed glaucoma from PowerPoint slide images, without direct patient examination. RESULTS: Inter-examiner agreement was good for GII (ĸ: 0.63; 95%CI, 0.53-0.72), moderate for GIII (ĸ: 0.58; 95%CI, 0.48-0.68) and GIV (ĸ: 0.41; 95%CI, 0.31-0.51), and low for GI (ĸ: 0.30; 95%CI, 0.20-0.39) (p<0.001). Diagnostic accuracy was higher in GIII (15.8 ± 1.82) than GI (12.95 ± 1.46, p<0.001) and higher in GII (16.25 ± 2.02) than GI and GIV (14.10 ± 2.24) (both p<0.001). For glaucoma patients only, diagnostic accuracy in GII and GIII was superior to that in GI and GIV (both p<0.001). Sensitivity and specificity were 59% and 70.5% in GI; 86.5% and 76% in GII, 86.5% and 71.5% in GIII; and 68.5% and 72.5% in GIV, respectively. Accuracy was highest in GII (81.3% [95%CI, 77.1-84.8]), followed by GIII (79% [95%CI, 74.7-82.7]), GIV (70,5% [95%CI, 65.9-74.8]), and GI (64.8% [95%CI, 60.0-69.3]). CONCLUSIONS: Non-glaucoma specialists could not differentiate glaucoma from increased physiological cupping when using color retinography assessment alone. Diagnostic accuracy and inter-rater agreement improved significantly with the addition of visual field or optical coherence tomography. However, the use of both modalities did not improve sensitivity/specificity.

The role of clinical examination of the optic nerve head in glaucoma today.

PURPOSE OF REVIEW: The study was undertaken to assess the current importance of clinical examination of the optic nerve head (ONH) in glaucoma management. RECENT FINDINGS: ONH changes in glaucoma are easily assessed with good specificity on examination, whereas imaging has not been able to identify pallor of the neuroretinal rim (NRR), disc hemorrhages, or vascular signs of acquired cupping. Glaucomatous neuropathy in primary open angle glaucoma (POAG) has extensive databases on imaging machines, however, other glaucomas such as primary angle closure glaucoma (PACG), juvenile open angle glaucoma (JOAG), congenital, and secondary glaucomas have been reported to have a significantly different ONH morphology. Clinical evaluation with knowledge of the underlying cause of glaucoma allows easy diagnosis of any abnormality, whereas the absence of an imaging database in such eyes makes diagnosis difficult on optical coherence tomography (OCT) and so forth. In eyes with congenital anomalies and dysplastic discs, clinical examination and history provide better identification of glaucomatous damage. Staging of glaucoma on ONH examination when perimetry is unreliable provides a means of determining the amount of damage and appropriate reduction of intraocular pressure (IOP). SUMMARY: Clinical examination of the ONH is very relevant for screening, diagnosis, staging, and management of all glaucomas, and is irreplaceable in detecting nonglaucomatous causes of ONH cupping and visual field defects similar to those in glaucoma.

Effectiveness of Low Vision Rehabilitation Using Microperimetric Acoustic Biofeedback Training in Patients with Central Scotoma.

PURPOSE: To evaluate the efficacy of visual rehabilitation with microperimeter biofeedback in patients with central scotoma. MATERIALS AND METHODS: 35 consecutive patients with central scotoma (17 age-related macular degeneration (AMD), 14 Stargardt disease, and 4 cone dystrophy) were included in the study. Visual acuity, reading performance by Minnesota Low Vision Reading Test (MNREAD), quality of life by 25-item National Eye Institute Visual Function Questionnaire (NEI VFQ-25), and fixation analysis by MAIA microperimeter were evaluated before and 1 month after training. The rehabilitation program consisted of 10 training sessions of 10 minutes. RESULTS: The median best-corrected visual acuity (BCVA) was 0.80 logMAR (range 0.3 to 1.3 logMAR). Fifty-nine percent of patients with AMD developed a preferred retinal locus (PRL) nasal to the fovea, and 64% of the patients with Stargardt disease preferred a PRL superior to the fovea. The PRL location in 3 of 4 cone dystrophy patients was nasal to the fovea. The mean PRL distance from the fovea was 7.57 ± 3.61 degrees. Fixation stability improved with P1 values of 22.34 ± 11.81 versus 32.05 ± 18.79 (p = .003) and 95% bivariate contour ellipse area (BCEA) values of 41.6 versus 23.6 (p = .018) before and after training, respectively. There was a significant difference in reading acuity between before and after training (p = 0.008). The overall score and near activities score of NEI VFQ-25 were found to be increased at the end of the rehabilitation (p < 0.001). CONCLUSION: Rehabilitation with acoustic biofeedback in patients with central scotoma looks like a useful technique for improving fixation stability, reading performance and quality of life.

Asian-specific vertical cup-to-disc ratio cut-off for glaucoma screening: An evidence-based recommendation from a multi-ethnic Asian population.

IMPORTANCE: Evidence-based guidelines are essential for glaucoma screening to work effectively. BACKGROUND: To derive a vertical cup-to-disc ratio (VCDR) cut-off for glaucoma screening in a multi-ethnic Asian population. DESIGN: The Singapore Epidemiology of Eye Diseases (SEED) study is a population-based study conducted from 2004 to 2011 in a single tertiary care research institute. PARTICIPANTS: SEED comprised of 10 033 Chinese, Malay and Indian adults aged ≥40 (response rate 75.6%). After excluding participants with a history of glaucoma medication or surgery, 9673 participants were included for analysis. METHODS: A systematic eye examination, which included applanation tonometry, visual field testing, gonioscopy and dilated fundus examination was conducted. MAIN OUTCOME MEASURE: Diagnosis of glaucoma. RESULTS: The distribution of VCDR and VCDR asymmetry were relatively homogenous in this multi-ethnic Asian population, with a 97.5th percentile value of 0.67 and 0.17, respectively. In the absence of more definite signs of glaucoma, VCDR ≥0.60 and VCDR asymmetry ≥0.20 provided the best balance between sensitivity (95.1%) and specificity (90.9%) in detecting glaucoma. For larger optic disc (≥2.0 mm), VCDR ≥0.65 with VCDR asymmetry ≥0.20 provided the best balance between sensitivity (84.8%) and specificity (93.2%). CONCLUSION AND RELEVANCE: Overall, VCDR ≥0.60 with VCDR ≥0.20 asymmetry provides a good balance between sensitivity and specificity in detecting glaucoma. For larger optic disc, VCDR ≥0.65 should be considered instead to mitigate against false-referrals due to larger physiological disc cupping. Our findings may act as a reference to populations with similar VCDR distribution.

Case Report: Nutritional and Toxic Optic Neuropathy: A Diagnostic Dilemma.

SIGNIFICANCE: Nutritional and toxic optic neuropathies are rare disorders characterized by visual impairment due to optic nerve damage by a toxin, usually with coexisting nutritional deficiencies. Its pathophysiology is still unclear, and multiple mechanisms implicated act synergistically to bring about this condition. The decline in its incidence and its confusing clinical appearance make diagnosing nutritional and toxic optic neuropathies challenging. PURPOSE: This is an observational clinical case report of an atypical clinical case of a nutritional and toxic optic neuropathy with a subacute presentation and papilledema at the time of diagnosis. The patient provided written informed consent for medical information and images to be published. CASE REPORT: A 47-year-old man presented with progressive, painless bilateral decrease in central vision over 15 days. The patient had a long-standing history of alcohol abuse and was a heavy smoker. The examination revealed dyschromatopsia, 20/400 visual acuity on both eyes, and no relative afferent pupillary defect. Funduscopy revealed bilateral papilledema. A visual field test showed generalized depression with centrocecal involvement in the left eye. Laboratory studies evidenced decreased vitamin B12/B1 and red blood cell folate levels, increased acute phase reactants, hypertransaminasemia, and macrocytic anemia. Serologies and methanol in urine were negative. After the discontinuation of tobacco use and alcohol accompanied by vitamin supplementation, our patient's visual field, visual acuity, and papilledema improved remarkably. After 5 months, visual acuity and funduscopy were normal. CONCLUSIONS: Although some hallmark signs were visible in this case, its subacute presentation and the presence of papilledema at diagnosis caused some diagnostic uncertainty. Nutritional and toxic optic neuropathy is a rare and challenging diagnosis because of a lack of biomarkers. Eye care clinicians should consider nutritional and toxic optic neuropathies to prevent severe and irreversible visual damage resulting from underdiagnosis and mismanagement.

Temporal Raphe Sign in Elderly Patients With Large Optic Disc Cupping: Its Evaluation as a Predictive Factor for Glaucoma Conversion.

PURPOSE: To determine baseline clinical features associated with conversion to glaucoma in elderly patients with large optic-disc cupping. DESIGN: Retrospective cohort study. METHODS: Seventy-two eyes of 72 untreated elderly (≥65-year-old) patients with large vertical cup-to-disc ratio (CDR ≥0.7) and without any other glaucomatous findings were included. They had undergone a full ophthalmologic examination twice per year for at least 5 years. The optic nerve head (ONH), peripapillary retinal nerve fiber layer (RNFL), and macular ganglion cell-inner plexiform layer (GCIPL) were imaged with Cirrus high-definition optical coherence tomography (OCT). Presence of temporal raphe sign on the OCT's GCIPL thickness map was assessed as one of the morphologic factors. Conversion to normal-tension glaucoma (NTG) was defined as structural or functional deterioration on either red-free RNFL photography or standard automated perimetry, respectively. The utility of the baseline factors associated with conversion to NTG were identified. RESULTS: During the 5.5-year follow-up, 19 eyes (26.4%) converted to NTG. There were no significant differences in demographics, systemic factors, intraocular pressure factors, or OCT parameters between the nonconverters and converters. Interestingly, the temporal raphe sign was observed in the converters (18/19, 94.7%) much more frequently than in the nonconverters (3/53, 5.7%, P < .001) at baseline. A Cox proportional hazards model indicated the significant influences of temporal raphe sign positivity (hazard ratio 6.823, 95% confidence interval 2.574, 18.088, P < .001) on conversion to NTG. CONCLUSIONS: In elderly subjects with large CDR, temporal raphe sign positivity on the baseline macular GCIPL thickness map was associated with faster conversion to NTG.

Management of Retinitis Pigmentosa via Platelet-Rich Plasma or Combination with Electromagnetic Stimulation: Retrospective Analysis of 1-Year Results.

PURPOSE: To investigate whether the natural progression rate of retinitis pigmentosa can be decreased by subtenon autologous platelet-rich plasma application alone or combination with retinal electromagnetic stimulation. METHODS: The study includes retrospective analysis of 60 patients with retinitis pigmentosa. Patients constitute three groups with similar demographic characteristics: the combined management group (group 1) consists of 20 patients with retinitis pigmentosa (40 eyes) who received combined retinal electromagnetic stimulation and subtenon platelet-rich plasma; the subtenon platelet-rich plasma-only group (group 2) consisted of 20 patients with retinitis pigmentosa (40 eyes); the natural course (control) group (group 3) consists of 20 patients with retinitis pigmentosa (40 eyes) who did not receive any treatment. Horizontal and vertical ellipsoid zone width, fundus perimetry deviation index, and best corrected visual acuity changes were compared within and between groups after a 1-year follow-up period. RESULTS: Detected horizontal ellipsoid zone percentage changes were + 1% in group 1, - 2.85% in group 2, and - 9.36% in group 3 (Δp 1 > 2 > 3). Detected vertical ellipsoid zone percentage changes were + 0.34% in group 1, - 3.05% in group 2, and - 9.09% in group 3 (Δp 1 > 2 > 3). Detected fundus perimetry deviation index percentage changes were + 0.05% in group 1, - 2.68% in group 2, and - 8.78% in group 3 (Δp 1 > 2 > 3). CONCLUSION: Platelet-rich plasma is a good source of growth factors, but its half-life is 4-6 months. Subtenon autologous platelet-rich plasma might more effectively slow down photoreceptor loss when repeated as booster injections and combined with retinal electromagnetic stimulation. TRIAL REGISTRATION: ClinicalTrials.gov identifier, NCT04252534.