Brolucizumab in recalcitrant neovascular age-related macular degeneration-real-world data in Chinese population.

This retrospective study aimed to determine the short-term efficacy and safety of brolucizumab treatment for recalcitrant neovascular age-related macular degeneration (nAMD) in a real-world setting in Taiwan. Recalcitrant nAMD patients who were treated with brolucizumab from November 2021 to August 2022 at Taipei Veterans General Hospital were included. Patients were followed for 3 months after switching to brolucizumab. The primary outcomes were changes in mean best-corrected visual acuity (BCVA) and central retinal thickness (CRT) from baseline to the third month. The secondary outcomes included the incidence of intraocular inflammation (IOI), proportion of patients with subretinal and intraretinal fluid (SRF and IRF), and change in pigment epithelial detachment (PED) height from baseline to the third month. The significance level was considered as p < .05 in all tests. A total of 38 patients (40 eyes) with a mean (±SD) age of 76.3 (±10.84) years were included. The baseline BCVA was 0.92±0.64 logMAR, and the CRT and PED height were 329.0±171.18 and 189.8±114.94 um, respectively. The patients had a significant reduction in CRT and resolution of IRF and SRF from baseline to the third month. There were numerical improvements in mean BCVA and PED height, but they were not significant. The percentages of achieving at least 0.1, 0.2, and 0.3 logMAR (equivalent to 5, 10, 15 ETDRS letters) visual gain were 50%, 37.5%, and 30%, respectively, during the first 3 months of follow-up. No IOI occurred in these patients. This study demonstrated that brolucizumab had good short-term structural and functional efficacy in recalcitrant nAMD patients.

Severe papilloedema with vision loss secondary to Guillain-Barré syndrome.

We discuss a patient who presented with bilateral VI and VII cranial nerve palsies, symmetric upper and lower limb weakness and areflexia, 2 weeks following an flu-like illness. At presentation, there was no papilloedema, and her visual function was normal. Cerebrospinal fluid analysis and electrophysiology supported the diagnosis of Guillain-Barré Syndrome (GBS). She received intravenous immunoglobulins. She subsequently developed headaches and vision loss. Funduscopy demonstrated severe papilloedema with visual acuity of 6/18 right eye, 6/12 left eye with bitemporal visual field depression. Lumbar puncture revealed elevated opening pressure with high protein and normal cell count. She received acetazolamide. There was resolution of papilloedema and normal visual function at 3 months. Of note, the patient's body mass index was 17 kg/m2Our case highlights the rare occurrence of papilloedema in GBS, reiterating the importance of performing funduscopy on patients with any neurological diagnosis. Early detection and prompt management of papilloedema can prevent permanent vision loss.

Quantifying the Relationship Between Unilateral Induced Metamorphopsia and Stereopsis Impairment.

Purpose: To investigate the relationship between unilateral metamorphopsia, characterized by visual distortions in one eye, and impaired stereopsis. Methods: Utilizing both near and distance measurements through advanced testing systems, including 4K smartphones and an active shutter three-dimensional system, we simulated varying degrees of unilateral metamorphopsia in 30 healthy young adults aged between 21 and 29 years. Two types of contour-based stereotest symbols, lines and squares, were developed. Distortions were classified into six distinct patterns, each further divided into eight grades of severity. Participants were tasked with identifying visual targets, and their stereothresholds were determined under different conditions of induced distortion. Stereopsis was measured within a range of 2.9 to 1.0 log arcsec, at 0.2 log arcsec intervals. Stereopsis changes under different distortion scenarios were analyzed using the generalized estimating equations, with a sequential Bonferroni adjustment applied for pairwise comparisons. Results: A direct and quantifiable correlation was observed between the severity of metamorphopsia and reductions in stereopsis. As the degree of visual distortion increased, notably in both frequency and amplitude, there was a corresponding decline in stereopsis. This relationship held true in both near and distance measurements of stereopsis. Statistical analyses further reinforced these findings, highlighting a significant detrimental effect of distortion components on stereoacuity. Conclusions: The findings highlight the clinical significance of understanding the interplay between unilateral metamorphopsia and stereopsis. Early interventions in conditions leading to metamorphopsia might be critical to maintaining optimal stereopsis.

Alterations of the alpha rhythm in visual snow syndrome: a case-control study.

BACKGROUND: Visual snow syndrome is a disorder characterized by the combination of typical perceptual disturbances. The clinical picture suggests an impairment of visual filtering mechanisms and might involve primary and secondary visual brain areas, as well as higher-order attentional networks. On the level of cortical oscillations, the alpha rhythm is a prominent EEG pattern that is involved in the prioritisation of visual information. It can be regarded as a correlate of inhibitory modulation within the visual network. METHODS: Twenty-one patients with visual snow syndrome were compared to 21 controls matched for age, sex, and migraine. We analysed the resting-state alpha rhythm by identifying the individual alpha peak frequency using a Fast Fourier Transform and then calculating the power spectral density around the individual alpha peak (+/- 1 Hz). We anticipated a reduced power spectral density in the alpha band over the primary visual cortex in participants with visual snow syndrome. RESULTS: There were no significant differences in the power spectral density in the alpha band over the occipital electrodes (O1 and O2), leading to the rejection of our primary hypothesis. However, the power spectral density in the alpha band was significantly reduced over temporal and parietal electrodes. There was also a trend towards increased individual alpha peak frequency in the subgroup of participants without comorbid migraine. CONCLUSIONS: Our main finding was a decreased power spectral density in the alpha band over parietal and temporal brain regions corresponding to areas of the secondary visual cortex. These findings complement previous functional and structural imaging data at a electrophysiological level. They underscore the involvement of higher-order visual brain areas, and potentially reflect a disturbance in inhibitory top-down modulation. The alpha rhythm alterations might represent a novel target for specific neuromodulation. TRIAL REGISTRATION: we preregistered the study before preprocessing and data analysis on the platform osf.org (DOI: https://doi.org/10.17605/OSF.IO/XPQHF , date of registration: November 19th 2022).

Development and validation of a questionnaire for assessing visual and auditory spatial localization abilities in dual sensory impairment.

Spatial localization is important for social interaction and safe mobility, and relies heavily on vision and hearing. While people with vision or hearing impairment compensate with their intact sense, people with dual sensory impairment (DSI) may require rehabilitation strategies that take both impairments into account. There is currently no tool for assessing the joint effect of vision and hearing impairment on spatial localization in this large and increasing population. To this end, we developed a novel Dual Sensory Spatial Localization Questionnaire (DS-SLQ) that consists of 35 everyday spatial localization tasks. The DS-SLQ asks participants about their difficulty completing different tasks using only vision or hearing, as well as the primary sense they rely on for each task. We administered the DS-SLQ to 104 participants with heterogenous vision and hearing status. Rasch analysis confirmed the psychometric validity of the DS-SLQ and the feasibility of comparing vision and hearing spatial abilities in a unified framework. Vision and hearing impairment were associated with decreased visual and auditory spatial abilities. Differences between vision and hearing abilities predicted overall sensory reliance patterns. In DSI rehabilitation, DS-SLQ may be useful for measuring vision and hearing spatial localization abilities and predicting the better sense for completing different spatial localization tasks.

Optic nerve head factors associated with initial central visual field defect in primary open-angle glaucoma.

We investigated optic nerve head factors associated with initial parafoveal scotoma (IPFS) in primary open-angle glaucoma. Eighty (80) patients with an IPFS and 84 patients with an initial nasal step (INS) were compared. Central retinal vascular trunk (CRVT) deviation from the Bruch's membrane opening (BMO) center was measured as a surrogate of lamina cribrosa (LC)/BMO offset, and its obliqueness was defined as the absolute value of angular deviation from the fovea-BMO axis. Proximity of retinal nerve fiber layer defect (RNFLD) was defined as the angular deviation of the inner RNFLD margin from the fovea-BMO axis. Microvasculature dropout (MvD) was defined as a focal sectoral capillary dropout with no visible microvascular network identified in the choroidal layer. Factors associated with IPFS, as compared with INS, were assessed using logistic regression analyses and conditional inference tree analysis. The IPFS group had more oblique CRVT offset (P < 0.001), RNFLD closer to the fovea (P < 0.001), more MvD (P < 0.001), and more LC defects (P < 0.001) compared to the INS group. In logistic regression analyses, obliqueness of CRVT offset (P = 0.002), RNFLD proximity (P < 0.001), and MvD (P = 0.001) were significant factors influencing the presence of IPFS. Conditional inference tree analysis showed that RNFLD closer to the fovea (P < 0.001) in the upper level, more oblique CRVT offset (P = 0.013) and presence of MvD (P = 0.001) in the lower level were associated with the probability of having IPFS. IPFS was associated with closer RNFLD location to the fovea when assessed from the BMO. Oblique LC/BMO offset may not only mask RNFLD proximity to the fovea due to a deviated funduscopic disc appearance, but also potentiate IPFS via focal LC defect and MvD.

Chemical eye injuries: a 10 year retrospective review of acute presentations and clinical outcomes in Auckland, New Zealand.

To assess the aetiologies, clinical characteristics, treatment regimens, and outcomes of acute chemical injuries treated at an emergency eye clinic. Retrospective, observational study of all cases of chemical eye injury that presented acutely to the Greenlane Clinical Centre in Auckland, New Zealand from 1 January 2012 through 31 December 2021. Patient demographics, activity at the time of injury, causative chemical, clinical characteristics of injury at presentation, severity (Dua) classification, admission and discharge best corrected visual acuity (BCVA), treatment regimen, time to epithelisation and number of follow-up appointments were recorded. In total, 1522 cases involving 1919 eyes were studied. The mean age was 40.6 ± 18.8 years and 65% were male. The majority of cases occurred at home (62%) and cleaning was the most common activity (38%). There were 1490 Grade I (98%), 22 Grade II (1.5%), 5 Grade III (0.3%), 1 Grade IV (0.07%), 0 Grade V, and 4 Grade VI (0.3%) cases. An epithelial defect was noted in 409 cases (26.9%), of which re-epithelialisation occurred within one week for 378 cases (92%) and within 30 days for 384 cases (94%). Moderate vision loss (BCVA ≤ 6/12) attributed to the injury occurred in 152 (10%), while severe vision loss (BCVA ≤ 6/60) occurred in 30 (2%). Lack of irrigation at the scene was associated with an increased risk of severe injury and longstanding visual impairment (p = 0.0001). Most acute chemical injuries are mild with good clinical outcomes. Although rare, severe injuries are associated with a lack of irrigation at the scene and worse visual outcomes.

Clinical sequencing of the retinitis pigmentosa gene RPGR in over 1,000 cases of vision loss.

RPGR pathogenic variants are the major cause of X-linked retinitis pigmentosa. Here, we report the results from 1,033 clinical DNA tests that included sequencing of RPGR. A total of 184 RPGR variants were identified: 78 pathogenic or likely pathogenic, 14 uncertain, and 92 likely benign or benign. Among the pathogenic and likely pathogenic variants, 23 were novel, and most were frameshift or nonsense mutations (87%) and enriched (67%) in RPGR exon 15 (ORF15). Identical pathogenic variants found in different families were largely on different haplotype backgrounds, indicating relatively frequent, recurrent RPGR mutations. None of the 16 mother/affected son pairs showed de novo mutations; all 16 mothers were heterozygous for the pathogenic variant. These last two observations support the occurrence of most RPGR mutations in the male germline.

Higher order aberrations in keratoconus .

INTRODUCTION: Keratoconus is a progressive disorder of the cornea that causes thinning (Sedaghat et al. in Sci Rep 11(1):11971, 2021), ectasia, and irregular astigmatism, resulting in poor visual acuity that cannot be corrected with standard sphero-cylindrical spectacle lenses. One feature distinguishing keratoconic corneas is ocular aberrations, manifesting up to five or six times the amount of higher-order aberrations than a normal, healthy eye. These aberrations can cause visual disturbances even at the very early stages of the disease. METHODS: In the past, a diagnosis was derived from clinical symptoms, but technological advances have revealed multiple pre-clinical features, allowing for the differentiation between keratoconic and normal eyes at a much earlier stage. These include anterior and posterior corneal surface elevations, the corneal pachymetry profile, corneal epithelial patterns, wavefront aberration metrics, and corneal biomechanics (Sedaghat et al. in Sci Rep 11(1):11971, 2021).This review discusses the aberrations associated with keratoconus, how to measure them, and treatment methods to minimize their negative influence. CONCLUSIONS: Early diagnosis can lead to early treatment and may allow for arresting progression, thereby improving the long-term prognosis. With the acceleration of refractive surgery, it is important to identify patients with keratoconus, as they are usually contraindicated for refractive surgery.

The Pseudotumor Cerebri Syndrome.

Pseudotumor cerebri syndrome is a syndrome of increased cerebrospinal fluid pressure without ventriculomegaly, mass lesion, or meningeal abnormality. It is either primary (idiopathic intracranial hypertension, IIH) or secondary. A secondary cause is unlikely when adhering to the diagnostic criteria. Permanent visual loss occurs if undetected or untreated, and the associated headaches may be debilitating. Fulminant disease may result in blindness despite aggressive treatment. This study addresses the diagnosis and management of IIH including new insights into the pathobiology of IIH, updates in therapeutics and causes of overdiagnosis.

Prevalence of computer vision syndrome: A systematic review and meta-analysis

Purpose This review aimed to estimate the prevalence of computer vision syndrome (CVS) in the general population and subgroups. Methods A search was conducted in the following the databases: PubMed, SCOPUS, EMBASE, and Web of Science until February 13, 2023. We included studies that assessed the prevalence of CVS in any population. The Joanna Briggs Institute's critical appraisal tool was used to evaluate the methodological quality. A meta-analysis of the prevalence of CVS was done using a random-effects model, assessing the sources of heterogeneity using subgroup and meta-regression analyses. Results A total of 103 cross-sectional studies with 66 577 participants were included. The prevalence of CVS was 69.0% (95% CI: 62.3 to 75.3; I2: 99.7%), ranging from 12.1 to 97.3% across studies. Point prevalence was higher in women than in men (71.4 vs. 61.8%), university students (76.1%), Africa (71.2%), Asia (69.9%), contact lens wearers (73.1% vs. 63.8%) in studies conducted before the COVID-19 pandemic (72.8%), and in those that did not use the CVS-Q questionnaire (75.4%). In meta-regression, using the CVS-Q scale was associated with a lower prevalence of CVS. Conclusion Seven out of ten people suffer from CVS. Preventive strategies and interventions are needed to decrease the prevalence of this condition which can affect productivity and quality of life. Future studies should standardize a definition of CVS. (AU)

Síndrome de nieve visual y su relación con la migraña / Visual snow syndrome and its relationship with migraine

Introducción El síndrome de nieve visual (SNV) es un trastorno del sistema nervioso central que implica la visión de forma constante de pequeños puntos blancos y negros en la totalidad del campo visual. Desarrollo El SNV puede presentarse desde la infancia hasta la tercera edad, siendo más frecuente en jóvenes y sin diferencia entre géneros. En sus criterios diagnósticos se incluye la presencia de nieve visual, pero también otros fenómenos visuales como palinopsia, fotofobia, nictalopía y otros fenómenos visuales persistentes. La fisiopatología del SNV es desconocida, pero se postulan como mecanismos la hiperexcitabilidad del córtex visual y una disfunción en el procesamiento visual de orden superior. La prevalencia de migraña en los pacientes con SNV es alta en comparación con la población general y cuando se presentan conjuntamente los síntomas son más severos. No se dispone de un tratamiento eficaz, pero el fármaco con mejores resultados es la lamotrigina, recomendándose únicamente en casos seleccionados con alta limitación funcional. Conclusiones El síndrome de nieve visual es una entidad poco conocida e infradiagnosticada, pero el creciente número de investigaciones durante los últimos años ha permitido definir unos criterios diagnósticos y acercarnos a su fisiopatología. Es una entidad íntimamente relacionada con la migraña, con solapamiento de síntomas y probablemente mecanismos fisiopatológicos comunes. (AU)

Introduction Visual snow syndrome (VSS) is a central nervous system disorder that consists of the constant perception of small black and white dots throughout the entire visual field. Development VSS can present from infancy to old age, with greater prevalence in the young population, and shows no difference between sexes. The diagnostic criteria include the presence of visual snow and such other visual phenomena as palinopsia, photophobia, nyctalopia, and other persistent visual phenomena. The pathophysiology of VSS is unknown, but hyperexcitability of the visual cortex and a dysfunction in higher-order visual processing are postulated as potential mechanisms. The prevalence of migraine among patients with VSS is high, compared to the general population, and symptoms are more severe in patients presenting both conditions. No effective treatment is available, but the drug with the best results is lamotrigine, which is recommended only in selected cases with severe functional limitation. Conclusions VSS is a little-known and underdiagnosed entity, but the increasing number of studies in recent years has made it possible to establish diagnostic criteria and begin studying its pathophysiology. This entity is closely related to migraine, with overlapping symptoms and probably shared pathophysiological mechanisms. (AU)

Síndrome de Alicia en el País de las Maravillas secundario a infección por SARS-CoV-2 / Alice in Wonderland syndrome in SARS-CoV-2 infection

El síndrome de Alicia en el País de las Maravillas es un conjunto de trastornos complejos de la percepción visual con múltiples etiologías, siendo las infecciones las más frecuentes en Pediatría. Es un cuadro poco frecuente, pero es importante conocerlo por su carácter generalmente autolimitado. Presentamos el caso de un niño de 11 años que, a las 48 horas de infección por SARS-CoV-2, inició distorsión visual de la forma corporal, micropsias y macropsias. Se descartaron otras causas orgánicas, manteniéndose actitud expectante dada su evolución benigna. Se destaca la aparición de este síndrome en contexto clínico de infección por SARS-CoV-2, no habiendo sido descrita hasta ahora su asociación en la literatura. (AU)

The Alice in Wonderland syndrome is defined by the presence of visual perception disorders with several etiologies, being infectious the most frequent in pediatrics. It is a rare clinical condition, but it is important to recognize it because of the generally self-limited nature. We present an 11 years old child who presented visual body distortions, micropsia and macropsia 48 after SARS-CoV-2 infection. Other organic causes were discarded, maintaining an expectant treatment because of its benign evolution. It is noteworthy that this syndrome associated with SARS-CoV-2 infection has not yet been published. (AU)

Knowing me, knowing you-A study on top-down requirements for compensatory scanning in drivers with homonymous visual field loss.

OBJECTIVE: It is currently still unknown why some drivers with visual field loss can compensate well for their visual impairment while others adopt ineffective strategies. This paper contributes to the methodological investigation of the associated top-down mechanisms and aims at validating a theoretical model on the requirements for successful compensation among drivers with homonymous visual field loss. METHODS: A driving simulator study was conducted with eight participants with homonymous visual field loss and eight participants with normal vision. Participants drove through an urban surrounding and experienced a baseline scenario and scenarios with visual precursors indicating increased likelihoods of crossing hazards. Novel measures for the assessment of the mental model of their visual abilities, the mental model of the driving scene and the perceived attention demand were developed and used to investigate the top-down mechanisms behind attention allocation and hazard avoidance. RESULTS: Participants with an overestimation of their visual field size tended to prioritize their seeing side over their blind side both in subjective and objective measures. The mental model of the driving scene showed close relations to the subjective and actual attention allocation. While participants with homonymous visual field loss were less anticipatory in their usage of the visual precursors and showed poorer performances compared to participants with normal vision, the results indicate a stronger reliance on top-down mechanism for drivers with visual impairments. A subjective focus on the seeing side or on near peripheries more frequently led to bad performances in terms of collisions with crossing cyclists. CONCLUSION: The study yielded promising indicators for the potential of novel measures to elucidate top-down mechanisms in drivers with homonymous visual field loss. Furthermore, the results largely support the model of requirements for successful compensatory scanning. The findings highlight the importance of individualized interventions and driver assistance systems tailored to address these mechanisms.

Subthreshold micropulse laser therapy for early postoperative macular thickening following surgical removal of epiretinal membrane.

BACKGROUND: This study aimed to investigate the functional and anatomical outcomes of subthreshold micropulse laser (SMPL) therapy in eyes with early postoperative macular thickening after idiopathic epiretinal membrane (iERM) removal. METHODS: This was a prospective and interventional study. Forty-eight eyes from 48 patients with macular edema at 1 month after iERM removal were randomly divided into two groups. Patients in the SMPL group (n = 24) received SMPL therapy while no special intervention was used for the observation group (n = 24). Baseline demographic data and clinical findings before and at 1 and 3 months after SMPL treatment or observation, including best-corrected visual acuity (BCVA) and the changes in central subfield thickness (CST) and average macular thickness (AMT), were analyzed. RESULTS: An improvement in BCVA with a decrease in CST and AMT from baseline to the 3-month follow-ups were observed in both SMPL and observation groups. No significant difference in BCVA was observed between the SMPL group and observation group either in the 1-month (0.26 [0.15, 0.52] vs. 0.26 [0.15, 0.39], P = 0.852) or the 3-month (0.15 [0.10, 0.30] vs. 0.23 [0.15, 0.30], P = 0.329) follow-up. There was a greater reduction in CST in the SMPL group versus observation group between baseline and the 3-month follow-up (-77.8 ± 72.3 µm vs. -45.0 ± 46.9 µm, P = 0.049). The alteration in AMT did not differ between the two groups in either 1-month (-16.5 ± 20.1 µm vs. -19.7 ± 16.3 µm, P = 0.547) or 3-month (-36.9 ± 26.9 µm vs. -34.0 ± 20.1 µm, P = 0.678) follow-up. CONCLUSIONS: SMPL therapy led to a significant decrease in CST at the 3-month follow-up while did not significantly improve the visual acuity in patients with postoperative macular thickening following iERM surgery. TRIAL REGISTRATION: The study was registered on Aug 27, 2020 (Trial Registration Number: ChiCTR 2000037227).

Hearing and vision impairment and social isolation over 8 years in community-dwelling older adults.

BACKGROUND: Little is known about the long-term impact of hearing and vision impairment on social isolation. This study quantifies the association between hearing, vision, and concurrent hearing and vision impairment (dual sensory impairment) and social isolation over 8 years among older adults. METHODS: Data were from the National Health and Aging Trends Study (NHATS), a cohort study (2011 - 2019) of U.S. Medicare beneficiaries aged 65 years and older. Social isolation was measured by a binary indicator incorporating four domains: living arrangement, core discussion network size, religious attendance, and social participation. Hearing, vision, and dual sensory impairments were measured by self-report and modeled categorically (no impairment [ref.], hearing impairment only, vision impairment only, dual sensory impairment). Associations between sensory impairments and odds of social isolation over 8 years were assessed using multivariate generalized logistic mixed models and adjusted for demographic and health characteristics. RESULTS: Among 5,552 participants, 18.9% self-reported hearing impairment, 4.8% self-reported vision impairment, and 2.3% self-reported dual sensory impairment. Over 8 years, hearing impairment only was associated with 28% greater odds of social isolation. Participants with hearing impairment only were more likely to live alone and have limited social participation. CONCLUSION: Greater clinical awareness of hearing impairment as a risk factor for social isolation can increase opportunities to identify and aid older adults who may benefit from resources and interventions to increase social connection and mitigate social isolation.

Understanding and identifying visual field progression.

Detecting deterioration of visual field sensitivity measurements is important for the diagnosis and management of glaucoma. This review surveys the current methods for assessing progression that are implemented in clinical devices, which have been used in clinical trials, alongside more recent advances proposed in the literature. Advice is also offered to clinicians on what they can do to improve the collection of perimetric data to help analytical progression methods more accurately predict change. This advice includes a discussion of how frequently visual field testing should be undertaken, with a view towards future developments, such as digital healthcare outside the standard clinical setting and more personalised approaches to perimetry.

Paraneoplastic vision loss.

Paraneoplastic vision loss, which represents a small percentage of paraneoplastic neurologic syndromes, can be a blinding disease. Presenting visual symptoms are variable, making diagnosis challenging. History of the presenting illness, ocular examination, and utilization of various modalities, such as automated perimetry, ocular coherence tomography, and electroretinogram allow for localization of vision loss to the optic nerves or retina, guiding in diagnosis and management. Paraneoplastic vision loss is often painless, bilateral, and subacute, and accompanies other neurologic symptoms but can be the first presenting symptom. Paraneoplastic optic neuropathy has been described in association with several antibodies, but most commonly anti-CRMP5. Cancer-associated retinopathy is the most common paraneoplastic autoimmune retinopathy; however, melanoma-associated retinopathy and bilateral diffuse uveal melanocytic proliferation have also been described to be associated with a paraneoplastic process affecting the retina. Paraneoplastic visual loss is an expanding field and advances in research have improved phenotypic characterization; however, further work is needed to identify more reliable biomarkers of disease and to better understand the underlying mechanisms and management.