Journal of Neuro-Ophthalmology最新文献

{"title":"Optic Atrophy in Wolfram Syndrome Type 1: A Retrospective Analysis of Visual Outcomes and Biomarker Correlates.","authors":"Bradley J McNeely, Abby F Tang, Fumihiko Urano, Nicholas R Levergood, Melissa W Ko, Devin D Mackay, Gregory P Van Stavern, Yunshuo Tang","doi":"10.1097/WNO.0000000000002451","DOIUrl":"10.1097/WNO.0000000000002451","url":null,"abstract":"<p><strong>Background: </strong>Wolfram syndrome type 1 (WS1) is a rare autosomal recessive disorder classically associated with diabetes mellitus (DM) and optic atrophy (OA). We aimed to characterize OA in WS1 and evaluate optical coherence tomography (OCT) and genetic biomarkers as tools for disease monitoring and prognostication.</p><p><strong>Methods: </strong>We conducted a retrospective chart review of genetically confirmed patients with WS1 seen at Washington University or Indiana University neuro-ophthalmology clinics between July 2017 and 2024. Data included demographics, clinical history, best corrected visual acuity (BCVA), OCT retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) thickness, and WFS1 mutation severity scores. Linear regression analyses assessed correlations between BCVA and clinical, structural, and genetic variables.</p><p><strong>Results: </strong>Thirty-six patients (22 women, 14 men; median age 20 years) were identified. Median mutation severity score was 3.5. Vision loss occurred in 31 patients; in 3 patients it was the only major symptom, in 5 patients it preceded DM, and in 6 patients it occurred without DM. Mean and median BCVA were 20/125 and 20/80, respectively. BCVA correlated inversely with RNFL thickness ( P = 0.0017, R 2 = 0.14), GCC thickness ( P = 0.0018, R 2 = 0.29), and mutation severity score ( P = 0.031, R 2 = 0.14).</p><p><strong>Conclusions: </strong>OA was the most common and sometimes earliest WS1 manifestation. Correlations between BCVA, OCT metrics, and mutation severity score support their potential value as biomarkers and prognostic tools. Findings also support considering genetic screening for WFS1 mutations in patients presenting with otherwise unexplained OA.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"329-334"},"PeriodicalIF":2.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147499195","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The Association Between Anemia and Pseudotumor Cerebri.","authors":"Alison B Gibbons, Ximin Li, Amanda D Henderson","doi":"10.1097/WNO.0000000000002469","DOIUrl":"10.1097/WNO.0000000000002469","url":null,"abstract":"<p><strong>Background: </strong>Contemporary literature suggests that anemia may be a risk factor for the development of pseudotumor cerebri (PTC) and associated with poorer visual outcomes. However, literature describing the association is limited, and whether laboratory testing for anemia should be done during a PTC work-up is frequently debated among neuro-ophthalmologists.</p><p><strong>Methods: </strong>International Classification of Diseases-10 codes were used to identify patients with PTC from the SOURCE database. Patients with PTC were matched for age, sex, and race with controls. Relative rates of anemia were compared between groups. A subgroup analysis compared demographic factors and outcomes for PTC patients with and without anemia.</p><p><strong>Results: </strong>Two-thousand two-hundred forty patients with PTC and 2,240 age-, race-, body mass index-, and sex-matched controls from a national, multicentered database were included in the study. Rates of anemia did not differ between PTC and non-PTC groups (23.4% vs 22.3%, P = 0.393). Among patients with PTC, anemia was associated with older age at onset (38.0 ± 14.1 vs 36.1 ± 13.6 [27.0-45.0] years, P = 0.013), male sex (16.2% vs 10.7%, P = 0.017), and lower visual acuity at presentation ( P < 0.001), but final visual acuities were similar when controlling for presenting acuity ( P = 0.123).</p><p><strong>Conclusions: </strong>Using the largest sample of patients with PTC and matched controls to date, anemia was not associated with PTC diagnosis. Among patients with PTC, anemia was associated with demographic features atypical for PTC.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"390-395"},"PeriodicalIF":2.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147773995","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Racial and Ethnic Disparities in Severe Ischemic Manifestations and Tocilizumab Use Among Patients With Giant Cell Arteritis: A Nationwide Retrospective Cohort Study Using TriNetX.","authors":"Daniel M Markowitz, Jose S Pulido, Jirat Nimworaphan, John J Chen, Robert C Sergott","doi":"10.1097/WNO.0000000000002458","DOIUrl":"10.1097/WNO.0000000000002458","url":null,"abstract":"<p><strong>Background: </strong>Giant cell arteritis (GCA) has traditionally been recognized as more common in White populations, but there is limited understanding of its epidemiology and severe ischemic manifestations in other racial and ethnic groups. This study investigated racial and ethnic differences in GCA ischemic manifestations and tocilizumab use.</p><p><strong>Methods: </strong>We conducted a retrospective cohort study using the TriNetX U.S. Collaborative Network. Age- and sex-matched cohorts of non-Hispanic White (hereafter referred to as White), non-Hispanic Black or African American (Black), non-Hispanic Asian (Asian), and Hispanic or Latino (Hispanic) patients diagnosed with GCA were analyzed for differences in severe clinical manifestations within 1 month of GCA diagnosis (ischemic optic neuropathy, blindness/low vision, central retinal artery occlusion, stroke, aortic aneurysm and dissection, acute myocardial infarction) and tocilizumab use within 1 year.</p><p><strong>Results: </strong>Compared with White patients, Black patients had lower odds of ischemic optic neuropathy (odds ratio [OR]: 0.46, 95% confidence interval [CI]: 0.33-0.65), higher stroke risk (OR: 1.68, 95% CI: 1.16-2.43), and were less likely to receive tocilizumab (OR: 0.44, 95% CI: 0.33-0.59). Hispanic patients had lower odds of ischemic optic neuropathy (OR: 0.61, 95% CI: 0.37-0.98) and receiving tocilizumab (OR: 0.51, 95% CI: 0.34-0.74) compared with White patients. Additionally, compared with White patients, Asian patients were less likely to be diagnosed with blindness/low vision (OR: 0.45, 95% CI: 0.27-0.76), had similar odds of ischemic optic neuropathy, and had no significant differences in tocilizumab use.</p><p><strong>Conclusions: </strong>There may be significant differences in severe ischemic manifestations of GCA among non-White patients compared with White patients. There also appear to be differences in treatment with tocilizumab between Black and White patients and between Hispanic and White patients, but not between Asian and White patients.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"408-413"},"PeriodicalIF":2.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147504106","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bilateral Cavernous Sinus Thrombosis in a Patient With Sinonasal Mucosal Melanoma With Orbital Extension.","authors":"Zahra A Markatia, Lucy I Mudie, George A Villatoro, Michael T Yen","doi":"10.1097/WNO.0000000000002465","DOIUrl":"10.1097/WNO.0000000000002465","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"e85-e87"},"PeriodicalIF":2.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147504127","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Quantification of Mitochondrial Stress in Nonarteritic Anterior Ischemic Optic Neuropathy Using Flavoprotein Fluorescence Imaging.","authors":"Rishita Rama Pujari, Yulan Ren, Jamie Zhang, Anas Alkhabaz, Hugo Wu, Collin A Rich, Yaping Joyce Liao","doi":"10.1097/WNO.0000000000002478","DOIUrl":"10.1097/WNO.0000000000002478","url":null,"abstract":"<p><strong>Background: </strong>Nonarteritic anterior ischemic optic neuropathy (NAION) or optic nerve stroke is the most common acute optic neuropathy in patients older than 50 years and results in severe vision loss. Endogenous flavoprotein fluorescence (FPF) imaging is a novel imaging modality to quantify axonal mitochondrial stress in the living central nervous system. We investigated the evolution of optic disc and macular FPF, and other ophthalmic measurements in the first 6 months after NAION onset.</p><p><strong>Methods: </strong>We retrospectively analyzed 10 patients (15 eyes) with acute NAION and performed serial assessments of visual function, optic disc FPF, macular FPF, and spectral-domain optical coherence tomography for 6 months. Longitudinal trends in optic disc FPF and peripapillary retinal nerve fiber layer (pRNFL) were modeled using generalized additive models with random effects to account for repeated measures. Linear mixed-effects models were used to assess changes from baseline to the 2-4 and 5-7-month follow-up periods.</p><p><strong>Results: </strong>In the acute phase (<45 days), pRNFL thickness was elevated, consistent with optic disc edema, while optic disc FPF values were within the normal range. Over time, optic disc FPF increased, and pRNFL thickness decreased, with relative stabilization after approximately 3 months, whereas visual acuity remained stable. Linear mixed-effects analysis of eyes with baseline imaging ≤45 days and follow-up at 2-4 months (5 eyes) and/or 5-7 months (6 eyes) demonstrated a significant increase in optic disc FPF (Δ = 5.77 dB, P < 0.001) and a significant decrease in pRNFL thickness by 2-4 months (Δ = -153.02 µm, P < 0.001), with no significant changes from the 2-4-month to the 5-7-month period.</p><p><strong>Conclusions: </strong>Optic disc FPF is normal in acute NAION because of masking by optic disc edema. As optic disc edema resolves, optic disc FPF score increases, reaching a plateau at around 3 months after onset, consistent with increased axonal metabolic stress.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"385-389"},"PeriodicalIF":2.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148456129","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Scoping Review of Pharmacologic and Nonpharmacologic Interventions to Treat Chronic Ocular Pain.","authors":"Lindsey B De Lott, Noreen Khan, Mana Yacim, Harini Adivikolanu, Anne Shea, Kate Saylor, Kathleen Digre, Anat Galor","doi":"10.1097/WNO.0000000000002510","DOIUrl":"10.1097/WNO.0000000000002510","url":null,"abstract":"<p><strong>Background: </strong>Chronic ocular pain (COP) is disabling and a common reason for referral to neuro-ophthalmologists. Many patients with COP suffer from amplified or dysfunctional pain processing in the peripheral nervous system (PNS) or central nervous system (CNS), rather than a problem with the eye itself. The extent of and evidence for therapeutic interventions targeting pain in the PNS and CNS among patients suffering from COP are unknown.</p><p><strong>Evidence acquisition: </strong>We performed a search of Ovid MEDLINE, Embase.com, Scopus.com, and ClinicalTrials.gov. We limited our search to English-language articles published since 2015. We included observational and experimental studies of pharmacologic and nonpharmacologic interventions (e.g., vagal nerve stimulation) used in adults 18 years or older with COP for at least 3 months. Studies were included if the impact on pain intensity, functioning, or quality of life was measured as primary, secondary, or exploratory outcomes. All articles were independently screened by 2 team members for inclusion. Data extraction was performed independently by 2 team members. Any disagreements during the screening and data extraction processes were adjudicated by a third team member and discussed as a group.</p><p><strong>Results: </strong>Our search identified 9,656 studies from 2015 to 2026. Of those, 21 met our inclusion/exclusion criteria. The study designs were mostly retrospective: 5 case reports, 9 observational, and 1 case-control study; 3 prospective studies were nonrandomized, observational studies. Three studies were randomized clinical trials, but one did not have published results. Most of the studies (67%, n = 14) were on pharmacologic interventions including all 3 completed prospective studies. Pharmacologic interventions included gabapentin, cryosim-3 applied to the eyelids, autologous serum tears, botulinum toxin Type A injections, and nerve blocks. The 6 studies including nonpharmacologic interventions used transcutaneous electrical nerve stimulation, trigeminal ganglion stimulation, and pulsed radiofrequency procedures. The 3 randomized controlled clinical trials were of transcutaneous electrical nerve stimulation, topical ocular cooling device, and topical OK-101 ophthalmic solutions at different concentrations. The most common pain measures used across the studies were pain intensity scales (range 0-10), such as the numeric rating scale. Improvements in COP were reported for most studies. The reporting of adverse events occurred in 8 studies (43%).</p><p><strong>Conclusions: </strong>Although COP is a common symptom, we found few studies on pharmacologic and nonpharmacologic treatment interventions with a few randomized controlled trials. Rigorous studies of treatment interventions aimed at reducing COP are needed. We provide recommendations for both the conduct of future studies and the current approach to COP treatment.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":"46 3","pages":"301-316"},"PeriodicalIF":2.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13488495/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148794390","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Retinal Impact of Flow-Diverting Stents in the Treatment of Carotid-Ophthalmic Artery Aneurysms.","authors":"Claire Simon, Valère Barrot, Geoffroy Mortemousque, Sophie Catanese, Grégoire Boulouis, Christine Serrhini, Marie-Laure Provost, Pierre-Jean Pisella, Richard Bibi, Kevin Janot, Raoul Kanav Khanna","doi":"10.1097/WNO.0000000000002396","DOIUrl":"10.1097/WNO.0000000000002396","url":null,"abstract":"<p><strong>Background: </strong>The aim of this study was to evaluate the retinal safety profile of flow-diverting stents in the treatment of carotid-ophthalmic aneurysms with ophthalmic artery coverage.</p><p><strong>Methods: </strong>In this cohort study, patients treated with a flow-diverting stent for carotid-ophthalmic aneurysm were prospectively included from January 2021 to January 2023. A systematic ophthalmological examination was performed before and at 15 days and at 6 months after the procedure. We compared the retinal thickness and the vascular density of the retinal capillary plexuses using optical coherence tomography (OCT).</p><p><strong>Results: </strong>Twenty-five patients were included (median age: 52.3 years, range: 36.6 to 76.4; women: 92.4%). Postoperative retinal events were reported in 24% patients (cotton wool spots in 6 patients [24%], retinal hemorrhages in 3 patients [12.5%]), with no functional repercussions detected on examination. The macular vascular framework assessed by OCT-angiography remained unchanged postoperatively. We observed immediate postoperative foveal thinning and temporal papillary optic fiber thickening which was not clinically significant. After 6 months, all clinical events had resolved, and both macular and peripapillary OCT had returned to normal.</p><p><strong>Conclusions: </strong>This study provides evidence for retinal safety when using flow-diverting stents covering the ophthalmic artery. Fundus changes may occur in the postoperative course, but without impact on visual function.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"317-323"},"PeriodicalIF":2.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145541042","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Between a Chiasm and a Hard Place.","authors":"Sofia A John, Sara Krachmalnick, Marianna B Ruzinova, Maggie E Pohlmeier, Francisco J Garcia-Ferrer, Gregory P Van Stavern, Leanne Stunkel","doi":"10.1097/WNO.0000000000002445","DOIUrl":"10.1097/WNO.0000000000002445","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"e65-e68"},"PeriodicalIF":2.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147504082","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Bilateral Compressive Optic Neuropathy Secondary to Severe Vitamin A Deficiency in an Adolescent With Autism Spectrum Disorder.","authors":"Priscila Correia de Farias, Matheus Andrighetti Rossi, Lucas Almeida Linhares, Daniel Gurgel Fernandes Távora, Natália Nogueira Carvão Aguiar Valle Rossi","doi":"10.1097/WNO.0000000000002470","DOIUrl":"10.1097/WNO.0000000000002470","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"e88-e90"},"PeriodicalIF":2.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147698862","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Intracanalicular Optic Nerve Sheath Schwannoma Masquerading as Atypical Optic Neuritis.","authors":"Shikha T Bassi, Krishnakumar Subramanian, Olma V Noronha, Roopesh V Kumar","doi":"10.1097/WNO.0000000000002460","DOIUrl":"10.1097/WNO.0000000000002460","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"e82-e84"},"PeriodicalIF":2.1,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147698952","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}