Journal of Neuro-Ophthalmology最新文献

{"title":"Advancing Optical Coherence Tomography Diagnostic Capabilities: Machine Learning Approaches to Detect Autoimmune Inflammatory Diseases.","authors":"Rachel C Kenney, Thomas A Flagiello, Anitha D' Cunha, Suhan Alva, Scott N Grossman, Frederike C Oertel, Friedemann Paul, Kurt G Schilling, Laura J Balcer, Steven L Galetta, Lekha Pandit","doi":"10.1097/WNO.0000000000002322","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002322","url":null,"abstract":"<p><strong>Background: </strong>In many parts of the world including India, the prevalence of autoimmune inflammatory diseases such as neuromyelitis optica spectrum disorders (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and multiple sclerosis (MS) is rising. A diagnosis is often delayed due to insufficient diagnostic tools. Machine learning (ML) models have accurately differentiated eyes of patients with MS from those of healthy controls (HCs) using optical coherence tomography (OCT)-based retinal images. Examining OCT characteristics may allow for early differentiation of these conditions. The objective of this study was to determine feasibility of ML analyses to distinguish between patients with different autoimmune inflammatory diseases, other ocular diseases, and HCs based on OCT measurements of the peripapillary retinal nerve fiber layer (pRNFL), ganglion cell-inner plexiform layer (GCIPL), and inner nuclear layers (INLs).</p><p><strong>Methods: </strong>Eyes of people with MS (n = 99 patients), NMOSD (n = 40), MOGAD (n = 74), other ocular diseases (OTHER, n = 16), and HCs (n = 54) from the Mangalore Demyelinating Disease Registry were included. Support vector machine (SVM) classification models incorporating age, pRNFL, GCIPL, and INL were performed. Data were split into training (70%) and testing (30%) data and accounted for within-patient correlations. Cross-validation was used in training to choose the best parameters for the SVM model. Accuracy and area under receiver operating characteristic curves (AUROCs) were used to assess model performance.</p><p><strong>Results: </strong>The SVM models distinguished between eyes of patients with each condition (i.e., MOGAD vs NMOSD, NMOSD vs HC, MS vs OTHER, etc) with strong discriminatory power demonstrated from the AUROCs for these comparisons ranging from 0.81 to 1.00. These models also performed with moderate to high accuracy, ranging from 0.66 to 0.81, with the exception of the MS vs NMOSD comparison, which had an accuracy of 0.53.</p><p><strong>Conclusions: </strong>ML models are useful for distinguishing between autoimmune inflammatory diseases and for distinguishing these from HCs and other ocular diseases based on OCT measures. This study lays the groundwork for future deep learning studies that use analyses of raw OCT images for identifying eyes of patients with such disorders and other etiologies of optic neuropathy.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-02-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143255472","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":"Recurrent Idiopathic Intracranial Hypertension-Related Papilledema After Abrupt Discontinuation of Semaglutide.","authors":"Mark J Phillips, Kimberly K Gokoffski","doi":"10.1097/WNO.0000000000002293","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002293","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143189319","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":"Prevalence and Clinical Associations of Peripapillary Hyperreflective Ovoid Mass-like Structures in Craniosynostosis.","authors":"Jacqueline G Jeon-Chapman, Tais Estrela, David Zurakowski, Yoon-Hee Chang, Linda R Dagi, Ryan A Gise","doi":"10.1097/WNO.0000000000002315","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002315","url":null,"abstract":"<p><strong>Background: </strong>Patients with craniosynostosis are at high risk of developing elevated intracranial pressure (ICP) causing papilledema and secondary optic atrophy. Diagnosing and monitoring optic neuropathy is challenging because of multiple causes of vision loss including exposure keratopathy, amblyopia, and cognitive delays that limit examination. Peripapillary hyperreflective ovoid mass-like structures (PHOMS) are an optical coherence tomography (OCT) finding reported in association with papilledema and optic neuropathy. We investigated the prevalence of PHOMS in patients with syndromic craniosynostosis and the relationship with known risk factors of optic neuropathy.</p><p><strong>Methods: </strong>This was a cross-sectional retrospective study was performed at Boston Children's Hospital and included 118 eyes (60 patients) with syndromic craniosynostosis that had at least one good quality optic nerve OCT volumetry scan between January 2010 and December 2023. Testing was reviewed by 2 neuro-ophthalmologists to determine the presence of PHOMS. Information collected included demographics, and clinical course including possible Chiari malformation, obstructive sleep apnea (OSA), history of elevated ICP, best-corrected visual acuity (VA), spherical equivalent (SE), retinal nerve fiber layer thickness, macular ganglion cell layer volume, and funduscopic examination. Our primary outcome measure was presence of PHOMS, and secondary outcome measure was the relationship between clinical characteristics and the presence of PHOMS.</p><p><strong>Results: </strong>Mean age at imaged OCT was 9.8 ± 5.2 years and 41/60 (68.3%) patients were female. The prevalence of PHOMS was 17/60 patients (28.3%) or 26/118 eyes (22.0%), higher than previously reported in children without craniosynostosis (P < 0.001). PHOMS were significantly associated with a history of elevated ICP (odds ratio [OR] 14.4, 95% CI: 1.9-111.0, P < 0.001) and with concurrent papilledema (OR 40.4, 95% CI: 6.6-248.0, P < 0.001). OSA, Chiari malformation, best-corrected VA, craniosynostosis variant, and SE were not independently associated with PHOMS.</p><p><strong>Conclusions: </strong>Eyes in children with craniosynostosis had a higher prevalence of PHOMS than reported in children without craniosynostosis. PHOMS were significantly more common with a history of elevated ICP and with concurrent papilledema. PHOMS may serve as a clinically useful indicator of optic neuropathy, and of recurrence of papilledema in patients with craniosynostosis and in other populations characterized by multiple sources of vision loss and cognitive changes that limit evaluation.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143066057","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":"Literature Commentary.","authors":"","doi":"10.1097/WNO.0000000000002321","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002321","url":null,"abstract":"<p><p>In this issue of JNO, Drs. Deborah I. Friedman and Mark L. Moster discuss the following 4 articles: Al Kasab S, Almallouhi E, Shu L, Kicielinski KP, Salehi Omran S, Liebeskind DS, Zubair AS, Vedovati MC, Paciaroni M, Antonenko K, Heldner MR, de Havenon A, Henninger N, Yaghi S. Outcomes and recurrence rates among patients with provoked and cryptogenic cerebral venous thrombosis: analysis of the ACTION CVT. Neurol Clin Pract. 2025;15:e200381 [Epub ahead of print].Kwon YN, Kim B, Kim JS, Park KS, Seo DY, Kim H, Lee EJ, Lim YM, Ju H, Chung YH, Min JH, Nam TS, Kim S, Sohn E, Shin KJ, Seok JM, Kim S, Bae JS, Lee S, Oh SI, Jung YJ, Park J, Kim SH, Kim KH, Kim HJ, Jung JH, Kim SJ, Kim SW, Jang MJ, Sung JJ, Waters P, Shin HY, Kim SM. Time to treat first acute attack of myelin oligodendrocyte glycoprotein antibody-associated disease. JAMA Neurol. 2024;81:1073-1084.Akosman S, Li R, Asahi M, Kwon B, Dossantos J, Tavakoli M, Chen JJ. Trends in plasma exchange use in optic neuritis hospitalizations in the United States. Ophthalmology. 2024;131:1207-1214.Chou CC, Pan SY, Sheen YJ, Lin JF, Lin CH, Lin HJ, Wang IJ, Weng CH. Association between semaglutide and non-arteritic anterior ischemic optic neuropathy: a multinational population-based real-world study. Ophthalmology. 2024:S0161-6420(24)00685-7 [Epub ahead of print].</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143066067","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":"Isla Williams, MD, MBBS, DO, FRACP, FRCPE (1934-2024).","authors":"Christian J Lueck, Owen B White, Celia S Chen, Clare L Fraser","doi":"10.1097/WNO.0000000000002308","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002308","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143059275","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":"Ocular Torsional Deviation in a Unilateral Paramedian Thalamo-Mesencephalic Infarction.","authors":"Tom Buelens, Ziyu Wang, Mihaela-Félicia Topciu, François Willermain","doi":"10.1097/WNO.0000000000002310","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002310","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143023503","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 Great Conversation With Alfredo Sadun.","authors":"Rachel A Calix, George T Park, Kathleen B Digre","doi":"10.1097/WNO.0000000000002323","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002323","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143022963","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":"When We Make Errors and Do Harm: A Narrative Review of Second Victim Syndrome and Implications for Neuro-Ophthalmologists.","authors":"David Rabinovitch, Lindsey B Delott, Edward Margolin","doi":"10.1097/WNO.0000000000002319","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002319","url":null,"abstract":"<p><strong>Background: </strong>In the aftermath of an adverse event, the first priority is to provide care for the patient, known as the first victim. However, the experiences of healthcare professionals (HCPs) involved in these events, known as \"second victims\", have been largely overlooked. This review aims to consolidate existing knowledge on second victim syndrome (SVS), explore its unique implications for neuro-ophthalmologists, and suggest support strategies to increase awareness and meet the needs of affected colleagues.</p><p><strong>Evidence acquisition: </strong>A comprehensive review of the literature was conducted using PubMed, analyzing peer-reviewed articles and reports on SVS across medical specialties. The review focused on the prevalence, risk factors, outcomes, and strategies for addressing SVS at both the individual and system-wide levels.</p><p><strong>Results: </strong>SVS is alarmingly prevalent among HCPs, with estimates ranging from 10% to 43%, and nearly half of all HCPs are expected to experience SVS at least once in their careers. Although direct evidence is lacking, neuro-ophthalmologists may be particularly vulnerable due to the complexity of their patients, frequent diagnostic challenges, and the risk of poor outcomes. Effective coping strategies include peer support at the institutional level, with an urgent need for healthcare systems to transition toward a \"Just Culture\" that fosters openness and collective learning.</p><p><strong>Conclusions: </strong>Neuro-ophthalmologists are likely among several specialty fields suffering in silence following medical errors. To reduce these errors and optimize patient care, we must focus on enhancing support for the physicians involved. Sustainable improvements in healthcare require both targeted clinician support and comprehensive system-level changes to foster resilience and improve quality of care. Key efforts should include formal peer support programs, targeted educational initiatives, and a deliberate shift toward a \"Just Culture.\" These initiatives are essential for fostering clinician resilience, promoting effective recovery, and ultimately improving the broader healthcare system and quality of care.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-01-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143023504","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":"Observational Characterization of the Retreatment Course of Patients With Thyroid Eye Disease.","authors":"Yuanyuan Chen, Taylor Linaburg, Sarah Wang, Gibran Merchant, Tejus Pradeep, Patrick Anthony Augello, Gui-Shuang Ying, César A Briceño, Madhura A Tamhankar","doi":"10.1097/WNO.0000000000002280","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002280","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;To characterize the retreatment course of patients with thyroid eye disease (TED), who had reactivation after initial therapy with teprotumumab.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;This was a single-center longitudinal cohort study of patients who received an initial course of teprotumumab for active TED and were followed for at least 6 months. Reactivation was defined as the increase of proptosis of 2 mm or more or an increase in Clinical Activity Score (CAS) of two points or more, as adapted from the Optic-X study. Data collection included patient age, sex, smoking status, history of thyroidectomy or radioactive iodine, proptosis measurements, clinical activity score (CAS) before initial infusion of teprotumumab, time interval to reactivation, diplopia assessment by the Bahn-Gorman scale, CAS at the time of reactivation, and CAS and proptosis measurements after completion of retreatment and retreatment modalities, including clinical monitoring, corticosteroids, teprotumumab, and/or surgery. Among the reactivated cohort, the treatment response of patients who received a second course of teprotumumab was compared with patients who were treated with intravenous (IV) steroids.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Twenty-six percent (11/42) of patients experienced reactivation of TED with an average time to reactivation of 9 (SD:5) months (range: 2-20 months), average CAS at reactivation of 4 (SD:1) (range: 3-7), and average increase in proptosis of 3 (SD:1) mm (range: 2-6 mm). Of the 11 patients who reactivated, 4 received a second course of teprotumumab, while 6 received IV steroids. One patient elected to monitor. The patients who received a second course of teprotumumab had a mean (SD) posttreatment CAS score of 0 reduction in proptosis of 4 (2) mm (range: 3-6). The patients who received IV steroids had a mean (SD) posttreatment CAS of 2 (1) (range: 1-4) and a reduction in proptosis of 0 (1) mm (range: [-1] to [2]). Univariate analyses to look at predictors of reactivation found no correlation between factors such as age, sex, duration of TED, smoking status, presence of diplopia, previous treatment with radioactive iodine, history of periorbital surgery, and/or thyroidectomy after initial completion of teprotumumab between the 2 cohorts. We found a significant correlation between the CAS scores before initial treatment (P = 0.036) and thyroid hormone dysregulation (P = 0.006) in those who experienced reactivation.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;Patients with TED may experience reactivation of the disease after initial therapy with teprotumumab. Reactivated disease responds to repeat therapy with teprotumumab with higher previous CAS and thyroid hormonal dysregulation being the variables that were significantly associated with reactivation. These data underscore the importance of long-term monitoring and exploring underlying triggers for disease reactivation. Understanding these factors could help predict which patien","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142950273","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":"Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids Presenting With Predominantly Neuro-Ophthalmic Features.","authors":"Heather M McDonald, Armin Handzic, Daniel M Mandell, Laura Donaldson, Jonathan D Trobe, Edward Margolin","doi":"10.1097/WNO.0000000000002271","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002271","url":null,"abstract":"<p><strong>Background: </strong>Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare and poorly understood inflammatory disorder of the central nervous system centered on the pons. It has a characteristic imaging appearance with enhancing and T2-hyperintense punctate and curvilinear lesions in the pons. The lesions lack restricted diffusion and have relatively little perilesional edema. Although patients typically present with gait ataxia and other sequelae of brainstem inflammation including diplopia, there is scant literature focusing on patients who present with primarily neuro-ophthalmic manifestations.</p><p><strong>Methods: </strong>Case series of 3 patients presenting with diplopia who had a final diagnosis of CLIPPERS.</p><p><strong>Results: </strong>Case descriptions of a 71-year-old man, 61-year-old woman, and 38-year-old man are reported. Diplopia was the chief presenting complaint, owing to internuclear ophthalmoplegia, sixth nerve palsy, or skew deviation. All patients had nystagmus and gait ataxia. Brain MRI displayed punctate or curvilinear enhancement of pontine/middle cerebellar peduncle lesions without restricted diffusion. All patients achieved rapid improvement after corticosteroid treatment.</p><p><strong>Conclusions: </strong>In 3 patients with CLIPPERS, the main presenting complaint was diplopia. The distinctive imaging signs led to a strong presumption of CLIPPERS, permitting a truncated evaluation and early corticosteroid treatment, which provided rapid reversal of clinical and imaging manifestations.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142950272","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}