Rachel Shemesh, Hadar N Rosenblatt, Ruth Huna-Baron, Ainat Klein, Ofira Zloto, Niv Levy
{"title":"The Risk of Ischemic Stroke in Patients With Nonarteritic Anterior Ischemic Optic Neuropathy: A Big Data Study.","authors":"Rachel Shemesh, Hadar N Rosenblatt, Ruth Huna-Baron, Ainat Klein, Ofira Zloto, Niv Levy","doi":"10.1097/WNO.0000000000002354","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002354","url":null,"abstract":"<p><strong>Background: </strong>This study explored ischemic stroke occurrence in patients with nonarteritic anterior ischemic optic neuropathy (NAION) and assess the link between NAION and carotid artery stenosis (CAS) as an ischemic stroke risk factor.</p><p><strong>Methods: </strong>A retrospective (2009-2019) nationwide, population-based study was conducted using a cohort database from Clalit, the largest healthcare network in Israel. Among 1,200,000 insured individuals, 605 NAION patients without prior ischemic stroke diagnosis were included, matched with a 10-fold larger comparison group by age, sex, and cardiovascular risk factors.</p><p><strong>Results: </strong>Results indicated a higher incidence of large-vessel ischemic stroke (18.7% vs 14.7% in the comparison group) and small-vessel ischemic stroke (4.8% vs 3.1% in the comparison group) in patients with NAION. CAS prevalence was elevated in patients with NAION both during the study and during follow-up. Multivariable analysis showed hazard ratios of 1.5 (P = 0.002) for large-vessel ischemic strokes and 1.56 (P = 0.031) for small-vessel ischemic strokes in patients with NAION. The cumulative probability of ischemic strokes for patients with NAION without risk factors was higher than for the comparison group without risk factors (log rank test, P = 0.017).</p><p><strong>Conclusions: </strong>These population-based study results revealed a higher incidence of ischemic stroke after a NAION episode compared with the comparison group. This association was valid regardless of any additional vascular risk factors. There is a need to consider specific management for patients with NAION diagnosed with carotid artery stenosis because of the potential for a triadic relationship among the 3 conditions.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-05-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144078620","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}
Armin Handzic, Marius P Furter, Brigitte C Messmer, Magdalena A Wirth, Yulia Valko, Fabienne C Fierz, Edward A Margolin, Konrad P Weber
{"title":"Multivariable Prediction Model for Suspected Ocular Myasthenia Gravis: Development and Validation.","authors":"Armin Handzic, Marius P Furter, Brigitte C Messmer, Magdalena A Wirth, Yulia Valko, Fabienne C Fierz, Edward A Margolin, Konrad P Weber","doi":"10.1097/WNO.0000000000002346","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002346","url":null,"abstract":"<p><strong>Objective: </strong>Diagnosing ocular myasthenia gravis (OMG) remains challenging despite recent diagnostic advances. We addressed this challenge by developing and validating a multivariable prediction model that estimates the OMG probability given the results of any partial selection of available diagnostic tests.</p><p><strong>Methods: </strong>The source data for our model were retrieved from our blinded prospective diagnostic accuracy study at the University Hospital Zurich (USZ). Patients with ptosis and/or diplopia whose presentation was suspicious for OMG underwent comprehensive diagnostic testing. An independent neuromuscular specialist made the final diagnosis. These data were used to fit and validate a Bayesian network model against additional retrospective USZ and the University of Toronto (UoT) patient data. The primary outcome was to predict the likelihood of a positive OMG diagnosis given the available diagnostic tests. For any set of tests, the model returns an OMG probability together with 95% credible intervals, indicating the prediction uncertainty.</p><p><strong>Results: </strong>Of 89 patients included in the development of the model, 39 were diagnosed with OMG. Based on our Bayesian network model, the following variables were the most useful predictors in descending order: edrophonium test, acetylcholine receptor (AChR) antibodies), single-fiber electromyogram (sfEMG), repetitive nerve stimulations (RNS) facial nerve, RNS accessory nerve, Besinger score, ice test, sustained upgaze test, dysarthria, dyspnea, dysphagia, diplopia, ptosis, age, and sex. The model was validated by determining the mean error rate and the area under the curve (AUC) by both 10-fold cross-validation and prediction on the retrospective USZ and UoT validation data consisting of 69 and 24 patients, respectively. Of all variables, edrophonium (sensitivity 94%, specificity 90%) and AChR antibody testing (sensitivity 85%, specificity 96%) showed the highest predictive value during validation with an AUC of 0.912 and 0.872, respectively. Incorporating more predictors reduced the predictive error in both validation data sets.</p><p><strong>Conclusions: </strong>Our prediction model serves as a basis to predict the OMG likelihood. It underwent successful internal and external validation and can be used to assist in clinical decision making.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-04-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144012385","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}
Venkata S Jonnakuti, Noor Laylani, Saif A Alryalat, Osama Al Deyabat, John J Chen, Andrew G Lee
{"title":"Myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis and Associated Retinal Ischemia.","authors":"Venkata S Jonnakuti, Noor Laylani, Saif A Alryalat, Osama Al Deyabat, John J Chen, Andrew G Lee","doi":"10.1097/WNO.0000000000002334","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002334","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144023407","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}
Deborah I Friedman, Fiona E Costello, Aki Kawasaki, Kathleen B Digre
{"title":"National Conference of Neuro-Ophthalmology in Chandigarh India February 7-9, 2025.","authors":"Deborah I Friedman, Fiona E Costello, Aki Kawasaki, Kathleen B Digre","doi":"10.1097/WNO.0000000000002356","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002356","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143803573","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}
Saskia L Grimm, Fabienne C Fierz, Christopher J Bockisch, Konrad P Weber
{"title":"The Spurious Palsy-Fluctuation of Ocular Myasthenia Gravis Symptoms Characterized by Orthoptics.","authors":"Saskia L Grimm, Fabienne C Fierz, Christopher J Bockisch, Konrad P Weber","doi":"10.1097/WNO.0000000000002331","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002331","url":null,"abstract":"<p><strong>Background: </strong>Although fluctuating muscular weakness is the hallmark of myasthenia gravis (MG), research into it, especially during the course of the day, remains limited. Understanding the dynamics of myasthenic symptoms is essential for diagnosis and anticipatory treatment. Therefore, in this study, we used orthoptic and other established quantitative and subjective methods to measure ocular myasthenia gravis (OMG) symptoms throughout the day, during the course of 2 months, and in response to treatment.</p><p><strong>Methods: </strong>The goal of the study was to determine the change of gaze deviation and ptosis during the course of the day, over 2 months, and 1 hour after pyridostigmine intake. Each subject participated in 3 sessions during the day, 2 follow-up sessions, and 1 measurement before and after pyridostigmine. Measurements included the Quantitative Myasthenia Gravis (QMG) score, palpebral fissure height (PFH) photography, the conventional Hess screen test, and a video Hess screen test using video-oculography. The Myasthenia Gravis Activities of Daily Living score (MG-ADL) was obtained on each assessment day. Sum scores were calculated for the gaze deviations of the inner and outer fields of the conventional and the video Hess screen tests.</p><p><strong>Results: </strong>Twelve patients were recruited, including 11 patients with ocular and 1 patient with generalized MG (mean age 65.7 years, SD 16.9 years; 11 males). The mean sum scores of both the conventional and the video Hess screen test showed a worsening in the evening, reaching significance in the outer field of the Hess screen test (mean increase 13.4°, SD 15.3°, P = 0.02). Similarly, ptosis also worsened during the day, with a significant decrease in PFH in the evening (mean decrease 0.53 mm, SD 0.55 mm, P = 0.04). Although ptosis improved significantly after pyridostigmine intake (mean increase 0.96 mm, SD 1.05, P = 0.03), no significant changes were observed in the sum deviations of the Hess screen tests (P = 0.6). Both ptosis and the sum scores generally improved over the 2-month period, even in some patients without any therapeutic adjustments. Correspondingly, the mean QMG and MG-ADL scores decreased.</p><p><strong>Conclusions: </strong>This prospective cohort study provides insight into the dynamics of OMG, which is crucial for the optimization of diagnostic and therapeutic approaches. Our orthoptic measurements demonstrated the worsening of OMG symptoms after daily activity and a better response of ptosis to pyridostigmine than diplopia. The complexity of this fluctuating disease leads to strong interindividual variability, which requires an individual approach to improve the quality of life of patients with MG.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143811595","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":"Anisocoria and Limited Eye Movements.","authors":"Prem S Subramanian, Dhruva Gupta","doi":"10.1097/WNO.0000000000002353","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002353","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143803570","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}
Victoria A Dezard, Heather M McDonald, Wayne T Cornblath, Edward A Margolin
{"title":"Immune Checkpoint Inhibitor-Induced Ocular Myasthenia Gravis: Case Series and Literature Review.","authors":"Victoria A Dezard, Heather M McDonald, Wayne T Cornblath, Edward A Margolin","doi":"10.1097/WNO.0000000000002330","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002330","url":null,"abstract":"<p><strong>Background: </strong>Immune checkpoint inhibitors (ICIs) have transformed cancer management by enhancing the immune system's ability to target cancer cells. However, they are known to be associated with immune-related adverse events, including ICI-induced myasthenia gravis (ICI-MG). There is limited literature on the isolated ocular form, ICI-induced ocular myasthenia gravis (ICI-OMG). The purpose of this study was to characterize clinical presentation and response to treatment of patients with ICI-OMG.</p><p><strong>Methods: </strong>This case series and literature review focuses on isolated ICI-OMG. Data collected from patients at 2 North American academic neuro-ophthalmology practices included demographics, ICI type and duration of use, malignancy type, metastasis, symptoms, examination results, and treatment responses. A comprehensive literature review on isolated ICI-OMG cases was also conducted.</p><p><strong>Results: </strong>Our data identified 3 patients with ICI-OMG with bilateral ptosis and diplopia, but no systemic or bulbar involvement at presentation. Literature review found 12 additional cases, with pembrolizumab being the most common ICI associated with OMG. All cases had ptosis, and most had diplopia. Testing for multiple OMG-associated antibodies was often negative and single-fiber electromyography (sfEMG) results varied. Treatments with corticosteroids, intravenous immunoglobulins, plasmapheresis, and pyridostigmine had mixed results.</p><p><strong>Conclusions: </strong>ICI-OMG is a rare and distinct condition, presenting significant diagnostic challenges due to often negative antibody tests and variable sfEMG results. Early recognition of ICI-OMG in patients with ptosis and/or diplopia is crucial. Treatment responses vary, and most patients do not return to baseline, indicating ICI-OMG is likely chronic. Further research is needed to improve diagnostic markers and treatment strategies.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143795720","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":"Age-Related Changes in Optic Disc Drusen Visibility and Their Anatomical Correlates.","authors":"Morten Jørgensen, Lasse Malmqvist, Steffen Hamann","doi":"10.1097/WNO.0000000000002348","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002348","url":null,"abstract":"<p><strong>Background: </strong>The visibility of optic disc drusen (ODD) increases with age, yet the anatomical changes related to this finding have not been established. Enhanced-depth imaging optical coherence tomography (EDI-OCT) provides a detailed imaging modality to evaluate the anatomical location of ODD within the optic nerve head. The aim of this study was to determine the factors contributing to the age-related increased visibility of ODD using EDI-OCT.</p><p><strong>Methods: </strong>A retrospective observational study was conducted on patients who were diagnosed with ODD using EDI-OCT of the optic nerve head according to the Optic Disc Drusen Studies Consortium guidelines from November 2017 to December 2023. Data on ophthalmoscopic ODD visibility, OCT-based anatomical location, with superficial ODD being above and deep ODD being below Bruch Membrane Opening, peripapillary retinal nerve fiber layer (RNFL) thickness, and macular ganglion cell layer volume were collected and analyzed.</p><p><strong>Results: </strong>A total of 411 eyes were included for analysis. ODD visibility increased significantly with age, with 6% being visible in the first decade of life and 90% being visible in patients >70 years of age based on fundus photography. The anatomical location of ODD was consistent across all age groups. RNFL thickness exhibited an age-related decline, with a mean thickness of 153 µm in the first decade decreasing to 70 µm in patients >70 years of age. In 17% of cases, ODD were classified as buried on fundus photography despite being superficial on OCT.</p><p><strong>Conclusions: </strong>Our study revealed that with age, ODD become more visible, while their anatomical location in the optic nerve head remains stable and the peripapillary RNFL thickness decreases. This suggests that RNFL thinning is the primary contributor to the age-related increased ODD visibility.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143719926","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":"JNO News: Game on for NANOS at the AAO 2024 Annual Meeting in Chicago.","authors":"Kimberly M Winges","doi":"10.1097/WNO.0000000000002344","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002344","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143709990","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}
Tianyi Wang, John Kim, Maria Masotti, Jonathan D Trobe
{"title":"MRI Indicators of Papilledema in Brain Tumors.","authors":"Tianyi Wang, John Kim, Maria Masotti, Jonathan D Trobe","doi":"10.1097/WNO.0000000000002342","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002342","url":null,"abstract":"<p><strong>Background: </strong>Intraventricular blockage of cerebrospinal drainage by brain tumors has been proposed as a more powerful mechanism of producing high intracranial pressure (ICP) than tumor size. We tested that hypothesis by investigating the MRI signs closely associated with high ICP in brain tumors.</p><p><strong>Methods: </strong>We conducted a single-institution retrospective chart review of patients with brain tumors between 1990 and 2023. We selected 100 cases from a larger eligible cohort without regard to clinical or imaging features. Based on ophthalmologic examination, 57 patients had papilledema and 43 patients did not. The study neuroradiologist, masked as to the presence of papilledema, noted relevant MRI brain and eye signs. Associations between these MRI signs and papilledema were analyzed statistically.</p><p><strong>Results: </strong>The median age of patients with papilledema was significantly younger than that of patients without papilledema. Among the MRI brain signs, the degree of ventriculomegaly was most highly associated with papilledema, and a temporal horn size of greater than 10 mm predicted a 100% chance of papilledema in patients aged 20 years or younger. Tumor size was not associated with papilledema. Posterior scleral flattening and optic disc protrusion were also highly associated with papilledema but were often difficult to identify because of their subtlety.</p><p><strong>Conclusions: </strong>The degree of ventriculomegaly, especially of the temporal horn, was the MRI brain sign most associated with papilledema, whereas tumor size was not associated with papilledema. These findings support the idea that intraventricular obstruction of cerebrospinal fluid flow is more likely than tumor size to elevate ICP. Together with posterior scleral flattening and optic disc protrusion, ventriculomegaly can be used to predict with high probability the presence of elevated ICP and as a substitute for finding papilledema on ophthalmologic examination, a service that may not be available in low resource settings.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143710071","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}