Journal of Neuro-Ophthalmology最新文献

{"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}

{"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}

{"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}

{"title":"Neuro-Ophthalmic Presentations of Polycythemia.","authors":"Grace Y Zhou, Sarah Aman, Saif Aldeen Alryalat, Osama Al Deyabat, Steffen Hamann, Andrew G Lee","doi":"10.1097/WNO.0000000000002268","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002268","url":null,"abstract":"<p><strong>Background: </strong>Ocular manifestations of hyperviscosity, particularly associated with the hemodynamic changes in polycythemia, can lead to various ocular vascular events, ranging from transient monocular blindness to potentially irreversible vision loss if left untreated.</p><p><strong>Methods: </strong>This is a multicenter, retrospective case series and review of literature involving 4 patients presenting with neuro-ophthalmic symptoms related to underlying polycythemia. A thorough search for prior reported cases with similar manifestations to ours was conducted through the PubMed database.</p><p><strong>Results: </strong>Four patients, 3 men and 1 woman, ranging in age from 68 to 71 years, were evaluated in the neuroophthalmology clinic. The patients had variable clinical presentations, ranging from transient unilateral or bilateral painless vision loss, nonarteritic anterior ischemic optic neuropathy, and acephalgic migraine with precipitation of visual auras. Polycythemia was secondary to testosterone therapy in 3 cases and primary in 1 case of polycythemia vera. Diagnostic challenges and treatment strategies are discussed in the context of each case. We also describe an additional 14 cases published in prior literature, with the majority presenting with variable symptoms of vision loss associated with polycythemia, and 1 case of amaurosis fugax attributed to a migrainous visual phenomenon.</p><p><strong>Conclusions: </strong>This case series highlights the role of polycythemia as a precipitating factor for ocular vascular events, necessitating a more careful approach in clinical assessment and management of such patients. Further research is needed to better understand the mechanisms underlying the hemodynamic alterations in polycythemia and their implications for ocular health to optimize therapeutic approaches.</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":"143710078","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 Significance of Incidental Neuroimaging Findings in Neuro-Ophthalmology: A Retrospective Analysis of 5,000 Cases.","authors":"Samira Jafari, Edward A Margolin, Jonathan A Micieli","doi":"10.1097/WNO.0000000000002332","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002332","url":null,"abstract":"<p><strong>Background: </strong>Patients referred to ophthalmology and neuro-ophthalmology clinics often present with complex neurological and visual symptoms, such as headaches, double vision, and vision loss, requiring thorough history, comprehensive examinations, and neuroimaging to identify the underlying cause. While advances in neuroimaging have improved diagnostic accuracy, they have also introduced challenges with incidental findings and unexpected abnormalities. This study aimed to determine the prevalence, spectrum, and clinical relevance of incidental findings in neuro-ophthalmology patients evaluated at an academic center.</p><p><strong>Methods: </strong>A retrospective, observational cohort study included 5,000 patients (59% women, 41% men; mean age 53.7 years) who underwent neuroimaging (MRI and CT) for neuro-ophthalmic symptoms at an academic neuro-ophthalmology clinic between July 2008 and June 2024. Details of imaging type, region scanned, and reason for imaging were recorded from electronic medical records. Incidental findings were defined as abnormalities unrelated to the original reason for imaging and categorized into 4 follow-up categories (none, routine, urgent, and emergency). Imaging reports were independently reviewed and categorized by an ophthalmologist and a neuro-ophthalmologist. Descriptive statistics were used to assess the prevalence of incidental findings and their clinical significance. Chi-square tests and analysis of variance were applied to assess associations between imaging modalities, age, and the presence of incidental findings.</p><p><strong>Results: </strong>Incidental findings were present in 31% (n = 1,532) of patients; 20.6% required no follow-up, 9.6% required routine follow-up, 0.34% required urgent follow-up, and 0.1% required emergency follow-up. The most common incidental findings were microangiopathic changes (14.2%), sinus changes (6.96%), and brain atrophy (3.26%). MRI showed a higher prevalence of incidental findings than CT (31.8% vs 25.1%, P < 0.05). There was no difference in incidental findings between 1.5T vs 3T MRI. Age was significantly associated with certain findings, such as microangiopathy and brain atrophy. The most common findings by follow-up category were microangiopathic changes (no follow-up), meningiomas (routine follow-up), suspected neoplasms (urgent), and suspected metastasis (emergency).</p><p><strong>Conclusions: </strong>Incidental findings are common in neuro-ophthalmology imaging, with the majority requiring no or routine follow-up. However, a small percentage (0.44%) of cases required urgent or emergency attention, underscoring the need for careful evaluation and management protocols. These findings emphasize the importance of developing guidelines to manage incidentalomas in neuro-ophthalmology, particularly for older patients and those undergoing MRI.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143615762","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":"On behalf of the North American Neuro-Ophthalmology Society, the editorial office of the Journal of Neuro-Ophthalmology acknowledges, with gratitude, our reviewers who dedicated their time and effort between January 1 and December 31, 2024.","authors":"","doi":"10.1097/WNO.0000000000002347","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002347","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143585975","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":"Correlation Between Visual Acuity and Automated Visual Field Foveal Threshold.","authors":"Kamar S Abdullahi, Asyvia Powell, Ziou Jiang, Michael S Lee","doi":"10.1097/WNO.0000000000002326","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002326","url":null,"abstract":"<p><strong>Background: </strong>Previous studies have determined a signficant correlation between, foveal threshold on automated visual field perimetry and best-corrected visual acuity. To our knowledge, these correlations have not been studied using the Octopus (Haag-Streit, Köniz, Switzerland) perimeter.</p><p><strong>Methods: </strong>Patients underwent Octopus automated visual field testing using the glaucoma tendency-oriented perimetry protocol where a foveal threshold value was collected. Visual acuity was determined in the patient's current refractive glasses and/or with pinhole acuity. Visual acuity was converted from Snellen to logarithm of the minimum angle of resolution (logMAR) for statistical analysis. Spearman correlation coefficient was used to determine correlation between visual acuity and foveal threshold value.</p><p><strong>Results: </strong>A total of 332 eyes (243 diseased eyes and 89 healthy eyes) were assessed. A statistically significant negative correlation was found between visual acuity and foveal threshold (P < 0.001). The estimated foveal threshold was statistically significantly decreased by 1.2 dB per every 0.1 logMAR increase of visual acuity (P < 0.001). A foveal threshold of 27.47 or higher predicted a visual acuity of 20/40 or better with 90% confidence.</p><p><strong>Conclusions: </strong>Patient best-corrected logMAR visual acuity has a negative correlation with foveal threshold in automated (Octopus) visual field testing. Our findings suggest that foveal threshold may predict measured visual acuity. We created a website application to predict visual acuity based on foveal threshold, age, and presence or absence of ocular pathology. Foveal threshold may represent a surrogate estimate of acuity for patients who are unable to read the Snellen chart and for patients with functional vision loss.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2025-03-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143537153","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}