Hye Jun Joo, Jae Ryong Song, Byung Chan Lim, Won Jae Kim, Mi Young Choi, Haeng-Jin Lee, Seung Ah Chung, Dong Gyu Choi, Hye Young Kim, Young-Woo Suh, Hyuna Kim, Joo Yeon Lee, Bo Young Chun, Ungsoo Samuel Kim, Hwan Heo, Hae Ri Yum, Eun Hee Hong, Jae Ho Jung, Seong-Joon Kim
{"title":"Six-Month Visual Outcomes in Pediatric Optic Neuritis: A Multicenter Study From South Korea.","authors":"Hye Jun Joo, Jae Ryong Song, Byung Chan Lim, Won Jae Kim, Mi Young Choi, Haeng-Jin Lee, Seung Ah Chung, Dong Gyu Choi, Hye Young Kim, Young-Woo Suh, Hyuna Kim, Joo Yeon Lee, Bo Young Chun, Ungsoo Samuel Kim, Hwan Heo, Hae Ri Yum, Eun Hee Hong, Jae Ho Jung, Seong-Joon Kim","doi":"10.1097/WNO.0000000000002474","DOIUrl":"https://doi.org/10.1097/WNO.0000000000002474","url":null,"abstract":"<p><strong>Background: </strong>Current knowledge regarding the causes, clinical course, treatment response, and long-term outcomes of pediatric optic neuritis remains largely derived from case reports and retrospective series. Considering the ethnic variability in its presentation and the critical role of antibody-based differential diagnoses in guiding treatment strategies, further research is necessary. The aim of this study was to provide insights into the etiology and clinical findings of pediatric optic neuritis, thereby improving our knowledge of the condition in the era of antibody-based approaches.</p><p><strong>Methods: </strong>This prospective multicenter cohort study was conducted at 31 referral medical centers. Patients younger than 18 years with first-episode optic neuritis were included. All patients underwent comprehensive neuro-ophthalmic examinations, including aquaporin-4-IgG and myelin oligodendrocyte glycoprotein-IgG testing and brain/orbit MRI. The primary outcome measure was best-corrected visual acuity (BCVA) at 6 months. Best-corrected visual acuity was further categorized into high vision (BCVA ≥ 20/40), moderate vision (20/200 < BCVA < 20/40), and low vision (BCVA ≤ 20/200).</p><p><strong>Results: </strong>Overall, 44 pediatric patients were enrolled, with a mean age at onset of 10.7 ± 3.5 years. Bilateral optic neuritis and optic disc swelling were observed in 56.8% and 81.8% of patients, respectively. The mean BCVA at enrolment was 0.99 ± 0.89 logMAR, which improved significantly to 0.24 ± 0.52 logMAR at 6 months. The final 6-month diagnoses included myelin oligodendrocyte glycoprotein antibody-associated disease (n = 30 [68.2%]), isolated optic neuritis (n = 11 [25.0%]), acute disseminated encephalomyelitis (n = 2 [4.5%]), multiple sclerosis (n = 1 [2.3%]). No patients had neuromyelitis optica. At the 6-month follow-up, 50.0% showed improvement in the visual category, 50.0% maintained their visual category, and none experienced deterioration. There were no significant predictors related to poor BCVA at enrolment and at 6 months.</p><p><strong>Conclusion: </strong>Pediatric optic neuritis exhibits severe visual deficits but shows good recovery. Myelin oligodendrocyte glycoprotein antibody-associated disease is the most common etiology. These findings reflect the distinct characteristics of pediatric optic neuritis and provide important baseline data for developing tailored treatment strategies.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":""},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148054857","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":"Surgery for Acquired Nystagmus in Patients With Cerebellar Dysfunction.","authors":"Ahmad M Halawa, Steven M Archer","doi":"10.1097/WNO.0000000000002413","DOIUrl":"10.1097/WNO.0000000000002413","url":null,"abstract":"<p><strong>Background: </strong>To report the association of downbeat nystagmus and chin-down head posture with cerebellar dysfunction and the results of treatment with bilateral superior rectus recession and bilateral inferior oblique anteriorization.</p><p><strong>Methods: </strong>This is a retrospective series of patients with cerebellar ataxia treated for downbeat nystagmus, oscillopsia, and abnormal head posture with bilateral superior rectus recession and bilateral inferior oblique anteriorization between 2000 and 2022.</p><p><strong>Results: </strong>Four patients underwent the procedure. All 4 patients had systemic signs and symptoms of cerebellar ataxia, confirmed by a neurologist as well as MRI findings involving the cerebellum. All patients had oscillopsia, downbeat nystagmus with damping in up gaze, and chin-down head posture. All patients underwent bilateral superior rectus recession and bilateral inferior oblique anteriorization. Median follow-up period was 3 years. Oscillopsia in primary position and abnormal head posture resolved in all patients.</p><p><strong>Conclusions: </strong>Downbeat nystagmus with oscillopsia associated with cerebellar disorders respond well to surgical treatment with bilateral superior rectus recession and bilateral inferior oblique anteriorization.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"203-204"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145458815","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":"Comparison of Olleyes Virtual Reality and Humphrey Visual Field Testing in Detecting Visual Field Defects in Patients With Neurosurgical Diagnoses.","authors":"Shu Feng, Alekya P Rajanala, Courtney E Francis","doi":"10.1097/WNO.0000000000002429","DOIUrl":"10.1097/WNO.0000000000002429","url":null,"abstract":"<p><strong>Background: </strong>Virtual reality-based visual field testing may offer an alternative to traditional Humphrey visual field testing, but the performance of such tests in detecting visual field abnormalities for patients with neurosurgical diagnoses has not previously been established.</p><p><strong>Methods: </strong>Patients presenting to neuro-ophthalmology clinic for visual field testing for neurosurgical diagnoses completed both the Humphrey Visual Field Analyzer (HVF) 30-2 SITA fast test and the Olleyes Advanced Vision Analyzer-Virtual Reality Perimeter (OE) 30-2 fast test during the same visit. Pearson correlation was used to compare mean deviation (MD) and pattern standard deviation (PSD) between the HVF and OE tests. A Bland-Altman analysis was performed, and 95% limits of agreement (LOA) were calculated to compare agreement of MD and PSD between OE and HVF tests. The sensitivity, specificity, positive predictive value, and negative predictive value of the OE test were calculated for detecting a clinically significant abnormal test on HVF testing.</p><p><strong>Results: </strong>Of 47 subjects recruited, the mean (SD) age was 54 (15) years, with 25 men. HVF testing resulted in 24 clinically significant abnormal findings. The R-squared for the Pearson correlation of MD and PSD between the HVF and OE tests were 0.45 and 0.79, respectively. Bland-Altman analysis of 95% LOA of HVF and OE tests were -10.42 to 11.16 (bias 0.37) for MD and -4.48 to 4.30 (bias -0.09) for PSD. The OE test had a sensitivity of 83.8% (95% CI 65.5%-92.4%) and specificity of 94.4% (95% CI 74.2%-99.7%) for detecting a clinically significant abnormal visual field finding on HVF testing. The positive predictive value of a clinically significant abnormal finding on OE testing was 96% (95% CI 80.5%-99.8%), for finding a clinically significant abnormal finding on HVF testing. The negative predictive value of no clinically significant abnormal finding on OE testing was 77.3% (95% CI 56.6%-89.9%).</p><p><strong>Conclusions: </strong>The OE device provides visual field results that correlate well with HVF testing and can be used as a screening test for visual field defects in patients with brain tumors. However, the moderate sensitivity for detecting a clinically relevant field deficit for the Olleyes tests warrants clinical discretion and potential repeat visual field testing with the gold standard HVF in patients with a high suspicion for visual field loss.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"238-242"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145762966","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}
Alexandria L Chaulk, Jennifer L McCarty, Rajan P Patel, Alice Z Chuang, Ore-Ofe Ooa Adesina
{"title":"Reply: Prognosticators of Optic Nerve Imaging in Malignant Pseudotumor Cerebri Syndrome.","authors":"Alexandria L Chaulk, Jennifer L McCarty, Rajan P Patel, Alice Z Chuang, Ore-Ofe Ooa Adesina","doi":"10.1097/WNO.0000000000002464","DOIUrl":"10.1097/WNO.0000000000002464","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"296-297"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147504032","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}
Annie Y Yao, Jose Efren Gonzalez Monroy, Camilo Jaimes, Amy E Roberts, Miya E Bernson Leung, Eric D Gaier
{"title":"FOXC1 -Related Morning Glory-Like Papillopathy and Cerebral Vasculopathy.","authors":"Annie Y Yao, Jose Efren Gonzalez Monroy, Camilo Jaimes, Amy E Roberts, Miya E Bernson Leung, Eric D Gaier","doi":"10.1097/WNO.0000000000002448","DOIUrl":"10.1097/WNO.0000000000002448","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"293-295"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147284400","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}
Yeji Moon, Byung J Lee, Hyunjin Kim, Young-Min Lim, Eun-Jae Lee
{"title":"Quantitative Analysis of Retrograde Trans-Synaptic Retinal Degeneration Based on Lesion Topography.","authors":"Yeji Moon, Byung J Lee, Hyunjin Kim, Young-Min Lim, Eun-Jae Lee","doi":"10.1097/WNO.0000000000002414","DOIUrl":"10.1097/WNO.0000000000002414","url":null,"abstract":"<p><strong>Background: </strong>Retrograde trans-synaptic retinal degeneration (RTSD) occurs after injury to the postgeniculate visual pathways, and the distribution of retinal thinning mirrors the pattern of visual field defects (VFDs), which reflects the topographical organization of the visual pathway. We aimed to quantitatively assess how RTSD evolves over time in a topographically specific manner based on lesion location and to identify factors associated with its progression.</p><p><strong>Methods: </strong>This longitudinal cohort study included patients with RTSD caused by structural lesions in the visual pathway, including 29 patients who have had a stroke and 22 with tumors. Optical coherence tomography was used to measure the thickness of the peripapillary retinal nerve fiber layer (pRNFL) and the macular ganglion cell-inner plexiform layer (mGCIPL) in RTSD and non-RTSD areas, defined by the side and VFD patterns. A mixed-effects model was applied to evaluate the longitudinal changes in pRNFL and mGCIPL thickness over the follow-up period and to identify associated factors.</p><p><strong>Results: </strong>In the RTSD area, the estimated rate of pRNFL thinning was 6.44 µm/log year (95% confidence interval [CI], 4.85-8.02 µm/log year), whereas the estimated rate of mGCIPL thinning was 5.44 µm/log year (95% CI, 4.66-6.21 µm/log year). Thinning was most pronounced during the first 4 years, after which the rate of thinning slowed and stabilized at 0.33 µm/year (95% CI, -0.30 to 0.97 µm/year) for pRNFL and 0.31 µm/year (95% CI, 0.06-0.56 µm/year) for mGCIPL. The thinning rates in the RTSD area were significantly faster than those in the non-RTSD area (pRNFL: 3.50 µm/log year [95% CI, 2.30-4.70 µm/log year], P = 0.005; mGCIPL: 0.29 µm/log year [95% CI, -0.14 to 0.72 µm/log year], P < 0.001). Older age, male sex, and more severe VFD were associated with faster RTSD progression. Regarding the causes, RTSD progression did not differ significantly between stroke and tumor, but hemorrhagic stroke and malignant tumors (glioblastoma, metastatic) showed faster thinning than ischemic stroke and meningioma, respectively.</p><p><strong>Conclusions: </strong>RTSD progression was pronounced in both the pRNFL and mGCIPL during the first 4 years, with greater severity observed in areas topographically linked to the brain lesion. Although RTSD developed regardless of lesion etiology, older age, male gender, severe VFD, intracerebral hemorrhage, and malignant tumors were associated with significantly faster progression.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"229-237"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145458617","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}
Edward Tran, Gautham Nair, Lulu L C D Bursztyn, J Alexander Fraser, Edsel B Ing
{"title":"The Diagnostic Yield of Antiacetylcholine Receptor Antibodies Versus Antimuscle Kinase Antibodies in Ocular Myasthenia Gravis: A Meta-Analysis.","authors":"Edward Tran, Gautham Nair, Lulu L C D Bursztyn, J Alexander Fraser, Edsel B Ing","doi":"10.1097/WNO.0000000000002382","DOIUrl":"10.1097/WNO.0000000000002382","url":null,"abstract":"<p><strong>Background: </strong>Ocular myasthenia gravis (OMG) is an autoimmune disease characterized by autoantibodies targeting postsynaptic proteins at the neuromuscular junction, leading to weakness and fatigability of the levator palpebrae superioris, orbicularis oculi and extraocular muscles. Although OMG is primarily a clinical diagnosis, serological antibody testing, predominantly acetylcholine receptor (AChR) antibodies, is usually performed. The clinical utility of muscle-specific kinase (MuSK) antibodies is less well established in OMG. This meta-analysis evaluates the use of anti-AChR and anti-MuSK in patients with OMG and the relative costs of simultaneous vs sequential testing.</p><p><strong>Methods: </strong>Studies were extracted from Cumulative Index to Nursing and Allied Health Literature (CINAHL), Embase (Ovid), Medline (Ovid), and additional gray literature. A systematic review was conducted using Covidence with 2 independent reviewers for study selection and data extraction. The meta-analysis was conducted with R version 4.4.1 on RStudio, and the meta package. Depending on the level of heterogeneity, either a fixed-effects or random-effects model was used to pool the data. Funnel plots were used to assess publication bias.</p><p><strong>Results: </strong>The pooled analysis of 44 studies (n = 4,937 patients with OMG) revealed 59% (95% confidence interval [CI]: 52%-66%) positivity for anti-AChR, whereas the pooled analysis of 34 studies with (n = 3,380) showed 5% (95% CI: 2%-9%) positivity for anti-MuSK. From 62 studies (n = 5,180), 4 patients (0.1%) were doubly seropositive for anti-AChR and anti-MuSK. In patients with OMG positive for AChR antibodies, 5 studies (n = 527) reported a thymoma prevalence of 35% (95% CI: 3%-90%), underscoring the clinical value of anti-AChR testing. Four studies (n= 259) showed that anti-AChR positive patients had a 1.82 (95% CI: 1.15-2.88) times greater risk of progressing from OMG to generalized myasthenia gravis.</p><p><strong>Conclusions: </strong>Almost two-thirds (59%) of the patients with OMG tested positive for AChR antibodies, but MuSK antibodies were only detected in 5% of patients. Positivity for anti-AChR in OMG was associated with a worse prognosis, including a higher prevalence of thymomas and an increased risk of disease generalization. Given the relatively low prevalence of anti-MuSK and the higher cost of anti-MuSK testing, clinicians could consider a stepwise approach to the serological diagnosis of OMG, where anti-MuSK is ordered only if the initial anti-AChR returns negative.</p>","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"195-202"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144760364","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}
Howard D Pomeranz, Ore-Ofe O Adesina, Andrew G Lee, Gregory P Van Stavern
{"title":"Should Patients Who Develop Nonarteritic Anterior Ischemic Optic Neuropathy While Using PDE-5 Inhibitors Continue Using These Medications?","authors":"Howard D Pomeranz, Ore-Ofe O Adesina, Andrew G Lee, Gregory P Van Stavern","doi":"10.1097/WNO.0000000000002421","DOIUrl":"10.1097/WNO.0000000000002421","url":null,"abstract":"","PeriodicalId":16485,"journal":{"name":"Journal of Neuro-Ophthalmology","volume":" ","pages":"279-284"},"PeriodicalIF":2.0,"publicationDate":"2026-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145804848","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}