Professor of Ophthalmology and of Neurology


Dr. Heather Moss is Professor in the Stanford Departments of Ophthalmology and Neurology & Neurological Sciences. She is also Vice Chair of Clinical Research and Director of the Neuro-ophthalmology Fellowship in the Stanford Department of Ophthalmology. She directs an innovative clinical-research program in biomarker discovery at the Spencer Center for Vision Research at Stanford and provides expert neuro-ophthalmic clinical care at the Byers Eye Institute at Stanford. Dr. Moss pursued undergraduate studies in biomedical engineering at the University of Guelph, followed by doctoral studies in medical engineering at Harvard and MIT, seeking to improve human health through application of engineering principles. Working with patients inspired her to become a physician; after earning her medical degree at Harvard, she completed residency training in Neurology and fellowship training in Neuro-ophthalmology at the University of Pennsylvania. She spent six years on the faculty of the University of Illinois at Chicago, serving as the Director of Neuro-ophthalmology and starting a neuro-ophthalmology fellowship training program, before joining the Stanford faculty in 2016. Her clinical expertise includes diagnosis and treatment of optic nerve diseases, eye movement disorders, and neurological pathology affecting visual pathways. She is a national leader in application of telemedicine to improve access to neuro-ophthalmic care. Her research lab is developing novel diagnostic approaches to neurological and neuro-ophthalmic diseases through application of innovative electrophysiology, imaging, and mathematical modeling. She has published over 100 articles in peer-reviewed journals, has authored numerous book chapters, and serves on the editorial board of four journals. She is a fellow of the American Academy of Neurology (AAN) and the North American Neuro-Ophthalmology Society (NANOS) and has been elected to leadership roles in both organizations. Outside of work Dr. Moss enjoys spending time with her two children, exploring northern California, and renovating her 100-year-old house.


  • Acute Optic Neuropathy in Older Adults: Differentiating Between MOGAD Optic Neuritis and Nonarteritic Anterior Ischemic Optic Neuropathy. Neurology(R) neuroimmunology & neuroinflammation Tisavipat, N., Stiebel-Kalish, H., Palevski, D., Bialer, O. Y., Moss, H. E., Chaitanuwong, P., Padungkiatsagul, T., Henderson, A. D., Sotirchos, E. S., Singh, S., Salman, A. R., Tajfirouz, D. A., Chodnicki, K. D., Pittock, S. J., Flanagan, E. P., Chen, J. J. 2024; 11 (3): e200214


    Myelin oligodendrocyte glycoprotein antibody-associated disease optic neuritis (MOGAD-ON) and nonarteritic anterior ischemic optic neuropathy (NAION) can cause acute optic neuropathy in older adults but have different managements. We aimed to determine differentiating factors between MOGAD-ON and NAION and the frequency of serum MOG-IgG false positivity among patients with NAION.In this international, multicenter, case-control study at tertiary neuro-ophthalmology centers, patients with MOGAD presenting with unilateral optic neuritis as their first attack at age 45 years or older and age-matched and sex-matched patients with NAION were included. Comorbidities, clinical presentations, acute optic disc findings, optical coherence tomography (OCT) findings, and outcomes were compared between MOGAD-ON and NAION. Multivariate analysis was performed to find statistically significant predictors of MOGAD-ON. A separate review of consecutive NAION patients seen at Mayo Clinic, Rochester, from 2018 to 2022, was conducted to estimate the frequency of false-positive MOG-IgG in this population.Sixty-four patients with unilateral MOGAD-ON were compared with 64 patients with NAION. Among patients with MOGAD-ON, the median age at onset was 56 (interquartile range [IQR] 50-61) years, 70% were female, and 78% were White. Multivariate analysis showed that eye pain was strongly associated with MOGAD-ON (OR 32.905; 95% CI 2.299-473.181), while crowded optic disc (OR 0.033; 95% CI 0.002-0.492) and altitudinal visual field defect (OR 0.028; 95% CI 0.002-0.521) were strongly associated with NAION. On OCT, peripapillary retinal nerve fiber layer (pRNFL) thickness in unilateral MOGAD-ON was lower than in NAION (median 114 vs 201 μm, p < 0.001; median pRNFL thickening 25 vs 102 μm, p < 0.001). MOGAD-ON had more severe vision loss at nadir (median logMAR 1.0 vs 0.3, p < 0.001), but better recovery (median logMAR 0.1 vs 0.3, p = 0.002). In the cohort of consecutive NAION patients, 66/212 (31%) patients with NAION were tested for MOG-IgG and 8% (95% CI 1%-14%) of those had false-positive serum MOG-IgG at low titers.Acute unilateral optic neuropathy with optic disc edema in older adults can be caused by either MOGAD-ON or NAION. Detailed history, the degree of pRNFL swelling on OCT, and visual outcomes can help differentiate the entities and prevent indiscriminate serum MOG-IgG testing in all patients with acute optic neuropathy.

    View details for DOI 10.1212/NXI.0000000000200214

    View details for PubMedID 38547435

  • A Comparative Study of Large Language Models, Human Experts, and Expert-Edited Large Language Models to Neuro-Ophthalmology Questions. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Tailor, P. D., Dalvin, L. A., Starr, M. R., Tajfirouz, D. A., Chodnicki, K. D., Brodsky, M. C., Mansukhani, S. A., Moss, H. E., Lai, K. E., Ko, M. W., Mackay, D. D., Di Nome, M. A., Dumitrascu, O. M., Pless, M. L., Eggenberger, E. R., Chen, J. J. 2024


    BACKGROUND: While large language models (LLMs) are increasingly used in medicine, their effectiveness compared with human experts remains unclear. This study evaluates the quality and empathy of Expert + AI, human experts, and LLM responses in neuro-ophthalmology.METHODS: This randomized, masked, multicenter cross-sectional study was conducted from June to July 2023. We randomly assigned 21 neuro-ophthalmology questions to 13 experts. Each expert provided an answer and then edited a ChatGPT-4-generated response, timing both tasks. In addition, 5 LLMs (ChatGPT-3.5, ChatGPT-4, Claude 2, Bing, Bard) generated responses. Anonymized and randomized responses from Expert + AI, human experts, and LLMs were evaluated by the remaining 12 experts. The main outcome was the mean score for quality and empathy, rated on a 1-5 scale.RESULTS: Significant differences existed between response types for both quality and empathy (P < 0.0001, P < 0.0001). For quality, Expert + AI (4.16 ± 0.81) performed the best, followed by GPT-4 (4.04 ± 0.92), GPT-3.5 (3.99 ± 0.87), Claude (3.6 ± 1.09), Expert (3.56 ± 1.01), Bard (3.5 ± 1.15), and Bing (3.04 ± 1.12). For empathy, Expert + AI (3.63 ± 0.87) had the highest score, followed by GPT-4 (3.6 ± 0.88), Bard (3.54 ± 0.89), GPT-3.5 (3.5 ± 0.83), Bing (3.27 ± 1.03), Expert (3.26 ± 1.08), and Claude (3.11 ± 0.78). For quality (P < 0.0001) and empathy (P = 0.002), Expert + AI performed better than Expert. Time taken for expert-created and expert-edited LLM responses was similar (P = 0.75).CONCLUSIONS: Expert-edited LLM responses had the highest expert-determined ratings of quality and empathy warranting further exploration of their potential benefits in clinical settings.

    View details for DOI 10.1097/WNO.0000000000002145

    View details for PubMedID 38564282

  • Functional Visual Disorder Is Primarily a Psychiatric Condition. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Trobe, J. D., Moss, H. E., Lee, A. G., Van Stavern, G. P. 2024

    View details for DOI 10.1097/WNO.0000000000002121

    View details for PubMedID 38526629

  • Multi-registry analysis of patients with multiple sclerosis and neuromyelitis optica to improve capture of demographic data and compare visual outcomes. Multiple sclerosis and related disorders Moss, H. E., Wiener, L., Rizy, C., Baxi, S., Kocher, M., Torres, A. Z., Mbagwu, M. 2024; 84: 105499


    IMPORTANCE: The American Academy of Neurology Axon Registry provides real-world data for patients with multiple sclerosis and neuro-myelitis optica. However, some data are incomplete (e.g. demographics) and some relevant outcomes are not systematically captured in neurology documentation (e.g. visual acuity). The American Academy of Ophthalmology IRIS Registry (Intelligent Research in Sight) contains demographic and visual function data that may complement Axon Registry-derived data to enhance understanding of real-world visual outcomes in neurological disease.OBJECTIVE: To combine Axon Registry and IRIS Registry data to reduce missingness of demographic information and characterize visual outcomes in patients with multiple sclerosis and neuro-myelitis optica.DESIGN: Cross-sectional study.SETTING: Outpatient neurology and ophthalmology clinical practices.PARTICIPANTS: Patients participating in both registries between January 1, 2014 through December 10, 2021 were included if they had repeat ICD-9/10 codes for with multiple sclerosis or neuro-myelitis optica in the Axon registry.EXPOSURE: Diagnosis (multiple sclerosis or neuro-myelitis optica).MAIN OUTCOME AND MEASURE: Age, sex, race and ethnicity were assessed in the individual registries and classified as conflicting, missing, or not missing in the combined data set. The IRIS Registry contributed visual acuity data.RESULTS: Among 60,316 patients with multiple sclerosis and 1,068 patients with neuro-myelitis optica in the Axon Registry, 14,085 and 252 had temporal overlap in the IRIS Registry. Combining data reduced missing or conflicting data for race and ethnicity by 15-19 % (absolute reduction, all p ≤ 0.0005), but not age (p = 1.0) or gender (p = 0.08). 10,907 patients with MS and 142 with NMO had visual acuity data in the IRIS Registry. Visual acuity averaged between eyes was worse in patients with NMO after adjusting for age and gender (0.17 logMAR, 95 %CI 0.12,0.21, p < 0.0005).CONCLUSION AND RELEVANCE: Using data from two registries reduced missing data for race and ethnicity and enabled examination of outcomes captured in the IRIS Registry for conditions that are diagnosed more frequently in the Axon Registry, demonstrating the utility of a multi-registry analysis.

    View details for DOI 10.1016/j.msard.2024.105499

    View details for PubMedID 38387161

  • Which OCT Measure of the Optic Nerve Head Improves Fastest? Towards Optimizing Early Detection of Resolving Papilledema in Children. Translational vision science & technology Majmudar, T. V., Moss, H. E., Avery, R. A. 2024; 13 (1): 12


    Optical coherence tomography (OCT) has been used to monitor papilledema. This study aims to determine which OCT-derived measures of the optic nerve head (ONH) detect resolving papilledema in children faster than standard OCT measures.Children (≤18 years of age) with papilledema who completed optic nerve SD-OCT pretreatment and had evidence of treatment response on one or more follow-up OCTs within 4 months were included. Standard (mean circumpapillary retinal nerve fiber layer [cpRNFL] thickness), device-derived (per-quadrant cpRNFL) and custom (ONH height, maximum Bruch's membrane displacement [BMD], ONH volume [ONHV], and BMD volume) OCT measures were calculated. Per-eye generalized estimating equations (GEEs) modelled changes in device-derived and custom measures as a function of mean cpRNFL to identify those measures that resolved faster during early (0-2 months) follow-up. Mean cpRNFL coefficients of greater than 1 indicated faster resolving papilledema.We included 52 eyes of 29 children (mean age, 12.8 years; 72.4% female). In analysis of early follow-up visits (38 eyes from 22 children), nasal cpRNFL and maximum BMD in each quadrant resolved faster than mean cpRNFL (GEE coefficients range, 1.14-3.37). Inferior cpRNFL, superior, nasal, and inferior ONH heights and ONHV resolved slower than mean cpRNFL (GEE coefficients range, 0.67-0.87).Nasal cpRNFL is a promising device-derived OCT measure for the early detection of resolving papilledema in children compared with mean cpRNFL. Maximum BMD, a custom measure, also shows promise, but its calculation has not yet been incorporated into commercial OCT devices.This study guides the optimal use of OCT in capturing resolving papilledema in children.

    View details for DOI 10.1167/tvst.13.1.12

    View details for PubMedID 38224329

  • Efficacy and Outcome Predictors of Acute Plasma Exchange for Myelin Oligodendrocyte Glycoprotein Antibody Associated Disease Thakolwiboon, S., Redenbaugh, V., Cacciaguerra, L., Tisavipat, N., Tajfirouz, D., Chiriboga, A., Tillema, J., Lotan, I., Forcadela, M., Clemence, B., Audoin, B., Pique, J., Vasileiou, E., Sotirchos, E., Henderson, A., Champsas, D., Maillart, E., Navarro, S., Moss, H., Boudot De La Motte, M., Deschamps, R., Wilf-Yarkoni, A., Stiebel-Kalish, H., Bonelli, L., Arnold, A., Choi, D., Danesh-Meyer, H., Marignier, R., Poullin, P., Huda, S., Levy, M., Pittock, S., Hacohen, Y., Flanagan, E., Chen, J. SAGE PUBLICATIONS LTD. 2023: 125-126
  • Unilateral optic disc swelling in older adults: MOGAD versus NAION Tisavipat, N., Stiebel-Kalish, H., Palevski, D., Bialer, O., Moss, H., Chaitanuwong, P., Padungkiatsagul, T., Henderson, A., Sotirchos, E., Singh, S., Pittock, S., Flanagan, E., Chen, J. SAGE PUBLICATIONS LTD. 2023: 408
  • Role of Sex in Participation During Virtual Grand Rounds in Ophthalmology. JAMA ophthalmology Hennein, L., Liu, Y., Shuman, E. A., Kim, J. S., Moss, H. E., Keenan, J. D., Seitzman, G. D. 2023


    Sex disparities exist in academia. Female attendees consistently ask fewer questions in scientific meetings than male attendees, even when they constitute half of the audience.To assess the role of sex in participation during virtual grand rounds (GR) at a major academic center.In this prospective cohort study, attendees of the Department of Ophthalmology at the University of California, San Francisco, GR from April 2020 to April 2021 were included. All GR were held via a synchronous live video communication platform. During each GR session, a predesignated attendee collected the sex of all attendees, sex of the GR speaker, and sex of each individual who asked a question to the GR speaker in order of inquiry. The GR speakers and audience were unaware of the study. Data were analyzed from June 2021 to April 2023.The main analysis assessed the association between being female and asking one of the first 3 questions.A total of 31 virtual ophthalmology GR sessions were observed. The sex of the GR speaker was female in 13 of 31 sessions (42%). The mean (SD) percentage of audience sex at each of the GR sessions was 47% (0.05) female, 45% (0.06) male, and 8% (0.03) unknown. Male attendees were more likely to ask one of the first 3 questions compared with female attendees (prevalence ratio, 3.1; 95% CI, 2.1-4.5; P < .001).Male attendees were more likely to ask questions during virtual ophthalmology GR compared with female attendees at an academic medical center. Strategies to encourage equal participation of sex in academic discourse should be encouraged.

    View details for DOI 10.1001/jamaophthalmol.2023.3010

    View details for PubMedID 37471083

  • Chiasmal Injury in Silent Pituitary Apoplexy Without Evidence of Mass Effect. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Phansalkar, R., Navarro, S. E., Chiang, H., Moss, H. E. 2023

    View details for DOI 10.1097/WNO.0000000000001956

    View details for PubMedID 37459375

  • Longitudinal imaging of microscopic scattering features in the foveal avascular zone of multiple sclerosis using adaptive optics ophthalmoscopy Hargrave, A., Navarro, S., Buickians, D., Kipp, L., Han, M., Kowalski, B., Dubra, A., Moss, H. ASSOC RESEARCH VISION OPHTHALMOLOGY INC. 2023
  • Retinal Vessel Tortuosity Index as a Potential Biomarker of Idiopathic Intracranial Hypertension: Analysis of the IIH Treatment Trial Buickians, D., Navarro, S., Moss, H., Feldon, S., Shahidi, M. ASSOC RESEARCH VISION OPHTHALMOLOGY INC. 2023
  • Comparison of macular Macrophage Like Cell and Muller Cell Density in Patients with Multiple Sclerosis using OCT Navarro, S., Soetikno, B., Hargrave, A., Buickians, D., Dubra, A., Moss, H. ASSOC RESEARCH VISION OPHTHALMOLOGY INC. 2023
  • Pilot Implementation and Evaluation of an Outpatient Teleneurology Curriculum for Medical Students Ko, M., Moss, H., Mackay, D., Ko, P., Chartier, K., Reese, L., Wallach, P., Allen, B. LIPPINCOTT WILLIAMS & WILKINS. 2023
  • Identifying Multiple Sclerosis Relapses from Clinical Notes Using Combined Rule-based and Deep Learning Methodologies Chin, I., Moss, H., Sands, K., Kocher, M., Akkas, O., Torres, A. LIPPINCOTT WILLIAMS & WILKINS. 2023
  • Advancing knowledge and clinical development in frontotemporal dementia (FTD) using the American Academy of Neurology's (AAN) Axon Registry<SUP>®</SUP> Oliver, D., Kane, E., Dodge, S., Doherty, R., Goldfeder, G., Popescu, O., Li, S., Torres, A., Moss, H. LIPPINCOTT WILLIAMS & WILKINS. 2023
  • Multi- registry Analysis of Patients with Multiple Sclerosis (MS) and Neuromyelitis Optica (NMO) to Improve Capture of Demographic Data and Compare Visual Outcomes Moss, H., Wiener, L., Rizy, C., Baxi, S., Kocher, M., Torres, A., Mbagwu, M. LIPPINCOTT WILLIAMS & WILKINS. 2023
  • SARS-CoV-2 Infection, Vaccination, and Neuro-Ophthalmic Complications. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Chaitanuwong, P., Moss, H. E., El Dairi, M. A. 2023; 43 (1): 1-4

    View details for DOI 10.1097/WNO.0000000000001812

    View details for PubMedID 36790060

  • Visual Outcomes Following Plasma Exchange for Optic Neuritis: An International Multicenter Retrospective Analysis of 395 Optic Neuritis Attacks. American journal of ophthalmology Chen, J. J., Flanagan, E. P., Pittock, S. J., Stern, N. C., Tisavipat, N., Bhatti, M. T., Chodnicki, K. D., Tajfirouz, D. A., Jamali, S., Kunchok, A., Eggenberger, E. R., Nome, M. A., Sotirchos, E. S., Vasileiou, E. S., Henderson, A. D., Arnold, A. C., Bonelli, L., Moss, H. E., Navarro, S. E., Padungkiatsagul, T., Stiebel-Kalish, H., Lotan, I., Wilf-Yarkoni, A., Danesh-Meyer, H., Ivanov, S., Huda, S., Forcadela, M., Hodge, D., Poullin, P., Rode, J., Papeix, C., Saheb, S., Boudot de la Motte, M., Vignal, C., Hacohen, Y., Pique, J., Maillart, E., Deschamps, R., Audoin, B., Marignier, R. 2023


    To evaluate the effectiveness of plasma exchange (PLEX) for optic neuritis (ON).We conducted an international multicenter retrospective study evaluating the outcomes of ON following PLEX. Outcomes were compared to raw data from the Optic Neuritis Treatment Trial (ONTT) using a matched subset.A total of 395 ON attack treated with PLEX from 317 patients were evaluated. The median age was 37 years (range 9 to 75) and 71% were female. Causes of ON included: multiple sclerosis (108), myelin-oligodendrocyte-glycoprotein-antibody-associated-disease (MOGAD) (92), aquaporin-4-IgG-positive-neuromyelitis-optica-spectrum-disorder (AQP4+NMOSD) (75), seronegative-NMOSD (34), idiopathic (83), and other (3). Median time from onset of vision loss to PLEX was 2.6 weeks (IQR, 1.4-4.0). Median visual acuity (VA) at time of PLEX was count fingers (IQR, 20/200-hand motion) and median final VA was 20/25 (IQR, 20/20-20/60) with no differences among etiologies except MOGAD-ON which had better outcomes. In 81 (20.5%) ON attacks, the final VA was 20/200 or worse. Patients with poor outcomes were older (p=0.002), had worse VA at time of PLEX (p<0.001), and longer delay to PLEX (p<0.001). In comparison with the ONTT subset with severe corticosteroid-unresponsive ON, a final VA of worse than 20/40 occurred in 6/50 (12%) PLEX-treated ON versus 6/18 (33%) from the ONTT treated with intravenous methylprednisolone without PLEX (p=0.04).Most ON attacks improved with PLEX, and outcomes were better than attacks with similar severity in the ONTT. The presence of severe vision loss at nadir, older age, and longer delay to PLEX predicted a worse outcome while MOGAD-ON had a more favorable prognosis.

    View details for DOI 10.1016/j.ajo.2023.02.013

    View details for PubMedID 36822570

  • Evolution of Disparities in Outpatient Ophthalmic Care at a Tertiary Care Center in California at the Beginning of and One Year into the COVID-19 Public Health Emergency. Ophthalmic epidemiology Shen, A. A., Gutierrez, K. G., Villegas, N. C., Moss, H. E. 2023: 1-10


    To compare disparities in outpatient ophthalmic care during early and later periods of the COVID-19 public health emergency.This cross-sectional study compared non-peri-operative outpatient ophthalmology visits by unique patients at an adult ophthalmology practice affiliated with a tertiary-care academic medical center in the Western US during three time periods: pre-COVID (3/15/19-4/15/19), early-COVID (3/15/20-4/15/20), and late-COVID (3/15/21-4/15/21). Differences in participant demographics, barriers to care, visit modality (telehealth, in person), and subspeciality of care were studied using unadjusted and adjusted models.There were 3095, 1172 and 3338 unique patient-visits during pre-COVID, early-COVID and late-COVID (overall age 59.5 ± 20.5 years, 57% female, 41.8% White, 25.9% Asian, 16.1% Hispanic). There were disparities in patient age (55.4 ± 21.8 vs. 60.2 ± 19.9 years), race (21.9% vs. 26.9% Asian), ethnicity (18.3% Hispanic vs. 15.2% Hispanic), and insurance (35.9% vs. 45.1% Medicare) as well as changes in modality (14.2% vs. 0% telehealth) and subspecialty (61.6% vs. 70.1% internal exam specialty) in early-COVID vs. pre-COVID (p < .05 for all). In late-COVID, only insurance (42.7% vs. 45.1% Medicare) and modality of care (1.8% vs. 0% telehealth) persisted as differences compared to pre-COVID.There were disparities in patients receiving outpatient ophthalmology care during early-COVID that returned close to pre-COVID baseline one year later. These results suggest that there has not been a lasting positive or negative disruptive effect of the COVID-19 pandemic on disparities in outpatient ophthalmic care.

    View details for DOI 10.1080/09286586.2023.2180807

    View details for PubMedID 36803530

  • Accuracy of International Classification of Diseases Codes for Identifying Acute Optic Neuritis. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Muro-Fuentes, E. A., Villarreal Navarro, S. E., Moss, H. E. 2023


    The accuracy of International Classification of Diseases (ICD) codes for identifying cases of acute optic neuritis (aON) is not known. A prior study reported 61% accuracy for ICD code plus MRI consistent with aON within 2 months. This study determined accuracy for ICD code plus MRI within 2 months regardless of results.Retrospective chart review was conducted using a medical record research repository of a tertiary care institution from 1998 to 2019. Subjects with ICD-9/10 codes for ON and an MRI brain and/or orbits within 2 months of earliest (initial) ICD code were included. MRI was classified as positive or negative for aON based on report noting gadolinium-contrast enhancement. Clinical diagnosis at the time of initial code was classified as aON, prior ON, considered ON, alternative diagnosis, or unknown based on review of physician authored clinical notes within 7 days of the initial code. Accuracy of ICD code for aON, acute or prior ON, and acute, prior, or considered ON were calculated for all subjects and stratified based on MRI result.Two hundred fifty-one subjects had MRI results within 2 months of their initial ON ICD code (49 positive MRI [previously reported]; 202 negative MRI). Among those with negative MRI, 32 (16%) had aON, 40 (20%) had prior ON, 19 (9%) considered ON as a diagnosis, 92 (46%) had other confirmed diagnoses, and 19 (9%) had unknown diagnosis at time of code. Considering all subjects, accuracy for ICD code was 25% for acute ON, 41% for acute or prior ON, and 48% for acute, prior, or considered ON. Positive MRI, increased number of ON ICD codes, a code given by an ophthalmologist or neurologist within 2 months, and the presence of a neurology encounter within 2 months were associated with an increased accuracy for clinical aON diagnosis.In the setting of an MRI within 2 months, ICD codes for ON have low accuracy for acute ON and only slightly better accuracy for acute or prior ON. Accuracy is higher for cases with a positive MRI than those with a negative MRI, suggesting positive MRI in conjunction with ICD codes may help more accurately identify cases. Reliance on ICD and Current Procedural Terminology codes alone to identify aON cases may introduce substantial misclassification bias in claims-based research.

    View details for DOI 10.1097/WNO.0000000000001805

    View details for PubMedID 36696226

  • Atypical Presentations of Extraparenchymal Neurocysticercosis. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Fan, J., Tang, R., Zhang, L., Hoang, P. T., Ayoade, F., Diaz-Perez, J. A., Moss, H. E., Jiang, H. 2023


    Neurocysticercosis (NCC) is the most common parasitic infection of the central nervous system and is typically diagnosed through visualization of the cysts in the cerebral parenchyma by neuro-imaging. However, neuro-imaging may not detect extraparenchymal neurocysticercosis (EPNCC), which is a rare manifestation of the disease involving the subarachnoid, meningeal, and intraventricular spaces. We report 2 cases of extraparenchymal neurocysticercosis, and discuss the diagnostic challenges and management of this entity.Two cases were identified through clinical records.Both patients had an insidious onset with slow progression of disease, and presented with papilledema and cerebrospinal fluid (CSF) eosinophilia. One case was diagnosed with spinal cord biopsy. The other was diagnosed with CSF serology and next-generation sequencing-based pathogen analysis. Both patients were treated with ventriculoperitoneal shunt, systemic antiparasitic agents, and immunosuppression.EPNCC is less common than parenchymal NCC. A high level of clinical suspicion is required given its rarity, long incubation period, and slow progression. Diagnosis and treatment can be challenging and requires a multidisciplinary approach.

    View details for DOI 10.1097/WNO.0000000000001782

    View details for PubMedID 36637411

  • Pituitary macroadenoma causing vision loss in Wyburn-Mason syndrome: illustrative case. Journal of neurosurgery. Case lessons Hug, N. F., Purger, D. A., Moss, H. E., Dodd, R. L. 2022; 4 (26)


    BACKGROUND: Wyburn-Mason syndrome (WMS) is a neurocutaneous disorder consisting of vascular malformations of the brain, eye, and skin. These include characteristically high-flow intracranial and intraorbital arteriovenous malformations (AVMs) that present commonly with visual deterioration, headache, and hemiplegia. Complete removal of these lesions is challenging. Most patients are followed closely, and intervention occurs only in the setting of worsening symptoms secondary to AVM growth or hemorrhage. Here the authors present the first known case of a patient with WMS and a pituitary macroadenoma.OBSERVATIONS: A 62-year-old man with a 30-year history of WMS with right basal ganglia and orbital AVMs and right eye blindness presented for new-onset left-sided vision loss. A pituitary adenoma was identified compressing the optic chiasm and left optic nerve. Magnetic resonance imaging and digital subtraction angiography studies were obtained for surgical planning, and the patient underwent an endoscopic transnasal transsphenoidal resection, with significant postoperative vision improvement.LESSONS: Given the variable presentation and poor characterization of this rare syndrome, patients with WMS presenting with new symptoms must undergo evaluation for growth and hemorrhage of known AVMs, as well as new lesions. Further, in patients undergoing intracranial surgery, extensive preoperative imaging and planning are crucial for safe and successful procedures.

    View details for DOI 10.3171/CASE22236

    View details for PubMedID 36572974

  • Details and outcomes of a large cohort of MOG-IgG associated optic neuritis. Multiple sclerosis and related disorders Chen, J. J., Flanagan, E. P., Bhatti, M. T., Tisavipat, N., Jamali, S., Kunchok, A., Eggenberger, E. R., Nome, M. D., Sotirchos, E. S., Vasileiou, E. S., Henderson, A. D., Arnold, A. C., Bonelli, L., Seleme, N., Mejia-Vergara, A. J., Moss, H. E., Padungkiatsagul, T., Stiebel-Kalish, H., Lotan, I., Wilf-Yarkoni, A., Hellmann, M. A., Vuppala, A., Hodge, D., Pittock, S. J. 2022; 68: 104237


    BACKGROUND: The goal of this study was to examine the temporal relationship of eye pain to visual loss and investigate whether timing of steroid treatment affects the rate and extent of visual recovery in optic neuritis (ON) from MOG-IgG associated disease (MOGAD) in a large cohort of MOGAD patients with ON.METHODS: This is a multicenter, retrospective cohort study of consecutive MOGAD patients with ON attacks seen from 2017 to 2021 fulfilling the following criteria: (1) clinical history of ON; (2) MOG-IgG seropositivity. ON attacks were evaluated for presence/duration of eye pain, nadir of vision loss, time to intravenous methylprednisolone (IVMP) treatment, time to recovery, and final visual outcomes.RESULTS: There were 107 patients with 140 attacks treated with IVMP and details on timing of treatment and outcomes. Eye pain was present in 125/140 (89%) attacks with pain onset a median of 3 days (range, 0 to 20) prior to vision loss. Among 46 ON attacks treated with IVMP within 2 days of onset of vision loss, median time to recovery was 4 days (range, 0 to 103) compared to 15 days (range, 0 to 365) in 94 ON attacks treated after 2 days (p=0.004). Those treated within 2 days had less severe VA loss at time of treatment (median LogMAR VA 0.48, range, 0.1 to 3) compared to those treated after 2 days (median LogMAR VA 1.7, range, 0 to 3; p<0.001), and were more likely to have a VA outcome of 20/40 or better (98% vs 83%, p=0.01). After adjustment for the initial VA at time of treatment, the differences in final VA were no longer significantly different (p=0.14). In addition, some patients were documented to recover without steroid treatment.CONCLUSION: This study suggests that pain precedes vision loss in the majority of ON attacks and early steroids may lead to better outcomes in MOG-IgG ON, but some patients can recover without steroid treatment. Prospective randomized clinical trials are required to confirm these findings.

    View details for DOI 10.1016/j.msard.2022.104237

    View details for PubMedID 36252317

  • Perioperative Care of the Patient with Eye Pathologies Undergoing Nonocular Surgery. Anesthesiology Roth, S., Moss, H. E., Vajaranant, T. S., Sweitzer, B. 2022


    The authors reviewed perioperative ocular complications and implications of ocular diseases during nonocular surgeries. Exposure keratopathy, the most common perioperative eye injury, is preventable. Ischemic optic neuropathy, the leading cause of perioperative blindness, has well-defined risk factors. The incidence of ischemic optic neuropathy after spine fusion, but not cardiac surgery, has been decreasing. Central retinal artery occlusion during spine fusion surgery can be prevented by protecting eyes from compression. Perioperative acute angle closure glaucoma is a vision-threatening emergency that can be successfully treated by rapid reduction of elevated intraocular pressure. Differential diagnoses of visual dysfunction in the perioperative period and treatments are detailed. Although glaucoma is increasingly prevalent and often questions arise concerning perioperative anesthetic management, evidence-based recommendations to guide safe anesthesia care in patients with glaucoma are currently lacking. Patients with low vision present challenges to the anesthesia provider that are becoming more common as the population ages.

    View details for DOI 10.1097/ALN.0000000000004338

    View details for PubMedID 36179149

  • Perioperative Ischemic Optic Neuropathy after Cardiac Surgery: Development and Validation of a Preoperative Risk Prediction Model. Journal of cardiothoracic and vascular anesthesia Shah, S. H., Xiao, L., Chen, Y., Moss, H. E., Rubin, D. S., Roth, S. 2022


    OBJECTIVE: Previous studies identified risk factors for ischemic optic neuropathy (ION) after cardiac surgery; however, there is no easy-to-use risk calculator for the physician to identify high-risk patients for ION before cardiac surgery. The authors sought to develop and validate a simple-to-use predictive model and calculator to assist with preoperative identification of risk and informed consent for this rare but serious complication.DESIGN: Retrospective case-control study.SETTING: Hospital discharge records.PATIENTS: A total of 5,561,177 discharges in the National Inpatient Sample >18 years of age, with procedure codes for coronary artery bypass grafting, heart valve repair/replacement, or left ventricular assist device insertion.INTERVENTIONS: All patients had undergone cardiac surgery.MEASUREMENTS AND MAIN RESULTS: Known preoperative risk factors for ION after cardiac surgery were assessed to develop a risk score and prediction model. This model was validated internally using the split-sample method. There were 771 cases of ION among 5,561,177 patients in the National Inpatient Sample. The risk factors for ION used in the model were carotid artery stenosis, cataract, diabetic retinopathy, macular degeneration, glaucoma, male sex, and prior stroke; whereas uncomplicated diabetes decreased risk. With the internal validation, the predictive model had an area under the receiver operating characteristic curve of 0.66. A risk score cutoff ≥3 had 98.4% specificity.CONCLUSIONS: This predictive model, based on previously identified preoperative factors, predicted risk of perioperative ION with a fair area under the receiver operating characteristic curve. This predictive model could enable screening to provide a more accurate risk assessment for ION, and consent process for cardiac surgery.

    View details for DOI 10.1053/j.jvca.2022.08.005

    View details for PubMedID 36114093

  • Tele-Neuro-Ophthalmology Utilization, Availability, and Attitudes: Update 1 Year Into the COVID-19 Public Health Emergency. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Moss, H. E., Lai, K. E., Ko, M. W. 2022


    Telehealth was rapidly adopted early in the COVID-19 pandemic as a way to provide medical care while reducing risk of SARS-CoV2 transmission. Since then, telehealth utilization has evolved differentially according to subspecialty. This study assessed changes in neuro-ophthalmology during the first year of the COVID-19 pandemic.Telehealth utilization and opinions pre-COVID-19, early pandemic (spring 2020), and 1 year later (spring 2021) were surveyed among practicing neuro-ophthalmologists in and outside the United States using an online platform. Demographics, self-reported utilization, perceived benefits, barriers, and examination suitability were collected over a 2-week period in May 2021.A total of 135 practicing neuro-ophthalmologists (81.5% United States, 47.4% females, median age 45-54 years) completed the survey. The proportion of participants using video visits remained elevated during COVID + 1 year (50.8%) compared with pre-COVID (6%, P < 0.0005, McNemar), although decreased compared with early COVID (67%, P < 0.0005). Video visits were the most commonly used methodology. The proportion of participants using remote testing (42.2% vs 46.2%), virtual second opinions (14.5% vs 11.9%, P = 0.45), and eConsults (13.5% vs 16.2%, P = 0.38) remained similar between early and COVID + 1 year (P = 0.25). The majority selected increased access to care, better continuity of care, and enhanced patient appointment efficiency as benefits, whereas reimbursement, liability, disruption of in-person clinic flow, limitations of video examinations, and patient technology use were barriers. Many participants deemed many neuro-ophthalmic examination elements unsuitable when collected during a live video session, although participants believed some examination components could be evaluated adequately through a review of ancillary testing or outside records.One year into the COVID-19 pandemic, neuro-ophthalmologists maintained telemedicine utilization at rates higher than prepandemic levels. Tele-neuro-ophthalmology remains a valuable tool in augmenting patient care.

    View details for DOI 10.1097/WNO.0000000000001663

    View details for PubMedID 36166781

  • High Altitude as a Risk Factor for the Development of Nonarteritic Anterior Ischemic Optic Neuropathy. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Liu, Y. A., Mesentier-Louro, L. A., Shariati, M. A., Moss, H. E., Beres, S. J., Liao, Y. J. 2022


    Episodic high-altitude exposure leads to optic disc edema and retinopathy. It is uncertain whether high-altitude exposure is a risk factor for nonarteritic anterior ischemic optic neuropathy (NAION).We performed a single-center, retrospective, cross-sectional case study of 5 patients with high-altitude-associated NAION (HA-NAION) from April 2014 to April 2019. Main study parameters included known vascular risk factors for NAION, evolution of visual acuity, visual field, optic disc, and macula measurements.We studied 5 eyes of 5 patients with HA-NAION that occurred at 7,000-9,000 ft above sea level, 28 patients with classic NAION that developed at sea level (normal altitude NAION or NA-NAION), and 40 controls. All 5 patients with HA-NAION had clinically confirmed NAION by a neuro-ophthalmologist within 3-21 days of onset and comprehensive follow-up evaluations (average follow-up of 23 months). Other than high-altitude exposure, 4 of 5 patients had undiagnosed obstructive sleep apnea (OSA, apnea-hypopnea index 5.4-22.2) and 1 had systemic vascular risk factors. All patients had disc-at-risk in the contralateral eye. The best-corrected distance visual acuity was 20/20 to 20/70 (median logMAR 0) at presentation and 20/70 to counting finger (median logMAR 0) at ≥6 months. Automated static perimetry revealed average mean deviation of -18.6 dB at presentation and -22.1 dB at ≥6 months. The average retinal nerve fiber layer was 244 µm (80-348 µm) at onset and 59 µm (55-80 µm) at ≥6 months. The average ganglion cell complex thickness was 50 µm (43-54 µm) at onset and 52 µm (50-55 µm) at ≥6 months. The patients with OSA were started on home continuous positive airway pressure treatment. Visual outcomes were similar in patients with HA-NAION and NA-NAION. - After addressing all NAION risk factors, no new events occurred in the HA-NAION group within 2-8 years with or without repeat high-altitude exposure.NAION can occur under high-altitude conditions. HA-NAION is associated with relatively younger age at onset, disc-at-risk, and OSA. These patients exhibit a relatively progressive course of vision loss after initial onset and severe thinning of optic nerves on optical coherence tomography. Treatment for OSA is recommended, especially with repeated high-altitude exposure.

    View details for DOI 10.1097/WNO.0000000000001629

    View details for PubMedID 36166787

  • Retinal Vessel Tortuosity Indices (VTIs) as a potential biomarker of idiopathic intracranial hypertension(IIH): Baseline analysis of the IIH treatment trial Buickians, D., Dahshan, D., Shahidi, M., Nankali, A., Feldon, S. E., Moss, H. ASSOC RESEARCH VISION OPHTHALMOLOGY INC. 2022
  • Tele-Neuro-Ophthalmology: An Update One Year Into The COVID-19 Pandemic Ng, A., Moss, H., Lai, K., Ko, M. LIPPINCOTT WILLIAMS & WILKINS. 2022
  • Representation of Women in Ophthalmology Subspecialty Societies over 20 OPHTHALMOLOGY Azad, A. D., Chandramohan, A., Li, A. S., Rosenblatt, T. R., Reeves, M. R., Veerappan-Pasricha, M., Ludwig, C. A., Nguyen, A., Winges, K. M., Wang, S. Y., Pan, C. K., Moss, H. E., Do, D. V., Fountain, T. R., Kossler, A. L. 2022; 129 (5): 587-590
  • Evaluation of Interventions Targeting Follow-up Appointment Scheduling After Emergency Department Referral to Ophthalmology Clinics Using A3 Problem Solving. JAMA ophthalmology Tan, C. H., Mickelsen, J., Villegas, N., Levina, M., Shows, A., Oruna, K., Erickson, B., Moss, H. E. 2022


    Importance: Many patients seen for eye-related issues in the emergency department do not receive recommended follow-up care. Prior evidence supports that scheduling appointments is a barrier to accomplishing the transition to outpatient ophthalmology care.Objective: To evaluate time until appointment scheduling following emergency department discharge with urgent outpatient ophthalmology referral.Design, Setting, and Participants: The A3 problem solving process was implemented by a multidisciplinary team as part of a structured quality improvement program with the goal of reducing the mean time between urgent referral placement in the emergency department and outpatient ophthalmology appointment scheduling. The study was conducted at Stanford Health Care, an academic medical center in Palo Alto, California, affiliated with Stanford University School of Medicine. Using medical center administrative records, all patients discharged from the adult emergency department with an urgent outpatient referral to the Stanford Department of Ophthalmology from August 9 to September 19, 2020 (baseline; n=43), and from October 26 to November 29, 2020 (after implementation of all interventions; n=21), were included.Interventions: Interventions developed to target the workflow of the ophthalmology resident, emergency department, ophthalmology clinic, and health system schedulers to address key drivers of the referral-scheduling process included medical record documentation guidelines, identification of responsible parties, preidentified appointment slots, patient education materials, and education of stakeholders, and were implemented by October 25, 2020.Main Outcomes and Measures: Mean time between urgent referral placement (ie, emergency department discharge) and appointment scheduling with outpatient ophthalmology at baseline vs postintervention.Results: At baseline, appointments were scheduled a mean (range) 2.8 (0-7) days after referral placement. In the 5 weeks following implementation of interventions, the mean (range) decreased to 1.3 (0-4) days, a difference of 1.5 days (95% CI, 0.20-2.74; P=.02). This corresponds to 642 (95% CI, 86-1173) days of reduced patient wait time annually. In addition, there was less variability in the number of days between referral and appointment scheduling after intervention compared with baseline.Conclusions and Relevance: The results suggest improvement in efficiency of outpatient ophthalmology appointment scheduling of urgent emergency department referrals could be achieved through application of a quality improvement methodology by a multidisciplinary team representing key stakeholders in the process.

    View details for DOI 10.1001/jamaophthalmol.2022.0889

    View details for PubMedID 35446350

  • Patient Personality and Illness Perceptions in Relation to Follow-Up Appointment Adherence in Neuro-Ophthalmology. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Aziz, R., Bindiganavale, M. P., Chang, R. T., Moss, H. E. 2022


    BACKGROUND: Improving patient attendance at medical follow-up visits may have a notable impact on disease and overall health outcomes. Understanding factors contributing to poor attendance is important for identifying at-risk patients and designing interventions to improve clinical outcomes. Our objective was to identify personality and illness perception factors associated with attendance at recommended follow-up visits in a neuro-ophthalmology practice.METHODS: New or established patients (≥18 years) with scheduled neuro-ophthalmology (study) or glaucoma (comparison) appointments at a tertiary care academic medical center completed the Brief Illness Perception Questionnaire and Ten-Item Personality Inventory. Physician recommendations made during the visit were recorded (medications, referrals, follow-up, testing, and procedures). A chart review was performed 18 months after enrollment to assess attendance at follow-up appointment and adherence with other physician recommendations. Multiple variable logistic regression models studied associations between follow-up appointment attendance and demographic factors, appointment factors, and survey responses.RESULTS: Among 152 respondents (97% response rate (152 of 157), aged 19-97 years, 58% female, 34% new, 80 neuro-ophthalmology, 72 glaucoma), neuro-ophthalmology subjects were younger, more likely to be White, non-Hispanic, female and new to the practice than subjects with glaucoma. They reported higher emotional impact, identity, and consequences related to their illness (P = 0.001-0.03). Neuro-ophthalmology physician recommendations included more referrals to other services (17.5% vs 1.4%, P = 0.001, chi-square) and more radiology studies (15% vs 0%, P = 0.001, chi-square), but fewer follow-up visits (75% vs 97%, P < 0.0005, chi-square). Among those with recommended follow-up visits, neuro-ophthalmology subjects had lower rates of on-time appointment attendance (55% vs 77%, P = 0.009, chi-square). In a multiple variable model, on-time follow-up attendance was associated with shorter recommended follow-up interval (≤90 days, P < 0.0005), established (vs new) patient status at enrollment visit (P = 0.04), and glaucoma (P = 0.08), but not subject demographics, illness perception, or personality factors.CONCLUSIONS: Patient demographics, illness perception, and personality traits were not associated with follow-up appointment attendance and therefore unlikely to be useful for identifying patients at risk of being lost to follow-up. New neuro-ophthalmology patients with a follow-up recommended ≥90 days in advance may benefit from targeted interventions to improve follow-up appointment adherence.

    View details for DOI 10.1097/WNO.0000000000001533

    View details for PubMedID 35421870

  • Management of Functional Vision Disorders. Current neurology and neuroscience reports Phansalkar, R., Lockman, A. J., Bansal, S., Moss, H. E. 2022


    PURPOSE OF REVIEW: The purpose of this review is to summarize current approaches to management of functional vision disorder (FVD).RECENT FINDINGS: Several retrospective studies of FVD in both adults and children have shed light on the range of outcomes and the prevalence of psychosocial stressors among FVD patients. While the first line of treatment for FVD is reassurance and education, recent case reports highlight the use of additional treatment modalities including psychotherapy, hypnosis, and transcranial magnetic stimulation in specific cases. Although the epidemiology and diagnosis of functional vision disorder are well described, there is limited evidence supporting treatment modalities. Nevertheless, the majority of patients improve with conservative management including reassurance, education, and appropriate follow-up. Additional approaches such as mental health care referral can be considered in refractory cases.

    View details for DOI 10.1007/s11910-022-01191-w

    View details for PubMedID 35320465

  • Predictive Value of International Classification of Diseases Codes for Idiopathic Intracranial Hypertension in a University Health System. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Khushzad, F., Kumar, R., Muminovic, I., Moss, H. E. 2022; 42 (1): 6-10


    Misclassification bias is introduced into medical claims-based research because of reliance on diagnostic coding rather than full medical record review. We sought to characterize this bias for idiopathic intracranial hypertension (IIH) and evaluate strategies to reduce it.A retrospective review of medical records was conducted using a clinical data warehouse containing medical records and administrative data from an academic medical center. Patients with 1 or more instances of International Classification of Diseases (ICD)-9 or -10 codes for IIH (348.2 or G93.2) between 1989 and 2017 and original results of neuroimaging (head CT or MRI), lumbar puncture, and optic nerve examination were included in the study. Diagnosis of IIH was classified as definite, probable, possible, or inaccurate based on review of medical records. The positive predictive value (PPV) for IIH ICD codes was calculated for all subjects, subjects with an IIH code after all testing was completed, subjects with high numbers of IIH ICD codes and codes spanning longer periods, subjects with IIH ICD codes associated with expert encounters (ophthalmology, neurology, or neurosurgery), and subjects with acetazolamide treatment.Of 1,005 patients with ICD codes for IIH, 103 patients had complete testing results and were included in the study. PPV of ICD-9/-10 codes for IIH was 0.63. PPV in restricted samples was 0.82 (code by an ophthalmologist n = 57), 0.70 (acetazolamide treatment n = 87), and 0.72 (code after all testing, n = 78). High numbers of code instances and longer duration between the first and last code instance also increased the PPV.An ICD-9 or -10 code for IIH had a PPV of 63% for probable or definite IIH in patients with necessary diagnostic testing performed at a single institution. Coding accuracy was improved in patients with an IIH ICD code assigned by an ophthalmologist. Use of coding algorithms considering treatment providers, number of codes, and treatment is a potential strategy to reduce misclassification bias in medical claims-based research on IIH. However, these are associated with a reduced sample size.

    View details for DOI 10.1097/WNO.0000000000001563

    View details for PubMedID 35500234

  • OCT retinal nerve fiber layer thickness differentiates acute optic neuritis from MOG antibody-associated disease and Multiple Sclerosis: RNFL thickening in acute optic neuritis from MOGAD vs MS. Multiple sclerosis and related disorders Chen, J. J., Sotirchos, E. S., Henderson, A. D., Vasileiou, E. S., Flanagan, E. P., Bhatti, M. T., Jamali, S., Eggenberger, E. R., Dinome, M., Frohman, L. P., Arnold, A. C., Bonelli, L., Seleme, N., Mejia-Vergara, A. J., Moss, H. E., Padungkiatsagul, T., Stiebel-Kalish, H., Lotan, I., Hellmann, M. A., Hodge, D., Oertel, F. C., Paul, F., Saidha, S., Calabresi, P. A., Pittock, S. J. 1800; 58: 103525


    BACKGROUND: Optic neuritis (ON) is the most common manifestation of myelin oligodendrocyte glycoprotein antibody associated disorder (MOGAD) and multiple sclerosis (MS). Acute ON in MOGAD is thought to be associated with more severe optic disk edema than in other demyelinating diseases, but this has not been quantitatively confirmed. The goal of this study was to determine whether optical coherence tomography (OCT) can distinguish acute ON in MOGAD from MS, and establish the sensitivity of OCT as a confirmatory biomarker of ON in these entities.METHODS: This was a multicenter cross-sectional study of MOGAD and MS patients with peripapillary retinal nerve fiber layer (pRNFL) thickness measured with OCT within two weeks of acute ON symptom. Cirrus HD-OCT (Carl Zeiss Meditec, Inc. Dublin, CA, USA) was used to measure the pRNFL during acute ON. Eyes with prior ON or disk pallor were excluded. A receiver operating characteristic (ROC) curve analysis was performed to assess the ability of pRNFL thickness to distinguish MOGAD from MS.RESULTS: Sixty-four MOGAD and 50 MS patients met study inclusion criteria. Median age was 46.5 years (interquartile range [IQR]: 34.3-57.0) for the MOGAD group and 30.4 years (IQR: 25.7-38.4) for the MS group (p<0.001). Thirty-nine (61%) of MOGAD patients were female compared to 42 (84%) for MS (p=0.007). The median pRNFL thickness was 164m (IQR: 116-212) in 96 acute MOGAD ON eyes compared to 103m (IQR: 93-113) in 51 acute MS ON eyes (p<0.001). The ROC area under the curve for pRNFL thickness was 0.81 (95% confidence interval 0.74-0.88) to discriminate MOGAD from MS. The pRNFL cutoff that maximized Youden's index was 118m, which provided a sensitivity of 74% and specificity of 82% for MOGAD. Among 31 MOGAD and 48 MS eyes with an unaffected contralateral eye or a prior baseline, the symptomatic eye had a median estimated pRNFL thickening of 45m (IQR: 17-105) and 7.5m (IQR: 1-18), respectively (p<0.001). All MOGAD affected eyes had a≥5m pRNFL thickening, whereas 26 (54%) MS affected eyes had a≥5m thickening.CONCLUSION: OCT-derived pRNFL thickness in acute ON can help differentiate MOGAD from MS. This can aid with early diagnosis and guide disease-specific therapy in the acute setting before antibody testing returns, and help differentiate borderline cases. In addition, pRNFL thickening is a sensitive biomarker for confirming acute ON in MOGAD, which is clinically helpful and could be used for adjudication of attacks in future MOGAD clinical trials.

    View details for DOI 10.1016/j.msard.2022.103525

    View details for PubMedID 35038647

  • Diagnostic performance of neuroimaging in suspected idiopathic intracranial hypertension. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia Wang, M. T., Prime, Z. J., Xu, W., McKelvie, J., Papchenko, T., Padungkiatsagul, T., Moss, H. E., Danesh-Meyer, H. V. 1800; 96: 56-60


    The diagnostic utility of neuroradiologic signs associated with idiopathic intracranial hypertension (IIH) for the evaluation of patients presenting with papilloedema remains yet to be elucidated. This multicentre retrospective cohort study assessed consecutive patients presenting with suspected papilloedema to Auckland District Health Board (NZ) and Stanford University Medical Centre (US), between 2005 and 2019, undergoing magnetic resonance imaging and venography (MRI/MRV) or computed tomography and venography (CT/CTV) prior to lumbar puncture assessment for diagnostic suspicion of IIH. Data were collected regarding demographic, clinical, radiologic, and lumbar puncture parameters, and the diagnosis of IIH was determined according to the Friedman criteria for primary pseudotumor cerebri syndrome. A total of 204 participants (174 females; mean±SD age 29.9±12.2years) were included, and 156 (76.5%) participants fulfilled the diagnostic criteria for IIH. The presence of any IIH-associated radiologic sign on MRI/MRV demonstrated a sensitivity (95% CI) of 74.8% (65.8%-82.0%) and specificity (95% CI) of 94.7% (82.7%-98.5%), while radiologic signs on CT/CTV exhibited a sensitivity (95% CI) of 61.0% (49.9%-71.2%) and specificity (95% CI) of 100.0% (83.2%-100.0%). In summary, the modest sensitivities of radiologic signs of IIH would support the routine use of lumbar puncture assessment following neuroimaging to secure the diagnosis. However, the high specificities might lend limited support for the judicious deferment of lumbar puncture assessment among typical IIH demographic patients who consent to the inherent small risk of missed pathology, which has been proposed by some clinicians.

    View details for DOI 10.1016/j.jocn.2021.12.024

    View details for PubMedID 34974249

  • Representation of Women in Ophthalmology Subspecialty Societies over 20 Years. Ophthalmology Azad, A. D., Chandramohan, A., Li, A. S., Rosenblatt, T. R., Reeves, M. R., Pasricha, M. V., Ludwig, C. A., Nguyen, A., Winges, K. M., Wang, S. Y., Pan, C. K., Moss, H. E., Do, D. V., Fountain, T. R., Kossler, A. L. 1800


    The representation of women has increased over the last 20 years among ophthalmology subspecialty society new membership, award winners, and executive committee membership; however, proportional representation is still lacking at most benchmarks.

    View details for DOI 10.1016/j.ophtha.2021.12.011

    View details for PubMedID 34958831

  • Emerging concepts in the treatment of optic neuritis: mesenchymal stem cell-derived extracellular vesicles. Stem cell research & therapy Aneesh, A., Liu, A., Moss, H. E., Feinstein, D., Ravindran, S., Mathew, B., Roth, S. 2021; 12 (1): 594


    BACKGROUND: Optic neuritis (ON) is frequently encountered in multiple sclerosis, neuromyelitis optica spectrum disorder, anti-myelin oligodendrocyte glycoprotein associated disease, and other systemic autoimmune disorders. The hallmarks are an abnormal optic nerve and inflammatory demyelination; episodes of optic neuritis tend to be recurrent, and particularly for neuromyelitis optica spectrum disorder, may result in permanent vision loss.MAIN BODY: Mesenchymal stem cell (MSC) therapy is a promising approach that results in remyelination, neuroprotection of axons, and has demonstrated success in clinical studies in other neuro-degenerative diseases and in animal models of ON. However, cell transplantation has significant disadvantages and complications. Cell-free approaches utilizing extracellular vesicles (EVs) produced by MSCs exhibit anti-inflammatory and neuroprotective effects in multiple animal models of neuro-degenerative diseases and in rodent models of multiple sclerosis (MS). EVs have potential to be an effective cell-free therapy in optic neuritis because of their anti-inflammatory and remyelination stimulating properties, ability to cross the blood brain barrier, and ability to be safely administered without immunosuppression.CONCLUSION: We review the potential application of MSC EVs as an emerging treatment strategy for optic neuritis by reviewing studies in multiple sclerosis and related disorders, and in neurodegeneration, and discuss the challenges and potential rewards of clinical translation of EVs including cell targeting, carrying of therapeutic microRNAs, and prolonging delivery for treatment of optic neuritis.

    View details for DOI 10.1186/s13287-021-02645-7

    View details for PubMedID 34863294

  • Variability of Retinal Vessel Tortuosity Measurements Using a Semiautomated Method Applied to Fundus Images in Subjects With Papilledema. Translational vision science & technology Moss, H. E., Cao, J., Wasi, M., Feldon, S. E., Shahidi, M. 1800; 10 (14): 32


    Purpose: To develop methods to quantitatively measure retinal vessel tortuosity from fundus images acquired in subjects with papilledema and assess sources of variability in these measurements.Methods: Digital fundus images from 30 eyes of subjects with untreated idiopathic intracranial hypertension and papilledema were analyzed. Retinal vein and artery tortuosities for three to four vessels of each type were measured in a region of interest 1.8 to 2.7 mm from the center of the optic nerve head. Measurements were averaged to generate a venous tortuosity index (VTI) and arterial tortuosity index (ATI) for each eye. One image of each eye was analyzed two times by the same rater. Two images of each eye, differing by focal depth, were analyzed by the same rater. Correlations between VTI and ATI for the same image and different images were calculated.Results: Intrarater Pearson correlations (r) were 0.8 (95% confidence interval [CI], 0.59-0.9) and 0.90 (95% CI, 0.73-0.96) for VTI and ATI, respectively, with one outlier removed. Interimage r values were 0.72 (95% CI, 0.48-0.87) and 0.96 (95% CI, 0.89-0.99) for VTI and ATI, respectively, with one outlier removed. The intraclass correlation coefficients for agreement and consistency were similar, suggesting that the discrepancy between measurements was due to residual random error.Conclusions: The finding of similar intrarater and interimage variability suggests that intrarater variability may be a more dominant source than physiology and image acquisition.Translational Relevance: Standardizing rater procedures and averaging multiple measuring sessions are strategies to reduce variability and improve reliability of detecting retinal vessel tortuosity changes in images of eyes with papilledema.

    View details for DOI 10.1167/tvst.10.14.32

    View details for PubMedID 34967836

  • Evolving Evidence in Idiopathic Intracranial Hypertension. Life (Basel, Switzerland) Mollan, S. P., Moss, H. E., Hamann, S. 2021; 11 (11)


    Idiopathic intracranial hypertension (IIH) is an enigmatic disorder characterized by raised intracranial pressure (ICP) with no known cause and it affects both children and adults [...].

    View details for DOI 10.3390/life11111225

    View details for PubMedID 34833101

  • Development and Preliminary Validation of a Virtual Reality Approach for Measurement of Torsional Strabismus. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Bindiganavale, M., Buickians, D., Lambert, S. R., Bodnar, Z. M., Moss, H. E. 2021


    BACKGROUND: Double Maddox rod (DMR), the gold-standard method for in-office measurement of cyclodeviation, requires an examiner and specialized equipment. The objective of this study was to develop a virtual reality (VR) technique for measurement of cyclodeviation and validate this against the DMR.METHODS: A VR-DMR was implemented using a smartphone and commercially available VR viewer. The app displayed a line to each eye and accepted touch inputs from the user to rotate the lines into perceived alignment. VR-DMR cyclodeviation measurements were compared with traditional DMR (T-DMR) cyclodeviation measurements in adults with and without strabismus and children without strabismus.RESULTS: Thirty-one subjects were studied (age 5-88 years, 20 with strabismus). VR-DMR had similar test-retest reliability as T-DMR. VR-DMR was highly correlated with T-DMR (r2 = 0.94, linear regression slope 1.12) with a slight positive bias (linear regression y intercept 1°). VR-DMR was preferred by 54% of subjects with 29% having no preference.CONCLUSIONS: A VR method of ocular cyclodeviation measurement using sensory techniques was implemented using commercially available hardware. VR measurements compared favorably with gold-standard DMR measurements, and user feedback was positive. The VR methodology has application for in office and home use by nonexperts for purposes of strabismus monitoring.

    View details for DOI 10.1097/WNO.0000000000001451

    View details for PubMedID 34812760

  • Development of a Prognostic Model for Predicting Multiple Sclerosis After Optic Neuritis: A Secondary Analysis of Data From the Optic Neuritis Treatment Trial. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Luo, W., Deng, X., Xu, X., Song, R., Luo, M., Moss, H. E., Du, Y. 2021


    BACKGROUND: Optic neuritis can be the initial manifestation of multiple sclerosis (MS). The purpose of this study was to develop a prognostic model for predicting the risk of MS development among patients with optic neuritis.METHODS: The data from 388 patients with optic neuritis were retrieved from the Optic Neuritis Treatment Trial (ONTT). Cox proportional hazards regression analysis was used to develop a prognostic model. The performance of the model was assessed by using Harrell's C-index and calibration curves. The rates of MS development were estimated using the Kaplan-Meier method.RESULTS: Among the enrolled subjects, a total of 154 (39.7%) patients developed clinically definite MS during a median follow-up period of 15.8 years (interquartile range, 7.2-16.9 years). The factors associated with the development of MS were the presence of brain lesions as on baseline MRI, previous nonspecific neurologic symptoms, commencing low-dose corticosteroids treatment, ocular pain, and absence of optic disc/peripapillary hemorrhage. After incorporating these 5 factors into the prognostic model, a C-index of 0.72 (95% confidence interval [CI], 0.69-0.76) and good calibration curves were obtained. The C-index of the model was significantly higher than the C-indexes of any single factor (P < 0.001 in all cases). The model was able to stratify the ONTT patient cohort into 3 risk groups with significantly different intergroup rates of developing MS (rates for developing MS within a 15-year period: high-risk group, 75.7% [95% CI, 65.6%-82.9%], intermediate-risk group, 44.7% [95% CI, 31.4%-55.4%]; and low-risk group, 20.8% [95% CI, 14.2%-26.8%]; log-rank P < 0.001).CONCLUSIONS: This prognostic model had a better prediction ability when compared with the standard practice that relies solely on using brain lesions on MRI. It can, therefore, help guide decision-making to initiate earlier disease-modifying therapy for patients with optic neuritis at risk of developing MS.

    View details for DOI 10.1097/WNO.0000000000001424

    View details for PubMedID 34860745

  • Optical coherence tomography demonstrates more severe peripapillary retinal nerve fiber layer thickening in acute optic neuritis from myelin oligodendrocyte glycoprotein antibody associated disorder than multiple sclerosis Chen, J., Sotirchos, E., Henderson, A., Vasileiou, E., Flanagan, E., Bhatti, M. T., Jamali, S., Eggenberger, E., Dinome, M., Frohman, L., Arnold, A., Bonelli, L., Seleme, N., Mejia-Vergara, A., Moss, H., Padungkiatsagul, T., Lotan, I., Hellmann, M., Stiebel-Kalish, H., Saidha, S., Calabresi, P., Pittock, S. SAGE PUBLICATIONS LTD. 2021: 485-486
  • The Impact of COVID-19 on Neuro-Ophthalmology Office Visits and Adoption of Telemedicine Services. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Moss, H. E., Ko, M. W., Mackay, D. D., Chauhan, D., Gutierrez, K. G., Villegas, N. C., Lai, K. E. 2021; 41 (3): 362-367


    BACKGROUND: The COVID-19 public health emergency (PHE) has significantly changed medical practice in the United States, including an increase in the utilization of telemedicine. Here, we characterize change in neuro-ophthalmic care delivery during the early COVID-19 PHE, including a comparison of care delivered via telemedicine and in office.METHODS: Neuro-ophthalmology outpatient encounters from 3 practices in the United States (4 providers) were studied during the early COVID-19 PHE (March 15, 2020-June 15, 2020) and during the same dates 1 year prior. For unique patient visits, patient demographics, visit types, visit format, and diagnosis were compared between years and between synchronous telehealth and in-office formats for 2020.RESULTS: There were 1,276 encounters for 1,167 patients. There were 30% fewer unique patient visits in 2020 vs 2019 (477 vs 670) and 55% fewer in-office visits (299 vs 670). Compared with 2019, encounters in 2020 were more likely to be established, to occur via telemedicine and to relate to an efferent diagnosis. In 2020, synchronous telehealth visits were more likely to be established compared with in-office encounters.CONCLUSIONS: In the practices studied, a lower volume of neuro-ophthalmic care was delivered during the early COVID-19 public health emergency than in the same period in 2019. The type of care shifted toward established patients with efferent diagnoses and the modality of care shifted toward telemedicine.

    View details for DOI 10.1097/WNO.0000000000001356

    View details for PubMedID 34415270

  • Gender Gap in Neurology Research Authorship (1946-2020). Frontiers in neurology Nguyen, A. X., Yoffe, L., Li, A., Trinh, X. V., Kurian, J., Moss, H. E., Wu, A. Y. 2021; 12: 715428


    Gender disparity in the field of neurology impedes scientific advancements and innovations. In 2018, 45.0% of neurology and neurological subspecialty residents were women. Despite a notable rise in the proportion of women neurologists over the past decades, inequalities regarding publication proportions between men and women persist in the field. This cohort study examines authorship trends in articles published in 155 international neurology journals, identified as those listed in the annual Journal Citation Reports' "Clinical Neurology" section. Authors' names, authorship positions and countries of affiliation were extracted from PubMed for indexed articles published from 1946 to 2020. Gender-API (a validated and highly accurate application program interface) assigned binary genders to authors. Author gender proportions were compared across subspecialties, authorship position and years. In 303,385 unique articles, 1,663,036 total authors were identified of which 34.1% were women. Neuroradiology demonstrated the lowest proportion of women authors (21.3%), while neurogenetics displayed the highest (44.5%). In articles with multiple authors, both men and women last authors were more likely to publish with a male first author, though this was significantly more pronounced for men last authors (1.86 vs. 1.08; p < 0.001). From 2002 to 2020, women remained in the minority of last (24.6%), first (36.2%), and middle author positions (35.8%). The authorship gender distribution in neurological journals neither reflects the gender proportion of neurologists in the field overall nor in any subspecialty examined. We also find a tendency for senior and junior authors of the same gender to publish together which perpetuates authorship inequity. Further work is needed to identify underlying causes so that interventions might be developed to improve authorship diversity.

    View details for DOI 10.3389/fneur.2021.715428

    View details for PubMedID 34497579

    View details for PubMedCentralID PMC8419229

  • Factors Associated With Increased Emergency Department Utilization in Patients With Acute Optic Neuritis. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Muro-Fuentes, E. A., Moss, H. E. 2021


    BACKGROUND: Symptoms of acute vision loss and eye pain may lead patients with optic neuritis to seek care in the emergency department (ED). Given the availability of lower cost alternatives for providing medical care for optic neuritis, this study aimed to identify factors associated with higher ED utilization.METHODS: Subjects with acute optic neuritis were identified through a chart review of adults with International Classification of Diseases-9 (ICD-9) or ICD-10 codes for optic neuritis with corresponding gadolinium contrast enhancement of the optic nerve on MRI in the medical record research repository of a tertiary care institution. Subjects were grouped based on the number of ED visits (0-1 and 2-3) within 2 months of either ICD code or MRI. Demographics, characteristics of disease presentation, type and location of medical care, testing (chest imaging, lumbar puncture, optical coherence tomography, spine MRI, visual field, and laboratory tests), treatment, provider specialty of follow-up visits, and duration of care were extracted from the medical record.RESULTS: Of 30 acute optic neuritis subjects (age 41 ± 16 years, range 18-76, 53% [16/30] female), 19 had 0-1 ED visit and 11 had 2-3 ED visits. Most subjects were Caucasian, non-Hispanic (47%), followed by Asian (23%), Hispanic/Latino (17%), Black (10%), and others (3%). Subjects had an initial clinical encounter primarily in the outpatient setting (63%) as compared with the ED (37%). The median time from symptom onset to initial clinical encounter was 4 days with a range of 0-13. Subjects were mostly insured through a private insurance (60%), followed by Medicare/Medicaid (23%) and uninsured (17%). Fewer ED visits were associated with an initial clinical encounter in an outpatient setting (P = 0.02, chi-square), but not residential distance from the hospital or insurance type. Subjects with a higher number of ED visits were more likely to be of Hispanic/Latino ethnicity (P = 0.047, Fisher exact). There was no significant difference in the ophthalmic, radiologic, or laboratory testing performed in both groups. Both groups presented in a similar time frame with similar symptoms and clinical signs. Treatment was similar in both groups.CONCLUSIONS: Subjects with their first clinical encounter for optic neuritis in the ED had more visits to the ED overall when compared with those first seen in an outpatient setting and thus strategies aimed at facilitating outpatient care may help reduce unnecessary ED visits, although some, such as insurance status, may be difficult to modify. Further study in a larger sample is needed to refine these observations.

    View details for DOI 10.1097/WNO.0000000000001294

    View details for PubMedID 34224527

  • Optic chiasm involvement in AQP-4 antibody-positive NMO and MOG antibody-associated disorder. Multiple sclerosis (Houndmills, Basingstoke, England) Tajfirouz, D., Padungkiatsagul, T., Beres, S., Moss, H. E., Pittock, S., Flanagan, E., Kunchok, A., Shah, S., Bhatti, M. T., Chen, J. J. 2021: 13524585211011450


    BACKGROUND: Optic neuritis (ON) is often the presenting symptom in inflammatory central nervous system demyelinating disorders.OBJECTIVE: To compare the frequency and pattern of optic chiasm involvement in patients with aquaporin-4-immunoglobulin G (AQP4-IgG)-associated ON to patients with myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG)-associated ON.METHODS: Retrospective review of all patients evaluated at Mayo Clinic, Stanford University and Ramathibodi Hospital who were found to have: (1) ON, (2) either MOG-IgG or AQP4-IgG by cell-based assay, and (3) magnetic resonance imaging (MRI) at the time of ON. MRI was reviewed for contrast enhancement of the optic chiasm and the pattern of involvement.RESULTS: One hundred and fifty-four patients (74 AQP4-IgG and 80 MOG-IgG) were included. Among patients with AQP4-IgG-ON, 20% had chiasmal involvement, compared with 16% of patients with MOG-IgG-ON (p = 0.66). In patients with chiasmal involvement, longitudinally extensive optic nerve enhancement (from orbit extending to chiasm) was identified in 54% of MOG-IgG-ON patients, compared with 7% of AQP4-IgG-ON patients (p = 0.01).CONCLUSION: Chiasmal involvement of MOG-IgG-ON and AQP4-IgG-ON occur at more similar frequencies than previously reported. Furthermore, MOG-IgG-ON chiasmal involvement is more likely to be part of a longitudinally extensive optic nerve lesion.

    View details for DOI 10.1177/13524585211011450

    View details for PubMedID 33975499

  • Neuro-Ophthalmic Emergencies. Neurologic clinics Spiegel, S. J., Moss, H. E. 2021; 39 (2): 631–47


    Neuro-ophthalmic emergencies include optic nerve, central visual pathway, and ocular motility disorders that, if not identified and treated promptly, may lead to permanent vision loss, other significant morbidity, or mortality. This article provides a framework for approaching patients with neuro-ophthalmic symptoms and reviews the presentation, evaluation, and treatment of select emergent conditions that can cause them. Emergent causes of blurry vision, transient vision loss, papilledema, and diplopia, including giant cell arteritis, cardioembolic disease, and aggressive infection, are discussed.

    View details for DOI 10.1016/j.ncl.2021.01.004

    View details for PubMedID 33896536

  • Factors Associated with Adherence to Followup Recommendations in Neuro-Ophthalmology Aziz, R., Moss, H. LIPPINCOTT WILLIAMS & WILKINS. 2021
  • Functional Vision Disorders in adults: A paradigm and nomenclature shift for ophthalmology. Survey of ophthalmology Raviskanthan, S., Wendt, S., Ugoh, P. M., Mortensen, P. W., Moss, H. E., Lee, A. G. 2021


    Vision loss with clinical findings that are incompatible with the symptoms and recognized neurological or ophthalmic conditions is a common presentation of patients to neurologists, ophthalmologists, and neuro-ophthalmologists. The accepted terminology to describe such patients has evolved over time including functional visual disorder (FVD), non-organic vision loss, non-physiologic vision loss, functional vision loss, psychogenic, psychosomatic, and medically unexplained visual loss. Likewise, attitudes and recommended management options have changed over the years in the fields of psychiatry and neurology. FVD is a diagnosis of inclusion, and it is critical that the diagnosis be made and delivered efficiently and effectively to reduce patient and physician duress. We review the current Diagnostic and Statistical Manual (DSM V) terminology and the prior literature on FVD and describe how the approaches to diagnosis and management have changed. We provide recommendations on the appropriate techniques and diagnostic approach for patients with FVD. We also propose a protocol for consistent and standardized discussion with the patient of the diagnosis of FVD. We believe that the adoption of FVD as both a paradigm and nomenclature shift in ophthalmology will improve patient care.

    View details for DOI 10.1016/j.survophthal.2021.03.002

    View details for PubMedID 33737039

  • Development and Implementation of a Handheld Pupillometer for Detection of Optic Neuropathies. Current eye research Bindiganavale, M. P., Moss, H. E. 2021: 1–4


    Purpose: Quantitative pupillometry has utility in research settings for measuring optic nerve and autonomic function. We configured a portable device to perform quantitative pupillometry with application to detecting unilateral optic neuropathies in the clinical setting. Materials & methods: Light stimuli were delivered, and pupil diameter responses recorded using customized software implemented on a commercial portable electroretinography device. Increasing pupillary constriction occurred with increasing duration and intensity of full field blue light (470nm) stimuli in healthy subjects. Flashes of 1 s dim (50cd/m2) and bright (316cd/m2) blue light were administered to both eyes of subjects with unilateral optic neuropathies (n=10) and controls (n=5). Maximum pupillary constriction (Cmax) for each stimulus was compared between control eyes and optic neuropathy eyes. Cmax for the inter-eye difference curve (Cdiffmax) was compared between control and optic neuropathy subjects. Results: The pupil protocol lasted 15minutes and was well tolerated by subjects. Cmax for bright and dim stimuli was reduced in eyes with optic neuropathy compared to fellow and control eyes (p <.0005 for all). Inter-eye Cdiffmax was larger in optic neuropathy subjects than control subjects for both dim and bright stimuli (p =.002, <0.0005). There was no overlap between groups for Cmax and Cdiffmax for either stimulus. Conclusions: A portable pupillometer was implemented on a commercial portable electroretinography platform and applied in a pilot manner to subjects with and without unilateral optic neuropathies. Optic neuropathy eyes were distinguished from non-optic neuropathy eyes both within and between subjects.

    View details for DOI 10.1080/02713683.2021.1878542

    View details for PubMedID 33541152

  • Head-Down Tilt Bed Rest Studies as a Terrestrial Analog for Spaceflight Associated Neuro-Ocular Syndrome. Frontiers in neurology Ong, J., Lee, A. G., Moss, H. E. 2021; 12: 648958


    Astronauts who undergo prolonged periods of spaceflight may develop a unique constellation of neuro-ocular findings termed Spaceflight Associated Neuro-Ocular Syndrome (SANS). SANS is a disorder that is unique to spaceflight and has no terrestrial equivalent. The prevalence of SANS increases with increasing spaceflight duration and although there have been residual, structural, ocular changes noted, no irreversible or permanent visual loss has occurred after SANS, with the longest spaceflight to date being 14 months. These microgravity-induced findings are being actively investigated by the United States' National Aeronautics Space Administration (NASA) and SANS is a potential obstacle to future longer duration, manned, deep space flight missions. The pathophysiology of SANS remains incompletely understood but continues to be a subject of intense study by NASA and others. The study of SANS is of course partially limited by the small sample size of humans undergoing spaceflight. Therefore, identifying a terrestrial experimental model of SANS is imperative to facilitate its study and for testing of preventative measures and treatments. Head-down tilt bed rest (HDTBR) on Earth has emerged as one promising possibility. In this paper, we review the HDTBR as an analog for SANS pathogenesis; the clinical and imaging overlap between SANS and HDTBR studies; and potential SANS countermeasures that have been or could be tested with HDTBR.

    View details for DOI 10.3389/fneur.2021.648958

    View details for PubMedID 33841315

  • Retinal Vein Changes as a Biomarker to Guide Diagnosis and Management of Elevated Intracranial Pressure. Frontiers in neurology Moss, H. E. 2021; 12: 751370


    Retinal vein changes, which can be observed on clinical exam or ophthalmic imaging, are promising non-invasive biomarkers for elevated intracranial pressure (ICP) as a complement to other markers of high ICP including optic nerve head swelling. Animal and human studies have demonstrated increase in retinal vein pressure associated with elevated ICP mediated by increase in cerebral venous pressure, compression of venous outflow by elevated cerebral spinal fluid pressure in the optic nerve sheath, and compression of venous outflow by optic nerve head swelling. Retinal vein pressure can be estimated using ophthalmodynamometry. Correlates of retinal vein pressure include spontaneous retinal venous pulsations, retinal vein diameter, and retinal vein tortuosity. All of these have potential for clinical use to diagnose and monitor elevated ICP. Challenges include diagnostic prediction based on single clinical measurements and accurate assessment of retinal vein parameters in cases where optic nerve head swelling limits visualization of the retinal veins.

    View details for DOI 10.3389/fneur.2021.751370

    View details for PubMedID 34733231

    View details for PubMedCentralID PMC8558235

  • Comparison of Machine Learning Approaches to Improve Diagnosis of Optic Neuropathy Using Photopic Negative Response Measured Using a Handheld Device. Frontiers in medicine Diao, T., Kushzad, F., Patel, M. D., Bindiganavale, M. P., Wasi, M., Kochenderfer, M. J., Moss, H. E. 1800; 8: 771713


    The photopic negative response of the full-field electroretinogram (ERG) is reduced in optic neuropathies. However, technical requirements for measurement and poor classification performance have limited widespread clinical application. Recent advances in hardware facilitate efficient clinic-based recording of the full-field ERG. Time series classification, a machine learning approach, may improve classification by using the entire ERG waveform as the input. In this study, full-field ERGs were recorded in 217 eyes (109 optic neuropathy and 108 controls) of 155 subjects. User-defined ERG features including photopic negative response were reduced in optic neuropathy eyes (p < 0.0005, generalized estimating equation models accounting for age). However, classification of optic neuropathy based on user-defined features was only fair with receiver operating characteristic area under the curve ranging between 0.62 and 0.68 and F1 score at the optimal cutoff ranging between 0.30 and 0.33. In comparison, machine learning classifiers using a variety of time series analysis approaches had F1 scores of 0.58-0.76 on a test data set. Time series classifications are promising for improving optic neuropathy diagnosis using ERG waveforms. Larger sample sizes will be important to refine the models.

    View details for DOI 10.3389/fmed.2021.771713

    View details for PubMedID 34926514

  • Novel Foveal Features Associated With Vision Impairment in Multiple Sclerosis. Investigative ophthalmology & visual science Hargrave, A., Sredar, N., Khushzad, F., Yarp, J., Tomczak, A., Han, M., Kipp, L., Dubra, A., Moss, H. E. 2021; 62 (12): 27


    To characterize scattering and hyperreflective features in the foveal avascular zone of people with multiple sclerosis (MS) using adaptive optics scanning laser ophthalmoscopy (AOSLO) and to evaluate their relationship with visual function and MS disease characteristics.Twenty subjects with MS underwent confocal reflectance and non-confocal split-detection AOSLO foveal imaging. Peripapillary retinal nerve fiber layer thickness was measured using optic nerve optical coherence tomography. Blood pressure, intraocular pressure (IOP), and best-corrected high-contrast visual acuity (HCVA) and low-contrast visual acuity (LCVA) were measured. AOSLO images were graded to determine the presence and characteristics of distinct structures.Two distinct structures were seen in the avascular zone of the foveal pit. Hyperreflective puncta, present in 74% of eyes, were associated with IOP and blood pressure. Scattering features, observed in 58% of eyes, were associated with decreased HCVA and LCVA, as well as increased MS duration and disability, but were not associated with retinal nerve fiber layer thickness. Hyperreflective puncta and scattering features were simultaneously present in 53% of eyes.Hyperreflective puncta were associated with parameters affecting ophthalmic perfusion, but they were not associated with MS disease parameters. Scattering features were associated with parameters corresponding to advanced MS, suggesting that they may be related to disease progression. Scattering features were also correlated with reduced visual function independent from ganglion cell injury, suggesting the possibility of a novel ganglion cell-independent mechanism of impaired vision in people with MS.

    View details for DOI 10.1167/iovs.62.12.27

    View details for PubMedID 34581726

  • Should Lumbar Puncture Be Required to Diagnose Every Patient With Idiopathic Intracranial Hypertension? Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Moss, H. E., Margolin, E. A., Lee, A. G., Van Stavern, G. P. 2021; 41 (3): 379-384

    View details for DOI 10.1097/WNO.0000000000001373

    View details for PubMedID 34415272

  • Validity of International Classification of Diseases Codes for Identifying Neuro-Ophthalmic Disease in Large Data Sets: A Systematic Review. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Hamedani, A. G., De Lott, L. B., Deveney, T., Moss, H. E. 2020; 40 (4): 514–19


    BACKGROUND: Administrative health claims data have been used for research in neuro-ophthalmology, but the validity of International Classification of Diseases (ICD) codes for identifying neuro-ophthalmic conditions is unclear.EVIDENCE ACQUISITION: We performed a systematic literature review to assess the validity of administrative claims data for identifying patients with neuro-ophthalmic disorders. Two reviewers independently reviewed all eligible full-length articles and used a standardized abstraction form to identify ICD code-based definitions for 9 neuro-ophthalmic conditions and their sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). A quality assessment of eligible studies was also performed.RESULTS: Eleven articles that met criteria for inclusion are as follows: 3 studies of idiopathic intracranial hypertension (PPV 54%-91% and NPV 74%-85%), 2 studies of giant cell arteritis (sensitivity 30%-96% and PPV 94%), 3 studies of optic neuritis (sensitivity 76%-99%, specificity 83%-100%, PPV 25%-100%, and NPV 98%-100%), 1 study of neuromyelitis optica (sensitivity 60%, specificity 100%, PPV 43%-100%, and NPV 98%-100%), 1 study of ocular motor cranial neuropathies (PPV 98%-99%), and 2 studies of myasthenia gravis (sensitivity 53%-97%, specificity 99%-100%, PPV 5%-90%, and NPV 100%). No studies met eligibility criteria for nonarteritic ischemic optic neuropathy, thyroid eye disease, and blepharospasm. Approximately 45.5% provided only one measure of diagnostic accuracy. Complete information about the validation cohorts, inclusion/exclusion criteria, data collection methods, and expertise of those reviewing charts for diagnostic accuracy was missing in 90.9%, 72.7%, 81.8%, and 36.4% of studies, respectively.CONCLUSIONS: Few studies have reported the validity of ICD codes for neuro-ophthalmic conditions. The range of diagnostic accuracy for some disorders and study quality varied widely. This should be taken into consideration when interpreting studies of neuro-ophthalmic conditions using administrative claims data.

    View details for DOI 10.1097/WNO.0000000000000971

    View details for PubMedID 33197163

  • More rapid recovery and improved outcome with early steroid therapy in mog-igg associated optic neuritis Chen, J., Flanagan, E., Bhatti, M., Jamali, S., Kunchok, A., Eggenberger, E., Acierno, M., Arnold, A., Seleme, E., Meija-Vergara, A., Moss, H., Padungkiatsagul, T., Stiebel-Kalish, H., Pittock, S. SAGE PUBLICATIONS LTD. 2020: 466-467
  • Numerical Investigation on the Role of Mechanical Factors Contributing to Globe Flattening in States of Elevated Intracranial Pressure. Life (Basel, Switzerland) Mehr, J. A., Moss, H. E., Hatami-Marbini, H. 2020; 10 (12)


    Flattening of the posterior eye globe in the magnetic resonance (MR) images is a sign associated with elevated intracranial pressure (ICP), often seen in people with idiopathic intracranial hypertension (IIH). The exact underlying mechanisms of globe flattening (GF) are not fully known but mechanical factors are believed to play a role. In the present study, we investigated the effects of material properties and pressure loads on GF. For this purpose, we used a generic finite element model to investigate the deformation of the posterior eyeball. The degree of GF in numerical models and the significance of different mechanical factors on GF were characterized using an automated angle-slope technique and a statistical measure. From the numerical models, we found that ICP had the most important role in GF. We also showed that the angle-slope graphs pertaining to MR images from five people with high ICP can be represented numerically by manipulating the parameters of the finite element model. This numerical study suggests that GF observed in IIH patients can be accounted for by the forces caused by elevation of ICP from its normal level, while material properties of ocular tissues, such as sclera (SC), peripapillary sclera (PSC), and optic nerve (ON), would impact its severity.

    View details for DOI 10.3390/life10120316

    View details for PubMedID 33260780

  • Telemedicine for neuro-ophthalmology: challenges and opportunities. Current opinion in neurology Liu, Y. A., Ko, M. W., Moss, H. E. 2020


    PURPOSE OF REVIEW: Telemedicine for neuro-ophthalmology (tele-neuro-ophthalmology) has the potential to increase access to neuro-ophthalmic care by improving efficiency and decreasing the need for long-distance travel for patients. Requirements for decreased person-to-person contacts during the COVID-19 pandemic accelerated adoption of tele-neuro-ophthalmology. This review highlights the challenges and opportunities with tele-neuro-ophthalmology.RECENT FINDINGS: Tele-neuro-ophthalmology programs can be used for triage, diagnostic consultation, and long-term treatment monitoring. Formats include telephone appointments, interprofessional collaborations, remote data interpretation, online asynchronous patient communication, and video visits. Barriers to long-term implementation of tele-neuro-ophthalmology arise from data quality, patient engagement, workflow integration, state and federal regulations, and reimbursement. General neurologists may collaborate with local eye care providers for ophthalmic examination, imaging, and testing to facilitate efficient and effective tele-neuro-ophthalmology consultation.SUMMARY: Tele-neuro-ophthalmology has tremendous potential to improve patient access to high-quality cost-effective neuro-ophthalmic care. However, many factors may impact its long-term sustainability.

    View details for DOI 10.1097/WCO.0000000000000880

    View details for PubMedID 33230033

  • MRI findings as markers of idiopathic intracranial hypertension. Current opinion in neurology Barkatullah, A. F., Leishangthem, L., Moss, H. E. 2020


    PURPOSE OF REVIEW: Negative findings on neuroimaging are part of the diagnostic criteria for idiopathic intracranial hypertension (IIH), a syndrome characterized by increased intracranial pressure (ICP). Some positive neuroimaging findings are associated with increased ICP, but their role in diagnosis of IIH has not been established. We provide an overview of these findings and their relevance for diagnosis of raised intracranial pressure.RECENT FINDINGS: MRI acquisition techniques have significantly improved in the last few decades leading to better characterization of the intracranial changes associated with IIH, including empty sella turcica, optic nerve tortuosity, distension of the optic nerve sheath, posterior globe flattening, slit-like ventricles, and venous sinus stenosis. These may be MRI biomarkers of increased ICP. Prevalence difference between people with and without increased ICP, and reversibility of these MRI findings following treatment of increased ICP inform evaluation of their diagnostic potential.SUMMARY: MRI and magnetic resonance venography findings are important tools in the diagnosis of IIH. Empty sella turcica, optic nerve protrusion, distension of the optic nerve sheath, optic nerve tortuosity, posterior globe flattening, and transverse sinus stenosis have been found to be the most promising diagnostic markers for IIH, although absence of these findings does not rule out the diagnosis.

    View details for DOI 10.1097/WCO.0000000000000885

    View details for PubMedID 33230036

  • Retinal Diseases that Can Masquerade as Neurological Causes of Vision Loss. Current neurology and neuroscience reports Padungkiatsagul, T., Leung, L., Moss, H. E. 2020; 20 (11): 51


    PURPOSE OF REVIEW: This review aims to discuss retinal diseases that may masquerade as neurological causes of vision loss and highlights modern ophthalmic ancillary testing that can help to establish these diagnoses.RECENT FINDINGS: Retinal diseases with signs and symptoms overlapping with neurological causes of vision loss include central serous chorioretinopathy, retinal ischemia, acute macular neuroretinopathy, Acute zonal occult outer retinopathy (AZOOR) complex diseases, paraneoplastic retinopathy, retinal dystrophy, and toxic retinopathy. Diagnosis is facilitated by electrophysiologic studies and multimodal ophthalmic imaging including optical coherence tomography and fundus autofluorescence imaging. Looking into the future, translation of adaptive optics ophthalmoscopy into clinical practice may facilitate early detection of microscopic retinal abnormalities that characterize these conditions. With conventional methods of physical examination, diagnosis of retinal diseases that may masquerade as neurological causes of vision loss can be challenging. Current advance in multimodal ophthalmic imaging along with electrophysiologic studies enhances the provider's ability to make early diagnosis and monitor progression of these conditions.

    View details for DOI 10.1007/s11910-020-01071-1

    View details for PubMedID 32930896

  • Higher Cortical Dysfunction Presenting as Visual Symptoms in Neurodegenerative Diseases. Frontiers in neurology Liu, Y., Pelak, V. S., van Stavern, G., Moss, H. E. 2020; 11: 679


    Introduction: As the population ages, increasing prevalence of neurodegenerative diseases will have profound implications for the health care system. Recognizing visual symptoms from neurodegenerative diseases can be challenging, especially in the presence of co-existing eye diseases. Methods: A seven-question survey was completed by attendees at the "neurodegenerative diseases in neuro-ophthalmology" symposium during the 2017 North American Neuro-ophthalmology Society annual meeting using a web-based audience response system. Content included demographics, patient prevalence, and perceived barriers. Results: Fifty-five practicing neuro-ophthalmologists (thirty-three ophthalmology-trained, twenty-two neurology-trained) participated in the survey. Twenty (36%) had <5 years of experience, and 19 (32%) had >15 years of experience. Forty-one (75%) reported seeing patients more than five half-day/week. Thirty (55%) reported that at least 1 of 10 or 1 of 20 new patients referred have a prior diagnosis of a neurodegenerative disease. Twenty-one (40%) of the respondents reported attributing visual complaints to higher order effects in at least 25% of patients with a prior diagnosis of neurodegenerative disease vs. five (9%) without a prior diagnosis. For those diagnosed with neurodegenerative disease by the neuro-ophthalmologist, reasons for referral were unknown cause of visual symptom (56%), to confirm diagnosis and/or treat visual complaint due to neurodegeneration (29%), and functional disorder (5%). Perceived barriers to diagnosing visual dysfunction due to neurodegenerative disease included difficulty making a referral to neuropsychologists or behavioral neurologists (73%), lack of time for in-depth assessment (62%), lack of tools to assess visual dysfunction due to neurodegenerative disease (40%), and lack of knowledge about presenting signs and symptoms (31%). Conclusion: Visual symptoms from neurodegenerative disease in patients with and without prior diagnoses of neurodegenerative disease are evaluated by neuro-ophthalmologists. Lack of time, resources, and knowledge are barriers to diagnosis. A larger study is warranted to guide programs to improve diagnosis of visual consequences of neurodegenerative disease.

    View details for DOI 10.3389/fneur.2020.00679

    View details for PubMedID 32903759

    View details for PubMedCentralID PMC7438888

  • Differences in Clinical Features of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Optic Neuritis in White and Asian Race. American journal of ophthalmology Padungkiatsagul, T., Chen, J. J., Jindahra, P., Akaishi, T., Takahashi, T., Nakashima, I., Takeshita, T., Moss, H. E. 2020


    PURPOSE: To determine whether clinical features and visual outcomes of myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-ON) differ between subjects who are White compared with those who are Asian.DESIGN: Multicenter retrospective cohort.METHODS: SETTING: Multicenter.PATIENT: 153 subjects who are White or Asian with history of adult-onset(age≥18years-old) optic neuritis and positive MOG-IgG serology by cell-based assay were enrolled from two unpublished cohorts(January 2017-November 2019) and nine published cohorts with case-level data available(2012-2018). Subjects with alternative etiologies of demyelinating disease and positive or lack of AQP-4-IgG serology result were excluded.MAIN OUTCOME MEASURES: Clinical features and final visual outcomes.RESULTS: One hundred and fifty-three subjects who are White(n=80) or Asian(n= 73) were included. 93(61%) were female, mean age of onset was 40.8±14.9 years-old, median follow up was 35.2(range1-432) months, which were all similar between White and Asian subjects. Subjects who are White were more likely to have recurrent optic neuritis(57(71%) vs. 20(27%);p=0.001) and extra-optic nerve manifestations(35(44%) vs. 8(11%);p=0.001). Optic disc swelling, neuroimaging findings, presenting visual acuity, treatment and final visual acuity did not differ according to subjects' race. Despite the high prevalence of severe visual loss(<20/200) during nadir, the majority of subjects had good recovery of visual acuity(>20/40) at final examination(51(66%) of 77 subjects who are White vs. 52(74%) of 70 subjects who are Asian).CONCLUSION: Subjects with MOG-ON who are White were more likely to have recurrent disease and extra-optic nerve manifestations. Visual outcomes were similar between subjects who are White and those who are Asian.

    View details for DOI 10.1016/j.ajo.2020.07.008

    View details for PubMedID 32681910

  • A Tearfully Painful Darkness. Survey of ophthalmology Leishangthem, L., Beres, S., Moss, H. E., Chen, J. 2020


    A 70-year-old woman presented with new onset of left eye and facial pain. Ophthalmic and neurological examinations, MRI brain, ESR and CRP were unrevealing. A few days later she developed vision loss in her left eye. Exam revealed decreased visual acuity with a relative afferent pupillary defect in the left eye, and a diffuse mild swelling of the left optic nerve head. Repeat MRI showed T2 hyperintensity and enhancement of the intraorbital optic nerve and surrounding tissues with no other intracranial abnormalities. Serum studies showed elevated myelin oligodendrocyte glycoprotein (MOG) IgG titer. She was treated with IV methylprednisolone 1000mg daily for 3 days and was discharged on prolonged prednisone taper with return of vision to baseline.

    View details for DOI 10.1016/j.survophthal.2020.06.002

    View details for PubMedID 32540257

  • Acute Inflammatory Optic Neuritis Associated with a Self-Taper of Oral Prednisone in a Patient Taking Adalimumab. Neuro-ophthalmology (Aeolus Press) Komandur, A., MacIntosh, P., Moss, H. 2020; 44 (3): 186–89


    Therapies, such as adalimumab, aimed at inhibiting the pro-inflammatory cytokine "tumour necrosis factor" (TNF) are effective and are frequently used in combination with non-biologic disease-modifying anti-rheumatic drugs to treat rheumatoid arthritis (RA) and other autoimmune diseases. Some reports indicate that, rarely, demyelinating CNS disorders such as optic neuritis can present in association with therapy initiation, whilst others suggest that there is no association between the two. Oral corticosteroids such as prednisone, though similarly effective in the treatment of inflammatory or auto-immune conditions, can be associated with adverse effects upon their discontinuation or tapering. We present a patient who developed an acute inflammatory optic neuropathy shortly after a self-taper of oral prednisone while being treated with adalimumab for RA, and discuss the challenge of deciding whether or not to halt anti-TNF therapy.

    View details for DOI 10.1080/01658107.2019.1566386

    View details for PubMedID 32395171

  • Optical coherence tomography use in idiopathic intracranial hypertension. Annals of eye science Malhotra, K., Padungkiatsagul, T., Moss, H. E. 2020; 5


    Idiopathic intracranial hypertension (IIH) is a condition in which elevated pressure in the cerebrospinal fluid can lead to optic nerve head (ONH) dysfunction and subsequent visual impairment. Physicians are currently limited in their ability to monitor and manage this condition, as clinical symptoms and exam findings are often delayed in response to changes in intracranial pressure. In order to find other biomarkers of disease, researchers are using imaging modalities such as optical coherence tomography (OCT) to observe microscopic changes in the eye in this condition. OCT can create 2-dimensional and 3-dimensional high definition images of the retina of the ONH and has been used to study various conditions such as glaucoma and multiple sclerosis. Numerous studies have used OCT in IIH as well, and they have shown that certain retinal layers and the ONH change in thickness and shape in both the short and long term with intracranial pressure changes. OCT is a promising modality for clinical and scientific evaluation of IIH as it is a noninvasive and practical tool to obtain in depth images. This review will discuss how OCT can be used to assess a patient with IIH, both before and after treatment, along with its limitations and future applications.

    View details for DOI 10.21037/aes.2019.12.06

    View details for PubMedID 32405617

  • Randomized Controlled Trials: A Primer for Neuro-Ophthalmologists. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Moss, H. E., Cao, J., Pineles, S. L. 2020; 40 (1): 3–7

    View details for DOI 10.1097/WNO.0000000000000873

    View details for PubMedID 32040067

  • Retinal vessel diameter changes after 6 months of treatment in the Idiopathic Intracranial Hypertension Treatment Trial. The British journal of ophthalmology Moss, H. E., Hollar, R. A., Fischer, W. S., Feldon, S. E. 2020


    BACKGROUND/AIMS: Prior studies support an association between increased retinal venule diameter and elevated intracranial pressure (ICP). The purpose of this study was to test the hypothesis that retinal venule diameters decrease in association with long-term therapy for high ICP in subjects with idiopathic intracranial hypertension (IIH).METHODS: This is a retrospective analysis of multicentre randomised controlled trial data. Standardised procedures were used to measure area of optic nerve head elevation (ONHA) and diameters of 4 arterioles and 4 venules 2.7mm from the optic disc centre on fundus photos collected at baseline and after 6 months of randomised treatment with placebo+dietor acetazolamide+diet in subjects participating in the IIH Treatment Trial (IIHTT) (n=115). Change in arteriole (Da) and venule (Dv) diameters from baseline to 6 months was studied as a function of IIH, haemodynamic and demographic variables.RESULTS: Dv decreased following 6 months of therapy (8.1m, 5.9%,p<0.0005) but Da did not change. Dv change was associated with ONHA change (p<0.0005, r=0.47) and this association persisted in multiple variable models.CONCLUSIONS: Retinal venule diameter decreased, and arteriole diameter did not change in association with treatment for elevated ICP with a weight loss intervention and placebo or acetazolamide in IIHTT participants. Further study is needed to determine how retinal vessel measurements can be combined with other clinical observations to inform disease management.

    View details for DOI 10.1136/bjophthalmol-2019-314648

    View details for PubMedID 31949093

  • Application of Hemifield Visual Electrophysiology to Diagnose Functional Vision Loss. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Moss, H. E., Jabbehdari, S. n. 2020


    Neuro-ophthalmologists frequently see patients who are experiencing vision loss not accounted for by a neuro-ophthalmic disorder. In this article, we describe a case of binasal hemianopia in an otherwise healthy 65-year-old woman who was initially diagnosed with glaucoma but ultimately proved to have functional visual loss. This diagnosis was made by confirming by confirming normal visual pathway function using hemifield visual-evoked potential studies.

    View details for DOI 10.1097/WNO.0000000000000882

    View details for PubMedID 32028454

  • Anterior Optic Neuropathy in a Patient With Cyclical Fevers. JAMA ophthalmology Jabbehdari, S. n., Lakusta, A. R., Moss, H. E. 2020

    View details for DOI 10.1001/jamaophthalmol.2019.4987

    View details for PubMedID 31944233

  • Peri-operative ischemic optic neuropathy in spinal fusion surgery: Validating a predictive scoring system. The spine journal : official journal of the North American Spine Society Moss, H. E., Xiao, L. n., Shah, S. H., Chen, Y. F., Joslin, C. n., Roth, S. n. 2020

    View details for DOI 10.1016/j.spinee.2020.05.554

    View details for PubMedID 32512229

  • Ultra-widefield Fundus Image in Oculocutaneous Albinism. JAMA ophthalmology Padungkiatsagul, T. n., Leishangthem, L. n., Moss, H. E. 2020; 138 (9): e200864

    View details for DOI 10.1001/jamaophthalmol.2020.0864

    View details for PubMedID 32910139

  • Evolution of the Journal of Neuro-Ophthalmology and the Clinical Ophthalmology Literature: A 20-Year Retrospective. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Blankshain, K. D., Joslin, C. E., Pineles, S. L., Moss, H. E. 2020; 40 (2): 141–43

    View details for DOI 10.1097/WNO.0000000000000891

    View details for PubMedID 32384416

  • The Case-Control Study in Neuro-Ophthalmology. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Hamedani, A. G., Pineles, S. L., Moss, H. E. 2020; 40 (2): 144–47

    View details for DOI 10.1097/WNO.0000000000000970

    View details for PubMedID 32384417

  • Predictive model of Ischemic optic neuropathy in spinal fusion surgery using a longitudinal medical claims database. The spine journal : official journal of the North American Spine Society Moss, H. E., Xiao, L. n., Shah, S. H., Chen, Y. F., Joslin, C. n., Roth, S. n. 2020


    Perioperative ischemic optic neuropathy (ION) is a devastating complication of spinal fusion surgery.To develop predictive models of this blinding condition using longitudinal medical administrative claims databases, which provide temporal sequence of perioperative ischemic optic neuropathy and potential risk factors.Nested case control study PATIENT SAMPLE: Participants in Cliniformatics® Data Mart medical claims database (2007-2017) with hospitalization involving lumbar or thoracic spinal fusion surgery and no history of ION.Peri-operative ION (or not) during hospitalization for lumbar or thoracic spinal fusion surgery.65 ION cases and 106,871 controls were identified. Matched controls (n=211) were selected based on year of surgery and zip code. Chronic and peri-operative variables were assigned based on medical claims codes. Least absolute shrinkage and selection (LASSO) penalized conditional logistic regression with ten-fold cross validation was used to select variables for the optimal predictive model from the subset of variables with p < 0.15 between cases and matched controls (unadjusted conditional logistic regression). Receiver operating characteristic (ROC) curves were generated for the strata-independent matched and full sample.The predictive model included age 57-65 years, male gender, diabetes with and without complications, chronic anemia, hypertension, heart failure, carotid stenosis, perioperative hemorrhage and perioperative organ damage in the predictive model. Area under ROC curve was 0.75 (95% CI: 0.68, 0.82) for the matched sample and 0.72 (95% CI: 0.66, 0.78) for the full sample.This predictive model for ION in spine fusion considering chronic conditions and perioperative conditions is unique to date in its use of longitudinal medical claims data, inclusion of ICD-10 codes and study of ophthalmic conditions as risk factors. Similar to other studies of this condition the multivariable model included age, male gender, peri-operative organ damage and peri-operative hemorrhage. Hypertension, chronic anemia and carotid artery stenosis were new predictive factors identified by this study.

    View details for DOI 10.1016/j.spinee.2020.11.011

    View details for PubMedID 33248270

  • Steroid-sparing maintenance immunotherapy for MOG-IgG associated disorder. Neurology Chen, J. J., Flanagan, E. P., Bhatti, M. T., Jitprapaikulsan, J. n., Dubey, D. n., Lopez Chiriboga, A. S., Fryer, J. P., Weinshenker, B. G., McKeon, A. n., Tillema, J. M., Lennon, V. A., Lucchinetti, C. F., Kunchok, A. n., McClelland, C. M., Lee, M. S., Bennett, J. L., Pelak, V. S., Van Stavern, G. n., Adesina, O. O., Eggenberger, E. R., Acierno, M. D., Wingerchuk, D. M., Lam, B. L., Moss, H. n., Beres, S. n., Gilbert, A. L., Shah, V. n., Armstrong, G. n., Heidary, G. n., Cestari, D. M., Stiebel-Kalish, H. n., Pittock, S. J. 2020


    Myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) associated disorder (MOGAD) often manifests with recurrent CNS demyelinating attacks. The optimal treatment for reducing relapses is unknown. To help determine the efficacy of long-term immunotherapy in preventing relapse in patients with MOGAD, we conducted a multicenter retrospective study to determine the rate of relapses on various treatments.We determined the frequency of relapses in patients receiving various forms of long-term immunotherapy for MOGAD. Inclusion criteria were history of ≥1 CNS demyelinating attacks, MOG-IgG seropositivity, and immunotherapy for ≥6 months. Patients were reviewed for CNS demyelinating attacks before and during long-term immunotherapy.Seventy patients were included. The median age at initial CNS demyelinating attack was 29 years (range 3-61 years; 33% <18 years), and 59% were female. The median annualized relapse rate (ARR) before treatment was 1.6. On maintenance immunotherapy, the proportion of patients with relapse was as follows: mycophenolate mofetil 74% (14 of 19; ARR 0.67), rituximab 61% (22 of 36; ARR 0.59), azathioprine 59% (13 of 22; ARR 0.2), and IV immunoglobulin (IVIG) 20% (2 of 10; ARR 0). The overall median ARR on these 4 treatments was 0.3. All 9 patients treated with multiple sclerosis (MS) disease-modifying agents had a breakthrough relapse on treatment (ARR 1.5).This large retrospective multicenter study of patients with MOGAD suggests that maintenance immunotherapy reduces recurrent CNS demyelinating attacks, with the lowest ARR being associated with maintenance IVIG therapy. Traditional MS disease-modifying agents appear to be ineffective. Prospective randomized controlled studies are required to validate these conclusions.

    View details for DOI 10.1212/WNL.0000000000009758

    View details for PubMedID 32554760

  • Survey of Telehealth Adoption by Neuro-ophthalmologists During the COVID-19 Pandemic: Benefits, Barriers and Utility. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Moss, H. E., Lai, K. E., Ko, M. W. 2020


    During the COVID-19 pandemic tele-health modalities have come to prominence as a strategy for providing patient care when in-person care provision opportunities are limited. The degree of adoption by neuro-ophthalmologists has not been quantified.Telehealth utilization pre- and peri-COVID-19 was surveyed among practicing neuro-ophthalmologists in and outside the US using an on-line platform. Demographics, perceived benefits, barriers, and utility for different neuro-ophthalmic conditions were collected. Data collection occurred over a 2-week period in May, 2020.208 practicing neuro-ophthalmologists (81.3% US, 50.2% female, age range < 35 to > 65, mode 35-44 years) participated in the survey. Utilization of all telehealth modalities increased from pre-COVID to peri-COVID (video visit 3.9% to 68.3%, p<0.0005, remote interpretation of testing 26.7% to 32.2%, p=0.09, on-line second opinion 7.9% to 15.3%, p=0.001, interprofessional e-consult 4.4% to 18.7%, p<0.0005, McNemar). The majority selected access, continuity, and patient efficiency of care as benefits and data quality as a barrier. Telehealth was felt to be most helpful for conditions relying on history, external exam, and previously collected ancillary testing and not helpful for conditions requiring funduscopic exam.Telehealth modality usage by neuro-ophthalmologists increased during the COVID-19 pandemic. Identified benefits have relevance both during and beyond COVID-19. Further work is needed to address barriers in their current and future states to maintain these modalities as viable care delivery options.

    View details for DOI 10.1097/WNO.0000000000001051

    View details for PubMedID 32639269

  • Reversal of Iris Heterochromia in Adult-Onset Acquired Horner Syndrome. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Padungkiatsagul, T. n., Leishangthem, L. n., Moss, H. E. 2020


    Iris heterochromia is typically seen in association with congenital Horner syndrome. A man in his 40s with congenital iris heterochromia, blue in the right and brown in the left, presented with left-sided Horner syndrome. This was associated with recent change in color of his brown left iris to blue similar to the right iris. This case demonstrates a unique case of adult-onset Horner syndrome with reversal of iris heterochromia.

    View details for DOI 10.1097/WNO.0000000000001089

    View details for PubMedID 32956222

  • Update on the Diagnosis and Treatment of Idiopathic Intracranial Hypertension. Seminars in neurology Ahmad, S. R., Moss, H. E. 2019; 39 (6): 682–91


    Advances in ophthalmic diagnostics and results of interventional clinical trials are shifting diagnosis and management of idiopathic intracranial hypertension (IIH) to be more technology- and evidence-based. In this article, the evidence supporting current diagnostic criteria, evaluation, and medical and surgical management of IIH are reviewed.

    View details for DOI 10.1055/s-0039-1698744

    View details for PubMedID 31847039

  • Big Data Research in Neuro-Ophthalmology: Promises and Pitfalls JOURNAL OF NEURO-OPHTHALMOLOGY Moss, H. E., Joslin, C. E., Rubin, D. S., Roth, S. 2019; 39 (4): 480–86
  • Chiasmal and Postchiasmal Disease. Continuum (Minneapolis, Minn.) Moss, H. E. 2019; 25 (5): 1310-1328


    PURPOSE OF REVIEW: This article reviews the anatomy, symptoms, examination findings, and causes of diseases affecting the optic chiasm, optic tracts, optic radiations, and occipital lobes.RECENT FINDINGS: Modern ophthalmic imaging can be used to monitor the effects of diseases of the optic chiasm and tract on the retinal ganglion cells. It can also be used to visualize transsynaptic degeneration of the anterior visual pathway in the setting of acquired retrogeniculate lesions. Visual prostheses that directly stimulate the occipital lobe are a potential strategy for rehabilitation that is in active clinical trials.SUMMARY: Detecting and characterizing visual deficits due to optic chiasm and retrochiasmal disease are important for the diagnosis, localization, and monitoring of neurologic disease; identifying patient disability; and guiding rehabilitation.

    View details for DOI 10.1212/CON.0000000000000785

    View details for PubMedID 31584539

  • Methods for Quantifying Optic Disc Volume and Peripapillary Deflection Volume Using Radial Optical Coherence Tomography Scans and Association With Intracranial Pressure FRONTIERS IN NEUROLOGY Patel, M., Malhotra, K., Shirazi, Z., Moss, H. E. 2019; 10
  • Methods for Quantifying Optic Disc Volume and Peripapillary Deflection Volume Using Radial Optical Coherence Tomography Scans and Association With Intracranial Pressure. Frontiers in neurology Patel, M. D., Malhotra, K., Shirazi, Z., Moss, H. E. 2019; 10: 798


    Purpose: Papilledema and peripapillary deformation of Bruch's membrane (BM) are associated with elevated intracranial pressure (ICP). We have developed a novel methodology to measure these parameters using a radial optical coherence tomography (OCT) scan pattern and apply this to test the hypothesis that ICP is associated with volumetric features of ophthalmic structures. Methods: 6-radial OCT B-scans centered over the optic nerve head were acquired in 17 subjects (30 eyes) before lumbar puncture with measurement of ICP (range: 10-55 cm H2O). Internal limiting membrane (ILM) and BM were segmented. Three definitions of BM were studied to account for imaging artifact affecting peripapillary BM: connecting rater-identified BM margins(traditional), connecting rater-identified BM 1.6 mm on either side of the ONH(estimated), and excluding BM in the central 3.2 mm of the images(excluded). Optic nerve head volume (ONHV), BM displacement volume (BMDV) and cup volume (CV) were calculated by interpolating between B-scans. Ganglion cell complex volume (GCCV) was measured in the macula. Linear generalized estimating equations (GEE) modeled ONVH, BMDV, and CV as a function of ICP and GCCV. Results: Increased ONHV was associated with elevated ICP for traditional (p = 0.006), estimated (p = 0.003) and excluded (p = 0.05) BM definitions. Decreased BMDV was associated with elevated ICP for traditional (p < 0.0005), estimated (p < 0.0005) and excluded (p = 0.001) definitions. Decreased ONHV was independently associated with decreased GCCV (p = 0.001) and decreased ICP (p = 0.031) in multivariable models. CV was neither associated with ICP nor GCCV in univariate or multivariable models. Conclusions: Elevated ICP is associated with ONHV increase and BMDV decrease, calculated from OCT images accounting for image artifact. Ganglion cell atrophy affects the relationship between ICP and ONHV. OCT derived volumetric measures of the posterior eye may have application as biomarkers for elevated ICP.

    View details for DOI 10.3389/fneur.2019.00798

    View details for PubMedID 31396151

    View details for PubMedCentralID PMC6668216

  • Predicting Risk of Perioperative Ischemic Optic Neuropathy: A Study Using the US National Inpatient Sample Roth, S., Shah, S., Chen, Y., Moss, H., Rubin, D., Joslin, C. ASSOC RESEARCH VISION OPHTHALMOLOGY INC. 2019
  • Perioperative Visual Loss in Cardiac Surgery JOURNAL OF CARDIOTHORACIC AND VASCULAR ANESTHESIA Raphael, J., Moss, H. E., Roth, S. 2019; 33 (5): 1420–29
  • Predicting Risk of Perioperative Ischemic Optic Neuropathy in Spine Fusion Surgery: A Cohort Study Using the National Inpatient Sample. Anesthesia and analgesia Shah, S. H., Chen, Y. F., Moss, H. E., Rubin, D. S., Joslin, C. E., Roth, S. n. 2019


    Ischemic optic neuropathy (ION) is a rare complication of anesthesia and surgery that causes vision loss in spine fusion. We sought to develop a predictive model based on known preoperative risk factors for perioperative ION to guide patient and physician preoperative decision-making.In the National Inpatient Sample (NIS) for 1998-2012, discharges for posterior thoracic, lumbar, and sacral spine fusion were identified and classified by ION status. Variables were selected without weighting via variable clustering using Principal Component Analysis of Mixed Data (PCA-MIX). Hierarchical clustering with 4 clusters was performed, and the variable with largest squared loading in each cluster was chosen. By splitting our sample into a training and testing data set, we developed and internally validated a predictive model. The final model using variables known preoperatively was constructed to allow determination of relative and absolute risk of developing perioperative ION and was tested for calibration and discrimination.The final predictive model based on hierarchical clustering contained 3 preoperative factors, age, male or female sex, and the presence of obstructive sleep apnea (OSA). The predictive model based on these factors had an area under the receiver operating characteristic curve (AUC) of 0.65 and good calibration. A score cutoff of >1 had 100% sensitivity, while score of 3 had 96.5% specificity. The highest estimated absolute risk (844.5/million) and relative risk of ION (46.40) was for a man, age 40-64 years, with OSA.The predictive model could enable screening for patients at higher risk of ION to provide more accurate risk assessment and surgical and anesthetic planning for perioperative ION in spine fusion.

    View details for DOI 10.1213/ANE.0000000000004383

    View details for PubMedID 31490255

  • Association Between Peripapillary Bruch's Membrane Shape and Intracranial Pressure: Effect of Image Acquisition Pattern and Image Analysis Method, a Preliminary Study. Frontiers in neurology Malhotra, K., Patel, M. D., Shirazi, Z., Moss, H. E. 2018; 9: 1137


    Background/Aims: High intracranial pressure (ICP) is associated with changes in peripapillary Bruch's membrane (pBM) shape on optical coherence tomography (OCT) images of the optic nerve head. It is not known if image acquisition pattern and analysis method impact this association. Materials and Methods: Cross sectional OCT scans of the optic nerve head were obtained at six angles using a radial scan pattern in 21 subjects immediately prior to ICP measurement via lumbar puncture. On each image, Bruch's membrane was manually segmented and defined by either 14 or 16 semi-landmarks and either rater identified, or distance identified boundaries. For each of these four image analysis strategies, geometric morphometric analysis identified the first principal component of Bruch's membrane shape for all images and for the set of images taken at each angle. Repeated measures ANOVA of the first principal component magnitude (PC1) for all images assessed for shape difference between image angles. Linear generalized estimating equation models assessed association between angle specific first principal component magnitudes (PC1) and ICP for each angle. Receiver operating characteristic analysis assessed angle specific PC1s' ability to differentiate elevated from normal ICP. Results: The first principal component represented deflection into the vitreous for all scan angles, but quantitatively differed across scan angles (p < 0.005, repeated measures ANOVA). Angle specific first principal components were positively correlated with ICP (p < 0.005 for all angles, generalized estimating equation models). All angle specific first principal components showed excellent ability to classify ICP (area under curve ≥ 0.8 for all). These results were independent from image analysis strategy. Discussion: Though qualitative changes in Bruch's membrane shape are similar regardless of cross-sectional angle of the 2-D OCT scan, they differ quantitatively between OCT scan angles, meaning that pBM is not axially symmetric and therefore PC1 extracted from different 2-D scan angles can't be compared between individuals. However, we do not identify an optimal scan angle for classification of ICP since there is a similarly strong linear relationship between the first principal component of shape and ICP and angle specific first principal components of Bruch's membrane shape showed similarly excellent ability to differentiate elevated from normal ICP. The results support development of Bruch's membrane shape extracted from 2-D cross sectional optic nerve head OCT scans as a biomarker of ICP and emphasize the importance of consistency of scan angle. This is relevant for developing diagnostic protocols that use OCT to detect high ICP states.

    View details for DOI 10.3389/fneur.2018.01137

    View details for PubMedID 30622510

    View details for PubMedCentralID PMC6308322

  • Association Between Peripapillary Bruch's Membrane Shape and Intracranial Pressure: Effect of Image Acquisition Pattern and Image Analysis Method, a Preliminary Study FRONTIERS IN NEUROLOGY Malhotra, K., Patel, M. D., Shirazi, Z., Moss, H. E. 2018; 9
  • Comparison of cross sectional optical coherence tomography images of elevated optic nerve heads across acquisition devices and scan protocols EYE AND VISION Patel, M. D., Khushzad, F., Moss, H. E. 2018; 5
  • Update on Perioperative Ischemic Optic Neuropathy Associated With Non-ophthalmic Surgery FRONTIERS IN NEUROLOGY Roth, S., Moss, H. E. 2018; 9
  • Update on Perioperative Ischemic Optic Neuropathy Associated With Non-ophthalmic Surgery. Frontiers in neurology Roth, S., Moss, H. E. 2018; 9: 557


    Perioperative visual loss (POVL) is a rare, serious complication of non-ophthalmic surgeries. Ischemic optic neuropathy (ION), and retinal arterial occlusion (RAO) are the main causes (1, 2). Less frequent are cortical blindness (3), acute glaucoma (4), and choroidal and vitreous hemorrhage (5). ION is the most common cause for which the neurologist or neuro-ophthalmologist is consulted as it is associated either with a normal ophthalmic exam (posterior ION, PION), or less often, with optic nerve (ON) head swelling (anterior ION, AION). The presumed cause is impaired blood supply to the optic nerve (Figure 1). The most common surgical procedures complicated by ION are cardiac surgery and spinal fusion. Retrospective studies, surveys, and case reports are the basis of most knowledge regarding peri-operative ION (poION), with cohort and case-control studies helping to identify candidate risk factors (6, 7). Animal models have provided insight regarding mechanisms (8). This mini-review is an update on the latest advancements regarding poION in non-ophthalmic surgeries in epidemiological, clinical, and animal studies.

    View details for DOI 10.3389/fneur.2018.00557

    View details for PubMedID 30042726

    View details for PubMedCentralID PMC6048244

  • Retinal Vessel Diameters Change Within 1 Hour of Intracranial Pressure Lowering TRANSLATIONAL VISION SCIENCE & TECHNOLOGY Moss, H. E., Vangipuram, G., Shirazi, Z., Shahidi, M. 2018; 7 (2): 6


    We tested the hypotheses that retinal venule diameter (Dv) is associated with baseline intracranial pressure (ICP) level and that Dv is reduced shortly after ICP lowering.Dv and arteriole diameter (Da) were extracted from scanning laser ophthalmoscopic images in 40 eyes of 20 adult human subjects (10 with and 10 without papilledema) immediately before and after measurement of ICP (range, 10-55 cm H2O) and ICP lowering by cerebrospinal fluid (CSF) drainage via lumbar puncture (LP). Generalized estimating equations (GEE) modeled the relationship between baseline ICP, Da and Dv before LP. Additional GEE modeled the relationship between initial ICP and change in Da and Dv (post-LP - pre-LP) following ICP lowering.Test-retest variability of diameter measurements ranged from 0.1 to 2.9 μm (0.1%-2.72%). Neither Da nor Dv pre-LP was associated with baseline ICP level (P = 0.140 Dv, P = 0.914 Da, GEE). Da and Dv change after ICP lowering was associated with baseline ICP, with vessel diameters increasing with lower baseline ICP and decreasing with elevated initial ICP (P = 0.030 baseline ICP vs. Dv change, P = 0.012 baseline ICP vs. Da change, GEE models).Retina arteriole and venule diameters change immediately following ICP lowering. The direction of change is dependent on the initial ICP; both increased in subjects with high ICP and both decreased in subjects with normal ICP.The relationship between initial ICP and direction of retinal vessel size change following ICP lowering suggests a potential effect of ICP on cerebral and ocular hemodynamics that is relevant when considering the use of retinal vessel measurements as a clinical marker of ICP change.

    View details for PubMedID 29576930

  • Perioperative Retinal Artery Occlusion: Incidence and Risk Factors in Spinal Fusion Surgery From the US National Inpatient Sample 1998-2013. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society Calway, T. n., Rubin, D. S., Moss, H. E., Joslin, C. E., Mehta, A. I., Roth, S. n. 2018; 38 (1): 36–41


    Retinal artery occlusion (RAO) is a rare but devastating complication of spinal fusion surgery. We aimed to determine its incidence and associated risk factors.Hospitalizations involving spinal fusion surgery were identified by searching the National Inpatient Sample, a database of hospital discharges, from 1998 to 2013. RAO cases were identified using ICD-9-CM codes. Using the STROBE guidelines, postulated risk factors were chosen based on literature review and identified using ICD-9-CM codes. Multivariate logistic models with RAO as outcome, and risk factors, race, age, admission, and surgery type evaluated associations.Of an estimated 4,784,275 spine fusions in the United States from 1998 to 2013, there were 363 (CI: 291-460) instances of RAO (0.76/10,000 spine fusions, CI: 0.61-0.96). Incidence ranged from 0.35/10,000 (CI: 0.11-1.73) in 2001-2002 to 1.29 (CI: 0.85-2.08) in 2012-2013, with no significant trend over time (P = 0.39). Most strongly associated with RAO were stroke, unidentified type (odds ratio, OR: 14.33, CI: 4.54-45.28, P < 0.001), diabetic retinopathy (DR) (OR: 7.00, CI: 1.18-41.66, P = 0.032), carotid stenosis (OR: 4.94, CI: 1.22-19.94, P = 0.025), aging (OR for age 71-80 years vs 41-50 years referent: 4.07, CI: 1.69-10.84, P = 0.002), and hyperlipidemia (OR: 2.96, CI: 1.85-4.73, P < 0.001). There was an association between RAO and transforaminal lumbar interbody fusion (OR: 2.95, CI: 1.29-6.75, P = 0.010). RAO was more likely to occur with spinal surgery performed urgently or emergently compared with being done electively (OR: 0.40, CI: 0.23-0.68, P < 0.001).Patient-specific associations with RAO in spinal fusion include aging, carotid stenosis, DR, hyperlipidemia, stroke, and specific types of surgery. DR may serve as an observable biomarker of heightened risk of RAO in patients undergoing spine fusion.

    View details for PubMedID 28665867

    View details for PubMedCentralID PMC5764807

  • Comparison of cross sectional optical coherence tomography images of elevated optic nerve heads across acquisition devices and scan protocols. Eye and vision (London, England) Patel, M. D., Khushzad, F., Moss, H. E. 2018; 5: 17


    Background: Optic nerve head measurements extracted from optical coherence tomography (OCT) show promise for monitoring clinical conditions with elevated optic nerve heads. The aim of this study is to compare reliability within and between raters and between image acquisition devices of optic nerve measurements derived from OCT scans in eyes with varying degrees of optic nerve elevation.Methods: Wide angle line scans and narrow angle radial scans through optic nerve heads were obtained using three spectral domain(SD) OCT devices on 5 subjects (6 swollen optic nerves, 4 normal optic nerves). Three raters independently semi-manually segmented the internal limiting membrane(ILM) and Bruch's membrane(BM) on each scan using customized software. One rater segmented each scan twice. Segmentations were qualitatively and quantitatively compared. Inter-rater, intra-rater and inter-device reliability was assessed for the optic nerve cross sectional area calculated from the ILM and BM segmentations using intraclass correlation coefficients and graphical comparison.Results: Line scans from all devices were qualitatively similar. Radial scans for which frame rate could not be adjusted were of lower quality. Intra-rater reliability for segmentation and optic nerve cross sectional area was better than inter-rater reliability, which was better than inter-device reliability, though all ICC exceeded 0.95. Reliability was not impacted by the degree of optic nerve elevation.Conclusions: SD-OCT devices acquired similar quality scans of the optic nerve head, with choice of scan protocol affecting the quality. For image derived markers, variability between devices was greater than that attributable to inter and intra-rater differences.

    View details for PubMedID 30009195

  • Electroretinography in idiopathic intracranial hypertension: comparison of the pattern ERG and the photopic negative response. Documenta ophthalmologica. Advances in ophthalmology Park, J. C., Moss, H. E., McAnany, J. J. 2018; 136 (1): 45–55


    To evaluate the relationship between electrophysiological measures of retinal ganglion cell (RGC) function in patients who have idiopathic intracranial hypertension (IIH).The pattern electroretinogram (pERG) and photopic negative response (PhNR) were recorded from 11 IIH patients and 11 age-similar controls. The pERG was elicited by a contrast-reversing checkerboard. The PhNR, a slow negative component following the flash ERG b-wave, was recorded in response to a long-wavelength flash presented against a short-wavelength adapting field. The PhNR was elicited using full-field (ffPhNR) and focal macular (fPhNR) stimuli. Additionally, Humphrey visual field mean deviation (HVF MD) was measured and ganglion cell complex volume (GCCV) was obtained by optical coherence tomography.The ffPhNR, fPhNR, and pERG amplitudes were outside of the normal range in 45, 9, and 45% of IIH patients, respectively. However, only mean ffPhNR amplitude was reduced significantly in the patients compared to controls (p < 0.01). The pERG amplitude correlated significantly with HVF MD and GCCV (both r > 0.65, p < 0.05). There were associations between ffPhNR amplitude and HVF MD (r = 0.58, p = 0.06) and with GCCV (r = 0.52, p = 0.10), but these did not reach statistical significance. fPhNR amplitude was not correlated significantly with HVF MD or GCCV (both r < 0.40, p > 0.20).Although the fPhNR is generally normal in IIH, other electrophysiological measures of RGC function, the ffPhNR and pERG, are abnormal in some patients. These measures provide complementary information regarding RGC dysfunction in these individuals.

    View details for PubMedID 29139045

    View details for PubMedCentralID PMC5812802

  • Quantitative Association Between Peripapillary Bruch's Membrane Shape and Intracranial Pressure. Investigative ophthalmology & visual science Gampa, A., Vangipuram, G., Shirazi, Z., Moss, H. E. 2017; 58 (5): 2739-2745


    The purpose of this study was to determine if there is a quantitative relationship between chronic intracranial pressure (ICP) and peripapillary Bruch's membrane (pp-BM) shape and to determine whether change in pp-BM shape can be detected within 1 hour after ICP lowering by lumbar puncture (LP).In this study, 30° nasal-temporal optical coherence tomography B-scans were obtained within 1 hour before and after LP in 39 eyes from 20 patients (age = 23-86 years, 75% female, ICP [opening pressure] = 10-55 cm H2O). A total of 16 semi-landmarks defined pp-BM on each image. Geometric morphometric analysis identified principal components of shape in the image set. Generalized estimating equation models, accounting for within-subject correlation, were used to identify principal components that were associated with chronic ICP (comparing pre-LP images between eyes) and/or acute ICP changes (comparing pre- and post-LP images within eyes). The pp-BM width and anterior pp-BM location were calculated directly from each image and were studied in the same manner.Principal component 1 scalar variable on pre-LP images was associated with ICP (P < 0.0005). Principal component 4 magnitude changed within eyes after LP (P = 0.003). For both principal components 1 and 4, lower ICP corresponded with a more posterior position of pp-BM. Chronic ICP was associated with both pp-BM width (6.81 μm/cm H2O; P = 0.002) and more anterior location of temporal and nasal pp-BM margins (3.41, 3.49 μm/cm H2O; P < 0.0005, 0.002).This study demonstrates a quantitative association between pp-BM shape and chronic ICP level. Changes in pp-BM shape are detectable within 1 hour of lowering ICP. pp-BM shape may be a useful marker for chronic ICP level and acute ICP changes. Further study is needed to determine how pp-BM shape changes relate to clinical markers of papilledema.

    View details for DOI 10.1167/iovs.17-21592

    View details for PubMedID 28549088

    View details for PubMedCentralID PMC5455169

  • The Longitudinal Idiopathic Intracranial Hypertension Trial: Outcomes from Months 6 - 12. American journal of ophthalmology Wall, M. n., Kupersmith, M. J., Thurtell, M. J., Moss, H. E., Moss, E. A., Auinger, P. n. 2017


    To determine whether the beneficial effects of acetazolamide (ACZ) in improving vision at 6 months continues to month 12 in participants of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT).non-randomized clinical study METHODS: In the IIHTT, subjects were randomly assigned to placebo-plus-diet or maximally tolerated dosage of acetazolamide-plus-diet. At 6 months subjects transitioned from study drug to ACZ. This resulted in the following groups 1) ACZ to ACZ; n = 34; 2) placebo to ACZ; n = 35; 3) ACZ to no treatment; n = 16; and 4) placebo to no treatment; n = 11. 96 IIHTT subjects had evaluations at 6 and 12 months. Our main outcome measure was change from month 6 to month 12 in visual field mean deviation with secondary measures being change in papilledema grade, ETDRS scores and quality of life (QoL) measures.The ACZ to ACZ group improved 0.35 dB , p=0.05; placebo subjects with no ACZ improved 0.81 dB MD, p = 0.07 at 12 mos. The other groups improved 0.35 to 0.46 dB MD. Mean improvements in papilledema grade occurred most markedly in the group that exchanged placebo for ACZ (0.91 units, p < 0.001). QoL and headache disability scores showed significant improvements in the placebo group added ACZ.Improvements in MD continued from month 6 to month 12 of the IIHTT in all treatment groups -most marked in the placebo group tapered off study drug. Adding ACZ to the placebo group significantly improved papilledema grade, headache and QoL measures.

    View details for DOI 10.1016/j.ajo.2017.01.004

    View details for PubMedID 28104417

  • Diagnostic Challenge: Sequential Unilateral Cranial Neuropathies Due to Perineural Spread of Carcinoma NEURO-OPHTHALMOLOGY Moss, H. E., MacIntosh, P. W., Slavin, K. V., Collins, J. M. 2017; 41 (4): 227–31


    An 86-year old man developed sequential dysfunction of trigeminal (V1), facial, abducens, trigeminal (v2), oculomotor, and hypoglossal cranial nerves on the right over 20 months. Magnetic resonance imaging (MRI) showed a lesion in the right cavernous sinus. Although there was clinical suspicion that this was related to perineural spread of an extracranial tumour, a primary lesion was not discovered. Stereotactic biopsies of the intracranial lesion were non-diagnostic, and the patient succumbed to his tumour following a period of rapid growth. Postmortem examination showed the intracranial lesion to be a carcinoma with squamous features. This case highlights the challenges of diagnosis of intracranial perineural spread and the potential for transformation from indolent to aggressive tumour behaviour.

    View details for DOI 10.1080/01658107.2017.1304968

    View details for Web of Science ID 000415700200012

    View details for PubMedID 29344066

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  • Cerebral Venous Thrombosis with Papilloedema Secondary to Skull Base Plasmacytoma NEURO-OPHTHALMOLOGY MacIntosh, P. W., Lin, A. Y., Kim, J., Testai, F. D., Moss, H. E. 2017; 41 (5): 284–86


    A 60-year-old woman with history of multiple myeloma was in remission after stem cell transplant 6 years prior. She was undergoing work-up for headaches that were thought to be secondary to a right mastoiditis seen on magnetic resonance imaging (MRI). On routine eye exam, papilloedema was noted. A lumbar puncture was performed, with elevated opening pressure with normal constituents. She was an atypical age for idiopathic intracranial hypertension, and her mastoiditis raised concern for secondary cerebral venous sinus thrombosis. Magnetic resonance venography (MRV) was performed showing poor flow in the right sigmoid sinus, and computed tomography venography (CTV) showed lack of contrast enhancement distal to the right sigmoid sinus, consistent with occlusion. There was also an enhancing mass inferior to the right occipital bone. Biopsy confirmed recurrent plasma cell myeloma. She was treated with chemotherapy, radiation, and warfarin for presumed cerebral venous sinus thrombosis.

    View details for PubMedID 29339964

    View details for PubMedCentralID PMC5762173