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Competing Interests: The authors report no conflicts of interest.

Background: Papilledema must be managed distinctly from other causes of optic disc edema (ODE) due to its basis in raised intracranial pressure (ICP). However, evidence indicates that the term "papilledema" is widely misused across specialties to describe ODE without raised ICP. Sources of this misconception remain undiscerned. Because all physicians consult medical databases, our objective was to evaluate whether nonspecific "papilledema" subject heading definitions misleadingly associate articles on other conditions with papilledema proper., Methods: Systematic review of case reports, prospectively registered on PROSPERO (CRD42022363651). MEDLINE and Embase were searched to July 2022 for any full-length case report indexed to the "papilledema" subject heading. Studies were graded for incorrect indexing, defined as cases lacking evidence for raised ICP. Nonpapilledema diagnoses were assigned to a predefined set of diseases and pathophysiological mechanisms for subsequent comparison., Results: Incorrect indexing occurred in 40.67% of 949 included reports. Embase-derived studies were misindexed significantly less than MEDLINE-derived studies ( P < 0.01). There was also significant heterogeneity in incorrect indexing among specific diseases ( P = 0.0015) and mechanisms ( P = 0.0003). The most commonly misindexed diseases were uveitis (21.24% of errors), optic neuritis (13.47%), and instances with no mention of ODE (13.99%). The most commonly misindexed mechanisms were inflammation (34.97%), other mechanism (e.g., genetic; 25.91%), and ischemia (20.47%)., Conclusions: Database subject headings, especially from MEDLINE, do not adequately distinguish between true papilledema and other causes of ODE. Inflammatory diseases were most often incorrectly indexed among other diseases and mechanisms. Current "papilledema" subject headings should be revised to reduce the probability of misinformation., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Purpose: To report a case of a 38-year-old woman with venous stasis retinopathy secondary to myelin oligodendrocyte glycoprotein-IgG optic neuritis., Methods: Observational case report., Results: We report a unique case of venous stasis retinopathy secondary to myelin oligodendrocyte glycoprotein-IgG optic neuritis with significant optic disc edema, tortuous and dilated retinal venules, and retinal hemorrhages, which resolved promptly with high-dose corticosteroids. The retinal changes were likely secondary to severe inflammation of the optic nerve and optic nerve sheath, which exhibited significant postcontrast enhancement on magnetic resonance imaging. Despite aggressive treatment with high-dose corticosteroids and plasmapheresis, the patient had a significant generalized visual field defect at 6 months., Conclusion: Venous stasis retinopathy may be secondary to myelin oligodendrocyte glycoprotein-IgG optic neuritis due to reduced venous outflow from significant optic nerve edema. This may be a poor prognostic factor and a marker for more severe optic nerve inflammation.

Background: To determine differential diagnosis and visual outcomes of patients with no light perception (NLP) vision related to neuro-ophthalmic conditions., Methods: Retrospective case series of patients seen at tertiary neuro-ophthalmology practices. Patients were included if they had NLP vision any time during their clinical course. Outcome measures were final diagnosis, treatment, and visual outcome., Results: Seventy-two eyes of 65 patients were included. The average age was 57.6 (range 18-93) years, and 58% were women. The Most common diagnosis (21 patients) was compressive optic neuropathy (CON) with meningioma being the most common culprit (12). Other diagnoses included optic neuritis (ON) (11 patients), infiltrative optic neuropathies (8), posterior ischemic optic neuropathy (7), nonarteritic anterior ischemic optic neuropathy (4), arteritic anterior ischemic optic neuropathy (3), ophthalmic artery occlusion (3), nonorganic vision loss (3), radiation-induced optic neuropathy (2), cortical vision loss (1), retinitis pigmentosa with optic disc drusen (1), and infectious optic neuropathy (1). Ten patients recovered vision: 7 ON, 2 infiltrative optic neuropathy, and 1 CON. Corticosteroids accelerated vision recovery in 7 of the 11 patients with ON to mean 20/60 (0.48 logMAR) over 9.0 ± 8.6 follow-up months. Eleven patients deteriorated to NLP after presenting with at least LP; their diagnoses included CON (3), ophthalmic artery occlusion (2), infiltration (2), ON (1), posterior ischemic optic neuropathy (1), arteritic anterior ischemic optic neuropathy (1), and radiation-induced optic neuropathy (1)., Conclusions: NLP vision may occur because of various diagnoses. Vision recovery was mainly seen in patients with ON. Serious systemic conditions may present or relapse with NLP vision, which clinicians should consider as an alarming sign in patients with known malignancies., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

Background/aims: Optic neuritis (ON) is the primary ophthalmic manifestation of myelin oligodendrocyte glycoprotein-IgG-associated disorder (MOGAD), but numerous reports have expanded the visual manifestations of this condition. The goal of this study was to synthesise the extensive literature on this topic to help ophthalmologists understand when testing for MOG-IgG should be considered., Method: A systematic review of the English-language literature was performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines and searches were conducted using Ovid MEDLINE (from January 1, 1948 to April 1, 2020) and Ovid EMBASE (from January 1, 1947 to April 1, 2020). Inclusion criteria included studies describing non-isolated ON ophthalmic manifestations where cell-based assays were used for the detection of MOG antibodies., Results: Fifty-one articles representing 62 patients with a median age of 32.0 (range 2-65), female gender (51%) and follow-up of 20.0 months (range: 1-240) were included. Twenty-nine patients had non-isolated ON afferent visual manifestations: uveitis, peripheral ulcerative keratitis, acute macular neuroretinopathy, neuroretinitis, venous stasis retinopathy, large preretinal macular haemorrhage, orbital inflammatory syndrome, orbital apex syndrome, optic perineuritis, papilloedema and homonymous visual field defects. Incomplete recovery of ON was associated with a case of Leber's hereditary optic neuropathy. Efferent ophthalmic manifestations included cranial neuropathies, internuclear ophthalmoplegia, central nystagmus, saccadic intrusions and ocular flutter. Cranial nerve involvement was secondary to enhancement of the cisternal portion or brainstem involvement. All included cases were treated with corticosteroids with 31% of cases requiring additional immunosuppressive therapy., Conclusions: MOGAD has been associated with various afferent and efferent ophthalmic manifestations apart from isolated ON. Awareness of these findings may result in earlier diagnosis and treatment., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To describe clinical characteristics of Canadian patients with myelin-oligodendrocyte glycoprotein immunoglobulin-G optic neuritis (MOG-IgG ON)., Methods: Retrospective observational case series of MOG-IgG seropositive patients with ON referred to tertiary neuro-ophthalmology practices. Outcome measures included clinical characteristics, radiologic findings, and visual outcomes., Results: Forty-six eyes of 30 patients were included. Twenty-three (76.7%) were women, mean onset age was 40.7 years (range 16-77), and most were Caucasian. Seventeen (56.7%) presented with their first ON episode. Sixteen (53.3%) had bilateral eye involvement. Isolated ON without associated neurological symptoms occurred in 90.0%. In 22 patients with acute ON (seen within 1 month of onset), presenting mean visual acuity (VA) was 20/258 (logMAR 1.11), mean deviation (MD) on Humphrey visual fields was -16.90 ± 10.83 dB, and peripapillary retinal nerve fiber layer (RNFL) thickness on ocular coherence tomography (OCT) was 164.23 ± 46.53 um. Orbital magnetic resonance imaging (MRI) within 1 month of symptom onset for 19 patients demonstrated orbital optic nerve enhancement in 11 (57.9%) and perineural enhancement in 11 (57.9%). Brain MRI was normal in 28 (93.3%) patients. Twenty out of 22 patients with acute presentation were treated with high-dose glucocorticoids and 5 with plasma exchange in addition to corticosteroids. Long-term immunosuppression was utilized in 9 (30%) out of all 30 patients. Final VA was 20/30 (logMAR 0.18), MD was -7.17 ± 8.85 dB, and RNFL thickness was 72.15 ± 20.16 um., Conclusion: MOG-IgG ON in Canada has a variable presentation with most patients having substantial initial vision loss with good recovery. This is the largest characterization of the disease in Canada to date.

A 35-year-old healthy African-American woman presented with a 4-month history of gradual loss of vision in the right eye from an optic neuropathy. MRI of the orbits with gadolinium showed isolated thickening and enhancement of the right optic nerve sheath. Chest x-ray and CT-scan of the chest were performed and showed bilateral hilar and mediastinal lymphadenopathy. This was suggestive of sarcoidosis, and the diagnosis was confirmed with histopathology. The patient promptly recovered vision with high-dose corticosteroids; the thickening of the optic nerve sheath also regressed. Isolated optic nerve sheath thickening from sarcoidosis is rare and may mimic compressive optic neuropathies such as optic nerve sheath meningiomas. A systemic evaluation for systemic inflammatory etiologies should be considered in such cases.