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Purpose: To determine the frequency and severity of further visual loss experienced by patients within ten weeks from diagnosis of acute nonarteritic anterior ischemic optic neuropathy (NAION)., Design: Retrospective case series., Methods: Electronic medical records (EMR) at an academic neuro-ophthalmology practice were searched for diagnosis of "NAION" and all identified charts were reviewed to determine eligibility. Patients diagnosed with acute NAION between February 2014 and December 2023 who presented within four weeks of symptom onset and were seen in follow-up within ten weeks were included. Clinically significant decline in best corrected visual acuity (BCVA) and peripheral VF were defined as decline of BCVA ≥2 Snellen lines and decrease of ≥2 decibels (dB) in mean deviation (MD) on perimetric testing., Results: Forty-nine eyes met inclusion and exclusion criteria. Sixty-seven percent of patients were male and average age at presentation was 66 years. Twenty-two percent of eyes demonstrated worsening of BCVA by ≥2 lines. Of these, 55% worsened by ≥4 lines and 27% by ≥8 lines. In 27% of eyes MD on perimetry worsened by ≥2 dB and in 18% by ≥4 dB. In total, 41% of eyes demonstrated clinically significant worsening of BCVA or VF., Conclusions: Subacute deterioration of BCVA and/or VF following acute NAION is not uncommon while optic disc edema is present, with sizeable proportion of patients experiencing dramatic visual decline. Deterioration in visual function within the first 10 weeks of presentation does not exclude the diagnosis of NAION and further investigations should only be performed if additional clinical features are discordant with this diagnosis., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Giant cell arteritis can present with atypical manifestations that delay treatment and risk severe complications., Objectives: To comprehensively describe all atypical signs/symptoms of giant cell arteritis., Design: In this systematic review, we searched MEDLINE, Embase, and Cochrane Central Register of Controlled Trials from inception to October 2022. Primary research articles that included at least one participant with an atypical sign/symptom of biopsy-proven giant cell arteritis were included. Study screening and data extraction were performed in duplicate. The primary outcome was the proportion of participants with atypical giant cell arteritis features. Time to treatment was compared between participants with atypical giant features only and participants with both typical and atypical features., Results: Of 21,828 screened records, 429 studies corresponding to 746 individuals (median [IQR] age 72 [IQR, 66-78] years, 63% female) with at least one atypical feature of GCA were included. Eighty-two percent had both atypical and at least one concurrent typical giant cell arteritis feature, whereas 18% of patients with atypical signs and symptoms only presented with atypical features. Patients with atypical symptoms presented to clinicians earlier than patients with typical features (p < 0.001). There was no difference between groups in proportion to elevated ESR and CRP (82.3% vs. 83.35%, p = 0.91) or mortality rate (8.2% vs. 10.8%, p = 0.42). Patients with atypical features only experienced greater delay in treatment initiation (p < 0.001). The most commonly reported atypical signs/symptoms were vertigo (11.9%), scalp necrosis/ulceration (7.9%), and dry cough (5.8%)., Conclusions: Eighteen percent of biopsy-proven giant cell arteritis cases with at least one atypical feature have only atypical features and are more likely to experience delays in treatment. Clinicians should be aware of atypical signs/symptoms of giant cell arteritis and order inflammatory markers early to prevent giant cell arteritis-associated morbidity., (© 2024. The Author(s), under exclusive licence to Society of General Internal Medicine.)

A middle-aged man developed subacute painless visual loss in the left eye leading to no light perception, and 2 weeks later developed subacute visual loss in the right eye to no light perception. He had a history of resected pituitary macroadenoma. MR scan of brain and orbits with contrast showed short prechiasmatic segments of enhancement in each optic nerve. Cerebrospinal fluid analysis was normal. Extensive diagnostic work-up was unrevealing but review of medical records identified a history of prophylactic radiotherapy to the pituitary gland following pituitary macroadenoma resection 1.5 years before. We diagnosed post-radiation optic neuropathy. This condition typically occurs 1-1.5 years after the radiotherapy given near the visual pathway. Its pathophysiology presumably relates to an endotheliopathy of the vasa nervosum supplying the optic nerve due to free radical accumulation following radiotherapy. It manifests with unilateral or bilateral sequential severe visual loss with imaging showing characteristic enhancement of the short segment of the affected intracranial optic nerve. There is no available definitive treatment, but hyperbaric oxygen therapy, given shortly after onset of visual loss, is a promising treatment., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Background: Symmetric optic neuropathy (SON) is commonly seen in neuro-ophthalmic practice and is often discovered incidentally. Although multiple investigations might be performed to discover the underlying cause, they are not always indicated. The aim of this study was to report a clinically reasonable and cost-effective approach to investigating patients with SON., Methods: SON was defined as bilateral optic neuropathy with normal and/or symmetrically decreased central visual acuity, absence of relative afferent pupillary defect, presence of symmetric optic disc pallor, symmetric thinning of peripapillary retinal nerve fiber layer on optical coherence tomography, and absence of other identifiable causes of optic neuropathy. Records of all patients diagnosed with SON seen at a tertiary university-affiliated neuro-ophthalmology practice from 2016 to 2022 were reviewed to identify the yield of various investigations. Clinical data from the initial and last follow-up visit were obtained. Subgroup analysis was performed to ascertain whether diagnostic yield is higher in patients with severe visual loss (central acuity worse than 20/40) compared with those with mild visual loss (acuity 20/40 or better)., Results: One hundred thirty-six patients met inclusion criteria. Testing for OPA1 and OPA2 mutations had the highest diagnostic yield (16.0%), followed by mitochondrial genome sequencing (13.6%), serum vitamin B12 (6.1%), and serum folate (1.6%). MRI brain was performed in 54.4% of patients and had a diagnostic yield of only 5%. Both patients who had abnormal MRI had symptoms of demyelination at presentation. Patients were followed for a mean of 15.0 (SD 21.3) months. The most frequently identified etiologies of SON were Leber hereditary optic neuropathy (8.1%), alcohol/tobacco amblyopia (7.4%), vitamin B12 deficiency (5.9%), and dominant optic atrophy (2.9%). Patients with severe visual impairment were more likely to have a final diagnosis compared with those with milder visual impairment (63.9% vs 12.0%, P < 0.001)., Conclusions: The diagnostic yield of investigating SON in patients with preserved visual function, normal diet, and absence of other neurological symptoms is very low. It is reasonable to observe patients with SON with mild visual impairment, reserving costly investigations for those with the visual acuity worse than 20/40 or progressive course., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Competing Interests: The authors report no conflicts of interest.

Purpose: This study aimed to determine whether magnetic resonance imaging (MRI) biomarkers are associated with visual prognosis in myelin oligodendrocyte protein (MOG)-associated optic neuritis (ON)., Design: Cross-sectional analysis., Participants: Patients meeting 2023 international diagnostic criteria for MOG antibody-associated disease who were seen for first episodes of MOG-associated ON at 3 tertiary neuro-ophthalmology practices between January 2017 and July 2023 were enrolled. Patients who received < 3 months of neuro-ophthalmic follow-up and did not demonstrate visual recovery (visual acuity [VA] ≥ 20/20 and visual field mean deviation [VFMD] > -5.0 dB) during this time were excluded., Methods: Patients underwent contrast-enhanced, fat-suppressed MRI of the brain and orbits within 1 month of symptom onset., Main Outcome Measures: The associations between radiologic biomarkers and poor VA outcome (< 20/40), incomplete VA recovery (< 20/20), and poor VFMD outcome (VFMD < -5.0 dB) were assessed using multivariable logistic regression adjusting for time from symptom onset to treatment and nadir VA or VFMD. Radiologic biomarkers included length of optic nerve enhancement (> 25% vs. < 25%; > 50% vs. < 50%; and > 75% vs. < 75%); degree of orbital, canalicular, and intracranial or chiasmal optic nerve enhancement (mild vs. moderate to severe compared with the lacrimal gland); and absence versus presence of optic nerve sheath enhancement on baseline T1-weighted MRI., Results: A total of 129 eyes of 92 patients (median age, 37.0 years [interquartile range, 20.8-51.3 years]; 65.2% female) were included. Poor VA outcome was seen in 6.2% of patients, incomplete VA recovery was seen in 19.4% of patients, and poor VFMD outcome was seen in 16.9% of patients. Compared with eyes with moderate to severe enhancement, eyes with mild orbital optic nerve enhancement were more likely to have poor VA outcome (odds ratio [OR], 8.57; 95% confidence interval [CI], 1.85-51.14; P = 0.009), incomplete VA recovery (OR, 7.31, 95% CI, 2.42-25.47; P = 0.001), and poor VFMD outcome (adjusting for time to treatment: OR, 6.81; 95% CI, 1.85-28.98; P = 0.005; adjusting for nadir VFMD: OR, 11.65; 95% CI, 1.60-240.09; P = 0.04). Lack of optic nerve sheath enhancement additionally was associated with incomplete VA recovery (OR, 3.86; 95% CI, 1.19-12.85; P = 0.02) compared with the presence of enhancement. These associations remained consistent in subgroup logistic regression analysis of MRIs performed before initiation of treatment but were not seen in pairwise analysis of MRIs performed after treatment., Conclusions: In eyes with first MOG-associated ON episodes, milder enhancement in the orbital optic nerve was associated with poorer VA and visual field recovery. Prospective and mechanistic studies are needed to confirm the prognostic usefulness of MRI in MOG-associated ON., Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article., (Copyright © 2024 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

Competing Interests: The authors report no conflicts of interest.

Background: Myelin oligodendrocyte glycoprotein-associated optic neuritis (MOG-ON) is typically a highly treatable condition that responds quickly to treatment with high doses of corticosteroids. We reviewed the cohort of patients with MOG-ON to identify patients who had poor visual outcome after the first attack despite rapid initiation of treatment., Methods: Records of all patients diagnosed with MOG-ON seen in a tertiary neuro-ophthalmology practice were reviewed to identify and describe those with poor visual recovery (final visual acuity of 20/200 or worse in at least one eye) after the first attack despite initiation of treatment within 1 week of symptoms onset., Results: Two patients of 36 fulfilled inclusion criteria: both had bilateral severe optic neuritis at presentation, and both were seen within 7 days of symptoms onset and treated immediately with pulse doses of intravenous corticosteroids followed by very-slow oral taper. Plasma exchange (PLEX) was performed 2 weeks after symptoms onset in both patients because of poor response to steroids, followed by monthly intravenous immunoglobulin infusions. Despite the use of all available treatment modalities, final visual outcome was poor in both patients. In both patients, there was enhancement of intracanalicular portion of optic nerve in the worse-seeing eye., Conclusions: In this cohort of patients with MOG-ON, 2 (5.6%) had very poor visual outcome after the first attack despite immediate initiation of treatment with pulse doses of corticosteroids and subsequent treatment with PLEX. These cases highlight that despite immediate initiation and escalation of treatment, some patients with MOG-ON can have very poor visual outcomes after the initial attack that may be because of the involvement of the intracanalicular portion of the optic nerves., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Competing Interests: The authors report no conflicts of interest.

Background: Transverse sinus stenting (TSS) is an increasingly common treatment for patients with idiopathic intracranial hypertension (IIH). However, detailed neuro-ophthalmic evidence on visual and pharmacotherapy outcomes after TSS is scarce and heterogeneous. This study aimed to describe the visual outcomes of patients undergoing TSS for IIH and to ascertain the proportion of patients who could be weaned off intracranial pressure (ICP)-lowering medication postoperatively., Methods: A retrospective chart review of all patients with IIH from 2 tertiary academic neuro-ophthalmology practices who underwent TSS between 2016 and 2022 was performed. Indications for stenting included failure of pharmacotherapy, intolerance of pharmacotherapy, and acute vision loss from severe papilledema. Data on demographics, symptoms, visual function, pharmacotherapy, and TSS were collected. The paired Wilcoxon rank sum test was used to compare changes in visual acuity (VA) and visual field mean deviation (VFMD) between the baseline and most recent visits., Results: Of the 435 patients with IIH, 15 (13 women) met inclusion criteria. After TSS, ICP-lowering pharmacotherapy was discontinued in 10 patients and decreased in 4; 1 patient was not on ICP-lowering medication before TSS. All patients experienced resolution or improvement of symptoms (10 resolution, 4 improved, 1 asymptomatic before TSS) and papilledema (11 resolution, 4 improved) after stenting. Papilledema resolution was confirmed with optical coherence tomography-measured peripapillary nerve fiber layer thickness (median decrease 147 µm, interquartile range 41.8-242.8 µm, P < 0.001). Change in VA between the baseline and most recent visit was not significant, but VFMD improved significantly after stenting (median increase 3.0, IQR 2.0-4.2, P < 0.001). No patient developed transverse sinus restenosis nor in-stent thrombosis postoperatively across a median venogram follow-up of 20.8 (11.3-49.8) weeks. In addition, no patient required subsequent surgical intervention for IIH., Conclusions: In this cohort of patients with IIH and fulminant presentation, medication resistance, or medication intolerance, TSS was an effective and safe treatment modality. Most patients were able to stop ICP-lowering medications while demonstrating striking improvement in symptomatology and visual function., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 North American Neuro-Ophthalmology Society.)

Competing Interests: The authors report no conflicts of interest.

Background: Patient education in ophthalmology poses a challenge for physicians because of time and resource limitations. ChatGPT (OpenAI, San Francisco) may assist with automating production of patient handouts on common neuro-ophthalmic diseases., Methods: We queried ChatGPT-3.5 to generate 51 patient education handouts across 17 conditions. We devised the "Quality of Generated Language Outputs for Patients" (QGLOP) tool to assess handouts on the domains of accuracy/comprehensiveness, bias, currency, and tone, each scored out of 4 for a total of 16. A fellowship-trained neuro-ophthalmologist scored each passage. Handout readability was assessed using the Simple Measure of Gobbledygook (SMOG), which estimates years of education required to understand a text., Results: The QGLOP scores for accuracy, bias, currency, and tone were found to be 2.43, 3, 3.43, and 3.02 respectively. The mean QGLOP score was 11.9 [95% CI 8.98, 14.8] out of 16 points, indicating a performance of 74.4% [95% CI 56.1%, 92.5%]. The mean SMOG across responses as 10.9 [95% CI 9.36, 12.4] years of education., Conclusions: The mean QGLOP score suggests that a fellowship-trained ophthalmologist may have at-least a moderate level of satisfaction with the write-up quality conferred by ChatGPT. This still requires a final review and editing before dissemination. Comparatively, the rarer 5% of responses collectively on either extreme would require very mild or extensive revision. Also, the mean SMOG score exceeded the accepted upper limits of grade 8 reading level for health-related patient handouts. In its current iteration, ChatGPT should be used as an efficiency tool to generate an initial draft for the neuro-ophthalmologist, who may then refine the accuracy and readability for a lay readership., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by North American Neuro-Ophthalmology Society.)

Abstract: A 61-year-old woman with a history of untreated low-grade B-cell lymphoma presented with blurry vision, unsteadiness, and worsening pain on touching skin of the upper trunk was enrolled. Blurry vision was attributed to oscillopsia from downbeat nystagmus, which later evolved into macrosaccadic oscillations. MRI brain and spine showed mild, longitudinally extensive T2 hyperintensity in the central gray matter of the spinal cord extending from the medulla to T11 level. Serum paraneoplastic panel was negative; however, she had very high titers of anti-Ma2 antibodies in cerebrospinal fluid. The diagnosis of paraneoplastic neurological syndrome was made. Empiric treatment with high dose of intravenous steroids followed by intravenous immunoglobulin infusions did not improve her symptoms. An extensive search for an underlying tumor commenced and was initially unrevealing. However, two-month follow-up positron emission tomography scan showed increased uptake in a right pulmonary nodule, which when biopsied confirmed diagnosis of extranodal marginal zone lymphoma. The final diagnosis was anti-Ma2 antibody-mediated paraneoplastic cerebellar degeneration and myeloneuropathy secondary to lymphoma., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Background: Bilateral transverse venous sinus stenosis (TVSS) or stenosis of a dominant venous sinus has been found to be very sensitive radiological findings in patients with intracranial hypertension (IH), yet there is still an ongoing debate whether they constitute reversible or permanent phenomena. Thus, the purpose of this study was to investigate the reversibility of TVSS in patients with IH, including conservatively treated patients with signs of IH as defined by the presence of papilledema., Methods: This was a retrospective chart review of all patients diagnosed with IH between 2016 and 2022, assessed from 2 tertiary university-affiliated neuro-ophthalmology practices. Inclusion criteria were the presence of papilledema, as quantified by optical coherence tomography, and bilateral TVSS, which is considered typical of IH on neuroimaging. During follow-up, included patients must have had confirmation of papilledema resolution as well as subsequent neuroimaging after conservative treatment or cerebrospinal fluid flow diversion. Patients with dural sinus vein thrombosis or intrinsic stenosis from sinus trabeculations or significant arachnoid granulations were excluded from the study. Either CT venography or MRI/MR venography was reviewed by a fellowship-trained neuroradiologist, and the degree of stenosis was scored through the combined conduit score (CCS), as described by Farb et al. The primary outcome was to assess TVSS changes after resolution or improvement of papilledema., Results: From 435 patients, we identified a subset of 10 who satisfied all inclusion criteria. Our cohort comprised entirely women with a median age of 29.5 years and a median BMI of 32.5 kg/m2. Treatment consisted of acetazolamide in 7 patients, of which 1 had additional topiramate and 2 underwent cerebrospinal fluid flow diversion. Furthermore, 6 patients demonstrated significant weight loss during follow-up. For the primary outcome, 5 of 10 patients exhibited no appreciable TVSS change, and 5 patients demonstrated significant improvement in TVSS, of which 4 received conservative treatment only. Papilledema resolution or improvement was statistically significantly associated with increasing average CCS, TVSS diameter, and grade., Conclusions: We were able to demonstrate that TVSS can be both irreversible and reversible in patients with resolved papilledema. The finding of TVSS reversibility from conservative treatment alone is novel and has important implications to optimize patient care. Future studies should work to identify factors associated with irreversible TVSS for subsequent targeted intervention and prevention., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by North American Neuro-Ophthalmology Society.)

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Objective: To investigate whether a video tutorial, highlighting important aspects of keratoconus provided prior to a scheduled follow-up consultation, has a specific effect on patients' knowledge after the consultation., Methods and Analysis: Single center, randomized controlled trial registered on ISRCTN registry (number ISCTN75317089, https://doi.org/10.1186/ISRCTN75317089). Consenting eligible keratoconus patients were randomly assigned to either receive a conventional face-to-face consultation (control group) or to an additional video tutorial (interventional group) on definition, risk factors and treatment options provided prior to the consultation. The main outcome measure was the difference of knowledge assessed by a questionnaire after the consultation. Of each participant, clinical characteristics, highest educational level and medical background were obtained. We also performed a meta-analysis of published reports assessing knowledge improvement by video-based patient education., Results: We assigned 22 patients to the interventional and 21 patients to the control group. Mean age was 29.0 years (SD 11.6), 8/43 (18.6%) were female and median disease duration was 2.5 years (interquartile range: 2-5years). Compared to the control group, knowledge was 12.0% (95%CI: 5.8%-18.2%; p<0.001) higher in the interventional group. Subjects with a university degree scored 6.8% (95%CI: 3.8%-13.3%; p=0.038) higher. There was no interaction between video information and university degree. Other parameters were not associated with patient knowledge. The meta-analysis of 566 subjects enrolled in 6 studies revealed a standardized mean difference in favor of video-based education of 0.47 (95% CI: 0.30-0.64; p<0.004)., Conclusion: The results suggest that supplementary video information embedded in the clinical management of keratoconus, helps conveying relevant disease knowledge., Competing Interests: Author LB was employed by medignition Inc. Research Consultants Zurich. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Sommer, Bachmann, Handzic, Iselin, Sanak, Pfaeffli, Kaufmann, Thiel and Baenninger.)

Purpose: To assess whether Swiss adult citizens diagnosed with keratoconus have the minimal knowledge that a corneal specialist would expect they should have., Methods: Experts defined the "minimal keratoconus knowledge" (MKK) with respect to definition, risk factors, symptoms, and possible treatment options of keratoconus. A survey was performed in 167 patients with keratoconus [mean age 38.8 years (SD 13.9), 77.7% male] in 5 specialized institutions. Of each participant, salient clinical characteristics, highest educational level, paramedical background, and specific health experience with keratoconus in the social surrounding were obtained. We calculated the proportion of MKK and examined whether patients with higher education and greater disease experience would perform better than those from other groups in multivariate analyses., Results: No single citizen reached 100% MKK. The mean MKK was 35.2%, and the range was 0% to 76.2%. Participants with a university degree had only a moderately higher MKK [+8.7% (95% confidence interval: 4.4-13.0); P < 0.001]. Per age decile, the MKK declined by 3.1% (95% confidence interval: 1.2-4.9), P = 0.002. Disease duration, severity of keratoconus in Kmax values, and history of surgical treatment did not significantly increase MKK. Surprisingly, MKK was also lower in patients with a paramedical background [-6.3% (-14.1 to 1.4); P = 0.107]., Conclusions: This sample of Swiss patients with keratoconus did not know more than a third of the MKK. We found a little difference within various subgroups. There is a substantial mismatch between caregivers' expectations of patients' knowledge and patients' active knowledge regarding their condition. This may lead to an inefficient care delivery and misunderstandings., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Competing Interests: The authors declare that they have no conflict of interest.

Competing Interests: The authors declare that they have no conflict of interest.

Aims: Inflammation is a key factor in the long-term outcome of acute coronary syndromes (ACS). The aim of the present study was to evaluate inflammatory markers in patients with ACS as predictors for major adverse cardiovascular events (MACE) and hard events., Methods: This study included 1548 patients with ACS. C-reactive protein (CRP), white blood count (WBC), and their subtypes were analyzed during hospitalization. Receiver operator characteristic (ROC) and Kaplan-Meier survival curves were used to assess the predictive value and hard events (nonfatal myocardial infarction and cardiac death) and MACE (hard events, hospitalization for cardiac causes, late revascularization and stroke) were obtained during 30 days., Results: ROC analysis of CRP and WBC to predict adverse events revealed cut-offs of 47.5 ng/l and 16.6 × 10/μl for MACE and 93.5 ng/l and 16.6 × 10/μl for hard events. The cumulative adverse event rates were significantly higher in patients with increased CRP (≥47.5 ng/l; 17 versus 4%, P < 0.001) and WBC (≥16.6 × 10/μl; 21 versus 5%, P < 0.001) for MACE and with elevated CRP (≥93.5 ng/l; 16 versus 2%, P < 0.001) and WBC (≥16.6 × 10/μl; 18 versus 2%, P < 0.001) for hard events, demonstrating highest event rates with elevation of both inflammatory markers: (28 versus 5%, P < 0.001) for MACE and (26 versus 2%, P < 0.001) for hard events. Analysis of CRP and WBC further revealed a substantial negative correlation with left ventricular function (P < 0.001). Moreover, markers of myocardial damage were significantly elevated in patients with abnormal CRP or WBC (P < 0.001)., Conclusion: Inflammatory markers such as CRP and WBC alone and, particularly, in combination are strong and independent predictors of outcome in patients with ACS.