Radiological predictors of visual outcome in myelin oligodendrocyte glycoprotein-related optic neuritis.

OBJECTIVE: This study aimed to determine whether magnetic resonance imaging (MRI) biomarkers are associated with visual prognosis in myelin oligodendrocyte protein (MOG)-associated optic neuritis (MOG-ON). DESIGN: Cross-sectional analysis. SUBJECTS: Patients meeting 2023 international diagnostic criteria for MOG antibody-associated disease who were seen for first episodes of MOG-ON at three tertiary neuro-ophthalmology practices between January 2017 and July 2023 were enrolled. Patients who received less than 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 received contrast-enhanced, fat-suppressed MRI of the brain and orbits within one month of symptom onset. MAIN OUTCOME MEASURES: The associations between radiological 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. Radiological biomarkers included length of optic nerve enhancement (below vs. above 25%, 50%, and 75%); degree of orbital, canalicular, and intracranial or chiasmal optic nerve enhancement (mild vs. moderate-severe compared to the lacrimal gland); and absence vs. presence of optic nerve sheath enhancement on baseline T1-weighted MRI. RESULTS: A total of 129 eyes of 92 patients (median [IQR] age 37.0 [20.8-51.3], 65.2% female) were included. Poor VA outcome was seen in 6.2% of cases, incomplete VA recovery in 19.4%, and poor VFMD outcome in 16.9%. Compared to eyes with moderate-severe enhancement, eyes with mild orbital optic nerve enhancement were more likely to have poor VA outcome (OR 8.57; 95% 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 was additionally associated with incomplete VA recovery (OR 3.86, 95% CI [1.19, 12.85], P=0.02) compared to 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-ON episodes, milder enhancement in the orbital optic nerve is associated with poorer VA and VF recovery. Prospective and mechanistic studies are needed to confirm the prognostic utility of MRI in MOG-ON.

Sensitivity of Magnetic Resonance Imaging of the Medial Longitudinal Fasciculus in Internuclear Ophthalmoplegia.

BACKGROUND: Internuclear ophthalmoplegia (INO) is a result of insult to the medial longitudinal fasciculus (MLF). Clinicoradiological correlation in patients with INO has been reported to be poor; however, prior studies have used low resolution MRI imaging techniques and included patients with subclinical INO. We aimed to determine the sensitivity of modern MRI interpreted by a specialist neuroradiologist to detect clinically evident INO. METHODS: A retrospective chart review of patients in 2 tertiary University-affiliated neuro-ophthalmology practices with the diagnosis of INO. MRI scans of all patients were reviewed and interpreted by a fellowship-trained neuroradiologist for the presence of lesion in MLF and concordance with the original imaging report. RESULTS: Forty-five patients were included in the study: 33 with demyelinating disease, 11 with stroke, and 1 with intracranial mass. A visible MLF lesion was present in 25/33 demyelinating cases and 7/11 ischemic cases. Lesions in 2 cases in each group were identified only after review by a fellowship-trained neuroradiologist. In demyelinating INO, patients with a visible MLF lesion were more likely to show other brainstem (72%) and supratentorial (51%) white matter lesions. CONCLUSIONS: In 25% of patients with demyelinating INO and 33% of patients with ischemic INO, no visible lesion was identified on current high-quality MRI imaging. Review of imaging by a neuroradiologist increased the possibility of lesion been identified.

The Leading Causes of Optic Disc Edema Seen in Tertiary Care, Academic Neuro-Ophthalmology Clinics.

BACKGROUND: Optic disc edema (ODE) is one of the most common reasons for referral to a neuro-ophthalmologist. There are various causes that require vastly different workup. Thus, differentiating among each cause is important. Our goal was to determine the causes of ODE and various clinical characteristics of consecutive patients with ODE presenting to neuro-ophthalmology clinics. METHODS: A retrospective review of consecutive patients with ODE over a period of 5 years were included. Fundus photographs were routinely obtained as part of clinical care. Clinical data including retinal nerve fiber layer thickness, best-corrected visual acuity, and visual field mean deviation were retrieved, and patients were grouped by etiology. RESULTS: A total of 654 patients (n = 462 or 70.6% women and 192 or 29.4% men) with ODE were included with a mean age of 41.2 ± 17.9 years. Female patients were significantly younger than male patients (mean age female participants: 38.7 years, male participants: 47.6; P < 0.001). The top 5 most common causes of ODE in our clinics were idiopathic intracranial hypertension (IIH; 351/654 or 53.7%), nonarteritic anterior ischemic optic neuropathy (NAION; 116/654 or 17.4%), non-IIH papilledema (71/654 or 10.9%), optic neuritis (ON; 46/654 or 7.0%), and uveitis (17/654 or 2.6%). When considering female-only patients, the top 3 causes of ODE were IIH, non-IIH papilledema, and NAION. Among male-only patients, the top 3 causes were NAION, IIH, and non-IIH. Among the top 5 causes of ODE, visual acuity was the worst at presentation in the ON group and the best in the patients with IIH. The Humphrey mean deviation was the worst in ON/NAION groups and best in IIH group. The ODE was most severe in patients with non-IIH papilledema and least severe in ON group. Non-IIH patients with papilledema were not significantly different in visual acuity and visual field parameters at presentation compared with patients with IIH papilledema. Patients with papilledema (both IIH and non-IIH etiologies) had significantly better visual function at presentation compared with other top causes of disc edema (P < 0.001). CONCLUSIONS: The most common cause of ODE seen in neuro-ophthalmology clinics was IIH, and these patients were more likely to present with preserved visual function. The higher prevalence of IIH was likely why most patients with ODE were women. Visual function at presentation was not able to differentiate the specific cause of papilledema; however, it was an important differentiating factor for all papilledema cases compared with all other causes of ODE including ON and NAION.

Utility of ChatGPT for Automated Creation of Patient Education Handouts: An Application in Neuro-Ophthalmology.

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.

Assessment of Reversibility of Transverse Venous Sinus Stenosis in Patients With Papilledema.

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.

Visual and Pharmacotherapy Outcomes After Transverse Sinus Stenting for Idiopathic Intracranial Hypertension.

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.

Inappropriate Use of the Term "Papilledema" in the Medical Literature: A Systematic Review of Case Reports across Specialties.

TOPIC: The term "papilledema" is often misused in different ways when describing nonintracranial pressure-related optic disc edema (ODE) or on fundoscopic examination. However, the extent of these errors is unknown among case reports. CLINICAL RELEVANCE: "Papilledema" refers to ODE secondary to raised intracranial pressure (ICP). Papilledema is a leading reason for referral to neuro-ophthalmologists. In any specialty setting, papilledema must be distinguished from other causes of ODE because it implies a unique pathophysiology and management strategy. If managed inappropriately, papilledema may cause permanent vision loss. METHODS: Cross-sectional evidence-based study with protocol registered on Open Science Framework (digital object identifier: 10.17605/OSF.IO/BUQS3). From Ovid MEDLINE and EMBASE, we included case reports with "papilledema" in their title, abstract, or article keywords between January 2011 and March 2022. Studies were graded for the following errors: type 1 (using "papilledema" without evidence of elevated ICP), type 2 (declaring "papilledema" on examination before finding evidence of elevated ICP), type 3 (both errors type 1 and 2), or no error. RESULTS: Among 722 case reports, there was a total of 482 errors (66.8%). There were 12 type 1 errors, 360 type 2 errors, 110 type 3 errors, and 240 studies with no errors. Ophthalmology, neurology, and neurosurgery were the most represented fields, yet only ophthalmology had a low prevalence of errors among all specialties. Regardless, all specialties with at least 10 included reports had a high error prevalence (>30%). Reduced error prevalence was significantly associated with higher impact factor journals, non-open-access publication models, and origin countries with an English official language. Error prevalence was not significantly associated with publication year. Also, the relationship between article citation counts and the prevalence of any type of error did not reach significance. Risk of bias analysis indicated that error occurrence was unrelated to the quality of case report study design. CONCLUSION: "Papilledema" is widely misused even among ophthalmology case reports. When allowed to propagate, this error can mislead care delivery to patients with ODE. Our results, although limited to case reports, indicate a need for more thorough peer-review standards and neuro-ophthalmology exposure in medical education.

Idiopathic Intracranial Hypertension and Socioeconomic Status in the Greater Toronto Area, Canada.

BACKGROUND: Previous studies have identified an association between obesity and socioeconomic variables such as poverty, minority status, and a low level of education. Because obesity is a major risk factor for the development of idiopathic intracranial hypertension (IIH), this study aims to identify and assess relationships between socioeconomic and geographic variables in patients with IIH in Canada. METHODS: A retrospective chart review was performed to identify female patients with IIH presenting to 2 neuro-ophthalmology clinics in Toronto between 2014 and 2022. Consecutive female patients younger than 50 years who did not have IIH were identified as controls. Patient age, body mass index (BMI), and postal code were obtained from electronic medical records. Patient postal codes were then converted to geographic dissemination areas based on the 2016 Canadian census, and data on socioeconomic outcomes were collected from Statistics Canada. RESULTS: Three hundred twenty-two female patients with IIH (mean age: 32.3 ± 10) and 400 female controls (mean age: 33.9 ± 9) were included. The mean BMI was 35.0 ± 8 for patients with IIH and 26.7 ± 7 for control patients ( P < 0.00001). There was a significant difference between dissemination areas resided by patients with IIH and control patients for median income ($34640 vs $36685 CAD, P = 0.02) and rate of postsecondary degree attainment (57.7% vs 60.5%, P = 0.01). There were no significant differences in the percentage of visible minorities, percentage of immigrants, knowledge of official languages, percentage of married individuals, average household size, or unemployment rate. There was a weak but significant inverse relationship between the rate of postsecondary degree attainment in dissemination areas resided by patients with IIH and their BMI ( P = 0.01, R 2 = 0.02). CONCLUSION: Patients with IIH reside in geographic areas with lower average levels of income and education than control patients. Patients with lower levels of education may be at higher risk of elevated BMI and therefore disease incidence and progression.

Unilateral Pendular Nystagmus in Multiple Sclerosis: A Case Series.

BACKGROUND: Acquired pendular nystagmus is most often seen in patients with demyelinating disease. Although it is often bilateral, rare cases may be monocular. There is paucity of data on the spectrum of clinical presentation, underlying mechanism, and response to treatment in patients with monocular pendular nystagmus. METHODS: Retrospective case series of patients with monocular pendular nystagmus seen in 2 tertiary neuro-ophthalmology clinics between January 2019 and June 2022. All patients underwent a complete neuro-ophthalmological assessment and MRI. RESULTS: We describe 5 patients (3 women) aged 31-49 with monocular pendular nystagmus. All had a diagnosis of multiple sclerosis. Three patients had horizontal and 2 had vertical pendular nystagmus. The Snellen visual acuity in the eye with pendular nystagmus varied from 20/20 to 20/200. Two patients were asymptomatic and 3 suffered visually debilitating oscillopsia. Treatment response was available for 2 patients, both of which responded well to treatment with memantine. The pendular nystagmus was observed in the eye with worse visual acuity in 4 of 5 cases (80%). Three patients had bilateral pontine lesions, and 2 had unilateral pontine lesion ipsilateral to the side of nystagmus. CONCLUSIONS: Monocular pendular nystagmus in adults is seen most often in patients with multiple sclerosis. Asymmetry in brainstem lesions and afferent visual input may be the culprit. Treatment with memantine may result in significant improvement in symptomatic patients.

Maculopathies Referred to Neuro-Ophthalmology Clinic as Optic Neuropathies: A Case Series.

BACKGROUND: The clinical features of maculopathies and optic neuropathies often overlap: Both present with decreased visual acuity and variable loss of color vision; thus, maculopathy can be misdiagnosed as optic neuropathy, leading to patient harm. We aimed to determine what findings and/or tests were most helpful in differentiating between optic neuropathy and maculopathy. METHODS: A retrospective chart review of consecutive patients over 4.5 years who were referred to neuro-ophthalmology clinics with the diagnosis of optic neuropathy but whose final diagnosis was maculopathy. Patient demographics, mode of presentation, clinical profile, complete ophthalmological examination, results of all ancillary testing, and final diagnosis were recorded. RESULTS: A total of 47 patients (27 women) were included. The median age was 55 years (range, 18-85). Most referrals were by ophthalmologists (72.3%) and optometrists (12.8%). The diagnosis of maculopathy was made in 51.1% of patients at the time of first neuro-ophthalmic consultation. Only 6.4% patients (3) had relative afferent pupillary defect. Benign disc anomalies (tilted, myopic, small, or anomalous discs) were present in 34.0%, and 21.3% had pathologic disc changes unrelated or secondary to maculopathy. Macular ocular coherence tomography (OCT) was abnormal in 84.4% (with outer retinal pathology in 42.2% and inner retina pathology in 17.8%). Retinal nerve fiber layer (RNFL) thickness was normal in 82.6% of patients. CONCLUSIONS: Macular OCT is a high-yield test in differentiating between optic neuropathy and maculopathy and should be obtained in patients with suspected optic neuropathies who have normal RNFL thickness. Macular dystrophies, particularly cone dystrophies, unspecified retinal disorders, and macular degeneration were the most common mimics of optic neuropathy. The diagnosis was often present on OCT of the macula. The presence of coexistent benign and pathological disc anomalies may lead to maculopathy being misdiagnosed as optic neuropathy.

The Causes of Optic Disc Edema in Patients Presenting With Significantly Compromised Vision.

BACKGROUND: To evaluate the most common causes of optic disc edema (ODE) in patients with significantly compromised vision (initial best-corrected visual acuity [BCVA] of 20/400 or worse) at presentation. METHODS: Retrospective chart review over a 5-year period of consecutive patients presenting to tertiary neuro-ophthalmology clinics at the University of Toronto. RESULTS: A total of 656 patients with ODE were included, and 49 patients (7.47%) had an initial BCVA of 20/400 or worse. There were 54 eyes included at baseline and 49 eyes at final follow-up. There were 29 female and 20 male patients. The mean age at first visit across patients was 55.9 years. Female patients (n = 29) were significantly older than male patients (n = 20) (P < 0.05). The causes of ODE were optic neuritis (ON) (n = 22; 40.7%), nonarteritic anterior ischemic optic neuropathy (NAION) (n = 22; 40.7%), arteritic anterior ischemic optic neuropathy (AAION) (n = 5; 9.26%), uveitis-related (n = 3; 5.56%), papilledema from idiopathic intracranial hypertension (IIH) (n = 1; 1.85%), and Vogt-Koyanagi-Harada disease (n = 1; 1.85%). Initial BCVA was not significantly different between ON and NAION groups (P = 0.52); however, final BCVA was significantly better in the ON group (P < 0.0001). The mean initial BCVA was worst in the AAION group (2.62 ± 0.54 logarithm of the minimum angle of resolution). The most common cause of ODE in patients <40 years old was ON (83.3%), whereas the 2 most common causes in patients >80 were NAION (60%) and AAION (40%). In patients between the ages of 60-80, NAION (100%) was the only cause. CONCLUSIONS: Patients with ODE and poor vision at presentation represent a minority of cases seen in neuro-ophthalmology clinics (<10%). Optic neuritis and NAION are the 2 most common causes of ODE with poor vision at presentation. These findings are limited by a small sample size and potential sampling bias.

Anemia and Idiopathic Intracranial Hypertension: A Case-Control Study.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a condition that mostly affects obese women of childbearing age but has been reported to be temporally related to new diagnoses of anemia. Despite these reports, the association between anemia and IIH remains controversial. The present study aimed to understand the strength of association between anemia and IIH through a case-control design. METHODS: Consecutive IIH patients were recruited from neuro-ophthalmology clinics and matched by age and sex to consecutive patients attending neuro-ophthalmology clinics with diagnoses other than IIH. Complete blood counts (CBCs) were reviewed within 6 months of neuro-ophthalmology presentation, and anemia was diagnosed by hemoglobin of <120 g/L in women and <130 g/L in men. Anemia was classified as microcytic (mean corpuscular volume (MCV) < 80 fL), normocytic (MCV 80-100 fL), and macrocytic (MCV > 100 fL) as well as mild (hemoglobin >110 g/L), moderate (hemoglobin 80-109 g/L), and severe (hemoglobin <80 g/L). RESULTS: One hundred twenty-three IIH patients and 113 controls were included in the study. More IIH patients than controls had anemia (22.8%, 28/123 vs 10.6%, 12/113, P = 0.01) with an odds ratio of 2.48 (95% CI: 1.19, 5.16). There were no differences in severity of anemia or MCV between IIH patients and controls, with anemia most often being mild (57.1%, 16/28 vs 75%, 9/12) and normocytic (50.0%, 14/28 vs 50.0%, 6/12). However, 17.9% (5/28) of IIH patients had severe anemia compared with zero in the control group. IIH patients had a higher mean platelet count than controls ( P < 0.001), and there were no differences on leukocyte counts. CONCLUSION: Idiopathic intracranial hypertension patients have a higher prevalence of anemia compared with non-IIH neuro-ophthalmology patients. The anemia was mostly mild and may be explained by other factors related to worse health status and obesity, including low socioeconomic status, poor nutrition, and polycystic ovary syndrome. However, as a CBC is a widely available test and anemia may influence the IIH disease course, we propose that this test be obtained in all patients with new papilledema.

Comparison of Idiopathic Intracranial Hypertension Patients With and Without Anemia.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a condition of elevated intracranial pressure without an identifiable cause. It mostly affects young obese women but has been reported in individuals newly diagnosed with anemia or with chronic anemia. The relationship between anemia and IIH is not well elucidated. This study aims to characterize the course of IIH in anemic patients. METHODS: Consecutive patients with IIH were recruited from neuro-ophthalmology clinics. Subsequent retrospective chart review obtained information on symptoms, complete blood counts (CBCs), visual acuity, visual fields, and optic disc edema at baseline and follow-up. Anemia was diagnosed by hemoglobin <120 g/L (women) and <130 g/L (men). RESULTS: One hundred twenty-three patients with IIH were recruited for this study, and 22.8% (28/123) had anemia. More anemic individuals had mild-to-moderate visual acuity impairment (logarithm of minimum angle of resolution 0.3-1, P = 0.01) and worse automated mean deviation ( P = 0.048). The median follow-up time was 47.4 weeks (interquartile range:20.1-91.8). Fifteen of 28 patients with anemia received anemia treatment and showed a trend toward increased hemoglobin. At follow-up, there were no differences in the visual acuity, but patients with anemia had worse automated mean deviation on visual fields ( P = 0.045). CONCLUSIONS: IIH patients with anemia had worse visual function at presentation and worse final visual field parameters. This suggests that CBCs should be obtained for patients with papilledema because it may influence final visual outcome.

Antivirals vs Antivirals and Steroids for Treatment of Herpes Zoster-Related Ophthalmoplegia: A Case Series and Systematic Review.

BACKGROUND: Up to 1/3 of patients with herpes zoster ophthalmicus (HZO) may develop ophthalmoplegia. Although zoster-related ophthalmoplegia (ZO) is typically treated with antiviral agents, there is controversy regarding the therapeutic role of systemic steroids. METHODS: This was a retrospective case series and case report-based systematic review. For the case series, participants were recruited from tertiary neuro-ophthalmology clinics. Eligible participants were those who developed cranial nerve palsies (CNP) within 1 month of HZO diagnosis. In the systematic review, all adults with ZO in the literature who were treated with antivirals or steroids only, or combination therapy were included. Main outcomes were initial presentation, investigations, neuroimaging, treatment regimen, and final outcomes of ophthalmoplegia. RESULTS: Eleven immunocompetent patients with ZO were included. The most common CNP was CN III (5/11), followed by CN VI (2/11) and CN IV (2/11). One patient had multiple CNPs. All patients were treated with antivirals, and 4 also treated with a short course of oral steroids. At 6-month follow-up, 75% of patients treated with combination therapy and 85.7% treated with antivirals alone had complete recovery of ZO. The systematic review identified 63 studies consisting of 76 cases of ZO. When comparing patients treated with antivirals with those treated with antivirals and steroids, patients on combination therapy had more severe ocular findings, including complete ophthalmoplegia (P < 0.001). Age was the only significant predictor of complete recovery of ophthalmoplegia on multivariable logistic regression (P = 0.037). CONCLUSIONS: The rate of complete recovery in immunocompetent patients with ZO was similar in patients treated with antivirals alone vs those treated with antivirals and oral steroids. The systematic literature review affirmed these findings. However, age may influence recovery of ophthalmoplegia.

Prevalence of Person-First Language in Idiopathic Intracranial Hypertension: A Systematic Review of Case Reports.

BACKGROUND: Person-first language (PFL) is a linguistic prescription, which places a person before their disease. It is considered an important tool to reduce stigma. However, PFL is not routinely used across the scientific literature, particularly in patients with overweight or obesity. Patients with idiopathic intracranial hypertension (IIH) face various stigmas through high rates of poverty, female gender, and frequent rates of comorbidities. Non-PFL language use intersects and worsen the health inequities faced by these patients. METHODS: A systematic review of case reports. MEDLINE and EMBASE were searched for all case reports with "pseudotumor cerebri" [MESH] OR "Idiopathic Intracranial Hypertension" as key word between January 1974 and August 2022. The primary criterion was the article's inclusion of patients with overweight or obesity. The secondary criterion was the article's discussion regarding obesity as risk factor. Articles not meeting primary or secondary criteria were excluded. RESULTS: Approximately 514/716 (71.8%) articles used non-PFL language. The publication year was predictive of non-PFL language: 1976-1991 (82.3%) vs 1992-2007 (72.3%, P = 0.0394) and 2008-2022 (68.3%, P = 0.0056). Non-PFL was significantly higher in obesity compared with other medical conditions (60.3% vs 7.3%, P < 0.001). The patient gender (P = 0.111) and ethnicity (P = 0.697), author's specialty (P = 0.298), and primary English-speaking status (P = 0.231), as well as the journal's impact factor (P = 0.795), were not predictive of non-PFL. CONCLUSIONS: Most literature focused on IIH use non-PFL when discussing overweight or obesity, regardless of the patient's gender and ethnicity, journal's impact factor, senior author's specialty, and English-speaking status. Non-PFL use is much more common when discussing obesity compared with other medical conditions. Appropriate use of PFL can decrease stigma and, more importantly, decrease the intersectionality of health stigma faced by patients with IIH.

Inappropriate Indexing of Case Reports to the "Papilledema" Subject Heading: A Systematic Review.

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.

A technique for excisional optic nerve biopsy.

PURPOSE: To describe a transconjunctival technique for full-thickness (excisional) optic nerve biopsy. METHOD: A medial transconjunctival approach to the optic nerve with disinsertion of the medial rectus is used. A small right-angle Mixter forcep is used to clamp the optic nerve far posteriorly, and then a microscalpel is directed metal-on-metal to cut the posterior optic nerve. The cut nerve is then rotated anteriorly to complete the proximal nerve cut. RESULT: A full-thickness specimen of 11 mm of more can be obtained without undue traction on the globe. The globe remains viable. CONCLUSION: A long length, excisional optic nerve biopsy can be readily and safely performed without endoscopic techniques.

Diagnosis of Acute Promyelocytic Leukemia After Presentation to Neuro-Ophthalmology.

BACKGROUND: Acute promyelocytic leukemia (APML) is a medical emergency that can initially present with neuro-ophthalmologic signs. Early recognition is crucial, and immediate treatment with all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) can be life-saving. The goal of this study was to describe patients who first presented to neuro-ophthalmology and were subsequently diagnosed with APML. METHODS: We retrospectively reviewed consecutive patients seen at a tertiary neuro-ophthalmology centre. Patients with an unknown diagnosis of APML at presentation who subsequently went on to receive this diagnosis were included. Clinical characteristics, neuro-ophthalmologic findings, and outcome were retrieved. RESULTS: A total of 3 patients (2 women and 1 men) with a mean age of 30.7 (range 24-33) years were included in the study. Neuro-ophthalmologic diagnoses at presentation were severe hemorrhagic papilledema related to dural venous sinus thrombosis, hemorrhagic bilateral optic disc edema, and left homonymous hemianopia related to an occipital lobe hemorrhage. At diagnosis, the average hemoglobin was 83.7g/L (range 78-104), and the platelet count was 39.3 × 109/L (range 15-77). All patients were treated with ATRA and ATO. One patient developed papilledema and sixth nerve palsies related to this treatment, which resolved with acetazolamide. Clinical follow-up ranged from 6 to 12 months, and all patients were in clinical remission about systemic APML. CONCLUSION: Neuro-ophthalmologic symptoms may be the first manifestations of APML, and a complete blood count is an essential test in patients presenting with optic disc edema, especially if hemorrhagic.

No Light Perception Vision in Neuro-Ophthalmology Practice.

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.

Paracentral Acute Middle Maculopathy Associated With Anterior Ischemic Optic Neuropathy and Cilioretinal Artery Occlusion in Giant Cell Arteritis.

ABSTRACT: Paracentral acute middle maculopathy (PAMM) is a relatively new optical coherence tomography finding, defined by hyperreflectivity in the inner nuclear layer. In this article, we present a case of a 73-year-old woman who presented with transient vision loss followed by the sudden onset of complete vision loss to counting fingers at 1 foot for one day in the left eye. Dilated examination showed a right cotton wool spot, left pallid optic disc edema, and retinal edema in the distribution of the cilioretinal artery. OCT demonstrated hyperreflective band at the level of the inner nuclear layer, compatible with PAMM. Clinical and laboratory findings were consistent with GCA, for which she was prescribed high-dose oral prednisone, with confirmation of GCA on a subsequent temporal artery biopsy. PAMM may be seen in the context of GCA, and OCT of the macula serves as an important adjunct to define the retinal manifestations of this condition.