Optic Neuritis and Cranial Neuropathies Diagnosis Rates before Coronavirus Disease 2019, in the Initial Pandemic Phase, and Post-Vaccine Introduction.

PURPOSE: To compare rates of diagnosis of neuro-ophthalmic conditions across the Coronavirus Disease 2019 (COVID-19) pandemic with pre-pandemic levels. DESIGN: Multicenter, retrospective, observational study. PARTICIPANTS: Patients seen for eye care between March 11, 2019, and December 31, 2021. METHODS: A multicenter electronic health record database, Sight Outcomes Research Collaborative (SOURCE), was queried for new diagnoses of neuro-ophthalmic conditions (cranial nerve [CN] III, IV, VI, and VII palsy; diplopia; and optic neuritis) and new diagnoses of other ophthalmic conditions from January 1, 2016, to December 31, 2021. Data were divided into 3 periods (pre-COVID, pre-COVID vaccine, and after introduction of COVID vaccine), with a 3-year look-back period. Logistic regressions were used to compare diagnosis rates across periods. Two-sample z-test was used to compare the log odds ratio (OR) of the diagnosis in each period with emergent ocular conditions: retinal detachment (RD) and acute angle-closure glaucoma (AACG). MAIN OUTCOME MEASURES: Diagnosis rate of neuro-ophthalmic conditions in each study period. RESULTS: A total of 323 261 unique patients (median age 59 years [interquartile range, 43-70], 58% female, 68% White) across 5 academic centers were included, with 180 009 patients seen in the pre-COVID period, 149 835 patients seen in the pre-COVID vaccine period, and 164 778 patients seen in the COVID vaccine period. Diagnosis rates of CN VII palsy, diplopia, glaucoma, and cataract decreased from the pre-COVID period to the pre-vaccine period. However, the optic neuritis diagnoses increased, in contrast to a decrease in RD diagnoses (P = 0.021). By comparing the diagnosis rates before and after widespread vaccination, all eye conditions evaluated were diagnosed at higher rates in the COVID vaccination period compared with pre-COVID and pre-vaccine periods. The log OR of neuro-ophthalmic diagnosis rates across every period comparison were largely similar to emergency conditions (RD and AACG, P > 0.05). However, the log OR of cataract and glaucoma diagnoses were different to RD or AACG (P < 0.05) in each period comparison. CONCLUSIONS: Neuro-ophthalmic diagnoses had a similar reduction in diagnosis rates as emergent eye conditions in the first part of the pandemic, except optic neuritis. After widespread COVID-19 vaccination, all ophthalmic diagnosis rates increased compared with pre-pandemic rates, and the increase in neuro-ophthalmic diagnosis rates did not exceed the increase in RD and AACG diagnosis rates. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Clinical Characteristics of Idiopathic Intracranial Hypertension in Older Adults.

BACKGROUND: Idiopathic intracranial hypertension (IIH) is a condition that classically affects obese women of child-bearing age. However, it is sometimes encountered in older patients. The purpose of this study was to help clinicians better understand how this disease can present differently in these age groups. METHODS: This is a retrospective chart review from a single academic center of baseline characteristics of adult patients diagnosed with IIH based on the modified Dandy criteria. The patients were divided into 2 groups: (1) those 18-44 years old and (2) those older than 45 years at diagnosis. RESULTS: One hundred sixty-seven patients were identified; 135 in the younger group and 32 in the older group. The younger group had a higher rate of headaches (90% vs 63%, P = 0.0004), higher body mass index (38.9 vs 36.1, P = 0.046), higher opening pressure (38 vs 31 cm H2O, P = 0.005), and thicker peripapillary retinal nerve fiber layer average thickness (right eye 178 vs 131 µm, P = 0.02; left eye 184 vs 136 µm, P = 0.045). The older group had higher rates of empty sella (90% vs 62%, P = 0.0039). In addition in the younger group, there was a trend toward higher rates of pulsatile tinnitus (63% vs 45%, P = 0.08), transient visual obscurations (50% vs 32%, P = 0.07), and lower rates of spontaneous cerebrospinal fluid leak (4% vs 13%, P = 0.08). Sex, rates of obesity, other MRI findings typical of elevated intracranial pressure, frequency and Frisen grading of papilledema, and visual field loss were not statistically different between the groups. CONCLUSIONS: The older age group had milder signs and symptoms of IIH and a higher prevalence of empty sella than the younger group, but otherwise had typical characteristics. These findings suggest that IIH in the older age group may represent milder chronic disease that was previously undiagnosed.

Objective Diplopia Outcomes for Patients Treated With Teprotumumab for Thyroid Eye Disease.

BACKGROUND: Thyroid eye disease (TED) is a condition caused by inflammatory damage to the periocular tissue that often leads to double vision. Teprotumumab is an insulin-like growth factor 1 receptor antibody that was FDA approved for the management of TED in 2020, although much is yet to be elucidated regarding its effects on diplopia outcomes among patients with TED. Diplopia is a significant and life-altering effect of TED. Previous studies have reported the effect of teprotumumab on double vision subjectively using the Gorman diplopia score. However, there is a gap in the literature addressing the effect of teprotumumab treatment on objective ocular alignment measures. The purpose of our study was to address this gap. METHODS: We performed a retrospective review of patients who were diagnosed with TED, presented with diplopia, and treated with teprotumumab in a single-center academic ophthalmology practice. The primary outcome was change in ocular alignment in primary gaze position at 6 months (completion of teprotumumab treatment). Secondary outcomes included change in ocular alignment in other gaze positions, proptosis, eyelid position, and clinical activity score (CAS) at 6 months compared with baseline. To determine what factors may predict ocular alignment response to teprotumumab, we analyzed baseline characteristics among 3 groups, divided based on whether ocular alignment was worsened, stable, or improved at 6 months. RESULTS: Seventeen patients met inclusion criteria, 3 (18%) worsened, 10 (59%) were stable, and 4 (24%) improved. CAS ( P = 0.02) was significantly different among the groups and was higher in those who worsened and those who improved compared with those who remained stable. Right gaze horizontal prism deviation ( P = 0.01) and left gaze horizontal prism deviation ( P = 0.03) were significantly different among the groups, with a greater degree of left gaze horizontal prism deviation in the worse group than the stable group ( P = 0.04). CONCLUSIONS: Our study demonstrated that most patients remained stable after teprotumumab treatment regarding ocular alignment in primary gaze and the number of patients who improved was slightly higher than the number of patients who worsened after teprotumumab treatment. There are some baseline measures, such as CAS and right and left gaze horizontal prism deviation that can help better predict how a patient will respond to teprotumumab treatment. Our results can better inform physicians of how to counsel patients with TED when considering teprotumumab therapy.

Stability of Ocular Alignment After Teprotumumab Therapy in a Cohort of Patients With Thyroid Eye Disease and Baseline Diplopia.

BACKGROUND: Teprotumumab is a monoclonal antibody that is effective in treating patients with thyroid eye disease (TED) and has been shown to produce subjective improvements in diplopia in this group. The aims of this study were to evaluate the rate and timing of recurrence/worsening of diplopia after teprotumumab treatment in a cohort of patients with TED presenting with diplopia at baseline. METHODS: A retrospective chart review of 15 patients with diplopia secondary to TED, treated with teprotumumab, was conducted in a single-center academic institution. The primary outcome was the rate of recurrence/worsening of diplopia after completing teprotumumab. Secondary outcomes include the time to recurrence/worsening of diplopia and clinical activity scores (CAS) to correlate with changes in ocular alignment. RESULTS: Fifteen patients met the inclusion criteria for this study, and 7 of these had recurrence of diplopia (46.7%). Two patients had worsening of CAS to ≥4, 6 had worsening of CAS to 3, and the other 7 had CAS ≤2 during the follow-up period. The mean follow-up period was 20.4 months (SD 7.2) after completing teprotumumab. The mean time to diplopia recurrence/worsening was 8.8 months (range 6.7-12.2, SD 1.8). CONCLUSIONS: Patients with TED and baseline diplopia have a substantial rate of recurrence/worsening of diplopia after teprotumumab therapy, suggesting that they may not have stable ocular alignment immediately after treatment. Strabismus surgeons may need to weigh the significant risk of disease relapse when planning optimum timing for surgical correction.

Optic Disc Cupping in Neuromyelitis Optica Spectrum Disorder, Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease, and Multiple Sclerosis and Its Relationship With Optical Coherence Tomography Parameters: A Multicenter Study.

BACKGROUND: Although cupping of the optic nerve is classically a sign of glaucomatous optic neuropathy, it has been shown that cupping can sometimes occur after an episode of optic neuritis (ON). The purpose of this study was to compare cupping in patients after ON from multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) and to investigate the relationship between cupping and retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) thinning. METHODS: This was a retrospective cohort involving patients (≥18 years) with ON from 3 institutions. Patients were eligible if they had optical coherence tomography (Cirrus, OCT) performed ≥6 months after a single unilateral ON. The amount of thinning and cupping was estimated from the difference in the OCT parameters between affected and unaffected eyes. Univariable and multivariable regressions were used to investigate the relationship between cupping and ON etiology. Pearson correlation was used to investigate the relationship between cupping and RNFL and GCC. RESULTS: Eighty-six subjects (MS: 35, NMOSD: 26, and MOGAD: 25) were included. There was no significant difference in gender and race between the groups, and most patients (86.1%) were female. Patients with NMOSD were significantly older than patients with MS or MOGAD (P = 0.002). In the univariate model, cupping was significantly higher in the NMOSD group (P = 0.017); however, after adjusting for age, GCC, and RNFL of the affected eye, the difference was no longer statistically significant (P = 0.949). The correlation between cupping asymmetry and RNFL and GCC of the affected eye was inversely strong in patients with MS (R = -0.60 and R = -0.64, respectively), inversely moderate in patients with MOGAD (R = -0.34 and R = -0.40, respectively), and weak in patients with NMOSD (R = -0.03 and R = -0.17, respectively). CONCLUSIONS: Our results demonstrated that cupping after ON is correlated with RNFL and GCC thinning; although cupping was overall greater in the NMOSD group, once adjusted for age, RNFL, and GCC, it did not differ among patients with MS, NMOSD, and MOGAD.

Identification of Factors that May Predict Career Trajectory Among Neuro-Ophthalmology Fellows.

BACKGROUND: There is modest literature regarding fellowship applicant factors that may predict future career achievement. We aim to characterize neuro-ophthalmology fellows and identify and analyze characteristics that may predict future career trajectory. METHODS: Data, including demographic information, academic background, scholarly activities, and practice information, were collected using publicly available sources, on individuals who completed neuro-ophthalmology fellowships from 2015 to 2021. Summary statistics describing the cohort were calculated. Prefellowship characteristics were compared with postfellowship characteristics to evaluate which prefellowship characteristics may predict postfellowship academic productivity and career achievement. RESULTS: Data were collected on 174 individuals (41.6% men, 58.4% women). Sixty-five percent were residency-trained in ophthalmology, 31% neurology, 1.7% both, and 1.7% pediatric neurology. Fifty-eight percent completed residency in the US, 8% in Canada, 32% internationally, and 2% in multiple locations. Among those practicing in the US/Canada, 63.8% practice at academic centers, 35.3% private practice, and 0.9% at both. Thirty-one percent completed additional subspecialty training and 17.8% additional graduate degrees. Completion of additional fellowship training or graduate degrees, and publication of more papers before fellowship, correlated with later academic productivity. There were no significant correlations between completion of an additional fellowship or graduate degree with current practice environment or attainment of leadership roles. There were no significant correlations between total publishing productivity prefellowship and practice environment or leadership roles postfellowship. CONCLUSIONS: Additional graduate degrees/subspecialty training, and prefellowship academic productivity, correlated with later academic productivity among neuro-ophthalmologists, suggesting that these metrics may be helpful in predicting future academic performance among fellowship applicants.

Evaluation of a STAT MRI Protocol for Emergent Ophthalmology Patients.

BACKGROUND: Evaluating patients with potentially sight-threatening conditions frequently involves urgent neuroimaging, and some providers recommend expediting emergency department (ED) evaluation. However, several factors may limit the practicality of ED evaluation. This pilot study assessed the feasibility and safety of a STAT magnetic resonance imaging (MRI) protocol, designed to facilitate outpatient MRI within 48 hours of referral, compared with ED evaluation for patients with optic disc edema. METHODS: A retrospective chart review was performed. Demographics, clinical data, and baseline ophthalmic measures were compared between patients in STAT and ED groups using the t test or Fisher exact test. Multivariate analyses compared changes in visual acuity (VA), visual field mean deviation (VF MD), retinal nerve fiber layer thickness, and edema grade between presentation and follow-up using a mixed-effects model adjusting for age, sex, and baseline measures. RESULTS: A total of 70 patients met the study criteria-24 (34.3%) in the STAT MRI cohort and 46 (65.7%) in the ED cohort. Demographic variables were similar between groups. Patients referred to the ED had worse VA ( P < 0.001), larger VF MD ( P < 0.001), and higher edema grade ( P = 0.002) at presentation. Four patients in the ED group and none in the STAT group were found to have space-occupying lesions. Multivariate analyses showed that follow-up measures were significantly associated with their baseline values (all P < 0.001) but not with referral protocol (all P > 0.099). The STAT MRI protocol was associated with lower average patient charges and hospital costs. CONCLUSIONS: The STAT MRI protocol did not result in inferior visual outcomes or delay in life-threatening diagnoses. Urgent outpatient evaluation, rather than ED referral, seems safe for some patients with optic disc edema. These findings support continued utilization of the protocol and ongoing improvement efforts.

Big Data Analysis of Inflammatory Conditions Associated With Optic Neuritis.

BACKGROUND: Previous studies in the United States established multiple sclerosis (MS) as the most common cause of optic neuritis (ON). ON can be associated with other systemic inflammatory conditions including sarcoidosis, neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and lupus; however, prospective studies to establish risk of ON associated with these diseases are lacking. Furthermore, appropriate workup for ON is still debated. METHODS: A deidentified electronic medical record of a tertiary care academic center was searched for ON and rheumatologic/neuro-inflammatory diseases in the medical history, diagnoses, and laboratory results; followed by the intersection of ON with each condition. We calculated frequency of systemic conditions among patients with ON and prevalence of ON in those conditions. We also calculated relative risk (RR) of underlying systemic conditions among patients with ON compared with the study patient population. RESULTS: In 6.7 million charts, 5,344 cases of ON were identified. Among those, MS occurred most commonly (20.6%), followed by NMOSD (10.5%). Conversely, ON occurred in 98.4% of NMOSD cases, 53.3% of MOGAD, and 10.0% of MS. NMOSD (RR = 1,233), MOGAD (RR = 688), and MS (RR = 126) had the highest RR among the conditions we evaluated. The subset analysis showed similar findings. CONCLUSIONS: The high RR for ON among patients with NMOSD and MOGAD suggests that clinical suspicion for ON should be high among patients with these conditions presenting with vision changes. Conversely, MS and NMOSD should initially be high on the differential diagnosis for any patient presenting with optic neuritis.

Baseline Characteristics and Clinical Presentation of Biopsy-Proven Giant Cell Arteritis in White Compared with Black Patients.

BACKGROUND: Giant cell arteritis (GCA) is the most prevalent systemic vasculitis in the elderly and can lead to permanent vision loss if left untreated. Most earlier studies have evaluated GCA in primarily white populations, and GCA was traditionally thought to occur at nearly negligible frequency in black populations. Our previous study showed that GCA may occur at similar rates in white and black patients, but little is known about the presentation of GCA in black patients. The purpose of this study is to examine baseline presentation of biopsy-proven GCA (BP-GCA) in a tertiary care center-based population with a sizeable proportion of black patients. METHODS: Retrospective study from a single academic institution of a previously described cohort of BP-GCA. Presenting symptoms, laboratory findings, and GCA Calculator Risk score were compared in black and white patients with BP-GCA. RESULTS: Among 85 patients with biopsy-proven GCA, 71 (84%) were white and 12 (14%) were black. White patients had higher rates of elevated platelet count (34% vs 0%, P = 0.04), whereas black patients had higher rates of diabetes mellitus (67% vs 12%, P < 0.001). There were no statistically significant differences in age, gender, biopsy classification (active vs healed arteritis), cranial symptoms, visual symptoms/ophthalmic findings, rates of abnormal erythrocyte sedimentation rate or C-reactive protein, unintentional weight loss, polymyalgia rheumatica, or GCA risk calculator score. CONCLUSIONS: Presenting features of GCA were similar between white and black patients in our cohort, except for rates of abnormal platelet level and diabetes. Physicians should feel comfortable relying on the usual clinical features for the diagnosis of GCA independent of race.

Presenting Features of Giant Cell Arteritis with Active Versus Healed Arteritis on Biopsy.

Giant cell arteritis (GCA) is often categorised as "active" or "healed" on temporal artery biopsy (TAB). The purpose of this study was to compare the initial clinical presentation of patients with GCA according to active versus healed arteritis on TAB. A retrospective chart review was performed for patients with biopsy-proven GCA (BP-GCA) at a single academic medical institution from a previously reported cohort. The arteritis on TAB was categorised as "active" or "healed" based on the pathological reports. Demographic information, clinical presentation, past medical history, and test results were collected from the date of TAB. These baseline characteristics were entered into the GCA Risk Calculator. Of 85 patients with BP-GCA, 80% had active and 20% had healed disease according to histopathology. A higher percentage of those with active arteritis had ischaemic optic neuropathy (ION) (36% versus 6%, p = .03), elevated erythrocyte sedimentation rates (92% versus 63%, p = .01), elevated C-reactive protein levels (79% versus 46%, p = .049), GCA risk score > 7.5% (99% sensitivity, 100% versus 71%, p < .001), higher mean GCA risk calculator scores (neural network p = .001; logistic regression p = .002). Patients with healed arteritis were less likely to have visual manifestations than the active arteritis group (38% versus 71%, p = .04). Patients with active vasculitis on biopsy had higher rates of ION and elevated inflammatory markers, as well as higher predictive scores from the GCA risk calculator. Further research is needed regarding correlation of biopsy findings and risk of complications or relapses.

Effect of Temporal Artery Biopsy Length and Laterality on Diagnostic Yield.

BACKGROUND: Giant cell arteritis (GCA) is the most common vasculitis in adults and is associated with significant morbidity and mortality. Temporal artery biopsy (TAB) remains the gold standard for diagnosis in the United States; however, practices vary in the length of artery obtained and whether bilateral simultaneous biopsies are obtained. METHODS: Retrospective chart review of all TABs performed at the Johns Hopkins Wilmer Eye Institute between July 1, 2007, and September 30, 2017. RESULTS: Five hundred eighty-six patients underwent TAB to evaluate for GCA. Of 404 unilateral biopsies, 68 (16.8%) were positive. Of 182 patients with bilateral biopsies, 25 (13.7%) had biopsies that were positive and 5 patients (2.7%) had biopsies that were discordant, meaning only 1 side was positive. There was no significant difference in the average postfixation length of positive and negative TAB specimens (positive mean length 1.38 ± 0.61 cm, negative mean length 1.39 ± 0.62 cm, P = 0.9). CONCLUSIONS: There is no significant association between greater length of biopsy and a positive TAB result in our data. Although the rate of positive results was not higher in the bilateral group compared with the unilateral group, 2.7% of bilateral biopsies were discordant, similar to previously published rates. Overall, this suggests that initial bilateral biopsy may increase diagnostic yield, albeit by a small amount.

Orbital inflammation in the setting of a nylon foil implant.

A 61-year-old man underwent left medial wall and floor fracture repair with a Suprafoil® implant. He had postoperative orbital congestion and lower eyelid swelling that persisted for over seven weeks. Examination demonstrated hyperglobus with supraduction, infraduction, and adduction deficits. Imaging revealed a 3.7 × 3.6 × 2.6 cm isodensity along the implant, thought to be hematoma. The patient elected to pursue exploration and possible drainage. Intraoperatively, there was no hematoma; rather, we found a fibroinflammatory rind along the periorbita surrounding the implant. This was biopsied, and the implant was removed, as the fractures had sufficiently healed. Pathology showed dense fibroconnective tissue with associated inflammation. The patient completed a steroid taper with improvement in all symptoms and resolution of diplopia. To our knowledge, this is the first reported case of such a prominent orbital inflammatory reaction to nylon foil, a departure from the delayed hematic cysts typically associated with these implants.

Neuro-Ophthalmic Manifestations of Acute Leukemia.

BACKGROUND: Ophthalmic involvement in acute leukemia is common, with 36% of patients having ophthalmic involvement at the time of diagnosis. However, neuro-ophthalmic involvement is relatively rare. We present a characterization of neuro-ophthalmic findings in patients with acute leukemia and discuss the implications of these findings on patient management and prognosis. METHODS: We performed a retrospective review of cases of acute leukemia with central nervous system (CNS) involvement and neuro-ophthalmic manifestations that were evaluated at the Wilmer Eye Institute between January 2013 and September 2019. Data collected included demographic information, leukemia details, results of diagnostic testing, and features of associated neuro-ophthalmic manifestations. RESULTS: Twelve patients with mean age 42 years (range 9-65, median 39) were included. Seven (58%) patients were men and 5 (42%) women. Eight (67%) were diagnosed with acute myeloid leukemia and 4 (33%) with acute lymphoid leukemia. Neuro-ophthalmic findings included 4 patients with isolated sixth nerve palsies, 2 with multiple cranial nerve palsies, 2 with orbital lesions with proptosis, 4 with optic disc swelling, and 1 with isolated fourth nerve palsy. Five (42%) neuro-ophthalmic presentations were associated with known CNS disease, 3 (25%) were associated with active disease but heralded the discovery of CNS involvement, 3 (25%) were the presenting features of relapse, and 1 (8%) led to the original leukemia diagnosis. Neuroimaging showed 4 with leptomeningeal enhancement, 4 with cranial nerve enhancement/thickening, 3 with optic nerve/sheath enhancement, 1 with lytic lesion of bone, 1 with soft tissue mass, and 1 with cytotoxic brain edema. One case had normal neuroimaging. Overall, patients had a poor prognosis, with 7 patients dying from leukemia or its complications and only 1 achieving a sustained remission. In 58% of the cases in our series, the discovery of neuro-ophthalmic leukemic involvement directly led to a change in leukemia treatment. CONCLUSIONS: Neuro-ophthalmic manifestations of leukemia may occur as presenting features of diagnosis, relapse, or CNS involvement, and portend a poor prognosis. Detection of neuro-ophthalmic involvement often triggers a prompt change in management. Therefore, familiarity with potential neuro-ophthalmic presentations of acute leukemia may avoid delayed diagnosis, and resultant inadequate treatment, of primary disease, relapse, or CNS involvement.

Neuro-Ophthalmic Manifestations of Sarcoidosis.

BACKGROUND: Sarcoidosis is an idiopathic, multisystem, inflammatory disease that has central nervous system involvement in 5%-15% of cases. The presentation of neurosarcoidosis is highly variable, and the MRI findings often mimic the appearance of other central nervous system diseases. Therefore, the diagnosis can be challenging. About one-third of neurosarcoidosis cases have neuro-ophthalmic manifestations and, thus, may be evaluated by a neuro-ophthalmologist early in the disease course. METHODS: We performed a retrospective review of 22 cases of biopsy-proven sarcoidosis with neuro-ophthalmic manifestations, seen at the Wilmer Eye Institute from January 2013 to September 2019, in which we described the demographic information, clinical presentations, neuroimaging findings, and diagnostic evaluations. RESULTS: Twenty-two patients were included. Fifteen patients identified as black and 7 as white. Fifteen were women, and 7 men. Mean age at sarcoidosis diagnosis was 45.9 years (range 26-66). Neuro-ophthalmic findings included optic neuropathy in 11 (50%); proptosis/orbital inflammation in 5 (23%); abducens palsy in 5 (23%); trochlear palsy, trigeminal distribution numbness, and bitemporal hemianopia in 2 each (9%); and oculomotor palsy, facial palsy, optic perineuritis, dorsal midbrain syndrome, central vestibular nystagmus, and papilledema in 1 each (5%). Eight (36%) had a pre-existing diagnosis of sarcoidosis; however, in 14 (64%), the neuro-ophthalmic presentation led to the sarcoidosis diagnosis. Patients with a pre-existing sarcoidosis diagnosis were younger than those without this diagnosis (38.5 vs 50.1 years, P = 0.035). Brain MRI was abnormal in all but 1 case (95%). In patients without a pre-existing sarcoidosis diagnosis, all 7 tested for angiotensin converting enzyme (ACE) had normal values, and 75% of the 12 who had computed tomography (CT) chest had findings suggestive of sarcoidosis (86% of black patients vs 50% of white patients). CONCLUSIONS: Patients with neurosarcoidosis may present initially with a neuro-ophthalmic manifestation, and this presentation may be more common in older patients. MRI usually is abnormal, although findings may be nonspecific. Serum testing for ACE is not helpful. Normal CT chest does not rule out underlying sarcoidosis, particularly in white patients.

Neurosarcoidosis Masquerading as Giant Cell Arteritis With Incidental Meningioma.

ABSTRACT: We present a case of vision loss secondary to neurosarcoidosis, which initially presented with severe bilateral vision loss, temporal headaches, and elevated erythrocyte sedimentation rate, concerning for giant cell arteritis. However, temporal artery biopsy was negative. Initial neuroimaging features were misinterpreted to represent a meningioma that did not account for his clinical presentation. Clinical course, including atypically rapid enlargement of presumed meningioma, development of skin lesions, appearance of optic nerve enhancement on MRI, and steroid response, strongly increased suspicion for sarcoidosis. Biopsy of a skin lesion demonstrated noncaseating granulomatous inflammation, consistent with sarcoidosis.

Acute Sixth Nerve Palsy and Retinal Hemorrhages as the Presenting Features of Acute Lymphoblastic Leukemia.

ABSTRACT: A 36-year-old apparently healthy man presented with acute onset of diplopia. Examination demonstrated left sixth nerve palsy with 3 retinal hemorrhages noted in one eye. Gadolinium-enhanced high-resolution skull base MRI revealed left sixth nerve enhancement involving the cisternal segment. Complete blood count, cerebrospinal fluid analysis, bone marrow biopsy, and flow cytometry confirmed acute T-cell lymphoblastic leukemia with central nervous system involvement. This case demonstrates the value of high-resolution MRI in the evaluation of cranial nerve palsy in young adults and also emphasizes the importance of systemic work up in these cases, particularly when retinal findings are present.

Effect of Initial Prednisone Dosing on Ocular Myasthenia Gravis Control.

BACKGROUND: Ocular myasthenia is an autoimmune condition that results in double vision or ptosis. It often requires treatment with prednisone for immunosuppression, but there have been no prospective trials to help clinicians determine ideal dosing. METHODS: This was a retrospective study comparing myasthenia symptom control at 1 month between patients treated with a maximum daily equivalent dose of prednisone less than 20 mg (low-dose group) vs 20 mg or more (medium-dose group). RESULTS: Thirty-nine patients were identified: 19 patients in the low-dose group with mean maximum daily dose of 10 mg and 20 patients in the medium-dose group with a mean maximum daily dose of 29 mg. The low-dose group had 75% controlled or significantly improved at 1 month, and the medium-dose group had 74% controlled or significantly improved at 1 month, P = 0.94. The overall seropositivity rate was 64%, with 84% of the antibody-positive group being controlled or significantly improved at 1 month and 57% of the antibody-negative group being controlled or significantly improved at 1 month, P = 0.07, and no difference in prednisone dosing between the 2 groups. CONCLUSION: Based on the results of this small retrospective study, it seems initial treatment for ocular myasthenia gravis with a mean maximum daily prednisone dose of 10 mg is similarly effective compared with mean maximum daily dose of 29 mg for control at 1 month.

Use of En Face Optical Coherence Tomography to Monitor Papilledema in Idiopathic Intracranial Hypertension: A Pilot Study.

BACKGROUND: En face optical coherence tomography (OCT) uses the data acquired during OCT of the optic disc, which typically is used to determine measurements of the peripapillary retinal nerve fiber layer (ppRNFL), to generate a coronal composite fundus image rather than a cross-sectional image. En face OCT has been reported to identify retinal changes related to papilledema in idiopathic intracranial hypertension (IIH) but has not been evaluated for monitoring papilledema. This study aimed to assess the reliability and validity of en face OCT for monitoring papilledema. METHODS: Using the Pearson correlation coefficient (R), these measurements were compared with ppRNFL thickness as well as average diameter and estimated area. Four fellowship-trained neuro-ophthalmologists were asked to qualitatively rank en face images by the area of optic disc edema while masked from all other clinical data. Rankings were compared with ppRNFL thickness as a gold standard and with en face OCT characteristics using the Pearson correlation coefficient (R). RESULTS: Experts were able to correctly identify an increase in average ppRNFL thickness >10 µm with a mean (SD) of 91% (±7%) accuracy. A ranking error among experts corresponded to a mean (standard error) change in the ppRNFL thickness of 6 (±6) µm. The mean Pearson correlation coefficient by the area of disc edema among experts was 0.92 (±0.13). CONCLUSIONS: Multiple objective parameters of en face OCT of optic disc edema have an excellent correlation with ppRNFL thickness. These results suggest that en face OCT is a valid clinical tool for monitoring papilledema in IIH.

Characterization of Visual Pathway Abnormalities in Infants With Congenital Zika Syndrome Using Computed Tomography and Magnetic Resonance Imaging.

BACKGROUND: Severe visual impairment is present in nearly all infants with congenital Zika syndrome (CZS); however, ocular abnormalities are present only in a subset of these infants. The purpose of this study was to characterize the visual pathway abnormalities seen on computed tomography (CT) and MRI scans in infants with CZS. METHODS: Preliminary neuroimaging information was obtained from a referred sample of 105 infants with clinical and epidemiologic data consistent with CZS in the Pernambuco state of Brazil. Subjects were excluded if Zika virus infection was not confirmed by serologic or cerebrospinal fluid studies or if images were nondiagnostic. Of the 105 subjects initially screened, head CT images adequate for interpretation were available for 54, and brain MRI images adequate for interpretation were available for 20. Four patients had both CT and MRI images. Magnetic resonance imaging and CT scans from infants with CZS were systematically reviewed for globe malformations, optic nerve and chiasmal atrophy, occipital cortical volume loss, white matter abnormalities, ventriculomegaly, and calcifications. Neuroimaging findings were correlated with measures of visual function and with ocular examinations in these infants. RESULTS: Thirty-three males and 37 females were included in the analysis. The mean age of the infants at the time of neuroimaging was 16.0 weeks (range 0 days-15.5 months), and the mean gestational age at the time of birth was 38 weeks. All patients were from the Pernambuco state of Brazil. Overall, 70 of 74 (95%) scans showed occipital volume loss, whereas 9 (12%) showed optic nerve atrophy, 3 (4%) showed chiasmal atrophy, and 1 (1%) showed an ocular calcification. Sixty-two of the infants underwent ophthalmologic examinations. A total of 34 (55%) infants had at least one documented structural ocular abnormality, and 26 (42%) had at least one structural ocular abnormality documented in both eyes. Of those with available visual acuity data, all had visual impairment. Among those with visual impairment and normal eye examinations, 100% had visual pathway abnormalities on neuroimaging, including 100% with occipital cortical volume loss, 8% with optic nerve atrophy, and 8% with chiasmal atrophy. CONCLUSION: Our results suggest that cortical visual impairment related to structural abnormalities of the occipital cortex is likely an important cause of visual impairment in children with CZS with normal eye examinations.

Tarsal Epithelial Cysts: Prevalence, Case Series, and Synthesis of Existing Literature.

PURPOSE: Tarsal epithelial cysts (TECs) are squamous epithelial-lined lesions of the eyelid that are often mistaken for chalazia or epidermal inclusion cysts. They remain poorly described in the literature. This study is designed to characterize the prevalence and clinical features of TEC. METHODS: We conducted a single-center retrospective review of adult patients with a diagnosis of eyelid neoplasm, eyelid cyst, hordeolum, stye, or chalazion between January 1, 2011 and July 1, 2017. Among this cohort, we identified patients with a histopathologic diagnosis of TEC. We also conducted a PubMed literature review and synthesis of existing clinical data of patients reported to have TEC, noting common clinical and histopathological features. RESULTS: Of 7,516 patients, we identified 6 patients with a histopathological diagnosis of TEC, amounting to a prevalence of 0.08% amongst patients with eyelid lesions. Average age was 49.7 years (range 18-76 years), with a 1:1 male to female ratio. The most common presenting symptom was a painless eyelid mass, and the majority (66.6%) had a preoperative diagnosis of chalazion. All but 1 patient had surgical excision from the posterior approach and there was 1 recurrence in the follow-up period. On review of the literature, we identified 68 prior cases of TEC from 18 clinical studies, with clinical features mirroring our case series. CONCLUSIONS: TEC has stereotypical clinical and histologic features that distinguish it from other tarsal lesions. Our review identified TEC as a relatively rare cause of eyelid lesions.