Acute Optic Neuropathy in Older Adults: Differentiating Between MOGAD Optic Neuritis and Nonarteritic Anterior Ischemic Optic Neuropathy.

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

Incidence of non-glaucomatous ocular disease in patients with asymmetric optic disc cupping.

PURPOSE: To evaluate the incidence of non-glaucomatous ocular disease in patients with asymmetric optic disc cupping. METHODS: A retrospective case series, including consecutive patients with cup-to-disc ratio (CDR) asymmetry greater than 0.2. All patients underwent a complete neuro-ophthalmological examination, automated perimetry with the Humphrey 24-2 visual fields program. Retinal nerve fibre layer thickness was measured by optical coherence tomography (OCT). The results of neuroimaging, macular OCT and blood tests were recorded as well. Patients were assigned a diagnosis of glaucomatous optic neuropathy (GON) or non-glaucomatous disease (NGD). The main outcome measure was the rate of non-glaucomatous ocular disease. RESULTS: A total of 120 (67 males) patients with a mean age of 71.1 ± 12.5 years met the inclusion criteria and were included in this study. The mean asymmetry in CDR between the eyes was 0.3 ± 0.13 (range, 0.2-0.9). Twenty patients (16.6%) had a visual field defect not typical for glaucoma and positive relative afferent pupillary defect was found in 24 patients (20%). Six patients were found to have newly diagnosed non-glaucomatous ocular disease: maculopathy in three patients, retinopathy in one patient and traumatic optic neuropathy in two patients. Patients with NGD were significantly younger than the patients with GON (59.8 ± 23.3 vs. 71.3 ± 11.5 years, P = 0.001). Optic disc pallor was found in 4/93 patients with glaucoma compared to 3/6 with newly diagnosed non-glaucomatous disease (4.7% vs. 50.0%, P = 0.03). CONCLUSIONS: Asymmetric optic disc cupping can be associated with non-glaucomatous disease and may warrant neuro-ophthalmological evaluation, especially in younger patients or those with optic disc pallor.

Clinical characteristics of progressive nonarteritic anterior ischemic optic neuropathy.

AIM: To study whether patients with progressive nonarteritic anterior ischemic optic neuropathy (NAION) present earlier than patients with stable NAION and to describe their clinical characteristics and visual outcome. METHODS: This was a retrospective chart review. All patients with NAION seen during the acute stage from January 2012 to December 2018 were reviewed. Patients were included if they had documented disc edema and follow up of at least 3mo. Patients with progressive NAION were identified if they worsened in 2 out of 3 parameters: visual acuity ≥3 Snellen lines; Color vision ≥4 Ishihara plates; the visual field defect involved a new quadrant. The clinical characteristics, time from symptom onset to presentation, systemic risk factors and visual outcome were compared to patients with stable NAION. RESULTS: Totally 122 NAION cases met the inclusion criteria. Mean age was 58.1y (range 22-74), 70% were men. Twenty cases (16.4%) had progressive NAION. Patients with progressive NAION did not differ from stable NAION in their demographics, systemic risk factors or in their initial visual deficit. At last follow up, median visual acuity was 1.0 logMAR (IQR 0.64-1.55) in patients with progressive NAION, vs 0.18 (IQR 0.1-0.63) in stable NAION (P<0.001). Median color vision testing was 0 plates correct (IQR 0-2.5%) vs 92% plates correct (IQR 50%-100%) in the stable NAION group (P<0.001). Patients with progressive NAION differed in the time from symptom onset to presentation (median 2d vs 5d, P=0.011). CONCLUSION: We find no identifiable risk factors associated with progressive NAION. Progressors arrive earlier for ophthalmological evaluation.

A Single Low-Dose of Methylphenidate Improves Abnormal Visual Field Testing.

PURPOSE: To evaluate the effect of methylphenidate on visual field testing in healthy adults with abnormal visual field results. METHODS: This prospective, randomized, controlled interventional clinical trial comprised all patients who had abnormal visual field test results and normal eye examination and ophthalmic history. Eligible patients were randomly assigned to either the study group or the control group. All patients repeated their visual field testing. Study group patients received a single dose of 10 mg methylphenidate prior to that. The main outcome measures were the percent difference in mean deviation and pattern standard deviation between the second and first visual fields. RESULTS: The methylphenidate group had greater improvement in all parameters. Mean deviation improved by median 68% (IQR 19%-78%) in the methylphenidate group vs. 27% [-5% to 55%] in the controls. However, this was not statistically significant (p = .83). Pattern standard deviation improved by median 49% (22%-59%) vs. 7% [-9% to 45%], respectively (p = .012). The visual fields were also reviewed by 3 masked experienced ophthalmologists. They indicated that the second visual field improved in 76.2% of the methylphenidate group vs. 48.5% of the controls (p = .04). A normal repeat visual field occurred in 57.7% vs. 21.2%, respectively. A subgroup analysis of patients with prior experience in visual field testing yielded an even more striking improvement in the methylphenidate group vs. controls. CONCLUSIONS: A single low dose of methylphenidate can improve visual field testing in subjects without ocular pathology, and even more in those with prior experience in perimetry.

[MALIGNANT OPTIC GLIOMA OF ADULTHOOD].

INTRODUCTION: A 61-year-old man presented with acute painless optic neuropathy with resultant no light perception in his left eye. Neuro-ophthalmological examination, optical coherence tomography and fluorescein angiography did not reveal the etiology. Since the patient had a cardiac pacemaker, he underwent a CT scan with contrast of the brain and orbits, which was normal. Five months later, the patient presented with visual field loss in his right eye. A repeat targeted CT scan was normal but after stopping his pacemaker, an MRI of the brain was obtained and revealed a space-occupying lesion involving the optic chiasm and both optic nerves. Lesion biopsy was consistent with glioblastoma multiforme. Despite treatment with radiotherapy and chemotherapy the patient died four months later. This case report emphasizes the importance of insisting on a high-quality brain MRI in the workup of optic neuropathy.

Accuracy of Scheimpflug Holladay equivalent keratometry readings after corneal refractive surgery in the absence of clinical history.

BACKGROUND AND OBJECTIVE: To identify the most accurate combination of Pentacam's equivalent keratometry readings (EKR) and intraocular lens power formula when the clinical history is unavailable. PATIENTS AND METHODS: A total of 18 patients underwent cataract surgery after refractive surgery. The Pentacam 4.5- and 3.0-mm EKR were combined with the SRK II, SRK/T, Hoffer-Q, and Holladay I and II formulas. RESULTS: The smallest deviation from the predicted value was achieved by combining the 4.5 EKR with the Holladay II formula (mean arithmetic deviation, -0.2 ± 0.4 dpt). CONCLUSION: The 4.5-mm EKR + Holladay II formula can accurately calculate intraocular lens power in patients with previous refractive surgery.

Orbital MRI appearance after remote retrobulbar alcohol injection.

An 88-year-old woman with a blind painful OS underwent a retrobulbar alcohol injection. Nine months following the procedure, she was evaluated in the clinic for discomfort surrounding her left brow and forehead and was found to have dysesthesia in the left V1 nerve distribution. There was no evidence of orbital inflammation on history or ocular examination. An MRI of the orbits showed intraconal abnormal signal with enhancement and enophthalmos. The chronic effect of retrobulbar alcohol injection on the orbital contents may mimic orbital inflammatory disease on the MRI, but the absence of clinical correlates should allow for appropriate diagnosis.

Meningoceles in idiopathic intracranial hypertension.

OBJECTIVE: MRI abnormalities have been described in patients with increased intracranial pressure (ICP), including in those with idiopathic intracranial hypertension (IIH). Spontaneous CSF-filled outpouchings of the dura (meningoceles) and secondary CSF leaks can occur from elevated ICP in patients with IIH; however, few studies have evaluated these findings. Our objective was to evaluate the frequency of spontaneous intracranial meningoceles among IIH patients and determine their association with visual outcome. MATERIALS AND METHODS: We performed a retrospective case-control study of consecutive IIH patients between 2000 and 2011 who underwent MRI that included T2-weighted imaging. Demographics, presenting symptoms, CSF opening pressure, and visual outcome were collected for the first and last evaluations. Control subjects included patients without headache or visual complaints who had normal brain MRI results. Stratified analysis was used to control for potential confounding by age, sex, race, and body mass index. RESULTS: We included 79 IIH patients and 76 control subjects. Meningoceles were found in 11% of IIH patients versus 0% of control subjects (p<0.003). Prominent Meckel caves without frank meningoceles were found in 9% of IIH patients versus 0% of control subjects (p<0.003). Among IIH patients, the presence of meningocele or prominent Meckel caves was not associated with demographics, symptoms, degree of papilledema, CSF opening pressure, visual acuity, or visual field defect severity. CONCLUSION: Meningoceles are significantly more common in IIH patients than in control subjects and can be considered an additional imaging sign for IIH. Meningoceles are not, however, associated with decreased CSF opening pressure or better visual outcome in IIH.

Hemifield Slide Phenomenon as a Result of Heteronymous Hemianopia.

Heteronymous hemianopic defects may lead to the hemifield slide phenomenon. We report one case of binasal and two of bitemporal visual field defects causing hemifield slide. Both patients with bitemporal visual field defects underwent strabismus surgery. One with adjustable sutures reported omission or duplication of a central strip of vision dependent on alignment during adjustment, but did not have long-term improvement of hemifield slide in two patients. Visual field improvement resolved hemifield in two patients, one of whom had strabismus surgery. Strabismus surgery to restore binocular visual function can be performed in hemifield slide patients. Patients with improving visual fields may have a better sensory prognosis.

Retinal NFL thinning on OCT correlates with visual field loss in pediatric craniopharyngioma.

OBJECTIVE: To investigate the use of peripapillary optical coherence tomography for monitoring optic neuropathy in pediatric craniopharyngioma. DESIGN: Retrospective, consecutive-cohort, single-centre chart analysis. PARTICIPANTS: Twenty children with craniopharyngioma treated at a pediatric medical centre from 1999 to 2011. METHODS: The medical files were reviewed for demographics and optic nerve function. Findings for visual acuity and visual fields were analyzed against repeated optical coherence tomography (OCT) measurements of peripapillary nerve fibre layer thickness (using either time-domain Stratus OCT or spectral-domain Cirrus OCT). RESULTS: Average age at diagnosis was 6.5 ± 3.88 years. The most common presenting symptom was headache; only 1 child complained of visual loss. Mean best corrected visual acuity (logMAR) was 0.036 ± 0.06 in the 17 healthy eyes and 1.05 ± 1.45 in the 23 eyes with optic neuropathy. Positive signs included relative afferent pupillary defect (8/20), visual acuity loss (7/20), temporal visual field loss (bilateral 4/15, unilateral 4/15), papilledema (3/20), and unilateral/bilateral optic disc pallor (14/20). RNFL thickness was significantly lower in eyes with optic neuropathy than in healthy eyes (65 ± 22 µm vs 86.2 ± 29 µm; p = 0.000) and correlated with visual acuity (r = -0.43 to -0.17, p = 0.0001) and presence or absence of a visual field defect (mean difference, 26.1 ± 5.8 µm, p = 0.003). Ten children showed no change in RNFL thickness over time (mean 18 ± 14.2 months). CONCLUSIONS: A thinner RNFL on ocular coherence tomography is correlated with poorer visual acuity and visual field loss. Ocular coherence tomography may serve as an objective method to quantify axonal loss caused by craniopharyngioma. Further investigation is needed to determine its use for evaluating progressive axonal loss over time.

Primary spontaneous cerebrospinal fluid leaks and idiopathic intracranial hypertension.

INTRODUCTION: Idiopathic intracranial hypertension (IIH) is increasingly recognized as a cause of spontaneous cerebrospinal fluid (CSF) leak in the otolarnygological and neurosurgical literature. The diagnosis of IIH in patients with spontaneous CSF leaks typically is made a few weeks after surgical repair of the leak when symptoms and signs of elevated intracranial pressure (ICP) appear. METHODS: Case reports and literature review. Two young obese women developed spontaneous CSF rhinorrhea related to an empty sella in one and a cribriform plate encephalocele in the other. Both patients underwent surgical repair of the CSF leak. A few weeks later, they developed chronic headaches and bilateral papilledema. Lumbar punctures showed elevated CSF opening pressures with normal CSF contents, with temporary improvement of headaches. A man with a 3-year history of untreated IIH developed spontaneous CSF rhinorrhea. He experienced improvement of his headaches and papilledema after a CSF shunting procedure, and the rhinorrhea resolved after endoscopic repair of the leak. RESULTS: These cases and the literature review confirm a definite association between IIH and spontaneous CSF leak based on: 1) similar demographics; 2) increased ICP in some patients with spontaneous CSF leak after leak repair; 3) higher rate of leak recurrence in patients with raised ICP; 4) patients with intracranial hypertension secondary to tumors may develop CSF leak, confirming that raised ICP from other causes than IIH can cause CSF leak. CONCLUSIONS: CSF leak occasionally may keep IIH patients symptom-free; however, classic symptoms and signs of intracranial hypertension may develop after a CSF leak is repaired, exposing these patients to a high risk of recurrence of the leak unless an ICP-lowering intervention is performed.

"Optic neuritis" that is neither.

A 28-year-old African American woman developed periocular pain worsening with eye movement and decreased vision in her left eye. A diagnosis of left optic neuropathy was made based on the findings of decreased vision in the left eye, a left relative afferent pupillary defect, and mild optic disc hyperemia in the left eye (figure 1), with normal retinae and maculae bilaterally. Automated perimetry showed a small central scotoma in the left eye. The pain with eye movements suggested an inflammatory mechanism (i.e., optic neuritis). Her neurologic examination was normal. MRI of the brain showed 2 small T2 hyperintense lesions in the right inferior frontal lobe and left periatrial white matter. The lesions did not enhance. Angiotensin converting enzyme level was slightly elevated. A chest CT was negative for pulmonary sarcoidosis. She did not receive steroids, and her visual function improved spontaneously over a few weeks. She had no neurologic symptoms and the diagnosis of clinically isolated syndrome was made. The patient declined treatment with an immunomodulatory agent.