Journal of Neuro-Ophthalmology




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One Eye or Two: Statistical Considerations in Ophthalmology With a Focus on Interventional Clinical Trials

Mollan, Susan P. FRCOphth; Homer, Victoria MsC; Gates, Simon PhD; Brock, Kristian PhD; Sinclair, Alex J. PhD

doi : 10.1097/WNO.0000000000001446

December 2021 - Volume 41 - Issue 4 - p 421-423

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Bedside Assessment of Vergence in Stroke Patients

Anagnostou, Evangelos MD, PhD; Koutsoudaki, Penelopi MD; Tountopoulou, Argyro MD, PhD; Spengos, Konstantinos MD, PhD; Vassilopoulou, Sophia MD, PhD

doi : 10.1097/WNO.0000000000001035

December 2021 - Volume 41 - Issue 4 - p 424-430

Given the widely distributed network of midbrain, pontine, cerebellar, and cortical areas involved in the neural control of vergence, one might expect various vergence deficits in stroke patients. In this article, we investigated the localizing value of bedside vergence testing with respect to different supratentorial and infratentorial infarction locations.

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Peripapillary Hyper-reflective Ovoid Mass-like Structure (PHOMS): An Optical Coherence Tomography Marker of Axoplasmic Stasis in the Optic Nerve Head

Fraser, J. Alexander MD; Sibony, Patrick A. MD; Petzold, Axel MD, PhD; Thaung, Caroline MB, ChB, DPhil; Hamann, Steffen MD, PhD;  for the ODDS Consortium

doi : 10.1097/WNO.0000000000001203

December 2021 - Volume 41 - Issue 4 - p 431-441

With the development and widespread adoption of spectral-domain optical coherence tomography (OCT), peripapillary hyper-reflective ovoid mass-like structures (PHOMS) have become a frequent OCT finding in neuro-ophthalmic practice. Although originally assumed to represent a form of buried optic disc drusen (ODD), PHOMS differ from ODD in many important ways. The histopathological underpinnings of PHOMS are now becoming more clearly understood.

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Effects of Parkinson Disease on Blur-Driven and Disparity-Driven Vergence Eye Movements

Gupta, Palak BS; Beylergil, Sinem PhD; Murray, Jordan PhD; Jacobs, Jonathan PhD; Kilbane, Camilla MD; Shaikh, Aasef G. MD, PhD; Ghasia, Fatema F. MD

doi : 10.1097/WNO.0000000000001422

December 2021 - Volume 41 - Issue 4 - p 442-451

Synchronous movements of the 2 eyes in the opposite direction, disconjugate movements such as vergence, facilitate depth perception. The vergence eye movements are affected in Parkinson disease (PD). Visual blur (accommodation) and fusion (retinal disparity) are important triggers for the vergence. The neural circuit responsible for blur-driven and disparity-driven vergence is tightly coupled. We investigated the effect of PD on these 2 vergence paradigms. In the experiment involving 14 patients with PD and 6 healthy controls, substantial differences between blur-driven and disparity-driven vergence were found. The gain (ratio of actual vs desired eye movements) was reduced in patients with PD in case of disparity-driven vergence but not in blur-driven vergence. The latency of disparity-driven vergence onset was significantly longer for patients with PD compared with healthy controls. Four strategies were used to drive disparity-driven vergence: a) pure disconjugate vergence, b) conjugate saccadic movements, c) disconjugate vergence followed by saccadic movements, and d) conjugate saccades followed by disconjugate vergence movements. Blur-driven vergence had only 2 strategies: a) conjugate saccades followed by disconjugate vergence and b) conjugate saccadic movements only. The results are consistent with the prediction that PD primarily affects disparity-driven vergence, but there are some effects on the strategies to execute blur-driven vergence. We speculate that the deep cerebellar nuclei and the supraoculomotor area of the midbrain that carry the disparity-driven and blur-driven vergence are affected in PD. It is possible to modulate their function through projections to the subthalamic nuclei.

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Neuroradiologic Imaging of Neurologic and Neuro-Ophthalmic Complications of Coronavirus-19 Infection

Marsiglia, Marcela MD, PhD; Chwalisz, Bart K. MD; Maher, Mary MD

doi : 10.1097/WNO.0000000000001454

December 2021 - Volume 41 - Issue 4 - p 452-460

To review the literature and provide a summary of COVID-19–related neurologic and neuro-ophthalmic complications.

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Teprotumumab in Clinical Practice: Recommendations and Considerations From the OPTIC Trial Investigators

Douglas, Raymond S. MD, PhD; Wang, Yao MD; Dailey, Roger A. MD; Harris, Gerald J. MD; Wester, Sara T. MD; Schiffman, Jade S. MD; Tang, Rosa A. MD; Fowler, Brian MD; Fleming, James MD; Smith, Terry J. MD

doi : 10.1097/WNO.0000000000001134

December 2021 - Volume 41 - Issue 4 - p 461-468

Thyroid eye disease (TED) is a vision-threatening and debilitating condition that until very recently had no Food and Drug Administration (FDA)-approved medical therapies. Teprotumumab has recently been approved to treat TED. We aim to provide guidance for its use, based on the input of the US investigators who participated in Phase 2 and Phase 3 clinical trials.

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Interocular Difference in Retinal Nerve Fiber Layer Thickness Predicts Optic Neuritis in Pediatric-Onset Multiple Sclerosis

Waldman, Amy T. MD, MSCE; Benson, Leslie MD; Sollee, John R. BS; Lavery, Amy M. PhD; Liu, Geraldine W. ALM; Green, Ari J. MD, MCR; Waubant, Emmanuelle MD; Heidary, Gena MD, PhD; Conger, Darrel CRA; Graves, Jennifer MD, PhD; Greenberg, Benjamin MD, MHS

doi : 10.1097/WNO.0000000000001070

December 2021 - Volume 41 - Issue 4 - p 469-475

Optical coherence tomography (OCT) is capable of quantifying retinal damage. Defining the extent of anterior visual pathway injury is important in multiple sclerosis (MS) as a way to document evidence of prior disease, including subclinical injury, and setting a baseline for patients early in the course of disease. Retinal nerve fiber layer (RNFL) thickness is typically classified as low if values fall outside of a predefined range for a healthy population. In adults, an interocular difference (IOD) in RNFL thickness greater than 5 ?m identified a history of unilateral optic neuritis (ON). Through our PERCEPTION (PEdiatric Research Collaboration ExPloring Tests in Ocular Neuroimmunology) study, we explored whether RNFL IOD informs on remote ON in a multicenter pediatric-onset MS (POMS) cohort.

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Variation in Evolving Optic Neuritis

Bouffard, Marc A. MD; Mallery, Robert M. MD; Liao, Yaping J. MD, PhD; Torun, Nurhan MD

doi : 10.1097/WNO.0000000000001310

December 2021 - Volume 41 - Issue 4 - p 476-479

The typical natural history of optic neuritis is subjected to important exceptions. Recognition of these exceptions has led to valuable insights regarding specific etiologies of optic neuritis. Exceptions to the natural history of recovering optic neuritis are well-defined (e.g., chronic relapsing inflammatory optic neuropathy), but exceptions to the natural history of evolving optic neuritis are less so.

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Referral Patterns of Central Retinal Artery Occlusion to an Academic Center Affiliated With a Stroke Center

Flowers, Alexis M. MD; Chan, Wesley MD, MSc; Meyer, Benjamin I. MD; Bruce, Beau B. MD, PhD; Newman, Nancy J. MD; Biousse, Valérie MD

doi : 10.1097/WNO.0000000000001409

December 2021 - Volume 41 - Issue 4 - p 480-487

Central retinal artery occlusion (CRAO) is a medical emergency, and patients who present acutely should be immediately referred to the nearest stroke center. We evaluated practice patterns for CRAO management at one academic center over the last decade.

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Utility of Ultrasound and Optical Coherence Tomography in Differentiating Between Papilledema and Pseudopapilledema in Children

Farazdaghi, Marybeth K. MD; Trimboli-Heidler, Carmelina CDOS; Liu, Grant T. MD; Garcia, Arielle COT; Ying, Gui-Shuang PhD; Avery, Robert A. DO, MSCE

doi : 10.1097/WNO.0000000000001248

December 2021 - Volume 41 - Issue 4 - p 488-495

Differentiating between papilledema and pseudopapilledema in children presenting with mild-to-moderate optic nerve head elevation is challenging. This study sought to determine which B-scan ultrasonography (BSUS) and optical coherence tomography (OCT) features, individually or in combination, are best able to differentiate between papilledema and pseudopapilledema in children.

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Variability Within Optic Nerve Optical Coherence Tomography Measurements Distinguishes Papilledema From Pseudopapilledema

Flowers, Alexis M. MD; Longmuir, Reid A. MD; Liu, Yuhan MS; Chen, Qingxia PhD; Donahue, Sean P. MD, PhD

doi : 10.1097/WNO.0000000000001137

December 2021 - Volume 41 - Issue 4 - p 496-503

To report a linear risk score obtained using clock-hour optical coherence tomography (OCT) data from papilledema and pseudopapilledema nerves that differentiates between the 2 diagnoses with high sensitivity and specificity.

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Good Visual Outcomes After Pituitary Tumor Surgery Are Associated With Increased Visual Cortex Functional Connectivity

Lang, Stefan T. MD; Ryu, Won Hyung A. MD, MSc, MTM; Starreveld, Yves P. MD, PhD; Costello, Fiona E. MD;  the PITNET Study Group

doi : 10.1097/WNO.0000000000001155

December 2021 - Volume 41 - Issue 4 - p 504-511

Patients presenting with visual impairment secondary to pituitary macroadenomas often experience variable recovery after surgery. Several factors may impact visual outcomes including the extent of neuroaxonal damage in the afferent visual pathway and cortical plasticity. Optical coherence tomography (OCT) measures of retinal structure and resting-state functional MRI (rsfMRI) can be used to evaluate the impact of neuroaxonal injury and cortical adaptive processes, respectively. The purpose of this study was to determine whether rsfMRI patterns of functional connectivity (FC) distinguish patients with good vs poor visual outcomes after surgical decompression of pituitary adenomas.

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Imaging Errors in Distinguishing Pituitary Adenomas From Other Sellar Lesions

Altshuler, David B. MD; Andrews, Chris A. PhD; Parmar, Hemant A. MD; Sullivan, Stephen E. MD; Trobe, Jonathan D. MD

doi : 10.1097/WNO.0000000000001164

December 2021 - Volume 41 - Issue 4 - p 512-518

Pituitary adenomas and nonadenomatous lesions in the sellar region may be difficult to distinguish by imaging yet that distinction is critical in guiding management. The nature of the diagnostic errors in this setting has not been well documented.

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Neuro-Ophthalmic Complications in Patients Treated With CTLA-4 and PD-1/PD-L1 Checkpoint Blockade

Sun, Michel M. MD, PhD; Seleme, Nicolas MD; Chen, John J. MD, PhD; Zekeridou, Anastasia MD, PhD; Sechi, Elia MD; Walsh, Ryan D. MD; Beebe, Johanna D. MD; Sabbagh, Osama MD; Mejico, Luis J. MD; Gratton, Sean MD; Skidd, Philip M. MD; Bellows, David A. MD; Falardeau, Julie MD; Fraser, Clare L. MD; Cappelen-Smith, Cecilia MBBS, PhD; Haines, Scott R. MD; Hassanzadeh, Bahareh MD; Seay, Meagan D. DO; Subramanian, Prem S. MD, PhD; Williams, Zoë MD; Gordon, Lynn K. MD, PhD

doi : 10.1097/WNO.0000000000001148

December 2021 - Volume 41 - Issue 4 - p 519-530

In recent years, CTLA-4 and PD-1/PD-L1 checkpoint inhibitors have proven to be effective and have become increasingly popular treatment options for metastatic melanoma and other cancers. These agents work by enhancing autologous antitumor immune responses. Immune-related ophthalmologic complications have been reported in association with checkpoint inhibitor use but remain incompletely characterized. This study seeks to investigate and further characterize the neuro-ophthalmic and ocular complications of immune checkpoint blockade treatment.

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Abnormal Vestibular–Ocular Reflexes in Children With Cortical Visual Impairment

Mansukhani, Sasha A. MBBS; Ho, Mai-Lan MD; Brodsky, Michael C. MD

doi : 10.1097/WNO.0000000000000999

December 2021 - Volume 41 - Issue 4 - p 531-536

To determine whether the vestibular–ocular reflexes (VORs) can be affected by central nervous system injury in children with cortical visual impairment (CVI).

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Failure to Obtain Urgent Arterial Imaging in Acute Third Nerve Palsies

Chung, Jennifer E. BA; Schroeder, Richard M. MD; Wilson, Bradley MA; Van Stavern, Gregory P. MD; Stunkel, Leanne MD

doi : 10.1097/WNO.0000000000001337

December 2021 - Volume 41 - Issue 4 - p 537-541

Isolated third nerve palsy may indicate an expanding posterior communicating artery aneurysm, thus necessitating urgent arterial imaging. This study aims to assess the rate and duration of delays in arterial imaging for new isolated third nerve palsies, identify potential causes of delay, and evaluate instances of delay-related patient harm.

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Progressive Functional and Neuroretinal Affectation in Patients With Multiple Sclerosis Treated With Fingolimod

Garcia-Martin, Elena PhD; Ruiz de Gopegui, Erika PhD; Satue, Maria; Gil-Arribas, Laura PhD; Jarauta, Laura MD; Ara, Jose R. PhD; Martin, Jesus PhD; Fernandez, Francisco J. PhD; Vilades, Elisa MD; Rodrigo, Maria J. PhD

doi : 10.1097/WNO.0000000000000991

December 2021 - Volume 41 - Issue 4 - p e415-e423

To evaluate the effect of fingolimod in visual function and neuroretinal structures in patients with multiple sclerosis (MS) for a period of 1 year.

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Alterations in the Retinal Vascular Network and Structure in MOG Antibody-Associated Disease: An Optical Coherence Tomography Angiography Study

Yu, Jian PhD; Huang, Yongheng PhD; Quan, Chao PhD; Zhou, Lei PhD; ZhangBao, Jingzi PhD; Wu, Kaicheng PhD; Zong, Yuan PhD; Zhou, Xujiao PhD; Wang, Min PhD

doi : 10.1097/WNO.0000000000001116

December 2021 - Volume 41 - Issue 4 - p e424-e432

To determine retinal vessel density in patients with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).

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Follow-up of Nonarteritic Anterior Ischemic Optic Neuropathy With Optical Coherence Tomography Angiography

Aghsaei Fard, Masoud MD, FICO; Ghahvechian, Hossein MD; Subramanian, Prem S. MD, PhD

doi : 10.1097/WNO.0000000000000997

December 2021 - Volume 41 - Issue 4 - p e433-e439

Peripapillary and macular microvasculature alterations after nonarteritic ischemic optic neuropathy (NAION) have been investigated in several studies. We aimed to explore the vascular changes from acute NAION (aNAION) to chronic NAION (cNAION).

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Optical Coherence Tomography Angiography Characteristics and Predictors of Visual Outcomes in Patients With Acute and Chronic Nonarteritic Anterior Ischemic Optic Neuropathy

Moon, Yeji MD; Song, Min Kyung MD; Shin, Joong Won MD; Lim, Hyun Taek MD, PhD

doi : 10.1097/WNO.0000000000001102

December 2021 - Volume 41 - Issue 4 - p e440-e450

To investigate the correlation between optical coherence tomography angiography (OCTA) characteristics and visual outcomes in patients with acute and chronic nonarteritic anterior ischemic optic neuropathy (NAION).

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Prevalence of Adrenal Insufficiency and Glucocorticoid Use in Pediatric Pseudotumor Cerebri Syndrome

Hoyos-Martinez, Alfonso MD; Horne, Vincent E. MD; Wood, Alexis C. PhD; Shah, Veeral MD, PhD

doi : 10.1097/WNO.0000000000001111

December 2021 - Volume 41 - Issue 4 - p e451-e457

The pathophysiology underlying pseudotumor cerebri syndrome (PTCS) is complex and not well understood. There are clear differences between PTCS in adults and pediatrics. Few and isolated case reports have suggested that adrenal function may be involved, yet no large cohort study has examined this relationship.

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Weight Loss in Idiopathic Intracranial Hypertension: A Retrospective Review of Outcomes in the Clinical Setting

Ang, Juan Lyn MD, MSc; Teo, Kai Z. MD; Fraser, Clare L. MBBS (Hons), MMed, FRANZCO

doi : 10.1097/WNO.0000000000001107

December 2021 - Volume 41 - Issue 4 - p e458-e463

Weight loss is important in the management of idiopathic intracranial hypertension (IIH); however, extremely caloric restrictive diets have been used in published studies, which may not be practical for all patients. We aim to review the weight loss outcomes of IIH patients and weight loss methods used by patients who achieve clinical remission (CR) in a standard clinical setting.

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Optic Nerve Angle in Idiopathic Intracranial Hypertension

Chen, Benson S. MBChB, FRACP; Asnafi, Solmaz MD; Lin, Mung Y. BS; Bruce, Beau B. MD, PhD; Lock, Jane H. MBBS, FRANZCO; Sharma, Rahul A. MD, MPH; Newman, Nancy J. MD; Biousse, Valérie MD; Saindane, Amit M. MD, MBA

doi : 10.1097/WNO.0000000000000986

December 2021 - Volume 41 - Issue 4 - p e464-e469

The tortuosity of the optic nerve can be quantified radiologically by measuring the angle of optic nerve deformation (the “optic nerve angle” [ONA]). In patients with idiopathic intracranial hypertension (IIH), lowering the intracranial pressure (ICP) to a normal range by lumbar puncture leads to straightening of the optic nerve and an increase in the measured sagittal ONA on MRI. It is uncertain whether there is any correlation between ONA and cerebrospinal fluid (CSF) opening pressure or visual function.

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Comparison of Peripapillary Vessel Density of Acute Nonarteritic Anterior Ischemic Optic Neuropathy and Other Optic Neuropathies With Disc Swelling Using Optical Coherence Tomography Angiography: A Pilot Study

Chuang, Jasmine C. MB, BS; Chan, Noel C. Y. FRCSEd(Ophth); Cheung, Carol Y. PhD; Lok, Jerry K. H. FCOphth(HK); Chan, Carmen K. M. MRCP, FRCSEd(Ophth)

doi : 10.1097/WNO.0000000000001106

December 2021 - Volume 41 - Issue 4 - p e470-e482

The purpose of this study is to quantitatively compare the peripapillary vessel density (PPVD), measured with optical coherence tomography angiography (OCT-A), between acute nonarteritic anterior ischemic optic neuropathy (NAION) and other causes of disc swelling (“others”).

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Enhanced Depth Imaging Optical Coherence Tomography Technology Reveals a Significant Association Between Optic Nerve Drusen Anterior Displacement and Retinal Nerve Fiber Layer Thinning Over Time

Ortiz-Toquero, Sara PhD; Muñoz-Negrete, Francisco J. MD, PhD; Rebolleda, Gema MD, PhD

doi : 10.1097/WNO.0000000000001103

December 2021 - Volume 41 - Issue 4 - p e483-e489

Optic disc drusen (ODD) are a dynamic phenomenon, and their appearance, size, and relative location may change. The purpose of this study is to evaluate and quantify the longitudinal changes of buried ODD with enhanced depth imaging (EDI) optical coherence tomography (OCT) over time.

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Prediction of Postoperative Risk of Raised Intracranial Pressure After Spontaneous Skull Base Cerebrospinal Fluid Leak Repair

Buchowicz, Bryce MD; Chen, Benson S. MBChB, FRACP; Bidot, Samuel MD; Bruce, Beau B. MD, PHD; Newman, Nancy J. MD; Saindane, Amit M. MD, MBA; Levy, Joshua M. MD, MPH; Biousse, Valérie MD;  for the CSF-Leak Study Group

doi : 10.1097/WNO.0000000000001118

December 2021 - Volume 41 - Issue 4 - p e490-e497

A relationship between idiopathic intracranial hypertension and spontaneous skull base cerebrospinal fluid (CSF) leaks has been proposed, by which CSF leak decreases intracranial pressure (ICP) and masks the symptoms and signs of elevated ICP. These patients are at risk of developing papilledema, symptoms of elevated ICP, or a recurrent CSF leak after CSF leak repair. The objective of this study was to assess whether radiographic signs of raised ICP on preoperative magnetic resonance or computed venography (MRI or CTV) are predictors of postoperative papilledema, recurrence of CSF leak, or need for CSF shunt surgery.

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Papilledema Secondary to Neurologic Lyme Borreliosis: A Meta-Case Series

Vaysbrot, Elizaveta E. MD, MS; Bannuru, Raveendhara R. MD, PhD; Christopher, Mia-Cara MPH; Osani, Mikala C. BA; Halperin, John J. MD

doi : 10.1097/WNO.0000000000000983

December 2021 - Volume 41 - Issue 4 - p e498-e508

Papilledema can be a manifestation of neurologic Lyme borreliosis (LB). The clinical manifestations and progression of these cases have not been comprehensively documented to date. We aimed to describe clinical and diagnostic features and to assess patient outcomes in cases of papilledema secondary to neurologic LB.

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Utility of Spectral-Domain Optical Coherence Tomography in Differentiating Papilledema From Pseudopapilledema: A Prospective Longitudinal Study

Jivraj, Imran BSc, MD, FRCSC; Cruz, Cesar A. MD; Pistilli, Maxwell MEd, MS; Kohli, Anita A. MD; Liu, Grant T. MD; Shindler, Kenneth S. MD, PhD; Avery, Robert A. DO, MSCE; Garvin, Mona K. MS, PhD; Wang, Jui-Kai MS, PhD; Ross, Ahmara MD; Tamhankar, Madhura A. MD

doi : 10.1097/WNO.0000000000001087

December 2021 - Volume 41 - Issue 4 - p e509-e515

Prospective and longitudinal studies assessing the utility of spectral-domain optical coherence tomography (SD-OCT) to differentiate papilledema from pseudopapilledema are lacking. We studied the sensitivity and specificity of baseline and longitudinal changes in SD-OCT parameters with 3D segmentation software to distinguish between papilledema and pseudopapilledema in a cohort of patients referred for evaluation of undiagnosed optic disc elevation.

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The Value of Macular Optical Coherence Tomography in Watchful Waiting of Suprasellar Masses: A 2-Year Observational Study

Jørstad, Øystein Kalsnes MD; Wigers, Andreas Reidar MD; Marthinsen, Pål Bache MD; Evang, Johan Arild MD, PhD; Moe, Morten Carstens MD, PhD

doi : 10.1097/WNO.0000000000000993

December 2021 - Volume 41 - Issue 4 - p e516-e522

A possible benefit of optical coherence tomography (OCT) in the approach to tumors involving the optic chiasm may be the ability to foresee visual deterioration. This study investigated the value of OCT in watchful waiting for compressive optic neuropathy as the primary management of suprasellar masses.

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Unilateral Isolated Paucisymptomatic Optic Disc Edema

Hawy, Eman MD; Sharma, Rahul A. MD, MPH; Peragallo, Jason H. MD; Dattilo, Michael MD, PhD; Newman, Nancy J. MD; Biousse, Valérie MD

doi : 10.1097/WNO.0000000000001136

December 2021 - Volume 41 - Issue 4 - p e523-e534

Unilateral isolated optic disc edema (UIODE) represents a challenging clinical presentation that frequently precipitates an extensive diagnostic work-up. Patients without an apparent diagnosis despite appropriate investigations are often categorized as having “papillophlebitis,” an entity that is poorly defined in the existing literature. Our aim was to describe the characteristics of a series of patients with paucisymptomatic UIODE, determine the optimal diagnostic approach to such cases, and clarify the clinical features of presumed papillophlebitis.

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Neuro-Ophthalmologic Monitoring in the Management of Increased Intracranial Pressure From Leaking Arachnoid Cysts

Deveney, Tatiana K. MD; Lebas, Maud MD; Lobo, Remy R. MD; Maher, Cormac O. MD; Trobe, Jonathan D. MD

doi : 10.1097/WNO.0000000000001143

December 2021 - Volume 41 - Issue 4 - p e535-e540

Intracranial arachnoid cysts are common incidental imaging findings. They may rarely rupture, leading to the development of subdural hygromas and high intracranial pressure (ICP). Neurosurgical intervention has been advocated in the past, but recent evidence indicates that most cases resolve spontaneously. The role of neuro-ophthalmologic monitoring in identifying the few cases that have persisting vision-threatening papilledema that justifies intervention has not been emphasized.

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The Radiologic Characteristics and Retinal Thickness Are Correlated With Visual Field Defect in Patients With a Pituitary Mass

Suh, Hiebum MD; Choi, Heeyoung MD, PhD; Jeon, Hyeshin MD

doi : 10.1097/WNO.0000000000001011

December 2021 - Volume 41 - Issue 4 - p e541-e547

Investigation of visual field defects (VFDs) is important to decide the treatment and to predict the prognosis in patients with a pituitary mass. The aim of this study was to evaluate the correlation among 2 diagnostic modalities—MRI and optical coherence tomography (OCT)—and VFDs.

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Visual Outcomes in Surgically Treated Intracranial Meningiomas

Zhang, Yibing BA; Kim, John MD; Andrews, Chris PhD; Archer, Erica MD; Bursztyn, Lulu MD; Grabe, Hilary MD; Margolin, Edward MD; Sullivan, Stephen MD; Trobe, Jonathan MD

doi : 10.1097/WNO.0000000000001205

December 2021 - Volume 41 - Issue 4 - p e548-e559

Intracranial meningiomas that arise from the medial sphenoid ridge, anterior clinoid process, tuberculum sellae, or planum sphenoidale often impair vision by compressing the optic nerves and optic chiasm. Although many studies have reported visual outcome following surgery for these tumors, documentation has often been incomplete and not validated by patient self-report.

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Optic Disc Cupping Due to Dolichoectatic Internal Carotid Artery Optic Nerve Compression

Micieli, Jonathan A. MD; Margolin, Edward A. MD

doi : 10.1097/WNO.0000000000001113

December 2021 - Volume 41 - Issue 4 - p e560-e565

To determine whether significant compression of the optic nerve by the internal carotid artery (ICA) can produce an optic neuropathy with optic disc cupping that resembles glaucoma in patients without elevated intraocular pressure (IOP).

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Unilateral Blindness With Bilateral Brain Infarction After Cosmetic Facial Filler Injection

Lee, Jong Suk MD; Kim, Jun Yup MD; Woo, Se Joon MD, PhD

doi : 10.1097/WNO.0000000000001009

December 2021 - Volume 41 - Issue 4 - p e566-e571

To describe a case of bilateral brain infarction associated with iatrogenic ophthalmic artery occlusion after cosmetic facial filler injection and to elucidate the pathogenic mechanism of bilateral brain infarction.

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The Frequency of Carotid Intraplaque Hemorrhage on Vessel Wall Imaging in Patients With Retinal Artery Occlusion: A Cross-Sectional Prevalence Study

Larson, Anthony S. BS; Chen, John J. MD, PhD; Bhatti, M. Tariq MD; Klaas, James P. MD; Lanzino, Giuseppe MD; Huston, John III MD; Brinjikji, Waleed MD

doi : 10.1097/WNO.0000000000001156

December 2021 - Volume 41 - Issue 4 - p e572-e577

Intraplaque hemorrhage (IPH) in a carotid artery plaque viewed on vessel wall imaging has been shown to be associated with ischemic stroke. Whether such an association between carotid IPH and retinal artery occlusion (RAO) exists remains unknown.

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Complete Visual Recovery From Severe Outer Retinitis After Tonsillitis

Kawashima, Rumi MD, PhD; Matsushita, Kenji MD, PhD; Hashida, Noriyasu MD, PhD; Kuniyoshi, Kazuki MD, PhD; Fujikado, Takashi MD, PhD; Nishida, Kohji MD, PhD

doi : 10.1097/WNO.0000000000001073

December 2021 - Volume 41 - Issue 4 - p e578-e583

To report a case of severe acute bilateral outer retinitis after tonsillitis and rapid morphologic and functional recovery after steroid treatment.

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Neuro-Ophthalmic Manifestations of Acute Leukemia

Alrobaian, Malek A. MD; Henderson, Amanda D. MD

doi : 10.1097/WNO.0000000000001071

December 2021 - Volume 41 - Issue 4 - p e584-e590

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.

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Neuro-Ophthalmic Manifestations of Sarcoidosis

Henderson, Amanda D. MD; Tian, Jing MS; Carey, Andrew R. MD

doi : 10.1097/WNO.0000000000001108

December 2021 - Volume 41 - Issue 4 - p e591-e597

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.

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

Henderson, Amanda D. MD; Ventura, Camila V. MD, PhD; Huisman, Thierry A. G. M. MD; Meoded, Avner MD; Hazin, Adriano N. MD; van der Linden, Vanessa MD; de Lima Petribu, Natacha C. MD; May, William N. MD

doi : 10.1097/WNO.0000000000001127

December 2021 - Volume 41 - Issue 4 - p e598-e605

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.

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Introducing the 24-2C Visual Field Test in Neuro-Ophthalmology

Yamane, Maya L. M. MD; Odel, Jeffrey G. MD

doi : 10.1097/WNO.0000000000001157

December 2021 - Volume 41 - Issue 4 - p e606-e611

The Humphrey 24-2C visual field test is a modified 24-2 visual field test that incorporates 10 additional test points in the central 10° of vision. This study compares the new 24-2C test to the standard Humphrey 10-2 visual field test in patients presenting for neuro-ophthalmology evaluation to evaluate its ability to detect central visual field defects.

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Cyclotorsion Measurement on Scanning Laser Ophthalmoscopy Imaging Compared With Fundus Photography in Patients With Fourth Nerve Palsy and Healthy Controls: CySLO-IV Study

Kanku, Madeleine S. MD; Sele, Silvano MSc; Held, Ulrike PhD; Jaggi, Gregor P. MD; Landau, Klara MD; Traber, Ghislaine L. MD

doi : 10.1097/WNO.0000000000000996

December 2021 - Volume 41 - Issue 4 - p e612-e618

The purpose of the study is to evaluate the agreement of the foveopapillary angle (FPA) on conventional fundus photography (c-FPA) with the FPA on scanning laser ophthalmoscopy (SLO) imaging (SLO-FPA) in patients with fourth nerve palsy and healthy controls (HCs).

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Comitant Ocular Deviation in Myasthenia Gravis

Pike-Lee, Tiffany MD; Hill, Jeremy MD; Li, Jianbo PhD; Kosmorsky, Gregory S. DO; Li, Yuebing MD, PhD

doi : 10.1097/WNO.0000000000001056

December 2021 - Volume 41 - Issue 4 - p e619-e621

Occurrence of comitant ocular deviation in myasthenia gravis (MG) is not well described.

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Effect of Initial Prednisone Dosing on Ocular Myasthenia Gravis Control

Shah, Yesha S. BBA, BSA; Henderson, Amanda D. MD; Carey, Andrew R. MD

doi : 10.1097/WNO.0000000000001058

December 2021 - Volume 41 - Issue 4 - p e622-e626

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.

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The Usefulness of Anti-acetylcholine Receptor Binding Antibody Testing in Diagnosing Ocular Myasthenia Gravis

Chung, In Young MBBS Hons, BBMED; Sheth, Shivanand J. MBBS, MS (Ophthal), FICO, FAICO, FRANZCO; Wells, Kristen K. MD, FRANZCO; Campbell, Thomas G. MBBS, DPhil

doi : 10.1097/WNO.0000000000001061

December 2021 - Volume 41 - Issue 4 - p e627-e630

Anti-acetylcholine receptor antibody (AChR–Abs) testing is a safe and simple ancillary method for confirming the diagnosis of myasthenia gravis. Despite the test's high sensitivity (85%–90%) for generalized myasthenia gravis, AChR–Abs testing has been reported to have a low sensitivity 44%–66% for ocular myasthenia gravis (OMG). The aim of the study is to assess the effectiveness of AChR binding Abs testing for diagnosing OMG by evaluating the test's sensitivity, specificity, positive predictive value, and negative predictive value.

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Orbital Apex Syndrome Secondary to Invasive Aspergillus Infection: A Case Series and Literature Review

Yuan, Melissa BA; Tandon, Anika MD; Li, Anfei PhD; Johnson, Editha DO; Greer, Christine MD; Tooley, Andrea MD; Tran, Ann Q. MD; Godfrey, Kyle J. MD; Dinkin, Marc MD; Oliveira, Cristiano MD

doi : 10.1097/WNO.0000000000001105

December 2021 - Volume 41 - Issue 4 - p e631-e638

Invasive fungal sinusitis carries high morbidity and mortality and often poses a diagnostic challenge. Orbital apex syndrome (OAS) is not an uncommon presentation in the setting of invasive fungal sinusitis. Delays in diagnosis and appropriate treatment can result in permanent visual dysfunction and, potentially, death. We present 2 cases of OAS secondary to invasive sinus aspergillosis, detailing the diagnostic process, treatment, and outcome for both patients. Subsequently, we present a review of the literature and combined analysis of our 2 patients plus 71 cases from previously published reports.

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Delayed-Onset Cranial Nerve Palsy After Transvenous Embolization of Indirect Carotid Cavernous Fistulas

Wang, Arthur MD; Halbach, Van V. MD; Dowd, Christopher F. MD; Alexander, Matthew D. MD; Hallam, Danial K. MD; Ghodke, Basavarj MD; Moazami, Golnaz MD; Mandigo, Grace K. MD; Lavine, Sean D. MD; Meyers, Philip M. MD

doi : 10.1097/WNO.0000000000001067

December 2021 - Volume 41 - Issue 4 - p e639-e643

Carotid cavernous fistulas (CCF) often present with diplopia secondary to cranial nerve palsy (CNP). Immediate development of postoperative CNP has been described in the literature. This study described delayed-onset of CNP after complete and reconfirmed obliteration of the CCF and resolution of initial CNP.

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Dorsal Midbrain Syndrome: Clinical and Imaging Features in 75 Cases

Yousif, Jonah E. BS; Liao, Eric MD; Trobe, Jonathan D. MD

doi : 10.1097/WNO.0000000000001052

December 2021 - Volume 41 - Issue 4 - p e644-e654

Dorsal midbrain syndrome (DMS) consists of a constellation of clinical features, including reduced upgaze, pupillary light-near dissociation, lid retraction, convergence retraction, and eye misalignment. This syndrome results mostly from intrinsic or extrinsic mesodiencephalic tumors or strokes, obstructive hydrocephalus, failure of cerebrospinal fluid shunting to correct obstructive hydrocephalus, and head trauma. Published reports that include imaging corroboration are based on relatively small cohorts and have not included comprehensive patient self-reports on the impact of these abnormalities on quality of life.

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Resolved External Ophthalmoplegia and Hearing Loss in Wernicke's Encephalopathy With Thiamine Replacement

Nyce, Maxwell Q. DO; Chisholm, Joshua S. DO; Szmanda, Julia A. DO; Boyce, A. Katharina AuD; Boczar, Caroline M. AuD; Kattah, Jorge C. MD

doi : 10.1097/WNO.0000000000001057

December 2021 - Volume 41 - Issue 4 - p e655-e660

Wernicke encephalopathy (WE) is classically described by a clinical triad consisting of confusion, ataxia, and ophthalmoplegia, but recent reports emphasize a history of malnutrition along with 2 elements of the WE triad (Caine's criteria) to enhance diagnostic sensitivity. The ophthalmoplegia, vestibular, and auditory expeditious improvement with intravenous thiamine usually confirms the diagnosis; serum levels generally provide additional diagnostic certainty.

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Eye Movements in Fragile X-Associated Tremor/Ataxia Syndrome

Hall, Deborah A. MD, PhD; Leehey, Maureen A. MD; Hagerman, Randi J. MD; Pelak, Victoria S. MD

doi : 10.1097/WNO.0000000000001082

December 2021 - Volume 41 - Issue 4 - p e661-e664

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder characterized by ataxia, tremor, and parkinsonism. Eye motility abnormalities on the clinical examination of FXTAS patients have not been formally studied.

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Ocular Motor and Vestibular Characteristics of Antiglutamic Acid Decarboxylase–Associated Neurologic Disorders

Wang, Yujie MD; Tourkevich, Roksolyana MD; Bosley, Justin; Gold, Daniel R. DO; Newsome, Scott D. DO

doi : 10.1097/WNO.0000000000001084

December 2021 - Volume 41 - Issue 4 - p e665-e671

Antiglutamic acid decarboxylase (GAD)-associated neurologic disorders are rare, with varied presentations, including stiff-person syndrome (SPS) and cerebellar ataxia (CA). Vestibular and ocular motor (VOM) dysfunction can be the main presentation in a subset of patients.

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HINTS Examination in Acute Vestibular Neuritis: Do Not Look Too Hard for the Skew

Green, Kemar E. DO; Gold, Daniel R. DO

doi : 10.1097/WNO.0000000000001013

December 2021 - Volume 41 - Issue 4 - p e672-e678

An ocular tilt reaction (OTR) is a triad of a skew deviation, head tilt, and ocular counter-roll that can be partial or complete. An OTR can occur anywhere along the utriculo-ocular motor pathways from the labyrinth to the interstitial nucleus of Cajal but is almost always central in origin. In acute vestibular neuritis (AVN), case reports have described patients with an OTR due to AVN, although it is unclear whether this examination finding is common or rare.

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Predictive Value of International Classification of Diseases Codes for Idiopathic Intracranial Hypertension in a University Health System

Khushzad, Fareshta BS; Kumar, Riya; Muminovic, Irma MD; Moss, Heather E. MD, PhD

doi : 10.1097/WNO.0000000000000992

December 2021 - Volume 41 - Issue 4 - p e679-e683

Misclassification bias is introduced into medical claims–based research because of reliance on diagnostic coding rather than full medical record review. We sought to characterize this bias for idiopathic intracranial hypertension (IIH) and evaluate strategies to reduce it.

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Antibody Development in Patients Treated Long-Term With OnabotulinumtoxinA for Benign Essential Blepharospasm and Hemifacial Spasm

Czyz, Craig N. DO; Burns, John A. MD; Bergstrom, Reece E. DO

doi : 10.1097/WNO.0000000000001171

December 2021 - Volume 41 - Issue 4 - p e684-e687

Report the development of onabotulinumtoxinA neutralizing antibodies in patients treated consecutively for 20 years or longer for benign essential blepharospasm (BEB), hemifacial spasm (HFS), and Meige Syndrome.

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Involuntary “Voluntary” Nystagmus

Rosenberg, Michael L. MD

doi : 10.1097/WNO.0000000000001196

December 2021 - Volume 41 - Issue 4 - p e688-e691

Voluntary nystagmus is a common phenomenon in the general population and is believed to be under voluntary control. Some patients have an eye movement disorder indistinguishable from voluntary nystagmus and yet have no control over these movements.

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Publication Rate of Abstracts Presented at the North American Neuro-Ophthalmology Society Annual Meeting From 2008 to 2017

Yuan, Po Hsiang (Shawn) BSc; MacLean, Luke J. MASc; Li, Erica A. MMASc; Yin, Shelly MD, CM; Micieli, Jonathan A. BSc

doi : 10.1097/WNO.0000000000001158

December 2021 - Volume 41 - Issue 4 - p e692-e698

Conference abstracts serve an important role in the timely dissemination of scientific and clinical advancements, but most fail to be published. The goal of this study was to investigate the publication rate and factors associated with publication of abstracts presented at the North American Neuro-Ophthalmology Society (NANOS) Annual Meeting over a 10-year period.

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Should All Patients With Nonarteritic Anterior Ischemic Optic Neuropathy Receive a Sleep Study?

Fraser, Clare L. MD; Hedges, Thomas R. MD; Lee, Andrew G. MD; Van Stavern, Gregory P. MD

doi : 10.1097/WNO.0000000000001144

December 2021 - Volume 41 - Issue 4 - p 542-546

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Multiple Cranial Neuropathies and Pachymeningitis in a Patient With a Pathogenic Nucleotide-Binding Oligomerization Domain 2 Polymorphism

Vegunta, Sravanthi MD; Bohnsack, John MD; Crum, Alison MD; Digre, Kathleen MD; Katz, Bradley MD, PhD; Seay, Meagan DO; Quigley, Edward MD, PhD; Kennedy, Sean MD; Mamalis, Nick MD; Warner, Judith MD

doi : 10.1097/WNO.0000000000001342

December 2021 - Volume 41 - Issue 4 - p 547-552

An 11-year-old boy presented with 2 weeks of intermittent headache, right orbital pain, and constant diplopia. Brain MRI showed dural thickening and enhancement of the right lateral cavernous sinus, right orbital apex, and tentorium. Initial cerebral spinal fluid analysis showed only mild pleocytosis, and serum diagnostics were unrevealing. The working diagnosis was Tolosa-Hunt syndrome. His pain and sixth nerve palsy resolved with corticosteroids. Five months after initial presentation, he developed new numbness of the right cheek, complete right ophthalmoplegia, and weakness and numbness of his right hand and leg, all of which were responsive to steroids. Fifteen months later, he returned to the emergency department with 2 weeks of left-sided headaches and acute diplopia. On examination, he had a left cranial nerve 6 palsy. Dural biopsy showed diffuse mononuclear inflammatory cell reaction consisting mostly of lymphocytes with no signs of granuloma formation, nor any epithelioid or giant cells. His clinical course was consistent with an autoinflammatory condition of unknown etiology. Genetic testing with an immunodeficiency panel showed a risk allele in NOD2 (nucleotide-binding oligomerization domain 2) c.3019dup (p.Leu1007Prof*2) that is associated with an increased risk for Crohn disease. His clinical condition had similarities to central nervous system sarcoidosis. Because of the similarities between our patient's clinical, imaging, and genetic findings and neurosarcoidosis, he was switched to a more targeted therapy—infliximab. His condition has since been stable for nearly 2 years. In conclusion, genetic testing should be considered in patients with suspected occult autoimmunity.

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Literature Commentary

Editor(s): Moster, Mark L. MD; Bhatti, M. Tariq MD

doi : 10.1097/WNO.0000000000001447

December 2021 - Volume 41 - Issue 4 - p 553-559

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Isolated Asymmetric Progressive Optic Neuropathy as a First Presentation of Charcot–Marie–Tooth Disease Type 2A

Gabel, Maya BS; Mitchell, Jamie MD; Pramanik, Bidyut MD; Geraci, Anthony MD; Harel, Asaff MD, MSc

doi : 10.1097/WNO.0000000000001100

December 2021 - Volume 41 - Issue 4 - p e699-e702

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Severe Vision Loss and Intracranial Hypertension Presenting as Delayed Sequelae of “Mild” Carbon Monoxide Poisoning

Garcia, James H. BS; Khanna, Sangeeta MD

doi : 10.1097/WNO.0000000000001104

December 2021 - Volume 41 - Issue 4 - p e703-e707

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Papilledema in a Patient With Intracranial Hypotension

Kwok, Jason M. MD; Mandell, Daniel M. MD, PhD; Margolin, Edward A. MD

doi : 10.1097/WNO.0000000000001112

December 2021 - Volume 41 - Issue 4 - p e708-e710

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Vitreopapillary Traction Masquerading as Papillitis and Papilledema

Fong, Joseph W. MD; Sallam, Ahmed B. MD; Chacko, Joseph G. MD

doi : 10.1097/WNO.0000000000001159

December 2021 - Volume 41 - Issue 4 - p e711-e712

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Optic Neuritis Associated With Tumor Necrosis Factor–Alpha Inhibitor Certolizumab

Chen, Ariel MD; Miller, Neil R. MD; Bosley, Thomas M. MD

doi : 10.1097/WNO.0000000000001150

December 2021 - Volume 41 - Issue 4 - p e713-e714

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Severe Leber Hereditary Optic Neuropathy Plus Disease in a Middle-Aged Man

Birnbaum, Faith A. MD; Gospe, Sidney M. III MD, PhD

doi : 10.1097/WNO.0000000000001151

December 2021 - Volume 41 - Issue 4 - p e715-e717

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Food Aversion Leading to Nutritional Optic Neuropathy in a Child With Severe Vitamin A Deficiency

Farrell, Maureen C. MS; Weiss, Stephanie J. DO; Goodrich, Clifford MD; Martinez Lehmann, Maria Patricia MD; Delarato, Nicole MD

doi : 10.1097/WNO.0000000000001142

December 2021 - Volume 41 - Issue 4 - p e718-e719

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Bilateral Optic Neuropathy Secondary to Toxicity From Tumor-Infiltrating Lymphocytes Therapy Combined With Checkpoint Blockade

Kabanovski, Anna BS; Jeeva-Patel, Trishal MD; Saibil, Sam MD, PhD; Margolin, Edward MD

doi : 10.1097/WNO.0000000000001153

December 2021 - Volume 41 - Issue 4 - p e720-e722

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BRAF V600E-Mutated Ganglioglioma of the Optic Pathway: A Case Report and Review of the Literature

Solomon, Alexander M. MD; Chia, Tao Ming T. MD; Juric-Sekhar, Gordana MD, PhD; Francis, Courtney E. MD

doi : 10.1097/WNO.0000000000001154

December 2021 - Volume 41 - Issue 4 - p e723-e727

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Reversal of Visual Loss From Skull Base Osteomyelitis in a Pediatric Patient

Ma, Kevin K. MD; Robson, Caroline D. MD; Gaier, Eric D. MD, PhD; Gise, Ryan MD

doi : 10.1097/WNO.0000000000001110

December 2021 - Volume 41 - Issue 4 - p e728-e730

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Anterior Ischemic Optic Neuropathy Secondary to Carotid Artery Dissection

Lains, Ines MD, PhD; Diaz, Jose D. MD; Gittinger, John W. Jr MD; Gaier, Eric D. MD, PhD

doi : 10.1097/WNO.0000000000001161

December 2021 - Volume 41 - Issue 4 - p e731-e733

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Neuro-Ophthalmic Presentations of Common Carotid Artery Occlusion: A Case Series

Zehden, Jason; Harish Bindiganavile, Shruthi MD; Bhat, Nita MD; Lee, Andrew G. MD

doi : 10.1097/WNO.0000000000001101

December 2021 - Volume 41 - Issue 4 - p e734-e737

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Third Nerve Palsy Due to Posterior Communicating Artery Aneurysm Rupture Into the Subdural Space

Jeeva-Patel, Trishal MD; Mandell, Daniel M. MD, PhD; Margolin, Edward MD

doi : 10.1097/WNO.0000000000001123

December 2021 - Volume 41 - Issue 4 - p e738-e740

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Slowly Progressive Ophthalmoplegia as a Presenting Symptom of Myotonic Dystrophy Type 1

Na, Seunghee MD; Lee, Eek-Sung MD, PhD; Kim, Young-Do MD, PhD; Lee, Tae-Kyeong MD, PhD

doi : 10.1097/WNO.0000000000001132

December 2021 - Volume 41 - Issue 4 - p e741-e742

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Delayed Onset of Mixed Cranial Neuropathies and Cluster Headache After Embolization of Indirect Carotid-Cavernous Fistula

George, Rebecca S. MD; Pollmann, André S. MD; Maxner, Charles E. MD, FRCPC

doi : 10.1097/WNO.0000000000001138

December 2021 - Volume 41 - Issue 4 - p e743-e745

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Dry Nonproductive Cough as the Presenting Symptom of Giant Cell Arteritis

Hubbard, Donald BS; Harish Bindiganavile, Shruthi MD; Divatia, Mukul MD; Bhat, Nita MD; Lee, Andrew G. MD

doi : 10.1097/WNO.0000000000001166

December 2021 - Volume 41 - Issue 4 - p e746-e748

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Bitemporal Hemianopia Secondary to Acute Zonal Occult Outer Retinopathy

Zaslavsky, Kirill MD; Eshtiaghi, Arshia BSc; Jeeva-Patel, Trishal MD; Christakis, Panos G. MD; Margolin, Edward MD

doi : 10.1097/WNO.0000000000001167

December 2021 - Volume 41 - Issue 4 - p e749-e751

A 58-year-old man noticed shadows in the temporal visual field of each eye and photopsias within these scotomas for the past year. Formal visual fields demonstrated bitemporal hemianopia. MRI of the sella was normal, but fundus autofluorescence (FAF) demonstrated an area of hyperautofluorescence in the nasal retina of each eye corresponding to the loss of retinal pigment epithelium and photoreceptors. A diagnosis of acute zonal occult outer retinopathy was made based on the presence of trizonal involvement on FAF. This case is a reminder that bitemporal hemianopia can be caused by a retinopathy involving nasal retina in each eye, and FAF is a very useful test when assessing patients with suspected outer retinal disorders.

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Sinonasal Chondrosarcoma Presenting With Isolated Severe Vision Loss

Nguyen, Michael T. B. MD; Farahvash, Armin BSc; Dickson, Brendan C. MD; Lee, John M. MD; Cusimano, Michael D. MD, PhD; Tsang, Derek S. MD, MSc; Micieli, Jonathan A. MD, CM

doi : 10.1097/WNO.0000000000001130

December 2021 - Volume 41 - Issue 4 - p e752-e755

A 24-year-old man presented with a 2-month history of progressive, painless vision loss in the right eye, with no history of headache, nasal congestion, rhinorrhea, or epistaxis. His visual acuity was counting fingers at 1 ft in the right eye and 20 of 20 in the left eye with a right relative afferent pupillary defect and mild temporal optic disc pallor. MRI of the brain and orbits showed a mass involving bilateral ethmoid and sphenoid sinuses and right nasal cavity. He underwent urgent extended endoscopic endonasal transsphenoidal approach for resection of the sinonasal skull base tumor and photon radiation therapy. Pathology revealed a well-differentiated cartilaginous neoplasm with focal areas of entrapped native bone, consistent with a chondrosarcoma WHO grade I/III. At 6-month follow-up after surgery, he had a visual acuity of 20/40 in the right eye and 20/20 in the left eye. Malignant tumors from the sinonasal area should be kept in the differential diagnosis for compressive optic neuropathies and may present with vision loss even in the absence of nasal or sinus symptoms.

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Characteristic Visual Field Defect From Lateral Geniculate Body Stroke

Naguib, Mina M. MD; Woodland, Matthew B. BS; Foroozan, Rod MD

doi : 10.1097/WNO.0000000000001226

December 2021 - Volume 41 - Issue 4 - p e756-e758

A 58-year-old man presented with a complaint of subjective visual field loss on the right side and hypertensive emergency. Examination revealed a right homonymous hemianopia. Computed tomography imaging revealed an acute stroke of the left lateral geniculate body. A few months later, automated perimetry revealed characteristic visual field changes associated with this lesion. In this report, the anatomy, pathophysiology, clinical findings, and previously reported etiologies of lateral geniculate body lesions are reviewed.

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Acute Complete Oculomotor Nerve Palsy in a Young Male Due to a Skull Base Myofibroma

Hoang, Tung Thanh MD, MOphth; Van Le, Thuy MD; Tran, Dat MD; Ho, Thuong Duc MD; Thi Le, Nhung Hong MD; Subramanian, Prem S. MD, PhD

doi : 10.1097/WNO.0000000000001177

December 2021 - Volume 41 - Issue 4 - p e759-e760

A 25-year-old male patient visited the ophthalmology clinic because of upper eye lid ptosis in the right eye, binocular double vision, and light sensitivity. He was diagnosed with a complete third nerve palsy caused by a skull base myofibroma, a rare clinical entity that has not been described before in oculomotor nerve palsy.

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Contained Rupture of a Posterior Communicating Artery Aneurysm in a Patient With a Third Nerve Palsy

Dhoot, Arjan S. BMSc, MD(C); Guest, William C. MD, PhD; Bharatha, Aditya MD; Micieli, Jonathan A. MD

doi : 10.1097/WNO.0000000000001224

December 2021 - Volume 41 - Issue 4 - p e761-e763

It is recommended that every patient with a new third nerve palsy undergo urgent neuroimaging (computed tomography angiography or magnetic resonance angiography) to exclude a posterior communicating artery aneurysm. Because of the novel coronavirus (COVID-19) pandemic, our institution noted a significant decline in the number of patients with aneurysmal subarachnoid hemorrhage presenting to the hospital. We report one such example of a patient who developed new-onset severe headache and vomiting and did not seek medical attention because of COVID-19. Two months later, she was noted to have ptosis during a routine follow-up and was found to have a complete, pupil-involving third nerve palsy. Computed tomography angiography was performed and revealed an irregular bilobed saccular aneurysm (7 × 9 × 5 mm) of the right posterior communicating (PComm) artery, but no acute hemorrhage was visible on CT. On MRI, immediately adjacent to the aneurysm, there was a small subacute hematoma in the right medial temporal lobe with surrounding vasogenic edema. This case had a fortunate and unique outcome as she had a contained hematoma adjacent to the ruptured PComm aneurysm and did not experience severe morbidity from the subarachnoid hemorrhage nor did she rebleed in the interval in which she did not seek care. This case highlights the importance of providing neuro-ophthalmic care even during a pandemic.

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Tonic Pupil Following COVID-19

Gopal, Madhumita MBBS, DO, DNB; Ambika, Selvakumar MBBS, DO, DNB; Padmalakshmi, Krishnakumar MBBS, DO, DNB

doi : 10.1097/WNO.0000000000001221

December 2021 - Volume 41 - Issue 4 - p e764-e766

A 37-year-old patient presented to our emergency department with sudden onset decreased vision with a history of being treated for COVID 19 3 weeks earlier. On examination, she was found to have a tonic right pupil, which was confirmed with a dilute pilocarpine test. As tonic pupils are known to be caused by neurotropic viruses and our current understanding of the SARS-CoV-2 is that it does affect the nervous system, we feel that the tonic pupil in our patient may be secondary to COVID 19.

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A Great Conversation With Dr. Shirley Wray

Digre, Kathleen B. MD; Calix, Rachel A. MD; Seay, Meagan D. DO

doi : 10.1097/WNO.0000000000001403

December 2021 - Volume 41 - Issue 4 - p e767-e773

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A Great Conversation With Dr. Valerie Purvin

Calix, Rachel A. MD; Seay, Meagan D. DO; Digre, Kathleen B. MD

doi : 10.1097/WNO.0000000000001397

December 2021 - Volume 41 - Issue 4 - p e774-e784

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International Headache Society September 8–12, 2021

Digre, Kathleen B. MD

doi : 10.1097/WNO.0000000000001459

December 2021 - Volume 41 - Issue 4 - p e785

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Myelin Oligodendrocyte Glycoprotein Antibody–Associated Optic Neuritis—A Review

Kaushik, Megha MBBS; Burdon, Michael A. MBBS

doi : 10.1097/WNO.0000000000001234

December 2021 - Volume 41 - Issue 4 - p e786-e795

Our understanding of demyelinating optic neuritis has substantially evolved over the past 2 decades. With advancements in serological testing, antibodies against myelin oligodendrocyte glycoprotein (MOG) have been recently discovered in a distinct subset of demyelinating neuroinflammatory disease. Although MOG-immunoglobulin G (IgG)–associated disorder (MOGAD) has previously been seen as a component of neuromyelitis optica spectrum disorder (NMOSD), evidence increasingly suggests that it should be distinguished as a separate condition. The distinction of MOGAD from aquaporin-4 IgG NMOSD is imperative as treatment plans need to be tailored to its unique disease course and prognosis. The purpose of this review is to explore the nature and outcomes of MOGAD optic neuritis to help guide acute and long-term immunosuppressive treatment decisions.

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Leukemic and Lymphomatous Optic Neuropathy: A Case Series

Lee, Victoria PhD; Farooq, Asim V. MD; Shah, Hassan A. MD

doi : 10.1097/WNO.0000000000001365

December 2021 - Volume 41 - Issue 4 - p e796-e802

Optic neuropathy in the context of leukemia and lymphoma raises concern for central nervous system involvement or relapse and warrants prompt evaluation and treatment. To date, a gold standard for the diagnosis and management of leukemic optic neuropathy has yet to be established.

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Contralateral Ocular Manifestations of a Carotid Cavernous Fistula Associated With Primitive Persistent Trigeminal Artery

Hoang, Tung Thanh MD; Nguyen, Cuong Ngoc MD; Nguyen, Anh Quoc MD; Nguyen, Hieu Lan MD, PhD; Subramanian, Prem S. MD, PhD; Pham, Van Trong MD, PhD

doi : 10.1097/WNO.0000000000001181

December 2021 - Volume 41 - Issue 4 - p e803-e805

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Surgical Outcomes of Transposition Surgery for the Correction of Large-Angle Strabismus

Gokoffski, Kimberly Kinga MD, PhD; Lifton, Jacob MD; Xu, Benjamin Yixing MD, PhD; Patel, Vivek Ravindra MD

doi : 10.1097/WNO.0000000000001372

December 2021 - Volume 41 - Issue 4 - p e806-e814

Many potential surgical options exist to address large-angle deviations and head turns that result from various forms of paralytic strabismus. Muscle transposition surgeries serve as suitable alternatives to simple resection–recessions. Here, we report outcomes of augmented Hummelsheim and X-type transpositions for the correction of large-angle strabismus and provide insights for surgical planning.

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Subacute Bilateral Vision Loss

Bekerman, Vladislav P.; Turbin, Roger; Frohman, Larry P.

doi : 10.1097/WNO.0000000000001270

December 2021 - Volume 41 - Issue 4 - p e815

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Papilledema in a Patient Being Treated for Multiple Sclerosis

Frohman, Larry MD; Turbin, Roger E. MD; Wawrzusin, Peter J. MD; Srivastava, Gaurav MD; Sakla, Nicole M. DO

doi : 10.1097/WNO.0000000000001346

December 2021 - Volume 41 - Issue 4 - p e816

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Acute Posterior Multifocal Placoid Pigment Epitheliopathy Complicated by Fatal Cerebral Vasculitis

Knox, David L. MD

doi : 10.1097/WNO.0000000000000987

December 2021 - Volume 41 - Issue 4 - p e817-e819

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Acute Posterior Multifocal Placoid Pigment Epitheliopathy Complicated by Fatal Cerebral Vasculitis: Response

Harocopos, George MD; Van Stavern, Gregory MD; Dahiya, Sonika MD; Stunkel, Leanne MD; Maamari, Robi MD

doi : 10.1097/WNO.0000000000001019

December 2021 - Volume 41 - Issue 4 - p e819-e821

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Neuro-Ophthalmologic Monitoring of Leaking Arachnoid Cysts

Jarocki, Adrienne BA; Liao, Eric MD; De Lott, Lindsey B. MD

doi : 10.1097/WNO.0000000000001440

December 2021 - Volume 41 - Issue 4 - p e821-e823

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Comments on Disorders of the Fourth Cranial Nerve

Rutstein, Robert P. OD

doi : 10.1097/WNO.0000000000001441

December 2021 - Volume 41 - Issue 4 - p e823-e824

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Comments on Disorders of the Fourth Cranial Nerve: Response

Demer, Joseph L. MD; Kline, Lanning B. MD; Vaphiades, Michael S. MD; Tavakoli, Mehdi MD

doi : 10.1097/WNO.0000000000001442

December 2021 - Volume 41 - Issue 4 - p e824-e825

Buy The Package and View The Article Online


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