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Neuro-Ophthalmology: Creating a Diverse, Equitable, and Inclusive Subspecialty Is the Responsibility of Everyone
Quiros, Peter A. MD; Gordon, Lynn K. MD, PhD
doi : 10.1097/WNO.0000000000001378
September 2021 - Volume 41 - Issue 3 - p 279-284
Pathology of the Brain and the Eye in Severe Acute Respiratory Syndrome Coronavirus-2–Infected Patients: A Review
Champion, Samantha N. MD; Williams, Imani M. MBS; Lage, Maria Martinez MD; Stagner, Anna M. MD
doi : 10.1097/WNO.0000000000001275
September 2021 - Volume 41 - Issue 3 - p 285-292
Patients with severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) may present or eventually develop central nervous system and ophthalmic signs and symptoms. Varying reports have emerged regarding isolation of viral RNA from these tissue sites, as well as largely autopsy-based histopathologic descriptions of the brain and the eye in patients with COVID-19.
Importance of the “Rule of the Pupil” in the Modern Neuroimaging Era
Garg, Anubhav BScH; Micieli, Jonathan A. MD
doi : 10.1097/WNO.0000000000001175
September 2021 - Volume 41 - Issue 3 - p 293-297
The “Rule of the Pupil” states that when aneurysms compress the oculomotor nerve, a dilated or sluggishly reactive pupil will result. In previous decades, when cerebral angiography was required to detect an intracranial aneurysm, the “Rule of the Pupil” was used to determine the relative risk of angiography and the likelihood of aneurysmal compression in patients with third nerve palsies (3NPs). Noninvasive imaging including computed tomography angiography (CTA) and magnetic resonance angiography has become readily accessible and can detect all aneurysms large enough to cause 3NPs. It is therefore recommended that all patients with 3NP undergo neuroimaging regardless of pupil status because the consequences of missing an aneurysm are high. The question therefore remains as to whether the “Rule of the Pupil” still has relevance in today's era of modern neuroimaging. We describe a 73-year-old man who developed a left complete, pupil-sparing 3NP and was found to have a paraclinoid meningioma in the left cavernous sinus. As compressive lesions are expected to impair the iris sphincter muscle, no intervention was recommended and his 3NP spontaneously improved within 3 months. We also describe a 54-year-old woman with diabetes and a complete 3NP with a dilated, nonreactive pupil. Initial CTA was reported as normal, but re-review of imaging revealed a posterior communicating artery aneurysm and immediate intervention to coil the aneurysm occurred. The “Rule of the Pupil” is still important in the modern neuroimaging era as demonstrated in cases of incidentally found lesions along the course of the oculomotor nerve and missed radiological findings.
Cross-Sectional Analysis of Baseline Visual Parameters in Subjects Recruited Into the RESCUE and REVERSE ND4-LHON Gene Therapy Studies
Moster, Mark L. MD; Sergott, Robert C. MD; Newman, Nancy J. MD; Yu-Wai-Man, Patrick MD, PhD; Carelli, Valerio MD, PhD; Bryan, Molly Scannell PhD; Smits, Gerard PhD; Biousse, Valérie MD; Vignal-Clermont, Catherine MD; Klopstock, Thomas MD; Sadun, Alfredo A. MD, PhD; DeBusk, Adam A. MD; Carbonelli, Michele MD; Hage, Rabih MD; Priglinger, Siegfried MD, PhD; Karanjia, Rustum MD, PhD; Blouin, Laure; Taiel, Magali MD; Katz, Barrett MD; Sahel, José Alain MD, PhD
doi : 10.1097/WNO.0000000000001316
September 2021 - Volume 41 - Issue 3 - p 298-308
This report presents a cross-sectional analysis of the baseline characteristics of subjects with Leber hereditary optic neuropathy enrolled in the gene therapy trials RESCUE and REVERSE, to illustrate the evolution of visual parameters over the first year after vision loss.
Long-Term Follow-Up After Unilateral Intravitreal Gene Therapy for Leber Hereditary Optic Neuropathy: The RESTORE Study
Biousse, Valérie MD; Newman, Nancy J. MD; Yu-Wai-Man, Patrick MD, PhD; Carelli, Valerio MD, PhD; Moster, Mark L. MD; Vignal-Clermont, Catherine MD; Klopstock, Thomas MD; Sadun, Alfredo A. MD, PhD; Sergott, Robert C. MD; Hage, Rabih MD; Esposti, Simona MD; La Morgia, Chiara MD, PhD; Priglinger, Claudia MD; Karanja, Rustum MD, PhD; Blouin, Laure MSc; Taiel, Magali MD; Sahel, José-Alain MD, PhD
doi : 10.1097/WNO.0000000000001367
September 2021 - Volume 41 - Issue 3 - p 309-315
RESCUE and REVERSE were 2 Phase 3 clinical trials that assessed the efficacy and safety of intravitreal gene therapy with lenadogene nolparvovec (rAAV2/2-ND4) for the treatment of Leber hereditary optic neuropathy (LHON). RESTORE is the long-term follow-up study of subjects treated in the RESCUE and REVERSE trials.
Leber's Hereditary Optic Neuropathy in Older Individuals Because of Increased Alcohol Consumption During the COVID-19 Pandemic
Zaslavsky, Kirill MD, PhD; Margolin, Edward A. MD
doi : 10.1097/WNO.0000000000001333
September 2021 - Volume 41 - Issue 3 - p 316-320
Leber's hereditary optic neuropathy (LHON) is a disorder affecting oxidative phosphorylation in mitochondria. A majority of affected patients are men of 15 to 35 years of age. Phenotypic penetrance of this condition is only 50% in man and 10% in women and increases if the cellular energy demands go up, with the most common risk factors being smoking and alcohol use.
Retinal and Optic Nerve Deformations Due to Orbital Versus Intracranial Venous Hypertension
Kupersmith, Mark J. MD; Sibony, Patrick A. MD
doi : 10.1097/WNO.0000000000001074
September 2021 - Volume 41 - Issue 3 - p 321-328
Abnormal forces around the optic nerve head (ONH) due to orbital diseases, intracranial hypertension (IH), glaucoma, and space travel, are associated with alterations of the ONH shape. Elevated cerebral and ophthalmic venous pressure can contribute to stress and strain on the ONH and peripapillary retina. We hypothesize that IH and elevated ophthalmic venous pressure without IH cause different ONH and retinal changes.
Relationship Between Retinal Layer Thickness and Disability Worsening in Relapsing-Remitting and Progressive Multiple Sclerosis
Cellerino, Maria MD; Priano, Luca MD; Bruschi, Nicol? MD; Boffa, Giacomo MD; Petracca, Maria MD; Novi, Giovanni MD; Lapucci, Caterina MD; Sbragia, Elvira MD; Uccelli, Antonio MD; Inglese, Matilde MD, PhD
doi : 10.1097/WNO.0000000000001165
September 2021 - Volume 41 - Issue 3 - p 329-334
Data regarding the predictive value of optical coherence tomography (OCT)-derived measures are lacking, especially in progressive multiple sclerosis (PMS). Accordingly, we aimed at investigating whether a single OCT assessment can predict a disability risk in both relapsing-remitting MS (RRMS) and PMS.
Factors Associated With Increased Emergency Department Utilization in Patients With Acute Optic Neuritis
Muro-Fuentes, Elena A. MS; Moss, Heather E. MD, PhD
doi : 10.1097/WNO.0000000000001294
September 2021 - Volume 41 - Issue 3 - p 335-341
Symptoms of acute vision loss and eye pain may lead patients with optic neuritis to seek care in the emergency department (ED). Given the availability of lower cost alternatives for providing medical care for optic neuritis, this study aimed to identify factors associated with higher ED utilization.
The Cost-Effectiveness of Tocilizumab (Actemra) Therapy in Giant Cell Arteritis
Jogimahanti, Arjun V. BS; Kini, Ashwini T. MD; Irwin, Lauren E. PhD; Lee, Andrew G. MD
doi : 10.1097/WNO.0000000000001220
September 2021 - Volume 41 - Issue 3 - p 342-350
Tocilizumab (Actemra) is a humanized anti–interleukin-6 receptor antibody that has been used as a steroid-sparing agent in giant cell arteritis (GCA). Although the clinical effects are well described in GCA, the cost-effectiveness of the use of tocilizumab in GCA is ill defined. The purpose of this study was to determine the cost-effectiveness of tocilizumab in GCA compared with prednisone alone.
A Case of Post-COVID-19–Associated Paracentral Acute Middle Maculopathy and Giant Cell Arteritis-Like Vasculitis
Jonathan, Go L. DO; Scott, Fair M. DO; Matthew, Kay D. MD
doi : 10.1097/WNO.0000000000001348
September 2021 - Volume 41 - Issue 3 - p 351-355
A 47-year-old man with a history of COVID-19 infection 2 months before presentation, presented with a scotoma of the paracentral visual field of the right eye. After thorough testing and evaluation, a diagnosis of paracentral acute middle maculopathy (PAMM) was established. Two months later, the patient developed temporal headache and jaw claudication. High-dose steroids were initiated, and workup for giant cell arteritis (GCA) was undertaken. The patient experienced resolution of the symptoms within 24 hours of steroid initiation. ESR, CRP, and temporal artery biopsy results were normal, although all were obtained more than 2 weeks after steroid initiation. To the best of our knowledge, our patient represents the first individual to date to potentially implicate COVID-19 in both small and large vessel vasculitis in the ophthalmic setting.
Telemedicine Evaluations in Neuro-Ophthalmology During the COVID-19 Pandemic: Patient and Physician Surveys
Conway, Jenna MD; Krieger, Penina MPhil; Hasanaj, Lisena BA; Sun, Linus MD, PhD; Scharf, Jackson M. BS; Odel, Jeffrey G. MD; Dinkin, Marc J. MD; Oliveira, Cristiano MD; Mackay, Devin D. MD; Rasool, Nailyn MD; Ko, Melissa MD; Rucker, Janet C. MD; Galetta, Steven L. MD; Balcer, Laura J. MD, MSCE
doi : 10.1097/WNO.0000000000001370
September 2021 - Volume 41 - Issue 3 - p 356-361
The novel coronavirus 2019 (COVID-19) pandemic has transformed health care. With the need to limit COVID-19 exposures, telemedicine has become an increasingly important format for clinical care. Compared with other fields, neuro-ophthalmology faces unique challenges, given its dependence on physical examination signs that are difficult to elicit outside the office setting. As such, it is imperative to understand both patient and provider experiences to continue to adapt the technology and tailor its application. The purpose of this study is to analyze both neuro-ophthalmology physician and patient satisfaction with virtual health visits during the time of the COVID-19 pandemic.
The Impact of COVID-19 on Neuro-Ophthalmology Office Visits and Adoption of Telemedicine Services
Moss, Heather E. MD, PhD; Ko, Melissa W. MD; Mackay, Devin D. MD; Chauhan, Divya MD; Gutierrez, Karen G. BS; Villegas, Natacha C. MD; Lai, Kevin E. MD
doi : 10.1097/WNO.0000000000001356
September 2021 - Volume 41 - Issue 3 - p 362-367
The COVID-19 public health emergency (PHE) has significantly changed medical practice in the United States, including an increase in the utilization of telemedicine. Here, we characterize change in neuro-ophthalmic care delivery during the early COVID-19 PHE, including a comparison of care delivered via telemedicine and in office.
Detection of Optic Disc Abnormalities in Color Fundus Photographs Using Deep Learning
Liu, T. Y. Alvin MD; Wei, Jinchi BSE; Zhu, Hongxi BS, MS; Subramanian, Prem S. MD, PhD; Myung, David MD, PhD; Yi, Paul H. MD; Hui, Ferdinand K. MD; Unberath, Mathias PhD; Ting, Daniel S. W. MD, PhD; Miller, Neil R. MD
doi : 10.1097/WNO.0000000000001358
September 2021 - Volume 41 - Issue 3 - p 368-374
To date, deep learning–based detection of optic disc abnormalities in color fundus photographs has mostly been limited to the field of glaucoma. However, many life-threatening systemic and neurological conditions can manifest as optic disc abnormalities. In this study, we aimed to extend the application of deep learning (DL) in optic disc analyses to detect a spectrum of nonglaucomatous optic neuropathies.
Lumbar Puncture for Diagnosis of Idiopathic Intracranial Hypertension in Typical Patients
Margolis, Mathew S. MD; DeBusk, Adam A. DO; Moster, Mark L. MD; Falardeau, Julie M. MD; Eggenberger, Eric R. DO; Sergott, Robert C. MD; Van Stavern, Gregory P. MD
doi : 10.1097/WNO.0000000000001319
September 2021 - Volume 41 - Issue 3 - p 375-378
Patients with typical features of pseudotumor cerebri syndrome (PTCS) must undergo lumbar puncture (LP) to demonstrate elevated opening pressure and cerebrospinal fluid (CSF) analysis to rule out alternative diagnoses. As LP may be associated with significant morbidity, this study aims to determine its necessity in diagnosing typical PTCS.
Should Lumbar Puncture Be Required to Diagnose Every Patient With Idiopathic Intracranial Hypertension?
Moss, Heather E. MD, PhD; Margolin, Edward A. MD; Lee, Andrew G. MD; Van Stavern, Gregory P. MD
doi : 10.1097/WNO.0000000000001373
September 2021 - Volume 41 - Issue 3 - p 379-384
Three “Red Lines” for Pattern Recognition-Based Differential Diagnosis Using Optical Coherence Tomography in Clinical Practice
Petzold, Axel MD, PhD, FRCP
doi : 10.1097/WNO.0000000000001173
September 2021 - Volume 41 - Issue 3 - p 385-398
Optical coherence tomography (OCT) devices for imaging of the eye are broadly available. The test is noninvasive, rapid, and well-tolerated by patients. This creates a large number of OCT images and patient referrals. Interpretation of OCT findings at the interface between neurological and ophthalmologic conditions has become a key skill in the neuro-ophthalmology service. Similar to the interpretation of visual fields, recogntion of the vertical and horizontal medians are helpful. A third “red line” is added, which will be reviewed here.
Double Vision and Gait Ataxia in an Immunocompetent 9-Year-Old Girl With Intracranial Phaeohyphomycosis
Pareek, Aishwarya V. MD; Lotze, Timothy E. MD; Demmler, Gail MD; Mohila, Carrie A. MD, PhD; Tran, Brandon MD; Shah, Veeral S. MD, PhD
doi : 10.1097/WNO.0000000000001204
September 2021 - Volume 41 - Issue 3 - p 399-403
A 9-year-old girl presented with morning headaches associated with vomiting, gait ataxia, and facial and ocular motor nerve palsies. Her initial imaging was concerning for demyelinating disease. After extensive infectious and rheumatologic workup returned negative, she was treated twice with intravenous immunoglobulin and intravenous steroids with near-complete resolution each time. She returned, however, with worsening neurologic deficits and imaging revealing focal ischemic infarction in the brainstem as well as new-onset hydrocephalus. A multispecialty workup was initiated without conclusive diagnosis. A novel, noninvasive test for plasma cell-free DNA established a diagnosis of Cladophialophora bantiana that was confirmed and validated by a brain biopsy taken during a clinical decompensation. Treatment was initiated with systemic voriconazole and intraventricular amphotericin B.
Thomas J. Carlow, MD, Founder of NANOS (1940–2021)
Selhorst, John B. MD
doi : 10.1097/WNO.0000000000001363
September 2021 - Volume 41 - Issue 3 - p 404-408
A Tribute to Jane Portnoy, MD
Liu, Grant T. MD; Shindler, Kenneth S. MD, PhD; Tamhankar, Madhura A. MD; Ross, Ahmara G. MD, PhD
doi : 10.1097/WNO.0000000000001380
September 2021 - Volume 41 - Issue 3 - p 409-410
Remembering Ivy Dreizen, MD (1948–2021)
Friedman, Deborah I. MD, MPH
doi : 10.1097/WNO.0000000000001382
September 2021 - Volume 41 - Issue 3 - p 411-412
Literature Commentary
doi : 10.1097/WNO.0000000000001381
September 2021 - Volume 41 - Issue 3 - p 413-419
Inability to Read After Prolonged COVID-19 Hospitalization: MRI With Clinical Correlation
Bondira, Inna P. DO; Lambert-Cheatham, Nathan A. DO; Sakuru, Ragha C. MD; Polinger-Hyman, David J. MD; Pipitone, Baldassare D. DO; Arnold, Kaitlyn E. BS; Nagia, Lina DO; Kaufman, David I. DO
doi : 10.1097/WNO.0000000000001121
September 2021 - Volume 41 - Issue 3 - p e277-e278
Acute Isolated Near Vision Difficulty in Patients With COVID-19 Infection
Umapathi, Thirugnanam MBBS, MRCP (UK); Li, Kelvin Zhenghao MBBS, FRCOphth; Chin, Chee Fang MBChB; Vijakumar, Kalpana MBBS, MRCP (UK); Tan, Glorijoy Shi En MBBS, MRCP (UK); Ung, Peck Houy MD; Yeo, Tun Kuan MBBS, FRCOphth; Agrawal, Rupesh MD, FRCS
doi : 10.1097/WNO.0000000000001120
September 2021 - Volume 41 - Issue 3 - p e279-e282
Perimetry Pitfalls in the Era of COVID-19
Mukharesh, Loulwah MD; Torun, Nurhan MD, FRCS(C); Bouffard, Marc A. MD
doi : 10.1097/WNO.0000000000001231
September 2021 - Volume 41 - Issue 3 - p e283-e285
Cranial Nerve III Palsy in the Setting of COVID-19 Infection
Fitzpatrick, John C. MD; Comstock, Jordan M. MD; Longmuir, Reid A. MD; Donahue, Sean P. MD, PhD; Fitzpatrick, John M. MD; Bond, John B. III MD
doi : 10.1097/WNO.0000000000001160
September 2021 - Volume 41 - Issue 3 - p e286-e287
Opsoclonus-Myoclonus-Ataxia Syndrome Related to the Novel Coronavirus (COVID-19)
Sanguinetti, Shayna Y. MD; Ramdhani, Ritesh A. MD
doi : 10.1097/WNO.0000000000001129
September 2021 - Volume 41 - Issue 3 - p e288-e289
Combined Optic Atrophy and Rod–Cone Dystrophy Expands the RTN4IP1 (Optic Atrophy 10) Phenotype
Rajabian, Firuzeh MD; Manitto, Maria Pia MD; Palombo, Flavia PhD; Caporali, Leonardo PhD; Grazioli, Alessio MD; Starace, Vincenzo MD; Arrigo, Alessandro MD; Cascavilla, Maria Lucia MD; La Morgia, Chiara MD, PhD; Barboni, Piero MD; Bandello, Francesco MD; Carelli, Valerio MD, PhD; Battaglia Parodi, Maurizio MD
doi : 10.1097/WNO.0000000000001124
September 2021 - Volume 41 - Issue 3 - p e290-e292
Ocular Manifestations of PNPT1-Related Neuropathy
Kuht, Helen J. BMedSci; Thomas, Kevin A. MBChB, MRCGP; Hisaund, Michael BMedSci; Maconachie, Gail D. E. BMedSci, PhD; Thomas, Mervyn G. MBChB, PhD
doi : 10.1097/WNO.0000000000001012
September 2021 - Volume 41 - Issue 3 - p e293-e296
Cryopyrin-Associated Periodic Syndrome in Neuro-Ophthalmology
Harish Bindiganavile, Shruthi MD; Beres, Shannon MD; Bhat, Nita MD; Lee, Andrew G. MD
doi : 10.1097/WNO.0000000000001080
September 2021 - Volume 41 - Issue 3 - p e297-e299
Bilateral Optic Neuritis After Vero Cell–Derived Antirabies Vaccination
Wang, Jue MD; Tian, Mingxia MD; Li, Fengjiao MD; Du, Xiangge MD; Han, Ying MD, PhD; Ma, Ping MD, PhD
doi : 10.1097/WNO.0000000000001077
September 2021 - Volume 41 - Issue 3 - p e300-e302
Juxtaposed Homonymous Hemianopsia Due to Neurotuberculosis
Wheelock-Gutierrez, Lorena MD; Harish Bindiganavile, Shruthi MD; Chévez-Barrios, Patricia MD; Fuller, Gregory N. MD, PhD; Bhat, Nita MD; Lee, Andrew G. MD
doi : 10.1097/WNO.0000000000001085
September 2021 - Volume 41 - Issue 3 - p e303-e306
Papilledema and Extensive Dural Sinus Thrombosis Due to JAK2 Mutation
Kisilevsky, Eli MD; Yu, Eugene MD; Margolin, Edward MD
doi : 10.1097/WNO.0000000000001096
September 2021 - Volume 41 - Issue 3 - p e307-e308
Ocular Ischemic Syndrome Secondary to Inflammatory Pseudotumor of the Skull Base Masquerading as Giant Cell Arteritis
Gostimir, Mi?o MD; Wehrli, Bret John MD, FRCPC; Fraser, J. Alexander MD, FRCPC
doi : 10.1097/WNO.0000000000001088
September 2021 - Volume 41 - Issue 3 - p e309-e311
Superior Ophthalmic Vein Thrombosis Secondary to Hyperhomocysteinemia
Schaffer, Lauren R. BA; Wells, Jill R. MD; Francis, Courtney E. MD
doi : 10.1097/WNO.0000000000001063
September 2021 - Volume 41 - Issue 3 - p e312-e313
Case of Bilateral Retinal Ischemia and Internal Carotid Artery Stenosis Associated With Graves' Disease
Kida, Teruyo MD; Oku, Hidehiro MD; Yagi, Ryokichi MD; Shigekiyo, Taro MD; Ikeda, Tsunehiko MD
doi : 10.1097/WNO.0000000000001028
September 2021 - Volume 41 - Issue 3 - p e314-e315
Novel Diagnosis of the Diffuse Variant of Infantile Orbital Fibromatosis
Matharu, Kanwal S. MD; Sweeney, Adam R. MD; Chévez-Barrios, Patricia MD; Edmond, Jane C. MD; Marx, Douglas P. MD; Allen, Richard C. MD, PhD; Shah, Veeral S. MD, PhD
doi : 10.1097/WNO.0000000000001055
September 2021 - Volume 41 - Issue 3 - p e316-e318
A Case of Recurrent Orbital Inflammation Secondary to Acute Myeloblastic Leukemia
Collett, Geoffrey MD; Haddad, Narmien BS; Peeler, Crandall E. MD; Distefano, Alberto G. MD
doi : 10.1097/WNO.0000000000001114
September 2021 - Volume 41 - Issue 3 - p e319-e321
Cavernous Sinus Syndrome After Barotraumatic Sneeze
Turfe, Zaahir MD; Saleh, Nancy BS; George, Christian MD; Rock, Jack MD; Craig, John R. MD
doi : 10.1097/WNO.0000000000001115
September 2021 - Volume 41 - Issue 3 - p e322-e323
Erdheim–Chester Disease Presenting With Diplopia: A Challenging Diagnosis With Effective Treatment
Tavakoli, Mehdi MD; Roghaee, Shiva MD; Gunturu, Manasa MD; Omuro, Antonio M. MD; Lam, Byron L. MD; Mendoza-Santiesteban, Carlos E. MD
doi : 10.1097/WNO.0000000000001076
September 2021 - Volume 41 - Issue 3 - p e324-e325
Pupil-Sparing Third Nerve Palsy and Papilledema Due to Granulomatosis With Polyangiitis
Vosoughi, Amir R. BSc; Streutker, Catherine J. MD, MSc; Yang, Stephanie P. MD, MSc; Lee, John M. MD, MSc; Micieli, Jonathan A. MD, CM
doi : 10.1097/WNO.0000000000001086
September 2021 - Volume 41 - Issue 3 - p e326-e330
Radiation-Induced Third Cranial Nerve Palsy With the Longest Interval Between Radiation Therapy and Onset of Cranial Neuropathy
Jeeva-Patel, Trishal MD; Mandell, Daniel MD, PhD; Le, Tran MD; Margolin, Edward MD, FRCSC
doi : 10.1097/WNO.0000000000001081
September 2021 - Volume 41 - Issue 3 - p e331-e332
Ptosis as Clinical Presentation in a Patient With Emery–Dreifuss Muscular Dystrophy Type 5
Douglas, Konstantinos A. A. MD, DVM; Douglas, Vivian Paraskevi MD, DVM; Gaier, Eric D. MD, PhD; Chwalisz, Bart K. MD
doi : 10.1097/WNO.0000000000001187
September 2021 - Volume 41 - Issue 3 - p e333-e334
Sixth Nerve Palsy and Myocarditis as the Only Presenting Manifestation of Giant Cell Arteritis
Margolin, Edward MD; Jeeva-Patel, Trishal MD
doi : 10.1097/WNO.0000000000001054
September 2021 - Volume 41 - Issue 3 - p e335-e336
Trigeminal-Abducens Pseudosynkinesis: Distinguishing Unconscious Motor Habits From True Synkinesis
Bourque, Pierre R. MD; Grimes, David A. MD; Lelli, Daniel A. MD
doi : 10.1097/WNO.0000000000001069
September 2021 - Volume 41 - Issue 3 - p e337-e338
Clinical and Imaging Manifestations of Trochlear Nerve Schwannomas—A Case Series
Gupta, Arvind MBBS, FRCS (Ophthalmology), FRCOphth; Lee, Emma C. H. MBBS; Kelly, Hillary R. MD; Chen, Robert C. MD
doi : 10.1097/WNO.0000000000001059
September 2021 - Volume 41 - Issue 3 - p e339-e342
A Case of Miller Fisher Syndrome Due to the Use of Cemiplimab
Ghosh, Arko BS; Harish Bindiganavile, Shruthi MD; Bhat, Nita MD; Lee, Andrew G. MD
doi : 10.1097/WNO.0000000000001062
September 2021 - Volume 41 - Issue 3 - p e343-e345
Miller Fisher Syndrome With Papilledema and Antecedent Helicobacter pylori Infection
Wawrzusin, Peter MD; Chung, Stella MD; Sakla, Nicole DO; Turbin, Roger MD; Frohman, Larry MD
doi : 10.1097/WNO.0000000000001079
September 2021 - Volume 41 - Issue 3 - p e346-e347
Abduction Variant One-and-a-Half Syndrome Due to a Massive Right Hemispheric Stroke With Uncal Herniation and Rapid Intracranial Hypertension
O'Marro, Maureen B. DO; Nazarian, Sarkis M. MD
doi : 10.1097/WNO.0000000000001075
September 2021 - Volume 41 - Issue 3 - p e348-e350
Postinfectious Optic Neuritis After Hand–Foot–Mouth Disease
Barrett, Spencer C. BS; Bhat, Nita N. MD; Bindiganavile, Shruthi H. MD; Lee, Andrew G. MD
doi : 10.1097/WNO.0000000000001163
September 2021 - Volume 41 - Issue 3 - p e351-e353
A 33-year-old man presented with acute painless loss of vision in his right eye after hand–foot–mouth disease (HFMD). Examination confirmed a right optic neuropathy. Neuroimaging and routine evaluations for alternative causes for an optic neuropathy were negative. He was treated with high dose corticosteroids and made an almost complete visual recovery. Postinfectious optic neuritis has been reported after a vast array of infections including: varicella zoster virus, influenza virus, herpes simplex virus, Epstein–Barr Virus, Lyme disease, and many others. Although Coxsackie virus infections are a known cause of HFMD and have been reported to cause maculopathy, to the best of our knowledge, this is the first reported case of optic neuritis after HFMD in the English language ophthalmic literature.
A Unique Radiologic Case of Optic Nerve Infarction in a Patient With Mucormycosis
Chaulk, Alexandria L. MD; Do, Thai H. MD; Supsupin, Emilio P. MD; Bhattacharjee, Meenakshi B. MD; Richani, Karina MD; Adesina, Ore-Ofe O. MD
doi : 10.1097/WNO.0000000000001179
September 2021 - Volume 41 - Issue 3 - p e354-e356
This is a rare presentation of a unilateral optic nerve infarction of the left eye caused by mucormycosis in a 51-year-old man with poorly controlled Type 2 diabetes. Diffusion-weighted MRI of the orbit demonstrated extensive infarction of the left optic nerve with ipsilateral cavernous sinus thrombosis and periorbital adnexal inflammation. Left orbital exenteration and sinus debridement were performed, and mucormycosis involving the optic nerve sheath was confirmed on histopathology.
Multimodal Imaging in Acute Macular Neuroretinopathy
Botsford, Benjamin W. MD; Kukkar, Priyanka MD; Bonhomme, Gabrielle MD
doi : 10.1097/WNO.0000000000001128
September 2021 - Volume 41 - Issue 3 - p e357-e359
A 44-year-old woman noticed bilateral irregular scotomata associated with photopsias of 6-month duration. Ophthalmoscopic examination demonstrated multiple bilateral, pericentral, hypopigmented, petaloid, macular lesions evident on Amsler grid associated with paracentral hyperreflective lesions on optical coherence tomography in the outer plexiform and nuclear layers. Indocyanine green angiography revealed patches of choroidal ischemia consistent with acute macular neuroretinopathy.
A Case of Transient Monocular Vision Loss and Paracentral Acute Middle Maculopathy
Betsch, Devin M. MD; Mishra, Amit V. MD; Freund, Paul R. MD, FRCSC
doi : 10.1097/WNO.0000000000001162
September 2021 - Volume 41 - Issue 3 - p e360-e362
A 77-year-old man with multiple cerebrovascular risk factors presented with a history of transient monocular vision loss and residual paracentral visual disturbance in the right eye. Carotid ultrasounds, erythrocyte sedimentation rate, and C-reactive protein were all within normal limits. He was found to have retinal whitening within the macula in the right eye, corresponding to an area of decreased retinal perfusion on optical coherence topography (OCT)-angiography and a hyperreflective middle retina band on spectral domain-OCT. This was consistent with a diagnosis of paracentral acute middle maculopathy (PAMM). PAMM should be considered a part of the differential diagnosis in patients with focal visual disturbances, and OCT studies are recommended as part of the work up as subtle fundus findings may be missed.
Atypical Ocular Coloboma in Tuberous Sclerosis-2: Report of Two Novel Cases
Bacci, Giacomo M. MD, PhD; Polizzi, Silvio MD; Mari, Francesco MD, PhD; Conti, Valerio PhD; Caputo, Roberto MD; Guerrini, Renzo MD, FRCP
doi : 10.1097/WNO.0000000000001099
September 2021 - Volume 41 - Issue 3 - p e363-e365
Tuberous sclerosis complex (TSC) is an autosomal dominant multisystemic disorder caused by mutations in either TSC1 or TSC2 genes and is characterized by hamartomas in multiple organs. The most frequent and best-known ocular manifestation in TSC is the retinal hamartoma. Less frequent ocular manifestations include punched out areas of retinal depigmentation, eyelid angiofibromas, uveal colobomas, papilledema, and sector iris depigmentation. In this article, we report 2 patients carrying known pathogenic variants in the TSC2 gene who exhibited an atypical, unilateral, iris coloboma associated with localized areas of retinal dysembryogenesis.
Partial Vision Loss After Orbital Decompression in a Patient With Thyroid Eye Disease, Chorioretinal Folds, and Disc Edema
DeMaria, Lauren N. MD; Tran, Ann Q. MD; Tooley, Andrea A. MD; Elmalem, Valerie I. MD; Belinsky, Irina MD
doi : 10.1097/WNO.0000000000001174
September 2021 - Volume 41 - Issue 3 - p e366-e368
Concomitant chorioretinal folds with disc edema can be seen in cases of thyroid eye disease presenting with compressive optic neuropathy and may portend optic nerve ischemia. We describe an unusual case of a 64-year-old man who developed partial vision loss after orbital decompression.
Isolated Oculomotor Neuritis After Dengue Fever
Bhate, Manjushree MD; Ponnaganti, Sandeep MD
doi : 10.1097/WNO.0000000000001223
September 2021 - Volume 41 - Issue 3 - p e369-e371
We report a rare case of isolated partial left III cranial nerve palsy due to inflammatory oculomotor neuritis after dengue fever with unique neuro-imaging findings of enhancement seen along the entire course of the oculomotor nerve.
Fascicular Sixth Nerve Palsy as a Presenting Sign of Metastatic Ovarian Carcinoma
Rohani, Niloufar BBA; Mortensen, Peter MD; Lee, Andrew G. MD
doi : 10.1097/WNO.0000000000001188
September 2021 - Volume 41 - Issue 3 - p e372-e374
The most common etiology of an isolated abducens nerve palsy in an older adult with vasculopathic risk factors is presumed microvascular ischemia. Because the diagnosis of microvascular ischemia in this setting would not alter patient management, there is debate as to whether further workup is warranted under these circumstances. In this report, we describe a fascicular sixth nerve palsy as the initial presenting sign of metastatic ovarian carcinoma, and we highlight the importance of considering additional workup in select cases of isolated abducens nerve palsies.
Gadoxetate Disodium-Enhanced Imaging of Gradenigo Syndrome in End-Stage Renal Disease
Sattarova, Victoria MD, MSc; Gencturk, Mehmet MD; Lee, Michael S. MD; McClelland, Collin M. MD
doi : 10.1097/WNO.0000000000001218
September 2021 - Volume 41 - Issue 3 - p e375-e377
A 65-year-old man with end-stage renal disease on peritoneal dialysis was admitted for new onset binocular double vision, failure to thrive, and debilitating right-sided headaches. Medical history was significant for tympanomastoidectomy for polymicrobial mastoiditis and treatment with IV antibiotics. MRI brain without contrast was read by radiology initially as showing expected postsurgical changes; however, given patient's history of mastoiditis infection, there was a high clinical suspicion for Gradenigo syndrome. MRI brain was repeated with gadoxetate contrast to minimize the risk of nephrogenic systemic fibrosis (NSF) in a patient with severe renal disease and this revealed an intracranial empyema involving both the trigeminal and abducens nerves confirming the diagnosis of Gradenigo syndrome. This case presented a diagnostic challenge because of suboptimal visualization on initial nonenhanced MRI. Gadoxetate was chosen because of its unique properties including high hepatobiliary excretion making it a safer form of gadolinium-based contrast agent that may not have the potential to precipitate NSF.
Facial Nerve Involvement Accompanying Optic Neuritis Secondary to Myelin Oligodendrocyte Glycoprotein Antibody–Associated Disorder
Chen, Zhiyong MRCP; Saini, Monica MRCP; Loo, Jing Liang FRCS (Ed)
doi : 10.1097/WNO.0000000000001145
September 2021 - Volume 41 - Issue 3 - p e378-e380
We present the first reported case of facial nerve involvement accompanying an optic neuritis in myelin oligodendrocyte glycoprotein antibody–associated disorder.
A Great Conversation With Dr. David Knox
Seay, Meagan D. DO; Calix, Rachel MD; Digre, Kathleen B. MD
doi : 10.1097/WNO.0000000000001338
September 2021 - Volume 41 - Issue 3 - p e381-e386
A Great Conversation With Dr. James Corbett
Digre, Kathleen B.; Seay, Meagan D.; Calix, Rachel A.
doi : 10.1097/WNO.0000000000001350
September 2021 - Volume 41 - Issue 3 - p e387-e393
The American Academy of Neurology Held Its First-Ever Virtual Meeting April 17–22, 2021
Digre, Kathleen B. MD; Seay, Meagan DO; Biousse, Valérie MD; Newman, Nancy J. MD
doi : 10.1097/WNO.0000000000001328
September 2021 - Volume 41 - Issue 3 - p e394
The 2021 American Headache Society Meeting
Digre, Kathleen B. MD
doi : 10.1097/WNO.0000000000001379
September 2021 - Volume 41 - Issue 3 - p e395
Neuro-Ophthalmology at Your Fingertips: A Free Website Authored by Jonathan Trobe, MD
Trobe, Jonathan D. MD
doi : 10.1097/WNO.0000000000001322
September 2021 - Volume 41 - Issue 3 - p e396
Neuro-Ophthalmology in India
Karna, Satya DO, DNB
doi : 10.1097/WNO.0000000000001252
September 2021 - Volume 41 - Issue 3 - p e397-e400
Possible Post-traumatic Abducens Nerve Palsy in a 16th Century Fresco (“the Chamber of the Giants” by Giulio Romano)
Perciaccante, Antonio MD; Cucu, Andrei I. MD
doi : 10.1097/WNO.0000000000001368
September 2021 - Volume 41 - Issue 3 - p e401-e402
Vitamin A as a Cause of Pseudotumor Cerebri Syndrome: Not to Be Forgotten
Fuerst, Jonathan S. MD; Wann, Robert C. MD; Kline, Lanning B. MD
doi : 10.1097/WNO.0000000000001230
September 2021 - Volume 41 - Issue 3 - p e403-e406
Neuro-Ophthalmic Manifestations of Mollaret Meningitis
Park, Benjamin BBA; Harish Bindiganavile, Shruthi MD; Nakawah, Mohammad O. MD; Bhat, Nita MD; Lee, Andrew G. MD
doi : 10.1097/WNO.0000000000001152
September 2021 - Volume 41 - Issue 3 - p e407-e409
HIV-Associated Rapidly Progressive Lymphoma of the Cavernous Sinus
Kvernland, Alexandra MD; Grossman, Scott N. MD; Levitan, Valeriya MD; Gold, Doria MD; Galetta, Steven L. MD
doi : 10.1097/WNO.0000000000001198
September 2021 - Volume 41 - Issue 3 - p e410-e412
Sixty-Eight–Year-Old Woman With Double Vision and Headache
Seery, Christopher W. MD; Frohman, Larry P. MD
doi : 10.1097/WNO.0000000000001272
September 2021 - Volume 41 - Issue 3 - p e413
An Older Patient With Difficulty Reading
Pelak, Victoria S. MD
doi : 10.1097/WNO.0000000000001345
September 2021 - Volume 41 - Issue 3 - p e414
