Journal of Neuro-Ophthalmology




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Why They Took the Oath: A Spotlight on Resident Sacrifice During the Pandemic

Engelhard, Stephanie B. MD; Bruce, Samuel S. MD; Chwalisz, Bart K. MD; Dinkin, Marc J. MD

doi : 10.1097/WNO.0000000000001202

March 2021 - Volume 41 - Issue 1 - p 1-5

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Resident and Fellow Training in a Pandemic

Grossman, Scott N. MD; Galetta, Steven L. MD; Lee, Andrew G. MD; Biousse, Valerie MD; Ishida, Koto MD

doi : 10.1097/WNO.0000000000001215

March 2021 - Volume 41 - Issue 1 - p 6-9

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Practical Approach to the Tele-Neuro-Ophthalmology and Neuro-Otology Visits: Instructional Videos

Calix, Rachel MBBS; Grossman, Scott N. MD; Rasool, Nailyn MD; Small, Leslie OD; Cho, Catherine MD; Galetta, Steven L. MD; Balcer, Laura J. MD, MSCE; Rucker, Janet C. MD

doi : 10.1097/WNO.0000000000001195

March 2021 - Volume 41 - Issue 1 - p 10-12

A collection of instructional videos that illustrate a step by step approach to tele-neuro-ophthalmology and neuro-otology visits. These videos provide instruction for patient preparation for their video visit, patient and provider interface with an electronic medical record associated video platform, digital applications to assist with vision testing, and practical advice for detailed remote neuro-ophthalmologic and neuro-otologic examinations.

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Increased Intracranial Pressure Without Hydrocephalus Associated With Spinal Cord Tumor: Literature Review

Zhou, Yujia MD, MS; McClelland, Collin M. MD; Lee, Michael S. MD

doi : 10.1097/WNO.0000000000001026

March 2021 - Volume 41 - Issue 1 - p 13-18

Spinal cord tumors (SCTs) may rarely cause increased intracranial pressure without hydrocephalus (IICPWH). A review of the English literature published after 1970 revealed 29 cases of IICPWH secondary to SCT. The following data were acquired: demographics, tumor characteristics, ophthalmic and neurological manifestations, and cerebral spinal fluid (CSF) features. We summarize the existing literature regarding various theories of pathophysiology, spinal imaging recommendations, and treatment modalities used in managing such patients. Patients with papilledema who also have neurological signs or symptoms of myelopathy or elevated CSF protein particularly in the setting of an atypical demographic for pseudotumor cerebri should raise a suspicion for a spinal tumor and prompt further investigation with a spinal MRI.

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Utilization of Visual Acuity Retroilluminated Charts for the Assessment of Afferent Visual System Dysfunction in a Pediatric Neuroimmunology Population

Sguigna, Peter V. MD; McCreary, Morgan C. PhD; Conger, Darrel L. CRA; Graves, Jennifer S. MD, PhD; Benson, Leslie A. MD; Waldman, Amy T. MD; Greenberg, Benjamin M. MD, MHS, on behalf of the PERCEPTION Collaboration

doi : 10.1097/WNO.0000000000001001

March 2021 - Volume 41 - Issue 1 - p 19-23

Visual acuity has been a significant outcome measure in clinical trials for patients suffering from neuro-ophthalmological diseases and multiple sclerosis; however, there are limited data on the comparison of various testing strategies in pediatric patients with these disorders. Clinical trials using vision as an outcome could include a variety of tools to assess the acuity, including 2-m and 4-m standardized retroilluminated charts.

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Site of Origin of the Ophthalmic Artery Influences the Risk for Retinal Versus Cerebral Embolic Events

Rossin, Elizabeth J. MD, PhD; Gilbert, Aubrey L. MD, PhD; Koen, Nicholas BS; Leslie-Mazwi, Thabele M. MD; Cunnane, Mary E. MD; Rizzo, Joseph F. III MD

doi : 10.1097/WNO.0000000000000883

March 2021 - Volume 41 - Issue 1 - p 24-28

Embolic events leading to retinal ischemia or cerebral ischemia share common risk factors; however, it has been well documented that the rate of concurrent cerebral infarction is higher in patients with a history of transient ischemic attack (TIA) than in those with monocular vision loss (MVL) due to retinal ischemia. Despite the fact that emboli to the ophthalmic artery (OA) and middle cerebral artery share the internal carotid artery (ICA) as a common origin or transit for emboli, the asymmetry in their final destination has not been fully explained. We hypothesize that the anatomic location of the OA takeoff from the ICA may contribute to the differential flow of small emboli to the retinal circulation vs the cerebral circulation.

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Neuro-Ophthalmologic Features and Outcomes of Thalamic Infarction: A Single-Institutional 10-Year Experience

Moon, Yeji MD; Eah, Kyu Sang MD; Lee, Eun-Jae MD, PhD; Kang, Dong-Wha MD, PhD; Kwon, Sun Uck MD, PhD; Kim, Jong Sung MD, PhD; Lim, Hyun Taek MD, PhD

doi : 10.1097/WNO.0000000000000864

March 2021 - Volume 41 - Issue 1 - p 29-36

Neuro-ophthalmologic deficit after thalamic infarction has been of great concern to ophthalmologists because of its debilitating impacts on patients' daily living. We aimed to describe the visual and oculomotor features of thalamic infarction and to delineate clinical outcomes and prognostic factors of the oculomotor deficits from an ophthalmologic point of view.

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Patterns of Retinal Ganglion Cell Damage in Nonarteritic Anterior Ischemic Optic Neuropathy Assessed by Swept-Source Optical Coherence Tomography

Jiang, Jingjing MD; Wang, Zhijun MSc; Chen, Yi MD; Li, Aihong MBBS; Sun, Chuan MD; Sun, Xinquan MBBS

doi : 10.1097/WNO.0000000000001025

March 2021 - Volume 41 - Issue 1 - p 37-47

To evaluate the ability of macular ganglion cell and inner plexiform layer (mGCIPL) and retinal nerve fiber layer (RNFL) thickness measurements by long-wavelength swept-source optical coherence tomography (SS-OCT) to assess retinal ganglion cell (RGC) damage in nonarteritic anterior ischemic optic neuropathy (NAION).

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Correlation Between Optic Disc Peripapillary Capillary Network and Papilledema Grading in Patients With Idiopathic Intracranial Hypertension: A Study of Optical Coherence Tomography Angiography

Rodriguez Torres, Yasaira MD; Lee, Patrick BS; Mihlstin, Melanie MD; Tomsak, Robert L. MD, PhD

doi : 10.1097/WNO.0000000000000877

March 2021 - Volume 41 - Issue 1 - p 48-53

The continued increase in idiopathic intracranial hypertension (IIH) prevalence has many implications for societal health care. Its potential vision-threatening consequences make ophthalmologists key players in its diagnosis and management. Newer technology such as optical coherence tomography angiography (OCT-A) enables evaluation of the branching complexity of the peripapillary capillary plexus, a region where accurate imaging via fluorescein angiography was previously limited.

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Magnetic Resonance Imaging Abnormalities of the Optic Nerve Sheath and Intracranial Internal Carotid Artery in Giant Cell Arteritis

Gospe, Sidney M. III MD, PhD; Amrhein, Timothy J. MD; Malinzak, Michael D. MD, PhD; Bhatti, M. Tariq MD; Mettu, Pradeep MD; El-Dairi, Mays A. MD

doi : 10.1097/WNO.0000000000000860

March 2021 - Volume 41 - Issue 1 - p 54-59

Giant cell arteritis (GCA) is an important diagnostic consideration in elderly patients with vision changes. Superficial temporal artery biopsy (TAB) has long been considered the gold standard diagnostic approach for GCA, but MRI has gained interest as an alternative diagnostic modality. Although most of the literature has focused on imaging abnormalities of branches of the external carotid artery, there have been a few reports of GCA-related inflammatory involvement of the orbit and internal carotid arteries (ICAs) on MRI.

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Shortened Pattern Electroretinogram Latency and Impaired Autoregulatory Dynamics to Steady-State Stimuli in Patients With Multiple Sclerosis

Jiang, Hong MD, PhD; Gameiro, Giovana R.; Hu, Huiling MD, PhD; Monsalve, Pedro F. MD; Dong, Chuanchui PhD; Hernandez, Jeffrey RN; Delgado, Silvia R. MD; Porciatti, Vittorio D. PhD; Wang, Jianhua MD, PhD

doi : 10.1097/WNO.0000000000000894

March 2021 - Volume 41 - Issue 1 - p 60-68

The steady-state pattern electroretinogram (PERG) is a sensitive measure of retinal ganglion cell (RGC) function that includes within-test progressive changes—adaptation—reflecting RGC autoregulatory dynamics. Comprehensive PERG assessment in patients with multiple sclerosis (MS) (with or without optic neuritis [ON]) may provide unique information about RGC dysfunction and its progression, as well as a comparison between functional loss and structural loss as measured by optical coherence tomography (OCT). The goal of this project was to measure steady-state PERG components and their associations with intraretinal layer thicknesses in MS.

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The Clinical and Imaging Profile of Skew Deviation: A Study of 157 Cases

Walter, Eyal MD; Trobe, Jonathan D. MD

doi : 10.1097/WNO.0000000000000915

March 2021 - Volume 41 - Issue 1 - p 69-76

Skew deviation, a vertical misalignment of the eyes caused by a lesion in the vestibulo-ocular pathway, is a common manifestation of brainstem dysfunction, yet comprehensive information about its clinical profile is lacking. The aim of this study was to document presenting symptoms, causes, ocular alignment features, accompanying neurologic signs, pertinent brain imaging abnormalities, and measures used to relieve diplopia.

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Optical Coherence Tomography Neuro-Toolbox for the Diagnosis and Management of Papilledema, Optic Disc Edema, and Pseudopapilledema

Sibony, Patrick A. MD; Kupersmith, Mark J. MD; Kardon, Randy H. MD, PhD

doi : 10.1097/WNO.0000000000001078

March 2021 - Volume 41 - Issue 1 - p 77-92

Distinguishing optic disc edema from pseudopapilledema is a common, sometimes challenging clinical problem. Advances in spectral-domain optical coherence tomography (SD-OCT) of the optic nerve head (ONH) has proven to be a cost effective, noninvasive, outpatient procedure that may help. At its core are tools that quantify the thickness of the retinal nerve fiber layer (RNFL) and ganglion cell–inner plexiform layer (GC-IPL). The SD-OCT also provides a set of tools that may be qualitatively interpreted in the same way that we read an MRI. They include the transverse axial, en face, and circular tomogram. Our goal is to describe a practical office-based set of tools using SD-OCT in the diagnosis and monitoring of papilledema, optic disc edema, and pseudopapilledema.

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Idiopathic Intracranial Hypertension: Glymphedema of the Brain

Nicholson, Patrick MD; Kedra, Alice MD; Shotar, Eimad MD; Bonnin, Sophie MD; Boch, Anne-Laure MD; Shor, Natalia MD; Clarençon, Frédéric MD, PhD; Touitou, Valérie MD, PhD; Lenck, Stephanie MD

doi : 10.1097/WNO.0000000000001000

March 2021 - Volume 41 - Issue 1 - p 93-97

During the last decade, our understanding of cerebrospinal fluid (CSF) physiology has dramatically improved, thanks to the discoveries of both the glymphatic system and lymphatic vessels lining the dura mater in human brains.

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Diagnostic Error of Neuro-ophthalmologic Conditions: State of the Science

Stunkel, Leanne MD; Newman-Toker, David E. MD, PhD; Newman, Nancy J. MD; Biousse, Valérie MD

doi : 10.1097/WNO.0000000000001031

March 2021 - Volume 41 - Issue 1 - p 98-113

Diagnostic error is prevalent and costly, occurring in up to 15% of US medical encounters and affecting up to 5% of the US population. One-third of malpractice payments are related to diagnostic error. A complex and specialized diagnostic process makes neuro-ophthalmologic conditions particularly vulnerable to diagnostic error.

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Nelson Syndrome: Clival Invasion of Corticotroph Pituitary Adenoma Resulting in Alternating Sixth Nerve Palsies

Douglas, Vivian P. MD, DVM, MBA; Douglas, Konstantinos A. A. MD, DVM, MBA; Rapalino, Otto MD; Champion, Samantha N. MD; Chwalisz, Bart K. MD

doi : 10.1097/WNO.0000000000001141

March 2021 - Volume 41 - Issue 1 - p 114-118

A 44-year-old woman presented with 2 painful and self-limited episodes of binocular horizontal diplopia within 1 year that at the beginning were thought to be secondary to microvascular insult. Her medical history was significant for Cushing syndrome status post transsphenoidal resection with bilateral adrenalectomy 4 years prior, hypertension, and diabetes mellitus.

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Is Cataract Surgery a Risk for Developing Nonarteritic Anterior Ischemic Optic Neuropathy?

McCulley, Timothy J. MD; Miller, Neil R. MD

doi : 10.1097/WNO.0000000000001109

March 2021 - Volume 41 - Issue 1 - p 119-125

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“On the Ophthalmoscopic Signs of Spinal Disease” 150 Years Later: A Tribute to Professor Sir Thomas Clifford Allbutt

Hernandez Fustes, Otto Jesus MD, MSc; Kamoi Kay, Cl?udia Suemi MD; Lorenzoni, Paulo José MD, PhD; Dal-Pr? Ducci, Renata MD, PhD; Werneck, Lineu Cesar MD, PhD; Scola, Rosana Herminia MD, PhD

doi : 10.1097/WNO.0000000000001139

March 2021 - Volume 41 - Issue 1 - p 126-127

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Literature Commentary

Moster, Mark L. MD; Bhatti, M. Tariq MD

doi : 10.1097/WNO.0000000000001201

March 2021 - Volume 41 - Issue 1 - p 128-134

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Idiopathic Intracranial Hypertension Without Papilledema With Improvement in Visual Field Defect Following Optic Nerve Sheath Fenestration: Comment

Margolin, Edward MD

doi : 10.1097/WNO.0000000000000968

March 2021 - Volume 41 - Issue 1 - p 135

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Scleral Lenses Versus Surgery for Ptosis in Progressive External Ophthalmoplegia Plus Respectively Kearns–Sayre Syndrome

Finsterer, Josef MD, PhD

doi : 10.1097/WNO.0000000000001065

March 2021 - Volume 41 - Issue 1 - p 136-137

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Severe Chronic Progressive External Ophthalmoplegia–Associated Ptosis Successfully Treated With Scleral Lenses: Response

Cherny, Christina BS; Sherman, Suzanne W. OD; Dagi Glass, Lora R. MD

doi : 10.1097/WNO.0000000000001066

March 2021 - Volume 41 - Issue 1 - p 137-138

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Oculomotor Palsy Due to Malignant Nerve Sheath Tumor: Aberrant Regeneration of the Third Nerve but Without Pupil Involvement

Toohey, Thomas P. BMed; Wong, Elizabeth L. S. BMed; Ting, Eugene MMed, MBBS; Xiradis, Nicholas BMedSc, MD; Khan, Muhammad BMed; Lim, Zhi Wei BSc(Hons); Blah, Tyler BBiomedSci(Hons); Jiang, James BSc, MD; Li, Yunding MBBS; Coroneo, Minas T. FRACS, MD; Agar, Ashish FRANZCO, PhD; Francis, Ian C. FRACS, PhD

doi : 10.1097/WNO.0000000000001093

March 2021 - Volume 41 - Issue 1 - p 138-140

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Oculomotor Palsy Due to Malignant Nerve Sheath Tumor: Aberrant Regeneration of the Third Nerve but Without Pupil Involvement: Response

Micieli, Jonathan A. MD, CM; Newman, Nancy J. MD

doi : 10.1097/WNO.0000000000001092

March 2021 - Volume 41 - Issue 1 - p 140

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Idiopathic Intracranial Hypertension Without Papilledema With Improvement in Visual Field Defect Following Optic Nerve Sheath Fenestration: Response

Scofield-Kaplan, Stacy M. MD; Patel, Kishan G. MD; Jones, Freddie Ray Jr MD; Hogan, Robert Nick MD, PhD

doi : 10.1097/WNO.0000000000000969

March 2021 - Volume 41 - Issue 1 - p 135-136

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A Zoom Conversation With Richard Sogg, MD, on the Occasion of His 90th Birthday, August 2020

Trobe, Jonathan D. MD

doi : 10.1097/WNO.0000000000001199

March 2021 - Volume 41 - Issue 1 - p e1-e5

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American Academy of Neurology Fall Conference 2020

Newman, Nancy J. MD

doi : 10.1097/WNO.0000000000001200

March 2021 - Volume 41 - Issue 1 - p e6

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Segmental Imaging of the Trochlear Nerve: Anatomic and Pathologic Considerations

Agarwal, Nivedita MD; Ahmed, Ali Karim MD; Wiggins, Richard H. III MD; McCulley, Timothy J. MD; Kontzialis, Marinos MD; Macedo, Leonardo L. MD; Choudhri, Asim F. MD; Ditta, Lauren C. MD; Ishii, Masaru MD; Gallia, Gary L. MD, PhD; Aygun, Nafi MD; Blitz, Ari M. MD

doi : 10.1097/WNO.0000000000001125

March 2021 - Volume 41 - Issue 1 - p e7-e15

The trochlear nerve (the fourth cranial nerve) is the only cranial nerve that arises from the dorsal aspect of the midbrain. The nerve has a lengthy course making it highly susceptible to injury. It is also the smallest cranial nerve and is often difficult to identify on neuroimaging.

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Superonasal Transconjunctival Optic Nerve Sheath Decompression: A Simplified Technique for Safe and Efficient Decompression

Melson, Andrew T. MD; Warmath, Jacob D. BS; Moreau, Annie MD; Farris, Bradley K. MD

doi : 10.1097/WNO.0000000000000898

March 2021 - Volume 41 - Issue 1 - p e16-e21

Severe, permanent vision loss is a feared sequela of untreated or refractory idiopathic intracranial hypertension (IIH). For patients with progressive vision loss despite maximally tolerated medical treatment, optic nerve sheath decompression (ONSD) remains a viable and effective option to protect vision. Our objective is to introduce a modified transconjunctival technique for ONSD and determine its safety, efficacy, and efficiency in patients with IIH.

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Visual Outcome Improvement in Tacrolimus-Related Bilateral Optic Neuropathy

Demontes, Marie MD; Thia-Soui-Tchong, Kim MD; Ronsin, Solène MD; Desestret, Virginie MD, PhD; Vighetto, Alain MD; Tilikete, Caroline MD, PhD

doi : 10.1097/WNO.0000000000000859

March 2021 - Volume 41 - Issue 1 - p e22-e24

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Photopsias in the Setting of Nivolumab Therapy

Ahluwalia, Aneesha BS; Kohli, Anita A. MD

doi : 10.1097/WNO.0000000000000909

March 2021 - Volume 41 - Issue 1 - p e25-e26

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Reversal of Vision Loss in a 49-Year-Old Man With Progressive Optic Atrophy Due to Profound Biotinidase Deficiency

Kellom, Elizabeth R. MS, CGC; Wolf, Barry MD, PhD; Rice, Gregory M. MD; Stepien, Kimberly E. MD

doi : 10.1097/WNO.0000000000000933

March 2021 - Volume 41 - Issue 1 - p e27-e30

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Idiopathic Intracranial Hypertension Without Papilledema With Improvement in Visual Field Defect Following Optic Nerve Sheath Fenestration

Scofield-Kaplan, Stacy M. MD; Patel, Kishan MD; Jones, Freddie Ray Jr MD; Hogan, Robert Nick MD, PhD

doi : 10.1097/WNO.0000000000000868

March 2021 - Volume 41 - Issue 1 - p e31-e33

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Papillitis, a Rare Cytomegalovirus Manifestation in an Immunocompetent Host

O'Brien, Timothy N. BPharm, MPharm, MB, BCh, BAO, MRCPI; O'Connor, Gerard M. FRCS, FRCOphth; Lefter, Stela MD, MRCPI

doi : 10.1097/WNO.0000000000000925

March 2021 - Volume 41 - Issue 1 - p e34-e35

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Ipsilateral Recurrence of Optic Disc Drusen–Associated Anterior Ischemic Optic Neuropathy in a 15-Year-Old Boy

Al-Bakri, Moug MD; Larsen, Ann-Cathrine MD, PhD; Malmqvist, Lasse MD, PhD; Hamann, Steffen MD, PhD

doi : 10.1097/WNO.0000000000000941

March 2021 - Volume 41 - Issue 1 - p e36-e38

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Slowly Progressive Optic Perineuritis as the First Clinical Manifestation of Sarcoidosis

Garr?, Federica MD; Rinaldi, Francesca MD; Perini, Paola MD; Miscioscia, Alessandro MD; Simonato, Davide MD; Pizzi, Marco MD; Margoni, Monica MD; Gallo, Paolo MD, PhD

doi : 10.1097/WNO.0000000000000924

March 2021 - Volume 41 - Issue 1 - p e39-e41

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Perineural Optic Nerve Enhancement in Giant Cell Arteritis: A Case Report and Review of the Literature

Nguyen, Kimberly K. BS; Al Othman, Bayan A. MD; Kini, Ashwini T. MD; Lee, Andrew G. MD

doi : 10.1097/WNO.0000000000000914

March 2021 - Volume 41 - Issue 1 - p e42-e45

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Myelin Oligodendrocyte Glycoprotein Antibody-Positive Optic Neuritis Presenting as Idiopathic Orbital Inflammatory Syndrome

Wagley, Sushant MD; Wang, Yao MD; McClelland, Collin M. MD; Chen, John J. MD, PhD; Lee, Michael S. MD

doi : 10.1097/WNO.0000000000000918

March 2021 - Volume 41 - Issue 1 - p e46-e47

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Sj?gren Disease and Myelin Oligodendrocyte Glycoprotein Antibody–Associated Optic Neuritis

Mittal, Aman MD; Baig, Iyza F. MD; Merchant, Arjun G. BS; Chen, John J. MD, PhD; Choi, Jeanie J. MD; Goldberg, Alla MD; Adesina, Ore-Ofe O. MD

doi : 10.1097/WNO.0000000000000945

March 2021 - Volume 41 - Issue 1 - p e48-e50

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Choriocapillaris Integrity in Relapsed Central Serous Chorioretinopathy in a Patient Treated With Fingolimod for Multiple Sclerosis: New Insights From Optical Coherence Tomography Angiography

Ziccardi, Lucia MD, PhD; Landi, Doriana MD, PhD; De Geronimo, Daniele MD; Barbano, Lucilla MD; Giorno, Paola MD; Marfia, Girolama Alessandra MD, PhD; Albanese, Maria MD, PhD; Parisi, Vincenzo MD; Parravano, Mariacristina MD

doi : 10.1097/WNO.0000000000000937

March 2021 - Volume 41 - Issue 1 - p e51-e53

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Spontaneous Globe Luxation–Induced Optic Neuropathy

Mammo, Danny A. MD; Wang, Yao MD; Lee, Michael S. MD; Mokhtarzadeh, Ali MD; Harrison, Andrew R. MD; McClelland, Collin M. MD

doi : 10.1097/WNO.0000000000000912

March 2021 - Volume 41 - Issue 1 - p e54-e56

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Atherosclerosis of Intracranial Internal Carotid Artery Causing Embolic Ocular Events

Tong, Lili MD; Lee, Michael MD; Margolin, Edward MD

doi : 10.1097/WNO.0000000000000934

March 2021 - Volume 41 - Issue 1 - p e57-e59

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Simultaneous Bilateral Serous Retinal Detachments and Cortical Visual Loss in the PRES HELLP Syndrome

Kini, Ashwini T. MD; Tabba, Subhan BS, MBA; Mitchell, Travis BS; Al Othman, Bayan MD; Lee, Andrew G. MD

doi : 10.1097/WNO.0000000000000942

March 2021 - Volume 41 - Issue 1 - p e60-e63

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Delayed Diagnosis of Posterior Reversible Encephalopathy Syndrome: A Case Report

Pegany, Roma MD; Olson, Daniel J. MD; Aldaas, Khalid M. BS; Sitko, Kevin R. MD

doi : 10.1097/WNO.0000000000000943

March 2021 - Volume 41 - Issue 1 - p e64-e65

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Cortical Visual Impairment Treated by Plasmapheresis in a Child With Metronidazole-Induced Encephalopathy

Chang, Melinda Y. MD; Borchert, Mark S. MD

doi : 10.1097/WNO.0000000000000871

March 2021 - Volume 41 - Issue 1 - p e66-e68

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Cutaneous Angiomyolipoma of the Eyelid in a 2-Year-Old With Tuberous Sclerosis Complex

Ditta, Lauren C. MD; Zhang, Jie MD; Bibars, Wafi MD; Bissler, John MD

doi : 10.1097/WNO.0000000000000863

March 2021 - Volume 41 - Issue 1 - p e69-e70

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Immune Checkpoint Inhibitor–Induced Ptosis in a Patient With Prostate Cancer

Williams, Katherine J. MD; Allen, Richard C. MD, PhD, FACS

doi : 10.1097/WNO.0000000000000927

March 2021 - Volume 41 - Issue 1 - p e71-e72

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Case of Facial Neuromyotonia

Rivera, Caleb Z. BS; Solomon, Alexander M. MD; Francis, Courtney E. MD

doi : 10.1097/WNO.0000000000000910

March 2021 - Volume 41 - Issue 1 - p e73-e74

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Pulmonary Arteriovenous Malformation-Related Embolic Stroke Causing Thalamic Esotropia

Panneerselvam, Sugi BA; Al Othman, Bayan MD; Kini, Ashwini MD; Lee, Andrew G. MD

doi : 10.1097/WNO.0000000000000866

March 2021 - Volume 41 - Issue 1 - p e75-e76

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Nonsecretory Recurrence of Multiple Myeloma Presenting as Sixth Nerve Palsy Secondary to Clival Plasmacytoma

Kaufman, Aaron R. MD; Quillen, Karen MD, MPH; Distefano, Alberto G. MD; Sloan, J. Mark MD

doi : 10.1097/WNO.0000000000000939

March 2021 - Volume 41 - Issue 1 - p e77-e78

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Fourth Cranial Nerve Palsy Associated With Sildenafil Citrate

Lee, Yumi MD; Park, Kyung-Ah PhD; Oh, Sei Yeul PhD; Min, Ju-Hong PhD; Kim, Byoung Joon PhD

doi : 10.1097/WNO.0000000000000938

March 2021 - Volume 41 - Issue 1 - p e79-e80

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Treatment of Horizontal Binocular Diplopia With Prismatic Contact Lenses

Lee, Samuel K.; Zabrowski, Cheryl OD; McClelland, Collin M. MD; Lee, Michael S. MD

doi : 10.1097/WNO.0000000000000936

March 2021 - Volume 41 - Issue 1 - p e81-e82

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Thyroid Eye Disease in Hashimoto Ataxia

Kanakamedala, Amritha D. BA; Al Othman, Bayan A. MD; Kini, Ashwini T. MD; Lee, Andrew G. MD

doi : 10.1097/WNO.0000000000000884

March 2021 - Volume 41 - Issue 1 - p e83-e84

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A Novel De Novo KIF21A Variant in a Patient With Congenital Fibrosis of the Extraocular Muscles With a Syndromic CFEOM Phenotype

Soliani, Luca MD; Spagnoli, Carlotta MD; Salerno, Grazia G. MD; Mehine, Miika PhD; Rizzi, Susanna MD; Frattini, Daniele MD; Koskenvuo, Juha MD, PhD; Fusco, Carlo MD

doi : 10.1097/WNO.0000000000000921

March 2021 - Volume 41 - Issue 1 - p e85-e88

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Metastatic Malignant Melanoma to the Cavernous Sinus: A Case of Recurrent Disease Presenting as Parkinson Syndrome

Dryden, Stephen C. MD; Kudrna, Jeremy J. MS, BS; Thompson, Barrett N. MD; Patel, Rutvi B. MD; Choudhri, Asim F. MD; Ditta, Lauren C. MD

doi : 10.1097/WNO.0000000000000886

March 2021 - Volume 41 - Issue 1 - p e89-e90

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RP2 Rod–Cone Dystrophy Causes Spasmus Nutans–Like Nystagmus

Yoo, Tae Keun MD; Han, Sueng-Han MD, PhD; Han, Jinu MD

doi : 10.1097/WNO.0000000000000880

March 2021 - Volume 41 - Issue 1 - p e91-e93

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Upbeat Nystagmus in Dorsolateral Pontine Infarction

Chang, Tzu-Pu MD; Zee, David S. MD; Gold, Daniel R. DO

doi : 10.1097/WNO.0000000000000928

March 2021 - Volume 41 - Issue 1 - p e94-e96

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Not All That Flickers Is Snow

Patel, Rachel C. MD; Vitale, Albert T. MD; Creel, Donnell J. PhD; Digre, Kathleen B. MD

doi : 10.1097/WNO.0000000000000935

March 2021 - Volume 41 - Issue 1 - p e97-e99

A woman presented with bilateral visual disturbances that had been diagnosed as visual snow. Dilated ophthalmic examination and multimodal imaging were strongly suggestive of birdshot chorioretinopathy, meriting initiation of systemic immunomodulatory therapy. Visual snow requires a thorough ophthalmologic exam to exclude other ocular diseases.

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Dilation of the Perioptic Subarachnoid Space Anterior to Optic Nerve Sheath Meningioma

Arnold, Anthony C. MD; Lee, Andrew G. MD

doi : 10.1097/WNO.0000000000000908

March 2021 - Volume 41 - Issue 1 - p e100-e102

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Optic Neuropathy Due to Compression by an Ectatic Internal Carotid Artery Within the Orbital Apex

Caton, Michael T. Jr MD; Zamani, Amir A. MD; Du, Rose MD, PhD; Prasad, Sashank MD

doi : 10.1097/WNO.0000000000000975

March 2021 - Volume 41 - Issue 1 - p e103-e104

Neurovascular compression is a rare but potentially treatable cause of optic neuropathy. Although incidental contact of the cisternal optic nerve and internal carotid artery (ICA) is common, compressive optic neuropathy occurring within the orbital apex has not been comprehensively described. We report a case of intra-orbital and intracanalicular optic nerve compression due to an ectatic ICA in a patient with congenital absence of the contralateral ICA. This report describes the complementary roles of advanced neuroimaging and neuro-ophthalmologic examination in rendering the diagnosis.

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Retrobulbar Spot Sign in Metachronous Bilateral Central Retinal Artery Occlusion of Cardioembolic Origin

Lottspeich, Christian MD; Mackert, Marc J. MD; K?hler, Anton MD; Bauer, Axel MD; Hoffmann, Ulrich MD; Czihal, Michael MD

doi : 10.1097/WNO.0000000000000956

March 2021 - Volume 41 - Issue 1 - p e105-e106

A 78-year-old man suffered sudden visual loss of his right eye. Five years earlier, he had experienced vision loss of his left eye due to central retinal artery occlusion (CRAO); back then, the etiology for the CRAO was not established. Current ocular ultrasound depicted a hyperechoic spot within the optic nerve in both eyes. Echocardiography identified a calcified mass adherent to the mitral valve as the embolic source of the CRAO. This case shows the value of ocular B-mode ultrasound in demonstration and proof of the etiology for CRAO.

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Multicolor Imaging for the Detection of Inner Nuclear Layer Microcysts Secondary to Optic Nerve Atrophy

Jimenez-Rolando, Belen MD; Carre?o, Ester MD, PhD; Alonso-Peralta, Miguel A. MD; Lopez-Molina, Maria I. MD; Fernandez-Sanz, Guillermo MD

doi : 10.1097/WNO.0000000000000976

March 2021 - Volume 41 - Issue 1 - p e107-e110

Inner nuclear layer (INL) microcysts have been reported in diseases affecting the optic nerve. The new ocular imaging techniques detect minimal structural alterations at the macula and correlate these findings to different etiologies with less invasive procedures. The relationship between ganglion cells distribution at the macula and chiasmal nerve fibers enables the diagnosis and location of neurological lesions by new generation optical coherence tomography (SD-OCT) imaging devices. We report the evaluation of a patient with a history of optic nerve trauma and macular INL microcysts with multicolor SD-OCT technology that shows a pattern that localizes the lesion to the left optic nerve and proximal segment of the chiasm.

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Optical Coherence Tomography Findings in the Junctional Scotoma of Traquair

Harish Bindiganavile, Shruthi MD; Bhat, Nita MD; Adesina, Ore-Ofeoluwatomi MD; Lee, Andrew G. MD

doi : 10.1097/WNO.0000000000000972

March 2021 - Volume 41 - Issue 1 - p e111-e113

A 43-year-old woman presented with a right-sided visual field defect in the right eye. The visual acuity was normal and there was a right relative afferent pupillary defect. Formal visual field testing revealed a junctional scotoma of Traquair. The fundus examination showed optic atrophy in the right eye and optical coherence tomography demonstrated unilateral band atrophy. Neuroimaging revealed a sellar and suprasellar cystic pituitary adenoma for which she underwent a transsphenoidal drainage. We demonstrate the clinical and radiographic features of the junctional scotoma of Traquair and describe the differentiating features vs the junctional scotoma.

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Restricted Diffusion in the Optic Nerve Head After Shock-Induced Anterior Ischemic Optic Neuropathy

Al Othman, Bayan A. MD; Naser, Maryam MD; Kini, Ashwini T. MD; Lee, Andrew G. MD

doi : 10.1097/WNO.0000000000000897

March 2021 - Volume 41 - Issue 1 - p e114-e115

Shock-induced anterior ischemic optic neuropathy (SIAION) is a known type of optic neuropathy in patients who experienced shock related to different etiologies such as anemia and severe intradialytic hypotension like in our patient.

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Multimodal Imaging in a Case of Peripapillary Choroidal Neovascular Membrane Associated With Idiopathic Intracranial Hypertension

Igwe, Chinedu N. MBBS; Nderitu, Paul MBChB; Eframidis, Stefanos MD; Eleftheriadis, Haralabos MD; O'Sullivan, Eoin MD

doi : 10.1097/WNO.0000000000000948

March 2021 - Volume 41 - Issue 1 - p e116-e118

Optical coherence tomography angiography is one of the latest noninvasive imaging modalities for visualizing the vasculature of retina and choroid. We describe its application in the diagnosis, treatment, and monitoring of a patient with peripapillary choroidal neovascular membrane in the setting of idiopathic intracranial hypertension, who responded well to a course of ranibizumab intravitreal injections.

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Splenial Restricted Diffusion as MRI Correlate of Diaschisis in a Blind Infant With Unilateral Posterior Cerebral Artery Stroke

Eton, Emily A. MD; Patel, Tapan P. MD, PhD; Srinivasan, Ashok MD; Moritani, Toshio MD; Trobe, Jonathan D. MD

doi : 10.1097/WNO.0000000000000954

March 2021 - Volume 41 - Issue 1 - p e119-e121

A 3-month-old male infant appeared on multiple clinical examinations to have acutely developed bilateral retrogeniculate blindness. Electroencephalography showed focal status epilepticus confined to the left posterior cerebral hemisphere. MRI demonstrated restricted diffusion in the domain of the left posterior cerebral artery consistent with acute stroke. Notably, the restricted diffusion extended across the midline in the splenium of the corpus callosum. This splenial sign may be the imaging correlate of cerebral diaschisis, a well-described phenomenon in which patients with new brain lesions develop acutely impaired neurologic function in related but nonlesioned brain regions. Diaschisis has been posited as the explanation for the temporary bilateral blindness in adult patients suffering from unilateral occipital infarctions.

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Neurosarcoidosis Masquerading as Giant Cell Arteritis With Incidental Meningioma

Solyman, Omar M. MD; Vizcaino, Maria Adelita MD; Fu, Roxana MD; Henderson, Amanda D. MD

doi : 10.1097/WNO.0000000000000967

March 2021 - Volume 41 - Issue 1 - p e122-e124

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

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Bilateral Scalp Necrosis in Giant Cell Arteritis

Somani, Anisha N. BS; Al Othman, Bayan MD; Kini, Tonse MD; Lee, Andrew G. MD

doi : 10.1097/WNO.0000000000000862

March 2021 - Volume 41 - Issue 1 - p e125-e127

Giant cell arteritis (GCA) is a medium-to-large vessel vasculitis of the elderly. Common constitutional clinical features include headache, scalp tenderness, and jaw claudication. Severe unilateral or bilateral visual loss is the most feared ophthalmic complication of GCA. Scalp necrosis is a known ischemic complication of GCA with approximately 100 cases reported in the literature to date. We report a case of scalp pain and an erythematous cutaneous lesion in the distribution of ophthalmic division of the trigeminal nerve that mimicked herpes zoster ophthalmicus. A temporal artery biopsy was positive for GCA, and small vessel arteritis was seen at the time of simultaneous skin biopsy. To the best of our knowledge, this is the first such report in the English language ophthalmic literature.

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Headache and Bilateral Optic Disc Edema as the Initial Manifestation of Vogt–Koyanagi–Harada Disease

Nichani, Prem MSc; Christakis, Panos G. MD; Micieli, Jonathan A. MD

doi : 10.1097/WNO.0000000000000917

March 2021 - Volume 41 - Issue 1 - p e128-e130

A 31-year-old healthy Asian man presented with new headaches and blurred vision. He was found to have bilateral optic disc edema (ODE) and peripapillary subretinal fluid and was initially investigated for causes of raised intracranial pressure. After referral to neuro-ophthalmology, he received a diagnosis of Vogt–Koyanagi–Harada (VKH) disease and his symptoms, ODE, and vision improved promptly with prednisone treatment. HLA typing was performed and returned positive for the HLA-DRB1*04 subtype. Although VKH disease usually presents with granulomatous uveitis or serous retinal detachments, ODE may manifest early in the disease course. Those with ODE in VKH disease are believed to be older and female, but this case demonstrates that these findings were also seen in a young man.

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

Alrobaian, Malek A. MD; Vizcaino, Maria Adelita MD; Henderson, Amanda D. MD

doi : 10.1097/WNO.0000000000000920

March 2021 - Volume 41 - Issue 1 - p e131-e133

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

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Unilateral Fourth Nerve Palsy due to Presumed Metastatic Melanoma

Pasol, Joshua MD; Mellon, Eric A. MD PhD; Feun, Lynn G. MD; Saigal, Gaurav M. MD; Diwanji, Tejan P. MD; Komotar, Ricardo J. MD

doi : 10.1097/WNO.0000000000000902

March 2021 - Volume 41 - Issue 1 - p e134-e135

An 81-year-old man with a history of metastatic melanoma presented with sudden onset of painless, binocular vertical diplopia. The clinical examination was consistent with a right fourth nerve palsy. An MRI of the head revealed a mass dorsal to the right tectum at the level of the inferior colliculus. An MRI just 4 months prior did not show a lesion in that location. An MRA of the head did not show an aneurysm. This is a rare case of an isolated fourth nerve palsy believed to be due to metastatic melanoma compressing the nerve along the dorsal midbrain.

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Localizing Thalamomesencephalic Afferent and Efferent Pupillary Defects

Sharifi, Ardalan MD; Sigireddi, Rohini R. MD; Lyons, Lance J. MD; Kini, Ashwini T. MD; Al Othman, Bayan A. MD; Lee, Andrew G. MD

doi : 10.1097/WNO.0000000000000903

March 2021 - Volume 41 - Issue 1 - p e136-e138

A 42-year-old Algerian man presented for binocular oblique diplopia, hypersomnolence with drop attacks, bilateral hearing loss, and thoracic pain. He had a right thalamomesencephalic hemorrhage due to an underlying cavernous malformation treated with subtotal surgical resection. On neuro-ophthalmic examination, the patient had a left relative afferent pupillary defect and a right oculosympathetic efferent pupillary defect (i.e., Horner syndrome) in addition to other thalamomesencephalic eye and neurologic signs (right fourth nerve palsy, hearing loss, hemiparesis, and thalamic pain). Clinicians should recognize the localizing value of this unique constellation of mesencephalic afferent and efferent pupillary defects.

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