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Patient-Reported Outcomes Research in Neuro-Ophthalmology
De Lott, Lindsey B. MD, MS; Ehrlich, Joshua R. MD, MPH
doi : 10.1097/WNO.0000000000001265
June 2021 - Volume 41 - Issue 2 - p 141-146
Long-Term Patient-Reported Outcomes of Visual Field Defects and Compensatory Mechanisms in Patients After Cerebral Hemispherectomy
Meer, Elana A. BA; Chen, Monica F. BA; Jones, Monika JD; Mathern, Gary W. MD; Pineles, Stacy L. MD, MS
doi : 10.1097/WNO.0000000000000998
June 2021 - Volume 41 - Issue 2 - p 147-153
In cases of intractable epilepsy resistant to drug therapy, hemispherectomy is often the only treatment option to mitigate seizures; however, the true long-term subjective visual outcomes are relatively unexplored. In this study, we sought to determine and characterize patient-reported visual function years after hemispherectomy.
Afferent and Efferent Neuro-Ophthalmic Complications of Coronavirus Disease 19
Tisdale, Alanna K. MD, MPH; Dinkin, Marc MD; Chwalisz, Bart K. MD
doi : 10.1097/WNO.0000000000001276
June 2021 - Volume 41 - Issue 2 - p 154-165
To provide a summary of the neuro-ophthalmic manifestations of coronavirus disease 19 (COVID-19) documented in the literature thus far.
SARS-CoV-2 Impairs Vision
Finsterer, Josef MD, PhD; Scorza, Fulvio A. MD; Scorza, Carla A. MD; Fiorini, Ana C. MD
doi : 10.1097/WNO.0000000000001273
June 2021 - Volume 41 - Issue 2 - p 166-169
Affection of the central nervous system and the eyes is increasingly recognized as manifestations of a SARS-CoV-2 infection (COVID-19). This review aims at summarizing and discussing recent advances concerning causes and locations of impaired vision because of an infection with SARS-CoV-2.
New Focus on Endovascular Therapy for Ischemic Stroke
Kelly, Sean MD, PhD; Raz, Eytan MD, PhD; Shapiro, Maksim MD; Ishida, Koto MD
doi : 10.1097/WNO.0000000000001279
June 2021 - Volume 41 - Issue 2 - p 170-175
Over the past 2 decades, a growing number of large-scale clinical trials have helped expand the toolkit for emergency management of acute ischemic stroke. This article is intended to be an up-to-date resource to aid nonstroke specialist neurology providers and ophthalmologists in identifying situations and patient populations in which urgent stroke evaluation should be completed with options for emergent reperfusion therapy considered.
Disorders of the Fourth Cranial Nerve
Kline, Lanning B. MD; Demer, Joseph L. MD; Vaphiades, Michael S. DO; Tavakoli, Mehdi MD
doi : 10.1097/WNO.0000000000001261
June 2021 - Volume 41 - Issue 2 - p 176-193
This review of disorders of the fourth cranial nerve includes discussion on anatomy, examination techniques, congenital and acquired etiologies, differential diagnosis, and management options. The findings of the superior oblique muscle on orbital MRI in patients with fourth nerve palsy have had a major impact on our understanding of this cranial neuropathy. In addition, briefly reviewed are rare disorders of the fourth nerve: superior oblique myokymia, Brown syndrome, and ocular neuromyotonia. It behooves the clinician to have a clear understanding of the role that the fourth cranial nerve plays in a variety of neuro-ophthalmic conditions.
Variation in the Anatomy of the Normal Human Optic Chiasm: An MRI Study
Bosler, Nicholas S. I. BBiomed, BSc, DipL; Ashton, David MBBS, FANZCR; Neely, Andrew J. BEng, MEngSc, PhD, FRAeS; Lueck, Christian J. MB, BChir, MA, PhD, FRACP FRCP(UK), FAAN
doi : 10.1097/WNO.0000000000000907
June 2021 - Volume 41 - Issue 2 - p 194-199
Compression of the optic chiasm typically leads to bitemporal hemianopia. This implies that decussating nasal fibers are selectively affected, but the precise mechanism is unclear. Stress on nasal fibers has been investigated using finite element modeling but requires accurate anatomical data to generate a meaningful output. The precise shape of the chiasm is unclear: A recent photomicrographic study suggested that nasal fibers decussate paracentrally and run parallel to each other in the central arm of an “H.” This study aimed to determine the population variation in chiasmal shape to inform future models.
Prevalence of Optic Disc Drusen in Young Patients With Nonarteritic Anterior Ischemic Optic Neuropathy: A 10-Year Retrospective Study
Fraser, J. Alexander MD; Ruel?kke, Lea L. MD; Malmqvist, Lasse MD, PhD; Hamann, Steffen MD, PhD
doi : 10.1097/WNO.0000000000000974
June 2021 - Volume 41 - Issue 2 - p 200-205
Nonarteritic anterior ischemic optic neuropathy (NAION) in young patients (age ?50) accounts for a minority of all cases of NAION and is more highly associated with crowding of the optic nerves and bilateral involvement than NAION in older patients. Optic disc drusen (ODD) are likewise associated with crowded optic nerves and are located in the prelaminar optic nerve head where they could contribute to NAION pathogenesis. The purpose of this study was to determine the prevalence of ODD in the eyes of young NAION patients using modern imaging methods and to compare it to the baseline 1.8%–2.0% prevalence of ODD in the general population.
Role of Ocular Ultrasonography to Distinguish Papilledema From Pseudopapilledema
Kohli, Anita A. MD; Pistilli, Maxwell MEd, MS; Alfaro, Cesar MD; Ross, Ahmara G. MD, PhD; Jivraj, Imran MD; Bagchi, Sheila MD; Chan, Jessie BS; May, Dionne BS; Liu, Grant T. MD; Shindler, Kenneth S. MD, PhD; Tamhankar, Madhura A. MD
doi : 10.1097/WNO.0000000000000984
June 2021 - Volume 41 - Issue 2 - p 206-211
We prospectively evaluated the sensitivity and specificity of ocular ultrasonography (OUS) to distinguish papilledema from pseudopapilledema.
Use of En Face Optical Coherence Tomography to Monitor Papilledema in Idiopathic Intracranial Hypertension: A Pilot Study
Carey, Andrew R. MD; Bosley, Thomas M. MD; Miller, Neil R. MD; McCulley, Timothy J. MD; Henderson, Amanda D. MD
doi : 10.1097/WNO.0000000000000940
June 2021 - Volume 41 - Issue 2 - p 212-216
En face optical coherence tomography (OCT) uses the data acquired during OCT of the optic disc, which typically is used to determine measurements of the peripapillary retinal nerve fiber layer (ppRNFL), to generate a coronal composite fundus image rather than a cross-sectional image. En face OCT has been reported to identify retinal changes related to papilledema in idiopathic intracranial hypertension (IIH) but has not been evaluated for monitoring papilledema. This study aimed to assess the reliability and validity of en face OCT for monitoring papilledema.
Elevated Intracranial Pressure Associated With Exogenous Hormonal Therapy Used for Gender Affirmation
Nguyen, Huy V. MD; Gilbert, Aubrey L. MD, PhD; Fortin, Elizabeth MD; Vodopivec, Ivana MD, PhD; Torun, Nurhan MD; Chwalisz, Bart K. MD; Cestari, Dean M. MD; Rizzo, Joseph F. III MD
doi : 10.1097/WNO.0000000000000926
June 2021 - Volume 41 - Issue 2 - p 217-223
Addison disease, corticosteroid withdrawal, and taking synthetic growth hormone have been linked with development of intracranial hypertension, but there is still debate on whether administration of other exogenous hormones plays a role in precipitating elevated pressure. The growing use of hormonal therapy for gender affirmation provides an opportunity to explore this possibility.
Complications of Ventriculoperitoneal Shunt for Idiopathic Intracranial Hypertension: A Single-Institution Study of 32 Patients
Brune, Anthony J. DO; Girgla, Taania BS; Trobe, Jonathan D. MD
doi : 10.1097/WNO.0000000000000922
June 2021 - Volume 41 - Issue 2 - p 224-232
Because there are no head-to-head studies of the efficacy of surgical options in the treatment of medically-intractable idiopathic intracranial hypertension (IIH), procedure selection is often based on expected complications. Cerebrospinal fluid (CSF) diversion by shunting has been reported to have a 23%–67% rate of shunt failure. But these figures derive from small cohorts or studies that do not separate the complication rates of the different shunt options—ventriculoperitoneal (VP), lumboperitoneal (LP), and ventriculoatrial (VA). The complication rate of LP shunts seems to be higher than that for VP shunts, the procedure currently in widest use. Our experience with VP shunts for IIH over the past decades suggests that the complication rate for that option is lower than reported series would suggest.
Optic Neuropathy in Charcot–Marie–Tooth Disease
Hamedani, Ali G. MD, MHS; Wilson, James A. BS; Avery, Robert A. DO, MSCE; Scherer, Steven S. MD, PhD
doi : 10.1097/WNO.0000000000000965
June 2021 - Volume 41 - Issue 2 - p 233-238
Charcot–Marie–Tooth disease Type 2A (CMT2A) presents with optic atrophy in a subset of patients, but the prevalence and severity of optic nerve involvement in relation to other CMT subtypes has not been explored.
Automated Pupillometry as an Adjunct to Clinical Examination in Patients With Acute Vision Loss
Bhatnagar, Roshni MD; Birnbaum, Andrea D. MD, PhD; Baqai, Jeanine MD; Volpe, Nicholas J. MD
doi : 10.1097/WNO.0000000000000919
June 2021 - Volume 41 - Issue 2 - p 239-245
Despite automated pupillometry's (AP) improved detection of relative afferent pupillary defects (RAPDs) compared with the Swinging Flashlight Test (SFT), AP remains uncommon in clinical practice. This study examined barriers to routine use of AP in evaluation of acute vision loss.
Trochleaectomy: An Effective Treatment of Trochlear Pain in Monocular Patients
Mettu, Pradeep MD; Mancini, Ronald MD; Friedman, Deborah I. MD, MPH; Garrity, James A. MD
doi : 10.1097/WNO.0000000000000916
June 2021 - Volume 41 - Issue 2 - p 246-250
Conventional treatment options for trochlear pain arising from trochleitis or primary trochlear headache include oral anti-inflammatory medications and/or local injection of corticosteroids and local anesthetic. Trochleaectomy is an additional option to consider for monocular patients with intractable trochlear pain.
Retinal Microvascular Alterations as the Biomarkers for Alzheimer Disease: Are We There Yet?
Jiang, Hong MD, PhD; Wang, Jianhua MD, PhD; Levin, Bonnie E. PhD; Baumel, Bernard S. MD; Camargo, Christian J. MD; Signorile, Joseph F. PhD; Rundek, Tania MD, PhD
doi : 10.1097/WNO.0000000000001140
June 2021 - Volume 41 - Issue 2 - p 251-260
Alzheimer disease (AD) is a heterogeneous and multifactorial disorder with an insidious onset and slowly progressive disease course. To date, there are no effective treatments, but biomarkers for early diagnosis and monitoring of disease progression offer a promising first step in developing and testing potential interventions.
Paraneoplastic Progressive Downbeat Nystagmus, Ataxia and Sensorineural Hearing Loss due to the ANTI-Kelch-11 Protein Antibody
Kattah, Jorge C. MD; Eggers, Scott D. MD; Bach, Sarah E. MD; Dubey, Divyanshu MD; McKeon, Andrew B. MD
doi : 10.1097/WNO.0000000000001194
June 2021 - Volume 41 - Issue 2 - p 261-265
A 45-year-old man with a history of testicular seminoma treated 8 years earlier presented with chronic progressive truncal and limb ataxia, progressive sensorineural hearing loss, and episodic vertigo. Eye movement and neuro-otology examinations showed localizing abnormalities to the bilateral cerebellar flocculus, vermis, and bilateral cerebellar hemispheres. Audiometric testing showed bilateral symmetric sensorineural hearing loss.
Should Immunosuppressive Therapy Be Modified During a Pandemic?
Bouffard, Marc A. MD; Levy, Michael MD; Lee, Andrew G. MD; Van Stavern, Gregory P. MD
doi : 10.1097/WNO.0000000000001274
June 2021 - Volume 41 - Issue 2 - p 266-271
JNO Literature Commentary
Moster, Mark L. MD; Bhatti, M. Tariq MD
doi : 10.1097/WNO.0000000000001267
June 2021 - Volume 41 - Issue 2 - p 272-277
Nonarteritic Anterior Ischemic Optic Neuropathy: Exceptions to the Rules
Vaphiades, Michael S. DO; Al-Sadah, Zakeya M. MD; Kline, Lanning B. MD
doi : 10.1097/WNO.0000000000000949
June 2021 - Volume 41 - Issue 2 - p e139-e141
Acute Visual Loss Related to Retinal Vascular Occlusion Secondary to Visual Pathway Primary Glioblastoma
Mu?oz-Cardona, Marta L. MD; Llano-Naranjo, Yuliana MD; Londo?o-Ocampo, Francisco MD; Preciado-Mesa, Esteban E. MD; Restrepo, Feliza MD; Garc?a, Lina MD; Pineda-Arrieta, Beatriz MD; Lopera-Mar?n, Beatriz MD; Reyes-Botero, Germ?n A. MD
doi : 10.1097/WNO.0000000000000957
June 2021 - Volume 41 - Issue 2 - p e142-e144
Granuloma Annulare, Scalp Necrosis, and Ischemic Optic Neuropathy From Giant Cell Arteritis After Varicella-Zoster Virus Vaccination
Nichani, Prem MSc; Micieli, Jonathan A. MD
doi : 10.1097/WNO.0000000000000947
June 2021 - Volume 41 - Issue 2 - p e145-e148
Giant Cell Arteritis Presenting as Bilateral Optic Perineuritis in an African Man
Emami, Seema MD; Howarth, David MD; Margolin, Edward MD
doi : 10.1097/WNO.0000000000000951
June 2021 - Volume 41 - Issue 2 - p e149-e152
Paracentral Acute Middle Maculopathy as a Manifestation of Giant Cell Arteritis
Ahuja, Abhimanyu S. BS; El-Dairi, Mays A. MD; Hadziahmetovic, Majda MD; Gospe, Sidney M. III MD, PhD
doi : 10.1097/WNO.0000000000001170
June 2021 - Volume 41 - Issue 2 - p e153-e156
Paracentral Acute Middle Maculopathy as the Initial Presentation of Giant Cell Arteritis
Broyles, Heather DO; Chacko, Joseph MD; Chancellor, John MD, MS; LoRusso, Frank MD; Phillips, Paul H. MD; Mashayekhi, Azin MD; Uwaydat, Sami MD
doi : 10.1097/WNO.0000000000001222
June 2021 - Volume 41 - Issue 2 - p e157-e159
Sea Fan Frond Neovascularization: A Unique Finding in a Patient With Susac Syndrome
Mansukhani, Sasha A. MBBS; Zalewski, Nicholas L. MD; Hur, Minjun MD; Ali, Farwa MD; Olsen, Timothy W. MD; Bhatti, M. Tariq MD; Chen, John J. MD, PhD
doi : 10.1097/WNO.0000000000001037
June 2021 - Volume 41 - Issue 2 - p e160-e162
Retinal Angiographic Evidence of Creutzfeldt-Jakob Disease Prion Disease in Humans
Lincoff, Norah S. MD; Balos, Lucia L. MD; Hojnacki, David MD; Farooq, Osman MD
doi : 10.1097/WNO.0000000000000995
June 2021 - Volume 41 - Issue 2 - p e163-e165
Absent Foveal Avascular Zone in Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay
Douglas, Vivian Paraskevi MD, DVM, MBA; Douglas, Konstantinos A. A. MD, DVM, MBA; Miller, John B. MD; Gaier, Eric D. MD, PhD
doi : 10.1097/WNO.0000000000001050
June 2021 - Volume 41 - Issue 2 - p e166-e168
Nutritional Optic Neuropathy From Chronic Omeprazole Use
Rehmani, Ahmad DO; Mehta, Isha DO; Siu, Nga Yan DO; Smith, Edward MD
doi : 10.1097/WNO.0000000000000979
June 2021 - Volume 41 - Issue 2 - p e169-e171
In-Flight G-Induced Retrobulbar Optic Neuropathy in a Fighter Pilot
Taylor, Kenneth R. MD; Chen, Yanjun MD, PhD
doi : 10.1097/WNO.0000000000001015
June 2021 - Volume 41 - Issue 2 - p e172-e173
Bilateral Atypical Retrobulbar Optic Neuritis Associated With Tuberculosis in an Immunocompetent Patient
Fung, Timothy H. M. FRCOphth; Pradeep, Archana FRCOphth
doi : 10.1097/WNO.0000000000001022
June 2021 - Volume 41 - Issue 2 - p e174-e176
Bilateral Optic Neuritis Secondary to Immune Etiology by anti-PD-L1 Antibody
Vicente-Pascual, Mikel MD; Molins-Rojas, Cristina MD; Rosas-Soto, Katiuska MD; Murata-Yonamine, Eleonor P. MD; V?zquez-Justes, Daniel MD; Purroy, Francisco MD, PhD; Traveset-Maeso, Alicia MD
doi : 10.1097/WNO.0000000000001029
June 2021 - Volume 41 - Issue 2 - p e177-e179
Immune-Mediated Optic Neuropathy in Chronically Immunosuppressed Transplant Patients
Kamal, Sarah M. MD; Winters, Jacob M. MD; Al Othman, Bayan A. MD; Kini, Ashwini T. MD; Beaver, Hilary A. MD; Lee, Andrew G. MD
doi : 10.1097/WNO.0000000000001030
June 2021 - Volume 41 - Issue 2 - p e180-e183
Presentation of High-Flow Cerebral Proliferative Angiopathy With Adie's-Like Pupil and Proptosis
Hoang, Tung Thanh MD; Tran, Huy Minh Dinh MD; Van Truong, Ban MD; Subramanian, Prem MD, PhD
doi : 10.1097/WNO.0000000000000946
June 2021 - Volume 41 - Issue 2 - p e184-e185
Horner Syndrome Due To Internal Carotid Artery Dissection With Normal Vascular Imaging: A Radiological Conundrum
Mansukhani, Sasha A. MBBS; Eckel, Laurence J. MD; Wu, Kristi Y. MD; Hassan, Mohamed B. MD; Van Loon, Jon A. MD; Chen, John J. MD, PhD; Bhatti, M. Tariq MD
doi : 10.1097/WNO.0000000000000981
June 2021 - Volume 41 - Issue 2 - p e186-e188
Severe Chronic Progressive External Ophthalmoplegia–Associated Ptosis Successfully Treated With Scleral Lenses
Cherny, Christina BS; Sherman, Suzanne W. OD; Dagi Glass, Lora R. MD
doi : 10.1097/WNO.0000000000000966
June 2021 - Volume 41 - Issue 2 - p e189-e191
Successful Treatment of Superior Oblique Myokymia With Cannabidiol Oil
Ma, Jingyi BMSc; Labbé, Stéphanie DMD; Micieli, Jonathan A. MD, CM
doi : 10.1097/WNO.0000000000000977
June 2021 - Volume 41 - Issue 2 - p e192-e193
Eyelid Closure Downbeat Nystagmus: A Rare Cause of Insomnia
Tipton, Philip W. MD; Dredla, Brynn K. MD; Castillo, Pablo R. MD; Mauricio, Elizabeth A. MD; Lemos, Jo?o MD; Eggenberger, Eric R. DO
doi : 10.1097/WNO.0000000000000982
June 2021 - Volume 41 - Issue 2 - p e194-e196
Acute Convergence and Divergence Paralysis in HIV-Related Rhombencephalitis
Martins, Ana I. MD; Jorge, André F. MD; Galego, Orlando D. MD; Nunes, César P. MD; Gonçalves, Raquel S. MD; Lemos, Jo?o M. MD, PhD
doi : 10.1097/WNO.0000000000001036
June 2021 - Volume 41 - Issue 2 - p e197-e199
Handheld Optical Coherence Tomography in a Young Infant With Albinism and Fovea Plana
Rufai, Sohaib R. BMBS, MRes; Lee, Helena PhD, FRCOphth; Gottlob, Irene MD, FRCOphth
doi : 10.1097/WNO.0000000000001094
June 2021 - Volume 41 - Issue 2 - p e200-e201
We present handheld optical coherence tomography (OCT) diagnosis of Grade 4 foveal hypoplasia (fovea plana) in a 28-day-old infant with albinism. Grade 4 foveal hypoplasia is characterized by the absence of the foveal pit, absence of outer segment lengthening, and absence of outer nuclear layer widening. Binocular visual acuity at 58 months follow-up was 1.2 logarithm of the minimal angle of resolution (logMAR). We describe our handheld OCT acquisition protocol and compare the morphological features with a healthy, age-matched control subject.
Absence of Decussation in Optic Pathway Inflammation in Neuromyelitis Optica and Its Implications for Astrocyte Localization
Harvey, Joshua P. MA (Oxon), BM BCh; Hart, Jonathan MA (Oxon), BM BCh, MRCS, FRCR; Palace, Jacqueline BM, FRCP, DM; O'Sullivan, Eoin P. MD, MRCP, FRCOphth
doi : 10.1097/WNO.0000000000000985
June 2021 - Volume 41 - Issue 2 - p e202-e204
A 39-year-old woman presented with acute visual loss in her right eye. Brain and orbit MRI demonstrated T2 hyperintensity along a long section of her right optic nerve, chiasm, and tract with no evidence of decussation of the inflammation. Subsequent seropositivity for the aquaporin 4 antibody confirmed a diagnosis of neuromyelitis optica. Posterior pathway involvement is typical in neuromyelitis optica and supports the hypothesis that the condition is an astrocytopathy. Furthermore, the absence of decussation in the condition may be a function of astrocyte localization within the chiasm.
Paracentral Acute Middle Maculopathy Mimicking Retrobulbar Optic Neuropathy
Scott, Rachel A. MD; Bhat, Nita MD; Harish Bindiganavile, Shruthi MD; Li, Helen K. MD; Lee, Andrew G. MD
doi : 10.1097/WNO.0000000000001041
June 2021 - Volume 41 - Issue 2 - p e205-e208
A 70-year-old woman presented with acute awareness of decreased color vision in her right eye. Fundus examination and a flash electroretinography (ERG) were both normal. A multifocal ERG (mfERG) however revealed foveal depression, and thinning of inner nuclear layer was noted on macular optical coherence tomography (OCT), and a diagnosis of resolved paracentral acute middle maculopathy was made. Clinicians should be aware of the complementary role of OCT and mfERG in unexplained acute central visual loss to distinguish retinal from neuro-ophthalmic etiologies. Structural ocular imaging with OCT shows features of inner, middle, and outer retinal localizations to the visual loss.
Magnetic Resonance Angiogram Findings of Internal Carotid Artery Narrowing and Anterior Cerebral Artery Hypoplasia Associated With Optic Atrophy After Neonatal Extracorporeal Membrane Oxygenation
Pogrebniak, Alexander E. MD; Marcus, Matthew A. MD
doi : 10.1097/WNO.0000000000001072
June 2021 - Volume 41 - Issue 2 - p e209-e211
We use MRA to elucidate a potential association of unilateral optic atrophy in infancy, ipsilateral internal carotid artery narrowing after extracorporeal membrane oxygenation, and ipsilateral hypoplasia of the A1 segment of the anterior cerebral artery.
Gazing Into the Crystal Ball: Calciphylaxis Causing Striking Retinal Vascular Calcification, Ocular Ischemic Syndrome, Crystalline Retinopathy, and Ischemic Optic Neuropathy
Cherayil, Neena R. MD; Scoles, Drew MD, PhD; Moran, Anna M. MD; Elder, David E. MBChb; Tamhankar, Madhura A. MD
doi : 10.1097/WNO.0000000000001090
June 2021 - Volume 41 - Issue 2 - p e212-e214
A 72-year-old woman with membranous glomerulonephritis and failed renal transplant on peritoneal dialysis presented with bilateral vision loss. She reported several months of diminishing right eye vision that worsened after cataract extraction. On presentation, visual acuity was hand motion in the right and 20/100 in the left eye with a right afferent pupillary defect. Confrontation visual fields were constricted bilaterally.
Bilateral Morning Glory Anomaly With Optic Nerve Multiple Cysts
Zhu, Xuemei MD; Wang, Yi MD; Liang, Jianhong MD
doi : 10.1097/WNO.0000000000001098
June 2021 - Volume 41 - Issue 2 - p e215-e216
We herein describe a pediatric case of bilateral morning glory anomaly whose retrobulbar cysts and renal disease were underdiagnosed, and finally, he was diagnosed with renal coloboma syndrome. We recommend patients with colobomatous optic nerve malformations undergo a complete workup to avoid missed diagnosis, which includes (i) general physical examination looking for heart defects, genitourinary, auricular, and midline facial defects among other abnormalities; (ii) simple nephrologic investigations such as routine urine test and/or renal ultrasound to check for renal hypoplasia; and (iii) neuroimaging check for cerebrovascular anomalies and midline cranial defects. Genetic testing can be a useful tool to guide the workup.
Compressive Optic Disc Edema and Contralateral Papilledema: Type 2 Foster Kennedy Variant Syndrome
Zehden, Jason; Harish Bindiganavile, Shruthi MD; Bhat, Nita MD; Lee, Andrew G. MD
doi : 10.1097/WNO.0000000000001091
June 2021 - Volume 41 - Issue 2 - p e217-e219
A 43-year-old woman presented with progressive anosmia, self-neglect, and lethargy for 1 year. Brain MRI demonstrated a mass that was compressing the optic nerve in the right eye and had a significant mass effect and cerebral edema producing increased intracranial pressure. Examination revealed the patient to have a variant of Foster Kennedy syndrome involving ipsilateral compressive optic neuropathy with superimposed optic disc edema and contralateral papilledema.
Optic Disc Edema and Posterior Globe Flattening Secondary to Ocular Hypotony: Case Report and Discussion Regarding Pathophysiology and Clinical Findings
Albano de Guimar?es, Juliana MD; Teixeira, Gabriela Carneiro MD; Silva, Taiane Kelly Lima da MD; Moura, Frederico Castelo PhD
doi : 10.1097/WNO.0000000000001095
June 2021 - Volume 41 - Issue 2 - p e220-e222
We describe a case of a young female patient presenting with ocular hypotension (4 mm Hg) secondary to cyclodialysis, and optic disc edema (ODE) after a blunt trauma in the right eye (right eye). MRI showed posterior globe flattening of the right eye, drawing our attention to the pathophysiology behind these findings. The combination of ODE and posterior globe flattening, as observed in the present case of ocular hypotony, is known from other conditions such as intracranial hypertension and space-flight neuro-ocular syndrome, pointing to a common pathophysiological mechanism, possibly resulting from axoplasmic stasis at the level of the lamina cribrosa due to a high translaminar pressure difference.
Idiopathic Intracranial Hypertension Triggering Hemifacial Spasm
Garcia, Maria D. MD; Mansukhani, Sasha A. MBBS; Chen, John J. MD, PhD; Bhatti, M. Tariq MD
doi : 10.1097/WNO.0000000000001048
June 2021 - Volume 41 - Issue 2 - p e223-e224
Idiopathic intracranial hypertension (IIH) is a syndrome associated with increased intracranial pressure without a clear underlying cause that is classically seen in young women. Patients typically present with headache and ocular findings, including disc edema and, less frequently, an abduction deficit. To make a diagnosis of IIH, other than cranial nerve 6 or 7 dysfunction, patients must have a normal neurologic examination. When cranial nerve 7 is affected patients can present with hemifacial spasm. We present the case of a young woman with IIH who had hemifacial spasm as one of the presenting symptoms. Her symptoms resolved once she was treated for IIH with acetazolamide.
Hemorrhagic Intracranial Cavernoma Presenting as a Homonymous Horizontal Sectoranopia
Costello, Fiona E. MD, FRCPC; Starreveld, Yves P. MD, PhD, FRCPS
doi : 10.1097/WNO.0000000000001014
June 2021 - Volume 41 - Issue 2 - p e225-e227
Hemorrhagic lateral geniculate nucleus (LGN) insults are rare but have been reported in association with tumors, vascular malformations, and trauma. The localization of LGN lesions is facilitated by recognition of pathognomonic visual field defects. A 21-year old woman developed a sudden onset painless left homonymous horizontal sectoranopia. Magnetic resonance imaging revealed a hemorrhagic cavernous malformation of the right temporal lobe.
Seven-Year Follow-Up of a Patient With a Retained Needlefish Jaw Fragment Abutting Against His Left Internal Carotid Artery
Daigle, Patrick MD, FRCSC; Mehta, Sonul MD; Bhayana, Rajesh MD, FRCPC; Das, Sunit MD, PhD; Bharatha, Aditya MD, FRCPC, DABR; Nijhawan, Navdeep MD, FRCSC, DABO
doi : 10.1097/WNO.0000000000001060
June 2021 - Volume 41 - Issue 2 - p e228-e229
A 51-year-old man presented to the ophthalmology service with binocular diplopia and facial numbness. The patient was returning from a trip to Mexico. He reported having been hit in the left periocular region by a fish while swimming. Local doctors repaired a laceration in the left lateral canthus shortly after the incident. Orbital imaging revealed 2 needle-like foreign bodies corresponding to retained pieces of a needlefish jaw in the left orbit. Given the location of the foreign bodies, observation with repeat imaging was deemed more appropriate than surgical exploration. Subsequent imaging studies showed no migration of the foreign body, and the patient did not suffer from any related complications more than 7 years after the initial injury.
Reversal of Iris Heterochromia in Adult-Onset Acquired Horner Syndrome
Padungkiatsagul, Tanyatuth MD; Leishangthem, Lakshmi MD; Moss, Heather E. MD, PhD
doi : 10.1097/WNO.0000000000001089
June 2021 - Volume 41 - Issue 2 - p e230-e231
Iris heterochromia is typically seen in association with congenital Horner syndrome. A man in his 40s with congenital iris heterochromia, blue in the right and brown in the left, presented with left-sided Horner syndrome. This was associated with recent change in color of his brown left iris to blue similar to the right iris. This case demonstrates a unique case of adult-onset Horner syndrome with reversal of iris heterochromia.
Recurrent Third Nerve Palsy Secondary to Instrinsic Schwannoma of the Third Cranial Nerve
Margolin, Edward MD; Jeeva-Patel, Trishal MD; Al Shafai, Laila MD
doi : 10.1097/WNO.0000000000000994
June 2021 - Volume 41 - Issue 2 - p e232-e233
A 78 year-old woman has experienced multiple episodes of transient right third nerve palsy over the course of 15 years and has undergone multiple imaging studies as well as investigations for myasthenia gravis and giant cell arteritis in search for the diagnosis. When seen after the most recent episode, MRI with contrast and Fast-Imaging Employing Steady-State Acquisition protocol revealed a subtle enlargement and enhancement of the cisternal and proximal cavernous portions of the right third cranial nerve. An empiric diagnosis of schwannoma intrinsic to third cranial nerve was made. All patients with cyclical third nerve palsies should have appropriate neuroimaging to rule out subtle structural lesions before other investigations are undertaken.
Pseudo-Gaze Deviation Resulting From Positive Angle Kappa and Esotropia
Rateaux, Maxence MSc; Espinasse-Berrod, Marie-Andrée MD; Couret, Chloé MD; Bremond-Gignac, Dominique MD, PhD; Robert, Matthieu P. MD, PhD
doi : 10.1097/WNO.0000000000001068
June 2021 - Volume 41 - Issue 2 - p e234-e236
A 6-year-old boy was referred for constant right gaze deviation. Rather than a gaze deviation, he constantly seemed to look on the left side of any displayed target. Examination revealed the association of a highly positive angle Kappa and an esotropia of equal values. He also exhibited signs of ocular albinism with no associated infantile nystagmus syndrome. The X-linked ocular albinism was confirmed genetically, explaining the presence of a positive angle Kappa. A highly positive angle Kappa can be associated with a convergent strabismus; in case both values offset each other, this can result in a constant “sidelooking,” which should not be confused with a gaze deviation.
Horizontal Gaze Palsy, Scoliosis, and Split Pons Sign in a 6-Year-Old Girl
Singanamalla, Bhanudeep MD; Chaurasia, Shweta MS; Jain, Chirag DM; Bhatia, Vikas DM; Sharma, Neeraj MD; Madaan, Priyanka DM; Gopinathan, Nirmal Raj MS; Saini, Lokesh DM
doi : 10.1097/WNO.0000000000001131
June 2021 - Volume 41 - Issue 2 - p e237-e238
A 6-year-old girl presented with complaints of absent horizontal eye movements since birth. There was also associated progressive scoliosis for past 1 year. Neuroimaging revealed split pons sign, butterfly-shaped medulla, and prominent inferior olivary nuclei. The presence of congenital horizontal gaze palsy, childhood onset progressive scoliosis, and abnormal neuroimaging findings confirmed the diagnosis of horizontal gaze palsy with progressive scoliosis. This case highlights the importance of neuroimaging in a child presenting with horizontal gaze palsy and scoliosis that helped for starting early rehabilitation of the child, prevention of permanent vision loss, and parental counseling for future pregnancies.
The 47th Annual Meeting of the North American Neuro-Ophthalmology Society Occurring Virtually
Seay, Meagan D. DO; Digre, Kathleen B. MD
doi : 10.1097/WNO.0000000000001266
June 2021 - Volume 41 - Issue 2 - p e239-e241
The American Headache Society Hosted the 2020 Virtual Scottsdale Headache Symposium
Digre, Kathleen B. MD
doi : 10.1097/WNO.0000000000001207
June 2021 - Volume 41 - Issue 2 - p e242
Neuro-Ophthalmology at American Academy of Ophthalmology 2020 Virtual
Subramanian, Prem S.
doi : 10.1097/WNO.0000000000001209
June 2021 - Volume 41 - Issue 2 - p e243
Oculomotor Nerve Palsy Due to Unusual Causes
Tian, Guohong MD, PhD; Kardon, Randy MD, PhD; Feng, Chaoyi MD, PhD; Hong, Rujian MD; Sha, Yan MD, PhD; Sun, Xinghuai MD, PhD; Wang, Min MD, PhD
doi : 10.1097/WNO.0000000000001032
June 2021 - Volume 41 - Issue 2 - p e244-e250
An isolated oculomotor nerve (CN III) palsy is a diagnostic concern because of the potential for serious morbidity or life-threatening causes. We present 5 unusual causes of oculomotor nerve palsy that escaped initial diagnosis in order to raise awareness of their associated features that will facilitate correct diagnosis.
In Memoriam: James Finbarr (Barry) Cullen
Tow, Sharon L. C. MBBS, FRCS(Ed), FAMS; McIlwaine, Gawn G. FRCOphth; Miller, Neil R. MD, FACS
doi : 10.1097/WNO.0000000000001244
June 2021 - Volume 41 - Issue 2 - p e251-e253
Vision Loss From Giant Cell Arteritis in Patients With Other Ocular Diagnoses
Nichani, Prem; Biousse, Valérie MD; Newman, Nancy J. MD; Micieli, Jonathan A. MD
doi : 10.1097/WNO.0000000000001064
June 2021 - Volume 41 - Issue 2 - p e254-e258
Vision problems from giant cell arteritis (GCA) can be difficult to diagnose as patients may present with vision loss in the absence of systemic symptoms, have other comorbidities that affect inflammatory blood markers, or have other ocular diagnoses. We present 3 cases illustrating this point including a patient with advanced glaucoma with worsening vision from posterior ischemic optic neuropathy from GCA, a patient with arteritic anterior ischemic optic neuropathy (AAION) erroneously diagnosed as optic neuritis without elevated inflammatory blood markers due to corticosteroid use, and a patient with AAION and a history of nonarteritic anterior ischemic optic neuropathy in her fellow eye and untreated obstructive sleep apnea. GCA should be kept in the differential diagnosis for patients over 50 years of age even if they carry other ocular diagnoses. Temporal artery biopsy remains the gold standard for GCA diagnosis and is often required in equivocal cases.
Neurosarcoidosis and Ocular Inflammation: A Case Series and Literature Review
Desai, Aishwary BS; Chaon, Benjamin MD; Berkenstock, Meghan MD
doi : 10.1097/WNO.0000000000001117
June 2021 - Volume 41 - Issue 2 - p e259-e266
To describe the ocular findings of neurosarcoidosis (NS) through a case series and review the current literature on the ocular complications of NS.
Pituitary Macroadenoma Presenting With Monocular Temporal Hemianopia
Gupta, Ankur MA, FRCOphth; Deshmukh, Mira BSc, MBBS; Palexas, George MBBCh, MMed
doi : 10.1097/WNO.0000000000001119
June 2021 - Volume 41 - Issue 2 - p e267-e268
A 35-year-old primigravida woman presented to the eye emergency department with reduced visual acuity in the right eye. Humphrey visual field testing showed a monocular right eye temporal hemianopia before delivery. An MRI after delivery revealed a largely symmetrical pituitary macroadenoma with chiasmal compression. This is a rare presentation of a pituitary macroadenoma especially when the tumor is largely symmetrical.
Double Vision and Headache
Frohman, Larry P. MD
doi : 10.1097/WNO.0000000000001253
June 2021 - Volume 41 - Issue 2 - p e269
Case of Progressively Worsening Vertical Nystagmus
Panneerselvam, Sugi; Raviskanthan, Subahari MBBS; Lee, Andrew G. MD
doi : 10.1097/WNO.0000000000001254
June 2021 - Volume 41 - Issue 2 - p e270
Fung T, Pradeep A. Bilateral Atypical Retrobulbar Optic Neuritis Associated With Tuberculosis in an Immunocompetent Patient: Response
Patil, Ajay D. MA (Cantab), MBBS
doi : 10.1097/WNO.0000000000001185
June 2021 - Volume 41 - Issue 2 - p e271
Bilateral Atypical Retrobulbar Optic Neuritis Associated With Tuberculosis in an Immunocompetent Patient: Response
Fung, Timothy H. M. FRCOphth; Pradeep, Archana FRCOphth
doi : 10.1097/WNO.0000000000001186
June 2021 - Volume 41 - Issue 2 - p e272
Idiopathic Intracranial Hypertension and Anemia: A Matched Case–Control Study
Plant, Gordon T. MD; Wong, Sui H. MBBS, MD, MA; Sundholm, Anna MD, PhD; Mollan, Susan P. MBBS
doi : 10.1097/WNO.0000000000001183
June 2021 - Volume 41 - Issue 2 - p e272-e273
Idiopathic Intracranial Hypertension and Anemia: A Matched Case–Control Study: Response
Lin, Weijie V. MD; Berry, Shauna E. DO; Nakawah, Mohammad O. MD; Sadaka, Ama MD; Lee, Andrew G. MD
doi : 10.1097/WNO.0000000000001184
June 2021 - Volume 41 - Issue 2 - p e273-e274
Neuro-Ophthalmic Features of Autoimmune Encephalitides: Letter to the Editor
Vaphiades, Michael S. DO
doi : 10.1097/WNO.0000000000001189
June 2021 - Volume 41 - Issue 2 - p e275
Idiopathic Intracranial Hypertension and Anemia: A Matched Case-Control Study: Erratum
doi : 10.1097/WNO.0000000000001190
June 2021 - Volume 41 - Issue 2 - p e276
Acute Care Treatment of Migraine: Erratum
doi : 10.1097/WNO.0000000000001191
June 2021 - Volume 41 - Issue 2 - p e276
Isolated Cranial Nerve 6 Palsy in 2 Patients With COVID-19 Infection: Erratum
doi : 10.1097/WNO.0000000000001213
June 2021 - Volume 41 - Issue 2 - p e276
