Publications by authors named "Heather E Moss"

89 Publications

Head-Down Tilt Bed Rest Studies as a Terrestrial Analog for Spaceflight Associated Neuro-Ocular Syndrome.

Front Neurol 2021 26;12:648958. Epub 2021 Mar 26.

Departments of Ophthalmology, Stanford University, Palo Alto, CA, United States.

Astronauts who undergo prolonged periods of spaceflight may develop a unique constellation of neuro-ocular findings termed Spaceflight Associated Neuro-Ocular Syndrome (SANS). SANS is a disorder that is unique to spaceflight and has no terrestrial equivalent. The prevalence of SANS increases with increasing spaceflight duration and although there have been residual, structural, ocular changes noted, no irreversible or permanent visual loss has occurred after SANS, with the longest spaceflight to date being 14 months. These microgravity-induced findings are being actively investigated by the United States' National Aeronautics Space Administration (NASA) and SANS is a potential obstacle to future longer duration, manned, deep space flight missions. The pathophysiology of SANS remains incompletely understood but continues to be a subject of intense study by NASA and others. The study of SANS is of course partially limited by the small sample size of humans undergoing spaceflight. Therefore, identifying a terrestrial experimental model of SANS is imperative to facilitate its study and for testing of preventative measures and treatments. Head-down tilt bed rest (HDTBR) on Earth has emerged as one promising possibility. In this paper, we review the HDTBR as an analog for SANS pathogenesis; the clinical and imaging overlap between SANS and HDTBR studies; and potential SANS countermeasures that have been or could be tested with HDTBR.
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http://dx.doi.org/10.3389/fneur.2021.648958DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8032981PMC
March 2021

Functional Vision Disorders in adults: A paradigm and nomenclature shift for ophthalmology.

Surv Ophthalmol 2021 Mar 15. Epub 2021 Mar 15.

Department of Ophthalmology, Blanton Eye Institute, Houston Methodist Hospital, Houston, TX, USA; Departments of Ophthalmology, Neurology, and Neurosurgery, Weill Cornell Medicine, New York, New York, USA; Department of Ophthalmology, University of Texas Medical Branch, Galveston, Texas; University of Texas MD Anderson Cancer Center, Houston, Texas, USA; Texas A and M College of Medicine, Bryan, Texas, USA; Department of Ophthalmology, The University of Iowa Hospitals and Clinics, Iowa City, Iowa. Electronic address:

Vision loss with clinical findings that are incompatible with the symptoms and recognized neurological or ophthalmic conditions is a common presentation of patients to neurologists, ophthalmologists, and neuro-ophthalmologists. The accepted terminology to describe such patients has evolved over time including functional visual disorder (FVD), non-organic vision loss, non-physiologic vision loss, functional vision loss, psychogenic, psychosomatic, and medically unexplained visual loss. Likewise, attitudes and recommended management options have changed over the years in the fields of psychiatry and neurology. FVD is a diagnosis of inclusion, and it is critical that the diagnosis be made and delivered efficiently and effectively to reduce patient and physician duress. We review the current Diagnostic and Statistical Manual (DSM V) terminology and the prior literature on FVD and describe how the approaches to diagnosis and management have changed. We provide recommendations on the appropriate techniques and diagnostic approach for patients with FVD. We also propose a protocol for consistent and standardized discussion with the patient of the diagnosis of FVD. We believe that the adoption of FVD as both a paradigm and nomenclature shift in ophthalmology will improve patient care.
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http://dx.doi.org/10.1016/j.survophthal.2021.03.002DOI Listing
March 2021

Development and Implementation of a Handheld Pupillometer for Detection of Optic Neuropathies.

Curr Eye Res 2021 Feb 5:1-4. Epub 2021 Feb 5.

Department of Ophthalmology, Stanford University , Palo Alto, USA.

: Quantitative pupillometry has utility in research settings for measuring optic nerve and autonomic function. We configured a portable device to perform quantitative pupillometry with application to detecting unilateral optic neuropathies in the clinical setting. : Light stimuli were delivered, and pupil diameter responses recorded using customized software implemented on a commercial portable electroretinography device. Increasing pupillary constriction occurred with increasing duration and intensity of full field blue light (470 nm) stimuli in healthy subjects. Flashes of 1 s dim (50 cd/m) and bright (316 cd/m) blue light were administered to both eyes of subjects with unilateral optic neuropathies (n = 10) and controls (n = 5). Maximum pupillary constriction (C) for each stimulus was compared between control eyes and optic neuropathy eyes. C for the inter-eye difference curve (C) was compared between control and optic neuropathy subjects. : The pupil protocol lasted 15 minutes and was well tolerated by subjects. C for bright and dim stimuli was reduced in eyes with optic neuropathy compared to fellow and control eyes ( < .0005 for all). Inter-eye C was larger in optic neuropathy subjects than control subjects for both dim and bright stimuli ( = .002, <0.0005). There was no overlap between groups for C and C for either stimulus. : A portable pupillometer was implemented on a commercial portable electroretinography platform and applied in a pilot manner to subjects with and without unilateral optic neuropathies. Optic neuropathy eyes were distinguished from non-optic neuropathy eyes both within and between subjects.
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http://dx.doi.org/10.1080/02713683.2021.1878542DOI Listing
February 2021

Numerical Investigation on the Role of Mechanical Factors Contributing to Globe Flattening in States of Elevated Intracranial Pressure.

Life (Basel) 2020 Nov 28;10(12). Epub 2020 Nov 28.

Computational Biomechanics Research Laboratory, Mechanical and Industrial Engineering Department, University of Illinois at Chicago, Chicago, IL 60612, USA.

Flattening of the posterior eye globe in the magnetic resonance (MR) images is a sign associated with elevated intracranial pressure (ICP), often seen in people with idiopathic intracranial hypertension (IIH). The exact underlying mechanisms of globe flattening (GF) are not fully known but mechanical factors are believed to play a role. In the present study, we investigated the effects of material properties and pressure loads on GF. For this purpose, we used a generic finite element model to investigate the deformation of the posterior eyeball. The degree of GF in numerical models and the significance of different mechanical factors on GF were characterized using an automated angle-slope technique and a statistical measure. From the numerical models, we found that ICP had the most important role in GF. We also showed that the angle-slope graphs pertaining to MR images from five people with high ICP can be represented numerically by manipulating the parameters of the finite element model. This numerical study suggests that GF observed in IIH patients can be accounted for by the forces caused by elevation of ICP from its normal level, while material properties of ocular tissues, such as sclera (SC), peripapillary sclera (PSC), and optic nerve (ON), would impact its severity.
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http://dx.doi.org/10.3390/life10120316DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7760332PMC
November 2020

Predictive model of ischemic optic neuropathy in spinal fusion surgery using a longitudinal medical claims database.

Spine J 2021 Mar 26;21(3):377-386. Epub 2020 Nov 26.

Department of Anesthesiology, University of Illinois, Chicago, IL, USA.

Background Context: Perioperative ischemic optic neuropathy (ION) is a devastating complication of spinal fusion surgery.

Purpose: To develop predictive models of this blinding condition using a longitudinal medical administrative claims database, which provides temporal sequence of perioperative ischemic optic neuropathy and potential risk factors.

Design: Nested case control study.

Patient Sample: Participants in Cliniformatics Data Mart medical claims database (2007-2017) with hospitalization involving lumbar or thoracic spinal fusion surgery and no history of ION.

Outcome Measures: Perioperative ION (or not) during hospitalization for lumbar or thoracic spinal fusion surgery.

Methods: Sixty-five ION cases and 106,871 controls were identified. Matched controls (n=211) were selected based on year of surgery and zip code. Chronic and perioperative variables were assigned based on medical claims codes. Least absolute shrinkage and selection (LASSO) penalized conditional logistic regression with 10-fold cross validation was used to select variables for the optimal predictive model from the subset of variables with p<.15 between cases and matched controls (unadjusted conditional logistic regression). Receiver operating characteristic (ROC) curves were generated for the strata-independent matched and full sample.

Results: The predictive model included age 57-65 years, male gender, diabetes with and without complications, chronic anemia, hypertension, heart failure, carotid stenosis, perioperative hemorrhage and perioperative organ damage. Area under ROC curve was 0.75 (95% confidence interval [CI]: 0.68, 0.82) for the matched sample and 0.72 (95% CI: 0.66, 0.78) for the full sample.

Conclusions: This predictive model for ION in spine fusion considering chronic conditions and perioperative conditions is unique to date in its use of longitudinal medical claims data, inclusion of International Classification of Disease-10 codes and study of ophthalmic conditions as risk factors. Similar to other studies of this condition the multivariable model included age, male gender, perioperative organ damage and perioperative hemorrhage. Hypertension, chronic anemia and carotid artery stenosis were new predictive factors identified by this study.
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http://dx.doi.org/10.1016/j.spinee.2020.11.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7887045PMC
March 2021

MRI findings as markers of idiopathic intracranial hypertension.

Curr Opin Neurol 2021 Feb;34(1):75-83

Departments of Ophthalmology, Neurology and Neurosciences, Stanford University, Palo Alto, California, USA.

Purpose Of Review: Negative findings on neuroimaging are part of the diagnostic criteria for idiopathic intracranial hypertension (IIH), a syndrome characterized by increased intracranial pressure (ICP). Some positive neuroimaging findings are associated with increased ICP, but their role in diagnosis of IIH has not been established. We provide an overview of these findings and their relevance for diagnosis of raised intracranial pressure.

Recent Findings: MRI acquisition techniques have significantly improved in the last few decades leading to better characterization of the intracranial changes associated with IIH, including empty sella turcica, optic nerve tortuosity, distension of the optic nerve sheath, posterior globe flattening, slit-like ventricles, and venous sinus stenosis. These may be MRI biomarkers of increased ICP. Prevalence difference between people with and without increased ICP, and reversibility of these MRI findings following treatment of increased ICP inform evaluation of their diagnostic potential.

Summary: MRI and magnetic resonance venography findings are important tools in the diagnosis of IIH. Empty sella turcica, optic nerve protrusion, distension of the optic nerve sheath, optic nerve tortuosity, posterior globe flattening, and transverse sinus stenosis have been found to be the most promising diagnostic markers for IIH, although absence of these findings does not rule out the diagnosis.
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http://dx.doi.org/10.1097/WCO.0000000000000885DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7856277PMC
February 2021

Telemedicine for neuro-ophthalmology: challenges and opportunities.

Curr Opin Neurol 2021 02;34(1):61-66

Departments of Ophthalmology, Neurology and Neurological Sciences, Stanford University, Stanford, California, USA.

Purpose Of Review: Telemedicine for neuro-ophthalmology (tele-neuro-ophthalmology) has the potential to increase access to neuro-ophthalmic care by improving efficiency and decreasing the need for long-distance travel for patients. Requirements for decreased person-to-person contacts during the COVID-19 pandemic accelerated adoption of tele-neuro-ophthalmology. This review highlights the challenges and opportunities with tele-neuro-ophthalmology.

Recent Findings: Tele-neuro-ophthalmology programs can be used for triage, diagnostic consultation, and long-term treatment monitoring. Formats include telephone appointments, interprofessional collaborations, remote data interpretation, online asynchronous patient communication, and video visits. Barriers to long-term implementation of tele-neuro-ophthalmology arise from data quality, patient engagement, workflow integration, state and federal regulations, and reimbursement. General neurologists may collaborate with local eye care providers for ophthalmic examination, imaging, and testing to facilitate efficient and effective tele-neuro-ophthalmology consultation.

Summary: Tele-neuro-ophthalmology has tremendous potential to improve patient access to high-quality cost-effective neuro-ophthalmic care. However, many factors may impact its long-term sustainability.
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http://dx.doi.org/10.1097/WCO.0000000000000880DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7856022PMC
February 2021

Validity of International Classification of Diseases Codes for Identifying Neuro-Ophthalmic Disease in Large Data Sets: A Systematic Review.

J Neuroophthalmol 2020 12;40(4):514-519

Department of Neurology (AGH), Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Department of Neurology and Ophthalmology & Visual Sciences (LBDL, TD), University of Michigan, Ann Arbor, Michigan; and Department of Ophthalmology and Neurology & Neurological Sciences (HEM), Stanford University, Palo Alto, California.

Background: Administrative health claims data have been used for research in neuro-ophthalmology, but the validity of International Classification of Diseases (ICD) codes for identifying neuro-ophthalmic conditions is unclear.

Evidence Acquisition: We performed a systematic literature review to assess the validity of administrative claims data for identifying patients with neuro-ophthalmic disorders. Two reviewers independently reviewed all eligible full-length articles and used a standardized abstraction form to identify ICD code-based definitions for 9 neuro-ophthalmic conditions and their sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). A quality assessment of eligible studies was also performed.

Results: Eleven articles that met criteria for inclusion are as follows: 3 studies of idiopathic intracranial hypertension (PPV 54%-91% and NPV 74%-85%), 2 studies of giant cell arteritis (sensitivity 30%-96% and PPV 94%), 3 studies of optic neuritis (sensitivity 76%-99%, specificity 83%-100%, PPV 25%-100%, and NPV 98%-100%), 1 study of neuromyelitis optica (sensitivity 60%, specificity 100%, PPV 43%-100%, and NPV 98%-100%), 1 study of ocular motor cranial neuropathies (PPV 98%-99%), and 2 studies of myasthenia gravis (sensitivity 53%-97%, specificity 99%-100%, PPV 5%-90%, and NPV 100%). No studies met eligibility criteria for nonarteritic ischemic optic neuropathy, thyroid eye disease, and blepharospasm. Approximately 45.5% provided only one measure of diagnostic accuracy. Complete information about the validation cohorts, inclusion/exclusion criteria, data collection methods, and expertise of those reviewing charts for diagnostic accuracy was missing in 90.9%, 72.7%, 81.8%, and 36.4% of studies, respectively.

Conclusions: Few studies have reported the validity of ICD codes for neuro-ophthalmic conditions. The range of diagnostic accuracy for some disorders and study quality varied widely. This should be taken into consideration when interpreting studies of neuro-ophthalmic conditions using administrative claims data.
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http://dx.doi.org/10.1097/WNO.0000000000000971DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7678809PMC
December 2020

Reversal of Iris Heterochromia in Adult-Onset Acquired Horner Syndrome.

J Neuroophthalmol 2020 Sep 11. Epub 2020 Sep 11.

Department of Ophthalmology (TP), Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; Department of Ophthalmology (TP, LL, HEM), Stanford University, Palo Alto, California; and Department of Neurology and Neurological Sciences (HEM), Stanford University, Palo Alto, California.

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.
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http://dx.doi.org/10.1097/WNO.0000000000001089DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7947023PMC
September 2020

Retinal Diseases that Can Masquerade as Neurological Causes of Vision Loss.

Curr Neurol Neurosci Rep 2020 Sep 15;20(11):51. Epub 2020 Sep 15.

Department of Ophthalmology, Stanford University, 2370 Watson Court, Suite 200, Palo Alto, CA, 94303, USA.

Purpose Of Review: This review aims to discuss retinal diseases that may masquerade as neurological causes of vision loss and highlights modern ophthalmic ancillary testing that can help to establish these diagnoses.

Recent Findings: Retinal diseases with signs and symptoms overlapping with neurological causes of vision loss include central serous chorioretinopathy, retinal ischemia, acute macular neuroretinopathy, Acute zonal occult outer retinopathy (AZOOR) complex diseases, paraneoplastic retinopathy, retinal dystrophy, and toxic retinopathy. Diagnosis is facilitated by electrophysiologic studies and multimodal ophthalmic imaging including optical coherence tomography and fundus autofluorescence imaging. Looking into the future, translation of adaptive optics ophthalmoscopy into clinical practice may facilitate early detection of microscopic retinal abnormalities that characterize these conditions. With conventional methods of physical examination, diagnosis of retinal diseases that may masquerade as neurological causes of vision loss can be challenging. Current advance in multimodal ophthalmic imaging along with electrophysiologic studies enhances the provider's ability to make early diagnosis and monitor progression of these conditions.
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http://dx.doi.org/10.1007/s11910-020-01071-1DOI Listing
September 2020

Ultra-widefield Fundus Image in Oculocutaneous Albinism.

JAMA Ophthalmol 2020 09 10;138(9):e200864. Epub 2020 Sep 10.

Department of Ophthalmology, Stanford University, Palo Alto, California.

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http://dx.doi.org/10.1001/jamaophthalmol.2020.0864DOI Listing
September 2020

Higher Cortical Dysfunction Presenting as Visual Symptoms in Neurodegenerative Diseases.

Front Neurol 2020 31;11:679. Epub 2020 Jul 31.

Department of Ophthalmology, Stanford University, Palo Alto, CA, United States.

As the population ages, increasing prevalence of neurodegenerative diseases will have profound implications for the health care system. Recognizing visual symptoms from neurodegenerative diseases can be challenging, especially in the presence of co-existing eye diseases. A seven-question survey was completed by attendees at the "neurodegenerative diseases in neuro-ophthalmology" symposium during the 2017 North American Neuro-ophthalmology Society annual meeting using a web-based audience response system. Content included demographics, patient prevalence, and perceived barriers. Fifty-five practicing neuro-ophthalmologists (thirty-three ophthalmology-trained, twenty-two neurology-trained) participated in the survey. Twenty (36%) had <5 years of experience, and 19 (32%) had >15 years of experience. Forty-one (75%) reported seeing patients more than five half-day/week. Thirty (55%) reported that at least 1 of 10 or 1 of 20 new patients referred have a prior diagnosis of a neurodegenerative disease. Twenty-one (40%) of the respondents reported attributing visual complaints to higher order effects in at least 25% of patients with a prior diagnosis of neurodegenerative disease vs. five (9%) without a prior diagnosis. For those diagnosed with neurodegenerative disease by the neuro-ophthalmologist, reasons for referral were unknown cause of visual symptom (56%), to confirm diagnosis and/or treat visual complaint due to neurodegeneration (29%), and functional disorder (5%). Perceived barriers to diagnosing visual dysfunction due to neurodegenerative disease included difficulty making a referral to neuropsychologists or behavioral neurologists (73%), lack of time for in-depth assessment (62%), lack of tools to assess visual dysfunction due to neurodegenerative disease (40%), and lack of knowledge about presenting signs and symptoms (31%). Visual symptoms from neurodegenerative disease in patients with and without prior diagnoses of neurodegenerative disease are evaluated by neuro-ophthalmologists. Lack of time, resources, and knowledge are barriers to diagnosis. A larger study is warranted to guide programs to improve diagnosis of visual consequences of neurodegenerative disease.
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http://dx.doi.org/10.3389/fneur.2020.00679DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7438888PMC
July 2020

Differences in Clinical Features of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Optic Neuritis in White and Asian Race.

Am J Ophthalmol 2020 11 15;219:332-340. Epub 2020 Jul 15.

Department of Ophthalmology, Stanford University, Palo Alto, California, USA; Department of Neurology and Neurological Sciences, Stanford University, Palo Alto, California, USA. Electronic address:

Purpose: To determine whether clinical features and visual outcomes of myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-ON) differ between White and Asian subjects.

Design: Multicenter retrospective cohort.

Methods: This was a multicenter study of 153 subjects who were White or Asian with a history of adult-onset (age 18 years or older) optic neuritis (ON) and positive MOG-IgG serology by cell-based assay. Subjects were enrolled from 2 unpublished cohorts (January 2017-November 2019) and 9 published cohorts with case-level data available (2012-2018). Subjects with alternative etiologies of demyelinating disease and positive or lack of aquaporin-4-IgG serology result were excluded. The main outcome measurements were clinical features and final visual outcomes.

Results: Of the 153 subjects who were White (n = 80) or Asian (n = 73) included in the study, 93 (61%) were women, mean age of onset was 40.8 ± 14.9 years, and median follow-up was 35.2 months (range: 1-432 months); all of these characteristics were similar between White and Asian subjects. White subjects were more likely to have recurrent ON (57 [71%] vs 20 [27%]; P = .001) and extra-optic nerve manifestations (35 [44%] vs 8 [11%]; P = .001). Optic disc swelling, neuroimaging findings, presenting visual acuity (VA), treatment, and final VA did not differ according to subjects' race. Despite the high prevalence of severe visual loss (<20/200) during nadir, most subjects had good recovery of VA (>20/40) at final examination (51/77 [66%] White subjects vs 52/70 [74%] Asian subjects).

Conclusion: White subjects with MOG-ON were more likely to have recurrent disease and extra-optic nerve manifestations. Visual outcomes were similar between White and Asian subjects.
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http://dx.doi.org/10.1016/j.ajo.2020.07.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7713508PMC
November 2020

Survey of Telehealth Adoption by Neuro-ophthalmologists During the COVID-19 Pandemic: Benefits, Barriers, and Utility.

J Neuroophthalmol 2020 09;40(3):346-355

Departments of Ophthalmology (HEM) and Neurology & Neurological Sciences (HEM), Stanford University, Palo Alto, California; Department of Ophthalmology (KEL, MWK), Indiana University School of Medicine, Indianapolis, Indiana; Ophthalmology Service (KEL), Richard L. Roudebush Veterans Administration Medical Center, Indianapolis, Indiana; and Departments of Neurology and Neurosurgery (MWK), Indiana University School of Medicine, Indianapolis, Indiana.

Background: During the COVID-19 pandemic, telehealth modalities have come to prominence as a strategy for providing patient care when in-person care provision opportunities are limited. The degree of adoption by neuro-ophthalmologists has not been quantified.

Methods: Telehealth utilization pre-COVID-19 and peri-COVID-19 was surveyed among practicing neuro-ophthalmologists in and outside the United States using an online platform. Demographics, perceived benefits, barriers, and utility for different neuro-ophthalmic conditions were collected. Data collection occurred over a 2-week period in May 2020.

Results: Two hundred eight practicing neuro-ophthalmologists (81.3% United States, 50.2% females, age range <35 to >65, mode 35-44 years) participated in the survey. Utilization of all telehealth modalities increased from pre-COVID to peri-COVID (video visit 3.9%-68.3%, P < 0.0005, remote interpretation of testing 26.7%-32.2%, P = 0.09, online second opinion 7.9%-15.3%, P = 0.001, and interprofessional e-consult 4.4%-18.7%, P < 0.0005, McNemar). The majority selected access, continuity, and patient efficiency of care as benefits and data quality as a barrier. Telehealth was felt to be most helpful for conditions relying on history, external examination, and previously collected ancillary testing and not helpful for conditions requiring funduscopic examination.

Conclusions: Telehealth modality usage by neuro-ophthalmologists increased during the COVID-19 pandemic. Identified benefits have relevance both during and beyond COVID-19. Further work is needed to address barriers in their current and future states to maintain these modalities as viable care delivery options.
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http://dx.doi.org/10.1097/WNO.0000000000001051DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7382419PMC
September 2020

A tearfully painful darkness.

Surv Ophthalmol 2021 May-Jun;66(3):543-549. Epub 2020 Jun 12.

Departments of Ophthalmology and Neurology, Mayo Clinic, Rochester, Minnesota, USA.

A 70-year-old woman presented with new onset of left eye and facial pain. Ophthalmic and neurological examinations, magnetic resonance imaging brain, erythrocyte sedimentation rate, and C-reactive protein were unrevealing. A few days later, she developed vision loss in her left eye. Examination revealed decreased visual acuity with a relative afferent pupillary defect in the left eye and a diffuse mild swelling of the left optic nerve head. Repeat magnetic resonance imaging showed T2 hyperintensity and enhancement of the intraorbital optic nerve and surrounding tissues with no other intracranial abnormalities. Serum studies showed elevated myelin oligodendrocyte glycoprotein IgG titer. She was treated with IV methylprednisolone 1000 mg daily for 3 days and was discharged on prolonged prednisone taper with return of vision to baseline.
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http://dx.doi.org/10.1016/j.survophthal.2020.06.002DOI Listing
June 2020

Optical coherence tomography use in idiopathic intracranial hypertension.

Ann Eye Sci 2020 Mar 15;5. Epub 2020 Mar 15.

Department of Ophthalmology, Stanford University, Palo Alto, CA, USA.

Idiopathic intracranial hypertension (IIH) is a condition in which elevated pressure in the cerebrospinal fluid can lead to optic nerve head (ONH) dysfunction and subsequent visual impairment. Physicians are currently limited in their ability to monitor and manage this condition, as clinical symptoms and exam findings are often delayed in response to changes in intracranial pressure. In order to find other biomarkers of disease, researchers are using imaging modalities such as optical coherence tomography (OCT) to observe microscopic changes in the eye in this condition. OCT can create 2-dimensional and 3-dimensional high definition images of the retina of the ONH and has been used to study various conditions such as glaucoma and multiple sclerosis. Numerous studies have used OCT in IIH as well, and they have shown that certain retinal layers and the ONH change in thickness and shape in both the short and long term with intracranial pressure changes. OCT is a promising modality for clinical and scientific evaluation of IIH as it is a noninvasive and practical tool to obtain in depth images. This review will discuss how OCT can be used to assess a patient with IIH, both before and after treatment, along with its limitations and future applications.
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http://dx.doi.org/10.21037/aes.2019.12.06DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7220123PMC
March 2020

The Case-Control Study in Neuro-Ophthalmology.

J Neuroophthalmol 2020 06;40(2):144-147

Department of Neurology (AGH), Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Translational Center of Excellence for Neuroepidemiology and Neurology Outcomes Research (AGH), University of Pennsylvania, Philadephia, Pennsylvania; Center for Clinical Epidemiology and Biostatistics (AGH), University of Pennsylvania, Philadelphia, Pennsylvania; Department of Ophthalmology (SLP), University of California, Los Angeles, California; Department of Ophthalmology (HEM), Stanford University, Palo Alto, California; Department of Neurology and Neurological Sciences (HEM), Stanford University, Palo Alto, California.

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http://dx.doi.org/10.1097/WNO.0000000000000970DOI Listing
June 2020

Evolution of the Journal of Neuro-Ophthalmology and the Clinical Ophthalmology Literature: A 20-Year Retrospective.

J Neuroophthalmol 2020 06;40(2):141-143

Department of Ophthalmology (KB), University of Cincinnati, Cincinnati, Ohio; Departments of Ophthalmology and Visual Sciences (CEJ) and Epidemiology (CEJ), University of Illinois at Chicago, Chicago, Illinois; Department of Ophthalmology (SLP), Stein Eye Institute, University of California, Los Angeles, California; Department of Ophthalmology (HEM), Byers Eye Institute, Stanford University, Palo Alto, California; and Department of Neurology and Neurological Sciences (HEM), Stanford University, Palo Alto, California.

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http://dx.doi.org/10.1097/WNO.0000000000000891DOI Listing
June 2020

Randomized Controlled Trials: A Primer for Neuro-Ophthalmologists.

J Neuroophthalmol 2020 03;40(1):3-7

Departments of Ophthalmology (HEM) and Neurology and Neurological Sciences (HEM), Stanford University, Palo Alto, California; Department of Statistical Science (JC), Southern Methodist University, University Park, Dallas, Texas; and Department of Ophthalmology (SLP), University of California at Los Angeles, Los Angeles, California.

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http://dx.doi.org/10.1097/WNO.0000000000000873DOI Listing
March 2020

Application of Hemifield Visual Electrophysiology to Diagnose Functional Vision Loss.

J Neuroophthalmol 2020 12;40(4):527-529

Departments of Ophthalmology (HEM), and Neurology and Neurological Sciences (HEM), Stanford University, Palo Alto, California; and Department of Ophthalmology and Visual Sciences (SJ), University of Illinois at Chicago, Chicago, Illinois.

Neuro-ophthalmologists frequently see patients who are experiencing vision loss not accounted for by a neuro-ophthalmic disorder. In this article, we describe a case of binasal hemianopia in an otherwise healthy 65-year-old woman who was initially diagnosed with glaucoma but ultimately proved to have functional visual loss. This diagnosis was made by confirming by confirming normal visual pathway function using hemifield visual-evoked potential studies.
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http://dx.doi.org/10.1097/WNO.0000000000000882DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7737647PMC
December 2020

Retinal vessel diameter changes after 6 months of treatment in the Idiopathic Intracranial Hypertension Treatment Trial.

Br J Ophthalmol 2020 10 16;104(10):1430-1434. Epub 2020 Jan 16.

David and Ilene Flaum Eye Institute, University of Rochester, Rochester, New York, USA.

Background/aims: Prior studies support an association between increased retinal venule diameter and elevated intracranial pressure (ICP). The purpose of this study was to test the hypothesis that retinal venule diameters decrease in association with long-term therapy for high ICP in subjects with idiopathic intracranial hypertension (IIH).

Methods: This is a retrospective analysis of multicentre randomised controlled trial data. Standardised procedures were used to measure area of optic nerve head elevation (ONHA) and diameters of 4 arterioles and 4 venules 2.7 mm from the optic disc centre on fundus photos collected at baseline and after 6 months of randomised treatment with placebo+diet or acetazolamide+diet in subjects participating in the IIH Treatment Trial (IIHTT) (n=115). Change in arteriole (Da) and venule (Dv) diameters from baseline to 6 months was studied as a function of IIH, haemodynamic and demographic variables.

Results: Dv decreased following 6 months of therapy (8.1 µm, 5.9%, p<0.0005) but Da did not change. Dv change was associated with ONHA change (p<0.0005, r=0.47) and this association persisted in multiple variable models.

Conclusions: Retinal venule diameter decreased, and arteriole diameter did not change in association with treatment for elevated ICP with a weight loss intervention and placebo or acetazolamide in IIHTT participants. Further study is needed to determine how retinal vessel measurements can be combined with other clinical observations to inform disease management.
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http://dx.doi.org/10.1136/bjophthalmol-2019-314648DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7713512PMC
October 2020

Anterior Optic Neuropathy in a Patient With Cyclical Fevers.

JAMA Ophthalmol 2020 03;138(3):314-315

Department of Ophthalmology, Stanford University, Palo Alto, California.

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http://dx.doi.org/10.1001/jamaophthalmol.2019.4987DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7713502PMC
March 2020

Update on the Diagnosis and Treatment of Idiopathic Intracranial Hypertension.

Semin Neurol 2019 12 17;39(6):682-691. Epub 2019 Dec 17.

Department of Neurology and Neurological Sciences, Stanford University, Palo Alto, California.

Advances in ophthalmic diagnostics and results of interventional clinical trials are shifting diagnosis and management of idiopathic intracranial hypertension (IIH) to be more technology- and evidence-based. In this article, the evidence supporting current diagnostic criteria, evaluation, and medical and surgical management of IIH are reviewed.
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http://dx.doi.org/10.1055/s-0039-1698744DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7713505PMC
December 2019

Chiasmal and Postchiasmal Disease.

Authors:
Heather E Moss

Continuum (Minneap Minn) 2019 Oct;25(5):1310-1328

Purpose Of Review: This article reviews the anatomy, symptoms, examination findings, and causes of diseases affecting the optic chiasm, optic tracts, optic radiations, and occipital lobes.

Recent Findings: Modern ophthalmic imaging can be used to monitor the effects of diseases of the optic chiasm and tract on the retinal ganglion cells. It can also be used to visualize transsynaptic degeneration of the anterior visual pathway in the setting of acquired retrogeniculate lesions. Visual prostheses that directly stimulate the occipital lobe are a potential strategy for rehabilitation that is in active clinical trials.

Summary: Detecting and characterizing visual deficits due to optic chiasm and retrochiasmal disease are important for the diagnosis, localization, and monitoring of neurologic disease; identifying patient disability; and guiding rehabilitation.
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http://dx.doi.org/10.1212/CON.0000000000000785DOI Listing
October 2019

Predicting Risk of Perioperative Ischemic Optic Neuropathy in Spine Fusion Surgery: A Cohort Study Using the National Inpatient Sample.

Anesth Analg 2020 04;130(4):967-974

Department of Ophthalmology and Visual Sciences, College of Medicine, University of Illinois at Chicago, Chicago, Illinois.

Background: Ischemic optic neuropathy (ION) is a rare complication of anesthesia and surgery that causes vision loss in spine fusion. We sought to develop a predictive model based on known preoperative risk factors for perioperative ION to guide patient and physician preoperative decision-making.

Methods: In the National Inpatient Sample (NIS) for 1998-2012, discharges for posterior thoracic, lumbar, and sacral spine fusion were identified and classified by ION status. Variables were selected without weighting via variable clustering using Principal Component Analysis of Mixed Data (PCA-MIX). Hierarchical clustering with 4 clusters was performed, and the variable with largest squared loading in each cluster was chosen. By splitting our sample into a training and testing data set, we developed and internally validated a predictive model. The final model using variables known preoperatively was constructed to allow determination of relative and absolute risk of developing perioperative ION and was tested for calibration and discrimination.

Results: The final predictive model based on hierarchical clustering contained 3 preoperative factors, age, male or female sex, and the presence of obstructive sleep apnea (OSA). The predictive model based on these factors had an area under the receiver operating characteristic curve (AUC) of 0.65 and good calibration. A score cutoff of >1 had 100% sensitivity, while score of 3 had 96.5% specificity. The highest estimated absolute risk (844.5/million) and relative risk of ION (46.40) was for a man, age 40-64 years, with OSA.

Conclusions: The predictive model could enable screening for patients at higher risk of ION to provide more accurate risk assessment and surgical and anesthetic planning for perioperative ION in spine fusion.
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http://dx.doi.org/10.1213/ANE.0000000000004383DOI Listing
April 2020

Methods for Quantifying Optic Disc Volume and Peripapillary Deflection Volume Using Radial Optical Coherence Tomography Scans and Association With Intracranial Pressure.

Front Neurol 2019 24;10:798. Epub 2019 Jul 24.

Department of Ophthalmology, Stanford University, Palo Alto, CA, United States.

Papilledema and peripapillary deformation of Bruch's membrane (BM) are associated with elevated intracranial pressure (ICP). We have developed a novel methodology to measure these parameters using a radial optical coherence tomography (OCT) scan pattern and apply this to test the hypothesis that ICP is associated with volumetric features of ophthalmic structures. 6-radial OCT B-scans centered over the optic nerve head were acquired in 17 subjects (30 eyes) before lumbar puncture with measurement of ICP (range: 10-55 cm HO). Internal limiting membrane (ILM) and BM were segmented. Three definitions of BM were studied to account for imaging artifact affecting peripapillary BM: connecting rater-identified BM margins(traditional), connecting rater-identified BM 1.6 mm on either side of the ONH(estimated), and excluding BM in the central 3.2 mm of the images(excluded). Optic nerve head volume (ONHV), BM displacement volume (BMDV) and cup volume (CV) were calculated by interpolating between B-scans. Ganglion cell complex volume (GCCV) was measured in the macula. Linear generalized estimating equations (GEE) modeled ONVH, BMDV, and CV as a function of ICP and GCCV. Increased ONHV was associated with elevated ICP for traditional ( = 0.006), estimated ( = 0.003) and excluded ( = 0.05) BM definitions. Decreased BMDV was associated with elevated ICP for traditional ( < 0.0005), estimated ( < 0.0005) and excluded ( = 0.001) definitions. Decreased ONHV was independently associated with decreased GCCV ( = 0.001) and decreased ICP ( = 0.031) in multivariable models. CV was neither associated with ICP nor GCCV in univariate or multivariable models. Elevated ICP is associated with ONHV increase and BMDV decrease, calculated from OCT images accounting for image artifact. Ganglion cell atrophy affects the relationship between ICP and ONHV. OCT derived volumetric measures of the posterior eye may have application as biomarkers for elevated ICP.
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http://dx.doi.org/10.3389/fneur.2019.00798DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6668216PMC
July 2019

Big Data Research in Neuro-Ophthalmology: Promises and Pitfalls.

J Neuroophthalmol 2019 12;39(4):480-486

Departments of Ophthalmology and Neurology & Neurological Sciences (HEM), Stanford University, Palo Alto, California; Departments of Ophthalmology and Visual Sciences (CEJ), University of Illinois, School of Public Health, College of Medicine, Epidemiology and Public Health, Chicago, Illinois; Department of Anesthesia and Critical Care (DSR), University of Chicago, Chicago, Illinois; and Departments of Anesthesiology, Ophthalmology and Visual Sciences (SR), College of Medicine, University of Illinois, Chicago, Illinois.

Background: Big data clinical research involves application of large data sets to the study of disease. It is of interest to neuro-ophthalmologists but also may be a challenge because of the relative rarity of many of the diseases treated.

Evidence Acquisition: Evidence for this review was gathered from the authors' experiences performing analysis of large data sets and review of the literature.

Results: Big data sets are heterogeneous, and include prospective surveys, medical administrative and claims data and registries compiled from medical records. High-quality studies must pay careful attention to aspects of data set selection, including potential bias, and data management issues, such as missing data, variable definition, and statistical modeling to generate appropriate conclusions. There are many studies of neuro-ophthalmic diseases that use big data approaches.

Conclusions: Big data clinical research studies complement other research methodologies to advance our understanding of human disease. A rigorous and careful approach to data set selection, data management, data analysis, and data interpretation characterizes high-quality studies.
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http://dx.doi.org/10.1097/WNO.0000000000000751DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6658354PMC
December 2019

Association Between Peripapillary Bruch's Membrane Shape and Intracranial Pressure: Effect of Image Acquisition Pattern and Image Analysis Method, a Preliminary Study.

Front Neurol 2018 21;9:1137. Epub 2018 Dec 21.

Department of Ophthalmology, Stanford University, Palo Alto, CA, United States.

High intracranial pressure (ICP) is associated with changes in peripapillary Bruch's membrane (pBM) shape on optical coherence tomography (OCT) images of the optic nerve head. It is not known if image acquisition pattern and analysis method impact this association. Cross sectional OCT scans of the optic nerve head were obtained at six angles using a radial scan pattern in 21 subjects immediately prior to ICP measurement via lumbar puncture. On each image, Bruch's membrane was manually segmented and defined by either 14 or 16 semi-landmarks and either rater identified, or distance identified boundaries. For each of these four image analysis strategies, geometric morphometric analysis identified the first principal component of Bruch's membrane shape for all images and for the set of images taken at each angle. Repeated measures ANOVA of the first principal component magnitude (PC1) for all images assessed for shape difference between image angles. Linear generalized estimating equation models assessed association between angle specific first principal component magnitudes (PC1) and ICP for each angle. Receiver operating characteristic analysis assessed angle specific PC1s' ability to differentiate elevated from normal ICP. The first principal component represented deflection into the vitreous for all scan angles, but quantitatively differed across scan angles ( < 0.005, repeated measures ANOVA). Angle specific first principal components were positively correlated with ICP ( < 0.005 for all angles, generalized estimating equation models). All angle specific first principal components showed excellent ability to classify ICP (area under curve ≥ 0.8 for all). These results were independent from image analysis strategy. Though qualitative changes in Bruch's membrane shape are similar regardless of cross-sectional angle of the 2-D OCT scan, they differ quantitatively between OCT scan angles, meaning that pBM is not axially symmetric and therefore PC1 extracted from different 2-D scan angles can't be compared between individuals. However, we do not identify an optimal scan angle for classification of ICP since there is a similarly strong linear relationship between the first principal component of shape and ICP and angle specific first principal components of Bruch's membrane shape showed similarly excellent ability to differentiate elevated from normal ICP. The results support development of Bruch's membrane shape extracted from 2-D cross sectional optic nerve head OCT scans as a biomarker of ICP and emphasize the importance of consistency of scan angle. This is relevant for developing diagnostic protocols that use OCT to detect high ICP states.
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http://dx.doi.org/10.3389/fneur.2018.01137DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6308322PMC
December 2018

Perioperative Visual Loss in Cardiac Surgery.

J Cardiothorac Vasc Anesth 2019 May 24;33(5):1420-1429. Epub 2018 Nov 24.

Department of Anesthesiology, University of Illinois College of Medicine, Chicago, IL; Department of Ophthalmology and Visual Sciences, University of Illinois College of Medicine, Chicago, IL. Electronic address:

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http://dx.doi.org/10.1053/j.jvca.2018.11.035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6451882PMC
May 2019