Dr. Heather Moss is Associate Professor and Director of Clinical Research in the Stanford Department of Ophthalmology. She directs an innovative clinical-research program in biomarker discovery at the Spencer Center for Vision Research at Stanford and provides expert neuro-ophthalmic clinical care at the Byers Eye Institute at Stanford.
Dr. Moss pursued undergraduate studies in biomedical engineering at the University of Guelph, followed by doctoral studies in medical engineering at Harvard and MIT, seeking to improve human health through application of engineering principles. Working with patients inspired her to become a physician; after earning her medical degree at Harvard, she completed residency training in Neurology and fellowship training in Neuro-ophthalmology at the University of Pennsylvania. She spent six years on the faculty of the University of Illinois at Chicago, serving as the Director of Neuro-ophthalmology and starting a neuro-ophthalmology fellowship training program, before joining the Stanford faculty in 2016.
Her clinical expertise includes diagnosis and treatment of optic nerve diseases, eye movement disorders, and neurological pathology affecting visual pathways. She is a national leader in application of telemedicine to improve access to neuro-ophthalmic care. Her research lab is developing novel diagnostic approaches to neurological and neuro-ophthalmic diseases through application of innovative electrophysiology, imaging, and mathematical modeling. She has published over 80 articles in peer-reviewed journals, has authored numerous book chapters, and serves on the editorial board of four journals. She is a fellow of the American Academy of Neurology (AAN) and the North American Neuro-Ophthalmology Society (NANOS) and has been elected to leadership roles in both organizations.
Outside of work Dr. Moss enjoys spending time with her two children, exploring northern California, and renovating her 100-year-old house.
- Papilledema, pseudotumor cerebrii, idiopathic intracranial hypertension
- optic neuritis
Director, Neuro-ophthalmology Fellowship, Stanford School of Medicine (2021 - Present)
Director, Clinical Research, Stanford Department of Ophthalmology (2020 - Present)
Director, Neuro-ophthalmology Fellowship, University of Illinois at Chicago Department of Ophthalmology (2011 - 2016)
Director, Neuro-ophthalmology Section, University of Illinois at Chicago Department of Ophthalmology (2011 - 2016)
Honors & Awards
Achievement Award, American Academy of Ophthalmology (2021)
Faculty Teaching Award (resident selected), Stanford Department of Ophthalmology (2020)
Sybil Harrington Special Scholar Award, Research to Prevent Blindness (2015)
Young Investigator of the Year, North American Neuro-ophthalmology Society (2015)
Teacher of the year, Chicago Curriculum in Ophthalmology (2012, 2014)
Zeritsky prize for research by a resident, University of Pennsylvania (2009)
Biomedical engineering pre-doctoral fellowship, Whitaker Foundation (1998-2003)
Science and Technology Award, Canadian Federation of University Women (1998)
Boards, Advisory Committees, Professional Organizations
Chair, New Knowledge in Neuro-ophthalmology Committee, North American Neuro-ophthalmology Society (2020 - Present)
Chair, Outcomes Committee, North American Neuro-ophthalmology Society (2020 - Present)
Chair, Practice Support Committee, North American Neuro-ophthalmology Society (2020 - Present)
Member, EyeNet Editorial Board, American Academy of Ophthalmology (2020 - Present)
Member, Health Information Management Committee, Stanford Health Care (2020 - Present)
Member, Quality Council, Stanford Department of Ophthalmology (2020 - Present)
Associate Editor, Neuro-ophthalmology, Medlink Inc. (2019 - Present)
Associate Editor, Neuro-ophthalmology, Frontiers in Neurology (2018 - Present)
Member, Basic and Clinical Science Curriculum Section 5 Committee, American Academy of Ophthalmology (2018 - Present)
Section Director, Clinical Research, Journal of Neuro-ophthalmology (2018 - Present)
Member, Board of Directors, North American Neuro-Ophthalmology Society (2017 - Present)
Fellow, American Academy of Neurology (2016 - Present)
Fellow, North American Neuro-ophthalmology Society (2016 - Present)
Review Editor, Current Eye Research (2016 - Present)
(Vice) Chair, Neuro-ophthalmology Neuro-otology Section, American Academy of Neurology (2016 - 2020)
Chair, Abstract Committee, North American Neuro-ophthalmology Society (2016 - 2020)
President, Upper Midwest Neuro-ophthalmology Group (2015 - 2016)
Assistant Editor, Neuro-ophthalmology (2013 - 2019)
Certificate, Stanford Medicine Leadership Academy, Leadership (2021)
Certificate, Realizing Improvement through Team Empowerment (RITE), Stanford Medicine, Quality Improvement (2020)
Certificate, Stanford Medicine Leadership Development Program, Leadership (2020)
Graduate Certificate, University of Illinois School of Public Health, Clinical and Translational Research (2014)
fellowship, University of Illinois at Chicago, Medical Education (2011)
fellowship, University of Pennsylvania, Neuro-ophthalmology (2010)
board certification, American Board of Psychiatry and Neurology, Adult Neurology (2010)
residency, University of Pennsylvania, Neurology (2009)
Internship: Massachusetts General Hospital Internal Medicine Residency (2006) MA
MD, Harvard Medical School (2005)
PhD (joint program), Harvard-MIT Division of Health Sciences and Technology, Medical Engineering & Medical Physics (2003)
PhD, Harvard University, Engineering Sciences (2003)
B.Sc. (eng), University of Guelph (Canada), Biological Engineering (1997)
Current Research and Scholarly Interests
Permanent vision loss caused by papilledema, the swelling of the optic nerve heads due to elevation in intracranial pressure (ICP), occurs in 50% of people with idiopathic intracranial hypertension (IIH) as well as individuals with high ICP from other neurological and neurosurgical diseases. One reason that blindness results from IIH, which is a treatable disease, is lack of timely, accurate clinical markers with which to identify those who are at risk of losing vision.
My research program seeks to identify and develop such markers through studies of papilledema physiology in humans affected by IIH. My current studies focus on humans with IIH because this accurately captures both the disease of interest and the target population. The conceptual frameworks that underlie my research program are drawn from my doctoral level engineering training. Using a mechanical (structural) framework I am evaluating the effect of changing intra-cerebral and intra-optic nerve forces from ICP and papilledema on the shape of the optic nerve and retinal blood vessels. Using an electrical (functional) framework I am evaluating patterns of visual pathway dysfunction in papilledema using non-invasive techniques of electrophysiology, pupillary light response and psychophysics. Markers based on both of these frameworks have the potential to capture the dynamics of pathophysiological changes associated with evolving and resolving papilledema with less delay than currently used clinical markers.
My aim is to develop non-invasive structural and functional markers of papilledema physiology that predict visual outcomes in IIH and guide tailored intervention that will improve visual outcomes and prevent blindness. The short-term objective of my research program is to evaluate candidate markers with regards to differences between untreated IIH, treated IIH and normal patients, changes over time in IIH patients receiving treatment, and differences between IIH patients with and without vision loss. The long-term objective of my research program is to elucidate markers of papilledema physiology that can be studied non-invasively and to ascertain their ability to predict future visual function in IIH and guide clinical management.
Other areas of active research include study of peri-operative vision loss and visual pathway based diagnosis of neuro-degenerative diseases. I am actively involved in clinical trials through the Neuro-Ophthalmology Research Disease Investigator Consortium (NORDIC).
Physiologically Based Markers of Idiopathic Intracranial Hypertension, Stanford University
Permanent visual impairment due to papilledema, an optic neuropathy characterized by optic nerve swelling, occurs in approximately half of patients with IIH. There is a significant clinical need for non-invasive biomarkers that will advance diagnosis and management of IIH. The objective of my research is to establish physiologically based markers of retinal ganglion cell(RGC) function and retinal/cerebral vasculature as markers of IIH that detect abnormalities, monitor treatment and distinguish peripheral vision outcomes. I have demonstrated that retinal vein diameter changes over the course of disease. Through collaboration with Dr. Ali Alaraj, an endovascular neurosurgeon, we have defined characteristic changes in cerebral venous blood flow and pressure in IIH patients. Through collaboration with Dr. McAnany, a psychophysics expert, we have demonstrated alterations in objective markers of optic nerve function that correlate with other measures of disease in IIH patients. These results are laying the scientific and technical foundation for the development of these markers as clinical tools and clinical trial outcome measures. Furthermore, the results are advancing scientific understanding of the pathophysiology underlying papilledema and other optic neuropathies.
Palo Alto, Ca
Risk factors for peri-operative vision loss, Stanford University, University of Illinois at Chicago, University of Chicago
Perioperative visual loss (POVL) is a devastating complication, with no known treatment or prevention,
most commonly due to ischemic optic neuropathy (ION), and retinal arterial occlusion (RAO), and less commonly
cortical blindness. We reported in 2009 that POVL had an estimated incidence of 3-10 cases/10,000
procedures in two of the highest volume surgical procedures.1 The resulting severe visual impairment costs >
$27,000/y, or $675,000 during the estimated remainder of a middle-aged individual’s life from increased health
care spending alone. Lost productivity costing > $250,000, and frequent litigation further increase costs. The
emotional toll of sudden, unexpected visual loss is immeasurable. It is imperative to understand the risk factors
for POVL in order to develop means to prevent these blinding complications. In collaboration with the University of Illinois at Chicago and University of Illinois at Chicago we are studying risk factors and developing a predictive model for perioperative visual loss (POVL) in spinal fusion and cardiac surgery.
Palo Alto, Ca
- Steven Roth, Professor, University of Illinois at Chicago
- Charlotte Joslin, Associate Professor, University of Illinois at Chicago
- Daniel Rubin, Assistant Professor, University of Chicago
Visual pathway based markers of neuro-degenerative disease
Clinical and post-mortem observations of pathological effects spreading beyond the motor system in some people with ALS have led to a shift from the classical characterization of ALS as a disease exclusively of motor neurons to that of a multisystem disorder. During my fellowship training in neuro-ophthalmology I led the largest characterization of clinical eye movement disorders in this population and discovered previously undocumented afferent visual dysfunction. Collaboration with Dr. Amani Fawzi at Northwestern University has indicated that retinal pathology may account for this observation. We have surveyed different tests of afferent visual function to determine which are abnormal in ALS patients and which has the best correlation with visual system pathology in ALS patients.
Current efforts involve infrastructure development to improve access to ophthalmic imaging in the neurosciences clinic to facilitate studies of ophthalmic markers of neurodegenerative disease and efforts to develop pupillometry apparatus with which to study diagnostic potential in neuro-degenerative disease.
- Daniel Joyce, School of Medicine
Neuro-ophthalmology clinical research
The rarity of many neuro-ophthalmic diseases is a barrier to effective clinical outcomes research. This barrier can be overcome through collaborations between investigators and institutions to increase sample size, and through application of advanced statistical techniques to clinical trial data sets to maximize data analysis efficiency. I am actively involved in the NIH sponsored Neuro-ophthalmology Research Disease Investigator Consortium. Current active treatment trials are studying nonarteritic anterior ischemic optic neuropathy, idiopathic intracranial hypertension, and Lebers hereditary optic neuropathy.
Palo Alto, CA
The Impact of COVID-19 on Neuro-Ophthalmology Office Visits and Adoption of Telemedicine Services.
Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2021; 41 (3): 362-367
BACKGROUND: The COVID-19 public health emergency (PHE) has significantly changed medical practice in the United States, including an increase in the utilization of telemedicine. Here, we characterize change in neuro-ophthalmic care delivery during the early COVID-19 PHE, including a comparison of care delivered via telemedicine and in office.METHODS: Neuro-ophthalmology outpatient encounters from 3 practices in the United States (4 providers) were studied during the early COVID-19 PHE (March 15, 2020-June 15, 2020) and during the same dates 1 year prior. For unique patient visits, patient demographics, visit types, visit format, and diagnosis were compared between years and between synchronous telehealth and in-office formats for 2020.RESULTS: There were 1,276 encounters for 1,167 patients. There were 30% fewer unique patient visits in 2020 vs 2019 (477 vs 670) and 55% fewer in-office visits (299 vs 670). Compared with 2019, encounters in 2020 were more likely to be established, to occur via telemedicine and to relate to an efferent diagnosis. In 2020, synchronous telehealth visits were more likely to be established compared with in-office encounters.CONCLUSIONS: In the practices studied, a lower volume of neuro-ophthalmic care was delivered during the early COVID-19 public health emergency than in the same period in 2019. The type of care shifted toward established patients with efferent diagnoses and the modality of care shifted toward telemedicine.
View details for DOI 10.1097/WNO.0000000000001356
View details for PubMedID 34415270
- Gender Gap in Neurology Research Authorship (1946-2020) FRONTIERS IN NEUROLOGY 2021; 12
Factors Associated With Increased Emergency Department Utilization in Patients With Acute Optic Neuritis.
Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
BACKGROUND: Symptoms of acute vision loss and eye pain may lead patients with optic neuritis to seek care in the emergency department (ED). Given the availability of lower cost alternatives for providing medical care for optic neuritis, this study aimed to identify factors associated with higher ED utilization.METHODS: Subjects with acute optic neuritis were identified through a chart review of adults with International Classification of Diseases-9 (ICD-9) or ICD-10 codes for optic neuritis with corresponding gadolinium contrast enhancement of the optic nerve on MRI in the medical record research repository of a tertiary care institution. Subjects were grouped based on the number of ED visits (0-1 and 2-3) within 2 months of either ICD code or MRI. Demographics, characteristics of disease presentation, type and location of medical care, testing (chest imaging, lumbar puncture, optical coherence tomography, spine MRI, visual field, and laboratory tests), treatment, provider specialty of follow-up visits, and duration of care were extracted from the medical record.RESULTS: Of 30 acute optic neuritis subjects (age 41 ± 16 years, range 18-76, 53% [16/30] female), 19 had 0-1 ED visit and 11 had 2-3 ED visits. Most subjects were Caucasian, non-Hispanic (47%), followed by Asian (23%), Hispanic/Latino (17%), Black (10%), and others (3%). Subjects had an initial clinical encounter primarily in the outpatient setting (63%) as compared with the ED (37%). The median time from symptom onset to initial clinical encounter was 4 days with a range of 0-13. Subjects were mostly insured through a private insurance (60%), followed by Medicare/Medicaid (23%) and uninsured (17%). Fewer ED visits were associated with an initial clinical encounter in an outpatient setting (P = 0.02, chi-square), but not residential distance from the hospital or insurance type. Subjects with a higher number of ED visits were more likely to be of Hispanic/Latino ethnicity (P = 0.047, Fisher exact). There was no significant difference in the ophthalmic, radiologic, or laboratory testing performed in both groups. Both groups presented in a similar time frame with similar symptoms and clinical signs. Treatment was similar in both groups.CONCLUSIONS: Subjects with their first clinical encounter for optic neuritis in the ED had more visits to the ED overall when compared with those first seen in an outpatient setting and thus strategies aimed at facilitating outpatient care may help reduce unnecessary ED visits, although some, such as insurance status, may be difficult to modify. Further study in a larger sample is needed to refine these observations.
View details for DOI 10.1097/WNO.0000000000001294
View details for PubMedID 34224527
Optic chiasm involvement in AQP-4 antibody-positive NMO and MOG antibody-associated disorder.
Multiple sclerosis (Houndmills, Basingstoke, England)
BACKGROUND: Optic neuritis (ON) is often the presenting symptom in inflammatory central nervous system demyelinating disorders.OBJECTIVE: To compare the frequency and pattern of optic chiasm involvement in patients with aquaporin-4-immunoglobulin G (AQP4-IgG)-associated ON to patients with myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG)-associated ON.METHODS: Retrospective review of all patients evaluated at Mayo Clinic, Stanford University and Ramathibodi Hospital who were found to have: (1) ON, (2) either MOG-IgG or AQP4-IgG by cell-based assay, and (3) magnetic resonance imaging (MRI) at the time of ON. MRI was reviewed for contrast enhancement of the optic chiasm and the pattern of involvement.RESULTS: One hundred and fifty-four patients (74 AQP4-IgG and 80 MOG-IgG) were included. Among patients with AQP4-IgG-ON, 20% had chiasmal involvement, compared with 16% of patients with MOG-IgG-ON (p = 0.66). In patients with chiasmal involvement, longitudinally extensive optic nerve enhancement (from orbit extending to chiasm) was identified in 54% of MOG-IgG-ON patients, compared with 7% of AQP4-IgG-ON patients (p = 0.01).CONCLUSION: Chiasmal involvement of MOG-IgG-ON and AQP4-IgG-ON occur at more similar frequencies than previously reported. Furthermore, MOG-IgG-ON chiasmal involvement is more likely to be part of a longitudinally extensive optic nerve lesion.
View details for DOI 10.1177/13524585211011450
View details for PubMedID 33975499
2021; 39 (2): 631–47
Neuro-ophthalmic emergencies include optic nerve, central visual pathway, and ocular motility disorders that, if not identified and treated promptly, may lead to permanent vision loss, other significant morbidity, or mortality. This article provides a framework for approaching patients with neuro-ophthalmic symptoms and reviews the presentation, evaluation, and treatment of select emergent conditions that can cause them. Emergent causes of blurry vision, transient vision loss, papilledema, and diplopia, including giant cell arteritis, cardioembolic disease, and aggressive infection, are discussed.
View details for DOI 10.1016/j.ncl.2021.01.004
View details for PubMedID 33896536
Functional Vision Disorders in adults: A paradigm and nomenclature shift for ophthalmology.
Survey of ophthalmology
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.
View details for DOI 10.1016/j.survophthal.2021.03.002
View details for PubMedID 33737039
Development and Implementation of a Handheld Pupillometer for Detection of Optic Neuropathies.
Current eye research
Purpose: 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. Materials & methods: 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 (470nm) stimuli in healthy subjects. Flashes of 1 s dim (50cd/m2) and bright (316cd/m2) blue light were administered to both eyes of subjects with unilateral optic neuropathies (n=10) and controls (n=5). Maximum pupillary constriction (Cmax) for each stimulus was compared between control eyes and optic neuropathy eyes. Cmax for the inter-eye difference curve (Cdiffmax) was compared between control and optic neuropathy subjects. Results: The pupil protocol lasted 15minutes and was well tolerated by subjects. Cmax for bright and dim stimuli was reduced in eyes with optic neuropathy compared to fellow and control eyes (p <.0005 for all). Inter-eye Cdiffmax was larger in optic neuropathy subjects than control subjects for both dim and bright stimuli (p =.002, <0.0005). There was no overlap between groups for Cmax and Cdiffmax for either stimulus. Conclusions: 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.
View details for DOI 10.1080/02713683.2021.1878542
View details for PubMedID 33541152
Head-Down Tilt Bed Rest Studies as a Terrestrial Analog for Spaceflight Associated Neuro-Ocular Syndrome.
Frontiers in neurology
2021; 12: 648958
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.
View details for DOI 10.3389/fneur.2021.648958
View details for PubMedID 33841315
Gender Gap in Neurology Research Authorship (1946-2020).
Frontiers in neurology
2021; 12: 715428
Gender disparity in the field of neurology impedes scientific advancements and innovations. In 2018, 45.0% of neurology and neurological subspecialty residents were women. Despite a notable rise in the proportion of women neurologists over the past decades, inequalities regarding publication proportions between men and women persist in the field. This cohort study examines authorship trends in articles published in 155 international neurology journals, identified as those listed in the annual Journal Citation Reports' "Clinical Neurology" section. Authors' names, authorship positions and countries of affiliation were extracted from PubMed for indexed articles published from 1946 to 2020. Gender-API (a validated and highly accurate application program interface) assigned binary genders to authors. Author gender proportions were compared across subspecialties, authorship position and years. In 303,385 unique articles, 1,663,036 total authors were identified of which 34.1% were women. Neuroradiology demonstrated the lowest proportion of women authors (21.3%), while neurogenetics displayed the highest (44.5%). In articles with multiple authors, both men and women last authors were more likely to publish with a male first author, though this was significantly more pronounced for men last authors (1.86 vs. 1.08; p < 0.001). From 2002 to 2020, women remained in the minority of last (24.6%), first (36.2%), and middle author positions (35.8%). The authorship gender distribution in neurological journals neither reflects the gender proportion of neurologists in the field overall nor in any subspecialty examined. We also find a tendency for senior and junior authors of the same gender to publish together which perpetuates authorship inequity. Further work is needed to identify underlying causes so that interventions might be developed to improve authorship diversity.
View details for DOI 10.3389/fneur.2021.715428
View details for PubMedID 34497579
View details for PubMedCentralID PMC8419229
Novel Foveal Features Associated With Vision Impairment in Multiple Sclerosis.
Investigative ophthalmology & visual science
2021; 62 (12): 27
To characterize scattering and hyperreflective features in the foveal avascular zone of people with multiple sclerosis (MS) using adaptive optics scanning laser ophthalmoscopy (AOSLO) and to evaluate their relationship with visual function and MS disease characteristics.Twenty subjects with MS underwent confocal reflectance and non-confocal split-detection AOSLO foveal imaging. Peripapillary retinal nerve fiber layer thickness was measured using optic nerve optical coherence tomography. Blood pressure, intraocular pressure (IOP), and best-corrected high-contrast visual acuity (HCVA) and low-contrast visual acuity (LCVA) were measured. AOSLO images were graded to determine the presence and characteristics of distinct structures.Two distinct structures were seen in the avascular zone of the foveal pit. Hyperreflective puncta, present in 74% of eyes, were associated with IOP and blood pressure. Scattering features, observed in 58% of eyes, were associated with decreased HCVA and LCVA, as well as increased MS duration and disability, but were not associated with retinal nerve fiber layer thickness. Hyperreflective puncta and scattering features were simultaneously present in 53% of eyes.Hyperreflective puncta were associated with parameters affecting ophthalmic perfusion, but they were not associated with MS disease parameters. Scattering features were associated with parameters corresponding to advanced MS, suggesting that they may be related to disease progression. Scattering features were also correlated with reduced visual function independent from ganglion cell injury, suggesting the possibility of a novel ganglion cell-independent mechanism of impaired vision in people with MS.
View details for DOI 10.1167/iovs.62.12.27
View details for PubMedID 34581726
- Should Lumbar Puncture Be Required to Diagnose Every Patient With Idiopathic Intracranial Hypertension? Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society 2021; 41 (3): 379-384
Validity of International Classification of Diseases Codes for Identifying Neuro-Ophthalmic Disease in Large Data Sets: A Systematic Review.
Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2020; 40 (4): 514–19
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.
View details for DOI 10.1097/WNO.0000000000000971
View details for PubMedID 33197163
More rapid recovery and improved outcome with early steroid therapy in mog-igg associated optic neuritis
SAGE PUBLICATIONS LTD. 2020: 466-467
View details for Web of Science ID 000596547101498
Numerical Investigation on the Role of Mechanical Factors Contributing to Globe Flattening in States of Elevated Intracranial Pressure.
Life (Basel, Switzerland)
2020; 10 (12)
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.
View details for DOI 10.3390/life10120316
View details for PubMedID 33260780
Telemedicine for neuro-ophthalmology: challenges and opportunities.
Current opinion in neurology
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.
View details for DOI 10.1097/WCO.0000000000000880
View details for PubMedID 33230033
MRI findings as markers of idiopathic intracranial hypertension.
Current opinion in neurology
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.
View details for DOI 10.1097/WCO.0000000000000885
View details for PubMedID 33230036
Retinal Diseases that Can Masquerade as Neurological Causes of Vision Loss.
Current neurology and neuroscience reports
2020; 20 (11): 51
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.
View details for DOI 10.1007/s11910-020-01071-1
View details for PubMedID 32930896
- Higher Cortical Dysfunction Presenting as Visual Symptoms in Neurodegenerative Diseases FRONTIERS IN NEUROLOGY 2020; 11
Differences in Clinical Features of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Optic Neuritis in White and Asian Race.
American journal of ophthalmology
PURPOSE: To determine whether clinical features and visual outcomes of myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-ON) differ between subjects who are White compared with those who are Asian.DESIGN: Multicenter retrospective cohort.METHODS: SETTING: Multicenter.PATIENT: 153 subjects who are White or Asian with history of adult-onset(age≥18years-old) optic neuritis and positive MOG-IgG serology by cell-based assay were enrolled from two unpublished cohorts(January 2017-November 2019) and nine published cohorts with case-level data available(2012-2018). Subjects with alternative etiologies of demyelinating disease and positive or lack of AQP-4-IgG serology result were excluded.MAIN OUTCOME MEASURES: Clinical features and final visual outcomes.RESULTS: One hundred and fifty-three subjects who are White(n=80) or Asian(n= 73) were included. 93(61%) were female, mean age of onset was 40.8±14.9 years-old, median follow up was 35.2(range1-432) months, which were all similar between White and Asian subjects. Subjects who are White were more likely to have recurrent optic neuritis(57(71%) vs. 20(27%);p=0.001) and extra-optic nerve manifestations(35(44%) vs. 8(11%);p=0.001). Optic disc swelling, neuroimaging findings, presenting visual acuity, treatment and final visual acuity did not differ according to subjects' race. Despite the high prevalence of severe visual loss(<20/200) during nadir, the majority of subjects had good recovery of visual acuity(>20/40) at final examination(51(66%) of 77 subjects who are White vs. 52(74%) of 70 subjects who are Asian).CONCLUSION: Subjects with MOG-ON who are White were more likely to have recurrent disease and extra-optic nerve manifestations. Visual outcomes were similar between subjects who are White and those who are Asian.
View details for DOI 10.1016/j.ajo.2020.07.008
View details for PubMedID 32681910
A Tearfully Painful Darkness.
Survey of ophthalmology
A 70-year-old woman presented with new onset of left eye and facial pain. Ophthalmic and neurological examinations, MRI brain, ESR and CRP were unrevealing. A few days later she developed vision loss in her left eye. Exam 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 MRI 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 (MOG) IgG titer. She was treated with IV methylprednisolone 1000mg daily for 3 days and was discharged on prolonged prednisone taper with return of vision to baseline.
View details for DOI 10.1016/j.survophthal.2020.06.002
View details for PubMedID 32540257
Acute Inflammatory Optic Neuritis Associated with a Self-Taper of Oral Prednisone in a Patient Taking Adalimumab.
Neuro-ophthalmology (Aeolus Press)
2020; 44 (3): 186–89
Therapies, such as adalimumab, aimed at inhibiting the pro-inflammatory cytokine "tumour necrosis factor" (TNF) are effective and are frequently used in combination with non-biologic disease-modifying anti-rheumatic drugs to treat rheumatoid arthritis (RA) and other autoimmune diseases. Some reports indicate that, rarely, demyelinating CNS disorders such as optic neuritis can present in association with therapy initiation, whilst others suggest that there is no association between the two. Oral corticosteroids such as prednisone, though similarly effective in the treatment of inflammatory or auto-immune conditions, can be associated with adverse effects upon their discontinuation or tapering. We present a patient who developed an acute inflammatory optic neuropathy shortly after a self-taper of oral prednisone while being treated with adalimumab for RA, and discuss the challenge of deciding whether or not to halt anti-TNF therapy.
View details for DOI 10.1080/01658107.2019.1566386
View details for PubMedID 32395171
Optical coherence tomography use in idiopathic intracranial hypertension.
Annals of eye science
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.
View details for DOI 10.21037/aes.2019.12.06
View details for PubMedID 32405617
- Randomized Controlled Trials: A Primer for Neuro-Ophthalmologists. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society 2020; 40 (1): 3–7
Retinal vessel diameter changes after 6 months of treatment in the Idiopathic Intracranial Hypertension Treatment Trial.
The British journal of ophthalmology
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 2.7mm from the optic disc centre on fundus photos collected at baseline and after 6 months of randomised treatment with placebo+dietor 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 (8.1m, 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.
View details for DOI 10.1136/bjophthalmol-2019-314648
View details for PubMedID 31949093
Application of Hemifield Visual Electrophysiology to Diagnose Functional Vision Loss.
Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
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.
View details for DOI 10.1097/WNO.0000000000000882
View details for PubMedID 32028454
- Anterior Optic Neuropathy in a Patient With Cyclical Fevers. JAMA ophthalmology 2020
- Peri-operative ischemic optic neuropathy in spinal fusion surgery: Validating a predictive scoring system. The spine journal : official journal of the North American Spine Society 2020
Higher Cortical Dysfunction Presenting as Visual Symptoms in Neurodegenerative Diseases.
Frontiers in neurology
2020; 11: 679
Introduction: 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. Methods: 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. Results: 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%). Conclusion: 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.
View details for DOI 10.3389/fneur.2020.00679
View details for PubMedID 32903759
View details for PubMedCentralID PMC7438888
- Ultra-widefield Fundus Image in Oculocutaneous Albinism. JAMA ophthalmology 2020; 138 (9): e200864
- Evolution of the Journal of Neuro-Ophthalmology and the Clinical Ophthalmology Literature: A 20-Year Retrospective. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society 2020; 40 (2): 141–43
- The Case-Control Study in Neuro-Ophthalmology. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society 2020; 40 (2): 144–47
Predictive model of Ischemic optic neuropathy in spinal fusion surgery using a longitudinal medical claims database.
The spine journal : official journal of the North American Spine Society
Perioperative ischemic optic neuropathy (ION) is a devastating complication of spinal fusion surgery.To develop predictive models of this blinding condition using longitudinal medical administrative claims databases, which provide temporal sequence of perioperative ischemic optic neuropathy and potential risk factors.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.Peri-operative ION (or not) during hospitalization for lumbar or thoracic spinal fusion surgery.65 ION cases and 106,871 controls were identified. Matched controls (n=211) were selected based on year of surgery and zip code. Chronic and peri-operative variables were assigned based on medical claims codes. Least absolute shrinkage and selection (LASSO) penalized conditional logistic regression with ten-fold cross validation was used to select variables for the optimal predictive model from the subset of variables with p < 0.15 between cases and matched controls (unadjusted conditional logistic regression). Receiver operating characteristic (ROC) curves were generated for the strata-independent matched and full sample.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 in the predictive model. Area under ROC curve was 0.75 (95% CI: 0.68, 0.82) for the matched sample and 0.72 (95% CI: 0.66, 0.78) for the full sample.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 ICD-10 codes and study of ophthalmic conditions as risk factors. Similar to other studies of this condition the multivariable model included age, male gender, peri-operative organ damage and peri-operative hemorrhage. Hypertension, chronic anemia and carotid artery stenosis were new predictive factors identified by this study.
View details for DOI 10.1016/j.spinee.2020.11.011
View details for PubMedID 33248270
Steroid-sparing maintenance immunotherapy for MOG-IgG associated disorder.
Myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) associated disorder (MOGAD) often manifests with recurrent CNS demyelinating attacks. The optimal treatment for reducing relapses is unknown. To help determine the efficacy of long-term immunotherapy in preventing relapse in patients with MOGAD, we conducted a multicenter retrospective study to determine the rate of relapses on various treatments.We determined the frequency of relapses in patients receiving various forms of long-term immunotherapy for MOGAD. Inclusion criteria were history of ≥1 CNS demyelinating attacks, MOG-IgG seropositivity, and immunotherapy for ≥6 months. Patients were reviewed for CNS demyelinating attacks before and during long-term immunotherapy.Seventy patients were included. The median age at initial CNS demyelinating attack was 29 years (range 3-61 years; 33% <18 years), and 59% were female. The median annualized relapse rate (ARR) before treatment was 1.6. On maintenance immunotherapy, the proportion of patients with relapse was as follows: mycophenolate mofetil 74% (14 of 19; ARR 0.67), rituximab 61% (22 of 36; ARR 0.59), azathioprine 59% (13 of 22; ARR 0.2), and IV immunoglobulin (IVIG) 20% (2 of 10; ARR 0). The overall median ARR on these 4 treatments was 0.3. All 9 patients treated with multiple sclerosis (MS) disease-modifying agents had a breakthrough relapse on treatment (ARR 1.5).This large retrospective multicenter study of patients with MOGAD suggests that maintenance immunotherapy reduces recurrent CNS demyelinating attacks, with the lowest ARR being associated with maintenance IVIG therapy. Traditional MS disease-modifying agents appear to be ineffective. Prospective randomized controlled studies are required to validate these conclusions.
View details for DOI 10.1212/WNL.0000000000009758
View details for PubMedID 32554760
Survey of Telehealth Adoption by Neuro-ophthalmologists During the COVID-19 Pandemic: Benefits, Barriers and Utility.
Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
During the COVID-19 pandemic tele-health 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.Telehealth utilization pre- and peri-COVID-19 was surveyed among practicing neuro-ophthalmologists in and outside the US using an on-line platform. Demographics, perceived benefits, barriers, and utility for different neuro-ophthalmic conditions were collected. Data collection occurred over a 2-week period in May, 2020.208 practicing neuro-ophthalmologists (81.3% US, 50.2% female, 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% to 68.3%, p<0.0005, remote interpretation of testing 26.7% to 32.2%, p=0.09, on-line second opinion 7.9% to 15.3%, p=0.001, interprofessional e-consult 4.4% to 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 exam, and previously collected ancillary testing and not helpful for conditions requiring funduscopic exam.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.
View details for DOI 10.1097/WNO.0000000000001051
View details for PubMedID 32639269
Reversal of Iris Heterochromia in Adult-Onset Acquired Horner Syndrome.
Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
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.
View details for DOI 10.1097/WNO.0000000000001089
View details for PubMedID 32956222
Update on the Diagnosis and Treatment of Idiopathic Intracranial Hypertension.
Seminars in neurology
2019; 39 (6): 682–91
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.
View details for DOI 10.1055/s-0039-1698744
View details for PubMedID 31847039
- Big Data Research in Neuro-Ophthalmology: Promises and Pitfalls JOURNAL OF NEURO-OPHTHALMOLOGY 2019; 39 (4): 480–86
Chiasmal and Postchiasmal Disease.
Continuum (Minneapolis, Minn.)
2019; 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.
View details for DOI 10.1212/CON.0000000000000785
View details for PubMedID 31584539
- Methods for Quantifying Optic Disc Volume and Peripapillary Deflection Volume Using Radial Optical Coherence Tomography Scans and Association With Intracranial Pressure FRONTIERS IN NEUROLOGY 2019; 10
Predicting Risk of Perioperative Ischemic Optic Neuropathy: A Study Using the US National Inpatient Sample
ASSOC RESEARCH VISION OPHTHALMOLOGY INC. 2019
View details for Web of Science ID 000488800703152
- Perioperative Visual Loss in Cardiac Surgery JOURNAL OF CARDIOTHORACIC AND VASCULAR ANESTHESIA 2019; 33 (5): 1420–29
Predicting Risk of Perioperative Ischemic Optic Neuropathy in Spine Fusion Surgery: A Cohort Study Using the National Inpatient Sample.
Anesthesia and analgesia
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.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.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.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.
View details for DOI 10.1213/ANE.0000000000004383
View details for PubMedID 31490255
Methods for Quantifying Optic Disc Volume and Peripapillary Deflection Volume Using Radial Optical Coherence Tomography Scans and Association With Intracranial Pressure.
Frontiers in neurology
2019; 10: 798
Purpose: 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. Methods: 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 H2O). 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. Results: Increased ONHV was associated with elevated ICP for traditional (p = 0.006), estimated (p = 0.003) and excluded (p = 0.05) BM definitions. Decreased BMDV was associated with elevated ICP for traditional (p < 0.0005), estimated (p < 0.0005) and excluded (p = 0.001) definitions. Decreased ONHV was independently associated with decreased GCCV (p = 0.001) and decreased ICP (p = 0.031) in multivariable models. CV was neither associated with ICP nor GCCV in univariate or multivariable models. Conclusions: 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.
View details for DOI 10.3389/fneur.2019.00798
View details for PubMedID 31396151
View details for PubMedCentralID PMC6668216
- Association Between Peripapillary Bruch's Membrane Shape and Intracranial Pressure: Effect of Image Acquisition Pattern and Image Analysis Method, a Preliminary Study FRONTIERS IN NEUROLOGY 2018; 9
- Comparison of cross sectional optical coherence tomography images of elevated optic nerve heads across acquisition devices and scan protocols EYE AND VISION 2018; 5
- Update on Perioperative Ischemic Optic Neuropathy Associated With Non-ophthalmic Surgery FRONTIERS IN NEUROLOGY 2018; 9
Retinal Vessel Diameters Change Within 1 Hour of Intracranial Pressure Lowering
TRANSLATIONAL VISION SCIENCE & TECHNOLOGY
2018; 7 (2): 6
We tested the hypotheses that retinal venule diameter (Dv) is associated with baseline intracranial pressure (ICP) level and that Dv is reduced shortly after ICP lowering.Dv and arteriole diameter (Da) were extracted from scanning laser ophthalmoscopic images in 40 eyes of 20 adult human subjects (10 with and 10 without papilledema) immediately before and after measurement of ICP (range, 10-55 cm H2O) and ICP lowering by cerebrospinal fluid (CSF) drainage via lumbar puncture (LP). Generalized estimating equations (GEE) modeled the relationship between baseline ICP, Da and Dv before LP. Additional GEE modeled the relationship between initial ICP and change in Da and Dv (post-LP - pre-LP) following ICP lowering.Test-retest variability of diameter measurements ranged from 0.1 to 2.9 μm (0.1%-2.72%). Neither Da nor Dv pre-LP was associated with baseline ICP level (P = 0.140 Dv, P = 0.914 Da, GEE). Da and Dv change after ICP lowering was associated with baseline ICP, with vessel diameters increasing with lower baseline ICP and decreasing with elevated initial ICP (P = 0.030 baseline ICP vs. Dv change, P = 0.012 baseline ICP vs. Da change, GEE models).Retina arteriole and venule diameters change immediately following ICP lowering. The direction of change is dependent on the initial ICP; both increased in subjects with high ICP and both decreased in subjects with normal ICP.The relationship between initial ICP and direction of retinal vessel size change following ICP lowering suggests a potential effect of ICP on cerebral and ocular hemodynamics that is relevant when considering the use of retinal vessel measurements as a clinical marker of ICP change.
View details for PubMedID 29576930
Perioperative Retinal Artery Occlusion: Incidence and Risk Factors in Spinal Fusion Surgery From the US National Inpatient Sample 1998-2013.
Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2018; 38 (1): 36–41
Retinal artery occlusion (RAO) is a rare but devastating complication of spinal fusion surgery. We aimed to determine its incidence and associated risk factors.Hospitalizations involving spinal fusion surgery were identified by searching the National Inpatient Sample, a database of hospital discharges, from 1998 to 2013. RAO cases were identified using ICD-9-CM codes. Using the STROBE guidelines, postulated risk factors were chosen based on literature review and identified using ICD-9-CM codes. Multivariate logistic models with RAO as outcome, and risk factors, race, age, admission, and surgery type evaluated associations.Of an estimated 4,784,275 spine fusions in the United States from 1998 to 2013, there were 363 (CI: 291-460) instances of RAO (0.76/10,000 spine fusions, CI: 0.61-0.96). Incidence ranged from 0.35/10,000 (CI: 0.11-1.73) in 2001-2002 to 1.29 (CI: 0.85-2.08) in 2012-2013, with no significant trend over time (P = 0.39). Most strongly associated with RAO were stroke, unidentified type (odds ratio, OR: 14.33, CI: 4.54-45.28, P < 0.001), diabetic retinopathy (DR) (OR: 7.00, CI: 1.18-41.66, P = 0.032), carotid stenosis (OR: 4.94, CI: 1.22-19.94, P = 0.025), aging (OR for age 71-80 years vs 41-50 years referent: 4.07, CI: 1.69-10.84, P = 0.002), and hyperlipidemia (OR: 2.96, CI: 1.85-4.73, P < 0.001). There was an association between RAO and transforaminal lumbar interbody fusion (OR: 2.95, CI: 1.29-6.75, P = 0.010). RAO was more likely to occur with spinal surgery performed urgently or emergently compared with being done electively (OR: 0.40, CI: 0.23-0.68, P < 0.001).Patient-specific associations with RAO in spinal fusion include aging, carotid stenosis, DR, hyperlipidemia, stroke, and specific types of surgery. DR may serve as an observable biomarker of heightened risk of RAO in patients undergoing spine fusion.
View details for PubMedID 28665867
View details for PubMedCentralID PMC5764807
Update on Perioperative Ischemic Optic Neuropathy Associated With Non-ophthalmic Surgery.
Frontiers in neurology
2018; 9: 557
Perioperative visual loss (POVL) is a rare, serious complication of non-ophthalmic surgeries. Ischemic optic neuropathy (ION), and retinal arterial occlusion (RAO) are the main causes (1, 2). Less frequent are cortical blindness (3), acute glaucoma (4), and choroidal and vitreous hemorrhage (5). ION is the most common cause for which the neurologist or neuro-ophthalmologist is consulted as it is associated either with a normal ophthalmic exam (posterior ION, PION), or less often, with optic nerve (ON) head swelling (anterior ION, AION). The presumed cause is impaired blood supply to the optic nerve (Figure 1). The most common surgical procedures complicated by ION are cardiac surgery and spinal fusion. Retrospective studies, surveys, and case reports are the basis of most knowledge regarding peri-operative ION (poION), with cohort and case-control studies helping to identify candidate risk factors (6, 7). Animal models have provided insight regarding mechanisms (8). This mini-review is an update on the latest advancements regarding poION in non-ophthalmic surgeries in epidemiological, clinical, and animal studies.
View details for PubMedID 30042726
View details for PubMedCentralID PMC6048244
Association Between Peripapillary Bruch's Membrane Shape and Intracranial Pressure: Effect of Image Acquisition Pattern and Image Analysis Method, a Preliminary Study.
Frontiers in neurology
2018; 9: 1137
Background/Aims: 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. Materials and Methods: 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. Results: The first principal component represented deflection into the vitreous for all scan angles, but quantitatively differed across scan angles (p < 0.005, repeated measures ANOVA). Angle specific first principal components were positively correlated with ICP (p < 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. Discussion: 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.
View details for PubMedID 30622510
View details for PubMedCentralID PMC6308322
Comparison of cross sectional optical coherence tomography images of elevated optic nerve heads across acquisition devices and scan protocols.
Eye and vision (London, England)
2018; 5: 17
Background: Optic nerve head measurements extracted from optical coherence tomography (OCT) show promise for monitoring clinical conditions with elevated optic nerve heads. The aim of this study is to compare reliability within and between raters and between image acquisition devices of optic nerve measurements derived from OCT scans in eyes with varying degrees of optic nerve elevation.Methods: Wide angle line scans and narrow angle radial scans through optic nerve heads were obtained using three spectral domain(SD) OCT devices on 5 subjects (6 swollen optic nerves, 4 normal optic nerves). Three raters independently semi-manually segmented the internal limiting membrane(ILM) and Bruch's membrane(BM) on each scan using customized software. One rater segmented each scan twice. Segmentations were qualitatively and quantitatively compared. Inter-rater, intra-rater and inter-device reliability was assessed for the optic nerve cross sectional area calculated from the ILM and BM segmentations using intraclass correlation coefficients and graphical comparison.Results: Line scans from all devices were qualitatively similar. Radial scans for which frame rate could not be adjusted were of lower quality. Intra-rater reliability for segmentation and optic nerve cross sectional area was better than inter-rater reliability, which was better than inter-device reliability, though all ICC exceeded 0.95. Reliability was not impacted by the degree of optic nerve elevation.Conclusions: SD-OCT devices acquired similar quality scans of the optic nerve head, with choice of scan protocol affecting the quality. For image derived markers, variability between devices was greater than that attributable to inter and intra-rater differences.
View details for PubMedID 30009195
Electroretinography in idiopathic intracranial hypertension: comparison of the pattern ERG and the photopic negative response.
Documenta ophthalmologica. Advances in ophthalmology
2018; 136 (1): 45–55
To evaluate the relationship between electrophysiological measures of retinal ganglion cell (RGC) function in patients who have idiopathic intracranial hypertension (IIH).The pattern electroretinogram (pERG) and photopic negative response (PhNR) were recorded from 11 IIH patients and 11 age-similar controls. The pERG was elicited by a contrast-reversing checkerboard. The PhNR, a slow negative component following the flash ERG b-wave, was recorded in response to a long-wavelength flash presented against a short-wavelength adapting field. The PhNR was elicited using full-field (ffPhNR) and focal macular (fPhNR) stimuli. Additionally, Humphrey visual field mean deviation (HVF MD) was measured and ganglion cell complex volume (GCCV) was obtained by optical coherence tomography.The ffPhNR, fPhNR, and pERG amplitudes were outside of the normal range in 45, 9, and 45% of IIH patients, respectively. However, only mean ffPhNR amplitude was reduced significantly in the patients compared to controls (p < 0.01). The pERG amplitude correlated significantly with HVF MD and GCCV (both r > 0.65, p < 0.05). There were associations between ffPhNR amplitude and HVF MD (r = 0.58, p = 0.06) and with GCCV (r = 0.52, p = 0.10), but these did not reach statistical significance. fPhNR amplitude was not correlated significantly with HVF MD or GCCV (both r < 0.40, p > 0.20).Although the fPhNR is generally normal in IIH, other electrophysiological measures of RGC function, the ffPhNR and pERG, are abnormal in some patients. These measures provide complementary information regarding RGC dysfunction in these individuals.
View details for PubMedID 29139045
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Quantitative Association Between Peripapillary Bruch's Membrane Shape and Intracranial Pressure.
Investigative ophthalmology & visual science
2017; 58 (5): 2739-2745
The purpose of this study was to determine if there is a quantitative relationship between chronic intracranial pressure (ICP) and peripapillary Bruch's membrane (pp-BM) shape and to determine whether change in pp-BM shape can be detected within 1 hour after ICP lowering by lumbar puncture (LP).In this study, 30° nasal-temporal optical coherence tomography B-scans were obtained within 1 hour before and after LP in 39 eyes from 20 patients (age = 23-86 years, 75% female, ICP [opening pressure] = 10-55 cm H2O). A total of 16 semi-landmarks defined pp-BM on each image. Geometric morphometric analysis identified principal components of shape in the image set. Generalized estimating equation models, accounting for within-subject correlation, were used to identify principal components that were associated with chronic ICP (comparing pre-LP images between eyes) and/or acute ICP changes (comparing pre- and post-LP images within eyes). The pp-BM width and anterior pp-BM location were calculated directly from each image and were studied in the same manner.Principal component 1 scalar variable on pre-LP images was associated with ICP (P < 0.0005). Principal component 4 magnitude changed within eyes after LP (P = 0.003). For both principal components 1 and 4, lower ICP corresponded with a more posterior position of pp-BM. Chronic ICP was associated with both pp-BM width (6.81 μm/cm H2O; P = 0.002) and more anterior location of temporal and nasal pp-BM margins (3.41, 3.49 μm/cm H2O; P < 0.0005, 0.002).This study demonstrates a quantitative association between pp-BM shape and chronic ICP level. Changes in pp-BM shape are detectable within 1 hour of lowering ICP. pp-BM shape may be a useful marker for chronic ICP level and acute ICP changes. Further study is needed to determine how pp-BM shape changes relate to clinical markers of papilledema.
View details for DOI 10.1167/iovs.17-21592
View details for PubMedID 28549088
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The Longitudinal Idiopathic Intracranial Hypertension Trial: Outcomes from Months 6 - 12.
American journal of ophthalmology
To determine whether the beneficial effects of acetazolamide (ACZ) in improving vision at 6 months continues to month 12 in participants of the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT).non-randomized clinical study METHODS: In the IIHTT, subjects were randomly assigned to placebo-plus-diet or maximally tolerated dosage of acetazolamide-plus-diet. At 6 months subjects transitioned from study drug to ACZ. This resulted in the following groups 1) ACZ to ACZ; n = 34; 2) placebo to ACZ; n = 35; 3) ACZ to no treatment; n = 16; and 4) placebo to no treatment; n = 11. 96 IIHTT subjects had evaluations at 6 and 12 months. Our main outcome measure was change from month 6 to month 12 in visual field mean deviation with secondary measures being change in papilledema grade, ETDRS scores and quality of life (QoL) measures.The ACZ to ACZ group improved 0.35 dB , p=0.05; placebo subjects with no ACZ improved 0.81 dB MD, p = 0.07 at 12 mos. The other groups improved 0.35 to 0.46 dB MD. Mean improvements in papilledema grade occurred most markedly in the group that exchanged placebo for ACZ (0.91 units, p < 0.001). QoL and headache disability scores showed significant improvements in the placebo group added ACZ.Improvements in MD continued from month 6 to month 12 of the IIHTT in all treatment groups -most marked in the placebo group tapered off study drug. Adding ACZ to the placebo group significantly improved papilledema grade, headache and QoL measures.
View details for DOI 10.1016/j.ajo.2017.01.004
View details for PubMedID 28104417
Diagnostic Challenge: Sequential Unilateral Cranial Neuropathies Due to Perineural Spread of Carcinoma
2017; 41 (4): 227–31
An 86-year old man developed sequential dysfunction of trigeminal (V1), facial, abducens, trigeminal (v2), oculomotor, and hypoglossal cranial nerves on the right over 20 months. Magnetic resonance imaging (MRI) showed a lesion in the right cavernous sinus. Although there was clinical suspicion that this was related to perineural spread of an extracranial tumour, a primary lesion was not discovered. Stereotactic biopsies of the intracranial lesion were non-diagnostic, and the patient succumbed to his tumour following a period of rapid growth. Postmortem examination showed the intracranial lesion to be a carcinoma with squamous features. This case highlights the challenges of diagnosis of intracranial perineural spread and the potential for transformation from indolent to aggressive tumour behaviour.
View details for DOI 10.1080/01658107.2017.1304968
View details for Web of Science ID 000415700200012
View details for PubMedID 29344066
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Cerebral Venous Thrombosis with Papilloedema Secondary to Skull Base Plasmacytoma
2017; 41 (5): 284–86
A 60-year-old woman with history of multiple myeloma was in remission after stem cell transplant 6 years prior. She was undergoing work-up for headaches that were thought to be secondary to a right mastoiditis seen on magnetic resonance imaging (MRI). On routine eye exam, papilloedema was noted. A lumbar puncture was performed, with elevated opening pressure with normal constituents. She was an atypical age for idiopathic intracranial hypertension, and her mastoiditis raised concern for secondary cerebral venous sinus thrombosis. Magnetic resonance venography (MRV) was performed showing poor flow in the right sigmoid sinus, and computed tomography venography (CTV) showed lack of contrast enhancement distal to the right sigmoid sinus, consistent with occlusion. There was also an enhancing mass inferior to the right occipital bone. Biopsy confirmed recurrent plasma cell myeloma. She was treated with chemotherapy, radiation, and warfarin for presumed cerebral venous sinus thrombosis.
View details for PubMedID 29339964
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Visual consequences of medications for multiple sclerosis: the good, the bad, the ugly, and the unknown.
Eye and brain
2017; 9: 13–21
Multiple sclerosis (MS) is associated with vision changes both due to MS effects on visual pathways and due to medication effects on the visual pathways. Distinguishing the causes of vision change are critical to appropriate diagnosis and management. The incidence, presentation, and treatment of fingolimod-associated macular edema, alemtuzumab-associated thyroid orbitopathy, and progressive multifocal leukoencephalopathy in MS patients are reviewed. Evidence for beneficial effects of acute, chronic, and symptomatic MS medications on vision is presented.
View details for PubMedID 28721111
Validation of Simplified Visual Acuity Testing Protocols in Amyotrophic Lateral Sclerosis.
Neuro-ophthalmology (Aeolus Press)
2017; 41 (5): 247–52
High- and low-contrast visual acuity (HCVA, LCVA) are potential quantitative markers of neurological dysfunction in amyotrophic lateral sclerosis (ALS). The complex nature and duration of gold standard (GS) protocols precludes widespread use in neurology settings. This study compares simplified to GS visual acuity (VA) protocols. Monocular HCVA and LCVA were measured in ALS (n= 10) and control (n= 4) subjects using six protocols, varying by two chart and three refraction methods. Intraclass correlation coefficients between simplified and GS protocols ranged from 0.83 to 0.98 (HCVA, excellent agreement) and 0.56 to 0.75 (LCVA, moderate agreement). Differences between LCVA and GS protocols exceeded test-retest reliability. Simplified HCVA protocols using LCD (liquid crystal display) tablet charts and/or pinhole correction produced valid measurements. None of the modified LCVA testing protocols produced valid measurements.
View details for DOI 10.1080/01658107.2017.1305422
View details for PubMedID 29339958
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Diffuse Colour Discrimination as Marker of Afferent Visual System Dysfunction in Amyotrophic Lateral Sclerosis.
Neuro-ophthalmology (Aeolus Press)
2017; 41 (6): 310–14
Abnormalities of the inner and intermediate retinal structures in patients with amyotrophic lateral sclerosis (ALS) have been described using optical coherence tomography and histopathology. Colour vision is a potential marker of these structural changes. The purpose of this study is to test the hypothesis that colour vision impairment is associated with ALS. Monocular (right eye) colour vision was assessed in subjects with definite or probable ALS (n= 25, aged 50-80 years) and control (n= 21, aged 46-89 years) subjects with corrected near visual acuity of at least 20/40 using the L'Anthony D15 color test (desaturated), scored by c-index, a measure of diffuse colour discrimination. Of ALS subjects, 16/25 (64%) had impaired colour vision (c-index >1.8). Comparing with our normal subjects and accounting for age, 72% (n= 18) of ALS subjects had colour vision below the 50th percentile, 52% (n= 13) had colour vision below the 25th percentile, 24% (n= 6) had colour vision below the 10th percentile, and 8% (n= 2) had colour vision below the 2nd percentile. In multivariate models of ln(c-index) and age, the intercept was higher and the slope was flatter in ALS subjects, suggesting that colour vision deficits are more prominent in younger ALS patients. Diffuse colour discrimination deficits are detected in ALS subjects at younger ages than in control subjects. Further study is needed to confirm these findings and to determine if the ALS colour discrimination abnormalities correlate with structural markers of retinal involvement and ALS disease severity.
View details for DOI 10.1080/01658107.2017.1326153
View details for PubMedID 29344070
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Perioperative Retinal Artery Occlusion: Risk Factors in Cardiac Surgery from the United States National Inpatient Sample 1998-2013.
2017; 124 (2): 189–96
To study the incidence and risk factors for retinal artery occlusion (RAO) in cardiac surgery.Retrospective study using the National Inpatient Sample (NIS).The NIS was searched for cardiac surgery. Retinal artery occlusion was identified by International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes. Postulated risk factors based on literature review were included in multivariate logistic models.Diagnosis of RAO.A total of 5 872 833 cardiac operative procedures were estimated in the United States from 1998 to 2013, with 4564 RAO cases (95% confidence interval [95% CI], 4282-4869). Nationally estimated RAO incidence was 7.77/10 000 cardiac operative procedures from 1998 to 2013 (95% CI, 7.29-8.29). Associated with increased RAO were giant cell arteritis (odds ratio [OR], 7.73; CI, 2.78-21.52; P < 0.001), transient cerebral ischemia (OR, 7.67; CI, 5.31-11.07; P < 0.001), carotid artery stenosis (OR, 7.52; CI, 6.22-9.09; P < 0.001), embolic stroke (OR, 4.43; CI, 3.05-6.42; P < 0.001), hypercoagulability (OR, 2.90; CI, 1.56-5.39; P < 0.001), myxoma (OR, 2.43; CI, 1.39-4.26; P = 0.002), diabetes mellitus (DM) with ophthalmic complications (OR, 1.89; CI, 1.10-3.24; P = 0.02), and aortic insufficiency (OR, 1.85; CI, 1.26-2.71; P = 0.002). Perioperative bleeding, aortic and mitral valve surgery, and septal surgery increased the odds of RAO. Negatively associated with RAO were female gender (OR, 0.77; CI, 0.66-0.89; P < 0.001), thrombocytopenia (OR, 0.79; CI, 0.62-1.00; P = 0.049), acute coronary syndrome (OR, 0.72; CI, 0.58-0.89; P = 0.003), atrial fibrillation (OR, 0.82; CI, 0.70-0.95; P = 0.01), congestive heart failure (OR, 0.73; CI, 0.60-0.88; P < 0.001), DM 2 (OR, 0.74; CI, 0.61-0.89; P = 0.001), and smoking (OR, 0.82; CI, 0.70-0.97; P = 0.02).Risk factors for RAO in cardiac surgery include giant cell arteritis, carotid stenosis, stroke, hypercoagulable state, and DM with ophthalmic complications; associated with lower risk were female gender, thrombocytopenia, acute coronary syndrome, atrial fibrillation, congestive heart failure, DM 2, and smoking. Surgery in which the heart was opened (e.g., septal repair) versus surgery in which it was not (e.g., CABG) and perioperative bleeding increased the risk of RAO.
View details for PubMedID 27914836
Ischemic Optic Neuropathy in Cardiac Surgery: Incidence and Risk Factors in the United States from the National Inpatient Sample 1998 to 2013.
Ischemic optic neuropathy is the most common form of perioperative visual loss, with highest incidence in cardiac and spinal fusion surgery. To date, potential risk factors have been identified in cardiac surgery by only small, single-institution studies. To determine the preoperative risk factors for ischemic optic neuropathy, the authors used the National Inpatient Sample, a database of inpatient discharges for nonfederal hospitals in the United States.Adults aged 18 yr or older admitted for coronary artery bypass grafting, heart valve repair or replacement surgery, or left ventricular assist device insertion in National Inpatient Sample from 1998 to 2013 were included. Risk of ischemic optic neuropathy was evaluated by multivariable logistic regression.A total of 5,559,395 discharges met inclusion criteria with 794 (0.014%) cases of ischemic optic neuropathy. The average yearly incidence was 1.43 of 10,000 cardiac procedures, with no change during the study period (P = 0.57). Conditions increasing risk were carotid artery stenosis (odds ratio, 2.70), stroke (odds ratio, 3.43), diabetic retinopathy (odds ratio, 3.83), hypertensive retinopathy (odds ratio, 30.09), macular degeneration (odds ratio, 4.50), glaucoma (odds ratio, 2.68), and cataract (odds ratio, 5.62). Female sex (odds ratio, 0.59) and uncomplicated diabetes mellitus type 2 (odds ratio, 0.51) decreased risk.The incidence of ischemic optic neuropathy in cardiac surgery did not change during the study period. Development of ischemic optic neuropathy after cardiac surgery is associated with carotid artery stenosis, stroke, and degenerative eye conditions.
View details for PubMedID 28244936
High and Low Contrast Visual Acuity Are Not Affected in Amyotrophic Lateral Sclerosis
2016; 11 (12)
The afferent visual system may be affected by neuro-degeneration in amyotrophic lateral sclerosis (ALS) based on observations of visual function impairment and retinal inclusions on histopathology in ALS patients. To test the hypothesis that visual acuity is impaired in ALS, we compared three measures of visual acuity in ALS patients (n = 25) attending a multidisciplinary ALS clinic and age matched control subjects (n = 25). Bilateral monocular and binocular visual acuities were assessed using high contrast (black letters on white background) and low contrast (2.5%, 1.25% grey letters on white background) visual acuity charts under controlled lighting conditions following refraction. Binocular summation was calculated as the difference between binocular and best monocular acuity scores. There were no associations between binocular or monocular high contrast visual acuity or low contrast visual acuity and amyotrophic lateral sclerosis diagnosis (generalized estimating equation models accounting for age). Binocular summation was similar in both amyotrophic lateral sclerosis and control subjects. There was a small magnitude association between increased duration of ALS symptoms and reduced 1.25% low contrast visual acuity. This study does not confirm prior observations of impaired visual acuity in patients with amyotrophic lateral sclerosis and does not support this particular measure of visual function for use in broad scale assessment of visual pathway involvement in ALS patients.
View details for DOI 10.1371/journal.pone.0168714
View details for Web of Science ID 000391226900038
View details for PubMedID 28033389
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- Unilateral Proptosis, Redness, Diplopia, and Numbness in a Young Woman JAMA OPHTHALMOLOGY 2016; 134 (11): 1325-1326
MACULAR DETACHMENT ASSOCIATED WITH ANOMALOUS OPTIC NERVES AND DURAL ECTASIA IN 49, XXXXY SYNDROME.
Retinal cases & brief reports
To present a case of a patient with XXXXY syndrome, anomalous optic nerves, and dural ectasia in conjunction with macular detachment.Case report.A 3-year-old boy with XXXXY chromosomal abnormality presented with bilateral maculopathy. On evaluation, he was found to have anomalous optic disks with serous detachment of the left eye. Magnetic resonance imaging of the brain revealed bilateral optic nerve dural ectasia without evidence of elevated intracranial pressure.XXXXY syndrome, like the related condition of Klinefelter syndrome, can manifest with ocular abnormalities. In the present case, the dural ectasia may have facilitated access of cerebrospinal fluid through anomalous optic nerves, resulting in neurosensory detachment.
View details for PubMedID 27617393
Perioperative Visual Loss in Spine Fusion Surgery: Ischemic Optic Neuropathy in the United States from 1998 to 2012 in the Nationwide Inpatient Sample.
2016; 125 (3): 457-464
Perioperative ischemic optic neuropathy (ION) causes visual loss in spinal fusion. Previous case-control studies are limited by study size and lack of a random sample. The purpose of this study was to study trends in ION incidence in spinal fusion and risk factors in a large nationwide administrative hospital database.In the Nationwide Inpatient Sample for 1998 to 2012, procedure codes for posterior thoracic, lumbar, or sacral spine fusion and diagnostic codes for ION were identified. ION was studied over five 3-yr periods (1998 to 2000, 2001 to 2003, 2004 to 2006, 2007 to 2009, and 2010 to 2012). National estimates were obtained using trend weights in a statistical survey procedure. Univariate and Poisson logistic regression assessed trends and risk factors.The nationally estimated volume of thoracic, lumbar, and sacral spinal fusion from 1998 to 2012 was 2,511,073. ION was estimated to develop in 257 patients (1.02/10,000). The incidence rate ratio (IRR) for ION significantly decreased between 1998 and 2012 (IRR, 0.72 per 3 yr; 95% CI, 0.58 to 0.88; P = 0.002). There was no significant change in the incidence of retinal artery occlusion. Factors significantly associated with ION were age (IRR, 1.24 per 10 yr of age; 95% CI, 1.05 to 1.45; P = 0.009), transfusion (IRR, 2.72; 95% CI, 1.38 to 5.37; P = 0.004), and obesity (IRR, 2.49; 95% CI, 1.09 to 5.66; P = 0.030). Female sex was protective (IRR, 0.30; 95% CI, 0.16 to 0.56; P = 0.0002).Perioperative ION in spinal fusion significantly decreased from 1998 to 2012 by about 2.7-fold. Aging, male sex, transfusion, and obesity significantly increased the risk.
View details for DOI 10.1097/ALN.0000000000001211
View details for PubMedID 27362870
JOURNAL OF NEURO-OPHTHALMOLOGY
2016; 36 (3): 308-312
We describe 2 unique cases of visual symptoms occurring during mastication in patients with lateral orbital wall defects. A 57-year-old man reported intermittent double vision and oscillopsia after a right fronto-temporal-orbito-zygomatic craniotomy with osteotomy of the lesser wing of the sphenoid for a complex invasive pituitary adenoma. Proptosis of the right globe was present only during mastication. Computed tomography (CT) revealed a bony defect in the right lateral orbital wall. A 48-year-old man presented with transient diplopia and scotoma in the right eye elicited by chewing. CT and magnetic resonance imaging demonstrated a bilobed lesion connecting the temporal fossa to the orbit through a defect in the right lateral orbital wall. The regional neuroanatomy and pathophysiology as pertaining to these cases are discussed.
View details for DOI 10.1097/WNO.0000000000000354
View details for Web of Science ID 000383274600020
View details for PubMedID 26919071
View details for PubMedCentralID PMC4772138
Research Registries: A Tool to Advance Understanding of Rare Neuro-Ophthalmic Diseases.
Journal of neuro-ophthalmology
2016; 36 (3): 317-323
Medical research registries (MRR) are organized systems used to collect, store, and analyze patient information. They are important tools for medical research with particular application to the study of rare diseases, including those seen in neuro-ophthalmic practice.Evidence for this review was gathered from the writers' experiences creating a comprehensive neuro-ophthalmology registry and review of the literature.MRR are typically observational and prospective databases of de-identified patient information. The structure is flexible and can accommodate a focus on specific diseases or treatments, surveillance of patient populations, physician quality improvement, or recruitment for future studies. They are particularly useful for the study of rare diseases. They can be integrated into the hierarchy of medical research at many levels provided their construction is well organized and they have several key characteristics including an easily manipulated database, comprehensive information on carefully selected patients, and comply with human subjects regulations. MRR pertinent to neuro-ophthalmology include the University of Illinois at Chicago neuro-ophthalmology registry, Susac Syndrome Registry, Intracranial Hypertension Registry, and larger-scale patient outcome registries being developed by professional societies.MRR have a variety of forms and applications. With careful planning and clear goals, they are flexible and powerful research tools that can support multiple different study designs, and this can provide the potential to advance understanding and care of neuro-ophthalmic diseases.
View details for DOI 10.1097/WNO.0000000000000391
View details for PubMedID 27389624
View details for PubMedCentralID PMC4988906
Objective Measures of Visual Function in Papilledema.
Advances in ophthalmology and optometry
2016; 1 (1): 231-247
Visual function is an important parameter to consider when managing patients with papilledema. Though the current standard of care uses standard automated perimetry (SAP) to obtain this information, this test is inherently subjective and prone to patient errors. Objective visual function tests including the visual evoked potential, pattern electroretinogram, photopic negative response of the full field electroretinogram, and pupillary light response have the potential to replace or supplement subjective visual function tests in papilledema management. This article reviews the evidence for use of objective visual function tests to assess visual function in papilledema and discusses future investigations needed to develop them as clinically practical and useful measures for this purpose.
View details for DOI 10.1016/j.yaoo.2016.03.004
View details for PubMedID 28451649
- Pearls & Oy-sters: A rare presentation of chronic intracranial hypertension with concurrent deafness and blindness NEUROLOGY 2016; 87 (3): E26-E28
Detection of retinal blood vessel changes in multiple sclerosis with optical coherence tomography
BIOMEDICAL OPTICS EXPRESS
2016; 7 (6): 2321-2330
Although retinal vasculitis is common in multiple sclerosis (MS), it is not known if MS is associated with quantitative abnormalities in retinal blood vessels (BVs). Optical coherence tomography (OCT) is suitable for examining the integrity of the anterior visual pathways in MS. In this paper we have compared the size and number of retinal blood vessels in patients with MS, with and without a history of optic neuritis (ON), and control subjects from the cross-sectional retinal images from OCT. Blood vessel diameter (BVD), blood vessel number (BVN), and retinal nerve fiber layer thickness (RNFLT) were extracted from OCT images collected from around the optic nerves of 129 eyes (24 control, 24 MS + ON, 81 MS-ON) of 71 subjects. Associations between blood vessel metrics, MS diagnosis, MS disability, ON, and RNFLT were evaluated using generalized estimating equation (GEE) models. MS eyes had a lower total BVD and BVN than control eyes. The effect was more pronounced with increased MS disability, and persisted in multivariate models adjusting for RNFLT and ON history. Twenty-nine percent (29%) of MS subjects had fewer retinal blood vessels than all control subjects. MS diagnosis, disability, and ON history were not associated with average blood vessel size. The relationship between MS and lower total BVD/BVN is not accounted for by RNFLT or ON. Further study is needed to determine the relationship between OCT blood vessel metrics and qualitative retinal blood vessel abnormalities in MS.
View details for DOI 10.1364/BOE.7.002321
View details for Web of Science ID 000377514000021
View details for PubMedID 27375947
View details for PubMedCentralID PMC4918585
Bariatric Surgery and the Neuro-Ophthalmologist
JOURNAL OF NEURO-OPHTHALMOLOGY
2016; 36 (1): 78-84
As the prevalence of obesity increases, so, too, do the prevalences of weight-related diseases and surgical procedures to promote weight loss. It is important for neuro-ophthalmologists to be familiar with these procedures and possible downstream effects on afferent and efferent visual function.Review of ophthalmology, neurology, general surgery, obesity, endocrinology, nutrition, psychiatry, and neurosurgery literature.Bariatric surgery is a safe and effective treatment for weight loss in obese individuals. There is Level IV evidence that it is associated with improvement in idiopathic intracranial hypertension (IIH). Laboratory nutrient deficiencies are common following some types of bariatric procedures. Symptomatic deficiencies are less common but can be devastating. Thiamine deficiency can cause nystagmus and other symptoms in weeks to months after surgery, whereas B12 or copper deficiency can cause optic neuropathy in years to decades following bariatric surgery.Bariatric surgery is a potential treatment for IIH. Postoperative vitamin deficiencies may cause nystagmus, optic neuropathy, nyctalopia, and/or ophthalmoparesis weeks to years after surgery.
View details for DOI 10.1097/WNO.0000000000000332
View details for Web of Science ID 000371534000019
View details for PubMedID 26764529
View details for PubMedCentralID PMC4755831
- Innocent Until Proven Guilty JOURNAL OF NEURO-OPHTHALMOLOGY 2016; 36 (1): 92-97
Cross-Sectional Analysis of Neurocognitive Function, Retinopathy, and Retinal Thinning by Spectral-Domain Optical Coherence Tomography in Sickle Cell Patients.
Middle East African journal of ophthalmology
2016; 23 (1): 79-83
The purpose was to examine the relationship between neurocognitive function and two distinct forms of retinopathy in sickle cell disease.Patients with sickle cell disease (n = 44, age range: 19-56 years, 70% female) were prospectively recruited for this cross-sectional study. Retinopathy was characterized by: (1) Presence of focal retinal thinning on spectral domain optical coherence tomography and (2) determination of the sickle retinopathy stage on funduscopic exam based on Goldberg classification. Neurocognitive function was assessed using the Philadelphia Brief Assessment of Cognition (PBAC), a validated test of cognition. Univariate and multivariate analyses for PBAC score outcomes were performed. Retinal thinning and retinopathy stage were primary variables of interest and age, gender, genotype, education, and history of stroke were covariates.Univariate analysis revealed associations with total PBAC score and age (P = 0.049), history of stroke (P = 0.04), and genotype (P < 0.001). Focal retinal thinning and Goldberg retinopathy stage were not associated with each other in this sample. Neither the presence of focal retinal thinning nor degree of retinopathy was associated with total PBAC score in univariate or multivariate analyses.We find an association between lower cognitive function and older age, history of stroke and sickle cell genotype SS in patients with sickle cell disease. Our data do not provide evidence to support an association between cognitive function and retinopathy in sickle cell patients.
View details for DOI 10.4103/0974-9233.150632
View details for PubMedID 26957844
View details for PubMedCentralID PMC4759909
Optic Neuropathy with Delayed Onset After Trauma: Case Report and Review of the Literature
2016; 40 (4): 188-191
We report a case of a 16 year-old healthy male, who experienced loss of vision in the right eye immediately after getting punched by a fist. His visual acuity improved to 20/20 within hours, and the optic nerve head appeared normal. CT scan of the orbits showed fractures of the right inferior orbital wall and lamina papyracea. The morning after the injury, he awoke with right eye vision decline to count fingers. There was pallid optic nerve swelling. MRI scan of the orbits showed right medial rectus enlargement and no optic canal abnormalities. The patient was treated with IV methylprednisolone with improvement in visual acuity. Literature of delayed traumatic optic neuropathy (TON) and anterior TON is reviewed.
View details for DOI 10.1080/01658107.2016.1177091
View details for Web of Science ID 000439344800005
View details for PubMedID 27570324
View details for PubMedCentralID PMC4996466
- Proceedings of the 37th Annual Upper Midwest Neuro-Ophthalmology Group Meeting, July 22, 2016, Chicago, Illinois, USA NEURO-OPHTHALMOLOGY 2016; 40 (6): 300-303
- Proceedings of the 36th Annual Upper Midwest Neuro-Ophthalmology Group Meeting, 24 July 2015, Chicago, Illinois, USA NEURO-OPHTHALMOLOGY 2016; 40 (1): 47-49
The Pupillary Light Reflex in Idiopathic Intracranial Hypertension
INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE
2016; 57 (1): 23-29
To evaluate the effects of idiopathic intracranial hypertension (IIH) on rod-, cone-, and melanopsin-mediated pupillary light reflexes (PLRs).Pupillary light reflexes elicited by full-field, brief-flash stimuli were recorded in 13 IIH patients and 13 normal controls. Subjects were dark-adapted for 10 minutes and the PLR was recorded in response to short-wavelength flashes (0.001 cd/m2: rod condition; 450 cd/m2: melanopsin condition). Subjects were then exposed to a rod-suppressing field and 10 cd/m2 long-wavelength flashes were presented (cone condition). Pupillary light reflexes were quantified as the maximum transient constriction (rod and cone conditions) and the post-illumination pupil constriction (melanopsin condition), relative to the baseline pupil size. Diagnostic power was evaluated using receiver operating characteristic (ROC) analysis.The IIH patients had significantly smaller PLRs under the melanopsin (P < 0.001) and rod (P = 0.04) paradigms; a trend for reduced cone-mediated PLRs was also found (P = 0.08). Receiver operating characteristic analysis indicated areas under the curves (AUC) of 0.83 (melanopsin-meditated; P = 0.001), 0.71 (rod-mediated; P = 0.07), and 0.77 (cone-mediated; P = 0.02). The AUC (0.90, P < 0.001), sensitivity (85%), and specificity (85%) were high for ROC analysis performed on the mean of the rod, cone, and melanopsin PLRs.Pupillary light reflex reductions in IIH patients indicate compromised RGC function. PLR measurement, particularly under rod- and melanopsin-mediated conditions, may be a useful adjunct to standard clinical measures of visual function in IIH.
View details for DOI 10.1167/iovs.15-18181
View details for Web of Science ID 000373589500004
View details for PubMedID 26746015
View details for PubMedCentralID PMC4713014
- Should Magnetic Resonance Venography be Performed Routinely in all Patients Undergoing Evaluation for Idiopathic Intracranial Hypertension? JOURNAL OF NEURO-OPHTHALMOLOGY 2015; 35 (4): 431-437
Quantitative Magnetic Resonance Venography is Correlated With Intravenous Pressures Before and After Venous Sinus Stenting: Implications for Treatment and Monitoring
2015; 77 (2): 254-260
Endovascular stenting is an effective treatment for patients with clinically significant cerebral venous sinus stenosis. Traditionally, stenting is indicated in elevated intravenous pressures on conventional venography; however, noninvasive monitoring is more desirable. Quantitative magnetic resonance angiography is an imaging modality that measures blood flow noninvasively. Established in the arterial system, applications to the venous sinuses have been limited.To examine quantitative magnetic resonance venography (qMRV) in the measurement of venous sinus flow in patients undergoing endovascular stenting and to identify a relationship with intravenous pressures.Five patients with intracranial hypertension secondary to venous sinus stenosis underwent cerebral venous stenting between 2009 and 2013 at a single institution. Preoperatively, venous sinus flow was determined by using qMRV, and intravenous pressure was measured during venography. After stenting, intravenous pressure, qMRV flow, and clinical outcomes were assessed and compared.A mean prestenotic intravenous pressure of 45.2 mm Hg was recorded before stenting, which decreased to 27.4 mm Hg afterward (Wilcoxon signed rank test P = .04). Total jugular outflow on qMRV increased by 260.2 mL/min. Analysis of the change in intravenous pressure and qMRV flow identified a linear relationship (Pearson correlation r = 0.926). All patients displayed visual improvement at 6 weeks.Venous outflow by qMRV increases after endovascular stenting and correlates with significantly improved intravenous pressures. These findings introduce qMRV as a potential adjunct to measure venous flow after stenting, and as a plausible tool in the selection and postoperative surveillance of the patient who has cerebral venous sinus stenosis.
View details for DOI 10.1227/NEU.0000000000000771
View details for Web of Science ID 000359981600028
View details for PubMedID 25860429
View details for PubMedCentralID PMC4506216
- Clinical Reasoning: An unusual diagnostic triad NEUROLOGY 2015; 85 (3): E17-E21
Retinal Vascular Changes are a Marker for Cerebral Vascular Diseases
CURRENT NEUROLOGY AND NEUROSCIENCE REPORTS
2015; 15 (7)
The retinal circulation is a potential marker of cerebral vascular disease because it shares origin and drainage with the intracranial circulation and because it can be directly visualized using ophthalmoscopy. Cross-sectional and cohort studies have demonstrated associations between chronic retinal and cerebral vascular disease, acute retinal and cerebral vascular disease, and chronic retinal vascular disease and acute cerebral vascular disease. In particular, certain qualitative features of retinopathy, retinal artery occlusion, and increased retinal vein caliber are associated with concurrent and future cerebrovascular events. These associations persist after accounting for confounding variables known to be disease-causing in both circulations, which supports the potential use of retinal vasculature findings to stratify individuals with regards to cerebral vascular disease risk.
View details for DOI 10.1007/s11910-015-0561-1
View details for Web of Science ID 000356253200003
View details for PubMedID 26008809
View details for PubMedCentralID PMC4743651
Improvement in venous outflow following superior sagittal sinus decompression after a gunshot wound to the head: case report
JOURNAL OF NEUROSURGERY
2015; 123 (1): 81-85
The most commonly described indications for surgical management of closed depressed skull fractures are hematoma evacuation and repair of extensive cosmetic deformity. Venous sinus injury, which occurs in a subset of depressed skull fractures, is not typically listed as an indication for surgical treatment due to the potential for major venous hemorrhage associated with surgery near these structures. However, if patients exhibit signs and symptoms of intracranial hypertension and radiographic findings demonstrate sinus compromise, surgical elevation of the depressed skull fragments is indicated. The authors present the case of a 25-year-old woman with a depressed skull fracture secondary to a gunshot wound with symptomatic compromise in venous outflow of the posterior one-third of the superior sagittal sinus. The patient was treated with surgical decompression via bilateral craniectomy along with intracranial pressure-lowering medical therapy and had almost full resolution of her presenting symptoms with documented improvement in flow through the superior sagittal sinus. While the use of surgical treatment for these types of injuries is highly debated, the authors demonstrate here that safe, effective surgical management of these patients is possible and that surgical decompression should always be considered in the case of symptomatic venous sinus flow obstruction.
View details for DOI 10.3171/2014.10.JNS141349
View details for Web of Science ID 000356981200011
View details for PubMedID 25839927
View details for PubMedCentralID PMC4490082
The Photopic Negative Response in Idiopathic Intracranial Hypertension
INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE
2015; 56 (6): 3709-3714
To evaluate the photopic negative response (PhNR) as an index of retinal ganglion cell (RGC) function in idiopathic intracranial hypertension (IIH).Amplitude and implicit time of the PhNR, as elicited by full-field, brief-luminance flashes, was measured in IIH (n = 10) and visually normal control (n = 15) subjects. Visual function was assessed in IIH subjects using standard automated perimetry mean deviation (SAP-MD) scores. Optic nerve structure was evaluated using the Frisén papilledema grading scale (FPG). Macula ganglion cell complex volume (GCCV) was extracted from optical coherence tomography images to assess RGC loss.Median PhNR amplitude was significantly lower in IIH subjects compared with control subjects (P = 0.015, Mann-Whitney Rank Sum [MW]), but implicit time was similar (P = 0.54, MW). In IIH subjects, PhNR amplitude and SAP-MD were correlated (Pearson's r = 0.78, P = 0.008). Ganglion cell complex volume was correlated with both SAP-MD (r = 0.72, P = 0.019) and PhNR amplitude (r = 0.77, P = 0.009). Multivariate linear regression models demonstrated that the correlation between GCCV and PhNR amplitude was improved by accounting for FPG in the model (r = 0.94, P < 0.0001), but the correlation between GCCV and SAP-MD was not (r = 0.74, P = 0.009).Photopic negative response amplitude, which can be decreased in IIH subjects, correlates well with a clinical measure of visual function (SAP-MD). In multivariate models, it correlated with both an imaging measure of chronic ganglion cell injury (GCCV) and a clinical measure of acute optic nerve head pathology (FPG). Further studies are needed to determine the clinical utility of PhNR as a marker for diagnosis and monitoring of IIH.
View details for DOI 10.1167/iovs.15-16586
View details for Web of Science ID 000357740200030
View details for PubMedID 26047172
View details for PubMedCentralID PMC4466812
Staphylococcus aureus Infection of the Optic Nerve
JOURNAL OF NEURO-OPHTHALMOLOGY
2015; 35 (1): 48-50
A 71-year-old woman presented with painful vision loss in the right eye followed by ophthalmoplegia. Magnetic resonance imaging demonstrated optic nerve sheath enlargement and enhancement. Biopsy of the optic nerve sheath revealed purulent and necrotic material that was positive for methicillin-sensitive Staphylococcus aureus. The patient underwent enucleation of the right eye and was treated with systemic antibiotics with clinical stabilization. Imaging, pathological and treatment aspects of optic nerve sheath abscess are discussed.
View details for DOI 10.1097/WNO.0000000000000190
View details for Web of Science ID 000350462300015
View details for PubMedID 25383588
View details for PubMedCentralID PMC4456004
- Proceedings of the 35th Annual Upper Midwest Neuro-Ophthalmology Group Meeting, July 25, 2014, Chicago, Illinois, USA NEURO-OPHTHALMOLOGY 2015; 39 (1): 46-47
Alternating Skew Deviation in Association with Anti-Glutamic Acid Decarboxylase Antibodies
2015; 39 (3): 140-143
The presence of an elevated anti-glutamic acid decarboxylase (GAD) antibody level has been associated with a number of eye movement abnormalities, as well as other findings including cerebellar ataxia and insulin dependent diabetes mellitus. Skew deviation in association with anti-GAD antibodies has not been previously reported. Here we report a case of alternating skew deviation along with cerebellar-brainstem signs in a patient with an elevated anti-GAD antibody titer. Follow-up neurologic evaluation after treatment with intravenous immunoglobulin revealed improvement in cerebellar-brainstem signs, while ophthalmic evaluation was stable.
View details for DOI 10.3109/01658107.2015.1011755
View details for Web of Science ID 000435883800005
View details for PubMedID 26594078
View details for PubMedCentralID PMC4460821
Clinicopathologic report of ocular involvement in ALS patients with C9orf72 mutation
AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL DEGENERATION
2014; 15 (7-8): 569-580
Our objective was to present clinicopathologic evidence of anterior visual pathway involvement in patients with amyotrophic lateral sclerosis (ALS) secondary to a C9orf72 mutation. Two related patients from an extended pedigree with ALS and GGGGCC hexanucleotide repeat expansion in the C9orf72 gene (C9-ALS) underwent neuro-ophthalmologic examination. Following death and tissue donation of the younger ALS patient, histopathologic examination of the retina, optic nerve and central nervous system (CNS) was performed. Ophthalmologic examination revealed contrast sensitivity impairment in the younger C9-ALS patient. Immunohistochemistry performed on this patient's donor tissue demonstrated p62-positive, pTDP43-negative perinuclear inclusions in the inner nuclear layer of the retina and CNS. Further colocalization with GLT-1 and recoverin suggested that the majority of retinal p62-positive inclusions are found within cone bipolar cells as well as some amacrine and horizontal cells. In conclusion, this is the first report that identifies disease-specific pathologic inclusions in the anterior visual pathway of a patient with a C9orf72 mutation. Cone bipolar cell involvement within the inner nuclear layer of the retina may explain the observed subtle visual function deficiencies in this patient. Further clinical and histopathologic studies are needed to fully characterize a larger population of C9-ALS patients and explore these findings in other forms of ALS.
View details for DOI 10.3109/21678421.2014.951941
View details for Web of Science ID 000346926100016
View details for PubMedID 25319030
View details for PubMedCentralID PMC4327840
A school of red herring
SURVEY OF OPHTHALMOLOGY
2014; 59 (6): 664-670
A 66-year-old man with chronic kidney disease presented with painless unilateral vision loss and bilateral optic disk swelling. Vision loss progressed in the affected eye and developed in the fellow eye. Evaluation for infectious, neoplastic, ischemic, and inflammatory disorders was unrevealing. High-dose corticosteroids appeared to stabilize his vision temporarily. Eventually he was diagnosed with papilledema in the setting of transverse venous sinus stenosis and dural arteriovenous fistula. His papilledema resolved, and his vision improved following stenting of the venous sinus stenosis and embolization of the fistula.
View details for DOI 10.1016/j.survophthal.2014.01.010
View details for Web of Science ID 000343967600010
View details for PubMedID 24913331
View details for PubMedCentralID PMC4207736
Orbital Congestion Complicating Treatment of Cerebral Vascular Anomalies
2014; 82 (1-2): 239.e13-7
The decision between conservative management and invasive treatment of juxtaorbital intracranial vascular anomalies can be challenging. Whereas arteriovenous malformations (AVMs) can lead to vision loss and are potentially life-threatening if they rupture, invasive endovascular and surgical procedures also carry risks.Two patients developed blinding orbital congestion soon after they were treated successfully for cerebral vascular anomalies. The first patient, a 36 year-old man, underwent partial embolization followed by resection of a congenital frontal-lobe AVM. The second patient, a 62 year-old woman, underwent embolization of a periorbital/skull base dural arteriovenous malformation. After intervention, both patients developed unilateral vision loss proptosis, chemosis, complete ophthalmoplegia, and increased intraocular pressure. The first patient suffered from acute orbital compartment syndrome in the absence of any acute localized hemorrhage or thrombosis. The second patient experienced refractory acute glaucoma from orbital congestion, secondary hyphema, and angle closure caused by superior ophthalmic vein and cavernous sinus thromboses.These cases highlight the potential for orbital congestion to worsen acutely after invasive treatment of juxtaorbital cerebral vascular anomalies and suggest diverse mechanisms of resultant visual and orbital compromise. The first case represents the first report of orbital compartment syndrome after resection of a congenital AVM.
View details for DOI 10.1016/j.wneu.2013.01.093
View details for Web of Science ID 000342911400148
View details for PubMedID 23369940
View details for PubMedCentralID PMC3766429
Retinal Vessel Diameter Assessment in Papilledema by Semi-Automated Analysis of SLO Images: Feasibility and Reliability
INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE
2014; 55 (4): 2049-2054
To report feasibility and reliability of a semi-automated image analysis method for retinal vessel diameter measurements in subjects with papilledema before and after treatment.Scanning laser ophthalmoscopy (SLO) was performed in seven normal, five pseudopapilledema, and seven papilledema subjects. In four papilledema subjects, SLO was performed both before and following treatment. Two observers measured diameters of superior and inferior retinal arteries and veins from SLO images using two methods: manual analysis and semi-automated customized analysis. Vessel measurements were compared between observers and between image analysis methods. Retinal vein and artery diameters for each subject were compared between papilledema, pseudopapilledema, and normal subjects, and before and following treatment for papilledema subjects.Interobserver reliability was 0.97 (Pearson's correlation, r) and 0.90 for semi-automated and manual measurements, respectively. Correlation coefficient of manual and semi-automated measurements was 0.85. Retinal vein diameter in papilledema subjects was larger than in pseudopapilledema and normal subjects (P = 0.03, 0.04, Mann-Whitney). Papilledema subjects had a decrease in retinal vein diameter following treatment for and resolution of papilledema (P = 0.04, Wilcoxon signed rank). Retinal artery diameters were not significantly different between papilledema and pseudopapilledema or normal groups, and did not significantly change following papilledema treatment.A feasible and reliable semi-automated image analysis method for measurement of retinal artery and vein diameters from SLO images of elevated optic nerves is reported. Further studies are needed to determine the clinical utility of retinal vein diameter measurements as a marker for diagnosis and treatment of papilledema.
View details for DOI 10.1167/iovs.13-13621
View details for Web of Science ID 000335913100005
View details for PubMedID 24609623
View details for PubMedCentralID PMC3979275
Association of Race/Ethnicity With Visual Outcomes Following Acute Optic Neuritis An Analysis of the Optic Neuritis Treatment Trial
2014; 132 (4): 421-427
IMPORTANCE Retrospective studies have demonstrated disparate outcomes following acute optic neuritis in individuals of African descent compared with individuals of white race/ethnicity. However, published analyses of the prospectively collected Optic Neuritis Treatment Trial (ONTT) data identified no association between worse visual outcomes and black race/ethnicity. OBJECTIVES To investigate the associations of age, sex, and race/ethnicity with visual outcomes following acute optic neuritis through application of longitudinal data analysis techniques to the ONTT data set. DESIGN Secondary analysis of the ONTT (a prospective randomized controlled trial) data set. Our models included effects of treatment (placebo, oral prednisone, or intravenous methylprednisolone), time, and treatment × time interaction, as well as demographic covariates of age, sex, and race/ethnicity. SETTING AND PARTICIPANTS The ONTT data were collected at multiple centers in the United States. Patients of black (n = 58) and white (n = 388) race/ethnicity with acute optic neuritis who enrolled in the ONTT within 8 days of symptom onset were included in analyses. MAIN OUTCOMES AND MEASURES The contrast sensitivity and visual acuity (logMAR) in the affected eye were modeled using 2-stage mixed-effects regression techniques. All available follow-up data from baseline to 15 to 18 years were included. RESULTS The data identified no relationship of age, sex, or treatment with contrast sensitivity or visual acuity outcomes. Race/ethnicity was significantly related to contrast sensitivity (P < .001) and visual acuity (P < .001) during a 15-year period following acute optic neuritis, with black race/ethnicity being associated with worse scores for both. CONCLUSIONS AND RELEVANCE Race/ethnicity seems to be associated with contrast sensitivity and visual acuity outcomes in affected eyes following acute optic neuritis. To our knowledge, this is the largest cohort of black race/ethnicity with acute optic neuritis to be studied and represents the first evidence from a prospectively collected data set to support a hypothesis of race/ethnicity-dependent visual outcomes of acute optic neuritis.
View details for DOI 10.1001/jamaophthalmol.2013.7995
View details for Web of Science ID 000337890500008
View details for PubMedID 24557028
View details for PubMedCentralID PMC4115276
- Proceedings of the 34th Annual Upper Midwest Neuro-Ophthalmology Group Meeting, July 26, 2013, Chicago, Illinois, USA NEURO-OPHTHALMOLOGY 2013; 37 (6): 268-270
Isolated third, fourth, and sixth cranial nerve palsies from presumed microvascular versus other causes: a prospective study.
2013; 120 (11): 2264-2269
To estimate the proportion of patients presenting with isolated third, fourth, or sixth cranial nerve palsy of presumed microvascular origin versus other causes.Prospective, multicenter, observational case series.A total of 109 patients aged 50 years or older with acute isolated ocular motor nerve palsy.Magnetic resonance imaging (MRI) of the brain.Causes of acute isolated ocular motor nerve palsy (presumed microvascular or other) as determined with early MRI and clinical assessment.Among 109 patients enrolled in the study, 22 had cranial nerve III palsy, 25 had cranial nerve IV palsy, and 62 had cranial nerve VI palsy. A cause other than presumed microvascular ischemia was identified in 18 patients (16.5%; 95% confidence interval, 10.7-24.6). The presence of 1 or more vasculopathic risk factors (diabetes, hypertension, hypercholesterolemia, coronary artery disease, myocardial infarction, stroke, and smoking) was significantly associated with a presumed microvascular cause (P = 0.003, Fisher exact test). Vasculopathic risk factors were also present in 61% of patients (11/18) with other causes. In the group of patients who had vasculopathic risk factors only, with no other significant medical condition, 10% of patients (8/80) were found to have other causes, including midbrain infarction, neoplasms, inflammation, pituitary apoplexy, and giant cell arteritis (GCA). By excluding patients with third cranial nerve palsies and those with GCA, the incidence of other causes for isolated fourth and sixth cranial nerve palsies was 4.7% (3/64).In our series of patients with acute isolated ocular motor nerve palsies, a substantial proportion of patients had other causes, including neoplasm, GCA, and brain stem infarction. Brain MRI and laboratory workup have a role in the initial evaluation of older patients with isolated acute ocular motor nerve palsies regardless of whether vascular risk factors are present.
View details for DOI 10.1016/j.ophtha.2013.04.009
View details for PubMedID 23747163
View details for PubMedCentralID PMC3795864
- Parenchymal Anaplastic Astrocytoma Presenting With Visual Symptoms Due to Bilateral Optic Nerve Sheath Involvement JOURNAL OF NEURO-OPHTHALMOLOGY 2013; 33 (3): 313-316
IgG4-related inflammatory pseudotumor of the central nervous system responsive to mycophenolate mofetil
JOURNAL OF THE NEUROLOGICAL SCIENCES
2012; 318 (1-2): 31-35
Orbital apex and skull base masses often present with neuro-ophthalmic signs and symptoms. Though the localization of these syndromes and visualization of the responsible lesion on imaging is typically straightforward, definitive diagnosis usually relies on biopsy. Immunohistochemistry is important for categorization and treatment planning. IgG4-related disease is emerging as a pathologically defined inflammatory process that can occur in multiple organ systems. We present two patients with extensive inflammatory mass lesions of the central nervous system with immunohistochemistry positive for IgG4 and negative for ALK-1 as examples of meningeal based IgG4-related inflammatory pseudotumors. In both patients, there was treatment response to mycophenolate mofetil.
View details for DOI 10.1016/j.jns.2012.04.010
View details for Web of Science ID 000305668500004
View details for PubMedID 22546342
View details for PubMedCentralID PMC3366053
De Novo Malignant Optic Chiasm Glioma: Initial Clinical Response to Steroids
2012; 36 (2): 59-63
Malignant optic nerve glioma (MONG) is a rare but uniformly fatal disease that remains poorly understood. We describe a notable case of this rare disease occurring in the optic chiasm. Normal brain imaging and normal ophthalmic examination two years prior to diagnosis provide evidence for de novo genesis of MONG in our patient. Early response to steroids highlights the degree to which MONG can initially mimic inflammatory optic neuropathies and chiasmal syndromes. Our case also demonstrates a poor outcome with MONG even with current advanced therapy for glioblastoma including radiotherapy plus concomitant and adjuvant temozolomide (the EORTC/NCIC regimen) and bevacizumab.
View details for DOI 10.3109/01658107.2012.658594
View details for Web of Science ID 000302900900006
View details for PubMedID 24031101
View details for PubMedCentralID PMC3769187
Cross-sectional evaluation of clinical neuro-ophthalmic abnormalities in an amyotrophic lateral sclerosis population
JOURNAL OF THE NEUROLOGICAL SCIENCES
2012; 314 (1-2): 97-101
Ocular motility abnormalities may be a marker of neuro-degeneration beyond motor neurons in amyotrophic lateral sclerosis (ALS). We formally compared clinical neuro-ophthalmic abnormalities in ALS patients and a control population.Patients attending a multidisciplinary ALS clinic (n=63, age 60.8+/-16.4 years) and their caregivers serving as controls (n=37, ages 55.0+/-12.7 years) participated in this cross-sectional study. Visual acuity was assessed. Video recordings of a standardized ocular motility exam including gaze fixation, voluntary saccades, reflex saccades, smooth pursuit, eyelid opening and Bell's phenomenon were rated by two senior neuro-ophthalmologists who were masked to subject group.Visual acuity was lower in ALS patients versus control subjects (OR 0.81 (0.71-0.93), p=0.003, logistic regression). Inter- and intra-rater reliability for ocular motility examination ratings were good (Cohen's Kappa>0.6). Findings observed only in ALS subjects included gaze impersistence (14%, p=0.01), moderately or severely restricted voluntary upgaze (13%, p=0.01), and moderate or severe eyelid opening apraxia (27%, p=0.0002). Accounting for age, moderately or severely saccadic horizontal smooth pursuits distinguished ALS from control subjects (OR 3.6 (1.2-10.9), p=0.02, logistic regression).Clinical findings of decreased visual acuity, gaze impersistence, voluntary upgaze restriction, eyelid opening apraxia, and saccadic horizontal smooth pursuits are more frequent in patients with ALS than in similar-aged controls. These findings are potential clinical markers of neurodegeneration beyond upper and lower motor neuron disease in ALS. Further study is warranted regarding their application to disease categorization and outcomes assessment.
View details for DOI 10.1016/j.jns.2011.10.016
View details for Web of Science ID 000301273000018
View details for PubMedID 22192877
View details for PubMedCentralID PMC3441141
Acute Optic Neuropathy Associated With an Intracranial Mass in a Patient With POEMS syndrome
JOURNAL OF NEURO-OPHTHALMOLOGY
2012; 32 (1): 45-47
A 43-year-old man with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes), including long-standing optic disc swelling, had sudden painless vision loss in the left eye. Brain MRI revealed an intracranial mass adjacent to the left optic nerve and enhancement of the optic nerve. The mass decreased in size following chemotherapy for myeloma with some recovery of vision. This represents a unique case of optic neuropathy due to presumed plasmacytoma in osteosclerotic IgA myeloma and POEMS syndrome.
View details for DOI 10.1097/WNO.0b013e318234db5d
View details for Web of Science ID 000300607000010
View details for PubMedID 21956018
View details for PubMedCentralID PMC3725956
Asymmetric bilateral demyelinating optic neuropathy from tacrolimus toxicity
JOURNAL OF THE NEUROLOGICAL SCIENCES
2011; 301 (1-2): 112-115
To report the first histopathologic description of optic nerve demyelination from tacrolimus (FK 506) toxicity in the absence of toxic levels of tacrolimus in a patient presenting with asymmetric bilateral visual loss after 5 years of tacrolimus therapy.We report a patient status post cardiac and renal transplantation who developed severe, progressive and asynchronous bilateral visual loss after prolonged treatment with tacrolimus. Orbital MRI showed an enlarged left optic nerve that enhanced with gadolinium.After extensive negative work up, biopsy of one optic nerve was performed. Microscopic analysis showed extensive demyelination in the absence of vasculitis, neoplastic or infectious etiologies. Our patient illustrates that demyelination of the optic nerve causing asynchronous vision loss can be associated with tacrolimus toxicity in the absence of toxic drug levels.
View details for DOI 10.1016/j.jns.2010.10.014
View details for Web of Science ID 000291330500024
View details for PubMedID 21112060
Visual and Systemic Outcomes in Pediatric Ocular Myasthenia Gravis
AMERICAN JOURNAL OF OPHTHALMOLOGY
2010; 150 (4): 453-459
To evaluate visual and systemic outcomes in pediatric patients with purely ocular myasthenia gravis (OMG) treated at the Children's Hospital of Philadelphia.Retrospective chart review.Pediatric patients with OMG seen at a single institution over a 16-year period with a minimum follow-up of 1 year were reviewed. Associations of demographic and clinical characteristics with disease resolution, amblyopia, and development of generalized symptoms of myasthenia gravis were analyzed.Thirty-nine patients were identified, with a mean age of 5.4 ± 4.8 years and mean follow-up of 4.8 ± 4.3 years. Fifteen patients were treated with pyridostigmine only, 19 (49%) also received steroids, and 15 (38%) underwent thymectomy. Four patients (10%) received steroid-sparing immunosuppressive therapy. Resolution occurred in 10 patients, and generalized symptoms eventually occurred in 9 patients. Although 10 patients were treated for amblyopia, only 1 had amblyopia at the final visit. There was no correlation between sex or age with amblyopia or development of generalized symptoms. Thymectomy, when performed before the onset of generalized symptoms, showed a trend toward protection from the development of generalized symptoms (P = .07).In our series, 24% of patients had disease resolution and 23% had generalized symptoms. Our larger cohort confirms previous findings that treated and untreated pediatric patients with OMG have a relatively low risk of developing generalized symptoms and that related amblyopia is readily reversible. Although our treatments were more aggressive than those previously reported, our rates of amblyopia and development of generalized symptoms are comparable.
View details for DOI 10.1016/j.ajo.2010.05.002
View details for Web of Science ID 000282867500003
View details for PubMedID 20678749
Noninvasive Measurement of Cerebral Blood Flow and Blood Oxygenation Using Near-Infrared and Diffuse Correlation Spectroscopies in Critically Brain-Injured Adults
2010; 12 (2): 173-180
This study assesses the utility of a hybrid optical instrument for noninvasive transcranial monitoring in the neurointensive care unit. The instrument is based on diffuse correlation spectroscopy (DCS) for measurement of cerebral blood flow (CBF), and near-infrared spectroscopy (NIRS) for measurement of oxy- and deoxy-hemoglobin concentration. DCS/NIRS measurements of CBF and oxygenation from frontal lobes are compared with concurrent xenon-enhanced computed tomography (XeCT) in patients during induced blood pressure changes and carbon dioxide arterial partial pressure variation.Seven neurocritical care patients were included in the study. Relative CBF measured by DCS (rCBF(DCS)), and changes in oxy-hemoglobin (DeltaHbO(2)), deoxy-hemoglobin (DeltaHb), and total hemoglobin concentration (DeltaTHC), measured by NIRS, were continuously monitored throughout XeCT during a baseline scan and a scan after intervention. CBF from XeCT regions-of-interest (ROIs) under the optical probes were used to calculate relative XeCT CBF (rCBF(XeCT)) and were then compared to rCBF(DCS). Spearman's rank coefficients were employed to test for associations between rCBF(DCS) and rCBF(XeCT), as well as between rCBF from both modalities and NIRS parameters.rCBF(DCS) and rCBF(XeCT) showed good correlation (r (s) = 0.73, P = 0.010) across the patient cohort. Moderate correlations between rCBF(DCS) and DeltaHbO(2)/DeltaTHC were also observed. Both NIRS and DCS distinguished the effects of xenon inhalation on CBF, which varied among the patients.DCS measurements of CBF and NIRS measurements of tissue blood oxygenation were successfully obtained in neurocritical care patients. The potential for DCS to provide continuous, noninvasive bedside monitoring for the purpose of CBF management and individualized care is demonstrated.
View details for DOI 10.1007/s12028-009-9305-x
View details for Web of Science ID 000275905800006
View details for PubMedID 19908166
View details for PubMedCentralID PMC2844468
Glazed (Vision) and Confused
SURVEY OF OPHTHALMOLOGY
2010; 55 (2): 169-173
A 60-year-old man presented with vitritis and optic neuropathy in the setting of headaches and behavioral changes. MRI brain revealed bilateral temporal lobe inflammation consistent with limbic encephalitis. He was subsequently diagnosed with small cell lung cancer with a paraneoplastic syndrome characterized by CRMP5 IgG as a cause of his symptoms. His visual symptoms improved markedly after anti-inflammatory therapy and his cognitive symptoms were mildly better following systemic chemotherapy. The clinical presentation, pathophysiology, and therapy of CRMP5 associated paraneoplastic syndromes are discussed.
View details for DOI 10.1016/j.survophthal.2009.03.008
View details for Web of Science ID 000275211700006
View details for PubMedID 19804892
Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis in Children and Adolescents
ANNALS OF NEUROLOGY
2009; 66 (1): 11-18
To report the clinical features of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in patients < or = 18 years old.Information was obtained by the authors or referring physicians. Antibodies were determined by immunocytochemistry and enzyme-linked immunosorbent assay (ELISA) using HEK293 cells ectopically expressing NR1.Over an 8-month period, 81 patients (12 male) with anti-NMDAR encephalitis were identified. Thirty-two (40%) were < or =18 years old (youngest 23 months, median 14 years); 6 were male. The frequency of ovarian teratomas was 56% in women >18 years old, 31% in girls < or =18 years old (p = 0.05), and 9% in girls < or =14 years old (p = 0.008). None of the male patients had tumors. Of 32 patients < or =18 years old, 87.5% presented with behavioral or personality change, sometimes associated with seizures and frequent sleep dysfunction; 9.5% with dyskinesias or dystonia; and 3% with speech reduction. On admission, 53% had severe speech deficits. Eventually, 77% developed seizures, 84% stereotyped movements, 86% autonomic instability, and 23% hypoventilation. Responses to immunotherapy were slow and variable. Overall, 74% had full or substantial recovery after immunotherapy or tumor removal. Neurological relapses occurred in 25%. At the last follow-up, full recovery occurred more frequently in patients who had a teratoma that was removed (5/8) than in those without a teratoma (4/23; p = 0.03).Anti-NMDAR encephalitis is increasingly recognized in children, comprising 40% of all cases. Younger patients are less likely to have tumors. Behavioral and speech problems, seizures, and abnormal movements are common early symptoms. The phenotype resembles that of the adults, although dysautonomia and hypoventilation are less frequent or severe in children. Ann Neurol 2009;66:11-18.
View details for DOI 10.1002/ana.21756
View details for Web of Science ID 000268847600006
View details for PubMedID 19670433
View details for PubMedCentralID PMC2826225
- Clinical Reasoning: A 42-year-old man with sequential monocular visual loss NEUROLOGY 2008; 71 (17): E43-E49
Measurement of vocal fold collision forces during phonation: Methods and preliminary data
JOURNAL OF SPEECH LANGUAGE AND HEARING RESEARCH
2005; 48 (3): 567-576
Forces applied to vocal fold tissue as the vocal folds collide may cause tissue injury that manifests as benign organic lesions. A novel method for measuring this quantity in humans in vivo uses a low-profile force sensor that extends along the length and depth of the glottis. Sensor design facilitates its placement and stabilization so that phonation can be initiated and maintained while it is in place, with minimal interference in vocal fold vibration. In 2 individuals with 1 vibrating vocal fold and 1 nonvibrating vocal fold, peak collision force correlates more strongly with voice intensity than pitch. Vocal fold collision forces in 1 individual with 2 vibrating vocal folds are of the same order of magnitude as in previous studies. Correlations among peak collision force, voice intensity, and pitch were indeterminate in this participant because of the small number of data points. Sensor modifications are proposed so that it can be used to reliably estimate collision force in individuals with 2 vibrating vocal folds and with changing vocal tract conformations.
View details for DOI 10.1044/1092-4388(2005/039)
View details for Web of Science ID 000232286300005
View details for PubMedID 16197273
Modeling mechanical stresses as a factor in the etiology of benign vocal fold lesions
JOURNAL OF BIOMECHANICS
2004; 37 (7): 1119-1124
Vocal fold tissue lesions such as nodules and polyps are thought to develop in response to mechanical stress that occurs during vocal fold collision. Two computational models of vocal fold collision during voice production are used to investigate this hypothesis. A one-dimensional lumped mass model, whose parameters are derived from vocal fold tissue dimensions and material properties, predicts stress perpendicular to the direction of impact (normal stress). A previously published three-dimensional finite element model that incorporates the same dimensions and properties predicts the entire stress tensor. The hypothesis is supported by predictions from the finite element model that three components of normal stress and one component of shear stress are increased during collision in the typical location of lesions (i.e. the center of the superior medial edge of the vocal fold in the middle of the vibrating and contact region). The lumped mass model predicts that mechanical stress is negatively correlated with mucosal thickness (increased by voice warm-up and hydration), is positively correlated with driving force (proportional to voice intensity), and is affected by voice production method. These relationships are consistent with clinical observations of vocal fold lesion risk factors and have implications for improving prevention and treatment of benign vocal fold lesions.
View details for DOI 10.1016/j.jbiomech.2003.11.007
View details for Web of Science ID 000222157500018
View details for PubMedID 15165883
A mechanical model of vocal-fold collision with high spatial and temporal resolutions
ACOUSTICAL SOC AMER AMER INST PHYSICS. 2003: 994-1000
The tissue mechanics governing vocal-fold closure and collision during phonation are modeled in order to evaluate the role of elastic forces in glottal closure and in the development of stresses that may be a risk factor for pathology development. The model is a nonlinear dynamic contact problem that incorporates a three-dimensional, linear elastic, finite-element representation of a single vocal fold, a rigid midline surface, and quasistatic air pressure boundary conditions. Qualitative behavior of the model agrees with observations of glottal closure during normal voice production. The predicted relationship between subglottal pressure and peak collision force agrees with published experimental measurements. Accurate predictions of tissue dynamics during collision suggest that elastic forces play an important role during glottal closure and are an important determinant of aerodynamic variables that are associated with voice quality. Model predictions of contact force between the vocal folds are directly proportional to compressive stress (r2 = 0.79), vertical shear stress (r2 = 0.69), and Von Mises stress (r2 = 0.83) in the tissue. These results guide the interpretation of experimental measurements by relating them to a quantity that is important in tissue damage.
View details for DOI 10.1121/1.1534100
View details for Web of Science ID 000180874900031
View details for PubMedID 12597193