136 results match your criteria loss neuro-ophthalmologic


Neuro-Ophthalmic Phenotype of OPA3.

J Neuroophthalmol 2021 Apr 14. Epub 2021 Apr 14.

Neuro-Ophthalmology Unit (RH-B), Goldschleger Eye Institute Chaim Sheba Medical Center, Tel-Hashomer, Israel; Department of Neurology (GY, SH-B), Sagol Neuroscience Center, Chaim Sheba Medical Center, Tel-Hashomer, Israel; Movement Disorders Clinic and Department of Neurology (GY), Shaare Zedek Medical Center, Jerusalem, Israel; Metabolic Disease Unit Edmond and Lily Safra Children's Hospital (YA), Chaim Sheba Medical Center, Tel-Hashomer, Israel; Pediatric Neurology Unit (BBZ), Edmond and Lily Safra Children's Hospital, Chaim Sheba Medical Center, Tel-Hashomer, Israel; Radiology Department (CH), Chaim Sheba Medical Center, Tel-Hashomer, Israel; and Sackler Faculty of Medicine (RH-B, YA, BBZ, CH, SH-B), Tel-Aviv University, Tel-Aviv, Israel.

Background: Type III 3-methylglutaconic aciduria (OPA 3) is a neuro-ophthalmologic syndrome consisting of early-onset bilateral optic atrophy. Since Costeff described the phenotype of 19 patients in 1989, several reports described approximately 50 patients, but most of them lack details about neuro-ophthalmic phenotype. Our aim was to characterize the clinical neuro-ophthalmic phenotype of this syndrome. Read More

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Neuro-Ophthalmologic Monitoring in the Management of Increased Intracranial Pressure From Leaking Arachnoid Cysts.

J Neuroophthalmol 2021 Mar 12. Epub 2021 Mar 12.

Departments of Ophthalmology (TKD, ML, JDT), Radiology (RRL), and Neurosurgery (COM, JDT), University of Michigan, Ann Arbor, Michigan.

Background: Intracranial arachnoid cysts are common incidental imaging findings. They may rarely rupture, leading to the development of subdural hygromas and high intracranial pressure (ICP). Neurosurgical intervention has been advocated in the past, but recent evidence indicates that most cases resolve spontaneously. Read More

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Patient Harm Due to Diagnostic Error of Neuro-Ophthalmologic Conditions.

Ophthalmology 2021 Mar 11. Epub 2021 Mar 11.

Department of Ophthalmology, Emory University, Atlanta, Georgia; Department of Neurology, Emory University, Atlanta, Georgia. Electronic address:

Purpose: To prospectively examine diagnostic error of neuro-ophthalmic conditions and resultant harm at multiple sites.

Design: Prospective, cross-sectional study.

Participants: A total of 496 consecutive adult new patients seen at 3 university-based neuro-ophthalmology clinics in the United States in 2019 to 2020. Read More

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Neuro-ophthalmologic manifestations of multiple sclerosis other than acute optic neuritis.

Mult Scler Relat Disord 2021 Feb 31;48:102730. Epub 2020 Dec 31.

Neurology Department, Centro Hospitalar de Vila Nova de Gaia/ Espinho, Vila Nova de Gaia, Portugal.

Acute optic neuritis is the most common neuro-opthalmologic manifestation of multiple sclerosis (MS). Treatment with high-dose intravenous corticosteroids accelerates visual recovery, although it has no long-term visual benefit. MS has several others, less common, neuro-ophthalmological manifestations, where corticotherapy may not be the best treatment option. Read More

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February 2021

Color vision in anterior ischemic optic neuropathy.

Am J Ophthalmol Case Rep 2020 Sep 17;19:100823. Epub 2020 Jul 17.

Department of Ophthalmology, Duke University Eye Center, Durham, NC, USA.

Purpose: The non-arteritic form of anterior ischemic optic neuropathy (AION) is the most common acute optic neuropathy among individuals over the age of fifty, yet little is known about how the disorder affects color vision. We tested the hypothesis that color vision correlates with visual acuity in patients with non-arteritic AION. We also evaluated the patterns of visual field loss in a subgroup of patients who manifested relative sparing of color vision. Read More

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September 2020

The Chiasmal Compression Index: An Integrative Assessment Tool for Visual Disturbances in Patients with Pituitary Macroadenomas.

World Neurosurg 2020 11 17;143:e44-e50. Epub 2020 Jun 17.

Department of Neurosurgery, Soroka University Medical Center and Faculty of Health Sciences, Ben Gurion University of the Negev, Beer Sheva, Israel.

Objective: Visual status is routinely evaluated by neuro-ophthalmologic examination and computerized visual field (VF) tests in patients with chiasmal compression secondary to pituitary macroadenoma. Currently, no relevant data exists to accurately quantify the extent of optic apparatus compromise to further guide clinical decision-making. We aimed to assess for a possible quantitative correlation between optic chiasm geometric properties on magnetic resonance imaging (MRI) and VF deficits. Read More

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November 2020

Clinical Characteristics, Management, and Treatment Outcomes of Primary Hypophysitis: A Monocentric Cohort.

Horm Metab Res 2020 Apr 8;52(4):220-227. Epub 2020 Apr 8.

Department of Endocrinology and Metabolism, Hacettepe University Medical School, Ankara, Turkey.

Primary hypophysitis (PH) is a rare autoimmune inflammatory disease of the pituitary gland. The aim of the study was to evaluate clinical characteristics, disease management, and outcomes of cases with PH. Medical records of PH patients admitted to Hacettepe University Hospital between 1999 and 2017 were analyzed retrospectively. Read More

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Bilateral central retinal/ophthalmic artery occlusion and near-complete ophthalmoplegia after bilateral lung transplant.

Am J Ophthalmol Case Rep 2019 Dec 11;16:100569. Epub 2019 Nov 11.

School of Medicine, National University of Ireland, Galway, Ireland.

Purpose: Recognize a rare yet existing risk of severe visual loss as a postoperative complication of bilateral lung transplant.

Observations: A 62-year-old male had undergone bilateral lung transplant for end-stage idiopathic pulmonary fibrosis and emphysema overlap syndrome. The operation was initially off-pump; however, during the left lung transplantation, cardiopulmonary bypass conversion was necessary to maintain intraoperative hemodynamic stability. Read More

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December 2019

Decreased retinal thickness in patients with Alzheimer's disease is correlated with disease severity.

PLoS One 2019 5;14(11):e0224180. Epub 2019 Nov 5.

Department of Neurology, Dankook University College of Medicine, Dankook University Hospital, Cheonan, Korea.

Background And Purpose: The loss of retinal ganglion cells observed in Alzheimer's disease (AD) may be attributable to a neurodegeneration of the neuro-retinal structure. Amnestic mild cognitive impairment (aMCI) has been considered a prodromal stage of AD. We evaluated retinal thicknesses in patients with aMCI and AD compared to healthy controls using spectral-domain optical coherence tomography (OCT) to investigate whether changes in retinal thickness are correlated with the clinical severity of dementia. Read More

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Vertebrobasilar ischemia (VBI) related neuro-ophthalmic syndromes after routine activities involving hyperextension or rotation of the neck (hairdresser syndrome).

Eye (Lond) 2020 05 30;34(5):901-905. Epub 2019 Sep 30.

Baylor College of Medicine, 1 Baylor Plaza, Houston, TX, 77030, USA.

Background: Vertebrobasilar insufficiency (VBI) after rotation or hyperextension of the neck during otherwise routine activities is uncommon "hairdresser syndrome" (HDS). We report three such cases presenting with neuro-ophthalmic complaints (Horner syndrome, ophthalmoplegia, and transient vision loss).

Methods: A retrospective review was performed of the electronic health records of three patients seen in the neuro-ophthalmology clinic of Houston Methodist Hospital with acute neuro-ophthalmological signs after neck hyperextension. Read More

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Clinical and Neuro-ophthalmologic Predictors of Visual Outcome in Idiopathic Intracranial Hypertension.

Neuroophthalmology 2018 Aug 9;42(4):201-208. Epub 2018 Jan 9.

Department of Neurology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

Despite the potential of Idiopathic Intracranial Hypertension (IIH) to cause visual morbidity, limited literature is available focussing on predictors of visual outcome in IIH. This study was planned to assess visual morbidity in patients of IIH in terms of clinical and neuro-ophthalmo- logical parameters. In this prospective study of 40 patients of IIH, neuro-ophthalmological parameters were noted in the form of visual acuity, visual field, contrast sensitivity, Retinal Nerve Fibre Layer thickness, and visual evoked potential. Read More

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Ipilimumab-induced Adenohypophysitis and Orbital Apex Syndrome: Importance of Early Diagnosis and Management.

Neuroophthalmology 2018 Jun 20;42(3):176-181. Epub 2017 Sep 20.

Department of Neurology, University of Illinois College of Medicine Peoria, Peoria, Illinois, USA.

Ipilimumab is a novel anti-melanoma agent known to infrequently cause multi-organ autoimmunity. We report a case of pituitary hypophysitis and orbital inflammation followed by an orbital apex syndrome. A 64-year-old woman with a history of skin melanoma, receiving ipilimumab treatment, was seen for near total loss of vision in the right eye and proptosis. Read More

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Management of non-functioning pituitary adenomas: surgery.

Pituitary 2018 Apr;21(2):145-153

Department of Neurological Surgery, Brigham and Women's Hospital, Harvard Medical School, 60 Fenwood Road, BTM, 4th Floor, Boston, MA, 02115, USA.

Non-functional pituitary adenomas (NFPAs) are benign tumors of the pituitary gland that do not over-secrete hormonal products, therefore, they are generally detected through symptoms of mass effect, including headache, vision loss, or hypopituitarism. There are multiple pathological subtypes of NFPAs, such as null cell adenomas, silent gonadotrophs, silent somatotrophs, silent corticotrophs, and silent subtype 3, all of which can be classified based on immunohistochemical studies and electron microscopy. Despite these numerous pathological subtypes, surgical resection remains the first-line treatment for NFPAs. Read More

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Genotype-phenotype and OCT correlations in Autosomal Dominant Optic Atrophy related to OPA1 gene mutations: Report of 13 Italian families.

J Neurol Sci 2017 Nov 14;382:29-35. Epub 2017 Sep 14.

Unit of Neurology and Neurometabolic Diseases, Department of Medical, Surgical and Neurological Sciences, University of Siena, Siena, Italy.

Mutations in OPA1 are responsible of 32-89% cases of Autosomal Dominant Optic Atrophy (ADOA). OPA1 ADOA usually presents in childhood with bilateral, progressive visual loss due to retinal ganglion cells neurodegeneration, but environmental factors are supposed to influence onset and phenotype. Sixty Italian OPA1 mutations carriers (fifty-two symptomatic), belonging to thirteen families, underwent neuro-ophthalmologic evaluation. Read More

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November 2017

The use of optical coherence tomography in neuro-ophthalmology.

Curr Opin Ophthalmol 2017 Nov;28(6):552-557

aHong Kong Eye Hospital bDepartment of Ophthalmology and Visual Sciences, the Chinese University of Hong Kong, Hong Kong.

Purpose Of Review: In the last decade, with the advances of optical coherence tomography (OCT) technology, different imaging protocols and analysis algorithms have been introduced to maximize the potential of this diagnostic tool in the evaluation of different eye diseases. This review aims to provide an update on these additional features, with respect to the management of a diverse range of neuro-ophthalmologic conditions.

Recent Findings: Macular ganglion cell complex (mGCC) analysis has been shown to be superior to peripapillary retinal nerve fiber layer (pRNFL) analysis in certain settings, such as differentiating Leber's hereditary optic neuropathy from functional visual loss; monitoring neurodegenerative diseases or multiple sclerosis; and predicting visual loss in nonarteritic ischemic optic neuropathy. Read More

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November 2017

[Neuro-ophthalmological conditions: Study of the clinical care pathway].

J Fr Ophtalmol 2017 Sep 15;40(7):580-587. Epub 2017 Jun 15.

Département d'ophtalmologie, DHU vision et handicaps, groupe hospitalier Pitié-Salpêtrière, 43, boulevard de l'Hôpital, 75013, Paris, France. Electronic address:

Introduction: Neuro-ophthalmologic conditions require specialized multidisciplinary management, both medical and surgical, for patients affected by visual loss due to nervous system disease. The primary goal of this study is to define the specificity of neuro-ophthalmology within the realm of visual health. The secondary goal is to review clinical care pathways by studying the organization of management, in terms of accessibility to care and personalization of the care pathway. Read More

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September 2017

Neuro-ophthalmological conditions: Study of the clinical care pathway.

J Fr Ophtalmol 2017 Jun 7;40(6):e169-e175. Epub 2017 Jun 7.

Département d'ophtalmologie, DHU vision et handicaps, groupe hospitalier Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France. Electronic address:

Introduction: Neuro-ophthalmologic conditions require specialized multidisciplinary management, both medical and surgical, for patients affected by visual loss due to nervous system disease. The primary goal of this study is to define the specificity of neuro-ophthalmology within the realm of visual health. The secondary goal is to review clinical care pathways by studying the organization of management, in terms of accessibility to care and personalization of the care pathway. Read More

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The impact of surgical timing on visual outcome in pituitary apoplexy: Literature review and case illustration.

Surg Neurol Int 2017 6;8:16. Epub 2017 Feb 6.

Department of Neurosciences, Division of Neurosurgery, King Faisal Specialist Hospital and Research Center, Riyadh 11211, Saudi Arabia; Alfaisal University, College of Medicine, Riyadh 11533, Saudi Arabia.

Background: Neuro-ophthalmologic signs are common clinical manifestations of pituitary apoplexy. Managing sudden visual loss is critical for achieving a good outcome. The timing of pituitary surgery remains controversial. Read More

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February 2017

Ectopic Suprasellar Thyrotropin-Secreting Pituitary Adenoma: Case Report and Literature Review.

World Neurosurg 2016 Nov 25;95:617.e13-617.e18. Epub 2016 Aug 25.

Department of Ophthalmology, Wuxi Second Hospital Affiliated with Nanjing Medical University, Wu'xi, Jiangsu Province, China.

Background: Ectopic thyroid-stimulating hormone (TSH)-secreting pituitary adenomas are rare and can often be misdiagnosed as primary hyperthyroidism. We present a case of an ectopic suprasellar TSH-secreting pituitary adenoma. A literature review of previously reported ectopic TSH-secreting and suprasellar pituitary adenomas is included to illustrate the clinical characteristics of this disease entity and the diversity of operative approaches to treating ectopic suprasellar pituitary adenomas. Read More

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November 2016

Retinal Ganglion Cell and Inner Plexiform Layer Loss Correlate with Visual Acuity Loss in LHON: A Longitudinal, Segmentation OCT Analysis.

Invest Ophthalmol Vis Sci 2016 Jul;57(8):3872-83

Neuro-Ophthalmology Department Wills Eye Hospital, Departments of Ophthalmology and Neurology, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, Pennsylvania, United States.

Purpose: Describe changes in the retina as vision loss progresses in Leber's Hereditary Optic Neuropathy (LHON) using spectral-domain optical coherence tomography (SD-OCT) autosegmentation, and determine if relationship exists between retinal changes and vision loss.

Methods: From patient records we identified nine LHON patients who underwent periodic neuro-ophthalmologic examinations and high-resolution SD-OCT as part of their care. We describe the impact of LHON progression on each retinal layer, and the relationship between these structural changes and visual acuity using generalized estimating equations and nonparametric tests. Read More

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Comparison of the Pattern of Macular Ganglion Cell-Inner Plexiform Layer Defect Between Ischemic Optic Neuropathy and Open-Angle Glaucoma.

Invest Ophthalmol Vis Sci 2016 Mar;57(3):1011-6

Eye Research Center Farabi Eye Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Purpose: To compare the pattern of macular ganglion cell plus inner plexiform layer (GCIPL) and peripapillary retinal nerve fiber layer (RNFL) thickness changes in moderate to severe primary open-angle glaucoma (POAG) with nonarteritic anterior ischemic optic neuropathy (NAION) using optical coherence tomography (OCT) auto-segmentation.

Methods: A total of 138 eyes (42 eyes with chronic unilateral NAION and their 42 unaffected fellow eyes, 32 eyes of 32 moderate to severe glaucoma patients, and 22 eyes of 22 healthy normal subjects) underwent neuro-ophthalmologic examinations and spectral-domain OCT in a cross-sectional study at a single academic institution. GCIPL and total retinal thicknesses were obtained from 20° by 20° cube scans of the macula centered around the fovea. Read More

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[Research status on the epidemiological characteristics of optic neuritis].

Zhonghua Yan Ke Za Zhi 2015 Dec;51(12):946-9

Department of Epidemiology and Biostatistics, School of Public Health, Peking University, Beijing 100191, China; Email:

Optic neuritis(ON) is one of common neuro-ophthalmologic diseases which cause vision loss in young and middle-aged population. There are obvious differences in the incidence and the epidemiological characteristics among different countries and regions. A number of studies on ON have been carried out at home and abroad. Read More

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December 2015

Visual, Ocular Motor, and Cochleo-Vestibular Loss in Patients With Heteroplasmic, Maternally-Inherited Diabetes Mellitus and Deafness (MIDD), 3243 Transfer RNA Mutation.

J Neuroophthalmol 2016 06;36(2):134-40

Department of Neurology (SC-R), Universidad Nacional Facultad de Medicina, Bogota, Colombia; and Illinois Neurologic Institute (AST, GB, JCK), University of Illinois College of Medicine, Peoria, Illinois.

Background: Mitochondrial encephalopathy, lactic acidosis, and stroke-like symptoms (MELAS) and MIDD (maternally-inherited diabetes mellitus and deafness) are caused by A3243G transfer RNA mutations that affect mitochondrial function. Hearing loss and early onset diabetes mellitus constitute the main MIDD phenotype. Regarding the ophthalmologic manifestations of MIDD, we hypothesized that decreased vestibulo-ocular reflex (VOR) gain in patients with MIDD may contribute to impaired dynamic visual acuity. Read More

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Visual findings as primary manifestations in patients with intracranial tumors.

Int J Ophthalmol 2015 18;8(4):800-3. Epub 2015 Aug 18.

Department of Ophthalmology, İzmir Atatürk Education and Research Hospital, Izmir 35360, Turkey.

Aim: To evaluate the visual findings as primary manifestations in patients with intracranial tumors.

Methods: The medical charts of the patients with intracranial tumors who initially admitted to the Neuro-ophthalmology and Strabismus Department with ocular complaints between August 1999 and December 2012 were reviewed retrospectively. The detailed clinical history and the findings of neuro-ophthalmologic examination were recorded. Read More

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Expanding the clinical and molecular characteristics of PIGT-CDG, a disorder of glycosylphosphatidylinositol anchors.

Mol Genet Metab 2015 Jun-Jul;115(2-3):128-140. Epub 2015 May 1.

Office of the Clinical Director, NHGRI, NIH, Bethesda, MD, USA; NIH Undiagnosed Diseases Program, Common Fund, Office of the Director, NIH, Bethesda, MD, USA.

PIGT-CDG, an autosomal recessive syndromic intellectual disability disorder of glycosylphosphatidylinositol (GPI) anchors, was recently described in two independent kindreds [Multiple Congenital Anomalies-Hypotonia-Seizures Syndrome 3 (OMIM, #615398)]. PIGT encodes phosphatidylinositol-glycan biosynthesis class T, a subunit of the heteropentameric transamidase complex that facilitates the transfer of GPI to proteins. GPI facilitates attachment (anchoring) of proteins to cell membranes. Read More

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Analysis of optic disk color changes in Alzheimer's disease: a potential new biomarker.

Clin Neurol Neurosurg 2015 May 9;132:68-73. Epub 2015 Mar 9.

Ophthalmology Department, Miguel Servet University Hospital, Zaragoza, Spain; Aragones Institute of Health Sciences, Zaragoza, Spain.

Purpose: In the present study, we evaluated changes in the retinal nerve fiber layer (RNFL) and optic disk color (i.e., the level of paleness as an indirect sign of axonal loss) in patients with Alzheimer's disease (AD) compared with healthy controls. Read More

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Distributed abnormalities of brain white matter architecture in patients with dominant optic atrophy and OPA1 mutations.

J Neurol 2015 May 21;262(5):1216-27. Epub 2015 Mar 21.

Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Via Olgettina, 60, 20132, Milan, Italy.

Using advanced MRI techniques, we investigated the presence and topographical distribution of brain grey matter (GM) and white matter (WM) alterations in dominant optic atrophy (DOA) patients with genetically proven OPA1 mutation as well as their correlation with clinical and neuro-ophthalmologic findings. Nineteen DOA patients underwent neurological, neuro-ophthalmologic and brainstem auditory evoked potentials (BAEP) evaluations. Voxel-wise methods were applied to assess regional GM and WM abnormalities in patients compared to 20 healthy controls. Read More

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Functional Loss of the Inner Retina in Childhood Optic Gliomas Detected by Photopic Negative Response.

Invest Ophthalmol Vis Sci 2015 Apr;56(4):2469-74

Department of Ophthalmology, Catholic University of Sacred Heart, Rome, Italy.

Purpose: To determine whether the Ganzfeld ERG photopic negative response (PhNR), an assay of inner retinal activity, is altered in childhood optic glioma (OPG).

Methods: Seventeen pediatric patients with a diagnosis of OPG, established on neuro-ophthalmologic and brain/orbit magnetic resonance imaging (MRI) criteria, were enrolled. The examination protocol included determination of visual acuity (VA), fundus examination, retinal nerve fiber layer (RNFL) measurement with spectral-domain optical coherence tomography (SD-OCT) and photopic ERG. Read More

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Medical management of hereditary optic neuropathies.

Front Neurol 2014 31;5:141. Epub 2014 Jul 31.

UOC Clinica Neurologica, IRCCS Istituto delle Scienze Neurologiche di Bologna, Ospedale Bellaria , Bologna , Italy ; Unità di Neurologia, Dipartimento di Scienze Biomediche e NeuroMotorie (DIBINEM), Università di Bologna , Bologna , Italy.

Hereditary optic neuropathies are diseases affecting the optic nerve. The most common are mitochondrial hereditary optic neuropathies, i.e. Read More

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HOXA1 Mutations are Not Commonly Associated with Non-Syndromic Deafness.

Can J Neurol Sci 2014 Jul;41(4):448-51

Objective: Homozygous homeobox A1 (HOXA1) mutations cause a spectrum of abnormalities in humans including bilateral profound deafness. This study evaluates the possible role of HOXA1 mutations in familial, non-syndromic sensorineural deafness.

Methods: Forty-eight unrelated Middle Eastern families with either consanguinity or familial deafness were identified in a large deafness clinic, and the proband from each family was evaluated by chart review, audiogram, neuroimaging, and HOXA1 sequencing. Read More

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