Pub Date : 2021-03-04eCollection Date: 2021-01-01DOI: 10.2147/EB.S234073
Brandon S Baksh, Julia Costa Garcia, Anat Galor
Dry eye and migraine are common diseases with large societal and economic burdens that have recently been associated in the literature. This review outlines the link between dry eye and migraine, which may have implications for reducing their respective burdens. We highlight possible shared pathophysiology, including peripheral and central sensitization, as the potential link between dry eye and migraine. Finally, therapies targeting similar pathophysiological mechanisms between dry eye and migraine are discussed.
{"title":"Exploring the Link Between Dry Eye and Migraine: From Eye to Brain.","authors":"Brandon S Baksh, Julia Costa Garcia, Anat Galor","doi":"10.2147/EB.S234073","DOIUrl":"10.2147/EB.S234073","url":null,"abstract":"<p><p>Dry eye and migraine are common diseases with large societal and economic burdens that have recently been associated in the literature. This review outlines the link between dry eye and migraine, which may have implications for reducing their respective burdens. We highlight possible shared pathophysiology, including peripheral and central sensitization, as the potential link between dry eye and migraine. Finally, therapies targeting similar pathophysiological mechanisms between dry eye and migraine are discussed.</p>","PeriodicalId":51844,"journal":{"name":"Eye and Brain","volume":null,"pages":null},"PeriodicalIF":4.4,"publicationDate":"2021-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/b3/e9/eb-13-41.PMC7939506.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25456818","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-01-29eCollection Date: 2021-01-01DOI: 10.2147/EB.S234075
Michael Duan, Jesse Skoch, Brian S Pan, Veeral Shah
Craniosynostosis, a premature fusion of cranial sutures that can be isolated or syndromic, is a congenital defect with a broad, multisystem clinical spectrum. The visual pathway is prone to derangements in patients with craniosynostosis, particularly in syndromic cases, and there is a risk for permanent vision loss when ocular disease complications are not identified and properly treated early in life. Extensive advancements have been made in our understanding of the etiologies underlying vision loss in craniosynostosis over the last 20 years. Children with craniosynostosis are susceptible to interruptions in visual input arising from strabismus, refractive errors, and corneal damage; any of these aberrations can result in understimulation of the visual cortex during childhood neurodevelopment and permanent amblyopia. Elevated intracranial pressure resulting from abnormal cranial shape or volume can lead to papilledema and, ultimately, optic atrophy and vision loss. A pediatric ophthalmologist is a crucial component of the multidisciplinary care team that should be involved in the care of craniosynostosis patients and consistent ophthalmologic follow-up can help minimize the risk to vision posed by such entities as papilledema and amblyopia. This article aims to review the current understanding of neuro-ophthalmological manifestations in craniosynostosis and explore diagnostic and management considerations for the ophthalmologist taking care of these patients.
{"title":"Neuro-Ophthalmological Manifestations of Craniosynostosis: Current Perspectives.","authors":"Michael Duan, Jesse Skoch, Brian S Pan, Veeral Shah","doi":"10.2147/EB.S234075","DOIUrl":"10.2147/EB.S234075","url":null,"abstract":"<p><p>Craniosynostosis, a premature fusion of cranial sutures that can be isolated or syndromic, is a congenital defect with a broad, multisystem clinical spectrum. The visual pathway is prone to derangements in patients with craniosynostosis, particularly in syndromic cases, and there is a risk for permanent vision loss when ocular disease complications are not identified and properly treated early in life. Extensive advancements have been made in our understanding of the etiologies underlying vision loss in craniosynostosis over the last 20 years. Children with craniosynostosis are susceptible to interruptions in visual input arising from strabismus, refractive errors, and corneal damage; any of these aberrations can result in understimulation of the visual cortex during childhood neurodevelopment and permanent amblyopia. Elevated intracranial pressure resulting from abnormal cranial shape or volume can lead to papilledema and, ultimately, optic atrophy and vision loss. A pediatric ophthalmologist is a crucial component of the multidisciplinary care team that should be involved in the care of craniosynostosis patients and consistent ophthalmologic follow-up can help minimize the risk to vision posed by such entities as papilledema and amblyopia. This article aims to review the current understanding of neuro-ophthalmological manifestations in craniosynostosis and explore diagnostic and management considerations for the ophthalmologist taking care of these patients.</p>","PeriodicalId":51844,"journal":{"name":"Eye and Brain","volume":null,"pages":null},"PeriodicalIF":4.4,"publicationDate":"2021-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/da/17/eb-13-29.PMC7853409.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"25332746","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-01-18eCollection Date: 2021-01-01DOI: 10.2147/EB.S293765
Jane W Chan, Noel C Y Chan, Alfredo A Sadun
Glaucoma, a group of diseases characterized by progressive optic nerve degeneration that results in irreversible blindness, can be considered a neurodegenerative disorder of both the eye and the brain. Increasing evidence from human and animal studies have shown that glaucoma shares some common neurodegenerative pathways with Alzheimer's disease (AD) and other tauopathies, such as chronic traumatic encephalopathy (CTE) and frontotemporal dementia. This hypothesis is based on the focal adhesion pathway hypothesis and the spreading hypothesis of tau. Not only has the Apolipoprotein E (APOE) gene been shown to be associated with AD, but also with primary open angle glaucoma (POAG). This review will highlight the relevant literature in the past 20 years from PubMed that show the pathogenic overlap between POAG and AD. Neurodegenerative pathways that contribute to transsynaptic neurodegeneration in AD and other tauopathies might also be similar to those in glaucomatous neurodegeneration.
{"title":"Glaucoma as Neurodegeneration in the Brain.","authors":"Jane W Chan, Noel C Y Chan, Alfredo A Sadun","doi":"10.2147/EB.S293765","DOIUrl":"https://doi.org/10.2147/EB.S293765","url":null,"abstract":"<p><p>Glaucoma, a group of diseases characterized by progressive optic nerve degeneration that results in irreversible blindness, can be considered a neurodegenerative disorder of both the eye and the brain. Increasing evidence from human and animal studies have shown that glaucoma shares some common neurodegenerative pathways with Alzheimer's disease (AD) and other tauopathies, such as chronic traumatic encephalopathy (CTE) and frontotemporal dementia. This hypothesis is based on the focal adhesion pathway hypothesis and the spreading hypothesis of tau. Not only has the Apolipoprotein E (APOE) gene been shown to be associated with AD, but also with primary open angle glaucoma (POAG). This review will highlight the relevant literature in the past 20 years from PubMed that show the pathogenic overlap between POAG and AD. Neurodegenerative pathways that contribute to transsynaptic neurodegeneration in AD and other tauopathies might also be similar to those in glaucomatous neurodegeneration.</p>","PeriodicalId":51844,"journal":{"name":"Eye and Brain","volume":null,"pages":null},"PeriodicalIF":4.4,"publicationDate":"2021-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/cf/07/eb-13-21.PMC7822087.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38798480","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2021-01-08eCollection Date: 2021-01-01DOI: 10.2147/EB.S235238
Ailin Song, Nicholas Johnson, Alexandria Ayala, Atalie C Thompson
Purpose: Although Alzheimer's disease (AD) is a leading cause of dementia worldwide, its clinical diagnosis remains a challenge. Optical coherence tomography (OCT) and OCT with angiography (OCTA) are non-invasive ophthalmic imaging tools with the potential to detect retinal structural and microvascular changes in patients with AD, which may serve as biomarkers for the disease. In this systematic review, we evaluate whether certain OCT and OCTA parameters are significantly associated with AD and mild cognitive impairment (MCI).
Methods: PubMed database was searched using a combination of MeSH terms to identify studies for review. Studies were organized by participant diagnostic groups, type of imaging modality, and OCT/OCTA parameters of interest. Participant demographic data was also collected and baseline descriptive statistics were calculated for the included studies.
Results: Seventy-one studies were included for review, representing a total of 6757 patients (2350 AD, 793 MCI, 2902 healthy controls (HC), and 841 others with a range of other neurodegenerative diagnoses). The mean baseline ages were 72.78±3.69, 71.52±2.88, 70.55±3.85 years for AD, MCI and HC groups, respectively. The majority of studies noted significant structural and functional decline in AD patients when compared to HC. Although analysis of MCI groups yielded more mixed results, a similar pattern of decline was often noted amongst patients with MCI relative to HC. OCT and OCTA measurements were also shown to correlate with established measures of AD such as neuropsychological testing or neuroimaging.
Conclusion: OCT and OCTA show great potential as non-invasive technologies for the diagnosis of AD. However, further research is needed to determine whether there are AD-specific patterns of structural or microvascular change in the retina and optic nerve that distinguish AD from other neurodegenerative diseases. Development of sensitive and specific OCT/OCTA parameters will be necessary before they can be used to detect AD in clinical settings.
{"title":"Optical Coherence Tomography in Patients with Alzheimer's Disease: What Can It Tell Us?","authors":"Ailin Song, Nicholas Johnson, Alexandria Ayala, Atalie C Thompson","doi":"10.2147/EB.S235238","DOIUrl":"10.2147/EB.S235238","url":null,"abstract":"<p><strong>Purpose: </strong>Although Alzheimer's disease (AD) is a leading cause of dementia worldwide, its clinical diagnosis remains a challenge. Optical coherence tomography (OCT) and OCT with angiography (OCTA) are non-invasive ophthalmic imaging tools with the potential to detect retinal structural and microvascular changes in patients with AD, which may serve as biomarkers for the disease. In this systematic review, we evaluate whether certain OCT and OCTA parameters are significantly associated with AD and mild cognitive impairment (MCI).</p><p><strong>Methods: </strong>PubMed database was searched using a combination of MeSH terms to identify studies for review. Studies were organized by participant diagnostic groups, type of imaging modality, and OCT/OCTA parameters of interest. Participant demographic data was also collected and baseline descriptive statistics were calculated for the included studies.</p><p><strong>Results: </strong>Seventy-one studies were included for review, representing a total of 6757 patients (2350 AD, 793 MCI, 2902 healthy controls (HC), and 841 others with a range of other neurodegenerative diagnoses). The mean baseline ages were 72.78±3.69, 71.52±2.88, 70.55±3.85 years for AD, MCI and HC groups, respectively. The majority of studies noted significant structural and functional decline in AD patients when compared to HC. Although analysis of MCI groups yielded more mixed results, a similar pattern of decline was often noted amongst patients with MCI relative to HC. OCT and OCTA measurements were also shown to correlate with established measures of AD such as neuropsychological testing or neuroimaging.</p><p><strong>Conclusion: </strong>OCT and OCTA show great potential as non-invasive technologies for the diagnosis of AD. However, further research is needed to determine whether there are AD-specific patterns of structural or microvascular change in the retina and optic nerve that distinguish AD from other neurodegenerative diseases. Development of sensitive and specific OCT/OCTA parameters will be necessary before they can be used to detect AD in clinical settings.</p>","PeriodicalId":51844,"journal":{"name":"Eye and Brain","volume":null,"pages":null},"PeriodicalIF":4.4,"publicationDate":"2021-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/43/90/eb-13-1.PMC7802785.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38820949","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-11-03eCollection Date: 2020-01-01DOI: 10.2147/EB.S277760
Caberry W Yu, Matthew Yau, Natalie Mezey, Ishraq Joarder, Jonathan A Micieli
Objective: Immune checkpoint inhibitors (ICIs) are novel cancer therapies that may be associated with immune-related adverse events (IRAEs) and come to the attention of neuro-ophthalmologists. This systematic review aims to synthesize the reported ICI-associated IRAEs relevant to neuro-ophthalmologists to help in the diagnosis and management of these conditions.
Methods: A systematic review of the literature indexed by MEDLINE, Embase, CENTRAL, and Web of Science databases was searched from inception to May 2020. Reporting followed the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) guidelines. Primary studies on ICIs and neuro-ophthalmic complications were included. Outcomes included number of cases and incidence of neuro-ophthalmic IRAEs.
Results: Neuro-ophthalmic complications of ICIs occurred in 0.46% of patients undergoing ICI and may affect the afferent and efferent visual systems. Afferent complications include optic neuritis (12.8%), neuroretinitis (0.9%), and giant cell arteritis (3.7%). Efferent complications include myasthenia gravis (MG) (45.0%), thyroid-like eye disease (11.9%), orbital myositis (13.8%), general myositis with ptosis (7.3%), internuclear ophthalmoplegia (0.9%), opsoclonus-myoclonus-ataxia syndrome (0.9%), and oculomotor nerve palsy (0.9%). Pembrolizumab was the most common causative agent for neuro-ophthalmic complications (32.1%). Mortality was highest for MG (19.8%). Most patients (79.8%) experienced improvement or complete resolution of neuro-ophthalmic symptoms due to cessation of ICI and immunosuppression with systemic corticosteroids.
Conclusion: While incidence of neuro-ophthalmic IRAEs is low, clinicians involved in the care of cancer patients must be aware of their presentation to facilitate prompt recognition and management. Collaboration between oncology and neuro-ophthalmology teams is required to effectively manage patients and reduce morbidity and mortality.
{"title":"Neuro-ophthalmic Complications of Immune Checkpoint Inhibitors: A Systematic Review.","authors":"Caberry W Yu, Matthew Yau, Natalie Mezey, Ishraq Joarder, Jonathan A Micieli","doi":"10.2147/EB.S277760","DOIUrl":"10.2147/EB.S277760","url":null,"abstract":"<p><strong>Objective: </strong>Immune checkpoint inhibitors (ICIs) are novel cancer therapies that may be associated with immune-related adverse events (IRAEs) and come to the attention of neuro-ophthalmologists. This systematic review aims to synthesize the reported ICI-associated IRAEs relevant to neuro-ophthalmologists to help in the diagnosis and management of these conditions.</p><p><strong>Methods: </strong>A systematic review of the literature indexed by MEDLINE, Embase, CENTRAL, and Web of Science databases was searched from inception to May 2020. Reporting followed the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) guidelines. Primary studies on ICIs and neuro-ophthalmic complications were included. Outcomes included number of cases and incidence of neuro-ophthalmic IRAEs.</p><p><strong>Results: </strong>Neuro-ophthalmic complications of ICIs occurred in 0.46% of patients undergoing ICI and may affect the afferent and efferent visual systems. Afferent complications include optic neuritis (12.8%), neuroretinitis (0.9%), and giant cell arteritis (3.7%). Efferent complications include myasthenia gravis (MG) (45.0%), thyroid-like eye disease (11.9%), orbital myositis (13.8%), general myositis with ptosis (7.3%), internuclear ophthalmoplegia (0.9%), opsoclonus-myoclonus-ataxia syndrome (0.9%), and oculomotor nerve palsy (0.9%). Pembrolizumab was the most common causative agent for neuro-ophthalmic complications (32.1%). Mortality was highest for MG (19.8%). Most patients (79.8%) experienced improvement or complete resolution of neuro-ophthalmic symptoms due to cessation of ICI and immunosuppression with systemic corticosteroids.</p><p><strong>Conclusion: </strong>While incidence of neuro-ophthalmic IRAEs is low, clinicians involved in the care of cancer patients must be aware of their presentation to facilitate prompt recognition and management. Collaboration between oncology and neuro-ophthalmology teams is required to effectively manage patients and reduce morbidity and mortality.</p>","PeriodicalId":51844,"journal":{"name":"Eye and Brain","volume":null,"pages":null},"PeriodicalIF":4.4,"publicationDate":"2020-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/9f/d1/eb-12-139.PMC7648547.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38587616","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-10-30eCollection Date: 2020-01-01DOI: 10.2147/EB.S268828
Maria Elisa Luís, Diogo Hipólito-Fernandes, Catarina Mota, Diogo Maleita, Catarina Xavier, Tiago Maio, João Paulo Cunha, Joana Tavares Ferreira
Introduction: Human coronavirus (HCoVs) are a group of viruses with recognized neurotropic and neuroinvasive capabilities. The reports on the neurological and ocular findings are increasing day after day and several central and peripheral neurological manifestations are already described. However, none specifically describes the neuro-ophthalmological manifestation of HCoVs. This is the first article specifically reviewing neuro-ophthalmological manifestations of HCoVs infection.
Methods: PubMed and Google Scholar databases were searched using the keywords: coronaviridae, coronavirus, COVID-19, SARS-CoV-2, SARS-CoV-1, MERS, ocular, ophthalmology, ophthalmological, neuro-ophthalmology, neurological, manifestations. A manual search through the reference lists of relevant articles was also performed. There were no restrictions concerning language or study type and publications not yet printed but available online were considered.
Results: Coronavirus eye involvement is not frequent and includes mostly a typical viral follicular conjunctivitis. Recently, retinal anatomical alterations were described using optic coherence tomography. Neuro-ophthalmological symptoms and signs can appear isolated or associated with neurological syndromes. The manifestations include headache, ocular pain, visual impairment, diplopia, and cranial nerve palsies secondary to Miller Fisher syndrome, Guillain-Barré syndrome, or encephalitis, and nystagmus.
Conclusion: Neurological and neuro-ophthalmological syndromes, symptoms, and signs should not be neglected and a complete ophthalmological examination of these patients should be performed to fully describe ocular manifestations related to HCoVs. We believe that major ocular and neuro-ophthalmological manifestations reports lack due to safety issues concerning detailed ophthalmological examination; on the other hand, in a large number of cases, the presence of life-threatening coronavirus disease hinders ocular examination and ophthalmologist's visit to the intensive care unit.
{"title":"A Review of Neuro-Ophthalmological Manifestations of Human Coronavirus Infection.","authors":"Maria Elisa Luís, Diogo Hipólito-Fernandes, Catarina Mota, Diogo Maleita, Catarina Xavier, Tiago Maio, João Paulo Cunha, Joana Tavares Ferreira","doi":"10.2147/EB.S268828","DOIUrl":"https://doi.org/10.2147/EB.S268828","url":null,"abstract":"<p><strong>Introduction: </strong>Human coronavirus (HCoVs) are a group of viruses with recognized neurotropic and neuroinvasive capabilities. The reports on the neurological and ocular findings are increasing day after day and several central and peripheral neurological manifestations are already described. However, none specifically describes the neuro-ophthalmological manifestation of HCoVs. This is the first article specifically reviewing neuro-ophthalmological manifestations of HCoVs infection.</p><p><strong>Methods: </strong>PubMed and Google Scholar databases were searched using the keywords: coronaviridae, coronavirus, COVID-19, SARS-CoV-2, SARS-CoV-1, MERS, ocular, ophthalmology, ophthalmological, neuro-ophthalmology, neurological, manifestations. A manual search through the reference lists of relevant articles was also performed. There were no restrictions concerning language or study type and publications not yet printed but available online were considered.</p><p><strong>Results: </strong>Coronavirus eye involvement is not frequent and includes mostly a typical viral follicular conjunctivitis. Recently, retinal anatomical alterations were described using optic coherence tomography. Neuro-ophthalmological symptoms and signs can appear isolated or associated with neurological syndromes. The manifestations include headache, ocular pain, visual impairment, diplopia, and cranial nerve palsies secondary to Miller Fisher syndrome, Guillain-Barré syndrome, or encephalitis, and nystagmus.</p><p><strong>Conclusion: </strong>Neurological and neuro-ophthalmological syndromes, symptoms, and signs should not be neglected and a complete ophthalmological examination of these patients should be performed to fully describe ocular manifestations related to HCoVs. We believe that major ocular and neuro-ophthalmological manifestations reports lack due to safety issues concerning detailed ophthalmological examination; on the other hand, in a large number of cases, the presence of life-threatening coronavirus disease hinders ocular examination and ophthalmologist's visit to the intensive care unit.</p>","PeriodicalId":51844,"journal":{"name":"Eye and Brain","volume":null,"pages":null},"PeriodicalIF":4.4,"publicationDate":"2020-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/EB.S268828","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38572150","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-10-21eCollection Date: 2020-01-01DOI: 10.2147/EB.S255184
Marcella Nebbioso, Antonietta Moramarco, Alessandro Lambiase, Sandra Giustini, Marco Marenco, Emanuele Miraglia, Pasquale Fino, Chiara Iacovino, Ludovico Alisi
Introduction: Neurofibromatosis type 1 (NF1) is a multisystemic disease caused by the mutation of Nf1 gene located on chromosome 17q11.2. The mutation determines the loss of function of the protein neurofibromin with consequent uncontrolled cellular proliferation. Patients are characterized by a wide range of dermatological, neurological, and ophthalmological symptoms.
Purpose: The aim of the study was to evaluate, through pattern visual evoked potentials (p-VEPs) and frequency doubling technology (FDT) Matrix perimetry, the objective and psychophysical functionality of the optic pathways in a group of NF1 patient.
Methods: The study group consisted of 26 patients affected by NF1 and 17 healthy controls. Each patient underwent a complete ophthalmological examination, p-VEPs with the evaluation of amplitude and latency of the P100 wave, and FDT perimetry, with the evaluation of central sensitivity (CS), mean deviation (MD), pattern standard deviation (PSD) and glaucoma hemifield test (GHT).
Results: NF1 patients showed a statistically significant alteration in the transmission of visual impulse. P-VEPs results highlighted a reduced amplitude and an increased latency of the P100 wave, suggesting an involvement of the visual pathway. Visual field analysis showed a significant reduction in all the observed parameters as well (CS, MD, PSD, and GHT).
Conclusion: The present study showed, in NF1 patients, a qualitative and quantitative alteration in the conduction of stimuli through the visual pathways. The observed alterations are present, although, only at a subclinical level. None of the patients included in the study showed any manifest visual deficit nor had any concomitant pathology that might have affected the outcome of the study. In conclusion, electrophysiological exams and computer perimetry may take part, alongside a wider array of exams, in the differential diagnosis and later monitoring of NF1.
{"title":"Neurofibromatosis Type 1: Ocular Electrophysiological and Perimetric Anomalies.","authors":"Marcella Nebbioso, Antonietta Moramarco, Alessandro Lambiase, Sandra Giustini, Marco Marenco, Emanuele Miraglia, Pasquale Fino, Chiara Iacovino, Ludovico Alisi","doi":"10.2147/EB.S255184","DOIUrl":"10.2147/EB.S255184","url":null,"abstract":"<p><strong>Introduction: </strong>Neurofibromatosis type 1 (NF1) is a multisystemic disease caused by the mutation of <i>Nf1</i> gene located on chromosome 17q11.2. The mutation determines the loss of function of the protein neurofibromin with consequent uncontrolled cellular proliferation. Patients are characterized by a wide range of dermatological, neurological, and ophthalmological symptoms.</p><p><strong>Purpose: </strong>The aim of the study was to evaluate, through pattern visual evoked potentials (p-VEPs) and frequency doubling technology (FDT) Matrix perimetry, the objective and psychophysical functionality of the optic pathways in a group of NF1 patient.</p><p><strong>Methods: </strong>The study group consisted of 26 patients affected by NF1 and 17 healthy controls. Each patient underwent a complete ophthalmological examination, p-VEPs with the evaluation of amplitude and latency of the P100 wave, and FDT perimetry, with the evaluation of central sensitivity (CS), mean deviation (MD), pattern standard deviation (PSD) and glaucoma hemifield test (GHT).</p><p><strong>Results: </strong>NF1 patients showed a statistically significant alteration in the transmission of visual impulse. P-VEPs results highlighted a reduced amplitude and an increased latency of the P100 wave, suggesting an involvement of the visual pathway. Visual field analysis showed a significant reduction in all the observed parameters as well (CS, MD, PSD, and GHT).</p><p><strong>Conclusion: </strong>The present study showed, in NF1 patients, a qualitative and quantitative alteration in the conduction of stimuli through the visual pathways. The observed alterations are present, although, only at a subclinical level. None of the patients included in the study showed any manifest visual deficit nor had any concomitant pathology that might have affected the outcome of the study. In conclusion, electrophysiological exams and computer perimetry may take part, alongside a wider array of exams, in the differential diagnosis and later monitoring of NF1.</p>","PeriodicalId":51844,"journal":{"name":"Eye and Brain","volume":null,"pages":null},"PeriodicalIF":3.1,"publicationDate":"2020-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/d9/9f/eb-12-119.PMC7585783.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38542778","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-10-19eCollection Date: 2020-01-01DOI: 10.2147/EB.S234076
Yosbelkys Martin Paez, Lucy I Mudie, Prem S Subramanian
Purpose: To present a systematic review of the current body of literature surrounding spaceflight associated neuro-ocular syndrome (SANS) and highlight priorities for future research.
Methods: Three major biomedical databases were searched with the following terms: ((neuro ocular) OR ((brain) AND (eye))) AND ((spaceflight) OR (astronaut) OR (microgravity)) AND (ENGLISH[Language]). Once duplicates were removed, 283 papers were left. Articles were excluded if they were not written in English or conference abstracts only. We avoided including review papers which did not provide any new information; however, two reviews on the pathophysiology of SANS were included for completeness. No limitations on date of publication were used. All included entries were then summarized for their contribution to knowledge about SANS.
Results: Four main themes among the publications emerged: papers defining the clinical entity of SANS, its pathophysiology, technology used to study SANS, and publications on possible prevention of SANS. The key clinical features of SANS include optic nerve head elevation, hyperopic shifts, globe flattening, choroidal folds, and increased cerebrospinal fluid (CSF) volume in optic nerve sheaths. Two main hypotheses are proposed for the pathophysiology of SANS. The first being elevated intracranial pressure and the second compartmentalization of CSF to the globe. These hypotheses are not mutually exclusive, and our understanding of the pathophysiology of SANS is still evolving. The use of optical coherence tomography (OCT) has greatly furthered our knowledge about SANS, and with the deployment of OCT to the International Space Station, we now have ability to collect intraflight data. No effective prevention for SANS has been found, although fortunately, even with persistent anatomic and physiologic neuro-ocular changes, any functional impact has been correctable with spectacles.
Conclusion: This is the first systematic review of SANS. Despite the limitations of studying a syndrome that can only occur in a small, discrete population, we present a thorough overview of the literature surrounding SANS and several key areas important for future research are identified.
{"title":"Spaceflight Associated Neuro-Ocular Syndrome (SANS): A Systematic Review and Future Directions.","authors":"Yosbelkys Martin Paez, Lucy I Mudie, Prem S Subramanian","doi":"10.2147/EB.S234076","DOIUrl":"https://doi.org/10.2147/EB.S234076","url":null,"abstract":"<p><strong>Purpose: </strong>To present a systematic review of the current body of literature surrounding spaceflight associated neuro-ocular syndrome (SANS) and highlight priorities for future research.</p><p><strong>Methods: </strong>Three major biomedical databases were searched with the following terms: ((neuro ocular) OR ((brain) AND (eye))) AND ((spaceflight) OR (astronaut) OR (microgravity)) AND (ENGLISH[Language]). Once duplicates were removed, 283 papers were left. Articles were excluded if they were not written in English or conference abstracts only. We avoided including review papers which did not provide any new information; however, two reviews on the pathophysiology of SANS were included for completeness. No limitations on date of publication were used. All included entries were then summarized for their contribution to knowledge about SANS.</p><p><strong>Results: </strong>Four main themes among the publications emerged: papers defining the clinical entity of SANS, its pathophysiology, technology used to study SANS, and publications on possible prevention of SANS. The key clinical features of SANS include optic nerve head elevation, hyperopic shifts, globe flattening, choroidal folds, and increased cerebrospinal fluid (CSF) volume in optic nerve sheaths. Two main hypotheses are proposed for the pathophysiology of SANS. The first being elevated intracranial pressure and the second compartmentalization of CSF to the globe. These hypotheses are not mutually exclusive, and our understanding of the pathophysiology of SANS is still evolving. The use of optical coherence tomography (OCT) has greatly furthered our knowledge about SANS, and with the deployment of OCT to the International Space Station, we now have ability to collect intraflight data. No effective prevention for SANS has been found, although fortunately, even with persistent anatomic and physiologic neuro-ocular changes, any functional impact has been correctable with spectacles.</p><p><strong>Conclusion: </strong>This is the first systematic review of SANS. Despite the limitations of studying a syndrome that can only occur in a small, discrete population, we present a thorough overview of the literature surrounding SANS and several key areas important for future research are identified.</p>","PeriodicalId":51844,"journal":{"name":"Eye and Brain","volume":null,"pages":null},"PeriodicalIF":4.4,"publicationDate":"2020-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/EB.S234076","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38542777","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2020-07-16eCollection Date: 2020-01-01DOI: 10.2147/EB.S260823
Peter S Spencer
Purpose: To reexamine the etiology of a unique retinal pathology (linear and vermiform sub-retinal tubular structures) described among subjects with and without neurodegenerative disease in former high-incidence foci of Western Pacific amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS/PDC) in Guam (USA) and the Kii peninsula of Honshu island (Japan).
Methods: Analysis of published and unpublished reports of 1) ALS/PDC and the retinal and cerebellar pathology associated therewith and 2) exogenous neurotoxic factors associated with ALS/PDC and the developing retina and cerebellum.
Results: ALS/PDC retinal and cerebellar pathology matches persistent retinal and cerebellar dysplasia found in laboratory animals given single in utero or postnatal systemic treatment with cycasin, the principal neurotoxic component in the seed of cycad plants traditionally used for food (Guam) or oral medicine (Kii-Japan), both of which have been linked to the human neurodegenerative disease.
Conclusion: ALS/PDC-associated retinal and cerebellar dysplasia could arise from in utero exposure to methylazoxymethanol, the genotoxic metabolite of cycasin that results from maternal ingestion of this azoxyglucoside. These results support the environmental toxic etiology of retinal and brain pathology in ALS/PDC.
{"title":"Etiology of Retinal and Cerebellar Pathology in Western Pacific Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex.","authors":"Peter S Spencer","doi":"10.2147/EB.S260823","DOIUrl":"https://doi.org/10.2147/EB.S260823","url":null,"abstract":"<p><strong>Purpose: </strong>To reexamine the etiology of a unique retinal pathology (linear and vermiform sub-retinal tubular structures) described among subjects with and without neurodegenerative disease in former high-incidence foci of Western Pacific amyotrophic lateral sclerosis and parkinsonism-dementia complex (ALS/PDC) in Guam (USA) and the Kii peninsula of Honshu island (Japan).</p><p><strong>Methods: </strong>Analysis of published and unpublished reports of 1) ALS/PDC and the retinal and cerebellar pathology associated therewith and 2) exogenous neurotoxic factors associated with ALS/PDC and the developing retina and cerebellum.</p><p><strong>Results: </strong>ALS/PDC retinal and cerebellar pathology matches persistent retinal and cerebellar dysplasia found in laboratory animals given single in utero or postnatal systemic treatment with cycasin, the principal neurotoxic component in the seed of cycad plants traditionally used for food (Guam) or oral medicine (Kii-Japan), both of which have been linked to the human neurodegenerative disease.</p><p><strong>Conclusion: </strong>ALS/PDC-associated retinal and cerebellar dysplasia could arise from in utero exposure to methylazoxymethanol, the genotoxic metabolite of cycasin that results from maternal ingestion of this azoxyglucoside. These results support the environmental toxic etiology of retinal and brain pathology in ALS/PDC.</p>","PeriodicalId":51844,"journal":{"name":"Eye and Brain","volume":null,"pages":null},"PeriodicalIF":4.4,"publicationDate":"2020-07-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/EB.S260823","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38239300","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Purpose: An immediate neuroimaging investigation in patients with isolated oculomotor nerve palsy (ONP) remains controversial. We aimed to develop a clinical prediction score to determine whether or not acquired isolated ONP patients require prompt imaging.
Methods: A retrospective study was performed. Demographic data and clinical presentations were collected to determine predictive factors favored for early brain imaging using multivariate logistic regression analysis.
Results: Ninety-seven eyes of 96 patients diagnosed with isolated ONP were included. Forty-one eyes (42.3%) were caused by ischemia, while the other 56 eyes (57.7%) were caused by non-ischemic etiologies, namely aneurysm (n = 22), trauma (n = 18), inflammation (n = 5), tumor (n = 4), and others (n = 7). Eighty-two eyes (84.5%) had undergone neuroimaging study due to initially suspected non-ischemic causes. Only 36 (43.9%) revealed concordant diagnosis. The potential clinical predictors favored for neuroimaging using multivariate logistic regression analysis were age 10‒50 years (adjusted odds ratio [aOR] 9.01, 95% CI: 1.25‒64.8), age 51‒70 years (aOR 1.71, 95% CI: 0.46‒6.35), history of head trauma (aOR 7.14, 95% CI: 1.19‒42.9), absence of vascular risk factors (aOR 3.85, 95% CI: 1.23‒12.1), and poor pupillary response (aOR 6.96, 95% CI: 1.99‒24.3). The predictor scores with an area under the ROC curve of 0.852 were 4, 1, 4, 3, and 4, respectively. The optimum cut-point was 3 for a sensitivity of 96% and specificity of 44%.
Conclusion: Patients with acquired isolated ONP should be considered early neuroimaging studies when they are younger than 50 years old, have a history of head trauma, have no history of vascular risk factors, or have poor pupillary reaction.
{"title":"Clinical Prediction Score for Early Neuroimaging in Acquired Isolated Oculomotor Nerve Palsy.","authors":"Juthamat Witthayaweerasak, Natchada Tansuebchueasai, Nipat Aui-Aree","doi":"10.2147/EB.S253305","DOIUrl":"https://doi.org/10.2147/EB.S253305","url":null,"abstract":"<p><strong>Purpose: </strong>An immediate neuroimaging investigation in patients with isolated oculomotor nerve palsy (ONP) remains controversial. We aimed to develop a clinical prediction score to determine whether or not acquired isolated ONP patients require prompt imaging.</p><p><strong>Methods: </strong>A retrospective study was performed. Demographic data and clinical presentations were collected to determine predictive factors favored for early brain imaging using multivariate logistic regression analysis.</p><p><strong>Results: </strong>Ninety-seven eyes of 96 patients diagnosed with isolated ONP were included. Forty-one eyes (42.3%) were caused by ischemia, while the other 56 eyes (57.7%) were caused by non-ischemic etiologies, namely aneurysm (n = 22), trauma (n = 18), inflammation (n = 5), tumor (n = 4), and others (n = 7). Eighty-two eyes (84.5%) had undergone neuroimaging study due to initially suspected non-ischemic causes. Only 36 (43.9%) revealed concordant diagnosis. The potential clinical predictors favored for neuroimaging using multivariate logistic regression analysis were age 10‒50 years (adjusted odds ratio [aOR] 9.01, 95% CI: 1.25‒64.8), age 51‒70 years (aOR 1.71, 95% CI: 0.46‒6.35), history of head trauma (aOR 7.14, 95% CI: 1.19‒42.9), absence of vascular risk factors (aOR 3.85, 95% CI: 1.23‒12.1), and poor pupillary response (aOR 6.96, 95% CI: 1.99‒24.3). The predictor scores with an area under the ROC curve of 0.852 were 4, 1, 4, 3, and 4, respectively. The optimum cut-point was 3 for a sensitivity of 96% and specificity of 44%.</p><p><strong>Conclusion: </strong>Patients with acquired isolated ONP should be considered early neuroimaging studies when they are younger than 50 years old, have a history of head trauma, have no history of vascular risk factors, or have poor pupillary reaction.</p>","PeriodicalId":51844,"journal":{"name":"Eye and Brain","volume":null,"pages":null},"PeriodicalIF":4.4,"publicationDate":"2020-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/EB.S253305","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"38238884","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}