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neuroretinal disorder

Rohan Ameratunga, Mark Donaldson
BACKGROUND: Idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome is a very rare primary retinal vasculitis. It seems to evolve in stages, where there is initially a florid vasculitis associated with aneurysm formation. Neuroretinitis and macula edema are common features. Subsequently, retinal ischemia ensues, leading to neovascularization. If untreated, further sight-threatening complications occur, including traction retinal detachment and secondary glaucoma. METHODS AND RESULTS: Here, we describe a patient with early idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome, who was treated with a combination of systemic immunosuppression and localized photocoagulation...
December 19, 2017: Retinal Cases & Brief Reports
Rebika Dhiman, Digvijay Singh, Shiva P Gantayala, Vaitheeswaran L Ganesan, Pradeep Sharma, Rohit Saxena
BACKGROUND: Neuro-ophthalmology as a specialty is underdeveloped in India. The aim of our study was to determine the spectrum and profile of patients presenting to a tertiary eye care center with neuro-ophthalmic disorders. METHODS: A retrospective hospital-based study was conducted, and records of all patients seen at the neuro-ophthalmology clinic of Dr. Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India, over a 1-year period were retrieved and evaluated...
November 9, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
Michael W Stewart
PURPOSE OF REVIEW: Ophthalmologic conditions were among the earliest described findings in patients with the acquired immunodeficiency syndrome (AIDS). The purpose of this review is to highlight recent changes in the pathophysiology and management of ophthalmologic conditions in patients infected with the human immunodeficiency virus (HIV). RECENT FINDINGS: The introduction of highly active antiretroviral therapy (HAART) in 1996 changed ophthalmologic findings from predominantly acute infectious diseases to chronic, slowly progressive, debilitating conditions...
October 19, 2017: Current Infectious Disease Reports
Fernanda Maria Silveira Souto, José Paulo Cabral de Vasconcellos, Mônica Barbosa de Melo, Edi Lúcia Sartorato, Frederico Castelo Moura
Glaucoma is a neurodegenerative disorder characterized by thinning of neuroretinal rim, enlarged cup-to-disc ratio (CDR) and visual field damage. Although raised intraocular pressure is main risk factor for development of glaucoma, it can occur with consistently normal measurements in the intraocular pressure as normal tension glaucoma (NTG). Enlargement of CDR is a classical sign of glaucoma, but it can also result from non-glaucomatous optic neuropathies such as Leber hereditary optic neuropathy (LHON). We describe a case of LHON with increased CDR, discuss its differential diagnosis with NTG and highlight the reasons for misdiagnoses between these two entities...
April 2017: Neuro-ophthalmology
Karina E Guziewicz, Divya Sinha, Néstor M Gómez, Kathryn Zorych, Emily V Dutrow, Anuradha Dhingra, Robert F Mullins, Edwin M Stone, David M Gamm, Kathleen Boesze-Battaglia, Gustavo D Aguirre
Bestrophinopathies, one of the most common forms of inherited macular degenerations, are caused by mutations in the BEST1 gene expressed in the retinal pigment epithelium (RPE). Both human and canine BEST1-linked maculopathies are characterized by abnormal accumulation of autofluorescent material within RPE cells and bilateral macular or multifocal lesions; however, the specific mechanism leading to the formation of these lesions remains unclear. We now provide an overview of the current state of knowledge on the molecular pathology of bestrophinopathies, and explore factors promoting formation of RPE-neuroretinal separations, using the first spontaneous animal model of BEST1-associated retinopathies, canine Best (cBest)...
May 2017: Progress in Retinal and Eye Research
Diego Torres Dias, Michele Ushida, Roberto Battistella, Syril Dorairaj, Tiago Santos Prata
BACKGROUND: To analyze the most common neurophthalmological conditions that may mimic glaucomatous optic neuropathy and to determine which most often lead to misdiagnosis when evaluated by a glaucoma specialist. METHODS: We reviewed the charts of consecutive patients with optic neuropathies caused by neurophthalmological conditions screened in a single Eye Clinic within a period of 24 months. Within these enrolled patients, we selected the eyes whose fundoscopic appearance could resemble glaucoma based in pre-defined criteria (vertical cup-to-disc ratio ≥0...
January 10, 2017: BMC Ophthalmology
Katherine J Wert, Vinit B Mahajan, Lijuan Zhang, Yuanqing Yan, Yao Li, Joaquin Tosi, Chun Wei Hsu, Takayuki Nagasaki, Kerstin M Janisch, Maria B Grant, MaryAnn Mahajan, Alexander G Bassuk, Stephen H Tsang
Diabetic retinopathy (DR) affects approximately one-third of diabetic patients and, if left untreated, progresses to proliferative DR (PDR) with associated vitreous hemorrhage, retinal detachment, iris neovascularization, glaucoma and irreversible blindness. In vitreous samples of human patients with PDR, we found elevated levels of hypoxia inducible factor 1 alpha (HIF1α). HIFs are transcription factors that promote hypoxia adaptation and have important functional roles in a wide range of ischemic and inflammatory diseases...
2016: Signal Transduction and Targeted Therapy
Nazli Demirkaya, Ferdinand W N M Wit, Thomas J T P van Den Berg, Katherine W Kooij, Maria Prins, Reinier O Schlingemann, Michael D Abramoff, Peter Reiss, Frank D Verbraak
PURPOSE: Loss of neuroretinal structure and function, ascribed to a 'HIV-associated Neuroretinal Disorder' (HIV-NRD), in the absence of ocular opportunistic infections, has been reported in HIV-infected individuals treated with combination antiretroviral therapy (cART). Whether HIV-infected individuals with prolonged well-suppressed infection remain at risk for HIV-NRD, is unknown. METHODS: Ninety-two HIV-infected men with suppressed viremia on cART for at least 12 months (HIV+) and 63 HIV-uninfected, highly comparable, male controls (HIV-), aged at least 45 years, underwent functional measurements of spatial (Pelli Robson contrast sensitivity [PR CS]) and temporal contrast sensitivity (TCS) and straylight, as well as spectral-domain optical coherence tomography analysis measured total and individual retinal layer thickness...
March 2016: Investigative Ophthalmology & Visual Science
Davin C Ashraf, K Patrick May, Gary N Holland, Mark L Van Natta, Albert W Wu, Jennifer E Thorne, Douglas A Jabs
PURPOSE: Some human immunodeficiency virus (HIV)-infected individuals have evidence of optic nerve or retinal dysfunction that manifests as decreased contrast sensitivity, even with good best-corrected visual acuity (BCVA). This condition, termed HIV-related neuroretinal disorder (HIV-NRD), is a risk factor for vision impairment (BCVA <20/40), blindness (BCVA ≤20/200), and increased mortality. We investigated the effect of HIV-NRD on vision-specific quality of life (QOL). DESIGN: Cross-sectional analysis of data from a prospective, observational study...
December 2015: Ophthalmology
Nazli Demirkaya, Ferdinand Wit, Reinier Schlingemann, Franciscus Verbraak
Subtle structural and functional retinal abnormalities, termed 'HIV-associated Neuroretinal Disorder (HIV-NRD)', have been reported in HIV patients receiving combination antiretroviral therapy (cART), without infectious retinitis or any apparent fundus abnormalities otherwise. In this review, we provide an overview of studies investigating HIV-NRD in HIV patients without opportunistic ocular infections in the cART era, and try to elucidate underlying mechanisms and associated risk factors. Most studies focused on patients with severe immune-deficiency and demonstrated that patients with nadir CD4 counts<100 cells/μL are most at risk for neuroretinal damage, with a thinner retinal nerve fiber layer, subtle loss of color vision and/or contrast sensitivity, visual field deficits, and subnormal electrophysiological responses...
October 2015: AIDS Patient Care and STDs
Alfredo Niro, Sabino Strippoli, Giovanni Alessio, Luigi Sborgia, Nicola Recchimurzo, Michele Guida
PURPOSE: To report the clinical features and management of mitogen-activated protein kinase kinase inhibitor-associated ocular side effects in 4 patients with advanced melanoma and a review of literature. DESIGN: Interventional case series. METHODS: Four patients with advanced cutaneous melanoma were treated with a mitogen-activated protein kinase kinase (MEK) inhibitor as single therapy or together with a v-raf murine sarcoma viral oncogene homolog B1 (BRAF) inhibitor...
November 2015: American Journal of Ophthalmology
Hyun-Kyung Cho, Wool Suh, Changwon Kee
PURPOSE: To investigate the visual and structural prognosis of untreated initial non-glaucomatous fellow eyes of unilateral normal tension glaucoma (NTG) patients. METHODS: In this retrospective observational cohort study, 50 NTG patients with unilateral visual field (VF) loss and no VF defect, retinal nerve fiber layer (RNFL) defect or neuroretinal rim (NRR) notching in the fellow eyes, and those who had initial non-glaucomatous fellow eyes untreated were included...
September 2015: Graefe's Archive for Clinical and Experimental Ophthalmology
Nazli Demirkaya, Sophie Cohen, Ferdinand W N M Wit, Michael D Abramoff, Reinier O Schlingemann, Taco W Kuijpers, Peter Reiss, Dasja Pajkrt, Frank D Verbraak
PURPOSE: Subtle structural and functional neuroretinal changes have been described in human immunodeficiency virus (HIV)-infected adults without retinitis treated with combination antiretroviral therapy (cART). However, studies on this subject in HIV-infected children are scarce. This study aimed to assess the presence of (neuro)retinal functional and structural differences between a group of perinatally HIV-infected children on cART and age-, sex-, ethnicity-, and socioeconomically matched healthy controls...
June 2015: Investigative Ophthalmology & Visual Science
Samar Younes, Meriem Abdellaoui, Fadoua Zahir, Idriss Benatiya, Hicham Tahri
Neuroretinitis (NR) is an inflammatory disorder characterized by optic disc edema and subsequent formation of a macular star. We present a case of a 33 year old woman patient admitted for a progressive bilateral visual loss since two weeks. Fundus examination showed bilateral stellate neuroretinitis. Physical examination revealed a malignant hypertension of 210/150 mmHg. Magnetic resonance imaging identified a left suprarenal mass, whereas urinary catecholamine level was abnormally high which supported a diagnosis of pheochromocytoma...
2015: Pan African Medical Journal
Immanuel P Seitz, Ahmad Zhour, Susanne Kohl, Pablo Llavona, Tobias Peter, Barbara Wilhelm, Eberhart Zrenner, Marius Ueffing, Karl Ulrich Bartz-Schmidt, M Dominik Fischer
PURPOSE: Choroideremia (CHM) is a X-chromosomal disorder leading to blindness by progressive degeneration of choroid, retinal pigment epithelium (RPE), and retinal neurons. A current clinical gene therapy trial (NCT01461213) showed promising safety and efficacy data in a carefully selected patient population. The present study was performed to shed light on pre-treatment characteristics of a larger cohort of CHM patients using a high resolution multi-modal approach. METHODS: In a retrospective cross-sectional study, data from 58 eyes of 29 patients with clinically confirmed CHM were analysed including best-corrected visual acuity (BCVA), refractive error, spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), perimetry, and tonometry...
December 2015: Graefe's Archive for Clinical and Experimental Ophthalmology
Manuel Gonzalez de la Rosa, Marta Gonzalez-Hernandez, Silvia Alayon
PURPOSE: To compare direct morphologic measurements with the ones deduced from functional tests. METHODS: A total of 111 normal eyes (examined twice) and 112 consecutive eyes with suspected or confirmed glaucoma were analyzed using Spark strategy (Oculus Easyfield Perimeter) and Cirrus optical coherence tomography (OCT). The threshold values were used to deduce the thickness of the retinal nerve fiber layer (RNFL) in 25 sectors. They were also used to derive a normalized value of the neuroretinal rim area, correcting the influence of the optic disc area size, and expressing the result as a percentage of their normal average relation...
November 2015: European Journal of Ophthalmology
Douglas A Jabs, Lea Drye, Mark L Van Natta, Jennifer E Thorne, Gary N Holland
PURPOSE: Patients with AIDS have an abnormality of retina/optic nerve function, manifested as decreased contrast sensitivity (in the absence of ocular opportunistic infections or media opacity), abnormalities on automated perimetry, and loss of retinal nerve fiber layer, even among those with good visual acuity, termed the "human immunodeficiency virus (HIV) neuroretinal disorder." The objectives of this study were to determine the prevalence, incidence, risk factors, and outcomes of HIV neuroretinal disorder...
April 2015: Ophthalmology
Konstantinos Karampatsas, Himanshu Patel, Sheikh N Basheer, Andrew J Prendergast
A previously well 12-year-old boy presented with a 2-week history of headache, nausea, vomiting and left-sided weakness. He subsequently developed meningism, right abducens nerve palsy, persistent papilloedema and reduced visual acuity in association with a bilateral macular star, consistent with neuroretinitis. Cerebrospinal fluid (CSF) examination indicated chronic meningitis and serological testing confirmed recent Mycoplasma pneumoniae infection, although PCR in CSF was negative. He was treated for aseptic meningitis with ceftriaxone, aciclovir, azithromycin and acetazolamide for intracranial hypertension, with gradual improvement in clinical condition and visual acuity over several weeks...
December 23, 2014: BMJ Case Reports
Neal M Rao, Pradeep S Prasad, Charles C Flippen, Aaron S Wagner, Catherine M Yim, Noriko Salamon, Harry V Vinters
A middle-aged woman who experienced recurrent episodes of unilateral vision loss and eye pain. On presentation, magnetic resonance imaging (MRI) demonstrated left optic nerve enhancement with patchy hyperintensities in the white matter of both frontal lobes and ill-defined enhancement in a lenticulostriate distribution. Ophthalmologic examination revealed left optic disc edema with a macular scar consistent with neuroretinitis. Her subsequent clinical course was notable for 2 episodes of painful vision loss, without associated neurologic symptoms, which resolved with intravenous and oral steroids...
December 2014: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
Masoud Soheilian, Ramin Nourinia, Ali Tavallali, Gholam A Peyman
PURPOSE: To describe an unusual case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome associated with a positive perinuclear antineutrophil cytoplasmic antibody test. METHODS: This is an interventional case report. A 22-year-old man with decreased vision underwent ophthalmoscopic, angiographic, and laboratory evaluation. The left eye underwent laser therapy due to circinate retinopathy and more severe involvement. RESULTS: Extensive systemic workup was negative...
2010: Retinal Cases & Brief Reports
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