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Optic nerve atrophy

Ewa Langwinska-Wosko, Marta Skowronska, Tomasz Kmiec, Anna Czlonkowska
BACKGROUND: Mitochondrial membrane protein-associated neurodegeneration (MPAN) is an neurodegeneration with brain iron accumulation (NBIA) subtype with mutation of C19orf12. Optic atrophy is one of the core symptoms in almost all MPAN cases, but the detailed ophthalmologic features of MPAN patients have not yet been described. METHODS: All consecutive symptomatic, gene proven MPAN patients underwent a detailed ophthalmological examination: best corrected visual acuity (BCVA), slit lamp examination, dilated fundus examination, tonometry, optical coherent tomography (OCT) and electrophysiological examinations...
November 15, 2016: Journal of the Neurological Sciences
Min Hee Suh, Linda M Zangwill, Patricia Isabel C Manalastas, Akram Belghith, Adeleh Yarmohammadi, Felipe A Medeiros, Alberto Diniz-Filho, Luke J Saunders, Robert N Weinreb
PURPOSE: To investigate factors associated with dropout of the parapapillary deep retinal layer microvasculature assessed by optical coherence tomography angiography (OCTA) in glaucomatous eyes. DESIGN: Cross-sectional study. PARTICIPANTS: Seventy-one eyes from 71 primary open-angle glaucoma (POAG) patients with β-zone parapapillary atrophy (βPPA) enrolled in the Diagnostic Innovations in Glaucoma Study. METHODS: Parapapillary deep-layer microvasculature dropout was defined as a complete loss of the microvasculature located within the deep retinal layer of the βPPA from OCTA-derived optic nerve head vessel density maps by standardized qualitative assessment...
October 18, 2016: Ophthalmology
Jang-Ho Park, Joon-Ho Ahn, Jun-Bum Park, Soohyun Joe
Charles Bonnet syndrome (CBS) can develop after trans-sphenoidal adenomectomy (TSA); however, the neural mechanisms remain unknown. Sensory deprivation and releasing phenomenon are both hypothetical explanations for this condition; however, there is no definite evidence that strongly supports either supposition. We report the first case of CBS after TSA without optic nerve atrophy. Postoperatively, the patient's vision seemed to be relatively well preserved, apart from the left-side hemianopsia in the right eye...
September 2016: Psychiatry Investigation
Isaac D Bleicher, Mays A El-Dairi
No abstract text is available yet for this article.
October 14, 2016: Journal of Pediatrics
Alexander U Brandt, Elena Meinert-Bohn, Jan Leo Rinnenthal, Hanna Zimmermann, Janine Mikolajczak, Timm Oberwahrenbrock, Sebastian Papazoglou, Caspar F Pfüller, Johann Schinzel, Björn Tackenberg, Friedemann Paul, Katrin Hahn, Judith Bellmann-Strobl
BACKGROUND: The PMP22 gene encodes a protein integral to peripheral myelin. Its deletion leads to hereditary neuropathy with liability to pressure palsies (HNPP). PMP22 is not expressed in the adult central nervous system, but previous studies suggest a role in CNS myelin development. The objective of this study was to identify potential structural and functional alterations in the afferent visual system in HNPP patients. METHODS: Twenty HNPP patients and 18 matched healthy controls (HC) were recruited in a cross-sectional study...
2016: PloS One
Neil K Bansal, Mari Hagiwara, Maria J Borja, James Babb, Sohil H Patel
BACKGROUND AND PURPOSE: Clinical history is known to influence interpretation of a wide range of radiologic examinations. We sought to evaluate the influence of the clinical history on MRI interpretation of optic neuropathy. MATERIALS AND METHODS: 107 consecutive orbital MRI scans were retrospectively reviewed by three neuroradiologists. The readers independently evaluated the coronal STIR sequence for optic nerve hyperintensity and/or atrophy (yes/no) and the coronal post-contrast T1WI for optic nerve enhancement (yes/no)...
September 2016: Heliyon
Yumi Ueki, Grisela Ramirez, Ernesto Salcedo, Maureen E Stabio, Frances Lefcort
Familial dysautonomia (FD) is an autosomal recessive congenital neuropathy that is caused by a mutation in the gene for inhibitor of kappa B kinase complex-associated protein (IKBKAP). Although FD patients suffer from multiple neuropathies, a major debilitation that affects their quality of life is progressive blindness. To determine the requirement for Ikbkap in the developing and adult retina, we generated Ikbkap conditional knockout (CKO) mice using a TUBA1a promoter-Cre (Tα1-Cre). In the retina, Tα1-Cre expression is detected predominantly in retinal ganglion cells (RGCs)...
September 2016: ENeuro
Patrick Yu-Wai-Man, Marcela Votruba, Florence Burté, Chiara La Morgia, Piero Barboni, Valerio Carelli
Mitochondrial optic neuropathies constitute an important cause of chronic visual morbidity and registrable blindness in both the paediatric and adult population. It is a genetically heterogeneous group of disorders caused by both mitochondrial DNA (mtDNA) mutations and a growing list of nuclear genetic defects that invariably affect a critical component of the mitochondrial machinery. The two classical paradigms are Leber hereditary optic neuropathy (LHON), which is a primary mtDNA disorder, and autosomal dominant optic atrophy (DOA) secondary to pathogenic mutations within the nuclear gene OPA1 that encodes for a mitochondrial inner membrane protein...
September 30, 2016: Acta Neuropathologica
Víctor Manuel Asensio-Sánchez, Javier Foncubierta
INTRODUCTION: Most pituitary adenomas are clinically inactive. In patients with long-standing compression of the optic chiasm, ganglion cells may undergo axonal degeneration. Spectral domain optical coherence tomography (SD-OCT) is able to identify retinal nerve fiber layer (RNFL) and ganglion cell loss in the retina. We present a case in which SD-OCT was used to diagnose an asymptomatic pituitary macroadenoma. CLINICAL CASE: A 48-year-old female presented with progressive vision loss in both eyes...
2016: International Medical Case Reports Journal
Hiroshi Kuroda, Yoshiyuki Mukai, Shuhei Nishiyama, Takayuki Takeshita, Maki Tateyama, Atsushi Takeda, Masashi Aoki
Thallium intoxication was reported in cases with accidental ingestion, suicide attempt, and criminal adulteration. Reported cases were mostly one-time ingestion, therefore, the clinical course of divisional ingestion has not been fully known. Here, we report a case with two-step thallium intoxication manifesting as tardily accelerated neurologic deterioration. A 16-year-old adolescent was cryptically poisoned with thallium sulfate twice at an interval of 52days. After the first ingestion, neurologic symptoms including visual loss, myalgia, and weakness in legs developed about 40days after the development of acute gastrointestinal symptoms and alopecia...
September 28, 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
J D Unterlauft, H Tegetmeyer
Background: With a cooperative patient, examination of the optic nerve head using optical coherence tomography (OCT) is fast and easy to perform and facilitates identification and monitoring of different pathological changes in the optic nerve head. Materials and Methods: Characteristic findings and scanning options are illustrated using case examples to simplify recognition of infrequent diseases of the optic nerve head and to facilitate treatment decisions using OCT results. Results: Pathological changes and characteristic OCT findings are shown for glaucoma, for different anomalies of the optic nerve head, for non-glaucomatous optic atrophies and for optic disc swelling for different reasons...
September 27, 2016: Klinische Monatsblätter Für Augenheilkunde
Jun-Feng Mao, Xiao-Bo Xia, Xiang-Bo Tang, Xue-Yong Zhang, Dan Wen
AIM: To investigate clinical features of optic nerve sheath meningioma (ONSM) that was misdiagnosed, and to find methods to reduce the misdiagnoses. METHODS: Retrospective series study. Twenty-five misdisgnosed patients with unilateral ONSM were collected from Jan. 2008 to Jan. 2015 and the clinical records reviewed. RESULTS: Patients were misdiagnosed with acute papillitis most frequently (n=17), immediately followed by optic atrophy (n=8), ischemic optic neuropathy (n=5), acute retrobulbar optic neuritis (n=5), optic disc vasculitis (n=3)...
2016: International Journal of Ophthalmology
Inês Dinis Aires, António Francisco Ambrósio, Ana Raquel Santiago
Glaucoma, a leading cause of blindness worldwide, is a degenerative disease characterized by retinal ganglion cell (RGC) loss and optic nerve atrophy. Elevated intraocular pressure (IOP) is a main risk factor for onset and progression of the disease. Since increased IOP is the only modifiable risk factor, relevant models for glaucoma would comprise RGC and optic nerve damage triggered by ocular hypertension. Animal models of glaucoma have greatly contributed to the understanding of the molecular mechanisms of this pathology, and they have also facilitated the development of new pharmacological interventions...
September 13, 2016: Ophthalmic Research
Michael M Altaweel, Sapna S Gangaputra, Jennifer E Thorne, James P Dunn, Susan G Elner, Glenn J Jaffe, Rosa Y Kim, P Kumar Rao, Susan B Reed, John H Kempen
BACKGROUND: The objective of this study is to describe a system for color photograph evaluation in uveitis and report baseline morphologic findings for the Multicenter Uveitis Steroid Treatment (MUST) Trial. Four-hundred seventy-nine eyes of 255 subjects with intermediate, posterior, and panuveitis had stereoscopic color fundus photographs obtained by certified photographers and evaluated by certified graders using standardized procedures to evaluate morphologic characteristics of uveitis...
December 2016: Journal of Ophthalmic Inflammation and Infection
Jonathan J Miner, Abdoulaye Sene, Justin M Richner, Amber M Smith, Andrea Santeford, Norimitsu Ban, James Weger-Lucarelli, Francesca Manzella, Claudia Rückert, Jennifer Govero, Kevin K Noguchi, Gregory D Ebel, Michael S Diamond, Rajendra S Apte
Zika virus (ZIKV) is an emerging flavivirus that causes congenital abnormalities and Guillain-Barré syndrome. ZIKV infection also results in severe eye disease characterized by optic neuritis, chorioretinal atrophy, and blindness in newborns and conjunctivitis and uveitis in adults. We evaluated ZIKV infection of the eye by using recently developed mouse models of pathogenesis. ZIKV-inoculated mice developed conjunctivitis, panuveitis, and infection of the cornea, iris, optic nerve, and ganglion and bipolar cells in the retina...
September 20, 2016: Cell Reports
Masoud Aghsaei Fard, Marjan Afzali, Parisa Abdi, Rebecca Chen, Mehdi Yaseri, Ebrahim Azaripour, Sasan Moghimi
PURPOSE: To compare optic nerve head (ONH) morphology of optic nerve atrophy between eyes with primary open-angle glaucoma (POAG) and eyes with a history of nonarteritic anterior ischemic optic atrophy (NAION) using enhanced depth imaging (EDI) with spectral-domain optical coherence tomography (SD-OCT). METHODS: In this cross-sectional study, 121 eyes of 91 patients consisted of moderate to severe POAG (n = 32 eyes), visual field mean deviation-matched NAION (n = 30 eyes) and their fellow eyes (n = 30 eyes), and healthy controls (n = 29)...
September 1, 2016: Investigative Ophthalmology & Visual Science
Edita Dervisevic, Suzana Pavljasevic, Almir Dervisevic, Sanja Sefic Kasumovic
INTRODUCTION: Glaucoma is the most common optic neuropathy which is characterized by progressive loss of retinal ganglion cells, the excavation of the optic nerve head, associated with defects in the visual field. It is not a disease, but the final result of united and yet completely unidentified cellular and subcellular processes and effects of many factors responsible for changes in retinal ganglion cells leading to their accelerated apoptosis. METHODS: This is a prospective-retrospective, comparative, randomized clinical trial that included 150 patients, 97 were female and 53 male...
June 2016: Medical Archives
Elena Garcia-Martin, Javier Garcia-Campayo, Marta Puebla-Guedea, Francisco J Ascaso, Miguel Roca, Fernando Gutierrez-Ruiz, Elisa Vilades, Vicente Polo, Jose M Larrosa, Luis E Pablo, Maria Satue
OBJECTIVE: To investigate whether fibromyalgia induces axonal damage in the optic nerve that can be detected using optical coherence tomography (OCT), as the retinal nerve fiber layer (RNFL) is atrophied in patients with fibromyalgia compared with controls. METHODS: Fibromyalgia patients (n = 116) and age-matched healthy controls (n = 144) were included in this observational and prospective cohort study. All subjects underwent visual acuity measurement and structural analysis of the RNFL using two OCT devices (Cirrus and Spectralis)...
2016: PloS One
Raimondo Forte, Florent Aptel, Audrey Feldmann, Christophe Chiquet
BACKGROUND: Posterior polar annular choroidal dystrophy (PPACD) is a rare disease. Patients with PPACD show loss of retinal pigment epithelium and choriocapillaries surrounding the vascular arcades and optic nerve. METHODS: Two patients with PPACD were evaluated with multimodal imaging, including fundus autofluorescence (FAF) and adaptive optics (AO). REPORT OF CASES: One patient (32 year old, one eye) with PPACD was followed up for 3 years...
August 30, 2016: Retinal Cases & Brief Reports
Khalil Ghasemi Falavarjani, Jack J Tian, Handan Akil, Giancarlo A Garcia, Srinivas R Sadda, Alfredo A Sadun
PURPOSE: To evaluate the optic nerve head microvasculature in eyes with acute and chronic optic neuropathies using swept-source optical coherence tomography angiography. METHODS: In this cross-sectional, observational study, optical coherence tomography angiography images were obtained from the optic nerve heads of 21 eyes of 12 patients with optic disk edema, pseudoedema and atrophy, and 12 eyes of 6 healthy subjects using a 1,050-nm optical coherence tomography angiography (Topcon DRI OCT; Triton)...
August 22, 2016: Retina
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