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Optic nerve atrophy

Femke Visser, Koenraad A Vermeer, Babak Ghafaryasl, Annemarie M M Vlaar, Valentin Apostolov, Jan van Hellenberg Hubar, Henry C Weinstein, Johannes F de Boer, Henk W Berendse
Thinning of the retinal nerve fiber layer (RNFL) is a recently discovered feature of Parkinson's disease (PD). Its exact pathological mechanism is yet unknown. We aimed to determine whether morphological changes of the RNFL are limited to RNFL thinning or also comprise an altered internal structure of this layer. Therefore, we investigated RNFL thickness and applied the RNFL attenuation coefficient (RNFL-AC), a novel method derived from optical coherence tomography, in PD patients and healthy controls (HCs)...
March 8, 2018: Journal of Neural Transmission
Maria Veronica Strehaianu, Dana Dascalescu, Catalina Ionescu, Miruna Burcel, Vasile Potop, Catalina Corbu
Glaucoma is an optic neuropathy that affects the ganglion cell complex in all its components: cell bodies, dendrites, and axons, the dendritic arbor being the first one damaged. This is the reason why the thickness of the ganglion cell and internal plexiform layers can be taken into account as an early predictor of the glaucomatous changes, along with the retinal nerve fiber layer (RNFL) thickness. However, due to disc tilting and peripapillary atrophy, the RNFL evaluation may be prone to errors in myopic patients...
October 2017: Romanian Journal of Ophthalmology
Michael Dietrich, Niklas Helling, Alexander Hilla, Annemarie Heskamp, Andrea Issberner, Thomas Hildebrandt, Zippora Kohne, Patrick Küry, Carsten Berndt, Orhan Aktas, Dietmar Fischer, Hans-Peter Hartung, Philipp Albrecht
BACKGROUND: In multiple sclerosis (MS), neurodegeneration is the main reason for chronic disability. Alpha-lipoic acid (LA) is a naturally occurring antioxidant which has recently been demonstrated to reduce the rate of brain atrophy in progressive MS. However, it remains uncertain if it is also beneficial in the early, more inflammatory-driven phases. As clinical studies are costly and time consuming, optic neuritis (ON) is often used for investigating neuroprotective or regenerative therapeutics...
March 7, 2018: Journal of Neuroinflammation
Kalpana Babu, Deepika C Parameswarappa
Purpose: We report an interesting case of atypical Tolosa Hunt syndrome with bilateral Sclerokeratitis and optic atrophy in a patient with Takayasu's arteritis. Observations: A 31-year- old lady presented with severe retroorbital pain in the right eye and right sided headache of 2 weeks duration. She had a history of 6th and 7th nerve palsies which improved with oral corticosteroids. Her BCVA was no perception of light in OD and 6/6 in OS. Slitlamp examination showed bilateral old sclerokeratitis and fundus examination showed bilateral optic atrophy...
April 2017: American Journal of Ophthalmology Case Reports
Razek Georges Coussa, Andre Ali-Ridha, Natalia Vila, Rayan Alshareef, John Chen
Purpose: To describe a case of Crohn disease presenting as occlusive vasculitis resulting in a central retinal artery occlusion (CRAO) in one eye and transient ischemic optic neuropathy in the fellow eye. Observations: An 18-year-old patient recently diagnosed with biopsy-proven Crohn disease presented with CRAO OD after a previous episode of transient visual loss OS. Extensive workup was negative for other autoimmune or infectious etiologies. The patient was started on intravenous methylprednisolone for 72 h followed by maintenance dose of azathioprine and oral prednisone...
April 2017: American Journal of Ophthalmology Case Reports
Jingbo Wang, Lanlan Jin, Jun Chen, Xiaobi Fang, Zhisu Liao
Objective: To explore the clinical relationship between auditory neuropathy (AN) and nervous system diseases. Methods: A total of 134 AN patients who were treated in our hospital from December 2011 to April 2016 were selected. Then 120 cases (240 ears) with complete data of pure tone audiometry and acoustic immittance test were selected as an AN1 group, which was compared with 30 patients (49 ears) with general sensorineural hearing loss (SHL) in regard to the results of pure tone audiometry and acoustic immittance test...
November 2017: Pakistan Journal of Medical Sciences Quarterly
Klaus Rüther
Hereditary optic nerve disorders are rare. For ophthalmologists, Leber's hereditary optic neuropathy (LHON) and autosomal dominant optic atrophy (ADOA) are of particular relevance. LHON and ADOA are diseases of the retinal ganglion cells and are caused by mitchochondrial dysfunction. LHON is based on mutations of the mitochondrial, ADOA of the nuclear DNA. LHON is a disease that usually leads to severe visual impairment (visual acuity < 0.1). Since there is an approved therapy for LHON (Idebenone [Raxone]), the diagnosis has to be confirmed immediately by means of molecular genetic diagnostics...
February 28, 2018: Klinische Monatsblätter Für Augenheilkunde
Raed Behbehani, Hussain Adnan, Abdullah Abu Al-Hassan, Ali Al-Salahat, Raed Alroughani
BACKGROUND: Multiple sclerosis is an inflammatory demyelinating disease characterized by progressive axonal loss affecting mainly the inner retinal layers. Optical coherence tomography (OCT) provides in-vivo quantification of the retinal layers and allows measuring progressive retinal changes. Our objective was to assess the longitudinal changes in the retina using spectral domain OCT (SDOCT) and to identify independent predictors affecting retinal thinning in MS patients. METHODS: A prospective study in a tertiary care MS center was conducted to study the longitudinal retinal changes in MS patients...
February 11, 2018: Multiple Sclerosis and related Disorders
Alberto Di Somma, Ariel Matias Kaen, Eugenio Cárdenas Ruiz-Valdepeñas, Luigi Maria Cavallo
In this study we report an uncommon endoscopic endonasal image of an atrophic optic nerve as seen after surgical removal of a suprasellar meningioma. The peculiarity of this case is the long-lasting underestimated ocular symptomatology of the patient who reported a 15-year history of impairment of vision on her left eye. After making serendipitously the diagnosis of a suprasellar mass, an endoscopic endonasal surgery was performed and, at the end of tumor removal, the impressive left optic nerve atrophy due to enduring local compression was visualized...
February 14, 2018: World Neurosurgery
Vanessa Andrada Păun, Zamfir-Radu Ionescu, Liliana Voinea, Monica Cîrstoiu, Alexandru Baroș, Ștefan Pricopie, Radu Ciuluvică
Ocular posterior pole modification are a pathological manifestation in complicated pregnancies, especially when pregnancy induced hypertension is present (PIH), as well as in preeclampsia (PE) or eclampsia. Nonetheless, as the pregnancy evolves, the possibility for an aggravated evolution with HELLP syndrome, disseminated intravascular coagulation, and idiopathic thrombocytopenic purpura may have an ocular manifestation that, mainly, implies a loss of visual field or acuity, that, left unattended, may constitute a permanent impairment...
April 2017: Romanian Journal of Ophthalmology
Anselm Jünemann, Robert Rejdak, Bettina Hohberger
Endothelial dysfunction and vascular dysregulation play a role in the multifactorial pathogenesis of glaucomatous optic nerve atrophy. Hyperhomocysteinemia is a risk factor for endothelial dysfunction and is associated with primary open-angle glaucoma as well as secondary open-angle glaucoma in the pseudoexfoliation syndrome. This paper reviews the literature on the association between homocysteine metabolism and glaucomatous disease and explains the possible role of hyperhomocysteinemia in the pathogenesis and progression of glaucoma...
February 2018: Klinische Monatsblätter Für Augenheilkunde
Andrea Accogli, Fadi F Hamdan, Chantal Poulin, Christina Nassif, Guy A Rouleau, Jacques L Michaud, Myriam Srour
Adaptor protein complex-4 (AP-4) is a heterotetrameric protein complex which plays a key role in vesicle trafficking in neurons. Mutations in genes affecting different subunits of AP-4, including AP4B1, AP4E1, AP4S1, and AP4M1, have been recently associated with an autosomal recessive phenotype, consisting of spastic tetraplegia, and intellectual disability (ID). The overlapping clinical picture among individuals carrying mutations in any of these genes has prompted the terms "AP-4 deficiency syndrome" for this clinically recognizable phenotype...
February 12, 2018: American Journal of Medical Genetics. Part A
Y J Yan, L Zhang, Z J Wang, A H Li, X Q Sun
Objective: To discuss the dynamic changes and correlation of macular ganglion cell (mGCC) and peripapillary retinal nerve fiber layer (pRNFL) of neuritis patients through optical coherence tomography (OCT). Methods: A retrospective case series study. Eleven eyes from 11 patients who have been diagnosed with neuritis and received regular follow-up during June 2013 through June 2015 were included. The dynamic characteristic changes of mGCC and pRNFL over the course of disease, as well as their correlations between best visual acuity and visual field have been analyzed based on OCT measurements...
January 11, 2018: [Zhonghua Yan Ke za Zhi] Chinese Journal of Ophthalmology
Sohan Singh Hayreh
Objective: To compare the severity of ischemic damage following posterior ciliary artery (PCA) occlusion in old, atherosclerotic, hypertensive monkeys to that in young monkeys. Design: Experimental study. Subjects: Seven eyes of normal, healthy rhesus monkeys and 8 of old, atherosclerotic, hypertensive monkeys. Methods: By lateral orbitotomy, all PCAs were cut behind the eyeball in both groups of animals. The fundus and the optic disc were evaluated by repeated ophthalmoscopy, color fundus photography and fluorescein fundus angiography, before and immediately after cutting the PCAs and serially thereafter during the follow-up period...
February 2018: Ophthalmology Retina
Eleonora M Lad, Dibyendu Mukherjee, Sandra S Stinnett, Scott W Cousins, Guy G Potter, James R Burke, Sina Farsiu, Heather E Whitson
Inner retina in Alzheimer's Disease (AD) may experience neuroinflammation resulting in atrophy. The objective of our study was to determine whether retinal GCIPL (ganglion cell-inner plexiform layer) or nerve fiber layer (NFL) thickness may serve as noninvasive biomarkers to diagnose AD. This cross-sectional case-control study enrolled 15 mild cognitive impairment (MCI) patients, 15 mild-moderate AD patients, and 18 cognitively normal adults. NFL and GCIPL thicknesses on optical coherence tomography (OCT) were measured using Duke Optical Coherence Tomography Retinal Analysis Program (DOCTRAP) and Spectralis software...
2018: PloS One
Ana C Rodrigues-Neves, Inês D Aires, Joana Vindeirinho, Raquel Boia, Maria H Madeira, Francisco Q Gonçalves, Rodrigo A Cunha, Paulo F Santos, António F Ambrósio, Ana R Santiago
Glaucoma is the second cause of blindness worldwide and is characterized by the degeneration of retinal ganglion cells (RGCs) and optic nerve atrophy. Increased microglia reactivity is an early event in glaucoma that may precede the loss of RGCs, suggesting that microglia and neuroinflammation are involved in the pathophysiology of this disease. Although global changes of the purinergic system have been reported in experimental and human glaucoma, it is not known if this is due to alterations of the purinergic system of microglial cells, the resident immune cells of the central nervous system...
2018: Frontiers in Pharmacology
Emmanuelle Sarzi, Marie Seveno, Camille Piro-Mégy, Lucie Elzière, Mélanie Quilès, Marie Péquignot, Agnès Müller, Christian P Hamel, Guy Lenaers, Cécile Delettre
Dominant optic atrophy (DOA) is a rare progressive and irreversible blinding disease which is one of the most frequent forms of hereditary optic neuropathy. DOA is mainly caused by dominant mutation in the OPA1 gene encoding a large mitochondrial GTPase with crucial roles in membrane dynamics and cell survival. Hereditary optic neuropathies are commonly characterized by the degeneration of retinal ganglion cells, leading to the optic nerve atrophy and the progressive loss of visual acuity. Up to now, despite increasing advances in the understanding of the pathological mechanisms, DOA remains intractable...
February 6, 2018: Scientific Reports
Narjes Jafari, Karl Golnik, Mansoor Shahriari, Parvaneh Karimzadeh, Sayena Jabbehdari
Purpose: We aimed to present the ophthalmic manifestations of neuro-metabolic disorders. Methods: Patients who were diagnosed with neuro-metabolic disorders in the Neurology Department of Mofid Pediatric Hospital in Tehran, Iran, between 2004 and 2014 were included in this study. Disorders were confirmed using clinical findings, neuroimaging, laboratory data, and genomic analyses. All enrolled patients were assessed for ophthalmological abnormalities. Results: A total of 213 patients with 34 different neuro-metabolic disorders were included...
January 2018: Journal of Ophthalmic & Vision Research
Mariana Flores-Pimentel, Alejandra G de Alba Campomanes
A premature infant born at 31 weeks' gestational age was evaluated for periocular laceration and retrobulbar hematoma secondary to injury during a percutaneous fetal procedure. He later developed optic nerve atrophy.
January 29, 2018: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
Zhu Li Yap, Sushma Verma, Yi Fang Lee, Charles Ong, Aditi Mohla, Shamira A Perera
There are two long-standing theories about the pathogenesis of glaucoma - barotrauma and the effect of vascular hypoxia. Currently, it is still unknown whether diminished blood flow is the cause or result of glaucomatous atrophy of ganglion cells and the optic nerve. Though many other imaging techniques used to directly assess ocular blood flow have been well studied, they are limited by their inability to directly assess metabolism in the ocular tissues or measure the oxygen carrying capacity in the vessels...
2018: Clinical Ophthalmology
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