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Optic neuropathy

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https://www.readbyqxmd.com/read/29040550/rescue-of-cell-death-and-inflammation-of-a-mouse-model-of-complex-1-mediated-vision-loss-by-repurposed-drug-molecules
#1
Alfred K Yu, Sandipan Datta, Marissa Z McMackin, Gino A Cortopassi
Inherited mitochondrial optic neuropathies, such as Leber's hereditary optic neuropathy (LHON) and Autosomal dominant optic atrophy (ADOA) are caused by mutant mitochondrial proteins that lead to defects in mitochondrial complex 1-driven ATP synthesis, and cause specific retinal ganglion cell (RGC) loss. Complex 1 defects also occur in patients with primary open angle glaucoma (POAG), in which there is specific RGC loss. The treatment of mitochondrial optic neuropathy in the US is only supportive. The Ndufs4 knockout (Ndufs4 KO) mouse is a mitochondrial complex 1-deficient model that leads to RGC loss and rapid vision loss and allows for streamlined testing of potential therapeutics...
October 12, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/29037018/role-of-fractionated-radiotherapy-in-patients-with-hemangioma-of-the-cavernous-sinus
#2
Sunmin Park, Sang Min Yoon, Sumin Lee, Jin-Hong Park, Si Yeol Song, Sang-Wook Lee, Seung Do Ahn, Jong Hoon Kim, Eun Kyung Choi
PURPOSE: We performed this retrospective study to investigate the outcomes of patients with hemangioma of the cavernous sinus after fractionated radiotherapy. MATERIALS AND METHODS: We analyzed 10 patients with hemangioma of the cavernous sinus who were treated with conventional radiotherapy between January 2000 and December 2016. The median patient age was 54 years (range, 31-65 years), and 8 patients (80.0%) were female. The mean hemangioma volume was 34.1 cm(3) (range, 6...
September 2017: Radiation Oncology Journal
https://www.readbyqxmd.com/read/29033621/nonarteritic-anterior-ischemic-optic-neuropathy-cause-effect-and-management
#3
REVIEW
Shauna Berry, Weijie V Lin, Ama Sadaka, Andrew G Lee
Nonarteritic anterior ischemic optic neuropathy (NAION) is the most common form of ischemic optic neuropathy and the second most common optic neuropathy. Patients are generally over the age of 50 years with vasculopathic risk factors (eg, diabetes mellitus, hypertension, and obstructive sleep apnea). The exact mechanism of NAION is not fully understood. In addition, several treatment options have been proposed. This article summarizes the current literature on the diagnosis, treatment, and management of NAION...
2017: Eye and Brain
https://www.readbyqxmd.com/read/29031856/relationship-between-sigma-1-receptor-and-bdnf-in-the-visual-system
#4
Barbara A Mysona, Jing Zhao, Sylvia Smith, Kathryn E Bollinger
Glaucoma is an incurable optic neuropathy characterized by dysfunction and death of retinal ganglion cells (RGCs). Brain derived neurotrophic factor (BDNF) is an essential neurotrophin that supports RGC function and survival. Despite BDNF's importance, our knowledge of molecular mechanisms that modulate BDNF processing and secretion is incomplete. Sigma-1 receptor (S1R) is associated with increased BDNF in hippocampus and with BDNF secretion by brain-derived astrocytes and neuronal cell lines. Much less is known about the relationship between S1R and BDNF in the visual system...
October 12, 2017: Experimental Eye Research
https://www.readbyqxmd.com/read/29029896/clemastine-fumarate-as-a-remyelinating-therapy-for-multiple-sclerosis-rebuild-a-randomised-controlled-double-blind-crossover-trial
#5
Ari J Green, Jeffrey M Gelfand, Bruce A Cree, Carolyn Bevan, W John Boscardin, Feng Mei, Justin Inman, Sam Arnow, Michael Devereux, Aya Abounasr, Hiroko Nobuta, Alyssa Zhu, Matt Friessen, Roy Gerona, Hans Christian von Büdingen, Roland G Henry, Stephen L Hauser, Jonah R Chan
BACKGROUND: Multiple sclerosis is a degenerative inflammatory disease of the CNS characterised by immune-mediated destruction of myelin and progressive neuroaxonal loss. Myelin in the CNS is a specialised extension of the oligodendrocyte plasma membrane and clemastine fumarate can stimulate differentiation of oligodendrocyte precursor cells in vitro, in animal models, and in human cells. We aimed to analyse the efficacy and safety of clemastine fumarate as a treatment for patients with multiple sclerosis...
October 10, 2017: Lancet
https://www.readbyqxmd.com/read/29024675/tempo-ral-was-the-heart-of-the-matter
#6
Francesco Pellegrini, Daniele Cirone, Piernicola Machin, Rod Foroozan
A 71-year-old woman was admitted with fever, headache and weight loss associated with elevated inflammatory markers. She developed acute bilateral ophthalmoplegia and asymmetrical ptosis rapidly followed by anterior ischemic optic neuropathy. Although the first temporal artery biopsy (TAB) was negative, contralateral TAB revealed features consistent with giant cell arteritis. Even while under steroid therapy, the patient died a few days later from myocardial infarction. Acute bilateral complete ophthalmoplegia is a rare presentation of a limited number of possible diseases...
October 9, 2017: Survey of Ophthalmology
https://www.readbyqxmd.com/read/29021915/optical-coherence-tomography-findings-in-methanol-toxicity
#7
Kendra A Klein, Alexis K Warren, Caroline R Baumal, Thomas R Hedges
BACKGROUND: Methanol toxicity poses a significant public health problem in developing countries, and in Southeast Asia, where the most common source of poisoning is via adulterated liquor in local drinks. Methanol toxicity can have devastating visual consequences and retinal specialists should be aware of the features of this toxic optic neuropathy. The authors report a case of severe systemic methanol toxicity and relatively mild optic neuropathy demonstrating unique retinal changes on optical coherence tomography (OCT)...
2017: International Journal of Retina and Vitreous
https://www.readbyqxmd.com/read/29018766/acute-angle-closure-following-periorbital-botulinum-toxin-injection-in-a-patient-with-retinitis-pigmentosa
#8
Jen-Chia Tsai
A 50-year-old female presented with bilateral retinitis pigmentosa (RP) and acute angle closure (AAC) with fixed mid-dilated pupil and high intraocular pressure (IOP) in the left eye following left side periorbital botulinum toxin A injection for blepharospasm. Glaucomatous optic neuropathy and retinal nerve fiber layer defect were observed in the affected eye using optical coherence tomography although the IOP was maintained at <21 mmHg after the treatment. Botulinum toxin acts at the cholinergic synapse and inhibits acetylcholine release; hence, it can cause transient mydriasis and may lead to AAC in high-risk populations such as patients with RP...
April 2017: Taiwan Journal of Ophthalmology
https://www.readbyqxmd.com/read/29016409/anterior-ischemic-optic-neuropathy-due-to-calciphylaxis
#9
Magne S Sivertsen, Erik H Strøm, Kim M A Endre
A 72-year-old woman experienced anterior ischemic optic neuropathy in her left eye. The funduscopic and fluorescein angiographic findings were strongly suggestive of giant cell arteritis. Temporal artery biopsy revealed extensive calcification in the vessel wall consistent with calciphylaxis. This unusual disorder should be considered in the differential diagnosis of anterior ischemic optic neuropathy, particularly the arteritic form.
October 6, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28994761/optical-coherence-tomography-imaging-mouse-retinal-ganglion-cells-in-vivo
#10
Jolanta Jagodzinska, Emmanuelle Sarzi, Mélanie Cavalier, Marie Seveno, Volker Baecker, Christian Hamel, Marie Péquignot, Cecile Delettre
Structural changes in the retina are common manifestations of ophthalmic diseases. Optical coherence tomography (OCT) enables their identification in vivo-rapidly, repetitively, and at a high resolution. This protocol describes OCT imaging in the mouse retina as a powerful tool to study optic neuropathies (OPN). The OCT system is an interferometry-based, non-invasive alternative to common post mortem histological assays. It provides a fast and accurate assessment of retinal thickness, allowing the possibility to track changes, such as retinal thinning or thickening...
September 22, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28994349/the-therapeutic-potential-of-a-calorie-restricted-ketogenic-diet-for-the-management-of-leber-hereditary-optic-neuropathy
#11
Mithu Storoni, Matthieu P Robert, Gordon T Plant
Leber hereditary optic neuropathy (LHON) is a maternally inherited, bilateral, sequential optic neuropathy that usually affects young males. LHON arises from a defect in complex I of the oxidative phosphorylation chain that generates increased reactive oxygen species and causes a decline in cellular ATP production. There exists no cure at present for LHON. Asymptomatic LHON mutation carriers show signs of increased mitochondrial biogenesis that may compensate for the compromise in complex I activity. Partial recovery in LHON is associated with a wider optic disc diameter and a younger age at disease onset, which may allow for greater mitochondrial bioenergetic capacity...
October 10, 2017: Nutritional Neuroscience
https://www.readbyqxmd.com/read/28991353/asymptomatic-leukemic-optic-nerve-infiltration-as-presentation-of-acute-lymphoblastic-leukemia-relapse
#12
Jane Caty, A Paula Grigorian, Florin Grigorian
The authors report a case of asymptomatic leukemic optic neuropathy as the first sign of acute lymphoblastic leukemia relapse in a 4-year-old boy. Routine ophthalmologic examination showed normal visual acuity and pupillary function in the presence of a tumoral mass covering the left optic disc. The mass resolved with preservation of vision after intrathecal chemotherapy. A routine ophthalmological examination is recommended for all patients with a history of acute lymphoblastic leukemia to exclude optic nerve involvement without systemic symptoms or signs...
October 9, 2017: Journal of Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/28991104/international-consensus-statement-on-the-clinical-and-therapeutic-management-of-leber-s-hereditary-optic-neuropathy
#13
Valerio Carelli, Michele Carbonelli, Irenaeus F de Coo, Aki Kawasaki, Thomas Klopstock, Wolf A Lagrèze, Chiara La Morgia, Nancy J Newman, Christophe Orssaud, Jan Willem R Pott, Alfredo A Sadun, Judith van Everdingen, Catherine Vignal-Clermont, Marcela Votruba, Patrick Yu-Wai-Man, Piero Barboni
Leber hereditary optic neuropathy (LHON) is currently estimated as the most frequent mitochondrial disease (1 in 27,000-45,000). Its molecular pathogenesis and natural history is now fairly well understood. LHON also is the first mitochondrial disease for which a treatment has been approved (idebenone-Raxone, Santhera Pharmaceuticals) by the European Medicine Agency, under exceptional circumstances because of the rarity and severity of the disease. However, what remains unclear includes the optimal target population, timing, dose, and frequency of administration of idebenone in LHON due to lack of accepted definitions, criteria, and general guidelines for the clinical management of LHON...
October 3, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28990081/leber-s-hereditary-optic-neuropathy-is-potentially-associated-with-a-novel-m-5587t-c-mutation-in-two-pedigrees
#14
Yanchun Ji, Lihua Qiao, Xiaoyang Liang, Ling Zhu, Yinglong Gao, Juanjuan Zhang, Zidong Jia, Qi-Ping Wei, Xiaoling Liu, Pingping Jiang, Min-Xin Guan
Mitochondrial (mt)DNA mutations have been revealed to be associated with Leber's hereditary optic neuropathy (LHON). The present study conducted clinical, genetic and molecular evaluations of two Han Chinese families. A total of 4 (3 men and 1 female) out of 14 matrilineal relatives in the families exhibited visual impairment with variable severity and age of onset. The average age of onset of visual loss was 20.5 years old. Molecular analysis of the complete mitochondrial genome in these pedigrees demonstrated that the three primary mutations associated with LHON were not detected; however, the homoplasmic m...
October 5, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28986994/microvasculature-dropout-detected-by-the-optical-coherence-tomography-angiography-in-nonarteritic-anterior-ischemic-optic-neuropathy
#15
Yi Song, Jing-Yu Min, Lei Mao, Yuan-Yuan Gong
OBJECTIVE: To investigate microcirculation characteristics of peripapillary superficial retina and optic disc in eyes with nonarteritic anterior ischemic optic neuropathy (NAION) using optical coherence tomography angiography (OCTA). METHODS: Forty-one eyes of 30 NAION patients and 30 eyes of 30 normal subjects were evaluated with OCTA (AngioVue, Optovue). The whole vessel density, inside disc vessel density, peripapillary vessel density, and vessel densities based on the sectorial division in the nerve head mode peripapillary superficial retina and RPC mode optic disc were measured respectively...
October 7, 2017: Lasers in Surgery and Medicine
https://www.readbyqxmd.com/read/28986670/traumatic-optic-neuropathy-treatment-trial-tontt-open-label-phase-3-multicenter-semi-experimental-trial
#16
Mohsen Bahmani Kashkouli, Sahar Yousefi, Marzieh Nojomi, Mostafa Soltan Sanjari, Farzad Pakdel, Morteza Entezari, Mohammad Etezad-Razavi, Mohammad Reza Razeghinejad, Manuchehr Esmaeli, Masoud Shafiee, Mansoureh Bagheri
PURPOSE: Intravenously administered erythropoietin (EPO) was firstly commenced (phase 1) in patients with indirect traumatic optic neuropathy (TON) by this group in 2011. It was re-tested by another group (phase 2) in 2014. This multicenter clinical trial was designed to compare its effect with intravenous steroid and observation. METHODS: Included were TON patients ≥5 years of age and with trauma-treatment interval of ≤3 weeks. Follow-up visits were set at 1, 2, 3, 7, 14, 30, and at least 90 days after treatment...
October 6, 2017: Graefe's Archive for Clinical and Experimental Ophthalmology
https://www.readbyqxmd.com/read/28986311/glaucoma-and-the-brain-trans-synaptic-degeneration-structural-change-and-implications-for-neuroprotection
#17
REVIEW
Mitchell Lawlor, Helen Danesh-Meyer, Leonard A Levin, Indran Davagnanam, Enrico De Vita, Gordon T Plant
A recent hypothesis to enter the literature suggests that glaucoma is a neuro-degenerative disease. The basis for this has been the finding of central nervous system (CNS) changes in glaucoma patients on histology and neuro-imaging. It is known that retinal ganglion cell (RGC) pathology of any cause leads to anterograde and retrograde RGC degeneration, as well as trans-synaptic (trans-neuronal) anterograde degeneration. Trans-synaptic degeneration has been demonstrated in a range of optic neuropathies including optic nerve transection, optic neuritis, and hereditary optic neuropathies...
October 3, 2017: Survey of Ophthalmology
https://www.readbyqxmd.com/read/28985827/ethambutol-optic-neuropathy-associated-with-enhancement-at-the-optic-chiasm
#18
Patricia G Lu, Nathan H Kung, Gregory P Van Stavern
No abstract text is available yet for this article.
October 2017: Canadian Journal of Ophthalmology. Journal Canadien D'ophtalmologie
https://www.readbyqxmd.com/read/28985584/strategies-to-prevent-ischemic-optic-neuropathy-following-major-spine-surgery-a-narrative-review
#19
REVIEW
Wilson Fandino
Postoperative vision loss following a major spine operation is a rare but life-changing event. Most of reports have been linked to ischemic optic neuropathy, and patients undergoing surgery for scoliosis correction or posterior lumbar fusion seem to be at the highest risk. Despite that some key risk factors have been identified, much of the pathophysiology still remain unknown. In fact, whereas only a minority of patients at high risk will present this complication, others with similar risk factors undergoing different procedures may not develop it at all...
October 3, 2017: Journal of Clinical Anesthesia
https://www.readbyqxmd.com/read/28985098/retinal-nerve-fiber-layer-thickness-in-human-t-cell-lymphotropic-virus-type-1-patients
#20
Harold Merle, Rabih Hage, Séverine Jeannin, Philippe Cabre, Stéphane Olindo
PURPOSE: To determine whether there is an optic neuropathy (ON) in patients with human T-cell lymphotropic virus type 1 (HTLV-1) infection. METHODS: We included HTLV-1 asymptomatic carriers (a.c.HTLV-1) and tropical spastic paraparesis/HTLV-1 associated myelopathy (TSP/HAM) patients between January 1st, 2014 and March 31st, 2015. All patients had complete eye examination. The visual acuity (VA) and retinal nerve fiber layer (RNFL) thickness were measured and compared to age- and sex-matched control groups including patients seen in our refraction clinic with no previous medical or surgical history...
October 6, 2017: Current Eye Research
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