The Experts below are selected from a list of 4401 Experts worldwide ranked by ideXlab platform
Catherine Vignal - One of the best experts on this subject based on the ideXlab platform.
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retinal inner nuclear layer microcystic changes in Optic Nerve Atrophy a novel spectral domain oct finding
Retina-the Journal of Retinal and Vitreous Diseases, 2013Co-Authors: Benjamin Wolff, Chrysanthi Basdekidou, Vivien Vasseur, Martine Maugetfaÿsse, José-alain Sahel, Catherine VignalAbstract:Purpose:Optic Atrophy constitutes the final stage in the evolution of Optic neuropathy. The aim of this study was to describe the presence of macular microcystic changes or pseudocysts in patients with advanced Optic Atrophy.Methods:The medical records of 24 patients who had retinal pseudocysts in a
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retinal inner nuclear layer microcystic changes in Optic Nerve Atrophy a novel spectral domain oct finding
Retina-the Journal of Retinal and Vitreous Diseases, 2013Co-Authors: Benjamin Wolff, Chrysanthi Basdekidou, Vivien Vasseur, Martine Maugetfaÿsse, José-alain Sahel, Catherine VignalAbstract:PURPOSE Optic Atrophy constitutes the final stage in the evolution of Optic neuropathy. The aim of this study was to describe the presence of macular microcystic changes or pseudocysts in patients with advanced Optic Atrophy. METHODS The medical records of 24 patients who had retinal pseudocysts in association with Optic Atrophy have been analyzed. All patients underwent a complete neuro-ophthalmologic assessment; peripapillary retinal Nerve fiber layer thickness and macular screening with spectral-domain Optical coherence tomography and Optical coherence tomography "en face" imaging analysis were also performed. RESULTS A total of 36 eyes were included in the study. Patients' mean age was 37 years. The major cause of Optic Atrophy was glaucoma (12 cases). The retinal pseudocysts were observed as hyporeflective lesions in the internal nuclear layer. Infrared images revealed a hyporeflective circular or semilunar shape corresponding to the location of the pseudocysts in all cases. In eyes with pseudocysts, mean thickness of the peripapillary retinal Nerve fiber layer was statistically significantly less than that of fellow eyes (P = 0.0003), whereas macular thickness was statistically significantly higher compared with fellow eyes (P < 0.005). CONCLUSION The presence of pseudocystic lesions always associated with severe Optic Nerve fiber loss is reported. The reason why pseudocystic lesions develop within the retina is not well understood. They might constitute the translation of degeneration of Muller cells in severe Optic Nerve fiber loss. Recognizing these pseudocysts is crucial because they may be confused with cystoid macular edema. Their prognostic value and role in the therapeutic process need to be further evaluated with prospective studies and molecular experiments in vivo.
Studer Michèle - One of the best experts on this subject based on the ideXlab platform.
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Mouse Nr2f1 haploinsufficiency unveils new pathological mechanisms of a human Optic Atrophy syndrome
'EMBO', 2020Co-Authors: Bertacchi Michele, Gruart Agnès, Kaimakis Polynikis, Allet Cécile, Serra Linda, Delgado-garcía, José María, Bovolenta Paola, Studer MichèleAbstract:Optic Nerve Atrophy represents the most common form of hereditary Optic neuropathies leading to vision impairment. The recently described Bosch-Boonstra-Schaaf Optic Atrophy (BBSOA) syndrome denotes an autosomal dominant genetic form of neuropathy caused by mutations or deletions in the NR2F1 gene. Herein, we describe a mouse model recapitulating key features of BBSOA patients—Optic Nerve Atrophy, Optic disc anomalies, and visual deficits—thus representing the only available mouse model for this syndrome. Notably, Nr2f1-deficient Optic Nerves develop an imbalance between oligodendrocytes and astrocytes leading to postnatal hypomyelination and astrogliosis. Adult heterozygous mice display a slower Optic axonal conduction velocity from the retina to high-order visual centers together with associative visual learning deficits. Importantly, some of these clinical features, such the Optic Nerve hypomyelination, could be rescued by chemical drug treatment in early postnatal life. Overall, our data shed new insights into the cellular mechanisms of Optic Nerve Atrophy in BBSOA patients and open a promising avenue for future therapeutic approaches.Spanish Ministry of Science and Innovation BFU2016-75412-R (with FEDER funds), PCIN-2015-176-C02-01/ERA-Net NeuronII and an Institutional CBMSO Grant from the Fundación Ramón Areces to P.B. The electrophysiological and behavioral study was supported by grants from the Spanish Ministry of Science and Innovation (BFU2017-82375-R), the Junta de Andalucía (Spain, BIO-122), and the Spanish Tatiana Pérez de Guzmán el Bueno Foundation to A.G. and J.M.D.-G
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Mouse Nr2f1 haploinsufficiency unveils new pathological mechanisms of a human Optic Atrophy syndrome
'EMBO', 2019Co-Authors: Bertacchi Michele, Gruart Agnès, Kaimakis Polynikis, Allet Cécile, Serra Linda, Bovolenta Paola, Giacobini Paolo, Delgado-garcía José, Studer MichèleAbstract:International audienceOptic Nerve Atrophy represents the most common form of hereditary Optic neuropathies leading to vision impairment. The recently described Bosch-Boonstra-Schaaf Optic Atrophy (BBSOA) syndrome denotes an autosomal dominant genetic form of neuropathy caused by mutations or deletions in the NR2F1 gene. Herein, we describe a mouse model recapitulating key features of BBSOA patients-Optic Nerve Atrophy, Optic disc anomalies, and visual deficits-thus representing the only available mouse model for this syndrome. Notably, Nr2f1-deficient Optic Nerves develop an imbalance between oligodendrocytes and astrocytes leading to postnatal hypomyelination and astrogliosis. Adult heterozygous mice display a slower Optic axonal conduction velocity from the retina to high-order visual centers together with associative visual learning deficits. Importantly, some of these clinical features, such the Optic Nerve hypomyelination, could be rescued by chemical drug treatment in early postnatal life. Overall, our data shed new insights into the cellular mechanisms of Optic Nerve Atrophy in BBSOA patients and open a promising avenue for future therapeutic approaches
Nagahisa Yoshimura - One of the best experts on this subject based on the ideXlab platform.
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quantitative comparison of disc rim color in Optic Nerve Atrophy of compressive Optic neuropathy and glaucomatous Optic neuropathy
Graefes Archive for Clinical and Experimental Ophthalmology, 2016Co-Authors: Eri Nakano, Masayuki Hata, Akio Oishi, Kazuaki Miyamoto, Akihito Uji, Masahiro Fujimoto, Manabu Miyata, Nagahisa YoshimuraAbstract:The purpose was to investigate an objective and quantitative method to estimate the redness of the Optic disc neuroretinal rim, and to determine the usefulness of this method to differentiate compressive Optic neuropathy (CON) from glaucomatous Optic neuropathy (GON). In our study there were 126 eyes: 40 with CON, 40 with normal tension glaucoma (NTG), and 46 normal eyes (NOR). Digital color fundus photographs were assessed for the redness of disc rim color using ImageJ software. We separately measured the intensity of red, green, and blue pixels from RGB images. Three disc color indices (DCIs), which indicate the redness intensity, were calculated through existing formulas. All three DCIs of CON were significantly smaller than those of NOR (P -6 dB), in which the extent of retinal Nerve fiber layer thinning is comparable, the DCIs of mild CON were significantly smaller than those of mild NTG (P < 0.05). In contrast, DCIs did not differ between moderate-to-severe stages of CON and NTG (MD ≤ -6 dB), though the retinal Nerve fibers of CON were more severely damaged than those of NTG. To differentiate between mild CON and mild NTG, all AUROCs for the three DCIs were above 0.700. A quantitative and objective assessment of Optic disc color was useful in differentiating early-stage CON from GON and NOR.
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comparison of Optic disc morphology of Optic Nerve Atrophy between compressive Optic neuropathy and glaucomatous Optic neuropathy
PLOS ONE, 2014Co-Authors: Masayuki Hata, Tadamichi Akagi, Yugo Kimura, Akio Oishi, Kazuaki Miyamoto, Yukiko Makiyama, Norimoto Gotoh, Nagahisa YoshimuraAbstract:Objectives To compare the Optic Nerve head (ONH) structure between compressive Optic neuropathy (CON) and glaucomatous Optic neuropathy (GON), and to determine whether selected ONH quantitative parameters effectively discriminate between GON and CON, especially CON cases presenting with a glaucoma-like disc. Methods We prospectively assessed 34 patients with CON, 34 age-matched patients with moderate or severe GON, and 34 age-matched healthy control subjects. The quantitative parameters of ONH structure were compared using the Heidelberg Retina Tomograph 2 (HRT2) and Spectralis Optical coherence tomography with an enhanced depth imaging method. Results The mean and maximum cup depths of CON were significantly smaller than those with GON (P<0.001 and P<0.001, respectively). The distance between Bruch's membrane opening and anterior surface of the lamina cribrosa (BMO-anterior LC) of CON was also significantly smaller than that of glaucoma but was similar to that of the healthy group (P<0.001 and P = 0.47, respectively). Based on Moorfields regression analysis of the glaucoma classification of HRT2, 15 eyes with CON were classified with a glaucoma-like disc. The cup/disc area ratio did not differ between cases of CON with a glaucoma-like disc and cases of GON (P = 0.16), but the BMO-anterior LC and mean and maximum cup depths of CON cases with a glaucoma-like disc were smaller than those in GON (P = 0.005, P = 0.003, and P = 0.001, respectively). Conclusions Measurements of the cup depths and the LC depth had good ability to differentiate between CON with a glaucoma-like disc and glaucoma. There was no laminar remodeling detected by laminar surface position in the patients with CON compared to those with GON.
Benjamin Wolff - One of the best experts on this subject based on the ideXlab platform.
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retinal inner nuclear layer microcystic changes in Optic Nerve Atrophy a novel spectral domain oct finding
Retina-the Journal of Retinal and Vitreous Diseases, 2013Co-Authors: Benjamin Wolff, Chrysanthi Basdekidou, Vivien Vasseur, Martine Maugetfaÿsse, José-alain Sahel, Catherine VignalAbstract:Purpose:Optic Atrophy constitutes the final stage in the evolution of Optic neuropathy. The aim of this study was to describe the presence of macular microcystic changes or pseudocysts in patients with advanced Optic Atrophy.Methods:The medical records of 24 patients who had retinal pseudocysts in a
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retinal inner nuclear layer microcystic changes in Optic Nerve Atrophy a novel spectral domain oct finding
Retina-the Journal of Retinal and Vitreous Diseases, 2013Co-Authors: Benjamin Wolff, Chrysanthi Basdekidou, Vivien Vasseur, Martine Maugetfaÿsse, José-alain Sahel, Catherine VignalAbstract:PURPOSE Optic Atrophy constitutes the final stage in the evolution of Optic neuropathy. The aim of this study was to describe the presence of macular microcystic changes or pseudocysts in patients with advanced Optic Atrophy. METHODS The medical records of 24 patients who had retinal pseudocysts in association with Optic Atrophy have been analyzed. All patients underwent a complete neuro-ophthalmologic assessment; peripapillary retinal Nerve fiber layer thickness and macular screening with spectral-domain Optical coherence tomography and Optical coherence tomography "en face" imaging analysis were also performed. RESULTS A total of 36 eyes were included in the study. Patients' mean age was 37 years. The major cause of Optic Atrophy was glaucoma (12 cases). The retinal pseudocysts were observed as hyporeflective lesions in the internal nuclear layer. Infrared images revealed a hyporeflective circular or semilunar shape corresponding to the location of the pseudocysts in all cases. In eyes with pseudocysts, mean thickness of the peripapillary retinal Nerve fiber layer was statistically significantly less than that of fellow eyes (P = 0.0003), whereas macular thickness was statistically significantly higher compared with fellow eyes (P < 0.005). CONCLUSION The presence of pseudocystic lesions always associated with severe Optic Nerve fiber loss is reported. The reason why pseudocystic lesions develop within the retina is not well understood. They might constitute the translation of degeneration of Muller cells in severe Optic Nerve fiber loss. Recognizing these pseudocysts is crucial because they may be confused with cystoid macular edema. Their prognostic value and role in the therapeutic process need to be further evaluated with prospective studies and molecular experiments in vivo.
Bertacchi Michele - One of the best experts on this subject based on the ideXlab platform.
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Mouse Nr2f1 haploinsufficiency unveils new pathological mechanisms of a human Optic Atrophy syndrome
'EMBO', 2020Co-Authors: Bertacchi Michele, Gruart Agnès, Kaimakis Polynikis, Allet Cécile, Serra Linda, Delgado-garcía, José María, Bovolenta Paola, Studer MichèleAbstract:Optic Nerve Atrophy represents the most common form of hereditary Optic neuropathies leading to vision impairment. The recently described Bosch-Boonstra-Schaaf Optic Atrophy (BBSOA) syndrome denotes an autosomal dominant genetic form of neuropathy caused by mutations or deletions in the NR2F1 gene. Herein, we describe a mouse model recapitulating key features of BBSOA patients—Optic Nerve Atrophy, Optic disc anomalies, and visual deficits—thus representing the only available mouse model for this syndrome. Notably, Nr2f1-deficient Optic Nerves develop an imbalance between oligodendrocytes and astrocytes leading to postnatal hypomyelination and astrogliosis. Adult heterozygous mice display a slower Optic axonal conduction velocity from the retina to high-order visual centers together with associative visual learning deficits. Importantly, some of these clinical features, such the Optic Nerve hypomyelination, could be rescued by chemical drug treatment in early postnatal life. Overall, our data shed new insights into the cellular mechanisms of Optic Nerve Atrophy in BBSOA patients and open a promising avenue for future therapeutic approaches.Spanish Ministry of Science and Innovation BFU2016-75412-R (with FEDER funds), PCIN-2015-176-C02-01/ERA-Net NeuronII and an Institutional CBMSO Grant from the Fundación Ramón Areces to P.B. The electrophysiological and behavioral study was supported by grants from the Spanish Ministry of Science and Innovation (BFU2017-82375-R), the Junta de Andalucía (Spain, BIO-122), and the Spanish Tatiana Pérez de Guzmán el Bueno Foundation to A.G. and J.M.D.-G
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Mouse Nr2f1 haploinsufficiency unveils new pathological mechanisms of a human Optic Atrophy syndrome
'EMBO', 2019Co-Authors: Bertacchi Michele, Gruart Agnès, Kaimakis Polynikis, Allet Cécile, Serra Linda, Bovolenta Paola, Giacobini Paolo, Delgado-garcía José, Studer MichèleAbstract:International audienceOptic Nerve Atrophy represents the most common form of hereditary Optic neuropathies leading to vision impairment. The recently described Bosch-Boonstra-Schaaf Optic Atrophy (BBSOA) syndrome denotes an autosomal dominant genetic form of neuropathy caused by mutations or deletions in the NR2F1 gene. Herein, we describe a mouse model recapitulating key features of BBSOA patients-Optic Nerve Atrophy, Optic disc anomalies, and visual deficits-thus representing the only available mouse model for this syndrome. Notably, Nr2f1-deficient Optic Nerves develop an imbalance between oligodendrocytes and astrocytes leading to postnatal hypomyelination and astrogliosis. Adult heterozygous mice display a slower Optic axonal conduction velocity from the retina to high-order visual centers together with associative visual learning deficits. Importantly, some of these clinical features, such the Optic Nerve hypomyelination, could be rescued by chemical drug treatment in early postnatal life. Overall, our data shed new insights into the cellular mechanisms of Optic Nerve Atrophy in BBSOA patients and open a promising avenue for future therapeutic approaches