Catherine Vignal - Academia.edu (original) (raw)
Papers by Catherine Vignal
Progress in Retinal and Eye Research
Elsevier Masson, Jan 2, 2019
Clinical Toxicology, 2021
Supplemental material, Table_S3_mai13 for Visual field loss and structure–function relationships ... more Supplemental material, Table_S3_mai13 for Visual field loss and structure–function relationships in optic neuritis associated with myelin oligodendrocyte glycoprotein antibody by Romain Deschamps, Manon Philibert, Cedric Lamirel, Jerome Lambert, Vivien Vasseur, Antoine Gueguen, Caroline Bensa, Augustin Lecler, Romain Marignier, Catherine Vignal and Olivier Gout in Multiple Sclerosis Journal
Journal of Neuro-Ophthalmology, 2022
BACKGROUND Giant cell arteritis (GCA) is a vasculitis often revealed by visual signs. Diagnosis i... more BACKGROUND Giant cell arteritis (GCA) is a vasculitis often revealed by visual signs. Diagnosis is challenging and urgent. Retinal angiography (RA) and MRI allow effective diagnosis. We compared those and proposed an imaging-based approach to diagnose GCA in ophthalmological practice. METHODS We conducted a retrospective study based on the data collected from patients suspected to have GCA on ophthalmological findings. Fluorescein (FA) and indocyanine green (ICG) RAs and MRI were performed and compared with final diagnosis. RESULTS Among the 41 patients included, 25 were diagnosed with GCA. Sensitivities and specificities of FA and ICG were not different. MRI showed a higher sensitivity and specificity. The approach consisting in performing RA followed by MRI provided a better accuracy. CONCLUSION Our study shows that RA can be supplemented by MRI in a specialized center to provide the most accurate diagnosis in GCA revealed by visual signs.
European Radiology, 2021
To assess the impact of timing from visual symptoms’ onset to diffusion-weighted (DW) 3 T MRI com... more To assess the impact of timing from visual symptoms’ onset to diffusion-weighted (DW) 3 T MRI completion to detect ischemic changes of the optic disc and optic nerve in AION patients. This IRB-approved retrospective single-center study included 3 T MRI data from 126 patients with AION and 111 controls with optic neuritis treated between January 2015 and May 2020. Two radiologists blinded to all data individually analyzed imaging. A senior neuroradiologist resolved any discrepancies by consensus. The primary judgment criterion was the restricted diffusion of the optic disc and/or the optic nerve assessed subjectively on the ADC maps. ADC values were also measured. Spearman rank correlations were used to examine the relationships between timing from visual symptoms’ onset to MRI completion and both the restricted diffusion and the ADC values. One hundred twenty-six patients (47/126 [37.3%] women and 79/126 [62.7%] men, mean age 69.1 ± 13.7 years) with AION were included. Restricted diffusion of the optic disc in AION eyes was more frequent in the early MRI group than in the late MRI group: 35/49 (71.4%) eyes versus 3/83 (3.6%) eyes, p < 0.001. ADC values of the pathological optic discs and optic nerves were lower in the early MRI group than in the late MRI group: 0.61 [0.52–0.94] × 10−3 mm2/s versus 1.28 [1.01–1.44] × 10−3 mm2/s, p < 0.001, and 0.74 [0.61–0.88] × 10−3 mm2/s versus 0.89 [0.72–1.10] × 10−3 mm2/s, p < 0.001, respectively. DWI MRI showed good diagnostic performance to detect AION when performed early after the onset of visual symptoms. • Restricted diffusion of the optic disc in eyes affected by AION was significantly more likely to be observed in patients who had undergone MRI within 5 days after onset of visual symptoms. • ADC values of the pathological optic discs and optic nerves were significantly lower in patients who had undergone MRI within 5 days after onset of visual symptoms of AION: 0.61 × 10−3mm2/s versus 1.28 × 10−3mm2/s, p < 0.001, and 0.74 × 10−3mm2/s versus 0.89 × 10−3mm2/s, p < 0.001, respectively. • The optimal threshold for timing from visual symptoms’ onset to MRI completion to detect restricted diffusion of the optic disc and/or optic nerve was 5 days, with an AUC of 0.88 (CI95%: 0.82–0.94).
Neurology, 2017
Objective: To assess the safety of a single intravitreal injection (IVI) of rAAV2/2- ND4 (GS010),... more Objective: To assess the safety of a single intravitreal injection (IVI) of rAAV2/2- ND4 (GS010), an investigational gene therapy for vision loss due to ND4 LHON. Background: LHON causes severe bilateral central vision loss. rAAV2/2- ND4 is a gene therapy utilizing allotopic expression to deliver wildtype ND4 protein to mitochondria. Design/Methods: A Phase I/IIa (NCT02064569) dose-escalation safety study enrolled ND4 LHON subjects, who received a single IVI of rAAV2/2- ND4 in their worst-seeing eye. Occurrence of adverse events (AE) was the primary objective; secondary objectives included immune response to AAV and evaluation of visual function. Results: Fifteen patients were enrolled. No systemic side effects or SAE were related to study drug or procedures. Common ocular side effects included inflammation, intraocular pressure (IOP) elevation and keratopathy. Ocular inflammation, expected based on pre-clinical studies, included anterior chamber or vitreous inflammation, was mostly...
European Radiology, 2019
Objectives To compare the diagnostic accuracy of 3D versus 2D contrast-enhanced vessel-wall (CE-V... more Objectives To compare the diagnostic accuracy of 3D versus 2D contrast-enhanced vessel-wall (CE-VW) MRI of extracranial and intracranial arteries in the diagnosis of GCA. Methods This prospective two-center study was approved by a national research ethics board and enrolled participants from December 2014 to October 2017. A protocol including both a 2D and a 3D CE-VW MRI at 3 T was performed in all patients. Two neuroradiologists, blinded to clinical data, individually analyzed separately and in random order 2D and 3D sequences in the axial plane only or with reformatting. The primary judgment criterion was the presence of GCA-related inflammatory changes of extracranial arteries. Secondary judgment criteria included inflammatory changes of intracranial arteries and the presence of artifacts. A McNemar's test was used to compare 2D to 3D CE-VW MRIs. Results Seventy-nine participants were included in the study (42 men and 37 women, mean age 75 (± 9.5 years)). Fifty-one had a final diagnosis of GCA. Reformatted 3D CE-VW was significantly more sensitive than axial-only 3D CE-VW or 2D CE-VW when showing inflammatory change of extracranial arteries: 41/51(80%) versus 37/51 (73%) (p = 0.046) and 35/50 (70%) (p = 0.03). Reformatted 3D CE-VW was significantly more specific than 2D CE-VW: 27/27 (100%) versus 22/26 (85%) (p = 0.04). 3D CE-VW showed higher sensitivity than 2D CE-VW when detecting inflammatory changes of intracranial arteries: 10/51(20%) versus 4/50(8%), p = 0.01. Interobserver agreement was excellent for both 2D and 3D CE-VW MRI: κ = 0.84 and 0.82 respectively. Conclusions 3D CE-VW MRI supported more accurate diagnoses of GCA than 2D CE-VW. Key Points • 3D contrast-enhanced vessel-wall magnetic resonance imaging is a high accuracy, non-invasive diagnostic tool used to diagnose giant cell arteritis. • 3D contrast-enhanced vessel-wall imaging is feasible for clinicians to complete within a relatively short time, allowing immediate assessment of extra and intracranial arteries. • 3D contrast-enhanced vessel-wall magnetic resonance imaging might be considered a diagnostic tool when intracranial manifestation of GCA is suspected.
European Journal of Neurology, 2019
Background and purpose: There are few clinico-radiological data on optic neuritis (ON) with myeli... more Background and purpose: There are few clinico-radiological data on optic neuritis (ON) with myelin oligodendrocyte glycoprotein antibody (MOG-IgG). The objective was to characterize the clinico-radiological phenotype and outcome of patients with MOG-IgG-related ON. Methods: The records of all adult patients admitted in three medical centres with MOG-IgG-associated ON who underwent orbital and brain magnetic resonance imaging (MRI) at the acute phase were reviewed. Spinal cord MRI within 1 month from the ON and all of the follow-up MRI were reviewed. Results: Of 62 patients, 41.9% had bilateral ON and 66.2% optic disc swelling. On initial MRI, lesions were anterior (92%), extensive (63%) and associated with optic perineuritis (46.6%). Silent brain lesions were found in 51.8% of patients but were mainly non-specific (81%). Of 39 individuals with spinal MRI at onset, nine had abnormal findings (four were asymptomatic). Two symptomatic patients had longitudinally extensive myelitis with concurrent Hsign. At last follow-up, 5% of patients had visual acuity ≤0.1. Brain MRI remained unchanged in 41 patients (87%). Conclusions: Our study supports a mostly benign ophthalmological course of MOG-IgG-associated ON, despite initially longitudinally extensive lesions and development of optic nerve atrophy on orbital MRI. Spinal MRI could be of interest in detecting silent suggestive lesions.
Journal of Neuroradiology, 2018
L'ataxie est un symptôme pouvant être issu d'une maladie neurodégénérative résultant d'altération... more L'ataxie est un symptôme pouvant être issu d'une maladie neurodégénérative résultant d'altérations du tronc cérébral, du cervelet et/ou spinocérébelleux. L'âge d'apparition des symptômes peut varier considérablement allant de l'enfance à la fin de l'âge adulte. Les ataxies cérébelleuses héréditaires sont considérées comme l'un des groupes les plus complexes en neurogénétique. En plus de leur hétérogénéité génétique, il existe une importante variabilité phénotypique dans l'expression de l'atteinte cérébelleuse, compliquant la recherche des mutations génétiques. Nous proposons une méthode d'analyse simple, basée sur l'analyse en IRM de l'atrophie cérébelleuse et du vermis ; la présence ou non d'hyposignaux SWI des noyaux dentelés ; une atrophie de la moelle cervicale ; des hypersignaux T2 des pédoncules cérébelleux moyens et/ou la présence de télangiectasies ; ainsi que sur l'âge de début des symptômes, afin de reconnaître les principales étiologies d'ataxies neurodégénératives héréditaires. Déclaration de liens d'intérêts Les auteurs déclarent ne pas avoir de liens d'intérêts.
Journal of Neurology, 2017
Journal of Neurology, 2016
Optic neuritis could lead to severe visual impairment despite corticosteroids. Our aim was to eva... more Optic neuritis could lead to severe visual impairment despite corticosteroids. Our aim was to evaluate the rate of visual improvement in patients treated with plasma exchange (PLEX) for severe steroid unresponsive optic neuritis and to identify predictive factors of outcome. Thirty-four patients (41 optic nerves damaged) with remaining visual acuity of 0.1 or less despite steroid pulse therapy were treated with PLEX from September 2010 to May 2015. Demographic and clinical neuro-ophthalmic findings, and spectral domain-optical coherence tomography data before PLEX treatment were analyzed. The mean symptom duration before PLEX was 34.6 days (median 28 days; range 6-92 days). After PLEX, the median final visual acuity was 0.8 and in 56 % of cases, final acuity was 0.5 or better. Past history of ipsilateral optic neuritis was associated significantly with poor outcome defined as final acuity less than 0.5. No significant difference in the visual outcome after PLEX was found between multiple sclerosis and neuromyelitis optica. In conclusion, this observational study showed that PLEX as second-line therapy led to a functionally important visual recovery in more than half patients with severe optic neuritis.
Bulletin des sociétés d'ophtalmologie de France, 1986
Journal of Vision, 2011
Abstract Literature data suggest that cortical reorganization can occur following peripheral as w... more Abstract Literature data suggest that cortical reorganization can occur following peripheral as well as cerebral visual system damage. Nevertheless, visual processing adaptation to either type of damage remains unclear especially in respect to the central visual field ( ...
Journal Français d'Ophtalmologie, 2007
Introduction La toxoplasmose est une etiologie frequente d’uveites posterieures chez le sujet imm... more Introduction La toxoplasmose est une etiologie frequente d’uveites posterieures chez le sujet immunocompetent. Elle se manifeste typiquement sous forme de foyers retinochoroidiens. La localisation papillaire est rare. Materiels et Methodes Nous rapportons trois cas de patientes atteintes de toxoplasmose papillaire. Observation Les 3 patientes etaient adressees pour baisse de l’acuite visuelle unilaterale en rapport avec un œdeme papillaire unilateral sans hyalite. La premiere patiente a recu une corticotherapie seule, qui a entraine rapidement une majoration de l’œdeme et l’apparition d’un foyer papillaire blanchâtre, faisant evoquer le diagnostic de toxoplasmose. La seconde patiente a ete adressee pour une suspicion de nevrite optique avec un profond scotome centro-caecal. Il existait un œdeme papillaire modere dont la partie temporale etait blanchâtre, faisant suspecter une retinochoroidite toxoplasmique. La troisieme patiente etait adressee pour baisse de l’acuite visuelle severe compliquant un important œdeme papillaire hemorragique. L’aspect blanchâtre de la saillie papillaire et l’angiographie etaient evocateurs de toxoplasmose. Dans les trois cas, une fois le diagnostic pose, le traitement a associe des antiparasitaires (sulfadiazine, pyrimethamine) rapidement suivis d’une corticotherapie a forte dose. Ce traitement a permis de stopper l’extension du foyer afin de limiter le retentissement sur la fonction visuelle. Discussion L’atteinte papillaire dans l’infection toxoplasmique peut etre retrouvee en l’absence de cicatrices evoquant des poussees anterieures et en l’absence de cellules dans le vitre. L’etude de nos trois cas illustre la difficulte diagnostique qui peut etre alors rencontree. Nous discuterons de la conduite a tenir diagnostique et therapeutique dans ces cas. Conclusion La toxoplasmose papillaire est une entite rare qui doit etre evoquee systematiquement devant un œdeme papillaire unilateral. Une fois le diagnostic pose, le traitement doit etre instaure en urgence afin de preserver la fonction visuelle de l’œil atteint.
Graefe's Archive for Clinical and Experimental Ophthalmology, 2012
Diagnostic and Interventional Imaging, 2021
PURPOSE The purpose of this study was to identify which combination of imaging modalities should ... more PURPOSE The purpose of this study was to identify which combination of imaging modalities should be used to obtain the best diagnostic performance for the non-invasive diagnosis of giant cell arteritis (GCA). MATERIALS AND METHODS This IRB-approved prospective single-center study enrolled participants presenting with a suspected diagnosis of GCA from December 2014 to October 2017. Participants underwent high-resolution 3T magnetic resonance imaging (MRI), temporal and extra-cranial arteries ultrasound and retinal angiography (RA), prior to temporal artery biopsy (TAB). Diagnostic accuracy of each imaging modality alone, then a combination of several imaging modalities, was evaluated. Several algorithms were constructed to test optimal combinations using McNemar test. RESULTS Forty-five participants (24 women, 21 men) with mean age of 75.4 ± 16 (SD) years (range: 59-94 years) were enrolled; of these 43/45 (96%) had ophthalmological symptoms. Diagnosis of GCA was confirmed in 25/45 (56%) patients. Sensitivity and specificity of MRI, ultrasound and RA alone were 100% (25/25; 95% CI: 86-100) and 86% (19/22; 95% CI: 65-97), 88% (22/25; 95% CI: 69-97) and 84% (16/19; 95% CI: 60-97), 94% (15/16; 95% CI: 70-100) and 74% (14/19; 95% CI: 49-91), respectively. Sensitivity, specificity, positive predictive and negative predictive values ranged from 95 to 100% (95% CI: 77-100), 67 to 100% (95% CI: 38-100), 81 to 100% (95% CI: 61-100) and 91 to 100% (95% CI: 59-100) when combining several imaging tests, respectively. The diagnostic algorithm with the overall best diagnostic performance was the one starting with MRI, followed either by ultrasound or RA, yielding 100% sensitivity (22/22; 95% CI: 85-100%) 100% (15/15; 95% CI: 78-100) and 100% accuracy (37/37; 95% CI: 91-100). CONCLUSION The use of MRI as the first imaging examination followed by either ultrasound or RA reaches high degrees of performance for the diagnosis of GCA and is recommended in daily practice.
Progress in Retinal and Eye Research
Elsevier Masson, Jan 2, 2019
Clinical Toxicology, 2021
Supplemental material, Table_S3_mai13 for Visual field loss and structure–function relationships ... more Supplemental material, Table_S3_mai13 for Visual field loss and structure–function relationships in optic neuritis associated with myelin oligodendrocyte glycoprotein antibody by Romain Deschamps, Manon Philibert, Cedric Lamirel, Jerome Lambert, Vivien Vasseur, Antoine Gueguen, Caroline Bensa, Augustin Lecler, Romain Marignier, Catherine Vignal and Olivier Gout in Multiple Sclerosis Journal
Journal of Neuro-Ophthalmology, 2022
BACKGROUND Giant cell arteritis (GCA) is a vasculitis often revealed by visual signs. Diagnosis i... more BACKGROUND Giant cell arteritis (GCA) is a vasculitis often revealed by visual signs. Diagnosis is challenging and urgent. Retinal angiography (RA) and MRI allow effective diagnosis. We compared those and proposed an imaging-based approach to diagnose GCA in ophthalmological practice. METHODS We conducted a retrospective study based on the data collected from patients suspected to have GCA on ophthalmological findings. Fluorescein (FA) and indocyanine green (ICG) RAs and MRI were performed and compared with final diagnosis. RESULTS Among the 41 patients included, 25 were diagnosed with GCA. Sensitivities and specificities of FA and ICG were not different. MRI showed a higher sensitivity and specificity. The approach consisting in performing RA followed by MRI provided a better accuracy. CONCLUSION Our study shows that RA can be supplemented by MRI in a specialized center to provide the most accurate diagnosis in GCA revealed by visual signs.
European Radiology, 2021
To assess the impact of timing from visual symptoms’ onset to diffusion-weighted (DW) 3 T MRI com... more To assess the impact of timing from visual symptoms’ onset to diffusion-weighted (DW) 3 T MRI completion to detect ischemic changes of the optic disc and optic nerve in AION patients. This IRB-approved retrospective single-center study included 3 T MRI data from 126 patients with AION and 111 controls with optic neuritis treated between January 2015 and May 2020. Two radiologists blinded to all data individually analyzed imaging. A senior neuroradiologist resolved any discrepancies by consensus. The primary judgment criterion was the restricted diffusion of the optic disc and/or the optic nerve assessed subjectively on the ADC maps. ADC values were also measured. Spearman rank correlations were used to examine the relationships between timing from visual symptoms’ onset to MRI completion and both the restricted diffusion and the ADC values. One hundred twenty-six patients (47/126 [37.3%] women and 79/126 [62.7%] men, mean age 69.1 ± 13.7 years) with AION were included. Restricted diffusion of the optic disc in AION eyes was more frequent in the early MRI group than in the late MRI group: 35/49 (71.4%) eyes versus 3/83 (3.6%) eyes, p < 0.001. ADC values of the pathological optic discs and optic nerves were lower in the early MRI group than in the late MRI group: 0.61 [0.52–0.94] × 10−3 mm2/s versus 1.28 [1.01–1.44] × 10−3 mm2/s, p < 0.001, and 0.74 [0.61–0.88] × 10−3 mm2/s versus 0.89 [0.72–1.10] × 10−3 mm2/s, p < 0.001, respectively. DWI MRI showed good diagnostic performance to detect AION when performed early after the onset of visual symptoms. • Restricted diffusion of the optic disc in eyes affected by AION was significantly more likely to be observed in patients who had undergone MRI within 5 days after onset of visual symptoms. • ADC values of the pathological optic discs and optic nerves were significantly lower in patients who had undergone MRI within 5 days after onset of visual symptoms of AION: 0.61 × 10−3mm2/s versus 1.28 × 10−3mm2/s, p < 0.001, and 0.74 × 10−3mm2/s versus 0.89 × 10−3mm2/s, p < 0.001, respectively. • The optimal threshold for timing from visual symptoms’ onset to MRI completion to detect restricted diffusion of the optic disc and/or optic nerve was 5 days, with an AUC of 0.88 (CI95%: 0.82–0.94).
Neurology, 2017
Objective: To assess the safety of a single intravitreal injection (IVI) of rAAV2/2- ND4 (GS010),... more Objective: To assess the safety of a single intravitreal injection (IVI) of rAAV2/2- ND4 (GS010), an investigational gene therapy for vision loss due to ND4 LHON. Background: LHON causes severe bilateral central vision loss. rAAV2/2- ND4 is a gene therapy utilizing allotopic expression to deliver wildtype ND4 protein to mitochondria. Design/Methods: A Phase I/IIa (NCT02064569) dose-escalation safety study enrolled ND4 LHON subjects, who received a single IVI of rAAV2/2- ND4 in their worst-seeing eye. Occurrence of adverse events (AE) was the primary objective; secondary objectives included immune response to AAV and evaluation of visual function. Results: Fifteen patients were enrolled. No systemic side effects or SAE were related to study drug or procedures. Common ocular side effects included inflammation, intraocular pressure (IOP) elevation and keratopathy. Ocular inflammation, expected based on pre-clinical studies, included anterior chamber or vitreous inflammation, was mostly...
European Radiology, 2019
Objectives To compare the diagnostic accuracy of 3D versus 2D contrast-enhanced vessel-wall (CE-V... more Objectives To compare the diagnostic accuracy of 3D versus 2D contrast-enhanced vessel-wall (CE-VW) MRI of extracranial and intracranial arteries in the diagnosis of GCA. Methods This prospective two-center study was approved by a national research ethics board and enrolled participants from December 2014 to October 2017. A protocol including both a 2D and a 3D CE-VW MRI at 3 T was performed in all patients. Two neuroradiologists, blinded to clinical data, individually analyzed separately and in random order 2D and 3D sequences in the axial plane only or with reformatting. The primary judgment criterion was the presence of GCA-related inflammatory changes of extracranial arteries. Secondary judgment criteria included inflammatory changes of intracranial arteries and the presence of artifacts. A McNemar's test was used to compare 2D to 3D CE-VW MRIs. Results Seventy-nine participants were included in the study (42 men and 37 women, mean age 75 (± 9.5 years)). Fifty-one had a final diagnosis of GCA. Reformatted 3D CE-VW was significantly more sensitive than axial-only 3D CE-VW or 2D CE-VW when showing inflammatory change of extracranial arteries: 41/51(80%) versus 37/51 (73%) (p = 0.046) and 35/50 (70%) (p = 0.03). Reformatted 3D CE-VW was significantly more specific than 2D CE-VW: 27/27 (100%) versus 22/26 (85%) (p = 0.04). 3D CE-VW showed higher sensitivity than 2D CE-VW when detecting inflammatory changes of intracranial arteries: 10/51(20%) versus 4/50(8%), p = 0.01. Interobserver agreement was excellent for both 2D and 3D CE-VW MRI: κ = 0.84 and 0.82 respectively. Conclusions 3D CE-VW MRI supported more accurate diagnoses of GCA than 2D CE-VW. Key Points • 3D contrast-enhanced vessel-wall magnetic resonance imaging is a high accuracy, non-invasive diagnostic tool used to diagnose giant cell arteritis. • 3D contrast-enhanced vessel-wall imaging is feasible for clinicians to complete within a relatively short time, allowing immediate assessment of extra and intracranial arteries. • 3D contrast-enhanced vessel-wall magnetic resonance imaging might be considered a diagnostic tool when intracranial manifestation of GCA is suspected.
European Journal of Neurology, 2019
Background and purpose: There are few clinico-radiological data on optic neuritis (ON) with myeli... more Background and purpose: There are few clinico-radiological data on optic neuritis (ON) with myelin oligodendrocyte glycoprotein antibody (MOG-IgG). The objective was to characterize the clinico-radiological phenotype and outcome of patients with MOG-IgG-related ON. Methods: The records of all adult patients admitted in three medical centres with MOG-IgG-associated ON who underwent orbital and brain magnetic resonance imaging (MRI) at the acute phase were reviewed. Spinal cord MRI within 1 month from the ON and all of the follow-up MRI were reviewed. Results: Of 62 patients, 41.9% had bilateral ON and 66.2% optic disc swelling. On initial MRI, lesions were anterior (92%), extensive (63%) and associated with optic perineuritis (46.6%). Silent brain lesions were found in 51.8% of patients but were mainly non-specific (81%). Of 39 individuals with spinal MRI at onset, nine had abnormal findings (four were asymptomatic). Two symptomatic patients had longitudinally extensive myelitis with concurrent Hsign. At last follow-up, 5% of patients had visual acuity ≤0.1. Brain MRI remained unchanged in 41 patients (87%). Conclusions: Our study supports a mostly benign ophthalmological course of MOG-IgG-associated ON, despite initially longitudinally extensive lesions and development of optic nerve atrophy on orbital MRI. Spinal MRI could be of interest in detecting silent suggestive lesions.
Journal of Neuroradiology, 2018
L'ataxie est un symptôme pouvant être issu d'une maladie neurodégénérative résultant d'altération... more L'ataxie est un symptôme pouvant être issu d'une maladie neurodégénérative résultant d'altérations du tronc cérébral, du cervelet et/ou spinocérébelleux. L'âge d'apparition des symptômes peut varier considérablement allant de l'enfance à la fin de l'âge adulte. Les ataxies cérébelleuses héréditaires sont considérées comme l'un des groupes les plus complexes en neurogénétique. En plus de leur hétérogénéité génétique, il existe une importante variabilité phénotypique dans l'expression de l'atteinte cérébelleuse, compliquant la recherche des mutations génétiques. Nous proposons une méthode d'analyse simple, basée sur l'analyse en IRM de l'atrophie cérébelleuse et du vermis ; la présence ou non d'hyposignaux SWI des noyaux dentelés ; une atrophie de la moelle cervicale ; des hypersignaux T2 des pédoncules cérébelleux moyens et/ou la présence de télangiectasies ; ainsi que sur l'âge de début des symptômes, afin de reconnaître les principales étiologies d'ataxies neurodégénératives héréditaires. Déclaration de liens d'intérêts Les auteurs déclarent ne pas avoir de liens d'intérêts.
Journal of Neurology, 2017
Journal of Neurology, 2016
Optic neuritis could lead to severe visual impairment despite corticosteroids. Our aim was to eva... more Optic neuritis could lead to severe visual impairment despite corticosteroids. Our aim was to evaluate the rate of visual improvement in patients treated with plasma exchange (PLEX) for severe steroid unresponsive optic neuritis and to identify predictive factors of outcome. Thirty-four patients (41 optic nerves damaged) with remaining visual acuity of 0.1 or less despite steroid pulse therapy were treated with PLEX from September 2010 to May 2015. Demographic and clinical neuro-ophthalmic findings, and spectral domain-optical coherence tomography data before PLEX treatment were analyzed. The mean symptom duration before PLEX was 34.6 days (median 28 days; range 6-92 days). After PLEX, the median final visual acuity was 0.8 and in 56 % of cases, final acuity was 0.5 or better. Past history of ipsilateral optic neuritis was associated significantly with poor outcome defined as final acuity less than 0.5. No significant difference in the visual outcome after PLEX was found between multiple sclerosis and neuromyelitis optica. In conclusion, this observational study showed that PLEX as second-line therapy led to a functionally important visual recovery in more than half patients with severe optic neuritis.
Bulletin des sociétés d'ophtalmologie de France, 1986
Journal of Vision, 2011
Abstract Literature data suggest that cortical reorganization can occur following peripheral as w... more Abstract Literature data suggest that cortical reorganization can occur following peripheral as well as cerebral visual system damage. Nevertheless, visual processing adaptation to either type of damage remains unclear especially in respect to the central visual field ( ...
Journal Français d'Ophtalmologie, 2007
Introduction La toxoplasmose est une etiologie frequente d’uveites posterieures chez le sujet imm... more Introduction La toxoplasmose est une etiologie frequente d’uveites posterieures chez le sujet immunocompetent. Elle se manifeste typiquement sous forme de foyers retinochoroidiens. La localisation papillaire est rare. Materiels et Methodes Nous rapportons trois cas de patientes atteintes de toxoplasmose papillaire. Observation Les 3 patientes etaient adressees pour baisse de l’acuite visuelle unilaterale en rapport avec un œdeme papillaire unilateral sans hyalite. La premiere patiente a recu une corticotherapie seule, qui a entraine rapidement une majoration de l’œdeme et l’apparition d’un foyer papillaire blanchâtre, faisant evoquer le diagnostic de toxoplasmose. La seconde patiente a ete adressee pour une suspicion de nevrite optique avec un profond scotome centro-caecal. Il existait un œdeme papillaire modere dont la partie temporale etait blanchâtre, faisant suspecter une retinochoroidite toxoplasmique. La troisieme patiente etait adressee pour baisse de l’acuite visuelle severe compliquant un important œdeme papillaire hemorragique. L’aspect blanchâtre de la saillie papillaire et l’angiographie etaient evocateurs de toxoplasmose. Dans les trois cas, une fois le diagnostic pose, le traitement a associe des antiparasitaires (sulfadiazine, pyrimethamine) rapidement suivis d’une corticotherapie a forte dose. Ce traitement a permis de stopper l’extension du foyer afin de limiter le retentissement sur la fonction visuelle. Discussion L’atteinte papillaire dans l’infection toxoplasmique peut etre retrouvee en l’absence de cicatrices evoquant des poussees anterieures et en l’absence de cellules dans le vitre. L’etude de nos trois cas illustre la difficulte diagnostique qui peut etre alors rencontree. Nous discuterons de la conduite a tenir diagnostique et therapeutique dans ces cas. Conclusion La toxoplasmose papillaire est une entite rare qui doit etre evoquee systematiquement devant un œdeme papillaire unilateral. Une fois le diagnostic pose, le traitement doit etre instaure en urgence afin de preserver la fonction visuelle de l’œil atteint.
Graefe's Archive for Clinical and Experimental Ophthalmology, 2012
Diagnostic and Interventional Imaging, 2021
PURPOSE The purpose of this study was to identify which combination of imaging modalities should ... more PURPOSE The purpose of this study was to identify which combination of imaging modalities should be used to obtain the best diagnostic performance for the non-invasive diagnosis of giant cell arteritis (GCA). MATERIALS AND METHODS This IRB-approved prospective single-center study enrolled participants presenting with a suspected diagnosis of GCA from December 2014 to October 2017. Participants underwent high-resolution 3T magnetic resonance imaging (MRI), temporal and extra-cranial arteries ultrasound and retinal angiography (RA), prior to temporal artery biopsy (TAB). Diagnostic accuracy of each imaging modality alone, then a combination of several imaging modalities, was evaluated. Several algorithms were constructed to test optimal combinations using McNemar test. RESULTS Forty-five participants (24 women, 21 men) with mean age of 75.4 ± 16 (SD) years (range: 59-94 years) were enrolled; of these 43/45 (96%) had ophthalmological symptoms. Diagnosis of GCA was confirmed in 25/45 (56%) patients. Sensitivity and specificity of MRI, ultrasound and RA alone were 100% (25/25; 95% CI: 86-100) and 86% (19/22; 95% CI: 65-97), 88% (22/25; 95% CI: 69-97) and 84% (16/19; 95% CI: 60-97), 94% (15/16; 95% CI: 70-100) and 74% (14/19; 95% CI: 49-91), respectively. Sensitivity, specificity, positive predictive and negative predictive values ranged from 95 to 100% (95% CI: 77-100), 67 to 100% (95% CI: 38-100), 81 to 100% (95% CI: 61-100) and 91 to 100% (95% CI: 59-100) when combining several imaging tests, respectively. The diagnostic algorithm with the overall best diagnostic performance was the one starting with MRI, followed either by ultrasound or RA, yielding 100% sensitivity (22/22; 95% CI: 85-100%) 100% (15/15; 95% CI: 78-100) and 100% accuracy (37/37; 95% CI: 91-100). CONCLUSION The use of MRI as the first imaging examination followed by either ultrasound or RA reaches high degrees of performance for the diagnosis of GCA and is recommended in daily practice.