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Purpose:: Myelin oligodendrocyte glycoprotein autoantibodies are associated with certain optic neuritis. Little data are known about the specificity of the initial ophthalmologic presentation., Methods:: A monocentric retrospective study (2013-2017) of all patients diagnosed with myelin oligodendrocyte glycoprotein+ optic neuritis in a tertiary ophthalmologic unit was conducted. The primary objective was to define the clinical ophthalmologic description of the first episode. The secondary objective was to evaluate the evolution and final diagnosis., Results:: A total of nine patients were included. There was no female predominance (sex ratio f/m = 0.8). At the first optic neuritis episode, the average age was 39.3 years (17-67 years, standard deviation: 18.4). Initial visual acuity was low (+1.07logMAR, standard deviation: 0.77); 5 eyes out of 15 had visual acuity +2logMAR or worse. Optic neuritis was mostly painful (8/9) and bilateral (6/9) but asymmetric. Optic disk swelling was reported in 9/15 eyes and 7/9 patients and was significantly associated with lower visual acuity in the acute phase (+1.46logMAR, standard deviation: 0.67 vs +0.5, standard deviation: 0.55; p = 0.03). After a mean observation period of 3.3 years (0.6-9.4 years, standard deviation: 3.4), median visual acuity was 0.05logMAR. All five patients were followed up for more than 1 year (5.4 years, standard deviation: 3.2) had 3-8 relapses (mean: 4.4, standard deviation: 2.1; annualized relapse rate: 1.2, standard deviation: 0.9). Final diagnosis was chronic relapsing idiopathic optic neuritis (n = 4), clinically isolated optic neuritis (n = 3), and neuromyelitis optica spectrum disorder aquaporin 4- (n = 2)., Conclusion:: Myelin oligodendrocyte glycoprotein+ optic neuritis has an atypical clinical presentation compared with multiple sclerosis and neuromyelitis optica spectrum disorder aquaporin 4+. Its evolution is closer to neuromyelitis optica spectrum disorder aquaporin 4+, with a better visual outcome.

Purpose: To define the prognostic factors for success and to evaluate the predictability of intracorneal ring segments (ICRS) in the treatment of keratoconus., Methods: In this retrospective study conducted at the University Hospital of Nantes, Keraring ICRS were implanted in 75 eyes of 65 patients with keratoconus. Best spectacle corrected visual acuity (BSCVA), manifest refraction and corneal topography were analysed. To define prognostic factors, we compared the results of 2 groups: "IMP" (gain of at least 2 lines of BSCVA) and "ROS" (the others). We evaluated the predictability of the nomogram with a mathematical model proposed by Pena-Garcia et al. (IOVS 2012)., Results: At 3 months, BSCVA improved from 0.3 to 0.2 logMAR (P<0.05). A total of 61 % of the patients experienced a gain of at least 1 line of BSCVA. Spherical equivalent decreased by 2.32 diopters (D), cylinder decreased by 2.47 D, and maximal keratometry by 2.62 D (P<0.05 for each compared with preoperative values). A total of 90 % of the patients whose BSCVA did not improve achieved a significant refractive improvement. A preoperative BSCVA>0.3 logMAR is a prognostic factor for gain of at least 2 lines of BSCVA (P=1.6E-3). Predictability was fair: only 43 % had a±1D difference from the spherical equivalent predicted by the nomogram. There was no statistically significative difference between gain or loss of BSCVA predicted by the mathematical model and the postoperative results., Conclusions: ICRS are visually and refractively effective. Predictability could be improved by using mathematical models and knowledge of prognostic factors for success, allowing for better patient selection., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Brown's syndrome is related to an abnormality of the superior oblique muscle: it is manifested by an oculomotor disorder with active and passive limitation of elevation in adduction, the field of action of the inferior oblique muscle. The origin is congenital or acquired secondary to multiple causes - inflammatory-infectious, traumatic or iatrogenic. The clinical and paraclinical signs are suggestive. Cerebral and orbital imaging including CT and magnetic resonance imaging (MRI) is essential for the diagnosis and management of congenital or acquired Brown's syndrome. The course may yield improvement (spontaneous or after etiological treatment) or a steady state. Treatment is rarely surgical. The indication for surgery requires specific functional and clinical signs: torticollis, hypotropia in primary position, associated strabismus and impaired binocular vision; the long-term results of the various surgical techniques are variable., (Copyright © 2018. Published by Elsevier Masson SAS.)

Objective: To describe clinical and radiologic features associated with myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) in a large French nationwide adult cohort, to assess baseline prognostic features of MOG-Ab-associated diseases after a first acute demyelinating syndrome, and to evaluate the clinical value of MOG-Ab longitudinal analysis., Methods: Clinical data were obtained from 197 MOG-Ab-positive patients ≥18 years of age. Complete imaging data were available in 108, and 54 serum samples were eligible for longitudinal evaluation. For survival analysis comparison, 169 aquaporin-4 antibody (AQP4-Ab)-positive patients from the NOMADMUS database were included., Results: Median age at onset was 36.46 (range 18.0-76.8) years, and patients were predominantly white (92.9%) with male:female ratio, 1.1. Clinical phenotype at onset included optic neuritis or myelitis in 90.86%, isolated brainstem or encephalopathy syndromes in 6.6%, and a combination of syndromes in 2.5%. Distinctive brain MRI findings in MOG-Ab-positive patients were thalamic and pontine lesions. Cortical and leptomeningeal lesions were found in 16.3% and 6.1%, respectively. The probability of reaching a first relapse after 2 and 5 years was 44.8% and 61.8%, respectively. MOG-Ab-positive patients were at lower risk at presentation of further clinical relapse (hazard ratio [HR] 0.45, 95% confidence interval [CI] 0.26-0.79) compared to AQP4-Ab-positive individuals. MOG-Ab-positive individuals had a lower risk of reaching Disability Status Scale score of 3.0 (HR 0.46, 95% CI 0.22-0.94) and visual acuity of 20/100 (HR 0.23, 95% CI 0.07-0.72). Finally, MOG-Ab titers were higher at relapse than in remission ( p = 0.009)., Conclusion: In adults, MOG-Ab-associated disease extends beyond clinical and radiologic abnormalities in the optic nerve and spinal cord. Despite the relapsing course, the overall visual and motor outcome is better compared with AQP4-Ab-positive patients., (© 2018 American Academy of Neurology.)

Purpose: Abnormal torsion could be associated with cyclovertical strabismus, but torsion measurements are not reliable in children. To assess an objective fundus torsion evaluation in a paediatric population, we used Non-Mydriatic Fundus photography (NMFP) in healthy and cyclovertical strabismus patients to evaluate the disc-foveal angle over time and observers., Methods: We used a retrospective set of NMFP including 24 A or V-pattern strabismus and 27 age-matched normal children (mean age 6.4 and 6.7 years respectively), taken during 2 distinct follow-up consultations (separated by 251 and 479 days respectively). Each disc-foveal angle measurement (from which the ocular torsion can be assessed) was performed by 5 different observers, using graphical software and based on reproducible fundus anatomical marks. Statistical analysis was performed with a multivariate ANOVA using group, time and observers as factors, in addition to intraclass coefficient correlation (ICC) to assess measurement reproducibility., Results: A significant difference of disc-foveal angle measures was observed between groups (p<0,001): 18.73° (SD = 6.42), -3,25° (SD = 5.51) and 6,89° (SD = 4,41) respectively for V-pattern, A- pattern and normal subjects. Neither observers (F = 0,2028 p = 0,9369) nor time between 1st and 2nd NMFP (F = 0,6312 p = 0,4271) seem to influence the measure of disc-foveal angle. The evaluation of disc-foveal angle was very reproducible between observers (ICC>0,97)., Conclusion: Abnormal amount of objective torsion could be associated with alphabet-pattern strabismus. Disc-foveal angle evaluation by NMFP in a children population appears as a non-invasive, reliable and reproducible method.

The aim of this study was the evaluation of the safety and efficacy of unilateral subretinal injection of the adeno-associated vector (AAV) serotypes 2 and 4 (AAV2/4) RPE65-RPE65 vector in patients with Leber congenital amaurosis (LCA) associated with RPE65 gene deficiency. We evaluated ocular and general tolerance and visual function up to 1 year after vector administration in the most severely affected eye in nine patients with retinal degeneration associated with mutations in the RPE65 gene. Patients received either low (1.22 × 10 10 to 2 × 10 10 vector genomes [vg]) or high (between 3.27 × 10 10 and 4.8 × 10 10 vg) vector doses. An ancillary study, in which six of the original nine patients participated, extended the follow-up period to 2-3.5 years. All patients showed good ophthalmological and general tolerance to the rAAV2/4-RPE65-RPE65 vector. We observed a trend toward improved visual acuity in patients with nystagmus, stabilization and improvement of the visual field, and cortical activation along visual pathways during fMRI analysis. OCT analysis after vector administration revealed no retinal thinning, except in cases of macular detachment. Our findings show that the rAAV2/4.RPE65.RPE65 vector was well tolerated in nine patients with RPE65-associated LCA. Efficacy parameters varied between patients during follow-up., (Copyright © 2017 The American Society of Gene and Cell Therapy. Published by Elsevier Inc. All rights reserved.)

Introduction: A growing number of articles have shown the negative impact of strabismus on self image, relationships with others and professional life. The AS-20 is a standardized questionnaire in English measuring the psychosocial impact of strabismus in adults. The goal of this study is to validate the AS-20 in the French language., Participants: Three hundred and ninety one patients took part in the study: 131 had strabismus (group 1), 128 other ocular diseases (group 2) and 132 "normal" patients (group 3)., Results: The AS-20 scale showed good psychometrical properties in 2 or 4 dimensions: internal consistency was very good (Cronbach's alpha coefficient>0.9 in all dimensions and overall) and the reproducibility was satisfactory (intra class coefficient>0.7). The comparison of the scores in the 2 dimension scale showed significant differences between the groups (P<0.001): lower score in strabismus (63.9±18.3) than in other ocular diseases (73.5±17.8) and normal group (89.4±12.0) (divergent validity). The 4 dimension psychometric analysis was performed by removing 2 items and grouping the 18 others in 4 groups. It confirmed the divergent and convergent validity, internal consistency and reproducibility of the scale. The results of the confirmatory factor analysis were better with the 4 dimension scale than the 2 dimension scale (adjustment coefficients>0.9)., Conclusion: We now have access to a health related questionnaire in French to quantify the impact of strabismus on quality of life and measure the results of treatment., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Background Although acute central retinal artery occlusion is as a stroke in the carotid territory (retinal artery), its management remains controversial. The aim of this study was to assess the feasibility and safety of intravenous thrombolysis delivered within 6 h of central retinal artery occlusion in French stroke units. Methods We performed a retrospective analysis of patients treated with intravenous alteplase (recombinant tissue-plasminogen activator), based on stroke units thrombolysis registers from June 2005 to June 2015, and we selected those who had acute central retinal artery occlusion. The feasibility was assessed by the ratio of patients that had received intravenous alteplase within 6 h after central retinal artery occlusion onset among those who had been admitted to the same hospital for acute central retinal artery occlusion. All adverse events were documented. Results Thirty patients were included. Visual acuity before treatment was limited to "hand motion", or worse, in 90% of the cases. The mean onset-to-needle time was 273 min. The individuals treated with intravenous alteplase for central retinal artery occlusion represented 10.2% of all of the patients hospitalized for central retinal artery occlusion in 2013 and 2014. We observed one occurrence of major bleeding, a symptomatic intracerebral hemorrhage. Conclusion When applied early on, intravenous thrombolysis appears to be feasible and safe, provided that contraindications are given due consideration. Whether intravenous thrombolysis is more effective than conservative therapy remains to be determined. In order to conduct a well-designed prospective randomized control trial, an organized network should be in place.

The management of severe keratoconus requires corneal transplantation, for which the gold standard is deep anterior lamellar keratoplasty (DALK), preserving the healthy Descemet's membrane and endothelium. The safety and reproducibility of corneal cuts have been improved by the evolution of femtosecond lasers in refractive surgery, and femtosecond laser in DALK would seem to provide the same advantages over the manual method. In our retrospective study, we compare functional and anatomical results of femtosecond assisted DALK versus manual trephination DALK in patients with keratoconus in stage 4 of the Krumeich classification. It is a retrospective study including all patients with stage 4 keratoconus who underwent femtosecond laser assisted DALK between November 2012 and November 2015 in Nantes hospital. We compared those patients to a group of patients who underwent manual DALK in the same period, paired by age and maximal keratometry. We assessed visual acuity, pachymetry, endothelial cell density (specular microscopy), and keratometry before surgery and at 4, 8 and 12 months of follow-up. Laser settings and intraoperative complications were recorded. Nineteen patients underwent surgery by femtosecond assisted DALK, 6 women and 12 men with average age 30.2±10.8 years at transplantation. They were paired with a group of 17 patients who underwent manual DALK in order to compare results. Before surgery, mean visual acuity in the femtosecond group was 0.90 logMAR versus 0.89 logMAR in the manual group, showing no statistically significant difference (P=0.96). Both groups were similar in terms of preoperative age, mean keratometry, pachymetry and endothelial cell density. Average visual acuity post-surgery was 0.27, 0.26; and 0.14 logMAR for femtosecond DALK versus 0.27, 0.17 et 0.25 for manual DALK at 4, 8 and 12 months follow-up, respectively showing no statistically significant difference. After surgery, at 4, 8 and 12 months, mean pachymetry was similar in both groups, and average endothelial cell density was 2390 cells/mm 2  in femtoDALK versus 2531 cells/mm 2  in manual DALK at 12 months of follow-up, showing no statistically significant difference (P=0.5726). The rate of Descemet's membrane microperforations during the procedure was low and similar for both groups. Our study allows for a 12 month follow-up, with assessment of visual recovery, anatomical result and endothelial safety in a sample of 19 femtosecond laser assisted DALK with no statistical significant difference versus the manual trephination group. Femtosecond laser allows for increased reproducibility of the DALK procedure without reducing adverse effects during surgery. Femtosecond laser seems to improve the technique of the DALK procedure, and future developments could improve the reproducibility of DALK even further. A medical economics study would be necessary to determine the cost effectiveness of femtosecond laser assisted DALK., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

The management of severe keratoconus requires corneal transplantation, for which the gold standard is deep anterior lamellar keratoplasty (DALK), preserving the healthy Descemet's membrane and endothelium. The safety and reproducibility of corneal cuts have been improved by the evolution of femtosecond lasers in refractive surgery, and femtosecond laser in DALK would seem to provide the same advantages over the manual method. In our retrospective study, we compare functional and anatomical results of femtosecond-assisted DALK versus manual trephination DALK in patients with keratoconus in stage 4 of the Krumeich classification. It is a retrospective study including all patients with stage 4 keratoconus who underwent femtosecond laser-assisted DALK between November 2012 and November 2015 in Nantes university medical center. We compared those patients to a group of patients who underwent manual DALK in the same period, paired by age and maximal keratometry. We assessed visual acuity, pachymetry, endothelial cell density (specular microscopy), and keratometry before surgery and at 4, 8 and 12 months of follow-up. Laser settings and intraoperative complications were recorded. Nineteen patients underwent surgery by femtosecond-assisted DALK, 6 women and 12 men with average age 30.2±10.8 years at transplantation. They were paired with a group of 17 patients who underwent manual DALK in order to compare results. Before surgery, mean visual acuity in the femtosecond group was 0.90 logMAR versus 0.89 logMAR in the manual group, showing no statistically significant difference (P=0.96). Both groups were similar in terms of preoperative age, mean keratometry, pachymetry and endothelial cell density. Average visual acuity post-surgery was 0.27; 0.26; and 0.14 logMAR for femtosecond DALK versus 0.27; 0.17 et 0.25 for manual DALK at 4, 8 and 12 months follow-up respectively, showing no statistically significant difference. After surgery, at 4, 8 and 12 months, mean pachymetry was similar in both groups, and average endothelial cell density was 2390 cells/mm 2 for femto DALK versus 2531 cells/mm 2 for manual DALK at 12 months of follow-up, showing no statistically significant difference (P=0.5726). The rate of Descemet's membrane microperforations during the procedure was low and similar for both groups. Our study allows for a 12-month follow-up, with assessment of visual recovery, anatomic result and endothelial safety in a series of 19 femtosecond laser-assisted DALK with no statistical significant difference versus the manual trephination group. Femtosecond laser allows for increased reproducibility of the DALK procedure without reducing adverse effects during surgery. Femtosecond laser seems to improve the technique of the DALK procedure, and future developments could improve the reproducibility of DALK even further. A medical economics study would be necessary to determine the cost-effectiveness of femtosecond laser-assisted DALK., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Background: Understanding the pathophysiologic mechanism of intracranial aneurysm (IA) formation is a prerequisite to assess the potential risk of rupture. Nowadays, there are neither reliable biomarkers nor diagnostic tools to predict the formation or the evolution of IA. Increasing evidence suggests a genetic component of IA but genetics studies have failed to identify genetic variation causally related to IA., Objective: To develop diagnostic and predictive tools for the risk of IA formation and rupture., Methods: The French ICAN project is a noninterventional nationwide and multicentric research program. Each typical IA of bifurcation will be included. For familial forms, further IA screening will be applied among first-degree relatives. By accurate phenotype description with high-throughput genetic screening, we aim to identify new genes involved in IA. These potential genetic markers will be tested in large groups of patients. Any relevant pathway identified will be further explored in a large cohort of sporadic carriers of IA, which will be well documented with clinical, biological, and imaging data., Expected Outcomes: Discovering genetic risk factors, better understanding the pathophysiology, and identifying molecular mechanisms responsible for IA formation will be essential bases for the development of biomarkers and identification of therapeutic targets., Discussion: Our protocol has many assets. A nationwide recruitment allows for the inclusion of large pedigrees with familial forms of IA. It will combine accurate phenotyping and comprehensive imaging with high-throughput genetic screening. Last, it will enable exploiting metadata to explore new pathophysiological pathways of interest by crossing clinical, genetic, biological, and imaging information., (Copyright © 2016 by the Congress of Neurological Surgeons)

Objective: To estimate long term future of pressure and vision of children who underwent DS for CPG., Material and Methods: Children affected by CPG, aged from 1 day to 3 years at the time of the diagnosis were retrospectively included between 1999 and 2014. All benefited from a DS with use of antimetabolites. Preoperative and long-term post-operative intraocular pressures (IOP), per and postoperative complications as well as visual acuity and refractive status were estimated. Complete IOP control was defined by a postoperative IOP ≤16 mmHg without medical treatment., Results: 141 eyes of 71 children were included (sex ratio: 1.02). Mean follow-up was 56.6 ±9.6 months. Final average IOP among the whole sample was 11.3 +/-7 mmHg. The rate of total success was 69.4 %, partial success 27.6 % and 3 % failure at the last check. No complications were seen in 85.1 % of cases (n=120 eyes). Among 37 children analyzed for visual acuity, maintained visual acuity was found in 50 % of cases (n= 37 eyes)., Conclusion: DS allows obtaining good IOP control with a very low rate of complications, and should be considered as a technique of choice in the surgical management of CPG. This study is of particular relevance because it has been led on a long term period and on an important staff considering the rare prevalence of the condition., (Copyright © 2017 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

The area of uveitis is related to numerous pathological entities. One of the main causes of decreased visual acuity in these patients is macular edema. One aspect of the treatment includes cortosteroids used peri- and intra-ocularly., Materials and Methods: The goal of our work was to estimate the criteria of efficacy (on improvement in visual acuity and macular edema, as well as time to recurrence) and safety (on intraocular pressure and cataract) of these various routes of administration of corticosteroid after a single injection. We compared patients treated with Ozurdex ® versus subconjonctival triamcinolone versus sub-tenon's triamcinolone. This is a retrospective study conducted in 2 tertiary centers, the university medical center of Nantes and La Pitié-Salpêtrière hospital from November, 2011 to November, 2013., Results: At presentation, 25 % of the patients displayed VA better than 5/10. During follow-up, this proportion increased to 45 % at M1, 50 % at M3, 49 % at M6 and 48 % at the end of follow-up. There was no significant difference between the groups with respect to VA gain. The reductions in mean CMT compared with D0 were all statistically significant (improvement of one line in log-OCT). We observed an improvement in macular thickness of 88 % at M1, 86 % at M3, 61 % at M6 and 60 % at the end of follow-up, significant at each time, with no significant difference between the three groups. A comparison of time to anatomic vs. functional recurrence was performed, showing no difference. The largest increase in IOP was observed at M1, statistically different from the other time points., Discussion: Intra- and periocular injections should be considered as an adjuvant therapy, since the majority of the conditions in question require systemic treatment. They allow for increased intravitreal concentrations with fewer systemic effects., Conclusion: We demonstrated neither any true superiority of any of the 3 treatments nor any difference in tolerability between the 3 groups., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Introduction: Syphilis may manifest as several distinct ophthalmological forms. The main objective of our study was to describe the diagnostic, therapeutic data and prognosis of ocular syphilis., Patients and Methods: We retrospectively analysed the cases of syphilitic uveitis diagnosed in the University Hospital of Nantes between 2000 and 2013., Results: Twenty-seven patients were included in our study. Their mean age was 53.1 years. Bilateral uveitis was the most frequent clinical presentation (67%). The average diagnostic delay after the first symptoms was 2.8 months. Three patients (11%) presented with an anterior uveitis and 24 (89%) with a posterior uveitis, panuveitis or papillitis. The most frequent clinical signs were papillar lesion (44%), multifocal choroiditis (33%) and chorio-retinitis (37%). Ninety-three percent of the patients were treated with benzathine penicillin intravenously, one patient was treated by intramuscular penicillin injections and another by ceftriaxone intramuscularly. Ocular manifestations alone were sufficient to make the diagnosis of syphilis in 25 patients (93%) and of HIV seroconversion in 3 patients (11%). Forty-one percent of the patients benefited from an adjuvant therapy with corticosteroids. The treatment allowed a visual improvement of -0.48 log MAR (P<0.0001) with an average reduction of retinal central thickness of 70.2 μm (P=0.33)., Conclusion: We did not find a significant difference neither in management nor in results between the HIV seropositive and HIV seronegative groups. Others studies remains controversial about this aspect. The increased frequency of ocular manifestation as well as the increase of syphilis makes systematic screening in uveitis of a great value., (Copyright © 2015 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Introduction: Intraocular pressure and visual outcomes in primary pediatric glaucoma treated with trabeculotomy., Materials Et Methods: All children undergoing trabeculotomy as first-line surgical treatment between January 2002 and January 2012 were included in a retrospective monocentric study. We report initial clinical features, surgical complications, ophthalmological outcome and clinical progression., Results: Twenty-nine eyes of 16 children met the inclusion criteria and were treated with trabeculotomy as first-line treatment (mean age: 6 months). Median follow-up was 7 years. No major perioperative complications occurred, but minor complications were observed in 11 eyes. Mean pre-operative and initial post-operative intraocular pressures were 25.4 and 9.1mmHg respectively, thus a decrease of 16.3 points (63%). Overall surgical success rate was 89% at first follow-up, 72% at one year and 64% on last follow-up. A mean intraocular pressure of 15mmHg on no medications was achieved for two-thirds of eyes. After 7 years of follow-up, over 50% of eyes did not require a 2nd surgery. Visual acuity was at least 20/30 for 62% of eyes (83% for eyes requiring only trabeculotomy)., Discussion and Conclusion: Trabeculotomy as first-line treatment of primary juvenile glaucoma is a safe and effective surgery in about two-thirds of cases. It allows pressure control and satisfactory visual outcome in the majority of children., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Triamcinolone acetonide (Kenacort) is a corticosteroid that can be administrated by subconjunctival injection, with an extended release for up to three months. Our retrospective study aims to analyze safety and efficacy of subconjunctival triamcinolone injections in the treatment of uveitic macular edema. We included 31 eyes of 30 patients, who had one or several injections. We studied the progression of visual acuity, central macular thickness by optical coherence tomography (OCT), intraocular pressure, and presence or absence of cataract, on the day of injection (T0), and at 1, 3, 6 and 12 months after injection. Twenty-one patients had only one injection; 10 patients had 2. The 12-month follow-up showed an improvement in visual acuity with an initial mean of 0.36 ± 0.27 logMAR to 0.23 ± 0.33 logMAR at 3 months of follow-up (P<0.0004), and to 0.24 ± 0.21 logMAR at 12 months (P=0.0371), for a two-line improvement. A decrease in mean central macular thickness was measured by OCT, from a mean of 444 ± 112 μm (0.24 ± 0.11 logSD-OCT) at T0 to 355 ± 103 μm (0.14 $ ± 0.10 logSD-OCT) at 3 months (P=0.0002). We did not find a significant increase in intraocular pressure, and we diagnosed one cataract during follow-up but this occurred in the uninjected eye as well. Subconjunctival injection of triamcinolone acetonide is a safe and effective treatment of macular edema related to uveitis. Initial clinical monitoring is necessary to detect iatrogenic events., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Introduction: Observational study of the diagnoses and the treatment modalities used for orbital inflammatory pathologies discussed in multidisciplinary meetings (MDM) in Nantes University Medical Center over 4 years., Material and Method: A multidisciplinary meeting to discuss eyelid and orbital pathologies (excluding cancer) has been held in Nantes three times per year since October 2008. This retrospective study focuses on the cases of orbital inflammatory pathologies discussed at this meeting from October 2008 to October 2012 (49 cases included)., Results: Twenty-eight (57%) patients were diagnosed with orbital inflammation, 16 (33%) cases with isolated myositis and 5 (10%) with dacryoadenitis. In the diagnostic work-up, orbital biopsy was performed in 64% of orbital inflammation cases, 38% of myositis cases and 80% of dacryoadenitis cases. These specimens led to the diagnosis of 9 lymphomas, 2 histiocytoses and 1 metastasis. The internal medicine work-up allowed for the diagnosis of 3 granulomatoses with polyangiitis, one patient with sarcoidosis, and one patient with tuberculosis. Despite work-up, there remained 14 (29%) idiopathic orbital inflammatory syndromes (IOIS)., Discussion: The MDM of orbital pathology allows for the collective approach to the most difficult cases of management and is not concerned with stable orbitopathies or those presenting no diagnostic dilemma. Despite a multidisciplinary, rational etiologic work-up (frequently involving a biopsy), we find a higher rate of IOIS than reported in the literature., Conclusion: A multidisciplinary approach appears to be useful in improving management of orbital disease., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: Autosomal dominant cerebellar ataxia is a rare heterogeneous group of diseases characterized by cerebellar symptoms, often associated with other multisystemic signs. Mild optic neuropathy has been associated with spinocerebellar ataxia type 1 (SCA1), but macular dysfunction has been reported in only 2 cases. We report the first family with SCA1 with several members affected by visual loss related to maculopathy., Methods: Cross-sectional clinical and electrophysiological study of a family with genetically confirmed SCA1. Patients with unexplained visual loss were included., Results: Four patients from the same family, carrying the same genetic mutation, were examined. Testing revealed an increased CAG trinucleotide repeat number within the SCA1 gene. Genetic testing results for SCA7 were negative. Visual acuities ranged between 20/20 and 20/200. Visual fields revealed central scotomas in most of the eyes, and funduscopy was unremarkable in most patients. Central retinal thinning of the retina or disorganized photoreceptor layers were found with optical coherence tomography (OCT). In one patient, multifocal electroretinography (mfERG) revealed central retinal dysfunction., Conclusions: A clinically subtle or even occult maculopathy can occur in association with SCA1. Macular OCT and mfERG can be abnormal even in asymptomatic patients. Unexplained visual loss in SCA1 patients should prompt evaluation of macular function, even if clinical signs of maculopathy are absent.

The time course of neural activity in human brain regions involved in mediating pursuit eye movements is unclear. To address this question, we recorded intracerebral electroencephalography activity in eight epileptic patients while they performed a pursuit task that dissociates reactive, predictive and inhibited pursuits. A sustained gamma band (50-150 Hz) activity corresponding to pursuit maintenance was observed in the pursuit (and not saccade) area of the frontal eye field (FEF), in the ventral intraparietal sulcus (VIPS) and in occipital areas. The latency of gamma increase was found to precede target onset in FEF and VIPS, suggesting that those areas could also be involved during pursuit preparation/initiation. During pursuit inhibition, a sustained gamma band response was observed within prefrontal areas (pre-supplementary-motor-area, dorso-lateral prefrontal and frontopolar cortex). This study describes for the first time the dynamics of the neural activity in four areas of the pursuit system, not previously available in humans. These findings provide novel timing constraints to current models of the human pursuit system and establish the relevance of direct recordings to precisely relate eye movement behavior with neural activity in humans., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Treatment strategies of congenital cataract provide evidence of recent improvement in the visual prognosis. This trend is likely to be accounted for by a combination of improved management of amblyopia occurring in parallel with advances in surgical techniques, instrumentation, and materials. Compliance with occlusion is the factor most strongly associated with visual outcome: to support, to encourage, to comfort, to explain, to require (demand) to put in front of the responsibilities, here are the other missions of the therapist which are not less important than the surgical gesture. Progress, in this "educational" care of the visual function remains to make but the way is drawn., (Copyright © 2011. Published by Elsevier Masson SAS.)

During smooth pursuit, the image of the target is stabilized on the fovea, implying that speed judgments made during pursuit must rely on an extraretinal signal providing precise eye speed information. To characterize the introduction of such extraretinal signal into the human visual system, we performed a factorial, functional magnetic resonance imaging experiment, in which we manipulated the factor eye movement, with "fixation" and "pursuit" as levels, and the factor task, with "speed" and "form" judgments as levels. We hypothesized that the extraretinal speed signal is reflected as an interaction between speed judgments and pursuit. Random effects analysis yielded an interaction only in dorsal early visual cortex. Retinotopic mapping localized this interaction on the horizontal meridian (HM) between dorsal areas visual 2 and 3 (V2/V3) at 1-2 degrees azimuth. This corresponded to the position the pursuit target would have reached, if moving retinotopically, at the time of the subject's speed judgment. Because the 2 V2/V3 HMs are redundant, both may be involved in speed judgments, the ventral one involving judgments based on retinal motion and the dorsal one judgments requiring an internal signal. These results indicate that an extraretinal speed signal is injected into early visual cortex during pursuit.

Introduction: Congenital nystagmus (CN) that is present by the age of 3 months is the most common form of nystagmus in childhood. We present a prospective study (2001-2008) in which we report imaging findings in 48 children with CN., Materials and Methods: Twenty-six boys and 22 girls with CN underwent a complete ophthalmologic assessment and a cerebral MRI (mean age of examination under general anesthesia: 11 months). Three CN groups were formed: neurologic (n=27), sensory visual disturbance (n=14), and isolated (n=7). Cerebral MRI was interpreted by the same pediatric neuroradiologist (NG). Of the children studied, 98 % were born at term. The MRI abnormalities were classified as morphologic abnormalities (malformative or nonmalformative) and as signal abnormalities. The location of brain abnormalities was within the posterior fossa, (brain stem, cerebellum, dental nuclei, cisterna magna) and the cerebral hemisphere (white matter, perivascular spaces, midline commissures, basal ganglia)., Results: Pendular nystagmus was prevalent in sensory and neurologic nystagmus. On fundus examination, optic disc abnormalities were present in 70 % (19) of neurologic CN and associated with white matter abnormalities of the optic radiations in 40 % of cases. On MRI, malformative morphologic abnormalities were present in 27 cases, nonmalformative abnormalities were found in 67, and signal abnormalities in 68. Within the brain stem, signal abnormalities were found as a cockade appearance of the posterior pons in the reticular regions (neurologic n=14, sensory n=6, isolated n=3). Other bright (most frequent) signal abnormalities were found within the dentate nuclei of the posterior fossa (neurologic n=10, sensory n=3, isolated n=3) and the cerebral white matter (neurologic n=17, sensory n=7, isolated n=5) of which 24 (neurologic n=15, sensory n=5, isolated n=4) involved the optic radiations. Most of these abnormalities were related and were seen most frequently in neurologic nystagmus. The most frequent association was signal abnormalities of the white matter, ventricular dilatation, and dilatation of the perivascular spaces (60.4 %) (neurologic n=13, sensory n=6)., Conclusion: This study showed the fundamental contribution of the cerebral MRI in CN. Cerebral abnormalities were found at the pathways for ocular motility, particularly at the saccadic pathways.