Efficacy and comparison of corticosteroids only and corticosteroids with plasmapheresis or intravenous immunoglobulin for the treatment of optic neuritis in demyelinating disease: A systematic review and network meta-analysis.

PURPOSE: To compare the efficacy of treatment of optic neuritis (ON) with corticosteroids (CTC) alone, CTC+plasmapheresis (PLP), and CTC+intravenous immunoglobulin (IVIG). DESIGN: After an episode of ON, although visual recovery is usually good, some patients may have significant visual sequelae. While the efficacy of first-line CTC is now indisputable, there is no consensus on the nature of second-line treatment. To date, no systematic review has compared the efficacy of treatment of ON with CTC alone, CTC+plasmapheresis (PLP), and CTC+intravenous immunoglobulin (IVIG). A meta-analysis is needed to compare the efficacy of PLP and IVIG in steroid-resistant ON. METHODS: This systematic review included all studies comparing at least two of the three treatments for steroid-resistant ON (CTC alone, CTC+PLP, and CTC+IVIG). From all articles published on PubMed between January 2000 and June 2022, two independent ophthalmologists selected studies of interest using the PRISMA method. Methodology, patient characteristics, and outcomes were identified. A network metaanalysis was then performed to compare the efficacy of the three treatments. RESULTS: Six comparative studies were included, representing 209 patients. The percentage of significant visual recovery after CTC alone, CTC+PLP, and CTC+IVIG in the acute treatment of steroid-resistant ON was 30 %, 45 %, and 77 %, respectively. Comparison of CTC+IVIG vs CTC alone, CTC+PLP vs CTC only, and CTC+PLP vs CTC+IVIG yielded odds ratios of 12.81, 2.47, and 0.19 respectively. CONCLUSION: Treatment of steroid-resistant ON with CTC+PLP or CTC+IVIG is more effective than treatment with CTC alone. Although no study has directly compared the two treatments, IVIG may be more effective than PLP.

Unplanned 30-day readmission rate after ophthalmological surgery as a quality-of-care indicator.

BACKGROUND: The 30-day readmission rate provides a standardised quantitative evaluation of some postoperative complications. It is widely used worldwide in many medical and surgical specialities, and the World Health Organization recommends its use for monitoring healthcare system performance. In ophthalmology, its measurement is biased by the frequent and close planned surgery on one eye and then the other, particularly in the case of cataract surgery. This study measures the 30-day unplanned readmission rate in ophthalmology, globally and by surgery subtype, and describes the causes of readmission. METHODS: All patients readmitted within 30 days of ophthalmic surgery at Nantes University Hospital between January 2017 and December 2020 were identified in the Medical Information System. An ophthalmologist examined each medical record and collected the following data: the reason for readmission, comorbidities, the pathology treated, surgery type, surgery duration, the surgeon's experience, anaesthesia type, severity and readmission morbidity. RESULTS: For the 8522 ophthalmic surgeries performed in the four-year study period, 282 30-day unplanned readmissions were identified. The overall 30-day unplanned readmission rate was 2.07% for elective surgery, with a high variability depending on the surgery type: 0.95% for phacoemulsification, 4.95% for vitreoretinal surgery (3.42% for non-elective vitreoretinal surgery, 5.44% for retinal detachment surgery), 5.66% for deep lamellar keratoplasty and 11.90% for trabeculectomy. The unplanned 30-day readmission rate for ocular trauma surgery (emergency care) was 11.0%. Seven percent of all unplanned 30-day readmissions were not associated with an ophthalmological problem. CONCLUSIONS: This study is the first to report 30-day unplanned readmission in ophthalmology, globally and by surgical subtype, for elective and urgent procedures. This indicator can be used longitudinally to detect an increase in risk or transversely to compare the quality of care between different public or private hospitals.

Educational Concerns About the Safety of Cataract Surgery During Residency: The E3CAPS Pedagogic Study.

INTRODUCTION: Cataract surgery is the most common surgical procedure performed in France. While the incidence of intraoperative complications affecting visual prognosis is extremely low, given the large number of patients operated on, the absolute number of patients affected by complications is quite high. Complication rates are significantly higher when ophthalmology residents (ORs) perform the surgery. Although lack of experience remains the main risk factor, sleep deprivation may adversely affect ORs' successful surgery rate. The value of the EyeSi® surgical simulator in initial training has been demonstrated to increase cataract surgery safety through the transfer of surgical skills from the simulator to the operating room. However, there is no consensus regarding how much training is needed before the first-time ORs are allowed to operate. There is also no scientific evidence that sleep deprivation is associated with a decrease in surgical performance. Establishing a validated protocol for cataract surgery training using the EyeSi surgical simulator (referred to further as the EyeSi) and identifying risk factors for intraoperative complications related to sleep deprivation will improve cataract surgery safety and lead to the reorganization of our healthcare systems. METHODS AND PLANNED OUTCOMES: This multi-centre educational cohort study will include two distinct axes which will both aim to reduce the risks of cataract surgery. Enrollment will include 16 first-year ORs for Axis 1 and 25 experienced residents for Axis 2, all from the University Hospitals of Nantes, Tours, Angers and Rennes. Axis 1 will focus on investigating the learning curve of first-year ORs using the EyeSi, following the training program recommended by the "College des Ophtalmologistes Universitaires de France" in order to set up a future "licence to operate." Axis 2 will evaluate the impact of sleep deprivation on the surgical performance of experienced ORs using the EyeSi. TRIAL REGISTRATION: ClinicalTrial.gov identifier: NCT05722080.

Assessing retinal hemorrhages with non-invasive post-mortem fundus photographs in sudden unexpected death in infancy.

INTRODUCTION: In the case of sudden unexpected death in infancy (SUDI), eye examination is systematic to detect retinal hemorrhages (RH) that are a crucial hallmark for abusive head trauma (AHT). The aim of this study is to assess the ability of non-invasive post-mortem fundus photographs (PMFP) to detect RH in case of SUDI. METHODS: Bicentric retrospective analysis of consecutive cases of SUDI under 2 years of age were managed by two French SUDI referral centers with PMFP by RetCam (Clarity Medical Systems USA). PMFP were reviewed randomly, twice, by three independent ophthalmologists blinded for clinical data. RESULTS: Thirty cases (60 eyes) were included. Median age was 3.5 months (interquartile [1.6; 6.0]). No child died of AHT. Image quality was sufficient to assert presence or absence of RH in 50 eyes (83%). Sufficient quality rate was significantly higher when the post-mortem interval was inferior to 18 h (91%, 42/46) as opposed to over 18 h (57%, 8/14, p=0.0096). RH were found in six eyes (10%), four children (13%), with excellent inter and intra-raters' concordance (Cohen's Kappa from 0.81 [0.56-1.00] to 1.00 [1.00-1.00]). CONCLUSION: PMFP can detect RH in case of SUDI and is a relevant systematic screening test to be carried out as soon as the deceased child arrives in the hospital. It can decrease the need of eye removal for pathological examination, but further studies are needed to define the best decision algorithm.

Management of orbital floor fractures in France: Results of a national online survey.

INTRODUCTION: Orbital floor fractures (OFF) are common facial trauma injuries, and there are no official guidelines for their medical and surgical management. The aim of this study was to provide an overview of the management of OFF in France. MATERIALS AND METHODS: An online questionnaire was sent to 144 surgeons at the 88 French centers involved in the management of OFF (2019 data from the National Health Insurance Body). The questions related to the preoperative clinical and radiographic examinations, the criteria for surgical indication, the materials used, and the elements of the postoperative period. RESULTS: Ultimately, 42 questionnaires were analyzed (32 from oral and maxillofacial surgeons (OMFS), 8 from ophthalmologists, and 2 from ENT or plastic surgeons). For 69% of the surgeons, a systematic ophthalmological examination was carried out, 3-7 days after the trauma, and based on a Lancaster test or visual acuity (97.6% and 83.3% of the responders, respectively). The most important criteria for the therapeutic decision were diplopia or oculomotor disorder that persisted for more than 7 days (76.2%), clinical enophthalmos (54.8%), a large fracture (52.4%), and ptosis of the orbital content on CT scan (38.1%). The mean surgical delay was 7-15 days for 54.8% of the responders. Resorbing sheets were the preferred materials to repair small fractures, while larger fractures required alloplastic implants (titanium mesh). CONCLUSION: This survey confirms the diversity of practices in France regarding the management of OFF. Further studies are needed before guidelines can be developed.

What are the visuo-motor tendencies of omnidirectional scene free-viewing in virtual reality?

Central and peripheral vision during visual tasks have been extensively studied on two-dimensional screens, highlighting their perceptual and functional disparities. This study has two objectives: replicating on-screen gaze-contingent experiments removing central or peripheral field of view in virtual reality, and identifying visuo-motor biases specific to the exploration of 360 scenes with a wide field of view. Our results are useful for vision modelling, with applications in gaze position prediction (e.g., content compression and streaming). We ask how previous on-screen findings translate to conditions where observers can use their head to explore stimuli. We implemented a gaze-contingent paradigm to simulate loss of vision in virtual reality, participants could freely view omnidirectional natural scenes. This protocol allows the simulation of vision loss with an extended field of view (\(\gt \)80°) and studying the head's contributions to visual attention. The time-course of visuo-motor variables in our pure free-viewing task reveals long fixations and short saccades during first seconds of exploration, contrary to literature in visual tasks guided by instructions. We show that the effect of vision loss is reflected primarily on eye movements, in a manner consistent with two-dimensional screens literature. We hypothesize that head movements mainly serve to explore the scenes during free-viewing, the presence of masks did not significantly impact head scanning behaviours. We present new fixational and saccadic visuo-motor tendencies in a 360° context that we hope will help in the creation of gaze prediction models dedicated to virtual reality.

Optic neuritis classification in 2021.

Optic neuritis (ON) can be associated with inflammatory disease of the central nervous system or can be isolated, with or without relapse. It can also be associated with infectious or systemic disease. These multiple associations based on a variety of clinical, radiological, and biological criteria that have changed over time have led to overlapping phenotypes: a single ON case can be classified in several ways simultaneously or over time. As early, intensive treatment is often required, its diagnosis should be rapid and precise. In this review, we present the current state of knowledge about diagnostic criteria for ON aetiologies in adults and children, we discuss overlapping phenotypes, and we propose a homogeneous classification scheme. Even if distinctions between typical and atypical ON are relevant, their phenotypes are largely overlapping, and clinical criteria are neither sensitive enough, nor specific enough, to assure a diagnosis. For initial cases of ON, clinicians should perform contrast enhanced MRI of the brain and orbits, cerebral spinal fluid analysis, and biological analyses to exclude secondary infectious or inflammatory ON. Systematic screening for MOG-IgG and AQP4-IgG IgG is recommended in children but is still a matter of debate in adults. Early recognition of neuromyelitis optica spectrum disorder, MOG-IgG-associated disorder, and chronic relapsing idiopathic optic neuritis is required, as these diagnoses require therapies for relapse prevention that are different from those used to treat multiple sclerosis.

Inflammatory optic neuropathy in granulomatosis with polyangiitis can mimick isolated idiopathic optic neuritis.

OBJECTIVE: We describe a clinico-radiological presentation of inflammatory optic neuropathy that mimicked optic neuritis. METHODS: Retrospective single-center case series and literature review of optic neuropathy without orbital pseudotumor. RESULTS: Five local patients fulfilled the inclusion criteria. Clinical presentation revealed rapidly progressive severe unilateral visual loss, retrobulbar pain (n = 4), and paralytic strabismus (simultaneous = 2, protracted = 2) without proptosis. Optic nerve abnormality was not appreciated on initial scan review. Patients did not have any general activity of the granulomatosis with polyangiitis. Upon follow-up magnetic resonance imaging and initial imaging review, all patients revealed orbital apex anomalies. Visual acuity improved in three patients who received high-dose intravenous glucocorticosteroids immediately. Relapse was frequent and visual outcome was poor (final vision > 20/40 in two patients only). Literature review identified 16 well-documented cases of granulomatosis with polyangiitis-related isolated optic neuropathies. Magnetic resonance imaging revealed no abnormality (n = 6), optic nerve and/or sheath involvement (n = 9), apex infiltration (n = 3), and/or pachymeningitis (n = 7). CONCLUSION: Granulomatosis with polyangiitis is a rare yet potentially blinding cause of inflammatory optic neuropathy. Optic neuropathy in granulomatosis with polyangiitis may occur in the absence of systemic symptoms of disease activity and is challenging to distinguish from other inflammatory and non-inflammatory disorders affecting visual acuity. Several clinical and imaging clues suggest that optic neuropathy results from the development of an extravascular granulomatous process within the optic nerve sheath in the orbital apex, a place that is difficult to image. In a granulomatosis with polyangiitis patient with unexplained visual loss and a seemingly normal workup (fundoscopy, biology, and imaging), clinician should keep a high index of suspicion.

Clinical evaluation of the efficacy of materials used for primary reconstruction of orbital floor defects: Meta-analysis.

BACKGROUND: The aim of this network meta-analysis (NMA) was to compare the clinical results obtained after primary reconstruction of orbital floor fractures (OFF) using different materials. METHODS: PubMed, Cochrane, and Google Scholar databases were screened from 1989 to 2019. For a study to be eligible, it had to evaluate two or more materials and report the following clinical parameters: diplopia and/or enophthalmos and/or other complications. RESULTS: Nine studies involving 946 patients presenting with an OFF were included. After the surgical procedure, 105 patients (11%) had diplopia, while 43 patients (4.5%) suffered from enophthalmos. The NMA revealed that less postoperative diplopia and enophthalmos were obtained either by using polydioxanone (PDS), or a polymer of l-lactic acid and dl-lactic acid (P[L/DL]LA), or porous polyethylene, or titanium mesh compared with the use of autologous bone grafts. CONCLUSION: P(L/DL)LA and PDS seem to be the best options for small and intermediate defects, whereas the association of porous polyethylene and titanium should be preferred for larger defects.

Predictive values of initial semi-quantitative assessment of relative afferent pupillary defect for neovascularization in central retinal vein occlusion.

PURPOSE: To measure the predictive values of relative afferent pupillary defect (RAPD) assessed semi-quantitatively, and visual acuity (VA) at onset of central retinal vein occlusion (CRVO), for neovascularization. METHODS: Retrospective analysis of the TROXHEMO trial that included patients with CRVO within 30 days after the onset. Inclusion criteria were as follows: semi-quantitative RAPD assessment at diagnosis and/or at one month. RAPD was 'severe' if ≥ 0.9 log. Exclusion criteria were as follows: prophylactic panretinal photocoagulation (PRP) before neovascularization. RESULTS: Among the 119 patients enrolled in the main centre, 101 were analysed. 26 had a neovascular complication during the twelve months of follow-up: rubeosis (19), glaucoma (7) and posterior neovascularization (15). The mean time to onset of a neovascular complication was 4.7 months (1 to 12, median 3 months). All the patients who had a neovascular complication had RAPD at first examination or at one month (negative predictive value (NPV) = 100%) but the positive predictive value (PPV) was low (31%, 95% CI [21%; 42%]). The association 'severe RAPD or VA < 35 letters (ETDRS) at inclusion or at one month' was the best compromise between PPV (53%, [39%; 68%]) and NPV (96%, [92%; 100%]). CONCLUSION: To predict neovascularization, RAPD should be routinely evaluated with filters: the risk of neovascular complication is (a) almost nil if there is no RAPD, (b) very low if there is no severe RAPD and if VA is higher than 35 letters, and (c) higher than 50% if RAPD is ≥ 0.9 log or if VA is less than 35 letters.

Efficacy of rituximab in patients with Graves' orbitopathy: a retrospective multicenter nationwide study.

PURPOSE: The clinical utility of rituximab (RTX) in Graves' orbitopathy (GO) treatment remains controversial since the discrepant results from 2 prospective randomized studies (Stan M et al. J Clin Endocrinol Metab 2015; Salvi M et al. J Clin Endocrinol Metab 2015). The aim of this study was to assess in real life the characteristics and the clinical outcomes of patients with GO treated with RTX in cases of corticosteroid resistance or corticosteroid dependence. METHODS: Multicenter French retrospective study including patients with moderate-to-severe GO requiring second-line treatment with RTX. Patients were classified according to three main baseline characteristics: clinical inflammation (CAS ≥ 3), oculomotor limitation, and visual dysfunction. Patients were considered as responders if, at 24 weeks (week 24), at least 1 of these 3 parameters improved with no worsening elsewhere. RESULTS: Forty patients were included (65% smokers, 38% dysthyroidism). Thirty-two patients were treated with RTX alone (one patient excluded owing to side effects): 64.5% had favorable responses at week 24 and significant reduction in baseline CAS (3.29 ± 1.6) at 12 weeks (1.93 ± 1.1; P < 0.001) and at week 24 (1.59 ± 1.1; P < 0.001); reduction in anti-TSH receptor antibodies at week 24 (P < 0.01); and significant improvement of visual acuity (P = 0.04) and ocular hypertonia (P = 0.04) at week 12, but no improvement in oculomotor dysfunction. Eight patients needed emergency treatment with concomitant RTX and orbital decompression, with favorable outcome for 5 patients. Predictive factors for a poor response to RTX were low baseline CAS, smoker, and baseline ocular hypertonia. All patients reported good tolerance except one serious side effect (a cytokine release syndrome). CONCLUSIONS: The efficiency results of RTX in reducing CAS in this cohort are just between those of Stan and Salvi. This could be explained by our delay before treatment initiation, quicker than Stan but longer than Salvi. RTX appears to be effective as a second-line treatment for the inflammatory component of GO, especially if the disease is highly active and recent.

MOG-Ab prevalence in optic neuritis and clinical predictive factors for diagnosis.

OBJECTIVE: What is the proportion of antibodies to myelin oligodendrocyte glycoprotein (MOG-Ab) in optic neuritis (ON) in adults and what would be the ON presentation for which MOG-Ab should be tested? METHODS: Multicentric prospective study conducted during 1 year on all patients diagnosed with acute ON in all ophthalmological units in hospitals in a region in western France. RESULTS: Sixty-five patients were included. MOG-Ab prevalence was 14% (9/65) during an acute ON and 13% (7/55) after exclusion of patients already diagnosed with multiple sclerosis (MS) (8) or MOG+ON (2). Compared with MS and clinically isolated syndrome, MOG+ON had no female preponderance (67% of men in case of MOG+ON and 22% of men in case of MS and clinically isolated syndrome, p<0.05) were more often bilateral (44% vs 3%, p<0.005) and associated with optic disc swelling (ODS) (78% vs 14%, p<0.001). To predict MOG+ON, the positive predictive values (PPVs) of male sex, ODS and bilateral involvement were 29% (95% CI 9% to 48%), 41% (95% CI 18% to 65%) and 40% (95% CI 10% to 70%), respectively, while the negative predictive values (NPV) were 93% (95% CI 86% to 100%), 96% (95% CI 90% to 100%) and 91% (95% CI 83% to 99%), respectively. The combined factor 'ODS or bilateral or recurrent ON' was the best compromise between PPV (31% (95% CI 14% to 48%)) and NPV (100% (95% CI 100% to 100%)). CONCLUSION: Among ON episodes, MOG-Ab were found in 14% of cases. MOG+ON occurred without female preponderance and was significantly associated with ODS and/or bilateral ON. Testing MOG-Ab only in patients presenting with ODS or bilateral or recurrent ON would limit MOG-Ab tests to fewer than half of all patients without the risk of missing any MOG+ON cases.

Efficacy and Safety of Interferon Alpha 2A and Pegylated Interferon Alpha 2A in Inflammatory Macular Edema.

Purpose: The aim of this study was to further explore the efficacy and safety of interferon-α in refractory non-infectious inflammatory macular edema and to compare interferon-α2a and pegylated interferon-α2b.Methods: 34 patients with refractory non-infectious uveitic macular edemaunder interferon-α were retrospectively reviewed.Results: Mean baseline best-corrected visual acuityimproved from 0.55 logMar to 0.37 logMAR (P < 0.001) at month (M) 1 and 0.40 logMAR (P < 0.001) at M6. The mean baseline CMT decreased from 554 µm to 367 µm (P < 0.001) at M1 and 394 µm (P < 0.001) at M6. Clinical adverse effects (AEs) were observed in a third of patients, leading to treatment discontinuation because of frequent mild AEs and few severe AE. No statistically significant difference was found between both molecules.Conclusions: Anatomically and functionally, interferon-α was rapidly effective despite a low dosage regimen and no difference in efficacy and tolerance was observed between interferon-α2a and pegylated interferon-α2b.

Predicting artificial visual field losses: A gaze-based inference study.

Visual field defects are a world-wide concern, and the proportion of the population experiencing vision loss is ever increasing. Macular degeneration and glaucoma are among the four leading causes of permanent vision loss. Identifying and characterizing visual field losses from gaze alone could prove crucial in the future for screening tests, rehabilitation therapies, and monitoring. In this experiment, 54 participants took part in a free-viewing task of visual scenes while experiencing artificial scotomas (central and peripheral) of varying radii in a gaze-contingent paradigm. We studied the importance of a set of gaze features as predictors to best differentiate between artificial scotoma conditions. Linear mixed models were utilized to measure differences between scotoma conditions. Correlation and factorial analyses revealed redundancies in our data. Finally, hidden Markov models and recurrent neural networks were implemented as classifiers in order to measure the predictive usefulness of gaze features. The results show separate saccade direction biases depending on scotoma type. We demonstrate that the saccade relative angle, amplitude, and peak velocity of saccades are the best features on the basis of which to distinguish between artificial scotomas in a free-viewing task. Finally, we discuss the usefulness of our protocol and analyses as a gaze-feature identifier tool that discriminates between artificial scotomas of different types and sizes.

Prospective evaluation of anesthetic protocols during pediatric ophthalmic surgery.

PURPOSE: To date, no protocol of anesthesia for pediatric ophthalmic surgery is unanimously recognized. The primary anesthetic risks are associated with strabismus surgery, including oculocardiac reflex, postoperative nausea and vomiting, and postoperative pain. METHODS: This was a prospective, monocentric, observational study conducted in a tertiary pediatric ophthalmic unit. Our anesthetic protocol for strabismus surgery included postoperative nausea and vomiting prevention using dexamethasone and ondansetron. No drug-based prevention of oculocardiac reflex or local/locoregional anesthesia was employed. RESULTS: A total of 106 pediatric ophthalmic surgeries completed between November 2015 and May 2016 were analyzed. The mean patient age was 4.4 (range: 0.2-7.3, standard deviation: 2.4) years. Ambulatory rate was 90%. Oculocardiac reflex incidence was 65% during strabismus surgery (34/52), 50% during congenital cataract surgery (4/8), 33% during intramuscular injection of botulinum toxin (1/3), and 0% during other procedures. No asystole occurred. Postoperative nausea and vomiting incidence was 9.6% after strabismus surgery (5/52) and 0% following the other procedures. One child was hospitalized for one night because of persistent postoperative nausea and vomiting. Postoperative pain generally occurred early on in the recovery room and was quickly controlled. Its incidence was higher in patients who underwent strabismus surgery (27%) than in those who underwent other procedures (9%). CONCLUSION: Morbidity associated with ophthalmic pediatric surgery is low and predominantly associated with strabismus surgery. The benefit-risk ratio and cost-effectiveness of oculocardiac reflex prevention should be questioned. Our postoperative nausea and vomiting rate is low, thanks to the use of a well-managed multimodal strategy. Early postoperative pain is usually well-treated but could probably be more effectively prevented.

Clinical presentation of optic neuritis with autoantibodies anti-myelin oligodendrocyte glycoprotein.

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.

Clinical spectrum and prognostic value of CNS MOG autoimmunity in adults: The MOGADOR study.

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.