BACKGROUND:The potential link between myasthenia gravis (MG) and COVID-19 infection or vaccination remains unclear. This study aimed to evaluate the relationship between these factors and new-onset MG. METHODS:A case-control study was conducted using Clalit Health Services' database. We applied a ML algorithm to reduce diagnostic misclassification. The study examined adults with new onset MG, aged 18 or older, between January 2020 and December 2022 for COVID-19 infection (Cohort I) and between January 2021 and December 2022 for Pfizer-BioNTech COVID-19 vaccine (Cohort II). For each new MG case, three controls matched by age, sex, and index date were selected. Prior exposure to either infection or vaccination was assessed within 90 and 180 days for cases and controls. RESULTS:In cohort I, 253 new MG cases were identified. A multivariate logistic regression model showed an odds ratio (OR) of 1.44 (95% CI 0.708-2.92) within 90 days post-infection and 1.67 (95% CI 0.98-2.84) within 180 days. In cohort II, 177 new MG cases were detected, with an OR of 1.76 (95% CI 1.049-2.95) within 90 days post-vaccination and 2.45 (95% CI 1.51-3.95) within 180 days. CONCLUSION:This study suggests no significant increased risk of new-onset MG following COVID-19 infection, but the Pfizer-BioNTech vaccine appears to be associated with a higher risk of developing MG, particularly within 180 days of vaccination.
Assess the safety and efficacy data of GA Depot treatment (for up to 112 weeks) in eleven primary progressive MS (PPMS) subjects enrolled out of the 24 planned.
INTRODUCTION/AIMS:Myasthenia gravis (MG) is associated with thymic neoplasms. However, an increased prevalence of extrathymic neoplasms has also been reported. This study aimed to evaluate the rates of malignancy in MG patients while accounting for risk factors such as disease characteristics and immunomodulatory treatments. METHODS:We conducted a case-control study using the Clalit Health Services database, applying a machine learning (ML) algorithm to minimize diagnosis misclassification. We included MG patients aged 18 years and older, with a sex- and age-matched control group in a 1:3 ratio. We compared the prevalence and hazard ratios of extrathymic neoplasms between the groups. RESULTS:A total of 1558 patients with a high probability of MG, according to our ML model, were included in the cohort, alongside a control group of 4674 individuals. MG patients had a higher prevalence of malignancy prior to MG diagnosis, with an odds ratio of 1.95 (95% CI, 1.65-2.08), and a higher incidence of malignancy after MG diagnosis, with a hazard ratio of 1.56 (95% CI, 1.38-1.77). The most prevalent extrathymic neoplasms after MG diagnosis were respiratory, intrathoracic, skin (specifically non-melanoma), urinary tract, soft tissue, and myelodysplastic syndrome. Risk factors for malignancy included age, male sex, and thymoma. Immunosuppressive treatment did not increase the risk of malignancy. DISCUSSION:MG patients have a higher prevalence of both solid and hematologic neoplasms compared to non-myasthenic controls, regardless of immunosuppressive treatment. This supports the notion that malignancy is related to MG disease itself rather than external factors.
BACKGROUND:Comorbidities occur in aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), and double seronegative NMOSD (DN-NMOSD), potentially contributing to a less favorable disease course. OBJECTIVES:To characterize comorbidities in AQP4-NMOSD, MOGAD, and DN-NMOSD and assess their association with optic neuritis (ON) outcomes by optical coherence tomography (OCT) in AQP4-NMOSD. METHODS:Four hundred and forty-two participants from the CROCTINO cohort were evaluated for comorbidities. RESULTS:In AQP4-NMOSD patients (n = 360), 43.5% (n = 161) had comorbidities, equally divided between single and multiple. In MOGAD (n = 49), 40.8% had comorbidities, with 75% (n = 15) single and 25% (n = 5) multiple. In DN-NMOSD (n = 33), 36.4% (n = 12) had comorbidities equally split. AQP4-NMOSD patients had more multiple comorbidities (50%, n = 81/161) than MOGAD (25%, n = 5/20, p = 0.03) and more autoimmune disorders (AID) (40.4%, n = 65) than MOGAD (20%, n = 4, p = 0.09) and DN-NMOSD (none, p = 0.004). Cardiovascular comorbidities and related risk factors (CVC/RF) occurred in 34.8% (n = 56) of AQP4-NMOSD, 50% (n = 10) of MOGAD, and 33.3% (n = 4) of DN-NMOSD. Expanded Disability Status Scale was higher in MOGAD (3.0 vs. 2.0, p = 0.006) and DN-NMOSD (5.0 vs. 2.0, p = 0.008) with comorbidities. AQP4-NMOSD patients with CVC/RF had higher ON relapse rates than those with AID (1.06 ± 3.33 vs. 0.49 ± 0.98, p < 0.001). OCT revealed reduced inner nuclear layer thickness in AQP4-NMOSD with comorbidities compared to non-comorbidity (B = -1.52, p = 0.047), more pronounced with CVC/RF (B = -2.96, p = 0.009). CONCLUSION:Comorbidities are frequent in AQP4-NMOSD and MOGAD and are associated with ON frequency and disability. These findings highlight the need for proactive comorbidity management to improve patient care.
Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction, most commonly associated with autoantibodies against the acetylcholine receptor (AChR). While familial clustering of autoimmune MG (fMG) has been described, its prevalence and clinical characteristics remain uncertain. This study aimed to characterize autoimmune fMG cases in an Israeli cohort and describe global data through a systematic literature review. We retrospectively analyzed the medical records of MG patients treated at Rabin Medical Center from 2000 to 2022. The clinical and demographic features of autoimmune fMG cases—defined by compatible clinical, serological, and electrophysiological features—were compared to those of sporadic MG. Additionally, a systematic review of published autoimmune fMG cases was performed according to PRISMA guidelines. Among 281 MG patients, 16 patients (5.7
Background and ObjectivesSusac syndrome (SuS) is a rare disorder characterized by encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss, often accompanied by vertigo. Recent updates to diagnostic criteria and treatment guidelines have been made. This study examines clinical manifestations; disease activity; and risk factors of disability, dependency, and return to work in patients with SuS.MethodsA retrospective multicenter study was conducted on 20 consecutive patients with SuS with at least 2 years of follow-up. Clinical and paraclinical activities were assessed and rated according to the severity at onset and the end of follow-up. Cognitive function was assessed using the Montreal Cognitive Assessment while disability and dependence in daily activities were measured using the modified Rankin Scale. Employment status was graded.ResultsThe mean age at onset was 38.9 years, with a mean follow-up of 55.9 months. The female-to-male ratio was 1.86, and 45% of patients had the complete clinical triad. Severe cerebral involvement at onset was associated with a higher risk of cerebral exacerbations within the first year and with an increased long-term disability and dependency. Cognitive function improved in 75% of patients during follow-up. At disease onset, hearing loss excluding low frequencies occurred in 46.7%. Relapse of hearing loss was associated with greater impairment in daily activities. Male sex and elevated CSF protein levels were linked to poorer prognosis. Cerebral and inner ear exacerbations were most common in the first year while retinal exacerbations occurred more frequently, mainly within the first 2 years. Approximately 50% of patients resumed employment while 25% did not return to work.DiscussionCurrent treatment strategies for SuS do not fully prevent relapses. Severe brain manifestation at onset, male sex, and high CSF protein levels are risk factors of a worse prognosis of disability and dependence, indicating the need for intensive treatment. High-frequency hearing loss does not exclude SuS diagnosis.
Background and objectives Myasthenia gravis (MG) is a chronic antibody-mediated autoimmune neuromuscular disorder causing fluctuating muscle weakness due to autoantibodies that target the acetylcholine receptor (AChR) in the neuromuscular junction (NMJ). Despite extensive research, MG remains unpredictable and heterogeneous, underscoring the need for better insight into disease pathogenesis and clinical course. The objective of this study was to identify clinical and immunologic disease-related parameters that correlate with disease severity. Methods This large-scale, multicenter, cross-sectional study included prospectively recruited patients with MG and MG serum samples procured from reputable biobanks. Inclusion criteria required an MG clinical presentation and a positive anti-AChR immunoglobulin G (IgG) titer (>= 0.5 nM), confirmed by 2 independent tests. Enrolled patients underwent profiling of subunit-specific anti-AChR IgG titers. Disease severity patterns and their relation to age, sex, disease onset, thymic involvement, anti-AChR titer, and anti-AChR subunit immunodominance were investigated. Results The study included 513 patients with MG (50.5% female) with a median age of 64 years (range 14-98.5). Extended clinical data were available for 232 patients. Anti-AChR titers increased monotonically with disease severity (rho = 0.31, 95% CI 0.17-0.44, p < 0.0001) and were higher in female patients (median 14.6 nM vs 7.7 nM, 95% CI for Delta 2.1-7.3 nM, p < 0.0001) in whom higher antibody titers decreased monotonically with age. Male patients' titers were predominantly alpha-immunodominant (male 23.2% vs female 16.6%, 95% CI for Delta 0%-7.6%, p = 0.031), while female patients' titers were gamma-immunodominant (female 30% vs male 24.5%, 95% CI for Delta 2%-11%, p = 0.0027). Gamma immunodominance correlated with higher anti-AChR titer (rho = 0.25, 95% CI 0.16-0.33, p < 0.0001) and severe (Myasthenia Gravis Foundation of America IV-V) disease (44% vs 27%, 95% CI for Delta 4%-34%, p = 0.0141). Unsupervised principal component analysis disclosed 2 distinct disease endotypes: female patients with moderate to severe early-onset MG, gamma subunit immunodominance, high anti-AChR titers, and frequent thymic hyperplasia (endotype A) and the remaining patients, mostly patients with late-onset MG, alpha subunit immunodominance, and low anti-AChR titers (endotype B). Discussion Two distinct MG endotypes emerge based on sex, age, thymic involvement, autoantibody titer, and immunoglobulin AChR subunit immunodominance-suggesting different underlying etiologies with potential implications for sex-dependent precision medicine, women's health, and the development of new therapeutic modalities for MG.
BACKGROUND:Observation off chronic steroids-sparing immunotherapy (watchful waiting) is often recommended after the onset attack of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) given the variable relapse risk, but the outcomes of this approach are unexplored. OBJECTIVES:To determine the risk of relapse and unfavorable disability outcomes in MOGAD patients who underwent watchful waiting approach after the onset attack. METHODS:In this international, multicenter, retrospective case series in four tertiary-care centers in Canada, Israel, Thailand, and the United States, patients who fulfilled the 2023 MOGAD diagnostic criteria, presented to the participating institutions with the first clinical attack (incident cases), and had at least 3 years of follow-up were identified (n = 81). Only incident cases were included to avoid referral bias. RESULTS:The median age was 31 (IQR 18-47) years, 57 % were female, and the median follow-up was 6.3 (IQR 4.5-10.1) years. After onset, 65 (80 %) underwent watchful waiting. Forty-one (63 %) had relapses with annualized relapse rate of 0.27 (IQR 0.15-0.44). Unfavorable disability outcomes (Expanded Disability Status Scale score ≥2.0) were observed in 12/64 (19 %) watchful-waiting patients (7 from the onset attack; 4 from relapses; 1 unclear). CONCLUSIONS:Given that one-third of watchful waiting patients remained monophasic and unfavorable disability outcomes from relapses were uncommon, a continued observation off chronic steroids-sparing immunotherapy could be an option for a subset of MOGAD patients.
Background Patients with neuromyelitis optica spectrum disorder (NMOSD) are often seropositive for antibodies against aquaporin-4 (AQP4). The importance of MRI monitoring in this disease requires evaluation. Purpose To profile MRI features from a large international cohort with AQP4 immunoglobulin G (IgG)-seropositive NMOSD (from the Parallel MRI in NMOSD [PAMRINO] study) and to evaluate and confirm existing knowledge regarding the incidence, location, and longitudinal development of characteristic lesions in the central nervous system associated with AQP4-IgG-seropositive NMOSD. Materials and Methods In this retrospective study (from August 2016 to January 2019), MRI and clinical data were collected from 17 NMOSD expert sites in 11 countries across four continents. Clinical features and lesions identified at cross-sectional and longitudinal MRI were assessed. No formal statistical tests were used to compare observations; however, means, SDs, and 95% CIs are reported when evaluating lesion frequencies. Results Available T1-weighted and T2-weighted MRI scans in patients with AQP4-IgG-seropositive NMOSD (n = 525) were read. Among the 525 patients, 320 underwent cerebral MRI examinations with T2-weighted hyperintense cerebral (264 of 320; 82.5%), cerebellar (44 of 320; 13.8%), and brainstem (158 of 321 [49.2%], including one lesion observed at cervical spinal cord [SC] MRI) lesions. Lesions in the optic nerves, analyzed from 152 MRI examinations, were mainly found in the central (81 of 92; 88%) and posterior (79 of 92; 86%) sections (bilaterally in 39 of 92; 42%). Longitudinally extensive transverse myelitis was the predominant SC lesion pattern (upper compartment from 322 MRI examinations, 133 of 210 [63.3%]; and lower compartment from 301 MRI examinations, 149 of 212 [70.3%]). However, nonlongitudinal extensive transverse myelitis lesions were also observed frequently (105 of 210; 50.0%) in the cervical SC. Clinical data (n = 349; mean age, 44 years ± 14 [SD]; 202 female patients) and acute lesions at contrast-enhanced (CE) MRI (n = 58, performed within 30 days of the last attack) were evaluated. CE lesions were detected in the cerebrum (eight of 13; 62%), optic nerves (14 of 19; 74%), or chiasm (three of four; 75%) within 15 days of any relapse. In the upper SC (29 of 44; 66%), CE lesions were frequently observed up to 20 days after a clinical myelitis event. Conclusion A high incidence of abnormal brain MRI examinations and nonlongitudinal extensive SC lesions was found in patients in PAMRINO with AQP4-IgG-seropositive NMOSD. © RSNA, 2024 Supplemental material is available for this article.
INTRODUCTION:Examining the safety of theBNT162b2 mRNA vaccine in multiple sclerosis (MS) patients remains inconclusive, particularly regarding the potential for disease exacerbations. This study aims to assess the effects of BNT162b2 COVID-19 vaccination on disease activity in MS patients through sequential MRI imaging. METHODS:A retrospective study of 84 MS patients from five Israeli hospitals was conducted. MS lesion load was determined from three brain MRI scans, one postvaccination and two prevaccination scans. A post hoc analysis compared subgroups featuring vaccinated and unvaccinated patients respectively, with early onset MS. RESULTS:The cohort included 70 women with early onset (mean age 16.4 ± 0.8 years) and adult onset (mean age 34.9 ± 1.1 years) MS. Among the early onset group, vaccinated patients showed an increased risk of new lesions (p = .00026), while there was no increased risk among adult-onset patients. Additionally, a comparison between early onset vaccinated and nonvaccinated groups revealed a higher risk of increased lesions in the vaccinated group (p = .024). DISCUSSION:Overall, the study suggests that the BNT162b2 vaccine is generally safe in MS patients, with no association found between vaccination and new lesions in most patients. However, close MRI follow-up is recommended for early-onset MS cases to monitor lesion development.
BACKGROUND AND OBJECTIVES:Susac syndrome (SuS) is a rare disorder characterized by encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss, often accompanied by vertigo. Recent updates to diagnostic criteria and treatment guidelines have been made. This study examines clinical manifestations; disease activity; and risk factors of disability, dependency, and return to work in patients with SuS. METHODS:A retrospective multicenter study was conducted on 20 consecutive patients with SuS with at least 2 years of follow-up. Clinical and paraclinical activities were assessed and rated according to the severity at onset and the end of follow-up. Cognitive function was assessed using the Montreal Cognitive Assessment while disability and dependence in daily activities were measured using the modified Rankin Scale. Employment status was graded. RESULTS:The mean age at onset was 38.9 years, with a mean follow-up of 55.9 months. The female-to-male ratio was 1.86, and 45% of patients had the complete clinical triad. Severe cerebral involvement at onset was associated with a higher risk of cerebral exacerbations within the first year and with an increased long-term disability and dependency. Cognitive function improved in 75% of patients during follow-up. At disease onset, hearing loss excluding low frequencies occurred in 46.7%. Relapse of hearing loss was associated with greater impairment in daily activities. Male sex and elevated CSF protein levels were linked to poorer prognosis. Cerebral and inner ear exacerbations were most common in the first year while retinal exacerbations occurred more frequently, mainly within the first 2 years. Approximately 50% of patients resumed employment while 25% did not return to work. DISCUSSION:Current treatment strategies for SuS do not fully prevent relapses. Severe brain manifestation at onset, male sex, and high CSF protein levels are risk factors of a worse prognosis of disability and dependence, indicating the need for intensive treatment. High-frequency hearing loss does not exclude SuS diagnosis.
BackgroundDetection of oligoclonal bands (OCBs) in the cerebrospinal fluid (CSF) is important for diagnosis of multiple sclerosis (MS). Previous studies reported that treatment with intravenous methylprednisolone (IVMP) before lumber puncture (LP) could suppress OCBs production. The aim of this study was to assess whether IVMP initiation prior to CSF collection affects OCBs results in patients with an acute demyelinating event. Additionally, we examined which clinical characteristics are associated with the presence of OCBs in the CSF.MethodsWe retrospectively evaluated patients admitted to the neurology department at Rabin Medical Center (RMC) between 2010 and 2022 who underwent LP with OCBs analysis as part of their demyelinating attack workup. Patients were divided into OCB-positive and OCB-negative groups and demographical and clinical characteristics (including timing and duration of acute steroid treatment and history of prior demyelinating attacks) were analyzed for association with OCBs results.ResultsA total of 342 patients were included with a median age of 35 years (IQR, 27-46). Two hundred thirty-eight (69.6%) were OCB-positive. Initiation of IVMP before LP was not associated with negative OCBs (11.8% Vs. 13.5%, P=0.721), nor was it correlated with OCBs positivity (OR=0.86, P=0.66). CSF cell count was higher in OCB-positive patients (5 Vs. 3, P=0.001), and a history of prior demyelinating attacks was associated with- (33.6% Vs. 20.2%, P=0.014) and predictive of OCBs positivity (OR=2, P=0.013).ConclusionsTiming of steroids was not associated with OCB positivity. However, pleocytosis and a prior attack were associated with OCB positivity in this cohort. Our results suggest that steroid treatment is unlikely to affect OCBs results. Ideally, larger prospective studies would be needed to confirm our observations.
OBJECTIVE:The first international consensus criteria for optic neuritis (ICON) were published in 2022. We applied these criteria to a prospective, global observational study of acute optic neuritis (ON). METHODS:We included 160 patients with a first-ever acute ON suggestive of a demyelinating CNS disease from the Acute Optic Neuritis Network (ACON). We applied the 2022 ICON to all participants and subsequently adjusted the ICON by replacing a missing relative afferent pupillary defect (RAPD) or dyschromatopsia if magnetic resonance imaging pathology of the optical nerve plus optical coherence tomography abnormalities or certain biomarkers are present. RESULTS:According to the 2022 ICON, 80 (50%) patients were classified as definite ON, 12 (7%) patients were classified as possible ON, and 68 (43%) as not ON (NON). The main reasons for classification as NON were absent RAPD (52 patients, 76%) or dyschromatopsia (49 patients, 72%). Distribution of underlying ON etiologies was as follows: 78 (49%) patients had a single isolated ON, 41 (26%) patients were diagnosed with multiple sclerosis, 25 (16%) patients with myelin oligodendrocyte glycoprotein antibody-associated disease, and 15 (9%) with neuromyelitis optica spectrum disorder. The application of the adjusted ON criteria yielded a higher proportion of patients classified as ON (126 patients, 79%). INTERPRETATION:According to the 2022 ICON, almost half of the included patients in ACON did not fulfill the requirements for classification of definite or possible ON, particularly due to missing RAPD and dyschromatopsia. Thorough RAPD examination and formal color vision testing are critical to the application of the 2022 ICON.
BACKGROUND:Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare autoimmune demyelinating disorder of the central nervous system. Optic neuritis (ON) is the most common clinical manifestation of MOGAD in adults. In 2023, new MOGAD diagnostic criteria were proposed, highlighting the importance of supplemental criteria when MOG-immunoglobulin G (IgG) titers are unavailable. OBJECTIVES:To investigate the applicability of the 2023 MOGAD criteria in patients diagnosed with MOGAD and treated before the availability of MOG-IgG titers. METHODS:We conducted a retrospective chart review of patients classified as MOGAD between 2010 and 2023 at Rabin Medical Center. Patient demographics as well as clinical and imaging data were collected, including visual acuity, expanded disability status score, core demyelinating events, antibody status, and brain and optic nerve magnetic resonance imaging data. Patients fulfilling the 2023 MOGAD criteria were reported as definite MOGAD. RESULTS:Fifteen patients met the 2023 MOGAD diagnostic criteria despite lack of MOG-IgG titer. The most common supplemental criterion meeting the 2023 MOGAD criteria was optic disc edema (n=12, 80%), followed by longitudinal optic nerve involvement (53%), bilateral ON (40%), and perineural optic sheath enhancement (33%). CONCLUSIONS:All patients with a clinical diagnosis of MOG-ON in our cohort fulfilled the 2023 MOGAD criteria despite the lack of antibody titers. The 2023 MOGAD criteria can be reliably applied to Israeli cohorts, prior to availability of MOGAD IgG titers, with particular attention to additional supplemental criteria. Since the 2023 MOGAD criteria were published, MOGAD IgG titers have been added to routine testing at our facility.
Background: The potential therapeutic benefit of intravenous immunoglobulins (IVIGs) for acute attacks of myelin oligodendrocyte glycoprotein antibody disease (MOGAD) is unknown. Objective: The objective was to describe the outcomes of IVIG treatment for acute MOGAD attacks. Methods: A retrospective observational study involving seven tertiary neuroimmunology centers. Data collection included patients’ demographics, Expanded Disability Status Scale (EDSS), and visual acuity (VA) before the attack, at the nadir of the attack before IVIG treatment, and at follow-up visits ⩾3 months after treatment. Results: Thirty-nine patients were included, of which 21 (53.8%) were female. The median age was 23 years (range 5–74 years), and the median disease duration was 4 months (range 0–93 months). The most common type of attack treated with IVIG was isolated optic neuritis (ON) (unilateral n = 14, bilateral n = 5, associated with transverse myelitis (TM), n = 1), followed by acute disseminated encephalomyelitis (ADEM) ( n = 8), multifocal ( n = 7), TM ( n = 3), brainstem ( n = 1), and other encephalitis ( n = 1). A significant improvement in both the EDSS and VA measures was observed at follow-up compared to the time of IVIG treatment initiation ( p < 0.0001 for both outcome measures). Conclusion: IVIG may be an effective treatment option for acute MOGAD attacks. Further prospective studies are warranted to validate our results.
Objective: To study the long-term outcomes of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) patients without chronic steroid-sparing immunotherapy. Background: MOGAD is a potentially relapsing autoimmune demyelinating disease with less morbidity than aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder. The optimal strategy for maintenance immunotherapy in MOGAD is uncertain. Design/Methods: A retrospective chart review of MOGAD patients at four tertiary-care centers in the US and Europe was performed. Patients included were: 1) diagnosed with MOGAD, 2) incident at the participating centers (seen initially with no more than 1 attack), and 3) observed for at least 3 years without any chronic immunotherapies other than steroids. Outcomes were collected at least 3 months after the last attack. Results: There were 55 incident cases observed for at least 3 years without chronic steroid-sparing immunotherapy. The median age of onset was 23.7 (IQR 8.9–47.0) years, 42% had a pediatric onset (<18 years), 66% were female, and 87% were White. During the median 75.8 (IQR 54.2–125.5) months of observation without chronic immunotherapy, 36.4% suffered a relapse with a median of 1 (IQR 1–2) attack. The median Expanded Disability Status Scale (EDSS) scores after recovery from the first attack and at the final follow-up was 0 (IQR 0–1.0) (p = 0.068). The median logMAR visual acuity of the worse eye was also unchanged with both being 0 (IQR 0–0, 0–0.1) (Snellen equivalent of 20/20) (p = 0.317). Seroreversion to MOG-IgG negative was seen in 11/41 (27%) patients with paired serum testing. Overall, three (6%) patients received >10 mg/day of oral prednisone for at least 6 months. Nine (16%) patients eventually received a steroid-sparing maintenance immunotherapy. Conclusions: Without chronic immunotherapy, some MOGAD patients had relapses, but overall did not have a significant decline in either EDSS or visual acuity. Close observation with timely acute treatment for relapses may be justified in certain MOGAD patients. Disclosure: Miss Tisavipat has nothing to disclose. Dr. Wilf-Yarkoni has nothing to disclose. The institution of Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received research support from Viela Bio. Dr. Flanagan has received research support from UCB. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Redenbaugh has nothing to disclose. Dr. Schwartzmann has nothing to disclose. Dr. Hellmann has nothing to disclose. Dr. Tolkovsky has nothing to disclose. Prof. Kalisch has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Prof. Kalisch has stock in Biontech. Dr. Lotan has nothing to disclose. Dr. Levy has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitsubishi Pharma. Dr. Levy has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB Pharma. Dr. Levy has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sanofi. Dr. Levy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Levy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Levy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Levy has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. Dr. Levy has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Various law firms. The institution of Dr. Levy has received research support from National Institutes Health. Ms. Salky has nothing to disclose. Adi Vaknin-Dembinsky has nothing to disclose. Dr. Ganelin-Cohen has nothing to disclose. Dr. Lopez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Lopez has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech . Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech, Inc.. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sage Therapeutics, Inc.. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Astellas. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Prime Therapeutics. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MedImmune/Viela Bio. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys Therapeutics. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche/Genentech. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB, Inc. Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Hoffman/LaRoche AG. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genetech. Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for F. Hofman/LaRoche. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Pittock has received research support from Grifols. The institution of Dr. Pittock has received research support from NIH. The institution of Dr. Pittock has received research support from Viela Bio/MedImmune/Horizon. The institution of Dr. Pittock has received research support from Alexion Pharmaceuticals. The institution of Dr. Pittock has received research support from F. Hoffman/LaRoche/Genentech. The institution of Dr. Pittock has received research support from NovelMed. The institution of Dr. Pittock has received research support from AstaZeneca. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. John J. Chen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB. John J. Chen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. John J. Chen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Horizon.
Acute optic neuritis treatment lacks standardized protocols. The value of oral prednisone taper (OPT) following intravenous methylprednisolone (IVMP) on visual outcome parameters in optic neuritis (ON) has never been explored. In the present retrospective study, we investigated whether OPT after IVMP affects the structural and functional visual outcomes of inaugural clinically isolated syndrome (CIS)- or multiple sclerosis (MS)-ON. Adult patients with acute, inaugural, unilateral CIS- or MS-ON, treated with IVMP in Germany and Israel were stratified into patients treated with IVMP alone-versus IVMP and OPT. Inclusion criteria were age ≥18, CIS or MS diagnosis according to McDonald criteria 2017, available visual acuity (VA) at nadir before treatment initiation and at follow-up ≥5 months, as well as a spectral domain optic coherence tomography (OCT) data scan at follow-up. Exclusion criteria included recurrent ON, concomitant ophthalmological comorbidities, optical coherence tomography (OCT) of insufficient quality and ON-related escalation therapy after IVMP. The structural outcome was defined as the average retinal nerve fiber layer (RNFL) difference between the ON-affected and the unaffected eye, while the functional outcome was defined as the final high-contrast best-corrected VA (HC-BCVA) at follow-up compared to nadir. The comparative analysis was performed using linear regression analysis, adjusted for sex, age, and days-to-treatment. Fifty-one patients met the inclusion criteria (25% male). The mean age was 33.9 (±10.23) years. Twenty-six patients (51%) received OPT following IVMP. There was no difference in nadir HC-BCVA between the groups (0.39 No OPT; 0.49 With OPT, P = 0.36). Adjusted linear regression analysis did not indicate an influence of OPT on RNFL thickness or on HC-BCVA (beta coefficient for RNFL difference in percentages: 0.51, 95%-CI: [-4.58, 5.59], beta coefficient for logMAR: 0.11, 95%; CI [-0.12, 0.35] at follow-up. In conclusion, the addition of OPT to IVMP did not affect RNFL thickness or the final VA in a retrospective cohort of 51 patients with inaugural acute CIS- or MS-ON. The results of this exploratory study are currently being re-examined in a large-scale, demographically diverse, prospective study.