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The objectives of the present study were to describe the frequency of aggressive multiple sclerosis (aMS) as well as to compare clinical and radiological characteristics in aMS and non-aMS patients included in RelevarEM (NCT03375177).The eligible study population and cohort selection included adult-onset patients (≥18 years) with definite MS. AMS were defined as those reaching confirmed EDSS ≥ 6 within 5 years from symptom onset. Confirmation was achieved when a subsequent EDSS ≥ 6 was recorded at least six months later but within 5 years of the first clinical presentation. AMS and non-aMS were compared using the χ2 test for categorical and the Mann-Whitney for continuous variables at MS onset and multivariable analysis was performed using forward stepwise logistic regression with baseline characteristics at disease onset.A total of 2158 patients with MS were included: 74 aMS and 2084 non-aMS. The prevalence of aMS in our cohort was 3.4% (95%CI 2.7-4.2). AMS were more likely to be male (p = 0.003), older at MS onset (p 0.001), have primary progressive MS (PPMS) phenotype (p = 0.03), multifocal presentation (p 0.001), and spinal cord as well as infratentorial lesions at MRI during disease onset (p = 0.004 and p = 0.002, respectively).3.4% of our patient population could be considered aMS. Men, patients older at symptom onset, multifocal presentation, PPMS phenotype, and spinal cord as well as brainstem lesions on MRI at clinical presentation all had higher odds of having aMS.

Resumen Introduccion El estatus epileptico de reciente inicio (NORSE, por sus siglas en ingles) es un sindrome clinico caracterizado por un estatus epileptico refractario en un paciente sin antecedentes de enfermedad neurologica previa. El objetivo del presente trabajo es describir una serie de pacientes con diagnostico de NORSE presentados en un centro de alta complejidad. Material y metodos Se seleccionaron 48 historias clinicas de pacientes con diagnostico de estatus epileptico internados durante julio del 2014 a julio del 2018, se identificaron 5 casos que cumplieron con los criterios diagnosticos de NORSE. Se describieron: datos demograficos, forma de presentacion clinica, tratamiento farmacologico, estudios complementarios y escala de Rankin modificada (eRm) a los 3 meses. Segun su etiologia se clasificaron en indeterminado: NORSE-I; criptogenico: NORSE-C y con etiologia definida: NORSE-D. Resultados El rango etario fue de 17 a 79 anos. Segun su etiologia se identificaron: un caso de NORSE-I, 2 casos de NORSE-C, un caso de NORSE-D de causa autoinmune (anti-NMDA) y una enfermedad de Creutzfeldt-Jakob. El electroencefalograma mostro descargas generalizadas en todos los pacientes. Tres de ellos recibieron tratamiento inmunologico empirico, presentando buena respuesta solo un caso. En relacion a su evolucion a 3 meses, 2 pacientes presentaron eRm 5, uno eRm 1 y 2 fallecieron. Conclusiones El NORSE es un sindrome clinico que representa una urgencia neurologica. En nuestra serie la mortalidad fue elevada, similar a lo reportado en la literatura. La unica etiologia potencialmente tratable identificada fue la autoinmune.

Identification of triggers that potentially instigate attacks in neuromyelitis optica spectrum disorders (NMOSD) and multiple sclerosis (MS) has remained challenging. We aimed to analyze the seasonality of NMOSD and MS attacks in an Argentinean cohort seeking differences between the two disorders.A retrospective study was conducted in a cohort of NMOSD and MS patients followed in specialized centers from Argentina and enrolled in RelevarEM, a nationwide, longitudinal, observational, non-mandatory registry of MS/NMOSD patients. Patients with complete relapse data (date, month and year) at onset and during follow-up were included. Attack counts were analyzed by month using a Poisson regression model with the median monthly attack count used as reference.A total of 551 patients (431 MS and 120 NMOSD), experiencing 236 NMOSD-related attacks and 558 MS-related attacks were enrolled. The mean age at disease onset in NMOSD was 39.5 ± 5.8 vs. 31.2 ± 9.6 years in MS (p 0.01). Mean follow-up time was 6.1 ± 3.0 vs. 7.4 ± 2.4 years (p 0.01), respectively. Most of the included patients were female in both groups (79% vs. 60%, p 0.01). We found a peak of number of attacks in June (NMOSD: 28 attacks (11.8%) vs MS: 33 attacks (5.9%), incidence rate ratio 1.82, 95%CI 1.15-2.12, p = 0.03), but no differences were found across the months in both disorders when evaluated separately. Strikingly, we observed a significant difference in the incidence rate ratio of attacks during the winter season when comparing NMOSD vs. MS (NMOSD: 75 attacks (31.7%) vs MS: 96 attacks (17.2%), incidence rate ratio 1.82, 95%CI 1.21-2.01, p = 0.02) after applying Poisson regression model. Similar results were observed when comparing the seropositive NMOSD (n = 75) subgroup vs. MS.Lack of seasonal variation in MS and NMOSD attacks was observed when evaluated separately. Future epidemiological studies about the effect of different environmental factors on MS and NMOSD attacks should be evaluated prospectively in Latin America population.

The objective of this study was to describe and compare the baseline epidemiological data of multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) patients included in RelevarEM (Clinical Trials registry number NCT03375177). RelevarEM is a longitudinal, strictly observational MS and NMOSD registry in Argentina. Epidemiological and comorbidity data from MS and NMOSD patients were described and compared. For comorbidities, the Charlson comorbidity index (CCI) was used to calculate the burden at entry. CCI was stratified in 0 and ≥ 1 and described for the entire cohort. A total of 1588 and 75 MS and NMOSD patients (respectively) were included. For MS patients, the mean age was 42 ± 7 years, female sex 65.3%, mean EDSS 2, and mean disease duration 8 ± 6 years. In NMOSD, the mean age was 40 ± 7 years, female sex 78.7%, mean disease duration 5 ± 3.5 years, and mean EDSS 2.5. The most frequent MS phenotype was RRMS in 82.4%. In MS, the CCI was 0 in 85.8.2% while ≥ 1 was in 14.2% of patients. Regarding phenotype stratification, CCI ≥ 1 was 3.9% in CIS, 13.5% in RRMS, 28.7% in SPMS, and 17.4% in PPMS (p

Resumen La hipertension endocraneana (HTE) en pacientes con insuficiencia renal cronica puede deberse a diferentes etiologias. Existen escasos reportes que sugieren que la HTE puede ocurrir en el contexto de una estenosis venosa asociada a fistulas arteriovenosas (FAV). Presentamos el caso clinico de una mujer joven que presento HTE luego de la realizacion de una FAV en el brazo derecho.

Resumen El sindrome de Tolosa-Hunt (STH) se caracteriza por compromiso del seno cavernoso y/o la fisura orbitaria superior. Es considerada una entidad benigna y muy poco frecuente. Su diagnostico es un reto al momento de definirlo entre la lista de diagnosticos diferenciales de oftalmoplejia dolorosa, requiriendo descartar causas mas frecuentes, algunas de ellas de mayor gravedad. Teniendo en cuenta los escasos reportes que existen en la literatura, presentamos una serie de 4 casos que cumplieron los criterios clinicos y radiologicos de STH.

Despite that different registries already exist in various countries in Europe and North America, no ongoing nationwide registry exists in Latin America (LATAM), a region where the disease behaves differently than in other regions. The objective of this document is to describe the methodology behind RelevarEM, the first nationwide MS registry in Argentina and LATAM. METHODS: In this article, we described the creation, implementation and data management of the nationwide MS registry in Argentina. The registry contains information on the structure, ethical aspects, implementation and variables of the registry (Clinical Trials registry number NCT NCT03375177). CONCLUSION: RelevarEM is the first MS nationwide registry in Argentina, as well as in LATAM, with the objective of providing reliable real-world data of MS in the country.

We aimed to analyze the accumulative risk of MRI and OB factors for evolution from RIS to MS in subjects included in the Argentinean MS registry (NCT03375177). RIS subjects were identified according to RIS diagnosis criteria. Subjects were longitudinally followed with clinical and MRI at intervals of 6 months. Time from RIS identification to the first clinical event was estimated using Kaplan–Meier. Multivariable Cox regression models were created to assess the independent predictive value of demographic characteristics, as well as clinical, OB and MRI data on time to the first clinical event. The single and increased risk factor of evolution of RIS was quantified. A total of 88 RIS subjects, mean follow-up time 42 ± 4 months were included. 39 (44.3%) and 23 (26.1%) had a new MRI lesion or a clinical event, respectively, during the follow-up. OB (HR 5.9, 95% CI 1.29–10.1, p = 0.004), infratentorial lesions (HR 3.7, 95% CI 1.09–7.5) and spinal cord lesions (HR 5.3, 95% CI 1.4–8.2, p = 0.01) at RIS identification were independent predictors associated with a subsequent clinical event. The accumulative risk showed that when two of the three factors (OB, infratentorial or spinal cord lesions) were present the HR was 10.4, 95% CI 4.4–22, p

Background There has been an increase in the number of reports of multiple sclerosis (MS) rebound activity (RA), which is usually defined as a severe disease reactivation after natalizumab or fingolimod withdrawal that exceeds pre-treatment baseline inflammatory activity. The frequency and risk factors that could predict RA remain unknown. Fingolimod is currently the most frequently prescribed disease modifying therapy for MS in Argentina, so that there is a need to determine possible predictors of RA. Objectives To identify risk factors for developing RA after fingolimod cessation; to describe RA characteristics, management and evolution. Methods The study was a multicenter, retrospective, case-control study of patients with MS who had discontinued fingolimod and were followed up to nine months after discontinuation. Demographic, clinical and paraclinical data was extracted, including age, gender, MS phenotype, reason for discontinuation, number of relapses during the year prior to suspension, time treated with fingolimod, EDSS before, during and after rebound, MRI findings. Results 26 cases of RA were matched 1:1 with patients without RA. The median time elapsed to RA was 50 days. 68% showed worsening of the EDSS in the evaluation at 3 months of RA. When compared with the control group, no difference was found in terms of age, gender, phenotype, EDSS at the moment of suspension, reason for discontinuation, number of relapses in the previous year, and time on therapy. Conclusion In this case-controlled study, no risk factors could be identified to predict RA after fingolimod cessation. Further controlled, prospective, better powered studies are needed to confirm these findings.

Background : In multiple sclerosis demographics there is a well-known female prevalence and male patients have been less specifically evaluated in clinical studies, though some clinical differences have been reported between sexes. Objective : The objective of this study was to assess clinical and demographic differences between male and female patients included in the national Argentine MS Registry – RelevarEM. Material and methods : This study was observational, retrospective, and was based on the data of 3,099 MS patients included as of 04 April 2021. The statistical analysis plan included bivariate analyses with the crude data and also after adjustment for the MS phenotype, further categorized as progressive-onset MS or relapsing-onset MS. In the adjusted analysis, the Mantel-Haenszel odds ratio was compared to the crude odds ratio, to account for the phenotype as a confounder. Results : The data from 1,074 (34.7%) men and 2,025 (65.3%) women with MS diagnosis were analysed. Males presented primary progressive disease two times more often than women (11% and 5%, respectively). In the crude analyses by sex, the presence of exclusively infratentorial lesions in the magnetic resonance imaging studies was more frequent in males than in females, but after adjustment by MS onset phenotype, such difference was only present in males with relapsing-onset MS (p = 0.00006). Similarly, worse Expanded Disability Status Scale scores were confirmed only in men with relapsing-onset disease after phenotype adjustment (p = 0.02). Conclusion : We did not find any statistically significant clinical or demographic difference between sexes when the progressive or remitting MS phenotype was specifically considered. However, the differences we found between the clinical phenotypes are in line with the literature and highlight the importance of stratifying the analyses by sex and phenotype when designing MS studies.

We aimed to assess platelet-to-lymphocyte (PLR) and neutrophil-to-lymphocyte ratios (NLR) for differentiating multiple sclerosis (MS) from aquaporin-4-antibody-positive neuromyelitis optica spectrum disorders (NMOSD) at disease onset.We retrospectively enrolled and reviewed the medical records of patients with MS (N = 50) and NMOSD (N = 33) followed in specialized MS/NMOSD centers from Argentina. Demographical and clinical (manifestation and disability) data and neuroradiological features (new/enlarging or contrast-enhancing lesions) were assessed at baseline, 1 and 2 years. Serum samples were obtained during the first relapse without a previous acute or chronic treatment, at 1 and 2 years. Mixed-effects model was used to identify independent associations between the log-transformed NLR or PLR and MS/NMOSD outcomes.PLR is increased in NMOSD when compared to MS (229.4 ± 86.74 vs. 186.6 ± 70.17, P = 0.01), but no significant differences were found for NLR (3.51 ± 1.29 vs. 3.30 ± 1.17, P = 0.43). PLR was the only independent predictor of poor physical disability score (EDSS ≥ 4) in NMOSD patients at 2 years (β=0.28, p = 0.02) after applying multivariate mixed-effects regression analysis. Additionally, multivariate logistic regression analysis showed that the PLR was the only independent predictor of EDSS ≥ 4 at 2 years (OR 28.8, p = 0.041) in the NMOSD group. The area under the receiver-operating characteristic curve was 0.841.PLR could be potentially useful as additional diagnostic tool in differentiating these two neuroinflammatory conditions at presentation. PLR can predict severity of neurological disability at 2 years in NMOSD patients.

As human and economic resources are limited, especially in Latin America (LATAM), it is important to identify research priorities to improve multiple sclerosis (MS) patients care in the region. The objective was to generate a multidisciplinary consensus on research priorities in MS for patients care in LATAM by involving healthcare professionals and MS patient associations. METHODS: consensus was reached through a four-step modified Delphi method designed to identify and rate research priorities in MS in LATAM. The process consisted of two qualitative assessments, a general ranking phase and a consensus meeting followed by a more detailed ranking phase RESULTS: a total of 62 participants (35 neurologists, 4 nurses, 12 kinesiologists, 7 neuropsychologists and 4 patient association members) developed the process. At the final ranking stage following the consensus meeting, each participant provided their final rankings, and the top priority research questions were outlined. 11 research priorities were identified focusing on healthcare access, costs of the disease, physical and cognitive evaluation and rehabilitation, quality of life, symptoms management, prognostic factors, the need of MS care units and patient's management in emergencies like COVID-19. CONCLUSION: this work establishes MS research priorities in LATAM from multiple perspectives. To pursue the actions suggested could launch the drive to obtain information that will help us to better understand the disease in our region and, especially, to better care for affected patients.

Introduction During the last 20 years, multiple sclerosis (MS) disease has seen major changes with new diagnostic criteria, a better identification of disease phenotypes, individualization of disease prognosis and the appearance of new therapeutic options in relapsing remitting as well as progressive MS. As a result, the management of MS patients has become more complex and challenging. The objective of these consensus recommendations was to review how the disease should be managed in Argentina to improve long-term outcomes in MS patients. Methods A panel of 36 experts in neurology from Argentina, dedicated to the diagnosis and care of MS patients, gathered both virtually and in person during 2018 and 2019 to carry out a consensus recommendation on the management of MS patients in Argentina. To achieve consensus, the methodology of “formal consensus-RAND/UCLA method” was used. Results Recommendations focused on diagnosis, disease prognosis, tailored treatment, treatment failure identification and pharmacovigilance process. Conclusions The recommendations of these consensus guidelines attempt to optimize the health care and management of patients with MS in Argentina.

Background Myelin oligodendrocyte glycoprotein antibodies (MOG-ab) have been described in aquaporin-4-antibodies(AQP4-ab)-negative neuromyelitis optica spectrum disorder (NMOSD) patients. We aimed to evaluate the percentage of AQP4-ab-negative NMOSD patients who are positive for MOG-ab in a cohort of Argentinean patients included in RelevarEM (Clinical Trials registry number NCT03375177). Methods RelevarEM is a longitudinal, strictly observational multiple sclerosis (MS) and NMOSD registry in Argentina. Of 3031 consecutive patients (until March 2020), 165 patients with phenotype of suspected NMOSD, whose relevant data for the purpose of this study were available, were included. Data on demographic, clinical, paraclinical and treatment in AQP4-ab (positive, negative and unknown) and MOG-ab (positive and negative) patients were evaluated. Results A total of 165 patients (79 AQP4-Ab positive, 67 AQP4-Ab negative and 19 unknown) were included. Of these, 155 patients fulfilled the 2015 NMOSD diagnostic criteria. Of 67 AQP4-Ab-negative patients, 36 (53.7%) were tested for MOG-Ab and 10 of them (27.7%) tested positive. Serum AQP4-ab levels were tested by means of cell-based assay (CBA) in 48 (35.2%), based on tissue-based indirect immunofluorescence assays in 58 (42.6%) and enzyme-linked immunosorbent assay in 4 (2.9%). All MOG-ab were tested by CBA. Optic neuritis (90%) was the most frequent symptom at presentation and optic nerve lesions the most frequent finding (80%) in neuroimaging of MOG-ab-associated disease. Of these, six (60%) patients were under immunosuppressant treatments at latest follow-up. Conclusion We observed that 27.7% (10/36) of the AQP4-ab-negative patients tested for MOG-ab were positive for this antibody, in line with results from other world regions.

Background Multiple sclerosis (MS) is the most common chronic immune-mediated neurological disorder in young adults, more frequently found in women than in men. Therefore, pregnancy-related issues have become an object of concern for MS professionals and patients. The aim of this work was to review the existing data to develop the first Argentine consensus for family planning and pregnancy in MS patients. Methods A panel of expert neurologists from Argentina engaged in the diagnosis and care of MS patients met both virtually and in person during 2019 to carry out a consensus recommendation for family planning and pregnancy in MS. To achieve consensus, the procedure of the “formal consensus-RAND/UCLA method” was used. Results Recommendations were established based on published evidence and expert opinion focusing on pre-pregnancy counseling, pregnancy, and postpartum issues. Conclusion The recommendations of these consensus guidelines are intended to optimize the management and treatment of MS patients during their reproductive age in Argentina.

Resumen La neuromielitis optica es una enfermedad inflamatoria, desmielinizante, inmunomediada y recurrente del sistema nervioso central que afecta a los nervios opticos y la medula espinal; puede coexistir con enfermedades autoinmunes, como el lupus eritematoso sistemico. Existen escasos reportes sobre el curso de la neuromielitis optica durante el embarazo y el posparto que describen exacerbaciones graves. Se presenta el caso de una mujer de 27 anos con diagnostico de neuromielitis optica y lupus eritematoso sistemico con embarazo sin complicaciones y recien nacido vivo a termino y sin malformaciones congenitas, con curso estable de neuromielitis optica y lupus eritematoso sistemico durante el embarazo y el puerperio. La evolucion de estas enfermedades durante el embarazo y el posparto parece ser deleterea. Deben extremarse medidas para prevenir toxicidad fetal de drogas utilizadas para tratar estas afecciones.

One of the biggest challenges in multiple sclerosis (MS) is the definition of treatment response/failure in order to optimize treatment decisions in affected patients. The objective of this consensus was to review how disease activity should be assessed and to propose recommendations on the identification of treatment failure in RRMS patients in Argentina. Methods A panel of experts in neurology from Argentina, dedicated to the diagnosis and care of MS patients, gathered both virtually and in person during 2016 and 2017 to carry out a consensus recommendation on the identification of treatment failure in RRMS patients. To achieve consensus, the methodology of “formal consensus-RAND/UCLA method” was used. Results Recommendations were established based on published evidence and the expert opinion. Recommendations focused on disease management, disease activity markers and treatment failure identification were determined. Main consensus were: ≥ 2 relapses during the first year of treatment and/or ≥ 3 new or enlarged T2 or T1 GAD + lesions and/or sustained increase of ≥ 2 points in EDSS or ≥ 100% in T25FW defines treatment failure in RRMS patients. Conclusions The recommendations of this consensus guidelines attempts to optimize the health care and management of patients with MS in Argentina.

Resumen Los tratamientos modificadores de la enfermedad inyectables para la esclerosis multiple recurrente remitente tienen una eficacia discreta, pero un conocido perfil de seguridad avalado por varios anos de uso en la practica clinica. Tienen la incomodidad de la via de administracion y de los conocidos efectos adversos en el sitio de puncion asi como del sindrome seudogripal. En los ultimos tiempos ha empezado a cambiar el paradigma de tratamiento con el desarrollo de farmacos orales y anticuerpo monoclonales. Se ha mejorado significativamente el nivel de eficacia frente a la enfermedad, pero tambien obliga al neurologo tratante a conocer el manejo y los efectos adversos de estos farmacos. Los farmacos orales probablemente mejoren los niveles de adherencia al tratamiento, traduciendose esto en un mejor control de la enfermedad. Fingolimod es el primer farmaco oral aprobado en el mundo para el tratamiento de las formas recurrentes de la enfermedad. Es un analogo de la esfingosina que produce una retencion rapida, selectiva y reversible de los linfocitos dentro de los ganglios linfaticos, produciendo consecuentemente una menor invasion de linfocitos autorreactivos al sistema nervioso central, y por lo tanto, dano inflamatorio. La presente revision intenta resumir los conocimientos actuales del fingolimod en cuanto a su mecanismo de accion, eficacia y seguridad.

Nora Fernandez Liguori a,∗, Juan Ignacio Rojas b, Mario Bana c,d, Andres Barboza e, Adriana Carra f, Jorge Correale g, Edgardo Cristiano b, Norma Deri h, Marcela Fiol g, Orlando Garcea i, Alejandra Martinez j, Cristina Martinez c, Daniel Munoz h,k, Marcela Parada Marcilla l, Liliana Patrucco b, Walter Perez m, Lucas Martin Romano n, Marina Romano o, Roberto Rotta Escalante p, Vladimiro Sinay q,r, Adriana Tarulla d,s, Silvia Tenembaum t, Andres Villa u, Maria Celica Ysrraelit g y por el Grupo de trabajo de enfermedades desmielinizantes y el Grupo de trabajo de neurofarmacologia de la Sociedad Neurologica Argentina

Like MS prevalence, oligoclonal bands (OCB) frequency seems to follow a latitudinal gradient. Argentina is extensive, latitude-wise, and previous studies have not found an MS prevalence latitudinal gradient. Our aim is to describe OCB prevalence in MS, clinically isolated syndrome (CIS) and radiologically isolated syndrome (RIS) patients included in the Argentinean MS and NMOSD registry (RelevarEM) and to investigate if it follows a latitudinal gradient.For each province, an average latitude was calculated, and OCB frequency was investigated. Multivariate logistical regression analysis and linear correlation were performed. Statistical analysis was repeated after excluding patients from centers using isoelectric focusing (IEF) in less than 95% of patients (CwIEF95).We included 2866 patients. OCB where positive in 73.9% of patients. No association or correlation were found between OCB and latitude of residence, even after excluding patients from (CwIEF95).OCB positivity does not follow a latitudinal gradient in Argentina. Also, OCB positivity is lower than described in other world regions.