[Core data set for real world data in multiple sclerosis: customization for latin america from a global task force recommendation]. / Core data set para la generación de datos de la vida real en esclerosis múltiple: adaptación de una iniciativa global para América Latina.

INTRODUCTION: The primary objective of the core data set is to reduce heterogeneity and promote harmonization among data sources in EM, thereby reducing the time needed to execute real life data collection efforts. Recently, a group led by the Multiple Sclerosis Data Alliance has developed a core data set for collecting real-world data on multiple sclerosis (MS) globally. Our objective was to adapt this global data set to the needs of Latin America, so that it can be implemented by the registries already developed and in the process of development in the region. MATERIAL AND METHODS: A working group was formed regionally, the core data set created globally was adapted (translation process into Spanish, incorporation of regional variables and consensus on variables to be used). Consensus was obtained through the remote Delphi methodology of a round of questionnaires and remote discussion of the core data set variables. RESULTS: A total of 25 professionals from Latin America carried out the adaptation process between November 2022 and July 2023. Agreement was established on a core data set of nine categories and 45 variables, version 2023 to suggest its implementation in developed or developing registries, and MS cohorts in the region. CONCLUSION: The core data set seeks to harmonize the variables collected by registries and cohorts in MS in Latin America in order to facilitate said collection and allow collaboration between sources. Its implementation will facilitate real life data collection and collaboration in the region.

TITLE: Core data set para la generación de datos de la vida real en esclerosis múltiple: adaptación de una iniciativa global para América Latina.Introducción. Los objetivos primarios del core data set son reducir la heterogeneidad y promover la armonización entre las fuentes de datos en la esclerosis múltiple (EM), reduciendo así el tiempo necesario para ejecutar esfuerzos en la recolección de datos de vida real. Recientemente, un grupo liderado por la Multiple Sclerosis Data Alliance ha desarrollado un core data set para la recolección de datos del mundo real en EM a nivel global. Nuestro objetivo ha sido adaptar y consensuar este conjunto de datos globales a las necesidades de América Latina para que pueda ser implementado por los registros ya desarrollados y en proceso de desarrollo en la región. Material y métodos. Se conformó un grupo de trabajo regionalmente y se adaptó el core data set creado globalmente (proceso de traducción al español, incorporación de variables regionales y consenso sobre variables que se iban a utilizar). El consenso se obtuvo a través de la metodología Delphi remoto de ronda de cuestionarios y discusión a distancia de las variables del core data set. Resultados. Veinticinco profesionales de América Latina llevaron adelante el proceso de adaptación entre noviembre de 2022 y julio de 2023. Se estableció un acuerdo sobre un core data set de nueve categorías y 45 variables, versión 2023, con la sugerencia de implementarlo en registros desarrollados o en vías de desarrollo y cohortes de EM en la región. Conclusión. El core data set busca armonizar las variables recolectadas por los registros y las cohortes de EM en América Latina con el fin de facilitar dicha recolección y permitir una colaboración entre fuentes. Su implementación facilitará la recolección de datos de vida real y la colaboración en la región.

Employment status monitoring in an Argentinian population of patients with multiple sclerosis: Particularities of a developing country.

BACKGROUND: Multiple sclerosis (MS) is a neurological chronic disease that causes a number of physical, cognitive and emotional symptoms. The identification of these factors will allow mitigating unemployment and improve quality of life of patients. The Buffalo Vocational Monitoring Survey (BVMS) is a tool to characterize Work-Challenged patients. OBJECTIVE: To describe and analyze BVMS data in people with multiple sclerosis (PwMS) from Argentina. To study the association with physical, cognitive and psychiatric morbidity in employed patients, comparing the performance of MS Work-Challenged and MS Work-Stable patients, with and without accommodations. METHODS: 119 MS patients were administered the Argentina adaptation of the BVMS, and completed measures of physical disability, fatigue, depression, cognitive processing speed, memory and verbal fluency. RESULTS: 65.54% of the patients were employed and 19.32% were unemployed, the remaining having roles of housewife, students and disability retirees. Within the employed subgroup, 60.26% were working as employees and 39.74% were self-employed. Cognitive and clinical variables differentiate patients with and without negative events and accommodations (p >  0.05). CONCLUSIONS: This Spanish version BVMS is considered a new tool to monitor employment difficulties in Spanish-speaking MS patients. MS Work-Challenged had a higher depression, fatigue and worse performance in cognitive variables.

[Argentinean consensus guidelines on the identification and clinical care of secondary progressive multiple sclerosis]. / Consenso sobre la identificación y seguimiento de la esclerosis múltiple secundaria progresiva en Argentina.

INTRODUCTION: The identification, diagnosis, follow-up, and treatment of patients with secondary progressive multiple sclerosis (SPMS) show significant differences between health care professionals in Argentina. AIM: To provide consensus recommendations on the management of patients with SPMS in Argentina to optimize patient care. DEVELOPMENT: A panel of expert neurologists from Argentina dedicated to the diagnosis and care of multiple sclerosis patients gathered during 2019 and 2020 to carry out a consensus recommendation on the diagnosis and treatment of SPMS patients in Argentina. To achieve consensus, the methodology of 'formal consensus-RAND/UCLA method' was used. Recommendations were established based on published evidence and the expert opinion. Recommendations focused on how to define SPMS and how to follow SPMS patients. CONCLUSION: The recommendations of this consensus guidelines attempt to optimize the care of SPMS patients in Argentina.

TITLE: Consenso sobre la identificación y seguimiento de la esclerosis múltiple secundaria progresiva en Argentina.Introducción. Existen diferencias significativas en el diagnóstico, la identificación y el seguimiento de pacientes con esclerosis múltiple secundaria progresiva (EMSP) entre los profesionales de la salud a cargo de su tratamiento. Objetivo. Proveer recomendaciones sobre el tratamiento de los pacientes con EMSP en Argentina con el fin de optimizar su cuidado. Desarrollo. Un grupo de neurólogos expertos en esclerosis múltiple de Argentina elaboró un consenso para el tratamiento de pacientes con EMSP en la región mediante metodología de ronda de encuestas a distancia y reuniones presenciales. Se establecieron 33 recomendaciones basadas en la evidencia publicada y en el criterio de los expertos que participaron. Las recomendaciones se enfocaron en el diagnóstico y el seguimiento de los pacientes con EMSP. Conclusión. Las recomendaciones establecidas en el presente consenso permitirían optimizar el cuidado y el seguimiento de los pacientes con EMSP en Argentina.

Cognitive impairment in neuromyelitis optica spectrum disorders: What do we know?

The aim of this study is to make a descriptive review of the bibliography available on cognitive dysfunction in patients with neuromyelitis optica spectrum disorders (NMOSD). We selected fifteen studies that quantitatively assess the relationship between NMOSD and one or more cognitive variables. Results showed that patients with NMOSD had a decrease in cognitive functions. Cognitive dysfunctions were found in 35-67% of patients with NMOSD, specifically in the attention, memory and information processing speed. Cognitive dysfunctions were found to relate to injuries in white matter as well as clinical variables and depression.

Increasing prevalence of multiple sclerosis in Buenos Aires, Argentina.

UNLABELLED: In 1996, the prevalence of multiple sclerosis (MS) for the metropolitan area of Buenos Aires using the capture-recapture method was estimated to be between 14 and 19.8 cases per 100,000 inhabitants. The aim of this study was to update the prevalence to 2014 following the same methodology. METHODS: Gran Buenos Aires is the denomination that refers to the megalopolis comprised by the autonomous city of Buenos Aires and the surrounding conurbation of the province of Buenos Aires. The study was carried out taking December 2014 as the prevalence month. We used the capture-recapture method to estimate the prevalence of MS cross-matching registries from 6 MS Centers from the metropolitan area of Buenos Aires. Log-linear model Poisson regression was used to estimate the number of affected MS patients not detected by any of the 6 sources considered. RESULTS: 1035 registries were obtained from the 6 lists from 910 different patients detected. The population of the area based on 2010 census was 12,806,866, the number of MS cases estimated amongst source interactions were 4901. The estimated prevalence was 38.2 per 100,000 inhabitants (95% CI 36.1-41.2). CONCLUSION: The study is an update almost 20 years after the first one in the area showing a significant increase in the previous reported prevalence. Our findings are in line with previous studies performed in other regions of the world.

Early treatment of multiple sclerosis: a Latin American experts meeting.

Patients with clinically isolated syndrome (CIS) by definition do not have multiple sclerosis (MS) but are at risk of developing it. While studies show earlier immunomodulating drug use is effective, treatment must consider likely patient prognosis. In this paper we review current diagnosis, prognosis, and treatment literature for patients with CIS within Latin American clinical settings. Latin American MS experts, convened by ACINDES (The Civil Association for Research and Development in Health), reviewed current CIS (and early MS) literature and drew consensus conclusions. Three subgroups addressed separate questionnaires on CIS issues: prognosis, diagnosis, and treatment. MRI can contribute to predicting MS risk in patients with CIS; in Latin America, investigation of haplotype presence associated with CIS would be appropriate. McDonald's criteria and subsequent revisions enable earlier, more accurate MS diagnosis. Type A evidence exists supporting all leading immunomodulating MS drugs for effective treatment of CIS with a high risk of conversion to MS. In conclusion, patients with CIS are usually young, with often-limited symptomatic manifestations, and must be adequately prepared to receive preventive treatment. This consensus review should contribute to the dialogue between physicians and patients.

Epidemiological characteristics of pregnancy, delivery, and birth outcome in women with multiple sclerosis in Argentina (EMEMAR study).

BACKGROUND: The influence of pregnancy on Multiple Sclerosis (MS) has been extensively studied but such influence on Latin American women with MS has not been characterized. Our objective was to describe the course of pregnancy and birth outcome in Argentinean MS patients and the evolution of MS during pregnancy and after delivery. METHOD: We used a retrospective design in eight MS centers in Argentina and administered a survey to women with definite MS (Mc Donald) with pregnancies during or after MS onset. We contacted 355 women of which 81 met inclusion criteria. We recorded 141 pregnancies. RESULTS: Involuntary abortion was observed in 16% of pregnancies (95% CI = 10-23). Thirty five women received immunomodulatory therapy (IMT) before 42 pregnancies. Twenty three (55%) out of 42 pregnancies were exposed to IMT. The mean time of IMT discontinuation before conception in 19 (45.2%) pregnancies without exposure, was 104 days (95% CI = 61.0-147.0). There were 103 deliveries: 79% full term. Birth defects were detected in 19% of pregnancies exposed to IMT (95% CI = 4-46) and in 2% of non-exposed (95% CI = 0.3-8.0). The mean relapse rate was: pre-pregnancy year: 0.22 (95% CI = 0.12-0.32); pregnancy: 0.31 in 1st (95% CI = 0.10-0.52), 0.19 (95% CI = 0.03-0.36) in 2nd, and 0.04 in 3rd trimester (95% CI = -0.04-0.12); 1st trimester post delivery: 0.82 (95% CI = 0.42-1.22). CONCLUSION: We observed a higher rate of birth defects among infants exposed to immunomodulators in utero than those not exposed. The reduction in MS relapses during 2nd and 3rd trimester of pregnancy and its increase during postpartum is consistent with previous reports.

Prevalence of multiple sclerosis in Buenos Aires, Argentina using the capture-recapture method.

BACKGROUND: Scarce data exist about multiple sclerosis (MS) prevalence in South America. The objective of the study is to determine the prevalence of MS in a high populated area from Argentina (Greater Buenos Aires Metropolitan area) using the capture-recapture methodology. METHODS: Greater Buenos Aires is the generic denomination that refers to the megalopolis comprised by the autonomous city of Buenos Aires and the surrounding conurbation of the province of Buenos Aires. The study was carried out taking July 1996 as the prevalence month. We used capture-recapture method to estimate the prevalence of MS cross matching registries from four MS Centers. RESULTS: A total of 803 registries were obtained from the four lists. Log-linear model for capture-recapture method was used to analyze the data. The population of the area based on the 1990 census was 12,594,974; the number of MS cases estimated amongst sources interactions were between 1833 and 2359; the prevalence estimated ranged from 14 to 19.8 cases per 100,000 inhabitants. CONCLUSIONS: This is the first study to provide epidemiological data on the prevalence of MS in a large population in Argentina (Greater Buenos Aires Metropolitan area). Further epidemiological studies will clarify the true prevalence of MS in South America.

Argentine Patagonia: prevalence and clinical features of multiple sclerosis.

There are few studies reporting multiple sclerosis prevalence rates in the Buenos Aires region, Argentina (latitude 34 degrees S) (between 12-18.5/100 000 inhabitants), and no studies have been performed in the larger region between parallels 36 degrees and 55 degrees S. The aim of this study is to determine the prevalence rates and clinical features of multiple sclerosis in residents of the Argentine Patagonia. Four cities from the region were selected for this study, giving a sample population of 417 666 inhabitants (approximately 24% of the total Patagonia population). 1(st) March 2002 was determined as prevalence day. Patients were ascertained using multiple case-finding methods. The point prevalence rate was 17.2/100 000 (17.2 age-adjusted to the world population). Prevalence rates were higher for women than for men, 22.1 versus 12.2/100 000 inhabitants (21.4 versus 12.7 sex-adjusted to the world population). The study population was mainly of European descent and mestizoes. Clinical features were similar to those reported in other countries. This study shows that Argentine Patagonia is a medium-risk area with no south-north gradient between parallels 55 degrees and 36 degrees S. The Patagonia population shows recent internal migration that makes it difficult to determine whether the exposure to potential risk factors has been long enough to modify the disease incidence.

Cognitive impairment in patients with multiple sclerosis using the Brief Repeatable Battery-Neuropsychology test.

AIMS: To assess the cognitive impairment of multiple sclerosis (MS) patients using the Brief Repeatable Battery-Neuropsychology (BRB-N) test. METHODS: The performance of 59 patients with MS in the BRB-N test was assessed and compared with 152 matched healthy controls (HC). RESULTS: In most tests, MS patients performed worse than controls. Age and educational level strongly influenced the performance of the subjects. The Symbol Digit Modality Test (SDMT) best correlated with the other individual tests and contributed most to the general BRB-N factor. Furthermore, a correlation between physical disability and performance in some BRB-N tests was observed. Indeed, patients with progressive MS and greater physical disability performed worse in some tests than less disabled patients with relapsing MS. By creating a global BRB-N Z score, we found that patients generally performed 0.7 standard deviation (SD) below the level of controls. We obtained cut-off values stratified by age and education to determinate cognitive impairment in MS patients. CONCLUSIONS: Our data show that cognitive impairment is prevalent amongst MS patients, and that a single cognitive measurement might be useful for monitoring patients during the progression of this illness.

Further studies on HTLV-I associated myelopathy in Argentina.

We report 10 HTLV-I virus seropositive subjects, eight of them with HTLV-I associated myelopathy (HAM), two of them also infected with HIV as well as two asymptomatic HTLV-I+ relatives of two unrelated patients. HTLV-I is endemic in several tropical areas, where it causes different neurological diseases. Only few patients have been reported in our country since 1994. We studied 8 patients, who fulfilled the clinical criteria for chronic spastic paraplegia, and 2 other non-symptomatic HTLV-I seropositive relatives, with electromyography (EMG), motor and sensory conduction velocities (NCV), somatosensory, visual and brainstem auditory evoked potentials (SSEP, VEP and BAEP), Magnetic Resonance Images (MRI) and cerobrospinal fluid (CSF) analysis. The latter was carried out only in seven symptomatic patients. In every case positive ELISA tests for HTLV-I/II were confirmed by Western Blot. The two asymptomatic persons were clinically and electromyographically assessed, one of them was also submitted to SSEPs studies. Three patients were males. Patient's ages ranged from 5 to 65 years old. All symptomatic patients showed muscular weakness, spasticity with pyramidal signs and sphincter disturbances. Five of them had paresthesias and 2 had burning pain on their feet. The EMGs and the NCVs were normal in 7 patients and in the 2 asymptomatic ones. SSEPs, obtained by stimulating the posterior tibial nerves, were impaired in 7 patients and in the asymptomatic person who received the procedure. The 7 symptomatic patients who underwent lumbar puncture had positive tests for HTLV-I in CSF, 3 out of these 7 patients had also high protein levels and 4 had increased number of lymphocytes. In 2 patients intrathecal IgG production could also be demonstrated. MRI were normal in 7 patients and in the 2 asymptomatics, the exception being a female who had bilateral hyperintense lesions in cerebral white matter in T2. In conclusion, tropical spastic paraparesis is apparently a rare disorder in Argentina. However, some cases have been reported recently. Most probably, its prevalence is currently underestimated. Its diagnosis should be considered in every patient with progressive spastic paraplegia.

Multiple sclerosis: assessment of lesional levels by means of transcranial stimulation.

Conduction in the Pyramidal Tracts (PTs) was studied in 30 healthy volunteers and 17 patients with definite Multiple Sclerosis. Central motor conduction time (CMCT) was assessed employing unipolar electrical transcranial stimulation and studies of the peripheral conduction time. Fourteen out of the seventeen studied patients showed CMCT abnormalities. No significative correlation was found between the intensity of muscle weakness and the slowness of conduction in the corresponding PTs. Subclinical abnormalities were detected in more than half of the studied patients. CMCT slowness showed a good correlation with the time course of the disease in the cortico-cervical (CCT) but not in the cortico-lumbar (CLT) tracts. Evidence favoring the existence of two different lesional levels along the PSs was found in 9 out of the 14 patients studied with recordings in the hand and leg muscles. Two patterns of abnormalities gave support to this conclusion. The first consisted of slowed conduction or inexcitability in both the CCT and CLT at one side, while only one of the contralateral PTs was impaired. In the second one, the 4 explored PTs showed a slowed conduction, but in addition, CMCT difference between the CLT and CCT was longer than normal. Both patterns suggest that one lesional level could be situated between the internal capsula and the lower cervical cord and the other in the spine below the 8th cervical methamera or in the corona radiata.

Hidrocefalia crónica obstructiva por histoplasmosis meningo-cerebral / Chronic obstructive hydrocephalus due to meningeal and cerebral histoplasmosis

El presente caso describe un caso de histoplasmosis del sistema nervioso central con hidrocefalia obstructiva, que ha estado presente durante 8 años sin mayores secuelas o evidencias clínicas de infección activa en otros órganos, en un huésped inmunocompatible. La punción lumbar y el análisis del líquido cefalorraquídeo (LCR) revelaron un elevado nivel de proteínas, linfocitosis e hipoglucorraquia. Los títulos de fijación de complementos séricos y del LCR para histoplasma fueron positivos. La tomografía computada (TC) demostró que existía hidrocefalia y dos lesiones periventriculares con un marcado refuerzo con contraste. Se inició el tratamiento con altas dosis de ketoconazol y a continuación se administró fluconazol con una mejoría parcial del estado clínico y los valores de LCR. Finalmente se le practicó al paciente un tratamiento con anfotericina mostrando una completa remisión clínica, pero sin curación con valores variables en los controles de seguimiento a largo plazo de los títulos de complemento sérico y del LCR. En la actualidad, el paciente permanece asintomático y está tratado con itraconazol oral

Hidrocefalia crónica obstructiva por histoplasmosis meningo-cerebral / Chronic obstructive hydrocephalus due to meningeal and cerebral histoplasmosis

El presente caso describe un caso de histoplasmosis del sistema nervioso central con hidrocefalia obstructiva, que ha estado presente durante 8 años sin mayores secuelas o evidencias clínicas de infección activa en otros órganos, en un huésped inmunocompatible. La punción lumbar y el análisis del líquido cefalorraquídeo (LCR) revelaron un elevado nivel de proteínas, linfocitosis e hipoglucorraquia. Los títulos de fijación de complementos séricos y del LCR para histoplasma fueron positivos. La tomografía computada (TC) demostró que existía hidrocefalia y dos lesiones periventriculares con un marcado refuerzo con contraste. Se inició el tratamiento con altas dosis de ketoconazol y a continuación se administró fluconazol con una mejoría parcial del estado clínico y los valores de LCR. Finalmente se le practicó al paciente un tratamiento con anfotericina mostrando una completa remisión clínica, pero sin curación con valores variables en los controles de seguimiento a largo plazo de los títulos de complemento sérico y del LCR. En la actualidad, el paciente permanece asintomático y está tratado con itraconazol oral

Chronic pachymeningitis associated to hypereosinophilia: case report.

A male 22 years old patient who consulted due to headache and increasing visual loss is being presented. Alterations in the exam were represented by right amaurosis and left temporal hemianopsis. Pathological and tomographical studies revealed chronic pachymeningitis with eosinophilic infiltration. An elevated eosinophilia and an increasing in anti-A and anti-B isoagglutinins were associated to the clinical course. They returned to normal values after treatment with tiabendazol. Even though there is not a certain conclusion as regards the etiology, the probable parasitary nature of the process is outlined.