The quality of life of people with multiple sclerosis in Portugal

Introduction: Multiple sclerosis significantly affects the quality of life of those suffering from this specific condition. Objective: To assess the quality of life of people with multiple sclerosis and analyse the correlation between the disease and its associated effects and different sociodemographic, clinical, and functional variables. Materials and Methods: An observational, cross-sectional, descriptive-correlational and quantitative study conducted using a non-probabilistic convenience sample composed of 70 patients suffering from multiple sclerosis registered with the Multiple Sclerosis Association of the Central Region of Portugal. The data collection protocol included sociodemographic and clinical questions, the Family Apgar Scale, and the Barthel Index. Descriptive and inferential statistics were used to process the data. Data collection took place between April and July 2021. Results: The majority of participants reported a moderate overall quality of life (M=51,78 ± 24,09). Higher scores were observed in the social relationships and environmental health domains, while lower scores were recorded for the physical domain. Better quality of life was found to be positively associated with being under 45 years old, having higher educational qualifications, living in functional families, and experiencing greater functional independence in activities of daily living. Discussion: The variables with the strongest association were those capable of influencing the physical and social domains. Those variables explained 59.00% and 53.00% of the variability. Conclusions: These results indicate that people with multiple sclerosis have a compromised quality of life, highlighting the need for new strategies focusing on early diagnosis and effective preventive interventions meant to improve quality of life across all its domains.

Protocolo para el manejo del paciente con esclerosis multiple / Protocol for the management of patients with multiple sclerosis

El manejo y el pronóstico de la Esclerosis Múltiple (EM) ha cambiado sustancialmente en los últimos años, a ser una enfermedad muy reconocida por los Neurólogos; el conocimiento de los aspectos epidemiológicos, clínicos, diagnósticos y terapéutico han conllevado a un mejor diagnóstico y pronóstico para los pacientes permitiendo al clínico establecer una terapia individualizada para los pacientes según el fenotipo de la enfermedad que presenten. Con la expansión del arsenal terapéuticos, estos fármacos ejercen su eficacia modulando o suprimiendo la respuesta inmunológica. Este documento proporciona la clasificación de la enfermedad, manifestaciones clínicas, diagnóstico y tratamiento individualizado (Terapias Modificadoras de la enfermedad-TMD) para la toma de decisiones en el manejo de pacientes. El presente protocolo está elaborado con evidencia clínica luego de una exhaustiva búsqueda de literatura actualizada; con el fin de brindar opciones de manejo terapéutico para profesionales de la Neurología, que deberán tomar decisiones individualizadas en el manejo integral de los pacientes con EM...(AU)

Alucinosis peduncular en esclerosis múltiple, síntoma poco común en la expresión de enfermedad desmielinizante / Peduncular hallucinosis in multiple sclerosis, a rare symptom in demyelinating disease

Introducción: La alucinosis peduncular (AP) hace referencia a alucinaciones autodiscriminadas, cuyo origen son lesiones en el mesencéfalo y en el puente. Presentación del caso: Paciente 27 años, femenina, con alucinaciones visuales, auditivas autodiscriminadas por ella misma, sin antecedentes previos de importancia y con lesiones en resonancia magnética cerebral y cervical en el pedúnculo cerebeloso superior, tegmento pontino, y en columna cervical con bandas oligoclonales patrón 2, que cumplían criterios de Mc Donalds para esclerosis múltiple. Discusión: La alucinosis peduncular hace referencia a la presencia de alucinaciones visuales, criticadas por el paciente, con la consecuencia de lesiones de las vías inhibitorias por deaferentación y desinhibición mesencéfalotalámicas, y retinogenículo calcarina, descritas como manifestación de múltiples patologías neurológicas como trauma, afectación vascular, tumores y pocos casos de enfermedad desmielinizante, entre otras. Conclusión: La alucinosis peduncular es una forma atípica de presentación de lesiones pontomesencefálicas descritas en varias patologías; se debe tener en cuenta en la localización de la lesión neurológica; se han reportado pocos casos como síntoma de la enfermedad desmielinizante.

Introduction: Peduncular hallucinosis (PA) refers to self-discriminating hallucinations, these are caused by lesions in the midbrain and pons. Presentation of the case: 27-year-old right handed female patient with visual and auditory hallucinations self-discriminated by the patient, with no prior history of importance and with lesions in cerebral and cervical Magnetic Resonance in the superior cerebellar peduncle, pontine tegmentum, and in the cervical spine with pattern 2 oligo clonal bands, which met Mc Donald's criteria for multiple sclerosis. Discussion: Peduncular hallucinosis refers to the presence of visual hallucinations criticized by the patient, consequence of lesions in the inhibitory pathways with deafferentation and disinhibition of the midbrain-thalamic and retinogeniculus-calcarine pathways. Described as a manifestation of multiple neurological pathologies such as trauma, vascular, tumor and few cases of demyelinating among others. Conclusion: Peduncular hallucinosis is an atypical form of presentation of pontomesencephalic lesions described in several pathologies, it must be taken into account when locating the neurological lesion, few cases have been reported as symptom of the demyelinating disease.

Una aproximación actualizada hacia el tratamiento de la esclerosis múltiple / An updated approach to the treatment of multiple sclerosis

La presente es una revisión bibliográfica actualizada sobre el manejo de la Esclerosis Múltiple (EM), enfermedad neurológica progresiva de tipo desmielinizante más frecuente a nivel mundial. En Chile, su presentación remitente-recurrente (RRMS) es patología GES, por lo que se vuelve relevante para el médico general y estudiantes del área de la salud reconocer e identificar las terapias disponibles para el control de esta patología. Si bien la EM no es un cuadro frecuente, su sintomatología es alarmante e incapacitante, por lo que, con frecuencia, el primer acercamiento del paciente es a los servicios de urgencia, tornándose necesario contar con nociones básicas sobre el tratamiento y manejo. La presente revisión recopiló artículos publicados entre 2019 y 2023 de distintos motores de búsqueda con énfasis en el tratamiento farmacológico y no farmacológico de esta enfermedad. Además de describir el tratamiento convencional como la inmunomodulación, las terapias biológicas, el soporte con glucocorticoides y los fármacos remielinizantes, se abordan nuevas líneas de investigación prometedoras, como el rol inmunogénico de la microbiota intestinal, la capacidad epigenética de la dieta, estrategias de rehabilitación cognitiva y el potencial uso de cannabinoides para el manejo paliativo del dolor. Se concluye que un tratamiento oportuno con fármacos modificadores de la enfermedad, tanto de primera línea como de segunda, son imprescindibles para el manejo de la EM, sin embargo, la calidad de vida puede verse significativamente acrecentada por la incorporación de estrategias que se encuentran al alcance del médico general y que no requieren de derivación a nivel secundario.

This is an updated bibliographical review on the management of Multiple Sclerosis (MS), the most common progressive neurological disease of demyelinating disorders worldwide. In Chile, its relapsing-remitting presentation (RRMS) is a state-covered illness pathology, so it becomes relevant for the general practitioner and med students to recognize and identify therapies available for the control of this desease. Although MS is not a frequent condition, its symptoms are alarming and disabling, which is why, frequently, the first approach of the patient is to the emergency services, making it necessary to have basic knowledge about treatment and management. The present review compiled articles published between 2019 and 2023 from different search engines with an emphasis on the pharmacological and non-pharmacological treatment of the MS. In addition to describing conventional treatment such as immunomodulation, biological therapies, glucocorticoid support and remyelinating drugs, new promising lines of research are addressed, such as the immunogenic role of the intestinal microbiota, the epigenetic capacity of the diet, strategies on cognition rehabilitation and the potential use of cannabinoids for the palliative management of pain. It is concluded that the classic treatment with disease-modifying drugs, both first-line and second-line, are essential for the management of MS; however, quality of life can be significantly increased by incorporating strategies found at the reach of the general practitioner and do not require referral at a greater complexity center.

Impacto de la pandemia COVID-19 en pacientes con enfermedades desmielinizantes / IMPACT OF THE COVID-19 PANDEMIC ON PATIENTS WITH DEMYELINIZING DISEASES

Introducción: A más de un año del inicio de la pandemia, el seguimiento y la atención presencial de pacientes con enfermedades desmielinizantes se ha visto modificado. Según la evidencia, pacientes con diagnóstico de esclerosis múltiple (EM), síndrome desmielinizante aislado (SDA), Síndrome Radiológico Aislado (SRA) o enfermedades del espectro de neuromielitis óptica (NMO) no parecen ser un grupo de riesgo para COVID19 por el hecho de tener la enfermedad. La presencia de ciertas condiciones puede hacerlos susceptibles de cursar infección severa. Se ha descripto una asociación de curso grave con drogas anti CD20, faltan datos sobre la respuesta a vacunas COVID19 en esta población. Objetivos: Establecer características clínico-epidemiológicas de pacientes con enfermedades desmielinizantes que han padecido COVID-19 y describir su evolución. Caracterizar población vacunada, evaluar acceso al seguimiento médico/ terapéutico durante la pandemia. Materiales y métodos: Estudio observacional descriptivo. Se revisaron las historias clínicas de 168 pacientes con EM, SDA y SRA y 33 pacientes con NMO correspondientes al Hospital de Clínicas José de San Martin. Mediante encuesta telefónica se evaluó adherencia al tratamiento, evolución clínica, infección COVID-19, vacunación y acceso durante la pandemia. Resultados: Se encontraron 49 pacientes que desarrollaron COVID-19 en el grupo de pacientes con EM, y 7 en el grupo de NMO. Del primer grupo ninguno requirió internación, mientras que en el segundo, 2 fueron hospitalizados y uno de ellos falleció. La complicación post-COVID más frecuente fue: astenia prolongada y 3 pacientes presentaron un brote de la enfermedad de base en los 3 meses posteriores. Cerca del 90% de nuestra población ya contaba con al menos 1 dosis de vacuna para SARS-CoV2. Se interrogó sobre el acceso a la consulta neurológica y casi el 70% de los pacientes otorgó máximo puntaje al acceso a consultas virtuales. Conclusión: Los pacientes con enfermedades desmielinizantes que cursaron COVID-19 no tuvieron complicaciones severas por la infección, con solamente 2 pacientes cursando un brote en los 3 meses posteriores. No observamos reacciones adversas severas post vaccinales, ni infección posterior, sólo 2 pacientes presentaron un brote en el período post aplicación. Gran cantidad de pacientes percibieron acceso fluido a sus neurólogos de manera virtual, lo que podría relacionarse con alta tasa de adherencia a sus tratamientos a pesar de la limitación a la consulta presencial.

Introduction: More than a year after the start of the pandemic, the follow-up and face-to-face care of patients with demyelinating diseases has been modified. According to the evidence, patients with a diagnosis of multiple sclerosis (MS), isolated demyelinating syndrome (ADS), Isolated Radiological Syndrome (RAS) or neuromyelitis optica (NMO) spectrum diseases do not seem to be a risk group for COVID19 due to the fact that they have the disease. The presence of certain conditions can make them susceptible to severe infection. A severe course association with anti-CD20 drugs has been described, data on the response to COVID19 vaccines in this population are lacking. Objectives: To establish clinical-epidemiological characteristics of patients with demyelinating diseases who have suffered from COVID-19 and describe their evolution. Characterize the vaccinated population, evaluate access to medical/therapeutic follow-up during the pandemic. Materials and methods: Descriptive observational study. The medical records of 168 patients with MS, ADS and ARS and 33 patients with NMO corresponding to the Hospital de Clínicas José de San Martin were reviewed. Through a telephone survey, adherence to treatment, clinical evolution, COVID-19 infection, vaccination, and access during the pandemic were evaluated. Results: 49 patients who developed COVID-19 were found in the MS patient group, and 7 in the NMO group. Of the first group, none required hospitalization, unlike in the second, 2 were hospitalized and one of them died. The most frequent post-COVID complication was: prolonged asthenia and 3 patients presented an outbreak of the underlying disease in the following 3 months. Close to 90% of our population already had at least 1 dose of SARS-CoV2 vaccine. Access to the neurological consultation was questioned and almost 70% of the patients gave the highest score to access to virtual consultations. Conclusion: Patients with demyelinating diseases who had COVID-19 did not have severe complications from the infection, with only 2 patients having an outbreak in the subsequent 3 months. We did not observe severe post-vaccinal adverse reactions, nor subsequent infection, only 2 patients presented an outbreak in the post-application period. A large number of patients perceived fluid access to their neurologists virtually, which could be related to a high rate of adherence to their treatments despite the limitation to face-to-face consultation

4-hydroxy-2-nonenal as a marker of the oxidative stress in brains of dogs with canine distemper / 4-hidroxi-2-nonenal como marcador de estresse oxidativo no cérebro de cães com cinomose

Canine Distemper is a disease caused by Canine morbillivirus (CM), a pantropic virus that can affect the central nervous system (CNS), causing demyelination. However, the pathogenesis of this lesion remains to be clarified. Brain samples of 14 naturally infected dogs by CM were analyzed to evaluate the presence of oxidative stress and demyelination. RT-PCR assay was performed to confirm a diagnosis of canine distemper in the brain, immunohistochemistry anti-CM was used to localize the viral proteins in the tissue, and anti-4-hydroxy-2-nonenal (4-HNE) was a marker of a product of lipid peroxidation. The results showed the presence of viral proteins in the demyelinated area with the presence of 4-HNE. Our results suggest that the CM virus infection causes oxidative stress leading to lipid peroxidation, which causes tissue damage and demyelination. In conclusion, oxidative stress plays a significant role in canine distemper pathogenesis in the CNS.(AU)

A cinomose canina é uma doença causada pelo Morbilivírus canino (CM), um vírus pantrópico que pode afetar o sistema nervoso central (SNC), causando desmielinização. No entanto, a patogênese dessa lesão não está totalmente esclarecida. RT-PCR e imuno-histoquímica foram realizadas para confirmação do diagnóstico de cinomose em amostras de encéfalo de 14 cães naturalmente infectados. Após confirmação, foi realizada uma avaliação do estresse oxidativo por imuno-histoquímica com uso de anti-4-hidroxi-nonenal (4HNE) como marcador de produtos resultantes da peroxidação lipídica. Os resultados sugerem que a infecção pelo CM causa estresse oxidativo no tecido, levando a peroxidação lipídica, a qual causa danos ao tecido, culminando com desmielinização. Conclui-se que o estresse oxidativo tem papel importante na patogênese da cinomose canina no sistema nervoso central.(AU)

Research advances in the clinical genetics of leukodystrophy in children / 中国当代儿科杂志

Leukodystrophy (LD) is a group of genetic heterogeneous diseases characterized by primary abnormalities in glial cells and myelin sheath, and it is a common nervous system disease in children and has significant genotype-phenotype correlation. In recent years, the improvement in high-throughput sequencing has changed the diagnostic and therapeutic mode of LD, and elaborative phenotype analysis, such as the collection of natural history and multimodal neuroimaging evaluation during development, also provides important information for subsequent genetic diagnosis. This article reviews LD from the perspective of clinical genetics, in order to improve the awareness of this disease among pediatricians in China.

Vitamin D3 mediates spatial memory improvement through nitric oxide mechanism in demyelinated hippocampus of rat

Abstract Studies have revealed beneficial role of vitamin D3 in neuro-cognitive function. There is also supporting evidence on the involvement of nitric oxide (NO) in the neuro-protective action. However, its over production could contribute to brain disorders. In this study, demyelination was induced by ethidium bromide (EB) injection into the right side of the hippocampus area of male rats. Vitamin D3 was administered to rats for 7 and 28 days prior to behavioral experiments using Morris water maze (MWM). Travelled distance, time spent to reach the platform, and time spent in target zone, were considered for learning and spatial memory evaluation. Nitrite oxide (NO2-) concentration was measured as an indicator for nitric oxide production. The time spent to reach the platform and the travelled distance were decreased significantly by 28 days of vitamin D3 administration (compared to 7 days experiment). Time spent in target quadrant was significantly lowered by administered vitamin on day 28. Therefore, considering a number of studies that have shown the effect of vitamin D3 on cognition, these findings could support their potential effect. Besides, nitric oxide concentration significantly differed in 28 days of vitamin D3 treated group compared with the groups treated with EB or 7 days of vitamin D3.

Experiencia en la unidad de enfermedades desmielinizantes a 2 años / Experience in the demyelinating diseases unit after 2 years

La esclerosis múltiple es una enfermedad desmielinizante crónica que produce discapacidad progresiva, por lo que el tratamiento se centra en retrasar la progresión, prevenir recaídas y disminuir los síntomas de manera efectiva. Realizamos un estudio observacional, descriptivo, longitudinal, de un solo centro, con los pacientes admitidos en la unidad de enfermedades desmielinizantes, desde diciembre 2017 hasta febrero 2020. Del total de pacientes, 62.5% recibieron tratamiento con ocrelizumab y completaron seguimiento por 12 meses, sin progresión de la enfermedad. Con este estudio, resaltamos la importancia y la efectividad de los tratamientos modificadores de la enfermedad.

Multiple sclerosis is a chronic demyelinating disease that causes progressive disability, so treatment focuses on slowing progression, preventing relapses, and effectively reducing symptoms. We conducted an observational, descriptive, longitudinal, single-center study with patients admitted to the demyelinating diseases unit from December 2017 to February 2020. Of the total number of patients, 62.5% received treatment with ocrelizumab and completed 12-month follow-up, without disease progression. With this study, we highlight the importance and effectiveness of disease-modifying treatments

Serum YKL-40 levels in patients with multiple sclerosis / Níveis séricos de YKL-40 em pacientes com esclerose múltipla

ABSTRACT Background: Multiple sclerosis (MS) is a chronic inflammatory disease affecting the central nervous system. The YKL-40 protein, which is secreted from various cells that contribute to inflammation and infection, plays a role in immune regulation. Objective: This study investigated the serum YKL-40 levels of patients with clinically isolated syndrome (CIS) and MS. Methods: The participants was divided into three groups: 1) patients with CIS (n = 20); 2) patients with relapsing-remitting MS (RRMS; n = 39); and 3) healthy individuals (n = 35). The YKL-40 levels in serum samples obtained from the participants were measured using enzyme-linked immunoassays. Results: The median serum YKL-40 level was 20.2 ng/mL (range 9.8-75.9 ng/mL) in the patients with CIS, 22.7 ng/mL (range 13.4-57.9 ng/mL) in the patients with RRMS and 11.0 ng/mL (range 10.0-17.3 ng/mL) in the control group (p < 0.001). The serum YKL-40 levels in the patients with RRMS were correlated with the patients' expanded disability status scale scores and ages (p < 0.05). No relationships were determined between the serum YKL-40 levels and the other variables (p > 0.05). The serum YKL-40 levels were higher in the CIS group than in the MS group. These findings show that the serum YKL-40 levels were high even at the beginning of the disease. The serum YKL-40 levels were also not involved in the progression to clinically definite MS. Conclusions: The findings from this study suggested that YKL-40 may be a useful marker for the inflammatory process of MS.

RESUMO Contexto: A Esclerose Múltipla (EM) é uma doença inflamatória crônica que afeta o sistema nervoso central. A proteína UKL-40, secretada de várias células que participam de processos inflamatórios e infecciosos, desempenha um importante papel na regulação imunológica. Objetivo: Este estudo investigou níveis séricos de YKL-40 em pacientes com Síndrome Clinicamente Isolada (SCI) e EM. Métodos: Os participantes foram divididos em três grupos: 1) pacientes com SCI (n = 20); 2) pacientes com EM recorrente-remitente (EMRR; n = 39); e 3) indivíduos saudáveis (n = 35). Os níveis de YKL-40 em amostras séricas obtidas dos participantes foram medidos usando-se imunoensaios ligados a enzimas. Resultados: O nível sérico médio de YKL-40 foi 20.2 ng/mL (range 9.8-75.9 ng/mL) em pacientes com CIS, 22.7 ng/mL (intervalo entre 13.4-57.9 ng/mL) em pacientes com EMRR e 11.0 ng/mL (intervalo entre 10.0-17.3 ng/mL) no grupo controle (p < 0.001). Os níveis séricos de YKL-40 em pacientes com EMRR estavam correlacionados às pontuações e idades dos pacientes na EDSS (p < 0.05). Não foram determinadas relações entre os níveis séricos de YKL-40 e outras variáveis (p > 0.05). Os níveis séricos de YKL-40 no grupo SCI estavam mais elevados do que no grupo EM. Estes resultados demonstram que os níveis séricos de YKL-40 estavam mais elevados até mesmo no início da doença. Os níveis séricos de YKL-40 também não estavam associados à progressão da EM clinicamente definida. Conclusões: A partir deste estudo, os resultados sugeriram que a proteína YKL-40 pode ser um indicador útil no processo inflamatório da EM.

Caracterización epidemiológica, clínica e imagenológica de pacientes con esclerosis múltiple / Epidemiological, clinical and imaging characterization of patients with multiple sclerosis

RESUMEN Introducción: la esclerosis múltiple es una enfermedad desmielinizante, degenerativa, crónica, autoinmune e inflamatoria, que afecta al sistema nervioso central. Esta afección constituye la primera causa de invalidez neurológica en adultos jóvenes. Objetivo: caracterizar a los pacientes con diagnóstico de esclerosis múltiple ingresados en el servicio de Neurología del Hospital Universitario Clínico-Quirúrgico «Arnaldo Milián Castro¼. Métodos: se realizó un estudio descriptivo y transversal con los pacientes con diagnóstico de esclerosis múltiple ingresados en el servicio de Neurología del Hospital Universitario Clínico-Quirúrgico «Arnaldo Milián Castro¼, Santa Clara, Villa Clara, durante el período de enero 2018 - diciembre 2019; la población de estudio quedó constituida por 30 pacientes. Resultados: el promedio de edad de debut fue de 38,7 años. La relación entre pacientes con color de piel blanca y no blanca fue 9:1, y la de mujeres y hombres de 14:1. La esclerosis múltiple recidivante-remitente representó el 70 %, y el grado mínimo de discapacidad el 60 %. El 48,27 %, 41,37 % y 37,93 % de los pacientes presentaron lesiones supratentoriales, infratentoriales y en número de dos a cuatro, respectivamente. Conclusiones: predominó el sexo femenino con edad de debut entre los 20 a 29 años. Los síntomas más comunes fueron: las alteraciones motoras, sensitivas y cerebelosas. La forma clínica de presentación predominante fue la esclerosis múltiple recidivante-remitente y el grado de discapacidad mínimo. Las lesiones supratentoriales e infratentoriales y la cantidad de lesiones en número dos a cuatro fueron las más frecuentes; la mayoría de los casos presentó cambio de intensidad del cuerpo calloso.

ABSTRACT Introduction: multiple sclerosis is a demyelinating, degenerative, chronic, autoimmune and inflammatory disease that affects the central nervous system. This condition is the leading cause of neurological disability in young adults. Objective: to characterize patients with a diagnosis of multiple sclerosis admitted to the Neurology service at "Arnaldo Milián Castro" Clinical and Surgical University Hospital. Methods: a descriptive and cross-sectional study was carried out in patients with a diagnosis of multiple sclerosis admitted to the Neurology Service at "Arnaldo Milián Castro" Clinical and Surgical University Hospital, Santa Clara, Villa Clara, from January 2018 to December 2019; the study population consisted of 30 patients. Results: the average age at debut was 38.7 years. The ratio between patients with white and non-white skin color was 9: 1, and that of women and men was 14: 1. Relapsing-remitting multiple sclerosis accounted for 70%, and the minimum degree of disability for 60%. The 48.27%, 41.37% and 37.93% of the patients had supratentorial and infratentorial lesions and two to four in number, respectively. Conclusions: female gender predominated with the age at debut between 20 to 29 years. Motor, sensory and cerebellar alterations were the most common symptoms. Relapsing-remitting multiple sclerosis was the predominant clinical presentation and minimal degree of disability. Supratentorial and infratentorial lesions and the number of lesions in number two to four were the most frequent; most of the cases had a change in intensity of the corpus callosum.

Eficacia de la movilización de células madre en pacientes con Polineuropatía Desmielinizante Inflamatoria Crónica refractaria / Efficacy of mobilization of stem cells in patients with Refractory Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Introducción: La polineuropatía desmielinizante inflamatoria crónica (CIDP) es una enfermedad desmielinizante e inflamatoria de mediación autoinmune. El tratamiento convencional es basado en la inmunomodulación e inmunosupresión. El uso de células madre es una terapia novedosa en los trastornos autoinmune, siendo incluida como terapia. Objetivo: Determinar la eficacia de la movilización de células madre mediante la aplicación del factor estimulador de colonias granulocíticas (F-ECG) en pacientes con CIDP que han recibido otras líneas de tratamiento. Material y Métodos: Se realizó un estudio aleatorizado, doble ciego sobre una cohorte de 45 pacientes con CIDP, donde se administró el (F-ECG) en 25 pacientes y 20 continuaron con el tratamiento habitual, tratados anteriormente con otras variantes terapéuticas por más de tres años, sin respuesta satisfactoria. Resultados: Predominio de los hombres para 64,4 por ciento, la Diabetes Mellitus tuvo mayor asociación y la medicación más usada fueron los esteroides. Los síntomas y signos clínicos mejoraron significativamente tras el tratamiento. Los valores de la puntuación del TCSS al mes y 3 meses después del tratamiento disminuyeron significativamente; pero este decremento no se mantuvo al final del estudio. La velocidad de conducción y el potencial de acción de los nervios sensoriales y motores mejoraron considerablemente después del tratamiento. Conclusiones: La efectividad de la aplicación del (F-ECG) para la mejoría de los síntomas clínicos y resultados de estudios neurofisiológicos evolutivamente son mayores que otras variantes terapéuticas en los primeros meses, con buena seguridad y tolerabilidad, por lo que se puede incluir en la terapéutica convencional para la CIDP(AU)

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune demyelinating disease. Conventional treatment is based on immunomodulation and immunosuppression. The use of stem cells is a novel therapy in autoimmune disorders, so it is included as therapy. Objective: To determine the efficacy of mobilization of stem cells by applying granulocyte colony-stimulating factor (G-CSF) in patients with CIDP who have followed other lines of treatment. Material and Methods: A randomized, double-blind study was carried out on a cohort of 45 patients with CIDP. G-CSF was administered to 25 patients and 20 of them continued with the usual treatment. These patients were previously treated with other therapeutic variants for more than three years without satisfactory response. Results: There was a prevalence of men (64.4 percent), Diabetes Mellitus had a greater association, and the most used medications were steroids. Clinical symptoms and signs improved significantly after treatment. TCSS scores significantly decreased at one and three months after treatment, but this decrease was not maintained at the end of the study. The conduction velocity and action potential of sensory and motor nerves improved considerably after treatment. Conclusions: The effectiveness of the use of G-CSF shows an improvement of clinical symptoms. The results of neurophysiological studies have a better course than other therapeutic variants during the first months, with good safety and tolerability, so it can be included in the conventional therapy for the CIDP(AU)

Corticosteroid-associated changes resembling a demyelinating brain lesion in Diffuse Large B-cell Lymphoma (DLBCL): A case report

@#Biopsies of suspected lymphoma cases with history of pre-biopsy corticosteroid therapy present several diagnostic issues, such as the inability to demonstrate the neoplastic hematolymphoid cells, the similarity of post-corticosteroid changes with inflammatory demyelinating lesions, and the possibility of a demyelinating lesion preceding a central nervous system lymphoma. This report presents the case of a 51-year-old immunocompetent male with a solitary callosal mass, with immunomorphologic features suggestive of a demyelinating lesion on initial biopsy, and upon re-biopsy after three months revealed a diffuse large B-cell lymphoma. Awareness of these issues in post-corticosteroid stereotactic biopsy specimens, together with adequate clinical and radiologic data, is important for proper diagnosis and further therapeutic guidance.

Medicamentos modificadores en esclerosis múltiple: esquemas terapéuticos actuales / Disease-Modifying drugs in multiple sclerosis: current therapeutic schemes

La esclerosis múltiple (EM) es una enfermedad crónica, autoinmune y neurodegenerativa; que tiene como principal característica la desmielinización de los axones en el sistema nervioso. Los medicamentos modificadores de la enfermedad (MME) logran retrasar la aparición de los síntomas y modificar parcialmente el progreso de la desmielinización y daño neuronal, resultando cada vez más complejo determinar un esquema terapéutico estandarizado según la condición particular de cada paciente. En este artículo se presenta una revisión actualizada de la evidencia clínica que ha llevado al uso de los esquemas terapéuticos en EM. La mayoría de los medicamentos aprobados actualmente son utilizados para la EM remitente-recurrente y se pueden dividir de acuerdo a la eficacia y seguridad. Los medicamentos de primera línea han mostrado una baja o moderada eficacia y alta seguridad; después de usar estos fármacos sin lograr una buena respuesta o ante una enfermedad avanzada se usan medicamentos de segunda y tercera línea que tienen una alta eficacia, pero son menos seguros, presentando mayores efectos secundarios y riesgos asociados para los pacientes. El ocrelizumab es el único fármaco aceptado para la EM primaria progresiva y el siponimod fue aprobado como una alternativa para la EM secundaria progresiva. El desarrollo de nuevos medicamentos y el seguimiento clínico de los ya aprobados permitirá establecer un mejor abordaje terapéutico logrando así mejorar la calidad de vida de cada paciente.

Multiple sclerosis (MS) is a chronic, autoimmune and neurodegenerative disease; whose main characteristic is the demyelination of axons in the nervous system. Disease-modifying drugs (DMD) can delay the onset of symptoms and partially modify the progression of demyelination and neuronal damage, making it increasingly complex to determine a standardized therapeutic scheme that is individualized to each patient. This article presents an updated review on the clinical evidence that has led to the use of current therapeutic schemes in MS with focus on DMD. Current medications in treating relapsing-remitting MS can be divided according to efficacy and safety. First-line drugs have shown low or moderate efficacy and high safety. Second- and third-line drugs are used after a poor response or in cases of advanced disease. These drugs are highly effective, but less safe, presenting greater side effects and associated risks for patients. Ocrelizumab is the only accepted drug for primary progressive MS and siponimod is accepted as an alternative for secondary progressive MS. The development of new medications and the clinical follow-up of those already approved will allow establishing a better therapeutic approach, thus improving the quality of life of each patient.

Eficacia de la rehabilitación cognitiva en un paciente con esclerosis múltiple progresiva primaria / Efficacy of cognitive rehabilitation in a patient with primary progressive multiple sclerosis

La esclerosis múltiple (EM) es una enfermedad inflamatoria crónica que cursa con la desmielinización y la neurodegeneración a nivel del sistema nervioso central. Existen tres tipos de EM en función de la progresión de la enfermedad, pero la mayor parte de los pacientes tienden a presentar déficits cognitivos. Por lo tanto, resulta imprescindible el desarrollo de programas de entrenamiento cognitivos dirigidos a la mejora de estos déficits y, en definitiva, a la mejora de la calidad de vida de estos pacientes. En este sentido, el objetivo principal de este estudio fue la puesta en marcha de un programa de entrenamiento cognitivo dirigido a un paciente con esclerosis múltiple progresiva primaria (EMPP) a lo largo de un año. Los resultados pusieron de manifiesto que algunos de los déficits cognitivos que presentó inicialmente el paciente mejoraron tras varios meses de intervención. En este sentido, el paciente presentó notables mejoras en el control inhibitorio y la flexibilidad cognitiva. No obstante, los déficits en la velocidad de procesamiento se mantuvieron constantes a lo largo de toda la intervención. Asimismo, aparecieron otros déficits a lo largo de la intervención que remitieron tras la adecuación de los objetivos de intervención. Por todo ello, nuestro estudio reforzó la importancia de la puesta en marcha de los programas de rehabilitación cognitiva dirigidos a pacientes con enfermedades desmielinizantes para paliar las secuelas cognitivas derivadas de las mismas. Además, es importante que estos programas de entrenamiento cognitivo sean revisados periódicamente para adecuar los objetivos del tratamiento.

Multiple sclerosis (MS) is a chronic inflammatory disease that involves demyelination and neurodegeneration at the level of the central nervous system. Despite the different characteristics of each of the three types of MS, most patients with this disease present significant cognitive deficits. Therefore, it is essential to develop cognitive training programs to improve these deficits and, ultimately, increase the quality of life of these patients. Thus, the main objective of this study was to implement a one-year cognitive training program with a patient with progressive primary multiple sclerosis (PPMS). The results showed that some of the cognitive deficits the patient initially presented improved after several months of intervention. In this regard, the patient presented noteworthy improvements in inhibitory control and cognitive flexibility. However, deficits in processing speed remained constant throughout the intervention. Likewise, other deficits appeared during the intervention that remitted after adapting the intervention objectives to the patient's needs. Therefore, our study reinforces the importance of implementing cognitive rehabilitation programs for patients with demyelinating diseases to alleviate the cognitive sequelae they produce. In addition, it is important to evaluate these cognitive training programs periodically in order to adapt the objectives and improve the patient's functionality.