Recomendaciones para la coordinación de los servicios de Neurología y Neurorradiología en la atención a pacientes con esclerosis múltiple / Recommendations for the coordination of Neurology and Neuroradiology Departments in the management of patients with multiple sclerosis

Introducción: El uso de la resonancia magnética (RM) está ampliamente extendido en el diagnóstico y el seguimiento de los pacientes con esclerosis múltiple (EM). La coordinación entre los servicios de Neurología y Neurorradiología es clave para la realización e interpretación de estudios radiológicos de la manera más eficaz posible. Sin embargo, esta coordinación es susceptible de mejoras en una gran parte de los hospitales nacionales. Métodos: Un panel de 17 neurólogos y neurorradiólogos de 8 hospitales españoles, presencialmente y a través de comunicación online, consensuaron una guía de buenas prácticas en la coordinación en EM. La guía se estableció en 4 fases: 1) definición del alcance de la guía y metodología del estudio; 2) revisión bibliográfica sobre buenas prácticas o recomendaciones en el uso de la RM en EM; 3) discusión y búsqueda de consenso entre los expertos; y 4) formalización y validación de los contenidos para elaborar el documento de consenso. Resultados: Se consensuaron un total de 9 recomendaciones dirigidas a la mejora de la coordinación entre los servicios de Neurología y Neurorradiología, que se pueden resumir en: 1) estandarizar las solicitudes de RM, informes y planificación; 2) crear protocolos compartidos para los estudios de RM; 3) establecer comités multidisciplinares y sesiones de coordinación, y 4) generar canales de comunicación formales entre los profesionales de ambos departamentos. Conclusiones: Se espera que las recomendaciones consensuadas sirvan de guía para optimizar la coordinación entre neurólogos y neurorradiólogos y que repercutan en la mejora del diagnóstico y seguimiento de los pacientes con EM.(AU)

Introduction: Magnetic resonance imaging (MRI) is widely used for the diagnosis and follow-up of patients with multiple sclerosis (MS). Coordination between Neurology and Neuroradiology departments is crucial for performing and interpreting radiological studies as efficiently and as accurately as possible. However, improvements can be made in the communication between these departments in many Spanish hospitals. Methods: A panel of 17 neurologists and neuroradiologists from 8 Spanish hospitals held in-person and online meetings to draft a series of good practice guidelines for the coordinated management of MS. The drafting process included 4 phases: 1) establishing the scope of the guidelines and the methodology of the study; 2) literature review on good practices or recommendations on the use of MRI in MS; 3) discussion and consensus between experts; and 4) validation of the contents. Results: The expert panel agreed a total of 9 recommendations for improving coordination between neurology and neuroradiology departments. The recommendations revolve around 4 main pillars: 1) standardising the process for requesting and scheduling MRI studies and reports; 2) designing common protocols for MRI studies; 3) establishing multidisciplinary committees and coordination meetings; and 4) creating formal communication channels between both departments. Conclusions: These consensus recommendations are intended to optimise coordination between neurologists and neuroradiologists, with the ultimate goal of improving the diagnosis and follow-up of patients with MS.(AU)

[15th Post-ECTRIMS Meeting: a review of the latest developments presented at the 2022 ECTRIMS Congress (Part II)]. / XV Reunión Post-ECTRIMS: revisión de las novedades presentadas en el Congreso ECTRIMS 2022 (II).

INTRODUCTION: On 4 and 5 November 2022, Madrid hosted the 15th edition of the Post-ECTRIMS Meeting, where neurologists specialised in multiple sclerosis outlined the latest developments presented at the 2022 ECTRIMS Congress, held in Amsterdam from 26 to 28 October. AIM: To synthesise the content presented at the 15th edition of the Post-ECTRIMS Meeting, in an article broken down into two parts. DEVELOPMENT: This second part describes the new developments in terms of therapeutic strategies for escalation and de-escalation of disease-modifying therapies (DMT), when and in whom to initiate or switch to highly effective DMT, the definition of therapeutic failure, the possibility of treating radiologically isolated syndrome and the future of personalised treatment and precision medicine. It also considers the efficacy and safety of autologous haematopoietic stem cell transplantation, different approaches in clinical trial design and outcome measures to assess DMT in progressive stages, challenges in the diagnosis and treatment of cognitive impairment, and treatment in special situations (pregnancy, comorbidity and the elderly). In addition, results from some of the latest studies with oral cladribine and evobrutinib presented at ECTRIMS 2022 are shown.

TITLE: XV Reunión Post-ECTRIMS: revisión de las novedades presentadas en el Congreso ECTRIMS 2022 (II).Introducción. El 4 y el 5 de noviembre se celebró en Madrid la Reunión Post-ECTRIMS, en la que neurólogos expertos en esclerosis múltiple resumieron las principales novedades presentadas en el congreso de ECTRIMS 2022, celebrado entre el 26 y el 28 de octubre en Ámsterdam. Objetivo. Sintetizar las ponencias que tuvieron lugar en la Reunión Post-ECTRIMS, en un artículo desglosado en dos partes. Desarrollo. En esta segunda parte, se presentan las novedades sobre las estrategias terapéuticas de escalado y desescalado de los tratamientos modificadores de la enfermedad (TME), cuándo y a quién iniciar o cambiar a TME de alta eficacia, la definición de fracaso terapéutico, la posibilidad de tratar el síndrome radiológico asilado, el futuro del tratamiento personalizado y la medicina de precisión, la eficacia y seguridad del autotrasplante de células madre hematopoyéticas, diferentes aproximaciones en el diseño de ensayos clínicos y en las medidas de resultados para evaluar TME en fases progresivas, retos en el diagnóstico y tratamiento del deterioro cognitivo, y tratamiento en situaciones especiales (embarazo, comorbilidad y personas mayores). Además, se muestran los resultados de algunos de los últimos estudios realizados con cladribina oral y evobrutinib presentados en el ECTRIMS 2022.

[15th Post-ECTRIMS Meeting: a review of the latest developments presented at the 2022 ECTRIMS Congress (Part I)]. / XV Reunión Post-ECTRIMS: revisión de las novedades presentadas en el Congreso ECTRIMS 2022 (I).

INTRODUCTION: On 4 and 5 November 2022, Madrid hosted the 15th edition of the Post-ECTRIMS Meeting, where neurologists specialised in multiple sclerosis (MS) outlined the most relevant novelties presented at the 2022 ECTRIMS Congress, held in Amsterdam from 26 to 28 October. AIM: To synthesise the content presented at the 15th edition of the Post-ECTRIMS Meeting, in an article broken down into two parts. DEVELOPMENT: In this first part, the initial events involved in the onset of MS, the role played by lymphocytes and the migration of immune system cells into the central nervous system are presented. It describes emerging biomarkers in body fluids and imaging findings that are predictive of disease progression and useful in the differential diagnosis of MS. It also discusses advances in imaging techniques which, together with a better understanding of the agents involved in demyelination and remyelination processes, provide a basis for dealing with remyelination in the clinical setting. Finally, the mechanisms triggering the inflammatory reaction and neurodegeneration involved in MS pathology are reviewed.

TITLE: XV Reunión Post-ECTRIMS: revisión de las novedades presentadas en el Congreso ECTRIMS 2022 (I).Introducción. El 4 y el 5 de noviembre se celebró en Madrid la XV edición de la Reunión Post-ECTRIMS, donde neurólogos expertos en esclerosis múltiple (EM) resumieron las principales novedades presentadas en el congreso de ECTRIMS 2022, celebrado en Ámsterdam entre el 26 y el 28 de octubre. Objetivo. Sintetizar las ponencias que tuvieron lugar en la Reunión Post-ECTRIMS, en un artículo desglosado en dos partes. Desarrollo. En esta primera parte se presentan los primeros eventos involucrados en el inicio de la EM, la implicación de los linfocitos y la migración de células del sistema inmunitario hacia el sistema nervioso central. Se describen los biomarcadores emergentes en fluidos corporales y los hallazgos de imagen que permiten predecir la evolución de la enfermedad, y que resultan útiles en el diagnóstico diferencial de la EM. También se exponen los avances en las técnicas de imagen que, junto con un mayor conocimiento de los agentes involucrados en los procesos de desmielinización y remielinización, proporcionan una base para abordar la remielinización en el entorno clínico. Por último, se repasan los mecanismos desencadenantes de la reacción inflamatoria y la neurodegeneración implicados en la patología de la EM.

Recommendations for the coordination of Neurology and Neuroradiology Departments in the management of patients with multiple sclerosis.

INTRODUCTION: Magnetic resonance imaging (MRI) is widely used for the diagnosis and follow-up of patients with multiple sclerosis (MS). Coordination between neurology and neuroradiology departments is crucial for performing and interpreting radiological studies as efficiently and as accurately as possible. However, improvements can be made in the communication between these departments in many Spanish hospitals. METHODS: A panel of 17 neurologists and neuroradiologists from 8 Spanish hospitals held in-person and online meetings to draft a series of good practice guidelines for the coordinated management of MS. The drafting process included 4 phases: 1) establishing the scope of the guidelines and the methodology of the study; 2) literature review on good practices or recommendations on the use of MRI in MS; 3) discussion and consensus between experts; and 4) validation of the contents. RESULTS: The expert panel agreed a total of 9 recommendations for improving coordination between neurology and neuroradiology departments. The recommendations revolve around 4 main pillars: 1) standardising the process for requesting and scheduling MRI studies and reports; 2) designing common protocols for MRI studies; 3) establishing multidisciplinary committees and coordination meetings; and 4) creating formal communication channels between both departments. CONCLUSIONS: These consensus recommendations are intended to optimise coordination between neurologists and neuroradiologists, with the ultimate goal of improving the diagnosis and follow-up of patients with MS.

13th Post-ECTRIMS Meeting: review of the new developments presented at the 2020 ECTRIMS Congress (II). / XIII Reunión Post-ECTRIMS: revisión de las novedades presentadas en el Congreso ECTRIMS 2020 (II).

INTRODUCTION: For more than a decade, after the ECTRIMS Congress, Spain has hosted the Post-ECTRIMS meeting, where neurologists with expertise in multiple sclerosis (MS) meet to review the new developments presented at the ECTRIMS. AIM: This article, published in two parts, summarises the presentations of the post-ECTRIMS meeting, held online on 16 and 17 October 2020. DEVELOPMENT: This second part highlights the importance of gender and age in understanding the pathology of the disease and optimising its management. The advances made in paediatric MS, from a neuropsychological and neuroimaging point of view, are presented. In turn, special attention is paid to the findings that contribute to a more personalised approach to therapy and to choosing the best treatment strategy (pharmacological and non-pharmacological) for each patient. Similarly, results related to possible strategies to promote remyelination are addressed. Although there are no major advances in the treatment of progressive forms, some quantitative methods for the classification of these patients are highlighted. In addition, the study also includes results on potential tools for assessment and treatment of cognitive deficits, and some relevant aspects observed in the spectrum of neuromyelitis optica disorders. Finally, the results of the papers considered as breaking news at the ECTRIMS-ACTRIMS are detailed. CONCLUSIONS: Most of the advances presented were related to the knowledge of paediatric MS, remyelination strategies and cognitive assessment in MS.

TITLE: XIII Reunión Post-ECTRIMS: revisión de las novedades presentadas en el Congreso ECTRIMS 2020 (II).Introducción. Desde hace más de una década, tras el Congreso ECTRIMS, se celebra en España la reunión post-ECTRIMS, donde neurólogos expertos en esclerosis múltiple (EM) se reúnen para revisar las novedades presentadas en el ECTRIMS. Objetivo. En el presente artículo, publicado en dos partes, se resumen las ponencias de la reunión post-ECTRIMS, celebrada los días 16 y 17 de octubre de 2020 virtualmente. Desarrollo. En esta segunda parte se destaca la importancia del género y la edad en la compresión de la patología de la enfermedad y la optimización de su manejo. Se exponen los avances realizados en la EM pediátrica desde un punto de vista neuropsicológico y de neuroimagen. Por su parte, cobran especial protagonismo los hallazgos que contribuyen a realizar un enfoque del tratamiento más personalizado y a elegir la mejor estrategia de tratamiento (farmacológica y no farmacológica) para cada paciente. De igual forma, se abordan los resultados relacionados con las estrategias posibles que promuevan la remielinización. Aunque no hay grandes avances en el tratamiento de formas progresivas, se destacan algunos métodos cuantitativos para la clasificación de estos pacientes. Además, se incluyen los resultados sobre herramientas potenciales de evaluación y tratamiento de los déficits cognitivos, y algunos aspectos relevantes observados en el espectro de los trastornos de la neuromielitis óptica. Por último, se detallan los resultados de las ponencias consideradas como noticias de última hora en el ECTRIMS-ACTRIMS. Conclusiones. Se presentaron avances principalmente sobre el conocimiento de la EM pediátrica, las estrategias de remielinización y la evaluación cognitiva en la EM.

13th Post-ECTRIMS Meeting: review of the new developments presented at the 2020 ECTRIMS Congress (I). / XIII Reunión Post-ECTRIMS: revisión de las novedades presentadas en el Congreso ECTRIMS 2020 (I).

INTRODUCTION: For more than a decade, following the ECTRIMS Congress, the Post-ECTRIMS Meeting has been held in Spain, where neurologists with expertise in multiple sclerosis (MS) from all over the country meet to review the most relevant latest developments presented at the ECTRIMS congress (on this occasion held together with ACTRIMS). AIM: This article, published in two parts, summarises the presentations that took place at the Post-ECTRIMS Meeting, held online on 16 and 17 October 2020. DEVELOPMENT: This first part includes the latest results regarding the impact of the environment and lifestyle on risk of MS and its clinical course, and the role of epigenetics and genetic factors on these processes. Findings from preclinical and clinical research on the lymphocyte subtypes identified and the involvement of lymphoid follicles and meningeal involvement in the disease are discussed. Changes in brain structure are addressed at the microscopic and macroscopic levels, including results from high-resolution imaging techniques. The latest advances on biomarkers for the diagnosis and prognosis of MS, and on the involvement of the microbiome in these patients are also reported. Finally, results from patient registries on the impact of COVID-19 in MS patients are outlined. CONCLUSIONS: There have been new data on MS risk factors, the impact of MS at the cellular and structural level, the role of the microbiome in the disease, biomarkers, and the relationship between COVID-19 and MS.

TITLE: XIII Reunión Post-ECTRIMS: revisión de las novedades presentadas en el Congreso ECTRIMS 2020 (I).Introducción. Desde hace más de una década, tras el congreso ECTRIMS, se celebra en España la reunión Post-ECTRIMS, donde neurólogos expertos en esclerosis múltiple (EM) de toda España se reúnen para revisar las principales novedades presentadas en el ECTRIMS (en esta ocasión, celebrado junto con el ACTRIMS). Objetivo. En el presente artículo, publicado en dos partes, se resumen las ponencias que tuvieron lugar en la reunión Post-ECTRIMS, celebrada los días 16 y 17 de octubre de 2020 de forma virtual. Desarrollo. En esta primera parte se incluyen los últimos resultados acerca del impacto del ambiente y el estilo de vida sobre el riesgo de EM y su curso clínico, y el papel de la epigenética y los factores genéticos sobre estos procesos. Se discuten los hallazgos en investigación preclínica y clínica sobre los subtipos de linfocitos identificados, y la implicación de los folículos linfoides y la afectación meníngea en la enfermedad. Los cambios en la estructura cerebral se abordan a nivel microscópico y macroscópico, incluyendo resultados de técnicas de imagen de alta resolución. También se presentan los últimos avances sobre biomarcadores para el diagnóstico y el pronóstico de la EM, y sobre la afectación del microbioma en estos pacientes. Por último, se esbozan los resultados de registros de pacientes sobre el impacto de la COVID-19 en los pacientes con EM. Conclusiones. Ha habido nuevos datos sobre factores de riesgo de la EM, impacto de la EM a nivel celular y estructural, papel del microbioma en la enfermedad, biomarcadores y la relación entre COVID-19 y EM.

Recommendations for the coordination of Neurology and Neuroradiology Departments in the management of patients with multiple sclerosis. / Recomendaciones para la coordinación de los servicios de Neurología y Neurorradiología en la atención a pacientes con esclerosis múltiple.

INTRODUCTION: Magnetic resonance imaging (MRI) is widely used for the diagnosis and follow-up of patients with multiple sclerosis (MS). Coordination between Neurology and Neuroradiology departments is crucial for performing and interpreting radiological studies as efficiently and as accurately as possible. However, improvements can be made in the communication between these departments in many Spanish hospitals. METHODS: A panel of 17 neurologists and neuroradiologists from 8 Spanish hospitals held in-person and online meetings to draft a series of good practice guidelines for the coordinated management of MS. The drafting process included 4 phases: 1) establishing the scope of the guidelines and the methodology of the study; 2) literature review on good practices or recommendations on the use of MRI in MS; 3) discussion and consensus between experts; and 4) validation of the contents. RESULTS: The expert panel agreed a total of 9 recommendations for improving coordination between neurology and neuroradiology departments. The recommendations revolve around 4 main pillars: 1) standardising the process for requesting and scheduling MRI studies and reports; 2) designing common protocols for MRI studies; 3) establishing multidisciplinary committees and coordination meetings; and 4) creating formal communication channels between both departments. CONCLUSIONS: These consensus recommendations are intended to optimise coordination between neurologists and neuroradiologists, with the ultimate goal of improving the diagnosis and follow-up of patients with MS.

Early diffuse demyelinating lesion in the cervical spinal cord predicts a worse prognosis in relapsing-remitting multiple sclerosis.

OBJECTIVE: To study the long-term outcome and persistence of two patterns of cervical spinal cord abnormality in early relapsing-remitting multiple sclerosis (RRMS). METHODS: RRMS patients with a spinal cord MRI performed during the first 3 years of the disease, a control MRI 5 years later and who have been followed up at least 10 years were included. Patients were grouped according the T2 spinal cord MRI into: (A) nodular pattern, if one or more focal lesions were present; and (B) diffuse pattern, defined as a poorly demarcated high signal area. The end point was defined as the time to reach an Expanded Disability Status Score (EDSS) of 4.0. RESULTS: Twenty-five patients were included; 12 in group A and 13 in group B. Three patients in group A and 9 in group B reached EDSS 4, in a mean time of 11 years in group A and 7 years in group B (log rank 10.3, p = 0.001). Multivariate Cox regression analysis assessing the risk of EDSS 4.0 including sex, age, number of relapses in the first 2 years, number of T2 brain lesions and spinal cord pattern showed higher risk for the diffuse pattern (hazard ratio 7.2, 95% confidence interval 1.4-36.4). Control MRI showed the persistence of the diffuse pattern in all patients, and the development of diffuse pattern in two patients with basal nodular lesions. CONCLUSIONS: The diffuse abnormality in cervical spinal cord at the beginning of the disease is persistent and predicts a worse prognosis in RRMS patients.

Effect of relapses over early progression of disability in multiple sclerosis patients treated with beta-interferon.

Observational study designed to explore the effect of demographical variables and number of relapses over the disability progression in the two first years of beta-interferon treatment for multiple sclerosis. One hundred and sixty two patients treated with beta-interferon for at least two years were included, 70.9% females, mean age 33.4 years, mean disease duration 75.1 months, mean EDSS 2.4, previous year relapse rate 1.3. Main end-point was defined as a sustained EDSS increase (1.5 if previous EDSS 0-2.0; 1.0 if previous EDSS 2.5-4.0; 0.5 if previous EDSS 4.5 or higher). 62.3% of patients presented one or more relapses and 32.7% patients reached sustained disability increase. The univariate and multivariate Cox regression analysis only showed statistical significance for the relapses in the two first years after the treatment (HR 1 relapse: 3.4, p = 0.05; HR > or = 2 relapses: 4.3, p < 0.001). The Kaplan-Meier survival analysis showed a higher probability of EDSS progression for patients with one relapse (log rank 10.9, p = 0.02) and with > or = 2 relapses (log rank 17.7, p < 0.001), with no differences between them (p = 0.38). In conclusion, patients with one or more relapses in the first two years of interferon treatment developed an earlier sustained progression of the disability.

High clinical inflammatory activity prior to the development of secondary progression: a prospective 5-year follow-up study.

OBJECTIVE: To study if there are different patterns of clinical activity--measured by the annual exacerbation rate (AER)--among relapsing-remitting multiple sderosis (RRMS), "early" secondary multiple sclerosis (SPMS) and "late" SPMS. METHODS: A prospective 5-year follow-up study in 80 MS patients has been carried out, calculating the AER and the mean expanded disability status scale (EDSS) change rate (MCR). RESULTS: A significant difference on the AER, among RRMS, early SPMS and late SPMS, has been found. CONCLUSIONS: The SPMS has a high clinical inflammatory activity before and during its transformation from a RRMS.

Deterioro cognitivo, formas clínicas y progresión en esclerosis múltiple / Cognitive impairment, clinic forms and progression in multiple sclerosis

Introducción. La esclerosis múltiple es una enfermedad desmielinizante del sistema nervioso central, de etiología desconocida y patogenia autoinmune. Se caracteriza por la destrucción de la mielina, el fallo en su reparación y por un grado variable de lesión axonal. Desarrollo. A pesar de las nuevas técnicas de neuroimagen, hoy por hoy, el diagnóstico sigue basándose en criterios clínicos que demuestran una diseminación espacial y temporal de las lesiones. La principal `herramienta' para la evaluación clínica de los pacientes es la escala del estado de incapacidad ampliada de Kurtzke (EDSS) basada en el análisis combinado de ocho sistemas funcionales, de los cuales sólo uno hace referencia a las funciones cognitivas. Charcot ya apreció que los enfermos de esclerosis múltiple acaban padeciendo una grave afectación de la memoria, pero este es un aspecto no suficientemente valorado. Actualmente se intenta tipificar el trastorno cognitivo con baterías de pruebas neuropsicológicas sensibles. Asimismo, se buscan correlaciones entre el grado de deterioro cognitivo y distintas variables de laboratorio, como pueden ser las lesiones objetivadas mediante resonancia magnética, las latencias y amplitudes del potencial P300, la cartografía electroencefalográfica, etc., que permiten, a su vez, relacionar distintos grados de afectación mental con las distintas formas evolutivas de la esclerosis múltiple, así como con la progresión de la misma (AU)

[Cognitive impairment, clinic forms and progression in multiple sclerosis].

INTRODUCTION: Multiple sclerosis-related cognitive impairment is an important clinical dimension of the disease by virtue of its prevalence and its functional significance. It is directly related to cerebral multiple sclerosis pathology, but relatively independent of physical impairment. DEVELOPMENT: Thus, the assessment of neuropsychologic outcomes complements the assessment of physical outcomes in multiples sclerosis clinical trials. Neuropsychologic measures fulfill most of the criteria identified for an optimal multiple sclerosis clinical outcome measure. Major drawbacks to their current use in assessing clinical trial outcomes are the heterogeneity inherent in neuropsychological test performance(both cross-sectionally and longitudinally), and limitations in our knowledge about the evolution of multiple sclerosis- related cognitive impairment and the psychometric properties of specific measures over time. At this point in time, we cannot identify the single "best" measure for assessing neuropsychologic change in multiple sclerosis patients, and we cannot specify what constitutes clinically significant change. Analyses of neuropsychologic data from recently completed trial will soon provide an empirical basis for selecting measures to use in future multiple sclerosis clinical trials and specifying criteria for clinically significant change on these measures.

[A study of various scales of fatigue and impact on the quality of life among patients with multiple sclerosis]. / Estudio de diversas escalas de fatiga e impacto en la calidad de vida de los pacientes afectos de esclerosis múltiple.

OBJECTIVES: To explore fatigue in multiple sclerosis and evaluate the specificity of three fatigue scales in this condition: the fatigue severity scale, the specific fatigue scale and the fatigue impact scale. MATERIAL AND METHODS: We sent out 60 questionnaires with the three scales and the quality of life scale, the Nottingham Health Profile, to patients with multiple sclerosis as clinically defined by Poser's criteria. Answers were received to 58 questionnaires and the data correlated by Sperman's correlation and the Student t test, with demographic variables (age, age of onset and sex) and clinical variables (clinical form, time the disease was present, period of time since the previous episode and Kurtzke scale (EDSS). RESULTS: Fatigue was present in 78% of the patients. There was correlation between fatigue severity scale and EDSS, pyramidal function, cerebellar function, the period of time the illness was present and the clinical form. We found that the specific fatigue scale is independent of EDSS. The fatigue impact scale was correlated with the EDSS apart from the questions concerning cognitive function. CONCLUSIONS: Fatigue is a common symptom of multiple sclerosis which has an independent effect on cognitive function. It is also related to involvement of the pyramidal and cerebellar systems, and depends on the degree of disability and time the disease has been present. The specific fatigue scale is a good tool for exploration of this symptom of multiple sclerosis.

[Axonal involvement in multiple sclerosis. Current concepts]. / Afectación axonal en la esclerosis múltiple. Conceptos actuales.

INTRODUCTION: Axon pathology in multiple sclerosis is an emerging concept, not because it is unknown but because it has been forgotten. However, clinical, functional and pathological aspects have clearly shown that it is damaged at a very early stage in development of the plaque of demyelination. There is sufficient clinical, radiological and pathological evidence to permit definition of axonal damage as the central element of the pathology and clinical features of multiple sclerosis. DEVELOPMENT AND CONCLUSIONS: Throughout this article we will see how the axon is affected in multiple sclerosis, how this affects the inflammatory response and which parameters allow us to measure axonal damage and its relation to disability. Finally we will see how a new physiopathogenic concept of multiple sclerosis appears, based on the axonal lesion, and how this fits current clinico-pathological concepts better.

Estudio de diversas escalas de fatiga e impacto en la calidad de vida de los pacientes afectos de esclerosis múltiple / A study of the various scales of fatigue and impact on quality of life of patients with multiple sclerosis

Objetivos. Explorar la fatiga en la esclerosis múltiple y valorar la especificidad de tres escalas de fatiga en esta enfermedad: la escala de severidad de fatiga, la escala específica de fatiga y la escala de impacto de fatiga. Materiales y métodos. Hemos remitido 60 encuestas con las tres escalas y la escala de calidad de vida Nottingham Health Profile a pacientes afectos de EM clínicamente definida según los criterios de Poser. Se han contestado 58 encuestas y los datos se han correlacionado mediante la correlación de Sperman y el test de la t de Student, con variables demográficas (edad, edad de inicio y sexo) y clínicas (forma clínica, tiempo de evolución, tiempo transcurrido desde el último brote, tiempo hasta el siguiente brote y escala del estado de discapacidad ampliada (EDSS). Resultados. La fatiga se hallaba presente en el 76 por ciento de los pacientes. Existía una correlación entre la escala de severidad de fatiga y la EDSS, la función piramidal, la función cerebelosa, el tiempo de evolución de la enfermedad y la forma clínica. Hemos encontrado que la escala específica de fatiga es independiente de la EDSS. La escala de impacto de fatiga se correlaciona con la EDSS salvo en las preguntas referidas a la función cognitiva. Conclusiones. La fatiga es un síntoma frecuente en la EM que afecta de forma independiente a la función cognitiva. Asimismo, se relaciona con la afectación del sistema piramidal y del sistema cerebeloso, y depende del grado de discapacidad y el tiempo de evolución. La escala específica de fatiga es una buena herramienta para explorar este síntoma en la EM (AU)

Afectación axonal en la esclerosis múltiple. Conceptos actuales / Axonal involvement in multiple sclerosis. Current concepts

Introducción. La patología del axón en la esclerosis múltiple es un concepto emergente, no por desconocido sino por olvidado. Pero tanto a nivel clínico, como funcional y patológico, es un hecho plenamente demostrado que está dañado en fases muy precoces del proceso de desarrollo de la placa de desmielinización. Existen suficientes evidencias clínicas, radiológicas y patológicas que permiten definir el daño axonal como el elemento central en la patología y la clínica de la esclerosis múltiple. Desarrollo y conclusiones. A lo largo de la presente exposición veremos cómo se afecta el axón en la esclerosis múltiple, cómo determina la respuesta inflamatoria esta afectación axonal y cuáles son los parámetros que nos permiten medir el daño axonal y su relación con la discapacidad. Por último, veremos cómo se vislumbra una nueva concepción fisiopatogénica de la esclerosis múltiple basada en la lesión axonal y cómo ésta se adapta mejor a los conceptos clínico-patológicos actuales (AU)

[Lacunar infarct and deep cerebral hemorrhage: a comparison of the risk factors]. / Infarto lacunar y hemorragia cerebral profunda: comparación de factores de riesgo.

INTRODUCTION: Lacunar infarcts (LI) and deep cerebral hemorrhages (DCH) have the same localization and a vasculopathy which appears to be similar, at the level of the small perforating arteries, classically attributed to arterial hypertension (AHT). OBJECTIVES: To compare the vascular risk factors of patients with lacunar ictus (LIc) and those with DCH, to try to determine how these may affect the appearance of one type of stroke or another. PATIENTS AND METHODS: We analyzed a prospective consecutive series of patients with cerebral vascular accidents (CVA), selecting 1,540 patients in the first 1,155 with a first CVA. We recorded demographic data and the following risk factors: previous transient ischemic accident (TIA), AHT, diabetes mellitus (DM), hypercholesterolemia, ischemic cardiopathy, atrial fibrillation and the presence of silent infarcts on CT. RESULTS: Two hundred and four patients had LIc and 163 had DCH. There was a significant dissociation between LIc and a history of TIA, DM, hypercholesterolemia and the presence of silent lacunar-type infarcts on CT. However, after multivariant analysis, DM did not continue to be an independent variable. Arterial blood pressure was found to be greater in the DCH group. CONCLUSIONS: The presence of different risk factors for LIc and DCH may be the key to understanding the mechanism which leads to one type or other of CVA.

Circadian variation in acute ischemic stroke: a hospital-based study.

BACKGROUND AND PURPOSE: We investigated circadian rhythm in ischemic stroke onset and its subtypes, differentiating between first-ever stroke and recurrent stroke. METHODS: A consecutive series of 1223 patients with ischemic stroke was admitted at 2 reference hospitals; the time of onset of symptoms was obtained, differentiating between onset while asleep and awake. We compared circadian rhythm between stroke types and between first-ever and recurrent stroke. RESULTS: The onset time was known in 914 patients; 25.6% experienced onset on awakening [higher incidence in thrombotic and lacunar stroke (28.9% and 28.4%, respectively) than in embolic stroke (18.8%)]. For all stroke subtypes, there was a significant diurnal variation, with a morning peak between 6 AM and noon; after redistributing the hour of onset of patients awakening with stroke, the morning peak was minimal in all types of stroke. There were no differences in circadian rhythm between patients with first-ever and recurrent stroke. CONCLUSIONS: Only hospitalized patients were studied. There is a circadian rhythm in all types of stroke, with higher frequency during the day and lower frequency in the last hours in the evening. The highest incidence in the early hours of the morning can be overestimated, due to patients who awaken with stroke. There is no difference in circadian rhythm between first-ever stroke and recurrent stroke.

[Familial multiple sclerosis: a study of six families]. / Esclerosis múltiple familiar: estudio de seis familias.

INTRODUCTION: Multiple sclerosis (MS) is a demyelinating disorder of the CNS, of autoimmune pathology and unknown aetiology. Several theories regarding its aetiology have been suggested, although none seems to be completely convincing. Genetically predisposed persons are affected, therefore groups of MS are seen in certain families. OBJECTIVES: To describe the family links, type of illness and evolution of 12 patients from six families with two or more members diagnosed as having MS, and to evaluate any differences from the other cases recorded in our data base. PATIENTS AND METHODS: We studied 12 patients diagnosed on the criteria of Poser, and with at least one first or second degree relation with MS. We compared clinical data, form of presentation and course with 127 patients recorded in the data base. RESULTS: We describe six families: two homozygotic twins, two families in which transmission was from father to child and three families with first degree cousins affected. We found no clinical variation in the presentation, number of attacks or evolution, as compared with the other patients. Nor was there homogeneity between the familial forms of MS. CONCLUSIONS: Familial forms make up approximately 10% of the series. We do not have any data available for early diagnosis nor for prognostic significance of familial MS.

[Sjögren syndrome and subacute demyelinating polyradiculopathy: an unusual association]. / Síndrome de Sjögren y polirradiculopatía desmielinizante subaguda: una rara asociación.

INTRODUCTION: Sjögren's syndrome is a chronic inflammatory condition of unknown aetiology and autoimmune pathology. The defining feature is the dry syndrome, expressed as xerophthalmia and xerostomia. Extra-glandular involvement at many other levels may also occur. Neurological involvement is not unusual. The peripheral nervous system is most frequently involved, and a predominantly sensitive symmetrical distal polyneuropathy may be the first sign of the condition. Other patterns of peripheral involvement are also associated with the syndrome. We present a case of subacute demyelinating polyradiculopathy associated with primary Sjögren's syndrome. CLINICAL CASE: A 28 year old woman with dry syndrome presented with paraesthesia in her hands and feet, distal weakness, which had progressed proximally in the muscles of her arms and legs, and bilateral facial weakness. The condition progressed for eight weeks. When complementary tests were done, alterations typical of this condition (FR, ANA, anti-Ro and anti-La) were seen and also others typical of the dry syndrome (Schirmer's test). Therefore, in view of these findings and the clinical features, after other conditions had been ruled out, a diagnosis of primary Sjögren's syndrome was made. The type of neuropathy was determined by the clinical features, electromyography and CSF findings. Treatment with corticosteroids gave good results. CONCLUSIONS: Demyelinating polyradiculopathy is a form of peripheral nervous system involvement which is rarely seen in this disorder. In the differential diagnosis Sjögren's syndrome should be considered, an orientative history taken, autoantibodies determined and an ophthalmological examination made.