River widening in mountain and foothill areas during floods: Insights from a meta-analysis of 51 European Rivers.

River widening, defined as a lateral expansion of the channel, is a critical process that maintains fluvial ecosystems and is part of the regular functioning of rivers. However, in areas with high population density, channel widening can cause damage during floods. Therefore, for effective flood risk management it is essential to identify river reaches where abrupt channel widening may occur. Despite numerous efforts to predict channel widening, most studies have been limited to single rivers and single flood events, which may not be representative of other conditions. Moreover, a multi-catchment scale approach that covers various settings and flood magnitudes has been lacking. In this study, we fill this gap by compiling a large database comprising 1564 river reaches in several mountain regions in Europe affected by floods of varying magnitudes in the last six decades. By applying a meta-analysis, we aimed to identify the types of floods responsible for more extensive widening, the river reach types where intense widening is more likely to occur, and the hydraulic and morphological variables that explain widening and can aid in predicting widening. Our analysis revealed seven groups of reaches with significantly different responses to floods regarding width ratios (i.e., the ratio between channel width after and before a flood). Among these groups, the river reaches located in the Mediterranean region and affected by extreme floods triggered by short and intense precipitation events showed significantly larger widening than other river reaches in other regions. Additionally, the meta-analysis confirmed valley confinement as a critical morphological variable that controls channel widening but showed that it is not the only controlling factor. We proposed new statistical models to identify river reaches prone to widening, estimate potential channel width after a flood, and compute upper bound width ratios. These findings can inform flood hazard evaluations and the design of mitigation measures.

La inserción académica y laboral como un factor asociado a la calidad de vida en pacientes con epilepsia farmacorresistente del lóbulo temporal / Academic and employment insertion as a factor related to quality of life in patients with drug-resistant temporal lobe epilepsy

Introducción: La inserción académica y laboral es uno de los problemas que más preocupan a las personas con epilepsia, pero permanece sin esclarecer su relación con la calidad de vida. Objetivo: Analizar los efectos de la inserción académica y laboral en la calidad de vida, la ansiedad, la depresión, el apoyo social y las funciones ejecutivas, así como la relación entre estas variables en pacientes con epilepsia farmacorresistente. Pacientes y métodos: Cincuenta y nueve pacientes con epilepsia farmacorresistente del lóbulo temporal fueron clasificados en dos grupos, con inserción académica o laboral (n = 25) y sin inserción (n = 34), y se les realizó una evaluación neuropsicológica. Resultados: Los pacientes con inserción presentaron una calidad de vida significativamente mayor, menor ansiedad rasgo y mayor apoyo social percibido, así como una tendencia a tener un menor porcentaje de errores y un mayor porcentaje de respuestas conceptuales que los pacientes sin inserción laboral. La inserción académica/laboral tuvo efectos indirectos en la calidad de vida a través de su relación con el apoyo social y la ansiedad rasgo. Conclusiones: Nuestros hallazgos ofrecen un modelo para entender la calidad de vida en los pacientes con epilepsia del lóbulo temporal desde una perspectiva integral del paciente y señalan el papel clave del aumento del apoyo social y de la reducción de la ansiedad asociados con la inserción académica y laboral para mejorar la calidad de vida. Estos resultados podrían favorecer la implementación de programas que promuevan la reinserción académica o laboral, considerando la relevancia de variables socioemocionales.(AU)

Introduction: Academic and employment insertion is one of the issues that most concern people with epilepsy, but little is known about its relationship with quality of life. Aim: We aimed to analyze the effects of the academic and employment insertion on quality of life, anxiety, depression, social support, and executive functions, and the relationships among these variables in patients with drug-resistant epilepsy. Patients and methods: Fifty-nine patients with drug-resistant temporal lobe epilepsy were classified into two groups: with academic or employment insertion (n = 25) and without insertion (n = 34) and underwent a neuropsychological evaluation. Results: Patients with insertion had a significantly better quality of life, lower trait anxiety, and higher social support, and tended to have a lower percentage of errors and higher percent conceptual level responses than those without insertion. Academic/employment insertion had indirect effects on quality of life through its relationship with global social support and trait anxiety. Conclusions: Our findings provide a model for understanding the quality of life in patients with temporal lobe epilepsy for an integral perspective of the patient and points out the key role of increased social support and reduced anxiety associated with academic and employment insertion to improve quality of life. These results could favor the implementation of programs that promote academic or employment reinsertion, considering the relevance of socio-emotional domains.(AU)

Academic and employment insertion as a factor related to quality of life in patients with drug-resistant temporal lobe epilepsy. / La inserción académica y laboral como un factor asociado a la calidad de vida en pacientes con epilepsia farmacorresistente del lóbulo temporal.

INTRODUCTION: Academic and employment insertion is one of the issues that most concern people with epilepsy, but little is known about its relationship with quality of life. AIM: We aimed to analyze the effects of the academic and employment insertion on quality of life, anxiety, depression, social support, and executive functions, and the relationships among these variables in patients with drug-resistant epilepsy. PATIENTS AND METHODS: Fifty-nine patients with drug-resistant temporal lobe epilepsy were classified into two groups: with academic or employment insertion (n = 25) and without insertion (n = 34) and underwent a neuropsychological evaluation. RESULTS: Patients with insertion had a significantly better quality of life, lower trait anxiety, and higher social support, and tended to have a lower percentage of errors and higher percent conceptual level responses than those without insertion. Academic/employment insertion had indirect effects on quality of life through its relationship with global social support and trait anxiety. CONCLUSIONS: Our findings provide a model for understanding the quality of life in patients with temporal lobe epilepsy for an integral perspective of the patient and points out the key role of increased social support and reduced anxiety associated with academic and employment insertion to improve quality of life. These results could favor the implementation of programs that promote academic or employment reinsertion, considering the relevance of socio-emotional domains.

TITLE: La inserción académica y laboral como un factor asociado a la calidad de vida en pacientes con epilepsia farmacorresistente del lóbulo temporal.Introducción. La inserción académica y laboral es uno de los problemas que más preocupan a las personas con epilepsia, pero permanece sin esclarecer su relación con la calidad de vida. Objetivo. Analizar los efectos de la inserción académica y laboral en la calidad de vida, la ansiedad, la depresión, el apoyo social y las funciones ejecutivas, así como la relación entre estas variables en pacientes con epilepsia farmacorresistente. Pacientes y métodos. Cincuenta y nueve pacientes con epilepsia farmacorresistente del lóbulo temporal fueron clasificados en dos grupos, con inserción académica o laboral (n = 25) y sin inserción (n = 34), y se les realizó una evaluación neuropsicológica. Resultados. Los pacientes con inserción presentaron una calidad de vida significativamente mayor, menor ansiedad rasgo y mayor apoyo social percibido, así como una tendencia a tener un menor porcentaje de errores y un mayor porcentaje de respuestas conceptuales que los pacientes sin inserción laboral. La inserción académica/laboral tuvo efectos indirectos en la calidad de vida a través de su relación con el apoyo social y la ansiedad rasgo. Conclusiones. Nuestros hallazgos ofrecen un modelo para entender la calidad de vida en los pacientes con epilepsia del lóbulo temporal desde una perspectiva integral del paciente y señalan el papel clave del aumento del apoyo social y de la reducción de la ansiedad asociados con la inserción académica y laboral para mejorar la calidad de vida. Estos resultados podrían favorecer la implementación de programas que promuevan la reinserción académica o laboral, considerando la relevancia de variables socioemocionales.

[Cognitive reserve as a modulating factor in the impact of surgery on visual memory and naming in temporal lobe epilepsy patients]. / La reserva cognitiva como factor modulador del impacto de la cirugía sobre la memoria visual y la denominación en pacientes con epilepsia del lóbulo temporal.

INTRODUCTION: Temporal lobe epilepsy (TLE) surgery is an effective procedure that produces cognitive changes. Factors modulating such changes have been proposed, but the influence of cognitive reserve remains unclear. OBJECTIVE: To examine the effect of intellectual quotient (IQ) on postsurgical changes in verbal fluency, naming, and verbal and visual memory in a sample of patients with TLE. PATIENTS AND METHODS: 64 adult patients with drug-resistant TLE (mean age ± SD: 39.16 ± 11.67) underwent a neuropsychological evaluation before and one year after surgery. RESULTS: Patients with high IQ showed better immediate visual memory before surgery than those with medium IQ, as well as an absence of postsurgical changes. Patients with high manipulative IQ had better naming before surgery than those with medium manipulative IQ, as well as a significant postsurgical worsening. Both before and after surgery, patients with high IQ showed better phonemic and semantic verbal fluency and short- and long-term verbal memory than those with medium IQ. CONCLUSIONS: IQ is a relevant factor in the evolution of immediate visual memory and naming after surgery in patients with TLE. Surgery does not impact on the advantage of high IQ patients in verbal fluency and verbal memory, suggesting that cognitive reserve has a positive effect on cognitive function, even after TLE surgery.

TITLE: La reserva cognitiva como factor modulador del impacto de la cirugía sobre la memoria visual y la denominación en pacientes con epilepsia del lóbulo temporal.Introducción. La cirugía de la epilepsia del lóbulo temporal (ELT) es un procedimiento eficaz que produce cambios cognitivos. Se han propuesto factores moduladores de dichos cambios, pero permanece sin esclarecer la influencia de la reserva cognitiva. Objetivo. Examinar el efecto del cociente intelectual (CI) sobre los cambios posquirúrgicos en medidas de fluencia verbal, denominación y memoria verbal y visual en una muestra de pacientes con ELT. Pacientes y métodos. Sesenta y cuatro pacientes adultos con ELT farmacorresistente (edad media ± desviación típica: 39,16 ± 11,67) fueron sometidos a una evaluación neuropsicológica antes y un año después de la cirugía. Resultados. Los pacientes con un CI alto presentaron un mejor funcionamiento de la memoria visual inmediata antes de la cirugía que los que tenían un CI medio, así como ausencia de cambios posquirúrgicos. Los pacientes con un CI manipulativo alto presentaron mejor denominación antes de la cirugía que los que tenían un CI manipulativo medio, así como un empeoramiento posquirúrgico significativo. Tanto antes como después de la cirugía, los pacientes con un CI alto presentaron mejor fluencia verbal fonémica y semántica y memoria verbal a corto y largo plazo que los que tenían un CI medio. Conclusiones. El CI es un factor relevante en la evolución de la memoria visual inmediata y de la denominación tras la cirugía en pacientes con ELT. La cirugía no repercute en la ventaja que tienen los pacientes con un CI alto en fluencia verbal y memoria verbal, lo que sugiere que la reserva cognitiva tiene un efecto positivo sobre la función cognitiva, incluso tras la cirugía de la ELT.

La reserva cognitiva como factor modulador del impacto de la cirugía sobre la memoria visual y la denominación en pacientes con epilepsia del lóbulo temporal / Cognitive reserve as a modulating factor in the impact of surgery on visual memory and naming in temporal lobe epilepsy patients

Introducción:La cirugía de la epilepsia del lóbulo temporal (ELT) es un procedimiento eficaz que produce cambios cognitivos. Se han propuesto factores moduladores de dichos cambios, pero permanece sin esclarecer la influencia de la reserva cognitiva. Objetivo: Examinar el efecto del cociente intelectual (CI) sobre los cambios posquirúrgicos en medidas de fluencia verbal, denominación y memoria verbal y visual en una muestra de pacientes con ELT. Pacientes y métodos: Sesenta y cuatro pacientes adultos con ELT farmacorresistente (edad media ± desviación típica: 39,16 ± 11,67) fueron sometidos a una evaluación neuropsicológica antes y un año después de la cirugía. Resultados: Los pacientes con un CI alto presentaron un mejor funcionamiento de la memoria visual inmediata antes de la cirugía que los que tenían un CI medio, así como ausencia de cambios posquirúrgicos. Los pacientes con un CI manipulativo alto presentaron mejor denominación antes de la cirugía que los que tenían un CI manipulativo medio, así como un empeoramiento posquirúrgico significativo. Tanto antes como después de la cirugía, los pacientes con un CI alto presentaron mejor fluencia verbal fonémica y semántica y memoria verbal a corto y largo plazo que los que tenían un CI medio. Conclusiones: El CI es un factor relevante en la evolución de la memoria visual inmediata y de la denominación tras la cirugía en pacientes con ELT. La cirugía no repercute en la ventaja que tienen los pacientes con un CI alto en fluencia verbal y memoria verbal, lo que sugiere que la reserva cognitiva tiene un efecto positivo sobre la función cognitiva, incluso tras la cirugía de la ELT.(AU)

Introduction: Temporal lobe epilepsy (TLE) surgery is an effective procedure that produces cognitive changes. Factors modulating such changes have been proposed, but the influence of cognitive reserve remains unclear. Objective: To examine the effect of intellectual quotient (IQ) on postsurgical changes in verbal fluency, naming, and verbal and visual memory in a sample of patients with TLE. Patients and methods: 64 adult patients with drug-resistant TLE (mean age ± SD: 39.16 ± 11.67) underwent a neuropsychological evaluation before and one year after surgery. Results: Patients with high IQ showed better immediate visual memory before surgery than those with medium IQ, as well as an absence of postsurgical changes. Patients with high manipulative IQ had better naming before surgery than those with medium manipulative IQ, as well as a significant postsurgical worsening. Both before and after surgery, patients with high IQ showed better phonemic and semantic verbal fluency and short- and long-term verbal memory than those with medium IQ. Conclusions: IQ is a relevant factor in the evolution of immediate visual memory and naming after surgery in patients with TLE. Surgery does not impact on the advantage of high IQ patients in verbal fluency and verbal memory, suggesting that cognitive reserve has a positive effect on cognitive function, even after TLE surgery.(AU)

Cannabidiol para el tratamiento del síndrome de Lennox-Gastaut y del síndrome de Dravet: recomendaciones de expertos sobre su uso en la práctica clínica en España / Cannabidiol for the treatment of Lennox-Gastaut syndrome and Dravet syndrome: experts recommendations for its use in clinical practice in Spain

Introducción: El cannabidiol (CBD) es uno de los componentes principales de la planta del cannabis que ha demostrado efecto ante las crisis epilépticas. Tras su desarrollo clínico, obtuvo su aprobación por la Agencia Europea del Medicamento en septiembre de 2019 para el tratamiento de las crisis epilépticas asociadas con el síndrome de Lennox-Gastaut (SLG) y el síndrome de Dravet (SD), en combinación con el clobazam (CLB), en pacientes a partir de los dos años. Objetivo: Establecer unas recomendaciones de manejo del CBD derivado de la planta altamente purificado consensuadas por expertos españoles en el tratamiento de la epilepsia para su uso en pacientes con SD y SLG, basándose en su experiencia clínica y en la evidencia científica. Desarrollo: Reunión de consenso de un grupo de cuatro neurólogos y neuropediatras españoles expertos en el manejo de la epilepsia asociada al SD y el SLG y con experiencia clínica en el uso de CBD. Se debatió sobre diferentes áreas, incluyendo la posología (dosis de inicio, pauta de escalada), la eficacia (valoración de resultados e indicaciones para la suspensión del tratamiento) y la seguridad (evaluación, interacciones entre fármacos, manejo de efectos adversos). Conclusiones: Para optimizar el tratamiento con CBD, se recomienda una pauta lenta de escalada de dosis (de cuatro semanas o más) hasta alcanzar la dosis máxima recomendada o el efecto deseado, reducir los fármacos anticrisis epilépticas concomitantes si aparecen efectos adversos por interacciones y mantener el tratamiento al menos seis meses si se tolera. La eficacia y la seguridad del CBD deben evaluarse de forma individual, considerando el beneficio y el riesgo para cada paciente.

Introduction: Cannabidiol (CBD) is one of the main components of the cannabis plant that has demonstrated anti-epileptic seizure effect. Following its clinical development, in September 2019 the European Medicines Agency approved its indication for the adjunctive therapy of epileptic seizures associated with Lennox-Gastaut syndrome (LGS) and Dravet syndrome (DS), combined with clobazam (CLB), in patients of 2 years of age and older.Aim: To establish recommendations on the use of plant-derived highly purified CBD on which Spanish experts have reached consensus for the treatment of epilepsy in patients with DS and LGS based on their clinical experience and the scientific evidence. Development: Consensus meeting with the participation of four Spanish neurologists and neuropediatric who are experts in epilepsy secondary to DS and LGS and with clinical experience in the use and management of CBD. They discussed on several topics, including posology (starting dose, dose escalation schema), efficacy (assessment of outcomes and indications for treatment withdrawal), and safety (evaluation, drug-drug interactions, adverse events management). Conclusions: In order to optimise CBD treatment, a slow dose escalation (≥ 4 weeks) is recommended until the maximum recommended dose or the desire effect is reached. It is also recommended that the concomitant antiseizure medications (ASMs) be reduced in case of adverse events due to interactions, and that the treatment continues for at least 6 months if it is well tolerated. The efficacy and safety of CBD must be assessed individually, considering the benefits and risks for individual patients.(AU)

Impact of COVID-19 on Spanish patients with Dravet syndrome and their caregivers: consequences of lockdown. / Impacto de la COVID-19 en pacientes españoles con síndrome de Dravet y sus cuidadores: consecuencias del confinamiento.

INTRODUCTION: The COVID-19 pandemic caused a state of alarm in Spain in March 2020. The necessary approach to the care of patients with Dravet syndrome (DS) makes them and their caregivers a vulnerable group in emergency situations. OBJECTIVES: To explore the impact of the COVID-19 pandemic on the management and condition of Spanish patients with DS and their caregivers and families. MATERIALS AND METHODS: Analysis of data belonging to Spanish families taken from a European online survey (14 April-17 May 2020). It included data on DS patients, on the disease and on caregivers before and after lockdown during the state of alarm. RESULTS: Sixty-nine Spanish families participated; average age of patients: 12.6 years. Except in 19% of the cases that were isolated, protective/isolation measures for patients were followed without increasing. Epilepsy remained stable, with no medication or resource/personnel availability issues. Sleep-wake pattern (61%) and behavior (41%) of patients changed. Behavior change was associated with seizures during lockdown and with caregiver emotional state (changes in 76%). Psychological support was offered to only 9% of caregivers. Thirty-eight per cent of patients did not receive remote care. CONCLUSIONS: The experience gathered during the lockdown has allowed the detection of points of improvement to ensure the proper management of DS and to keep the situation of patients and caregivers stable. All of this with a prominent role of telemedicine.

TITLE: Impacto de la COVID-19 en pacientes españoles con síndrome de Dravet y sus cuidadores: consecuencias del confinamiento.Introducción. La pandemia por COVID-19 implicó el estado de alarma en España en marzo de 2020. El abordaje necesario para el cuidado de los pacientes con síndrome de Dravet (SD) los convierte, junto con sus cuidadores, en un grupo vulnerable en situaciones de emergencia. Objetivos. Explorar el impacto de la pandemia por COVID-19 en el manejo y la condición de los pacientes españoles con SD, y de sus cuidadores y familias. Materiales y métodos. Análisis de los datos pertenecientes a familias españolas extraídos de una encuesta en línea europea (14 de abril-17 de mayo de 2020). Incluía datos de los pacientes con SD, de la enfermedad y de los cuidadores antes y después del confinamiento, durante el estado de alarma. Resultados. Participaron 69 familias españolas; edad media de los pacientes: 12,6 años. Excepto en el 19% de los casos que fueron aislados, las medidas de protección/aislamiento del paciente continuaron sin incrementar. La epilepsia se mantuvo estable, sin problemas de medicación ni disponibilidad de recursos/personal. Cambió el patrón de sueño/vigilia (61%) y la conducta (41%) de los pacientes. El cambio de conducta se asoció con las crisis durante el confinamiento y el estado anímico del cuidador (cambios en el 76%). Sólo se ofreció apoyo psicológico al 9% de los cuidadores. El 38% de los pacientes no recibió atención telemática. Conclusiones. La experiencia recogida durante el confinamiento ha permitido detectar puntos de mejora para asegurar el apropiado manejo del SD y mantener estable la situación de los pacientes y cuidadores, todo ello con un papel destacado de la telemedicina.

Impacto de la COVID-19 en pacientes españoles con síndrome de Dravet y sus cuidadores: consecuencias del confinamiento / Impact of COVID-19 on Spanish patients with Dravet syndrome and their caregivers: consequences of lockdown

Introducción: La pandemia por COVID-19 implicó el estado de alarma en España en marzo de 2020. El abordaje necesario para el cuidado de los pacientes con síndrome de Dravet (SD) los convierte, junto con sus cuidadores, en un grupo vulnerable en situaciones de emergencia. Objetivos: Explorar el impacto de la pandemia por COVID-19 en el manejo y la condición de los pacientes españoles con SD, y de sus cuidadores y familias. Materiales y métodos: Análisis de los datos pertenecientes a familias españolas extraídos de una encuesta en línea europea (14 de abril-17 de mayo de 2020). Incluía datos de los pacientes con SD, de la enfermedad y de los cuidadores antes y después del confinamiento, durante el estado de alarma. Resultados: Participaron 69 familias españolas; edad media de los pacientes: 12,6 años. Excepto en el 19% de los casos que fueron aislados, las medidas de protección/aislamiento del paciente continuaron sin incrementar. La epilepsia se mantuvo estable, sin problemas de medicación ni disponibilidad de recursos/personal. Cambió el patrón de sueño/vigilia (61%) y la conducta (41%) de los pacientes. El cambio de conducta se asoció con las crisis durante el confinamiento y el estado anímico del cuidador (cambios en el 76%). Sólo se ofreció apoyo psicológico al 9% de los cuidadores. El 38% de los pacientes no recibió atención telemática. Conclusiones: La experiencia recogida durante el confinamiento ha permitido detectar puntos de mejora para asegurar el apropiado manejo del SD y mantener estable la situación de los pacientes y cuidadores, todo ello con un papel destacado de la telemedicina.(AU)

Introduction: The COVID-19 pandemic caused a state of alarm in Spain in March 2020. The necessary approach to the care of patients with Dravet syndrome (DS) makes them and their caregivers a vulnerable group in emergency situations. Objectives: To explore the impact of the COVID-19 pandemic on the management and condition of Spanish patients with DS and their caregivers and families. Materials and methods: Analysis of data belonging to Spanish families taken from a European online survey (14 April-17 May 2020). It included data on DS patients, on the disease and on caregivers before and after lockdown during the state of alarm. Results: Sixty-nine Spanish families participated; average age of patients: 12.6 years. Except in 19% of the cases that were isolated, protective/isolation measures for patients were followed without increasing. Epilepsy remained stable, with no medication or resource/personnel availability issues. Sleep-wake pattern (61%) and behavior (41%) of patients changed. Behavior change was associated with seizures during lockdown and with caregiver emotional state (changes in 76%). Psychological support was offered to only 9% of caregivers. Thirty-eight per cent of patients did not receive remote care. Conclusions: The experience gathered during the lockdown has allowed the detection of points of improvement to ensure the proper management of DS and to keep the situation of patients and caregivers stable. All of this with a prominent role of telemedicine.(AU)

New therapeutic approach in Dravet syndrome and Lennox-Gastaut syndrome with cannabidiol. / Cannabidiol en los síndromes de Dravet y Lennox-Gastaut: un nuevo abordaje terapéutico.

INTRODUCTION: Dravet syndrome (DS) and Lennox-Gastaut syndrome (LGS) are two serious epileptic syndromes with paediatric onset which are refractory to therapy and are associated with an important increase in mortality rates and comorbidities compared to the general population. These pathologies have a strong impact on the lives of patients and their families, because they undergo multiple pharmacological therapies (many of them without specific indication), with frequent changes due to poor efficacy and associated adverse effects. The specialists who care for these patients highlight unmet needs and the lack of specific, safe and effective treatments for better management of the syndrome. DEVELOPMENT: A group of four neurologists specializing in epilepsy has met to review the scientific literature and evaluate the efficacy and safety of oral solution cannabidiol in the treatment of these syndromes, both in randomized clinical trials (CT) and in some observational studies. CONCLUSIONS: Cannabidiol is positioned as an innovative therapy that allows better control of epileptic seizures and comorbidities of DS and LGS, furthermore its efficacy and safety have been evaluated in more than 700 patients. In CTs, cannabidiol significantly reduced the percentage of convulsive seizures and drop seizures compared to placebo in patients with DS and LGS respectively, which could improve their quality of life and that of their family members. The most frequent adverse effects reported were somnolence and decreased appetite. Elevated liver aminotransferase levels were also reported, especially in patients given concomitant sodium valproate. This therapy may allow better control of the epileptic seizures associated with these syndromes.

TITLE: Cannabidiol en los síndromes de Dravet y Lennox-Gastaut: un nuevo abordaje terapéutico.Introducción. Los síndromes de Dravet (SD) y Lennox-Gastaut (SLG) son dos síndromes epilépticos graves y de inicio en la edad pediátrica, refractarios al tratamiento, asociados a un notable incremento en las tasas de mortalidad y comorbilidades respecto a la población general. Suponen un fuerte impacto en la vida de los pacientes y sus familiares, ya que los pacientes están sometidos a múltiples terapias farmacológicas (muchas sin indicación específica), con cambios frecuentes debido a la escasa eficacia y a los efectos adversos. Los especialistas que les atienden destacan las necesidades no cubiertas y la falta de tratamientos específicos, seguros y eficaces para un mejor manejo de la enfermedad. Desarrollo. Se ha reunido un grupo formado por cuatro neurólogos especialistas en epilepsia para hacer una revisión de la literatura científica y evaluar los resultados de eficacia y seguridad de la solución oral de cannabidiol en el tratamiento de estos síndromes, tanto en ensayos clínicos aleatorizados como en diversos estudios observacionales. Conclusiones. El cannabidiol se sitúa como una terapia innovadora que permite un mejor control de las crisis epilépticas y comorbilidades del SD y el SLG; además, su eficacia y seguridad han sido evaluadas en más de 700 pacientes. En los ensayos clínicos redujo significativamente el porcentaje de crisis convulsivas y de caída en comparación con placebo en los pacientes con SD y SLG, respectivamente, y puede mejorar su calidad de vida y la de sus familiares. Los efectos adversos más frecuentes fueron la somnolencia y la disminución del apetito. También se notificaron niveles elevados de aminotransferasas hepáticas, especialmente en pacientes tratados concomitantemente con ácido valproico. Esta terapia podría permitir un mejor control de las crisis epilépticas asociadas a estas patologías.

Cannabidiol en los síndromes de Dravet y Lennox-Gastaut: un nuevo abordaje terapéutico / New therapeutic approach in Dravet syndrome and Lennox-Gastaut syndrome with cannabidiol

Introducción: Los síndromes de Dravet (SD) y Lennox-Gastaut (SLG) son dos síndromes epilépticos graves y de inicio en la edad pediátrica, refractarios al tratamiento, asociados a un notable incremento en las tasas de mortalidad y comorbilidades respecto a la población general. Suponen un fuerte impacto en la vida de los pacientes y sus familiares, ya que los pacientes están sometidos a múltiples terapias farmacológicas (muchas sin indicación específica), con cambios frecuentes debido a la escasa eficacia y a los efectos adversos. Los especialistas que les atienden destacan las necesidades no cubiertas y la falta de tratamientos específicos, seguros y eficaces para un mejor manejo de la enfermedad. Desarrollo: Se ha reunido un grupo formado por cuatro neurólogos especialistas en epilepsia para hacer una revisión de la literatura científica y evaluar los resultados de eficacia y seguridad de la solución oral de cannabidiol en el tratamiento de estos síndromes, tanto en ensayos clínicos aleatorizados como en diversos estudios observacionales. Conclusiones: El cannabidiol se sitúa como una terapia innovadora que permite un mejor control de las crisis epilépticas y comorbilidades del SD y el SLG; además, su eficacia y seguridad han sido evaluadas en más de 700 pacientes. En los ensayos clínicos redujo significativamente el porcentaje de crisis convulsivas y de caída en comparación con placebo en los pacientes con SD y SLG, respectivamente, y puede mejorar su calidad de vida y la de sus familiares. Los efectos adversos más frecuentes fueron la somnolencia y la disminución del apetito. También se notificaron niveles elevados de aminotransferasas hepáticas, especialmente en pacientes tratados concomitantemente con ácido valproico. Esta terapia podría permitir un mejor control de las crisis epilépticas asociadas a estas patologías.(AU)

Introduction: Dravet syndrome (DS) and Lennox-Gastaut syndrome (LGS) are two serious epileptic syndromes with paediatric onset which are refractory to therapy and are associated with an important increase in mortality rates and comorbidities compared to the general population. These pathologies have a strong impact on the lives of patients and their families, because they undergo multiple pharmacological therapies (many of them without specific indication), with frequent changes due to poor efficacy and associated adverse effects. The specialists who care for these patients highlight unmet needs and the lack of specific, safe and effective treatments for better management of the syndrome. Development: A group of four neurologists specializing in epilepsy has met to review the scientific literature and evaluate the efficacy and safety of oral solution cannabidiol in the treatment of these syndromes, both in randomized clinical trials (CT) and in some observational studies. Conclusions: Cannabidiol is positioned as an innovative therapy that allows better control of epileptic seizures and comorbidities of DS and LGS, furthermore its efficacy and safety have been evaluated in more than 700 patients. In CTs, cannabidiol significantly reduced the percentage of convulsive seizures and drop seizures compared to placebo in patients with DS and LGS respectively, which could improve their quality of life and that of their family members. The most frequent adverse effects reported were somnolence and decreased appetite. Elevated liver aminotransferase levels were also reported, especially in patients given concomitant sodium valproate. This therapy may allow better control of the epileptic seizures associated with these syndromes.(AU)

El consumo de un extracto de calafate (Berberis microphylla) modifica marcadores de respuesta inmune en ratones delgados y obesos

RESUMEN La obesidad ha sido identificada como factor de riesgo de severidad de infecciones respiratorias. Apoyar la respuesta inmune en sujetos obesos es de interés. El presente trabajo evaluó el efecto del consumo de un extracto de calafate sobre marcadores de respuesta inmune en ratones delgados y obesos. Ratones C57BL/6J machos fueron expuestos por 82 días a dieta estándar (DE) y alta en grasas (DAG). A un subgrupo de ambos grupos, se les administró 50 y 100 mg [polifenoles totales]/kg peso de animal/día, de extracto, en las últimas dos semanas. Se evaluó expresión génica y secreción de marcadores de respuesta inmune, en tejido pulmonar y plasma. Se observó un efecto del tratamiento con extracto en la expresión de IFN-ϓ. Se observaron efectos inducidos por la DAG y el tratamiento con extracto de manera independiente, en la expresión de IL-12. Se observó un efecto global de la DAG sobre IFN-ϓ plasmático, específicamente una disminución en animales alimentados con DAG. Se observó una interacción entre la dieta y el tratamiento con extracto sobre IL-12 plasmática. El tratamiento utilizado modula marcadores que activan la respuesta inmune ante infecciones respiratorias principalmente de origen viral, en animales delgados y obesos.

ABSTRACT Obesity has been identified as a risk factor for severity of respiratory infections. Thus, the support of the immune response in obese subjects is of interest. The present work evaluated the effect of the consumption of a calafate extract on markers of the immune response in lean and obese mice. Male C57BL/6J mice were exposed for 82 days to a standard or a high-fat diet (HFD). A subgroup of both groups was given 50 and 100 mg [total polyphenols]/kg body weight/day of extract in the last two weeks. Gene expression and secretion of immune response markers were evaluated in lung tissue and plasma. An effect of extract treatment on IFN-ϓ expression was observed. Effects induced by the HFD and treatment with extract were observed independent of the expression of IL-12. An overall effect of the HF diet on plasma IFN-ϓ was observed, specifically a decrease in animals fed the HFD. An interaction between diet and extract treatment was observed over plasma IL-12. The treatment used modulates markers that activate the immune response to respiratory infections, mainly of viral origin, in lean and obese animals.

[Diagnostic challenges in epilepsy]. / Desafios diagnosticos en epilepsia.

INTRODUCTION: Epileptic seizures and epilepsy are part of daily clinical practice in neurology. Yet, the number of false positive diagnoses is surprisingly high. Almost one out of every five patients treated for epilepsy does not really have this diagnosis, which is a high percentage bearing in mind the social and medical consequences that being diagnosed with epilepsy entails. AIMS: To summarise the most important diagnostic challenges in epilepsy, to describe possible sources of diagnostic error and to offer advice on how to avoid them. DEVELOPMENT: Epilepsy is characterised by a tendency to suffer unprovoked epileptic seizures. The greatest obstacle when it comes to diagnosing a case of epilepsy is the fact that epileptic seizures are transient phenomena that occur relatively infrequently and the physician who must carry out the diagnosis will rarely see them. Moreover, there are other clinical events, such as syncopes or non-epileptic seizures, that may be similar to epileptic seizures in appearance and, consequently, can be mistaken for them. Finally, when interpreting the two most important complementary diagnostic techniques in epileptology, the electroencephalogram and magnetic resonance imaging of the brain, the most common errors must be taken into account in order to prevent mistaken diagnoses. CONCLUSIONS: The diagnosis of epilepsy is a challenge and must be based on a detailed and specific medical record. If there are any reasonable doubts, from the outset, about the diagnosis of epilepsy or if the patient does not respond well to the antiepileptic treatment, we recommend referring the patient to a specialised centre to establish a definitive diagnosis.

TITLE: Desafios diagnosticos en epilepsia.Introduccion. Las crisis epilepticas y la epilepsia son parte de la practica clinica diaria en neurologia. No obstante, el numero de diagnosticos falsos positivos es sorprendentemente alto. Casi uno de cada cinco pacientes tratado por epilepsia en realidad no tiene ese diagnostico, un porcentaje elevado teniendo en cuenta las consecuencias sociomedicas que conlleva el diagnostico de epilepsia. Objetivos. Resumir los desafios diagnosticos mas importantes en epilepsia, describir posibles fuentes de error en el diagnostico y proporcionar consejos sobre como evitarlos. Desarrollo. La epilepsia se caracteriza por una tendencia a sufrir crisis epilepticas no provocadas. El mayor obstaculo al diagnosticar una epilepsia radica en que las crisis epilepticas son fenomenos transitorios que ocurren relativamente con poca frecuencia y el medico que realiza el diagnostico raramente llega a verlas. Ademas, existen otros eventos clinicos, como por ejemplo sincopes o crisis no epilepticas, que pueden tener una apariencia similar a las crisis epilepticas y, en consecuencia, confundirse con ellas. Finalmente, al interpretar las dos tecnicas diagnosticas complementarias mas importantes en epileptologia, el electroencefalograma y la resonancia magnetica cerebral, deben tenerse en cuenta los errores mas comunes para prevenir diagnosticos erroneos. Conclusiones. El diagnostico de una epilepsia es un reto y debe basarse en una historia clinica detallada y especifica. Si desde el inicio existen dudas razonables sobre el diagnostico de epilepsia o si el paciente no responde bien al tratamiento antiepileptico, recomendamos derivar al paciente a un centro especializado que establezca un diagnostico definitivo.

Cascading processes in a changing environment: Disturbances on fluvial ecosystems in Chile and implications for hazard and risk management.

The compound hazard effects of multiple process cascades severely affect Chilean river systems and result in a large variety of disturbances on their ecosystems and alterations of their hydromorphologic regimes leading to extreme impacts on society, environment and infrastructure. The acute, neo-tectonically pre-determined susceptibility to seismic hazards, the widespread volcanic activity, the increasing glacier retreat and the continuous exposure to forest fires clearly disturb entire riverine systems and concur to trigger severe floods hazards. With the objective to refine the understanding of such cascading processes and to prospect feasible flood risk management strategies in such a rapidly changing environment we first classify the large river basins according to a set of disturbances (i.e. volcanic eruptions, earthquakes, glacier lake outburst floods, wild fires and mass movements). Then, we describe emblematic cases of process cascades which affected specific Chilean drainage basins and resulted in high losses as tangible examples of how the cascading processes may unfold in other river basins with similar characteristics. As an attempt to enrich the debate among management authorities and academia in Chile, and elsewhere, on how to sustainably manage river systems, we: a) highlight the pivotal need to determine the possible process cascades that may profoundly alter the system and b) we suggest to refine hazard and risk assessments accordingly, accounting for the current and future exposure. We advocate, finally, for the adoption of holistic approaches promoting anticipatory adaptation which may result in resilient system responses.

Accelerated long-term forgetting in resected and seizure-free temporal lobe epilepsy patients.

Episodic memory impairments caused by temporal lobe epilepsy (TLE) are well documented in the literature. Standard clinical episodic memory tests typically include a 30-min delayed recall test. However, in the past decade, it has become apparent that this standard test does not capture the full range of memory problems in TLE patients. Some patients perform well on a standard 30-min delayed recall test, but show Accelerated Long-term Forgetting (ALF) after 24 h. Although ALF has been investigated in patients with different types of epilepsy, current research on resected TLE patients is missing. In the present study, resected TLE patients were compared to a control group matched on initial learning. They showed normal performance on verbal recall after 30 min, but impairments became apparent after one week. Moreover, the significant interaction between participant group and memory test delay demonstrated that the patients indeed showed an acceleration in forgetting. Furthermore, ALF was present in both left and right resected TLE patients, which contradicts the presence of material-specific hemispheric differences in ALF. In addition, ALF was observed in seizure-free resected TLE patients, thereby demonstrating that this factor is not crucial for long-term memory deficits. The outcome shows that clinicians are likely to underestimate memory deficits in resected TLE patients and, therefore, advocates for the inclusion of ALF tests in standard clinical batteries for both pre- and post-surgery testing sessions.

[«Apuntes en Neurologia¼ (Notes in Neurology): a synthesis of the evidence on common paroxysmal neurological disorders and on neurodegenerative disorders]. / «Apuntes en Neurologia¼: una sintesis de la evidencia en trastornos neurologicos comunes paroxisticos y en trastornos neurodegenerativos.

«Apuntes en Neurologia¼ is an initiative in which prominent national and international leaders, with broad academic recognition, came together to synthesise the most outstanding clinical aspects within their area of interest and to discuss the latest developments in a more accessible language. Understanding the factors that affect the onset and progression of any neurological disease through a review is important to be able to develop strategies to reduce the burden of these diseases. Moreover, knowledge of the clinical aspects is essential to solve the problems of daily clinical practice. The data collected here reflect the weight of evidence and some of them anticipate a promising future in the treatment of these diseases. This first edition focuses on common paroxysmal neurological disorders such as migraine, epilepsy and sleep disorders, as well as neurodegenerative disorders such as Parkinson's disease and cognitive impairment. These are clearly different pathologies, although some of them such as migraine and epilepsy, may share clinical symptoms. Sleep disorders, however, are important manifestations of neurodegenerative diseases that are sometimes clinically apparent long before the onset of other neurological symptoms. After recalling pathophysiology and diagnosis, the current review focuses on bringing together the main advances in five of the major neurological diseases.

TITLE: «Apuntes en Neurologia¼: una sintesis de la evidencia en trastornos neurologicos comunes paroxisticos y en trastornos neurodegenerativos.«Apuntes en Neurologia¼ es una iniciativa en la cual lideres de primera linea nacional e internacional, con amplio reconocimiento academico, se reunieron para sintetizar los aspectos clinicos mas destacables dentro de su area de interes y acercar las novedades en una lengua mas proxima. Entender los factores que afectan al inicio y progresion de cualquier enfermedad neurologica a traves de una revision es importante para el desarrollo de estrategias en pro de reducir la carga de estas enfermedades, y conocer los aspectos clinicos es esencial para poder resolver los problemas de la practica clinica diaria. Los datos aqui recogidos reflejan el peso de la evidencia y algunos de ellos anticipan un futuro prometedor en el tratamiento de estas enfermedades. Esta primera edicion se centra en trastornos neurologicos comunes paroxisticos como la migraña, la epilepsia y las alteraciones del sueño, y en trastornos neurodegenerativos como la enfermedad de Parkinson y el deterioro cognitivo. Se trata de patologias claramente diferentes, si bien algunas de ellas, como la migraña y la epilepsia, pueden compartir sintomatologia clinica. Los trastornos del sueño, por su parte, son manifestaciones importantes de enfermedades neurodegenerativas que, en ocasiones, son clinicamente evidentes mucho antes del inicio de otros sintomas neurologicos. Tras recordar la fisiopatologia y el diagnostico, la revision actual se centra en acercar los principales avances en cinco de las principales enfermedades neurologicas.

Consenso Delphi EPICON: recomendaciones sobre el manejo adecuado del cambio a acetato de eslicarbazepina en epilepsia / EPICON consensus: recommendations for proper management of switching to eslicarbazepine acetate in epilepsy

INTRODUCCIÓN: El objetivo del proyecto EPICON es desarrollar una serie de recomendaciones sobre la forma adecuada de realizar el cambio de carbamazepina (CBZ) y oxcarbazepina (OXC) a acetato de eslicarbazepina (ESL) en determinados pacientes con epilepsia. MÉTODOS: Un comité coordinador preparó un cuestionario con 56 preguntas en relación con el cambio de CBZ u OXC a ESL en la práctica clínica (metodología y situaciones del cambio). Posteriormente, se consultó a 54 expertos en epilepsia con el empleo de metodología Delphi (2 rondas de consulta). Se definió un consenso en acuerdo o desacuerdo si las respuestas para el ítem estudiado alcanzaban una mediana ≥ 7 o ≤ 3, respectivamente, y un rango intercuartílico relativo ≤ 0,40. Se analizaron los resultados y se formularon las conclusiones. RESULTADOS: Las recomendaciones fundamentales fueron: el cambio de CBZ a ESL debe ser realizado en 1-3 semanas, con una equivalencia de dosis CBZ:ESL de 1:1.3, siendo recomendado en pacientes con olvidos de medicación, trabajos por turnos, polimedicados, problemas cognitivos, osteoporosis-osteopenia severa, dislipidemia o enfermedad hepática (ausencia de fallo hepático grave), así como en varones con disfunción eréctil causada por CBZ. El cambio de OXC a ESL puede realizarse de un día para otro con una equivalencia de dosis 1:1 y es recomendado en pacientes con olvidos de medicación, trabajos por turnos, polimedicados o problemas cognitivos. Se desaconsejó el cambio en caso de rash con CBZ u OXC. CONCLUSIÓN: El proyecto EPICON proporciona algunas recomendaciones sobre el manejo clínico del cambio de CBZ u OXC a ESL, mediante el empleo de la metodología Delphi

INTRODUCTION: The objective of the EPICON Project is to develop a set of recommendations on how to adequately switch from carbamazepine (CBZ) and oxcarbazepine (OXC) to eslicarbazepine acetate (ESL) in some patients with epilepsy. METHODS: A steering committee drafted a questionnaire of 56 questions regarding the transition from CBZ or OXC to ESL in clinical practice (methodology and change situation). The questionnaire was then distributed to 54 epilepsy experts in 2 rounds using the Delphi method. An agreement/disagreement consensus was defined when a median ≥ 7 points or ≤ 3 was achieved, respectively, and a relative interquartile range ≤ 0.40. We analysed the results obtained to reach our conclusions. RESULTS: Our main recommendations were the following: switching from CBZ to ESL must be carried out over a period of 1 to 3 weeks with a CBZ:ESL dose ratio of 1:1.3 and is recommended for patients who frequently forget to take their medication, those who work rotating shifts, polymedicated patients, subjects with cognitive problems, severe osteoporosis-osteopaenia, dyslipidaemia, or liver disease other than acute liver failure, as well as for men with erectile dysfunction caused by CBZ. The transition from OXC to ESL can take place overnight with an OXC:ESL dose ratio of 1:1 and it is recommended for patients who frequently forget to take their medication, those who work rotating shifts, polymedicated patients, or those with cognitive problems. The transition was not recommended for patients with prior rash due to CBZ or OXC use. CONCLUSION: The EPICON Project offers a set of recommendations about the clinical management of switching from CBZ or OXC to ESL, using the Delphi method

Evidence for degraded low frequency verbal concepts in left resected temporal lobe epilepsy patients.

According to a large neuropsychological and neuroimaging literature, the bilateral anterior temporal lobe (ATL) is a core region for semantic processing. It seems therefore surprising that semantic memory appears to be preserved in temporal lobe epilepsy (TLE) patients with unilateral ATL resection. However, recent work suggests that the bilateral semantic system is relatively robust against unilateral damage and semantic impairments under these circumstances only become apparent with low frequency specific concepts. In addition, neuroimaging studies have shown that the function of the left and right ATLs differ and therefore left or right ATL resection should lead to a different pattern of impairment. The current study investigated hemispheric differences in the bilateral semantic system by comparing left and right resected TLE patients during verbal semantic processing of low frequency concepts. Picture naming and semantic comprehension tasks with varying word frequencies were included to investigate the pattern of impairment. Left but not right TLE patients showed impaired semantic processing, which was particularly apparent on low frequency items. This indicates that, for verbal information, the bilateral semantic system is more sensitive to damage in the left compared to the right ATL, which is in line with theories that attribute a more prominent role to the left ATL due to connections with pre-semantic verbal regions.

Lacosamide monotherapy in clinical practice: A retrospective chart review.

OBJECTIVE: To assess effectiveness and tolerability of first-line and conversion to lacosamide monotherapy for focal seizures. MATERIALS AND METHODS: Retrospective, non-interventional chart review of lacosamide monotherapy patients aged ≥16 years in Europe. Outcomes included retention rate at observational point (OP) 3 (12 ± 3 months), seizure freedom rates at OP2 (6 ± 3 months) and OP3 and adverse drug reactions (ADRs). RESULTS: A total of 439 patients were included (98 first-line and 341 conversion to monotherapy; 128 aged ≥65 years [25 first-line and 103 conversion to monotherapy]). First-line and conversion to monotherapy retention rates were 60.2% (59/98; 95% confidence interval [CI] 49.8%-70.0%) and 62.5% (213/341; 57.1%-67.6%), respectively. Kaplan-Meier estimates of 12-month retention rates were 81.2% and 91.4% for first-line and conversion to monotherapy, respectively. First-line and conversion to monotherapy retention rates in patients aged ≥65 years were 60.0% (38.7%-78.9%) and 68.9% (59.1%-77.7%), respectively. At OP2, 66.3% of first-line and 63.0% of conversion to monotherapy patients were seizure free. At OP3, 60.2% of first-line and 52.5% of conversion to monotherapy patients were seizure free. In the ≥65 years subgroup, seizure freedom rates at OP2 were 72.0% and 68.0% for first-line and converted to monotherapy, respectively, and at OP3, 68.0% and 56.3%, respectively. Overall, 52 of 439 (11.8%) patients reported ADRs (16.4% in ≥65 years subgroup), most commonly dizziness (5.0%), headache (2.1%) and somnolence (1.6%). CONCLUSIONS: Lacosamide was effective and well tolerated as first-line or conversion to monotherapy in a clinical setting in adult and elderly patients with focal seizures.

EPICON consensus: recommendations for proper management of switching to eslicarbazepine acetate in epilepsy. / Consenso Delphi EPICON: recomendaciones sobre el manejo adecuado del cambio a acetato de eslicarbazepina en epilepsia.

INTRODUCTION: The objective of the EPICON Project is to develop a set of recommendations on how to adequately switch from carbamazepine (CBZ) and oxcarbazepine (OXC) to eslicarbazepine acetate (ESL) in some patients with epilepsy. METHODS: A steering committee drafted a questionnaire of 56 questions regarding the transition from CBZ or OXC to ESL in clinical practice (methodology and change situation). The questionnaire was then distributed to 54 epilepsy experts in 2 rounds using the Delphi method. An agreement/disagreement consensus was defined when a median ≥ 7 points or ≤ 3 was achieved, respectively, and a relative interquartile range ≤ 0.40. We analysed the results obtained to reach our conclusions. RESULTS: Our main recommendations were the following: switching from CBZ to ESL must be carried out over a period of 1 to 3 weeks with a CBZ:ESL dose ratio of 1:1.3 and is recommended for patients who frequently forget to take their medication, those who work rotating shifts, polymedicated patients, subjects with cognitive problems, severe osteoporosis-osteopaenia, dyslipidaemia, or liver disease other than acute liver failure, as well as for men with erectile dysfunction caused by CBZ. The transition from OXC to ESL can take place overnight with an OXC:ESL dose ratio of 1:1 and it is recommended for patients who frequently forget to take their medication, those who work rotating shifts, polymedicated patients, or those with cognitive problems. The transition was not recommended for patients with prior rash due to CBZ or OXC use. CONCLUSION: The EPICON Project offers a set of recommendations about the clinical management of switching from CBZ or OXC to ESL, using the Delphi method.

Efficacy of perampanel in a patient with epilepsia partialis continua.

Perampanel is the first-in-class selective and noncompetitive α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor antagonist. It is authorized in the U.S. and Europe as an add-on antiepileptic drug for partial-onset seizures, and for primary generalized tonic-clonic seizures. Single reports have also indicated a potential efficacy for myoclonic jerks. Here, we report a patient whose drug-resistant epilepsia partialis continua completely resolved after adding perampanel. She has remained seizure-free in an eighteen-month follow-up period. Epilepsia partialis continua reemerged transiently after perampanel was temporarily discontinued, with no recurrence after its reintroduction. Therefore, this effect was reproducible, and suggests that it might be worth trying perampanel in similar settings.