Cognitive rehabilitation program in patients with multiple sclerosis: A pilot study.

INTRODUCTION: In recent years, there has been an increase of studies dedicated to cognitive rehabilitation in patients with multiple sclerosis (MS); however, few of these analyze the impact on such variables as cognitive reserve. The study aims to explore the effects of a cognitive rehabilitation program comprising a combination of cognitive and physical exercises, as well as group sessions to improve cognitive performance, emotional state, and cognitive reserve index. METHOD: Fifty patients with MS were subdivided into 2 groups: the control group, which performed aerobic exercise (n=25), and the experimental group (n=25), which participated in the integrated cognitive rehabilitation program (ICRP). All participants were evaluated 3 times (baseline, post-treatment, and long-term) with the Brief Repeatable Battery of Neuropsychological Tests, Cognitive Reserve Scale, Beck Depression Inventory, and a scale evaluating trait and state anxiety. RESULTS: Compared with the control group, patients in the experimental group showed improvements in cognitive function, with significant changes in measures of information processing speed, attention, memory, cognitive reserve index, and long-term mood. CONCLUSIONS: The ICRP was effective in improving cognitive and emotional function in MS, and increased the cognitive reserve index.

Cognitive rehabilitation program in patients with multiple sclerosis: A pilot study / Programa de rehabilitación cognitiva para pacientes con esclerosis múltiple: estudio piloto

Introduction In recent years, there has been an increase of studies dedicated to cognitive rehabilitation in patients with multiple sclerosis (MS); however, few of these analyze the impact on such variables as cognitive reserve. The study aims to explore the effects of a cognitive rehabilitation program comprising a combination of cognitive and physical exercises, as well as group sessions to improve cognitive performance, emotional state, and cognitive reserve index. Method Fifty patients with MS were subdivided into 2 groups: the control group, which performed aerobic exercise (n = 25), and the experimental group (n = 25), which participated in the integrated cognitive rehabilitation program (ICRP). All participants were evaluated 3 times (baseline, post-treatment, and long-term) with the Brief Repeatable Battery of Neuropsychological Tests, Cognitive Reserve Scale, Beck Depression Inventory, and a scale evaluating trait and state anxiety. Results Compared with the control group, patients in the experimental group showed improvements in cognitive function, with significant changes in measures of information processing speed, attention, memory, cognitive reserve index, and long-term mood. Conclusions The ICRP was effective in improving cognitive and emotional function in MS, and increased the cognitive reserve index. (AU)

Introducción En los últimos años se ha observado un interés creciente por la rehabilitación cognitiva en pacientes con esclerosis múltiple. Sin embargo, pocos estudios han analizado su impacto en variables como la reserva cognitiva. Analizamos el efecto de un programa de rehabilitación cognitiva que incluye ejercicios físicos y cognitivos, así como sesiones en grupo enfocadas a mejorar el rendimiento cognitivo, el estado emocional y el índice de reserva cognitiva. Métodos Nuestro estudio incluyó a 50 pacientes con esclerosis múltiple, divididos en 2 grupos: un grupo control (n = 25), en el que los pacientes realizaban ejercicio aeróbico, y un grupo experimental (n = 25), al que se administró un programa integral de rehabilitación cognitiva. Evaluamos a todos los pacientes en 3 momentos diferentes (al inicio, tras el tratamiento, y a largo plazo) con la Batería Neuropsicológica Breve, la Escala de Reserva Cognitiva, el Inventario de Depresión de Beck y una escala para medir la ansiedad rasgo y la ansiedad estado. Resultados Los pacientes del grupo experimental mostraron un mejor rendimiento cognitivo que los controles, con cambios significativos en medidas de velocidad de procesamiento de la información, atención, memoria, índice de reserva cognitiva y estado de ánimo a largo plazo. Conclusión Nuestros resultados demuestran la eficacia del programa de rehabilitación cognitiva para mejorar las funciones cognitiva y emocional de los pacientes con esclerosis múltiple y aumentar el índice de reserva cognitiva. (AU)

Fumadores y riesgo de muerte hospitalaria por COVID calculado con el procesamiento de lenguaje natural de SAVANA en el ámbito de Castilla-La Mancha / Smokers and risk of hospital death by COVID calculated with SAVANA's natural language processing in the Castilla-La Mancha area

Introducción Durante la pandemia de COVID se especuló que los pacientes con el virus que tenían relación con el tabaco podrían tener una menor probabilidad de agravamiento de la enfermedad o muerte. Para evaluar si existe una asociación entre el tabaquismo y el riesgo de mortalidad intrahospitalaria se utiliza la tecnología de Big Data y Procesamiento del Lenguaje Natural (PLN) de SAVANA. Método Se llevó a cabo un estudio de cohortes retrospectivo, observacional y sin intervención basado en datos de vida real extraídos de registros médicos de toda Castilla-La Mancha utilizando las técnicas de PLN e inteligencia artificial desarrolladas por SAVANA. El estudio abarcó toda la población de esta Comunidad con historia clínica electrónica en SESCAM que presentara diagnóstico de COVID desde el 1 de marzo de 2020 al 28 de febrero de 2021. Resultados Los fumadores tienen mayor porcentaje de factores de riesgo cardiovascular (hipertensión arterial, dislipemia y diabetes), EPOC, asma, EPID, CI, ECV, TEP, cáncer en general y cáncer de pulmón en particular, bronquiectasias, insuficiencia cardíaca y antecedentes de neumonía, de forma significativa (p<0,0001). Los pacientes exfumadores, fumadores y no fumadores tienen una diferencia de edad significativa. En cuanto a las muertes hospitalarias, fueron más frecuentes en el caso de los exfumadores, siguiendo los fumadores y luego los no fumadores (p<0,0001). Conclusión Existe un mayor riesgo de mortalidad intrahospitalaria en los pacientes infectados por SARS-CoV-2 que sean fumadores activos o hayan fumado en el pasado. (AU)

Introduction During the COVID pandemic, it was speculated that patients with the virus who were smoking-related might have a lower likelihood of disease exacerbation or death. To assess whether there is an association between smoking and risk of in-hospital mortality, SAVANA's big data and natural language processing (NLP) technology is used. Method A retrospective, observational, non-interventional cohort study was conducted based on real-life data extracted from medical records throughout Castilla-La Mancha using natural language processing and artificial intelligence techniques developed by SAVANA. The study covered the entire population of this region with Electronic Medical Records in SESCAM presenting with a diagnosis of COVID from March 1, 2020 to February 28, 2021. Results Smokers had a significantly higher percentage of cardiovascular risk factors (hypertension, dyslipidemia and diabetes), COPD, asthma, IDP, IC, CVD, PTE, cancer in general and lung cancer in particular, bronchiectasis, heart failure and a history of pneumonia (P<.0001). Former smokers, current smokers and non-smokers have a significant age difference. As for in-hospital deaths, they were more frequent in the case of ex-smokers, followed by smokers and then non-smokers (P<.0001). Conclusion There is an increased risk of dying in hospital in SARS-CoV-2-infected patients who are active smokers or have smoked in the past. (AU)

Fumadores y riesgo de muerte hospitalaria por COVID calculado con el procesamiento de lenguaje natural de SAVANA en el ámbito de Castilla-La Mancha / Smokers and risk of hospital death by COVID calculated with SAVANA's natural language processing in the Castilla-La Mancha area

Introducción Durante la pandemia de COVID se especuló que los pacientes con el virus que tenían relación con el tabaco podrían tener una menor probabilidad de agravamiento de la enfermedad o muerte. Para evaluar si existe una asociación entre el tabaquismo y el riesgo de mortalidad intrahospitalaria se utiliza la tecnología de Big Data y Procesamiento del Lenguaje Natural (PLN) de SAVANA. Método Se llevó a cabo un estudio de cohortes retrospectivo, observacional y sin intervención basado en datos de vida real extraídos de registros médicos de toda Castilla-La Mancha utilizando las técnicas de PLN e inteligencia artificial desarrolladas por SAVANA. El estudio abarcó toda la población de esta Comunidad con historia clínica electrónica en SESCAM que presentara diagnóstico de COVID desde el 1 de marzo de 2020 al 28 de febrero de 2021. Resultados Los fumadores tienen mayor porcentaje de factores de riesgo cardiovascular (hipertensión arterial, dislipemia y diabetes), EPOC, asma, EPID, CI, ECV, TEP, cáncer en general y cáncer de pulmón en particular, bronquiectasias, insuficiencia cardíaca y antecedentes de neumonía, de forma significativa (p<0,0001). Los pacientes exfumadores, fumadores y no fumadores tienen una diferencia de edad significativa. En cuanto a las muertes hospitalarias, fueron más frecuentes en el caso de los exfumadores, siguiendo los fumadores y luego los no fumadores (p<0,0001). Conclusión Existe un mayor riesgo de mortalidad intrahospitalaria en los pacientes infectados por SARS-CoV-2 que sean fumadores activos o hayan fumado en el pasado. (AU)

Introduction During the COVID pandemic, it was speculated that patients with the virus who were smoking-related might have a lower likelihood of disease exacerbation or death. To assess whether there is an association between smoking and risk of in-hospital mortality, SAVANA's big data and natural language processing (NLP) technology is used. Method A retrospective, observational, non-interventional cohort study was conducted based on real-life data extracted from medical records throughout Castilla-La Mancha using natural language processing and artificial intelligence techniques developed by SAVANA. The study covered the entire population of this region with Electronic Medical Records in SESCAM presenting with a diagnosis of COVID from March 1, 2020 to February 28, 2021. Results Smokers had a significantly higher percentage of cardiovascular risk factors (hypertension, dyslipidemia and diabetes), COPD, asthma, IDP, IC, CVD, PTE, cancer in general and lung cancer in particular, bronchiectasis, heart failure and a history of pneumonia (P<.0001). Former smokers, current smokers and non-smokers have a significant age difference. As for in-hospital deaths, they were more frequent in the case of ex-smokers, followed by smokers and then non-smokers (P<.0001). Conclusion There is an increased risk of dying in hospital in SARS-CoV-2-infected patients who are active smokers or have smoked in the past. (AU)

La señal de la cruz: un automatismo muy poco frecuente en la epilepsia del lóbulo temporal. Descripción de dos casos / The sign of the cross: a very rare automatism in temporal lobe epilepsy. Two case reports

Introducción: En la evaluación de la epilepsia farmacorresistente, el análisis detallado de la semiología es fundamental para establecer una hipótesis diagnóstica de la localización de la zona epileptógena. La señal de la cruz es un automatismo manual complejo muy infrecuente descrito por primera vez en 2008 y poco referido en la bibliografía. Caso clínico: Presentamos dos casos con presencia de la señal de la cruz de nuestra serie de pacientes monitorizados mediante videoelectroencefalograma, uno de ellos estudiado también con electrodos profundos, en los que describimos la localización de la descarga en el momento de realizar la señal de la cruz. Se realiza también una revisión bibliográfica para intentar establecer un valor localizador y/o lateralizador de este signo.(AU)

Introduction: In the evaluation of drug-resistant epilepsy, a detailed analysis of the semiology is essential to establish a diagnostic hypothesis of the location of the epileptogenic zone. Cross-sign (CS) is a very infrequent complex manual automatism described for the first time in 2008 and rarely reported in the literature. Case report: We present two cases from our series of patients monitored by video-EEG, one of whom also studied with deep electrodes, in which we describe the location of the discharge while performing the CS. A bibliographic review is also carried out to try to establish a localizing and/or lateralizing value of this sign. Conclusion: The sign of the cross is a rare ictal automatism that occurs in patients with temporal lobe epilepsy. The hand used to make the sign of the cross is the dominant one, regardless of the origin of the crises.(AU)

[The sign of the cross: a very rare automatism in temporal lobe epilepsy. Two case reports]. / La señal de la cruz: un automatismo muy poco frecuente en la epilepsia del lóbulo temporal. Descripción de dos casos.

INTRODUCTION: In the evaluation of drug-resistant epilepsy (DRF), a detailed analysis of the semiology is essential to establish a diagnostic hypothesis of the location of the epileptogenic zone. Cross-sign (CS) is a very infrequent complex manual automatism described for the first time in 2008 and rarely reported in the literature. CASE REPORT: We present two cases from our series of patients monitored by videoEEG, one of whom also studied with deep electrodes, in which we describe the location of the discharge while performing the CS. A bibliographic review is also carried out to try to establish a localizing and/or lateralizing value of this sign. CONCLUSION: The sign of the cross is a rare ictal automatism that occurs in patients with temporal lobe epilepsy. The hand used to make the sign of the cross is the dominant one, regardless of the origin of the crises.

TITLE: La señal de la cruz: un automatismo muy poco frecuente en la epilepsia del lóbulo temporal. Descripción de dos casos.Introducción. En la evaluación de la epilepsia farmacorresistente, el análisis detallado de la semiología es fundamental para establecer una hipótesis diagnóstica de la localización de la zona epileptógena. La señal de la cruz es un automatismo manual complejo muy infrecuente descrito por primera vez en 2008 y poco referido en la bibliografía. Caso clínico. Presentamos dos casos con presencia de la señal de la cruz de nuestra serie de pacientes monitorizados mediante videoelectroencefalograma, uno de ellos estudiado también con electrodos profundos, en los que describimos la localización de la descarga en el momento de realizar la señal de la cruz. Se realiza también una revisión bibliográfica para intentar establecer un valor localizador y/o lateralizador de este signo. Conclusión. La señal de la cruz es un raro automatismo ictal que ocurre en pacientes con epilepsia del lóbulo temporal. La mano empleada para la señal de la cruz es la dominante, independientemente del origen de las crisis.

Group or individual housing does not reduce socio-sexual and reproductive responses in anestrous goats during the first contact with the photo-stimulated buck.

Herein, we examined the effect of the type of housing (individual vs group housing) of anestrus female goats on plasma cortisol concentrations and socio-sexual behaviors during first contact with photo-stimulated male goats and determined the impact on sexual and reproductive responses after 15 d of contact with photo-stimulated males. Therefore, after weaning, 10 females each were individually and group-housed, respectively. Ten bucks were rendered sexually active by exposure to artificially long days (16 h of light and 8 h of darkness per day) for 2.5 mo. During the naturally increasing photoperiod, 15-mo-old females were exposed and maintained with males. On day 0, during the first contact with males, individually- and group-housed females exhibited similar plasma cortisol concentrations (22.6 vs 27.4 ng/mL, respectively). Likewise, socio-sexual behaviors did not differ between examined groups on day 0. Moreover, the interval from first contact with males to the first estrus, short and normal ovulatory cycles, ovulation, fertility, and prolificacy rates were similar between differently housed females. Furthermore, mounting attempts did not differ between males in contact with either female group. In conclusion, individually- and group-housed anestrus females displayed elevated and similar plasma cortisol concentrations during first contact with photo-stimulated males and similar socio-sexual behaviors and reproductive responses when exposed to photo-stimulated males.

Effects of non-stationary blur on texture biomarkers of bone using Ultra-High Resolution CT.

Purpose: To advance the development of radiomic models of bone quality using the recently introduced Ultra-High Resolution CT (UHR CT), we investigate inter-scan reproducibility of trabecular bone texture features to spatially-variant azimuthal and radial blurs associated with focal spot elongation and gantry rotation. Methods: The UHR CT system features 250×250 µm detector pixels and an x-ray source with a 0.4×0.5 mm focal spot. Visualization of details down to ~150 µm has been reported for this device. A cadaveric femur was imaged on UHR CT at three radial locations within the field-of-view: 0 cm (isocenter), 9 cm from the isocenter, and 18 cm from the isocenter; we expect the non-stationary blurs to worsen with increasing radial displacement. Gray level cooccurrence (GLCM) and gray level run length (GLRLM) texture features were extracted from 237 trabecular regions of interest (ROIs, 5 cm diameter) placed at corresponding locations in the femoral head in scans obtained at the different shifts. We evaluated concordance correlation coefficient (CCC) between texture features at 0 cm (reference) and at 9 cm and 18 cm. We also investigated whether the spatially-variant blurs affect K-means clustering of trabecular bone ROIs based on their texture features. Results: The average CCCs (against the 0 cm reference) for GLCM and GLRM features were ~0.7 at 9 cm. At 18 cm, the average CCCs were reduced to ~0.17 for GLCM and ~0.26 for GLRM. The non-stationary blurs are incorporated in radiomic features of cancellous bone, leading to inconsistencies in clustering of trabecular ROIs between different radial locations: an intersection-over-union overlap of corresponding (most similar) clusters between 0 cm and 9 cm shift was >70%, but dropped to <60% for the majority of corresponding clusters between 0 cm and 18 cm shift. Conclusion: Non-stationary CT system blurs reduce inter-scan reproducibility of texture features of trabecular bone in UHR CT, especially for locations >15 cm from the isocenter. Radiomic models of bone quality derived from UHR CT measurements at isocenter might need to be revised before application in peripheral body sites such as the hips.

Utilidad del modelo 5MPB-Toledo para predecir bacteriemia en el paciente con infección del tracto urinario en el servicio de urgencias / Usefulness of the 5MPB-Toledo model to predict bacteremia in patients with urinary tract infections in the emergency department

Objetivo: Analizar la utilidad de un nuevo modelo predictivo de bacteriemia (5MPB-Toledo) en pacientes atendidos por infección del tracto urinario (ITU) en los servicios de urgencias (SU). Métodos: Estudio observacional, prospectivo y multicéntrico de los hemocultivos (HC) obtenidos en pacientes mayores de 18 años atendidos por ITU en 65SU desde el 1 de octubre de 2019 hasta el 30 de abril de 2020. Se analizó la capacidad predictiva del modelo con el área bajo la curva (ABC) de la característica operativa del receptor (COR) y se calculó el rendimiento diagnóstico del punto de corte elegido con su sensibilidad, especificidad, valor predictivo positivo y valor predictivo negativo. Resultados: Se incluyeron 1.499 episodios de HC extraídos. De ellos se consideraron como bacteriemias verdaderas 277 (18,5%) y como HC negativos 1.222 (81,5%). Entre los negativos 94 (6,3%) se consideraron contaminados. El ABC-COR del modelo fue de 0,937 (IC 95%: 0,926-0,949). El rendimiento diagnóstico del modelo con un PC≥5 puntos consigue una sensibilidad de 97,47% (IC 95%: 94,64-98,89), especificidad de 76,68% (IC 95%: 74,18-79,00), un valor predictivo positivo de 48,65% (IC 95%: 44,42-52,89) y un valor predictivo negativo de 99,26% (IC 95%: 98,41-99,67). Conclusión: El modelo 5MPB-Toledo podría ser útil para predecir bacteriemia en los pacientes adultos atendidos en el SU por un episodio de ITU (AU)

Objective: To analyze the usefulness of a new predictive model of bacteremia (5MPB-Toledo) in patients treated for urinary tract infection (UTI) in the emergency department (ED). Methods: Prospective and multicenter observational cohort study of the blood cultures (BC) ordered for patients with UTIs in 65 Spanish ED from October 1, 2019, to April 30, 2020. The predictive ability of the model was analyzed with the area under the Receiver Operating Characteristic curve (AUC-ROC). The diagnostic performance was calculated with the chosen cut-off point for sensitivity, specificity, positive predictive value, and negative predictive value. Results A total of 1,499 blood cultures were evaluated. True cases of bacteremia were confirmed in 277 (18.5%). The remaining 1,222 cultures (81.5%) were negative. Ninety-four (6.3%) were considered contaminated. The model's area under the ROC curve was 0.937 (95% CI, 0.926-0.949). The prognostic performance with a model's cut-off value of≥5 points achieved 97.47% (95% CI, 94.64-98.89) sensitivity, 76.68% (95% CI, 74.18-79.00) specificity, 48.65% (95% CI, 44.42-52.89) positive predictive value and 99.26% (95% CI, 98.41-99.67) negative predictive value. Conclusion: The 5MPB-Toledo score is useful for predicting bacteremia in patients with UTIs who visit the ED (AU)

Usefulness of the 5MPB-Toledo model to predict bacteremia in patients with urinary tract infections in the emergency department.

OBJECTIVE: To analyze the usefulness of a new predictive model of bacteremia (5MPB-Toledo) in patients treated for urinary tract infection (UTI) in the emergency department (ED). METHODS: Prospective and multicenter observational cohort study of the blood cultures (BC) ordered for patients with UTIs in 65 Spanish ED from November 1, 2019, to March 31, 2020. The predictive ability of the model was analyzed with the area under the Receiver Operating Characteristic curve (AUC-ROC). The diagnostic performance was calculated with the chosen cut-off point for sensitivity, specificity, positive predictive value, and negative predictive value. RESULTS: A total of 1,499 blood cultures were evaluated. True cases of bacteremia were confirmed in 277 (18.5%). The remaining 1,222 cultures (81.5%) were negative. Ninety-four (6.3%) were considered contaminated. The model's area under the ROC curve was 0.937 (95% CI, 0.926-0.949). The prognostic performance with a model's cut-off value of ≥5 points achieved 97.47% (95% CI, 94.64-98.89) sensitivity, 76.68% (95% CI, 74.18-79.00) specificity, 48.65% (95% CI, 44.42-52.89) positive predictive value and 99.26% (95% CI, 98.41-99.67) negative predictive value. CONCLUSION: The 5MPB-Toledo score is useful for predicting bacteremia in patients with UTIs who visit the ED.

Cognitive rehabilitation program in patients with multiple sclerosis: A pilot study.

INTRODUCTION: In recent years, there has been an increase of studies dedicated to cognitive rehabilitation in patients with multiple sclerosis (MS); however, few of these analyze the impact on such variables as cognitive reserve. The study aims to explore the effects of a cognitive rehabilitation program comprising a combination of cognitive and physical exercises, as well as group sessions to improve cognitive performance, emotional state, and cognitive reserve index. METHOD: Fifty patients with MS were subdivided into 2 groups: the control group, which performed aerobic exercise (n=25), and the experimental group (n=25), which participated in the integrated cognitive rehabilitation program (ICRP). All participants were evaluated 3 times (baseline, post-treatment, and long-term) with the Brief Repeatable Battery of Neuropsychological Tests, Cognitive Reserve Scale, Beck Depression Inventory, and a scale evaluating trait and state anxiety. RESULTS: Compared with the control group, patients in the experimental group showed improvements in cognitive function, with significant changes in measures of information processing speed, attention, memory, cognitive reserve index, and long-term mood. CONCLUSIONS: The ICRP was effective in improving cognitive and emotional function in MS, and increased the cognitive reserve index.

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)

Perampanel effectiveness and safety as early add-on treatment for focal-onset seizures: PEREAGAL study.

BACKGROUND: Perampanel (PER) is an effective adjunctive therapy for controlling focal-onset seizures (FOS), but few studies have examined its effects as an early add-on for the treatment of FOS in daily clinical practice. METHODS: Our retrospective, multicenter, observational study evaluated the effectiveness and safety of PER as an early add-on in 77 patients with FOS, with and without focal to bilateral tonic-clonic seizures (FBTCS) after 3, 6 and 12 months in a real-world setting. RESULTS: After 12 months of treatment (median dose 6 [4,8] mg/day), the retention rate was 79.2 % and 60 % of patients (39/65) experienced a ≥50 % reduction in seizure frequency relative to baseline. The seizure-free rate was 38.5 % for all seizures (25/65) and 60 % for FBTCS (12/20). The responder rate at 12 months was significantly higher when PER was given with one concomitant AED (72.2 %) compared to when PER was given with two concomitant AEDs (44.8 %). Drug-related adverse events (AEs) were reported in 40.3 % of patients, most of them being mild (64.2 %). Twelve patients (15.6 %) discontinued treatment because of AEs. CONCLUSIONS: PER is an effective and safe early add-on for patients with refractory FOS, especially for those with FBTCS.