Androgen treatment effects on neurocognition in female-to-male transgender adolescents. / Efectos del tratamiento con andrógenos sobre la neurocognición en adolescentes transgénero de mujer a hombre.

INTRODUCTION: It has been hypothesized that cognitive and memory-related brain function in transgender during cross-sex hormonal treatment might be activated towards that of the subjective gender. However, research on this topic has produced inconsistent results, and to the best of our knowledge no studies have investigated neurocognitive changes in androgen-treated female-to-male (FM) transgender adolescents. SUBJECTS AND METHODS: A total of 15 FM transgender adolescents (14-17 years) underwent neuropsychological testing in order to examine the effects of androgen on visuo-spacial abilities, verbal memory language, processing speed and executive functions. We used a longitudinal design in which 10 participants were tested twice, before and after receiving 12 months of testosterone treatment. This group was also compared with 5 FM transgender adolescents off-androgen treatment. RESULTS: Participants tested before and after 12 months of androgen treatment improved significantly on processing speed in a visuo-spatial (Rey-Osterrieth complex figure test) and in a visuo-oral task (Stroop), their performance on a verbal memory task (TAVEC) and on interference (Stroop) and they exhibited lower impulsivity control (CARAS-R). On-androgen treatment adolescents exhibited worse cognitive impulsivity control than off-androgen treatment adolescents. CONCLUSIONS: The results indicate that androgen has an influence on immediate verbal memory, cognitive interference, impulsivity control and processing speed.

TITLE: Efectos del tratamiento con andrógenos sobre la neurocognición en adolescentes transgénero de mujer a hombre.Introducción. Se ha planteado la hipótesis de que la neurocognición en personas transgénero durante el tratamiento hormonal cruzado podría aproximarse a la del género subjetivo. Sin embargo, la investigación sobre este tema ha producido resultados inconsistentes y, hasta donde sabemos, ningún estudio ha investigado los cambios neurocognitivos en adolescentes transgénero de mujer a hombre (FM) tratados con andrógenos. Sujetos y métodos. Quince adolescentes transgénero FM (14-17 años) se sometieron a pruebas neuropsicológicas para examinar los efectos de los andrógenos en sus habilidades visuoespaciales, memoria verbal, velocidad de procesamiento y funciones ejecutivas. Utilizamos un diseño longitudinal en el que se evaluó a 10 participantes dos veces, antes y después de recibir, durante 12 meses, tratamiento con testosterona. Este grupo también se comparó con cinco adolescentes transgénero FM sin tratamiento con andrógenos. Resultados. Los participantes evaluados antes y después de 12 meses de tratamiento con andrógenos mejoraron significativamente en velocidad de procesamiento en una tarea visuoespacial (prueba de la figura compleja de Rey-Osterrieth) y en una tarea visual (Stroop), en una tarea de memoria verbal (test de aprendizaje verbal España-Complutense) y en interferencia (Stroop), y exhibieron un menor control de la impulsividad (test de percepción de diferencias revisado). Los adolescentes que recibieron tratamiento con andrógenos mostraron un peor control de la impulsividad cognitiva que los adolescentes que no recibieron tratamiento con andrógenos. Conclusiones. Los resultados indican que los andrógenos influyen en la memoria verbal, la interferencia cognitiva, el control de la impulsividad y la velocidad de procesamiento.

Estado nutricional y prevalencia de disfagia en parálisis cerebral infantil. Utilidad del cribado mediante la escala Eating and Drinking Ability Classification System y su relación con el grado de afectación funcional según el Gross Motor Function Classification System / Nutritional status and prevalence of dysphagia in cerebral palsy: Usefulness of the Eating and Drinking Ability Classification System scale and correlation with the degree of motor impairment according to the Gross Motor Function Classification System

Introducción y objetivos: La enfermedad digestiva es una de las comorbilidades más frecuentes en niños con parálisis cerebral infantil (PCI). Nuestro objetivo es analizar el estado nutricional de los pacientes con PCI, la prevalencia de disfagia según la afectación motriz (GMFCS) y su repercusión en la calidad de vida.Material y métodosEstudio descriptivo transversal y abierto en pacientes con PCI seguidos en un hospital terciario de la Comunidad de Madrid mediante una entrevista estructurada y la clasificación de la disfagia según la escala Eating and Drinking Ability Classification System (EDACS). Recogimos datos demográficos y antropométricos y relacionamos el nivel de disfagia con el nivel funcional según el Gross Motor Function Classification System (GMFCS).ResultadosLa muestra incluyó 44 pacientes (65,9% varones), con una edad media de 9,34 ± 5 años y un IMC de 18,5 ± 4,9. El 43% tenía limitaciones en seguridad y/o eficiencia (EDACS > II). El porcentaje de pacientes afectados fue mayor cuanto más extensa desde el punto de vista topográfico fue la PCI (tetraparesia 60%), más variada la semiología clínica (87% en formas mixtas) y peor el nivel funcional (100% en GMFCS V). La repercusión nutricional aumentó con puntuaciones más altas en EDACS y GMFCS.ConclusionesPresentamos el primer estudio sobre la utilidad de la escala EDACS en una muestra representativa de niños y adolescentes españoles con PCI. Los resultados deben hacernos reflexionar sobre la importancia del cribado de disfagia en estos pacientes, independientemente del grado de afectación motriz y la necesidad de una intervención precoz para evitar sus principales consecuencias: desnutrición (hipocrecimiento, déficit de micronutrientes, osteopenia, etc.), microaspiraciones o infecciones de repetición que empeoran el estado neurológico. (AU)

Introduction and objectives: Digestive disorders are one of the most common comorbidities among children with cerebral palsy (CP). The aim of this study is to examine the nutritional status of patients with CP, the prevalence of dysphagia by degree of motor impairment, and the impact of digestive disorders on quality of life.Material and methodsWe conducted a descriptive, cross-sectional, open-label study of outpatients with CP from a tertiary hospital in the Region of Madrid using a structured interview, classifying dysphagia using the Eating and Drinking Ability Classification System (EDACS). We gathered demographical and anthropometric data, and analysed the correlation between severity of dysphagia and functional status as measured with the Gross Motor Function Classification System (GMFCS).ResultsOur sample included 44 patients (65.9% boys), with a mean (standard deviation) age of 9.34 (5) years and a mean BMI of 18.5 (4.9). Forty-three percent presented safety and efficiency limitations (EDACS level > II). Safety and efficiency limitations were associated with more extensive motor involvement (60% had tetraparesis), more varied clinical manifestations (87% had mixed forms) and poorer functional capacity (100% on GMFCS V). The impact on nutritional status increased with higher EDACS and GMFCS scores.ConclusionsThis is the first study into the usefulness of the EDACS scale in a representative sample of Spanish children and adolescents with CP. Our findings underscore the importance of screening for dysphagia in these patients, regardless of the level of motor impairment, and the need for early treatment to prevent the potential consequences: malnutrition (impaired growth, micronutrient deficiencies, osteopaenia, etc.), microaspiration, or recurrent infections that may worsen patients’ neurological status. (AU)

Nutritional status and prevalence of dysphagia in cerebral palsy: usefulness of the Eating and Drinking Ability Classification System scale and correlation with the degree of motor impairment according to the Gross Motor Function Classification System.

INTRODUCTION AND OBJECTIVES: Digestive disorders are one of the most common comorbidities among children with cerebral palsy (CP). The aim of this study is to examine the nutritional status of patients with CP, the prevalence of dysphagia by degree of motor impairment, and the impact of digestive disorders on quality of life. MATERIAL AND METHODS: We conducted a descriptive, cross-sectional, open-label study of out-patients with CP from a tertiary hospital in the Region of Madrid using a structured interview, classifying dysphagia using the Eating and Drinking Ability Classification System (EDACS). We gathered demographical and anthropometric data, and analysed the correlation between severity of dysphagia and functional status as measured with the Gross Motor Function Classification System (GMFCS). RESULTS: Our sample included 44 patients (65.9% boys), with a mean (standard deviation) age of 9.34 (5) years and a mean BMI of 18.5 (4.9). Forty-three percent presented safety and efficiency limitations (EDACS level > II). Safety and efficiency limitations were associated with more extensive motor involvement (60% had tetraparesis), more varied clinical manifestations (87% had mixed forms) and poorer functional capacity (100% on GMFCS V). The impact on nutritional status increased with higher EDACS and GMFCS scores. CONCLUSIONS: This is the first study into the usefulness of the EDACS scale in a representative sample of Spanish children and adolescents with CP. Our findings underscore the importance of screening for dysphagia in these patients, regardless of the level of motor impairment, and the need for early treatment to prevent the potential consequences: malnutrition (impaired growth, micronutrient deficiencies, osteopaenia, etc.), microaspiration, or recurrent infections that may worsen patients' neurological status.

Utilidad de la telemedicina en la atención pediátrica urgente durante la pandemia por COVID-19 / Usefulness of telemedicine in urgent pediatric care during the COVID-19 pandemic

Introducción: nuestro sistema sanitario ha sufrido una reorganización sin precedentes priorizando la atención de los pacientes con sintomatología COVID-19. El uso de telemedicina se presenta como una alternativa útil en la era pos-COVID. El objetivo del estudio fue valorar la utilidad del servicio de mensajería de Twitter como herramienta de telemedicina para el cribado de patología urgente. Material y métodos: estudio descriptivo, retrospectivo y transversal de un programa de telemedicina desarrollado por un equipo de especialistas en Pediatría y sus Áreas Específicas durante el estado de alarma. Se recogieron datos demográficos, número y motivos de consultas según signos, síntomas y su forma de presentación (texto, foto o vídeo). Se analizó el número de consultas resueltas, derivaciones y el grado de satisfacción. Resultados: se atendió un total de 182 consultas realizadas en su mayoría por mujeres (71%), durante las primeras semanas del confinamiento (70%). El 100% fueron mensajes de texto, acompañados casi en un tercio de los casos de material audiovisual (27,2% fotos, 4,6% vídeos). La edad media de los pacientes atendidos fue de 2,72 ± 2,74 y los principales motivos de consulta: fiebre, exantemas y dificultad respiratoria. El 18,13% tuvo relación con la COVID-19, y solamente el 8,24% fue derivado. Conclusiones: aunque la telemedicina no puede reemplazar la valoración presencial y todavía existen limitaciones técnicas y legales, nuestros resultados sugieren que podría ser una alternativa prometedora para mejorar el acceso, reducir los tiempos de triaje, coordinar los recursos disponibles, y disminuir el riesgo de contagio y saturación de las instalaciones sanitarias (AU)

Introduction: our healthcare system has undergone an unprecedented reorganization, prioritizing the care of patients with COVID-19 symptoms. Telemedicine has emerged as a useful alternative in the post-COVID era. The aim of the study was to assess the usefulness of the Twitter® messaging service as a telemedicine tool for the screening of urgent pathology.Material and methods: cross-sectional, retrospective and descriptive study of a telemedicine programme developed by a team of specialists in paediatrics and its subspecialities during the state of alarm. We collected demographic data and the number and reasons for consultations based on the presenting signs and symptoms and how they were conveyed (text, photo and/or video). We analysed the number of resolved concerns, referrals and the degree of user satisfaction.Results: the service managed a total of 182 consultations, mostly made by women (71%) and during the first weeks of the survey (70%). All consultations included text, accompanied in almost 1/3 of the cases by audiovisual content (27.2% photo, 4.6% video). The average age of the managed patients was 2.72 ± 2.74 years and the main reasons for consultation were fever, exanthema and respiratory difficulty. Of all consultations, 18.13% were related to COVID-19, and only 8.24% led to referral.Conclusions: although telemedicine cannot replace face-to-face assessment and there are still technical and legal limitations, our results suggest that it could be a promising alternative to improve access, reduce triage times, coordinate available resources, and decrease the risk of contagion and the saturation of health care facilities. (AU)

Nutritional status and prevalence of dysphagia in cerebral palsy: Usefulness of the Eating and Drinking Ability Classification System scale and correlation with the degree of motor impairment according to the Gross Motor Function Classification System. / Estado nutricional y prevalencia de disfagia en parálisis cerebral infantil. Utilidad del cribado mediante la escala Eating and Drinking Ability Classification System y su relación con el grado de afectación funcional según el Gross Motor Function Classification System.

INTRODUCTION AND OBJECTIVES: Digestive disorders are one of the most common comorbidities among children with cerebral palsy (CP). The aim of this study is to examine the nutritional status of patients with CP, the prevalence of dysphagia by degree of motor impairment, and the impact of digestive disorders on quality of life. MATERIAL AND METHODS: We conducted a descriptive, cross-sectional, open-label study of outpatients with CP from a tertiary hospital in the Region of Madrid using a structured interview, classifying dysphagia using the Eating and Drinking Ability Classification System (EDACS). We gathered demographical and anthropometric data, and analysed the correlation between severity of dysphagia and functional status as measured with the Gross Motor Function Classification System (GMFCS). RESULTS: Our sample included 44 patients (65.9% boys), with a mean (standard deviation) age of 9.34 (5) years and a mean BMI of 18.5 (4.9). Forty-three percent presented safety and efficiency limitations (EDACS level>II). Safety and efficiency limitations were associated with more extensive motor involvement (60% had tetraparesis), more varied clinical manifestations (87% had mixed forms) and poorer functional capacity (100% on GMFCS V). The impact on nutritional status increased with higher EDACS and GMFCS scores. CONCLUSIONS: This is the first study into the usefulness of the EDACS scale in a representative sample of Spanish children and adolescents with CP. Our findings underscore the importance of screening for dysphagia in these patients, regardless of the level of motor impairment, and the need for early treatment to prevent the potential consequences: malnutrition (impaired growth, micronutrient deficiencies, osteopaenia, etc.), microaspiration, or recurrent infections that may worsen patients' neurological status.

Utilidad del tratamiento como uso compasivo de mecasermina (factor de crecimiento insulínico recombinante humano tipo 1) en ataxia de Friedreich / Compassionate use of human recombinant insulin-like growth factor-1 therapy in Friedreich's ataxia

No disponible

Utilidad del tratamiento con infiltraciones ecoguiadas de toxina botulínica A en el desequilibrio muscular de niños con parálisis obstétrica del plexo braquial. Descripción del procedimiento y protocolo de actuación / Utility of ultrasound-guided injection of botulinum toxin type A for muscle imbalance in children with obstetric brachial plexus palsy: Description of the procedure and action protocol

Introducción: La parálisis braquial obstétrica (PBO) suele tener un pronóstico favorable; sin embargo, casi un tercio de los casos graves presentan secuelas permanentes que generan gran discapacidad. Valoramos la eficacia del tratamiento con infiltraciones ecoguiadas de toxina botulínica A (TB-A) y describimos el procedimiento. Pacientes y métodos: Estudio prospectivo, descriptivo, de los casos de PBO moderada-grave tratados entre enero del 2010 y diciembre del 2014. Recogimos datos demográficos, tipo de PBO y evolución. Valoramos eficacia con la escala de movimiento activo (EMA), Mallet Classification System y videofilmación. Resultados: Valoramos a 14.133 recién nacidos vivos, con 15 casos de PBO (1,06 por cada 1.000). El 40% casos graves (0,4/1000), nacidos de parto distócico, con APGAR < 5 y peso medio de 4.038 g. Edad media de inicio de infiltraciones 11,5 meses. El pronator teres, subescapularis, teres major, latissimus dorsi y/o pectoralis major fueron los más frecuentemente infiltrados. Mejoría progresiva hasta 3 niveles en EMA y una media de 19,5 puntos sobre 25 en la escala de Mallet a los 2 años en todos los casos de PBO que completaron el seguimiento (83%). Todos mejoraron funcionalidad y posturas anormales. Evitamos la cirugía en 3 pacientes y se retrasó en uno. Los efectos adversos del tratamiento fueron leves y autolimitados. Conclusiones: Por su seguridad y eficacia, parece razonable la utilización "off label" de TB-A como tratamiento adyuvante a las terapias físicas y/o tratamiento quirúrgico en las PBO moderadas-graves. La ecografía podría aumentar la eficacia y disminuir efectos adversos

Introduction: Obstetric brachial plexus palsy (OBPP) usually has a favourable prognosis. However, nearly one third of all severe cases have permanent sequelae causing a high level of disability. In this study, we explore the effectiveness of ultrasound-guided injection of botulinum toxin A (BoNT-A) and describe the procedure. Patients and methods: We designed a prospective, descriptive study including patients with moderate to severe OBPP who were treated between January 2010 and December 2014. We gathered demographic data, type of OBPP, and progression. Treatment effectiveness was assessed with the Active Movement Scale (AMS), the Mallet classification, and video recordings. Results: We gathered a total of 14 133 newborns, 15 of whom had OBPP (1.6 per 1000 live births). Forty percent of the cases had severe OBPP (0.4/1000), a dystocic delivery, and APGAR scores < 5; mean weight was 4038 g. Mean age at treatment onset was 11.5 months. The muscles most frequently receiving BoNT-A injections were the pronator teres, subscapularis, teres major, latissimus dorsi, and pectoralis major. All the patients who completed the follow-up period (83%) experienced progressive improvements: up to 3 points on the AMS and a mean score of 19.5 points out of 25 on the Mallet classification at 2 years. Treatment improved muscle function and abnormal posture in all cases. Surgery was avoided in 3 patients and delayed in one. Adverse events were mild and self-limited. Conclusions: Due to its safety and effectiveness, BoNT-A may be used off-label as an adjuvant to physical therapy and/or surgery in moderate to severe OBPP. Ultrasound may increase effectiveness and reduce adverse effects

Utility of ultrasound-guided injection of botulinum toxin type A for muscle imbalance in children with obstetric brachial plexus palsy: Description of the procedure and action protocol. / Utilidad del tratamiento con infiltraciones ecoguiadas de toxina botulínica A en el desequilibrio muscular de niños con parálisis obstétrica del plexo braquial. Descripción del procedimiento y protocolo de actuación.

INTRODUCTION: Obstetric brachial plexus palsy (OBPP) usually has a favourable prognosis. However, nearly one third of all severe cases have permanent sequelae causing a high level of disability. In this study, we explore the effectiveness of ultrasound-guided injection of botulinum toxin A (BoNT-A) and describe the procedure. PATIENTS AND METHODS: We designed a prospective, descriptive study including patients with moderate to severe OBPP who were treated between January 2010 and December 2014. We gathered demographic data, type of OBPP, and progression. Treatment effectiveness was assessed with the Active Movement Scale (AMS), the Mallet classification, and video recordings. RESULTS: We gathered a total of 14 133 newborns, 15 of whom had OBPP (1.6 per 1000 live births). Forty percent of the cases had severe OBPP (0.4/1000), a dystocic delivery, and APGAR scores < 5; mean weight was 4038g. Mean age at treatment onset was 11.5 months. The muscles most frequently receiving BoNT-A injections were the pronator teres, subscapularis, teres major, latissimus dorsi, and pectoralis major. All the patients who completed the follow-up period (83%) experienced progressive improvements: up to 3 points on the AMS and a mean score of 19.5 points out of 25 on the Mallet classification at 2 years. Treatment improved muscle function and abnormal posture in all cases. Surgery was avoided in 3 patients and delayed in one. Adverse events were mild and self-limited. CONCLUSIONS: Due to its safety and effectiveness, BoNT-A may be used off-label as an adjuvant to physical therapy and/or surgery in moderate to severe OBPP. Ultrasound may increase effectiveness and reduce adverse effects.

Estudio piloto de la eficacia de empoderar a pacientes mediante coaching como tratamiento asociado en el trastorno por déficit de atención e hiperactividad / Pilot study of the efficacy of empowering patients through coaching as a complementary therapy in attention deficit hyperactivity disorder

Introducción: El trastorno por déficit de atención e hiperactividad (TDAH) es el trastorno del neurodesarrollo más frecuente y debe ser considerado un problema de primer orden de salud pública por sus repercusiones funcionales a nivel escolar, familiar y social. Empoderar en salud es un modelo innovador en el cuidado de pacientes con enfermedades crónicas, basado en la educación de automanejo. Nuestro objetivo es valorar la eficacia de empoderar mediante coaching, dentro de un plan de tratamiento multimodal en pacientes pediátricos con TDAH. Material y métodos: estudio descriptivo, abierto y prospectivo. Incluimos a niños de entre 6 y 12 años pertenecientes a una asociación de pacientes de un área suburbana de la Comunidad de Madrid. Valoramos la situación previa y posterior a 5 sesiones gratuitas de coaching mediante el Cuestionario de conducta de Conners de dificultades a lo largo del día (D-DTODS) y escalas de satisfacción. Resultados: Incluimos a 49 pacientes, el 73,5% varones, con una edad media de 8,5 años. El 63,3% tenía TDAH subtipo hiperactivo/impulsivo y el 77,6% algún tipo de comorbilidad. Todos tratados con metilfenidato y mala evolución clínica. El 79,6% mejoró clínicamente, con una reducción media ± DT de los síntomas del 34,6 ± 11,1% y mantenida en el 79,6% tras 6 meses de seguimiento post coaching. Alcanzamos un nivel de satisfacción de 7,8 ± 1,7 sobre 10 y el 95,9% recomendó el tratamiento a otras familias. Conclusiones: Nuestros resultados aportan información sobre los posibles beneficios del coaching como tratamiento asociado en el TDAH

Introduction: Attention deficit hyperactivity disorder (ADHD) is the most frequent neurodevelopmental disorder and must be considered a public health priority because of its functional repercussions in school, family, and social settings. Health empowerment is an innovative model of care for patients with chronic diseases based on self-management education. Our goal is to evaluate the effectiveness of empowerment using coaching within a multimodal treatment plan in paediatric patients with ADHD. Material and methods: Descriptive open prospective study. We included children between 6 and 12 years old belonging to patient association in a suburban area of the Region of Madrid. We evaluated the situation before and after 5 cost-free coaching sessions using the Conners Questionnaire, Dundee difficult times of day scale, and satisfaction scales. Results: We included 49 patients (73.5% males) with an average age of 8.5 years. The ADHD hyperactive-impulsive subtype was present in 63.3% and 77.6% had some type of comorbidity. All were treated with methylphenidate and their clinical course was poor. Clinical improvements were observed in 79.6% with a 34.6% mean reduction in symptoms (SD 11.1), and improvements remained stable at 6 months follow-up after coaching. We reached a satisfaction level of 7.8 out of 10 (SD 1.7), and 95.9% of the participants recommended this treatment to other families. Conclusions: Our results provide information on the potential benefits of coaching as complementary treatment for ADHD

Pilot study of the efficacy of empowering patients through coaching as a complementary therapy in attention deficit hyperactivity disorder.

INTRODUCTION: Attention deficit hyperactivity disorder (ADHD) is the most frequent neurodevelopmental disorder and must be considered a public health priority because of its functional repercussions in school, family, and social settings. Health empowerment is an innovative model of care for patients with chronic diseases based on self-management education. Our goal is to evaluate the effectiveness of empowerment using coaching within a multimodal treatment plan in paediatric patients with ADHD. MATERIAL AND METHODS: Descriptive open prospective study. We included children between 6 and 12 years old belonging to patient association in a suburban area of the Region of Madrid. We evaluated the situation before and after 5 cost-free coaching sessions using the Conners Questionnaire, Dundee difficult times of day scale, and satisfaction scales. RESULTS: We included 49 patients (73.5% males) with an average age of 8.5 years. The ADHD hyperactive-impulsive subtype was present in 63.3% and 77.6% had some type of comorbidity. All were treated with methylphenidate and their clinical course was poor. Clinical improvements were observed in 79.6% with a 34.6% mean reduction in symptoms (SD 11.1), and improvements remained stable at 6 months follow-up after coaching. We reached a satisfaction level of 7.8 out of 10 (SD 1.7), and 95.9% of the participants recommended this treatment to other families. CONCLUSIONS: Our results provide information on the potential benefits of coaching as complementary treatment for ADHD.

Tratamiento farmacológico estimulante del TDAH / Stimulant drug treatment

El trastorno por déficit de atención e hiperactividad (TDAH) es el trastorno del neurodesarrollo más frecuente en la infancia. Es un trastorno cognitivo-conductual con una base genética y neurobiológica (neuroanatómica, neuroquímica y neuropsicológica) bien establecida y una sintomatología clínica muy heterogénea aunque identificable a través de unos síntomas nucleares de inatención, que evolucionan a lo largo de la vida. Debe ser considerado un problema de primer nivel individual (académicas, laborales y sociales) y socioeconómico ( elevado riesgo de abuso de sustancias, exclusión social e incluso criminalidad). Por tanto, su diagnóstico y tratamiento precoz son de vital importancia en el pronóstico a largo plazo del mismo. El tratamiento debe ser individualizado y ajustarse a las necesidades del individuo según su edad, gravedad de los síntomas, repercusión funcional e incluso preferencias del paciente y su familia. Sin embargo, diversos estudios demuestran que, de forma aislada y salvo excepciones, el tratamiento farmacológico tanto con psico-estimulantes como con fármacos no estimulante es el más eficaz. Los psico-estimulantes tienen un tamaño de efecto mayor que los no psico-estimulantes y un porcentaje de éxito entre el 70-80% de los pacientes en los que se inicia, mostrando eficacia y seguridad contrastada desde hace muchos años. las formas de liberación prolongada son, a priori, nuestra primera elección pues facilitan el cumplimiento terapéutico con un efecto más estable a lo largo del día y menor riego de abuso. En este artículo revisaremos las características, indicaciones, dosis y efectos adversos del tratamiento farmacológico del TDAH con psicoestimulantes (metilfenidato, lisdexanfetamina, etc.) (AU)

Attention Deficit Hyperactivity Disorder (ADHD) is the most frequent childhood and adolescence neurodevelopment disorder. ADHD is a neurocognitive disorder with a well-established genetic and neurobiological basis (neuroanatomical, neurochemical, and neuropsychological). The core behavioral symptoms of ADHD are inattention, hyperactivity, and impulsivity which evolve over the life. Considering the individual (academic, job, social) and socioeconomic consequences (e.g. risk of substance abuse, social exclusion, or even criminality), ADHD should be considered a mayor health issue. Therefore an early diagnosis and treatment are of primary importance to make long-term prognosis even better. Treatment should be individualized and adjusted to the individual characteristics (age, severity of symptoms, functional impairment, or the preferences of the patient or their relatives). Multimodal treatment is often the best way to treat, being pharmacological intervention with stimulants or non-stimulants drugs, the treatment of choice in children and adolescents with ADHD. Stimulants have a larger effect size than non-stimulants, being around 70-80% responders and the safety profile is acceptable and widely known for years. The use of extended-release formulations are preferable due to several advantages such as a lower risk of abuse, easy dosing and administration, lower patient variability and uniform action throughout the day. The current study reviews the characteristics, indications, doses, and side effects of stimulants on ADHD (AU)

Influencia de la comorbilidad médica y neuropsiquiátrica en la evolución de la epilepsia infantil / Influence of medical and neuropsychiatric comorbidities on the course of childhood epilepsy

INTRODUCCIÓN: El creciente reconocimiento de la comorbilidad y su carga plantea la necesidad de incluir en el manejo de los pacientes con epilepsia su investigación, prevención y tratamiento. MATERIAL Y MÉTODOS: Estudio descriptivo de todos los pacientes con epilepsia, perteneciente a un área suburbana de la Comunidad de Madrid, seguidos en consulta al menos durante un año. Excluimos a menores de 2 años, las crisis febriles y sintomáticas agudas. RESULTADOS: Incluimos a 46 pacientes (54% varones y edad media 9,1 años). El 52,5% en monoterapia. El 45,7% «libre de crisis», el 23,9% epilepsia «farmacorresistente» y el 30,4% «indeterminada». El 28,3% tenía patología médica crónica asociada y un 41,3% neuropsiquiátrica. El 32,6% acudió de manera imprevista por crisis, con un riesgo de 15 y 8,3 veces mayor aquellos con comorbilidad médica crónica y neuropsiquiátrica respecto al de los pacientes sin comorbilidades. CONCLUSIONES: La comorbilidad puede desempeñar un papel importante en el curso de la epilepsia

INTRODUCTION: Comorbidity has a significant influence in the management of patients with epilepsy. MATERIAL AND METHODS: A descriptive study of all patients with epilepsy, from a suburban area in the Community of Madrid followed up for at least 1 year. Children under 2 years, those with symptomatic acute febrile seizures were excluded. RESULTS: Out of a total of 46 patients (54% male, age median 9.1 years), more than half (52.5%) were on monotherapy, 45.7% were ''free of seizures'', 23.9% had ''drug resistant epilepsy'', and 30.4% were ''undetermined''. As regards comorbidities, 28.3% had chronic medical conditions, and 41.3% associated neuropsychiatric disorders. In32.6%, the seizures were of sudden onset, and those with chronic medical and neuropsychiatric comorbidities had a risk of 15 and 8.3 times, respectively, than those patients without comorbidities. CONCLUSIONS: Comorbidities may have an important role in the course of epilepsy

[Influence of medical and neuropsychiatric comorbidities on the course of childhood epilepsy]. / Influencia de la comorbilidad médica y neuropsiquiátrica en la evolución de la epilepsia infantil.

INTRODUCTION: Comorbidity has a significant influence in the management of patients with epilepsy. MATERIAL AND METHODS: A descriptive study of all patients with epilepsy, from a suburban area in the Community of Madrid followed up for at least 1 year. Children under 2 years, those with symptomatic acute febrile seizures were excluded. RESULTS: Out of a total of 46 patients (54% male, age median 9.1 years), more than half (52.5%) were on monotherapy, 45.7% were "free of seizures", 23.9% had "drug resistant epilepsy", and 30.4% were "undetermined". As regards comorbidities, 28.3% had chronic medical conditions, and 41.3% associated neuropsychiatric disorders. In32.6%, the seizures were of sudden onset, and those with chronic medical and neuropsychiatric comorbidities had a risk of 15 and 8.3 times, respectively, than those patients without comorbidities. CONCLUSIONS: Comorbidities may have an important role in the course of epilepsy.

[Current situation of the demand for health care in neuropaediatrics. Characteristics of consultations and comparison with other paediatric specialties]. / Situacion actual de la demanda asistencial en neuropediatria. Caracteristicas de la consulta y comparacion con otras especialidades pediatricas.

INTRODUCTION: In recent years it seems we are witnessing an increasing demand for neuropaediatric care. Epidemiological studies are needed to make this demand more widely known and thus promote appropriate management of health care resources. AIMS: To determine what proportion of all visits to the paediatric department in our hospital are neuropaediatric consultations, the annual consultation rate in child neurology per 1,000 inhabitants under 14 years of age, and the characteristics of that consultation (demographic data, reasons for the visit and others). PATIENTS AND METHODS: We conducted a retrospective, descriptive study on the health care activity of paediatric and neuropaediatric units in a level-II public hospital in the south of Madrid, over the period 2008-2012. RESULTS: Since our centre opened, the number of paediatric consultations has increased sharply, neuropaediatric visits being the most frequently demanded. In the year 2012 a total of 2,129 patients were seen (718 first visits), with a successive/first visit index of 1.96. Of all the paediatric consultations carried out in the hospital, 23.49% took place in neuropaediatrics. The mean rate of first visits in the period under study was 72.86/1,000 children. The main reasons for the consultation were learning disabilities/conduct disorders (24.1%), followed by headaches (21.9%), paroxysmal episodes (14.8%) and delayed psychomotor development (9%). CONCLUSIONS: The increase in demand for neuropaediatrics health care was clearly higher than that of other paediatric specialities over the same period of time. In the five years included in the study, the rate of first visits increased threefold. This health care overload could condition the care dispensed to patients with severe neurological pathologies. Further studies of a similar nature in different regions are required to determine the real situation of neuropaediatrics in Spain.

TITLE: Situacion actual de la demanda asistencial en neuropediatria. Caracteristicas de la consulta y comparacion con otras especialidades pediatricas.Introduccion. En los ultimos años parecemos asistir a una creciente demanda asistencial en neuropediatria. Los estudios epidemiologicos son necesarios para dar a conocer dicha demanda y asi favorecer una adecuada gestion de los recursos sanitarios. Objetivo. Conocer el peso proporcional de las consultas de neuropediatria en el global de las consultas pediatricas en nuestro hospital, la tasa anual de consulta en neurologia infantil por cada 1.000 habitantes menores de 14 años y las caracteristicas de dicha consulta (datos demograficos, motivos de consulta y otras). Pacientes y metodos. Estudio retrospectivo, descriptivo, sobre la actividad asistencial de consultas pediatricas y neuropediatricas en un hospital publico de nivel II en el sur de Madrid, durante el periodo 2008-2012. Resultados. Desde la apertura de nuestro centro, las consultas de pediatria han experimentado un marcado crecimiento, siendo las de neuropediatria las mas demandadas, ya que en el año 2012 atendieron a un total de 2.129 pacientes (718 primeras consultas), con un indice de sucesiva/primera consulta de 1,96. En neuropediatria, se atendieron el 23,49% de todas las consultas pediatricas realizadas en el hospital. La tasa media de primeras consultas en el periodo de estudio fue de 72,86/1.000 niños. Los principales motivos de consulta fueron los problemas de aprendizaje/trastornos de conducta (24,1%), seguidos de cefalea (21,9%), episodios paroxisticos (14,8%) y retraso del desarrollo psicomotor (9%). Conclusiones. El incremento en la demanda asistencial de la neuropediatria ha resultado claramente superior al de las otras especialidades pediatricas que llevan en funcionamiento el mismo periodo. En los cinco años de estudio, la tasa de primeras visitas se ha triplicado. Esta sobrecarga asistencial podria condicionar la atencion a los pacientes con patologia neurologica grave. Serian necesarios estudios similares en diferentes regiones para conocer la realidad de la neuropediatria española.

Eficacia y seguridad de la toxina botulínica en el tratamiento de la sialorrea en niños con diversas alteraciones neurológicas / Efficacy and safety of botulinum toxin in the treatment of sialorrhea in children with neurological disorders

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Síndrome de Marin-Amat y Marcus-Gunn invertido. Presentación de 2 casos de sincinesia facial / Marin-Amat and inverted Marcus-Gunn syndrome. Two case report

El síndrome de Marin-Amat es una sincinesia trigémino-facial generalmente adquirida, debida a una regeneración aberrante del nervio facial que produce una contracción simultánea de orbicular de los párpados y los pterigoideos externos. Clínicamente, se manifiesta con blefarospasmo al abrir la boca o sonreír. Existen casos congénitos conocidos como fenómeno de Marcus-Gunn invertido por tener clínica inversa, ptosis palpebral con la apertura bucal, pero con la misma sincinesia (III-VII). Presentamos 2 casos clínicos, uno adquirido secundario a cirugía de una adenitis cervical tuberculosa y otro congénito, de forma gráfica y haciendo referencia a la forma de presentación, evolución y el tratamiento. Concluimos que es una entidad infrecuente en pediatría, con pocos casos descritos, aunque probablemente infradiagnosticada por su desconocimiento. El diagnóstico es esencialmente clínico y no precisa pruebas complementarias para su confirmación, si bien, la electromiografía puede ser de gran utilidad para demostrar la sincinesia (AU)

Marin-Amat’s syndrome is a rare associated movement, wherein contraction of or bicularisoculi is brought about by opening of the jaw in association with aberrant regeneration of facial nerve. This is the opposite of the Marcus-Gunn phenomenon; hence it has also been called Inverted Marcus-Gunn Phenomenon. Moreover in this case the opened the mouth or moved the jaw, the eye would close. This phenomenon is congenital and the closure of the eye is due to inhibition of the levator palpebrae superior is. We present two cases, one acquired after the surgery of tuberculosus cervical adenitis and other congenital with reference to the presentation, course and treatment. This entity is rare in children, with few reported cases, although probably will be found more frequently if looked for carefully. The diagnosis is clinical and does not require additional tests, although EMG may be useful to demonstrate the synkinesis (AU)

[Marin-Amat and inverted Marcus-Gunn syndrome. Two case reports]. / Síndrome de Marin-Amat y Marcus-Gunn invertido. Presentación de 2 casos de sincinesia facial.

Marin-Amat's syndrome is a rare associated movement, wherein contraction of orbicularis oculi is brought about by opening of the jaw in association with aberrant regeneration of facial nerve. This is the opposite of the Marcus-Gunn phenomenon; hence it has also been called Inverted Marcus-Gunn Phenomenon. Moreover in this case the opened the mouth or moved the jaw, the eye would close. This phenomenon is congenital and the closure of the eye is due to inhibition of the levator palpebrae superioris. We present two cases, one acquired after the surgery of tuberculosus cervical adenitis and other congenital with reference to the presentation, course and treatment. This entity is rare in children, with few reported cases, although probably will be found more frequently if looked for carefully. The diagnosis is clinical and does not require additional tests, although EMG may be useful to demonstrate the synkinesis.

Acidosis láctica secundaria a inhalación de dosis elevadas de salbutamol / No disponible

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