[Use of benzodiazepines in prolonged seizures and status epilepticus in the community]. / Uso benzodiacepinas en crisis prolongadas y estado epiléptico en la comunidad.

Prolonged seizures and status epilepticus are common neurological medical emergencies. Early and appropriate treatment is essential to reduce morbidity and mortality. Most seizures occur in the community, so parents and caregivers must be prepared for their management. Benzodiazepines (BZD) are the first-line drugs used, with rectal diazepam (DZPr) being the most commonly used in pre-hospital treatment in Spain. In September 2011, the European Medicines Agency (EMA) authorized the use of oromucosal midazolam (MDZb) for the treatment of prolonged acute convulsive seizures in patients aged 3 months to <18 years. MDZb has a rapid onset, short duration of effect, and avoids first-pass hepatic metabolism. MDZb has shown to be at least as or more effective than DZPr to stop the seizures. Buccal administration is easier and more socially accepted, especially in adolescents and adults. It is a safe drug with similar effects to other BZD; MDZb improves the overall cost-effectiveness of seizures management.

Uso benzodiacepinas en crisis prolongadas y estado epiléptico en la comunidad / Use of benzodiazepines in prolonged seizures and status epilepticus in the community

Las crisis prolongadas y el estado epiléptico son emergencias médicas neurológicas frecuentes y su tratamiento adecuado y precoz es fundamental para reducir su morbi-mortalidad. La mayoría de las crisis se inician en un medio extrahospitalario, lo que obliga a familiares y cuidadores a estar preparados en las medidas de actuación iniciales ante una crisis convulsiva. En su manejo, las benzodiacepinas (BZD) son los fármacos de primera línea, siendo el uso de diazepam rectal (DZPr) el más extendido en el ámbito prehospitalario en España. La Agencia Europea del Medicamento (EMA) autorizó en septiembre del 2011 el empleo de midazolam bucal (MDZb) para el manejo de las crisis epilépticas repetidas en pacientes entre los 3 meses y 18 años. Es un fármaco de acción rápida por evitar la metabolización hepática y con efecto de corta duración. MDZb ha demostrado al menos igual o mayor eficacia que el DZPr y la vía de administración bucal es más sencilla y mejor aceptada socialmente, sobre todo en adolescentes y adultos. Es un fármaco seguro, con efectos adversos similares a otras BZD. Estudios de farmacoeconomía demuestran un buen coste-efectividad global en el manejo de las crisis frente al DZPr, reduciendo traslados en ambulancia e ingresos hospitalarios

Prolonged seizures and status epilepticus are common neurological medical emergencies. Early and appropriate treatment is essential to reduce morbidity and mortality. Most seizures occur in the community, so parents and caregivers must be prepared for their management. Benzodiazepines (BZD) are the first-line drugs used, with rectal diazepam (DZPr) being the most commonly used in pre-hospital treatment in Spain. In September 2011, the European Medicines Agency (EMA) authorized the use of oromucosal midazolam (MDZb) for the treatment of prolonged acute convulsive seizures in patients aged 3 months to < 18 years. MDZb has a rapid onset, short duration of effect, and avoids first-pass hepatic metabolism. MDZb has shown to be at least as or more effective than DZPr to stop the seizures. Buccal administration is easier and more socially accepted, especially in adolescents and adults. It is a safe drug with similar effects to other BZD; MDZb improves the overall cost-effectiveness of seizures management

Programa de detección precoz de trastornos generalizados del desarrollo en las áreas de salud de Salamanca y Zamora / Pervasive developmental disorders screening program in the health areas of Salamanca and Zamora in Spain

OBJETIVO: Evaluar los resultados obtenidos por el programa de detección precoz de trastornos generalizados del desarrollo (TGD), actualmente en marcha en las áreas de salud de Salamanca y Zamora, en términos de participación y opinión de los profesionales hacia el mismo, así como la validez de la herramienta con la finalidad de extender su uso tanto a nivel autonómico como nacional. MÉTODO: Un total de 54 equipos de pediatría participaron en las sesiones de formación del programa de cribado de TGD en septiembre de 2005 y accedieron a administrar el cuestionario M-CHAT a todos los padres que acudían a sus consultas de pediatría en cualquiera de estas 2 visitas: 18 y/o los 24 meses dentro del Programa del Control del Niño Sano. Hasta diciembre de 2012 han participado un total de 9.524 niños. Adicionalmente, se ha evaluado la participación y opinión de los equipos de pediatría participantes mediante cuestionarios y entrevistas y se han estimado los costes por caso positivo detectado. RESULTADOS: Del total, 852 (8,9%) sujetos resultaron sospechosos de TGD en el cuestionario M-CHAT, de los cuales 61 (7,1%) se confirmaron mediante la entrevista de seguimiento. De estos, 22 obtuvieron un diagnóstico TGD y 31 de otros trastornos de inicio en la infancia según DSM-IV-TR. El 74% de los equipos encuestados consideran el programa totalmente viable y el 22% viable aunque con reservas (n = 54). CONCLUSIONES: Este estudio ha conseguido mostrar por primera vez en España, la viabilidad de realizar un programa de cribado de TGD de base poblacional dentro del sistema público de salud. La formación sobre los problemas del desarrollo comunicativo y social y la difusión de las señales de alerta entre los pediatras, además de la utilización del M-CHAT, es fundamental para el avance en la detección temprana de estos trastornos

OBJECTIVE: To evaluate the results of the Pervasive Developmental Disorders (PDD) screening program currently ongoing in the public health services in the health area of Salamanca and Zamora, Spain, in terms of feasibility, reliability and costs, with the purpose of extending the program at regional and national levels. METHOD: A total of 54 paediatric teams (nurses and paediatricians) from the provinces of Salamanca and Zamora participated in the training sessions for the PDD Screening Programme in September 2005, and agreed to administer the questionnaire M-CHAT1 to all parents attending their clinics in any of these two visits: 18 months and/or 24 months within the Well-baby Checkup Program. A total of 9,524 children have participated up to December 2012. Additionally, we evaluated the participation and opinions of the paediatric teams using questionnaires, and costs per positive case have estimated. RESULTS: Out of a total of 852 (8.9%) children determined as PDD high-risk with the M-CHAT questionnaire results, 61 (7.1%) were confirmed as positive with the M-CHAT follow-up interview. Of these, 22 were diagnosed with a PDD and 31 other disorders of childhood onset according toDSM-IV-TR2. Almost three-quarters (74%) of respondents felt the program was totally feasible, and 22% viable, but with reservations (n=54). CONCLUSIONS: This study has been able to show for the first time in Spain, the feasibility of a population-based PDD screening program within the public health system. Training in social and communicative development, and dissemination of the early signs of PDD among paediatricians, as well as the use of the M-CHAT, is essential for progress in the early detection of these disorders

[Pervasive developmental disorders screening program in the health areas of Salamanca and Zamora in Spain]. / Programa de detección precoz de trastornos generalizados del desarrollo en las áreas de salud de Salamanca y Zamora.

OBJECTIVE: To evaluate the results of the Pervasive Developmental Disorders (PDD) screening program currently ongoing in the public health services in the health area of Salamanca and Zamora, Spain, in terms of feasibility, reliability and costs, with the purpose of extending the program at regional and national levels. METHOD: A total of 54 paediatric teams (nurses and paediatricians) from the provinces of Salamanca and Zamora participated in the training sessions for the PDD Screening Programme in September 2005, and agreed to administer the questionnaire M-CHAT(1) to all parents attending their clinics in any of these two visits: 18 months and/or 24 months within the Well-baby Check-up Program. A total of 9,524 children have participated up to December 2012. Additionally, we evaluated the participation and opinions of the paediatric teams using questionnaires, and costs per positive case have estimated. RESULTS: Out of a total of 852 (8.9%) children determined as PDD high-risk with the M-CHAT questionnaire results, 61 (7.1%) were confirmed as positive with the M-CHAT follow-up interview. Of these, 22 were diagnosed with a PDD and 31 other disorders of childhood onset according to DSM-IV-TR(2). Almost three-quarters (74%) of respondents felt the program was totally feasible, and 22% viable, but with reservations (n=54). CONCLUSIONS: This study has been able to show for the first time in Spain, the feasibility of a population-based PDD screening program within the public health system. Training in social and communicative development, and dissemination of the early signs of PDD among paediatricians, as well as the use of the M-CHAT, is essential for progress in the early detection of these disorders.

Epilepsia como forma de presentación de un aneurisma intracraneal no roto / Epilepsy as the presenting symptom of an unruptured intracranial aneurysm

No disponible

No disponible

Desarrollo y resultados del programa de cribado de los T.E.A. en las provincias de Salamanca y Zamora / Development and results of the screening program of autistic spectrum disorders in the provinces of Salamanca and Zamora

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[Attention deficit and hyperactivity disorder, a current problem]. / Trastorno por déficit de atención e hiperactividad: un problema actual.

INTRODUCTION: Attention deficit disorder and hyperactivity (ADDH), is a condition that affects the normal development of children. The symptoms include difficulty of controlling physical activity, inattention and learning disorders. The ADDH must be diagnosed in accordance with the clinical findings defined in the DSM IV. OBJECTIVE: To describe the epidemiology and clinical characteristics of children diagnosed with ADDH in our hospital. MATERIAL AND METHODS: Biannual observational study. Variables evaluated were: age, sex, personal and family medical history, symptoms, therapy and treatment response. RESULTS: There 83 participants (87 % Male and 13 % Female), of which 32.5 % were diagnosed during the study. Ages ranged from 3-8 years (84 %). There was a family history related to ADDH in 38 % of patients, and personal history of prematurity, acute foetal distress, small for gestational age, convulsions were reported. Association of hyperactivity and attention deficit was found in 65 % of participants. Other related symptoms were cognitive disorder (62 %), language disabilities (41 %) and motor disorders (35 %). Treatment was on-going in 65 % of the patients, 27.7 % of them having adverse effect. Evolution with therapy was favourable in 61 %. CONCLUSIONS: These findings suggest that ADDH is one of the most common childhood psychiatric disorders, mainly affecting boys. There is usually a family history. Failure in school was one of the principal conditions. Association between attention deficit and hyperactivity, mainly hyperactivity, is the most common presentation. Other disorders such as motor and language disabilities are also common in these patients. Methylphenidate showed favourable outcomes in 61 % of the patients studied.

Trastorno por déficit de atención e hiperactividad: un problema actual / Attention deficit and hyperactivity disorder, a current problem

Introducción. El trastorno por déficit de atención e hiperactividad (TDAH) es un trastorno del desarrollo normal del niño, manifestado por una exagerada e inapropiada hiperactividad, impulsividad y dificultad para mantener la atención. Su etiología es desconocida. El diagnóstico se fundamenta en criterios clínicos recogidos en la 4.ª edición del Manual diagnóstico y estadístico de los trastornos mentales (DSM-IV). Una detección y tratamiento precoces ayudarán a controlar los síntomas, mejorando el aprendizaje escolar y las interacciones sociales. Objetivo. Describir las características epidemiológicas y clínicas de los pacientes con TDAH valorados en la consulta de Neurología Pediátrica del Hospital Universitario de Salamanca durante un período de 2 años. Material y métodos. Estudio observacional transversal, en niños diagnosticados de TDAH en la consulta de Neurología Pediátrica desde enero de 2004 hasta diciembre de 2005, ambos meses inclusive. Se analizan las variables de edad, sexo, antecedentes personales y familiares relacionados con el TDAH, síntomas al diagnóstico, tratamiento y respuesta terapéutica. Resultados. El número de niños estudiados fue de 83 (87 % de varones). El 32,5 % fueron diagnosticados durante el período de estudio. El 84 % de los pacientes tenían edades comprendidas entre los 3 y los 8 años en el momento del diagnóstico. La principal vía de derivación a la consulta fue a través de los equipos psicopedagógicos del Ministerio de Educación. Se hallaron antecedentes familiares relacionados con TDAH en un 38,5 % de los pacientes, entre los cuales destacaban: ansiedad, depresión, epilepsia, debilidad mental y TDAH. La mitad de los pacientes tenían antecedentes personales de interés: prematuridad (16,5 %), síndrome fetal alcohólico (SFA) (14,4 %), crecimiento intrauterino retardado (CIR) (4,8 %) y convulsiones (19,2 %). La asociación de déficit de atención e hiperactividad se observó en el 65 %. El déficit de atención como único síntoma se observó en un 7,2 % de los casos. Más de un tercio de los pacientes presentaron retraso motor. Se constató un retraso escolar en el 62 % de los niños y del lenguaje, en un 41 %. Ambos retrasos se asociaron en un 20,3 % de los niños. En 44 pacientes se realizó un estudio psicométrico; se halló un cociente intelectual inferior al normal en el 40 %. El tratamiento con metilfenidato se pautó en el 65 % de los niños, manifestando efectos secundarios el 27,7 % de los niños tratados (anorexia, cefalea y alteraciones del sueño). La evolución con tratamiento multidisciplinario fue favorable, con mejoría del comportamiento y del rendimiento escolar en un 61 % de los niños valorados. Conclusiones. El TDAH presenta una elevada incidencia, predominando en varones de 3-8 años. Los antecedentes familiares y personales están presentes en la mitad de los pacientes. El principal motivo de la derivación a la consulta médica fue el retraso escolar. El subtipo clínico predominante es la combinación de déficit de atención e hiperactividad con predominio de la hiperactividad. La asociación del retraso motor y del lenguaje es frecuente en estos niños. El tratamiento con metilfenidato fue efectivo en un porcentaje elevado de niños

Introduction. Attention deficit disorder and hyperactivity (ADDH), is a condition that affects the normal development of children. The symptoms include difficulty of controlling physical activity, inattention and learning disorders. The ADDH must be diagnosed in accordance with the clinical findings defined in the DSM IV. Objective. To describe the epidemiology and clinical characteristics of children diagnosed with ADDH in our hospital. Material and methods. Biannual observational study. Variables evaluated were: age, sex, personal and family medical history, symptoms, therapy and treatment response. Results. There 83 participants (87 % Male and 13 % Female), of which 32.5 % were diagnosed during the study. Ages ranged from 3-8 years (84 %). There was a family history related to ADDH in 38 % of patients, and personal history of prematurity, acute foetal distress, small for gestational age, convulsions were reported. Association of hyperactivity and attention deficit was found in 65 % of participants. Other related symptoms were cognitive disorder (62 %), language disabilities (41 %) and motor disorders (35 %). Treatment was on-going in 65 % of the patients, 27.7 % of them having adverse effect. Evolution with therapy was favourable in 61 %. Conclusions. These findings suggest that ADDH is one of the most common childhood psychiatric disorders, mainly affecting boys. There is usually a family history. Failure in school was one of the principal conditions. Association between attention deficit and hyperactivity, mainly hyperactivity, is the most common presentation. Other disorders such as motor and language disabilities are also common in these patients. Methylphenidate showed favourable outcomes in 61 % of the patients studied

Convulsiones febriles. Protocolo diagnóstico-terapéutico / Fever seizures. Diagnostic-therapeutic diagnosis

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Dosage programming of phenobarbital in neonatal seizures.

Owing to the changes occurring in the organism as a result of biological maturation, disposition kinetics of phenobarbital in newborns is significantly different to that observed in the paediatric and adult populations. Moreover, the disposition parameters change constantly during the first days of life. The data on the serum levels of phenobarbital in 17 newborns were analysed to quantify the changes in the elimination half-life of phenobarbital during the first weeks of life. The half-life of the drug was estimated to be (mean +/- SD) 114.2 +/- 43.0 h, 73.19 +/- 24.17 h and 41.23 +/- 13.95 h in patients 1-10, 11-30 and 31-70 days old, respectively. According to these values and assuming phenobarbital serum levels of 20 mg/l to be safe and effective in neonatal seizures, the initial dosing recommended is 2.9, 4.8 and 6.0 mg/kg/day in newborns 1-10, 11-30 and 31-70 days old, respectively.

[Clinical polymorphism of the XYY syndrome]. / Polimorfismo clínico del síndrome XYY.

The XYY syndrome presents with a wide variation in the clinical features, both of the physical and behavioral nature. We report two new cases which illustrate this statement. The first case presented with aggressive behaviour and cryptorchidism. The second case was associated with pathological short height, pubertal delay and cardiac features (extrasystoles and short PR interval). We revise some of the aspects of XYY syndrome.

[Facial paralysis in infancy. Clinical, diagnostic and therapeutic aspects]. / Parálisis facial en la infancia. Aspectos clínicos, diagnósticos y terapéuticos.

We present seven cases of facial paralysis in newborn and infants from a series of 88 patients (pediatrics and non pediatrics) seen in a fourteen months period (October 1987 to December 1988). Current approaches to diagnosis and treatment, including pharmacological treatment with steroids and surgical treatment are described. All patients we present in this paper recovered a good facial activity and no secondary effects were found during treatment.

Subcutaneous fat necrosis of the newborn and idiopathic hypercalcemia.

A newborn with important signs of fetal disturbance and respiratory distress and respiratory distress developed plaques on both buttocks 14 days after birth, with the clinical and histological characteristics of subcutaneous fat necrosis of the newborn. Fifteen days after the onset of the cutaneous signs, a discrete degree of hypercalcemia was detected. Its outcome was favorable due to the early establishment of a diet rich in medium-chain triglycerides and devoid of calcium and vitamin D. Sixteen cases of idiopathic hypercalcemia in newborn with subcutaneous fat necrosis have been reported of which 3 died. Serial serum calcium determinations should be made in such infants and they should be observed closely for signs and symptoms of hypercalcemia.

Effect of sodium valproate on phenobarbital serum levels in children and adults.

The influence of sodium valproate on serum levels of phenobarbital during combination treatment was studied in 29 children and 50 adults with epilepsy. Steady-state drug levels in serum were determined immediately prior to drug administration using immunoenzymatic analysis. The serum level/dose ratio of phenobarbital increased significantly (p less than 0.001) when sodium valproate was added to the treatment. The increase had a mean value of 50.9% in adults and 112.5% in children, suggesting marked interindividual variability in the intensity of the interaction. Almost half of the patients required a decrease in the dose of phenobarbital prescribed. The interaction was more pronounced in patients with high serum levels of phenobarbital, while the dose of phenobarbital and the serum levels and dose of sodium valproate did not seem to affect the extent of the interaction. Close monitoring of the serum levels of phenobarbital is recommended during simultaneous treatment with sodium valproate.

[Goldenhar syndrome. Report of a new case]. / Síndrome de goldenhar. Aportación de un nuevo caso.

Authors present a patient with Goldenhar's syndrome, with severe cardiac, vascular and brain malformations. Goldenhar's syndrome is a condition characterized by epibulbar dermoids or lipodermoid , malformation auricular, and vertebral anomalies. Many other abnormalities have been described, but these signs are the most constant and give the syndrome its' identity.

[Threshold for bicarbonate in epileptic children in treatment with anticonvulsant drugs (author's transl)]. / Descenso del umbral renal para el bicarbonato en niños tratados con anticonvulsivantes.

The study comprised 31 ambulatory epileptic children aged 1,5-14 years (mean: 7.37), receiving treatment with anticonvulsant drugs. Authors found that renal threshold for bicarbonate was inferior when compared to control group (mean = 21.3 mEq/l., SD = 1.75; mean = 25.34 mEq/l., SD = 1.29; p less than 0.0001), low calcemia (mean = 9.12 mg./dl., SD = 0.51; mean = 9.43, SD = 0.45; p less than 0.01) and elevated alkaline phosphatase (mean = 212.6 mU/ml., SD = 75.9; mean = 127.4, SD = 50.2; p less than 0.01). No significant difference in urinary excretion of cyclic AMP, phosphate or calcium was observed. Nineteen patients who had subnormal threshold (inferior to -2 SD in the control group) when compared with control group, had: low calcemia (p less than 0.01), high alkaline phosphatase (p less than 0.0001) and a similar urinary cyclic AMP, calciuria and phosphaturia. A negative correlation between renal threshold to bicarbonate and serum phosphate (r = -0.49, p less than 0.01) and a negative correlation between the urinary cyclic AMP and the duration of treatment (r = -0.42, p less than 0.05) was found. It is commented that although in deficiency rickets, proximal tubular acidosis is due to secondary hyperparathyroidism, in their patients despite they had biochemical characteristics of rickets, low calcemia and elevated alkaline phosphatase, the descent of renal threshold to bicarbonate is a nonparathoromone mediated phenomenon.