[Current state of the attention deficit hyperactivity disorder approach in neuropediatrics]. / Estado actual del enfoque del trastorno por deficit de atencion/hiperactividad en neuropediatria.

AIM: To know the current state of the approach of attention deficit hyperactivity disorder (ADHD) in neuropediatricians. SUBJECTS AND METHODS: A telematic survey was carried out to collect preliminary information on the interest, difficulties in the management and treatment of ADHD to the 437 fellowship of the Neuropediactric Spanish Society (SENEP). RESULTS: Only 32.49% of the sent questionnaires were answered, with important geographic variability. 97.89% stated that 50% of their consultations were children with learning disabilities and ADHD. Regarding who started treatment for ADHD in their area, the majority answered that the neuropediatrician (57.97%), followed by the child psychiatrist (34.78%) and the primary care pediatrician (5.31%). The lack of a psycho-pedagogical study by the school (49.79%), followed by the lack of time in the consultation (29.11%), was cited as the greatest difficulty in the initial assessment of children with suspected ADHD. Concerning the difficulties in the follow-up, the biggest complaint was the lack of coordination between professionals, the school and parents. And, lastly, regarding the type of treatment use, most patients were on prolonged-release methylphenidate, a stable percentage using immediate release methylphenidate as a single or combined treatment, and in a lower range was the use of clonidine and atomoxetine, and an incipient use of lisdexamphetamine were observed. 80% of the patient showed adherence to pharmacological treatment after one year. CONCLUSIONS: It is necessary to advance in the training and continuous education of our neuropediatric specialists in ADHD and to homogenize the clinical practice and coordination with education system in the Spanish territory.

TITLE: Estado actual del enfoque del trastorno por deficit de atencion/hiperactividad en neuropediatria.Objetivo. Conocer el estado actual del enfoque del trastorno por deficit de atencion/hiperactividad (TDAH) entre los neuropediatras. Sujetos y metodos. Se realizo una encuesta telematica que recogia informacion preliminar sobre el interes, las dificultades en el manejo y el tratamiento del TDAH a los 437 socios de la Sociedad Española de Neurologia Pediatrica. Resultados. Respondio un 32,49% de los cuestionarios enviados, con una importante variabilidad geografica. El 97,89% afirmo que el 50% de sus consultas eran niños con trastornos de aprendizaje y TDAH. Respecto a quien iniciaba el tratamiento para el TDAH en su area, la mayoria contesto que el neuropediatra (57,97%), seguido del psiquiatra infantil (34,78%) y del pediatra de atencion primaria (5,31%). Respecto a las mayores dificultades para la valoracion inicial de los niños con sospecha de TDAH, se citaron la falta de un estudio psicopedagogico por parte de la escuela (49,79%), seguido de la falta de tiempo en la consulta (29,11%). Sobre las dificultades en el seguimiento, la mayor queja se produjo por la falta de coordinacion entre los profesionales, la escuela y los padres. Respecto a la medicacion, la mayoria de los pacientes se encontraba en tratamiento con algun tipo de metilfenidato de liberacion prolongada, un porcentaje estable utilizaba metilfenidato de liberacion inmediata como tratamiento unico o combinado, y se observo en un rango inferior el uso de clonidina y atomoxetina, y un incipiente uso de lisdexanfetamina. La adhesion al tratamiento farmacologico al año fue alrededor del 80%. Conclusiones. Es necesario avanzar en la capacitacion y educacion continua de nuestros especialistas neuropediatricos en el manejo del TDAH, y en homogeneizar la practica clinica y la coordinacion con educacion en el territorio español.

Asfixia grave en una lactante por uso incorrecto del «pañuelo portabebés» / Infant suffocation associated with the incorrect use of a baby sling

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Enfermedad de Menkes: diagnóstico clínico, bioquímico y molecular / Menkes disease: its clinical, biochemical and molecular diagnosis

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[Diagnosis of malformations of the central nervous system using foetal magnetic resonance imaging]. / Diagnóstico mediante resonancia magnética fetal de las malformaciones del sistema nervioso central.

INTRODUCTION AND DEVELOPMENT: Ultrasonography is the preferred diagnostic imaging technique for studying the intrauterine foetus. Yet, occasionally certain circumstances arise where it is necessary to use other techniques in order to achieve a better analysis of the foetal structures. The development of ultra-high speed magnetic resonance imaging (MRI) has now been applied to the study of the foetus, since it allows images to be obtained in extremely short times and the effects of movements by the mother or the foetus have little influence on the results. It is a type of imaging that does not use ionising radiations and offers high contrast images in any plane without being influenced by the physical characteristics of the expectant mother or the position of the foetus. AIMS: To make the technique and the use of MRI in foetal studies more widely known, as well as to review the role played by MRI in the study and treatment of foetal deformations of the central nervous system detected within the uterus. CONCLUSIONS: Anomalies of the central nervous system constitute the most frequent cause of visits for prenatal diagnosis. They usually have a poor prognosis due to the likelihood of important sequelae and are a cause of great concern to the parents. They are often associated with genetic and chromosomal disorders.

Diagnóstico mediante resonancia magnética fetal de las malformaciones del sistema nervioso central / Diagnosis of malformations of the central nervous system using foetal magnetic resonance imaging

Introducción y desarrollo. La ecografía es la técnica dediagnóstico por imagen de elección para el estudio fetal intraútero;no obstante, existen circunstancias donde se hacen necesarias otrastécnicas de estudio para un mejor análisis de las estructuras fetales.La aparición de las secuencias ultrarrápidas en resonanciamagnética (RM) ha permitido que esta modalidad de imagen puedaaplicarse al estudio del feto, ya que posibilita la obtención de imágenesen tiempos extraordinariamente cortos, poco influidos porlos movimientos maternos o fetales. Es una modalidad que no utilizaradiaciones ionizantes y proporciona imágenes de alto contrastey en cualquier plano del espacio, sin verse influida por las característicasfísicas de la gestante ni la posición fetal. Objetivos.Dar a conocer la técnica y el uso de la RM en el estudio fetal y revisarel papel que desempeña la RM en el estudio y el tratamiento delas malformaciones fetales del sistema nervioso central detectadasintraútero. Conclusión. Las anomalías del sistema nervioso centralconstituyen la causa más frecuente de consulta de diagnóstico prenatal.Habitualmente tienen mal pronóstico por la probabilidad desecuelas importantes y causan gran preocupación a los padres.Muchas veces se asocian a trastornos genéticos y cromosomopatías

Introduction and development. Ultrasonography is the preferred diagnostic imaging technique for studying theintrauterine foetus. Yet, occasionally certain circumstances arise where it is necessary to use other techniques in order toachieve a better analysis of the foetal structures. The development of ultra-high speed magnetic resonance imaging (MRI) hasnow been applied to the study of the foetus, since it allows images to be obtained in extremely short times and the effects ofmovements by the mother or the foetus have little influence on the results. It is a type of imaging that does not use ionisingradiations and offers high contrast images in any plane without being influenced by the physical characteristics of the expectantmother or the position of the foetus. Aims. To make the technique and the use of MRI in foetal studies more widely known, aswell as to review the role played by MRI in the study and treatment of foetal deformations of the central nervous system detectedwithin the uterus. Conclusions. Anomalies of the central nervous system constitute the most frequent cause of visits for prenataldiagnosis. They usually have a poor prognosis due to the likelihood of important sequelae and are a cause of great concern tothe parents. They are often associated with genetic and chromosomal disorders

[Episodic benign unilateral mydriasis. Clinical case in a girl]. / Midriasis unilateral benigna episódica. Caso clínico en una niña.

INTRODUCTION: Pupil asymmetry is an alarm signal which should lead to investigation to rule out severe underlying neurological disorders. Among its causes are tumors, aneurysms and hernia of the uncus. The differential diagnosis should also include other conditions such as Adie's tonic pupil, the Pourfour de Petit syndrome and local disorders such as closed angle glaucoma or segmental spasm of the iris dilator muscle. In practice however, exposure to mydriatic substance is one of the commonest causes. Another cause of this sign is benign episodic unilateral mydriasis. This uncommon condition has been defined as an isolated benign cause of pupil asymmetry. The underlying physiopathology is not always clear and may involve either parasympathetic deficiency or sympathetic hyperactivity affecting the iris. Usually related to migraine, some authors classify it as a limited form of ophthalmoplegic migraine, although some cases have been described with no accompanying headache. CLINICAL CASE: We describe a case of benign episodic unilateral mydriasis in a six-year-old girl who presented with intermittent episodes of pupil asymmetry with no other neurological symptoms. CONCLUSION: We underline the rarity of this condition in children with no simultaneous headache.

Midriasis unilateral benigna episódica.Caso clínico en un niño / Benign episodic unilateral mydriasis. A report of a case in a child

Introducción. La asimetría pupilar es un signo de alarma que debe llevar a descartar patología neurológica grave subyacente. Entre las posibles causas de este hallazgo se encuentran tumoraciones, aneurismas y herniación uncal. En el diagnóstico diferencial deben incluirse también otros cuadros como la pupila tónica de Adie, el síndrome de Pourfour du Petit y patologías locales como el glaucoma de ángulo cerrado o el espasmo segmentario del dilatador del iris. En la práctica, sin embargo, la exposición a sustancias midriáticas es una de sus causas más frecuentes. Otra causa de este hallazgo es la midriasis unilateral benigna episódica. Esta entidad poco frecuente, se ha definido como causa aislada y benigna de asimetría pupilar. La fisiopatología del cuadro no es siempre clara, en ella podría intervenir tanto un déficit parasimpático como una hiperactividad simpática sobre el iris. Generalmente relacionada con la migraña, ha sido catalogada por algunos autores como una forma limitada de migraña oftalmopléjica, a pesar de haberse descrito algunos casos no acompañados de cefalea. Caso clínico. Se expone un caso de midriasis unilateral benigna episódica en una niña de 6 años que ha presentado episodios intermitentes de asimetría pupilar sin otra sintomatología neurológica. Conclusión. Destacamos la rareza del cuadro por aparecer en edad pediátrica y sin cefalea acompañante (AU)

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[Periventricular leukomalacia: its retrospective diagnosis in children with spastic diplegia]. / Leucomalacia periventricular: diagnóstico retrospectivo en niños con diplejía espástica.

Typical images of residual periventricular leukomalacia (PVL) have been searched for in a review of the CT scans of 135 children with spastic diplegia. Among the 135 children studied, 90 (66.6%) of them had had a CT scan with 51 (56.6%) showing typical images of residual PVL. Only 6 (11.7%) of these 51 children were identified in the early stages by cranial ultrasound and 11 (21.5%) of the 51 were diagnosed by CT scan. The remaining 34 (66.6%) were diagnosed retrospectively during our study and none of them were identified as PVL in the first examination by CT scan. In addition to CT scan examination, 3 children were explored by MRI that confirmed the diagnosis of PVL. The results of our study indicate that images in CT scans reported as slight ventricular dilation, normotensive hydrocephalus, hydrocephalus with irregular limits, subcortical atrophy or periventricular heterotopia could corresponds to images of residual PVL in the appropriate context. We emphasize the importance of CT scan examination for the retrospective diagnosis of PVL in elder children in which cranial ultrasound examination had not been performed during the neonatal period.

[Slipping rib syndrome as a cause of recurrent abdominal pain in children]. / Síndrome de la costilla deslizante como causa de dolor abdominal recidivante en la edad pediátrica.

The slipping rib syndrome can be produced by a direct or indirect traumatism over the sternal tip of the costal cartilages 8, 9 and 10. The main complains are abdominal pain in the right or left upper abdominal, quadrants, and sometimes a sensation of rubbing or slipping of the ribs. The hoocking maneuver is useful in the diagnosis of this syndrome. Three clinical cases of the slipping rib syndrome are reported. This syndrome must be taken into account is the differential diagnosis of the abdominal pain in pediatric patients.