[Breath-holding spell or epileptic seizure? A description of three girls with epileptic seizures triggered by anoxia]. / ¿Espasmo del sollozo prolongado o crisis epiléptica?: descripción de tres niñas con crisis epilépticas desencadenadas por anoxia.

TITLE: ¿Espasmo del sollozo prolongado o crisis epiléptica?: descripción de tres niñas con crisis epilépticas desencadenadas por anoxia.

Displasia cortical focal en paciente de 3 años con epilepsia parcial continua / Focal cortical dysplasia in a three years old patient with epilepsia partialis continua

No disponible

[Restless legs syndrome in patients diagnosed with attention deficit hyperactivity disorder]. / Sindrome de piernas inquietas en pacientes diagnosticados de trastorno por deficit de atencion/hiperactividad.

INTRODUCTION: Restless legs syndrome (RLS) is a common neurological disease. RLS has been linked to various psychiatric disorders, especially with attention deficit hyperactivity disorder (ADHD). AIMS: The main objective was to describe the frequency of RLS in pediatric patients diagnosed with ADHD. Secondary objectives of the study were describe other sleep disorders in ADHD patients. PATIENTS AND METHODS: A multicentre prospective study was conducted in nine Spanish centers. We included children aged 6-18 years diagnosed with ADHD between January and June 2015. Data were collected by 13 researchers doctors through an interview with the parent/caregiver and with the child. To assess the degree of functioning of patients with ADHD we used the Children's Global Assessment Scale. The Sleep Disturbance Scale for Children was applied to screening sleep disorders in childhood. RESULTS: A sample of 73 patients was collected. Five patients (6.8%) met diagnostic criteria for RLS: four of them definitive and one probable. CONCLUSIONS: RLS is a frequent condition in adulthood but also in adolescence and childhood. ADHD patients have an increased risk of an RLS.

TITLE: Sindrome de piernas inquietas en pacientes diagnosticados de trastorno por deficit de atencion/hiperactividad.Introduccion. El sindrome de piernas inquietas (SPI) es una patologia neurologica comun. Se ha relacionado con diferentes trastornos psiquiatricos, especialmente con el trastorno por deficit de atencion/hiperactividad (TDAH). Objetivos. El objetivo principal fue describir la frecuencia del SPI en pacientes pediatricos diagnosticados de TDAH. Los objetivos secundarios del estudio fueron describir otros trastornos del sueño en pacientes con TDAH. Pacientes y metodos. Se realizo un estudio descriptivo prospectivo multicentrico en nueve centros españoles de niños de 6-18 años con diagnostico de TDAH entre enero y junio de 2015. Los datos fueron recogidos por 13 medicos investigadores mediante entrevista con el padre/cuidador y con el menor. Para valorar el grado de funcionamiento de los pacientes con TDAH se utilizo la Children's Global Assessment Scale. Se aplico la Sleep Disturbance Scale for Children de Bruni para el cribado del trastorno de sueño de la infancia. Resultados. Se recogio una muestra de 73 pacientes. Cinco pacientes (6,8%) cumplen criterios diagnosticos de SPI: cuatro de ellos definitivos y uno probable. Conclusiones. El SPI es una entidad frecuente en la edad adulta, pero tambien en la adolescencia y en la infancia. Los pacientes con TDAH tienen un riesgo aumentado de presentar de manera concomitante un SPI.

Sueño y obesidad en la infancia / Sleep and obesity in childhood

La obesidad y el síndrome metabólico son dos de los grandes problemas de salud infantil de la sociedad occidental. De forma paralela, en los últimos años también estamos asistiendo a una disminución de las horas de sueño entre la población infantil. Desde 1992, numerosos estudios epidemiológicos, tanto transversales como longitudinales, demuestran que la privación crónica de sueño en niños se asocia a un mayor riesgo de desarrollo de sobrepeso-obesidad y/o síndrome metabólico (hipertensión arterial, aumento de resistencia a la insulina y dislipemia). Los mecanismos precisos por los que dormir menos horas provoca obesidad son desconocidos, aunque parecen estar implicados múltiples factores, como las alteraciones en el eje hipotálamo-hipofisario (varios neuropéptidos como la leptina y la grelina), un mayor apetito con especial avidez por comidas de alto valor energético, o una mayor sensación de fatiga, con la consiguiente reducción de la actividad física y aparición de alteraciones en el ciclo sueño-vigilia (alteraciones del ritmo de la melatonina). En este trabajo se han revisado las evidencias actuales sobre la relación entre el sueño y la obesidad en la población pediátrica. Actualmente se acepta que los niños que duermen menos horas tienen mayor probabilidad de desarrollar sobrepeso y síndrome metabólico, aunque no se ha podido aún demostrar una clara relación de causalidad. Es necesario promover un sueño adecuado entre la población infantil, ya no sólo por los numerosos efectos beneficiosos conocidos (cognitivos, inmunológicos, conductuales, metabólicos e incluso de disminución de la mortalidad), sino también por un posible efecto preventivo de la obesidad y el síndrome metabólico (AU)

Obesity and metabolic syndrome are two of the major child health problems in Western society. During the last years, it has been also noticed a decline in sleeping hours among children. Since 1992, many epidemiological studies, both cross-sectional and longitudinal, have shown that chronic sleep deprivation in children relates to an increasing risk of developing overweight-obesity and/or metabolic syndrome (hypertension, increasing resistance to insulin and dyslipidemia). Although precise proccess that make obesity leads to a reduction of sleeping hours are currently unknown, a number of proccess that seem to be somehow involved have been identified. Among others, we may mention the alterations in the hypothalamic-pituitary (several neuropeptides such as leptin and ghrelin), an increasing appetite with special foods high avidity energy, a greater sense of fatigue with consequent reduction in physical activity, and changes in sleep-wake cycle (melatonin rhythm disturbances). This work contains a review of current evidence on the relationship between sleep and obesity within the pediatric population. It is now accepted that children who sleep less hours are more likely to develop overweight and metabolic syndrome, but it has not been possible to demonstrate a clear causality yet. It is necessary to promote adequate sleep among children, not only due to the many beneficial effects already known (cognitive, immunological, behavioral, metabolic and even decrease mortality), but also given its potential ability to prevent obesity and metabolic syndrome (AU)

Hamartoma hipotalámico en la edad pediátrica: características clínicas, evolución y revisión de la literatura / Hypothalamic hamartoma in paediatric patients: clinical characteristics, outcomes and review of the literature

Objetivos: Describir las características epidemiológicas, clínico-electroencefalográficas y la morbilidad asociada de los pacientes con hamartoma hipotalámico, así como la evolución y el tratamiento seguido. Pacientes y métodos: Se han revisado retrospectivamente las historias clínicas de 10 pacientes diagnosticados de hamartoma hipotalámico por resonancia magnética en los últimos 20 años.Resultados: La edad de debut de la epilepsia en los pacientes con hamartoma hipotalámico en nuestra serie está comprendida entre los primeros días de vida y los 2 años. De los 10 pacientes totales, 8 tuvieron crisis epilépticas en su evolución. Todos ellos presentaron crisis gelásticas, además de otros tipos de crisis, siendo las más frecuentes las parciales simples. Los hallazgos electroencefalográficos registrados fueron muy variables. Uno de los pacientes desarrolló encefalopatía epiléptica. Cinco pacientes presentaron algún tipo de trastorno de conducta. Cinco pacientes presentaron problemas cognitivos. En los 8 pacientes que presentaron crisis se ensayaron al menos 2 fármacos antiepilépticos diferentes y en 6 pacientes de estos se recurrió a alguna modalidad de tratamiento no farmacológica con el objetivo del control de las crisis. Solo en 3 de los 8 pacientes se ha conseguido aceptable control de su epilepsia. Cinco pacientes de la serie desarrollaron pubertad precoz. El tiempo medio de seguimiento de la serie es de 6 años. Conclusiones: La epilepsia es la manifestación más frecuente de los hamartomas hipotalámicos, siendo en la mayoría de los casos farmacorresistente, lo que conlleva dificultades en el manejo de estos pacientes, precisando en muchas ocasiones cirugía para su control. Es frecuente la aparición de comorbilidad psiquiátrica y afectación cognitiva (AU)

Objective: To describe the epidemiological and clinical-electroencephalographic characteristics, and associated morbidity of patients with hypothalamic hamartoma, as well as the treatment followed and outcomesPatients and methods: We have retrospectively reviewed the medical histories of 10 patients diagnosed with hypothalamic hamartoma by magnetic resonance imaging over the last 20 years. Results: The age of onset of epilepsy in patients with hypothalamic hamartoma in our series was between the first days of life and 2 years. Of the 10 total patients, 8 had epileptic seizures during its progress. All of them had gelastic seizures, in addition to other types of seizures, with the most common being partial simple seizures. The electroencephalographic findings recorded were highly variable. One of the patients developed epileptic encephalopathy. Five patients had some kind of conduct disorder. Five patients had cognitive problems. At least 2 different antiepileptic drugs were measured in 8 of the patients who had seizures, and in 6 of these some type of non-pharmacological treatment had been used with the objective of seizure control. Only in 3 of 8 patients has been achieved Acceptable control of epilepsy had only been achieved in 3 out the 8 patients. Five patients of the series developed precocious puberty. The average time of follow-up of the series was approximately 6 years.Conclusions: Epilepsy is the most frequent manifestation of hypothalamic hamartomas. Most cases were drug-resistant, which led to difficulties in the management of these patients, requiring surgery for their control on many occasions. Psychiatric comorbidity and cognitive impairment is common (AU)

[Hypothalamic hamartoma in paediatric patients: clinical characteristics, outcomes and review of the literature]. / Hamartoma hipotalámico en la edad pediátrica: características clínicas, evolución y revisión de la literatura.

OBJECTIVE: To describe the epidemiological and clinical-electroencephalographic characteristics, and associated morbidity of patients with hypothalamic hamartoma, as well as the treatment followed and outcomes PATIENTS AND METHODS: We have retrospectively reviewed the medical histories of 10 patients diagnosed with hypothalamic hamartoma by magnetic resonance imaging over the last 20 years. RESULTS: The age of onset of epilepsy in patients with hypothalamic hamartoma in our series was between the first days of life and 2 years. Of the 10 total patients, 8 had epileptic seizures during its progress. All of them had gelastic seizures, in addition to other types of seizures, with the most common being partial simple seizures. The electroencephalographic findings recorded were highly variable. One of the patients developed epileptic encephalopathy. Five patients had some kind of conduct disorder. Five patients had cognitive problems. At least 2 different antiepileptic drugs were measured in 8 of the patients who had seizures, and in 6 of these some type of non-pharmacological treatment had been used with the objective of seizure control. Only in 3 of 8 patients has been achieved Acceptable control of epilepsy had only been achieved in 3 out the 8 patients. Five patients of the series developed precocious puberty. The average time of follow-up of the series was approximately 6 years. CONCLUSIONS: Epilepsy is the most frequent manifestation of hypothalamic hamartomas. Most cases were drug-resistant, which led to difficulties in the management of these patients, requiring surgery for their control on many occasions. Psychiatric comorbidity and cognitive impairment is common.

Epilepsias benignas de la infancia: dificultades académicas y alteraciones comportamentales / Benign childhood epilepsies: academic difficulties and behavioural disorder

Introducción. Se han publicado artículos en los que se muestra que los pacientes pueden presentar trastornos del comportamiento y dificultad para el aprendizaje en las epilepsias benignas de la infancia (EBI). Objetivos. Hacer una revisión de los pacientes con diagnóstico de EBI en nuestro hospital e identificar si presentan dichas alteraciones. Pacientes y métodos. Revisión retrospectiva de las historias clínicas de los pacientes con diagnóstico de EBI. Se realizó electroencefalograma (EEG) o video-EEG-poligrafía de sueño a todos los pacientes. Para la valoración intelectual se utilizaron los tests de inteligencia para niños de Wechsler. Resultados. Se recogieron 102 pacientes con diagnóstico de EBI. El 51,6% de los pacientes con epilepsia rolándica mostraba una atención dispersa y el 16,2% evidenciaba un temperamento impulsivo. En el grupo con síndrome de Panayiotopoulos, un 30,3% mostraba una atención dispersa y un 27,3% presentaba un temperamento impulsivo. Se llevó a cabo una valoración psicométrica en 43 pacientes. El valor del cociente intelectual total medio fue de 95 (rango: 55-126). En los tres grupos el rendimiento escolar fue bueno en aproximadamente la mitad, regular en cerca del 30% y malo en alrededor del 15%. En el grupo con epilepsia rolándica se encontró relación entre paroxismos frontales (p = 0,039) y occipitales (p = 0,004) en el EEG y un peor rendimiento escolar. En este grupo, los niños con conductas calificadas como dispersa, impulsiva o hiperactiva mostraban con más frecuencia paroxismos izquierdos (p = 0,030). Conclusiones. Las EBI son entidades con buen pronóstico, pero parecen asociar trastornos del aprendizaje y conductuales. Sería conveniente realizar estudios neuropsicológicos a estos pacientes para detectar tales alteracione (AU)

Introduction. Some papers published in the literature have shown that patients can present behavioural disorders and learning difficulties in benign childhood epilepsies (BCE). Aims. To review the patients diagnosed with BCE in our hospital and to determine whether they present such disorders. Patients and methods. The study consisted in a retrospective review of the medical records of patients diagnosed with BCE. An electroencephalogram (EEG) or video-EEG-polygraph recordings were performed on all patients during sleep. The Wechsler Intelligence Scale for Children was used to evaluate intelligence. Results. Data were collected for 102 patients diagnosed with BCE. Dispersed attention was observed in 51.6% of the patients with rolandic epilepsy and 16.2% displayed an impulsive temperament. In the group of patients with Panayiotopoulos syndrome, 30.3% displayed dispersed attention and 27.3% presented an impulsive temperament. A psychometric evaluation was carried out in 43 patients. The overall mean intelligence quotient was 95 (range: 55-126). In the three groups, academic achievement was good in approximately half the sample, regular in about 30% and poor in around 15%. In the group with rolandic epilepsy, the EEG showed a relation between frontal (p = 0.039) and occipital paroxysms (p = 0.004) and poorer academic achievement. In this group, the children with behaviours classed as dispersed, impulsive or hyperactive showed left-side paroxysms more frequently (p = 0.030). Conclusions. BCE are conditions with a good prognosis, but seem to be associated to learning and behavioural disorders. Neuropsychological studies should be conducted on these patients to detect these disorders (AU)

Polimicrogiria: epidemiología, factores neurológicos y anatómicos y evolución clínica de una serie de 34 casos / Polymicrogyria: epidemiology, neurological and anatomical factors and clinical outcome in a series of 34 cases

Objetivo: Describir la epidemiología y evolución clínica, así como los factores anatómicos y neurológicos implicados, en una serie de casos de 34 pacientes con esta afectación. Pacientes y métodos: Se han recopilado 34 pacientes diagnosticados y/o en seguimiento en la sección de neuropediatría del Hospital Infantil Universitario Niño Jesús entre 1995 y 2010. Todos los pacientes tienen una resonancia magnética indicativa de polimicrogiria y la mayoría sigue controles periódicos, por lo que conocemos su evolución. Resultados: El 76,5% de los pacientes son varones. La media de edad de inicio de la clínica neurológica es de 10 meses; el motivo del estudio fue por retraso psicomotor (44%) seguido por crisis (38,2%). En su evolución los pacientes presentaron epilepsia (61,7%), parálisis cerebral infantil (47%), retraso psicomotor/mental (94,1%), trastorno generalizado del desarrollo (26,4%), alteraciones conductuales (38,2%), déficits neurosensoriales (35,2%) y microcefalia el 67,6%. En el 82,3% la afectación es bilateral (42,8% perisilviana). El 58,8% asoció otras alteraciones en la resonancia magnética. El electroencefalograma al diagnóstico estaba alterado en el 41,1% y a lo largo de la evolución aumentó hasta el 67,6%. Reciben tratamiento antiepiléptico el 61,7%, precisando ≥ 2 fármacos el 52,3%. Dos pacientes se sometieron a cirugía de la epilepsia. Presentó algún tipo de secuela el 91,1%. La etiología es desconocida en el 61,7%; se sospecha infección congénita en 10 y cuadro sindrómico o polimalformativo en 3. Conclusiones: Con este estudio se pone en evidencia la amplitud de expresión clínica y radiológica posible en la polimicrogiria, además de las posibilidades futuras en cuanto a una aproximación etiológica en esta patología (AU)

Introduction: The aim of our study is to describe the epidemiology, clinical evolution, and the anatomical and neurological factors involved in polymicrogyria in 34 patients with this disorder. Subjects and methods: We have compiled 34 patients diagnosed and/or in follow-up at the Department of Paediatric Neurology of the Hospital Infantil Niño Jesús between 1995 and 2010. All the patients had a magnetic resonance imaging suggestive of polymicrogyria, and most of the patients still have periodic checks, thus their outcome is known. Results: The large majority were male (76.5%). The median age at presentation was 10 months; the reason for the study was psychomotor or mental delay (44%) followed by seizures (38.2%). During the condition patients presented with epilepsy (61.7%), infantile cerebral palsy (47%), psychomotor/mental retardation (94.1%), pervasive developmental disorder (26.4%), behavioural disturbances (38.2%), neurosensory deficit (35.2%) and microcephaly 67.6%. In 82.3% of patients there was bilateral involvement (42.8% perisylvian). Other abnormalities were observed in the MRI of 58.8% of patients. The electroencephalogram at diagnosis showed changes in 41.1%, and this rose to 67.6% during follow-up. 61.7% received antiepileptic treatment was received by 61.7% of patients, with 52.3% requiring ≥2 drugs. Epilepsy surgery was performed on two patients. Some type of sequelae was observed in 91.1% of patients. The aetiology was unknown in 61.7%; a congenital infection was suspected in 10 patients and syndromic or polymalformative disorder in three patients. Conclusions: This study shows the range of clinical and radiological expression in polymicrogyria, in addition to the possibilities for the future in terms of determining the aetiology of this pathology (AU)

Behçet infantil: a propósito de un caso / Infantile Behçet’s disease: an actual case

La enfermedad de Behçet es una vasculitis de pequeño vaso que cursa en forma de brotes recurrentes de fiebre y aumento de reactantes de fase aguda. Su diagnóstico es clínico y su importancia está en la posible aparición de complicaciones graves, como la afectación neurológica u ocular. Se presenta el caso de un niño marroquí de 3 años de edad, cuyo diagnóstico final fue el de enfermedad de Behçet infantil tras presentar episodios repetidos de fiebre, aftas orales, exantema en el tronco y uveítis. Su evolución clínica, en general, fue buena después de la administración de varios fármacos inmunomoduladores, pero presentó un daño ocular severo, pendiente de aprobación de fármacos anti-TNF para su tratamiento (AU)

Behçet’s disease is a small vessel vasculitis that attends in the form of recurrent outbreaks of fever and increase of acute phase reactants. The diagnosis is clinical and their significance is in the possible development of neurological or ocular involvement. We present the case of a Moroccan child of 3 years of age whose final diagnosis was the infantile Behçet's disease because the child presented repeated episodes of fever, recurrent oral ulcers, rash on the trunk and uveitis. The clinical evolution was on the whole good after several immune modulating drugs, but with severe ocular damage, pending approval of treatment with anti-TNF drugs (AU)

[Polymicrogyria: epidemiology, neurological and anatomical factors and clinical outcome in a series of 34 cases]. / Polimicrogiria: epidemiología, factores neurológicos y anatómicos y evolución clínica de una serie de 34 casos.

INTRODUCTION: The aim of our study is to describe the epidemiology, clinical evolution, and the anatomical and neurological factors involved in polymicrogyria in 34 patients with this disorder. SUBJECTS AND METHODS: We have compiled 34 patients diagnosed and/or in follow-up at the Department of Paediatric Neurology of the Hospital Infantil Niño Jesús between 1995 and 2010. All the patients had a magnetic resonance imaging suggestive of polymicrogyria, and most of the patients still have periodic checks, thus their outcome is known. RESULTS: The large majority were male (76.5%). The median age at presentation was 10 months; the reason for the study was psychomotor or mental delay (44%) followed by seizures (38.2%). During the condition patients presented with epilepsy (61.7%), infantile cerebral palsy (47%), psychomotor/mental retardation (94.1%), pervasive developmental disorder (26.4%), behavioural disturbances (38.2%), neurosensory deficit (35.2%) and microcephaly 67.6%. In 82.3% of patients there was bilateral involvement (42.8% perisylvian). Other abnormalities were observed in the MRI of 58.8% of patients. The electroencephalogram at diagnosis showed changes in 41.1%, and this rose to 67.6% during follow-up. 61.7% received antiepileptic treatment was received by 61.7% of patients, with 52.3% requiring ≥2 drugs. Epilepsy surgery was performed on two patients. Some type of sequelae was observed in 91.1% of patients. The aetiology was unknown in 61.7%; a congenital infection was suspected in 10 patients and syndromic or polymalformative disorder in three patients. CONCLUSIONS: This study shows the range of clinical and radiological expression in polymicrogyria, in addition to the possibilities for the future in terms of determining the aetiology of this pathology.