[Developmental amnesia and early brain damage: neuropsychology and neuroimaging]. / Amnesia del desarrollo y daño cerebral temprano: neuropsicologia y neuroimagen.

AIM: To contribute to neuropsychological profiling of developmental amnesia subsequent to bilateral damage to both hippocampi in early age. SUBJECTS AND METHODS: The total sample of 24 schoolchildren from both sexes is distributed in three groups: perinatal hypoxic-ischaemic encephalopathy and everyday complaints of memory in school age (n = 8); perinatal hypoxic-ischaemic encephalopathy without memory complaints (n = 7); and a group of typically developing (n = 9). All participants in every groups did have normal general intelligence and attention. Both clinical groups had, as another clinical consequence, spastic cerebral palsy (diplegia). Neuropsychological exam consisted on tests of general intelligence, attentional abilities, declarative memory and semantic knowledge. All participants had a brain magnetic resonance image and spectroscopy of hippocampi. Scheltens criteria were used for visual estimation of hippocampal atrophy. Parametric and non-parametric statistical contrasts were made. RESULTS: Despite preservation of semantic and procedural learning, declarative-episodic memory is impaired in the first group versus the other two groups. A significant proportion of bilateral hippocampal atrophy is only present in the first group versus the other two non-amnesic groups using Scheltens estimation on MRI. Two cases without evident atrophy did have diminished NAA/(Cho + Cr) index in both hippocampi. CONCLUSIONS: Taken together, these results contribute to delineate developmental amnesia as an specific impairment due to early partial bihippocampal damage, in agreement with previous studies. After diagnosis of developmental amnesia, a specific psychoeducational intervention must be made; also this impairment could be candidate for pharmacological trials in the future.

TITLE: Amnesia del desarrollo y daño cerebral temprano: neuropsicologia y neuroimagen.Objetivo. Contribuir a la descripcion de la amnesia del desarrollo como sindrome especifico en niños que sufrieron agresion temprana, pero no masiva, de ambos hipocampos. Sujetos y metodos. Muestra de 24 escolares de ambos sexos, de 6-16 años de edad. Se distribuye en tres grupos: pacientes afectos de encefalopatia hipoxico-isquemica perinatal, con paralisis cerebral espastica, inteligencia normal y fallos de memoria (n = 8); pacientes con similares caracteristicas, pero sin quejas de memoria (n = 7); y escolares sanos sin antecedentes de riesgo (n = 9) como grupo control. Se aplican escalas y tests para comprobar la normalidad intelectual y atencional, y para medir el perfil de rendimiento en tareas de memoria. En todos los sujetos, mediante resonancia magnetica, se estima la presencia y grado de atrofia hipocampica con la escala de Scheltens, y se calcula el indice espectroscopico NAA/(Cho + Cr). Resultados. El perfil neuropsicologico de los ocho pacientes del primer grupo es claramente sugestivo de amnesia del desarrollo, que contrasta con la normalidad en los otros grupos. En siete escolares con amnesia se constata bilateralmente algun grado de atrofia bihipocampica o disminucion significativa del indice NAA/(Cho + Cr). Conclusiones. La amnesia del desarrollo se caracteriza por afectacion de la memoria episodica con preservacion del aprendizaje semantico y procedimental. Se explica por daño parcial bihipocampico temprano. El correcto diagnostico permite una intervencion psicoeducativa especifica. En el futuro cabria ensayar terapias farmacologicas asociadas a la intervencion psicoeducativa.

[The Spanish adapted version of the Children's Communication Checklist identifies disorders of pragmatic use of language and differentiates between clinical subtypes]. / La adaptacion al castellano de la Children's Communication Checklist permite detectar las dificultades en el uso pragmatico del lenguaje y diferenciar subtipos clinicos.

INTRODUCTION: The Children's Communication Checklist (CCC) by Bishop is a useful scale for evaluation of pragmatic verbal abilities in school children. The aim of the study is to ascertain the validity and reliability of the CCC in Spanish. SUBJECTS AND METHODS: Answers to the CCC items by parents of 360 children with normal intelligence were analyzed. There were five groups: 160 control children; 68 children with attention deficit hyperactivity disorder, 77 with procedural non-verbal disorder, 25 children with social communication disorder and 30 with autism spectrum disorder. Investigations included: factorial analysis in order to cluster checklist items, reliability analyses of the proposed scales and discriminant analysis to check whether the scale correctly classifies children with pragmatic verbal abilities. RESULTS: Seven factors were obtained (Kaiser-Meyer-Olkin: 0.852) with moderate similarity with those of the original scale: social relationships, interests, and five more that can be grouped into pragmatic verbal ability (conversational abilities, coherence-comprehension, empathy nonverbal communication and appropriateness). All factors are significantly correlated with each other in the control group, and the five that compose pragmatic verbal ability correlate with each other in the clinical groups (Pearson r). The scales have good reliability (Cronbach's alpha: 0.914). The questionnaire correctly classifies 98.9% of grouped cases with and without pragmatic disorder and 78% of subjects in their appropriate clinical group. Besides, the questionnaire allows to differentiate the pathologies according to the presence and intensity of the symptoms. CONCLUSIONS: This Spanish version of the CCC is highly valid and reliable. The proposed statistics can be used as normative-reference values.

TITLE: La adaptacion al castellano de la Children's Communication Checklist permite detectar las dificultades en el uso pragmatico del lenguaje y diferenciar subtipos clinicos.Introduccion. La Children's Communication Checklist (CCC) de Bishop es una prueba util para la valoracion de la pragmatica verbal en los escolares. El objetivo del trabajo es comprobar la fiabilidad y la validez de esta escala en castellano. Sujetos y metodos. Se analiza la CCC contestada por los padres de 360 niños/as de 4-12 años, con inteligencia normal, 160 controles y 200 de cuatro grupos clinicos: trastorno por deficit de atencion/hiperactividad (n = 68), trastorno de aprendizaje no verbal procedimental (n = 77), trastorno de la comunicacion social (n = 25) y trastornos del espectro autista de nivel 1 (n = 30). Se realizan analisis: factorial para agrupar los items del cuestionario, de fiabilidad de las nuevas escalas y discriminante para comprobar si clasifica bien a los afectos de dificultades en el uso del lenguaje. Resultados. Se obtienen siete factores (Kaiser-Meyer-Olkin: 0,852) con moderada similitud a las de la CCC original: relaciones sociales, intereses y otros cinco que constituyen pragmatica (habilidades conversacionales, coherencia-comprension, compenetracion, comunicacion no verbal y pertinencia). La correlacion es significativa entre todos ellos, en el grupo control, y entre los cinco que configuran pragmatica, en los grupos clinicos (r de Pearson). La fiabilidad de las escalas es buena (alfa de Cronbach: 0,914). El cuestionario clasifica bien al 98,9% de los casos agrupados con y sin trastorno pragmatico; y al 78% de los participantes en sus correspondientes grupos clinicos. Ademas, permite diferenciar las patologias segun la presencia e intensidad de los sintomas. Conclusiones. Esta version española de la CCC es altamente valida y fiable. Los estadisticos aportados pueden utilizarse como valores de referencia.

Motricidad fetal durante el segundo trimestre de gestación: estudio ecográfico longitudinal / Motor behavior of human fetuses during the second trimester of gestation: A longitudinal ultrasound study

INTRODUCCIÓN: El objetivo de esta investigación es contribuir al conocimiento de la conducta motora fetal humana espontánea normal durante el 2.◦ trimestre de gestación. Se focaliza sobre 5 patrones de movimiento fetal: sobresaltos masivos (SM), ritmias axo-rizomélicas (RAR), estiramientos axiales (EA), movimientos generales (MG) y excursiones diafragmáticas (ED). MÉTODOS: Se ha observado la motricidad fetal espontánea, mediante ecografía obstétrica en 2 D, en una cohorte de 13 sujetos, en las semanas 12, 16, 20 y 24 de gestación. Constituye criterio de inclusión comprobar posteriormente la normalidad del estado neurológico neonatal a término y del desarrollo motor y cognitivo hasta la edad de 5 a˜nos. RESULTADOS: Los 5 patrones de movimiento citados se observan en todos los fetos durante el 2.◦ trimestre gestacional, pero su presencia y cualidad varían con la edad. Los movimientos fásicos SM y RAR son prominentes en las semanas 12 y 16 de gestación; en cambio, los movimientos prolongados EA y MG poseen mayor incidencia, duración, extensión y complejidad en las semanas 20 y 24. Las ED aumentan su incidencia a lo largo del 2.◦ trimestre, generalmente en series de 2-6 excursiones, con amplitud irregular. El ritmo cardiaco se acelera durante los periodos de movimiento fetal, frente al estado de reposo. CONCLUSIONES: Los 5 patrones de conducta estudiados ecográficamente reflejan el progresivo afinamiento de generadores de patrones motores en el sistema nervioso humano normal durante el 2.◦ trimestre de gestación. Llamamos la atención sobre las RAR, no diferenciadas en otros estudios

INTRODUCTION: The aim of this research is to contribute to knowledge of the normal spontaneous motor behavior of the human fetus during the second trimester of pregnancy. This study focuses on five patterns of spontaneous fetal movement: startle (S), axo-rhizomelic rhythmia (ARR), axial stretching (AS), general movement (GM), and diaphragmatic contraction (DC). METHODS: A cohort of 13 subjects was followed up using 2 D obstetrical ultrasound images at 12, 16, 20, and 24 weeks of gestation. As inclusion criteria, neonatal neurological examination and general movements after eutocic delivery at term were normal in all of the subjects, and their neuromotor and cognitive development until the end of pre-school age were also normal. RESULTS: All these five motor patterns are present at the beginning of the 2nd gestational trimester, but their quantitative and qualitative traits are diverse according to gestational ages. The phasic, isolated or rhythmically repeated movements, S and ARR, are prominent at 12 and 16 weeks of gestation, and then their presence gradually diminishes. By contrast, tonic and complex AS and GM movements increase their presence and quality at 20 and 24 weeks. RAR constitute a particular periodic motor pattern not described in previous literature. Moreover, the incidence of DC is progressive throughout the trimester, in clusters of 2-6 arrhythmic and irregular beats. Fetal heart rate increases during fetal motor active periods. CONCLUSIONS: All five normal behavioral patterns observed in the ultrasounds reflect the progressive tuning of motor generators in human nervous system during mid-pregnancy

[Motor behavior of human fetuses during the second trimester of gestation: a longitudinal ultrasound study]. / Motricidad fetal durante el segundo trimestre de gestación: estudio ecográfico longitudinal.

INTRODUCTION: The aim of this research is to contribute to knowledge of the normal spontaneous motor behavior of the human fetus during the second trimester of pregnancy. This study focuses on five patterns of spontaneous fetal movement: startle (S), axo-rhizomelic rhythmia (ARR), axial stretching (AS), general movement (GM), and diaphragmatic contraction (DC). METHODS: A cohort of 13 subjects was followed up using 2D obstetrical ultrasound images at 12, 16, 20, and 24 weeks of gestation. As inclusion criteria, neonatal neurological examination and general movements after eutocic delivery at term were normal in all of the subjects, and their neuromotor and cognitive development until the end of pre-school age were also normal. RESULTS: All these five motor patterns are present at the beginning of the 2(nd) gestational trimester, but their quantitative and qualitative traits are diverse according to gestational ages. The phasic, isolated or rhythmically repeated movements, S and ARR, are prominent at 12 and 16 weeks of gestation, and then their presence gradually diminishes. By contrast, tonic and complex AS and GM movements increase their presence and quality at 20 and 24 weeks. RAR constitute a particular periodic motor pattern not described in previous literature. Moreover, the incidence of DC is progressive throughout the trimester, in clusters of 2-6 arrhythmic and irregular beats. Fetal heart rate increases during fetal motor active periods. CONCLUSIONS: All five normal behavioral patterns observed in the ultrasounds reflect the progressive tuning of motor generators in human nervous system during mid-pregnancy.

Inventario del espectro autista y childhood autism rating scale: correspondencia con criterios DSM-IV-TR en pacientes con trastornos generalizados del desarrollo / Clinical usefulness of IDEA and CARS: concordance with DSM- IV -TR in children and adolescents with suspicion of PDD

INTRODUCCIÓN: El empleo de escalas basadas en la observación directa resulta útil tanto para estimar la severidad de los síntomas característicos de los trastornos generalizados del desarrollo (TGD) como para monitorizar su evolución. OBJETIVOS: a) Analizar la concordancia entre los diagnósticos basados en el Inventario del Espectro Autista (IDEA) y el Childhood Autism Rating Scale (CARS), respecto a los criterios DSM- IV -TR, en pacientes con sospecha de TGD; b) estudiar la capacidad de ambas escalas para discriminar entre diferentes diagnósticos clínicos situados dentro del espectro autista. PACIENTES Y MÉTODOS: Cincuenta y seis niños y adolescentes, entre 2 y 20 años, que acudieron a la consulta de Neuropediatría por sospecha de TGD. De forma independiente, 2 clínicos evaluaron la presencia de síntomas de TGD; uno de ellos según los criterios DSM- IV -TR y el otro de acuerdo con las escalas IDEA y CARS. RESULTADOS: La concordancia del IDEA y del CARS respecto a la clasificación DSM- IV -TR fue del 73 y 82%, con sensibilidad de 1 y 0,83 y especificidad de 0,61 y 0,82, respectivamente. Ambas escalas discriminan correctamente entre el trastorno autista y los demás diagnósticos clínicos. CONCLUSIONES: Tanto el IDEA como el CARS constituyen instrumentos útiles para la detección y monitorización del autismo en la práctica clínica diaria

INTRODUCTION: Observational scales are useful to estimate the severity of symptoms in PDD as well as to monitor their evolution. OBJECTIVES: a) To analyze the concordance between diagnoses based on the Autism Spectrum Inventory (Inventario del Espectro Autista, IDEA)) and the Childhood Autism Rating Scale (CARS), compared to DSM-IV-TR criteria, in subjects with a suspicion of pervasive developmental disorders (PDD), and b) to study the discrimination power of both scales to differentiate between a clinical diagnosis situated in the autism spectrum. PATIENTS AND METHODS: Fifty-six children and adolescents, between 2 and 20 years-old, who attended our Neuropediatric Unit due to suspicion of PDD. Independently, two clinicians evaluated the presence of PDD symptoms; one of them according to DSM-IV-TR criteria and the other one based on the application of IDEA and CARS. RESULTS: The concordance of IDEA and CARS when compared to DSM-IV-TR classification was73 and 82%, respectively, with a sensitivity of 1 and 0,83 and a specificity of 0,61 and 0,82, respectively. Both scales correctly discriminated between autistic disorder and other clinical diagnoses. CONCLUSIONS: Both IDEA and CARS are useful instruments to detect and monitor autism symptoms in the context of routine clinical practice

[Clinical usefulness of IDEA and CARS: concordance with DSM-IV-TR in children and adolescents with suspicion of PDD]. / Inventario del espectro autista y childhood autism rating scale: correspondencia con criterios DSM-IV-TR en pacientes con trastornos generalizados del desarrollo.

INTRODUCTION: Observational scales are useful to estimate the severity of symptoms in PDD as well as to monitor their evolution. OBJECTIVES: a) To analyze the concordance between diagnoses based on the Autism Spectrum Inventory (Inventario del Espectro Autista, IDEA)) and the Childhood Autism Rating Scale (CARS), compared to DSM-IV-TR criteria, in subjects with a suspicion of pervasive developmental disorders (PDD), and b) to study the discrimination power of both scales to differentiate between a clinical diagnosis situated in the autism spectrum. PATIENTS AND METHODS: Fifty-six children and adolescents, between 2 and 20 years-old, who attended our Neuropediatric Unit due to suspicion of PDD. Independently, two clinicians evaluated the presence of PDD symptoms; one of them according to DSM-IV-TR criteria and the other one based on the application of IDEA and CARS. RESULTS: The concordance of IDEA and CARS when compared to DSM-IV-TR classification was 73 and 82%, respectively, with a sensitivity of 1 and 0,83 and a specificity of 0,61 and 0,82, respectively. Both scales correctly discriminated between autistic disorder and other clinical diagnoses. CONCLUSIONS: Both IDEA and CARS are useful instruments to detect and monitor autism symptoms in the context of routine clinical practice.

[Autologous concentrated bone marrow graft in the treatment of femoral head avascular necrosis: clinical outcome after two years of follow up in a non-controlled prospective study]. / Implante de concentrado de médula ósea autógeno en el tratamiento de la necrosis isquémica de cabeza femoral: evolución clínica al segundo año de seguimiento de un estudio prospectivo no controlado.

OBJECTIVE: To assess the efficacy of implanting concentrated bone marrow rich in mesenchymal stem cells (MSC) for the treatment of femoral head avascular necrosis (AVN) to prevent or delay total hip replacement (THR). MATERIAL AND METHODS: A single-centre, prospective, non-controlled clinical study was conducted on patients with a diagnosis of AVN. The parameters assessed were, patient demographics, Harris Hip Score (HHS), imaging test (X-ray and NMR), and staging using the Arlet-Ficat scale. The patients were followed up for 1, 6, 12 and 24 months. The bone marrow was aspirated from the iliac crest, concentrated with the Harvest SmartPReP 2 system, and infused into the necrotic area by means of core decompression. RESULTS: A total of 22 hips in 17 patients were recruited between the years 2006 to 2012, with a minimum follow-up of 2 years. A mean of 119.5 mL of aspirate was extracted, with 15.25 ml of MSC being implanted. During the first 2 years of the infusion, 5 hips (24.7%) required THR, with no differences in the baseline Arlet-Ficat stage, and 4 of these (80%) had femoral head involvement equal to or higher than 30%. A significant increase of 14.27 (P=.026) in the HHS and a 0.98 (P=.089) decrease in the VAS was observed two years after the infusion in the remaining 17 hips. The results suggest that the infusion of concentrated bone marrow rich in MSC, combined with surgical decompression of the nucleus, improves hip function (HHS), and avoids THR in 75.3% of patients with AVN treated during the first 2 years.

Implante de concentrado de médula ósea autógeno en el tratamiento de la necrosis isquémica de cabeza femoral: evolución clínica al segundo año de seguimiento de un estudio prospectivo no controlado / Autologous concentrated bone marrow graft in the treatment of femoral head avascular necrosis: Clinical outcome after two years of follow up in a non-controlled prospective study

Objetivo. Evaluar la utilidad del aporte de concentrado de médula ósea rico en células troncales mesenquimales (MSC) como tratamiento de la necrosis avascular de cabeza femoral (NAV) para evitar o retrasar la artroplastia total de cadera (ATC). Material y métodos. Estudio clínico unicéntrico, prospectivo, no controlado, de pacientes con diagnóstico de NAV. Evaluación de parámetros demográficos, Harris Hip Score (HHS), pruebas de imagen (radiografía y RMN); estadificación mediante escala de Arlet-Ficat. Seguimiento a 1, 6, 12 y 24 meses. Aspirado de médula ósea de cresta ilíaca, concentrado con sistema Harvest SmartPReP 2, e infusión en zona de necrosis mediante forages. Resultados. Veintidós caderas en 17 pacientes fueron reclutados entre los años 2006-2012, seguimiento mínimo 2 años. Extracción media de 119,5ml de aspirado, implantándose 15,25ml de concentrado de MSC. Durante los primeros 2 años de la infusión 5 caderas (24,7%) precisaron ATC, sin diferencias en estadio de Ficat basal, y 4 (80%) de ellas presentaban afectación de cabeza femoral igual o superior al 30%. En las 17 caderas restantes, se observó a los 2 años de la infusión un aumento significativo del HHS (14,27) (p=0,026) y disminución del dolor (0,98) en la EVA (p=0,089). Nuestros resultados sugieren que la infusión de concentrado de médula ósea rico en MSC asociado a descompresión quirúrgica del núcleo mejora significativamente la funcionalidad de la cadera (HHS) y evita la ATC en el 75,3% de los pacientes con NAV tratados durante los primeros 2 años (AU)

Objective. To assess the efficacy of implanting concentrated bone marrow rich in mesenchymal stem cells (MSC) for the treatment of femoral head avascular necrosis (AVN) to prevent or delay total hip replacement (THR). Material and methods. A single-centre, prospective, non-controlled clinical study was conducted on patients with a diagnosis of AVN. The parameters assessed were, patient demographics, Harris Hip Score (HHS), imaging test (X-ray and NMR), and staging using the Arlet-Ficat scale. The patients were followed up for 1, 6, 12 and 24 months. The bone marrow was aspirated from the iliac crest, concentrated with the Harvest SmartPReP 2 system, and infused into the necrotic area by means of core decompression. Results. A total of 22 hips in 17 patients were recruited between the years 2006 to 2012, with a minimum follow-up of 2 years. A mean of 119.5mL of aspirate was extracted, with 15.25ml of MSC being implanted. During the first 2 years of the infusion, 5 hips (24.7%) required THR, with no differences in the baseline Arlet-Ficat stage, and 4 of these (80%) had femoral head involvement equal to or higher than 30%. A significant increase of 14.27 (P=.026) in the HHS and a 0.98 (P=.089) decrease in the VAS was observed two years after the infusion in the remaining 17 hips. The results suggest that the infusion of concentrated bone marrow rich in MSC, combined with surgical decompression of the nucleus, improves hip function (HHS), and avoids THR in 75.3% of patients with AVN treated during the first 2 years (AU)

Dificultades en la percatación rápida de incongruencias en el trastorno de aprendizaje procedimental: posible disfunción de la coherencia central / Difficulties in the swift recognition of contradictions in procedural learning disorder: possible central coherence dysfunction

Introducción. El trastorno de aprendizaje procedimental, o trastorno de aprendizaje no verbal, afecta a la automatización de destrezas y de rutinas perceptivomotoras y cognitivas. Objetivo. Profundizar en la disfunción neurocognitiva de este trastorno; en concreto, analizar las dificultades que experimentan al pasar del reconocimiento de los elementos de un conjunto a la comprensión del todo. Desarrollo. La confrontación de informaciones visuales simultáneas y la percatación rápida de incongruencias son habilidades especialmente difíciles en niños con trastorno de aprendizaje procedimental. Conclusiones. Estas dificultades pueden reflejar una disfunción de la coherencia central y explicar, en parte, el déficit en la adaptación de la conducta social que presentan estos niños (AU)

Introduction. Procedural learning disorder, or non-verbal learning disorder, affects the automation of perceptual motor and cognitive skills and routines. Aim. To further our knowledge of the neurocognitive dysfunction in this disorder and, more specifically, to analyse the difficulties experienced when going from recognition of the constituting elements of something to an understanding of the whole. Development. Contrasting simultaneous visual information and the swift recognition of contradictions are especially difficult abilities for children with procedural learning disorder. Conclusions. These difficulties may reflect a central coherence dysfunction and can partly account for the deficient ability to adapt their social behaviour displayed by these children (AU)

[Procedural learning disorder: neuropsychological characteristics]. / Trastorno de aprendizaje procedimental: características neuropsicológicas.

AIM: This research aims at neurocognitive delineation of the core features of procedural learning disorder (PLD), otherwise labeled as motor coordination disorder or non-verbal learning disorder. PATIENTS AND METHODS: A sample of 209 correlative outpatients (73% males), aged 6-12 years, all of them having QI ranging from 81 to 120, was clustered into the following neurobehavioural groups: PLD (n = 16), PLD plus attention deficit hyperactivity disorder (ADHD) (n = 37), ADHD combined type (n = 47), ADHD predominantly inattentive type (n = 23), specific language impairment (n = 68), and semantic-pragmatic language impairment (n = 18). Two additional groups of patients were included for some comparisons: children with periventricular leukomalacia (PVL) without learning disability (n = 8) or associating PLD (n = 17). A set of behavioural scales and neurocognitive tests was used to evaluate verbal and non-verbal IQ, attention, impulsivity control, visuo-motor coordination, declarative memory, procedural memory and learning, formal and functional dimensions of language, peer relationships and academic achievement. Parametric analysis were used to test the differences and similarities of neurobehavioural variables between groups. RESULTS AND CONCLUSIONS: Our results allow us to conclude that PLD implies a difficult acquisition of automatized motor, cognitive and communicative abilities required in school work and peer social relationships. PLD is different from autistic spectrum disorders. It is frequently associated to inattentive ADHD. Operational criteria for diagnosis of PLD are proposed, according to our results. A bilateral posterior parietal dysfunction is a plausible explanation of its physiopathology. Preserved general intelligence and formal linguistic abilities are the clues for intervention designs.

Trastorno de aprendizaje procedimental: características neuropsicológicas / Procedural Learning Disorder: Neuropsychological characteristics

Objetivo. Definir las características neuropsicológicas nucleares del trastorno de aprendizaje procedimental (TAP),también conocido como trastorno de la coordinación motora o del aprendizaje no verbal. Pacientes y métodos. La muestra,formada por 209 pacientes (73% varones) de edades entre los 6 y 12 años y con un cociente intelectual > 80 (rango: 81-120),se clasifica, tras análisis cluster, en los siguientes grupos: TAP (n = 16), TAP con trastorno por déficit de atención/hiperactividad(TDAH) (n = 37), TDAH combinado (n = 47), TDAH inatento (n = 23), trastorno específico del lenguaje (TEL) (n = 68)y TEL semántico-pragmático (n = 18). Además, se añaden dos grupos con leucomalacia periventricular (LPV), sin dificultadesde aprendizaje (n = 8) y con TAP (n = 17), para realizar algunas comparaciones adicionales. Se utiliza un elenco de pruebasneuropsicológicas para evaluar: cociente intelectual verbal y manipulativo, atención, control de impulsividad, integraciónvisuoespacial, memoria declarativa y procedimental, dimensiones lingüísticas formales y funcionales, lectoescritura ycompetencia social. Mediante comparaciones paramétricas, se prueban las diferencias y similitudes neurocognitivas entre losgrupos. Resultados y conclusiones. El TAP implica un déficit en los automatismos motores, cognitivos y comunicativos requeridosen el aprendizaje escolar y en la interacción social, aunque no es un trastorno de la gama autista. Frecuentemente, asociaTDAH inatento. Se proponen unos criterios operativos para el diagnóstico de TAP. Nuestros resultados apoyan que su fisiopatologíaresida en una disfunción bilateral de las estructuras parietales posteriores. Las habilidades formales del lenguajeconservadas son la clave en los planes de intervención (AU)

Aim. This research aims at neurocognitive delineation of the core features of procedural learning disorder (PLD),otherwise labeled as motor coordination disorder or non-verbal learning disorder. Patients and methods. A sample of 209correlative outpatients (73% males), aged 6-12 years, all of them having QI ranging from 81 to 120, was clustered into thefollowing neurobehavioural groups: PLD (n = 16), PLD plus attention deficit hyperactivity disorder (ADHD) (n = 37), ADHDcombined type (n = 47), ADHD predominantly innattentive type (n = 23), specific language impairment (n = 68), andsemantic-pragmatic language impairment (n = 18). Two additional groups of patients were included for some comparisons:children with periventricular leukomalacia (PVL) without learning disability (n = 8) or associating PLD (n = 17). A set ofbehavioural scales and neurocognitive tests was used to evaluate verbal and non-verbal IQ, attention, impulsivity control,visuo-motor coordination, declarative memory, procedural memory and learning, formal and functional dimensions oflanguage, peer relationships and academic achievement. Parametric analysis were used to test the differences and similaritiesof neurobehavioural variables between groups. Results and conclusions. Our results allow us to conclude that PLD implies adifficult acquisition of automatized motor, cognitive and communicative abilities required in schoolwork and peer socialrelationships. PLD is different from autistic spectrum disorders. It is frequently associated to innattentive ADHD. Operationalcriteria for diagnosis of PLD are proposed, according to our results. A bilateral posterior parietal dysfunction is a plausibleexplanation of its physiopathology. Preserved general intelligence and formal linguistic abilities are the clues for interventiondesigns. (AU)

Estilo comportamental al inicio del segundo año de vida: estudio retrospectivo en escolares afectados de trastorno por déficit de atención e hiperactividad / Children with ADHD: A retrospective description of their behavioural features as toddlers

Objetivos: Estudiar la posible relación entre el perfil comportamental de escolares afectos de trastorno por déficit de atención e hiperactividad (TDAH) y el estilo de conducta que poseían al inicio del segundo año de vida. Sujetos y métodos: Los padres de 50 niños afectados de TDAH y los padres de 30 niños controles contestaron una versión española del TBQ (Toddler Behaviour Questionnaire) acerca de sus percepciones retrospectivas de la conducta de sus hijos al inicio del segundo año de vida. Los ítems del TBQ se agruparon mediante un análisis factorial. Se estudiaron las diferencias (t-Student) de las puntuaciones del TBQ entre ambos grupos y se aplicó un análisis de correlación múltiple entre las puntuaciones en el TBQ y en la escala del Manual Diagnóstico y Estadístico de los Trastornos Mentales IV del TDAH. Resultados: Según la percepción de sus padres, los niños afectos de TDAH (como grupo) habían mostrado al inicio del segundo año un estilo de conducta diferente al de los niños del grupo control (p<0,05); estas diferencias se daban en las siguientes dimensiones de la conducta: regularidad, humor y estabilidad durante el juego. Conclusiones: Aunque los resultados del presente estudio deben considerarse con precaución, parecen mostrar que desde el quinto trimestre de vida es diferenciable un particular estilo de conducta en lo tocante a regularidad, humor y estabilidad durante el juego, que puede indicar riesgo de TDAH en edades posteriores. Este estilo de conducta debe tenerse en cuenta en la educación temprana y ser objeto de estudios prospectivos (AU)

Objectives: To study the relationship between behavioural profile of children suffering from Attention Deficit Hyperactivity Disorder (ADHD) and the previous behavioural style of these patients as toddlers. Subjects and methods: We asked the parents of 50 schoolchildren with ADHD, and those of 30 controls, to fill in a Spanish version of the Toddler Behaviour Questionnaire (TBQ) from their retrospective perception of their children's behaviour as toddlers. TBQ items were grouped by factor analysis; t-Student between the scores of both groups and a multiple correlation analysis of TBQ and DSM-IV-ADHD-RS in each of the groups were used. Results: Children in the ADHD group were reported by parents to have had a different toddler behavioural profile in comparison to that of control children (P<0.05). These differences were associated with adapting to new environments, mood, regularity and stability of play behaviour. A correlation was found between behavioural profile in DSM-IV-ADHD- RS and TBQ. Conclusions: The results of this study should be interpreted with caution. However, they suggest that in the fifth trimester of life a particular behavioural style as regards regularity, stability of play, and mood, could indicate a risk of developing ADHD in the future. This behavioural style should be taken into consideration in rearing and early education prospective studies (AU)

[Children with ADHD: a retrospective description of their behavioural features as toddlers]. / Estilo comportamental al inicio del segundo año de vida: estudio retrospectivo en escolares afectados de trastorno por déficit de atención e hiperactividad.

OBJECTIVES: To study the relationship between behavioural profile of children suffering from Attention Deficit Hyperactivity Disorder (ADHD) and the previous behavioural style of these patients as toddlers. SUBJECTS AND METHODS: We asked the parents of 50 schoolchildren with ADHD, and those of 30 controls, to fill in a Spanish version of the Toddler Behaviour Questionnaire (TBQ) from their retrospective perception of their children's behaviour as toddlers. TBQ items were grouped by factor analysis; t-Student between the scores of both groups and a multiple correlation analysis of TBQ and DSM-IV-ADHD-RS in each of the groups were used. RESULTS: Children in the ADHD group were reported by parents to have had a different toddler behavioural profile in comparison to that of control children (P<0.05). These differences were associated with adapting to new environments, mood, regularity and stability of play behaviour. A correlation was found between behavioural profile in DSM-IV-ADHD- RS and TBQ. CONCLUSIONS: The results of this study should be interpreted with caution. However, they suggest that in the fifth trimester of life a particular behavioural style as regards regularity, stability of play, and mood, could indicate a risk of developing ADHD in the future. This behavioural style should be taken into consideration in rearing and early education prospective studies.

[Detection and specific studies in procedural learning difficulties]. / Detección y estudios específicos en el trastorno de aprendizaje procesal.

INTRODUCTION: The main disabilities in non-verbal learning disorder (NLD) are: the acquisition and automating of motor and cognitive processes, visual spatial integration, motor coordination, executive functions, difficulty in comprehension of the context, and social skills. AIMS. To review the research to date on NLD, and to discuss whether the term 'procedural learning disorder' (PLD) would be more suitable to refer to NLD. DEVELOPMENT: A considerable amount of research suggests a neurological correlate of PLD with dysfunctions in the 'posterior' attention system, or the right hemisphere, or the cerebellum. Even if it is said to be difficult the delimitation between NLD and other disorders or syndromes like Asperger syndrome, certain characteristics contribute to differential diagnosis. Intervention strategies for the PLD must lead to the development of motor automatisms and problem solving strategies, including social skills. CONCLUSIONS: The basic dysfunction in NLD affects to implicit learning of routines, automating of motor skills and cognitive strategies that spare conscious resources in daily behaviours. These limitations are partly due to a dysfunction in non-declarative procedural memory. Various dimensions of language are also involved: context comprehension, processing of the spatial and emotional indicators of verbal language, language inferences, prosody, organization of the inner speech, use of language and non-verbal communication; this is why the diagnostic label 'PLD' would be more appropriate, avoiding the euphemistic adjective 'non-verbal'.

Detección y estudios específicos en el trastorno de aprendizaje procesal / Detection and specific studies in procedural learning difficulties

Introducción. Los déficit que caracterizan el trastorno de aprendizaje no verbal (TANV) afectan a la automatización de procedimientos motores y cognitivos, la integración visuoespacial, la coordinación motora, las funciones ejecutivas, la comprensión contextual y las habilidades sociales. Objetivos. Revisar el estado de la cuestión del TANV y justificar la mayor adecuación del término ‘trastorno de aprendizaje procesal’ (TAP) para referirse a este trastorno. Desarrollo. Aunque los límites entre el TANV y otros trastornos o síndromes como el síndrome de Asperger son a veces difusos, ciertas características específicas contribuyen al diagnóstico diferencial. Numerosas investigaciones sugieren, en cuanto a su correlato neurológico, disfunciones del sistema atencional ‘posterior’, hemisferio derecho, y cerebelo. Los recursos de intervención en el TAP deben ir encaminados al entrenamiento de los automatismos motores y de las estrategias de resolución de problemas. Conclusiones. La disfunción básica que subyace al TANV es una dificultad para el aprendizaje implícito de rutinas, de la automatización de procesos motores y de estrategias cognitivas que facilitan gran parte de las conductas habituales ahorrando costo de recursos conscientes. Estas limitaciones se deben en parte a una disfunción en la memoria procedimental. Además, diversas dimensiones del lenguaje están afectadas: comprensión contextualizada, procesamiento de los indicadores espaciales y emocionales en la producción verbal, realización de inferencias lingüísticas, organización del discurso, prosodia, uso del lenguaje y comunicación gestual, entre otras; de este modo, quedaría justificado evitar el adjetivo ‘no verbal’ en la denominación de esta agrupación sindrómica y adoptar la etiqueta diagnóstica de ‘TAP’ (AU)

Introduction. The main disabilities in non-verbal learning disorder (NLD) are: the acquisition and automating of motor and cognitive processes, visual spatial integration, motor coordination, executive functions, difficulty in comprehension of the context, and social skills. Aims. To review the research to date on NLD, and to discuss whether the term ‘procedural learning disorder’ (PLD) would be more suitable to refer to NLD. Development. A considerable amount of research suggests a neurological correlate of PLD with dysfunctions in the ‘posterior’ attention system, or the right hemisphere, or the cerebellum. Even if it is said to be difficult the delimitation between NLD and other disorders or syndromes like Asperger syndrome, certain characteristics contribute to differential diagnosis. Intervention strategies for the PLD must lead to the development of motor automatisms and problem solving strategies, including social skills. Conclusions. The basic dysfunction in NLD affects to implicit learning of routines, automating of motor skills and cognitive strategies that spare conscious resources in daily behaviours. These limitations are partly due to a dysfunction in non-declarative procedural memory. Various dimensions of language are also involved: context comprehension, processing of the spatial and emotional indicators of verbal language, language inferences, prosody, organization of the inner speech, use of language and non-verbal communication; this is why the diagnostic label ‘PLD’ would be more appropriate, avoiding the euphemistic adjective ‘non-verbal’ (AU)

[Aphasia in children and adolescents: evolutive aspects]. / Afasia en niños y adolescentes: aspectos evolutivos.

INTRODUCTION AND DEVELOPMENT: Reported observations of acquired aphasia in children contain all the symptoms and syndromes that have been described in adult aphasiology. Recovery of colloquial language is good even if anomia and verbal memory deficits persist as the most frequent defects. At long term outcome there are serious difficulties in dealing with written material and in academic performance in most patients.

Normal neurological outcome after congenital thyrotoxicosis: prognostic value of observation of general movements.

We followed up a patient born preterm with congenital thyrotoxicosis by observing her general movements (GMs) in accordance with Prechtl's method. Initially a chaotic pattern was observed. Along with the normalization of thyroid hormones, the GM pattern changed to a poor repertoire at four weeks of life, full-blown writhing movements at six weeks and fidgety movements at the age of four months. This is the first report of chaotic GMs in a neonate reflecting transient neurological dysfunction related to congenital thyrotoxicosis, with subsequent normal neurological and cognitive outcome.

[Prognosis of neurodevelopmental disorders from clinically-based criteria: usefulness of the observation of general movements]. / Pronóstico de los trastornos del neurodesarrollo a través de la clínica: movimientos generales.

INTRODUCTION AND DEVELOPMENT: Some of the most known classical forms for neurological examination of neonates and infants are reviewed, and their predictive qualities are discussed. Moreover the Prechtl's method of observation of general movements in preterm and term neonates and young infants up to four months posterm is particularly powerful for prediction of normality or abnormality of the future motor control. CONCLUSION: In general, the clinical approach is the principal tool in developmental neurology, even if it must be complemented by technological examinations, specially neuroimaging.

Pronóstico de los trastornos del neurodesarrollo a través de la clínica: movimientos generales / Prognosis of neurodevelopmental disorders from clinically-based criteria: usefulness of the observation of general movements

Introducción y desarrollo. Se revisan algunos de los protocolos clásicos más difundidos para el examen neurológico de neonatos y lactantes y se estudian sus cualidades pronósticas del desarrollo. Una técnica que ha emergido con particular fuerza en los últimos veinte años es el método de observación de los movimientos generales, que posee una alta fiabilidad para predecir, desde el primer trimestre de vida, la normalidad o el riesgo de patología del control motor en años ulteriores sin necesidad de manipular al bebé. Conclusión. Los métodos clínicos siguen siendo la mejor herramienta en neurología del desarrollo, ya que permiten discernir y ver emerger las formas de comportamiento normal o anómalo en los niños con riesgo, aunque los hallazgos deben complementarse con los datos que nos ofrece la tecnología actual, especialmente la neuroimagen (AU)

Introduction and development. Some of the most known classical forms for neurological examination of neonates and infants are reviewed, and their predictive qualities are discussed. Moreover the Prechtl’s method of observation of general movements in preterm and term neonates and young infants up to four months posterm is particularly powerful for prediction of normality or abnormality of the future motor control. Conclusion. In general, the clinical approach is the principal tool in developmental neurology, even if it must be complemented by technological examinations, specially neuroimaging (AU)

Tratamiento de la incontinencia salival en el niño con patología neurológica / Salivary incontinence in a child with neurological disease

La incontinencia salival, o babeo, es un problema frecuentemente asociado a varios cuadros neuropediátricos. En los casos severos conlleva grandes consecuencias clínicas y psicosociales. Se han propuesto múltiples procedimientos para su tratamiento, con resultados variables. El primer escalón terapéutico consiste en la combinación de entrenamiento logopédico o neurorrehabilitador y la aplicación de fármacos anticolinérgicos, entre los cuales los más utilizados son el trihexifenidilo y la escopolamina, este último aplicado mediante parches transdérmicos. Dados los efectos indeseables a distancia de estos fármacos, se comienza a utilizar la infiltración de las glándulas submaxilares con toxina botulínica A, bajo control ecográfico. Este procedimiento es efectivo en el 50% de los casos, pero presenta el inconveniente de que su efecto es temporal, y se requiere repetir la infiltración cada 4-6 meses. Finalmente, en pacientes con babeo invalidante que no responden a la terapia farmacológica y rehabilitadora, a largo plazo estaría indicada la realización de una técnica supresora definitiva. En la actualidad la técnica es más segura y eficaz es la combinación de una submaxilectomía bilateral y la ligadura de ambos conductos parotídeos. Este sencillo procedimiento elimina de forma completa la incontinencia salival en más del 90% de los casos, con mínimas complicaciones, y debe seguirse de una correcta higiene bucodental

In several pediatric neurological conditions, drooling is a major problem, leading to negative clinical and psychosocial consequences. The first therapeutic measure to improve salivary continence is a combination of neurorehabilitative techniques, including speech therapy. However, the results are frequently unsastisfactory in mentally handicapped patients with pseudobulbar dysfunction. Several anticholinergic drugs – trihexiphenidyl, glycopyrrolate and transdermal scopolamine – have been tried with variable beneficial effects on salivary incontinence; however, given their general muscarinic effects, these drugs are poorly tolerate by many patients. A recent open trial with botulinum toxin type A, administered by means of ultrasound-guided injection into submaxillary glands, produced encouraging results in a series of children with cerebral palsy, and undesirable effects were rare. Further controlled trials are to be carried out. Moreover, the literature on surgical procedure is reviewed. A combination of bilateral excision of submaxillary glands and parotid duct ligation seems to be the best approach for patients with refractory and disabling salivary incontinence