[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.

[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.

[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.

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

Introducción y desarrollo. El análisis de las observaciones publicadas de afasia por lesión perisilviana cortical o subcortical en niños y adolescentes muestra que la semiología en este tramo etario es de la misma riqueza que en el adulto, si bien los trastornos de la comprensión son proporcionalmente menores en los niños. Por otra parte, la recuperación del lenguaje coloquial es más rápida y satisfactoria, aunque frecuentemente persisten déficit, de los cuales el más habitual es la anomia; los déficit en memoria verbal y la dificultad para trabajar con material escrito se observan en la mayoría de los casos, todo lo cual interfiere con el progreso académico (AU)

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 (AU)

[Subtypes of specific language impairment in Spanish-speaking children: a cluster analysis of linguistic features]. / Subtipos de trastorno específico del desarrollo del lenguaje: perfiles clínicos en una muestra hispanohablante.

INTRODUCTION: Specific language impairment (SLI) can be viewed as a continuum with different subtypes depending on the combination of deficits and strengths in each of the components of linguistic behavior. AIM. To analyze the phenomenological profiles in a correlative series of Spanish children in order to facilitate their endophenotypic differentiation and the choice of strategies of intervention. PATIENTS AND METHODS: 86 referred children, aged 4 to 9 years, with a mean non-verbal intelligence quotient of 93 (range 72-114) and accomplishing clinical criteria of SLI. Apart from clinical interview and neurological examination, a protocol of questionnaires and tests was used in all subjects in order to measure their formal (phonological, syntactic, lexical) and functional (semantic and pragmatic) linguistic abilities. A cluster analysis of variables was used in order to investigate SLI subtypes. RESULTS: In the total sample, a 24% of whole series have a pure phonological expressive disorder. 55% of subjects have mixed receptive/expressive disorders; from these, the most pervasive subtype is due to verbal agnosia (11%), but more frequently observed subtypes affect syntax reception and expression combined to difficulties in phonological programming (36%) or in lexical retrieval (8%). Furthermore, 21% of the total sample present with difficulties in pragmatic use of language even if their formal linguistic abilities and non-verbal behavior are normal. CONCLUSIONS: Our empirical approach confirms that the admitted SLI subtypes in international literature applies to Spanish-speaking children for endophenotyping and intervention-planning purposes.

Subtipos de trastorno específico del desarrollo del lenguaje: perfiles clínicos en una muestra hispanohablante / Subtypes of specific language impairment in spanish-speaking children: a cluster analysis of linguistic features

Introducción. Los subtipos internacionalmente admitidosen el continuo de los trastornos específicos del desarrollo dellenguaje (TEL) derivan del análisis de niños angloparlantes, perono se han contrastado suficientemente hasta ahora en una poblacióncon el castellano como idioma de base. Objetivo. Se trata deobtener una clasificación derivada de la fenomenología clínica depacientes hispanohablantes. Pacientes y métodos. 86 pacientes correlativos,de 4 a 9 años, que cumplen criterios clínicos de TEL,con un cociente intelectual no verbal entre 72 y 114 (media: 93). Atodos se les aplicó un protocolo de entrevista y de pruebas paramedir sus capacidades lingüísticas en los aspectos formales y funcionales.Los datos se han sometido a análisis de conglomerados.Resultados. Del total de la serie, un 24% presenta un trastornolimitado a la programación fonológica expresiva. En otro 55%concurren trastornos mixtos en las vertientes de comprensión y deexpresión, que van desde la afectación masiva derivada de unaagnosia verbal congénita (11%) a trastornos menos graves quecombinan dificultad en la sintaxis receptiva y expresiva con trastornode la fonología (36%) o de acceso al léxico (8%). Por otraparte, el 21% de la serie se caracteriza por dificultades en la pragmáticadel lenguaje, pese a que tanto el dominio formal de lasreglas lingüísticas como la conducta extraverbal estén en límitesaceptables. Conclusiones. Nuestro estudio confirma la adecuación,en niños hispanohablantes, de los subtipos de TEL actualmenteadmitidos en la bibliografía internacional, con vistas al fenotipadoy a la planificación de estrategias de intervención

Introduction. Specific language impairment (SLI) can be viewed as a continuum with different subtypes depending onthe combination of deficits and strengths in each of the components of linguistic behavior. Aim. To analyze the phenomenologicalprofiles in a correlative series of Spanish children in order to facilitate their endophenotypic differentiation and the choice ofstrategies of intervention. Patients and methods. 86 referred children, aged 4 to 9 years, with a mean non-verbal inteligencequotient of 93 (range 72-114) and accomplishing clinical criteria of SLI. Apart from clinical interview and neurologicalexamination, a protocol of questionnaires and tests was used in all subjects in order to measure their formal (phonological,syntactic, lexical) and functional (semantic and pragmatic) linguistic abilities. A cluster analysis of variables was used inorder to investigate SLI subtypes. Results. In the total sample, a 24% of whole series have a pure phonological expressivedisorder. 55% of subjects have mixed receptive/expressive disorders; from these, the most pervasive subtype is due to verbalagnosia (11%), but more frequently observed subtypes affect syntax reception and expression combined to difficulties inphonological programming (36%) or in lexical retrieval (8%). Furthermore, 21% of the total sample present with difficultiesin pragmatic use of language even if their formal linguistic abilities and non-verbal behavior are normal. Conclusions. Ourempyrical approach confirms that the admitted SLI subtypes in international literature applies to Spanish-speaking childrenfor endophenotyping and intervention-planning purposes

[Memory and attention in children with brain dysfunction]. / Trastornos de memoria y de atención en disfunciones cerebrales del niño.

INTRODUCTION: For conscious behaviours to continue, attention and working memory must be brought into play in order to match the data from the current reality with those in the long-term memory and to the sequential plans for adaptive action. PATIENTS AND METHODS: We present the preliminary findings of our study of 50 patients between 7 and 16 years of age, who were divided into three groups according to their dominant neurocognitive disorder: a 'pure' ADHD group, an ADHD/LD group (with comorbid learning disorder) and an RMEM (risk of memory disorder due to bilateral temporomesial damage) group. All of them were administered a battery of tests designed to evaluate attentional control and short and long-term memory. RESULTS: Verbal IQ was above 80 in all the participants, with no differences among the three groups. The ADHD/LD group had a manipulative IQ and level of sustained attention below those of the ADHD group, but no long-term memory deficit was observed in either of these two groups. In contrast, the RMEM group's performance was poorer in verbal memory tasks, although their behaviour is more attentive and stable compared to that of the other two groups. CONCLUSIONS: Neuropsychological analysis of these dissociations allows adjustments to be made in the strategies for therapeutic interventions. Performance in long-term learning in children with ADHD and ADHD/LD can be improved with psychostimulants owing to the activating effect they have on working memory.

Trastornos de memoria y de atención en disfunciones cerebrales del niño / Memory and attention in children with brain dysfunction

Introducción. La continuidad de las conductas conscientes requiere la puesta en juego de la atención y de la memoria operativa, o memoria de trabajo, para conjugar los datos de la realidad actual con los de la memoria a largo plazo y con los planes secuenciales de acción adaptativa. Pacientes y métodos. Mostramos los resultados preliminares de nuestro estudio de 50 pacientes con edades entre 7 y 16 años, distribuidos en tres grupos según el trastorno neurocognitivo dominante: grupo TDAH ‘puro’, grupo TDAH/TA (con trastorno de aprendizaje comórbido) y grupo RMEM (riesgo de trastorno de memoria por daño temporomesial bilateral). A todos se les ha aplicado una batería de pruebas de control atencional y de memoria a corto y largo plazo. Resultados. El CI verbal es superior a 80 en todos los participantes, sin diferencias entre los tres grupos. El grupo TDAH/TA tiene un CI manipulativo y una atención sostenida más bajos que el grupo TDAH, pero no se han objetivado déficit de memoria a largo plazo en ninguno de estos dos grupos. En cambio, el grupo RMEM rinde más bajo en tareas de memoria verbal, a pesar de que su conducta es más atenta y estable, comparativamente con los otros dos grupos. Conclusiones. El análisis neuropsicológico de estas disociaciones permite ajustar las estrategias de intervención terapéutica. El rendimiento en aprendizajes a largo plazo en niños con TDAH y TDAH/TA puede ser mejorado con psicoestimulantes por su efecto activador de la memoria de trabajo (AU)

Introduction. For conscious behaviours to continue, attention and working memory must be brought into play in order to match the data from the current reality with those in the long-term memory and to the sequential plans for adaptive action. Patients and methods. We present the preliminary findings of our study of 50 patients between 7 and 16 years of age, who were divided into three groups according to their dominant neurocognitive disorder: a ‘pure’ ADHD group, an ADHD/LD group (with comorbid learning disorder) and an RMEM (risk of memory disorder due to bilateral temporomesial damage) group. All of them were administered a battery of tests designed to evaluate attentional control and short and long-term memory. Results. Verbal IQ was above 80 in all the participants, with no differences among the three groups. The ADHD/LD group had a manipulative IQ and level of sustained attention below those of the ADHD group, but no long-term memory deficit was observed in either of these two groups. In contrast, the RMEM group’s performance was poorer in verbal memory tasks, although their behaviour is more attentive and stable compared to that of the other two groups. Conclusions. Neuropsychological analysis of these dissociations allows adjustments to be made in the strategies for therapeutic interventions. Performance in long-term learning in children with ADHD and ADHD/LD can be improved with psychostimulants owing to the activating effect they have on working memory (AU)

[Neurocognitive abilities in schoolchildren with periventricular leukomalacia. Preliminary results in 15 patients]. / Habilidades neurocognitivas asociadas a la leucomalacia periventricular. Resultados preliminares en 15 sujetos.

INTRODUCTION: Apart from chronic motor disorder and the possible sensory deficits (visual, propioception) associated to periventricular leukomalacia (PVL), disorders involving the integration of higher functions due to bilateral injury to the occipital parietal junction are relatively common. AIMS: Our aim was to further our knowledge of the neuropsychological characteristics of this non progressive cerebral motor disorder. Patients and methods. We analysed a sample of 15 patients with spastic diplegia due to PVL who did not present mental retardation (verbal IQ> 75 and mean verbal IQ= 91, WISC R) in order to evaluate their visual gnosis praxis, verbal and visual memory, psycholinguistic and attentional capabilities. RESULTS: Disorders involving visuospatial integration and visual constructive memory are frequent. Long term verbal memory is poor (in fact in some cases it is clearly deficient), but can be improved with associative learning. They do not present any difficulties regarding attention and some subjects display deficiencies in the pragmatic use of language. This research is still underway and the findings presented here are preliminary. CONCLUSIONS: The neuropsychological profile of the diplegic children who were studied shares the characteristics of non verbal learning disorder. The declarative memory disorder observed in several of the subjects in our sample, who were all born premature and below weight, can be accounted for by a bihippocampal pathology or by a dysexecutive disorder.

[Clinical profiles and transitions in the spectrum of specific language impairment in childhood]. / Perfiles clínicos evolutivos y transiciones en el espectro del trastorno específico del desarrollo del lenguaje.

OBJECTIVES: To test the clinical nature and diversity of specific language impairment (SLI) in Spanish individuals, in the framework of the neuropsycholinguistic model of Chevrie-Muller and the classification of Rapin and Allen. PATIENTS AND METHODS: Forty two language impaired children with normal intelligence and audition have been followed during a mean period of 3 years 7 months in preschool and school ages; all of them have participated in an intervention program for language abilities. The evolutive neuropsychological evaluation at our hospital has comprised measurement of general cognitive abilities, formal (phonology, vocabulary, syntax) and pragmatic aspects of language. RESULTS: Phonological and syntactic abilities are the better evolutive predictors. The group clinical profiles, in general, fill into the clustering system proposed by Rapin and Allen, but mixed transitional forms are observed in individuals. Also in six patients a transition from one to another clinical form have been observed; in these subjects, mixed phonological syntactical syndrome is an obligate step during their evolutive changes; these changes can be attributed to natural evolutive processes and/or to effects of intervention. CONCLUSION: Our results permit hypothesize that the spectrum of SLI is an unique basic disorder whose clinical manifestations (interindividual and intraindividual during evolution) can be diverse in form and severity. This clinical interpretation is reinforced by the results of recent publications showing that members of the same familial group with identical genetic mutation had different forms of SLI.

Perfiles clínicos evolutivos y transiciones en el espectro del trastorno específico del desarrollo del lenguaje / Clinical profiles and evolutive transitions in the spectrum of specific language impairment in childhood

Introducción. Con la denominación trastorno específico del desarrollo del lenguaje (TEDL) o disfasia nos referimos al déficit que presentan algunos niños en el desarrollo de las habilidades para descodificar y codificar el lenguaje, teniendo unas capacidades sensoriales, cognitivas y motóricas no afectadas. Objetivo. Se pretende profundizar en la naturaleza del TEDL, así como sus características clínicas y evolutivas, siguiendo el modelo neuropsicolingüístico de Chevrie-Muller y la clasificación clínica de Rapin y Allen. Pacientes y métodos. En una muestra de extracción clínica formada por 42 niños disfásicos se ha estudiado el lenguaje en dos dimensiones: formal (fonología y morfosintaxis) y funcional (uso cognitivo y pragmático), a través de la evaluación neuropsicológica realizada en diferentes momentos evolutivos (media del tiempo de seguimiento: tres años y siete meses). Resultados. El desarrollo fonológico y el sintáctico se señalan como variables predictoras de la evolución de un sujeto. Se descubren los criterios homogéneos de clasificación y las transiciones diagnósticas, y se aportan los perfiles clínicos de cada uno de los subgrupos del TEDL, que constituyen un continuo en el espectro de esta patología. Conclusiones. En este estudio descriptivo se evidencia la unicidad del lenguaje y se considera que un sujeto con TEDL puede pasar de una categoría diagnóstica a otra, puesto que se tratan de distintas manifestaciones del mismo trastorno ligadas a variables evolutivas y de intervención psicopedagógica. (AU)

Objectives. To test the clinical nature and diversity of specific language impairment (SLI) in Spanish individuals, in the framework of the neuropsycholinguistic model of Chevrie-Muller and the classification of Rapin and Allen. Patients and methods. Forty-two language impaired children with normal intelligence and audition have been followed during a mean period of 3 years 7 months in preschool and school ages; all of them have participated in an intervention program for language abilities. The evolutive neuropsychological evaluation at our hospital has comprised measurement of general cognitive abilities, formal (phonology, vocabulary, syntax) and pragmatic aspects of language. Results. Phonological and syntaxic abilities are the better evolutive predictors. The group clinical profiles, in general, fill into the clustering system proposed by Rapin and Allen, but mixed-transitional forms are observed in individuals. Also in six patients a transition from one to another clinical form have been observed; in these subjects, mixed phonological-syntactical syndrome is an obligate step during their evolutive changes; these changes can be attributed to natural evolutive processes and/or to effects of intervention. Conclusion. Our results permit hypothetize that the spectrum of SLI is an unique basic disorder whose clinical manifestations (interindividual and intraindividual during evolution) can be diverse in form and severity. This clinical interpretation is reinforced by the results of recent publications showing that members of the same familial group with identical genetic mutation had different forms of SLI (AU)