[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.

[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.

[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.

[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.

[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'.

[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.

[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.

[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.

[Executive system and frontal lobe lesions in childhood]. / El sistema ejecutivo y las lesiones frontales en el niño.

INTRODUCTION AND DEVELOPMENT: Frontal lobe syndrome in adulthood is characterised by executive function deficits leading to altered behavioural control with difficulties in social interactions and in maintaining stable jobs and interpersonal relationships. Generalisation of this concept to children with early frontal lobe damage is not straightforward. There are complex interactions between the effects of the lesion itself and the effects in other interconnected regions, timing of lesion, how long it was since the lesion occurred to the time of evaluation, and how old the child is at examination. These facts lead to consider that there might be a number of 'frontal syndromes' in childhood rather than a unique one. We report 9 cases of children with early frontal lobe lesions who were followed up for an average of 10 years. CONCLUSION: A variety of different outcomes suggests that prognosis for these patients might be better that previously reported.

[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.

[Developmental amnesias]. / Amnesias del desarrollo.

OBJECTIVE: The literature on clinical and physiophatologic characteristics of the spectrum of memory disorders in childhood is reviewed in this article. DEVELOPMENT: There are only a few detailed reports of permanent specific memory disorders in children. Early anoxo ischaemic bihippocampal injuries can cause a selective permanent impairment of episodic daily life memory with preservation of semantic learning and general intelligence; this dissociation has been related to partial hippocampal damage whilst the entorhinal, parahippocampal and prefrontal cortices, which are critical to systematized memorizing and work memory, keep normal. Biological psychiatry research has shown that early childhood amnesias after psychological maltreatment or abuse could be related to damage in neuronal systems which support memory, caused by glutamatergic cascade. Both severe bilateral hippocampal sclerosis (also mediated by toxic neurotransmitters) in early malignant epilepsies, and massive bilateral damage of mesial temporal lobes due to herpex virus encephalitis or Reye's syndrome, cause severe amnesic deficits, frequently accompanied by absence of any language development and autism with features of Klüver-Bucy syndrome. There are also on record some examples of Korsakoff's syndrome in children with midfossa tumors. CONCLUSIONS: All types of classical amnesias described in adults have been observed in children. Developmental amnesias are probably more frequent than currently presumed. It must be paid special attention to selective autobiographical memory impairments in individuals who underwent a partial bihippocampal damage in perinatal or early postnatal periods; they are to be distinguished from, although it may coexist with, other clinical situations such as attention deficit disorder or semantic pragmatic disorder.

[Genetic studies in communication disorders]. / Estudios genéticos de los trastornos de la comunicación.

OBJECTIVES: To review current literature on population, cytogenetic and molecular studies of specific language disorders (SLD) and pervasive developmental disorders (PDD). DEVELOPMENT: Clinical concordance studies in twins and in vertical familial groups suggest polygenic multifactorial modes of inheritance, but in some families an autosomal dominant model may be present. The data favour not a modular, but rather a molar model of the relationship between genes and neural abilities for communicative behaviors. Several extensive genome screenings have demonstrated linkage to specific markers on 7q for SLD, and on 7q and 2q for PDD. The strong evidence of linkage on 7q for both disorders has led to the hypothesis that this region contains several separate quantitative trait loci (QTL) related to different communicative abilities. Mutations in different QTL would facilitate the different disabilities and stereotyped behaviors associated with the phenotypic spectrum of PDD. There are other candidate regions for QTLs but the linkage is weaker and there is little agreement between studies; due, in part, to over extensive inclusion criteria and small sizes of familial groups. CONCLUSIONS: To enhance linkage research in further molecular genetic studies, clinicians must refine behavioral target traits when selecting familial groups and enlarge the size of familial groups by including non handicapped members with related behavioral traits. At present, a chromosome region in 7q shows the strongest evidence for communication related QTL, but other QTL need to be identified elsewhere in the genome in order to explain the genetic contribution to the large spectrum of language and autistic disorders.

Gait epilepsy. A case report of gait-induced seizures.

Reflex epilepsy includes a group of epileptic syndromes in which seizures are induced by a stimulus, either simple (visual, somatosensory, olfactory, auditory) or more complex (e.g., eating, thinking, reading). We document a case of reflex epilepsy in which focal seizures are triggered exclusively by gait. The patient is a young boy whose walking was impaired by abnormal motor phenomena on the left side. These phenomena were elicited by gait and were accompanied by a distinctive ictal pattern with centro-temporal discharges. After comparing this patient with others reported in the literature, we determined that he has an unusual type of reflex epilepsy for which we coined the term "gait epilepsy." This disorder must be considered when physicians are making a differential diagnosis in patients who have symptoms that suggest paroxysmal kinesigenic dystonia (PKD) or selective epileptic gait disorder.