Showing papers in "Developmental Medicine & Child Neurology in 2008"

Development and reliability of a system to classify gross motor function in children with cerebral palsy

Abstract: To address the need for a standardized system to classify the gross motor function of children with cerebral palsy, the authors developed a five-level classification system analogous to the staging and grading systems used in medicine. Nominal group process and Delphi survey consensus methods were used to examine content validity and revise the classification system until consensus among 48 experts (physical therapists, occupational therapists, and developmental pediatricians with expertise in cerebral palsy) was achieved. Interrater reliability (kappa) was 0.55 for children less than 2 years of age and 0.75 for children 2 to 12 years of age. The classification system has application for clinical practice, research, teaching, and administration.

Content validity of the expanded and revised Gross Motor Function Classification System.

Abstract: The aim of this study was to validate the expanded and revised Gross Motor Function Classification System (GMFCS-E&R) for children and youth with cerebral palsy using group consensus methods. Eighteen physical therapists participated in a nominal group technique to evaluate the draft version of a 12- to 18-year age band. Subsequently, 30 health professionals from seven countries participated in a Delphi survey to evaluate the revised 12- to 18-year and 6- to 12-year age bands. Consensus was defined as agreement with a question by at least 80% of participants. After round 3 of the Delphi survey, consensus was achieved for the clarity and accuracy of the descriptions for each level and the distinctions between levels for both the 12- to 18-year and 6- to 12-year age bands. Participants also agreed that the distinction between capability and performance and the concept that environmental and personal factors influence methods of mobility were useful for classification of gross motor function. The results provide evidence of content validity of the GMFCS-E&R. The GMFCS-E&R has utility for communication, clinical decision making, databases, registries, and clinical research.

The gross motor function measure: a means to evaluate the effects of physical therapy.

Abstract: This paper reports the results of a study to validate a measure of gross motor function in detecting change in the motor function of disabled children. Physiotherapists used this instrument to assess 111 patients with cerebral palsy, 25 with head injury and 34 non-disabled preschool children on two occasions, the second after an interval of four to six months. Parents and therapists independently rated the children's function within two weeks of each assessment, and a sample of paired assessments was videotaped for 'blind' evaluation by therapists. Correlations between scores for change on this measure and the judgments of change by parents, therapists and 'blind' evaluators supported the hypothesis that the instrument would be responsive to both negative and positive changes.

Developmental Dyslexia: A Diagnostic Approach Based on Three Atypical Reading-Spelling Patterns

Abstract: SUMMARY A method of diagnosing developmental dyslexia is presented, based on three atypical patterns of reading and spelling. These three atypical patterns enable dyslexic children to be classified into one of three main subtypes—dysphonetic (unable to integrate symbols with their sounds), dyseidetic (unable to perceive letters and whole words as configurations or gestalts), or a combination of both. One of these three patterns is found in all severely retarded readers who fulfil standard diagnostic criteria for developmental dyslexia, and none are found among children who read and spell normally. The relationship between a dyslexic child's pattern of reading and pattern of spelling is so consistent that one is predictive of the other. Long-term observation of the children in this study shows that the reading-spelling pattern remains consistent, though less obvious, even when the actual level of reading and spelling rises significantly. These findings strongly suggest that the three reading-spelling patterns are diagnostic in themselves, each of the three subtypes having different prognostic and therapeutic implications. RESUME Dyslexie de developpement: approche diagnostique basee sur trois schemes atypiques de lecture et d'orthographe Une methode de diagnostic de la dyslexie de developpement est decrite, basee sur trois schemes atypiques de lecture et d'orthographe. Ces trois schemes atypiques permettent de classer les enfants dyslexiques en trois sous-groupes principaux: dysphonetiques (incapables d'integrer les symboles et les sons), dyseidetiques (incapables de considerer les lettres et les mots comme des ensembles ou gestalts) ou une combinaison des deux. Un de ces trois patterns est trouve chez tous les enfants gravement retardes dans la lecture et qui repondent aux criteres diagnostiques standard de dyslexie de developpement et aucun ne sont trouves chez les enfants qui lisent et epellent normalement. La relation entre le type de lecture et le type d'orthographe chez un enfant dyslexique est tellement forte que la presence d'un pattern permet de prevoir l'autre. Une observation a long terme des enfants de cette etude montre que le pattern de lecture et d'orthographe persiste alors meme que l'efficacite reelle de lecture et d'orthographe augmente significativement. Ces decouvertes suggerent nettement que les trois schemes de lecture-orthographe constituent des categories diagnostiques, chacun des trois sous-groupes ayant un pronostic et des implications therapeutiques differents. ZUSAMMENFASSUNG Entwicklungsstorung des Lesens: ein diagnostischer Annaherungsversuch begrundet auf 3 atypischen Lese- und Buchstabierverhaltensweisen Eine Methode, eine Entwicklungsstorung des Lesens zu diagnostizieren wird vorgestellt. Sie basiert auf drei atypischen Verhaltensweisen beim Lesen und Buchstabieren. Diese Verhaltensweisen ermoglichen es legasthenische Kinder in eine der drei Untergruppen einzuordnen, in die dysphonetische Gruppe (Unfahigkeit Symbole und Laute zusammenzufugen) in die dyseidetische Gruppe (Unfahigkeit die Bedeutung von Buchstaben und Wortern als Gestalten wahrzunehmen) und in die Gruppe, die auf einer Kombination beider Unfahigkeiten beruht. Eine dieser drei Arten ist bei allen schwer lesegestorten Personen zu finden sein, die die ublichen diagnostischen Kriterien einer Leseschwache aufweisen, keine davon wird man bei normal lesenden und buchstabierenden Kindern finden. Die verwandtschaft der Art zu Lesen und zu Buchstabieren ist bei einem legasthenischen Kind so ubereinstimmend, dass eines aus dem anderen hervorgeht. Langzeitbeobachtungen der Kinder dieser Studie zeigen, das die Verhaltensweisen beim Lesen und Buchstabieren bestehen bleibt, selbst wenn die Fahigkeit zu Lesen und zu Buchstabieren signifikant zugenommen hat. Diese Ergebnisse legen nahe, das die drei atypischen Lese und Buchstabierverhaltensweisen in ihrer Art diagnostizierbar sind. Jede der drei Gruppen hat eine unterschiedliche Prognose und verlangt eine unterschiedliche Therapie. RESUMEN Dislexia de desarrollo; una aproximacion diagnostica basada en tres patrones atipicos de lectura y ortografia Se presenta un metodo de diagnosticar la dislexia de desarrollo basado en tres patrones atipicos de lectura y ortografia. Estos tres patrones hacen posible el clasificar los ninos dislexicos en uno de los tres siguientes subgrupos: “Disfonetico” (incapaz de integrar simbolos y sonidos), “diseidetico” (incapaz de percibir las letras y las palabras como esquemas, o gestalts) o bien una combinacion de ambos. Uno de estos tres patrones se encuentra en todos los ninos con grave retraso de lectura, que cumplen con los criterios diagnosticos standard para la dislexia de desarrollo, mientras que no se encuentra ninguno en los ninos que leen y escriben normalmente. La relacion entre los patrones de lectura y de ortografia en el nino dislexico es tan consistente que uno es predictivo del otro. La observacion a largo plazo de los ninos en este estudio muestra que el patron de lecto-escritura permanece consistente, aunque menos obvio, incluso si el nivel de lectura y ortografia aumenta significativamente. Estos hallazgos sugieren fuertemente que los tres patrones de lecto-escritura son diagnosticos por si mismos, si bien cada uno de los tres subgrupos tiene una diferente implicacion pronostica y terapeutica.

Cerebral Palsy Epidemiology: Where are We Now and Where are We Going?

Abstract: OVER the past decade, regular international meetings devoted to the state of the art in the epidemiology of cerebral palsy have been held: in California in 1987, in Cambridge in 1989 and in Brioni, Yugoslavia, in 1990. Among the longstanding problems considered have been those of case definition, case-finding, reported changes in prevalence, the bases on which distinctions between different clinical types and meaningful classification can be achieved, and the crucial question of the timing and nature of the original insult. Reports of the meetings have not been published, however, so this annotation is intended to document some of the recurrent questions that have been considered.

Terminology and classification of cerebral palsy.

Abstract: A SMALL group” met recently in Edinburgh to discuss the terminology and classification of cerebral palsy. At the meeting, a good deal of thinking and discussion went into the basis of medical classification in general, and attention was drawn to the extremely loose way in which, in the cerebral palsy field, we have used terms in the past and continue to do so today. The group reached agreement on a definition of cerebral palsy, but ran into difficulties after this. Cerebral palsy, they agreed, is one of a group of conditions generally described as ‘Syndromes of Cerebral Dysfunction’. This fitted into the proposals put forward by DENHOFF and ROBINAULT,~ and the only serious criticism was that the term ‘cerebral’ does not strictly include all parts of the brain, although most people would accept this extension of its meaning. Cerebral palsy is therefore defined as : ‘A disorder of movement and posture due to a defect or lesion of the immature brain.’ For practical purposes it is usual to exclude from cerebral palsy those disorders of posture and movement which are (1) of short duration, (2) due to progressive disease, or (3) due solely to mental deficiency. The group felt that this simple sentence could be readily translated into other languages and hoped that it might be universally accepted. They felt that it is wiser at the present time not to define precisely what they meant by ‘immature brain’, as any such definition might lead to administrative difficulties. The individual clinician dealing with the few cases arising in older children could decide whether it was reasonable to describe a certain child as having cerebral palsy. Translators do not always find the word ‘palsy’ easy to cope with, as the more exactminded expect ‘palsy’ to mean the same as ‘paralysis’ and by ‘paralysis’ understand what others would term ‘complete paralysis’. It might be well in English and American neurological and medical writing to make greater use of the word ‘paresis’ where power is present but impaired. The group ran into considerable difficulty when they went on to discuss the classification of various types of cerebral paresis. Indeed, they barely got beyond discussing the word ‘spastic’. It was not only that the word is used by lay people to describe anybody with cerebral palsy, but that there are various medical uses of the term. Sometimes it is used to describe a particular clinical syndrome rather common in the whole group of cerebral palsy, whatever the stage of evolution of the clinical picture. Sometimes its use is limited

Clumsiness in Children‐Do they Grow out Of It? A 10‐Year Follow‐Up Study

Abstract: The question of whether problems of motor co-ordination in early childhood recede with age has rarely been addressed. This paper reports the findings from a follow-up study of 17 children, identified by their teachers as having poor motor co-ordination at age six. Now age 16, these children and their matched controls completed a battery of assessments. The results suggest that the majority of children still have difficulties with motor co-ordination, have poor self-concept and are experiencing problems of various kinds in school. However, there are individual differences in the extent to which the children have learned to cope with their continuing difficulties over the years.

‘Puppet’ Children A Report on Three Cases

Abstract: UMMARY Three unrelated children are described with similar physical abnormalities of congenital origin, reminiscent of puppet children, and profound mental retardation. RESUME Enfants ‘marionnettes’ L'auteur decrit trois enfants atteints d'anomalies physiques similaires d'origine congenitale et d'un retard mental profond. ZUSAMMENFASSUNG ‘Puppenkinder’ Drei Kinder werden beschrieben, mit einander gleichenden physischen Abnormitaten kongenitalen Ursprungs sowie schwerwiegender geistiger Zuruckgebliebenheit. RESUMEN Ninos ‘munecos’ Se describen tres ninos con anormalidades fisicas parecidas, de origen congenita, ademas de atraso mental profundo.

Development of the Gross Motor Function Classification System for cerebral palsy.

Abstract: The Gross Motor Function Classification System (GMFCS) for cerebral palsy has been widely used internationally for clinical, research, and administrative purposes. This paper recounts the ideas and work behind the creation of the GMFCS, reports on the lessons learned, and identifies some philosophical challenges inherent in trying to develop an ordered, valid, and consistent system to describe function in children and adolescents with developmental differences. It is hoped that these ideas will be useful to others who choose to expand the field with additional systems in other areas of childhood neurodisability.

Lower‐Extremity strength profiles in spastic cerebral palsy

Abstract: Although weakness has been identified in cerebral palsy (CP) in isolated muscle groups, the magnitude of weakness in multiple muscles and the patterns of weakness across joints have not been documented. The maximum voluntary contraction of eight muscle groups in the lower extremities of 15 children with spastic diplegia, 15 with spastic hemiplegia, and 16 age-matched peers was determined using a hand-held dynamometer. Children with spastic diplegia were shown to be weaker than age-matched peers in all muscles tested, as were the children with hemiplegia on the involved side, with strength differences also noted on the uninvolved side. Weakness was more pronounced distally in the groups with CP, and the hip flexors and ankle plantarflexors in spastic CP tended to be relatively stronger than their antagonists as compared with the strength ratios of the comparison group. In conclusion, children with spastic CP demonstrate quantifiable lower-extremity weakness and muscle imbalance across joints.

Autism in thalidomide embryopathy: a population study

Abstract: Of a population of 100 Swedish thalidomide embryopathy cases, at least four met full criteria for DSM-III-R autistic disorder and ICD-10 childhood autism. Thalidomide embryopathy of the kind encountered in these cases affects fetal development early in pregnancy, probably on days 20 to 24 after conception. It is argued that the possible association of thalidomide embryopathy with autism may shed some light on the issue of which neural circuitries may be involved in autism pathogenesis.

Dyslexia in Children and Young Adults: Three Independent Neuropsychological Syndromes

Abstract: UMMARY In an attempt to delineate causal factors in dyslexia, 113 children and young adults (age-range eight to 18 years) were divided into three groups: those with brain damage who could read (n = 31), those with brain damage who were dyslexic (n = 53), and those without brain damage who were dyslexic (n = 29). A battery of neuropsychological tests was presented to each participant. No significant differences were found between the two dyslexic groups. Three syndromes -language disorder, articulation and graphomotor dysco-ordination, and visuo-perceptual disorder—were found among the great majority of those with dyslexia. The results support a model of dyslexia as being caused by multiple independent defects in higher cortical functioning, as opposed to the theory of a single causal defect. A clinical description of each syndrome is given and models of dyslexia are discussed. The authors stress the desirability of including brain-damaged readers as a control group in any future study on causal factors in dyslexia. RESUME Dyslexie chez les enfants et les adolescents: trois syndromes neuropsychologiques independants Dans le but de determiner les facteurs en cause dans la dyslexie, 113 enfants et adolescents (ages de 8 a 18 ans) ont ete divises en trois groupes: ceux qui pouvaient lire malgre une lesion cerebrale (n = 31), ceux qui presentaient une lesion cerebrale et une dyslexie (n = 53) et ceux qui sans lesion cerebrale avaient une dyslexie (n 29). Une batterie de tests neuropsychologiques ont ete proposes a chaque sujet. Aucune difference significative n'a ete trouvee entre les deux groupes dyslexiques. Trois syndromes: troubles du langage, incoordination de l'articulation et de la graphomotricite, et troubles visuo-perceptif ont ete notes chez la grande majorite des dyslexiques. Ces resultats sont en faveur d'une conception de la dyslexie causee par de multiples facteurs independants dans le fonctionnement cortical haut et non d'une conception d'une cause unique. Une description clinique de chaque syndrome est donnee et des modeles de dyslexie sont discutes. Les auteurs insistent sur la necessite d'inclure des lecteurs atteints de troubles cerebraux comme groupe controle dans toute etude ulterieure sur les facteurs en cause dans la dyslexie. ZUSAMMENFASSUNG Leseschwache bei Kindern und Heraimachsenden: drei unabhangige neuropsychologische Syndrome Um die Kausalfaktoren der Dyslexie zu beschreiben, wurden 113 Kinder und Heran-wachsende (Alter 8–18 Jahre) in der Untersuchung in drei Gruppen geteilt: solche mit Hirnschaden, die aber lesen konnten (n = 31), solche mit Hirnschaden und Dyslexie (n = 53) und solche ohne Hirnschaden mit Dyslexie (n = 29). Jeder Versuchsteilnehmer wurde einer Reihe neuropsychologischer Tests unterzogen. Zwischen den leseschwachen Gruppen fand sich keine signifikante Differenz. Bei der grosen Mehrheit derer mit Dyslexie fanden sich drei Symtome—Sprachstorungen, Artikulations- und graphomotorische Dyskoordination und visuell-perzeptive Storungen. Die Ergebnisse unterstutzen eine Modellvorstellung der Dyslexie, die sie durch multiple voneinander unabhangige Defekte hoherer corticaler Funktionen verursacht ansieht, im Gegensatz zu der Theorie eines einzelnen kausalen Defekts. Es wird eine klinische Beschreibung eines jeden Syndroms gegeben und Modelle der Dyslexie werden diskutiert. Die Autoren betonen, das es wunschenswert ist bei jeder weiteren Studie uber Kausalfaktoren der Dyslexie Hirngeschadigte, die lesen konnen, als eine Kontrollgruppe mit einzubeziehen. RESUMEN Dislexia en ninos y jovenes adultos: Tres sindromes neuropsicologicos independientes En un intento de delinear los factores causales en la dislexia, 113 ninos y adultos jovenes (de edad entre 8 y 18 anos) fueron divididos en tres grupos: Unos con lesion cerebral y que podian leer (n = 31), otros con lesion cerebral pero que eran dislexicos (n = 53) y otros sin lesion cerebral y que eran dislexicos (n = 29). A cada participante se le presento una bateria de tests neuropsicologicos. No se hallo ninguna diferencia significativa entre los dos grupos dislexicos. Tres sindromes (alteracion en el lenguaje, discoordinacion de la articulacion y grafomotora y alteracion visuo-perceptiva) fueron hallados en la gran mayoria de los que padecian dislexia. Los resultados apoyan la idea de que la dislexia esta causada por multiples defectos independientes de las funciones corticales superiores, en oposicion a la teoria de un unico defecto causal. Se da una descripcion clinica de cada sindrome y se discuten los modelos de dislexia. Los autores subrayan el deseo de incluir a los lectores con lesion cerebral como un grupo control en estudios futuros que se hagan sobre los factores causales de la dislexia.

Developmental dyscalculia: prevalence and demographic features

Abstract: UMMARY One hundred and forty-three 11-year-old children with developmental dyscalculia, from a cohort of 3029 students, were studied to determine demographic features and prevalence of this primary cognitive disorder. They were evaluated for gender, IQ, linguistic and perceptual skills, symptoms of attention-deficit hyperactivity disorder (ADHD), socio-economic status and associated learning disabilities. The IQs of the 140 children (75 girls and 65 boys) retained in the study group (three were excluded because of low IQs) ranged from 80 to 129 (mean 98.2, SD 9.9). 26 per cent of the children had symptoms of ADHD, and 17 per cent had dyslexia. Their socio-economic status was significantly lower than that of the rest of the cohort, and 42 per cent had first-degree relatives with learning disabilities. The prevalence of dyscalculia in the original cohort was 6.5 per cent, similar to that of dyslexia and ADHD. However, unlike these other learning disabilities, dyscalculia affected the two sexes in about the same proportions. RESUME Dyscalculie de developpement: prevalence et caracteristiques demographiques Cent quarante-trois enfants de 11 ans issus d'un groupe de 3029 ecoliers et presentant une dyscalculie de developpement on eteetudies pour determiner les caracteristiques demographiques et la prevalence de ce trouble cognitif primaire. Ont ete releves, le sexe, le Q.L, les capacites perceptives ct linguistiques, les symptomes du trouble d'instabilite/hyperactivite (ADHD), le statut socio-economique et les troubles d'apprentissage associes. Trois cas furent exclus du groupe pour Q.L trop bas et les Q.L des 140 ecoliers rcstants allaient de 80 a 129 avec une moyenne de 98,2 et un ecart type de 9,9. Des symptomes d'ADHD etaient retrouves chez 26 pour cent des enfants et une dyslexie chez 17 pour cent. Lcur statut-socio-economique etait significativement plus bas que celui du groupe de reference et pour 42 pour cent, il etait note des difficultes d'apprentissage dans la famille proche. La prevalence de la dyscalculie dans le groupe initial etait de 6,5 pour cent, semblable a celui de la dyslexie et de l'ADHD. Mais en contraste avec ces deux derniers troubles, la prevalance etait identique dans les deux sexes. ZUSAMMENFASSUNG Entwicklungsbedingte Dyskalkulic: Haufigkeit und demographische Merkmale Aus einer Gruppe von 3029 Schulern wurden 143 Kinder im Alter von 11 Jahren mit Dyskalkulie untersucht, um demographische Merkmale und Haufigkeit dieser primaren kognitiven Stoning festzustellen. Die Kinder wurden hinsichtlich Geschlecht, IQ, sprachlicher und pcrzeptiver Fahigkeiten, Symptomen fur attention-deficit hyperactivity disorder (ADHD), sozio-okonomischem Status und Lcrnschwache beurteilt. Die lQ-Werte der 140 Kinder (75 Madchen und 65 Jungen), die in der Studiengruppe verblieben (drei waren wegen niedriger IQ-Werte ausgeschlossen worden) betrugen zwischen 80 und 129 (im Mittel 98.2, SD 9.9). 26 Prozent der Kinder hatten Symptome eines ADHD und 17 Prozent hatte eine Dyslexie. Ihr sozio-okonomischer Status war signifikant niedriger als der ubrigen Gruppe und 42 Prozent hatten Verwandte ersten Grades mit Lemschwache. Die Haufigkeit der Dyskalkulie in der Indexgruppe betrug 6.5 Prozent, ahnlich wie“bei der Dyslexie und ADHD. Im Gegensatz zu dicsen anderen Lemproblemen betraf die Dyskalkulie jodoch beide Geschlechter in etwa gleichem Mase. RESUMEN Discalculia de desanollo: prevalencia y caracteres demograficos Ciento cuarenta y tres ninos de 11 anos de edad con discalculia de desarrollo, de una cohorte de 3029 estudiantes, fueron estudados para determinar los caracteres demograficos y la prevalencia de esta alteracion primaria del conocimiento. Fueron evaluados segun el sexo, CI, habilidades linguistica y perceptiva. sintomas de deficit de atencion e hiperactividad (DAH), estatus socioeconomic y alteraciones del aprendizaje anadidas. El CI de los 140 ninos (75 hembras y 65 varones) que permanecieron en el grupo estudiado (tres fueron excluidos por un CI demasiado bajo) oscilaba entre 80 y 129 (promedio 98.2, SD 9.9). El 26 por ciento de los ninos tenian sintomas de DAH y el 17 por ciento tenian dislexia. Su estatus socioeconomico era significativamente mas majo que el resto de la cohorte y el 42 por ciento tenian parientes de primer grado con alteraciones del aprendizaje. La prevalencia de discalculia en la cohorte original aprendizaje. La prevalencia de discalculia en la cohorte original fue del 6.5 por ciento, similar al de dislexia y de DAH. Sin embargo, al contrario de otras deficiencias de aprendizaje, al discalculia afectaba a ambos sexos en proporcion similar.

Genetic-basis of specific language impairment - evidence from a twin study

Abstract: SUMMARY Concordance rates were compared for 63 monozygotic (MZ) and 27 dizygotic (DZ) same-sex twin pairs, aged seven years and over, selected because at least one twin met diagnostic criteria for specific speech or language impairment. There was significant heritability for developmental speech and language disorder, defined according to DSM-III-R criteria. When the definition of the phenotype was broadened to include those with a past history of disorder and those with a less pronounced discrepancy between verbal and non-verbal ability, concordance for MZ twins was close to 100 per cent, and that for DZ twins approximately 50 per cent. There was also close similarity between concordant twins for type of disorder. There is good evidence that genetic factors play a role in the aetiology of speech and language impairment; twin data may help us arrive at a clearer conception of the phenotype as well as quantifying the extent of the genetic contribution. RESUME Bases genetiques des troubles specifiques de langage: evidence a partir d'une etude de jumeaux Les taux de concordance ont ete comparees entre 63 paires de jumeaux monozygotes et 27 paires dizygotes de meme sexe, âges de sept ans ou plus et selectionnes du fait que l'un des jumeaux au moins remplissait les criteres diagnostiques pour un trouble specifique de parole ou de langage. II y avail une heritabilite significative pour les troubles developpementaux de parole et de langage, scion les criteres du DSM-III-R. Quand la definition du phenotype etait etendue au cas de troubles passes et a un ecart moins prononce entre capacityes verbales et non verbalcs, la concordance pour les jumeaux monozygotes etait proche de 100 pour cent et elle etait d'environ 50 pour cent pour les jumeaux dizygotes. 11 y avait egalement une correspondance etroite entre jumeaux pour le type de troubles. 11 y a done de bonnes raisons pour penser que les facteurs genetiques jouent un role dans l'etiologie des troubles de parole et de langage; ces donnees de jumeaux peuvent nous aider a avoir une conception plus claire du phenotype, aussi bien que des aspects quantifyes de la contribution genetique. ZUSAMMENFASSUNG Genetische Grundlage der spezifischen Sprachstorung: Befunde aus einer Zwillingsstudie Es wurden die Konkordanzraten von 63 monozygoten (MZ) und 27 dizygoten, gleichgeschlechtlichen (DZ) Zwillingspaarcn im Alter von sieben Jahren oder daruber verglichen, die ausgewahlt worden waren, weil mindestens ein Zwillingskind die diagnostischen Kriterien fur eine spezifische Sprach-oder Sprachverstandnisstorung erfullte. Es bestand eine signifikante Erblichkeit fur die Entwicklung von Sprach-und Sprachverstandnisstorungen, definiert nach den DSM-III-R Kriterien. Erweiterte man die Definition des Phanotyps und nahm die mit einer fruheren Storung in der Anamnese und die mit einer weniger ausgepragten Diskrepanz zwischen verbalen und non-verbalen Fahigkeiten hinzu, war die Konkordanz fur MZ Zwillinge nahezu 100 Prozent und die fur DZ Zwillinge ungefahr 50 Prozent. Es besteht auch eine weitgehende Ubereinstimmung zwischen den konkordanten Zwillingen fur die Art der Storung. Es gibt Hinweise, das genetische Faktoren fur die Atiologie der Sprach- und Sprachverstandnisstorungen eine Rolle spielen; Zwillingsdaten konnen dazu beitragen, eine klarere Vorstellung vom Phanotyp zu bekommen, sowie den genetischen Anteil zu quantifizieren. RESUMEN Base genetica de la alteracion especifica del lenguaje: evidencia a partir de un estudio en gemelos Se compareo la concordancia de porcentajes comparando 63 mellizos monozigotos (MZ) y 27 dizigotos (DZ) del mismo sexo, de siete o mas anos de edad, seleccionados porque, por lo menos uno de la pareja de mellizos, cumplia los criterios diagnosticos de una alteracion especifica del habla o del lenguaje. Habia una herencia significativa para la alteracion en el desarrollo del habla o del lenguaje, de acuerdo con los criterios de DSM-III-R. Cuando la definition del fenotipo se ampliaba para incluir los que tenian una historia anterior y los que tenian una discrepancia menos pronunciada entre la capacidad verbal y la no verbal, la concordancia en los mellizos MZ era de cerca del 100 por ciento y en DZ aproximadamente del 50 por ciento. Tambien habia una marcada similitud entre los mellizos concordantes scgun el tipo de alteracion. Hay una buena evidencia dc que los factores geneticos juegan un papel importante en la etiologia de la alteracion en el habla y cl lenguaje. Los datos a partir de gemelos nos pueden ayudar a obtener un concepto mas elaro del fenotipo y cuantificar el grado de la contributeon genetica.

Thalidomide and congenital abnormalities.

Abstract: THALIDOMIDE* was first synthesised in Germany in 1954, and after extensive trials was made available there without prescription in 1957. In the United Kingdom it has been generally available on prescription since 1959. At the end of 1961, L E N Z ~ and PFEIFFER and KOSENOW~O called attention to an association between thalidomide and gross limb and other deformities in newborn babies. Since then there have been similar reports from Australia,8 Scotland,14 England,12 Ireland,3 Canada,9 East Germany, Switzerland and Sweden,' but there have so far been no reports from the United States. About 100 cases are already known in England, Scotland and Northern Ireland. The sex-incidence is about equal.1° The typical changes (see Figure) involve the limbs (amelia, phocomelia, hemimelia). However, the lesions may be widespread and include rudimentary scapulae and maldevelopment of the sacrum, stenosis, atresia and malrotation of the gut, imperforate anus, absence or aplasia of the appendix or gall-bladder, abnormal mobility of the caecum or ascending colon, choanal atresia, haemangioma of the lip and nose, dysplasia of ears and eyes, hydronephrosis, genitourinary anomalies, and congenital heart disease. LENZ' estimates the incidence of heart lesions in affected cases as about 10 per cent. An unexplained feature observed in two infants has been excessive sweating of the head and face.5* l1 Serum and sweat electrolytes were normal in one of these infant^.^ The chromosomes are probably normal. 43 lo

Attention deficits and clumsiness in Swedish 7-year-old children

Abstract: A population study of 409 seven-year-old children in a middle-sized Swedish town was performed. All children were examined by the same doctor and evaluated by means of parent interview, motor examinations, and teacher reports on behaviour in the classroom. Follow-up was carried out 8 months later. The rate of severe problems in the fields of attention deficit-hyperactivity disorder (ADHD), developmental coordination disorder (DCD), and deficits in attention, motor control, and perception (DAMP) (the combination of ADHD and DCD) was 6.1%, with boys being affected more frequently than girls. There was considerable overlap between ADHD and DCD, with about half of each diagnostic group also meeting criteria for the other diagnosis. Attention deficits at diagnosis strongly predicted attention deficits at follow-up. If parents had noted attention deficits in the home setting, then teachers almost always independently agreed that there were similar problems in the classroom. However, the reverse did not always apply. Clumsiness also showed striking stability over time. The diagnosis of DAMP, particularly severe DAMP, had a stronger association with classroom dysfunction and with high Conners scores than did diagnoses of ADHD or DCD. It is concluded that DAMP may be a clinically valid diagnostic construct.

Assessment of visual acuity in infants and children: the acuity card procedure.

Abstract: The 'acuity card' procedure described here is a simplified method of testing visual acuity of infants and young children, and has been developed to allow preferential looking to be assessed in a laboratory or clinic. A higher proportion of children can be tested successfully than has been reported for more traditional procedures. Initial studies indicate that the acuity card procedure is a fast, accurate method for assessing the acuity of normal infants and children, and those with visual or neurological impairments, across a wide age-range and in both clinical and laboratory settings.

Botulinum toxin in the management of the lower limb in cerebral palsy

Abstract: The role of intramuscular botulinum toxin A in the treatment of 26 children with cerebral palsy was evaluated. The indication for injection was the presence of a dynamic contracture of lower-limb muscles interfering with positioning or walking. Spastic target muscles were identified by clinical examination and, in ambulant children, by gait analysis. Between 50 and 320 units of botulinum toxin were injected into each muscle group to a total dose of 100 to 400 units per child. The effects of injection were monitored by repeated clinical examination and gait analysis. There were no clinically detectable systemic side-effects, and all but one patient had a reduction in tone, which occurred within three days and persisted for two to four months. There were significant improvements in ambulatory status and in sagittal-plane kinematics. In some cases these gains persisted after the tone-reducing effects of the toxin had worn off.

Results of treatment of myelomeningocele. An analysis of 524 unselected cases, with special reference to possible selection for treatment.

Abstract: UMMARY It has been shown that, in the absence of treatment, most infants born with myelomeningocele die early in infancy, and it is inferred that almost all the more severe cases do so. Massive efforts in treatment have led to a considerably increased survival rate, but the large majority of survivors have major physical defects and are often mentally retarded. Two unselected series of cases are analysed here from a personal experience of more than 1200 cases treated: the first a series of 323 infants treated between 1959 and 1963; the second a series of 201 infants treated during 1967-68. In all cases, treatment was started on the first day of life. In the first series, 50 per cent survived to 2 years, compared with 64 per cent in the second series. The proportions with serious intellectual or physical handicaps did not decrease in the second series. The importance of many other factors is analysed, particularly those of the associated hydrocephalus and its severity, as well as the degree of muscle paralysis. The infants who fared particularly badly were those with extensive paralysis at birth, those with a head circumference exceeding the 90th percentile by 2 cm or more, and those born with a gross kyphosis or with major associated congenital defects. Objective data are presented to suggest that selection for treatment can be made on a humanitarian basis. RESUME Resultats du traitement de myelomeningocele Il a ete demontre qu'en l'absence de traitement la plupart des enfants nes avec myelomeningocele meurent tot dans l'enfance et on en deduit que presque tous les cas severes evoluent ainsi. Des efforts massifs de traitement ont permis une elevation considerable du taux de survie, mais la grande majorite des survivants presentent des handicaps physiques majeurs et sont souvent des retardes mentaux. Deux series non selectionnees de cas sont analyses ici a partir d'une experience personnels de plus de 1200 cas traites: la premiere serie concerne 323 enfants traites entre 1959 et 1963; la seconde serie 201 enfants traites en 1967–1968. Dans tons les cas le traitement a debute au premier jour de la vie. II y a 50 pour cent de survie a deux ans dans la premiere serie et 64 pour cent dans la seconde. La proportion de handicaps severes, intellectuels ou physiques, n'est pas plus faible dans la seconde serie. L'importance de nombreux autres facteurs a ete analysee particulierement l'association d'une hydrocephalic et sa gravite, et le degre de paralysie musculaire. Les enfants ayant evolue le plus mal sont ceux qui presentaient une paralysie tres etendue a la naissance, ceux dont le tour de tete excedait le quatrieme percentile de 2 centimetres ou plus et ceux nes avec une cyphose importante ou avec des alterations congenitales associees graves. L'auteur presente des donnees objectives suggerant une selection pour le traitement, etablie sur une base humanitaire. ZUSAMMENFASSUNG Ergebnisse in der Behandlung der Myeolomeningocele Es ist gezeigt worden, das ohne Behandlung fast alle Kinder mit angeborener Myelo-meningocele fruh sterben, und es wird geschlossen, das fast alle schweren Falle so verlaufen. Grose Anstrengungen in der Behandlung haben zu einerbe betrachtlich hoheren Uberlebensrate gefuhrt, aber die grose Mehrzahl der Uberlebenden leidet unter ernsten physischen Defekten und ist oft geistig zuruckgeblieben. Zwei unausgewahlte Reihen von Fallen wurden hier aus einer personlichen Erfahrung von mehr als 1200 behandelten Fallen analysiert: die erste Serie von 323 Kindern, behandelt zwischen 1959 und 1963; die zweite Serie von 201 Kindern behandelt 1967 und 1968. In alien Fallen wurde die Behandlung am ersten Lebenstage begonnen. In der ersten Serie uberlebten 50 prozent bis zu zwei Jahren, verglichen mit 64 prozent in der zweiten Serie. Der Anteil schwerer intellektueller und physischer Storungen nahm in der zweiten Serie nicht ab. Die Bedeutung vieler anderer Faktoren wird untersucht, vor allem die eines gleichzeitig bestehenden Hydrocephalus und dessen Schweregrades, sowie auch das Ausmass der Muskelparalyse. Die Kinder, denen es besonders schlecht ging, hatten bei der Geburt ausgepragte Lahmungen, einen Schadelumfang von 2 cm oder mehr uber 90 prozent, oder eine schwere Kyphose oder andere schwere angeborene Defekte. Objektive Ergebnisse werden dargelegt, die eine Auswahl zur Behandlung auf humanitarer Basis nahelegen. RESUMEN Resultados en el tratamiento del mielomeningocele Se ha puesto en evidencia que en ausencia del tratamiento, la mayoria de los ninos nacidos con mielomeningocele mueren precozmente en el periodo de lactancia, infiriendose que asi lo hacen la mayoria de los casos graves. Los esfuerzos masivos en el tratamiento han conducido a un aumento considerable del porcentaje de sobrevivencias, pero la gran mayoria de los supervivientes tienen mayores defectos fisicos y con frecuencia son retrasados mentales. Se analizan 2 series no seleccionadas de casos a partir de una experiencia personal de mas de 1.200 casos tratados: la primera serie consta de 323 lactantes tratados entre 1959 y 1963 y la segunda de 201 ninos tratados durante 1967-68. En todos los casos el tratamiento se comenzo en el primer dia de la vida. El primer grupo el 50 por ciento sobrevivio hasta los 2 anos, en comparacion con el 64 por ciento del segundo grupo. La proportion de casos con defectos intelectuales o fisicos graves no disminuyo en el segundo grupo. Se analiza la importancia de muchos otros factores, particularmente la hidrocefalia asociada y su gravedad, asi como el grado de paralisis muscular. Los ninos que siguieron un curso especialmente malo eran los que presentaban extensa parailisis a1 nacer, los que tenian un perimetro cefalico superior a1 percentil 90 en 2 cm o mas, y los nacidos con una grave cifosis o con otros defectos congenitos graves. Se presentan datos objetivos para sugerir que la seleccion para el tratamiento pueda realizarse sobre una base humanitaria

Development of co-ordination of sucking, swallowing and breathing: ultrasound study of term and preterm infants.

Abstract: Fourteen newborn babies of different gestational ages (33 to 40 weeks) but similar postnatal age (four to 19 days) were studied during bottle-feeding using real-time ultrasonography, combined with respiratory monitoring. Previously undescribed tongue movements and graded changes in the temporal relationships between tongue movements, swallowing and breathing were observed among infants of differing maturity. These were most marked in the least mature infants, but were occasionally seen in term infants. The results suggest that adequate neuromuscular co-ordination is more a function of gestational maturity than of postnatal sucking experience. The pattern of intraoral events for infants of differing maturity described in this study provides a framework for the study of feeding problems of term and preterm infants.

Participation and enjoyment of leisure activities in school-aged children with cerebral palsy

Abstract: The objective of this study was to characterize participation in leisure activities in children with cerebral palsy (CP) and identify determinants of greater involvement. Ninety-five children of school age (9y 7mo [SD 2y 1mo]) with CP were recruited, and participation was evaluated with the Children’s Assessment of Participation and Enjoyment in a subset (67/95; 42 males, 25 females) who could actively participate in completion of the assessment. Most had mild motor dysfunction (Gross Motor Function Classification System: 59% level I, 23% level II, 18% levels III–V) and had a spastic subtype of CP (23 hemiplegia, 17 diplegia, 16 quadriplegia, 11 other). Biomedical, child, family and environmental predictor variables were considered in the analysis. Results demonstrated that these children were actively involved in a wide range of leisure activities and experienced a high level of enjoyment. However, involvement was lower in skill-based and active physical activities as well as community-based activities. Mastery motivation and involvement in rehabilitation services enhanced involvement (intensity and diversity) in particular leisure activities, whereas cognitive and behavioral difficulties, activity limitations, and parental stress were obstacles to participation.

A Longitudinal Study Of Children With Developmental Language Delay At Age Three: Later Intelligence, Reading And Behaviour Problems

Abstract: A large sample of Dunedin (New Zealand) children were assessed at age three to identify those with language delay. 2.6 per cent were defined as delayed in verbal comprehension only, 2.3 per cent as delayed in verbal expression only, and 2.3 per cent as delayed in both ('general language delay'). Most of these children, and the remainder of the sample, were assessed for intelligence, reading and behaviour problems at ages seven, nine and 11. Those in every language-delay group had significantly lower mean IQs and lower mean reading scores than the remainder of the sample. They also more often had a low IQ or a lower reading score at ages seven and nine and a lower Verbal and Full-scale IQ at 11. The groups with delayed verbal comprehension and general language delay had significantly more behaviour problems than the remainder of the sample. The group with general language delay was consistently the most disadvantaged in later intelligence, reading and behaviour. Two of the language-delay groups (comprehension and general language) had significantly higher scores on a family disadvantage index. The results of this study confirm the importance of early language delay as a predictor of lower than average intelligence and reading ability and increased behaviour problems.

Asperger syndrome in 23 swedish children

Abstract: UMMARY Twenty-three Swedish children aged five to 18 years who fulfilled specific criteria for Asperger syndrome were examined and compared with an age- and IQ-matched group with infantile autism. The boy:girl ratio was 10:1. Less than 10 per cent were mentally retarded and 17 per cent were of above-average intelligence. Apart from motor clumsiness (very common in the Asperger group) and reduced optimality in the prenatal and perinatal periods (more common in the autistic group), there was very little in the clinical or neurobiological backgrounds to suggest a clear distinction between Asperger syndrome and infantile autism. RESUME Syndrome d'Asperger chez 23 jeunes suedois: etude clinique Vingt trois jeunes suedois ages de cinq a 18 ans et presentant les signes caracteristiques du syndrome d'Asperger ont ete examines et compared avec un groupe d'autisme infantile, apparte pour l'âge et le QI. Le ratio garcon/fille etait de 10:1. Moins de 10 pour cent etalent mentalement retardes et 17 pour cent etaient d'intelligence supeieure a la moyenne. En dehors de la maladresse motrice (tres habituelle dans le groupe Asperger) et de l'optimalite reduite pour la periode prenatale et perinatale (plus frequente dans le groupe autistique), il y avait tres peu de manifestations cliniques ou neurobiologiques pouvant suggeier une nette distinction entre syndrome d'Aspeger et autisme infantile. ZUSAMMENFASSUNG Asperger Syndrom bei 23 schwedischen Kindern: eine klinische Studie 23 schwedische Kinder im Alter von funf bis 18 Jahren, die bestimmte Kriterien fur das Asperger Syndrom erfullten, wurden untersucht und mit einer Gruppe von Kindern mit kindlichem Autismus mit entsprechendem Alter und IQ verglichen. Das Verhaltnis Jungen zu Madchen betrug 10:1. Weniger als 10 Prozent waren geistig retardiert und 17 waren uberdurchschnittlich intelligent. Neben motorischer Unbeholfenheit (sehr haufig beim Asperger Syndrom) und verminderter Optimalitat in der Pra- und Perinatalperiode (haufiger in der autistischen Gruppe) fand sich klinisch und neurobiologisch wenig, was eine klare Unterscheidung zwischen dem Asperger Syndrom und dem kindlichen Autismus darstellt. RESUMEN Sindrome de Asperger en 23 ninos suecos: estudio clinico Veintitres ninos suecos de cinco a 18 aflos de edad, que cumplian los criterios especificos del sindrome de Asperger, fueron examinados y comparados con un grupe semejante de ninos con autismo. La relacion nino/nifla era de 10:1. Menos del 10 por ciento eran retrasados mentales y 17 por ciento tenian una inteligencia por encima de la media. Aparte de una torpeza motora (mas corriente en el grupo Asperger) y una optimalidad reducida en lose periodos pre y perinatal (mas corriente en el grupo autfstico), habia muy pocos caracteres elfnicos y neurobiologicos que pudiesen sugeir una distincion clara entre el sindrome de Asperger y el autismo infantil.

Chronology of Neuron Development: Animal Studies and their Clinical Implications

Abstract: Because different parts of the central nervous system form at different stages of development, there is not one critical period but many critical periods. Some neurons are formed around the time of closure of the neural tube: these include the motor horn cells of the spinal cord and some motor nuclei of the brain stem. Other neurons, most notably the granule cells of the cerebellum, olfactory bulb and hippocampus, are produced in great numbers after birth. This review focuses on the mouse, the species for which the most data on neurogenesis are available, and draws parallels with other species. The clinical significance of the chronology of neuron formation is discussed in the context of recent studies of experimentally-induced congenital brain damage.

Asperger's syndrome and autism: comparison of early history and outcome

Abstract: UMMARY The authors compared children with Asperger syndrome (AS) with high-functioning autistic children and psychiatric outpatient controls on measures of early history and outcome. In terms of their early history, the autistic probands showed more social impairment, a higher frequency of echolalia and pronoun reversal, and a more restricted range of activities than the AS group. Cluster analysis suggested refinements to the diagnostic criteria, which resulted in larger differences between the groups on these early history measures. In terms of their outcome, the autistic probands spent more time in special education classes but developed fewer accessory psychiatric symptoms than the AS children. It was clear, however, that there were no substantive, qualitative differences between the AS and autistic groups, indicating that AS should be considered a mild form of high-functioning autism. The inclusion of AS among the autistic spectrum of disorders has implications both for aetiological studies and for prevalence estimates of the pervasive developmental disorders. RESUME Syndrome d'Asperger et autisme: comparaison sur l'histoire precoce et le devenir Les auteurs comparent des enfants presentant un syndrome d'Asperger (AS) avec des enfants a autisme avec riche symptomatologie et des controles consultants externes en psychiatrie, sur les caracteristiques de I'histoire precoce et le devenir. En terme d'histoire precoce, les sujets autistiques presentaient plus de troubles de la relation sociale, une plus grande frequence d'echolalie et de reversion des pronoms, et une etendue d'activites plus reduites que le groupe AS. L'analyse de groupe suggerait des precisions dans les criteres diagnostiques se traduisant par des plus grandes differences entre les groupes sur ces caracteristiques d'histoire precoce. En terme de devenir les sujets autistiques passaient plus de temps dans les classes d'education specialie mais developpaient moins de symptomes psychiatriques accessoires que les enfants AS. Il etait cependant claire qu'il n'y avait pas de difference qualitative substentielle entre les groupes AS et autistiques indiquant que l'AS pourrait etre considered comme une forme mineure de l'autisme a riche symptomatologie. L'inclusion de l'AS dans le spectre autistique des troubles a des implications a la fois pour des etudes etiologiques et pour les estimations de prevalence des troubles developpementaux envahissants. ZUSAMMENFASSUNG Asperger Syndrom und Autismus: Vergleich von Fruhanamnese und Outcome Die Autoren vergleichen Kinder mit Asperger Syndrom (AS) mit hochbegabten autistischen Kindern und psychiatrischen ambulanten Kontrollpatienten hinsichtlich Fruhanamnese und Outcome. In ihrer Fruhanamnese hatten die autistischen Kinder starkere Storungen im sozialen Bereich, ein haufigeres Auftreten von Echolalie und Pronomenumkehrung und einen eingeschrankteren Aktivatatsradius als die AS Gruppe. Die Gruppenanalyse empfahl eine Verfeinerung der diagnostischen Kriterien, die bei diesen fruhananamnestischen Parametern zu goseren Unterschieden zwischen den Gruppen fuhrte. Hinsichtlich ihres Outcome waren die autistischen Kinder langere Zeit in Sonderklassen, aber sie entwickelten weniger psychiatrische Begleitsymptome als die AS Kinder. Es war jedoch klar, das es erhebliche qualitative Unterschiede zwischen den AS Kindern und den autistischen Kindern gab, so das das AS als milde Form des Autismus mit hoher Begabung angesehen werden sollte. Die Einbeziehung des AS in das Spektrum der autistischen Erkrankungen ist sowohl fur die athiologischen Untersuchungen als auch fur die Pravalenzbestimmungen der pervasiven Entwicklungserkrankungen von Bedeutung. RESUMEN Sindrome de Asperger y autismo: comparaciones en la historia precoz y la evolucion Los autores compararon ninos con el sindrome de Asperger (AS), con ninos autisticos de alto funcionamiento y controles psiquiatricos de dispensario por lo que hace a la historia precoz y el curso evolutivo. En termino de su historia precoz los autisticos probandos mostraron una alteracion social mayor, una mayor frecuencia de ecolalia y de reversion pronominal y un margen de actividades mas restringido que el grupo AS. Un analisis mas proximo sugirio unos refinamientos en los criterios diagnosticos, lo cual dio lugar a mayores diferencias en los dos grupos en sus historias precoces. En terminos de su evolucion, los autistas probandos pasaron mas tiempo en clases de education especial, pero desarrollaron menos sintomas psiquiatricos accesorios que los ninos AS. Sin embargo quedaba claro que no habia diferencias substantivas ni cualitativas entre los grupos AS y los autisticos, lo que indica que AS debe ser considerado como una forma moderada del autismo altamente funcionante. La inclusion del AS en el espectro de los autistas tiene implicaciones tanto para los estudios etiologicos, como por la prevalencia de estimacion de las alteracions pervasivas del desarrollo.

A systematic review of the clinimetric properties of neuromotor assessments for preterm infants during the first year of life

Abstract: This systematic review evaluates assessments used to discriminate, predict, or evaluate the motor development of preterm infants during the first year of life. Eighteen assessments were identified; nine met the inclusion criteria. The Alberta Infant Motor Scale (AIMS), Bayley Scale of Infant and Toddler Development -- Version III, Peabody Developmental Motor Scales -- Version 2, Test of Infant Motor Performance (TIMP), and Toddler and Infant Motor Examination have good discriminative validity when examined in large populations. The AIMS, Prechtl's Assessment of General Movements (GMs), Neuro Sensory Motor Development Assessment (NSMDA), and TIMP were designed for preterm infants and are able to detect more subtle changes in movement quality. The best predictive assessment tools are age dependent: GMs, the Movement Assessment of Infants, and TIMP are strongest in early infancy (age 4 mo or less) and the AIMS and NSMDA are better at older ages (8-12 mo). The TIMP is the only tool that has demonstrated a difference between groups in response to intervention in two randomized controlled trials. The AIMS, TIMP, and GMs demonstrated the highest levels of overall reliability (interrater and intrarater intraclass correlation coefficient or kappa>0.85). Selection of motor assessment tools during the first year of life for infants born preterm will depend on the intended purpose of their use for discrimination, prediction, and/or evaluation.

Cognitive and educational outcome of very‐low‐birthweight children in early adolescence

Abstract: A cohort of 138 very-low-birthweight (VLBW) 12-year-old children and matched control children were assessed on objective cognitive and educational measures. School performance was rated by teachers and by the children themselves. VLBW children were shown to have lower IQ scores, and poorer scores on all objective educational measures compared with control children. Controlling for IQ differences, mathematics and reading-comprehension scores remained significantly lower for VLBW children. Teachers rated VLBW children lower in all curriculum areas. Significantly more VLBW children were found to be 'failing' in one or more subject and an increased proportion compared with the control children had received remedial education. The VLBW group showed no evidence of 'catch up' between 6 and 12 years of age. Multiple regression analyses were used to identify predictors of cognitive and educational outcome. The duration of mechanical ventilation in the neonatal period was inversely related to outcome. Full-Scale IQ at 6 years, motor-skills score at 6 years, and head circumference at 12 years all predicted outcome at 12 years, as did maternal education, family income and size. Individually, many VLBW children perform satisfactorily, but as a group VLBW children appear to be at a long-term disadvantage to peers in the areas of cognitive and educational performance.

Term infants with hypoxic-ischemic encephalopathy: outcome at 3.5 years

Abstract: A total of 167 term neonates with a diagnosis of hypoxic-ischemic encephalopathy (HIE) had detailed neurodevelopmental follow-up at 3.5 years of age. All 66 children with mild HIE were free from handicap; all seven with severe HIE were severely handicapped; and of the 94 with moderate HIE at birth, 21.3 per cent were handicapped. Mean IQ was significantly related to the category of HIE. Within the moderate HIE category, the neurological examination at discharge from the Neonatal Intensive Care Unit was more useful than the presence of neonatal convulsions in identifying children with subsequent developmental delay. Abnormalities on this examination related significantly to an increased number of handicapped children, decreased motor and language skills, and lower IQs. Although neonatal convulsions were associated with an increased number of handicapped children, they did not significantly affect most other developmental outcome measures. In term infants with documented HIE at birth, major neurodevelopmental dysfunction at 3.5 years depended more on prospectively established category of HIE than on other perinatal or social factors.

Fetal valproate syndrome: clinical and neuro-developmental features in two sibling pairs.

Abstract: The clinical and neurodevelopmental features are presented of four children--two sibling pairs--who were exposed in utero to valproic acid. One of each pair of children presented for diagnosis and assessment of developmental delay; the other sibling was examined at a later date. Three of the children were globally developmentally delayed with marked speech disability, and had dysmorphic features consistent with fetal valproate syndrome. One also had features of infantile autism. The fourth child had some of the dysmorphic features connected with fetal valproate syndrome, but had normal intellect, with his verbal ability being significantly below his non-verbal ability. He currently attends a school for learning-disabled children.