bluuebunny/crossref_metadata_2025 huggingface dataset:
Infants' individuation of agents and inert objects
Authors: Surian Luca , Caldi Stefania
Published: June 2009

Using the violation‐of‐expectancy method, we investigated 10‐month‐old infants’ ability to rely on dynamic features in object individuation processes. Infants were first familiarized to events in which two different objects repeatedly appeared and disappeared, one at a time from behind a screen; at test, the screen was removed, revealing either one or two objects. In Experiment 1, one self‐moving non‐rigid agent and one inert object were involved in each trial, while in Experiment 2 two different agents were presented. Infants preferred to look at one‐object outcomes in Experiment 1, but they did not show any preference for one‐ or two‐object outcomes in Experiment 2. The results suggest that infants can use dynamic information to detect agents in complex individuation tasks before they can rely on shape or surface features. We propose that the sortals agent and inert object appear in development before 12 months without a substantial contribution of linguistic experience. These findings may motivate a revision of current theories on the development of kind‐based individuation and object files.
Comp‐Teffects: Variation in the position and features of C*
Authors: Lohndal Terje
Published: June 2009

Abstract. That‐trace effects have been studied for over thirty years, and have turned out to be an intriguing phenomenon. This paper discusses a recent proposal regarding howthat‐trace in English should be analyzed, and extends this theory to the Scandinavian languages. However, since the late 1970s it has been known thatthat‐trace effects are but one instance of Comp‐trace effects. The present paper attempts to show how an account ofthat‐trace can be extended to a general Comp‐trace parameter. It is also shown how the present analysis carries over to relative clauses, and the paper broadens the current empirical knowledge of Norwegian relative clauses. The latter has crucial consequences for the analysis of the relative complementizersom, and makes it possible to account for the fact that relative clauses show a “reversed” Comp‐trace effect.
Explaining the immobility of conjuncts*
Authors: Zhang Niina Ning
Published: June 2009

Abstract. This paper aims to explain the well‐observed constraint that no conjunct may move. It is claimed that, with respect to final conjuncts, this constraint simply manifests a morphological property of coordinators shared with many other types of head elements: they need to be adjacent to their complement, and thus neither deletion nor movement of their complement is possible. Final conjuncts therefore may not move. Based on the assumption that the categorial features of initial conjuncts are transferred to the coordinatorand, it is claimed that this transference keeps initial conjuncts in situ, since elements without category‐features may not move overtly. This new account for the immobility of initial conjuncts is supported by two generalizations. Firstly, in the Chinesedeconstruction, kernel elements may not move because they provide categorial features forde, which has no intrinsic categorial features and is the head of the whole complex. Secondly, in the comitative coordinate construction in Chinese, initial conjuncts may move because they do not provide categorial features for the coordinators, which have their own intrinsic categorial features. This paper specifies the semantic condition of initial conjunct movement: the coordination must be non‐distributive. This new account of the immobility of conjuncts suggests that the constraint is not a construction‐specific syntactic constraint. Instead, it is related to the lexical/morphological makeup of coordinators. Conjuncts as regular Spec and Complement elements may undergo syntactic movement. It has been generally assumed that movement is driven by morphological considerations. This study further shows that the blocking of movement can also be related to morphological properties of specific syntactic elements in addition to the generally recognized locality restrictions.
Reference points in linguistic construal: Scalar adjectives revisited*
Authors: Tribushinina Elena
Published: June 2009

Abstract. This paper addresses one of the central assumptions in the analysis of scalar adjectives that their positive form is obligatorily interpreted vis‐à‐vis an average value. By this view,tallandtaller than averageare equivalent expressions. Counter to this well‐established view, I argue that the two constructions are not equivalent; there are important semantic and functional differences between them. First, not all uses of the positive form are interpreted vis‐à‐vis an average value. Second, even in contexts where the averageisrelevant, it is not sufficient by itself: several standards of comparison are involved in the interpretation of scalar adjectives. Third, bare scalars and theAer‐than‐averageconstruction select different scale parts as their profile and are in this sense different construals of the gradual scale.
Who Cares About Corporate Taxation? Asymmetric Tax Effects on Outbound FDI
Authors: Overesch Michael , Wamser Georg
Published: June 2009

This paper investigates whether different types of FDI are asymmetrically affected by corporate taxation. We classify investment projects according to several characteristics such as the general motivation for FDI, the type of business activity, or the degree of internationalisation of the multinational firm. Subsequently, we analyse how local taxes influence the number of German outbound investments in European countries. The analysis reveals significant asymmetries with regard to tax effects: vertically integrated investments are more sensitive to host‐country taxation than horizontal FDI; larger tax rate elasticities are estimated if business activities are considered highly mobile; and in accordance with profit‐shifting considerations, subsidiaries of more internationalised companies are less tax responsive to host‐country taxation.
How Does FDI Affect Domestic Firms’ Exports? Industrial Evidence
Authors: Sun Sizhong
Published: June 2009

Using a Heckman sample selection model estimated using pooled four‐year firm‐level data, this paper explores the export spillovers from the FDI in the cultural, educational and sporting product manufacturing industry of the manufacturing sector in China from 2000 to 2003. The manufacturing sector contributes around 40 per cent of the GDP in the Chinese economy, and the cultural, educational and sporting product manufacturing industry has a significant proportion of FDI activities, and firms in this sector are active in exporting. Through the empirical exercise, we find that there exist export spillovers from FDI in the industry, for which the magnitude depends on firms’ geographical location, sale cost and revenue ratio, and ownership structure. On average, domestic firms located in Western China suffer from a foreign presence, irrespective of whether they are privately owned or state and collectively owned. For firms in Central China, both the privately owned and state and collectively owned firms appear to benefit from foreign presence. Regarding firms located in Coastal China, the privately owned firms suffer from the foreign presence, while in contrast the state and collectively owned firms benefit from the foreign presence. In addition, in this industry there are more firms that benefit from the presence of FDI than those that suffer, which to some extent justifies the government's policy to attract the FDI inflow.
International Migration, the Brain Drain and Poverty: A Cross‐country Analysis
Authors: Cattaneo Cristina
Published: June 2009

The aim of the paper is threefold. First, it empirically investigates the effect of international migration on poverty in origin countries, using data from a cross‐country analysis. Second, it explores the specific part of the income distribution from which migrants are drawn, by comparing the effect of international migration on different income quintiles of the population. Finally, given that the selection of migrants depends not only on the income level of their households, but also on the skill levels of movers, the paper casts some light on the implications of the emigration of skilled workers on poverty. This article shows that the stock of international migrants have a positive effect on the income of the poor while it does not have a significant impact on the income of the middle and top quintiles of the population. In particular, a 10 per cent increase in the per capita stock of migrants resident in OECD nations augments the income of the poor by 1 per cent, on average andceteris paribus. Finally, the poverty‐reducing effect of migration holds even when the emigration process selects the best and the brightest.
Gene duplication and evolutionary novelty in plants
Authors: Flagel Lex E. , Wendel Jonathan F.
Published: June 2009

Duplication is a prominent feature of plant genomic architecture. This has led many researchers to speculate that gene duplication may have played an important role in the evolution of phenotypic novelty within plants. Until recently, however, it was difficult to make this connection. We are now beginning to understand how duplication has contributed to adaptive evolution in plants. In this review we introduce the sources of gene duplication and predictions of the various fates of duplicates. We also highlight several recent and pertinent examples from the literature. These examples demonstrate the importance of the functional characteristics of genes and the source of duplication in influencing evolutionary outcome.
An appreciation of Ronnie Mac Keith (1978)
Authors: Bax Martin
Published: June 2009

It is 100 years since Ronnie Mac Keith's birth and 50 years since he started the Journal Developmental Medicine & Child Neurology (DMCN; initially called The Cerebral Palsy Bulletin), the first number being a reprint of William Little's original article. Scope, then The Spastics Society, had just begun to raise significant sums of money and Ronnie persuaded them not only to put some money into medical research, which they did, funding the research laboratories at Guy's, but also, uniquely, to spend some money on educating and informing doctors. This led to financial backing, happily still continuing, to the publishers of DMCN, now the Mac Keith Press.

Initially, it was published under the title Spastics International Medical Publications but this was a clumsy and difficult title because of the unfortunate use of the word 'spastics’and soon after Ronnie's death, who was then senior editor, the Mac Keith Press Board were delighted that his family agreed that the Press would be named after him.

In the late 1950s and early 1960s, Ronnie was collecting a team around him to develop the Journal and the books, and contacted me because he knew I had literary interests. I didn't really want to edit a medical journal but I was interested in paediatrics so in the end I got involved! I worked very closely with Ronnie, both clinically and at the Mac Keith Press, and also with the Medical Education Information Unit of the Spastics Society on the meetings he ran. When he died, I tried to pull together something of Ronnie's nature in this personal memoir below, which supplemented the more formal statements about his life and career which can be found in the relevant number of the Journal.1 One hopes that Ronnie would be pleased with what we have done and I know that he would be hoping that we would continue for another 50 years developing ideas and approaches which were essentially developed by Ronnie Mac Keith.
Partial body‐weight‐supported treadmill training can improve walking in children with cerebral palsy: a clinical controlled trial
Authors: Dodd Karen J , Foley Sarah
Published: June 2009

This matched‐pairs, clinical controlled trial evaluated the effects of a school‐based, partial body‐weight‐supported treadmill training (PBWSTT) programme, conducted twice a week for 6 weeks, on the walking speed and endurance of children with cerebral palsy (CP). Pairs of children (10 males, four females; mean age 8y 10mo [SD 2y 6mo], range 5–14y) matched for sex, age, type of CP (athetoid quadriplegia, n=6; spastic quadriplegia, n=6; spastic diplegia, n=2), and Gross Motor Function Classification System level (10 at Level I V, four at Level III) were allocated to the experimental or control group. Compared with the control group, the seven treadmill‐training participants increased their self‐selected walking speed over 10 metres (Mann‐Whitney U=9.00, z=−1.98, p=0.048; mean difference 4.21m/min). A trend was also found for increased distance walked over ground in 10 minutes (t[12]=1.88, p=0.083; mean difference 19.81m). A relatively short PBWSTT programme can improve the walking speed of children with CP and moderate to severe disabilities. Walking endurance might also improve in some children. Treadmill training seems to be a useful gait training option for children with C P, and it seems feasible to conduct such a programme within a school environment.
Differentiating prospective control information for catching in at‐risk and control adolescents
Authors: Aanondsen Chris M , Van Der Meer Audrey L H , Brubakk Ann‐Mari , Evensen Kari Anne I , Skranes Jon S , Myhr Gunnar E , Van Der Weel FR (Ruud)
Published: June 2009

This paper investigated the use of prospective control in catching and how the results can be used as a sensitive tool to detect diffuse signs of brain dysfunction. A detailed analysis of 286 catching movements of eight adolescents (two males, six females [four very‐low‐birth weight {VLBW}, one small for gestational age {SGA}, and three appropriate for gestational age]; mean age 14y 5mo [SD 6mo]; range 14–15y) was performed blind for this purpose. The moving target approached the participants from the side at three different, non‐constant accelerations. The results showed that three adolescents used less advanced timing strategies that involved the lower‐order variables of distance or velocity to govern movement initiation of at least one of their hands. Two of these were preterm VLBW and one was term SGA. The remaining adolescents, on the other hand, all relied on the higher‐order variable of time‐to‐contact to initiate their hand movements, and were, therefore, considered low‐risk. These results were compared with the cerebral magnetic resonance imaging results of the adolescents. The findings show that timing strategy pinpoints successfully those adolescents at risk of neurological problems. It was, therefore, concluded that the skill of using prospective information for catching can be used as a tool to detect diffuse signs of motor dysfunction, which are not readily detected by standard behavioural tests alone.
Verbal and memory skills in males with Duchenne muscular dystrophy
Authors: Hinton VJ , Fee R J , Goldstein E M , De Vivo D C
Published: June 2009

Duchenne muscular dystrophy (DMD) is a progressive pediatric disorder that affects both muscle and brain. Children with DMD have mean IQ scores that are about one standard deviation lower than population means, with lower Verbal IQ than Performance IQ scores. For the present study, verbal skills and verbal memory skills were examined in males with DMD with the Clinical Evaluation of Language Fundamentals, 3rd edition, and the California Verbal Learning Test for Children. Performance of 50 males with DMD (age range 6–14y, mean 9y 4mo [SD 2y 1mo]) was compared to normative values. Two subsets of the probands were also compared with two comparison groups: unaffected siblings (n=24; DMD group age range 6–12y, mean 9y 1mo [SD 1y 8mo]; sibling age range 6–15y, mean 9y 11mo [SD 2y 4mo]) and males with cerebral palsy (CP); (n=23; DMD group age range 6–9y, mean 7y 8mo [SD 1y 2mo]; CP age range 6–8y, mean 6y 8mo [SD 0y 8mo]). Results demonstrated that although males with DMD performed slightly more poorly than normative values, they performed comparably to the controls on most measures. Consistent deficits were observed only on tests requiring immediate repetition for verbal material (Recalling Sentences, and Concepts and Directions). On other language tasks, including tests of understanding and use of grammar, and understanding of semantic relationships, the males with DMD performed well. Moreover, the males with DMD performed well on multiple indices of verbal recall, and there was no evidence of declarative memory deficits. DMD is a single‐gene disorder that is selectively associated with decreased verbal span capacity, but not impaired recall.
Increased frequency of severe major anomalies in children conceived by intracytoplasmic sperm injection
Authors: Sanchez‐Albisua I , Borell‐Kost S , Mau‐Holzmann U A , Licht P , Krägeloh‐Mann I
Published: June 2009

The neurodevelopmental outcome of children born after intracytoplasmic sperm injection (ICSI) is controversial. We compared the medical and developmental outcome of 34 singletons born after ICSI (20 males, 14 females; mean ages of 18mo and 40mo [SD 9mo]; range 2y 10mo–4y 8mo) with 39 case control studies (21 males, 18 females; mean ages of 18mo and 40mo [SD 4mo]; range 3y–4y 1mo). Each child was assessed physically and tested in three development domains (fine motor, gross motor, and language). Five children born after ICSI versus two control children (p=0.2) had major congenital anomalies (MaCAs). Four children born after ICSI versus no control children had severe MaCAs (p=0.04). These were defined as having a significant impact on development or causing chronic disease: Angelman syndrome (n=1), lissencephaly (n=1), Hanhart syndrome (n=1), and persistent hyperinsulinemic hypoglycaemia of infancy (n=1). Karyotyping in 23 children born after ICSI revealed no abnormalities. An imprinting defect was found in the child with Angelman syndrome. Results of developmental assessment were in all cases normal at the age of 18 months except for the three children with Angelman and Hanhart syndromes, and lissencephaly. At the second assessment, five more children born after ICSI and four control children showed abnormalities in one or more developmental domains. We conclude that there seems to be a higher frequency of severe major anomalies in children born after ICSI. An increased risk for imprinting defects cannot be excluded. If we exclude children with severe MaCAs, the incidence of an abnormal somatic or neurodevelopmental outcome in the fourth year of life in children born after ICSI is similar to that of spontaneously conceived children.
Sjögren‐Larsson syndrome
Authors: Gordon Neil
Published: June 2009

Sjögren—Larsson syndrome is a recessively inherited syndrome caused by deficiency of fatty aldehyde dehydrogenase. The most common symptoms and signs are described, especially ichthyosis, spastic diplegia, and severe learning difficulties; but also other less frequent ones. Special investigations include sensory evoked potentials, electromyography, and proton magnetic resonance spectroscopy. Post‐mortem examination shows, in particular, an accumulation of lipid substances in specific regions of the brain. The diagnosis depends on the measurement of fatty aldehyde dehydrogenase in cultured fibroblasts from skin biopsies, and by identifying known mutations by allele‐specific polymerase chain reaction assay. Prenatal diagnosis is possible by using the same technique. The disorder is located on gene 17, and many mutations have been identified. Most mutations are unique to an affected family, but clinical variations may be due to unknown genetic and environmental factors. The deficiency of the enzyme impairs the oxidation of medium and long chain fatty aldehydes, and this may explain the link between the brain and skin disorders. The treatment of affected children needs input from a number of specialists, and their contributions are discussed.
Outcome assessments in children with cerebral palsy, Part I: descriptive characteristics of GMFCS Levels I to III
Authors: Oeffinger Donna , Gorton George , Bagley Anita , Nicholson Diane , Barnes Douglas , Calmes Janine , Abel Mark , Damiano Diane , Kryscio Richard , Rogers Sarah , Tylkowski Chester
Published: June 2009

This prospective cross‐sectional multicenter study assessed the relationships between Gross Motor Function Classification System (GMFCS) level and scores on outcome tools used in pediatric orthopedics. Five hundred and sixty‐two participants with cerebral palsy (CP; 339 males, 223 females; age range 4‐18y, mean age 11y 1mo [SD 3y 7mo]; 400 with diplegia, 162 with hemiplegia; GMFCS Levels I‐III;) completed the study. The Functional Assessment Questionnaire (FAQ), Gross Motor Function Measure (GMFM) Dimensions D and E, Pediatric Quality of Life Inventory (PedsQL), the Pediatric Outcomes Data Collection Instrument (PODCI), Pediatric Functional Independence Measure (WeeFIM), temporal‐spatial gait parameters, and O2cost were collected during one session. Descriptive characteristics are reported by GMFCS level clinicians can use for comparison with individual children. Tools with a direct relationship between outcome scores and GMFCS levels were the PODCI Parent and Child Global Function, Transfers & Basic Mobility, and Sports and Physical Function; PODCI Parent Upper Extremity Function; WeeFIM Self‐care and Mobility; FAQ Question 1; GMFM Dimensions D and E; GMFM‐66; O2cost; and temporal‐spatial gait parameters. Child report scores differed significantly higher than Parent scores for six of eight PODCI subscales and three of four PedsQL dimensions. Children classified into different GMFCS levels function differently.
Outcome assessments in children with cerebral palsy, Part II: discriminatory ability of outcome tools
Authors: Bagley Anita M , Gorton George , Oeffinger Donna , Barnes Douglas , Calmes Janine , Nicholson Diane , Damiano Diane , Abel Mark , Kryscio Richard , Rogers Sarah , Tylkowski Chester
Published: June 2009

Discriminatory ability of several pediatric outcome tools was assessed relative to Gross Motor Function Classification System (GMFCS) level in patients with cerebral palsy. Five hundred and sixty‐two patients (400 with diplegia, 162 with hemiplegia; 339 males, 223 females; age range 4‐18y, mean 11y 1mo [SD 3y 7mo]), classified as GMFCS Levels I to III, participated in this prospective multicenter, cross‐sectional study. All tools were completed by parents and participants when appropriate. Effect size indices (ESIs) for parametric variables and odds ratios for non‐parametric data quantified the magnitude of differences across GMFCS levels. Binary logistic regression models determined discrimination, and receiver operating characteristic curves addressed sensitivity and specificity. Between Levels I and II, the most discriminatory tools were Gross Motor Function Measure (GMFM‐66), velocity, and WeeFIM Mobility. Between Levels II and III, the most discriminatory tools were GMFM Dimension E, Pediatric Functional Independence Measure (WeeFIM) Self‐Care and Mobility, cadence, and Gillette Functional Assessment Questionnaire Question 1. Large ESIs were noted for Parent and Child reports of Pediatric Outcomes Data Collection Instrument (PODCI) Sports & Physical Function, Parent report of PODCI Global Function, GMFM Dimension E, and GMFM‐66 across all GMFCS level comparisons. The least discriminatory tools were the Quality of Life and cognition measures; however, these are important in comprehensive assessments of treatment effects.
Long‐term follow‐up of children with obstetric brachial plexus palsy I: functional aspects
Authors: Strömbeck Christina , Krumlinde‐Sundholm Lena , Remahl Sten , Sejersen Thomas
Published: June 2009

The aims of this study were to describe the development of sequelae in obstetric brachial plexus palsy (OBPP) and to identify possible differences in functional outcome from 5 years of age to follow‐up, 2 to 15 years later. A cohort of 70 participants (35 males, 35 females; age range 7‐20y, mean 13y 6mo [SD 4y 3mo], median 13y) with OBPP of varying degrees of severity were monitored. Differences in status between 5 years of age and follow‐up were studied. Active joint motion in the shoulder and hand function, especially grip strength, generally remained unchanged or improved, whereas a slight but significant deterioration occurred in elbow function. Shoulder surgery resulted in considerable improvement of shoulder function. Participants with nerve reconstruction had a similar profile of change as the non‐operated group. It was concluded that ongoing follow‐up of children with OBPP, beyond the preschool years, is required due to decreases in elbow function, a commonly occurring restriction in external rotation of the shoulder, together with individual variations in long‐term outcomes. In a related article (part II: neurophysiological aspects) long‐term neurophysiological and sensory aspects of OBPP are reported.
No deterioration in epilepsy and motor function in children with medically intractable epilepsy ineligible for surgery
Authors: Van Empelen Ron , Helders Paul J M , Van Rijen Peter C , Jennekens‐Schinkel Aag , Van Nieuwenhuizen Onno
Published: June 2009

The aim of this study was to examine whether severity of epilepsy, motor functioning, and epilepsy‐related restrictions change in children with medically intractable epilepsy who are ineligible for epilepsy surgery. The study was a prospective, longitudinal, 2‐year follow‐up of 28 children (14 females, 14 males). Their median age was 6 years 1 month (range 7mo–15y 4mo). Seizure types were: complex partial seizures (n=16), secondary generalized seizures (n=7), simple partial seizures (n=2), and mixed seizures (n=3). Severity of seizures, motor impairments, motor development, activities of daily life, and epilepsy‐related restrictions were rated at baseline and 6, 12, and 24 months thereafter. Seizure severity did not change significantly, nor did muscle strength, range of motion, or muscle tone. Motor retardation was ubiquitous but did not increase in 20 children without spasticity. Motor function of eight children with spasticity improved (Gross Motor Function Measure: baseline 70.5 [SD 35.5]; 24 months later 81.6 [SD 29.6], p < 0.05) but remained below reference values in four children. In the entire group, functional skills increased and caregiver assistance lessened. Restrictions did not change significantly. We conclude that during a 2‐year follow‐up period, in children with medically intractable epilepsy who do not have surgical intervention, seizure severity does not deteriorate, motor impairments do not increase, motor development does not deflect negatively, and activities of daily living and restrictions do not worsen.
Bilateral periventricular nodular heterotopia, severe learning disability, and epilepsy in a male patient with 46,XY,der(19)t(X;19) (q11.1–11.2;p13.3)
Authors: Balci Sevim , Unal Aysun , Engiz Ozlem , Aktas Dilek , Liehr Thomas , Gross Madelaine , Mrasek Kristin , Saygi Serap
Published: June 2009

Periventricular nodular heterotopia (PNH) is a rare neuronal migration disorder in which immature neurons fail to undergo a directed migration from the ventricular and subventricular zones to the cerebral cortex. Classic PNH occurs predominantly in females and is associated with periods of epilepsy and near‐normal intelligence. One gene associated with PNH was mapped to chromosome Xq28. PNH with learning disability is reported in 15 male patients with several syndromes and various congenital abnormalities such as craniosynostosis, frontonasal malformation, and agenesis of the corpus callosum. We present a 26‐year‐old male patient who was followed up with the diagnosis of epilepsy from the age of 1 year. Additionally the patient had severe learning disability, obesity, and hypogonadism. Imaging of his brain demonstrated PNH. Klinefelter syndrome was clinically suspected, and analysis of his chromosomes revealed a karyotype 46,XY,der(19)t(X;19) (q11.1–11.2;p13.3). Molecular techniques, such as subtelomere‐specific fluorescent in‐situ hybridization and multicolour banding, were also used. The same translocation was demonstrated in his mother and his maternal grandmother. This family might help to explain the gene localization of X‐linked recessive PNH. In our patient, PNH is associated with familial (X;19) translocation. To our knowledge, this unique combination has not been reported in the medical literature.
Quality of life among adolescents with cerebral palsy: what does the literature tell us?
Authors: Livingston Michael H , Rosenbaum Peter L , Russell Dianne J , Palisano Robert J
Published: June 2009

This review describes trends in quality of life (QOL) and health‐related quality of life (HRQOL) among adolescents with cerebral palsy (CP). Twenty original articles were identified by a structured search of multiple databases and grouped by design. Categories included descriptive cross‐sectional studies (n=8), measurement validation studies (n=9), and exploratory qualitative studies (n=3). Several trends were apparent. First, individuals with CP are reported to have decreased QOL and HRQOL compared with a normative population in some but not all areas of well‐being. Second, functional status measures such as the Gross Motor Function Classification System are reliable indicators of variations in physical function, but do not correlate consistently with psychosocial well‐being. Third, although adolescents with CP have different life issues than adults or children, limited research on factors associated with QOL and HRQOL has been described for this age range. We recommend that clinicians and researchers interested in assessing well‐being among adolescents with CP include participants from across the spectrum of motor impairment, allow adolescents to self‐report whenever possible, and assess adolescents independently, rather than including them with individuals from other age groups or clinical populations.
Clinimetric properties of participation measures for 5‐ to 13‐year‐old children with cerebral palsy: a systematic review
Authors: Sakzewski Leanne , Boyd Roslyn , Ziviani Jenny
Published: June 2009

This study systematically reviewed the validity, reliability, sensitivity to change, and clinical utility of measurements of participation for children with cerebral palsy. Sixteen measures were identified and seven met the inclusion criteria of having 30% content measuring participation, for use with children aged 5 to 13 years with physical disability, and were condition specific. The Children's Assessment of Participation and Enjoyment (CAPE) assessed participation in leisure and recreation, while the School Function Assessment (SFA) and School Outcome Measure addressed participation in the school environment. The Assessment of Life Habits for Children (LIFE‐H) measured participation in home, school, and community life, and the Children Helping Out: Responsibilities and Expectations assessed children's participation in household duties. The Canadian Occupational Performance Measure (COPM) and Goal Attainment Scaling (GAS) were individualized tools used to evaluate goal achievement. Results showed most instruments had adequate reliability and validity. The COPM and GAS were the only measures that reported adequate responsiveness to detect clinically significant change. Limited data are currently available to determine the responsiveness of the CAPE, LIFE‐H, and SFA. A combination of assessments is required to capture participation of children in home, school, and community environments.
Binding of visual and spatial short‐term memory in Williams syndrome and moderate learning disability
Authors: Jarrold Christopher , Phillips Caroline , Baddeley Alan D
Published: June 2009

A main aim of this study was to test the claim that individuals with Williams syndrome have selectively impaired memory for spatial as opposed to visual information. The performance of 16 individuals with Williams syndrome (six males, 10 females; mean age 18y 7mo [SD 7y 6mo], range 9y 1mo‐30y 7mo) on tests of short‐term memory for item and location information was compared with that shown by individuals with moderate learning difficulties (12 males, four females; mean age 10y 3mo [SD 1y], range 8y 6mo‐11y 7mo) and typically developing children (six males, 10 females; mean age 6y 8mo [SD 7mo], range 5y 10mo‐7y 9mo) of an equivalent level of visuospatial ability. A second aim was to determine whether individuals had impaired ability to ‘bind’ visual spatial information when required to recall ‘item in location’ information. In contrast to previous findings, there was no evidence that individuals with Williams syndrome were more impaired in the spatial than the visual memory condition. However, individuals with both Williams syndrome and moderate learning difficulties showed impaired memory for item in location information, suggesting that problems of binding may be generally associated with learning disability.*
Binaural interaction in specific language impairment: an auditory evoked potential study
Authors: Clarke Elaine M , Adams Catherine
Published: June 2009

The aim of the study was to examine whether auditory binaural interaction, defined as any difference between binaurally evoked responses and the sum of monaurally evoked responses, which is thought to index functions involved in the localization and detection of signals in background noise, is atypical in a group of children with specific language impairment (SLI). Binaural interaction components (BICs) in the brainstem were compared in 19 children with SLI (13 males, six females; age range 7y 4mo‐11y 10mo; mean age 9y 7mo [SD 1y 2mo]), and 31 comparison children with typical language development (16 males, 15 females; age range 7y 1mo‐11y 4mo; mean age 9y 7mo [SD 1y 5mo]). Children with SLI had a significantly smaller BIC amplitude than the comparison group. However, no clear relationship was found between BIC measures and severity of language impairment. We conclude that, for some children, SLI may be associated with reduced binaural interaction which may hinder the detection or localization of speech sounds from noisy contexts during critical periods of language acquisition.
Hippocampus, amygdala, and basal ganglia morphometrics in children after moderate‐to‐severe traumatic brain injury
Authors: Wilde Elisabeth A , Bigler Erin D , Hunter Jill V , Fearing Michael A , Scheibel Randall S , Newsome Mary R , Johnson Jamie L , Bachevalier Jocelyne , Li Xiaoqi , Levin Harvey S
Published: June 2009

While closed head injury frequently results in damage to the frontal and temporal lobes, damage to deep cortical structures, such as the hippocampus, amygdala, and basal ganglia, has also been reported. Five deep central structures (hippocampus, amygdala, globus pallidus, putamen, and caudate) were examined in 16 children (eight males, eight females; aged 9–16y), imaged 1 to 10 years after moderate‐to‐severe traumatic brain injury (TBI), and in 16 individually‐matched uninjured children. Analysis revealed significant volume loss in the hippocampus, amydala, and globus pallidus of the TBI group. Investigation of relative volume loss between these structures and against five cortical areas (ventromedial frontal, superomedial frontal, lateral frontal, temporal, and parieto‐occipital) revealed the hippocampus to be the most vulnerable structure following TBI (i.e. greatest relative difference between the groups). In a separate analysis excluding children with focal hippocampal abnormalities (e.g. lesions), group differences in hippocampal volume were still evident, suggesting that hippocampal damage may be diffuse rather than focal.
Dyslexia, learning, and pedagogical neuroscience
Authors: Fawcett Angela J , Nicolson Roderick I
Published: June 2009

The explosion in neuroscientific knowledge has profound implications for education, and we advocate the establishment of the new discipline of ‘pedagogical neuroscience’ designed to combine psychological, medical, and educational perspectives. We propose that specific learning disabilities provide the crucible in which the discipline may be forged, illustrating the scope by consideration of developmental dyslexia. Current approaches have failed to establish consensus on fundamental issues such as theoretical causes, diagnostic methods, and treatment strategies. We argue that these difficulties arise from diagnosis via behavioural or cognitive symptoms, even though they may arise from diverse causes. Rather than an inconvenience, variability of secondary symptoms within and across learning disabilities can inform both diagnosis and treatment. We illustrate how brain‐based theories lead to radical restructuring of diagnostic methods and propose that there is an urgent need to develop genetic and brain‐based diagnostic methods designed to lead to individually‐appropriate remediation and treatment methods.
Developmental coordination disorder at 8 years of age in a regional cohort of extremely‐low‐birthweight or very preterm infants
Authors: Davis N M , Ford G W , Anderson P J , Doyle L W ,   Victorian Infant Collaborative Study Group
Published: June 2009

The aims of this study were to determine the motor outcome of extremely‐low‐birthweight (ELBW; <1000g) or very preterm (<28wks) children compared with normal birthweight (NBW) children, to establish the perinatal associations of developmental coordination disorder (DCD) and its cognitive and behavioural consequences. Participants were consecutive surviving ELBW or very preterm children and randomly selected NBW(>2499g) children born in the state of Victoria, Australia, in 1991 or 1992. Main outcomes were: (1) results of the Movement Assessment Battery for Children (MABC) at 8 years of age; (2) cognitive function; (3) academic progress; and (4) behaviour. Of 298 consecutive ELBW/very preterm survivors, 255 (85.6%; 117 males, 138 females) had the MABC at a mean age of 8 years 8 months. More ELBW/very preterm children (9.5%) had DCD than the NBW group (2%, p=0.001). Only male sex increased the likelihood of DCD in ELBW/very preterm children (p=0.017). ELBW/very preterm children with DCD had worse cognitive function and academic test scores (up to 1SD below those without DCD); they also had more adaptive behaviour and externalizing problems, but not internalizing problems. DCD is more common in ELBW/very preterm children, has few perinatal correlates, and is associated with poor cognitive and academic performance as well as increased behaviour problems.
A randomized controlled trial comparing botulinum toxin A dosage in the upper extremity of children with spasticity
Authors: Kawamura Anne , Campbell Kent , Lam‐Damji Sophie , Fehlings Darcy
Published: June 2009

This study compared the effects of low and high doses of botulinum toxin A (BTX‐A) to improve upper extremity function. Thirty‐nine children (22 males, 17 females) with a mean age of 6 years 2 months (SD 2y 9mo) diagnosed with spastic hemiplegia or triplegia were enrolled into this double‐blind, randomized controlled trial. The high‐dose group received BTX‐A in the following doses: biceps 2U/kg, brachioradialis 1.5U/kg, common flexor origin 3U/kg, pronator teres 1.5U/kg, and adductor/opponens pollicis 0.6U/kg to a maximum of 20U. The low‐dose group received 50% of this dosage. Outcomes were measured at baseline and at 1 and 3 months after injection, and results were analyzed with a repeated‐measures analysis of variance. There was no significant difference between the low‐dose and high‐dose groups in upper extremity function over the 3‐month period as measured by the Quality of Upper Extremity Skills Test (F[1,37]=0.18, p=0.68). There was no difference between the groups in the Pediatric Evaluation of Disability Inventory Self Care Domain (F[1,37]=0.05, p=0.83). Although grip strength decreased over the 3‐month period, there was no difference between groups (F[1,32]=0.45, p=0.51). These findings indicate that there is no difference in hand and arm function between a low dose and a high dose of BTX‐A at 1 and 3 months after injection. This information can be used to guide dosage of BTX‐A for the management of upper extremity spasticity in children.
Self‐concept of children with cerebral palsy compared with that of children without impairment
Authors: Shields Nora , Loy Yijun , Murdoch Alison , Taylor Nicholas F , Dodd Karen J
Published: June 2009

This study examined whether the self‐concept of children with cerebral palsy (CP) differed from that of children without impairment. Forty‐seven children (24 males, 23 females; mean age 11y 8mo [SD 2y 6mo]) with spastic diplegia or hemiplegia were matched with children without impairment. The level of disability of the children with CP was classified as Gross Motor Function Classification System Level I (n=24), Level II (n=16), or Level III (n=7). The 36‐item Self‐Perception Profile for Children was used to assess six domains of self‐concept. No difference was found between the groups for Global Self‐worth, Physical Appearance, or Behavioural Conduct. Children with CP scored lower on Scholastic Competence (t92=‐2.75, p=0.01), Social Acceptance (t92=‐1.96, p=0.05), and Athletic Competence (t92=‐3.63, p<0.01) than children without impairment. Males with CP had lower scores for Scholastic Competence (t46=‐3.54, p<0.01) than males without impairment. Females with CP had lower scores for Social Acceptance (t44=‐2.31, p=0.03) than females without impairment. Both males and females with CP had lower scores for Athletic Competence than their peers without impairment. These results suggest that children with CP do not have a lower Global Self‐worth even though they may feel less competent in certain aspects of their self‐concept. Clinicians need to account for this when deciding on management strategies and may need to educate parents, carers, and health professionals that a lower self‐concept may not necessarily be associated with a diagnosis of CP.
The Capacity Profile: a method to classify additional care needs in children with neurodevelopmental disabilities
Authors: Meester‐Delver Anke , Beelen Anita , Hennekam Raoul , Nollet Frans , Hadders‐Algra Mijna
Published: June 2009

The aim of this study was to determine the interrater reliability and stability over time of the Capacity Profile (CAP). The CAP is a standardized method for classifying additional care needs indicated by current impairments in five domains of body functions: physical health, neuromusculoskeletal and movement‐related, sensory, mental, and voice and speech, in children from 3 to 18 years of age. The intensity of care in each domain is defined from 0 (no need for additional care) to 5 (needs help with every activity). The intensity of additional care in each of the five separate domains indicates the CAP for the individual child. We developed the CAP to inform the parents and other caregivers of children with non‐progressive, permanent neurodevelopmental disabilities, such as cerebral palsy and myelomeningocele, about the consequences of these conditions. To determine interrater agreement and stability over time, the CAPs of 67 children (39 males, 28 females) with a neurodevelopmental disability (mean age 18y [SD 1.2y]; range 14‐22y) were assessed based on a semi‐structured interview. In addition, the CAPs of the same individuals at the age of 3 years were determined based on a chart review. Interrater agreement of the CAP at the age of 3 was good to very good (weighted kappa 0.64‐0.92). Agreement between the CAP at the age of 18 and the CAP at the age of 3 (providing evidence for stability over time) was also good (weighted kappa 0.68‐0.77), except for the domain ‘physical health functions’, about which agreement was relatively poor (0.47). We conclude that the CAP is a reliable instrument for classifying the additional needs of a child with a non‐progressive, permanent neurodevelopmental disability. The preliminary evidence for the stability over time of such needs according to the CAP should be validated in a prospective study.
Gastrostomy placement in paediatric patients with neuromuscular disorders: indications and outcome
Authors: Ramelli Gian Paolo , Aloysius Annie , King Caroline , Davis Trak , Muntoni Francesco
Published: June 2009

Studies of children with neurodevelopmental disorders have shown that receiving nutrition through a gastrostomy can improve clinical outcomes and quality of life. However, there is little information on gastrostomy and its effect in patients with neuromuscular disorders. A retrospective casenote review of all patients with a gastrostomy, followed‐up at the Hammersmith Hospital, London, was undertaken to assess the indications for, and outcomes of, gastrostomy placement. Notes for 32 patients (17 males, 15 females) were reviewed (age range 32mo–31y; median age 12y 5mo). We found three main groups of diagnoses: congenital muscular dystrophy (n=15), structural congenital myopathies (n=11), and other neuromuscular disorders (n=6). Two main patterns of feeding problems were identified before gastrostomy: swallowing difficulties, and nutrition and growth problems. The follow‐up period after gastrostomy was from 12 months to 19 years (mean 5y). Weight faltering was reversed in 17 out of 22 patients, and height faltering in 9 out of 14, where data were available. Twenty‐six patients had a reduced frequency of chest infections. No significant complication of gastrostomy placement was documented. Twenty‐eight patients or their families were happy with the results of the gastrostomy. Gastrostomy seems to have a substantial positive impact in patients with neuromuscular disease and feeding difficulties.
A novel microdeletion in 1(p34.2p34.3), involving the SLC2A1 (GLUT1) gene, and severe delayed development
Authors: Vermeer Sascha , Koolen David A , Visser Gepke , Brackel Hein J L , Van Der Burgt Ineke , De Leeuw Nicole , Willemsen Michèl A A P , Sistermans Erik A , Pfundt Rolph , De Vries Bert B A
Published: June 2009

A de novo 4.1‐megabase microdeletion of chromosome 1p34.2p34.3 has been identified by array‐based comparative genomic hybridization in a young male with severely delayed development, microcephaly, pronounced hypotonia, and facial dysmorphism. The deleted region encompasses 48 genes, among them the glucose transporter 1 (SLC2A1 or GLUT1) gene. The deletion of the GLUT1 gene was in line with the abnormal ratio of cerebrospinal fluid (CSF) glucose to blood glucose, indicative of GLUT1 deficiency syndrome (MIM #606777). GLUT1 deficiency syndrome is characterized by therapy‐resistant infantile seizures, developmental delay, acquired microcephaly, spasticity, ataxia, and a low concentration of glucose in the CSF. It is known that a ketogenic diet can lead to better control of seizures. This case study shows that identifying a microdeletion as the cause of learning disability is not only important for genetic counselling but might also lead to therapeutic intervention.
Does surgical management of the hand in children with spastic unilateral cerebral palsy affect functional outcome?
Authors: Van Munster Judith C , Maathuis Karel G B , Haga Nienke , Verheij Nienke P , Nicolai Jean‐Philippe A , Hadders‐Algra Mijna
Published: June 2009

The aim of this review was to examine the literature on the effects of surgery of the spastic hand in children with cerebral palsy on functional outcome and muscle coordination. We performed a search of the relevant literature in Medline, Embase, and Biological Abstracts from 1966 to June 2006. The search resulted in eight studies on the effect of surgery on functional outcome and three studies on the effect of surgery on muscle coordination. Heterogeneity in outcome measures precluded meta‐analysis. The studies revealed that – at the level of impairment – surgery has a positive effect on supination and dorsiflexion of the wrist. At the level of function, surgery might improve grip strategy and induce an increase of the repertoire of grips and spontaneous use of the hand. The evidence for the functional effects is, however, limited. Whether the possibly improved function is mediated by alterations in muscle coordination patterns remains unclear. The muscle coordination studies provided inconclusive results. We concluded that surgery improves the position of the hand and there are indications that it might improve hand function. Future research should address the question: does surgery improve hand function and, if so, is this due solely to a better hand position or does change in muscle coordination play an additional role?