bluuebunny/crossref_metadata_2025 huggingface dataset:
Visual–proprioceptive mapping in children with developmental coordination disorder
Authors: Mon‐Williams M A , Wann J P , Pascal E
Published: July 2010

Developmental coordination disorder (DCD) occurs in a small but significant proportion of children who present with impaired body–eye coordination and show poor acquisition of motor skills. This study investigated the visual–proprioceptive mapping ability of children with DCD from a small selected group, with particular reference to the use of vision in matching tasks. The children with DCD in this study were significantly poorer than control children on all matching tasks. They seemed to have particular difficulty in cross‐modal judgements that required the use of visual information to guide proprioceptive judgements of limb position. A distinction is drawn between tasks that can be achieved purely through sensory matching and those that require body‐centred spatial judgements, suggesting that it is the latter that posits a particular difficulty for children with DCD.
Evidence of cognitive visual problems in children with hydrocephalus: a structured clinical history‐taking strategy
Authors: Houliston Mary Jane , Taguri Abdel H , Dutton Gordon N , Hajivassiliou Constantinos , Young Daniel G
Published: July 2010

Damage to the occipital cortex in children can result in many complex disorders of cognitive visual function. A series of clinical questions, developed from the specific problems of a cohort of children with cortical visual impairment, was asked of the parents of 200 children with no history of cerebral pathology, aged 5 to 12 years. One hundred and ninety‐two parents gave reliable consistent responses. The results show a progressive improvement in performance with age, culminating in few 11‐ and 12‐year olds having frequent problems, apart from 8% having frequent difficulty with orientation in new surroundings and 2% having problems with simultaneous perception tasks. The parents of 52 children (aged 5 to 17 years) with shunted hydrocephalus were then asked the same set of questions. Evidence of cognitive visual problems was identified in 27 of these children of whom 16 manifested multiple difficulties. The disabilities identified by our study comprised problems with: shape recognition, simultaneous perception, perception of movement, colour perception, orientation, object recognition, and face recognition. The range, nature, and combinations of these disorders are presented in this paper.
Respiratory tract infections due to direct and reflux aspiration in children with severe neurodisability
Authors: E Morton R , R Wheatley , J Minford
Published: July 2010

Lower respiratory tract infections in children with severe neurodisability are usually caused by aspiration of stomach contents from gastroesophageal reflux (GOR) or direct aspiration (DA) of food due to oral and pharyngeal motor problems. To determine the contributions and interactions of GOR and DA, oesophageal 24‐hour pH monitoring and feeding videofluoroscopy were performed in 34 children (age range 7 months to 16 years, mean 7 years) who had severe physical and learning disabilities and who were slow feeders. Subjects were divided into three groups according to the frequency of their respiratory tract infections. Subjects in group 1 had no respiratory tract infections (N=10); five had GOR and none had DA. Subjects in group 2 had minor respiratory tract infections but had not received more than one course of antibiotics for this in the previous year (N=8); two had GOR alone, four had DA alone, and two had neither. All subjects in group 3 had recurrent respiratory tract infections (N=16); one had GOR alone, seven had DA alone, and eight had both GOR and DA. This study suggests that oral and pharyngeal motor problems are the major cause of respiratory tract infection in children with severe neurodisability. These problems lead to DA and, if GOR is present, to the aspiration of stomach contents. Those children with both DA and GOR are more likely to have severe respiratory tract infections which may lead to gastrostomy feeding (together with fundoplication). GOR without sufficient oral and pharyngeal motor problems to cause DA is less likely to cause respiratory tract infection in children with severe neurodisability.
Speech discrimination and phonological working memory in children with ADHD
Authors: Norrelgen Fritjof , Lacerda Francisco , Forssberg Hans
Published: July 2010

This study examined phonological working memory and speech discrimination among children with attention‐deficit–hyperactivity disorder (ADHD) with and without motor problems. Forty‐one children were assigned to three groups; children with ADHD (N=9), children with ADHD plus developmental coordination disorder (ADHD+, N=13), and age‐matched control children (N=19). The subjects’ability to classify stimulus pairs was examined in two experiments. The first experiment required subjects to discriminate pairs of monosyllabic stimuli with contrasting consonants to test speech discrimination without using a working‐memory load. In the second protocol, subjects were exposed to two‐ to five‐syllabic non‐word pairs with contrasting vowels in order to test speech discrimination with a working‐memory load. The subjects classified the pairs as being either the same or different in both experiments. No significant differences were found between the subject groups in the discrimination task with monosyllables. When exposed to the two‐ to five‐syllabic stimuli, the ADHD+ group scored significantly lower than both other groups. This was attributed to a higher sensitivity to working‐memory load. Some possible explanations of this effect are discussed.
Refractive errors in children with cerebral palsy, psychomotor retardation, and other non‐cerebral palsy neuromotor disabilities
Authors: Sobrado Paloma , Suá rez Julia , García‐Sá Francisco Alberto , Usón Edmundo
Published: July 2010

The aim of this study was to analyse the refractive state of four different groups of children: those with spastic cerebral palsy (CP), aged between 7 and 81 months(N=50); psychomotor retardation, aged between 19 and 70 months (N=16); other neuromotor dysfunctions, aged between 12 and 75 months (N=37); and without psychomotor retardation, aged between 9 and 73 months (N=181). Refractive errors were determined using cycloplegic retinoscopy and non‐cycloplegic retinoscopy (Mohindra's technique). We found higher percentages of hyperopia, tendency toward hyperopia, and other refractive anomalies in all the pathological groups of children than in the non‐pathological control groups. Children from both the non‐CP pathological control group and the group with psychomotor retardation had similar or even higher levels of hyperopia than children from the group with spastic CP. Our results in different age groups indicate a less effective normal emmetropization course in all the pathological groups of children studied. The correction of refractive errors is needed in these children before the end of the neural plasticity period.
Reliability and responsiveness of the Barry–Albright Dystonia Scale
Authors: Barry Margaret J , VanSwearingen Jessie M , Albright A Leland
Published: July 2010

The reliability and responsiveness of the Barry–Albright Dystonia (BAD) Scale, a 5‐point ordinal severity scale for secondary dystonia, was assessed. For interrater reliability, 13 raters scored 10 videotaped patients; for intrarater reliability, two raters rated the videotape again. For test–retest reliability, patients were rated on two occasions. Four inexperienced raters scored patients, received training, then scored additional patients. To assess responsiveness, we compared patient and physician global ratings of change (better, same, and worse) with BAD Scale score changes for 18 patients on intrathecal baclofen (ITB) trials. We assessed reliability with the intraclass correlation coefficient (ICC). The mean ICC for total BAD Scale scores were as follows: interrater reliability 0.866, intrarater reliability 0.967 and 0.978, test–retest reliability 0.978 (before training) and 0.967 (after training). We found the BAD Scale responsive to change, with most improved scores in patients rated by the patient, family, and neurosurgeon as‘better'. The total scores were reliable for experienced raters. We recommend training for clinicians interested in using the scale.
Cutaneomuscular reflex responses recorded from the lower limb in children and adolescents with cerebral palsy
Authors: Gibbs John , Harrison Linda M , Stephens John A , Evans Andrew L
Published: July 2010

Cutaneomuscular reflex (CMR) responses were recorded from lower‐limb and trunk muscles in 27 subjects with cerebral palsy (CP) (spastic, 21; athetoid, six) and in neurologically healthy (control) subjects, aged 3 to 15 years, while standing. In the 21 subjects with spastic CP, but not in the six subjects with athetoid CP, CMR responses were more widely distributed between ipsilateral lower‐limb and trunk muscles compared with age‐matched control children. CMR responses in older subjects with CP were similar to younger control subjects, lacking supraspinally mediated, long‐latency components. Short‐latency, spinally‐mediated, excitatory CMR components were seen simultaneously in pairs of distal, antagonistic lower‐limb muscles in half of the subjects with spastic CP, but in none of the control children. In subjects with spastic‐type CP, the abnormal reflex responses indicate disordered spinal and supraspinal inputs to motor neurones, although there was no convincing correlation between these responses and the severity of spasticity.
Continuum of reflex excitability in hemiplegia: influence of muscle length and muscular transformation after heel‐cord lengthening and immobilization on the pathophysiology of spasticity and clonus
Authors: Lin Jean‐Pierre , Brown J Keith , Walsh E Geoffrey
Published: July 2010

The electromyographic (EMG) responses to tendon taps at the ankle and ensuing muscular twitch forces and temporal parameters were studied at varying angles across the joint range in 18 children, aged 3 to 14.9 years, with congenital hemiparetic cerebral palsy and 22 healthy (control) children, aged 3 to 13.6 years. Those subjects with hemiparesis were community ambulators without assistance. In all subjects, passive muscle stretch caused a waxing of the reflex EMG and twitch force near neutral (with the sole of the foot at right angles to the tibia) and a diminution of these with further dorsiflexion. Twitch times increased with each dorsiflexing increment, being slowest at maximum dorsiflexion and fastest at the resting plantarflexion angle. Heterogeneity of the hemiparetic‐limb data is evident when compared with data of non‐paretic and unaffected limbs, with clear differences in the clonic (fast twitch) as opposed to non‐clonic (slow twitch) muscles. In four cases with clinical clonus, clonus frequency was reduced by passive dorsiflexion. Plaster immobilization for 1 month produced clonus which was previously absent in one subject, and caused a fast‐twitch phenotype to emerge in two subjects. Follow‐up after heel‐ cord lengthening in one subject showed that clonus frequency diminished from 9 to 3 Hz with slowing and strengthening of muscle‐twitch phenotype. Short‐ and long‐term peripheral manipulations appear to regulate neuromuscular excitability according to whether muscles are loaded or unloaded. Although damage to the nervous system provides the setting for reflex excitability, the data suggest that the muscle length (which specifies the joint angle) and the muscle‐twitch phenotype of any given limb for any given case appear to dictate the actual speed and strength of reflex muscle‐twitch and clonus profiles. This study illustrates how peripheral manipulations of muscles and tendons may alter the expression of what have hitherto been considered as exclusively central phenomena.
Behavioral style of young boys with fragile X syndrome
Authors: Hatton Deborah D , Bailey Donald B , Hargett‐Beck Melissa Q , Skinner Martie , Clark Renee D
Published: July 2010

To study the behavioral style or temperament of 45 boys, aged 47 to 88 months, with full‐mutation fragile X syndrome (FXS), 102 parent ratings on the Behavioral Style Questionnaire (McDevitt and Carey 1978) were recorded. These ratings were analysed with a variety of statistical techniques. Considerable variability was evident in temperament profiles; consequently, a characteristic profile was not identified for FXS. Boys with FXS differed significantly from the reference sample on five of nine temperament dimensions. They were more active and less intense, approachable, adaptable, and persistent. No significant differences were found in distractibility, rhythmicity, mood, or sensory threshold. Only 16 of the 45 boys in the sample could be classified as easy, difficult, or slow to warm up. There was no link between severity of developmental disability and temperament ratings. This supports the theory that intelligence and temperament are separate constructs. Scores on temperament dimensions were stable over time. Our results suggest that many of the behaviors observed in boys with FXS may be related to temperament. Consequently, parent counseling and environmental modifications should be considered as first line treatment. The question of whether the behavior problems observed in boys with FXS are innate or whether they result from poorness of fit between child and environment is an important issue that needs further study.
Corpus callosum may be similar in children with ADHD and siblings of children with ADHD
Authors: Overmeyer S , Simmons A , Santosh J , Andrew C , Williams SCR , Taylor A , Chen W , Taylor E
Published: July 2010

No previous studies have used morphological neuroimaging to compare children with ADHD with siblings of children with ADHD. To test the hypothesis that the total size of the corpus callosum is altered in children with hyperkinetic disorder, the corpus callosum was outlined from a single midline proton‐density weighted slice (containing the septum pellucidum). Fifteen boys with a refined phenotype of ADHD (mean age 10.2 years) and 15 healthy male siblings of children with ADHD (mean age 10.6 years) were enrolled in the study. The two groups were compared for global brain size and the callosal areas of Witelson. No significant differences were found between the study and comparison groups for any of the corpus callosum areas, even after age, global brain size, and handedness were covaried (using MANOVA). In addition, corpus callosum sizes do not seem to differ between children with ADHD and unaffected siblings of children with ADHD. Clinicians should not base their pathophysiological diagnosis of this disorder on an abnormality of callosal development.
New ocular movement detector system as a communication tool in ventilator‐assisted Werdnig‐Hoffmann disease
Authors: Kubota Masaya , Sakakihara Yoichi , Uchiyama; Yoshiaki , Nara; Atsushi , Nagata Tsutomu , Nitta Hiroshi , Ishimoto; Koh , Oka Akira , Horio Keizo , Yanagisawa Masayoshi
Published: July 2010

A non‐contact communication system was developed for a ventilator‐assisted patient with Werdnig‐Hoffmann disease who had lost all voluntary movements except for those of the eye. The system detects the extraocular movements and converts them to either a‘yes’signal (produced by one lateral eyeball movement) or a‘no’signal (produced by two successive lateral eyeball movements) using a video camera placed outside the patient's visual field. The patient is thus able to concentrate on performing a task without any intrusion from the detection system. Once the setting conditions of the device have been selected, there is no need for any resetting, as the patient is unable to move his body. In addition to playing television games, the child can use the device to select television channels, compose music, and learn written Japanese and Chinese characters. This seems to broaden the patient's daily world and promote mental development.
Monozygotic boys with fragile X syndrome
Authors: Sheldon Laurence , Turk Jeremy
Published: July 2010

Monozygotic twin boys with fragile X syndrome underwent thorough genetic, psychiatric, neurological, and language evaluations at 10 years of age. They both demonstrated physical features, speech and language difficulties, social problems, and attentional deficits that characterize the behavioural phenotype of fragile X syndrome. Despite identical genetic constitutions, there were important developmental and behavioural heterogeneities. Twin A showed less social interaction and symbolic play and more speech and language dysfunction than twin B. Twin A also had significantly larger caudate volumes. It is suggested that the Xq27.3 anomaly may not be sufficient to account for all the behavioural phenotypic and neuroanatomical features of fragile X syndrome.
Cerebellar ataxia, anterior horn cell disease, learning difficulties, and dystonia: a new syndrome.
Authors: Wilmshurst Jo M , Surtees Robert , Cox Tim , Robinson Richard O
Published: July 2010

The following case reports describe a new condition of cerebellar ataxia, anterior horn cell disease, dystonia, and learning difficulties. Four cases are described. The condition appears to be of autosomal recessive inheritance as the group is made up of two pairs of sisters. All cases were evident by 3 years of age. Anterior horn cell disease was of a type not previously described at this age in association with cerebellar ataxia. Further genetic studies suggest the condition is not allelic with spinal muscular atrophy having no evidence of deletion of the survival motor neurone gene.
Aetiology in severe and mild mental retardation: a population‐based study of Norwegian children
Authors: Strømme Petter , Hagberg Gudrun
Published: July 2010

The aetiology of mental retardation (MR) was studied in a population‐based series of Norwegian children derived from 30 037 children born between 1980 and 1985. The study included 178 children, 79 with severe MR (SMR) (IQ<50) and 99 with mild MR (MMR) (IQ 50 to 70). Aetiology was divided into two main groups: biopathological and unspecified. The biopathological group comprised 96% of SMR and 68% of MMR, and was subdivided into prenatal (70% and 51%), perinatal (4% and 5%), and postnatal damage (5% and 1%), and a group of undetermined timing of the damaging event (18% and 11%). Single‐gene disorders accounted for 15 of the 63 children with genetic disorders, including X‐linked recessive in six. During the course of the study, at least 27 (15%) children had their aetiological diagnosis revised. Gestational age <32 weeks, birthweight <1500g, and Apgar scores 0 to 2 at 1 and 5 minutes implied a significantly increased risk of MR, but contributed to only 4% of the children in the study. Decreased birthweight (1500 to 2499 g) and Apgar scores 3 to 6 at 1 and 5 minutes showed increased probability of MR. Despite extensive investigations, 4% of SMR and 32% of MMR were not identified with any biological markers and were considered as unspecified MR, several most probably representing the lower end of the normal IQ distribution in the population.
Analgesic effects of botulinum toxin A: a randomized, placebo‐controlled clinical trial
Authors: Barwood Shane , Baillieu Charles , Boyd Roslyn , Brereton Kate , Low Janette , Nattrass Gary , Graham H Kerr
Published: July 2010

Postoperative pain in children with spastic cerebral palsy (CP) is often attributed to muscle spasm and is difficult to manage using opiates and benzodiazepines. Adductor‐release surgery to treat or prevent hip dislocation in children with spastic CP is frequently performed and is often accompanied by severe postoperative pain and spasm. A double‐blinded, randomized, placebo‐controlled clinical trial of 16 patients (mean age 4.7 years) with a mainly spastic type of CP (either diplegic or quadriplegic in distribution) was used to test the hypothesis that a significant proportion of postoperative pain is secondary to muscle spasm and, therefore, might be reduced by a preoperative chemodenervation of the target surgical muscle by intramuscular injection of botulinum toxin A (BTX/A). Compared with the placebo, BTX/A was found to be associated with a reduction in mean pain scores of 74% (P<0.003), a reduction in mean analgesic requirements of approximately 50% (P<0.005), and a reduction in mean length of hospital admission of 33% (P<0.003). It was concluded that an important component of postoperative pain in the patient population is due to muscle spasm and this can be managed effectively by preoperative injection with BTX/A. These findings may have implications for the management of pain secondary to muscle spasm in other clinical settings.
How does the brain learn language? Insights from the study of children with and without language impairment
Authors: Bishop D V M
Published: July 2010

Neurobiological studies have generated new ways of thinking about development of brain structure and function. Development involves more than just growth from simple to complex structures. The initial over‐abundance of neurons and synaptic connections is subsequently pruned of those that are non‐functional. In addition, as behavioural and cognitive functions emerge and become automatized, the underlying brain representations are reorganized. In this paper, I shall argue that these different modes of neurodevelopmental change provide a useful metaphor for examining language acquisition. It will be argued that language acquisition can involve learning to ignore and inhibit irrelevant information, as well as forming new ways of representing complex information economically. Modular organization is not present from the outset, but develops gradually. This analysis suggests a new way of assessing specific language impairment (SLI). There has been much debate as to whether children with SLI lack specific modular components of a language processing system. I propose instead that these children persist in using inefficient ways of representing language. Finally, I consider what we know about the neurobiological basis of such a deficit. There is mounting evidence that children with SLI have subtle structural anomalies affecting the language areas of the brain, which are largely genetically determined. We should not, however, conclude that the language difficulties are immutable.
The findings in paediatric obstetric brachial palsy differ from those in older patients: a suggested explanation
Authors: Vredeveld Jan W , Blaauw Gerhard , Slooff Bart A C J , Richards René , Rozeman Stan C A M
Published: July 2010

An EMG and nerve‐conduction‐study protocol has been developed and used in all patients with brachial plexus lesions since 1985. The findings of EMG and nerve‐conduction studies in obstetric brachial palsy (OBP) mostly suggest a falsely optimistic prognosis. To analyse this, all subjects were selected with complete avulsion of both roots C5 and C6 and/or complete rupture of the upper trunk verified during operation from a group of 162 infants with OBP (aged 4 to 14 months) and a group of 184 child and adult patients (aged 6 to 74 years) with a traumatic brachial plexus lesion. Fourteen infants, approximately 4 months old, with OBP, and 19 adults (aged 16 to 30 years) met the selection criteria. The infants from the group with OBP had at 4 months a nearly normal recruitment pattern of motor units in the biceps brachii and deltoid muscles, with little or no denervation. However, in the older group with traumatic brachial palsy, the same lesion caused complete denervation of both muscles. From the group with OBP, a third group (N=3) with the same lesion plus an additional lesion of C7 or a rupture of the middle trunk was selected. This additional lesion resulted in nearly complete denervation of both muscles. This means that C7 largely contributes to the innervation of both muscles at the time of birth, but this innervation is lost during later life in normally developing individuals (apoptosis). A central mechanism might be responsible for the clinical palsy and later spontaneous improvement which is always found in the infants with OBP.
Identification of malnutrition in children with cerebral palsy: poor performance of weight‐for‐height centiles
Authors: Samson‐Fang Lisa J , Stevenson Richard D
Published: July 2010

This study aims to evaluate the use of the United States National Center for Health Statistics (NCHS) weight‐for‐height centiles (WHC) in screening children with cerebral palsy (CP) for depleted body fat and to identify an alternate screening method. Growth data from 276 children aged from 3 to 12 years with CP were analyzed retrospectively. Height or a proxy for height, mid‐upper arm circumference, weight, and skinfold thicknesses were recorded. Mid‐upper arm fat area was calculated for each participant. The sensitivities and specificities of WHC and a number of alternative anthropometric screening methods for identifying participants with severely depleted fat stores were determined. WHC <10th centile failed to identify 45% of children with severely depleted fat stores. Triceps skinfold thickness <10th centile identified 96% of malnourished children. WHC standards lack adequate sensitivity for identification of severely depleted fat stores in children with CP. Use of triceps skinfold thickness, using cut‐off value of <10th centile for age and sex, is recommended to screen for suboptimal fat stores in children with CP.
Sensitivity of the pendulum test for assessing spasticity in persons with cerebral palsy
Authors: Fowler Eileen Greenan , Nwigwe Azuka I , Ho Teresa Wong
Published: July 2010

The sensitivity of the pendulum test to variation in spasticity in persons with spastic cerebral palsy (CP) was tested in 30 participants with CP and 10 participants without CP (controls) (mean age 13.8 years). The participants with CP were classified into three groups, with normal (mean age 15.9 years), mild/moderate (13.0 years), or severe (23.0 years) muscle tone, as assessed clinically using a modified Ashworth scale. Joint motion during the pendulum test was measured with an electrogoniometer. Muscle relaxation was confirmed using surface EMG. Outcome measures from the pendulum test were (1) number of oscillations, (2) duration of oscillations, (3) excursion of the first backward swing, and (4) relaxation index (first swing excursion/difference between the starting and resting angles). Data were assessed using one‐way analysis of variance. Outcome measures 1 to 3 differed significantly between control participants and participants with CP (p<0.05). The first swing excursion was the best predictor of the degree of spasticity in persons with CP, being significantly different between all groups (p<0.05). The number of oscillations and their duration differentiated between control participants and all participants with CP (p<0.05) but not between participants with CP who had mild/moderate versus severe spasticity (p>0.05). The relaxation index was not a sensitive measure (p>0.05 between most study groups). We conclude that the pendulum test is a valid tool for assessing spasticity in persons with CP and that the first swing excursion is the most sensitive outcome measure.
Impaired force coordination during object release in children with hemiplegic cerebral palsy
Authors: Eliasson Ann‐Christin , Gordon Andrew M
Published: July 2010

Recent studies on hand motor control in children with cerebral palsy (CP) have focused on the coordination of fingertip forces during the grasping and lifting of objects. However, little is known about the ability to replace and release an object from grasp, a function that is just as important for fine dexterity. The present study examines the coordination of fingertip forces during the replacement and release of an object (either 200 g or 400 g) from grasp in 14 children (aged between 7 and 13 years) with hemiplegic CP and in 14 age‐matched typically developing children. The results indicate that children with hemiplegic CP abruptly replaced the object but had a prolonged and uncoordinated release of the grasp. Unlike what was seen in the control children, the grip and load forces decreased sequentially in the children with CP. However, all the children could appropriately scale the rate of force decrease based on somatosensory weight‐related information from the ongoing lift. The results provide further information about the impaired hand skills in children with hemiplegic CP.
Visual evoked potentials in young persons with newly diagnosed diabetes: a long‐term follow‐up
Authors: Verrotti Alberto , Lobefalo Lucio , Trotta Daniela , Della Loggia Giuseppe , Chiarelli Francesco , Luigi Colangelo , Morgese Guido , Gallenga Pierenrico
Published: July 2010

To evaluate the presence of electrophysiological abnormalities in the visual function of young persons with diabetes, visual evoked potentials were recorded, in basal conditions and after photostress, in 30 patients with newly diagnosed insulin‐dependent diabetes mellitus. Their mean age was 17.6 years (3.6 SD), and their glycosated haemoglobin (HbA1c) was 9.4% (1.6 SD). Thirty healthy age‐ and sex‐matched individuals were evaluated as the control group. This study showed that the P100 latency was significantly delayed in patients with diabetes compared with the control group (p<0.01), while the N75 to P100 amplitude was similar in both groups. These measurements were repeated after 6 months, when all participants with diabetes had achieved good metabolic control (HbA1c 7.2% [1.5 SD]). At this second evaluation a complete normalisation of all parameters was observed. These findings suggest that early functional abnormalities of the optic nerve can be detected at onset of diabetes, and that glycaemic control reverses these abnormalities.
Development of ideomotor praxis representation
Authors: Njiokiktjien C , Verschoor C A , Vranken M , Vroklage L M
Published: July 2010

To create a short screening instrument to investigate the development of ideomotor praxis representation (IPR), 357 normally developing children between the ages of 21/2 and 91/2 years were investigated. The IPR screening consisted of six mimed actions which had to be performed on verbal request. The greatest change in the representational style is the transition from performing the action using the body as the object to performing with an imaginary object (i.e. symbolic representation). This transition suggests a development from a concrete and egocentric level of performance to an abstract and allocentric level. Before the age of 6 years differences are evident between self‐directed and externally directed gestures. The level of symbolic representation has to be dissociated from the degree of motor precision, which shows hardly any improvement after 6 years of age. With the proposed model one may interpret the level of IPR in clinical practice. IPR screening is clinically relevant from the age of 41/2 years onwards.
Prevalence of psychiatric diagnoses in children with mental retardation: data from a population‐based study
Authors: Strømme Petter , Diseth Trond H
Published: July 2010

The main purpose of the study was to estimate the prevalence of psychiatric diagnoses in children with mental retardation (MR) (IQ≤70). All children born between 1980 and 1985 (N=30037) in Akershus County, Norway, were screened for possible MR and assessed with either IQ tests or standardized developmental tests. A total of 178 children, 79 with severe mental retardation (SMR) (IQ<50) and 99 with mild mental retardation (MMR) (IQ 50 to 70) were included for further study. Psychiatric symptomatology was assessed as a standard part of the neurodevelopmental examination, which included a semistructured parent interview, a clinical child interview, and retrieval of the charts of previous child psychiatric examinations. Psychiatric diagnoses were classified according to the International Classification of Disease (ICD‐10). In total, 65 (37%) of the total population with MR (95% confidence intervals 29 to 44) were registered to have psychiatric diagnoses, the most common being hyperkinesia (n=28) and pervasive developmental disorder (n=15). Psychiatric diagnoses were present in 42% of the population with SMR and 33% of the population with MMR (p=0.4). Of all children found to have a psychiatric diagnosis, approximately one‐third had previously been examined by a child psychiatrist and indicated a previously unrecognized need for these services to children with MR.
Explicit memory in low‐risk infants aged 19 months born between 27 and 42 weeks of gestation
Authors: de Haan Michelle , Bauer Patricia J , Georgieff Michael K , Nelson Charles A
Published: July 2010

The aim of this study was to determine whether there are primary effects of prematurity on the development of explicit memory. Elicited imitation of action sequences was used to compare immediate and 15‐minute delayed memory in term and preterm infants (19 months corrected age; n=48) who were at low risk: none had experienced the medical or social risk factors often associated with preterm birth. Relative to infants born at term (38 to 40 weeks’gestation), children who had been born at 27 to 34 weeks’gestation showed lower levels of ordered recall; performance of healthy infants born at 35 to 37 weeks’gestation was intermediate and did not differ significantly from that of the other groups. These results suggest that specific cognitive deficits can occur as a function of preterm birth even in low‐risk infants.
Ten‐year follow‐up of adolescent‐onset anorexia nervosa: physical health and neurodevelopment
Authors: Wentz Elisabet , Gillberg I Carina , Gillberg Christopher , Råstam Maria
Published: July 2010

To study the development of physical health and some neuromotor functions in anorexia nervosa (AN) 51 individuals (48 females, three males) with a mean AN onset of 14 years, recruited after community screening, were followed prospectively together with 51 age‐, sex‐, and school‐matched individuals without AN (controls). About 10 years after AN onset, all individuals were examined in respect of physical health and neurodevelopment. There were no deaths. Weight and height had normalised, except in three participants with persistent AN. Significantly more participants with AN had a physical complaint/disorder, including hirsutism. This might be a long‐term complication in weight restored AN. Dysdiadochokinesis occurred almost exclusively among individuals with former AN in accordance with our previous studies.
Multicore myopathy: respiratory failure and paraspinal muscle contractures are important complications
Authors: Rowe P W , Eagle M , Pollitt C , Bullock R E , Bushby K M D
Published: July 2010

Three ambulant males with multicore myopathy, a rare congenital myopathy, are reported with nocturnal hypoventilation progressing to respiratory failure at the age of 9, 13, and 21 years. Deterioration in these individuals occurred over several months without any precipitating event. Patients had clinical evidence of nocturnal hypoventilation with hypoxaemia and hypercapnia. Forced vital capacity was significantly reduced (20 to 43% of predicted level). These parameters improved on institution of overnight ventilation using a BiPAP pressure support ventilator with face mask or nasal pillows with O2 saturation maintained above 90% overnight and an increase in forced vital capacity by as much as 100% (0.3 to 0.6 litres). This was matched by a symptomatic and functional improvement. Also present in these patients and not previously reported is the association of multicore myopathy with paraspinal contractures which produce a characteristic scoliosis described as a 'side‐sliding’spine. This may be improved by spinal bracing or surgery.
Perinatal infection is an important risk factor for cerebral palsy in very‐low‐birthweight infants
Authors: Wheater Mary , Rennie Janet M
Published: July 2010

Sixty‐nine very‐low‐birthweight infants out of a population of 923 had cerebral palsy (CP) at an 18‐month follow‐up. Thirty‐nine of these had cranial ultrasound abnormalities in the neonatal period and 30 had normal cranial ultrasounds. The distribution of subtypes of CP differed markedly between the two groups, with hemiplegia predominating in those with abnormal cranial ultrasounds and diplegia in those with normal cranial ultrasounds. Regardless of ultrasound appearance, the relative risk of CP increased approximately fourfold with a neonatal history of sepsis.
Specific language impairment with or without hyperactivity: neuropsychological evidence for frontostriatal dysfunction
Authors: Williams D , Stott C M , Goodyer I M , Sahakian B J
Published: July 2010

A neuropsychological investigation of the association between specific language impairment (SLI) and hyperactivity was conducted on four groups of 6‐year‐old children. The groups, each consisting of five boys and five girls, were: children with SLI alone, hyperactivity alone, both SLI and hyperactivity, and neither disorder (controls). Cognitive functioning was examined using selected non‐verbal tests from the Cambridge Neuropsychological Test Automated Battery. SLI was not associated with significantly reduced performance on any of the neuropsychological measures. However, hyperactivity was associated with deficits on a test of attentional set shifting previously demonstrated to be sensitive to frontostriatal dysfunction. Further, hyperactive children showed significantly reduced spatial spans on a test of spatial working memory thought to measure parietal lobe functioning. There were no substantial differences between the groups on tests of frontal lobe function except for reduced performance on a task of attention in the hyperactive group. There were no significant interactions between hyperactivity and SLI on any of the neuropsychological measures. The pattern of neuropsychological deficits shown by children with attention‐deficit‐hperactivity disorder (ADHD) suggests relatively specific problems in inhibitory control of attentional selection. This cognitive deficit implicates brain areas including basal ganglia and ventro‐lateral prefrontal cortical areas in ADHD, thus providing evidence for disruption of frontostriatal functional loops. SLI and hyperactivity can occur as comorbid syndromes or symptom complexes and this should be noted when interpreting results of tests measuring cognitive performance. The findings suggest that these two groups of disorders have different cognitive correlates.
Twelve‐year follow‐up of children exposed to alcohol in utero
Authors: Autti‐Rämö Ilona
Published: July 2010

Eighty‐two women who were consuming alcohol while pregnant attended a special clinic at the University Central Hospital, Helsinki with the aim of reducing heavy drinking during pregnancy. The children born to these women were followed up regularly. During their preschool years the children were assessed to have fetal alcohol syndrome, fetal alcohol effects, alcohol‐related neurodevelopmental disorder, pre‐ and/or postnatal growth retardation, or they were assessed to have normal cognitive and somatic growth. Of the original children, 70 of 82 could be traced at the age of 12 years. Through semistructured interview and contact with the health and social care authorities, information was gathered about schooling, family structure, whether help had been sought for behavioural difficulties and major adverse events in the family. The longer the intrauterine alcohol exposure and the more severe the diagnosis related to prenatal alcohol exposure, the more often the children required special education, were temporarily or permanently taken into care, and had behavioural problems. There is a considerable need for prolonged multidisciplinary follow‐up and support of all children whose mothers have not been able to reduce drinking in early pregnancy, whether or not cognitive disturbances are evident in early childhood.
An international perspective on Tourette syndrome: selected findings from 3500 individuals in 22 countries
Authors: Freeman Roger D , Fast Diane K , Burd Larry , Kerbeshian Jacob , Robertson Mary M , Sandor Paul
Published: July 2010

We have established a multisite, international database of 3500 individuals diagnosed with Tourette syndrome (TS). The male:female ratio is 4.3:1 for the total sample, with wide variation among sites; the male excess occurs at every site. Anger control problems, sleep difficulties, coprolalia, and self‐injurious behavior only reach impressive levels in individuals with comorbidity. Anger control problems are strongly correlated with comorbidity, regardless of site, region, or whether assessed by neurologists or psychiatrists. The mean age at onset of tics is 6.4 years. At all ages, about 12% of individuals with TS have no reported comorbidity. The most common reported comorbidity is attention‐deficit‐hyperactivity disorder. Males are more likely to have comorbid disorders than females. The earlier the age at onset, the greater the likelihood of a positive family history of tics. An understanding of the factors producing these and other variations might assist in better subtyping of TS. Because behavioral problems are associated with comorbidity, their presence should dictate a high index of suspicion of the latter, whose treatment may be at least as important as tic reduction. The established database can be used as the entry point for further research when large samples are studied and generalizability of results is important.
Early posttraumatic seizures in non‐accidental head injury: relation to outcome
Authors: Barlow Karen M , Spowart Jacqui J , Minns Robert A
Published: July 2010

To document the characteristics of early posttraumatic seizures (EPTS) in non‐accidental head injury (NAHI), and examine their relation with outcome, a retrospective study was carried out. All children with NAHI admitted to the Royal Hospital for Sick Children, Edinburgh, since 1981 were identified. The characteristics of EPTS, EEG, and outcome were noted. Forty‐four cases were identified. The average age of children at presentation was 5.9 months. Thirty‐two of these children had EPTS. The median length of follow‐up was 3 years. The mortality rate was six in 44 (14%). The neurodevelopmental outcome correlated significantly with the presence and severity of EPTS (Tau=0.317,p=0.017). Of survivors, 22% developed late posttraumatic epilepsy; the outcome in those with epilepsy was significantly worse than those without (p<0.0001). It was concluded that the severity of the primary brain injury dictates the severity of the EPTS and neurodevelopmental status at follow‐up.
Maternal expressed emotion and treatment compliance of children with epilepsy
Authors: Otero Soraya , Hodes Matthew
Published: July 2010

This study investigated the association between family relationships and compliance in the treatment of childhood epilepsy. It was a prospective study of 21 families with a child who had epilepsy attending Central Middlesex Hospital, London. There were 13 boys and eight girls, with a mean age of 12.0 years(SD 2.9) at initial assessment. Mothers were interviewed for assessment of expressed emotion as a measure of parent‐child relationships. Assessment of the mothers’adjustment using the General Health Questionnaire (Goldberg 1978), and psychological adjustment of the children using the Rutter Scales (Rutter et al. 1970a), were completed by mothers and teachers. Reassessment was 3 to 4 years after initial contact, including a paediatric case‐note review to assess clinic attendance and overall treatment compliance. Significantly more of the group who had a good level of compliance had recovered from epilepsy at follow‐up. Good treatment compliance was found to be associated with less maternal hostility and criticism. Children and mothers in the good compliance group had fewer psychiatric symptoms. Poor treatment compliance and the associated psychological disturbances suggest that assertive paediatric and psychosocial intervention may be needed for some children with epilepsy.
Changes in the incidence of childhood autism and other autistic spectrum disorders in preschool children from two areas of the West Midlands, UK
Authors: Powell JE , Edwards A , Edwards M , Pandit BS , Sungum‐Paliwal SR , Whitehouse W
Published: July 2010

The incidence of childhood autism and other autistic spectrum disorders (ASDs) in preschool children was determined for two areas of the West Midlands between 1991 and 1996. Children diagnosed before the age of 5 years and residing within the study areas at diagnosis were detected from the records of four child development centres. The incidence rate per 10000 children per year for the combined areas was 8.3 for all children with ASDs, 3.5 for classical childhood autism (CA), and 4.8 for other ASDs. Rates were similar in both areas, despite differences in social deprivation and proportions of ethnic minorities. While rates for classical CA increased by 18% per year, a much larger increase (55% per year) was seen for ‘other ASDs’, suggesting that clinicians are becoming increasingly able and/or willing to diagnose ASDs in preschool children.
Single cause, polymorphic neuronal migration disorders: an animal model
Authors: Rosen Glenn D , Galaburda Albert M
Published: July 2010

Injury to the developing cortical plate can result in a variety of neuronal migration disorders. The results are reported of experimental research aimed at determining whether these different types of neocortical malformations are the consequence of comparable injury of varying intensity. Freezing probes were placed on the skulls of 44 newborn rats (age equivalent to 4 to 5 months of gestation in humans) and induced either one or two freezing injuries of durations ranging from 2 to 20 seconds. A variety of cortical malformations including minor laminar dysplasias, molecular layer ectopias, microgyria, and porencephalic cysts were seen in the brains of these animals when they were examined on postnatal day (P)2, P21, and P60. The severity of the malformation was directly related to the strength (number of hits and duration) of the freezing injury. These results suggest that a single etiologic event of varying severity during neuronal migration to the neocortex can induce widely disparate malformations of the cortex.
Effects of preterm extrauterine visual experience on the development of the human visual system: a flash VEP study
Authors: Tsuneishi Syuichi , Casaer Paul
Published: July 2010

To compare the functional maturation of the human visual system between intra‐ and extrauterine course flash visual evoked potentials (VEPs) in preterm infants (gestational age 24 to 36 weeks). Previously established normal values, with special reference to the two components of the N1 wave, were employed (Tsuneishi 1995). A cross‐sectional analysis of 124 infants at 36 weeks postmenstrual age (PMA), showed that there are no differences in the absolute values of VEP peak latencies depending on the postnatal age (PNA). Conversely, the N1 wave form changes with increasing PNA from a wave in which the early peak (N1a) has a higher amplitude than the late peak (N1b) into the reverse situation with a higher amplitude of the N1b as compared to N1a. This observation may correlate with the maturation of the neuronal networks in the visual cortex. In a longitudinal analysis of 50 infants followed for more than 5 sessions of weekly recordings, we found that the individual rapid decrease in the N1a latency, which may reflect the initiation of myelination in the optic radiation, most frequently occurs at around 37 weeks PMA, regardless of PNA. Preterm extrauterine visual experience has little effect on the myelination process in the visual pathway, but has a marked effect on the developmental changes in VEP wave form which reflect the developmental changes of the neuronal networks in the visual cortex.