bluuebunny/crossref_metadata_2025 huggingface dataset:
Effectiveness of speech intervention for phonological disorders: a randomized controlled trial
Authors: Deborah Almost MCISc, MSc  , Peter Rosenbaum MD, FRCP
Published: July 2010

Thirty children of preschool age with severe phonological disorders were randomly assigned to two treatment groups. Group 1 received treatment for 4 months followed by 4 months without treatment while group 2 underwent 4 months without treatment followed by 4 months of treatment. The outcome measures used were the Assessment of Phonological Processes ‐ Revised (APP‐R), the Goldman‐Fristoe Test of Articulation (GFTA), the Percentage Consonants Correct (PCC), and Mean Length of Utterance (MLU). Group 1 showed significant differences on scores of phonological measures (APP‐R, GFTA, and PCC) after the first 4 months of the study. At the 8‐month assessment point the measures for conversational speech intelligibility continued to be significantly different, with group 1 scores higher than those of group 2. The expressive language measure did not detect a difference between groups at any time; however group 1 scores were consistently higher than group 2 scores.
MRI brain changes in subjects with Down syndrome with and without dementia
Authors: Godfrey D Pearlson MD  , Steven N Breiter MD  , Elizabeth H Aylward PhD  , Andrew C Warren MD, PhD  , Matthew Grygorcewicz BA  , Sophia Frangou MD  , Patrick E Barta MD PhD  , Margaret B Pulsifer PhD
Published: July 2010

Individuals with Down syndrome (DS), a disorder of known genetic etiology (trisomy of chromosome 21), exhibit several types of structural brain abnormalities that are detectable pathologically and by MRI. In addition, in middle age, individuals with DS develop histological and, in some cases, clinical features of Alzheimer's disease (AD). Abnormalities in MRI scans of 50 adults with DS, 11 of whom had clinical dementia, are described and compared with those of 23 cognitively normal, healthy subjects who were matched for age, sex, and race. Qualitative visual analogue scale (VAS) ratings on MRI hard copies for all subjects and computer‐aided volume measures for a subsample of subjects were carried out. On VAS, subjects with DS had larger lateral ventricles, a higher frequency of posterior fossa arachnoid cysts/megacisterna magna and fewer scans rated as normal compared with controls. Quantitatively, total brain and gray‐matter volumes were reduced in DS, as were the volumes of the left hippocampus and amygdala; ventricle volumes were larger. Post hoc comparisons of subjects with DS with and without dementia revealed that on VAS the former had more generalized atrophy for age, mesial temporal shrinkage, and third ventricular enlargement. Similarly, total train, left hippocampus, and left amygdala volumes were reduced quantitatively in subjects with DS with dementia, while ventricular volumes were increased.
Autism and autistic epileptiform regression with occipital spikes
Authors: Nass Ruth , Gross Anna , Devinsky Orrin
Published: July 2010

The electroencephalographs abnormalities seen in Landau‐Kleffner syndrome (LKS) (language deterioration) are non‐specific, and consist of a variety of epileptiform discharge patterns including continuous slow spike‐wave discharges during sleep, focal sharp waves with spikes, and centrotemporal (rolandic) spikes. Similarly, the EEG abnormalities seen in autistic epileptiform regression (language and social/behavioral deterioration) are nonspecific and overlap with those seen in LKS. By contrast, distinct epilepsy syndromes in otherwise normal children occur in the EEG‐deflned benign focal epilepsies of childhood. Occipital spikes or spike‐wave present either in the older child with visual symptoms and headache or in the younger child with autonomic symptoms followed by brief or prolonged partial motor seizures. Seven young children (five from a consecutive series of 42) presenting clinically with autism or autistic regression and possible or definite seizures, whose EEGs revealed occipital spikes or spike‐wave characteristic of the benign epilepsies, are reported. Although occipital spikes are commonly seen in young children as an age‐dependent EEG‐defined benign focal epilepsy, their high frequency in this population with cognitive difficulties suggests a possible causal relation. The effects of the epileptiform discharge on cognitive functioning presumably reflect extension into temporal and parietal lobes, rather than occipital disturbances per se.
Effects of fluoxetine treatment in young children with idiopathic autism
Authors: DeLong G Robert , Teague Lou Anne , Kamran Myra McSwain
Published: July 2010

Thirty‐seven children, aged between 2 and 7 years, with idiopathic autism underwent an open‐label trial of fluoxetine treatment. All had assessment of diagnosis, developmental status, and family psychiatric history. Independent developmental testing before and after starting fluoxetine permitted quantification of language acquisition in a subgroup. Twenty‐two of the 37 children had a beneficial treatment response sustained during continuing treatment for 13 to 33 months (mean 21 months). Eleven had an excellent response and were able to attend mainstream classrooms. Eleven had a good response though they remained identifiably autistic. Fifteen children had no benefit. Responders showed behavioral, language, cognitive, affective, and social improvements. Responders with adequate testing showed marked increases in language acquisition at every stage of development as compared with (1) pretreatment status, (2) responses to other treatments, (3) ability in non‐language (matching) tasks, and (4) historical controls from the literature. The response to fluoxetine strongly correlated with a family history of major affective disorder. These preliminary findings implicate serotonergic mechanisms in autistic symptomatology and warrant further study with controlled trials.
Acute transverse myelitis in childhood: nine cases and review of the literature
Authors: Knebusch Martin , Strassburg Hans Michael , Reiners Karlheinz
Published: July 2010

Acute transverse myelitis (ATM) is a rare disease in childhood and adolescence. It is characterized by paraplegia with or without sensory symptoms and bladder dysfunction, and typically manifests itself over a period of hours to 1 week. This is a report of nine patients who were treated between 1993 and 1996. To exclude treatable conditions, spinal and cranial MRI with and without contrast medium, electrophysiologic tests, and CSF examinations are performed as soon as possible after onset. At present post‐ or parainfectious inflammation is thought to be the most frequent cause of ATM. Some causes of ATM can be proved only by follow‐up examination. The most important differential diagnoses are multiple sclerosis and Guillain‐Barré syndrome with its variants. After exclusion of spinal cord compression, and if specific antibiotic treatment is not possible, a 3‐day high‐dose IV steroid pulse therapy is the most promising treatment. Prognosis is variable and residual symptoms are common. A controlled multicenter study is suggested to assess epidemiology, etiology, and prognosis of ATM.
Children presenting with convulsions (including status epilepticus) to a paediatric accident and emergency department: an audit of a treatment protocol
Authors: Garr R E , Appleton R E , Robson W J , Molyneux E M
Published: July 2010

All children who presented in a convulsion, including convulsive status epilepticus, to the accident and emergency department over a 12‐month period and who required treatment, were reviewed retrospectively to identify the effectiveness and safety of a specific treatment protocol. This protocol recommends the initial use of one, or if necessary, two doses of rectal or intravenous diazepam (0.4 mg/kg) followed by the simultaneous administration of phenytoin (18 mg/kg) and rectal paraldehyde (0.4 mL/kg), with instructions for maximum doses and timings of administration. Eighty‐one evaluable children (52 male) were audited. The mean age of the study population was 4.1 (range 0.1 to 14.9) years. Overall, the presenting convulsion was successfully terminated in 76 children (94%) within the accident and emergency department. In 69 children (85% of the entire study population) this was after a single dose of diazepam (rectal in 41 and intravenous in 28). In only an additional two children did the presenting convulsion stop after a second dose of diazepam. In five of the 10 children (50%) who received paraldehyde and phenytoin as a combination, the convulsion stopped. Nine patients (11%) required admission to the intensive‐care unit, five because of persisting convulsive activity, and four because of respiratory depression. The results of this retrospective audit suggest that the current treatment protocol appears to be effective and relatively safe in treating acute convulsions, including convulsive status epilepticus. The audit is to be repeated prospectively to either confirm or refute these findings before recommending any changes to the protocol.
Cognitive predictors of young children's readiness for powered mobility
Authors: Tefft Donita , Guerette Paula , Furumasu Jan
Published: July 2010

Independent mobility in early childhood has been associated with the development of various cognitive and psychosocial skills. However, children with physical disabilities are not always able to move independently and may be at risk for delays in these areas. Early provision of powered mobility can offer young children an opportunity for independent mobility. Despite this, there is little information to help determine when a young child has the cognitive skills necessary to operate a powered wheelchair safely. This current research aims to identify these skills. A cognitive assessment battery and a wheelchair mobility training and assessment program were developed. Twenty‐six children with physical disabilities between the ages of 20 and 36 months were evaluated on the cognitive assessment and participated in the wheelchair training and assessment program. A stepwise regression analysis was used to determine which of the cognitive skills predicted wheelchair mobility performance. The cognitive domains of spatial relations and problem solving were found to be significant and accounted for 57% of the variance in wheelchair skills. Developmental cut‐off points on these scales as they relate to wheelchair skills are presented and clinical applications are discussed.
Predictors of developmental delay at 18 months and later school achievement problems
Authors: Sonnander Karin , Claesson Malin
Published: July 2010

The aim of the study was to examine the predictive value of the variables of parental assessment score, pre‐, peri‐, and postpartum optimality, sex, socioeconomic status (SES), and maternal education with respect to developmental delay at 18 months, and intellectual disability and school achievement problems at 8 and 14 years. The sample studied comprised 101 children (53 low scorers and 48 controls) originally from a total population of 2783 children assessed by their parents at 18 months using a screening instrument. Data were analysed by logistic regression. The results yielded moderate but statistically significant correlations between predictor and outcome variables. Optimality score and maternal education were the best predictors of developmental delay at 18 months. At 8 years, parental assessment score and maternal education constituted the best predictors of school achievement problems. At 14 years, SES together with parental assessment score were included in the model, when school achievement problems were predicted. Parental assessment score showed the strongest association with school achievement problems at both 8 and 14 years, when children with intellectual disability were included in the analysis. The exclusion of children with intellectual disability from the analyses yielded a stronger association between maternal education (at 8 years) and SES (at 14 years) and school achievement problems. The overall classification accuracy of the models varied between 67% and 88%. Specificity varied from 65% at 18 months to 95% at 14 years. Sensitivity varied from 70% at 18 months and 55% (all cases) and 42% (children with intellectual disability excluded) at 14 years.
Carbamazepine hypersensitivity and the use of lymphocyte proliferation responses
Authors: Brown K L , Henderson D C , Nadel S , Tanveer A , Booy R
Published: July 2010

This report describes a case of multisystem failure in a 5‐year‐old boy who was being treated with carbamazepine for a seizure disorder. Carbamazepine hypersensitivity was diagnosed from the combination of the clinical constellation and the results of the patient's peripheral blood monocyte proliferation responses to both carbamazepine and a panel of other medications.
Autistic disorders in children with CHARGE association
Authors: Fernell Elisabeth , Olsson Vivi‐Ann , Karlgren‐Leitner Carina , Norlin Berit , Hagberg Bibbi , Gillberg Christopher
Published: July 2010

Three children with CHARGE association (coloboma, heart defect, atresia of the choanae, retarded growth and development, genital hypoplasia, ear anomalies and hearing defects) as well as concomitant autistic disorder are reported. Two of the children also had mental retardation. Several types of cerebral maldevelopment/dysfunction have been reported in CHARGE association. With regard to aetiology, involvement of the neural crest has been suggested. Autism in CHARGE association may represent a neuro‐endocrine dysfunction. Children with a CHARGE association have many medical problems and therefore autistic behaviour can easily be overlooked. A multidisciplinary approach with respect to assessment, treatment, and habilitation is of vital importance.
A population‐based approach to the investigation of osteopenia in Rett syndrome
Authors: Leonard H , Thomson MR , Glasson E J , Fyfe S , Leonard S , Bower C , Christodoulou J , Ellaway C
Published: July 2010

This study compares bone mass in a national sample of girls with Rett syndrome (RS) with a sample of control children. The Australian RS Database was the source of cases for this population‐based study. Hand radiographs were available from 101 of 137 subjects (74% of the known Australian population of girls with RS aged ? 20 years). Control radiographs matched for age, sex, and laterality were obtained from hospital radiology departments. A measure of cortical thickness was made from the difference between the outer diameter and the medullary space in the second metacarpal bone. A meanz‐score value for cortical thickness and percentage cortical area for each individual was calculated. The mean cortical thickness (zscore) for girls with RS was –1.94 compared with –0.38 for control children (P<0.001). In girls with RS, the mean cortical thickness decreased with age (P<0.001). In girls who were taking epilepsy medication it was –2.21 compared with –1.23 in those not taking epilepsy medication (P<0.001). There was no evidence of a beneficial effect of increased calcium intake on cortical thickness. A similar pattern was obtained when percentage cortical area was estimated. In multivariate analysis, increasing age and use of anticonvulsant medication were associated with decreased cortical thickness and only use of anticonvulsant medication with decreased percentage cortical area. Fractures had occurred in one‐third of cases and it was estimated that just over 40% of girls would sustain a fracture by the age of 15 years. Girls with RS may be at increased risk of fractures and their bone quality compromised as determined by cortical thickness and percentage cortical area measurements from the second metacarpal.
Quality of general movements in infancy is related to neurological dysfunction, ADHD, and aggressive behaviour
Authors: Hadders‐Algra Mijna , M C Groothuis Annelies
Published: July 2010

The quality of general movements (GMs) was assessed repeatedly during the first postnatal months in a mixed group of 52 children at either low or high risk for neurodevelopmental disorders. In addition, all children were reexamined at 4 to 9 years. The follow‐up assessment consisted of a neurological examination and an evaluation of behaviour by means of parental questionnaires. The quality of GMs changed frequently, to stabilize in the final phase. The final GM phase is that of the so‐called fidgety GMs which occurs between 2 and 4 months postterm. The quality of the fidgety GMs predicted outcome very well. Definitely abnormal GMs were associated with a high risk for the development of cerebral palsy, whereas mildly abnormal GMs were associated with the development of minor neurological dysfunction, attention‐deficit–hyperactivity disorder, and aggressive behaviour.
A long‐term follow‐up of cognitive, emotional, and behavioural sequelae to Reye syndrome
Authors: Meekin S L , Glasgow J F T , McCusker C G , Rooney N
Published: July 2010

Eighteen adolescents who had survived Reye syndrome (RS) in early childhood were assessed on cognitive, emotional, and behavioural variables in a second follow‐up study tracking this group. Siblings were used as controls. The entire group with RS had survived with no obvious neurological damage at the first follow‐up study. Indeed, current findings suggested that long‐term cognitive, emotional, and behavioural functioning was comparable to siblings in approximately half of the group with RS. However, two factors were associated with a less favourable outcome. Cognitive, emotional, and behavioural functioning were significantly poorer in the subgroup of survivors whose illness had occurred in the first year of life. In addition, loss of consciousness, although the association with poor outcome was not as noticeable, was also associated with relative deficits on some scales of cognitive ability. Many of these deficits had not been obvious at the first follow‐up and the importance of neurodevelopmental factors are considered. Finally, the implications of these findings for research and interventions in RS and other such encephalopathies are discussed.
Apneustic breathing in children with brainstem damage due to hypoxic–ischemic encephalopathy
Authors: Saito Yoshiaki , Hashimoto Toshiaki , Iwata Hideki , Takahashi Kazutoshi , Fukumizu Michio , Sasaki Masayuki , Hanaoka Shigeru , Sugai Kenji
Published: July 2010

To confirm the presence of apneusis in patients with hypoxic–ischemic encephalopathy and to clarify which factors influence their respiratory patterns, polygraphic studies were performed on two patients. Apneusis was clinically suspected in both patients who had severe brainstem damage. In one subject, inputs of vagal afferents from the gastrointestinal tract and the urinary bladder often resulted in extreme tachypnea instead of apneusis. Lung inflation facilitated expiration during inspiratory arrest. Expiration preceded a periodic inhibition of rigospastic discharge in the right biceps muscle. In the other subject, prolonged inspiratory pauses with cyanosis occurred with or without preceding epileptic seizure. Both phenytoin dose reduction and treatment with tandospirone, a serotonin‐1A agonist, were effective in improving the respiratory distress in this subject.
Predictive utility of the Bayley Infant Neurodevelopmental Screener (BINS) risk status classifications: clinical interpretation and application
Authors: Aylward Glen P , Verhulst Steven J
Published: July 2010

Predictive validity and clinical implications of the increasingly popular Bayley Infant Neurodevelopmental Screener (BINS) risk status classifications have not been previously reported. In this longitudinal follow‐up study, the BINS was administered to high‐risk infants at 6, 12, and 24 months of age, and the McCarthy Scales at 3 years of age. Ninety‐two children were evaluated at 6 and 36 months, 105 at 12 and 36 months, and 118 at 24 and 36 months; 190, 125, and 140 infants were included in the comparisons at 6 to 12, 6 to 24, and 12 to 24 months. BINS risk status was classified as low, moderate, or high; or as a binary variable, LOWRISK/HIGHRISK. The three BINS items groups were moderately correlated. Consistency was most variable in the moderate‐risk group. BINS risk was predictive of 36‐month function in 18 out of 18 comparisons. Odds ratios, ranging from 2.76 to 54.70, were significant in 15 out of 18 logistic models. An early high‐risk classification was associated with increased probability of later developmental morbidity. The BINS offers an alternative to detailed assessment in high‐volume clinical applications and has good concurrent and predictive validity.
Outcome of infants with unilateral Sturge‐Weber syndrome and early onset seizures
Authors: Kramer Uri , Kahana Esther , Shorer Zamir , Ben‐Zeev Bruria
Published: July 2010

Patients with Sturge‐Weber syndrome often present with seizures during the first year of life. Currently, only patients with clinically significant seizures who do not respond to medical treatment are candidates for early epileptic surgery. However, a delay of surgical treatment may result in cognitive deterioration. We studied the correlation between parameters and outcome of seizures to re‐examine the criteria for early epilepsy surgery. We performed a retrospective chart review combined with telephone interviews of parents of all Israeli infants with unilateral Sturge‐Weber syndrome and early onset seizures, and we examined whether age of seizure onset and seizure intensity were correlated with cognitive level and the degree of hemiparesis at follow‐up. We recruited a total of 15 patients with unilateral Sturge‐Weber syndrome and early onset seizures, five of whom underwent epilepsy surgery. The mean follow‐up period of all the patients was 15 years: six patients had normal intelligence, four had borderline cognitive level, three had mild mental retardation* and two had moderate mental retardation. Eight of the ten non‐operated patients still experience seizures at follow‐up. Cognitive delay was significantly correlated with seizure intensity in the early period, but not with the age of seizures onset, the degree of hemiparesis, or the presence of ongoing seizures. We conclude that high seizure intensity in young patients with Sturge‐Weber syndrome is a prognostic marker for mental deterioration.
Changes in synergistic movement patterns after selective dorsal rhizotomy
Authors: Olree Kenneth S , Engsberg Jack R , Ross Sandy A , Park T S
Published: July 2010

The purpose of this investigation was to quantitatively compare synergistic movement patterns between seven children (four male, three female; aged 3 to 17 years; mean 6.7, SD 5.3) without cerebral palsy (CP) (controls) and 27 children (15 male, 12 female; aged 2 to 16 years; mean 5.7, SD 3.7) with spastic diplegic CP before and after selective dorsal rhizotomy (SDR). The study design was also descriptive, comparing results of before and after SDR to control children. A two dimensional video system and retroreflective markers were used to obtain sagittal plane angles for the hip, knee, and ankle during maximum active knee flexion and extension. Correlations were calculated between the knee and hip and between the knee and ankle joint pairs. Control children demonstrated non‐synergistic movement patterns (‐0.75 and ‐0.61). These results were significantly different from children with CP (0.40 and 0.43,p<0.05). Eight months after SDR, synergistic patterns did not significantly change from preoperative results (0.23 and 0.36,p>0.05) and remained significantly different from control children (p<0.05). We conclude that it may not be possible to significantly alter synergistic patterns after SDR.
Audit of a children's epilepsy clinic
Authors: Robinson R O , Edwards M , Madigan C , Ledgar S , Boutros A
Published: July 2010

A newly established tertiary epilepsy clinic at Guy's Hospital, London was evaluated using a structured questionnaire. Fifty parents of children with epilepsy completed this questionnaire, with some contribution from the children. While a high degree of satisfaction was obtained by the parents and where appropriate the children, specific questions about additional resources implied a large number of unmet needs. This raises methodological issues about the value of open‐ended questions in audit questionnaires. In addition, concerns were expressed about many areas, some of which had direct medical implications that were not always raised with the doctors. It is concluded that the service has benefited from both clinical and neuropsychological support. The audit has prompted a number of improvements, including establishing a discussion group for adolescents.
Set‐shifting in healthy children and in children with idiopathic or cryptogenic epilepsy
Authors: Schouten A , Oostrom K J , Peters A C B , Verloop D , Jennekens‐Schinkel A
Published: July 2010

Set‐shifting is an important aspect of attention and regulation of behaviour. The ability of young children to shift set is debated. Set‐shifting is usually examined using sorting tasks that are designed for adults and hence are inadequate for children. In this study, an experimental Sorting Task for Children (STC) is administered to 54 healthy children (aged 4 to 10 years) and compared with a widely used adult sorting task, the Modified Card Sorting Test. This experiment shows that preschool children are capable of set‐shifting. An extensive error analysis of the STC reveals that perseverations are not predominant. Hence, set‐shifting in young children may be characterised by ‘trial and error’ rather than by being stuck in a mental set, which is suggested to be typical of adults with frontal lobe damage. A pilot study of 10 children with idiopathic or cryptogenic epilepsy shows unstable set‐shifting rather than inability to shift set, which is concordant with known problems in regulation of behaviour.
Outcome after ischaemic stroke in childhood
Authors: Ganesan V , Hogan A , Shack N , Gordon A , Isaacs E , Kirkham F J
Published: July 2010

A parental questionnaire was used to investigate the outcome for children who had had ischaemic stroke, who were seen at Great Ormond Street Hospital, London between 1990 and 1996. The results of functional assessments carried out by a physiotherapist and an occupational therapist, and of quantitative evaluations carried out by a neuropsychologist were used for validation where possible. The relationship between clinical and radiological factors and outcome were examined. The children were aged between 3 months and 15 years at the time of stroke (median age 5 years) and the period of follow‐up ranged from 3 months to 13 years (median duration 3 years). Of the 90 children for whom data were obtained, 13 (14%) had no residual impairments. Outcome was good in 37 children (40%) and poor in 53 (60%) (defined according to whether impairments interfered with daily life). Agreement, as measured by Cohen's kappa, was good or very good between the parents’responses and the qualitative measures provided by the medical professionals and the therapists, but only fair to moderate for the quantitative measures provided by the neuropsychologists. This may reflect different parental perceptions of the physical and cognitive aspects of outcome. Younger age at time of the stroke was the only significant predictor of adverse outcome.
Prevalence of oral health problems in a group of individuals with Down syndrome in France
Authors: Hennequin M , Allison P J , Veyrune J L
Published: July 2010

This cross‐sectional study describes oral health problems in a sample of children with Down syndrome (DS) compared with their siblings. Data were collected using the Oral Assessment ‐ Down Syndrome questionnaire, a validated French language evaluation to be completed by the child's carer. Parents attending a national meeting anonymously answered a questionnaire for their child with DS and for the sibling closest in age and returned their replies by post. Data concerning 204 individuals with DS (103 males, 101 females; mean age 9.6 years) and 161 of their siblings (80 males, 81 females; mean age 11.8 years) were returned. The association between DS status, age, and oral health was analysed. There was a greater frequency of oral health problems in those with DS with regard to function, clinical signs, disability, and development. The prevalence of chewing problems did not improve with age, the prevalence of bleeding gums and mouth breathing increased with age, and that of tongue protrusion reduced with age. Most parents rated the general and oral health of their children with DS highly, although worse than for their siblings.
Surveillance of cerebral palsy in Europe: a collaboration of cerebral palsy surveys and registers
Authors: Cans Christine
Published: July 2010

Although cerebral palsy (CP) is the most common cause of motor deficiency in young children, it occurs in only 2 to 3 per 1000 live births. In order to monitor prevalence rates, especially within subgroups (birthweight, clinical type), it is necessary to study large populations. A network of CP surveys and registers was formed in 14 centres in eight countries across Europe. Differences in prevalence rates of CP in the centres prior to any work on harmonization of data are reported. The subsequent process to standardize the definition of CP, inclusion/exclusion criteria, classification, and description of children with CP is outlined. The consensus that was reached on these issues will make it possible to monitor trends in CP rate, to provide a framework for collaborative research, and a basis for services planning among European countries.
ADHD in preschool children: parent‐rated psychosocial correlates
Authors: De Wolfe Nadine A , Byrne Joseph M , Bawden Harry N
Published: July 2010

Twenty‐five children (21 males, 4 females; mean age 4.8 years) with attention‐deficit‐hyperactivity disorder (ADHD) participated in this study in addition to 25 typically developing children (21 males, 4 females; mean age 4.9 years). Parental ratings of the preschool children and preschool child self‐ratings were examined within the framework of three domains: behavioral disturbance, social competence, and familial environment. Compared to their typically developing peers, preschool children classified as having ADHD were rated by their parents as significantly more aggressive, more demanding of parental time, less socially skilled, less adaptable to change in routine, and as exhibiting more non‐compliance. In contrast to these parental ratings, preschool children with ADHD perceived themselves as equally competent, and as socially accepted as their peers. Parents of preschool children with ADHD rated themselves as less competent parents, and as experiencing a restricted parenting role. Although parenting a preschool child with ADHD was viewed as stressful, the parents did not rate general family functioning to be adversely affected.
Reorganization of the primary somatosensory area in epilepsy associated with focal cortical dysplasia
Authors: Gondo Kenjiro , Kira Ryutaro , Tokunaga Yoichi , Harashima Chie , Tobimatsu Shozo , Yamamoto Tomoya , Hara Toshiro
Published: July 2010

A 5‐year‐old boy with focal cortical dysplasia was referred to our hospital because of epileptic seizures. He showed mild weakness of the left hand without sensory disturbance. Brain MRI revealed extensive cortical dysplasia with pachygyria and microgyria around the right central sulcus. On EEG examination, interictal spikes were noted over the right fronto/centro/parietal region. A 37‐channel magnetometer revealed that the sources of the spikes were in a small, restricted region of the normal frontal lobe adjacent to the dysplastic brain. Somatosensory evoked magnetic fields indicated that the location of the current source of N20 was in the same area. Our patient shows a unique case of plasticity and reorganization of the somatosensory function due to cortical dysplasia.
Regulation of stretch reflex threshold in elbow flexors in children with cerebral palsy: a new measure of spasticity
Authors: Jobin Annik , Levin Mindy F
Published: July 2010

The tonic stretch reflex threshold in children with cerebral palsy (CP) was measured to determine its test‐retest reliability and its concurrent validity as a potential measure of spasticity. Fourteen children with spastic CP aged 6 to 18 years were tested on three separate occasions for clinical spasticity and stretch reflex thresholds of affected elbow flexors. Electromyographic (EMG) recordings were obtained by surface electrodes for elbow flexors and extensors during mechanical displacements of the passive joint towards extension. Displacements were produced by a torque motor at seven velocities which randomly varied from trial to trial. EMG activity was measured in the stretched flexor muscles to determine threshold angles and velocities for each velocity of stretch. These were plotted on a velocity‐angle‐phase diagram and regression analysis was used to determine the static stretch reflex threshold for each participant. The measure showed good test‐retest reliability for the group (ICC 0.73,p<0.001) whereas a significant correlation between the measure and the clinical spasticity scale was not found. This technique is a potential outcome variable for measuring the efficacy of treatments aimed at decreasing spasticity in children with CP.
Sign language in childhood epileptic aphasia (Landau‐Kleffner syndrome)
Authors: Perez Eliane Roulet , Prélaz Anne‐Claude , Metz‐Lutz Marie‐Noëlle , Braem Penny Boyes , Deonna Thierry
Published: July 2010

Acquired epileptic aphasia (AEA, or Landau‐Kleffner syndrome) is a unique condition in which children can lose oral language (OL) comprehension and expression for a prolonged period. These children can benefit from visual forms of language, mainly sign language (SL), but the quality of SL has never been analyzed. The case is reported here of a boy with AEA who lost speech comprehension and expression from 3 years 6 months to 7 years and was educated in SL from the age of 6 years. His SL was evaluated at the age of 13 years and 6 months and compared with a control child with congenital sensorineural deafness. It was found that: (1) our patient achieved the same proficiency in SL as the control child with deafness; (2) SL learning did not compete with, but perhaps even hastened, the recovery of OL. Intact ability to learn a new linguistic code such as SL suggests that higher‐order language areas were preserved and received input from a separate visual route, as shown by neuropsychological and functional imaging research in deaf and hearing signers.
Sensory modulation dysfunction in children with attention‐deficit‐hyperactivity disorder
Authors: Mangeot Shanley Donelan , Miller Lucy Jane , McIntosh Daniel N , McGrath‐Clarke Jude , Simon Jody , Hagerman Randi J , Goldson Edward
Published: July 2010

This study investigates the presence of sensory modulation dysfunction (SMD) among children with attention‐deficit‐hyperactivity disorder (ADHD). Twenty‐six children with ADHD (mean age 8.3 years, 18 males, 8 females), and 30 typically developing children (mean age 8.2 years, 21 males, 9 females) were tested using a laboratory procedure that gauges responses to repeated sensory stimulation by measuring electrodermal reactivity (EDR). Parental report measures of limitations in sensory, emotional, and attentional dimensions were administered using the Short Sensory Profile, the Leiter International Performance Scale‐Revised, Parent Rating subscales, and the Child Behavior Checklist (CBCL). Compared to the typical sample, the children with ADHD displayed greater abnormalities in sensory modulation on both physiological and parent‐report measures. The children with ADHD also displayed more variability in responses. Within the group with ADHD, levels of SMD were highly correlated with measures of psychopathology on the CBCL. Implications of findings relate to the importance of considering sensory processing abilities in a subgroup of children with ADHD.
Developmental patterns of rhythmic suck and swallow in preterm infants
Authors: Gewolb Ira H , Vice Frank L , Schweitzer‐Kenney Erika L , Taciak Vicki L , Bosma James F
Published: July 2010

Twenty healthy preterm infants (gestational age 26 to 33 weeks, postmenstrual age [PMA] 32.1 to 39.6 weeks, postnatal age [PNA] 2.0 to 11.6 weeks) were studied weekly from initiation of bottle feeding until discharge, with simultaneous digital recordings of pharyngeal and nipple (teat) pressure and nasal thermistor and thoracic strain gauge readings. The percentage of sucks aggregated into‘runs’(defined as ≥ 3 sucks with ≤ 2 seconds between suck peaks) increased over time and correlated significantly with PMA (r=0.601, p<0.001). The length of the sucking‐runs also correlated significantly with PMA (r=0.613, p<0.001). The stability of sucking rhythm, defined as a function of the mean/SD of the suck interval, was also directly correlated with increasing PMA (r=0.503, p=0.002), as was increasing suck rate (r=0.379, p<0.03). None of these measures was correlated with PNA. Similarly, increasing PMA, but not PNA, correlated with a higher percentage of swallows in runs (r=0.364, p<0.03). Stability of swallow rhythm did not change significantly from 32 to 40 weeks’PMA. In low‐risk preterm infants, increasing PMA is correlated with a faster and more stable sucking rhythm and with increasing organization into longer suck and swallow runs. Stable swallow rhythm appears to be established earlier than suck rhythm. The fact that PMA is a better predictor than PNA of these patterns lends support to the concept that these patterns are innate rather than learned behaviors. Quantitative assessment of the stability of suck and swallow rhythms in preterm infants may allow prediction of subsequent feeding dysfunction as well as more general underlying neurological impairment. Knowledge of the normal ontogeny of the rhythms of suck and swallow may also enable us to differentiate immature (but normal) feeding patterns in preterm infants from dysmature (abnormal) patterns, allowing more appropriate intervention measures.
Development of bladder control in children and adolescents with cerebral palsy
Authors: Roijen LEG , Postema K , Limbeek VJ , Kuppevelt VH J M
Published: July 2010

The study aimed to investigate the development of bladder control in children with cerebral palsy (CP) and to determinate subgroups with deviant development of bladder control and a higher risk of not achieving urinary continence. Children and adolescents between the ages of 4 and 18 years with a diagnosis of CP, from six Dutch rehabilitation centres were included in the study (n=601). Parents of these participants were sent a questionnaire, of whom 76% responded. Prevalence of primary urinary incontinence was 23.5%. Survival curves were made to determine the development of achieving bladder control in CP. For participants who achieved urinary continence, the development was delayed in comparison with normally developing children and adolescents. The most important factors influencing the occurrence of urinary incontinence in CP were tetraplegia and low intellectual capacity. At age six, 54% of participants with spastic tetraplegia and 80% with spastic hemiplegia or diplegia gained urinary continence spontaneously. Of those who had low intellectual capacity, 38% were dry at this age.
Time costs of caring for children with severe disabilities compared with caring for children without disabilities
Authors: Curran A L , Sharples P M , White C , Knapp M
Published: July 2010

To assess time costs of caring for children with severe disabilities in the community compared to caring for children without disabilities, a diary‐ and questionnaire‐based study was carried out. Sixteen complete data sets were obtained from families with children who have disabilities (mean age 8.7 years) and 31 complete data sets from families with normally developing children (mean age 4.9 years). Diagnoses in the study group included cerebral palsy, autism, Sanfillipo syndrome, lissencephaly, and osteogenesis imperfecta. Items of personal care per waking hour were significantly greater in children with disabilities than non‐disabled children (p<0.001). In the study group, there was no correlation (r=‐0.12) between age and frequency of care whereas a significant correlation was observed between degree of disability as measured by the Functional Independence Measure for children (WeeFIM) and frequency of care items (r=0.89). Twelve of the 16 mothers in the study group were not in paid employment. Twelve had little or no extended family support. Benefits awarded did not correlate with the degree of disability as measured by the WeeFIM (r=‐0.11). Care needs of children with severe disabilities are significantly greater than those of non‐disabled children and do not decrease with advancing age. Mothers of children with disabilities are unable to work outside the home because of these care needs. This brings the family income, even when benefits are included, to a level that is less than peer families with non‐disabled children. A Functional Disability Score may help to achieve more appropriate allocation of state resources.
Influence of two different sitting positions on postural adjustments in children with spastic diplegia
Authors: Brogren Eva , Forssberg Hans , Hadders‐Algra Mijna
Published: July 2010

The present study addressed the question whether the deviant postural adjustments in children with spastic diplegia can be attributed to their crouched sitting position or primarily to their neural deficit. Postural adjustments during sitting in an erect and in a crouched position on a movable platform were assessed in 10 children, aged 3 to 7 years 6 months, with mild‐to‐severe forms of spastic diplegia and 10 age‐ and sex‐matched control children. Multiple surface EMGs of neck, trunk, and leg muscles and kinematics of head, body sway, and pelvis were recorded during forward and backward translations. The children with normal motor development showed a distinct adaptation of postural adjustments to sitting position. The children with cerebral palsy (CP) had a deficient adaptational capacity which was more pronounced in the erect than in the crouched position. Thus, the crouched sitting position did not induce postural deficiency but seemed to offer a solution to the sensory‐motor problem of the instability experienced. Children with severe diplegia exhibited a lack of direction specificity in the leg muscles during backward body sway, which points to a basic deficit in postural control. In addition, these children showed marked dysfunctions in the precise tuning of the postural adjustments to task‐specific conditions. In the children with mild‐to‐moderate forms of CP the basic level of control was intact.
Changes in rhythmic suckle feeding patterns in term infants in the first month of life
Authors: Qureshi Misbah A , Vice Frank L , Taciak Vicki L , Bosma James F , Gewolb Ira H
Published: July 2010

To quantify parameters of rhythmic suckle feeding in healthy term infants and to assess developmental changes during the first month of life, we recorded pharyngeal and nipple pressure in 16 infants at 1 to 4 days of age and again at 1 month. Over the first month of life in term infants, sucks and swallows become more rapid and increasingly organized into runs. Suck rate increased from 55/minute in the immediate postnatal period to 70/minute by the end of the first month (p<0.001). The percentage of sucks in runs of ≧3 increased from 72.7% (SD 12.8) to 87.9% (SD 9.1; p=0.001). Average length of suck runs also increased over the first month. Swallow rate increased slightly by the end of the first month, from about 46 to 50/minute (p=0.019), as did percentage of swallows in runs (76.8%, SD 14.9 versus 54.6%, SD 19.2;p=0.002). Efficiency of feeding, as measured by volume of nutrient per suck (0.17, SD 0.08 versus 0.30, SD 0.11cc/suck; p=0.008) and per swallow (0.23, SD 0.11 versus 0.44, SD 0.19 cc/swallow; p=0.002), almost doubled over the first month. The rhythmic stability of swallow‐swallow, suck‐suck, and suck‐swallow dyadic interval, quantified using the coefficient of variation of the interval, was similar at the two age points, indicating that rhythmic stability of suck and swallow, individually and interactively, appears to be established by term. Percentage of sucks and swallows in 1:1 ratios (dyads), decreased from 78.8% (SD 20.1) shortly after birth to 57.5% (SD 25.8) at 1 month of age (p=0.002), demonstrating that the predominant 1:1 ratio of suck to swallow is more variable at 1 month, with the addition of ratios of 2:1, 3:1, and so on, and suggesting that infants gain the ability to adjust feeding patterns to improve efficiency. Knowledge of normal development in term infants provides a gold standard against which rhythmic patterns in preterm and other high‐risk infants can be measured, and may allow earlier identification of infants at risk of neurodevelopmental delay and feeding disorders.
Epilepsy in fragile X syndrome
Authors: Berry‐Kravis Elizabeth
Published: July 2010

Epilepsy is reported to occur in 10 to 20% of individuals with fragile X syndrome (FXS). A frequent seizure/EEG pattern in FXS appears to resemble that of benign focal epilepsy of childhood (BFEC, benign rolandic epilepsy). To evaluate seizure frequency and type in a Chicago FXS cohort, data regarding potential seizure history were reviewed for 136 individuals with FXS (age range 2 to 51 years: 113 males and 23 females). Seizures occurred in 15 males (13.3%) and one female (4.3%): of these, 12 had partial seizures. EEG findings were available for 35 individuals (13 of 16 with seizures and 22 of 120 without seizures) and showed an epileptiform abnormality in 10 (77%) individuals with seizures and five (23%) individuals without seizures ‐ the most common epileptiform pattern being centrotemporal spikes. Seizures were easily controlled in 14 of the 16 individuals with seizures. Many individuals, including all with centrotemporal spikes, had remission of seizures in childhood. The most common seizure syndrome resembled BFEC and this pattern had the best prognosis for epilepsy remission. Deficiency of FMRP (fragile X mental retardation protein) appears to lead to increased neuronal excitability and susceptibility to epilepsy, but particularly seems to facilitate mechanisms leading to the BFEC pattern.
Systemic hypotension and white‐matter damage in preterm infants
Authors: Dammann Olaf , Allred Elizabeth N , Kuban Karl C K , van Marter Linda J , Pagano Marcello , Sanocka Ulana , Leviton Alan ,   for the Developmental Epidemiology Network. See Appendix II for committee members.
Published: July 2010

This study was designed to test the hypothesis that systemic hypotension during the first postnatal week increases the risk of ultrasonographic echolucency in the white matter of preterm infants (≦28 weeks’gestation) while adjusting for confounders. From a study base of 1607 very‐low‐birth weight neonates (500 to 1500 g), a subsample of 243 preterm infants (122 females; ≦28 weeks’gestation) was selected for echolucency and data collection prospectively for the entire first postnatal week. Data analyses were performed separately for the first 24 hours of life, for the interval from the end of the first 24 hours to the end of the fourth postnatal day, and for days 5, 6, and 7. Systemic hypotension was defined as the mean arterial blood pressure in the lowest quartile for the infant's week of gestational age. Protocol cranial ultrasounds were those obtained closest to days 1, 7, and 21. A committee of sonologists classified the infants as having either echolucency (echolucency group) or not (control group). Systemic hypotension during the first week of life appeared to be associated with echolucency in univariable analyses but the association did not persist after adjustment for potential confounders. Detailed summaries of 13 previous studies, the majority of which did not show an association between systemic hypotension and white‐matter damage, are presented. In sum, these results do not support the hypothesis that systemic hypotension contributes to echolucency among preterm infants.