bluuebunny/crossref_metadata_2025 huggingface dataset:
Visual‐spatial neglect in a child following sub‐cortical tumor resection
Authors: Billingsley Rebecca L , Lang Frederick F , Slopis John M , Schrimsher Gregory W , Ater Joann L , Moore Bartlett D
Published: July 2010

Although clinical syndromes of visual‐spatial neglect have been well described in adults, clinical features of neglect associated with subcortical dysfunction are infrequently reported in children and have not been described in detail. Unilateral visual‐spatial neglect in a 7‐year‐old male following removal of a right subcortical juvenile pilocytic astrocytoma is reported. Preoperative baseline neurocognitive assessment of the patient established intact attentional and intellectual functioning. Postoperatively visual‐spatial neglect was observed that was not accounted for by the patient's visual field deficit. Consistent with classic features of attentional neglect, increases in attentional demands led to greater errors in performance. The risk of unilateral neglect following resection of subcortical tumors that abut the thalamus or disrupt thalamo‐cortical projections was confirmed in a retrospective analysis of patients referred for neurocognitive testing at our site. It was concluded that the ventral thalamus may play a role in visual‐spatial attention early in development.
Prevalence of, and risk factors for, physical ill‐health in people with Prader‐Willi syndrome: a population‐based study
Authors: Butler J V , Whittington J E , Holland A J , Boer H , Clarke D , Webb T
Published: July 2010

The medical findings from a population‐based study of Prader‐Willi syndrome (PWS) are discussed (in which birth incidence of PWS was estimated at 1:22 000 and death rate at over 3% per annum). In this study the prevalence of specific medical disorders that might account for a shortened life expectancy were investigated. Of all people with a possible diagnosis of PWS, only those meeting clinical criteria and/or with a confirmed genetic diagnosis were included in the study. Sixty‐six individuals, 40 males and 26 females with a mean age of 19 years (range of 0 to 46 years) agreed to participate in the population‐based study group. A prevalence rate of 25% for non‐insulin dependent diabetes mellitus (NIDDM) was found in adults. Mean age at onset was 20 years. Those with NIDDM had a higher past maximum body weight and a greater likelihood of positive family history. Nearly 50% across the age groups reported a history of recurrent respiratory infections. High rates of fractures (29%), leg ulceration (22% in adults), sleep disorders (20%), and severe scoliosis (15% in childhood) were also reported. It is postulated that hypotonia is a possible contributory factor to the risk of strabismus, scoliosis, and respiratory infections. Other causes of morbidity, in particular the high rates of NIDDM, may be due to a failure to manage over‐eating resulting in severe obesity. Early diagnosis and clear guidance to families about these risks and how they might be prevented is recommended. It is hypothesized that the high pain threshold may result in the presence of some illness not being apparent.
The BREV neuropsychological test: Part II. Results of validation in children with epilepsy
Authors: Billard C , Motte J , Farmer M , Livet M O , Vallée L , Gillet P , Vol S
Published: July 2010

The Battery for Rapid Evaluation of Cognitive Functions (Batterie Rapide d'Evaluation des Fonctions Cognitives: BREV) is a quick test to screen children with higher‐functioning disorders and to define the patterns of their disorders. After standardization tests in 500 normally developing children aged 4 to 8 years, validation consisted of comparative evaluation of the specificity and sensitivity of the BREV with a wide reference battery in 202 children with epilepsy (108 males, 94 females; mean age 6 years 6 months, SD 1 year 8 months). Children were divided into 10 age groups from 4 to 8 years of age and represented eight epileptic syndromes. The reference battery included verbal and non‐verbal intelligence assessment using the Wechsler scale, oral language assessment with a French battery for oral language study, drawing with the Rey figure, verbal and visuo‐spatial memory with the McCarthy scale subtest and the Rey figure recall, and educational achievement with the Kaufman subtests. Every function evaluated with the BREV was significantly correlated with the reference battery testing a similar function (p=0.01 to 0.001). Specificity and sensitivity of the BREV verbal and non‐verbal scores were correlated with those of the Wechsler scale in more than 75% of children. The BREV, therefore, appears to be a reliable test which has been carefully standardized and validated and is valuable in screening for cognitive impairment in children.
Transient dystonias revisited: a comparative study of preterm and term children at 21/2 years of age
Authors: de Vries Alies M , de Groot Laila
Published: July 2010

Minor neurological dysfunctions (MND) have frequently been reported as an outcome of preterm birth. Behavioural and learning difficulties are a known feature, but coordination problems are especially described in preterm infants at later ages. All preterm infants in our study were born with a gestational age of <32 weeks and a birth weight of <1500 g. The aim of the study was to determine if in a normal clinical situation, children at risk for developing coordination problems could be detected by focusing particularly on their spontaneous, although elicited, motor performance. Forty‐two children of 2 years 6 months of age were studied of whom 25 were‘low‐risk’preterm and 17 were born at term. All children had been followed up since birth. In a structured, free‐field situation the quality of body rotation, spontaneous reaching‐out, and manipulation during parts of the Bayley Developmental Test were observed and videotaped. Body rotations were scored on a 2‐point scale, and arm and hand functions on a 3‐point scale. Most preterm infants showed non‐optimal body rotations and borderline or non‐optimal arm and hand functions in contrast to most term children. A clear connection was found between less optimal body rotations and poor arm and hand functions. Retrospectively, poor arm and hand functions at 2 years 6 months seemed to be related to those at 39 weeks. At the age of 39 weeks a clear relation had been found between poor postural control (many preterm infants could not sit independently) and earlier hyperextension of the trunk at 18 weeks and quality of arm‐hand functions. In the present study at 2 years 6 months a correlation with the former hyperextension could no longer be found, but there was a significant relation between poor trunk rotation and arm and hand function and the earlier arm and hand functions at 39 weeks.
Skin‐to‐skin contact (Kangaroo Care) accelerates autonomic and neurobehavioural maturation in preterm infants
Authors: Feldman Ruth , Eidelman Arthur I
Published: July 2010

The effects of mother‐infant skin‐to‐skin contact (Kangaroo Care; KC) on autonomic functioning, state regulation, and neurobehavioural status was examined in 70 preterm infants, half of whom received KC over 24.31 days (SD 7.24) for a total of 29.76 hours (SD 12.86). Infants were matched for sex (19 males and 16 females in each group); birth weight (KC, 1229.95g [SD 320.21]; controls, 1232.17g [SD 322.15]); gestational age (GA) (KC, 30.28 weeks [SD 2.54]; controls, 30.19 weeks [SD 2.65]); medical risk; and family demographics. Vagal tone was calculated from 10 minutes of heart rate before KC and again at 37 weeks’GA. Infant state was observed in 10‐second epochs during four consecutive hours before KC and again at 37 weeks’GA.

Neurobehavioural status was assessed at 37 weeks’GA with the Neonatal Behavioral Assessment Scale (NBAS). Infants receiving KC showed a more rapid maturation of vagal tone between 32 and 37 weeks’GA (p=0.029). More rapid improvement in state organization was observed in KC infants, in terms of longer periods of quiet sleep (p=0.016) and alert wakefulness (p=0.013) and shorter periods of active sleep (p=0.023). Neurodevelopmental profile was more mature for KC infants, particularly habituation (p=0.032) and orientation (p=0.007). Results underscore the role of early skin‐to‐skin contact in the maturation of the autonomic and circadian systems in preterm infants.
Peripheral neuropathies of infancy
Authors: Wilmshurst Jo M , Pollard John D , Nicholson Garth , Antony Jayne
Published: July 2010

Over a 33‐year period, 260 patients (<17 years of age; 119 males, 141 females) from New South Wales, Australia who had peripheral neuropathies confirmed by nerve biopsy, were studied. Of these, 50 infants presented with symptoms or signs of neuropathy under 1 year of age: including 24 patients with demyelinating neuropathies and 21 axonal neuropathies; a further five patients had spinal muscular atrophy with associated secondary sensory axonopathy. Nineteen infants had hereditary motor sensory neuropathy, of whom 13 had myelin protein mutations confirmed by molecular genetic studies. Peripheral neuropathy is not an unusual diagnosis in infancy. Awareness of this association will aid early diagnosis and prognosis as well as facilitate interventional patient management.
ADHD and epilepsy in childhood
Authors: Dunn David W , Austin Joan K , Harezlak Jaroslaw
Published: July 2010

Attention‐deficit‐hyperactivity disorder (ADHD) has been associated with childhood epilepsy; prevalence figures have ranged from 8 to 77%, depending on the sample studied and the criteria used for diagnosis. In the general population the prevalence of ADHD is approximately 5%, with the majority of affected children having ADHD combined type. As part of a larger study of behavioral problems in children with epilepsy, we assessed 175 children (90 males, 85 females; age range 9 to 14 years, mean age was 11 years 10 months, SD 1 year 8 months) for evidence of ADHD. The children had at least a 6‐month history of epilepsy. The primary caregiver completed both the Child Behavior Checklist (CBCL) and the Child Symptom Inventory‐4 (CSI) or Adolescent Symptom Inventory‐4 (ASI). On the CBCL, the mean attention problem T score was 64.6 (SD 10.5) for adolescents and 67.9 (SD 11.6) for children. On the CSI or ASI, 20 of 175 children met DSM‐IV criteria for ADHD combined type; 42 of 175 had ADHD predominantly inattentive type; and 4 of 175 met criteria for ADHD predominantly hyperactive‐impulsive type. There were significant correlations between the CBCL attention score and inattention (r=0.68) and hyperactivity‐impulsivity (r=0.59). Sex, seizure type, and focus of seizure discharge were not predictors of symptoms of ADHD. Children with epilepsy are at risk for symptoms of ADHD. They differ from other samples of children with ADHD by the higher proportion of children with ADHD predominantly inattentive type and by an equal male: female ratio.
A qualitative analysis of the benefits of strength training for young people with cerebral palsy
Authors: McBurney Helen , Taylor Nicholas F , Dodd Karen J , Graham H Kerr
Published: July 2010

This qualitative study investigated the positive and negative outcomes of a home‐based strength‐training programme for young people with cerebral palsy (CP). Eleven young people with spastic diplegic CP (seven females, four males; mean age 12 years 9 months, SD 2 years 10 months; range 8 to 18 years) and their parents were interviewed. Gross Motor Function Classification System scores ranged from I (walks without limitations) to III (walks with assistive device), with a mode of III. The strength‐training programme, which was conducted in the participants’homes three times per week for 6 weeks (total of 18 prescribed sessions), comprised three exercises targeting the major support muscles of the lower limbs. Exercises were bilateral half squats, heel raises, and step‐ups. The training load was increased by adding free weights to a backpack so that 8 to 10 repetitions of each exercise could be performed. Using thematic coding, three categories of outcome emerged: body function and structure, activity, and participation, which were influenced by environmental and personal contextual factors. The programme generated overwhelmingly positive outcomes with only minor negative responses about some equipment and the need for parental involvement. Benefits included perceptions that strength, flexibility, posture, walking, and the ability to negotiate steps had improved. In addition, participants reported psychological benefits such as a feeling of increased well‐being and improved participation in school and leisure activities. The contextual factors highlighted the fact that sufficient clinician resources must be allocated to solve individual exercise and equipment problems. As well as providing further evidence that strength training can be beneficial, this study provides useful indicators to guide future quantitative studies of outcomes that are meaningful for people with CP.
A3243G mitochondrial mutation associated with polymicrogyria
Authors: Keng WT , Pilz DT , Minns B , Patrick DR Fitz
Published: July 2010

The mitochondrial transfer ribonucleic acid for leucine is encoded by nucleotides 3230–3304. A‐to‐G transition at nucleotide 3243 can cause maternally transmitted diabetes mellitus‐deafness syndrome, and MELAS syndrome. MELAS syndrome is a rare disorder of mitochondrial energy production, and is an acronym for myopathy, encephalopathy, lactic acidosis, and stroke‐like episodes. Cortical malformations are heterogeneous and result from abnormal cell proliferation/apoptosis, migration, and/or differentiation of neuroepithelial cells. They are an important and relatively common cause of intractable epilepsy and neurodevelopmental disorders. The association between these A3243G mutations and cortical malformation has never before been reported. Here a 14‐year‐old female with A3243G mutation and polymicrogyria is described and possible aetiologies of this association are discussed.
Abnormal rhythmic motor response in children with attention‐deficit‐hyperactivity disorder
Authors: Ben‐Pazi Hilla , Gross‐Tsur Varda , Bergman Hagai , Shalev Ruth S
Published: July 2010

Children with attention‐deficit‐hyperactivity disorder (ADHD) have difficulties with motor control, inhibition of motor responses, motor flexibility, and motor preparedness. We proposed that motor abnormalities in ADHD might result, at least in part, from an abnormal neuronal oscillatory mechanism necessary for motor temporal regulation. The aim of this study was to assess pacing in children with ADHD, by testing for rhythmic abnormalities of motor activity using a tapping test. Twenty‐seven children (21 males, six females; aged 6 to 14 years 6 months; mean age 11 years 4 months, SD 2 years 2 months) diagnosed with ADHD according to DSM‐IV clinical criteria, and 33 controls (25 males, eight females; aged 6 to 14 years 6 months; mean 11 years 1 month, SD 2 years 2 months), underwent a finger‐tapping test requiring rhythmic responses to frequencies from 1 to 6Hz. All participants who were treated on a daily basis with methylphenidate (n=22) were medication‐free on the day of the test. Most of the children with ADHD responded at a constant rate regardless of stimulus frequency, a phenomenon only seen in a small number of the controls. This specific error pattern, also seen in Parkinson's disease, has been attributed to an abnormal oscillatory mechanism mediated by dopaminergic fronto‐striatal circuitry, which might also be pathophysiologically relevant for ADHD.
Acquired epileptic dysgraphia: a longitudinal study
Authors: Mayor Claire , Zesiger Pascal , Roulet Eliane , Maeder Malin , Deonna Thierry
Published: July 2010

A male presenting with benign partial epilepsy with rolandic spikes from the age of 7 years was evaluated at age 11 years for worsening of his epilepsy associated with a specific regression of graphomotor skills. A longitudinal study over nearly 2 years showed an improvement in handwriting to an almost normal level under modified antiepileptic therapy. A detailed analysis with a computer‐monitored graphics table showed at first a rapid improvement of skills followed by protracted slower progress. We argue that the initial rapid recovery of skills was directly linked to the improvement of his epilepsy. The slower late acquisition of motor programmes that had never been fully established was due to longstanding interference by his epilepsy. The specificity of the deficit within the graphomotor system and its possible neurobiological basis are also discussed. The analytical method and approach used in a single patient might provide an example for other patients in whom epilepsy can interfere in the acquisition, progress, and maintenance of new skills and can be responsible for selective deficits.
Opioid peptides and dipeptidyl peptidase in autism
Authors: Hunter L C , O'Hare A , Herron; W J , Fisher L A , Jones G E
Published: July 2010

It has been hypothesized that autism results from an‘opioid peptide excess'. The aims of this study were to (1) confirm the presence of opioid peptides in the urine of children with autism and (2) determine whether dipeptidyl peptidase IV (DPPIV/CD26) is defective in children with autism. Opioid peptides were not detected in either the urine of children with autism (10 children; nine males, one female; age range 2 years 6 months to 10 years 1 month) or their siblings (10 children; seven males, three females; age range 2 years 3 months to 12 years 7 months) using liquid chromatography‐ultraviolet‐mass spectrometric analysis (LC‐UV‐MS). Plasma from 11 normally developing adults (25 years 5 months to 55 years 5 months) was also tested. The amount and activity of DPPIV in the plasma were quantified by an ELISA and DPPIV enzyme assay respectively; DPPIV was not found to be defective. The percentage of mononuclear cells expressing DPPIV (as CD26) was determined by flow cytometry. Children with autism had a significantly lower percentage of cells expressing CD3 and CD26, suggesting that they had lower T‐cell numbers than their siblings. In conclusion, this study failed to replicate the findings of others and questions the validity of the opioid peptide excess theory for the cause of autism.
Cerebral palsy rates among low‐birthweight infants fell in the 1990s
Authors: Surman Geraldine , BEd Helen Newdick , Johnson Ann
Published: July 2010

Using a population‐based register, this study sought to ascertain changes in the rate and severity of cerebral palsy (CP) in a geographically defined area of the UK among infants weighing less than 1500g and born between 1984 and 1995. There were 417414 live births in the area, which included Berkshire, Buckinghamshire, Northamptonshire, and Oxfordshire. Of the 898 children with CP (526 males, 372 females), 194 (21.6%) weighed less than 1500g at birth. The overall CP rate for neonatal survivors fell from 2.5 out of every 1000 in 1984 to 1986 to 1.7 in 1993 to 1995. The rate for those weighing less than 1000g rose to 90 out of every 1000 neonatal survivors in 1987 to 1989 and then fell to 57 in 1993 to 1995. A similar pattern is seen among infants weighing 1000 to 1499g at birth, the rate rising to 77 in 1987 to 1988 and then falling to 40 in 1993 to 1995. The rate of severe motor disability among infants weighing less than 1500g also decreased (24.6 in 1984–1986 to 12.5 in 1993–1995). The relation of these findings to changes in perinatal care in the early 1990s is not known.
Late sequelae of low birth weight: mediators of poor school performance at 11 years
Authors: Weindrich Diana , PhD Christine Jennen‐Steinmetz , Laucht Manfred , Schmidt Martin H
Published: July 2010

This study examined the effect of low birth weight on school achievement and the mediating roles of cognitive and behavioural factors. The sample (115 females, 100 males) was selected from a longitudinal study of first‐born singleton children, born between 1986 and 1988 of German‐speaking parents, recruited from eight hospitals of the Rhine‐Neckar region in Germany. Twenty‐nine very‐low‐birth weight (VLBW; less than 1500g), 74 low‐birth weight (LBW; 1500 to 2500g) and 112 normal‐birth weight children (NBW; more than 2500g), all without severe neurological disability, were assessed at 11 years on cognitive, motor, scholastic, and behavioural measures. The scholastic measures included a school performance score and a teacher recommendation for type of secondary school the child should attend. LBW and VLBW children performed less well than the NBW group in all areas. LBW and VLBW groups remained at risk for school difficulties into late childhood, even when not neurologically impaired. Arithmetic, vocabulary, concentration, non‐verbal intelligence, and attention problems were significant mediators of the effect of low birth weight on the school performance score. Arithmetic, vocabulary, motor skills, and attention problems were found to be mediating factors of birth weight on teacher's recommendations. The poor outcome of the low birth weight children could not be attributed to further obstetric risk factors. Parents and pediatricians should be made aware of specific long‐term deficits of low birth weight children that may impair school performance, although they may be within a normal curriculum.
Mothers' decisions about gastrostomy tube insertion in children: factors contributing to uncertainty
Authors: Guerriere Denise N , McKeever Patricia , PhD Hilary Llewellyn‐Thomas , Berall Glenn
Published: July 2010

The purpose of this study was to explore O'Connor's four factors contributing to mothers’uncertainty concerning gastrostomy tube (G‐tube) insertion in their children (lack of information; unclear value trade‐offs; lack of support; social pressure) in a substitute decision‐making context. Fifty mothers participated in one semi‐structured interview at the time of their children's G‐tube insertion. Children's ages ranged from 2 weeks to 17 years, slightly more than half were male, and most had a primary diagnosis related to a neurological (n=27) or cardiac (n=10) condition. Two‐thirds of the mothers identified topics about which they wanted more information, the majority reported both gains and losses associated with their decision, three‐quarters reported that they had received support during decision making, and half reported that they had felt pressure from family and health care professionals. Results indicate that mothers’decisions about G‐tube insertion are complex and difficult. The existence and importance of O'Connor's factors in this context are confirmed by mothers’accounts. Because these factors are believed to be modifiable, health care professionals have the opportunity to potentially minimize the extent to which the factors contribute to decision uncertainly. It is recommended that health care professionals implement interventions focused on minimizing uncertainty.
Improved cognitive and motor development in a community‐based intervention of psychosocial stimulation in northeast Brazil
Authors: Eickmann Sophie H , V Ana C , Guerra Miriam Q , Lima Marilia C , C Pedro I , Huttly Sharon R A , Worth Ann Ash
Published: July 2010

The aim of the study was to compare the development of children with and without a programme of psychosocial stimulation in‘control’and‘intervention’sites in a poorly resourced area of northeast Brazil. The sample (n=156, born 1998) was from a larger cohort. The cohort was tested at 12 months (baseline) with the Bayley Scales of Infant Development. All children in the intervention site with a mental development index (MDI) and/or psychomotor development index (PDI) ≤100 were enrolled. Each time such a child was enrolled, the next child tested in that site of the same sex and with an index of 101 to 115 was also enrolled, and the next two children matched for sex and scores of A ≤100 and 101 to 115 in the control sites were recruited in parallel. The intervention comprised 14 contacts between 13 and 17 months of age. All children were tested again at age 18 months. The intervention and control groups were similar at baseline for a range of socioeconomic, demographic, environmental, and biological variables, and their MDI and PDI were also similar. At 18 months, the mean differences between the intervention and control groups were +9.4 points for MDI and +8.2 points for PDI (p<0.001 in each case). For children with an initial score of A ≤100, the mean difference between the intervened and control groups was +11.2 points for MDI (p<0.001), and +10.8 points for PDI (p=0.001). The intervention was thus associated with significant improvements in cognitive and motor development.
Stroke in the developing brain and intractable epilepsy: effect of timing on hippocampal sclerosis
Authors: Squier Waney , Salisbury Helen , Sisodiya Sanjay
Published: July 2010

A detailed study was made of the pathology of specimens removed by hemispherectomy for the treatment of intractable epilepsy in children with unilateral middle cerebral artery stroke. Neuropathological criteria were used to differentiate strokes that occurred in early intrauterine development (before 28 weeks gestational age) from those occurring in the last trimester, at birth, or after birth: 19 children had early strokes and 21 late. There was no difference in seizure history or occurrence of febrile convulsions in these two groups. Hippocampal tissue was available in 20 patients; pathology in the hippocampus, remote from the infarcted area, showed a marked difference between early‐onset and late‐onset groups. Hippocampal sclerosis was uncommon in children with early‐onset strokes but developed in most of the children whose strokes were of later origin. However, hippocampal sclerosis was more closely related to a clinical history of a late initial precipitating insult irrespective of infarct timing. These findings demonstrate the changing vulnerability of the developing brain and show that hippocampal pathology is more closely related to the timing of an insult than seizure history or the occurrence of febrile convulsions.
Patient with partial trisomy 9q and learning disability but no pyloric stenosis
Authors: Hengstschläger Markus , Prusa Andrea‐Romana , Repa Christa , Drahonsky Regina , Bernaschek Gerhard
Published: July 2010

Partial trisomy of the long arm of chromosome 9 represents a very rare and heterogeneous group of chromosomal aberrations. Associated clinical features include learning disability* and pyloric stenosis. We present the first patient to be reported with a duplication of the chromosome region 9q22.1 → q33. The patient (female, age 17 years) presented with growth retardation, microcephaly, facial dysmorphia, oesophageal atresia, aortic stenosis, ventricular septal defect, atrial septal defect II, hypothyroidism, and learning disability, but no pyloric stenosis. A review of all cases of partial trisomy 9q reported in the literature demonstrates that learning disability is a characteristic feature of this group of chromosomal aberrations. However, there are cases of duplications of the same chromosome 9 material, with and without pyloric stenosis. This study provides new information for future genetic counselling, especially in cases of prenatal diagnosis of partial trisomy 9q.
Clinical course of spontaneous spinal epidural haematoma mimicking Guillain‐Barré syndrome in a child: a case report and literature review
Authors: Çakir Ertuǧrul , Karaarslan Gökalp , Usul Haydar , Baykal Süleyman , Kuzeyli Kayhan , Mungan İlke , Yazar Uǧur , Peksoylu Bekircan , Aynaci Müjgan , Çakir Feraye
Published: July 2010

We describe a 9‐year‐old female with thoracic epidural haematoma. The clinical course simulated Guillain‐Barré syndrome (GBS) so intravenous immunoglobulin therapy was started at the paediatric clinic. Magnetic resonance imaging (MRI) 3 days after admission showed thoracic epidural haematoma between T2 and T8. An emergency laminectomy was performed and the patient's neurological symptoms began to improve immediately after surgery and she made a full recovery during the 2 weeks of follow‐up. Time is a very important factor in achieving reversibility of symptoms of compressive cord lesions, such as spinal epidural haematoma, and MRI is mandatory for patients with progressive paraplegia, even though the signs and symptoms might suggest GBS.
Specific cognitive deficits are common in children with Duchenne muscular dystrophy
Authors: Wicksell Rikard K , Kihlgren Margareta , Melin Lennart , Eeg‐Olofsson Orvar
Published: July 2010

A neuropsychological assessment was conducted to study cognition, with emphasis on memory, information processing/learning ability, and executive functions in boys with Duchenne muscular dystrophy (DMD). A group of 20 boys with DMD, aged 7 to 14 years (mean age 9 years 5 months, SD 2 years 2 months), was contrasted with 17 normally developing age‐matched comparison individuals, using specific neuropsychological tests (Block Span, Digit Span, Story Recall, Rey Auditory Verbal Learning Test, Rey Complex Figure Test, Spatial Learning Test, Verbal Fluency, Trail Making Test, Tower of London, Memory for Faces, and Raven's Coloured Progressive Matrices). The DMD group performed significantly worse on all aspects of memory, learning, and executive functions. There was no significant difference in general intellectual ability between the two groups. Analyses of group differences indicate that problems in short‐term memory are the most apparent, suggesting specific cognitive deficits. The differences between the groups were similar for both verbal‐auditory and visuospatial tests, thus contradicting the idea that cognitive deficits are related to type of stimulus presented. It is concluded from this study that short‐term memory deficits might play a critical role in the cognitive impairment and intellectual development seen in those with DMD.
Intraobserver reliability of the modified Tardieu scale in the upper limb of children with hemiplegia
Authors: Mackey Anna H , Walt Sharon E , Lobb Glenis , Stott N Susan
Published: July 2010

This study investigated the reliability of the modified Tardieu scale in the assessment of biceps spasticity in the upper limbs of children with hemiplegic cerebral palsy (CP). Ten children, with hemiplegic CP participated in the study: six males (mean age 9 years, SD 4 years) and four females (mean age 12 years, SD 3 years). Blinded, duplicate measures of dynamic elbow extension were performed on the hemiplegic arm at time 0 and 7 days later, using the three angular velocities described in the Tardieu scale (V1, slow; V2, speed of gravity; V3, as fast as possible). The resulting elbow joint angles were defined as R1, the angle of catch following a fast velocity stretch at either V2 or V3; and R2, the passive range of movement achieved following a slow velocity stretch at V1. Both elbow joint angle and movement angular velocity were measured by three‐dimensional kinematics. Median error in measured elbow joint angle within one session ranged from 3 to 5°. Between sessions median absolute differences in measured elbow joint angle ranged from 4 to 13°, with measurement errors of up to 25 to 30° in some participants at the fastest velocity (V3). The therapist was able to apply three significantly different angular velocities as required for the Tardieu scale (p < 0.001). However, the ranges of the three angular velocities overlapped, with fast velocities for some participants being equivalent to slow velocities for other participants. Three out of 10 participants had an intersessional difference in their R2‐R1 score of more than 20°. From this study, we concluded that the R2‐R1 value determined from the modified Tardieu scale may be of limited value in assessing biceps spasticity the upper limbs in children with hemiplegic CP.
Relevance of the Pediatric Powered Wheelchair Screening Test for children with cerebral palsy
Authors: Furumasu Jan , Guerette Paula , Tefft Donita
Published: July 2010

In a previous study we developed a cognitive assessment battery called the Pediatric Powered Wheelchair Screening Test (PPWST) to help clinicians determine a young child's readiness to drive a powered wheelchair. The current multicenter study sought to determine: (1) whether the PPWST is appropriate for use in a population of children with cerebral palsy (CP) who use joysticks to drive their wheelchair; (2) whether two additional factors (symbolic representation and coping) would increase the predictive power of the PPWST for this group and for children with orthopedic or neuromuscular disabilities only; and (3) whether the test was appropriate for children with severe motor impairments who use switches to control their wheelchair. Fifty children (27 males, 23 females) between the ages of 21 months and 6 years 11 months participated. Twenty‐six children (mean age 4 years 4 months, SD 15 months) had triplegic or tetraplegic CP and 24 children (mean age 27 months, SD 5 months) had orthopedic or neuromuscular disabilities. Sixty‐nine per cent of children had some limited form of mobility (such as rolling or scooting) but none was a functional ambulator. Each child was assessed with the PPWST and with measures of symbolic representation and coping. After six wheelchair training sessions, driving ability was scored. The PPWST was found to be predictive of functional driving ability for children with CP who used a joystick to control their wheelchair. Assessment of symbolic representation skills increased the predictive power for this group but not for children with orthopedic or neuromuscular disabilities; coping scores did not increase the predictive power for either group. The PPWST accounted for only 20% of the variance in overall driving skills for switch users, and thus is not yet considered an adequate screening device for this group. The PPWST is designed to help clinicians determine whether a child currently has the specific cognitive skills found to be related to powered wheelchair driving but is not intended to be used exclusively to determine whether or not a child is ultimately a candidate for powered mobility.
Coordination of suck‐ swallow‐respiration in infants born to mothers with drug‐abuse problems
Authors: Gewolb Ira H , Fishman Daniel , Qureshi Misbah A , Vice Frank L
Published: July 2010

To delineate quantitatively differences in suck‐swallow‐ respiration coordination during feeding in term infants exposed to drugs in utero, 16 control infants (10 females, six males; mean birthweight 3209g, standard error of the mean [SEM] 103g) and 15 drug‐exposed infants (seven females, eight males; seven cocaine, eight opiates; neonatal abstinence [Finnegan] scores less than 10; mean birthweight 3001g, SEM 93g) were studied in the first three days of life and again at one month of age. Coefficients of variation (COVs) of suck‐suck, swallow‐swallow, and breath‐breath intervals were used as measures of stability of individual rhythms. COVs of suck‐swallow and swallow‐breath intervals were used to determine stability of multiply integrated rhythms. In the first three days of life, a significantly higher percentage of‘apneic swallows’(runs of three or more swallows not associated with breathing movements, divided by total run‐swallows) was noted during feeding in the drug‐exposed infants (mean 5.3% SEM 1.7%) compared with controls (mean 0.9%, SEM 0.4%; p<0.02), who also had less breath‐breath rhythmic stability (higher COV) and shorter swallow‐breath intervals. These differences were no longer evident at one month of age. Opiate‐exposed infants were generally more affected than the cocaine‐exposed infants. Swallowing was less rhythmic in the drug‐exposed cohort, especially in opiate‐exposed infants, but this difference also disappeared by one month. In the first days of life, drug‐exposed infants were less efficient feeders, ingesting less volume per run‐swallow than controls (mean 0.31ml, SEM 0.05ml, versus 0.45ml, SEM 0.05ml; p<0.05). The decreased efficiency appeared to be compensated for by a slightly faster swallow rate (mean 53 swallows per minute SEM 2 versus 47 swallows per min, SEM 1, in controls; p<0.005). By one month of age these differences between groups had disappeared as well. Intrauterine drug exposure, even in infants with relatively mild symptoms of withdrawal, may adversely impact development of brainstem respiratory and swallow centers, thus affecting, albeit transiently, the underlying biorhythms of feeding.
Neuropsychology of academic and behavioural limitations in school‐age survivors of bacterial meningitis
Authors: Koomen Irene , Van Furth A Marceline , Kraak Miriam AC , Grobbee Diederick E , Roord John J , Jennekens‐Schinkel Aag
Published: July 2010

Neuropsychological impairments possibly underlying academic and/or behavioural limitations were studied in 149 school‐age survivors of bacterial meningitis, 68 with and 81 without academic and/or behavioural limitations. Academic limitations affected mathematics, reading, and writing. Behavioural limitations were inferred from scores in the clinical range on the Child Behaviour Checklist. These children had been selected from a cohort of 674 children (57% males) who had recovered from non‐Haemophilus influenzae type B bacterial meningitis and who had a mean age at infection of 2 years 4 months (range 1mo to 9y 5mo). They had neither‘complex onset’meningitis, prior cognitive or behavioural problems, nor severe disease sequelae. They were assessed with standardized assessment methods a mean of 7.8 years (range 4 to 10.4) after meningitis. Children with limitations (32% of the cohort) performed generically poorly on measures of cognitive functioning, speed, and motor steadiness, rather than having impairments in specific neuropsychological domains. The presence of two or more minor neurological signs was more frequent in the group with than in the group without limitations (30% versus 9%); this may explain the relatively poor speed and motor steadiness of the group with limitations.
Correlative value of magnetic resonance imaging for neurodevelopmental outcome in periventricular leukomalacia
Authors: Serdaroglu Gul , Tekgul Hasan , Kitis Omer , Serdaroglu Erkin , Gökben Sarenur
Published: July 2010

The aim of this study was to evaluate the correlative value of magnetic resonance imaging (MRI) in children with periventricular leukomalacia (PVL) for neurodevelopmental outcome. MRI examinations of 89 children (46 males, 43 females) with PVL (median age 4y, range 1 to 14y) were reevaluated. PVL was graded as follows: grade I, unilateral or bilateral areas of periventricular hyperintensity (1–3); grade II, hyperintensity more than 3; grade III, hyperintense lesions more than 3 and ventricular wall irregularity; grade IV, diffuse PVL and ventricular dilatation. Localizations of PVL and brain abnormalities associated with PVL were also noted. Assignment to PVL grades on MRI was as follows: PVL I (n=22), PVL II (n=18), PVL III (n=30), and PVL IV (n=19). Cerebral palsy was slightly less common in children with PVL I and II compared with PVL III to IV. Motor function was normal in 50% of children with PVL grade I, but severely impaired in 73.7% of children with PVL grade IV. Results of visual function were normal in all with PVL I, but pathological in 42.1% of patients with PVL IV. Developmental tests were appropriate for age in 75% of patients with PVL I, but significantly delayed in all patients with PVL IV. Thinning of the corpus callosum and presence of cortical atrophy were also correlated with neurological outcome. Significant risk factors associated with developmental delay were asphyxia at birth (odds ratio [OR] 4.3), PVL localization numbers over 3 (OR 4.4), PVL III to IV (OR 15), thinning of corpus callosum, and cortical atrophy.
Long‐term prognosis of cerebral venous sinus thrombosis in childhood
Authors: De Schryver Els LLM , Blom Ingrid , J Kees P , Kappelle L Jaap , Boudewyn A C , PhD Aag Jennekens‐Schinkel
Published: July 2010

Cerebral venous sinus thrombosis (CVST) is a rare but potentially serious disorder in children. There is no literature on the long‐term neuropsychological and emotional sequelae and implications for quality of life. We studied 17 children who had CVST after the neonatal period, aged between 1 month and 16 years at the time of CVST (mean age at CVST was 6 years, median 4 years 8 months). Five children died during follow‐up. The cause of death was related to CVST in one child. Twelve children participated in a clinical follow‐up assessment. Mean follow‐up was 2 years 8 months. One child had physical sequelae with impairment of skilled movement. All children had average or high intelligence scores. Two children with CVST due to an uncomplicated mastoiditis had mild cognitive deficits: one child had difficulty with written language; the other had diminished cognitive efficiency with concentration and attention problems associated with decreased psychosocial functioning. Decreased physical well‐being was reported in three of 12 children. We conclude that children who had survived CVST had a fair prognosis. Most had normal cognitive and physical development, although mild cognitive deficits or decreased physical and psychosocial well‐being can occur.
Children with rolandic epilepsy have abnormalities of oromotor and dichotic listening performance
Authors: Lundberg Staffan , Frylmark Astrid , Eeg‐Olofsson Orvar
Published: July 2010

Twenty children (11 females; age range 8y 2mo to 14y 7mo, mean 10y 6mo, SD 1y 8mo) with electroclinically typical rolandic epilepsy (RE), were investigated to demonstrate possible occurrence of abnormal oromotor functions and oral sensibility, linguistic problems, and impaired auditory discrimination. Twelve children were treated with an antiepileptic drug (carbamazepine, valproate, or sulthiame). They were compared with an age‐ and sex‐matched control group of 24 children (14 females; age range 8y 2mo to 14y 5mo, mean 11y, SD 1y 8mo). The test battery included tests for facial expressions, lip and tongue movements, repeated syllables, and articulation using nonsense and tongue‐twisting words. Sensibility on tongue and lips was tested with two‐point discrimination. As language tests, Rapid Confrontation Naming, orthographic, and phonologic decoding tests were used. A dichotic listening test was performed in 13 children with RE and in 14 controls. Compared to control children, those with RE had significantly greater problems concerning tongue movements (p<0.05) and articulation (nonsense words and tongue‐twisting words;p<0.01), and worse performance on dichotic listening (p<0.05). Oro‐lingual sensibility and results of language tests did not differ between those with RE and control children. The results indicate that children with RE have distinct but mild problems with oromotor performance and auditory discrimination.
Foreword
Authors: Baxter Peter
Published: July 2010

The abstracts for the EACD meeting this year to be held in November in Monaco have some changes.

The oral presentations and the poster presentations are run together. This is at the insistence of two of us (Professors Evrard and Tardieu) who feel strongly that people should recognize that a good poster may be a better way of presenting data than an oral presentation and for this reason we were keen not to discriminate between them. The abstracts are listed alphabetically and are numbered so that it is possible to trace an abstract by both methods.

The organizers this year had the largest number of abstracts we have ever had sent in for the meeting and some abstracts are not in this publication but will be available in a booklet at the meeting. For practical reasons, including deadline and finances, we had to limit the number of abstracts printed in this supplement of the journal DMCN. Those absent from this publication are those that arrived too late, those that did not fit exactly in the usual format of the EACD meetings and of this journal, and some that were submitted in languages other than English. The abstracts provided by invited speakers in the EACD meeting and in the satellite symposia will be available also in the booklet distributed at the meeting.

With this rich feast you have before you in this supplement remember there will be still many more abstracts when you get to Monaco.

Finally, remember that however carefully drafted they were, and however careful we were in selecting material for presentation, these are only abstracts. It is always preferable to wait until the definite paper appears before quoting results in the literature.

Our thanks to all our enthusiastic supporters of the EACD who have given us such a wealth of material to choose from.

Philippe Evrard, President of the EACD Monaco Conference 2005 Marc Tardieu, Vice‐President Christian Richelme, Secretary General Giovanni Cioni, Chair, EACD Scientific Committee Martin Box, Chair, EACD
Palatal tremor in childhood: clinical and therapeutic considerations
Authors: Campistol‐Plana J , MRCPCH A Majumdar , PhD E Fernández‐Alvarez
Published: July 2010

Palatal tremor (PT) is a rhythmic movement of the soft palate that often causes an ear click. PT can be symptomatic (SPT) or essential (EPT). The symptomatic form usually occurs in adults and the essential form mainly occurs in children. Several different treatments for EPT in children appear in the literature with variable reported efficacy. This report details four paediatric patients with EPT (three males, one female; mean age 6y 4mo [SD 6mo]; age at onset 6–7y) treated with piracetam (2‐oxo‐1‐pyrrolidine acetamide). Piracetam was used to treat EPT because of its antimyoclonic properties. All children showed a good response to doses of 100 to 300mg/kg/day. EPT relapsed on withdrawal of piracetam and remitted on reintroduction. Piracetam's effect on EPT was sustained. It is concluded that piracetam is an effective drug for the treatment of EPT in children.
The role of somatic threat feature detectors in the attentional bias toward pain: Effects of spatial attention
Authors: Dowman Robert
Published: July 2010

Our previous work suggests that somatic threat feature detectors indexed by a pain‐evoked midlatency negative scalp potential play an important role in the attentional bias toward pain. In these studies the somatic threat feature detectors facilitated the shift in attention to a somatic threat when attention was focused on another stimulus modality but not when it was focused on another spatial location. This experiment used the Posner cuing paradigm to investigate possible explanations for this discrepancy. The results demonstrate that the different somatic threat effects observed in previous modal and spatial cuing studies are not due to attentional set. Rather, this discrepancy may be related to differences in contingent attention capture. This study also verifies earlier findings suggesting that some of the pain‐related P2 component generators are involved in stimulus‐driven shifts in spatial attention toward task‐relevant stimuli.
The closely related transcription factors Sox4 and Sox11 function as survival factors during spinal cord development
Authors: Thein Daniela C. , Thalhammer Johannes M. , Hartwig Anna C. , Bryan Crenshaw III E. , Lefebvre Veronique , Wegner Michael , Sock Elisabeth
Published: July 2010

J. Neurochem. (2010) 115, 131–141.

Development of the mouse CNS was reported to be normal in the absence of either Sox4 or its close relative Sox11 despite strong and widespread expression of both transcription factors. In this study, we show that combined absence of both Sox proteins in the mouse leads to severe hypoplasia of the developing spinal cord. Proliferation of neuroepithelial precursor cells in the ventricular zone was unaffected. These cells also acquired their correct positional identity. Both glial and neuronal progenitors were generated and neurons appeared in a similar spatiotemporal pattern as in the wild‐type. Rates of cell death were however dramatically increased throughout embryogenesis in the double deficient spinal cord arguing that Sox4 and Sox11 are jointly and redundantly required for cell survival. The absence of pronounced proliferation, patterning, specification, and maturation defects furthermore indicates that the decreased cell survival is not a secondary effect of one of these events. We therefore conclude that the two Sox proteins directly function as pro‐survival factors during spinal cord development in neural cell types.