bluuebunny/crossref_metadata_2025 huggingface dataset:
Transient, idiopathic nystagmus in infants
Authors: Good William V , Hou Chuan , Carden Susan M
Published: July 2010

The aim of this study was to characterize children with transient nystagmus. Eleven children(six males, five females) developed nystagmus in infancy and then experienced regression of the problem, usually within a few months. Mean age at onset was 2.7 months, and mean age at regression was 8.5 months. No etiology could be ascertained in any of the patients, although four children had other eye or vision abnormalities (regressed retinopathy of prematurity,n=1; asymmetric fundus colobomata,n=1; delayed visual maturation,n=2). Results of this study suggest that mechanisms which allow ocular motor stability undergo a period of postnatal maturation, during which nystagmus can occur, but also during which nystagmus may disappear. Not every case of transient nystagmus should be categorized as spasmus nutans. There is a subset of infants and young children who develop transient nystagmus with no other findings and in whom the nystagmus disappears.
Prevalence of tic disorders and Tourette syndrome in a Swedish school population
Authors: Khalifa Najah , von Knorring Anne‐Liis
Published: July 2010

The aim of the study was to find the epidemiological distribution of tic disorders and Tourette syndrome (TS) in Swedish school children aged 7 to 15 years. A total population of 4479 children and their parents were asked to fill in a questionnaire covering both motor and vocal tics. A three‐stage procedure was used: screening, interview, and clinical investigation. Two hundred and ninety‐seven children (190 males, 107 females) were found to have tics. TS, according to DSM‐IV criteria, was found in 0.6% of the total population, another 0.8% had chronic motor tics, and 0.5% had chronic vocal tics. Further, 4.8% of the children had transient tics. All together 6.6% of 7‐ to 15‐year‐old children currently had or had experienced some kind tic disorder during the last year. Prevalence of different tic disorders was higher among younger children and in males, and was highly associated with school dysfunction. The prevalence of TS was higher than was previously thought but other tic disorders were more common in this childhood population.
Findings from a multidisciplinary clinical case series of females with Rett syndrome
Authors: Cass Hilary , Reilly Sheena , Owen Lucy , Wisbeach Alison , Weekes Lyn , Slonims Vicky , Wigram Tony , Charman Tony
Published: July 2010

Systematic data from a multidisciplinary clinical assessment of a large series of females with Rett syndrome (RS; n=87) is presented. Participants’ ages ranged from 2 years 1 month to 44 years 10 months. Areas assessed included oromotor skills, feeding problems, growth, breathing abnormalities, mobility, postural abnormalities and joint deformities, epilepsy, hand use and stereotypies, self‐care, and cognitive and communication skills. Many previously reported trends in the presentation of RS over time were confirmed, notably the increasingly poor growth and near pervasiveness of fixed joint deformities and scoliosis in adulthood. In contrast, there was a slight trend towards improved autonomic function in adulthood, whereas feeding difficulties increased into middle childhood and then reached a plateau. Improvements in mobility into adolescence were followed by a decline in those skills in adulthood. Levels of dependency were high, confirming findings from previous studies. Despite the presence of repetitive hand movements, a range of hand‐use skills was seen in individuals of all ages. Cognitive and communication skills were limited, but there was little evidence of deterioration of these abilities with age. These findings confirm that RS is not a degenerative condition and indicate that intervention and support to maintain and increase motor skills, daily living skills, and cognitive and communicative functioning are appropriate targets for individuals with RS.
Balint's syndrome in a 10‐year‐old male
Authors: Gillen Jennifer A , Dutton Gordon N
Published: July 2010

A 10‐year‐old male was referred with difficulties at school. He had particular difficulty with reading long words, following the sequence of text down a page, writing words in the correct order, writing words in line, and copying from the blackboard. He had a history of infective endocarditis complicated by intracerebral haemorrhage at the age of three years. Detailed history taking revealed symptoms typical of ‘dorsal stream’ pathology, namely a deficit of ‘vision for action’ . This included a spatial disorder of attention (simultanagnosia), defective hand and foot movements under visual control (optic ataxia), and acquired oculomotor apraxia which are consistent with Balint's syndrome. Strategies were suggested for coping with the symptoms and one year later a distinct improvement in adapting to the disability was found.
Complementary and alternative medicine use in families of children with cerebral palsy
Authors: Hurvitz Edward A , Leonard Christina , Ayyangar Rita , Nelson Virginia Simson
Published: July 2010

In order to assess patterns of usage of complementary and alternative medicine (CAM) in families of children with cerebral palsy (CP), 213 families with a child (0 to 18 years) with CP were recruited at the university medical center in Ann Arbor, MI, USA as part of a descriptive survey. Two hundred and thirty‐five surveys were distributed. Mean age of the child was 8 years 6 months (SD 4y: 9mo) and 56% of the sample was male with 35% full‐time independent ambulators, while the rest used an assistive device or a wheelchair. Fifty‐four percent were in special education classrooms. Families were given a survey on functional status of the child with CP, CAM usage of the child and the parent, factors influencing the decision to use CAM, demographics, and clinical information. Of the families, 56%, used one or more CAM techniques. Massage therapy (25%) and aquatherapy (25%) were the most common. Children of families that used CAM were significantly younger (7y: 9mo, SD 4y: 7mo) than non‐users (9y: 6mo, SD 4y: 6mo: t‐test p < 0.01 two‐tailed). Children with quadriplegic CP, with spasticity, and those who could not walk independently were more commonly exposed to CAM (Pearson's X2 [PX2] p=0.01 two‐tailed; for mobility, odds ratio [OR] of 2.5 with regression). Mothers with a college degree had a greater tendency to use CAM for their child than those without (PX2p=0.01 two‐tailed). Fathers of children who used CAM were older than fathers of those who did not (37y: 9mo versus 33y: 2mo, p=0.04 two‐tailed). There was no significant difference between groups for mother's age, father's education, income, or for population of home town. Parents who used CAM for themselves were more likely to try CAM for their child (70% versus 47%, OR 2.1), and were much more likely to be pleased with the outcome (71% versus 42%, OR 3.5). Child's age (younger), lack of independent mobility, and parental use of CAM were the most significant predictive factors identified via logistic regression.
Clinical and aetiological aspects of epilepsy in children with cerebral palsy
Authors: Carlsson Malin , Hagberg Gudrun , Olsson Ingrid
Published: July 2010

The aims of this retrospective and population‐based study were to describe the frequency and characteristics of epilepsy in 146 children (82 males and 64 females) with cerebral palsy (CP) born from 1987 to 1994 in the Göteborg area of Sweden. The frequency of epilepsy was found to be 38% (55 children). All children with tetraplegic CP and about one‐third of the children with other CP types developed epilepsy. Age at onset of epilepsy varied with type of CP: children with tetraplegic CP tended to have an earlier onset of epilepsy than children with other CP types. Partial seizures were the most common seizure type; all children with hemiplegic CP had partial seizures. Children with cognitive impairment had a higher frequency of epilepsy than those without cognitive impairment. CP aetiology may predict the development and outcome of epilepsy, as children with CP caused by CNS malformation, CNS infection, and grey matter damage all showed a higher frequency of epilepsy than children with CP of other aetiology, and also had less chance of becoming seizure‐free.
Long‐term risks and benefits of a separate CSF access device with ventriculoperitoneal shunting in childhood hydrocephalus
Authors: Lo T Y M , Myles L M , Minns R A
Published: July 2010

To determine the long‐term risks and benefits of a separate CSF reservoir in the management of 52 children (23 males, 29 females) with shunted hydrocephalus, a retrospective study was performed comparing the use and complications after separate reservoir insertion, with a prereservoir control period. Median age at first shunt insertion was 1 month and median age at reservoir insertion was 2 years 6 months. Median follow‐up for shunt with the additional reservoir was 19 years 1 month. There was no mortality due to shunt failure nor CNS infection, and there were significantly fewer episodes of ventriculitis (p<0.01) and shunt blockage (p<0.0001) compared with the prereservoir period. There was no hemiplegia, epilepsy, visual, nor cognitive loss from the additional reservoir. The reservoir was used for access in 344 attendances (mean 6.62 attendances per patient) for diagnosis or treatment of raised pressure or CNS infection. It was concluded that a separate CSF reservoir is useful in the long‐term management of patients with shunted hydrocephalus and is without mortality or significant increased morbidity.
Early recovery of walking in children and youths after traumatic brain injury
Authors: Haley Stephen M , Dumas Helene M , Rabin Jeffrey P , Ni Pengsheng
Published: July 2010

A consecutive series of 106 children and adolescents (mean age 10 years, 6 months; SD 4 years, 8 months) with recent traumatic brain injury admitted to a regional hospital‐based rehabilitation program was assessed to determine the rate of walking recovery, and characteristics that distinguish between independent walkers, non‐walkers, and device‐assisted walkers at hospital discharge. Data were collected through a retrospective medical record review of patients admitted between 1994 and 2001. Mean hospital stays were 66.7 days (SD 88.5, range 7 to 140 days). All children (72 male, 34 female) had recent injuries (from 1 to 8 weeks after onset of traumatic brain injury) and were independent walkers before injury. Sixty‐four children (60.4%) were discharged as independent walkers, 13 (12.3%) walked with the assistance of a device, and 29 (27.3%) were non‐walkers. Non‐walkers had a higher proportion of prolonged loss of consciousness, lower‐extremity injury, impaired responsiveness, and lower‐extremity spasticity than independent walkers. In addition, non‐walkers had poorer discharge mobility and social function scores, longer average hospital stays, and a greater proportion of non‐community discharges. Device‐only walkers were older, more likely to be male, and had a higher proportion of lower‐extremity injuries than independent walkers. Results highlight several demographic, clinical, and outcome variables that distinguish independent walkers from device‐assisted walkers and non‐walkers. These variables might help to determine the prognosis for ambulation, resource needs, and discharge plans for children and adolescents with traumatic brain injury after episodes of inpatient rehabilitation.
Life expectancy in pediatric patients with cerebral palsy and neuromuscular scoliosis who underwent spinal fusion
Authors: Tsirikos Athanasios I , Miller Freeman , Glutting Joseph
Published: July 2010

The aim of this study was to document the rate of survival among 288 severely affected pediatric patients (154 females, 134 males) with spasticity and neuromuscular scoliosis who underwent spinal fusion (mean age at surgery 13 years 11 months, SD 3 years 4 months), and to identify exposure variables that could significantly predict survival times. Kaplan‐Meier survival analysis was performed demonstrating a mean predicted survival of 11 years 2 months after spinal surgery for this group of globally involved children with cerebral palsy (CP). Cox's proportional hazards model was used to evaluate predictive efficacy of exposure variables, such as sex, age at surgery, level of ambulation, cognitive ability, degree of coronal and sagittal plane spinal deformity, intraoperative blood loss, surgical time, days in hospital, and days in the intensive care unit. Number of days in intensive care unit after surgery and the presence of severe preoperative thoracic hyperkyphosis were the only factors affecting survival rates. This demonstrated statistically significant predictability for decreased life expectancy after spinal fusion in children with CP.
Behavior and mental health problems in children with epilepsy and low IQ
Authors: Buelow Janice M , Austin Joan K , Dunn David W , Fastenau Philip S
Published: July 2010

The purpose of this cross‐sectional descriptive study was to describe the particular types of behavioral problems, self‐concept, and symptoms of depression experienced by children with both low IQ and epilepsy. Three groups of children (83 males, 81 females; mean age 11 years 10 months, SD 1 year 10 months; age range 9 to 14 years) with epilepsy were compared: (Group 1) Low IQ (<85), n=48, 25 males, 23 females; (Group 2) Middle IQ (85 to 100), n=58, 24 males, 34 females; and (Group 3) High IQ (>100), n=58, 34 males, 24 females. The Child Behavior Checklist, Piers‐Harris Self‐Concept Scale, and Children's Depression Inventory were used to measure behavior, self‐concept, and depression respectively. Results indicated that children in the Low IQ group had the most behavioral and mental health problems. Additionally, there were IQ group‐by‐sex interactions, with females in the Low IQ group being at the highest risk for poor self‐concept. Findings suggest that children with both epilepsy and low IQ should be carefully assessed for mental health problems in the clinical setting.
Attention‐deficit‐hyperactivity disorder with and without oppositional defiant disorder in 3‐ to 7‐year‐old children
Authors: PhD Christina Kadesjö , Hägglöf Bruno , Gillberg Christopher
Published: July 2010

Attention‐deficit‐hyperactivity disorder (ADHD) is a common neurodevelopmental disorder with a high degree of associated behavioural problems. In order to study characteristics of ADHD with and without oppositional defiant disorder (ODD) in a representative group of young children with clinical impairment in Sweden, 131 children (101 males, 30 females) with ADHD (mean age 5 years, SD 1 year 5 months; range 3 to 7 years) were clinically examined, and their parents interviewed. Independent questionnaire data (Child Behavior Checklist, ADHD Rating Scale‐IV, ODD Rating Scale‐IV, Conners Hyperactivity Index) were collected. For comparison 131 children without ADHD were matched for sex, age, marital status, and socioeconomic status (115 males, 16 females; mean age 4 years 10 months, SD 1 year 5 months; range 3 to 7 years). Sixty percent of those with ADHD met full DSM‐IV criteria for ODD. Only 10 of the 131 children with ADHD had no symptoms of ODD at all. The rate of children meeting full diagnostic criteria for ODD was similar across all age cohorts. Males were overrepresented in ODD, as were children of divorced parents and of mothers with low socioeconomic status. ADHD combined subtype was a stable independent factor influencing the diagnosis of ODD, regardless of psychosocial factors. Those with ADHD with ODD consistently showed higher rates of ADHD symptoms than did those with ADHD without ODD. The prevalent comorbidity of ADHD with ODD indicates that all children presenting with ADHD or ODD symptoms need to be assessed with a view to exploring both types of problem behaviours. The link between ODD and some psychosocial variables indicates the need to address these, possibly by measures such as parent training and network support.
Vertebral artery fibromuscular dysplasia: an unusual cause of stroke in a 3‐year‐old child
Authors: Camacho Ana , Villarejo Alberto , Moreno Teresa , MD Rogelio Simón , MD Alberto Muñoz , Mateos Fernando
Published: July 2010

Fibromuscular dysplasia (FMD) is a systemic arteriopathy which tends to affect renal arteries followed by cervicocranial vessels. It can lead to cerebral infarction if cephalic arteries are involved. FMD is an unusual cause of stroke in childhood that generally affects the carotid area. Only four cases of vertebral FMD and subsequent stroke have been reported previously and we present the youngest patient of all. A healthy 3‐year‐old female was admitted to Hospital Doce de Octubre in Madrid, Spain with cerebellar infarction. Angiography disclosed basilar artery thrombosis and typical signs of FMD in both vertebral arteries. No other angiographic alteration was noted in the other vessels studied. Her phenotype and other investigations were unremarkable. The patient was treated with anti‐aggregation therapy (aspirin) and the outcome was excellent. Investigation of the occurrence in childhood of this kind of arteriopathy may lead to clarification of its natural history and speculation about its unclear pathogenesis.
Learning of grasp control in children with hemiplegic cerebral palsy
Authors: Duff Susan V , Gordon Andrew M
Published: July 2010

This study examined whether children with hemiplegic cerebral palsy (CP) have anticipatory control of fingertip forces during lifts of familiar objects, and what type of practice (blocked or random) best enhances the retention of anticipatory control during lifts of novel objects. Eighteen children with hemiplegic CP (7 females, 11 males; 7 to 14 years of age, mean age 10 years, SD 1.8) and 18 age‐matched typically developing children (8 males, 10 females; mean age 10.4 years, SD 1.7) participated in the study. In the first experiment the children lifted familiar objects of various weights and sizes five times each, while the vertical lifting (load) force was measured. Most participants demonstrated higher rates of load force increase for heavier (and larger) objects already during the first lift, indicating anticipatory control. Furthermore, the load force rates generally were similar across the five lifts for each object, suggesting that they had stable representations of the objects’properties. In the second experiment children lifted three novel objects varying in weight (but identical in volume) 27 times each, in either a blocked or a random order, followed by nine immediate and nine delayed (24 hours) retention trials. Blocked practice resulted in greater differentiation of the force rates between objects during acquisition than did random practice. Both practice schedules resulted in similar retention. These findings suggest that children with hemiplegic CP have a priori internal representations used for anticipatory force scaling with familiar objects. Furthermore, the results indicate that these children can form and retain internal representations of novel objects for anticipatory control, irrespective of the type of practice schedule employed. Thus, clinically based practice sessions that incorporate lifts with novel objects may enhance anticipatory force scaling and related prehensile function in children with hemiplegic CP.
Conscious visual abilities in a patient with early bilateral occipital damage
Authors: Giaschi Deborah , Jan James E , Bjornson Bruce , Au Simon , MSc Matthew Tata , Lyons Christopher J , KH Peter
Published: July 2010

A 21‐year‐old male presented with occipital lobes that were extensively damaged by bilateral infarcts present at birth. The absence of the striate cortex was confirmed with anatomic and functional MRI and high‐resolution EEG. His cortical visual impairment was severe, but he retained a remarkable ability to see fast‐moving stimuli. Horizontal optokinetic nystagmus could be elicited from either eye. Resolution acuity was close to normal providing the patient was allowed to move his head and eyes. The direction of motion in random‐dot patterns could be discriminated with perfect accuracy at speeds above 2 deg/s, and the patient reported that he could 'see’the motion at fast but not at slow speeds. This conscious residual vision for motion is known as Riddoch's phenomenon, but it has never been reported in the complete absence of the striate cortex. Functional neuroimaging revealed activation that was outside the motion‐responsive regions of the extrastriate cortex. This case demonstrates remarkable plasticity in the human visual system and may have implications for understanding the functional organization of the motion pathways.
Stressful life events in pregnancy and head circumference at birth
Authors: Obel Carsten , Hedegaard Morten , Brink Henriksen Tine , Jørgen Niels
Published: July 2010

A strong association between stress in pregnancy and small head circumference in infants at birth was reported in 1994. This important finding has never been replicated. In a follow‐up study of 4211 participants with singleton pregnancies, information on life events was collected twice during pregnancy and head circumference measured shortly after birth following standard procedures. No association was found between experienced or perceived stress as a result of life events during pregnancy and head circumference in the infants. In conclusion, stress in pregnancy may influence foetal brain development in many ways, but we found no support for an effect on the size of the brain as measured by head circumference at birth.
Intracerebral varicella‐zoster virus reactivation in congenital varicella syndrome
Authors: Sauerbrei A , Pawlak J , Luger C , Wutzler P
Published: July 2010

Patients with congenital varicella syndrome (CVS) typically present with clinical symptoms consisting of skin lesions, neurological defects, eye diseases, and/or limb hypoplasia. In rare cases, isolated manifestations in the brain or eye have been reported. The varicella‐zoster virus (VZV), as the causative agent of CVS, could only be detected in a few infants with CVS. In addition, there is little in the literature on antiviral treatment of infants born with signs of CVS. We report a case of CVS in a male infant who presented with generalized clonic cerebral seizures at age 4 months. An endogenous intracerebral viral reactivation following intrauterine VZV infection was assumed. After the diagnosis was confirmed virologically, acyclovir was administered intravenously for 10 days and afterwards orally for 3 weeks. This antiviral treatment was aimed at preventing progression of the disease. We concluded from this case that infants with intrauterine VZV infection can suffer intracerebral VZV reactivations that require antiviral treatment.
Motor, cognitive, and behavioural disorders in children born very preterm
Authors: Foulder‐Hughes LA , Cooke RWI
Published: July 2010

Children born preterm have been shown to exhibit poor motor function and behaviour that is associated with school failure in the presence of average intelligence. A geographically determined cohort of two‐hundred and eighty preterm children (151 males, 129 females) born before 32 weeks’gestation and attending mainstream schools were examined at 7 to 8 years of age together with 210 (112 males, 98 females) age‐ and sex‐matched control participants were tested for motor, cognitive, and behavioural problems. Tests applied were the Movement Assessment Battery for Children (MABC), Clinical Observations of Motor and Postural Skills (COMPS), Developmental Test of Visual‐Motor Integration (VMI), Wechsler Intelligence Scale for Children, and Connors’Teacher Rating Scale for attention‐deficit‐hyper‐activity disorder (ADHD). Control children scored significantly better than the preterm group on all motor, cognitive, and behavioural measures. The lowest birthweight and most preterm individuals tended to score the lowest. Motor impairment was diagnosed in 86 (30.7%) of the preterm group and 14 (6.7%) of the control children using the MABC; 97 (42.7%) and 18 (10.2%) using the COMPS; and 68 (24.3%) and 17 (8.1%) respectively using the VMI. Each test of motor function identified different children with disability, although 23 preterm children were identified as having motor disability by all three tests. Preterm children were more likely to have signs of inattention and impulsivity and have a diagnosis of ADHD. Minor motor disabilities persist in survivors of preterm birth despite improvements in care and are not confined to the smallest or most preterm infants. They may exist independently of cognitive and behavioural deficits, although they often co‐exist. The condition is heterogeneous and may require more than one test to identify all children with potential learning problems.
Modes and patterns of self‐mutilation in persons with Lesch‐Nyhan disease
Authors: Robey Kenneth L , Reck John F , Giacomini Karen D , Barabas Gabor , Eddey Gary E
Published: July 2010

Lesch‐Nyhan disease (LND) is a rare X‐linked recessive genetic disorder associated with cognitive impairment, choreoathetosis, hyperuricemia, and the hallmark symptom of severe and involuntary self‐mutilation. This study examines data gathered from a survey of 64 families in the USA and abroad regarding the self‐injury of their family members who have LND. The individuals with LND ranged in age from 1 to 40 years (mean 16 years 7 months, SD 11 years 2 months) and, with the exception of one, were males. The most common initial mode of self‐mutilation, and the most frequently cited past or current behavior, was biting of lips and/or fingers. Other behaviors, in order of frequency, included head banging, extension of arms when being wheeled through doorways, tipping of wheelchairs, eye‐poking, fingers in wheelchair spokes, and rubbing behaviors. Hierarchical cluster analysis identified patterns of association among the types of self‐mutilation. Modes of self‐mutilation in which external surfaces (such as a wheelchair component) served as instruments of self‐injury tended to co‐occur, as did biting of lips and fingers.
Uptime normative values in children aged 8 to 15 years
Authors: Eldridge B , Galea M , McCoy A , Wolfe R , Graham H K
Published: July 2010

The‘Uptimer’is a custom‐made lightweight battery‐operated remote activity monitor that records the amount of time an individual spends in the upright position, which is also known as‘uptime’. The aims of this study were to determine levels of uptime over 24 hours and the relation between uptime and a child's age, sex, height, weight, and body mass index (BMI). Uptime was recorded in 529 normally developing children (318 females, 211 males), aged between 8 and 15 years. All children wore an Uptimer continuously for a 24‐hour period that included a typical school day. Mean uptime for children in this study was 5.4 hours (SD 1.3; range 1.5 to 10.3 hours), over a 24‐hour period. Uptime followed approximately a normal distribution in this population and did not have a linear relation to age, height, weight, nor BMI. Results of this study may be used as a normative database for the evaluation of uptime in children with physical disabilities.
Effect of muscle activity and botulinum toxin dilution volume on muscle paralysis
Authors: Sook Kim Hyeon , Hye Hwang Ji , Tak Jeong Soon , Taek Lee Yong , Lim Yeon , Sup Shim Jong
Published: July 2010

The purpose of this study was to evaluate the effects of botulinum toxin A (BTX‐A, Botox) dilution volume and post‐injection exercise with electrical stimulation on muscle paralysis. We injected 10 units of BTX‐A diluted with 0.1 ml (B1, n=8) or 0.5 ml (B5, n=8) normal saline into both gastrocnemius muscles of 16 New Zealand white rabbits; two controls received no BTX‐A. After BTX‐A injection, all rabbits received calf muscle stretching exercise and electrical stimulation for 2 hours on the left leg. The compound muscle action potential (CMAP) decrease was most pronounced at 1 week and progressive recovery was observed (i.e. recovery from paralysis, increase of CMAP). There was a significant decrease of CMAP amplitudes in the B5 group compared with the B1 group at week 1 and week 4 (p<0.001). Left limbs with stretching exercise and electrical stimulation showed lower CMAP amplitudes compared with control right limbs of all rabbits. To maximize the muscle paralysis effect of BTX‐A, increasing dilution volume and performing post‐injection stretching exercise with electrical stimulation may be a promising strategy for increasing the beneficial effect of BTX‐A treatment. Future studies are needed to investigate the clinical application of this finding.
Book Reviews
Authors: Jan J E , Wiggs Luci
Published: July 2010

Book reviewed in this article:

The Developing Visual Brain By Janette Atkinson Oxford University Press: Oxford Psychology Series No 32, First published 2000. First published in paperback 2002, pp 211

Awakening and Sleep‐Wake Cycle Across Development Edited by Piero Salzarulo and Gianluca Ficca Amsterdam, John Benjamins Publishing Company, 2002, pp 299
Development of hand function among children with cerebral palsy: growth curve analysis for ages 16 to 70 months
Authors: Hanna Steven E , Law Mary C , Rosenbaum Peter L , King Gillian A , Walter Stephen D , Pollock Nancy , Russell Dianne J
Published: July 2010

This study documents the development of hand and upper‐extremity function in young children who have cerebral palsy (CP) with upper‐extremity involvement using longitudinal data. Assessments of hand function and the quality of upper‐extremity movement were conducted on 29 males and 22 females (mean age 36.2 months, SD 10.6; age range 16 to 60 months at baseline) and on four other occasions over 10 months. Linear mixed effects modeling was used to estimate average developmental curves and the degree of individual differences in the patterns of development which were conditional on the body‐site distribution of CP and severity of impairments. Results indicate that hand function in this clinical population develops differently from overall upper‐extremity skills with declines in function in upper‐extremity skills being more common and pronounced among older children. However, there is substantial interindividual variation. Distribution of CP and severity of impairments were significant predictors of development. Results are discussed in terms of their clinical implications.
Early prognostic factors for intellectual outcome in CHARGE syndrome
Authors: Raqbi F , Bihan C Le , MD MP Morisseau‐Durand , Dureau P , Lyonnet S , Abadie V
Published: July 2010

CHARGE syndrome (coloboma, heart disease, atresia of the choanae, retarded growth and mental development, genital anomalies, and ear malformations and hearing loss) is a heterogeneous condition for which early prediction of intellectual outcome is important but difficult. The psychomotor milestones and intellectual outcome of a consecutive series of children with CHARGE syndrome who were observed by the same team from the neonatal period to the time of study were analyzed retrospectively. Twenty‐one children (11 males and 10 females, aged from 5 to 12 years, mean 8 years 7 months, SD 2 years 5 months) were included. The influence of 19 early identifiable parameters that could be considered as deleterious for intellectual outcome was recorded. Generally, the main psychomotor milestones (0 to 4 years) were severely delayed, although intellectual outcome (at primary‐school age) was satisfactory for half the children in this series. We show that extensive bilateral coloboma resulting in low vision, microcephaly, and brain malformation were the only three parameters that were predictive of poor intellectual outcome. Conversely, severe neonatal medical conditions, such as tracheotomy, conditions requiring long stays in hospital, or cardiac surgery were not predictive of poor intellectual outcome. Severe hearing loss was not found to be negatively correlated with intellectual outcome once coloboma had been taken into account.
Contributing factors to muscle weakness in children with cerebral palsy
Authors: Elder Geoffrey C B , BSc Geoff Stewart , PT Kathryn Cook , MSc Derek Weir , Marshall Arthur , Leahey Lorne
Published: July 2010

The aim of this study was to determine the extent of ankle muscle weakness in children with cerebral palsy (CP) and to identify potential causes. Maximal voluntary contractions of plantar (PF) and dorsiflexors (DF) were determined at optimal angles in knee flexion and extension in both legs of 14 children with hemiplegia (7 males, 7 females) and 14 with diplegia (8 males, 6 females). Their results were compared to 14 age‐ and weight‐matched control participants (5 males, 9 females). Muscle cross‐sectional areas of soleus, posterior, and anterior compartment muscles were determined from MRIs in 14 children with CP (eight diplegia, six hemiplegia) and 18 control children. Specific tension (torque/unit area) of PF and DF was determined from torque and cross‐sectional area results. Muscle volumes of PF and DF were also determined in both legs of five control children and five with hemiplegia. Muscle EMG was recorded from soleus, medial gastrocnemius, and tibialis anterior during each maximal voluntary contraction. Mean amplitude was significantly reduced in PF and DF in both CP groups and significantly higher levels of coactivation of antagonists were found compared to control participants. Strength of PF and DF was significantly reduced in both CP groups, but more importantly the muscles were found to be weak based on significantly reduced specific tensions. The PF were most affected, particularly in the group with hemiplegia. It is believed that an inability to maximally activate their muscles contributed to this weakness. A combination of incomplete activation and high levels of PF coactivation are thought to have contributed to DF weakness.
Prevalence of drooling in children with cerebral palsy attending special schools
Authors: Tahmassebi JF
Published: July 2010

Prevalence of drooling in children with cerebral palsy (CP) and its severity across dental age (i.e. children with primary, mixed, and permanent dentitions) was investigated. One hundred and sixty children and adolescents (91 males, 69 females; mean age 10 years 10 months, SD 4 years 2 months; range 4 to 18 years) with CP who attended special schools were involved in this study. Sixty eight per cent of participants had spastic CP with the majority (45%) diagnosed as having quadriplegia. Presence or absence of drooling was determined by direct observation of the participants. Other data, such as age, sex, learning disability*, and type of CP were collected using questionnaires. Results showed 93 of 160 children (58%) with CP had a drooling condition and of these 53 (33%) had severe drooling. It was found that the degree of drooling decreased as the child's dental age increased. Prevalence of drooling in children with primary dentition was found to be 75% as compared with 43% of those with permanent dentition, although this difference was not statistically significant. It was concluded that any invasive treatment to reduce drooling should, perhaps, be postponed until the child's oromusculature has matured.
Electrophysiological approach to the study of essential tremor in children and adolescents
Authors: Fusco C , MD J Valls‐Solé , MD E Fernández‐Alvarez
Published: July 2010

Surface electromyography and accelerometry provide essential information on the neurophysiological characteristics of essential tremor. There are many reports on neurophysiological features in adult‐onset essential tremor, but to our knowledge there have been no similar investigations of essential tremor in children. We conducted a neurophysiological study of nine children, six males and three females, with definite essential tremor. They were subdivided into two groups according to age: a‘children's group’, consisting of four patients aged from 7 to 12 years, and an‘adolescent group’, consisting of five patients aged from 14 to 16 years. Finger tremor as opposed to hand tremor was studied. In children the mean tremor frequency was 5.3Hz (SD 0.5) with arms extended, which increased to 8.2Hz (SD 1.5) when we added a mass of 300g. In adolescents the mean tremor frequency was 9.0Hz (SD 1.4) with arms extended, and 7.2Hz (SD 1.8) with added mass. We discuss several hypotheses to find an explanation for these results.
Arterial dissection complicating tonsillectomy
Authors: MRCPCH E Wraige , Ganesan V , London College
Published: July 2010

Tonsillectomy is a frequently performed surgical procedure. Surgical complications can be serious. We report the case of a 7–year‐old female who experienced an arterial ischaemic cerebral infarction following elective adenotonsillectomy.
Normal and abnormal development of the central nervous system II
Authors: GILLES FLOYD
Published: July 2010

In this brief review, I have summarized some of the ‘norma’ events as found in fetal autopsy material and depicted the prevalence of several specific pathological abnormalities as well as their risk factors.
Effects of very low birthweight on brain structure in adulthood
Authors: Allin Matthew , Henderson Max , Suckling John , Nosarti Chiara , Rushe Teresa , Fearon Paul , Stewart Ann L , Bullmore ET , Rifkin Larry , Murray Robin
Published: July 2010

Very‐low‐birth weight (VLBW) individuals are at high risk of brain injury in the perinatal period. We wished to determine how such early brain lesions affect brain structure in adulthood. Thirty‐two VLBW adults (20 female, 12 male) and 18 term, normal birthweight sibling control individuals (nine female, nine male) underwent structural MRI at a mean age of 23 years 4 months (range 17 to 33 years; SD 3.4). Images were analyzed using an automated tissue segmentation algorithm in order to estimate whole brain tissue class volumes in native space. Images were then warped to a template image in standard space. There was no significant between‐group difference in whole brain, grey matter, white matter, or total cerebral spinal fluid (CSF) volumes. However, lateral ventricular volume was significantly increased by 41% in those with VLBW. The ratio of grey to white matter was also significantly increased (by 10%) in those with VLBW. Group comparison maps showed widespread changes in the distribution of grey and white matter, and relative excess of ventricular CSF, in the brains of VLBW individuals. Increased ventricular volume predicted decreased grey matter in subcortical nuclei and limbic cortical structures, and decreased periventricular white matter. We conclude that these diffuse abnormalities of grey and white matter are a consequence of the interaction of perinatal brain injury and ongoing neurodevelopmental processes.
White matter alterations associated with chromosomal disorders
Authors: García‐Cazorla Angels , Sans Anna , Baquero Miguel , García‐Bargo María Dolores , Arellano Montse , Poo Pilar , Gean Esther , Campistol Jaume
Published: July 2010

White matter alterations in chromosomal disorders have been reported mainly in 18q‐syndrome. Our aim was to evaluate white matter alterations in patients with chromosomal abnormalities detected through conventional cytogenetic techniques. Forty‐four patients with chromosomal abnormalities, excluding trisomy 21, were diagnosed in our hospital between May 1999 and December 2002 (24 males, 20 females; mean age 6 years 4 months [SD 3 years 2 months], range 0 to 18 years). Of the 44 patients, 14 had brain magnetic resonance imaging (12 males, 2 females; mean age 4 years 2 months [SD 4 years 4 months]; five with sex chromosomal disorders [SCD] and nine with autosomal chromosomal disorders [ACD]). Of these 14 patients, eight (four with SCD and four with ACD) had abnormal white matter findings of similar patterns. These patients had pseudonodular, subcortical, and periventricular white matter high signal intensity images in T2, and fluid‐attenuated inversion recovery sequences that were isolated or confluent. The images did not correlate with the neurological clinical state. Given that eight of the 14 patients showed these lesions, their prevalence in different chromosomal abnormalities appears to be high, even though they have not been well reported in the literature. To our knowledge, these alterations have never been described in SCD. We concluded that unknown factors related to the myelination processes may be localized in different chromosomes.
Neuropathic bladder and intermittent catheterization: social and psychological impact on children and adolescents
Authors: Edwards Melinda , Borzyskowski †Malgorzata , Cox Antony , Badcock Janet
Published: July 2010

This paper describes part of larger study involving 40 families of children and adolescents with neuropathic bladder looking at the psychosocial impact of catheterization on them and their families. This study focuses on their experiences of clean intermittent catheterization (CIC) and self‐catheterization (SC). Twenty‐eight children and adolescents (11 males, 17 females; mean age 9 years, SD 2 years 7 months, range 5 to 20 years) with neuropathic bladder (mostly with spina bifida)participated in a semi‐structured interview to explore their understanding of micturition and catheterization, and their views about the introduction and practical use of this technique. Participants were invited to draw self‐portraits and figure diagrams to depict their understanding of CIC and SC. Age‐appropriate self‐esteem measures (Harter Pictorial Scale of Perceived Competence, the Self‐Perception Profile, and the Culture‐Free Self‐Esteem Inventory) were administered to see if catheterization status affected emotional well‐being. In an extension of this study, children's experiences of SC were explored by a postal questionnaire to all children successfully using SC who were attending the Paediatric Neuropathic Bladder Clinic. Children aged < 5 years, those with learning difficulties, and those using indwelling catheters were excluded. From a total sample of 66 (31 females, 35 males), 52 parents and 42 children and adolescents (28 females and 14 males) responded. No significant difference was observed in self‐esteem for those successfully catheterizing. Specific challenges involved learning SC and practical use of the technique. Concerns were leakage and being wet, and peers finding out about their continence management. Implications for the education, problem solving, and support of families and young people to promote cooperation and optimal benefits from catheterization are discussed.
ADHD: increased dopamine receptor availability linked to attention deficit and low neonatal cerebral blood flow
Authors: Lou Hans C , Rosa; Pedro , Pryds Ole , Karrebæk Hanne , Lunding Jytte , Cumming Paul , Gjedde Albert
Published: July 2010

Attention‐deficit‐hyperactivity disorder (ADHD), while largely thought to be a genetic disorder, has environmental factors that appear to contribute significantly to the aetiopathogenesis of the disorder. One such factor is pretern birth with vulnerable cerebrovascular homeostasis. We hypothesised that cerebral ischaemia at birth could contribute to persistent deficient dopaminergic neurotransmission, which is thought to be the pathophysiological basis of the disorder. We examined dopamine D2/3 receptor binding with positron emission tomography (PET) using [11C] raclopride as a tracer, and continuous reaction times (RT) with a computerized test of variables (TOVA) in six adolescents (12–14 years of age, one female) who had been examined with cerebral blood flow (CBF) measurements at preterm birth and had a subsequent history of attention deficit. We found that high dopamine receptor availability (‘empty receptors’) was linked with increased RT and RT variability, supporting the concept of a dopaminergic role in symptomatology. High dopamine receptor availability was predicted by low neonatal CBF, supporting the hypothesis of cerebral ischaemia as a contributing factor in infants susceptible to ADHD.
Identification and description of environmental factors that influence participation of children with cerebral palsy
Authors: Mihaylov Svetozar I , Jarvis Stephen N , Colver Allan F , Beresford Bryony
Published: July 2010

Physical, social, and attitudinal environment may restrict participation in children with cerebral palsy (CP). Here we discuss existing/possible approaches in order to identify and describe this environment. We used a critical review of evidence from the World Health Organization Literature Review on Environmental Factors; a search of electronic databases; and talked to specialists in order to find unpublished papers and‘grey’literature. Both children with disabilities and their parents identified a range of barrier and facilitator factors. These included psychosocial pressures (family, school), financial difficulties, and inadequate public services. Observational studies suggest that building structure, loss of income, and provision of specific equipment have a direct impact on levels of child participation. Some available instruments attempt to capture environmental factors by client survey or objective measurement; most relate to adult contexts, but there are a few child‐specific instruments for surveying attitudes of children to peers with disabilities and for observation of the school environment. Defining and measuring potential environmental determinants of participation for children with CP needs further development; and here we propose how this might be done.
Bodily pain and health‐related quality of life in children with cerebral palsy
Authors: Houlihan Christine M , O'Donnell Maureen , Conaway Mark , Stevenson Richard D
Published: July 2010

Pain frequency and its association with participation, function, and health‐related quality of life were evaluated in a sample of 198 children (58% male; mean age 10 years 7 months, SD 3 years 11 months; range 5 to 18 years) with moderate to severe cerebral palsy (CP). Gross Motor Functional Classification System (GMFCS) levels were III to V (23% level III, 23% level IV, 31% level V without a gastrostomy tube, and 23% were level V with a gastrostomy tube). Assessment was carried out using the parent‐report Child Health Questionnaire (CHQ) and questions assessing health, medical utilization, and medication use. Pain frequency ranged from‘none’to‘every day’. Eleven percent of parents reported pain almost every day (z score=‐0.38, p < 0.001). Pain was related to severity of motor impairment and the presence of a gastrostomy; parents of the most severely affected children reported the highest pain frequency (p=0.05). Pain was correlated with school days missed (p=0.03) and days in bed (p=0.01). Children taking gastrointestinal medications were reported to have greater pain frequency (p < 0.001). The Parental Impact ‐Emotional subscale of the CHQ was correlated with reported pain (r=0.38, p < 0.001). Pain was frequent in children with moderate or severe CP. Pain was more prevalent with more severe impairment and was associated with educational and social consequences.
Photosensitivity and epilepsy: a follow‐up study
Authors: Verrotti Alberto , Trotta Daniela , Salladini Carmela , Di Corcia Giovanna , Latini Giuseppe , Cutarella Rocco , Chiarelli Francesco
Published: July 2010

To understand the evolution of photosensitivity and to evaluate if its disappearance is related to the response to anticonvulsant therapy, we performed a long‐term study of 42 patients (17 males, 25 females; age at onset 6 years 9 months, SD 5 years 2 months, range 5 years to 12 years 1 month) who had electroencephalography (EEG) evidence of photosensitive epilepsy. Of the patients, 36 were treated with valproate (VPA) monotherapy and four received VPA in combination with other antiepileptic drugs (AEDs), which were carbamazepine and lamotrigine. Two patients were given no drugs, but treated with stimuli avoidance. All patients were investigated with EEG by using intermittent photic stimulation. The photoparoxysmal response indicated the presence of photosensitivity. At the end of follow‐up, the photoparoxysmal response had disappeared in 25 patients. Thirty‐three patients became seizure‐free. Our study confirms that photosensitive epilepsy has a good prognosis for seizure control that is independent of the persistence or disappearance of photosensitivity.