Showing papers in "Developmental Medicine & Child Neurology in 2001"

Brain dysmorphology in individuals with severe prenatal alcohol exposure.

Abstract: Our previous studies revealed abnormalities on structural MRI (sMRI) in small groups of children exposed to alcohol prenatally. Microcephaly, disproportionately reduced basal ganglia volume, and abnormalities of the cerebellar vermis and corpus callosum were demonstrated. The present study used sMRI to examine in detail the regional pattern of brain hypoplasia resulting from prenatal exposure to alcohol using a higher resolution imaging protocol and larger sample sizes than reported previously. Fourteen participants (mean 11.4 years; eight females, six males) with fetal alcohol syndrome (FAS) and 12 participants (mean 14.8 years; four females, eight males) with prenatal exposure to alcohol (PEA) but without the facial features of FAS were compared to a group of 41 control participants (mean 12.8 years, 20 females, 21 males). Findings of significant microcephaly and disproportionately reduced basal ganglia volumes in the FAS group were confirmed. Novel findings were that in FAS participants, white matter volumes were more affected than gray matter volumes in the cerebrum, and parietal lobes were more affected than temporal and occipital lobes. Among subcortical structures, in contrast to the disproportionate effects on caudate nucleus, the hippocampus was relatively preserved in FAS participants. Differences between the PEA group and controls were generally non-significant; however, among a few of the structures most affected in FAS participants, there was some evidence for volume reduction in PEA participants as well, specifically in basal ganglia and the parietal lobe. There were no group differences in cerebral volume asymmetries. Severe prenatal alcohol exposure appears to produce a specific pattern of brain hypoplasia.

Fetal valproate syndrome and autism: additional evidence of an association

Abstract: Autism has been described in association with a variety of medical and genetic conditions. We previously reported on a patient whose clinical phenotype was compatible with both fetal valproate syndrome (FVS) and autism. Here we present five additional patients with FVS and autism. In all five of our patients, there was evidence of cognitive deficits, manifestations of autism, and typical phenotypic characteristics of FVS. The association between this known teratogen and autism has both clinical and research implications.

The 2nd to 4th digit ratio and autism

Abstract: It has been suggested that autism may arise as the result of exposure to high concentrations of prenatal testosterone. There is evidence that the ratio of the lengths of the 2nd and 4th digit (2D:4D) may be negatively correlated with prenatal testosterone. We measured 2D:4D in 95 families recruited via the National Autistic Society, UK. The sample comprised a total 72 children with autism (62 males, 10 females; age range 2 to 14 years), including 23 children (20 males, three females) with Asperger syndrome (AS), 34 siblings, 88 fathers, 88 mothers and sex- and age-matched control participants. We found that the 2D:4D ratios of children with autism, their siblings, fathers and mothers were lower than population normative values. Children with AS, who share the social and communicative symptoms of autism but have normal or even high IQ, had higher 2D:4D ratios than children with autism but lower ratios than population normative values. There were positive associations between 2D:4D ratios of children with autism and the ratios of their relatives. Children with autism had lower than expected 2D:4D ratios and children with AS higher ratios than expected in relation to their fathers' 2D:4D ratio. It was concluded that 2D:4D ratio may be a possible marker for autism which could implicate prenatal testosterone in its aetiology.

Functional status of adults with cerebral palsy and implications for treatment of children

Abstract: This study examined the evolution of individuals with cerebral palsy (CP) from childhood to adulthood. Seventy-two adults with a diagnosis of CP born between 1934 and 1980 were studied. Individuals were recruited and data comprehensively collected using case notes and through direct assessments of the majority of participants from three rehabilitation units in Bologna, Padua, and Rovigo in Italy. The main findings can be summarized as follows: contact with health and rehabilitation services was radically reduced once individuals reached adulthood; more individuals who were integrated into mainstream schools achieved and maintained literacy than those who had attended special schools; in a high number of participants, motor performance deteriorated once into adulthood. Independent walking or other forms of supported locomotion were lost in many on reaching adulthood. Of those who continued to walk, walking deteriorated in terms of distance. It was concluded that even though CP has been considered as predominantly a childhood pathological condition, the evolution of the effects of CP do not stop at 16 or 18 years of age. For this reason, the traditional child- (or infant-) oriented approach concentrating mainly or exclusively on the achievement of independent walking, may not be an ideal approach to children with CP. Instead a more independence-oriented therapeutic approach would be appropriate.

Adults with cerebral palsy: a survey describing problems, needs, and resources, with special emphasis on locomotion

Abstract: The purpose of this study was to describe problems and resources of adults with cerebral palsy (CP) with special emphasis on locomotion. A questionnaire concerning demographic facts, locomotion, musculoskeletal problems, and present physical activity was mailed to 363 adults with CP. Two hundred and twenty-one adults, (125 male and 96 female; mean age 36 years, range 20 to 58 years) answered the questionnaire. Seventy-seven per cent reported problems with spasticity. Eighty-four per cent lived in their own apartments, with or without home services. Twenty-four per cent worked full-time and 18% had full disability pension. Twenty-seven per cent had never been able to walk, 64% could walk with or without walking aids, 35% reported decreased walking ability, and 9% had stopped walking. Eighty per cent reported contractures and 18% had pain every day. Approximately 60% were regularly physically active, and despite their disability, 54% considered that they were not limited in their ability to move about in the community.

Parent and teacher report of pragmatic aspects of communication: use of the children's communication checklist in a clinical setting.

Abstract: The Children's Communication Checklist (CCC) was developed to provide an objective assessment of pragmatic aspects of children's communication difficulties. We aimed to (1) see whether the checklist provided valid and reliable information when completed by parents, and (2) consider its usefulness in a clinical context. Checklists were completed by parents and a professional who knew the child well for all 5 to 17-year-old referrals to a tertiary developmental paediatrics centre over a 31-month period. Children who were not yet speaking in sentences were excluded. From a sample of 151 children (81% male; mean age 8.7 years) with pervasive or specific developmental disorders, valid checklists were completed by 119 parents and 93 professionals. Reliability, as measured by internal consistency, was 0.7 or higher for most scales. Correlations between ratings for parents and professionals were in the range of 0.30 to 0.58 for individual pragmatic scales, with a correlation of 0.46 (n=82) for the pragmatic composite. For both parents and professionals, the pragmatic composite was lowest for children with a diagnosis of autism; intermediate for those with a diagnosis of Asperger syndrome, pervasive developmental disorder not otherwise specified or attention-deficit-hyperactivity disorder (ADHD); and highest for those with a diagnosis of specific learning disability. The strongest relation between the pragmatic composite and diagnosis was seen when ratings from parents and professionals were combined. Differences between diagnostic groups were not explicable in terms of age or verbal IQ.

Effects of neurodevelopmental treatment (NDT) for cerebral palsy: an AACPDM evidence report

Abstract: The objective of the American Academy for Cerebral Palsy and Developmental Medicine (AACPDM) evidence reports is to provide the biomedical research and clinical practice communities with the current state of evidence about various interventions for the management of developmental disabilities. AACPDM evidence reports aggregate all that has been published about outcomes of an intervention for a medical condition, gauge the credibility (i.e. strength of the internal validity) of that evidence, and identify gaps in our scientific knowledge. The original version of this report was published in the ‘AACPDM Database of Evidence Reports’ on the internet (www.aacpdm.org) where evidence reports are regularly updated to include new research.

Sensory modulation dysfunction in children with attention-deficit–hyperactivity disorder

Abstract: This study investigates the presence of sensory modulation dysfunction (SMD) among children with attention-deficit-hyperactivity disorder (ADHD). Twenty-six children with ADHD (mean age 8.3 years, 18 males, 8 females), and 30 typically developing children (mean age 8.2 years, 21 males, 9 females) were tested using a laboratory procedure that gauges responses to repeated sensory stimulation by measuring electrodermal reactivity (EDR). Parental report measures of limitations in sensory, emotional, and attentional dimensions were administered using the Short Sensory Profile, the Leiter International Performance Scale-Revised, Parent Rating subscales, and the Child Behavior Checklist (CBCL). Compared to the typical sample, the children with ADHD displayed greater abnormalities in sensory modulation on both physiological and parent-report measures. The children with ADHD also displayed more variability in responses. Within the group with ADHD, levels of SMD were highly correlated with measures of psychopathology on the CBCL. Implications of findings relate to the importance of considering sensory processing abilities in a subgroup of children with ADHD.

Neuropsychological disorders related to interictal epileptic discharges during sleep in benign epilepsy of childhood with centrotemporal or Rolandic spikes

Abstract: Nine children (five males, four females; age range 6 years 1 month to 11 years 1 month) affected by benign epilepsy of childhood with centrotemporal or Rolandic spikes (BECRS) with EEG evidence of marked activation of interictal epileptic discharges (IEDs) during sleep, and nine unaffected control children matched for age, sex, and socioeconomic status, were enrolled in a prospective study. At the time of detection of IED activation during sleep, patients showed a mean Full-Scale IQ score within the normal range, but significantly below that of control participants; neuropsychological assessment revealed disorders in visuospatial short-term memory (Corsi's Block Tapping Test), attention, and cognitive flexibility (Trail Making Test and Stroop Color-Word Test), picture naming, and fluency (Benton's Naming Test and Word Fluency), visuoperceptual skill (Ghent-Poppelreuter and Street Gestalt Completion Tests) and visuomotor coordination (Bender Test). After detection of IED activation during sleep, children were followed up for 2 years. At the time of IED remission (T1), neuropsychological re-evaluation showed a notable increase in IQ score and a significant improvement (t-test: p<0.007) in visuomotor coordination, non-verbal short-term memory, sustained attention and mental flexibility, picture naming, and visual-perceptual performance. At T1, patients' performance did not differ from the controls (Mann-Whitney U test).

Collagen accumulation in muscles of children with cerebral palsy and correlation with severity of spasticity.

Abstract: Muscle function often becomes progressively more compromised in children with spastic cerebral palsy, leading to reduced mobility. This study aimed to examine the role that muscle connective tissue plays in this process. Severity of spasticity as determined by a range of clinical measures was assessed in 26 children (14 males 12 females; age range 4 to 17 years) with either diplegic or quadriplegic cerebral palsy (CP). Muscle biopsies from the vastus laeralis muscle were obtained for biomedical and histological analysis during orthopaedic surgery as part of the child's ongoing care. Total collagen was quantified by hydroxyproline determination. Two clinical measures of severity, Modified Ashworth Scale and Balance, were shown to have a highly significant correlation with collagen content, and Ambulatory Status, Clonus, and Selective Muscle Control all showed positive trends. Collagen I accumulated in spastic muscle's endomysium which appeared to be thickened, and fibrotic regions with sparse muscle fibres were evident in more severe cases. This suggests that collagen may be involved in increases in muscle stiffness observed in spasticity. Once developed, these changes are essentially irreversible and we suggest that future treatments should consider including prevention of muscle fibrosis.

Health status of children with moderate to severe cerebral palsy.

Abstract: The aim of the study was to evaluate the health of children with cerebral palsy (CP) using a global assessment of quality of life, condition-specific measures, and assessments of health care use. A multicenter population-based cross-sectional survey of 235 children, aged 2 to 18 years, with moderate to severe impairment, was carried out using Gross Motor Function Classification System (GMFCS) levels III (n = 56), IV (n = 55), and V (n = 122). This study group scored significantly below the mean on the Child Health Questionnaire (CHQ) for Pain, General Health, Physical Functioning, and Impact on Parents. These children used more medications than children without CP from a national sample. Fifty-nine children used feeding tubes. Children in GMFCS level V who used a feeding tube had the lowest estimate of mental age, required the most health care resources, used the most medications, had the most respiratory problems, and had the lowest Global Health scores. Children with the most severe motor disability who have feeding tubes are an especially frail group who require numerous health-related resources and treatments. Also, there is a relationship among measures of health status such as the CHQ, functional abilities, use of resources, and mental age, but each appears to measure different aspects of health and well-being and should be used in combination to reflect children's overall health status.

Reliability of the Melbourne assessment of unilateral upper limb function.

Abstract: This study examines the reliability of the Melbourne Assessment of Unilateral Upper Limb Function: a quantitative test of quality of movement in children with neurological impairment. The assessment was administered to 20 children aged from 5 to 16 years (mean age 9 years 10 months, SD 2 years 10 months) who had various types and degrees of cerebral palsy (CP). The performances of the 20 children during assessment were videotaped for subsequent scoring by 15 occupational therapists. Scores were analyzed for internal consistency of test items, inter- and intrarater reliability of scorings of the same videotapes, and test-retest reliability using repeat videotaping. Results revealed very high internal consistency of test items (alpha=0.96), moderate to high agreement both within and between raters for all test items (intraclass correlations of at least 0.7) apart from item 16 (hand to mouth and down), and high interrater reliability (0.95) and intrarater reliability (0.97) for total test scores. Test-retest results revealed moderate to high intrarater reliability for item totals (mean of 0.83 and 0.79) for each rater and high reliability for test totals (0.98 and 0.97). These findings indicate that the Melbourne Assessment of Unilateral Upper Limb Function is a reliable tool for measuring the quality of unilateral upper-limb movement in children with CP.

Developmental patterns of rhythmic suck and swallow in preterm infants.

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

Intrathecal baclofen for generalized dystonia

Abstract: The aim of this study was to evaluate the effects of intrathecal bacolfen (ITB) on patients with severe generalized dystonia. Eighty-six participants ranging in age from 3 to 42 years (median age 13 years) with generalized dystonia refractory to oral medications were offered treatment with ITB. Dystonia was associated with cerebral palsy in 71% of participants. Response to ITB was tested by continuous infusions in 72%, and by bolus injections in 17% of participants who had both dystonia and spasticity. Ninety-one percent of participants responded to the screening infusion and 93% to the bolus injections. Pumps were implanted in 77 participants. Dystonia scores at 3, 6, 12, and 24 months were significantly decreased (p<0.005) compared with baseline scores. Dystonia scores were significantly lower in those with intrathecal catheters positioned at T4, or higher than in those with catheters at T6 or lower (p=0.005). Ninety-two percent of participants implanted with a pump retained their responses to ITB during a median follow-up of 29 months. Patient questionnaires indicated that quality of life and ease of care improved in 86% and speech improved in 33%. Side effects of ITB occurred in 26% of participants. Surgical complications occurred in 38% and included CSF leaks, infections, and catheter problems. ITB is probably the treatment of choice for generalized dystonia if oral medications are ineffective.

Developmental outcome of school-age children born to mothers with heroin dependency: importance of environmental factors.

Abstract: Development of children aged 5 to 12 years born to mothers with heroin dependency raised at home or adopted was studied in comparison with: (1) children with environmental deprivation alone (i.e. low parental socioeconomic status [SES] and evidence of neglect), (2) children born to fathers with heroin dependency fathers, and (3) control individuals of average SES. One hundred and sixty children (84 males and 76 females; average age at examination 8 years) were evaluated between 1998 and 1999. All were attending mainstream schools. All participants were examined by a paediatrician and a psychologist using standard neurological and psychological age-appropriate tests, as well as tests and questionnaires to assess learning ability and attention span. The Conners and Achenbach questionnaires and the Pollack Taper test were used to assess possible presence of attention-deficit-hyperactivity disorder (ADHD). Mothers were assessed for ADHD using Wender's questionnaire. Children born to parents with heroin dependency raised at home and those of low SES exhibited intellectual impairment both on verbal and performance skills. They also had impaired reading and arithmetic skills. Children born to mothers with heroin dependency but who were adopted at a young age had normal intellectual and learning abilities, except for some reduced function on the performance Wechsler Intelligence Scale for Children-Revised. We found a high rate of ADHD among all children born to parents with heroin dependency, including those adopted, as well as in children with low parental SES. The highest rate of ADHD was in children born to mothers with heroin dependency raised at home, being twice that observed in the other groups. Mothers of these groups of children also had a high rate of ADHD.

Randomized controlled trial of physiotherapy in 56 children with cerebral palsy followed for 18 months.

Abstract: This study aimed to determine whether motor function and performance is better enhanced by intensive physiotherapy or collaborative goal-setting in children with cerebral palsy (CP). Participants were a convenience sample of 56 children with bilateral CP classified at level III or below on the Gross Motor Function Classification System (GMFCS), aged between 3 and 12 years. A 2 x 2 factorial design was used to compare the effects of routine amounts of physiotherapy with intensive amounts, and to compare the use of generalized aims set by the child's physiotherapist with the use of specific, measurable goals negotiated by the child's physiotherapist with each child, carer, and teacher. Following the six-month treatment period there was a further six-month period of observation. Changes in motor function and performance were assessed by a masked assessor using the Gross Motor Function Measure (GMFM) and the Gross Motor Performance Measure (GMPM) at three-month intervals. There was no statistically significant difference in the scores achieved between intensive and routine amounts of therapy or between aim-directed and goal-directed therapy in either function or performance. Inclusion of additional covariates of age and severity levels showed a trend towards a statistically significant difference in children receiving intensive therapy during the treatment period. This advantage declined over the subsequent six months during which therapy had reverted to its usual amount. Differences in goal-setting procedures did not produce any detectable effect on the acquisition of gross motor function or performance.

Intelligence and Duchenne muscular dystrophy: full-scale, verbal, and performance intelligence quotients.

Abstract: Duchenne muscular dystrophy (DMD) is a disease which has as its hallmarks progressive muscle weakness and degeneration of skeletal muscle. Also observed among those with DMD is a higher rate of mental retardation than in the normally developing population.

Architecture of the medial gastrocnemius in children with spastic diplegia.

Abstract: Ultrasound images were obtained of the medial gastrocnemius at different ankle joint positions with the knee extended. Fascicle length and deep fascicle angle were measured in five normally developing adults (mean age 33 years, age range 24 to 36 years) and in five normally developing children (mean age 7.8 years, age range 7 to 11 years), and in seven children with spastic diplegia (mean age 10 years, age range 6 to 13 years). These architectural variables were similar in the groups of normally developing adults and children. Importantly, no statistical difference could be found between the normally developing children and those with diplegia for fascicle length. Deep fascicle angles were reduced significantly in the clinical group at a particular ankle joint angle but not at the resting angles. The difference in deep fascicle angles is explained as a function of resting muscle length and is not attributed any clinical importance. Our results do not explain the structural origin of muscle contracture explicitly. However, they do indicate that most of the fixed shortness in the medial gastrocnemii of ambulant children with spastic diplegia is not due to reduced muscle fascicle length. We suggest that muscle contracture may be better explained in terms of shortness of the aponeuroses of pennate muscles, such as the medial gastrocnemius, through reduced muscle fascicle diameter.

Neuromotor development from 5 to 18 years. Part 1: timed performance.

Abstract: Timed performance in specific motor tasks is an essential component of a neurological examination applied to children with motor dysfunctions. This article provides centile curves describing normal developmental course and interindividual variation of timed performances of non-disabled children from 5 to 18 years. In a cross-sectional study (n=662) the following motor tasks were investigated: repetitive finger movements, hand and foot movements, alternating hand and foot movements, sequential finger movements, pegboard, and dynamic and static balance. Intraobserver, interobserver, and test-retest reliability for timed measurements were moderate to high. Timed performances improved throughout the entire prepubertal period, but differed among various motor tasks with respect to increase in speed and when the 'adolescent plateau' was reached. Centile curves of timed performance displayed large interindividual variation for all motor tasks. At no age were clinically relevant sex differences noted, nor did socioeconomic status significantly correlate with timed performance. Our results demonstrate that timed motor performances between 5 and 18 years are characterized by a long-lasting developmental change and a large interindividual variation. Therefore, a well standardized test instrument, and age-specific standards for motor performances are necessary preconditions for a reliable assessment of motor competence in school-age children.

General movement assessment as a method of developmental neurology: new paradigms and their consequences. The 1999 Ronnie MacKeith lecture.

Abstract: During the past 40 years, research in developmental neurology has provided a number of significant new paradigms on the functional development of the human nervous system. It is important to understand the historical background to the previous paradigms and the reasons why they became outdated and how they have been replaced on empirical grounds.

Comparison of three ankle–foot orthosis configurations for children with spastic diplegia

Abstract: This study compared the functional efficacy of three commonly prescribed ankle-foot orthosis (AFO) configurations (solid [SAFO], hinged [HAFO], and posterior leaf spring [PLS]). Sixteen independently ambulatory children (10 males, six females; mean age 8 years 4 months, SD 2 years 4 months; range 4 years 4 months to 11 years 6 months) with spastic diplegia participated in this study. Four children were classified at level I of the Gross Motor Function Classification System (GMFCS; Palisano et al. 1997); the remaining 12 were at level II. Children were assessed barefoot (BF) at baseline (baseline assessment of energy consumption was performed with shoes on, no AFO) and in each orthotic configuration after three months of use, using gait analysis, oxygen consumption, and functional outcome measures. AFO use did not markedly alter joint kinematics or kinetics at the pelvis, hip, or knee. All AFO configurations normalized ankle kinematics in stance, increased step/stride length, decreased cadence, and decreased energy cost of walking. Functionally, all AFO configurations improved the execution of walking/running/jumping skills, upper extremity coordination, and fine motor speed/dexterity. However, the quality of gross motor skill performance and independence in mobility were unchanged. These results suggest that most children with spastic diplegia benefit functionally from AFO use. However, some children at GMFCS level II demonstrated a subtle but detrimental effect on function with HAFO use, shown by an increase in peak knee extensor moment in early stance, excessive ankle dorsiflexion, decreased walking velocity, and greater energy cost. Therefore, constraining ankle motion by using a PLS or SAFO should be considered for most, but not all, children with spastic diplegia.

Lateralized cognitive deficits in children following cerebellar lesions.

Abstract: The aim of this preliminary study was to examine the developing cognitive profiles of children with cerebellar tumours in a consecutive series of clinical patients. MRI and longitudinal intellectual profiles were obtained on seven children (two females, five males; mean age 3 years at diagnosis; mean age 7 years at first assessment). Tumours in three of the children were astrocytomas; of the remaining tumours, two were medulloblastomas, one low-grade glioma, and one ependymoma. In right-handed children, we observed an association between greater damage to right cerebellar structures and a plateauing in verbal and/or literacy skills. In contrast, greater damage to left cerebellar structures was associated with delayed or impaired non-verbal/spatial skills. Long-term cognitive development of the children studied tentatively supports a role for the cerebellum in learning/development. These findings suggest that lateralized cerebellar damage may selectively impair the development of cognitive functions subserved by the contralateral cerebral hemisphere and, in addition, that all children with cerebellar lesions in early childhood should routinely undergo long-term monitoring of their intellectual development.

Life expectancy among people with cerebral palsy in Western Australia

Abstract: This report describes trends, predictors, and causes of mortality in persons with cerebral palsy (CP) using individuals identified by the Western Australian Cerebral Palsy Register and born between 1958 and 1994. Two thousand and fourteen people were identified (1154 males, 860 females), of whom 225 had died by 1 June 1997. Using date-of-death data, crude and standardized mortality rates were estimated and predictors of mortality sought using survival analysis stratified by decade of birth, description of impairments, and demographic and perinatal variables. For those born after 1967, the cause of death profile was examined over time. Mortality exceeded 1% per annum in the first 5 years and declined to age 15 years after which it remained steady at about 0.35% for the next 20 years. The strongest single predictor was intellectual disability, but all forms of disability contributed to decreased life expectancy. Half of those with IQ/DQ score <20 survived to adulthood, increasing to 76% with IQ/DQ score 20-34, and exceeding 92% for higher scores. Severe motor impairment primarily increased the risk of early mortality. Despite there being 72 persons aged from 25 to 41 years with severe motor impairment in our data set, none had died after the age of 25 years. Infants born after more than 32 weeks' gestation were at significantly higher risk of mortality than very preterm infants, accounted for by their higher rates of intellectual disability. No improvements in survival of persons with CP were seen over the study period despite advances in medical care, improved community awareness, and the increasing proportion of very preterm births among people with CP. This may be the result of improved neonatal care enabling the survival of infants with increasingly severe disabilities.

Neuromotor development from 5 to 18 years. Part 2: associated movements.

Abstract: Associated movements (AMs) are the most frequently assessed parameters of movement quality in children with motor dysfunctions. In this article, reference curves of duration and degree of AMs from 5 to 18 years are provided. In a cross-sectional study of non-disabled children (n=662) duration and degree of AMs were estimated at six specific ages while children performed repetitive finger, hand, and foot movements, alternating hand and foot movements, diadochokinesis, sequential finger movements, pegboard, stress gaits, and dynamic balance. Moderate-to-high intraobserver and interobserver reliability for the assessment of AMs were noted. Duration and degree of AMs displayed a non-linear developmental course that was a function of the motor task's complexity. AMs decreased most with age in repetitive movements, less in alternating and sequential movements, and least in the pegboard and dynamic balance. Reference curves demonstrated large interindividual variations for duration and degree of AMs. Both the variable developmental course and large interindividual variation need to be taken into account in the assessment of movement quality of school-age children. In contrast to timed performance, considerable sex differences for AMs were observed.

Recent advances in cortical visual impairment.

Abstract: Cortical visual impairment (CVI) is the leading cause of bilateral visual impairment in children in Western countries. This finding reflects better methods for identifying visual impairment due to CNS injury and also advances in perinatal care, which have increased the survival rate of children with neurological morbidity. This review will describe advances in the diagnosis and management of CVI. Central to our discussion is a definition of CVI that includes a decrease in visual acuity. New treatment and rehabilitative measures are badly needed for this disorder. We hope to stimulate interest in CVI, a disease that has become a significant public health problem.

Differential development of infants at risk for psychopathology: the moderating role of early maternal responsivity.

Abstract: The development of behaviour problems in infants born with biological risk (low birthweight) and psychosocial risk (psychosocially disadvantaged family) was studied in a sample of 347 children (171 males, 176 females) at the ages of 2, 4:6, and 8 years. In the search for factors that moderate the effects of early risks, the role of early responsive caregiving was examined. Results indicate that infants at psychosocial risk exhibited both more externalizing and internalizing problems across ages than infants not at psychosocial risk, while no overall differences were apparent between normal- and low-birthweight groups. With one exception, no interactions between biological and psychosocial risk factors emerged, suggesting that their simultaneous effect is largely additive. Maternal responsivity was found to moderate the effects of low birthweight on hyperkinetic and internalizing problems as well as to influence the consequences of family disadvantage on total problems. These findings stress the importance of early parenting in the behavioural development of at-risk children.

Effects of ketogenic diet on development and behavior: preliminary report of a prospective study.

Abstract: The ketogenic diet is increasingly used for the management of difficult-to-control seizures in children. Here, we describe the first prospective study of the effects of the diet on development, behavior, and parenting stress. Participants were 65 children (36 males, 29 females) with intractable seizures, ages 18 months to 14 years 6 months, enrolled in a prospective study at the Johns Hopkins Hospital, Baltimore, MD, USA, to study the diet's efficacy. Children were assessed before diet initiation and at 1-year follow-up. At follow-up, 52% (34 of 65) children remained on the diet. Mean seizure frequency decreased from 25 per day before diet initiation to less than two per day 1 year later. At follow-up, mean developmental quotient showed statistically significant improvement (p<0.05), with significant behavioral improvements in attention and social functioning. Parental stress was essentially unchanged. No baseline factor examined predicted diet adherence, and the primary reason for diet discontinuation was insufficient seizure control. These preliminary results support prior anecdotal reports of the beneficial effects of the diet on cognition and behavior.

Visual and visuospatial development in young children with Williams syndrome

Abstract: This study investigated the relation between sensory visual problems and the severity of visuospatial difficulties in a large group of young children with Williams' syndrome (WS). A questionnaire describing visual and associated problems was completed by the families of 108 children with WS and detailed follow-up assessments were conducted, including visual, spatial, motor, visuocognitive, and linguistic tests of 73 of these children (mean age 7 years 3 months; 40 males, 73 females). Children with WS showed a much higher incidence of common paediatric sensory vision problems (strabismus, visual acuity loss, amblyopia, reduced stereopsis) than normally developing children. It was found that delays with respect to age normative values increased with age on all tests. No significant correlation was found between the presence of a visual deficit and the severity of the visuospatial problems, suggesting that the difficulties children with WS have in understanding spatial arrangements are not simply a result of their earlier sensory visual problems. Results confirm the dissociation between visuospatial and language abilities in children with WS, and support the neurobiological model of a split between ventral and dorsal stream processing of visual information with a generalized deficit in dorsal stream processing in young children with WS.

Health-related quality of life and functional outcome measures for children with cerebral palsy.

Abstract: The aim of this study was to examine measures of health-related quality of life (HRQL) in children with cerebral palsy (CP) by comparing scores of a generic HRQL measure, the Child Health Questionnaire (CHQ); a disease-specific HRQL measure for children with CP, the Caregiver Questionnaire (CQ); and a pediatric functional measure, the Wee-Functional Independence Measure (WeeFIM). Participants included 30 caregivers of children with CP. The caregivers' children were a mean age of 8 years 6 months (17 females, 13 males). The ethnic origin of the children was 18 African-American, 8 white, 3 Hispanic, and 1 Middle Eastern. Significant correlations were found between the CQ and WeeFIM total and subscale scores (r=0.388 to 0.641). There was no correlation between the CHQ and CQ total summary scores, but significant correlations were found between the CHQ subscales related to parent time and family cohesion and the CQ total and subscale scores (r=0.386 to 0.481). The lack of correlation between the CHQ and WeeFIM indicates HRQL and function are different constructs that cannot be inferred from each other. The fair relationship found between the CQ and WeeFIM suggests that the constructs measured in these two assessments overlap. The lack of correlation between the total summary scores of the CHQ and CQ suggests the CQ may be a more specific measure of HRQL for this population that reflects the impact of the child's condition on the caregiver.

Powered wheelchairs and independence in young children with tetraplegia

Abstract: The aim of this study was to assess the effect of early provision (≦ 8 years) of a powered wheelchair (PWC) in children with tetraplegia. Twenty-nine children (15 males, 14 females; mean age 6 years 3 months, age range 3 to 8 years) with spastic or dystonic tetraplegia were studied. All participants had severe motor impairment. Treatment outcomes were investigated in several dimensions of disablement: Impairment, Functional Limitation/Activity, Disability/Participation. It was found that the level of independence improved significantly after PWC provision, while motor impairment, IQ, and quality of life did not. The majority of children (21 of 27) reached a level of driving competence which allowed them to move around with or without minimal (i.e. verbal) adult support. Achievement of this competence was not statistically related to IQ or motor impairment but correlated to the time spent in the PWC. The majority of parents (21 of 25) were not in favour of the PWC when the study started but after PWC provision, 23 of 25 parents expressed positive feelings about it. Reactions of the majority of children (23 of 25) were positive from the beginning of the study and did not change over time. The authors concluded that PWCs can aid independence and socialization and the majority of children can achieve a good-enough driving competence, even those with severe learning disability or motor deficit. PWCs should not be viewed as a last resort but as a means of providing efficient self-locomotion in children with a severe motor deficit.