There are few well-controlled, published evaluations of sleep interventions for children with developmental disabilities. This paper evaluates a parent training programme using behavioural principles to reduce sleep problems in children with autism or fragile X syndrome (FXS). Training included bedtime routines, reinforcement, effective instructions, partner support, and extinction (removing reinforcement to reduce a behaviour). Programme efficacy was demonstrated by using a multiple baseline across-participant design. Social validity was also assessed. Five children with autism and one with Asperger syndrome (four males, two females; mean age 5y 6mo; age range 3y 5mo to 7y 4mo) and their parents; and seven children with FXS (six males, one female; mean age 4y 9mo; age range 1y 11mo to 9y 1mo) and their parents participated. Ten families completed intervention within the multiple baseline design. Most parents' goals were achieved and visual analysis of the sleep data indicated improvement. Settling problems, night waking, and co-sleeping were effectively reduced. The programme had high social validity and outcomes were clinically significant and maintained at follow-up.

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.

This study aimed at investigating the possible dissociation between visual–object and visual–spatial working memory (WM) in individuals with Williams syndrome (WS) and Down syndrome (DS). Four study groups were included: WS group (10 males, 5 females) with a mean chronological age (CA) of 19 years 8 months (SD 6y 1mo) and a mean mental age (MA)of 6 years 11 months (SD 1y 5mo); WS comparison group (7 males, 8 females) comprised of typically developing children with a mean CA of 6 years 10 months (SD 10mo) and a mean MA of 6 years 11 months (SD 8mo)matched as a group with the participants with WS on the basis of mental age; DS group (11 males, 7 females) with a mean CA of 15 years 10 months (SD 5y 8mo) and a mean MA of 5 years 2 months (SD 8mo); and DS comparison group (10 males, 8 females) with a mean CA of 5 years and 1 month (SD 7mo)and a mean MA of 5 years 2 months (SD 8mo) selected to match the DS group on the basis of mental age. They were all administered tests that explored visual perception (Visual Perception Test – Subtest 4 and Line Orientation tests), visual imagery (imaging the colour of objects and the tail length of well-known animals), spatial imagery (mental rotation of visually presented or verbally evoked objects), and WM for visual–object and visual–spatial information. Individuals with WS exhibited specific difficulties in the visual–spatial, but not the visual–object, WM task. Instead, people with DS showed reduced performance in both tests. However, whereas the observed deficit in individuals with DS persisted when perceptual abilities were taken into account, the deficit in individuals with DS was compensated when their scores were adjusted for performance on perceptual tasks. These results support the hypothesis of a dissociation within the sketch-pad slave system in the WM model and reinforce the view of intellectual disability as a non-unitary condition.

This study assessed the effect of an oral stimulation program on the maturation of sucking skills of preterm infants. Thirty-two preterm infants (13 males, 19 females), appropriate size for gestational age (gestational age at birth 28 wks, SD 1.2wks; birthweight 1002g, SD 251g), were randomly placed into experimental and control groups. The experimental group received a daily 15-minute oral stimulation program, consisting of stroking the peri- and intra-oral structures, for 10 days before the start of oral feedings. Sucking measures were monitored with a specially-designed nipple-bottle apparatus. Results indicate that the experimental group achieved full oral feedings 7 days sooner than the control group, and demonstrated greater overall intake (%), rate of milk transfer (mL/min), and amplitude of the expression component of sucking (mmHg). There was no difference in sucking stage maturation, sucking frequency, and amplitude of the suction component of sucking. Endurance, defined as ability to sustain the same sucking stage, sucking burst duration, and suction and expression amplitudes throughout a feeding session, was not significantly different between the two groups. The stimulation program enhanced the expression component of sucking, resulting in better oral feeding performance.

To study the coordination of respiration and swallow rhythms we assessed feeding episodes in 20 preterm infants (gestational age range at birth 26–33wks; postmenstrual age [PMA] range when studied 32–40wks) and 16 term infants studied on days 1 to 4 (PMA range 37–41wks) and at 1 month (PMA range 41–45wks). A pharyngeal pressure transducer documented swallows and a thoracoabdominal strain gauge recorded respiratory efforts. Coefficients of variation (COVs) of breath–breath (BR–BR) and swallow–breath (SW–BR) intervals during swallow runs, percentage of apneic swallows (at least three swallows without interposed breaths), and phase of respiration relative to swallowing efforts were analyzed. Percentage of apneic swallows decreased with increasing PMA (16.6% [SE 4.7] in preterm infants [les ]35wks' PMA; 6.6% [SE 1.6] in preterms >35wks; 1.5% [SE 0.4] in term infants; p<0.001). Term infants had significantly lower BR–BR COV (0.405 [SE 0.016]) than preterm infants (0.641 [SE 0.052] at >35wks' PMA; 0.693 [SE 0.059] at [les ]35wks' PMA). Phase relation between swallowing and respiration stabilized with increasing PMA, with decreased apnea, and a significant increase in percentage of swallows occurring at end-inspiration. These data indicate that unlike the stabilization of suck and suck–swallow rhythms, which occur before about 36 weeks' PMA, improvement in coordination of respiration and swallow begins later. Coordination of swallow–respiration and suck–swallow rhythms may be predictive of feeding, respiratory, and neurodevelopmental abnormalities.

During the last century, knowledge of the mechanisms governing the functions of the central nervous system increased rapidly as sophisticated physiological, neurochemical, and imaging techniques developed. In the field of motor control, better understanding of neurophysiology caused a gradual shift from the concept that motor behaviour is largely controlled by reflex mechanisms towards the notion that motility is the net result of complex spinal or brainstem activity, which is subtly modulated by segmental afferent information and ingeniously controlled by supraspinal networks. Nowadays it is assumed that motor control of rhythmical movements like locomotion, respiration, sucking, and mastication is based on so-called central pattern generators (CPGs): neuronal networks which can generate complex basic activation patterns of the muscles without any sensory signals. Nevertheless, sensory information of the movement is important in adapting the movement to the environment. The activity of the networks, which are usually located in the spinal cord or brainstem, is controlled from supraspinal areas via descending motor pathways. The supraspinal activity itself is organized in large-scale networks in which cortical areas are functionally connected through direct recursive interaction or through intermediary cortical or subcortical (striatal, cerebellar) structures.

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.

The aim of this paper is to evaluate correlative magnetic resonance imaging (MRI) and histological parameters of development of cortical afferents during pathfinding and target selection in transient fetal cerebral laminas in human fetuses and preterm infants. The transient fetal subplate zone, situated between the fetal white matter (i.e. intermediate zone) and the cortical plate, is the crucial laminar compartment for development of thalamocortical and corticocortical afferents. The prolonged coexistence of transient (endogenously active) and permanent (sensory-driven) circuitry within the transient fetal zones is a salient feature of the fetal and preterm cortex; this transient circuitry is the substrate of cerebral functions in preterm infants. Another transient aspect of organization of developing fibre pathways is the abundance of extracellular matrix and guidance molecules in periventricular crossroads of projection and corticocortical pathways. Both the subplate zone and periventricular crossroads are visible on MRI in vivo and in vitro. Hypoxic-ischaemic lesions of periventricular crossroads are the substrate for motor, sensory, and cognitive deficits after focal periventricular leukomalacia (PVL). Lesions of distal portions of the white matter and the subplate zone are the substrate for diffuse PVL. The neuronal elements in transient fetal zones form a developmental potential for plasticity after perinatal cerebral lesions.

This study investigated sex and age differences in standing balance. Movement of the centre of pressure (COP) was calculated from ground reaction force data collected from a force platform during bipedal stance with eyes open and eyes closed. Three groups of 60 children, with 30 girls and 30 boys in each, were assessed. Mean ages of each group were as follows: 9 years 11 months (standard deviation [SD] 3mo); 12 years 11 months (SD2mo); and 15 years 11 months (SD3mo) respectively. Summary sway parameters and frequency domain variables were calculated in the anteroposterior and mediolateral directions. Boys exhibited greater COP movement than girls at 9 to 10 years of age. Age-related ‘improvements’ in sway occurred in boys, thus some aspects of postural control are still developing after 9 to 10 years of age. As very little age-related difference was seen in girls, boys may lag behind somewhat in terms of developing postural control. Thus there is a need to study the sexes separately when investigating balance in children.

Postoperative pain in children with spastic cerebral palsy (CP) is often attributed to muscle spasm and is difficult to manage using opiates and benzodiazepines. Adductor-release surgery to treat or prevent hip dislocation in children with spastic CP is frequently performed and is often accompanied by severe postoperative pain and spasm. A double-blinded, randomized, placebo-controlled clinical trial of 16 patients (mean age 4.7 years) with a mainly spastic type of CP (either diplegic or quadriplegic in distribution) was used to test the hypothesis that a significant proportion of postoperative pain is secondary to muscle spasm and, therefore, might be reduced by a preoperative chemodenervation of the target surgical muscle by intramuscular injection of botulinum toxin A (BTX/A). Compared with the placebo, BTX/A was found to be associated with a reduction in mean pain scores of 74% (P<0.003), a reduction in mean analgesic requirements of approximately 50% (P<0.005), and a reduction in mean length of hospital admission of 33% (P<0.003). It was concluded that an important component of postoperative pain in the patient population is due to muscle spasm and this can be managed effectively by preoperative injection with BTX/A. These findings may have implications for the management of pain secondary to muscle spasm in other clinical settings.

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 2X2 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.

Passive stretching is widely used for individuals with spasticity in a belief that tightness or contracture of soft tissues can be corrected and lengthened. Evidence for the efficacy of passive stretching on individuals with spasticity is limited. The aim of this review was to evaluate the evidence on the effectiveness of passive stretching in children with spastic cerebral palsy. Seven studies were selected according to the selection criteria and scored against the Physiotherapy Evidence Database scale. Effect size and 95% confidence intervals were calculated for comparison. There was limited evidence that manual stretching can increase range of movements, reduce spasticity, or improve walking efficiency in children with spasticity. It appeared that sustained stretching of longer duration was preferable to improve range of movements and to reduce spasticity of muscles around the targeted joints. Methods of passive stretching were varied. Further research is required given the present lack of knowledge about treatment outcomes and the wide use of this treatment modality.

Postural control deficits have been suggested to be a major component of gait disorders in cerebral palsy (CP). Standing balance was investigated in 23 ambulatory children and adolescents with spastic diplegic CP, ages 5 to 18 years, and compared with values of 92 children without disability, ages 5 to 18 years, while they stood on a force plate with eyes open or eyes closed. The measurements included center of pressure calculations of path length per second, average radial displacement, mean frequency of sway, and Brownian random motion measures of the short-term diffusion coefficient, and the long-term scaling exponent. In the majority of children with CP (14 of 23) all standing balance values were normal. However, approximately one-third of the children with CP (eight of 23) had abnormal values in at least two of the six center of pressure measures. Thus, mean values for path length, average radial displacement, and diffusion coefficient were higher for participants with CP compared with control individuals with eyes open and closed (p<0.05). Mean values for frequency of sway and the long-term scaling exponent were lower for participants with CP compared with control participants (p<0.05). Increased average radial displacement was the most common (nine of 23) postural control deficit. There was no increase in abnormal values with eyes closed compared with eyes open for participants with CP, indicating that most participants with CP had normal dependence on visual feedback to maintain balance. Identification of those children with impaired standing balance can delineate factors that contribute to the patient's gait disorder and help to guide treatment.

It is well known that individuals with cerebral palsy (CP) are subject to higher mortality than the general population but the causes of this have not been systematically analysed. This study investigates mortality in a Californian population of 45292 individuals with CP, 4028 of whom died during the 1986 to 1995 study period. The aims of this study were to identify diseases that may be causally linked with CP, and diseases whose diagnosis and/or treatment need improvement. Overall, the standardized mortality ratio (SMR) was 8.4. Mortality from breast cancer was three times that of the general population, suggesting poorer detection and/or treatment. The dramatic elevation of mortality due to brain cancer, especially in children (SMR=24), raises the possibility of a link between this and CP. Cause of death was non-specific in some individuals. Therefore, these SMRs are conservative. As expected, SMRs due to respiratory diseases were very high but, contrary to anecdotal reports, such diseases did not account for most deaths. High SMRs were also found for diseases of the circulatory and digestive systems. Finally, a marked elevation of deaths was due to external causes, including drowning and being hit by motor vehicles.

Neuropsychological and behavioral status were examined in 57 children aged 7 to 16 years with complex partial seizures (CPS) and compared with 27 sibling control children of the same age. Epilepsy had a significant effect on both cognitive and behavioral adjustment measures. Children with CPS had significant impairment across all seven cognitive domains assessed, reflective of a profile of relatively diffuse and generalized cognitive dysfunction. Age at onset of recurrent seizures was the strongest and most consistent predictor of adequacy of cognitive functioning; earlier age at onset was associated with poorer cognitive status. Children with CPS also had more problems compared with sibling control children on measures of social and school competence and internalizing behavior problems, but not externalizing behaviors. Further, frequency of seizure activity in the past year, rather than age at seizure onset, emerged as the strongest predictor of these behavioral difficulties. These findings are discussed in the context of understanding the impact of CPS on cognition and behavioral adjustment, and identifying the contribution of various aspects of the neurodevelopmental course of CPS to these issues.

This study evaluated the cognitive profiles of children with idiopathic generalized epilepsy (IGE), uniformly treated with valproic acid with well-controlled seizures. Twenty-four were neuropsychologically evaluated. They comprised: 14 females, 10 males: 12 with generalized tonic–clonic seizures (GTCS), mean age 14y 4mo, SD 1y 7mo, range 12y to 16y 4 mo; 12 with absence seizures (AS) mean age 14y 5mo, SD 1y 10mo, range 11y to 16y 4mo, with intellectual abilities within the normal range and age-appropriate scholastic skills, and 20 healthy controls (12 females, 8 males; mean age 14y 5mo, SD 1y 10mo, range 10y 7mo to 16y 7mo). As a group, children with IGE performed significantly poorer in all tests (non-verbal and verbal attention, verbal learning and memory, word fluency, and controlled sequential fine motor responses) excluding non-verbal memory. Analysis according to type of seizure revealed that both patient groups (AS and GTCS) had an attention deficit, whereas only children with AS showed deficits in verbal learning and memory, word fluency, and controlled fine motor responses. These results suggest a long-term risk of learning impairment for children with IGE, even if they have normal intelligence and their seizures are well controlled.

Cerebral palsy (CP) can occur in term infants with or without preceding newborn encephalopathy. We compared the type and severity of CP and associated disability in these two groups. Participants from a population-based case-control study of term newborn encephalopathy were followed up for 6 years and linked to the Western Australian Cerebral Palsy Register. The remaining term infants with CP for the same period were also identified from the Cerebral Palsy Register. 13% of neonatal survivors of term newborn encephalopathy had CP, a rate of 116 per 1000 term live births. Overall, 24% of term infants with CP followed newborn encephalopathy. CP following newborn encephalopathy was more likely to: affect males (72% vs 56%); be severe (47% vs 25%); and be of spastic quadriplegia or dyskinetic types. Cognitive impairment was more common (75% vs 43%) and severe (41% vs 16%), as was epilepsy (53% vs 29%) in survivors of encephalopathy. These children were also more likely to: be non-verbal (47% vs 22%); have a severe composite disability score (47% vs 26%); and die between time of diagnosis of CP and age 6 years (5-year cumulative mortality 19% vs 5%). Children born at term who develop CP following newborn encephalopathy have a poorer prognosis than those with CP who were not encephalopathic in the first week of life.

Benign childhood epilepsy with centrotemporal spikes (BCECTS) is a well-known idiopathic age- and localization-related epileptic syndrome with characteristic clinical and EEG manifestations. Due to the reported benign evolution of this epilepsy syndrome, neuropsychological assessment has been considered unnecessary. However, the benign nature of BCECTS has recently been challenged: verbal dysfunction as well as impaired visuomotor coordination, specific learning disabilities, and attention deficit have been noticed. These findings prompted this research study in which all children with BCECTS attending our epilepsy clinic underwent neuropsychological assessment. Seventeen children (10 boys and seven girls) aged 7 to 14 years were investigated with a neuropsychological test battery focusing on immediate and delayed recall of auditory-verbal and visual material, verbal fluency, problem-solving ability, and visuospatial constructional ability. Raven's coloured matrices and questionnaires regarding school functioning and behaviour were also administered. The children were matched with control subjects for age, sex, and school. Children with BCECTS had significantly lower scores than their control subject partners on the neuropsychological items. Intellectual abilities did not differ and neither did school functioning or behaviour according to teachers. Parents, however, recognized greater difficulties with concentration, temperament, and impulsiveness in children with BCECTS.

This study aimed to investigate the prevalence of sleep problems in Chinese children with pervasive developmental disorders (PDD) in Hong Kong and their relationship to parental stress. A cross-sectional survey was conducted in six child assessment centres. All parents of the children with PDD completed the Children's Sleep Habits Questionnaire, the Parenting Stress Index – Short Form, and questions on sleep practice. A total of 210 out of 250 questionnaires (response rate 84%) were returned. Mean age of the children was 3 years 6 months (SD 1y 4mo; range 2y–7y 7mo; 168 males, 25 females). The prevalence of parent-defined sleep problems in various sleep domains ranged from 9.3 to 45.6%, with 67.9% of children having significant problems in at least one sleep domain. The most common problems reported were bedtime resistance and parasomnias. Similar sleep problems occurred in all the PDD subgroups. The factor most significantly associated with sleep problems was the occurrence of sleep problems before the age of 2 years. The parents of children with PDD with sleep problems experienced a higher level of stress than those whose children had no sleep problems. A high prevalence of significant sleep problems was reported in Chinese children in Hong Kong with PDD. A higher stress level among the parents of those children with PDD with sleep problems suggests the need for systematic early detection and management of sleep problems in children with PDD.

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.