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.

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.

Infants with bronchopulmonary dysplasia (BPD) often have difficulty achieving coordinated suckle feeding. To analyze rhythmic differences during feeding in infants with BPD we performed weekly studies of 14 infants with BPD (eight male, six female; postmenstrual age [PMA] 32.1 to 39.7 weeks); and a PMA-matched control group without BPD (n=20), from initiation of bottle feeding until discharge, with simultaneous digital recordings of pharyngeal and nipple (teat) pressure. Unlike the control group, there was no significant correlation between PMA and stability of suckle rhythm, aggregation of suckles or swallows into runs, or length of suckle runs. Comparing those infants >35 weeks' PMA, the group with BPD had significantly decreased stability of suckle rhythm (increased coefficient of variation of suckle–suckle intervals: 0.34, SE 0.02 vs 0.254, SE 0.014; p=0.003), decreased aggregation into suckle runs (71.1, SE 3.4% vs 85.4, SE 2%; p=0.001), and decreased length of suckle runs (7.2, SE 0.9 vs 13.1, SE 1.9 suckles/run; p=0.003). Percentage of swallows in runs was also decreased in the cohort with BPD (58, SE 3.8% vs 77.2, SE 3.5%; p<0.001), as was length of swallow run (5.3, SE 0.5 vs 10.7, SE 1.1; p<0.001). Thus, in infants with BPD, anticipated maturational patterns of suckle and swallow rhythms did not occur. Delay in attainment of stable suckle and swallow rhythms in preterm infants, especially after 35 weeks' PMA, may predict subsequent feeding and neurological problems.

The relationship of preterm birth to health-related quality of life (HRQoL) was examined for children aged 1 to 4 years. Three gestational age groups with a NICU history were selected, <32 weeks (n=65), 32 to 36 weeks (n=41), [ges ]37 weeks (n=54), and a reference group from the open population (n=50). The main instrument was the TNO-AZL Preschool Quality Of Life (TAPQOL) questionnaire, which was completed by the parents. In addition, other outcome measures obtained from parents or neonatologists were investigated. Children born <32 weeks had significantly lower HRQoL than the reference group in the scales for lungs, stomach, eating disorders, motor functioning, communication, and anxiety. Parental feelings towards the child were related to the child's HRQoL. We found differences between the neonatologists' and parents' perceptions of the children's situation, which can have clinical consequences (e.g. different opinions about what needed treatment). Neonatal intensive care after birth has HRQoL implications for all children, particularly in children born at <32 weeks of gestation.

This study compared adolescents with cerebral palsy (CP) and their families to adolescents without physical disabilities and their families as the child enters and leaves adolescence (age ranges 13 to 15 years and 19 to 23 years). Families of 90 individuals with CP (42 females, 48 males) and 75 individuals without physical disabilities (34 females, 41 males) participated. They completed the Family Assessment Device, Life Situation Survey, Multidimensional Scale of Perceived Social Support, and Future Questionnaire. There were few differences in family functioning, life satisfaction, or perceived social support between the groups. Expectations of young adults with CP and parents of both adolescents and young adults regarding future independence and success were lower than the expectations of the control group. While the group results emphasize similarities between families during the two stages of adolescence, individual families and individual family members report specific challenges.

Bladder involvement is common in transverse myelitis but its characteristics and natural history, particularly in relation to neurological outcome, have not been described in a paediatric population previously. Ten children, with ages ranging from 8 months to 16 years, who had a diagnosis of acute transverse myelitis were studied with video urodynamics and followed up in a tertiary paediatric neurourology clinic. Nine of ten children had obstructive urinary tract symptoms at presentation and all went on to develop ‘irritative’ urinary tract symptoms (frequency and urgency) about 1 month after initial presentation. Video urodynamics showed a combination of irritative (detrusor hyperreflexia) and obstructive (detrusor–sphincter dyssynergia) abnormalities in most patients and enabled management to be specifically directed towards these. The patients progress was followed up for a median duration of 36 months. All had residual bladder dysfunction, only four were asymptomatic on treatment. The degree of recovery of bladder function was not related to the degree of motor recovery.

Individuals with Angelman syndrome (AS) have severe learning disability, ataxia, seizures, dysmorphic facies, a happy, sociable disposition, and inability to speak. Most of the literature concerning the clinical features of AS has concentrated on younger children. This study aimed to look at the natural history of AS by documenting the clinical features in a group of 28 adolescents and adults with AS (12 males, 16 females; age range 16 to 40 years). Specific aspects studied included physical characteristics, general health, mobility, seizure disorder, behaviour and communication, and self-help skills. Problems seen in this older group of individuals differed significantly from those typically observed in younger children. The incidence of scoliosis and joint contractures increased with age. Facial features were more striking in adults. Hyperactivity and concentration improved and the sociable disposition persisted. There were subtle clinical differences between the groups of individuals with different underlying genetic abnormalities. Information obtained from this study is relevant to diagnosis and management of older individuals with AS.

Quantitative analyses of cross-sectional areas of the thalami, caudate nuclei, and lentiform nuclei were performed in 29 preterm infants (16 males, 13 females; mean age 29.6 weeks, age range 27 to 24 weeks,) with periventricular leukomalacia (PVL). MRI was carried out in the infants between 9 and 18 months of corrected age and in 16 control infants. Bilateral thalami, caudate nuclei, lentiform nuclei, cerebral hemispheres, and cerebellum were measured by computer. Ratios of the areas of the thalami (Th), caudate nuclei (Ca), lentiform nuclei (Le), and cerebral hemispheres (CH) to that of the cerebellum (Ce) were calculated in each infant. The ratio of Th:Ce was significantly smaller in infants with moderate and severe PVL than in the control group bilaterally. Abnormal intensity areas were not observed in the thalami in any infants with PVL. CH:Ce was also smaller in infants with severe PVL than in the control group. No significant difference was observed between the groups in ratios Le:Ce or Ca:Ce. Results of our study suggest that the volume of the thalami is reduced and that thalamic involvement is present in infants with white matter lesions who have moderate to severe PVL.

Functional MRI (fMRI) of the visual cortex was evaluated in 42 sedated 18-month-old infants (mean corrected age; 31 males, 11 females) with or without periventricular leukomalacia (PVL). Data from 14 infants could not be evaluated because of movement artefacts. Ten of the remaining 28 infants showed no significant fMRI response upon visual stimulation. In 18 infants, a significant signal change upon stimulation was found in the visual cortex: in 17 a signal decrease and in one a signal increase. Functional changes were located mainly in the anterior part of the visual cortex. Seven of the 28 infants had normal MRI and 21 showed variable occipital PVL. An fMRI response was equally frequent in infants without PVL (4 of 7 infants) and with PVL (14 of 21 infants). In conclusion, fMRI was shown to be feasible in sedated infants. No correlation was found between functional activation and the presence or absence of occipital PVL. Type of fMRI response (signal decrease) and localization (anterior part of the visual cortex) are different from those seen in adults, probably reflecting a combination of sedation effects and immaturity of the visual system. At present, fMRI is a highly promising research tool; its clinical relevance still has to be established.

Twelve children with vertebrobasilar artery stroke are reported (seven males, five females; aged 6 months to 15 years). Patient 1 showed an arcuate foramen in the posterior arch of the atlas, an anatomical variant occurring in 3 to 15% of the population. It was hypothesized that the presence of the arcuate foramen might cause tethering of the vertebral artery and lead to its dissection by repetitive trauma. Lateral plain films of the cervical spine in cases of posterior circulation stroke were taken. Eight of 11 patients showed aberrant arcuate foramina. Of the remaining three patients, one had normal cervical spine X-rays, one had an absent right posterior arch of the atlas following previous surgery for a cervical meningocele, and one patient had incomplete ossification of the vertebrae. Seven of the nine patients with arcuate foramina had vertebral angiograms. In all cases this showed the vertebral artery passing through the arcuate foramen before entering the brain and an appearance consistent with arterial dissection and occlusion at the same site below the foramen. Most documented cases of posterior circulation stroke in children follow trauma, which may be minimal or repetitive, with thrombotic occlusion of the artery at C1–C2 level. The association with an arcuate foramen and its possible causative role in the genesis of posterior circulation stroke in children has not been previously recognized. There may be a causal association between the presence of an arcuate foramen, tethering of the vertebral artery in the foramen, and dissection from repetitive trauma with movement of the neck.

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.