The purposes of this study were to evaluate whether resistance to stretch could be measured in a reproducible manner from day to day in children with severe multiple disabilities, and whether 6 weeks of daily activity, including one daily stretch, would modify passive resistance to stretch. Passive resistance to stretch was measured in six children using an established model that synchronously and continuously measured knee flexion moment (Nm) and EMG activity of the hamstring, while the velocity and angle of a static stretch was controlled. Resistance to stretch was evaluated in the absence of EMG activity. Measurements were taken on two separate occasions with a 2-week hiatus, and before and after a 6-week activity period which included a daily 1-minute static stretch. There was no significant change in resistance to stretch when tested 2 weeks apart (test 1, 8.6±1.2Nm; test 2, 8.3±1.2Nm, p<0.05) which yielded a strong correlation coefficient (Spearman r=1.0, p<0.01; Pearson r=0.953, p<0.01). Similarly, there was no difference in resistance to stretch before and after the 6-week period (mean difference 0.22±0.75Nm, p<0.05). Data from this study demonstrate that resistance to stretch of the hamstring muscle group could be measured in a reproducible manner from day to day in children with multiple disabilities. Further, resistance to stretch was unchanged after 6 weeks of daily activity, including a daily 1-minute static stretch.

Set-shifting is an important aspect of attention and regulation of behaviour. The ability of young children to shift set is debated. Set-shifting is usually examined using sorting tasks that are designed for adults and hence are inadequate for children. In this study, an experimental Sorting Task for Children (STC) is administered to 54 healthy children (aged 4 to 10 years) and compared with a widely used adult sorting task, the Modified Card Sorting Test. This experiment shows that preschool children are capable of set-shifting. An extensive error analysis of the STC reveals that perseverations are not predominant. Hence, set-shifting in young children may be characterised by ‘trial and error’ rather than by being stuck in a mental set, which is suggested to be typical of adults with frontal lobe damage. A pilot study of 10 children with idiopathic or cryptogenic epilepsy shows unstable set-shifting rather than inability to shift set, which is concordant with known problems in regulation of behaviour.

Efforts to examine the structural neuroanatomy of autism by using traditional methods of imaging analysis have led to variable findings, often based on methodological differences in image acquisition and analysis. A voxel-based computational method of whole-brain anatomy allows examination of small patterns of tissue differences between groups. High-resolution structural magnetic resonance images were acquired for nine males with high-functioning autism (HFA; mean age 14y [SD3y 4mo]), 11 with Asperger syndrome (ASP; mean age 13y 6mo [SD2y 5mo]), and 13 comparison (COM) participants (mean age 13y 7mo [SD3y 1mo]). Using statistical parametric mapping, we examined contrasts of gray matter differences between the groups. Males with HFA and ASP had a pattern of decreased gray matter density in the ventromedial regions of the temporal cortex in comparison with males from an age-matched comparison group. Examining contrasts revealed that the COM group had increased gray matter density compared with the ASP or combined HFA and ASP group in the right inferior temporal gyrus, entorhinal cortex, and rostral fusiform gyrus. The ASP group had less gray matter density in the body of the cingulate gyrus in comparison with either the COM or HFA group. The findings of decreased gray matter density in ventromedial aspects of the temporal cortex in individuals with HFA and ASP lends support to theories suggesting an involvement of these areas in the pathophysiology of autism, particularly in the integration of visual stimuli and affective information.

In a population-based follow-up study (the Stockholm Neonatal Project), 182 children with a birthweight of 1500g or less (very-low birthweight: VLBW) and a control group of 125 children born healthy at term were examined with the Wechsler Preschool and Primary Scale of Intelligence–Revised (WPPSI–R) and a neuropsychological test battery (Nepsy) at 5½ years of age. The WPPSI–R results of the VLBW children fell well within the normal range: WPPSI–R full-scale IQ 95.7, verbal subscale IQ 99.9, and performance subscale IQ 91.6. Nevertheless, the control group had significantly better results than the VLBW group which could be attributed to the greater variability of the VLBW group, with a larger proportion falling in the lower area of the IQ distribution, especially on the performance subscale. Likewise, the control group displayed better executive function (Nepsy). Paternal education was equal in the two groups and was the single most important predictor of IQ, possibly acting as a protective factor. The need for glasses or lenses was inversely associated with all IQ measures and severe retinopathy of prematurity (ROP) had the most negative impact on full-scale and performance IQ. These two IQ measures were also negatively associated with intrauterine growth retardation late in pregnancy. We conclude that VLBW children, in the absence of these identified risk factors, have normal cognitive development.

Congenital bilateral perisylvian polymicrogyria syndrome (CBPS) has a cerebral cortical localization and its phenotype was thought to be purely central. This study of seven children with CBPS (five males, two females; mean age 5y [SD 3y 6mo]; range 1mo–11y 10mo) documents electrophysiological evidence of lower motor neuron involvement in association with congenital contractures (limb or jaw) in six of the seven children studied. This is not an expected association and does not conform to the traditional lesional classification system of the cerebral palsies. Possible pathogenic mechanisms are discussed but this association of upper and lower motor neuron involvement is likely to be a previously unsuspected part of a genetic or other pathogenic sequence.

Methodological concerns are a major reason behind having to turn down papers offered to Developmental Medicine and Child Neurology. One important cause is inadequate validation of measures used in the work. The validity of a test is one of its most important attributes, whether it is a clinical assessment, laboratory assay, electrophysiological or neuroimaging investigation, or a measurement of function, such as motor or IQ scales. With such a plethora of tests used in both everyday clinical work and research, and with the difficulties involved, it is not surprising that some are incompletely validated.

To summarize our 10-year experience with autistic children at the Tel Aviv Child Development Center, the files of all 55 children with autism treated at our center over a 10-year period were retrospectively reviewed. Particular attention was addressed to the value of the medical work-up in detecting the etiology of autism and to factors differentiating infantile autism (IA) from autistic-like behavior (ALB). Twenty-four subjects (44%) had IA and 31 (56%) had ALB. These subgroups were compared for demographic, perinatal, familial, neurological, and psychological findings, and outcome at discharge. Associated medical conditions and the yield of metabolic work-up and neuroimaging and electroencephalography studies are discussed. The two subgroups differed only in severity of autistic symptoms and cognitive function (P<0.05), but not in demographic or neurobiological findings. It was concluded that IA and ALB are similar conditions, and autism has a wide continuum of clinical expressions.

The aim of this study was to establish the reliability and validity of visual gait assessment in children with spastic diplegia, who were community or household ambulators, using a modified version of the Physicians Rating Scale, known as the Observational Gait Scale (OGS). Two clinicians viewed edited split-screen video recordings of 20 children/adolescents (11 males, 9 females; mean age 12 years, range 6 to 21 years) made at the time of three-dimensional gait analysis (3-DGA). Walking ability in each child was scored at initial assessment and reassessed from the same videos three months later using the first seven sections of the OGS. Validity of the OGS score was determined by comparison with 3-DGA. The OGS was found to have acceptable interrater and intrarater reliability for knee and foot position in mid-stance, initial foot contact, and heel rise with weighted kappas (wk) ranging from 0.53 to 0.91 (intrarater) and 0.43 to 0.86 (interrater). Comparison with 3-DGA suggests that these sections might also have high validity(wk range 0.38–0.94). Base of support and hind foot position had lower interrater and intrarater reliabilities (wk 0.29 to 0.71 and wk 0.30 to 0.78 respectively) and were not easily validated by 3-DGA.

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–hyperactivity 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.

Differences in motor development and the relationship between motor and language development were studied in 88 children with familial risk for dyslexia (43 females, 45 males; at-risk group) and 88 children without familial risk for dyslexia (35 females, 53 females; control group; n=176) during the first two years of life. A structured parental questionnaire was used to assess motor development. Expressive language skills were assessed at the age of 18 months with the Reynell Developmental Language Scales and at 18 and 24 months with the MacArthur Communicative Development Inventories. At group level, the motor development of children in both the at-risk and control groups was similar. However, motor development showed a different pattern in these groups. Cluster analyses revealed three clusters in the control group: ‘fast motor development’, ‘slow fine motor development’, and ‘slow gross motor development’. In the at-risk group, only two clusters were found: ‘slow motor development’ and ‘fast motor development’. A significant difference (p=0.03) was found between the clusters in the MacArthur Communicative Development Inventories. Children with familial risk for dyslexia and slow motor development had a smaller vocabulary and produced shorter sentences than all the other children. Associations between motor and language difficulties are discussed.

The aim of this study was to: (1) describe the usual mobility methods of children with cerebral palsy (CP) at home, school, and outdoors or in the community and (2) examine whether children with CP are more dependent on adult assistance for mobility in certain settings. The participants were a stratified random sample of 636 children with CP (355 males and 281 females; 2 to 12 years of age, mean 6.8 years SD 2.7), receiving rehabilitation services in Ontario, Canada. Children were grouped by age and Gross Motor Function Classification System (GMFCS) level. Among the five levels of the GMFCS, there were 185 children classified at level I, 81 children at level II, 113 children at level III, 132 children at level IV, and 125 children at level V. Information on children's usual mobility was obtained by parent report. The results of logistic regression indicated that compared with the school setting, children were more dependent on adult assistance for mobility when outdoors/in the community and less dependent at home. The majority of children aged from 4 to 12 years at levels III to V used wheelchair mobility at school and outdoors or in the community, however, only a small percentage self-propelled their wheelchair or used powered mobility. Of the children aged 4 to 12 years at level V, 39% were carried at home. The findings suggest that environmental setting is an important consideration for assessment and intervention to improve mobility of children with CP. For children who do not walk, attention should be given to the needs of caregivers and factors that are important for successful powered mobility.

The aim of the study was to determine audiological function at 14 years of age of very-low-birthweight (VLBW [les ]1500g) children compared with a cohort of normal birthweight (NBW >2499g) children. Participants were consecutive surviving preterm children of birthweight <1000g born between 1977 and 1982 (n=86) and of birthweight 1000 to 1500g born between 1980 and 1982 (n=124) and randomly selected NBW children born between 1981 and 1982 (n=60). Audiometric tests included pure tone audiometry, tympanometry, stapedius muscle reflexes, and measures of central auditory processing. Psychometric tests included measures of IQ, academic achievement, and behaviour. There were no significant differences in rates of hearing impairment, abnormal tympanograms, figure–ground problems, or digit recall between VLBW children and NBW control children. VLBW children had higher rates of some central auditory processing problems, which in turn were associated with poorer intellectual, academic, and behavioural progress.

This study aimed to investigate the prevalence of undernutrition in children with cerebral palsy (CP) and to determine the relation with feeding ability. Ninety children with CP from special needs schools were examined. Undernutrition was diagnosed on one or more of the following criteria: weight <2nd centile, triceps or subscapular skinfold measurement <3rd centile, mid-arm circumference <5th centile. Feeding competence was scored with respect to seven specific oromotor tasks using the Multidisciplinary Feeding Profile. Thirty-six participants (40%) had diplegia, 29 (33%) quadriplegia, 13 (14%) hemiplegia, and 12 participants (13%) had dyskinetic CP. Age ranged from 2.6 to 18.7 years (mean 10.8 years). Forty-six percent (41 of 90) were undernourished. In all aspects of feeding, those undernourished had lower feeding competence scores compared to adequately nourished children (p<0.002). Each modality of feeding competence correlated significantly to the centiles of weight, triceps or subscapular skinfold measurement and mid-arm circumference (p<0.02). A positive association of weight, triceps skinfold measurement, and mid-arm circumference with chewing ability was present independent of other feeding modalities (p<0.05). Undernutrition was common in this group and was associated with poorer feeding ability.

Impact of white matter abnormalities (WMAs) on neuropsychological functioning in children with early-treated phenylketonuria (ETPKU) was examined. Children with ETPKU (20 males, 12 females, mean age 11 years 2 months, SD 3 years 6 months) and controls (20 males, 14 females, mean age 10 years 4 months, SD 3 years 1 month) aged 7 to 18 years were assessed using tests of attention, processing speed, memory and learning, executive function, and academic achievement. Those with ETPKU, exhibiting WMAs extending into subcortical/frontal regions (n=14), displayed significant impairments in a number of domains. Children with ETPKU but no WMAs (n=6), or pathology restricted to the posterior periventricular region (n=12), displayed only mild deficits. Concurrent phenylalanine levels correlated weakly with cognitive parameters, whereas lifetime phenylalanine levels were associated with deficits in several cognitive domains. Impairments in children with extensive WMAs are consistent with compromised neural transmission, which is characterized by dysmyelination. However, children with no detectable, or mild WMAs, also displayed cognitive problems, indicating that neuropsychological functioning in children with ETPKU is determined by a complex interaction of biological and environmental factors.

Interest in the area of cerebral palsy (CP) and electrical stimulation continues to grow because it has potential as a passive, non-invasive, home-based therapy, which is claimed to result in gains in strength and motor function. If proved effective it might provide an alternative to resistive exercise techniques for children with poor selective muscle control, or indeed it might improve treatment compliance in those children who find exercise programmes difficult. Unfortunately, early reports on the efficacy of this intervention are undermined by poor methodology. A lack of consensus on optimal treatment parameters and variation in the physical abilities of the participants further confound interpretation of the literature.

‘Cerebral palsy’ is an emotive term, which to parents often has the same impact as cancer or other dreaded diagnoses. For a child it is a label which can open doors to extra provision in schools and in the community, but which in itself may mean segregation, causing difficulties in participation. For a clinician it can be a diagnostic trap due to the failure to consider other conditions that impair motor function. For both clinicians and researchers it can be difficult to specify. Concerned about limitations of the current concept, a group of selected experts met last year to discuss the definition and classification of cerebral palsy. The preliminary results of their work are printed in this issue (p 571–576), with accompanying invited personal commentaries. We hope that it will provoke vigorous discussion.