Five children (three males, two females; four right-, one left-handed; age range 6 to 14 years) who developed aphasia after gross-total excision of left predominantly thalamic tumors are reported. Three patients had Broca aphasia, one had mixed transcortical aphasia, and one patient had conduction aphasia. In the months after surgery three children improved while receiving radiation and/or chemotherapy, although none recovered completely. Two patients with malignant tumors developed worsening aphasia when the tumor recurred and later died. Two patients (of three tested) had visuospatial difficulties in addition to language deficits. Attention and executive functioning were affected in three of three patients tested. Memory, verbal and/or visual functioning, were affected in four of four patients tested. Both patients who were tested showed transient right hemineglect. Two (of two tested) were probably apraxic. The wide range of deficits in these children highlights the importance of the thalamus and other subcortical structures in developing cognition.

High levels of alcohol consumption during pregnancy affect the development of the baby's central nervous system. Pregnant women were interviewed about their alcohol consumption during pregnancy, when they first visited the maternity hospital in Roubaix, France. Of the 698 women interviewed, 156 of their children were investigated at the age of 4 ½ years with a standardised examination to assess the effects of prenatal alcohol exposure on neurological status. Two scores were calculated: a posture score, which measured items while the child was standing; and a minor neurological signs score. The posture score was not related to prenatal alcohol exposure. Consumption of 21 drinks/week (3 drinks/day) or more during pregnancy was significantly associated with a higher number of minor neurological signs, after controlling for relevant covariables. Although prenatal alcohol exposure was related to a lower General Cognitive Index (GCI), minor neurological signs were associated with prenatal alcohol exposure after controlling for GCI.

We have established a multisite, international database of 3500 individuals diagnosed with Tourette syndrome (TS). The male:female ratio is 4.3:1 for the total sample, with wide variation among sites; the male excess occurs at every site. Anger control problems, sleep difficulties, coprolalia, and self-injurious behavior only reach impressive levels in individuals with comorbidity. Anger control problems are strongly correlated with comorbidity, regardless of site, region, or whether assessed by neurologists or psychiatrists. The mean age at onset of tics is 6.4 years. At all ages, about 12% of individuals with TS have no reported comorbidity. The most common reported comorbidity is attention-deficit–hyperactivity disorder. Males are more likely to have comorbid disorders than females. The earlier the age at onset, the greater the likelihood of a positive family history of tics. An understanding of the factors producing these and other variations might assist in better subtyping of TS. Because behavioral problems are associated with comorbidity, their presence should dictate a high index of suspicion of the latter, whose treatment may be at least as important as tic reduction. The established database can be used as the entry point for further research when large samples are studied and generalizability of results is important.

Functional electrical stimulation (FES) of the upper limb has been used for patients with a variety of neurological conditions, although few studies have been conducted on its use on the upper limb of children with cerebral palsy (CP). The aim of this study was to investigate the effect of cyclic FES on the wrist extensor muscles of a group of eight children (five boys, three girls) with hemiplegic CP (mean age 10 years). The study design involved a baseline(3 weeks), treatment (6 weeks), and follow-up (6 weeks). FES was applied for 30 minutes daily during the treatment period of the study. Improvements in hand function (p[ges ]0.039) and active wrist extension (p=0.031) were observed at the end of the treatment period. These improvements were largely maintained until the end of the follow-up period. No significant change was observed in the measurements of wrist extension moment during the treatment period (p=0.274). Hand function in this group of children improved after they were exposed to FES of wrist extensor muscles. This suggests that FES could become a useful adjunct therapy to complement existing management strategies available for this patient population.

The purpose of this investigation was to quantitatively compare synergistic movement patterns between seven children (four male, three female; aged 3 to 17 years; mean 6.7, SD 5.3) without cerebral palsy (CP) (controls) and 27 children (15 male, 12 female; aged 2 to 16 years; mean 5.7, SD 3.7) with spastic diplegic CP before and after selective dorsal rhizotomy (SDR). The study design was also descriptive, comparing results of before and after SDR to control children. A two dimensional video system and retroreflective markers were used to obtain sagittal plane angles for the hip, knee, and ankle during maximum active knee flexion and extension. Correlations were calculated between the knee and hip and between the knee and ankle joint pairs. Control children demonstrated non-synergistic movement patterns (–0.75 and –0.61). These results were significantly different from children with CP (0.40 and 0.43, p<0.05). Eight months after SDR, synergistic patterns did not significantly change from preoperative results (0.23 and 0.36, p>0.05) and remained significantly different from control children (p<0.05). We conclude that it may not be possible to significantly alter synergistic patterns after SDR.

The aim of this study was to assess psychomotor development, using Griffiths' test, and the incidence of minor anomalies at birth in children who had been exposed to antiepileptic drugs (AEDs) in utero. The study sample comprised 100 children of mothers who were treated with AEDs during pregnancy and 100 matched control children. Women with epilepsy were recruited from a pregnant urban population (450 000 inhabitants). The lowest possible dose of the fewest AEDs to maintain seizure control was used. Drug levels were controlled on a monthly basis. The children were assessed at 9 months of age. The study children had a significant increase in the number of minor anomalies (31 compared with 18 control children; odds ratio 2.4, CI 1.15 to 5.02, P=0.02 McNemars test), and an increased number of facial anomalies after carbamazepine exposure (11 compared with six control children). Drug exposure did not influence the Griffiths' score at 9 months of age. Even a meticulous AED treatment strategy during pregnancy increases the number of minor anomalies. However, treatment with AEDs does not necessarily influence short-term psychomotor development.

This study was designed to investigate birth defects found in association with newborn encephalopathy. All possible birth defects were ascertained in a population-based study of 276 term infants with moderate or severe encephalopathy and 564 unmatched term control infants. A strong association between birth defects and newborn encephalopathy was found with defects affecting 27.5% of children with encephalopathy and 4.3% of control children (odds ratio 8.55; 95% confidence interval 5.25 to 13.91; p<0.001). In 11.8% of infants with a birth defect the defect was not diagnosed until after the newborn period, illustrating one of the difficulties in attempting to exclude infants with birth defects from studies of newborn encephalopathy. The majority of defects (89%) were not specific anomalies of the CNS. In 36.8% of children with encephalopthy who had a birth defect, the defect was considered to be the probable cause of the encephalopathy. Infants with birth defects who had encephalopathy had a poorer prognosis than those without: they were twice as likely to die by the age of 2 years and three times more likely to have cerebral palsy. This study catalogues the spectrum of birth defects associated with newborn encephalopathy and illustrates the importance of their inclusion when investigating both the aetiology and outcome of this condition.

An EMG and nerve-conduction-study protocol has been developed and used in all patients with brachial plexus lesions since 1985. The findings of EMG and nerve-conduction studies in obstetric brachial palsy (OBP) mostly suggest a falsely optimistic prognosis. To analyse this, all subjects were selected with complete avulsion of both roots C5 and C6 and/or complete rupture of the upper trunk verified during operation from a group of 162 infants with OBP (aged 4 to 14 months) and a group of 184 child and adult patients (aged 6 to 74 years) with a traumatic brachial plexus lesion. Fourteen infants, approximately 4 months old, with OBP, and 19 adults (aged 16 to 30 years) met the selection criteria. The infants from the group with OBP had at 4 months a nearly normal recruitment pattern of motor units in the biceps brachii and deltoid muscles, with little or no denervation. However, in the older group with traumatic brachial palsy, the same lesion caused complete denervation of both muscles. From the group with OBP, a third group (N=3) with the same lesion plus an additional lesion of C7 or a rupture of the middle trunk was selected. This additional lesion resulted in nearly complete denervation of both muscles. This means that C7 largely contributes to the innervation of both muscles at the time of birth, but this innervation is lost during later life in normally developing individuals (apoptosis). A central mechanism might be responsible for the clinical palsy and later spontaneous improvement which is always found in the infants with OBP.

To document the characteristics of early posttraumatic seizures (EPTS) in non-accidental head injury (NAHI), and examine their relation with outcome, a retrospective study was carried out. All children with NAHI admitted to the Royal Hospital for Sick Children, Edinburgh since 1981 were identified. The characteristics of EPTS, EEG, and outcome were noted. Forty-four cases were identified. The average age of children at presentation was 5.9 months. Thirty-two of these children had EPTS. The median length of follow-up was 3.0 years. The mortality rate was six in 44 (14%). The neurodevelopmental outcome correlated significantly with the presence and severity of EPTS (Tau = 0.317, p=0.017). Of survivors, 22% developed late posttraumatic epilepsy, the outcome in those with epilepsy was significantly worse than those without (p<0.0001). It was concluded that the severity of the primary brain injury dictates the severity of the EPTS and neurodevelopmental status at follow-up.

The purpose of this study is to compare the work experiences of adolescents with spinal cord injuries (SCI) to peers without disabilities. Twenty-eight adolescents with SCI and 25 age-matched individuals without disabilities (controls) took part in the study. A structured telephone questionnaire was used to assess their experience of household chores, volunteer jobs, and paid work. The mean age at interview was 16.2 years for those with SCI and 16.0 years for control participants. There was no significant difference between the individuals with SCI and the control participants in chores or volunteer work experiences, but the individuals with SCI were significantly less likely to have paid work experience than control participants (χ2 = 13.670, p<0.001). Of the older adolescents, aged >15 to 19 years, five of 15 individuals with SCI and 16 of 16 control participants had paid work (Fisher exact test p<0.001). Of individuals with SCI, those with milder injuries and those injured at a younger age were more likely to have work experience

To evaluate the clinical results of the treatment and to assess the factors that influenced the rate of scoliosis progression, a retrospective study of spinal orthosis in 86 patients with spastic quadriplegic cerebral palsy was performed. The mean age of the patients was 13.8 years (range 5 to 33 years). Their scoliotic deformities were treated with custom-moulded, polypropylene thoraco-lumbar-sacral orthoses. Cobb angles were measured on radiographs taken in a sitting position before treatment, in orthosis, and during follow-up. The mean initial Cobb angle was 68.4° (range 25 to 131°). The mean correction in orthosis was 25° (range 3 to 60°). Seventy-two patients had a follow-up period of more than 2 years. At the latest follow-up, average 6.3 years (range 2 to 14 years) after the start of treatment, the mean Cobb angle without orthosis was 93.1° (range 40 to 145°). The mean progression per year was 4.2° (range –3 to 21°). Linear multiple regression revealed that age and initial correction in orthosis were the only variables that significantly influenced the rate of progression. Twenty-two patients had no progression or progression <1.0° per year. Correction in orthosis was the only variable that predicted progression <1.0° per year in both age groups (<15 years and [ges ]15 years). Of the 57 patients who were still alive and had not undergone surgical fusion, 72% used their orthoses at a mean age of 22 years. Parents and caregivers expressed satisfaction with the use of orthosis, mainly because of improved sitting stability which gave better overall function.

To compare the functional maturation of the human visual system between intra- and extrauterine course flash visual evoked potentials (VEPs) in preterm infants (gestational age 24 to 36 weeks). Previously established normal values, with special reference to the two components of the N1 wave, were employed (Tsuneishi 1995). A cross-sectional analysis of 124 infants at 36 weeks postmenstrual age (PMA), showed that there are no differences in the absolute values of VEP peak latencies depending on the postnatal age (PNA). Conversely, the N1 wave form changes with increasing PNA from a wave in which the early peak (N1a) has a higher amplitude than the late peak (N1b) into the reverse situation with a higher amplitude of the N1b as compared to N1a. This observation may correlate with the maturation of the neuronal networks in the visual cortex. In a longitudinal analysis of 50 infants followed for more than 5 sessions of weekly recordings, we found that the individual rapid decrease in the N1a latency, which may reflect the initiation of myelination in the optic radiation, most frequently occurs at around 37 weeks PMA, regardless of PNA. Preterm extrauterine visual experience has little effect on the myelination process in the visual pathway, but has a marked effect on the developmental changes in VEP wave form which reflect the developmental changes of the neuronal networks in the visual cortex.

No previous studies have used morphological neuroimaging to compare children with ADHD with siblings of children with ADHD. To test the hypothesis that the total size of the corpus callosum is altered in children with hyperkinetic disorder, the corpus callosum was outlined from a single midline proton-density weighted slice (containing the septum pellucidum). Fifteen boys with a refined phenotype of ADHD (mean age 10.2 years) and 15 healthy male siblings of children with ADHD (mean age 10.6 years) were enrolled in the study. The two groups were compared for global brain size and the callosal areas of Witelson. No significant differences were found between the study and comparison groups for any of the corpus callosum areas, even after age, global brain size, and handedness were covaried (using MANOVA). In addition, corpus callosum sizes do not seem to differ between children with ADHD and unaffected siblings of children with ADHD. Clinicians should not base their pathophysiological diagnosis of this disorder on an abnormality of callosal development.

A neuropsychological investigation of the association between specific language impairment (SLI) and hyperactivity was conducted on four groups of 6-year-old children. The groups, each consisting of five boys and five girls, were: children with SLI alone, hyperactivity alone, both SLI and hyperactivity, and neither disorder (controls). Cognitive functioning was examined using selected non-verbal tests from the Cambridge Neuropsychological Test Automated Battery. SLI was not associated with significantly reduced performance on any of the neuropsychological measures. However, hyperactivity was associated with deficits on a test of attentional set shifting previously demonstrated to be sensitive to frontostriatal dysfunction. Further, hyperactive children showed significantly reduced spatial spans on a test of spatial working memory thought to measure parietal lobe functioning. There were no substantial differences between the groups on tests of frontal lobe function except for reduced performance on a task of attention in the hyperactive group. There were no significant interactions between hyperactivity and SLI on any of the neuropsychological measures. The pattern of neuropsychological deficits shown by children with attention-deficit–hyperactivity disorder (ADHD) suggests relatively specific problems in inhibitory control of attentional selection. This cognitive deficit implicates brain areas including basal ganglia and ventro-lateral prefrontal cortical areas in ADHD, thus providing evidence for disruption of frontostriatal functional loops. SLI and hyperactivity can occur as comorbid syndromes or symptom complexes and this should be noted when interpreting results of tests measuring cognitive performance. The findings suggest that these two groups of disorders have different cognitive correlates.

Mirror movements in individuals with hemiplegic cerebral palsy (CP) may result from a reorganization of the central sensorimotor system. Motor performances of both hands were measured to characterize mirror activity (or mirroring) and hand functions in 22 participants (6 to 18 years) with hemiplegic CP and in 17 control participants. During a unimanual repetitive squeezing task, contractions of the active hand and fingertip forces of the opposite hand were recorded simultaneously. In the control group, slight mirror activity (or mirroring) was found that decreased with age. In participants with CP, mirror activity was 15 times stronger than in the control group, and was found at all age levels. Mirroring was more prominent in the unaffected hand of the CP group. The amount of mirror activity was not related to the degree of hemiplegia, which was assessed with measures of spasticity, strength, and dexterity. Mirror movements disturbed functional bimanual skills, although to some extent they could be suppressed by voluntary effort.

Recent studies on hand motor control in children with cerebral palsy (CP) have focused on the coordination of fingertip forces during the grasping and lifting of objects. However, little is known about the ability to replace and release an object from grasp, a function that is just as important for fine dexterity. The present study examines the coordination of fingertip forces during the replacement and release of an object (either 200 g or 400 g) from grasp in 14 children (aged between 7 and 13 years) with hemiplegic CP and in 14 age-matched typically developing children. The results indicate that children with hemiplegic CP abruptly replaced the object but had a prolonged and uncoordinated release of the grasp. Unlike what was seen in the control children, the grip and load forces decreased sequentially in the children with CP. However, all the children could appropriately scale the rate of force decrease based on somatosensory weight-related information from the ongoing lift. The results provide further information about the impaired hand skills in children with hemiplegic CP.

This study aims to evaluate the use of the United States National Center for Health Statistics (NCHS) weight-for-height centiles (WHC) in screening children with cerebral palsy (CP) for depleted body fat and to identify an alternate screening method. Growth data from 276 children aged from 3 to 12 years with CP were analyzed retrospectively. Height or a proxy for height, mid-upper arm circumference, weight, and skinfold thicknesses were recorded. Mid-upper arm fat area was calculated for each participant. The sensitivities and specificities of WHC and a number of alternative anthropometric screening methods for identifying participants with severely depleted fat stores were determined. WHC <10th centile failed to identify 45% of children with severely depleted fat stores. Triceps skinfold thickness <10th centile identified 96% of malnourished children. WHC standards lack adequate sensitivity for identification of severely depleted fat stores in children with CP. Use of triceps skinfold thickness, using cut-off value of <10th centile for age and sex, is recommended to screen for suboptimal fat stores in children with CP.

Little is known about the developmental course of striking action. This cross-sectional study explored the refinement of striking in 28 children aged between 4 and 12 years and investigated how well they could use kinaesthesis to gauge the length of an unseen bat. The kinematic data (including smoothness of movement) showed quantitative differences between the age groups. In contrast, no differences were found in the children's ability to judge the length of the unseen bat: within three strikes all of the children had made a clean hit, indicating that they had successfully judged bat length. The children then appeared to memorize the bat with which they had accurately hit the target and made: (1) minimal errors when using this bat in later trials and (2) predictable errors when using two other bats of different sizes. The results show that the striking action becomes optimized over childhood, with smoothness of movement providing an index of this refinement. The findings also suggest that young children have a higher level of kinaesthetic sensitivity than has been assumed previously on the basis of static limb positioning tasks. The results suggest that the striking task used in this study might be a useful tool for investigating the development of movement skills in children with developmental disorders.

The aim of this study was to determine whether there are primary effects of prematurity on the development of explicit memory. Elicited imitation of action sequences was used to compare immediate and 15-minute delayed memory in term and preterm infants (19 months corrected age; n=48) who were at low risk: none had experienced the medical or social risk factors often associated with preterm birth. Relative to infants born at term (38 to 40 weeks' gestation), children who had been born at 27 to 34 weeks' gestation showed lower levels of ordered recall; performance of healthy infants born at 35 to 37 weeks' gestation was intermediate and did not differ significantly from that of the other groups. These results suggest that specific cognitive deficits can occur as a function of preterm birth even in low-risk infants.

The increased survival of sick and preterm neonates may be associated with long-term problems which must be recognised and managed if outcome is to be optimised. In a prospective study of 35 neonates (median gestational age at birth 34 weeks) admitted to a neonatal intensive care unit over a 3-month period, we have documented a high incidence (14 of 35) of immature or abnormal feeding patterns when infants were assessed at 36 to 40 weeks postmenstrual age. Neonates with prolonged respiratory support and delayed enteral and oral feeding were most affected. Compared with neonates who have normal initial feeding assessments, neonates with disorganised or dysfunctional feeding were six times more likely to vomit and three times more likely to cough when offered solid food at 6 months of age. At 12 months of age significant differences were also found in tolerating lumpy food and enjoying mealtimes. We hypothesise that these feeding problems contribute to failure to thrive and psychosocial distress after discharge from the neonatal unit and propose potential neonatal measures to reduce their incidence.