Parents and paediatric neurologists need information on the long-term social prognosis of children with cerebral palsy (CP). No large population-based study has been performed on this topic. On 31 December 1999, to find predictors in childhood of subsequent education and employment, 819 participants with CP born between 1965 and 1978 (471 males; mean age 28y 10mo, SD 4y, range 21 to 35y) in the Danish Cerebral Palsy Registry were compared with 4406 controls without CP born between 1965 and 1978 (2546 males; mean age 28y 10mo, SD 4y, range 21 to 35y). Diagnostic subtypes of the 819 participants with CP were: 31% hemiplegia, 43% diplegia, 18% tetraplegia, and 8% other types. Level of motor impairment with respect to walking ability was: 62% able to walk without assistance, 21% with assistance, and 16% not able to walk (for 1% of study children walking ability was not known). Relevant information was obtained from Denmark's unique registries. Of the participants with CP, 33% vs 77% of controls, had education beyond lower secondary school (i.e. after age 15–16y), 29% were competitively employed (vs 82% of controls), 5% were studying, and 5% had specially created jobs. Excluding participants with CP with an estimated developmental quotient (DQ) of less than 50 or inability to walk at age 5 to 6 years, the odds ratios (multivariate analysis) for not being competitively employed were 1.9 for diplegia versus hemiplegia, 22.5 for DQ 50 to 85 versus DQ greater than 85, and 3.7 for those with epilepsy versus those without epilepsy. The severity of motor impairment among participants with CP able to walk had just a minor influence. Only half the participants with CP who had attended mainstream schooling were employed. In conclusion several childhood characteristics seemed to predict long-term social prognosis.

The Pediatric Pain Questionnaire and relevant medical records were used to investigate the frequency, intensity, location, and duration of pain experienced by children with spina bifida and to examine the concordance between child self-report and parent proxy report of pain. There were 68 children with spina bifida (30 males, 38 females) between the ages of 8 and 19 years (mean age 12y 8m). Fifty-nine children (87%) were diagnosed with myelomeningocele, six (9%) with lipomyelomeningocele, and three (4%) with lipomeningocele (4%). Forty-nine children (72%) had shunted hydrocephalus. Twenty-six children (38%) ambulated full-time. Fifty-six per cent of children reported experiencing pain once a week or more often. For these children, pain occurred most frequently in the head, back, abdomen, neck, shoulders, legs, and hands. Of the 49 children with shunted hydrocephalus, 43 (88%) reported headaches; 15 of 19 children (79%) without hydrocephalus reported headaches. Children who reported more intense pain also reported more frequent pain and more pain locations. Parents were most reliable at reporting locations of their children's severe pain. Parents of younger children significantly underestimated their children's current pain and worst pain in the past 7 days. Lesion level and ambulatory status were not associated with any of the pain variables. It was concluded that children with spina bifida frequently report clinically significant, yet under-recognized and untreated pain.

Little is known about the development of postural adjustments during early ontogeny. We examined postural adjustments due to sudden perturbations during sitting in 40 healthy term infants (28 males, 12 females) assessed in groups of eight at 1, 2, 3, 4, and 5 months of age. Surface electromyograms of neck, trunk, and leg muscles were recorded while the infants were exposed to a random series of horizontal forward and backward displacements of the surface of support. Video recordings of spontaneous motor behaviour were analyzed. For part of the analyses, previously collected data on 26 infants aged 6 to 10 months were included. In general, postural adjustments at all ages were direction specific and showed large variation. Within the variation developmental changes could be observed, revealing a transient decrease in postural activity at 3 months of age. After this transition, direction-specific postural activity was correlated with spontaneous motor behaviour. This was true, in particular, for dorsal postural activity. The clinical relevance of these findings is discussed.

We have previously reported that significant hyperopia at 9 months predicts mild deficits on visuocognitive and visuomotor measures between 2 years and 5 years 6 months. Here we compare the motor skills of children who had been hyperopic in infancy (hyperopic group) with those who had been emmetropic (control group), using the Movement Assessment Battery for Children (Movement ABC). Children were tested at 3 years 6 months (hyperopic group: 47 males, 63 females, mean age 3y 7mo, SD1.6mo; control group: 61 males, 70 females, mean age 3y 7mo, SD1.2mo) and at 5 years 6 months (hyperopic group: 43 males, 56 females, mean age 5y 4mo, SD1.7mo; control group: 51 males, 62 females, mean age 5y 3mo, SD1.6mo). The hyperopic group performed significantly worse at both ages, overall and on at least one test from each category of motor skill (manual dexterity, balance, and ball skills). Distributions of scores showed that these differences were not due to poor performance by a minority but to a widespread mild deficit in the hyperopic group. This study also provides the first normative data on the Movement ABC for children below 4 years of age, and shows that it provides a useful measure of motor development at this young age.

Ocular motor apraxia (OMA), a disorder of saccadic initiation, may be congenital or acquired. While the acquired form is frequently associated with significant neuropathology, the congenital form is often regarded as relatively benign. Many children with congenital OMA who were observed clinically have shown neurodevelopmental disturbance over time. A retrospective review was taken of 34 consecutive patients (22 males and 12 females), seen over a 20-year period, to evaluate the frequency and type of associated neurodevelopmental problems. Age at presentation ranged from 8 weeks to 14 years, with a mean age of 10 years. Of 29 children with congenital OMA, 15 had imaging evidence of structural central nervous system abnormalities (with cerebellar hypoplasia the most frequent abnormality detected). Eleven of the 14 patients with no structural abnormality showed abnormal neurodevelopment. This study suggests that congenital OMA is not a benign diagnosis, even in the absence of overt neurological disturbance at the time of presentation.

In this pilot study we investigated the feasibility of The Infant Behavioral Assessment and Intervention Program (IBAIP) in a group of preterm infants. At the age of 6 months, the neurobehavioural organization and self-regulatory competence of an intervention group was compared with a control group who had received the standard follow-up care. The intervention group consisted of 13 males and seven females (mean gestational age [GA] 29.2 weeks, SD1.3wks; mean birthweight 1232g, SD320g). The control group consisted of 11 males and nine females (mean GA 29wks, SD1.6wks; mean birthweight 1198g, SD397g). Inclusion criteria were: a GA of 32 weeks and family residence in the district of Amsterdam. Exclusion criteria were: severe congenital abnormalities, intraventricular haemorrhage grade III or IV, periventricular leukomalacia grade III or IV, and infants whose mothers had a history of illicit drug use. The intervention infants received 6 to 8 IBAIP interventions at home, from discharge until 6 months of age. The Neonatal Behavioral Assessment Scale was administered at term; the Infant Behavioral Assessment (IBA) at term, 3, and 6 months of age; and the Bayley Scales of Infant Development–II at 3 and 6 months (corrected age). At 6 months, intervention infants showed less stress and more approach behaviours on the IBA compared with control infants. These promising results warrant further evaluation in a randomized controlled trial.

This study investigated mortality and causes of death between 1988 and 1999 in 14781 persons (6702 female) with Down syndrome in California, comparing age, sex, ethnicity, and other factors. Mean age at the start of follow-up was 14 years 8 months (SD 14y 10mo). During the study period 600 persons died. The standardized mortality ratio (SMR) for the population was 5.5. Blacks were at greater risk than whites, Hispanics, or Asians (relative risk=1.5). Mortality declined during the period, especially for children with congenital heart defects. Leukemia (SMR=17), respiratory illnesses (SMR=27), congenital anomalies (SMR=72), and circulatory diseases (SMR=5.3) accounted for most of the excess mortality. With the exception of leukemia, cancer mortality was not different from that of the general population.

The aim of the study was to determine whether pediatric patients with cerebral palsy (CP; 24.3% hemiplegic, 30.4% diplegic, 38.5% quadriplegic) can self-report their health-related quality of life (HRQOL) with the Pediatric Quality of Life Inventory Version 4.0 (PedsQL 4.0) Generic Core Scales. The PedsQL was administered to 148 families (84 males, 64 females; mean 10y, SD 3y 9mo, range 5 to 18y) recruited from a children's hospital clinic and state medical therapy clinics. Sixty-nine children (47%) were able to self-report. Children with CP self-reported a significantly lower HRQOL (mean 65.9) than healthy children (mean 83.8). Children with CP self-reported a similar HRQOL to pediatric cancer patients receiving treatment (mean 68.9). Children diagnosed with quadriplegia (mean 49.4) self-reported more impaired HRQOL than children with diplegia (mean 69.1) and hemiplegia (mean 72.4). Parent–child concordance was lowest for emotional functioning. Providers and parents should obtain the children's HRQOL perceptions whenever possible.

This study explored possible neural mechanisms that contribute to improvements in balance control produced by reactive balance training in children with cerebral palsy (CP). Six children with CP (four males, two females; mean age 9y 4mo), two with spastic hemiplegia (Gross Motor Function Classification System [GMFCS] level I) and four with spastic diplegia (GMFCS level II,) were given 5 days of intensive training in reactive balance control (100 perturbations per day on a moveable force platform). Surface electromyography was used to characterize changes in neuromuscular responses pretraining, immediately posttraining, and 1 month posttraining. Training in reactive balance control resulted in improvements in directional specificity of responses (a basic level of response organization) and other spatial/temporal characteristics including: (1) faster activation of muscle contraction after training, allowing children to recover stability faster; (2) emergence of a distal–proximal muscle sequence; and (3) improved ability to modulate the amplitude of muscle activity (increased amplitude of agonist and decreased amplitude of antagonist, reducing coactivation). Each child with spastic hemiplegia or diplegia showed a different combination of factors that contributed to improved performance; the level of change in neural factors depended on the severity of involvement of the child: hemiplegia vs diplegia, and level of involvement within each diagnostic category.

the aim of this study was to establish the rate and spectrum of psychiatric disorder among children before and after temporal lobe surgery for epilepsy. data were examined for associations between psychopathology and seizure outcome following surgery, or association between psychopathology and other variables, such as laterality of lesion, sex, cognitive level, and underlying pathology. participants were 60 children (35 males, 25 females) who had focal seizures of temporal lobe origin and who had undergone temporal lobe resection between 1992 and 1998; mean age at time of operation 10y 7mo, (sd 4y 11mo) range 7mo to 17y 11mo. mean length of follow-up was 5.1 years (sd 2.3, range 2 to 10y). twenty-eight (47%) children had undergone right temporal lobectomy. diagnosis of a psychiatric disorder was present in 50/60 (83%) children at some point, with high rates of psychiatric comorbidity. common childhood psychiatric disorders of attention-deficit–hyperactivity disorder, oppositional defiant disorder/conduct disorder, and emotional disorders were present in about 25% of children. disorders rarely seen in the general child population were over-represented: disruptive behaviour disorder – not otherwise specified (30/60 [50%]), and pervasive developmental disorder (autistic spectrum disorder; 23/60 [38%]). there was no significant relationship between pathology, sex, seizure frequency, or postoperative seizure outcome and psychiatric disorder, other than for pervasive developmental disorder. the same proportion of children had psychiatric diagnoses pre- and postoperatively (43/60 [72%] and 41/57 [72%] respectively). although mental health problems are common in children undergoing temporal lobe resection, there are few predictors of psychiatric outcome following epilepsy surgery. parents require counselling on these issues in the preoperative work-up.

We have developed a gait nomogram based on dynamic similarity to characterize and compare neuromuscular function. We used temporal–distance data based on 669 normal participants (age range 5 to 98 years), and 78 children and young adults with spastic diplegia (43 males, 35 females; mean age 10y 8mo, standard deviation 3y 11mo, range 5 to 20y), all of whom were independent ambulators. A new statistical algorithm known as fuzzy clustering was implemented and five cluster centres were identified, each representing distinct walking strategies adopted by children with cerebral palsy. Using just three easily obtained parameters – leg length in metres, stride length in metres, and cadence in steps per minute – our program calculates a child's dimensionless step length and step frequency, generates the individual's membership values for each of the five clusters, and plots the gait nomogram. The clinical utility of our approach has been demonstrated for two test participants with spastic diplegia, using pre- and postoperative data (one neurosurgical and one orthopaedic), where changes in membership of the five clusters provide objective measures of improvement in their neuromuscular function.

The concurrent validity of a 1 minute walk test at a child's maximum walking speed was assessed in children with bilateral spastic cerebral palsy (BSCP). The distance covered during the 1 minute walk test was compared with the children's gross motor function as assessed by the Gross Motor Function Measure (GMFM). Twenty-four male and 10 female children with CP (mean age 11y, range 4 to 16y) participated in the study. Gross Motor Function Classification System (GMFCS) levels were; level I (n=3), level II (n=17), level III (n=10), and level IV (n=4). Participants had clinical diagnoses of symmetrical diplegia (n=19), asymmetrical diplegia (n=14), and quadriplegia (n=1). Results showed a significant correlation between GMFM score and the distance covered during the 1 minute walk (r=0.92; p<0.001). There was also a significant decrease in the distance walked with increasing GMFCS level (p<0.001). We concluded that the 1 minute walk test is a valid measure for assessing functional ability in children with ambulatory BSCP. Its cost-effectiveness and user friendliness make it a potentially useful tool in the clinical setting. Further study needs to address its reliability and ability to detect change over time.

The purpose of this study was to investigate cognitive and other disabilities in children and adolescents with 22q11 deletion syndrome. Thirty-three children (15 females, 18 males; age range 3 to 19y, median 7y 6mo) with 22q11 deletion were investigated for growth, development, neurology, cognition, motor function, and participation (measured as handicap). Half of the children had never crawled, although they had shuffled, and commencement of walking was delayed (mean 18mo, SD6mo). Hypotonia was found in 25 and poor balance in 24 of the 33 children; 17 out of 27 had definite motor problems, including two with spastic hemiplegia. Intelligence quotient (IQ) range was 50 to 100. Eleven patients had an IQ below 70, and 15 between 70 and 84. Verbal IQ was higher than Performance IQ. Level of handicap within the study group was considered moderate, and all but one child had extra support at school. We conclude that children with 22q11 deletion syndrome have multiple neurological, motor, and cognitive problems. Although the severity and number of problems varies, the combination of impairments and disabilities results in a low level of participation.

This study investigated the adaptive gait pattern in obstacle clearance in 12 normally developing (ND) children (six males, six females; mean age 10y 2mo, SD 10mo) and 12 children who had spastic diplegic cerebral palsy (CP) and who were independent ambulators (10 males, two females; mean age 13y 5mo, SD 2y 7mo). Children in both groups had 8 to 12 years of walking experience. They walked up to and crossed over obstacles of a height of 0%, 10%, and 20% of their leg length. Kinematic aspects of the three-dimensional leg movements were captured with the Vicon system and analyzed with one-way analysis of variance with repeated measure. Both groups increased foot clearance for higher obstacles. Children with CP were slower in approach and crossing speed, and used a wider base of support than the ND children when the obstacle height was increased. Results suggest that motor problems in obstacle clearance in children with CP lie in the implicit process of motor control, i.e. torque dynamics, not the explicit process, i.e. movement-match with the environmental constraints.

self-injury is a defining feature of lesch–nyhan disease (lnd) but does not occur in the less severely affected lesch–nyhan variants (lnv). the aim of this study was to quantify behavioral and emotional abnormalities in lnd and lnv. thirty-nine informants rated 22 patients with lnd (21 males, 1 female), 11 males with lnv, and 11 healthy controls (hc; 10 males, 1 female) using two well-validated rating scales. the age of patients with lnd ranged from 12 years 7 months to 38 years 3 months (mean 22y 11mo; sd 7y 8mo), whereas the age range of those with lnv was 12 years 9 months to 65 years (mean 30y 7mo; sd 15y 2mo), and the healthy controls were aged 12 years 4 months to 31 years 3 months (mean 17y 10mo; sd 5y 7mo). behavioral ratings were based on the child behavior checklist and the american association on mental retardation's adaptive behavior scale – residential and community, 2nd edition. statistical analyses revealed that patients with lnd showed severe self-injury together with problematic aggression, anxious-depressed symptoms, distractibility, motor stereotypes, and disturbing interpersonal behaviors. patients with lnv were rated as being intermediate between the hc and lnd groups on all behavior scales. although the lnv group did not differ from hcs on most scales, their reported attention problems were as severe as those found in lnd. we conclude that self-injurious and aggressive behaviors are nearly universal and that other behavioral abnormalities are common in lnd. although patients with lnv typically do not self-injure or display severe aggression, attention problems are common and a few patients demonstrate other behavioral anomalies.

The aim of this study was to examine the stability of the second edition of the Bayley Scales of Infant Development (Bayley II) by correlating Bayley II scores during the first year of life with Bayley II scores at approximately 18 months of age. Bayley II Mental and Motor Scales were administered to 119 infants (69 males, 50 females) during their first year of life; mean age for low-risk group 7 months (SD 3.01) and mean age for high-risk group 7.8 months (SD 2.46). A second set of Bayley II scores was collected on 104 of the infants at 17 to 22 months of age; mean age for low-risk group 18.5 months (SD 1.07) and mean age for high-risk group 18.9 months (SD 1.11). Mean time interval between the two assessments was 11.5 months for the low-risk group and 11.15 months for the high-risk group. Stability of the scores over time was assessed. Correlations for the Bayley II Mental Developmental Index and Psychomotor Developmental Index between administrations during the first and second years of life were r=0.49 (p<0.001) and r=0.48 (p<0.001) respectively. We conclude that approximately 23 to 24% of the variance in the infants' later Bayley II scores could be explained by the earlier scores. This is one of only two studies to examine the stability of Bayley II over time. Replication studies are needed to evaluate the consistency of these findings across other samples of infants.

Children with epilepsy often experience poor social and educational outcomes. This study aimed to determine the timing of services with respect to the onset of seizures. It also aimed to identify the aspects of childhood epilepsy (type of epilepsy, etiology, seizure control, and treatment) that are associated with the use of special education services. As part of a prospective community-based study, 613 children were recruited when first diagnosed with epilepsy. Mean age at first seizure was 5 years 11 months (SD 4, range 1mo to 15y 8mo). Parents were interviewed 5 years after children were first diagnosed with epilepsy (n=542; 276 [51%] males). Children's mean age at time of interview was 11 years 10 months (SD 4y 1mo, range 5y 8mo to 21y 8mo). Etiology was classified as idiopathic (n=181, 33.4%), cryptogenic (n=261, 48.2%), and remote symptomatic (n=100, 18.5%). Service use was reported in 315 (58%) children. Compared with neurologically intact children (i.e. cryptogenic and idiopathic etiology; n=415, 77%), children with a remote symptomatic etiology and/or an epileptic encephalopathy (n=127, 23%) received services more frequently (88% vs 49%, p<0.001). In the former group, services were initiated for 66 (15%) children before their first seizure; according to age at onset, services were initiated before the first seizures in 12/164 (7.3%) if <5 years, 34/171 (19.9%) if 5–9 years, and 20/80 (25%) if >10 years. A large proportion of children with epilepsy, even if neurologically otherwise normal, receive special education services. Initiation of services often precedes onset of seizures even in neurologically intact children. This suggests that behavioral and cognitive abnormalities may predate the onset of epilepsy and are not necessarily the direct consequences of epilepsy.

A cohort of 48 children with Noonan syndrome, with a mean age of 9 years 10 months (SD 3y 7mo; 28 males, 20 females), was recruited from a national study. Children were assessed using the Wechsler Intelligence Scales and Test of Motor Impairment–Revised (TOMI-R). The Piers–Harris Self-evaluation Questionnaire was also completed in a subgroup of age-appropriate children. Children's parents and teachers completed the Rutter A and B scales to assess of the degree of behavioural and emotional problems respectively. Mean Full-scale IQ score was 84, and one-quarter of the participants had learning disabilities. Verbal IQ tended to be slightly lower than Performance IQ. About half of the group showed evidence of mild to moderate impairment, confirming the impression of ‘clumsiness/developmental coordination disorder’ on the TOMI-R. Level of self-esteem, as determined by the Piers–Harris Questionnaire, was comparable to that of a standardized population. This research has identified some characteristic psychological features in Noonan syndrome. However, a specific behavioural phenotype could not be identified.

The purpose of this article is to demonstrate: (1) the accuracy and (2) the reduction in amount of time and effort in assessing physical functioning (self-care and mobility domains) of children and adolescents using computer-adaptive testing (CAT). A CAT algorithm selects questions directly tailored to the child's ability level, based on previous responses. Using a CAT algorithm, a simulation study was used to determine the number of items necessary to approximate the score of a full-length assessment. We built simulated CAT (5-, 10-, 15-, and 20-item versions) for self-care and mobility domains and tested their accuracy in a normative sample (n=373; 190 males, 183 females; mean age 6y 11mo [SD 4y 2m], range 4mo to 14y 11mo) and a sample of children and adolescents with Pompe disease (n=26; 21 males, 5 females; mean age 6y 1mo [SD 3y 10mo], range 5mo to 14y 10mo). Results indicated that comparable score estimates (based on computer simulations) to the full-length tests can be achieved in a 20-item CAT version for all age ranges and for normative and clinical samples. No more than 13 to 16% of the items in the full-length tests were needed for any one administration. These results support further consideration of using CAT programs for accurate and efficient clinical assessments of physical functioning.

The aim of this study was to propose a classification system for childhood arterial ischaemic stroke (AIS). Subtypes from the Trial of Org 10172 in Acute Stroke Therapy (TOAST) classification, previously shown to be applicable to children, were retained in the proposed Paediatric Stroke Classification (PSC). Additional important paediatric AIS aetiologies were identified from a literature review. Preliminary validation was performed by three raters who categorized clinical vignettes from 135 patients (66 male; median age 6.3y, range 0.1 to 16y). Eight aetiological subtypes were identified and defined, as follows: (1) sickle cell disease; (2) cardioembolic; (3) moyamoya syndrome; (4) cervical arterial dissection; (5) steno-occlusive cerebral arteriopathy; (6) other determined aetiology; (7) multiple probable/possible aetiologies; and (8) undetermined aetiology. There was very good agreement between the raters about categorization of the vignettes. Causes of disagreement were identified and final categories and definitions were modified accordingly. We conclude that the PSC enables the categorization of children with AIS into aetiological subtypes relevant to this age group. This will be useful in multicentre studies of natural history and treatment but will require further independent validation.