The aims of this study were to investigate fine motor skills of children with both attention-deficit–hyperactivity disorder (ADHD) and developmental coordination disorder (DCD) and those of a control group, and to examine the effects of methylphenidate on these skills. A group of 12 children with ADHD–DCD (11 males, one female; mean age 9y 8mo [SD 1y 7mo]) and 12 age- and sex-matched controls (mean age 9y 7mo [SD 1y 2 mo]) participated. The manual dexterity subtests of the Movement Assessment Battery for Children, the concise assessment method for children's handwriting, and a computerized graphomotor task were used. Results demonstrated that children with ADHD–DCD performed more poorly on the manual dexterity subtests, had poorer quality of handwriting, and drew more rapidly, more fluently, but less accurately than controls on the graphomotor task. On methylphenidate, manual dexterity and quality of handwriting improved, and strokes on the graphomotor task became less fluent but more accurate. ADHD is characterized by persistent symptoms of inattention, impulsivity, and hyperactivity, affecting 3 to 5% of school-age children. Up to 50% of children with ADHD also have motor coordination problems that are severe enough to meet criteria for DCD. In DCD, children demonstrate functional motor performance deficits not explained by the child's (chronological) age or intellect, or by other neurological or psychiatric disorders.

The aim of this preliminary study was to examine the developing cognitive profiles of children with cerebellar tumours in a consecutive series of clinical patients. MRI and longitudinal intellectual profiles were obtained on seven children (two females, five males; mean age 3 years at diagnosis; mean age 7 years at first assessment). Tumours in three of the children were astrocytomas; of the remaining tumours, two were medulloblastomas, one low-grade glioma, and one ependymoma. In right-handed children, we observed an association between greater damage to right cerebellar structures and a plateauing in verbal and/or literacy skills. In contrast, greater damage to left cerebellar structures was associated with delayed or impaired non-verbal/spatial skills. Long-term cognitive development of the children studied tentatively supports a role for the cerebellum in learning/development. These findings suggest that lateralized cerebellar damage may selectively impair the development of cognitive functions subserved by the contralateral cerebral hemisphere and, in addition, that all children with cerebellar lesions in early childhood should routinely undergo long-term monitoring of their intellectual development.

Cortical visual impairment (CVI) is the leading cause of bilateral visual impairment in children in Western countries. This finding reflects better methods for identifying visual impairment due to CNS injury and also advances in perinatal care, which have increased the survival rate of children with neurological morbidity. This review will describe advances in the diagnosis and management of CVI. Central to our discussion is a definition of CVI that includes a decrease in visual acuity. New treatment and rehabilitative measures are badly needed for this disorder. We hope to stimulate interest in CVI, a disease that has become a significant public health problem.

To obtain a preliminary validation of the Non-Communicating Children's Pain Checklist for individuals with inability to communicate verbally, 32 caregivers of individuals with cognitive impairments aged 3 to 44 years retrospectively completed the Non-Communicating Children's Pain Checklist and rated item usefulness (0 to 10). In the second part of the study 33 caregivers completed the checklist after two painful (e.g. burns, falls, surgery), one distressful (e.g. unwanted grooming, feared animal/noise), and one calm event (e.g. watching television). Checklist scores did not correlate with the age, sex, or physical limitations of individuals with inability to communicate verbally. In the first part of the study Cronbach's alpha was 0.66; all mean usefulness ratings exceeded 5 out of 10. In the second part of the study after four items were removed, Cronbach's alpha was 0.79. Checklist scores during pain correlated with numerical ratings of pain intensity, did not differ between the two pain events, and differed significantly from calm scores. Differences in Checklist scores during pain and distress were found for two subscales. The checklist exhibits internal consistency, and preliminary evidence suggests it can detect pain and is reliable over time.

Our previous studies revealed abnormalities on structural MRI (sMRI) in small groups of children exposed to alcohol prenatally. Microcephaly, disproportionately reduced basal ganglia volume, and abnormalities of the cerebellar vermis and corpus callosum were demonstrated. The present study used sMRI to examine in detail the regional pattern of brain hypoplasia resulting from prenatal exposure to alcohol using a higher resolution imaging protocol and larger sample sizes than reported previously. Fourteen participants (mean 11.4 years; eight females, six males) with fetal alcohol syndrome (FAS) and 12 participants (mean 14.8 years; four females, eight males) with prenatal exposure to alcohol (PEA) but without the facial features of FAS were compared to a group of 41 control participants (mean 12.8 years, 20 females, 21 males). Findings of significant microcephaly and disproportionately reduced basal ganglia volumes in the FAS group were confirmed. Novel findings were that in FAS participants, white matter volumes were more affected than gray matter volumes in the cerebrum, and parietal lobes were more affected than temporal and occipital lobes. Among subcortical structures, in contrast to the disproportionate effects on caudate nucleus, the hippocampus was relatively preserved in FAS participants. Differences between the PEA group and controls were generally non-significant; however, among a few of the structures most affected in FAS participants, there was some evidence for volume reduction in PEA participants as well, specifically in basal ganglia and the parietal lobe. There were no group differences in cerebral volume asymmetries. Severe prenatal alcohol exposure appears to produce a specific pattern of brain hypoplasia.

The main purpose of the study was to estimate the prevalence of psychiatric diagnoses in children with mental retardation (MR) (IQ[les ]70). All children born between 1980 and 1985 (N=30037) in Akershus County, Norway, were screened for possible MR and assessed with either IQ tests or standardized developmental tests. A total of 178 children, 79 with severe mental retardation (SMR) (IQ<50) and 99 with mild mental retardation (MMR) (IQ 50 to 70) were included for further study. Psychiatric symptomatology was assessed as a standard part of the neurodevelopmental examination, which included a semistructured parent interview, a clinical child interview, and retrieval of the charts of previous child psychiatric examinations. Psychiatric diagnoses were classified according to the International Classification of Disease (ICD-10). In total, 65 (37%) of the total population with MR (95% confidence intervals 29 to 44) were registered to have psychiatric diagnoses, the most common being hyperkinesia (n=28) and pervasive developmental disorder (n=15). Psychiatric diagnoses were present in 42% of the population with SMR and 33% of the population with MMR (p=0.4). Of all children found to have a psychiatric diagnosis, approximately one-third had previously been examined by a child psychiatrist and indicated a previously unrecognized need for these services to children with MR.

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.

The aim of the study was to identify the comorbidity of autistic spectrum disorders in a population of children with Down syndrome (DS). All children with DS within a defined population of South Birmingham were identified. The Asperger Syndrome Screening Questionnaire and the Child Autism Rating Scale were completed and diagnosis made according to ICD-10 criteria following interview and observation. Thirty-three of 58 identified children completed the measures, four of whom received a diagnosis of an autistic spectrum disorder. This is equivalent to a minimum comorbid rate of 7%. The questionnaire items concerning social withdrawal, restricted or repetitive interests, clumsiness, and unusual eye contact were associated with an autistic disorder. Of the remaining 29 participating children, 11 also displayed marked obsessional and ritualistic behaviours. The comorbid occurrence of autism and DS is at least 7%. It is important that these children are identified and receive appropriate education and support. A full assessment of social, language, and communication skills and behaviour is crucial, particularly in children with DS who appear different from other children with DS. Potential mechanisms accounting for this comorbidity are discussed.

Parental report may provide an inexpensive alternative to standardized assessments of children's development. We have adapted and validated a parental questionnaire on cognitive development for use with very preterm infants. Sixty-four 2-year-olds (28 males, 36 females) born less than 30 weeks' gestation (median 28.5 weeks, range 23 to 31 weeks), median birthweight 980g (range 500 to 1905g) were assessed using the Mental Development Index (MDI) of the Bayley Scales of Infant Development-II and the parental questionnaire. Significant correlations between parent report and MDI scores (r=0.54–0.68, p<0.001) indicated good concurrent validity. Diagnostic use of the parent report for predicting an MDI score of less than 70 was assessed by using receiver operating characteristic (ROC) curves. The optimal cut off produced equal sensitivity and specificity (81%), indicating good discriminatory power in diagnosing developmental delay. Test–retest reliability was demonstrated and accuracy of parent reporting was not affected by sociodemographic factors. Our questionnaire provides a valid outcome measure for use in randomized trials or large population surveys.

Cognitive performance in 7- to 9-year-old preterm neonatal intensive-care survivors was compared with that in age-matched control children. Non-verbal memory span, spatial working-memory abilities, planning, set-shifting, and recognition memory for both spatial and patterned stimuli were assessed using the Cambridge Neuropsychological Testing Automated Battery. Relative to children in the control group, neonatal intensive-care unit (NICU) survivors demonstrated 25% more memory errors on the spatial working-memory task. Their use of strategy on this task was similar to a control group of 5-year-olds. Planning times on ‘Tower of London’ problems were long relative to those of term controls. NICU survivors demonstrated poorer pattern recognition as well as a shorter spatial memory span. The groups did not differ in visual-discrimination learning or in spatial-recognition memory. No specific neonatal risk factor accounted for the observed differences, although scores on the Neurobiological Risk Score (NBRS), a composite measure of neonatal risk, did predict several aspects of later task performance. Whether these data reflect a developmental delay in brain maturation in NICU survivors or the presence of a permanent information-processing deficit due to adverse neonatal events must be assessed through continued follow-up.

This purpose of this study was to investigate visual-object and visual-spatial long-term memory (LTM) abilities in individuals with Williams syndrome (WS) and Down syndrome (DS). Four groups comprised of 15 participants were included: WS group (10 males) with a mean chronological age (CA) of 18 years 5 months, SD 6 years 4 months, and mean mental age (MA) of 6 years 8 months, SD 1 year 5 months; WS control group (eight males) comprised of typically developing children (CA mean 6y 7mo, SD 8mo); DS group, (10 males, CA mean 16y 5mo, SD 5y 10mo; MA mean 5y 4mo, SD 8mo); and DS control group (seven males) formed by typically developing children (CA mean 5y 6mo, SD 7mo). In the WS and DS groups mental age and IQ were evaluated with the Form L-M of the Stanford-Binet Intelligence Scale. Results showed that individuals with WS showed decreased learning of visual-spatial material but substantially typical learning of visual-object patterns as compared to a group of mental-age-matched typically developing children. Individuals with DS showed the opposite profile, i.e. typical learning of visual-spatial sequences but impaired learning of visual-object patterns. These results, showing an interesting double dissociation between these two genetic syndromes in the learning of visual-object patterns as opposed to visual-spatial data, support the interpretation of learning disability as a heterogeneous condition, characterized by potentially very different qualitative profiles of cognitive impairment.

The study combined prospective neuropsychological and EEG results of 22 children presenting with typical benign partial epilepsy with rolandic spikes (19 participants) and occipital spikes (three participants). The aims were to assess the types of cognitive problems which may be encountered in this population, to evaluate the course of cognitive and learning capacities during the active phase of epilepsy, and to see if there was a correlation with paroxysmal activity on the EEG. Average age at entry in the study was 8.4 years and each child was seen 2 to 4 times over a period of 1 to 3 years. EEGs showed persistent spike foci in most cases that worsened in three cases, but there were no continuous spike–waves during sleep. No child had persistent stagnation, marked fluctuations, or a regression in cognitive abilities. Of 22 children, 21 had average IQ (>80). Eight children had school difficulties requiring special adjustment. No single cognitive profile was identified. Four children had delayed language development and eight children had transient weak scores in one isolated domain (verbal, visuospatial, memory) which improved or normalized during the course of the study with concomitant EEG improvement or normalization. In two of the three children with aggravation of the paroxysmal EEG activity, clinical changes were documented. A proportion of children with typical benign partial epilepsy with rolandic spikes showed mild, varied, and transient cognitive difficulties during the course of their epilepsy and in most cases this probably had a direct relation with the paroxysmal EEG activity.

The aim of this study was to investigate the relationship between motor performance, attention deficit, impulsiveness, and hyperactivity in children with attention-deficit–hyperactivity disorder (ADHD). Participants were 42 school-aged children with ADHD (36 males, 6 females; mean age 8 years 2 months, SD 1 year 2 months; range 6 years to 11 years), and 42 age- and sex-matched children without ADHD (mean age 8 years 3 months, SD 1 year 1 month; range 6 years to 11 years). Motor abilities were assessed with the Bruininks-Oseretsky Test of Motor Proficiency. Attention and impulse control were measured with the Gordon Diagnostic System, and assessment of activity level was based on two Activity Level Rating Scales that were completed by parents and teachers. Analysis by t-test revealed a significant difference between children with and without ADHD in fine and gross motor skills, impulse control, and attention. Stepwise regression indicated that attention, impulse control, and parent ratings of activity level were the three best predictors of gross motor skills for children with ADHD, accounting for 55.9% of the variance. Attention and impulse control were the two best predictors of fine motor skills, accounting for 45.7% of the variance. Attention and impulse control were consistently found to be important predictors of both fine and gross motor skills in children with ADHD. However, the fact that activity level was a predictor for gross motor proficiency but not for fine motor tasks suggests that different behavioral processes are involved in fine and gross motor performance to different extents.

The aim of this study was to examine the prevalence of secondary impairments in young adults with spina bifida and to relate the prevalence to the type of spina bifida and the level of lesion. This cross-sectional study is part of the ASPINE (Adolescents with Spina Bifida in the Netherlands) study. Data were collected on medical history, hydrocephalus (shunt: yes/no), neurological level of lesion (International Standards for Neurological and Functional Classification of Spinal Cord Injury), visual acuity (Landolt rings), spasticity (Modified Ashworth Scale), contractures (range of motion), scoliosis (deviation from perpendicular), ambulation (Hoffer criteria), pressure sores and blood pressure (physical examination), epilepsy, pain, incontinence and sexuality (questionnaire), and cognitive functioning (Raven Standard Progressive Matrices). In total, 179 patients with spina bifida participated (41% male, age range 16 to 25 years, mean 20 years 9 months, SD 2 years 11 months). These were 37 patients with spina bifida occulta, 119 patients with spina bifida aperta and hydrocephalus (AHC+) and 23 patients with spina bifida aperta without hydrocephalus (AHC–). Of our patient group, 73 had a high-level lesion (L2 and above), 68 a mid-level lesion (L3 to L5), and 38 a low-level lesion (S1 and below). Both subdivisions were strongly related with patients with higher lesions more often having hydrocephalus. Most secondary impairments were found for patients with AHC+, and patients with AHC– were mostly comparable to patients with spina bifida occulta. According to level of lesion, most medical problems were found in the high-level lesion group. However, all subgroups suffered from health problems.

The ketogenic diet is increasingly used for the management of difficult-to-control seizures in children. Here, we describe the first prospective study of the effects of the diet on development, behavior, and parenting stress. Participants were 65 children (36 males, 29 females) with intractable seizures, ages 18 months to 14 years 6 months, enrolled in a prospective study at the Johns Hopkins Hospital, Baltimore, MD, USA, to study the diet's efficacy. Children were assessed before diet initiation and at 1-year follow-up. At follow-up, 52% (34 of 65) children remained on the diet. Mean seizure frequency decreased from 25 per day before diet initiation to less than two per day 1 year later. At follow-up, mean developmental quotient showed statistically significant improvement (p<0.05), with significant behavioral improvements in attention and social functioning. Parental stress was essentially unchanged. No baseline factor examined predicted diet adherence, and the primary reason for diet discontinuation was insufficient seizure control. These preliminary results support prior anecdotal reports of the beneficial effects of the diet on cognition and behavior.

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.

To assess the prevalence of macrocephaly (head circumference [ges ]1.88 standard deviations above normative data for age and sex or >97th centile) in autism and other pervasive developmental disorders, 41 children with autism, and a comparison group of 21 children with tuberous sclerosis complex (TSC) or an unspecified seizure disorder were studied. Familiality of head circumference was also assessed from measurements of 133 first-degree relatives. Significantly higher rates of macrocephaly were found in probands with autism (12.2%) and their first-degree relatives (15.5%) when compared against a published normative sample. The incidence of macrocephaly in the comparison group of probands with TSC and seizure disorder (9.5%) and their first-degree relatives (8.3%) was higher than normative data as well, although the relation between macrocephaly and autism was more pronounced. Head circumference and extreme scores reflecting macrocephaly were moderately heritable in the present sample (H2=0.47). The increased prevalence of macrocephaly in relatives of children with autism compared with control children suggests that this characteristic may be a familial risk factor in the pathogenesis of autism.

It has been suggested that autism may arise as the result of exposure to high concentrations of prenatal testosterone. There is evidence that the ratio of the lengths of the 2nd and 4th digit (2D:4D) may be negatively correlated with prenatal testosterone. We measured 2D:4D in 95 families recruited via the National Autistic Society, UK. The sample comprised a total 72 children with autism (62 males, 10 females; age range 2 to 14 years), including 23 children (20 males, three females) with Asperger syndrome (AS), 34 siblings, 88 fathers, 88 mothers and sex- and age-matched control participants. We found that the 2D:4D ratios of children with autism, their siblings, fathers and mothers were lower than population normative values. Children with AS, who share the social and communicative symptoms of autism but have normal or even high IQ, had higher 2D:4D ratios than children with autism but lower ratios than population normative values. There were positive associations between 2D:4D ratios of children with autism and the ratios of their relatives. Children with autism had lower than expected 2D:4D ratios and children with AS higher ratios than expected in relation to their fathers' 2D:4D ratio. It was concluded that 2D:4D ratio may be a possible marker for autism which could implicate prenatal testosterone in its aetiology.

Little is known about long-term physical sequelae, cognitive functioning, and quality of life in children who have experienced ischemic stroke. Thirty-seven patients under 16 years of age were studied; the median interval after stroke was 7 years. CT-scans were reassessed to determine the type of infarction at baseline. Occurrences of death, of new cardiovascular events, and of seizures during follow-up were recorded. Surviving patients were invited for a follow-up examination, including physical check-up, global screening of cognition, and an inventory of subjective health perception. Only two patients were lost to follow-up. During follow-up four died, nine developed seizures, eight had transient ischemic attacks, and two experienced a recurrent ischemic stroke. None of the patients had cardiac complications during follow-up. In 11 of 27, no functional impairment was found, in 15 there was a hemiparesis of varying severity, and in one a paraplegia. There was a significant shift in cognitive functioning towards lower levels, especially in children with epilepsy. Remedial teaching was frequently needed. Many of the parents perceived their child's behavior to be very changeable. Three-quarters of the children considered themselves as healthy as other children, and almost all of them as happy. The physical and functional prognosis after ischemic stroke in childhood is relatively good, particularly in children with no serious causative illness, but special education is often needed and social changes occur.

spasticity, a neurological problem secondary to an upper motor neuron lesion, has a significant effect on skeletal muscle. the upper motor neuron lesions may be secondary to a cerebral vascular accident, head injury, spinal cord injury, or degenerative diseases such as multiple sclerosis, or perinatal brain injuries such as cerebral palsy. functional ability in these patients can be severely compromised but the basic mechanisms underlying these deficits are not clearly understood. in this review we evaluate the current evidence in the literature that suggests that skeletal muscle tissue itself is altered in spastic conditions. experimental studies were evaluated that included a variety of methods encompassing joint mechanics, tissue mechanics, and muscle morphology. taken together, the literature strongly supports the assertion that ‘spastic muscles’ are altered in a way that is unique among muscle plasticity models and inconsistent with simple transformation due to chronic stimulation or disuse. further studies are required to detail the intra- and extracellular modifications of skeletal muscle that occur secondary to spasticity so that novel therapeutic treatments can be developed for this impairment.