The Melbourne Assessment of Unilateral Upper Limb Function (Melbourne Assessment) is an evaluation tool that objectively measures upper-extremity function in children with cerebral palsy (CP). This study investigates how well performance on the Melbourne Assessment relates to the child's actual performance in functional tasks. Eighteen children with CP (5 to 14 years of age; nine males, nine females) were assessed using the Melbourne Assessment and the Pediatric Evaluation of Disability Inventory (PEDI). Five children had spastic quadriplegia, eight had spastic diplegia, two had spastic hemiplegia and diplegia, two had athetosis, and one had hypotonic quadriplegia with mobile ventilator dependence. Children's performances were statistically correlated using Spearman's rho to establish the relation between these tools. Very high correlation coefficients were calculated between the Melbourne Assessment and self-care (0.939) and mobility domains (0.783) of the PEDI and the overall functional skills section of the PEDI (0.718). The Melbourne Assessment demonstrates excellent construct validity for upper limb functioning.

This study documents the development of hand and upper-extremity function in young children who have cerebral palsy (CP) with upper-extremity involvement using longitudinal data. Assessments of hand function and the quality of upper-extremity movement were conducted on 29 males and 22 females (mean age 36.2 months, SD 10.6; age range 16 to 60 months at baseline) and on four other occasions over 10 months. Linear mixed effects modeling was used to estimate average developmental curves and the degree of individual differences in the patterns of development which were conditional on the body-site distribution of CP and severity of impairments. Results indicate that hand function in this clinical population develops differently from overall upper-extremity skills with declines in function in upper-extremity skills being more common and pronounced among older children. However, there is substantial interindividual variation. Distribution of CP and severity of impairments were significant predictors of development. Results are discussed in terms of their clinical implications.

All mainstream primary schools in Cardiff were invited in July 1998 to participate in a prevalence survey of autistic spectrum disorder. Teachers of each class filled in a questionnaire based on ICD-10 criteria for autistic disorders. The Autism Spectrum Screening Questionnaire (ASSQ) was completed on children identified with problems identified by the questionnaire. A total of 11692 children born between 1 September 1986 and 31 August 1990 were screened: 234 (2%) children were identified as requiring an ASSQ; 151 of 234 (65%) ASSQs were returned. Of the 151, 60 children (52 male, 8 female; 40%) scored 22 or more. Their notes and the involved professionals were consulted. Thirty-five children, unknown to specialist services or with complex features, required additional assessment. Seventeen children (all male) were found to be on the autistic spectrum. When the overall rubric was disentangled we found a diverse population of affected children including a handful who did not fit easily into ICD-10 classification. Correcting for incomplete ascertainment we found a minimum prevalence of 20.2 out of every 10000 (SE=4.5) for autistic spectrum disorder in this population.

The aim of this study was to evaluate whether EEG (i.e. positive Rolandic sharp waves) can be used to predict neurodevelopment in newborn infants with periventricular leukomalacia and compare the predictive value with that of MRI. A sequential cohort of neonates (n=45; 33 males, 12 females; mean gestational age 31.2 weeks, SD 2.7, range 27 to 37.8 weeks; mean birthweight 1592g, SD 601g) with periventricular hyperechogenicities on cranial ultrasound was recruited for this study. EEGs were analyzed for positive Rolandic sharp waves. Neurodevelopment was evaluated at the ages of 12 and 18 months. In the whole group the probability of a poor outcome was 24% and the probability of any impairment was 33%. If the number of positive Rolandic sharp waves was no more than 0.1 per minute, the probability of a poor outcome was reduced to 9% (95% confidence interval [95%CI] 2 to 27%) and the probability of any impairment was reduced to 13% (95%CI 4 to 32%). In all infants with more than 0.1 positive Rolandic sharp waves per minute the probability of a poor outcome was 41% (95%CI 23 to 61%) and of any impairment was 55% (95%CI 34 to 73%). In these infants MRI identified infants with a poor outcome with a sensitivity of 1.00 (95%CI 0.70 to 1.00) and a specificity of 0.92 (95%CI 0.67 to 0.99), and infants with any impairment with a sensitivity of 0.83 (95%CI 0.55 to 0.95) and a specificity of 1.00 (95%CI 0.72 to 1.00). Results suggest that if an EEG of an infant with periventricular leukomalacia contains no more than 0.1 positive Rolandic sharp waves per minute the probability of a normal or mildly delayed development is high (0.91, 95%CI 0.73 to 0.98). MRI enhances the accuracy of the outcome prediction slightly; however, owing to a wide confidence interval, this advantage is negligible. However, if the frequency of the positive Rolandic sharp waves exceeds 0.1 per minute, MRI can significantly enhance the precision of the prediction of outcome.

Fatigue is commonly reported among adults with cerebral palsy (CP) and in the general population. The aim of this study was to examine the prevalence of perceived fatigue in adults with CP compared with the general Norwegian population. A multidimensional questionnaire, containing among other instruments the Fatigue Questionnaire, was sent to persons with CP aged 18 years or older with no intellectual disabilities documented in their medical records. Results were compared with recently published results of a study of fatigue in the general population in Norway. There were 406 respondents (209 males, 197 females; mean age 34 years, SD 11 years; age range 18 to 72 years). Distribution of the different types of CP coincided with international epidemiological studies on CP. Adults with CP reported significantly more physical, but not more mental fatigue, than the general population. The strongest predictors associated with fatigue were bodily pain, deterioration of functional skills, limitations in emotional and physical role function, and low life satisfaction. Fatigue was not significantly associated with type of CP but was most prevalent in persons with a self-reported moderate grade of CP. Results of the present study indicate that physical fatigue is a pronounced problem in adults with CP, which has an impact on the preservation of functional skills and life satisfaction. Fatigue and factors related to fatigue should be addressed specifically in follow-up programmes for persons with CP.

Three groups of children with communication disorders were examined using a series of psycholinguistic markers to explore whether the tasks could identify children with impairments other than specific language impairment (SLI), and to examine whether the different groups within this clinical population could be distinguished reliably from one another. The groups comprised children with autistic spectrum disorders (ASD; n=13, all males; mean age 10 years 10 months, range 10 years 2 months to 12 years 6 months); children with primary pragmatic language impairment (PLI) but who did not have definite ASD diagnoses (n=25, 22 males, three females; mean age 11 years 3 months, range 10 years 2 months to 12 years 5 months); and children with specific language impairment (SLI) without marked pragmatic language difficulties (n=29, 25 males, 4 females; mean age 10 years 10 months, range 10 years 2 months to 11 years 9 months). Clinical markers examined were: the Children's Non-Word Repetition (CNRep), the Past Tense Task (PTT), and the Clinical Evaluation of Language Fundamentals, Recalling Sentences. First, it was found that the a priori groupings were not sufficiently defined and that four groups were actually present. The PLI group was in fact two separate samples: those with PLI pure and those with some autistic-like behaviours (referred to here as PLI plus, following Bishop 1998). Second, group comparisons indicated that CNRep was significantly lower for children with SLI than all other groups (although this measure was not such a good discriminator using a specificity analysis). Third, the markers were able to discriminate between all types of communication impairment in normal control participants (n=100; 51 females, 49 males; mean age 11 years, range 10 years 5 months to 11 years 6 months) with sensitivity levels of at least 75% and specificity of 80%. Recalling Sentences was the most efficient marker for all groups. Finally, analysis showed that children with PLI plus could be accurately distinguished from all others, scoring most favourably overall on communication markers and on performance IQ scores.

Children with attention-deficit–hyperactivity disorder (ADHD) have difficulties with motor control, inhibition of motor responses, motor flexibility, and motor preparedness. We proposed that motor abnormalities in ADHD might result, at least in part, from an abnormal neuronal oscillatory mechanism necessary for motor temporal regulation. The aim of this study was to assess pacing in children with ADHD, by testing for rhythmic abnormalities of motor activity using a tapping test. Twenty-seven children (21 males, six females; aged 6 to 14 years 6 months; mean age 11 years 4 months, SD 2 years 2 months) diagnosed with ADHD according to DSM-IV clinical criteria, and 33 controls (25 males, eight females; aged 6 to 14 years 6 months; mean 11 years 1 month, SD 2 years 2 months), underwent a finger-tapping test requiring rhythmic responses to frequencies from 1 to 6Hz. All participants who were treated on a daily basis with methylphenidate (n=22) were medication-free on the day of the test. Most of the children with ADHD responded at a constant rate regardless of stimulus frequency, a phenomenon only seen in a small number of the controls. This specific error pattern, also seen in Parkinson's disease, has been attributed to an abnormal oscillatory mechanism mediated by dopaminergic fronto-striatal circuitry, which might also be pathophysiologically relevant for ADHD.

A strong association between stress in pregnancy and small head circumference in infants at birth was reported in 1994. This important finding has never been replicated. In a follow-up study of 4211 participants with singleton pregnancies, information on life events was collected twice during pregnancy and head circumference measured shortly after birth following standard procedures. No association was found between experienced or perceived stress as a result of life events during pregnancy and head circumference in the infants. In conclusion, stress in pregnancy may influence foetal brain development in many ways, but we found no support for an effect on the size of the brain as measured by head circumference at birth.

This study examined the effect of massed practice in balance recovery of stability in six children (four males, two females; mean age 9 years 2 months, SD 2 years, range 7 years 5 months to 12 years 11 months) with cerebral palsy (CP). Four children were diagnosed with spastic diplegia (Gross Motor Function Classification System [GMFCS] level II) and two with spastic hemiplegia (GMFCS level I). A single-subject, multiple-baseline experimental design involving three pairs of children matched for diagnosis was used. A moveable forceplate system was used to test and train reactive balance control. Area per second (i.e. area covered by the center of pressure over a one second period) and time to stabilization from center of pressure measures were calculated following perturbations. The intervention phase consisted of massed practice on the moving platform (100 perturbations/day for 5 days). Analysis included hierarchical linear modeling and a repeated measures ANOVA. All children demonstrated a significant improvement in their ability to recover stability as demonstrated by reduced center of pressure area and time to stabilization following training. These improvements were still present 30 days following completion of training. Results suggest that postural control mechanisms in school-age children (7 to 13 years) with CP are modifiable.

The purpose of this study was to determine whether electrical stimulation of the gluteus maximus would improve hip extensor strength, decrease excessive passive and dynamic internal hip rotation, and improve gross motor function in children with cerebral palsy (CP). Twenty-two ambulant children (15 females, 7 males, mean age 8 years 6 months, SD 2 years 9 months, aged 5 to 14 years) with diplegic (n=14), hemiplegic (n=7), and quadriplegic (n=1) CP participated in this study. All were randomly assigned to either the stimulation or control group. The stimulation group (n=11) received electrical stimulation of the gluteus maximus of the most affected legs for 1 hour a day, 6 days a week for a period of 8 weeks. Electrodes were applied proximally and distally over the gluteus maximus, with the active electrode initially positioned over the motor points. The control group (n=11) did not receive any extra treatment. Measurements of hip extensor strength, gait analysis, passive limits of hip rotation, and section E of the Gross Motor Function Measure were made before and after treatment for both groups. Subjectively, 7 of the 11 parents thought that the treatment made a difference to their child. However, no statistically or clinically significant improvement was found in the stimulation group when compared with the control group.

Using a population-based register, this study sought to ascertain changes in the rate and severity of cerebral palsy (CP) in a geographically defined area of the UK among infants weighing less than 1500g and born between 1984 and 1995. There were 417414 live births in the area, which included Berkshire, Buckinghamshire, Northamptonshire, and Oxfordshire. Of the 898 children with CP (526 males, 372 females), 194 (21.6%) weighed less than 1500g at birth. The overall CP rate for neonatal survivors fell from 2.5 out of every 1000 in 1984 to 1986 to 1.7 in 1993 to 1995. The rate for those weighing less than 1000g rose to 90 out of every 1000 neonatal survivors in 1987 to 1989 and then fell to 57 in 1993 to 1995. A similar pattern is seen among infants weighing 1000 to 1499g at birth, the rate rising to 77 in 1987 to 1988 and then falling to 40 in 1993 to 1995. The rate of severe motor disability among infants weighing less than 1500g also decreased (24.6 in 1984–1986 to 12.5 in 1993–1995). The relation of these findings to changes in perinatal care in the early 1990s is not known.

Considerable attention has been given in recent years to fingertip force coordination during grasping and lifting small objects in children with cerebral palsy (CP). However, little is known about the children's ability to replace and release an object from grasp. The present study examined the coordination of fingertip forces during replacement and release of an object from grasp under varying task constraints in the involved hand of 15 children (10 males, five females, age range 7 to 14 years) with hemiplegic CP and in the non-dominant hand of 15 age-matched, typically-developing children (seven males, eight females). Participants released an object, instrumented with force transducers and held with a precision grip, onto a stable surface and onto an unstable surface (requiring higher accuracy) at self-paced and fast-as-possible speeds. Temporal and force measures were recorded and the dependent measures were tested using analyses of variance. Results showed that force coordination was impaired in children with hemiplegia, resulting in prolonged and uncoordinated replacement and release of the object (p<0.05). Differences between controls and children with hemiplegia were greater when speed and accuracy constraints were imposed (i.e. task performance was affected by these constraints to a greater extent in the children with CP, p<0.05). Impairments in temporal coordination of object release were also observed in the non-involved hand under all conditions (p<0.05). These results provide additional information about impaired hand function in children with hemiplegic CP. Clinical implications of these findings are discussed.

In the this study, both fine and gross motor ability of males with attention-deficit–hyperactivity disorder (ADHD) were compared with a group of control children. Three groups of males with the following ADHD subtypes: predominantly inattentive (ADHD-PI; n=50), hyperactive/impulsive (ADHD-HI; n=16), or combined (ADHD-C; n=38) were compared with 39 control males. Mean ages for the control group were 10 years 4 months (SD 1 year 4 months, range 7 years 8 months to 12 years 11 months); for the ADHD-PI group, 10 years (SD 1 year 2 months, range 7 years 10 months to 13 years); for the ADHD-HI group, 9 years 11 months (SD 1 year 2 months), range 7 years 11 months to 12 years 6 months); and for the ADHD-C group 10 years 2 months (SD 1 year 4 months, range 8 to 13 years). The Australian Disruptive Behaviours Scale and Connors' Parent Rating Scale-Revised were used to assess ADHD symptomatology. Verbal IQ was estimated using two verbal subtests of the Wechsler Intelligence Scale for Children, and movement ability was assessed using the Movement Assessment Battery for Children (MABC) and the Purdue Pegboard test. Findings demonstrated that the children with ADHD had significantly poorer movement ability than control children. A high percentage of these children displayed movement difficulties consistent with developmental coordination disorder (DCD). In addition, the current study found that the type and degree of movement difficulty differed between subtypes. The Total Impairment score, as derived from the MABC, was less severe for the ADHD-HI group than the other two ADHD groups, but more severe than for the control group. Males with ADHD-PI and ADHD-C had significantly poorer fine motor ability (p<0.001) than control males, whereas the ADHD-HI group did not differ significantly from any of the other groups. As children with ADHD only and the control group did not differ significantly on fine motor ability but were significantly better than children categorized with both ADHD and DCD, it was argued that poorer fine motor ability found in children with ADHD could not be attributed to deficits in attention and concentration, but rather to factors relating to their motor ability.

This study examined whether children with hemiplegic cerebral palsy (CP) have anticipatory control of fingertip forces during lifts of familiar objects, and what type of practice (blocked or random) best enhances the retention of anticipatory control during lifts of novel objects. Eighteen children with hemiplegic CP (7 females, 11 males; 7 to 14 years of age, mean age 10 years, SD 1.8) and 18 age-matched typically developing children (8 males, 10 females; mean age 10.4 years, SD 1.7) participated in the study. In the first experiment the children lifted familiar objects of various weights and sizes five times each, while the vertical lifting (load) force was measured. Most participants demonstrated higher rates of load force increase for heavier (and larger) objects already during the first lift, indicating anticipatory control. Furthermore, the load force rates generally were similar across the five lifts for each object, suggesting that they had stable representations of the objects' properties. In the second experiment children lifted three novel objects varying in weight (but identical in volume) 27 times each, in either a blocked or a random order, followed by nine immediate and nine delayed (24 hours) retention trials. Blocked practice resulted in greater differentiation of the force rates between objects during acquisition than did random practice. Both practice schedules resulted in similar retention. These findings suggest that children with hemiplegic CP have a priori internal representations used for anticipatory force scaling with familiar objects. Furthermore, the results indicate that these children can form and retain internal representations of novel objects for anticipatory control, irrespective of the type of practice schedule employed. Thus, clinically based practice sessions that incorporate lifts with novel objects may enhance anticipatory force scaling and related prehensile function in children with hemiplegic CP.

The aim of this study was to characterize children with transient nystagmus. Eleven children(six males, five females) developed nystagmus in infancy and then experienced regression of the problem, usually within a few months. Mean age at onset was 2.7 months, and mean age at regression was 8.5 months. No etiology could be ascertained in any of the patients, although four children had other eye or vision abnormalities (regressed retinopathy of prematurity, n=1; asymmetric fundus colobomata, n=1; delayed visual maturation, n=2). Results of this study suggest that mechanisms which allow ocular motor stability undergo a period of postnatal maturation, during which nystagmus can occur, but also during which nystagmus may disappear. Not every case of transient nystagmus should be categorized as spasmus nutans. There is a subset of infants and young children who develop transient nystagmus with no other findings and in whom the nystagmus disappears.

The aim of this study was to determine the reliability and magnitude of error of three lower-limb clinical measures for children with cerebral palsy (CP): the Modified Ashworth Scale of Spasticity (MAS), passive range of movement (PROM) and the modified Tardieu scale (MTS). Six physiotherapists measured 11 females and seven males (mean age 6 years 4 months, SD 2 years 4 months; age range 2 years 4 months to 10 years) on two occasions using a repeated measures design, collecting all data over 6 days. The severity of CP spanned all five levels of the Gross Motor Function Classification System and all children demonstrated varying degrees of spasticity. Exclusion criteria included botulinum toxin injections, inhibitory plasters, and orthopaedic surgery within the 6 months before study entry. For PROM and the MTS interrater reliability was acceptable with an intraclass correlation coefficient of 0.7, but results for MAS were lower. Standard error of measurement for repeated measures of PROM and MTS was about five degrees, but 95% confidence interval ranges were considerably higher. Test–retest results varied widely, particularly for the MAS. These measurement tools should be used with caution when evaluating changes in young children with CP.

In order to test the practicability and safety of whole-body cooling in term neonates with moderate-to-severe hypoxic–ischaemic encephalopathy (HIE) and to report outcomes, a prospective pilot study was carried out in 25 term infants (median postmenstrual age 38 weeks, range 36 to 41 weeks; 20 males, five females). Whole-body cooling, to a target core temperature of 33 to 34°C, started within 6 hours of birth and was maintained for 72 hours. Of the 25 newborn infants (19 Sarnat II and six Sarnat III, 18 outborn), 18 survived, including 13 (72%) with normal cerebral signal by MRI. Temperature instability occurred during cooling in 15 infants, but neither severe haemodynamic instability nor renal failure was seen. Thrombocytopenia developed in 12 infants, including seven with biological disseminated intravascular coagulation. One patient had hypoxaemia with right-to-left shunting through the ductus arteriosus, and seven had limited meningeal or subdural bleeding. Whole-body cooling is feasible in term neonates, with no life-threatening adverse events. Improvements are needed to obtain stable hypothermia for 72 hours.

Lesch–Nyhan disease (LND) is a rare X-linked recessive genetic disorder associated with cognitive impairment, choreoathetosis, hyperuricemia, and the hallmark symptom of severe and involuntary self-mutilation. This study examines data gathered from a survey of 64 families in the USA and abroad regarding the self-injury of their family members who have LND. The individuals with LND ranged in age from 1 to 40 years (mean 16 years 7 months, SD 11 years 2 months) and, with the exception of one, were males. The most common initial mode of self-mutilation, and the most frequently cited past or current behavior, was biting of lips and/or fingers. Other behaviors, in order of frequency, included head banging, extension of arms when being wheeled through doorways, tipping of wheelchairs, eye-poking, fingers in wheelchair spokes, and rubbing behaviors. Hierarchical cluster analysis identified patterns of association among the types of self-mutilation. Modes of self-mutilation in which external surfaces (such as a wheelchair component) served as instruments of self-injury tended to co-occur, as did biting of lips and fingers.

A male presenting with benign partial epilepsy with rolandic spikes from the age of 7 years was evaluated at age 11 years for worsening of his epilepsy associated with a specific regression of graphomotor skills. A longitudinal study over nearly 2 years showed an improvement in handwriting to an almost normal level under modified antiepileptic therapy. A detailed analysis with a computer-monitored graphics table showed at first a rapid improvement of skills followed by protracted slower progress. We argue that the initial rapid recovery of skills was directly linked to the improvement of his epilepsy. The slower late acquisition of motor programmes that had never been fully established was due to long-standing interference by his epilepsy. The specificity of the deficit within the graphomotor system and its possible neurobiological basis are also discussed. The analytical method and approach used in a single patient might provide an example for other patients in whom epilepsy can interfere in the acquisition, progress, and maintenance of new skills and can be responsible for selective deficits.

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.