Neuropsychological outcome at 14 to 15 years of age of a cohort of 75 participants(39 male, 36 female) born at <33 weeks' gestation was investigated. Research was conducted parallel to a recent MRI study by Stewart and colleagues which reported that 55% of this cohort had evidence of brain abnormality. One aim of the study was to compare neuropsychological function in those very preterm children with and without MRI abnormality. Compared to a control sample of term adolescents, very preterm participants had impairment only on a measure of word production. On measures of attention, memory, perceptual skill, and visuomotor and executive function, the adolescents born very preterm performed in the normal range, whether or not they had evidence of MRI abnormality. Our findings are encouraging as the neuropsychological consequences of damage to the very preterm brain, still evident on MRI at 14 to 15 years of age, appear to be minor.

The cutaneous flexion reflex has been used to study spinal sensory processing in 68 infants (37 female, 31 male) aged between 28 and 42 weeks postconceptional age (PCA). Mechanical and electrical stimuli were singularly and repeatedly applied to the foot, and single-surface EMG responses were recorded from the biceps femoris muscle. A clear correlation was demonstrated between the mechanical stimulus intensity and latency and the amplitude of the reflex. Mechanical threshold normally increased with age, but the flexion-reflex threshold was lowered by local limb-tissue damage in the contralateral limb. The incidence of response to repeated mechanical stimulation at 2.8 × threshold decreased significantly with increasing age. Repeated mechanical stimulation at 2.8 × threshold caused a build-up in the size of the response followed by a diminution. The flexion reflex can, therefore, be used to investigate sensory processing in the neonate, and the effects of tissue damage. The importance of using natural rather than electrical stimulation is highlighted.

The development of behaviour problems in infants born with biological risk (low birthweight) and psychosocial risk (psychosocially disadvantaged family) was studied in a sample of 347 children (171 males, 176 females) at the ages of 2, 4:6, and 8 years. In the search for factors that moderate the effects of early risks, the role of early responsive caregiving was examined. Results indicate that infants at psychosocial risk exhibited both more externalizing and internalizing problems across ages than infants not at psychosocial risk, while no overall differences were apparent between normal- and low-birthweight groups. With one exception, no interactions between biological and psychosocial risk factors emerged, suggesting that their simultaneous effect is largely additive. Maternal responsivity was found to moderate the effects of low birthweight on hyperkinetic and internalizing problems as well as to influence the consequences of family disadvantage on total problems. These findings stress the importance of early parenting in the behavioural development of at-risk children.

Ankle spasticity and strength in 27 children with spastic diplegic cerebral palsy (CP) (mean age 9 years, range 3 to 18 years) and a group of 12 children without CP (comparison group) (mean age 9 years, range 5 to 18 years) were observed. To measure spasticity, a KinCom dynamometer dorsiflexed the passive ankle at five different speeds and recorded the resistive plantarflexion torques. Work values for the torque-angle data were calculated at each speed. Using this data, linear regression was used to measure spasticity. To measure strength, the dynamometer rotated the ankle from maximum dorsiflexion to maximum plantarflexion at a speed of 10°/s while the child performed a maximum plantarflexion concentric contraction. The movement was reversed to record maximum dorsiflexion. Maximum torques and work by the plantarflexors and dorsiflexors were calculated. The group with CP had significantly more spasticity in the plantarflexors and significantly less strength in the plantarflexors and dorsiflexors than the group without CP. Results provide objective information quantifying ankle spasticity and strength in children with CP.

Cranial ultrasound abnormalities, especially a white-matter echolucency, predict disability (Paneth and Pinto-Martin 1990, Whitaker et al. 1990, Dammann and Leviton 1997), but with limitations. For example, half of very preterm infants who develop cerebral palsy (CP) do not have any abnormality of the white matter (Pinto-Martin et al. 1995, O'Shea et al. 1998). In addition, echolucencies that predict spastic CP are seldom located with descending fibers from the motor cortex (Rogers et al. 1994). These findings have led to the ‘tip of the iceberg’ hypothesis which postulates that cranial ultrasound scans do not detect the entirety of the white-matter damage (Leviton and Gilles 1996).

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 aim of the study was to test the hypotheses that elevated cerebral lactate, detected by proton spectroscopy performed within 18 hours of suspected birth asphyxia, is associated with adverse outcome, and that increased lactate can be used to predict adverse outcome. Thirty-one term infants suspected of having had birth asphyxia and seven control infants underwent proton magnetic resonance spectroscopy, using three-dimensional chemical shift imaging, within 18 hours of birth. Adverse outcome was defined as death or neurodevelopmental impairment at 1 year of age or more. Nine infants had an adverse outcome. The other 22 and all of the control infants remained normal. Median (range) lactate/creatine plus phosphocreatine (lactate/creatine) ratios in the abnormal, the normal, and the control group were 1.14 (0.17 to 3.81), 0.33 (0 to 1.51), and 0.05 (0 to 0.6) respectively (P=0.003). Lactate/creatine >1.0 predicted neurodevelopmental impairment at 1 year of age with sensitivity of 66% and specificity of 95%, positive and negative predictive values of 86% and 88%, and a likelihood ratio of 13.2. Elevated cerebral lactate/creatine within 18 hours of birth asphyxia predicts adverse outcome.

Two-hundred and fifty-one children (98 girls and 153 boys, aged from 3 to 17 years) with documented diagnosis of epileptic syndrome, IQ measurement, and information on school placement were included in this retrospective study. The relations between these three parameters as well as effects of age at onset and duration of epilepsy, seizure frequency, and number of antiepileptic drugs (AEDs) were analysed. Both IQ and schooling were univariately related to epileptic syndrome, age at onset and duration of epilepsy, and number of AEDs; seizure frequency was related to IQ but not to school placement. Multiple regression showed that IQ was independently related to epileptic syndrome and AED; multiple logistic regression showed that type of school (mainstream versus adapted or special) was independently related to IQ and AED. Children with idiopathic generalised or with localisation-related epilepsy had higher IQ scores and higher probability of mainstream schooling than those with symptomatic or cryptogenic generalised epilepsies or epileptic syndromes which were undetermined. Subtests profile of intelligence scale in localisation-related epilepsies showed different specific cognitive deficits, according to the location of the epileptic focus.

The present study examines the coordination of fingertip forces during object manipulation in both the involved and non-involved hand of 14 children with hemiplegic cerebral palsy (CP) aged between 8 and 14 years. While no impairment could be observed in the non-involved hand, subtle deficits in the sequencing of the grip–lift movement were observed in all children tested in this hand. However, they were able to scale the fingertip force output of the non-involved hand in advance (use anticipatory control) based on the object's weight. In the second experiment in this paper, we tested whether the anticipatory control can be generalized across hands. The results indicate that sensory information from the non-involved hand can be used for anticipatory scaling of isometric force increase during subsequent lifts with the contralateral involved hand. These findings suggest that the initial lack of anticipatory control usually observed in the involved hand of children with hemiplegic CP is likely to be based on disturbed sensory input.

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.

Adductor spasticity in children with cerebral palsy (CP) impairs motor function and development. In a placebo-controlled, double-blind, randomized multicentre study, we evaluated the effects of botulinum toxin A(BTX-A) in 61 children (37 males, 24 females; mean age 6 years 1 month [SD 3y 1mo]) with CP (leg-dominated tetraparesis, n=39; tetraparesis, n=22; GMFCS level I, n=3; II, n=6; III, n=17; IV, n=29; V, n=6). Four weeks after treatment, a significant superiority of BTX-A was observed in the primary outcome measure (knee–knee distance ‘fast catch’, p=0.002), the Ashworth scale (p=0.001), and the Goal Attainment Scale (p=0.037).

A longitudinal comparison was made between development of verbal and visuo-spatial short-term memory and vocabulary in children with Down syndrome (DS), children with specific language impairment (SLI), and typically developing children as a control group. Participants were 12 children with DS (6 males, 6 females; mean chronological age 9y 9mo [SD 2.8mo], range 8y 6mo to 11y 4mo); nine children with SLI (4 males, 5 females; mean chronological age 3y 9mo [SD 4.8mo], range 3y 3mo to 4y 5mo); and 12 typically developing children (5 males, 7 females; mean chronological age 4y 4mo [SD 3.9mo], range 3y 3mo to 4y 3mo). Participants were matched on mental age (mean mental age 4y 3mo). All participants completed verbal short-term memory, visuo-spatial short-term memory, and expressive and receptive vocabulary tasks on three occasions over 1 year. Similarities were seen in the clinical groups for verbal short-term memory. There was some evidence of difficulty in visuo-spatial short-term memory in the children with SLI relative to the other groups, but all three groups showed overlap in visuo-spatial short-term memory performance. At the final time-point vocabulary performance in the clinical groups was similar; the typically developing children showed higher vocabulary abilities than both clinical groups.

The relation between spasticity and strength in individuals with cerebral palsy (CP) has not been extensively researched. Knee and ankle spasticity and strength were quantified in a retrospective analysis of 60 individuals with spastic diplegic CP (mean age 12 years, range 3 to 38) and a group of 50 individuals without disabilities (WD group; mean age 12 years, range 4 to 36). Spasticity was measured using a KinCom dynamometer that stretched the passive knee flexors or ankle plantarflexors at different speeds and recorded the amount of resistive torques. For the strength tests, the participant performed a maximum contraction of the knee flexors/extensors and ankle plantarflexors/dorsiflexors throughout their range of motion at a speed of 10°/s on the dynamometer. Pearson's correlation coefficient was used to determine if a relation existed between spasticity and strength within the same muscle group and in opposing muscle groups at the knee and ankle joints. A t-test was performed to determine if greater spasticity and less strength existed at the ankles compared with the knees in those with CP. Results show that there was no relation between spasticity and strength either within the same muscle group or at opposing muscle groups at the knee and ankle joints in persons with CP. Individuals with spastic diplegic CP were more involved (greater spasticity, less strength) distally at the ankles compared with the knees. The findings conflict with the literature, which contains several assumptions, one of which is that a spastic muscle is a strong muscle and that spasticity causes weakness in the opposing muscle group. We found no relation between spasticity and strength in individuals with CP. Our findings support the literature, which states that individuals with spastic diplegic CP are more involved distally compared with proximally in the lower extremities.

The objective of this study was to determine whether the use of intramuscular botulinum toxin A (BTX-A) increases upper limb function and skills in the context of a specific therapy programme in children with hemiparetic cerebral palsy. Twenty children (nine females, 11 males) aged 4 to 16 years who were thought likely to benefit from BTX-A treatment were included. After matched pairs were made, on the basis of Zancolli grade and age, randomization took place. All patients were given structured rehabilitation (physiotherapy and occupational therapy three times a week for 6 months), and half of the patients received intramuscular BTX-A. No placebo injections were given in the control group. Participants were assessed at baseline, at 2 and 6 weeks, and at 3, 6, and 9 months after injection. The Ashworth scale, active range of motion of arm joints, the Melbourne assessment of upper limb function, the Pediatric Evaluation of Disability Inventory, and the nine-hole peg test were used for outcome measurement. Observers were blinded for treatment allocation only for scoring the Melbourne test. The children in the treatment group showed a clinically relevant increase in active dorsal flexion, and tone reduction of the wrist. For the functional outcome measures, no statistically significant differences between the groups could be demonstrated. Intramuscular BTX-A added to an intensive therapy programme reduces impairment for at least 9 months; the effect on activity level is still uncertain.

As yet, there is no aetiology-based intervention for autistic spectrum disorders (ASD); despite this, parents and professionals still need to make informed decisions regarding treatment options for children with ASD. This paper seeks to evaluate widely used interventions according to specific research criteria. Interventions presented are grouped into psychoeducational/behavioural approaches, psychopharmacological interventions, and the less traditional or complementary approaches. The conclusions are less than favourable: while some interventions do have empirical support, others have been proven to have no positive effects, and furthermore, there are no robust data favouring one approach over the others. Nevertheless, several criteria for choosing between treatment options are briefly discussed.

Objective measures (kinematics and kinetics) were used to study prospectively the effects of botulinum toxin A (BTX/A) on the gastroc–soleus muscle in ambulant children with cerebral palsy. In this prospective before and after trial, 15 children with diplegia and 10 children with hemiplegia were studied (mean age 5 years 7 months, range 4 years to 9 years). A range of standardized clinical measures was undertaken but the emphasis for this report is on the three-dimensional gait analysis (3DGA) results. All children showed improvements in sagittal ankle kinematics, as has been previously reported. Two new measures of ankle kinetics were devised: ankle moment quotient (AMQ), and ankle power quotient (APQ). Before intervention, ankle moments were characterized by a ‘double bump’ ankle moment. A typical abnormal baseline ankle-power curve was triphasic with an initial trough of absorption followed by abnormal mid-stance power generation, instead of the usual A1 pattern, and reduced terminal stance power generation (A2). Three weeks after treatment with BTX/A alone there was a statistically significant improvement of AMQ and APQ; some patients required potentiation of BTX/A with a short period of serial casts. Both groups (BTX/A alone and BTX/A plus casting) continued to show improvement in ankle kinetics from baseline after 12 and 24 weeks. This is the first study to demonstrate improvements in the typical abnormal ankle kinetics which we believe provides evidence of the ‘biomechanical transformation of muscle’.

This study aimed to analyse the relationship between supratentorial irradiation dose and the intellectual outcome in 36 children (aged between 5 and 15 years) treated for medulloblastoma. The supratentorial radiation dose was reduced to 25Gy in 23 children and given at the standard dose, 35Gy, in 13 other children. Neuropsychological evaluation was performed at a mean of 4.3 years (SD 4.7 years) after radiotherapy. The supratentorial radiation dose was the principal risk factor associated with impaired intellectual outcome. Verbal fluency, immediate word list recall, block design, and fine motricity of the dominant hand were significantly lower in children irradiated at the standard doses than in those irradiated at reduced doses. These findings suggest that the dose of radiotherapy applied to the brain strongly influences later verbal and non-verbal skills in children with medulloblastoma. This should be taken into account in treatment planning and in rehabilitation programs.

Botulinum toxin A (BTX-A) is widely used in the management of muscle spasticity in children. However, at present the dose of BTX-A for a given patient is selected empirically. The aim of this study is to provide dosage guidelines that are based on risk/benefit assessment. This was a multicentre retrospective study of the safety profile and efficacy of BTX-A in children with chronic muscle spasticity. Data in 758 patients who received a total of 1594 treatments were analysed (mean age 7.2 years; 429 males, 329 females). Spastic cerebral palsy (CP) was the most common diagnosis (94% of the study sample). Of all treatments 7% resulted in adverse events; incidence was related to the total dose rather than the dose calculated on the basis of body weight. The highest incidence of adverse events was observed in patients who received >1000 IU of BTX-A per treatment session. The odds of an adverse event was 5.1 times greater for this group of patients than for those who had 250 IU or less (p<0.001). A good overall response to treatment was reported in 82% and treatment goals were fully or partially achieved in 3% and 94% of participants respectively. More patients in the highest dose group reported functional deterioration. Interestingly, multilevel treatments resulted in a better response than single-level treatments (odds ratio 1.7, 95% CI 1.3 to 2.2, p=0.001).

The presenting characteristics of 18 (11 female, seven male) children with Landau–Kleffner syndrome (LKS) were studied with respect to course and outcome of their condition at a mean length of 67 (SD 46) months' follow-up. All had regression of receptive language (mean age of onset 4 years 9 months) and electrical status epilepticus in sleep (ESES). Length of ESES correlated strongly with length of period between onset of illness and onset of recovery (p<0.006) and also with eventual receptive (p<0.001) and expressive (p<0.007) language. Behaviour during the acute phase was severely affected in nine children and associated with frontal lobe discharges in awake EEGs (p<0.004). Age at onset was not correlated with outcome. All children had impaired short-term memory at follow-up. Three children had language outcome within the normal range. No child with ESES lasting longer than 36 months had normal language outcome. These data lend support for intervention in ending ESES by 36 months using multiple subpial transection (MST) if steroids are ineffective or cause unacceptable side effects.

The present study investigated the reliability of self-reported rating of Gross Motor Function Classification System (GMFCS) levels compared with professional rating, and changes in gross motor function over time, in adults with cerebral palsy. Twenty-nine females and 33 males aged between 18 years 5 months and 62 years 11 months (mean age 34y 7mo [SD 10y 6mo]) participated in the study. Participants rated their current gross motor function using the GMFCS and reported their judgement of their gross motor function at age 10 to 12 years. The project leader, a physical therapist, also classified participants' current GMFCS levels and conducted a chart review on all accessible medical records of participants' gross motor function when they were 10 to 12 years old, rating the GMFCS level accordingly. Intraclass correlation coefficients (ICC) between self-reported and professional ratings showed excellent agreement (ICC=0.93–0.95, 95% confidence interval [CI] 0.89–0.97). More than half the participants experienced a stable gross motor function from the age of 10 to 12 years to the present. Those at GMFCS Levels II and III at the age of 10 to 12 years (according to the professional rating) had significant change for the worse in gross motor function over time, with odds ratios of 9.30 (95% CI 1.2–73.0, p=0.03) and 7.00 (95% CI 1.1–43.0, p=0.04) respectively. Interview data on circumstances regarding changes in gross motor function since childhood are also reported. Changes in GMFCS level were mostly associated with physical or social environmental factors.