This study compares the urinary excretion of the main melatonin metabolite, 6-sulfatoxymelatonin (6SMT), in infants who have and have not experienced a life-threatening event (ALTE). 6SMT was assessed in the following groups of infants: 15 infants with ALTE for whom home monitoring had been recommended, 15 infants who had had an abrupt cyanotic apneic event but did not require mouth-to-mouth resuscitation, 15 siblings of those who had died from sudden infant death syndrome (SIDS), and 35 age-matched healthy comparison infants. All 80 infants were between 48 and 58 weeks of postconceptional age. On a double-blind basis, the total amount of 6SMT excreted over 24 hours and the diurnal rhythm in the rate of 6SMT excretion were assessed using urine samples taken from disposable diapers (nappies). The mean daily excretion of 6SMT was significantly lower in the ALTE (1588ng/24 hour) than in the comparison infants (3961ng/24 hour). No such difference was found between the infants with a cyanotic apneic event (3268ng/24 hour) and the SIDS siblings (2962ng/24 hour). The diurnal 6SMT rhythms in the ALTE infants were characterized by lower 24-hour mean and amplitude values, whereas the time of peak and nadir excretion rates (07:15 to 08:45 hours and 14:45 to 16:15 hours respectively) was similar in all four infant groups. Follow-up of the ALTE infants, performed 6 to 8 weeks later (59 to 66 weeks of postconceptional age), revealed that 6SMT excretion increased in all of them, suggesting a delayed ontogeny rather than permanent deficiency of melatonin production in ALTE.

The effects of recreational horseback riding therapy (HBRT) on gross motor function in children with cerebral palsy (CP: spastic diplegia, spastic quadriplegia, and spastic hemiplegia) were determined in a blinded study using the Gross Motor Function Measure (GMFM). Seventeen participants (nine females, eight males; mean age 9 years 10 months, SE 10 months) served as their own control. Their mean Gross Motor Function Classification System score was 2.7 (SD 0.4; range 1 to 5). HBRT was 1 hour per week for three riding sessions of 6 weeks per session (18 weeks). GMFM was determined every 6 weeks: pre-riding control period, onset of HBRT, every 6 weeks during HBRT for 18 weeks, and 6 weeks following HBRT. GMFM did not change during pre-riding control period. GMFM Total Score (Dimensions A–E) increased 7.6% (p<0.04) after 18 weeks, returning to control level 6 weeks following HBRT. GMFM Dimension E (Walking, Running, and Jumping) increased 8.7% after 12 weeks (p<0.02), 8.5% after 18 weeks (p<0.03), and remained elevated at 1.8% 6 weeks following HBRT (p<0.03). This suggests that HBRT may improve gross motor function in children with CP, which may reduce the degree of motor disability. Larger studies are needed to investigate this further, especially in children with more severe disabilities. Horseback riding should be considered for sports therapy in children with CP.

Aicardi-Goutières syndrome (AGS) is an early-onset progressive encephalopathy characterized by calcifications of the basal ganglia, white matter abnormalities, chronic cerebrospinal fluid (CSF) lymphocytosis, and/or a raised level of CSF interferon (INF)-α. We report a female with mitochondrial respiratory chain deficiency fulfilling the criteria of AGS. Disease onset was in the first year of age with seizures and psychomotor regression. To date, at 4 years of age, she presents a severe encephalopathy, increased INF-α in the CSF, and calcifications of basal ganglia on computerized tomography. Cerebral magnetic resonance imaging showed bilateral and symmetric hypersignal of the posterior white matter. A complex I deficiency of the mitochondrial respiratory chain was found in skeletal muscle, which was associated with a complex IV deficiency in cultured skin fibroblasts. The question of whether this oxidative phosphorylation deficiency is primary or secondary in AGS is open to debate. We suggest giving consideration to systematic evaluation of the mitochondrial respiratory chain in skeletal muscle and skin fibroblasts of other AGS patients.

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.

The aim of this study was to evaluate the predictive value of corrected head circumference (HC) centiles at 2 years of age with respect to developmental performance in a series of high-risk neonatal intensive care unit (NICU) survivors with microcephaly. The study used a retrospective review of the clinical files of children seen in a clinic devoted to the follow-up of all high-risk survivors of a hospital's level III NICU. All children with microcephaly (occipital–frontal circumference below the 2nd centile for sex) at 2 years of age were identified. The HC obtained at 2 years was corrected to the ages for which the absolute HC corresponded to either the 50th or 2nd centile for the child's sex. Of 312 high-risk patients followed, 38 (12.2%) were microcephalic. Fifteen performed below the 50th age-corrected HC centile (severe developmental delay), 12 performed between the 50th and 2nd age-corrected HC centile (moderate developmental delay), and 11 performed above the 2nd age-corrected HC centile (mild developmental delay). The absolute value of HC measurement was not a predictor of developmental performance. Of all clinical factors evaluated, only coexisting epilepsy was found to be a significant predictor of less than the 50th age-corrected HC centile developmental performance (χ2=6.134, p=0.01). We conclude that in a high-risk population, the presence of microcephaly implies developmental impairment, though neither the absolute HC measurement nor the corrected HC centile is predictive. Coexisting epilepsy in this context appears to worsen developmental outcome.

The aim of this prospective follow-up study was to assess the effects of pre- and perinatal factors on children's behaviour at the age of 3 years. Nulliparous women who attended a maternity health care clinic were invited to participate: 1443 women were included. Data were obtained from the mother, father, and clinic nurse. A Finnish translation of Achenbach's Child Behavior Checklist for Ages 2–3 was completed by 1086 families at the child's 3-year visit to the well-baby clinic. The results were analysed using Pearson's χ2, multivariate regression analysis, and coefficient of determination (100xr2). A good basic education (i.e. >9 years) of the mother and age over 25 years were associated with low syndrome-scale scores, indicating fewer behavioural problems. Poor health of the mother, a high number of pregnancy-related symptoms, and low Apgar scores for the newborn infant were associated with high syndrome-scale scores. In this population sample, the mother's health during pregnancy together with level of education, and marital status are more important in explaining the variation in a child's behaviour than biological risks during pregnancy.

Neurofibromatosis type 2 (NF2) remains a challenging diagnosis in childhood where there may be no neurological involvement. A 12-month-old male in whom NF2 was suspected because of characteristic ophthalmological and cutaneous lesions is reported. Cranial MRI showed no tumours. A pathogenic mutation was identified on NF2 gene analysis. The child developed hypertension due to renal vascular disease. Although renal vascular disease is a recognized complication of neurofibromatosis type 1 (NF1), it has not been reported in NF2.

Respiratory sinus arrhythmia (RSA), a non-invasive indicator of vagal regulation of the heart, and heart period (HP) were monitored before, during, and after oral or gastric-tube bolus feedings in 32 preterm infants. Group 1 infants (n=15) were ≤30 weeks gestational age (GA) at birth (mean 28.3 weeks) and group 2 infants (n=17) were ≥31 weeks GA at birth (mean=33.2 weeks). Mean postmenstrual ages at the time of study were 33.5 ± 2.3 (SD) weeks in group 1 and 33.9 ± 1.6 (SD) weeks in group 2. RSA and HP decreased in both groups during feeding. However, postfeeding RSA and HP increased toward prefeed levels only for group 2 infants. In addition, RSA and HP changes during feeding were correlated only for group 2 infants. The results suggest that the preterm infant may experience a maturational lag in vagal function and in the influence of vagal activity on metabolic mechanisms (i.e. heart rate) related to ingestive needs. This maturational lag may contribute to continued feeding difficulties and may be a measurable marker of subtle neurodevelopmental problems.

Attempts at subtyping attention-deficit–hyperactivity disorder (ADHD) along the hyperactivity dimension are considered controversial. This study addresses this issue by dividing a non-clinical sample of Brazilian children (mean age, 9.4 years; SD, 2.9), who were attending a mainstream school in the Greater Rio de Janeiro area, into four behavioral domain groups (normal [NO, N=324], hyperactive/impulsive [HI, N=17], inattentive [IA, N=48], and combined [C, N=13]) on the basis of teacher ratings on an ADHD scale. The groups did not differ in intellectual level as determined by the Human Figure Drawing test. Comparisons were made between groups along the factorial dimensions extracted from the Composite Teacher Rating Scale, academic performance and neuropsychological measures were then performed. Our data showed that IA and C children are less independent and more prone to socialization problems than NO children, and that HI and C children are less anxious and fearful than IA children. Furthermore, the groups differed in academic and neuropsychological performance. The results could be considered consistent with the hypothesis that ADD with hyperactivity (ADD/+) and ADD without hyperactivity (ADD/−) represent singular nosological entities.

Secondary tethering of the spinal cord in people with myelomeningocele (MMC) is almost universal but there is a relatively low incidence of the secondary tethered cord syndrome (S-TCS). In view of this, we wish to explore the notion that cord pulsation, as demonstrated by ultrasound of the spinal cord, can become an independent measure for increased tension of the spinal cord in patients with MMC. One-hundred and six patients with MMC underwent ultrasonographic examination of their back, a thorough physical examination, and a careful review of their clinical history. Of these patients, 53 had MRI of their back. Cord pulsation was graded and the statistical relation between pulsation and various clinical and MRI findings was analysed. Age and arm span were not correlated with grades of cord pulsation. The absence of cord pulsation correlated with the presence of symptoms but this relation may be explained by MRI findings of certain structural cord malformations and not by tethering per se. Assuming that cord pulsation is a reliable marker for the tension within the spinal cord, the results of our study suggest that overstretching of the cord is not the main cause for the development of the S-TCS.

This study aimed to determine the frequency and outcome of possible cerebral herniation in relation to lumbar puncture (LP) in postneonatal infants and children with pyogenic meningitis in the tropics. Children with meningitis aged between 6 weeks and 15 years (mean age 4.07 years; n=123) were recruited consecutively over 3 ½ years at the University of Maiduguri Teaching Hospital, Nigeria. The frequency of possible herniation was determined by clinical evaluation in relation to the severity of illness (high versus low risk) on presentation and performance of LP. Previously described scoring schemes were used. Eighteen (15%) patients had evidence of herniation on presentation. The relative risk (RR) of herniation in high- versus low-risk patients was 66.6 (9.3 to 477.1, 95% CI), p<0.0001 while the RR of death or neurological sequelae in high- versus low-risk patients was 2.6 (1.8 to 3.7, 95% CI), p<0.0001. In 99 patients with known outcomes who had LP on presentation, 21 of 81 without herniation pre- or post-LP, four of four with herniation pre- and post-LP, seven of eight with herniation post-LP only, and five of six with herniation pre-LP only died or recovered with sequelae (χ2 = 25.24, df = 3, p<0.0001). It is concluded that outcome depends on the severity of illness and the presence and timing of herniation. A policy of selective rather routine LPs may improve the outcome in meningitis in developing countries.

The purpose of this pilot study was to evaluate the effect of treatment with botulinum toxin A (BTX-A) injections on function in children with cerebral palsy (CP) using pedobarometric data collected in a standing position. Ten children with CP (seven males, three females) participated, five with diplegia and five with hemiplegia. All children exhibited signs of unilateral dynamic equinus foot disorder and were community ambulators. Age range at the time of treatment was 6 to 13 years (mean age 9y, SD 2.5). A comprehensive clinical examination and a pedobarometric evaluation were performed before and after BTX-A injection. All children received at least one injection in each spastic muscle. BTX-A was given to eight children unilaterally and to two children bilaterally. A maximum dose of 4 to 8 units of BTX-A per kilogram body weight was administered. In comparison with pretreatment values, the pedobarometric evaluation revealed a significant increase in the whole plantar surface area on the affected side (p<0.05), an increase in the peak pressure value at the hindfoot on the affected side (p<0.05), and a significant shift in the body centre of mass to the affected limb (p<0.05) 1 month after treatment with BTX-A. A significant decrease in the peak pressure value at the hindfoot on the unaffected side was also observed. Similarly, a decrease in the modified Ashworth scale and an increase in the range of motion were observed. Thus, BTX-A produces a significant plantar surface modification and a significant shift of the body centre of mass which represents an indirect measure of postural attitude. Pedobarometric evaluation can also be useful in quantifying clinical changes after treatment with BTX-A.

The aim of this study was to determine the long-term outcome of neurosurgical untethering on neurosegmental motor level and ambulation level in children with tethered spinal cord syndrome. Forty-four children were operated on (17 males, 27 females; mean age at operation 6 years 2 months, SD 5 years). Sixteen patients had myelomeningocele, nine had lipomyelomeningocele, and 19 had other types of spinal dysraphism. Motor level and ambulation level were assessed pre- and three times postsurgery (mean duration of follow-up 7 years 1 month, SD 1 year 8 months). Deterioration of motor level was seen in five of 44 patients, 36 of 44 remained stable, while improvement was seen in three of 44 patients. Deterioration of ambulation level was seen in five of 44 patients, and remained stable in 26 of 44. Thirteen of 44 children were too young to ambulate at time of operation (<2 years 6 months). Late deterioration of motor or ambulation level was only seen in (lipo)myelomeningocele patients. Deterioration of ambulatory status was strongly associated with obesity and retethering. Revision of the initial tethered cord release was performed in nine of 44 patients, mainly in those with lipomyelomeningocele.

A four-year-old male with symptomatic generalized epilepsy presented with ataxia, eye rolling, and episodes of back arching which were of non-epileptic origin following the introduction of clobazam at 0.75mg/kg/day. Concurrent antiepileptic medication was lamotrigine at 13mg/kg/day. Clobazam plasma levels were within the normal range, while N-desmethylclobazam (DCLB) concentrations were between five and seven times above the upper limit of the normal range. The plasma elimination half-life for DCLB was prolonged, suggesting a genetic variability in DCLB metabolism leading to toxicity. Reduction in the dose of clobazam to 0.3mg/kg/day was associated with resolution of the non-epileptic neurological symptoms, reduction in DCLB plasma levels, and maintenance of seizure control.

This study evaluated the efficacy and safety of three doses of botulinum toxin A (BTX-A; Dysport®) in 125 patients (mean age 5.2 years, SD 2; 54% male)with dynamic equinus spasticity during walking. Participants were randomized to receive Dysport (10, 20, or 30 units/kg) or placebo to the gastrocnemius muscle of both legs. Muscle length was calculated from electrogoniometric measurements and the change in the dynamic component of gastrocnemius shortening at four weeks was prospectively identified as the primary outcome measure. All treatment groups showed statistically significant decreases in dynamic component compared with placebo at 4 weeks. Mean improvement in dynamic component was most pronounced in the 20 units/kg group, being equivalent to an increase in dorsiflexion with the knee extended at 19°, and was still present at 16 weeks. The safety profile of the toxin appears satisfactory.

This paper describes part of larger study involving 40 families of children and adolescents with neuropathic bladder looking at the psychosocial impact of catheterization on them and their families. This study focuses on their experiences of clean intermittent catheterization (CIC) and self-catheterization (SC). Twenty-eight children and adolescents (11 males, 17 females; mean age 9 years, SD 2 years 7 months, range 5 to 20 years) with neuropathic bladder (mostly with spina bifida) participated in a semi-structured interview to explore their understanding of micturition and catheterization, and their views about the introduction and practical use of this technique. Participants were invited to draw self-portraits and figure diagrams to depict their understanding of CIC and SC. Age-appropriate self-esteem measures (Harter Pictorial Scale of Perceived Competence, the Self-Perception Profile, and the Culture-Free Self-Esteem Inventory) were administered to see if catheterization status affected emotional well-being. In an extension of this study, children's experiences of SC were explored by a postal questionnaire to all children successfully using SC who were attending the Paediatric Neuropathic Bladder Clinic. Children aged <5 years, those with learning difficulties, and those using indwelling catheters were excluded. From a total sample of 66 (31 females, 35 males), 52 parents and 42 children and adolescents (28 females and 14 males) responded. No significant difference was observed in self-esteem for those successfully catheterizing. Specific challenges involved learning SC and practical use of the technique. Concerns were leakage and being wet, and peers finding out about their continence management. Implications for the education, problem solving, and support of families and young people to promote cooperation and optimal benefits from catheterization are discussed.

In the past few years there have been a number of new surgical interventions in the treatment of cerebral palsy (CP) and these were reviewed in a meeting at the London Royal Society of Medicine, arranged by the Mac Keith Meetings committee, in October 1997. One such intervention, selective dorsal rhizotomy (SDR), is a modification of a technique first used at the turn of this century. In the mid-1980s, SDR was reintroduced by Peacock and Arens for children with CP. It was established in South Africa and the United States, then spread to Canada and parts of Europe. The continuous infusion of intrathecal baclofen (CIBI) to relieve spasticity in CP has only been used this decade. It was pioneered mainly in North America, and followed experience with its use in spinal spasticity, for which it is particularly effective. Lastly, we considered the practice of multilevel orthopaedic surgery which started over 20 years ago, but has recently become popular throughout North America and Europe. Guided by clinical examination and information from laboratory gait analysis, this approach addresses all of the mechanical problems in the lower limb at one stage and attempts to avoid the need for separate procedures throughout childhood – the ‘birthday’ syndrome. All of these new techniques are costly in effort and time for the child and all involved, and thus need careful evaluation. This was provided at the meeting by speakers from a variety of backgrounds and countries.

Studying the increase in the rate of oxygen uptake (V[dot above]O2) during the transition from rest to exercise (V[dot above]O2 kinetics) provides important information about aerobic function during a metabolic state that mimics habitual activity far more closely than traditional steady-state exercise. The time constant of V[dot above]O2 (τ V[dot above]O2) provides a method of quantifying the V[dot above]O2 kinetic response in the non-steady state. It can also be used to quantify the O2 deficit, which provides a means of assessing the ‘strain’ on non-aerobic energy sources at exercise onset. Children with spastic cerebral palsy (CP) do not tolerate exercise well, and report early fatigue. Precise causalities are not known, although investigating V[dot above]O2 kinetics in this population has the potential to enhance our understanding of the underlying mechanisms. In addition, there are an increasing number of practitioners in the field of paediatric CP who use V[dot above]O2 measurements to evaluate the efficacy of interventions and rehabilitation regimens. Measuring V[dot above]O2 kinetics can complement the traditional cardiorespiratory outcome measures, providing much useful information about the combined function of the respiratory, circulatory, and metabolic systems. Importantly, this can be achieved by using submaximal exercise intensities, reducing the stress placed on participants. To our knowledge there are no published data describing the V[dot above]O2 kinetic responses of children with CP. This review seeks (1) to summarize the main findings from V[dot above]O2 kinetics research involving non-impaired adults and children so that measuring V[dot above]O2 kinetics in children with CP can be considered within an appropriate framework, and (2) to address methodological issues directly related to measuring kinetics in children with CP.

A study of the psychosocial functioning of young adults with a history of learning disability (LD) in childhood is reported. The design was a non-clinic community follow-up investigation of a cohort of children who had received special education, the sample frame for which was a birth-period cohort survey. Participants were 149 young adults (89 males and 60 females derived from a survey of the 33800 children born in the Cambridge Health District between 1967 and 1973) at the stage of transition to adult services (age 18–22y), whose measured IQ scores in childhood were <80, and who had received special education during school years. Most of the young adults were living at home (n=108) and many were in full-time employment (independent employment n=41, sheltered employment n=20), this survey having been carried out in an area of high employment. A strong correlation was found between the child IQ score and subsequent adult Vineland Scale Score. Greater efforts should be made to detect and assess LD and its attendant problems in children in order to plan future care and transition to adulthood, especially for those with more severe disabilities.