This study investigated the accuracy of prediction of neurodevelopmental outcome at 1 year using cerebral proton magnetic resonance spectroscopy (MRS) and structured neonatal neurological assessment in term infants after presumed hypoxic–ischaemic brain injury. Eighteen control infants and 28 infants with presumed hypoxic–ischaemic brain injury underwent proton MRS investigation. Studies were carried out as soon as possible after the cerebral insult, most within 48 hours. Infants had an early structured neurological assessment at a median of 19 hours (range 0 hours to 9 days) from the presumed hypoxic–ischaemic insult and a late assessment at a median of 7 days (range 3 to 25 days) during recovery. The maximum cerebral peak–area ratio lactate:N-acetylaspartate measured by proton MRS accurately predicted adverse outcome at 1 year with a specificity of 93% and positive predictive value of 92%. Neurological assessment had a tendency for false-positive predictions. However, both early and late neurological examination can be used as a reliable indicator for a favourable outcome at 1 year having negative predictive values of 100% and 91% respectively.

According to traditional neurophysiological theory, consciousness requires neocortical functioning, and children born without cerebral hemispheres necessarily remain indefinitely in a developmental vegetative state. Four children between 5 and 17 years old are reported with congenital brain malformations involving total or near-total absence of cerebral cortex but who, nevertheless, possessed discriminative awareness: for example, distinguishing familiar from unfamiliar people and environments, social interaction, functional vision, orienting, musical preferences, appropriate affective responses, and associative learning. These abilities may reflect ‘vertical’ plasticity of brainstem and diencephalic structures. The relative rarity of manifest consciousness in congenitally decorticate children could be due largely to an inherent tendency of the label ‘developmental vegetative state’ to become a self-fulfilling prophecy.

Is it possible to predict relatively early in the life of children with disabilities those who are likely to develop peer problems if they get no extra help? This question is examined in our prospective study of a representative sample of children with hemiplegia attending mainstream schools. Hemiplegia is a particularly suitable model for studying integration as it is a relatively homogeneous condition that does not usually preclude mainstream placement. Our aim was to investigate whether the excess of peer problems could be predicted from information obtained some 4 years earlier. A representative sample of 55 children with hemiplegia in mainstream education was followed prospectively from a mean age of 7.1 years (Time 1) to a mean age of 10.7 years (Time 2). Using standardized measures of peer rejection, lack of friends, and victimization, two-thirds of the sample had at least one of these problems at Time 2. A greater number of peer problems was primarily predicted by two Time-1 variables: lower IQ and more teacher-reported externalizing problems (disruptiveness and hyperactivity). A risk index based on these two variables identified a high-risk subgroup that might particularly have benefited from early intervention to reduce behavioural problems, and nurture social skills. As more children with special needs are integrated into mainstream schools, it is increasingly important to remember that supporting these children requires appropriate provision to foster their social as well as their academic and physical development.

It is well known that individuals with cerebral palsy (CP) are subject to higher mortality than the general population but the causes of this have not been systematically analysed. This study investigates mortality in a Californian population of 45292 individuals with CP, 4028 of whom died during the 1986 to 1995 study period. The aims of this study were to identify diseases that may be causally linked with CP, and diseases whose diagnosis and/or treatment need improvement. Overall, the standardized mortality ratio (SMR) was 8.4. Mortality from breast cancer was three times that of the general population, suggesting poorer detection and/or treatment. The dramatic elevation of mortality due to brain cancer, especially in children (SMR=24), raises the possibility of a link between this and CP. Cause of death was non-specific in some individuals. Therefore, these SMRs are conservative. As expected, SMRs due to respiratory diseases were very high but, contrary to anecdotal reports, such diseases did not account for most deaths. High SMRs were also found for diseases of the circulatory and digestive systems. Finally, a marked elevation of deaths was due to external causes, including drowning and being hit by motor vehicles.

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.

Handedness is the most prominent manifestation of behavioural lateralization in the human population. However, little is known about its ontogeny. This study examined the prenatal development of behavioural lateralization by observing, longitudinally, the occurrence of left- and right-arm movements in 17 human fetuses at 3-week intervals from 12 to 27 weeks gestational age. Fetuses exhibited significantly more right-arm movements than left-arm movements at every gestational age. Throughout all periods of observation, 83.3% of fetuses showed more right-arm than left-arm movements. The number of arm movements observed peaked at 15 to 18 weeks' gestation and declined rapidly in mid-gestation. The occurrence of laterality in early gestation indicates that asymmetric behaviour is probably under muscular or spinal, rather than cortical, control and points to a ‘genetic’ origin for such behaviour. The presence of lateralized motor behaviour in early gestation suggests it may have a potential causative role for subsequent lateralized behaviour and asymmetric brain development.

The present investigation examines the relation between various clinical measures and the performance of a functional precision grip–lift task. Fifteen children with hemiplegic cerebral palsy (CP), aged 8 to 14 years, and 15 age-matched control children grasped and lifted an object whose surface texture was varied while their fingertip forces were recorded. The force coordination was compared with tactile sensibility, grip strength, manual dexterity, and spasticity using correlational and regression analyses. The findings highlight the importance of tactile sensibility in this task. However, the manner in which sensibility was related varied for the sensory adaptation of fingertip forces, the anticipatory scaling of the force increase, and the smooth transitions between the temporal phases comprising the grip–lift task. The findings also indicate that spasticity affects some measures of the task, but not others, suggesting that the relation between spasticity and motor performance may not be absolute. The results further suggest that the impairments in grasping in children with hemiplegic CP are largely but not exclusively due to disturbed sensory mechanisms which may have direct implications for therapeutic intervention.

Damage to the occipital cortex in children can result in many complex disorders of cognitive visual function. A series of clinical questions, developed from the specific problems of a cohort of children with cortical visual impairment, was asked of the parents of 200 children with no history of cerebral pathology, aged 5 to 12 years. One hundred and ninety-two parents gave reliable consistent responses. The results show a progressive improvement in performance with age, culminating in few 11- and 12-year olds having frequent problems, apart from 8% having frequent difficulty with orientation in new surroundings and 2% having problems with simultaneous perception tasks. The parents of 52 children (aged 5 to 17 years) with shunted hydrocephalus were then asked the same set of questions. Evidence of cognitive visual problems was identified in 27 of these children of whom 16 manifested multiple difficulties. The disabilities identified by our study comprised problems with: shape recognition, simultaneous perception, perception of movement, colour perception, orientation, object recognition, and face recognition. The range, nature, and combinations of these disorders are presented in this paper.

The aim of this study was to examine children with myelomeningocele (spina bifida) who were investigated according to the level of neurosegmental lesion and to determine the number who walked and their average age at commencement/cessation of walking. The data have been collected since 1978 at the Royal Children's Hospital, Melbourne during annual evaluations of patients with spina bifida and high-level paralysis. After screening for established criteria and assigning to a neurosegmental lesion level, 173 children were examined for their ability to walk, with or without aids, in a community setting. Thirty-five children had lesions at the thoracic level, and seven walked at a mean age of 4 years 6 months. Three children with a lesion at the thoracic level ceased walking at a mean age of 7 years 6 months. Ten children had lesions at the high-lumbar level (L1/2), and five walked at a mean age of 5 years 2 months. Three children who had lesions at the high-lumbar level ceased walking at a mean age of 6 years 11 months. Fifteen children had lesions at the mid-lumbar level (L3) and nine walked at a mean age of 5 years. Three ceased walking at a mean age of 7 years. Forty-five children had lesions at the low-lumbar level (L4/5) and 38 walked at a mean age of 3 years 10 months. Five ceased walking at a mean age of 9 years 1 month. Sixty-eight children had lesions at the sacral level; all of whom walked at an average age of 2 years 2 months. None had ceased walking by the end of the study period. Delay in achieving ambulation can be expected in all children with spina bifida, including those with low neurosegmental level lesions. Furthermore, some children with high lesion level, given the opportunity, might be expected to ambulate effectively in the community but may cease walking after 3 to 4 years of this activity, which is earlier than previously recorded.

Between 1989 and 1994, 18 children with cryptogenic infantile spasms – defined by normal development before onset of spasms, symmetrical hypsarrhythmia or multifocal spikes, and typical spasms on presentation, and no abnormal findings on aetiological studies including neuroradiology – were diagnosed and treated. To assess the risk of cognitive impairment later in life, 15 of these 18 children whose spasms completely resolved within the first year of life were studied. Age at onset of spasms varied between 4.4 and 9.8 months (mean 6.5 months). Children were effectively treated with adrenocorticotrophic hormone (10 children), pyridoxine (three), vigabatrin (one), or sodium valproate (one). Spasms lasted between 11 and 138 days (mean 50 days) and stopped between the age of 6.3 and 10.2 months (mean 8.1 months). EEGs normalized between the age of 7.1 and 13.2 months (mean 9.4 months). Early development was assessed on presentation and within a few months after spasms had stopped. A detailed neuropsychological assessment was performed between the age of 4.0 and 5.9 years. Twelve children had normal intelligence; specific cognitive deficits were found in five. Three children had mild learning disability. Abnormal developmental status at age 8 to 15 months after complete resolution of spasms and EEG abnormalities was associated with cognitive deficits at age 4 to 6 years.

This paper investigates the prognostic value of flash visual evoked potentials (VEPs) recorded in preterm infants at birth and at term age with respect to severe neurological outcome. Flash VEPs were recorded in 81 preterm infants at birth (i.e. <35 weeks' gestation) and repeated in 56 of these infants at term age. The preterm infants were assigned to either a healthy or at-risk subgroup based on clinical birth factors. Normal ranges of flash-VEP latencies, amplitudes, and number of components present were obtained from the subgroup of healthy preterm infants and from 68 term infants tested postnatally. The flash-VEP results of the entire preterm group were compared with the normal ranges and any abnormalities noted. Seven preterm infants in the at-risk group died, six of whom had abnormal flash VEPs before term age. Of the five infants from the at-risk group diagnosed with cerebral palsy (CP), three had abnormal flash VEPs before term age. Thus the sensitivity and specificity of the flash VEP with respect to survival was 86% and 89% respectively, and with respect to the development of CP was 60% and 92%. The abnormal features of the flash VEP associated with adverse outcomes comprised a delayed N3 component before term age and the absence of a positive component (P2) at term age.

The underlying risk factors, presenting features, and outcome of 22 children with sacral agenesis and associated neuropathic bladder were studied retrospectively. The age of children at presentation was bimodally distributed, with peaks below 1 year and between 4 and 5 years of age. Ten patients presented after 1 year. The oldest was diagnosed at 12 years of age. In 12 children there was maternal diabetes, orthopaedic anomalies in 14, skin defects in 11, and anorectal/tracheooesophageal anomalies in three. Most children had persistent dribbling of urine on presentation associated with frequency, urgency, recurrent urinary tract infections, failure to respond to medication, and/or constipation. Twenty-one children had abnormal neurology in the lower limbs. Videourodynamics showed neuropathic vesicourethral dysfunction in all children and vesicoureteric reflux in 10. Nineteen had a history of urinary tract infections. Seven had renal scarring, with renal impairment in three at presentation. Clean intermittent catheterization was recommended for 20 of the children. Bladder or bowel surgery has been carried out in seven and neurosurgery performed in two. Twenty of the 22 children underwent operative procedures. Ten operations were performed before sacral agenesis was diagnosed. Over a third of the children have required psychological support. The combination of urinary symptomatology and any of the above risk factors should give rise to a high level of suspicion and low threshold to perform investigations to exclude sacral agenesis. All these children have abnormal bladder and urethral function which not only causes incontinence but puts the kidneys at risk. Early detection allows effective multidisciplinary input specifically aimed at continence, preservation of renal function, and adequate psychological support.

Thirty-nine ambulant children (22 with hemiplegia, 17 with diplegia) with spastic cerebral palsy receiving isolated gastrocnemius muscle injection with botulinum toxin A were studied prospectively. The children had a mean age of 6 years (range 3 to 13 years). Measurement of gastrocnemius muscle length was used to estimate the dynamic component of each child's spasticity and to quantify the response. There was a strong correlation between the dynamic component of spasticity before injection and the corresponding magnitude of the response after injection. Children undergoing repeated injections showed similar correlations. A strong correlation was found between the duration of response and the dynamic component. Children with hemiplegia showed twice the duration for a given dynamic component compared with those with diplegia when injected with the same total dose per unit body weight. Long-term lengthening did not occur for the cohort, although some patients showed a response at a 12-month follow-up. By delaying shortening, the injections may have a role in delaying the need for surgery. Injections were well tolerated with few side effects.

This study examines whether a small head circumference (HC) and low head-circumference growth velocity (HGV) during the first year of life predict consequences at school age in learning, cognition, and concentration. A total of 124 extremely-low-birthweight (ELBW) infants (birthweight 500 to 999 g) born between 1977 and 1986 were eligible for follow-up at the corrected ages of 4, 8, and 12 months and 2, 4, and 6 years. Infants were categorized as having a small HC (<3% or 3 to 10%) on the basis of the 1990 British growth data which allowed standardized z-scores to be calculated for HC, independent of gestation and corrected age. HGV measurements were calculated using differences in the HC z-scores. In 1995, parents of 87 children agreed to participate in a study of learning and attention at school age. Attention-deficit–hyperactivity disorder (ADHD) was assessed using the Du Paul Rating Scale. Academic performances were based on a teacher questionnaire dealing with aspects of reading, writing, mathematics, and spelling. A child was considered to have a learning difficulty if academic problems were present in at least one of these four areas. Intellectual ability was assessed using the McCarthy Scale at 6 years. HC <3% and 3 to 10% at 8 months' corrected age was strongly associated with school-aged learning problems (P=0.004), with a moderate specificity (70%), positive predictive value (PPV) (67%), and sensitivity (67%). HGV [les ]10% from birth to 4 months was also associated with learning problems at school age (P=0.01) with a higher specificity (98%) and PPV (88%) but lower sensitivity (20%). A logistic regression analysis was performed with the risk for learning difficulties at 8 months as the dependent variable. Sex, gestation, birthweight, multiple births, and a history of intraventricular hemorrhage did not substantially alter the unadjusted odds ratio (4.7; 95% CI 1.9 to 13.6). Maternal age and education did not confound the relation. No association was found between HC or HGV and ADHD. HC <3% at 4 months (P<0.02), 8 months (P=0.02), and 12 months (P=0.04), and HGV between birth and 4 months (P<0.01) were significantly associated with low cognitive ability at 6 years.

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.

Developmental dysphasia, a severe childhood learning disorder, is thought to result from problems in hemispheric specialization involving both left and right cerebral hemispheres. Regional cerebral blood flow (rCBF) was measured at rest and during stimulation of both hemispheres independently: dichotic listening for the left, dichaptic palpation for the right. Eight right-handed boys with expressive dysphasia, aged 8 to 12 years, were investigated using SPECT and compared with eight right-handed age-matched boys with Duchenne muscular dystrophy with reading disorders but normal speech. rCBF values at rest were also compared with those of five right-handed age-matched normal boys. In the dichotic task, children with dysphasia differed from children with dystrophia by failure to increase rCBF in the left hemisphere, in Broca's area, but rCBF increased in the right hemisphere, in the region homologous to Broca's area. In the dichaptic task, rCBF increased bilaterally for children with dysphasia whereas in children with dystrophia rCBF increased only in the right hemisphere. At rest the physiological asymmetry was reversed in favor of the right hemisphere in all areas except Broca's area. Surprisingly, the same applied at rest and for all areas in children with dystrophia. These results confirm that functional specialization of both hemispheres is impaired in developmental dysphasia. Moreover, they suggest that learning disabilities associated with Duchenne muscular dystrophy could also be related to abnormal hemispheric specialization.

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.

The purpose of this study, through a retrospective epidemiological survey carried out over three geographical areas in France, was to characterize the aetiological factors involved in severe mental retardation (SMR) within a geographically defined population of children with disabilities aged between 7 and 16 years. The inclusion criteria for SMR (IQ<50) were met by 1150 children born between 1976 and 1985. Of these children, aetiology was known in 25%, suspected (or unclear) in 26%, and unknown in 49%. These rates of known and suspected aetiology varied between the groups of children with CP and those without CP. An analysis of factors associated with SMR was undertaken among the 144 subjects with SMR, of suspected or unknown aetiology, who had been referred to a neonatal care unit with or without intensive care (NCU) during their neonatal period. These subjects with SMR were compared with 864 children without SMR (control children) who were also referred to an NCU during their neonatal period. The main specific associated factors were a prolonged intubation of more than 24 hours, a very low birthweight (<1500 g) for children with an associated clinical feature of CP, and the presence of isolated neonatal fits and a time of transfer to the NCU of more than 4 hours after birth for children without an associated clinical feature of CP. Although common associated factors were encountered in the children with SMR with CP and the children with SMR without CP, the results of this study suggest differences in the underlying pathogenic factors.

The quality of general movements (GMs) was assessed repeatedly during the first postnatal months in a mixed group of 52 children at either low or high risk for neurodevelopmental disorders. In addition, all children were reexamined at 4 to 9 years. The follow-up assessment consisted of a neurological examination and an evaluation of behaviour by means of parental questionnaires. The quality of GMs changed frequently, to stabilize in the final phase. The final GM phase is that of the so-called fidgety GMs which occurs between 2 and 4 months postterm. The quality of the fidgety GMs predicted outcome very well. Definitely abnormal GMs were associated with a high risk for the development of cerebral palsy, whereas mildly abnormal GMs were associated with the development of minor neurological dysfunction, attention-deficit–hyperactivity disorder, and aggressive behaviour.

The effects of age on basic fine motor functions were assessed using a kinematic analysis of simple repetitive drawing movements in a sample of 53 right-handed children aged 7 to 14 years 11 months and 16 adults aged 27 to 54 years with no detectable abnormality with neurological examination. Lines, circles, and half-circles were drawn at a self-chosen and maximum velocity with a pressure-sensitive pen on a computer graphics tablet. Patterns generated by finger, wrist, and arm movements, and those generated by combined finger–wrist movements with and without a change in the drawing direction were analysed. The frequencies of all movement patterns increased until adulthood: 0.1 Hz per year for half-circles, 0.15 to 0.2 Hz per year for circles and lines generated by arm and wrist movements. The greatest increase (0.35 Hz per year) was observed for lines produced by finger movements. Differences in movement frequencies between finger versus wrist or arm movements when drawing lines decreased from about 3 Hz in 7- to 8-year-olds to 0.5 to 1 Hz in adolescents. In adults the difference between maximum finger, wrist, and arm movement frequencies decreased to almost zero. In contrast, the differences in movement patterns with and without change in direction remained stable until adulthood. Based on these results it is hypothesized that the differential effects of finger and more proximally generated movements on age reflect maturation of distinct neuronal sites of the cortico-striatal–cerebellar circuit rather than acquisition of motor skills or myelinization of corticospinal pathways.