Previous studies have shown that recurrent severe hypoglycaemia can cause long-term cognitive impairment in children with type-1 diabetes, but the results are controversial, possibly due to the heterogeneity of samples and lack of comprehensive neuropsychological assessments of children. The aim of this study was to assess the effects of diabetes and severe hypoglycaemia on the neurocognitive functioning of children with a standardized, wide age-range neuropsychological test battery designed for the assessment of children. Eleven children with diabetes and a history of severe hypoglycaemia, 10 children with diabetes without a history of severe hypoglycaemia, and 10 healthy control children (a total of 31 children: 14 males and 17 females, age range 5 years 6 months to 11 years 11 months, mean 9 years 4 months, SD 1 year 11 months) were studied using the Wechsler Intelligence Scale for Children–Revised (WISC-R) and the NEPSY, a Developmental Neuropsychological Assessment. The NEPSY assessed development in attention and executive functions, language, sensorimotor functions, visuospatial processing, and learning and memory. Children with a history of severe hypoglycaemia had more neuropsychological impairments, more learning difficulties (as reported by parents), and needed more part-time special education than those in the other groups. Significant differences were found in verbal short-term memory and phonological processing. Results suggest that severe hypoglycaemia is a risk factor for learning due to deficits in auditory–verbal functioning.

This was a qualitative research study of parental perceptions of gastrostomy feeding before surgery using an in-depth interview in the parental home to examine the factors parents consider when gastrostomy feeding is recommended and to identify the need for support. Participants were a subgroup of families taking part in a larger research study evaluating gastrostomy placement in children with severe neurodevelopmental disabilities at a major paediatric centre in the UK between 1998 and 2000. Parents of 22 children (13 males, nine females; mean age 4 years 8 months [SD 3 years 6 months]; age range 1 year 1 month to 13 years 3 months) were interviewed. Categories of disability were: cerebral palsy (n=10), a syndrome of chromosomal or genetic origin (n=10), and unconfirmed diagnoses (n=2). Thirteen children were recommended for a gastrostomy and seven were recommended for a gastrostomy with an antireflux procedure. Four families were undecided about surgery at the time of the interview, two of whom went ahead with the procedure some time later. Parental accounts of oral and tube feeding were both contradictory and ambivalent. Concerns about the loss of oral feeding, which was regarded as having a range of psychosocial effects for the child and family, were raised. Both oral and tube feeding have multiple meanings for parents and signify more than obtaining an adequate nutritional intake. The need for additional information about the risks and benefits of gastrostomy and how tube feeding fits into the context of everyday life were dominant themes. The biomedical emphasis on health and weight-gains may fail to reflect parental concerns about tube and oral feeding. There is a need for greater practical and emotional support for families feeding children with severe disabilities.

To spare more children from painful muscle biopsy, a new non-invasive diagnostic motor performance test is undergoing development. Fifteen functional items were used to measure muscle strength and muscle endurance in 68 patients (47 males, 21 females; mean age 7y 8mo, SD 2y 2mo; range 4 to 11y), who had been referred to our specialist centre in the past 3 years on suspicion of myopathy. All the patients had undergone muscle biopsy. To correct the patients' outcomes for age, sex, and body size, regression prediction equations were obtained from a stratified random sample of 64 normally developing primary-school children aged 4 to 11 years (32 males, 32 females; mean age 8y 1mo, SD 2y 4mo). Feasibility was evaluated on the basis of five criteria. Validity was assessed using logistic regression analysis, receiver operating characteristic analysis, and sensitivity and specificity at a specifically chosen cut-off point. Reproducibility was evaluated by test–retest reliability in a stratified random sample of 40 patients who returned for re-measurements using the intraclass correlation coefficient. Seven items satisfied all five feasibility criteria, had high diagnostic power, and high test–retest reliability. The motor performance test can improve diagnostic procedure in children suspected of having myopathy.

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.

Cerebral palsy (CP) consists of a wide range of lifetime physical disabilities caused by damage to the brain during intrauterine and early neonatal life. The risk of CP is increased among survivors of prematurity and in low-birthweight (LBW) infants. Increased prematurity and LBW rates are more common in multifoetal pregnancies, therefore, these pregnancies have also been associated with increased rates of CP. Given the world wide epidemic dimensions of multiple births, this paper discusses current concepts regarding CP in multifoetal gestations.

Skeletal health in children is made up of a number of factors including the overall pattern of the skeleton, the appropriate size and shape of individual bones, bone mass and internal architecture of both tubular and plate-like bones, and bone metabolism and turnover. Where among this group of attributes does skeletal maturity fit in?

This study aimed to describe clinical findings, pedigrees, and possible environmental risk factors in children with clinical anophthalmos and remnant microphthalmos in either eye in southern India. Twenty-four children (14 male, 10 female; mean age 10.3 years, age range 1.3 to 18 years,) were recruited from schools for the blind, hospitals, and community-based rehabilitation programmes in Andhra Pradesh, India, over 1 year. Family members were examined, and mothers interviewed. Fifteen children had anophthalmos and nine had remnant microphthalmos in one or both eyes. Twelve children had associated systemic findings, of which six were major and six were minor abnormalities. Information on consanguinity was available in 19 children, 12 of whom had consanguineous parents. Five children had a positive family history. Two mothers had a history of night blindness, and one had a history of pesticide exposure during pregnancy. High rates of consanguinity suggest a genetic recessive aetiology.

Sixty-seven consecutive patients (59 females, eight males; mean age 13.6 years, age range 8.1 to 18 years) with adolescent idiopathic scoliosis were evaluated for segmental dystonia, thermal and vibratory thresholds, and cortical somatosensory evoked potentials (cSEPs). Clinically, there were no signs of segmental dystonia. No significant side difference was found in cSEPs and thermal thresholds. Vibration thresholds were increased on the right side with a significant side difference at all sites. However, these changes did not correlate to curve size, rotation, curve type, or convexity. It was concluded that, at the present level of somatosensory testing, no segmental neurophysiological disturbance can be related to the appearance of idiopathic scoliosis. The importance of asymmetric vibratory thresholds measurements cannot be deduced from this study.

Very-low-birthweight (VLBW) individuals are at high risk of brain injury in the perinatal period. We wished to determine how such early brain lesions affect brain structure in adulthood. Thirty-two VLBW adults (20 female, 12 male) and 18 term, normal birthweight sibling control individuals (nine female, nine male) underwent structural MRI at a mean age of 23 years 4 months (range 17 to 33 years; SD 3.4). Images were analyzed using an automated tissue segmentation algorithm in order to estimate whole brain tissue class volumes in native space. Images were then warped to a template image in standard space. There was no significant between-group difference in whole brain, grey matter, white matter, or total cerebral spinal fluid (CSF) volumes. However, lateral ventricular volume was significantly increased by 41% in those with VLBW. The ratio of grey to white matter was also significantly increased (by 10%) in those with VLBW. Group comparison maps showed widespread changes in the distribution of grey and white matter, and relative excess of ventricular CSF, in the brains of VLBW individuals. Increased ventricular volume predicted decreased grey matter in subcortical nuclei and limbic cortical structures, and decreased periventricular white matter. We conclude that these diffuse abnormalities of grey and white matter are a consequence of the interaction of perinatal brain injury and ongoing neurodevelopmental processes.

Attention-deficit–hyperactivity disorder (ADHD) has been associated with childhood epilepsy; prevalence figures have ranged from 8 to 77%, depending on the sample studied and the criteria used for diagnosis. In the general population the prevalence of ADHD is approximately 5%, with the majority of affected children having ADHD combined type. As part of a larger study of behavioral problems in children with epilepsy, we assessed 175 children (90 males, 85 females; age range 9 to 14 years, mean age was 11 years 10 months, SD 1 year 8 months) for evidence of ADHD. The children had at least a 6-month history of epilepsy. The primary caregiver completed both the Child Behavior Checklist (CBCL) and the Child Symptom Inventory-4 (CSI) or Adolescent Symptom Inventory-4 (ASI). On the CBCL, the mean attention problem T score was 64.6 (SD 10.5) for adolescents and 67.9 (SD 11.6) for children. On the CSI or ASI, 20 of 175 children met DSM-IV criteria for ADHD combined type; 42 of 175 had ADHD predominantly inattentive type; and 4 of 175 met criteria for ADHD predominantly hyperactive–impulsive type. There were significant correlations between the CBCL attention score and inattention (r=0.68) and hyperactivity–impulsivity (r=0.59). Sex, seizure type, and focus of seizure discharge were not predictors of symptoms of ADHD. Children with epilepsy are at risk for symptoms of ADHD. They differ from other samples of children with ADHD by the higher proportion of children with ADHD predominantly inattentive type and by an equal male:female ratio.

The purpose of this study was to explore O'Connor's four factors contributing to mothers' uncertainty concerning gastrostomy tube (G-tube) insertion in their children (lack of information; unclear value trade-offs; lack of support; social pressure) in a substitute decision-making context. Fifty mothers participated in one semi-structured interview at the time of their children's G-tube insertion. Children's ages ranged from 2 weeks to 17 years, slightly more than half were male, and most had a primary diagnosis related to a neurological (n=27) or cardiac (n=10) condition. Two-thirds of the mothers identified topics about which they wanted more information, the majority reported both gains and losses associated with their decision, three-quarters reported that they had received support during decision making, and half reported that they had felt pressure from family and health care professionals. Results indicate that mothers' decisions about G-tube insertion are complex and difficult. The existence and importance of O'Connor's factors in this context are confirmed by mothers' accounts. Because these factors are believed to be modifiable, health care professionals have the opportunity to potentially minimize the extent to which the factors contribute to decision uncertainly. It is recommended that health care professionals implement interventions focused on minimizing uncertainty.

In this issue of the journal, there are three papers which each contribute to the discussion of a major issue in autism today.

Badawi and colleagues (p 85) report on a positive association between newborn encephalopathy (NE) in term neonates and the risk of autism spectrum disorder (ASD). The study they conducted has several strengths worth mentioning that include the population-based nature of their sample, the systematic ascertainment of cases of NE in a well-defined population, the selection of a proper control series, and a comprehensive independent assessment of case status at follow-up.

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.

Pediatric feeding/swallowing specialists commonly observe infants and toddlers who present with gagging or significant behavior state changes as a result of touch to oral and body regions. To date, this behavior has not been fully characterized or documented experimentally. This paper describes an exploratory study aimed at filling in these lacunae and providing a possible explanation. The study compares the responses to firm tactile pressure of 40 children between the ages of 3 and 18 months. The experimental group consisted of 20 children (12 males, 8 females) with heterogeneous medical diagnoses, at least 2 weeks of alternative feedings during the first 3 months of life, and a common history of persistent feeding difficulties. The comparison group consisted of 20 children with an unremarkable medical history matched by age and sex to the experimental group. Firm, tactile pressure was applied in a predetermined sequence following dermatome regions from legs to mouth. Response was recorded in terms of presence/absence of gagging and/or behavior state changes. Fifteen of the 20 children in the experimental group showed gagging (14) or inconsolable crying (1) in response to touch. None of the matched comparison group demonstrated either gagging or behavior state changes. The experimental and comparison groups showed significant differences in response to tactile input. Further, the source of these abnormal responses must be sought in similarity of experience across heterogeneous medical diagnoses and interventions. One possible explanation is a history of oral deprivation due to alternative feedings.

Inflicted traumatic brain injury (ITBI) or shaken baby syndrome is recognized as a major cause of disability and death in the pediatric population. Although advances have been made in the recognition of the clinical, radiographic, and pathological findings of ITBI, less is known about the long-term outcomes of survivors. Health care providers recognize that these infants and children frequently have poor outcomes. Although an infant or child who sustains an ITBI may look well immediately after the trauma, that child may be left with serious and permanent disabilities.

A 5-year-old boy with focal cortical dysplasia was referred to our hospital because of epileptic seizures. He showed mild weakness of the left hand without sensory disturbance. Brain MRI revealed extensive cortical dysplasia with pachygyria and microgyria around the right central sulcus. On EEG examination, interictal spikes were noted over the right fronto/centro/parietal region. A 37-channel magnetometer revealed that the sources of the spikes were in a small, restricted region of the normal frontal lobe adjacent to the dysplastic brain. Somatosensory evoked magnetic fields indicated that the location of the current source of N20 was in the same area. Our patient shows a unique case of plasticity and reorganization of the somatosensory function due to cortical dysplasia.

In this discussion, children are considered to have multiple disabilities and low functioning when they have a combination of severe neurodevelopmental disabilities, such as visual impairment, epilepsy, intellectual deficits, cerebral palsy, attention-deficit–hyperactivity disorder (ADHD), autism, eating difficulties, and other neuropsychiatric problems.

Deprivation of sensory input affects neurological development. Our objective was to explore clinically the role of hearing in development of sensorimotor integration and non-verbal cognition. The study involved 54 children (15 males, 39 females; 5 to 9 years old) with severe or profound bilateral prelocutive deafness but without neurological or cognitive impairment. Of these, 25 had received an early cochlear implant (CIm). Patients were compared with 40 children with normal hearing. All were given a battery of non-verbal neuropsychological tests and a balance test, and were timed for simple and complex movement of limbs. Deafness, whether treated by CIm or not, resulted in a delay in development of complex motor sequences and balance. Lack of auditory input was also associated with lower, but non-pathological, scores in visual gnoso-praxic tasks and sustained attention. Such differences were not observed in children with CIm. Hearing contributes to clinical development of spatial integration, motor control, and attention. An early CIm enables good verbal development and might also improve non-verbal capacities.