The Children's Communication Checklist (CCC) was developed to provide an objective assessment of pragmatic aspects of children's communication difficulties. We aimed to (1) see whether the checklist provided valid and reliable information when completed by parents, and (2) consider its usefulness in a clinical context. Checklists were completed by parents and a professional who knew the child well for all 5 to 17-year-old referrals to a tertiary developmental paediatrics centre over a 31-month period. Children who were not yet speaking in sentences were excluded. From a sample of 151 children (81% male; mean age 8.7 years) with pervasive or specific developmental disorders, valid checklists were completed by 119 parents and 93 professionals. Reliability, as measured by internal consistency, was 0.7 or higher for most scales. Correlations between ratings for parents and professionals were in the range of 0.30 to 0.58 for individual pragmatic scales, with a correlation of 0.46 (n=82) for the pragmatic composite. For both parents and professionals, the pragmatic composite was lowest for children with a diagnosis of autism; intermediate for those with a diagnosis of Asperger syndrome, pervasive developmental disorder not otherwise specified or attention-deficit–hyperactivity disorder (ADHD); and highest for those with a diagnosis of specific learning disability. The strongest relation between the pragmatic composite and diagnosis was seen when ratings from parents and professionals were combined. Differences between diagnostic groups were not explicable in terms of age or verbal IQ.

The aim of this study was to evaluate the incidence of visual function abnormalities in children with infantile hemiplegia, and the relation between visual abnormalities and type of lesion, as shown by brain MRI. Visual function was tested (grating acuity, visual field size, binocular optokinetic nystagmus [OKN], and ocular movements) in a group of 47 children with congenital or early acquired hemiplegic cerebral palsy (mean age 25 months, range 8 to 52 months). The cohort was subdivided into four groups according to MRI findings: brain malformations (n=5), abnormalities of the periventricular white matter (n=20), cortical–subcortical lesions (n=16), and non-progressive postnatal brain injuries (n=6). More than 80% of the children showed abnormal results in at least one visual test: acuity was the least impaired function, while visual field and OKN were abnormal in more than 50% of the cohort. No specific correlation could be identified between the type and timing of the lesions and visual function. Unlike adults with stroke, visual field defects were not always related to contralateral damage in the optic radiations or in the visual cortex. These results indicate that visual abnormalities are common in children with hemiplegia, and that they cannot always be predicted by MRI. All children with hemiplegia need a detailed assessment of visual function.

The present study addressed the question whether the deviant postural adjustments in children with spastic diplegia can be attributed to their crouched sitting position or primarily to their neural deficit. Postural adjustments during sitting in an erect and in a crouched position on a movable platform were assessed in 10 children, aged 3 to 7 years 6 months, with mild-to-severe forms of spastic diplegia and 10 age- and sex-matched control children. Multiple surface EMGs of neck, trunk, and leg muscles and kinematics of head, body sway, and pelvis were recorded during forward and backward translations. The children with normal motor development showed a distinct adaptation of postural adjustments to sitting position. The children with cerebral palsy (CP) had a deficient adaptational capacity which was more pronounced in the erect than in the crouched position. Thus, the crouched sitting position did not induce postural deficiency but seemed to offer a solution to the sensory–motor problem of the instability experienced. Children with severe diplegia exhibited a lack of direction specificity in the leg muscles during backward body sway, which points to a basic deficit in postural control. In addition, these children showed marked dysfunctions in the precise tuning of the postural adjustments to task-specific conditions. In the children with mild-to-moderate forms of CP the basic level of control was intact.

The early development of self-injurious behaviour in three young boys (aged 17, 25, and 30 months at start of study) with Lesch-Nyhan syndrome was examined by means of parental interviews and by direct observations completed at 3 to 4 monthly intervals over an 18-month period. Results suggest that the self-injury began in a different way from that of other young children with autism and/or developmental disabilities in that, from the start, self-injurious responses were sudden and violent, rather than emerging gradually over time. Drastic measures, such as removal of the teeth or provision of tooth guards, were often taken to prevent further tissue damage. Direct observations showed that the boys' self-injury occurred at lower rates, but their carers were highly concerned about the behaviour. Sequential analysis of the observational data indicated that on some occasions the children were more likely to self-injure during periods of low social interaction, suggesting that their self-injury may have been influenced by environmental factors. The theoretical and practical implications of these findings are discussed.

Autism is a behaviourally defined syndrome characterized by disturbances of social interaction and communication and restrictions of behaviour patterns and imagination. The pathogenesis of autism is unknown but it is suspected that a number of genetic factors may be involved. Neurotrophic factors such as insulin-like growth factor-I (IGF-I) play a role in early brain development. The aim of this study was to determine whether IGF-I levels might be associated with the development of autism. IGF-I levels were measured in the CSF of 11 children with autism (4 females, 7 males; mean age 3.8 years, SD 1.1) using a sensitive radioimmunoassay method and compared with levels in 11 control participants (6 females, 5 males; mean age 3.8 years). Levels of IGF-I in the CSF were statistically significantly lower in the children with autism than in the control children (p=0.03). IGF-I may play a role in pathogenetic mechanisms of autism and the role of neurotrophic factors in autism and other neurodevelopmental diseases should be studied further.

In order to identify the prevalence and severity of feeding problems in children with Silver-Russell syndrome(SRS) the feeding difficulties of 32 children with SRS and 32 age- and sex-matched control children were assessed using the Feeding Assessment Questionnaire (Harris and Booth 1992). Children's ages ranged from 2 to 11 years, with 19 male and 13 female pairs. In a subset of matched pairs, parent–child interaction at mealtimes was examined using video recording; food intake was also assessed over a 3-day period using weighed food diaries. Children with SRS experienced significantly more feeding problems than children without growth disorders. Common feeding problems for children with SRS were poor appetite, fussiness, slow feeding, and problems associated with oral-motor dysfunction. Feeding problems did not significantly affect intake of kilocalories, protein, fat, or carbohydrates. Mealtime interaction between children with SRS and their parents was significantly more negative than interactions between control-group children and their parents. The focus of intervention packages in SRS should be to reduce negative parent–child interactions and reduce parental anxiety about feeding, growth, and weight.

Strength, power, and coactivation levels were measured during knee extension and flexion tasks in 20 children (13 males, 7 females) with developmental coordination disorder (DCD) and 20 age-matched control children (12 males, 8 females). Participants were classified according to results obtained on the McCarron Assessment of Neuromuscular Development, with each group including two age levels: 6 to 7 and 9 to 10 years. Tasks were performed on a Biodex dynamometer under isometric and isokinetic conditions with surface EMG recorded from the vastus lateralis and biceps femoris muscles. Significant effects of group and age were observed for the measures of peak torque (isometric and isokinetic) and power, with the decreased power in the group with DCD being more apparent at higher velocities. A lower flexor–extensor percentage was recorded for the group with DCD but only under isokinetic conditions. From a neuromuscular perspective, the group with DCD had increased levels of coactivation (RMS agonist:RMS antagonist) during the isometric flexion and isokinetic extension actions. Possible causes of the strength and power deficits observed in children with DCD are discussed.

This study compared adolescents with cerebral palsy (CP) and their families to adolescents without physical disabilities and their families as the child enters and leaves adolescence (age ranges 13 to 15 years and 19 to 23 years). Families of 90 individuals with CP (42 females, 48 males) and 75 individuals without physical disabilities (34 females, 41 males) participated. They completed the Family Assessment Device, Life Situation Survey, Multidimensional Scale of Perceived Social Support, and Future Questionnaire. There were few differences in family functioning, life satisfaction, or perceived social support between the groups. Expectations of young adults with CP and parents of both adolescents and young adults regarding future independence and success were lower than the expectations of the control group. While the group results emphasize similarities between families during the two stages of adolescence, individual families and individual family members report specific challenges.

In order to classify attention-deficit–hyperactivity disorder (ADHD) in 11-year-old children, the role of specific attentional and motor deficits was examined. Participants comprised 22 children with ADHD (19 male, 3 female; median age 11 years, range 8.8 to 13.5 years) and 20 control children (17 male, 3 female; median age 10.6 years, range 8.2 to 12.6 years). Neuromotor assessment indicated that while both groups needed more time to complete finger compared to hand movements, this increase was more pronounced in children with ADHD. Reaction-time testing with continuous-force recording identified both motor and attentional deficits in children with ADHD. Longer intervals between force onset and force peak, and higher rate of responses with multiple force peaks (particularly in the bilateral condition) revealed specific deficits in the speed and quality of their motor output. Increase in errors and variability of force onsets indicated attentional deficits. Prediction analysis indicated that force-onset variability contributed significantly to group classification which was 85.7% correct. Neither neuromotor assessment nor specific motor deficits contributed significantly to classification, indicating that pure motor-speed measures play a minor role in characterizing ADHD in this age range.

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.

The study aimed to investigate the development of bladder control in children with cerebral palsy (CP) and to determinate subgroups with deviant development of bladder control and a higher risk of not achieving urinary continence. Children and adolescents between the ages of 4 and 18 years with a diagnosis of CP, from six Dutch rehabilitation centres were included in the study (n=601). Parents of these participants were sent a questionnaire, of whom 76% responded. Prevalence of primary urinary incontinence was 23.5%. Survival curves were made to determine the development of achieving bladder control in CP. For participants who achieved urinary continence, the development was delayed in comparison with normally developing children and adolescents. The most important factors influencing the occurrence of urinary incontinence in CP were tetraplegia and low intellectual capacity. At age six, 54% of participants with spastic tetraplegia and 80% with spastic hemiplegia or diplegia gained urinary continence spontaneously. Of those who had low intellectual capacity, 38% were dry at this age.

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 aim of this preliminary study was to examine the developing cognitive profiles of children with cerebellar tumours in a consecutive series of clinical patients. MRI and longitudinal intellectual profiles were obtained on seven children (two females, five males; mean age 3 years at diagnosis; mean age 7 years at first assessment). Tumours in three of the children were astrocytomas; of the remaining tumours, two were medulloblastomas, one low-grade glioma, and one ependymoma. In right-handed children, we observed an association between greater damage to right cerebellar structures and a plateauing in verbal and/or literacy skills. In contrast, greater damage to left cerebellar structures was associated with delayed or impaired non-verbal/spatial skills. Long-term cognitive development of the children studied tentatively supports a role for the cerebellum in learning/development. These findings suggest that lateralized cerebellar damage may selectively impair the development of cognitive functions subserved by the contralateral cerebral hemisphere and, in addition, that all children with cerebellar lesions in early childhood should routinely undergo long-term monitoring of their intellectual development.

This study investigates the presence of sensory modulation dysfunction (SMD) among children with attention-deficit-hyperactivity disorder (ADHD). Twenty-six children with ADHD (mean age 8.3 years, 18 males, 8 females), and 30 typically developing children (mean age 8.2 years, 21 males, 9 females) were tested using a laboratory procedure that gauges responses to repeated sensory stimulation by measuring electrodermal reactivity (EDR). Parental report measures of limitations in sensory, emotional, and attentional dimensions were administered using the Short Sensory Profile, the Leiter International Performance Scale–Revised, Parent Rating subscales, and the Child Behavior Checklist (CBCL). Compared to the typical sample, the children with ADHD displayed greater abnormalities in sensory modulation on both physiological and parent-report measures. The children with ADHD also displayed more variability in responses. Within the group with ADHD, levels of SMD were highly correlated with measures of psychopathology on the CBCL. Implications of findings relate to the importance of considering sensory processing abilities in a subgroup of children with ADHD.

Little is known about chronic posttraumatic headache (PTH) in children and adolescents. In this study we report on symptoms, clinical findings, and management of all children seen in our headache clinic who presented with recurrent headache following head injuries. A total of 23 children were identified over a period of 4 years. Twenty-one children (13 male, eight female; mean age 11.2 years, age range 3.3 to 14.9 years, median 12.1 years) fulfilled the International Headache Society's clinical criteria for the diagnosis of chronic PTH. Mean duration of headache was 13.3 months (range 2 to 60 months, median 7 months). Children were followed up for a period of 5 to 29 months (mean 12.5 months, median 9 months). Head injuries were classified as serious in four patients and minor in 17. Clinical variables were compared between children with PTH and those without a history of head injury. The headaches were migraine in five patients, tension type in 13, and mixed in three patients. Tension-type headache was significantly more common in children with chronic PTH than in those with no history of head injury, but the clinical course was comparable in the two groups.

The International Headache Society (IHS) classification system (1988) was developed primarily for headache disorders in adults and its validity for paediatric age is currently under discussion; in 1995 Seshia et al. proposed a revision of the criteria for migraine without aura to make diagnostic criteria more applicable to children. The purposes of the current study were to: (1) compare the IHS classification with the Seshia proposal, (2) compare the children affected by migraine without aura (MO) with the children affected by tension headache (TH) as defined by Seshia, for characteristics which are not included in the classification. The patients are a series of 320 children (mean age 9.9, SD 2.6 years; 144 males, 176 females) with recurrent or chronic headaches referred to a headache clinic in Milan, Italy. Using the Seshia criteria instead of the IHS criteria a higher number of children were included in the MO category: bilateral pain and family history of migraine were the most important factors which allowed a shift of children into this category. However, with the Seshia classification there was no reduction in the number of unclassifiable children. The reason why some children could not be classified was a short duration of attacks; the majority of unclassifiable children were 6 years old or less. No relevant difference was found between children with MO and children with TH for the following variables: occurrence of attacks in the afternoon or evening after school, reduction of attacks during school holidays, full-time schooling, after-school activities on school days, disordered daily life. On the contrary children with MO when compared with those with TH showed a higher number of precipitating factors and for the following factors a significant difference was found: exposure to TV or a computer, sleep deficiency, and strong emotions. Furthermore, children with MO showed a greater severity of attacks.

This study aimed to assess the visuospatial abilities of five children with Williams syndrome (four males aged 9 years 3 months, 7 years 11 months, 8 years 1 month, and 10 years 8 months respectively, and one female aged 6 years 3 months). First, the children's visuospatial abilities were examined by asking them to copy a figure. Second, their cognitive processing abilities were assessed using the Japanese Kaufman Assessment Battery for Children. This test was used because it is an objective one, standardized in Japan, and is a measure of fluid ability including spatial localization. Participants scored significantly low on the spatial memory subtest indicating that there was a deficit in spatial localization. Children's performance in line copying tasks improved when the dots were in colour. Results suggest a deficit in the dorsal stream of visual cognition, with a relatively preserved ventral stream.

Bladder involvement is common in transverse myelitis but its characteristics and natural history, particularly in relation to neurological outcome, have not been described in a paediatric population previously. Ten children, with ages ranging from 8 months to 16 years, who had a diagnosis of acute transverse myelitis were studied with video urodynamics and followed up in a tertiary paediatric neurourology clinic. Nine of ten children had obstructive urinary tract symptoms at presentation and all went on to develop ‘irritative’ urinary tract symptoms (frequency and urgency) about 1 month after initial presentation. Video urodynamics showed a combination of irritative (detrusor hyperreflexia) and obstructive (detrusor–sphincter dyssynergia) abnormalities in most patients and enabled management to be specifically directed towards these. The patients progress was followed up for a median duration of 36 months. All had residual bladder dysfunction, only four were asymptomatic on treatment. The degree of recovery of bladder function was not related to the degree of motor recovery.

A study of a clinic-based sample of 25 individuals (12 females, 13 males; age at diagnosis 14.4 years, SD 7.4 years; age range 4 to 33 years) with Down syndrome (DS) and autism spectrum disorders, demonstrates that autism is by no means rare in DS. Results showed that there was a considerable delay in the diagnosis of autism as compared with children with autism who did not have DS. In 11 participants medical factors were identified that were likely to be of importance in contributing to the development of autism, and in four further participants there were factors of possible significance. Such factors include a history of autism or autism-related disorders in first- or second-degree relatives (n=5), infantile spasms (n=5), early hypothyroidism (n=3), evidence of brain injury after complicated heart surgery (n=2), or a combination of these factors. It is important that autism is recognised, identified, and fully assessed in individuals with DS in order for them to receive appropriate education and support.

Neurological lesions that cause dyskinetic cerebral palsy (CP) commonly involve ocular movements. This report describes a group of 14 children (nine males, five females) whose CP is associated with severe dyskinetic eye movements. Ages ranged from 4 months to 13 years (mean 6.9 years). Clinical features of this eye movement disorder are discussed and defined. The visual function of these children is slow, variable, and highly inefficient. They are often misdiagnosed as blind, due to cortical visual impairment. Early recognition of dyskinetic eye moment disorder and appropriate developmental and educational management are important.