Considerable attention has been given in recent years to fingertip force coordination during grasping and lifting small objects in children with cerebral palsy (CP). However, little is known about the children's ability to replace and release an object from grasp. The present study examined the coordination of fingertip forces during replacement and release of an object from grasp under varying task constraints in the involved hand of 15 children (10 males, five females, age range 7 to 14 years) with hemiplegic CP and in the non-dominant hand of 15 age-matched, typically-developing children (seven males, eight females). Participants released an object, instrumented with force transducers and held with a precision grip, onto a stable surface and onto an unstable surface (requiring higher accuracy) at self-paced and fast-as-possible speeds. Temporal and force measures were recorded and the dependent measures were tested using analyses of variance. Results showed that force coordination was impaired in children with hemiplegia, resulting in prolonged and uncoordinated replacement and release of the object (p<0.05). Differences between controls and children with hemiplegia were greater when speed and accuracy constraints were imposed (i.e. task performance was affected by these constraints to a greater extent in the children with CP, p<0.05). Impairments in temporal coordination of object release were also observed in the non-involved hand under all conditions (p<0.05). These results provide additional information about impaired hand function in children with hemiplegic CP. Clinical implications of these findings are discussed.

The ability to move two fingers at the same time was measured in young and older adults. This study measured the accuracy with which 45 normally developing children (23 boys, 22 girls; mean ages 8.4 and 8.3 years respectively) and 49 young adults (25 males, 24 females; mean ages 19.8 and 20.5 years respectively) could synchronize movements of two fingers when making or breaking contact with circular metal discs. A portable electronic instrument displayed the timings. To measure ‘in-phase’ skills, attempts were made to contact both discs simultaneously with the two index fingers or to break both contacts; asynchrony was only a few milliseconds and differences between children and adults were insignificant. To measure ‘antiphase’ skills, attempts were made with the index and middle fingers to make contact simultaneously with one disc while breaking contact with the other; asynchrony was larger. Usually one contact was made and later the other was broken; for a while both fingers touched (overlap). Rarely one contact was broken before the other was made; for a while neither finger touched (gap). Boys' periods of overlap were longer than those of men and women; on both sides these differences were highly significant statistically (p<0.0001). Boys' errors on the right side were also significantly greater than those of girls. On both sides, mean errors of girls were higher than those of men and women: three of the four sets of data reached statistical significance. While boys' performance improved with age, that of girls was static. Children achieved an essentially adult level of control for in-phase skills but with antiphase skills the children, and especially the boys, had clearly poorer performances.

This study examined the relationship between lead exposure and attention regulation in children from low-income families. Children with lead exposure were expected to have lower attention regulation abilities than children who were not exposed. The 57 participants (39% male, 61% female) were children aged 4 and 5 years (mean age 4y 7mo [SD5mo]); 24 children were lead-exposed (10–29µg/dl) and 33 were non-exposed comparisons (1–6µg/dl). Both groups qualified to be enrolled in programs for low-income families. A puzzle-matching task was completed in parent–child and child-alone conditions. Attention allocation and puzzle-matching performance were assessed in the two conditions. Children who were lead-exposed demonstrated less self-regulated attention than comparisons and had poorer puzzle performance in the child-alone condition. Attention patterns did not differ in the parent–child condition. This study extends previous knowledge concerning effects of lead exposure on preschool children from disadvantaged environments and suggests that lead exposure affects specific attention regulation abilities.

We investigated a male, aged 4 years 11 months, who fulfilled the criteria of Asperger syndrome). In addition to the typical pattern of autistic symptoms, psychological testing revealed prosopagnosia in tasks for face recognition and matching. Prosopagnosia was also present when he tried to identify the faces of his parents and himself in photographs whenever these were presented with photographs of other persons. Although impairment in reciprocal social interaction in individuals with Asperger syndrome is closely correlated to their impaired perceptional abilities in non-verbal communication, especially facial expression, overt prosopagnosia seems to be a rare neuropsychological symptom in persons with autistic disorders.

Cerebral arteriovenous malformations (AVMs) are fairly common and the majority of paediatric patients with this condition also present with intracranial haemorrhage. Two patients who had an incidental finding of an AVM associated with papilloedema are described here. The first was a 13-year-old male who presented after an accidental kick to the eyes. Examination revealed bilateral papilloedema. He gave a 2-year history of intermittent headache. Brain magnetic resonance imaging (MRI) showed an unruptured AVM in the temporal lobe. Lumbar puncture revealed elevated cerebrospinal fluid pressure. Visual acuity and visual fields were normal. He was treated with acetazolamide and improved within a few weeks. He subsequently underwent stereotactic radiosurgery to the AVM. He discontinued acetazolamide due to adverse side effects and there was no recurrence of headache and papilloedema. The second patient was a 14-year-old male who had polyarticular juvenile chronic arthritis and received low-dose steroids and methotrexate. Bilateral papilloedema was discovered during routine ophthalmology surveillance and he was otherwise asymptomatic neurologically. Brain MRI revealed an AVM in the posterior fossa. He had three embolization procedures, which have resulted in significant reduction in lesion size. The papilloedema resolved completely after the first two procedures, and visual acuity and fields remained normal. Here, possible underlying mechanism of raised intracranial pressure and importance of visual assessment in those with AVMs and their management are discussed.

In this study, 10 sagittal lower limb range-of-motion measures were conducted in a blinded fashion in 25 children with spastic diplegic cerebral palsy and in 25 age- and sex-matched controls. The participants comprised 22 males, mean age 10 years 8 months and 28 females, with mean age 9 years 8 months; age range 6 to 17 years. One paediatric physical therapist performed duplicate goniometric measures at zero time and 7 days later using the same sequence of measures, location, and time of day. Mean absolute differences for measures within one session ranged from 0.7 to 2.9° in controls and from 1 to 4.2° in children with spastic diplegia. Most intraclass correlation coefficients (ICCs) for intra-sessional measures were more than 0.90 in both groups. Measures between sessions were less reliable. Mean absolute differences between sessions were up to 7.1° for children with spastic diplegia and 8.6° for controls, with most ICCs being less than 0.80. Inter-sessional variation in measures was similar in both groups, suggesting that measurement variability is not influenced by the presence of spasticity. Averaging of two measures did not improve inter-sessional reliability compared with the use of a single measure. Dynamic measures (R1) were as reliable as passive measures (R2), but there were inter-sessional differences in calculations using R1 and R2 measures of up to 30°.

Historically, observation of infant behaviour has been fundamental to the understanding of developmental neurology and ontogenetic adaptation. Darwin's account of his infant son's development is an evocative example. More recently, assessment of the quality of spontaneous general movements (GM) in the fetus and young infant has advanced insight into the development and integrity of the nervous system. How can these observations contribute to the diagnosis of neurodisability? Does this model have useful clinical applications?

We evaluated gross motor function following botulinum toxin A (BTX-A) injections in the lower limbs of children with spastic cerebral palsy in a randomized clinical trial, using a cross-over design. Forty-nine children (24 males, 25 females, age range 22 to 80 months) were randomly allocated to two groups: group 1 received BTX-A and physiotherapy, and group 2 received physiotherapy alone for 6 months. At the end of this period, group 2 received BTX-A and physiotherapy and group 1 continued with physiotherapy alone. Assessment measures were the Gross Motor Function Measure (GMFM), the Vulpe Assessment Battery (VAB), joint range of movement, the Modified Ashworth Scale, and a parental questionnaire. Sustained gains in gross motor function were found in both groups of children but the only additional benefit found in group 1 was a significant increase in fine motor rating on the VAB. By contrast, parents rated the benefit of treatment highly. It is likely that assessment at 3 and 6 months post injection was too late to demonstrate peak gross motor function response and that changes in GMFM are not sustained over 6 months with a single dose. Further studies should investigate changes over shorter time periods and consider covariables such as BTX-A dosage, number of injection sites, and the role of repeated injections combined with other interventions such as casting.

In this preliminary study, visual search for targets within and beyond the initial field of view was investigated in seven school-aged children (five females, two males; mean age at testing 8 years 10 months, SD 1 year 3 months; range 6 to 10 years) with various acquired, postnatal, focal brain injuries (haematoma, haemorrhage, meningioma, neuroblastoma, and cerebral abscess) in anterior or posterior sites of the left or right hemisphere, and seven control children (matched for age and sex) were also studied. All participants attended mainstream primary schools. The children with lesions underwent surgery after diagnosis (mean age at diagnosis 5 years 4 months, SD 2 years 7 months). Group results indicated that for the overall scores on three psychometric tests of visuospatial and fine motor abilities (Southern California Figure Ground Perception Test, Visual Organization Test, and Visual–Motor Integration Test), no difference between the children with left and right lesions was present. However, children with lesions in the right hemisphere, and not in the left hemisphere, took significantly more time than the controls to locate visual targets presented within and beyond the field of view. Examination of individual data suggested that, in accordance with brain imaging research, right-sided anterior cerebral lesions sustained in early childhood might have an enduring detrimental effect on voluntary visual search performance during development. This persistent effect of early brain injury might imply that developmental plasticity of the brain does not apply to certain specific functions of particular areas of the right hemisphere.

It has been hypothesized that autism results from an ‘opioid peptide excess’. The aims of this study were to (1) confirm the presence of opioid peptides in the urine of children with autism and (2) determine whether dipeptidyl peptidase IV (DPPIV/CD26) is defective in children with autism. Opioid peptides were not detected in either the urine of children with autism (10 children; nine males, one female; age range 2 years 6 months to 10 years 1 month) or their siblings (10 children; seven males, three females; age range 2 years 3 months to 12 years 7 months) using liquid chromatography-ultraviolet-mass spectrometric analysis (LC-UV-MS). Plasma from 11 normally developing adults (25 years 5 months to 55 years 5 months) was also tested. The amount and activity of DPPIV in the plasma were quantified by an ELISA and DPPIV enzyme assay respectively; DPPIV was not found to be defective. The percentage of mononuclear cells expressing DPPIV (as CD26) was determined by flow cytometry. Children with autism had a significantly lower percentage of cells expressing CD3 and CD26, suggesting that they had lower T-cell numbers than their siblings. In conclusion, this study failed to replicate the findings of others and questions the validity of the opioid peptide excess theory for the cause of autism.

determining clinically whether or not a child with abnormal movements is having seizures is rarely easy. several non-epileptic causes of apparent seizures are well described, especially gastro-oesophageal reflux. stool withholding is very common in childhood, and can start very soon after birth. although it can cause major issues for the child and family, it is rarely considered by health care professionals to be more than a nuisance. a major feature of this is pain related to defaecation. further, in order to enhance withholding and thus prevent defaecation, a child may adopt positions and facial features that can mimic seizures. this paper presents four children (two males, two females) who presented, between the ages of 3 and 13 months, over a 2-year period to a general paediatric clinic (held in hospitals in watford and hemel hempstead, hertfordshire, uk). all received multiple investigations before accurate diagnosis. this highlights the fact that stool withholding should be added to the differential diagnosis of seizures in infancy.

Over a 33-year period, 260 patients (<17 years of age; 119 males, 141 females) from New South Wales, Australia who had peripheral neuropathies confirmed by nerve biopsy, were studied. Of these, 50 infants presented with symptoms or signs of neuropathy under 1 year of age: including 24 patients with demyelinating neuropathies and 21 axonal neuropathies; a further five patients had spinal muscular atrophy with associated secondary sensory axonopathy. Nineteen infants had hereditary motor sensory neuropathy, of whom 13 had myelin protein mutations confirmed by molecular genetic studies. Peripheral neuropathy is not an unusual diagnosis in infancy. Awareness of this association will aid early diagnosis and prognosis as well as facilitate interventional patient management.

The aim of the study was to determine whether learning and memory are compromised in school children with recently diagnosed idiopathic and/or cryptogenic epilepsy and to study relationships between learning and memory and psychosocial and epilepsy variables. Word span and learning of locations were assessed within 48 hours after diagnosis of epilepsy and three and 12 months later, in 69 school children with epilepsy (aged 9.1 years, SD 2.7; 33 males, 36 females) and 66 classmates. Results showed that patients and controls performed similarly in registration, recall, and retention. Patients recalled slightly less than controls when probed under conditions of increased demand on working memory. Maladaptive reactions of parents and children to the onset of epilepsy and not reaching 6-months of seizure remission contributed to poor performance. Individually, those patients who required special assistance at school, under-performed occasionally in one or the other component of memory. Although the proportion of under-performers was stable over time, the children composing the group did change. It was concluded that school children with new onset idiopathic or cryptogenic epilepsy are inordinately vulnerable when processing memory tasks. The vulnerability is neither persistent nor memory-specific.

The objective of this study was to develop a method to use digital signal processing (DSP) technology to describe quantitatively and statistically swallow-associated sounds in preterm infants and to use this method to analyze changes as infants mature. Twelve recordings of accelerometric and physiological data on bottle-feeding preterm infants between 32 and 39 weeks' postmenstrual age (PMA) were analyzed. Cervical auscultation was performed using an accelerometer attached over the larynx. Acoustic data were recorded and graphically displayed using DSP software. Initial discrete sounds (IDSs) were identified and used to construct an average waveform from which a ‘variance index’ (VI) was calculated for each infant. The shape of the IDS waveforms became progressively more uniform with advancing PMA, as indicated by a significant inverse correlation between VI and PMA (r=0.739; p=0.006). DSP technology facilitated the development of a new method to quantitatively analyze feeding in preterm infants. This method provides an elegant tool to track maturation of infant feeding and assessing feeding readiness. This technique makes the interpretation of cervical auscultation data less subjective by replacing the verbal description of the sounds of feeding with quantitative numeric values. It is anticipated that this method can be automated to facilitate further the analysis of cervical accelerometry data.

A white Italian boy, aged 5 years and 8 months, is reported with failure to thrive, hypotonia, truncal ataxia, psychomotor retardation, and congenital horizontal pendular nystagmus with only waves I and II on auditory brainstem responses. Our patient's clinical picture resembles that previously reported in 10 male Oriental patients. He did not manifest spastic diplegia by the age of 2 years, as did the subjects reported in the literature, but knee-jerk hyperreflexia was evident at the most recent clinical reevaluation. Serial brain MRI studies revealed a cystic brain lesion and peritrigonal hyperintensities with no brainstem abnormalities. To date, no other child with a similar syndrome has been described either in Europe or in America. The clinical features of this condition are consistent and characteristic. A definitive diagnosis is achieved by demonstrating the absence of all waves following wave I or wave II on auditory brainstem responses as early as 3 months of age. Due to the predominance of males, the occurrence in siblings, the early age at onset, the non-progressive course, and the characteristic auditory brainstem response findings, the syndrome may have a genetic origin and be attributable to a dysgenetic brainstem lesion.

Twenty-nine patients (16 males, 13 females) with Joubert syndrome were identified from ophthalmology, neurology, and genetic databases covering a 15-year period at Great Ormond Street Hospital, London. Criteria for diagnosis included absent or markedly hypoplastic cerebellar vermis, abnormal eye movements, and developmental delay. Five patients had died. Scans and notes were available for 22 patients, and 18 cases were clinically reviewed. The median age was 10 years 10 months (range 3mo to 19y) and the median follow-up was 8 years 5 months (range 3mo to 19y, with one new patient seen at 3mo of age). Cerebellar vermis hypoplasia/aplasia with ‘molar tooth sign’ in the axial plane was present in 22 of 22 patients, coloboma in 6 of 22, and polydactyly in 6 of 22. In the 18 clinically reviewed, apnoea occurred in 13 patients. Five had renal problems with cysts and 4 of 5 had abnormal electroretinograms (ERGs). Visual electrophysiology was abnormal in 14 of 18 patients, and in 6 there was evidence of deterioration in the ERG. Blood investigations of organic acids, phytanic acid, very-long-chain fatty acid, and transferrin were normal in 12 patients tested. Developmental assessment showed that 6 of 15 patients aged more than 5 years were at mainstream school, and 12 of 18 had started walking between 22 months and 10 years. Speech difficulties and behavioural problems were prominent.

The Ely Test (or Duncan-Ely test) has been accepted as a clinical tool to assess rectus femoris spasticity by passively flexing the knee rapidly while the patient lies prone in a relaxed state. In this retrospective review, patients' dynamic knee range of motion (ROM) during gait and an electromyogram (EMG) were compared with the results of the Ely test. Data for 70 patients (44 males, 26 females; 104 limbs) were included. Mean age of patients was 13 years, SD 9 years, range 4 years 5 months to 54 years. All patients were diagnosed with cerebral palsy (spastic diplegia, n=42; spastic quadriplegia, n=15, and hemiplegia, n=13). All patients were ambulatory (50 independent, 20 with assistive devices). A standard matrix was used to calculate sensitivity and specificity of the Ely test as well as its positive and negative predictive value. For the gait variables examined (decreased dynamic knee ROM, timing of peak knee flexion, and abnormal EMG in swing) the sensitivity of the Ely test ranged from 56 to 59% and the specificity ranged from 64 to 85%. For the same variables the positive predictive value ranged from 91 to 98% and the negative predictive value ranged from 4 to 19%. The Ely test was shown to have a good positive predictive value (i.e. the certainty about the presence of rectus spasticity in patients with a positive Ely test result) for rectus femoris dysfunction during gait.