Upper limb impairment can affect the ability to perform and participate in activities of daily living. The level of activity and participation limitation in the home environment for children with hemiplegic cerebral palsy (CP) is poorly understood. A greater understanding of these limitations could be used to provide targeted and appropriate intervention programmes. Level of activity and participation limitation were investigated, with the use of the Assessment of Motor and Process Skills (AMPS) in a representative sample of 54 South Australian children (31 males, 23 females) with hemiplegic CP, aged 3 to 12 years (mean age 7y 4mo [SD 2y 5mo]). Two AMPS tasks that were familiar to the child were performed in the home under the supervision of a trained occupational therapist. Findings suggested that younger children (3–8y) performed significantly better than older children (9–12y) for motor skill ability (0.46 vs 0.09, p=0.041) but not for process skill ability (–0.25 vs –0.28, p=0.885). Functional performance for the sample was below that of age-matched normative data. Motor performance in activities of daily living as detected by the AMPS seemed to worsen with age in children with hemiplegic CP. Further investigation into what can influence this outcome is required.

preschool children diagnosed with either global developmental delay (gdd) or developmental language impairment (dli) were reassessed during their early school years with standardized developmental (battelle developmental inventory [bdi]) and functional (vineland adaptive behavior scale [vabs]) outcome measures. of an original cohort of 99 children with gdd and 70 children with dli assessed and diagnosed at a mean age of 3 years 5 months (sd 1.1) and 3 years 7 months (sd 0.7) respectively, 48 children (34 [71%] males) with gdd and 43 children (36 [84%] males) with dli were reassessed at a mean age of 7 years 4 months (sd 0.9) and 7 years 5 months (sd 0.7) respectively. the overall total mean bdi score for children with gdd was 66.4 (sd 4.3) versus 71.9 (sd 8.2) for children with dli (p=0.002). on each subdomain of the bdi, except communication, mean scores for the gdd group were significantly lower than for the dli group (p<0.05). similarly, the vabs total score for the gdd group was significantly lower than for the dli group (p<0.001). for each subdomain of the vabs, the gdd group scored significantly lower than the dli group (p<0.001). the proportion of children falling below meaningful cut-offs on the outcome measures selected was significantly higher for those initially diagnosed with gdd. preschool diagnosis of either gdd or dli has later prognostic validity with regard to persisting developmental and functional deficits.

This study investigated whether in preterm children who had ventricular dilatation (VD) on neonatal cranial ultrasound outcome at age 8 years was influenced by the additional presence of germinal matrix haemorrhage – intraventricular haemorrhage (GMH–IVH). Six-hundred and ninety-nine preterm infants (<33wks' gestation, mean 29.6wks [SD 2.1]) with either normal cranial ultrasound (n=616; 286 females, 330 males), or with VD with (n=66; 32 females, 34 males) or without (n=17; 4 females, 13 males) GMH–IVH were enrolled in the study. At age 8 years outcome was assessed in 567 (81%) of the 699 children by neurological examination, the Test of Motor Impairment (TOMI), the test of Visuo-Motor Integration (VMI), and the Wechsler Intelligence Scales for Children. Results showed that the proportion of children with disabling impairments was higher in the group with VD and GMH–IVH. Performance on TOMI and VMI (even in those without disabling impairments) was poorer in those with VD and GMH–IVH than in children with normal scans or those with VD only. Children with VD and GMH–IVH had significantly lower performance IQ than children with normal ultrasound, whereas those with VD only were not different from those with normal scans. Results suggest the presence of subtle white matter injury that has not been identified by neonatal cranial ultrasound. Although this study did not investigate biochemical markers of haemorrhage, we hypothesize that non-protein-bound iron is likely to be a contributing factor to white matter damage in preterm infants.

The aim of this study was to provide a comprehensive profile of the sensory, motor, language, and intellectual functioning of a non-referred community sample of 49 preschool children with attention-deficit–hyperactivity disorder (ADHD; 39 males, 10 females; mean age 4y 7mo [SD 7mo]; range 3y 10mo–6y) and 48 typically developing children (38 males, 10 females; mean age 4y 8mo [SD 6mo]; range 3y 11mo–6y) matched by age, sex, and maternal education who underwent a broad battery of neurodevelopmental tests. The results showed that the scores of the ADHD group were significantly lower than the comparison group on all measures. In addition, 23 (47%) of the children with ADHD had clinically significant co-occurring deficits in two or more areas. Logistic regression indicated that the only significant predictors of group classification were scores of verbal intelligence and motor and sensory functioning, accounting for 44.1% of the variance. These findings suggest that preschool children with ADHD have multiple developmental deficits over and above the core symptoms of ADHD and emphasize the importance of evaluating the sensorimotor functioning of preschool children with ADHD symptoms.

The aim of this study was to propose a classification system for childhood arterial ischaemic stroke (AIS). Subtypes from the Trial of Org 10172 in Acute Stroke Therapy (TOAST) classification, previously shown to be applicable to children, were retained in the proposed Paediatric Stroke Classification (PSC). Additional important paediatric AIS aetiologies were identified from a literature review. Preliminary validation was performed by three raters who categorized clinical vignettes from 135 patients (66 male; median age 6.3y, range 0.1 to 16y). Eight aetiological subtypes were identified and defined, as follows: (1) sickle cell disease; (2) cardioembolic; (3) moyamoya syndrome; (4) cervical arterial dissection; (5) steno-occlusive cerebral arteriopathy; (6) other determined aetiology; (7) multiple probable/possible aetiologies; and (8) undetermined aetiology. There was very good agreement between the raters about categorization of the vignettes. Causes of disagreement were identified and final categories and definitions were modified accordingly. We conclude that the PSC enables the categorization of children with AIS into aetiological subtypes relevant to this age group. This will be useful in multicentre studies of natural history and treatment but will require further independent validation.

A prospective study was designed to determine whether the combined use of neuromuscular electrical stimulation (NMES) and dynamic bracing was more effective than use of either alone in reducing upper-extremity spasticity in children with spastic hemiplegic cerebral palsy. Twenty-four patients (12 males, 12 females; mean age 8y 7mo [SD 4y 2mo]; age range 3–18y) diagnosed with spastic hemiplegic CP were randomly allocated to three groups: group 1 had two 30-minute sessions of NMES a day applied on the antagonist extensors without bracing; group 2 had two 30-minute sessions of dynamic bracing per day; and group 3 had two 30-minute sessions of NMES and dynamic bracing every day. Treatment was continued for 6 months in all groups and applied only to the affected extremity. Patients were evaluated before therapy, at monthly intervals during the therapy, and 3 months after completion of the therapy. Three measures of outcome were taken: the Melbourne Assessment, grip strength, and posture evaluation with Zancolli's classification. The therapist performing the outcome assessments was blinded as to groups. Statistically significant differences were found in all three measures for only those treated with combined NMES and dynamic bracing. However, this significant effect lasted for only 2 months after discontinuation of the treatment. We conclude that the combined use of NMES and bracing is more effective than either alone but requires continuous application.

Hydrocephalus in children has many aetiologies, and can cause multiple ophthalmic and visual disorders. This study sets out to detect and quantify visual and visuoperceptual dysfunction in children who have received surgical treatment for hydrocephalus with and without myelomeningocele, and to relate the results to the associated diagnoses and results from a comparison group. Seventy-five school-aged children (41 males, 34 females) with surgically-treated hydrocephalus and 140 comparison children (76 males, 64 females) matched for age and sex underwent comprehensive ophthalmologic examination. Median age at examination was 9 years and 4 months (range 7y 4mo–12y 10mo). Visual function deficits were identified in 83% (62/75) of the children with hydrocephalus. Visual impairment (binocular visual acuity <0.3) was found in 15% (11/73; comparison group 0%) but in none with myelomeningocele. Strabismus was found in 69% (51/74; comparison group 4% [5/140], p<0.001), and refractive errors were found in 67% (47/70; comparison group 20% [28/140], p<0.001). Cognitive visual dysfunction was identified in 59% (38/64; comparison group 3% [4/140], p<0.001). These disorders were identified in various combinations and comprised impaired ability to plan movement through depth (e.g. going down a stair), impaired simultaneous perception, impaired perception of movement, impaired orientation, and (least frequently) impaired recognition. In this study, children with hydrocephalus associated with myelomeningocele were least commonly affected. Visual disorders were most frequent in those with epilepsy, cerebral palsy, and/or cognitive disability.

Little is known about the development of postural adjustments during early ontogeny. We examined postural adjustments due to sudden perturbations during sitting in 40 healthy term infants (28 males, 12 females) assessed in groups of eight at 1, 2, 3, 4, and 5 months of age. Surface electromyograms of neck, trunk, and leg muscles were recorded while the infants were exposed to a random series of horizontal forward and backward displacements of the surface of support. Video recordings of spontaneous motor behaviour were analyzed. For part of the analyses, previously collected data on 26 infants aged 6 to 10 months were included. In general, postural adjustments at all ages were direction specific and showed large variation. Within the variation developmental changes could be observed, revealing a transient decrease in postural activity at 3 months of age. After this transition, direction-specific postural activity was correlated with spontaneous motor behaviour. This was true, in particular, for dorsal postural activity. The clinical relevance of these findings is discussed.

A high incidence of long-bone fractures has been observed in children and young adults with quadriplegic cerebral palsy in residential care. This study aimed to determine factors that contribute to these fractures and to institute preventive treatment. Twenty individuals (12 males, eight females) of a cohort of 88 residents with spastic quadriplegia in residential care in Gauteng, South Africa who had sustained fractures were compared with a random sample of age-matched control participants (10 males, 10 females) from the same facility. Participants ranged in age from 6 to 29 years (median 17.5 years). The majority of fractures were in the upper extremities. There was radiological and biochemical evidence of rickets and osteomalacia in both groups. However, the severity of the disease was more pronounced in the group with fractures. There was a significant relation (p=0.002) between the number of fractures and the use of anticonvulsant therapy (ACT). Three months of vitamin D administration (calciferol 5000iu/day) resulted in a marked clinical improvement. There were no fractures during this period in either group. In addition, the mean serum calcium (Ca) and phosphate (Pi)levels increased (Ca from 2.17 to 2.35mmol/L and Pi from 1.13 to 1.66mmol/L) and mean total alkaline phosphatase level decreased (from 1123 to 423U/L). We concluded that vitamin D deficiency was the major factor contributing to the occurrence of fractures in this population. Unless sunlight exposure can be guaranteed, vitamin D supplementation should be considered for children and adults in residential care, especially if they are on ACT, even in areas with year-round sunshine.

Intrathecal baclofen infusion (IBI) is being used with increasing frequency in children to treat spasticity and dystonia. In this report, we summarize the clinical course of a 9-year-old boy with quadriplegic cerebral palsy with mixed tonal abnormalities (spasticity and dystonia) experiencing withdrawal from intrathecal baclofen. His clinical course is compared to that of adults experiencing withdrawal from IBI and to neuroleptic malignant syndrome. If unrecognized, this disorder may have significant potential for morbidity and mortality. Clues to diagnosis, appropriate evaluation, and potential treatments are discussed. When a child treated with IBI presents with unexplained multiorgan system dysfunction, particularly if accompanied by evidence of rhabdomyolysis, the integrity of the IBI system must be evaluated. In some cases, evaluation might necessitate surgical exploration. Caregivers most commonly seek urgent evaluation and treatment from their primary care provider when their child experiences fever or acute illness. Primary care providers of children treated with IBI should be made aware of this clinical scenario to prevent delays in diagnosis.

The primary aim of this investigation was to assess the correlation between the POSNA Musculoskeletal Functional Health Questionnaire (POSNA) and gait analysis in children with cerebral palsy (CP). POSNA and computerized gait analysis were used to evaluate individuals with CP. Correlations were investigated between POSNA scales, gait parameters, and the Gillette Functional Assessment Questionnaire (FAQ) in 63 children (31 males, 32 females; mean age 9.17 years [SD 3.06], age range 3.75 to 16.44 years) with spastic CP. Twelve participants had hemiplegia, 29 diplegia, 12 quadriplegia, and 10 triplegia. The result of backwards stepwise multiple regression analysis indicated that the Log normalcy index (NI) was a significant predictor of the POSNA Global Function and Comfort scale. Energy expenditure (EE) did not add significantly to the prediction. The POSNA scales differentiated between the different topographical types of CP. The POSNA scale is a valid and useful clinical measure. Used in conjunction with the NI, EE, and FAQ, the POSNA scale provides a more complete appraisal of change in functioning.

Outcome was studied of all children and adolescents with pseudoseizures without epilepsy, who were referred and treated as in-patients and/or day patients in the tertiary psychiatric ward at Birmingham Children's Hospital, UK between 1988 and 1994. Information was derived from case-note data. Freedom from seizures for six months was defined as ‘cure’, as no recurrences after this period were noted. Kaplan–Meier survival analysis was used. Seventeen patients were identified: 15 females and two males; mean age at presentation to the tertiary psychiatric service was 12 years 9 months (SD 26 months; range 8 years 3 months to 15 years 9 months). Fourteen participants recovered and resumed regular school attendance. Three were lost to follow-up due to being referred on to other agencies before recovery because they became too old for the service. Recovery followed an exponential distribution, with a mean symptom survival time following treatment of 1.5 years. These results are consistent with a treatment effect. Younger age at presentation, female sex, having more types of seizures, and not receiving both in-patient and outpatient treatment were associated with better outcome. The deteriorating prognosis with age at treatment suggests prompt identification may be important in ensuring a good outcome.

Moderate to severe sleep-disordered breathing (SDB) was identified in 20% (17 of 83) of children with spina bifida/myelomeningocele (SB/MM) at the Montreal Children's Hospital. The prevalence of SDB in patients with SB/MM elsewhere has not been determined. To establish current practices for identifying SDB in patients with SB/MM, questionnaires were sent to the coordinators of the 212 spina-bifida clinics in Canada and in the United States. Eighty-six (41%) questionnaires were returned, representing data on 13349 patients. Although 67% of the responding centers reported availability of cardiorespiratory sleep studies, only 996 (7.5%) patients with SB/MM had been tested and only 418 (3.1%) patients had been diagnosed with SDB. Across clinics, the prevalence of SDB was directly related to the frequency of testing. Of 380 deaths over the past 10 years, SDB and sudden unexplained death during sleep were identified as the cause of death in 49 (12.8%) and 34 (8.9%) patients, respectively. Moderate to severe SDB may not have been identified in a significant number of patients with SB/MM because they have not been tested.

The aim of this study was to determine whether there are primary effects of prematurity on the development of explicit memory. Elicited imitation of action sequences was used to compare immediate and 15-minute delayed memory in term and preterm infants (19 months corrected age; n=48) who were at low risk: none had experienced the medical or social risk factors often associated with preterm birth. Relative to infants born at term (38 to 40 weeks' gestation), children who had been born at 27 to 34 weeks' gestation showed lower levels of ordered recall; performance of healthy infants born at 35 to 37 weeks' gestation was intermediate and did not differ significantly from that of the other groups. These results suggest that specific cognitive deficits can occur as a function of preterm birth even in low-risk infants.

this report concerns the development and stability of recall memory at 12, 24, and 36 months of age in preterm (<1750g birthweight) and term children from a prospective, longitudinal study. there were 56 preterm children (55.4% male; mean birthweight 1119.5g [sd 279.2], range 551 to 1742g; mean gestational age 29.7 weeks [sd 2.9], range 25 to 36wks) and 126 term controls (51.6% male; mean birthweight 3453.8g [sd 438.2], range 2500 to 4564g; mean gestational age 40.2 weeks [sd 1.1], range 38 to 42wks). recall memory was assessed using an elicited imitation task in which children attempted to reproduce a sequence of actions 15 minutes after they were modeled by the examiner. relative to term children, the preterm group performed poorly at all three ages: they reproduced fewer actions overall and fewer in the correct order. lower birthweight and lengthier postnatal hospitalization were associated with poorer performance in preterm children at 24 and 36 months. although performance improved considerably for both groups with age, there was no evidence of preterm ‘catch-up’: the deficits detected at 12 months did not diminish with age. individual differences in recall were modestly stable from one year to the next, with cross-age correlations highest from 24 to 36 months (r=0.43 to 0.49). these results suggest that preterm children have deficits in recall memory that emerge by 12 months and persist into early childhood.

Following obstetrical brachial plexus injury, infants are unable to learn specific patterns of movement due to the disruption of neural pathways. Even with successful reinnervation (spontaneously or post surgical reconstruction), function can be suboptimal due to overactivity in antagonist muscles preventing movement of reinnervated muscles. Botulinum toxin type A (BTX-A) was used to temporarily weaken antagonistic muscles early in the reinnervation process following brachial plexus injury, with the aim of facilitating functional improvement. A case series of eight children (five females, three males; mean age 12.5mo [SD 6.43]; range 5–22mo) with significant muscle imbalances but evidence of reinnervation were given BTX-A injections into the triceps, pectoralis major, and/or latissimus dorsi muscles. After a single injection, all parents reported improvement in function. Active Movement Scale total score changed significantly between pre BTX-A and 1 month (p=0.014), and 4 months (p=0.022) post BTX-A injection. It is proposed that BTX-A facilitated motor learning through improved voluntary relaxation of antagonist muscles while allowing increased activity in reinnervated muscles.

Language and learning disabilities occur in almost half of individuals with oral clefts. The characteristics of these cognitive dysfunctions vary according to the cleft type, and the mechanisms underlying the relation between cleft type, cognitive dysfunction, and cleft-caused middle-ear disease are unknown. This study investigates preattentive auditory discrimination, which plays a significant role in language acquisition and usage, in infants with different cleft types. A mismatch negativity (MMN) component of brain evoked potentials, which indexes preconscious sound discrimination, and brain responses to rare sine-wave tones were recorded in 12 healthy infants and 32 infants with oral clefts at the ages of 0 and 6 months. Infants with clefts were subdivided into two categories: those with cleft lip and palate (CLP) (n=11 at birth, n=6 at the age of 6 months) and those with cleft palate only (CPO) (n=17 at birth, n=8 at the age of 6 months). At both ages, brain responses to rare sounds tended to be smaller in both cleft subgroups than in healthy peers. However, in the latency range of 300 to 500 ms, the MMN was significantly smaller in infants with CPO. In infants with CLP, the MMN was comparable to that of healthy infants. Differences in auditory discrimination between infants with CLP and CPO, as reflected by MMN, were detectable at birth and persisted into later infancy. This pattern parallels known behavioural differences between children with these cleft types. Brain responses to rare sounds, in contrast, had no differentiative power with respect to the cleft type.

This study reports on cognitive abilities and attainment in 29 children and 21 adults with Smith-Magenis syndrome. There were 13 boys and 16 girls aged 6 to 16 years, and nine men and 12 women aged 16 to 52 years. All had mild to severe learning disabilities with no differences overall between verbal and performance skills, but with a particular profile of cognitive strengths and weaknesses. Levels of attainment and of adaptive behaviour were strikingly low, and the group of adults emerged as much more dependent on carers than might have been expected from their general level of intellectual functioning. Reasons for this discrepancy are explored in terms of the severe behavioural difficulties characteristic of the syndrome.

Attention-deficit–hyperactivity disorder (ADHD) is a common neurodevelopmental disorder with a high degree of associated behavioural problems. In order to study characteristics of ADHD with and without oppositional defiant disorder (ODD) in a representative group of young children with clinical impairment in Sweden, 131 children (101 males, 30 females) with ADHD (mean age 5 years, SD 1 year 5 months; range 3 to 7 years) were clinically examined, and their parents interviewed. Independent questionnaire data (Child Behavior Checklist, ADHD Rating Scale-IV, ODD Rating Scale-IV, Conners Hyperactivity Index) were collected. For comparison 131 children without ADHD were matched for sex, age, marital status, and socioeconomic status (115 males, 16 females; mean age 4 years 10 months, SD 1 year 5 months; range 3 to 7 years). Sixty percent of those with ADHD met full DSM-IV criteria for ODD. Only 10 of the 131 children with ADHD had no symptoms of ODD at all. The rate of children meeting full diagnostic criteria for ODD was similar across all age cohorts. Males were overrepresented in ODD, as were children of divorced parents and of mothers with low socioeconomic status. ADHD combined subtype was a stable independent factor influencing the diagnosis of ODD, regardless of psychosocial factors. Those with ADHD with ODD consistently showed higher rates of ADHD symptoms than did those with ADHD without ODD. The prevalent comorbidity of ADHD with ODD indicates that all children presenting with ADHD or ODD symptoms need to be assessed with a view to exploring both types of problem behaviours. The link between ODD and some psychosocial variables indicates the need to address these, possibly by measures such as parent training and network support.

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.