Rehabilitation service utilization in children and youth with cerebral palsy.

AIM: To describe the pattern of use of rehabilitation services in children and adolescents with cerebral palsy (CP), and to identify factors associated with use. METHODS: In this study, parents of 91 school-age children and 167 adolescents with CP completed a questionnaire regarding educational and rehabilitation resources received within the last 6 months. Rehabilitation services included occupational therapy (OT), physical therapy (PT), speech language pathology (SLP), psychology and special education. Demographic characteristics were documented and developmental and functional status was assessed. Relationships between service utilization and sociodemographic factors, functioning and school setting were determined. RESULTS: Over half of children (53.2%) and adolescents (57.5%) were in regular schools; however, 41% of these required special education resources. The remainder (42.5-46.8%) was in special schools. The majority of children (84.6%) were receiving at least one rehabilitation service although this decreased (68.1%) in adolescence. PT and OT were most common and services were provided predominantly in the school setting. Services were primarily weekly direct interventions at school age, with weekly interventions or consultations most common for adolescents. Younger age was associated with service receipt at school age only. Children with greater motor limitations, lower IQ and greater activity limitations were more likely to receive OT, PT, SLP or special education. Children in segregated schools were significantly more likely to receive rehabilitation services, when compared with children in regular schools. CONCLUSIONS: The majority of children and youth received one or more services. Individuals with greater motor or cognitive challenges were more likely to receive a range of school-based services from rehabilitation specialists. When compared with children of school age, adolescents were less likely to receive services and when provided, services were more likely to be consultative. Services may need to be more optimally organized through childhood to enhance benefits to children with CP across activity limitation profiles.

Respect for autonomy in the healthcare context: observations from a qualitative study of young adults with cerebral palsy.

BACKGROUND: Respect for patient autonomy is a cornerstone of contemporary medical ethics and clinical practice. In its different shapes and forms (e.g. being informed, being engaged in discussions and decisions about medical care and being supported in developing healthcare preferences and choices), patient autonomy has been fostered by both paediatric and adult professional societies. The transition from paediatric to adult care creates a complex situation where autonomy for medical decisions shifts to the developing adolescent. More specific challenges to respect for autonomy may be experienced by young adults with cerebral palsy in the transition period where, for example, language and motor impairments may affect communication skills and this may be conflated with cognitive disability. AIM: To characterize perspectives towards autonomy in the healthcare context for young adults with cerebral palsy. METHOD: We carried out semi-structured interviews with 14 young adults (aged 18-25) with cerebral palsy. The audiotaped interviews were transcribed verbatim and analysed using a conventional thematic qualitative content analysis. RESULTS: Participants displayed a range of attitudes towards autonomy, suggesting that the value of autonomy is considered in light of competing values and of context. Testimonials from participants demonstrated that both contextual (e.g. ill-adapted health care, lack of specialized public transport) and relational (e.g. attitudes towards parental involvement in decision making) factors negatively or positively impact autonomy. CONCLUSION: We observed that there were four key elements interwoven in participants' characterization of autonomy: the coupling of decisional and physical autonomy, the influences of family and society on autonomy, the influence of healthcare professionals on autonomy and the need for preparation for autonomy.

Characterizing the Subcortical Structures in Youth with Congenital Heart Disease.

BACKGROUND AND PURPOSE: Congenital heart disease is a leading cause of neurocognitive impairment. Many subcortical structures are known to play a crucial role in higher-order cognitive processing. However, comprehensive anatomic characterization of these structures is currently lacking in the congenital heart disease population. Therefore, this study aimed to compare the morphometry and volume of the globus pallidus, striatum, and thalamus between youth born with congenital heart disease and healthy peers. MATERIALS AND METHODS: We recruited youth between 16 and 24 years of age born with congenital heart disease who underwent cardiopulmonary bypass surgery before 2 years of age (n = 48) and healthy controls of the same age (n = 48). All participants underwent a brain MR imaging to acquire high-resolution 3D T1-weighted images. RESULTS: Smaller surface area and inward bilateral displacement across the lateral surfaces of the globus pallidus were concentrated anteriorly in the congenital heart disease group compared with controls (q < 0.15). On the lateral surfaces of bilateral thalami, we found regions of both larger and smaller surface areas, as well as inward and outward displacement in the congenital heart disease group compared with controls (q < 0.15). We did not find any morphometric differences between groups for the striatum. For the volumetric analyses, only the right globus pallidus showed a significant volume reduction (q < 0.05) in the congenital heart disease group compared with controls. CONCLUSIONS: This study reports morphometric alterations in youth with congenital heart disease in the absence of volume reductions, suggesting that volume alone is not sufficient to detect and explain subtle neuroanatomic differences in this clinical population.

Functional interhemispheric asymmetries at birth as demonstrated by somatosensory evoked potentials.

Functional cerebral hemispheric asymmetries detectable at birth have been suggested by a number of neuroanatomic, neuroradiologic, and clinical neurophysiologic modalities. The aim of this study was to determine whether functional interhemispheric asymmetries can be identified using electrophysiologic measures. As part of a prospective study, somatosensory evoked potentials following median nerve stimulation were recorded in nine healthy full-term newborns on day 2 or 3 of life, and somatosensory evoked potentials were repeated at 2 and 6 months of age. These children were subsequently examined at 1 and 3 years of age by a pediatric neurologist and all had normal examinations. Handedness was determined at 3 years by questioning the parent and by clinical observation. Three of nine were left-handers. All three left-handers had clearly identifiable neonatal N19 parietal potentials over the right hemisphere. Following right median nerve stimulation, contralateral parietal potentials were absent on two of the three and questionable in the third. Asymmetries were not clearly present in right-handers although only one showed an increased maturation of the right hemisphere relative to the left. At 2 months of age, interhemispheric differences were no longer clearly evident. This data suggests that preferential hemispheric asymmetries are masked by 2 months of age. This differential rate of development demonstrated by neonatal somatosensory evoked potentials may be an early indicator of ultimate handedness.

Prediction of outcome at school age in neonatal intensive care unit graduates using neonatal neurologic tools.

Prediction of outcome for neonatal intensive care unit graduates is clinically useful to counsel families effectively and target those who may benefit from early interventions. Evoked potentials have proven prognostic value of neurologic outcomes in early childhood; however, their long-term predictive validity remains to be determined. The objective of this prospective study was to determine the long-term predictive value of three neonatal neurologic assessments: brainstem auditory evoked potentials, somatosensory evoked potentials, and the Einstein Neonatal Neurobehavioral Assessment Scale. Seventy-eight high-risk newborns and 28 healthy controls were recruited and were assessed in the newborn period using these tests. At 8 to 9 years of age, 42 subjects and 13 controls were re-evaluated for developmental progress using a range of psychologic, sensorimotor, and neurologic measures. Findings indicated that the somatosensory evoked potential was most accurate at predicting outcome at school age, with high specificity (83-100%) across all domains tested and good sensitivity (80-100%) for intellectual performance and sensorimotor abilities. The brainstem auditory evoked potential was limited by false-negatives, whereas the neonatal neurobehavioral assessment yielded many false-positives. This study provides new evidence that associations between neonatal somatosensory evoked potentials and developmental sequelae continue to be significant at school age.

The effect of gestational age at birth on somatosensory-evoked potentials performed at term.

Multimodality-evoked potentials are widely used in newborns to assess the maturation and integrity of the sensory pathways. Reliable normative data are needed to maximize the utility of this technique as a diagnostic and research tool. Several electrophysiologic studies on the maturational changes of the auditory brain-stem response have demonstrated that latency measurements decrease as a function of increasing conceptional age. However, maturational studies of the somatosensory-evoked potential, particularly in low-risk premature infants, are limited. The existing evoked potential literature in healthy newborns proposes that maturation of the central nervous system occurs at a predictable rate, irrespective of a given gestational age at birth. Behavioral studies of premature infants suggest that neurologic development may be altered by early extrauterine exposure. The purpose of this study was to determine whether brain-stem auditory- or somatosensory-evoked potential conduction times were comparable in premature and full-term infants matched for conceptional age. The results of this study suggest that myelination is determined by conceptional age, independent of premature birth.

Long-term developmental outcome of asphyxiated term neonates.

Asphyxia remains one of the main causes of later disability in term infants. Despite many publications identifying possible predictors of outcome in this population of interest, little is known of the long-term developmental outcome of asphyxiated term neonates. Observational studies have largely focused on short-term outcomes, with an emphasis on significant neurologic sequelae and intellectual impairments. This article reviews the literature that has described the developmental outcome of asphyxiated term newborns. As part of this review, we have also highlighted the evolution of the definition of asphyxia and delineated appropriate markers that should be used in future research on this population.

The determination of sensory deficits in children with hemiplegic cerebral palsy.

Therapeutic intervention for children with cerebral palsy begins with accurate appraisal of abilities and disabilities. Currently, treatment focuses on the identified motor deficits, with any underlying sensory deficits often being overlooked. Sensory input is an essential component of motor function and motor control. Therefore, the objective of this study was to determine the presence and extent of sensory deficits in school-aged hemiplegic children using a formal clinical sensory battery, as well as somatosensory evoked potentials. A cross-sectional comparative study was performed comparing sensory function in hemiplegic children and healthy controls. Nine hemiplegic children and 41 healthy controls between 4 and 19 years of age were assessed. Hemiplegic children were included if they had a minimum level of receptive language function of 30 to 33 months and expressive language ability of 24 to 27 months, and no severe limitation of joint range of the hand. Significant bilateral sensory deficits (88.8%) were ascertained in hemiplegic children (P < .05), when compared to the performance of the healthy controls. Stereognosis and proprioception were the chief modalities affected bilaterally. The extent of sensory loss did not mirror the severity of motor deficit. Conversely, findings on somatosensory evoked potentials were closely related to motor function. Thus, a clinical sensory evaluation should be an integral part of the assessment of children with cerebral palsy. The likelihood of sensory impairment in one or more modalities on the hemiplegic or nonhemiplegic side is underappreciated and needs to be identified by rehabilitation specialists to maximize the functional potential of these children.

Neurologic perspectives of Crigler-Najjar syndrome type I.

Limited information exists on the neurologic sequelae of Crigler-Najjar syndrome type I despite this being the major morbidity of this rare autosomal recessive disorder of bilirubin conjugation that results in chronic unconjugated hyperbilirubinemia. Two patients with identical underlying genetic mutations resulting in Crigler-Najjar syndrome type I were assessed from a neurodevelopmental perspective in late childhood using age appropriate standardized measures. In addition, the English language literature of case reports and series describing the outcomes of patients with this disorder was reviewed (descriptive meta-analysis) and summarized with particular reference to neurologic symptomatology, pattern of neurologic disability, age of onset of symptoms, and therapeutic interventions. Despite radically different therapeutic interventions, our two patients did not differ in outcome measures. Review of the literature reveals distinct, often age-related, patterns of neurologic sequelae reflecting injury to basal ganglia, cerebellar, and likely hippocampal structures. Definitive prevention of the neurologic sequelae that often occur within the context of Crigler-Najjar syndrome type I requires that curative treatment (hepatic transplantation, presently, and gene therapy in the future) be applied prior to the possible onset of neurologic symptoms in adolescence.

Etiologic yield of autistic spectrum disorders: a prospective study.

At present, the etiologic yield in community-derived samples of young children with an autistic spectrum disorder is not known. To address this question, all young children (under 5 years of age) referred for an initial assessment to ambulatory pediatric neurology or developmental pediatric clinics at a tertiary university center over an 18-month period for a suspected developmental delay were prospectively identified. Specific diagnostic testing was left to the discretion of the evaluating physician. In all, 50 children with an autistic spectrum disorder were assessed. Detailed history or physical examination was informative with respect to suggesting the possibility of an underlying etiology in a minority (10/50,20%). Genetic studies (FMR-1, karyotype), electroencephalography (EEG), and neuroimaging were carried out in a majority (42/50, 34/50, and 33/50, respectively) of the children, for the most part on a screening rather than an indicated basis (31/42, 34/34, and 28/33, respectively). Etiologic yield was low (1/50, 2%), with only a single child identified with a possible Landau-Kleffner variant on sleep EEG tracing. The results suggest an evaluation paradigm with reference to etiologic determination for young children with autistic spectrum disorder that does not presently justify metabolic or neuroimaging on a screening basis. Recurrence risk and treatment implications, however, suggest that strong consideration be given to genetic (FMR-1, karyotype) testing and EEG study despite a relatively low yield.

Profile of referrals for early childhood developmental delay to ambulatory subspecialty clinics.

The objective of this study was to determine the profile and pattern of referral to subspecialty clinics of young children with suspected developmental delay together with the factors prompting their referral. All children under 5 years of age referred to either developmental pediatrics or pediatric neurology clinics at a single tertiary hospital over an 18-month period were prospectively identified. Standardized demographic and referral information were collected at intake, final developmental delay subtype diagnosed was identified, and referring physicians were surveyed regarding factors prompting referral. A total of 224 children met study criteria. There was a marked male preponderance (166/224), especially among those with either cognitive or language delay. Two delay subtypes, global developmental delay and developmental language disorder, accounted for two thirds of the diagnoses made. For slightly more than one third of the children (75/224), the delay subtype diagnosed following specialty evaluation was different from that initially suspected by the referring physician. A mean delay of 15.5 months was observed for the cohort as a whole between initial parental concern and specialty assessment. For referring physicians, the major factor prompting referral was the severity of the observed delay. The most important aspects of the specialty evaluation according to referral sources were the identification of a possible etiology and confirmation of delay. A profile of referrals and the rationale thereof for a cohort of children with suspected developmental delay is presented that, although locale specific, has implications for service provision and training.

Association between electroencephalographic findings and neurologic status in infants with congenital heart defects.

Neurologic status is of concern in infants with congenital heart defects undergoing open heart surgery. The association between perioperative electroencephalography (EEG) with acute neurologic status and subsequent outcome was examined in a cohort of 60 infants. Preoperative EEG and neurologic examinations were performed within 1 to 2 days prior to surgery (n = 27) and postoperatively (n = 47). Prior to surgery, 15 of 27 infants had normal EEG, whereas 5 had epileptiform activity and 9 had disturbances in background activity that were primarily moderate (8/9) and diffuse (7/9). Postoperatively, only 17 of 47 infants had normal recordings. Newborns (<1 month) were more likely (P< .001) to demonstrate EEG abnormalities than infants. Epileptiform activity was documented in 15, whereas 28 had background abnormalities that were moderate-severe (22/28) and diffuse (20/28) in most. Epileptiform activity prior to surgery was always associated with an abnormal neurologic examination, and this association persisted postoperatively (86%). Moderate to severe background abnormalities in the postoperative EEG was also strongly associated with acute neurologic abnormalities (93%). Severe background abnormalities (n = 5) were 100% predictive of death or severe disability. Long-term follow-up revealed that all children with normal postoperative EEGs had positive neurologic outcomes (P = .04); however, there were many false positives. Perioperative EEG abnormalities increased the likelihood for acute neurologic findings, whereas normal recordings following surgery were reassuring with regard to a favorable outcome.

Multimodality evoked potential findings in infants with congenital heart defects.

Evoked potentials are sensitive prognostic tools in young infants at risk for developmental disability. The objective of this prospective study was to determine whether infants with congenital heart defects demonstrate evoked potential abnormalities prior to or following open heart surgery, and to examine the association between these abnormalities and developmental status 1 year following surgery. A consecutive series of newborns (less than 1 month old) and infants (1 month to 2 years old) were recruited. Somatosensory and brain stem auditory evoked potentials were carried out before or after cardiac surgery, or both. One year later, neurologic examination and standardized measures of motor performance and functional independence were carried out. Twenty-seven newborns and 31 infants underwent perioperative somatosensory evoked potential recordings. Results indicate that perioperative somatosensory evoked potential abnormalities were common in newborns (41%) but not in infants (13%) with congenital heart defects. Brainstem conduction times were within normal limits in all subjects; however, 32% presented with mild elevations in hearing thresholds. All newborns with abnormal somatosensory evoked potentials had abnormal neurologic examinations both perioperatively and again 1 year after open heart surgery. Moreover, standardized developmental assessments 1 year following surgery indicate that all newborns with somatosensory evoked potential abnormalities had developmental deficits in one or more domains. Somatosensory evoked potential abnormalities in the perioperative period are common in newborns with congenital heart defects, and are strongly predictive of persistent developmental delay later.

Benefits of early intervention for children with developmental disabilities.

Early intervention programs are designed to enhance the developmental competence of participants and to prevent or minimize developmental delays. Children targeted for early intervention may either include environmentally or biologically vulnerable children, or those with established developmental deficits. There is growing consensus based on the best available evidence that early interventions can exert moderate positive effects. However, this literature is limited by substantial methodological weaknesses in most studies. Therefore further randomized clinical trials are needed to ascertain which programs best meet the needs of children with or at risk for developmental disability.

Developmental progress of children with congenital heart defects requiring open heart surgery.

Recent advances for infants requiring early open heart surgery have resulted in a dramatic decline in mortality and severe morbidity. The developmental progress of these new survivors is currently being defined. Causes contributing to brain injury are multifactorial, and may involve preoperative, intraoperative, and postoperative events. Before surgery, these children often exhibit hypotonia, poor state regulation, microcephaly, and developmental delays. These findings are particularly prevalent in newborns. In the acute postoperative period, neurodevelopmental deficits continue to manifest clinically. Long-term follow-up studies indicate that subtle neurological deficits and global developmental lags are characteristic of this population. Overall, severe neurological sequelae are uncommon; however, mild to moderate developmental disabilities are prevalent. Functional limitations, academic achievements, and health-related quality of life are areas that deserve further attention.

Etiologic determination of childhood developmental delay.

To determine the etiologic yield in young children with developmental delay referred to sub-specialty clinics for evaluation. Over an 18-month period, all children less than 5 years of age referred to the ambulatory pediatric neurology or developmental pediatrics clinics of the Montreal Children's Hospital for initial evaluation of a suspected developmental delay were enrolled. Features evident on history or physical examination were determined at intake as were the laboratory tests (and their rationale) requested by the evaluating physicians. Six months post initial assessment, detailed chart review was undertaken to determine if an etiology was found and the basis for such a determination. Bivariate and multivariate logistic regression was used to test for associations between factors present at intake and successful ascertainment of an underlying etiology. Two hundred and twenty-four children met study criteria. Etiologic yield varied across childhood developmental delay subtypes, and was 44/80 for global developmental delay [GDD] (55%), 13/22 for motor delay [MD] (59.1%), 3/72 for developmental language disorders [DLD] (4.2%), and 1/50 for autistic spectrum disorders [ASD] (2%). For GDD, the presence of historical features or findings on physical examination was associated with greater likelihood for successful etiologic determination with the following items significant in multiple logistic regression analysis; microcephaly, antenatal toxin exposure, focal findings. For MD, physical findings or the co-existence of a cerebral palsy symptom complex predicted a successful search for etiology. For both groups, the severity of the delay did not predict etiologic yield. For both groups, a small number of etiologic categories accounted for the majority of diagnoses made. Etiologic yield in childhood developmental delay is largely dependent on the specific developmental delay subtype. Paradigms for systematic evaluation of this common child health problem can be suggested, however they await validation.

Reliability of parental recall of developmental milestones.

The developmental history is a key element of the pediatric evaluation. Precise determination of a child's milestones depends on the parent's recollection of developmental events. As part of a prospective study examining neonatal predictors of outcome, healthy and high-risk newborns were assessed by a neurologist at 1, 3, and 5 years of age. The consistency of a parent's recall of two milestones (i.e., first steps and first meaningful word) was examined at 3 and 5 years. The answers provided by the parent(s) at the first visit (mean/S.D.: 12.67/1.19 months) provided the comparison standard. Twenty-five healthy and 50 high-risk infants had acquired 1 or both developmental skills by the first visit. Of 75, 63 were reexamined at 3 and/or 5 years by the same neurologist. For first steps, excellent correlations were obtained at 3 years (r = 0.74) and modest correlations were determined at 5 years (r = 0.41). The mean difference in age reported between 1 and 3 years was 0.36 +/- 1.81 months, and 0.33 +/- 2.46 months between 1 and 5 years. For first word, correlations were poor at 3 and 5 years (r = 0.27 and r = -0.11, respectively), with mean discrepancies in recall of 2.43 +/- 4.37 months (3-year visit) and 2.74 +/- 5.56 months (5-year visit). Twenty percent (n = 13) of parents reported discrepancies of 6 or more months (mean: 9.4; range: 6-25 months). Furthermore, this error in reporting of first word was later than the correct age in all patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Evoked potentials as predictors of outcome in neonatal intensive care unit survivors: review of the literature.

Neonatal intensive care unit survivors are at substantial risk for a range of neurodevelopmental sequelae, and therefore a variety of clinical diagnostic techniques have been evaluated as predictors of outcome. We summarize the prognostic value of evoked potentials in newborns at risk. A review of the literature reveals that brainstem conduction abnormalities in auditory brainstem evoked potentials are associated with neuromotor impairment; however, there are many false negative studies. Visual evoked potentials are highly accurate in predicting neurologic deficits in early childhood in asphyxiated term neonates. Sensitivity and specificity are consistently high for somatosensory evoked potentials in term newborns; however, correlations with outcome in premature infants is controversial. Several studies have compared neonatal findings on neuroimaging studies and evoked potentials, and concordant results between these two tests are highly predictive. However, neurologic sequelae often can most accurately be predicted by visual or somatosensory evoked potentials. Evoked potentials may therefore be a useful adjunct to the clinical investigation and prognostication of outcome in the high risk newborn.

Clinical features of developmental disability associated with cerebellar hypoplasia.

Sporadic nonsyndromic cerebellar hypoplasia is a radiological diagnosis with clinical features and a relation with developmental disability that are presently not known. Through a retrospective review of a comprehensive standardized computerized database containing more than 2,500 patients examined consecutively by a pediatric neurologist, 11 with nonfamilial, nonsyndromic cerebellar hypoplasia on neuroimaging (CT and/or MRI) were identified. With the exception of two patients, all had been originally referred during infancy or the preschool years for assessment of a developmental disability. All 11 had "cerebellar" findings on initial examination and five were microcephalic; three others were below the 10th percentile. All exhibited developmental disability, commonly of mild to moderate degree. Motor involvement predominated, often involving fine motor skills more than gross motor functions. Imaging consistently disclosed vermis hypoplasia with additional supratentorial cerebral dysgenesis in one child and cerebellar hemispheric hypoplasia in another. Cerebellar hypoplasia is a developmental anomaly that appears to be either etiologically related to, or a marker for, developmental disability, thus confirming the intact cerebellum's integral role in normal psychomotor development. This series suggests that cerebellar hypoplasia should be considered in the young child presenting with developmental delay with prominent motor involvement, together with cerebellar signs and/or microcephaly.

Influence of gestational age, birth weight, and asphyxia on neonatal neurobehavioral performance.

The objectives of this study were to determine the influence of birth weight, gestational age, and asphyxia on neurobehavioral performance at 40 weeks conceptional age, and to demonstrate that this performance may differ in preterm high-risk infants when using low-risk preterm rather than healthy term infant norms. A cross-sectional comparative design was used to compare neurologic behavior in healthy and high-risk newborns at 40 weeks conceptional age using the Einstein Neonatal Neurobehavioral Assessment Scale. Seventy-four high-risk newborns were selected from a consecutive sample of asphyxiated, very-low-birth-weight, and small-for-gestational-age neonates. Controls did not fulfill any of the high-risk criteria and had no pre- or perinatal complications. There were 37 healthy term and 17 low-risk preterm controls. Statistically significant differences occurred between high-risk newborns and term controls for the total score (P < .001) as well as for most individual items (P < .05). Analysis of high-risk subgroups revealed that small-for-gestational-age and term asphyxiated newborns had the most abnormal responses. When comparing test performance between preterm high-risk and term controls, the majority of test items achieved significance (P < .05); however, when compared to low-risk preterm controls, fewer items were abnormal, and the probability level on abnormal items was less significant than expected. This disparity in interpretation of neurobehavioral status in preterm high-risk neonates using a preterm versus term reference sample warrants further investigation. Appropriate normative standards for preterm infants require definition because it may increase the utility of the neonatal neurologic examination and its prognostic value.