Chantal Poulin

Participation and Enjoyment of Leisure Activities in School-Aged Children with Cerebral Palsy.

The objective of this study was to characterize participation in leisure activities in children with cerebral palsy (CP) and identify determinants of greater involvement. Ninety‐five children of school age (9y 7mo [SD 2y 1mo]) with CP were recruited, and participation was evaluated with the Children’s Assessment of Participation and Enjoyment in a subset (67/95; 42 males, 25 females) who could actively participate in completion of the assessment. Most had mild motor dysfunction (Gross Motor Function Classification System: 59% level I, 23% level II, 18% levels III–V) and had a spastic subtype of CP (23 hemiplegia, 17 diplegia, 16 quadriplegia, 11 other). Biomedical, child, family and environmental predictor variables were considered in the analysis. Results demonstrated that these children were actively involved in a wide range of leisure activities and experienced a high level of enjoyment. However, involvement was lower in skill‐based and active physical activities as well as community‐based activities. Mastery motivation and involvement in rehabilitation services enhanced involvement (intensity and diversity) in particular leisure activities, whereas cognitive and behavioral difficulties, activity limitations, and parental stress were obstacles to participation.

Leisure Activity Preferences for 6- To 12-Year-Old Children with Cerebral Palsy.

Aim  The objective was to describe leisure activity preferences of children with cerebral palsy (CP) and their relationship to participation. Factors associated with greater interest in leisure activities were identified.

Level of motivation in mastering challenging tasks in children with cerebral palsy

Aim  The aim of this study was to describe and identify factors associated with motivation in children with cerebral palsy (CP).

Mutations in FLVCR1 Cause Posterior Column Ataxia and Retinitis Pigmentosa

The study of inherited retinal diseases has advanced our knowledge of the cellular and molecular mechanisms involved in sensory neural signaling. Dysfunction of two specific sensory modalities, vision and proprioception, characterizes the phenotype of the rare, autosomal-recessive disorder posterior column ataxia and retinitis pigmentosa (PCARP). Using targeted DNA capture and high-throughput sequencing, we analyzed the entire 4.2 Mb candidate sequence on chromosome 1q32 to find the gene mutated in PCARP in a single family. Employing comprehensive bioinformatic analysis and filtering, we identified a single-nucleotide coding variant in the feline leukemia virus subgroup C cellular receptor 1 (FLVCR1), a gene encoding a heme-transporter protein. Sanger sequencing confirmed the FLVCR1 mutation in this family and identified different homozygous missense mutations located within the proteins transmembrane channel segment in two other unrelated families with PCARP. To determine whether the selective pathologic features of PCARP correlated with FLVCR1 expression, we examined wild-type mouse Flvcr1 mRNA levels in the posterior column of the spinal cord and the retina via quantitative real-time reverse-transcriptase PCR. The Flvcr1 mRNA levels were most abundant in the retina, followed by the posterior column of the spinal cord and other brain regions. These results suggest that aberrant FLVCR1 causes a selective degeneration of a subpopulation of neurons in the retina and the posterior columns of the spinal cord via dysregulation of heme or iron homeostasis. This finding broadens the molecular basis of sensory neural signaling to include common mechanisms that involve proprioception and vision.

Indicators of distress in families of children with cerebral palsy

Purpose: To describe family distress as reported by parents of children with cerebral palsy (CP) and to identify factors associated with distress. Method: In this descriptive, historical cohort study, parents of school-age children (9.2 ± 2.1 years) with CP completed the Parenting Stress Index, the Impact on Family Scale and family-related items on the Child Health Questionnaire. Predictor variables considered were sociodemographic factors, motor, cognitive and behavioral difficulties and functional limitations. These were assessed using the Gross Motor Function Measure, Leiter IQ, Strengths and Difficulties Questionnaire and Vineland Adaptive Behavior Scale. Results: Parents of 95 children were recruited, of whom 45% were highly stressed and 11% defensive. Half indicated that their child’s health impacted on their time, emotional status and family activities. Family distress measures were modestly associated with motor (r = 0.30–0.48) and cognitive abilities (r = 0.29–0.37) but more strongly correlated with particular behavioral difficulties (r = −0.42 to 0.55). Activity limitations across domains were highly associated with measures of distress. Conclusions: Parents of school-aged children with CP are likely to experience high stress, increased time constraints and financial and psychological burden. Findings illustrate the need to monitor family functioning intermittently as the child develops and direct appropriate resources to optimize child and family well-being. Implications for Rehabilitation School age is a stage of development that brings new challenges to a child with cerebral palsy. Parents of school-aged children with cerebral palsy experience high levels of stress, time constraints and emotional and financial burden. The child’s activity limitations and especially behavioral difficulties may contribute to greater family distress. Family well-being should be monitored periodically, and appropriate resources and supports provided when families are in distress.

Play and Be Happy? Leisure Participation and Quality of Life in School-Aged Children with Cerebral Palsy

The objective of this study was to examine the association between leisure participation and quality of life (QoL) in school-age children with cerebral palsy (CP). Leisure participation was assessed using the Childrens Assessment of Participation and Enjoyment (CAPE) and QoL using the Pediatric Quality of Life Inventory (PedsQL). Pearson correlation coefficients were calculated to examine the association between CAPE and PedsQL scores, and a multiple linear regression model was used to estimate QoL predictors. Sixty-three children (mean age 9.7 ± 2.1 years; 39 male) in GMFCS levels I–V were included. Intensity of participation in active-physical activities was significantly correlated with both physical (r = 0.34, P = 0.007) and psychosocial well-being (r = 0.31, P = 0.01). Intensity and diversity of participation in skill-based activities were negatively correlated with physical well-being (r = −0.39, P = 0.001, and r = −0.41, P = 0.001, resp.). Diversity and intensity of participation accounted for 32% (P = 0.002) of the variance for physical well-being and 48% (P < 0.001) when age and gross motor functioning were added. Meaningful and adapted leisure activities appropriate to the childs skills and preferences may foster QoL.

Topiramate for the Treatment of Neonatal Seizures

Therapeutic options for treating neonatal seizures, such as phenobarbital and phenytoin, lack efficacy and are potentially harmful to the developing brain. Topiramate appears effective as both an antiseizure and neuroprotective agent in animal models of newborn brain injury. Although topiramate is a common add-on agent in newborns, its use in this population has not yet been reported. We performed a retrospective cohort study of clinical topiramate use in newborns with acute symptomatic seizures that were refractory to standard agents. In four of six newborns, apparent reduction or no further seizures occurred. None of the children experienced side effects resulting in discontinuation of the drug, either during the hospital admission or after discharge. Prospective studies evaluating the safety and efficacy of topiramate for both seizures and neuroprotection will be important in determining whether it deserves widespread use in clinical practice.

Stability of motor impairment in children with cerebral palsy

Classification of the quality and topographical pattern of motor impairment is used to describe cerebral palsy (CP). As an adjunct to a study characterizing the quality of life and participation of school‐age children with C P, initial and follow‐up classification of CP were compared. A cohort of 93 children (58 males, 35 females) were initially assessed at a mean age of 2 years 6 months (SD 2y 2mo) and re‐assessed at 9 years 4 months (SD 2y 2mo) with a mean interval of 6 years 6 months (SD 2y 4mo) between assessments. Sixty children had Gross Motor Function Classification System levels I‐III. All but one of the children were still classified has having CP at follow‐up. Type of CP documented remained constant in 67 children (72%; 95% confidence interval 62‐80). Clinical factors that were statistically significant (p<0.05) as possible predictors of CP subtype change were original classification of a non‐spastic subtype or a non‐spastic quadriparetic subtype. Change in CP subtype occurs in an appreciable minority of children with CP, which is likely to reflect a combination of intrinsic and extrinsic influences. Such change may challenge efforts to monitor the effects of interventions in this population.

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.

Behavioral Difficulties in Adolescents With Cerebral Palsy

Behavioral problems are common in children with cerebral palsy; however, little is known about the persistence of these difficulties during adolescence. This study aimed to describe the nature and frequency of behavioral difficulties in adolescents with cerebral palsy and to explore associated factors. Parents of the participants completed the Strengths and Difficulties Questionnaire. Participants’ motor and cognitive abilities, functional status, as well as parental stress were evaluated. Overall, the study enrolled 160 adolescents with cerebral palsy (65 girls, mean age 15.4 ± 2.17 years). Behavioral difficulties were present in 36.9% of the adolescents, with peer problems the most frequently reported (61.9%). Prosocial behaviors were associated with better function (r = 0.24-0.65) whereas hyperactive symptoms were associated with greater limitations (r = –0.19 to –0.55). Weak associations were found between parental stress and externalized problems (r = 0.22−0.24). Behavioral difficulties remained frequent in adolescents with cerebral palsy, particularly in those with greater functional limitations.