Postural adjustment of children with spastic diplegic cerebral palsy during seated hand reaching in different directions.

OBJECTIVES: To examine the effect of reaching in different directions on postural adjustment in children with diplegic cerebral palsy (CP), and to examine the relationship between hand reach performance and postural adjustment, and between postural control ability and postural adjustment. DESIGN: Cross-sectional study. SETTING: A movement science laboratory at a medical university. PARTICIPANTS: Children with CP (n=12) and typically developing (TD) children (n=16). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Two force platforms were used to measure the ground reaction force (GRF) and center of pressure (COP) data. Absolute peak COP velocity, COP sway ratio (SR), and mean GRF in the anterior posterior direction during the acceleration and deceleration segments of a reaching task were the main outcome measures. RESULTS: Children with CP showed a greater absolute peak COP velocity in the medial lateral direction, a smaller SR (wider COP pattern), and greater amplitude of force modulation (exaggerated postural adjustments) than TD children in lateral or medial reaches. There was a moderate correlation between SR and total Pediatric Reach Test score. The chair SR was also negatively correlated with the hand movement units. CONCLUSIONS: Children with CP showed wider, more crooked, and less efficient COP patterns than TD children, especially on medial or lateral reaches. Reaching medially or laterally involves trunk rotation, which produces more postural challenges than reaching anteriorly to children with CP. The patterns of postural adjustments in children with CP were correlated with their postural control ability and hand-reach smoothness.

Examining changes in self-perceived quality of life in children and adolescents with physical disability using a longitudinal design.

PURPOSE: The study aimed to investigate the changes in self-perceived quality of life (QOL) of children and adolescents with physical disability (PD). METHOD: Two hundred students aged 10-18 with PD were recruited from primary and high schools in Taiwan to participate in this longitudinal study. The Student Version of the Comprehensive Quality of Life Scale (COMQOL-S) was used to measure self-perceived QOL, which was recorded at baseline, and 6, 12 and 18 months post-baseline. Statistically, linear mixed models were used to compare the QOL scores among the four time points. RESULTS: At the second, third and fourth visit, 192, 151 and 128 participants completed COMQOL-S, respectively. Significant changes over time were found in overall objective and subjective OQL scores (slope = 0.8, p = 0.002 and slope = -1.3, p < 0.001, respectively). Specifically, significant reductions of the subjective QOL scores over the period of the four visits were observed in five domains, namely, Productivity (slope  =  -1.7, p = 0.005), Intimacy (slope  =  -1.6, p = 0.004), Safety (slope = -1.6, p = 0.008), Place in community (slope  =  -1.4, p = 0.019) and Emotional wellbeing (slope  =  -2.5, p < 0.001). Age was negatively correlated with the change of overall subjective score. Positive correlation was found between the change in mean objective scores and mean subjective scores in the Intimacy and Productivity domains. CONCLUSIONS: Even though the conditions of material wellbeing and health can be kept steady, the overall QOL of children and adolescents with PD could still decline over time. This might be attributable to the fact that social life becomes more sophisticated when children become older.

Effect of task constraint on reaching performance in children with spastic diplegic cerebral palsy.

The purposes of the study were to examine the effect of task constraint on the reaching performance in children with spastic cerebral palsy (CP) and to examine the correlations between the reaching performance and postural control. Eight children with CP and 16 typically developing (TD) children participated in the study. They performed a reach-and-return task with a seated posture on a stool. The target for reaching was set at a 120% arm-length distance in three directions (anterior, medial, and lateral). Reaching speed was modulated with a metronome at a rate of 46 beats/min. A motion analysis system recorded the kinematic data of reaching at a sampling rate of 150 Hz. Postural control was assessed with a pediatric reaching test. Movement time (MT), straightness ratio (SR), hand peak velocity (PV), and movement unit (MU) of reaching were compared between groups and among task conditions with repeated measure ANOVAs. Pearson's product-moment correlation coefficients were used to examine the correlations between reaching and postural control. Children with CP presented longer MT, larger SR and more MU than did TD children. Further, the children with CP showed larger SR while reaching medially and laterally than anteriorly. But TD children were not affected by these task constraints. Moderate correlations between postural control ability and SR and MU were noted. In conclusion, the children with CP showed a slower, more skewed, less efficient and less coordinated pattern of reaching than the TD children. Reaching laterally and medially seemed to impair the reaching performance (more skewed and less efficient) of the children with CP, but not of the TD children. Reaching laterally and medially may involve trunk rotation which produces more postural challenges than reaching anteriorly. This finding may explain the difference in the effect of task constraint on hand reaching performance between the two groups of children. Moreover, the better the postural control ability, the straighter, and more efficient and coordinated reaching performance the children showed.

Effect of seat surface inclination on postural stability and forward reaching efficiency in children with spastic cerebral palsy.

The purpose of this study was to examine the effect of seat surface inclination on postural stability and forward reaching efficiency in 10 children with spastic cerebral palsy (CP) and 16 typically developing (TD) children. The children performed a static sitting and a forward reaching task while sitting on a height- and inclination-adjustable stool at flat, three anterior-inclined, and three posterior-inclined positions. Postural stability was expressed as normalized (with body weight) peak vertical ground reaction force, center of pressure displacement in the anterior/posterior directions (COP_AP), in the medial/lateral directions (COP_ML), and sway ratio (COP_AP/COP_ML). Reaching efficiency was expressed as reaction time and movement time of arm reaching forward to a target. The results showed that seat inclination affected children's postural stability and the effects were comparable for CP and TD children in all measures except for COP_ML. Children with CP presented much larger COP_ML than TD children at the posterior-inclined positions relative to the flat and the anterior-inclined positions. Seat inclination affected reaching efficiency for both groups of children equally. Efficiency was better at the anterior positions than the posterior positions. Anterior-inclined positions improved postural stability and reaching efficiency. Posterior positions posed greater postural challenge and the challenge was tougher for children with CP.

Do physical disabilities affect self-perceived quality of life in adolescents?

PURPOSE: We examined to what extent physical disabilities (PD) affect self-perceived quality of life (QOL) among adolescents. METHOD: A survey was conducted on 157 adolescents (aged 15.6 +/- 1.6 years) with PD, who were attending high schools in Taiwan; 855 students (15.3 +/- 1.6 years) from the same geographic regions and without a disability were recruited as controls. The Student Version of the Comprehensive Quality of Life Scale (COMQOL-S) was used to assess their subjective and objective well-being. RESULTS: No significant differences in overall objective QOL score were found between the two groups but the PD group was poorer in health and material well-being. Adolescents with PD scored significantly higher in overall subjective QOL and all the seven domains examined. Stratified analysis showed that older students and female students with PD had lower life satisfaction in some domains. There were no significant differences in overall objective (62.1 +/- 8.3 vs. 60.9 +/- 6.4; p = 0.55) or subjective (72.3 +/- 12.6 vs. 74.4 +/- 13.6; p = 0.15) QOL between students in mainstream and special schools. CONCLUSIONS: With national health care and educational coverage, the QOL of adolescents with PD in Taiwan do not seem to be affected by the disabilities, regardless of whether they are in mainstream or special schools. However, the negative effect of PD on QOL becomes a concern with increasing age; females with PD also appear to have a lower subjective QOL in health and emotion.

The effect of hydroxyurea in spinal muscular atrophy cells and patients.

BACKGROUND: Spinal muscular atrophy (SMA) is a degenerative motor neuron disease caused by homozygous mutations of the survival motor neuron 1 (SMN1) gene. Effective treatment for SMA is unavailable at present. The aim of this study was to investigate the effect of hydroxyurea (HU) in SMA cells and patients. MATERIALS AND METHODS: Fifteen SMA lymphoid and three fibroblast cell lines, 2 from SMA patients and 1 control, were treated with HU at different concentrations, and 33 patients (types II, III) randomized into three groups on different HU dosage, 20, 30, 40 mg/kg/day, were treated for 8 weeks and followed up for another drug-free 8 weeks. The effect of HU on SMN2 gene expression and clinical manifestations was evaluated. RESULTS: After treatment, in vitro, full-length mRNA level and gems number increased significantly, and hnRNP A1 protein decreased. In vivo, there were slight increases in muscle strength scores at 4 weeks and full-length SMN mRNA at 8 weeks in 30 mg/kg/day subgroup. CONCLUSIONS: Treating with HU enhanced SMN2 gene expression in SMA cells and showed slight trend towards improvement in some clinical outcome measures in SMA patients which suggests HU may be safe to use in SMA patients but larger randomized, placebo-controlled, double-blind trials are needed to further investigate its efficacy.

Self-perceived quality of life for adolescents with physical disabilities - a preliminary study.

The purpose of this study was to explore the effect of physical disabilities (PD) on the quality of life (QoL) of adolescents aged from 10 to 18 years. Sixty-three adolescents with PD (aged 14.9 +/- 2.4 years) from primary (5th grade or above) to high schools in Kaohsiung City volunteered to participate in this research; 282 children without disability (aged 13.8 +/- 2.3 years) attending schools in the same geographical region were recruited as controls. The Student Version of the Comprehensive Quality of Life Scale was used in this study. This is a multidimensional self-report, global measure of subjective and objective QoL. Multivariate analysis of variance revealed that the two groups were significantly different in objective QoL (F = 11.53, p < 0.001). Material wellbeing was substantially lower in the PD group when compared to the control group. In contrast, domains such as productivity, safety, and emotion were higher in the PD group. Among the subjective scales, the PD group showed higher productivity and better emotion when compared to the control group. No significant correlation was observed between objective and subjective overall QoL scores (r = 0.20, p = 0.12) in the PD group. These findings showed that subjects with PD in regular schools demonstrated different patterns in objective and subjective QoL when compared to those without PD. Both subjective and objective domains are important when measuring QoL of adolescents with PD.

Joint range of motion limitations in children and young adults with spinal muscular atrophy.

OBJECTIVES: To elicit descriptive data about limited joint range of motion (ROM) in subjects with type II or III spinal muscular atrophy (SMA) and to examine the relation between the number of motions with limited range and both age and functional ability. DESIGN: Descriptive cross-sectional study. SETTING: Neurologic pediatric outpatient clinic at a hospital in Taiwan. PARTICIPANTS: Twenty-seven subjects with SMA type II (mean age, 9.8+/-6.5y) and 17 with SMA type III (mean age, 12.2+/-8.7y). Intervention Measurement with transparent goniometers of joint ROM bilaterally of the shoulder, elbow, wrist, hip, knee, and ankle. MAIN OUTCOME MEASURES: The proportion of participants with each ROM limitation compared with all participants with the same SMA type, age distribution of the participants with each ROM limitation, mean range loss of each motion limitation, and the contracture index (risk index of joint contracture). RESULTS: Eighty-nine percent of the participants with SMA type II experienced knee extension limitation. Approximately 50% of the participants with both types of SMA had ankle dorsiflexion limitation. The motions of knee and hip extension and ankle dorsiflexion also had a relatively high contracture index. The number of motions with limited range positively correlated ( P <.001) with age and upper-extremity functional grade (the higher the functional grade, the poorer the functional ability) for SMA type II. CONCLUSIONS: We found varying degrees of joint ROM limitation. Certain motions were noted to be high risks for the development of contractures. This risk was higher mostly in younger children.

Promoting balance and jumping skills in children with Down syndrome.

The purpose of this study was to investigate the changes in balance and qualitative and quantitative jumping performances by 20 children with Down syndrome (3 to 6 years) on jumping lessons. 30 typical children ages 3 to 6 years were recruited as a comparison group. Before the jumping lesson, a pretest was given subjects for balance and jumping skill measures based on the Motor Proficiency and Motor Skill Inventory, respectively. Subjects with Down syndrome received 3 sessions on jumping per week for 6 weeks but not the typical children. Then, a posttest was administered to all subjects. Analysis of covariance showed the pre- and posttest differences on scores for floor walk, beam walk, and horizontal and vertical jumping by subjects with Down syndrome were significantly greater than those for the typical children.