Lower limb functioning and its impact on quality of life in ambulatory children with cerebral palsy

BackgroundChildren with cerebral palsy (CP) are confronted on a daily basis with their motor problems affecting gait, which might impact on their quality of life (QOL).AimThe goal of this study was to evaluate the impact of gross motor and gait function on QOL in ambulatory children with CP attending regular school.MethodsA condition-specific questionnaire (CP QOL-Child), including a parent/proxy and child self-report, was used to assess the relation between patient characteristics, lower limb impairments and functioning, and the different aspects of the child's QOL. Data on therapy management, lower limb impairments, and gross motor and gait function was collected for 81 children with CP (10.5 ± 3.0 years). CP QOL-Child questionnaires were completed by a parent/proxy for all 81 children and by 39 of the children over 9 years.ResultsThe mean self-reported QOL score (78.2 ± 9.6) was significantly higher than the mean parent/proxy reported score (73.9 ± 10.2). Lower limb spasticity and higher frequency of BTX-A injections correlated with worse scores for the pain and impact of disability domain. Results further showed the adverse impact of the severity of gait pathology on QOL perception for the parents/proxy and the child self-report. Gait speed was an important factor for the parents/proxy, though correlated less with self-perceived QOL for the children.ConclusionsChildren and parents identified similar factors that adversely affect QOL, whereby the amount of gait pathology was shown to play a crucial role. Only gait speed seemed to be of higher importance for the parents compared to the children.     

European study of frequency of participation of adolescents with and without cerebral palsy

Children with cerebral palsy participate less in everyday activities than children in the general populations. During adolescence, rapid physical and psychological changes occur which may be more difficult for adolescents with impairments.Within the European SPARCLE project we measured frequency of participation of adolescents with cerebral palsy by administering the Questionnaire of Young People's Participation to 667 adolescents with cerebral palsy or their parents from nine European regions and to 4666 adolescents from the corresponding general populations. Domains and single items were analysed using respectively linear and logistic regression.Adolescents with cerebral palsy spent less time with friends and had less autonomy in their daily life than adolescents in the general populations. Adolescents with cerebral palsy participated much less in sport but played electronic games at least as often as adolescents in the general populations. Severity of motor and intellectual impairment had a significant impact on frequency of participation, the more severely impaired being more disadvantaged. Adolescents with an only slight impairment participated in some domains as often as adolescents in the general populations. Regional variation existed. For example adolescents with cerebral palsy in central Italy were most disadvantaged according to decisional autonomy, while adolescents with cerebral palsy in east Denmark and northern England played sports as often as their general populations.Participation is an important health outcome. Personal and environmental predictors of participation of adolescents with cerebral palsy need to be identified in order to design interventions directed to such predictors; and in order to inform the content of services.     

Effect of upper limb botulinum toxin-A therapy on health-related quality of life in children with hemiplegic cerebral palsy

Aim:   Currently, the use of upper limb botulinum toxin-A (UL BTX-A) is based on evidence of functional efficacy without supporting evidence of positive change in health-related quality of life (HRQOL). While function may improve, this cannot be directly correlated with an improvement in HRQOL. Most paediatric studies use caregiver/parent proxy reports. The inclusion of child self-reports is increasing as poor correlation with proxy reports is being demonstrated. This paper aims to study the effect of UL BTX-A therapy on HRQOL in children with hemiplegic cerebral palsy (CP).
Method:   Design: Pilot prospective randomised trial. Participants: 22 children with hemiplegic CP aged 7 years 0 month−13 years 11 months (12 treatment, 10 control). Treatment: One series BTX-A injections into UL. HRQOL assessed at baseline, and 1, 3 and 6 months post-injection by completion of Pediatric Quality of Life (PedsQL) 4.0 Generic Core Scales and PedsQL 3.0 CP Module. Outcome: 1. Change in PedsQL scores. 2. Concordance between child self-report and parent proxy-report scores.
Results:   No statistically significant difference between treatment and control groups was observed for any domain of HRQOL. Intraclass concordance was good for the PedsQL CP Module Daily Activities, and Speech and Communication scores ( P  = 0.0005).
Conclusion:   This pilot work adds to the emerging evidence that UL BTX-A therapy has no statistically significant effect on the HRQOL of children with hemiplegic CP. With the increasing use of this therapy in children with CP, further research across the broader CP population is needed to identify whether this therapy is indicated in other target populations. Both child and parent proxy reports should be collected when assessing HRQOL in this population.     

Longitudinal changes in bone density in children and adolescents with moderate to severe cerebral palsy

OBJECTIVE: To assess the natural history of "growth" in bone mineral density (BMD) in children and adolescents with moderate to severe cerebral palsy (CP). STUDY DESIGN: A prospective, longitudinal, observational study of BMD in 69 subjects with moderate to severe spastic CP ages 2.0 to 17.7 years. Fifty-five subjects were observed for more than 2 years and 40 subjects for more than 3 years. Each evaluation also included assessments of growth, nutritional status, Tanner stage, general health, and various clinical features of CP. RESULTS: Lower BMD z-scores at the initial evaluation were associated with greater severity of CP as judged by gross motor function and feeding difficulty, and with poorer growth and nutrition as judged by weight z-scores. BMD increased an average of 2% to 5%/y in the distal femur and lumbar spine, but ranged widely from +42%/y to -31%. In spite of increases in BMD, distal femur BMD z-scores decrease with age in this population. CONCLUSIONS: Children with severe CP develop over the course of their lives clinically significant osteopenia. Unlike elderly adults, this is not primarily from true losses in bone mineral, but from a rate of growth in bone mineral that is diminished relative to healthy children. The efficacy of interventions to increase BMD can truly be assessed only with a clear understanding of the expected changes in BMD without intervention.     

The contribution of developmental surveillance to early detection of cerebral palsy

Abstract A retrospective study of the contribution of examinations at Child Health Centres (CHC) for early detection of cerebral palsy (CP) was performed in 23 924 children, born in 1986-90, in the south-western part of Stockholm County. The outcome assessed was age of referral to a habilitation unit. A total of 66 children with CP (2.76/1000) were identified through the register at the responsible habilitation unit. The age of referral was, median 8 months, mean 12.4 months and range 0.5–54 months. A peak of referrals at 8–9 months was produced by CHC checks at 6 months of age. No other examination at the CHC resulted in a significant number of referrals. Nineteen records from CHC concerning moderate and severe cases were reviewed. Out of all examinations with aberrant findings only 11/20 resulted in referrals or further examinations. No child with moderate or severe CP was referred due to developmental screening test findings only.     

Diverticulosis of the urinary bladder in a child with cerebral palsy

Urological abnormalities are not usually encountered in children with cerebral palsy (CP), although there may be functional bladder problems. We report a case of bladder diverticulosis in a 2-year old female with CP who presented with a suprapubic mass. Bladder diverticulosis itself is a very rare abnormality.     

Uroflowmetry in the management of lower urinary tract symptoms of children and adolescents with cerebral palsy

ObjectiveTo evaluate uroflow measurements in the initial management of lower urinary tract dysfunction in children and adolescents with cerebral palsy.Materials and methodsA total of 54 patients was enrolled in this study. All patients reported their urinary symptoms and underwent a physical examination, renal and urinary tract ultrasonography, and uroflow assessment.ResultsTwenty-three patients were female. Mean age was 9 years and 6 months (SD: 2 years and 10 months), with a range of 5–18 years. Twenty-eight of the patients (51.8%) were symptomatic. Urgency (42.6%), urge incontinence (40.7%), and enuresis (16.7%) were the most frequently observed symptoms. No association was found between gender, ambulatory status, or distribution of the paralysis and uroflow parameters. Symptomatic patients presented a statistically lower maximum flow (Q_max) than asymptomatic patients (17.2 ± 7.8 ml/s vs 22.6 ± 7.5 ml/s, p = 0.013, respectively). Normal bell-shaped curves were observed more frequently in asymptomatic patients, while abnormal curves were observed more frequently in symptomatic patients (p = 0.022).ConclusionsGender, ambulatory status, and the distribution of the paralysis do not affect Q_max rate or flow pattern. Symptomatic patients present lower Q_max and may also have an abnormal uroflow curve. Uroflowmetry may be useful in the initial urological evaluation.     

以健康测量量表评价癫及哮喘患儿生活质量的对照研究

摘要 目的 以健康测量量表（SF 36）评估癫痫及哮喘患儿的生活质量。方法 采用SF-36量表对2007年6至12月在复旦大学附属儿科医院神经专科、呼吸专科及儿童保健门诊就诊的癫痫患儿、哮喘患儿和健康儿童进行生活质量评定。结果 研究期间85例癫痫患儿（癫痫组）、81例哮喘患儿（哮喘组）和87名健康儿童（正常对照组）进入分析。3组儿童的年龄、性别和受教育程度相匹配。①疾病及治疗情况：癫痫组和哮喘组服用1种药物者分别为70/85例和59/81例,癫痫组63/85例和哮喘组56/81例患病后规则服药,两组在服药种类和依从性上差异无统计学意义（P均＞0.05）;癫痫组36/85例至本次调查前至少有1年癫痫未发作;哮喘组29/81例最大呼气峰流速监测持续保持个人最佳值的80％以上。②生活质量评价：癫痫组的生活质量总分和8个分项的终得分与正常对照组差异均有统计学意义（P<0.01）;哮喘组的生活质量总分及生理功能、身体疼痛、总体健康和活力4个分项的终得分与正常对照组差异均有统计学意义（P<0.01）;无论疾病控制与否,癫痫组生活质量均较哮喘组差;癫痫组情感功能及精神健康状况明显差于哮喘组,其心理障碍不随疾病控制而明显改善。结论 ①癫痫和哮喘患儿生活质量较健康儿童明显下降;②癫痫患儿较哮喘患儿生活质量损害更为明显;③SF-36量表可作为评价疾病控制程度的手段,辅助评价疾病治疗的疗效;④癫痫 患儿的心理障碍应引起临床高度关注。     

Quality of life and functional outcome in early school-aged children after neonatal stroke: A prospective cohort study

BackgroundQuality of life (QoL) is recognized internationally as an efficient tool for evaluating health interventions. To our knowledge, QoL has not been specifically assessed in children after neonatal arterial ischemic stroke (AIS).AimTo study the QoL of early school-aged children who suffered from neonatal AIS, and QoL correlation to functional outcome.MethodWe conducted a multicenter prospective cohort study as part of a larger study in full-term newborns with symptomatic AIS. Participating families were sent anonymous QoL questionnaires (QUALIN). Functional outcome was measured using the Wee-FIM scale. Healthy controls in the same age range were recruited in public schools. Their primary caregivers filled in the QUALIN questionnaires anonymously. We used Student's t-test and a rank test to compare patients and controls' QoL and functional outcomes.Results84 children with neonatal AIS were included. The control group was composed of 74 children, of which ten were later excluded due to chronic conditions. Mean ages and QUALIN median scores did not differ between patients and controls. Median Wee-FIM scores were lower in hemiplegic children than in non-hemiplegic ones (p < 0.001). QoL scores did not seem correlated to functional outcome.InterpretationThose results could support the presence of a “disability paradox” in young children following neonatal AIS.     

Identification of feeding risk factors for impaired nutrition status in paediatric patients with cerebral palsy

Aim: To assess the nutrition status of children with CP, applying WHO growth standards, to indentify feeding risk factors and to evaluate their impact on the growth of children with CP. Methods: In 42 paediatric patients (mean age 8.00 ± 4.00 years), anthropometry and food intake records were assessed. z ‐scores were calculated using WHO Anthro software. Intake to requirements ratio (I/R) was calculated, and patients were classified according to their feeding ability (PFA). Overall diet quality was assessed using the Diet Quality Index International (DQI‐I). Results: Based on WAz, 15 patients (38.1%) were undernourished. No association was found between I/R ratio and BMI z‐score, while PFA and DQI‐I displayed a significant correlation to both (p < 0.05). Diet Quality Index International was also correlated with macronutrient distribution (p < 0.05). Patients with CP were undernourished in a considerable proportion. Malnutrition in patients with CP is not associated with the intake of estimated energy requirements. Among the other feeding risk factors studied, PFA and DQI‐I represented important parameters associated with malnutrition. Conclusion: WHO z‐scores represent accurate parameters for the assessment of malnutrition in patients with CP. Together with anthropometry and PFA evaluation, the use of the DQI‐I would add prognostic value to both the initial growth assessment and the patients’ growth monitoring.     

Communication ability in cerebral palsy: A study from the CP register of western Sweden

BackgroundCommunication is often impaired in cerebral palsy (CP). Tools are needed to describe this complex function, in order to provide effective support.AimTo study communication ability and the relationship between the Communication Function Classification System (CFCS) and CP subtype, gross motor function, manual ability, cognitive function and neuroimaging findings in the CP register of western Sweden.MethodsSixty-eight children (29 girls), 14 with unilateral spastic CP, 35 with bilateral spastic CP and 19 with dyskinetic CP, participated. The CFCS, Gross Motor Function Classification System (GMFCS) and Manual Ability Classification System (MACS) levels, cognitive impairment and neuroimaging findings were recorded.ResultsHalf the children used speech, 32% used communication boards/books and 16% relied on body movements, eye gaze and sounds. Twenty-eight per cent were at the most functional CFCS level I, 13% at level II, 21% at level III, 10% at level IV and 28% at level V. CFCS levels I–II were found in 71% of children with unilateral spastic CP, 46% in bilateral spastic CP and 11% in dyskinetic CP (p = 0.03). CFCS correlated with the GMFCS, MACS and cognitive function (p < 0.01). Periventricular lesions were associated with speech and more functional CFCS levels, while cortical/subcortical and basal ganglia lesions were associated with the absence of speech and less functional CFCS levels (p < 0.01).ConclusionCommunication function profiles in CP can be derived from the CFCS, which correlates to gross and fine motor and cognitive function. Good communication ability is associated with lesions acquired early, rather than late, in the third trimester.     

Health related quality of life and manual ability 5 years after neonatal ischemic stroke

AimTo investigate health-related quality of life (HRQOL) and manual ability five years after neonatal arterial ischemic stroke (NAIS).MethodsData was prospectively obtained by the Swiss Neuropaediatric Stroke Registry between 2000 and 2010. Two years after NAIS, cognitive and motor outcomes was assessed using the Bayley Scales of Infant Development (BSID-II). After 5 years, HRQOL was assessed with the KIDSCREEN-27 and manual ability with the ABILHAND-Kids. Manual ability and HRQOL were compared between children with and without cerebral palsy (CP) and HRQOL was correlated with manual ability.ResultsSeventy-four patients were examined at the age of 2 years, at the age of 5 years 61 patients underwent a follow-up examination. Two years after NAIS, 29 children (39.1%) were diagnosed with CP. HRQOL 5 years after NAIS was comparable to normative values. Children with CP had a significantly lower HRQOL-index (p = 0.013) and lower scores in the subscale psychological well-being (p = 0.012) and social support & peers (p = 0.048). The ABILHAND-Kids measure was significantly lower in children with CP compared to children without CP (p < 0.001). Manual ability correlated significantly with HRQOL.ConclusionFive years after NAIS, HRQOL is comparable to that of typically developing peers, but reduced in children with CP. Poorer manual ability is associated with lower HRQOL. Interventions improving hand function might influence HRQOL and should be considered early on.     

The relationship of dystonia and choreoathetosis with activity,participation and quality of life in children and youth with dyskinetic cerebral palsy

Aim

To relate dystonia and choreoathetosis with activity, participation and quality of life (QOL) in children and youth with dyskinetic Cerebral Palsy (CP).