Gross Motor Function Classification System IV-V Research Articles

Physical activity, quality of life and parenting stress in children with cerebral palsy

Children with cerebral palsy (CP) are less physically active than their typically developing peers. The effects of decreased physical activity on children's quality of life (QOL) or caregiver's lives are not well understood. This study aimed to investigate the association between physical activity, QOL, and parenting stress in children with CP. A prospective cross-sectional study was done in children with CP. Daily physical activity was measured over 7 days using an accelerometer (ActiGraph™). Caregivers completed the Child Health Questionnaire - Parent Form 50 and Parenting Stress Index - Short Form. Multiple regression analysis was used. In total, data from 65 children with Gross Motor Function Classification System (GMFCS) levels I-V, aged 4-13 years, were analyzed. Non-ambulatory (GMFCS IV-V) children had significantly lower activity counts and moderate to vigorous physical activity (MVPA) in comparison with ambulatory (GMFCS I-II) and marginally ambulatory (GMFCS III) children. The ambulatory group had better physical QOL than the non-ambulatory group. Caregivers of the non-ambulatory or marginally ambulatory children with CP reported higher parenting stress levels than those of the ambulatory group. Time spent in MVPA and activity counts were positively associated with physical QOL in children with CP. Low activity counts and low amounts of MVPA of children with CP were significant predictors of high parenting stress. Physical activity in children with CP is associated with children's QOL and parenting stress. These results suggest that clinicians should conduct physical activity assessment and counseling to promote physical activity.

Sleep: How is it affected in patients with DMD and their mothers?

To investigate whether children with Duchenne muscular dystrophy (DMD) have sleep disorders, and the sleep quality and daytime sleepiness of mothers who are primary caregivers. Clinical data and gross motor functional status of 24 patients with DMD were measured using the gross motor function classification system (GMFCS). Sleep Disturbance Scale for Children scores were evaluated to determine their sleep features. The Pittsburgh Sleep Quality Index scores were used to measure sleep quality, and Epworth Sleepiness Scale scores were used to measure daytime sleepiness of 24 mothers. Sleep disturbances were observed in 62.5% (n = 15) of patients, 41.6% (n = 5) of those who were GMFCS I-II-III and in 83.3% (n = 10) who were IV-V level. A disturbed sleep pattern was observed in 3 (33.3%) of 9 patients who were younger than 10 and in 12 (80.0%) of 15 patients who were older than 10. Of mothers, 54.2% had low sleep quality, which was present in 83.3% of mothers with GMFCS IV-V children and 54.2% of those with GMFCS I-II-III children. Ten (41.6%) mothers had increased daytime sleepiness, which was present in 66.6% of mothers with GMFCS IV-V children and 16.6% of mothers with GMFCS I-II-III children. Sleep disturbances increase in parallel to loss of gross motor functions in patients with DMD, which has had negative impact on the sleep quality and daytime sleepiness of the caregiver.

Ankle contractures are frequent among children with cerebral palsy and associated with lower gross motor function and degree of spasticity.

To estimate yearly prevalence of ankle contractures among children with cerebral palsy (CP). Moreover, to investigate whether age, gross motor function or spasticity are associated with ankle contracture. We examined yearly prevalence of ankle contractures among 933 children based on data from a national clinical quality database from 2012 to 2019. We used the Gross Motor Function Classification System (GMFCS) and the Modified Ashworth Scale (MAS) to assess gross motor function and spasticity in the plantar flexors. Ankle contracture was defined as dorsiflexion with an extended knee equal to or below 0 degrees. Associations between age, GMFCS, spasticity and ankle contractures were analysed using multivariable regression and presented as odds ratios (OR) with 95% confidence intervals (95%CI). The prevalence of ankle contracture was 32% and did not change with calendar year. GMFCS IV-V compared to I-III (40.6% vs. 28.9%, OR=1.5 (95%CI: 1.07-2.11) and MAS 2-4 compared to 0 (44.6% vs. 24.4%, OR=2.5 (95%CI: 1.59-3.91) were associated with a higher prevalence of ankle contracture. Age was not associated with ankle contracture. Ankle contractures are frequent among children with CP. Lower gross motor function and severe spasticity were associated with ankle contracture.

Prognostic value of cranial ultrasonography in comparison with magnetic resonance imaging in children with cerebral palsy: a population-based study.

SUMMARYThe aim of this population-based study was to evaluate the characteristics of cerebral palsy (CP) in relation to the predominant pattern of the Magnetic Resonance Imaging Classification System (MRICS) that was analogously applied to the neonatal/early infant cranial ultrasound (CUS). The study included children born during the 2004-2007 period from the Croatian part (C28 RCP-HR) of the Surveillance of Cerebral Palsy in Europe (SCPE) CP register. Motor functions, accompanying impairments and brain MRI were evaluated in 227 children, 185 of which also had CUS. Concerning CP types, 56% of children had bilateral spastic, 34% unilateral spastic, 9% dyskinetic and 1% ataxic CP type. Gross Motor Function Classification System (GMFCS) revealed that 62.05% had mild (GMFCS I-III) and 37.85% had severe motor impairment (GMFCS IV-V). CUS showed white matter injury in 60%, gray matter injury in 12%, maldevelopments in 8%, miscellaneous changes in 14%, while 6% were normal; MRI showed significant agreement (κ=0.675, p<0.001). Neuroimaging findings of maldevelopments and predominant gray matter injury were associated with more severe CP, but 7% of children with CP had normal MRI. As we found very good agreement between CUS and MRI findings, CUS is recommended in children at an increased risk of CP if MRI is not available.

Clinical features of 54 cases of leukoencephalopathy with vanishing white matter disease in children

Objective: To summarize the clinical features of leukoencephalopathy with vanishing white matter disease (VWM) in children. Methods: A retrospective cohort study was performed on 54 genetically diagnosed VWM patients in Peking University First Hospital from January 2007 to March 2019. Paper registration form and electronic medical record system were used to collect the data,and the children were divided into five groups according to the age of disease onset:<1 year, 1-<2 years, 2-<4 years, 4-<8 years and 8-<18 years respectively. The progression of motor function, episodic aggravation, epileptic seizures, survival time, brain magnetic resonance imaging (MRI) and genotype features were analyzed and compared. Non-parametric test, χ(2) test or Fisher's exact test were used for comparison among groups; Kaplan-Meier survival curve was adopted to delineate the survival status of the children. Results: Fifty-four VWM patients were included in the study, including 34 males and 20 females.The age of disease onset was 2 years and 8 months (ranged from 6 months to 9 years and 7 months). Onset age was less than 1 year in 5 cases; onset age was 1-<2 years in 12 cases; onset age was 2-<4 years in 25 cases; onset age was 4-<8 years, in 10 cases; onset age was 8-<18 years in 2 cases; 94% (51/54) of patients had complaint of motor regression at the first visit; 87% (47/54) of patients suffered from episodic aggravation. Episodic seizures occurred in 43% (23/54) patients. In survivors with disease durations of 1-3 years, in 38% (9/24) patients the disease was classified as grades Ⅳ-Ⅴ by gross motor function classification system (GMFCS). For the onset age 1-<2 years group, 1 patient was classified as GMFCS Ⅳ among 3 survivors with disease durations of 1-3 years. As for the 2-<4 years group, 6 patients were classified as GMFCS Ⅳ-Ⅴ among 15 patients with disease durations of 1-3 years, whereas 1 patient was classified as GMFCS Ⅳ-Ⅴ among 4 patients with disease durations of 1-3 years in the 4-<8 years group. Lesions, liquefaction and diffusion restriction in brain MRI were compared among different groups, and it was revealed that the earlier the age of disease onset was, the more likely the subcortical white matter (frontal lobe P<0.01,temporal and parieto-occipital lobe both P=0.002), internal capsule (anterior limb P<0.01, posterior limb P=0.00) and brain stem (midbrain P=0.001, pons P<0.01) were to be involved. In addition, internal capsule (anterior limb P=0.002, posterior limb P=0.005) and brain stem (midbrain P=0.001, pons P=0.003) showed more diffuse restricted diffusion. Moreover, the subcortical white matter (frontal and parieto-occipital lobe both P<0.01, temporal lobe P=0.005) showed earlier rarefaction. The 1-year and 2-year survival rates of the overall patients were 81% and 75% respectively, while the 15-year survival rate was 45%. EIF2B5 gene variation was the most common, which accounts for 43% (23/54), followed by EIF2B3 (22%, 12/54). Conclusions: The majority of VWM patients complained of motor regression at the first visit, episodic aggravation and epileptic seizures are common in the course. Earlier age at onset is associated with more rapid clinical progression, shorter survival time as well as more extensive lesions, liquefaction and diffusion restriction in brain MRI. The most common variant gene is EIF2B5, followed by EIF2B3.

Use of outcome measures in children with severe cerebral palsy: A survey of U.K. physiotherapists.

To investigate the use of outcome measures for children with cerebral palsy (CP) by paediatric physiotherapists (PTs) who are based in the United Kingdom, as limited research exists regarding their use in this population, and to explore therapists' use of measures within different Gross Motor Function Classification System (GMFCS) levels and for different types of CP. A six-item online survey was advertised through two paediatric therapy special interest groups inviting physiotherapists to participate. Descriptive statistics (range, frequencies, and percentages) were used to analyse survey data. Two hundred and seven physiotherapists returned completed survey questionnaires. One hundred and seventy-six (85%) therapists reported using a wide variety of outcome measures (57). Eighty-one per cent of therapists' responses for having "most difficulty" in identifying responsive outcome measures were for children with impaired mobility at GMFCS Levels IV-V and children with more significant body involvement (76%), for example, spastic quadriplegia or dyskinesia. Eighty-six per cent of therapists' responses for having "greatest ease" of identifying responsive outcome measures were for children within GMFCS Levels I-III and for children with less significant body involvement (72%), for example, hemiplegia. The variety of outcome measures used by therapists with children within GMFCS IV-V was less (16). The majority (85%) of the PTs surveyed used outcome measures with children with CP, but 81% perceived a difficulty in identifying responsive measures for children with more severe impairments who are classified as GMFCS IV-V. The reasons for this perceived difficulty warrant investigation and may suggest a need for training regarding relevant measures and/or a need to develop new measures for this group of children.

Development of disorder-specific normative data for growth in children with cerebral palsy.

The purpose of this study was to create growth-percentiles for Caucasian children with cerebral palsy (CP). The studied parameters were height and age. In a retrospective analysis, we converted measurements collected in our center to create disorder-specific percentiles of normative data. Patients were stratified due to sex (male and female) and to mobility levels using the gross motor function classification system (GMFCS) (A = walking; GMFCS I-III, B = non walking; GMFCS IV-V) into four groups. In total, 2363 measurements in patients 0-18years were collected. The mean age for group "Am" was 6.8years (n = 862), group "Bm" 7.6years (n = 563), group "Af" 7.7years (n = 600), and group "Bf" 8.2years (n = 366). The created percentiles for all groups were below the reference percentiles for healthy Caucasian children (KiGGS). The median curve for children with GMFCS levels I-III is slightly above the 3rd percentile, whereas the 50th percentile for GMFCS levels IV-V is mostly below the 3rd KiGGS centile.Conclusion: In conclusion, children with cerebral palsy are smaller than healthy children. The difference between 50th percentile of CP patients compared to healthy children supports the need for the use of disorder-specific growth charts. Those charts can help clinicians differentiate growth disorders in patients with CP. What is Known: • Children with cerebral palsy are shorter than healthy children and height is influenced by level of ambulation. • Currently, only reference percentiles of American children with mixed ethical backgrounds are available to evaluate growth. What is New: • This paper presents disorder-specific reference percentiles for longitudinal growth of Caucasian children with cerebral palsy depending on motor function. • These percentiles allow to asses longitudinal growth in children with cerebral palsy to detect other additional diseases impairing growth.

Noncommunicable disease and multimorbidity in young adults with cerebral palsy.

PurposeIndividuals with cerebral palsy (CP) are at increased risk for frailty and chronic disease due to factors experienced throughout the lifespan, such as excessive sedentary behaviors and malnutrition. However, little is known about noncommunicable diseases (NCDs) and multimorbidity profiles in young adults with CP. The study objective was to compare NCD and multimorbidity profiles between young adults with and without CP.MethodsA clinic-based sample of adults (18–30 years) with (n=452) and without (n=448) CP was examined at the University of Michigan Medical Center. The prevalence and predictors of 13 NCDs were evaluated, including existing diagnoses or historical record of musculoskeletal, cardiometabolic, and pulmonary morbidities. The level of motor impairment was determined by the Gross Motor Function Classification System (GMFCS) and stratified by less vs more severe motor impairment (GMFCS I–III vs IV–V). Logistic regression was used to determine the odds of NCD morbidity and multimorbidity in adults with CP compared to adults without CP, and for GMFCS IV–V compared to GMFCS I–III in those with CP, after adjusting for age, sex, body mass index, and smoking.ResultsAdults with CP had a higher prevalence of osteopenia, osteoporosis, hypertension, myocardial infarction, hyperlipidemia, asthma, and multimorbidity compared to adults without CP, and higher odds of musculoskeletal (odds ratio [OR]: 6.97) and cardiometabolic morbidity (OR: 1.98), and multimorbidity (OR: 2.67). Adults with CP with GMFCS levels IV–V had a higher prevalence of osteopenia/osteoporosis, osteoarthritis, hypertension, other cardiovascular conditions, pulmonary embolism, and multimorbidity, and higher odds of musculoskeletal (OR: 3.41), cardiometabolic (OR: 2.05), pulmonary morbidity (OR: 1.42), and multimorbidity (OR: 3.45) compared to GMFCS I–III.ConclusionYoung adults with CP have a higher prevalence of chronic NCDs and multimorbidity compared to young adults without CP, which is pronounced in those with more severe motor impairment. These findings reiterate the importance of early screening for prevention of NCDs in CP.

Intrathecal Baclofen Therapy Prior to Spinal Fusion for Patients With Gross Motor Function Classification System IV-V Cerebral Palsy.

Patients with Gross Motor Function Classification System (GMFCS) IV-V cerebral palsy (CP) have significant spasticity and frequently develop scoliosis. Intrathecal baclofen (ITB) pumps are effective in managing spasticity. The effect of ITB therapy on the postoperative course following spinal fusion in patients with GMFCS IV-V CP has not been described. This study sought to compare postoperative recovery, including complications, in patients using ITB therapy with those with no ITB therapy. Evaluate the effect of ITB on the postoperative recovery for patients with GMFCS IV-V CP who undergo spinal fusion for scoliosis. Health records for patients with GMFCS IV-V CP who underwent a spinal fusion for scoliosis at a major quaternary-care children's hospital from January 2009 to October 2015 were reviewed and relevant data were abstracted. Descriptive statistics and regression models were used to compare patients. Sixty-nine patients were included-19 ITB therapy and 50 no ITB therapy. Demographic and operative characteristics were similar across groups. The mean length of stay for patients in the ITB therapy group was 11.2 days and 14.3 days for the no ITB therapy group, with no difference between groups (p = .12). Pain scores in both groups decreased at the same rate, with scores in the ITB therapy group averaging one-half point lower (p = .32). The average amount of morphine equivalents (p = .71) and benzodiazepine equivalents (p = .53) used were similar between groups. Complication rates were significantly different between groups. Four (21%) of the ITB therapy patients had 1 or more complications whereas 28 (56%, p = .01) in the no ITB therapy group had 1 or more complications. The average number of complications per patient in the ITB therapy group was 0.3 (SD: 0.075, range: 0-3) and the no ITB therapy group was 1.1 (SD: 1.1, range: 0-6, p = .01). There was no significant difference in length of stay, pain scores, or pain/spasticity medication use between groups after spinal fusion, but there was a significantly lower incidence of complications in the ITB therapy group.

Treatment with Botulinum toxin A in a total population of children with cerebral palsy - a retrospective cohort registry study

BackgroundBotulinum toxin A (BTX-A) has been used to reduce spasticity in children with cerebral palsy (CP) for decades. The purpose of this study was to analyze to what extent BTX-A treatment was used to treat spasticity in a total population of children with CP. We investigated 1) the use of BTX-A in relation to age, sex, and Gross Motor Function Classification System (GMFCS) level, 2) the most common muscle groups treated with BTX-A in relation to the same variables, and 3) changes in the proportions of children treated with BTX-A between two time points (2010 and 2015).MethodsThe study was based on data from CPUP, a combined Swedish follow-up program and national healthcare registry, comprising >95% of all children with CP in Sweden. The participants (N = 3028) were born in 2000 or later. Potential BTX-A treatment and treated muscle groups were included from all CPUP assessments recorded in the registry in 2014–2015. In Aim 3, BTX-A administration in 3–5 year-olds at two time points was assessed. Crosstabs and 95% confidence intervals (CIs) for binominal proportions were calculated and logistic regression was used to regress age, sex, and GMFCS level on BTX-A treatment. Muscle groups treated with BTX-A were assessed using crosstabs and 95% CIs. Proportional change in BTX-A treatment over a 5-year period was analyzed using chi-square.ResultsWe included 3028 children (57% boys; median age 7 years) of whom 26% received BTX-A. Significantly more boys (28%) than girls (23%) received BTX-A (OR = 1.25, [95% CI 1.05–1.48]). Significant differences were found for age and GMFCS levels; 4–6 year-olds and those at GMFCS III-IV were more likely to receive BTX-A. BTX-A treatment in the gastrocnemius muscle was most common in the 4–6 year-olds and at GMFCS I-III, whereas treatment of the hamstring and adductor muscles was more common in older children and at GMFCS IV-V. No significant change in the proportion of BTX-A administered in 2010 and 2015 was demonstrated.ConclusionsBTX-A treatment differed based on age, sex, and GMFCS level. Proportion of BTX-A treatment in Sweden has remained stable during the past five years.

Multimorbidity in Middle-Aged Adults with Cerebral Palsy

ObjectiveIndividuals with cerebral palsy have less lean body mass, greater relative adiposity, and lower fitness and physical activity participation, and yet the prevalence of age-related multimorbidity in this population has yet to be established. The study objective was to examine the prevalence of lifestyle-related chronic conditions and multimorbidity in a sample of middle-aged adults with cerebral palsy. MethodsA clinic-based sample of middle-aged adults with cerebral palsy was examined using Electronic Medical Records Search Engine software. Our cohort included 435 individuals aged 40 to 60 years, with an International Classification of Diseases, Clinical Modification, 9th and 10th Revisions Diagnosis Code for cerebral palsy. Prevalence of 12 chronic conditions was evaluated, including existing diagnoses or historical record of osteopenia/osteoporosis, myocardial infarction, stroke, coronary artery disease, impaired glucose tolerance/type 2 diabetes, other cardiovascular conditions, rheumatoid arthritis, osteoarthritis, asthma, emphysema, prehypertension/hypertension, and hyperlipidemia. Multivariate logistic models were used to estimate multimorbidity (ie, ≥2 chronic conditions), adjusting for age, sex, smoking status, obesity, and Gross Motor Function Classification System (GMFCS). ResultsThere were 137 unique multimorbidity combinations. Multimorbidity was significantly more prevalent among obese versus nonobese individuals for both GMFCS I-III (75.8% vs 53.6%) and GMFCS IV-V (79.0% vs 64.2%), but was also significantly higher in nonobese individuals with GMFCS IV-V (64.2%) compared with nonobese individuals with GMFCS I-III (53.6%). Both the obesity status (odds ratio, 2.20; 95% confidence interval, 1.32-2.79) and the GMFCS IV-V category (odds ratio, 1.81; 95% confidence interval, 1.32-3.68) were independently associated with multimorbidity. ConclusionsMiddle-aged adults with cerebral palsy have high estimates of multimorbidity; both obesity and higher GMFCS levels are independently associated with greater risk.

Reliability of radiologic measures of hip displacement in a cohort of preschool-aged children with cerebral palsy.

Radiologic hip surveillance is recommended for children with cerebral palsy (CP) at risk of hip displacement. Young children with abnormal proximal femoral geometry (Hilgenreiner epiphyseal angle, HEA) may be more likely to develop hip displacement, less likely to respond to nonsurgical intervention, and may benefit from earlier surgical referral. The reliability of radiographic measures of migration percentage (MP) in the immature pelvis of young children has been reported in smaller retrospective studies; HEA has not been examined in this population. This prospective study describes the reliability of MP and HEA in very young children with CP. Participants were entered from tertiary referral center CP clinics into a prospectively recruited population-based cohort for hip surveillance with pelvic radiography using standardized patient position, at 18, 24, 30, 36, and 48 months. All Gross Motor Function Classification System (GMFCS) levels were included. Two independent raters assessed radiographs for HEA and MP. The intraclass correlation coefficient (ICC) was computed as a measure of interrater and intrarater reliability. The correlation coefficient between HEA and femoral position was computed. Ninety-eight children less than 25 months (spasticity=83, 85%; GMFCS IV-V=38, 39%), and 114 children 25 to 48 months (spasticity=96, 85%; GMFCS IV-V=37, 32%) were included from 133 unique participants (spasticity=111, 84%; GMFCS IV-V=42, 32%). Of these 79 children were studied in both age groups. Overall interrater and intrarater reliability of MP was high [ICC=0.93; 95% confidence interval (CI), 0.91-0.95]; SEM was 3.9% (single) and 5.5% (sequential). Perfect concordance for classification of marked hip displacement (MP>30%) occurred in 217 cases (95.2%); nonweighted κ=0.80; 95% CI, 0.68-0.91. For HEA, overall reliability was high (ICC=0.89; 95% CI, 0.85-0.93); SEM=4.8% (single) and 6.7% (sequential). Correlation between changes in HEA and femoral abduction was poor (coefficient=-0.27, P=0.244). MP and HEA can be reliably applied to very young children with CP, with high reliability for both measures. Measured HEA values appear to be independent of patient position, and may reflect genuine changes in proximal femoral geometry. A longitudinal study should be performed to determine the relationship between HEA and later hip outcomes. Level I/II--testing and development of diagnostic tests.