Longitudinal community walking activity in Duchenne muscular dystrophy.

INTRODUCTION: Natural history studies for Duchenne muscular dystrophy (DMD) have not included measures of community ambulation. METHODS: Step activity (SA) monitors quantified community ambulation in 42 boys (ages 4-16 years) with DMD with serial enrollment up to 5 years by using a repeated-measures mixed model. Additionally, data were compared with 10-meter walk/run (10mWR) speed to determine validity and sensitivity. RESULTS: There were significant declines in average strides/day and percent strides at moderate, high and pediatric high rates as a function of age (P < 0.05). Significant correlations for 10mWR versus high and low stride rates were found at baseline (P < 0.05). SA outcomes were sensitive to change over 1 year, but the direction and parameter differed by age group (younger vs. older). Changes in strides/day and percentages of high frequency and low frequency strides correlated significantly with changes in 10mWR speed (P < 0.05). DISCUSSION: Community ambulation data provide valid and sensitive real-world measures that may inform clinical trials. Muscle Nerve 57: 401-406, 2018.

Bone density in premenopausal women and men under 50 years of age with cerebral palsy.

OBJECTIVE: To determine bone mineral density (BMD) z scores in adults with cerebral palsy (CP), an understudied population. DESIGN: Cross-sectional. SETTING: Medical facility. PARTICIPANTS: Adults (N=48; mean age, 34.3±5.8y; range, 25-46y) with CP. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: BMD z scores at the lumbar spine and hip using dual-energy x-ray absorptiometry, Gross Motor Function Classification System (GMFCS), body mass index (BMI), and ambulatory status. RESULTS: Mean BMD z scores were -1.40 for the lumbar spine, -1.36 for the total hip, and -1.02 for the femoral neck. The z scores were significantly lower for the nonambulatory group at all 3 sites (P<.05). Significant differences were found among GMFCS levels for the lumbar spine and total hip z scores (P<.05). For the lumbar spine, the mean z scores for level V (the lowest mobility level) were significantly lower than the mean for levels I/II (P=.001), III (P=.002), and IV (P=.013). For the total hip, the mean z scores for level V were significantly lower than the mean for levels I/II (P=.045). A significant positive relationship between the z scores and age was found for the lumbar spine (Spearman ρ=.40, P=.005). Significant positive relationships between BMI and z scores were found for all sites (P<.05). CONCLUSIONS: This study contributes to the sparse literature about bone health in adults with CP. In contrast with pediatric data, z scores did not decrease as a function of age in this adult cohort. This information is important for clinicians considering treatment options for this population.

What are the functional outcomes of endoprosthestic reconstructions after tumor resection?

BACKGROUND: The majority of published functional outcome data for tumor megaprostheses comes in the form of subjective functional outcome scores. Sparse objective data exist demonstrating functional results, activity levels, and efficiency of gait after endoprosthetic reconstruction in patients treated for orthopaedic tumors. Patients embarking on massive surgical operations, often in the setting of debilitating medical therapies, face mortality and a myriad of unknowns. Objective functional outcomes provide patients with reasonable expectations and a means to envision life after treatment. Objective outcomes also provide a means for surgeons to compare techniques, rehabilitation protocols, and implants. QUESTIONS/PURPOSES: We asked the following questions: (1) What is the efficiency of gait (ie, oxygen consumption) at final recovery from endoprosthetic reconstruction for oncologic resections? (2) What is the knee strength after lower extremity endoprosthetic reconstruction as compared with the contralateral limb? (3) How active are patients with tumor megaprostheses at home and in the community? METHODS: Sixty-nine patients with endoprosthetic reconstructions for primary lower extremity bone sarcoma met inclusion criteria and were invited by mailing to undergo oxygen cost study and strength testing. Twenty-four patients (seven proximal femoral replacements, nine distal femoral replacements, and eight proximal tibia replacements) underwent evaluation in the gait laboratory at a mean of 13.2 years after their reconstruction. All patients were then asked to wear step activity monitors at home and in the community for 7 consecutive days. RESULTS: Median O2 consumption (in mL/kg/m) among the endoprothesis groups was not different from the control patients with the numbers available (proximal femoral replacement 0.17, distal femoral replacement 0.16, proximal tibia replacement 0.18, control 0.15, p = 0.21). With the numbers available, there was no difference in walking speed as compared with the control group (proximal femoral replacement 1.20 m/s, distal femoral replacement 1.27 m/s, proximal tibia replacement 1.12 m/s, control 1.27 m/s, p = 0.08). Patients with proximal tibia replacements had reduced knee extension and flexion strength compared with patients in other reconstruction groups (84% reduction in extension versus those with proximal femoral replacements, 35%, and distal femoral replacement, 53%, p = 0.001, and 43% reduction in flexion versus proximal femoral replacement, 11%, distal femoral replacement, 2%, p = 0.006). With the numbers available, mean strides per day were not different among the reconstruction groups (proximal femoral replacement = 4709 strides/day [3094-6696], distal femoral replacement = 2854 [2461-6015], and proximal tibia replacement = 4411 [3093-6215], p = 0.53). CONCLUSIONS: Although knee strength was reduced in patients with proximal tibia replacements compared with femoral reconstructions, all groups had an efficient gait and were active at home and in the community at a mean of 13.2 years after surgery. Despite the magnitude of these surgeries, these patients are similarly active as patients after standard total hip arthroplasty. These findings provide objective data from which patients undergoing tumor megaprosthesis reconstructions of the lower extremity can reasonably base expectations of efficient gait and active lifestyles outside of the hospital setting. These data may provide hope and long-term goals for patients facing the uncertainty of chemotherapy and surgical treatment. LEVEL OF EVIDENCE: Level III, therapeutic study. See Instructions for Authors for a complete description of levels of evidence.

Selective voluntary motor control influences knee joint torque, work and power in children with spastic cerebral palsy.

BACKGROUND: Children with spastic cerebral palsy (CP) have damage to the corticospinal tracts that are responsible for selective motor control (SMC). Force, velocity and timing of joint movement are related biomechanical features controlled by the corticospinal tracts (CSTs) that are important for skilled movement. RESEARCH QUESTION: Does SMC influence knee joint biomechanics in spastic CP? METHODS: In this prospective study, relationships between SMC and knee biomechanics (peak torque, total work, average power) across a range of velocities (0-300 deg/s) were assessed using an isokinetic dynamometer in 23 children with spastic CP. SMC was assessed using Selective Control Assessment of the Lower Extremity (SCALE). Logistic and linear regression models were used to evaluate relationships between SCALE and biomechanical measures. RESULTS: The ability to produce knee torque diminished with increasing velocity for both Low (0-4 points) and High (5-10 points) SCALE limb score groups (p < 0.01). More knees in the High group produced extension torque at 300 deg/s (p < 0.05) and flexion torque at 30, 90,180, 240 and 300 deg/s (p < 0.05). The ability to produce torque markedly decreased above 180 deg/s for Low group flexion. For knees that produced torque, significant positive correlations between SCALE limb scores and joint torque (0 and 120 deg/s), work (120 deg/s) and power (120 deg/s) were found (p < 0.05). Greater knee torque, work and power for the High group was found for the extensors at most velocities and the flexors for up to 120 deg/s (p < 0.05). Few Low group participants generated knee flexor torque above 120 deg/s limiting comparisons. SIGNIFICANCE: Biomechanical impairments found for children with low SMC are concerning as skilled movements during gait, play and sport activities occur at high velocities. Differences in SMC should be considered when designing individualized assessments and interventions.

The PEDALS stationary cycling intervention and health-related quality of life in children with cerebral palsy: a randomized controlled trial.

AIM: The aim of this study was to assess health-related quality of life (HRQOL) following a stationary cycling intervention in children with cerebral palsy (CP). METHOD: This was a phase I multisite randomized controlled trial with single blinding. HRQOL was evaluated using the Pediatric Quality of Life Inventory SF15 (PedsQL; children) and Pediatric Outcomes Data Collection Instrument (PODCI; parent proxy) before and after a 3-month stationary cycling intervention. Sixty-two children (29 male, 33 female; mean age 11y; range 7-18y) with spastic diplegic CP, classified as levels I to III on the Gross Motor Function Classification System, were enrolled. Paired and independent t-tests were used to evaluate within- and between-group differences respectively. RESULTS: Between-group differences, favoring the cycling group, were found for PedsQL emotional functioning (p=0.046) and Parental PODCI treatment expectations scores (p=0.006). Between-group differences were not found for other scales. Within-group improvements were found in the cycling group: PedsQL total score (+5.8; p=0.006), psychosocial health summary (+6.9; p=0.008), and school functioning (+8.0; p=0.038). PODCI satisfaction with symptoms decreased significantly only in the control group (-12.0; p=0.046). INTERPRETATION: A beneficial influence of exercise on pediatric emotional well-being and parental treatment expectations was found. The evidence was not strong for other aspects of HRQOL. Results support the positive relationship between physical fitness and emotional well-being in the general population. A child's perception is important when examining change in his or her emotional well-being due to intervention.

Case reports: the influence of selective voluntary motor control on gait after hamstring lengthening surgery.

BACKGROUND: Preliminary evidence suggests selective voluntary motor control (SVMC), defined as performance of isolated voluntary joint movement on request, may be an important factor affecting functional movement tasks. Individuals with poor SVMC are unable to dissociate hip and knee synergistic movement during the swing phase of gait and have difficulty extending their knee while the hip is flexing during terminal swing regardless of hamstring length. This pattern may limit their ability to take advantage of hamstring-lengthening surgery (HLS) and may explain a lack of improved stride length postoperatively. QUESTIONS/PURPOSES: Provide a preliminary clinical and conceptual framework for using SVMC to predict swing phase parameters of gait after HLS. PATIENTS AND METHODS: We contrasted two patients with spastic diplegia of similar age, gross motor function, and spasticity but with different SVMC scores using the Selective Control Assessment of the Lower Extremity (SCALE). The patients underwent bilateral HLS. Popliteal angles, joint kinematics, step length, stride length, and walking velocity were assessed pre- and postoperatively. RESULT: Popliteal angles, terminal knee extension, and knee range of motion improved for both patients. However, only the patient with higher SCALE scores improved stride length postoperatively. CONCLUSION: Although preliminary, the data suggest that SVMC, as measured by SCALE, may be a prognostic factor for improved stride length after HLS in patients with spastic diplegia. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

A Longitudinal Study of Quantitative Muscle Strength and Functional Motor Ability in Ambulatory Boys with Duchenne Muscular Dystrophy.

BACKGROUND: Duchenne muscular dystrophy (DMD) is an X-linked recessive genetic disorder, that is characterized by progressive muscle degeneration and loss of ambulation between 7-13 years of age. Novel pharmacological agents targeting the genetic defects and disease mechanisms are becoming available; however, corticosteroid (CS) therapy remains the standard of care. OBJECTIVE: The purpose of this longitudinal study was to elucidate the effect of CS therapy on the rate of muscle strength and gross motor skill decline in boys with DMD and assess the sensitivity of selected outcome measures. METHODS: Eighty-four ambulatory boys with DMD (49-180 months), 70 on CS, 14 corticosteroid naïve (NCS), participated in this 8-year multi-site study. Outcomes included; isokinetic dynamometry, the Standing (STD) and Walking/Running/jumping (WRJ) dimensions of the Gross Motor Function Measure (GMFM), and Timed Function Tests (TFTs). Nonlinear mixed modeling procedures determined the rate of change with age and the influence of steroids. RESULTS: Despite CS therapy the rate of decline in strength with age was significant in all muscle groups assessed. CS therapy significantly slowed decline in knee extensor strength, as the NCS group declined at 3x the rate of the CS group. Concurrently, WRJ skills declined in the NCS group at twice the rate of the CS group. 4-stair climb and 10 meter walk/run performance was superior in the boys on CS therapy. CONCLUSION: CS therapy slowed the rate of muscle strength decline and afforded longer retention of select gross motor skills in boys on CS compared to boys who were NCS. Isokinetic dynamometry, Walk/Run/Jump skills, and select TFTs may prove informative in assessing the efficacy of new therapeutics in ambulatory boys with DMD.

Gait outcomes following proximal tibial tumor resection and endoprosthetic reconstruction.

BACKGROUND: Despite the proximal tibia being a common site of primary malignant bone tumors, there is limited information about gait function following proximal tibial tumor resection and endoprosthetic reconstruction (PTR). RESEARCH QUESTION: What is the impact of PTR on gait and quality of life? METHODS: This was a cross-sectional study of patients ≥18 years old who were ≥2 years post-PTR compared to a control group of similar age and sex distribution. Eighteen participants (9 PTR, 9 Control) were recruited. Gait spatial-temporal data, joint kinematics and kinetics were collected at preferred and fast walking speeds. Community walking cadence, health-related quality of life (SF-36) and knee joint torque were assessed. Comparisons were performed using one-way ANOVAs with Bonferroni corrections for multiple comparisons. Nonparametric tests were used for data not normally distributed. RESULTS: Mean age was 31 years for each group (PTR range = 18-42 yrs, Control range = 18-44 yrs). Compared to both control and nonsurgical limbs, the surgical limb exhibited significantly decreased % single limb support time, reduced heel rise during terminal stance and an absence of normally occurring knee flexion angles, extensor moments and power generation during initial double limb support. Additionally, a reduced peak plantar flexor moment was found for the surgical as compared to the control limb. The number of gait abnormalities increased during fast walking. Significantly reduced surgical knee extensor torque on isokinetic testing and weakness of the knee and ankle on clinical examination support gait findings. During community walking, the number of low frequency strides was an average of 5.3 % greater for the PTR group (p <  0.05). Norm-based PTR group SF-36 component scores were within normal values (53.4 physical, 56.5 mental). SIGNIFICANCE: Gait abnormalities were consistent with ankle muscle resection and transposition and knee extensor mechanism disruption. Despite these deficits, walking speed and quality of life were relatively normal.

Lower-extremity selective voluntary motor control in patients with spastic cerebral palsy: increased distal motor impairment.

AIM: Multiple impairments contribute to motor deficits in spastic cerebral palsy (CP). Selective voluntary motor control (SVMC), namely isolation of joint movement upon request, is important, but frequently overlooked. This study evaluated the proximal to distal distribution of SVMC impairment among lower extremity joints. METHOD: Using a recently developed tool, the Selective Control Assessment of the Lower Extremity (SCALE), we evaluated the SVMC of the hip, knee, ankle, subtalar joint, and toes in a cross-sectional, observational study of 47 participants with spastic, diplegic, hemiplegic, and quadriplegic CP (22 males, 25 females; mean age 11 y 9 mo, SD 4 y 8 mo; Gross Motor Function Classification System levels I-IV). RESULTS: Statistically significant decreases in SCALE scores from hip to toes were found using the Page statistical test for trend (p<0.001). Statistically significant differences (p<0.05) were found between all joint pairs, except toes versus subtalar, toes versus ankle, and right ankle versus subtalar joints. Cross-tabulation of score frequencies for all pairs revealed that proximal joint scores were higher or equal to distal ones 81 to 100% of the time. Excluding toes versus subtalar joints, proximal scores exceeded distal ones 94 to 100% of the time. INTERPRETATION: We confirmed increasing proximal to distal SVMC impairment, which may have implications for treatment and research.

Stationary cycling and children with cerebral palsy: case reports for two participants.

These case reports describe a stationary cycling intervention and outcomes for two child participants (P1 and P2) with spastic diplegic cerebral palsy. Each child completed a 12-week, 30-session cycling intervention consisting of strengthening and cardiorespiratory fitness phases. P1 exhibited higher training intensities, particularly during the cardiorespiratory phase. Average training heart rates were 59% and 35% of maximum heart rate for P1 and P2, respectively. Lower extremity peak knee flexor and extensor moments, gross motor function (Gross Motor Function Measure (GMFM-66)), preferred walking speed (thirty-second walk test), and walking endurance (600-yard walk-run test) were measured pre- and postintervention. Changes in outcome measurements corresponded with differences in exercise intensity. Greater gains in peak knee extensor moments, GMFM-66 scores (+4.2 versus +0.9), 600-yard walk-run test (-29% versus 0%) occurred for P1 versus P2, respectively. Preferred walking speeds did not increase substantially for P1 and decreased for P2.

Brain Metabolism During A Lower Extremity Voluntary Movement Task in Children With Spastic Cerebral Palsy.

Reduced selective voluntary motor control (SVMC) is a primary impairment due to corticospinal tract (CST) injury in spastic cerebral palsy (CP). There are few studies of brain metabolism in CP and none have examined brain metabolism during a motor task. Nine children with bilateral spastic CP [Age: 6-11 years, Gross Motor Function Classification System (GMFCS) Levels II-V] completed this study. SVMC was evaluated using Selective Control Assessment of the Lower Extremity (SCALE) ranging from 0 (absent) to 10 (normal). Brain metabolism was measured using positron emission tomography (PET) scanning in association with a selective ankle motor task. Whole brain activation maps as well as ROI averaged metabolic activity were correlated with SCALE scores. The contralateral sensorimotor and superior parietal cortex were positively correlated with SCALE scores (p < 0.0005). In contrast, a negative correlation of metabolic activity with SCALE was found in the cerebellum (p < 0.0005). Subsequent ROI analysis showed that both ipsilateral and contralateral cerebellar metabolism correlated with SCALE but the relationship for the ipsilateral cerebellum was stronger (R 2 = 0.80, p < 0.001 vs. R 2 = 0.46, p = 0.045). Decreased cortical and increased cerebellar activation in children with less SVMC may be related to task difficulty, activation of new motor learning paradigms in the cerebellum and potential engagement of alternative motor systems when CSTs are focally damaged. These results support SCALE as a clinical correlate of neurological impairment.

Selective Control Assessment of the Lower Extremity (SCALE): development, validation, and interrater reliability of a clinical tool for patients with cerebral palsy.

Normal selective voluntary motor control (SVMC) can be defined as the ability to perform isolated joint movement without using mass flexor/extensor patterns or undesired movement at other joints, such as mirroring. SVMC is an important determinant of function, yet a valid, reliable assessment tool is lacking. The Selective Control Assessment of the Lower Extremity (SCALE) is a clinical tool developed to quantify SVMC in patients with cerebral palsy (CP). This paper describes the development, utility, validation, and interrater reliability of SCALE. Content validity was based on review by 14 experienced clinicians. Mean agreement was 91.9% (range 71.4-100%) for statements about content, administration, and grading. SCALE scores were compared with Gross Motor Function Classification System Expanded and Revised (GMFCS-ER) levels for 51 participants with spastic diplegic, hemiplegic, and quadriplegic CP (GMFCS levels I - IV, 21 males, 30 females; mean age 11y 11mo [SD 4y 9mo]; range 5-23y). Construct validity was supported by significant inverse correlation (Spearman's r=-0.83, p<0.001) between SCALE scores and GMFCS levels. Six clinicians rated 20 participants with spastic CP (seven males, 13 females, mean age 12y 3mo [SD 5y 5mo], range 7-23y) using SCALE. A high level of interrater reliability was demonstrated by intraclass correlation coefficients ranging from 0.88 to 0.91 (p<0.001).

The effect of lower extremity selective voluntary motor control on interjoint coordination during gait in children with spastic diplegic cerebral palsy.

Damage to motor tracts in the periventricular white matter is a primary etiology in spastic diplegic cerebral palsy (CP). These tracts are responsible for the production of selective voluntary motor control (SVMC). Lower extremity motor control has been suggested as being an important predictor of improvement following interventions. While there are multiple impairments in spastic CP, the inability to perform purposeful voluntary movement is a critical factor in determining functional ability that merits investigation. The purpose of this study was to examine the relationship between SVMC ability and hip and knee coordination during the swing phase of gait in participants with spastic CP. Gait analysis and SVMC assessments were conducted for 15 participants with CP. Relative phase analysis was used to calculate the minimum relative phase (MRP) angle during swing; a measurement of interjoint coordination between the hip and the knee. SVMC ability was measured using the Selective Control Assessment of the Lower Extremity (SCALE) tool. Significant correlations were found between SCALE scores and both MRP values (p<0.0001) and duration of out-of-phase movement (p<0.005) during swing. These findings supported our hypothesis that SVMC ability is related to a patient's ability to move in an uncoupled pattern during the swing phase of gait (i.e., extending the knee while flexing the hip). An understanding of influence of SVMC on swing phase gait mechanics may help establish appropriate goals for interventions, in particular hamstring lengthenings.

The effect of direct measurement versus cadaver estimates of anthropometry in the calculation of joint moments during above-knee prosthetic gait in pediatrics.

Joint reaction forces, moments and powers are important in interpreting gait mechanics and compensatory strategies used by patients walking with above-knee prostheses. Segmental anthropometrics, required to calculate joint moments, are often estimated using data from cadaver studies. However, these values may not be accurate for patients following amputation as prostheses are composed of non-biologic material. The purpose of this study was to compare joint moments using anthropometrics calculated from cadaver studies versus direct measurements of the residual limb and prosthesis for children with an above-knee amputation. Gait data were collected for four subjects with above-knee prostheses walking at preferred and fast speeds. Joint moments were computed using anthropometrics from cadaver studies and direct measurements for each subject. The difference between these two methods primarily affected the inertia couple (Ialpha term) and the inertial effect due to gravity, which comprised a greater percentage of the total joint moment during swing as compared to stance. Peak hip and knee flexor and extensor moments during swing were significantly greater when calculated using cadaver data (p<0.05). These differences were greater while walking fast as compared to slow speeds. A significant difference was not found between these two methods for peak hip and knee moments during stance. A significant difference was found for peak ankle joint moments during stance, but the magnitude was not clinically important. These results support the use of direct measurements of anthropometry when examining above-knee prosthetic gait, particularly during swing.

Promotion of physical fitness and prevention of secondary conditions for children with cerebral palsy: section on pediatrics research summit proceedings.

Inadequate physical fitness is a major problem affecting the function and health of children with cerebral palsy (CP). Lack of optimal physical activity may contribute to the development of secondary conditions associated with CP such as chronic pain, fatigue, and osteoporosis. The purpose of this article is to highlight the content and recommendations of a Pediatrics Research Summit developed to foster collaborative research in this area. Two components of physical fitness-muscle strength and cardiorespiratory fitness-were emphasized. Although there is evidence to support the use of physical fitness interventions, there are many gaps in our current knowledge. Additional research of higher quality and rigor is needed in order to make definitive recommendations regarding the mode, intensity, frequency, and duration of exercise. Outcome measurements have focused on the body functions and structures level of the International Classification of Functioning, Disability and Health (ICF), and much less is known about effects at the activities and participation levels. Additionally, the influence of nutritional and growth factors on physical fitness has not been studied in this population, in which poor growth and skeletal fragility have been identified as serious health issues. Current intervention protocols and outcome measurements were critically evaluated, and recommendations were made for future research.

Pediatric endurance and limb strengthening for children with cerebral palsy (PEDALS)--a randomized controlled trial protocol for a stationary cycling intervention.

BACKGROUND: In the past, effortful exercises were considered inappropriate for children with spastic cerebral palsy (CP) due to concern that they would escalate abnormalities including spasticity and abnormal movement patterns. Current scientific evidence indicates that these concerns were unfounded and that therapeutic interventions focused on muscle strengthening can lead to improved functional ability. However, few studies have examined the potential benefits of cardiorespiratory fitness exercises in this patient population. METHODS/DESIGN: The rationale and design of a randomized controlled trial examining the effects of a stationary cycling intervention for children with CP are outlined here. Sixty children with spastic diplegic CP between the ages of 7 and 18 years and Gross Motor Function Classification System (GMFCS) levels of I, II, or III will be recruited for this study. Participants will be randomly assigned to either an intervention (cycling) or a control (no cycling) group. The cycling intervention will be divided into strengthening and cardiorespiratory endurance exercise phases. During the strengthening phase, the resistance to lower extremity cycling will be progressively increased using a uniquely designed limb-loaded mechanism. The cardiorespiratory endurance phase will focus on increasing the intensity and duration of cycling. Children will be encouraged to exercise within a target heart rate (HR) range (70-80% maximum HR). Thirty sessions will take place over a 10-12 week period. All children will be evaluated before (baseline) and after (follow-up) the intervention period. Primary outcome measures are: knee joint extensor and flexor moments, or torque; the Gross Motor Function Measure (GMFM); the 600 Yard Walk-Run test and the Thirty-Second Walk test (30 sec WT). DISCUSSION: This paper presents the rationale, design and protocol for Pediatric Endurance and Limb Strengthening (PEDALS); a Phase I randomized controlled trial evaluating the efficacy of a stationary cycling intervention for children with spastic diplegic cerebral palsy.

Reproductive healthcare experiences of women with cerebral palsy.

BACKGROUND: Little is known about pregnancy rates in women with disabilities in general and even less is known about women with child-onset disabilities such as cerebral palsy (CP). HYPOTHESIS: We hypothesized that discussions about pregnancy with healthcare providers and pregnancy rates for woman with CP would be related to their functional levels. METHODS: Survey methodology was used to gather information about demographics, function, whether women were asked about their desire for children, pregnancy outcomes, and services offered during pregnancy and postpartum. RESULTS: Of the 375 women with CP who participated in the survey, 76 (20%) reported 149 pregnancies resulting in 100 live births. Using Chi square statistics, mobility, manual dexterity, and communication function were significantly higher in women who were queried about or who experienced pregnancy. More than half of the women experienced a loss of mobility during pregnancy but few received referrals for physical or occupational therapy. Few reported screening for postpartum depression. A higher rate of Cesarean sections (50.4%), preterm births (12.1%), low birth weight infants (15.7%), and very low birth weight infants (7.1%) was reported by women with CP compared to national statistics. CONCLUSIONS: Pregnancy rates and discussions were related to functional levels. As 20% of women with CP surveyed experienced pregnancy, there is a need to increase awareness, education, support, and advocacy for achievement of optimal reproductive health. More research is needed to identify factors contributing to maternal and infant health in women with CP.

Joint moment contributions to swing knee extension acceleration during gait in individuals with spastic diplegic cerebral palsy.

The mechanisms contributing to swing phase knee acceleration in individuals with spastic diplegic cerebral palsy (CP) are not well understood, but evidence suggests that selective voluntary motor control (SVMC) may play a role. The purpose of this study was to examine the relationship between lower limb SVMC, measured using Selective Control Assessment of the Lower Extremity (SCALE), and joint moment contributions to swing knee extension acceleration in participants with spastic diplegic CP. Eighteen participants were recruited (mean age=13.8 years, range=6-30 years, Gross Motor Function Classification System Levels I-III). Induced acceleration analysis was performed during the swing phase of gait. Average joint moment contributions to swing knee extension acceleration were calculated. Contributions from stance limb and swing limb joint moments were correlated with SCALE scores using Pearson's correlations. A strong correlation was found (p<0.0001, r=0.85) between SCALE score and the total swing joint moment contributions to swing knee extension acceleration. As SCALE score increased, swing joint moments provided less resistance to knee extension acceleration. No relationship (p=0.18) was found between stance moment contributions to swing knee acceleration and stance limb SCALE scores. Excessive contributions from swing limb joint moments appear to be the factor limiting swing knee extension in spastic diplegic CP gait. Interventions that address negative contributions due to spasticity may not be effective in patients who cannot generate adequate knee extension due to poor SVMC.

Secondary mediation and regression analyses of the PTClinResNet database: determining causal relationships among the International Classification of Functioning, Disability and Health levels for four physical therapy intervention trials.

BACKGROUND: Each of the 4 randomized clinical trials (RCTs) hosted by the Physical Therapy Clinical Research Network (PTClinResNet) targeted a different disability group (low back disorder in the Muscle-Specific Strength Training Effectiveness After Lumbar Microdiskectomy [MUSSEL] trial, chronic spinal cord injury in the Strengthening and Optimal Movements for Painful Shoulders in Chronic Spinal Cord Injury [STOMPS] trial, adult stroke in the Strength Training Effectiveness Post-Stroke [STEPS] trial, and pediatric cerebral palsy in the Pediatric Endurance and Limb Strengthening [PEDALS] trial for children with spastic diplegic cerebral palsy) and tested the effectiveness of a muscle-specific or functional activity-based intervention on primary outcomes that captured pain (STOMPS, MUSSEL) or locomotor function (STEPS, PEDALS). OBJECTIVE: The focus of these secondary analyses was to determine causal relationships among outcomes across levels of the International Classification of Functioning, Disability and Health (ICF) framework for the 4 RCTs. METHOD AND DESIGN: With the database from PTClinResNet, we used 2 separate secondary statistical approaches-mediation analysis for the MUSSEL and STOMPS trials and regression analysis for the STEPS and PEDALS trials-to test relationships among muscle performance, primary outcomes (pain related and locomotor related), activity and participation measures, and overall quality of life. RESULTS: Predictive models were stronger for the 2 studies with pain-related primary outcomes. Change in muscle performance mediated or predicted reductions in pain for the MUSSEL and STOMPS trials and, to some extent, walking speed for the STEPS trial. Changes in primary outcome variables were significantly related to changes in activity and participation variables for all 4 trials. Improvement in activity and participation outcomes mediated or predicted increases in overall quality of life for the 3 trials with adult populations. LIMITATIONS: Variables included in the statistical models were limited to those measured in the 4 RCTs. It is possible that other variables also mediated or predicted the changes in outcomes. The relatively small sample size in the PEDALS trial limited statistical power for those analyses. CONCLUSIONS: Evaluating the mediators or predictors of change between each ICF level and for 2 fundamentally different outcome variables (pain versus walking) provided insights into the complexities inherent across 4 prevalent disability groups.