Kristie F. Bjornson

Ambulatory Physical Activity Performance in Youth With Cerebral Palsy and Youth Who Are Developing Typically

Background and Purpose Assessment of walking activity in youth with cerebral palsy (CP) has traditionally been “capacity-based.” The purpose of this study was to describe the day-to-day ambulatory activity “performance” of youth with CP compared with youth who were developing typically. Subjects Eighty-one youth with CP, aged 10 to 13 years, who were categorized as being in Gross Motor Function Classification System (GMFCS) levels I to III and 30 age-matched youth who were developing typically were recruited. Methods Using a cross-sectional design, participants wore the StepWatch monitor for 7 days while documenting average daily total step counts, percentage of time they were active, ratio of medium to low activity levels, and percentage of time at high activity levels. Results The youth with CP demonstrated significantly lower levels of all outcomes than the comparison group. Discussion and Conclusion Daily walking activity and variability decreased as functional walking level (GMFCS level) decreased. Ambulatory activity performance within the context of the daily life for youth with CP appears valid and feasible as an outcome for mobility interventions in CP.

Selective dorsal rhizotomy: efficacy and safety in an investigator‐masked randomized clinical trial

The objective of this single‐center investigator‐masked randomized clinical trial was to investigate the efficacy and safety of selective dorsal rhizotomy (SDR) in children with spastic diplegia. Forty‐three children with spastic diplegia were randomly assigned on an intention‐to‐treat basis to receive SDR plus physical therapy (PT), or PT alone. Thirty‐eight children completed follow‐up through 24 months. Twenty‐one children received SDR (SDR+PT group) and 17 received PT (PT Only group). SDR was guided with electrophysiological monitoring and performed by one experienced neurosurgeon. All subjects received equivalent PT. Spasticity was quantified with an electromechanical torque measurement device (spasticity measurement system [SMS]). The Gross Motor Function Measure (GMFM) was used to document changes in functional mobility. Primary outcome measures were collected at baseline, 6, 12, and 24 months by evaluators masked to treatment.

Psychometric properties of the quality of life questionnaire for children with CP

This paper describes the development and psychometric properties of a condition‐specific quality of life instrument for children with cerebral palsy (CP QOL‐Child). A sample of 205 primary caregivers of children with CP aged 4 to 12 years (mean 8y 5mo) and 53 children aged 9 to 12 years completed the CP QOL‐Child. The children (112 males, 93 females) were sampled across Gross Motor Function Classification System (GMFCS) levels (Level I=18%, II=28%, III=14%, IV=11%, V=27%). Primary caregivers also completed other measures of child health (Child Health Questionnaire; CHQ), QOL (KIDSCREEN), and functioning (GMFCS). Internal consistency ranged from 0.74 to 0.92 for primary caregivers and from 0.80 to 0.90 for child self‐report. For primary caregivers, 2‐week test‐retest reliability ranged from 0.76 to 0.89. The validity of the CP QOL is supported by the pattern of correlations between CP QOL‐Child scales with the CHQ, KIDSCREEN, and GMFCS. Preliminary statistics suggest that the child self‐report questionnaire has acceptable psychometric properties. The questionnaire can be freely accessed at http://www.deakin.edu.ac/hmnbs/chase/cerebralpalsy/cp_qol_home.php

Botulinum Toxin for Spasticity in Children With Cerebral Palsy: A Comprehensive Evaluation

BACKGROUND. Spasticity is a prevalent disabling clinical symptom for children with cerebral palsy. Treatment of spasticity with botulinum toxin in children with cerebral palsy was first reported in 1993. Botulinum toxin provides a focal, controlled muscle weakness with reduction in spasticity. Interpretation of the literature is difficult because of the paucity of reliable measures of spasticity and challenges with measuring meaningful functional changes in children with disabilities. OBJECTIVE. This study documents the effects of botulinum toxin A injections into the gastrocnemius muscles in children with spastic diplegia. Outcomes are evaluated across all 5 domains of the National Centers for Medical and Rehabilitation Research domains of medical rehabilitation. METHODS. A randomized, double-masked, placebo-controlled design was applied to 33 children with spastic diplegia with a mean age of 5.5 and Gross Motor Function Classification System Levels of I through III. Participants received either 12 U/kg botulinum toxin A or placebo saline injections to bilateral gastrocnemius muscles. Outcomes were measured at baseline and 3, 8, 12, and 24 weeks after injection. RESULTS. Significant decreases in the electromyographic representation of spasticity were documented 3 weeks after botulinum toxin A treatment. A significant decrease in viscoelastic aspects of spasticity was present at 8 weeks, and subsequent increases in dorsiflexion range were documented at 12 weeks for the botulinum toxin A group. Improvement was found in performance goals at 12 weeks and in maximum voluntary torque and gross motor function at 24 weeks for the botulinum toxin A. There were no significant differences between groups in satisfaction with performance goals, energy expenditure, Ashworth scores, or frequency of adverse effects. CONCLUSIONS. The safety profile of 12 U/kg of botulinum toxin A is excellent. Although physiologic and mechanical effects of treatment with botulinum toxin A were documented with functional improvement at 6 months, family satisfaction with outcomes were no different. Communication is needed to ensure realistic expectations of treatment.

Long‐term safety and efficacy of continuous intrathecal baclofen

Long-term continuous intrathecal baclofen (CITB) infusion is a treatment option used to manage otherwise intractable spasticity and is delivered via an implantable pump. The purpose of this single-center multidisciplinary review was to report on the long-term safety and efficacy of CITB in the treatment of 21 children with intractable severe spasticity of cerebral origin. Nineteen recipients had spastic quadriplegia and two had spastic diplegia. Seven recipients had level IV severity on the Gross Motor Functional Classification System and 14 had level V. Median age at implantation was 12 years (range 4 to 20). Fifteen recipients were male, 6 were female. Seventeen recipients were alive at the end of the follow-up period (31 to 78 months; mean 53, SD 4). The Ashworth scale showed a substantial decrease in spasticity in the upper and lower extremities at 6 months and at the most recent follow-up. The Gross Motor Function Measure and Pediatric Evaluation of Disability Inventory showed no functional change. Most treatment goals were at least partly achieved. Caregivers reported a reduction in use of oral medication for spasticity, and improvements in comfort, function, and ease of care. Caregiver satisfaction was high. During 80 recipient-years of pump operation, 153 treatment-associated adverse events occurred: 27 of these were device-related. There were four deaths unrelated to CITE, including one from acute pancreatitis. Our findings might assist in establishing patient selection criteria and treatment goals, improving patient follow-up, and monitoring adverse events.

THE ROLE OF SELECTIVE DORSAL RHIZOTOMY IN cérébral PALSY: CRITICAL EVALUATION OF A PROSPECTIVE CLINICAL SERIES

This is a prospective observational study of a consecutive series of 34 children with spastic cérébral palsy treated at a single center. 10 had spastic quadriplegia and 24 had spastic diplegia. AH were followed for at least one year. After selective dorsal rhizotomy (SDR), all children received one month of physical therapy at the center and were prescribed a program of physical therapy in their community. The children were assessed before and one year after SDR and physical therapy, using the Ashworth Scale, deep tendon reflex response, range of motion and the Gross Motor Function Measure. The results show that there is often a decrease in lower‐extremity spasticity and functional improvement after SDR with physical therapy, but that there is considerable variability in outcome. Randomized prospective clinical trials with masked objective outcome measures are needed to determine the efficacy of SDR.

Quantitative measurement of spasticity in children with cerebral palsy

Spasticity was quantified in nine children with spastic diplegia, using a sinusoidal displacement of the foot at frequencies from 3 to 12Hz. Ankle‐joint stiffness was separated into elastic (energystoring) and viscous (energy‐dissipating) components. ‘Path length’ was used to represent the variation in stiffness over this frequency range. Compared with 11 unaffected children, a significant difference in path lengths was demonstrated for the children with spasticity. An age‐dependent effect was demonstrated when path lengths of unaffected children were compared with those of 10 unaffected adults. A modified path‐length measure is proposed which minimizes age dependency, yet enables detection of spasticity. Passive stiffness properties of unaffected adults showed higher elastic stiffness, viscosity and friction than unaffected children. A method was developed to evade the need for temporary nerve blocks to calculate inertial properties of the foot in persons with spasticity.

The relationship of physical activity to health status and quality of life in cerebral palsy

Purpose: To compare the influence of functional level, ambulatory, and physical activity performance on self-reported health status and quality of life (QOL) of youth with cerebral palsy (CP) and with typical development. Methods: A cross-sectional comparison cohort design was used in 81 youth with CP, ages 10 to 13 years and 30 youth with typical development. Participants wore the StepWatch™ monitor for 7 days and completed the Activity Scale for Kids, Child Health Questionnaire-Child Form, and Youth Quality of Life Questionnaire. Multiple regression analysis was used. Results: Self-reported activity performance influenced self-reported physical (β = 0.36), behavioral (β = 0.32), and emotional (β = 0.29) health. Functional level and performance did not influence QOL. Conclusions: Measures of ambulatory and physical activity and youth-reported health status separated from the measure of QOL seem helpful in defining the specific health issues of ambulatory youth with CP and have implications for physical activity intervention.

Self-Reported Health Status and Quality of Life in Youth With Cerebral Palsy and Typically Developing Youth

OBJECTIVE To describe self-reported health status and quality of life (QOL) of ambulatory youths with cerebral palsy (CP) compared with sex- and age-matched typically developing youth (TDY). DESIGN Prospective cross-sectional cohort comparison. SETTING Community-based. PARTICIPANTS A convenience sample of 81 youth with CP (age range, 10-13 y) with Gross Motor Function Classification System (GMFCS) levels I through III and 30 TDY participated. They were recruited from 2 regional childrens hospitals and 1 regional military medical center. INTERVENTIONS Not applicable. MAIN OUTCOME MEASURES Participants completed the Child Health Questionnaire-Child Form (CHQ-CF87) for health status and the Youth Quality of Life for QOL. RESULTS Youth with CP reported significantly lower health status than age- and sex-matched TDY in the following CHQ-CF87 subscales: role/social behavioral physical, bodily pain, physical function, and general health (CP mean rank, 46.8-55.2; TDY mean rank, 62.2-80.9). There were significant differences across GMFCS levels. There were no significant differences in self-reported QOL. CONCLUSIONS Self-reported health status, but not QOL, appears sensitive to the functional health issues experienced by ambulatory youth with CP. Pain management and psychosocial support may be indicated for them.

Validity of the gross motor function measure

The objective of this study was to evaluate the validity of the Gross Motor Function Measure (GMFM) in children with spastic diplegia. The GMFM is a criterion-referenced evaluative tool designed to quantify change in motor function over time. The GMFM was administered to 37 children with spastic diplegia three times: once as a baseline and again after 12− and 24-month intervals. Paired videotapes of the GMFM assessments were viewed by a therapist familiar with the GMFM, but unfamiliar with the child. The therapist was asked to judge how much change in motor skills was observed between the tapes. The hypothesis was that correlation of change scores on the GMFM and masked therapists judgment of change by videotaped assessment would be ≥0.45. Spearman correlation coefficients for the GMFM total percent score were 0.66 and 0.79 at 12 and 24 months, respectively. The study provides evidence of the construct validity of the GMFM to measure change in motor function over a 12− and 24-month period of time in children with spastic diplegia.