Susan Sienko Thomas

Comparing botulinum toxin A with casting for treatment of dynamic equinus in children with cerebral palsy

The purpose of this study was to compare the cumulative efficacy (three treatment sessions) of botulinum toxin A (BTX‐A) alone, casting alone, and the combination of BTX‐A and casting in the management of dynamic equinus in ambulatory children with spastic cerebral palsy (CP). Thirty‐nine children with spastic CP (mean age 5y 10mo, range 3 to 9y) were enrolled in the study. A multicenter, randomized, double blind, placebo‐controlled prospective study was used. Children were randomly assigned to one of three treatment groups: BTX‐A only (B), placebo injection plus casting (C), or BTX‐A plus casting (B+C). The dosage for the BTX‐A injections was 4U/kg per extremity. Assessments were performed at baseline, 3,6,7.5, and 12 months with a total of three treatments administered after the evaluations at baseline, 3, and 6 months. Primary outcome measures were ankle kinematics, velocity, and stride length. Secondary outcome measures were ankle spasticity, strength, range of motion, and ankle kinetics. Group B made no significant change in any variable at any time. Groups C and B+C demonstrated significant improvements in ankle kinematics, spasticity, passive range of motion, and dorsiflexor strength. Results of this 1‐year study indicate that BTX‐A alone provided no improvement in the parameters measured in this study, while casting and BTX‐A/casting were effective in the short‐ and long‐term management of dynamic equinus in children with spastic CP.

Calcaneal gait in spastic diplegia after heel cord lengthening: a study with gait analysis

Calcaneal gait or deformity can be significant complication after heel cord lengthenig. After heel cord lenghtening, 20 children wirh spastic deiplegia were evaluated by gait analysis to define calcaneal gait objectively and describe associated morbidity. Mean age 5 years 2 months (range 2 years 7 months to 8 years 2 months), and mean length of follow-up was 5 years 8 months (range 1 years 1 month 11 year 3 months). Calcaneal gait was defined as dirsuflexion 1 SD beyond the mean in the sagittal plane for all phases of stance. In creased ankle dorsiflexion during midstance most accurately predicts calcaneal gait. Through gait analysis, a 30% (6 of 20) prevalence of calcaneal gait suggests that an incresed incidence of caolcaneal fair may be present after heel cord incidence of calcaneal gait may be present after heel cord lengthening.

Spinal deformity after selective dorsal rhizotomy in ambulatory patients with cerebral palsy.

Thirty-four patients with ambulatory spastic diplegia (ages 10–19.8 years) who were part of a prospective study of selective dorsal rhizotomy (SDR) had standardized radiographs before and after SDR. Follow-up ranged from 5 to 11.6 years after surgery. Two different surgical approaches were used: laminectomy (14 patients) and laminoplasty (20 patients). Radiographs were measured for coronal and sagittal balance. Thirty patients had a spinal deformity at long-term follow-up compared with 10 patients before surgery. Seventeen patients (50%) developed lumbar hyperlordosis greater than 60°. Six patients (18%) developed grade 1 spondylolisthesis, Scoliosis occurred de novo in eight patients (24%) and progressed by greater than 5° in two patients with preoperative scoliosis. No significant differences were found between laminoplasty and laminectomy patients. None of the patients have undergone any surgical intervention for spinal deformity. There was a higher incidence of spinal deformity after SDR than in normals and an historical control population, which warrants clinical and radiographic long-term follow-up.

Gait Changes in Children with Spastic Diplegia After Selective Dorsal Rhizotomy

Twenty-six ambulatory children underwent preoperative and 1-year postoperative assessments after selective dorsal rhizotomy. These included spasticity, passive range of motion, tone, three-dimensional motion analysis, and electromyography. Independent and dependent ambulators were evaluated separately. A decrease in spasticity was found in all lower extremity muscle groups. An increase in passive range of motion was found only at the hip for both independent and dependent ambulators. Gait changes included increases in velocity and stride length in the independent ambulators. An improvement in hip extension during stance was found in the dependent ambulators only; however, an increase in knee extension and dorsiflexion in stance were seen in both groups. Selective dorsal rhizotomy improves both passive and dynamic range of motion in children with spastic diplegia.

Relationship Between Clinical Outcome Measures and Parent Proxy Reports of Health-Related Quality of Life in Ambulatory Children With Duchenne Muscular Dystrophy

In Duchenne muscular dystrophy, data directly linking changes in clinical outcome measures to patient-perceived well-being are lacking. This study evaluated the relationship between clinical outcome measures used in clinical trials of ambulatory Duchenne muscular dystrophy (Vignos functional grade, quantitative knee extension strength, timed functional performance measures, and gait velocity) and 2 health-related quality of life measures—the Pediatric Outcomes Data Collection Instrument and Pediatric Quality of Life Inventory—in 52 ambulatory Duchenne muscular dystrophy subjects and 36 controls. Those with the disease showed significant decrements in parent proxy-reported health-related quality of life measures versus controls across all domains. The Pediatric Outcomes Data Collection Instrument transfers/basic mobility and sports/ physical function and the Pediatric Quality of Life Inventory physical functioning domains had significant associations with age (and hence disease progression) and traditional clinical outcome measures employed in clinical trials of ambulatory boys with Duchenne muscular dystrophy. Selected domains of the Pediatric Outcomes Data Collection Instrument and generic Pediatric Quality of Life Inventory are potential patient-reported outcome measures for clinical trials in ambulatory individuals with the disease.

Classification of the Gait Patterns of Boys With Duchenne Muscular Dystrophy and Their Relationship to Function

Corticosteroids have recently been shown to reduce expected loss of muscle strength in patients with Duchenne muscular dystrophy and extend the time they can walk. We evaluated 43 boys with the condition to determine whether taking corticosteroids is associated with differences in gait pattern, gross motor skills, energy efficiency, and timed motor performance. We used the gait deviation index to quantify the degree of gait pathology and a single measure of gait quality. There were minimal differences in gait pattern, gross motor skills, energy efficiency, or timed motor performance in boys who took corticosteroids compared with those who did not. Clustering by gait deviation index, however, revealed subtle differences between groups in gait patterns, gross motor skills, and energy efficiency. We conclude that, in boys with Duchenne muscular dystrophy, gait pattern deviations are related to function, which can provide further insight into the understanding of disease progression and treatment options to enhance function and maintain ambulation.

Stair locomotion in children with spastic hemiplegia: the impact of three different ankle foot orthosis (AFOs) configurations.

The purpose of this study was to investigate the impact of three different ankle foot orthoses (AFO) configurations on the function and kinematics of stair locomotion in children with spastic hemiplegia. Nineteen children were evaluated barefoot and with a hinged, posterior leaf spring (PLS) and solid AFO during stair ascent and descent. Stair specific items from the Pediatric Evaluation of Disability Inventory (PEDI) were used to evaluate function, while a motion measurement system was used to evaluate kinematics. The PEDI revealed no significant differences between AFOs and barefoot, although a greater percentage of children were able to keep up with their peers while wearing a hinged AFO. At the ankle, the hinged AFO provided the greatest amount of dorsiflexion during stance. All AFOs reduced plantarflexion in comparison to barefoot. The results of this study indicate that for children with spastic hemiplegia the use of an AFO did not impair stair ambulation.

Does gait continue to improve 2 years after selective dorsal rhizotomy

Although changes in the gait pattern of children with spastic diplegia 1 year after selective dorsal rhizotomy have been well documented, minimal information exists regarding the continued changes in the gait pattern over time. Despite improvements in gait after rhizotomy, 66-75% of patients still require orthopaedic surgery for residual deformities. The optimal timing of the orthopaedic surgery after selective dorsal rhizotomy is not well established because of the lack of information regarding changes in gait over a long term. Using three-dimensional gait analysis, the gait pattern of 23 children was evaluated preoperatively, 1 and 2 years postoperatively. There were significant improvements in hip, knee, and ankle motion at 1 year after surgery. Although improvements in the gait pattern were found between 1 and 2 years after surgery, the changes were not significant. Therefore orthopaedic intervention may be undertaken at 1 year after rhizotomy to enhance function, as changes in gait from 1 to 2 years after rhizotomy are minimal.

Simulated gait patterns: the resulting effects on gait parameters, dynamic electromyography, joint moments, and physiological cost index

Abstract Equinus, crouch and crouch/equinus are common gait patterns seen in children with cerebral palsy. Six normal subjects simulated each of the above gait abnormalities to determine the effect on gait parameters, electromyography, joint moments and physiological cost index. Assessment of normal and the pathological gait patterns was performed using gait analysis. The results indicate that as the complexity of the gait pattern increases, the gait parameters decrease and the physiological cost index increases. Although abnormalities were found in electromyographic activity and joint moments, the patterns were similar to those found in children with cerebral palsy when walking in equinus, crouch and crouch/equinus. Abnormal joint positions produce significant effects on the parameters of gait used to assess the child with cerebral palsy. During interpretation of the gait analysis data significant emphasis must be placed on clinical evaluation and the determination of primary and secondary responses.

Variability and minimum detectable change for walking energy efficiency variables in children with cerebral palsy

For individuals with neuromuscular disorders, the assessment of walking energy efficiency is useful as a clinical outcome measure. Issues surrounding data collection methodology, normalization of the data, and variability and clinical utility of energy efficiency data preclude universal application. This study examined the variability and the clinical utility of velocity, energy efficiency index (EEI), gross cost, and net nondimensional cost (NNcost) in children and adolescents with spastic diplegic cerebral palsy (CP) in Gross Motor Function Classification System (GMFCS) levels I to III. The energy efficiency of walking was evaluated in 23 children and adolescents (12 males, 11 females, mean age 11y 3mo [SD 3y 5mo]; range 7−17y). Day‐to‐day variability was similar for all energy efficiency variables, with no significant differences in magnitude of variability between GMFCS levels. Correlations between EEI and gross cost and EEI and NNcost were fairly good (r=0.65, p<0.001, and r=0.74, p<0.001 respectively). However, only gross cost and NNcost discriminated between GMFCS levels in children with CP. Gross cost required the greatest amount of change to be considered clinically significant, whereas NNcost and EEI required a similar amount of change. For cohorts of children with CP who are evaluated over time, NNcost is the best normalization method as it reduces the variability between participants of different ages, height, and weight while evaluating only the amount of energy used to ambulate.