Improved Myelination following Camp Leg Power, a Selective Motor Control Intervention for Children with Spastic Bilateral Cerebral Palsy: A Diffusion Tensor MRI Study.

BACKGROUND AND PURPOSE: Children with spastic cerebral palsy have motor deficits associated with periventricular leukomalacia indicating WM damage to the corticospinal tracts. We investigated whether practice of skilled lower extremity selective motor control movements would elicit neuroplasticity. MATERIALS AND METHODS: Twelve children with spastic bilateral cerebral palsy and periventricular leukomalacia born preterm (mean age, 11.5 years; age range, 7.3-16.6 years) participated in a lower extremity selective motor control intervention, Camp Leg Power. Activities promoted isolated joint movement including isokinetic knee exercises, ankle-controlled gaming, gait training, and sensorimotor activities (3 hours/day, 15 sessions, 1 month). DWI scans were collected pre- and postintervention. Tract-Based Spatial Statistics was used to analyze changes in fractional anisotropy, radial diffusivity, axial diffusivity, and mean diffusivity. RESULTS: Significantly reduced radial diffusivity (P < . 05) was found within corticospinal tract ROIs, including 28.4% of the left and 3.6% of the right posterior limb of the internal capsule and 14.1% of the left superior corona radiata. Reduced mean diffusivity was found within the same ROIs (13.3%, 11.6%, and 6.6%, respectively). Additionally, decreased radial diffusivity was observed in the left primary motor cortex. Additional WM tracts had decreased radial diffusivity and mean diffusivity, including the anterior limb of the internal capsule, external capsule, anterior corona radiata, and corpus callosum body and genu. CONCLUSIONS: Myelination of the corticospinal tracts improved following Camp Leg Power. Neighboring WM changes suggest recruitment of additional tracts involved in regulating neuroplasticity of the motor regions. Intensive practice of skilled lower extremity selective motor control movements promotes neuroplasticity in children with spastic bilateral cerebral palsy.

Selective Motor Control is a Clinical Correlate of Brain Motor Tract Impairment in Children with Spastic Bilateral Cerebral Palsy.

BACKGROUND AND PURPOSE: Selective voluntary motor control is an important factor influencing gross motor function, interjoint coordination, and the outcome of hamstring-lengthening surgery in spastic cerebral palsy. Using DTI, we investigated whether selective voluntary motor control would show strong correlations with WM motor tract microstructure and whether selective voluntary motor control is more sensitive to global WM impairment than gross motor function. MATERIALS AND METHODS: Children with spastic bilateral cerebral palsy born preterm and typically developing children were recruited. The Selective Control Assessment of the Lower Extremity (SCALE) and Gross Motor Function Measure (GMFM) were assessed in participants with cerebral palsy. Participants underwent brain MR imaging to collect DWI data. Tract-Based Spatial Statistics was used to analyze the WM for between-group differences and correlations with SCALE and GMFM. ROI analyses compared motor regions. RESULTS: Twelve children with cerebral palsy (mean age, 11.5 years) and 12 typically developing children (mean age, 10.3 years) participated. Altered DTI outcomes were found throughout the whole brain for the cerebral palsy group. SCALE, developed to evaluate selective voluntary motor control in cerebral palsy, showed significant positive correlations with fractional anisotropy in more WM voxels throughout the whole brain and for motor regions, including the corticospinal tract and corpus callosum, compared with GMFM. A significant negative correlation between radial diffusivity and SCALE, but not GMFM, was found within the corpus callosum. CONCLUSIONS: SCALE was a more sensitive clinical correlate of motor and whole-brain WM tract impairment in children with spastic bilateral cerebral palsy, suggesting greater anisotropy and myelination in these regions for those with higher selective voluntary motor control.

Albuterol increases lean body mass in ambulatory boys with Duchenne or Becker muscular dystrophy.

BACKGROUND: Albuterol is a beta-2 agonist that has been demonstrated to increase muscle strength in studies in animals and humans. Based on a pilot study of extended-release albuterol Repetabs in children with dystrophinopathies, the authors conducted a randomized, double-blind, placebo-controlled study with a crossover design. METHODS: Fourteen boys with Duchenne or Becker muscular dystrophy, 6 to 11 years old, completed two treatment periods (albuterol and placebo), 12 weeks each, separated by a 12-week washout period. As the albuterol Repetab formulation was no longer available, an alternate extended release albuterol was used (Volmax, 12 mg per day). Outcome measurements included 1) lean body mass, 2) fat mass, 3) isometric knee extensor and flexor moments, 4) manual muscle testing, and 5) timed functional tests. RESULTS: Lean body mass was significantly higher for subjects following albuterol treatment compared to placebo treatment, while fat mass was significantly lower. No differences were found in isometric knee moments or manual muscle tests. Time to run/walk 30 feet was improved following albuterol. CONCLUSIONS: Short-term treatment with extended release albuterol may increase lean body mass, decrease fat mass, and improve functional measures in patients with dystrophinopathies. However, the significant change in strength of specific muscle groups found in the pilot study was not observed in the present study. These findings may be attributed to differences in the drug release and kinetics between Repetab and Volmax formulations as they affect the concentration of available beta-2 receptors on the muscle cell surface differently.

Pilot trial of albuterol in Duchenne and Becker muscular dystrophy.

The authors conducted a randomized, crossover, double-blind, placebo-controlled pilot study of albuterol in nine boys with dystrophinopathies. Primary outcomes were 1) isometric knee extensor and flexor strength; and 2) manual muscle testing (MMT). Isometric knee extensor strength and MMT scores were significantly higher in patients taking albuterol vs placebo. Therefore, 12-week treatment with extended-release albuterol may increase strength in patients with dystrophinopathies. A larger, double-blind, randomized study is necessary to confirm these results.

Functional assessment of children with cerebral palsy following limited (L4-S1) selective posterior rhizotomy--a preliminary report.

OBJECTIVE: Selective Posterior Rhizotomy (SPR) is effective for reducing spasticity in children with cerebral palsy (CP). Nonetheless, extensive sensory deafferentation associated with this procedure can lead to prolonged postoperative hypotonia that delays the functional recovery of the patient. As lumbar rhizotomy provokes suprasegmental hypotonia, we hypothesized that reducing the extent of the deafferentation to the roots of L4-S1 levels would reduce the risk of postoperative hypotonia. METHODS AND RESULTS: Five patients with spastic cerebral palsy (4 males and 1 females, age range: 4-12 years) underwent limited selective dorsal rhizotomy (LSDR) of three (L4-S1) dorsal roots. All patients were able to walk independently prior to surgery. Functional assessments of these patients were performed pre and post operatively. Assessments included spasticity evaluation, passive range of motion, and sagittal plane kinematics of the hip, knee, and ankle during walking. Following surgery, reduced spasticity, increased passive range of motion and improved joint motion during walking was observed. Specifically, peak hip and knee extension and peak ankle dorsiflexion increased while peak plantarflexion decreased. CONCLUSIONS: Strength and motor control were not adversely affected by this procedure in any of the subjects and all patients actually demonstrated improvements. Previous studies have demonstrated that LSDR is highly effective in reducing spasticity and achieving functional outcome in spastic children. The results of this study demonstrated improved function during walking as assessed using gait analysis techniques.

The effect of quadriceps femoris muscle strengthening exercises on spasticity in children with cerebral palsy.

BACKGROUND AND PURPOSE: The Bobath neurodevelopmental treatment approach advised against the use of resistive exercise, as proponents felt that increased effort would increase spasticity. The purpose of this study was to test the premise that the performance of exercises with maximum efforts will increase spasticity in people with cerebral palsy (CP). Spasticity, in the present study, was defined as a velocity-dependent hyperexcitability of the muscle stretch reflex. SUBJECTS: Twenty-four subjects with the spastic diplegic form of CP (mean age=11.4 years, SD=3.0, range=7-17) and 12 subjects without known neurological impairments (mean age=11.6 years, SD=3.5, range=7-17) were assessed. METHODS: Knee muscle spasticity was assessed bilaterally using the pendulum test to elicit a stretch reflex immediately before and after 3 different forms of right quadriceps femoris muscle exercise (isometric, isotonic, and isokinetic) during a single bout of exercise training. Pendulum test outcome measures were: (1) first swing excursion, (2) number of lower leg oscillations, and (3) duration of the oscillations. RESULTS: There were no changes in spasticity following exercise between the 2 groups of subjects. DISCUSSION AND CONCLUSION: These results do not support the premise that exercises with maximum efforts increase spasticity in people with CP.

Sensitivity of the pendulum test for assessing spasticity in persons with cerebral palsy.

The sensitivity of the pendulum test to variation in spasticity in persons with spastic cerebral palsy (CP) was tested in 30 participants with CP and 10 participants without CP (controls) (mean age 13.8 years). The participants with CP were classified into three groups, with normal (mean age 15.9 years), mild/moderate (13.0 years), or severe (23.0 years) muscle tone, as assessed clinically using a modified Ashworth scale. Joint motion during the pendulum test was measured with an electrogoniometer. Muscle relaxation was confirmed using surface EMG. Outcome measures from the pendulum test were (1) number of oscillations, (2) duration of oscillations, (3) excursion of the first backward swing, and (4) relaxation index (first swing excursion/difference between the starting and resting angles). Data were assessed using one-way analysis of variance. Outcome measures 1 to 3 differed significantly between control participants and participants with CP (p<0.05). The first swing excursion was the best predictor of the degree of spasticity in persons with CP, being significantly different between all groups (p<0.05). The number of oscillations and their duration differentiated between control participants and all participants with CP (p<0.05) but not between participants with CP who had mild/moderate versus severe spasticity (p>0.05). The relaxation index was not a sensitive measure (p>0.05 between most study groups). We conclude that the pendulum test is a valid tool for assessing spasticity in persons with CP and that the first swing excursion is the most sensitive outcome measure.

Contrasts in gait mechanics of individuals with proximal femoral focal deficiency: Syme amputation versus Van Nes rotational osteotomy.

Lower-limb kinematics and kinetics during preferred and fast speeds of walking were measured in persons with proximal femoral focal deficiency to compare outcomes after Syme amputation (nine subjects) with those after Van Nes rotational osteotomy (10 subjects). Subjects with a Van Nes rotational osteotomy and full tibial rotation (seven subjects) demonstrated prosthetic knee function during stance as they were able to support a flexed-knee posture at both speeds and produced greater knee-extensor moments at preferred speeds as compared with the Syme group (p < 0.05). Nonprosthetic limb compensatory mechanics were significantly exacerbated in subjects with a Syme amputation compared with the Van Nes group: (a) stance-phase vaulting, resulting in greater inappropriate ankle-power generation at both walking speeds, (b) excessive hip-extensor moments at fast speeds, (c) excessive hip-power absorption and generation at both speeds, and (d) excessive knee-joint power generation at both speeds (p < 0.05). The improved gait after Van Nes rotational osteotomy is one factor that should be considered when making clinical decisions for children with proximal femoral focal deficiency.

Relationship between ankle muscle and joint kinetics during the stance phase of locomotion in the cat.

The purpose of this study was to examine the relationship between internal force production in selected skeletal muscles and the externally calculated joint moment during overground locomotion in the adult cat. Hindlimb segments were modelled as a linked system of rigid bodies and a generalized muscle moment (GMM), the sum over all active and passive tissues acting about the joint, was calculated using principles of inverse dynamics. Moments produced by individual muscles were calculated using tendon transducers implanted in freely moving cats and muscle moment arm information. Results indicated that the externally measured variables of peak ground reaction force and joint position were equally important to the determination of peak ankle GMM. Examination of peak moments revealed that increases in peak ankle GMM were met by increases in medial (MG) and lateral (LG) gastrocnemius output. Peak soleus (SOL) moments did not change significantly as a function of peak ankle GMM. The role of the plantaris (PLT) was less clear, with peak moments increasing significantly as a function of peak ankle GMM in one cat. All four ankle extensors were important to the attainment of peak ankle GMM early in stance. Subsequently, SOL and PLT contributed substantially to the ankle GMM throughout stance, LG moments declined to near zero, soon after peak ankle GMM; and MG moments demonstrated a substantial but more gradual decline. The relative contributions of these individual muscles to the ankle GMM were supported by their respective architecture, uniarticular versus multiarticular function, and physiological profiles.

Differential kinetics of fast and slow ankle extensors during the paw-shake in the cat.

Force, length, and EMG were assessed in the medial gastrocnemius and soleus muscles of two cats during the paw-shake response. The medial gastrocnemius produced high forces and significant electrical activity while force production and electrical activity were negligible in the soleus. This observation is significant as it provides evidence, through the direct measurement of muscle force, of selective recruitment of a fast muscle when a slow synergist is not activated. Additionally, the relationship among force, length, and neural activation indicates that the role of the medial gastrocnemius during the paw-shake response is to decelerate muscle lengthening and begin muscle shortening.