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Angelman Syndrome is a rare neurodevelopmental disorder characterized by developmental delay, lack of speech, seizures, intellectual disability, characteristic behavior, and movement disorders. Clinical gait analysis provides the opportunity for movement quantification to investigate an observed maladaptive change in gait pattern and offers an objective outcome of change. Pressure-sensor-based technology, inertial and activity monitoring, and instrumented gait analysis (IGA) were employed to define motor abnormalities in Angelman syndrome. Temporal-spatial gait parameters of persons with Angelman Syndrome (pwAS) show deficiencies in gait performance through walking speed, step length, step width, and walk ratio. pwAS walk with reduced step lengths, increased step width, and greater variability. Three-dimensional motion kinematics showed increased anterior pelvic tilt, hip flexion, and knee flexion. PwAS have a walk ratio more than two standard deviations below controls. Dynamic electromyography showed prolonged activation of knee extensors, which was associated with a decreased range of motion and the presence of hip flexion contractures. Use of multiple gait tracking modalities revealed that pwAS exhibit a change in gait pattern to a flexed knee gait pattern.  Cross-sectional studies of individuals with AS show a regression toward this maladaptive gait pattern over development in pwAS ages 4-11. PwAS unexpectedly did not have spasticity associated with change in gait pattern. Multiple quantitative measures of motor patterning may offer early biomarkers of gait decline consistent with critical periods of intervention, insight into appropriate management strategies, objective primary outcomes, and early indicators of adverse events.

Background: A rectus femoris transfer (RFT) surgery with and without a hamstring lengthening (HSL) is used to treat stiff-knee gait in children with cerebral palsy (CP). While current literature has reported that a RFT surgery improves the kinematics at the knee, little is known about the kinematic changes at the hip.Research Question: Does a RFT surgery change hip joint kinematics in children with CP?Methods: This retrospective study included children (<18 years old) diagnosed with CP, who underwent a RFT procedure, and who were seen at our institution's accredited clinical motion laboratory. Patients with both pre- and post-operative gait analysis were identified and comparison between those analyses were performed to identify kinematic differences at the hip and knee. A total of 66 legs from 46 children (mean age: 11.1 ± 3.6) met the inclusion criteria.Results: Overall results revealed that a RFT did not change kinematics at the hip [p > 0.05], however, a RFT did increase the maximum knee flexion during the swing period [Mean Difference Post - Pre: 8.3°, 95% CI: 4.9-11.8, p < 0.0001]. Additionally, it was found that changes in hip extension during the terminal stance phase were significantly different between the combined RFT and HSL compared to solely an RFT. The results of this study also revealed that children whose stiff-knee gait did not improve, tended to have increased hip external rotation during terminal stance and swing and greater hip extension during terminal stance, compared to children whose stiff-knee gait did improve.Significance: Overall, a RFT with and without a HSL surgery improves hip and knee kinematics in the sagittal plane, however, improvements at the hip were not clinically significant. As a result, a RFT or a combined RFT with HSL should not be used to change hip kinematics in children with CP. [ABSTRACT FROM AUTHOR]