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Introduction: Biomarkers of disease severity in Charcot-Marie-Tooth disease (CMT) are required to evaluate early responses to treatment. In this study we used magnetic resonance imaging (MRI) to evaluate the relationship between muscle volume and intramuscular fat accumulation with weakness, disability, and impaired gait in affected children and adolescents.Methods: Fifty-five participants underwent MRI of the anterior compartment of the lower leg. Muscle and fat volumes were calculated. Strength was measured using hand-held dynamometry, disability using the CMT Pediatric Scale, and 3-dimensional gait analysis using an 8-camera Vicon Nexus motion capture system.Results: Lower muscle volume was significantly associated with reduced dorsiflexion strength, increased disability, impaired gait profile score, and foot drop. Intramuscular fat accumulation was associated with reduced dorsiflexion strength and impaired gait profile score.Discussion: The MRI protocol described was feasible, reliable, and sensitive to the magnitude of weakness, disability, and walking difficulties in children with CMT. Muscle Nerve 59:213-217, 2019. [ABSTRACT FROM AUTHOR]

Aims:To determine whether electrical stimulation (ES) following botulinum toxin A (BoNT/A) injection increases passive extensibility of the hamstring muscles in children with spastic diplegia.Methods:Six children undergoing bilateral BoNT/A injections to the hamstrings participated in this within-participant single blind randomized controlled trial. One leg of each child was randomised to the experimental condition and the other to the control condition. The experimental leg received daily stretch and ES to the hamstrings for 12 weeks, while the control leg received only daily stretch. The primary outcome was passive hamstring extensibility reflected by popliteal angle measured with a standardised torque. Secondary outcomes were two goniometric measures of popliteal angle using the Modified Tardieu Scale (R1 and R2), and parents' perceptions of treatment effectiveness. Outcomes were measured at baseline, 4 weeks, 12 weeks and 6 months.Results:The mean between-group difference (95% CI) at 4 weeks was 2° (−2 to 5) for popliteal angle measured with a standardised torque, favouring the experimental leg. Tardieu results for R1 and R2 were 0° (−4 to 3) and 7° (0 to 14), respectively.Conclusion:ES does not improve passive extensibility of the hamstring muscles at 4 weeks over any possible effects of BoNT/A alone. [ABSTRACT FROM PUBLISHER]

Aim: The purpose of this study was to examine the intra-rater and inter-rater reliability of physiotherapists using the Pirani scoring system for clubfoot to score digital photographs of infants' feet. Methods: Twelve infant feet were photographed to reflect variations in the six clinical signs of clubfoot. Physiotherapists viewed the photographs and scored the feet using the Pirani scoring system on two separate occasions. Results: Intra-rater reliability was excellent when scoring photographs, while inter-rater reliability was found to be fair to good for most subscales. Experienced therapists demonstrated higher reliability than novice therapists but not significantly so. Reliability was equivalent between the Pirani scoring system and a modified five-point scoring system created for this study. Scoring the rigidity of equinus subscale was found to be more reliable than the other subscales (P<0.01), which did not differ significantly from each other. [ABSTRACT FROM AUTHOR]

We report on a female patient with stiff-knee gait resulting from rectus femoris fibrosis, following multiple injections into the quadriceps musculature. Treatment planning and outcome were aided by instrumented three-dimensional gait analysis. One year after surgery, improvements were found in walking speed, step length, anterior pelvic tilt, knee flexion in swing, and ankle dorsiflexion in swing. Instrumented three-dimensional gait analysis was a useful tool for characterizing gait impairments and detecting changes after surgical intervention. Intramuscular injections into the rectus femoris muscle should be avoided to prevent stiff-knee gait and associated disability.

This study examines inter-rater reliability between physiotherapists using the Pirani scoring system for clubfoot, and whether the addition of two scale points to give a modified five-point severity scale improves reliability. A total of 65 infant feet were assessed by two raters, with 21 different rater combinations used. The Pirani scoring system was found to be a reliable assessment tool when used by physiotherapists to score clubfoot, with a minimum of fair to good inter-rater reliability demonstrated across all clinical signs. The modified five-point scale proved significantly more reliable than the three-point scale; however, the benefit is not sufficient to warrant varying the original three-point scale.

Normative reference values are essential to identify deviation from normal and evaluate response to treatment. As joint range of motion datasets specific to the pediatric population are infrequently reported in the literature, we determined lower limb passive joint range of motion and bone torsion values from 53 typically developing children aged 4-16 years. Our reference values were consistent with previously published norms, although for some measures, large variability in the literature exists. A clear correlation between joint range and age was observed in most measures. Our results highlight the importance of applying age-matched norms when attempting to identify deviation from normal in the growing child.