How is Physical Activity Measured in Spinal Muscular Atrophy and Duchenne Muscular Dystrophy?

BACKGROUND: Physical activity (PA) provides many substantial benefits to help reduce risk for cardiometabolic disease, improve cognitive function, and improve quality of life. Individuals with neuromuscular disorders (NMDs), such as spinal muscular atrophy (SMA) and Duchenne muscular dystrophy (DMD) are characterized by muscular weakness and fatigue, which limits the capacity to reach the recommended guidelines of PA. Measuring PA in these populations can provide insight to participation in daily activities, track disease progression, and monitor efficacy of drug treatments. OBJECTIVE: The objective of this study was to identify how PA is measured in SMA and DMD using instrumented and self-report methods, and how these methods are employed in ambulatory and non-ambulatory groups. METHODS: A scoping review was performed to identify studies that reported PA in these neuromuscular disorders. Inclusion was determined after a multi-stage review process by several reviewers, followed by an in-depth analysis of metrics reported by each tool that was used. RESULTS: A total of nineteen studies were identified and included in this review. Sixteen studies included instrumented measures and four studies utilized self-reported measures, with eleven studies also reporting PA information from a non-ambulatory group. A variety of metrics have been reported using both classes of measurement tools. CONCLUSION: Although a wide variety of research exists that details both instrumented and self-reported measurement tools, feasibility, cost, and study aims are important factors to consider in addition to testing methodology when selecting which type of tool to use. We recommend using a combination of instrumented and self-report measures to provide context to the PA measured in these populations. Improvements in both instrumented and self-report methodologies will add valuable knowledge about the disease burden and efficacy of treatment and disease management methods in SMA and DMD.

Effects of Power Training on Gait, Power, and Function in Children with Cerebral Palsy.

AIMS: This scoping review aims to: 1) examine available literature regarding the effects of power training on gait speed, power, and function in ambulatory children with CP and 2) identify the variations in exercise dosage and rehabilitation recommendations for power training and plyometrics in children with CP. METHODS: Four databases (PubMed, CINAHL, Embase, and Cochrane) were searched for papers including power or plyometric training with outcome measures for gait, power or functional performance. ES was calculated for RCTs. Cohorts and case series/studies were evaluated qualitatively. RESULTS: Ten articles fit search criteria: four RCTs, three cohort studies, one case series, and two case studies. Power training consistently demonstrated improvements in muscle power compared to its effects on gait and function. ES of mean MPST (W) ranged from 0.36-1.13. 1 MWT and SSGS ES were 1.31 and 1.15, respectively. TUG ES ranged from -0.33 to -2.42. ES for GMFM-66 was 0.13 and 1.11 for Dimension D and Dimension E, respectively. CONCLUSIONS: There is limited, but promising evidence to support that power training may improve gait speed, power, and function in children with CP. Future, more robust research is required to examine effects in a larger, diverse population, to determine long-term effects and exercise prescription.

Nutrition & Exercise Interventions in Pediatric Patients with Brain Tumors: A Narrative Review.

Brain tumors have been the most common pediatric solid tumor and leading cause of morbidity and mortality. Improved survival emphasizes the importance of adverse treatment effects especially related to nutrition and exercise. Although studies have examined nutrition and exercise outcomes, few randomized trials exist. This narrative review included a systematic literature search with analysis of controlled or single group studies examining clinical and quality-of-life impact of nutrition or exercise interventions. Seven articles were included. Three nutrition studies demonstrated improvement with proactive feeding tubes, nutritional supplementation, and nutritional status. Two exercise studies showed improvement in measures of fitness and neuroanatomy with exercise in pediatric brain tumor survivors; two cohort studies demonstrated a link between quality of life and physical activity. Preliminary studies show nutrition and exercise may improve physical well-being and quality of life, suggesting future controlled studies are warranted to inform clinical care of children with brain tumors.