Energetics of walking in individuals with cerebral palsy and typical development, across severity and age: A systematic review and meta-analysis

Background: Individuals with cerebral palsy (CP) report physical fatigue as a main cause of limitation, deterioration and eventually cessation of their walking ability. A consequence of higher level of fatigue in individuals with CP leads to a less efficient and long-distance walking ability. Research question: This systematic review investigates the difference in 1) walking energy expenditure between individuals with CP and age-matched typically developing (TD) individuals; and 2) energetics of walking across Gross Motor Function Classification System (GMFCS) levels and age. Methods: Five electronic databases (PubMed, Web of Science, CINAHL, ScienceDirect and Scopus) were searched using search terms related to CP and energetics of walking. Results: Forty-one studies met inclusion criteria. Thirty-one studies compared energy expenditure between CP and age-matched controls. Twelve studies correlated energy expenditure and oxygen cost across GMFCS levels. Three studies investigated the walking efficiency across different ages or over a time period. A significant increase of energy expenditure and oxygen cost was found in individuals with CP compared to TD age-matched individuals, with a strong relationship across GMFCS levels. Significance: Despite significant differences between individuals with CP compared to TD peers, variability in methods and testing protocols may play a confounding role. Analysis suggests oxygen cost being the preferred/unbiased physiological parameter to assess walking efficacy in CP. To date, there is a knowledge gap on age-related changes of walking efficiency across GMFCS levels and wider span of age ranges. Further systematic research looking at longitudinal age-related changes of energetics of walking in this population is warranted

Reduced Skeletal Muscle Satellite Cell Number Alters Muscle Morphology After Chronic Stretch But Allows Limited Serial Sarcomere Addition

Introduction: Muscles add sarcomeres in response to stretch, presumably to maintain optimal sarcomere length. Clinical evidence from patients with cerebral palsy, who have both decreased serial sarcomere number and reduced satellite cells (SCs), suggests a hypothesis that SCs may be involved in sarcomere addition. Methods: A transgenic Pax7‐DTA mouse model underwent conditional SC depletion, and their soleii were then stretch‐immobilized to assess the capacity for sarcomere addition. Muscle architecture, morphology, and extracellular matrix (ECM) changes were also evaluated. Results: Mice in the SC‐reduced group achieved normal serial sarcomere addition in response to stretch. However, muscle fiber cross‐sectional area was significantly smaller and was associated with hypertrophic ECM changes, consistent with fibrosis. Conclusions: While a reduced SC population does not hinder serial sarcomere addition, SCs play a role in muscle adaptation to chronic stretch that involves maintenance of both fiber cross‐sectional area and ECM structure

Lifelong Fitness in Ambulatory Children and Adolescents with Cerebral Palsy I: Key Ingredients for Bone and Muscle Health

Physical activity of a sufficient amount and intensity is essential to health and the prevention of a sedentary lifestyle in all children as they transition into adolescence and adulthood. While fostering a fit lifestyle in all children can be challenging, it may be even more so for those with cerebral palsy (CP). Evidence suggests that bone and muscle health can improve with targeted exercise programs for children with CP. Yet, it is not clear how musculoskeletal improvements are sustained into adulthood. In this perspective, we introduce key ingredients and guidelines to promote bone and muscle health in ambulatory children with CP (GMFCS I–III), which could lay the foundation for sustained fitness and musculoskeletal health as they transition from childhood to adolescence and adulthood. First, one must consider crucial characteristics of the skeletal and muscular systems as well as key factors to augment bone and muscle integrity. Second, to build a better foundation, we must consider critical time periods and essential ingredients for programming. Finally, to foster the sustainability of a fit lifestyle, we must encourage commitment and self-initiated action while ensuring the attainment of skill acquisition and function. Thus, the overall objective of this perspective paper is to guide exercise programming and community implementation to truly alter lifelong fitness in persons with CP

Mitochondrial physiology

As the knowledge base and importance of mitochondrial physiology to evolution, health and disease expands, the necessity for harmonizing the terminology concerning mitochondrial respiratory states and rates has become increasingly apparent. The chemiosmotic theory establishes the mechanism of energy transformation and coupling in oxidative phosphorylation. The unifying concept of the protonmotive force provides the framework for developing a consistent theoretical foundation of mitochondrial physiology and bioenergetics. We follow the latest SI guidelines and those of the International Union of Pure and Applied Chemistry (IUPAC) on terminology in physical chemistry, extended by considerations of open systems and thermodynamics of irreversible processes. The concept-driven constructive terminology incorporates the meaning of each quantity and aligns concepts and symbols with the nomenclature of classical bioenergetics. We endeavour to provide a balanced view of mitochondrial respiratory control and a critical discussion on reporting data of mitochondrial respiration in terms of metabolic flows and fluxes. Uniform standards for evaluation of respiratory states and rates will ultimately contribute to reproducibility between laboratories and thus support the development of data repositories of mitochondrial respiratory function in species, tissues, and cells. Clarity of concept and consistency of nomenclature facilitate effective transdisciplinary communication, education, and ultimately further discovery

Mitochondrial physiology

As the knowledge base and importance of mitochondrial physiology to evolution, health and disease expands, the necessity for harmonizing the terminology concerning mitochondrial respiratory states and rates has become increasingly apparent. The chemiosmotic theory establishes the mechanism of energy transformation and coupling in oxidative phosphorylation. The unifying concept of the protonmotive force provides the framework for developing a consistent theoretical foundation of mitochondrial physiology and bioenergetics. We follow the latest SI guidelines and those of the International Union of Pure and Applied Chemistry (IUPAC) on terminology in physical chemistry, extended by considerations of open systems and thermodynamics of irreversible processes. The concept-driven constructive terminology incorporates the meaning of each quantity and aligns concepts and symbols with the nomenclature of classical bioenergetics. We endeavour to provide a balanced view of mitochondrial respiratory control and a critical discussion on reporting data of mitochondrial respiration in terms of metabolic flows and fluxes. Uniform standards for evaluation of respiratory states and rates will ultimately contribute to reproducibility between laboratories and thus support the development of data repositories of mitochondrial respiratory function in species, tissues, and cells. Clarity of concept and consistency of nomenclature facilitate effective transdisciplinary communication, education, and ultimately further discovery

Can the period of postnatal codevelopment of the rubrospinal and corticospinal systems provide new insights into refinement of limb movement?

The corticospinal and the rubrospinal tracts are thought to synergistically contribute to the limb control during motor development. Williams et al. (J Neurosci 34: 4432-4441, 2014) demonstrate that the postnatal maturation of red nucleus motor map and the rubrospinal tract develops earlier than the corticospinal tract, to support early forelimb control. They have two distinct phases of maturation; a "precorticospinal" phase characterized by development of the rubrospinal system, and a "cocorticospinal" phase where they overlap with corticospinal development