Skeletal muscle maximal mitochondrial activity in ambulatory children with cerebral palsy

2021 ◽  
Author(s):  
Sudarshan Dayanidhi ◽  
Elisa H Buckner ◽  
Robin S Redmond ◽  
Henry G Chambers ◽  
Simon Schenk ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Cerebral Palsy ◽  
Mitochondrial Activity ◽  
Children With Cerebral Palsy

scholarly journals Skeletal Muscle Mitochondrial Physiology in Children With Cerebral Palsy: Considerations for Healthy Aging

2021 ◽  
Vol 12 ◽  
Author(s):  
Sudarshan Dayanidhi
Keyword(s):  
Skeletal Muscle ◽  
Cerebral Palsy ◽  
Endurance Exercise ◽  
Gene Networks ◽  
Healthy Aging ◽  
Mitochondrial Protein ◽  
Mitochondrial Activity ◽  
Endurance Capacity ◽  
Inner Mitochondrial Membrane ◽  
Children With Cerebral Palsy

Skeletal muscle contractile proteins require a constant supply of energy to produce force needed for movement. Energy (ATP) is primarily produced by mitochondrial organelles, located within and around muscle fibers, by oxidative phosphorylation that couples electron flux through the electron transport chain to create a proton gradient across the inner mitochondrial membrane that is in turn used by the ATP synthase. Mitochondrial networks increase in size by biogenesis to increase mitochondrial abundance and activity in response to endurance exercise, while their function and content reduce with constant inactivity, such as during muscle atrophy. During healthy aging, there is an overall decline in mitochondrial activity and abundance, increase in mitochondrial DNA mutations, potential increase in oxidative stress, and reduction in overall muscular capacity. Many of these alterations can be attenuated by consistent endurance exercise. Children with cerebral palsy (CP) have significantly increased energetics of movement, reduced endurance capacity, and increased perceived effort. Recent work in leg muscles in ambulatory children with CP show a marked reduction in mitochondrial function. Arm muscles show that mitochondrial protein content and mitochondria DNA copy number are lower, suggesting a reduction in mitochondrial abundance, along with a reduction in markers for mitochondrial biogenesis. Gene expression networks are reduced for glycolytic and mitochondrial pathways and share similarities with gene networks with aging and chronic inactivity. Given the importance of mitochondria for energy production and changes with aging, future work needs to assess changes in mitochondria across the lifespan in people with CP and the effect of exercise on promoting metabolic health.

scholarly journals The Appendicular Lean Mass Index Is a Suitable Surrogate for Muscle Mass in Children with Cerebral Palsy

2019 ◽  
Vol 149 (10) ◽  
pp. 1863-1868
Author(s):  
Ibrahim Duran ◽  
Kyriakos Martakis ◽  
Mirko Rehberg ◽  
Christina Stark ◽  
Anne Koy ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Cerebral Palsy ◽  
Muscle Mass ◽  
Lean Mass ◽  
Skeletal Muscle Mass ◽  
Rehabilitation Program ◽  
Appendicular Lean Mass ◽  
Significant Difference ◽  
Children With Cerebral Palsy ◽  
Level I

ABSTRACT Background Densitometrically measured lean body mass (LBM) is often used to quantify skeletal muscle mass in children with cerebral palsy (CP). Since LBM depends on the individual's height, the evaluation of $\frac{{{\rm{LBM}}}}{{heigh{t^2}}}\ $ (lean BMI) is often recommended. However, LBM includes not only skeletal muscle mass but also the mass of skin, internal organs, tendons, and other components. This limitation applies to a far lesser extent to the appendicular lean mass index (LMIapp). Objectives The aim of the study was to evaluate skeletal muscle mass in children with CP using total lean BMI (LMItot) and LMIapp. Methods The present study was a monocentric retrospective analysis of prospectively collected data among children and adolescents with CP participating in a rehabilitation program. In total, 329 children with CP [148 females; Gross Motor Function Classification Scale (GMFCS) I, 32 children; GMFCS II, 73 children; GMFCS III, 133 children; GMFCS IV, 78 children; and GMFCS V, 13 children] were eligible for analysis. The mean age was 12.3 ± 2.75 y. Pediatric reference centiles for age-adjusted LMIapp were generated using data from NHANES 1999–2004. Low skeletal muscle mass was defined as a z score for DXA determined LMItot and LMIapp less than or equal to −2.0. Results The z scores for LMIapp were significantly lower than LMItot in children with CP, GMFCS levels II–V (P < 0.001), with the exception of GMFCS level I (P = 0.121), where no significant difference was found. The prevalence of low LMItot (16.1%; 95% CI: 16.1, 20.1%) was significantly lower (P < 0.001) than the prevalence of LMIapp (42.2%; 95% CI: 36.9, 47.9%) in the study population. Conclusions The prevalence of low skeletal muscle mass in children with CP might be underestimated by LMItot. LMIapp is more suitable for the evaluation of skeletal muscle mass in children with CP.

scholarly journals Skeletal Muscle Mitochondrial Capacity Is Similar In Ambulatory And Non-ambulatory Children With Cerebral Palsy

2020 ◽  
Vol 52 (7S) ◽  
pp. 295-296
Author(s):  
Sudarshan Dayanidhi ◽  
Alexia Rudofski ◽  
Marysol Encarnacíon ◽  
Jill Larson ◽  
Vineeta Swaroop
Keyword(s):  
Skeletal Muscle ◽  
Cerebral Palsy ◽  
Children With Cerebral Palsy ◽  
Mitochondrial Capacity

scholarly journals Adipose Tissue Infiltration of Skeletal Muscle in Children with Cerebral Palsy

2009 ◽  
Vol 154 (5) ◽  
pp. 715-720.e1 ◽  
Author(s):  
David L. Johnson ◽  
Freeman Miller ◽  
Pravesh Subramanian ◽  
Christopher M. Modlesky
Keyword(s):  
Skeletal Muscle ◽  
Adipose Tissue ◽  
Cerebral Palsy ◽  
Children With Cerebral Palsy

scholarly journals Reduced mitochondrial DNA and OXPHOS protein content in skeletal muscle of children with cerebral palsy

2021 ◽  
Author(s):  
Ferdinand von Walden ◽  
Ivan J Vechetti ◽  
Davis Englund ◽  
Vandré C Figueiredo ◽  
Rodrigo Fernandez‐Gonzalo ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Mitochondrial Dna ◽  
Cerebral Palsy ◽  
Protein Content ◽  
Children With Cerebral Palsy

Prevalence of Speech Problems and the Use of Augmentative and Alternative Communication in Children With Cerebral Palsy: A Registry-Based Study in Norway

2010 ◽  
Vol 19 (1) ◽  
pp. 12-20 ◽  
Author(s):  
Guro Andersen ◽  
Tone R. Mjøen ◽  
Torstein Vik
Keyword(s):  
Cerebral Palsy ◽  
Motor Function ◽  
Gestational Age ◽  
Augmentative And Alternative Communication ◽  
Alternative Communication ◽  
Gross Motor ◽  
Gross Motor Function ◽  
Children With Cerebral Palsy ◽  
Communicative Abilities ◽  
Normal Speech

Abstract This study describes the prevalence of speech problems and the use of augmentative and alternative communication (AAC) in children with cerebral palsy (CP) in Norway. Information on the communicative abilities of 564 children with CP born 1996–2003, recorded in the Norwegian CP Registry, was collected. A total of 270 children (48%) had normal speech, 90 (16%) had slightly indistinct speech, 52 (9%) had indistinct speech, 35 (6%) had very indistinct speech, 110 children (19%) had no speech, and 7 (1%) were unknown. Speech problems were most common in children with dyskinetic CP (92 %), in children with the most severe gross motor function impairments and among children being totally dependent on assistance in feeding or tube-fed children. A higher proportion of children born at term had speech problems when compared with children born before 32 weeks of gestational age 32 (p > 0.001). Among the 197 children with speech problems only, 106 (54%) used AAC in some form. Approximately 20% of children had no verbal speech, whereas ~15% had significant speech problems. Among children with either significant speech problems or no speech, only 54% used AAC in any form.

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