Exercise is potentially therapeutic for neuromuscular disorders, but a risk of harm exists due to overwork weakness. We aimed to assess the safety and efficacy of progressive resistance exercise for ...foot dorsiflexion weakness in children with Charcot-Marie-Tooth disease.
We did this randomised, double-blind, sham-controlled trial across the Sydney Children's Hospitals Network (NSW, Australia). Children aged 6–17 years with Charcot-Marie-Tooth disease were eligible if they had foot dorsiflexion weakness (negative Z score based on age-matched and sex-matched normative reference values). We randomly allocated (1:1) children, with random block sizes of 4, 6, and 8 and stratification by age, to receive 6 months (three times per week on non-consecutive days; 72 sessions in total) of progressive resistance training (from 50% to 70% of the most recent one repetition maximum) or sham training (negligible non-progressed intensity), using an adjustable exercise cuff to exercise the dorsiflexors of each foot. The primary efficacy outcome was the between-group difference in dorsiflexion strength assessed by hand-held dynamometry (expressed as a Z score) from baseline to months 6, 12, and 24. The primary safety outcome was the between-group difference in muscle and intramuscular fat volume of the anterior compartment of the lower leg assessed by MRI (expressed as a scaled volume) from baseline to 6 months and 24 months. Participants, parents, outcome evaluators, and investigators other than the treatment team were masked to treatment assignment. Analysis was by intention to treat. The trial is registered with the Australian New Zealand Clinical Trials Registry, number ACTRN12613000552785.
From Sept 2, 2013, to Dec 11, 2014, we randomly assigned 60 children to receive progressive resistance exercise (n=30) or sham training (n=30), and 55 (92%) children completed the trial. ANCOVA-adjusted Z score differences in dorsiflexion strength between groups were 0 (95% CI −0·37 to 0·42; p=0·91) at 6 months, 0·3 (−0·23 to 0·81; p=0·27) at 12 months, and 0·6 (95% CI 0·03 to 1·12; p=0·041) at 24 months. Scaled muscle and fat volume was comparable between groups at 6 months (ANCOVA-adjusted muscle volume difference 0, 95% CI −0·03 to 0·10, p=0·24; and fat volume difference 0, 95% CI −0·01 to 0·05, p=0·25) and 24 months (0, −0·08 to 0·12, p=0·67; and 0, −0·05 to 0·03, p=0·58). No serious adverse events were reported.
6 months of targeted progressive resistance exercise attenuated long-term progression of dorsiflexion weakness without detrimental effect on muscle morphology or other signs of overwork weakness in paediatric patients with Charcot-Marie-Tooth disease.
Muscular Dystrophy Association and Australian National Health and Medical Research Council.
Response to Raphael Mendes Ritti Dias Parmenter, Belinda J.; Raymond, Jacqueline; Dinnen, Paul ...
Journal of the American Geriatrics Society,
20/May , Letnik:
62, Številka:
5
Journal Article
The objective of this study was to verify the long-term effects of exercise on energy expenditure and body composition in individuals with spinal cord injury (SCI), as very little information is ...available on this population under free-living conditions.
Free-living energy expenditure and body composition using doubly labeled water (DLW) was measured in 13 individuals with SCI, subdivided in 2 groups: (1) sedentary (SED; N = 7) and (2) regularly engaged in any exercise program, for at least 150 min·wk(-1) (EXE; N = 6).
The total daily energy expenditure (TDEE) was significantly higher in the EXE group (33 ± 4.5 kcal·kg(-1)·day(-1)) if compared with SED group (27 ± 4.3 kcal·kg(-1)·day(-1)). The percentage of body fat was significantly higher in SED group than in EXE group (38 ± 6% and 28 ± 9%).
Our findings revealed that, despite the severity of SCI, the actual ACSM's guidelines for weight management for healthy adults exercise could significantly increase TDEE and BMR and improve body composition in individuals who regularly perform exercise. However, the EXE group still showed a high percentage of body fat, suggesting that a more specific approach might be considered (ie, increased intensity or volume, or combining with a diet program).
Does 4 weeks of serial night casting followed by 4 weeks of stretching of the gastrocnemius and soleus improve ankle dorsiflexion range and other outcomes compared with no intervention in children ...and young adults with Charcot-Marie-Tooth disease?
Randomised trial with concealed allocation, assessor blinding, and intention-to-treat analysis.
30 children and young adults with Charcot-Marie-Tooth disease and restricted ankle dorsiflexion range.
The experimental group received 4 weeks of serial night casting followed by 4 weeks of weightbearing stretches. The control group received no intervention.
Primary outcome was ankle dorsiflexion range; secondary outcomes included foot deformity, mobility, balance, falls, and self-reported activity limitations. Outcomes were measured at baseline, 4, and 8 weeks.
By 4 weeks, serial night casting had increased ankle dorsiflexion range by a mean of 4 deg (95% CI 2 to 6) more in the experimental group than the control group. After a further 4 weeks of weightbearing stretches, the experimental group still had a mean of 3 deg (95% CI 0 to 5) more ankle dorsiflexion range than the control group. Other than reduced time to walk 10 metres at self-selected pace favouring night casting at 4 weeks, outcomes did not differ between groups at any time point. Two minor adverse events were reported in the experimental group.
4 weeks of serial night casting increased ankle dorsiflexion range compared with no intervention, but at 8 weeks there was no significant difference between groups.
ACTRN12605000011684.
The objective of this study was to determine whether doubly labeled water (DLW) and a multi-sensor armband (SWA) could detect the variation in energy expenditure incurred by a period of increased ...exercise (EXE) versus a period of high sedentary activity (SED), in individuals with spinal cord injury (SCI).
Eight participants with SCI were submitted to 2 testing periods of energy expenditure assessment: 1) a 14-day phase during which sedentary living conditions were imposed and 2) a 14-day phase during which an exercise training intervention was employed. For each phase, total daily energy expenditure (TDEE) and physical activity energy expenditure (PAEE) were measured by DLW and SWA.
Mean TDEE assessed by DLW, was significantly higher during EXE versus SED (11,605 ± 2151 kJ·day⁻¹ and 10,069 ± 2310 kJ·day⁻¹). PAEE predicted by DLW was also significantly higher during EXE versus SED (5422 ± 2240 kJ·day⁻¹ and 3855 ± 2496 kJ·day⁻¹). SWA-predicted PAEE significantly underestimated PAEE measured by the DLW during SED and EXE.
DLW is sensitive to detect variation in within-individual energy expenditure during voluntary increase in physical activity in individuals with SCI. SWA failed to detect statistically significant variations in energy expenditure between periods of high versus low activity in SCI.
The continued engagement of the professional workforce as supervisors is critical for the sustainability and growth of work-integrated learning activities in university degrees. This Australian study ...investigated factors that influence the willingness and ability of clinicians to continue to supervise clinical exercise physiology work-integrated learning opportunities and makes recommendations for future supervision engagement. Themes identified from a supervisor survey were: staffing and time availability; administrative processes and support; student quality, knowledge and attitudes; student learning experiences; supporting the profession; service benefit; clinical personal benefit; funding; workplace support; staff qualifications and experience; prior positive experiences; future recruitment; facilities and infrastructure; and supporting the university. The responses resulted in five key recommendations for future enhanced and sustainable placement supervision. These were: adoption of efficient supervision structures; development and use of a competency checklist; enhanced recognition of supervision; standardised placement paperwork and assessment tools; and broadening of placement scheduling. Author abstract, ed
This study was a systematic review with meta-analysis examining the efficacy of carbohydrate (CHO) ingestion compared with placebo (PLA) on endurance exercise performance in adults. Relevant ...databases were searched to January 2011. Included studies were PLA-controlled, randomized, crossover designs in which CHO ingestion not exceeding 8% and between 30 and 80 g/h during exercise of ≥1 h was evaluated via time trial (TT) or exercise time to exhaustion (TTE). The between-trial standardized mean differences effect size (ES) and pooled estimates of the effect of CHO ingestion were calculated. Of the 41,175 studies from the initial search, 50 were included. The ES for submaximal exercise followed by TT was significant (ES = 0.53; 95% CI = 0.37–0.69; P < 0.001) as was the ES for TT (ES = 0.30; 95% CI = 0.07–0.53; P = 0.011). The weighted mean improvement in exercise performance favored CHO ingestion (7.5 and 2.0%, respectively). TTE (ES = 0.47; 95% CI = 0.32–0.62; P < 0.001) and submaximal exercise followed by TTE (ES = 0.44; 95% CI = 0.08–0.80; P = 0.017) also showed significant effects, with weighted mean improvements of 15.1 and 54.2%, respectively, with CHO ingestion. Similar trends were evident for subanalyses of studies using only male or trained participants, for exercise of 1–3 h duration, and where CHO and PLA beverages were matched for electrolyte content. The data support that ingestion of CHO between 30 and 80 g/h enhances endurance exercise performance in adults.
The continued engagement of the professional workforce as supervisors is critical for the sustainability and growth of work-integrated learning activities in university degrees. This study ...investigated factors that influence the willingness and ability of clinicians to continue to supervise clinical exercise physiology work-integrated learning opportunities and makes recommendations for future supervision engagement. Themes identified from a supervisor survey were: staffing and time availability; administrative processes and support; student quality, knowledge and attitudes; student learning experiences; supporting the profession; service benefit; clinical personal benefit; funding; workplace support; staff qualifications and experience; prior positive experiences; future recruitment; facilities and infrastructure; and supporting the university. The responses resulted in five key recommendations for future enhanced and sustainable placement supervision. These were: adoption of efficient supervision structures; development and use of a competency checklist; enhanced recognition of supervision; standardized placement paperwork and assessment tools; and broadening of placement scheduling.
The effects of high frequency stimulation (HFS) of the primary vestibular afferents on synaptic transmission in the ventral
part of the medial vestibular nuclei (vMVN) were studied during postnatal ...development and compared with the changes in the
expression of the group I metabotropic glutamate receptor (mGluR) subtypes, mGluR1 and mGluR5. During the first stages of
development, HFS always induced a mGluR5- and GABA A -dependent long-term depression (LTD) which did not require NMDA receptor and mGluR1 activation. The probability of inducing
LTD decreased progressively throughout the development and it was zero at about the end of the second postnatal week. Conversely,
long-term potentiation (LTP) appeared at the beginning of the second week and its occurrence increased to reach the adult
value at the end of the third week. Of interest, the sudden change in the LTP frequency occurred at the time of eye opening,
about the end of the second postnatal week. LTP depended on NMDA receptor and mGluR1 activation. In parallel with the modifications
in synaptic plasticity, we observed that the expression patterns and localizations of mGluR5 and mGluR1 in the medial vestibular
nuclei (MVN) changed during postnatal development. At the earlier stages the mGluR1 expression was minimal, then increased
progressively. In contrast, mGluR5 expression was initially high, then decreased. While mGluR1 was exclusively localized in
neuronal compartments and concentrated at the postsynaptic sites at all stages observed, mGluR5 was found mainly in neuronal
compartments at immature stages, then preferentially in glial compartments at mature stages. These results provide the first
evidence for a progressive change from LTD to LTP accompanied by a distinct maturation expression of mGluR1 and mGluR5 during
the development of the MVN.
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