Muscle weakness has a limited effect on motor control of gait in Duchenne muscular dystrophy

Vandekerckhove, Ines; Beukelaer, Nathalie De; Hauwe, M. van den; Shuman, Benjamin R.; Steele, Katherine M.; Campenhout, Anja Van; Goemans, Nathalie; Desloovere, Kaat; Goudriaan, Marije

doi:10.1371/journal.pone.0238445

Cited by 12 publications

(9 citation statements)

References 60 publications

(121 reference statements)

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“…In the DMD cohort, the torque deficits ranged from 41.4% for PF to 80.3% for HE (Supplementary Table S2). Similar DF and KF strength deficits were found in previous literature, while the PF strength deficit was smaller in the current study (Mathur et al, 2010;Lerario et al, 2012;Wokke et al, 2014;Goudriaan et al, 2018b;Vandekerckhove et al, 2020). The KE torque deficit from the current study was in agreement with previously reported results from our research group (Goudriaan et al, 2018b;Vandekerckhove et al, 2020).…”

Section: Part Two: Validitysupporting

confidence: 93%

“…Similar DF and KF strength deficits were found in previous literature, while the PF strength deficit was smaller in the current study (Mathur et al, 2010;Lerario et al, 2012;Wokke et al, 2014;Goudriaan et al, 2018b;Vandekerckhove et al, 2020). The KE torque deficit from the current study was in agreement with previously reported results from our research group (Goudriaan et al, 2018b;Vandekerckhove et al, 2020). While larger deficits for the KE strength have been found by other research groups using different strength assessments (Mathur et al, 2010;Lerario et al, 2012;Wokke et al, 2014).…”

Section: Part Two: Validitysupporting

confidence: 92%

“…The required sample size was estimated based on previous research ( Goudriaan et al, 2018a ; Vandekerckhove et al, 2020 ). The Wilcoxon effect sizes r ( Fritz et al, 2012 ) of the differences in muscle strength between CP and TD were calculated from the available data of Goudriaan et al (2018a) and ranged between 0.55 and 0.80 ( Goudriaan et al, 2018a ).…”

Section: Methodsmentioning

confidence: 99%

“…The Wilcoxon effect sizes r (Fritz et al, 2012) of the differences in muscle strength between CP and TD were calculated from the available data of Goudriaan et al (2018a) and ranged between 0.55 and 0.80 (Goudriaan et al, 2018a). Vandekerckhove et al (2020) reported similar Wilcoxon effect sizes r ranging between 0.65 and 0.79 for differences in muscle strength between DMD and TD (Vandekerckhove et al, 2020). Taking into account a Cohen's D effect size of 1.3, which corresponds to the smallest Wilcoxon effect size r (0.55) reported in previous studies, an α error probability of 0.0036 (to correct for the comparison of 14 parameters) and a power of 0.95, a minimal sample size of 26 for each cohort was required.…”

Section: Participantsmentioning

confidence: 99%

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Instrumented strength assessment in typically developing children and children with a neural or neuromuscular disorder: A reliability, validity and responsiveness study

Verreydt

Vandekerckhove²,

Stoop³

et al. 2022

Front. Physiol.

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The aim of this study was to determine the clinimetric properties, i.e., reliability, validity and responsiveness of an instrumented strength assessment in typically developing (TD) children and children with cerebral palsy (CP) and Duchenne muscular dystrophy (DMD). Force (N), torque (Nm) and normalized torque (Nm/kg) were defined for maximal voluntary isometric contractions (MVICs) of the lower limb muscles using a pre-established protocol. Intraclass correlation coefficient (ICC), standard error of measurement (SEM) and minimal detectable change (MDC) of TD children (n = 14), children with CP (n = 11) and DMD (n = 11) were used to evaluate intra-rater reliability for the three cohorts and the inter-rater intersession as well as inter-rater intrasession reliability for TD children. Construct validity was assessed by comparing MVICs in TD children (n = 28) to children with CP (n = 26) and to children with DMD (n = 30), using the Kruskal Wallis and post-hoc Mann-Whitney U tests. Responsiveness was investigated by assessing changes in MVICs following a strength intervention in CP (n = 26) and a 1 and 2 year follow-up study in DMD (n = 13 and n = 6, respectively), using the Wilcoxon Signed-Rank test. The overall intra-rater reliability, was classified as good to excellent for 65.1%, moderate for 27.0% and poor for 7.9% of the measures (47.6%, 76.2%, and 66.7% good-excellent; 28.6%, 23.8%, and 33.7% moderate; 23.8%, 0%, and 0% poor in TD, CP, and DMD, respectively), while ICC values for TD children were slightly lower for inter-rater intrasession reliability (38.1% good-excellent, 33.3% moderate and 26.6% poor) and for inter-rater intersession reliability (47.6% good-excellent, 23.8% moderate and 28.6% poor). Children with CP and DMD were significantly weaker than TD children (p < 0.001) and the majority of these strength differences exceeded the MDC. Children with CP significantly improved strength after training, with changes that exceeded the SEMs, whereas only limited strength decreases over time were observed in the DMD cohort. In conclusion, the investigated instrumented strength assessment was sufficiently reliable to confirm known-group validity for both cohorts and could detect the responsiveness of children with CP after a strength intervention. However, more research is necessary to determine the responsiveness of this assessment in children with DMD regarding their natural decline.

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Section: Part Two: Validitysupporting

confidence: 93%

Section: Part Two: Validitysupporting

confidence: 92%

Section: Methodsmentioning

confidence: 99%

Section: Participantsmentioning

confidence: 99%

See 2 more Smart Citations

Instrumented strength assessment in typically developing children and children with a neural or neuromuscular disorder: A reliability, validity and responsiveness study

Verreydt

Vandekerckhove²,

Stoop³

et al. 2022

Front. Physiol.

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“…The typical clinical sign is muscular weakness which influences walking ability. According to the few studies focusing on the relation between muscle weakness and motor control, muscle synergies do not change significantly between the DMD group and the control group ( Goudriaan et al,2018 ; Vandekerckhove et al, 2020 ).…”

Section: Introductionmentioning

confidence: 97%

Recent developments in muscle synergy analysis in young people with neurodevelopmental diseases: A Systematic Review

Beltrame

Scano

Marino

et al. 2023

Front. Bioeng. Biotechnol.

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The central nervous system simplifies motor control by sending motor commands activating groups of muscles, known as synergies. Physiological locomotion can be described as a coordinated recruitment of four to five muscle synergies. The first studies on muscle synergies in patients affected by neurological diseases were on stroke survivors. They showed that synergies can be used as biomarkers for motor impairment as they vary in patients with respect to healthy people. Likewise, muscle synergy analysis has been applied to developmental diseases (DD). The need for a comprehensive view of the present findings is crucial for comparing results achieved so far and promote future directions in the field. In the present review, we screened three scientific databases and selected thirty-six papers investigating muscle synergies extracted from locomotion in children affected by DD. Thirty-one articles investigate how cerebral palsy (CP) influences motor control, the currently exploited method in studying motor control in CP and finally the effects of treatments in these patients in terms of synergies and biomechanics; two articles investigate how muscle synergies vary in Duchenne muscular dystrophy (DMD), and three other articles assess other developmental pathologies, such as chronic and acute neuropathic pain. For CP, most of the studies demonstrate that the number of synergies is lower and that the synergy composition varies in the affected children with respect to normal controls. Still, the predictability of treatment’s effects and the etiology of muscle synergy variation are open questions, as it has been reported that treatments minimally modify synergies, even if they improve biomechanics. The application of different algorithms in extracting synergies might bring about more subtle differences. Considering DMD, no correlation was found between non-neural muscle weakness and muscle modules’ variation, while in chronic pain a decreased number of synergies was observed as a possible consequence of plastic adaptations. Even if the potential of the synergistic approach for clinical and rehabilitation practices is recognized, there is not full consensus on protocols nor widely accepted guidelines for the systematic clinical adoption of the method in DD. We critically commented on the current findings, on the methodological issues and the relative open points, and on the clinical impact of muscle synergies in neurodevelopmental diseases to fill the gap for applying the method in clinical practice.

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Assessment of Motor Unit Potentials Duration as the Biomarker of DT-DEC01 Cell Therapy Efficacy in Duchenne Muscular Dystrophy Patients up to 12 Months After Systemic–Intraosseous Administration

Niezgoda,

Biegański,

Wachowiak

et al. 2023

Arch. Immunol. Ther. Exp.

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Duchenne muscular dystrophy (DMD) is a lethal X-linked disease caused by mutations in the dystrophin gene, leading to muscle degeneration and wasting. Electromyography (EMG) is an objective electrophysiological biomarker of muscle fiber function in muscular dystrophies. A novel, DT-DEC01 therapy, consisting of Dystrophin Expressing Chimeric (DEC) cells created by fusing allogeneic myoblasts from normal donors with autologous myoblasts from DMD-affected patients, was assessed for safety and preliminary efficacy in boys of age 6–15 years old (n = 3). Assessments included EMG testing of selected muscles of upper (deltoideus, biceps brachii) and lower (rectus femoris and gastrocnemius) extremities at the screening visit and at 3, 6, and 12 months following systemic–intraosseous administration of a single low dose of DT-DEC01 therapy (Bioethics Committee approval no. 46/2019). No immunosuppression was administered. Safety of DT-DEC01 was confirmed by the lack of therapy-related Adverse Events or Serious Adverse Events up to 22 months following DT-DEC01 administration. EMG of selected muscles of both, ambulatory and non-ambulatory patients confirmed preliminary efficacy of DT-DEC01 therapy by an increase in motor unit potentials (MUP) duration, amplitudes, and polyphasic MUPs at 12 months. This study confirmed EMG as a reliable and objective biomarker of functional assessment in DMD patients after intraosseous administration of the novel DT-DEC01 therapy.

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Muscle weakness has a limited effect on motor control of gait in Duchenne muscular dystrophy

Cited by 12 publications

References 60 publications

Instrumented strength assessment in typically developing children and children with a neural or neuromuscular disorder: A reliability, validity and responsiveness study

Instrumented strength assessment in typically developing children and children with a neural or neuromuscular disorder: A reliability, validity and responsiveness study

Recent developments in muscle synergy analysis in young people with neurodevelopmental diseases: A Systematic Review

Assessment of Motor Unit Potentials Duration as the Biomarker of DT-DEC01 Cell Therapy Efficacy in Duchenne Muscular Dystrophy Patients up to 12 Months After Systemic–Intraosseous Administration

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