Gait deviations in Duchenne muscular dystrophy—Part 1. A systematic review
Introduction
Duchenne muscular dystrophy (DMD) is the most common of muscular dystrophies affecting one in 3500–5000 boys that are born [1]. DMD is caused by a defective gene on the X-chromosome, which codes for the protein dystrophin. Dystrophin is expected to play an important role in “the stability of the muscle cell membrane in protecting the muscle fibers from contraction induced damage” [2]. When damage finally gets the overhand, muscles will predominantly consist of fibrofatty tissue [1]. So far, no cure has been found, and the affected children usually die in their third or fourth decade due to cardiac failure or pulmonary infections [[1], [3]]. One of the treatment goals in children with DMD is to keep them ambulant as long as possible, aiming to preserve a clinically important function and to postpone spinal deformities and muscle contractures [[1], [4]]. To achieve this goal, the altered gait pattern of children with DMD needs to be delineated and the potential underlying causes of their gait deviations need to be specified.
While 3D gait analysis to assess walking performance is part of the standard clinical care in children with neuromotor problems, such as cerebral palsy [[5], [6]], this evaluation procedure is less often applied in children with DMD. In a clinical setting, walking ability in DMD is generally evaluated by means of the 6 min walk test [7], which is also used to assess treatment effect in clinical trials [8]. Although the 6 min walk test has been found to be a valid general measurement tool [[7], [8]], it does not allow for a detailed analysis of joint kinematics, −kinetics, or muscle activity patterns of gait.
In 1981, Sutherland et al. were one of the first researchers using motion analysis to objectively quantify gait deviations in children with DMD [4]. They determined that three kinematic features could quantify disease progression: increased pelvic tilt, decreased dorsiflexion angle during swing and decreased cadence [4]. More recent studies used 3D motion analysis systems to study the gait pattern in children with DMD thereby calculating several spatiotemporal-, kinematic-, kinetic-, and/or muscle activity parameters [[9], [10], [11], [12], [13], [14], [15], [16]]. However, their results are difficult to compare, due to differences in measurement methods, data analysis procedures and extracted parameter sets [[9], [10], [11], [12], [13], [14], [15], [17]]. Hence, even though prolonged ambulation is considered important in children with DMD [1], their gait deviations appear not well-defined.
Therefore, the goals of part 1 of this research project were to create an overview of published studies that applied motion analysis to objectively quantify gait deviations in children with DMD, to provide a summary of the alterations in spatiotemporal-, kinematic-, kinetic- and muscle activity parameters reported in literature, and to assess the quality of the included studies. To meet this goal, we conducted a systematic literature search for studies published before the end of June 2017, based on exploration of six online databases.
Section snippets
Literature search
First, the PICO (Population, Intervention, Comparison and Outcome) tool was applied to assist with forming relevant keywords to conduct the systematic search [18]. Based on this PICO, the following medical subheadings (MeSH) were chosen: “muscular dystrophy, Duchenne” and “gait”. If usage of MeSH-terms was not allowed, the following keywords were used: “Duchenne” or “Duchenne’s”; combined with “dystrophy” or “muscular dystrophy” or “disease”. Also; only the abbreviation “DMD” was applied. As a
Literature search
No systematic reviews, describing gait deviations in children with DMD, were found in the online databases. Based on title and abstract and after removal of duplicates, the systematic search yielded 16 articles [[4], [9], [10], [11], [12], [13], [14], [15], [16], [17], [21], [32], [33], [34], [35], [36]]. All 16 articles could be obtained via the online databases or through the library of the KU Leuven. Of these 16 articles, seven articles were excluded. Reasons for exclusion were: one article
Discussion
The aim of this systematic review was to summarize the gait deviations in children with DMD reported in literature, to assess the quality of the included studies, and to analyze if there is need for further research. Our systematic literature search yielded nine eligible articles of which three were of the same research group. Generalizability of the results reported in these nine studies was poor, due to an insufficient description of the study population and measurement devices. Also, a
Conclusion
Although sustained walking ability is considered important in DMD, there is limited agreement on how DMD gait deviates from a typical gait pattern concerning spatiotemporal-, kinematic-, kinetic-, and muscle activity parameters. Due to the discrepancies between the nine included studies there remains a substantial lack of generalizability of the outcomes. Therefore, for future studies analyzing gait deviations in children with DMD, it is important that the materials and methods (including
Conflicts of interest
There were no conflicts of interest.
Acknowledgements
MG was funded by the MD-Paedigree project: A Model-Driven Paediatric European Digital Repository, partially funded by the European Commission under P7-ICT-2011-9 program (600932). MG and MVH were both funded by the Rondou Foundation (https://rf4duchenne.org/).
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Ankle contractures and functional motor decline in Duchenne muscular dystrophy
2022, Brain and DevelopmentProgression of muscular co-activation and gait variability in children with Duchenne muscular dystrophy: A 2-year follow-up study
2020, Clinical BiomechanicsCitation Excerpt :This study deeply investigated the function of co-activation in patients with DMD, highlighting the relationship among muscle co-activation during gait and disability, gait performance and postural stability. Like spatio-temporal and kinetic parameters, muscle activity indicators may also depend on variability associated with differing processing or recording factors (Goudriaan et al., 2018a, 2018b). In this study, the choice of processing parameters, such as the cut-off frequency or the technique for evaluating muscle co-activation, was based on a preliminary study on a smaller sample (Rinaldi et al., 2018).
Evaluation of gait in Duchenne Muscular Dystrophy: Relation of 3D gait analysis to clinical assessment
2019, Neuromuscular DisordersCitation Excerpt :Indeed, several discrepancies emerged in main joint kinematics of patients when compared to typically-developed age-matched controls [5,21–26]. Goudriaan et al. [20] recently reviewed the wide number of variables obtained from 3D gait analysis that differentiates the gait of children with DMD from typically-developed individuals. In particular, the reduced stride length and increased step width were indicated [24] as significant spatiotemporal parameters in this population.
Gait deviations in Duchenne muscular dystrophy—Part 2. Statistical non-parametric mapping to analyze gait deviations in children with Duchenne muscular dystrophy
2018, Gait and PostureCitation Excerpt :Our hypotheses concerning the first research question were partly confirmed. The children with DMD showed a significant increase in knee flexion angle during swing in the children with DMD, which could have contributed to the increased range of motion at the knee [1]. This increased knee flexion during swing could be a compensation for a decreased dorsiflexion during swing to ensure foot clearance.