Elsevier

Gait & Posture

Volume 62, May 2018, Pages 247-261
Gait & Posture

Gait deviations in Duchenne muscular dystrophy—Part 1. A systematic review

https://doi.org/10.1016/j.gaitpost.2018.03.020Get rights and content

Highlights

  • We conducted a systematic literature review to map gait deviations in DMD.

  • Only nine articles analyzed gait deviations in children with DMD.

  • Generalizability of these nine papers is poor.

  • Chances of a type I error are high in six of the nine included articles.

  • There is need for standardized measurement- and analyses methods.

Abstract

Background

Although prolonged ambulation is considered important in children with Duchenne muscular dystrophy (DMD), articles describing gait deviations in DMD are scarce.

Research question

Therefore, our research questions were the following: 1) what are the most consistently reported spatiotemporal-, kinematic-, kinetic-, and muscle activity deviations in children with DMD in literature, 2) what is the quality of the studies describing these deviations, and 3) is there need for further research?

Methods

We conducted a systematic literature search for studies published before the end of June 2017 in six online databases. We created a data extraction form to define information on materials and methods and on the analyzed gait parameters for each paper included in the review. If enough information was available, we calculated standardized mean differences (SMDs).

Results

The search yielded nine articles, but generalizability was poor. Seventy-nine parameters were analyzed by seven research groups, but they only agreed on a decrease in walking speed (minimal SMD: 1.26), stride length (1.83), step length (1.80), dorsiflexion during swing (1.43), maximal power generation at the hip (0.92), maximal knee extension torque (0.99), maximal dorsiflexion torque (−1.30), and maximal power generation at the ankle (0.92), and an increased knee range of motion (−0.82) in DMD.

Significance

In order to keep children with DMD ambulant as long as possible, a clear understanding of their pathological gait pattern is necessary. However, gait deviations in DMD appear not well defined. Previous studies appear to be of an exploratory nature while using predefined gait parameters to assess an undirected null hypothesis. This made them prone to regional focus bias, thereby increasing the chance of a type I error. Therefore, further research is required to define the altered gait pattern in children with DMD.

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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