Effectiveness of conservative non-pharmacological interventions in people with muscular dystrophies: a systematic review and meta-analysis

Abstract

Introduction: Management of muscular dystrophies (MD) relies on conservative non-pharmacological treatments, but evidence of their effectiveness is limited and inconclusive.

Objective: To investigate the effectiveness of conservative non-pharmacological interventions for MD physical management.

Methods: This systematic review and meta-analysis followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines and searched Medline, CINHAL, Embase, AMED and Cochrane Central Register of Controlled Trial (inception to August 2022). Effect size (ES) and 95% Confidence Interval (CI) quantified treatment effect.

Results: Of 31,285 identified articles, 39 studies (957 participants), mostly at high risk of bias, were included. For children with Duchenne muscular dystrophy (DMD), trunk-oriented strength exercises and usual care were more effective than usual care alone in improving distal upper-limb function, sitting and dynamic reaching balance (ES range: 0.87 to 2.29). For adults with Facioscapulohumeral dystrophy (FSHD), vibratory proprioceptive assistance and neuromuscular electrical stimulation respectively improved maximum voluntary isometric contraction and reduced pain intensity (ES range: 1.58 to 2.33). For adults with FSHD, Limb-girdle muscular dystrophy (LGMD) and Becker muscular dystrophy (BMD), strength-training improved dynamic balance (sit-to-stand ability) and self-perceived physical condition (ES range: 0.83 to 1.00). A multicomponent programme improved perceived exertion rate and gait in adults with Myotonic dystrophy type 1 (DM1) (ES range: 0.92 to 3.83).

Conclusions: Low-quality evidence suggests that strength training, with or without other exercise interventions, may improve perceived exertion, distal upper limb function, static and dynamic balance, gait and well-being in MD. Although more robust and larger studies are needed, current evidence supports the inclusion of strength training in MD treatment, as it was found to be safe.

Figure 1

Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) flow chart of the study selection process. CBT, Cognitive Behavioural Therapy.

Figure 2

Risk of bias summary for randomised controlled trials only.

Figure 3

Sunburst chart of the characteristics of the included studies. AFO, Ankle Foot Orthosis; BMD, Becker Muscular Dystrophy; CBT, Cognitive Behavioural Therapy; CMD, Congenital Myotonic Dystrophy; DM1, Myotonic Dystrophy type 1; DM2, Myotonic Dystrophy type 2; DMD, Duchenne Muscular Dystrophy; FES, Functional Electrical Stimulation; FO; Foot Orthosis; FSHD, Facioscapulohumeral Dystrophy; HVPGS, High Voltage Pulsed Galvanic Stimulation; KAFO, Knee Ankle Foot Orthosis; LGMD, Limb Girdle Muscular Dystrophy; LGMD2l, Limb Girdle Muscular Dystrophy type 2l; MD, Muscular Dystrophy; NMES, Neuromuscular Electrical Stimulation; VPA, Vibratory Proprioceptive Assistance; WBVT, Whole-Body Vibration Training.

Figure 4

Scatter plots of the effect sizes of the included conservative non-pharmacological interventions. A&P, Activity and Participation; AFO/FO, Ankle Foot Orthosis/Foot Orthosis; AROM, Active Range of Motion; BF, Body Function; BMD, Becker Muscular Dystrophy; BS, Body Structure; CBT, Cognitive Behavioural Therapy; CMD, Congenital Muscular Dystrophy; DM1, Myotonic Dystrophy type 1; DM2, Myotonic Dystrophy type 2; DMD, Duchenne Muscular Dystrophy; ES, Effect Size; FES, Functional Electrical Stimulation; FSHD, Facioscapulohumeral Muscular Dystrophy; HPVGS, High Volt Pulsed Galvanic Stimulator; KAFO, Knee Ankle Foot Orthosis; LGMD, Limb-Girdle Muscular Dystrophy; MD, Muscular Dystrophy; NMES, Neuromuscular Electrical Stimulation; PROM, Passive Range Of Motion; QoL, Quality of Life; VPA, Vibratory Proprioceptive Assistance; WBVT, Whole-Body Vibration Training.

Figure 5

Forest plot of the significant effect sizes in the included studies. ES, Effect Size; MVIC, Maximum Voluntary Isometric Contraction; NMES, Neuromuscular Electrical Stimulation; PSPP-R, Physical Self-Perception Profile Revised; PUL, Performance of Upper Limb; RPE, Rating of Perceived Exertion; STS, Sit To Stand; TCMS, Trunk Control Measurement Scale; VAS, Visual Analogue Scale; VPA, Vibratory Proprioceptive Assistance.