A Cochrane review [Abstract] 1 [withdrawn from publication] included 4 studies with a total of 152 subjects. One study included patients with Charcot-Marie-Tooth (CMT) disease, one patients with either myotonic dystrophy or CMT disease, one study boys with Duchenne muscular dystrophy and one adults with facioscapulohumeral muscular dystrophy. Interventions included strength training, orthoses and surgery. The primary outcome measure was quantified ability to walk.In a trial with CMT disease (n=26) night splinting of the ankle did not significantly affect muscle force or range of movement of ankle. In a trial with CMT (n=26) and myotonic dystrophy patients (n=28) 24 weeks of strength training significantly improved 6-meter timed walk in the CMT group compared to the control group (mean difference (MD) 0.70 seconds, favouring strength training, 95% CI 0.23 to 1.17), but not in the myotonic dystrophy group (MD -0.20 seconds, favouring the control group, 95% CI -0.79 to 0.39). No significant differences were observed for the 50 meter timed walk in the CMT disease group (MD 1.90 seconds, favouring the training group, 95% CI -0.29 to 4.09) or the myotonic dystrophy group (MD -0.80 seconds, favouring the control group, 95% CI -5.29 to 3.69). In a trial with facioscapulohumeral muscular dystrophy (n=65), 26 weeks of strength training did not affect ankle strength. After one year, MD in maximum voluntary isometric contraction was -0.43 kg, favouring the control group (95%CI -2.49 to 1.63) and MD in dynamic strength was 0.44 kg, favouring the training group (95%CI -0.89 to 1.77). Early surgery did not significantly affect walking speed in a trial including 20 children with Duchenne muscular dystrophy; after one year MD of the 28 feet walking time was 0.00 seconds (95% CI -0.83 to 0.83) and the MD of the 150 feet walking time was -2.88 seconds, (95% CI -8.18 to 2.42).
Comment: The quality of evidence is downgraded by study quality (inadequate allocation concealment), inconsistency (heterogeneity in patients, interventions and outcomes) and imprecise results(limited study size for each comparison).
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