Summary of findings 2. Strength training compared to no training for facioscapulohumeral muscular dystrophy.
| Strength training compared to no training for facioscapulohumeral muscular dystrophy | ||||||
| Patient or population: people with facioscapulohumeral muscular dystrophy Setting: at home Intervention: strength training Comparison: without strength training | ||||||
| Outcomes | Mean (SD) without strength training | Mean (SD) with strength training | Difference (95% CI) | Certainty of the evidence (GRADE) | What happens | |
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Muscle strength: maximum voluntary isometric contraction Assessed with Quantitative Muscle Assessment fixed myometry testing system Follow‐up: mean 52 weeks 35 participants (1 RCT) |
Elbow flexors ‐ maximum voluntary isometric contraction | The mean change in maximum voluntary isometric contraction of the elbow flexors without strength training was a decrease of 0.6 (1.9) kg | The mean change in maximum voluntary isometric contraction of the elbow flexors with strength training was a decrease of 0.1 (1.9) kg | MD 0.5 kg higher (0.7 lower to 1.8 higher) | ⊕⊕⊝⊝ Lowa,b | May be little or no effect on isometric muscle strength of elbow flexors |
| Elbow flexors ‐ dynamic strength | The mean change in dynamic strength of the elbow flexors without strength training was an increase of 1.4 (2.0) kg | The mean change in dynamic strength of the elbow flexors with strength training was an increase of 2.5 (2.1) kg | MD 1.2 kg higher (0.2 lower to 2.6 higher) | ⊕⊕⊝⊝ Lowa,b | May be little or no effect on dynamic muscle strength of elbow flexors | |
| Ankle dorsiflexors ‐ maximum isometric voluntary contraction | The mean change in maximum isometric voluntary contraction of the ankle dorsiflexors without strength training was a decrease of 1.6 (4.2) kg | The mean change in maximum isometric voluntary contraction of the ankle dorsiflexors with strength training was a decrease of 1.1 (4.3) kg | MD 0.4 kg higher (2.4 lower to 3.2 higher) | ⊕⊕⊝⊝ Lowa,b | May be little or no effect on isometric muscle strength of ankle dorsiflexors | |
| Ankle dorsiflexors ‐ dynamic strength | The mean change in dynamic strength of the ankle dorsiflexors without strength training was a decrease of 11 (2.8) kg | The mean change in dynamic strength of the ankle dorsiflexors with strength training was a decrease of 1.5 (2.7) kg | MD 0.4 kg lower (2.3 lower to 1.4 higher) | ⊕⊕⊝⊝ Lowa,b | May be little or no effect on dynamic muscle strength of ankle dorsiflexors | |
| Elbow flexors ‐ muscle endurance | The mean change in muscle endurance of the elbow flexors without strength training was a decrease of 3.0 (35.5) kgF/s | The mean change in muscle endurance of the elbow flexors with strength training was a decrease of 11.0 (65.0) kgF/s | MD 8.0 kgF/s lower (42.0 lower to 26.0 higher) | ⊕⊕⊝⊝ Lowa,b | May be little or no effect on muscle endurance of elbow flexors | |
| Ankle dorsiflexors ‐ muscle endurance | The mean change in muscle endurance of the ankle dorsiflexors without strength training was a decrease of 29.0 (28.0) kgF/s | The mean change in muscle endurance of the ankle dorsiflexors with strength training was a decrease of 46.0 (25.3) kgF/s | MD 17.0 kgF/s lower (34.8 lower to 0.8 higher) | ⊕⊕⊝⊝ Lowa,b | May be little or no effect on muscle endurance of ankle dorsiflexors | |
| Aerobic capacity | No data were provided for this outcome | |||||
| Time‐scored functional assessments of muscle performance | No data were provided for this outcome | |||||
| Quality of life | No data were provided for this outcome | |||||
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Pain Follow‐up: mean 52 weeks 34 participants (1 RCT) |
11 out of 34 participants in the training group reported pain in neck and shoulder region to the physical therapist during his home visits. 5 mentioned a period with elbow complaints. The number of neck‐shoulder and elbow complaints did not differ between groups at baseline and at the final visit | ⊕⊕⊝⊝ Lowa,b | May be no effect on pain experience | |||
| Experienced fatigue | No data were provided for this outcome | |||||
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Adverse effects requiring withdrawal Follow‐up: mean 52 weeks 35 participants: 35 (1 RCT) |
1 participant stopped training because of recurring, training‐related muscle soreness and fatigue. She had a second diagnostic workup, revealing a mitochondrial myopathy as well as FSHD. The training programme was well tolerated. Participants experienced no notable general fatigue or muscle soreness. The training‐induced muscle fatigue lasted less than an hour, so daily activities could be carried out normally afterwards. | ⊕⊕⊝⊝ Lowa,b | May be few or no adverse effects requiring withdrawal | |||
| CI: confidence interval; MD: mean difference; RCT: randomised controlled trial; SD: standard deviation | ||||||
| GRADE Working Group grades of evidence High certainty: we are very confident that the true effect lies close to that of the estimate of the effect. Moderate certainty: we are moderately confident in the effect estimate: the true effect is likely to be close to the estimate of the effect, but there is a possibility that it is substantially different. Low certainty: our confidence in the effect estimate is limited: the true effect may be substantially different from the estimate of the effect. Very low certainty: we have very little confidence in the effect estimate: the true effect is likely to be substantially different from the estimate of effect. | ||||||
aDowngraded one level for study limitations: participants and personnel were not blinded, as blinding of participants and personnel was not possible. bDowngraded one level for imprecision: sample size of 35.