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Strength training and aerobic exercise training for muscle
disease (Review)
Voet NBM, van der Kooi EL, Riphagen II, Lindeman E, van Engelen BGM, Geurts ACH
This is a reprint of a Cochrane review, prepared and maintained by The Cochrane Collaboration and published in The Cochrane Library
2010, Issue 1
http://www.thecochranelibrary.com
Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
T A B L E O F C O N T E N T S
1HEADER . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
1ABSTRACT . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
2PLAIN LANGUAGE SUMMARY . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
2BACKGROUND . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
3OBJECTIVES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
3METHODS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
5RESULTS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Figure 1. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
Figure 2. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
Figure 3. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
Figure 4. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10
Figure 5. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
Figure 6. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
Figure 7. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
Figure 8. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11
Figure 9. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
Figure 10. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
Figure 11. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
Figure 12. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
Figure 13. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
Figure 14. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13
15DISCUSSION . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
17AUTHORS’ CONCLUSIONS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
18ACKNOWLEDGEMENTS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
18REFERENCES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
21CHARACTERISTICS OF STUDIES . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
29DATA AND ANALYSES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Analysis 1.1. Comparison 1 Strength training versus control in myotonic dystrophy, Outcome 1 Muscle strength -
maximum isotonic knee torque extension. . . . . . . . . . . . . . . . . . . . . . . . . 30
Analysis 1.2. Comparison 1 Strength training versus control in myotonic dystrophy, Outcome 2 Muscle strength -
maximum isotonic knee torque flexion. . . . . . . . . . . . . . . . . . . . . . . . . . 31
Analysis 1.3. Comparison 1 Strength training versus control in myotonic dystrophy, Outcome 3 Muscle strength -
maximum isometric voluntary contraction. . . . . . . . . . . . . . . . . . . . . . . . . 31
Analysis 2.1. Comparison 2 Strength training versus control in facioscapulohumeral muscular dystrophy, Outcome 1
Muscle strength elbow flexors - maximum voluntary isometric contraction. . . . . . . . . . . . . . 32
Analysis 2.2. Comparison 2 Strength training versus control in facioscapulohumeral muscular dystrophy, Outcome 2
Muscle strength elbow flexors - dynamic strength. . . . . . . . . . . . . . . . . . . . . . 32
Analysis 2.3. Comparison 2 Strength training versus control in facioscapulohumeral muscular dystrophy, Outcome 3
Muscle strength ankle dorsiflexors - maximum isometric voluntary contraction. . . . . . . . . . . . 33
Analysis 2.4. Comparison 2 Strength training versus control in facioscapulohumeral muscular dystrophy, Outcome 4
Muscle strength ankle dorsiflexors - dynamic strength. . . . . . . . . . . . . . . . . . . . . 33
Analysis 3.1. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 1 Muscle strength
shoulder press - maximum dynamic isotonic voluntary contraction. . . . . . . . . . . . . . . . 34
Analysis 3.2. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 2 Muscle strength
butterfly - maximum dynamic isotonic voluntary contraction. . . . . . . . . . . . . . . . . . 34
Analysis 3.3. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 3 Muscle strength
bicep curls - maximum isotonic dynamic voluntary contraction. . . . . . . . . . . . . . . . . 35
Analysis 3.4. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 4 Work capacity -
mean time until exhaustion in cycle test. . . . . . . . . . . . . . . . . . . . . . . . . 35
Analysis 3.5. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 5 Work capacity-
mean distance until exhaustion in cycle test. . . . . . . . . . . . . . . . . . . . . . . . 36
iStrength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Analysis 3.6. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 6 Work capacity -
mean distance walked until exhaustion in shuttle walking test. . . . . . . . . . . . . . . . . . 36
Analysis 3.7. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 7 Quality of
life. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 37
Analysis 3.8. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 8 Myoglobin. 37
37APPENDICES . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
38WHAT’S NEW . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
39HISTORY . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
39CONTRIBUTIONS OF AUTHORS . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
39DECLARATIONS OF INTEREST . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
39DIFFERENCES BETWEEN PROTOCOL AND REVIEW . . . . . . . . . . . . . . . . . . . . .
39INDEX TERMS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
iiStrength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
[Intervention Review]
Strength training and aerobic exercise training for muscle
disease
Nicoline BM Voet1, Elly L van der Kooi2, Ingrid I Riphagen3, Eline Lindeman4, Baziel GM van Engelen5, A CH Geurts1
1Department of Rehabilitation, Radboud University Nijmegen Medical Centre, Nijmegen Centre for Evidence Based Practice, Ni-
jmegen, Netherlands. 2Department of Neurology, Medical Centre Leeuwarden, Leeuwarden, Netherlands. 3Unit for Applied Clinical
Research, Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway. 4Rehabilitation Centre Utrecht
& University Medical Centre Utrecht, Utrecht, Netherlands. 5Neuromuscular Center Nijmegen, Radboud University Nijmegen Med-
ical Centre, Nijmegen, Netherlands
Contact address: Nicoline BM Voet, Department of Rehabilitation, Radboud University Nijmegen Medical Centre, Nijmegen Centre
for Evidence Based Practice, Huispost 898, P.O. Box 9101, Nijmegen, Gelderland, 6500 HB, Netherlands. N.Voet@reval.umcn.nl.
Editorial group: Cochrane Neuromuscular Disease Group.
Publication status and date: Edited (conclusions changed), published in Issue 1, 2010.
Review content assessed as up-to-date: 13 July 2009.
Citation: Voet NBM, van der Kooi EL, Riphagen II, Lindeman E, van Engelen BGM, Geurts ACH. Strength training and
aerobic exercise training for muscle disease. Cochrane Database of Systematic Reviews 2010, Issue 1. Art. No.: CD003907. DOI:
10.1002/14651858.CD003907.pub3.
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
A B S T R A C T
Background
Strength training or aerobic exercise programmes might optimise muscle and cardiorespiratory function and prevent additional disuse
atrophy and deconditioning in people with a muscle disease.
Objectives
To examine the safety and efficacy of strength training and aerobic exercise training in people with a muscle disease.
Search strategy
We searched the Cochrane Neuromuscular Disease Group Trials Specialized Register (July 2009), the Cochrane Rehabilitation and
Related Therapies Field Register (October 2002, August 2008 and July 2009), The Cochrane Central Register of Controlled Trials
(The Cochrane Library Issue 3, 2009) MEDLINE (January 1966 to July 2009), EMBASE (January 1974 to July 2009), EMBASE
Classic (1947 to 1973) and CINAHL (January 1982 to July 2009).
Selection criteria
Randomised or quasi-randomised controlled trials comparing strength training or aerobic exercise programmes, or both, to no training,
and lasting at least 10 weeks.
For strength training
Primary outcome: static or dynamic muscle strength. Secondary: muscle endurance or muscle fatigue, functional assessments, quality
of life, muscle membrane permeability, pain and experienced fatigue.
For aerobic exercise training
Primary outcome: aerobic capacity expressed as work capacity. Secondary: aerobic capacity (oxygen consumption, parameters of cardiac
or respiratory function), functional assessments, quality of life, muscle membrane permeability, pain and experienced fatigue.
1Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Data collection and analysis
Two authors independently assessed trial quality and extracted the data.
Main results
We included three trials (121 participants). The first compared the effect of strength training versus no training in 36 people with
myotonic dystrophy. The second trial compared strength training versus no training, both combined with albuterol or placebo, in 65
people with facioscapulohumeral muscular dystrophy. The third trial compared combined strength training and aerobic exercise versus
no training in 18 people with mitochondrial myopathy. In the myotonic dystrophy trial there were no significant differences between
training and non-training groups for primary and secondary outcome measures. In the facioscapulohumeral muscular dystrophy trial
only a +1.17 kg difference (95% confidence interval 0.18 to 2.16) in dynamic strength of elbow flexors in favour of the training group
reached statistical significance. In the mitochondrial myopathy trial there were no significant differences in dynamic strength measures
between training and non-training groups. Exercise duration and distance cycled in a submaximal endurance test increased significantly
in the training group compared to the control group.
Authors’ conclusions
In myotonic dystrophy and facioscapulohumeral muscular dystrophy, moderate-intensity strength training appears not to do harm but
there is insufficient evidence to conclude that it offers benefit. In mitochondrial myopathy, aerobic exercise combined with strength
training appears to be safe and may be effective in increasing submaximal endurance capacity. Limitations in the design of studies in
other muscle diseases prevent more general conclusions in these disorders.
P L A I N L A N G U A G E S U M M A R Y
Strength training or comprehensive aerobic exercise training for muscle disease
Strength training, which is performed to improve muscle strength and muscle endurance, or aerobic exercise programmes, which involve
training at moderate levels of intensity for extended periods of time (for example, distance cycling) might optimise physical fitness and
prevent additional muscle wasting in people with muscle disease. However, people with muscle disease and clinicians are still afraid of
overuse and have a cautious approach to training. This updated review included two eligible trials on strength training and one new
trial on strength training combined with aerobic exercise. These showed that moderate-intensity strength training appears not to harm
muscles in people with myotonic dystrophy or with facioscapulohumeral muscular dystrophy, and has a very limited positive effect on
muscle strength in facioscapulohumeral muscular dystrophy. Strength training combined with aerobic exercise appears to be safe and
may be effective in increasing endurance in people with mitochondrial myopathy. However, there is insufficient evidence for general
prescription of exercise programmes in these disorders. More research is needed in all muscle diseases.
B A C K G R O U N D
When a person is diagnosed as having a muscle disease, ques-
tions arise about the prognosis, possible interventions and genetics.
However, people with muscle disease are usually also concerned
about everyday issues such as participation in sports, work and
hobbies. We cannot give evidence-based advice about these issues
because we do not know how physical exercise affects the diseased
muscular system and the cardiorespiratory system. To answer these
questions controlled trials of aerobic exercise and strength training
in people with a muscle disease are needed.
Weakness and impaired cardiorespiratory function are common in
people with muscle disease. In healthy persons the best interven-
tion to improve strength and cardiorespiratory function is training.
Strength training or aerobic exercise programmes in people with
muscle disease might maximise muscle and cardiorespiratory func-
tion and prevent additional disuse atrophy (Vignos 1983). How-
ever, reports of weakness after exercise in people with myopathies
have encouraged a cautious approach to training. The refore, many
people with a muscle disease were advised to avoid physical exer-
tion (Brouwer 1992;Fowler 1982;Fowler 1984;Johnson 1971).
The relative rarity of some muscle diseases has led many researchers
2Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
to group participants with different neuromuscular disorders to-
gether, including myopathies, neuropathies and motor neuron
disease (Aitkens 1993;Dawes 2006;Kilmer 1994;Kilmer 2005;
McCartney 1988;Milner-Brown 1988a;Milner-Brown 1988b;
Wright 1996). As the pathophysiology of these disorders differs,
their reaction to an intervention might be different. Therefore,
conclusions about the effect of training derived from these mixed
populations cannot readily be extrapolated to people with specific
muscular disorders (Lindeman 1995).
The benefit from strength training or aerobic exercise training
in muscle diseases is still not clear (Kilmer 1998), therefore in
this update we systematically reviewed controlled trials of these
interventions for people with specified muscle diseases.
O B J E C T I V E S
The objective was to systematically review the evidence from ran-
domised controlled trials concerning the efficacy and safety of
strength training and aerobic exercise training in people with mus-
cle disease.
M E T H O D S
Criteria for considering studies for this review
Types of studies
We included all randomised or quasi-randomised controlled trials
that made any of the following comparisons:
•strength training versus no training;
•aerobic exercise training versus no training;
•combined strength training and aerobic exercise versus no
training.
Types of participants
We selected all trials including participants with a well-described
diagnosis of a muscle disease, such as inflammatory myopathies,
metabolic myopathies, muscular dystrophies, muscle diseases with
myotonia and other well-defined myopathies. We decided not to
include studies looking at strength training or aerobic exercise
training for people in whom muscle weakness was not the primary
feature, but might have been secondary to chronic renal insuffi-
ciency, chronic heart failure, renal or heart transplantation, or cor-
ticosteroid use. We did not review the effects of respiratory muscle
training.
Types of interventions
We included all forms of strength training and aerobic exercise
training lasting at least 10 weeks.
Definitions
•Training, or physical fitness training: a planned, structured
regimen of regular physical exercise deliberately performed to
improve one or more of the following components of physical
fitness: cardiorespiratory fitness, body composition, muscle
strength and endurance, and flexibility (Pollock 1998;
USDHHS 1996).
•Strength training: training performed primarily to improve
muscle strength and endurance. It is typically carried out making
repeated muscle contractions against resistance (Saunders 2004).
•Aerobic exercise training, or cardiorespiratory fitness
training: training that consists of an activity or combination of
activities that uses large muscle groups, that can be maintained
continuously, and that is rhythmical and aerobic in nature, for
example walking-hiking, running-jogging, cycling-bicycling,
aerobic dance exercise or swimming (Pollock 1998).
Types of outcome measures
Primary outcomes
The primary outcome measure for strength training was:
•muscle strength, expressed in measures of static (i.e.
isometric) or dynamic strength.
The secondary outcome measure specific to strength training was:
•muscle endurance or muscle fatigue.
The primary outcome measure for aerobic exercise training was:
•aerobic capacity, expressed in measures of work capacity.
The secondary outcome measure specific to aerobic exercise train-
ing was:
•aerobic capacity, expressed in measures of oxygen
consumption, parameters of cardiac function or parameters of
respiratory function.
Secondary outcomes
Secondary outcome measures applicable to both strength training
and aerobic exercise training were:
•timed-scored functional assessments of muscle performance;
•quality of life;
•parameters of muscle membrane permeability (serum
creatine kinase level, myoglobin level) to assess the safety;
•experienced pain;
•experienced fatigue.
3Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
We compared data on outcome measures at baseline with those
obtained after at least 10 weeks of training. When there were as-
sessments at more than one time (during the intervention, after
cessation of the intervention), our preference was for data on out-
come measures obtained at the end of the intervention.
Search methods for identification of studies
We searched the Cochrane Neuromuscular Disease Group Trials
Specialized Register for randomised trials (14 July 2009) using
the following search terms: “muscle dis*” or “muscle weakness”
or “muscular dis*” or “neuromuscular dis*” or “myopath*” or
“dystroph*” or “myotoni*” or “myositis” or “polio*” or “muscle
fibre*” or “muscle strength” and “exercise therapy” or “exercise
training” or “exercise program*” or“strength training” or “aerobic
training” or “aerobic exercise” or “training program” or “resistive
exercise” or “endurance training” or “muscle exercise”.
One of the authors (IIR) designed and ran similar search strate-
gies in The Cochrane Central Register of Controlled Trials (The
Cochrane Library Issue 3, 2009), MEDLINE (January 1966 to
July 2009), EMBASE (January 1974 to July 2009), EMBASE
Classic (1947 to 1973) and CINAHL (January 1982 to July 2009).
The Cochrane Rehabilitation and Related Therapies Field helped
to search the literature in the rehabilitation and physical therapy
field (database and handsearched material) (August 2008). We re-
viewed the bibliographies of the trials identified and other reviews
covering the subject, and contacted some of the authors in the
field to identify additional published and unpublished data. As
the search methods used in this updated review have not been
changed, we also included studies from the previous version of this
review.
Data collection and analysis
Selection of studies
Two authors ( Voet, van der Kooi) checked th e referencesidentified
by the search strategy.The full text of all potentially relevant studies
was obtained for independent assessment by both authors. We
decided which trials fitted the inclusion criteria.
Data extraction and management
We independently extracted the data from the included trials onto
a specially designed data extraction form, and graded the method-
ological quality and certain other aspects of the design of the in-
cluded trials.
Assessment of risk of bias in included studies
Aspects of trial design that we assessed were as follows.
A. Diagnostic criteria. We used the Diagnostic Criteria for Neu-
romuscular Disorders as defined by the European Neuromuscular
Centre (ENMC) as a guide (Emery 1997).
B. Design of the training programme - because there are no evi-
dence based prescriptions of exercise programmes for people with
a muscle disease, we used the recommendations from the Ameri-
can College of Sports Medicine (ACSM) Position Stand on ’The
Recommended Quantity and Quality of Exercise for Developing and
Maintaining Cardiorespiratory and Muscular Fitness, and Flexibility
in Healthy Adults’ as minimal requirements to evaluate the quality
of the training programmes (Pollock 1998).
•For effective strength training the ACSM recommendations
are that it should be an individualised programme, which is
progressive in nature, and which provides a stimulus to all the
major muscle groups. At least one set of eight to ten exercises
should condition the major muscle groups two to three days per
week. Most persons should complete eight to 12 repetitions of
each exercise but older or frail persons should do 10 to 15
repetitions with lower resistance. In addition, we required that
the type of strength training exercise, i.e. static (isometric) or
dynamic (concentric, excentric, isokinetic) should be indicated.
We also required that the duration of an exercise session should
not exceed 60 minutes, in order not to interfere too much with
other daily activities. The load required to increase maximal
strength in untrained individuals is fairly low. Loads of 45% to
50% of the one repetition maximum (1RM), and less, have been
shown to increase dynamic muscular strength in previously
untrained individuals (Kraemer 2002).
•For aerobic exercise training the ACSM states that the
mode of activity can be any activity that uses large muscle
groups, which can be maintained continuously, and is rhythmic
and aerobic in nature. The optimal frequency of training is three
to five days per week. Intensity of training should be at 55% to
90% of maximum heart rate, or 40% to 85% of maximum
oxygen uptake reserve or maximum heart rate reserve. The
duration of training should be 20 to 60 minutes continuously or
intermittently in bouts of at least 10 minutes.
•Based on what is known from adaptations of strength
training in healthy individuals, we decided that the entire
programme duration should be at least 10 weeks to be able to
detect training effects based on both neural adaptation (which
has its maximum contribution in first four to six weeks) and
muscle hypertrophy (which has its main contribution after six
weeks).
•The type of exercise supervision was also evaluated, because
the regular supervision of training improves the effect and safety
of exercise, and the compliance of the participants.
We described and ranked the following qualities of the training
programmes: type of exercise training, intensity, frequency, dura-
4Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
tion per exercise session, duration of the entire programme, (num-
ber of) trained muscle groups, supervision of training.
C. Methodological quality - we assessed risk of bias and other
aspects according to the Cochrane approach using the updated
guidance in the Cochrane Handbook for Systematic Reviews of
Interventions (Higgins 2008). We assessed the included studies
for randomisation sequence generation, allocation concealment,
blinding (participants and outcome assessors), incomplete out-
come data, selective outcome reporting and other sources of bias.
When there was uncertainty, authors were contacted for clarifica-
tion. We resolved disagreement about fulfilment of inclusion or
quality criteria by discussion between the two authors. We made
a judgement on each of these criteria relating to the risk of bias,
such that a judgement of ’yes’ indicated a low risk of bias, ’no’
a high risk of bias and ’unclear’ an unclear or unknown risk of
bias. Whenever characteristics of study design or drop-out rates
were likely to cause a higher risk of bias, this would be noted and
the possibility of differences in treatment effects varying with the
degree of this problem would be investigated.
Data synthesis
We intended to combine trial results for appropriate pairings of
treatments by calculating a weighted mean of the difference be-
tween their effects using the Cochrane statistical package Review
Manager 5.0 (RevMan) (RevMan 2008). Because pooling of the
results of trials on different muscle diseases is usually not appro-
priate, we expressed, when possible, the results per muscle disease
as mean differences (WMD) with 95% confidence intervals (95%
CI) for continuous outcomes, and risk ratios (RR) with 95% CI
for dichotomous outcome measures. The intended testing for het-
erogeneity, and consequent actions, turned out to be unnecessary.
Subgroup analysis and investigation of heterogeneity
We decided, in advance, not to perform subgroup analyses based
on sex or age because we anticipated that the differences in mus-
cle disease severity would have a much bigger influence on out-
come than sex or age. Moreover, the American College of Sports
Medicine stated in their Position Stand (Pollock 1998) that rel-
ative improvements resulting from aerobic and strength training
are similar for young and old, male and female.
R E S U L T S
Description of studies
See: Characteristics of included studies;Characteristics of excluded
studies.
In this update, following further screening, we identified 24 com-
pleted trials (19 in the original review) that studied strength train-
ing as an intervention, 18 trials studying aerobic exercise train-
ing (nine in the original review), and 11 trials studying combined
strength training and aerobic exercise (eight in the original re-
view), sometimes incorporated in more comprehensive rehabili-
tation programmes. Most strength training trials included people
with the following muscle diseases: slowly progressive dystrophies
(mostly myotonic dystrophy, limb-girdle dystrophies, facioscapu-
lohumeral muscular dystrophy), and in the older studies non-
specified progressive muscular dystrophies and inflammatory my-
opathies. Studies on the effects of aerobice xercise training included
mainly people with slowly progressive dystrophies and metabolic
myopathies (mostly unspecified mitochondrial myopathies).
Studies have generally been limited by small sample sizes. We
excluded 44 trials because there was no randomised controlled
comparison between training and non-training patients (see
Characteristics of excluded studies). Six studies randomly assigned
one limb to be exercised, with the contralateral non-exercised side
serving as the control limb (Aitkens 1993;De Lateur 1979;Kilmer
1994;McCartney 1988;Milner-Brown 1988b;Tollbäck 1999).
In all these six studies, strength gains in th e exercised limb were the
same or only slightly greater than in the non-exercised limb. In the
resistance exercise trial of Aitkens, for example, strength gains did
not significantly differ between the exercised and non-exercised
limbs in either group (Aitkens 1993). This concept is called cross-
education, and has been described with different forms of exer-
cises. A meta-analysis of 16 randomised studies concluded that,
on average, the magnitude of cross-education is 8% of the initial
strength of the untrained limb (Munn 2004). Neural adaptations
to training and learning effects due to testing are postulated as
explanations (Lee 2007;Munn 2005;Sale 1988;Shima 2002).
Moreover, the results may well be confounded by the presence of
asymmetric weakness of both limbs, as the absolute gain in muscle
strength resulting from strength training is related to pre-exercise
muscle weakness (Kilmer 2002). Therefore, a non-exercised limb
is not an appropriate control, even if training was randomly as-
signed. For this reason, we have excluded studies using a within-
subjects design. We will revise the protocol to a priori exclude these
studies in the next update.
The majority of the studies did not have a non-training control
group of patients, or used a healthy control group. Only five studies
(three in the original review) were randomised controlled trials
making a comparison between training and non-training patients.
Regrettably, the extension of the initially randomised controlled
six-week aerobic exercise study in people with polymyositis and
dermatomyositis by Wiesinger et al lost its randomised controlled
design due to a decision of the ethics committee. We had to exclude
the first part of this study (Wiesinger 1998a) as it did not meet
our predefined criterion that the training programme should be at
least 10 weeks long, and the second part (Wiesinger 1998b) as it
was no longer randomised controlled. randomised controlled trial
5Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
on the effect of combined strength training and aerobic exercise
in a group of participants with different muscle diseases in this
update due to the training duration of only eight weeks (Dawes
2006).
In conclusion, two strength training trials and one strength train-
ing combined with aerobic exercise trial, newly identified in this
update, met all the inclusion criteria. The first strength training
trial compared the effect of 24 weeks of training versus no train-
ing in 36 adults with myotonic dystrophy and 30 adults with
hereditary motor and sensory neuropathy (Lindeman 1995). As
this review is concerned with muscle disease, we will not discuss
the results of the hereditary motor and sensory neuropathy pa-
tient group. The second strength training trial compared 52 weeks
of strength training versus no training, both combined with al-
buterol or placebo as add-on after the first 26 weeks of training
in 65 adult participants with facioscapulohumeral muscular dys-
trophy (van der Kooi 2004). The trial which combined aerobic
exercise and strength training compared 12 weeks of cycle exer-
cises and dynamic and isokinetic strength training in 18 people
with mitochondrial myopathy (Cejudo 2005) (see Characteristics
of included studies) .
Risk of bias in included studies
In the myotonic dystrophy trial (Lindeman 1995) participants
with myotonic dystrophy were individually matched for muscle
strength and performance in a stair-climbing test. Within each
matched pair, participants were randomly assigned to the train-
ing or control group. There was no published information on the
method of randomisation or on allocation concealment but the
first author (Lindeman) informed us that two independent per-
sons drew one sealed name per matched pair and allocated it by
tossing a coin to the training or non-training group. We graded the
intention to blind the clinical evaluators as adequate although ap-
proximately 20% of the myotonic dystrophy participants revealed
information to the clinical evaluators that resulted in unblinding
during the course of the trial. Baseline data for both experimental
groups were presented. The authors considered the comparability
as suboptimal because the training group contained more women,
was somewhat older, had longer time scores for stair climbing (a
measure of functional ability) and had higher knee torques (a mea-
sure of muscle strength). They argued that the first three items
could have resulted in an underestimation of the training effect,
whereas the last item could have resulted in an overestimation of
the training effect. They concluded that the differences in exper-
imental group composition did not seem to explain the absence
of differences in outcomes between treatment groups. We con-
sidered the way the authors presented and discussed the baseline
differences as adequate. Three of the initially 36 randomised par-
ticipants withdrew before disclosure of treatment allocation. The
33 participants starting the trial made 15 matched pairs. During
the trial one person dropped out. Because of the matched pair
design only complete pairs were analysed, thus eventually 28 of
the initial 36 randomised participants were analysed. Follow up
was thus incomplete and analysis was not by ’intention-to-treat’.
However, the flow path of participants was well documented.
In the facioscapulohumeral muscular dystrophy trial (van der Kooi
2004) 65 participants were stratified into two groups based on
muscle strength. Participants in both strata were randomly as-
signed to one of the four treatment groups according to a com-
puter generated randomisation list. The treatments consisted of
training plus albuterol, training plus placebo, non-training plus
albuterol, or non-training plus placebo. Training or non-training
was the first intervention, starting just after the baseline visit until
after the final visit at 52 weeks. After 26 weeks participants started
using the blinded trial medication. Information on the assignment
to training or non-training was disclosed to the participants by
the physical therapist (supervising the training programme) after
their baseline visit. Participants received the blinded trial medica-
tion from the pharmacy department. The clinical evaluator was
blinded for the assignment to both interventions. The participants,
physical therapist and the neurologist evaluating the side effects
were blinded to the study medication. The blinding of the clinical
evaluator was considered adequate, although one of the main sec-
ondary outcome measures, the one repetition maximum (1RM)
measurement for assessing dynamic strength, was performed by
the physical therapist who supervised the training, and who was
therefore not blinded to the allocation to training or non-training.
Allocation to the training or non-training group was unmasked in
three cases, due to unintentional remarks. The success of blind-
ing for the study medication was not formally checked. Baseline
characteristics were presented for all treatment groups. One par-
ticipant stopped training and four participants stopped using their
study medication, but they still attended all trial visits, resulting in
complete follow up of all participants. Data analysis was by inten-
tion-to-treat principle. As no statistically significant interactions
between the two interventions (i.e. training versus non-training;
albuterol versus placebo) could be detected, the effect sizes, being
the differences in mean change from baseline, were presented for
each intervention.
In the mitochondrial myopathy trial (Cejudo 2005), 20 partic-
ipants were randomly assigned to the training or control group.
There was no published information on the method of randomi-
sation, allocation concealment, or blinding of the evaluators. The
author (Cejudo) informed us that participants were randomly as-
signed according to a computer generated randomisation list. The
evaluators were not blinded to the intervention allocation but
knew to which group each participant was assigned. One partici-
pant in each group failed to finish the study for personal reasons.
Baseline assessment data were available for these participants, but
not published. Follow up was therefore incomplete and analysis
was not done by ’intention-to-treat’. No flow path of participants
was documented. Baseline characteristics were presented for both
groups, except for the participants lost to follow up. The authors
6Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
considered both groups as comparable with respect to anthropo-
metric features, as well as to each measured variable at baseline.
Each criterion was ranked using the Cochrane approach. The re-
view authors’ judgements about each methodological quality item
for included studies are presented in Figure 1.
Figure 1. Methodological quality summary: review authors’ judgements about each methodological quality
item for each included study.
Quality diagnostic criteria
This quality assessment took into account if and how diagnoses
were verified. In the myotonic dystrophy trial participants were
recruited via neurologists, physiatrists and the Dutch association
for neuromuscular diseases (Vereniging Spierziekten Nederland)
on clinical grounds and without genetic verification. We therefore
graded the quality of the diagnostic criteria as inadequate. In the
facioscapulohumeral muscular dystrophy trial either participants,
or a first-degree relative, had the associated deletion at chromo-
some 4 (Deidda 1996). The quality of the diagnosis was therefore
graded as adequate. In the mitochondrial myopathy trial partic-
ipants were recruited from a larger group of patients followed at
the university hospital of Sevilla, Spain. Diagnosis was based on
clinical and muscle biopsy data. Biopsy findings were determined
by biochemical and histological techniques without genetic veri-
fication. One participant in each group only had a probable di-
agnosis of mitochondrial myopathy. We graded the quality of the
diagnostic criteria as uncertain.
7Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Quality training programme
The training programmes of the myotonic dystrophy, facioscapu-
lohumeral muscular dystrophy and mitochondrial myopathy tri-
als fulfilled most of the minimal requirements as defined in the
Methods section. The training scheme for all trials was inadequate
only with respect to the number of muscle groups trained, as the
ACSM recommends eight to 10 exercises of all the major muscle
groups. Only four muscle groups were trained in the myotonic
dystrophy trial, two in the facioscapulohumeral muscular dystro-
phy trial and three in the mitochondrial myopathy trial. All stud-
ies focused on a limited number of muscle groups for reasons of
effect evaluation, safety and time restraints per training session. A
description of the training programmes and their scores are listed
in Table 1.
Table 1. Quality of diagnostic criteria and training programme
Study Diagnosis Type of ex-
ercise
Intensity Frequency Duration of
session
Duration of
programme
Muscle
groups
Supervision
Lindeman
1995
36 adults
with my-
otonic dys-
trophy;
34 classical
adult type, 2
congen-
ital form, di-
agnosis not
verified
Dynamic
with weights
Individ-
ualised pro-
gressive
overload, 3
sets from 25
repetitions
at 60% of
1RM, via 15
repetitions
at 70%,
to 10 repeti-
tions at 80%
3 times/
week
Within 30
minutes
24 weeks Knee exten-
sors
and flexors,
hip exten-
sors and ab-
ductors
Super-
vised home
training pro-
gramme
Scores C A A A A A C A
van der Kooi
2004
65
genetically
confirmed
adults with
facioscapu-
lohumeral
muscular
dystrophy
Dynamic
and isomet-
ric with
weights
Individ-
ualised pro-
gressive
overload, 2
sets dynamic
from 10 rep-
etitions at
10RM, via
8 repetitions
at 8RM, to
5 repetitions
at 5RM, and
30s iso-
metric with
same weight
3 times/
week
Within 30
minutes
52 weeks El-
bow flexors,
ankle dorsi-
flexors
Super-
vised home
training pro-
gramme
Scores A A A A A A C A
8Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Table 1. Quality of diagnostic criteria and training programme (Continued)
Cejudo
2005
20 partic-
ipants diag-
nosed with
mitochon-
drial myopa-
thy on the
basis of clin-
ical, familial
and muscle
biopsy data
Endurance
bicycle
training, dy-
namic
isotonic
with weights
Aerobic
train-
ing: individ-
ualised work
rate, 30-
minute
leg exercise
on an ergo
cycle, 70%
of the peak
work rate
Strength
train-
ing: one set
dynamic
and isotonic
of 10
to 15 repeti-
tions at 50%
1RM load,
to 2 or 3 sets
Adjust-
ments
on workload
changed ev-
ery 2 weeks
3 times/
week
Approxi-
mately 60
minutes
12 weeks Shoulder,
upper back,
arm, pec-
toralis ma-
jor, biceps
brachii and
brachialis
muscles
Supervised
training pro-
gramme by
specialised
nurses and a
physiatrist
specialist in
a Rehabili-
tation Unit
on an outpa-
tient basis
Scores C A A (strength
training)
A (aer-
obic exercise
training)
A A A C A
Effects of interventions
We intended to combine trial results for appropriate pairings of
treatments by calculating a weighted mean of the difference be-
tween their effects using the Cochrane statistical package RevMan.
Because we could not obtain the original data for the mitochon-
drial myopathy trial, we will describe the results of this trial as
published in the article.
Primary outcome measure for strength training:
muscle strength - expressed in measures of static (i.e.
isometric) or dynamic strength
Muscle strength was the primary outcome measure for the my-
otonic dystrophy and facioscapulohumeral muscular dystrophy
trials. In the myotonic dystrophy trial (Lindeman 1995) differ-
ences in muscle strength were measured isokinetically as maxi-
mum concentric knee torques at three velocities, and isometrically
as maximum voluntary contraction. Mean differences between
groups were 3.90 Nm (95% CI -4.11 to 11.91) for isokinetic knee
torque extension (Figure 2), 3.70 Nm (95% CI -3.78 to 11.18)
for isokinetic knee torque flexion (Figure 3) and 2.10 Nm (95%
CI -7.52 to 11.72) for maximum isometric voluntary contraction
(Figure 4), all in favour of the training group, although insignifi-
cant.
9Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Figure 2. Forest plot of comparison: 1 Strength training versus control in Myotonic Dystrophy, outcome: 1.1
Muscle strength - maximum isotonic knee torque extension.
Figure 3. Forest plot of comparison: 1 Strength training versus control in Myotonic Dystrophy, outcome: 1.2
Muscle strength - maximum isotonic knee torque flexion.
Figure 4. Forest plot of comparison: 1 Strength training versus control in Myotonic Dystrophy, outcome: 1.3
Muscle strength - maximum isometric voluntary contraction.
The primary outcome measure in the facioscapulohumeral mus-
cular dystrophy trial (van der Kooi 2004) was a change in maxi-
mum voluntary isometric strength of the elbow flexors and ankle
dorsiflexors. After 52 weeks the isometric strength of the elbow
flexors did not differ significantly between the training and non-
training group (mean difference right side 0.54 kgF, 95% CI -0.38
to 1.46, with the better score being for the training group, Figure
5). Dynamic strength was evaluated using the one repetition max-
imum (1RM), the weight a personcan lift once, but not twice, at a
steady controlled pace through the full range of joint motion. The
1RM showed a significantly larger increase in the training group
compared to the non-training group (mean difference right side
1.20 kg, 95% CI 0.18 to 2.16, Figure 6). Both strength measures
of the ankle dorsiflexors decreased significantly and markedly in
all treatment groups. This decrease was not influenced by training
(on the right side mean difference in maximum voluntary isomet-
ric contraction (MVIC) 0.43 kgF, 95% CI -1.62 to 2.48, more for
the training group (Figure 7), in 1RM -0.44 kg, 95% CI -1.77 to
0.89, less for the training group (Figure 8). Changes in strength
measures for the left-sided trained muscle groups did not differ
significantly from the right-sided results.
10Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Figure 5. Forest plot of comparison: 2 Strength training versus control in FSHD, outcome: 2.1 Muscle
strength elbow flexors - maximum voluntary isometric contraction.
Figure 6. Forest plot of comparison: 2 Strength training versus control in FSHD, outcome: 2.2 Muscle
strength elbow flexors - dynamic strength.
Figure 7. Forest plot of comparison: 2 Strength training versus control in FSHD, outcome: 2.3 Muscle
strength ankle dorsiflexors - maximum isometric voluntary contraction.
Figure 8. Forest plot of comparison: 2 Strength training versus control in FSHD, outcome: 2.4 Muscle
strength ankle dorsiflexors - dynamic strength.
11Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Muscle strength was a secondary outcome for the mitochondrial
myopathy trial (Cejudo 2005). Weight-lifting capacity was mea-
sured as the heaviest weight that could be lifted throughout the
complete range of movement (1RM test). After the study pe-
riod, all participants showed increases in all 1RM tests. After 12
weeks weight-lifting capacity did not differ significantly between
the training and non-training group. Mean differences in 1RM
between groups were -5.00 kg (95% CI -14.71 to 4.71) for the
shoulder press exercise (Figure 9), 6.40 kg (95% CI -2.89 to 15.69)
for the butterfly exercise (Figure 10) and 7.30 kg (95% CI -2.91
to 17.51) for the biceps curls exercise (Figure 11).
Figure 9. Forest plot of comparison: 5 Aerobic exercise and strength training in mitochondrial myopathy,
outcome: 5.1 Muscle strength shoulder press - maximum dynamic isotonic voluntary contraction.
Figure 10. Forest plot of comparison: 5 Aerobic exercise and strength training in mitochondrial myopathy,
outcome: 5.2 Muscle strength butterfly - maximum dynamic isotonic voluntary contraction.
Figure 11. Forest plot of comparison: 5 Aerobic exercise and strength training in mitochondrial myopathy,
outcome: 5.3 Muscle strength biceps curls - maximum isotonic dynamic voluntary contraction .
12Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Primary outcome measure for aerobic exercise
training: aerobic capacity, expressed in measures of
work capacity
In the mitochondrial myopathy trial (Cejudo 2005), work capac-
ity was measured in a cycle test and in the shuttle walking test. En-
durance time was measured in a submaximal cycling test at a con-
stant workload of 70% of the maximum power output achieved
during the baseline incremental cycle test. The mean difference in
time and distance cycled till exhaustion and leg fatigue or breath-
lessness exhaustion differed significantly between groups after 12
weeks. The mean differences in time and distance cycled till ex-
haustion between groups were 23.70 minutes (95% CI 2.63 to
44.77) (Figure 12) and 9.70 km (95% CI 1.51 to 17.89) (Figure
13), respectively. The distance walked until exhaustion was mea-
sured in the shuttle walking test. The mean difference between
groups was 78.00 metres (95% CI -144.86 to 300.86) (Figure 14).
Figure 12. Forest plot of comparison: 5 Aerobic exercise and strength training in mitochondrial myopathy,
outcome: 5.4 Work capacity - mean time till exhaustion in cycle test.
Figure 13. Forest plot of comparison: 5 Aerobic exercise and strength training in mitochondrial myopathy,
outcome: 5.5 Work capacity- mean distance till exhaustion in cycle test.
Figure 14. Forest plot of comparison: 5 Aerobic exercise and strength training in mitochondrial myopathy,
outcome: 5.6 Work capacity - mean distance walked till exhaustion in shuttle walking test.
13Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Secondary outcome measures for aerobic exercise or
strength training, or both:
Aerobic capacity - expressed in measures of oxygen uptake
(i.e. VO2max)
In the mitochondrial myopathy trial, oxygen uptake (VO2max)
was noninvasively determined in a maximal incremental cycle ex-
ercise test (Cejudo 2005). VO2max differed significantly between
groups; mean difference was 14.60 ml/min/kg (95% CI 8.70 to
20.80).
Muscle strength - expressed in measures of endurance or
fatigue
This outcome was published for the myotonic dystrophy and fa-
cioscapulohumeral muscular dystrophy studies. In the myotonic
dystrophy trial endurance was measured as maximum duration of
contraction at 80% of mean voluntary isometric contraction. The
difference between groups, 13.1 seconds (95% CI 2.2 to 24.0)
longer for the training group, was significant. This difference was
mainly due to a decrease in endurance in the non-training group.
In the facioscapulohumeral muscular dystrophy trial muscle en-
durance was expressed as a Force-Time Integral (FTI30) of a sus-
tained 30 seconds maximal isometric contraction. The FTI30 of
the elbow flexors did not differ significantly between the train-
ing and non-training group (mean difference right side 2 kgF.s,
95% CI -18 to 22, in favour of the training group). The FTI30
of the ankle dorsiflexors decreased significantly and markedly in
all treatment groups. This decrease was not influenced by training
(mean difference right side -1 kgF.s, 95% CI -42 to 41). Changes
in FTI30 for the left-sided trained muscle groups did not differ
significantly from the right-sided results.
(Time-scored) functional assessments of muscle
performance
This outcome was available for the myotonic dystrophy and fa-
cioscapulohumeral muscular dystrophy trials. In the myotonic
dystrophy trial functional assessments comprised the following
time-scored activities: ascending and descending stairs, rising from
a chair, rising from supine, walking 50 metres as fast as possible,
and walking 6 metres at natural speed. In the facioscapulohumeral
muscular dystrophy trial the functional tests consisted of the as-
sessment of a functional upper extremity grade and functional
lower extremity grade (Personius 1994), and the following timed-
scored tasks: standing from lying supine, standing from sitting,
walking 30 feet (9.14 metres), and climbing three standard stairs
(Personius 1994). None of the outcomes demonstrated relevant or
significant changes in mean differences between treatment groups
in either trial.
Quality of life
This outcome was assessed in the facioscapulohumeral muscular
dystrophy trial using the Sickness Impact Profile (SIP) and the
Symptom-Checklist (SCL-90-R). The mean total of the SIP and
its subscales did not demonstrate relevant or significant changes
for either the training or non-training groups. In addition, for
both groups the mean SCL total did not change between the
baseline and final visit. In the mitochondrial myopathy trial, the
Nottingham Health Profile (NHP) questionnaire was used. Scores
ranged from 0 (no problem) to 100 (maximum problem). The
mean difference in overall score between both groups was -9.80
(95% CI -25.70 to 6.14).
Parameters of muscle membrane permeability (serum
creatine kinase level, serum myoglobin level)
This outcome was available for the myotonic dystrophy and mito-
chondrial myopathy trial. In the myotonic dystrophy trial, serum
myoglobin levels were assessed just before and one hour after the
measurement session at the baseline visit and at the final visit.
Changes in serum myoglobin activity one hour after a standard-
ised test should reflect changes in muscle fibre permeability due
to muscle damage. The mean rise in serum myoglobin levels did
not differ significantly between the training and the non-training
group (mean difference -21.00 ng/l, 95% CI -48.35 to 6.35). In
the mitochondrial myopathy trial, the authors state that serum
creatine kinase levels of participants remained unaltered after the
intervention period. However, data for the serum creatine kinase
level were not published. In the facioscapulohumeral muscular
dystrophy trial, one patient stopped training because of recurring,
training-related muscle soreness and fatigue. A diagnostic work-up
revealed a mitochondrial myopathy as wellas facioscapulohumeral
muscular dystrophy. In the mitochondrial myopathy trial, cancel-
lations by participants happened because of muscle soreness asso-
ciated with the exercise activity. However, every patient was able
to tolerate the exercise training regimen without complications.
In the myotonic dystrophy trial, a few participants complained of
muscle soreness and transient strength reduction after eight weeks.
However, no signs of muscle damage were found over the entire
period of 24 weeks.
Experienced pain
This outcome was available in both the facioscapulohumeral mus-
cular dystrophy and mitochondrial myopathy trials. In the fa-
cioscapulohumeral muscular dystrophy trial, 11 out of 34 partici-
pants in the training group reported pain in the neck and shoulder
region to the physical therapist during home visits. Five people
mentioned a period with elbow complaints. However, the num-
ber of people with neck-shoulder and elbow complaints did not
differ between treatment groups at baseline nor at the final visit.
Moreover, the number of participants with neck-shoulder and el-
14Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
bow complaints slightly decreased in both groups. Relative risk at
the final visit was 1.02 (95% CI 0.66 to 1.58) for neck-shoulder
and 1.82 (95% CI 0.17 to 19.13) for elbow complaints in favour
of the non-training group. Although not formally quantified, the
authors mentioned that participants experienced no notable mus-
cle soreness after training. At the final visit, scores on the visual
analogue scale for pain and the mean daily rated pain scores did
not demonstrate significant changes for either group.
In the mitochondrial myopathy trial, participants’ arm and leg
myalgia were recorded in a simple questionnaire and scored as
mild, moderate or severe. Two people in the exercise group and
three people in the control group reported severe myalgia in arms
and legs. Seven people in the exercise group and five people in
the control group reported moderate myalgia in arms and legs.
After the 12-week training programme no participants in the exer-
cise group and five participants in the control group still reported
symptoms of myalgia.
Experienced fatigue
In the facioscapulohumeral muscular dystrophy trial, experienced
fatigue was measured by the fatigue severity subscale Checklist In-
dividual Strength (CIS-fatigue). At the final visit, the mean score
on the CIS-fatigue did not change significantly between the base-
line and final visit for either group. The mean daily rated fatigue
score of the participants in the training group slightly decreased,
whereas the score in the non-training group showed a small in-
crease.
In the mitochondrial myopathy trial, participants’ usual fatiga-
bility was recorded in a simple questionnaire and scored as mild,
moderate or severe. Three participants in the exercise group and
five participants in the control group reported severe fatigue in
arms and legs. At the end of the study period, no participants
in the exercise group and five participants in the control group
reported severe fatigue in arms and legs. Six participants in the
exercise group and two participants in the control group reported
moderate fatigue. After the intervention period, five participants
in the exercise group and two participants in the control group
still reported moderate fatigue.
D I S C U S S I O N
Only five out of the 53 identified studies on the effect of training
in people with muscle disease used a randomised controlled de-
sign. Two of these five trials were excluded because of a training
duration of less than 10 weeks. The first excluded randomised con-
trolled trial evaluated the effect of six weeks of cycle exercises and
step aerobics in 14 people with polymyositis or dermatomyosi-
tis. After six weeks the peak isometric torque of the hip flexors
and knee extensors, and the peak oxygen consumption differed
significantly between the training and non-training group (mean
difference of peak isometric torque 18.30 Nm, 95% CI 8.20 to
28.30, and mean difference of VO2max 14.60 ml/min/kg, 95%
CI 8.72 to 20.48, respectively) (Wiesinger 1998a). The second
excluded randomised controlled trial evaluated the effect of home-
based walking and strength training for eight weeks in 18 people
with different muscle diseases. After eight weeks of training, only
the mean difference in muscle strength of the right quadriceps
reached statistical difference. The mean difference between groups
was 4.26 kg (95% CI 0.66 to 7.86). The mean difference of dis-
tance walked in the two minute walking test between groups was
-11.62 metres (95% CI -31.11 to 7.87), in favour of the control
group (Dawes 2006). There is no evidence in the literature for
excluding trials with a training duration of less than 10 weeks in
muscle disease. Because the two studies with a training duration
less than 10 weeks showed benefits, we will revise the protocol to
include trials with a training duration of at least six weeks in a fu-
ture update. Only three trials fulfilled the predefined criterion of a
minimum of 10 weeks duration of training. The strength training
trials in myotonic dystrophy and facioscapulohumeral muscular
dystrophy participants had minor methodological shortcomings.
The methodological quality for both strength training trials was
therefore judged as adequate. In the facioscapulohumeral muscu-
lar dystrophy trial one of the main secondary outcome measures,
the 1RM strength measurement, was performed by a physical ther-
apist not blinded to the allocation to training or non-training. In
the myotonic dystrophy trials diagnoses were not adequately ver-
ified. Furthermore, analysis in the myotonic dystrophy trial was
not by intention-to-treat partly due to the matched-pair design.
The methodological quality of the combined strength training
and aerobic exercise trial in mitochondrial myopathy participants
had several minor methodological shortcomings and was therefore
judged as uncertain. In the mitochondrial myopathy trial, clinical
evaluators were not blinded, which may have led to an overestima-
tion of the training effect on muscle strength and aerobic capacity.
Analysis in this trial was not by intention-to-treat.
Most mean differences in muscle strength outcomes (isometric,
dynamic and endurance) between groups in all trials showed small,
non-significant beneficial effects in favour of the training groups.
Only changes in the endurance measure in the myotonic dystro-
phy trial (13.10 seconds longer maximum duration of an isometric
contraction; 95% CI 2.20 to 24.00) and in the dynamic strength
measure for the elbow flexors in the facioscapulohumeral muscu-
lar dystrophy trial (concentric contraction with 1.20 kg heavier
weight; 95% CI 0.18 to 2.16) reached statistical significance. The
absent or limited positive effects of strength training on muscle
strength could reflect the inability of the diseased muscular system
to respond with normal neural and hypertrophic adaptations to
the applied training stimuli. However, part of this lack of response
could be due to the specificity of the training (Lindeman 1995).
All adaptations to training are specific to the stimuli applied. Spe-
15Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
cific strength training essentially involves exercising the muscles
in the same manner as the expected use (Kraemer 2002). This
means that a training programme with dynamic exercises increases
dynamic strength more than isometric strength, and vice versa.
This phenomenon of specificity of training has implications for
the sensitivity of the outcome measures; e.g. the positive effect
of a dynamic strength training programme may be captured by
using a dynamic evaluation technique, but might be missed using
an isometric strength measure. The size of the carry-over effect
from, for example, dynamic strength to isometric strength cannot
be predicted and it may be that there is a diminished ability of
the diseased muscular system to transfer effects of a specific train-
ing programme from one strength modality to another (van der
Kooi 2004). In the facioscapulohumeral muscular dystrophy trial,
training did not influence strength of the ankle dorsiflexors, in
contrast to the elbow flexors. The authors thought that a differ-
ence in grade of muscle weakness at baseline between elbow and
ankle dorsiflexors might provide the explanation for the difference
in their response to training. In this study elbow flexors were eligi-
ble for testing and training when strength according to the MRC
scale grade was three or more, whereas ankle dorsiflexors were el-
igible when the muscles moved the ankle joint in a position be-
tween dorsiflexion and plantarflexion, which potentially includes
MRC grades less than three (Medical Research Council 1981).
Therefore, pre-exercise weakness might have been more severe in
ankle dorsiflexors compared to elbow flexors. In patients with a
muscle disease it is assumed that absolute gain in muscle strength
resulting from strength training is probably related to pre-exercise
muscle strength, and that severely weak muscles (< 10% of nor-
mal strength) may not be able to improve. However, this widely
reported assumption is based on one published observation only
(Milner-Brown 1988a). In the mitochondrial myopathy trial, the
mean difference in aerobic capacity as measured in a submaxi-
mal cycle test differed significantly between the training and non-
training group after the study period. Participants in the train-
ing group cycled on average 23.70 minutes and 9.70 kilometres
longer (95% CI 2.63 to 44.77 and 1.51 to 17.89, respectively)
than participants in the control group. The distance walked in the
shuttle walking test did not differ between groups. This could be
explained by the specificity of training, because training consisted
of cycling rather than walking exercises.
The timed-scored functional assessments did not demonstrate
any relevant or significant changes in mean differences between
treatment groups in either the myotonic dystrophy, or in the fa-
cioscapulohumeral muscular dystrophy trial. This may be due to
the small number of muscle groups trained, the absent or limited
effects on muscle strength, and the specificity of the training stim-
uli applied.
In all trials no signs of overuse, such as a decline in strength mea-
sures (Lindeman 1995;van der Kooi 2004), a rise in parameters
of muscle membrane permeability (Lindeman 1995), or training-
related increase in pain or fatigue (van der Kooi 2004) were seen.
This is of major clinical importance because these findings do
not support the hypothesis of increased risk of muscle strain in
these two slowly progressive muscular dystrophies. However, ad-
verse events were only mentioned in general and not compared
between groups. Moreover, several patients in all trials experienced
muscle soreness. An enhanced liability for overwork weakness in
more severely affected facioscapulohumeral muscular dystrophy
patients cannot be excluded, because patients unable to walk in-
dependently were not included in the facioscapulohumeral mus-
cular dystrophy trial. Furthermore, all strength training studies,
including these three, imposed a controlled strain for a relatively
short period. Hence, exertion of longer duration may still have an
undetermined effect on disease progression.
Based on the evidence of the three selected randomised trials in
this review concerning myotonic dystrophy (Lindeman 1995), fa-
cioscapulohumeral muscular dystrophy (van der Kooi 2004) and
mitochondrial myopathy (Cejudo 2005), patients with these spe-
cific disorders can be advised that ’normal’ participation in sports
and work appears not to harm their muscles, but there is still
insufficient evidence that it offers benefit. There is insufficient
evidence for general prescription of strength training and aero-
bic exercise programmes in myotonic dystrophy and facioscapulo-
humeral muscular dystrophy, and some evidence in mitochondrial
myopathy.Unfortunately, no clearly defined exercise protocols can
be drawn from the current research evidence.
The results of the non-selected studies concerning other muscle
diseases suggest a positive effect of strength training and do not
point towards enhanced susceptibility concerning muscle over-
strain, but limitations in the design of these studies prevent valid
conclusions. The number of recent studies lacking a randomised
controlled design is striking. At least for the relatively frequent
muscle diseases one should aim for randomised controlled training
studies. A non-exercised limb should not serve as a control, because
of possible cross-education effects and a possible baseline differ-
ence in muscle weakness. More importantly,one can hardly expect
meaningful effects of a single-limb training programme on daily
activities, social participation and well-being of patients. While
participants with different neuromuscular disorders can partici-
pate in the same study, the data should be presented and anal-
ysed individually for each specific muscle disease, as differences in
the type of muscle disease may cause different responses to train-
ing. Specific diagnostic criteria should be given for all muscle dis-
eases included. The severity of the impairments (loss of functions)
should be presented to allow readers to assess the generalisabil-
ity of the results to other patients. Although it may be difficult
to quantify disease severity in some patients, ideally measures of
disease severity should be presented as well, because differences
among patients may strongly influence the outcome of training. In
trials with a small sample size, participants should be stratified for
disease severity. Another related characteristic that may influence
16Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
outcome is the level of activity (sedentary versus active) at base-
line, because in the healthy population untrained persons respond
with higher percentages and rates of gain in strength, compared
to trained individuals (Kraemer 2002).
The active training arm in trials could have additional non-specific
benefits for the participants due to the regular interaction with
a skilled therapist in contrast to the non-treatment group. This
interaction may influence several outcome parameters, for example
quality of life. Therefore, future studies should preferably have
an appropriate control intervention rather than “no training” in
order to assess the specific benefits of aerobic exercise and strength
training exercise.
In strength training and aerobic exercise intervention studies, the
training programme should be described in detail, just like the
well-known prescription of drugs. Authors should provide infor-
mation about the type(s) of exercises, the intensity (including pro-
gression rate), frequency, duration per exercise session, the dura-
tion of the entire programme, as well as trained muscle groups,
and (about) the supervision of training. The recommendations
from the ACSM Position Stand on ’The Recommended Quan-
tity and Quality of Exercise for Developing and Maintaining Car-
diorespiratory and Muscular Fitness, and Flexibility in Healthy
Adults’ (Pollock 1998) can be used as requirements to achieve an
effective, safe and individualised exercise prescription taking into
account the pre-training level of fitness. The ACSM recommen-
dations were almost all adhered to by most of the included and
excluded studies in this review. The only criterion that was rarely
met was that eight to ten major muscle groups should be exercised
in strength training programmes. This is probably partly due to
limitations in time available to evaluate the effects of training by
multiple assessments covering the different outcome measures. In
addition, expenses for (adjusted) training equipment can be high.
Thirdly, investigators were perhaps too cautious in order not to
strain participants too much.
More studies on the effects of aerobic exercise and strength train-
ing programmes in specific muscle diseases on the basic level of
muscle function and aerobic capacity are needed. There are well-
validated outcome measures that are able to assess positive and -
at least equally important - negative effects on the diseased mus-
cular system. The expertise to deliver training programmes is al-
ready present in sports medicine and experts in exercise physiol-
ogy should be consulted. If strength training and aerobic exercise
training programmes prove effective, we can then aim to develop
and evaluate programmes adjusted to each different muscle dis-
ease. In patients with muscular disorders, combinations of mus-
cle weakness, fatigue, pain and difficulty exercising can all lead
to reduced physical activity and a sedentary lifestyle (McDonald
2002). Physical inactivity negatively impacts quality of life and
health outcomes (McDonald 2002). In healthy young adults, the
elderly, and in cardiac patients, increasing physical activity and
participation in comprehensive exercise programmes incorporat-
ing aerobic activities, strength training and flexibility exercises has
been shown to reduce the risk of several chronic diseases (e. g. coro-
nary heart disease, obesity, diabetes and osteoporosis) (Kraemer
2002). Therefore, indicators of chronic disease risk such as blood
pressure, resting heart rate, body mass, glucose tolerance and bone
density could be useful as additional outcome measures (Kilmer
2002), although little is known about the risks of comorbidity in
patients with a muscle disease. Cost-benefit analyses are only rel-
evant if the benefit of training is much higher than studies have
shown so far.
In summary, the authors’ recommendations for future studies are
as follows.
•Participants with different muscle disorders can participate
in one study, but data should be presented and analysed for each
disease individually, and the power should be sufficient for each
individual disorder.
•Randomised controlled comparisons should be made with
participants having the same muscle disease. The effect of
training in patients with a muscle disease should be compared to
a non-exercising control group of patients with the same muscle
disease and not to healthy individuals, or to contralateral non-
exercised limbs.
•Stratification is strongly advised with regard to disease
severity, particularly in studies with a small sample size. It should
also be considered for pre-training level of activity (sedentary
versus active, particularly in aerobic intervention studies).
•The following aspects of the training intervention should be
specified: type(s) of exercise training, intensity and progression
rate, frequency, duration per exercise session and of the entire
programme, trained muscle groups, and supervision of training.
Duration of the training intervention should be at least six weeks.
•Outcomes should at least include measures of muscle
function (e.g. strength, endurance) and aerobic capacity (e.g.
work capacity), and functional assessments. Researchers should
be aware of the specificity of training effects in their choice of
outcome measures. The following evaluations are strongly
advised: measures of quality of life, experienced pain and
experienced fatigue.
•Outcomes assessors should be blinded to avoid
measurement bias.
•An appropriate placebo intervention is recommended in
order to measure exercise-specific benefits.
A U T H O R S ’ C O N C L U S I O N S
Implications for practice
Based on the evidence of three randomised trials in this re-
view, moderate-intensity strength training in myotonic dystrophy
17Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
(Lindeman 1995) and facioscapulohumeral muscular dystrophy
(van der Kooi 2004) shows no significant benefit or harm. A com-
bination of aerobic exercise and strength training in mitochon-
drial myopathy shows no harm and could be beneficial for aerobic
capacity (Cejudo 2005). The small number of included studies
and limitations in the design of studies in other muscle diseases
prevent general conclusions in these disorders.
Implications for research
There is a need for more research to establish whether strength
training and aerobic exercise training is beneficial in all forms of
muscle disease, and to define the optimal exercise programmes for
patients with a muscle disease.
A C K N O W L E D G E M E N T S
The Netherlands Organisation for Scientific Research (NWO),
the Health Research and Development Councilof the Netherlands
(ZON) and the Prinses Beatrix Fonds (the Dutch Public Fund
for Neuromuscular Disorders) for supporting three of the authors
(Voet, van der Kooi, Lindeman) in related neuromuscular research
projects.
R E F E R E N C E S
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Facioscapulohumeral dystrophy natural history study:
standardization of testing procedures and reliability of
measurements. The FSH DY Group. Physical Therapy 1994;74(3):
253–63.
Pollock 1998
Pollock M, Gaesser G, Butcher J, Dishman J-P, Franklin B, Garber
C. Position stand on the recommended quantity and quality of
exercise for developing and maintaining cardiorespiratory and
muscular fitness, and flexibility in healthy adults. Medicine and
Science in Sports and Exercise 1998;30(6):975–91. [MEDLINE:
98287757 98287757]
RevMan 2008
The Nordic Cochrane Centre. The Cochrane Collaboration.
Review Manager (RevMan). 5.0. Copenhagen: The Nordic
Cochrane Centre. The Cochrane Collaboration, 2008.
Sale 1988
Sale DG. Neural adaptation to resistance training. Medicine and
Science in Sports and Exercise 1988;20(Suppl 5):135–45.
Saunders 2004
Saunders DH, Greig CA, Young A, Mead GE. Physical fitness
training for stroke patients. Cochrane Database of Systematic Reviews
2004, Issue 1. [Art. No.: CD003316. DOI: 10.1002/
14651858.CD003316.pub2]
Shima 2002
Shima N, Ishida K, Katayama K, Morotome Y, Sato Y, Miyamura
M. Cross education of muscular strength during unilateral
resistance training and detraining. European Journal of Applied
Physiology 2002;86(4):287–94.
USDHHS 1996
U.S. Department of Health and Human Services, Centers for
Disease Control and Prevention, National Center for Chronic
Disease Prevention and Health Promotion. Physical activity and
health: a report of the surgeon general. Atlanta: Centers for Disease
Control and Prevention, 1996.
Vignos 1983
Vignos PJ Jr. Physical models of rehabilitation in neuromuscular
disease. Muscle & Nerve 1983;6(5):323–38. [MEDLINE:
83297471]
∗Indicates the major publication for the study
21Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
C H A R A C T E R I S T I C S O F S T U D I E S
Characteristics of included studies [ordered by study ID]
Cejudo 2005
Methods Parallel group randomised clinical trial
Participants 20 adults with mitochondrial myopathy
Interventions Strength training and aerobic exercise training versus no training
Outcomes Primary: exercise capacity - expressed in measures of oxygen uptake (i.e. VO2max),
endurance time and distance walked in the shuttle walking test. Secondary outcomes
were: peripheral muscle strength (1RM test), quality of life, symptoms of myalgia,cr amps
and fatigability and functional exercise capacity.
Notes -
Risk of bias
Item Authors’ judgement Description
Adequate sequence generation? Yes Quote: “Patients were randomly assigned to a
training group or control group”.
Comment: no published information on the
sequence generation. The author (Cejudo) in-
formed us that patients were randomly as-
signed according to a computer generated ran-
domisation list.
Allocation concealment? Yes Quote: “Patients were randomly assigned to a
training group or control group”.
Comment: no published information on the
allocation concealment. The author (Cejudo)
informed us that patients were randomly as-
signed according to a computer generated ran-
domisation list.
Blinding?
All outcomes
No Comment: no published information on the
blinding of the outcome assessors and person-
nel. The author (Cejudo) told us that the eval-
uators knew to which group each patient was
assigned.
Incomplete outcome data addressed?
All outcomes
No Quote: “...one patient in each group failed to
finish the study for personal reasons”.
Comment: baseline outcome data assessed,
but not available for these patients. So 1/10
missing from intervention group and 1/10
22Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Cejudo 2005 (Continued)
missing from control group.
Free of selective reporting? No No primary and secondary outcome(s) de-
fined in the article
Free of other bias? Yes No risk of bias from other sources detected
Lindeman 1995
Methods Evaluator blind, matched-control, randomised controlled trial
Participants 36 adults with myotonic dystrophy (2 congenital form, 34 classical type)
Interventions Strength training versus no training
Outcomes Primary: muscle strength by isokinetically measured knee torques and isometrically as
maximum voluntary contraction (MVIC). Main secondary outcomes were: endurance
by maximum duration of contraction at 80% of MVIC, functional performance by
timed motor performance tests and by questionnaires. Serum myoglobin levels to detect
changes in muscle fibre membrane permeability.
Notes Participants were matched basedon muscle strength (knee extension torque/body weight)
and on performance in a stair-climbing test. Only complete pairs were analysed.
Risk of bias
Item Authors’ judgement Description
Adequate sequence generation? Yes Comment: there was no published infor-
mation on the sequence generation but the
author (Lindeman) informed us that 2 in-
dependent persons drew a sealed lot per
matched pair and allocated it by tossing a
coin to the training or non-training group.
Allocation concealment? Yes Comment: there was no published infor-
mation on the method of allocation con-
cealment but the author (Lindeman) in-
formed us that 2 independent persons al-
located the training, after tossing the coin,
to the training or non-training group.
Blinding?
All outcomes
Yes Quote: “observers of the outcome measure-
ments were blinded for treatment alloca-
tion”
Comment: approximately 20% of the my-
otonic dystrophy participants revealed in-
formation to the clinical evaluators that re-
23Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Lindeman 1995 (Continued)
sulted in unblinding during the course of
the trial
Incomplete outcome data addressed?
All outcomes
No 3 of the initially 36 randomised partici-
pants withdrew before disclosure of treat-
ment allocation. The 33 participants start-
ing the trial made 15 matched pairs. Dur-
ing the trial 1 person dropped out. Because
of the matched pair design only complete
pairs were analysed, therefore eventually 28
of the initial 36 randomised participants
were analysed. Follow up was therefore in-
complete and analysis was not by ’inten-
tion-to-treat’. However, the flow path of
participants was well documented.
Free of selective reporting? Yes No evidence found for selective reporting
Free of other bias? Yes No risk of bias from other sources detected
van der Kooi 2004
Methods Evaluator blind, parallel group, randomised controlled trial
Participants 65 adults with facioscapulohumeral muscular dystrophy
Interventions Strength training versus no training (and as add-on in a double blind randomised con-
trolled design albuterol or placebo)
Outcomes Primary: difference in muscle strength of elbow flexors and ankle dorsiflexors after
52 weeks using the maximum voluntary isometric strength (MVIC). Main secondary
outcomes were muscle endurance (MVIC Force-Time Integral) and dynamic muscle
strength (1RM). Other measures included functional tests and timed motor per formance
tasks.
Notes Outcomes are presented for the 4 treatment groups (i.e. the 4 combinations of training
versus non-training, and albuterol versus placebo). Effect sizes are presented by inter-
vention as well.
Risk of bias
Item Authors’ judgement Description
Adequate sequence generation? Yes Quote: “...participants were randomly as-
signed to one of the four treatment groups
according to a computer generated ran-
domisation list”
24Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
van der Kooi 2004 (Continued)
Allocation concealment? Yes Quote: “information on the assignment to
training or non-training was disclosed to
the participants by the physical therapist”
Blinding?
All outcomes
Yes Quote: “The RM measurements were per-
formed by the physical therapist, who was
not blinded for the allocation to training or
non-training, as this specific measurement
carried too great a risk of unblinding the
clinical evaluator”
Comment: adequate although one of the
main secondary outcome measures, the one
repetition maximum (1RM) measurement
for assessing dynamic strength, was per-
formed by the physical therapist, who su-
pervised the training, and was therefore not
blinded to the allocation to training or non-
training. Unblinding during the trial was
adequately registered. Allocation to train-
ing or non-training was unmasked in 3
cases, due to unintentional remarks.
Incomplete outcome data addressed?
All outcomes
Yes Quote: “One patient stopped training be-
cause of recurring, training-related mus-
cle soreness and fatigue. Four participants
stopped using their study medication be-
cause of side effects. Data for the partic-
ipants who discontinued an intervention
were analysed in the assigned treatment
group”
Comment: complete follow up of all par-
ticipants
Free of selective reporting? Yes No evidence found for selective reporting
Free of other bias? Yes No risk of bias from other sources detected
Characteristics of excluded studies [ordered by study ID]
Abramson 1952 Not a randomised controlled trial
Aitkens 1993 Not a randomised controlled trial. Exercised versus non-exercised control limb (randomly assigned) and
patients versus healthy volunteers.
Alexanderson 1999 Pilot study. Not a randomised controlled trial.
25Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
(Continued)
Alexanderson 2000 Extension of a pilot study Alexanderson 1999. Not a randomised controlled trial.
Alexanderson 2007 Not a randomised controlled trial. Training programme duration of 7 weeks.
Arnardottir 2003 Not a randomised controlled trial
Dastmalchi 2007 Not a randomised controlled trial
Dawes 2006 Training duration of 8 weeks
De Lateur 1979 Not a randomised controlled trial. Exercised versus non-exercised control limb (randomly assigned).
Escalante 1993 Not a randomised controlled trial
Florence 1984a Not a randomised controlled trial
Florence 1984b Not a randomised controlled trial
Fowler 1965 Not a randomised controlled trial. Exercise combined with medication.
Heikkila 2001 Not a randomised controlled trial. Training programme duration of 3 weeks.
Hicks 1989 Not a randomised controlled trial. Training programme duration of 1 month.
Hoberman 1955 Not a randomised controlled trial. 3 drugs added to a comprehensiveregimen of therapies, including breathing
and resistive exercises.
Jeppesen 2006 Not a randomised controlled trial
Johnson 2007 Not a randomised controlled trial
Kelm 2001 Not a randomised controlled trial. Training programme duration of 6 weeks.
Kilmer 1994 Not a randomised controlled trial. Exercised versus non-exercised control limb (randomly assigned) and
patients versus healthy volunteers.
Kilmer 2005 Not a randomised controlled trial
Lenman 1959 Not a randomised controlled trial. Training programme duration for patients with muscle disorders ranged
from approximately 1 to 21 months.
Mate-Munoz 2007 Not a randomised controlled trial
McCartney 1988 Not a randomised controlled trial. Exercised versus non-exercised control limb (randomly assigned). Training
programme duration of 9 weeks.
Mielke 1990 Not a randomised controlled trial. Training programme duration of 6 weeks.
26Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
(Continued)
Milner-Brown 1988a Not a randomised controlled trial. Training programme duration for patients with muscle disorders ranged
from approximately 2 to 48 months.
Milner-Brown 1988b Not a randomised controlled trial. Intervention is not training versus non-training, but training added to
electric stimulation or electric stimulation only in 1 limb versus a non-stimulated, non-exercised control
limb.
Milner-Brown 1990 Not a randomised controlled trial. Intervention is not training versus no training, but amitriptyline added
to strength training.
Murphy 2008 Not a randomised controlled trial
Na 1996 Not a randomised controlled trial. Intervention is not training versus non-training, but training and daily
quinine sulfate.
Olsen 2005 Not a randomised controlled trial
Orngreen 2005 Not a randomised controlled trial
Scott 1981 A randomised controlled trial that makes a comparison between 2 different training regimes. No comparison
of training versus non-training patients.
Siciliano 2000 Not a randomised controlled trial
Spector 1997 Not a randomised controlled trial
Sunnerhagen 2004 Not a randomised controlled trial. Training programme duration 8 weeks.
Sveen 2007 Not a randomised controlled trial
Sveen 2008 Not a randomised controlled trial
Taivassalo 1998 Not a randomised controlled trial. Training programme duration of 8 weeks.
Taivassalo 1999 Not a randomised controlled trial. Training programme duration of 8 weeks.
Taivassalo 2001 Not a randomised controlled trial
Taivassalo 2006 Not a randomised controlled trial
Tollbäck 1999 Not a randomised controlled trial. Exercised versus non-exercised control limb (randomly assigned).
Trenell 2006 Not a randomised controlled trial
Varju 2003 Not a randomised controlled trial. Training programme duration of 3 weeks.
Vignos 1966 Not a randomised controlled trial
27Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
(Continued)
Wiesinger 1998a Training duration of 6 weeks
Wiesinger 1998b A non-randomised extension of a randomised controlled trial (Wiesinger 1998a).
Wright 1996 Not a randomised controlled trial
Yildirim 2007 Not a randomised controlled trial
28Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
D A T A A N D A N A L Y S E S
Comparison 1. Strength training versus control in myotonic dystrophy
Outcome or subgroup title No. of
studies
No. of
participants Statistical method Effect size
1 Muscle strength - maximum
isotonic knee torque extension
1 28 Mean Difference (IV, Fixed, 95% CI) 3.9 [-4.11, 11.91]
2 Muscle strength - maximum
isotonic knee torque flexion
1 28 Mean Difference (IV, Fixed, 95% CI) 3.70 [-3.78, 11.18]
3 Muscle strength - maximum
isometric voluntary contraction
1 28 Mean Difference (IV, Fixed, 95% CI) 2.10 [-7.52, 11.72]
Comparison 2. Strength training versus control in facioscapulohumeral muscular dystrophy
Outcome or subgroup title No. of
studies
No. of
participants Statistical method Effect size
1 Muscle strength elbow flexors -
maximum voluntary isometric
contraction
1 65 Mean Difference (IV, Fixed, 95% CI) 0.54 [-0.38, 1.46]
2 Muscle strength elbow flexors -
dynamic strength
1 65 Mean Difference (IV, Fixed, 95% CI) 1.17 [0.18, 2.16]
3 Muscle strength ankle
dorsiflexors - maximum
isometric voluntary contraction
1 65 Mean Difference (IV, Fixed, 95% CI) 0.43 [-1.62, 2.48]
4 Muscle strength ankle
dorsiflexors - dynamic strength
1 65 Mean Difference (IV, Fixed, 95% CI) -0.44 [-1.77, 0.89]
Comparison 3. Aerobic exercise and strength training in mitochondrial myopathy
Outcome or subgroup title No. of
studies
No. of
participants Statistical method Effect size
1 Muscle strength shoulder press
- maximum dynamic isotonic
voluntary contraction
1 18 Mean Difference (IV, Fixed, 95% CI) -3.00 [-14.71, 4.71]
2 Muscle strength butterfly -
maximum dynamic isotonic
voluntary contraction
1 18 Mean Difference (IV, Fixed, 95% CI) 6.4 [-2.89, 15.69]
3 Muscle strength bicep curls -
maximum isotonic dynamic
voluntary contraction
1 18 Mean Difference (IV, Fixed, 95% CI) 7.3 [-2.91, 17.51]
29Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
4 Work capacity - mean time until
exhaustion in cycle test
1 18 Mean Difference (IV, Fixed, 95% CI) 23.7 [2.63, 44.77]
5 Work capacity- mean distance
until exhaustion in cycle test
1 18 Mean Difference (IV, Fixed, 95% CI) 9.70 [1.51, 17.89]
6 Work capacity - mean distance
walked until exhaustion in
shuttle walking test
1 18 Mean Difference (IV, Fixed, 95% CI) 78.0 [-144.86,
300.86]
7 Quality of life 1 18 Mean Difference (IV, Fixed, 95% CI) -9.8 [-25.74, 6.14]
8 Myoglobin 1 30 Mean Difference (IV, Fixed, 95% CI) -21.0 [-48.35, 6.35]
Analysis 1.1. Comparison 1 Strength training versus control in myotonic dystrophy, Outcome 1 Muscle
strength - maximum isotonic knee torque extension.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 1 Strength training ver sus control in myotonic dystrophy
Outcome: 1 Muscle strength - maximum isotonic knee torque extension
Study or subgroup Training Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
Lindeman 1995 14 5.3 (12.9) 14 1.4 (8.2) 100.0 % 3.90 [ -4.11, 11.91 ]
Total (95% CI) 14 14 100.0 % 3.90 [ -4.11, 11.91 ]
Heterogeneity: not applicable
Test for overall effect: Z = 0.95 (P = 0.34)
-10 -5 0 5 10
Control Training
30Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Analysis 1.2. Comparison 1 Strength training versus control in myotonic dystrophy, Outcome 2 Muscle
strength - maximum isotonic knee torque flexion.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 1 Strength training ver sus control in myotonic dystrophy
Outcome: 2 Muscle strength - maximum isotonic knee torque flexion
Study or subgroup Training Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
Lindeman 1995 14 7.4 (11.4) 14 3.7 (8.6) 100.0 % 3.70 [ -3.78, 11.18 ]
Total (95% CI) 14 14 100.0 % 3.70 [ -3.78, 11.18 ]
Heterogeneity: not applicable
Test for overall effect: Z = 0.97 (P = 0.33)
-10 -5 0 5 10
Control Training
Analysis 1.3. Comparison 1 Strength training versus control in myotonic dystrophy, Outcome 3 Muscle
strength - maximum isometric voluntary contraction.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 1 Strength training ver sus control in myotonic dystrophy
Outcome: 3 Muscle strength - maximum isometric voluntar y contraction
Study or subgroup Training Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
Lindeman 1995 14 8.7 (14.7) 14 6.6 (11) 100.0 % 2.10 [ -7.52, 11.72 ]
Total (95% CI) 14 14 100.0 % 2.10 [ -7.52, 11.72 ]
Heterogeneity: not applicable
Test for overall effect: Z = 0.43 (P = 0.67)
-20 -10 0 10 20
Control Training
31Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Analysis 2.1. Comparison 2 Strength training versus control in facioscapulohumeral muscular dystrophy,
Outcome 1 Muscle strength elbow flexors - maximum voluntary isometric contraction.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 2 Strength training versus control in facioscapulohumeral muscular dystrophy
Outcome: 1 Muscle strength elbow flexors - maximum voluntar y isometric contraction
Study or subgroup Training Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
van der Kooi 2004 34 -0.06 (1.93) 31 -0.6 (1.87) 100.0 % 0.54 [ -0.38, 1.46 ]
Total (95% CI) 34 31 100.0 % 0.54 [ -0.38, 1.46 ]
Heterogeneity: not applicable
Test for overall effect: Z = 1.15 (P = 0.25)
-4 -2 0 2 4
Control Training
Analysis 2.2. Comparison 2 Strength training versus control in facioscapulohumeral muscular dystrophy,
Outcome 2 Muscle strength elbow flexors - dynamic strength.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 2 Strength training versus control in facioscapulohumeral muscular dystrophy
Outcome: 2 Muscle strength elbow flexors - dynamic strength
Study or subgroup Training Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
van der Kooi 2004 34 2.56 (2.09) 31 1.39 (1.97) 100.0 % 1.17 [ 0.18, 2.16 ]
Total (95% CI) 34 31 100.0 % 1.17 [ 0.18, 2.16 ]
Heterogeneity: not applicable
Test for overall effect: Z = 2.32 (P = 0.020)
-10 -5 0 5 10
Control Training
32Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Analysis 2.3. Comparison 2 Strength training versus control in facioscapulohumeral muscular dystrophy,
Outcome 3 Muscle strength ankle dorsiflexors - maximum isometric voluntary contraction.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 2 Strength training versus control in facioscapulohumeral muscular dystrophy
Outcome: 3 Muscle strength ankle dor siflexors - maximum isometric voluntary contraction
Study or subgroup Treatment Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
van der Kooi 2004 34 -1.13 (4.28) 31 -1.56 (4.16) 100.0 % 0.43 [ -1.62, 2.48 ]
Total (95% CI) 34 31 100.0 % 0.43 [ -1.62, 2.48 ]
Heterogeneity: not applicable
Test for overall effect: Z = 0.41 (P = 0.68)
-10 -5 0 5 10
Control Training
Analysis 2.4. Comparison 2 Strength training versus control in facioscapulohumeral muscular dystrophy,
Outcome 4 Muscle strength ankle dorsiflexors - dynamic strength.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 2 Strength training versus control in facioscapulohumeral muscular dystrophy
Outcome: 4 Muscle strength ankle dor siflexors - dynamic strength
Study or subgroup Treatment Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
van der Kooi 2004 34 -1.5 (2.68) 31 -1.06 (2.78) 100.0 % -0.44 [ -1.77, 0.89 ]
Total (95% CI) 34 31 100.0 % -0.44 [ -1.77, 0.89 ]
Heterogeneity: not applicable
Test for overall effect: Z = 0.65 (P = 0.52)
-10 -5 0 5 10
Control Training
33Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Analysis 3.1. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 1
Muscle strength shoulder press - maximum dynamic isotonic voluntary contraction.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 3 Aerobic exercise and strength training in mitochondrial myopathy
Outcome: 1 Muscle strength shoulder press - maximum dynamic isotonic voluntary contraction
Study or subgroup Experimental Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
Cejudo 2005 9 5.7 (11) 9 10.7 (10) 100.0 % -5.00 [ -14.71, 4.71 ]
Total (95% CI) 9 9 100.0 % -5.00 [ -14.71, 4.71 ]
Heterogeneity: not applicable
Test for overall effect: Z = 1.01 (P = 0.31)
-100 -50 0 50 100
Favours experimental Favours control
Analysis 3.2. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 2
Muscle strength butterfly - maximum dynamic isotonic voluntary contraction.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 3 Aerobic exercise and strength training in mitochondrial myopathy
Outcome: 2 Muscle strength butterfly - maximum dynamic isotonic voluntary contraction
Study or subgroup Experimental Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
Cejudo 2005 9 7 (9) 9 0.6 (11) 100.0 % 6.40 [ -2.89, 15.69 ]
Total (95% CI) 9 9 100.0 % 6.40 [ -2.89, 15.69 ]
Heterogeneity: not applicable
Test for overall effect: Z = 1.35 (P = 0.18)
-100 -50 0 50 100
Favours experimental Favours control
34Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Analysis 3.3. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 3
Muscle strength bicep curls - maximum isotonic dynamic voluntary contraction.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 3 Aerobic exercise and strength training in mitochondrial myopathy
Outcome: 3 Muscle strength bicep curls - maximum isotonic dynamic voluntar y contraction
Study or subgroup Experimental Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
Cejudo 2005 9 8 (10) 9 0.7 (12) 100.0 % 7.30 [ -2.91, 17.51 ]
Total (95% CI) 9 9 100.0 % 7.30 [ -2.91, 17.51 ]
Heterogeneity: not applicable
Test for overall effect: Z = 1.40 (P = 0.16)
-100 -50 0 50 100
Favours experimental Favours control
Analysis 3.4. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 4
Work capacity - mean time until exhaustion in cycle test.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 3 Aerobic exercise and strength training in mitochondrial myopathy
Outcome: 4 Work capacity - mean time until exhaustion in cycle test
Study or subgroup Experimental Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
Cejudo 2005 9 21 (28) 9 -2.7 (16) 100.0 % 23.70 [ 2.63, 44.77 ]
Total (95% CI) 9 9 100.0 % 23.70 [ 2.63, 44.77 ]
Heterogeneity: not applicable
Test for overall effect: Z = 2.20 (P = 0.027)
-100 -50 0 50 100
Favours experimental Favours control
35Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Analysis 3.5. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 5
Work capacity- mean distance until exhaustion in cycle test.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 3 Aerobic exercise and strength training in mitochondrial myopathy
Outcome: 5 Work capacity- mean distance until exhaustion in cycle test
Study or subgroup Experimental Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
Cejudo 2005 9 8.8 (11) 9 -0.9 (6) 100.0 % 9.70 [ 1.51, 17.89 ]
Total (95% CI) 9 9 100.0 % 9.70 [ 1.51, 17.89 ]
Heterogeneity: not applicable
Test for overall effect: Z = 2.32 (P = 0.020)
-100 -50 0 50 100
Favours experimental Favours control
Analysis 3.6. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 6
Work capacity - mean distance walked until exhaustion in shuttle walking test.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 3 Aerobic exercise and strength training in mitochondrial myopathy
Outcome: 6 Work capacity - mean distance walked until exhaustion in shuttle walking test
Study or subgroup Experimental Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
Cejudo 2005 9 95 (222) 9 17 (259) 100.0 % 78.00 [ -144.86, 300.86 ]
Total (95% CI) 9 9 100.0 % 78.00 [ -144.86, 300.86 ]
Heterogeneity: not applicable
Test for overall effect: Z = 0.69 (P = 0.49)
-200 -100 0 100 200
Favours experimental Favours control
36Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
Analysis 3.7. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 7
Quality of life.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 3 Aerobic exercise and strength training in mitochondrial myopathy
Outcome: 7 Quality of life
Study or subgroup Experimental Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
Cejudo 2005 9 -8.3 (16.8) 9 1.5 (17.7) 100.0 % -9.80 [ -25.74, 6.14 ]
Total (95% CI) 9 9 100.0 % -9.80 [ -25.74, 6.14 ]
Heterogeneity: not applicable
Test for overall effect: Z = 1.20 (P = 0.23)
-100 -50 0 50 100
Favours experimental Favours control
Analysis 3.8. Comparison 3 Aerobic exercise and strength training in mitochondrial myopathy, Outcome 8
Myoglobin.
Review: Strength training and aerobic exercise training for muscle disease
Comparison: 3 Aerobic exercise and strength training in mitochondrial myopathy
Outcome: 8 Myoglobin
Study or subgroup Experimental Control Mean Difference Weight Mean Difference
N Mean(SD) N Mean(SD) IV,Fixed,95% CI IV,Fixed,95% CI
Lindeman 1995 15 20 (34) 15 41 (42) 100.0 % -21.00 [ -48.35, 6.35 ]
Total (95% CI) 15 15 100.0 % -21.00 [ -48.35, 6.35 ]
Heterogeneity: not applicable
Test for overall effect: Z = 1.51 (P = 0.13)
-100 -50 0 50 100
Favours experimental Favours control
37Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
A P P E N D I C E S
Appendix 1. CENTRAL (through Wiley Interscience, The Cochrane Library 2009, issue 3) search
strategy
#1“muscle dis*” or “muscle weakness” or “muscular dis*” or “neuromuscular dis*” or myopath* or dystroph* or myotoni* or myositis
or polio* or “muscle fibre*” or “muscle strength” or fibromyalgia
#2“exercise therapy” or “exercise training” or “exercise program*” or “strength training” or “aerobic training” or “aerobic exercise” or
“training program” or “resistive exercise” or “endurance training” or “muscle exercise”
#3(#1 AND #2)
Appendix 2. Cochrane Neuromuscular Disease Group Specialised Trials Register search strategy
(“muscle dis*” or “muscle weakness” or “muscular dis*” or “neuromuscular dis*” or myopath* or dystroph* or myotoni* or myositis or
polio* or “muscle fibre*” or “muscle strength” or fibromyalgia) and (“exercise (therapy” or “exercise training” or “exercise program*”
or “strength training” or “aerobic training” or “aerobic exercise” or “training program” or “resistive exercise” or “endurance training”
or “muscle exercise”
Appendix 3. MEDLINE (through OvidSP 2009 July week 3) search strategy
1 (muscle disease* or muscle disorder* or muscular disease* or muscular disorder* or neuromuscular disease* or neuromuscular disorder*
or myopath* or dystroph* or myotoni* or myositis).mp. or exp muscle disease/
2 (exercise therap* or exercise program* or exercise training or strength training or aerobic training or aerobic exercis* or training
program* or resistive exercis* or resistiv training or endurance exercis* or endurance training or muscle exercis*).mp. or exp exercise/
or exp muscle exercise/ or exp excessive training/ or exp kinesiotherapy/
3 (trial* or random*).mp. or exp clinical trail/ or major clinical study/ or exp controlled study/
4 1 and 2 and 3
Appendix 4. EMBASE (through OvidSP 2009 week 29) and EMBASE Classic (through OvidSp)
search strategy
1 (muscle disease* or muscle disorder* or muscular disease* or muscular disorder* or neuromuscular disease* or neuromuscular disorder*
or myopath* or dystroph* or myotoni* or myositis).mp. or exp muscle disease/
2 (exercise therap* or exercise program* or exercise training or strength training or aerobic training or aerobic exercis* or training
program* or resistive exercis* or resistiv training or endurance exercis* or endurance training or muscle exercis*).mp. or exp exercise/
or exp muscle exercise/ or exp excessive training/ or exp kinesiotherapy/
3 (trial* or random*).mp. or exp clinical trail/ or major clinical study/ or exp controlled study/
4 1 and 2 and 3
Appendix 5. CINAHL (through EBSCOhost) search strategy
S4 S1 and S2 and S3
S3 Tx Trial* OR Tx random* OR PT Systematic review OR PT Clinical trial OR MH “Clinical trials+”
S2 Tx (exercise therap* or exercise program* or exercise training or strength training or aerobic training or aerobic exercis* or training
program* or resistive exercis* or resistive training or endurance exercis* or endurance training or muscle exercis* ) or MH “Therapeutic
exercise+”
S1 Tx (muscle disease* or muscle disorder* or muscular disease* or muscular disorder* or neuromuscular disease* or neuromuscular
disorder* or myopath* or dystroph* or myotoni* or myositis) or MH “Muscular Diseases+”
38Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
W H A T ’ S N E W
Last assessed as up-to-date: 13 July 2009.
20 July 2009 New citation required and conclusions have changed Search updated to July 2009. Review updated to include a
new study of people with mitochondrial myopathy (Cejudo
2005). The results and conclusions of the review have been
amended accordingly.
H I S T O R Y
Protocol first published: Issue 4, 2002
Review first published: Issue 1, 2005
2 July 2008 Amended Converted to new review format.
23 September 2004 New citation required and conclusions have changed Substantive amendment.
C O N T R I B U T I O N S O F A U T H O R S
I Riphagen searched all databases. NBM Voet and EL van der Kooi identified and assessed potentially relevant studies, and extracted
the data from included studies. NBM Voet prepared the final draft. ACH Geurts, EL van der Kooi and E Lindeman edited each draft
and agreed the final text of the review.
D E C L A R A T I O N S O F I N T E R E S T
One author (van der Kooi) carried out a randomised controlled trial on the effect of strength training and albuterol in facioscapulo-
humeral muscular dystrophy (van der Kooi 2004). The other (Lindeman) has co-ordinated a randomised controlled trial on the effects
of strength training in myotonic dystrophy (Lindeman 1995).
D I F F E R E N C E S B E T W E E N P R O T O C O L A N D R E V I E W
In this review we have excluded studies in which the the contralateral non-exercised side served as the control limb. Although there is
evidence in the literature as to why these trials should not be included this was not pre-specified as an exclusion criteria in the protocol.
Therefore, we will revise the protocol on the next update.
39Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
I N D E X T E R M S
Medical Subject Headings (MeSH)
∗Exercise; Mitochondrial Myopathies [∗rehabilitation]; Muscular Dystrophy, Facioscapulohumeral [∗rehabilitation]; Myotonic Dys-
trophy [∗rehabilitation]; Physical Fitness; Randomized Controlled Trials as Topic
MeSH check words
Humans
40Strength training and aerobic exercise training for muscle disease (Review)
Copyright © 2010 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.
