Abstract
Objective
Data Sources
Study Selection
Data Extraction
Data Synthesis
Conclusions
Key Words
Methods
Search Strategy
Selection Criteria
Procedure for Inclusion

Methodologic Quality
Classification of Included Studies
Study | Design | V | D | N | Diagnosis | Type of Intervention (type of exercises, type of muscles, load, number of repetitions, sets, progression) | Frequency and Duration | Body Functions | Activities/Participation |
---|---|---|---|---|---|---|---|---|---|
1. Motoneuron disorders | |||||||||
Muscle strengthening exercises | |||||||||
Drory et al 17 | RCT | 3 | 7 | 25 | ALS | AROM exercises for muscles of limbs and trunk against modest loads | Twice a day 15min for 12mo | 1/8 | 1/4 |
Klein et al 50 | RCT | 4 | 4 | 29 | PPS | 3−5 AROM exercises for hip and knee extensors against gravity. RPE 12−14, increasing number of repetitions | Daily 30min for 16wk | 2/5 | NV |
Prins et al 55 | RCT | 4 | 5 | 16 | PPS | Aquatic exercise: swimming and exercises for arms and legs using fins and paddles | 3×/wk 45−70min for 8wk | 7/39 | NV |
Chan et al 48 | RCT | 3 | 7 | 10 | PPS | 3 sets of eight 3−5s 50% MVC of thenar muscle. If possible increase of 10% a week until 70% MVC level | 3×/wk for 12wk | 2/2 | NV |
Fillyaw et al 59 | OD | 4 | 5 | 17 | PPS | Full AROM exercises for knee or elbow: 3 sets of 10 repetitions with weights. 1st set weight of 50% of 10-RM; 2nd set 75% and 3rd set 100% of 10-RM. 10-RM weight was evaluated every 2 weeks | Every other day up to 2y | 2/3 | NV |
Einarsson 58 | OD | 4 | 7 | 12 | PPS | 12 sets of 8 maximal isokinetic knee extensors contractions at 180°/s angular speed interposed with 12 sets of isolated 4-s isometric contractions | 3×/wk (96s) for 6wk | 5/7 | NV |
Agre et al 56 | OD | 5 | 6 | 12 | PPS | 6 repetitions (30s each) of knee extension with weight of 1−1.5kg until RPE 17 or until 10 reps. If RPE <17 weight was increased (.23 kg) | Every other day up to 12wk | 0/5 | NV |
Agre et al 57 | OD | 5 | 6 | 7 | PPS | Tuesday and Friday: 3 sets of 12 reps with ankle weights (1–1.5 kg). If RPE <19, the weight increased (.23kg) the next session. Monday and Thursday: 3 sets of 4 max effort isometric quadriceps contractions (5s) | 4d/wk for 12wk | 6/6 | NV |
Aerobic exercises | |||||||||
Jones et al 60 | RCT | 5 | 7 | 37 | PPS | Cycle ergometer at intensity of 70%−75% of HRR plus resting HR. Bouts of 2−5min, 1-min rest | 3×/wk 15−30min for 16wk | 4/7 | NV |
Dean and Ross 53 | CCT | 4 | 6 | 20 | PPS | Treadmill walking at comfortable speed, RPE ≤5 (somewhat heavy) | 3×/wk 20−40min for 6wk | 1/2 | |
Muscle strengthening and aerobic exercises | |||||||||
Willen et al 61 | CCT | 4 | 5 | 28 | PPS | Fitness in water: resistance and endurance activities, balance, stretching and relaxation, avoiding muscle fatigue | 2×/wk 40min for 8mo | 3/20 | 0/4 |
Lifestyle modification | |||||||||
Klein et al 50 | RCT | 4 | 4 | 29 | PPS | Lifestyle modification to avoid shoulder overuse | Monthly for 16wk | 1/5 | NV |
Muscle strengthening exercise and lifestyle modification | |||||||||
Klein et al 50 | RCT | 4 | 4 | 29 | PPS | 3−5 AROM exercises for hip and knee extensors against gravity, increasing number of repetitions. RPE 12−14 | Daily 30min for 16wk | 0/5 | NV |
2. Motor nerve root disorders and peripheral nerve disorders | |||||||||
Muscle strengthening exercises | |||||||||
Lindeman et al 13 , 25 | RCT | 6 | 7 | 29 | HMSN | Exercises of muscles of hip and knee. Weeks 1−8: 3 sets of 25 repetitions of 60% of 1-RM; weeks 9−16: 3 sets of 15 repetitions of 70% of 1-RM; from week 18: 10 repetitions of 80% of 1-RM | 3×/wk 30min for 24wk | 1/4 | 1/6 |
Chetlin et al 23 , 24 | OD | 5 | 6 | 20 | HMSN | 3 sets of varying repetitions for flexion and extension at 40% to 50% MVIS for knee and 20% to 30% MVIS for elbow. Every 4 weeks, resistance increased. Reps increased weekly from 4 to 6 to 8 to 10 | Daily for 12wk | 3/9 | 4/4 |
Muscle strengthening and aerobic exercises | |||||||||
Ruhland and Shields 51 | RCT | 4 | 5 | 28 | CPN | Exercises with Theraband with no resistance and progressing to light and medium resistance with 10 repetitions. Aerobic conditioning at 60%−70% of estimated maximal HR or “somewhat hard” on Borg RPE Scale | Daily 20min for 6wk | 2/4 | 1/11 |
3. Neuromuscular transmission disorders | |||||||||
Breathing exercises | |||||||||
Fregonezi et al 26 | RCT | 6 | 6 | 27 | MG | Ten minutes of diaphragmatic breathing, followed by 10-min interval-based inspiratory muscle training and 10min of pursed lips breathing. Initial load 20% of Pimax, increased to 30% in week 3, 45% in the fifth week, and 60% in week 7. | 3×/wk 45min for 8wk |
| |
4. Muscle disorders | |||||||||
Muscle strengthening exercises | |||||||||
Van der Kooi et al 14 | RCT | 7 | 7 | 65 | FSHD | Dynamic and isometric exercises for elbow flexors and ankle dorsiflexors, weeks 1−8: 2 sets of 5−10 repetitions with 10-RM weights, interspersed with 30s isometric exercise with 10-RM; weeks 9−17: sets of 8 reps with 8-RM weights; from week 18: 5 reps of 5-RM. | 3×/wk for 52wk | 2/12 | 0/3 |
Lindeman et al 13 , 25 | RCT | 6 | 7 | 33 | MMD | Exercises of muscles of hip and knee. Weeks 1−8: 3 sets of 25 reps of 60% of 1-RM; weeks 9−16: 3 sets of 15 reps of 70% of 1-RM; from week 18: 10 reps of 80% of 1-RM | 3×/wk 30min for 24wk | 0/4 | 0/6 |
Belanger and Noel 52 | CCT | 4 | 6 | 6 |
| Maximal contractions of dorsiflexors and plantarflexors with ankle exerciser. Two sets of 10 MVC of 3−5s for dorsiflexors and for plantarflexors | Daily for 4mo | 0/2 | 0/4 |
Alexanderson et al 63 | OD | 6 | 6 | 11 |
| Warming up. Exercises for shoulder mobility and grip strength with a pulley apparatus, exercises for quadriceps and hip muscles, sit ups, and stretching. If FI score >38 exercises with weights (.25−2.0kg) were included. Also 15-min walking at self-selected speed | 5×/wk 15min for 12wk | 4/5 | 3/8 |
Arnadottir et al 64 | OD | 6 | 5 | 7 | sIBM | Exercises for shoulder mobility with a pulley apparatus, resistive exercises for shoulder and hip muscles, quadriceps and neck and trunk muscles. (10 reps of each exercise); weight cuffs (.25−2.0kg) depending on FI score (>38) and self-paced walking (15min) | 5×/wk for 12wk | 0/3 | NV |
Aldehag et al 62 | OD | 6 | 6 | 5 | MMD 1 | Hand exercises with silicone-based putty with isolated (1-2-3 sets of 3 reps or 1-2-3 sets of 5 reps) and mass (1-2-3 sets of 10 reps or 1-2-3 sets of 15 reps) movements, starting with 1 set of 3–5 reps in isolated movements and 1 set of 10−15 reps in mass movements. Number of sets increased every 4 weeks. Stretching was also included | 3×/wk 4min for 12wk | 7/12 | 3/4 |
Sandin and Jonsson 65 | OD | 5 | 5 | 12 | WM | Hand exercises with silicone-based putty with isolated (1-2-3 sets of 3 reps or 1-2-3 sets of 5 reps) and mass (1-2-3 sets of 10 reps or 1-2-3 sets of 15 reps) movements, starting with 1 set of 3–5 reps in isolated movements and 1 set of 10−15 reps in mass movements. Number of sets increased every 4 weeks. Stretching was also included | 3×/wk 45min for 12wk | 4/14 | 1/2 |
Tollbäck et al 66 | OD | 4 | 6 | 9 | MMD | Maximal AROM exercises for knee extensors with weights on an iron shoe with increasing load. Week 1: 60% of 1-RM; week 2: 70% of 1-RM; thereafter 80% of 1-RM. 3 sets of 8 reps were performed | 3×/wk for 12wk | 1/4 | NV |
Aerobic exercises | |||||||||
Taivassalo et al 70 | OD | 4 | 5 | 24 |
| Treadmill exercise 70% to 85% of HRR | 3−4×/wk 20−30min for 8wk | 2/2 | 1/1 |
Taivassalo et al 69 | OD | 5 | 5 | 10 | MM |
| 3−4×/wk 20−30min for 8wk | 4/4 | 1/1 |
Taivassalo et al 71 | OD | 5 | 5 | 10 | MM | Cycling at 70%−80% of maximal HR | 3−4×/wk 30−40min for 14wk | 4/6 | NV |
Trenell et al 72 | OD | 5 | 5 | 10 | MM | Cycling at 70%−80% of maximal HR | 3×/wk 30min for 12wk | 2/3 | 1/1 |
Haller et al 67 | OD | 4 | 5 | 8 | MD | Cycling at 60%−70% of maximal HR | 4×/wk 30−40min for 14wk | 3/3 | NV |
Olsen et al 68 | OD | 5 | 5 | 8 | FSHD | Cycling at 65% of V̇o2max | 5×/wk 35min for 12wk | 2/2 | NV |
Sunnerhagen et al 73 | OD | 5 | 5 | 8 | HM |
| 5×/wk 30min for 8wk | 1/3 | 1/4 |
Muscle strengthening and aerobic exercises | |||||||||
Cejudo et al 49 | RCT | 4 | 7 | 20 | MM | Cycling 30min at 70% of peak work rate; and 3 dynamic isotonic arm weight (50% 1-RM, repeated every 2wk) lifting procedures through full ROM; shoulder press (shoulder, upper back, arm muscles), butterfly (pectoralis major) and biceps curls (biceps brachii, brachialis). Week 1 and 2: 1 set of 10−15 reps; next weeks: 2−3 sets | 3×/wk 60min for 12wk | 13/18 | 2/4 |
Wiesinger et al 27 | RCT | 6 | 7 | 14 | DM/PM |
| 2−3×/wk 60min for 6wk | 2/2 | 1/1 |
Wiesinger et al 54 | CCT | 5 | 7 | 8 | DM/PM |
| 1−3×/wk 60min for 6mo | 6/8 | 1/1 |
Muscle strengthening exercises, respiratory exercises and mud/massage/bath | |||||||||
Varju et al 74 | OD | 4 | 5 | 21 | DM/PM |
| 5×/wk 40−60min for 3wk | 9/18 | 2/4 |
Breathing exercises | |||||||||
Ugalde et al 75 | OD | 4 | 5 | 11 | MMD | Pursed lips breathing as voluntary expiratory blowing through partially closed lips to create a resistance at the mouth compared to matched volume breathing (exhaling without pursing lips) and tidal breathing (comfortable breathing without pursing lips) | 120s of each breathing condition | 6/9 | NV |
5. Heterogeneous neuromuscular disorders | |||||||||
Strengthening exercises | |||||||||
Aitkens et al 76 | OD | 4 | 4 | 27 | MMD (n=12) HMSN (n=8) LGD (n=3) SMA (n=2) FSHD (n=2) | Initially 3 sets of 4 reps with speed of 30/s for knee extensor with ankle weights of 30% of max isometric knee extension force; and also for elbow flexors with weight of 10% of max elbow flexion force. Also 3 sets of 4 reps for hand grip exercises. Increasing resistance and reps | 3×/wk 15−20min for 12wk | 7/11 | NV |
Kilmer et al 77 | OD | 6 | 5 | 10 | MMD (n=5) LGD (n=3) HMSN (n=2) | Knee extensors and elbow flexors with ankle and wrist cuff weights, 1 set of 10 reps with 12-RM, gradually increasing until 5 sets of 10 reps during 4d/wk. After week 5, resistance was increased by .45kg/wk (1lb/wk) if feasible. After week 9, from 4 to 5 sets | 3−4×/wk for 12wk | 3/10 | NV |
Aerobic exercises | |||||||||
Florence and Hagberg 28 | CCT | 6 | 5 | 12 | CCD (n=1) NM (n=1) MyM (n=1) SMA (n=1) CMD (n=2) LGD (n=3) CMT (n=2) FSHD (n=1) | Cycling on cycle ergometer 5-min exercise bouts, 2-min rest at 70% of V̇o2max: resistance was adjusted to continually elicit 70% of V̇o2max | 3×/wk for 12wk | 3/5 | 0/2 |
Wright et al 78 | OD | 4 | 5 | 11 | MMD (n=7); HMSN (n=3) LGD (n=1) |
| 3−4×/wk 15−20−30min 12wk | 1/33 | NV |
Muscle strengthening and aerobic exercises | |||||||||
Dawes et al 21 | RCT | 7 | 6 | 18 | Becker MD (n=4) MMD (n=4) PM (n=1) FSHD (n=3) IMB (n=1) LGD (n=4) CM (n=1) | Walking for as long as possible at light subjective exercise intensity up to 20min, then increase toward a moderate intensity (Borg CR-10 scale). Two exercises for each leg muscle endurance and core stability increasing the number of repetitions and range until 2.5min for each exercise. Then increasing difficulty with gravity as resistance, increasing number of repetitions | Alternate days for 8wk |
|
Best Evidence Synthesis
Results
Selection of Studies
Methodologic Quality
Best Evidence Synthesis
Motoneuron Disorders
Muscle strengthening exercises
Aerobic exercises
A combination of muscle strengthening and aerobic exercises
Lifestyle modification with or without muscle strengthening exercises
Motor Nerve Root Disorders and Peripheral Nerve Disorders
Muscle strengthening exercises
A combination of muscle strengthening and aerobic exercises
Neuromuscular Transmission Disorders
Breathing exercises
Muscle Disorders
Muscle strengthening exercises
Aerobic exercises
A combination of muscle strengthening and aerobic exercises
A combination of muscle strengthening and breathing exercises and mud/massage/bath
Breathing exercises
Heterogeneous Group of Patients With NMD
Muscle strengthening exercises
Aerobic exercises
Muscle strengthening and aerobic exercises
Adverse Effects
Other PT Modalities
Discussion
Methodology
Exercise Intensity
Uniformity
Conclusions
Acknowledgment
References
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Footnotes
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