Van der Kooi 2004.

Methods	Evaluator‐blind, parallel‐group RCT
Participants	Sample size Intervention group: 19 adults with FSHD type 1, control group 16 adults with FSHD type 1 Inclusion criteria Eligible participants were aged 18‐65 years, were willing to train if allocated to the training group, agreed to refrain from training if allocated to the non‐training group, and agreed to use the study medication as prescribed. Participants had to have at least 2 trainable muscle groups (fully supinated elbow flexion Medical Research Council (MRC) 3, ankle dorsiflexor 0° dorsiflexion from neutral ankle position). Exclusion criteria Inability to walk independently (ankle‐foot orthoses and canes were accepted); history of (treated) hypertension, heart failure, or ischaemic heart disease, arrhythmias, diabetes mellitus, or thyrotoxicosis; use of sympathicomimetics, beta‐blockers, or systemic corticosteroids during the last 3 months; (planned) pregnancy or breastfeeding; and articular diseases of the elbow or ankle joints. Participants with abnormalities on their electrocardiogram, but without a cardiovascular history or hypertension, were seen by the cardiologist to decide on their inclusion. Baseline demographics The training group comprised 8 women and 11 men, mean age 36 years (SD 9). The control group comprised 7 women and 9 men, mean age 39 years (SD 9).
Interventions	Strength training vs no training (and as add‐on in a double‐blind, randomised controlled design albuterol or placebo) Type of training and exercise Dynamic and isometric strength training with weights Intensity Individualised progressive overload, 2 sets dynamic from 10 repetitions at 10RM, via 8 repetitions at 8RM, to 5 repetitions at 5RM, and 30 s isometric with same weight Frequency 3 times/week Setting At home (the Netherlands) Duration Session: within 30 min. Programme: 52 weeks Muscle groups Elbow flexors, ankle dorsiflexors Supervision Supervised at home by a physical therapist
Outcomes	Primary Difference in muscle strength of elbow flexors and ankle dorsiflexors after 52 weeks using the MVIC Secondary Muscle endurance (MVIC Force‐Time Integral) and dynamic muscle strength (1RM). Other measures included functional tests and timed motor performance tasks Time‐points measured Before and after 52 weeks of control or training period
Dates	Study dates not reported
Funding/ declarations of interest	"Supported by a government grant of the Health Research and Development Council of the Netherlands (ZON‐MW), the Prinses Beatrix Fonds, the Dutch Public Fund for Neuromuscular Disorders (VSN), and the Dutch FSHD Foundation"
Notes	Outcomes are presented for the 4 treatment groups (i.e. the 4 combinations of training vs non‐training, and albuterol vs placebo). Effect sizes are presented by intervention as well
Risk of bias
Bias	Authors' judgement	Support for judgement
Random sequence generation (selection bias)	Low risk	Quote: " (...) participants were randomly assigned to one of the four treatment groups according to a computer generated randomisation list"
Allocation concealment (selection bias)	Unclear risk	Quote: "information on the assignment to training or non‐training was disclosed to the participants by the physical therapist"
Blinding of participants and personnel (performance bias) All outcomes	High risk	Not blinded as it is impossible to blind exercise training compared to no exercise training.
Blinding of outcome assessment (detection bias) All outcomes	Low risk	Quote: "The RM measurements were performed 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 1RM measurement for assessing dynamic strength, was performed by the physical therapist, who supervised the training, and was therefore not blinded to the allocation to training or non‐training. Unblinding during the trial was adequately registered. Allocation to training or non‐training was unmasked in 3 cases, due to unintentional remarks
Incomplete outcome data (attrition bias) All outcomes	Low risk	Quote: "One patient stopped training because of recurring, training‐related muscle soreness and fatigue. Four participants stopped using their study medication because of side effects. Data for the participants who discontinued an intervention were analysed in the assigned treatment group" Although there was not total adherence, analysis was by ITT.
Selective reporting (reporting bias)	Low risk	Comment: no evidence found for selective reporting
Other bias	Low risk	Comment: no risk of bias from other sources detected