Both aerobic exercise and cognitive-behavioral therapy reduce chronic fatigue in FSHD: an RCT.

To investigate the effect of aerobic exercise training (AET) and cognitive-behavioral therapy (CBT) on chronic fatigue in patients with facioscapulohumeral muscular dystrophy (FSHD). We performed a multicenter, assessor-blinded, randomized clinical trial (RCT). Fifty-seven patients with FSHD type 1 with severe chronic fatigue were randomly allocated to AET, CBT, or usual care (UC). Outcomes were assessed before treatment, following 16 weeks of intervention, and after a 12-week follow-up. A linear mixed model for repeated measurements was used to study the estimated group differences. Following treatment, both the AET (28 participants) and CBT (25 participants) intervention groups had less fatigue relative to the UC group (24 participants), with a difference of -9.1 for AET (95% confidence interval [CI] -12.4 to -5.8) and -13.3 for CBT (95% CI -16.5 to -10.2). These beneficial effects lasted through follow-up, with a difference of -8.2 for AET (95% CI -12.4 to -5.8) and -10.2 for CBT (95% CI -14.0 to -6.3). The patients who received CBT had an increase in registered and experienced physical activity, sleep quality, and social participation. The patients who received AET had an increase in registered physical activity only. The increase in registered physical activity in both groups and the improvement in social participation following CBT were still present at follow-up. This RCT shows that AET and CBT can ameliorate chronic fatigue in patients with FSHD. This study provides Class III evidence that, in patients with FSHD type 1 and severe chronic fatigue, AET or CBT reduces the severity of chronic fatigue.

Voet N ，Bleijenberg G ，Hendriks J ，de Groot I ，Padberg G ，van Engelen B ，Geurts A ... -  《-》

Effect of aerobic exercise training and cognitive behavioural therapy on reduction of chronic fatigue in patients with facioscapulohumeral dystrophy: protocol of the FACTS-2-FSHD trial.

Voet NB ，Bleijenberg G ，Padberg GW ，van Engelen BG ，Geurts AC ... -  《BMC Neurology》

Quantitative MRI reveals decelerated fatty infiltration in muscles of active FSHD patients.

To investigate the effects of aerobic exercise training (AET) and cognitive-behavioral therapy (CBT), directed towards an increase in daily physical activity, on the progression of fatty infiltration and edema in skeletal muscles of patients with facioscapulohumeral muscular dystrophy (FSHD) type 1 by T2 MRI. Quantitative T2 MRI (qT2 MRI) and fat-suppressed T2 MRI of the thigh were performed at 3T on 31 patients, 13 of whom received usual care (UC), 9 AET, and 9 CBT. Muscle-specific fat fractions (%), derived from qT2 MRI, were recorded pretreatment and posttreatment. Intervention effects were analyzed by comparing fat fraction progression rates of the UC with the treated groups using Mann-Whitney tests, and intermuscle differences by a linear mixed model. Edematous hyperintense lesions were identified on the fat-suppressed T2 MRI. The intraclass correlation coefficient for reproducibility of qT2 MRI fat assessment was 0.99. In the UC group, the fat fraction increased by 6.7/year (95% confidence interval [CI] 4.3 to 9.1). This rate decreased to 2.9/year (95% CI 0.7 to 5.2) in the AET (p = 0.03) and 1.7/year (95% CI -0.2 to 3.6) in the CBT group (p = 0.00015). The treatment effect differed among individual muscles. Fewer new edematous lesions occurred after therapy. Fat fraction derived from qT2 MRI is a reproducible and sensitive biomarker to monitor the effects of increased physical activity in individual muscles. This biomarker reports a favorable effect of AET and CBT on the rate of muscular deterioration in FSHD as reflected in decelerated fat replacement. This study provides Class II evidence that for patients with FSHD type 1, both AET and CBT decrease the rate of fatty infiltration in muscles.

Janssen B ，Voet N ，Geurts A ，van Engelen B ，Heerschap A ... -  《-》

Mindfulness-based cognitive therapy for people with chronic fatigue syndrome still experiencing excessive fatigue after cognitive behaviour therapy: a pilot randomized study.

Cognitive behaviour therapy (CBT) is an effective treatment for chronic fatigue syndrome (CFS; sometimes known as myalgic encephalomyelitis). However, only a minority of patients fully recover after CBT; thus, methods for improving treatment outcomes are required. This pilot study concerned a mindfulness-based cognitive therapy (MBCT) intervention adapted for people with CFS who were still experiencing excessive fatigue after CBT. The study aimed to investigate the acceptability of this new intervention and the feasibility of conducting a larger-scale randomized trial in the future. Preliminary efficacy analyses were also undertaken. Participants were randomly allocated to MBCT or waiting list. Sixteen MBCT participants and 19 waiting-list participants completed the study, with the intervention being delivered in two separate groups. Acceptability, engagement and participant-rated helpfulness of the intervention were high. Analysis of covariance controlling for pre-treatment scores indicated that, at post-treatment, MBCT participants reported lower levels of fatigue (the primary clinical outcome) than the waiting-list group. Similarly, there were significant group differences in fatigue at 2-month follow-up, and when the MBCT group was followed up to 6 months post-treatment, these improvements were maintained. The MBCT group also had superior outcomes on measures of impairment, depressed mood, catastrophic thinking about fatigue, all-or-nothing behavioural responses, unhelpful beliefs about emotions, mindfulness and self-compassion. In conclusion, MBCT is a promising and acceptable additional intervention for people still experiencing excessive fatigue after CBT for CFS, which should be investigated in a larger randomized controlled trial. Only about 30% of people with chronic fatigue syndrome (CFS) recover after cognitive behaviour therapy (CBT); thus, methods for improving treatment outcomes are needed. This is the first pilot randomized study to demonstrate that a mindfulness-based intervention was associated with reduced fatigue and other benefits for people with CFS who were still experiencing excessive fatigue after a course of CBT. Levels of acceptability, engagement in the intervention and rated helpfulness were high. A larger-scale randomized controlled trial is required.

Rimes KA ，Wingrove J 《-》

Aerobic training and postexercise protein in facioscapulohumeral muscular dystrophy: RCT study.

To investigate the effect of regular aerobic training and postexercise protein-carbohydrate supplementation in patients with facioscapulohumeral muscular dystrophy (FSHD). In this randomized, double-blind, placebo-controlled parallel study, we randomized untrained men (n = 21) and women (n = 20) with FSHD (age 19-65 years) to 2 training groups-training with protein supplement (n = 18) and training with placebo supplement (n = 13)-and a nonintervention control group (n = 10). We assessed fitness, walking speed, muscle strength, questionnaires, and daily activity levels before and after 12 weeks of interventions. Training involved 36 sessions of 30-minute cycle-ergometer training. After each session, patients drank either a protein-carbohydrate or placebo beverage. In the trained participants, fitness, workload, and walking speed improved (10% [confidence interval (CI) 4%-15%], 18% [CI 10%-26%], 7% [CI 4%-11%], respectively, p < 0.001, number needed to treat = 2.1). Self-assessed physical capacity and health (Short Form-36) also improved. Muscle strength and daily activity levels did not change with training. Protein-carbohydrate supplementation did not result in further improvements in any tests compared to training alone. This randomized, controlled study showed that regular endurance training improves fitness, walking speed, and self-assessed health in patients with FSHD without causing muscle damage. Postexercise protein-carbohydrate supplementation does not add any further improvement to training effects alone. This study provides Class II evidence that regular aerobic training with or without postexercise protein-carbohydrate supplementation improves fitness and workload in patients with FSHD.

Andersen G ，Prahm KP ，Dahlqvist JR ，Citirak G ，Vissing J ... -  《-》