The effects of 12 weeks' resistance training on psychological parameters and quality of life in adults with Facioscapulohumeral, Becker, and Limb-girdle dystrophies.

PURPOSE: Investigate the impact of 12-weeks' moderate-intensity resistance training on psychological parameters in ambulatory adults with Facioscapulohumeral, Becker, and Limb-girdle muscular dystrophy. METHODS: Seventeen adults with Facioscapulohumeral (n = 6), Limb-girdle (n = 6; types 2A, 2B, 2L, and 2I), or Becker (n = 5) muscular dystrophy took part. Participants were tested at baseline (PRE), after a 12-week control period (PRE2), and after a 12-week supervised resistance training programme (POST). Training included multi-joint and single-joint resistance exercises. Outcomes from self-report questionnaires were health-related quality of life, depressive symptoms, trait anxiety, self-esteem, and physical self-worth. RESULTS: No difference in outcome measures, except depressive symptoms, was found in the control period (PRE to PRE2). Symptoms of depression were reduced by 9% from PRE to PRE2 (p < 0.05) and by a further 19% from PRE2 to POST (p < 0.05). Other changes from PRE2 to POST were that trait anxiety reduced by 10%, self-esteem increased by 10%, physical self-worth increased by 20%, and quality of life improved in 8 domains (p < 0.05). CONCLUSION: These findings demonstrate the positive impact of moderate-intensity resistance training on psychological health and quality of life in adults with Facioscapulohumeral, Becker, and Limb-girdle muscular dystrophies.Implications for rehabilitationResistance training can have a positive impact on psychological health and quality of life in adults with Facioscapulohumeral, Becker, and Limb-girdle muscular dystrophy.Healthcare professionals should consider including moderate-intensity resistance training within the management and treatment programmes of adults with Facioscapulohumeral, Becker, and Limb-girdle muscular dystrophy.

Psychological parameters impact health-related quality of life in mental and physical domains in adults with muscular dystrophy.

The impacts of potentially treatable psychological parameters on quality of life are relatively unreported in adults with Facioscapulohumeral, Becker and Limb-girdle muscular dystrophy. The purpose of this study was to compare quality of life, psychological parameters, and physical function between adults with muscular dystrophy and controls, and to examine relationships among these parameters in muscular dystrophy. Twenty-one adults with muscular dystrophy (n = 7 Becker, n = 8 Facioscapulohumeral, n = 6 Limb-girdle) and ten age-matched controls participated. Outcome measures were health-related quality of life, depressive symptoms, trait anxiety, self-esteem, physical self-worth and six-minute walk distance. Quality of life scores were lower in the muscular dystrophy groups than the control (p < .05). Depressive symptoms had the greatest association with quality of life in the Mental Health domain (r= -0.89, p < .001). Depressive symptoms also had the most associations with quality of life (7 of 10 domains), followed by trait anxiety (6 of 10 domains), physical self-worth (5 of 10 domains), self-esteem (4 of 10 domains) and six-minute walk distance (3 of 10 domains). Psychological parameters and, to a lesser extent, physical function impact quality of life in muscular dystrophy. This study provides a rationale to include psychological assessment and treatment within muscular dystrophy healthcare.

The Effects of Resistance Exercise Training on Strength and Functional Tasks in Adults With Limb-Girdle, Becker, and Facioscapulohumeral Dystrophies.

Background: The inclusion of resistance training in the treatment and management of muscular dystrophy has previously been discouraged, based on mainly anecdotal evidence. There remains a lack of experimental investigation into resistance training in individuals with muscular dystrophy. The aim of the current study was therefore, to determine the effect of a 12-week resistance training programme on muscle strength and functional tasks in ambulatory adults with muscular dystrophy. Methods: Seventeen ambulatory adults with muscular dystrophy (Facioscapulohumeral muscular dystrophy: n = 6, Limb-Girdle muscular dystrophy: n = 6, Becker muscular dystrophy: n = 5) were recruited for this study. Participants attended three testing sessions: one session at baseline, one session after a 12-week control period and one session after a 12-week resistance training period. Each testing session consisted of measurements of isometric knee extensor and knee flexor maximum voluntary contraction (MVC) torque (Cybex dynamometer). Participants also completed a timed sit-to-stand, a four steps-stair ascent, and a four steps-stair decent. The 12-week resistance training period consisted of two supervised sessions a week. Each training session included a 5-min warm-up, a step-up exercise, free-standing or assisted squats, knee flexion and knee extension exercises, and an additional 6 single-joint exercises specific to each individual's needs. Results: Knee flexor MVC torque increased by 13% after the 12-week resistance training programme (p < 0.05), with no change over the control period. Knee extensor MVC torque did not significantly change after the training programme or the control period. Time taken to complete sit-to-stand, stair ascent and stair descent all decreased (improved) following the 12-week training programme (p < 0.05). Conclusions: A twice-a-week, 12-week, resistance training programme resulted in increased knee flexion strength and improvements in functional tasks in ambulatory adults with muscular dystrophy. This provides support for the inclusion of resistance training in the treatment programmes for these forms of muscular dystrophy.

The cardiorespiratory response and physiological determinants of the assisted 6-minute handbike cycle test in adult males with muscular dystrophy.

INTRODUCTION: The assisted 6-minute cycle test (A6MCT) distance was assessed in adults with muscular dystrophy (MD). METHODS: Forty-eight males, including those with Duchenne MD (DMD), limb-girdle MD (LGMD), fascioscapulohumeral MD (FSHD), and Becker MD (BMD), as well as a group without MD (CTRL), completed handgrip strength (HGS), lung function [forced expiratory volume in 1 second (FEV1 ) and forced vital capacity (FVC)], body fat, and biceps thickness assessments. During the A6MCT, ventilation (VE), oxygen uptake (VO2 ), carbon dioxide (VCO2 ), and heart rate (HR) were recorded. RESULTS: A6MCT and HGS were lower in MD than CTRL subjects. FEV1 , FVC, and biceps thickness were lower in MD than CTRL; lower in DMD than BMD, LGMD, and FSHD; but were not different between BMD, LGMD, and FSHD. A6MCT correlated with HGS, FEV1 , FVC, body fat, VO2 , VCO2 , HR, and VE (r = 0.455-0.708) in pooled BMD, LGMD, and FSHD participants. DISCUSSION: A shorter A6MCT distance in adult males with MD was attributable to HGS and lung function. The A6MCT is appropriate for assessment of physical function in adults with MD. Muscle Nerve 58: 427-433, 2018.

Resting Energy Expenditure in Adults with Becker's Muscular Dystrophy.

PURPOSE: The purpose of this study was: 1) To compare Resting energy expenditure (REE) in adult males with Becker's Muscular Dystrophy (BeMD, n = 21, 39 ±12 years) and healthy controls (CTRL, n = 12, 37 ±12 years) 2) Determine whether other physiological parameters correlate with REE in BeMD, and 3) Compare current prediction methods of REE with measured REE. METHODS: REE was calculated via indirect calorimetry using continuous, expired gas analysis following an overnight fast. Fat free mass (FFM) and fat mass were measured by bioelectrical impedance. B-mode ultrasound measured Tibialis Anterior (TA) and Gastrocnemius Medialis (GM) anatomical cross sectional area (ACSA). The Bone Specific Physical Activity Questionnaire measured physical activity. RESULTS: No difference in REE was found between CTRL and BeMD groups (1913 ±203 & 1786 ±324 Kcal respectively). Other physiological comparisons showed increased fat mass (+54%), decreased TA ACSA (-42%), increased GM ACSA (+25%) as well as reduced respiratory function (FVC -28%; FEV1-27%) in BeMD adults compared to controls. REE estimated from prediction equations (Schofield's) in Muscular Dystrophy were different from measured REE (P<0.05, bias = -728kcal), while the Mifflin equation was no different from measured REE (r2 = 0.58, Bias = -8kcal). Within the present BeMD, REE predicted from FFM (REE = FFM x 34.57-270; r2 = 0.85) and body mass (REE = BM x 15.65 + 421.5; r2 = 0.66), were not different from measured REE (bias equals 0 and 0.2kcals, respectively). CONCLUSIONS: Despite no differences in REE between CTRL and BeMD adults, increased fat masses highlights the requirement for explicit nutritional guidelines, as well as maintenance of physical activity levels, where possible. Prediction equations are frequently used in clinical settings, however these have been shown to be less accurate in BeMD; therefore, the equations proposed here should be used where possible.