Objective and subjective measures of sleep in men with Muscular Dystrophy.

Purpose: Despite poor sleep quality being recognised in Duchenne Muscular Dystrophy, reports from milder forms of Muscular Dystrophy (MD), and accompanied associations with quality of life (QoL), pain and fatigue, remain limited however.

Methods: Adult males (n = 15 Beckers MD (BMD), n = 12 Limb-Girdle MD (LGMD), n = 12 Fascioscapulohumeral (FSHD), n = 14 non-MD (CTRL)) completed assessments of body composition (Bio-electrical impedance), sleep (7-day 24-hour tri-axial accelerometer, Pittsburgh Sleep Quality Index (PSQI) and Insomnia Severity Index, QoL (SF36-v2), pain (Visual analogue scale), fatigue (Modified Fatigue Index Scale) and functional assessments (Brookes and Vignos).

Results: FSHD and BMD reported worse sleep than CTRL on the PSQI.

Quantitative assessment of sitting time in ambulant adults with Muscular Dystrophy.

Background: Current investigations into physical behaviour in Muscular Dystrophy (MD) have focussed largely on physical activity (PA). Negative health behaviours such as sedentary behaviour (Physical Behaviour) and sitting time (Posture Classification) are widely recognised to negatively influence health, but by contrast are poorly reported, yet could be easier behaviours to modify.

Methods: 14 ambulant men with MD and 12 healthy controls (CTRL) subjects completed 7-days of free-living with wrist-worn accelerometry, assessing physical behaviour (SB or PA) and Posture Classification (Sitting or Standing), presented at absolute (minutes) or relative (% Waking Hours).

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 ( = 6), Limb-girdle ( = 6; types 2A, 2B, 2L, and 2I), or Becker ( = 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).

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.

12-Month changes of muscle strength, body composition and physical activity in adults with dystrophinopathies.

Purpose: Muscular dystrophy (MD) is an umbrella term for muscle wasting conditions, for which longitudinal changes in function and body composition are well established in children with Duchenne (DMD), however, changes in adults with DMD and Beckers (BMD), respectively, remain poorly reported. This study aims to assess 12-month changes in lower-limb strength, muscle size, body composition and physical activity in adults with Muscular Dystrophy (MD).

Methods: Adult males with Duchenne MD (DMD;  = 15) and Beckers MD (BMD;  = 12) were assessed at baseline and 12-months for body composition (Body fat and lean body mass (LBM)), Isometric maximal voluntary contraction (Knee-Extension (KEMVC) and Plantar-Flexion (PFMVC)) and physical activity (tri-axial accelerometry).

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

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.

Quality of life in adults with muscular dystrophy.

Background: Muscle weakness is a defining characteristic of Muscular Dystrophy (MD); however, yet while speculated, objective measures of muscle weakness has not been reported in relation to quality of life in adults with MD.

Objectives: 1) compare the self-reported QoL of adults with Duchenne MD (DMD), Beckers MD (BMD), Limb-Girdle MD (LGMD) and Fascioscapulohumeral MD (FSHD, and a non-MD (CTRL) group; 2) present and compare between groups measures of Impairment (Muscle Strength and Activities of Daily Living) and Perception (Fatigue, Pain and Self-Efficacy); and 3) identify associations between QoL domains and measures of Impairment and Perception (See above).

Methods: Seventy-Five males, including MD classifications DMD, BMD, LGMD, FSHD and CTRL, completed measures for QoL, Knee-Extension Maximal Voluntary Contraction (KEMVC), Fatigue, Pain, Self-Efficacy and Activities of Daily Living (ADL).

Frequency of reported pain in adult males with muscular dystrophy.

Introduction: The purpose of this study was to present and compare pain between adult males with Duchenne (DMD), Becker's (BMD), Limb-Girdle (LGMD) Facioscapulohumeral (FSHD) forms of Muscular Dystrophy (MD), and healthy controls (CTRL), using three different methods of assessment.

Methods: Pain was assessed using 1) a whole body visual analogue scale (VAS) of pain, 2) a generalised body map and 3) a localised body map.

Results: All types of MD reported more VAS pain than CTRL, with 97% of all MD participants reporting pain; however, no differences were reported between types of MD.

Impaired Glucose Tolerance in Adults with Duchenne and Becker Muscular Dystrophy.

The aim of this study was to determine the response to an oral glucose tolerance test (OGTT) in adult males with Becker muscular dystrophy (BMD) and Duchenne muscular dystrophy (DMD), and to investigate whether body composition contributes to any variance in the glucose response. Twenty-eight adult males with dystrophinopathy (BMD, = 13; DMD, = 15) and 12 non-dystrophic controls, ingested 75 g oral anhydrous glucose solution. Fingertip capillary samples were assessed for glucose at 30-min intervals over 2-h post glucose ingestion.

Relationships between muscle size, strength, and physical activity in adults with muscular dystrophy.

Background: Muscular dystrophy (MD) is characterized by progressive muscle wasting and weakness, yet few comparisons to non-MD controls (CTRL) of muscle strength and size in this adult population exist. Physical activity (PA) is promoted to maintain health and muscle strength within MD; however, PA reporting in adults with MD is limited to recall data, and its impact on muscle strength is seldom explored.

Methods: This study included 76 participants: 16 non-MD (CTRL, mean age 35.

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 (FEV ) and forced vital capacity (FVC)], body fat, and biceps thickness assessments. During the A6MCT, ventilation (VE), oxygen uptake (VO ), carbon dioxide (VCO ), and heart rate (HR) were recorded.

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.

Gastrocnemius medialis muscle architecture and physiological cross sectional area in adult males with Duchenne muscular dystrophy.

Objectives: To describe muscle size and architecture of the gastrocnemius medialis (GM) muscle in eleven adult males with Duchenne Muscular Dystrophy (DMD, age 24.5±5.4 years), and a control group of eleven males without DMD (CTRL, age 22.

The spectrum of pathology in central core disease.

Central core disease is a congenital myopathy with muscle weakness defined pathologically by the presence of extensive areas in muscle fibres that are devoid of oxidative enzyme activity. The gene responsible has been shown to be the ryanodine receptor 1 on chromosome 19q13 and mutations have now been identified in several patients. Some cases with the morphological defect remain molecularly undefined, particularly those studied before molecular studies were available.