The relationship between regional body composition and quantitative strength in facioscapulohumeral muscular dystrophy (FSHD)

Regional body composition and functional impairment in patients with myotonic dystrophy

“…Such results have also been reported in other NMDs, such as dystrophinopathies, facioscapulohumeral dystrophy, spinal muscular atrophy, and amyotrophic lateral sclerosis. 8,10,[14][15][16][17][18] Percent fat mass increased with increasing age in DM1 patients, as opposed to no significant change in matched controls. This could reflect the evolution of muscular involvement in DM1, with a progressive loss of muscle with fat infiltration.…”

“…Dual-energy X-ray absorptiometry (DEXA) is a noninvasive body composition technique that provides regional estimations of fat-free mass (FFM) and fat mass (FM). 8 It has been used successfully in the assessment of whole body and regional body composition in subjects with neuromuscular diseases. 7,9,10 However, such studies have not been done in DM1 patients.…”

188th ENMC International Workshop: Inclusion Body Myositis, 2–4 December 2011, Naarden, The Netherlands

“…DEXA determination of regional limb muscle mass, eliminating some of noise from measurement of the trunk, will likely give more reliable measures of muscle mass. There is good correlation between DEXA whole body and DEXA limb values with muscle strength demonstrated for facioscapulohumeral muscular dystrophy (FSHD) and Duchenne Muscular Dystrophy (DMD) [51,52]. In normal subjects increases in lean body mass of less than 5% correlated significantly with changes in strength.…”

Reproducibility of Regional DEXA Examinations of Abdominal Fat and Lean Tissue

“…This technique is frequently used in obesity-related research [14,15,16,17] and when evaluating the effect of surgery for obesity [17,18]. Recent DEXA computer software can automatically calculate abdominal fat in regions according to bony anatomical landmarks [1,13,19,20]. Regions on DEXA images may be set also with great accuracy manually [21].…”