Abstract
BackgroundSarcopenic obesity has been observed in people with neuromuscular impairment, and is linked to adverse health outcomes. It is unclear, however, if sarcopenic obesity develops in adults with facioscapulohumeral muscular dystrophy (FSHD).MethodsThe purpose of this study was to determine if adults with FSHD meet criteria for sarcopenic obesity (appendicular lean mass index (ALMI) scores of < 7.26 or 5.45 kg/m2; % fat mass (FM) ≥ 28 or 40% in men/women). Ten people with FSHD (50 ± 11 years, 2 females) and ten age/sex-matched controls (47 ± 13 years, 2 females) completed one visit, which included a full-body dual-energy x-ray absorptiometry (DXA) scan. Regional and whole body total mass, fat mass (FM), and lean mass (LM) were collected and body mass index (BMI) and sarcopenia measures were computed.ResultsPeople with FSHD and controls had a similar whole body total mass (84.5 ± 12.9 vs. 81.8 ± 13.5 kg, respectively, p = 0.65). Though BMI was 2% lower in the FSHD group (p = 0.77), the % FM was 46% higher in FSHD, compared with controls (p < 0.01). In addition, ALM volume was 23% lower (p = 0.02) and ALMI was 27% lower in FSHD compared with controls (p < 0.01). Whole body LM trended to be lower in FSHD vs. controls (p = 0.05), and arm and leg LM were both lower in FSHD compared with controls (p < 0.05). Furthermore, the % LM was 18% lower in FSHD vs. controls (p < 0.01). FSHD participants exhibited greater total body FM (p < 0.01) and total leg FM (p < 0.01), but were similar in volume of total arm FM compared with controls (p = 0.09).ConclusionFindings from this study suggest that people with FSHD, although similar in BMI and total body mass compared with controls, commonly meet the definition of sarcopenic obesity. Adults with co-existing FSHD and sarcopenic obesity may be at risk for significant impairments in quality of life, and encounter additional challenges in the management of FSHD manifestations.
Highlights
Facioscapulohumeral muscular dystrophy (FSHD) is one of the most common dominantly-inherited muscular dystrophies, with prevalence frequencies ranging from 1:15,000 to 1:21,000 worldwide (Deenen et al, 2014)
FSHD and control participants were similar in age, weight, height, and Body mass index (BMI) (p > 0.05 for all, Table 1)
In the FSHD group, all 10 participants self-reported as non-Hispanic (NH) White; among controls, self-reported race was as follows: NH White: 7 (5 men, 2 women), Black: 1, Asian: 1, Hispanic: 1
Summary
Facioscapulohumeral muscular dystrophy (FSHD) is one of the most common dominantly-inherited muscular dystrophies, with prevalence frequencies ranging from 1:15,000 to 1:21,000 worldwide (Deenen et al, 2014). It is speculated that this functional impairment may be partially driven by alterations in body composition, which likewise have been linked to high rates of skeletal muscle atrophy (Statland and Tawil, 2016; Wang and Tawil, 2016) and corresponding increases in fatty tissue infiltration of the muscular compartments (Janssen et al, 2014). Janssen et al (2014) demonstrated that up to 26% of individuals with FSHD may experience severe rates of fatty infiltration, whereby as much as 75% of lean tissue in certain muscular compartments is replaced by fat mass, an observation that may have clinical and functional implications for people with FSHD. Sarcopenic obesity has been observed in people with neuromuscular impairment, and is linked to adverse health outcomes It is unclear, if sarcopenic obesity develops in adults with facioscapulohumeral muscular dystrophy (FSHD)
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