Self-reported physical activity in people with limb-girdle muscular dystrophy and Charcot-Marie-Tooth disease in Norway

Abstract

Background: Physical activity is associated with positive health effects, but individuals with neuromuscular disease (NMD) may experience constraints being physically active. There is a gap in the literature on the activity level of people with NMDs, and therefore we did a study to determine the physical activity level in people with Limb-Girdle muscular dystrophy (LGMD) and Charcot-Marie-Tooth disease (CMT). Methods: This study used a cross-sectional design to obtain self-reported physical activity and sitting time among individuals with LGMD and CMT who were recruited from the Norwegian registry for hereditary and congenital neuromuscular diseases. Results: A total of 127 respondents who filled out questionnaires about either physical activity or sitting time were included in the analysis. Seventy (55.1%) had a diagnosis of CMT and 57 (44.9%) had a diagnosis of LGMD. Seventythree (57.5%) respondents were female and 54 (42.5%) were male. Among the 108 respondents with available physical activity data, 44.4% reported being physically inactive. Among the 109 respondents with available sitting time data, the average sitting time was 8.6 h. Longer sitting time was associated with higher physical inactivity. Conclusion: Among people with LGMD and CMT in our study, 55.6% reported being physically active. Respondents with LGMD and CMT reported longer sitting time and less physical activity compared with healthy respondents in other studies. Further research should explore variables and measures that can promote physical activity among people with neuromuscular conditions.

Quantitative assessment of sitting time in ambulant adults with Muscular Dystrophy

“…To the authors knowledge this is the first work to objectively assess sitting time in ambulatory adults with MD, as such comparisons are difficult to make. The 9.9 hours reported here is greater than the 9.1 hour self-reported in adults with Limb-Girdle MD alone [21], however that study did not include any objective analysis of sitting time, nor information regarding the functional status of participants.…”

“…The inclusion of both ambulatory and non-ambulatory adults with MD may be problematic in the assessment of SB, as SB time is known to be 20% lower in non-ambulatory compared to ambulatory boys with Duchenne MD (SB time = 91% in Non-Ambulant, and 71% in Ambulant) [19]. In addition to SB determined through METs, advances in accelerometry have now allowed posture classification, time spent Sitting or Standing in waking hours, to be identified [20], which has only previously been reported (9.1 Hours) using self-report methods in ambulant adults with MD [21]. Self-report methods however have been demonstrated to underestimate negative health behaviours such as SB and sitting time by up to 40%, when compared to objective assessments using accelerometers [22].…”

Sarcopenic Obesity in Facioscapulohumeral Muscular Dystrophy

“…In addition, physical inactivity has been widely cited as a contributor in the development of obesity ( Myers et al, 2017 ). Observations of physical inactivity have been noted in other forms of muscular dystrophy, whereby 44% of people with limb-girdle or Charcot-Marie-Tooth muscular dystrophy exhibit an inability to meet minimum threshold recommendations for daily exercise ( Andries et al, 2020 ). Thus, the physical inactivity demonstrated in our FSHD cohort likely not only contributed to sarcopenia, but also obesity.…”

Low Bone Mass in Ambulatory Spinal Muscular Atrophy: A Proactive Approach for an Often-Overlooked Impairment

“…MET levels are weighted based on intensity of activity. IPAQ-SF was validated to evaluate physical activity in individuals 15–69 years old [ 26 , 27 ] and in several neuromuscular populations, including Charcot–Marie–Tooth disease and limb–girdle muscular dystrophy [ 28 ].…”