A study of physical activity comparing people with Charcot-Marie-Tooth disease to normal control subjects

Abstract

Purpose Charcot Marie Tooth disease (CMT) describes a group of hereditary neuropathies that present with distal weakness, wasting and sensory loss. Small studies indicate that people with CMT have reduced daily activity levels. This raises concerns as physical inactivity increases the risk of a range of co- morbidities, an important consideration in the long-term management of this disease. This study aimed to compare physical activity, patterns of sedentary behavior and overall energy expenditure of people with CMT and healthy matched controls. Methods We compared 20 people with CMT and 20 matched controls in a comparison of physical activity measurement over seven days, using an activity monitor. Patterns of sedentary behavior were explored through a power law analysis. Results Results showed a decrease in daily steps taken in the CMT group, but somewhat paradoxically, they demonstrate shorter bouts of sedentary activity and more frequent transitions from sedentary to active behaviors. No differences were seen in energy expenditure or time spent in sedentary, moderate or vigorous activity. Conclusion The discrepancy between energy expenditure and number of steps could be due to higher energy requirements for walking, but also may be due to an over-estimation of energy expenditure by the activity monitor in the presence of muscle wasting. Alternatively, this finding may indicate that people with CMT engage more in activities or movement not related to walking.

Physical activity in children and adolescents with Charcot-Marie-Tooth disease: A cross-sectional case-controlled study

“…The children with CMT who were less active had greater disease severity and reported greater limitations to their gait-related activities, similar to the findings of a small study of children with mixed neuromuscular diagnoses (34). Prior research suggests this may be a lifelong concern, as adults with CMT also report limitations to physical activity (8,9). The current study expands on the studies in adults to establish that physical activity is limited from childhood in CMT, similar to other paediatric disorders that present with physical impairments such as cerebral palsy, muscular dystrophy and spina bifida (7).…”

“…The natural history of CMT is of progressive weakness and worsening of disease-related disability. Adults with CMT are less physically active than unaffected adults (8).…”

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

“…Few other studies have explored physical activity among people with disabilities due to NMDs [40][41][42]. In one of the studies, Saebu and Sørensen [40] obtained self-reported physical activity levels using the same instrument (IPAQ-sf) in the people with various disability conditions.…”

“…However, in the study by Saebu and Sørensen [40], only 14.7% of the participants were having disabilities due to muscle problem without further description about specific diagnoses. Other studies in people with CMT by Ramdharry et al [41], and Anens et al [42] used different instruments to collect information about physical activity. Ramdharry used objective measurement [41] and Anens used Physical Activity Disability Survey-Revised (PADS-R) questionnaire [42].…”

“…Other studies in people with CMT by Ramdharry et al [41], and Anens et al [42] used different instruments to collect information about physical activity. Ramdharry used objective measurement [41] and Anens used Physical Activity Disability Survey-Revised (PADS-R) questionnaire [42]. In Anens et al [42] study, there was no description about physical activity level in METs.…”