Article

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.
Implications for Rehabilitation Charcot-Marie-Tooth disease: * People with
Charcot-Marie-Tooth disease did not show a difference in energy expenditure over
seven days compared to healthy controls, but this may be due to higher energy
costs of walking, and/or an over estimation of energy expenditure by the activity
monitor in a population where there is muscle wasting. This needs to be
considered when interpreting activity monitor data in people with neuromuscular
diseases. * Compared to healthy controls, people with Charcot-Marie-Tooth disease
had a lower step count over seven days, but exhibited more frequent transitions
from sedentary to active behaviors * High Body Mass Index and increased time
spent sedentary were related factors that have implications for general health
status. * Understanding the profile of physical activity and behavior can allow
targeting of rehabilitation interventions to address mobility and fitness.

Langue : ANGLAIS