Discussion The main result of this study is that CMT1A patient

.. Discussion The main result of this study is that CMT1A patients showed

a lower amount and intensity in some daily living activities with respect to the FG-4592 in vitro healthy individuals of the control group. Patients carried out a lower number of both ascending and descending steps and sit to stands, and selected a lower speed of walking and step climbing. Moreover, in CMT1A patients, the number of both ascending and descending steps and sit to stands was correlated with muscle strength. From the analysis of generic physical activity indexes, such as total distance covered and number of steps performed during the whole day, it Inhibitors,research,lifescience,medical has been shown that CMT1A patients did not Inhibitors,research,lifescience,medical differ from healthy individuals of the control group. Moreover, both groups spent a similar

amount of time in resting activities. Although there are no studies in the literature measuring daily living activities in CMT1A patients by means of inertial sensors, our results appear to be in contrast with previous observations of Aitkens and colleagues (Aitkens et al. 2005), based on self-reported levels of physical activity, which were lower in patients with various neuromuscular diseases (CMT, myotonic dystrophy, limb-girdle Inhibitors,research,lifescience,medical syndrome) compared to healthy individuals. This discrepancy could be ascribed not only to the inaccuracy of daily activity logs with respect to inertial sensors, but also to the heterogeneity of the patients’ group, which may have included individuals with higher Inhibitors,research,lifescience,medical impairment in physical performances than our patients. Even if CMT1A patients covered the same distance and performed the same number of steps as healthy controls, they carried out a lower number of both ascending and descending steps and performed a lower number of sit-to-stand and stand-to-sit transitions, which is one of the most innovative results of this study due to the inertial sensors’ feature of discriminating specific changes in posture and body motions. It can be speculated that CMT1A patients avoid most Inhibitors,research,lifescience,medical demanding tasks requiring

high-intensity contractions of the lower limbs muscles, both eccentric and concentric, however as a consequence of their functional limits. Moreover, the low number of sit to stands means that CMT1A patients have a more sedentary lifestyle that could be one of the reasons to explain the decline in aerobic capacity reported in literature in patients with neuromuscular diseases (Wright et al. 1996; Fowler 2002; Kilmer 2002; El Mhandi et al. 2008). With regard to physical exercise intensity, mean speed of walking and step climbing during the 24-h sessions was significantly lower in CMT1A patients with respect to healthy controls, which is in line with the results of other researchers who measured speed of walking in a laboratory environment (Kalkman et al. 2005; El Mhandi et al. 2008; Menotti et al. 2011).

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