Abstract
BackgroundCharcot–Marie–Tooth 1A (CMT1A) patients show a reduction of spontaneous activities of daily living measured by means of questionnaires or pedometers, which are quite inaccurate compared to recent measurement techniques.AimThe study aimed at quantifying daily living activities in CMT1A patients by means of inertial sensors, which give information not only on the amount but also on the intensity of these activities.Materials and methodsTime and count (amount), and velocity and power (intensity) of 24 h daily living activities were measured in eight patients (20–48 years; Barthel >90; Tinetti >20) and eight healthy individuals, matched for age and gender, by means of a wearable inertial sensor device.ResultsThere were no differences between patients and controls in the 24-h distance covered and count of steps. However, count of step climbing and sit to stand were lower in patients than in controls (139.93 ± 141.66 vs. 341.06 ± 164.07 n and 58.23 ± 7.82 vs. 65.81 ± 4.75 n, respectively; P < 0.05) as well as mean daily step-climbing and walking velocities (1.07 ± 0.17 vs. 1.21 ± 0.10 m/sec and 1.16 ± 0.31 vs. 1.87 ± 0.50 m/sec, respectively; P < 0.05). In CMT1A patients there was a positive correlation between strength of the knee extensor muscles and both count of steps climbed (R = 0.80) and sit to stand (R = 0.79).Discussion and conclusionThe reduced ability of CMT1A patients to carry out activities at high intensity, which was correlated with strength, suggests that strength training might be a rehabilitation tool for improving the 1 ability to carry out these activities.
Highlights
Charcot–Marie–Tooth 1A disease (CMT1A), referred to as hereditary motor and sensory neuropathy (HMSN1A), is a genetic and progressive neuropathy affecting the neuromuscular system (Casasnovas et al 2008; Banchs et al 2009)
Charcot–Marie–Tooth 1A (CMT1A) patients show a reduction of spontaneous activities of daily living measured by means of questionnaires or pedometers, which are quite inaccurate compared to recent measurement techniques
Walking distance did not differ between groups (4573 Æ 2949 m for CMT1A patients and 4759 Æ 1259 m for healthy controls)
Summary
Charcot–Marie–Tooth 1A disease (CMT1A), referred to as hereditary motor and sensory neuropathy (HMSN1A), is a genetic and progressive neuropathy affecting the neuromuscular system (Casasnovas et al 2008; Banchs et al 2009). Previous studies reported CMT1A patients with several functional limitations: muscle weakness or atrophy in both upper and lower limbs (Lindeman et al 1999; Menotti et al 2012), high level of experienced fatigue and impaired recovery from fatigue after exhausting motor tasks (Schillings et al 2007; Zwarts et al 2008; Menotti et al 2012), modification of walking patterns (Don et al 2007), high energy cost of walking (Menotti et al 2011, 2013), and low aerobic capacity and cardiovascular fitness (Wright et al 1996; Fowler 2002; Kilmer 2002; El Mhandi et al 2008) Most of these functional limitations are the result of the progression of the neuropathy itself, but can be exacerbated by a sedentary lifestyle. Discussion and conclusion: The reduced ability of CMT1A patients to carry out activities at high intensity, which was correlated with strength, suggests that strength training might be a rehabilitation tool for improving the 1 ability to carry out these activities
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