title: Amount and intensity of daily living activities in Charcot-Marie-Tooth 1A patients.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  The 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.
  
  
  METHODS
  Time 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.
  
  
  RESULTS
  There 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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Human Movement, Social and Health Sciences, University of Rome Foro Italico Rome, Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Menotti
authors.affiliation: Department of Human Movement, Social and Health Sciences, University of Rome Foro Italico Rome, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Laudani
authors.affiliation: Sezione Laziale, Unione Italiana Lotta alla Distrofia Muscolare (UILDM) Rome, Italy.
authors.forename: Antonello
authors.initials: A
authors.lastname: Damiani
authors.affiliation: Department of Human Movement, Social and Health Sciences, University of Rome Foro Italico Rome, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Macaluso
mesh_terms: D056044:Actigraphy; D000203:Activities of Daily Living; D000328:Adult; D002607:Charcot-Marie-Tooth Disease / Q000503:physiopathology*; D004734:Energy Metabolism / Q000502:physiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018670:Monitoring, Ambulatory; D009043:Motor Activity / Q000502:physiology*; D053580:Muscle Strength / Q000502:physiology; D055815:Young Adult
keywords: Energy expenditure; hereditary neuromuscular disorder; neuropathies; rehabilitation; speed of walking