Article

Foot drop and plantar flexion failure determine different gait strategies in Charcot-Marie-Tooth patients

Department of Physical Medicine and Rehabilitation, Movement Analysis Laboratory, La Sapienza University of Rome, Italy.
Clinical Biomechanics (Impact Factor: 1.88). 11/2007; 22(8):905-16. DOI: 10.1016/j.clinbiomech.2007.06.002
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ABSTRACT To describe the temporal, kinetic, kinematic, electromyographic and energetic aspects of gait in Charcot-Marie-Tooth patients with foot drop and plantar flexion failure.
A sample of 21 patients fulfilling clinical, electrodiagnostic and genetic criteria for Charcot-Marie-Tooth disease were evaluated by computerized gait analysis system and compared to a group of matched healthy subjects. Patients were classified as having isolate foot drop (group 1) and association of foot drop and plantar flexion failure (group 2).
While it was impossible to detect a reliable gait pattern when the group of patients was considered as a whole and compared to healthy subjects, we observed two distinctive gait patterns when patients were subdivided as group 1 or 2. Group 1 showed a gait pattern with some characteristics of the "steppage pattern". The complex motor strategy adopted by this group leads to reduce the swing velocity and to preserve the step length in spite of a high energy consumption. Group 2 displayed a "clumsy pattern" characterized by very slow gait with reduced step length, a broader support area and great reduction in the cadence. This group of patients is characterized by a low energy consumption and greater energy recovery, due above all to the primary deficit and the various compensatory mechanisms.
Such between-group differences in gait pattern can be related to both primary motor deficits and secondary compensatory mechanisms. Foot drop and plantar flexion failure affect the overall gait strategy in Charcot-Marie-Tooth patients.

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    • "Therefore, we expected to record a higher daily energy expenditure in CMT1A patients as they covered the same distance and spent the same time in walking activities with respect to the healthy controls. It is likely that this unexpected result can be attributed to the inaccuracy of the IDEEA device in estimating daily energy expenditure as it does not take into account the effects of altered walking patterns in CMT1A patients (Mazzaro et al. 2005; Don et al. 2007; Newman et al. 2007). Charcot–Marie–Tooth 1A patients showed lower isometric strength of the knee extensor muscles with respect to healthy individuals, which is consistent with previous results of other authors (Lindeman et al. 1999; Kalkman et al. 2005). "
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    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. Aim. 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. Materials and 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). 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.
    Brain and Behavior 01/2014; 4(1):14-20. DOI:10.1002/brb3.187
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    • "Recent studies have distinguished between patients having weakness in dorsiflexor muscles versus weakness in both dorsiflexor and plantar flexor muscles and have determined that compensatory gait strategies are different [10]. CMT subjects experiencing only dorsiflexor weakness adopt a steppage gait which preserves step length but involves high metabolic energy cost. "
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    • "They are commonly administered to people with CMT to reduce foot drop and diminish compensatory activity of proximal muscles. Increased use of proximal muscles to compensate for distal impairments has been described in two gait analysis studies (Don et al., 2007; Ramdharry et al., 2009a). A comparison of commercially available AFOs observed both distal and proximal improvements in gait patterns in 14 people with CMT (Ramdharry et al., 2007). "
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