Foot drop and plantar flexion failure determine different gait strategies in Charcot-Marie-Tooth patients.
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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ABSTRACT: Custom carbon-fiber composite ankle foot orthoses (AFOs) have been anecdotally reported to improve gait of Charcot-Marie-Tooth (CMT) patients. The purpose of the study was to characterize the spatio-temporal, joint kinetic and mechanical responses of a custom carbon fiber AFO during locomotion for persons diagnosed with CMT. Eight volunteers were fitted with custom AFOs. Three of the devices were instrumented with eight strain gauges to measure surface deformation of the shell during dynamic function. Following a minimum 10 weeks accommodation period, plantar- and dorsiflexor strength was measured bilaterally. Volunteers then walked unbraced and braced, at their preferred pace over a force platform and instrumented walkway while being tracked with a 12-camera motion capture system. Strength, spatio-temporal and lower extremity joint kinetic parameters were evaluated between conditions (single subject) using the model statistic procedure. Mechanical loads were presented descriptively. All participants walked faster (89.4±13.3 vs 115.6±18.0cm/s) in the braced condition with ankle strength negatively correlated to speed increase. As Δvelocity increased, maximum joint moments during loading response shifted from the hip joint to the ankle and knee joints. During propulsion, the hip joint moment dominated. Subjects exhibiting the greatest and least Δvelocity imposed an average load of 54.6% and 16.6% of body weight on the braces, respectively. Energy storage in the brace averaged 9.6±6.6J/kg. Subject-specific effects of a custom AFO on gait for CMT patients were documented. The force-deflection properties of carbon-fiber composite braces may be important considerations in their design.Gait & posture 08/2013; · 2.58 Impact Factor
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ABSTRACT: Background:Ankle-foot orthoses are commonly prescribed in Charcot-Marie-Tooth type 1A disease to improve quality of walking and reduce the risk of falling due to the foot drop.Objectives:This study aimed at assessing the effect of an anterior ankle-foot orthosis on walking economy in a group of Charcot-Marie-Tooth type 1A patients.Study design:Within-group comparisons.Methods:7 Charcot-Marie-Tooth type 1A patients (four women and three men; 37 ± 11 years; age range = 22-53 years) were asked to walk on a circuit at their self-selected speeds ('slow', 'comfortable' and 'fast') in two walking conditions: (1) with shoes only and (2) with Taloelast(®) anterior elastic ankle-foot orthoses. Speed of walking and metabolic cost of walking energy cost per unit of distance were assessed at the three self-selected speeds of walking for both walking conditions.Results:Speed of walking at the three self-selected speeds did not differ between shoes only and anterior elastic ankle-foot orthoses, whereas walking energy cost per unit of distance at comfortable speed was lower in patients using anterior elastic ankle-foot orthoses with respect to shoes only (2.39 ± 0.22 vs 2.70 ± 0.19 J kg(-1) m(-1); P < 0.05).Conclusions:In Charcot-Marie-Tooth type 1A patients, the use of anterior elastic ankle-foot orthoses improved walking economy by reducing the energy cost of walking per unit of distance, thus reflecting a lower level of metabolic effort and improved mechanical efficiency in comparison with shoes only.Clinical relevanceFrom a practical perspective, Charcot-Marie-Tooth type 1A patients with anterior elastic ankle-foot orthoses can walk for a longer duration with a lower level of physical effort. Improvements in walking economy due to ankle-foot orthoses are likely a consequence of the reduction in steppage gait.Prosthetics & Orthotics International 10/2013; · 0.56 Impact Factor
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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.