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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Available from: Alberto Ranavolo, Sep 17, 2014
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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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    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; 39(1). DOI:10.1016/j.gaitpost.2013.07.121 · 2.30 Impact Factor
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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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    ABSTRACT: Background and Purpose. Ankle foot orthoses (AFOs) are commonly prescribed for people with Charcot-Marie-Tooth (CMT) disease. Scant evidence exists to guide the type and timing of orthotic prescription. This study explores the latter issue by investigating the differences in presentation and gait function of people with CMT disease who wore AFOs for daily mobility (n = 11) and a group who did not (n = 21). The aim was to see if there was a difference in the characteristics in people who regularly wear AFOs. Methods. Primary measures of gait function were a 10-m timed walk (comfortable and maximum speed) and a 6-minute walk test. Means of the variables were compared using independent t-tests. Secondary measures included disease severity, lower limb muscle strength, sensory impairment, walking effort, fatigue severity and perceived walking ability. Results. AFO wearers walked slower with higher effort. They also had greater disease severity, weaker leg muscles and perceived greater walking difficulty. Subjects not wearing AFOs showed significant relationships between gait variables and muscle strength, whereas AFO wearers showed significant relationships between gait variables and perceived walking ability, fatigue severity and effort. Conclusions. People who regularly wore AFOs were more severely affected, had a slower maximum walking speed, higher energy cost of walking and worse perceived walking ability. Walking ability in this group was related to fatigue, perceived exertion during walking and perceived walking ability. Gait function of people not using AFOs was determined by lower limb muscle function. People prescribed AFOs, those who do not wear them and those not prescribed AFOs were similar in presentation, suggesting that people choose to wear orthoses when their condition becomes sufficiently severe. Copyright © 2012 John Wiley & Sons, Ltd.
    Physiotherapy Research International 12/2012; 17(4). DOI:10.1002/pri.531
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