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Updated: Feb 11, 2026
Modeling Charcot-Marie-Tooth Disease In Vitro by Transfecting Mouse Primary Motoneurons
Published on: January 7, 2019
Electromyographic and biomechanical analysis of step negotiation in Charcot Marie Tooth subjects whose level walk is
Tiziana Lencioni1, Giuseppe Piscosquito2, Marco Rabuffetti1
1Fondazione Don Carlo Gnocchi, Milan, Italy.
Background:
Charcot-Marie-Tooth (CMT) is a slowly progressive disease characterized by muscular weakness and wasting with a length-dependent pattern. Mildly affected CMT subjects showed slight alteration of walking compared to healthy subjects (HS).
Research Question:
To investigate the biomechanics of step negotiation, a task that requires greater muscle strength and balance control compared to level walking, in CMT subjects without primary locomotor deficits (foot drop and push off deficit) during walking.
Methods:
We collected data (kinematic, kinetic, and surface electromyographic) during walking on level ground and step negotiation, from 98 CMT subjects with mild-to-moderate impairment. Twenty-one CMT subjects (CMT-NLW, normal-like-walkers) were selected for analysis, as they showed values of normalized ROM during swing and produced work at push-off at ankle joint comparable to those of 31 HS. Step negotiation tasks consisted in climbing and descending a two-step stair. Only the first step provided the ground reaction force data. To assess muscle activity, each EMG profile was integrated over 100% of task duration and the activation percentage was computed in four phases that constitute the step negotiation tasks.
Results:
In both tasks, CMT-NLW showed distal muscle hypoactivation. In addition, during step-ascending CMT-NLW subjects had relevant lower activities of vastus medialis and rectus femoris than HS in weight-acceptance, and, on the opposite, a greater activation as compared to HS in forward-continuance. During step-descending, CMT-NLW showed a reduced activity of tibialis anterior during controlled-lowering phase.
Significance:
Step negotiation revealed adaptive motor strategies related to muscle weakness due to disease in CMT subjects without any clinically apparent locomotor deficit during level walking. In addition, this study provided results useful for tailored rehabilitation of CMT patients.
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