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

OBJECTIVE To describe the temporal, kinetic, kinematic, electromyographic and energetic aspects of gait in Charcot-Marie-Tooth patients with foot drop and plantar flexion failure. METHODS 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). RESULTS 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. CONCLUSIONS 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.

[1]  F. Pierelli,et al.  Quality of life in patients with Charcot–Marie–Tooth disease , 2005, Neurology.

[2]  R. Hughes,et al.  Epidemiology of peripheral neuropathy. , 1997, Journal of neurology, neurosurgery, and psychiatry.

[3]  J. Vance,et al.  Hereditary motor and sensory neuropathies. , 1991, Journal of medical genetics.

[4]  J. Perry,et al.  Gait Analysis , 2024 .

[5]  H. Hermens,et al.  European recommendations for surface electromyography: Results of the SENIAM Project , 1999 .

[6]  R. Adams,et al.  Principles of Neurology , 1996 .

[7]  D. Lyttle,et al.  Pathogenesis of Charcot-Marie-Tooth disease. Gait analysis and electrophysiologic, genetic, histopathologic, and enzyme studies in a kinship. , 1984, Clinical orthopaedics and related research.

[8]  T. Hortobágyi,et al.  Functional Knee Brace Effects During Walking in Patients With Anterior Cruciate Ligament Reconstruction , 1998, The American journal of sports medicine.

[9]  H Labelle,et al.  Functional roles of ankle and hip sagittal muscle moments in able-bodied gait. , 2001, Clinical biomechanics.

[10]  Carlos A. Garcia A Clinical Review of Charcot‐Marie‐Tooth , 1999, Annals of the New York Academy of Sciences.

[11]  Christopher L. Vaughan,et al.  Dynamics of human gait , 1992 .

[12]  Y. Agid,et al.  Charcot-Marie-Tooth disease type 1A with 17p11.2 duplication. Clinical and electrophysiological phenotype study and factors influencing disease severity in 119 cases. , 1997, Brain : a journal of neurology.

[13]  F. Zajac,et al.  Contributions of the individual ankle plantar flexors to support, forward progression and swing initiation during walking. , 2001, Journal of biomechanics.

[14]  Jun Yu Li,et al.  Reliability and validity of the CMT neuropathy score as a measure of disability , 2005, Neurology.

[15]  G. Cavagna,et al.  The sources of external work in level walking and running. , 1976, The Journal of physiology.

[16]  P. Vinci,et al.  Footdrop, foot rotation, and plantarflexor failure in Charcot-Marie-Tooth disease. , 2002, Archives of physical medicine and rehabilitation.

[17]  D. Kerrigan,et al.  The vertical displacement of the center of mass during walking: a comparison of four measurement methods. , 1998, Journal of biomechanical engineering.

[18]  G. Cavagna,et al.  Pendular energy transduction within the step in human walking. , 2002, The Journal of experimental biology.

[19]  F. Pierelli,et al.  Lower limb manual muscle testing in the early stages of Charcot-Marie-Tooth disease type 1A. , 2006, Functional neurology.

[20]  J. Ashton-Miller,et al.  Falls and Gait Characteristics Among Older Persons with Peripheral Neuropathy , 2007, American journal of physical medicine & rehabilitation.

[21]  H. Weiner,et al.  Intensive immunosuppression in progressive multiple sclerosis. A randomized, three-arm study of high-dose intravenous cyclophosphamide, plasma exchange, and ACTH. , 1983, The New England journal of medicine.

[22]  Vladimir M. Zatsiorsky,et al.  The Mass and Inertia Characteristics of the Main Segments of the Human Body , 1983 .

[23]  F. Baas,et al.  Comparison of CMT1A and CMT2: similarities and differences , 2006, Journal of Neurology.

[24]  Giancarlo Ferrigno,et al.  Elite: A Digital Dedicated Hardware System for Movement Analysis Via Real-Time TV Signal Processing , 1985, IEEE Transactions on Biomedical Engineering.

[25]  R. B. Davis,et al.  A gait analysis data collection and reduction technique , 1991 .

[26]  Dario Farina,et al.  A fast and reliable technique for muscle activity detection from surface EMG signals , 2003, IEEE Transactions on Biomedical Engineering.

[27]  N. Cartlidge Technique of the neurological examination By W. DeMyer, 448 pages. McGraw-Hill, New York, N.Y., 1974, £8.00 , 1975, Journal of the Neurological Sciences.

[28]  Rory O'Sullivan,et al.  The characteristics of gait in Charcot-Marie-Tooth disease types I and II. , 2007, Gait & posture.

[29]  O. Combarros,et al.  Charcot-Marie-Tooth disease type 1A with 17p duplication in infancy and early childhood , 1998, Neurology.

[30]  C. Detrembleur,et al.  Energy cost, mechanical work, and efficiency of hemiparetic walking. , 2003, Gait & posture.

[31]  P K Thomas,et al.  The clinical features of hereditary motor and sensory neuropathy types I and II. , 1980, Brain : a journal of neurology.