Titre :
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Compensatory mechanisms during gait in response to muscle weakness in spinal muscular atrophy, type III : Actes de colloque - International Congress of Myology, 26 - 30 may 2008 Marseille
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contenu dans :
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Auteurs :
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Krajnik J ;
Olensek O ;
Matjacic Z ;
Klemen A ;
Eymard B ;
Zupan A
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Type de document :
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Article
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Editeur :
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AFM-TELETHON, 2008
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Pages :
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p. 170
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Langues:
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Anglais
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Mots-clés :
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amyotrophie spinale proximale type 3
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analyse de la marche
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colloque
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dynamométrie
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évaluation fonctionnelle
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faiblesse musculaire
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force de réaction du sol
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marche
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trouble de la marche
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Résumé :
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Neuromuscular disorders are heterogeneous group of diseases of motor unit with muscular weakness as the predominant clinical sign. One of the most frequent complaint patients have are difficulties in gait. Spinal muscular atrophy (SMA) types I, II, and III is a hereditary disease that causes mostly symmetric and predominantly proximal weakness (specifically psoas quadriceps, triceps) and wasting of the voluntary muscles. Most patients with SMA III experience slow loss of function over time that is difficult to measure. Clinical data on gait in patients with SMA III are scarce. We asked ourselves whether we can determine characteristic compensatory mechanisms in pathologically disturbed gait in patients with SMA III. For that purpose, we used isokinetic dynamometry and clinical gait analysis (kinematic, kinetic and EMG data) in 7 patients with SMA III and 10 healthy subjects (control group). The results show that patients with SMA III negotiate gait problems by: 1. reducing mechanical output requirements of weakened muscles (lower gait velocity, shorter step length, decrease in swing time); 2. minimizing external moments produced by ground reaction force (GRF) on the knee and hip by: * stronger activity of plantar flexors which controls centre of pressure during loading response and midstance to facilitate minimization of external flexion moment acting on the knee and hip; * anterior rotation of pelvis which facilitates rapid hip extension to secure GRF in front of the knee and behind the hip; * prolonged activity of contralateral hip abductors which decreases weight acceptance. We conclude that the most important muscle groups enabling efficient compensation of diminished strength of knee and hip muscles are ankle plantarflexors, hip rotators and hip abductors, which might have direct application in rehabilitation programs. We also propose clinical gait analysis as a method for functional evaluation and measuring the progression of the disease.
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Voir aussi :
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