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Postural instability in Charcot-Marie-Tooth 1A disease
Institution:1. Institute of Myology, Pitié-Salpêtrière Hospital, Paris, France;2. Centre de Référence de Pathologie Neuromusculaire Paris-Est, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière Hospital, Paris, France;3. Université Laval, Québec, Canada;4. Centre Interdisciplinaire de Recherche en Réadaptation et Intégration Sociale, Québec, Canada;5. Department of Pathology, Henri Mondor University Hospital, Créteil, France;1. Center for Motion Analysis, Connecticut Children''s Medical Center, 399 Farmington Ave. Farmington, CT, USA;2. Division of Orthopedics, Connecticut Children''s Medical Center, 399 Farmington Ave. Farmington, CT, USA;3. Division of Neurology, Connecticut Children''s Medical Center, 505 Farmington Ave. Farmington, CT, USA;4. Children''s Orthopedic Center, Children''s Hospital Los Angeles, 4650 Sunset Bvld, Los Angeles, CA, USA
Abstract:The aim of this study was to evaluate the influence of somatosensory impairment, distal muscle weakness and foot deformities on the balance in 21 CMT1A patients using a baropodometric platform.Stabilometric analysis by measuring sway area and velocity of a centre of pressure (CoP) both at open and closed eyes were used to assess postural imbalance. Static analysis, by measuring the load and the plantar surface of forefoot, midfoot and hindfoot was used to define the footprint shape and to assess as a whole foot deformities. Stabilometric and static results were compared with those of a control group. In CMT1A patients, stabilometric findings were correlated with static parameters, Achilles’ tendon retraction, distal muscle strength and CMT examination score (CMTES). CMT1A patients compared to controls had lower plantar surface and load on midfoot, and higher load on a forefoot. CMT1A patients had a greater postural instability, since they had a higher CoP velocity, both at open and closed eyes. Moreover, the CoP velocity correlated inversely with the strength of ankle dorsi-flexion muscles and directly with CMTES as whole and with the item “motor symptoms legs”. Postural imbalance was not correlated with sensory impairment and foot deformities as expressed by static analysis and Achilles’ tendon retraction.In this study we demonstrated an altered balance in CMT1A patients during upright standing. The imbalance in our CMT patients seems to be related to the weakness of ankle dorsi-flexor muscles rather than sensory impairment or foot deformities. These results could be due to a mildly affected CMT1A population, evaluated in an early stage of the disease.
Keywords:Charcot-Marie-Tooth  Postural imbalance  Distal muscle weakness
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