title: Postural stabilization and balance assessment in Charcot-Marie-Tooth 1A subjects.
abstract: The aim of the present study was to assess postural stabilization skill in adult subjects affected by Charcot-Marie-Tooth disease (CMT) type 1A. For this purpose ground reaction force (GRF) was measured by means of a piezoelectric force platform during the sit-to-stand (STS) movement, until a steady state erect posture was achieved. Specific indexes to quantify Centre of Mass acceleration, both during postural stabilization and during quiet standing, were computed using a mathematical model. Forty-seven CMT1A subjects were recruited for the study, and the control group was formed by forty-one age- and sex-matched healthy subjects. The results show that CMT1A subjects are less stable than controls during the quiet stance. Greater difficulty (high values of Yinf, the final instability rate) to maintain erect posture appears to be mainly associated with plantar-flexor muscle weakness, rather than to damage of the proprioceptive system. The worst performances shown by CMT1A subjects in the stabilization phase (high values of I, the global index of postural stabilization performance) seem to be associated with reduced muscle strength and the loss of large sensory nerve fibres. Distal muscle weakness appears to affect both postural stabilization and quiet erect posture. The presented protocol and the analysis of postural stabilization parameters provide useful information on CMT1A balance disorders.
authors.affiliation: Biomedical Technology Department, IRCCS Foundation Don Carlo Gnocchi Onlus, Milano, Italy. Electronic address: tlencioni@dongnocchi.it.
authors.forename: T
authors.initials: T
authors.lastname: Lencioni
authors.affiliation: Biomedical Technology Department, IRCCS Foundation Don Carlo Gnocchi Onlus, Milano, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Rabuffetti
authors.affiliation: Unit of Clinic of Central and Peripheral Degenerative Neuropathies, IRCCS Foundation, C. Besta Neurological Institute, Milano, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Piscosquito
authors.affiliation: Unit of Clinic of Central and Peripheral Degenerative Neuropathies, IRCCS Foundation, C. Besta Neurological Institute, Milano, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Pareyson
authors.affiliation: Department of Neuroscience, Ophthalmology and Genetics, University of Genoa, Genova, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Aiello
authors.affiliation: Centro S. Maria della Pace, Foundation Don Gnocchi Onlus, Roma, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Di Sipio
authors.affiliation: Centro S. Maria della Pace, Foundation Don Gnocchi Onlus, Roma, Italy.
authors.forename: L
authors.initials: L
authors.lastname: Padua
authors.affiliation: Polo Riabilitativo del Levante Ligure, Foundation Don Gnocchi Onlus, Sarzana, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Stra
authors.affiliation: Biomedical Technology Department, IRCCS Foundation Don Carlo Gnocchi Onlus, Milano, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Ferrarin
mesh_terms: D000328:Adult; D002607:Charcot-Marie-Tooth Disease / Q000503:physiopathology* / Q000534:rehabilitation; D005260:Female; D006801:Humans; D035002:Lower Extremity / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D009068:Movement / Q000502:physiology; D018908:Muscle Weakness / Q000503:physiopathology* / Q000534:rehabilitation; D026741:Physical Therapy Modalities; D004856:Postural Balance / Q000502:physiology*
keywords: Balance; Charcot–Marie–Tooth; Muscle strength; Plantar flexion; Posture
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