title: Alterations of thoraco-abdominal volumes and asynchronies in patients with spinal muscle atrophy type III.
abstract: Spinal muscular atrophy (SMA) is characterized by degeneration of motor neurons resulting in muscle weakness. For the mild type III form, a sub-classification into type IIIA and IIIB, based on age of motor impairment, was recently proposed. To investigate if SMA IIIA (more severe) and IIIB differ also in terms of respiratory function, thoracoabdominal kinematics was measured during quiet breathing, inspiration preceding cough and inspiratory capacity on 5 type IIIA and 9 type IIIB patients. Four patients with SMA II (more severe than types III) and 19 healthy controls were also studied. Rib cage motion was similar in SMA IIIB and controls. Conversely, in SMA IIIA and SMA II it was significantly reduced and sometime paradoxical during quiet breathing in supine position. Our results suggest that in SMA IIIA intercostal muscles are weakened and the diaphragm is preserved similarly to SMA II, while in SMA IIIB the action of all inspiratory muscles is maintained. Sub-classification of type III seems feasible also for respiratory function.
authors.affiliation: TBM Lab, Dipartimento di Elettronica, Informazione e Bioingegneria, Politecnico di Milano, Milano, Italy. Electronic address: antonella.lomauro@polimi.it.
authors.forename: Antonella
authors.initials: A
authors.lastname: LoMauro
authors.affiliation: IRCCS E. Medea, Bosisio Parini, Lc, Italy.
authors.forename: Marianna
authors.initials: M
authors.lastname: Romei
authors.affiliation: TBM Lab, Dipartimento di Elettronica, Informazione e Bioingegneria, Politecnico di Milano, Milano, Italy.
authors.forename: Rita
authors.initials: R
authors.lastname: Priori
authors.affiliation: TBM Lab, Dipartimento di Elettronica, Informazione e Bioingegneria, Politecnico di Milano, Milano, Italy.
authors.forename: Marianna
authors.initials: M
authors.lastname: Laviola
authors.affiliation: IRCCS E. Medea, Bosisio Parini, Lc, Italy.
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: D'Angelo
authors.affiliation: TBM Lab, Dipartimento di Elettronica, Informazione e Bioingegneria, Politecnico di Milano, Milano, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Aliverti
mesh_terms: D034861:Abdominal Wall / Q000503:physiopathology*; D000293:Adolescent; D000328:Adult; D001696:Biomechanical Phenomena; D002648:Child; D002675:Child, Preschool; D003371:Cough / Q000503:physiopathology; D005260:Female; D006801:Humans; D001239:Inhalation / Q000502:physiology; D008176:Lung Volume Measurements; D008297:Male; D009068:Movement; D010991:Plethysmography; D012119:Respiration; D012129:Respiratory Function Tests; D012132:Respiratory Muscles / Q000503:physiopathology*; D014897:Spinal Muscular Atrophies of Childhood / Q000145:classification / Q000503:physiopathology*; D013147:Spirometry; D016683:Supine Position / Q000502:physiology; D035441:Thoracic Wall / Q000503:physiopathology*; D055815:Young Adult
keywords: Abdomen; Breathing pattern; Chest wall; Opto-electronic plethysmography; Respiratory muscles; Rib cage; Spinal muscle atrophy; Spirometry