Respiratory pattern in an adult population of dystrophic patients

M. G. D'Angelo, M. Romei, A. Lo Mauro, E. Marchi, S. Gandossini, S. Bonato, G. P. Comi, F. Magri, A. C. Turconi, A. Pedotti, N. Bresolin, A. Aliverti

Research output: Contribution to journalArticlepeer-review

Abstract

We studied respiratory function and Chest Wall kinematics in a large population of adult patients affected by slow course muscular dystrophies such as Limb-Girdle Muscular Dystrophy (LGMD, n = 38), Becker Muscular Dystrophy (BMD, n = 20) and Facio-Scapulo Humeral Dystrophy (FSHD, n = 30), through standard spirometry and through the Optoelectronic Plethysmography, to measure the thoraco-abdominal motion during Quiet Breathing and Slow Vital Capacity maneuvers. Within the restrictive pulmonary syndrome characterizing LGMD and FSHD, several different thoraco-abdominal patterns compared to those of healthy subjects were present in the more advanced stages of the disease. These differences were present in the seated position, during the execution of a maximal maneuver such as Slow Vital Capacity. A global respiratory (both inspiratory and expiratory) muscle involvement was more pronounced in the LGMD and FSHD than in the BMD patients, and a significant reduction of abdominal contribution in wheelchair bound patients was observed. In conclusion, OEP technique is able to reveal mild initial modifications in the respiratory muscles in FSHD and LGMD patients, which could be helpful for functional and new therapeutic strategy evaluation.

Original languageEnglish
Pages (from-to)54-61
Number of pages8
JournalJournal of the Neurological Sciences
Volume306
Issue number1-2
DOIs
Publication statusPublished - Jul 15 2011

Keywords

  • Becker Muscular Dystrophy
  • Chest Wall kinematics
  • Facio-Scapulo Humeral Dystrophy
  • Limb-Girdle Muscular dystrophy
  • Muscular dystrophy
  • Pulmonary function
  • Respiratory pattern

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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