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.
Keywords
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References
- A 2009 perspective on the 2004 American Thoracic Society statement, “respiratory care of the patient with Duchenne muscular dystrophy”.Pediatrics. 2009; 123: S239-S241
- Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement.Am J Respir Crit Care Med. 2004; 170: 456-465
- ENMC: 117th ENMC workshop: ventilatory support in congenital neuromuscular disorders — congenital myopathies, congenital muscular dystrophies, congenital myotonic dystrophy and SMA (II) 4–6 April 2003, Naarden, The Netherlands.Neuromuscul Disord. 2004; 14: 56-69
- Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy.Chest. 1997; 112: 1024-1028
- Changes in spirometry over time as a prognostic marker in patients with Duchenne muscular dystrophy.Am J Respir Crit Care Med. 2001; 164: 2191-2194
- Respiratory management of the infant with type 1 spinal muscular atrophy.Arch Dis Child. 2005; 90: 709-711
- Pulmonary function and scoliosis in children with spinal muscular atrophy types II and III.J Paediatr Child Health. 2003; 39: 673-676
- Lung and respiratory muscle function in limb girdle muscular dystrophy.Thorax. 1994; 49: 61-65
- Control of breathing in patients with limb girdle dystrophy: a controlled study.Thorax. 1995; 50: 962-968
- Diagnosis and management of the limb girdle muscular dystrophies.Pract Neurol. 2009; 9: 314-323
- Mechanism of CO2 retention in patients with neuromuscular disease.Chest. 2000; 117: 447-453
- Comparison study of chest physiotherapy home training programs on respiratory functions in patients with muscular dystrophy.Clin Rehabil. 2008; 22: 741-748
- Prevalence of cardiomyopathy in Duchenne and Becker's muscular dystrophy.J Ayub Med Coll Abbottabad. 2008; 20: 7-13
- Pulmonary complications of chronic neuromuscular diseases and their management.Muscle Nerve. 2004; 29: 5-27
- Respiratory complications of the muscular dystrophies.Semin Respir Crit Care Med. 2002; 23: 231-238
- Sleep disordered breathing in facioscapulohumeral muscular dystrophy.J Neurol Sci. 2009; 285: 54-58
- Ventilatory support in facioscapulohumeral muscular dystrophy.Neurology. 2004; 63: 176-178
- Alveolar hypoventilation as an early symptom of muscle weakness in facioscapulohumeral muscular dystrophy.Sleep Med. 2009; 10: 592-593
- Lung and respiratory muscle function in facioscapulohumeral muscular dystrophy.Muscle Nerve. 2009; 39: 729-734
- Limb-girdle muscular dystrophies.Curr Opin Neurol. 2008; 21: 576-584
- Diagnosis and new treatments in muscular dystrophies.J Neurol Neurosurg Psychiatry. 2009; 80: 706-714
- The diagnostic criteria for facioscapulohumeral dystrophy.Neuromuscul Disord. 1991; 1: 231-234
- Facioscapulohumeral muscular dystrophy.Curr Opin Neurol. 2009; 22: 539-542
- Manual muscle testing.Muscle Nerve. 1990; 13: S16-S20
- Chest wall and lung volume estimation by optical reflectance motion analysis.J Appl Physiol. 1996; 81: 2680-2689
- Human respiratory muscle actions and control during exercise.J Appl Physiol. 1997; 83: 1256-1269
- Abdominal volume contribution to tidal volume as an early indicator of respiratory impairment in Duchenne muscular dystrophy.Eur Respir J. 2010; 35: 1118-1125
- Effects of gender and posture on thoraco-abdominal kinematics during quiet breathing in healthy adults.Respir Physiol Neurobiol. 2010; 172: 184-191
- Value of spirometry in detecting volume restriction in interstitial lung disease patients spirometry in interstitial lung diseases.Respiration. 2004; 71: 374-379
- Evaluation of cardiac and respiratory involvement in sarcoglycanopathies.Neuromuscul Disord. 2001; 11: 178-185
- Respiratory involvement in primary muscle disorders: assessment and management.Q J Med. 1993; 86: 175-189
- Profiles of neuromuscular diseases. Becker's muscular dystrophy.Am J Phys Med Rehabil. 1995; 74: S93-S103
- Compartmental analysis of breathing in the supine and prone positions by optoelectronic plethysmography.Ann Biomed Eng. 2001; 29: 60-70
- Optoelectronic plethysmography in intensive care patients.Am J Respir Crit Care Med. 2000; 161: 1546-1552
- Breathing pattern and chest wall volumes during exercise in patients with cystic fibrosis, pulmonary fibrosis and COPD before and after lung transplantation.Thorax. 2010; 65: 808-814
- Respiratory muscle testing: a valuable tool for children with neuromuscular disorders.Am J Respir Crit Care Med. 2006; 174: 67-74
- Magnetic stimulation for the measurement of respiratory and skeletal muscle function.Eur Respir J. 2004; 24: 846-860
- Limitations of sniff nasal pressure in patients with severe neuromuscular weakness.J Neurol Neurosurg Psychiatry. 2003; 74: 1685-1687
- EFNS guideline on diagnosis and management of limb girdle muscular dystrophies.Eur J Neurol. 2007; 14: 1305-1312
Article info
Publication history
Published online: April 29, 2011
Accepted:
March 29,
2011
Received in revised form:
March 11,
2011
Received:
December 28,
2010
Identification
Copyright
© 2011 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
