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Published ahead of print on March 30, 2006, doi:10.1164/rccm.200512-1841OC
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American Journal of Respiratory and Critical Care Medicine Vol 174. pp. 67-74, (2006)
© 2006 American Thoracic Society
doi: 10.1164/rccm.200512-1841OC

Original Article

Respiratory Muscle Testing

A Valuable Tool for Children with Neuromuscular Disorders

Frédéric Nicot, Nicholas Hart, Véronique Forin, Michèle Boulé, Annick Clément, Michael I. Polkey, Frédéric Lofaso and Brigitte Fauroux

AP-HP, Hopital Armand Trousseau, Research Unit INSERM U 719, Université Pierre et Marie Curie-Paris 6; Pediatric Pulmonary Department, and Pediatric Physical Rehabilitation, Hopital Armand Trousseau, Paris; AP-HP, Department of Clinical Physiology, Hopital Raymond Poincaré, Garches, France; Lane Fox Respiratory Unit, Guy's and St. Thomas' NHS Foundation Trust, St. Thomas' Hospital; and Respiratory Muscle Laboratory, Royal Brompton Hospital, London, United Kingdom

Correspondence and requests for reprints should be addressed to Pr. Brigitte Fauroux, M.D., Ph.D., AP-HP, Hopital Armand Trousseau, Pediatric Pulmonary Department, Research Unit INSERM U 719, Université Pierre et Marie Curie-Paris6, 28 avenue du Docteur Arnold Netter, Paris F-75012, France. E-mail: brigitte.fauroux{at}trs.aphp.fr

Rationale: Data on respiratory muscle performance in children with neuromuscular disorders are limited.

Objectives: The aim of this study was to assess respiratory muscle strength by volitional and nonvolitional tests and to compare these tests with forced vital capacity.

Methods: Inspiratory muscle strength was assessed by measuring transdiaphragmatic and esophageal pressures generated during volitional and nonvolitional maneuvers, whereas expiratory muscle strength was assessed by measuring the gastric pressure generated during a cough maneuver. Lung volumes were assessed by measuring forced vital capacity.

Measurements and Main Results: Forty-one patients with Duchenne muscular dystrophy (n = 20), spinal amyotrophy (n = 8), and congenital myopathy (n = 13) were included, aged 2 to 18 yr. All the patients were able to perform the sniff and the cough maneuver. Sniff transdiaphragmatic pressure decreased with age in Duchenne patients, whereas it increased with age in patients with spinal amyotrophy and congenital myopathy. Magnetic stimulation of the phrenic nerves was obtained in all patients. Twenty-five (61%) patients were able to perform forced vital capacity. In the three groups of patients, a positive correlation was observed between volitional, assessed by the sniff maneuver, and nonvolitional respiratory muscle tests, assessed by the magnetic stimulation of the phrenic nerves. Also, forced vital capacity correlated with sniff transdiaphragmatic pressure and cough gastric pressure.

Conclusions: Volitional respiratory muscle tests correlated with nonvolitional tests and with forced vital capacity. Simple volitional respiratory muscle tests constitute a valuable tool for the assessment of respiratory muscle strength in young patients with neuromuscular disorders.

Key Words: congenital myopathy • cough maneuver • Duchenne muscular dystrophy • sniff maneuver • spinal amyotrophy




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