Respiratory muscle testing: a valuable tool for children with neuromuscular disorders.
BACKGROUND
Data on respiratory muscle performance in children with neuromuscular disorders are limited.


OBJECTIVE
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.


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.
AP-HP, Hopital Armand Trousseau, Pediatric Pulmonary Department, Research Unit INSERM U 719, Université Pierre et Marie Curie-Paris 6, Paris, France.
Frédéric
F
Nicot
Nicholas
N
Hart
Véronique
V
Forin
Michèle
M
Boulé
Annick
A
Clément
Michael I
MI
Polkey
Frédéric
F
Lofaso
Brigitte
B
Fauroux
D000293:Adolescent; D002648:Child; D005240:Feasibility Studies; D006801:Humans; D053580:Muscle Strength / Q000502:physiology*; D020388:Muscular Dystrophy, Duchenne / Q000503:physiopathology* / Q000523:psychology; D020914:Myopathies, Structural, Congenital / Q000503:physiopathology* / Q000523:psychology; D011312:Pressure; D015656:Respiratory Mechanics / Q000502:physiology; D012132:Respiratory Muscles / Q000503:physiopathology*; D014897:Spinal Muscular Atrophies of Childhood / Q000503:physiopathology* / Q000523:psychology; D014797:Vital Capacity / Q000502:physiology*; D014836:Volition