ABSTRACT
The development of respiratory failure is common in patients with neuromuscular disorders
that involve the respiratory muscles. However, the high incidence of sleep-related
breathing problems in this population is less well known. In patients with neuromuscular
disease, nocturnal breathing abnormalities frequently precede respiratory failure
during wakefulness by months or even years. These nocturnal breathing problems are
caused by multiple factors, including diaphragm and upper airway muscle weakness,
scoliosis, obesity, and central respiratory control problems. Advances in the understanding
of the links between sleep-disordered breathing and the development of daytime dysfunction
and respiratory failure has revolutionized the management of these individuals. Mask
positive pressure therapy is now available to improve both quality of life and longevity
for these individuals. The lack of correlation between daytime testing and the severity
of nocturnal breathing abnormalities makes it difficult to predict the presence of
sleep-disordered breathing. Further, patients may not always be aware of symptoms
associated with sleep-disordered breathing, even if specifically questioned. However,
simple bedside measurements of vital capacity and inspiratory muscle strength can
provide useful guides for when nocturnal respiratory monitoring is indicated.
KEYWORDS
REM hypoventilation - nocturnal respiratory failure - obstructive sleep apnea - sleep
