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Disorders Of Sleep And Breathing During Sleep In Neuromuscular Disease

E. V. Lunteren, H. Kaminski
Published 1999 · Medicine

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The role of upper airway muscle contractile dysfunction in the pathophysiology of sleep apnea is not clear. The conventional understanding of this disorder is that anatomical narrowing of the upper airway combined with inadequate activation of upper airway dilator muscles leads to airway closure and obstructive apneas.1 The possibility of upper airway muscle contractile failure playing a role in this process is based on a number of findings: (a) increased motor activity to these muscles in humans and dogs with compared to without sleep apnea,2,3 (b) studies of upper airway muscle contractile and structural properties indicating low fatigue resistance due to a high proportion of fast fibers and myosins,4–8 (c) changes in pharyngeal muscle properties in conditions associated with sleep apnea (e.g., hypothyroidism, Down syndrome, old age),6,8,9 and (d) abnormalities in pharyngeal muscle structural properties in humans and bulldogs with sleep apnea.10–12 In support of the notion of pharyngeal muscle dysfunction leading to sleep apnea and alterations in sleep architecture are a number of studies examining sleep and breathing during sleep in (generally a small number of) subjects with disorders of skeletal muscles or the neuromuscular junction—myotonic dystrophy, Duchenne muscular dystrophy, and myasthenia gravis.13–18 These studies have described a high prevalence of sleep apnea (e.g., 11 of 20 subjects with myasthenia gravis studied at time when they were clinically stable had a respiratory disturbance index of greater than 5),18 worsening of nighttime hypoxemia over time (e.g., 9 of 10 patients with muscular dystrophy who were hypoxemic at their initial evaluation had worsened hypoxemia at a follow-up study),16 and altered sleep architecture Disorders of Sleep and Breathing during Sleep in Neuromuscular Disease
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