Breathing During Sleep in Patients with Myotonic Dystrophy and Non-myotonic Respiratory Muscle Weakness

Somnolence in myotonic dystrophy (DM) has not been measured using a reliable daytime somnolence scale. The aim of this study was to compare somnolence in DM patients with healthy controls and Charcot-Marie-Tooth disease (CMT) patients using such a scale and to compare this with potential contributory factors. We investigated 35 subjects with adult-onset DM, 16 healthy controls and 13 CMT controls. The Epworth Sleepiness Scale (ESS) was the principal measurement of daytime somnolence. Nocturnal sleep was assessed using a sleep diary. Other assessments measured daytime respiratory function, cognitive function, motor impairment, disability, swallowing capacity and depression. DM and CMT patients had greater daytime sleepiness than unaffected controls. In the DM group significant correlations were found between somnolence and measures of disability, sleep quality and some measures of depression. It was concluded that there is an abnormal level of daytime somnolence in DM, which is partially associated with disability.

show abstract

Disorders of Sleep and Breathing during Sleep in Neuromuscular Disease

Lunteren

Kaminski

1999

Sleep Breath

View full text Add to dashboard Cite

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][11][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

show abstract

scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.

Contact Info

customersupport@researchsolutions.com

10624 S. Eastern Ave., Ste. A-614

Henderson, NV 89052, USA

This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.

Blog Terms and Conditions API Terms Privacy Policy Contact Cookie Preferences Do Not Sell or Share My Personal Information

Made with 💙 for researchers

Part of the Research Solutions Family.

Breathing During Sleep in Patients with Myotonic Dystrophy and Non-myotonic Respiratory Muscle Weakness

Cited by 23 publications

References 0 publications

Psychostimulants for hypersomnia (excessive daytime sleepiness) in myotonic dystrophy

Psychostimulants for hypersomnia (excessive daytime sleepiness) in myotonic dystrophy

Daytime somnolence in myotonic dystrophy

Disorders of Sleep and Breathing during Sleep in Neuromuscular Disease

Contact Info

Product

Resources

About