Sleep and Neuromuscular Disease

Abstract

Sleep disorders in patients with neuromuscular disease are common, but underrecognized by health care providers, and sometimes by patients themselves. Their symptoms may be confused with those of the underlying disease. Their recognition is an important part of the management of patients with neuromuscular disorders, improving quality of life, and sometimes increasing survival. Inadequate ventilation underlies many sleep disorders, and sleep-related disorders may presage daytime ventilatory disorders with disease progression. Involvement of the central or peripheral nervous system, or both, may disrupt sleep, with the relative contribution of each depending on the specific disorder. The pertinent anatomy, physiology, and clinical features of sleep disorders in neuromuscular diseases and a basic approach to their assessment is discussed. Specific neuromuscular disorders in which sleep is commonly affected are reviewed and the principles of management of sleep disorders summarized.

The Relationship between Amyotrophic Lateral Sclerosis and Parkinson’s Disease and Sleep Disorders: Pathophysiological and Clinical Approach

“…Automatic ventilation during sleep is almost completely dependent on the diaphragm (particularly in REM sleep) therefore diaphragmatic dysfunction (such as that seen in ALS) can predispose to hypoventilation and nocturnal hypoxemia. The sensitivity of chemoreceptors is also reduced during sleep [1].…”

“…This prevents upper airway collapse. Weakness of pharyngeal muscles (such as in ALS) leaves one susceptible to airway collapse and the upper airway resistance seen in obstructive sleep apnea [1].…”

“…Sleep-breathing disorders are common in ALS. Oropharyngeal weakness affecting dilatory muscles leads to obstructive sleep apnea that presents like in non-ALS OSA patients with snoring, snorting, apneas, and frequent nocturnal arousals [16]. In a study of 18 patients with ALS, it was found on polysomnography that total sleep time was decreased, and frequency of sleep-disordered breathing and arousals were higher than in age-matched controls.…”

“…In general, sleep disorders are underrecognized in this population, as their secondary effects can mimic symptoms of the underlying disease. Recognizing sleep-related disorders in patients with neuromuscular disease such as Amyotrophic Lateral Sclerosis (ALS) is of utmost importance, as they are potentially treatable problems and their correction can improve quality of life and even increase survival time [1].…”

Office Evaluation of the “Tired” or “Sleepy” Patient

“…The September 2009 issue of Seminars in Neurology discussed treatment of specific sleep disorders in more detail. [37][38][39][40][41][42][43][44][45][46][47][48][49][50][51][52] When tiredness is caused by hypersomnia and supported by an abnormal MSLT, a trial of stimulant or wake-promoting medications is usually indicated when treatment of the underlying cause of the hypersomnia fails or is incompletely effective. The American Academy of Sleep Medicine published practice parameters for treatment of hypersomnia in 2007.…”