Abstract

Recognize sleep disorders associated with neuromuscular diseases. Neuromuscular diseases can be associated with various sleep disorders. Diaphragmatic weakness, bulbar dysfunction, pharyngeal neuropathy, and central neurodegeneration cause sleep-disordered breathing. Spinal cord hyperexcitability, loss of inhibitory descending pathways, and neuropathy promote restless legs syndrome. Reduced cerebrospinal fluid (CSF) hypocretin, central dysfunction of sleep regulation, and degeneration of GABAergic intracortical circuits may contribute to central hypersomnia. Dysfunction of the nigrostriatal dopaminergic system, associated with neurodegenerative diseases and certain neuromuscular disorders, may be a common pathophysiologic mechanism responsible for the loss of rapid eye movement (REM) sleep muscle atonia and REM sleep behavior disorder (RBD). Different neuromuscular disorder may be more susceptible to specific sleep disturbances. A low threshold needs to be maintained for the diagnosis and treatment of sleep disorders in neuromuscular diseases, due to their high prevalence, lack of symptom specificity, and presence even in minimally symptomatic patients. However, loss of REM atonia may be protective against sleep-disordered breathing. There are reasonable pathophysiologic mechanisms such as respiratory muscle weakness, upper airway obstruction, and central dysfunction that explain the link between sleep disorders and neuromuscular diseases

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