• Deborah F Gelinas.
• Department of Neurology, California Pacific Medical Center, San Francisco, California, USA.
• Semin Neurol. 2003 Mar 1;23(1):89-96.

AbstractPatients who suffer from neuromuscular diseases often have complications from respiratory insufficiencies. Some neuromuscular diseases, for example Landry Guillain-Barré syndrome, may only require temporary tracheal intubation; patients with other neuromuscular diseases, such as amyotrophic lateral sclerosis, may decide with the assistance of their doctor and family to opt for lifelong noninvasive ventilatory support. Other patients may only opt for noninvasive positive pressure ventilation. Respiratory dysfunction is caused by weakness of the upper airway muscles, which can lead to sleep apnea, abnormal swallow, and decreased respiratory muscle strength, as well as a decrease in total lung volume. Early respiratory changes in patients with neuromuscular disease are often best detected during sleep. During rapid eye movement sleep, there is a reduction in respiratory drive predisposing to hypopneas and apneas. The majority of neuromuscular patients with respiratory insufficiency may be monitored and treated in the outpatient setting, thus allowing them to remain in their homes.

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