Sleep and neuromuscular disorders in children

Children suffering from neuromuscular diseases are at an increased risk of steep-related breathing disorders (SRBD) such as obstructive steep apnea syndrome (OSAS) and hypoventilation as well as central steep apnea, which is frequent in these patients due to diaphragmatic weakness. They are at higher risk for developing complications of nocturnal hypoxemia, including pulmonary hypertension, cor pulmonate and neurocognitive dysfunction. Neuromuscular disorders and OSAS are both prevalent disorders and frequently overlap. Steep-related hypoventilation/hypoxemia due to neuromuscular diseases may be exacerbated in the presence of OSAS; these children are likely to experience greater severity and duration of sleep-related hypoxemia than are children with either disorder atone. Additionally, some of these children have reduced central neural chemo responsiveness. The development of SRBD in these patients further impairs their quality of life and worsens their respiratory status. We review the literature on the diagnosis and treatment of SRBD in children with a variety of neuromuscular disorders. (C) 2008 Elsevier Ltd. All rights reserved.