Ondine’s curse (also known as congenital central hypoventilation syndrome) is a rare, severe form of sleep apnea in which the affected individual completely stops breathing when falling asleep.
Ondine’s curse is named after a mythical story about a heartbroken water nymph who curses her unfaithful husband to stop breathing should he ever fall asleep again. In medical terms, central hypoventilation represents an extreme form of sleep apnea.
What Causes Central Hypoventilation?
Central hypoventilation affects about one in 30 million people, which means only several hundred people have it in the world. A genetic mutation appears to be the underlying cause. It is thought to occur when the brain fails to prompt breathing, as may also be seen in central sleep apnea.
This condition may be present from birth or it may develop after damage to the brainstem, which controls the drive to breathe. Central hypoventilation may be associated with difficulty swallowing, intestinal problems called Hirschsprung’s disease, or tumors called neuroblastoma.
How is Central Hypoventilation Treated?
Treatment of this condition involves the use of a ventilator connected to a tracheostomy tube at the front of the throat whenever the affected individual goes to sleep, even during naps. If this were not used, someone with this condition could die anytime they fall asleep.
Source:
Kryger, MH et al. "Principles and Practice of Sleep Medicine." Elsevier Saunders, 5th edition, 2011.
