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Ondine’s curseJoe Segen2016-12-07T00:57:31+00:00
An über rare–300 cases in the world literature–inherited condition (OMIM:209880) characterised by respiratory arrest during sleep, daytime drowsiness, fatigue, cyanosis, dysphagia, pupillary dysfunction and hypersensitivity to sedatives and narcotics.
Molecular pathology Defects of PHOX2B, which encodes a protein involved in specific noradrenergic neurons, including those of the locus coeruleus, cause Ondine’s curse.
Ondine’s curse has also been (mis)used for:
(1) Central apnoea caused by strokes, trauma–e.g., MVA/RTA to the brain stem, and
(2) Sleep apnoea syndrome
To the profound disappointment of literati, the term is discouraged from active use. That said, it is a way for attendings and consultants to strut their grey matter in public, especially with junior doctors. The name was coined with reference to Giraudoux’s play, Ondine (1939). In this German folk epic, Ondine, a water nymph, falls in love with Hans, a mortal. When Hans is unfaithful to her, the king of the nymphs curses him, forcing Hans to remember to perform all bodily functions, even those that occur automatically, such as breathing. When Hans falls asleep, he “forgets” to breathe and dies.
Because it was the king, not Ondine, who cursed the mortal, and because those with congenital central hypoventilation syndrome do not actually “forget” to breathe, the term Ondine’s curse is a misnomer. In another telling of the tale, Ondine herself cursed Hans.
Synonyms Congenital central hypoventilation syndrome, central apnoea, primary alveolar hypoventilation