Ondine’s Curse (Congenital Central Hypoventilation Syndrome)
Ondine’s curse, also called congenital central hypoventilation syndrome (CCHS) or primary alveolar hypoventilation, is a respiratory disorder that is fatal if untreated. Persons afflicted with Ondine’s curse classically suffer from respiratory arrest during sleep.
CCHS is congenital or developed due to severe neurological trauma to the brainstem. The diagnosis may be delayed because of variations in the severity of the manifestations or lack of awareness in the medical community, particularly in milder cases. (Chin, 2006). There are also cases when the diagnosis is made in later life and middle age, although the symptoms are usually obvious in retrospect. Again, lack of awareness in the medical community may cause such a delay.
This is a very rare and serious form of central nervous system failure, involving an inborn failure of autonomic control of breathing. About 1 in 200,000 live born children have the condition. In 2006, there were only about 200 known cases worldwide. In all cases, episodes of apnea occur in sleep, but in a few patients, at the most severe end of the spectrum, apnea also occurs while awake.
CCHS susceptibility is not known to be affected by gender.