ABSTRACT
In 1962, Severinghaus first used the term ‘‘Ondine’s curse’’ to describe three adults
who lacked ventilatory responsivity to CO2 following surgery to the brain stem (1).
This term has been most frequently used to describe a rare disorder where infants
appear to breathe reasonably well while awake, but severely hypoventilate and/or
become apneic during sleep. Central hypoventilation syndromes, although uncom-
mon, are now more frequently diagnosed. They range in frequency, severity, and
clinical presentation, but advances in our knowledge and technology now permit
promptdiagnosis and comprehensive treatment. Thus,manyof these patients survive
with a good quality of life. This chapter will describe some of the disorders in infants
and children known to cause hypoventilation during sleep.
