Sudden death due to central alveolar hypoventilation syndrome (Ondine's curse) in a 39-year-old woman with heterotopia of the inferior olive

Failure of automatic involuntary respiration with preservation of voluntary respiratory drive (Ondine's curse) is a rare occurrence which has been reported following a variety of morphologic lesions near respiratory centers in the lower brainstem. We report the case of a 39-year-old woman with a syndrome of fulminant respiratory failure with features of Ondine's curse in whom neuropathologic examination disclosed a preexisting malformation of the lower brainstem, as well as acute local subarachnoid bleeding. Mechanisms in the present case are discussed and a review of similar cases published so far is given. The necessity of sound investigation, including neuropathologic studies in cases of sudden unexplained death, is underlined.