Acquired Ondine's curse: case report

We report and discuss the case of a 55-year old man who presented a history of stroke as well as chronic obstructive pulmonary disease. When admitted into the emergency room, he was diagnosed with a vertebro-basilar syndrome. A brain MRI showed a hyperintense area in the lower right brainstem laterally within the medulla, which corresponds to the area of the pathways descending from the autonomic breathing control center. During hospitalization, the patient had several episodes of prolonged apnea, mainly when asleep, having often to be "reminded" to breath. A tracheostomy was then performed with the patient under mechanical ventilation. Treatment with medroxyprogesterone, fluoxetine and acetazolamide was also started. He was discharged after 64 days breathing environmental air with no apparent episodes of apnea. He returned to the emergency room in the following day with a clinical picture of aspiration bronchopneumonia, followed by septic shock and death.

the Ondine's curse is one of the posterior stroke's presentation characterized by loss of automatic breathing and for the unpredictability of clinical evolution and prognosis. Such a syndrome has rarely been reported in adults and the diagnostic criteria are not consensual in the reviewed literature. Thus any diagnostic confirmation should be flexible. There are many therapeutic symptomatic options in such cases, ranging from pharmacologic approach, use of bilevel positive airway pressure and implantation of diaphragmatic pacemaker.