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A rare syndrome ("Ondine curse") of unknown cause
Characterized by inadequate alveolar ventilation despite normal airways, lungs, chest wall, respiratory muscles, and neurologic function
Patients are usually nonobese males in their 40s and 50s
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Lethargy, headache, and somnolence
Dyspnea is absent
Cyanosis
Evidence of pulmonary hypertension and cor pulmonale
Erythrocytosis is common
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Ventilatory stimulants are not effective
Mechanical augmentation of ventilation via phrenic nerve stimulation, rocking bed, or ventilators may be helpful
Supplemental oxygen should be given, with the caveat that nocturnal oxygen be given only if a sleep study demonstrates that it is effective and safe