Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 9-40: Primary Alveolar Hypoventilation + Key Features Download Section PDF Listen +++ ++ 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 + Clinical Findings Download Section PDF Listen +++ ++ Lethargy, headache, and somnolence Dyspnea is not present Cyanosis Evidence of pulmonary hypertension and cor pulmonale Erythrocytosis may be present + Diagnosis Download Section PDF Listen +++ ++ Arterial blood gas values show hypoxemia and hypercapnia, which improve with voluntary hyperventilation + Treatment Download Section PDF Listen +++ ++ 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