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Key Features

  • 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

  • Lethargy, headache, and somnolence

  • Dyspnea is not present

  • Cyanosis

  • Evidence of pulmonary hypertension and cor pulmonale

  • Erythrocytosis may be present

Diagnosis

  • Arterial blood gas values show hypoxemia and hypercapnia, which improve with voluntary hyperventilation

Treatment

  • 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

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