Late-onset Central Alveolar Hypoventilation Syndrome in a Patient with Hemorrhagic Brainstem Lesion: Improvement after Medroxyprogesterone Treatment

Abstract

Central hypoventilation syndrome, a rare condition, is defined as the failure of the automatic control of breathing. Secondary central hypoventilation syndrome should be distinguished from congenital central hypoventilation syndrome by the abnormalities in the brainstem, the place of respiratory control. Patients with this syndrome manifest hypoventilation during sleep, sometimes accompanied by hypoventilation during periods of wakefulness. Patients also lack a ventilatory response to hypercapnia and progressive hypoxia. We report the case of a 57-year-old woman who presented with chronic alveolar hypoventilation syndrome secondary to a brainstem lesion. The initial manifestation was a sense of dyspnea during sleep, and daytime sleepiness. The ventilatory response to CO2 was markedly decreased. She was treated successfully with a central stimulator (oral medroxyprogesterone 30 mg per day). We concluded that central alveolar hypoventilation should be highly suspected in patients with hypercapnia and a brainstem lesion, which can-be treated with a central stimulant.