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July 1966

Primary (Neurogenic) Alveolar Hypoventilation: A Case Report and Review of the Literature

Author Affiliations

From the Department of Medicine of the Beth Israel Hospital and the Department of Medicine, Harvard Medical School, Boston (Dr. Stein).

Arch Neurol. 1966;15(1):94-99. doi:10.1001/archneur.1966.00470130098011

THE SYNDROME of primary (neurogenic) alveolar hypoventilation is believed to result from impairment of brain stem mechanisms subserving respiration without concomitant disease of the lungs, chest wall, or the muscles of respiration. Lack of a normal ventilatory response to increases of arterial blood carbon dioxide tension is the hallmark of the syndrome. The consequences of chronic alveolar hypoventilation are hypercapnia, hypoxemia, cyanosis, somnolence, and erythremia. Pulmonary hypertension and right ventricular strain may develop. Since 1955 twenty-two patients with this syndrome have been described.1-14 To our knowledge this is the first patient described in whom the diagnosis of primary (neurogenic) alveolar hypoventilation was made prior to a surgical operation requiring general anesthesia, and, therefore, in whom specific steps were taken in management to prevent respiratory arrest postoperatively. In addition, the literature of the syndrome is reviewed.

This report describes a patient in whom the syndrome was demonstrated during