Respiration, like Gaul, is divided into three parts: ventilation, perfusion, and diffusion. Inadequate flow of gases to the alveolar space may exist even with normal perfusion of the lungs by pulmonary blood flow and with normal diffusion of gases across the alveolar capillary membranes. The subsequent inadequate ventilation results in severe hypoxemia and hypercapnia with respiratory acidosis. As the syndrome becomes chronic, atelectasis progresses, and changes in perfusion may occur, thereby further increasing the abnormalities. The syndrome may be caused by diseases of the lung parenchyma itself, by diseases of the chest wall, such as neuromuscular disorders, or by disease of the central nervous system involving the pons, medulla, or hypothalamus.
The acute form of the condition, seen during poliencephalomyelitis epidemics, is familiar to most physicians. The chronic form is rare and marked by cardiac decompensation with cor pulmonale, secondary polycythemia, somnolence, seizures, signs and symptoms of increased intracranial pressure,
ONDINE'S CURSE. JAMA. 1965;193(12):1048–1049. doi:10.1001/jama.1965.03090120056016
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