Alveolar hypoventilation due to involvement of the respiratory center by obscure disease of the central nervous system

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      In four patients with alveolar hypoventilation secondary to dysfunction of the respiratory center, the clinical picture resembled, respectively, congenital heart disease, primary polycythemia, arteriosclerotic heart disease and narcolepsy.
      The diagnosis of primary alveolar hypoventilation should be suspected in a polycythemic or cyanotic patient if central nervous system symptoms such as somnolence, headache and easy fatigability are prominent, or if electrocardiographic and roentgenologic evidence of right ventricular enlargement or strain are present in the absence of dsypnea. The diagnosis can be established by demonstrating the arterial blood gas changes to alveolar hypoventilation (elevated carbon dioxide, reduced oxygen saturation, normal or low pH) in the absence of lung disease, extreme obesity or disease of the neuromuscular apparatus of respiration.
      It is suggested that the syndrome may occur as a complication of many neurologic diseases because of the susceptibility of the medullary respiratory center to noxious agents. Other manifestations of the underlying neurologic disease may be slight or absent, and the clinical picture may be dominated by the sequelae of hypoventilation, anoxemia, hypercapnia, polycythemia, right ventricular hypertrophy, strain and failure.
      Our experience suggests that the primary hypoventilation syndrome is compatible with prolonged life and that therapy should be directed primarily at maintaining normal blood viscosity and cardiac compensation.
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