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December 1965

The Diencephalic Syndrome of Infancy: Report of Two Cases

Author Affiliations

From the Department of Pediatrics, Kaiser Foundation Hospital, and the Permanente Medical Group, San Francisco.

Am J Dis Child. 1965;110(6):689-696. doi:10.1001/archpedi.1965.02090030717020

THE SUBTLETY of differential diagnosis of failure to thrive in infancy has been greatly enhanced by consideration of such widely different causes as intestinal malabsorption and emotional deprivation. But the pediatrician's very awareness that so many possible reasons for cachexia exist may lead him to overlook the relatively specific diencephalic syndrome of infancy—depletion of subcutaneous fat beginning within the first year of life, often presenting as failure to thrive. This syndrome is "relatively specific" in the sense that it is clearly associated with a tumor in the diencephalic region, and that it is characterized by a peculiarly drastic depletion of subcutaneous fat in an infant whose food intake is normal or greater than normal in amount. Other clinical aspects of the syndrome observed in the 23 cases reported heretofore,1-9 hyperkinesia, euphoria, and a variety of other evidences of autonomic dysfunction, are less well defined, and the intermediate process through

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