PEDIATRICS Vol. 41 No. 1 January 1968, pp. 130-138
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CEREBRAL GIGANTISM

John N. Stephenson M.D.1, Raymond C. Mellinger M.D.1, and Gordon Manson M.D.1

1 Department of Pediatrics and Division of Endocrinology Henry Ford Hospital, Detroit

Studies are reported of 10 children having physical findings previously reported in cases classified as showing cerebral gigantism. These findings included mental retardation; advanced height, weight, and bone age; and a characteristic facies. Six had pneumoencephalography and all showed communicating hydrocephalus. Karyograms were normal. Urinary 17-KS steroid excretion was increased for chronologic age, but levels were compatible with physiologic age. Adrenal response to dexamethasone suppression and metyrapone administration was normal. The condition is regarded as a reflection of congenital central nervous system dysfunction with mental retardation and altered hypothalamic control of anterior pituitary function.

Submitted on May 20, 1967
Accepted on August 17, 1967


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