Abstract
Two cases of cerebral gigantism in childhood are reported, and 14 earlier cases are reviewed. The major manifestations of this non-progressive neurological disorder included gigantism, macrocrania, dolichocephaly, mental retardation, characteristic facies, high arched palate, and ataxia or clumsiness. Pneumoencephalography in 10 out of 11 cases revealed a dilated ventricular system. Normal fasting plasma growth hormone levels were found in our patients, but both showed evidence of impaired function of the hypothalamic-pituitary axis in that these levels failed to rise following marked hypoglycemia. Abnormal dermatoglyphic patterns are reported and their value as an aid to diagnosis is mooted. Both the cause and the nature of the neurological lesion remain obscure. The evidence favors a pathogenic mechanism operative in utero.
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