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May 1977

Picture of the Month

Author Affiliations

From the Center for Genetic Counseling and Birth Defect Evaluation, New England Medical Center Hospital, Boston.

Am J Dis Child. 1977;131(5):579-580. doi:10.1001/archpedi.1977.02120180093019

Denouement and Discussion 

Cerebral Gigantism (Sotos Syndrome) 

Manifestations  Major manifestations include increased birth weight and length, rapid growth during the first few years of life, characteristic facial appearance, large hands and feet, and mental retardation. The skull is usually dolichocephalic. The characteristic facial appearance consists of apparent ocular hypertelorism, antimongoloid slant of the palpebral fissures, large ears, prognathism, high arched palate, broad bridge of the nose, and occasionally, a broad nose. Neurologically, the patients may be clumsy or ataxic and have seizures. In the neonatal period, drooling, respiratory distress, and feeding problems may be present. Rapid growth occurs during the first four or five years of life, and then height and weight parallel the normal growth curve. The severity of the mental retardation varies, but is usually in the moderate range. Laboratory studies, including various endocrinological tests, are almost always within normal limits. The ventricles are dilated on pneumoencephalography, and

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