Cerebral Gigantism

Abstract

In 1964 cerebral gigantism emerged as a syndrome. The original five patients grew excessively during the first four years of life and had advanced height, weight, and bone age thereafter. They had distinctive facies, large dolichocephalic heads, hypertelorism, antimongoloid slant of the palpebral features, high arched palates, long arm span, and large hands and feet. Four of the five children were mentally retarded and clumsy; yet none showed gross neurological abnormalities. Pneumoencephalography revealed enlarged ventricles in three of the patients. After ruling out all the known causes of excessive growth, such as neurofibromatosis, Marfan's syndrome, adrenogenital syndrome, thyrotoxicosis, and excessive growth hormone secretion, to name a few, the authors concluded that the pattern of clinical findings failed to conform to any of the recognized causes of tall stature and that the remarkable similarity among the cases justified their grouping as a syndrome.¹ Thirteen years have passed, and there are