Diencephalic syndrome of infantile emaciation. Analysis of literature and report of further 3 cases

Abstract

Three children with diencephalic tumours are described, and an analysis of 45 cases from the literature together with our own 3 is presented. Boys are affected more frequently than girls (1·8:1) and the onset is during the first 12 months of life in 92% of cases. The main clinical features are emaciation, overactivity, pleasant disposition, and above-average height. The natural course of the disease is variable. Treatment is by radiotherapy. Operative intervention, except to relieve intracranial hypertension, is not usually indicated.