Diencephalic syndrome of infantile emaciation analysis of literature and report of further 3 cases

Abstract

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