SEPTO-OPTIC DYSPLASIA (DE MORSIERS SYNDROME) ASSOCIATED WITH TOTAL CALLOSAL ABSENCE - A NEW-TYPE OF THE ANOMALY

It is currently believed that there are two subsets of septo-optic dys plasia (de Morsier's syndrome), one with schizencephaly and the other without schizencephaly. Also, some authors consider septo-optic dyspla sia as a mild form of holoprosencephaly. This article describes a thir d form of the anomaly which is associated with total callosal absence. Two patients similar to this one have previously been reported. None of these three patients had interhemispheric fusion in any form, exclu ding holoprosencephaly. They had normal facies, All of them presented with seizures, and no hormonal abnormality was found. This new clinico radiological type of the anomaly is suggested to be labelled as callos o-septo-optic or calloso-optic dysplasia.