Sener R N
Department of Radiology, Ege University Hospital, Bornova, Izmir, Turkey.
J Neuroradiol. 1996 Sep;23(2):79-81.
It is currently believed that there are two subsets of septo-optic dysplasia (de Morsier's syndrome), one with schizencephaly and the other without schizencephaly. Also, some authors consider septo-optic dysplasia as a mild form of holo-prosencephaly. This article describes a third 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, excluding holoprosencephaly. They had normal facies. All of them presented with seizures, and no hormonal abnormality was found. This new clinicoradiological type of the anomaly is suggested to be labelled as calloso-septo-optic or calloso-optic dysplasia.
目前认为，视隔发育不良（德莫西埃综合征）有两个亚组，一组伴有脑裂畸形，另一组不伴有脑裂畸形。此外，一些作者认为视隔发育不良是全前脑畸形的一种轻度形式。本文描述了该异常的第三种形式，它与胼胝体完全缺如有关。之前曾报道过两名与该病例相似的患者。这三名患者均无任何形式的半球间融合，可排除全前脑畸形。他们面容正常。所有患者均有癫痫发作，未发现激素异常。建议将这种新的临床放射学类型的异常标记为胼胝体 - 视隔发育不良或胼胝体 - 视神经发育不良。
