Journal of the Korean Ophthalmological Society 1993;34(1):70-74.
Published online January 1, 1993.
A Case of Septo-Optic Dysplasia-Schizencephaly Complex.
Hyo Sook Ahn, Bang Kyun Ahn, Hong Joo Han
Department of Ophthalmology, College of Medicine Chonbuk National University, Chonju, Korea.
열두와 동반된 중격시신경형성장애
안효숙(Hyo Sook Ahn),안방균(Bang Kyun Ahn),한홍주(Hong Joo Han)
Abstract
Septo-optic dysplasia is diagnosed when optic nerve hypoplasia is combined with dysgenesis of the septum pellucidum and nearly two-thirds of them have hypothalamic-pituitary dysfunction. A number of these patients have schizencephaly and usually present seizures. The migrational disorders including schizencephaly, lissencephaly, heterotopia and polymicrogyria are a rare group of congenital malformations of the brain Septo-optic dysplasia-schizencephaly complex is frequently associated with endocrinolo gic, ophthalmologic, and neurologic symptoms and signs. We recently experienced a case of septo-optic dysplasia-schizencephaly, who showed severe visual impairment associated nystagmus and bilateral optic nerve hypoplasia agenesis of septum pellucidum with schizencephaly and hypsarrythmia. Because some forms of migrational disorders and septo-optic dysplasia can be inherited, parental counseling is essential for the accurate diagnosis.
Key Words: agenesis of septum pellucidum;optic nerve hypoplasia;schizencephaly


ABOUT
BROWSE ARTICLES
EDITORIAL POLICY
FOR CONTRIBUTORS
Editorial Office
SKY 1004 Building #701
50-1 Jungnim-ro, Jung-gu, Seoul 04508, Korea
Tel: +82-2-583-6520    Fax: +82-2-583-6521    E-mail: kos08@ophthalmology.org                

Copyright © 2024 by Korean Ophthalmological Society.

Developed in M2PI

Close layer
prev next