J Korean Ophthalmol Soc.  1993 Jan;34(1):70-74.

A Case of Septo-Optic Dysplasia-Schizencephaly Complex

Affiliations
  • 1Department of Ophthalmology, College of Medicine Chonbuk National University, Chonju, Korea.

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.

Keyword

agenesis of septum pellucidum; optic nerve hypoplasia; schizencephaly

MeSH Terms

Brain
Counseling
Diagnosis
Humans
Lissencephaly
Malformations of Cortical Development
Neurologic Manifestations
Optic Nerve
Parents
Seizures
Septo-Optic Dysplasia
Septum Pellucidum
Vision Disorders
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