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De morsier syndrome (septooptic dysplasia) 356


De Morsier Syndrome (Septooptic Dysplasia)& 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;   & 858f57i nbsp;    356

General: Absence of the septum pellucidum; agenesis of corpus callosum; enlargement of ventricles; infundibulum primary site of structural derangement in patients with optic nerve hypoplasia.

Ocular: Optic disk hypoplasia; bitemporal hemianopia; poor vision; nystagmus.

Clinical:

Growth retardation; pituitary insufficiency; diabetes insipidus; normal cognitive development; intact neurologic status; normal language development; late appropriate behavior; abnormal early poor motor coordination; subtle visual alterational problems; association between optic nerve hypoplasia and cerebral hemispheric abnormalities, especially schizencephaly, due to migration anomalies.

Brodsky MC, Glasier CM. Optic nerve hypoplasia. Clinical significance of associated central nervous system abnormalities on magnetic resonance imaging. Arch Ophthalmol 1993; 11:66-74.

de Morsier G. Median craniocephalic dysraphia and olfactogenital dysplasia. World Neurol

Rush JA, Bajandas FJ. Septo-optic dysplasia (de Morsier syndrome). Am J Ophthalmol

Williams J, et al. Septo-optic dysplasia: the Clinical insignificance of an absent septum pellucidum. Dev Med Child Neurol 1993; 35:490-501.




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