Septooptic Dysplasia (de Morsier syndrome)

Unknown Brain Case 10


Presentation

6 y/o boy with history of seizures from birth.

Image Findings

Absence of the corpus collosum is associated with mild hypoplasia of the optic nerves and chiasm. Evidence of prior right temporal lobe resection for intractable seizures is noted. Posteriorly, deformity of the  right sylvian fissure with abnormal grey matter is noted on the proton-density coronal and T2-weighted axial images.

Discussion

Septooptic dysplasia may be considered a mild form of lobar holoprosencephaly. Absence of the septum pellucidum, hypoplastic optic nerves, squared-off frontal horns and schizencephaly are frequent findings.


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Last modified December 30, 1996
Copyright 1996 Ray Ballinger, University of Florida and VA Medical Center, Gainesville, FL.
URL: http://www.xray.ufl.edu/~rball/teach/4052.html