Septooptic Dysplasia (de Morsier syndrome)
6 y/o boy with history of seizures from birth.
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.
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.