Abstract

Purpose: To report a rare case of monocular temporal hemianopia associated with septo-optic dysplasia. Methods: Case report. Results: A twenty-six-year-old male was noted to have a right monocular temporal hemianopic visual field defect together with an ipsilateral afferent pupillary defect and nasal optic disc hypoplasia. His visual acuity was 20/20 (OU) and he had normal color sensation in both eyes. The patient has a history of epilepsy. Brain MRI showed a partial dysgenesis of corpus callosum with absence of septum pellucidum. VEP revealed attenuated response in the right eye. Conclusions: Septo-optic dysplasia (SOD) is a disorder characterized by intracranial malformation, including agenesis of the corpus callosum, schizencephaly, and lobar holoprosencephaly. It is also associated with abnormal development of the optic disc, pituitary deficiencies, and often agenesis (absence) of the septum pellucidum. Monocular temporal hemianopsia is a rare clinical manifestation in septo-optic dysplasia.

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