J Korean Ophthalmol Soc.
2002 Jul;43(7):1335-1339.
A Case of DeMorsier Syndrome Misdiagnosed as Amblyopia
- Affiliations
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- 1Department of Ophthalmology, Taejeon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Korea. KILee@djsungmo.com
Abstract
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PURPOSE: We report a 32-year-old man with DeMorsier syndrome, who was referred for seizures, esotropia and visual disturbance of left eye.
METHODS
Fundus examination, visual field examination and the visual evoked potential were performed, and other diagnostic studies included endocrine determinations, the electroencephalogram and MR imaging.
RESULTS
Ophthalmic examination revealed left optic nerve hypoplasia with the "double ring" sign. Axial MR T1 weighted image showed tiny left optic nerve and absence of the septum pellucidum. Subsequent evaluation revealed temporal visual field defect, abnormal VEP and deuteranomaly of left eye.
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