A Case of DeMorsier Syndrome Misdiagnosed as Amblyopia.

Lim, Chang Hyun; Lee, Kyoo Il; Bae, Sun Ryang

Case Report

Journal of the Korean Ophthalmological Society 2002;43(7):1335-1339.
Published online July 1, 2002.

A Case of DeMorsier Syndrome Misdiagnosed as Amblyopia.

Chang Hyun Lim, Kyoo Il Lee, Sun Ryang Bae

Department of Ophthalmology, Taejeon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Korea. KILee@djsungmo.com

약시로 오진된 De Morsier 증후군

임창현 ( Chang Hyun Lim ) , 이규일 ( Kyoo Il Lee ) , 배선량 ( Sun Ryang Bae )

Abstract

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.

Key Words: DeMorsier syndrome;Double ring sign;Septum pellucidum