Monocular Temporal Hemianopia in a Young Adult with Septo-Optic Dysplasia-A Case Report

成人腦室中隔缺損合併視神經發育不全所引起之單眼顳側偏盲：案例報告

林均昶(Chun-Chang Lin) ； 汪宜樺(I-Hua Wang) ； 林思源(Szu-Yuan Lin) ； 周介仁(Jieh-Ren Jou)

《中華民國眼科醫學會雜誌》 48卷1期 (2009/03) Pp. 98-104

https://doi.org/10.30048/ACTASOS.200903.0015

摘要

目的：報告一因腦室中隔缺損合併視神經發育不全所引起單眼顯側偏盲之稀有病例
方法：病例報告
結果：一26年輕男性經檢查後發現右眼顯側視野缺損併同側瞳孔傳入障礙及鼻側視神經盤發育不良。患者雙眼視力為20/20，且辨色力正常。患者有癲癇病史。在腦部核磁共振顯影下發現患者有部份胼胝體發育不良併透明膈缺損。右眼的視覺電生理有逐漸減弱的現象。
結論：腦室中隔缺損合併視神經發育不全為顱內發育不全的一種疾病，其特色包括胼胝體發育不良，裂腦孔洞腦畸形，腦葉前腦發育畸形症。亦常伴隨著神經盤發育不正常，腦下腺分泌缺損，以及透明膈發育缺失。因腦室中隔缺損合併視神經發育不全所造成的單眼顯側偏盲臨床上極為少見。

並列摘要

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.

並列關鍵字

Monocular temporal hemianopia ； septo-optic dysplasia ； nasal disc hypoplasia