PDF(Pubmed)
Abstract：
UNASSIGNED: Oculogyric crisis (OGC) is a dystonic movement disorder of varying durations that manifests as bilateral paroxysmal upward eye deviation accompanied by involuntary blinking, tongue protrusion, and autonomic symptoms. Separately, septo-optic dysplasia (SOD) is a congenital disorder involving hypoplasia of the optic nerve as well as hypothalamic and pituitary abnormalities. In the presented case, we report a case of OGC in the setting of SOD with proposed pathogenesis.
UNASSIGNED: A 27-year-old female presented with a history of SOD (optic nerve hypoplasia and hypopituitarism) with acute, recurrent, painless, bilateral, intermittent, simultaneous tonic conjugate upward eye deviation (i.e., OGC) and dystonic body posturing. She experienced her first episode upon meeting her biological sister for the first time at a loud, crowded public restaurant with continued episodes of OGC increasing in frequency and duration over the subsequent months. She later responded well to treatment with carbidopa/levodopa.
UNASSIGNED: Based on our current understanding of OGC, we hypothesize that acute stressful life events in the setting of prior hypothalamic-pituitary axis dysfunction secondary to SOD could lower the threshold for developing OGC. Although most cases of OGC are idiopathic, various etiologies including medications, stress, and hormonal imbalance have been postulated as possible pathogenic mechanisms. We describe a case of SOD with OGC, and based upon our review of the English language ophthalmic literature, we believe that our case is novel.
UNASSIGNED: A 27-year-old female presented with a history of SOD (optic nerve hypoplasia and hypopituitarism) with acute, recurrent, painless, bilateral, intermittent, simultaneous tonic conjugate upward eye deviation (i.e., OGC) and dystonic body posturing. She experienced her first episode upon meeting her biological sister for the first time at a loud, crowded public restaurant with continued episodes of OGC increasing in frequency and duration over the subsequent months. She later responded well to treatment with carbidopa/levodopa.
UNASSIGNED: Based on our current understanding of OGC, we hypothesize that acute stressful life events in the setting of prior hypothalamic-pituitary axis dysfunction secondary to SOD could lower the threshold for developing OGC. Although most cases of OGC are idiopathic, various etiologies including medications, stress, and hormonal imbalance have been postulated as possible pathogenic mechanisms. We describe a case of SOD with OGC, and based upon our review of the English language ophthalmic literature, we believe that our case is novel.
摘要：
■嗅觉危机（OGC）是一种持续时间不同的肌张力障碍运动障碍，表现为双侧阵发性向上偏斜并伴有不自主眨眼，舌头突出，和自主神经症状。分别,视间隔发育不良（SOD）是一种先天性疾病，涉及视神经发育不全以及下丘脑和垂体异常。在本案中,我们报道了一例OGC在SOD背景下的发病机制。
■一名27岁女性，有SOD（视神经发育不全和垂体功能低下）病史，急性，经常性,无痛,双边,间歇性,同时补品共轭向上眼睛偏离(即，OGC）和肌张力障碍的身体姿势。她第一次大声见到她的亲生妹妹时经历了她的第一集，在随后的几个月中，拥挤的公共餐厅不断增加，OGC的发作频率和持续时间不断增加。她后来对卡比多巴/左旋多巴治疗反应良好。
■根据我们目前对OGC的理解，我们假设，在先前的下丘脑-垂体轴功能障碍继发于SOD的情况下，急性应激性生活事件可能会降低OGC的发生阈值。尽管大多数OGC病例是特发性的，各种病因，包括药物，压力，和荷尔蒙失调被假定为可能的致病机制。我们描述了一个OGC的SOD案例，根据我们对英语眼科文献的回顾，我们认为我们的案子很新颖。
■一名27岁女性，有SOD（视神经发育不全和垂体功能低下）病史，急性，经常性,无痛,双边,间歇性,同时补品共轭向上眼睛偏离(即，OGC）和肌张力障碍的身体姿势。她第一次大声见到她的亲生妹妹时经历了她的第一集，在随后的几个月中，拥挤的公共餐厅不断增加，OGC的发作频率和持续时间不断增加。她后来对卡比多巴/左旋多巴治疗反应良好。
■根据我们目前对OGC的理解，我们假设，在先前的下丘脑-垂体轴功能障碍继发于SOD的情况下，急性应激性生活事件可能会降低OGC的发生阈值。尽管大多数OGC病例是特发性的，各种病因，包括药物，压力，和荷尔蒙失调被假定为可能的致病机制。我们描述了一个OGC的SOD案例，根据我们对英语眼科文献的回顾，我们认为我们的案子很新颖。
