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帕里-龙贝格综合征的眼球运动功能障碍:4例报告

Ocular motor dysfunction in Parry-Romberg syndrome: four cases.

作者信息

Prescott Christina R, Hasbani M Joshua, Levada Andrew J, Silbert Jonathan E, Winterkorn Jacqueline M S, Lesser Robert L

机构信息

Department of Ophthalmology and Visual Science, Yale University School of Medicine, New Haven, Connecticut, USA.

出版信息

J Pediatr Ophthalmol Strabismus. 2011 Dec 6;48 Online:e63-6. doi: 10.3928/01913913-20111129-02.

Abstract

The authors describe four patients with Parry-Romberg syndrome (PRS) who had abnormal eye movements, ptosis, and facial hemiatrophy. There were delays in diagnosis in all cases, often requiring multiple imaging studies, prior to diagnosis of PRS. These cases demonstrate the variable ophthalmic manifestations of PRS, which can lead to difficulty in diagnosis. Case 1 was a 23-year-old woman with lateral rectus paresis who presented with horizontal diplopia and developed ptosis and facial hemiatrophy. Case 2 was a 28-year-old woman with medial rectus weakness adjusted to diplopia by head turn and gaze preference. Case 3 was a 68-year-old woman who presented with dry eye symptoms and was noted to prefer right gaze and have left hemifacial atrophy. Case 4 was a 68-year-old woman who presented with ptosis, enophthalmos, and restriction of up gaze. The ocular motor defect in all of these cases is best explained by muscle fibrosis rather than nerve paresis.

摘要

作者描述了4例伴有异常眼球运动、上睑下垂和面部半侧萎缩的帕里-龙贝格综合征(PRS)患者。所有病例均存在诊断延迟,在诊断PRS之前通常需要进行多项影像学检查。这些病例显示了PRS多样的眼部表现,这可能导致诊断困难。病例1是一名23岁女性,患有外直肌麻痹,表现为水平复视,随后出现上睑下垂和面部半侧萎缩。病例2是一名28岁女性,内直肌无力,通过转头和注视偏好来调整复视。病例3是一名68岁女性,出现干眼症状,被发现偏好右眼注视并伴有左侧面部萎缩。病例4是一名68岁女性,表现为上睑下垂、眼球内陷和上视受限。所有这些病例中的眼球运动缺陷最好用肌肉纤维化而非神经麻痹来解释。

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