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眼咽型肌营养不良的视轴矫正及视频眼动图分析

Orthoptic and video-oculographic analyses in oculopharyngeal muscular dystrophy.

作者信息

Renard Dimitri, Ferraro Adelaide, Lorenzini Marie-Celine, Jeanjean Luc, Portal Marie-Claire, Llinares Elisabeth, Labauge Pierre, Castelnovo Giovanni

机构信息

Department of Neurology, CHU Nîmes, Hôpital Caremeau, Place du Pr Debré, 30029, Nîmes Cedex 4, France.

Department of Ophthalmology, CHU Nîmes, Hôpital Caremeau, Nîmes, France.

出版信息

Muscle Nerve. 2015 Oct;52(4):554-8. doi: 10.1002/mus.24600. Epub 2015 Aug 26.

DOI:10.1002/mus.24600
PMID:25677583
Abstract

INTRODUCTION

Mild ophthalmoparesis can be seen in oculopharyngeal muscular dystrophy (OPMD).

METHODS

Orthoptic analysis included assessment of phoria/tropia, eye excursion, saccades, pursuit, stereoacuity, and Hess-Lancaster screen test. Video-oculography included fixation, horizontal and vertical saccades, and pursuit.

RESULTS

Orthoptic abnormalities were: tropia (4 of 6); abnormal eye excursion (4 of 6, 78% involved lateral or superior rectus muscles); abnormal horizontal or vertical saccades (2 of 6); abnormal pursuit (0 of 6); abnormal stereoacuity (2 of 6); and pathological Hess-Lancaster screen (4 of 6). Video-oculographic abnormalities were present for: fixation (1 of 6); saccade latency (1 of 6); horizontal pursuit (3 of 6); and vertical pursuit (0 of 6). For horizontal saccades, mean velocity, peak velocity, and gain were pathological in 5 of 6, 5 of 6 (61% of pathological mean and peak velocities involved abducting eye movements), and 3 of 6, respectively. For vertical saccades, mean velocity, peak velocity, and gain were pathological in 4 of 6, 4 of 6 (53% involved upward movements), and 3 of 6, respectively.

CONCLUSION

The data indicate preferential involvement of lateral and (to a lesser degree) superior rectus muscles in OPMD.

摘要

引言

轻度眼肌麻痹可见于眼咽型肌营养不良症(OPMD)。

方法

视轴矫正分析包括对隐斜/显斜、眼球运动、扫视、跟踪、立体视锐度和Hess-Lancaster屏面试验的评估。眼震电图检查包括注视、水平和垂直扫视以及跟踪。

结果

视轴矫正异常情况如下:显斜(6例中有4例);眼球运动异常(6例中有4例,78%累及外直肌或上直肌);水平或垂直扫视异常(6例中有2例);跟踪异常(6例中0例);立体视锐度异常(6例中有2例);以及Hess-Lancaster屏面试验结果异常(6例中有4例)。眼震电图异常情况如下:注视(6例中有1例);扫视潜伏期(6例中有1例);水平跟踪(6例中有3例);以及垂直跟踪(6例中0例)。对于水平扫视,平均速度、峰值速度和增益在6例中有5例、6例中有5例(61%的异常平均速度和峰值速度涉及外展眼球运动)以及6例中有3例分别出现异常。对于垂直扫视,平均速度、峰值速度和增益在6例中有4例、6例中有4例(53%涉及向上运动)以及6例中有3例分别出现异常。

结论

数据表明在OPMD中外直肌和(程度较轻的)上直肌受累更为常见。

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