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评估莱伯遗传性视神经病变患者的固视模式和阅读能力。

Evaluation of fixation pattern and reading ability in patients with Leber hereditary optic neuropathy.

机构信息

Low Vision Clinic and Research Laboratory (EKA, BRB, XNN, ST-K), and University Eye Clinic (BL-K), Center for Ophthalmology, University of Tübingen, Tübingen, Germany.

出版信息

J Neuroophthalmol. 2013 Dec;33(4):344-8. doi: 10.1097/WNO.0b013e31829d1f5b.

Abstract

BACKGROUND

Leber hereditary optic neuropathy (LHON) is characterized by progressive loss of central vision leading to impaired reading ability. The aim of this study was to evaluate sensory adaptation and reading ability in LHON patients.

METHODS

This prospective pilot study included 12 male patients with a clinical diagnosis and a positive genetic analysis of LHON, who matched the inclusion criteria of a central scotoma on visual field testing and the use of magnifying aids to read. Examination included best-corrected visual acuity, magnification need, reading speed, and evaluation of fixation by corneal reflexes and by Rodenstock scanning laser ophthalmoscope (SLO). Central scotoma was assessed by conventional perimetry (Tübingen Automated Perimeter) and microperimetry (NIDEK MP1).

RESULTS

Mean magnification need was 13.2 ± 7.3-fold (range: 2- to 25-fold). Mean reading speed was 53 ± 18 words per minute (WPM) (range: 24-85 WPM). With automated perimetry, all patients showed central scotomas with a mean radius of 13° ± 7° (range: 1°-30°) in the better eye. Microperimetry in all patients showed fenestrated central scotomas. Eccentric fixation with a preferred retinal locus (PRL) was detected with SLO examination and microperimetry correlated well in 11 of 12 patients. The SLO results showed no systematic pattern in the placement of the PRL; however, 7 of 12 patients (58%) placed their PRL in an unfavorable location left or below the fovea. In 8 of 12 patients, fixation was unstable. Between reading speed and central scotoma size, there was a statistically significant negative correlation (P = 0.021, r = -0.65).

CONCLUSIONS

The percentage of unfavorable PRL locations was extremely high compared with other disorders with central scotomas. Unstable fixation and fenestrated central scotomas led to difficulties in reading. Early rehabilitation and, if necessary, eccentric viewing training should be considered in LHON patients.

摘要

背景

Leber 遗传性视神经病变(LHON)的特征是中央视力逐渐丧失,导致阅读能力受损。本研究的目的是评估 LHON 患者的感觉适应和阅读能力。

方法

这是一项前瞻性的初步研究,纳入了 12 名具有临床诊断和 LHON 阳性基因分析的男性患者,这些患者符合视野测试中出现中央暗点和使用放大辅助工具阅读的纳入标准。检查包括最佳矫正视力、放大需求、阅读速度以及通过角膜反射和 Rodenstock 扫描激光检眼镜(SLO)评估固视。中央暗点通过传统视野计(图宾根自动视野计)和微视野计(尼德克 MP1)进行评估。

结果

平均放大需求为 13.2±7.3 倍(范围:2 倍至 25 倍)。平均阅读速度为 53±18 个单词/分钟(WPM)(范围:24-85 WPM)。使用自动视野计,所有患者在较好眼的平均半径为 13°±7°(范围:1°-30°)显示出中央暗点。所有患者的微视野均显示出有孔的中央暗点。通过 SLO 检查发现偏心固视,并在 11 例 12 例患者中与微视野相关性良好。SLO 结果显示,PRL 的位置没有系统模式;然而,12 例患者中有 7 例(58%)将 PRL 置于不利位置(左眼或低于黄斑)。在 12 例患者中有 8 例患者固视不稳定。阅读速度和中央暗点大小之间存在统计学上显著的负相关(P=0.021,r=-0.65)。

结论

与其他具有中央暗点的疾病相比,不利 PRL 位置的比例极高。不稳定的固视和有孔的中央暗点导致阅读困难。LHON 患者应考虑早期康复,如果需要,还应进行偏心注视训练。

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