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杜兴氏肌营养不良症:暗视闪光视网膜电图阴性及暗适应正常。

Duchenne muscular dystrophy: negative scotopic bright-flash electroretinogram and normal dark adaptation.

作者信息

Tremblay F, De Becker I, Riddell D C, Dooley J M

机构信息

Department of Ophthalmology, Dalhousie University, Halifax, NS.

出版信息

Can J Ophthalmol. 1994 Dec;29(6):280-3.

PMID:7834567
Abstract

Recent reports indicate that most subjects with Duchenne muscular dystrophy (DMD) have a negative scotopic bright-flash electroretinogram (ERG). We performed dark adaptometry in seven patients with the DMD phenotype. Three patients had the negative bright-flash response combined with severely reduced rod-related activity; their mean psychophysical dark-adapted threshold was -5.5 +/- 0.3 (normally -5.4 +/- 0.3) cd.m-2. Two of the three patients were able to sit through the complete test: the profile of their dark adaptation curve was normal. The four other patients had a normal scotopic ERG, and their mean psychophysical dark-adapted threshold was -5.3 +/- 0.3 cd.m-2. One of the four was able to complete the entire procedure, and a normal profile was found. In patients with DMD the dark adaptation measurements are normal despite abnormalities in rod-related ERG activity.

摘要

近期报告表明,大多数杜氏肌营养不良症(DMD)患者的暗视闪光视网膜电图(ERG)呈阴性。我们对7例具有DMD表型的患者进行了暗适应测定。3例患者的闪光反应呈阴性,同时与视杆细胞相关的活动严重降低;他们心理物理学暗适应阈值的平均值为-5.5±0.3(正常为-5.4±0.3)cd.m-2。这3例患者中有2例能够全程完成测试:他们暗适应曲线的形态正常。另外4例患者的暗视ERG正常,其心理物理学暗适应阈值的平均值为-5.3±0.3 cd.m-2。这4例患者中有1例能够完成整个测试过程,且发现其曲线形态正常。在DMD患者中,尽管视杆细胞相关的ERG活动存在异常,但暗适应测量结果正常。

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