fMRI of retina-originated phosphenes experienced by patients with Leber congenital amaurosis.
fMRI of retina-originated phosphenes experienced by patients with Leber congenital amaurosis.
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DOI：
10.1371/journal.pone.0086068
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发表时间：
2014
期刊：
影响因子：
3.7
通讯作者：
Bennett J
中科院分区：
文献类型：
--
作者：
Ashtari M;Cyckowski L;Yazdi A;Viands A;Marshall K;Bókkon I;Maguire A;Bennett J
一种现象的特点是看到光的经验，没有任何光实际进入眼睛被称为光幻视或光幻视。光幻视可以自发发生或通过外部刺激的诱导发生。先前关于光幻视的报道主要集中在外部诱导的光幻视，例如通过向皮层施加交流或直流电。其中一些报告使用功能性磁共振（fMRI）来研究皮层光幻视诱导的激活。然而，有没有功能磁共振成像报告自发光幻视起源于视网膜和由此产生的模式的皮层激活。我们进行了功能磁共振成像在一个逆转棋盘范式在三个LCA患者谁接受了单侧基因治疗，并报告经历了频繁的光幻视每天。我们观察到，当患者报告经历光幻视时，双侧皮质激活覆盖整个视觉皮质。相反，在光幻视的情况下，激活受患者的视觉能力调节，并且由于基因治疗而表现出改善的皮质激活。这些fMRI结果说明了光幻视知觉对视觉功能的潜在影响，它们可以解释临床医生在视网膜变性的视觉功能测试中发现的一些变异性。虽然我们没有进行视觉功能和光幻视之间的相关性，但我们希望这里提供的数据能够提高人们对这种现象及其对视觉功能的潜在影响以及对临床测试的影响的认识。我们建议对视网膜疾病患者进行基因或分子治疗时，应全面了解光幻视的病史。最后，这些数据说明了功能磁共振成像作为基因治疗的结果测量的潜在力量和光幻视可能对视力测试产生的负面影响。功能磁共振成像已被证明是一个敏感的，非侵入性的，可重复的测试范式，为这些目的，可以补充标准的视觉功能测试。
A phenomenon characterized by the experience of seeing light without any light actually entering the eye is called phosphenes or photopsias. Phosphenes can occur spontaneously or via induction by external stimuli. Previous reports regarding phosphenes have primarily focused on externally induced phosphenes such as by applying alternating or direct current to the cortex. A few of these reports used functional magnetic resonance (fMRI) to study activations induced by cortical phosphenes. However, there are no fMRI reports on spontaneous phosphenes originating from the retina and the resulting pattern of cortical activations. We performed fMRI during a reversing checkerboard paradigm in three LCA patients who underwent unilateral gene therapy and reported experiencing frequent phosphene on a daily basis. We observed bilateral cortical activation covering the entire visual cortices when patients reported experiencing phosphenes. In contrast, in the absence of phosphenes, activation was regulated by patient's visual ability and demonstrated improved cortical activation due to gene therapy. These fMRI results illustrate the potential impact of phosphene perception on visual function and they may explain some of the variability that clinicians find in visual function testing in retinal degeneration. Although we did not perform correlations between visual function and phosphenes, we hope data presented here raises awareness of this phenomenon and its potential effect on visual function and the implications for clinical testing. We recommend a thorough history for phosphene experiences be taken in patients with retinal disease who are candidates for gene or molecular therapy. Lastly, these data illustrate the potential power of fMRI as an outcome measure of gene therapy and the negative impact phosphenes may have on vision testing. fMRI has proven to be a sensitive, non-invasive, and reproducible test paradigm for these purposes and can complement standard visual function testing.
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