Macular nerve fibre and ganglion cell layer changes in acute Leber's hereditary optic neuropathy

Macular nerve fibre and ganglion cell layer changes in acute Leber's hereditary optic neuropathy
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DOI:
10.1136/bjophthalmol-2015-307326
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发表时间:
2016-09-01
影响因子:
4.1
通讯作者:
Barboni, Piero
Barboni, Piero
中科院分区:
医学2区
文献类型:
--
作者:
Balducci, Nicole;Savini, Giacomo;Barboni, Piero

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目的 评价急性莱伯氏遗传性视神经病变(LHON)中纵向视网膜神经节细胞内丛状层(GC-IPL)和黄斑视网膜神经纤维层(mRNFL)厚度的变化。方法对4例LHON患者的6只眼在失明后第1、3、6和12个月进行SD-OCT(光学相干断层扫描)。两只眼睛的检查是在症状前阶段进行的。生成接受者操作特征下面积 (AUROC) 的关系和曲线,以评估每个参数检测神经节细胞丢失的能力。结果 在 LHON 中检测到 GC-IPL 和 mRNFL 的显着纵向变薄。在症状前阶段,鼻内环的偏差图中可以检测到 GC-IPL 变薄,然后按照离心和螺旋模式逐渐扩展。同样,mRNFL 变薄始于下鼻部分,并逐渐扩大。第3个月后没有检测到进一步的统计学显着变化。第1个月时在鼻部检测到最高水平的AUROC值,并且鼻下mRNFL厚度达到AUROC值=1。所有参数同样能够检测第 2 个月至第 12 个月的神经节细胞损失。结论 GC-IPL 变薄的自然史遵循特定的减少模式,反映了乳头黄斑纤维的解剖过程。第 6 个月代表 GC-IPL 损失结束。 GC-IPL 和 mRNFL 变薄在视力丧失发生前即可检测到。这些观察结果可以帮助未来针对高转化风险的 LHON 携带者和急性早期 LHON 患者的治疗方法。
Aims To evaluate longitudinal retinal ganglion cell inner plexiform layer (GC-IPL) and macular retinal nerve fibre layer (mRNFL) thickness changes in acute Leber's hereditary optic neuropathy (LHON).Methods Six eyes of four patients with LHON underwent SD-OCT (optical coherence tomography) at month 1, 3, 6 and 12 after visual loss. In two eyes, the examination was carried out in the presymptomatic stage. The relationship and curves for area under the receiver operator characteristic (AUROC) were generated to assess the ability of each parameter to detect ganglion cell loss.Results Significant longitudinal thinning of GC-IPL and mRNFL was detected in LHON. GC-IPL thinning was detectable in the deviation map during the presymptomatic stage in the inner ring of the nasal sector and then it progressively extended following a centrifugal and spiral pattern. Similarly, mRNFL thinning began in the inferonasal sector and it progressively extended. No further statistically significant changes were detected after month 3. The highest level of AUROC values at 1month were detected in the nasal sectors and inferonasal mRNFL thickness reached AUROC value=1. All the parameters were equally able to detect ganglion cell loss from month 2 to 12.Conclusions The natural history of GC-IPL thinning follows a specific pattern of reduction, reflecting the anatomical course of papillomacular fibres. Month 6 represents the end of GC-IPL loss. GC-IPL and mRNFL thinning is detectable before onset of visual loss. These observations can help future therapeutic approaches for both LHON carriers at high risk of conversion and patients with acute early LHON.