Reduction of oscillatory potentials and photopic negative response in patients with autosomal dominant optic atrophy with OPA1 mutations
Reduction of oscillatory potentials and photopic negative response in patients with autosomal dominant optic atrophy with OPA1 mutations
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DOI：
10.1167/iovs.06-0845
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发表时间：
2007-02-01
影响因子：
4.4
通讯作者：
Terasaki, Hiroko
中科院分区：
文献类型：
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作者：
Miyata, Kentaro;Nakamura, Makoto;Terasaki, Hiroko
目的.目的研究OPA 1基因突变的常染色体显性视神经萎缩（ADOA）患者的视网膜电图（ERG）表现。研究了8例OPA 1突变的ADOA患者（年龄范围24 - 55岁，平均41岁）。除常规眼科检查外，还记录了全视野ERG，包括视杆反应、混合视杆-视锥反应、振荡电位（OPs）、单闪光视锥反应和明视负反应（PhNR），并与25名年龄匹配的对照组进行比较。ERG数据与光学相干断层扫描测量的视盘周围平均视网膜神经纤维层（RNFL）厚度、静态视野检查（Humphrey 30 - 2程序）的平均偏差或矫正视力之间的相关性也进行了检查。ADOA患者的PhNR和OPs的振幅均明显小于对照组（P < 0.01），两者均起源于视网膜内层。两组其他ERG成分的振幅差异无统计学意义。OP振幅与患者年龄呈负相关。ADOA患者的RNFL较正常人薄，静态视野检查的视网膜敏感度较正常人低，但与PhNR和OPs的振幅无关。这些结果表明，有功能障碍不仅在神经节细胞层，而且在内核层和丛状层，包括无长突细胞的ADOA患者与OPA 1突变。
PURPOSE. To study the electroretinographic (ERG) findings in patients with autosomal dominant optic atrophy (ADOA) with OPA1 mutations.METHODS. Eight ADOA patients (age range, 24 - 55 years; mean, 41 years) with OPA1 mutations were studied. In addition to routine ophthalmological tests, full-field ERGs including the rod response, mixed rod-cone response, oscillatory potentials (OPs), single-flash cone response, and photopic negative response (PhNR) were recorded and compared with those from 25 age-matched controls. The correlation between the ERG data and averaged retinal nerve fiber layer (RNFL) thickness around the optic disk measured by optical coherent tomography, mean deviation of the static perimetry (Humphrey 30 - 2 program), or corrected visual acuity was also examined.RESULTS. Amplitudes of the PhNR and OPs, both of which are believed to originate from inner retinal layers, were significantly smaller in ADOA patients than in control subjects (P < 0.01). Amplitudes of other ERG components were not statistically different in the two groups. OP amplitude was inversely correlated with the patient's age. The RNFL was thinner and the retinal sensitivities obtained by static perimetry were lower in ADOA patients, but these values were not correlated with the amplitude of PhNR or OPs.CONCLUSIONS. These results suggested that there are functional impairments not only in the ganglion cell layer but also in the inner nuclear and plexiform layers, including the amacrine cells of ADOA patients with OPA1 mutations.