Macular nerve fibers temporal to fovea may have a greater potential to recover function in patients with Leber's hereditary optic neuropathy
Macular nerve fibers temporal to fovea may have a greater potential to recover function in patients with Leber's hereditary optic neuropathy
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DOI：
10.1016/s0021-5155(02)00562-2
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发表时间：
2002-11-01
影响因子：
2.4
通讯作者：
Oguchi, Y
中科院分区：
文献类型：
--
作者：
Mashima, Y;Sato, EA;Oguchi, Y
目的：众所周知，年轻的Leber遗传性视神经病变(LHON)患者即使在视力丧失后也能恢复视力。本研究的目的是确定携带11778突变的LHON患者的平均发病年龄，并确定中心视力恢复的模式。方法：对1980年至1999年间在庆应大学附属医院就诊的35例伴有11778突变的LHON患者进行了2~20年的回顾性研究。对视力恢复的患者进行Goldmann视野计、Humphrey视野计和扫描激光眼底镜(SLO)标志性驱动的眼底微视野计检查。结果：35例患者中有9例(14眼)至少有1眼视力恢复到0.3以上。视力恢复组平均发病年龄为15.9±4.6岁，无视力恢复组平均发病年龄为25.5±8.9岁。这一平均发病年龄的差异有显著意义(P=0.0001；韦尔奇t检验)。9例(14只眼)经Humphrey 10-2阈值和SLO微视野检查显示中央开窗暗点。在Humphrey 10-2阈值测试的9例患者中，7例鼻侧中央视野的敏感度高于颞侧。通过SLO微视野计在患者的中央视网膜鼻侧发现了对11778分贝不敏感的区域，固定稳定性与临床视力的高低有关。结论：LHON患者的视力更容易恢复，且发病年龄较早。视野检查结果提示，LHON患者鼻野(视网膜至中心凹)的神经纤维束可能具有更大的恢复功能的潜力。(C)2002年日本眼科学会。
Purpose: It is known that even after visual loss, younger patients with Leber's hereditary optic neuropathy (LHON) can recover vision. The purpose of this study was to determine the mean age at onset for LHON patients with and without visual recovery who carried the 11778 mutation, and to determine the pattern of central vision recovery.Methods: Thirty-five LHON patients with the 11778 mutation of mitochondrial DNA who had visited the Keio University Hospital between 1980 and 1999 and were followed for 2 to 20 years, were the subjects of this retrospective study. The patients who had recovered vision were tested by Goldmann perimetry, Humphrey perimetry, and landmark-driven fundus microperimetry with a scanning laser ophthalmoscope (SLO). The fixation status was assessed by SLO microperimetry.Results: Nine of the 35 patients (14 of 70 eyes) demonstrated a recovery of visual acuity to better than 0.3 in at least one eye. The mean age of disease onset was 15.9 +/- 4.6 years in patients with visual recovery and 25.5 +/- 8.9 years in patients without visual recovery. This difference in the mean age at onset was significant (P = .0001; Welch t-test). These 9 patients (14 eyes) showed fenestrated central scotomas in testing by Humphrey 10-2 threshold and SLO microperimetry. The nasal side of the central visual fields had a higher sensitivity than the temporal side in 7 of the 9 patients in Humphrey 10-2 threshold testing. Areas insensitive to 0 dB were detected on the nasal side of the central retina in these patients by SLO microperimetry, and fixation stability was related to the degree of clinical visual acuity.Conclusion: The LHON patients with the 11778 mutation and a younger age of onset were more likely to show visual recovery. The findings made by perimetry suggest that the nerve fiber bundles in the nasal field (retina temporal to the fovea) may have a greater potential to recover function in LHON patients. (C) 2002 Japanese Ophthalmological Society.