Mashima Yukihiko, Kigasawa Kazuteru, Shinoda Kei, Wakakura Masato, Oguchi Yoshihisa
Department of Ophthalmology, Keio University School of Medicine, 35 Sninanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
Kyorin Eye Center, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka-shi, Tokyo, 181-8611, Japan.
BMC Ophthalmol. 2017 Oct 18;17(1):192. doi: 10.1186/s12886-017-0583-3.
Patients with Leber hereditary optic neuropathy (LHON) have a progressive decrease of their visual acuity which can deteriorate to <0.1. Some patients can have a partial recovery of their vision in one or both eyes. One prognostic factor associated with a recovery of vision is an early-age onset. The purpose of this study was to determine other clinical factors that are predictive of a good visual recovery.
Sixty-one Japanese LHON patients, with the 11,778 mutation and a mean age of 23.1 ± 12.1 years at the onset, were studied. All patients were initially examined at an acute stage of LHON and were followed for 3 to 10 years. At 1 year after the onset, the lowest visual acuity was <0.1 in all eyes. We studied the following parameters of patients with/without a final visual acuity of ≥ 0.2: sex; heavy consumption of cigarettes and alcohol; taking idebenone; mean age at onset; mean lowest visual acuity; and distribution of the lowest and the final visual acuity.
Fifteen (24.6%) of the 61 patients or 25 (20.5%) of the 122 eyes had a recovery of their visual acuity to ≥ 0.2. The mean age at onset of these 15 patients with visual recovery to ≥ 0.2 was 17.5 ± 7.7 years, and that of the 46 patients without visual recovery to ≥ 0.2 was 25.0 ± 12.8 years (P = 0.02, Mann-Whitney U test). The mean lowest visual acuity of the 25 eyes with visual recovery ≥ 0.2 was 0.04, and that of the 97 eyes without visual recovery to ≥ 0.2 was 0.015 (P < 0.001, Mann-Whitney U test). Fifty percent (15/30) of the eyes whose lowest visual acuity was ≥ 0.04 during 1 year after the onset had a visual recovery to ≥ 0.2, while 11% (10/92) of the eyes whose the lowest visual acuity was ≤ 0.03 had a visual recovery to ≥ 0.2 (P < 0.001, χ test). There were no significant differences in the other clinical factors.
A final visual acuity of ≥ 0.2 was associated with a less severe reduction of the visual acuity at 1 year after the onset. Our findings can be used to predict the visual prognosis in LHON patients.
Leber遗传性视神经病变（LHON）患者的视力会逐渐下降，可恶化至<0.1。一些患者的一只或两只眼睛的视力可部分恢复。与视力恢复相关的一个预后因素是发病年龄早。本研究的目的是确定其他可预测良好视力恢复的临床因素。
对61例携带11778突变、发病时平均年龄为23.1±12.1岁的日本LHON患者进行了研究。所有患者均在LHON急性期进行了初始检查，并随访3至10年。发病后1年时，所有眼睛的最低视力均<0.1。我们研究了最终视力≥0.2和未达到该标准的患者的以下参数：性别；大量吸烟和饮酒；服用艾地苯醌；发病时的平均年龄；平均最低视力；以及最低视力和最终视力的分布情况。
61例患者中有15例（24.6%）或122只眼中有25只（20.5%）视力恢复至≥0.2。这15例视力恢复至≥0.2的患者的发病平均年龄为17.5±7.7岁，46例视力未恢复至≥0.2的患者的发病平均年龄为25.0±12.8岁（曼-惠特尼U检验，P=0.02）。25只视力恢复≥0.2的眼睛的平均最低视力为0.04，97只视力未恢复至≥0.2的眼睛的平均最低视力为0.015（曼-惠特尼U检验，P<0.001）。发病后1年内最低视力≥0.04的眼睛中有50%（15/30）视力恢复至≥0.2，而最低视力≤0.03的眼睛中有11%（10/92）视力恢复至≥0.2（χ检验，P<0.001）。其他临床因素无显著差异。
最终视力≥0.2与发病后1年时视力下降程度较轻相关。我们的研究结果可用于预测LHON患者的视力预后。
