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ObservationalPloS one · 2026

Ten-year natural history of visual function in Japanese patients with Leber hereditary optic neuropathy: A retrospective cohort study.

Yasuyuki Takai, Akiko Yamagami, Mayumi Iwasa … Masato Wakakura

OphthalmologyObservational

By 10 years, only about 45% of eyes reached BCVA ≤1.6 logMAR.

Ten-year natural history of visual function in Japanese patients with Leber hereditary optic neuropathy: A retrospective cohort study.

Yasuyuki Takai … Masato Wakakura

PloS one · 2026

Background

Leber hereditary optic neuropathy (leber hereditary optic neuropathy) is a mitochondrial optic neuropathy that typically causes severe bilateral central vision loss.

Methods

Among 174 genetically confirmed leber hereditary optic neuropathy patients, we identified 27 patients (53 eyes) who presented within 6 months of onset and were followed continuously for ≥10 years.

n = 53 eyes

Results

less than half of eyes recovered even modest reading-level vision by 10 years

45%

BCVA ≤1.6 logMAR

26%

BCVA ≤1.3 logMAR

More results

Although several natural-history studies have reported visual outcomes, long-term trajectories beyond 5 years remain incompletely quantified, particularly in Asian cohorts.

Kaplan-Meier analyses estimated time to achieving BCVA ≤1.6 and ≤1.3 logMAR.

More results

During the Chronic phase, BCVA showed a small but statistically significant improvement (overall -0.030 logMAR/year, 95% confidence interval (CI) -0.045 to -0.015; -0.025 logMAR/year, 95% CI -0.044 to -0.007 in a pre-specified m.11778G > A subgroup).

“

Conclusion

Given the modest sample size and selection of long-term attendees, these estimates should be interpreted as descriptive, phase-specific benchmarks rather than definitive evidence of long-term stability or ethnic differences.

Post

Ten-year natural history of visual function in Japanese patients with Leber hereditary optic neuropathy: A retrospective cohort study.

By 10 years, only about 45% of eyes reached BCVA ≤1.6 logMAR.

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