Highlight
– Late-onset LHON differs from classical LHON with a lower male predominance and significant environmental and hormonal influences.
– High-dose idebenone (≥900 mg/day) is associated with improved visual recovery, particularly in patients harboring J or T mitochondrial haplogroups.
– Smoking and postmenopausal status are prevalent among late-onset LHON patients, suggesting these as potential disease conversion triggers.

