LHON (Leber Hereditary Optic Neuropathy) is a rare, inherited mitochondrial disorder that primarily affects the optic nerve, leading to sudden, painless vision loss.
Quick overview:
- Inherited through the maternal line (mitochondrial DNA)
- Most commonly affects young men (ages 15–35)
- Onset: rapid vision loss in one eye, with the second eye often affected weeks later
- Cause: mutations in mitochondrial genes → impaired energy supply to optic nerve cells
- Result: degeneration of retinal ganglion cells → permanent visual impairment
- No cure, but early treatment (e.g., idebenone) may influence the course
Typical signs: central visual field defects, washed-out color perception, while peripheral vision often remains preserved.
LHON belongs to the group of mitochondrial optic neuropathies.