Leber's hereditary optic neuropathy (LHON) or Leber optic atrophy

Is a mitochondrially inherited (transmitted from mother to offspring) degeneration of retinal ganglion cells (RGCs) and their axons that leads to an acute or subacute loss of central vision; this affects predominantly young adult males.

Symptoms:

  • Clinically, there is an acute onset of visual loss, first in one eye, and then a few weeks to months later in the other. This typically evolves to very severe optic atrophy and a permanent decrease of visual acuity. Both eyes become affected either simultaneously  or sequentially  with a median inter-eye delay of 8 weeks. Rarely only one eye may be affected.

Preliminary plan:

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