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Hereditary optic neuropathy (LHON] Leber’s HON

It is a rare disease which is the result of a maternal mitochondrial DNA mutation at point 11778. (Worse prognosis)
  1. Clinical Features
    Commonly in adolescent males, atypically may involve females.
    Unilateral, acute, severe, painless retrobulbar neuropathy (It is Not Neuritis) followed by involvement of other eye.
  2. On Examination
    1. Mild optic disc hyperaemia
    2. Telangiectatic microangiopathy

Note -Pupillary reactions to light often remain fairly brisk inspite of severe visual loss. *
  1. Treatment
    Prognosis is relatively poor with generally severe, bilateral and permanent visual loss.
    Genetic counseling for females only as males do not transmit Mitochondrial inheritance. 

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