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Medicine (MD/DO)

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Mitochondrial Myopathies

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Leber Hereditary Optic Neuropathy

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Leber Hereditary Optic Neuropathy

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Leber Hereditary Optic Neuropathy

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Leber hereditary optic neuropathy (LHON) is a mitochondrial disease that causes optic atrophy. It typically affects adolescent males, who may present with painless vision loss.

4 KEY FACTS

Mitochondrial diseases occur from defects in mitochondrial DNA. LHON is one of these diseases, which leads to dysfunction in mitochondrial energy production.

LHON is characterized by optic nerve fiber atrophy. This is most pronounced in the papillomacular bundle, which can lead to central scotomas.

This disease most often occurs in male adolescents, between 12-18 years of age.

Painless vision loss can be a feature of LHON. The vision loss starts in one eye and gradually affects the other.

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