Leber's hereditary optic atrophy neuropathy

Leber's Hereditary Optic Atrophy Neuropathy

Leber's hereditary optic neuropathy (LHON) is an inherited disease caused by mutations in the mitochondrial genes. It is the most common of a group of disorders referred to as mitochondrial cytopathies which affect the mitochondria, the energy generators within cells. LHON affects the nerve cells responsible for vision, causing a slow, painless loss of central vision.

The most common symptom of LHON is a sudden decrease in central vision usually in both eyes. This may come on over days or weeks and can be accompanied by some blurring of other parts of the visual field. Other symptoms can include an inability to see clearly in dim or poor lighting, sensitivity to bright lights, eye pain, lack of color vision, and red-green color deficiencies. Most people with LHON eventually lose all central vision.

LHON is an autosomal condition, which means that it is not usually inherited from parent to child. Most cases are caused by spontaneous genetic changes that occur in the mitochondrial DNA. It affects both men and women, although males tend to be more severely affected than females, often presenting with greater vision loss.

  • LHON affects the nerve cells responsible for vision, causing a slow, painless loss of central vision.
  • The most common symptom is a sudden decrease in central vision usually in both eyes.
  • Other symptoms can include an inability to see clearly in dim or poor lighting, sensitivity to bright lights, eye pain, lack of color vision, and red-green color deficiencies.
  • Most cases are caused by spontaneous genetic changes that occur in the mitochondrial DNA.
  • Males tend to be more severely affected than females, often presenting with greater vision loss.