What is Leber optic atrophy?

Leber hereditary optic neuropathy (LHON) is often characterized by bilateral, painless subacute loss of central vision during young adult life. In most cases, symptoms begin with one eye first, followed a few weeks later by visual failure in the other eye.

What age group is affected by optic atrophy?

Optic atrophy type 1 (OPA1, or Kjer type optic atrophy) is characterized by bilateral and symmetric optic nerve pallor associated with insidious decrease in visual acuity (usually between ages 4 and 6 years), visual field defects, and color vision defects.

What is optical atrophy?

Optic atrophy is the end result of any disease that damages nerve cells anywhere between the retinal ganglion cells and the lateral geniculate body.

What happens in Leber hereditary optic neuropathy?

Leber Hereditary Optic Neuropathy (Sudden Vision Loss) Leber Hereditary Optic Neuropathy (LHON) is a disease inherited from your mother. It causes you to lose your eyesight, starting with painless blurriness. Your central vision, which you need to drive, read and recognize faces, will be affected first.

Is optic atrophy hereditary?

Optic atrophy type 1 is inherited in an autosomal dominant manner. This means that having a genetic change (pathogenic variant or mutation ) in one copy of the OPA1 gene can cause the signs and symptoms of optic atrophy type 1.

What is the treatment for optic atrophy?

Unfortunately, there is no effective treatment for optic atrophy. Once the nerve fibers in the optic nerve are lost they never heal or grow back. However, early diagnosis and treatment of the underlying causes of optic atrophy can help prevent further damage from the disease.

Does optic atrophy get worse?

In general, people with optic atrophy type 1 have worsening vision loss over time. However, some people only have mild vision loss, and for some people the vision loss does not worsen with time.

How common is optic atrophy?

Optic atrophy type 1 is estimated to affect 1 in 35,000 people worldwide. This condition is more common in Denmark, where it affects approximately 1 in 10,000 people.

Is LHON a rare disease?

Leber hereditary optic neuropathy (LHON) is an inherited form of vision loss. Although this condition usually begins in a person’s teens or twenties, rare cases may appear in early childhood or later in adulthood. For unknown reasons, males are affected much more often than females.

Why does LHON affect males more than females?

An analysis of X-inactivation also argues against X-linkage as the cause of sex-bias [4]. Furthermore, LHON typically has an earlier age of onset in males than females. Present evidence therefore suggests that males are more susceptible to the mitochondrial defects.

What does optic atrophy, hereditary, Leber mean?

Leber’s hereditary optic neuropathy (LHON) or Leber hereditary 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.

How is Leber hereditary optic neuropathy diagnosed?

Leber’s Hereditary Optic Neuropathy (LHON) is diagnosed through a number of assessments which help with providing the correct diagnosis. The eye doctor will ask about a person’s medical history, including any family history of eye conditions.

What is the history of Leber hereditary optic neuropathy?

Leber Hereditary Optic Neuropathy (LHON) , also known as Leber Optic Atrophy (LOA), was first described in 1871 as a sudden loss of vision in young men with a family history of blindness. It is the most common of the hereditary Optic Atrophies.

What is treatment for optic neuropathy?

The main treatment options for traumatic optic neuropathy (TON) include systemic corticosteroids and surgical optic nerve decompression, either alone or in combination. Review and analysis of the literature are complicated by the variety of therapeutic approaches and a lack of randomized, controlled studies on the use of these modalities for TON.