Leber's Hereditary Optic Neuropathy

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Mitochondria Damage

Leber's Hereditary Optic Neuropathy, or Leber Optic Atrophy, is an uncommon condition in which mitochondria in retinal nerve cells are mutated causing loss of central vision. It inherited and primarily affects young men aged 12 to 30. However, symptoms can happen at any age, to men or women.

Self Help & Tips

Every cell in our body contains chromosomes that carry inherited information in genes. Within each cell we have mitochondria that are the energy producers within each cell. These mitochondria also carry genes, and are inherited only from the mother. So when mitochondria are mutated, she will pass it on to all of her children, and her daughters will pass it on to all of their children. However, not all siblings develop the condition.

Leber's generally develops in one eye, and then a few weeks or months later it develops in the other eye. The time when someone is losing their eyesight is often called the 'acute' period. After a few more weeks, the eyesight stops getting worse. Leber's can also develop more gradually over a period of years.

Symptoms

Sudden or gradual onset of central vision, usually starting in one eye.

Causes

  • Leber's is inherited through a gene which is only passed on through the egg cell from the mother.
  • It has suspected that toxic substances which are inhaled, eaten or absorbed via the skin can contribute to onset of eye disease and blindness, particularly for those with a genetic disposition toward an eye disease (or any other disease for that matter). Recent research has found that cigarette smoking can trigger malfunctioning of the genes which otherwise had not been expressed as a problem.
  • People who smoke are far more likely to develop Leber's than non-smokers, as it appears to trigger the condition in at-risk patients. Learn more about smoking and Leber's.

Conventional Treatment

Work on gene therapy in 2009 presents some possibilities, otherwise, the condition is not known to be treatable conventionally.

It may be that some factor in women's X chromosomes may be protective - since women get the condition more rarely.

Clinical trials of ibedenone has been shown to be moderately effective.

Self Help

Many eye problems are related to your overall health. Therefore diet and lifestyle choices are helpful in having and maintaining good vision.

Learn more about vitamins and supplements that support the optic nerve

Leber's News

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Studies

Research on Leber's.

References

Klopstock T,Yu-Wai-Man P, Dimitriadis K, Rouleau J, Heck S, Bailie M, Atawan A, Chattopadhyay S, Schubert M, Garip A, Kernt M, Petraki D, Rummey C, Leinonen M, Metz G, Griffiths PG, Meier T, Chinnery PF. 2011. A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy. Brain,

Tsao K, Aitken PA, Johns DR. 1999. Smoking as an aetiological factor in a pedigree with Leber's hereditary optic neuropathy, Br J Ophthalmol. May 83:577-581.

Ritch R. 2007. Natural compounds: evidence for a protective role in eye disease. Can J Ophthalmol. 2007 Jun;42(3):425-38

Shrader, W. D.; Amagata, A.; Barnes, A.; Enns, G. M.; Hinman, A.; Jankowski, O.; Kheifets, V.; Komatsuzaki, R. et al. 2011. Alpha-Tocotrienol quinone modulates oxidative stress response and the biochemistry of aging. Bioorganic & Medicinal Chemistry Letters 21 (12): 3693-3698

Sudan, A., Acquired mitochondrial impairment as a cause of optic nerve disease, Trans Am Ophthalmol Soc. 1998;96:881-923