Disease Information for Optic atrophy, hereditary, of Leber

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Clinical Manifestations
Signs & Symptoms
Nystagmus
Central Vision Loss/Scotoma
Chronic Vision Loss, Persistent
Color vision dimmimg, monocular
Decreasing visual acuity
Decreasing visual acuity in Children
Eye symptoms/signs
Loss of color vision
Pallor/white optic disk
Vision Loss
Visual symptoms
True Vertigo Sign Confirmed
Disease Progression
Course/Chronic disorder
Course/Chronic only
Demographics & Risk Factors
Population Group
Child
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Child-Infant Only
Population/Children/all
Diagnostic Test Results
Other Tests & Procedures
TEST/Color vision abnormality
Visual Fields Test Abnormal
Visual fields/Central scotomata
Visual Fields/Central visual field loss
Associated Diseases & Rule outs
Rule Outs
Multiple sclerosis
Optic neuritis
Associated Disease & Complications
Blindness
Blindness in Children
Congenital Nystagmus
Optic atrophy/hereditary Leber's
Disease Mechanism & Classification
Class
CLASS/Ophthalmology Specialty Population
CLASS/Ophthalmology disorders
CLASS/Eyes/accessory (category)
Pathophysiology
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Pathophysiology/Gene mutation Retinoic acid/Retina cells
Pathophysiology/Gene mutation RPE65
Process
PROCESS/Atrophic disorders (ex)
PROCESS/Hereditofamilial (category)
PROCESS/Use/Age/atrophic disorder (category)
Synonyms
Synonym
atrophy Leber, Atrophy optic Leber's, Leber atrophy, Leber heredit optic neuropath, Leber heredit optic neuropathy, Leber Hereditary Optic Atrophy, Leber hereditary optic neuropathy, LEBER HEREDITARY OPTIC NEUROPATHY LHON, Leber optic atrophy, Leber's Hereditary Optic Atrophy, Leber's hereditary optic atrophy neuropathy, Leber's hereditary optic neuropathy, Lebers Optic Atrophy, Leber's optic atrophy, Leber's optic atrophy (disorder), LHON, LHON Leber hered optic neuro, LHON Leber hereditary optic neuropathy, LHON Leber's hereditary optic neuropathy, LHON Leber hered optic neuropa, MTCO1*LHON7444A, MTCO3*LHON9438A, MTCO3*LHON9804A, MTCYB*LHON15257A, MTCYB*LHON15812A, MTND1*LHON3394C, MTND1*LHON3460A, MTND1*LHON3733G A, MTND1*LHON4136G, MTND1*LHON4160C, MTND1*LHON4171A, MTND1*LHON4216C, MTND2*LHON4640A, MTND2*LHON4917G, MTND2*LHON5244A, MTND4*LHON11778A, MTND5*LHON13708A, MTND5*LHON13730A, MTND6*LHON14482A, MTND6*LHON14484C, MTND6*LHON14495G, NADH DEHYDROGENASE SUBUNIT 1 MITOCHONDRIAL MUTATION IN, Optic Atrophy Hereditary Leber, Optic Atrophy Leber, Optic Atrophy Leber Hereditary, Optic atrophy Leber's, Synonym/Hereditary optic neuritis, Synonym/Leber's disease
Treatment
Surgical Procedures or Treatments
SX/Gene Therapy Eye
Definition

Leber"s Hereditary Optic Neuropathy (LHON) , also known as Leber"s Optic Atrophy (LOA) , Leber"s Optic Neuropathy (LON) or Leber"s Disease; It is often referred to as just Leber"s; Leber"s Hereditary Optic Neuropathy is a rare condition which can cause loss of central vision; It usually affects men, most commonly in the late twenties or early thirties, but the symptoms can happen at any age, to men or women; Usually Leber"s affects one eye first, so central vision is lost in that eye over a period of a few weeks; One or two months later, the second eye is affected in the same way; The time when someone is losing their eyesight is often called the "acute" period; After a few more weeks, the eyesight stops getting worse; Although that describes the most common pattern for Leber"s, it can also affect someone very suddenly, or can affect them more gradually over a period of years; Leber"s is a genetic condition, that is, it is passed down through the family; Not everyone in a family affected by Leber"s will lose their eyesight; Leber"s Hereditary Optic Neuropathy is linked to a number of genes, all in the DNA of structures called Mitochondria; These provide energy to the cells of the body; We believe that the particular gene changes linked to Leber"s Hereditary Optic Neuropathy lower the amount of energy available to the cells of the optic nerve and retina; These cells are damaged and can even die because of this lack of energy; The damage to the optic nerve and retina is what causes the symptoms of Leber"s;----------[Leber ewebsite 2005]-------------------

Leber Hereditary Optic Neuropathy; Hereditary Optic Neuroretinopathy; Leber"s Disease; Leber"s Optic Atrophy;

Leber"s Optic Neuropathy (LHON); Leber hereditary optic neuropathy (LHON) is a rare inherited disorder of the eye that is characterized by the relatively slow, painless, progressive loss of vision; This disorder can initially affect one eye or both, but both eyes are usually affected within six months; In most cases, visual loss is permanent; LHON is a genetic disorder that occurs as the result of a mutation in the mitochondrial DNA that is inherited from the mother or arises as a new sporadic mitochondrial DNA mutation--------[NORD 2005]----------------

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External Links Related to Optic atrophy, hereditary, of Leber
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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