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Disease Information for Optic atrophy, hereditary, of Leber
- 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
- Wikipedia
- Merck
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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)